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TUMBUH KEMBANG REMAJA. M. Bambang Edi Susyanto Blok 8 2010 FKIK UMY. REMAJA. Periode yang ditandai dengan pertumbuhan dan perkembangan yang cepat dari fisik, emosi, kognitif dan sosial yang menjembatani masa kanak-kanak dan dewasa - PowerPoint PPT Presentation
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TUMBUH KEMBANG REMAJA
M Bambang Edi Susyanto
Blok 8 2010
FKIK UMY
REMAJA
bull Periode yang ditandai dengan pertumbuhan dan perkembangan yang cepat dari fisik emosi kognitif dan sosial yang menjembatani masa kanak-kanak dan dewasa
bull Batasan usia relative tidak jelas Merujuk pada periode antara anak-anak dan dewasa ketika perkembangan biopsikososial telah terjadi
bull Umur 11-12 tahun sampai 18-21 tahun
Remaja awal (11-14 tahun)
bull Percepatan pertumbuhan fisik Perempuan biasanya lebih tinggi daripada teman laki-laki sebayanya
bull Isu penting perubahan fisik yang luar biasa cepat (apakah saya normal) dan kemandirian
Remaja Tengah (15-17 tahun)
Pubertas biasanya hampir tuntas sehingga perhatian remaja terfokus pada identitas pribadi dan aliansi dengan teman sebayanya
bull Isu otonomi Pengaruh teman sebaya sangat kuat
bull
Remaja Lanjut (Usia 18-21 tahun)
bull Perhatian remaja beralih pada masa depan mereka Keterlibatan dengan teman sebaya biasanya tidak lagi dengan suatu kelompok saja Mulai ada komitmen dalam hubungan antar personal Berfikir formal dan konseptual
FISIOLOGI PUBERTAS
Pubertas
bull Peralihan dari imaturitas seksual ke masa potensial subur yang berhubungan dengan munculnya tanda kelamin sekunder Jadi awal dan akhir pubertas lebih jelas daripada awal dan akhir remaja
bull Biasanya awal pubertas wanita 2 tahun lebih awal
Tanda Pubertas Perempuan
bull Tanda pertama thelarche (perkembangan payudara) dan pada 5 adrenarche (tumbuhnya rambut pubis)
bull Onset perkembangan payudara kadang unilateral biasanya seimbang dalam 6 bulan
bull Sebagian besar menarche terjadi dalam masa 6 bulan masa puncak kecepatan pertambahan tinggi badan
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
REMAJA
bull Periode yang ditandai dengan pertumbuhan dan perkembangan yang cepat dari fisik emosi kognitif dan sosial yang menjembatani masa kanak-kanak dan dewasa
bull Batasan usia relative tidak jelas Merujuk pada periode antara anak-anak dan dewasa ketika perkembangan biopsikososial telah terjadi
bull Umur 11-12 tahun sampai 18-21 tahun
Remaja awal (11-14 tahun)
bull Percepatan pertumbuhan fisik Perempuan biasanya lebih tinggi daripada teman laki-laki sebayanya
bull Isu penting perubahan fisik yang luar biasa cepat (apakah saya normal) dan kemandirian
Remaja Tengah (15-17 tahun)
Pubertas biasanya hampir tuntas sehingga perhatian remaja terfokus pada identitas pribadi dan aliansi dengan teman sebayanya
bull Isu otonomi Pengaruh teman sebaya sangat kuat
bull
Remaja Lanjut (Usia 18-21 tahun)
bull Perhatian remaja beralih pada masa depan mereka Keterlibatan dengan teman sebaya biasanya tidak lagi dengan suatu kelompok saja Mulai ada komitmen dalam hubungan antar personal Berfikir formal dan konseptual
FISIOLOGI PUBERTAS
Pubertas
bull Peralihan dari imaturitas seksual ke masa potensial subur yang berhubungan dengan munculnya tanda kelamin sekunder Jadi awal dan akhir pubertas lebih jelas daripada awal dan akhir remaja
bull Biasanya awal pubertas wanita 2 tahun lebih awal
Tanda Pubertas Perempuan
bull Tanda pertama thelarche (perkembangan payudara) dan pada 5 adrenarche (tumbuhnya rambut pubis)
bull Onset perkembangan payudara kadang unilateral biasanya seimbang dalam 6 bulan
bull Sebagian besar menarche terjadi dalam masa 6 bulan masa puncak kecepatan pertambahan tinggi badan
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Remaja awal (11-14 tahun)
bull Percepatan pertumbuhan fisik Perempuan biasanya lebih tinggi daripada teman laki-laki sebayanya
bull Isu penting perubahan fisik yang luar biasa cepat (apakah saya normal) dan kemandirian
Remaja Tengah (15-17 tahun)
Pubertas biasanya hampir tuntas sehingga perhatian remaja terfokus pada identitas pribadi dan aliansi dengan teman sebayanya
bull Isu otonomi Pengaruh teman sebaya sangat kuat
bull
Remaja Lanjut (Usia 18-21 tahun)
bull Perhatian remaja beralih pada masa depan mereka Keterlibatan dengan teman sebaya biasanya tidak lagi dengan suatu kelompok saja Mulai ada komitmen dalam hubungan antar personal Berfikir formal dan konseptual
FISIOLOGI PUBERTAS
Pubertas
bull Peralihan dari imaturitas seksual ke masa potensial subur yang berhubungan dengan munculnya tanda kelamin sekunder Jadi awal dan akhir pubertas lebih jelas daripada awal dan akhir remaja
bull Biasanya awal pubertas wanita 2 tahun lebih awal
Tanda Pubertas Perempuan
bull Tanda pertama thelarche (perkembangan payudara) dan pada 5 adrenarche (tumbuhnya rambut pubis)
bull Onset perkembangan payudara kadang unilateral biasanya seimbang dalam 6 bulan
bull Sebagian besar menarche terjadi dalam masa 6 bulan masa puncak kecepatan pertambahan tinggi badan
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Remaja Tengah (15-17 tahun)
Pubertas biasanya hampir tuntas sehingga perhatian remaja terfokus pada identitas pribadi dan aliansi dengan teman sebayanya
bull Isu otonomi Pengaruh teman sebaya sangat kuat
bull
Remaja Lanjut (Usia 18-21 tahun)
bull Perhatian remaja beralih pada masa depan mereka Keterlibatan dengan teman sebaya biasanya tidak lagi dengan suatu kelompok saja Mulai ada komitmen dalam hubungan antar personal Berfikir formal dan konseptual
FISIOLOGI PUBERTAS
Pubertas
bull Peralihan dari imaturitas seksual ke masa potensial subur yang berhubungan dengan munculnya tanda kelamin sekunder Jadi awal dan akhir pubertas lebih jelas daripada awal dan akhir remaja
bull Biasanya awal pubertas wanita 2 tahun lebih awal
Tanda Pubertas Perempuan
bull Tanda pertama thelarche (perkembangan payudara) dan pada 5 adrenarche (tumbuhnya rambut pubis)
bull Onset perkembangan payudara kadang unilateral biasanya seimbang dalam 6 bulan
bull Sebagian besar menarche terjadi dalam masa 6 bulan masa puncak kecepatan pertambahan tinggi badan
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Remaja Lanjut (Usia 18-21 tahun)
bull Perhatian remaja beralih pada masa depan mereka Keterlibatan dengan teman sebaya biasanya tidak lagi dengan suatu kelompok saja Mulai ada komitmen dalam hubungan antar personal Berfikir formal dan konseptual
FISIOLOGI PUBERTAS
Pubertas
bull Peralihan dari imaturitas seksual ke masa potensial subur yang berhubungan dengan munculnya tanda kelamin sekunder Jadi awal dan akhir pubertas lebih jelas daripada awal dan akhir remaja
bull Biasanya awal pubertas wanita 2 tahun lebih awal
Tanda Pubertas Perempuan
bull Tanda pertama thelarche (perkembangan payudara) dan pada 5 adrenarche (tumbuhnya rambut pubis)
bull Onset perkembangan payudara kadang unilateral biasanya seimbang dalam 6 bulan
bull Sebagian besar menarche terjadi dalam masa 6 bulan masa puncak kecepatan pertambahan tinggi badan
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
FISIOLOGI PUBERTAS
Pubertas
bull Peralihan dari imaturitas seksual ke masa potensial subur yang berhubungan dengan munculnya tanda kelamin sekunder Jadi awal dan akhir pubertas lebih jelas daripada awal dan akhir remaja
bull Biasanya awal pubertas wanita 2 tahun lebih awal
Tanda Pubertas Perempuan
bull Tanda pertama thelarche (perkembangan payudara) dan pada 5 adrenarche (tumbuhnya rambut pubis)
bull Onset perkembangan payudara kadang unilateral biasanya seimbang dalam 6 bulan
bull Sebagian besar menarche terjadi dalam masa 6 bulan masa puncak kecepatan pertambahan tinggi badan
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Pubertas
bull Peralihan dari imaturitas seksual ke masa potensial subur yang berhubungan dengan munculnya tanda kelamin sekunder Jadi awal dan akhir pubertas lebih jelas daripada awal dan akhir remaja
bull Biasanya awal pubertas wanita 2 tahun lebih awal
Tanda Pubertas Perempuan
bull Tanda pertama thelarche (perkembangan payudara) dan pada 5 adrenarche (tumbuhnya rambut pubis)
bull Onset perkembangan payudara kadang unilateral biasanya seimbang dalam 6 bulan
bull Sebagian besar menarche terjadi dalam masa 6 bulan masa puncak kecepatan pertambahan tinggi badan
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Tanda Pubertas Perempuan
bull Tanda pertama thelarche (perkembangan payudara) dan pada 5 adrenarche (tumbuhnya rambut pubis)
bull Onset perkembangan payudara kadang unilateral biasanya seimbang dalam 6 bulan
bull Sebagian besar menarche terjadi dalam masa 6 bulan masa puncak kecepatan pertambahan tinggi badan
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Tanda Pubertas Perempuan
bull Tanda progresi lainnya ndash Breast budingndash Pubic hair growthndash Peak high velocityndash Menarche
bull Pertumbuhan biasanya berhenti 2-3 tahun setelah menarche
bull
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Tanda Pubertas Laki-laki
bull Tanda pertama pembesaran testis
bull Tanda lainnya ndash Tumbuhnya rambut pubis ndash Pembesaran penisndash Kecepatan puncak pertambahan tinggindash Pertumbuhan biasanya berhenti 2-3 tahun
setelah puncak kecepatan pertambahan tinggi
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Sexual Maturity Rating
bull Dipublikasikan Tanner pada tahun 1962
bull Skala Tanner tingkat perkembangan genital secara klinis
bull Laki-laki pertumbuhan rambut genital dan pubis
bull Perempuan perubahan rambut pubis dan payudara
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Pubertas Perempuan
bull Terlalu awal jika perkembangan payudara sebelum usia 8 tahun atau menarke kurang dari 10 tahun
bull Terlalu lambat jika payudara tidak berkembang pada usia 13 tahun atau belum menarke pada usia 16 tahun
bull
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Pubertas Laki-laki
bull Terlalu awal jika pembesaran testis (gt25 cm) sebelum usia 9 tahun
bull Terlalu lambat jika pembesaran testis tidak terjadi hingga usia 14 tahun
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Pertumbuhan fisik
Pertumbuhan fisik dan perkembangan fisik hasil aktivasi aksis hipotalamus-hipofisis-gonad
Pubertas inhibisi GnRH di hipotalamus hilang - produksi dan pelepasan pulsatil gonadotropin luteinizing hormone (LH) dan follicle stimulating hormone (FSH)
Awal dan tengah masa remaja kenaikan frekuensi dan amplitudo stimulasi gonad produksi estrogen atau testosteron
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Pertumbuhan fisik
Wanita
FSH stimulasi maturasi ovarium fungsi sel granulosa dan sekresi estradiol
awal inhibisi pelepasan LH dan FSH
lalu perangsang LH dan FSH siklis
LH ovulasi pembentukan korpus luteum dan sekresi progesteron
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Pertumbuhan fisik
Pria
LH stimulasi sel interstitial testis testosteron selama pubertas testosteron sirkulasi = 20 kali lipat staudium fisik dan maturasi tulang rangka
FSH pembentukan spermatosit
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Lonjakan pertumbuhan
bull Biasanya 2-4 tahun
bull Perempuan 2 tahun lebih awal
bull Kecepatan tinggi puncak ndash W 115-12 tahunndash L 135-14 tahun
bull Pertambahan BB sampai 2 x
bull Pertambahan TB 15-20
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Perkembangan psikososial
bull Mencari jati diri apa yang ingin dilakukan dan kekuatan-kelemahan
bull Periode progresif dan perpisahan dari keluarga
bull Fase-fase perkembangan psikososial--gt
3 fase remaja (lihat bagian depan)
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
MASALAH-MASALAH REMAJA
bull Morbiditas
bull Mortalitas
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Morbiditas
bull Kehamilan yang tak diinginkan
bull Penyakit menular seksual
bull Penyalahgunaan zat
bull Merokok
bull Depresi
bull Psikofifiologis
bull Kekerasan fisik
bull Lari dari rumah
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Masalah lain
bull Depresibull Bunuh diri remajabull Penyalahgunaan zatbull Gangguan makanbull Obesitas eksogenbull Kegagalan di sekolahbull Gangguan payudarabull Kelainan ginekologis bull PMS dan Penyakit radang pelvis
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Psychological Problems in Adolescence
bull Depression 13 of teens have experienced some symptoms of depression
bull Rates are higher among girls than boys
bull Rates are higher among African-American and Native American teens
bull Additionally lack of popularity rejection death of a loved one contributes
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Psychological Problems in Adolescence
bull Teen suicide rate has tripled in last 30 years
bull Annual rate now 122 per 100000 3rd most common cause of death for those age 15-24
bull Successful suicide rate is higher in boys (more lethal means) but girls attempt suicide more often
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Teen suicide (contrsquod)
bull Risk factors ndash Depression Social inhibition
Perfectionism
ndash Anxiety Family conflicts romantic rejection
ndash History of drugalcohol abuse gaylesbian orientation
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Cluster Suicides
bull One suicide in a teen community leads to attempts by others to kill themselves especially if the first suicide is high profile and well publicized
bull Schools increasing using crisis teams to counsel students after one student is successful in suicide
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Warning signs of suicide
bull Direct or indirect talk of suicidebull School difficulties writing a willbull Giving stuff away arranging for pet
carebull Change in appetite general
depressionbull Changes in behavior preoccupation
with death in art music or literature
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Indikator remaja berisiko tinggi
1 Penurunan kemampuan belajar
2 Absen sekolah yang berlebihan
3 Keluhan psikosomatik yang seringmenetap
4 Perubahan kebiasaan tidur atau makan
5 Kesulitan konsentrasi atau kebosanan yang menetap
6 Tanda dan gejala stres atau kecemasan
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Indikator remaja berisiko tinggi
7 Menarik diri atau berpindah kelompok8 Perilaku menentang atau kekerasan yang
hebat dan atau perubahan kepribadian yang radikal
9 Konflik dengan orang tua10 Perilaku seksual yang berlebihan11 Konflik dengan hukum12 Memperlihatkan pikiran bunuh diri13 Penyalahgunaan obat dan alkohol14 Melarikan diri dari rumah
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Gejala psikofisiologis
bull Reaksi konversi perasaan tidak menyenangkan dikomunikasikan dengan gejala fisik ldquoperolehan sekunderrdquo
bull Riwayat dan temuan fisik tidak konsisten dengan konsep anatomi dan fisiologis
bull Cenderung mempunyai OT yang overprotektif dan menjadi semakin tergantung pada OT
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Terapi gejala psikofisiologi
bull Jelaskan hubungan antara penyebab fisik dari nyeri emosional atau penyebab emosional dari nyeri fisik
bull Memberi dorongan untuk mengerti bahwa gejala dapat menetap
bull Membantu pasien meneruskan aktivitas harian yang normal
bull Obat-obatan jarang membantubull Dokter suportif dan tidak menduga bahwa
nyeri tidak benar-benar terjadi
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
KLINIK REMAJA
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Sikap Dokter Dalam Menghadapi Pasien Remaja
bull Dokter tampil jujur sederhana tidak perlu tampil ldquoprofesionalrdquo berlebihan
bull Remaja kurang PD dokter hati-hati
bull Sensitif terhadap tingkat perkembangan
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Pemberian pelayanan kesehatan
bull Membangun hubungan saling percaya merupakan dasar dalam pemenuhan kebutuhan pelayanan kesehatan pasien remaja pasien tidak bohong memberikan informasi yang penting untuk diagnosis dan terapi yang tepat
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Kerahasiaan
bull Beritahu remaja dan orang tuanya tentang kerahasiaan yang akan dijaga
bull Waktu adekuat
bull Yakinkan bahwa dokter tidak akan mencampuri kehidupan pribadi remaja tetapi merupakan hal penting untuk kesehatannya
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
What are The importan Aspects of an Adolescent History
bull HEADSSS untuk melacak informasi psikososial yang penting dari pasien remaja
bull H Homehealthbull E EducationEmploymentEatingbull A ActivitiesAspirationAffiliationbull D Drugsbull S Sexbull S SleepSuicidebull S Shoplifting
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
How to Talk to Teens about Puberty
1048708 Be open and honest
1048708 Treat the teen with respect
1048708 Talk directly to the teen
1048708 Begin conversation with least
threatening topics
1048708 Provide confidentiality
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Wawancara
Wawancara terpimpinPenilaian tugas-tugas psikoperkembanganPemeriksaan sistemik meliputi 1 Nutrisi2 Tidur3 Perawatan diri pengetahuan ttg
pemeriksaan sendiri 4 Olah raga5 Hubungan keluarga dan sahabat6 Teman sebaya
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Wawancara
7 Sekolah8 Minat pendidikan dan pekerjaan 9 Tembakau10 Penyalahgunaan zat11 Seksualitas12 Kesehatan mental
Usahakan pasien mendapat kesan tertarik dan seperti mempunyai ldquodokternyardquo sendiri
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
LAMPIRAN
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
PHYSIOLOGY OF PUBERTYPhysical Changes of Puberty (Tanner)
A Female The first sign is an increase in growth velocity pubertal growth spurt - Breast development is the first sign of puberty noted by most examiners - Increased estrogen secretion at the time of menarche
- Other features reflecting estrogen action include enlargement of the labia minora and majora dulling of the vaginal mucosa (reddish) and production of aclear or slightly whitish vaginal secretion prior to menarche
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
- Pubic hair development is determined chiefly by adrenal and ovarian androgen secretion
Stages of breast development (Marshall and Tanner)
- Stage B1 Preadolescent elevation of papilla only- Stage B2 Breast bud stage elevation of breast and papilla
as a small mound and enlargement of areolar diameter - Stage B3 Further enlargement of breast and areola with no
separation of their contours - Stage B4 Projection of areola and papilla to form a
secondary mound above the level of the breast - Stage B5 Mature stage projection of papilla only owing to
recession of the areola to the general contour of the breast
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Stages of female pubic hair dev (Marshall and Tanner)
- Stage P1 Preadolescent the vellus over the area is no further developed than that over the anterior abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly along the labia difficult to see on photographs and is subtle
- Stage P3 Hair is considerably darker coarser and curlier The hair spreads sparsely over the junction of the labia majora
- Stage P4 Hair is now adult in type there is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle of the classic feminine pattern inverse triangle
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
B Male Changes - The first sign of normal puberty in boys is usually
increase in the size of the testes to over 25 cm in the longest diameter
- Pubic hair development is caused by adrenal and
testicular androgens
- The appearance of spermatozoa in early morning urinary specimens (spermarche) occurs at a mean chronologic age of 134 years this usually occurs at gonadal stage
3-4 and pubic hair stage 2-4
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Stages of male genital and pubic hair development ( Marshall and Tanner)
Genital - Stage G1 Preadolescent Testes scrotum and penis
are about the same size and proportion as in early childhood
- Stage G2 The scrotum and testes have enlarged and there is a change in the texture and some reddening of the scrotal skin There is no enlargement of the penis
- Stage G3 Growth of the penis has occurred at first mainly in length but with some increase in breadth further growth of testes and scrotum
- Stage G4 Penis further enlarged in length and girth with development of glans Testes and scrotum further enlarged The scrotal skin has further darkened
- Stage G5 Genitalia adult in size and shape No further enlargement takes place after stage G5 is reached
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Pubic hair - Stage P1 Preadolescent The vellus is no further developed
than that over the abdominal wall ie no pubic hair
- Stage P2 Sparse growth of long slightly pigmented downy hair straight or only slightly curled appearing chiefly at the base of the penis This is subtle
- Stage P3 Hair is considerably darker coarser and curlier and spreads sparsely
- Stage P4 Hair is now adult in type but the area it covers is still considerably smaller than in most adults There is no spread to the medial surface of the thighs
- Stage P5 Hair is adult in quantity and type distributed as an inverse triangle Spread is to the medial surface of the thighs but not up the linea alba Most men will have further spread of pubic hair
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
DELAYED PUBERTY OR ABSENT PUBERTY
(Sexual Infantilism)
- Any girl of 13 or boy of 14 years of age with no signs of pubertal development falls more than 25 SD below the mean and is considered to have delayed puberty
- By this definition 06 of the healthy population are classified as having constitutional delay in growth and adolescence
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
CASE
bull This is a 15 year old boy who is seen by his primary care physician for short stature and delayed sexual development His past medical history is unremarkable except for asthma during early childhood which has been well controlled He is currently on no medications He is an average student currently in the 9th grade and is the smallest in his class He has been harassed by older classmates because of his size His parents are concerned because Jim is becoming withdrawn and a loner
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull Females initially show a deposition of adipose tissue and widening of the pelvis and changes in the contour of the hips The first clinical sign is thelarche (the appearance of breast buds) and adrenarche (the appearance of dark straight pubic hair over the mons veneris also called the mons pubis)
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull These changes identify a SMR stage (or Tanner stage) II (see tables 1 and 2) Breast development over the next 4 years will proceed from breast stage II (secondary mound of breast tissue to adult breast stage V) Development of pubic hair starts about 1 year after breast budding and may take place over a 15 to 35 year period
bull During SMR stage 3 girls experience a very rapid increase in their height The peak of their height growth (PHV=peak height velocity) should take place before the onset of menarche in most girls Menarche occurs six months after the PHV and just prior to stage IV of breast development Most western girls achieve their menarche around 124 to 128 years of age African-American girls are maturing earlier
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull Puberty in boys also follows a regular sequence of events but lacks the clear cut landmarks such as breast development and menarche In the male the pubertal growth spurt is a late event starting about two years later than in females The onset of pubertal changes however are only about 6 months later than in females (see tables 2 and 3) Enlargement of the testes indicates the transition from genital stage I to Stage II beginning at an average age of 115 years
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull Penile growth occurs about one year later This is usually preceded by the appearance of pubic hair at the base of the phallus progressing through pubic hair stages II to V Pubic hair stage III is followed by the appearance of axillary and facial hair growth Testicular growth is completed anytime between 135 and 17 years of age Growth of the penis reaches a SMR (Tanner) stage V between 125 and 165 years of age Nocturnal emissions (wet dreams) may first appear during SMR stage III
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull There is a common misconception that the difference between the onset of puberty in males and females is 2 years This applies only to the growth spurt and not to pubertal (SMR) changes
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull The patient described above is not only short statured but is delayed in his pubertal development On the basis of the physical findings described he would fit a presumptive diagnosis of constitutional delay of growth and maturation
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull Boys with a constitutional delay of growth and maturation usually have a normal birth weight and length and progress along their normal growth centile for the first several years of life following which they begin to deviate and grow at or below the 3rd percentile throughout childhood
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull At the time when normal puberty should begin there is often a marked fall off in growth (pre-adolescent dip) due to a diminished secretion of growth hormone This transient fall in growth hormone is probably due to failure of sex hormone production and stimulation
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull Skeletal maturation is usually delayed When the bone age eventually reaches the skeletal age when puberty is expected it is likely that early signs of sexual maturation will also appear which is the stage of testicular enlargement (SMR genital stage II)
bull
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull Often a familial pattern of pubertal delay is reported The incidence of affected males is about 10 Patients with constitutional delay in growth and maturation usually do not reach their mid parent or predicted height Catch up growth is largely dependent upon the delay of bone maturation at the time of diagnosis indicating that there may be a genetic or familial component to their short stature
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull In most males a watch and wait approach is indicated for six to twelve months The patient presented could have been prescribed a short term course of testosterone or gonadotropins in order to stimulate sexual maturation and growth hormone production In general such treatment has been reserved for teenagers with significant behavior or psychological (self image) problems due to their delayed puberty
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull In most cases the evaluation of a patient suspected of delayed sexual maturity can be conservative A thorough family history physical examination and assessment of sexual maturity stage will often show signs of early pubertal changes The bone age is usually delayed and reflects the physical delays and the height age of the patient (the age corresponding to the 50ile of the patients actual height)
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull Gonadotropins usually reflect the sexual maturity status of the patient A chromosomal karyotype is indicated for all short statured girls who are delayed (for possible Turner syndrome) and for boys who are tall with small soft testes with or without delayed sexual maturity (Klinefelters syndrome)
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Causes of Short Staturebull I Constitutional Short Staturebull II Primordial Dwarfism (intrauterine growth retardation)bull III Endocrine Causesbull A Growth Hormone Deficiencybull 1 Congenitalbull 2 Acquiredbull a HypothalamicPituitary Tumorsbull b Head Traumabull c CNS infectionsbull d Psychosocial Dwarfismbull 3 Laron Dwarfismbull 4 Hypothyroidismbull 5 Syndromes of Short Staturebull a Turner Syndrome (gonadal dysgenesis)bull b Noonans Syndromebull c Prader-Willi Syndromebull
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Causes of Short Stature
bull IV Chronic Diseasebull A Heart Diseasebull B Pulmonarybull 1 Cystic Fibrosisbull 2 Asthmabull C GI Disordersbull D Hepatic Diseasebull E Renalbull V Iatrogenicbull A Corticosteroids anabolic steroidsbull B ADHD meds
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
bull Causes of Delayed Pubertybull I Constitutional Delay in Growth and Maturationbull II Hypogonadotropic hypogonadismbull A Central nervous system disordersbull 1 Tumors bull a Craniopharyngiomasbull b Gliomasbull c Germinomasbull 2 Radiation Therapybull 3 Congenital Malformationsbull
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Causes of Delayed Puberty
bull B Isolated Growth Hormone Deficiencybull 1 Kallmanns Syndromebull C Miscellaneous Disordersbull 1 Prader-Willi Syndromebull 2 Hypothyroidismbull 3 Malnutritionbull 4 Anorexia Nervosabull 5 Exercise amenorrheabull 6 Cushingsbull 7 Diabetesbull
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Causes of Delayed Puberty
bull III Hypergonadotropic hypogonadism
bull A Turner Syndrome
bull B XX and XY gonadal dysgenesis
bull C Polycystic ovary Syndrome
bull D Noonans Syndrome
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Classification of delayed puberty - Constitutional delay in growth and adolescence Hypogonadotropic hypogonadism - Central nervous system disorders - Tumors- Other acquired disorders- Congenital disorders - Isolated gonadotropin deficiency- Kallmanns syndrome- Gonadotropin deficiency with normal sense of smellMultiple pituitary hormonal
deficiencies- Miscellaneous disorders - Prader-Willi syndrome- Laurence-Moon Bardet-Biedl syndromes- Chronic disease- Weight loss- Anorexia nervosa- Increased physical activity in female athletes- Hypothyroidism- Hypergonadotropic hypogonadism - Males Klinefelters syndrome Other forms of primary testicular failureAnorchia or
cryptorchism- Females Turners syndromeOther forms of primary ovarian failure Pseudo-Turners
syndrome- Noonans syndrome- XX and XY gonadal dysgenesis
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
PRECOCIOUS PUBERTY (Sexual Precocity)
- The appearance of secondary sexual development before the age of 7 years in Caucasian girls and 6 years in African-American girls 9 years in boys of either race constitutes precocious sexual development
- When the cause is premature activation of the hypothalamic-pituitary axis the diagnosis is complete (true) precocious puberty
- If ectopic gonadotropin secretion occurs in boys or autonomous sex steroid secretion occurs in either sex the diagnosis is incomplete precocious puberty
- In all forms of sexual precocity there is an increase in growth velocity somatic development and skeletal maturation
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Classification of precocious puberty - Central (complete or true) isosexual precocious puberty - Constitutional Idiopathic Central nervous system disorders Following androgen exposure Incomplete isosexual precocious puberty - Males Gonadotropin-secreting tumors Excessive androgen production Premature Leydig and germinal cell maturation- Females ovarian cysts Estrogen-secreting neoplasms- Males and females Severe hypothyroidism McCune-Albright syndrome Sexual precocity due to gonadotropin or sex steroid exposure
Variation in pubertal development - Premature thelarche- Premature menarche- Premature pubarche- Adolescent gynecomastia
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
Boy 2512 years of age with idiopathic true precocious
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development
13412-year-old girl with constitutional delay in growth and puberty History normal growth rate but short stature at all ages Physical examination height of 138 cm (-45 SD) and a weight of 286 kg (-3 SD) The patient had early stage 2 breast development There was no pubic hair Karyotype was 46XX Bone age was 10 years After administration of GnRH LH and FSH rose in a pubertal pattern Estradiol was 40 pgmL She has since spontaneously progressed through pubertal development