PEDIATRICS BOARD REVIEWEDWARD M. SANTOS, MDDEPARTMENT OF PEDIATRICSUERMMMC

1. WHICH OF THE FOLLOWING IS NOT A COMPONENT OF TETRALOGY OF FALLOT?

a.Atrial septal defectb.Ventricular septal defectc.Pulmonary stenosisd.Overriding aorta

TETRALOGY OF FALLOT

TOF Dyspnea is relieved by squatting Paroxysmal hypercyanotic attacks (hypoxic,

“blue”, or “tet” spells Place infant on the abdomen in knee chest position Oxygen Morphine sq not in excess of 0.2mg/k NaHCO3 if spell is unusually severe Other meds: IV phenylephrine, propranolol

Systolic thrill and murmur On CXR- boot or wooden shoe (coeur en sabot) Blalock-Taussig shunt (subclavian and ipsilateral

PA) and Gore-Tex interposition shunt TAPVR - Snowman appearance

2. A 6-MONTH OLD BABY WAS BROUGHT TO THE ER BECAUSE OF DOB. ON PE, SHE WAS TACHYPNEIC,, WITH RALES AND NOTE OF A GR 3/6 HOLOSYSTOLIC MURMUR ON THE LLSB. CXR SHOWED CONGESTION AND CARDIOMEGALY LV FORM. ABG SHOWED PO2 OF 175 OFF OXYGEN. THE MOST LIKELY CARDIAC LESION IS:

a.Pulmonary stenosisb.Atrial septal defectc.Ventricular septal defectd.Transposition of the great arteries

RECALL Congenital Heart Diseases Acyanotic vs Cyanotic Acyanotic

Increased volume load L R shunting (ASD, VSD, PDA, AV septal

defects) Increased pressure load

Normal blood flow is obstructed (pulmonic stenosis, etc)

Cyanotic Decreased pulmonary blood flow

Obstruction and a shunt (ex. TOF) Increased pulmonary blood flow

Not associated with obstruction but with abnormal mixing of systemic venous and pulmonary venous blood ( ex TGA)

RECALL

No cyanosis (+) congestion Increased volume load decreased

pulmonary compliance increased work of breathing

Acyanotic CHD with increased volume load VSD

Tools in the Assessment of Heart Disease

FIVE BASIC QUESTIONS

1. Is the patient cyanotic or acyanotic?2. Is the pulmonary arterial flow increased or not ?3. Does the malformation originate in the

left or right side of the heart?4. Which is the dominant ventricle? 5. Is pulmonary hypertension present or

not ?

HISTORYPHYSICAL EXAM

CHEST X- Ray

EKG (15 lead)

Others: 2D EchoCardiac cath

PHYSIOLOGIC CLASSIFICATION OF CONGENITAL HEART DISEASE

CYANOTICCYANOTICACYANOTICACYANOTIC

Increased PBF

Normal orDecreased PBF

No SHUNT

RVH LVH

PS AS

With SHUNT

RVH LVH

ASD VSD/PDAAP- Window

Increased PBF

Decreased PBF

RVH BVH

TGATAPVR

TRUNCUS

RVH LVH

TOF/DORVEBSTEIN

PVA

VENTRICULAR SEPTAL DEFECT

Most common form of CHD (25% of CHDs) Most defects are of the membranous type Size of the VSD is a major determinant of the

LR shunt Restrictive VSDs= < 0.5cm2, RV pressure is

N, shunt is LR Non restrictive VSDs >1.0 cm2 RV and LV

pressure is equalized

ATRIAL SEPTAL DEFECT

Most common form is the ostium secundum defect in the region of the fossa ovalis

Other forms: primum and sinus venosus defects

Infants and children with ASDs are usually asymptomatic

Widely split and fixed S2 Gr 2-3/6 SEM

PATENT DUCTUS ARTERIOSUS

Common problem in premature infants Left PA and the descending aorta If smallasymptomatic If large tachycardia and exertional dyspnea Dynamic precordium, systolic thrill at the

ULSB, bounding peripheral pulses with wide PP

Indomethacin

CLOSURE OF THE DUCTUS ARTERIOSUS Functional closure occurs by constriction of

the medial smooth muscles in the ductus : occurs 10 -15 hours after birth

Anatomic closure completed in 2-3 weeks of life by permanent changes in the endothelium and subintimal layers of the ductus

FACTORS THAT MAY FAVOR CLOSURE OF THE DUCTUS ARTERIOSUS Oxygen - strongest stimulus for

constriction of smooth muscles

Decrease Prostaglandin E2 levels

Responsiveness of the ductal smooth muscle to oxygen for closure depends on :gestational age of the newborndoes not depend on the lack of smooth muscle

development

3. THE FOLLOWING ARE ANTI-HEART FAILURE MEDICATIONS, EXCEPT:

a.Dopamineb.Furosemidec.Phenytoind.Digoxin

CONGESTIVE HEART FAILURE Results from CHDs or acquired heart diseases Volume and pressure overload or from

myocardial insufficiency Treatment

Elimination of precipitating causes Control of heart failure (meds)

Drug Therapy Diuretics (rapid acting like furo) Digitalis glycosides (Digoxin) Other Inotropic agents (Dopamine) Afterload reducing agents (hydralazine,

nitroglycerine, captopril, enalapril, nitroprusside)

4. A 10 YEAR OLD WENT TO YOU FOR CONSULT DUE TO DIFFICULTY IN WALKING. ON PE, YOU NOTE THAT HE IS ALMOST PULSELESS. YOU SUSPECT:

a. Juvenile Rheumatoid Arthritisb. Takayasu Arteritisc. Panarteritis nodosad. Systemic lupus

erythematosus

CLUE: PULSELESSNESS

Differentials Coarctation of the aorta

Femoral pulses are weak and delayed or absent Vasculitis syndromes

Vasculitis syndromes HSP, Takayasu arteritis, PAN Takayasu – involves large arteries, pulseless

disease PAN- small to medium sized arteries, presents

with fever Difficulty in walking – could be due to arthritis

5. WHICH OF THE FOLLOWING IS THE EXCEPTION TO FULFILLING JONES CRITERIA IN ORDER TO DIAGNOSE RHEUMATIC FEVER

a. Subcutaneous nodulesb. Migratory polyarthritisc. Endocarditisd. Chorea

JONES CRITERIA 5 major

Carditis, polyarthritis, erythema marginatum, subcutaneous nodules, chorea

Chorea may occur as the only manifestation of acute RF

4 minor Arthralgia, fever, elevated ESR, elevated CRP,

prolonged PR interval Evidence of recent strep group A infection

(+) throat culture Elevated ASO

JONES: 2 major or 1 major + 2 minor, with an evidence of recent strep infection

6. WHICH OF THE FOLLOWING IS NOT AN INDICATION FOR TONSILLECTOMY?

a. Obstructive sleep apnea due to hypertrophic adenoids and tonsils

b. Severe rheumatic fever/rheumatic heart disease manifestation

c. To rule out tumord. 5 infections in each of the last 2

years for chronic tonsillitis patients

INDICATIONS FOR TONSILLECTOMY (CHILDREN’S HOSPITAL OF PITTSBURG)

7 or more throat infections treated with antibiotics in the preceding year

5 or more throat infections treated in each of the preceding 2 years

3 or more throat infections treated in each of the preceding 3 years

8. A PX COMES IN WITH TRISMUS, DIFFICULTY OF SWALLOWING AND “HOT POTATO” VOICE. YOUR DX IS

a. Retropharyngeal abscessb. Croupc. Peritonsillar abscessd. Epiglottitis

NECK ABSCESSES

Retropharyngeal and Lateral pharyngeal abscess Common in young children 3-4 years old or younger Fever , irritability, decreased oral intake and

drooling Bulging posterior pharyngeal wall polymicrobial

Peritonsillar cellulitis/abscess Relatively common Older child, adolescent Sore throat, fever, trismus, dysphagia Asymmetric tonsillar bulge with displaced uvula “hot potato” voice

CROUP VS EPIGLOTITIS Croup – viral (parainfluenza),; low grade

fever, non toxic; no distress; responds to racemic epinephrine and dexamethasone ; “steeple sign”

Epiglotitis- bacterial (H influenza); life-threatening; high fever, drooling, toxic child, acute onset; sitting forward; critical airway; “thumb sign”

9. A LANKY TEENAGER WENT TO THE ER DUE TO SUDDEN ONSET DYSPNEA. HE HAS RETRACTIONS AND DECREASED BREATH SOUNDS OVER THE INVOLVED AREA; AND ON CXR THERE IS A MEDIASTINAL SHIFT TOWARDS THE UNINVOLVED SIDE. THE MOST LIKELY DIAGNOSIS IS

a. Pneumoniab. Spontaneous pneumothoraxc. Lung tumord. Atelectasis

PNEUMOTHORAX Accumulation of extrapulmonary air within the chest Primary vs secondary Spontaneous, traumatic, iatrogenic or catamenial Spontaneous pneumothorax – common among young

adults and teenagers who are tall and thin On CXR – evidence of tension includes shift of

mediastinal structures away from the side of the air leak

Treatment Small (<5%) or moderate – resolves (1 week) 100% oxygen hastens resolution Analgesics Chest tube drainage Chemical pleurodesis (talc, doxycycline,

iodopovidone)

10. TRUE ABOUT EPIGLOTITTIS

a. Rapidly progressive respiratory obstruction

b. Etiologic agent: Haemophilus influenza b

c. A medical emergencyd. All of the above

EPIGLOTITIS bacterial (H influenza) life-threatening high fever, drooling, toxic child acute onset sitting forward critical airway “thumb sign”

11. WHICH OF THE FOLLOWING INFECTIONS PRESENT AS PHARYNGEAL INJECTION, WITH TONSILLAR MEMBRANE AND “ BULL NECK” APPEARANCE?

a. Diphtheriab. Pertussisc. Tetanusd. Polio

CLUE: BULLNECK AND THE MEMBRANE

Caused by Corynebacterium diphtheriae 62 k polypeptide exotoxin Pseudomembrane Bull neck appearance – underlying soft tissue

edema and enlarged lymph nodes Toxic cardiomyopathy – 50-60% of deaths Tx: antitoxin, antibiotics (erythro and

penicillin)

12. GOLD STANDARD IN THE TREATMENT OF UNCOMPLICATED SALMONELLOSIS

a. Penicillinb. Ceftriaxonec. Aspirind. Chloramphenicol

SALMONELLOSIS

Nontyphoidal salmonellosis Acute enteritis, bacteremia, extraintestinal focal

infections Tx: antibiotics for bacteremia and extra

intestinal infections (ceftriaxone) For acute enteritis: no antibiotics, only for infants

<3 mos, malnourished, immunocompromised Enteric or typhoid fever

S. Ser Typhi, Paratyphi A, Paratyphi B, Paratyphi C

Relative bradycardia, rose spots Dx: bone marrow culture Tx: Chloramphenicol remains the gold standard

13. WHICH IS CONSIDERED AS POSITIVE PPD?

a. Below age 5 years with BCG > 5 mm

b. 5 years and above: > 5 mmc. 7 years and above : > 10 mmd. 7 years and above : > 12 mm

Mantoux test 5 TU PPD-S read at 48-72 hrs Induration of >5mm is considered positive in

the presence of any of the following: history of a close contact with a known or suspected infectious case of TB, clinical findings suggestive of TB, cxr suggestive of TB and immunocompromised condition

Otherwise, >10mm is considered positive

POSITIVE PPD

INDURATION > 5 MM Close contacts of TB pxs, immunocompromised,

those with clinical and radio findings INDURATION > 10 MM

Infants and children <4 yrs, those with underlying medical conditions, frequently exposed to high risk adults

INDURATION > 15 MM > 4yrs old with no risk factors

14. WHICH OF THE FOLLOWING ANTITUBERCULOSIS MEDICATIONS IS CONTRAINDICATED FOR CHILDREN BELOW 7 YEARS OLD?

a. Rifampicinb. Pyrazinamidec. Streptomycind. Ethambutol

TB In Infancy and Childhood Diagnosis: if 3 or more of the following

criteria are presentExposure to an adult/adolescent with active TB

diseaseSigns and symptoms suggestive of TBPositive tuberculin testAbnormal chest radiograph suggestive of TBOther lab finings suggestive of TB (histological,

cytological, biochemical, etc)

Signs and Symptoms Cough/ wheezing of 2 weeks or more Unexplained fever of 2 weeks or more Loss of appetite, loss of weight, failure to

gain weight Failure to respond to 2 weeks of appropriate

antibiotics Failure to gain previous state of health after

two weeks of a viral infection or exanthem Fatigue, reduced playfullness, or lethargy

Mantoux test 5 TU PPD-S read at 48-72 hrs Induration of >5mm is considered positive in

the presence of any of the following: history of a close contact with a known or suspected infectious case of TB, clinical findings suggestive of TB, cxr suggestive of TB and immunocompromised condition

Otherwise, >10mm is considered positive

Clinical Forms of TB Pulmonary/Intrathoracic TB

Primary DiseaseProgressive primary diseasePleurisy with effusionEndobronchial Tbchronic pulmonary TBMiliary TBTuberculomaPericardial TB

Extrapulmonary/ExtrathoracicScrofula, TB adenitis, CNS TB, etc

Management of TB Intensive Phase (2 mos) Continuation Phase (4 mos) DOTS : decrease the risk of infection, reduce

morbidity and the transmission of infection, prevent TB deaths

Anti TB drugs INH: 10-15mkdRifampicin: 10-20mkdPyrazinamide: 20-40mkdStreptomycin: 20-40mkdEthambutol: 15-25

ANTI TB MEDS

Isoniazid Peripheral neuritis and hepatotoxicity

Rifampicin Orange discoloration of urine and tears,

thrombocytopenia and influenza like syndrome Pyrazinamide

hyperuricemia Streptomycin

Hearing loss Ethambutol

Optic neuritis and red-green color blindness Not recommended for use in children

15. WHICH OF THE FOLLOWING VIRAL INFECTIONS PRESENTS WITH HIGH FEVER WHICH ABRUPTLY LYSES AND DISCRETE PINK, RAISED , NON-PRURITIC RASH ON THE TRUNK WHICH SPREADS TO THE NECK, FACE AND PROXIMAL EXTREMITIES?

a. Rubellab. Roseolac. Rubeolad. Mumps

ROSEOLA INFANTUM Exanthem subitum or 6th disease Recall: 1st – measles, 2nd scarlet, 3rd rubella,

4th atypical scarlet( Filatov-Duke’s), 5th erythema infectiosum

Human herpesvirus 6 and 7 ( more of 6) Nagayama spots – ulcers at the

uvulopalatoglossal junction Primary HHV 6 infxn – 1/3 of febrile seizure

cases

16. WHICH IS THE VECTOR OF DENGUE FEVER?

a. Anopheles mosquitob. Culex mosquitoc. Aedes aegypti mosquitod. Aedes africanus

Aedes aegypti Flight range: < 25 m in open, urban environm’t

Dispersal: 30-50 m/d

Visits not more than 2-3 houses

Remains infective during lifetime

High humidity, longer mosquito survival

Ave lifespan: 8-15 d

17. MOST SEVERE FORM OF MALARIA, PRESENTING WITH INTENSE PARASITISM

a. Malariaeb. Falcifarumc. Vivaxd. Ovale

MALARIA P. falcifarum is the most severe form and is

associated with more intense parasitemia P. Vivax – less severe; ruptured spleen P. malariae is the mildest and most chronic P. Vivax and P. Ovale – daily fever spikes P. Malariae – every other or third day fever

spikes Periodicity of fever- less apparent in

falciparum Tx; chloroquine; for chloroquine resistance-

quinine Blackwater fever, algid malaria

18. DRUG OF CHOICE FOR PREVENTION OF RELAPSES OF P. VIVAX AND P. OVALE INFECTION

a. Quinineb. Chloroquinec. Primaquined. Pyrimethamine –sulfadoxime

RELAPSE

Common in P. Vivax, ovale and malariae

Caused by release of merozoites from an exoerythrocytic source in the liver (vivax an ovale) or persistence within the erythrocyte (malariae)

Primaquine OD x 14 days

19. WHICH OF THE FOLLOWING INFESTATIONS IS NOT DETECTED BY DIRECT FECAL SMEAR?

a. Enterobiasisb. Trichuriasisc. Hookwormsd. Diphyllobotriasis

HELMINTHIC DISEASES

Direct fecal examAscaris, hookworms, trichuriasis,

strongyloides, diphyllobotriasis Microscopic exam of cellophane tape -

enterobius Demonstration in the blood - filariasis Stool exam by Kato thick smear -

schistosomiasis

20. WHICH OF THE FOLLOWING VACCINES IS PART OF THE EPI?

a. Tetanus toxoidb. Varicella vaccinec. Rotavirus vaccined. Typhoid vaccine

EXPANDED PROGRAM ON IMMUNIZATION

BCG HEPATITIS B DPT POLIO MEASLES RECENT INCLUSIONS: HiB, MMR, ROTAVIRUS

21. WHAT IS THE MOST COMMON HEMATOLOGIC DISEASE OF INFANCY AND CHILDHOOD?

a. Acute leukemiab. Iron deficiency anemiac. Idiopathic thrombocytopenic

purpurad. Hereditary spherocytosis

Most common hematologic disease of infancy and childhood

Newborn – contains 0.5g of iron Adult – 5g Iron stores in term infants are sufficient for

blood formation in the first 6-9 months Therefore, it is unusual to have anemia due

to inadequate dietary iron before 6 months (usually occurs around 9-24 months)

Chronic iron def : peptic ulcer, Meckel, polyp, hemangioma, or inflammatory bowel disease

Hookworm infestation

IRON DEFICIENCY ANEMIA

22. IN PHYSIOLOGIC ANEMIA OF INFANCY, HEMOGLOBIN DECLINES AT ____ WEEKS FOR TERM INFANTS

a. 3-6b. 10-12c. 6-8d. 2-4

PHYSIOLOGIC ANEMIA OF INFANCY

Newborns have higher Hgb and Hct Within the 1st week, Hgb starts to decrease

and may persist for 6-8 weeks Increase in blood oxygen content and tissue

oxygen delivery downregulates erythropoietin (EPO) production

Peak: 8-12 weeks (Hgb 9-11) Prematures: more exaggerated (3-6 wks, Hgb

7-9)

23. WHICH OF THE FOLLOWING PRODUCES PERIPHERAL PANCYTOPENIA WITH HYPOPLASTIC MARROW?

a. Acute leukemiab. Iron deficiency anemiac. Idiopathic thrombocytopenic

purpurad. Aplastic anemia

Aplastic Anemia

Hallmark is peripheral pancytopenia, coupled with hypoplastic or aplastic bone marrow

Severe aplastic anemia – 2 or more cell components have become seriously compromised (ANC < 500/mm3, PC 20,000-100,000/mm3, retic count <1% after correction for hematocrit)

Moderate aplastic anemia – ANC 500 -1,500/mm3, PC 20,000 -100,000 and retic count <1%

24. MOST COMMON EXTRACRANIAL TUMOR OF CHILDHOOD, MOST FREQUENTLY DIAGNOSED DURING INFANCY, AND MOSTLY ARISING FROM THE ABDOMEN

a. Wilm’s tumorb. Hepatoblastomac. Neuroblastomad. Nephroblastoma

CANCER AND BENIGN TUMORS Neuroblastoma

Most common extracranial solid tumor in children and the most commonly diagnosed malignancy in infants

Embryonal cancer of the peripheral SNS Median age of dx is 2 yr; 90% before 5 yr Most cases arise in the abdomen CT scan: calcification and hemorrhage Tumor markers: VMA and HVA

Wilm’s Tumor Also known as Nephroblastoma Most common primary malignant renal tumor of childhood;

2nd most common malignant abdominal tumor in childhood 3 elements: blastema, epithelia and stroma Associated with hemihypertrophy, aniridia, and other

congenital anomalies (WAGR, Denys-Drash, Beckwith-Wiedemann)

Occurs in children between 2-5 years old CT: no calcification

25. A 15-YEAR OLD CONSULTED FOR ARM SWELLING AND PAIN. X-RAY SHOWED SCLEROTIC DESTRUCTION OF THE PROXIMAL HUMERUS WITH SUNBURST PATTERN. THE MOST LIKELY DIAGNOSIS IS

a. Osteosarcomab. Ewing’s sarcomac. Rhabdomyosarcomad. Enchondroma

CANCER AND BENIGN TUMORS Bone Tumors

Feature Osteosarcoma Ewing’s

AgeRaceSex (M:F)CellPredispositionSitePresentationRadiographic findingMetastasisTxOutcome

Second decadeAll races1.5:1Spindle cellRetinoblastoma, Paget’sMetaphyses of long bonesLocal pain & swellingSunburst patternLungs, bonesChemotherapyWithout mets: 70% curedWith mets: < 20% survival

Second decadePrimarily whites1.5:1Small round cellNoneDiaphyses, flat bonesLocal pain &swellingOnion skinningLungs, bonesChemotherapyW/o mets: 60% curedWith mets: 20-30% survival

26. DEFICIENCY IN WHICH OF THE FOLLOWING FACTORS WILL YOU NEED TO GIVE VITAMIN K?

a. Factors II, V, VII, IXb. Factors V, VIII, IX, X, XIc. Factors II, VII, IX, Xd. Factors II, VIII, IX, X

27. A NEWBORN PRESENTED WITH “BLUEBERRY MUFFIN” RASH, CATARACTS, PULMONARY STENOSIS AND HEARING LOSS. YOU SUSPECT THAT THE BABY HAS

a. Congenital varicella syndrome

b. Neonatal herpes syndromec. Congenital syphillisd. Congenital rubella syndrome

CONGENITAL INFECTIONS

Congenital Syphilis Early and late signs Early – snuffles; late –Hutchinsons teeth, saber

shins, olympian brow Congenital varicella – malformations of the

extremities with cicatricial scarring Congenital rubella – cataracts, heart defects,

deafness, blueberry muffin lesions (same as in CMV)

Neonatal herpes

28. A BABY WAS BROUGHT TO YOUR CLINIC BECAUSE OF FAILURE TO THRIVE. HE HAD DELAYED PASSAGE OF MECONIUM AND CHRONIC CONSTIPATION. HE HAS ABOMINAL DISTENTION AND A PALPABLE MASS ON THE LLQ. THE RECTAL VAULT WAS EMPTY BUT A GUSH OF FOUL-SMELLING GAS WAS NOTED ON RECTAL EXAM. BARIUM ENEMA SHOWED A DILATED PROXIMAL COLON AND SMALL CALIBER DISTAL COLON. SHORT OF A BIOPSY, YOUR DIAGNOSIS IS

a. Intussusceptionb. Congenital aganglionic megacolonc. Hypertrophic pyloric stenosisd. Gastric volvulus

Congenital Aganglionic Megacolon (Hirschspung Disease) Most common cause of lower intestinal obstruction in

neonates Caused by abnormal innervation of the bowel

(absence of ganglion cells), beginning in the internal anal sphincter and extending proximally to involve a variable length of gut

Symptoms usually begin at birth with the delayed passage of meconium (In 99% of full term infants, meconium is passed within the first 48 hours)

Rectal exam demonstrates normal anal tone and is usually followed by an explosive discharge of foul smelling feces and gas

Rectal manometry and rectal suction biopsy Barium enema is useful in determining the extent of

aganglionosis Definitive treatment is operative intervention

29. PRECOCIOUS PUBERTY IS DEFINED AS ONSET OF PUBERTY BEFORE AGE 8 IN GIRLS AND __ IN BOYS

a. 8b. 9c. 10d. 11

PRECOCIOUS PUBERTY

Onset of secondary sexual characteristics before 8 years of age in girls and 9 years in boys

Conditions causing precocious pubertyGonadotropin dependent pubertyCombined gonadotropin dependent

and independent(ex. McCune –Albright)

Gonadotropin independentIncomplete

30. WHICH OF THE FOLLOWING HORMONES IS NOT FOUND IN THE ANTERIOR PITUITARY

a. Oxytocinb. Prolactinc. Leutinizing hormoned. Follicle stimulationg hormone

PITUITARY HORMONES

Anterior PituitaryGH, prolactin, TSH, ACTH, LH, FSH,

POMC Posterior Pituitary

Vasopressin and oxytocin

31. A 12 YEAR OLD MALE WAS BROUGHT IN FOR DEHYDRATION. HE HAS BEEN POLYURIC AND POLYDIPSIC FOR QUITE SOME TIME. HE HAS LOW URINE AND SERUM OSMOLALITY, AND HYPERNATREMIA. YOU SUSPECT DIABETES INSIPIDUS. TO DIFFERENTIATE BETWEEN CENTRAL AND NEPHROGENIC ORIGIN, YOU DO:

a. Insulin challenge testb. DDAVP testc. Erythropoietin testd. Beta agonist challenge

DIABETES INSIPIDUS Polyuria, polydipsia (exceeding 2L/m2/24hr),

hypernatremia Serum osmolality >300 mOsm/kg and urine

osmolality <300 mOsm/kg Central vs nephrogenic Central DI

Genetic mutations to the vasopressin gene, trauma to vasopressin neurons, congenital malformations of the hypothalamus or pituitary, neoplasms, infections, autoimmune diseases, inc vasopressin metabolism

Nephrogenic DI Vasopressin insensitive Genetic (less common but more severe) or

acquired causes

32. A KNOWN INSULIN DEPENDENT DIABETIC COMES TO YOUR CLINIC AND COMPLAINS OF EARLY MORNING HYPERGLYCEMIA. YOU SUSPECT BRITTLE DIABETES. YOU MEASURE HIS 3, 4, AND 7 AM GLUCOSE CONCENTRATION. THE 3 AND 4 AM LEVELS ARE BELOW 60 MG/DL, WHILE THE 7 AM IS MARKEDLY HIGH. THE PX IS EXPERIENCING

a. Diabetic ketoacidosisb. Insulin shockc. Somogyi phenomenond. Dawn phenomenon

DIABETES MELLITUS

Somogyi phenomenon Theoretical rebound from late night or early

morning hypoglycemia Exaggerated counterregulatory response rare

Dawn phenomenon Due to overnight GH secretion and inc insulin

clearance Usually recurrent and modestly elevates morning

levels Brittle diabetes

Unexplained wide fluctuations in glucose levels Usually an adolescent female with recurrent DKA

taking large doses of insulin

33. A NEIGHBOR SHOWS YOU HER 3-WEEK OLD BABY FOR CONSULT. SHE TELLS YOU THAT THE BABY IS SLUGGISH, DOES NOT FEED WELL AND IS CONSTIPATED. YOU NOTE THAT SHE IS JAUNDICED WITH A GLAZED LOOK, ENLARGED ABDOMEN, UMBILICAL HERNIA, AND MOTTLED SKIN. WHICH OF THE FOLLOWING LABORATORY EXAMS WILL YOU PRIORITIZE TO HELP YOU WITH YOUR DIAGNOSIS?

a. Chest x-rayb. Lumbar tapc. Serum T3, T4, TSHd. Serum Na, K, Ca

CONGENITAL HYPOTHYROIDISM Most common cause is thyroid dysgenesis (80-

85%); 15% - inborn error of thyroxine synthesis Detected by newborn screening F>M May be normal at birth (but head size may be

slightly increased due to myxedema of the brain)

Prolongation of physiologic jaundice – earliest sign

Other findings: feeding difficulties, sluggishness, lack of interest, somnolence, choking spells during nursing, constipation, large abdomen, umbilical hernia

Low serum T4 and elevated TSH levels Tx: levothyroxine

34. YOUR ADOLESCENT NIECE TALKS TO YOU ABOUT HER HEIGHT. SHE IS VERY TALL (90TH PERCENTILE), AND ASKS IF THERE IS SOMETHING WRONG WITH HER. HER PARENTS AND SIBLINGS ARE ALL TALL, AND SHE IS IN EXCELLENT HEALTH. WHAT WILL YOU DO?

a. Assure her that she is normalb. Have x-rays done to check her

epiphysisc. Get her growth hormone levelsd. Have her undergo cranial CT scan

35. A WEEK OLD BABY WAS BROUGHT TO THE ER BECAUSE OF WEAKNESS. SHE HAS BEEN VOMITING AND FEEDING POORLY. HER LAB EXAMS ARE UNREMARKABLE EXCEPT FOR SEVERE METABOLIC ACIDOSIS. YOU TELL THE ATTENDING PHYSICIAN THAT WHILE DOING YOUR PE, YOU NOTED THAT HER URINE SMELLED SWEET, LIKE MAPLE SYRUP. YOU THEN ASK FOR AMINOACID ASSAY, AND EXPECT THE FOLLOWING RESULT:

a. High leucine, isoleucine and valine levels and low alanine levels

b. High isovaleric acid, isovaleryglycerine and hydroxyvaleric acid levels

c. High methylmalonyl acid levelsd. High glutamine and alanine levels

INBORN ERRORS OF METABOLISM ASSOCIATED WITH ABNORMAL ODOR

Inborn Error of Metabolism Urine Odor

Glutaric Acidemia (Type III) Sweaty feet, Acrid

Hawkinsinuria Swimming pool

Isovaleric Acidemia Sweaty feet, Acrid

Maple Syrup Urine Disease Maple syrup

Hypermethioninemia Boiled cabbage

Multiple carboxylase deficiency Tomcat urine

Phenylketonuria Mousy or musty

Trimethylaminuria Rotting fish

Tyrosinemia Boiled cabbage, rancid butter

MAPLE SYRUP URINE DISEASE

DiagnosisPeculiar odor of maple syrupElevated levels of branched chain amino

acids (ILV) Treatment

Aim: rapid removal of branched chain amino acids and their metabolites

DietHydrationDialysis

36. A NEWBORN WAS BROUGHT TO THE ER FOR SEIZURES. SHE WAS BORN AT HOME AND A SHARPENED BAMBOO STICK WAS USED TO CUT THE CORD. THE MOTHER DID NOT HAVE PRENATAL CHECK-UP AND HER IMMUNIZATION STATUS WAS UNKNOWN. YOUR DIAGNOSIS IS NEONATAL TETANUS. THE DRUG OF CHOICE ISa. Penicillinb. Metronidazolec. Ceftriaxoned. Chloramphenicol

NEONATAL TETANUS Most common form of tetanus An acute spastic paralytic illness caused by the

neurotoxin (tatanospasmin) produced by Clostridium tetani

Tetanospasmin – 2nd most poisonous substance known (surpassed in potency only by the botulinum toxin)

Manifestations In infants – progressive difficulty in feeding, associated

hunger and crying In older children- trismus (masseter muscle spasm or

lockjaw), sardonic smile ( intractable spasms of the facial and buccal muscles)

Treatment Human Tetanus Ig Penicillin G – antibiotic of choice; alternatives:

metronidazole, erythromycin, Tetracycline Surgery

37. A TEENAGER WAS BROUGHT TO THE ER FOR RESPIRATORY DISTRESS. HE WAS IMMEDIATELY INTUBATED. THE ILLNESS STARTED AS DIFFICULTY IN WALKING, THEN WEAKNESS OF THE UPPER TRUNK AND EXTREMITIES AND FINALLY FACIAL WEAKNESS. THE HX IS UNREMARKABLE EXCEPT FOR A NON SPECIFIC VIRAL ILLNESS 2 WEEKS AGO. PE SHOWS COMPLETE PARALYSIS WITH ABSENT TENDON REFLEXES. THE LIKELY DX IS

a. Bell palsyb. Transverse myelitisc. Spinal muscular atrophyd. Guillain-Barre Syndrome

GUILLAIN – BARRE SYNDROME Postinfectious polyneuropathy involving mainly motor

but sometimes also sensory and autonomic nerves Usually follows a nonspecific viral infection Weakness begins in the lower extremities and

progressively involves the trunk, the upper limbs, and finally the bulbar muscles (a pattern known as Landry ascending paralysis)

Proximal and distal muscles are involved relatively symmetrically

CSF – protein is elevated to more than 2x the upper limit of normal

Dissociation between high CSF protein and a lack of cellular response is diagnostic

Treatment: IVIG Alternative Tx: Plasmapheresis and

immunosuppressive drugs

38. MOST COMMON INFRATENTORIAL TUMOR IN CHILDREN

a. Medulloblastomab. Brainstem gliomac. Cerebellar astrocytomad. ependymoma

CANCER AND BENIGN TUMORS Brain Tumors in Children

Categories Juvenile pilocytic astrocytoma – MOST COMMON Medulloblastoma Diffuse astrocytoma Ependymoma craniopharyngioma

Location Infratentorial (43.2%) Supratentorial (40.9%) Spinal cord (4.9%) Multiple sites (11%)

Important! Within the 1st year –supratentorial 1-10 yr – infratentorial Cranial exposure to ionizing radiation – inc incidence

39. A NEWBORN IS SUSPECTED TO HAVE SEPSIS. LUMBAR TAP WAS DONE AS PART OF THE WORK-UP. CSF STUDIES SHOWED RBC=0, WBC 10/MM2, PROTEIN 10 MG/DL, GLUCOSE 60% OF SERUM GLUCOSE. THE PATIENT HAS:

a. TB meningitisb. Bacterial meningitisc. Normal CSFd. Viral meningitis

CEREBROSPINAL FLUID LP: L3-L4 or L4-L5 OP: 100 mm/Hg ( 60-180) Contraindications

Elevated ICPs/sx of pending cerebral herniationCritical illnessSkin infection at the siteThrombocytopenia (<20 x 109)

Normal CSFClear, wbc up to 5 (for NB up to15), no

rbcs, protein 10-40 mg/dl (120 in a neonate), glucose 60%

40. CRANIAL CT SCAN OF A CHILD WITH MULTIPLE CONGENITAL ANOMALIES SHOWED ABSENCE OF CONVOLUTIONS. THIS IS KNOWN AS

a. Porencephalyb. Lissencephalyc. Schizencephalyd. Holoprosencephaly

DISORDERS OF NEURONAL MIGRATION

LissencephalyAbsence of convolutions (agyria)

SchizencephalyPresence of unilateral or bilateral clefts

within the hemispheres Porencephaly

Presence of cysts or cavities within the brain

HoloprocencephalyDefective cleavage of the procencephalon

41. YOUR FRIEND TELLS YOU THAT HIS CHILD WAKES UP AROUND MIDNIGHT SCREAMING AND HYPERVENTILATING. THE CHILD GOES BACK TO SLEEP IN A FEW MINUTES, AND ACTS AS IF NOTHING HAPPENED IN THE MORNING. THE DIAGNOSIS IS

a. Night terrorb. Rage attackc. Choreoathetosisd. Nightmares

NIGHT TERRORS VS NIGHTMARES

NightmaresCommon and usually involve vivid,

scary or exciting events which the child can recall upon waking up

Night terrorsLess commonLast 10-15 minutes, during which

the child is not easily aroused and appears frightened or agitated

No recollection of the event

42. A CHILD COMPLAINS OF INTENSE PRURITUS OF THE SKIN, PARTICULARLY AT NIGHT. THERE ARE EXCORIATED, CRUSTING, AND SCALING PAPULES ON THE TRUNK, PARTICULARLY THE WAIST AREA, THE INTERDIGITAL SPACES AND FLEXOR AREAS. THE TREATMENT OF CHOICE IS

a. Lindaneb. Topical corticosteroidc. Selsun blued. Tretinoin

SCABIES

Caused by burrowing and release of toxic and antigenic substances by the female mite Sarcoptes scabiei var hominis

Most important factor that determines spread of scabies is the extent and duration of physical contact with an affected individual

Intense pruritus particularly at night Threadlike burrows- classic lessions Treatment: Permethrin 5% Additional therapies: Lindane 1% lotion or

cream and oral ivermectin

43. A NEWBORN’S WEIGHT GAIN IS APPROXIMATELY __ GMS/MONTH AT AGE 2-6 MONTHS

a. 300b. 400c. 500d. 600

WEIGHT GAIN DURING THE FIRST YEAR

0-2 MOS30 g/day

2-6 MOS20 g/day

6-9 MOS15 g/day

9-12 MOS12 g/day

44. THE FIRST PRIMARY TEETH TO ERUPT ARE

a. Upper central incisorsb. Lower central incisorsc. Upper lateral incisorsd. cuspids

CHRONOLOGY OF HUMAN DENTITION(PRIMARY)

AGES (MOS) AGES (MOS)

Maxillary Mandibular

Central incisors

6-8 5-7

Lateral incisors 8-11 7-10

Cuspids 16-20 16-20

First molars 10-16 10-16

Second molars 20-30 20-30

45. HANDEDNESS IS USUALLY ESTABLISHED AT AGE ___ YEARS

a. 2b. 3c. 4d. 5

Preschool Years

Between 2 and 5 years old Emergence of language and exposure of children

to an expanding social sphere Handedness is usually established at 3 y/o Bowel and bladder control; bedwetting up to age

4 in girls and 5 years in boys Piaget’s preoperational stage: magical thinking,

egocentrism and thinking that is dominated by perception, not abstraction

46. THE FIRST VISIBLE SIGN OF PUBERTY IN MALES IS

a. Voice changeb. Pubic hairc. Growth spurtd. Testicular enlargement

ADOLESCENCE

10-20 years old Early, middle and late adolescence Rapid changes in body size, shape,

physiology, and psychological and social functioning

In boys: testicular enlargement In girls: appearance of breast buds

47. A 3-YEAR OLD WAS BROUGHT TO YOU FOR EVALUATION OF DELAYED SPEECH. THE CHILD HAS REPETITIVE BEHAVIOR, NO EYE CONTACT, ALONE. HE IS SUSPECTED TO HAVE

a. Asperger disorderb. Autismc. Schizophreniad. Attention deficit disorder

PERVASIVE DEVELOPMENTAL DISORDERS

Autistic Disorder Asperger Disorder Childhood Disintegrative Disorder Rett Disorder Childhood Schizophrenia

Autism Develops before 36 months and is typically

diagnosable at 18 mos of age Qualitative impairment in verbal and non verbal

communication, in imaginative activity and in reciprocal social interactions

Males>females Poor eye contact, little symbolic play, repetitive

behaviors, limited joint attention or orienting to one’s name, and reliance on non verbal communication with delay in use of words

Autism

CHAT- checklist for autism in toddlers Intensive behavioral therapy before 3 yrs old

targeted toward speech and language development

Educational programming, behavior modification

Pharmacotherapy: SSRIs, clonidine

Asperger Disorder – no severe language impairments as compared to patients with autism

Rett Disorder – x-linked dominant disorder affecting girls exclusively; boys die at birth development is normal until about 1-2 years old

until motor and language development regress

48. YOU EXPECT A SEVEN MONTH OLD TO BE ABLE TO

a. Pull to standb. Sit without supportc. Walk aloned. Grasp object with thumb and

forefinger

MOTOR DEVELOPMENT

Dependent on brain maturitySequence : Gross: cephalocaudal

Fine : proximodistalWeakest correlation to IQ

GROSS MOTOR MILESTONESMean Age (mos)Mean Age (mos) MilestonesMilestones

33 Head controlHead control

55 Roll overRoll over

66 Sit with supportSit with support

88 Sit aloneSit alone

99 Pulls to stand holding onPulls to stand holding on

1111 Stand independentlyStand independently

1515 Walk aloneWalk alone

2424 Climb up and down stairsClimb up and down stairs

3636 Ride a trikeRide a trike

4848 Hop on one footHop on one foot

FINE MOTOR MILESTONESMean Age (mos)Mean Age (mos) MilestonesMilestones

3.5 3.5 Grasps rattleGrasps rattle

44 Reaches for objectsReaches for objects

44 Palmar grasp gonePalmar grasp gone

5.55.5 Transfers objects hand to handTransfers objects hand to hand

88 Pincer graspPincer grasp

1212 Turns pages of booksTurns pages of books

1313 ScribblesScribbles

1515 Builds tower of 2 cubesBuilds tower of 2 cubes

2222 Builds tower of 6 cubesBuilds tower of 6 cubes

49. XEROPHTHALMIA, NYCTALOPIA, AND PHOTOPHOBIA ARE CAUSED BY DEFICIENCY IN WHICH VITAMIN?

a. Ab. Bc. Cd. D

VITAMIN DEFICIENCIES

Vitamin A: photophobia, xerophthalmia, blindnessVitamin B: beriberi, fatigue, anorexia,

constipationRiboflavinNiacin: pellagraFolacin: megaloblastic anemiaCyanocobalamin: pernicious anemiaBiotin: dermatitis, seborrheaPyridoxine: irritability, convulsions

Vitamin C: scurvy and poor wound healingVitamin D: ricketsVitamin E: red blood cell hemolysisVitamin K: hemorrhage

50. WHICH OF THE FOLLOWING MINERAL WILL CAUSE MOTTLING OF THE TEETH WHEN TAKEN IN EXCESS?

a. Calciumb. Ironc. Magnesiumd. Fluoride

FLUOROSIS

Mottled enamel Results from systemic fluoride consumption

(>0.05mkd) during enamel formation Severe brownish discoloration and hypoplasia

– also seen with fluoride concentrations in the drinking water >5ppm

For prevention of dental caries – 1ppm Children < 6y/o : pea sized amount of

toothpaste Children < 2 y/o : “smear”

51. AN EMACIATED CHILD WAS BROUGHT TO THE ER FOR MEDICAL MANAGEMENT. HE HAS WRINKLED LOOSE SKIN, SLIGHTLY DISTENDED ABDOMEN, MUSCLE ATROPHY. HIS FACE LOOKS WIZENED DUE TO LACK OF SUBCUTANEOUS FAT. HIS EXTREMITIES ARE COOL, AND TEMP IS SUBNORMAL. THE DIAGNOSIS IS

a. Kwashiorkorb. Marasmusc. Pellagrad. Rickets

PROTEIN/ENERGY MALNUTRITION (PEM) Edematous (Kwashiorkor) and

Nonedematous (Marasmus) PEM Edematous: edema, psychomotor changes,

flag sign, flaky dermatosis, moonface, anemia, diarrhea

Marasmus: gross loss of subcutaneous fat, obvious muscle wasting, potbelly, winged scapula

Treatment of PEM: 3 phases First : stabilization, correction of dehydration,

antibiotics Second: Diet providing maintenance requirements of

protein and energy Third: Recovery diet- 150 cal/kg/d and 4g/kg/d of

protein

52. A CHILD WITH HYPOTONIA, SLANTED PALPEBRAL FISSURES, EPICANTHAL FOLD, SIMIAN CREASE, COMPLETE ATRIO-VENTRICULAR SEPTAL DEFECT, AND SHORT BROAD HANDS HAS

a. 7q23 deletionb. Trisomy 18c. Trisomy 21d. 22q11 deletion

TRISOMY 21 (DOWN SYNDROME) Incidence: 1/600-800 births Findings

Hypotonia Upward and slanted palpebral fissures and

epicanthic folds Speckled irises (Brushfield spots) Varying degrees of mental and growth

retardation Cardiac malformations Simian crease

53. PREMATURE BIRTH IS DEFINED AS LIVE BIRTH BEFORE __ WEEKS FROM THE FIRST DAY OF THE LAST MENSTRUAL PERIOD:

a. 39b. 38c. 37d. 36

54. VERY LOW BIRTHWEIGHT INFANTS WEIGH LESS THAN ____ GMS

a. 1,000b. 2,500c. 2,000d. 1,500

Perinatal Mortality - influenced by prenatal, maternal and fetal factors IUGR, asphyxia, placental insufficiency, severe

congenital malformations, early onset neonatal infections

Neonatal Deaths - associated with preterm birth and low birth weight and lethal congenital anomalies

Low Birth Weight LBW : 2500g or less VLBW: 1500g or less

55. DIFFERENTIAL DIAGNOSES FOR JAUNDICE IN THE FIRST 24 HOURS OF LIFE, EXCEPT:

a. Physiologic jaundiceb. ABO incompatibilityc. Sepsis d. Hemorrhage

HYPERBILIRUBINEMIA Newborns appear jaundiced when the bil level is >

7 mg/dl Physiologic hyperbilirubinemia

Onset > 24 hours Inc RBC production Inc enterohepatic circulation Defective uptake Decreased conjugation Decreased hepatic secretion

Pathologic Onset < 24 hrs Rise of >0.5 mg/dl/hr >8 days (term), 14 days (preterm) symptomatic

KERNICTERUS Bilirubin encephalopathy resulting from the

deposition of unconjugated (indirect) bilirubin in the basal ganglia and brainstem nuclei

> 25mg/dl, range: 21-50 Clinical features

Acute Phase 1: poor suck, hypotonia, seizures Phase 2: hypertonia of extensor muscles, opisthotonos,

retrocolis, fever Phase 3: hypertonia

Chronic 1st year : hypotonia, active deep tendon reflexes After 1st year: movement disorders, SNHL, upward gaze

KERNICTERUS Treatment of Hyperbilirubinemia

Phototherapy Photoisomerization Complication; loose stools, rash, overheating, dehydration,

bronze baby syndrome Intravenous Immunoglobulin Metalloporphyrins

Competitive enzymatic inhibition of the rate limiting conversion of heme protein to biliverdin by heme oxygenase

Exchange Transfusion double volume exchange transfusion

56. A CHILD WAS BROUGHT TO THE ER DUE TO EDEMA. IT STARTED ON THE FEET PROGRESSING TOWARDS THE THIGHT. THE EYES WERE NOTED TO BE PUFFY PARTICULARLY IN THE MORNING. THE MOTHER CANNOT RECALL ANY PRIOR ILLNESS. ON PE, YOU NOTICE SEVERAL HEALING WOUNDS ON THE LEGS, AND HIS BP IS HIGH. ON FURTHER PROBING, THE CHILD CONFESSES THAT HIS URINE IS COLA COLORED. YOUR PRIMARY DIAGNOSIS IS:

a. Nephrotic syndromeb. Renal tubular acidosisc. Acute glomerulonephritisd. IgA nephropathy

POST STREPTOCOCCAL GLOMERULONEPHRITIS

Most common in children age 5 to 12 years

Uncommon before the age of 3 Phases:

Latent: hematuria, edemaOliguric: edema, hypertension, azotemia,

oligoanuriaDiuretic: spontaneous voiding, clinical

improvementEarly convalescent: increased well being

POST-STREPTOCOCCAL GLOMERULONEPHRITIS Serochemical findings:

Decreased C3 Elevated ASO or anti DNAse B Hyperkalemia Normal CBC, may have dilutional anemia Elevated BUN and creatinine during the oliguric

phase Urinalysis: proteins, rbcs

ACUTE POSTSTREP GN

Acute onset of gross hematuria, edema, hypertension, and renal insufficiency

Follows infection of the throat or skin by nephritogenic strains of group A B-hemolytic strep

Kidneys are enlarged; on EM – there are “humps” on the GBM

Low C3 levels Edema is due to salt and water retention

58. THE MOST COMMON CAUSE OF IDIOPATHIC NEPHROTIC SYNDROME IS

a. Focal segmental sclerosisb. Membranous nephropathyc. Membranoproliferative GN type 1d. Minimal change nephrotic

syndrome

NEPHROTIC SYNDROME

Heavy proteinuria , hypoalbuminemia, edema and hyperlipidemia

90% idiopathicCauses of idiopathic: minimal

change disease(85%), mesangial proliferation (5%), and focal segmental glomerulosclerosis (10%)

59. YOU NOTE THAT A NEWBORN BABY BOY’S PREPUCE CANNOT BE RETRACTED. HE HAS

a. Hypospadiasb. Phimosisc. Chordeed. Paraphimosis

ANOMALIES OF THE PENIS AND URETHRA

Hypospadias- a urethral opening that is on the ventral surface of the penile shaft (1/250)

Chordee without hypospadias – mild or moderate ventral penile curvature (chordee) and incomplete development of the foreskin but the urethral meatus is at the tip of the glans

Phimosis – inability to retract the prepuce Paraphimosis – occurs when the foreskin is

retracted past the coronal sulcus and the prepuce cannot be pulled back over the glans

60. A 3-MONTH OLD BABY WITH UNILATERAL UNDESCENDED TESTIS WAS BROUGHT TO YOU FOR THE FIRST TIME. THE MOTHER ASKS IF SURGERY IS REQUIRED TO BRING IT DOWN. YOU TELL HER THAT YOU WILL OBSERVE FOR THE MEANTIME. HOW LONG WILL YOU OBSERVE?

a. 1 monthb. 2 monthsc. 3 monthsd. 4 months

UNDESCENDED TESTES (CRYPTORCHIDISM)

Most common disorder of sexual differentiation in boys

4.5% of boys at birthBilateral in 10% of casesMajority descend spontaneously

by 3 monthsIf the testis does not descend by

4 months, it will remain undescended

61. WHICH IS TRUE ABOUT STATUS EPILEPTICUS?

a. Continuous convulsion lasting for 25 minutes

b. Serial convulsions with return of consciousness in between

c. Always generalizedd. A medical emergency

STATUS EPILEPTICUS

Continuous convulsion lasting longer that 30 minutes

Serial convulsions with no return of consciousness in between

May be generalized or partialMedical emergency

62. THE FOLLOWING ARE PART OF THE MANAGEMENT OF HYPERCYANOTIC SPELLS, EXCEPT:

a. Hydrationb. Oxygen supportc. Lactate administrationd. Beta blocker administration

TREATMENT OF HYPOXIC SPELLS Knee chest position – traps systemic venous blood in

the legs thereby temporarily decreasing the systemic venous return and helping calm the baby. This also increases the systemic venous resistance (SVR) by reducing arterial blood flow through the femoral arteries

Morphine sulfate – suppresses the respiratory center and abolishes hyperpnea

NaHCO3 – corrects acidosis and eliminates the respiratory center-stimulating effects of acidosis

Administration of oxygen may improve arterial oxygen saturation a little

Vasoconstrictors such as phenylephrine raise SVR Ketamine – increases SVR and sedates the patient Propranolol

63. HOW WILL YOU MANAGE A PATIENT WITH ACUTE OTITIS MEDIA WHO HAS BEEN TREATED WITH AMOXICILLIN AT 50 MKD FOR 3 DAYS, BUT STILL HAS FEVER, EAR PAIN AND EAR DISCHARGE? THE PATIENT HAS NOT BEEN TREATED WITH ANY OTHER ANTIBIOTIC PRIOR TO THIS EPISODE?a. Continue his medicationb. Do tympanocentesisc. Give clindamycin at 40 mkdd. Increase the amoxicillin dose to

80-90 mkd

ACUTE OTITIS MEDIA

Etiology: Strep pneumoniae, non-typable H influenzae, Moraxella catarrhalis

Treatment:1st line : Amoxicillin 40 mkd 80-100mkdHigher dose for children < 2 yrs, children

who have recently received treatment with B lactam drugs, children who are exposed to large number of children

2nd line: Amoxicillin-clavulanic acid, cefuroxime, ceftriaxone

65. A MOTHER IS CONCERNED ABOUT HER DAUGHTER’S WEIGHT. THE 9 YEAR OLD GIRL WEIGHS 26 KG. NUTRITIONAL HISTORY REVEALS A WELL-BALANCED DIET. WHAT ADVICE WILL YOU GIVE?

a. Enrol her daughter on a weight loss program

b. Prescribe multivitamins and mineral supplements

c. Assure her that weight for age is normal and continue with her diet

d. Tell her to wait for her menarche and then you will reassess

WEIGHT• Best index of growth and nutrition• Mnemonics:

• Infants < 6 months

• Wt. in gms = Age in mos x 600 + BW

• 6-12 months

• Wt. in gms = Age in mos x 500 + BW

• 2 years and up

• Wt. in kgs = Age in years x 2 + 8

66. WHICH OF THE FOLLOWING DISEASES IS PART OF THE NEONATAL NEWBORN SCREENING?

a. Congenital toxoplasmosisb. Congenital rubellac. Congenital adrenal

hyperplasiad. Congenital varicella

NEWBORN SCREENING

METABOLIC DISEASES

Inborn Errors of MetabolismAminoacids: PKU, MSUD, TyrosinemiaLipids: ALDCarbohydrates: Von Gierke’s, Pompe’s,

GalactosemiaMucopolysaccharides: Hurler’s, Hunter’s, SanfilippoPurine and Pyrimidines: Gout, Lesch-Nyhan

Newborn ScreeningPKU, CAH, CH, GAL, G6PDRepublic Act 9288: Newborn Screening Act of 2004Done after the 24th HOL and not later than 72 HOL

67. A NEONATE WITH A HEART RATE OVER 100/MIN, SHOW IRREGULAR BREATHING, ACTIVE MOTION, GRIMACE, PINK BODY BUT BLUE EXTREMITIES HAS AN APGAR SCORE OF __ AT 1 MINUTE

a. 7b. 8c. 9d. 10

PHYSICAL EXAM

APGAR SCOREDictates the need to resuscitate Normal: 7- 9 Sign of distress: </= 6 Severe asphyxia: 1-3 5 mins: prolonged distress in

utero, some degree of damage, expect neurologic sequelae

10 mins: if still< 7, continue taking APGAR until >7 is REACHED!!!

APGAR EVALUATION OF NEWBORNS

SIGN 0 1 2

Heart Rate Absent Below 100 Over 100

Respiratory Effort

Absent Slow, irregular Good, crying

Muscle tone Limp Some flexion of extremities

Active motion

Response to catheter in nostril

No response Grimace Cough or sneeze

Color Blue, pale Body pink, extremities blue

Completely pink

68. A PATIENT WAS BROUGHT TO THE ER DUE TO FLACCID PARALYSIS. YOU SUSPECT THAT HE HAS AN ELECTROLYTE IMBALANCE. THE PATIENT WAS HOOKED TO A CARDIAC MONITOR AND YOU NOTE THAT HE HAS PROLONGED PR INTERVAL, WIDENED QRS AND PEAKED T WAVES. THESE FINDINGS ARE CONSISTENT WITH WHICH ELECTROLYTE ABNORMALITY?

a. Hypercalcemiab. Hypocalcemiiac. Hypokalemiad. Hyperkalemia

HYPERKALEMIA

Most important effects are due to the role of K in membrane polarization

Peaked T waves , ST segment depression, increased PR interval, flattening of the P wave and widened QRS complex

Can lead to ventricular fibrillation Paresthesias, fasciculations, weakness and

even an ascending paralysis Cardiac toxicity precedes all these symptoms

HYPOKALEMIA

Common in children with most cases related to gastroenteritis

Clinical Manifestations Heart and skeletal muscles are vulnerable – ECG

shows flattened T waves, depressed ST segment, and the appearance of a U wave

Muscle weakness and cramps Paralysis if <2.5meq’L Poluria and polydipsia

69. YOU DIAGNOSE AN 8-YEAR OLD BOY AS HAVING ADRENOLEUKOYSTROPHY WITH MILD CEREBRAL INVOLVEMENT. WHAT IS THE MOST EFFECTIVE THERAPY FOR HIM?

a. Steroidsb. Bone marrow transplantationc. Lorenzo’s oild. Baclofen

ALD

X-linked Accumulation of unbranched saturated VLCFA Treatment

CorticosteroidBMT – benefits those with neurologic

disabilityLorenzo’s oilBaclofen- for spasms

70. A CHILD WITH UNEXPLAINED BONE PAIN, EASY BRUISABILITY, AND HEPATOMEGALY WAS SUSPECTED TO HAVE LIPID STORAGE DISEASE. ENZYME ASSAY OF CULTURED FIBROBLASTS SHOWED DEFECTS IN B-GLUCOSIDASE ACTIVITY. WHICH OF THE FOLLOWING DISEASES DOES HE HAVE?

a. Gaucher diseaseb. Niemann-Pick diseasec. Tay-sachs diseased. Sandhoff disease

LIPIDOSES

Disorder Enzyme defect

Gaucher B glucosidase

Niemann-Pick Acid sphingomyelinase

Tay-Sachs B hexoseaminidase

Sandhoff B hexoseaminidase

71. WHICH OF THE FOLLOWING IS TRUE ABOUT ANOREXIA NERVOSA/

a. Fear of not being able to stop eating

b. Refusal to maintain body weight over a minimal weight for age and height

c. Absence of at least 2 consecutive expected menstrual cycles

d. Self evaluation is not influenced by weight and height

ANOREXIA NERVOSA AND BULIMIA Anorexia

Intense fear of becoming obese, which does not diminish as weight loss progresses

Disturbance in the way in which one’s body weight, size or shape is experienced

Refusal to maintain BW over a minimal normal weight for age and height

Absence of at least 3 consecutive menstrual cycles Bulimia

Recurrent episodes of binge eating Fear of not being able to stop eating Self induced vomiting Minimum average of 2 binge eating per week x 3

mos Self evaluation is unduly influenced by boy weight

and shape

72.A TEENAGE GIRL WITH ENDOMETRIOSIS IS COMPLAINING OF PAINFUL CRAMPS DURING MENSES. SHE HAS

a. Dysfunctional uterine bleeding

b. Primary dysmenorrheac. Secondary dysmenorrhead. Premenstrual syndrome

PAINFUL MENSTRUAL CRAMPS

Primary DysmenorrheaAbsence of any specific pelvic

pathologic conditionMost commonProstaglandins F2 and E2

Secondary dysmenorrheaResults from an underlying structural

abnormality of the cervix or uterus, a foreign body , endometriosis or endometritis

73. DRUG OF CHOICE FOR ANAPHYLAXIS

a. Epinephrineb. Diphenhydraminec. Loratadined. Cyproheptadine

ANAPHYLAXIS

A serious allergic reaction that is rapid in onset and may cause death

Pathologic features – acue pulmonary hyperinflation, pulmonary edema, intra alveolar hemorrhaging, visceral congestion, laryngeal edema and urticaria and angioedema

Activation of mast cells and basophils via cell bound allergen specific IgE molecules

Treatment: IM or IV epinephrine

74. WHICH OF THE FOLLOWING IS RARELY TRANSMITTED PERCUTANEOUSLY?

a. Hepatitis Ab. Hepatitis Bc. Hepatitis Cd. Hepatitis D

75. WHICH OF THE FOLLOWING MARKERS TELLS YOU THAT YOUR PATIENT WITH HEPATITIS B IS HIGHLY INFECTIVE?

a. HBsAgb. HBeAgc. IgM anti-HBcd. Anti-HBc

HEPATITIS B MARKERS

HBsAg – first marker to appear; its rise coincide with appearance of symptoms

HBeAg – present during acute phase and indicates a highly infectious state

Anti-HBcAg IgM – rises early after infectionMost valuable single serolologic

marker of acute HBV infection Anti-HBc IgG – replaces IgM Anti HBsAg – present among

immunized persons

76. THE BEST WAY TO PREVENT DIARRHEA IN INFANCY IS

a. Proper hygieneb. Immunizationc. Boil waterd. Breastfeed

77. A 1 YEAR OLD CHILD WITH MEASLES MUST BE GIVEN VIT A. WHICH IS THE CORRECT DOSE?

a. 50,000 IUb. 100,000 IUc. 150,000 IUd. 200,000 IU

VITAMIN A IN MEASLES

Reduces morbidity and mortality

6 mos- 1yr : 100,000 IU1 yr older : 200,000 IU

78. WHICH OF THE FOLLOWING IS EXPECTED OF A 4 YEAR OLD?

a. Tell a storyb. Name 4 colorsc. Draw a triangled. Dress and undress

GROSS MOTOR MILESTONES

Mean Age (mos)Mean Age (mos) MilestonesMilestones

33 Head controlHead control

55 Roll overRoll over

66 Sit with supportSit with support

88 Sit aloneSit alone

99 Pulls to stand holding onPulls to stand holding on

1111 Stand independentlyStand independently

1515 Walk aloneWalk alone

2424 Climb up and down stairsClimb up and down stairs

3636 Ride a trikeRide a trike

4848 Hop on one footHop on one foot

EXPRESSIVE LANGUAGE MILESTONES

AgeAge

(mos)(mos)

MilestonesMilestones AgeAge

(yrs)(yrs)

MilestonesMilestones

33 Vocalizes and coosVocalizes and coos 22 2-word phrases2-word phrases

66 BabblesBabbles 33 3-word sentence3-word sentence

Gives full name,age,sexGives full name,age,sex

88 Says dada,mama Says dada,mama non-specificallynon-specifically

44 Repeats 3-word sentenceRepeats 3-word sentence

Speech 100% intelligibleSpeech 100% intelligible

1010 First words other First words other than mama, dadathan mama, dada

55 Uses past tense of eat, Uses past tense of eat, run, gorun, go

1212 Immature Immature jargon,second wordjargon,second word

66 Gives word for definitionGives word for definition

79. WHICH OF THE FOLLOWING IS A CONTRAINDICATION TO ROUTINE CHILHOOD IMMUNIZATION?

a. Maternal viral illnessb. Antibiotic treatment of a

childc. Fever (temperature of 37.8C)d. None of the above

80. PULMONARY VASCULAR RESISTANCE FALLS RAPIDLY AT BIRTH DUE TO

a. Rise in arterial pO2b. Closure of the ductus

arteriosusc. Increase in pulmonary blood

flowd. Decrease in tortuosity of the

pulmonary vasculature

Pulmonary resistance & pressure

blood from R ventricle enter the

pulmonary circulation

L atrial pressure

physiologic closure of foramen ovale

O2 content

muscular constriction and functional closure

of patent ductus arteriosus

81. AN 18-YEAR OLD GIRL WITH PRIMARY AMENORRHEA, SHORT STATURE, WEBBED NECK AND COARCTATION OF THE AORTA CONSULTS AT YOUR CLINIC. THE MOST LIKELY DIAGNOSIS IS:

a. Marfan syndromeb. Down syndromec. Palau syndromed. Turner syndrome

TURNER SYNDROME Incidence: 1 /4000 Complete or partial absence of the

x chromosome 45x Findings

Phenotypically female Short stature Underdeveloped gonads Webbed neck

82. NOT A CONTRAINDICATION TO LUMBAR TAP

a. Papilledemab. Gaping wound at L2-L5 areac. Pending cerebral herniationd. Platelet count of 100 x 109 /L

83. A PATIENT WITH HEMOPHILIA B IS BLEEDING PROFUSELY. WHICH OF THE FOLLOWING WILL YOU TRANSFUSE

a. Fresh frozen plasmab. Factor IX concentratec. Cryoprecipitated. Platelet concentrate

Hereditary Clotting Factor Deficiencies Factor VIII or IX (Hemophilia A or B) Inc APTT, normal PC, BT, PT, thrombin time Hallmark: hemarthrosis

Von Willebrand Disease Most common hereditary bleeding disorder Sx of mucocutaneous bleeding Inc BT, PTT, normal PC

Liver Disease Clotting factors produced in the liver except VIII Treatment consists of replacement with FFP or

cryoprecipitate

HEMORRHAGIC AND THROMBOTIC DISEASES

84. THE PATENT FORAMEN OVALE FUNCTIONALLY CLOSES AT:

a. 10-15 hours of lifeb. 24 hours of lifec. 1 month of lifed. 3 months of life

CLOSURE OF SHUNTS

Foramen ovaleFunctionally closed by the 3rd

monthDustus arteriosus

Functional closure by the 10-15th HOL

85. CYSTIC HYGROMA OCCURS MOST FREQUENTLY IN THE

a. Abdomenb. Extremityc. Head and neckd. Chest

CYSTIC HYGROMAS AND LYMPHANGIOMAS

Arise in the embryonic lymph sac and are the 2nd most common benign vascular tumors in children (1st – hemangiomas)

Half – located in the head and neck area 50% are present at birth, with most

presenting by 2 y/o

86. WHICH IS THE DRUG OF CHOICE FOR PROPHYLACTIC TREATMENT OF FEBRILE SEIZURES?

a. Phenobarbitalb. Phenytoinc. Carbamazepined. None of the above

FEBRILE SEIZURES 6 mos – 60 mosRare before 9 mos and after 5 years oldRapid increase in temperature (>39C)r/o CNS infection or metabolic causesUsually generalized, tonic-clonic and

lasts for a few secondsSimple vs complex (>15 min, repeated

convulsions, focal findings)LP, EEG, imaging proceduresAnticonvulsants and Diazepam?

87. WHICH OF THE FOLLOWING IS ASSOCIATED WITH GASTRITIS AND PEPTIC ULCER DISEASE?

a. Escherichia colib. Mycoplasma pneumoniaec. Helicobacter pylorid. Enterobius vermicularis

88. WHICH OF THE FOLLOWING IS AN INDICATION FOR PACKED RBC TRANSFUSION IN CHILDREN?

a. Acute blood loss of 15% of total blood volume

b. Hemoglobin of 11 mg/dl preoperatively

c. Hemoglobin of 9 mg/dl but asymptomatic

d. Hemoglobin of 10 mg/dl in congestive heart failure

PEDIATRIC RBC TRANSFUSIONS

Acute loss > 25% of circulating blood volume

Hgb < 8 g/dl in perioperative period Hgb <13 g/dl and severe

cardiopulmonary disease Hgb <8 g/dl and symptomatic chronic

anemia Hgb < 8 g/dl and marrow failure

89. WHICH OF THE FOLLOWING VALUES TELLS YOU THAT A CHILD HAS SEVERE MALNUTRITION BASED ON WATERLOWE CLASSIFICATION?

a. 60%b. 70%c. 80%d. 90%

91. ANTIDOTE FOR IRON TOXICITY

a. N-acetylcysteineb. Deferoxaminec. Naloxoned. EDTA

POISONING Acetaminophen

Acute toxic dose: 200mg/k Tx: N-acetylcysteine (NAC)

Salicylates 150mg/k Gastric decontamination, hydration, urinary excretion,

Dialysis Iron

>60 mg/k of elemental iron Deferoxamine

92. WHICH OF THE FOLLOWING IS A DIAGNOSTIC ABDOMINAL X-RAY FINDING OF NECROTIZING ENTEROCOLITIS?

a. Air fluid levels in the bowelsb. Dilated bowel loopsc. Pneumatosis intestinalisd. Dilated proximal bowel with

constriction distally

NECROTIZING ENTEROCOLITIS

Necrotizing Enterocolitis- most common life threatening emergency of the GI tract in the newborn periodPneumatosis intestinalisTriad: abdominal distention, gastric

retention, bloody stoolsTx: no definitive tx for established NEC;

only supportive care and preventing further injury

If tx is needed: surgery and Metronidazole

93. A CHILD WITH ACRODERMATITIS ENTEROPATHICA, POOR WOUND HEALING AND GROWTH RETARDATION HAS:

a. Iron deficiencyb. Selenium deficiencyc. Taurine deficiencyd. Zinc deficiency

ZINC

Acrodermatitis enteropathica – rare autosomal disorder caused by an inability to absorb sufficient zinc from the diet

94. CONTRAINDICATION TO MMR VACCINATION

a. Breastfeedingb. Pregnancyc. Tuberculosisd. Diarrhea

95. PEUTZ-JEGHERS SYNDROME PRESENTS AS POLYPOSIS OF THE SMALL BOWELS AND WHICH OF THE FOLLOWING?

a. Nasal polyposisb. Hemangiomac. Hyperpigmented macules on

the face lips, and buccal mucosa

d. Periungual desquamation

PEUTZ-JEGHERS SYNDROME

Characterized by melanotic papules on the lips and mucous membranes and by gastrointestinal polyposis

Autosomal dominant Polyposis usually involves the jejunum and

ileum but may also involve the stomach, duodenum, colon and rectum

96. AN IMPORTANT REFLEX TO FACILITATE BREASTFEEDING

a. Moro reflexb. Palmar grasp reflexc. Rooting reflexd. Parachute reflex

NUTRITION Breastfeeding vs Formula feeding

Advantages Techniques

Rooting reflex Sucking reflex: prolactin (AP) – milk secretion

oxytocin (PP) - contraction Swallowing reflex

Contraindications Septicemia HIV Active TB Typhoid fever Breast CA Malaria Substance abuse and severe neuroses or psychoses

97. AT WHAT AGE CAN THE SNELLEN CHART BE USED TO ASSESS VISUAL ACUITY

a. 2 yearsb. 3 yearsc. 4 yearsd. 5 years

98. A 15 YEAR OLD HAD HIS UPPER CENTRAL INCISOR KNOCKED OUT OFF THE SOCKET. HE WANTS IT REPLANTED. IN WHAT MEIUM SHOULD THE TOOTH BE TRANSPORTED?

a. Cow’s milkb. Tap waterc. Normal salined. Water with sugar

A viable periodontal ligament (PDL) is important for proper physiologic healing of replanted teeth. One of the critical factors affecting outcome is the way the tooth is handled during the extraoral period. A suitable storage medium should have a physiologic osmolality in order to keep the PDL cells in as healthy a condition as possible. Milk has an osmolality within physiologic limits. In vitro studies suggest that it is superior to saliva (Blomlof and Otteskog 1980) and to Eagle’s medium (Blomlof 1981) and other media (Ashkenazi et al. 1999, Lekic et al. 1998) in terms of PDL cell viability. Milk appears to be the storage medium of choice in an emergency situation, although a new storage medium: Propolis may be better (Martin and Pileggi 2004)

99. WHICH OF THE FOLLOWING SLEEPING POSITIONS HAS BEEN NOTED TO DECREASE THE INCIDENCE OF SUDDEN INFANT DEATH SYNDROME?

a. Supineb. Pronec. Sided. Any of the above

SIDS Sudden death of an infant that is

unexpected by history and unexplained by thorough post mortem examination, which includes autopsy, investigation of the scene of death, and review of the medical history

Environmental and genetic risk factors Infant sleep environment: sleeping

prone has consistently shown to increase the risk of SIDS

Recommendation: supine position

100. A CHILD ACCIDENTALLY INGESTED A BLEACHING SOLUTION (ZONROX). WHICH OF THE FOLLOWING SHOULD BE THE INITIAL MANAGEMENT?

a. Give large amount of milkb. Induce emesisc. Gastric lavaged. Rinse the mucosa

POISONING

Caustics Acids and Alkali Thorough removal Emesis and lavage are contraindicated Activated charcoal should not be used

Insecticides SLUDGE ( salivation, lacrimation, urination,

defacation, gastrointestinal cramps, emesis) Antidotes: atropine and pralidoxime

THANK YOU!