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Τριμηνιαία έκδοση της Ελληνικής Παιδιατρικής Εταιρείας
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¢ÈÌËÓÈ·›· ¤Î‰ÔÛË Ù˘
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Î·È ÂÊ‹‚Ô˘˜
∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹
82 §ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ - ¡ÂfiÙÂÚ·
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µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹
89 ∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË
Ã. ∫Ô˘ÙÛ·˘Ù›ÎË, ∂. ª¿ÓÙ˙ÈÔ˘, ¡. ª˘ÚÈÔÎÂÊ·ÏÈÙ¿Î˘
97 ¡ÂfiÙÂÚ· ‰Â‰Ô̤ӷ ÁÈ· ÙË Û˘ÁÁÂÓ‹
‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË
Ã. ∆۷ηϛ‰Ë˜, ∂. √˘ÚÁηÓÙ˙fiÁÏÔ˘, ¡. ¡ÈÎÔÏ·˝‰Ë˜
∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™
109 ∂›‰Ú·ÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
Ì˘ÂÏÔ‡ ÔÛÙÒÓ ÛÙËÓ ex vivo ¤ÎÙ˘ÍË
·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜
π. ¶ÂÏ·ÁÈ¿‰Ë˜, ∂. ¢ËÌËÙÚ›Ô˘, ∂. ™ÙÂȷοÎË, Ã. ¶ÂÚ‰ÈÎÔÁÈ¿ÓÓË, ∂. ÷Ù˙ˉ¿ÎË, ª. ∫·ÏÌ·ÓÙ‹
117 ¶·ÓÂÏÏ‹ÓÈ· ÔÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË
Ë·Ù›Ùȉ·˜ ∞ Û ·È‰È¿ ËÏÈΛ·˜ 0-14 ÂÙÒÓ
µ. ¶··Â˘·ÁÁ¤ÏÔ˘, ∞. ∫‡Úη, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜, ¢. ∫·Û›ÌÔ˜, ∫. ¶·ÓÙÂÏ¿ÎË, ∂. ¶·‡ÏÔ˘, ª. ∆˙Ô‡ÊË,ª. ª·˘ÚÔÎÒÛÙ·, ∞. ∆˙È‚¿Ú·˜, •. ¶Â‰¤ÏË, ∞. ÷Ù˙ËÌȯ·‹Ï, º. ∞ı·Ó·ÛÈ¿‰Ô˘, ¢. ∫·ÊÂÙ˙‹˜, ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜
123 §¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿:
ÂÌÂÈÚ›· 28 ÂÙÒÓ
∞. ¶Ô˘ÚÙÛ›‰Ë˜, ¢. ¢ÔÁ¿Ó˘, ª. ª¿Î·, ¢. ªÔ˘¯Ô‡ÙÛÔ˘, ª. µ·Ú‚Ô˘ÙÛ‹, ª. ™˘ÓÔ‰ÈÓÔ‡,∞ÈÎ. ªÈ¯·‹Ï-™ÙÚ¿ÓÙ˙È·, ∂. ∫ÔÛÌ›‰Ë
¶ƒ∞∫∆π∫√ £∂ª∞
132 √È ÂÈÙÒÛÂȘ Ù˘ ÂÚÙÚÔÊ›·˜ ÙˆÓ
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ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ
∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘
139 √Ú›˙ÔÓÙ·˜ ÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋
·¯˘Û·ÚΛ· ÛÙËÓ Ú¿ÍË:
ÌÂıÔ‰ÔÏÔÁÈο ‰ÈÏ‹ÌÌ·Ù·
¶. ¶ÂÚ‚·Ó›‰Ô˘
∂¡¢π∞º∂ƒ√À™A ¶∂ƒπ¶∆ø™H
145 ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘
Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜
ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘
Ã. ∆۷ηϛ‰Ë˜, µ. §·ÌÚfiÔ˘ÏÔ˜, Œ. √˘ÚÁηÓÙ˙fiÁÏÔ˘, ¢. ƒ¿ÏÏ˘, ∞. ¶ÂÙÚfiÔ˘ÏÔ˜, ¡. ¡ÈÎÔÏ·˝‰Ë˜
∫§π¡π∫√ ∫√Àπ∑
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151 ¡¤· ·fi ÙÔÓ ÒÚÔ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ
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¡. ™˘Ú›‰Ë˜
∂§§∏¡π∫∏ ∂ƒ∂À¡∞ ™∆∏ ¢π∂£¡∏µπµ§π√°ƒ∞ºπ∞
154 ∂ÏÏËÓÈ΋ ·È‰È·ÙÚÈ΋ ¤Ú¢ӷ Û ‰ÈÂıÓ‹
ÂÚÈÔ‰Èο, 2008
∂. °·Ï·Ó¿Î˘
¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√
156 πÛÙÔÛÂÏ›‰· Ù˘ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘
¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ
™. ∫¯·ÁÈ¿˜, ¢. ∞Ó·ÁÓÒÛÙÔ˘
¶·È‰È·ÙÚÈ΋∆fiÌÔ˜ 72 ñ ∆‡¯Ô˜ 2 ñ ª¿ÚÙÈÔ˜-∞Ú›ÏÈÔ˜ 2009
∫ˆ‰ÈÎfi ¢È‡ı˘ÓÛË ∂ÔÙ›· ªª∂: 3889
ISSN 0377-2551
Pediatri Mar-Apr 09 10-04-09 15:35 ™ÂÏ›‰·1
Bimonthly Publication of
the Greek Paediatric Society
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iae.pdf
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Contents
REVIEW ARTICLES
75 Acute lymphoblastic leukaemia in children
and adolescents
E. Stiakaki, M. Kalmanti
82 Childhood lymphomas - Novel therapeutic
approaches
V. Danilatou, M. Kalmanti
89 Haemophagocytic lymphohistiocytosis - HLH
C. Koutsaftiki, E. Mantziou, N. Myriokefalitakis
97 Current data on congenital diaphragmatic
hernia
C. Tsakalidis, E. Ourgantzoglou, N. Nikolaidis
ORIGINAL ARTICLES
109 The effect of paediatric bone-marrow-
derived stromal cells on the ex-vivo
expansion of cord-blood haemopoietic
stem cells
I. Pelagiadis, H. Dimitriou, E. Stiakaki, C. Perdikogianni, E. Hatzidaki, M. Kalmanti
117 Seroepidemiological study of hepatitis A in
Greek children aged 0-14 years
V. Papaevagelou, A. Kyrka, A. Tragiannidis, D. Kassimos, K. Pantelaki, E. Pavlou, M. Tzoufi,M. Mavrokosta, A. Tzivaras, X. Pedeli, A. Hatzimichail, F. Athanassiadou, D. Kafetzis, A. Konstantopoulos
123 Hodgkin’s lymphoma in children:
28 years of experience
A. Pourtsidis, D. Doganis, M. Baka, D. Bouhoutsou, M. Varvoutsi, M. Synodinou, A. Michael-Strantzia, E. Kosmidi
PRACTICAL ISSUES
132 Cardiovascular consequences of
adenotonsillar hypertrophy in children
K. Papadopoulou-Legbelou, G. Varlamis
139 Defining obesity in children and
adolescents: methodological dilemmas
P. Pervanidou
CASE REPORT
145 Bochdalek congenital diaphragmatic
hernia with intrathoracic ectopic kidney -
a case report
C. Takalidis, V. Lampropoulos, E. Ourgantzoglou,D. Rallis, A. Petropoulos, N. Nikolaidis
CLINICAL QUIZ
150 Thirteen-year-old boy with recurrent
episodes of painful ankle joint
¡. Markeas, π. Papachristos
PAEDIATRIC NEWS IN BRIEF
151 News on paediatric infections
N. Spyridis
HELLENIC RESEARCH IN THEINTERNATIONAL LITERATURE
154 Hellenic paediatric research in
international journals, 2008
E. Galanakis
NEWS FROM THE INTERNET
156 Site of the 2nd Department of Paediatrics
of the University of Athens
S. Kehagias, D. Anagnostou
PaediatrikiVolume 72 ñ Number 2 ñ March-April 2009
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·3
75∞¡∞™∫√¶∏™∏ REVIEW ARTICLE
¶·È‰È·ÙÚÈ΋ 2009;72:75-000
√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜
∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹
¶ÂÚ›ÏË„Ë: ∏ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Â›Ó·È Ë Û˘¯ÓfiÙÂÚË Î·ÎÔ‹ıÂÈ· ÛÙ· ·È‰È¿. ∏ ÚfiÔ‰Ô˜ ÛÙˉȿÁÓˆÛË Î·È Ë ıÂڷ›· Û‡Ìʈӷ Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ ÙÔ˘ οı ·È‰ÈÔ‡ ¤¯Ô˘Ó ‰ÒÛÂÈ ÔÛÔÛÙ¿ ›·-Û˘ Ô˘ ÏËÛÈ¿˙Ô˘Ó ÙÔ 90%. ∏ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ÌÂϤÙË ÙˆÓ ‚Ï·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·›˙ÂÈ ÛËÌ·ÓÙÈÎfi Úfi-ÏÔ ÛÙËÓ ÚÔÛ·ÚÌÔÁ‹ Ù˘ ıÂڷ›·˜ Î·È ÛÙËÓ ·Ó·˙‹ÙËÛË Ó¤ˆÓ ıÂڷ¢ÙÈÎÒÓ ÛÙfi¯ˆÓ. ∏ ηٿٷÍË ÛÂÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ ‚·Û›˙ÂÙ·È Û ÎÏÈÓÈο Î·È Î˘ÙÙ·ÚÔÁÂÓÂÙÈο ‰Â‰Ô̤ӷ, ηıÒ˜ Î·È ÛÙËÓ ÂÎÙ›ÌËÛË Ù˘ ÂÏ¿-¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘, Ô˘ Ê·›ÓÂÙ·È Ó· ·ÔÙÂÏ› ¤Ó·Ó ·fi ÙÔ˘˜ ÈÛ¯˘ÚfiÙÂÚÔ˘˜ ÚÔÁÓˆÛÙÈÎÔ‡˜·Ú¿ÁÔÓÙ˜ Î·È ·Ú¤¯ÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ÁÈ· ۯ‰fiÓ ÂÍ·ÙÔÌÈÎÂ˘Ì¤ÓË ıÂڷ›·. ∂ÈÚfiÛıÂÙ·, ÚfiÛÊ·Ù˜ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó fiÙÈ ÔÈ ¤ÊË‚ÔÈ Î·È ÔÈ Ó·ÚÔ› ÂÓ‹ÏÈΘ ¤¯Ô˘Ó Ôχ ÌÂÁ·Ï‡ÙÂÚ· ÔÛÔÛÙ¿ ›·Û˘ fiÙ·Ó ıÂ-ڷ‡ÔÓÙ·È Ì ·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ·, Û˘ÁÎÚÈÙÈο Ì ÂΛÓÔ˘˜ Ô˘ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ıÂڷ¢ÙÈοۯ‹Ì·Ù· ÙˆÓ ÂÓËϛΈÓ.
§¤ÍÂȘ ÎÏÂȉȿ: √Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·, ·È‰È¿, ¤ÊË‚ÔÈ.
Acute lymphoblastic leukaemia in children and adolescents
E. Stiakaki, M. Kalmanti
Abstract: Acute lymphoblastic leukaemia is the most common malignancy in childhood. Progress indiagnosis and treatment according to risk classification have produced cure rates that now approach 90%.Molecular genetics of the blasts plays a crucial role in tailoring therapy and identifying new targets forspecific treatment. Current risk classification schemes are based on clinical characteristics and cytogeneticfeatures, and on minimal residual disease assessment, which appears to be among the strongestprognostic factors and provides an opportunity for more precise risk tailored treatments. Recent studieshave shown that adolescents and young adults have a much better outcome when treated with paediatricthan with adult protocols.
Key words: Acute lymphoblastic leukaemia, children, adolescents
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘
AÏÏËÏÔÁÚ·Ê›·:
∂˘Ù˘¯›· ™ÙÂȷοÎË[email protected]¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜,¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô∏Ú·ÎÏ›Ԣ, 71110 ∏Ú¿ÎÏÂÈÔ∫Ú‹Ù˘
Department of PaediatricHaematology/Oncology,University of Crete, Greece
Correspondence:
Eftichia [email protected] of PaediatricHaematology/Oncology,University Hospital ofHeraklion, 71110 Heraklion,Crete, Greece
™˘ÓÙÔÌÔÁڷʛ˜:
ALL acute lymphoblastic leukemia
BFM Berlin-Frankfurt-Muenster
CCG Children’s Cancer Group
COG Children’s Oncology Group
DFCI Dana-Farber Cancer Institute
MRD Minimal Residual Disease (ÂÏ¿¯ÈÛÙË
˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ˜)
NCI National Cancer Institute
POG Pediatric Oncology Group
√§§ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·
∫¡™ ∫ÂÓÙÚÈÎfi ¡Â˘ÚÈÎfi ™‡ÛÙËÌ·
∂ÈÛ·ÁˆÁ‹
∏ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· (√§§)
Â›Ó·È Ë Û˘¯ÓfiÙÂÚË Î·ÎÔ‹ıÂÈ· ÛÙ· ·È‰È¿ ηÈ
·ÔÙÂÏ› ÙÔ 75-80% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ Ó¤ˆÓ Â-
ÚÈÙÒÛÂˆÓ Ï¢¯·ÈÌ›·˜. ∏ Û˘¯ÓfiÙËÙ¿ Ù˘ ›ӷÈ
35 Ì 40 Ӥ˜ ÂÚÈÙÒÛÂȘ ÙÔ ¯ÚfiÓÔ ·Ó¿
1000.000 ·È‰È¿ ËÏÈΛ·˜ <15 ÂÙÒÓ, Ì ̤ÁÈÛÙË
›وÛË ÛÙËÓ ËÏÈΛ· ÙˆÓ 4 ÂÙÒÓ (1).
∏ ÚfiÔ‰Ô˜ ÛÙË ıÂڷ›· Ù˘ √§§ ÛÙ· ·È-
‰È¿ ·ÔÙÂÏ› ÌÂÁ¿ÏÔ Â›Ù¢ÁÌ· Ù˘ Û‡Á¯ÚÔÓ˘
ÎÏÈÓÈ΋˜ ÔÁÎÔÏÔÁ›·˜, ηı’ fiÛÔÓ ÙÔ 1970 ÙÔ Ô-
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ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂڷ›·˜ ηÈ, ‚¤‚·È·,
ÛÙËÓ Ù·ÍÈÓfiÌËÛË Î·È ıÂڷ›· Ì ‚¿ÛË ÙÔ˘˜
·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ (3).
∂Ú¢ÓËÙÈο, ÙÔ ÂӉȷʤÚÔÓ ¤¯ÂÈ ÂÛÙÈ·ÛÙ›
ÛÙË ÌÂϤÙË ÙÔ˘ ÚfiÏÔ˘ ÙˆÓ ÌÔÚÈ·ÎÒÓ ‰È·Ù·Ú·-
¯ÒÓ ÛÙË Ï¢¯·ÈÌÔÁ¤ÓÂÛË, Û ʷÚÌ·ÎÔÁÂÓÂÙÈ-
Τ˜ ÌÂϤÙ˜ Î·È ÛÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÚfiÏÔ˘ Ù˘
˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘.
¶·Ú¿ ÙËÓ ÚfiÔ‰Ô Ô˘ ¤¯ÂÈ ÂÈÙ¢¯ı›, ¤Ó·
ÔÛÔÛÙfi ·È‰ÈÒÓ Ï·Ì‚¿ÓÂÈ ›Ûˆ˜ ÂÚÈÛÛfiÙÂÚË
ıÂڷ›· ·fi ÂΛÓË Ô˘ Ú·ÁÌ·ÙÈο ¯ÚÂÈ¿˙Â-
Ù·È ÁÈ· Ó· È·ı›, 10-20% ÙˆÓ ·È‰ÈÒÓ Ì ·Ú¯Èο
¢ÓÔ˚ÎÔ‡˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÛÙË
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·75
76 ∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹
Paediatriki 2009;72:76-000
Û˘Ó¤¯ÂÈ· ˘ÔÙÚÔÈ¿˙Ô˘Ó, ÂÓÒ ˘¿Ú¯ÂÈ Â›Û˘ ÌÈ·
ÔÌ¿‰· ·ÛıÂÓÒÓ Ì ÓfiÛÔ ·ÓıÂÎÙÈ΋ ÛÙË ıÂڷ›·.
∂Âȉ‹ Ë ÂÚ·ÈÙ¤Úˆ ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂڷ›·˜
‰ÂÓ Ê·›ÓÂÙ·È Ó· ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÚfiÁÓˆÛË, ÙÔ ÂӉȷ-
ʤÚÔÓ ¤¯ÂÈ ÛÙÚ·Ê› Û ӤԢ˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ·-
ÚÂÌ‚·›ÓÔ˘Ó ÛÙË Ï¢¯·ÈÌÔÁ¤ÓÂÛË Î·È ÛÙËÓ ·ÓÙÔ¯‹
ÛÙ· Ê¿Ú̷η, ηıÒ˜ Î·È ÛÙËÓ ·Ó·ÁÓÒÚÈÛË ÂÈÚfi-
ÛıÂÙˆÓ ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ¤ÙÛÈ ÒÛÙÂ Ë Ù·-
ÍÈÓfiÌËÛË Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ Ó· Â›Ó·È ÈÔ ·ÎÚÈ‚‹˜.
∆·ÍÈÓfiÌËÛË Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘
°È· ÔÏϤ˜ ‰ÂηÂٛ˜, Ë ıÂڷ›· ‚·Û›ÛÙËΠÛÂ
ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ËÏÈΛ·, ÙÔ Ê‡-
ÏÔ, Ô ·ÚÈıÌfi˜ ÙˆÓ Ï¢ÎÒÓ ÛÙË ‰È¿ÁÓˆÛË, Ë ·ÚÔ˘-
Û›· ÓfiÛÔ˘ ÛÙÔ ∫¡™, Ô ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô˜ Î·È ÔÈ Î˘Ù-
Ù·ÚÔÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ ‚Ï·ÛÙÒÓ, ηÈ, ÌÂÙ·ÁÂ-
Ó¤ÛÙÂÚ·, Ë ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· (4,5).
™Ù· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ıÂڷ›·˜ ¤¯ÂÈ ÚÔ-
ÛÙÂı› Ë ÂÎÙ›ÌËÛË Ù˘ ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘. ∏ ËÏÈ-
Λ· Î·È Ô ·ÚÈıÌfi˜ ÙˆÓ Ï¢ÎÒÓ ÛÙË ‰È¿ÁÓˆÛË ·Ú·-
̤ÓÔ˘Ó ‰È·¯ÚÔÓÈο ÛÙ·ıÂÚÔ› Î·È ·ÓÂÍ¿ÚÙËÙÔÈ ÚÔ-
ÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Û fiÏ· Ù· ÚˆÙfiÎÔÏÏ· ıÂÚ·-
›·˜. ™‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· ÙÔ˘ NCI, Ù· ·È‰È¿
Ì √§§ µ-ÛÂÈÚ¿˜, ËÏÈΛ·˜ 1-9 ÂÙÒÓ Î·È Ì ÏÂ˘Î¿
<50.000 Îί ıˆÚÔ‡ÓÙ·È ÛÙ·ıÂÚÔ‡ ÎÈÓ‰‡ÓÔ˘, ÂÓÒ Ù·
˘fiÏÔÈ· ·È‰È¿ ηٷٿÛÛÔÓÙ·È ÛÙËÓ ÔÌ¿‰· ˘„Ë-
ÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (4). ∞ÓÙ›ÛÙÔȯ·, Î·È ÛÙ· ÚˆÙfiÎÔÏÏ·
BFM ̤¯ÚÈ Î·È ÙÔ 1995, Ë ËÏÈΛ· 1-6 ÂÙÒÓ Î·È Ô ·ÚÈı-
Ìfi˜ Ï¢ÎÒÓ <20.000 Îί ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ÙËÓ
ηٿٷÍË ÙˆÓ ·ÛıÂÓÒÓ ÛÙËÓ ÔÌ¿‰· ÛÙ·ıÂÚÔ‡ ÎÈÓ-
‰‡ÓÔ˘ (5).
∏ Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ∫¡™ ÛÙË ‰È¿ÁÓˆÛË (¶›Ó·Î·˜
1) ¤¯ÂÈ Â›Û˘ ·Ô‰Âȯı› ÛËÌ·ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜
ÛÙËÓ ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ (∂ÈÎfiÓ· 1). ∞Ú¯Èο, Ë
·Ó·ÁÓÒÚÈÛË ÙÔ˘ ÚfiÏÔ˘ Ù˘ ÚÔʇϷ͢ ÙÔ˘ ∫¡™ ‹
-ÈÔ ÛˆÛÙ¿- Ë ıÂڷ›· Ù˘ ˘ÔÎÏÈÓÈ΋˜ ÓfiÛÔ˘,
·fi Ù· Ù¤ÏË Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ 1960, Û˘Ó¤‚·Ï ٷ
̤ÁÈÛÙ· ÛÙË ‚ÂÏÙ›ˆÛË Ù˘ ÂÈ‚›ˆÛ˘/›·Û˘. ™‹ÌÂ-
Ú·, Ë ıÂڷ›· Ù˘ ˘ÔÎÏÈÓÈ΋˜ ÓfiÛÔ˘ ‚·Û›˙ÂÙ·È
ÛÙËÓ ÂÈıÂÙÈ΋ Û˘ÛÙËÌ·ÙÈ΋ ¯ËÌÂÈÔıÂڷ›·
(HDMTX ) Î·È ÛÙËÓ ÂÓ‰ÔÚÚ·¯È·›· ¯ÔÚ‹ÁËÛË ÌÂıÔ-
ÙÚÂÍ¿Ù˘ ÛÙÔ 80-90% ÙˆÓ ·È‰ÈÒÓ. ™ÙÔ˘˜ ·ÛıÂÓ›˜
˘„ËÏÔ‡ ‹ Ôχ „ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÔÚËÁÂ›Ù·È ÙÚÈÏ‹
ÂÓ‰ÔÚÚ·¯È·›· ¯ËÌÂÈÔıÂڷ›· Î·È ·ÎÙÈÓÔ‚ÔÏ›· ÎÚ·-
Ó›Ô˘ (12Gy) Û fiÏ· ۯ‰fiÓ Ù· ΤÓÙÚ·, Ù·ÎÙÈ΋ Ô˘
¤¯ÂÈ ÌÂÈÒÛÂÈ ÙÔ ÔÛÔÛÙfi ÙˆÓ ˘ÔÙÚÔÒÓ ÌÂ Û˘ÌÌÂ-
ÙÔ¯‹ ÙÔ˘ ∫¡™ Û <5% Î·È ÙˆÓ ÌÂÌÔÓˆÌ¤ÓˆÓ ˘Ô-
ÙÚÔÒÓ ∫¡™ ÛÙÔ 1,1%. ∂Í·›ÚÂÛË ·ÔÙÂÏ› ÙÔ St.
Jude, ÛÙ· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ÙÔ˘ ÔÔ›Ô˘ ‰ÂÓ ¯Ô-
ÚËÁÂ›Ù·È ·ÎÙÈÓÔ‚ÔÏ›· Ô‡Ù ÛÙÔ˘˜ ·ÛıÂÓ›˜ ˘„ËÏÔ‡
ÎÈÓ‰‡ÓÔ˘ (6,7). ∆ËÓ ÙÂÏÂ˘Ù·›· ‰ÂηÂÙ›· ·Ó·ÁÓˆÚ›-
ÛÙËΠfiÙÈ Ë ÙÚ·˘Ì·ÙÈ΋ ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙË-
ÛË ÛÙË ‰È¿ÁÓˆÛË ÂÈ‚·Ú‡ÓÂÈ ÙËÓ ÙÂÏÈ΋ Â΂·ÛË ÙˆÓ
·ÛıÂÓÒÓ, Ì ÔÛÔÛÙfi ˘ÔÙÚÔÒÓ ÙÔ˘ ∫¡™ ÛÙ· Â-
ÚÈÛÙ·ÙÈο ·˘Ù¿ ÂÚ›Ô˘ 8% (8). ŒÙÛÈ, ‹‰Ë ÛÙ· Úˆ-
ÙfiÎÔÏÏ· BFM -95 Î·È St. Jude XIII ·˘Í‹ıËÎÂ Ô ·ÚÈı-
Ìfi˜ ÙˆÓ ÂÓ‰ÔÚÚ·¯È·›ˆÓ ¯ÔÚËÁ‹ÛÂˆÓ ÛÙȘ ÂÚÈÙÒ-
ÛÂȘ ·˘Ù¤˜.
∞ÓÔÛÔÊ·ÈÓfiÙ˘Ô˜
∏ ÌÂϤÙË ÙÔ˘ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô˘ ÙˆÓ ‚Ï·ÛÙÒÓ
ÛÙË ‰È¿ÁÓˆÛË Ù˘ √§§ ¤¯ÂÈ ·ÚÔ˘ÛÈ¿ÛÂÈ ÙÂÚ¿ÛÙÈ·
ÚfiÔ‰Ô Ù· ÙÂÏÂ˘Ù·›· ÚfiÓÈ·, Ì ÙË ‰È¿ıÂÛË ÌÂÁ¿ÏÔ˘
·ÚÈıÌÔ‡ ÌÔÓÔÎψÓÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ï¢ÎÔ-
΢ÙÙ·ÚÈÎÒÓ ·ÓÙÈÁfiÓˆÓ Î·È ÙÔ Û¯Â‰È·ÛÌfi ¢·›ÛıËÙ˘
Î·È ·ÍÈfiÈÛÙ˘ ΢ÙÙ·ÚÔÌÂÙÚ›·˜ ÚÔ‹˜. ¢Â›ÎÙ˜ ȉȷ›-
ÙÂÚ· ¢·›ÛıËÙÔÈ ÁÈ· ÙË µ Î·È ∆ ÛÂÈÚ¿ ·ÓÙ›ÛÙÔȯ· ›-
Ó·È Ù· CD19 Î·È CD7 Î·È ‰Â›ÎÙ˜ Ì ÌÂÁ¿ÏË ÂȉÈÎfi-
ÙËÙ· Ù· ΢ÙÙ·ÚÔÏ·ÛÌ·ÙÈο CD79a, CD3 ÁÈ· µ ηÈ
∆ ÏÂÌÊÔ·ÙÙ·Ú·, ·ÓÙ›ÛÙÔȯ·. ªÂ ÙÔÓ ·ÓÔÛÔÊ·ÈÓfi-
Ù˘Ô ÌfiÓÔ, Ë ‰È¿ÁÓˆÛË ÌÔÚ› Ó· ÙÂı› ÛÙÔ 99% ÙˆÓ
ÂÚÈÙÒÛÂˆÓ (9).
ªÂ ‚¿ÛË ÙÔÓ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô ÙˆÓ ‚Ï·ÛÙÒÓ, Ë
·ÚÈ· ‰È¿ÎÚÈÛË Â›Ó·È ÌÂٷ͇ √§§ B-΢ÙÙ·ÚÈ΋˜
ÚÔ¤Ï¢Û˘ (µ-lineage ‹ µ-precursor ALL) (85%
ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÛÙ· ·È‰È¿) Î·È ∆-
√§§, Ô˘ ·ÔÙÂÏ› ÙÔ ˘fiÏÔÈÔ 15%. ∏ √§§ B-
΢ÙÙ·ÚÈ΋˜ ÚÔ¤Ï¢Û˘ ÌÔÚ› Ó· ‰È·ÎÚÈı› ÂÚ·È-
Ù¤Úˆ Û ÚÒÈÌË ÚÔ-µ, ÚÔ-µ, ÌÂÙ·‚·ÙÈ΋ ÚÔ-µ
∂ÈÎfiÓ· 1. §ÂÌÊÔ‚Ï¿ÛÙ˜ Û ΢ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ∂¡À.
¶›Ó·Î·˜ 1. ∆·ÍÈÓfiÌËÛË Ù˘ Û˘ÌÌÂÙÔ¯‹˜ ÙÔ˘ ∫¡™ ÛÙËÓ √§§
¡fiÛÔ˜ ÛÙÔ ∫¡™ñ CNS 1
∞Ô˘Û›· ‚Ï·ÛÙÒÓ ÛÙËÓ Î˘ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ÙÔ˘ ∂¡Àñ CNS 2
<5 WBC/Ìl ÛÙËÓ Î˘ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ÌÔÚÊÔÏÔÁÈο‚Ï¿ÛÙ˜
ñ CNS 3≥5 WBC/Ìl ÌÔÚÊÔÏÔÁÈο ‚Ï¿ÛÙ˜
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·76
77√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·
¶·È‰È·ÙÚÈ΋ 2009;72:10-15
Î·È µ-√§§ (10). ŸÛÔÓ ·ÊÔÚ¿ ÙÔÓ Û¯Â‰È·ÛÌfi Ù˘ ıÂ-
ڷ›·˜ Ù˘ √§§ µ-ÛÂÈÚ¿˜, Ë ÌfiÓË ÛËÌ·ÓÙÈ΋ ‰È¿-
ÎÚÈÛË Â›Ó·È ÌÂٷ͇ ÒÚÈÌˆÓ µ Î·È Úfi‰ÚÔÌˆÓ µ-΢Ù-
Ù¿ÚˆÓ. ∞Ó¿ÏÔÁ·, Ë ∆-√§§ ÌÔÚ› Ó· Ù·ÍÈÓÔÌËı› ÌÂ
‚¿ÛË ÙÔ ÛÙ¿‰ÈÔ ‰È·ÊÔÚÔÔ›ËÛ˘ ÙˆÓ ı˘ÌÔ΢ÙÙ¿ÚˆÓ
‹ ÙÔ ÛÙ¿‰ÈÔ ¤ÎÊÚ·Û˘ ÙÔ˘ ˘Ô‰Ô¯¤· ÙÔ˘ ∆-΢ÙÙ¿-
ÚÔ˘, ·ÏÏ¿ ·˘Ù‹ Ë Ù·ÍÈÓfiÌËÛË ¤¯ÂÈ ÂÚÈÔÚÈṲ̂ÓË
ÎÏÈÓÈ΋ ¯ÚËÛÈÌfiÙËÙ·.
∫˘ÙÙ·ÚÔÁÂÓÂÙÈο ·Ú·ÎÙËÚÈÛÙÈο
™ÙÔ 90% Ù˘ √§§ ·Ú·ÙËÚÔ‡ÓÙ·È ÂȉÈΤ˜ ΢ÙÙ·-
ÚÔÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜, ·ÚÈıÌËÙÈΤ˜ ‹ ‰ÔÌÈΤ˜. ∏
΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ‚Ï·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
¤¯ÂÈ ÌÂÁ¿ÏË ÛËÌ·Û›· ÛÙË ‰È¿ÁÓˆÛË, ıÂڷ›· ηÈ
ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ (11). ∫ÏÈÓÈο ÛËÌ·ÓÙÈΤ˜ ΢Ù-
Ù·ÚÔÁÂÓÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ Â›Ó·È Ô ˘ÂÚ‰ÈÏÔÂȉÈ-
ÛÌfi˜, Ô ˘Ô‰ÈÏÔÂȉÈÛÌfi˜, ÔÈ ¯ÚˆÌÔÛˆÌÈΤ˜ ÌÂÙ·-
ı¤ÛÂȘ fiˆ˜ t(12;21), t(1;19), t(9;22), Ì ·ÓÙ›ÛÙÔȯ·
ÚÔ˚fiÓÙ· Û‡ÓÙË͢ ÙˆÓ ÁÔÓȉ›ˆÓ TEL-AML1, E2A-
PBX1, BCR-ABL Î·È ÌÈ· ÔÈÎÈÏ›· ·Ó·‰È·Ù¿ÍÂˆÓ ÙÔ˘
MLL ÁÔÓȉ›Ô˘. √ ˘ÂÚ‰ÈÏÔÂȉÈÛÌfi˜ (>50 ¯ÚˆÌÔ-
ÛÒÌ·Ù· ‹ DNA index ≥1,16 ), ηıÒ˜ Î·È ÔÈ ÙÚÈÛˆ-
̛˜ 4,10,17 ·ÏÏ¿ ΢ڛˆ˜ Ë ÙÚÈÏ‹ ÙÚÈۈ̛· 4,10,17,
¤¯Ô˘Ó ıÂÙÈ΋ ›وÛË ÛÙËÓ ¤Î‚·ÛË, ÂÓÒ Ô ˘Ô‰È-
ÏÔÂȉÈÛÌfi˜ (<45 ¯ÚˆÌÔÛÒÌ·Ù· Ì DI <0,95 ‹ <44
¯ÚˆÌÔÛÒÌ·Ù· Î·È DI <0,81) Û¯ÂÙ›˙ÂÙ·È Ì η΋
ÚfiÁÓˆÛË (12-14) (∂ÈÎfiÓ· 2).
∏ ÌÂÙ¿ıÂÛË t(12;21) ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 25% Ù˘
√§§ ΢ڛˆ˜ µ ÛÂÈÚ¿˜ Î·È Ô ÚÔÁÓˆÛÙÈÎfi˜ Ù˘ ÚfiÏÔ˜
‹Ù·Ó ÁÈ· ÔÏÏ¿ ¯ÚfiÓÈ· ·ÌÊÈÏÂÁfiÌÂÓÔ˜. ∞Ú¯Èο ıˆ-
Ú‹ıËΠ¢ÓÔ˚Îfi˜, Ì ÂÈ‚›ˆÛË >90% ÛÙËÓ ÔÌ¿‰·
·˘Ù‹, ÂÓÒ ·ÎÔÏÔ‡ıËÛ·Ó ·Ó·ÊÔÚ¤˜ ÁÈ· ·˘ÍË̤ÓË Û˘-
¯ÓfiÙËÙ· fi„ÈÌˆÓ ˘ÔÙÚÔÒÓ, ¤ÙÛÈ ÒÛÙÂ Ë ÂÈ‚›ˆÛË
Ó· ÌË ‰È·Ê¤ÚÂÈ ÌÂÙ¿ ·fi Ì·ÎÚ¿ ÂÚ›Ô‰Ô ·Ú·ÎÔ-
ÏÔ‡ıËÛ˘ (15,16). ¶ÚfiÛÊ·Ù·, ÔÈ ‰È·ÊÔÚ¤˜ ÛÙËÓ
¤Î‚·ÛË ·Ô‰›‰ÔÓÙ·È ÛÙÔ Â›‰Ô˜ Î·È ÙËÓ ÂÈıÂÙÈÎfiÙË-
Ù· ÙˆÓ ıÂڷ¢ÙÈÎÒÓ ÚˆÙÔÎfiÏψÓ. ∫·Ï‡ÙÂÚË
¤Î‚·ÛË Û¯ÂÙ›˙ÂÙ·È Ì ˘„ËϤ˜ ‰fiÛÂȘ ÌÂıÔÙÚÂÍ¿Ù˘
Î·È L-·Û·Ú·ÁÈÓ¿Û˘. ¶ÚÔÔÙÈ΋ ÌÂϤÙË ¤‰ÂÈÍ fiÙÈ
Ì ÂÈıÂÙÈ΋ ¯ËÌÂÈÔıÂڷ›· ÛÙ· ·È‰È¿ ·˘Ù¿, Ë
5ÂÙ‹˜ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÊÙ¿ÓÂÈ ÙÔ 97%, ¯ˆÚ›˜
fï˜ Ë ÌÂÙ¿ıÂÛË TEL-AML1 Ó· ·Ô‰ÂÈÎÓ‡ÂÙ·È ·ÓÂ-
Í¿ÚÙËÙÔ˜ ÚÔÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ fiÙ·Ó Ï·Ì‚¿ÓÔ-
ÓÙ·È ˘fi„Ë Ë ËÏÈΛ· Î·È Ô ·ÚÈıÌfi˜ Ï¢ÎÒÓ (17).
∏ ‰Â‡ÙÂÚË ÛÂ Û˘¯ÓfiÙËÙ· ÌÂÙ¿ıÂÛË ÛÙËÓ √§§
ÛÙ· ·È‰È¿, Ë t(1;19), Û¯ÂÙ›˙ÂÙ·È Ì ÔÛÔÛÙ¿ ›·Û˘
̤¯ÚÈ 90%, ·ÚfiÏÔ Ô˘ ÛÙÔ ·ÚÂÏıfiÓ Â›¯Â ıˆÚË-
ı› ‰˘ÛÌÂÓ‹˜ ÚÔÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ (18).
∏ t(9;22) ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 3% ÂÚ›Ô˘ ÙˆÓ ·È-
‰ÈÒÓ Ì √§§, ·ÔÙÂÏ› ‰˘ÛÌÂÓ‹ ÚÔÁÓˆÛÙÈÎfi ·-
Ú¿ÁÔÓÙ· Î·È Û¯ÂÙ›˙ÂÙ·È Ì ¯·ÌËÏ¿ ÔÛÔÛÙ¿ ›Ù¢-
͢ ‡ÊÂÛ˘, Û˘¯Ó¤˜ Î·È ÚÒÈ̘ ˘ÔÙÚÔ¤˜ ηÈ
ÊÙˆ¯‹ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË (19). ™Ù· ·È‰È¿ ·˘Ù¿, Ë
ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ, ·ÎfiÌ· Î·È ·fi
ÌË Û˘ÁÁÂÓ‹ ‰fiÙË, ıˆÚÂ›Ù·È ıÂڷ›· ÂÎÏÔÁ‹˜ ÁÈ·
›·ÛË, ÂÓÒ Ë ¯ÔÚ‹ÁËÛË ÈÌ·ÙÈÓ›Ì˘ Û˘ÓÈÛÙ¿Ù·È Û˘Á-
¯ÚfiÓˆ˜ Ì ÙË ¯ËÌÂÈÔıÂڷ›· (20).
√È ·Ó·‰È·Ù¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ MLL ·Ú·ÙËÚÔ‡-
ÓÙ·È ÛÙÔ 8% ÙˆÓ ·È‰ÈÒÓ Ì √§§, ÂÓÒ ·ÔÙÂÏÔ‡Ó
ÙËÓ ÈÔ Û˘¯Ó‹ ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹ ÛÙ· ‚Ú¤ÊË. ∏ ÌÂ-
Ù¿ıÂÛË t(4;11) [MLL-AF4] Â›Ó·È Ë ÈÔ Û˘¯Ó‹ ÛÙËÓ
ÔÌ¿‰· ·˘Ù‹ Î·È Û¯ÂÙ›˙ÂÙ·È Ì ‰˘ÛÌÂÓ‹ ÚfiÁÓˆÛË
(21). ∞Ó Î·È ÛÙÔ ·ÚÂÏıfiÓ Ë √§§ ÛÙ· ‚Ú¤ÊË ÁÂÓÈο
Û˘Û¯ÂÙ›ÛÙËΠ̠η΋ ÚfiÁÓˆÛË, Û‹ÌÂÚ· ÁÓˆÚ›-
˙Ô˘Ì fiÙÈ, ÌÂÙ¿ ÙË ¯Ú‹ÛË ÂȉÈÎÒÓ ÈÔ ÂÈıÂÙÈÎÒÓ
ÚˆÙÔÎfiÏÏˆÓ ÁÈ· Ù· ‚Ú¤ÊË, η΋ ÚfiÁÓˆÛË ¤¯Ô˘Ó
ÌfiÓÔ ÂΛӷ Ì MLL ·Ó·‰È·Ù¿ÍÂȘ (22,23).
∆Ô 12%-14% Ù˘ √§§ ÛÙ· ·È‰È¿ Â›Ó·È ∆- ÛÂÈ-
Ú¿˜. ∏ ∆-√§§ Â›Ó·È ÈÔ Û˘¯Ó‹ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ ηÈ
Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ Ì ˘„ËÏfi ·ÚÈıÌfi Ï¢ÎÒÓ Î·È Â›-
Û˘ Û˘Óԉ‡ÂÙ·È Û˘¯ÓfiÙÂÚ· ·fi ‰È‡ڢÓÛË ÌÂÛÔ-
ıˆÚ·Î›Ô˘, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·
Î·È ‰È‹ıËÛË ∫¡™. ªÂ ÙË ¯ÔÚ‹ÁËÛË ÂÈıÂÙÈ΋˜ ¯Ë-
ÌÂÈÔıÂڷ›·˜, Ë ‰˘ÛÌÂÓ¤ÛÙÂÚË ÚfiÁÓˆÛË Û ۯ¤-
ÛË Ì ÙËÓ √§§ µ-ÛÂÈÚ¿˜ ¤¯ÂÈ ‚ÂÏÙȈı›, Ì ÔÛÔÛÙ¿
›·Û˘ 75% (24,25). ™ÙËÓ ÔÌ¿‰· ·˘Ù‹, Ë ËÏÈΛ· Î·È Ô
·ÚÈıÌfi˜ Ï¢ÎÒÓ ‰ÂÓ Ê·›ÓÂÙ·È Ó· ¤¯Ô˘Ó ÈÛ¯˘Ú‹ ÚÔ-
ÁÓˆÛÙÈ΋ ·Í›·, fiˆ˜ ›Û˘ ‰ÂÓ Â›Ó·È Û·Ê‹˜ Ô ÚÔ-
ÁÓˆÛÙÈÎfi˜ ÚfiÏÔ˜ ÙˆÓ Î˘ÙÙ·ÚÔÁÂÓÂÙÈÎÒÓ ‰È·Ù·Ú·-
¯ÒÓ (24). ªÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ NOTCH ¤¯Ô˘Ó
·Ú·ÙËÚËı› ÛÙÔ 50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ∆-√§§,
·ÏÏ¿ Ë ÚÔÁÓˆÛÙÈ΋ ÙÔ˘˜ ÛËÌ·Û›· Â›Ó·È ·Û·Ê‹˜
(27-28).
¶ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· Î·È ÂÏ¿¯ÈÛÙË
˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ˜
∏ ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· ·ÔÙÂÏ›
ÈÛ¯˘Úfi ÚÔÁÓˆÛÙÈÎfi ·Ú¿ÁÔÓÙ·, ÁÈ·Ù› Û¯ÂÙ›˙ÂÙ·È ÌÂ
ÙËÓ Â˘·ÈÛıËÛ›· ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙ· Ê¿Ú̷η, ηıÒ˜
Î·È Ì ٷ Ê·ÚÌ·ÎÔ‰˘Ó·ÌÈο-Ê·ÚÌ·ÎÔÁÂÓÂÙÈο ¯·-
Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ·ÛıÂÓÔ‡˜ (29). ¶·Ú¿ÌÂÙÚÔÈ Ô˘
¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·¿-
ÓÙËÛ˘ ÛÙË ıÂڷ›· Â›Ó·È Ô ·ÚÈıÌfi˜ ÙˆÓ ‚Ï·ÛÙÒÓ
ÛÙÔ ·›Ì· ÙËÓ 8Ë Ë̤ڷ ıÂڷ›·˜, Ô Ì˘ÂÏfi˜ ÙËÓ 7Ë ‹
14Ë Ë̤ڷ ηÈ, ÚfiÛÊ·Ù·, Ë ÂÎÙ›ÌËÛË Ù˘ ÂÏ¿¯ÈÛÙ˘
˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ (30-34).
∏ ÔÌ¿‰· ÙÔ˘ BFM ÂÈÛ‹Á·Á ·fi ÙÔ 1983 ÙËÓ
ÚÔÁÓˆÛÙÈ΋ ·Í›· Ù˘ ·¿ÓÙËÛ˘ ÛÙËÓ Ú‰ÓÈ˙fiÓË.
∆· ÚˆÙfiÎÔÏÏ· BFM ¯ÚËÛÈÌÔÔÈÔ‡Ó ÙË Ì›ˆÛË ÙÔ˘
·ÚÈıÌÔ‡ ÙˆÓ ‚Ï·ÛÙÒÓ Û <1000/Ìl ÙËÓ 8Ë Ë̤ڷ, ÌÂ-
Ù¿ ·fi ¯ÔÚ‹ÁËÛË Ú‰ÓÈ˙fiÓ˘ Î·È ÌÈ·˜ ÂÓ‰ÔÚÚ·¯È-
·›·˜ ¤Á¯˘Û˘ MTX, ˆ˜ ÎÚÈÙ‹ÚÈÔ ÁÈ· ¢ÓÔ˚΋ Úfi-
ÁÓˆÛË (35). √ Ú˘ıÌfi˜ οı·ÚÛ˘ ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙÔ
ÂÚÈÊÂÚÈÎfi ·›Ì· ¤¯ÂÈ ÚÔÁÓˆÛÙÈ΋ ·Í›· Û ∆- Î·È µ-
ÛÂÈÚ¿˜ √§§. √ ·ÚÈıÌfi˜ ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙÔ ·›Ì· 7 Ë̤-
Ú˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ÔÏ˘·Ú·ÁÔÓÙÈ΋˜ ıÂڷ›·˜
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·77
·ÔÙÂÏ› ›Û˘ ÚÔÁÓˆÛÙÈÎfi ·Ú¿ÁÔÓÙ· Û ¿ÏÏ·
ÚˆÙfiÎÔÏÏ· (32).
ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ ·¿ÓÙËÛË ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ
ÔÛÙÒÓ, ÔÈ ·ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó Ì›ˆÛË ÙˆÓ ‚Ï·ÛÙÒÓ
Û <5% Û 7-14 Ë̤Ú˜ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÔÏ˘-
·Ú·ÁÔÓÙÈ΋˜ ¯ËÌÂÈÔıÂڷ›·˜ ¤¯Ô˘Ó ȉȷ›ÙÂÚ· ¢-
ÓÔ˚΋ ÚfiÁÓˆÛË. ∏ ÌÔÚÊÔÏÔÁÈ΋ ·¿ÓÙËÛË ÛÙË ıÂ-
ڷ›· ÂÍ·ÎÔÏÔ˘ı› Ó· ¤¯ÂÈ ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›·
Î·È ÛÙ· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ÙÔ˘ COG (37).
∏ ÂÎÙ›ÌËÛË Ù˘ ÚÒÈÌ˘ ·¿ÓÙËÛ˘ ÛÙË ıÂڷ›·,
Ì ÙÔÓ ˘ÔÏÔÁÈÛÌfi Ù˘ MRD ÛÙÔÓ Ì˘ÂÏfi, ·ÔÙÂÏ›
Û‹ÌÂÚ· ÙÔÓ ÈÔ ÈÛ¯˘Úfi ›Ûˆ˜ ÚÔÁÓˆÛÙÈÎfi ·Ú¿ÁÔÓÙ·,
Ô˘ ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ÁÈ· ·ÎÚÈ‚‹ Ù·ÍÈÓfiÌËÛË ÛÂ
ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ Î·È ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂڷ›·˜
(34,38-40). √È ·ÛıÂÓ›˜ Ô˘ ÂÈÙ˘Á¯¿ÓÔ˘Ó ·ÓÔÛÔÏÔÁÈ-
΋ ‹ ÌÔÚȷ΋ ‡ÊÂÛË, Ô˘ ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ˆ˜ Ì›ˆÛË
ÙˆÓ ‚Ï·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Û <0,01% ÙˆÓ Â̇ÚËÓˆÓ
΢ÙÙ¿ÚˆÓ ÙÔ˘ Ì˘ÂÏÔ‡ ÛÙÔ Ù¤ÏÔ˜ Ù˘ ·ÁˆÁ‹˜ ÛÙËÓ
‡ÊÂÛË, ¤¯Ô˘Ó ÂÍ·ÈÚÂÙÈ΋ ÚfiÁÓˆÛË (41,42).
√§§ Û ÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ
πÛÙÔÚÈο, Ù· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ ÌÂ
√§§ ¤¯Ô˘Ó ¯ÂÈÚfiÙÂÚË ÚfiÁÓˆÛË, Û ۯ¤ÛË Ì ٷ ÌÈ-
ÎÚfiÙÂÚ· ·È‰È¿. µÈÔÏÔÁÈο, ÔÈ ¤ÊË‚ÔÈ ‰È·Ê¤ÚÔ˘Ó
·fi Ù· ÌÈÎÚfiÙÂÚ· ·È‰È¿ Î·È ÚÔÛ¤Ú¯ÔÓÙ·È Ì ‰˘-
ÛÌÂÓ›˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ηٿ ÙË ‰È¿-
ÁÓˆÛË, fiˆ˜ ˘„ËÏfi˜ ·ÚÈıÌfi˜ Ï¢ÎÒÓ, ∆ ·ÓÔÛÔÊ·È-
ÓfiÙ˘Ô˜ Î·È ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· ‰˘ÛÌÂÓÒÓ Î˘Ù-
Ù·ÚÔÁÂÓÂÙÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ (43). √È ‰È·ÊÔÚ¤˜, fï˜,
ÛÙËÓ ¤Î‚·ÛË ·Ô‰›‰ÔÓÙ·È Î·È ÛÙË ÏÈÁfiÙÂÚÔ ÂÈıÂÙÈ-
΋ ıÂڷ›· Ô˘ Ï·Ì‚¿ÓÔ˘Ó fiÙ·Ó ıÂڷ‡ÔÓÙ·È ÌÂ
ÚˆÙfiÎÔÏÏ· ÂÓËϛΈÓ. ¶ÚfiÛÊ·Ù· ‰ËÌÔÛȇÙËÎ·Ó 3
·Ó·‰ÚÔÌÈΤ˜ ÌÂϤÙ˜ ·fi ÙËÓ ∂˘ÚÒË Î·È Ì›· ÚÔ-
‰ÚÔÌÈ΋ ·fi ÙÔ DFCI ÛÙȘ ∏¶∞, Ô˘ ‰Â›¯ÓÔ˘Ó fiÙÈ ÔÈ
¤ÊË‚ÔÈ Î·È ÔÈ Ó·ÚÔ› ÂÓ‹ÏÈΘ Ì √§§ ¤¯Ô˘Ó ÛËÌ·-
ÓÙÈο ηχÙÂÚ· ÔÛÔÛÙ¿ ›·Û˘ fiÙ·Ó ıÂڷ‡ÔÓÙ·È
Ì ·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ· (44-47). ∏ Û‡ÁÎÚÈÛË
Ù˘ 5ÂÙÔ‡˜ ÂÈ‚›ˆÛ˘ ·ÛıÂÓÒÓ 15-21 ÂÙÒÓ ¤‰ÂÈÍÂ
̤¯ÚÈ Î·È 30% ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ›·Û˘ Ì ٷ
121086420
100
90
80
70
60
50
40
30
20
10
0
ŒÙË ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË
∂
È‚›ˆ
ÛË
ÂÏ
‡ı
ÂÚË
Û˘
Ì‚
·Ì
¿Ù
ˆÓ
(%
)
E2A-PXB1 (n=19)Hyperdiploidy >50 chromosomes (n=112)
Other subtypes (n=148)
T-cell ALL (n=78)
MLL-AF4 (n=15)BCR-AµL (n=14)
TEL-AML1 (n=81)
∂ÈÎfiÓ· 2. ∫·Ì‡Ï˜ ÂÈ‚›ˆÛ˘ ˘ÔÔÌ¿‰ˆÓ Ì ‚¿ÛË Ù· ΢ÙÙ·ÚÔÁÂÓÂÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο (∞fi Pui C et al. NEJM 2004).
78 ∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹
Paediatriki 2009;72:10-15
¶›Ó·Î·˜ 2. ∞ÓÔÛÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË √§§
ÀÔÔÌ¿‰· ŒÎÊÚ·ÛË ·ÓÙÈÁfiÓˆÓ ™˘¯ÓfiÙËÙ· (%)
¶ÚÒÈÌË ÚÔ-B CD19+, CD22+, CD79a+, CD10+/-, CD7-, CD3-a, 57-65%cIgÌ-, sIgÎ-, sIgÏ-
¶ÚÔ-µ CD19+, CD22+, CD79a+,CD10+/-, CD7-,CD3-a, 20-25%cIgÌ+, sIgÌ-, sIgÎ-, sIgÏ-
ªÂÙ·‚·ÙÈ΋ ÚÔ-µ CD19+, CD22+, CD79a+, CD10+/-b, CD7-, CD3-a, 2-3%cIgÌ+, sIgÌ+, sIgÎ-, sIgÏ-
B- √§§ CD19+, CD22+, CD79a+, CD10+/-b, CD7-, CD3-a, 2-3%cIgÌ+, sIgÌ+, sIgÎ+, sIgÏ+
T- √§§ CD19+/-c, CD22-, CD79a-, CD10+/-b, 13-15%CD7+, CD3+a, cIgÌ-, sIgÌ-, sIgÎ-, sIgÏ-
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·78
79√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·
¶·È‰È·ÙÚÈ΋ 2009;72:10-15
·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ· ıÂڷ›·˜. ∞Ó Î·È ÔÈ ‰È·-
ÊÔÚ¤˜ ·˘Ù¤˜ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ÂÚÌËÓ¢ıÔ‡Ó ÌfiÓÔ
·fi Ù· ‚ÈÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο Î·È ÙȘ ÁÂÓÂÙÈΤ˜
‰È·Ù·Ú·¯¤˜, Â›Ó·È È‰È·›ÙÂÚ· ·ÍÈÔÛËÌ›ˆÙÔ ÙÔ fiÙÈ ÔÈ
¤ÊË‚ÔÈ Î·È ÔÈ Ó·ÚÔ› ÂÓ‹ÏÈΘ, fiÙ·Ó ÂÓÙ¿ÛÔÓÙ·È ÛÂ
ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Î·È ıÂڷ‡ÔÓÙ·È Ì ·È‰È·ÙÚÈο
ÚˆÙfiÎÔÏÏ·, ÌÔÚ› Ó· ÂÈÙ‡¯Ô˘Ó ÔÛÔÛÙ¿ ›·Û˘
Ô˘ Êı¿ÓÔ˘Ó ÙÔ 70%.
∆Ú¤¯Ô˘Û· ıÂڷ¢ÙÈ΋ ÛÙÚ·ÙËÁÈ΋ Î·È Ó¤Â˜
ÚÔÁÓˆÛÙÈΤ˜ ÔÌ¿‰Â˜
H Ù·ÍÈÓfiÌËÛË ÙˆÓ ·È‰ÈÒÓ Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘
¤¯ÂÈ ˆ˜ ÛÙfi¯Ô ÙËÓ ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂڷ›·˜, ¤ÙÛÈ
ÒÛÙ ÈÔ ÂÈıÂÙÈ΋ ηÈ, ÂÔ̤ӈ˜, ÈÔ ÙÔÍÈ΋ ıÂÚ·-
›· Ó· Ï·Ì‚¿ÓÔ˘Ó ÌfiÓÔ Ù· ·È‰È¿ Ô˘ ÙË ¯ÚÂÈ¿˙Ô-
ÓÙ·È ÁÈ· Ó· È·ıÔ‡Ó.
∏ ÚfiÔ‰Ô˜ ÛÙË ÌÔÚȷ΋ Ù·ÍÈÓfiÌËÛË Î·È ÙËÓ ·Ó›-
¯Ó¢ÛË ÁÂÓÂÙÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ Ô‰ËÁ› ÛÙËÓ ·Ó·˙‹-
ÙËÛË Ô‰ÒÓ Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ·ÓÙ›ÛÙ·ÛË ÛÙË ıÂÚ·-
›·, ·ÏÏ¿ Î·È Ó¤ˆÓ ıÂڷ¢ÙÈÎÒÓ ÛÙfi¯ˆÓ (48). ™Ë-
Ì·ÓÙÈÎfi ·Ú¿‰ÂÈÁÌ· ·ÔÙÂÏ› Ë ¯ÔÚ‹ÁËÛË ÙÔ˘
imatinib mesylate ÛÙ· ·È‰È¿ Ì √§§ Î·È Û‡ÓÙËÍË
ÙˆÓ bcr-abl ÁÔÓȉ›ˆÓ (20).
∆· ÚˆÙfiÎÔÏÏ· ÙÔ˘ BFM ηٷٿÛÛÔ˘Ó ÛÙËÓ
ÔÌ¿‰· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Ù· ·È‰È¿ Ì ÊÙˆ¯‹ ·¿-
ÓÙËÛË ÛÙËÓ Ú‰ÓÈ˙fiÓË, ηıÒ˜ Î·È Ù· ·È‰È¿ Ô˘
¤¯Ô˘Ó ÙȘ ÌÂÙ·ı¤ÛÂȘ t(9;22) Î·È t(4;11). ™ÙÔ ÚˆÙfi-
ÎÔÏÏÔ ALL BFM 2000, ÔÈ ·ÛıÂÓ›˜ Ù·ÍÈÓÔÌÔ‡ÓÙ·È ÌÂ
‚¿ÛË fi¯È ÌfiÓÔ ÙËÓ ·¿ÓÙËÛË ÛÙËÓ Ú‰ÓÈ˙fiÓË Î·È
ÙȘ ΢ÙÙ·ÚÔÁÂÓÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, ·ÏÏ¿ Î·È ÙËÓ
MRD ÙËÓ Â‚‰ÔÌ¿‰· 4 Î·È 12. ŒÙÛÈ, ˜ ÛÙ·ıÂÚÔ‡ ÎÈÓ-
‰‡ÓÔ˘ ηٷٿÛÛÔÓÙ·È Ù· ·È‰È¿ Ì ·ÚÓËÙÈ΋ MRD
ÙËÓ 4Ë Î·È 12 ‚‰ÔÌ¿‰·, ÂӉȿÌÂÛÔ˘ ÎÈÓ‰‡ÓÔ˘ Ù·
·È‰È¿ Ì MRD <10-3 Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Ù· ·È-
‰È¿ Ì MRD ≥10-3 ÙË 12Ë Â‚‰ÔÌ¿‰·. ∆· ·È‰È¿ ÌÂ
ÊÙˆ¯‹ ·¿ÓÙËÛË ÛÙËÓ Ú‰ÓÈ˙fiÓË ıˆÚÔ‡ÓÙ·È
˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔÓ ÚÔÛ‰ÈÔÚÈ-
ÛÌfi Ù˘ MRD, ÂÓÒ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ›Û˘ ıˆ-
ÚÔ‡ÓÙ·È ÔÈ ·ÛıÂÓ›˜ Ì ÌÂÙ¿ıÂÛË t(9;22) Î·È t(4;11),
·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ·¿ÓÙËÛË ÛÙË ıÂڷ›· (49).
∞fi ÙÔ DFCI ¤¯ÂÈ ÚÔÙ·ı› ›Û˘ Ó¤Ô Û‡ÛÙËÌ·
Ù·ÍÈÓfiÌËÛ˘ ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì √§§ µ-ÛÂÈÚ¿˜. √È
·ÛıÂÓ›˜ ·Ú¯Èο Ù·ÍÈÓÔÌÔ‡ÓÙ·È ˆ˜ ÛÙ·ıÂÚÔ‡ ‹ ˘„Ë-
ÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Ì ‚¿ÛË ÙËÓ ËÏÈΛ·, ÙÔÓ ·ÚÈıÌfi ÙˆÓ
Ï¢ÎÒÓ Î·È ÙËÓ ·ÚÔ˘Û›· ÓfiÛÔ˘ ÛÙÔ ∫¡™. ªÂÙ¿ ÙË
¯ÔÚ‹ÁËÛË ÙˆÓ 4 ‚‰ÔÌ¿‰ˆÓ Ù˘ ıÂڷ›·˜ ·Áˆ-
Á‹˜ ÛÙËÓ ‡ÊÂÛË, ÔÈ ·ÛıÂÓ›˜ Ì MRD ≥0,1% Ù·ÍÈÓÔ-
ÌÔ‡ÓÙ·È ˆ˜ Ôχ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÂÓÒ ÂΛÓÔÈ ÌÂ
MRD <0,1% Û˘Ó¯›˙Ô˘Ó fiˆ˜ Ù·ÍÈÓÔÌ‹ıËÎ·Ó ·Ú¯È-
ο. ™Ùfi¯Ô˜ Â›Ó·È Ó· ÚÔÛ‰ÈÔÚÈÛı› Â¿Ó Ë ÂÓÙ·ÙÈÎÔ-
Ô›ËÛË Ù˘ ıÂڷ›·˜ ı· ‚ÔËı‹ÛÂÈ ÙÔ˘˜ ·ÛıÂÓ›˜
Ì ˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ ÛÙÔ Ù¤ÏÔ˜ Ù˘ ·ÁˆÁ‹˜
ÛÙËÓ ‡ÊÂÛË. √È ·ÛıÂÓ›˜ Ì ∆-√§§ Ï·Ì‚¿ÓÔ˘Ó ıÂ-
ڷ›· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ MRD
ÛÙË Ê¿ÛË ·˘Ù‹. ø˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÓÙÈÌÂÙˆ›˙Ô-
ÓÙ·È Â›Û˘ ÔÈ ·ÛıÂÓ›˜ Ì ˘Ô‰ÈÏÔÂȉÈÛÌfi, ·ÓÂ-
Í¿ÚÙËÙ· ·fi ÙÔÓ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô ‹ ÙËÓ ÂÏ¿¯ÈÛÙË
˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ.
™ÙÔ St. Jude Children’s Research Hospital, Ë Ù·ÍÈ-
ÓfiÌËÛË Â›Û˘ ‚·Û›˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙ· ›‰· Ù˘
MRD ÌÂÙ¿ ·fi 6 ‚‰ÔÌ¿‰Â˜ ıÂڷ›·˜ ·ÁˆÁ‹˜
ÛÙËÓ ‡ÊÂÛË Î·È ‰È·ÎÚ›ÓÂÈ ÙÔ˘˜ ·ÛıÂÓ›˜ Û ¯·ÌËÏÔ‡
(MRD <0,01%), ÛÙ·ıÂÚÔ‡ (MRD 0,01% ¤ˆ˜ <1%)
Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (MRD >1%).
To 2000, Ë ‰ËÌÈÔ˘ÚÁ›· ÙÔ˘ COG ·fi ÙË Û˘Ó¤Óˆ-
ÛË ÙÔ˘ CCG Î·È ÙÔ˘ POG ¤‰ˆÛ ÙË ‰˘Ó·ÙfiÙËÙ· Ó·
·Ó·Ï˘ıÔ‡Ó ·Ó·‰ÚÔÌÈο Ù· ÎÏÈÓÈο ‚ÈÔÏÔÁÈο ‰Â‰Ô-
̤ӷ Î·È Ë ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· 18.000
·ÛıÂÓÒÓ Ì √§§, ËÏÈΛ·˜ 1-21.99 ÂÙÒÓ, Î·È Ó· ÚÔ-
Ù·ı› ¤Ó· Ó¤Ô Û‡ÛÙËÌ· Ù·ÍÈÓfiÌËÛ˘ (37). ªÂ ‚¿ÛË
ÙÔ ÚÔÙÂÈÓfiÌÂÓÔ Û‡ÛÙËÌ·, ÔÈ ·ÛıÂÓ›˜ Ì √§§ µ-
ÛÂÈÚ¿˜ ‰È·ÎÚ›ÓÔÓÙ·È Û ¯·ÌËÏÔ‡, ÛÙ·ıÂÚÔ‡, ˘„ËÏÔ‡
Î·È Ôχ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ·,
ÙÔÓ ·ÚÈıÌfi ÙˆÓ Ï¢ÎÒÓ, ÙȘ ΢ÙÙ·ÚÔÁÂÓÂÙÈΤ˜ ‰È·-
Ù·Ú·¯¤˜, ÙÔÓ Ì˘ÂÏfi Ù˘ Ë̤ڷ˜ 14 Î·È ÙËÓ MRD ÛÙÔ
Ù¤ÏÔ˜ Ù˘ ·ÁˆÁ‹˜ ÛÙËÓ ‡ÊÂÛË.
∞Ú¯Èο ‰È·ÎÚ›ÓÔÓÙ·È Û ÛÙ·ıÂÚÔ‡ Î·È ˘„ËÏÔ‡
ÎÈÓ‰‡ÓÔ˘ Ì ‚¿ÛË ÙËÓ ËÏÈΛ· Î·È ÙÔÓ ·ÚÈıÌfi ÙˆÓ
Ï¢ÎÒÓ. √È ·ÛıÂÓ›˜ Ì ÌÂÙ¿ıÂÛË t(12;21) ‹ ÙÚÈÛˆ-
̛˜ 4, 10 Î·È 17 ıˆÚÔ‡ÓÙ·È "standard risk-low".
ŸÏ· Ù· ·È‰È¿ Ì ∆-√§§ Ù·ÍÈÓÔÌÔ‡ÓÙ·È ˆ˜ ˘„ËÏÔ‡
ÎÈÓ‰‡ÓÔ˘, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔÓ ·ÚÈıÌfi ÙˆÓ Ï¢ÎÒÓ.
∏ ÙÂÏÈ΋ Ù·ÍÈÓfiÌËÛË ÛÙË Û˘Ó¤¯ÂÈ· ‚·Û›˙ÂÙ·È ÛÙËÓ
ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· Î·È ÛÙ· ΢ÙÙ·ÚÔÁÂ-
ÓÂÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο. ∏ ÔÌ¿‰· ÙˆÓ Ôχ ˘„ËÏÔ‡
ÎÈÓ‰‡ÓÔ˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ·ÛıÂÓ›˜ Ì t(9;22), ˘Ô‰È-
ÏÔÂȉÈÛÌfi (<44 ¯ÚˆÌÔÛÒÌ·Ù·), MLL ·Ó·‰È·Ù¿-
ÍÂȘ, ·ÚÁ‹ ÌÔÚÊÔÏÔÁÈ΋ ·¿ÓÙËÛË, ª3 Ì˘ÂÏfi ÙËÓ
Ë̤ڷ 29 ‹ ª2 Ì˘ÂÏfi ‹/Î·È MRD >1% ÙȘ Ë̤Ú˜ 29
Î·È 43.
ŸÏ· Ù· Û˘ÛÙ‹Ì·Ù· Ù·ÍÈÓfiÌËÛ˘ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô
Ó· ‚ÂÏÙÈÒÛÔ˘Ó Ù· ÔÛÔÛÙ¿ ›·Û˘ ÙˆÓ ·ÛıÂÓÒÓ Ô˘
‰È·ÙÚ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚÔ Î›Ó‰˘ÓÔ ˘ÔÙÚÔ‹˜ Ì ÙËÓ
ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂڷ›·˜ ÙÔ˘˜ Î·È Ó· ÂÚÈÔÚ›-
ÛÔ˘Ó ÙËÓ ÙÔÍÈÎfiÙËÙ· Ù˘ ıÂڷ›·˜ Û ·ÛıÂÓ›˜ ¯·-
ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÌÂÈÒÓÔÓÙ·˜ ÂÚ·ÈÙ¤Úˆ ÙË ¯ËÌÂÈÔ-
ıÂڷ›·.
µÈ‚ÏÈÔÁÚ·Ê›·
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82 ∞¡∞™∫√¶∏™∏ REVIEW ARTICLE
Paediatriki 2009;72:82-000
§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ - ¡ÂfiÙÂÚ· ıÂڷ¢ÙÈο ‰Â‰Ô̤ӷ
µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹
¶ÂÚ›ÏË„Ë: ∆· ÏÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ ·ÔÙÂÏÔ‡Ó ÙËÓ 3Ë ÈÔ Û˘¯Ó‹ ηÎÔ‹ıÂÈ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜. ¢È·-ÎÚ›ÓÔÓÙ·È Û ‰‡Ô Ù‡Ô˘˜, Ù· ÌË-Hodgkin Ô˘ Â›Ó·È Î·È ÈÔ Û˘¯Ó¿ Î·È ·ÔÙÂÏÔ‡Ó ÙÔ 60% ÙÔ˘ Û˘ÓfiÏÔ˘,Î·È Ù· Hodgkin ÏÂÌÊÒÌ·Ù·. ∏ ıÂڷ›· ÙÔ˘˜ ÂÚÈÏ·Ì‚¿ÓÂÈ Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂڷ¢ÙÈÎÒÓ Ê·ÚÌ¿-ÎˆÓ Î·È ·ÎÙÈÓÔıÂڷ›·˜ Î·È Ë ÚfiÁÓˆÛË Û˘Ó‹ıˆ˜ Â›Ó·È ÂÍ·ÈÚÂÙÈο ηϋ, Û ·ÓÙ›ıÂÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ.¶·ÚfiÏ· ·˘Ù¿, οÔÈ· ·È‰È¿ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ˘ÔÙÚÔÈ¿˙Ô˘Ó ‹ Ó· ÂÌÊ·Ó›˙Ô˘Ó ·ÒÙÂÚ˜ ÂÈÏÔΤ˜·fi ÙË ıÂڷ›·. √È ÓÂfiÙÂÚ˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô ÙËÓ ·Ó·ÁÓÒÚÈÛË ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·Áfi-ÓÙˆÓ, ÙËÓ ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂڷ¢ÙÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ Î·È ÙËÓ ·ÓÙ·-fiÎÚÈÛË ÛÙË ıÂڷ›· οı ·ÛıÂÓÔ‡˜, ¤ÙÛÈ ÒÛÙ ӷ ‚ÂÏÙȈı› Ë ÂÈ‚›ˆÛË, Ì ٷ˘Ùfi¯ÚÔÓË ÂÏ¿ÙÙˆÛË Ù˘̷ÎÚÔ¯ÚfiÓÈ·˜ ÙÔÍÈÎfiÙËÙ·˜ Ù˘ ıÂڷ›·˜. ∏ ·Ó·Î¿Ï˘„Ë Ó¤ˆÓ Ê·ÚÌ¿ÎˆÓ Ô˘ ÛÙÔ¯Â‡Ô˘Ó ÂȉÈο Û ÌÔ-ÚÈ·Îfi Â›Â‰Ô ·Ó·Ì¤ÓÂÙ·È Ó· Û˘Ì‚¿ÏÂÈ ÛËÌ·ÓÙÈο ÛÙÔÓ ÙÔ̤· ·˘Ùfi. ¶·Ú¿ÏÏËÏ·, Ë ·Ó¿Ù˘ÍË Â˘·›ÛıË-ÙˆÓ ÌÂıfi‰ˆÓ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂڷ›· Î·È Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ Ù˘˘ÔÙÚÔ‹˜ Î·È Ë ·Ó›¯Ó¢ÛË ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ ı· ‚ÔËı‹ÛÔ˘Ó ÛÙËÓ ÚÒÈÌË ·Ó·ÁÓÒÚÈÛËÙ˘ ¯ËÌÂÈÔ-¢·ÈÛıËÛ›·˜ ‹ ·ÓıÂÎÙÈÎfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Î·È ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ηٷÏÏËÏfiÙÂÚÔ˘ ıÂڷ¢ÙÈ-ÎÔ‡ Û¯‹Ì·ÙÔ˜. ∏ ¯Ú‹ÛË Û‡Á¯ÚÔÓˆÓ ‚ÈÔÏÔÁÈÎÒÓ ÌÂÏÂÙÒÓ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ÂÎÙÂÓ‹ ÁÔÓȉȷ΋ ηÈÚˆÙÂ˚ÓÈ΋ ·Ó¿Ï˘ÛË ı· ‰ÒÛÂÈ Ó¤· ‰È¿ÛÙ·ÛË ÛÙËÓ Î·Ù·ÓfiËÛË ÙˆÓ ·ıÔÁÂÓÂÙÈÎÒÓ Ì˯·ÓÈÛÌÒÓ, ÛÙËÓ Ù·-ÍÈÓfiÌËÛË ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Î·È ÛÙË ‚ÂÏÙÈÛÙÔÔ›ËÛË ÙˆÓ ıÂڷ¢ÙÈÎÒÓ ÌÂıfi‰ˆÓ.
§¤ÍÂȘ ÎÏÂȉȿ: §¤Ìʈ̷, ·È‰È΋ ËÏÈΛ·, Hodgkin, non-Hodgkin, ¯ËÌÂÈÔıÂڷ›·, ·ÎÙÈÓÔıÂڷ›·.
Childhood lymphomas - Novel therapeutic approaches
V. Danilatou, M. Kalmanti
Abstract: Childhood lymphoma is the third most common paediatric malignancy. Non-Hodgkinlymphoma accounts for approximately 60% of all lymphomas and Hodgkin’s disease for the rest.Combined chemotherapy and radiotherapy is the treatment of choice and the prognosis is excellent forchildren and adolescents, in contrast with that for adults. However, some patients relapse or suffer fromlong-term toxicity. πn order to improve the overall survival, the aims of current clinical trials are theidentification of prognostic markers and the individualization of treatment according to risk group andtreatment response. Targeted therapies directed against tumour-characteristic molecules will probablycontribute to the reduction of toxicity. The development of sensitive methods for monitoring minimalresidual disease and early diagnosis of relapse will assist in choosing the appropriate treatment schedulefor each patient. Genomic and proteomic analysis will provide new dimensions in the understanding ofthe pathogenetic mechanisms, in disease classification and in the optimization of treatment strategies.
Key words: Lymphoma, childhood, Hodgkin, non-Hodgkin, chemotherapy, radiotherapy.
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜/√ÁÎÔÏÔÁ›·˜,¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ∫Ú‹Ù˘
AÏÏËÏÔÁÚ·Ê›·:
µ·ÛÈÏÈ΋ ¢·ÓËÏ¿ÙÔ˘[email protected]@gmail.com¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜/√ÁÎÔÏÔÁ›·˜,¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô∏Ú·ÎÏ›Ԣ ∫Ú‹Ù˘, ∆.∫. 71110
Department of PaediatricHematology/Oncology,University Hospital ofHeraklion, Crete, Greece
Correspondence:
Vassiliki [email protected]@gmail.comDepartment of PaediatricHematology/Oncology,University Hospital ofHeraklion, 71110 Crete,Greece
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∞§ª∫ ·Ó·Ï·ÛÙÈÎfi ϤÌʈ̷ ·fi ÌÂÁ¿Ï·Î‡ÙÙ·Ú·
µ§ Burkitt ϤÌʈ̷¢§ªµ§ ‰È¿¯˘ÙÔ Ï¤Ìʈ̷ ·fi ÌÂÁ¿Ï·
µ-ÏÂÌÊÔ·ÙÙ·Ú· ∏§ Hodgkin ÏÂÌÊÒÌ·Ù·∫¡™ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ·§∂ ÏÂÌÊÔÂÈÎÚ·Ù¤˜ §§ ÏÂÌÊÔ‚Ï·ÛÙÈÎfi ϤÌʈ̷§¶ ÏÂÌÊÔÂÓÈÎfiª∏§ ÌË-Hodgkin ÏÂÌÊÒÌ·Ù·ª∫ ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ √§∂∏§ Ô˙҉˜ ÏÂÌÊÔÂÈÎÚ·Ù¤˜ √™ Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË
∂ÈÛ·ÁˆÁ‹
∆· ÏÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ ·ÔÙÂÏÔ‡Ó ÂÚ›-Ô˘ ÙÔ 13% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ Î·ÎÔËıÂÈÒÓ Ù˘·È‰È΋˜ ËÏÈΛ·˜ (1). ¢È·ÎÚ›ÓÔÓÙ·È Û ‰‡Ô Ù‡-Ô˘˜: Ù· ÌË-Hodgkin (ª∏§), Ô˘ Â›Ó·È Î·È ÈÔÛ˘¯Ó¿ Î·È ·ÔÙÂÏÔ‡Ó ÙÔ 60% ÙÔ˘ Û˘ÓfiÏÔ˘, Î·È Ù·Hodgkin ÏÂÌÊÒÌ·Ù· (∏§). ¶ÚfiÎÂÈÙ·È ÁÈ· ÎψÓÈ-Τ˜ ηÎÔ‹ıÂȘ ˘ÂÚϷۛ˜ ÙˆÓ µ Î·È ∆-ÏÂÌÊÔ-΢ÙÙ¿ÚˆÓ Û ‰È¿ÊÔÚ· ÛÙ¿‰È· ˆÚ›Ì·ÓÛ˘ (2).
∞. MË Hodgkin §ÂÌÊÒÌ·Ù· (ª∏§)
™Â ·ÓÙ›ıÂÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ Ô˘ ·Ó·-Ù‡ÛÛÔ˘Ó ÈÔ Û˘¯Ó¿ ‚Ú·‰¤ˆ˜ ·Ó·Ù˘ÛÛfiÌÂÓ·,
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·82
83§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿
¶·È‰È·ÙÚÈ΋ 2009;72:83-000
¯·ÌËÏÔ‡ ‚·ıÌÔ‡ ηÎÔ‹ıÂÈ·˜ ÏÂÌÊÒÌ·Ù·, Ù· ª∏§
ÛÙ· ·È‰È¿ Â›Ó·È ÈÔ Û˘¯Ó¿ ÂÈıÂÙÈο ÏÂÌÊÒÌ·Ù·
˘„ËÏÔ‡ ‚·ıÌÔ‡ ηÎÔ‹ıÂÈ·˜ (1). ∆Ô ÈÔ Û˘¯Ófi ϤÌ-
ʈ̷ ÛÙ· ·È‰È¿ Â›Ó·È ÙÔ Ï¤Ìʈ̷ Burkitt (µ§) ηÈ
·ÎÔÏÔ˘ıÔ‡Ó ÙÔ ÏÂÌÊÔ‚Ï·ÛÙÈÎfi ϤÌʈ̷ (§§), ÙÔ
‰È¿¯˘ÙÔ Ï¤Ìʈ̷ ·fi ÌÂÁ¿Ï· µ-ÏÂÌÊÔ·ÙÙ·Ú·
(¢§ªµ§) Î·È ÙÔ ·Ó·Ï·ÛÙÈÎfi ϤÌʈ̷ ·fi ÌÂÁ¿Ï·
·ÙÙ·Ú· (∞§ª∫). √È ˘fiÙ˘ÔÈ ª∏§ ¯·Ú·ÎÙËÚ›˙Ô-
ÓÙ·È ·fi ‰È·ÊÔÚÂÙÈΤ˜ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ, fiˆ˜
ÔÈΛÏÔ˘ ‚·ıÌÔ‡ ÚԉȿıÂÛË ÁÈ· ‰È‹ıËÛË ÙÔ˘ Ì˘Â-
ÏÔ‡ ÙˆÓ ÔÛÙÒÓ Î·È/‹ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘-
ÛÙ‹Ì·ÙÔ˜ (∫¡™), ηıÒ˜ Î·È ÌÔÚȷ΋ Î·È Î˘ÙÙ·ÚÔ-
ÁÂÓÂÙÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ·, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ··ÈÙÔ‡Ó
‰È·ÊÔÚÂÙÈΤ˜ ıÂڷ¢ÙÈΤ˜ ÚÔÛÂÁÁ›ÛÂȘ (3).
ª¤¯ÚÈ ÚfiÛÊ·Ù·, ˘‹Ú¯·Ó ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜
ÚÔÛÂÁÁ›ÛÂȘ ÁÈ· ÙÔÓ ‰È·¯ˆÚÈÛÌfi ÙˆÓ ıÂڷ¢ÙÈÎÒÓ
ÔÌ¿‰ˆÓ: ·Ó¿ÏÔÁ· Ì ÙËÓ ÈÛÙÔÏÔÁÈ΋ ÔÌ¿‰· Î·È ·Ó¿-
ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ (4). ¶Ùˆ¯Ô› ÚÔÁÓˆ-
ÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Â›Ó·È Ë ˘„ËÏ‹ LDH ÔÚÔ‡, Ë ËÏÈ-
Λ· >15 ÂÙÒÓ, Ë ÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· ÚfiÊ·-
Û˘ Ì ΢ÎÏÔʈÛÊ·Ì›‰Ë, ‚ÈÓÎÚÈÛÙ›ÓË Î·È Ú‰ÓÈ˙Ô-
ÏfiÓË Î·È ÔÈ Â͈ÏÂÌÊ·‰ÂÓÈΤ˜ ÂÓÙÔ›ÛÂȘ (1,5). ∞Í›-
˙ÂÈ Ó· ·Ó·ÊÂÚı› fiÙÈ ÚfiÛÊ·Ù· ·Ó·ÁÓˆÚ›ÛÙËÎ·Ó Û˘-
ÁÎÂÎÚÈ̤ÓÔÈ ÔÏ˘ÌÔÚÊÈÛÌÔ› ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ
«·Ú¿ÁÔÓÙ· ı·Ó¿ÙˆÛ˘ fiÁΈӻ (TNF) Î·È ÏÂÌÊÔ-
ÙÔ͛Ӣ-· (LT-·) Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ÚfiÁÓˆÛË
ÙˆÓ ·ÛıÂÓÒÓ Ì µ§ (6).
ªÂ ‚¿ÛË ·˘Ù¿ Ù· ÎÚÈÙ‹ÚÈ·, ˘¿Ú¯Ô˘Ó ÙÚÂȘ ÔÌ¿-
‰Â˜ ıÂڷ¢ÙÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘. ∏ ÚÒÙË ÔÌ¿‰·
·ÊÔÚ¿ Ù· §§, Ô˘ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ڈÙfiÎÔÏ-
Ï· Ù‡Ô˘ ÔÍ›·˜ ÏÂÌÊÔ‚Ï·ÛÙÈ΋˜ Ï¢¯·ÈÌ›·˜ ηÈ
‚·Û›˙ÔÓÙ·È ÛÂ Û˘Ó¯‹ ¤ÎıÂÛË Û ΢ÙÙ·ÚÔÛÙ·ÙÈο
Ê¿Ú̷η ÁÈ· Ì·ÎÚfi ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·. ∏ ‰Â‡ÙÂÚË
ÔÌ¿‰· ·ÊÔÚ¿ Ù· µ§ Î·È ¢§ªµ§, Ô˘ ·ÓÙÈÌÂÙˆ›˙Ô-
ÓÙ·È Ì ‚Ú·¯Â›·˜ ‰È¿ÚÎÂÈ·˜ ·ӷϷ̂·ÓfiÌÂÓÔ˘˜
·ÎÏÔ˘˜ ÂÓÙ·ÙÈ΋˜ Û˘Ó‰˘·Ṳ̂Ó˘ ¯ËÌÂÈÔıÂڷ›-
·˜. ∆¤ÏÔ˜, Ë ÙÚ›ÙË ÔÌ¿‰· ·ÊÔÚ¿ Ù· ∞§ª∫, ÁÈ· Ù·
ÔÔ›·, ·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ·Ú¯Èο ·ÓÙÈÌÂÙˆ›ÛÙË-
Î·Ó ÂÈÙ˘¯Ò˜ Ì ڈÙfiÎÔÏÏ· Î·È ·fi ÙȘ ‰‡Ô ÔÌ¿-
‰Â˜, ÙÂÏÈο ·Ó·Ù‡¯ıËÎ·Ó Í¯ˆÚÈÛÙ¿ ıÂڷ¢ÙÈο
ÚˆÙfiÎÔÏÏ· (4). ∂Âȉ‹ Ë ÚÒÙË ÔÌ¿‰· ¯ÚËÛÈÌÔ-
ÔÈ› ÚˆÙfiÎÔÏÏ· ıÂڷ›·˜ Ù‡Ô˘ Ï¢¯·ÈÌ›·˜, ı·
Á›ÓÂÈ ·Ó·ÊÔÚ¿ ÌfiÓÔ ÁÈ· ÙȘ ‰‡Ô ÙÂÏÂ˘Ù·›Â˜ ÔÌ¿‰Â˜.
∆Ô µ§ ·ÔÙÂÏ› ÙÔ 50% ÙˆÓ ª∏§ ÛÙ· ·È‰È¿ ηÈ
¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÒÚÈÌÔ µ-Ê·ÈÓfiÙ˘Ô, ÌÂ Tdt-,
CD10+, CD19+, CD20+, sIg+, CD22+ (4). ™Â ÔÛÔ-
ÛÙfi 25% ·ÓȯÓ‡ÂÙ·È ÁÔÓȉ›ˆÌ· ÙÔ˘ ÈÔ‡ Epstein-Barr
(EB). ¶ÈÔ Û˘¯Ó¿ ·ÓȯÓ‡ÂÙ·È Ë ¯ÚˆÌÔÛˆÌÈ΋ ÌÂÙ¿-
ıÂÛË t(8;14) Î·È Û·ÓÈfiÙÂÚ· Ë t(8;22) ‹ Ë t(2;8), ÌÂ
·Ó·‰È¿Ù·ÍË ÙÔ˘ c-myc. ∏ ÓfiÛÔ˜ Û˘Ó‹ıˆ˜ ÂÓÙÔ›˙Â-
Ù·È ÛÙËÓ ÎÔÈÏÈ¿, ÙÔ ∫¡™, ÙËÓ øƒ§ ÂÚÈÔ¯‹ Î·È ÙÔÓ
Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ. ∆Ô Û‡Ó‰ÚÔÌÔ Ï‡Û˘ fiÁÎÔ˘, Ô˘
·ÔÙÂÏÔ‡Û ÛËÌ·ÓÙÈÎfi Úfi‚ÏËÌ· ·Ï·ÈfiÙÂÚ· ηٿ
ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ¤¯ÂÈ ÂÏ·¯ÈÛÙÔÔÈËı› ÌÂ-
Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÚfiÊ·Û˘ ÛÙË ıÂڷ›· (Ì ÎÔÚÙÈ-
ÎÔÛÙÂÚÔÂȉ‹ Î·È ¯·ÌËÏ‹ ‰fiÛË Î˘ÎÏÔʈÛÊ·Ì›‰Ë˜)
Î·È ÙË ¯Ú‹ÛË Ù˘ Ú·Û‚Ô˘ÚÈοÛ˘ Ì ÂÓÙ·ÙÈ΋ ÂÓ˘-
‰¿ÙˆÛË. ∏ ·ÎÙÈÓÔıÂڷ›· Î·È Ë ÂÎÙÂٷ̤ÓË ¯ÂÈ-
ÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ‰ÂÓ ¤¯Ô˘Ó ϤÔÓ ı¤ÛË ÛÙË ıÂÚ·-
¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÓfiÛÔ˘ (7,8).
∆· ¢§ªµ§ ·ÔÙÂÏÔ‡Ó ÙÔ 10-20% ÙˆÓ ª∏§ ÛÙ·
·È‰È¿ Î·È ÂÌÊ·Ó›˙ÔÓÙ·È ÈÔ Û˘¯Ó¿ ÙË 2Ë ‰ÂηÂÙ›·
Ù˘ ˙ˆ‹˜. ™˘Ó‹ıˆ˜ Â›Ó·È ÂÓÙÔÈṲ̂ӷ Î·È Û·ÓÈfiÙÂ-
Ú· ÂÌÊ·Ó›˙Ô˘Ó Â͈ÏÂÌÊ·‰ÂÓÈΤ˜ ÂÓÙÔ›ÛÂȘ ÛÙÔÓ
Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Î·È ÙÔ ∫¡™. À¿Ú¯ÂÈ ‚ÈÔÏÔÁÈ΋
‰È·ÊÔÚ¿ Ù˘ ÓfiÛÔ˘ Ì ·˘Ù‹Ó ÙˆÓ ÂÓËϛΈÓ. ¶ÈÔ Û˘-
ÁÎÂÎÚÈ̤ӷ, ·ÓȯÓ‡ÂÙ·È Û¿ÓÈ· Ë ¯ÚˆÌÔÛˆÌÈ΋ ÌÂ-
Ù¿ıÂÛË t(14;18) [IgH/bcl-2] Ì ·Ó·‰È¿Ù·ÍË ÙÔ˘ bcl-2
ÛÙËÓ ÂÚÈÔ¯‹ Ù˘ ‚·ÚÈ¿˜ ·Ï‡ÛÔ˘ ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈ-
ÓÒÓ, ÂÓÒ ÈÔ Û˘¯Ó¿ ·ÓȯÓ‡ÔÓÙ·È ·Ó·‰È·Ù¿ÍÂȘ ÙÔ˘
c-myc. ∏ ÛËÌ·Û›· ÙˆÓ ÛˆÌ·ÙÈÎÒÓ ˘ÂÚÌÂÙ·ÏÏ¿ÍÂ-
ˆÓ ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ, Ô˘ Â›Ó·È ÁÓˆÛÙ‹ ÛÙÔ˘˜
ÂÓ‹ÏÈΘ, ÛÙ· ·È‰È¿ ‰ÂÓ ¤¯ÂÈ ·ÔÛ·ÊËÓÈÛÙ›. ∏ Â-
ÓÙ·ÂÙ‹˜ ÂÈ‚›ˆÛË Â›Ó·È Ôχ ηϋ Î·È Î˘Ì·›ÓÂÙ·È
Û ÔÛÔÛÙfi 90%. ∂Í·›ÚÂÛË ·ÔÙÂÏ› ÙÔ ÚˆÙÔ·-
ı¤˜ ϤÌʈ̷ ·fi µ-·ÙÙ·Ú· ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘,
Ô˘ ¤¯ÂÈ ¯ÂÈÚfiÙÂÚË ÚfiÁÓˆÛË (9).
√È ˘¿Ú¯Ô˘Û˜ ·ÚȘ ıÂڷ¢ÙÈΤ˜ ÂÈÏÔÁ¤˜ ÁÈ·
Ù· B§ Î·È ¢§ªµ§ Â›Ó·È ‚Ú·¯Â›·˜ ‰È¿ÚÎÂÈ·˜ ÂÓÙ·ÙÈο
¯ËÌÂÈÔıÂڷ¢ÙÈο ÚˆÙfiÎÔÏÏ· (8). ∆· ÚˆÙfiÎÔÏ-
Ï· ¯ÚËÛÈÌÔÔÈÔ‡Ó ¤Ó· Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂڷ¢ÙÈ-
ÎÒÓ Ê·ÚÌ¿ÎˆÓ Ì ‰È·ÊÔÚÂÙÈ΋ ΢ÙÙ·ÚÔÙÔÍÈ΋ ‰Ú¿-
ÛË Î·È ‰È·ÊÔÚÂÙÈ΋ ÙÔÍÈÎfiÙËÙ· Î·È ¤¯Ô˘Ó ˆ˜ ÊÈÏÔÛÔ-
Ê›· ÙË Û˘Ó¯‹ ‹ ÎÏ·ÛÌ·ÙÔÔÈË̤ÓË ¤ÎıÂÛË ÙˆÓ
˘„ËÏ¿ ÔÏÏ·Ï·ÛÈ·˙fiÌÂÓˆÓ Î·ÎÔËıÒÓ Î˘ÙÙ¿ÚˆÓ
Û ΢ÙÙ·ÚÔÙÔÍÈο Ê¿Ú̷η. ¶ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Â·Ó·-
Ï·Ì‚·ÓfiÌÂÓ˜ Û˘Ó‰ڛ˜ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ, ‚ÈÓÎÚÈ-
ÛÙ›Ó˘, ΢ÎÏÔʈÛÊ·Ì›‰Ë˜ ‹ ÈʈÛÊ·Ì›‰Ë˜, ˘„ËÏ‹˜
‰fiÛ˘ ÌÂıÔÙÚÂÍ¿Ù˘, Î˘Ù·Ú·‚›Ó˘, ·‰ÚÈ·Ì˘Î›Ó˘ ηÈ
ÂÙÔÔÛ›‰Ë˜, Û˘Ó‹ıˆ˜ Û ·ÎÏÔ˘˜ ÙˆÓ 3 ‚‰ÔÌ¿‰ˆÓ,
Ì Ôχ ηϿ ·ÔÙÂϤÛÌ·Ù· (ÂÈ‚›ˆÛË ÂχıÂÚË
Û˘Ì‚·Ì¿ÙˆÓ ÛÙ· 3-5 ¤ÙË 77-91%). ∂ÈϤÔÓ, ÏfiÁˆ
Ù˘ ÂÍ·ÈÚÂÙÈ΋˜ Ù¿Û˘ ÙÔ˘ µ§ ÁÈ· ‰È‹ıËÛË ÙÔ˘ ∫¡™,
Á›ÓÔÓÙ·È ÂÓ‰ÔÚÚ·¯È·›Â˜ ÂÁ¯‡ÛÂȘ Ê·ÚÌ¿ÎˆÓ (4,10).
¢Â‰Ô̤ÓÔ˘ fiÙÈ ÔÈ ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ¤¯Ô˘Ó
ÂÍ·ÈÚÂÙÈο ηϋ ÚfiÁÓˆÛË Î·È ÂÈ‚›ˆÛË, Û‡Á¯ÚÔÓ˜
ÔÏ˘ÎÂÓÙÚÈΤ˜ ÌÂϤÙ˜ ÚÔÛ¿ıËÛ·Ó Ó· ÂÏ·ÙÙÒ-
ÛÔ˘Ó ÙË ‰fiÛË Î·È ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜. ŒÙÛÈ,
¤ÁÈÓ ÚÔÛ¿ıÂÈ· Ó· ÂÏ·ÙÙˆı› Ë ‰fiÛË Ù˘ ËÌÂÈÔıÂ-
ڷ›·˜ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÌÂ
·ÚÓËÙÈÎfi fï˜ ·ÔÙ¤ÏÂÛÌ· ÛÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË,
ÂÓÒ Û ÂӉȷ̤ÛÔ˘ ÎÈÓ‰‡ÓÔ˘ ·ÛıÂÓ›˜ ÂÏ·ÙÙÒıËΠÌÂ
ÂÈÙ˘¯›· Ë ‰fiÛË Ù˘ ¯ËÌÂÈÔıÂڷ›·˜, ¯ˆÚ›˜ ·ÚÓËÙÈ-
ο ·ÔÙÂϤÛÌ·Ù· ÛÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË (11-13).
Ÿˆ˜ ·Ó·Ê¤ÚıËÎÂ, ÔÈ ·ÛıÂÓ›˜ Ì µ§ ¤¯Ô˘Ó ·˘-
ÍË̤ÓË Èı·ÓfiÙËÙ· ÓfiÛÔ˘ ÛÙÔ ∫¡™. ∏ Û˘ÛÙËÌ·ÙÈ΋
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·83
84 µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹
Paediatriki 2009;72:1-9
¯ÔÚ‹ÁËÛË ˘„ËÏ‹˜ ‰fiÛ˘ ÌÂıÔÙÚÂÍ¿Ù˘ Î·È ÙÚÈÏ‹˜
ÂÓ‰ÔÚÚ·¯È·›·˜ ıÂڷ›·˜ ÂÏ·ÙÙÒÓÂÈ ÙËÓ Èı·ÓfiÙË-
Ù· ˘ÔÙÚÔ‹˜ ÛÙÔ ∫¡™ Û ·ÛıÂÓ›˜ Ô˘ ‹Ù·Ó ·ÚÓË-
ÙÈÎÔ› ÁÈ· ÓfiÛÔ ÛÙÔ ∫¡™ ηٿ ÙË ‰È¿ÁÓˆÛË. ∞ÓÙ›ıÂÙ·,
Ë ÚfiÁÓˆÛË Â›Ó·È ¯ÂÈÚfiÙÂÚË ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ô˘
¤¯Ô˘Ó ÚÔÛ‚ÔÏ‹ ÙÔ˘ ∫¡™ ηٿ ÙË ‰È¿ÁÓˆÛË (14).
¢ÂÓ ˘¿Ú¯Ô˘Ó ·ÎfiÌË Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ Ô˘
Ó· Û˘ÁÎÚ›ÓÔ˘Ó ÙÔÓ ÚfiÏÔ Ù˘ ·ÎÙÈÓÔıÂڷ›·˜ ÛÙÔ˘˜
·ÛıÂÓ›˜ Ì ÚÔÛ‚ÔÏ‹ ∫¡™ (4).
∞Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÙÂÏÂ˘Ù·›· ¤¯ÂÈ ÂÓۈ̷-
Ùˆı› Ë ÌÂϤÙË ÙÔ˘ ÁÔÓȉȷÎÔ‡ ÚÔÊ›Ï ÙˆÓ ÏÂÌʈ-
Ì¿ÙˆÓ ÛÙ· ·È‰È¿ Ì ¢§ªµ§ Î·È B§ (8). Ÿˆ˜ ›-
Ó·È ÁÓˆÛÙfi, Ù· ¢§ªµ§ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÂÁ¿ÏË ÎÏÈ-
ÓÈ΋ Î·È ‚ÈÔÏÔÁÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, Ë
ÔÔ›· ·ÓÙ·Ó·ÎÏ¿Ù·È Û ‰È·ÊÔÚÂÙÈ΋ ÚfiÁÓˆÛË Î·È
·ÓÙ·fiÎÚÈÛË ÛÙË ıÂڷ›·. √È Alizadeh Î·È Û˘Ó.,
¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙËÓ Ù¯ÓÈ΋ ÁÔÓȉȷ΋˜ ·Ó¿Ï˘Û˘
Ì ÌÈÎÚÔÛ˘ÛÙÔȯ›Â˜ DNA, ηٿÊÂÚ·Ó Ó· ·Ó·ÁÓˆÚ›-
ÛÔ˘Ó ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜ ˘Ô-ÔÌ¿‰Â˜ Ì ÚÔÁÓˆÛÙÈ-
΋ ÛËÌ·Û›·, ÙËÓ «Ù‡Ô˘ ‚Ï·ÛÙÈÎÔ‡ ΤÓÙÚÔ˘ µ-ÏÂÌ-
ÊÔ΢ÙÙ¿ÚˆÓ» Î·È ÙËÓ «ÂÓÂÚÁÔÔÈË̤ÓË», ÂÓÒ ·ÚÁfi-
ÙÂÚ· ÔÈ Rosenwald Î·È Û˘Ó. ÂȂ‚·›ˆÛ·Ó ÙËÓ ‡·Ú-
ÍË ÙˆÓ ‰‡Ô ˘Ô-ÔÌ¿‰ˆÓ Î·È ·Ó·Î¿Ï˘„·Ó Î·È Ì›·
ÙÚ›ÙË ÔÌ¿‰· «Ù‡Ô˘ 3» (15,16). ¶·ÚfiÌÔȘ ÌÂϤÙ˜
‰ÂÓ ¤¯Ô˘Ó ·ÎfiÌË ‰ËÌÔÛÈ¢ı› ÁÈ· ·È‰È¿ ÌÂ
¢§ªµ§. ¶·ÚfiÏ· ·˘Ù¿, ÚfiÛÊ·ÙË ÌÂϤÙË, ¯ÚËÛÈ-
ÌÔÔÈÒÓÙ·˜ ·ÓÔÛÔ˚ÛÙÔ¯ËÌ›· Î·È «Â› ÙfiÔ˘»
˘‚ÚȉÈÛÌfi ÊıÔÚÈÛÌÔ‡ (FISH), η٤‰ÂÈÍ fiÙÈ ÛÙ·
·È‰È¿ Ù· ¢§ªµ§ Â›Ó·È ÈÔ Û˘¯Ó¿ «Ù‡Ô˘ ‚Ï·ÛÙÈ-
ÎÔ‡ ΤÓÙÚÔ˘ µ-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ» Î·È ÁÈ’ ·˘Ùfi ¤¯Ô˘Ó
Î·È Î·Ï‡ÙÂÚË ¤Î‚·ÛË (17).
™Â ÂÚÈÙÒÛÂȘ ˘ÔÙÚÔ‹˜ ÙÔ˘ µ§, Ë ÚfiÁÓˆÛË
·Ú·Ì¤ÓÂÈ ‰˘ÛÙ˘¯Ò˜ ÂÍ·ÈÚÂÙÈο ‰˘ÛÌÂÓ‹˜. H ÌÂÙ·-
ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·ÔÙÂÏ› ÁÈ· ÔÏÏÔ‡˜
ıÂڷ›· ÂÎÏÔÁ‹˜ Ô˘ ›Ûˆ˜ ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÂÈ‚›ˆ-
ÛË, ÂÓÒ ·Ú¿ÏÏËÏ· Á›ÓÔÓÙ·È Î·È ÂÈÚ·Ì·ÙÈΤ˜ ÛÙÔ-
¯Â˘Ì¤Ó˜ ıÂڷ›˜ (18). ¶Ôχ ÛËÌ·ÓÙÈÎfi˜ ÚÔ-
ÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Û ÂÚ›ÙˆÛË ˘ÔÙÚÔ‹˜
Â›Ó·È Ë Â›Ù¢ÍË ‰Â‡ÙÂÚ˘ ‡ÊÂÛ˘ Î·È Ë ·¿ÓÙËÛË
ÛÙË ıÂڷ›· ‰È¿ÛˆÛ˘ (19,20).
T· ∞§ª∫ ·ÔÙÂÏÔ‡Ó ÙÔ 10% ÙˆÓ ª∏§ ÛÙ· ·È-
‰È¿. ™˘Ó‹ıˆ˜ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ∆-Ê·ÈÓfiÙ˘Ô ÌÂ
ÁÂÓÈÎÂ˘Ì¤ÓË ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· Î·È µ-Û˘ÌÙÒÌ·Ù·,
ÂÓÒ ‰ÂÓ Â›Ó·È Û¿ÓȘ Î·È ÔÈ Â͈ÏÂÌÊ·‰ÂÓÈΤ˜ ÂÓÙÔ-
›ÛÂȘ ÛÙÔ ‰¤ÚÌ·, ÙÔ˘˜ Ó‡ÌÔÓ˜, ÙÔ ‹·Ú, Ù· ÔÛÙ¿
Î·È Ù· ̷Ϸο ÌfiÚÈ·. ™Â ÔÛÔÛÙfi 90%, ·ÓȯÓ‡ÂÙ·È Ô
·ÓÔÛÔÊ·ÈÓÔÙ˘ÈÎfi˜ ‰Â›ÎÙ˘ CD30+ Î·È Ë ¯ÚˆÌÔÛˆ-
ÌÈ΋ ÌÂÙ¿ıÂÛË t(2;5)(p23;q35) Ô˘ Ô‰ËÁ› ÛÙÔ Úˆ-
ÙÂ˚ÓÈÎfi Û‡ÌÏÂÁÌ· NPM/ALK Ì ‰Ú·ÛÙËÚÈfiÙËÙ· Ù˘-
ÚÔÛÈÓÈ΋˜ ÎÈÓ¿Û˘ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ¢ÓÔ˚Îfi-
ÙÂÚË ÚfiÁÓˆÛË, Û ·ÓÙ›ıÂÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ. ∆Ô
Û‡ÌÏÂÁÌ· ·˘Ùfi Ô‰ËÁ› Û ÂÏ·Ùو̤ÓË ·fiÙˆÛË,
̤ۈ ÙˆÓ ÌÔÓÔ·ÙÈÒÓ PI3K Î·È PKB/AKT, ηıÒ˜ ηÈ
Û ÂÓ›Û¯˘ÛË ÙÔ˘ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡, ̤ۈ ÙˆÓ ÌÔÓÔ-
·ÙÈÒÓ JAK/STAT Î·È ÙÔ˘ ÔÁÎÔÁÔÓȉ›Ô˘ myc (3,7).
¢È¿ÊÔÚ· ¯ËÌÂÈÔıÂڷ¢ÙÈο ÚˆÙfiÎÔÏÏ· ¤¯Ô˘Ó
¯ÚËÛÈÌÔÔÈËı› Ì Ôχ ηϿ ·ÔÙÂϤÛÌ·Ù· (ÂÈ-
‚›ˆÛË ÂχıÂÚË ÓfiÛÔ˘ 65-75%) (21-23). ∂›Ó·È ·Ú·-
ÎÙËÚÈÛÙÈÎfi fiÙÈ Ù· ıÂڷ¢ÙÈο ÚˆÙfiÎÔÏÏ· ›ӷÈ
ÂÙÂÚÔÁÂÓ‹ Î·È ÂÔ̤ӈ˜ Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÍ·¯ıÔ‡Ó
Û˘ÌÂÚ¿ÛÌ·Ù· Û¯ÂÙÈο Ì ÙÔÓ ÚfiÏÔ Î¿ı ʷÚÌ·-
΢ÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ͯˆÚÈÛÙ¿. ¶Èı·ÓÒ˜ Ë ‰ÔÍÔ-
ÚÔ˘ÌÈΛÓË, Ë ‚ÈÓÎÚÈÛÙ›ÓË Î·È Ù· ÛÙÂÚÔÂȉ‹ Ó· ·Ô-
ÙÂÏÔ‡Ó ÛËÌ·ÓÙÈο Ê¿Ú̷η ÛÙË ıÂڷ›· (4). ∂›-
Û˘, Û ·ÓÙ›ıÂÛË Ì ٷ ¿ÏÏ· ª∏§, Ë ‰È·ÛÙڈ̿و-
ÛË Ù˘ ıÂڷ›·˜ ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘
Î·È ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ ‰ÂÓ Ê·›ÓÂÙ·È Ó· Â›Ó·È ÛËÌ·-
ÓÙÈ΋ ÛÙÔ ∞§ª∫. ∂Í·›ÚÂÛË ›Ûˆ˜ ·ÔÙÂÏÔ‡Ó ÔÈ
·ÛıÂÓ›˜ Ì ϋڈ˜ ¯ÂÈÚÔ˘ÚÁÈο ÂÍ·ÈÚ¤ÛÈÌÔ fiÁÎÔ,
Ô˘ Ê·›ÓÂÙ·È Ó· ¯ÚÂÈ¿˙ÔÓÙ·È ÏÈÁfiÙÂÚË ıÂڷ›·, ηÈ
Ë ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂڷ›·˜ Û ·ÚÔ˘Û›· ·-
Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ (.¯. ÚÔÛ‚ÔÏ‹ ‰¤ÚÌ·ÙÔ˜, ÌÂ-
ÛÔıˆÚ·Î›Ô˘, ÛÏ¿¯ÓˆÓ) Ô˘ ‚Ú›ÛÎÂÙ·È ˘fi ÌÂϤÙË.
∆¤ÏÔ˜, ·Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÔÈ Û‡Á¯ÚÔÓ˜ ÌÂϤÙ˜
ÂÓۈ̷ÙÒÓÔ˘Ó ‰È¿ÊÔÚ˜ ‚ÈÔÏÔÁÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜,
fiˆ˜ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô, ›‰· ΢ÙÔÎÈÓÒÓ, ÚÔÛ-
‰ÈÔÚÈÛÌfi ÙÔ˘ CD30 ÛÙÔ Ï¿ÛÌ·, ÌÂϤÙË ˘ÔÏÂÈfi-
ÌÂÓ˘ ÓfiÛÔ˘ Î·È ÁÔÓȉȷÎÒÓ ÌÈÎÚÔÛ˘ÛÙÔȯÈÒÓ DNA,
Ì ÛÙfi¯Ô ÙËÓ ·ÍÈÔÏfiÁËÛ‹ ÙÔ˘˜ ˆ˜ ‰È·ÁÓˆÛÙÈÎÔ‡˜ ‹
ÚÔÁÓˆÛÙÈÎÔ‡˜ ‰Â›ÎÙ˜ (24,25). ¶Úfi‰ÚÔÌ· ·ÔÙÂ-
ϤÛÌ·Ù· ·Ó·Ê¤ÚÔ˘Ó fiÙÈ ÙÔ ÁÔÓ›‰ÈÔ clusterin ÂÎÊÚ¿-
˙ÂÙ·È ÂȉÈο Û ∞§ª∫, ÂÓÒ Â›Ó·È ·ÚÓËÙÈÎfi Û ¿ÏÏ·
ÏÂÌÊÒÌ·Ù· Î·È Èı·ÓÒ˜ Ó· ·ÔÙÂϤÛÂÈ ‰È·ÁÓˆÛÙÈÎfi
ÎÚÈÙ‹ÚÈÔ ÌÂÏÏÔÓÙÈο (26).
√È ÂÚÈÛÛfiÙÂÚ˜ ˘ÔÙÚÔ¤˜ Û˘Ì‚·›ÓÔ˘Ó Û¯ÂÙÈ-
ο ÓˆÚ›˜, ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ 15 ÌËÓÒÓ.
™Â ÂÚ›ÙˆÛË ˘ÔÙÚÔ‹˜, Ë ÚfiÁÓˆÛË ÙˆÓ ∞§ª∫
Â›Ó·È ÂÍ·ÈÚÂÙÈο Ùˆ¯‹. ™Â ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ Á›-
ÓÂÙ·È Î·È ‰È¿ÛˆÛË Ì ·˘ÙfiÏÔÁË ‹ ·ÏÏÔÁÂÓ‹ ÌÂÙ·Ìfi-
Û¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (4,27).
µ. Hodgkin §ÂÌÊÒÌ·Ù· (∏§)
H ÓfiÛÔ˜ ÙÔ˘ Hodgkin ÂÚÈÁÚ¿ÊËÎÂ ÁÈ· ÚÒÙË
ÊÔÚ¿ ·fi ÙÔÓ Thomas Hodgkin (1832). ∆Ô ¯·Ú·ÎÙË-
ÚÈÛÙÈÎfi ·ıÔÁÓˆÌÔÓÈÎfi ·ÙÙ·ÚÔ Â›Ó·È ÙÔ Reed-
Sternberg (R-S), Ô˘ ¤¯ÂÈ µ-΢ÙÙ·ÚÈ΋ ÚԤϢÛË.
™Â ÔÛÔÛÙfi 30% ·ÓȯÓ‡ÂÙ·È Ô Èfi˜ ∂µ. ∆fiÛÔ ÛÙÔ EB
ıÂÙÈÎfi fiÛÔ Î·È ÛÙÔ ·ÚÓËÙÈÎfi ∏§, ·Ú·ÙËÚÂ›Ù·È ÂÓÂÚ-
ÁÔÔ›ËÛË ÙˆÓ ÌÂÙ·ÁÚ·ÊÈÎÒÓ ·Ú·ÁfiÓÙˆÓ STAT6
Î·È NfkB, Ô˘ Ô‰ËÁÔ‡Ó Û ÂÓ›Û¯˘ÛË ÙÔ˘ ÔÏÏ·Ï·-
ÛÈ·ÛÌÔ‡ Î·È Ù˘ ÂÈ‚›ˆÛ˘ ÙˆÓ ÓÂÔÏ·ÛÌ·ÙÈÎÒÓ
΢ÙÙ¿ÚˆÓ R-S. ™ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÌÂÙ·ÁÚ·ÊÈ-
ÎÒÓ ·˘ÙÒÓ ·Ú·ÁfiÓÙˆÓ Û˘ÌÌÂÙ¤¯Ô˘Ó ‰È¿ÊÔÚ˜
΢ÙÙ·ÚÔΛÓ˜, fiˆ˜ Ë IL-13 Î·È Ô TNF-·, ÂÓÒ ˘¿Ú-
¯ÂÈ ·ÏÏËÏ›‰Ú·ÛË Î·È Ì ˈÛÈÓfiÊÈÏ· Î·È ÏÂÌÊÔ-
·ÙÙ·Ú·, ̤ۈ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ CD30L Î·È CD40L.
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·84
85§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿
¶·È‰È·ÙÚÈ΋ 2009;72:1-9
∏ ÚˆÙ½ÓË LMP1ÙÔ˘ ÈÔ‡ EB ÂÓÂÚÁÔÔÈ› ·Â˘ı›·˜
ÙÔÓ ÌÂÙ·ÁÚ·ÊÈÎfi ·Ú¿ÁÔÓÙ· NfkB (28-30).
∏ ÈÛÙÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË ÙˆÓ ∏§ Û‡Ìʈӷ ÌÂ
ÙËÓ International Lymphoma Study Group, REAL ηÈ
ÙÔÓ WHO (31) Û˘ÓÔ„›˙ÂÙ·È ÛÙÔÓ ¶›Ó·Î· 1.
∆Ô ∏§ ÂÌÊ·Ó›˙ÂÙ·È ÈÔ Û˘¯Ó¿ Û ËÏÈ˘ ÌÂÁ·Ï‡-
ÙÂÚ˜ ÙˆÓ 10 ÂÙÒÓ. √È ˘fiÙ˘ÔÈ ÏÂÌÊÔÂÈÎÚ·ÙÔ‡-
ÓÙÔ˜ Î·È ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ ¤¯Ô˘Ó ÙËÓ Î·Ï‡ÙÂ-
ÚË ÚfiÁÓˆÛË, ÂÓÒ Ù· ÏÂÌÊÔÂÓÈο ¤¯Ô˘Ó ÙË ¯ÂÈÚfi-
ÙÂÚË ÚfiÁÓˆÛË.
∏ ‰È¿ÁÓˆÛË Ù›ıÂÙ·È Ì ‚ÈÔ„›· Ù˘ Ì¿˙·˜. ∞ÂÈÎÔ-
ÓÈÛÙÈΤ˜ ÂÍÂÙ¿ÛÂȘ Ô˘ Û˘ÓÂÈÎÔ˘ÚÔ‡Ó Â›Ó·È Ë ·ÎÙÈ-
ÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜, ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ·, Ë ·ÍÔÓÈ΋
(CT) Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· (MRI), ÙÔ ÛÈÓıË-
ÚÔÁÚ¿ÊËÌ· Ì Á¿ÏÏÈÔ Î·È Ë ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›·
(FDP-PET). H ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· Ù›ÓÂÈ Ó·
·ÓÙÈηٷÛÙ‹ÛÂÈ ÙÔ ÛÈÓıËÚÔÁÚ¿ÊËÌ· Ì Á¿ÏÏÈÔ, η-
ıÒ˜ ‰È·ı¤ÙÂÈ ˘„ËÏfiÙÂÚË ‰È·ÎÚÈÙÈ΋ ÈηÓfiÙËÙ· ηÈ
¢·ÈÛıËÛ›·, ¯·ÌËÏfiÙÂÚË ‰fiÛË ·ÎÙÈÓÔ‚ÔÏ›·˜ ÁÈ· ÙÔÓ
·ÛıÂÓ‹, ÌÈÎÚfiÙÂÚÔ ¯ÚfiÓÔ ÂͤٷÛ˘ Î·È ·ÔÙÂÏÂÛÌ·-
ÙÈÎfiÙÂÚË ·Ó›¯Ó¢ÛË Ù˘ ÛÏËÓÈ΋˜ ÚÔÛ‚ÔÏ‹˜. ∏
ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÌÔÚ› Ó· ·ÏÏ¿ÍÂÈ ÙÔ ÛÙ¿-
‰ÈÔ Ù˘ ÓfiÛÔ˘ Û ÔÛÔÛÙfi 15-20% ÙˆÓ ·ÛıÂÓÒÓ. ¶·-
ÚfiÏ· ·˘Ù¿, ¤¯ÂÈ ¯·ÌËÏ‹ ¢·ÈÛıËÛ›· ÛÙËÓ ·Ó›¯Ó¢ÛË
Ù˘ Ì˘ÂÏÈ΋˜ Û˘ÌÌÂÙÔ¯‹˜ Î·È ‰ÂÓ ÌÔÚ› Ó· ·ÓÙÈη-
Ù·ÛÙ‹ÛÂÈ ÙË ‚ÈÔ„›· Ì˘ÂÏÔ‡. À¿Ú¯Ô˘Ó, fï˜, ÂÚÈ-
ÙÒÛÂȘ Ì ıÂÙÈÎfi PET Î·È ·ÚÓËÙÈ΋ ‚ÈÔ„›· Ì˘ÂÏÔ‡.
∆Ô PET ‰ÂÓ ÌÔÚ› Ó· ·ÓÙÈηٷÛÙ‹ÛÂÈ ÙË CT. √ Û˘Ó-
‰˘·ÛÌfi˜ ƒ∂∆/CT ¤¯ÂÈ ÌÂÁ·Ï‡ÙÂÚË ‰È·ÁÓˆÛÙÈ΋ ·Í›·.
¢ÂÓ ¯ÚÂÈ¿˙ÂÙ·È ÁÈ· ÙË ÛÙ·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘ ÚÈÓ
ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ηıÒ˜ ¤¯ÂÈ Ù¿ÛË ÁÈ·
˘ÂÚ-ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ·ÛıÂÓÒÓ (28,32-37).
∏ ÛÙ·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘ ηٿ Ann-Arbor Á›ÓÂ-
Ù·È Û ٤ÛÛÂÚ· ÛÙ¿‰È· I-IV, ·Ó¿ÏÔÁ· Ì ÙËÓ ¤ÎÙ·ÛË
Ù˘ ÓfiÛÔ˘, Î·È Î·ÙËÁÔÚÈÔÔÈÂ›Ù·È Û A Î·È µ ·Ó¿ÏÔÁ·
Ì ÙËÓ ·ÚÔ˘Û›· ‹ fi¯È µ-Û˘ÌÙˆÌ¿ÙˆÓ (ηÎÔ˘¯›·,
˘ÚÂÙfi˜), Û à Û ÂÚÈÙÒÛÂȘ ÌÂÁ¿Ï˘ Ì¿˙·˜ (>10
cm), Û ∂ Û ·ÚÔ˘Û›· Â͈ÏÂÌÊ·‰ÂÓÈÎÒÓ Ì·˙ÒÓ Î·È
Û S Û ÂÚÈÙÒÛÂȘ ÛÏËÓÈ΋˜ ÚÔÛ‚ÔÏ‹˜ (38).
∏ ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ ¤¯ÂÈ ‚ÂÏÙȈı› ÛËÌ·ÓÙÈ-
ο Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· Ì ÙË ¯Ú‹ÛË Û˘Ó‰˘·Ṳ̂Ó˘
¯ËÌÂÈÔıÂڷ›·˜ Î·È ·ÎÙÈÓÔıÂڷ›·˜ Î·È ÙËÓ ÚÔ-
Û·ÚÌÔÁ‹ Ù˘ ıÂڷ›·˜ ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘
ÓfiÛÔ˘. ∆· Û˘Ó‹ıË ¯ËÌÂÈÔıÂڷ¢ÙÈο Û¯‹Ì·Ù· Ô˘
¯ÔÚËÁÔ‡ÓÙ·È Û ·È‰È¿ Î·È ÂÓ‹ÏÈΘ Ì ϤÌʈ̷
Hodgkin Â›Ó·È Û˘Ó‰˘·ÛÌfi˜ Ê·ÚÌ¿ÎˆÓ Ô˘ ÂÚȤ¯ÂÈ
·Óıڷ΢ÎϛӘ Î·È ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜. ªÂ-
ÚÈο ·Ú·‰Â›ÁÌ·Ù· ›ӷÈ: ABVD (‰ÔÍÔÚÔ˘ÌÈΛÓË,
ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÌÏ·ÛÙ›ÓË Óٷηڂ·˙›ÓË),
OPPA±COPP Û ı‹Ï· (‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ˙fiÓË,
ÚÔηڂ·˙›ÓË, ‰ÔÍÔÚÔ˘ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë,
Ú‰ÓÈ˙fiÓË, ÚÔηڂ·˙›ÓË), OEPA±COPP Û ¿ÚÚÂ-
Ó˜ (‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ˙fiÓË, ÂÙÔÔÛ›‰Ë, ‰ÔÍÔÚÔ˘-
ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë, Ú‰ÓÈ˙fiÓË, ÚÔηڂ·-
˙›ÓË), BEACOPP (ÌÏÂÔÌ˘Î›ÓË, ÂÙÔÔÛ›‰Ë, ‰ÔÍÔ-
ÚÔ˘ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë, ‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ-
˙fiÓË, ÚÔηڂ·˙›ÓË), DBVE-PC (‰ÔÍÔÚÔ˘ÌÈΛÓË,
ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÎÚÈÛÙ›ÓË, ÂÙÔÔÛ›‰Ë, Ú‰ÓÈ˙fiÓË,
΢ÎÏÔʈÛÊ·Ì›‰Ë), MOPP/ABV (̯ψڷÈı·Ì›ÓË,
‚ÈÓÎÚÈÛÙ›ÓË, ÚÔηڂ·˙›ÓË, Ú‰ÓÈ˙fiÓË, ‰ÔÍÔÚÔ˘-
ÌÈΛÓË, ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÌÏ·ÛÙ›ÓË) (39). ∏ ıÂÚ·-
›· Á›ÓÂÙ·È ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ ηÈ
ÌÔÚ› Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Û˘Ó‰˘·ÛÌfi ·ÎÙÈÓÔıÂڷ›·˜
ÚÔۂ‚ÏËÌ¤ÓˆÓ Â‰›ˆÓ, Ë ‰Â ÂÈ‚›ˆÛË Â›Ó·È Ôχ
˘„ËÏ‹ Î·È ÔÈΛÏÏÂÈ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡-
ÓÔ˘ (¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘: ÔÏÈ΋ ÂÈ‚›ˆÛË 96-100%,
ÂӉȷ̤ÛÔ˘ Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘: 85-100%).
¶·ÚfiÏÔ Ô˘ Ë ÂÈ‚›ˆÛË Â›Ó·È Ôχ ηϋ, Ë ÙÔÍÈ-
ÎfiÙËÙ· ÙfiÛÔ ·fi ÙË ¯ËÌÂÈÔıÂڷ›· fiÛÔ Î·È ·fi
ÙËÓ ·ÎÙÈÓÔıÂڷ›· ·Ú·Ì¤ÓÔ˘Ó ÛÔ‚·Ú¤˜. ¶ÈÔ Û˘-
ÁÎÂÎÚÈ̤ӷ, ÔÈ ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜ Ô‰ËÁÔ‡Ó
Û ÛÙ›ڈÛË Î·È ˘ÔÁÔÓÈÌfiÙËÙ·, ÔÈ ·Ï΢ÏÈÔ‡ÓÙ˜
·Ú¿ÁÔÓÙ˜ Î·È Ë ÂÙÔÔÛ›‰Ë ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ·
ÂÌÊ¿ÓÈÛË ‰Â˘ÙÂÚÔ·ıÔ‡˜ Ï¢¯·ÈÌ›·˜, Ë ÌÏÂÔÌ˘Î›-
ÓË Ô‰ËÁ› Û ‰Â˘ÙÂÚÔ·ı‹ ›ÓˆÛË Î·È ÔÈ ·Óıڷ΢ÎÏ›-
Ó˜ Û ηډÈÔÌ˘Ô¿ıÂÈ·. ∏ ·ÎÙÈÓÔıÂڷ›· ¢ı‡-
ÓÂÙ·È ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Û˘Ì·ÁÒÓ fiÁΈÓ, Ï¢¯·È-
ÌÈÒÓ Î·È Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎÒÓ Û˘Ó‰ÚfïÓ, ·ıËÚÔ-
ÛÎÏËÚ˘ÓÙÈ΋˜ ηډȷ΋˜ ÓfiÛÔ˘, ‰˘ÛÏÂÈÙÔ˘ÚÁÈÒÓ
ÙˆÓ ‚·Ï‚›‰ˆÓ, Ó¢ÌÔÓÈ΋˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜, ÛÙ›-
ÚˆÛ˘, ˘ÔÁÔÓÈÌfiÙËÙ·˜ Î·È ˘Ôı˘ÚÂÔÂȉÈÛÌÔ‡/ Ca
ı˘ÚÂÔÂȉԇ˜ (39).
°È· ÙÔ ÏfiÁÔ ·˘Ùfi, Ù· Û‡Á¯ÚÔÓ· ıÂڷ¢ÙÈο
ÚˆÙfiÎÔÏÏ· ÚÔÛ·ıÔ‡Ó Ó· ÚÔÛ‰ÈÔÚ›ÛÔ˘Ó ÙË
‚¤ÏÙÈÛÙË ‰fiÛË ¯ËÌÂÈÔıÂڷ›·˜ ηÈ/‹ ·ÎÙÈÓÔıÂÚ·-
›·˜ ‹ Ó· ·ÔʇÁÔ˘Ó ÙËÓ ·ÎÙÈÓÔıÂڷ›· ÚÔÎÂÈ-
̤ÓÔ˘ Ó· ÂÏ·¯ÈÛÙÔÔÈ‹ÛÔ˘Ó ÙËÓ ÙÔÍÈÎfiÙËÙ·. ŒÙÛÈ,
ÔÈ Nachman Î·È Û˘Ó. ¯ÔÚ‹ÁËÛ·Ó ¯ËÌÂÈÔıÂڷ›·
·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ Î·È Â¤ÏÂÍ·Ó Ù˘-
¯·›· ÙÔ˘˜ ·ÛıÂÓ›˜ ÁÈ· Ó· Ï¿‚Ô˘Ó ‹ fi¯È ·ÎÙÈÓÔıÂÚ·-
›·, ·Ó¿ÏÔÁ· Ì ÙËÓ ·¿ÓÙËÛË ÛÙË ¯ËÌÂÈÔıÂÚ·-
›· (40). √È ·ÛıÂÓ›˜ Ì ϋÚË ·ÓÙ·fiÎÚÈÛË ÛÙË
¯ËÌÂÈÔıÂڷ›· Ô˘ ¤Ï·‚·Ó ·ÎÙÈÓÔıÂڷ›· ·ÎÔ-
ÏÔ‡ıˆ˜ ‰ÂÓ Â›¯·Ó ηӤӷ ÏÂÔÓ¤ÎÙËÌ· ÛÙË Û˘ÓÔ-
ÏÈ΋ ÂÈ‚›ˆÛË. ¶·ÚÔÌÔ›ˆ˜, ÔÈ Rühl Î·È Û˘Ó. ‰ÂÓ
¶›Ó·Î·˜ 1. ∏ ÈÛÙÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Hodgkin
1. Hodgkin ÎÏ·ÛÈÎfi ϤÌʈ̷ ñ √˙҉˘ ÛÎÏ‹Ú˘ÓÛË - √™ [nodular sclerosis (NS)]ñ §ÂÌÊÔÂÈÎÚ·Ù¤˜ - §∂ [lymphocyte rich classical
Hodgkin’s lymphoma (LRCHD)]ñ §ÂÌÊÔÂÓÈÎfi - §¶ [lymphocyte depleted subtypes (LD)]ñ ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ - ª∫ [mixed cellularity (MC)]
2. √˙҉˜ §ÂÌÊÔÂÈÎÚ·Ù¤˜ - √§∂∏§ [nodular lymphocytepredominance type (¡LPHD)]
3. ∞Ù·ÍÈÓfiÌËÙ· [unclassifiable cases (UC)]
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·85
‰È·›ÛÙˆÛ·Ó ˘ÂÚÔ¯‹ ÛÙËÓ ÂÈ‚›ˆÛË ÌÂÙ¿ ·fi Û˘-
ÌÏËڈ̷ÙÈ΋ ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔıÂڷ›·˜ Û ¯·-
ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÛıÂÓ›˜ (41). ŸÌˆ˜, ÛÙÔ˘˜ ·ÛıÂ-
Ó›˜ ÂӉȷ̤ÛÔ˘ Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, Ë ¯ÔÚ‹ÁËÛË
·ÎÙÈÓÔıÂڷ›·˜ ÚÔۂ‚ÏË̤ÓÔ˘ ‰›Ô˘ Û˘ÌÏË-
ڈ̷ÙÈο ÌÂÙ¿ ÙË ¯ÔÚ‹ÁËÛË ¯ËÌÂÈÔıÂڷ›·˜ ‚ÂÏ-
Ù›ˆÛ ÛËÌ·ÓÙÈο ÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂ-
ÓÒÓ. ∞ÓÙ›ıÂÙ·, Ì›· ÈÔ ÚfiÛÊ·ÙË ÌÂϤÙË ·Ó·Ê¤ÚÂÈ
·ÚfiÌÔÈ· ÂÈ‚›ˆÛË ÙfiÛÔ ÛÙÔ˘˜ ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡
fiÛÔ Î·È ÛÙÔ˘˜ ·ÛıÂÓ›˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (42). ∞fi
ÙȘ ÌÂϤÙ˜ ·˘Ù¤˜ ÚÔ·ÙÂÈ fiÙÈ Ë ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔ-
ıÂڷ›·˜ ÚÔۂ‚ÏË̤ÓÔ˘ ‰›Ô˘ ÌÂÙ¿ ·fi ¯ËÌÂÈ-
ÔıÂڷ›·, Ô˘ ÚÔÛ·ÚÌfi˙ÂÙ·È ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿-
‰ÈÔ Ù˘ ÓfiÛÔ˘, ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÂÈ‚›ˆÛË ÂχıÂÚË
Û˘Ì‚·Ì¿ÙˆÓ, fi¯È fï˜ Î·È ÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË. ∏
‰Â ¯ÔÚ‹ÁËÛË ÈÛ¯˘ÚfiÙÂÚ˘ ¯ËÌÂÈÔıÂڷ›·˜ ÚÔÎÂÈ-
̤ÓÔ˘ Ó· ·ÔÊ¢¯ı› Ë ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔıÂڷ›·˜
‰ÂÓ ÛËÌ·›ÓÂÈ fiÙÈ ¤¯ÂÈ Î·È ÏÈÁfiÙÂÚË ÙÔÍÈÎfiÙËÙ·. ¶¿-
ÓÙˆ˜, Ë ¯ÔÚ‹ÁËÛË ¯·ÌËÏfiÙÂÚ˘ ‰fiÛ˘ ¯ËÌÂÈÔıÂÚ·-
›·˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·ÎÙÈÓÔıÂڷ›· ÚÔۂ‚ÏË-
̤ÓÔ˘ ‰›Ô˘ ¤¯ÂÈ ·ÚfiÌÔÈ· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·
Ì ÙË ¯ÔÚ‹ÁËÛË ÌfiÓÔ ¯ËÌÂÈÔıÂڷ›·˜ ˘„ËÏfiÙÂÚ˘
‰fiÛ˘, ·ÏÏ¿ ·ÚÔ˘ÛÈ¿˙ÂÈ ÏÈÁfiÙÂÚË ÙÔÍÈÎfiÙËÙ·.
πÛÙÔÚÈο, Ô Û¯Â‰È·ÛÌfi˜ Ù˘ ¯ËÌÂÈÔıÂڷ›·˜
·Ï·ÈfiÙÂÚ· ÂÚÈÂÏ¿Ì‚·Ó ¢ڇÙÂÚ· ‰›· ·ÎÙÈÓÔ-
‚fiÏËÛ˘ Ù‡Ô˘ Ì·Ó‰‡·, Ô˘ ¤¯Ô˘Ó ηٷÚÁËı› ηÈ
·ÓÙÈηٷÛÙ·ı› ·fi ·ÎÙÈÓÔ‚fiÏËÛË ÚÔۂ‚ÏË̤-
ÓÔ˘ ‰›Ô˘. ª¿ÏÈÛÙ·, Ë Û‡Á¯ÚÔÓË Ù¿ÛË ÙˆÓ ·ÎÙÈÓÔ-
ıÂڷ¢ÙÒÓ Â›Ó·È Ó· ·ÎÙÈÓÔ‚ÔÏÔ‡Ó ÌfiÓÔ ÙÔ˘˜ ÚÔ-
ۂ‚ÏË̤ÓÔ˘˜ ÏÂÌÊ·‰¤Ó˜, Ì ·ÔÙ¤ÏÂÛÌ· Ë ‰fiÛË
·ÎÙÈÓÔ‚fiÏËÛ˘ ÛÙÔ˘˜ Ê˘ÛÈÔÏÔÁÈÎÔ‡˜ ÈÛÙÔ‡˜ Ó· ›-
Ó·È ÂÚÈÔÚÈṲ̂ÓË (43).
∏ Û‡Á¯ÚÔÓË Ù¿ÛË ÙˆÓ ÓÂfiÙÂÚˆÓ ıÂڷ¢ÙÈÎÒÓ
ÚˆÙÔÎfiÏÏˆÓ Â›Ó·È Ë ÚÔÛ·ÚÌÔÁ‹ Ù˘ ıÂڷ›·˜
·Ó¿ÏÔÁ· Ì ÙËÓ Ù·¯Â›· ‹ ‚Ú·‰Â›· ÚÒÈÌË ·¿ÓÙËÛË
ÛÙË ıÂڷ›·. ™Ùfi¯Ô˜ Â›Ó·È Ë ·Ó·ÁÓÒÚÈÛË ·ÛıÂÓÒÓ
Ì ¢ÓÔ˚ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Î·È Ë ÂÏ¿ÙÙˆÛË Ù˘ ¤ÓÙ·-
Û˘ Ù˘ ıÂڷ›·˜ Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ª›·
·fi ÙȘ ÚÒÙ˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÂÈ Î·Ï¿ ·ÔÙÂϤ-
ÛÌ·Ù· Û ·È‰È¿ Ì ÂÓÙÔÈṲ̂ÓÔ ∏§ Ô˘ ··ÓÙÔ‡Ó
ηϿ ÛÙË ¯ËÌÂÈÔıÂڷ›·, Ì ¯ÔÚ‹ÁËÛË ¯ËÌÂÈÔıÂ-
ڷ›·˜ ¯ˆÚ›˜ ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜ Î·È ·ÓıÚ·-
΢ÎϛӘ Î·È ·ÎÔÏÔ‡ıˆ˜ ¯·ÌËÏ‹ ‰fiÛË ·ÎÙÈÓÔıÂÚ·-
›·˜ (44). ¶·ÚÔÌÔ›ˆ˜, Ì›· ¿ÏÏË ÌÂϤÙË ÚÔÛ¿ıË-
Û ӷ ÂÍ·ÙÔÌÈ·ÛÂÈ ÙË ıÂڷ›· ·Ó¿ÏÔÁ· Ì ÙËÓ
ÚÒÈÌË ·¿ÓÙËÛË Û ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘,
Ì ηϿ ·ÔÙÂϤÛÌ·Ù· (45). ™‡Á¯ÚÔÓË Ù¿ÛË ÙˆÓ
ÎÏÈÓÈÎÒÓ ÌÂÏÂÙÒÓ Â›Ó·È Ë ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂÚ·-
›·˜ ·Ó¿ÏÔÁ· Ì ÙËÓ Ù·¯Â›· ‹ ‚Ú·‰Â›· ·¿ÓÙËÛË
Î·È ÙËÓ Ï‹ÚË ‹ ÌÂÚÈ΋ ·ÓÙ·fiÎÚÈÛË. ¶ÚÔÎÂÈ̤ÓÔ˘
Ó· ‚ÂÏÙȈı› Ë Ù·ÍÈÓfiÌËÛË ÙˆÓ ·ÛıÂÓÒÓ Û ÔÌ¿‰Â˜
ÎÈÓ‰‡ÓÔ˘, ÚfiÛÊ·Ù· ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› ÌÈ· ̤ıÔ-
‰Ô˜ ÂÎÙÂٷ̤Ó˘ ÌÂϤÙ˘ ÚˆÙÂ˚ÓÈÎÒÓ ‰ÂÈÎÙÒÓ ÛÙÔ
·›Ì· (surface enhanced laser desorption/ionization,
SELDI), Û ÌÈ· ÚÔÛ¿ıÂÈ· ·Ó·ÁÓÒÚÈÛ˘ ÌÈ·˜ Úˆ-
ÙˆÌÈ΋˜ ˘ÔÁÚ·Ê‹˜ Ô˘ ‰È·¯ˆÚ›˙ÂÈ Ù· ‰È¿ÊÔÚ·
ÎÏÈÓÈο ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘ (47). ∏ ÌÂϤÙË ÙˆÓ ÁÔÓÈ-
‰È·ÎÒÓ ˘ÔÁÚ·ÊÒÓ Û 29 ·ÛıÂÓ›˜ Ì ÎÏ·ÛÛÈÎfi ∏§
·Ó¤‰ÂÈÍÂ Û˘ÁÎÂÎÚÈ̤ӷ ÁÔÓ›‰È· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ
ÙËÓ ·¿ÓÙËÛË ÛÙË ıÂڷ›· (48). ªÂÏÏÔÓÙÈο, Ë
·Ó·ÁÓÒÚÈÛË ‚ÈÔÏÔÁÈÎÒÓ ‰ÂÈÎÙÒÓ ÂÓ‰¤¯ÂÙ·È Ó· ·Ô-
ÙÂϤÛÂÈ ÛËÌ·ÓÙÈÎfi ÂÚÁ·ÏÂ›Ô ÁÈ· ÙËÓ ¤ÁηÈÚË Úfi-
ÁÓˆÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂڷ›·, ÙËÓ ·Ó›¯Ó¢ÛË
ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ Î·È ÙËÓ Úfi‚Ï„Ë
Èı·ÓÒÓ ÌÂÏÏÔÓÙÈÎÒÓ ˘ÔÙÚÔÒÓ.
∆Ô √§∂∏§ Â›Ó·È Í¯ˆÚÈÛÙ‹ ÔÓÙfiÙËÙ· ·fi ÙÔ
ÎÏ·ÛÈÎfi ∏§, Ì ‰È·ÊÔÚÂÙÈο ÈÛÙÔ·ıÔÏÔÁÈο ηÈ
·ÓÔÛÔÊ·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο, ¢ÓÔ˚ÎfiÙÂÚË
ÚfiÁÓˆÛË, ·ÏÏ¿ Î·È Û˘¯ÓfiÙÂÚË ÂÌÊ¿ÓÈÛË ˘ÔÙÚÔ-
ÒÓ Î·È ‰Â˘ÙÂÚÔ·ıÒÓ ª∏§. ∏ ‰˘Ó·ÙfiÙËÙ· ÌfiÓÔ
¯ÂÈÚÔ˘ÚÁÈ΋˜ ÂÎÙÔÌ‹˜ Û ¯·ÌËÏÔ‡ ÛÙ·‰›Ô˘ ÓfiÛÔ
‚Ú›ÛÎÂÙ·È ˘fi ÌÂϤÙË (49).
∆¤ÏÔ˜, Ë ÌÂϤÙË ÔÏ˘ÌÔÚÊÈÛÌÒÓ Û ÁÔÓ›‰È· Ô˘
Έ‰ÈÎÔÔÈÔ‡Ó ¤Ó˙˘Ì· ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙˆÓ Ê·ÚÌ¿-
ΈÓ, fiˆ˜ ÙÔ Î˘Ùfi¯ÚˆÌ· P450, Î·È ·ÔÙÔ͛ӈÛ˘
ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡, fiˆ˜ Ë S-ÙÚ·ÓÛÊÂÚ¿ÛË Ù˘ ÁÏÔ˘-
Ù·ıÂÈfiÓ˘, ¤¯ÂÈ ˆ˜ ÛÙfi¯Ô ÙËÓ ·Ó·ÁÓÒÚÈÛË ·ÛıÂÓÒÓ
Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÂÌÊ¿ÓÈÛ˘
ÙÔÍÈÎfiÙËÙ·˜ ·fi ÙË ıÂڷ›·. ∆Ú¤¯Ô˘Û˜ ÌÂϤÙ˜
¤¯Ô˘Ó ÂÈÛ·Á¿ÁÂÈ ÙËÓ ÂÊ·ÚÌÔÁ‹ Ê·ÚÌ·ÎÔÁÂÓˆÌÈÎÒÓ
ÌÂıfi‰ˆÓ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ·Ó·ÁÓˆÚ›ÛÔ˘Ó ‚ÈÔÏÔÁÈ-
ÎÔ‡˜ ‰Â›ÎÙ˜ Ô˘ ı· ÂÈÙÚ¤„Ô˘Ó ÌÂÏÏÔÓÙÈο ÙËÓ
ÂÈÏÔÁ‹ ÙÔ˘ ηٿÏÏËÏÔ˘ ıÂڷ¢ÙÈÎÔ‡ Û¯‹Ì·ÙÔ˜
Î·È ı· ÂÏ·ÙÙÒÛÔ˘Ó ÙËÓ ÂÌÊ¿ÓÈÛË ÂÈÏÔÎÒÓ (50).
™˘ÌÂÚ·ÛÌ·ÙÈο, Ë Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÙˆÓ ·È-
‰ÈÒÓ Ì ÏÂÌÊÒÌ·Ù· Â›Ó·È Ôχ ηϋ, fï˜ οÔÈ·
·È‰È¿ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ˘ÔÙÚÔÈ¿˙Ô˘Ó ‹ Ó· ÂÌÊ·-
Ó›˙Ô˘Ó ·ÒÙÂÚ˜ ÂÈÏÔΤ˜. √È ÓÂfiÙÂÚ˜ ÎÏÈÓÈΤ˜
ÌÂϤÙ˜ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô ÙËÓ ·Ó·ÁÓÒÚÈÛË ÚÔÁÓˆÛÙÈ-
ÎÒÓ ·Ú·ÁfiÓÙˆÓ, ÙËÓ ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂڷ¢ÙÈ-
΋˜ ÚÔÛ¤ÁÁÈÛ˘ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ ηÈ
ÙËÓ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂڷ›· οı ·ÛıÂÓÔ‡˜, ¤ÙÛÈ
ÒÛÙ ӷ ‚ÂÏÙȈı› Ë ÂÈ‚›ˆÛË, Ì ٷ˘Ùfi¯ÚÔÓË ÂÏ¿Ù-
ÙˆÛË Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ÙÔÍÈÎfiÙËÙ·˜ Ù˘ ıÂڷ›·˜.
∏ ·Ó·Î¿Ï˘„Ë Ó¤ˆÓ Ê·ÚÌ¿ÎˆÓ Ô˘ ÛÙÔ¯Â‡Ô˘Ó ÂȉÈο
Û ÌÔÚÈ·Îfi Â›Â‰Ô ı· Û˘Ì‚¿ÏÂÈ ÛËÌ·ÓÙÈο. ¶·Ú¿Ï-
ÏËÏ·, Ë ·Ó¿Ù˘ÍË Â˘·›ÛıËÙˆÓ ÌÂıfi‰ˆÓ ÁÈ· ÙËÓ ·-
Ú·ÎÔÏÔ‡ıËÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂڷ›· Î·È Ù˘
¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ Ù˘ ˘ÔÙÚÔ‹˜ Î·È Ë ·Ó›¯Ó¢ÛË
ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ ı· ‚ÔËı‹ÛÔ˘Ó ÛÙËÓ
ÚÒÈÌË ·Ó·ÁÓÒÚÈÛË Ù˘ ¯ËÌÂÈÔ-¢·ÈÛıËÛ›·˜ ‹ ·ÓıÂ-
ÎÙÈÎfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Î·È ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ηٷÏÏË-
ÏfiÙÂÚÔ˘ ıÂڷ¢ÙÈÎÔ‡ Û¯‹Ì·ÙÔ˜. ∏ ¯Ú‹ÛË Û‡Á¯ÚÔ-
ÓˆÓ ‚ÈÔÏÔÁÈÎÒÓ ÌÂÏÂÙÒÓ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È
ÂÎÙÂÓ‹ ÁÔÓȉȷ΋ Î·È ÚˆÙÂ˚ÓÈ΋ ·Ó¿Ï˘ÛË ı· ‰ÒÛÂÈ
Ó¤· ‰È¿ÛÙ·ÛË ÛÙËÓ Î·Ù·ÓfiËÛË ÙˆÓ ·ıÔÁÂÓÂÙÈÎÒÓ
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Haemophagocytic lymphohistiocytosis - HLH
C. Koutsaftiki, E. Mantziou, N. Myriokefalitakis
Abstract: Haemophagocytic lymphÔhistiocytosis is a rare syndrome characterized by a reactive disorderof the mononuclear phagocytic system which gives rise to benign, generalized histiocytic proliferation inmany organs, with marked haemophagocytosis. The symptoms and signs that are associated with thediagnosis, along with their frequency of appearance, are: persistent fever (100%), cytopenia (96%), liverdysfunction (96%), hepatosplenomegaly (84%), jaundice (55%), lymphadenopathy (43%),coagulopathy (75%) and central nervous system symptoms (32%). Lymphohistiocytosis, with thepresence of haemophagocytes in the bone marrow and the reticuloendothelial system, is observed in allcases. The laboratory findings that are usually present are due to the associated hyperinflammatoryprocess and usually consist of hypercytokinaemia, hyperferritinaemia, elevated blood levels of lactatedehydrogenase, hypertriglyceridaemia, hypofibrinogenaemia, transaminasaemia, hyperbilirubinaemia,etc. Two types of haemophagocytic lymphÔhistiocytosis have been identified, the familial or primarysyndrome and the secondary, but there is difficulty in distinguishing between them, as similar mutationsand clinical and laboratory findings may be observed in both, and they have roughly the same prognosis.In addition, the triggering factor for both can be, and is in most cases, an infection. It is easy for diagnosis tobe delayed, because the syndrome can mimic a variety of diseases. Delay in diagnosis can be fatal, sinceprompt treatment, specifically bone marrow transplantation, can achieve a cure in most cases.
Key words: ∏aemophagocytic lymphÔhistiocytosis, haemophagocytic syndrome, children.
89∞¡∞™∫√¶∏™∏ REVIEW ARTICLE
∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ¶ÂÓÙ¤Ï˘
AÏÏËÏÔÁÚ·Ê›·:
ÃÚ˘Û‹ ∫Ô˘ÙÛ·˘Ù›ÎË[email protected]∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ¶ÂÓÙ¤Ï˘, 152 36, ¶. ¶ÂÓÙ¤ÏË
1st Department ofPaediatrics, GeneralChildren’s Hospital ofPenteli, Athens, Greece
Correspondence:
Chryssie [email protected] Department ofPaediatrics, GeneralChildren’s Hospital ofPenteli, Palaia Penteli 152 36,Athens, Greece
¶·È‰È·ÙÚÈ΋ 2009;72:89-000
∂ÈÛ·ÁˆÁ‹ - √ÚÈÛÌÔ›
∏ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿Úˆ-ÛË ‹ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ (∞º™)·ÓÙÈÚÔۈ‡ÂÈ ¤Ó· Ê¿ÛÌ· ·fi ÎÏËÚÔÓÔÌÔ‡-ÌÂÓ˜ Î·È Â›ÎÙËÙ˜ ηٷÛÙ¿ÛÂȘ, Ì ¿ÏÏÔÙ¿ÏÏË ‚·Ú‡ÙËÙ· ÛÙË ‰È·Ù·Ú·¯‹ Ù˘ ·ÓÔÛÔÏÔÁÈ-΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (1).
¶ÂÚÈÏ·Ì‚¿ÓÂÈ ‰‡Ô ·ÚȘ ηٷÛÙ¿ÛÂȘ, ÌÂÎÔÈÓ¿ ÎÏÈÓÈο Î·È ·ıÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο:
ÙÔ ÚˆÙÔ·ı¤˜ ‹ ÔÈÎÔÁÂÓ¤˜ ∞º™ (Familial
Haemophagocytic Lymphoistiocytosis, FHL) ηÈ
ÙÔ ‰Â˘ÙÂÚÔ·ı¤˜ ∞º™ (secondary Haemo-
phagocytic Lymphoistiocytosis, HLH) (1).
∏ ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ·ÔÙÂÏ› ·ıÔÏÔÁÔ-
·Ó·ÙÔÌÈÎfi ‡ÚËÌ·, fiÔ˘ ÂÓÂÚÁÔÔÈË̤ӷ Ì·-
ÎÚÔÊ¿Á· Ê·ÁÔ΢ÙÙ·ÚÒÓÔ˘Ó Î·È Î·Ù·ÛÙÚ¤ÊÔ˘Ó
ÂÚ˘ıÚÔ·ÙÙ·Ú·, Ï¢ÎÔ·ÙÙ·Ú·, ¿ÏÏ· Ì·ÎÚÔÊ¿-
Á·, ηıÒ˜ Î·È Úfi‰ÚÔÌ· ·ÙÙ·Ú¿ ÙÔ˘˜ (2).
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·89
∆Ô ∏LH, ÚˆÙÔ·ı¤˜ Î·È ‰Â˘ÙÂÚÔ·ı¤˜, ÌÔÚ›
Ó· Â›Ó·È ı·Ó·ÙËÊfiÚÔ, ÙfiÛÔ ÁÈ· Ù· ·È‰È¿ fiÛÔ Î·È ÁÈ·
ÙÔ˘˜ ÂÓ‹ÏÈΘ, ȉȷ›ÙÂÚ· ·Ó ·Ú·Ì›ÓÂÈ ·‰È¿ÁÓˆÛÙÔ
Î·È ‰ÂÓ ·ÓÙÈÌÂÙˆÈÛÙ› (3).
™˘¯ÓfiÙËÙ·
∏ Û˘¯ÓfiÙËÙ· Ù˘ ÓfiÛÔ˘ ‰È·Ê¤ÚÂÈ ·fi ¯ÒÚ· ÛÂ
¯ÒÚ·, ÂÓÒ, ÂÍ ·ÈÙ›·˜ ÙˆÓ ‰˘ÛÎÔÏÈÒÓ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ
ÛÙË ‰È¿ÁÓˆÛË, Â›Ó·È Èı·Ófi ÔÈ ·ÚÈıÌÔ› Ô˘ ·Ó·Ê¤ÚÔ-
ÓÙ·È Ó· ÌËÓ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ·.
™ÙË ™Ô˘Ë‰›·, Ë Â›ÙˆÛË ÙÔ˘ FLH ÔÏÔÁ›˙ÂÙ·È
Û 1:50.000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ‚ÚÂÊÒÓ, Ì ·Ó·ÏÔÁ›·
·ÚÚ¤ÓˆÓ:ıËϤˆÓ ~1:1 ‹ 1,2 ÂÚÈÙÒÛÂȘ:1.000.000
·È‰È¿ ÙÔ ÚfiÓÔ (1,4). ªÂÁ¿Ï˜ ÛÂÈÚ¤˜ ÂÚÈÙÒÛˆÓ
Ì ∞º™ ¤¯Ô˘Ó ·Ó·ÎÔÈÓˆı› ·fi ÙËÓ π·ˆÓ›· ηÈ
ÙËÓ ∆·˚‚¿Ó, ·ÏÏ¿ ‰ÂÓ Â›Ó·È ÁÓˆÛÙfi ·Ó Ë Û˘¯ÓfiÙËÙ·
Â›Ó·È ˘„ËÏfiÙÂÚË ÛÙÔ˘˜ ∞ÛÈ¿Ù˜ ·fi fi,ÙÈ ÛÙÔ˘˜ ∂˘-
Úˆ·›Ô˘˜ (5).
™Â ÌÂϤÙË ·fi ¤Ó· ΤÓÙÚÔ ÛÙËÓ ∆Ô˘ÚΛ·, Û ‰È¿-
ÛÙËÌ· 7 ÂÙÒÓ, ‰È·ÈÛÙÒıËΠHLH Û 18 ·ÛıÂÓ›˜
Î·È FHL Û 19 ·ÛıÂÓ›˜, ·fi 34.250 Ô˘ ÓÔÛËχÙË-
Î·Ó ÛÙÔ ›‰ÈÔ ‰È¿ÛÙËÌ· (0,052% Î·È 0,055% ·ÓÙÈÛÙÔ›-
¯ˆ˜) (6).
°ÂÓÈο ÁÈ· ÙÔ FHL
¶ÂÚ›Ô˘ 25% fiÏˆÓ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ∞º™ ›ӷÈ
ÚˆÙÔ·ı›˜-ÔÈÎÔÁÂÓ›˜ Î·È Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË Û˘-
¯ÓfiÙËÙ· Û˘ÁÁ¤ÓÂÈ·˜ ÌÂٷ͇ ÙˆÓ ÁÔÓ¤ˆÓ.
¶ÚÒÙË ÂÚÈÁÚ·Ê‹ ÙÔ˘ FLH ¤ÁÈÓ ÙÔ 1952 (7).
¶ÚfiÎÂÈÙ·È ÁÈ· ·˘ÙÔۈ̷ÙÈ΋ ˘ÔÏÂÈfiÌÂÓË ‰È·Ù·-
Ú·¯‹ Ô˘ ÚÔÛ‚¿ÏÏÂÈ Î˘Ú›ˆ˜ ‚Ú¤ÊË, ·ÏÏ¿ Î·È ·È-
‰È¿ ÌÈÎÚ‹˜ ËÏÈΛ·˜, ÂÓÒ ¤¯Ô˘Ó ·Ó·ÊÂÚı› Î·È ÛÔÚ·-
‰ÈΤ˜ ÂÚÈÙÒÛÂȘ. ∂ΉËÏÒÓÂÙ·È Û˘¯ÓfiÙÂÚ· ÛÙÔÓ
ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜ Î·È Û¯Â‰fiÓ ¿ÓÙ· ÚÈÓ ÙÔÓ
¤‚‰ÔÌÔ (7,8,9,10,11,12).
∏ ¤Î‚·ÛË Â›Ó·È ÌÔÈÚ·›·, Â¿Ó Ë ÓfiÛÔ˜ ‰ÂÓ ·ÓÙÈÌÂ-
ÙˆÈÛÙ›. ∏ ̤ÛË ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ‰ÂÓ
˘Ô‚¿ÏÏÔÓÙ·È Û ıÂڷ›· Â›Ó·È 2 Ì‹Ó˜, ÁÂÁÔÓfi˜
Ô˘ ‰Â›¯ÓÂÈ ÙËÓ ¤ÌÊ·ÛË Ô˘ Ú¤ÂÈ Ó· ‰Ôı› ÛÙËÓ
¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È ıÂڷ›· (12).
¶ÚfiÛÊ·Ù· ¤ÁÈÓ ٷÍÈÓfiÌËÛË Ù˘ FHL Û FHL-1,
FHL-2, FHL-3, ηıÒ˜ Î·È ¿ÏÏÔ˘˜ ÔÙ‡Ô˘˜, Ì ‚¿-
ÛË ÙȘ ÂοÛÙÔÙ ¢ÚÈÛÎfiÌÂÓ˜ ÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜
(10). ∏ ÁÂÓÂÙÈ΋ ·ÓˆÌ·Ï›· ÌÔÚ› Ó· ·ÊÔÚ¿ ÙËÓ
ÚˆÙ½ÓË ÂÚÊÔÚ›ÓË, Ë ÔÔ›· Û˘ÓÙÂÏ› ÛÙËÓ Î˘ÙÙ·-
ÚÔÙÔÍÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Ê˘ÛÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
ÊÔÓ¤ˆÓ (natural killers ‹ NK) Î·È ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆
(13). √ ˘Â‡ı˘ÓÔ˜ ÁÔÓȉȷÎfi˜ ÙfiÔ˜ ‚Ú›ÛÎÂÙ·È ÛÙÔ
¯ÚˆÌfiۈ̷ 10q21-22, ¯·Ú·ÎÙËÚ›˙ÂÈ ÙËÓ FHL-2 ηÈ
··ÓÙ¿Ù·È ÛÙÔ 20-30% ÙˆÓ ·ÛıÂÓÒÓ (10).
√È ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ MUNC 13-4, Ô˘
‰ڿ˙ÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 17q25, Ô‰ËÁÔ‡Ó ÛÙËÓ ÂÎ-
‰‹ÏˆÛË Ù˘ FHL-3, ÛÙËÓ ÔÔ›· ˘¿Ú¯ÂÈ ·Ó¿ÚÎÂÈ·
Ù˘ ÚˆÙ½Ó˘ Munc 13-4, Ë ÔÔ›· ÂÌϤÎÂÙ·È Â›-
Û˘ ÛÙÔÓ Ì˯·ÓÈÛÌfi ηٷÛÙÚÔÊ‹˜ (10).
∆¤ÏÔ˜, ÛÙËÓ FHL-1 ¤¯Ô˘Ó ·Ó·ÊÂÚı› ÌÂÙ·ÏÏ¿-
ÍÂȘ ÛÙÔ 9q21.3-22, ÂÓÒ ˘¿Ú¯Ô˘Ó Î·È ÂÚÈÙÒÛÂȘ
ÛÙȘ Ôԛ˜ ‰ÂÓ ·Ó¢ڛÛÎÂÙ·È Î·Ì›· ·fi ÙȘ ·Ú·-
¿Óˆ ÌÂÙ·ÏÏ¿ÍÂȘ. ∏ ÌÔÚÊ‹ ·˘Ù‹ ¯·Ú·ÎÙËÚ›˙ÂÙ·È
ˆ˜ non FHL-2/3, ¯ˆÚ›˜ Ó· ¤¯ÂÈ ‰È¢ÎÚÈÓÈÛı› Ë Û¯¤-
ÛË Ù˘ Ì ÙËÓ FHL-1 ‹ Ì ¿ÏϘ Ӥ˜ ÁÔÓȉȷΤ˜ ÌÂ-
Ù·ÏÏ¿ÍÂȘ (10).
∆ÂÏÂ˘Ù·›·, Á›ÓÂÙ·È ÏfiÁÔ˜ ÁÈ· ÙËÓ FHL-4, fiÔ˘
˘¿Ú¯ÂÈ ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ¯ÚˆÌfiۈ̷ 6q24, ‰ËÏ·‰‹
ÛÙÔ ÁÔÓ›‰ÈÔ Ù˘ Û˘ÓÙ·Í›Ó˘ 11 Ô˘ ÂËÚ¿˙ÂÈ ÙÔ Û‡-
ÛÙËÌ· Ê·ÁÔ΢ÙÙ¿ÚˆÛ˘, Ë ÔÔ›· ÂÓÙÔ›ÛÙËΠÛÂ
ÌÈÎÚ‹ ÔÌ¿‰· ∫Ô‡Ú‰ˆÓ ·ÛıÂÓÒÓ (14).
°ÂÓÈο ÁÈ· ÙÔ ‰Â˘ÙÂÚÔ·ı¤˜ ∏LH
∆Ô ∏LH ·Ó·Ê¤ÚÂÙ·È Î˘Ú›ˆ˜ ÛÙË ‰Â˘ÙÂÚÔ·ı‹
ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘, fiÔ˘ Ë ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ÂÈ-
ÎfiÓ· Â›Ó·È ·ÚfiÌÔÈ· Ì Ù˘ FHL, ‰ÂÓ ˘¿Ú¯Ô˘Ó
fï˜ Û¯ÂÙÈΤ˜ ÏËÚÔÊÔڛ˜ ·fi ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi
ÈÛÙÔÚÈÎfi. ™˘Ó‹ıˆ˜ ÂÌÊ·Ó›˙ÂÙ·È Û ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ-
˘ ·’ fi,ÙÈ Ë FHL Î·È ÌÔÚ› Ó· ·Ó·Î¿Ì„ÂÈ ·˘Ùfi-
Ì·Ù·. ∂›Ó·È ۷ʤ˜ fiÙÈ Û¯ÂÙ›˙ÂÙ·È Ì ÏÔÈÌÒÍÂȘ, Ï‹„Ë
Ê·Ú̿ΈÓ, Û˘ÛÙËÌ·ÙÈΤ˜ ÓfiÛÔ˘˜ (.¯. ™∂§, ¡ƒ∞)
Î·È ÁÂÓÂÙÈο ‹ ·ÎfiÌ· Î·È Î·ÎÔ‹ıË ÓÔÛ‹Ì·Ù·
(1,2,3,13,15,16,17,18,19).
√È ÏÔÈÌÔÁfiÓÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È
ÁÈ· ÙËÓ ÚfiÎÏËÛË HLH, ·ÏÏ¿ ÂÓ›ÔÙÂ ˘ÚÔ‰ÔÙÔ‡Ó
Î·È ÂÓÂÚÁÔÔÈÔ‡Ó ÙËÓ FLH, Â›Ó·È Ô EBV (ηٿ ·ÚÈÔ
ÏfiÁÔ), Ô CMV, ¿ÏÏÔÈ ÂÚËÙÔ˚Ô›, Ô Èfi˜ ·ÓÂÌ¢ÏÔÁÈ¿˜-
¤ÚËÙ· ˙ˆÛÙ‹Ú·, Ô Èfi˜ Ù˘ ÈÏ·Ú¿˜, Ù˘ ÂÚ˘ıÚ¿˜, Ô
·Ú‚Ô˚fi˜ µ-19, Ô Èfi˜ Ù˘ Áڛ˘ ∞, ÔÈ ÈÔ› ∂CHO,
Arbo, ηıÒ˜ Î·È ÙÔ Ì˘ÎÔ‚·ÎÙËÚ›‰ÈÔ Ù˘ Ê˘Ì·Ù›ˆ-
Û˘, ÙÔ Ì˘ÎfiÏ·ÛÌ·, Ë ‚ÚԢΤÏÏ·, Ë Û·ÏÌÔÓ¤ÏÏ·
ÙÔ˘ Ù‡ÊÔ˘, Ë ÏÂ˚ÛÌ¿ÓÈ·, Ë ÚÈΤÙÛÈ· Coxiella burnetti
Î·È ‰È¿ÊÔÚÔÈ Ì‡ÎËÙ˜ (9,20,21,22,23,24,25).
∆¤ÏÔ˜, HLH ÌÔÚ› Ó· ÂΉËψı› ‡ÛÙÂÚ· ·fi
HIV Ïԛ̈ÍË ‹/Î·È ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÔÓ ·ÓıÚÒÈÓÔ
ÂÚËÙÔ˚fi 8, ·ÏÏ¿ Î·È Ï›ÁÔ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÓÙÈ-
ÚÂÙÚÔ˚΋˜ ıÂڷ›·˜ ηٿ ÙÔ˘ ∏πV-AIDS (26).
∏ ÏËıÒÚ· ÙˆÓ ˘Â‡ı˘ÓˆÓ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ
οÓÂÈ ÈÔ Â‡ÎÔÏ· ηٷÓÔËÙfi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô ·Ú¯È-
Îfi˜ fiÚÔ˜ (1979) «HLH Û¯ÂÙÈ˙fiÌÂÓÔ Ì ›ˆÛË» (virus-
associated haemophagocytic syndrome, VAHS) ¿Ï-
Ï·Í ·ÚÁfiÙÂÚ· Û «HLH Û¯ÂÙÈ˙fiÌÂÓÔ Ì Ïԛ̈ÍË»
(infection-associated haemophagocytic syndrome,
IAHS). ∆Ô IAHS ÂÌÊ·Ó›˙ÂÈ ıÓËÙfiÙËÙ· ̤¯ÚÈ Î·È 50%
(18,27,28,29).
∆Ô HLH ÌÔÚ› Ó· ÂΉËψı› Î·È ‡ÛÙÂÚ· ·fi
¯ÔÚ‹ÁËÛË Ê·Ú̿ΈÓ, fiˆ˜ ·ÓÙÈÂÈÏËÙÈÎÒÓ (Ê·È-
Ó˘ÙÔ˝ÓË, Ï·ÌÔÙÚÈÁ›ÓË), ·ÓÙÈ‚ÈÔÙÈÎÒÓ (ÁÏ˘ÎÔÂÙ›-
‰È·, ÎÔÙÚÈÌÔÍ·˙fiÏË) Î·È ÂӉ¯Ô̤ӈ˜ ÌÂÊ·ÈÓ·ÌÈ-
ÎÔ‡ ÔͤԘ (2,30,31).
90 Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.
Paediatriki 2009;72:90-000
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·90
√È Î·ÎÔ‹ıÂȘ Ì ÙȘ Ôԛ˜ Û˘Ó‰¤ÂÙ·È ÙÔ HLH
·ÊÔÚÔ‡Ó Î˘Ú›ˆ˜ ÙÔ ·ÈÌÔÔÈËÙÈÎfi Û‡ÛÙËÌ· (Ï¢¯·È-
̛˜-ÏÂÌÊÒÌ·Ù·) Î·È ÌÔÚ› Ó· ¤ÏıÂÈ ÚÈÓ ‹ ηٿ
ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜, Ó· Û¯ÂÙ›˙ÂÙ·È Ì Ïԛ̈-
ÍË ‹ Ó· ¤گÂÙ·È ¯ˆÚ›˜ ÂÌÊ·Ó‹ ÏfiÁÔ (32,33,34,
35,36).
∆Ô HLH, ·ÏÏ¿ Î·È ÙÔ FLH, ¤¯ÂÈ ‰È·ÈÛÙˆı› fiÙÈ
Û¯ÂÙ›˙ÔÓÙ·È Ì ÔÚÈṲ̂Ó˜ ·ÓÔÛÔ·Ó¿ÚÎÂȘ, fiˆ˜
ÙÔ Ê˘ÏÔÛ‡Ó‰ÂÙÔ ÏÂÌÊÔÔÏÏ·Ï·ÛÈ·ÛÙÈÎfi Û‡Ó‰ÚÔ-
ÌÔ (X-linked lymphoproliferative syndrome: XLP),
ÙÔ Û‡Ó‰ÚÔÌÔ Griscelli, ÙÔ Û‡Ó‰ÚÔÌÔ Hermansky-
Pudlak type II, Ë ÓfiÛÔ˜ Kawasaki Î·È ÙÔ Û‡Ó‰ÚÔÌÔ
Chediak- Higashi. ∆¤ÏÔ˜, HLH ÌÔÚ› Ó· ÂΉËψ-
ı› ‡ÛÙÂÚ· ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ÓÂÊÚÒÓ-‹·ÙÔ˜ ηÈ
ÌÔÚ› Ó· Û˘Óԉ‡ÂÈ ÏÂÌÊÔ¸ÂÚÏ·ÛÙÈÎfi Û‡Ó-
‰ÚÔÌÔ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔÓ EBV (37,38,39,40,
41,42).
∞ÓÙ›ÛÙÚÔÊ·, ÙÔ HLH Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔÓ EBV
ÌÈÌÂ›Ù·È ‚ÈÔÏÔÁÈο ÙÔ Ï¤Ìʈ̷ ∆, ÂÓÒ ·Ú·ÙËÚ›ٷÈ
Û˘¯Ó¿ ÂͤÏÈÍË ÙÔ˘ HLH Û ϤÌʈ̷ ∆ (5,36).
∂›Û˘, Û ·ÚÎÂÙ¤˜ ÂÓ‰ÔÁÂÓ›˜ ‰È·Ù·Ú·¯¤˜ ÙÔ˘
ÌÂÙ·‚ÔÏÈÛÌÔ‡ ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÎÏÈÓÈ΋ ÂÈÎfiÓ· Ô˘
ÌÔÈ¿˙ÂÈ ÌÂ HLH (43,44).
π‰È·›ÙÂÚÔ˜ ÏfiÁÔ˜ Ú¤ÂÈ Ó· Á›ÓÂÈ ÁÈ· ÙÔ Û‡Ó‰ÚÔ-
ÌÔ ÙˆÓ ÂÓÂÚÁÔÔÈËÌ¤ÓˆÓ Ì·ÎÚÔÊ¿ÁˆÓ (MAS:
macrophage activation syndrome), Ô˘ ·Ó·Ê¤ÚÂÙ·È
ÛÙË ÌÔÚÊ‹ ÂΛÓË ÙÔ˘ ‰Â˘ÙÂÚÔ·ıÔ‡˜ HLH Ô˘
Û¯ÂÙ›˙ÂÙ·È Ì ÓÔÛ‹Ì·Ù· ÙÔ˘ Û˘Ó‰ÂÙÈÎÔ‡ ÈÛÙÔ‡. ∞·-
ÓÙ¿Ù·È ÈÔ Û˘¯Ó¿ ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ Û˘ÛÙËÌ·ÙÈ-
΋˜ Ó·ÓÈ΋˜ ÚÂ˘Ì·ÙÔÂȉԇ˜ ·ÚıÚ›Ùȉ·˜ (™¡ƒ∞) ηÈ
Û·ÓÈfiÙÂÚ· Ì ¿ÏÏ· ÓÔÛ‹Ì·Ù· ÙÔ˘ Û˘Ó‰ÂÙÈÎÔ‡
ÈÛÙÔ‡ ÛÙ· ·È‰È¿, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤ÓÔ˘ ÙÔ˘ Û˘-
ÛÙËÌ·ÙÈÎÔ‡ ÂÚ˘ıËÌ·ÙÒ‰Ô˘˜ χÎÔ˘ (15).
∏ Û˘¯ÓfiÙËÙ· ÙÔ˘ MAS ÛÙË ™¡ƒ∞ ·Ó¤Ú¯ÂÙ·È ÛÂ
6,7%-13% (45,46,47). ªÔÚ› Ó· ÂΉËψı› ÔÔÙÂ-
‰‹ÔÙ ηٿ ÙËÓ ÔÚ›· Ù˘ ÓfiÛÔ˘, ·Ó Î·È Ë ÏÂÈÔ-
„ËÊ›· ÙˆÓ ·È‰ÈÒÓ ¤¯ÂÈ ÂӉ›ÍÂȘ ÂÓÂÚÁÔ‡ ÓfiÛÔ˘
ηٿ ÙË ‰È¿ÁÓˆÛË ÙÔ˘ MAS. ¢ÂÓ ˘¿Ú¯ÂÈ ÔÌÔʈӛ·
ˆ˜ ÚÔ˜ ÙÔ ·Ó ÙÔ MAS ı· Ú¤ÂÈ Ó· ÂÍÂÙ¿˙ÂÙ·È ˆ˜
ͯˆÚÈÛÙ‹ ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ· ‹ ˆ˜ ÌÔÚÊ‹ ÙÔ˘ ‰Â˘ÙÂ-
ÚÔ·ıÔ‡˜ HLH. £ÂˆÚÂ›Ù·È fiÙÈ ı· ˘¿ÚÍÂÈ ÂÈÙ˘¯‹˜
·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÌfiÓÔ fiÙ·Ó Á›ÓÂÈ ¯Ú‹-
ÛË ÙˆÓ ‰È·ı¤ÛÈÌˆÓ ıÂڷ¢ÙÈÎÒÓ Û¯ËÌ¿ÙˆÓ Ô˘
˘¿Ú¯Ô˘Ó ÁÈ· ÙÔ HLH (15).
¶·Ú¿ÁÔÓÙ˜ Ô˘ ÌÔÚÔ‡Ó Ó· ˘ÚÔ‰ÔÙ‹ÛÔ˘Ó ÙËÓ
ÂΉ‹ÏˆÛË ÙÔ˘ MAS Û ·ÛıÂÓ›˜ Ì ™¡ƒ∞ Â›Ó·È ‰È¿-
ÊÔÚ˜ ÏÔÈÌÒÍÂȘ (EBV, Coxsackie, Parvo B-19,
Varicella, ∏epatitis A, Salmonella, Pneumocystis
carinii, Enterococcus), Ê¿Ú̷η (·ÛÈÚ›ÓË, ª™∞º,
ÌÂıÔÙÚÂÍ¿ÙË, etanercept, anakinra, ¿Ï·Ù· ¯Ú˘ÛÔ‡,
ÛÔ˘ÏÊ·Û·Ï·˙›ÓË Î.¿.), ηıÒ˜ Î·È Ë ·˘ÙfiÏÔÁË ÌÂÙ·-
ÌfiÛ¯Â˘ÛË ·Ú¯¤ÁÔÓˆÓ Î˘ÙÙ¿ÚˆÓ Û ·ÛıÂÓ›˜ Ì ·Ó-
ıÂÎÙÈ΋ ÌÔÚÊ‹ ™¡ƒ∞ (15,45,48).
∫ÏÈÓÈ΋ ÂÈÎfiÓ·
∏ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ HLH (ÚˆÙÔ·ıÔ‡˜ ηÈ
‰Â˘ÙÂÚÔ·ıÔ‡˜) ÂÚÈÏ·Ì‚¿ÓÂÈ Â›ÌÔÓÔ ˘ÚÂÙfi,
Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ÂÍ¿ÓıË-
Ì· Î·È ÔÈÎÈÏ›· Ó¢ÚÔÏÔÁÈÎÒÓ Û˘Ìو̿وÓ, fiˆ˜
¢ÂÚÂıÈÛÙfiÙËÙ·, Û·ÛÌÔ‡˜, ·Ú¿Ï˘ÛË ÎÚ·ÓÈ·ÎÒÓ
Ó‡ڈÓ, ·Ù·Í›·, Ó˘ÛÙ·ÁÌfi, ‰˘Û¯¤ÚÂÈ· ÛÙË ‚¿‰ÈÛË,
„˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ËÌÈÏËÁ›·, ÙÂÙÚ·ÏË-
Á›·, ÔÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, Â̤ÙÔ˘˜, ·˘¯ÂÓÈ΋ ‰˘-
Ûη̄›· Î·È ÛËÌ›· ·˘ÍË̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›Â-
Û˘ (8,49). ∏ Ó¢ÌÔÓÈ΋ Û˘ÌÌÂÙÔ¯‹ Â›Ó·È Â›Û˘
Û˘¯Ó‹ (4).
∂ȉÈÎfiÙÂÚ· ÁÈ· ÙËÓ ÂÈÓ¤ÌÂÛË ÙÔ˘ ∫¡™ Û ¿-
Û¯ÔÓÙ˜ ·fi HLH, Ú¤ÂÈ Ó· ˘ÔÁÚ·ÌÌÈÛÙ› fiÙÈ Ù·
Ó¢ÚÔ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· Â›Ó·È ‰‡ÛÎÔÏÔ Ó·
·ÍÈÔÏÔÁËıÔ‡Ó, ΢ڛˆ˜ ·Ó Ë ‰È¿ÁÓˆÛË Á›ÓÂÈ ÌÂÙ¿ ÙÔ
ı¿Ó·Ùfi ÙÔ˘˜. ∂ÈϤÔÓ, Û οÔÈÔ˘˜ ·fi ·˘ÙÔ‡˜
ÙÔ˘˜ ·ÛıÂÓ›˜, Ù· Â˘Ú‹Ì·Ù· ÛÙÔÓ ÂÁΤʷÏÔ Â›¯·Ó
·Ô‰oı› Û ÚÔÛ‚ÔÏ‹ ·fi οÔÈÔÓ Èfi ‹ Û ÌË ÂÈ-
‰È΋ ÌËÓÈÁÁÔÂÁÎÂÊ·Ï›Ùȉ· (8). ∏ Û˘¯ÓfiÙËÙ· ÂÌÊ¿-
ÓÈÛ˘ ‰È·Ù·Ú·¯ÒÓ ·fi ÙÔ ∫¡™ ·Ó¤Ú¯ÂÙ·È Û 20-
100% (8). ªÔÚ› Ó· ·Ú·ÙËÚËıÔ‡Ó ·ÈÌÔÚÚ·Á›Â˜
ÛÙÔÓ ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹, Û·ÛÌÔ›, ÎÒÌ·, ‰ËÏ·‰‹
ÛÙÔȯ›· Ô˘ ‰˘ÓËÙÈο Ô‰ËÁÔ‡Ó ÛÙËÓ ÂÛÊ·Ï̤ÓË
‰È¿ÁÓˆÛË Ù˘ ηÎÔÔ›ËÛ˘, ÚÔηÏÒÓÙ·˜ ‰˘ÛÌÂ-
Ó›˜ Û˘Ó¤ÂȘ ÁÈ· ÙËÓ ÔÈÎÔÁ¤ÓÂÈ·, ·ÏÏ¿ ΢ڛˆ˜ η-
ı˘ÛÙÂÚÒÓÙ·˜ ÂÈΛӉ˘Ó· ÙË ÛˆÛÙ‹ ‰È¿ÁÓˆÛË Î·È
ıÂڷ›· (50).
¢ÂÓ ˘¿Ú¯ÂÈ Û·Ê‹˜ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙˆÓ Ó¢-
ÚÔ·ıÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ Î·È ÙˆÓ ÈÛÙÔ·ıÔÏÔÁÈ-
ÎÒÓ ‚Ï·‚ÒÓ Û ¿ÏÏ· fiÚÁ·Ó·, ÂÓÒ ¤¯ÂÈ ÂÚÈÁÚ·Ê›
ÂÚ›ÙˆÛË HLH Ì ·ÔÎÏÂÈÛÙÈ΋ ÂÓÙfiÈÛË ÛÙÔ
∫¡™ (8,11).
¶·ıÔÊ˘ÛÈÔÏÔÁ›·
√È ˘Â‡ı˘ÓÔÈ ·ıÔÊ˘ÛÈÔÏÔÁÈÎÔ› Ì˯·ÓÈÛÌÔ› ‰ÂÓ
Â›Ó·È ·Ôχو˜ ÁÓˆÛÙÔ› Î·È Î·Ù·ÓÔËÙÔ›, ¤¯Ô˘Ó ·Ô-
Î·Ï˘Êı› fï˜ ÔÏϤ˜ ·fi ÙȘ ·ÏÏ·Á¤˜ Ô˘ Û˘Ì-
‚·›ÓÔ˘Ó Û ΢ÙÙ·ÚÈÎfi Î·È ÌÔÚÈ·Îfi Â›Â‰Ô (12).
£ÂˆÚÂ›Ù·È Û‡Ó‰ÚÔÌÔ ˘ÂÚÊÏÂÁÌÔÓ‹˜, fiˆ˜ Û·-
ÊÒ˜ ÚÔ·ÙÂÈ ·fi ÙË ‰È·›ÛÙˆÛË ˘ÂÚ΢ÙÙ·ÚÔÎÈ-
Ó·ÈÌ›·˜, ηıÒ˜ Î·È ÙËÓ ·‡ÍËÛË ¿ÏÏˆÓ ÚÔÊÏÂÁÌÔ-
Óˆ‰ÒÓ ÌÂÛÔÏ·‚ËÙÒÓ, fiˆ˜ Ô ·Ú¿ÁÔÓÙ·˜ Ó¤ÎÚˆ-
Û˘ ÙˆÓ fiÁÎˆÓ (TNF-·), ÔÈ ÈÓÙÂÚÏ¢ΛÓ˜ 6-8-12-18
(IL-6, IL-8, IL-12, IL-18 Î.¿.), Ô ‰È·Ï˘Ùfi˜ ˘Ô‰Ô¯¤·˜
Ù˘ ÈÓÙÂÚÏ¢ΛÓ˘ 2 (sCD25), Ë ÊÏÂÁÌÔÓ҉˘ Úˆ-
Ù½ÓË ÙˆÓ Ì·ÎÚÔÊ¿ÁˆÓ (macrophage inflammatory
protein: MIP-·), Ë ÈÓÙÂÚÊÂÚfiÓË Á (INF-Á) Î·È Ï‹-
ıÔ˜ ·ÈÌÔÔÈËÙÈÎÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Ô˘
·ÂÏ¢ıÂÚÒÓÔÓÙ·È ·fi ÂÓÂÚÁÔÔÈË̤ӷ ÏÂÌÊÔ·Ù-
Ù·Ú· Î·È ÈÛÙÈÔ·ÙÙ·Ú· Û ÔÏÏ¿ fiÚÁ·Ó· (51-52).
™ËÌ·ÓÙÈÎfi˜ ÚfiÏÔ˜ ·Ô‰›‰ÂÙ·È Î·È Û ·ıÔÏÔ-
ÁÈΤ˜ ÚˆÙ½Ó˜ Ô˘ Έ‰ÈÎÔÔÈÔ‡ÓÙ·È ·fi Ù·
91∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË
¶·È‰È·ÙÚÈ΋ 2009;72:16-22
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·91
ÌÂÙ·ÏÏ·Á̤ӷ ÁÔÓ›‰È· Ù· ÔÔ›· ˘¿Ú¯Ô˘Ó Û ¿ÙÔÌ·
Ì FHL, ·ÏÏ¿ Î·È ÛÙÔ HLH, Ì ΢ÚÈfiÙÂÚÔ ÂÎÚfiÛˆ-
Ô ÙËÓ ÂÚÊÔÚ›ÓË.
∏ INF-Á ·ÚÔ˘ÛÈ¿˙ÂÈ ·ÚÎÂÙ¿ „ËÏfiÙÂÚ˜ Û˘ÁÎÂ-
ÓÙÚÒÛÂȘ ÛÙÔ Ï¿ÛÌ· ηٿ ÙËÓ ÂÓÂÚÁfi Ê¿ÛË Ù˘ Ófi-
ÛÔ˘, ·fi fi,ÙÈ Û ·ÛıÂÓ›˜ Ì ÛË„·ÈÌ›· ‹ ·˘ÙÔ¿ÓÔÛ˜
·ı‹ÛÂȘ. √È Î˘ÙÙ·ÚÔΛÓ˜ ¢ı‡ÓÔÓÙ·È ÁÈ· ÙȘ ·Ú·-
ÙËÚÔ‡ÌÂÓ˜ ηٷÛÙ¿ÛÂȘ Ù˘ ÓfiÛÔ˘, fiˆ˜ ÔÈ Î˘ÙÙ·-
ÚÔÂӛ˜, Ë ˘ÂÚÙÚÈÁÏ˘ÎÂÚȉ·ÈÌ›· Î·È ÔÈ ‰È·Ù·Ú·¯¤˜
Ù˘ ‹Í˘. ∏ ÊÏÂÁÌÔÓ҉˘ ΢ÙÙ·ÚÔΛÓË IL-1‚ ‰ÂÓ
Â›Ó·È ·˘ÍË̤ÓË ÛÙȘ ÎÏ·ÛÈΤ˜ ÂÚÈÙÒÛÂȘ HLH,
ÂÓÒ ·ÓÙÈı¤Ùˆ˜ Ôχ ·˘ÍË̤ӷ ›‰· sCD-25
(>10000 U/ml) ‰ËÏÒÓÔ˘Ó ‰˘ÛÌÂÓ‹ ÚfiÁÓˆÛË ÁÈ·
fiϘ ÙȘ ηÙËÁÔڛ˜ ·ÛıÂÓÒÓ (13,10,60).
∏ ÊÂÚÚÈÙ›ÓË, ›Û˘ ÛËÌ·ÓÙÈÎfi˜ ‰Â›ÎÙ˘, ·˘Í¿-
ÓÂÙ·È ÏfiÁˆ ÙÔ˘ ÔÍÂȉˆÙÈÎÔ‡ stress Î·È ÌÔÚ› Ó·
Û¯ÂÙ›˙ÂÙ·È Ì ·ÓÙÈÛÙ·ıÌÈÛÙÈ΋ ·ÓÔÛÔÏÔÁÈ΋ ·¿-
ÓÙËÛË ÛÙÔ HLH.
∏ ·ÓÂͤÏÂÁÎÙË ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÓÔÛÔÏÔÁÈÎÒÓ
΢ÙÙ¿ÚˆÓ Á›ÓÂÙ·È Èı·ÓÒ˜ ÏfiÁˆ ·Ó·ÔÙÂÏÂÛÌ·ÙÈ-
΋˜ ·ÓÔÛÔÏÔÁÈ΋˜ ·¿ÓÙËÛ˘ Ô˘ ˘ÚÔ‰ÔÙ›ٷÈ
·fi Â͈ÁÂÓ‹ ÂÚÂı›ÛÌ·Ù·, fiˆ˜ Â›Ó·È ÔÈ ÏÔÈÌÔÁfiÓÔÈ
ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ› Î·È ÔÈ ÙÔ͛Ә, ‹ ·fi ÂÓ‰ÔÁÂÓ›˜
·Ú¿ÁÔÓÙ˜, fiˆ˜ ÙÔ ÔÍÂȉˆÙÈÎfi stress, Ë ÈÛÙÈ΋ η-
Ù·ÛÙÚÔÊ‹ ‹ ÚÔ˚fiÓÙ· ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ (13).
∆Ô ˘ÔΛÌÂÓÔ ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÏÂÈÌÌ· Ô˘ Û˘Ì-
‚¿ÏÏÂÈ ÛÙËÓ ·Ó¿Ù˘ÍË HLH ÌÔÚ› Ó· Â›Ó·È ÎÏËÚÔ-
ÓÔÌÔ‡ÌÂÓË ‰È·Ù·Ú·¯‹ ÙˆÓ ·ÓÔÛÔ˚ηÓÒÓ Î˘ÙÙ¿ÚˆÓ
‹ ›ÎÙËÙË, fiˆ˜ .¯. È·ÙÚÔÁÂÓ¤˜ ·ÓÔÛÔÏÔÁÈÎfi ¤Ï-
ÏÂÈÌÌ· ηÙfiÈÓ ÌÂÙ·ÌfiÛ¯Â˘Û˘ ‹ ΢ÙÙ·ÚÔÙÔÍÈ΋˜
ıÂڷ›·˜. ∆Ô Â›ÎÙËÙÔ ¤ÏÏÂÈÌÌ· ÛÙËÓ ·ÓÔÛ›· ÌÔ-
Ú› ›Û˘ Ó· Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ïԛ̈͢ ÙˆÓ ·ÓÔ-
ÛÔ˚ηÓÒÓ Î˘ÙÙ¿ÚˆÓ ‹ Ó· ÚÔηÏÂ›Ù·È ¤ÌÌÂÛ· ̤ۈ
·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ‰Ú¿Û˘ ÙˆÓ ÊÏÂÁÌÔÓˆ‰ÒÓ ÌÂ-
ÛÔÏ·‚ËÙÒÓ.
°ÂÓÈο, ÙÔ HLH Â›Ó·È ˘ÂÚÊÏÂÁÌÔÓ҉˜ Û‡Ó‰ÚÔ-
ÌÔ, Ô˘ ˘Ô‰È·ÁÈÁÓÒÛÎÂÙ·È Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi
˘ÂÚ‰Ú·ÛÙ‹ÚÈ· Ê·ÁÔ·ÙÙ·Ú·, ÌË ·ÔÙÂÏÂÛÌ·ÙÈ΋
¤Ó·ÚÍË Ù˘ ·fiÙˆÛ˘ Î·È ÌÂȈ̤ÓË ‰Ú·ÛÙËÚÈfiÙË-
Ù· ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆ Î·È ¡∫, Ô˘ Â›Ó·È ÌfiÓÈÌË ÛÙÔ˘˜
ÚˆÙÔ·ı›˜ ÁÂÓÂÙÈο Ù‡Ô˘˜ (5,13).
∏ ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ‰Ú¿ÛË ÙˆÓ ¡∫ Î·È Ù˘ ΢Ù-
Ù·ÚÔÙÔÍÈÎfiÙËÙ·˜ ÙˆÓ ∆, Û‡ÌÊ˘ÙË ‹ ›ÎÙËÙË, ÌÔ-
Ú› Ó· Â›Ó·È ÎÔÈÓfi˜ Ì˯·ÓÈÛÌfi˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ ÌÂ
HLH, Ô˘ Ô‰ËÁ› Û ·ÚfiÌÔȘ ÎÏÈÓÈΤ˜ ÂΉËÏÒ-
ÛÂȘ (13).
¢˘ÛÙ˘¯Ò˜, ÔÈ ÂÍÂÙ¿ÛÂȘ Ô˘ ÌÔÚÔ‡Ó Ó· ·Ôη-
χ„Ô˘Ó ÙÔ˘˜ ÂοÛÙÔÙ ÂÌÏÂÎfiÌÂÓÔ˘˜ Ì˯·ÓÈ-
ÛÌÔ‡˜, Á›ÓÔÓÙ·È ÌfiÓÔ Û ΤÓÙÚ· Ì ˘„ËÏ‹ ÂÍÂȉ›-
΢ÛË Î·È Ù· ·ÔÙÂϤÛÌ·Ù¿ ÙÔ˘˜ Û¿ÓÈ· Â›Ó·È ‰È·-
ı¤ÛÈÌ· ÛÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘, ÙfiÙ ‰ËÏ·‰‹ Ô˘
Ú¤ÂÈ Ó· ÏËÊıÔ‡Ó ·ÔÊ¿ÛÂȘ ÁÈ· ÙË ıÂڷ›· ηÈ
ÙËÓ ÂÚ·ÈÙ¤Úˆ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ (53).
∂ÚÁ·ÛÙËÚȷο - ÈÛÙÔÏÔÁÈο Â˘Ú‹Ì·Ù·
∆· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Ô˘ ˘ÔÛÙËÚ›˙Ô˘Ó
ÙË ‰È¿ÁÓˆÛË ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1
(13,28,50). ∏ ÔÌ¿‰· ÌÂϤÙ˘ ÙÔ˘ 1994 (HLH-94)
ıˆÚ› fiÙÈ Ù· ›‰· ÊÂÚÚÈÙ›Ó˘ >500 Ìg/L ‰È·ı¤-
ÙÔ˘Ó ÂȉÈÎfiÙËÙ· 80% ÁÈ· ÙË ‰È¿ÁÓˆÛË HLH. ∂›-
Û˘, ·Ú·ÙËÚÔ‡ÓÙ·È ·˘ÍË̤Ó˜ ÙÈ̤˜ Û ΢ÙÙ·ÚÔ-
ΛÓ˜, INF-Á, ∆¡F-· Î·È ÌÂȈ̤ÓË ‰Ú·ÛÙÈÎfiÙËÙ·
ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¡∫ Î·È ÌÂȈ̤ÓË Î˘ÙÙ·ÚÔÙÔÍÈ΋
‰Ú¿ÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆.
∆· ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙÔ HLH Â›Ó·È ·fiÚ-
ÚÔÈ· ÙÔ˘ ÂÈıÂÙÈÎÔ‡ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡ ÙˆÓ Î·Ù¿ Ù·
¿ÏÏ· Ê˘ÛÈÔÏÔÁÈÎÒÓ ÈÛÙÈÔ΢ÙÙ¿ÚˆÓ Î·È ÏÂÌÊÔ΢ÙÙ¿-
ÚˆÓ ∆ ÛÙÔ˘˜ ‰È¿ÊÔÚÔ˘˜ ÈÛÙÔ‡˜. ∆Ô Ô˘ÛÈ·ÛÙÈÎfiÙÂÚÔ
‰È·ÁÓˆÛÙÈÎfi ‡ÚËÌ· Â›Ó·È Ë ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË
ÙˆÓ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ, ¿ÏÏˆÓ Ï¢ÎÒÓ ·ÈÌÔ-
ÛÊ·ÈÚ›ˆÓ ‹ ·ÈÌÔÂÙ·Ï›ˆÓ ̤۷ ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ
ÔÛÙÒÓ, ÛÙÔÓ ÛÏ‹Ó· Î·È ÛÙÔ˘˜ ÏÂÌÊ·‰¤Ó˜, Ô˘
ÌÔÚ› Ó· ÌËÓ Â›Ó·È ÂÌÊ·Ó‹˜ ·fi ÙËÓ ·Ú¯‹ Î·È Ó·
¯ÚÂÈ·ÛÙÔ‡Ó Â·Ó·Ï·Ì‚·ÓfiÌÂÓ˜ ÂÍÂÙ¿ÛÂȘ ÙˆÓ
ÈÛÙÒÓ ·˘ÙÒÓ. ™Â ·ÛıÂÓ›˜ ·fi ÙË °ÂÚÌ·Ó›·, ·ÈÌÔ-
Ê·ÁÔ΢ÙÙ¿ÚˆÛË ‹Ù·Ó ·ÚÔ‡Û· ÌfiÓÔ ÛÙÔ 32% ÙˆÓ
92 Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.
Paediatriki 2009;72:16-22
¶›Ó·Î·˜ 1. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋˜ ÏÂÌÊÔ˚-ÛÙÈÔ΢ÙÙ¿ÚˆÛ˘ (28)
1. ªÔÚȷ΋ ‰È¿ÁÓˆÛË Û˘Ì‚·Ù‹ Ì ∞º™2. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ∞º™: Ú¤ÂÈ Ó· ÏËÚÔ‡ÓÙ·È 5 ‹
ÂÚÈÛÛfiÙÂÚ· ·fi Ù· ·ÎfiÏÔ˘ı· 8: i) ¶˘ÚÂÙfi˜
ii) ™ÏËÓÔÌÂÁ·Ï›·iii) ∫˘ÙÙ·ÚÔÂӛ˜ (Ó· ÂËÚ¿˙ÔÓÙ·È ≥2 ΢ÙÙ·ÚÈΤ˜
ÛÂÈÚ¤˜ ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì·) - ∞ÈÌÔÛÊ·ÈÚ›ÓË <90 g/L (ÓÂÔÁÓ¿ <4 ‚‰ÔÌ¿‰ˆÓ:
·ÈÌÔÛÊ·ÈÚ›ÓË <100 g/L)- ∞ÈÌÔÂÙ¿ÏÈ· <100x109/L- √˘‰ÂÙÂÚfiÊÈÏ· <1,0x109/L
iv) ÀÂÚÙÚÈÁÏ˘ÎÂÚȉ·ÈÌ›· ηÈ/‹ ˘Ô˚Óˆ‰ÔÁÔÓ·ÈÌ›·:- ∆ÚÈÁÏ˘ÎÂÚ›‰È· Ó‹ÛÙˆ˜ ≥265 mg/dl- πÓˆ‰ÔÁfiÓÔ ≤1,5 g/L
Ó) ∞ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ÛÂ: Ì˘ÂÏfi ÔÛÙÒÓ, ÛÏ‹Ó·, ÏÂÌÊ·‰¤Ó˜ ‹ ∂¡À: ¯ˆÚ›˜ ÂӉ›ÍÂȘ ηÎÔ‹ıÂÈ·˜
vi) ∂Ï·Ùو̤ÓË ‹ Ï‹Ú˘ ¤ÏÏÂÈ„Ë ‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÙˆÓ΢ÙÙ¿ÚˆÓ ¡∫ (Û‡Ìʈӷ Ì ÙȘ ÙÈ̤˜ ·Ó·ÊÔÚ¿˜ ÙÔ˘ÂοÛÙÔÙ ÂÚÁ·ÛÙËÚ›Ô˘)
vii) ∞˘ÍË̤ӷ ›‰· ÊÂÚÚÈÙ›Ó˘ (≥500 Ìg/L)viii) ¢È·Ï˘Ùfi˜ ˘Ô‰Ô¯¤·˜ Ù˘ ÈÓÙÂÚÏ¢ΛÓ˘ 2 (sCD25)
¿Óˆ ·fi Ù· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· ÁÈ· ÙËÓ ËÏÈΛ·
∂ÈÎÔ˘ÚÈΤ˜ ∂Ӊ›ÍÂȘ:ñ ™˘ÌÙÒÌ·Ù· ∫¡™ Î·È ∂˘Ú‹Ì·Ù· ∂¡À: ·)̤ÙÚÈ· ÏÂÈÔ΢Ù-
Ù¿ÚˆÛË 20-80 ÏÂÌÊÔ·ÙÙ·Ú·/ÌL ENY (΢ڛˆ˜ ∆ ÏÂÌÊÔ-·ÙÙ·Ú·) ‚) ·‡ÍËÛË Ï¢ÎÒÌ·ÙÔ˜ Û 0,5-1 g/L ENY Á) ·ÈÌÔ-Ê·ÁÔ΢ÙÙ¿ÚˆÛË ÛÙ· ÈÛÙÈÔ·ÙÙ·Ú·-Ì·ÎÚÔÊ¿Á· ÙÔ˘ ∂¡À
ñ ∆Ú·ÓÛ·ÌÈÓ·Û·ÈÌ›·ñ ÃÔÏÂÚ˘ıÚÈÓ·ÈÌ›·ñ LDH ÔÚÔ‡ >1000 U/L
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·92
ÂÚÈÙÒÛÂˆÓ ÛÙËÓ ÚÒÙË Ï‹„Ë Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ
Î·È ÛÙÔ 85% ÙË ÛÙÈÁÌ‹ Ù˘ ‰È¿ÁÓˆÛ˘ (13).
∏ Ë·ÙÈ΋ ·Ó¿ÚÎÂÈ· (ÏfiÁˆ ‰È‹ıËÛ˘ ÙÔ˘ ‹·-
ÙÔ˜ Î·È Ù˘ ‰Ú¿Û˘ ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ) Â›Ó·È Âͤ¯ÔÓ
¯·Ú·ÎÙËÚÈÛÙÈÎfi Û ÔÏÏÔ‡˜ ·ÛıÂÓ›˜, Ô˘ ÌÔÚ›
Ó· ÂÌÊ·Ó›˙Ô˘Ó È‰È·ÈÙ¤Úˆ˜ ·˘ÍË̤ӷ ›‰· Ë·ÙÈ-
ÎÒÓ ÂÓ˙‡ÌˆÓ, ¯ÔÏÂÚ˘ıÚ›Ó˘, ·ÏÏ¿ Î·È ˘ÔÏÂ˘ÎˆÌ·-
ÙÈÓ·ÈÌ›· Î·È ÌÂȈ̤ÓË ‰Ú·ÛÙËÚÈfiÙËÙ· ÔÏÏÒÓ ·fi
ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ‹Í˘ (54).
∏ ÙÂÎÌËÚ›ˆÛË Ù˘ ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛ˘ ÛÙÔ
ÚÒÙÔ ‰Â›ÁÌ· Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ, Û ÂÚ›Ô˘ 20%
ÙˆÓ ÂÚÈÙÒÛˆÓ, Â›Ó·È ‰‡ÛÎÔÏË ‹ Î·È ·‰‡Ó·ÙË,
fï˜ ÙÔ ÁÂÁÔÓfi˜ ·˘Ùfi ‰ÂÓ ı· Ú¤ÂÈ Ó· ·ÔÙÂÏ›
ÂÌfi‰ÈÔ ÛÙËÓ ¿ÌÂÛË ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ÂÊfiÛÔÓ
ÏËÚÔ‡ÓÙ·È Ù· ¿ÏÏ· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ·
(13,15,28). ∂›Û˘, Â¿Ó ‰È·ÈÛÙˆı› ÌÂÙ¿ÏÏ·ÍË ÛÙÔ
ÁÔÓ›‰ÈÔ Ù˘ ÂÚÊÔÚ›Ó˘, ÙfiÙ ‰ÂÓ ¯ÚÂÈ¿˙ÔÓÙ·È ¿ÏÏ·
‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ·.
∂ÈϤÔÓ, ˘ÔÛÙËÚ›˙ÂÙ·È fiÙÈ ÁÈ· ÙË ‰È¿ÁÓˆÛË
HLH Ú¤ÂÈ Ó· Û˘Ó˘¿Ú¯ÂÈ Ï‹Ú˘ ¤ÏÏÂÈ„Ë ‰Ú·-
ÛÙËÚÈfiÙËÙ·˜ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¡∫, ÌÂ Û˘ÁÎÂÓÙÚÒÛÂȘ
ÊÂÚÚÈÙ›Ó˘ >500 Ìg/L Î·È sCD25 >2400 U/ml
(15,28). √ ‰È·Ï˘Ùfi˜ ˘Ô‰Ô¯¤·˜ ·ÈÌÔÛÊ·ÈÚ›Ó˘-
·ÙÔÛÊ·ÈÚ›Ó˘ CD163, Ô˘ Â›Ó·È ÂȉÈÎfi˜ ÁÈ· Ù· Ì·-
ÎÚÔÊ¿Á·, ·ÔÙÂÏ› Ó¤Ô ‰Â›ÎÙË ÁÈ· ÙÔÓ ¤ÏÂÁ¯Ô Ù˘
‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÙÔ˘ HLH. ∆· ›‰¿ ÙÔ˘ Û¯ÂÙ›˙Ô-
ÓÙ·È ÛÙÂÓ¿ Ì ÙË ‰Ú·ÛÙËÚÈfiÙËÙ· Ù˘ ÓfiÛÔ˘ Î·È Â›-
Û˘ Ì ÙË ÊÂÚÚÈÙ›ÓË ÙÔ˘ ÔÚÔ‡.
∏ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ÎÏÈÓÈÎÒÓ Î·È ÈÛÙÔÏÔÁÈÎÒÓ
¢ÚËÌ¿ÙˆÓ Û˘ÓÔ„›˙ÂÙ·È ÛÙÔ˘˜ ¶›Ó·Î˜ 2 Î·È 3 (8).
¶ÚÔÛ‚ÔÏ‹ ÙÔ˘ ∫¡™
ŸÙ·Ó ˘¿Ú¯ÂÈ ÂÈÓ¤ÌÂÛË ÙÔ˘ ∫¡™, ÙÔ Î˘ÚÈfiÙÂÚÔ
ÈÛÙÔ·ıÔÏÔÁÈÎfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi Â›Ó·È Ë ÂÎÙÂٷ̤-
ÓË, ÌË Î·ÎÔ‹ı˘ ‰È‹ıËÛË ·fi ÏÂÌÊÔ·ÙÙ·Ú· ηÈ
Ì·ÎÚÔÊ¿Á·, Ù˘ÈÎfiÙÂÚ· Ì ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË,
Û˘Ó‹ıˆ˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ‰È‹ıËÛË Î·È Û ¿ÏÏ· fiÚ-
Á·Ó·, fiˆ˜ ÛÏ¿¯Ó·, ÏÂÌÊ·‰¤Ó˜, Ì˘ÂÏfi˜ ÙˆÓ
ÔÛÙÒÓ Î.¿. (55). ªÔÚ› Ó· ˘¿Ú¯ÂÈ ÂÈÓ¤ÌËÛË Î·È
ÙˆÓ ÂÚÈÊÂÚÈÎÒÓ ÓÂ‡ÚˆÓ (8).
∂›Û˘, Â›Ó·È ÂӉȷʤÚÔÓ fiÙÈ ¤¯ÂÈ ‰È·ÈÛÙˆı›
Û˘Û¯¤ÙÈÛË Ù˘ ÎÏÈÓÈ΋˜ ‚·Ú‡ÙËÙ·˜ Ù˘ ÚÔÛ‚ÔÏ‹˜
ÙÔ˘ ∫¡™ Î·È ÙˆÓ Ó¢ÚÔ·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎÒÓ ·Ï-
ÏÔÈÒÛÂˆÓ (8).
∏ ÓfiÛÔ˜ ÙÔ˘ ∫¡™ Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ÈÔ Û˘¯Ó‹
ÛÙ· ¿ÙÔÌ· Ô˘ ʤÚÔ˘Ó ÙË ÌÂÙ¿ÏÏ·ÍË ªunc 13-4
(FHL-3) (56).
√È ·ÛıÂÓ›˜ Ì ÚÔÛ‚ÔÏ‹ ÙÔ˘ ∫¡™ ÌÔÚÔ‡Ó Ó·
Ù·ÍÈÓÔÌËıÔ‡Ó ˆ˜ ¿Û¯ÔÓÙ˜ ›Ù ·fi ÏÂÌÊÔ΢ÙÙ·-
ÚÈ΋ ÌËÓÈÁÁ›Ùȉ·/ÂÁÎÂÊ·Ï›Ùȉ·, fiÔ˘ ‰ÂÓ ˘¿Ú¯ÂÈ
ηӤӷ ¿ÏÏÔ Ó¢ÚÔÏÔÁÈÎfi Û‡Ìو̷ ÂÎÙfi˜ ·fi ÌË-
ÓÈÁÁÈÛÌfi Î·È ·ıÔÏÔÁÈÎfi ∂¡À, ›Ù ·fi «Ó¢ÚÔÏÔÁÈ-
΋ Û˘Ìو̷ÙÔÏÔÁ›·», ‰ËÏ·‰‹ ÔÔÈÔ‰‹ÔÙ Ó¢-
ÚÔÏÔÁÈÎfi Úfi‚ÏËÌ· ‹ Û·ÛÌÔ‡˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ
‰È·Ù·Ú·¯¤˜ ÙÔ˘ ∫¡™ (55).
∆· Â˘Ú‹Ì·Ù· ·fi ÙÔ ∂¡À ÛÙË ÏÂÌÊÔ΢ÙÙ·ÚÈ΋
ÌËÓÈÁÁ›Ùȉ· ÙÔ˘ ∞º™ Û˘ÓÔ„›˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 4.
∂›Û˘, Â›Ó·È Ú‹ÛÈÌË Ë Ì¤ÙÚËÛË ∂¡À ΢ÙÙ·ÚÔΛÓ˘
Î·È ÓÂÔÙÂÚ›Ó˘ ÛÙÔ ∂¡À (8,55).
∆Ô Î˘ÙÙ·ÚÈÎfi ÂÚȯfiÌÂÓÔ ÙÔ˘ ∂¡À ‰ÂÓ Ê·›ÓÂ-
Ù·È fiÙÈ ·ÔÙÂÏ› ȉȷ›ÙÂÚ· ¯Ú‹ÛÈÌÔ ‰Â›ÎÙË Ù˘ ‚·-
Ú‡ÙËÙ·˜ ÂÈÓ¤ÌËÛ˘ ÙÔ˘ ∫¡™, Û ·ÓÙ›ıÂÛË Ì ÙÔ
χΈ̷, ÙÔ ÔÔ›Ô ‹Ù·Ó ȉȷ›ÙÂÚ· ·˘ÍË̤ÓÔ ÛÂ
·È‰È¿ Ì ÈÔ ÛÔ‚·Úfi ÎÏÈÓÈÎfi ÛÙ¿‰ÈÔ (ÛÙ¿‰ÈÔ 3),
ηıÒ˜ Î·È Ì ÙËÓ ÈÔ ÛÔ‚·Ú‹ Ó¢ÚÔ·ıÔÏÔÁÔ·Ó·-
ÙÔÌÈ΋ ÛÙ·‰ÈÔÔ›ËÛË (ÛÙ¿‰ÈÔ πππ) (8). ¶·ÚfiÏ· ·˘-
Ù¿, ÙÔ ∂¡À ÌÔÚ› Ó· Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi, ·ÎfiÌ· ηÈ
¶›Ó·Î·˜ 4. ∂˘Ú‹Ì·Ù· ·fi ÙÔ ∂¡À ÛÙË ÏÂÌÊÔ΢ÙÙ·ÚÈ΋ ÌËÓÈÁÁ›-Ùȉ· ÙÔ˘ ∞º™
i) ª¤ÙÚÈ· ÏÂÈÔ΢ÙÙ¿ÚˆÛË: 20-80 ÏÂÌÊÔ·ÙÙ·Ú·/ÌL ∂¡À(΢ڛˆ˜ ÏÂÌÊÔ·ÙÙ·Ú· ∆)
ii) ∞‡ÍËÛË Ù˘ Û˘ÁΤÓÙÚˆÛ˘ ÙÔ˘ Ï¢ÎÒÌ·ÙÔ˜ 0,5-1 g/L∂¡À
iii) ∞ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ÛÙ· ÈÛÙÈÔ·ÙÙ·Ú· Î·È Ì·ÎÚÔÊ¿Á·ÙÔ˘ ∂¡À (Û¿ÓÈÔ Â‡ÚËÌ·, ·ÏÏ¿ ÈÛ¯˘Ú¿ ‰ËψÙÈÎfi ÁÈ· ÙËÓfiÛÔ)
93∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË
¶·È‰È·ÙÚÈ΋ 2009;72:16-22
¶›Ó·Î·˜ 2. ∫ÏÈÓÈ΋ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ Û˘Ìو̿-ÙˆÓ ÛÙÔ ∫¡™-∞º™
™Ù¿‰ÈÔ 0: ¶Ï‹Ú˘ ¤ÏÏÂÈ„Ë Ó¢ÚÔÏÔÁÈÎÒÓ ÂΉËÏÒÛÂˆÓ ‹ÌË ÂȉÈΤ˜ Ó¢ÚÔÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜
™Ù¿‰ÈÔ 1: ªË ÂȉÈο Ó¢ÚÔÏÔÁÈο ÛËÌ›·, fiˆ˜ Ûʇ˙Ô˘-Û· ËÁ‹ ‹ ˘ÔÙÔÓ›·
™Ù¿‰ÈÔ 2: ∂ȉÈο Ó¢ÚÔÏÔÁÈο ÛËÌ›·, fiˆ˜ ·˘¯ÂÓÈ΋‰˘Ûη̄›·, Û·ÛÌÔ›, ·Ú¤ÛÂȘ
™Ù¿‰ÈÔ 3: ™Ô‚·Ú‹ Ó¢ÚÔÏÔÁÈ΋ ÓfiÛÔ˜, fiˆ˜ ÚÔԉ¢ÙÈ-΋ „˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË ‹ ·ÒÏÂÈ· Û˘-Ó›‰ËÛ˘ (ÎÒÌ·)
¶›Ó·Î·˜ 3. πÛÙÔÏÔÁÈ΋ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ Â˘ÚËÌ¿ÙˆÓ ÛÙÔ ∫¡™-∞º™ (8)
™Ù¿‰ÈÔ 0: ¶Ï‹Ú˘ ¤ÏÏÂÈ„Ë Ó¢ÚÔ-·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎÒÓ¢ÚËÌ¿ÙˆÓ
™Ù¿‰ÈÔ π: ªfiÓÔ ÏÂÙÔÌËÓÈÁÁÈΤ˜ ‰ÈËı‹ÛÂȘ ·fi ÏÂÌÊÔ-·ÙÙ·Ú· Î·È ÈÛÙÈÔ·ÙÙ·Ú· /Ì·ÎÚÔÊ¿Á·. ¶ÂÚÈ-ÛÙ·Ûȷο ÂÚÈ·ÁÁÂȷ΋ ‰È‹ıËÛË.
™Ù¿‰ÈÔ ππ: ◊È· ·ÚÂÁ¯˘Ì·ÙÈ΋ Û˘ÌÌÂÙÔ¯‹ Ì ÂÓÙÔÓfiÙÂ-ÚË ÂÚÈ·ÁÁÂȷ΋ ‰È‹ıËÛË, ·ÏÏ¿ Î·È ÂÓÙÔÓfiÙÂ-ÚË ‰È‹ıËÛË ÙˆÓ ÌËÓ›ÁÁˆÓ, Ô˘ ÂÓ›ÔÙ ÂÚÈ-Ï·Ì‚¿ÓÂÈ Î·È ÙÔÓ ˘ÔΛÌÂÓÔ ÂÁÎÂÊ·ÏÈÎfi ÈÛÙfi.
™Ù¿‰ÈÔ πππ: ∂ÈϤÔÓ Ù˘ ¤ÓÙÔÓ˘ Ì·˙È΋˜ ÈÛÙÈ΋˜ ‰È‹ıË-Û˘ ·fi ÏÂÌÊÔ·ÙÙ·Ú· Î·È ÈÛÙÈÔ·ÙÙ·Ú·/Ì·-ÎÚÔÊ¿Á·, ·Ó¢ڛÛÎÂÙ·È Ó¤ÎÚˆÛË ÙÔ˘ ÂÁÎÂÊ·-ÏÈÎÔ‡ ÈÛÙÔ‡ Ì ÛËÌ·ÓÙÈ΋ ·ÛÙÚÔÁÏÔ›ˆÛË Î·ÈÛ¯ÂÙÈο Ï›Á· ·ÊÚÒ‰Ë Ì·ÎÚÔÊ¿Á·. ªÔÚ› Ó·˘¿Ú¯Ô˘Ó ·ۂÂÛÙÒÛÂȘ.
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·93
ηٿ ÙË ‰È¿ÚÎÂÈ· ÎÏÈÓÈο ÚÔ¯ˆÚË̤Ó˘ ÓfiÛÔ˘ ÙÔ˘
∫¡™ (8).
∞ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù·
ÀÂÚ˯ÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ηٿ ÙËÓ ·Ú¯È΋
ÚÔۤϢÛË ÙˆÓ ·ÛıÂÓÒÓ Â›Ó·È (Ì Êı›ÓÔ˘Û· ÛÂÈ-
Ú¿ Û˘¯ÓfiÙËÙ·˜): ·) ÛÏËÓÔÌÂÁ·Ï›·, ‚) Ë·ÙÔÌÂ-
Á·Ï›·, Á) ·ÛΛÙ˘, ‰) ¿¯˘ÓÛË ÙÔ˘ ÙÔȯÒÌ·ÙÔ˜ Ù˘
¯ÔÏˉfi¯Ô˘ ·ÛÙ˘, Â) ·˘ÍË̤ÓË ÂÚÈ˘Ï·›· ˯Ô-
Á¤ÓÂÈ·, ÛÙ) ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ˙) Ï¢ÚÈÙÈÎfi ÂÍ›-
‰ÚˆÌ· ηÈ, Û·ÓÈfiÙÂÚ·, Ë) ÔÏϷϤ˜ ˘fi˯˜
ÂÚÈÔ¯¤˜ ÛÙÔÓ ÛÏ‹Ó·, ÓÂÊÚÔÌÂÁ·Ï›·, Ï›ıÔÈ ÛÙË
¯ÔÏˉfi¯Ô ·ÛÙË, ·˘ÍË̤ÓË Ë¯ÔÁ¤ÓÂÈ· ÙÔ˘ ÊÏÔÈÔ‡
ÙˆÓ ÓÂÊÚÒÓ Î·È ¿¯˘ÓÛË ÙÔ˘ ÙÔȯÒÌ·ÙÔ˜ ÙÔ˘ ‰ˆ-
‰Âη‰·ÎÙ‡ÏÔ˘ (12).
™ÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜, Â›Ó·È Èı·Ófi Ó·
˘¿Ú¯Ô˘Ó ΢„ÂÏȉÈΤ˜ Î·È ‰È¿ÌÂÛ˜ ÛÎȤ˜ ̠ϢÚÈ-
ÙÈ΋ Û˘ÏÏÔÁ‹, Û˘¯Ó¿ Ì ٷ¯Â›· ÂͤÏÈÍË Î·È ·Ôηٿ-
ÛÙ·ÛË-χÛË, Ô˘ ÔÊ›ÏÔÓÙ·È Û ‰ÈËı‹ÛÂȘ ΢ڛˆ˜ ÙÔ˘
ÂÚÈ·ÁÁÂÈ·ÎÔ‡, ÙÔ˘ ÂÚÈ‚ÚÔÁ¯ÈÎÔ‡ ÈÛÙÔ‡ Î·È ÙÔ˘ ÂÓ‰Ô-
΢„ÂÏȉÈÎÔ‡ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ (57). ∆· ·Ú·¿Óˆ ¢ڋ-
Ì·Ù· ÌÔÚ› Ó· ·Ú·¤ÌÔ˘Ó Û SARS ‹ ARDS (57).
™ÙËÓ ˘ÔÏÔÁÈÛÙÈ΋ Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·, Ù·
Â˘Ú‹Ì·Ù· ÔÈΛÏÏÔ˘Ó Î·È ÌÔÚ› Ó· Â›Ó·È ·ÎfiÌ· ηÈ
Ê˘ÛÈÔÏÔÁÈο, ·Ú¿ Ù· ¤ÓÙÔÓ· Û˘ÌÙÒÌ·Ù· ·fi ÙÔ
∫¡™ (8). ÕÏÏÔÙ ÌÔÚ› Ó· ¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ ·˘ÍË-
̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›ÂÛ˘, ·ÓÒÌ·ÏË ÏÂÙÔÌËÓÈÁÁÈ΋
ÂÓ›Û¯˘ÛË, ÂÛÙȷ΋ Ó¤ÎÚˆÛË Î·È ˘fi˘ÎÓ˜ ÓÂÎÚˆÙÈ-
Τ˜ ÂÚÈÔ¯¤˜, Ì ·ÒÏÂÈ· ·ÚÂÁ¯˘Ì·ÙÈÎÔ‡ fiÁÎÔ˘ ηÈ
·ÙÚÔÊ›· (8,50). ™ÙË Û˘Ó¤¯ÂÈ·, ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó
ÂÚÈÔ¯¤˜ Ì ӤÎÚˆÛË Î·È ÂÛÙȷ΋ ‹ ‰È¿¯˘ÙË ·ÔÌ˘Â-
ϛӈÛË. ∂›Û˘, ÂÚÈÁÚ¿ÊÔÓÙ·È ÂÛÙȷΤ˜ ·ÈÌÔÚÚ·Á›Â˜
Î·È ‰È¿Ù·ÛË ÙÔ˘ ˘ÔÛÎÏËÚ›‰ÈÔ˘ ¯ÒÚÔ˘ (50). ™ÙÔ˘˜
·ÛıÂÓ›˜ Ì ÌËÓÈÁÁ›Ùȉ· ÌfiÓÔ, ÌÔÚ› Ó· ·ÓȯÓ‡ÔÓÙ·È
ÌfiÓÔ ·ÓˆÌ·Ï›Â˜ ÛÙË Ï¢΋ Ô˘Û›· (55).
¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË
∆Ô ∞º™ ÌÔÚ› Ó· ÌÈÌÂ›Ù·È ‰È¿ÊÔÚ· ÓÔÛ‹Ì·Ù·
Î·È ÁÈ’ ·˘Ùfi Ú¤ÂÈ Ó· ‰È·ÊÔÚԉȷÁÓˆÛı› ·fi ÔÏ-
Ï·Ï‹ ·Ó¿ÚÎÂÈ· ÔÚÁ¿ÓˆÓ, ˘ÚÂÙfi ·ÁÓÒÛÙÔ˘ ·È-
ÙÈÔÏÔÁ›·˜, ÛË„·ÈÌ›·, Ë·Ù›Ùȉ·, ÔÏÏ·Ï‹ ÛÎÏ‹-
Ú˘ÓÛË, Û‡Ó‰ÚÔÌÔ Reye, ARDS, SARS, ÔÍ›· ÓÂÎÚˆÙÈ-
΋ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·, ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙÔ˘ Leigh, η-
ÎfiËı˜ ÁÏÔ›ˆÌ·, ÔÍ›· ·ÈÌÔÚÚ·ÁÈ΋ Ï¢ÎÔÂÁÎÂÊ·-
Ï›Ùȉ·, ۈ̷ÙÈ΋ ηÎÔÔ›ËÛË, ·ÏÏÂÚÁÈ΋ ÔÚʇڷ,
ÈÛÙÈÔ΢ÙÙ¿ÚˆÛË ·fi ·ÙÙ·Ú· ÙÔ˘ Langerhans, ϤÌ-
ʈ̷ ‹ ¿ÏϘ ηÎÔ‹ıÂȘ (3,11,12,49,50,61).
∞fi Ù· ·Ú·¿Óˆ Á›ÓÂÙ·È Î·Ù·ÓÔËÙfi ÁÈ·Ù› Ë ‰È¿-
ÁÓˆÛË Û˘Ó‹ıˆ˜ ηı˘ÛÙÂÚ› Î·È Á›ÓÂÙ·È Û˘Ó‹ıˆ˜ ÌÂ-
Ù¿ ÙÔ ı¿Ó·ÙÔ.
£Âڷ›·
√ ¿ÌÂÛÔ˜ ÛÙfi¯Ô˜ Ù˘ ıÂڷ›·˜ Â›Ó·È Ë Î·Ù·ÛÙÔ-
Ï‹ Ù˘ ÛÔ‚·Ú‹˜ ˘ÂÚÊÏÂÁÌÔÓ‹˜, Ô˘ ıˆÚ›ٷÈ
˘Â‡ı˘ÓË ÁÈ· Ù· ·ÂÈÏËÙÈο ÁÈ· ÙË ˙ˆ‹ Û˘ÌÙÒÌ·-
Ù·. ¢Â˘ÙÂÚ¢fiÓÙˆ˜, ÛÎÔfi˜ Â›Ó·È Ë Î·Ù·ÛÙÚÔÊ‹ ÙˆÓ
·Û¯fiÓÙˆÓ ·ÓÙÈÁÔÓÔ·ÚÔ˘ÛÈ·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È
Ë ÂÍÔ˘‰ÂÙ¤ÚˆÛË Ì ÙÔÓ ÙÚfiÔ ·˘Ùfi ÙÔ˘ ÂÚÂı›ÛÌ·ÙÔ˜
ÁÈ· ¤ÓÙÔÓË ·ÏÏ¿ ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ‰Ú·ÛÙËÚÈÔÔ›Ë-
ÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¡∫ Î·È Î·Ù·ÛÙ·ÏÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
∆. ™ÙȘ ÂÚÈÙÒÛÂȘ Ì ÁÂÓÂÙÈÎfi ˘fiÛÙڈ̷, Ô ·fi-
Ï˘ÙÔ˜ ÛÙfi¯Ô˜ ı· Ú¤ÂÈ Ó· Â›Ó·È Ë ·ÓÙÈηٿÛÙ·ÛË
ÙÔ˘ ÌÂÈÔÓÂÎÙÈÎÔ‡ ·Ì˘ÓÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ·fi ·ÙÙ·-
Ú· Ê˘ÛÈÔÏÔÁÈ΋˜ ·ÓÔÛÔÏÔÁÈ΋˜ ηٿÛÙ·Û˘ (13).
∆Ô 1994, Ë πÛÙÈÔ΢ÙÙ·ÚÈ΋ ∂Ù·ÈÚ›· (Histiocyte
Society), ÍÂΛÓËÛ ÚÔÔÙÈ΋ ıÂڷ¢ÙÈ΋ ¤Ú¢ӷ
Û˘ÓÂÚÁ·Û›·˜ (ÙÔ ÚˆÙfiÎÔÏÏÔ HLH-94), ÌÂ ÛÙfi¯Ô ÙË
‚ÂÏÙ›ˆÛË ÙÔ˘ ÔÛÔÛÙÔ‡ ÂÈ‚›ˆÛ˘ (1,28).
ª¤¯ÚÈ ÙfiÙÂ, Ë ıÓËÙfiÙËÙ· ·ÓÂÚ¯fiÙ·Ó Û >90%. ∆Ô
2004 ÍÂΛÓËÛÂ Ë ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ÚˆÙÔÎfiÏÏÔ˘ HLH-
2004, ÌÈ·˜ Ó¤·˜ ¤Î‰ÔÛ˘ ÙÔ˘ ·Ï·ÈfiÙÂÚÔ˘ (58).
™Ù· ÚˆÙfiÎÔÏÏ· ·˘Ù¿ Û˘Ó‰˘¿˙ÔÓÙ·È Ë ¯ËÌÂÈÔ-
ıÂڷ›· Î·È Ë ·ÓÔÛÔıÂڷ›·, ÌÂ Û˘Ó·ÎfiÏÔ˘ıË
ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ÁÈ· ÙȘ ›ÌÔÓ˜,
˘ÔÙÚÔÈ¿˙Ô˘Û˜ ‹/Î·È ÔÈÎÔÁÂÓ›˜ ÌÔÚʤ˜ (1).
∏ ıÂڷ›· ¤Ó·Ú͢ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙË ¯ÔÚ‹ÁËÛË
‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È ÂÙÔÔÛ›‰Ë˜, Ô˘ ·ÎÔÏÔ˘ıÔ‡-
ÓÙ·È ·fi Û˘Ó¯‹ ıÂڷ›· Ì ΢ÎÏÔÛÔÚ›ÓË ∞, Ì·˙›
Ì ÒÛÂȘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È ÂÙÔÔÛ›‰Ë˜, ÁÈ· ÂÚ›-
Ô˘ 1 ÚfiÓÔ. ∞˘Ù‹ Ë ıÂڷ›· ÌÔÚ› Ó· Â›Ó·È Â·Ú-
΋˜ ÁÈ· ·È‰È¿ ¯ˆÚ›˜ ÂÏÏ›ÌÌ·Ù· ÛÙ· ·ÙÙ·Ú· ¡∫,
¯ˆÚ›˜ ÂӉ›ÍÂȘ HLH ‹ fiÙ·Ó ÙÔ HLH Â›Ó·È Î·ı·Ú¿
·ÔÙ¤ÏÂÛÌ· Ïԛ̈͢, ΢ڛˆ˜ ·fi ÙÔÓ EBV (59).
∏ ÂÙÔÔÛ›‰Ë Â›Ó·È ¤Ó· ÂÍ·ÈÚÂÙÈÎfi Ê¿ÚÌ·ÎÔ ÁÈ·
Ó· ÚÔηϤÛÂÈ ¤Ó·ÚÍË Ù˘ ·fiÙˆÛ˘, Ì ·ÚÂÓ¤Ú-
ÁÂȘ ÙË ÛÔ‚·Ú‹ Ô˘‰ÂÙÂÚÔÂÓ›· ηÈ, Û·ÓÈfiÙÂÚ·, ÙË
‰ËÌÈÔ˘ÚÁ›· ÔÍ›·˜ Ì˘ÂÏÔÁÂÓÔ‡˜ Ï¢¯·ÈÌ›·˜ Î·È Ì˘Â-
ÏÔ‰˘ÛÏ·ÛÙÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ (1).
∏ ‰ÂÍ·ÌÂı·˙fiÓË ‰ÈÂÈÛ‰‡ÂÈ ÛÙÔÓ ·ÈÌ·ÙÂÁÎÂÊ·ÏÈ-
Îfi ÊÚ·ÁÌfi Ì ηϤ˜ ·ÓÙÈÊÏÂÁÌÔÓÒ‰ÂȘ Î·È ÚÔ·Ô-
ÙˆÙÈΤ˜ ȉÈfiÙËÙ˜, ÂÓÒ Ë Î˘ÎÏÔÛÔÚ›ÓË ∞ ÌÂÈÒÓÂÈ
ÙË ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆, Ô˘ Â›Ó·È ·˘ÍË-
̤ÓË ÛÙÔ HLH Î·È ÌÔÚ› Ó· ÌÂÈÒÛÂÈ ÙË Û˘¯ÓfiÙËÙ·
ÙˆÓ ı·Ó·ÙËÊfiÚˆÓ ÏÔÈÌÒÍÂˆÓ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ
ÙËÓ Ô˘‰ÂÙÂÚÔÂÓ›· (1,60).
™ÙË ÁÂÓÈÎfiÙÂÚË ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÓfiÛÔ˘ ¤¯Ô˘Ó
¯ÚËÛÈÌÔÔÈËı› Ï·ÛÌ·Ê·›ÚÂÛË, ·Ê·ÈÌ·ÍÔÌÂÙ¿ÁÁÈ-
ÛË, ·ÈÌԉȇÏÈÛË Î·È ¯ÔÚ‹ÁËÛË ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ, ÂÓÒ
·ÌÊÈÏÂÁfiÌÂÓË ·Ú·Ì¤ÓÂÈ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·
Ù˘ ÂÓ‰ÔÚÚ·¯È·›·˜ ¤Á¯˘Û˘ ÌÂıÔÙÚÂÍ¿Ù˘ (3,59).
™ÙȘ ‹È˜ ÌÔÚʤ˜ HLH ·fi ÏÔÈÌ҉˜ ·›ÙÈÔ, ·Ú-
Λ ÌfiÓÔ ·ÓÙÈÏÔÈÌ҉˘ ·ÁˆÁ‹, fiˆ˜ Û ·ÛıÂÓ›˜ ÌÂ
ÏÂ˚ÛÌ·Ó›·ÛË.
∏ ÂÙÂÚfiÏÔÁË ÌÂÙ·ÌfiÛ¯Â˘ÛË ·ÈÌÔÔÈËÙÈÎÒÓ Î˘Ù-
Ù¿ÚˆÓ ·Ú¤¯ÂÈ Ù· ηχÙÂÚ· ıÂڷ¢ÙÈο ·ÔÙÂϤ-
ÛÌ·Ù· (3,11,50,55,56). ∞ӷʤÚÂÙ·È ÂÈ‚›ˆÛË 62% ÛÂ
94 Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.
Paediatriki 2009;72:16-22
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·94
‰È¿ÊÔÚ˜ ÌÂϤÙ˜, ÔÛÔÛÙfi ۯ‰fiÓ ·ÓÔÌÔÈfiÙ˘Ô
(62%±12%) Ì ÙËÓ ÙÚÈÂÙ‹ ÂÈ‚›ˆÛË ·È‰ÈÒÓ (¿-
Û¯ÔÓÙ· ·fi FHL ‹ ›ÌÔÓ˜-˘ÔÙÚÔÈ¿˙Ô˘Û˜ ÌË
ÔÈÎÔÁÂÓ›˜ ÌÔÚʤ˜) Ô˘ ·ÎÔÏÔ‡ıËÛ·Ó ÙÔ HLH-94
ÚˆÙfiÎÔÏÏÔ (1,13). ∫·ıÒ˜ Ë ‰È¿ÁÓˆÛË Û˘¯Ó¿ ηı˘-
ÛÙÂÚ› Î·È Ë ÓfiÛÔ˜ Â›Ó·È ·ÚÎÂÙ¿ ÂÈıÂÙÈ΋, ·ÚÎÂÙÔ›
·ÛıÂÓ›˜ ηٷϋÁÔ˘Ó ÚÈÓ Ï¿‚Ô˘Ó HCT. ∞˘Ù‹ Ë ıÂ-
ڷ›· ·ÊÔÚ¿ ÔÌÔ˙˘ÁÒÙ˜ ÁÈ· ÌÂÙ·ÏÏ¿ÍÂȘ ‰È·Êfi-
ÚˆÓ ÁÔÓȉ›ˆÓ Û¯ÂÙÈο Ì ÙÔ ∞º™, ·ÛıÂÓ›˜ Ô˘
··ÓÙÔ‡Ó ·Ó·ÚÎÒ˜ ÛÙËÓ ·Ú¯È΋ ıÂڷ›· ηÈ
ÂΛÓÔ˘˜ Ì ÓfiÛÔ ÙÔ˘ ∫¡™.
√È ÂÚÈÛÛfiÙÂÚÔÈ ÂȉÈÎÔ› ıˆÚÔ‡Ó fiÙÈ Ë ¿ÌÂÛË
¤Ó·ÚÍË ·ÓÔÛÔ¯ËÌÂÈÔıÂڷ›·˜, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜
ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÚԷӷʤÚıËηÓ, ·ÎÔÏÔ˘ıÔ‡-
ÌÂÓË ·fi HCT, Ê·›ÓÂÙ·È ˆ˜ Ë ÌfiÓË ıÂڷ¢ÙÈ΋ ‰È·-
‰Èηۛ· Ô˘ Â›Ó·È ÈηӋ Ó· ·Ó·ÛÙ›ÏÂÈ ÙËÓ ÂͤÏÈÍË Ù˘
ÓfiÛÔ˘ ÙÔ˘ ∫¡™ Î·È Ó· ÂÈʤÚÂÈ ÙËÓ ›·ÛË, ·Ó Á›ÓÂÈ Û¯Â-
ÙÈο ÓˆÚ›˜, ·ÊÔ‡ ÚÒÙ· ÂÈÙ¢¯ı› ‡ÊÂÛË (12,55).
∆ÂÏÂÈÒÓÔÓÙ·˜, ˘ÔÁÚ·ÌÌ›˙ÂÙ·È Ë ·Ó¿ÁÎË ÁÈ· ÙËÓ
¢·ÈÛıËÙÔÔ›ËÛË ÙˆÓ È·ÙÚÒÓ ÚÔ˜ ÙË ÓfiÛÔ Î·È ÙËÓ
¤ÁηÈÚË ‰È¿ÁÓˆÛ‹ Ù˘, ÁÈ·Ù› ηٷϋÁÂÈ Û¯Â‰fiÓ ¿-
ÓÙ· Û ı¿Ó·ÙÔ, ·Ó ‰ÂÓ ¯ÔÚËÁËı› ıÂڷ›·.
µÈ‚ÏÈÔÁÚ·Ê›·
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95∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË
¶·È‰È·ÙÚÈ΋ 2009;72:16-22
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96 Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.
Paediatriki 2009;72:16-22
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·96
¡ÂfiÙÂÚ· ‰Â‰Ô̤ӷ ÁÈ· ÙË Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË
Ã. ∆۷ηϛ‰Ë˜1, ∂. √˘ÚÁηÓÙ˙fiÁÏÔ˘2, ¡. ¡ÈÎÔÏ·˝‰Ë˜1
¶ÂÚ›ÏË„Ë: ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) ·ÔÙÂÏ› ¤Ó· ÛËÌ·ÓÙÈÎfi Úfi‚ÏËÌ· ÙˆÓ ÓÂÔÁÓÒÓ, Ì¢„ËÏ‹ ÓÔÛËÚfiÙËÙ· Î·È ıÓËÙfiÙËÙ·. ∫·Ù·Ù¿ÛÛÂÙ·È Û ‰È¿ÊÔÚÔ˘˜ Ù‡Ô˘˜, ·Ó¿ÏÔÁ· Ì ÙȘ ˘¿Ú¯Ô˘Û˜·Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜. ∏ Û˘¯ÓfiÙËÙ· Ù˘ ™¢∫ Â›Ó·È 1 ·Ó¿ 2.000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ÓÂÔÁÓÒÓ. ∆Ș ÙÂÏ¢-Ù·›Â˜ ‰‡Ô ‰ÂηÂٛ˜, Ù· ÔÛÔÛÙ¿ ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ¤¯Ô˘Ó ·˘ÍËı›, Ë ·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ™¢∫¤¯ÂÈ Î·Ù·ÓÔËı› ηχÙÂÚ· Î·È Ë ÎÏÈÓÈ΋ ·ÓÙÈÌÂÙÒÈÛË ¤¯ÂÈ ‚ÂÏÙȈı›. ∆· ÓÂÔÁÓ¿ Ì ™¢∫ Û˘¯Ó¿ ¤¯Ô˘Ó ·Ó¿-ÁÎË ÂÓÙ·ÙÈ΋˜ ıÂڷ›·˜ Î·È ·Ú·ÙÂٷ̤Ó˘ ÓÔÛËÏ›·˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË Î·È ÂÌÊ·Ó›˙Ô˘Ó Û˘Ó˘¿Ú¯Ô˘-Û˜ ·ÓˆÌ·Ï›Â˜. √È ÛËÌ·ÓÙÈÎfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ Â›Ó·È ˘Â‡ı˘ÓÔÈ ÁÈ· ÙËÓ Î·Î‹ ÚfiÁÓˆÛË Â›Ó·È Ë˘ÔÏ·Û›· ÙˆÓ Ó¢ÌfiÓˆÓ Î·È Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÔ˘˜ È·ÙÚÔÁÂÓ›˜ ÙÚ·˘Ì·ÙÈ-ÛÌÔ‡˜ Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi. ∂ÓÙÔ‡ÙÔȘ, ÓÂfiÙÂÚ· ‰Â‰Ô̤ӷ Ù˘ ·ıÔÊ˘ÛÈÔÏÔ-Á›·˜ Ù˘ ÓfiÛÔ˘ Î·È ÙˆÓ Ì˯·ÓÈÛÌÒÓ Ô˘ ÂÚÌËÓÂ‡Ô˘Ó ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· ¤¯Ô˘Ó Ô‰ËÁ‹ÛÂÈ ÛÂÛËÌ·ÓÙÈΤ˜ ·ÏÏ·Á¤˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÓÂÔÁÓÒÓ Ì ™¢∫.
§¤ÍÂȘ ÎÏÂȉȿ: ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÓÂÔÁÓfi, ıÂڷ›·.
Current data on congenital diaphragmatic hernia
C. Tsakalidis1, E. Ourgantzoglou2, N. Nikolaidis1
Abstract: Congenital diaphragmatic hernia (CDH) is considered a serious problem of the newborns,associated with high mortality and morbidity. It can be subdivided into several different types,depending on the affected anatomical structures. The incidence of CDH may be as high as 1 in 2,000of living births. Over the past two decades, antenatal diagnosis rates have increased, thepathophysiology of CDH has become better understood and advances in clinical care have occurred.Infants with CDH often have other congenital anomalies and require intensive treatment after birth,leading to prolonged hospitalisation. The major factors which are associated with poor prognosis arepersistent pulmonary hypertension and lung hypoplasia in combination with iatrogenic injuriescaused by the mechanical ventilation. However, newer data of pathophysiology of the disease andthe mechanisms of the respiratory distress have resulted in improving the treatment of newbornswith CDH.
Key words: Congenital diaphragmatic hernia, neonate, treatment.
97∞¡∞™∫√¶∏™∏ REVIEW ARTICLE
1 µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋,∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ£ÂÛÛ·ÏÔӛ΢,¡ÔÛÔÎÔÌ›ԫ¶··ÁˆÚÁ›Ô˘»,£ÂÛÛ·ÏÔÓ›ÎË
2 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ£ÂÛÛ·ÏÔӛ΢,¡ÔÛÔÎÔÌÂ›Ô «∞Ã∂¶∞»,£ÂÛÛ·ÏÔÓ›ÎË
AÏÏËÏÔÁÚ·Ê›·:
ÃÚ‹ÛÙÔ˜ ∆۷ηϛ‰Ë˜[email protected]µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋,∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ£ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌ›ԫ¶··ÁˆÚÁ›Ô˘»,£ÂÛÛ·ÏÔÓ›ÎË
1 2nd Department ofNeonatal Paediatrics,“Aristotelio” University ofThessaloniki,“Papageorgiou” Hospital,Thessaloniki, Greece
2 2nd Department ofPaediatrics, “Aristotelio”University of Thessaloniki,“AHEPA” Hospital,Thessaloniki, Greece
Correspondence:
Christos [email protected] Department of NeonatalPaediatrics, “Aristotelio”University of Thessaloniki,“Papageorgiou” Hospital,Thessaloniki, Greece
¶·È‰È·ÙÚÈ΋ 2009;72:97-000
∂ÈÛ·ÁˆÁ‹
∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) ·Ú·-
̤ÓÂÈ ·ÎfiÌË ¤Ó· Úfi‚ÏËÌ·-ÚfiÎÏËÛË, ÙfiÛÔ
ÁÈ· ÙÔ˘˜ ÓÂÔÁÓÔÏfiÁÔ˘˜ fiÛÔ Î·È ÁÈ· ÙÔ˘˜ ·È‰Ô-
¯ÂÈÚÔ˘ÚÁÔ‡˜. ¶·Ú¿ ÙȘ ÚfiÛÊ·Ù˜ ÚÔfi‰Ô˘˜
ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛ‹ Ù˘, ÂÍ·ÎÔÏÔ˘ı› Ó· ¯·Ú·-
ÎÙËÚ›˙ÂÙ·È ·fi ˘„ËÏ‹ ÓÔÛËÚfiÙËÙ· Î·È ıÓËÙfi-
ÙËÙ·. ∏ ˘ÔÏ·Û›· ÙˆÓ Ó¢ÌfiÓˆÓ, Ë Ó¢ÌÔ-
ÓÈ΋ ˘¤ÚÙ·ÛË, Ë ‚ÈÔ¯ËÌÈ΋ Î·È ‰ÔÌÈ΋ ·ÓˆÚÈ-
ÌfiÙËÙ· ÙˆÓ Ó¢ÌfiÓˆÓ, ·fi ÎÔÈÓÔ‡ Ì ÙÔ˘˜ È·-
ÙÚÔÁÂÓ›˜ ÙÚ·˘Ì·ÙÈÛÌÔ‡˜ Ô˘ ÚÔηÏÔ‡ÓÙ·È
·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi, ıˆÚÔ‡ÓÙ·È ÔÈ ÛË-
Ì·ÓÙÈÎfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ Â›Ó·È ˘Â‡ı˘ÓÔÈ
ÁÈ· ÙË ÊÙˆ¯‹ ÚfiÁÓˆÛË.
∂ÓÙÔ‡ÙÔȘ, Ù· ÓÂfiÙÂÚ· ‰Â‰Ô̤ӷ Ù˘ ·ıÔ-
Ê˘ÛÈÔÏÔÁ›·˜ Ù˘ ÓfiÛÔ˘ Î·È ÙˆÓ Ì˯·ÓÈÛÌÒÓ
Ô˘ ÂÚÌËÓÂ‡Ô˘Ó ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿Ú-
ÎÂÈ· ¤¯Ô˘Ó Ô‰ËÁ‹ÛÂÈ Û ÛËÌ·ÓÙÈΤ˜ ·ÏÏ·Á¤˜
ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÓÂÔÁÓÒÓ Ì ™¢∫.
√ÚÈÛÌfi˜ - ·Ó·ÙÔÌÈ΋ ηٿٷÍË
∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) ›ӷÈ
‰˘ÛÏ·Û›· ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È ÚfiÙˆ-
ÛË ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ ̤۷ ÛÙÔÓ ıÒÚ·-
η, ‰È·Ì¤ÛÔ˘ ÂÓfi˜ ·Ó·ÙÔÌÈÎÔ‡ ÂÏÏ›ÌÌ·ÙÔ˜
ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. ∏ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÌÔ-
Ú› Ó· ·ÊÔÚ¿ ‰È·ÊÔÚÂÙÈο ÙÌ‹Ì·Ù· ÙÔ˘ ‰È·-
ÊÚ¿ÁÌ·ÙÔ˜. ¶ÚfiÛÊ·Ù· ÚÔÙ¿ıËÎÂ ÌÈ· ·Ó·ÙÔ-
ÌÈ΋ ηٿٷÍË ÙˆÓ ·ÓˆÌ·ÏÈÒÓ ÙÔ˘ ‰È·ÊÚ¿Á-
Ì·ÙÔ˜ (1). √ ÈÔ ÎÔÈÓfi˜ Ù‡Ô˜ ‰È·ÊÚ·ÁÌ·ÙÔ-
΋Ï˘ Â›Ó·È Ë Ï·ÁÈÔ›ÛıÈ· ΋ÏË ÙÔ˘
Bochdalek, Ë Û˘¯ÓfiÙËÙ· Ù˘ ÔÔ›·˜ ÂÎÙÈÌ¿Ù·È
ÂÚ›Ô˘ ÛÙÔ 90-95% (2). T· ÔÛÔÛÙ¿ ÙˆÓ ‰ÂÍÈ¿
Î·È ·ÚÈÛÙÂÚ¿ ÂÓÙÔÈ˙fiÌÂÓˆÓ ™¢∫ ÔÈΛÏÏÔ˘Ó,
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·97
Ì ÙËÓ ·ÚÈÛÙÂÚ‹ Ó· ·Ó¤Ú¯ÂÙ·È ÛÙÔ 73-90%, ÙË ‰ÂÍÈ¿ÛÙÔ 8-24% Î·È ÙËÓ ·ÌÊÔÙÂÚfiÏ¢ÚË ‹ ÎÂÓÙÚÈ΋ ÛÙÔ1,2-4,6% (3-5). √È ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜Ô˘ ‰ÂÓ ÂÓÙÔ›˙ÔÓÙ·È ÛÙ· Ï¿ÁÈ· Â›Ó·È Û¿ÓȘ ηÈÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 1-6% ÙˆÓ ·ÛıÂÓÒÓ. √È ÏÈÁfiÙÂÚÔÛ˘¯ÓÔ› Ù‡ÔÈ ™¢∫ ·ÊÔÚÔ‡Ó ÙËÓ ÔÈÛıÔÛÙÂÚÓÈ΋‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ‹ ΋ÏË ÙÔ˘ Morgagni, ÙËÓ Úfi-ÛıÈ· ‹ ÂÁοÚÛÈ· ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÙËÓ ÎÂÓÙÚÈ΋‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÙËÓ ·Ó‡„ˆÛË ‹ Ï¤Ù˘ÓÛË ÙÔ˘‰È·ÊÚ¿ÁÌ·ÙÔ˜ Î·È ÙËÓ ·ÁÂÓÂÛ›· ·˘ÙÔ‡ (1) (∂ÈÎfiÓ·1). ∫ËÏÈÎfi˜ Û¿ÎÔ˜ ·ÔÙÂÏÔ‡ÌÂÓÔ˜ ·fi ÏÂÙ‹ ÌÂ-ÛÔıËÏȷ΋ ÌÂÌ‚Ú¿ÓË ÂÌÊ·Ó›˙ÂÙ·È Û ÏÈÁfiÙÂÚÔ ·fi10% ÙˆÓ ÂÚÈÙÒÛˆÓ. ™ÙËÓ ·ÚÈÛÙÂÚ‹ ™¢∫, Ù· ÎÔÈ-Ïȷο ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó ÛÙÔÓ ıÒڷη ›-Ó·È ÙÔ ÛÙÔÌ¿¯È, ÙÔ ÏÂÙfi ¤ÓÙÂÚÔ, ÙÔ ÎfiÏÔÓ, Ô ÛÏ‹-Ó·˜ Î·È Ô ·ÚÈÛÙÂÚfi˜ ÏÔ‚fi˜ ÙÔ˘ ‹·ÙÔ˜. ¶ÂÚÈÛÙ·ÛÈ·-ο ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÙÔ ¿ÁÎÚ·˜, Ô ÓÂÊÚfi˜ Î·È ÙÔ·ÚÈÛÙÂÚfi ÂÈÓÂÊÚ›‰ÈÔ. ™ÙË ‰ÂÍÈ¿ ™¢∫, Ôχ ÏÈÁfiÙÂ-ÚÔ ¤ÓÙÂÚÔ ‚Ú›ÛÎÂÙ·È ÛÙÔÓ ıÒڷη, ‰ÈfiÙÈ ÙÔ ‹·Ú·ÔÊÚ¿ÛÛÂÈ ÙÔ ¤ÏÏÂÈÌÌ·.
™˘¯ÓfiÙËÙ·
∏ Û˘¯ÓfiÙËÙ· Ù˘ ™¢∫ ·Ó¤Ú¯ÂÙ·È ·fi 1 ÛÙȘ 2.000¤ˆ˜ 1 ÛÙȘ 4.000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ÓÂÔÁÓÒÓ Î·È ·Ô-ÙÂÏ› ÙÔ 8% fiÏˆÓ ÙˆÓ Û˘ÁÁÂÓÒÓ ‰˘ÛÏ·ÛÈÒÓ (6). ∆·ÂÚÈÛÛfiÙÂÚ· ÓÂÔÁÓ¿ Â›Ó·È ÙÂÏÂÈfiÌËÓ·. §›Á˜ ‰ËÌÔ-ÛȇÛÂȘ ·Ó·Ï‡Ô˘Ó ÙË ‰È·ÊÔÚ¿ ʇÏÔ˘ ÛÙË ™¢∫. ∏Û¯¤ÛË ¿ÚÚÂÓ·:ı‹Ï· ˘ÔÏÔÁ›˙ÂÙ·È Û 1:1,25 (7), 3:2(8), ÂÓÒ ¿ÏϘ ‰ËÌÔÛȇÛÂȘ ‰ÂÓ ·Ó·Ê¤ÚÔ˘Ó ‰È·ÊÔ-Ú¿ fiÛÔÓ ·ÊÔÚ¿ ÙÔ Ê‡ÏÔ (9). √È ·Ó·ÊÔÚ¤˜ ÂÚÈÛÙ·ÙÈ-ÎÒÓ ÂÌÊ¿ÓÈÛ˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ Û ‰È‰‡ÌÔ˘˜,Û˘ÁÁÂÓ›˜ Î·È ·ÔÁfiÓÔ˘˜ ÙˆÓ ·ÛıÂÓÒÓ Â›Ó·È ÛÔÚ·-‰ÈΤ˜. ™Â ÔÈÎÔÁ¤ÓÂȘ Ô˘ ·Ú·ÙËÚ‹ıËΠϋÚ˘·ÁÂÓÂÛ›· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ‚Ú¤ıËΠÎÏËÚÔÓÔÌÈÎfi˜·˘ÙÔۈ̷ÙÈÎfi˜ ˘ÔÏÂÈfiÌÂÓÔ˜ ¯·Ú·ÎÙ‹Ú·˜.
™˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜
∂ÎÙfi˜ ·fi ÙȘ ηٷÛÙ¿ÛÂȘ Ô˘ ıˆÚÔ‡ÓÙ·È fiÙÈ·ÔÙÂÏÔ‡Ó Ì¤ÚÔ˜ ÙÔ˘ «Û˘Ó‰ÚfiÌÔ˘ Ù˘ ‰È·ÊÚ·ÁÌ·-ÙÔ΋Ï˘» (Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·, ·ÓÔȯÙfi˜ ·ÚÙË-ÚÈ·Îfi˜ fiÚÔ˜, ·ÓÔȯÙfi ÔÂȉ¤˜ ÙÚ‹Ì· Î·È ·ÙÂÏ‹˜ Û˘-ÛÙÚÔÊ‹ ÙÔ˘ ÂÓÙ¤ÚÔ˘), ÂÚ›Ô˘ ÙÔ 40% ÙˆÓ ˙ÒÓÙˆÓ
ÓÂÔÁÓÒÓ Ì ™¢∫ ¤¯Ô˘Ó Ì›· ‹ ÂÚÈÛÛfiÙÂÚ˜ Û˘ÓÔ‰¤˜
‰È·Ì·Úٛ˜, Û˘¯Ó¿ ˆ˜ ̤ÚÔ˜ ÂÓfi˜ ·Ó·ÁÓˆÚÈṲ̂ÓÔ˘
Û˘Ó‰ÚfiÌÔ˘ ‹ ¯ÚˆÌ·ÙÔÛˆÌÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ (¶›Ó·-
η˜ 1) (10,2). ™ÙÔ 60% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û˘Ó˘¿Ú-
¯Ô˘Ó ηډȷΤ˜ ‰È·Ì·Úٛ˜, ÂÓÒ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ Ô˘-
ÚÔÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ··ÓÙÔ‡Ó ÛÙÔ 15% ÙˆÓ
ÛÔÚ·‰ÈÎÒÓ Î·È ÛÙÔ 18% ÙˆÓ ÔÈÎÔÁÂÓÒÓ ÂÚÈÙÒÛÂ-
ˆÓ (11). ∞ӈ̷ϛ˜ ÙÔ˘ Á·ÛÙÚÂÓÙÂÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜
ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 17% ÙˆÓ ÂÚÈÙÒÛˆÓ, ÙÔ˘ ∫¡™
ÛÙÔ 14%, ÂÓÒ ÛÙÔ 10% Û˘Ó˘¿Ú¯Ô˘Ó ¯ÚˆÌÔۈ̷ÙÈ-
Τ˜ ·ÓˆÌ·Ï›Â˜. ∆Ô Û‡Ó‰ÚÔÌÔ Ù˘ ˘ÔÏ·ÛÙÈ΋˜ ·ÚÈ-
ÛÙÂÚ‹˜ ηډ›·˜ ·ÚÔ˘ÛÈ¿˙ÂÙ·È Ì ¤Ó· ¢ڇ Ê¿ÛÌ·
·ÓˆÌ·ÏÈÒÓ, Ô˘ Î˘Ì·›ÓÂÙ·È ·fi ÙËÓ ˘ÔÏ·ÛÙÈ΋
·ÚÈÛÙÂÚ‹ ηډȿ ¤ˆ˜ ÙËÓ ·Ó·ÙÔÌÈο Ê˘ÛÈÔÏÔÁÈ΋
ηډȿ, Ë ÔÔ›· fï˜ ÛÙÔ Û‡ÓÔÏfi Ù˘ Â›Ó·È ˘ÔÏ·-
ÛÙÈ΋ (3). ¢ÂÓ ¤¯ÂÈ ‰È¢ÎÚÈÓÈÛÙ› ÁÈ·Ù› Û˘Ì‚·›ÓÂÈ ·˘-
Ùfi. ª›· ıˆڛ· Â›Ó·È fiÙÈ Ë ÂÈÛÙÚÔÊ‹ ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡
ÊÏ‚ÈÎÔ‡ ·›Ì·ÙÔ˜ ‰È·Ì¤ÛÔ˘ ÙÔ˘ ˆÔÂȉԇ˜ ÙÚ‹Ì·ÙÔ˜
ÛÙËÓ ·ÚÈÛÙÂÚ‹ ÏÂ˘Ú¿ Ù˘ ηډȿ˜ ÌÔÚ› Ó· ÂÏ·Ù-
Ùˆı› ·fi ÙË ÌÂÙ·ÙfiÈÛË Ù˘ ηډȿ˜ ̤۷ ÛÙÔÓ ıÒ-
ڷη, ÔfiÙÂ Ë ·ÏÏ·Á‹ ÛÙË ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜ ÌÔÚ›
Ó· ÚÔηϤÛÂÈ ˘ÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜
(12). ¢˘ÛÌÔÚÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο Î·È ·ÓˆÌ·Ï›Â˜
ÙˆÓ ¿ÎÚˆÓ Â›Ó·È Û˘¯ÓfiÙÂÚ· ÛÙËÓ ·ÚÈÛÙÂÚ¿ ÂÓÙÔÈ˙fi-
ÌÂÓË ™¢∫ (13). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ë Û˘¯ÓfiÙËÙ·
ÙˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡
Î·È ÙÔ˘ Á·ÛÙÚÂÓÙÂÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÌÂÈÒıËΠÛË-
Ì·ÓÙÈο Î·È ·˘Ùfi ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È ÛÙË ‚ÂÏÙ›ˆÛË
ÙÔ˘ ÚÔÁÂÓÓËÙÈÎÔ‡ ÂϤÁ¯Ô˘ Ì ÙË ¯Ú‹ÛË ÙˆÓ ˘ÂÚ‹-
¯ˆÓ. ∏ Ì›ˆÛË, fï˜, ÙˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ÛÙ·
‰‡Ô ·˘Ù¿ Û˘ÛÙ‹Ì·Ù· ÂÈÛËÁÂ›Ù·È Â›Û˘ fiÙÈ Ë Â›Ùˆ-
ÛË ÙˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ¤¯ÂÈ ·ÏÏ¿ÍÂÈ Ù· ÙÂÏ¢-
Ù·›· ¯ÚfiÓÈ· (9).
98 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:98-000
¶›Ó·Î·˜ 1. ∞Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ Ô˘ Û˘ÓÔ‰Â‡Ô˘Ó ÙË ™¢∫
™‡ÛÙËÌ· ∫‡ÚÈ· ·ÓˆÌ·Ï›·
∫·Ú‰È·ÁÁÂÈ·Îfi ªÂÛÔÎÔÏÈ΋ ÂÈÎÔÈÓˆÓ›·ªÂÛÔÎÔÈÏȷ΋ ÂÈÎÔÈÓˆÓ›·™Ù¤ÓˆÛË ·ÔÚÙ‹˜∞ӈ̷ϛ· EbsteinÀÔÏ·ÛÙÈ΋ ·ÚÈÛÙÂÚ‹ ηډ›·
°·ÛÙÚÂÓÙÂÚÈÎfi °·ÛÙÚfiÛ¯ÈÛË√ÌÊ·ÏÔ΋ÏË∞ÙÚËÛ›· ÔÈÛÔÊ¿ÁÔ˘
√˘ÚÔÁÂÓÓËÙÈÎfi À‰ÚÔÓ¤ÊÚˆÛ˶ÔÏ˘Î˘ÛÙÈÎÔ› ÓÂÊÚÔ›
∫¡™ À‰ÚÔÎÂÊ·Ï›·¢Èۯȉ‹˜ Ú¿¯Ë
™ÎÂÏÂÙÔ‡-ÚÔÛÒÔ˘ ŒÏÏÂÈ„Ë Ï¢ÚÒÓ¶ÔÏ˘‰·ÎÙ˘Ï›·™˘Ó‰·ÎÙ˘Ï›·¶ÚÔ¤¯oÓ Ì¤ÙˆÔµÚ·¯˘ÎÂÊ·Ï›·¶Ï·ÁÈÔÎÂÊ·Ï›·
∂ÈÎfiÓ· 1. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË: ·) Ù‡Ô˘ Bochdalec, ‚) Ù‡-Ô˘ Morgagni Î·È ÚfiÛıÈ· ‹ ÂÁοÚÛÈ· ™¢∫, Á) ÎÂÓÙÚÈ΋ ™¢∫.
Ù‡Ô˘ MorgagniÚfiÛıÈ· ‹ ÂÁοÚÛÈ· ™¢∫
· ‚ Á
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·98
°ÂÓÂÙÈ΋
∏ ™¢∫ ¯ˆÚ›˜ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ÂÌÊ·Ó›˙ÂÙ·ÈÛÔÚ·‰Èο. √ÈÎÔÁÂÓ›˜ ÂÚÈÙÒÛÂȘ ··ÓÙÔ‡Ó ÛÙÔ2% (14,15), Û˘Ó‹ıˆ˜ ‰ÂÓ Û˘Óԉ‡ÔÓÙ·È ·fi ¿ÏÏÂ˜Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ Î·È ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚË Û˘¯Ófi-ÙËÙ· ·ÌÊÔÙÂÚfiÏ¢Ú˘ ÂÓÙfiÈÛ˘. √ ΛӉ˘ÓԘ·ÓÂÌÊ¿ÓÈÛ˘ ˘ÔÏÔÁ›˙ÂÙ·È Î·Ù¿ ÚÔÛ¤ÁÁÈÛË ÛÂ2% › ·Ô˘Û›·˜ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ ÈÛÙÔÚÈÎÔ‡ (16). ∏™¢∫ ÌÔÚ› Ó· ··ÓÙ¿Ù·È ˆ˜ ̤ÚÔ˜ ÂÓfi˜ Û˘Ó‰Úfi-ÌÔ˘ (17). ∆Ô˘Ï¿¯ÈÛÙÔÓ ÙÔ 10% ÙˆÓ ·ÛıÂÓÒÓ Ì ™¢∫Î·È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ·Ó‹ÎÂÈ Û οÔÈÔ ÁÓˆÛÙfiÛ‡Ó‰ÚÔÌÔ (18) (¶›Ó·Î·˜ 2), fiˆ˜ ÙÔ Û‡Ó‰ÚÔÌÔFryns (19,20). ¶Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ÛÙÔ ¯ÚˆÌfiۈ̷1q41-q42.12 ‚Ú›ÛÎÔÓÙ·È ¤Ó· ‹ ÂÚÈÛÛfiÙÂÚ· ÁÔÓ›‰È·Ô˘ ··ÈÙÔ‡ÓÙ·È ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·-ÙÔ˜. ∆· ·ÔÙÂϤÛÌ·Ù· ÁÂÓÂÙÈÎÒÓ ÌÂÏÂÙÒÓ ‰Â›¯ÓÔ˘ÓfiÙÈ ÛÙÔ ÛËÌÂ›Ô Û‡Ó‰ÂÛ˘ ÙˆÓ ÂÚÈÔ¯ÒÓ 1q41 ηÈ1q42.11 ‚Ú›ÛÎÂÙ·È Ô ÁÂÓÂÙÈÎfi˜ ÙfiÔ˜ Ô˘ Â›Ó·È˘Â‡ı˘ÓÔ˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË ™¢∫ Î·È Û˘Ó‰ÚfiÌÔ˘Fryns (19). À¿Ú¯Ô˘Ó ÌÂÌÔӈ̤Ó˜ ·Ó·ÊÔÚ¤˜ ÂÚÈ-ÙÒÛÂˆÓ ™¢∫ Ì ʷÈÓfiÙ˘Ô Ù‡Ô˘ Û˘Ó‰ÚfiÌÔ˘Fryns Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ‰ÈÏ·ÛÈ·ÛÌfi ÙÔ˘ 1q24-q31.2, ÂÍ¿ÏÂÈ„Ë ÙÔ˘ ÙÂÏÈÎÔ‡ ÙÌ‹Ì·ÙÔ˜ ÙˆÓ 6q,8p23.1, 15q26 ¯ÚˆÌ·ÙÔÛˆÌ¿ÙˆÓ Î·È ÌÂÚÈ΋ ÙÚÈÛˆ-Ì›· 22 (21,22,23). √ Ì˯·ÓÈÛÌfi˜ ‰ËÌÈÔ˘ÚÁ›·˜ Ù˘‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ÛÙ· Û‡Ó‰ÚÔÌ· ·˘Ù¿ ·Ú·Ì¤ÓÂÈ·‰È¢ÎÚ›ÓÈÛÙÔ˜. ∞ÚÎÂÙ¿ Û‡Ó‰ÚÔÌ· Ô˘ Û¯ÂÙ›˙ÔÓÙ·ÈÌ ™¢∫ ÂÌÊ·Ó›˙Ô˘Ó ÌÂÓ‰¤ÏÂÈ· ÎÏËÚÔÓÔÌÈÎfiÙËÙ·Î·È Û ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ Ë ÂÚÈÔ¯‹ Ô˘ ‰ڿ˙Ô-ÓÙ·È Ù· ÁÔÓ›‰È· ·˘Ù¿ ‹ Î·È Ë Ù·˘ÙfiÙËÙ· ÙˆÓ ÁÔÓȉ›ˆÓ·˘ÙÒÓ Â›Ó·È ÁÓˆÛÙ¤˜. ∏ ‡·ÚÍË ÁÂÓÂÙÈÎÒÓ Û˘Ó‰Úfi-ÌˆÓ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ™¢∫ ·ÔÙÂÏ› ÈÛ¯˘Ú‹ ·fi-‰ÂÈÍË fiÙÈ ÛÙËÓ ÂÌÊ¿ÓÈÛ‹ Ù˘ ·›˙Ô˘Ó ÚfiÏÔ ÁÂÓÂÙÈÎÔ›·Ú¿ÁÔÓÙ˜. ∆Ô ÁÔÓ›‰ÈÔ WT1 ·›˙ÂÈ ÚfiÏÔ ÛÙË Ê˘-ÛÈÔÏÔÁÈ΋ ·Ó¿Ù˘ÍË ÙÔ˘ Ô˘ÚÔÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·-ÙÔ˜. ∞fi ÌÂϤÙ˜ Û ÔÓÙ›ÎÈ· ÔÌÔ˙˘ÁÒÙ˜ ÁÈ· ÙȘÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ WT1 ‚Ú¤ıËΠfiÙÈ ÔÈ ÌÂ-Ù·ÏÏ¿ÍÂȘ ÙÔ˘ ¢ı‡ÓÔÓÙ·È ÁÈ· ·ÓˆÌ·Ï›Â˜ ÙÔ˘ Ô˘ÚÔ-
ÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ ÙÔȯÒÌ·-
ÙÔ˜ Î·È ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ Ô˘ Ô‰ËÁÔ‡Ó ÛÙË ‰ËÌÈ-
Ô˘ÚÁ›· ™¢∫. ∫¿ÙÈ ·ÓÙ›ÛÙÔÈ¯Ô ‰ÂÓ ·Ô‰Â›¯ıËΠÛÂ
·ÓıÚÒÔ˘˜ (24). À¿Ú¯Ô˘Ó ·˘Í·ÓfiÌÂÓ· ÛÙÔȯ›·
Ô˘ ‰Â›¯ÓÔ˘Ó fiÙÈ ÙÔ Ì·ÎÚ‡ ÛΤÏÔ˜ ÙÔ˘ ¯ÚˆÌ·ÙÔÛÒ-
Ì·ÙÔ˜ 15 ‰È·‰Ú·Ì·Ù›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÂÌÊ¿-
ÓÈÛË ™¢∫ Î·È ÔÚÈÛÌ¤ÓˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ. ∏ Â-
ÚÈÔ¯‹ ·˘Ù‹ ÙÔ˘ ¯ÚˆÌ·ÙÔÛÒÌ·ÙÔ˜ 15 Έ‰ÈÎÔÔÈ›
ÙËÓ Î˘ÙÙ·ÚÈ΋ ÚˆÙ½ÓË-1 Ô˘ ‰ÂÛ̇ÂÈ ÙÔ ÚÂÙÈÓÔ˚-
Îfi Ô͇ (25,26,27). √È ÙÚÈۈ̛˜ 13, 18, 21 Î·È 45à ›-
Ó·È ÔÈ ÈÔ Û˘¯Ó¤˜ ·Ó¢ÏÔÂȉ›Â˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ
™¢∫ (9). ∞ӈ̷ϛ˜ fiˆ˜ ÂÍ·Ï›„ÂȘ, ‰ÈÏ·ÛÈ·-
ÛÌÔ›, ·Ó·ÛÙÚÔʤ˜ Î·È ÌÂÙ·ı¤ÛÂȘ ۯ‰fiÓ fiÏˆÓ ÙˆÓ
¯ÚˆÌ·ÙÔÛˆÌ¿ÙˆÓ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› Û ۯ¤ÛË Ì ÙË
™¢∫ (28,29,18) Î·È ¤¯Ô˘Ó ·Ó·ÁÓˆÚÈÛÙ› ˆ˜ ÛÔ˘‰·›-
Ô˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜, ÌÂ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜
Ô˘ ··ÓÙÔ‡Ó ÛÙÔ 33% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (18). ∫·-
Ú˘fiÙ˘Ô˜ ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È Û οı ·È‰› ÌÂ
™¢∫ Î·È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ Ô˘ ‰ÂÓ ·ÔÙÂÏÔ‡Ó
‰Â˘ÙÂÚ‡ԢÛ˜ Û˘Ó¤ÂȘ ·˘Ù‹˜.
¶·ıÔÊ˘ÛÈÔÏÔÁ›·
∏ ·ıÔÁ¤ÓÂÈ· Ù˘ ™¢∫ ‰ÂÓ Â›Ó·È ·fiÏ˘Ù· ηٷ-
ÓÔËÙ‹ ¤ˆ˜ Û‹ÌÂÚ·. ∏ ıˆڛ· Ù˘ ·ÔÙ˘¯Ë̤Ó˘ Û‡-
ÁÎÏÂÈÛ˘ Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘¯‹˜ Â›Ó·È Â˘-
Ú¤ˆ˜ ·Ô‰ÂÎÙ‹. ∏ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ·Ú-
¯›˙ÂÈ ·fi ÙÔ ÚfiÛıÈÔ ÙÌ‹Ì· ÙÔ˘, ˆ˜ ¤Ó· ¯ÒÚÈÛÌ· ÌÂ-
ٷ͇ Ù˘ ηډȿ˜ Î·È ÙÔ˘ ‹·ÙÔ˜, Î·È ÚÔ¯ˆÚ¿ÂÈ
ÚÔ˜ Ù· ›Ûˆ ÁÈ· Ó· ÎÏ›ÛÂÈ ÙÂÏÈο ÚÔ˜ ÙÔ ·ÚÈÛÙÂÚfi
ÙÚ‹Ì· ÙÔ˘ Bochdalek, Á‡Úˆ ÛÙËÓ 8Ë Ì 10Ë Â‚‰ÔÌ¿-
‰· ·ËÛ˘. ∆Ô ¤ÓÙÂÚÔ ÌÂÙ·Ó·ÛÙ‡ÂÈ ·fi ÙÔÓ ÏÂÎÈıÈ-
Îfi Û¿ÎÔ ÂÚ›Ô˘ ÙË 10Ë Â‚‰ÔÌ¿‰· ·ËÛ˘ ηÈ, ·Ó
ÊÙ¿ÛÂÈ ÚÈÓ ÙÔ ÎÏ›ÛÈÌÔ ÙÔ˘ ÙÚ‹Ì·ÙÔ˜, ‰ËÌÈÔ˘ÚÁ›-
Ù·È Ë Î‹ÏË. ¶ÈÛÙ‡ÂÙ·È fiÙÈ fiÙ·Ó Û˘Ó˘¿Ú¯ÂÈ ·ÓÒÌ·-
ÏË ÛÙÚÔÊ‹ ÙÔ˘ ÂÓÙ¤ÚÔ˘, ·˘Ù‹, ÏfiÁˆ ı¤Û˘, ‰ÂÓ ÂÈ-
ÙÚ¤ÂÈ ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ÌÂ Û˘Ó¤-
ÂÈ· ÙË ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË. ¢ÂÓ Â›Ó·È ÁÓˆÛÙfi ÔȘ
·Èٛ˜ Ô‰ËÁÔ‡Ó ÛÙËÓ ·ÔÙ˘¯›· Û‡ÁÎÏÂÈÛ˘ Ù˘ Ï¢-
ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘¯‹˜. ¢Â‰Ô̤ӷ ·fi ¤Ú¢Ó˜ ÛÂ
˙Ò· Ô‰ËÁÔ‡Ó ÛÙËÓ ˘fiıÂÛË ˆ˜ ¤Ó· ÌË Ì˘˚Îfi, ÌÂ-
ÛÂÁ¯˘Ì·ÙÈÎfi ÛÙÔÈ¯Â›Ô Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘-
¯‹˜ Â›Ó·È ÂÏ·Ùو̷ÙÈÎfi Î·È ‰ÂÓ ÚÔ¿ÁÂÈ ÙÔÓ Û¯ËÌ·-
ÙÈÛÌfi ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. ∞˘Ù‹ Ë ˘fiıÂÛË ÂÍËÁ› ÙË
‰È·Ù·Ú·¯‹ ÛÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ·ÏÏ¿
‰ÂÓ ‰ÈηÈÔÏÔÁ› ÙȘ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ (9).
√ Aderson ÙÔ 1941 ·Ú·Ù‹ÚËÛ fiÙÈ Ë ·Ó¿Ú-
ÎÂÈ· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ·˘Í¿ÓÂÈ ÙËÓ Â›ÙˆÛË Ù˘
™¢∫. ÕÏÏ· ‰Â‰Ô̤ӷ ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ ÙÔ Û‡ÛÙËÌ·
Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ Û˘ÌÌÂÙ¤¯ÂÈ ÛÙË ‰ËÌÈÔ˘ÚÁ›· ‰È·-
ÊÚ·ÁÌ·ÙÔ΋Ï˘, Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜ Î·È Î·Ú-
‰È·ÎÒÓ ·ÓˆÌ·ÏÈÒÓ (30,31,26). ªÂϤÙ˜ ÙÔ˘ Major
(1998) ¤‰ÂÈÍ·Ó fiÙÈ ÔÈ ÌËÙ¤Ú˜ Î·È Ù· ÓÂÔÁÓ¿ Ì ™¢∫
›¯·Ó ¯·ÌËÏfiÙÂÚ· ›‰· ÚÂÙÈÓfiÏ˘ ÛÙÔ Ï¿ÛÌ·,
99™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË
¶·È‰È·ÙÚÈ΋ 2009;72:99-000
¶›Ó·Î·˜ 2. ™‡Ó‰ÚÔÌ· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·-ÙÔ΋ÏË
ñ ™‡Ó‰ÚÔÌÔ Frynsñ ™‡Ó‰ÚÔÌÔ Beckwith-Wiedemannñ ™‡Ó‰ÚÔÌÔ Brachman-de Langeñ ™‡Ó‰ÚÔÌÔ Simpson-Golabi-Behmelñ ™‡Ó‰ÚÔÌÔ Donnaiñ ™‡Ó‰ÚÔÌÔ Denys-Drashñ ™‡Ó‰ÚÔÌÔ Perlmanñ ™‡Ó‰ÚÔÌÔ Craniofrontonasalñ ™‡Ó‰ÚÔÌÔ Spondylocostalñ ™‡Ó‰ÚÔÌÔ Marfanñ ™‡Ó‰ÚÔÌÔ ™‡Ó‰ÚÔÌÔ Donnai-Barrowñ ™‡Ó‰ÚÔÌÔ Gershoni-Baruchñ ™‡Ó‰ÚÔÌÔ Matthew-Wood
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·99
Û ۇÁÎÚÈÛË Ì ÂΛӷ ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÌËÙ¤ÚˆÓ
Î·È ÓÂÔÁÓÒÓ (32).
¶ÚfiÛÊ·Ù· Ë ™¢∫ Û¯ÂÙ›ÛÙËΠÁÂÓÂÙÈο Ì ÙÔ˘˜
·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ 10 (FGF-10) Î·È 18 (FGF-
18) Ù˘ ÊÈÌÚÔÏ·ÛÙ›Ó˘, Ô˘ ÂÎÎÚ›ÓÔÓÙ·È ·fi ÙÔ
¿ˆ Ó¢ÌÔÓÈÎfi ÌÂÛ¤Á¯˘Ì·. √ ·Ú¿ÁÔÓÙ·˜ FGF-10
ÚÔ¿ÁÂÈ ÙË ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ
΢ÙÙ¿ÚˆÓ Û ·ÙÙ·Ú· ÙˆÓ Ï›ˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ ÙˆÓ
·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ Ô˘ ·›˙Ô˘Ó ÚfiÏÔ ÛÙË ‰È·Ù‹ÚËÛË
Ù˘ ÂÓ‰Ô·˘ÏÈ΋˜ ›ÂÛ˘ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Î·Ù¿ ÙËÓ
ÚÒÈÌË Ê¿ÛË Ù˘ ·Ó¿Ù˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ Î·È
ÛÙË Ú‡ıÌÈÛË Ù˘ ¢ÂÓ‰ÔÙfiÙËÙ·˜ ÙˆÓ Ó¢ÌfiÓˆÓ
ÛÙ· ÙÂÏÂ˘Ù·›· ÛÙ¿‰È· Ù˘ ·ËÛ˘ (33). ∏ ·Ó¿Ú-
ÎÂÈ¿ ÙÔ˘ ÌÔÚ› Ó· ·˘Í‹ÛÂÈ ÙËÓ Â˘¿ıÂÈ· ÙˆÓ Ó¢-
ÌfiÓˆÓ ÛÙÔÓ ÙÚ·˘Ì·ÙÈÛÌfi ·fi Ù· ÂÓ‰ÔÎÔÈÏȷο
ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó. √ ·Ú¿ÁÔÓÙ·˜ FGF-18
ÚÔ¿ÁÂÈ ÙËÓ ·Ú·ÁˆÁ‹ ÂÏ·ÛÙÈÎÒÓ ÈÓÒÓ Î·È Ë ·ÓÂ-
¿ÚÎÂÈ¿ ÙÔ˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ÂÏ·Ùو̤ÓË ·-
Ú·ÁˆÁ‹ ·˘ÙÒÓ (34).
√ Ì˯·ÓÈÛÌfi˜ Ù˘ Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜ ÛÙË
™¢∫ ·ÔÙÂÏ› ÛËÌÂ›Ô ‰È¯ÔÁӈ̛·˜. ™‡Ìʈӷ Ì ÙËÓ
ÈÔ ·Ô‰Â¯Ù‹ ¿Ô„Ë, ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÓ‰ÔÌ‹-
ÙÚÈ·˜ ·Ó¿Ù˘Í˘, Ù· ÂÓÙfi˜ ÙÔ˘ ıÒڷη ÎÔÈÏȷο
ÛÏ¿¯Ó· Ȥ˙Ô˘Ó ÙÔÓ Û‡ÛÙÔÈ¯Ô Ó‡ÌÔÓ·, ÁÂÁÔÓfi˜
Ô˘ ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ Û ÌÈÎÚfiÙÂÚÔ ‚·ıÌfi ηÈ
ÙÔÓ ÂÙÂÚfiÏ¢ÚÔ Ó‡ÌÔÓ·, ÏfiÁˆ Ù˘ ÎÈÓËÙÈÎfiÙË-
Ù·˜ ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘ ÛÙÔ ¤Ì‚Ú˘Ô. ∞˘Ù‹ Ë ¿Ô„Ë
¤¯ÂÈ ÂȂ‚·Èˆı› ÂÈÚ·Ì·ÙÈο Û ÔÓÙÈÎÔ‡˜ ηÈ
·ÌÓÔ‡˜ (35). ™‡Ìʈӷ Ì ÙËÓ ˘fiıÂÛË ÙˆÓ «‰‡Ô
¯Ù˘ËÌ¿ÙˆÓ», ÙÔ «ÚÒÙÔ ¯Ù‡ËÌ·» ¤گÂÙ·È Óˆ-
Ú›˜ ÛÙËÓ ·Ó¿Ù˘ÍË Î·È ÂËÚ¿˙ÂÈ ÂÍ›ÛÔ˘ ÙËÓ ·Ó¿-
Ù˘ÍË ÙˆÓ ‚ÚfiÁ¯ˆÓ Î·È ÙˆÓ ‰‡Ô Ó¢ÌfiÓˆÓ. ∆Ô
«‰Â‡ÙÂÚÔ ¯Ù‡ËÌ·» Û˘Ì‚·›ÓÂÈ ÛÙ· ÙÂÏÂ˘Ù·›· ÛÙ¿‰È·
·Ó¿Ù˘Í˘ Î·È ÚÔ¤Ú¯ÂÙ·È Î˘Ú›ˆ˜ ·fi ÙÔ ‰È¿ÊÚ·Á-
Ì·, Ô˘ Ì·˙› Ì ٷ ÂÓ‰ÔÎÔÈÏȷο ÛÏ¿¯Ó· Ô˘ ÚÔ-
Û›ÙÔ˘Ó ÂËÚ¿˙ÂÈ ÙÔÓ Û‡ÛÙÔÈ¯Ô Ó‡ÌÔÓ·. ∞˘Ùfi
Ô‰ËÁ› Û Ì›ˆÛË ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÎÈÓ‹ÛÂˆÓ ÙÔ˘
ÂÌ‚Ú‡Ô˘ Î·È ÂÏ¿ÙÙˆÛË ÙÔ˘ Ú˘ıÌÔ‡ ·Ú·ÁˆÁ‹˜
Ó¢ÌÔÓÈÎÔ‡ ˘ÁÚÔ‡ (36). ŸÙ·Ó Ù· Û˘ÌÙÒÌ·Ù· ÂÌ-
Ê·Ó›˙ÔÓÙ·È ÓˆÚ›˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, Ë ˘ÔÏ·Û›· ηÈ
ÙˆÓ ‰‡Ô Ó¢ÌfiÓˆÓ Â›Ó·È ¤Ó· ÛÙ·ıÂÚfi ‡ÚËÌ·. ∏
·ÌÊÔÙÂÚfiÏ¢ÚË Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· Â›Ó·È ÛË-
Ì·ÓÙÈο ·˘ÍË̤ÓË Û ·ÛıÂÓ›˜ Ì ‰ÂÍÈ¿ ™¢∫. √È
Ó‡ÌÔÓ˜ ¤¯Ô˘Ó ÂÏ·Ùو̤ÓÔ ·ÚÈıÌfi ‚ÚfiÁ¯ˆÓ, ÏÈ-
ÁfiÙÂÚ· ‚ÚÔÁ¯ÈfiÏÈ· (Ù˘Èο 6-8 ÁÂÓ¤˜ ‚ÚfiÁ¯ˆÓ,
¤Ó·ÓÙÈ ÙˆÓ 15 ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÓÂÔÁ¤ÓÓËÙˆÓ ‚ÚÂ-
ÊÒÓ) Î·È ÂÏ·Ùو̤ÓË Î˘„ÂÏȉÈ΋ ÂÈÊ¿ÓÂÈ· (37). ∏
·ÁÁ›ˆÛË ÙˆÓ Ó¢ÌfiÓˆÓ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·‡-
ÍËÛË ÙÔ˘ ¿¯Ô˘˜ ÙÔ˘ Ï›Ԣ Ì˘˚ÎÔ‡ ¯ÈÙÒÓ· ÙˆÓ ·Á-
Á›ˆÓ, Ë ÔÔ›· ÂÂÎÙ›ÓÂÙ·È ¤ˆ˜ Î·È ÙÔ Â›Â‰Ô ÙˆÓ
ÙÚȯÔÂȉÒÓ ÙˆÓ Î˘„ÂÏ›‰ˆÓ. √ ·ÚÈıÌfi˜ ÙˆÓ ·ÚÙËÚÈ-
‰›ˆÓ Â›Ó·È Î·Ù¿ ·Ó·ÏÔÁ›· ÂÏ·Ùو̤ÓÔ˜ (38). √È ™¢∫
Ô˘ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Î·Ù¿ ÙËÓ ÂÓ‰ÔÌ‹ÙÚÈ· Î·È ¿ÌÂ-
ÛË ÌÂÙ·ÁÂÓÓËÙÈ΋ ÂÚ›Ô‰Ô Û¯ÂÙ›˙ÔÓÙ·È Ì Ì›ˆÛË
Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, ÂÓÒ ·˘Ù¤˜ Ô˘ ÂÌÊ·-
Ó›˙ÔÓÙ·È ·ÚÁfiÙÂÚ· Û¿ÓÈ· Û¯ÂÙ›˙ÔÓÙ·È Ì Ó¢ÌÔÓÈ-
΋ ˘ÔÏ·Û›· (39,40). ∏ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›·
ÂÏ·ÙÙÒÓÂÙ·È ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ ·fi ÙËÓ ·˘Í·Ófi-
ÌÂÓË ›ÂÛË ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›·, Ì ·ÔÙ¤ÏÂ-
ÛÌ· ÙË ‰ÂÍÈÔ-·ÚÈÛÙÂÚ‹ ‰È·Ê˘Á‹ (41). ™Ù· ÓÂÔÁÓ¿
·˘Ù¿, Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Â›Ó·È Û¯Â‰fiÓ Î·ıÔÏÈ-
Îfi ‡ÚËÌ· Î·È Ë ·Ó·Ó¢ÛÙÈ΋ ‹ ÌÂÙ·‚ÔÏÈ΋ Ôͤˆ-
ÛË ÙËÓ ÂȉÂÈÓÒÓÂÈ. ∏ ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË Û˘Ó‹ıˆ˜
ÔÊ›ÏÂÙ·È ÛÙËÓ ÂÏ·Ùو̤ÓË Î·Ú‰È·Î‹ ·ÚÔ¯‹ Ô˘
ÚÔηÏÂ›Ù·È ·fi ÙËÓ ˘ÔÍ›· Î·È ÙËÓ ˘ÔÏ·ÛÙÈ΋
·ÚÈÛÙÂÚ‹ ÎÔÈÏ›· (42,43).
™Â ¿ÙÔÌ· Ì ™¢∫, Ë ‚ÈÔ¯ËÌÈ΋ ·ÓˆÚÈÌfiÙËÙ· ÙˆÓ
Ó¢ÌfiÓˆÓ ¤¯ÂÈ ·ÍÈÔÏÔÁËı› Û Ó‡ÌÔÓ˜ ·Ôı·-
ÓfiÓÙˆÓ ·ÙfiÌˆÓ (44), ÛÙÔ ·ÌÓÈ·Îfi ÁÚfi ηÈ, ÈÔ Úfi-
ÛÊ·Ù·, ÛÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· (45). ™ÙÔÓ
Ó¢ÌÔÓÈÎfi ÈÛÙfi ÓÂÔÁÓÒÓ Ì ™¢∫, Ë Û˘ÁΤÓÙÚˆÛË
ʈÛÊ·ÙȉԯÔÏ›Ó˘ Â›Ó·È ¯·ÌËÏfiÙÂÚË ÛÙ· ÂÚÈÛÛfi-
ÙÂÚ· ¿ÙÔÌ· (44). ™ÙÔ ·ÌÓÈ·Îfi ÁÚfi ¤¯Ô˘Ó ·Ó·ÊÂÚı›
ÌÂȈ̤ÓË ·Ó·ÏÔÁ›· ÏÂÎÈı›Ó˘/ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ ηÈ
ÂÏ·Ùو̤Ó˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ʈÛÊ·ÙȉԯÔÏ›Ó˘ ηÈ
ÚˆÙ½Ó˘ ∞ ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·
(46,47), ÂÓÒ ¿ÏÏÔÈ ÂÚ¢ÓËÙ¤˜ ·Ó·Ê¤ÚÔ˘Ó Ê˘ÛÈÔÏÔÁÈ-
Τ˜ ·Ó·ÏÔÁ›Â˜ ÏÂÎÈı›Ó˘/ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ Î·È Ê˘ÛÈÔ-
ÏÔÁÈ΋ Û˘ÁΤÓÙÚˆÛË Ù˘ ʈÛÊ·ÙȉÔÁÏ˘ÎÂÚfiÏ˘
(48). ™ÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· ÓÂÔÁÓÒÓ ÌÂ
™¢∫, ÌÈ· ÚfiÛÊ·ÙË ÌÂϤÙË ÙˆÓ Ijsselstijn Î·È Û˘Ó.
·Ó·Ê¤ÚÂÈ fiÙÈ ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ ʈÛÊ·ÙȉԯÔÏ›Ó˘
Î·È ÊˆÛÊ·ÙȉÔÁÏ˘ÎÂÚfiÏ˘, Î·È ÔÈ ·Ó·ÏÔÁ›Â˜ ÏÂÎÈı›-
Ó˘/ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ Â›Ó·È ·ÚfiÌÔȘ Ì ÂΛӘ ÓË-
›ˆÓ ›‰È·˜ ËÏÈΛ·˜ Ô˘ ·Ó‹Î·Ó ÛÙËÓ ÔÌ¿‰· ÙˆÓ Ì·Ú-
Ù‡ÚˆÓ (45) Î·È ÚÔÙ›ÓÂÈ fiÙÈ ÌÈ· ÚfiˆÚË ·Ó¿Ú-
ÎÂÈ· ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· Â›Ó·È Ì¿Ï-
ÏÔÓ ·›ı·ÓË ÛÙ· ‚Ú¤ÊË Ì ™¢∫. ÕÏÏË ÌÂϤÙË (49)
·Ó·Ê¤ÚÂÈ fiÙÈ Ù· ÓÂÔÁÓ¿ Ì ™¢∫ ¤¯Ô˘Ó ÛËÌ·ÓÙÈο ·-
ÌËÏfiÙÂÚ· ›‰· ʈÛÊ·ÙȉÔÁÏ˘ÎÂÚfiÏ˘ Î·È Úˆ-
Ù½Ó˘ ∞ ÛÙÔ ˘ÏÈÎfi ·fi ·Ó·ÚÚfiÊËÛË Ù˘ ÙÚ·¯Â›·˜.
∞˘Ù¿ Ù· ›‰· ÌÂÈÒÓÔÓÙ·È ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ
ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘, Û ۇÁÎÚÈÛË Ì ·˘Ù¿
ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÓÂÔÁÓÒÓ ›‰È·˜ ËÏÈΛ·˜ Ô˘ ·Ô-
Ù¤ÏÂÛ·Ó ÙËÓ ÔÌ¿‰· ÙˆÓ Ì·ÚÙ‡ÚˆÓ. ∞˘Ù¿ Ù· Û˘ÌÂ-
Ú¿ÛÌ·Ù· ‰ËÏÒÓÔ˘Ó ÙËÓ ‡·ÚÍË ·Ó¿ÚÎÂÈ·˜ ÙÔ˘
ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· Û ·ÛıÂÓ›˜ ÌÂ
™¢∫, Ë ÔÔ›· ı· ÌÔÚÔ‡Û ӷ ÔÊ›ÏÂÙ·È ÛÙËÓ ‡·Ú-
ÍË ÌÈÎÚfiÙÂÚ˘ ΢„ÂÏȉÈ΋˜ ÂÈÊ¿ÓÂÈ·˜ ÛÙÔ˘˜ ÂË-
Ú·Ûı¤ÓÙ˜ Ó‡ÌÔÓ˜ ‹ ÛÙË ÌÂȈ̤ÓË Û‡ÓıÂÛË ‹
ÛÙÔÓ ·˘Í·ÓfiÌÂÓÔ Î·Ù·‚ÔÏÈÛÌfi ÙÔ˘ (50).
∫¿ÔÈ· Ê¿Ú̷η ¤¯Ô˘Ó Û¯ÂÙÈÛÙ› Ì ÙËÓ ÂÌÊ¿ÓÈ-
ÛË ™¢∫, fiˆ˜ Ë ı·ÏȉÔÌ›‰Ë, Ë ÎÈÓ›ÓË Î·È Ù· ·ÓÙÈÂÈ-
ÏËÙÈο (51,52). ∂›Û˘, ™¢∫ ¤¯ÂÈ ·Ó·ÊÂÚı› Û ÓÂ-
ÔÁÓfi ‰È·‚ËÙÈ΋˜ ÌËÙ¤Ú·˜ (53). ∏ ÔÚ‹ÁËÛË ÔÚÌÔÓÒÓ
ÌÔÚ› Ó· Â›Ó·È ÛËÌ·ÓÙÈ΋. ∆· ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ‹
100 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:100-000
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·100
ÚÔ¿ÁÔ˘Ó ÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ Ó¢ÌfiÓˆÓ Î·È ÛÙ·ıÂ-
ÚÔÔÈÔ‡Ó ÙÔ Ó¢ÌÔÓÈÎfi ·ÁÁÂÈ·Îfi ÙÔ›¯ˆÌ·, ÚÔη-
ÏÒÓÙ·˜ ·‡ÍËÛË ÙÔ˘ ¿¯Ô˘˜ ÙÔ˘ ÙÔȯÒÌ·Ùfi˜ ÙÔ˘˜
ÛÙ· ˙Ò· Ì ™¢∫ Î·È ·˘Í¿ÓÔ˘Ó ÙÔ˘˜ ˘Ô‰Ô¯Â›˜ ÙˆÓ
ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉÒÓ ÛÙ· ·È‰È¿ Ì ™¢∫ (54).
¶ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË
™ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi, ÔÈ ‰È·ÊÚ·ÁÌ·ÙÔ΋-
Ϙ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ÚÔÁÂÓÓËÙÈο ηٿ ÙÔÓ ˘Â-
Ú˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ ÂÌ‚Ú‡Ô˘. ∆Ô ÔÛÔÛÙfi
ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ‰È·Ê¤ÚÂÈ ÛËÌ·ÓÙÈο ÛÙȘ
‰È¿ÊÔÚ˜ ‰ËÌÔÛȇÛÂȘ, ·ÏÏ¿ ηٿ ̤ÛÔ fiÚÔ ‚Ú›-
ÛÎÂÙ·È ÛÙÔ 50-54% (55,7,9), Ì ‡ÚÔ˜ Ô˘ Î˘Ì·›ÓÂ-
Ù·È ·fi 29-100% (56,57).∆Ô ÔÛÔÛÙfi ·˘Ùfi ·˘Í¿ÓÂ-
Ù·È ÛÙÔ 72% fiÙ·Ó Û˘Ó˘¿Ú¯Ô˘Ó ÂÈÚfiÛıÂÙ˜ ·Óˆ-
̷ϛ˜ ‹ ·ıÔÏÔÁÈÎfi˜ ηڢfiÙ˘Ô˜ (56). ŒÓ· ÛËÌ·-
ÓÙÈÎfi˜ ·ÚÈıÌfi˜ ™¢∫ ‰È·ÁÈÁÓÒÛÎÂÙ·È ÌÂÙ¿ ÙËÓ 24Ë
‚‰ÔÌ¿‰· ·ËÛ˘. ∏ ̤ÛË ËÏÈΛ· ·ËÛ˘ ÁÈ· ÙË
‰È¿ÁÓˆÛË Ù˘ ·Ï‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ Â›Ó·È Ë
25Ë Â‚‰ÔÌ¿‰· ·ËÛ˘ (55). √È ‰È·ÊÚ·ÁÌ·ÙÔ΋Ϙ
Ì ·ÌÊÔÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË ·ÚÔ˘ÛÈ¿˙Ô˘Ó Û˘-
¯ÓfiÙÂÚ· Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜, Ô˘ Ô‰ËÁÔ‡Ó Û ˘„Ë-
ÏfiÙÂÚÔ ÔÛÔÛÙfi ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ηÈ
Ùˆ¯‹ ¤Î‚·ÛË (55). ∏ ™¢∫ ‰È·ÁÈÁÓÒÛÎÂÙ·È ˘ÂÚË-
¯ÔÁÚ·ÊÈο Ì ÙËÓ ·Ó‡ÚÂÛË ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÔÚÁ¿-
ÓˆÓ ÂÓÙfi˜ Ù˘ ıˆÚ·ÎÈ΋˜ ÎÔÈÏfiÙËÙ·˜. ŒÌÌÂÛÔÈ ‰Â›-
ÎÙ˜ Â›Ó·È ÙÔ ÔÏ˘˘‰Ú¿ÌÓÈÔ, Ô ·ÓÒÌ·ÏÔ˜ ηډȷ-
Îfi˜ ¿ÍÔÓ·˜ Î·È Ë ÌÂÙ·ÙfiÈÛË ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘.
¶ÂÚÈÛÙ·ÏÙÈΤ˜ ÎÈÓ‹ÛÂȘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Î·È ÙÔ˘ ÏÂ-
ÙÔ‡ ÂÓÙ¤ÚÔ˘ ÂÓÙfi˜ ÙÔ˘ ıÒڷη ÌÔÚ› Ó· Á›ÓÔ˘Ó
·ÓÙÈÏËÙ¤˜ ˘ÂÚ˯ÔÁÚ·ÊÈο ÛÙ· ÙÂÏÈο ÛÙ¿‰È·
Ù˘ ·ËÛ˘. √È ‰È·ÊÚ·ÁÌ·ÙÔ΋Ϙ Ô˘ ÂÓÙÔ›˙Ô-
ÓÙ·È ‰ÂÍÈ¿ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ÚÔÁÂÓÓËÙÈο ÈÔ ‰‡-
ÛÎÔÏ·, ‰ÈfiÙÈ ÙÔ ‹·Ú ¤¯ÂÈ ·ÚfiÌÔÈ· ˯ÔÁ¤ÓÂÈ· ÌÂ
ÙÔ˘˜ ÂÌ‚Ú˘˚ÎÔ‡˜ Ó‡ÌÔÓ˜ Î·È ÌÔÚ› Ó· Â›Ó·È ÙÔ
ÌfiÓÔ fiÚÁ·ÓÔ Ô˘ ÚÔÛ›ÙÂÈ ÂÓÙfi˜ ÙÔ˘ ıÒڷη. ∏
ı¤ÛË Ù˘ ¯ÔÏˉfi¯Ô˘ ·ÛÙ˘ Î·È ÔÈ ¤ÌÌÂÛÔÈ ‰Â›ÎÙ˜
ÌÔÚ› Ó· Ê·ÓÔ‡Ó È‰È·›ÙÂÚ· ¯Ú‹ÛÈÌÔÈ Û ·˘Ù‹ ÙËÓ
ÂÚ›ÙˆÛË (55).
∫ÏÈÓÈ΋ ÂÈÎfiÓ·
∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ·ÚÔ˘ÛÈ¿˙ÂÙ·È
Û˘¯ÓfiÙÂÚ· ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô Ì ÛÔ‚·Ú‹ ·Ó·-
Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· (39). ™ÙÔ 5-30% ÙˆÓ ÂÚÈ-
ÙÒÛˆÓ, Ë ‰È¿ÁÓˆÛË Î·ı˘ÛÙÂÚ› ¤Ú·Ó Ù˘ ÓÂÔ-
ÁÓÈ΋˜ ÂÚÈfi‰Ô˘ (58,59) Î·È ÌÔÚ› Ó· ÂΉËψı›
·ÚÁfiÙÂÚ· Ì ÔÈΛÏË ÎÏÈÓÈ΋ ÂÈÎfiÓ· (60). ªÂÙ¿ ÙË
Á¤ÓÓËÛË, fiÙ·Ó Ë Î‹ÏË ÁÂÌ›˙ÂÈ Ì ·¤Ú·, Ë Û˘Ì›ÂÛË
ÙˆÓ Ó¢ÌfiÓˆÓ ÂȉÂÈÓÒÓÂÙ·È. ∏ ÚfiÁÓˆÛË ÂÍ·Ú-
Ù¿Ù·È ·fi ÙÔÓ ‚·ıÌfi Ù˘ Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜
(61) Î·È ÙË Û˘Ó˘¿Ú¯Ô˘Û· ÂÏ¿ÙÙˆÛË ÙˆÓ Î˘„ÂÏÈ-
‰ÈÎÒÓ Î·È ·ÁÁÂÈ·ÎÒÓ ÂÈÊ·ÓÂÈÒÓ. ªÔÚ› Ó· Û˘Ó˘-
¿Ú¯ÂÈ ÛÔ‚·Ú‹ ˘ÂÚηӛ· Î·È ˘ÔÍ·ÈÌ›·, Ì·˙› ÌÂ
‚·ÚÈ¿ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË, Ì ·ÔÙ¤ÏÂÛÌ· ‰Â-
ÍÈÔ-·ÚÈÛÙÂÚ‹ ‰È·Ê˘Á‹ ÛÙÔ Â›Â‰Ô ÙˆÓ ÎfiÏˆÓ Î·È
ÙÔ˘ ·ÚÙËÚÈ·ÎÔ‡ fiÚÔ˘. ∆· Û˘¯ÓfiÙÂÚ· Û˘ÌÙÒÌ·-
Ù· ·ÊÔÚÔ‡Ó ÙÔ ·Ó·Ó¢ÛÙÈÎfi (43%), ÙÔ Á·ÛÙÚÂÓÙÂ-
ÚÈÎfi (33%), ÙÔ ·Ó·Ó¢ÛÙÈÎfi Î·È Á·ÛÙÚÂÓÙÂÚÈÎfi
Û‡ÛÙËÌ· (13%), ÂÓÒ ·Û˘Ìو̷ÙÈΤ˜ ÂÚÈÙÒÛÂȘ
·Ó·Ê¤ÚÔÓÙ·È Û ÔÛÔÛÙfi 11%. °È· Ù· ÓÂÔÁÓ¿ ÛÙ·
ÔÔ›· ‰ÂÓ Á›ÓÂÙ·È Ë ‰È¿ÁÓˆÛË ÂÓ‰ÔÌ‹ÙÚÈ·, Ë ¤Ó·Ú-
ÍË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Û˘Ì‚·›ÓÂÈ Û˘Ó‹ıˆ˜ Ì ·Ó·-
Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· ̤۷ ÛÙȘ ÚÒÙ˜ ÒÚ˜ ‹
Ë̤Ú˜ ˙ˆ‹˜. ∆· ÓÂÔÁÓ¿ Ì ÙËÓ ÈÔ ‚·ÚÈ¿ Û˘ÌÙˆ-
Ì·ÙÔÏÔÁ›· ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙËÓ ·›ıÔ˘Û· ÙÔÎÂÙÔ‡ ÌÂ
‰˘Û¯¤ÚÂÈ· ÛÙËÓ ·Ó¿ÓË„Ë Î·È Ì ˘„ËÏ¿ ÔÛÔÛÙ¿
Ó¢ÌÔıÒڷη. √È ·Ó·Ó¢ÛÙÈÎÔ› ‹¯ÔÈ Â›Ó·È ·fi-
ÓÙ˜ ÛÙËÓ ¿Û¯Ô˘Û· ÏÂ˘Ú¿, Ô ıÒڷη˜ ¤¯ÂÈ Î˘-
ÏÈÓ‰ÚÈÎfi Û¯‹Ì·, Ë ÎÔÈÏÈ¿ Â›Ó·È ÛηÊÔÂȉ‹˜ Î·È ÔÈ
ηډȷÎÔ› ÙfiÓÔÈ ÌÂÙ·ÙÔ›˙ÔÓÙ·È ‰ÂÍÈ¿. ∏ ÏÂÈÔÓfi-
ÙËÙ· ÙˆÓ ‰ÂÍÈ¿ ÂÓÙÔÈ˙fiÌÂÓˆÓ ™¢∫ ÂΉËÏÒÓÂÙ·È
ÌÂ Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi (62). ∏ Û‡-
ÁÎÏÂÈÛË ÙÔ˘ ·Ó·ÙÔÌÈÎÔ‡ ÂÏÏ›ÌÌ·ÙÔ˜ ·fi ÙÔ ‹·Ú
·ÔÙÚ¤ÂÈ ÙË ÌÂÙ¤ÂÈÙ· ÚfiÙˆÛË ÎÔ›ÏˆÓ ÔÚÁ¿-
ÓˆÓ Î·È ÙËÓ ÂÌÊ¿ÓÈÛË Û˘ÌÙˆÌ¿ÙˆÓ ·fi ÙÔ Á·-
ÛÙÚÂÓÙÂÚÈÎfi.
¢ÈÂÚ‡ÓËÛË
∏ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη Î·È ÎÔÈÏ›·˜ ·ÔÙÂÏ› ÙËÓ
ÚÒÙË ÂͤٷÛË Û fiÏ· Ù· ·È‰È¿ Ì ·ÓÂÍ‹ÁËÙ· Û˘-
ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi ‹ Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡-
ÛÙËÌ· (60,62). ¢˘ÛÎÔÏ›· ÛÙËÓ ·ÎÙÈÓÔÏÔÁÈ΋ ‰È¿ÁÓˆ-
ÛË ··ÓÙ¿ Û ÔÛÔÛÙfi 25%, Ì ÙÔÓ Ó¢ÌÔıÒڷη
Î·È ÙÔÓ ˘‰ÚÔıÒڷη ˆ˜ ÈÔ ÎÔÈÓ¤˜ Ï·Óı·Ṳ̂Ó˜ ‰È·-
ÁÓÒÛÂȘ (62). ∏ ÙÔÔı¤ÙËÛË ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎÔ‡ η-
ıÂÙ‹Ú· ÁÈ· ÙË ‰È·›ÛÙˆÛË Ù˘ ı¤Û˘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘
Î·È Ô ·ÎÙÈÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì ÛÎÈ·ÛÙÈÎfi ÙÔ˘ ·ÓÒÙÂ-
ÚÔ˘ ÂÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ‚ÔËıÔ‡Ó ÛÙË ÛˆÛÙ‹ ‰È¿-
ÁÓˆÛË Ù˘ ·ÚÈÛÙÂÚ‹˜ ™¢∫. ∏ CT ıÒڷη Â›Ó·È Ú‹ÛÈ-
ÌË ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù˘ ‰ÂÍÈ¿˜ ™¢∫, fiÙ·Ó ÚÔÛ›ÙÂÈ
ÙÔ ‹·Ú ÂÓÙfi˜ Ù˘ ıˆÚ·ÎÈ΋˜ ÎÔÈÏfiÙËÙ·˜ (62).
¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË
∏ ‚Ï¿‚Ë ÙˆÓ Ó¢ÌfiÓˆÓ Ô˘ Û˘Ì‚·›ÓÂÈ Î·Ù¿ ÙË
‰È¿ÚÎÂÈ· Ù˘ ÂÌ‚Ú˘˚΋˜ ˙ˆ‹˜ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ
ÛÙËÓ ÂÌÊ¿ÓÈÛË Û˘ÁÁÂÓÒÓ ·ÓˆÌ·ÏÈÒÓ, fiˆ˜ Û˘ÁÁÂÓ‹
·‰Âӈ̷ÙÒ‰Ë Î˘ÛÙÈ΋ ‰˘ÛÏ·Û›·, Û˘ÁÁÂÓ¤˜ ÏÔ‚Ò-
‰Â˜ ÂÌʇÛËÌ·, ‰È¿¯˘Ù˜ Ó¢ÌÔÓÈΤ˜ ·ÛÙÂȘ ηÈ,
Û·ÓÈfiÙÂÚ·, Ó¢ÌÔÓÈ΋ ·ÁÂÓÂÛ›·, ˘‰ÚÔıÒڷη,
Î·È ÙÂÏÈο ÛÙÔÓ ı¿Ó·ÙÔ ÙÔ˘ ÂÌ‚Ú‡Ô˘ ‹ ÙÔ˘ ÓÂÔÁÓÔ‡
·ÚÁfiÙÂÚ·. ∏ ‰È¿ÁÓˆÛË ÙˆÓ Û˘ÁÁÂÓÒÓ ·˘ÙÒÓ ·ÓˆÌ·-
ÏÈÒÓ ÚÔÁÂÓÓËÙÈο Â›Ó·È ‰‡ÛÎÔÏË. ªÂÙ·ÁÂÓ¤ÛÙÂÚ·,
Ë CT ıÒڷη ÌÔÚ› Ó· ı¤ÛÂÈ ÙË ‰È¿ÁÓˆÛË. ªÂÙ¿ ÙË
Á¤ÓÓËÛË, Ë ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÂÈ Â›-
Û˘ ÙÔÓ Ó¢ÌÔıÒڷη, ÙË ‰ÂÍÈÔηډ›· Î·È ÙË Ï·-
Ú˘ÁÁÔÙÚ·¯Âȷ΋ ·fiÊÚ·ÍË.
101™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË
¶·È‰È·ÙÚÈ΋ 2009;72:101-000
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·101
£Âڷ›·
∏ ‡·ÚÍË Â͈ۈ̷ÙÈ΋˜ ÌÂÌ‚Ú¿Ó˘ Ô͢ÁfiÓˆ-
Û˘ (ECMO), Ë ‰˘Ó·ÙfiÙËÙ· ÚÔÂÁ¯ÂÈÚËÙÈ΋˜ ÛÙ·-
ıÂÚÔÔ›ËÛ˘ ÙÔ˘ ÓÂÔÁÓÔ‡ Î·È Ë ÚfiÔ‰Ô˜ fiÛÔÓ ·ÊÔ-
Ú¿ ÛÙËÓ ÂÓ‰ÔÌ‹ÙÚÈÔ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Û˘ÁÁÂÓÔ‡˜
‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ÛÙËÚ›˙Ô˘Ó ÙËÓ ÚÔÛ¿ıÂÈ· ÌË
ÂÈıÂÙÈ΋˜ ·ÔηٿÛÙ·Û˘ ÙˆÓ ‚Ï·‚ÒÓ. ¶·Ï·ÈfiÙÂ-
Ú·, Ë ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ıˆÚÔ‡ÓÙ·Ó Â›ÁÔ˘Û· η-
Ù¿ÛÙ·ÛË, ÛÙËÓ ÔÔ›· ›¯Â ·fiÏ˘ÙË ¤Ó‰ÂÈÍË Ë ¿ÌÂÛË
¯ÂÈÚÔ˘ÚÁÈ΋ ·Ú¤Ì‚·ÛË, ÒÛÙ ӷ ÂÈÙ¢¯ı› ÙÔ Î·-
χÙÂÚÔ ‰˘Ó·Ùfi ·ÔÙ¤ÏÂÛÌ·. ∞˘Ù‹ Ë ÛÙÚ·ÙËÁÈ΋ ¤¯ÂÈ
·ÓÂÎÙÈÌËı›, ÏfiÁˆ Ù˘ ηٷÓfiËÛ˘ ÙÔ˘ ÚfiÏÔ˘ Ù˘
Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ
Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· Î·È ÙËÓ Â›‰Ú·ÛË Ù˘ ¯ÂÈ-
ÚÔ˘ÚÁÈ΋˜ ·Ú¤Ì‚·Û˘ ÛÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘Ú-
Á›·. ∂›Ó·È ϤÔÓ ÍÂοı·ÚÔ fiÙÈ ÙÔ Î‡ÚÈÔ Úfi‚ÏËÌ·
Ô˘ ÚÔηÏ› ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· ›-
Ó·È Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Î·È Ë ˘ÔÏ·Û›· ÙˆÓ
Ó¢ÌfiÓˆÓ Î·È fi¯È Ë Û˘Ì›ÂÛË Ô˘ ÚÔηÏÔ‡Ó Ù·
ÂÓ‰ÔÂÚÈÙÔÓ·˚ο ÛÏ¿¯Ó· Ô˘ ¤¯Ô˘Ó ÂÈÛ¤ÏıÂÈ ÛÙË
ıˆÚ·ÎÈ΋ ÎÔÈÏfiÙËÙ·.
™Â ÓÂÔÁÓ¿ Ì ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Î·È Û ·˘Ù¿
Ô‡ Ù›ıÂÙ·È ¤ÁηÈÚ· Ë ˘fiÓÔÈ· Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋-
Ï˘ ÙÔÔıÂÙÂ›Ù·È ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎfi˜ ηıÂÙ‹Ú·˜ ηÈ
Á›ÓÂÙ·È Û˘Ó¯Ҙ ·Ó·ÚÚfiÊËÛË ÙÔ˘ ÛÙÔÌ¿¯Ô˘, ÒÛÙ ӷ
ÂÏ·ÙÙˆı› Ë ÔÛfiÙËÙ· ÙÔ˘ ·¤Ú· ̤۷ ÛÙËÓ Î‹ÏË Î·È
Ó· ÌÂȈı› Ë Û˘Ì›ÂÛË ÙˆÓ Ó¢ÌfiÓˆÓ. ∆Ô ÓÂÔÁÓfi
Ú¤ÂÈ Ó· ‰È·ÛˆÏËÓÒÓÂÙ·È ·Ì¤Ûˆ˜ Î·È Ó· ÂÊ·ÚÌfi-
˙ÔÓÙ·È ıÂÙÈΤ˜ ȤÛÂȘ. √ Ì˯·ÓÈÎfi˜ ·ÂÚÈÛÌfi˜ ·Ô-
ÙÂÏ› ÙË ‚¿ÛË Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ™¢∫. ™ÙËÓ
ÚÔÛ¿ıÂÈ· ÂÏ¿ÙÙˆÛ˘ ÙÔ˘ ‚·ÚÔÙÚ·‡Ì·ÙÔ˜ Î·È ÙÔ˘
Ó¢ÌÔıÒڷη ÚÔÙÈÌ¿Ù·È Ô ‹ÈÔ˜ ·ÂÚÈÛÌfi˜
(63,65). ™Â ÂÚÈÙÒÛÂȘ ·ÓıÂÎÙÈ΋˜ ·Ó·Ó¢ÛÙÈ΋˜
·Ó¿ÚÎÂÈ·˜ Î·È ·Ú·Ì¤ÓÔ˘Û·˜ Ó¢ÌÔÓÈ΋˜ ˘¤Ú-
Ù·Û˘ ÂӉ›ÎÓ˘Ù·È Ë ÂÊ·ÚÌÔÁ‹ „›Û˘¯ÓÔ˘ ·ÂÚÈÛÌÔ‡,
ÌfiÓÔ˘ ‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÂÈÛÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ
ÙÔ˘ ·˙ÒÙÔ˘ (65). °È· Ù· ÓÂÔÁÓ¿ Ì ‚·ÚÈ¿ ·Ó·Ó¢ÛÙÈ-
΋ ·Ó¿ÚÎÂÈ·, Ì ÌÂÁ¿ÏË ˘ÔÛÙ‹ÚÈÍË ÛÙÔÓ Û˘Ì‚·-
ÙÈÎfi Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi, Ô˘ ÏËÚÔ‡Ó Ù· ÎÚÈÙ‹ÚÈ· ÁÈ·
ECMO, Ô ‚·ıÌfi˜ ÂÈÙ˘¯›·˜ ÙÔ˘ „›Û˘¯ÓÔ˘ ·ÂÚÈÛÌÔ‡
ÚÈÓ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ECMO ÊÙ¿ÓÂÈ ÙÔ 33% (67).
∆Ô ÂÈÛÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘ ¤¯ÂÈ ¯ÚËÛÈ-
ÌÔÔÈËı› ›Û˘ Ì ÂÈÙ˘¯›· ÛÙË ıÂڷ›· Ù˘ ‚·-
ÚÈ¿˜ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ ÙÔ˘ ÓÂÔÁÓÔ‡ Î·È ‰È¿-
ÊÔÚÔÈ ÂÚ¢ÓËÙ¤˜ ‰ÔÎ›Ì·Û·Ó ·˘Ù‹Ó ÙË ıÂڷ›· ÛÂ
ÓÂÔÁÓ¿ Ì ÛÔ‚·Ú‹ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Ô˘ ÂÈ-
ϤÎÂÈ ÙËÓ ÔÚ›· Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ (64).
∏ ¯ÔÚ‹ÁËÛË ÓÙÔ·Ì›Ó˘ ‹ ÓÙÔÌÔ˘Ù·Ì›Ó˘ Û˘-
Ó‹ıˆ˜ ‚ÂÏÙÈÒÓÂÈ ÙËÓ Î·Ú‰È·Î‹ ·ÚÔ¯‹ Î·È ÙË Û˘-
ÛÙËÌ·ÙÈ΋ ›ÂÛË, ÂÏ·ÙÙÒÓÂÈ ÙË ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË
Î·È ¤ÌÌÂÛ· ÌÂÈÒÓÂÈ ÙËÓ ·˘ÍË̤ÓË ›ÂÛË ÛÙ· Ó¢-
ÌÔÓÈο ·ÁÁ›·. ∏ ÂÊ¿·Í ¯ÔÚ‹ÁËÛË ‰ÈÙÙ·ÓıÚ·ÎÈÎÔ‡
Ó·ÙÚ›Ô˘ ÌÔÚ› Ó· Ê·Ó› ¯Ú‹ÛÈÌË. ¶¿ÓÙˆ˜, Ô Â·Ú-
΋˜ ·ÂÚÈÛÌfi˜, Ë Ô͢ÁfiÓˆÛË Î·È ÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘
·˘Í¿ÓÔ˘Ó ÙË Û˘ÛÙËÌ·ÙÈ΋ ›ÂÛË ·ÔÙÂÏÔ‡Ó ÙËÓ
·Ú΋ ıÂڷ›·. ∏ ¯ÔÚ‹ÁËÛË ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡
·Ú¿ÁÔÓÙ· ÌÔÚ›, fi¯È ÌfiÓÔ Ó· ·ÓÙÈÌÂÙˆ›ÛÂÈ ÙËÓ
·Ó¿ÚÎÂÈ¿ ÙÔ˘, ·ÏÏ¿ Î·È Ó· ‚ÂÏÙÈÒÛÂÈ ÙËÓ Â˘ÂÓ‰Ô-
ÙfiÙËÙ·, Ó· ÂÏ·ÙÙÒÛÂÈ ÙȘ Ó¢ÌÔÓÈΤ˜ ·ÁÁÂȷΤ˜
·ÓÙÈÛÙ¿ÛÂȘ Î·È Ó· ‚ÂÏÙÈÒÛÂÈ ÙË ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜
ÛÙÔÓ Ó‡ÌÔÓ· (68,69).
∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ™¢∫ ‰ÂÓ Â›Ó·È ¿ÌÔÈÚË ÂÈ-
ÏÔÎÒÓ. ¶Ó¢ÌÔıÒڷη˜ ÛÙËÓ ·ÓÙ›ıÂÙË ÏÂ˘Ú¿
ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙ› Ï›Á· ÏÂÙ¿ ÌÂÙ¿ ÙËÓ ÂÊ·ÚÌÔ-
Á‹ ·ÂÚÈÛÌÔ‡ Ì ˘„ËϤ˜ ȤÛÂȘ. ∆· ÓÂÔÁÓ¿ Ì ˘Ô-
Ï·ÛÙÈÎÔ‡˜ Ó‡ÌÔÓ˜ Â›Ó·È ÈÔ ÂÈÚÚ‹ ÛÙËÓ
·Ó¿Ù˘ÍË Ó¢ÌÔıÒڷη. ÕÌÂÛË ·Ó Î·È Û¿ÓÈ· ÌÂ-
ÙÂÁ¯ÂÈÚËÙÈ΋ ÂÈÏÔ΋ ·ÔÙÂÏ› Ô ¯˘ÏÔıÒڷη˜,
Èı·ÓÒ˜ ÔÊÂÈÏfiÌÂÓÔ˜ Û Ì˯·ÓÈÎfi ÙÚ·˘Ì·ÙÈÛÌfi
ÙˆÓ ÏÂÌÊ·ÁÁ›ˆÓ Á‡Úˆ ·fi ÙÔ ‰È¿ÊÚ·ÁÌ· (70,71).
¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜
∏ ÚfiÁÓˆÛË ÂÍ·ÚÙ¿Ù·È ·fi Ù· ÚÔÁÂÓÓËÙÈο ¢-
Ú‹Ì·Ù·, Ù· ÔÔ›· ÔÚÈÔıÂÙÔ‡Ó ÙË ÛÔ‚·ÚfiÙËÙ· Ù˘
™¢∫ Î·È ·ÔÙÂÏÔ‡Ó ÙË ‚¿ÛË Ù˘ ÂÓË̤ڈÛ˘ ηÈ
ÚÔÁÂÓÓËÙÈ΋˜ ηıÔ‰‹ÁËÛ˘ ÙˆÓ ÁÔÓ¤ˆÓ. ∏ ¤Î‚·ÛË
ÂÍ·ÚÙ¿Ù·È ·fi ·Ú¿ÁÔÓÙ˜ fiˆ˜ Ë ·ÚÔ˘Û›· Û˘ÓÔ-
‰ÒÓ ·ÓˆÌ·ÏÈÒÓ, ÙÔ ÛÙ¿‰ÈÔ Î‡ËÛ˘ ÛÙÔ ÔÔ›Ô Û˘Ó¤-
‚Ë Ë ÚfiÙˆÛË ÙˆÓ ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÔÚÁ¿ÓˆÓ ÂÓÙfi˜
ÙÔ˘ ıÒڷη, Ô fiÁÎÔ˜ ÙˆÓ ÛÏ¿Á¯ÓˆÓ Ô˘ ÚÔÛÙ›-
ÙÔ˘Ó ÂÓÙfi˜ ÙÔ˘ ıÒڷη Î·È Ô ‚·ıÌfi˜ Ù˘ Ó¢ÌÔ-
ÓÈ΋˜ ˘ÔÏ·Û›·˜ Î·È ÙˆÓ Î·Ú‰È·ÁÁÂÈ·ÎÒÓ ·ÏÏ·ÁÒÓ
Ô˘ ¤¯Ô˘Ó ÂÈÛ˘Ì‚Â› (¶›Ó·Î·˜ 3).
√È ¯ÚˆÌÔۈ̷ÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ηıÒ˜ Î·È ÔÈ
ÛÔ‚·Ú¤˜ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ηډȷÁÁÂÈ·ÎÔ‡, ·›˙Ô˘Ó
ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ¤Î‚·ÛË. ¶Ùˆ¯Ô› ÚÔÁÓˆÛÙÈ-
ÎÔ› ‰Â›ÎÙ˜ ıˆÚÔ‡ÓÙ·È: Ë ‰È¿ÁÓˆÛË ÚÈÓ ·fi ÙËÓ
25Ë Â‚‰ÔÌ¿‰· ·ËÛ˘, Ë ·ÚÔ˘Û›· ÙÔ˘ ÛÙÔÌ¿¯Ô˘
ÂÓÙfi˜ ÙÔ˘ ıÒڷη (Ë ÂÈ‚›ˆÛË Â›Ó·È ˘„ËÏfiÙÂÚË
·fi 90% Â¿Ó ÙÔ ÛÙÔÌ¿¯È ‚Ú›ÛÎÂÙ·È Î¿Ùˆ ·fi ÙÔ
‰È¿ÊÚ·ÁÌ·) (72), ÙÔ ÔÏ˘˘‰Ú¿ÌÓÈÔ (ÔÊÂÈÏfiÌÂÓÔ
ÛÙËÓ ›ÂÛË ÙÔ˘ ÔÈÛÔÊ¿ÁÔ˘ ·fi ÙÔÓ ÌÂÁ¿ÏÔ ÂÓ‰Ô-
ıˆÚ·ÎÈÎfi fiÁÎÔ), Ë ÚfiÙˆÛË ÙÔ˘ ‹·ÙÔ˜ (fiÙ·Ó ÙÔ
‹·Ú ‰ÂÓ Û˘ÁηٷϤÁÂÙ·È ÛÙ· fiÚÁ·Ó· Ô˘ ÚÔÛ›-
ÙÔ˘Ó Ë ÚfiÁÓˆÛË Â›Ó·È Î·Ï‡ÙÂÚË, ·ÏÏ¿, ηıÒ˜
ÛÙÔ 75% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ¤Ó· ÌÈÎÚfi ̤ÚÔ˜ ÙÔ˘
‹·ÙÔ˜ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙËÓ Î‹ÏË, Ë ·Í›· ÙÔ˘ ·-
Ú¿ÁÔÓÙ· ·˘ÙÔ‡ ıˆÚÂ›Ù·È ÌÈÎÚ‹) (73), Ô ‡‰Úˆ·˜
(Û¯ÂÙ›˙ÂÙ·È Ì و¯‹ ÚfiÁÓˆÛË) Î·È Ô ÏfiÁÔ˜ Ó‡-
ÌÔÓ· ÚÔ˜ ÎÂÊ·Ï‹ (lung to head ratio, LHR) ÌÈÎÚfi-
ÙÂÚÔ˜ ·fi 1. √ ÏfiÁÔ˜ LRH ÚÔÛ‰ÈÔÚ›˙ÂÙ·È Î·Ù¿ ÙÔ
˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÙÔ˘ ÂÌ‚Ú‡Ô˘ Î·È ÈÛÔ‡Ù·È Ì ÙÔÓ
ÏfiÁÔ Ù˘ ÂÈÊ¿ÓÂÈ·˜ ÙÔ˘ Ó‡ÌÔÓ· Ù˘ Ê˘ÛÈÔÏÔÁÈ-
΋˜ ÏÂ˘Ú¿˜ ÙÔ˘ ÛÒÌ·ÙÔ˜ ÚÔ˜ ÙËÓ ÂÚÈʤÚÂÈ·
Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘ ÂÌ‚Ú‡Ô˘ (74). √ LHR ÂÌÊ·Ó›˙Â-
Ù·È Ó· Â›Ó·È Ô Î·Ï‡ÙÂÚÔ˜ ÚÔÁÓˆÛÙÈÎfi˜ ‰Â›ÎÙ˘.
102 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:102-000
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·102
LHR <1 Û¯ÂÙ›˙ÂÙ·È Ì ıÓËÙfiÙËÙ· 100%, ÂÓÒ LHR>1,4 Û¯ÂÙ›˙ÂÙ·È Ì ÂÈ‚›ˆÛË 100% (75,76).
∆Ô ÚÔÁÂÓÓËÙÈÎfi ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜ÌÔÚ› Ó· ·Ó·ÁÓˆÚ›ÛÂÈ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ Ù˘ ηÚ-‰È¿˜. ¶·Ú¿ÁÔÓÙ˜ Ùˆ¯‹˜ ¤Î‚·Û˘ ıˆÚÔ‡ÓÙ·È Ë˘ÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ (77) Î·È Ô ·˘ÍË-̤ÓÔ˜ fiÁÎÔ˜ ·›Ì·ÙÔ˜ ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›· (78).
√ ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ÂÌ‚Ú‡Ô˘ Ì ̷-ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÏÂÔÓÂÎÙ›. ¢ÂÓ ÂÚÈÔÚ›˙ÂÙ·È·fi ÙÔ ¿¯Ô˜ ÙˆÓ ÎÔÈÏÈ·ÎÒÓ ÙÔȯˆÌ¿ÙˆÓ Ù˘ ÌËÙ¤-Ú·˜ ‹ ÙÔ ÔÏÈÁÔ¸‰Ú¿ÌÓÈÔ Ì ηχÙÂÚË ·ÂÈÎfiÓÈÛËÙˆÓ Ì·Ï·ÎÒÓ ÈÛÙÒÓ, ÌÔÚ› Ó· ÂȂ‚·ÈÒÛÂÈ ÙËÓ·ÚÔ˘Û›· Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ (79) Î·È Â›Ó·È ·ÔÙÂ-ÏÂÛÌ·ÙÈÎfi˜ ÛÙË ‰È¿ÁÓˆÛË ‰ÂÍÈ¿˜ ™¢∫ Ì ÚfiÙˆÛËÙÔ˘ ‹·ÙÔ˜.
∞ÚÎÂÙÔ› ÌÂÙ·ÁÂÓÓËÙÈÎÔ› ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔ-ÓÙ˜ ¤¯Ô˘Ó ÂÚ¢ÓËı›, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤ÓÔ˘ ηÈÙÔ˘ Ùˆ¯Ô‡ ·ÂÚÈÛÌÔ‡ ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙËÓ ·ÎÙÈÓÔ-ÁÚ·Ê›· ıÒڷη (80).
¡ÔÛËÚfiÙËÙ·
∂ÎÙÈÌ¿Ù·È fiÙÈ ÏÈÁfiÙÂÚÔ ·fi ÙÔ 1/3 ÙˆÓ ÂÌ‚Ú‡ˆÓÌ ™¢∫ Ô˘ ‰ÈÂÁÓÒÛıË ÚÔÁÂÓÓËÙÈο ı· ÂÈ‚ÈÒÛÂȯˆÚ›˜ ·˘ÍË̤ÓË ÓÔÛËÚfiÙËÙ·, Ë ÔÔ›· Û ÌÂÚÈΤ˜ÂÚÈÙÒÛÂȘ ÔÊ›ÏÂÙ·È Û ·Ó·ÙÔÌÈο ·›ÙÈ· Î·È Â›Ó·È·Ó·fiÊ¢ÎÙË, ÂÓÒ Û ¿ÏϘ ·ÔÙÂÏ› Û˘Ó¤ÂÈ· ÙˆÓıÂڷ¢ÙÈÎÒÓ ¯ÂÈÚÈÛÌÒÓ Î·È Â›Ó·È ‰˘Ó·Ùfi Ó· ÂÚÈÔ-ÚÈÛÙ› ÛÙÔ Ì¤ÏÏÔÓ.
∞Ó·Ó¢ÛÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·
∏ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· ·ÔÙÂÏ› ÙËÓ ÈÔÛËÌ·ÓÙÈ΋ ·ÈÙ›· ÓÔÛËÚfiÙËÙ·˜.
™ÙȘ ·ÒÙÂÚ˜ ÂÈÏÔΤ˜ Ù˘ ™¢∫ ÂÚÈÏ·Ì‚¿-
ÓÔÓÙ·È Ë ÂÚÈÔÚÈÛÙÈ΋ ÓfiÛÔ˜ ÙÔ˘ Ó‡ÌÔÓ·, Ô˘
·ÓÙ·Ó·ÎÏ¿ ÙËÓ Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·, fiˆ˜ Ê·›-
ÓÂÙ·È ·fi ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ˘ÔÏÂÈfiÌÂÓË ¯ˆÚËÙÈÎfi-
ÙËÙ·, Î·È Ë ·˘Í·ÓfiÌÂÓË Â›ÙˆÛË Ù˘ ‚ÚÔÁ¯È΋˜
˘ÂÚ·ÓÙȉڷÛÙÈÎfiÙËÙ·˜. ∆Ô 1/3 ÙˆÓ ·ÛıÂÓÒÓ ÌÂ
Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ¤¯ÂÈ ÎÏÈÓÈο Î·È ·ÎÙÈÓÔ-
ÏÔÁÈο Â˘Ú‹Ì·Ù· ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ‰˘ÛÏ·Û›·˜,
Ô˘ Â›Ó·È Û˘¯ÓfiÙÂÚ· Û ÓÂÔÁÓ¿ Ô˘ ·Ú¤ÌÂÈÓ·Ó ÁÈ·
ÌÂÁ·Ï‡ÙÂÚÔ ‰È¿ÛÙËÌ· Û Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi Î·È ÌÂ
¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ (81). ªÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋
·ÔηٿÛÙ·ÛË, ·Ú·ÙËÚÂ›Ù·È ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË
Ù˘ ·Ó¿Ù˘Í˘ Î·È Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ Ó¢ÌfiÓˆÓ,
Ô˘ Â›Ó·È È‰È·›ÙÂÚ· ÂÌÊ·Ó‹˜ ÌÂÙ¿ ÙÔ˘˜ ÚÒÙÔ˘˜ 6
Ì‹Ó˜ ˙ˆ‹˜ (82). ∆· ˘„ËÏ¿ ÔÛÔÛÙ¿ ‰˘ÛÏÂÈÙÔ˘Ú-
Á›·˜ ÙˆÓ Ó¢ÌfiÓˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ¿ÌÂÛ˘
ÌÂÙ·ÁÂÓÓËÙÈ΋˜ ÂÚÈfi‰Ô˘ ·ÎÔÏÔ˘ıÔ‡ÓÙ·È ·fi η-
ÓÔÓÈ΋ ‹ ۯ‰fiÓ Î·ÓÔÓÈ΋ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›·
ÛÙË ÌÂÙ¤ÂÈÙ· ·È‰È΋ Î·È ÂÊË‚È΋ ˙ˆ‹ (82,83). √È
‰Â›ÎÙ˜ Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ‚ÂÏÙÈÒÓÔÓÙ·È
ÛËÌ·ÓÙÈο Ì ÙËÓ ·‡ÍËÛË Ù˘ ËÏÈΛ·˜, ÙÔ˘ ‡„Ô˘˜
Î·È ÙÔ˘ ‚¿ÚÔ˘˜ ÙˆÓ ·ÛıÂÓÒÓ. ∏ ÌÂȈ̤ÓË ÚfiÛÏË-
„Ë ‚¿ÚÔ˘˜ Â›Ó·È Û˘¯Ó¿ ÙÔ Î‡ÚÈÔ Úfi‚ÏËÌ· Û ÓÂÔ-
ÁÓ¿ ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, Èı·ÓÒ˜ ÂÂȉ‹
ÙÔ ·˘Í·ÓfiÌÂÓÔ ¤ÚÁÔ ·Ó·ÓÔ‹˜ ··ÈÙ› ˘„ËÏ‹ ηٷ-
ӿψÛË ÂÓ¤ÚÁÂÈ·˜, ÂÓÒ ÂÚÈÔÚ›˙ÂÈ ÙËÓ ÈηÓfiÙËÙ·
ÙÔ˘ ·ÛıÂÓÔ‡˜ Ó· ‰È·ÙÚ·Ê› ·ÚÎÒ˜. ∞ÓÙ›ıÂÙ·, Ë
‚ÂÏÙ›ˆÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÂÈÙÚ¤ÂÈ
ηχÙÂÚË ·Ó¿Ù˘ÍË (82). ™˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ ÙÔ˘
·Ó·Ó¢ÛÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 24%
ÙˆÓ ·ÛıÂÓÒÓ Ì ‹È· ¤ˆ˜ ÌÂÙÚ›Ô˘ ‚·ıÌÔ‡ Ó¢ÌÔ-
ÓÈ΋ ˘ÔÏ·Û›· (84).
∞ÎÔ‹
∂ËÚ·ÛÌfi˜ Ù˘ ·ÈÛıËÙËÚȷ΋˜ ·ÎÔ‹˜ ¤¯ÂÈ Â-
ÚÈÁÚ·Ê› Û ÓÂÔÁÓ¿ Ì ™¢∫ Î·È ·ÚÔ˘ÛÈ¿˙ÂÈ ¤Ó·
¢ڇ Ê¿ÛÌ· ›وÛ˘. ¢˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘ ·ÎÔ‹˜
·Ó·Ê¤ÚıËΠ۠ÓÂÔÁÓ¿, ›Ù ıÂڷ‡ÙËÎ·Ó ÌÂ
ECMO ›Ù fi¯È (85). ∏ ·Ú·ÙÂٷ̤ÓË ¯Ú‹ÛË ÙÔ˘
·ÓÎÔ˘Ú·Ó›Ô˘ (86) Î·È ‰ÈÔ˘ÚËÙÈÎÒÓ Û ÓÂÔÁÓ¿ ÌÂ
¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ÓfiÛÔ (85) ¤¯ÂÈ ·Ó·ÊÂÚı› ˆ˜
Èı·Ófi˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜. ø˜ ·Ú¿ÁÔÓÙ˜
ÎÈÓ‰‡ÓÔ˘ ıˆÚÔ‡ÓÙ·È ÔÈ ·Ú·Î¿Ùˆ: Ë ‰È¿ÚÎÂÈ· ıÂ-
ڷ›·˜ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, Û˘ÌÂÚÈ-
Ï·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ECMO, Î·È Ë ‰È¿ÚÎÂÈ· ¯Ô-
Ú‹ÁËÛ˘ ˆÙÔÙÔÍÈÎÒÓ Ê·Ú̿ΈÓ, fiˆ˜ Â›Ó·È Ù· ‰È-
Ô˘ÚËÙÈο Ù˘ ·Á·Ï˘ Î·È ÙÔ ·ÓÎÔ˘Ú¿ÓÈÔ, Ô˘
ÂËÚ¿˙ÂÈ ÙË ÓÂÊÚÈ΋ ÏÂÈÙÔ˘ÚÁ›· (87). √È Ù·ÎÙÈÎÔ›
¤ÏÂÁ¯ÔÈ Ù˘ ·ÎÔ‹˜ ˆ˜ ÙËÓ ËÏÈΛ· ÙˆÓ 3 ÂÙÒÓ ıˆ-
ÚÔ‡ÓÙ·È ··Ú·›ÙËÙÔÈ. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Ù˘ ÌÂÈ-
ˆÌ¤Ó˘ ·ÎÔ‹˜ ·ÔÙÚ¤ÂÈ ÙËÓ Î·ı˘ÛÙ¤ÚËÛË ¤Ó·Ú-
͢ Ù˘ ÔÌÈÏ›·˜ Î·È ÙËÓ ÂÌÊ¿ÓÈÛË ‰È·Ù·Ú·¯ÒÓ ÂÈ-
ÎÔÈÓˆÓ›·˜.
103™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË
¶·È‰È·ÙÚÈ΋ 2009;72:103-000
¶›Ó·Î·˜ 3. ¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÛÙË ™¢∫
ñ ¶ÚÔÁÂÓÓËÙÈ΋ ÂÎÙ›ÌËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙÔ˘ Ó‡ÌÔÓ·- √ ÏfiÁÔ˜ LRH- √ ÏfiÁÔ˜ ‰È·Ì¤ÙÚÔ˘ ÙÔ˘ Ó‡ÌÔÓ· ÚÔ˜ ÙËÓ ÂÚÈʤÚÂÈ·
ÙÔ˘ ıÒڷη- √ fiÁÎÔ˜ Ó‡ÌÔÓ· ˘ÔÏÔÁÈ˙fiÌÂÓÔ˜ Ì ˘ÂÚ˯ÔÁÚ¿ÊË-
Ì· Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ñ ¶ÚÔÁÂÓÓËÙÈ΋ ÂÎÙ›ÌËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ-
΋Ï˘- ∂Ó‰ÔıˆÚ·ÎÈ΋ ÂÓÙfiÈÛË ÙÔ˘ ÛÙÔÌ¿¯Ô˘ ÛÙËÓ ·ÚÈÛÙÂÚ‹
™¢∫- ¶ÚfiÙˆÛË ÙÔ˘ ‹·ÙÔ˜- ¶ÚfiÙˆÛË ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ- ¶ÚÒÈÌË ‰È¿ÁÓˆÛË Ù˘ ™¢∫ (<25Ë Â‚‰ÔÌ¿‰· ·ËÛ˘)- ªÂÙ·ÙfiÈÛË ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘- ¢È¿ÌÂÙÚÔ˜ Ù˘ ÎÔÈÏÈ¿˜
ñ ™˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ñ ÃÚˆÌÔۈ̷ÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ñ ¶ÔÏ˘˘‰Ú¿ÌÓÈÔñ À‰ÚÔıÒڷη˜ñ ÀÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ηډ›·˜ñ ¶Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·103
¶ÚÔ‚Ï‹Ì·Ù· ÔÚıÔ‰Èο
√È ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ÛÎÂÏÂÙÔ‡, fiˆ˜ Â›Ó·È Ë ·Û˘Ì-
ÌÂÙÚ›·, ÔÈ ·Ú·ÌÔÚÊÒÛÂȘ ÙÔ˘ ıÒڷη Î·È Ë ÛÎÔ-
Ï›ˆÛË (88,89), Â›Ó·È Ù· ÈÔ Û˘ÓËıÈṲ̂ӷ ÔÚıÔ‰Èο
ÚÔ‚Ï‹Ì·Ù· Î·È ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Û˘¯ÓfiÙÂÚ· Û ÌÂ-
Á¿Ï˜ ™¢∫ Î·È Â›Ó·È Èı·ÓfiÓ Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ·ÚÁfi-
ÙÂÚ· Ì ÙËÓ ·Ó¿Ù˘ÍË. Ÿˆ˜ ·Ó·Ê¤ÚÂÙ·È ·fi ÙÔÓ
Lund, Ë ÛÎÔÏ›ˆÛË Î·È ÔÈ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ıÒڷη
ÌÔÚ› Ó· Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ·‡ÍËÛË ÙˆÓ ÂÓ‰Ôıˆ-
Ú·ÎÈÎÒÓ Î·È ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ È¤ÛÂˆÓ Î·Ù¿ ÙË ¯ÂÈ-
ÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË. √È ÂÚÈÛÛfiÙÂÚ˜ ·fi ÙȘ
·Ú·ÌÔÚÊÒÛÂȘ ·˘Ù¤˜ Â›Ó·È ‹È˜ Î·È ‰ÂÓ ¯Ú‹˙Ô˘Ó
¯ÂÈÚÔ˘ÚÁÈ΋˜ ·ÔηٿÛÙ·Û˘ (90).
£Ú¤„Ë Î·È ·Ó¿Ù˘ÍË
√È ‰È·Ù·Ú·¯¤˜ ·Ó¿Ù˘Í˘ ÔÊ›ÏÔÓÙ·È ÛÙȘ ·˘ÍË̤-
Ó˜ ÂÓÂÚÁÂȷΤ˜ ··ÈÙ‹ÛÂȘ (·˘ÍË̤ÓÔ ¤ÚÁÔ ·Ó·ÓÔ‹˜
Û ¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ÓfiÛÔ) Î·È ÛÙË Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ-
΋ ·ÏÈÓ‰ÚfiÌËÛË (°√¶), Ô˘ ·Ó·Ê¤ÚÂÙ·È ÛÙÔ 89%
ÙˆÓ ·ÛıÂÓÒÓ (91,88,92,90). ¶·Ú·ÙËÚÔ‡ÓÙ·È Û˘¯ÓfiÙÂ-
Ú· Û ·ÛıÂÓ›˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (Ô˘ ·ÚÔ˘Û›·Û·Ó
Û˘ÌÙÒÌ·Ù· ÂÓÙfi˜ ÙˆÓ ÚÒÙˆÓ 6 ÚÒÓ ·fi ÙË Á¤ÓÓË-
ÛË) Ì ‰È¿Ù·ÛË ÔÈÛÔÊ¿ÁÔ˘ Î·È Û ·ÛıÂÓ›˜ ÛÙÔ˘˜ ÔÔ›-
Ô˘˜ ··ÈÙ‹ıËΠÂÊ·ÚÌÔÁ‹ ECMO (93,88). ∏ ‰È¿Ù·ÛË
ÙÔ˘ ÔÈÛÔÊ¿ÁÔ˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ÂÁÁÂÓ‹ ‰˘ÛÎÈÓËÛ›·
ÙÔ˘ ÔÚÁ¿ÓÔ˘, Ô˘ ·ÓÙ·Ó·ÎÏ¿ ÙËÓ Î·ı˘ÛÙÂÚË̤ÓË Î¤-
ÓˆÛË ÙÔ˘ Á·ÛÙÚÈÎÔ‡ ÂÚȯÔ̤ÓÔ˘ (100). À¿Ú¯Ô˘Ó
Ì·ÎÚÔÚfiıÂÛÌ· ÚÔ‚Ï‹Ì·Ù· ·fi ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi,
fiˆ˜ ¯ÚfiÓÈ· ÔÈÛÔÊ·Á›Ùȉ· (Û¯ÂÙÈ˙fiÌÂÓË Ì °√¶) ηÈ
ηı˘ÛÙÂÚË̤ÓË ÂÓÙÂÚÈ΋ ΤӈÛË. ∏ ˘„ËÏ‹ ›وÛË
Ù˘ ÔÈÛÔÊ·Á›Ùȉ·˜ Î·È ÙÔ˘ ÔÈÛÔÊ¿ÁÔ˘ Barrett ÛÙÔ˘˜
ÂÓ‹ÏÈÎÔ˘˜ ·ÛıÂÓ›˜ Ì ÈÛÙÔÚÈÎfi Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·Á-
Ì·ÙÔ΋Ï˘ ηıÈÛÙ¿ ·Ó·Áη›· ÙËÓ ÂÊ·ÚÌÔÁ‹ ÚÔÁÚ¿Ì-
Ì·ÙÔ˜ ÂÚ·ÈÙ¤Úˆ ·Ú·ÎÔÏÔ‡ıËÛ‹˜ ÙÔ˘˜ ηٿ ÙË ‰È¿Ú-
ÎÂÈ· Ù˘ ÂÓ‹ÏÈ΢ ˙ˆ‹˜ ÙÔ˘˜ (90). ¶ÚÔÙ›ÓÔÓÙ·È ‰È·-
ÙÚÔÊ‹ ÏÔ‡ÛÈ· Û ıÂÚÌ›‰Â˜ Î·È Ê·Ú̷΢ÙÈ΋ ·ÓÙÈÌÂ-
ÙÒÈÛË Ù˘ Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘ ÌÂ
∏2-·ÔÎÏÂÈÛÙ¤˜ ˆ˜ ıÂڷ¢ÙÈο ̤ÙÚ· (61).
¡Â˘ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË
∆· ÓÂÔÁÓ¿ Ì ™¢∫ ‚Ú›ÛÎÔÓÙ·È Û ·˘ÍË̤ÓÔ Î›Ó-
‰˘ÓÔ ˘ÔÍÈ΋˜-ÈÛ¯·ÈÌÈ΋˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜. ∆· ÓÂ-
ÔÁÓ¿ Ì ÛÔ‚·Ú‹ Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· ·ÓÙÈÌÂÙˆ-
›˙Ô˘Ó Î›Ó‰˘ÓÔ ˘ÔÍ·ÈÌ›·˜, ÔͤˆÛ˘ Î·È Ùˆ¯Ô‡
·ÂÚÈÛÌÔ‡. ∂ÁÎÂÊ·ÏÈ΋ ·Ú¿Ï˘ÛË ¤¯ÂÈ ·Ú·ÙËÚËı›
ÛÙÔ 4% (89) ÛÙÔ 5% (95) Î·È ÛÙÔ 9% ÙˆÓ ·ÛıÂÓÒÓ
(96) Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜. ÕÏϘ Ó¢ÚÔ·Ó·Ù˘ÍÈ·-
Τ˜ ÂÈÏÔΤ˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û·ÛÌÔ‡˜, Ì·ıËÛÈ·-
Τ˜ ‰˘ÛÎÔϛ˜, ·ÒÏÂÈ· ·ÎÔ‹˜ Î·È Î·ı˘ÛÙ¤ÚËÛË
·fiÎÙËÛ˘ ÎÈÓËÙÈÎÒÓ ‰ÂÍÈÔÙ‹ÙˆÓ (97).
ŒÎ‚·ÛË
∂›Ó·È ÁÂÓÈο ·o‰ÂÎÙfi fiÙÈ ÔÈ ·ÛıÂÓ›˜ Ì ‰È·-
ÊÚ·ÁÌ·ÙÔ΋ÏË ˙Ô˘Ó ÌÈ· Ê˘ÛÈÔÏÔÁÈ΋ ˙ˆ‹. øÛÙfiÛÔ,Û¯ÂÙÈΤ˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›Â˜, fiˆ˜ ηı˘ÛÙ¤ÚËÛË ·Ó¿-Ù˘Í˘, Ùˆ¯‹ ·Ó¿Ù˘ÍË, Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ΋ ·ÏÈÓ-‰ÚfiÌËÛË, ·ÒÏÂÈ· ·ÎÔ‹˜, Ì˘ÔÛÎÂÏÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜Î·È ·Ó·Ó¢ÛÙÈο ÚÔ‚Ï‹Ì·Ù·, ·Ú·Ì¤ÓÔ˘Ó Û ·Ú-ÎÂÙÔ‡˜ ·fi ·˘ÙÔ‡˜ (88,61,94). ∏ ·Ô˘Û›· Û˘ÓÙÔÓÈ-Ṳ̂ÓÔ˘ Ï¿ÓÔ˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙˆÓ ·ÛıÂÓÒÓ ·˘-ÙÒÓ Ô‰ËÁ› Û ˘ÔÙ›ÌËÛË Ù˘ ÂÈÎÚ¿ÙËÛ˘ Û˘ÁÎÂ-ÎÚÈÌ¤ÓˆÓ ÂÈÏÔÎÒÓ Û ·ÛıÂÓ›˜ ÌÂÁ·Ï‡ÙÂÚ˘ ËÏÈ-Λ·˜ Ì ÈÛÙÔÚÈÎfi Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ (61).
™Â ÚfiÛÊ·ÙË ¤Ú¢ӷ ‚Ú¤ıËΠˆ˜ Ù· ÓÂÔÁÓ¿ Ù·ÔÔ›· ¯ÚÂÈ¿˙ÔÓÙ·Ó ıÂڷ›· ̤۷ ÛÙȘ ÚÒÙ˜ 6ÒÚ˜ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ıÓËÙfiÙËÙ· ÛÂÔÛÔÛÙfi 44%, ÂÓÒ ÌˉÂÓÈ΋ ıÓËÙfiÙËÙ· ·ÚÔ˘ÛÈ¿-˙Ô˘Ó ·˘Ù¿ Ô˘ ¯ÚÂÈ¿˙ÔÓÙ·Ó ıÂڷ›· ÌÂÙ¿ ÙȘ ÚÒ-Ù˜ 6 ÒÚ˜ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ (98,99). √È Û˘ÓÔ‰¤˜ ·Óˆ-̷ϛ˜ ‰ÂÓ Â›Ó·È ˘Ô¯ÚˆÙÈο ‰Â˘ÙÂÚ‡ԢÛ˜ Î·È ËÛ˘Ó‡·ÚÍ‹ ÙÔ˘˜ ÌÔÚ› Ó· ¯ÂÈÚÔÙÂÚ‡ÂÈ ÙËÓ Úfi-ÁÓˆÛË. ∫·ıÔÚÈÛÙÈ΋˜ ÛËÌ·Û›·˜ ÁÈ· ÙËÓ ¤Î‚·ÛË ÙˆÓÓÂÔÁÓÒÓ ·˘ÙÒÓ Â›Ó·È Ë Û˘Ó‡·ÚÍË ÌÂÈ˙fiÓˆÓ ·Ó·ÙÔ-ÌÈÎÒÓ ‹ ¯ÚˆÌÔۈ̷ÙÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ, Ô˘ ·˘Í¿ÓÂÈÙË ıÓËÙfiÙËÙ· ÛÙÔ 89% (36). ™ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·-Ê›·, Ù· ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘ Û ۯ¤ÛË Ì ÙËÓ ÂÓÙfiÈ-ÛË Ù˘ ™¢∫ ÔÈΛÏÏÔ˘Ó. ŸÙ·Ó Ë ‚Ï¿‚Ë ÂÓÙÔ›˙ÂÙ·ÈÛÙÔ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ Ë ÂÈ‚›ˆÛË ·Ó¤Ú¯ÂÙ·È ÛÂ78%, fiÙ·Ó ÂÓÙÔ›˙ÂÙ·È ÛÙÔ ‰ÂÍÈfi Û 21%, ÂÓÒ Û ·Ì-ÊÔÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË ·Ó¤Ú¯ÂÙ·È Û 1% (101).¶ÈÔ ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó ·‡ÍËÛË (102,103)Î·È ÔÚÈṲ̂Ó˜ ÂÏ¿ÙÙˆÛË Ù˘ ıÓËÙfiÙËÙ·˜ (103) ÛÙˉÂÍÈ¿ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÂÓÒ ¿ÏϘ ‰Â›¯ÓÔ˘Ó fiÙÈ ËÂÓÙfiÈÛË Ù˘ ·ÓˆÌ·Ï›·˜ ‰ÂÓ ÂËÚ¿˙ÂÈ ÙËÓ ¤Î‚·ÛË(55). ∆· ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘ ÁÈ· ·Ï‹ ‰È·ÊÚ·ÁÌ·ÙÔ-΋ÏË Â›Ó·È ÂÚ›Ô˘ 47% Î·È ·˘Í¿ÓÔÓÙ·È ÛÙË ‰È¿Ú-ÎÂÈ· ÙÔ˘ ¯ÚfiÓÔ˘, ·Ú·Ì¤ÓÔ˘Ó fï˜ ¯·ÌËÏ¿ ÁÈ· ÙȘÂÚÈÙÒÛÂȘ fiÔ˘ Û˘Ó˘¿Ú¯Ô˘Ó Û˘ÓÔ‰¤˜ ·ÓˆÌ·-ϛ˜ (55). ÕÏϘ ÌÂϤÙ˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ˘„ËÏfiÙÂÚ·ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘ Ô˘ Î˘Ì·›ÓÔÓÙ·È ·fi 67% (89)¤ˆ˜ 82% (104).
∏ ÚÔÛ¤ÁÁÈÛË ÙÔ˘ ÓÂÔÁÓÔ‡ Ì ™¢∫ ¤¯ÂÈ Û·ÊÒ˜·ÏÏ¿ÍÂÈ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿-ÁÓˆÛË ÂÈÙÚ¤ÂÈ ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÁÈ· È-ı·Ó¤˜ ÂÈÚfiÛıÂÙ˜ ·ÓˆÌ·Ï›Â˜, ηıÒ˜ Î·È ÙËÓ ÂÓË-̤ڈÛË ÙˆÓ ÁÔÓ¤ˆÓ ÁÈ· ÙȘ Èı·Ó¤˜ ıÂڷ¢ÙÈΤ˜ÚÔÛÂÁÁ›ÛÂȘ Î·È ÙËÓ ÚfiÁÓˆÛË. ∏ ÂÈÎÔÈÓˆÓ›· ÌÂÙÔ˘˜ ÁÔÓ›˜ Ú¤ÂÈ Ó· ·Ú¯›˙ÂÈ ·fi ÙË ÛÙÈÁÌ‹ Ù˘ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Î·È Ó· Û˘Ó¯›˙ÂÙ·È Û fiÏËÙËÓ ·È‰È΋ ËÏÈΛ·.
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89. Jaillard S, Pierrat V, Dubois A, Truffert P, Lequien P,Wurtz A, et al. Outcome at 2 years of infants withcongenital diaphragmatic hernia: a population-basedstudy. Ann Thorac Surg 2003;75:250-256.
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107™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË
¶·È‰È·ÙÚÈ΋ 2009;72:107-000
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·107
108 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:108-000
Quiz
1. H ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Ù˘ Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘:·. ¢ÂÓ Â›Ó·È ‰˘Ó·Ù‹‚. °›ÓÂÙ·È ÌÂÙ¿ ÙËÓ 24Ë Â‚‰ÔÌ¿‰· ·ËÛ˘ Ì ¯Ú‹ÛË ˘ÂÚ‹¯ˆÓÁ. °›ÓÂÙ·È ÌfiÓÔ ÛÙÔ 10% ÙˆÓ ÂÚÈÙÒÛˆӉ. √È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ‰˘ÛÎÔÏÂ‡Ô˘Ó ÙË ‰È¿ÁÓˆÛË
2. ∏ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· ÓÂÔÁÓÔ‡ ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ÔÊ›ÏÂÙ·È ÛÂ:·. ¶Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·‚. §Ô›ÌˆÍËÁ. ¶Ó¢ÌÔıÒڷη‰. ¶Ó¢ÌÔÓÈ΋ ·ÈÌÔÚÚ·Á›·
3. ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek ÂÓÙÔ›˙ÂÙ·È Û˘¯ÓfiÙÂÚ·:·. ¢ÂÍÈ¿‚. ∞ÚÈÛÙÂÚ¿Á. ∞ÌÊÔÙÂÚfiÏ¢ڷ‰. ∫ÂÓÙÚÈο
4. ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÂΉËÏÒÓÂÙ·È Û˘¯ÓfiÙÂÚ· ÌÂ Û˘ÌÙÒÌ·Ù· ·fi:·. ∆Ô Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ·‚. ∆Ô ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ·Á. ∂›Ó·È ·Û˘Ìو̷ÙÈ΋‰. ∆Ô ·Ó·Ó¢ÛÙÈÎfi Û‡ÛÙËÌ·
5. ™ÙË Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Û˘¯ÓfiÙÂÚ· Û˘Ó˘¿Ú¯Ô˘Ó ·ÓˆÌ·Ï›Â˜ ·fi:·. ∆Ô Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ·‚. ∆Ô Ô˘ÚÔÁÂÓÓËÙÈÎfi Û‡ÛÙËÌ·Á. ∆Ô Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ·‰. ∆ÔÓ ÛÎÂÏÂÙfi
√È ÛˆÛÙ¤˜ ··ÓÙ‹ÛÂȘ ÛÙË ÛÂÏ. 122
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·108
∂›‰Ú·ÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì˘ÂÏÔ‡ ÔÛÙÒÓ
ÛÙËÓ ex vivo ¤ÎÙ˘ÍË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜*
π. ¶ÂÏ·ÁÈ¿‰Ë˜, ∂. ¢ËÌËÙÚ›Ô˘, ∂. ™ÙÂȷοÎË, Ã. ¶ÂÚ‰ÈÎÔÁÈ¿ÓÓË, ∂. ÷Ù˙ˉ¿ÎË, ª. ∫·ÏÌ·ÓÙ‹
¶ÂÚ›ÏË„Ë
∂ÈÛ·ÁˆÁ‹: ∆· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ˆ˜ ̤ÚÔ˜ ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ Ì˘ÂÏÈÎÔ‡ ÌÈÎÚÔÂÚÈ‚¿ÏÏÔÓÙÔ˜, ı·ÌÔÚÔ‡Û·Ó Ó· ÚÔ¿ÁÔ˘Ó ÙË ‰È·‰Èηۛ· ¤ÎÙ˘Í˘ ·Ú¯¤ÁÔÓˆÓ/ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·›Ì·ÙÔ˜ ÔÌÊ¿ÏÈÔ˘ÏÒÚÔ˘, Ô˘ ·ÔÙÂÏ› ‡ÎÔÏ· ÚÔÛ‚¿ÛÈÌË ËÁ‹ ΢ÙÙ¿ÚˆÓ ÁÈ· ÌÂÙ·ÌfiÛ¯Â˘ÛË, ·ÏÏ¿ ··ÈÙÂ›Ù·È ex vivo
¤ÎÙ˘Í‹ ÙÔ˘˜ ÁÈ· ¯Ú‹ÛË Û ·È‰È¿ ÌÂÁ·Ï‡ÙÂÚÔ˘ ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜ Î·È Û ÂÓ‹ÏÈΘ. ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ·ÏÏËÏ›‰Ú·Û˘ ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ ·›Ì·ÙÔ˜ ÔÌÊ¿ÏÈÔ˘ ÏÒÚÔ˘ (√§) Ì ÌÂÛÂÁ-¯˘Ì·ÙÈο ·ÙÙ·Ú· (ª∫) Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (ª√) ·È‰ÈÒÓ Î·È Ë Û‡ÁÎÚÈÛË Ì ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë Â›‰Ú·ÛËÛ˘Ó‰˘·ÛÌÔ‡ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ÃÚËÛÈÌÔÔÈ‹ıËÎ·Ó Î‡ÙÙ·Ú· CD34+ Ô˘ ·ÔÌÔÓÒıËÎ·Ó ·fi ·›Ì· √§ (n=10) Î·È ª∫Ô˘ ·ÔÌÔÓÒıËÎ·Ó ·fi ª√ ·È‰ÈÒÓ Ì ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· (n=9). ∆· ·ÙÙ·Ú· Ù¤ıËηÓÛ ‰È·ÊÔÚÂÙÈΤ˜ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜, ·ÚÔ˘Û›· ‹ ÌË Û˘Ó‰˘·ÛÌÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ηȤÁÈÓ ·ÓÔÛÔÊ·ÈÓÔÙ˘ÈÎfi˜ ¯·Ú·ÎÙËÚÈÛÌfi˜, ·ÍÈÔÏfiÁËÛË Ù˘ ¤ÎÙ˘Í˘ Î·È ÂÎÙ›ÌËÛË Ù˘ ÎψÓÔÁÔÓÈÎfiÙËÙ·˜(·Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ CFU-GM Î·È BFU-E) ÙˆÓ CD34+ ΢ÙÙÙ¿ÚˆÓ.
∞ÔÙÂϤÛÌ·Ù·: ∏ ·ÚÔ˘Û›· ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· ÙˆÓ CD34+, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔ-ÓÙ˜, ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË ÌÂÁ·Ï‡ÙÂÚË ¤ÎÙ˘ÍË ÙfiÛÔ ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÙÙ¿ÚˆÓ fiÛÔ Î·ÈÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ CD34+. ∏ ·ÚÔ˘Û›· ª∫ ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ¤‰ˆÛ ÙÔ ˘„ËÏfiÙÂÚÔ ÔÛÔ-ÛÙfi ÙˆÓ ˘ÔÏËı˘ÛÌÒÓ CD34+CD38-, CD34+CD33-, ηıÒ˜ Î·È CD34 Ô˘ ıˆÚÔ‡ÓÙ·È Î·È ÔÈ Ï¤ÔÓ ·Ú-¯¤ÁÔÓÔÈ. ∂ÈϤÔÓ, Û ·˘Ù‹ ÙË Û˘Óı‹ÎË Ù· ÂÎÙ˘Á̤ӷ ·ÙÙ·Ú· ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ˘„ËÏ‹ ÎψÓÔÁÔ-ÓÈÎfiÙËÙ·, ·ÊÔ‡ ¤‰ˆÛ·Ó ÙÔÓ ÌÂÁ·Ï‡ÙÂÚÔ ·ÚÈıÌfi ·ÔÈÎÈÒÓ ÙfiÛÔ ÎÔÎÎÈÒ‰Ô˘˜ fiÛÔ Î·È ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜.
™˘ÌÂÚ¿ÛÌ·Ù·: ™˘ÌÂÚ·ÛÌ·ÙÈο, Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· ª√ ˘ÔÛÙËÚ›˙Ô˘Ó ÙËÓ ¤ÎÙ˘ÍË Î·È Û˘Ì-‚¿ÏÏÔ˘Ó ÛÙË ‰È·Ù‹ÚËÛË Ù˘ ·ˆÚfiÙËÙ·˜ ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ ÛÂ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜, ·Ú¤¯Ô-ÓÙ·˜ ÏËı˘ÛÌfi Ô˘ Ê·›ÓÂÙ·È Ó· ‰È·ı¤ÙÂÈ ¯·Ú·ÎÙËÚÈÛÙÈο Ô˘ ÙÔÓ Î·ıÈÛÙÔ‡Ó Î·Ù¿ÏÏËÏÔ ÁÈ· ¯Ú‹ÛË ÙÔ˘Û ÌÂÙ·ÌfiÛ¯Â˘ÛË.
§¤ÍÂȘ ÎÏÂȉȿ: CD34+, ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ex vivo ¤ÎÙ˘ÍË, ·›Ì· ÔÌÊ¿ÏÈÔ˘ ÏÒÚÔ˘, Ì˘ÂÏfi˜ ÔÛÙÒÓ ·È‰ÈÒÓ.
The effect of paediatric bone-marrow-derived stromal cells on the
ex-vivo expansion of cord-blood haemopoietic stem cells
I. Pelagiadis, H. Dimitriou, E. Stiakaki, C. Perdikogianni, E. Hatzidaki, M. Kalmanti
Abstract
Background: A major limitation of the use of umbilical cord blood (UCB) haematopoietic stem cells (HSC)for allogeneic transplantation is the insufficient numbers of cells available in the graft. In order to overcomethis restriction, ex-vivo expansion of HSC has been considered. Mesenchymal stromal cells (MSC)constitute an essential element of the normal bone marrow (BM) microenvironment, participating in theregulation of haematopoiesis. The aim of this study was to evaluate the interaction between UCB CD34+
cells and MSC used as a feeder layer and to compare it with the corresponding effect of different growthfactor (GF) combinations on the expansion of HSC.
Methods: CD34+ HSC from the UCB of full term deliveries (n=10) and MSC isolated and expanded fromthe BM of children were co-cultured under different conditions in the presence or absence of GFcombinations. The expansion capability was evaluated, the various populations were immuno-phenotypically characterized and HSC clonogenicity (expressed as the development-formation of CFU-GM/BFU-E colonies) was assessed after 10 days of culture.
Results: CD34+ UCB cells cultured in the presence of both MSC and either GF combination resulted in ahigher expansion of both total cell number and CD34+ cells. Those cultured on the MSC feeder layer onlyyielded a higher percentage of CD34+, CD34+CD38- and CD34+CD33- subpopulations, and enhanced CFU-GM and BFU-E colony development.
109∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞ ORIGINAL ARTICLE
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜.¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ,∆Ì‹Ì· π·ÙÚÈ΋˜¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘,∏Ú¿ÎÏÂÈÔ, ∫Ú‹ÙË
AÏÏËÏÔÁÚ·Ê›·:
ª·Ú›· ∫·ÏÌ·ÓÙ‹ [email protected]¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜,¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô∏Ú·ÎÏ›Ԣ, 71110 ∏Ú¿ÎÏÂÈÔ∫Ú‹Ù˘
Department of PaediatricHaematology-Oncology,University Hospital ofHeraklion, University of CreteMedical School, Heraklion,Crete, Greece
Correspondence:
Maria [email protected] of PaediatricHaematology-Oncology,University Hospital ofHeraklion, 71110, Heraklion,Crete, Greece
¶·È‰È·ÙÚÈ΋ 2009;72:109-000
* ∏ ÂÚÁ·Û›· ·˘Ù‹ ‚Ú·‚‡ıËΠ̠ÙÔ A’ XˆÚ¤ÌÂÈÔ Œ·ıÏÔ, ηٿ ÙÔ 46Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ∫¤Ú΢ڷ 2008
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·109
∂ÈÛ·ÁˆÁ‹
∆Ô ÔÌÊ·ÏÈÔÏ·ÎÔ˘ÓÙÈ·Îfi ·›Ì· (√§) ·Ô-
ÙÂÏ› ‡ÎÔÏ· ÚÔÛ‚¿ÛÈÌË ËÁ‹ ÁÈ· ÌÂÙ·Ìfi-
Û¯Â˘ÛË Î·È ‰È·ı¤ÙÂÈ ÛËÌ·ÓÙÈο ÏÂÔÓÂÎÙ‹Ì·-
Ù·, ‰ËÏ. ÌÂÁ·Ï‡ÙÂÚË ÂÚÈÂÎÙÈÎfiÙËÙ· Û ÚÔÁÔ-
ÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú·, ÌÈÎÚfiÙÂÚÔ ÔÛÔ-
ÛÙfi ÂÌÊ¿ÓÈÛ˘ GVHD Î·È ‰˘Ó·ÙfiÙËÙ· ÌÂÙ·Ìfi-
Û¯Â˘Û˘ Û ÌË Û˘Ì‚·Ùfi ‰¤ÎÙË (1-2 HLA ·ÓÙÈ-
ÁfiÓ·) (1-3). ∆·˘Ùfi¯ÚÔÓ·, fï˜, Ô Û¯ÂÙÈο ÌÈ-
ÎÚfi˜ ·ÚÈıÌfi˜ ΢ÙÙ¿ÚˆÓ Ô˘ ÂÚȤ¯ÔÓÙ·È ·Ó¿
ÌÔÓ¿‰· ·›Ì·ÙÔ˜ ÏÒÚÔ˘ ηıÈÛÙ¿ Ú·ÎÙÈο ·‰‡-
Ó·ÙË ÙË ¯ÔÚ‹ÁËÛ‹ ÙÔ˘ Û ·È‰È¿ Ì ۈ̷ÙÈÎfi
‚¿ÚÔ˜ ÌÂÁ·Ï‡ÙÂÚÔ ÙˆÓ 30 ÎÈÏÒÓ (4,5).
ªÂ ÛÎÔfi ÙËÓ Â˘Ú‡ÙÂÚË ¯Ú‹ÛË ·›Ì·ÙÔ˜ √§
ÛÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË, Á›ÓÔÓÙ·È ÚÔÛ¿ıÂȘ ÁÈ· ex
vivo ¤ÎÙ˘ÍË Î˘ÙÙ¿ÚˆÓ. √È ‰È·‰Èηۛ˜ ¤ÎÙ˘-
͢ ̤¯ÚÈ ÚfiÛÊ·Ù· ÂÚÈÏ¿Ì‚·Ó·Ó ÙË ¯Ú‹ÛË
‰È·ÊÔÚÂÙÈÎÒÓ Û˘Ó‰˘·ÛÌÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·Áfi-
ÓÙˆÓ, ÔÈ Ôԛ˜ fï˜ ηٷϋÁÔ˘Ó Û ۯÂÙÈο
‰È·ÊÔÚÔÔÈË̤ÓÔ˘˜ ΢ÙÙ·ÚÈÎÔ‡˜ ˘ÔÏËı˘-
ÛÌÔ‡˜ (6-8). ¶ÚfiÛÊ·Ù· ÍÂΛÓËÛÂ Ë ‰ÈÂÚ‡ÓËÛË
Ù˘ ‰˘Ó·ÙfiÙËÙ·˜ ¯Ú‹Û˘ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘Ù-
Ù¿ÚˆÓ (ª∫) ÛÙÚÒÌ·ÙÔ˜ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ
(ª√), ηıÒ˜ Î·È ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ, ÁÈ·
ÙË ‚ÂÏÙ›ˆÛË Ù˘ ex vivo ¤ÎÙ˘Í˘ (9-13).
∆· ª∫ ·ÔÙÂÏÔ‡Ó Ì¤ÚÔ˜ ÙÔ˘ ÌÈÎÚÔÂÚÈ-
‚¿ÏÏÔÓÙÔ˜ ÙÔ˘ ª√, ÙÔ ÔÔ›Ô Û˘ÌÌÂÙ¤¯ÂÈ ÛÙË
Ú‡ıÌÈÛË Ù˘ ·ÈÌÔÔ›ËÛ˘. √ ª√ ·ÔÙÂÏ› ÙËÓ
ÂÚÈÛÛfiÙÂÚÔ ÌÂÏÂÙËı›۷, ÈÔ ÚÔÛ‚¿ÛÈÌË Î·È
·Ú¿ÏÏËÏ· ÏÔ˘ÛÈfiÙÂÚË ËÁ‹ ·Ú¯¤ÁÔÓˆÓ ª∫
(14,15).
H Û˘Á¯ÔÚ‹ÁËÛË ·˘ÙfiÏÔÁˆÓ ª∫ Ì ÚÔÁÔ-
ÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· Û ·ÛıÂÓ›˜ ÌÂ
ηÚΛÓÔ ÙÔ˘ Ì·ÛÙÔ‡ Î·È ·ÈÌ·ÙÔÏÔÁÈΤ˜ ηÎÔ‹-
ıÂȘ ¤‰ÂÈÍ ٷ¯‡ÙÂÚË ·ÔηٿÛÙ·ÛË ÙˆÓ ÂÈ-
¤‰ˆÓ ÙˆÓ Ô˘‰ÂÙÂÚfiÊÈÏˆÓ Î·È ÙˆÓ ·ÈÌÔÂÙ·-
Ï›ˆÓ, ÂÓÒ ·Ú¿ÏÏËÏ· Ë Ì¤ıÔ‰Ô˜ Ê¿ÓËΠӷ ›-
Ó·È ·ÛÊ·Ï‹˜, ¯ˆÚ›˜ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ
(16,17).
™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘
›‰Ú·Û˘ ÙˆÓ ª∫ ÛÙÚÒÌ·ÙÔ˜ Ì˘ÂÏÔ‡ ÙˆÓ
ÔÛÙÒÓ ÛÙË ‰˘Ó·ÙfiÙËÙ· ¤ÎÙ˘Í˘ CD34+ ΢ÙÙ¿-
ÚˆÓ ·›Ì·ÙÔ˜ ÔÌÊ¿ÏÈÔ˘ ÏÒÚÔ˘ Î·È Ë Û‡ÁÎÚÈÛË
Ì ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë Â›‰Ú·ÛË Û˘Ó‰˘·ÛÌÒÓ ·˘-
ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ
™˘ÏÏÔÁ‹ / ‰È·¯ˆÚÈÛÌfi˜ CD34+ ΢ÙÙ¿ÚˆÓ ·fi ·›Ì·
ÔÌÊ¿ÏÈÔ˘ ÏÒÚÔ˘
∞›Ì· √§ (n=10) Û˘ÏϤ¯ıËΠÌÂÙ¿ ÙËÓ ÔÏÔÎÏ‹Úˆ-
ÛË ÙÔ˘ ÙÔÎÂÙÔ‡ (ŒÁÎÚÈÛË ∂ÈÙÚÔ‹˜ ¢ÂÔÓÙÔÏÔÁ›·˜
ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ Ì·˜). ∆· ÌÔÓÔ‡ÚËÓ· ·ÙÙ·Ú·
(ª¡¶) ‰È·¯ˆÚ›ÛÙËÎ·Ó Ì ›ÛÙÚˆÛË Û Ficoll
Hypaque (Lymphoprep-Nycomed d=1077 g/ml) Î·È Ë
·ÔÌfiÓˆÛË ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ ¤ÁÈÓ Ì ·ÓÔÛÔ-Ì·-
ÁÓËÙÈÎfi ‰È·¯ˆÚÈÛÌfi (Mini MACS-Separation
Columns), Û‡Ìʈӷ Ì ÙȘ Ô‰ËÁ›Â˜ ÙÔ˘ ηٷÛ΢·ÛÙ‹
(Miltenyi Biotec), ÂÓÙfi˜ 24 ˆÚÒÓ ·fi ÙË Ï‹„Ë ÙÔ˘
‰Â›ÁÌ·ÙÔ˜.
AÔÌfiÓˆÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ (ª∫) ·fi
Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ
∏ Û˘ÏÏÔÁ‹ ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ ª√ ¤ÁÈÓ ·fi ·È‰È¿
Ì ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· (ȉÈÔ·ı‹ ıÚÔÌ-
‚ÔÂÓÈ΋ ÔÚʇڷ, n=9). ∆· MN¶ ηÏÏÈÂÚÁ‹ıËηÓ
Û a-MEM, ÂÌÏÔ˘ÙÈṲ̂ÓÔ Ì ÂÈÏÂÁ̤ÓÔ ÔÚfi ÂÌ-
‚Ú‡Ô˘ ‚Ôfi˜ (FCS), ÂÓÈÎÈÏÏ›ÓË/ÛÙÚÂÙÔÌ˘Î›ÓË 10%,
ÁÏÔ˘Ù·Ì›ÓË 10% Î·È ·˘ÍËÙÈÎfi ·Ú¿ÁÔÓÙ· ÈÓÔ‚Ï·-
ÛÙÒÓ (FGF-2) 1 ng/ml. ŸÙ·Ó ‰ËÌÈÔ˘ÚÁ‹ıËΠÛÙÚÒÌ·
>75%, ¤ÁÈÓ ıÚ˘„ÈÓÔÔ›ËÛË (ıÚ˘„›ÓË 0,25% +
EDTA) Î·È Ù· ·ÙÙ·Ú· ·ӷηÏÏÈÂÚÁ‹ıËÎ·Ó Û η-
Ù¿ÏÏËÏË Û˘ÁΤÓÙÚˆÛË (P1). ∏ ‰È·‰Èηۛ· ·ӷϋ-
ÊıËΠ̤¯ÚÈ ƒ4.
∞Ó¿Ù˘ÍË CFU-F ·ÔÈÎÈÒÓ - ¯ÚfiÓÔ˜ ΢ÙÙ·ÚÈÎÔ‡ ‰ÈÏ·-
ÛÈ·ÛÌÔ‡: °È· ÙËÓ ·Ó¿Ù˘ÍË CFU-F ·ÔÈÎÈÒÓ 10 ª∫/cm2
ÙÔÔıÂÙ‹ıËÎ·Ó Û ÙÚ˘‚Ï›· Petri 20 cm2 Û ·-ª∂ª (ÂȘ
‰ÈÏÔ‡Ó). ªÂÙ¿ ·fi 14 Ë̤Ú˜ ηÏÏȤÚÁÂÈ·˜, ¤ÁÈÓÂ
¯ÚÒÛË Ì Giemsa Î·È Î·Ù·ÌÂÙÚ‹ıËÎ·Ó ÔÈ ·ÔÈ˘ ÛÂ
·Ó¿ÛÙÚÔÊÔ ÌÈÎÚÔÛÎfiÈÔ.
√ ¯ÚfiÓÔ˜ ΢ÙÙ·ÚÈÎÔ‡ ‰ÈÏ·ÛÈ·ÛÌÔ‡ [cell doubling
time (DT)] ˘ÔÏÔÁ›ÛÙËΠ‚¿ÛÂÈ ÙÔ˘ Ù‡Ô˘:
DT = t / n = t x log2 / log (·ÚÈıÌfi˜ ΢ÙÙ¿ÚˆÓ Ô˘
ÚԤ΢„·Ó ·fi ÙË ıÚ˘„ÈÓÔÔ›ËÛË/ ·ÚÈıÌfi ΢ÙÙ¿ÚˆÓ
Ô˘ ¿Ú¯ÈÛ ÙËÓ Î·ÏÏȤÚÁÂÈ·) (t : Ô ¯ÚfiÓÔ˜ Ô˘ ÌÂÛÔÏ¿-
‚ËÛ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ηÏÏȤÚÁÂÈ·˜ ̤¯ÚÈ ÙË ıÚ˘„È-
ÓÔÔ›ËÛË).
∫·ÏÏȤÚÁÂȘ ‰È·ÊÔÚÔÔ›ËÛ˘ ª∫: ™ÙËÓ ƒ2 ‹ ƒ3 ¤ÁÈÓÂ
·ÁˆÁ‹ ÙˆÓ ª∫ ÚÔ˜ ÏÈÔ·ÙÙ·Ú· (§), ÔÛÙÂÔ·ÙÙ·Ú·
(√) Î·È ¯ÔÓ‰ÚÔ·ÙÙ·Ú· (Ã) Û ÂȉÈÎfi ıÚÂÙÈÎfi ˘ÏÈÎfi.
ªÂ ÈÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË ÂϤÁ¯ıËÎÂ Ë ‰È·ÊÔÚÔÔ›ËÛË
ÚÔ˜ § (Oil red O), √: (Von Kossa) Î·È Ã (Alcian Blue).
ªÂ RT-PCR ·ÓȯÓ‡ıËÎÂ Ë ¤ÎÊÚ·ÛË ÁÔÓȉ›ˆÓ ¯·Ú·ÎÙË-
ÚÈÛÙÈÎÒÓ ÁÈ· § (LPL, aP2), ÁÈ· √ (Osteocalcin,
Osteoprotegerin) Î·È ÁÈ· à (Aggrecan, Collagen II).
∫·ÏÏȤÚÁÂÈ· CD34+ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
·ÚÔ˘Û›· ª∫
°È· ÙË Û˘ÁηÏÏȤÚÁÂÈ· ÙˆÓ ª∫ Ì ٷ ·Ú¯¤ÁÔÓ·
110 π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:110-000
Conclusions: MSC derived from BM of children, used as a feeder layer, supports the ex-vivo expansionand preserves the characteristics of ‘stemness’ of CD34+ HSC in co-culture conditions, providing apopulation large enough to overcome the problems caused by limited HSC numbers for CBtransplants.
Key words: CD34+, mesenchymal stromal cells, ex vivo expansion, cord blood, children bone marrow.
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·110
CD34+ ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ª∫ ƒ3 ‹ ƒ4Ô˘ ÙÔÔıÂÙ‹ıËÎ·Ó Û ÙÚ˘‚Ï›· 24 ‚ÔıÚ›ˆÓ ÂÈÛÙڈ̤ӷ ÌÂ˙ÂÏ·Ù›ÓË, ÛÂ Û˘ÁΤÓÙÚˆÛË 3x104/‚ÔıÚ›Ô. ŸÙ·Ó Ù· ª∫ ‰ËÌÈÔ‡Ú-ÁËÛ·Ó ÛÙÚÒÌ·, ÚÔÛÙ¤ıËÎ·Ó CD34+ ·ÙÙ·Ú· (2,5x103/‚ÔıÚ›Ô )Î·È Âˆ¿ÛÙËÎ·Ó ÁÈ· 10 Ë̤Ú˜ Û 37ÔC 5% CO2, Ì ÚÔÛı‹ÎËıÚÂÙÈÎÔ‡ ˘ÏÈÎÔ‡ Î·È ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÙËÓ 5Ë Ë̤ڷ.∆Ô Î·ÏÏÈÂÚÁËÙÈÎfi ˘ÏÈÎfi ·ÔÙÂÏ›ÙÔ ·fi a-MEM + 10% FCS +10% ÂÓÈÎÈÏÏ›ÓË /ÛÙÚÂÙÔÌ˘Î›ÓË + 10% ÁÏÔ˘Ù·Ì›ÓË, ·ÚÔ˘-Û›· ‹ ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ÃÚËÛÈÌÔÔÈ‹ıËηӉ‡Ô Û˘Ó‰˘·ÛÌÔ› ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ GFmix1 Î·È GFmix2.
GFmix1: SCF: 10 ng/ml, IL-3: 5 ng/ml, G-CSF: 100 ng/mlÎ·È GM-CSF: 1 ng/ml Î·È GFmix2: SCF: 20 ng/ml, TPO: 25ng/ml, IL-6: 20 ng/ml, Flt-3 ligand: 50 ng/ml.
√È Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ Ô˘ ÂÍÂÙ¿ÛıËÎ·Ó ‹Ù·Ó: ™˘Óı‹ÎË ∞: MK+CD34+
™˘Óı‹ÎË µ: MK+CD34+ +GFmix1 ™˘Óı‹ÎË °: MK+CD34+ +GFmix2™˘Óı‹ÎË ¢: CD34+ +GFmix1 ™˘Óı‹ÎË ∂: CD34+ +GFmix2 ∆Ë 10Ë Ë̤ڷ Ù˘ Û˘ÁηÏÏȤÚÁÂÈ·˜ Û˘ÏϤ¯ıËÎ·Ó Ù· ·Ù-
Ù·Ú· ÙÔ˘ ˘ÂÚÎÂÈ̤ÓÔ˘, ÂÓÒ Ù· ÚÔÛÎÔÏÏË̤ӷ ·ÙÙ·Ú··ÔÌ·ÎÚ‡ÓıËÎ·Ó Ì ıÚ˘„ÈÓÔÔ›ËÛË.
∏ ¤ÎÙ˘ÍË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ˘ÔÏÔÁ›ÛÙËÎÂ Û˘ÁÎÚ›ÓÔÓÙ·˜ÙÔÓ ·ÚÈıÌfi ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙË ‰¤Î·ÙË Ë̤ڷ Û˘ÁηÏÏȤÚÁÂÈ·˜Ì ÙÔÓ ·Ú¯ÈÎfi ·ÚÈıÌfi ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ.
∞ÓÔÛÔÊ·ÈÓÔÙ˘ÈÎfi˜ ·Ú·ÎÙËÚÈÛÌfi˜
ªÂÙ¿ ·fi Û‹Ì·ÓÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÌÂ Û˘˙¢Á̤ӷ ÊıÔÚ›-˙ÔÓÙ· ÌÔÓÔÎψÓÈο ·ÓÙÈÛÒÌ·Ù· ·ÎÔÏÔ‡ıËÛ ΢ÙÙ·ÚÔÌÂÙÚ›·ÚÔ‹˜. ŒÁÈÓ ·Ó¿Ï˘ÛË ÁÈ·: CD34-PE, CD45-FITC, CD33-PC5,CD38-PC5, CD105-PE, CD146-P∂ Î·È Ë ‰È¿ÎÚÈÛË ÙˆÓ ª∫·fi Ù· ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ‚·Û›ÛÙËΠÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘CD45 Î·È ÛÙȘ ‰È·ÊÔÚ¤˜ ÙˆÓ ‰‡Ô ΢ÙÙ·ÚÈÎÒÓ Ù‡ˆÓ ·Ó¿ÏÔÁ·Ì ÙÔ Ì¤ÁÂıÔ˜ Î·È ÙËÓ ÎÔÎΛˆÛË (FS/SS).
∞Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ ÎÔÎÎÈÒ‰Ô˘˜ (CFU-GM) Î·È ÂÚ˘ıÚ¿˜
(BFU-E) ÛÂÈÚ¿˜ - ηÏÏȤÚÁÂȘ ÌÂı˘ÏÔ΢ÙÙ·Ú›Ó˘
™Â ηÏÏÈÂÚÁËÙÈÎfi ˘ÏÈÎfi 1% ÌÂı˘ÏÔ΢ÙÙ·Ú›Ó˘ (STEMCELL Technologies HCC4230) ÚÔÛÙ¤ıËÎ·Ó 5x103 ·ÙÙ·Ú·Î·È ÔÈ ·˘ÍËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ G-CSF: 100 ng/ml, IL-3: 5 ng/ml,GM-CSF: 1 ng/ml Î·È ÂÚ˘ıÚÔÔÈËÙ›ÓË (EPO): 4 πU/ml.
√È Î˘ÙÙ·ÚÔηÏÏȤÚÁÂȘ ˆ¿ÛÙËÎ·Ó ÁÈ· 14 Ë̤Ú˜ ÛÂ37ÔC 5% CO2 Î·È Ë Î·Ù·Ì¤ÙÚËÛË ·ÔÈÎÈÒÓ ¤ÁÈÓ Û ·Ó¿-ÛÙÚÔÊÔ ÌÈÎÚÛÎfiÈÔ. ŸÏ· Ù· ÂÈÚ¿Ì·Ù· ¤ÁÈÓ·Ó ÂȘ ‰ÈÏÔ‡Ó.
™Ù·ÙÈÛÙÈ΋ ∞Ó¿Ï˘ÛË
∆· ·ÔÙÂϤÛÌ·Ù· ÂÎÊÚ¿˙ÔÓÙ·È ˆ˜ ̤ÛË ÙÈÌ‹±ÛÙ·ıÂÚfi
ÛÊ¿ÏÌ· Ù˘ ̤Û˘ ÙÈÌ‹˜ (SEM). √È ÌÂÙ·‚ÏËÙ¤˜ Ì ÌË Î·ÓÔ-ÓÈ΋ ηٷÓÔÌ‹ Û˘ÁÎÚ›ıËÎ·Ó Ì Mann-Whitney U test. ŸÏ˜ÔÈ Û˘ÁÎÚ›ÛÂȘ Â›Ó·È ‰ÈÏ‹˜ ηÙ‡ı˘ÓÛ˘. ™Ù·ÙÈÛÙÈÎÒ˜ ÛËÌ·-ÓÙÈο ıˆڋıËÎ·Ó Ù· ·ÔÙÂϤÛÌ·Ù· Ì p<0,05. ∏ ·Ó¿Ï˘ÛˤÁÈÓ Ì ÙÔ Statistical Package for Social Sciences (SPSS).
∞ÔÙÂϤÛÌ·Ù·
÷ڷÎÙËÚÈÛÙÈο ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
∆· ª∫ ÂÌÊ¿ÓÈÛ·Ó ÛÙËÓ ÚÒÙË Î·ÏÏȤÚÁÂÈ· ηȉȷًÚËÛ·Ó ÛÙȘ ÂfiÌÂÓ˜ ·ӷηÏÏȤÚÁÂȘ ÙÔ¯·Ú·ÎÙËÚÈÛÙÈÎfi ·ÙÚ·ÎÙÔÂȉ¤˜ Û¯‹Ì· (∂ÈÎfiÓ· 1).∞ÓÔÛÔÊ·ÈÓÔÙ˘Èο, ‹‰Ë ·fi ÙËÓ ÚÒÙË Â·Ó·Î·Ï-ÏȤÚÁÂÈ· ‰ÂÓ ·Ú·ÙËÚ‹ıËΠ¤ÎÊÚ·ÛË ÙˆÓ ·ÈÌÔÔÈË-ÙÈÎÒÓ ‰ÂÈÎÙÒÓ CD34 Î·È CD45, ÂÓÒ ÔÈ Û¯ÂÙÈ˙fiÌÂÓÔÈÌ ٷ MK ‰Â›ÎÙ˜, Ô˘ ÂÎÊÚ¿˙ÔÓÙ·Ó Û Ôχ ÌÈÎÚfiÔÛÔÛÙfi ÛÙÔÓ ÏËı˘ÛÌfi ÙˆÓ M¶¡ (CD105:1,85±0,3%, CD146: 0,93±0,3%), ÂÎÊÚ¿˙ÔÓÙ·Ó Û¢„ËÏfi ÔÛÔÛÙfi ÛÙȘ ·ӷηÏÏȤÚÁÂȘ ÙˆÓ ª∫(CD105: 99,4±0,23% Î·È CD146: 94,05±1,45%).
™ÙËÓ ƒ2 ÙÔ Û‡ÓÔÏÔ ÙˆÓ ·ÔÈÎÈÒÓ CFU-F ‹Ù·Ó38,05±3,7 Î·È ÛÙËÓ P4 ‹Ù·Ó 24,03±3. √ ¯ÚfiÓÔ˜ ΢Ù-Ù·ÚÈÎÔ‡ ‰ÈÏ·ÛÈ·ÛÌÔ‡ ÛÙËÓ ƒ2 ‹Ù·Ó 1,93±0,1 Ë̤-Ú˜ Î·È ÛÙËÓ ƒ4 2,6±0,2 Ë̤Ú˜.
∞fi ÙËÓ ÈÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË Ê¿ÓËΠfiÙÈ Ù· ª∫¤¯Ô˘Ó ÈηÓfiÙËÙ· ‰È·ÊÔÚÔÔ›ËÛ˘ ÚÔ˜ ÔÛÙÂÔ·Ù-Ù·Ú·, ÏÈÔ·ÙÙ·Ú·, ¯ÔÓ‰ÚÔ·ÙÙ·Ú· (∂ÈÎfiÓ· 2). ∏·Ó¿Ï˘ÛË Ì RT-PCR ¤‰ÂÈÍ fiÙÈ Ù· ·ÙÙ·Ú· ·˘Ù¿ ÂÎ-ÊÚ¿˙Ô˘Ó ÌÔÚÈ·ÎÔ‡˜ ‰Â›ÎÙ˜ ÂȉÈÎÔ‡˜ ÁÈ· ÔÛÙÂÔ·Ù-Ù·Ú·, ÏÈÔ·ÙÙ·Ú· Î·È ¯ÔÓ‰ÚÔ·ÙÙ·Ú· (‰ÂÓ ·Ú·Ù›-ıÂÓÙ·È Ù· ‰Â‰Ô̤ӷ).
∞ÔÌfiÓˆÛË CD34+ ΢ÙÙ¿ÚˆÓ
√ ̤ÛÔ˜ fiÁÎÔ˜ Û˘ÏÏÔÁ‹˜ √§ ‹Ù·Ó 39,8±2,2 ml,ÂÓÒ ÌÂÙ¿ ÙËÓ Â›ÛÙÚˆÛË Û Ficoll-Hypaque ·ÔÌÔ-ÓÒıËÎ·Ó 2,9±0,5x108 ª¡¶. ªÂÙ¿ ÙÔÓ ·ÓÔÛÔÌ·ÁÓË-ÙÈÎfi ‰È·¯ˆÚÈÛÌfi, Ù· ·ÙÙ·Ú· ‹Ù·Ó 54,3±2,1x104 ηÈÂͤÊÚ·˙·Ó CD34 Û ÔÛÔÛÙfi 93±1,43%.
∂›‰Ú·ÛË Ù˘ ·ÚÔ˘Û›·˜ ª∫ ÛÙËÓ ¤ÎÙ˘ÍË ÙÔ˘
Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ΢ÙÙ¿ÚˆÓ
∏ Ù·˘Ùfi¯ÚÔÓË ·ÚÔ˘Û›· ª∫ Î·È ·˘ÍËÙÈÎÒÓ
111ªÂÛÂÁ¯˘Ì·ÙÈο Î·È ¤ÎÙ˘ÍË ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜
¶·È‰È·ÙÚÈ΋ 2009;72:111-000
∂ÈÎfiÓ· 1. ·) ªÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ‚) ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ÌÂÙ¿ ·fi ‰¤Î· Ë̤Ú˜ ηÏÏȤÚÁÂÈ·˜ ·ÚÔ˘Û›· ÌfiÓÔ ·˘ÍËÙÈÎÒÓ ·Ú·Áfi-ÓÙˆÓ, Á) ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· Û ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· ÛÙÚÒÌ·ÙÔ˜ ÙË ‰¤Î·ÙË Ë̤ڷ Û˘ÁηÏÏȤÚÁÂÈ·˜.
· ‚ Á
Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·111
·Ú·ÁfiÓÙˆÓ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ·
ÙË ÌÂÁ·Ï‡ÙÂÚË ¤ÎÙ˘ÍË ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ
΢ÙÙ¿ÚˆÓ (Û˘Óı‹ÎË B/° Û ۇÁÎÚÈÛË ÌÂ Û˘Óı‹Î˜
¢/∂) (∂ÈÎfiÓ· 1), ÂÓÒ ÔÈ Û˘Ó‰˘·ÛÌÔ› ÙˆÓ ·˘ÍËÙÈÎÒÓ
·Ú·ÁfiÓÙˆÓ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ‰ÂÓ ‰È¤ÊÂÚ·Ó
ÛÙËÓ Â›‰Ú·ÛË Ô˘ ›¯·Ó ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ Î˘ÙÙ¿-
ÚˆÓ (¶›Ó·Î·˜ 1, Û˘Óı‹ÎË µ Î·È °). ∆· ·ÈÌÔÔÈËÙÈο
·ÙÙ·Ú· Ô˘ ηÏÏÈÂÚÁ‹ıËÎ·Ó ·ÔÎÏÂÈÛÙÈο ·ÚÔ˘-
Û›· ΢ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ (Û˘Óı‹ÎË ∞) ·˘Í‹ıËηÓ
ηٿ 10,9±5,73 ÊÔÚ¤˜ Î·È ·˘Ù‹ Ë Û˘Óı‹ÎË ·¤ÊÂÚÂ
ÙË ÌÈÎÚfiÙÂÚË ¤ÎÙ˘ÍË (p≤0,05) Û ۇÁÎÚÈÛË Ì fiϘ
ÙȘ ˘fiÏÔȘ (¶›Ó·Î·˜ 1).
∂›‰Ú·ÛË Ù˘ ·ÚÔ˘Û›·˜ ª∫ ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ
·Ú¯¤ÁÔÓˆÓ/ÚÔÁÔÓÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ
∏ Ù·˘Ùfi¯ÚÔÓË ·ÚÔ˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ
-·ÓÂÍ·Úًو˜ Û˘Ó‰˘·ÛÌÔ‡- Î·È ÛÙÚÒÌ·ÙÔ˜ (Û˘Óı‹-
ÎË µ Î·È °, ¶›Ó·Î·˜ 1) ¤ÊÂÚ ÙË ÌÂÁ·Ï‡ÙÂÚË ¤Î-
Ù˘ÍË ÛÙ· CD34+ ·ÙÙ·Ú·, ÂÓÒ ÙÔ Ùˆ¯fiÙÂÚÔ ÔÛÔ-
ÙÈο ·ÔÙ¤ÏÂÛÌ· ›¯Â Ë Û˘Óı‹ÎË ∞ (p=0,025). ∏
ηÏÏȤÚÁÂÈ· ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì ·˘ÍË-
ÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÌfiÓÔ (Û˘Óı‹Î˜ ¢ Î·È ∂) ¤ÊÂ-
Ú ÈηÓÔÔÈËÙÈ΋ ¤ÎÙ˘Í‹ ÙÔ˘˜, Ë ÔÔ›·, ·Ó ηÈ
·ÚÈıÌËÙÈο ‹Ù·Ó ÌÈÎÚfiÙÂÚË ·˘Ù‹˜ Ô˘ ÚÔ·ÙÂÈ
·ÚÔ˘Û›· ÛÙÚÒÌ·ÙÔ˜, ÂÓ ÙÔ‡ÙÔȘ ‰ÂÓ ÊÙ¿ÓÂÈ ÛÙ·
fiÚÈ· Ù˘ ÛÙ·ÙÈÛÙÈ΋˜ ÛËÌ·ÓÙÈÎfiÙËÙ·˜.
∞ÓÔÛÔÊ·ÈÓÔÙ˘Èο ·Ú·ÎÙËÚÈÛÙÈο ΢ÙÙ¿ÚˆÓ
ÛÙÚÒÌ·ÙÔ˜ Î·È ÂÎÙ˘ÁÌ¤ÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ
΢ÙÙ¿ÚˆÓ
√ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ¤ÎÊÚ·Û˘ ÙÔ˘ CD34 ¤‰ÂÈÍÂ
ˆ˜ Ë ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ·fi ÙËÓ Î·Ï-ÏȤÚÁÂÈ· ¤¯ÂÈ ˆ˜ Û˘Ó¤ÂÈ· ÌÂÁ·Ï‡ÙÂÚÔ ÂηÙÔÛÙÈ·›ÔÔÛÔÛÙfi ΢ÙÙ¿ÚˆÓ Ô˘ ÙÔ ÂÎÊÚ¿˙Ô˘Ó (¶›Ó·Î·˜ 1).
∆· ÔÛÔÛÙ¿ ÙˆÓ ÏËı˘ÛÌÒÓ CD34+38- ηÈCD34+33- Ô˘ Ô‰ÂÈÎÓ‡Ô˘Ó Î‡ÙÙ·Ú· Ì ·Ú·ÎÙËÚÈ-ÛÙÈο ·ˆÚfiÙËÙ·˜ ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚ· ÛÙË Û˘Óı‹ÎË ∞(·ÚÔ˘Û›· ÌfiÓÔ ª∫), Û ۇÁÎÚÈÛË Ì ÙȘ ˘fiÏÔÈÂ˜Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ (µ/°/¢ Î·È ∂). ∆Ô ÔÛÔÛÙfiÙˆÓ CD34+33- ΢ÙÙ¿ÚˆÓ ‹Ù·Ó 40,5±3,6% ÛÙË Û˘Óı‹-ÎË ∞, 4,1±0,9% ÛÙË Û˘Óı‹ÎË µ Î·È 4±1,1% ÛÙË Û˘Ó-ı‹ÎË ¢. (∂ÈÎfiÓ· 3). ∆· ·ÓÙ›ÛÙÔȯ· ÔÛÔÛÙ¿ ÁÈ· Ù·CD34+38- ·ÙÙ·Ú· ‹Ù·Ó 5,6±3,4%, 1,3±0,9% ηÈ4,2±3,8%. ¢ÂÓ ·Ú·ÙËÚ‹ıËΠ‰È·ÊÔÚ¿ ·˘ÙÒÓ ÙˆÓ΢ÙÙ·ÚÈÎÒÓ ˘ÔÏËı˘ÛÌÒÓ ÂÍ·ÚÙÒÌÂÓË ·fi ÙÔÓÛ˘Ó‰˘·ÛÌfi ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (Û˘Óı‹Î˜µ ÚÔ˜ ° Î·È ¢ ÚÔ˜ ∂) (∂ÈÎfiÓ· 3).
∞Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ ÎÔÎÎÈÒ‰Ô˘˜ (CFU-GM) ηÈ
ÂÚ˘ıÚ¿˜ (BFU-E) ÛÂÈÚ¿˜
∞Ó¿Ù˘ÍË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÔÈÎÈÒÓ CFU-GM (186,81±9,9) ·Ú·ÙËÚ‹ıËΠ·ÚÔ˘Û›· ÌfiÓÔ΢ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ (∂ÈÎfiÓ· 4). ™˘ÁÎÚ›ÓÔÓÙ·˜ ÌÂfiϘ ÙȘ ˘fiÏÔȘ Û˘Óı‹Î˜, Ê·›ÓÂÙ·È ˆ˜ Ë ·-ÚÔ˘Û›· ÌfiÓÔ ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ·, ÌÔÏÔÓfiÙÈ ‰ÂÓÚÔ¿ÁÂÈ ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ Î˘ÙÙ¿ÚˆÓ (ÌÈÎÚfi-ÙÂÚË ¤ÎÙ˘ÍË ÙÔ˘ ÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ Î·È ÙÔ˘ ·ÚÈıÌÔ‡ÙˆÓ CD34+), ÂÓ ÙÔ‡ÙÔȘ ·Ú¤¯ÂÈ Î‡ÙÙ·Ú· Ì ·˘ÍË̤-ÓË ÎψÓÔÁÔÓÈ΋ ÈηÓfiÙËÙ·. ∂›Û˘, ·Ú·ÙËÚ›ٷȉȷÊÔÚÂÙÈÎfi˜ Ú˘ıÌfi˜ ·Ó¿Ù˘Í˘ ·ÔÈÎÈÒÓ ·Ó¿ÏÔÁ·Ì ÙÔÓ Û˘Ó‰˘·ÛÌfi ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Î·È ÔGFmix1 (Û˘Óı‹ÎË ¢) ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ·Ó¿Ù˘ÍËÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÔÈÎÈÒÓ (147,0±12,9), ÛÂ
¶›Ó·Î·˜ 1. ∞ÔÙÂϤÛÌ·Ù· ¤ÎÙ˘Í˘ ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÙÙ¿ÚˆÓ Î·È ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ Î·È ÔÛÔÛÙfi ¤ÎÊÚ·Û˘ ÙÔ˘ CD34 ·ÓÙÈ-ÁfiÓÔ˘ ÂÈÊ·Ó›·˜ ÌÂÙ¿ ·fi ‰¤Î· Ë̤Ú˜ Û˘ÁηÏÏȤÚÁÂÈ·˜
™˘Óı‹ÎË ŒÎÙ˘ÍË ÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ŒÎÙ˘ÍË CD34+ % CD34΢ÙÙ¿ÚˆÓ (x)
∞ CD34∆ + ª∫ 10,9 ± 5,7 2,2 ± 0 ,7 50,6 ± 5,5µ CD34∆ + ª∫ + GFmix1 164,4 ± 42,7* 20,9 ± 5,9* 13,1± 0,9*° CD34∆ + ª∫ + GFmix2 165,8 ± 35,8* 16,8 ± 0,5* 11,4 ± 2,0*¢ CD34∆ + + GFmix1 101, 9 ± 36,3* 13,7 ± 9,7 10,9 ± 5,5∂ CD34∆ + + GFmix2 134,2 ± 10,8* 14,2 ± 1,7* 10,5 ± 0,4
*ηٷ‰ÂÈÎÓ‡ÂÈ ÛÙ·ÙÈÛÙÈ΋ ÛËÌ·ÓÙÈÎfiÙËÙ·, p<0,05 Û ۇÁÎÚÈÛË Ì ÙË Û˘Óı‹ÎË ∞
∂ÈÎfiÓ· 2. πÛÙÔ¯ËÌÈΤ˜ ¯ÚÒÛÂȘ ·fi ‰È·ÊÔÚÔÔÈË̤ӷ ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·: ·) ÏÈÔ·ÙÙ·Ú·, ‚) ÔÛÙÂÔ·ÙÙ·Ú·, Á) ¯ÔÓ‰ÚÔ·ÙÙ·Ú·.
112 π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:112-000
· ‚ Á
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·112
Û‡ÁÎÚÈÛË Ì ÙÔ GFmix2 (Û˘Óı‹ÎË ∂) (58,5±11,9)
(p=0,02) (∂ÈÎfiÓ· 4).
∞Ó·ÊÔÚÈο Ì ÙȘ ·ÔÈ˘ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, Ë
·ÚÔ˘Û›· ÁÂÓÈο ª∫ ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ó¿-
Ù˘ÍË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÔÈÎÈÒÓ. ™˘ÁÎÂÎÚÈ̤-
Ó·, ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ·ÔÈ˘ ‰ËÌÈÔ˘ÚÁ‹ıËÎ·Ó ·fi Ù·
·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ·ÚÔ˘Û›· ÌfiÓÔ ª∫ (Û˘Óı‹ÎË
∞), ÂÓÒ ÔÈ ÏÈÁfiÙÂÚ˜ ÛÙȘ Û˘Óı‹Î˜ ¢ Î·È ∂ fiÔ˘
·Ô˘Û›·˙·Ó ÙÂÏ›ˆ˜ Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο (p=0,03 ηÈ
p=0,01, ·ÓÙ›ÛÙÔȯ·, Û ۇÁÎÚÈÛË Ì ™˘Óı‹ÎË ∞) (∂ÈÎfi-
Ó· 4). ¶·ÚÔÌÔ›ˆ˜, fiˆ˜ Î·È ÁÈ· ÙȘ ·ÔÈ˘ Ù˘ ÎÔÎ-
ÎÈÒ‰Ô˘˜ ÛÂÈÚ¿˜, Ô Û˘Ó‰˘·ÛÌfi˜ ·Ú·ÁfiÓÙˆÓ GMix1
¤‰ˆÛ ÂÚÈÛÛfiÙÂÚ˜ ·ÔÈ˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, Û ۯ¤-
ÛË Ì ÙÔÓ Û˘Ó‰˘·ÛÌfi GMix 2 (Û˘Óı‹ÎË µ/° Î·È ¢/∂).
™˘˙‹ÙËÛË
∆· ·ÙÙ·Ú· ÛÙÚÒÌ·ÙÔ˜ ·ÔÙÂÏÔ‡Ó Ì¤ÚÔ˜ ÙÔ˘ ÌÈ-
ÎÚÔÂÚÈ‚¿ÏÏÔÓÙÔ˜ ÙÔ˘ ª√ Î·È Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙË
Ú‡ıÌÈÛË Ù˘ ·˘ÙÔ·Ó·Ó¤ˆÛ˘, ÙÔ˘ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡
Î·È Ù˘ ‰È·ÊÔÚÔÔ›ËÛ˘ ÙˆÓ ·Ú¯¤ÁÔÓˆÓ/ÚÔÁÔÓÈÎÒÓ
·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. √ ·ÎÚÈ‚‹˜ Ì˯·ÓÈÛÌfi˜ ·˘-
Ù‹˜ Ù˘ ‰È·‰Èηۛ·˜ ‰ÂÓ ¤¯ÂÈ ·ÔÛ·ÊËÓÈÛÙ›, ·ÏÏ¿
A
E
A
E
A AA
40,48% 27,45%
E¢¢¢°
B
4,15%
BB
15,20%
CD
33
PC
5
CD34 PE
1,40% 4,00% 17,95% 0,45%
A
E
A
E
A AA
5,63% 7,15%
E¢¢¢°
B
1,35%
BB
2,83%
CD
38
PC
5
CD34 PE
0,40% 4,20% 20,18% 0,45%
∂ÈÎfiÓ· 3. ∞ÓÔÛÔÊ·ÈÓÔÙ˘È΋ ·Ó¿Ï˘ÛË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ √§ ÌÂÙ¿ ·fi ‰¤Î· Ë̤Ú˜ ηÏÏȤÚÁÂÈ·˜ Ì ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·ÛÙÚÒÌ·ÙÔ˜. ∏ ·Ó¿Ï˘ÛË ¤ÁÈÓ ˆ˜ ÚÔ˜ ÙËÓ ¤ÎÊÚ·ÛË CD34 CD33 Î·È CD34CD38 ÛÙÔÓ CD45+ ÏËı˘ÛÌfi.
113ªÂÛÂÁ¯˘Ì·ÙÈο Î·È ¤ÎÙ˘ÍË ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜
¶·È‰È·ÙÚÈ΋ 2009;72:113-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·113
Èı·ÓÒ˜ ÂÈÙ˘Á¯¿ÓÂÙ·È Ì¤Ûˆ ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ Î·È
¿ÏÏˆÓ ÂÎÎÚÈÓfiÌÂÓˆÓ ·fi Ù· ª∫ ·Ú·ÁfiÓÙˆÓ, ηıÒ˜
Î·È Ì ÙËÓ Â·Ê‹ ·ÙÙ·ÚÔ-·ÙÙ·ÚÔ (18).
∆ËÓ ÙÂÏÂ˘Ù·›· 15ÂÙ›· ·ÂÙ¤ÏÂÛ ·ÓÙÈΛÌÂÓÔ
ÂÎÙÂٷ̤Ó˘ ÌÂϤÙ˘ Ë ex vivo ¤ÎÙ˘ÍË ·›Ì·ÙÔ˜ √§,
ÛÙËÓ ÚÔÛ¿ıÂÈ· ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ÌÂÈÔÓÂÎÙËÌ¿-
ÙˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ √§ ˆ˜ ÌÔۯ‡̷ÙÔ˜, ÒÛÙ ӷ ·ÍÈÔ-
ÔÈËıÔ‡Ó Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· Ô˘ ·Ú¤¯ÂÈ ˆ˜ ËÁ‹
·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ™Ù· Ï·›ÛÈ·
·˘Ù¿ ‰ÔÎÈÌ¿ÛÙËÎ·Ó ‰È¿ÊÔÚ˜ Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ-
·˜, ÔÈ Ôԛ˜ ÂÚÈÂÏ¿Ì‚·Ó·Ó ΢ڛˆ˜ ‰È·ÊÔÚÂÙÈÎÔ‡˜
Û˘Ó‰˘·ÛÌÔ‡˜ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (6-8,19).
™ÎÔfi˜ ‹Ù·Ó Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ È‰·ÓÈÎÒÓ Û˘Ó-
ıËÎÒÓ Î·È Ù˘ ›‰Ú·Û˘ Û˘Ó‰˘·ÛÌÔ‡ ·Ú·ÁfiÓÙˆÓ
Ô˘, ÌÂÙ¿ ·fi Û¯ÂÙÈο Û‡ÓÙÔÌË Î·ÏÏȤÚÁÂÈ·, ı·
ÌÔÚÔ‡Û ӷ ·Ú¤¯ÂÈ ·Ú¯¤ÁÔÓ· ·ÈÌÔÔÈËÙÈο ·ÙÙ·-
Ú· Û ·˘ÍË̤ÓÔ ·ÚÈıÌfi Î·È Ì ‰È·Ù‹ÚËÛË -Û fiÛÔ ÙÔ
‰˘Ó·ÙfiÓ ÌÂÁ·Ï‡ÙÂÚÔ ‚·ıÌfi- ÙˆÓ È‰ÈÔÙ‹ÙˆÓ ÙÔ˘ «·Ú-
¯¤ÁÔÓÔ˘» ΢ÙÙ¿ÚÔ˘. ∆· ‰Â‰Ô̤ӷ ÔÏÏÒÓ ÌÂÏÂÙÒÓ,
·Ó Î·È ·Ú›¯·Ó ÂÓı·ÚÚ˘ÓÙÈο ·ÔÙÂϤÛÌ·Ù·, ‰ÂÓ
ÌÔÚ› Ó· ıˆÚËı› fiÙÈ ·ÓÙÈÌÂÙÒÈÛ·Ó ÈηÓÔÔÈËÙÈ-
ο ÙÔ Úfi‚ÏËÌ·, ·ÊÔ‡, Û‡Ìʈӷ Ì ÎÏÈÓÈΤ˜ ÌÂϤ-
Ù˜ Ê¿Û˘ π, ‰ÂÓ ·Ú·ÙËÚ‹ıËΠÛÙ·ıÂÚ‹ ·Ôηٿ-
ÛÙ·ÛË Ù˘ ·ÈÌÔÔ›ËÛ˘, Èı·ÓÒ˜ ÁÈ·Ù› ηٿ ÙË ‰È¿Ú-
ÎÂÈ· Ù˘ ¤ÎÙ˘Í˘ ÔÈ Î·ÏÏÈÂÚÁËÙÈΤ˜ Û˘Óı‹Î˜ Ô‰Ë-
ÁÔ‡Û·Ó Û ÈÔ ‰È·ÊÔÚÔÔÈË̤ӷ ·ÙÙ·Ú· (20-21).
¶ÈÔ ÚfiÛÊ·Ù·, ÂÍÂÙ¿ÛÙËÎÂ Â¿Ó Ù· ª∫ ı· ÌÔ-
ÚÔ‡Û·Ó Ó· Û˘ÁηÏÏÈÂÚÁËıÔ‡Ó Ì ·Ú¯¤ÁÔÓ· ·ÈÌÔÔÈ-
ËÙÈο ·ÙÙ·Ú· ÁÈ· Ó· ÂÈÙ¢¯ı› ηχÙÂÚË ex vivo
¤ÎÙ˘ÍË ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ (22).
™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ ‹Ù·Ó Ë in vitro
ÌÂϤÙË Ù˘ ›‰Ú·Û˘ ÙˆÓ ª∫ ·fi ª√ ·È‰ÈÒÓ ÌÂ
ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· Û CD34+ ·ÙÙ·Ú·
·›Ì·ÙÔ˜ √§ Î·È Ë Û‡ÁÎÚÈÛË ÙˆÓ ·ÔÙÂϤÛÌ·ÙˆÓ ÌÂ
·ÓÙ›ÛÙÔȯ˜ ηÏÏȤÚÁÂȘ, ·ÚÔ˘Û›· Û˘Ó‰˘·ÛÌÔ‡
·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ.
∆· ·ÔÙÂϤÛÌ·Ù· ¤‰ÂÈÍ·Ó fiÙÈ Ë ·ÚÔ˘Û›· ÌfiÓÔ
ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· Û˘Ì‚¿ÏÏÂÈ Û ۯÂÙÈο ÂÚÈÔÚÈ-
Ṳ̂ÓË ¤ÎÙ˘ÍË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ/ÚÔÁÔÓÈÎÒÓ Î˘Ù-
Ù¿ÚˆÓ, ·ÏÏ¿ ·ÔʤÚÂÈ ÏËı˘ÛÌfi Ô˘ ÂÎÊÚ¿˙ÂÈ ÛÂ
ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÙÔ ·ÓÙÈÁfiÓÔ ÂÈÊ·Ó›·˜ CD34.
∆· ·ÔÙÂϤÛÌ·Ù· ·˘Ù¿ Û˘ÁÎÏ›ÓÔ˘Ó Ì ·˘Ù¿ Ô˘
·Ó·Ê¤ÚÔÓÙ·È ·fi ÙÔ˘˜ Jang et al. (23), ÔÈ ÔÔ›ÔÈ fï˜
¯ÚËÛÈÌÔÔ›ËÛ·Ó ª∫ ·fi ·›Ì· √§. ∞Í›˙ÂÈ Ó· ÛËÌÂÈ-
ˆı› fiÙÈ Ë ÂÈÙ˘¯‹˜ ·ÔÌfiÓˆÛË ª∫ ·fi ·›Ì· √§
·Ú·Ì¤ÓÂÈ ‰‡ÛÎÔÏË ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ Î·È ·ÎfiÌË
·ÌÊÈÏÂÁfiÌÂÓË ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· (24-26). ™ÙËÓ ›‰È·
ÌÂϤÙË ¤ÁÈÓ ›Û˘ Û‡ÁÎÚÈÛË Ù˘ ¤ÎÙ˘Í˘ CD34+
Ì ٷ ·ÔÙÂϤÛÌ·Ù· Ô˘ ÚԤ΢„·Ó ·fi ÙËÓ ¤Î-
Ù˘ÍË CD34+ ÛÂ Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ Ì ª∫ Ì˘Â-
ÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·fi ˘ÁÈ›˜ ÂÓ‹ÏÈΘ. ∞fi Ù· ·ÔÙÂ-
ϤÛÌ·Ù· Ê·›ÓÂÙ·È fiÙÈ Ù· ÙÂÏÂ˘Ù·›· ˘ÛÙÂÚÔ‡Ó ÛÙËÓ
·fi‰ÔÛË CD34+ ÏËı˘ÛÌÔ‡, Û ۇÁÎÚÈÛË Ì ·˘Ù¿
ÙÔ˘ √§ Î·È Û˘ÁÎÚÈÙÈο Ì ٷ ª∫ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ
·È‰ÈÒÓ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÛÙËÓ ·ÚÔ‡Û· ÌÂ-
ϤÙË. √È Yamaguchi et al. (10) ›Û˘ ·Ó·Ê¤ÚÔ˘Ó
·Ó¿ÏÔÁË ¤ÎÙ˘ÍË CD34+ Ì ÙË ¯Ú‹ÛË ÌfiÓÔ Î˘ÙÙ¿-
ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ ·fi ª√ ÂÓ‹ÏÈÎˆÓ ‰ÔÙÒÓ ‹ ÂÓ‹ÏÈ-
ÎˆÓ ·ÛıÂÓÒÓ Ì ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù·, ¯ˆÚ›˜ Ó·
ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ÙÔ Â›‰Ô˜ ÙÔ˘ ·ÈÌ·ÙÔÏÔÁÈÎÔ‡ ÓÔÛ‹Ì·-
ÙÔ˜. ∆· ·ÙÙ·Ú· ÛÙÚÒÌ·ÙÔ˜ ·fi ª√, ÂÓ ÙÔ‡ÙÔȘ, ‰ÂÓ
·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙËÓ ÔÌÔÈÔÁ¤ÓÂÈ· ÙÔ˘ ÏËı˘ÛÌÔ‡ ÙˆÓ
ª∫ Î·È Èı·Ó¿ ÚÔÛı¤ÙÔ˘Ó Î·È ¿ÏϘ ·Ú·Ì¤ÙÚÔ˘˜,
ÂÚÈÔÚ›˙ÔÓÙ·˜ ÙË ‰˘Ó·ÙfiÙËÙ· Û‡ÁÎÚÈÛ˘ ÙˆÓ ·ÔÙÂ-
ÏÂÛÌ¿ÙˆÓ. ∂ÈϤÔÓ, ÛÙË ÌÂϤÙË ·˘Ù‹ ‰ÂÓ ·Ó·Ê¤ÚÂ-
Ù·È ·Ó ˘‹ÚÍ·Ó ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ˘ÁÈÒÓ Î·È ·ÛıÂÓÒÓ
ÛÙËÓ ¤ÎÙ˘ÍË ÙÔ˘ ˘ÔÏËı˘ÛÌÔ‡ ÙˆÓ ÚÔÁÔÓÈÎÒÓ
·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ (10).
114 π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:114-000
180
150
120
90
60
30
0A B ° ¢ ∂
™˘Óı‹ÎË
∞Ú
ÈıÌ
fi˜
·
ÔÈÎ
ÈÒÓ/
5x1
03 Î
‡ÙÙ
·Ú
·
CFU-GM
BFU-E
∂ÈÎfiÓ· 4. ∞Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ CFU-GM Î·È BFU-E ÛÙȘ ‰È·ÊÔÚÂÙÈΤ˜ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜.
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·114
°È· Ó· ÂÏÂÁ¯ıÔ‡Ó ·ÊÂÓfi˜ Ë ÈηÓfiÙËÙ· ÙˆÓ ª∫ Ó·
ÂÓÈÛ¯‡ÛÔ˘Ó ÙËÓ ¤ÎÙ˘ÍË ÙˆÓ ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈ-
ÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È ·ÊÂÙ¤ÚÔ˘ Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ
ÏËı˘ÛÌÒÓ Ô˘ ÚÔ‹Ïı·Ó ·fi ÙËÓ Î·ÏÏȤÚÁÂÈ· ¤Î-
Ù˘Í˘, ¤ÁÈÓ·Ó Î·ÏÏȤÚÁÂȘ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË CFU-
GM Î·È BFU-E ·ÔÈÎÈÒÓ. ¶·Ú·ÙËÚ‹ıËΠÌÂÁ·Ï‡ÙÂ-
ÚÔ˜ ·ÚÈıÌfi˜ Î·È ÙˆÓ ‰‡Ô Ù‡ˆÓ ·ÔÈÎÈÒÓ ÛÙȘ Û˘Ó-
ı‹Î˜ fiÔ˘ ˘‹Ú¯·Ó ÌfiÓÔ ª∫. ™ÙË ‚È‚ÏÈÔÁÚ·Ê›·
·Ó·Ê¤ÚÂÙ·È (23) ÌË ·Ó¿Ù˘ÍË CFU-GM ÌÂÙ¿ ÙËÓ 7Ë
Ë̤ڷ ηÏÏȤÚÁÂÈ·˜, Ô˘ Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ÔÊ›ÏÂ-
Ù·È ÛÙÔ fiÙÈ Ù· ·ÙÙ·Ú· fi ÙȘ Û˘ÁÎÂÎÚÈ̤Ó˜ Û˘Óı‹-
Θ ˆı‹ıËÎ·Ó Û ‰È·ÊÔÚÔÔ›ËÛË ·ÊÔ‡ ‹Ïı η-
ٷӿψÛË ‹ ·Ú·›ˆÛË ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ
Ô˘ ÚÔ¿ÁÔ˘Ó ÙËÓ ·˘ÙÔ·Ó·Ó¤ˆÛË. ∞ÓÙ›ÛÙÔÈ¯Ë ÌË
·Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ ·Ú·ÙËÚ‹ıËΠ›Û˘ Î·È ·-
ÚÔ˘Û›· ÌfiÓÔ Î˘ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ ÌÂÙ¿ ÙËÓ 7Ë Ë̤-
Ú· ηÏÏȤÚÁÂÈ·˜ (23). ∞Í›˙ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ ÔÈ Û˘Óı‹-
Θ ηÏÏȤÚÁÂÈ·˜ ‰ÂÓ Â›Ó·È Ù·˘ÙfiÛË̘ ÛÙȘ Û˘ÁÎÚÈ-
ÓfiÌÂÓ˜ ÌÂϤÙ˜, ηıÒ˜, ¤Ú·Ó Ù˘ ÌÂÁ·Ï‡ÙÂÚ˘
‰È¿ÚÎÂÈ·˜ Ù˘ ηÏÏȤÚÁÂÈ·˜, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÔÈÔ-
ÙÈο Î·È ÔÛÔÙÈο ‰È·ÊÔÚÂÙÈÎÔ› Û˘Ó‰˘·ÛÌÔ› ·˘ÍËÙÈ-
ÎÒÓ ·Ú·ÁfiÓÙˆÓ, ηıÒ˜ Î·È ª∫ ΢ڛˆ˜ √§, ·ÏÏ¿
Î·È ÂÓËϛΈÓ. ∞fi Ù· ‰Â‰Ô̤ӷ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤ-
Ù˘ Ê·›ÓÂÙ·È, ÏÔÈfiÓ, fiÙÈ Ë ¯Ú‹ÛË ª∫ ÛÙËÓ ¤ÎÙ˘ÍË
·¤‰ˆÛ ÙÔÓ ÌÂÁ·Ï‡ÙÂÚÔ ·ÚÈıÌfi ·ÔÈÎÈÒÓ ÙfiÛÔ Ù˘
ÎÔÎÎÈÒ‰Ô˘˜ fiÛÔ Î·È Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ ·fi ÔÔÈ·-
‰‹ÔÙ ¿ÏÏË Û˘Óı‹ÎË Î·ÏÏȤÚÁÂÈ·˜ Ô˘ ‰ÔÎÈÌ¿ÛÙË-
ÎÂ, ÂÓ‰ÂÈÎÙÈÎfi Èı·Ó‹˜ «ÔÈÔÙÈ΋˜» ‰È·ÊÔÚ¿˜ ÙˆÓ
ª∫ ·È‰ÈÒÓ, ·ÊÔ‡ Ë ·ÚÔ˘Û›· ÙÔ˘˜ ÚÔ¿ÁÂÈ ÙËÓ ¤Î-
Ù˘ÍË ÏËı˘ÛÌÔ‡ Ì ·˘ÍË̤ÓË ÎψÓÔÁÔÓÈÎfiÙËÙ·.
∏ ·ÓÔÛÔÊ·ÈÓÔÙ˘È΋ ·Ó¿Ï˘ÛË ¤‰ÂÈÍ fiÙÈ Ù·
CD34+CD38- Î·È CD34+CD33-, Ô˘ ·ÔÙÂÏÔ‡Ó ˘Ô-
ÏËı˘ÛÌÔ‡˜ ÚÔÁÔÓÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ,
·ÚÔ˘Û›·Û·Ó ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ¤ÎÊÚ·Û˘ ›-
Û˘ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· fiÔ˘ Ù· CD34+ ·ÈÌÔÔÈËÙÈο
Û˘Ó˘‹Ú¯·Ó Ì ª∫ ÌfiÓÔ. √È Chute et al. (27) ¤‰ÂÈ-
Í·Ó ÂȉÈÎfiÙÂÚ· fiÙÈ Ù· CD34+CD38- ÌÔÚÔ‡Ó, ·Áfi-
ÌÂÓ· Û ΢ÙÙ·ÚÈÎfi ·ÎÏÔ, Ó· ÂÎÙ˘¯ıÔ‡Ó in vitro. T·
·ÙÙ·Ú· Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙËÓ ¤ÎÙ˘ÍË ‰È·ÙË-
ÚÔ‡Ó ÙËÓ ¤ÎÊÚ·ÛË ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘, Ù· ÔÔ›·
Èı·ÓÒ˜ ¤¯Ô˘Ó ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÂÁηٿÛÙ·ÛË
ÙˆÓ ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ÌÔ-
ۯ‡̷ÙÔ˜ Î·È ÛÙËÓ ·ÔηٿÛÙ·ÛË Ù˘ ·ÈÌÔÔ›Ë-
Û˘. ∏ ȉÈfiÙËÙ· ·˘Ù‹ ÙˆÓ ª∫ ÌÔÚ› Ó· ·ÍÈÔÔÈË-
ı› ÎÏÈÓÈο ÛÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË.
∞Ó Î·È Ô Ì˯·ÓÈÛÌfi˜ ·ÏÏËÏ›‰Ú·Û˘ ÙˆÓ ª∫
Ì ٷ ÚÔÁÔÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ‰ÂÓ ¤¯ÂÈ ‰È-
¢ÎÚÈÓÈÛÙ› (18), Â›Ó·È ÁÓˆÛÙfi fiÙÈ Ù· ·ÙÙ·Ú·
ÛÙÚÒÌ·ÙÔ˜ ·Ú¿ÁÔ˘Ó ¤Ó· Û‡ÓÔÏÔ Î˘ÙÙ·ÚÔÎÈÓÒÓ,
ÌÂٷ͇ ÙˆÓ ÔÔ›ˆÓ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÔÈ SCF, IL-1,
IL-3, IL-6, IL-7, IL-11, IL-12, TPO, Flt-3L, G-CSF,
GM-CSF Î·È LIF (28,29). ∞fi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘
·ÚÔ‡Û·˜ ÌÂϤÙ˘ Ê·›ÓÂÙ·È fiÙÈ ÔÈ ‰‡Ô ‰È·ÊÔÚÂÙÈ-
ÎÔ› Û˘Ó‰˘·ÛÌÔ› ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Ô˘ ¯ÚË-
ÛÈÌÔÔÈ‹ıËÎ·Ó ‰ÂÓ ÚÔηÏÔ‡Ó ÙÔ ›‰ÈÔ ·ÔÙ¤ÏÂÛÌ·
Ì ÙÔ˘˜ ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÎÎÚ›ÓÔÓÙ·È
·fi Ù· ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ·. ™˘ÁÎÂÎÚÈ̤ӷ, ·-
ÚÔ˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ·Ú·ÙËÚ‹ıËÎÂ
·Ó¿Ù˘ÍË-‰È·ÊÔÚÔÔ›ËÛË Û ϤÔÓ ÒÚÈÌ· ÛÙÔÈ-
¯Â›· Ù˘ ηÏÏȤÚÁÂÈ·˜ (ÔÏÈÎfi˜ ·ÚÈıÌfi˜ ΢ÙÙ¿ÚˆÓ,
CD34+ Ì ÌÈÎÚfiÙÂÚË ÎψÓÔÁÔÓÈÎfiÙËÙ· Î.Ï.), ·ÓÂ-
Í¿ÚÙËÙ· ·fi ÙÔ Â¿Ó Ô Û˘Ó‰˘·ÛÌfi˜ ΢ÙÙ·ÚÔÎÈÓÒÓ
Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËΠÂȉڿ ΢ڛˆ˜ Û ·Ú¯¤ÁÔÓ·
‹ ÈÔ ÒÚÈÌ· ‰ÂÛÌÂ˘Ì¤Ó· ÚÔÁÔÓÈο ·ÈÌÔÔÈËÙÈο
·ÙÙ·Ú· (30).
™˘ÌÂÚ·ÛÌ·ÙÈο, Ù· ª∫ ÛÙÚÒÌ·ÙÔ˜ Ì˘ÂÏÔ‡ ÙˆÓ
ÔÛÙÒÓ ·È‰ÈÒÓ Ì ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù·
ÚÔ¿ÁÔ˘Ó ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚ· ÙËÓ ex vivo ¤ÎÙ˘ÍË
·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·fi ·›Ì· √§,
Û ۇÁÎÚÈÛË Ì ÙËÓ ¤ÎÙ˘ÍË ·ÚÔ˘Û›· ·˘ÍËÙÈÎÒÓ
·Ú·ÁfiÓÙˆÓ, ·Ô‰›‰ÔÓÙ·˜ ¤Ó·Ó ÏËı˘ÛÌfi Ì ¯·Ú·-
ÎÙËÚÈÛÙÈο ·ˆÚfiÙËÙ·˜ Î·È È‰ÈfiÙËÙ˜ ηٿÏÏËϘ ÁÈ·
ÙËÓ ·ÔηٿÛÙ·ÛË Ù˘ ·ÈÌÔÔ›ËÛ˘ ÌÂÙ¿ ·fi ÌÂ-
Ù·ÌfiÛ¯Â˘ÛË.
µÈ‚ÏÈÔÁÚ·Ê›·
1. Wynter EA, Emmerson AJ, Testa NG. Properties of
peripheral blood and cord blood stem cells. Baillieres’ Best
Pract Res Clin Haematol 1999;12:1-17.
2. Lewis ID, Verfaillie CM. Multi-lineage expansion potential
of primitive hematopoietic progenitors: superiority of
umbilical cord blood compared to mobilized peripheral
blood. Exp Hematol 2000;28:1087-1095.
3. Zhang J, Niu C, Ye L, Huang H, He X, Tong WG, et al.
Identification of the haematopoietic stem cell niche and
control of the niche size. Nature 2003;425:836-841.
4. Cohen Y, Nagler A. Umbilical cord blood transplantation:
how, when and for whom? Blood Rev 2004;18:167-179.
5. Cohen Y, Kreiser D, Mayorov M, Nagler A. Unrelated and
related cord blood banking and hematopoietic graft
engineering. Cell Tissue Bank 2003;4:29-35.
6. Gammaitoni L, Bruno S, Sanavio F, Gunetti M, Kollet O,
Cavalloni G, et al. Ex vivo expansion of human adult stem
cells capable of primary and secondary hemopoietic
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7. Kobari L, Pflumio F, Giarratana M, Li X, Titeux M, Izac B,
et al. In vitro and in vivo evidence for the long-term
multilineage (myeloid, B, NK, and T) reconstitution
capacity of ex vivo expanded human CD34+ cord blood
cells. Exp Hematol 2000;28:1470-1480.
8. Piacibello W, Sanavio F, Garetto L, Severino A, Dané A,
Gammaitoni L, et al. Differential growth factor
requirement of primitive cord blood hematopoietic stem
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9. Ueda T, Tsuji K, Yoshino H, Ebihara Y, Yagasaki H,
Hisakawa H, et al. Expansion of human NOD/ SCID-
repopulating cells by stem cell factor, Flk2/Flt3 ligand,
thrombopoietin, IL-6, and soluble IL-6 receptor. J Clin
Invest 2000;105:1013-1021.
10. Yamaguchi M, Hirayama F, Kanai M, Sato N, Fukazawa K,
115ªÂÛÂÁ¯˘Ì·ÙÈο Î·È ¤ÎÙ˘ÍË ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜
¶·È‰È·ÙÚÈ΋ 2009;72:115-000
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Yamashita K, et al. Serum-free co-culture system for exvivo expansion of human cord blood primitive progenitorsand SCID mouse-reconstituting cells using human bonemarrow primary stromal cells. Exp Hematol 2001;29:174-182.
11. Shih CC, Hu MC, Hu J, Medeiros J, Forman SJ. Long-termex vivo maintenance and expansion of transplantablehuman hematopoietic stem cells. Blood 1999;94:1623-1636.
12. Thiemann FT, Moore KA, Smogorzewska EM, LemischkaIR, Crooks GM. The murine stromal cell line AFT024 actsspecifically on human CD34-CD38- progenitors tomaintain primitive function and immunophenotype invitro. Exp Hematol 1998;26:612-619.
13. Kawada H, Ando K, Tsuji T, Shimakura Y, Nakamura Y,Chargui J, et al. Rapid ex vivo expansion of humanumbilical cord hematopoietic progenitors using a novelculture system. Exp Hematol 1999;27:904-915.
14. Woodbury D, Reynolds K, Black IB. Adult bone marrowstromal stem cells express germline, ectodermal,endodermal, and mesodermal genes prior to neurogenesis.J Neurosci Res 2002;69:908-917.
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19. Dimitriou H, Vorgia P, Stiakaki E, Mavroudis D, MarkakiEA, Koumantakis E, et al. In vitro proliferative anddifferentiating characteristics of CD133(+) and CD34(+)cord blood cells in the presence of thrombopoietin (TPO) orerythropoietin (EPO). Potential implications for hemato-poietic cell transplantation. Leuk Res. 2003;27:1143-1151.
20. Kögler G, Nürnberger W, Fischer J, Niehues T, Somville T,
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24. Wexler SA, Donaldson C, Denning-Kendall P, Rice C,Bradley B, Hows JM. Adult bone marrow is a rich source ofhuman mesenchymal stem cells but umbilical cord andmobilized adult blood are not. Br J Haematol 2003;121:368-374.
25. Yu M, Xiao Z, Shen L, Li L. Mid-trimester fetal blood-derived adherent cells share characteristics similar tomesenchymal stem cells but full-term umbilical cord blooddoes not. Br J Haematol 2004;124:666-675.
26. Mareschi K, Biasin E, Piacibello W, Aglietta M, Madon E,Fagioli F. Isolation of human mesenchymal stem cells:bone marrow versus umbilical cord blood. Haematologica2001;86:1099-1100.
27. Chute JP, Saini AA, Kampen RL, Wells MR, Davis TA. Acomparative study of the cell cycle status and primitive celladhesion molecule profile of human CD34+ cells culturedin stromal-free versus porcine microvascular endothelialcell cultures. Exp Hematol 1999;27:370-379.
28. Haynesworth SE, Baber MA, Caplan AI. Cytokineexpression by human marrow-derived mesenchymalprogenitor cells in vitro: effects of dexamethasone and IL-1alpha. J Cell Physiol 1996;166:585-592.
29. Dormady SP, Bashayan O, Dougherty R, Zhang XM, BaschRS. Immortalized multipotential mesenchymal cells andthe hematopoietic microenvironment. J Hematother StemCell Res 2001;10:125-140.
30. Ueda T, Tsuji K, Yoshino H, Ebihara Y, Yagasaki H,Hisakawa H, et al. Expansion of human NOD/SCID-repopulating cells by stem cell factor, Flk2/Flt3 ligand,thrombopoietin, IL-6, and soluble IL-6 receptor. J ClinInvest 2000;105:1013-1021.
116 π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:116-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·116
¶·ÓÂÏÏ‹ÓÈ· ÔÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞ Û ·È‰È¿
ËÏÈΛ·˜ 0-14 ÂÙÒÓ*
µ. ¶··Â˘·ÁÁ¤ÏÔ˘1, ∞. ∫‡Úη1, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜2, ¢. ∫·Û›ÌÔ˜3, ∫. ¶·ÓÙÂÏ¿ÎË4, ∂. ¶·‡ÏÔ˘2, ª. ∆˙Ô‡ÊË5,
ª. ª·˘ÚÔÎÒÛÙ·6, ∞. ∆˙È‚¿Ú·˜4, •. ¶Â‰¤ÏË7, ∞. ÷Ù˙ËÌȯ·‹Ï3, º. ∞ı·Ó·ÛÈ¿‰Ô˘2, ¢. ∫·ÊÂÙ˙‹˜1,
∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜7
¶ÂÚ›ÏË„Ë
∂ÈÛ·ÁˆÁ‹: ∏ Ë·Ù›Ùȉ· ∞ ·ÔÙÂÏ› ÂÓ‰ËÌÈÎfi ÓfiÛËÌ· Û ÔÏϤ˜ ÂÚÈÔ¯¤˜ ÙÔ˘ ÎfiÛÌÔ˘. ∏ ∂ÏÏ¿‰· ηٷ-Ù¿ÛÛÂÙ·È ÛÙȘ ¯ÒÚ˜ ÂӉȿÌÂÛ˘ ÂÓ‰ËÌÈÎfiÙËÙ·˜, ·ÏÏ¿ ‰ÂÓ ˘¿Ú¯Ô˘Ó ·Ú΋ ÚfiÛÊ·Ù· ÔÚÔÂȉËÌÈÔ-ÏÔÁÈο ‰Â‰Ô̤ӷ ÁÈ· ÙË ÓfiÛÔ. ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ·ÍÈÔÏfiÁËÛË Ù˘ ÔÚÔÂȉËÌÈÔÏÔÁ›·˜ÙÔ˘ ÈÔ‡ Ù˘ Ë·Ù›Ùȉ·˜ ∞, ·Ó¿ ËÏÈΛ· Î·È ÁˆÁÚ·ÊÈÎfi ‰È·Ì¤ÚÈÛÌ· Ù˘ ¯ÒÚ·˜, Û ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›Á-Ì· ·È‰ÈÒÓ ËÏÈΛ·˜ 0-14 ÂÙÒÓ.
ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ: ™˘ÏϤ¯ıËÎ·Ó 100 ‰Â›ÁÌ·Ù· ÔÚÒÓ ·Ó¿ ËÏÈÎȷ΋ ÔÌ¿‰·, ‚¿ÛÂÈ ·ÔÁÚ·ÊÈÎÒÓ ‰Â‰Ô-̤ӈÓ, ·fi fiÏ· Ù· ÁˆÁÚ·ÊÈο ‰È·ÌÂÚ›ÛÌ·Ù· Ù˘ ¯ÒÚ·˜, Û‡Ìʈӷ Ì ÙÔ Û¯‹Ì· Û˘ÏÏÔÁ‹˜ ÂÓ·ÔÌÂÈ-Ó¿ÓÙˆÓ ÔÚÒÓ Ô˘ ¯ÚËÛÈÌÔÔÈ› ÙÔ Â˘Úˆ·˚Îfi ‰›ÎÙ˘Ô ÔÚÔÂÈÙ‹ÚËÛ˘ ÓÔÛËÌ¿ÙˆÓ Ô˘ ÚÔÏ·Ì‚¿ÓÔÓÙ·ÈÌ ÂÌ‚ÔÏÈ·ÛÌfi (∂SEN II), ÛÙÔ˘˜ ÔÔ›Ô˘˜ ¤ÁÈÓ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ IgG ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ Ù˘˷ٛÙȉ·˜ ∞ (AXSYM, Abbott). ∫·Ù·ÁÚ¿ÊËÎ·Ó Î·È ·Ó·Ï‡ıËÎ·Ó Ù· ÛÙÔȯ›· 1.393 ·È‰ÈÒÓ.
∞ÔÙÂϤÛÌ·Ù·: ∆· ·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ, ηıÒ˜ Î·È Ù· ·È‰È¿ Ô˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ·ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜, ›¯·ÓÛÙ·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›ËÛ˘. ™Ù· ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿, Ë ÔÚÔıÂÙÈÎfiÙËÙ··Ó‹Ïı ÛÙÔ 17,07%. ™Ù· ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ Î·Ù·ÁÚ¿ÊËΠ˘„ËÏfiÙÂÚÔ ÔÛÔÛÙfi ÔÚÔıÂÙÈ-ÎfiÙËÙ·˜ ÏfiÁˆ Ê˘ÛÈ΋˜ ÓfiÛËÛ˘, Û ۇÁÎÚÈÛË Ì ٷ ∂ÏÏËÓfiÔ˘Ï· (22,38% ¤Ó·ÓÙÈ 15,9%, OR=1,52, p=0,064).∂›Û˘, ηٷÁÚ¿ÊËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ‰È·ÊÔÚÂÙÈÎÒÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ.
™˘ÌÂÚ¿ÛÌ·Ù·: ∞fi Ù· ÛÙÔȯ›· Ù˘ ÌÂϤÙ˘ Ì·˜ ÚÔ·ÙÂÈ fiÙÈ ÂÈ‚¿ÏÏÂÙ·È Ë ÂÊ·ÚÌÔÁ‹ ηıÔÏÈÎÔ‡ ÂÌ-‚ÔÏÈ·ÛÌÔ‡ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ ÛÙË ¯ÒÚ· Ì·˜.
§¤ÍÂȘ ÎÏÂȉȿ: ∏·Ù›Ùȉ· ∞, ÂÌ‚fiÏÈÔ, ÔÚÔÂȉËÌÈÔÏÔÁÈ΋.
Seroepidemiological study of hepatitis A in Greek children
aged 0-14 years
V. Papaevagelou1, A. Kyrka1, A. Tragiannidis2, D. Kassimos3, K. Pantelaki4, E. Pavlou2, M. Tzoufi5,
M. Mavrokosta6, A. Tzivaras4, X. Pedeli7, A. Hatzimichail3, F. Athanassiadou2, D. Kafetzis1,
A. Konstantopoulos7
Abstract
Background: Hepatitis A is a disease endemic in many areas of the world. Greece is considered a countryof intermediate endemicity, but recent data are lacking. A national cross-sectional seroprevalence surveywas conducted to evaluate the current seroepidemiology of hepatitis A in children in the age group 0-14years living in Greece.
Methods: Using the ESSEN II methodology, 100 sera per year of age, stratified by geographic region, werecollected and tested for the presence of anti-HAV IgG antibodies (AXSYM, Abbott Laboratories). Data from1,393 children were analyzed.
Results: Immigrant children and children residing in rural areas were significantly less likely to beimmunized. Among unvaccinated children, the seroprevalence was 17.07%. Nationality had a marginallysignificant effect, since nonimmunized immigrant children had a higher seroprevalence rate (22.38%versus 15.9%, OR=1.52, p=0.064). Significant differences were observed between geographic areas inboth vaccination coverage and natural immunity.
Conclusions: These data indicate that hepatitis A continues to prevail in Greece and therefore universalinfant immunization should be considered.
Key words: Hepatitis A, vaccine, seroepidemiology.
117∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞ ORIGINAL ARTICLE
1 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
2 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞.¶.£, ¡ÔÛÔÎÔÌ›Ô∞Ã∂¶∞, £ÂÛÛ·ÏÔÓ›ÎË
3 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¢.¶.£, ∞ÏÂÍ·Ó‰ÚÔ‡ÔÏË
4 ∂ÚÁ·ÛÙ‹ÚÈÔ ∞ÈÌÔ‰ÔÛ›·˜,°.¡. ¶·›‰ˆÓ «∞. ™ÔÊ›·»,∞ı‹Ó·
5 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ πˆ·ÓÓ›ÓˆÓ
6 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,«µÂÓÈ˙¤ÏÂÈÔ» ¡ÔÛÔÎÔÌ›Ô∏Ú·ÎÏ›Ԣ
7 ™¯ÔÏ‹ ÀÁÈÂÈÓ‹˜ ηÈ∂ȉËÌÈÔÏÔÁ›·˜ ∂∫¶∞
AÏÏËÏÔÁÚ·Ê›·:
µ·ÛÈÏÈ΋ ¶··Â˘·ÁÁ¤ÏÔ˘[email protected], [email protected] µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ«¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
1 2nd Department ofPaediatrics, GeneralChildren’s Hospital “P. & A. Kyriakou”,University of Athens, Athens, Greece
2 2nd Department ofPaediatrics, “AHEPA”Hospital, “Aristotelio”University of Thessaloniki,Thessaloniki, Greece
3 Department of Paediatrics,“Dimokritio” University ofThrace, Alexandroupolis,Greece
4 Laboratory for BloodDonation, “Agia Sophia”General Children’s Hospital,Athens, Greece
5 Department of Paediatrics,University of Ioannina,Ioannina, Greece
6 Department of Paediatrics,“Venizelio” Hospital ofHeraklion, Heraklion, Greece
7 School of Hygiene andEpidemiology, EKPA
Correspondence:
Vassiliki Papaevagelou [email protected],[email protected] 2nd Department ofPaediatrics, General Children’sHospital “P. & A. Kyriakou”,University of Athens, Athens,Greece
¶·È‰È·ÙÚÈ΋ 2009;72:117-000
* ∏ ÂÚÁ·Û›· ·˘Ù‹ ‚Ú·‚‡ıËΠ̠ÙÔ Œ·ıÏÔ ¢ÔÍÈ¿‰Ë, ηٿ ÙÔ 46Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ∫¤Ú΢ڷ 2008
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·117
∂ÈÛ·ÁˆÁ‹
∏ Ë·Ù›Ùȉ· ∞ ·ÔÙÂÏ› Û˘¯Ófi ÏÔÈÌ҉˜ ÓfiÛË-
Ì·, ·Ú¿ ÙËÓ Î˘ÎÏÔÊÔÚ›· ·ÔÙÂÏÂÛÌ·ÙÈÎÔ‡ ÂÌ‚ÔÏ›-
Ô˘ (1). ∞Ó Î·È Ù· ÔÛÔÛÙ¿ Ù˘ ›وۋ˜ Ù˘ ¤¯Ô˘Ó
ÌÂȈı› ÛËÌ·ÓÙÈο ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÔËÁÔ‡-
ÌÂÓˆÓ ‰ÂηÂÙÈÒÓ ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ·Ó·Ù˘Á̤Ó˜
¯ÒÚ˜, ÛËÌ·ÓÙÈ΋ ·ÏÏ·Á‹ ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ¤¯ÂÈ
·Ú·ÙËÚËı› ÛÙȘ ÂÚÈÔ¯¤˜ fiÔ˘ ÂÊ·ÚÌfiÛÙËΠη-
ıÔÏÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ (1-3). ™ÙËÓ ∂ÏÏ¿‰· ˘¿Ú¯Ô˘Ó
ÛÙÔȯ›· ·fi ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Û ÔÚÈṲ̂Ó˜
ÂÚÈÔ¯¤˜ Ù˘ ¯ÒÚ·˜ Ô˘ ·Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Ù· ÔÛÔ-
ÛÙ¿ ›وÛ˘ Ù˘ ÓfiÛÔ˘ ¤¯Ô˘Ó ÌÂȈı› ÛËÌ·ÓÙÈο
Î·È Ë Ì¤ÛË ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ Ì ˷ٛÙȉ· ∞ ¤¯ÂÈ
·˘ÍËı›, fiˆ˜ ÂÚÈÁÚ¿ÊÂÙ·È Î·È Û ¿ÏϘ Â˘Úˆ·˚-
Τ˜ ¯ÒÚ˜ (4-9). ∂ÓÙÔ‡ÙÔȘ, ÂıÓÈο ÔÚÔÂȉËÌÈÔÏÔ-
ÁÈο ÛÙÔȯ›· ‰ÂÓ Â›Ó·È ‰È·ı¤ÛÈÌ· ̤¯ÚÈ Û‹ÌÂÚ·, ·Ó
Î·È Ë ÔÍ›· Ë·Ù›Ùȉ· ∞ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙȘ ·Ûı¤-
ÓÂȘ Ô˘ Ú¤ÂÈ Ó· ‰ËÏÒÓÔÓÙ·È ˘Ô¯ÚˆÙÈο ÛÙÔ
∂ÏÏËÓÈÎfi ∫¤ÓÙÚÔ ∂ϤÁ¯Ô˘ Î·È ¶ÚfiÏ˄˘ ¡ÔÛËÌ¿-
ÙˆÓ (∫∂.∂§.¶.¡√).
°È· ÙËÓ ÂÈÛ·ÁˆÁ‹ ÂÓfi˜ ÂÌ‚ÔÏ›Ô˘ ÛÙÔ˘˜ ··Ú·›-
ÙËÙÔ˘˜ ÂÌ‚ÔÏÈ·ÛÌÔ‡˜ ÎÚ›ÓÂÙ·È ÛÎfiÈÌË Ë Û˘ÏÏÔÁ‹
‰Â‰ÔÌ¤ÓˆÓ ÁÈ· ÙÔÓ ÂÈÔÏ·ÛÌfi Ù˘ ÓfiÛÔ˘ Û ÌÈ·
ÏËı˘ÛÌȷ΋ ÔÌ¿‰·, ηıÒ˜ Î·È Ë Î·Ù·ÁÚ·Ê‹ ÙˆÓ
ÂÈÏÔÎÒÓ Ù˘. ¢˘ÛÙ˘¯Ò˜, Ù· ‰Â‰Ô̤ӷ ·fi ÙÔÓ ÂÏ-
ÏËÓÈÎfi ¯ÒÚÔ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÛÙÔȯ›· Û ÙÔÈÎfi ΢-
Ú›ˆ˜ Â›Â‰Ô Î·È ÂӉ¯Ô̤ӈ˜ ı· ÌÔÚÔ‡Û ӷ Á›-
ÓÂÈ ÂÎÙ›ÌËÛË Ù˘ ÓfiÛÔ˘ ÌfiÓÔ Ì ·Ó·‰ÚÔÌÈ΋ Û˘ÏÏÔ-
Á‹, ‚¿ÛÂÈ ÛÙÔȯ›ˆÓ ÙˆÓ ÂÈÛ·ÁˆÁÒÓ Û ·È‰È·ÙÚÈΤ˜
ÎÏÈÓÈΤ˜.
™ÎÔfi˜, ÂÔ̤ӈ˜, Ù˘ ·ÚÔ‡Û·˜ ÔÚÔÂȉËÌÈÔ-
ÏÔÁÈ΋˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ·ÔÙ‡ˆÛË Ù˘ ·ÓÔÛ›·˜
¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞, ·Ó¿ ËÏÈΛ· Î·È ÁˆÁÚ·ÊÈÎfi
‰È·Ì¤ÚÈÛÌ· Ù˘ ¯ÒÚ·˜, Û ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›Á-
Ì· ·È‰ÈÒÓ 0-14 ÂÙÒÓ ·fi fiÏË ÙËÓ ∂ÏÏ¿‰·. ∆· ·Ô-
ÙÂϤÛÌ·Ù· Ù¤ÙÔÈ·˜ ÌÂϤÙ˘ ı· ·Ú¿Û¯Ô˘Ó ÂӉ¯Ô-
̤ӈ˜ ¯Ú‹ÛÈÌ· ÛÙÔȯ›· ÛÙËÓ ∂ıÓÈ΋ ∂ÈÙÚÔ‹
∂Ì‚ÔÏÈ·ÛÌÒÓ.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ
∞ÛıÂÓ›˜
√Úfi˜ ·›Ì·ÙÔ˜ ·ÓÙÈÚÔÛˆÂ˘ÙÈÎÔ‡ ‰Â›ÁÌ·ÙÔ˜ ·È‰ÈÒÓ 0-
14 ÂÙÒÓ ·fi fiÏË ÙËÓ ∂ÏÏ¿‰· ÂÍÂÙ¿ÛÙËΠÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË
·ÓÙÈ-HAV ·ÓÙÈۈ̿وÓ. ™˘ÓÔÏÈο, ¤ÁÈÓÂ Û˘ÏÏÔÁ‹ 100 ÔÚÒÓ
ÁÈ· οı ËÏÈÎȷ΋ ÔÌ¿‰· Û ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÂÓfi˜ ¤ÙÔ˘˜,
Û‡Ìʈӷ Ì ÙÔ Û‡ÛÙËÌ· Ù˘ Û˘ÏÏÔÁ‹˜ ÂÓ·ÔÌÂÈÓ¿ÓÙˆÓ ÔÚÒÓ
Ô˘ ¯ÚËÛÈÌÔÔÈ› ÙÔ Â˘Úˆ·˚Îfi ‰›ÎÙ˘Ô ÔÚÔÂÈÙ‹ÚËÛ˘ ÙˆÓ
ÓÔÛËÌ¿ÙˆÓ Ô˘ ÚÔÏ·Ì‚¿ÓÔÓÙ·È ÌÂ ÂÌ‚fiÏÈ· (∂SEN II), ÌÂ
ÛÎÔfi Ó· ¤¯Ô˘ÌÂ Û˘ÁÎÚ›ÛÈÌ· ·ÔÙÂϤÛÌ·Ù· Ì ·ÓÙ›ÛÙÔȯ˜
Â˘Úˆ·˚Τ˜ ÌÂϤÙ˜. ∆Ô ÔÛÔÛÙfi ÙˆÓ ·È‰ÈÒÓ ·fi οı Áˆ-
ÁÚ·ÊÈ΋ ÂÚÈÔ¯‹ ˘ÔÏÔÁ›ÛÙËΠ̠‚¿ÛË ÙËÓ ÂıÓÈ΋ ·ÔÁÚ·-
Ê‹ ÙÔ˘ 2001. ∂ÈϤÔÓ, ·fi ÙË ÌÂϤÙË ·ÔÎÏ›ÛÙËÎ·Ó Ù·
·È‰È¿ Ì ¯ÚfiÓÈ· ÓÔÛ‹Ì·Ù·, ÚˆÙÔ·ı›˜, ‰Â˘ÙÂÚÔ·ı›˜
·ÓÔÛÔ·Ó¿ÚÎÂȘ, ηıÒ˜ Î·È ÂΛӷ Ì ÚfiÛÊ·ÙË Ï‹„Ë ÎÔÈ-
Ó‹˜ Á-ÛÊ·ÈÚ›Ó˘. ∆· ‰Â›ÁÌ·Ù· ÙˆÓ ÔÚÒÓ ÛÙ¿ÏıËÎ·Ó Ù·¯˘‰ÚÔ-
ÌÈο ÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∞Á›· ™ÔÊ›·». ∆ËÚ‹ıËΠÙÔ
·fiÚÚËÙÔ ÙˆÓ ÚÔÛˆÈÎÒÓ ‰Â‰ÔÌ¤ÓˆÓ ÙˆÓ ·ÛıÂÓÒÓ Ô˘
Û˘ÌÌÂÙ›¯·Ó ÛÙË ÌÂϤÙË. ∆· ‰Â›ÁÌ·Ù· ‰È·ÙËÚ‹ıËÎ·Ó Û ıÂÚ-
ÌÔÎÚ·Û›· -20ÔC ̤¯ÚÈ ÙËÓ Âͤٷۋ ÙÔ˘˜. ∆· ‰Â‰Ô̤ӷ ÙˆÓ
·ÛıÂÓÒÓ ÂÈÛ‹¯ıËÛ·Ó Û ÂȉÈο ‰È·ÌÔÚʈ̤ÓË ‚¿ÛË ‰Â‰Ô̤-
ÓˆÓ Ì Έ‰ÈÎfi ·ÚÈıÌfi, ÒÛÙ ӷ ÂÍ·ÛÊ·ÏÈÛÙ› ·ÊÂÓfi˜ Ë ·Óˆ-
Ó˘Ì›· Î·È ·ÊÂÙ¤ÚÔ˘ Ë Ï‹Ú˘ ÚÔÛÙ·Û›· ÙˆÓ ÚÔÛˆÈÎÒÓ
‰Â‰ÔÌ¤ÓˆÓ Î¿ı ·ÛıÂÓÔ‡˜. °È· οı ·È‰› ¤ÁÈÓ ηٷÁÚ·Ê‹
Ù˘ ËÏÈΛ·˜, ÙÔ˘ ʇÏÔ˘, ÙÔ˘ ÙfiÔ˘ ‰È·ÌÔÓ‹˜, Ù˘ ˘ËÎÔfiÙË-
Ù·˜ Î·È Ù˘ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ ÙÔ˘ Î¿Ï˘„˘. ∆· ‰Â›ÁÌ·Ù· ÔÚÒÓ
ÂÍÂÙ¿ÛÙËÎ·Ó ÁÈ· ÙËÓ ·ÚÔ˘Û›· ·ÓÙÈÛˆÌ¿ÙˆÓ ·ÓÙÈ-HAV IgG
ÌÂ ¯Ú‹ÛË ÙÔ˘ ÙÂÛÙ HAVAB 2.0 (AXSYM, Abbott).
ŸÛÔÓ ·ÊÔÚ¿ ÛÙ· ‚Ú¤ÊË ËÏÈΛ·˜ <12 ÌËÓÒÓ, Ë ·Ó‡ÚÂÛË
ıÂÙÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ·Ô‰fiıËΠ۠·ıËÙÈ΋ ·ÓÔÛ›· ÏfiÁˆ
ÌËÙÚÈÎÒÓ ·ÓÙÈۈ̿وÓ.
™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË
∏ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ¤ÁÈÓ Ì ÙË ¯Ú‹ÛË ÙÔ˘ ÏÔÁÈÛÌÈÎÔ‡
·Î¤ÙÔ˘ Stata (version 8) Î·È ˆ˜ fiÚÈÔ ÛÙ·ÙÈÛÙÈ΋˜ ÛËÌ·ÓÙÈ-
ÎfiÙËÙ·˜ ıˆڋıËÎÂ Ë ÙÈÌ‹ p<0,05. ªÂ ¯Ú‹ÛË ÌÔÓÔÌÂÙ·‚ÏË-
Ù‹˜ ÏÔÁÈÛÙÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘, ÂϤÁÍ·Ì ÙËÓ Â›‰Ú·ÛË Ù˘
ËÏÈΛ·˜, Ù˘ ÂıÓÈÎfiÙËÙ·˜ Î·È ÙÔ˘ ÙfiÔ˘ ‰È·ÌÔÓ‹˜ ÙˆÓ ·ÛıÂ-
ÓÒÓ ÛÙËÓ Â›ÙˆÛË Ù˘ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ ÙÔ˘˜ Î¿Ï˘„˘, ·ÏÏ¿
Î·È Ù˘ Ê˘ÛÈ΋˜ ·ÓÔÛ›·˜ Û ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿. ∆·
·ÔÙÂϤÛÌ·Ù· ÂÎÊÚ¿ÛÙËÎ·Ó Ì Odds Ratio (OR) Î·È 95%
‰È¿ÛÙËÌ· ·ÍÈÔÈÛÙ›·˜. ∏ ÛÙ·ÙÈÛÙÈ΋ ÛËÌ·ÓÙÈÎfiÙËÙ· (p-
value) ˘ÔÏÔÁ›ÛÙËΠ̠ÙÔ Wald test.
∞ÔÙÂϤÛÌ·Ù·
∏ Û˘ÏÏÔÁ‹ ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ Ù˘ ÌÂϤÙ˘ ¤ÁÈÓ ÌÂ-ٷ͇ ÙÔ˘ π·ÓÔ˘·Ú›Ô˘ 2006 Î·È ÙÔ˘ ¢ÂÎÂÌ‚Ú›Ô˘ 2006,Û‡Ìʈӷ Ì ÙË ÌÂıÔ‰ÔÏÔÁ›· Ù˘ ÌÂϤÙ˘, ·fi ÙȘ‰È¿ÊÔÚ˜ ÂÚÈÔ¯¤˜ Ù˘ ∂ÏÏ¿‰·˜. ∞fi ÙËÓ ·Ó¿Ï˘ÛËÙˆÓ ‰ËÌÔÁÚ·ÊÈÎÒÓ ÛÙÔȯ›ˆÓ 1.393 ‰ÂÈÁÌ¿ÙˆÓ ÚÔ-¤Î˘„ fiÙÈ Ù· ·È‰È¿ ·fi ÔÈÎÔÁ¤ÓÂȘ ÌÂÙ·Ó·ÛÙÒÓ·ÓÙÈÚÔÛÒ¢·Ó ÙÔ 12,6% (175/1383), ÂÓÒ Û‡Ìʈ-Ó· Ì ÚfiÛÊ·Ù· ·ÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ (2001), ÔÈÌÂÙ·Ó¿ÛÙ˜ ·ÔÙÂÏÔ‡Ó ÂÚ›Ô˘ ÙÔ 10% ÙÔ˘ ÏËı˘-ÛÌÔ‡ Ù˘ ¯ÒÚ·˜ Ì·˜. ™˘ÓÔÏÈο ·Ó·Ï‡ıËÎ·Ó 1.374‰Â›ÁÌ·Ù· ·›Ì·ÙÔ˜, ·ÊÔ‡ ·fi ÙÔ Û‡ÓÔÏÔ ÙˆÓ 1.393‰ÂÈÁÌ¿ÙˆÓ Ù· 19 ‹Ù·Ó ·ÈÌÔÏ˘Ì¤Ó· Î·È ÂÔ̤ӈ˜·ÔÎÏ›ÛıËÎ·Ó ·fi ÙË ÌÂϤÙË.
∞fi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ Ì·˜ ÚԤ΢-„ fiÙÈ ÙÔ 38% (438/1152) ÙˆÓ ·È‰ÈÒÓ ËÏÈΛ·˜ >24ÌËÓÒÓ Â›¯Â ÂÌ‚ÔÏÈ·ÛÙ› ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞. ∆ÔÔÛÔÛÙfi ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ Î¿Ï˘„˘ (·ÓÔÛ›·˜) ÛÙ··È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ ‹Ù·Ó ÛÙ·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ·-ÌËÏfiÙÂÚÔ (22,3%, 35/157), Û ۯ¤ÛË Ì ÙÔ ·ÓÙ›ÛÙÔÈ-¯Ô ÙˆÓ EÏÏËÓfiÔ˘ÏˆÓ (40,5%, 403/995) (OR=0,42,95%CI: 0,28-0.63, p<0,001) (∂ÈÎfiÓ· 1). ∂ÈϤÔÓ, Ù··È‰È¿ Ô˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ·ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜ÂÌÊ¿ÓÈ˙·Ó ¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ ο-Ï˘„˘ (·ÓÔÛ›·˜), Û ۇÁÎÚÈÛË Ì ٷ ·ÓÙ›ÛÙÔȯ··ÛÙÈÎÒÓ ÂÚÈÔ¯ÒÓ (31,04% ¤Ó·ÓÙÈ 40,88%,OR=1,54, 95% CI: 1,17-2,01, p=0,002) (∂ÈÎfiÓ· 1).
™Ù· ·ÓÔÛÔÔÈË̤ӷ ·È‰È¿, Ë ÔÚÔıÂÙÈÎfiÙËÙ··Ó‹Ïı ÛÙÔ 90,1% (393/436) ÁÈ· ·È‰È¿ Ô˘ ¤Ï·‚·Ó
118 µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.
Paediatriki 2009;72:118-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·118
Ì›· ‰fiÛË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ Î·È ÛÙÔ 91,2% (323/354) ÁÈ·
ÂΛӷ Ô˘ ¤Ï·‚·Ó 2 ‰fiÛÂȘ. ∞fi Ù· 131 ‚Ú¤ÊË ËÏÈ-
Λ·˜ 0-12 ÌËÓÒÓ Ù˘ ÌÂϤÙ˘, 41 (31,29%) ‹Ù·Ó ÔÚÔ-
ıÂÙÈο, ÁÂÁÔÓfi˜ ÂÓ‰ÂÈÎÙÈÎfi ·ıËÙÈ΋˜ ·ÓÔÛ›·˜ ÏfiÁˆ
ÌËÙÚÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞.
Ÿˆ˜ ‹Ù·Ó ·Ó·ÌÂÓfiÌÂÓÔ, Ù· ·È‰È¿ ËÏÈΛ·˜ <12 ÌË-
ÓÒÓ Ô˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ÔÈÎÔÁ¤ÓÂȘ ÌÂÙ·Ó·ÛÙÒÓ
›¯·Ó ÛÙ·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿
ÔÚÔıÂÙÈÎfiÙËÙ·˜ (23/41, 56,1%), Û ۇÁÎÚÈÛË Ì ÙÔ
·ÓÙ›ÛÙÔÈ¯Ô ÔÛÔÛÙfi ÙˆÓ ∂ÏÏËÓfiÔ˘ÏˆÓ (18/90,
20%) (OR=5,11, 95% CI: 2,29-11,43, p<0,001).
∞fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ fiÛÔÓ
·ÊÔÚ¿ ÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë ¤Ó·ÓÙÈ Ù˘ Ë·Ù›-
Ùȉ·˜ ∞ ·Ó¿ ÁˆÁÚ·ÊÈ΋ ÂÚÈÔ¯‹, ÚԤ΢„·Ó ˘„Ë-
Ï¿ ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›ËÛ˘ Û ∂ÏÏËÓfiÔ˘Ï· ÚÔÂÚ-
¯fiÌÂÓ· ·fi ÙËÓ ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· (54,07%), ÂÓÒ Ù·
¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ηٷÁÚ¿ÊËÎ·Ó ÛÙËÓ ÂÚÈÔ¯‹
Ù˘ µfiÚÂÈ·˜ ∂ÏÏ¿‰·˜ (21,82%). ∆· ÔÛÔÛÙ¿ ·ÓÔÛÔ-
Ô›ËÛ˘ ∂ÏÏËÓfiÔ˘ÏˆÓ ‰È¤ÊÂÚ·Ó ÛËÌ·ÓÙÈο ÌÂÙ·-
͇ ÙˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ, fiˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ
¶›Ó·Î· 1.
∆Ô ÔÛÔÛÙfi ÔÚÔıÂÙÈÎfiÙËÙ·˜ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›ÙÈ-
‰·˜ ∞ Û ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰ÈÒÓ ËÏÈΛ·˜ >12 ÌË-
ÓÒÓ, ÏfiÁˆ Ê˘ÛÈ΋˜ ÓfiÛËÛ˘, ·Ó‹Ïı Û 17,07%
(136/797). ∆Ô ÔÛÔÛÙfi ÙˆÓ ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ÔÚÔ-
ıÂÙÈÎÒÓ ∂ÏÏËÓfiÔ˘ÏˆÓ ‹Ù·Ó ¯·ÌËÏfiÙÂÚÔ (15,90%,
104/654), Û ۇÁÎÚÈÛË Ì ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô ÛÙ· ·È‰È¿
ÌÂÙ·Ó·ÛÙÒÓ (32/143, 22,38%) (OR=1,52, 95% CI:
0,98-2,38, p=0,064), ÂÓÒ ÛÙÔÓ ¶›Ó·Î· 2 Ê·›ÓÔÓÙ·È Ù·
·ÓÙ›ÛÙÔȯ· ÔÛÔÛÙ¿ ·Ó¿ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· ηÈ
ÁˆÁÚ·ÊÈÎfi ‰È·Ì¤ÚÈÛÌ· Ù˘ ¯ÒÚ·˜.
∞fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ù˘ ÌÂϤÙ˘
Ì·˜ ·Ó¿ ÏËı˘ÛÌȷ΋ ÔÌ¿‰·, ηٷÁÚ¿ÊËΠÛÙ·ÙÈ-
ÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ·ÓÔÛ›·
ÏfiÁˆ ÓfiÛËÛ˘ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ ÌfiÓÔ ÁÈ·
·È‰È¿ ·fi ÙËÓ ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰·, Ô˘ ·ÓÙÈÚÔÛˆ-
‡ÂÈ Î·È ÙËÓ ÂÚÈÔ¯‹ Ì ÙË ÌÂÁ·Ï‡ÙÂÚË ÂÌ‚ÔÏÈ·-
ÛÙÈ΋ Î¿Ï˘„Ë (9/191, 4,71% ¤Ó·ÓÙÈ 14/76, 18,42%)
(OR=4,57, 95% CI: 1,88-11,07, p=0,001) (¶›Ó·Î·˜
3). ∂›Û˘, ·ÍÈÔÛËÌ›ˆÙ· Â›Ó·È Ù· ˘„ËÏ¿ ÔÛÔÛÙ¿
Ê˘ÛÈ΋˜ ·ÓÔÛ›·˜ ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ·È‰ÈÒÓ ·fi
ÙËÓ ÂÚÈÔ¯‹ Ù˘ µfiÚÂÈ·˜ ∂ÏÏ¿‰·˜, ÙfiÛÔ ÁÈ· Ù·
¶›Ó·Î·˜ 1. ¢È·ÊÔÚ¤˜, ·Ó¿ÏÔÁ· Ì ÙÔÓ ÙfiÔ ‰È·ÌÔÓ‹˜ Î·È ÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞, ÌÂٷ͇ ∂ÏÏËÓfiÔ˘ÏˆÓ Î·È ÌÂÙ·Ó·ÛÙÒÓ
∂ÏÏËÓfiÔ˘Ï· ¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓN (%) OR ∆ÈÌ‹ p N (%) OR ∆ÈÌ‹ p
(95% CI) (95% CI)
∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· 226/418 Ref† 26/102 Ref†
(54,07%) (25,49%)µfiÚÂÈ· ∂ÏÏ¿‰· 79/362 0,24 <0,001 5/44 0,37 0,062
(21,82%) (0,17-0,32) (11,36%) (0,13-1,05)¶ÂÏÔfiÓÓËÛÔ˜ 108/294 0,49 <0,001 4/32 0,42 0,133Î·È ÓËÛÈ¿ (36,73%) (0,36-0,67) (12,50%) (0,13-1,30)™‡ÓÔÏÔ 413/1074 35/178
(38,45%) (19,66%)
† Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜
119√ÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞
¶·È‰È·ÙÚÈ΋ 2009;72:119-000
45
40
35
30
25
20
15
10
5
0
40
35
30
25
20
15
10
5
%p<0,001
∂ÏÏËÓfiÔ˘Ï· ¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ
40,5
22,3
p=0,002
∞ÛÙÈΤ˜ ÂÚÈÔ¯¤˜ ∞ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜
40,8
31
∂ÈÎfiÓ· 1. ¶ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›ËÛ˘ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ Û ·È‰È¿ ËÏÈΛ·˜ >24 ÌËÓÒÓ, Û ۯ¤ÛË Ì ÙËÓ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· Î·È ÙÔÓÙfiÔ ‰È·ÌÔÓ‹˜.
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·119
∂ÏÏËÓfiÔ˘Ï· fiÛÔ Î·È ÁÈ· Ù· ·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ
(26,98% Î·È 41,03% ·ÓÙ›ÛÙÔȯ·) (¶›Ó·Î·˜ 3).
™˘˙‹ÙËÛË
√ Èfi˜ Ë·Ù›Ùȉ·˜ ∞ (HAV) ÌÂÙ·‰›‰ÂÙ·È Ì ÙËÓ
ηٿÔÛË ÌÔÏ˘ÛÌ¤ÓˆÓ ÙÚÔÊ›ÌˆÓ ‹ ‡‰·ÙÔ˜. ∏ Ìfi-
Ï˘ÓÛË, fiˆ˜ ›Û˘ Î·È Ô ÂÌ‚ÔÏÈ·ÛÌfi˜, ·Ú¤¯Ô˘Ó
ÈÛfi‚È· ·ÓÔÛ›· (1,9). ∏ Èı·ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ Û˘-
ÌÙˆÌ¿ÙˆÓ ·fi ÙË ÌfiÏ˘ÓÛË Ì HAV Û˘Û¯ÂÙ›˙ÂÙ·È
Ì ÙËÓ ËÏÈΛ·, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Û ·È‰È¿ ÌÈÎÚfiÙÂÚ·
ÙˆÓ 6 ÂÙÒÓ ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ÌÔχÓÛÂȘ Â›Ó·È ·Û˘-
Ìو̷ÙÈΤ˜ ‹ ·ÓÈÎÙÂÚÈΤ˜ (1,2). ∆· ·È‰È¿ Ì ·Û˘-
Ìو̷ÙÈ΋ ÌfiÏ˘ÓÛË ÌÔÚÔ‡Ó Ó· ·ÔÙÂϤÛÔ˘Ó Ë-
Á‹ ÌfiÏ˘ÓÛ˘ ÁÈ· Ù· ËÏÈÎȈ̤ӷ ¿ÙÔÌ· (3).
∆Ș ÙÂÏÂ˘Ù·›Â˜ ‰ÂηÂٛ˜, ÙÔ ÔÛÔÛÙfi ÙˆÓ ·Ùfi-
ÌˆÓ Ì ·ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ Ù˘ Ë·Ù›Ùȉ·˜ ∞
¤¯ÂÈ ÌÂȈı› ÛÙ· ÂÚÈÛÛfiÙÂÚ· ̤ÚË ÙÔ˘ ÎfiÛÌÔ˘ (8).
∂Ó ÙÔ‡ÙÔȘ, ÂÚ›Ô˘ 1,4 ÂηÙÔÌ̇ÚÈ· Ӥ˜ ÌÔχÓ-
ÛÂȘ ·fi ÙÔÓ Èfi ·Ó·Ì¤ÓÂÙ·È Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ·ÁÎÔ-
ÛÌ›ˆ˜ οı ¯ÚfiÓÔ (8). ¶·Ú¿ ÙË Ì›ˆÛË Ô˘ ηٷ-
ÁÚ¿ÊÂÙ·È ÛÙ· ÔÚÔÂȉËÌÈÔÏÔÁÈο ÔÛÔÛÙ¿, Ë Ë·Ù›-
Ùȉ· ∞ Û˘Ó¯›˙ÂÈ Ó· Â›Ó·È Ì›· ·fi ÙȘ Ôχ Û˘¯Ó¿
·Ó·ÊÂÚfiÌÂÓ˜ ·Ûı¤ÓÂȘ Ô˘ ÌÔÚÔ‡Ó Ó· ÚÔÏË-
ÊıÔ‡Ó Ì ÂÌ‚ÔÏÈ·ÛÌfi ·ÁÎÔÛÌ›ˆ˜ (8).
∏ ∂ÏÏ¿‰· ıˆÚÂ›Ù·È ¯ÒÚ· Ì ÂӉȿÌÂÛË ÂÓ‰ËÌÈ-
ÎfiÙËÙ·, ·ÚfiÏÔ Ô˘ ‰ÂÓ ¤¯Ô˘Ì ÚfiÛÊ·Ù· ‰Â‰Ô̤-
Ó·. ∫¿ÔȘ ÌÈÎÚ¤˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó Ì›ˆÛË ÙÔ˘
ÂÈÔÏ·ÛÌÔ‡ Ù˘ ÓfiÛÔ˘ ‹‰Ë ·fi ÙË ‰ÂηÂÙ›· ÙÔ˘
’70 (5,6). ™‡Ìʈӷ Ì ÙȘ ÌÂϤÙ˜ ·˘Ù¤˜, ÔÛÔÛÙfi
ÌÈÎÚfiÙÂÚÔ ·fi 10% ÙˆÓ ·È‰ÈÒÓ ·ÏÏ¿ ÌÂÁ·Ï‡ÙÂÚÔ
·fi 90% ÙˆÓ ÂÓËÏ›ÎˆÓ ¿Óˆ ÙˆÓ 45 ÂÙÒÓ ¤¯ÂÈ ·ÓÙÈ-
ÛÒÌ·Ù· ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ (5-7). ™Â ÌÈ· ·Ó·‰ÚÔÌÈ΋ ÌÂ-
ϤÙË Ô˘ ‰ÈÂÓÂÚÁ‹ıËΠÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ∂ȉÈÎÒÓ
¶·ı‹ÛÂˆÓ £ÂÛÛ·ÏÔӛ΢, ÌÂٷ͇ ÙˆÓ ÂÙÒÓ 1985-
1992 ηٷÁÚ¿ÊËΠÛÙ·ıÂÚ‹ Ì›ˆÛË ÙˆÓ ÂÈÛ·ÁˆÁÒÓ
(6). ∂ÈϤÔÓ, ÛËÌÂÈÒıËΠ·‡ÍËÛË Ù˘ ̤Û˘ ËÏÈ-
Λ·˜ ÙˆÓ ·ÛıÂÓÒÓ, ·fi 0-5 ¤ÙË ÙÔ 1985 Û 21-25 ¤ÙË
ÙÔ 1992 (6). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÚÔËÁÔ‡ÌÂÓ˘ ‰Â-
ηÂÙ›·˜, ˘‹ÚÍ ÌÂÁ¿ÏË ÏËı˘ÛÌȷ΋ ·ÏÏ·Á‹ ÛÙËÓ
∂ÏÏ¿‰·, ÏfiÁˆ Ù˘ ÂÈÛfi‰Ô˘ ÛÙË ¯ÒÚ· ÔÈÎÔÓÔÌÈÎÒÓ
ÚÔÛʇÁˆÓ ·fi ÙËÓ ∞Ó·ÙÔÏÈ΋ ∂˘ÚÒË Î·È Ù· µ·Ï-
οÓÈ·. ™‡Ìʈӷ Ì ÙËÓ ÚfiÛÊ·ÙË ·ÔÁÚ·Ê‹ ÙÔ˘
2001, ÔÈ ÌÂÙ·Ó¿ÛÙ˜ Û˘Óı¤ÙÔ˘Ó ÂÚ›Ô˘ ÙÔ 10% ÙÔ˘
ÏËı˘ÛÌÔ‡ Ù˘ ¯ÒÚ·˜. ™‡Ìʈӷ Ì ÙÔ˘˜ Frosner
Î·È Û˘Ó., ˘¿Ú¯Ô˘Ó ‰Â‰Ô̤ӷ fiÙÈ ÔÈ ÌÂÙ·Ó¿ÛÙ˜
¤¯Ô˘Ó ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ HAV ·ÓÙÈÛˆ-
Ì¿ÙˆÓ (10). ∆¤ÏÔ˜, ·Ó Î·È Ë Ë·Ù›Ùȉ· ∞ ·ÔÙÂÏ›
¤Ó· ·fi Ù· ÓÔÛ‹Ì·Ù· Ô˘ ‰ËÏÒÓÔÓÙ·È ˘Ô¯ÚˆÙÈο
¶›Ó·Î·˜ 3. ¢È·ÊÔÚ¤˜, ·Ó¿ÏÔÁ· Ì ÙËÓ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· Î·È ÙË Ê˘ÛÈ΋ ·ÓÔÛ›· ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ·È‰ÈÒÓ >12 ÌËÓÒÓ, ÌÂٷ͇ ÁˆÁÚ·-ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ
∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· µfiÚÂÈ· ∂ÏÏ¿‰· ¶ÂÏÔfiÓÓËÛÔ˜ Î·È ÓËÛÈ¿N (%) OR p N (%) OR p N (%) OR p
(95% CI) (95% CI) (95% CI)
∂ÏÏËÓfiÔ˘Ï· 9/191 Ref† 75/278 Ref† 20/185 Ref†
(4,71%) (26,98%) (10,81%)ªÂÙ·Ó¿ÛÙ˜ 14/76 4,57 0,001 16/39 1,88 0,073 2/28 0,63 0,555
(18,42%) (1,88-11,07) (41,03%) (0,94-3,76) (7,14%) (0,14-2,88)™‡ÓÔÏÔ 23/267 91/317 22/213
(8,61%) (28,71%) (10,33%)
† Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜
120 µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.
Paediatriki 2009;72:120-000
¶›Ó·Î·˜ 2. ¢È·ÊÔÚ¤˜, ·Ó¿ÏÔÁ· Ì ÙÔÓ ÙfiÔ ‰È·ÌÔÓ‹˜ Î·È ÙË Ê˘ÛÈ΋ ·ÓÔÛ›· ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ·È‰ÈÒÓ ËÏÈΛ·˜>12 ÌËÓÒÓ, ÌÂٷ͇ ∂ÏÏËÓfiÔ˘ÏˆÓ Î·È ÌÂÙ·Ó·ÛÙÒÓ
∂ÏÏËÓfiÔ˘Ï· ¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓN (%) OR ∆ÈÌ‹ p N (%) OR ∆ÈÌ‹ p
(95% CI) (95% CI)
∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· 9/191 Ref† 14/76 Ref†
(4,71%) (18,42%)µfiÚÂÈ· ∂ÏÏ¿‰· 75/278 7,47 <0,001 16/39 3,08 0,011
(26,98%) (3,64-15,35) (41,03%) (1,30-7,30) 0,174¶ÂÏÔfiÓÓËÛÔ˜ 20/185 2,45 0,031 2/28 0,34Î·È ÓËÛÈ¿ (10,81%) (1,09-5,53) (7,14%) (0,07-1,61)™‡ÓÔÏÔ 104/654 32/143
(15,90%) (22,38%)
† Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·120
·fi Ù· Ù¤ÏË ÙÔ˘ ’90, Ë Î·Ù·ÁÚ·Ê‹ Â›Ó·È ÂÏÏÈ‹˜.
™ÙÔȯ›· ·fi ÙÔ ∫∂∂§ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë Ë·Ù›Ùȉ· ∞
·ÔÙÂÏ› Úfi‚ÏËÌ· ‰ËÌfiÛÈ·˜ ˘Á›·˜ ÁÈ· ÙËÓ ∂ÏÏ¿-
‰· Î·È ÙÔ 25-50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ô˘ ‰ËÏÒÓÔ-
ÓÙ·È ÂÙËÛ›ˆ˜ Â›Ó·È ·È‰È¿.
∆Ô ÂÌ‚fiÏÈÔ Ë·Ù›Ùȉ·˜ ∞ ¤¯ÂÈ ¿‰ÂÈ· ¯ÔÚ‹ÁËÛ˘
Û ·È‰È¿ >2 ÂÙÒÓ Î·È Û ÂÓ‹ÏÈΘ (2). ¶ÚfiÛÊ·Ù·,
ÙÔ FDA ÂÓ¤ÎÚÈÓ ÙË ¯Ú‹ÛË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ VAQTA
ÛÙ· Ó‹È· 12-24 ÌËÓÒÓ (11). ∞Ó Î·È ·Ú¯Èο Ô ÂÌ‚Ô-
ÏÈ·ÛÌfi˜ Û˘ÛÙ‹ıËΠÁÈ· ÙȘ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÔÌ¿-
‰Â˜ ÌfiÓÔ, ¤¯Ô˘Ó Á›ÓÂÈ ÚÔÁÚ¿ÌÌ·Ù· Ì·˙ÈÎÔ‡ ÂÌ‚Ô-
ÏÈ·ÛÌÔ‡ ÛÙȘ ÂÚÈÔ¯¤˜ Ì ÂӉȿÌÂÛË ÂÓ‰ËÌÈÎfiÙËÙ·
·fi ÙÔ 1997, fiˆ˜ ÁˆÁÚ·ÊÈο ‰È·ÌÂÚ›ÛÌ·Ù· ÛÙËÓ
πÙ·Ï›·, πÛ·Ó›· Î·È ∞˘ÛÙÚ·Ï›· (12). ¶ÚfiÛÊ·Ù·, ÙÔ
πÛÚ·‹Ï ·ÚÔ˘Û›·Û ÌÈ· ÛËÌ·ÓÙÈ΋ Ì›ˆÛË (>95%)
ÙÔ˘ ÂÙ‹ÛÈÔ˘ ÔÛÔÛÙÔ‡ ›وÛ˘ Ù˘ Ë·Ù›Ùȉ·˜ ∞,
5 ¯ÚfiÓÈ· ÌÂÙ¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ˘Ô¯ÚˆÙÈÎÔ‡ ÂÌ‚Ô-
ÏÈ·ÛÌÔ‡ ÙˆÓ ÌÈÎÚÒÓ ·È‰ÈÒÓ ÙÔ 1999 (13). ¶ÚfiÛÊ·-
Ù· ‰ËÌÔÛÈÂ˘Ì¤Ó· ·ÔÙÂϤÛÌ·Ù· ÂÌÊ·Ó›˙Ô˘Ó ÛËÌ·-
ÓÙÈ΋ Ì›ˆÛË Ù˘ Ë·Ù›Ùȉ·˜ ∞ Û ÔÏÈÙ›˜ ÙˆÓ
∏¶∞ fiÔ˘ ÂÊ·ÚÌfiÛÙËÎÂ Ë ÔÏÈÙÈ΋ ÙÔ˘ Ì·˙ÈÎÔ‡
ÂÌ‚ÔÏÈ·ÛÌÔ‡ (88%) (ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë 50%)
(14). ∏ ÌÂÁ·Ï‡ÙÂÚË Ì›ˆÛË ·Ú·ÙËÚ‹ıËΠ۠·È-
‰È¿ 2-9 ÂÙÒÓ, ·ÏÏ¿ Ë Â›ÙˆÛË ¤¯ÂÈ ÌÂȈı› ÛËÌ·-
ÓÙÈο Î·È ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, ηٷ‰ÂÈÎÓ‡ÔÓÙ·˜ ÌÂȈ̤-
ÓË ÌÂÙ¿‰ÔÛË ÙÔ˘ ÈÔ‡ ̤۷ ÛÙËÓ ÎÔÈÓfiÙËÙ· (14).
∞fi Ù· ‰Â‰Ô̤ӷ Ù˘ ÌÂϤÙ˘ Ì·˜ ÚԤ΢„ fiÙÈ
¤Ó· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ·È‰ÈÒÓ ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 2
ÂÙÒÓ (38%) ÂÌ‚ÔÏÈ¿˙ÂÙ·È ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞,
·ÚfiÏÔ Ô˘ ̤¯ÚÈ Û‹ÌÂÚ· ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ÂÌ‚fiÏÈÔ
‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙÔ ∂ıÓÈÎfi ¶ÚfiÁÚ·ÌÌ· ∂Ì‚Ô-
ÏÈ·ÛÌÒÓ. ∂Ô̤ӈ˜, Ë ·ÁÔÚ¿ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ÂÈ‚·-
Ú‡ÓÂÈ ÙÔÓ ÁÔÓ¤·, ÂÓÒ Ë Û˘Ì‚ÔÏ‹ ÙÔ˘ ·È‰È¿ÙÚÔ˘ ÎÚ›-
ÓÂÙ·È Ô˘ÛÈ·ÛÙÈ΋ ÛÙËÓ ˘„ËÏ‹ ÁÈ· Ù· ÂıÓÈο ‰Â‰Ô̤-
Ó· ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë.
∞fi Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜ ÚԤ΢„Â, fiˆ˜ ¿Ï-
ψÛÙ ·Ó·ÌÂÓfiÙ·Ó, fiÙÈ Ù· ·È‰È¿ ÂÏÏËÓÈ΋˜ ηٷ-
ÁˆÁ‹˜, ηıÒ˜ Î·È ÂΛӷ ·ÛÙÈÎÒÓ ÂÚÈÔ¯ÒÓ, ›¯·Ó
˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ Î¿Ï˘„˘, ÛÂ
Û‡ÁÎÚÈÛË Ì ٷ ·ÓÙ›ÛÙÔȯ· ÌÂÙ·Ó·ÛÙÒÓ ‹ ∂ÏÏËÓfi-
Ô˘ÏˆÓ Ô˘ ‰È·‚ÈÔ‡Ó ÛÙËÓ ÂÚÈʤÚÂÈ·.
∏ ·Ó›¯Ó¢ÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜
∞ Û ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿ <12 ÌËÓÒÓ ˘Ô‰ËÏÒ-
ÓÂÈ ·ÓÔÛ›· ÌÂÙ¿ ·fi Ê˘ÛÈ΋ Ïԛ̈ÍË. ™‡Ìʈӷ ÌÂ
Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜, 17% ÙˆÓ ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӈÓ
·È‰ÈÒÓ Â›¯·Ó ÓÔÛ‹ÛÂÈ ·fi Ë·Ù›Ùȉ· ∞. ∆Ô ÔÛÔ-
ÛÙfi ·˘Ùfi Â›Ó·È ÂÍ·ÈÚÂÙÈο ·˘ÍË̤ÓÔ Î·È ‰ÂÓ ÌÔÚ›
Ó· ·Ô‰Ôı› ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜.
™ËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÙfiÛÔ ÛÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ ο-
Ï˘„Ë fiÛÔ Î·È ÛÙË Ê˘ÛÈ΋ ·ÓÔÛ›· ‚Ú¤ıËΠÌÂٷ͇ ÙˆÓ
‰È·ÊfiÚˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ Ù˘ ¯ÒÚ·˜ (¶›Ó·-
η˜ 3). ™˘ÁÎÂÎÚÈ̤ӷ, ÛÙËÓ ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· ηٷ-
ÁÚ¿ÊËÎÂ Ë ˘„ËÏfiÙÂÚË ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë Î·È Ù·
¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÔÚÔıÂÙÈÎfiÙËÙ·˜ ÏfiÁˆ Ê˘ÛÈ΋˜ÓfiÛËÛ˘. √È ‰È·ÊÔÚ¤˜ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ÌfiÓÔ ÁÈ· Ù· ∂ÏÏËÓfiÔ˘Ï·, ÏfiÁˆ Ù˘ ¯·ÌËÏ‹˜ ÂÌ‚Ô-ÏÈ·ÛÙÈ΋˜ Î¿Ï˘„˘ ÙˆÓ ·È‰ÈÒÓ ÌÂÙ·Ó·ÛÙÒÓ.
∞Í›˙ÂÈ ÂÈϤÔÓ Ó· ÙÔÓÈÛÙ› fiÙÈ, ÂÓÒ ÛÙÔ Û˘ÓÔÏÈ-Îfi ‰Â›ÁÌ· Ë ‰È·ÊÔÚ¿ ÔÚÔıÂÙÈÎfiÙËÙ·˜ ÌÂٷ͇ ∂ÏÏË-ÓfiÔ˘ÏˆÓ Î·È ·È‰ÈÒÓ ÌÂÙ·Ó·ÛÙÒÓ ‰ÂÓ ‹Ù·Ó ÛÙ·ÙÈ-ÛÙÈο ÛËÌ·ÓÙÈ΋, ÛÙ· ·È‰È¿ Ô˘ ‰È·‚ÈÔ‡Ó ÛÙËÓ ∫Â-ÓÙÚÈ΋ ∂ÏÏ¿‰· (fiÔ˘ ηٷÁÚ¿ÊËÎÂ Ë ˘„ËÏfiÙÂÚËÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë) Ë ‰È·ÊÔÚ¿ ‹Ù·Ó ÛËÌ·ÓÙÈ΋(¶›Ó·Î·˜ 3). ∏ ‰È·ÊÔÚ¿ ÛÙËÓ Â›ÙˆÛË Ù˘ Ê˘ÛÈ΋˜·ÓÔÛ›·˜ Ô˘ ηٷÁÚ¿ÊÂÙ·È ÌÂٷ͇ ÂÚÈÔ¯ÒÓ ·Ó¿ÏÔ-Á· Ì ÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë ÂÍËÁÂ›Ù·È Èı·ÓÒ˜·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Û ÂÚÈÔ¯¤˜ ¯·ÌËÏ‹˜ ÂÌ‚ÔÏÈ·-ÛÙÈ΋˜ Î¿Ï˘„˘ Ë ‰È·ÛÔÚ¿ ÙÔ˘ ÈÔ‡ Ù˘ Ë·Ù›Ùȉ·˜∞ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË Î·È ÌÔχÓÔÓÙ·È Â›ÓÔÛ· ·È‰È¿·ÓÂÍ·Úًو˜ ηٷÁˆÁ‹˜.
∆· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜ ¤Ú¯ÔÓÙ·È Û ·ÓÙ›ıÂÛË ÌÂÂΛӷ ÌÈ·˜ ÚfiÛÊ·Ù˘ ÔÚÔÂȉËÌÈÔÏÔÁÈ΋˜ ÌÂϤ-Ù˘ Ô˘ ·ÊÔÚÔ‡Û ÙËÓ Ë·Ù›Ùȉ· µ, ÁÈ· ÙËÓ ÔÔ›·ÂÊ·ÚÌfi˙ÂÙ·È Ì·˙ÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ ÙˆÓ ‚ÚÂÊÒÓ ·fiÙÔ 1998 (15). ™ÙË Û˘ÁÎÂÎÚÈ̤ÓË ÌÂϤÙË, ‰ÂÓ ‚Ú¤ıËÎÂÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÌÂٷ͇ ÙˆÓ ‰È¿ÊÔ-ÚˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ, ÏfiÁˆ Ù˘ ÂÓÈ·›·˜ ÔÏÈ-ÙÈ΋˜ ˘Á›·˜ Ô˘ ÂÊ·ÚÌfi˙ÂÙ·È Û fiÏË ÙË ¯ÒÚ· (15).∂Ô̤ӈ˜, ·fi Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜, ÛÂ Û˘Ó‰˘·ÛÌfiÌ ÙËÓ ÚÔ·Ó·ÊÂÚfiÌÂÓË ·Ú·Ù‹ÚËÛË, ‰È·Ê·›ÓÂÙ·È Ë·Ó¿ÁÎË ÂÊ·ÚÌÔÁ‹˜ Ì·˙ÈÎÔ‡ ÂÌ‚ÔÏÈ·ÛÌÔ‡ ÙˆÓ ·È-‰ÈÒÓ Î·È ÁÈ· ÙËÓ Ë·Ù›Ùȉ· ∞, fiˆ˜ Û˘Ó¤‚Ë Î·È Û¿ÏϘ ¯ÒÚ˜, Î·È Ì¿ÏÈÛÙ· ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 12 ÌË-ÓÒÓ (11-14). ª¿ÏÈÛÙ·, Ì ‚¿ÛË Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ÌÂϤÙ˘ ·˘Ù‹˜, Ë ∂ıÓÈ΋ ∂ÈÙÚÔ‹ ∂Ì‚ÔÏÈ·ÛÌÒÓ ÂÈ-ÛËÁ‹ıËΠÙËÓ ¤ÓÙ·ÍË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ Ù˘ Ë·Ù›Ùȉ·˜∞ ÛÙÔ˘˜ ˘Ô¯ÚˆÙÈÎÔ‡˜ ÂÌ‚ÔÏÈ·ÛÌÔ‡˜, Ì ·ÔÙ¤ÏÂ-ÛÌ· ÙÔ ÂÌ‚fiÏÈÔ Ó· ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙÔ Ó¤Ô ∂ıÓÈÎfi¶ÚfiÁÚ·ÌÌ· ∂Ì‚ÔÏÈ·ÛÌÒÓ ÙÔ˘ 2008.
µÈ‚ÏÈÔÁÚ·Ê›·
1. American Academy of Pediatrics. Hepatitis A. In:Pickering LK, ed. 2003 Red Book: Report of the Committeeon Infectious Diseases. 26th ed. Elk Grove Village, IL:American Academy of Pediatrics; 2003:309-318.
2. CDC: Prevention of Hepatitis A through Active or PassiveImmunization: Recommendations of the advisoryCommittee on Immunization Practices (ACIP). MMWR1999,48(No. RR-12).
3. Staes C, Schlenker T, Risk I, et al. Source of infectionamong persons with acute hepatitis A and no identificatiedrisk factors. Salt Lake County, Utah, 1996 [Abstract 302].Clin Infect Dis 1997;24:411.
4. Lionis C, Koulentaki M, Biziagos E, Kouroumalis E.Current prevalence of hepatitis A, B nad C in a well definedarea in rural Krete, Greece. J Viral Hepat 1997;4:55-61.
5. Kremastinou J, Kalapothaki V, Trichopoulos D. Thechanging epidemiologic pattern of hepatitis A infection inurban Greece. Am J Epidemiol 1984;120:703-706.
121√ÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞
¶·È‰È·ÙÚÈ΋ 2009;72:121-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·121
6. Papadopoulou V, Stavrati A, Patsialis T, et al. Changes inthe Epidemiology of acute hepatitis A during 1985-1992period. Pediatr N Gr 1995;7:30-34.
7. Dalekos GN, Zervou E, Karabini F, Tsianos EV. Prevalenceof viral markers among refugees from southern Albania:increased incidence of infection with hepatitis A, B and Dviruses. Eur J Gastrenterol Hepatol 1995;7:553-558.
8. Jacobsen KH, Koopman JS. Declining hepatitis Aseroprevalance: a global review and analysis EpidemiolInfect 2004;132:1005-1022.
9. World Health Organization. Hepatitis A. WHO/CDS//CSR/EDC/2000.7
10. Frosner GG, Papaevangelou G, Butler R, Iwarson S,Lindholm A, Couroucé-Pauty A, et al. Antibody againsthepatitis A in seven European countries I: comparison ofprevalence data in different age groups. Am J Epidemiol1979;110:63-69.
11. FDA Approval of VAQTA (hepatitis A vaccine, inactivated)in children > 1 year. MMWR 2005;54:1026.
12. Van Damme P, Van Herck K. Effect of Hepatitis AVaccination Programs. JAMA 2005;294:246-248.
13. Dagan R, Leventha A, Anis E, Slatter P, Ashur Y, ShouvalD. Incidence of Hepatitis A in Israel Following UniversalImmunization of Toddlers. JAMA 2005;294:202-210.
14. Wasley A, Samandari T, Bell BP. Incidence of Hepatitis Ain the United States in the era of vaccination. JAMA2005;294:194-201.
15. Papaevangelou V, Hadjichristodoulou C, Cassimos D,Pantelaki K, Tzivaras A, Hatzimichael et al. Seroepi-demiology of hepatitis B in Greek children six years afterthe implementation of universal vaccination. Infection2008;36:135-139
122 µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.
Paediatriki 2009;72:122-000
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Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·122
§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿: ÂÌÂÈÚ›· 28 ÂÙÒÓ
∞. ¶Ô˘ÚÙÛ›‰Ë˜1, ¢. ¢ÔÁ¿Ó˘1, ª. ª¿Î·1, ¢. ªÔ˘¯Ô‡ÙÛÔ˘1, ª. µ·Ú‚Ô˘ÙÛ‹1, ª. ™˘ÓÔ‰ÈÓÔ‡2,
∞ÈÎ. ªÈ¯·‹Ï-™ÙÚ¿ÓÙ˙È·3, ∂. ∫ÔÛÌ›‰Ë1
¶ÂÚ›ÏË„Ë
∂ÈÛ·ÁˆÁ‹: ∆Ô Ï¤Ìʈ̷ Hodgkin ·ÔÙÂÏ› ÂÚ›Ô˘ ÙÔ 4-5% ÙˆÓ Î·ÎÔËıÂÈÒÓ ÛÙ· ·È‰È¿. ∆· ÙÂÏÂ˘Ù·›·30 ¯ÚfiÓÈ·, Ë ¯Ú‹ÛË Û˘Ó‰˘·ÛÌÔ‡ ¯ËÌÂÈÔıÂڷ¢ÙÈÎÒÓ ÚˆÙÔÎfiÏψÓ, Ì ‹ ¯ˆÚ›˜ ·ÎÙÈÓÔıÂڷ›·, Ô‰‹-ÁËÛ Û ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÚfiÁÓˆÛ˘ Î·È ¤Î‚·Û˘ ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ.
∞ÛıÂÓ›˜ Î·È Ì¤ıÔ‰ÔÈ: ∆· ÙÂÏÂ˘Ù·›· 28 ¯ÚfiÓÈ·, ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÛÙÔ ÙÌ‹Ì· Ì·˜ 73 ·È‰È¿ Ì ÓfiÛÔHodgkin. ™Ù· ·È‰È¿ ·˘Ù¿ ÌÂÏÂÙ‹ıËÎ·Ó ÂȉËÌÈÔÏÔÁÈο, ÎÏÈÓÈο Î·È ÈÛÙÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο, Ô ÙÚfi-Ô˜ ıÂڷ›·˜ Î·È Ë ¤Î‚·ÛË Î·È ·ÍÈÔÏÔÁ‹ıËÎ·Ó ‰˘ÓËÙÈÎÔ› ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ ÂȉÚÔ‡Ó ÛÙËÓÚfiÁÓˆÛË.
AÔÙÂϤÛÌ·Ù·: ∏ ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ (46 ·ÁfiÚÈ·, 27 ÎÔÚ›ÙÛÈ·) Î˘Ì·ÈÓfiÙ·Ó ·fi 3,5 ¤ˆ˜ 15 ¤ÙË. ™ÙË Û˘-ÓÙÚÈÙÈ΋ ÏÂÈÔ„ËÊ›·, Ë ·ÈÙ›· Ù˘ ·Ú¯È΋˜ ÚÔÛ¤Ï¢Û˘ ‹Ù·Ó Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· (81%), ÂÓÒ Ë ‰È¿Ú-ÎÂÈ· ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ÚÈÓ ÙË ‰È¿ÁÓˆÛË Î˘Ì·ÈÓfiÙ·Ó ·fi Ï›Á˜ ̤Ú˜ ¤ˆ˜ Î·È 12 Ì‹Ó˜. ªÂٷ͇ ÙˆÓÈÛÙÔÏÔÁÈÎÒÓ ˘fiÙ˘ˆÓ ˘ÂÚ›¯Â Ë Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË (63%), ÂÓÒ 44% ÙˆÓ ·ÛıÂÓÒÓ Â›¯·Ó ÚÔ¯ˆÚË-̤ÓË ÓfiÛÔ (ÛÙ·‰›Ô˘ πππ-IV). ∏ ıÂڷ›· ÂÚÈÂÏ¿Ì‚·Ó ÌfiÓÔ ¯ËÌÂÈÔıÂڷ›· Û 5 ·ÛıÂÓ›˜, ÌfiÓÔ ·ÎÙÈ-ÓÔıÂڷ›· Û 9 Î·È Û˘Ó‰˘·ÛÌfi Û 59. ∏ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ·Ó¤Ú¯ÂÙ·È Û 97,3%. OÎÙÒ ·È‰È¿ ˘Ô-ÙÚÔ›·Û·Ó 20-65 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË Î·È ·fi ·˘Ù¿ ˙Ô˘Ó Ù· 7, ÂÓÒ ‰Â‡ÙÂÚË ‹ ÙÚ›ÙË Î·ÎÔ‹ıÂÈ· ‰È·-ÈÛÙÒıËΠ۠4 ·È‰È¿, ·fi Ù· ÔÔ›· ˙Ô˘Ó Ù· 3. ¢ÂÓ ‰È·ÈÛÙÒıËÎ·Ó ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ Ó·Û˘Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ.
™˘ÌÂÚ¿ÛÌ·Ù·: ∏ ¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ì ÓfiÛÔ Hodgkin Â›Ó·È ÂÍ·ÈÚÂÙÈ΋ ÌÂ Û˘Ó‰˘·ÛÌfi¯ËÌÂÈÔıÂڷ›·˜ Î·È ·ÎÙÈÓÔıÂڷ›·˜. ∏ Û˘Ó¯È˙fiÌÂÓË ·Ú·ÎÔÏÔ‡ıËÛË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·È‰ÈÒÓÎÚ›ÓÂÙ·È ··Ú·›ÙËÙË ÁÈ· Ó· ‰È¢ÎÚÈÓÈÛÙ› ·Ó ˘¿Ú¯Ô˘Ó ‰˘ÓËÙÈο ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ù˘ ¤Î‚·ÛË˜ÙˆÓ ·È‰ÈÒÓ Ì ÓfiÛÔ Hodgkin.
§¤ÍÂȘ ÎÏÂȉȿ: §¤Ìʈ̷ ∏odgkin, ·È‰È΋ ËÏÈΛ·, ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜.
Hodgkin’s lymphoma in children: 28 years of experience
A. Pourtsidis1, D. Doganis1, M. Baka1, D. Bouhoutsou1, M. Varvoutsi1, M. Synodinou2,
A. Michael-Strantzia3, E. Kosmidi1
Abstract
Background: In the past 30 years the use of combined chemotherapy regimes, with or withoutradiotherapy, has improved significantly the prognosis for patients with Hodgkin’s lymphoma.
Methods: A retrospective study was made of 73 children with Hodgkin’s lymphoma treated at this centreover the past 28 years. The following data were analyzed and correlated with the outcome: age, sex, stage,histology, treatment, follow-up period.
Results: Among patients, there were 46 boys and 27 girls. The age at diagnosis varied from 3.3 to 15 years,and 15 patients were 8 years old or younger. Most of the patients (81%) presented withlymphadenopathy, with a time interval of 3 days to 12 months between the first appearance of clinicalsigns and the diagnosis. Nodular sclerosis was the predominant histological subtype (63%), and 44% ofpatients had advanced disease (stage III or IV). Chemotherapy alone was used in 5 patients, radiation in9 and combined treatment in 59. Relapses occurred in 8 children and 4 developed second or thirdmalignancies. The overall survival was 97.3%. One patient died from the disease and one after a thirdmalignancy. No specific factors related to the outcome were detected.
Conclusions: The outcome of children with Hodgkin’s disease is excellent with chemotherapy combinedwith low dose radiation. A longer follow-up of a greater number of patients is necessary to detectprognostic factors related to the outcome of children and adolescents with Hodgkin’s disease.
Key words: Hodgkin’s lymphoma, children, prognostic factors.
123∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞ ORIGINAL ARTICLE
1 √ÁÎÔÏÔÁÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
2 ∞ÎÙÈÓÔıÂڷ¢ÙÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
3 ¶·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎfi∂ÚÁ·ÛÙ‹ÚÈÔ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
∞fiÛÙÔÏÔ˜ ¶Ô˘ÚÙÛ›‰Ë˜[email protected]√ÁÎÔÏÔÁÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
1 Department of Oncology,“P. & A. Kyriakou”Children’s Hospital,Athens, Greece
2 Department ofRadiotherapy, “P. & A. Kyriakou”Children’s Hospital,Athens, Greece
3 Laboratory of Pathology,“P. & A. Kyriakou”Children’s Hospital,Athens, Greece
Correspondence:
Apostolos [email protected] of Oncology, “P. & A. Kyriakou” Children’sHospital, Athens, Greece
¶·È‰È·ÙÚÈ΋ 2009;72:123-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·123
∂ÈÛ·ÁˆÁ‹
∆Ô Ï¤Ìʈ̷ Hodgkin ·ÔÙÂÏ› ÙÔ 4-5% ÙˆÓ Î·-
ÎÔËıÂÈÒÓ ÛÙ· ·È‰È¿ Î·È ÙÔ ¤ÎÙÔ Î·Ù¿ ÛÂÈÚ¿ Û˘¯Ófi-
ÙËÙ·˜ ÓÂÔÏ·ÛÌ·ÙÈÎfi ÓfiÛËÌ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜
ÛÙËÓ ∂˘ÚÒË Î·È ÛÙȘ ∏.¶.∞., ÌÂÙ¿ ÙË Ï¢¯·ÈÌ›·,
ÙÔ˘˜ fiÁÎÔ˘˜ ÂÁÎÂÊ¿ÏÔ˘, ÙÔ Ó¢ÚÔ‚Ï¿Ûو̷, ÙÔ ÓÂ-
ÊÚÔ‚Ï¿Ûو̷ Î·È ÙÔ ÌË Hodgkin ϤÌʈ̷. ∏ ›-
ÙˆÛË Ù˘ ÓfiÛÔ˘ ‰È·Ê¤ÚÂÈ ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ·,
ÙÔ Ê‡ÏÔ, ÙËÓ ÂıÓÈÎfiÙËÙ· Î·È ÙÔÓ ÈÛÙÔÏÔÁÈÎfi Ù‡Ô.
™‡Ìʈӷ Ì ٷ ‰Â‰Ô̤ӷ Ù˘ International Agency
for Research on Cancer (πARC), Ë ÂÙ‹ÛÈ· ›وÛË
ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin Û ·È‰È¿ ËÏÈΛ·˜ οو
ÙˆÓ 15 ¯ÚfiÓˆÓ ˘ÔÏÔÁ›˙ÂÙ·È Û 5,5 ÂÚÈÙÒÛÂȘ ·Ó¿
ÂηÙÔÌ̇ÚÈÔ ÛÙÔÓ ·ÓÂÙ˘Á̤ÓÔ ÎfiÛÌÔ, ·ÏÏ¿ ÛÙÔ˘˜
ÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ ËÏÈΛ·˜ 15-20 ¯Úfi-
ÓˆÓ Êı¿ÓÂÈ ÙȘ 12,1. ∞ÓÙÈı¤Ùˆ˜, ÛÙË ¢˘ÙÈ΋ ∞Û›· Ë
›وÛË ÛÙ· ·È‰È¿ Â›Ó·È ÛÙ·ıÂÚ¿ ¿Óˆ ·fi ÙȘ 7
ÂÚÈÙÒÛÂȘ ·Ó¿ ÂηÙÔÌ̇ÚÈÔ (1). ™ÙËÓ ∂ÏÏ¿‰·, Ë
›وÛË ÛÙ· ·È‰È¿ ·Ó¤Ú¯ÂÙ·È Û 7,2 ·Ó¿ ÂηÙÔÌ-
̇ÚÈÔ ¿ÓıÚˆÔ-¤ÙË. ÀÔÏÔÁ›ÛÙËΠÁÈ· ÚÒÙË ÊÔÚ¿
ÛÙË ¯ÒÚ· Ì·˜ Ì ‚¿ÛË Ù· ·Ó·Ï˘ÙÈο ‰Â‰Ô̤ӷ Ô˘
Û˘ÁÎÂÓÙÚÒÓÔÓÙ·È ÛÙÔ ∂ÏÏËÓÈÎfi ∞Ú¯Â›Ô ¶·È‰ÈÎÒÓ
∞ÈÌ·ÙÔÏÔÁÈÎÒÓ ∫·ÎÔËıÂÈÒÓ, Ô˘ ÏÂÈÙÔ˘ÚÁ› ÛÙÔ
∂ÚÁ·ÛÙ‹ÚÈÔ ÀÁÈÂÈÓ‹˜ Î·È ∂ȉËÌÈÔÏÔÁ›·˜ Î·È Û˘ÁÎÂ-
ÓÙÚÒÓÂÈ ÂıÓÈο ‰Â‰Ô̤ӷ (2). ∞·ÓÙ¿Ù·È Û˘¯ÓfiÙÂÚ·
ÛÙÔ˘˜ ∫·˘Î¿ÛÈÔ˘˜, ÂÓÒ ˘¿Ú¯Ô˘Ó ÙÚÂȘ ‰È·ÎÚÈÙÔ›
Ù‡ÔÈ fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ËÏÈΛ·: ·˘Ùfi˜ Ô˘ ·ÊÔÚ¿ ·È-
‰È¿ ÌÈÎÚfiÙÂÚ· ÙˆÓ 15 ÂÙÒÓ, ·˘Ùfi˜ ÙˆÓ Ó·ÚÒÓ ÂÓË-
Ï›ÎˆÓ Î·È ·˘Ùfi˜ ÙˆÓ ÌÂÁ·Ï‡ÙÂÚˆÓ ÂÓËÏ›ÎˆÓ (·ÛıÂ-
Ó›˜ ¿Óˆ ÙˆÓ 55 ÂÙÒÓ). ∏ ÓfiÛÔ˜ ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÈ· ‰È-
ÎfiÚ˘ÊË ËÏÈÎȷ΋ ηٷÓÔÌ‹, Ì ̛· ·È¯Ì‹ ÛÙËÓ ÂÊË-
‚›· Î·È Ì›· Û ·ÛıÂÓ›˜ ¿Óˆ ÙˆÓ 50 ÂÙÒÓ, ÂÓÒ Â›Ó·È
Û·ÓÈfiÙ·ÙË Û ·È‰È¿ ÌÈÎÚfiÙÂÚ· ÙˆÓ 5 ÂÙÒÓ (3).
∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ 30 ¯ÚfiÓˆÓ, Ë
¯Ú‹ÛË Û˘Ó‰˘·ÛÌ¤ÓˆÓ ¯ËÌÂÈÔıÂڷ¢ÙÈÎÒÓ Û¯ËÌ¿-
ÙˆÓ, Ì ‹ ¯ˆÚ›˜ ·ÎÙÈÓÔıÂڷ›·, Ô‰‹ÁËÛ Û ÛËÌ·-
ÓÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÚfiÁÓˆÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì ϤÌ-
ʈ̷ Hodgkin, ·‡ÍËÛË ÙˆÓ ÔÛÔÛÙÒÓ ›·Û˘ ηÈ
Û˘Á¯ÚfiÓˆ˜ Ì›ˆÛË Ù˘ ·ÒÙÂÚ˘ ÙÔÍÈÎfiÙËÙ·˜. H
ÂÓÙ·ÂÙ‹˜ ÂχıÂÚË Û˘Ì‚¿Ì·ÙÔ˜ ÂÈ‚›ˆÛË (EFS)
ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ· ˘ÂÚ‚·›ÓÂÈ ÙÔ 90%
ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ ·ÌËÏÔ‡ Î·È ÂӉȿÌÂÛÔ˘ ÎÈÓ‰‡ÓÔ˘
Ì ÂÓÙÔÈṲ̂ÓË ÓfiÛÔ (4) Î·È ÙÔ 60-70% ÁÈ· ·ÛıÂÓ›˜
˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Ì ÚÔ¯ˆÚË̤ÓË ÓfiÛÔ - Û˘Ó‹ıˆ˜
ÛÙ·‰›Ô˘ IV (5, 6).
∏ ·ÚÔ‡Û· ÌÂϤÙË Î·Ù·ÁÚ¿ÊÂÈ ÂȉËÌÈÔÏÔÁÈο,
ÂÚÁ·ÛÙËÚȷο, ÈÛÙÔÏÔÁÈο Î·È ·ÂÈÎÔÓÈÛÙÈο ¯·Ú·-
ÎÙËÚÈÛÙÈο, ηıÒ˜ ›Û˘ ÙÔ Â›‰Ô˜ ıÂڷ›·˜ Î·È ÙËÓ
¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÛÙÔ ÙÌ‹-
Ì· Ì·˜. ∆·˘Ùfi¯ÚÔÓ·, ¤¯ÂÈ ˆ˜ ÛÙfi¯Ô Ó· ÚÔÛ‰ÈÔÚ›ÛÂÈ
ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ‰˘ÓËÙÈο Û¯ÂÙ›˙Ô-
ÓÙ·È Ì ÙËÓ ÙÂÏÈ΋ ¤Î‚·ÛË Î·È ÙȘ ·ÒÙÂÚ˜ Û˘Ó¤ÂÈ-
˜ Ù˘ ıÂڷ›·˜ ÙˆÓ ·È‰ÈÒÓ Ì ϤÌʈ̷ Hodgkin.
∞ÛıÂÓ›˜ Î·È Ì¤ıÔ‰ÔÈ
∏ ÌÂϤÙË Â›Ó·È ·Ó·‰ÚÔÌÈ΋ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ fiÏ· Ù· ·È-
‰È¿ Ì ϤÌʈ̷ Hodgkin Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÛÙÔ √ÁÎÔ-
ÏÔÁÈÎfi ÙÌ‹Ì· ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡»
·fi ÙÔ 1978 ¤ˆ˜ ÙÔ 2005. ∏ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ·ÛıÂÓÒÓ ¤ÁÈ-
Ó Ì ¯Ú‹ÛË ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ηٿ Ann Arbor (7), ÂÓÒ Ë ‰È¿-
ÎÚÈÛË ÙˆÓ ÈÛÙÔÏÔÁÈÎÒÓ ˘fiÙ˘ˆÓ ¤ÁÈÓ ۇÌʈӷ Ì ÙËÓ Ù·-
ÍÈÓfiÌËÛË Î·Ù¿ Rye (8).
T· Û¯‹Ì·Ù· ¯ËÌÂÈÔıÂڷ›·˜ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËηÓ
ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·-
η 1. ∏ ıÂڷ¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË ‰È¤ÊÂÚ ·Ó¿ÏÔÁ· Ì ÙË ÚÔ-
ÓÈ΋ ÂÚ›Ô‰Ô. ¶ÂÚ›Ô‰Ô˜ ∞ (1979-1988, 19 ·ÛıÂÓ›˜): ·ÓÙÈÌÂ-
Ùˆ›ÛıËÎ·Ó Ì ·ÎÙÈÓÔıÂڷ›· ÌfiÓÔ, ¯ËÌÂÈÔıÂڷ›· ÌfiÓÔ
(MOPP ‹ ABVD) ‹ Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂڷ›·˜ (ªOPP ‹
ABVD) Î·È ·ÎÙÈÓÔıÂڷ›·˜ 30-40 Gy. ¶ÂÚ›Ô‰Ô˜ µ (1989-
1993, 26 ·ÛıÂÓ›˜): ÔÈ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ π ‹ ππ∞ ¤Ï·‚·Ó 4 ·-
ÎÏÔ˘˜ ∞µVD Î·È ·ÎÔÏÔ‡ıˆ˜ ·ÎÙÈÓÔıÂڷ›· Ì 20 Gy ÛÙȘ
·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜, ÂÓÒ ÔÈ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ
ππµ, III ‹ πV ¤Ï·‚·Ó ·fi 3 ·ÎÏÔ˘˜ MOPP Î·È ABVD ÂÓ·ÏÏ¿Í
Î·È ÛÙË Û˘Ó¤¯ÂÈ· ·ÎÙÈÓÔıÂڷ›· 20 Gy ÛÙȘ ·Ú¯Èο ÚÔÛ‚Â-
‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜, ÙÔ ÛÏ‹Ó· Î·È ÙÔ˘˜ ·Ú·ÔÚÙÈÎÔ‡˜ ÏÂÌ-
Ê·‰¤Ó˜ (9). ¶ÂÚ›Ô‰Ô˜ ° (1994-2005, 28 ·ÛıÂÓ›˜): Ù· ·È‰È¿
·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Û‡Ìʈӷ Ì ÙÔ ÚˆÙfiÎÔÏÏÔ Ù˘ °·ÏÏÈ-
΋˜ ∂Ù·ÈÚ›·˜ ¶·È‰È·ÙÚÈ΋˜ √ÁÎÔÏÔÁ›·˜, MDH 90 (10): ÁÈ·
ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ π ‹ ππ ¯ÔÚËÁ‹ıËÎ·Ó 4 ·ÎÏÔÈ VBVP
Î·È ·ÎÙÈÓÔ‚ÔÏ›· Ì 20 Gy ÛÙȘ ·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ Â-
ÚÈÔ¯¤˜. √È ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ πππ ¤Ï·‚·Ó ·fi 2 ·ÎÏÔ˘˜
MOPP Î·È ABVP ÂÓ·ÏÏ¿Í Î·È ·ÎÙÈÓÔıÂڷ›· Ì 20 Gy ÛÙȘ
·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜, ÙÔ ÛÏ‹Ó· Î·È ÙÔ˘˜ ·Ú·-
ÔÚÙÈÎÔ‡˜ ÏÂÌÊ·‰¤Ó˜. √È ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ IV ¤Ï·‚·Ó 2 ·-
ÎÏÔ˘˜ OPPA Î·È 4 ·ÎÏÔ˘˜ COPP Î·È ÛÙË Û˘Ó¤¯ÂÈ· ·ÎÙÈÓÔ-
ıÂڷ›· Ì 20 Gy ÛÙÔ˘˜ ·Ú¯Èο ÚÔۂ‚ÏË̤ÓÔ˘˜ ÏÂÌÊ·-
‰¤Ó˜ Î·È Ù· ·Ú¯Èο ÚÔۂ‚ÏË̤ӷ fiÚÁ·Ó·. ∏ ‰fiÛË Ù˘
·ÎÙÈÓÔıÂڷ›·˜ ‹Ù·Ó Û fiϘ ÙȘ ÂÚÈÙÒÛÂȘ 20 Gy, ÂÊfi-
ÛÔÓ Ë ·ÂÈÎÔÓÈÛÙÈ΋ ·¿ÓÙËÛË ˘ÂÚ¤‚·ÈÓ ÙÔ 70%. ∂ȉÈο
ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ IV, ÁÈ· ÙÔ ‹·Ú ¯ÔÚËÁ‹ıËÎ·Ó 12
¶›Ó·Î·˜ 1. £Âڷ›· ÙˆÓ ·ÛıÂÓÒÓ Ì ÓfiÛÔ Hodgkin
1978-1988 A£ ª√¡√ (9*)ã ª√¡√ (5) ª√PP:2*
ABVD:1∞§§√:2
X£ & ∞£ (5) ª√PP:2ABVD:3
1989-1993 ã & ∞£ (26) ª√PP & ABVD: 15ABVD: 10A§§√: 1
1994-2005 X£ & ∞£ (28) ª√PP & ABVP:8VBVP:11
√PPA & COPP: 8ABVP/COP: 1
* ·ÚÈıÌfi˜ ·ÛıÂÓÒÓMOPP (mustine, vincristine, procarbazine, prednisone) ABVD/P (doxorubicin, bleomycin, vinblastine, DTIC/prednisone)VBVP (vinblastine, bleomycin, etoposide, prednisone), OPPA (vincristine, prednisone, procarbazine, doxorubicin) COPP (cyclophosfamide, vincristine, procarbazine,prednisone)
124 ∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:124-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·124
Gy, ÁÈ· Ù· ÔÛÙ¿ 20-30 Gy, ÂÓÒ ÁÈ· ÙÔ˘˜ Ó‡ÌÔÓ˜ ¯ÔÚËÁ‹ıË-
Î·Ó 12 Gy ÌfiÓÔ ·Ó ‰ÂÓ ˘‹Ú¯Â Ï‹Ú˘ ‡ÊÂÛË ÌÂÙ¿ ·fi ‰‡Ô
Û¯‹Ì·Ù· √PPA. ÃÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Ì¤ıÔ‰Ô˜ Kaplan-Meier
ÁÈ· ÙÔÓ Î·ıÔÚÈÛÌfi Ù˘ Û˘ÓÔÏÈ΋˜ ÂÈ‚›ˆÛ˘ Î·È Ù˘ ¯ˆÚ›˜
ÓfiÛÔ ‹ ۇ̂·Ì· ÂÈ‚›ˆÛ˘. ø˜ ۇ̂·Ì· ıˆڋıËÎÂ Ô ı¿Ó·-
ÙÔ˜, Ë ˘ÔÙÚÔ‹ Î·È Ë ‰Â‡ÙÂÚË Î·ÎÔ‹ıÂÈ·. °È· ÙË ÛÙ·ÙÈÛÙÈ΋
·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÔÈ ÂÍ‹˜ ÛÙ·ÙÈ-
ÛÙÈΤ˜ ‰ÔÎÈ̷ۛ˜: (1) ÌË ·Ú·ÌÂÙÚÈ΋ ‰ÔÎÈÌ·Û›· Mann-
Witney, (2) chi-square test Î·È (3) Fisher’s exact test.
∞ÔÙÂϤÛÌ·Ù·
™˘ÓÔÏÈο ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÛÙÔ ÙÌ‹Ì· Ì·˜ 73
·È‰È¿, 46 ·ÁfiÚÈ· Î·È 27 ÎÔÚ›ÙÛÈ· (·ÁfiÚÈ·/ÎÔÚ›ÙÛÈ·:
1,7/1). ∏ ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ Î˘Ì·ÈÓfiÙ·Ó ·fi 3,5-15
ÂÙÒÓ (‰È¿ÌÂÛË ÙÈÌ‹ 12 ¤ÙË) (Ù· ·ÁfiÚÈ· ·fi 3,5-15
ÂÙÒÓ Î·È Ù· ÎÔÚ›ÙÛÈ· ·fi 7,5-15 ÂÙÒÓ) (∂ÈÎfiÓ· 1). √
¯ÚfiÓÔ˜ ·fi ÙËÓ ÂÌÊ¿ÓÈÛË ÙÔ˘ ÚÒÙÔ˘ Û˘ÌÙÒÌ·ÙÔ˜
‹ ÎÏÈÓÈÎÔ‡ ÛËÌ›Ԣ ¤ˆ˜ ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ ΢-
Ì·ÈÓfiÙ·Ó ·fi 3 Ë̤Ú˜ ¤ˆ˜ 12 Ì‹Ó˜ (¢∆ 2 Ì‹Ó˜),
¯ˆÚ›˜ Ó· ‰È·ÈÛÙˆı› Û˘Û¯¤ÙÈÛË ·˘ÙÔ‡ Ì ÙËÓ ËÏÈΛ·
‹ ÙÔ Ê‡ÏÔ. ™ÙËÓ ÏÂÈÔ„ËÊ›·, Ë ·ÈÙ›· Ù˘ ·Ú¯È΋˜
‰ÈÂÚ‡ÓËÛ˘ ÙÔ˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·
(59 ·È‰È¿, 81%), ÂÓÒ 4 ·È‰È¿ ‰ÈÂÁÓÒÛıËÛ·Ó ÛÙ·
Ï·›ÛÈ· ‰ÈÂÚ‡ÓËÛ˘ ÂÌ˘Ú¤ÙÔ˘, 2 ÏfiÁˆ ¯ÚfiÓÈÔ˘
‚‹¯·, 3 ÏfiÁˆ Ù˘¯·›Ô˘ ·ÂÈÎÔÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘ Î·È 5
ÏfiÁˆ ¿ÏÏ˘ Û˘Ìو̷ÙÔÏÔÁ›·˜ ‹ ÛËÌÂÈÔÏÔÁ›·˜ (ÛÂ
‰‡Ô ·È‰È¿ Ë ‰È¿ÁÓˆÛË ÂÙ¤ıË ÛÙ· Ï·›ÛÈ· ‰ÈÂÚ‡ÓË-
Û˘ ÓÂÊÚˆÛÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘, Ô˘ ·ÔÙÂÏ› ·Ú·ÓÂ-
ÔÏ·ÛÌ·ÙÈ΋ ÂΉ‹ÏˆÛË Ù˘ ÓfiÛÔ˘ Hodgkin). ™Â 50
·È‰È¿ (68,5%) ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó Û˘ÓÔ‰¿ Û˘ÌÙÒ-
Ì·Ù·, ÂÓÒ ÛÙ· ˘fiÏÔÈ·, ÂÎÙfi˜ ÙÔ˘ ·ÚÈÔ˘ Û˘ÌÙÒ-
Ì·ÙÔ˜/ÛËÌ›Ԣ, Û˘Ó˘‹Ú¯·Ó ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤-
ÚÂÈ·, ‚‹¯·˜-Ï¢ÚÔ‰˘Ó›·, Â̇ÚÂÙÔ, ÏÂÌÊ·‰ÂÓÔ¿-
ıÂÈ· ‹ Û˘Ó‰˘·ÛÌfi˜ ÙˆÓ ·ÓˆÙ¤Úˆ. ™˘ÌÙÒÌ·Ù· Ù‡-
Ô˘ µ ‰È·ÈÛÙÒıËÎ·Ó Û 21 ·È‰È¿ (28,8%) Î·È ÂȉÈ-
ÎfiÙÂÚ· ·Ú·ÙÂÈÓfiÌÂÓÔ ˘Ú¤ÙÈÔ (¿Óˆ ÙˆÓ 10 ËÌÂ-
ÚÒÓ) ‰È·ÈÛÙÒıËΠ۠10, ·ÒÏÂÈ· ‚¿ÚÔ˘˜ >10% ÛÂ
10, ÂÊȉÚÒÛÂȘ Û 7 Î·È ÎÓËÛÌfi˜ Û ¿ÏÏ· 2. §ÂÌÊ·-
‰ÂÓÔ¿ıÂÈ· ηٿ ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËÎÂ
Û 68 ·ÛıÂÓ›˜ (93%). ™Â 12 ÂÍ ·˘ÙÒÓ ‹Ù·Ó ·ÌÊÔÙÂ-
ÚfiÏ¢ÚË Î·È Û 56 ÂÙÂÚfiÏ¢ÚË. ∂ÓÙfiÈÛË ÛÙÔÓ
ÙÚ¿¯ËÏÔ ‰È·ÈÛÙÒıËΠ۠55 ·È‰È¿, ÛÙÔ˘˜ ˘ÂÚ-
ÎÏ›‰ÈÔ˘˜ ‚fiıÚÔ˘˜ Û 41, ÛÙȘ Ì·Û¯·ÏÈ·›Â˜ ÎÔÈÏfiÙË-
Ù˜ Û 13 Î·È ÛÙË ‚Ô˘‚ˆÓÈ΋ ¯ÒÚ· Û 4. ∫·Ù¿ ÙË Ê˘-
ÛÈ΋ ÂͤٷÛË, Ë·ÙÔÌÂÁ·Ï›· ‰È·ÈÛÙÒıËΠ۠2 ·È-
‰È¿ Î·È ÛÏËÓÔÌÂÁ·Ï›· Û 5. ∆· ÂÚÁ·ÛÙËÚȷο ¢ڋ-
Ì·Ù· ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ ηٿ ÙË ‰È¿ÁÓˆÛË ÂÚÈÁÚ¿-
ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 2.
∞ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜
ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ ˘Ô‚Ï‹ıËÎ·Ó Û ·ÂÈÎÔÓÈÛÙÈΤ˜
ÂÍÂÙ¿ÛÂȘ ÁÈ· ÛÙ·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘. ∆Ô ˘ÂÚ˯Ô-
ÁÚ¿ÊËÌ· ÙÚ·¯‹ÏÔ˘ ‹Ù·Ó ıÂÙÈÎfi ÁÈ· Â˘Ú‹Ì·Ù· Û ·Ó·-
ÏÔÁ›· 86%, Ë ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ Û 43% Î·È Ë
˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ Û ·Ó·ÏÔÁ›·
67%. MÂÁ¿ÏË ‹ ̤ÙÚÈ· ‰È‡ڢÓÛË ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘
¶›Ó·Î·˜ 2. ∂ÚÁ·ÛÙËÚȷΤ˜ ·Ú¿ÌÂÙÚÔÈ ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÌÂϤÙ˘ ηٿ ÙË ‰È¿ÁÓˆÛË
™‡ÓÔÏÔ º‡ÏÔ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
¢∆* ŸÚÈ· ¢∆* ¢∆* ƒ
∏ÏÈΛ· (¤ÙË) 12 3,5-12 10,3 13 0,001WBC (x 103/mm3) 10,2 4,2-25 9,3 11,25 0,183¶ÔÏ˘ (%) 66 39-91 64,3 74 0,044∏ˆÛ (%) 3 0-11 3 2,5 0,768Hb (g/dl) 11,1 7,6-15 11,1 11,1 0,35∆∫∂ (mm 1˘ ÒÚ·˜) 61 5-150 52 82 0,123PLT (x 103/mm3) 383 200-820 367 387 0,614CRP (mg/l) 72 0-173 51,8 81 0,577LDH (mg/dl) 380 206-1300 381 335 0,672
*¢∆: ‰È¿ÌÂÛË ÙÈÌ‹
125§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿
¶·È‰È·ÙÚÈ΋ 2009;72:125-000
151413121110987654
%
20
10
0
∫ÔÚ›ÙÛÈ·
∞ÁfiÚÈ·
3
7
6
5
3
2
1 23
10
43
8
21
33
6
1
∏ÏÈΛ· (¤ÙË)
∂ÈÎfiÓ· 1. ¢ËÌÔÁÚ·ÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο.
¶›
19
19
19
* ·MABVBOPCO
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·125
‰È·ÈÛÙÒıËΠÛÂ Û˘¯ÓfiÙËÙ· 37% (7/19) Î·È 78%
(36/46) ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÈÛÙÔÏÔÁÈÎfi ˘fiÙ˘Ô ÌÈ-
ÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ Î·È Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘
·ÓÙ›ÛÙÔȯ·. ™Â ÂÚ›ÙˆÛË ‰È‡ڢÓÛ˘ ÌÂÛÔıˆÚ·Î›-
Ô˘ ηٷ‰ÂÈÎÓ˘fiÌÂÓ˘ Ì ˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›·
(100%), Ë ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· ·ÔÎ¿Ï˘Ù ÙË ‰È‡-
Ú˘ÓÛË ÛÙÔ 86%. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÎÔÈÏ›·˜ Î·È Ë
˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÎÔÈÏ›·˜ ·ÔÎ¿Ï˘„·Ó ·-
ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙÔ 49% Î·È 40%, ·ÓÙ›ÛÙÔȯ·.
∏·ÙÔÌÂÁ·Ï›· ‰È·ÈÛÙÒıËΠ۠7 ·È‰È¿, ÛÏËÓÔ-
ÌÂÁ·Ï›· Û 13, Ô˙Ò‰ÂȘ ‰ÈËı‹ÛÂȘ ¯·Ú·ÎÙËÚÈÛÙÈΤ˜
ÏÂÌÊÒÌ·ÙÔ˜ ∏odgkin ‰È·ÈÛÙÒıËÎ·Ó ÛÙÔ ‹·Ú Û 3
·È‰È¿, ÛÙÔ ÛÏ‹Ó· Û 14 ·È‰È¿, ÂÓÒ ·ÓÔÌÔÈÔÁ¤ÓÂÈ·
ÓÂÊÚÒÓ ‰È·ÈÛÙÒıËΠ۠¤Ó· ·È‰›. ™Â 12 ·È‰È¿
‰È·ÈÛÙÒıËÎÂ Û˘Ó‰˘·ÛÌfi˜ 2 ‹ 3 ÂÎ ÙˆÓ ·ÓˆÙ¤Úˆ
¢ÚËÌ¿ÙˆÓ, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ÏÂÌÊ·-
‰ÂÓÔ¿ıÂÈ·˜. ∆¤ÏÔ˜, ÏÂÌÊ·ÁÁÂÈÔÁÚ·Ê›· (ÎÏ·ÛÈ΋ ‹
Ú·‰ÈÔ˚ÛÔÙÔÈ΋) Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠17 ·È‰È¿
Î·È ‹Ù·Ó ·ıÔÏÔÁÈ΋ Û 6.
™Ù·‰ÈÔÔ›ËÛË
ªÂ ‚¿ÛË ÎÏÈÓÈο Î·È ·ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù·, 16
(22%) ·È‰È¿ Ù·ÍÈÓÔÌ‹ıËÎ·Ó ˜ ÛÙ·‰›Ô˘ π, 25 (34%)
ˆ˜ ÛÙ·‰›Ô˘ ππ (II∞: 11, IIB: 14), 20 (27,5%) ˆ˜ ÛÙ·‰›-
Ô˘ πππ (III∞: 6, IIIB: 14) Î·È 12 (16,5%) ˆ˜ ÛÙ·‰›Ô˘ IV
(IV∞: 5, IVB: 7).
™Â ¯ÂÈÚÔ˘ÚÁÈ΋ ÛÙ·‰ÈÔÔ›ËÛË Ì ÛÏËÓÂÎÙÔÌ‹
˘Ô‚Ï‹ıËÎ·Ó 3 ·È‰È¿ ÛÙ· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ÌÂϤ-
Ù˘. ¢È‹ıËÛË ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ‰È·ÈÛÙÒıËÎÂ
Ì ÔÛÙÂÔÌ˘ÂÏÈ΋ ‚ÈÔ„›· ÛÙÔ 5% ÙˆÓ ·È‰ÈÒÓ. ™Â
‚ÈÔ„›· ‹·ÙÔ˜ ˘Ô‚Ï‹ıËÎ·Ó 5 ·È‰È¿ Î·È Û fiÏ·
‹Ù·Ó ·ÚÓËÙÈ΋. ™˘ÌÌÂÙÔ¯‹ ·fi Ù· ÔÛÙ¿ ‰È·ÈÛÙÒ-
ıËΠ̠ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÔÛÙÒÓ Û 4/73 ·È‰È¿
Î·È ·ÂÈÎÔÓÈÛÙÈο ÔÚ·Ù¤˜ Ó¢ÌÔÓÈΤ˜ ‰ÈËı‹ÛÂȘ
ÛÂ 3/73.
πÛÙÔÏÔÁÈÎfi˜ Ù‡Ô˜
™ÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ·È‰ÈÒÓ (46/73, 63%) ‰È·-
ÈÛÙÒıËÎÂ Ô ÈÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜ Ù˘ Ô˙Ò‰Ô˘˜
ÛÎÏ‹Ú˘ÓÛ˘. √ ˘fiÙ˘Ô˜ Ù˘ ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›-
ıÂÈ·˜ ‰È·ÈÛÙÒıËÎÂ Û˘¯ÓfiÙÂÚ· Û ·ÛıÂÓ›˜ ÛÙ·‰›-
Ô˘ π & ππI, Ë Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË Û ·ÛıÂÓ›˜ ÛÙ·‰›-
Ô˘ ππ, Ô ÏÂÌÊÔÂÈÎÚ·ÙÒÓ Û ·ÛıÂÓ›˜ ÛÙ·‰›Ô˘ π, ÂÓÒ
Ù· ‰‡Ô ·È‰È¿ Ì ÏÂÌÊÔÂÓÈÎfi ˘fiÙ˘Ô ‹Ù·Ó ÛÙ·-
‰›Ô˘ πV (¶›Ó·Î·˜ 3). ∏ Û˘Û¯¤ÙÈÛË ÙÔ˘ ÈÛÙÔÏÔÁÈÎÔ‡
˘ÔÙ‡Ô˘ Î·È ÙÔ˘ ÛÙ·‰›Ô˘ Ù˘ ÓfiÛÔ˘ Ì ÂȉËÌÈÔ-
ÏÔÁÈο Î·È ÂÚÁ·ÛÙËÚȷο ‰Â‰Ô̤ӷ ÂÚÈÁÚ¿ÊÂÙ·È
ÛÙÔÓ ¶›Ó·Î· 4.
¶›Ó·Î·˜ 4. ™˘Û¯¤ÙÈÛË ÂȉËÌÈÔÏÔÁÈÎÒÓ Î·È ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ·Ú·Ì¤ÙÚˆÓ Ì ÙÔ ÛÙ¿‰ÈÔ Î·È ÙËÓ ÈÛÙÔÏÔÁ›·
™Ù¿‰ÈÔ πÛÙÔÏÔÁ›·***π ππ πππ πV P ª §¶ §∂ √™ ƒ
º‡ÏÔ (·Á:ÎÔ)* 13:3 13:12 12:8 8:4 0,393 18:1 2:0 6:0 20:26 0,000∏ÏÈΛ· (¤ÙË)** 9,3 12,1 12,1 13 0,102 10 11,6 10,5 12,7 0,017WBC (x 103/mm3)** 8,75 12,19 9 10,4 0,027 8,4 4,7 8,5 11,4 0,007¶ÔÏ˘ (%)** 55 74 62 77 0,002 63 60,5 57 70 ns#
∏ˆÛ (%)** 3 2 3 2,4 0,173 2,8 2,5 3 3 nsHb (g/dl)** 12,9 11,1 11,1 10,9 0,014 11,1 10,6 13,8 11,1 nsPLT (x 103/mm3)** 200 400 403 383 0,022 367 384 301 393 ns∆∫∂ (mm 1˘ ÒÚ·˜)** 16,5 70 75 115 0,001 59 65 16 70 nsCRP (mg/l)** 20 57 80 110 0,398 57 - 27 74 nsLDH (mg/dl)** 498 330 380 404 0,44 323 - 380 383 ns
* ·Á: ·ÁfiÚÈ·, ÎÔ: ÎÔÚ›ÙÛÈ·** ‰È¿ÌÂÛ˜ ÙÈ̤˜
*** ª: ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜, §¶: ÏÂÌÊÔÂÓÈÎfi˜, §∂: ÏÂÌÊÔÂÈÎÚ·ÙÒÓ, √™: Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘# ÌË ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿, ¶√§À: ÔÏ˘ÌÔÚÊÔ‡ÚËÓ·, ∏ø™: ˈÛÈÓfiÊÈÏ·
126 ∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:126-000
¶›Ó·Î·˜ 3. πÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜ Î·È ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘
™‡ÓÔÏÔ ™Ù¿‰ÈÔπ ππ πππ IV
ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ 19* (26)** 6 (37,5) 4 (16) 7 (35) 2 (16,7)§ÂÌÊÔÂÓÈÎfi˜ 2 (2,7) - - - 2 (16,7)§ÂÌÊÔÂÈÎÚ·ÙÒÓ 6 (8,2) 4 (25) 1 (4) 1 (5) -√˙҉˘ ÛÎÏ‹Ú˘ÓÛË 46 (63) 6 (37,5) 20 (80) 12 (60) 8 (66,6)™‡ÓÔÏÔ 73 (100) 16 (22) 25 (34) 20 (27,5) 12 (16,5)
*·ÚÈıÌfi˜ ·ÛıÂÓÒÓ, **(%) ·Ó·ÏÔÁ›· ·ÛıÂÓÒÓ
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·126
£Âڷ›·
∏ ıÂڷ›· ÎÚ›ıËΠˆ˜ ·Ú΋˜ Û 68 ·ÛıÂÓ›˜
Î·È ·Ó·Ú΋˜ Û 5. ™Â 68 ·È‰È¿ Ô˘ ¤Ï·‚·Ó ·ÎÙÈ-
ÓÔıÂڷ›·, ·˘Ù‹ ‹Ù·Ó ·Ú΋˜ Û 66. ™Â fiÏÔ˘˜
ÙÔ˘˜ ·ÛıÂÓ›˜ Ô˘ ¤Ï·‚·Ó ∞£, ·˘Ù‹ ‹Ù·Ó ÂÛÙȷ΋,
ÂÓÒ ÔÈ 56 ÂÍ ·˘ÙÒÓ ¤Ï·‚·Ó 20 Gy, ÔÈ 4 30 Gy Î·È ÔÈ 8
40 Gy ÛÙ· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ÌÂϤÙ˘.
ŒÎ‚·ÛË
™ÙÔ˘˜ 73 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘ ÛËÌÂÈÒıËÎ·Ó 8
˘ÔÙÚÔ¤˜ (11%), 20-65 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË (¢∆
30). ∆· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ·ÚÔ˘Û›·-
Û·Ó ˘ÔÙÚÔ‹ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 5. ∏ ·Ú-
¯È΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ˘ÔÙÚÔ‹˜ ÂÚÈÂÏ¿Ì‚·ÓÂ
ÌfiÓÔ Ã£ ‰Â‡ÙÂÚ˘ ÁÚ·ÌÌ‹˜ Û 3 ÂÚÈÙÒÛÂȘ ηÈ
Û˘Ó‰˘·ÛÌfi ã & ∞£ Û 5. ™Â 6/8 ·È‰È¿ ¤ÁÈÓ ηÈ
·˘ÙfiÏÔÁË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ. ∞fi Ù·
8 ·È‰È¿ Ô˘ ·ÚÔ˘Û›·Û·Ó ˘ÔÙÚÔ‹ ˙Ô˘Ó Ù· 7, 12-
270 Ì‹Ó˜ (¢∆ 95) ·fi ÙË ‰È¿ÁÓˆÛË Ù˘ ˘ÔÙÚÔ‹˜
Î·È 32-310 Ì‹Ó˜ (¢∆ 125) ·fi ÙË ‰È¿ÁÓˆÛË Ù˘ Ófi-
ÛÔ˘ (ÂÈ‚›ˆÛË Û 2Ë ‡ÊÂÛË: 89%). ¢Â‡ÙÂÚË ‹ ÙÚ›ÙË
ηÎÔ‹ıÂÈ· ‰È·ÈÛÙÒıËΠ۠4 ·È‰È¿, ·fi Ù· ÔÔ›·
˙Ô˘Ó Ù· 3. ∆· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ÂÌ-
Ê¿ÓÈÛ·Ó ‰Â‡ÙÂÚË ‹ ÙÚ›ÙË Î·ÎÔ‹ıÂÈ· ÂÚÈÁÚ¿ÊÔÓÙ·È
ÛÙÔÓ ¶›Ó·Î· 6. ™˘ÓÔÏÈο ˙Ô˘Ó 71/73 ·ÛıÂÓ›˜ (Û˘ÓÔ-
ÏÈ΋ ÂÈ‚›ˆÛË: 97,3%), 24-366 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆ-
ÛË (¢∆ 156 Ì‹Ó˜) (ÂÈ‚›ˆÛË ÂχıÂÚË Û˘Ì‚¿Ì·ÙÔ˜:
83,6% - 61/73) (∂ÈÎfiÓ˜ 2, 3). ∆· 64 ·fi Ù· 73 ·È‰È¿
(87,7%) ˙Ô˘Ó Û ϋÚË ÚÒÙË ‡ÊÂÛË.
¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜
∏ Û˘Û¯¤ÙÈÛË ÂȉËÌÈÔÏÔÁÈÎÒÓ, ÎÏÈÓÈÎÒÓ, ·ÂÈ-
ÎÔÓÈÛÙÈÎÒÓ, ÂÚÁ·ÛÙËÚÈ·ÎÒÓ Î·È ıÂڷ¢ÙÈÎÒÓ ·-
ڷ̤ÙÚˆÓ Ì ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘ ÂÚÈÁÚ¿ÊÂÙ·È
ÛÙÔÓ ¶›Ó·Î· 7.
™˘˙‹ÙËÛË
∆Ô Ï¤Ìʈ̷ ‹ ÓfiÛÔ˜ ÙÔ˘ Hodgkin ÂÚÈÁÚ¿ÊËÎÂ
¶›Ó·Î·˜ 6. ÷ڷÎÙËÚÈÛÙÈο ·ÛıÂÓÒÓ Ì ‰Â‡ÙÂÚË ‹ ÙÚ›ÙË Î·ÎÔ‹ıÂÈ·
Ã√ ºÀ ∏§π ™∆ π™∆ £Âڷ›· ∂›‰Ô˜ ∂›‰Ô˜ ∞£ ŒÎ‚·ÛË ÃÚfiÓԘ·Ú΋˜ ıÂڷ›·˜ ã ∂·Ú΋˜ ¢fiÛË ∂ÓÙfiÈÛË
1 204* ∞° 13** π §∂ ¡∞π ∞£ ¡∞π 40Gy ∂ÛÙȷ΋ ¢ÂÓ ˙ÂÈ2 144 ∞° 4,9 π √™ ¡∞π ã & ∞£ ∞µVD ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 178*3 48 ∫√ 12,2 ππ √™ ¡∞π ã & ∞£ VBVP + OPPA ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 614 71 ∫√ 11,2 ππ √™ ¡∞π ã & ∞£ ABVD + MOPP ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 175
Ã√: ¯ÚfiÓÔ˜ ·fi ÙË ‰È¿ÁÓˆÛË, ºÀ: ʇÏÔ, ∞°: ·ÁfiÚÈ·, ∫√: ÎÔÚ›ÙÛÈ·, ∏§π: ËÏÈΛ·, ™∆: ÛÙ¿‰ÈÔ, I™∆: ÈÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜, §∂:ÏÂÌÊÔÂÈÎÚ·ÙÒÓ, √™: Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË*Ì‹Ó˜ ·fi ‰È¿ÁÓˆÛË, **¤ÙË1. ∞‰ÂÓÔηÚΛӈ̷ ÛÙÔÌ¿¯Ô˘ (2Ë) Î·È Î·ÚΛÓÔ˜ Ì·ÛÙÔ‡ (3Ë) 228 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË, ÏfiÁˆ ÙÔ˘ ÔÔ›Ô˘ η٤ÏËÍÂ2. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜3. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜4. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜ (2Ë) Î·È ÔÛÙÂÔÛ¿ÚΈ̷ (3Ë) 94 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË
127§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿
¶·È‰È·ÙÚÈ΋ 2009;72:127-000
¶›Ó·Î·˜ 5. ÷ڷÎÙËÚÈÛÙÈο ·ÛıÂÓÒÓ Ì ˘ÔÙÚÔ‹
Ã√ ºÀ ∏§π ™∆ π™∆ £Âڷ›· ∂›‰Ô˜ ∂›‰Ô˜ ∞£ ŒÎ‚·ÛË ÃÚfiÓԘ·Ú΋˜ ıÂڷ›·˜ ã ∂·Ú΋˜ ¢fiÛË ∂ÓÙfiÈÛË
1 20* ∫√ 13.5** πV √™ ¡∞π ã & ∞£ OPPA + ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 32*COPP
2 30 ∞° 4.5 π ª∫ ¡∞π ∞£ ¡∞π 40 Gy ∂ÛÙȷ΋ ∑ÂÈ 2533 24 A° 8 ππ ª∫ ¡∞π ã & ∞£ VBVP ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 904 40 ∫√ 14.2 πππ √™ √Ãπ ã §√π¶∞ ¢ÂÓ ˙ÂÈ5 40 ∫√ 14 πππ √™ √Ãπ ã MOPP ∑ÂÈ 3106 30 ∞° 13.2 πππ √™ ¡∞π ã & ∞£ ABVD + ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 125
MOPP7 25 ∞° 6 πV √™ √Ãπ ã & ∞£ ABVD + ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 223
MOPP8 65 ∞° 14 πV √™ √Ãπ ã & ∞£ OPPA + √Ãπ 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 78
COPP
Ã√: ¯ÚfiÓÔ˜ ·fi ÙË ‰È¿ÁÓˆÛË, ºÀ: ʇÏÔ, ∞°: ·ÁfiÚÈ·, ∫√: ÎÔÚ›ÙÛÈ·, ∏§π: ËÏÈΛ· ™∆: ÛÙ¿‰ÈÔ. I™∆: ÈÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜, √™: Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË, ª∫: ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜, §∂: ÏÂÌÊÔÂÈÎÚ·ÙÒÓ*Ì‹Ó˜ ·fi ‰È¿ÁÓˆÛË, **¤ÙË
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·127
ÁÈ· ÚÒÙË ÊÔÚ¿ ·fi ÙÔÓ Thomas Hodgkin ÙÔ 1932.
∏ ·ÈÙÈÔÏÔÁ›· Ù˘ ÓfiÛÔ˘ ·Ú·Ì¤ÓÂÈ ·‰È¢ÎÚ›ÓÈÛÙË.
∞ÔÙ¤ÏÂÛ ÁÈ· ÔÏϤ˜ ‰ÂηÂٛ˜ ·ÓÙÈΛÌÂÓÔ ÂÈ-
‰ËÌÈÔÏÔÁÈÎÒÓ ÂÚ¢ÓÒÓ, Ù· Â˘Ú‹Ì·Ù· ÙˆÓ ÔÔ›ˆÓ
Ê·›ÓÂÙ·È Ó· Ô‰ÂÈÎÓ‡Ô˘Ó ‰È·ÊÔÚÂÙÈÎÔ‡˜ ·ÈÙÈÔÏÔÁÈ-
ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ·. ™‡Ìʈӷ
Ì ÙÔ ™‡ÛÙËÌ· ∆·ÍÈÓfiÌËÛ˘ ÙÔ˘ ¶·ÁÎfiÛÌÈÔ˘ √ÚÁ·-
ÓÈÛÌÔ‡ ÀÁ›·˜ (¶√À), ηıÔÚ›˙ÔÓÙ·È ‰‡Ô ·ÚÈÔÈ Ù‡-
ÔÈ ÏÂÌÊˆÌ¿ÙˆÓ Hodgkin, ηı¤Ó·˜ ·fi ÙÔ˘˜ ÔÔ›-
Ô˘˜ ¤¯ÂÈ ÂȉÈο ÎÏÈÓÈο Î·È ÚÔÁÓˆÛÙÈο ¯·Ú·ÎÙËÚÈ-
ÛÙÈο: Ë ÎÏ·ÛÈ΋ ÓfiÛÔ˜ (Classical Hodgkin’s
lymphoma, CHL) Î·È Ô Ù‡Ô˜ Ô˙Ò‰Ô˘˜ ÏÂÌÊÔ΢Ù-
Ù·ÚÈ΋˜ ÂÈÎÚ¿ÙËÛ˘ (Nodular lymphocyte
predominance Hodgkin’s lymphoma, NLPHL) (11).
√È ÎÏ·ÛÈÎÔ› ˘fiÙ˘ÔÈ Ù˘ ÓfiÛÔ˘ Û˘Ó‰¤ÔÓÙ·È Û˘Ó‹-
ıˆ˜ Ì ÛÙ¿‰ÈÔ ππ-πππ (63-78% ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ηÈ
62% ÛÙË ÌÂϤÙË Ì·˜). O Ù‡Ô˜ Ù˘ Ô˙Ò‰Ô˘˜ ÏÂÌÊÔ-
΢ÙÙ·ÚÈ΋˜ ÂÈÎÚ¿ÙËÛ˘ ··ÓÙ¿Ù·È Û ËÏÈ˘ 25-
45 ÂÙÒÓ, Ì ÂÈÎÚ¿ÙËÛË ÙÔ˘ ¿ÚÚÂÓÔ˜ ʇÏÔ˘, ηÈ
·ÔÙÂÏ› ÙÔ 5% ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Hodgkin (12).
∞fi ÙÔ ÎÏ·ÛÈÎfi ϤÌʈ̷ Hodgkin, Ô ÏÂÌÊÔÂÈÎÚ·-
ÙÒÓ ˘fiÙ˘Ô˜ ‰È·ÈÛÙÒÓÂÙ·È ÛÙÔ 10-15% ÙˆÓ ·ÛıÂ-
ÓÒÓ, Â›Ó·È ÈÔ Û˘¯Ófi˜ ÛÙÔ ¿ÚÚÂÓ Ê‡ÏÔ Î·È ÙÔ˘˜ ÌÈ-
ÎÚfiÙÂÚÔ˘˜ Û ËÏÈΛ· ·ÛıÂÓ›˜, ÂÓÒ Û˘Ó‰¤ÂÙ·È Û˘-
¯ÓfiÙÂÚ· Ì ÂÓÙÔÈṲ̂ÓË ÓfiÛÔ (3). √ ÏÂÌÊÔÂÓÈÎfi˜
˘fiÙ˘Ô˜ Â›Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈÔ˜ ÛÙ· ·È‰È¿
(~5%), ÂÓÒ Û˘Ó‰¤ÂÙ·È Û˘¯Ó¿ Ì HIV Ïԛ̈ÍË.
∞ÛıÂÓ›˜ Ì ·˘ÙfiÓ ÙÔÓ Ù‡Ô Û˘Ó‹ıˆ˜ ¤¯Ô˘Ó Û˘-
ÌÙÒÌ·Ù· Ù‡Ô˘ µ, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ٷ¯¤ˆ˜ ÂÍÂ-
ÏÈÛÛfiÌÂÓË ÓfiÛÔ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· ÔÛÙ¿ Î·È ÙÔÓ
Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Î·È ·ÚÔ˘ÛÈ¿˙ÂÈ ÙË ¯ÂÈÚfiÙÂÚË
ÚfiÁÓˆÛË ·fi fiÏÔ˘˜ ÙÔ˘˜ ˘fiÙ˘Ô˘˜ (3,5). √ ˘fi-
Ù˘Ô˜ Ù˘ ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ ÂÌÊ·Ó›˙ÂÙ·È ÛÙÔ
14-38% ÙˆÓ ·ÛıÂÓÒÓ (5,6,11), ‰È·ÈÛÙÒÓÂÙ·È Û˘¯Ófi-
ÙÂÚ· Û ·È‰È¿ 10 ÂÙÒÓ ‹ ÌÈÎÚfiÙÂÚ· Î·È ÂÌÊ·Ó›˙ÂÙ·È
Û˘¯Ó¿ ˆ˜ ÚÔ¯ˆÚË̤ÓË ÓfiÛÔ˜ Ì Â͈ÏÂÌÊ·‰ÂÓÈ΋
ÂÓÙfiÈÛË. ∏ Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË ·ÔÙÂÏ› ÙÔÓ ÈÔ
Û˘¯Ófi ˘fiÙ˘Ô ÛÙȘ ·ÓÂÙ˘Á̤Ó˜ ¯ÒÚ˜ Î·È ÂÌÊ·-
Ó›˙ÂÙ·È Û˘¯ÓfiÙÂÚ· ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ Î·È ÙÔ˘˜ Ó·ÚÔ‡˜
ÂÓ‹ÏÈΘ. ¶·Ú·ÙËÚÂ›Ù·È ÛÙÔ 40% ÙˆÓ ÓÂfiÙÂÚˆÓ
·ÛıÂÓÒÓ Î·È ÛÙÔ 70% ÙˆÓ ÂÊ‹‚ˆÓ. ∞ÔÙÂÏ› ÙÔÓ
ÌfiÓÔ ˘fiÙ˘Ô ÛÙÔÓ ÔÔ›Ô ‰ÂÓ ÂÈÎÚ·Ù› ÙÔ ¿ÚÚÂÓ
ʇÏÔ, ηıÒ˜ ‰È·ÈÛÙÒÓÂÙ·È Ì ›ÛË Û˘¯ÓfiÙËÙ· Û ¿Ú-
ÚÂÓ· Î·È ı‹Ï· (13). °ÂÓÈο, Ô ÏÂÌÊÔÂÈÎÚ·ÙÒÓ
˘fiÙ˘Ô˜ Î·È ·˘Ùfi˜ Ù˘ Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘ Û¯Â-
Ù›˙ÔÓÙ·È Ì ÓfiÛÔ ¯·ÌËÏÔ‡ ÛÙ·‰›Ô˘, Ô ÏÂÌÊÔÂÓÈÎfi˜
Ì ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È·, ÂÓÒ Ô ˘fiÙ˘Ô˜ Ù˘ ÌÈ-
ÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ Ì ÂӉȿÌÂÛ·. ™ÙÔ˘˜ ·ÛıÂ-
Ó›˜ Ì·˜ ÂÈÎÚ·Ù› Ë Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË (63%), ÂÓÒ
ÌfiÓÔ Û ‰‡Ô ·È‰È¿ ‰È·ÈÛÙÒıËΠÏÂÌÊÔÂÓÈÎfi˜
˘fiÙ˘Ô˜. ∆¤ÏÔ˜, fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ¤Î‚·ÛË ‰ÂÓ ‰È·-
ÈÛÙÒıËΠ›‰Ú·ÛË Ù˘ ÈÛÙÔÏÔÁ›·˜, ÛÂ Û˘Ìʈӛ·
Ì ٷ Â˘Ú‹Ì·Ù· ¿ÏÏˆÓ ÌÂÏÂÙÒÓ (5,6). º·›ÓÂÙ·È ˆ˜
ÌÂÙ¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙˆÓ Û‡Á¯ÚÔÓˆÓ Û¯ËÌ¿ÙˆÓ ËÌÂÈ-
ÔıÂڷ›·˜, Ù· ÈÛÙÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο ¤¯Ô˘Ó
ÌÈÎÚfiÙÂÚË ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· (14).
∏ ‰È¿ÌÂÛË ÙÈÌ‹ ËÏÈΛ·˜ ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÌÂϤ-
Ù˘ Ì·˜ ‹Ù·Ó 12 ¤ÙË, ÂÓÒ ·ÍÈÔÛËÌ›ˆÙË Â›Ó·È Ë Û˘-
¯ÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ ËÏÈΛ·˜ 5 ÂÙÒÓ ‹ ÌÈÎÚfiÙÂÚ˘
(9,6%, 7 ·ÛıÂÓ›˜). ∆Ô ÔÛÔÛÙfi ·È‰ÈÒÓ Ì ËÏÈΛ·
οو ÙˆÓ 5 ÂÙÒÓ ÛÙËÓ ÌÂϤÙË Ì·˜ Â›Ó·È ˘„ËÏfiÙÂÚÔ
·fi ·ÓÙ›ÛÙÔȯ· Û ·ÓÂÙ˘Á̤Ó˜ ¯ÒÚ˜ (4), ·ÏÏ¿
ÚÔÛÂÁÁ›˙ÂÈ ‹ Û·ÊÒ˜ ÔÏ›ÂÙ·È ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô Ô-
ÛÔÛÙfi ¯ˆÚÒÓ Ù˘ ¢˘ÙÈ΋˜ ∞Û›·˜ Î·È ¿ÏÏˆÓ ·Ó·-
Ù˘ÛÛfiÌÂÓˆÓ ¯ˆÚÒÓ (15,16). ø˜ ÚÔ˜ ÙÔ Ê‡ÏÔ, ÛÙË
‚È‚ÏÈÔÁÚ·Ê›· ‰È·ÈÛÙÒÓÂÙ·È ÌÈ· ÂÏ·ÊÚ¿ ÂÈÎÚ¿ÙË-
ÛË ÙˆÓ ·ÚÚ¤ÓˆÓ (1.5:1), Ô˘ fï˜ Êı›ÓÂÈ Ì ÙËÓ ¿-
ÚÔ‰Ô Ù˘ ËÏÈΛ·˜, Ì ÙËÓ ·Ó·ÏÔÁ›· Ó· Â›Ó·È ·Úfi-
ÌÔÈ· ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ (3). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ‰È·-
ÈÛÙÒÓÂÙ·È Û·Ê‹˜ ÂÈÎÚ¿ÙËÛË ÙÔ˘ ¿ÚÚÂÓÔ˜ ʇÏÔ˘,
Ì ٷ ÎÔÚ›ÙÛÈ· Ó· ¤¯Ô˘Ó Û ÛËÌ·ÓÙÈÎfi ‚·ıÌfi ÌÂÁ·-
χÙÂÚË ËÏÈΛ·. ÷ڷÎÙËÚÈÛÙÈο ‰È·ÈÛÙÒÓÔ˘Ì ˆ˜
128 ∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:128-000
ª‹Ó˜4003002001000
¯1
00
%1,00
0,80
0,60
0,40
0,20
0,00
∂ÈÎfiÓ· 2. ∫·Ì‡ÏË Û˘ÓÔÏÈ΋˜ ÂÈ‚›ˆÛ˘.
ª‹Ó˜4003002001000
¯1
00
%
1,00
0,80
0,60
0,40
0,20
0,00
∂ÈÎfiÓ· 3. ∫·Ì‡ÏË ÂÈ‚›ˆÛ˘ ÂχıÂÚ˘ Û˘Ì‚¿Ì·ÙÔ˜.
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·128
fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ ËÏÈΛ·˜ 7 ÂÙÒÓ ‹ ÌÈÎÚfiÙÂÚ˘ ‹Ù·Ó
·ÁfiÚÈ· (∂ÈÎfiÓ· 1). ∏ ÓfiÛÔ˜ ÛÙ· ·È‰È¿ ¤¯ÂÈ Û˘Ó‰Âı›
Ì ˘„ËÏfi ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎfi Â›Â‰Ô (ÛÙȘ ‚ÈÔ-
Ì˯·ÓÈΤ˜ ¯ÒÚ˜), ·ÏÏ¿ Î·È Ì ¤ÎıÂÛË Û ÏÔÈÌÒ‰ÂȘ
·Ú¿ÁÔÓÙ˜ (EBV, ÂÚËÙÔ˚fi˜ 6, CMV) ‹ Ì ·ÓÔÛÔ·-
Ó¿ÚÎÂÈ· (3). ™ËÌ·ÓÙÈ΋ ·Ó·ÏÔÁ›· ÙˆÓ ·ÛıÂÓÒÓ ÌÂ
ÓfiÛÔ ∏odgkin ¤¯Ô˘Ó ˘„ËÏÔ‡˜ Ù›ÙÏÔ˘˜ ·ÓÙÈۈ̿وÓ
¤Ó·ÓÙÈ ÙÔ˘ EBV (17). ™ÙËÓ ÂÚ›ÙˆÛË Ù˘ ÎÏ·ÛÈ΋˜
ÓfiÛÔ˘ Hodgkin, ÛÙȘ ¢˘ÙÈΤ˜ ¯ÒÚ˜ Ë Û‡Ó‰ÂÛË ÌÂ
EBV Ïԛ̈ÍË Êı¿ÓÂÈ ÙÔ 50% (18). ™ÙÔ˘˜ ·ÛıÂÓ›˜
Ì·˜, Ë ·Ó·ÏÔÁ›· ·˘ÙÒÓ Ì ·ÚÔ˘Û›· EBV-IgG ¤Êı·-
Ó ÙÔ 41%, ¯ˆÚ›˜ Ó· ·Ô‰›‰Ô˘Ì ȉȷ›ÙÂÚË ÛËÌ·Û›·
ÛÙÔ Â‡ÚËÌ¿ Ì·˜, ηıÒ˜ ˘„ËÏfi ÔÛÔÛÙfi ·È‰ÈÒÓ Î·È
ÂÊ‹‚ˆÓ ÛÙË ¯ÒÚ· Ì·˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ÓÙÈÛÒÌ·Ù·
¤Ó·ÓÙÈ ÙÔ˘ EBV. ∂›Û˘, Ë ÓfiÛÔ˜ ∏odgkin ¤¯ÂÈ Û˘Ó-
‰Âı› Î·È Ì ·˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù· (19).
∆Ô Û˘¯ÓfiÙÂÚÔ ÎÏÈÓÈÎfi ÛËÌÂ›Ô Ù˘ ÓfiÛÔ˘
Hodgkin Â›Ó·È Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, Ì ·ÚÔ˘Û›· ÛÙÔ
90% ÙˆÓ ·ÛıÂÓÒÓ (20), ·Ó Î·È Û¯Â‰fiÓ Î¿ı fiÚÁ·ÓÔ
ÌÔÚ› Ó· ÚÔÛ‚ÏËı›. ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ‰È·È-
ÛÙÒıËΠÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· Û ÔÛÔÛÙfi 93%. ªË ÂÈ-
‰Èο Û˘ÌÙÒÌ·Ù· Ù˘ ÓfiÛÔ˘ ·ÔÙÂÏÔ‡Ó Ë Î·ÎÔ˘¯›·,
Ë ·ÓÔÚÂÍ›·, ÙÔ ·Ú·ÙÂÈÓfiÌÂÓÔ ˘Ú¤ÙÈÔ Î·È Ë ·Ò-
ÏÂÈ· ‚¿ÚÔ˘˜ (3). ∂›Û˘, ÌÔÚ› Ó· Û˘Ó˘¿Ú¯ÂÈ ÌË
·Ú·ÁˆÁÈÎfi˜ ¯ÚfiÓÈÔ˜ ‚‹¯·˜ ‹ ¿ÏÏ· Û˘ÌÙÒÌ·Ù·
·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi, ÏfiÁˆ Ù˘ ‰È‡ڢÓÛ˘ ÙÔ˘ ÌÂ-
ÛÔıˆÚ·Î›Ô˘, Ô˘ ÚÔηÏ› ›ÂÛË Ù˘ ÙÚ·¯Â›·˜ ηÈ
ÙˆÓ ‚ÚfiÁ¯ˆÓ. ∏ ·ÚÔ˘Û›· µ-Û˘Ìو̿وÓ, fiˆ˜
·Ó·Ê¤ÚıËΠ‹‰Ë, ıˆÚÂ›Ù·È ˆ˜ ¤¯ÂÈ ÛËÌ·ÓÙÈ΋
ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· Î·È Û˘Ó‰¤ÂÙ·È Ì ÂÈıÂÙÈÎfi-
ÙÂÚË ÓfiÛÔ (21). ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ·Ó·Ê¤ÚıËηÓ
Û ·Ó·ÏÔÁ›· 29%, Ì ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚË ·Ó·ÏÔ-
Á›· Û ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È· (44% Û ÛÙ¿‰ÈÔ πππ ‹
IV), ·ÏÏ¿ ‰ÂÓ ‰È·ÈÛÙÒıËÎÂ Û˘Û¯¤ÙÈÛË Ì ÙËÓ ¤Î‚·-
ÛË Ù˘ ÓfiÛÔ˘, ÛÂ Û˘Ìʈӛ· Ì ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜
(5). ∞ÓÙ›ıÂÙ·, ÔÈ Smith Î·È Û˘Ó. ‰È·›ÛÙˆÛ·Ó Û˘Û¯¤-
ÙÈÛË Ù˘ ·ÚÔ˘Û›·˜ µ-Û˘ÌÙˆÌ¿ÙˆÓ Ì ‰˘ÛÌÂÓ¤-
ÛÙÂÚË ÚfiÁÓˆÛË (13). ™ÙË ÌÂϤÙË Ì·˜ ‰ÂÓ ÚԤ΢-
„Â Û˘Û¯¤ÙÈÛË ÂÚÁ·ÛÙËÚÈ·ÎÒÓ Â˘ÚËÌ¿ÙˆÓ Î·Ù¿ ÙË
‰È¿ÁÓˆÛË, fiˆ˜ Ï¢ÎÔ΢ÙÙ¿ÚˆÛË, ˈÛÈÓÔÊÈÏ›·,
129§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿
¶·È‰È·ÙÚÈ΋ 2009;72:129-000
¶›Ó·Î·˜ 7. ¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ˆ˜ ÚÔ˜ ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘
∞ÚÈıÌfi˜ % ƒ·ÛıÂÓÒÓ % DFS EFS OS DFS EFS OS
∏ÏÈΛ· (¤ÙË≤ 8 15 20,5 80 73,3 100 ns ns ns> 8 58 79,5 91,4 86,2 96,6
º‡ÏÔ∞ÁfiÚÈ· 46 63 89,1 84,8 97,8 ns ns ns∫ÔÚ›ÙÛÈ· 27 37 88,9 81,5 96,3
™Ù¿‰ÈÔπ 16 22 93,8 81,3 93,8ππ 25 34,2 96 92 100 ns ns nsπππ 20 27,4 85 80 95IV 12 16,4 75 75 100
πÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ 19 26,1 89,5 89,5 100§ÂÌÊÔÂÓÈÎfi˜ 2 2,7 100 100 100 ns ns ns§ÂÌÊÔÂÈÎÚ·ÙÒÓ 6 8,2 100 83,3 83,3√˙҉˘ ÛÎÏ‹Ú˘ÓÛË 46 63 87 80,4 97,8
µ-Û˘ÌÙÒÌ·Ù·¶·ÚfiÓÙ· 21 29 90,5 90,5 95,2 ns ns ns∞fiÓÙ· 52 71 88,5 80,8 98,1
ªÂÛÔıˆÚ¿ÎÈÔªÂÁ¿ÏË ‰È‡ڢÓÛË 13 17,8 92,3 84,6 100ª¤ÙÚÈ· ‰È‡ڢÓÛË 33 45,2 87,9 84,8 97 ns ns nsŸ¯È ‰È‡ڢÓÛË 27 37 88,9 81,5 96,3
§Â˘Î¿ ·ÈÌÔÛÊ·›ÚÈ· (x103/mm3)≤10,2 34 50,7 88,2 82,4 94,1 ns ns ns>10,2 33 49,3 90,9 84,8 100
Hb (g/dl)≤11,1 36 53 88,9 80,6 100 ns ns ns>11,1 32 47 90,6 87,5 93,8
TKE (mm 1˘ ÒÚ·˜)≤61 34 50 88,2 82,4 94,1 ns ns ns>61 34 50 91,2 85,3 100
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·129
·Ó·ÈÌ›·, ·˘ÍË̤ÓË ÙÈÌ‹ ∆∫∂ Î·È ·˘ÍË̤ÓË ÙÈÌ‹
LDH, Ì ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘. ™Â Û¯¤ÛË Ì ٷ
·ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù·, Ë ¤Î‚·ÛË ‰ÂÓ ‰È·ÈÛÙÒıË-
Πӷ ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ·ÚÔ˘Û›· ‰È‡ڢÓÛ˘
ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘, ·ÎfiÌË Î·È ÂÎÙÂٷ̤Ó˘, ·Ó ηÈ
ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È ˆ˜ Ì¿˙· ÌÂÛÔıˆÚ·-
ΛԢ Ô˘ ˘ÂÚ‚·›ÓÂÈ ÙÔ 1/3 Ù˘ ÌÂÁ·Ï‡ÙÂÚ˘ ÂÓ‰Ô-
ıˆÚ·ÎÈ΋˜ ‰È·Ì¤ÙÚÔ˘ Û˘Ó‰¤ÂÙ·È Ì ÚÔ¯ˆÚË̤ÓË
ÓfiÛÔ, Ùˆ¯fiÙÂÚË ÚfiÁÓˆÛË Î·È ·Ó¿ÁÎË ÂÈıÂÙÈÎfi-
ÙÂÚ˘ ıÂڷ›·˜ (22).
™˘ÓÔÏÈο, Ë ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ ‹Ù·Ó ÂÍ·È-
ÚÂÙÈ΋ Û fiϘ ÙȘ ¯ÚÔÓÈΤ˜ ÂÚ›Ô‰Ô˘˜ ·ÓÙÈÌÂÙÒÈ-
Û˘ ·˘ÙÒÓ Î·È ·ÓÂÍ¿ÚÙËÙË ·fi ÙÔ Â›‰Ô˜ Ù˘ ıÂڷ›-
·˜. ∏ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË Êı¿ÓÂÈ ÙÔ 97,3%, ÂÓÒ ÂÍ·È-
ÚÂÙÈ΋ Â›Ó·È Î·È Ë ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ˘ÔÙÚÔ‹
- ÂÈ‚ÈÒÓÔ˘Ó ÔÈ 7/8. ∏ ÂÈ‚›ˆÛË ÌÂÙ¿ ·fi ˘ÔÙÚÔ‹
ÁÂÓÈο Î˘Ì·›ÓÂÙ·È ÛÙÔ 50-80% (3). ™ÙË ÌÂϤÙË Ì·˜, ÔÈ
˘ÔÙÚÔ¤˜ ¤Ï·‚·Ó ¯ÒÚ· 20-65 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆ-
ÛË. √È ˘ÔÙÚÔ¤˜ Û˘Ì‚·›ÓÔ˘Ó Û˘Ó‹ıˆ˜ Ù· ÚÒÙ· 3
¤ÙË, ·ÏÏ¿ ÔÚÈṲ̂ÓÔÈ ·ÛıÂÓ›˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Î·È fi„È-
̘ ˘ÔÙÚÔ¤˜ (¿Óˆ ·fi 10 ¤ÙË) (23).
√È ·ÏÏ·Á¤˜ ÛÙÔÓ ÙÚfiÔ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ÓfiÛÔ˘
‰È·¯ÚÔÓÈο ÔÊ›ÏÔÓÙ·È ÛÙËÓ ÂÌÂÈÚ›· ·fi ÙËÓ ÂÊ·Ú-
ÌÔÁ‹ ·ÚfiÌÔÈˆÓ ıÂڷ¢ÙÈÎÒÓ ÚÔÛÂÁÁ›ÛˆÓ. ™ÙËÓ
ÚÒÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô, Ù· ·È‰È¿ ·ÓÙÈÌÂÙˆ›ÛÙË-
Î·Ó ˆ˜ ÂÓ‹ÏÈΘ Î·È ÛÙË ıÂڷ›· ΢ÚÈ¿Ú¯ËÛ·Ó ÔÈ
˘„ËϤ˜ ‰fiÛÂȘ ·ÎÙÈÓÔ‚ÔÏ›·˜ (̤¯ÚÈ Î·È 40 Gy) ‹ Ë
·ÓÙÈÌÂÙÒÈÛË Ì ÔÏÏ¿ Û¯‹Ì·Ù· ¯ËÌÂÈÔıÂڷ›·˜
¯ˆÚ›˜ ·ÎÙÈÓÔıÂڷ›·. ªÂÙ¿ Ù· ÚÒÙ· ÌËӇ̷ٷ ÁÈ·
ÛÔ‚·Ú¤˜ ·ÒÙÂÚ˜ Û˘Ó¤ÂȘ, fiÛÔÓ ·ÊÔÚ¿ ΢ڛˆ˜
ÛÎÂÏÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ Î·È ÚÔ‚Ï‹Ì·Ù· ÂÓ‰ÔÎÚÈÓÔ-
ÏÔÁÈο, ‹ÚÍ ·ÏÏ·Á‹ ÛÙË ıÂڷ¢ÙÈ΋ ÛÙÚ·ÙËÁÈ΋
Î·È Ù· ·È‰È¿-·ÛıÂÓ›˜ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Ì Ì›ˆÛË
Ù˘ ¯ËÌÂÈÔıÂڷ›·˜ ÛÙËÓ ÂÓÙÔÈṲ̂ÓË ÓfiÛÔ Î·È,
‚¤‚·È·, Ì ηıȤڈÛË ¯·ÌËÏÒÓ ‰fiÛÂˆÓ ·ÎÙÈÓÔ‚Ô-
Ï›·˜ (20 Gy) ÌfiÓÔ ÛÙȘ ·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ Â-
ÚÈÔ¯¤˜. ∆¤ÏÔ˜, ÛÙËÓ ÙÚ›ÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô, Ë ıÂÚ·-
¢ÙÈ΋ ÔÌ¿‰·, ‰È·ÙËÚÒÓÙ·˜ ÙȘ ¯·ÌËϤ˜ ‰fiÛÂȘ
·ÎÙÈÓÔ‚ÔÏ›·˜, ÚÔ¯ÒÚËÛÂ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ
·È‰ÈÒÓ Ì ÂÓÙÔÈṲ̂ÓË ¯·ÌËÏÔ‡ Î·È ÂӉȿÌÂÛÔ˘
ÎÈÓ‰‡ÓÔ˘ ÓfiÛÔ Ì ¯ËÌÂÈÔıÂڷ¢ÙÈο Û¯‹Ì·Ù· ¯ˆ-
Ú›˜ ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜ Î·È ·Óıڷ΢ÎϛӘ,
Ô˘ Â›Ó·È Ê¿Ú̷η ˘Â‡ı˘Ó· ÁÈ· ÛËÌ·ÓÙÈ΋ ·ÒÙÂ-
ÚË ÙÔÍÈÎfiÙËÙ·. °ÂÓÈο, fiÏ· ·˘Ù¿ Ù· ¯ÚfiÓÈ· ÔÈ ÛÙfi¯ÔÈ
Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ Ì ÓfiÛÔ
∏odgkin ‹Ù·Ó Ë ‰È·Ù‹ÚËÛË ÙˆÓ ˘„ËÏÒÓ ÔÛÔÛÙÒÓ
ÂÈ‚›ˆÛ˘, Ì ٷ˘Ùfi¯ÚÔÓË Ì›ˆÛË Ù˘ Ì·ÎÚÔÚfiıÂ-
ÛÌ˘ ÙÔÍÈÎfiÙËÙ·˜ (ηډÈÔÙÔÍÈÎfiÙËÙ·, Ó¢ÌÔÓÔ¿-
ıÂÈ·, ÛÎÂÏÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÂÓ‰ÔÎÚÈÓÔÏÔÁÈΤ˜ ‰È·-
Ù·Ú·¯¤˜, ‰Â‡ÙÂÚ˜ ηÎÔ‹ıÂȘ) (24). ∞fi ÙÔ˘˜ ·ÛıÂ-
Ó›˜ Ì·˜, Ù¤ÛÛÂÚȘ ·ÚÔ˘Û›·Û·Ó ‰Â‡ÙÂÚË ‹ Î·È ÙÚ›ÙË
ηÎÔ‹ıÂÈ·. ∏ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ‰Â‡ÙÂÚ˘ η-
ÎÔ‹ıÂÈ·˜ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ÂÊ·ÚÌÔÁ‹ ‹ fi¯È ·ÎÙÈÓÔ-
ıÂڷ›·˜ Î·È ÙË ‰fiÛË ·˘Ù‹˜, ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ ‹ÙËÓ ‡·ÚÍË ˘ÔÙÚÔ‹˜ Î·È ÙËÓ ·ÚÔ˘Û›· ·ÓÔÛÔη-Ù·ÛÙÔÏ‹˜. π‰È·›ÙÂÚÔ ÚfiÏÔ Ê·›ÓÂÙ·È Ó· ·›˙ÂÈ Ë Ú‹ÛË·Ï΢ÏȈÙÈÎÒÓ ·Ú·ÁÒÁˆÓ (ª√PP), ηıÒ˜ Î·È ÔÈ ‰fi-ÛÂȘ Ù˘ ¯ËÌÂÈÔıÂڷ›·˜ (25). √ ΛӉ˘ÓÔ˜ ÁÈ· ÂÌ-Ê¿ÓÈÛË ÌË ÏÂÌÊÔ‚Ï·ÛÙÈ΋˜ Ï¢¯·ÈÌ›·˜ ‹ ÌË∏odgkin ÏÂÌÊÒÌ·ÙÔ˜ Êı¿ÓÂÈ ÙÔ 8,1% ÌÂÙ¿ ·fi ıÂ-ڷ›· ÁÈ· ÓfiÛÔ Hodgkin, ÂÓÒ Ô Î›Ó‰˘ÓÔ˜ ÁÈ· Û˘Ì·-Á‹ fiÁÎÔ Êı¿ÓÂÈ ÙÔ 13,1% ÛÙ· 20 ¤ÙË (26).
ª¤¯ÚÈ Û‹ÌÂÚ·, Ë ıÂڷ¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Ù˘ÓfiÛÔ˘ ÛÙËÚ›˙ÂÙ·È Û ÚÔÛÂÁÁ›ÛÂȘ ÚÔÛ·ÚÌÔṲ̂Ó˜ÛÙËÓ ·¿ÓÙËÛË ÛÙËÓ ·Ú¯È΋ ıÂڷ›·, ÛÙÔ Ê‡ÏÔ Î·ÈÙËÓ ËÏÈΛ·. √ Û‡Á¯ÚÔÓÔ˜ ÙÚfiÔ˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ·ÛıÂÓÒÓ Ì ÂÓÙÔÈṲ̂ÓË ·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÓfiÛÔ Â-ÚÈÏ·Ì‚¿ÓÂÈ ÏÈÁfiÙÂÚÔ ÙÔÍÈ΋ ıÂڷ›· Ì 2-4 ·ÎÏÔ˘˜¯ËÌÂÈÔıÂڷ›·˜ Î·È ¯·ÌËϤ˜ ‰fiÛÂȘ ·ÎÙÈÓÔ‚ÔÏ›·˜,ÂÓÒ ÁÈ· ÙÔ˘˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Î·È Ì ÚÔ¯ˆÚË̤ÓËÓfiÛÔ ·ÛıÂÓ›˜ ‰ÔÎÈÌ¿˙ÔÓÙ·È ÂÚÈÛÛfiÙÂÚÔ ÂÓÙ·ÙÈοۯ‹Ì·Ù· ıÂڷ›·˜ (27). ∆ÂÏÂ˘Ù·›·, ÙÔ Î˘Ú›·Ú¯ÔÂÚÒÙËÌ· ÙˆÓ ÌÂÁ¿ÏˆÓ Û˘ÓÂÚÁ·ÙÈÎÒÓ ÌÂÏÂÙÒÓ Â›Ó·È«ÔÈÔÈ ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ ÌÔÚÔ‡Ó Ó· È·ıÔ‡Ó ¯ˆÚ›˜ÙË Ú‹ÛË ·ÎÙÈÓÔıÂڷ›·˜;». √ ·ӷÚÔÛ‰ÈÔÚÈÛÌfi˜ÙˆÓ ÔÌ¿‰ˆÓ ÎÈÓ‰‡ÓÔ˘ Ì ӤԢ˜ ‚ÈÔÏÔÁÈÎÔ‡˜ Î·È ÌÔ-ÚÈ·ÎÔ‡˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ‰˘ÓËÙÈο ı·Ô‰ËÁ‹ÛÂÈ Û ÂÚ·ÈÙ¤Úˆ ÂÚÈÔÚÈÛÌfi Ù˘ ıÂڷ›·˜ ‹Û ÂÓÙ·ÙÈÎÔÔ›ËÛË ·˘Ù‹˜. √È ÚfiÔ‰ÔÈ ÛÙË ıÂڷ›·ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin ¤¯Ô˘Ó ÌÂÈÒÛÂÈ ÙËÓ ÚÔ-ÁÓˆÛÙÈ΋ ·Í›· ·Ú·‰ÔÛÈ·ÎÒÓ ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·-ÁfiÓÙˆÓ, fiˆ˜ Ë ËÏÈΛ·, ÙÔ Ê‡ÏÔ, Ë ÈÛÙÔÏÔÁ›·, ÙÔ ÛÙ¿-‰ÈÔ Î·È Ù· Û˘ÌÙÒÌ·Ù· Ù‡Ô˘ µ. ∏ ·¿ÓÙËÛË ÛÙËÓ·Ú¯È΋ ıÂڷ›· ıˆÚÂ›Ù·È Û‹ÌÂÚ· ÚÔÛ‰ÈÔÚÈÛÙÈÎfi˜·Ú¿ÁÔÓÙ·˜ ÁÈ· ÙËÓ Â·ÎfiÏÔ˘ıË ıÂڷ¢ÙÈ΋ ÚÔ-Û¤ÁÁÈÛË. √È ÚfiÔ‰ÔÈ ÛÙȘ ·ÂÈÎÔÓÈÛÙÈΤ˜ ÌÂıfi‰Ô˘˜,fiˆ˜ Ë ¯Ú‹ÛË ˘ÔÏÔÁÈÛÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ ˘„ËÏ‹˜Â˘ÎÚ›ÓÂÈ·˜ (HRCT), Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ (MRI),·ÏÏ¿, ÙÂÏÂ˘Ù·›·, Î·È Ë Ú‹ÛË ÙÔÌÔÁÚ·Ê›·˜ ÂÎÔÌ‹˜Ô˙ÈÙÚÔÓ›ˆÓ (PET) [ÂÎÙÈÌ¿ ÙËÓ ˘ÂÚÏ·ÛÙÈ΋ ‰Ú·-ÛÙËÚÈfiÙËÙ· ÙˆÓ ÏÂÌÊ·‰¤ÓˆÓ ‰È· ̤ÛÔ˘ Ù˘ ·˘ÍË̤-Ó˘ ÚfiÛÏ˄˘ ÙÔ˘ 18-fluoro-2-deoxyglucose(FDG)] ¤¯Ô˘Ó ʤÚÂÈ Â·Ó¿ÛÙ·ÛË ÛÙË ÛÙ·‰ÈÔÔ›ËÛËÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin. ∏ ÙÔÌÔÁÚ·Ê›· ÂÎÔÌ‹˜Ô˙ÈÙÚÔÓ›ˆÓ Î·È ÂȉÈο Ô Û˘Ó‰˘·ÛÌfi˜ Ù˘ Ì ·ÍÔÓÈ΋ÙÔÌÔÁÚ·Ê›· (PET-CT) ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂÁ¿ÏË Â˘·ÈÛıË-Û›· ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·ÓÙ·fiÎÚÈÛ˘ Î·È ÙËÓ ÔÚıfi-ÙÂÚË ¤ˆ˜ Î·È ÂÍ·ÙÔÌÈÎÂ˘Ì¤ÓË ıÂڷ›·, Ì ÚÔÔÙÈ-΋ ÙËÓ ·ÔÊ˘Á‹ Ù˘ ·ÎÙÈÓÔıÂڷ›·˜ Û ÛËÌ·ÓÙÈÎfiÔÛÔÛÙfi ·ÛıÂÓÒÓ (28).
µÈ‚ÏÈÔÁÚ·Ê›·
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130 ∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.
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9. Oberlin O, Leverger G, Pacquement H, Raquin MA,Chompret A, Habrand JL, et al. Low-dose radiation therapyand reduced chemotherapy in childhood Hodgkin’s disease:the experience of the French Society of Pediatric Oncology. JClin Oncol 1992;10:1602-1608.
10. Landman-Parker J, Pacquement H, Leblanc T, HabrandJL, Terrier-Lacombe MJ, et al. Localized ChildhoodHodgkin’s Disease: Response-Adapted ChemotherapyWith Etoposide, Bleomycin, Vinblastine, and PrednisoneBefore Low-Dose Radiation Therapy - Results of theFrench Society of Pediatric Oncology Study MDH90. JClin Oncol 2000;18:1500-1507.
11. Feldman AL, Pittaluga S, Jaffe ES. Classification andhistopathology of the lymphomas. In: Canellos GP, ListerTA, Young B (eds) The lymphomas. 2nd ed., SaundersElsevier, Philadelphia; 2006, pp. 2-25.
12. Nogovaã L, Rudiger T, Engert A. Biology, clinical courseand management of nodular lymphocyte-predominanthodgkin lymphoma. Hematology Am Soc Hematol EducProgram 2006;1:266-272.
13. Smith RS, Chen Q, Hudson MM, Link MP, Kun L,Weinstein H et al. Prognostic factors for children withHodgkin’s disease treated with combined-modality therapy.J Clin Oncol. 2003;21:2026-2033.
14. Schwartz CL. Prognostic Factors in Pediatric HodgkinDisease. Current Oncology Reports 2003;5:498-504.
15. Belgaumi A, Al-Kofide A, Joseph N, Jamil-Malik R,Khafaga Y, Sabbah R. HL in very young children: Clinicalcharacteristics and outcome of treatment. LeukLymphoma 2008;49:910-916.
16. Viani GA, Castilho MS, Novaes PE, Antonelli CG, FerrignoR, Pellizzon CA, et al. Chemotherapy followed by low doseradiotherapy in childhood Hodgkin’s disease: Retro-spective analysis of results and prognostic factors.Radiation Oncology 2006;1:38-45.
17. Dinand V, Dawar R, Arya LS, Unni R, Mohanty B, Singh R,et al. Hodgkin’s lymphoma in Indian children: Prevalenceand significance of Epstein-Barr virus detection inHodgkin’s and Reed-Sternberg cells. European Journal ofCancer 2007;43:161-168.
18. Portis T, Dyck P, Longnecker R. Epstein-Barr Virus (EBV)LMP2A induces alterations in gene transcription similar tothose observed in Reed-Sternberg cells of Hodgkinlymphoma. Blood 2003;102:4166-4178.
19. Landgren O, Engels EA, Pfeiffer RM, Gridley G, MellemkjaerL, Olsen JH, et al. Autoimmunity and susceptibility toHodgkin lymphoma: a population-based case-control studyin Scandinavia. J Natl Cancer Inst 2006;98:1321-1330.
20. Mullen E, Zhong Y. Hodgkin Lymphoma: An Update.Journal for Nurse Practitioners 2007;3:393-403.
21. Schwartz CL. The management of Hodgkin disease in theyoung child. Curr Opin Pediatr 2003;15:10-16.
22. Diehl V, Stein H, Hummel M, Zollinger R, Connors JM.Hodgkin's lymphoma: biology and treatment strategies forprimary, refractory, and relapsed disease. Hematology. AmSoc Hematol Educ Program 2003;1:225-247.
23. Cashen ∞, Bartlett ¡. Therapy of Relapsed HodgkinLymphoma. Blood Reviews. 2007;21:233-243.
24. Bartlett ¡. Modern treatment of Hodgkin lymphoma.Current Opinion in Hematology 2008;15:408-414.
25. Ng AK, Mauch PM. The impact of treatment on the risk ofsecond malignancy after Hodgkin's disease. Ann Oncol2006;17:1727-1729.
26. van Leeuwen FE, Klokman WJ, Hagenbeek A, Noyon R,van den Belt-Dusebout AW, van Kerkhoff EH, et al.Second cancer risk following Hodgkin's disease: a 20-yearfollow-up study. J Clin Oncol 1994;12:312-325.
27. Olson MR, Donaldson SS. Treatment of pediatric Hodgkinlymphoma. Curr Treat Options Oncol 2008;9:81-94.
28. Malik E. Juweid. Utility of Positron Emission Tomography(PET) Scanning in Managing Patients with HodgkinLymphoma. Hematology, Am Soc Hematol Educ Program2006;1:259-265.
131§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿
¶·È‰È·ÙÚÈ΋ 2009;72:131-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·131
√È ÂÈÙÒÛÂȘ Ù˘ ÂÚÙÚÔÊ›·˜ ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È
·Ì˘Á‰·ÏÒÓ ÛÙËÓ Î·Ú‰È·Î‹ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ
∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘
¶ÂÚ›ÏË„Ë: ∏ ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ ·ÔÙÂÏ› ÙÔ Û˘¯ÓfiÙÂÚÔ ·›ÙÈÔ ·fiÊڷ͢ ÙˆÓ·ÓÒÙÂÚˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ, Ô˘ ÌÔÚ› Ó· ¤¯ÂÈ ÛÔ‚·Ú¤˜ ÂÈÙÒÛÂȘ ÛÙËÓ Î·Ú‰ÈÔÓ¢ÌÔÓÈ΋ ÏÂÈÙÔ˘Ú-Á›· ÙˆÓ ·È‰ÈÒÓ. ∏ ·fiÊÚ·ÍË ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ÏfiÁˆ ÂÏ¿ÙÙˆÛ˘ ÙÔ˘ Ó¢ÚÔ-Ì˘˚ÎÔ‡ ÙfiÓÔ˘, Î·È Ô‰ËÁ› Û ˘fiÓÔÈ· ‹ ¿ÓÔȘ ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘. √È ÙÂÏÂ˘Ù·›Â˜ Û˘Óԉ‡ÔÓÙ·È ·fiÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜, ˘ÂÚηӛ·˜ Î·È ·Ó·Ó¢ÛÙÈ΋˜ ÔͤˆÛ˘ (Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜‡ÓÔ˘). ∆· ˘ÔÙÚÔÈ¿˙ÔÓÙ· ÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜ Î·È ˘ÂÚηӛ·˜ ÚÔηÏÔ‡Ó Ó¢ÌÔÓÈ΋ ·ÁÁÂÈ-ÔÛ‡Û·ÛË, Ë ÔÔ›· Ô‰ËÁ› Û ÌfiÓÈ̘ ·ÏÏÔÈÒÛÂȘ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ. √È ÂÈÙÒÛÂȘ ÙÔ˘ Û˘Ó-‰ÚfiÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÂÂÈÛfi‰È· ˘ÂÚÙ·-ÛÈÎÒÓ ÎÚ›ÛÂˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ ηÈ, Û ·Ú·ÌÂÏË̤Ó˜ÂÚÈÙÒÛÂȘ, ÙËÓ ÂÌÊ¿ÓÈÛË Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ Î·È ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜. ∞Ó Î·È Ë ¯ÚfiÓÈ·Ó¢ÌÔÓÈ΋ ηډȿ Â›Ó·È Û¿ÓÈ· Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, ÌÔÚ› Ó· ˘¿Ú¯ÂÈ ·Û˘Ìو̷ÙÈ΋ Ó¢ÌÔÓÈ΋˘¤ÚÙ·ÛË Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ª¤¯ÚÈ Û‹ÌÂÚ·, ˆ˜ ıÂڷ›· ÚÒÙ˘ ÂÎÏÔÁ‹˜ Û ·È‰È¿ Ì ۇÓ-‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ ·Ú·Ì¤ÓÂÈ Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙˆÓ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ.√È ·ÛıÂÓ›˜ Ì ÛÔ‚·Ú‹ ·fiÊÚ·ÍË Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË ‹/Î·È Î·Ú‰È·Î‹ ‰˘ÛÏÂÈ-ÙÔ˘ÚÁ›· ¤¯Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ¿ÌÂÛ˜ ÌÂÙÂÁ¯ÂÈÚËÙÈΤ˜ ÂÈÏÔΤ˜, fiˆ˜ Âȉ›ӈÛË ÙÔ˘ Û˘Ó‰Úfi-ÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‹ ÂΉ‹ÏˆÛË Ó¢ÌÔÓÈÎÔ‡ Ôȉ‹Ì·ÙÔ˜. ŸÛÔÓ ·ÊÔÚ¿ ÛÙȘ ·ÒÙÂÚ˜ ÌÂÙÂÁ¯ÂÈ-ÚËÙÈΤ˜ ÂÈÏÔΤ˜, ·˘Ù¤˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ·Ú·ÌÔÓ‹ ΢„ÂÏȉÈÎÔ‡ ˘Ô·ÂÚÈÛÌÔ‡ (Ô˘ ÌÔÚ› Ó· ¯ÚÂÈ·-Ûı› ̤¯ÚÈ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ‹ ÙÚ·¯ÂÈÔÛÙÔÌ›·), ›ÌÔÓË ˘ÔÍ·ÈÌ›·, ‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘ ‹ Î·È Û˘Ó‰˘·-ÛÌfi ·˘ÙÒÓ. °È· ÙÔ˘˜ ·ÓˆÙ¤Úˆ ÏfiÁÔ˘˜, Û ·ÛıÂÓ›˜ Ì ÛÔ‚·Ú‹ ˘ÂÚÙÚÔÊ›· Û˘ÓÈÛÙ¿Ù·È ˘ÂÚ˯ÔηډÈÔ-ÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÚÈÓ ·fi ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË. ÃÚÂÈ¿˙ÂÙ·È Â›Û˘ ÎÏÈÓÈ΋ ·ÓÂÎÙ›ÌËÛË ·˘ÙÒÓÙˆÓ ·ÛıÂÓÒÓ ÌÂÙ¿ ·fi 6-8 ‚‰ÔÌ¿‰Â˜ ·fi ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔÌ‹-·Ì˘Á‰·ÏÂÎÙÔÌ‹, ÁÈ· Ó· ‰È·ÈÛÙˆı› ·Ó··ÈÙÂ›Ù·È ÂÈÚfiÛıÂÙË ıÂڷ›·.
§¤ÍÂȘ ÎÏÂȉȿ: ÀÂÚÙÚÔÊ›· ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ, Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘, Ó¢ÌÔÓÈ΋
˘¤ÚÙ·ÛË, ¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ηډȿ.
Cardiovascular consequences of adenotonsillar hypertrophy in
children
K. Papadopoulou-Legbelou, G. Varlamis
Abstract: Adenotonsillar hypertrophy is the most common cause of upper airway obstruction, which maylead to serious cardiopulmonary complications in children. Upper airway obstruction can be exacerbatedby the decrease in neuromuscular tone that occurs during sleep, leading to hypopnoea or obstructivesleep apnoea. Obstructive sleep apnoea produces episodic hypoxia, hypercapnia and respiratory acidosis(obstructive sleep apnoea syndrome). Episodes of recurrent hypoxia and hypercapnia are potentmediators of pulmonary vasoconstriction that can lead to chronic changes in the pulmonary vasculature.Cardiovascular consequences of adenotonsillar hypertrophy include repetitive nocturnal surges in bloodpressure, breathing rhythm disturbances and, particularly in severe cases, pulmonary hypertension andcor pulmonale. Although overt right heart failure now occurs less often, asymptomatic degrees ofpulmonary hypertension can occur. To date, adenotonsillectomy is the first line of treatment for childrenwith obstructive sleep apnoea syndrome. Children with significant airway obstruction associated withpulmonary hypertension and/or cardiac dysfunction are at greater risk for perioperative complications,such as worsening of their obstructive sleep apnoea, and pulmonary oedema. Potential postoperativecomplications are persistent airway obstruction (including the need for reintubation or tracheostomy),desaturation, sleep disturbances, or a combination of these. Thus, patients with severe obstruction shouldbe screened by echocardiography preoperatively. Clinical reevaluation is also recommended 6 to 8 weeksafter adenotonsillectomy, to determine whether additional treatment is required.
Key words: Adenotonsillar hypertrophy, obstructive sleep apnoea syndrome, pulmonary hypertension, cor
pulmonale.
132 ¶ƒ∞∫∆π∫√ £∂ª∞ PRACTICAL ISSUE
¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÚÈÛÙÔÙ¤ÏÂÈÔ˘ ¶·ÓÂÈÛÙ‹ÌÈÔ˘£ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌ›ԫ¶··ÁˆÚÁ›Ô˘»
AÏÏËÏÔÁÚ·Ê›·:
∫˘Úȷ΋ ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘[email protected]¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£,¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘»¶ÂÚÈÊÂÚÂȷ΋ Ô‰fi˜£ÂÛ/ӛ΢, ¡. ∂˘Î·Ú›·,56403, £ÂÛÛ·ÏÔÓ›ÎË
4th Department ofPaediatrics, “Aristotelio”University of Thessaloniki,“PapageÔrgiou” Hospital,Thessaloniki, Greece
Correspondence:
Kyriaki [email protected] Department ofPaediatrics, “Aristotelio”University of Thessaloniki,“PapageÔrgiou” HospitalRing Road, N. Efkarpia,56403, Thessaloniki, Greece
Paediatriki 2009;72:132-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·132
™˘ÓÙÔÌÔÁڷʛ˜:
∞¶ ·ÚÙËÚȷ΋ ›ÂÛË¡™ Ó¢ÚÈÎfi Û‡ÛÙËÌ·™∞∞À Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘
∂ÈÛ·ÁˆÁ‹
∏ ˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ·ÔÙÂÏ› ÙÔ Û˘¯ÓfiÙÂÚÔ ·›ÙÈÔ ¯ÚfiÓÈ·˜ ·fiÊÚ·ÍË˜ÙˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ, Ô˘ ÌÔÚ› Ó· ¤¯ÂÈ ÛÔ‚·Ú¤˜ÂÈÙÒÛÂȘ ÛÙËÓ Î·Ú‰ÈÔÓ¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· وӷȉÈÒÓ. ∏ ·fiÊÚ·ÍË ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿ ÙË ‰È¿Ú-ÎÂÈ· ÙÔ˘ ‡ÓÔ˘, fiÔ˘ ¯·Ï·ÚÒÓÔ˘Ó ÔÈ Ê·Ú˘ÁÁÈÎԛ̇˜, ηıÒ˜ ›Û˘ ·fi Û˘Ó˘¿Ú¯Ô˘Û˜ ηٷÛÙ¿-ÛÂȘ, fiˆ˜ ˘ÔÙÚÔÈ¿˙Ô˘Û˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·ÓÒÙÂ-ÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ‹ ·¯˘Û·ÚΛ· (1). ™Â ÛÔ‚·Ú‹˘ÂÚÙÚÔÊ›·, ·Ú·ÙËÚÔ‡ÓÙ·È Â›Û˘ ·ÔÊÚ·ÎÙÈÎÔ‡Ù‡Ô˘ ¿ÓÔȘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ÔÈ ÔÔ›-˜ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ·‰˘Ó·Ì›· ÂÈÛfi‰Ô˘ ·¤Ú· ·-Ú¿ ÙËÓ ‡·ÚÍË ·Ó·Ó¢ÛÙÈ΋˜ ÚÔÛ¿ıÂÈ·˜ ηȉȷÎÔ‹ ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ·ÂÚÈÛÌÔ‡ Î·È Ù˘ ·Ú¯ÈÙÂ-ÎÙÔÓÈ΋˜ ÙÔ˘ ‡ÓÔ˘ (Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜¿ÓÔÈ·˜ ‡ÓÔ˘) (2).
∆Ô Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘(™∞∞À) ÛÙ· ·È‰È¿ Â›Ó·È ÈÔ Û˘¯Ófi Û ËÏÈΛ· 2-8¯ÚfiÓˆÓ, fiÔ˘ ˘¿Ú¯ÂÈ Î·È Ë ÌÂÁ·Ï‡ÙÂÚË ˘ÂÚÙÚÔ-Ê›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ÙˆÓ ·Ì˘Á‰·ÏÒÓ (2,3). ¶Ú¤-ÂÈ Ó· ‰È·ÊÔÚԉȷÁÓˆÛÙ› ·fi ÙÔ ÚˆÙÔ·ı¤˜ ÚÔ-¯·ÏËÙfi, ÛÙÔ ÔÔ›Ô ‰ÂÓ Û˘Ó˘¿Ú¯Ô˘Ó ·ÔÊÚ·ÎÙÈÎÔ‡Ù‡Ô˘ ¿ÓÔȘ, Û˘¯Ó¤˜ ·Ê˘Ó›ÛÂȘ ‹ ‰È·Ù·Ú·¯¤˜Ù˘ ·ÓÙ·ÏÏ·Á‹˜ ÙˆÓ ·ÂÚ›ˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ (2). ªÂϤ-Ù˜ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ Î·ÈÙ˘ ‡·Ú͢ ™∞∞À (4,5). ∞˘Ùfi Û˘Ì‚·›ÓÂÈ ‰ÈfiÙÈ ÙÔ™∞∞À Â›Ó·È Û˘Ó‰˘·ÛÌfi˜ ‰ÔÌÈÎÒÓ Î·È Ó¢ÚÔÌ˘˚ÎÒÓ·Ú·ÁfiÓÙˆÓ (3).
∏ Û˘¯ÓfiÙËÙ· ÙÔ˘ ™∞∞À ˘ÔÏÔÁ›˙ÂÙ·È Û 2%(2,6-8), ÂÓÒ Ë Û˘¯ÓfiÙËÙ· ÙÔ˘ ÚÔ¯·ÏËÙÔ‡ Â›Ó·È ÔχÌÂÁ·Ï‡ÙÂÚË Î·È Î˘Ì·›ÓÂÙ·È Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ ÌÂ-ٷ͇ 8-27% (6,7,9-11). ŸÛÔÓ ·ÊÔÚ¿ ÛÙÔ Ê‡ÏÔ, Ë Û˘-¯ÓfiÙËÙ· Â›Ó·È ›ÛË ÌÂٷ͇ ÙˆÓ ‰‡Ô Ê‡ÏˆÓ (2,3).
H ·¯˘Û·ÚΛ·, Ô˘ ·˘Í¿ÓÂÙ·È ÛËÌ·ÓÙÈο Ù· ÙÂ-ÏÂ˘Ù·›· ¯ÚfiÓÈ· ÛÙËÓ ·È‰È΋ ËÏÈΛ·, ·ÔÙÂÏ› ϤÔÓ¤Ó· ÛËÌ·ÓÙÈÎfi ÚԉȷıÂÛÈÎfi ·Ú¿ÁÔÓÙ· ÙÔ˘ ™∞∞À.ŒÙÛÈ, ÙÔ ™∞∞À ‰È·ÎÚ›ÓÂÙ·È ÛÙȘ ÂÍ‹˜ ηÙËÁÔڛ˜: Ù‡-Ô˘ π, ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È ÛÔ‚·Ú‹ ˘ÂÚÙÚÔÊ›·ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ, Ù‡Ô˘ ππ, ÛÙËÓÔÔ›· ˘¿Ú¯ÂÈ ËÈfiÙÂÚË ·fiÊÚ·ÍË Ù˘ ·ÓÒÙÂÚ˘·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·¯˘Û·ÚΛ·,Î·È Ù‡Ô˘ πππ, ÛÙËÓ ÔÔ›· ÙÔ ™∞∞À ÔÊ›ÏÂÙ·È Û ÎÚ·-ÓÈÔÚÔÛˆÈΤ˜ ·ÓˆÌ·Ï›Â˜ ‹ Ó¢ÚÔÌ˘˚Τ˜ ‰È·Ù·Ú·-¯¤˜ (Û‡Ó‰ÚÔÌ· Crouzon, Apert, Teacher Collins,Pierre Robin, Down, ·¯ÔÓ‰ÚÔÏ·Û›·, ÌËÓÈÁÁÔÌ˘ÂÏÔ-΋ÏË, ÂÁÎÂÊ·ÏÈ΋ ·Ú¿Ï˘ÛË) (12).
√È ·ÛıÂÓ›˜ Ì ™∞∞À Ô˘ ‰ÂÓ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È
¤ÁηÈÚ· ¤¯Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÂÌÊ¿ÓÈÛ˘ ÛÔ‚·-
ÚÒÓ ¯ÚfiÓÈˆÓ Î·Ú‰È·ÁÁÂÈ·ÎÒÓ Î·È ÌÂÙ·‚ÔÏÈÎÒÓ ‰È·-
Ù·Ú·¯ÒÓ. ∞˘Ù¤˜ Û˘Ó›ÛÙ·ÓÙ·È Û ˘„ËÏfiÙÂÚ· ›‰·
·ÚÙËÚȷ΋˜ ›ÂÛ˘, ·‡ÍËÛË Ù˘ C ·ÓÙȉÚÒÛ·˜ Úˆ-
Ù½Ó˘ (Ë ÔÔ›· Û˘Û¯ÂÙ›˙ÂÙ·È Ì ηډȷÁÁÂȷΤ˜ Ófi-
ÛÔ˘˜), ·ÓÙ›ÛÙ·ÛË ÛÙËÓ ÈÓÛÔ˘Ï›ÓË Î·È ˘ÂÚÙÚÔÊ›·
Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ (13).
ŸÛÔÓ ·ÊÔÚ¿ ÛÙÔÓ Ù‡Ô π (ÌÂÌÔӈ̤ÓË ˘ÂÚÙÚÔ-
Ê›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ), ¤¯ÂÈ ‰È·ÈÛÙˆ-
ı› fiÙÈ Ë ¯ÚfiÓÈ· ‰È·ÏÂ›Ô˘Û· ˘ÔÍ·ÈÌ›· Î·È ÙÔ ÔÍÂÈ-
‰ˆÙÈÎfi stress Ô‰ËÁÔ‡Ó ÛÙËÓ ·Ú·ÁˆÁ‹ ‰È·ÊfiÚˆÓ
ÊÏÂÁÌÔÓˆ‰ÒÓ Î˘ÙÔÎÈÓÒÓ Î·È Û ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙÔ˘
ÂÓ‰ÔıËÏ›Ô˘ ÙˆÓ ·ÁÁ›ˆÓ (13). ∏ Û˘Ó‡·ÚÍË ÙÔ˘
™∞∞À Î·È Ù˘ ·¯˘Û·ÚΛ·˜ ÛÙÔÓ Ù‡Ô ππ ÂȉÂÈÓÒ-
ÓÂÈ ÙÔ ÔÍÂȉˆÙÈÎfi stress Î·È ÙË Û˘ÛÙËÌ·ÙÈ΋ ÊÏÂÁÌÔ-
ÓÒ‰Ë ·ÓÙ›‰Ú·ÛË Î·È ÂÈÙ·¯‡ÓÂÈ ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘
·ÓÙ›ÛÙ·Û˘ ÛÙËÓ ÈÓÛÔ˘Ï›ÓË Î·È, ηٿ Û˘Ó¤ÂÈ·, ÙÔ˘
ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ (14).
∞ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÙÒÛÂȘ ÙÔ˘ ™∞∞À
∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, Ù· ·ÓÂÈÏËÌ̤ӷ
ÂÂÈÛfi‰È· ÌÂÚÈ΋˜ ‹ Ï‹ÚÔ˘˜ ·fiÊڷ͢ Ù˘ ·ÓÒ-
ÙÂÚ˘ ·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡ ÚÔηÏÔ‡Ó ˘Ô·ÂÚÈÛÌfi
(˘fiÓÔÈ·) ‹ ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘ ¿ÓÔȘ. √È ÙÂ-
ÏÂ˘Ù·›Â˜ ÚÔηÏÔ‡Ó ÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜, ÌÂ
Û˘ÓÔ‰fi ˘ÂÚηӛ· Î·È ·Ó·Ó¢ÛÙÈ΋ ÔͤˆÛË (15).
1) √Í›˜ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÙÒÛÂȘ: √È ·ÔÊÚ·-
ÎÙÈΤ˜ ¿ÓÔȘ ÚÔηÏÔ‡Ó Â·Ó·Ï·Ì‚·ÓfiÌÂÓ·
ÂÂÈÛfi‰È· ÙÒÛ˘ Ù˘ ÂÓ‰ÔıˆÚ·ÎÈ΋˜ ›ÂÛ˘, Ô˘
¤¯Ô˘Ó ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·‡ÍËÛË ÙÔ˘ ÌÂÙ·ÊÔÚÙ›Ô˘
Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜, ÂÏ¿ÙÙˆÛË ÙÔ˘ fiÁÎÔ˘ ·ÏÌÔ‡
Î·È Ù˘ ηډȷ΋˜ ·ÚÔ¯‹˜. ∆·˘Ùfi¯ÚÔÓ· ·˘Í¿ÓÂÙ·È
Ë ÊÏ‚È΋ ÂÈÛÙÚÔÊ‹ ·›Ì·ÙÔ˜, ÏfiÁˆ ÂÏ¿ÙÙˆÛ˘ Ù˘
ÎÂÓÙÚÈ΋˜ ÊÏ‚È΋˜ ›ÂÛ˘, Ì ·ÎfiÏÔ˘ıÔ ÙËÓ ·‡-
ÍËÛË ÙÔ˘ ÙÂÏԉȷÛÙÔÏÈÎÔ‡ fiÁÎÔ˘ Ù˘ ‰ÂÍÈ¿˜ ÎÔÈ-
Ï›·˜. ªÂ ÙËÓ ·Ê‡ÓÈÛË ‰È·ÎfiÙÂÙ·È Ë ¿ÓÔÈ· ηÈ
·˘Í¿ÓÂÙ·È Ë Û˘ÛÙËÌ·ÙÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË Î·È Ë
ηډȷ΋ ·ÚÔ¯‹ (15).
2) ÃÚfiÓȘ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÙÒÛÂȘ: ∏ ¯ÚfiÓÈ·
˘ÔÍ·ÈÌ›· ÚÔηÏ› ·ÁÁÂÈÔÛ‡Û·ÛË ÙˆÓ ÎÏ¿‰ˆÓ
Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜. ∆· ·ÓÂÈÏËÌ̤ӷ ÂÂÈ-
Ûfi‰È· ·ÁÁÂÈÔÛ‡Û·Û˘ ηٷÛÙÚ¤ÊÔ˘Ó ÙÔ Ó¢ÌÔÓÈ-
Îfi ·ÁÁÂÈ·Îfi ÂÓ‰Ôı‹ÏÈÔ, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ó¿Ù˘-
ÍË Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ Î·È ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ-
΋˜ ηډȿ˜ (15).
∫ÏÈÓÈ΋ Û˘Ìو̷ÙÔÏÔÁ›· ÙÔ˘ ™∞∞À
™Â ·Ú¯ÈÎfi ÛÙ¿‰ÈÔ ·Ú·ÙËÚÂ›Ù·È ·‰ÂÓÔÂȉ¤˜ ÚÔ-
ۈ›Ô, ÛÙÔÌ·ÙÈ΋ ·Ó·ÓÔ‹ Î·È ÚÔ¯·ÏËÙfi ÛÙÔÓ
‡ÓÔ. ™Â ÈÔ ÚÔ¯ˆÚË̤ÓÔ ÛÙ¿‰ÈÔ ÂÌÊ·Ó›˙ÔÓÙ·È
·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘ ¿ÓÔȘ ÛÙÔÓ ‡ÓÔ, ‰È·Ù·Ú·¯¤˜
‡ÓÔ˘ (Û˘¯Ó¤˜ ·Ê˘Ó›ÛÂȘ, Ó˘¯ÙÂÚÈÓÔ› ȉÚÒÙ˜), Ó˘-
¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË, ÎÂÊ·Ï·ÏÁ›· ηٿ ÙËÓ ·Ê‡ÓÈÛË,
133∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ
¶·È‰È·ÙÚÈ΋ 2009;72:133-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·133
ËÌÂÚ‹ÛÈ· ˘ÓËÏ›· Î·È Ì·ıËÛȷΤ˜ ‰È·Ù·Ú·¯¤˜. ™Â
ÙÂÏÈÎfi ÛÙ¿‰ÈÔ ·Ú·ÙËÚÂ›Ù·È ˘ÔıÚ„›·, ·Ó·Ó¢-
ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, ΢¿ÓˆÛË Î·È Û˘ÌÙÒÌ·Ù· ‰ÂÍÈ¿˜
‹ ·ÚÈÛÙÂÚ‹˜ ηډȷ΋˜ ·Ó¿ÚÎÂÈ·˜ (2,3).
™ËÌ·ÓÙÈ΋ ËÌÂÚ‹ÛÈ· ˘ÓËÏ›· ‰ÂÓ ·Ú·ÙËÚ›ٷÈ
Û˘¯Ó¿ Û ·È‰È¿ Ô˘ ¤¯Ô˘Ó ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘
¿ÓÔȘ (16). ¶·ÚfiÏ· ·˘Ù¿, ·ÎfiÌË Î·È ·È‰È¿ ÌÂ
·Ïfi ÚÔ¯·ÏËÙfi ÌÔÚ› Ó· ¤¯Ô˘Ó ‰È·Ù·Ú·¯¤˜ Û˘ÌÂ-
ÚÈÊÔÚ¿˜ Î·È Ì·ıËÛȷΤ˜ ‰È·Ù·Ú·¯¤˜ (17,18), ÔÈ
Ôԛ˜ Â›Ó·È ÌÂÚÈÎÒ˜ ·Ó·ÛÙÚ¤„È̘, Ì ÙËÓ ¿ÚÛË
Ù˘ ·fiÊڷ͢ (19,20).
ŸÛÔÓ ·ÊÔÚ¿ ÛÙË ÌÂȈ̤ÓË ÛˆÌ·ÙÈ΋ ·Ó¿Ù˘ÍË,
Û‡Ìʈӷ Ì ÙÔ˘˜ Marcus et al. (21) ·˘Ù‹ ÔÊ›ÏÂÙ·È
ÛÙËÓ ÂÚÁÒ‰Ë ·Ó·ÓÔ‹, Ë ÔÔ›· Ô‰ËÁ› Û ·˘ÍË̤Ó˜
ÌÂÙ·‚ÔÏÈΤ˜ ·Ó¿ÁΘ, ÂÓÒ Û ÌÈ· ¿ÏÏË ÌÂϤÙË ıˆ-
Ú‹ıËΠˆ˜ Èı·Ófi˜ ÔÚÌÔÓÈÎfi˜ Ì˯·ÓÈÛÌfi˜ (ÂÏ¿ÙÙˆ-
ÛË ÙÔ˘ ·Ú¿ÁÔÓÙ· IGF-I) (22). ∞Ó Î·È Ë ÌÂȈ̤ÓË
ۈ̷ÙÈ΋ ·Ó¿Ù˘ÍË ‰ÂÓ Â›Ó·È Û˘¯Ó‹ Ù· ÙÂÏÂ˘Ù·›·
¯ÚfiÓÈ·, Ù· ·È‰È¿ Ô˘ οÓÔ˘Ó ·‰ÂÓÔÂȉÂÎÙÔÌ‹ ηÈ
·Ì˘Á‰·ÏÂÎÙÔÌ‹ ÏfiÁˆ ™∞∞À ÂÌÊ·Ó›˙Ô˘Ó ·‡ÍËÛË
ÙÔ˘ ۈ̷ÙÈÎÔ‡ ÙÔ˘˜ ‚¿ÚÔ˘˜ ÌÂÙ¿ ·fi ·˘Ù‹Ó
(21,22).
∏ ¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ηډȿ (cor pulmonale) ÌÂ
Û˘ÓÔ‰fi ηډȷ΋ ·Ó¿ÚÎÂÈ· Â›Ó·È Û¿ÓÈ· Ù· ÙÂ-
ÏÂ˘Ù·›· ¯ÚfiÓÈ·. ¶·ÚfiÏ· ·˘Ù¿, Û˘¯Ó¿ ˘¿Ú¯ÂÈ ·Û˘-
Ìو̷ÙÈ΋ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË (3).
¢È¿ÁÓˆÛË ÙÔ˘ ™∞∞À
∂ÎÙfi˜ ·fi ÙÔ ÈÛÙÔÚÈÎfi Î·È ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË, ÔÈ
‰È·ÁÓˆÛÙÈΤ˜ ̤ıÔ‰ÔÈ Ô˘ ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó
ÛÙË ‰È¿ÁÓˆÛË Â›Ó·È Ë Ô͢ÌÂÙÚ›· Î·È Ë ÔÏ˘Î·Ù·ÁÚ·-
ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘. ™Ùfi¯Ô˜ Ù˘ ‰È¿ÁÓˆÛ˘ Ù˘ ‡·Ú-
͢ ™∞∞À Â›Ó·È Ë ·Ó›¯Ó¢ÛË ·ÛıÂÓÒÓ ˘„ËÏÔ‡ ÎÈÓ‰‡-
ÓÔ˘ ÁÈ· ÙËÓ ÂΉ‹ÏˆÛË ÛÔ‚·ÚÒÓ ÂÈÏÔÎÒÓ.
øÛÙfiÛÔ ÙÔ ÈÛÙÔÚÈÎfi ‰ÂÓ ÌÔÚ› Ó· ‰ÒÛÂÈ ·ÍÈfiÈ-
ÛÙ˜ ÏËÚÔÊÔڛ˜ ÁÈ· ÙËÓ ‡·ÚÍË ‹ fi¯È ·ÔÊÚ·ÎÙÈ-
ÎÔ‡ Ù‡Ô˘ ·ÓÔÈÒÓ, ÁÈ· ÙÔ˘˜ ÂÍ‹˜ ÏfiÁÔ˘˜: ÚÒÙÔÓ, Ë
¤ÓÙ·ÛË ÙÔ˘ ÚÔ¯·ÏËÙÔ‡ ‰ÂÓ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ‡·ÚÍË
·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘ ·ÓÔÈÒÓ. ∂›Û˘, ÔÈ ÔÏ˘Î·Ù·-
ÁÚ·ÊÈΤ˜ ÌÂϤÙ˜ ‡ÓÔ˘ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ ÔÈ ¿ÓÔȘ
·Ú·ÙËÚÔ‡ÓÙ·È Î·Ù¿ ÙȘ ÚÒÙ˜ ÚˆÈÓ¤˜ ÒÚ˜, ÛÙË
Ê¿ÛË REM ÙÔ˘ ‡ÓÔ˘, fiÔ˘ ÔÈ ÁÔÓ›˜ ÎÔÈÌÔ‡ÓÙ·È.
∞ÎfiÌË, ÌÂÚÈο ·È‰È¿ ¤¯Ô˘Ó ÌÈ· ÌfiÓÈÌË ·fiÊÚ·ÍË
Ù˘ ·ÓÒÙÂÚ˘ ·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡, Ô˘ Û˘Óԉ‡ÂÙ·È
·fi ‰È·Ù·Ú·¯‹ Ù˘ ·ÓÙ·ÏÏ·Á‹˜ ·ÂÚ›ˆÓ Î·È ‰ÂÓ ÂÌÊ·-
Ó›˙Ô˘Ó ÙȘ Ù˘ÈΤ˜ ΢ÎÏÈÎÔ‡ Ù‡Ô˘ ¿ÓÔȘ, ÒÛÙ ӷ
Á›ÓÔ˘Ó ·ÓÙÈÏËÙ¤˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ (2).
∏ ÔÏÔÓ‡ÎÙÈ· ηٷÁÚ·Ê‹ Ù˘ Ô͢ÌÂÙÚ›·˜ ÌÔÚ›
›Û˘ Ó· ‰Â›ÍÂÈ ÂÂÈÛfi‰È· ΢ÎÏÈ΋˜ ÙÒÛ˘ ÙÔ˘
ÎÔÚÂÛÌÔ‡, ·ÏÏ¿ ÌÔÚ› Ó· ¤¯ÂÈ „¢‰Ò˜ ıÂÙÈο ·Ô-
ÙÂϤÛÌ·Ù· Û ·È‰È¿ Ì ·¯˘Û·ÚΛ· ‹ ¿ÛıÌ·. ∏ Ìfi-
ÓË Û›ÁÔ˘ÚË ‰È·ÁÓˆÛÙÈ΋ ̤ıÔ‰Ô˜, ÏÔÈfiÓ, Â›Ó·È Ë
ÔÏ˘Î·Ù·ÁÚ·ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘, Ë ÔÔ›· ÌÔÚ› Ó·
‰È·ÎÚ›ÓÂÈ ÙÔ ·Ïfi ÚÔ¯·ÏËÙfi ·fi ÙÔ Û‡Ó‰ÚÔÌÔ ·Ô-
ÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ (2). ø˜ ÚÔ˜ ÙÔ fiÙ ڤ-
ÂÈ Ó· ıÂڷ‡ÂÙ·È ¤Ó· ·È‰› Ì ۇӉÚÔÌÔ Ù˘ ·Ô-
ÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜, ·˘Ùfi Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÔˆÛ-
‰‹ÔÙ fiÙ·Ó ˘¿Ú¯Ô˘Ó ¿Óˆ ·fi 10 ·ÔÊÚ·ÎÙÈο
·ÓÔ˚ο ÂÂÈÛfi‰È· ·Ó¿ ÒÚ· (23).
∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÙÔ˘ ™∞∞À ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi
Û‡ÛÙËÌ·
√È ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙËÓ ÂÎ-
‰‹ÏˆÛË ˘ÂÚÙ·ÛÈÎÒÓ ÎÚ›ÛÂˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ·
·ÓÔÈÒÓ, ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡, Ó¢-
ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘, ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜
Î·È Û˘ÌÊÔÚËÙÈ΋˜ ηډȷ΋˜ ·Ó¿ÚÎÂÈ·˜.
™∞∞À Î·È ¤ÚÙ·ÛË
∏ ·fiÊÚ·ÍË Ô‰ËÁ› Û ·ӷϷ̂·ÓfiÌÂÓ· ÂÂÈ-
Ûfi‰È· ˘ÂÚÙ·ÛÈÎÒÓ ÎÚ›ÛÂˆÓ Î·Ù¿ ÙÔÓ ‡ÓÔ, Ô˘
Û˘Ó‰˘¿˙ÔÓÙ·È Ì ٷ ·ÓÔ˚ο ÂÂÈÛfi‰È·. ∂›Û˘
˘¿Ú¯ÂÈ ·˘ÍË̤ÓÔ˜ ΛӉ˘ÓÔ˜ Û˘ÛÙËÌ·ÙÈ΋˜ ˘¤Ú-
Ù·Û˘ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (24). √È ·ıÔÁÂÓÂÙÈÎÔ› ÌË-
¯·ÓÈÛÌÔ› Ô˘ Èı·ÓÔÏÔÁÔ‡ÓÙ·È Â›Ó·È: ·) ·˘ÍË̤ÓË
‰Ú·ÛÙËÚÈfiÙËÙ· ÙÔ˘ Û˘Ì·ıËÙÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹-
Ì·ÙÔ˜ (¡™), Ì ·ÔÙ¤ÏÂÛÌ· ·ÂÏ¢ı¤ÚˆÛË Î·Ù¯Ô-
Ï·ÌÈÓÒÓ (25), ‚) ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ·ÁÁÂÈÔÛ˘Û·-
ÛÙÈÎÒÓ Ô˘ÛÈÒÓ ·fi ÙÔ ÂÓ‰Ôı‹ÏÈÔ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ
·ÁÁ›ˆÓ, fiˆ˜ Ù˘ ÂÓ‰ÔıËÏ›Ó˘ 1 (26), Î·È Á) ‰È¤-
ÁÂÚÛË ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ÚÂÓ›Ó˘-·ÁÁÂÈÔÙÂÓÛ›Ó˘-·Ï-
‰ÔÛÙÂÚfiÓ˘ ÏfiÁˆ ˘ÔÍ›·˜ (27).
∆Ô ™∞∞À ÛÙÔ˘˜ ÂÓ‹ÏÈΘ Û¯ÂÙ›˙ÂÙ·È Ì ·˘ÍË̤-
ÓË Èı·ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ Û˘ÛÙËÌ·ÙÈ΋˜ ˘¤ÚÙ·Û˘
(28,29). §›Á˜ ·Ó¿ÏÔÁ˜ ÌÂϤÙ˜ ˘¿Ú¯Ô˘Ó Î·È ÛÙ·
·È‰È¿. √ Marcus et al. (30) ‰È·›ÛÙˆÛ·Ó ·‡ÍËÛË
Ù˘ ‰È·ÛÙÔÏÈ΋˜ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ ηٿ ÙË ‰È¿Ú-
ÎÂÈ· ÙÔ˘ ‡ÓÔ˘, Ë ÔÔ›· ¤ÌÂÓÂ Î·È ÌÂÙ¿ ÙËÓ ¤ÁÂÚ-
ÛË. √È Amin et al. (31) ‚Ú‹Î·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈο
·˘ÍË̤ÓË ‰È·Î‡Ì·ÓÛË Ù˘ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ (∞¶)
ηٿ ÙËÓ ÂÁÚ‹ÁÔÚÛË Î·È ÙÔÓ ‡ÓÔ, ˘„ËÏfiÙÂÚË Û¯¤-
ÛË Ó˘¯ÙÂÚÈÓ‹˜/ËÌÂÚ‹ÛÈ·˜ ∞¶ Î·È ÌÈÎÚfiÙÂÚË ÙÒÛË
Ù˘ ̤Û˘ ∞¶ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜. ∂›Û˘,
Û ¿ÏÏË ÌÂϤÙË ÙˆÓ Amin et al. (32) Ê¿ÓËÎ·Ó ÌÂÙ·-
‚ÔϤ˜ ÙÔ˘ ¿¯Ô˘˜ ÙÔ˘ ÙÔȯÒÌ·ÙÔ˜ Ù˘ ·ÚÈÛÙÂÚ‹˜
ÎÔÈÏ›·˜ Ô˘ Â›Ó·È ÂÓ‰ÂÈÎÙÈΤ˜ ·˘ÍËÌ¤ÓˆÓ ÙÈÌÒÓ Û˘-
ÛÙÔÏÈ΋˜ ·ÚÙËÚȷ΋˜ ›ÂÛ˘. ∞Ó Î·È ¯ÚÂÈ¿˙ÔÓÙ·È
ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜ Û ·È‰È¿, Â›Ó·È ÚÔÊ·Ó¤˜ fiÙÈ
ÔÈ ÌÂÙ·‚ÔϤ˜ ÙÔ˘ ÙfiÓÔ˘ ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ ¡™ Ô˘ ·-
Ú·ÙËÚÔ‡ÓÙ·È Û ·ÛıÂÓ›˜ Ì ™∞∞À Úԉȷı¤ÙÔ˘Ó ÛÂ
ÚÒÈÌË ÂÌÊ¿ÓÈÛË ˘¤ÚÙ·Û˘ (8).
™∞∞À Î·È ‰È·Ù·Ú·¯¤˜ ÙÔ˘ Ú˘ıÌÔ‡
™Â ¿ÙÔÌ· Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡-
Ô˘ ¿ÓÔȘ ·Ú·ÙËÚÂ›Ù·È ÂȉÂÈÓÔ‡ÌÂÓË ‚Ú·‰˘-
ηډ›· ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·ÓÔ˚ÎÔ‡ ÂÂÈÛÔ‰›Ô˘,
134 ∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘
Paediatriki 2009;72:134-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·134
Ô˘ ·ÎÔÏÔ˘ıÂ›Ù·È ·fi Ù·¯˘Î·Ú‰›·. ∂ÈÚfiÛıÂÙ·,
ÌÔÚ› Ó· ·Ú·ÙËÚËıÔ‡Ó ¤ÎÙ·ÎÙ˜ ÎÔÈÏȷΤ˜ Û˘-
ÛÙÔϤ˜, ηډȷΤ˜ ·‡ÛÂȘ >3 sec Î·È 2Ô˘ ‚·ıÌÔ‡
ÎÔÏÔÎÔÈÏÈ·Îfi˜ ·ÔÎÏÂÈÛÌfi˜ Ù‡Ô˘ Mobitz II (33).
ø˜ ·ıÔÁÂÓÂÙÈÎÔ› Ì˯·ÓÈÛÌÔ› ıˆÚÔ‡ÓÙ·È: ·) ÔÈ
·˘ÍË̤Ó˜ ·Ó¿ÁΘ Û Ô͢ÁfiÓÔ Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È
ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·ÓÔ˚ÎÔ‡ ÂÂÈÛÔ‰›Ô˘ Î·È Ô‰Ë-
ÁÔ‡Ó Û ÈÛ¯·ÈÌ›· ÙÔ˘ Ì˘Ôηډ›Ô˘ (34) Î·È ‚) Ë ¯ÚfiÓÈ·
˘ÔÍ·ÈÌ›· Î·È Ë ˘ÂÚηӛ·, ÔÈ Ôԛ˜ ÚÔηÏÔ‡Ó
‰È¤ÁÂÚÛË ÙÔ˘ Û˘Ì·ıËÙÈÎÔ‡ ¡™ Î·È ·ÂÏ¢ı¤ÚˆÛË
ηÙ¯ÔÏ·ÌÈÓÒÓ Ô˘ ¤¯Ô˘Ó ·ÚÚ˘ıÌÈÔÁfiÓÔ ‰Ú¿ÛË (35).
¶Ó¢ÌÔÓÈ΋ ¤ÚÙ·ÛË - ÚfiÓÈ· Ó¢ÌÔÓÈ΋
ηډȿ (cor pulmonale)
∆Ô ‚·ÛÈÎfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi Ù˘ Ó¢ÌÔÓÈ΋˜
ηډȿ˜ Â›Ó·È Ë Ó¢ÌÔÓÈ΋ ¤ÚÙ·ÛË, Ë ÔÔ›· ›ӷÈ
˘Â‡ı˘ÓË ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ˘ÂÚÙÚÔÊ›·˜ Ù˘
‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È ÙËÓ Î·Ú‰È·Î‹ ·Ó¿ÚÎÂÈ· Ù˘ ‰Â-
ÍÈ¿˜ (36). ø˜ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË ¯·Ú·ÎÙËÚ›˙ÂÙ·È
Ë ·‡ÍËÛË Ù˘ ̤Û˘ ›ÂÛ˘ ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙË-
Ú›· >25 mm Hg Û ËÚÂÌ›· Î·È >30 mm Hg ηٿ ÙËÓ
¿ÛÎËÛË (37).
∞ÈÙÈÔÏÔÁ›· Ó¢ÌÔÓÈ΋˜ ¤ÚÙ·Û˘
√È ·ÈÙÈÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ Ô‰ËÁÔ‡Ó ·fi ÙË
¯ÚfiÓÈ· ·fiÊÚ·ÍË ÛÙËÓ ÂΉ‹ÏˆÛË Ó¢ÌÔÓÈ΋˜
˘¤ÚÙ·Û˘ ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈṲ̂ÓÔÈ. ∏
¯ÚfiÓÈ· ·fiÊÚ·ÍË Î·È Ë Û˘ÓÔ‰fi˜ ˘ÔÍ·ÈÌ›·, ˘ÂÚη-
Ó›· Î·È ÔͤˆÛË Ô‰ËÁÔ‡Ó Û Ó¢ÌÔÓÈ΋ ·ÁÁÂÈÔÛ‡-
Û·ÛË (ÔÍ›· ‹ ¯ÚfiÓÈ·) (1). ∏ ÔÍ›· Á›ÓÂÙ·È Ì¤Û· ÛÂ
Ï›Á· ÏÂÙ¿ Î·È Â›Ó·È ·Ó·ÛÙÚ¤„ÈÌË Ì ÙËÓ ¿ÚÛË Ù˘
·fiÊڷ͢. ∏ ¯ÚfiÓÈ· ÔÊ›ÏÂÙ·È Û ‰ÔÌÈΤ˜ ·ÓˆÌ·-
ϛ˜ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ (˘ÂÚÙÚÔÊ›· ÙÔ˘ Ì˘-
˚ÎÔ‡ ¯ÈÙÒÓ· ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÚÙËÚÈÒÓ Ì¤ÛÔ˘ ηÈ
ÌÈÎÚÔ‡ ÌÂÁ¤ıÔ˘˜). ªÂ ÙËÓ ¿ÚÛË Ù˘ ·fiÊڷ͢, ÔÈ
ÌÂÙ·‚ÔϤ˜ ·˘Ù¤˜ ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ·Ó·ÛÙÚ¤„È̘
(1). ∏ Ó¢ÌÔÓÈ΋ ·ÁÁÂÈÔÛ‡Û·ÛË Ô‰ËÁ› Û ·‡ÍËÛË
ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁÂÈ·ÎÒÓ ·ÓÙÈÛÙ¿ÛÂˆÓ Î·È ·‡ÍË-
ÛË ÙÔ˘ ηډȷÎÔ‡ ÌÂÙ·ÊÔÚÙ›Ô˘ Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜.
∏ ÙÂÏÂ˘Ù·›· Ô‰ËÁ› Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ ÛÂ
˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È Û ‰ÂÍÈ¿ ηډȷ-
΋ ·Ó¿ÚÎÂÈ· (1). ™ÙË Û˘Ó¤¯ÂÈ·, Â¿Ó ‰ÂÓ ·ÓÙÈÌÂÙˆ-
ÈÛÙ› Ë Î·Ù¿ÛÙ·ÛË, ÌÔÚ› Ó· ·ÎÔÏÔ˘ı‹ÛÂÈ ‰˘ÛÏÂÈ-
ÙÔ˘ÚÁ›· Î·È Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜, Ì ÂÏ¿ÙÙˆÛË ÙÔ˘
fiÁÎÔ˘ ·ÏÌÔ‡ Î·È Û˘ÌÊÔÚËÙÈ΋ ηډȷ΋ ·Ó¿Ú-
ÎÂÈ·. √ Û˘Ó‰˘·ÛÌfi˜ Ù˘ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ Ù˘ ·ÚÈÛÙÂ-
Ú‹˜ Î·È ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ ÌÔÚ› Ó· ÂÈ‚·Ú‡ÓÂÈ ÙËÓ
Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· Î·È Èı·ÓfiÓ Ó· Ô‰ËÁ‹ÛÂÈ
ÛÙËÓ ÂΉ‹ÏˆÛË Ó¢ÌÔÓÈÎÔ‡ Ôȉ‹Ì·ÙÔ˜ (1).
¶ÚÔ˜ ÙÔ ·ÚfiÓ ‰ÂÓ ˘¿Ú¯Ô˘Ó ÌÂϤÙ˜ Ô˘ Ó·
·Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Û ·Ô˘Û›· ËÌÂÚ‹ÛÈ·˜ ˘ÔÍ·È-
Ì›·˜ ÌÔÚ› Ó· ÚÔÎÏËı› ÌË ·Ó·ÛÙÚ¤„ÈÌË Ó¢ÌÔ-
ÓÈ΋ ¤ÚÙ·ÛË. ∞ÓÙ›ıÂÙ·, Ë Û˘Ó‡·ÚÍË ÛÔ‚·Ú‹˜ Ó˘-
¯ÙÂÚÈÓ‹˜ ˘ÔÍ·ÈÌ›·˜ Ì ‹È· ¤ˆ˜ ̤ÙÚÈ· ËÌÂÚ‹ÛÈ·
˘ÔÍ·ÈÌ›· ‹/Î·È ˘ÂÚηӛ· ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ
ÛÙËÓ ÂÁηٿÛÙ·ÛË Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ (38).
™˘Ìو̷ÙÔÏÔÁ›· ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜
∆· Û˘ÌÙÒÌ·Ù· Ù˘ ÂÍÂÏÈÛÛfiÌÂÓ˘ Ó¢ÌÔÓÈ΋˜
˘¤ÚÙ·Û˘ ·Ú¯Èο Â›Ó·È ·Û‹Ì·ÓÙ·, ̤¯ÚÈ Ó· Û˘Ì‚Â›
ÛÔ‚·Ú‹ ηډȷ΋ ·ÔÚÚ‡ıÌÈÛË (39). ™ÙȘ Û¿ÓȘ
ÂÚÈÙÒÛÂȘ Ô˘ ¤¯ÂÈ Î·Ù·Ï‹ÍÂÈ Û ¯ÚfiÓÈ· Ó¢ÌÔ-
ÓÈ΋ ηډȿ, ÂÌÊ·Ó›˙ÂÙ·È ‰‡ÛÓÔÈ· (·fi ÂÏ¿ÙÙˆÛË
Ù˘ ‰È·Ù·ÛÈÌfiÙËÙ·˜ ÙˆÓ Ó¢ÌfiÓˆÓ), ‡ÎÔÏË Îfiˆ-
ÛË (·fi ÂÏ¿ÙÙˆÛË Ù˘ ηډȷ΋˜ ·ÚÔ¯‹˜), Û˘ÁÎÔ-
Èο ÂÂÈÛfi‰È· (·fi ·ÚÔ‰È΋ ÂÁÎÂÊ·ÏÈ΋ ÈÛ¯·È-
Ì›·), ÚÔοډȷ ¿ÏÁË ÛÙËı·Á¯ÈÎÔ‡ ¯·Ú·ÎÙ‹Ú· ηÈ
·›ÛıËÌ· ·ÏÌÒÓ. ∂›Û˘, ÙÔ Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ-
΋˜ ¿ÓÔÈ·˜ ηٿ ÙÔÓ ‡ÓÔ Ú¤ÂÈ Ó· ÙÔ ˘Ô„È·ÛÙ›
ηÓ›˜ Û ·È‰› Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ·ÓÂÍ‹ÁËÙË Î·Ú‰È·-
΋ ·Ó¿ÚÎÂÈ· Î·È ¤¯ÂÈ ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ˘ÂÚÙÚÔ-
Ê›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ (36).
∫ÏÈÓÈο Â˘Ú‹Ì·Ù· ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜
∫·Ù¿ ÙËÓ ·ÎÚfi·ÛË Ù˘ ηډȿ˜ ·Ú·ÙËÚ›ٷÈ
·˘ÍË̤ÓÔ ÙÔ Ó¢ÌÔÓÈÎfi ÛÙÔÈ¯Â›Ô ÙÔ˘ 2Ô˘ ÙfiÓÔ˘,
Û˘ÛÙÔÏÈÎfi ʇÛËÌ· ·Ó¿ÚÎÂÈ·˜ Ù˘ ÙÚÈÁÏÒ¯ÈÓ·˜ ‹
‰È·ÛÙÔÏÈÎfi ʇÛËÌ· ·Ó¿ÚÎÂÈ·˜ Ù˘ Ó¢ÌÔÓÈ΋˜,
3Ô˜ ‹ 4Ô˜ ÙfiÓÔ˜. ∂›Û˘, ÌÔÚ› Ó· ˘¿Ú¯ÂÈ ‰È¿Ù·ÛË
ÙˆÓ ÊÏ‚ÒÓ ÙÔ˘ ÙÚ·¯‹ÏÔ˘, Ë·ÙÔÌÂÁ·Ï›· Î·È ÂÚÈ-
ÊÂÚÈο Ôȉ‹Ì·Ù· (1).
∞ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜
ηډȿ˜
™ÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ·Ú·ÙËÚÂ›Ù·È ‰È¿-
Ù·ÛË Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È ÙÔ˘ ‰ÂÍÈÔ‡ ÎfiÏÔ˘, ‰È¿-
Ù·ÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜ Î·È Ó¢ÌÔÓÈ΋
Û˘ÌÊfiÚËÛË.
∏ÏÂÎÙÚÔηډÈÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ÚfiÓÈ·˜
Ó¢ÌÔÓÈ΋˜ ηډȿ˜
∂›Ó·È ÂÌÊ·Ó‹ Û ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È·, fiÔ˘
˘¿Ú¯ÂÈ ˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È Î·Ú‰È·-
΋ ·Ó¿ÚÎÂÈ· Ù˘ ‰ÂÍÈ¿˜. ¶·Ú·ÙËÚÔ‡ÓÙ·È ‰ÂÍÈ¿
ÛÙÚÔÊ‹ ÙÔ˘ ¿ÍÔÓ·, ˘„ËÏ¿ Ô͢ÎfiÚ˘Ê· P, ȉ›ˆ˜ ÛÙȘ
··ÁˆÁ¤˜ II, V1 Î·È V2 (·fi ÙËÓ ˘ÂÚÙÚÔÊ›· ÙÔ˘ ‰Â-
ÍÈÔ‡ ÎfiÏÔ˘), ˘„ËÏ¿ R ÛÙȘ ‰ÂÍȤ˜ ÚÔοډȘ ··-
ÁˆÁ¤˜ Î·È ‚·ıÈ¿ S ÛÙȘ ·ÚÈÛÙÂÚ¤˜ ÚÔοډȘ ··-
ÁˆÁ¤˜ (·fi ˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜). ªÔ-
Ú› ›Û˘ Ó· ·Ú·ÙËÚËıÔ‡Ó ‰˘ÛÚ˘ı̛˜.
∏¯ÔηډÈÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ÚfiÓÈ·˜
Ó¢ÌÔÓÈ΋˜ ηډȿ˜
™ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜, ˘¿Ú¯ÂÈ ‰È¿Ù·ÛË
Î·È ˘ÂÚÙÚÔÊ›· ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È ‰ÂÍÈÔ‡ ÎfiÏÔ˘ ηÈ
‰È¿Ù·ÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜. ªÂ ÙË ÌÂϤÙË
135∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ
¶·È‰È·ÙÚÈ΋ 2009;72:135-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·135
Doppler ‰È·ÈÛÙÒÓÂÙ·È ·Ó¿ÚÎÂÈ· ÙÚÈÁÏÒ¯ÈÓ·˜ ηÈ
Ó¢ÌÔÓÈ΋˜ ‚·Ï‚›‰·˜ Î·È ÌÂÙÚ¿Ù·È Ë ›ÂÛË ÛÙËÓ
Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›·, Ë ÔÔ›· Â›Ó·È ·˘ÍË̤ÓË (1).
™Â ÈÔ ÚÔ¯ˆÚË̤Ó˜ ηٷÛÙ¿ÛÂȘ ÌÔÚ› ·Îfi-
ÌË Ó· ·Ú·ÙËÚËı› ‰È·ÛÙÔÏÈ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘
·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ ‹/Î·È ˘ÂÚÙÚÔÊ›· Ù˘ ·ÚÈÛÙÂÚ‹˜
ÎÔÈÏ›·˜ (40,41).
£Âڷ›· ÙÔ˘ ™∞∞À
∞‰ÂÓÔÂȉÂÎÙÔÌ‹ Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹
∏ ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È
·Ì˘Á‰·ÏÒÓ ·Ú·Ì¤ÓÂÈ Ë ıÂڷ›· ÚÒÙ˘ ÂÎÏÔÁ‹˜
Î·È Â›Ó·È ·Ó·Áη›· ÛÙ· ·È‰È¿ Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó
™∞∞À (8,42,43).
∆· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ Ô˘ ¿Û¯Ô˘Ó ·fi ™∞∞À
‚ÂÏÙÈÒÓÔÓÙ·È ÛËÌ·ÓÙÈο ÌÂÙ¿ ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔÌ‹
Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹ (3,44-47). ∂›Û˘, ÔÈ Sdralis ηÈ
Berkovitz ÌÂϤÙËÛ·Ó ·Û˘Ìو̷ÙÈο ·È‰È¿ ÌÂ
˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ Î·È
‰È·›ÛÙˆÛ·Ó fiÙÈ ÔÏÏ¿ ·fi ·˘Ù¿ ·ÚÔ˘Û›·˙·Ó ΢-
„ÂÏȉÈÎfi ˘Ô·ÂÚÈÛÌfi, Ô˘ ‚ÂÏÙÈÒıËΠÌÂÙ¿ ÙË ¯ÂÈ-
ÚÔ˘ÚÁÈ΋ ¤̂·ÛË (48).
ŸÛÔÓ ·ÊÔÚ¿ ÛÙË ÌÂÌÔӈ̤ÓË ·‰ÂÓÔÂȉÂÎÙÔÌ‹,
οÔȘ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ ‰ÂÓ Â›Ó·È ·ÔÙÂÏÂÛÌ·-
ÙÈ΋ (42,49), ÂÓÒ ÌÈ· ÌÂϤÙË Ô˘ Û˘ÁÎÚ›ÓÂÈ ÙËÓ ·Ì˘-
Á‰·ÏÂÎÙÔÌ‹-·‰ÂÓÔÂȉÂÎÙÔÌ‹ Ì ÙË ÌÂÌÔӈ̤ÓË
·‰ÂÓÔÂȉÂÎÙÔÌ‹ ‰ÂÓ ¤‰ÂÈÍ ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜
·Ó¿ÌÂÛ· ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ (44).
∂›Û˘, ¿ÏÏÔ˜ ¤Ó·˜ ÛËÌ·ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ù˘
·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ Ù˘ ¤̂·Û˘ Â›Ó·È Ë ‚·Ú‡-
ÙËÙ· Ù˘ ·fiÊڷ͢. ªÈ· ÌÂϤÙË Ô˘ Û˘ÁÎÚ›ÓÂÈ ÙËÓ
·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ¤̂·Û˘ Ì ÙË ‚·Ú‡ÙË-
Ù· Ù˘ ·fiÊڷ͢ ¤‰ÂÈÍ fiÙÈ Ù· ·È‰È¿ Ô˘ ›¯·Ó
ÛÔ‚·Ú‹ ·fiÊÚ·ÍË Û˘Ó¤¯ÈÛ·Ó Ó· ¤¯Ô˘Ó ‰È·Ù·Ú·¯¤˜
‡ÓÔ˘ Î·È ÌÂÙ¿ ÙËÓ Â¤Ì‚·ÛË (50). ÕÏÏÔÈ ·Ú¿ÁÔ-
ÓÙ˜ Ô˘ Úԉȷı¤ÙÔ˘Ó Û ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À ÌÂ-
Ù¿ ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔÌ‹ Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹ Â›Ó·È Ë
Û˘Ó‡·ÚÍË ·¯˘Û·ÚΛ·˜ Î·È ÙÔ ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi
ÈÛÙÔÚÈÎfi (8,51).
√È ·ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó ÛÔ‚·Ú‹ ·fiÊÚ·ÍË ÌÔ-
Ú› Ó· ¤¯Ô˘Ó Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË ‹ Î·È Î·Ú‰È·Î‹
‰˘ÛÏÂÈÙÔ˘ÚÁ›·, ÁÈ’ ·˘Ùfi Î·È Â›Ó·È Èı·Ófi Ó· ¤¯Ô˘Ó
ÂÈÏÔΤ˜ ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË. √È ÂÈ-
ÏÔΤ˜ ÌÔÚ› Ó· Â›Ó·È ¿ÌÂÛ˜, fiˆ˜ Âȉ›ӈÛË
ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ ‹
Ó¢ÌÔÓÈÎfi Ô›‰ËÌ·, Î·È ·ÒÙÂÚ˜, fiˆ˜ ·Ú·ÌÔÓ‹
·ÚÔ‰ÈÎÔ‡ ΢„ÂÏȉÈÎÔ‡ ˘Ô·ÂÚÈÛÌÔ‡ (Ô˘ ÌÔÚ›
Ó· ¯ÚÂÈ·Ûı› ̤¯ÚÈ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ‹ ÙÚ·¯ÂÈÔÛÙÔ-
Ì›·) ‹ ›ÌÔÓË ˘ÔÍ·ÈÌ›· Î·È ‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘
(1,52). °È· ÙÔ˘˜ ·ÓˆÙ¤Úˆ ÏfiÁÔ˘˜, Û˘ÓÈÛÙ¿Ù·È Î·Ú-
‰ÈÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË Ì ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜
ÚÔÂÁ¯ÂÈÚËÙÈο Î·È ÂÈÛٷ̤ÓË ÌÂÙÂÁ¯ÂÈÚËÙÈ΋ ·-
Ú·ÎÔÏÔ‡ıËÛË Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜.
ÕÏϘ ıÂڷ›˜
™Â ·È‰È¿ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À
ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË Û˘ÓÈÛÙ¿Ù·È Ë ¯Ú‹ÛË
ÙÔ˘ ÚÈÓÈÎÔ‡ CPAP. ∂›Û˘, ¤¯ÂÈ ·Ú·ÙËÚËı› ‚ÂÏ-
Ù›ˆÛË ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÂȉÒÓ ÂÓ‰ÔÚÚÈÓÈ-
ο Û ·È‰È¿ Ì ÂÏ·ÊÚ¿ ¤ˆ˜ ̤ÙÚÈ· ·fiÊÚ·ÍË Ô˘
‰ÂÓ ¤¯Ô˘Ó ¯ÂÈÚÔ˘ÚÁËı› (53), ηıÒ˜ ›Û˘ Û ·È-
‰È¿ Ì ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À ÌÂÙ¿ ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔ-
Ì‹ Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹ (54).
∏ ¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡-
ÎÙ·˜ ÁÈ· ÙËÓ ·ÔÊ˘Á‹ Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÔÍ·ÈÌ›·˜ ‰ÂÓ
‚ÔËı¿ÂÈ Û fiÏÔ˘˜ ÙÔ˘˜ Ù‡Ô˘˜ ÙÔ˘ ™∞∞À, ‰ÈfiÙÈ ‰ÂÓ
ÚÔÊ˘Ï¿ÛÛÂÈ ·fi ÙËÓ ·fiÊÚ·ÍË ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·-
Ó¢ÛÙÈÎÔ‡ Î·È ÂÈϤÔÓ ÌÔÚ› Ó· ÂȉÂÈÓÒÛÂÈ ÙÔÓ
˘Ô·ÂÚÈÛÌfi (55). ™˘ÓÈÛÙ¿Ù·È fï˜ Ë ¯ÔÚ‹ÁËÛË Ô͢-
ÁfiÓÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ ÛÙÔ ™∞∞À Ù‡Ô˘
ππ, ·Ú¿ÏÏËÏ· ÌÂ ÙÔ ÚÈÓÈÎfi CPAP, ÂÓÒ Ë ¯ÔÚ‹ÁËÛË
Ô͢ÁfiÓÔ˘ >18 ÒÚ˜ ËÌÂÚËÛ›ˆ˜ Û˘ÓÈÛÙ¿Ù·È Û ·ÛıÂ-
Ó›˜ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó Î·È ËÌÂÚ‹ÛÈ· ˘ÔÍ·ÈÌ›· (38).
™Â ηډȷ΋ ·Ó¿ÚÎÂÈ· ÌÔÚ› ›Û˘ Ó· ÔÚË-
ÁËıÔ‡Ó ‰·ÎÙ˘Ï›Ùȉ· Î·È ‰ÈÔ˘ÚËÙÈο ̤¯ÚÈ ÙËÓ ¿ÚÛË
Ù˘ ·fiÊڷ͢ (36).
∆· ·È‰È¿ Ô˘ ¤¯Ô˘Ó ˘ÔÛÙ› ¯ÂÈÚÔ˘ÚÁÈ΋ ¤Ì-
‚·ÛË ÁÈ· ™∞∞À Ú¤ÂÈ Ó· ·ÓÂÎÙÈÌËıÔ‡Ó ÌÂÙ¿
·fi 6-8 ‚‰ÔÌ¿‰Â˜ ÁÈ· Ó· Ê·Ó› Â¿Ó ¤¯Ô˘Ó ‚ÂÏÙȈ-
ı› ‹ ¯ÚÂÈ¿˙ÔÓÙ·È ÂÈÚfiÛıÂÙË ıÂڷ›·, fiˆ˜ ÙÔ
ÚÈÓÈÎfi CPAP (1,2).
µÈ‚ÏÈÔÁÚ·Ê›·
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137∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ
¶·È‰È·ÙÚÈ΋ 2009;72:137-000
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findings of patients with adenotonsillar hypertrophy. Int JPediatr Otorhinolaryngol 2001;59:41-46.
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138 ∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘
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Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·138
√Ú›˙ÔÓÙ·˜ ÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ·¯˘Û·ÚΛ· ÛÙËÓ Ú¿ÍË:
ÌÂıÔ‰ÔÏÔÁÈο ‰ÈÏ‹ÌÌ·Ù·
¶. ¶ÂÚ‚·Ó›‰Ô˘
¶ÂÚ›ÏË„Ë: √ ÔÚÈÛÌfi˜ Î·È Ë Ì¤ÙÚËÛË Ù˘ ·¯˘Û·ÚΛ·˜ ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ· ·ÊÔÚÔ‡Ó Û ÌÂ-Á¿ÏÔ ‚·ıÌfi ÙÔÓ ·È‰›·ÙÚÔ Û‹ÌÂÚ·. √È ‰ÈÂıÓ›˜ ÔÚÈÛÌÔ› Ù˘ ·¯˘Û·ÚΛ·˜, Ô˘ ‚·Û›˙ÔÓÙ·È Î·Ù¿ ·ÚÈÔÏfiÁÔ ÛÙÔÓ ¢Â›ÎÙË ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™) Î·È ÛÙ· ÔÚȷο ÛËÌ›· ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙÔ˘˜ ÂÓ‹ÏÈΘ, ¯ÚËÛÈÌÔ-ÔÈÔ‡ÓÙ·È Û ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Î·È Ê·›ÓÂÙ·È fiÙÈ ‰ÂÓ Â›Ó·È Î·Ù¿ÏÏËÏÔÈ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ·È‰ÈÒÓ ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË. ∏ Û˘ÓÈÛÙÒÌÂÓË ÎÏÈÓÈ΋ ·ÍÈÔÏfiÁËÛË ÙÔ˘ ·È‰ÈÔ‡ ‹ ÂÊ‹‚Ô˘ ÍÂÎÈÓ¿ Ì ÙËÓ ÙÔÔı¤ÙËÛË Ù˘ ÙÈÌ‹˜ ÙÔ˘ ¢Â›-ÎÙË ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™) ÛÙ· ÈÔ Û‡Á¯ÚÔÓ· ·ÓıÚˆÔÌÂÙÚÈο ÚfiÙ˘· ‰È·ÁÚ¿ÌÌ·Ù· ÙÔ˘ ¢ª™ ·Ó¿Ê‡ÏÔ Î·È ·Ó¿ ¯ÒÚ·. ∏ ÚÔ¤ÎÙ·ÛË ÙˆÓ ÔÚÈ·ÎÒÓ ÛËÌ›ˆÓ ÙÔ˘ ˘¤Ú‚·ÚÔ˘ Î·È ÙÔ˘ ·¯‡Û·ÚÎÔ˘ ÁÈ· ÙÔ˘˜ÂÓ‹ÏÈΘ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë Î·Ù¿Ù·ÍË ÙˆÓ ·È‰ÈÒÓ ÂÏÏËÓÈ΋˜ ηٷÁˆÁ‹˜, ÂÓÒÁÈ· ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÔÛÔÛÙÔ‡ ·¯˘Û·ÚΛ·˜ ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 18 ÚfiÓˆÓ, Û ¤Ó· Û˘ÁÎÂÎÚÈ̤ÓÔ ÏË-ı˘ÛÌfi, Û˘ÓÈÛÙ¿Ù·È Ë Ì¤ÙÚËÛË ÙÔ˘ ¢ª™ Û ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ ·fi ÙÔ Ì¤ÛÔ fiÚÔ (z-score). ŸÏ˜ ÔÈ Ì¤-ıÔ‰ÔÈ Ì¤ÙÚËÛ˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÏÂÔÓÂÎÙ‹Ì·Ù· Î·È ÌÂÈÔÓÂÎÙ‹Ì·Ù·.
§¤ÍÂȘ ÎÏÂȉȿ: ¶·¯˘Û·ÚΛ·, ·È‰›, ‰Â›ÎÙ˘ Ì¿˙·˜ ÛÒÌ·ÙÔ˜, ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ, z-score.
Defining obesity in children and adolescents: methodological
dilemmas
P. Pervanidou
Abstract: πn defining obesity in children and adolescents, clinicians are attempting to identify thoseindividuals with excessive adiposity. However, the accurate estimation of body fat content and of theconsequent health implications is not an easy task. International definitions of childhood obesity, based onBody Mass Index (BMI) and adult cut-off points, which are used in large epidemiologic studies are lessvaluable in clinical practice.Definitions dependent upon a reference population, based on the concept of a certain level of deviationfrom the mean BMI of the population and using centiles and z-scores, may present variable cut-off points,as the reference curves are likely to vary between countries and may change over time within one country.In monitoring patients in the clinical setting, however, this approach provides a continuous measure in thesame patient and a comparison measure of obesity status between groups.
Key words: Obesity, child, body mass index, percentiles, z-score.
139¶ƒ∞∫∆π∫√ £∂ª∞ PRACTICAL ISSUE
π·ÙÚÂ›Ô ¶·È‰È΋˜ &∂ÊË‚È΋˜ ¶·¯˘Û·ÚΛ·˜, ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∏ ∞Á›· ™ÔÊ›·»
AÏÏËÏÔÁÚ·Ê›·:
¶·Ó·ÁÈÒÙ· ¶ÂÚ‚·Ó›‰Ô˘[email protected]∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∏ ∞Á›· ™ÔÊ›·», ∞ı‹Ó·
Childhood and AdolescenceObesity Clinic, First Department ofPediatrics, Athens UniversityMedical School, “Aghia Sophia” Children’sHospital, Athens, Greece
Correspondence:
Panagiota [email protected] for Developmental andBehavioral Paediatrics, First Department ofPaediatrics, Athens UniversityMedical School, “Aghia Sophia” Children’sHospital, Athens, Greece
¶·È‰È·ÙÚÈ΋ 2009;72:23-28
∏ ·¯˘Û·ÚΛ· ÁÂÓÈο ÔÚ›˙ÂÙ·È ˆ˜ Ë ˘ÂÚ-
‚ÔÏÈ΋ Û˘ÛÛÒÚ¢ÛË Ï›Ô˘˜ ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi, ÛÂ
‚·ıÌfi Ô˘ ÂËÚ¿˙ÂÙ·È Ë ˘Á›· ÙÔ˘ ·ÙfiÌÔ˘
(1). ∏ ÔÛÔÙÈÎÔÔ›ËÛË, fï˜, ÙÔ˘ ÏÈÒ‰Ô˘˜
ÈÛÙÔ‡, ηıÒ˜ Î·È Ô ‚·ıÌfi˜ ›‰Ú·Û˘ Ù˘ Â-
Ú›ÛÛÂÈ·˜ Ï›Ô˘˜ ÛÙËÓ ˘Á›· ÙÔ˘ ·ÙfiÌÔ˘ ‰ÂÓ Â›-
Ó·È Â‡ÎÔÏÔ Ó· ·ÍÈÔÏÔÁËıÔ‡Ó ÛÙËÓ Î·ıËÌÂÚÈÓ‹
ÎÏÈÓÈ΋ Ú¿ÍË. OÈ Û‡Á¯ÚÔÓ˜ ̤ıÔ‰ÔÈ Ì¤ÙÚË-
Û˘ Ù˘ Ì¿˙·˜ ÙÔ˘ ÏÈÒ‰Ô˘˜ ÈÛÙÔ‡, fiˆ˜ Ë Ì¤-
ÙÚËÛË Ù˘ ۈ̷ÙÈ΋˜ ˘ÎÓfiÙËÙ·˜ οو ·fi ÙÔ
ÓÂÚfi, Ë ÂÚÈÂÎÙÈÎfiÙËÙ· ۈ̷ÙÈÎÔ‡ Ï›Ô˘˜
fiˆ˜ ÂÎÙÈÌ¿Ù·È Ì¤Ûˆ ÙÔ˘ ·ÔÚÚÔÊËÛÈÔÌÂÙÚË-
Ù‹ ‰ÈÏ‹˜ ÂÓ¤ÚÁÂÈ·˜ ·ÎÙ›ÓˆÓ Ã (dual-energy X-
ry absorptiometer, DEXA), ηıÒ˜ Î·È ÔÈ Ó¤Â˜
Ù¯ÓÈΤ˜, fiˆ˜ Â›Ó·È Ë ·ÂÈÎfiÓÈÛË Ì·ÁÓËÙÈÎÔ‡
Û˘ÓÙÔÓÈÛÌÔ‡ (ªagnetic Resonance Imaging,
MRI) Î·È Ë ˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›·
(Computed Tomography, CT), ¤ÙÚ„·Ó Ó·
ÂÚÈÁÚ·Ê› Ì ·ÎÚ›‚ÂÈ· Ô ÏÈ҉˘ ÈÛÙfi˜, ·ÏÏ¿
·ÔÙÂÏÔ‡Ó ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙÔ˘˜ ‰··ÓËÚ¤˜
Î·È ¯ÚÔÓÔ‚fiÚ˜ ÂÍÂÙ¿ÛÂȘ, ÁÂÁÔÓfi˜ Ô˘ ÂÚÈÔ-
Ú›˙ÂÈ ÙË ¯Ú‹ÛË ÙÔ˘˜ ÛÙËÓ ÎÏÈÓÈ΋ ¤Ú¢ӷ (2).
√ ¢Â›ÎÙ˘ ª¿˙·˜ ™ÒÌ·ÙÔ˜
ª¤¯ÚÈ Û‹ÌÂÚ·, Û ÌÂÁ¿Ï˜ ÏËı˘ÛÌȷΤ˜
ÌÂϤÙ˜ Î·È ·ÓȯÓ¢ÙÈÎÔ‡˜ ÂϤÁ¯Ô˘˜ ¤¯Ô˘Ó ¯ÚË-
ÛÈÌÔÔÈËı› ‰Â›ÎÙ˜ ‚¿ÚÔ˘˜ ‹ Ì¿˙·˜ ÛÒÌ·ÙÔ˜,
ÔÚÈ˙fiÌÂÓÔÈ ˜ ‰È¿ÊÔÚÔÈ Û˘Ó‰˘·ÛÌÔ› ÙÔ˘ ‚¿ÚÔ˘˜
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·139
ÚÔ˜ ÙÔ ‡„Ô˜. ∞fi ÙÔ˘˜ ‰Â›ÎÙ˜ ·˘ÙÔ‡˜ ·Ó·‰Â›¯ÙËÎÂ
Ô ¢Â›ÎÙ˘ ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™), Ô˘ ÔÚ›˙ÂÙ·È ˆ˜
‚¿ÚÔ˜ ÛÒÌ·ÙÔ˜ Û ÎÈÏ¿ (kg) ‰È·ÈÚÔ‡ÌÂÓÔ ‰È· ÙÔ˘ ÙÂ-
ÙÚ·ÁÒÓÔ˘ ÙÔ˘ ‡„Ô˘˜ Û ̤ÙÚ· (m2), ˆ˜ ¤Ó· ·Ïfi ηÈ
·Ó¤ÍÔ‰Ô Ì¤ÛÔ ÔÚÈÛÌÔ‡ Ù˘ ·¯˘Û·ÚΛ·˜ ÙfiÛÔ ÛÙÔ˘˜
ÂÓ‹ÏÈΘ fiÛÔ Î·È ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜. √
¢ª™ ¤¯ÂÈ ‚ÚÂı› fiÙÈ ¤¯ÂÈ Ôχ ÈÛ¯˘Ú‹ ıÂÙÈ΋ Û˘Û¯¤-
ÙÈÛË Ì ÙÔ ÛˆÌ·ÙÈÎfi ϛԘ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ (3,4).
√ ¢ª™ ¯ÚËÛÈÌÔÔÈÂ›Ù·È Â‡ÎÔÏ· ·fi ÎÏÈÓÈÎÔ‡˜
ÁÈ·ÙÚÔ‡˜ ÔÏÏÒÓ ÂȉÈÎÔًوÓ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰È·-
ÎÚ›ÓÂÈ Ù· ·¯‡Û·Úη ·fi Ù· ˘¤Ú‚·Ú· Î·È Î·ÓÔÓÈ-
ÎÔ‡ ‚¿ÚÔ˘˜ ¿ÙÔÌ·. √ ¢ª™ >25 Î·È <30 ÔÚ›˙ÂÈ Ù·
˘¤Ú‚·Ú· Î·È Ô ¢ª™ ≥ 30 Ù· ·¯‡Û·Úη ÂÓ‹ÏÈη
¿ÙÔÌ· ÛÙË Ï¢΋ Ê˘Ï‹. ™Ù· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹-
‚Ô˘˜, Ë ·¯˘Û·ÚΛ· ÔÚ›˙ÂÙ·È Ì ‚¿ÛË ÙÔÓ ¢ª™ ·Ó¿
ʇÏÔ Î·È ËÏÈΛ·. ∫·Ù¿ ÙËÓ ÚÔÛ¤ÁÁÈÛË ·˘Ù‹, ¤¯Ô˘Ó
ÚÔÙ·ı› ÔÚȷο ÛËÌ›· (cut-off points) ÁÈ· ÙÔ
˘¤Ú‚·ÚÔ Î·È ÙËÓ ·¯˘Û·ÚΛ·, ˆ˜ ÔÛÔÛÙ¿ ¿Óˆ
·fi ¤Ó·Ó ‰Â‰Ô̤ÓÔ ¢ª™, ÁÈ· ÙËÓ ËÏÈΛ· Î·È ÙÔ Ê‡ÏÔ
ÙÔ˘ ·ÙfiÌÔ˘. √ ‰Â‰Ô̤ÓÔ˜ ·˘Ùfi˜ ¢ª™ Â›Ó·È Û˘Ó‹-
ıˆ˜ Ë Ì¤ÛË (mean) ‹ Ë ‰È¿ÌÂÛË (median) ÙÈÌ‹ Ù˘
ηٷÓÔÌ‹˜ ÂÓfi˜ ÏËı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜. H ·fiÛÙ·-
ÛË ·fi ÙË Ì¤ÛË ‹ ‰È¿ÌÂÛË ·˘Ù‹ ÙÈÌ‹ ÌÂÙÚÈ¤Ù·È Â›ÙÂ
̤ۈ ÙˆÓ ÂηÙÔÛÙÈ·›ˆÓ ı¤ÛÂˆÓ Â›Ù ̤ۈ Ù˘ ÛÙ·-
ıÂÚ‹˜ ·fiÎÏÈÛ˘ (˘ÔÏÔÁÈÛÌfi˜ ÙÔ˘ z-score).
√È ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ
∆· ‰È·ÁÚ¿ÌÌ·Ù· ·Ó·ÊÔÚ¿˜ ·‡ÍËÛ˘ (‚¿ÚÔ˘˜,
‡„Ô˘˜ Î·È ¢ª™) Ô˘ ¤¯Ô˘Ó ·Ó·Ù‡ÍÂÈ ÔÏϤ˜ ¯Ò-
Ú˜ ‚·Û›˙ÔÓÙ·È ÛÂ Û˘Á¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ ÙÔÌ‹˜
(cross sectional) ·ÓÙÈÚÔÛˆÂ˘ÙÈÎÔ‡ ‰Â›ÁÌ·ÙÔ˜
·È‰ÈÒÓ, ·fi ÙË Á¤ÓÓËÛË ˜ ÙËÓ ÂÓËÏÈΛˆÛË. ∆· Â-
ÚÈÛÛfiÙÂÚ· Û˘ÛÙ‹Ì·Ù· ηٿٷ͢ Û˘ÌʈÓÔ‡Ó Î·È
ÔÚ›˙Ô˘Ó ÙËÓ 85Ë ÂηÙÔÛÙÈ·›· ı¤ÛË (∂£) ÛÙÔ ‰È¿-
ÁÚ·ÌÌ· ¢ª™ ˆ˜ ÙÔ fiÚÈÔ ¿Óˆ ·fi ÙÔ ÔÔ›Ô ¤Ó·
·È‰› ‹ ¤ÊË‚Ô˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ˘¤Ú‚·ÚÔ˜ ηÈ
ÙËÓ 95Ë (‹ 97Ë) ∂£ ˆ˜ ÙÔ fiÚÈÔ ¿Óˆ ·fi ÙÔ ÔÔ›Ô
ÙÔ ¿ÙÔÌÔ Î·Ù·Ù¿ÛÛÂÙ·È ˆ˜ ·¯‡Û·ÚÎÔ (1,5-8). °È·
ÙȘ ¯ÒÚ˜ Ô˘ ¤¯Ô˘Ó ·Ó·Ù‡ÍÂÈ ÂıÓÈο ‰È·ÁÚ¿ÌÌ·-
Ù·, Ô ÎÏÈÓÈÎfi˜ ÔÚÈÛÌfi˜ ÙÔ˘ ˘¤Ú‚·ÚÔ˘ Î·È Ù˘ ·-
¯˘Û·ÚΛ·˜ Û˘ÓÈÛÙ¿Ù·È Ó· ‚·Û›˙ÂÙ·È ÛÙ· ‰È·ÁÚ¿ÌÌ·-
Ù· ·˘Ù¿ (8), ‰È·ÊÔÚÂÙÈο ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈ-
Ô‡ÓÙ·È Ù· ÏËÛȤÛÙÂÚ· ‰È·ı¤ÛÈÌ·.
∞˘Ùfi˜ Ô ÙÚfiÔ˜ ηٿٷ͢, fï˜, ‚·Û›˙ÂÙ·È
ÛÙËÓ È‰¤· ˆ˜ Ë ·¯˘Û·ÚΛ· ÔÚ›˙ÂÙ·È Ì ‚¿ÛË ¤Ó·
ÔÚÈṲ̂ÓÔ Â›Â‰Ô ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘ ·fi ÙÔÓ Ì¤-
ÛÔ fiÚÔ ÛÙÔÓ Û˘ÁÎÂÎÚÈ̤ÓÔ ÏËı˘ÛÌfi Ô˘ ÌÂÏÂÙ¿-
Ù·È. √ ̤ÛÔ˜ ·˘Ùfi˜ fiÚÔ˜, fï˜, ÌÔÚ› Ó· ÔÈΛÏÏÂÈ
Û ‰È¿ÊÔÚ˜ ¯ÒÚ˜, ηıÒ˜ Î·È ÛÙËÓ ›‰È· ¯ÒÚ·, fiÙ·Ó
ÌÂ ÙËÓ ¿ÚÔ‰Ô ÙˆÓ ¯ÚfiÓˆÓ ÌÂÙ·‚¿ÏÏÔÓÙ·È Ù· ·Ó-
ıÚˆÔÌÂÙÚÈο ¯·Ú·ÎÙËÚÈÛÙÈο ·˘Ù‹˜.
∏ ¯Ú‹ÛË ÙˆÓ ‰È·ÁÚ·ÌÌ¿ÙˆÓ ∂£ ÂÈÙÚ¤ÂÈ Â›-
Û˘ ÙÔÓ ¿ÌÂÛÔ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ ËÏÈΛ·˜ ·Ó·ÛÙÚÔ-
Ê‹˜ ÙÔ˘ ¢ª™ (adiposity rebound), ‰ËÏ·‰‹ Ù˘ ËÏÈ-
Λ·˜ ‰Â‡ÙÂÚ˘ ·‡ÍËÛ˘ ÙÔ˘ ¢ª™ ÌÂÙ¿ ÙË Ì›ˆÛ‹
ÙÔ˘ ηٿ ÙËÓ ÚÔÛ¯ÔÏÈ΋ ËÏÈΛ·. √ ‰Â›ÎÙ˘ ·˘Ùfi˜
·ÔÙÂÏ› ÈÛ¯˘Úfi ÚÔÁÓˆÛÙÈÎfi ‰Â›ÎÙË ÁÈ· ÙËÓ ·Ó¿-
Ù˘ÍË ·¯˘Û·ÚΛ·˜: fiÛÔ ÓˆÚ›ÙÂÚ· Û˘Ì‚Â› Ë ·Ó·-
ÛÙÚÔÊ‹ ÙÔ˘ ¢ª™, ÙfiÛÔ ÌÂÁ·Ï‡ÙÂÚÔ˜ Ô Î›Ó‰˘ÓÔ˜
·Ó¿Ù˘Í˘ ·¯˘Û·ÚΛ·˜.
∆Ô z-score
ª›· ÈÔ ·ÎÚÈ‚‹˜ Î·È ÔχÏÔÎË Ì¤ıÔ‰Ô˜ ÁÈ· ÙË
̤ÙÚËÛË Ù˘ ·¯˘Û·ÚΛ·˜ ·ÔÙÂÏ› Ô ˘ÔÏÔÁÈÛÌfi˜
ÙÔ˘ ∑ (z-score), Ô˘ ÚÔ·ÙÂÈ ·fi ÙËÓ ·Ê·›ÚÂÛË
Ù˘ ̤Û˘ ÙÈÌ‹˜ ·Ó·ÊÔÚ¿˜ ·fi ÙÔ ÌÂÙÚÔ‡ÌÂÓÔ Ì¤-
ÁÂıÔ˜ (¢ª™ ÛÙËÓ ÚÔΛÌÂÓË ÂÚ›ÙˆÛË) Î·È ÙË ‰È-
·›ÚÂÛË ‰È· Ù˘ ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘ ÙÔ˘ ÏËı˘ÛÌÔ‡
·Ó·ÊÔÚ¿˜ (¢ª™ ·ÙfiÌÔ˘ Ô˘ ÌÂÙÚ¿Ì -- ̤ÛË ÙÈÌ‹
¢ª™ ÙÔ˘ ÏËı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜ / ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË
ÏËı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜). ∆˘Èο, ¤Ó· z-score ÌÂÁ·Ï‡-
ÙÂÚÔ ‹ ›ÛÔ ÙÔ˘ +2 (2 ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ ¿Óˆ ·fi
ÙË Ì¤ÛË ÙÈÌ‹) ÔÚ›˙ÂÈ ÙËÓ ·¯˘Û·ÚΛ·.
∏ ¯Ú‹ÛË ÙÔ˘ z-score ·ÔÙÂÏ› ÈÔ ·ÎÚÈ‚‹ ̤ıÔ-
‰Ô ÔÛÔÙÈÎÔÔ›ËÛ˘ ÙÔ˘ ¢ª™ Î·È Û˘ÓÈÛÙ¿Ù·È Ó·
¯ÚËÛÈÌÔÔÈÂ›Ù·È Û ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ Ô˘ ··È-
ÙÔ‡Ó ÙË Û‡ÁÎÚÈÛË ÔÌ¿‰ˆÓ Ô˘ ÚÔ¤Ú¯ÔÓÙ·È fï˜
·fi ÙÔÓ ›‰ÈÔ ÏËı˘ÛÌfi (¤¯Ô˘Ó ÂÎÙÈÌËı› Ì ‚¿ÛË
ÙȘ ›‰È˜ η̇Ϙ) ‹ Ó· ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙËÓ ÈÔ
·ÎÚÈ‚‹ ·Ú·ÎÔÏÔ‡ıËÛË ·ÛıÂÓÒÓ Ô˘ Â›Ó·È Û Úfi-
ÁÚ·ÌÌ· ·Ú¤Ì‚·Û˘. øÛÙfiÛÔ, Ë Ì¤ıÔ‰Ô˜ ·˘Ù‹, ·Ó
Î·È ÈÔ ·ÎÚÈ‚‹˜ ·fi ÙȘ ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ, ·ÚÔ˘-
ÛÈ¿˙ÂÈ Ù· ›‰È· ·ÎÚÈ‚Ò˜ ÌÂÈÔÓÂÎÙ‹Ì·Ù·, ÁÈ·Ù› ‚·Û›˙Â-
Ù·È ÛÙËÓ ›‰È· ȉ¤·: ·fiÛÙ·ÛË ·fi ÙË Ì¤ÛË ÙÈÌ‹ ÂÓfi˜
Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ÏËı˘ÛÌÔ‡, ÌÈ· Û˘ÁÎÂÎÚÈ̤ÓË ¯ÚÔ-
ÓÈ΋ ÂÚ›Ô‰Ô. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ Ë ÚÔÛ¤ÁÁÈÛË ·˘Ù‹
‰ÂÓ ÌÔÚ› Ó· ‰Â›ÍÂÈ ÙËÓ ·‡ÍËÛË ÙÔ˘ ÂÈÔÏ·ÛÌÔ‡
Ù˘ ·¯˘Û·ÚΛ·˜ Û ¤Ó·Ó ÏËı˘ÛÌfi, ÁÈ·Ù› Ô Ì¤ÛÔ˜
¢ª™ ÁÈ· ÙÔ Ê‡ÏÔ Î·È ÙËÓ ËÏÈΛ· ·˘Í¿ÓÂÙ·È ÛÙÔÓ
ÏËı˘ÛÌfi ·Ó·ÊÔÚ¿˜. ∂›Û˘, ÔÈ Î·Ì‡Ï˜ ·Ó·ÊÔ-
Ú¿˜ ‰È·Ê¤ÚÔ˘Ó ÌÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ ˆÚÒÓ Î·È ¿Ú·
ÔÈ ÌÂÙÚ‹ÛÂȘ ÙÔ˘ z-score, fiˆ˜ Î·È Ù˘ ÂηÙÔÛÙÈ·›·˜
ı¤Û˘, ‰ÂÓ Â›Ó·È Û˘ÁÎÚ›ÛÈ̘ ÌÂٷ͇ ÙˆÓ ¯ˆÚÒÓ (9).
OÈ ‰ÈÂıÓ›˜ ÔÚÈÛÌÔ› Î·È Ë ÚÔ¤ÎÙ·ÛË ÙˆÓ
ÔÚÈ·ÎÒÓ ÛËÌ›ˆÓ ÙˆÓ ÂÓËϛΈÓ
∏ ·Ó¿ÁÎË ÁÈ· ÂÓÔÔ›ËÛË ÙÔ˘ ÔÚÈÛÌÔ‡ ÙÔ˘ ˘¤Ú-
‚·ÚÔ˘ Î·È ·¯‡Û·ÚÎÔ˘ Ì ·˘ÙfiÓ ÙˆÓ ÂÓËÏ›ÎˆÓ Ô‰‹-
ÁËÛ ÙÔÓ ¢ÈÂıÓ‹ √ÚÁ·ÓÈÛÌfi ÁÈ· ÙËÓ ∫·Ù·ÔϤÌËÛË
Ù˘ ¶·¯˘Û·ÚΛ·˜ (International Obesity Task Force-
IOTF) Ó· ÚÔÙ›ÓÂÈ ¤Ó·Ó ÎÔÈÓfi Î·È ‰ÈÂıÓ‹ ÔÚÈÛÌfi ÁÈ·
ÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ·¯˘Û·ÚΛ· (10). √È ÂÚ¢-
ÓËÙ¤˜ Û˘ÌÊÒÓËÛ·Ó fiÙÈ, ·ÚfiÙÈ Ô ¢ª™ ‰ÂÓ ·ÔÙÂÏ›
ÙÔÓ È‰·ÓÈÎfi ‰Â›ÎÙË ·¯˘Û·ÚΛ·˜, ÚÔÙ›ÓÂÙ·È ˆ˜ ÙÔ
ϤÔÓ Î·Ù¿ÏÏËÏÔ Ì¤ÛÔ ÔÚÈÛÌÔ‡ Ù˘ ·È‰È΋˜ ηÈ
ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜. ∂›Û˘, Ì ‚¿ÛË ÙÔÓ ÔÚÈÛÌfi
140 ¶. ¶ÂÚ‚·Ó›‰Ô˘
Paediatriki 2009;72:140-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·140
ÙÔ˘ IOTF, Ù· ÔÚȷο ÛËÌ›· ηٿٷ͢, Î·È ¿Ú· ÎÈÓ-‰‡ÓÔ˘, ÁÈ· Ù· ·È‰È¿ ‚Ú›ÛÎÔÓÙ·È ÛÙȘ ÂηÙÔÛÙÈ·›Â˜ı¤ÛÂȘ Ô˘ ·ÔÙÂÏÔ‡Ó ÚÔ¤ÎÙ·ÛË ÙˆÓ ÙÈÌÒÓ¢ª™=30 Î·È 25 ÙˆÓ ÂÓËÏ›ÎˆÓ ÁÈ· ÙÔÓ ÔÚÈÛÌfi ÙÔ˘·¯‡Û·ÚÎÔ˘ Î·È ÙÔ˘ ˘¤Ú‚·ÚÔ˘ ·ÓÙ›ÛÙÔȯ·.
ªÂ ‚¿ÛË ÙËÓ ÚfiÙ·ÛË ÙÔ˘ IOTF, oÈ Cole et al.(11) ·Ó¤Ù˘Í·Ó ›Ó·Î˜ Ì ÔÚȷο ÛËÌ›·, ·Ó¿ÏÔÁ·Ì ÙÔ Ê‡ÏÔ Î·È ÙËÓ ËÏÈΛ· (·Ó¿ 6 Ì‹Ó˜), Ù· ÔÔ›·ÚԤ΢„·Ó ·fi Ù· ‰Â‰Ô̤ӷ ¤ÍÈ ¯ˆÚÒÓ (µÚ·˙ÈÏ›·,ªÂÁ¿ÏË µÚÂÙ·Ó›·, Hong Kong, √ÏÏ·Ó‰›·, ™ÈÁηԇ-ÚË Î·È ∏ӈ̤Ó˜ ¶ÔÏÈÙ›˜ ∞ÌÂÚÈ΋˜). ∆· ÎÚÈÙ‹ÚÈ·ÙÔ˘ Cole ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1. ªÂ ÙÔÓÙÚfiÔ ·˘Ùfi, Ù· ÔÚȷο ÛËÌ›· ÔÚÈÛÌÔ‡ Ù˘ ·È‰È΋˜·¯˘Û·ÚΛ·˜ ‚·Û›˙ÔÓÙ·È ÛÙÔÓ ˘„ËÏfi ΛӉ˘ÓÔ ÓÔÛË-ÚfiÙËÙ·˜ ÛÙËÓ ÂÓ‹ÏÈÎË ˆ‹ Î·È fi¯È ÛÙÔÓ Ì¤ÛÔ fiÚÔ ÙÔ˘˘fi ÌÂϤÙË ÏËı˘ÛÌÔ‡, ÁÂÁÔÓfi˜ Ô˘ ÂÈÙÚ¤ÂÈ Î·ÈÙË Û‡ÁÎÚÈÛË ÙˆÓ ‰È·ÊfiÚˆÓ Ï·ÒÓ ÌÂٷ͇ ÙÔ˘˜.
∆· ÔÚȷο ÛËÌ›· ÙÔ˘ Cole ·ÔÙÂÏÔ‡Ó Û‹ÌÂÚ·
ÙËÓ ÚÔÙÂÈÓfiÌÂÓË Ì¤ıÔ‰Ô ÔÚÈÛÌÔ‡ Ù˘ ·È‰È΋˜ ηÈ
ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜ ÛÂ Û˘ÁÎÚÈÙÈΤ˜ ÂȉËÌÈÔÏÔ-
ÁÈΤ˜ ÌÂϤÙ˜, Â›Ó·È fï˜ ·Î·Ù¿ÏÏËÏË Ì¤ıÔ‰Ô˜ ‰È·-
¯ÚÔÓÈ΋˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÂÓfi˜ ·È‰ÈÔ‡ ÛÙËÓ ÎÏÈÓÈ-
΋ Ú¿ÍË (8).
¶ÂÚÈÔÚÈÛÌÔ› ÛÙË Ú‹ÛË ÙÔ˘ ¢ª™
∞Ó Î·È Ë Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ¢ª™ Î·È ÛˆÌ·ÙÈÎÔ‡
Ï›Ô˘˜ Â›Ó·È ÈÛ¯˘Ú‹ ÛÙȘ ÌÂϤÙ˜ ÂÓËϛΈÓ, Ô ¢ª™
‰ÂÓ ÌÔÚ› Ó· ‰È·¯ˆÚ›ÛÂÈ ÙË ÏÈÒ‰Ë ·fi ÙË ÌË ÏÈ-
Ò‰Ë Ì¿˙·. °È· ·Ú¿‰ÂÈÁÌ·, ÁÓˆÚ›˙Ô˘Ì fiÙÈ ÁÂÓÈο
ÙÔ ÔÛÔÛÙfi Ï›Ô˘˜ Â›Ó·È ˘„ËÏfiÙÂÚÔ ÛÙ· ı‹Ï· ·fi
fi,ÙÈ ÛÙ· ¿ÚÚÂÓ· Ì ÙÔÓ ›‰ÈÔ ¢ª™. ¶Ôχ ÂÚÈÛÛfiÙÂ-
ÚÔ, ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ Ë ·ÍÈÔÏfiÁËÛË Ì¤Ûˆ
ÙÔ˘ ¢ª™ ‰ÂÓ Ï·Ì‚¿ÓÂÈ ˘’ fi„Ë ÙËÓ Â›‰Ú·ÛË Ù˘
·‡ÍËÛ˘ Î·È Ù˘ ÂÊ˂›·˜, Ô‡Ù ÙȘ Ê˘ÛÈÔÏÔÁÈΤ˜
·ÏÏ·Á¤˜ ÛÙË Û‡ÛÙ·ÛË ÛÒÌ·ÙÔ˜. ∂ÈÚfiÛıÂÙ·, Ë ËÏÈ-
Λ· ¤Ó·Ú͢ Ù˘ ÂÊ˂›·˜ Î·È ÔÈ Û¯ÂÙÈ˙fiÌÂÓ˜ Ì ·˘-
Ù‹ ÌÂÙ·‚ÔϤ˜ ÔÈΛÏÏÔ˘Ó ÌÂٷ͇ ‰È·ÊfiÚˆÓ ÂıÓÔÏÔ-
ÁÈÎÒÓ ÔÌ¿‰ˆÓ. ∆¤ÏÔ˜, Ù· fiÚÈ· ÙÔ˘ ¢ª™ Ô˘ ÔÚ›˙Ô˘Ó
ÙËÓ ·¯˘Û·ÚΛ· Î·È ÙÔ ˘¤Ú‚·ÚÔ ÛÙ· ·È‰È¿ ‰ÂÓ
¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ Û¯ÂÙ›˙ÔÓÙ·È Ì ‚ÈÔÏÔÁÈÎfi ΛӉ˘-
ÓÔ Î·Ú‰ÈÔÌÂÙ·‚ÔÏÈÎÒÓ ÂÈÏÔÎÒÓ Û¯ÂÙÈ˙fiÌÂÓˆÓ ÌÂ
ÙËÓ ·¯˘Û·ÚΛ· ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (12,13).
H ÂÚ›ÌÂÙÚÔ˜ ̤Û˘
H ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ (¶ª) ¤¯ÂÈ ÚÔÙ·ı› ·fi
ÔÏÏÔ‡˜ ÂÚ¢ÓËÙ¤˜ ˜ ¤Ó·˜ ÂÚÈÛÛfiÙÂÚÔ ·ÍÈfiÈÛÙÔ˜
‰Â›ÎÙ˘ ÙÔ˘ ÂȤ‰Ô˘ ÙÔ˘ ÂÓ‰ÔÎÔÈÏÈ·ÎÔ‡ Ï›Ô˘˜ ηÈ
·ÔÙÂÏ› ·Ú¿ÁÔÓÙ· ηډÈÔÌÂÙ·‚ÔÏÈÎÔ‡ ÎÈÓ‰‡ÓÔ˘
ÛÙÔ˘˜ ÂÓ‹ÏÈΘ. ∏ ÛÔ˘‰·ÈfiÙËÙ· Ù˘ ̤ÙÚËÛ˘ ·˘-
Ù‹˜ Ê·›ÓÂÙ·È ·fi ÙË ¯Ú‹ÛË Ù˘, ·ÓÙ› ÙÔ˘ ¢ª™, ÛÙÔÓ
ÔÚÈÛÌfi ÙÔ˘ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ,
Û‡Ìʈӷ Ì ÙËÓ ∂ıÓÈ΋ √Ì¿‰· ∂ÈÌfiÚʈÛ˘ ÁÈ· ÙË
ÃÔÏËÛÙÂÚfiÏË (National Cholesterol Education Panel,
NCEP) Î·È ÙËÓ ¶·ÁÎfiÛÌÈ· √ÌÔÛÔÓ‰›· ¢È·‚‹ÙË
(International Diabetes Federation, IDF) (15). AÎfiÌË
ÂÚÈÛÛfiÙÂÚÔ, Ë ¯Ú‹ÛË Ù˘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘, ·ÓÙ›
ÙÔ˘ ¢ª™, ÂÂÎÙ¿ıËΠÚfiÛÊ·Ù· ÛÙËÓ ·È‰È΋ ηÈ
ÂÊË‚È΋ ËÏÈΛ·, Ì ·ÔÙ¤ÏÂÛÌ· ÙË Û‡ÌʈÓË ‰‹Ïˆ-
ÛË (consensus statement) ÂÚ¢ÓËÙÒÓ Ó· ¯ÚËÛÈÌÔÔÈ-
Â›Ù·È ÛÙËÓ ·È‰È΋ ·¯˘Û·ÚΛ· Ë ÂÚ›ÌÂÙÚÔ˜ ̤Û˘
≥90˘ ∂£, ·ÓÙ› ÙÔ˘ ¢ª™, ÛÙÔÓ ÔÚÈÛÌfi ÙÔ˘ ÌÂÙ·‚ÔÏÈ-
ÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ÛÙ· ·È‰È¿, Û‡Ìʈӷ Ì ÙËÓ ÚfiÙ·-
ÛË ÙÔ˘ πDF (16).
¶·ÚfiÙÈ ‰ÂÓ Â›Ó·È ··ÁÔÚ¢ÙÈÎfi Ó· ¯ÚËÛÈÌÔÔÈ‹-
ÛÂÈ Î·Ó›˜ ÙȘ η̇Ϙ Ô˘ ¤¯Ô˘Ó ·Ó·Ù˘¯ı› ÛÂ
¿ÏϘ ¯ÒÚ˜, Ë ÂÈÏÔÁ‹ ·˘Ù‹ Ú¤ÂÈ Ó· Â›Ó·È Ë ÙÂ-
ÏÂ˘Ù·›· Î·È Ó· Á›ÓÂÙ·È ÌfiÓÔ fiÙ·Ó ‰ÂÓ ˘¿Ú¯Ô˘Ó
ηıfiÏÔ˘ ‰È·ı¤ÛÈÌ· ÂÓ·ÏÏ·ÎÙÈο ÛÙÔȯ›· Ô˘ Ó·
ÚÔ¤Ú¯ÔÓÙ·È ·fi ÂıÓÈÎfi ÏËı˘ÛÌfi ·Ó·ÊÔÚ¿˜. °È·
ÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘ÛÌfi, ‰ËÌÔÛȇÙËÎ·Ó ÚfiÛÊ·Ù·
141√ÚÈÛÌfi˜ Ù˘ ·È‰È΋˜ Î·È ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜
¶·È‰È·ÙÚÈ΋ 2009;72:141-000
¶›Ó·Î·˜ 1. √ÚȷΤ˜ ÙÈ̤˜ ‰Â›ÎÙË Ì¿˙·˜ ÛÒÌ·ÙÔ˜ (¢ª™) ÁÈ·˘¤Ú‚·Ú· Î·È ·¯‡Û·Úη ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜, ·ÓÙ›ÛÙÔȯ˜ Ì ÙȘÔÚȷΤ˜ ÙÈ̤˜ ¢ª™ ÙˆÓ 25 kg/m2 Î·È ÙˆÓ 30 kg/m2 Ô˘ ¤¯Ô˘Ó ÔÚÈ-Ûı› ÁÈ· ÙÔ˘˜ ÂÓ‹ÏÈΘ (11).
∏ÏÈΛ· À¤Ú‚·ÚoÈ ¶·¯‡Û·ÚÎÔÈ(¯ÚfiÓÈ·) ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ· ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
2 18,4 18,0 20,1 19,82,5 18,1 17,8 19,8 19,63 17,9 17,6 19,6 19,43,5 17,7 17,4 19,4 19,24 17,6 17,3 19,3 19,24,5 17,5 17,2 19,3 19,15 17,4 17,2 19,3 19,25,5 17,5 17,2 19,5 19,36 17,6 17,3 19,8 19,76,5 17,7 17,5 20,2 20,17 17,9 17,8 20,6 20,57,5 18,2 18,0 21,1 21,08 18,4 18,4 21,6 21,68,5 18,8 18,7 22,2 22,29 19 19,1 22,8 22,89,5 19,5 19,5 23,4 23,510 19,8 19,9 24,0 24,110,5 20,2 20,3 24,6 24,811 20,6 20,7 25,1 25,411,5 20,9 21,2 25,6 26,112 21,2 21,7 26,0 26,712,5 21,6 22,1 26,4 27,213 21,9 22,6 26,8 27,813,5 22,3 23,0 27,3 28,214 22,6 23,3 27,6 28,614,5 23 23,7 28,0 28,915 23,3 23,9 28,3 29,115,5 23,6 24,2 28,6 29,316 23,9 24,4 28,9 29,416,5 24,2 24,5 29,1 29,617 24,5 24,7 29,4 29,717,5 24,7 24,9 29,7 29,818 25 25,0 30,0 30,0
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·141
η̇Ϙ ÁÈ· ÙËÓ ÂÚ›ÌÂÙÚÔ Ì¤Û˘ Û ·È‰È¿ ηÈ
ÂÊ‹‚Ô˘˜ Ù˘ ∫Ú‹Ù˘ (17). ™ÙÔ ¿ÚıÚÔ ·˘Ùfi, ·Ú¤¯Ô-
ÓÙ·È ›Ó·Î˜ Ì ÙȘ ∂£ ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, Ô˘
ÌÔÚÔ‡Ó Ó· ÚËÛÈÌÔÔÈËıÔ‡Ó Û ÎÏÈÓÈ΋ Ú‹ÛË, Ì·-
˙› Ì ÙÔÓ ¢ª™, ÁÈ· ·È‰È¿ Ù˘ ∫Ú‹Ù˘. ¶·Ú¤¯ÂÙ·È
›Û˘ ›Ó·Î·˜ Ì ÙË Ì¤ÛË ÙÈÌ‹ Î·È ÙË ÛÙ·ıÂÚ‹
·fiÎÏÈÛË ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, Ô˘ ÌÔÚ› Ó· ¯ÚË-
ÛÈÌÔÔÈËı› ÁÈ· ÙÔÓ ÈÔ ·ÎÚÈ‚‹ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ z-
score Ù˘ ¶ª ·ÔÎÏÂÈÛÙÈο Û ·È‰È¿ Ù˘ ∫Ú‹Ù˘.
øÛÙfiÛÔ, Ë ¤ÏÏÂÈ„Ë ‰ËÌÔÛÈÂ˘Ì¤ÓˆÓ ÛÙÔȯ›ˆÓ ÁÈ·
ÙËÓ ¶ª ·fi ¿ÏϘ ÂÚÈÔ¯¤˜ Ù˘ ∂ÏÏ¿‰·˜ ·Ó·‚¿ÏÏÂÈ
ÙË Ú‹ÛË Ù˘ ¶ª ÛÙÔ Û‡ÓÔÏÔ ÙÔ˘ ÂÏÏËÓÈÎÔ‡ ÏËı˘-
ÛÌÔ‡ ÁÈ· ÙÔ Ì¤ÏÏÔÓ.
ÕÏÏÔÈ ‰Â›ÎÙ˜ ·¯˘Û·ÚΛ·˜
∏ ̤ÙÚËÛË ÙÔ˘ ¿¯Ô˘˜ ÙˆÓ ‰ÂÚÌ·ÙÔÙ˘¯ÒÓ
·ÔÙÂÏ› ÌÈ· ·Ï‹ ̤ıÔ‰Ô ·ÍÈÔÏfiÁËÛ˘ Ù˘ ηٷ-
ÓÔÌ‹˜ ÙÔ˘ Ï›Ô˘˜, ÁÈ·Ù› ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı›
Û ÔÏÏ¿ ̤ÚË ÙÔ˘ ÛÒÌ·ÙÔ˜. ∏ ̤ÙÚËÛË Ù˘ ‰ÂÚÌ·-
ÙÔÙ˘¯‹˜ ÙÔ˘ ‰ÈÎÂÊ¿ÏÔ˘ ıˆÚÂ›Ù·È fiÙÈ Û¯ÂÙ›˙ÂÙ·È
ηϿ Ì ÙË ÏÈÒ‰Ë Ì¿˙·. ∏ ̤ıÔ‰Ô˜ ·˘Ù‹ ÌÔÚ› Ó·
·Ó··Ú·¯ı› ‡ÎÔÏ·, ÏfiÁˆ ÙˆÓ Ì¤ÙÚÈˆÓ Ù¯ÓÈÎÒÓ
ÈηÓÔÙ‹ÙˆÓ Ô˘ ··ÈÙ›, ·ÏÏ¿ ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂÁ¿ÏË
‰È·Î‡Ì·ÓÛË ·Ó¿ÌÂÛ· Û ‰È¿ÊÔÚÔ˘˜ ÂÍÂÙ·ÛÙ¤˜ ηÈ
‰ÂÓ ıˆÚÂ›Ù·È ·ÎÚÈ‚‹˜ (18).
√ ÏfiÁÔ˜ Ù˘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘ ÚÔ˜ ÙËÓ ÂÚ›ÌÂ-
ÙÚÔ ÁÔÊÒÓ (Waist to Hip Ratio, W/H) ¯ÚËÛÈÌÔÔÈ›-
Ù·È ÁÈ· ÙËÓ ·ÍÈÔÏfiÁËÛË Ù˘ ηٷÓÔÌ‹˜ ÙÔ˘ ۈ̷ÙÈ-
ÎÔ‡ Ï›Ô˘˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ. ∏ ¯ÚËÛÈÌfiÙËÙ· ÙÔ˘ ‰Â›-
ÎÙË ·˘ÙÔ‡ Î·È Ë Û¯¤ÛË ÙÔ˘ Ì ÙÔÓ ÌÂÏÏÔÓÙÈÎfi ηÚ-
‰È·ÁÁÂÈ·Îfi ΛӉ˘ÓÔ ‰ÂÓ Â›Ó·È ÍÂοı·Ú˜ ÛÙËÓ ·È‰È-
΋ ËÏÈΛ·. ∂›Û˘, Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÎÙÈÌËıÔ‡Ó ÌÂ-
Ù·‚ÔϤ˜ ÛÙÔ˘˜ ‰Â›ÎÙ˜ ·˘ÙÔ‡˜ ÛÙËÓ ·È‰È΋ ηÈ
ÂÊË‚È΋ ËÏÈΛ·, ÏfiÁˆ ÙˆÓ ·ÏÏ·ÁÒÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó
ηٿ ÙËÓ ·‡ÍËÛË Î·È ÙËÓ ÂÊ˂›·. ∆¤ÏÔ˜, ‰ÂÓ ¤¯Ô˘Ó
·Ó·Ù˘¯ı› η̇Ϙ ÁÈ· ÙËÓ ÂÚ›ÌÂÙÚÔ ÁÔÊÒÓ ‹
ÁÈ· ÙÔÓ ÏfiÁÔ W/H ÛÙ· ·È‰È¿ (18).
√È ÔÚÈÛÌÔ› Â‰Ò Î·È ÙÒÚ·: ÚËÛÈÌÔÔÈÒÓÙ·˜ Ù·
˘¿Ú¯ÔÓÙ· ÂÏÏËÓÈο ‰Â‰Ô̤ӷ ÛÙËÓ Ú¿ÍË
√È Î·Ì‡Ï˜ ÙÔ˘ ¢ª™ ÁÈ· ÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘-
ÛÌfi Ô˘ ‰ËÌÔÛȇÙËÎ·Ó ÙÔ 2004 (19) ·ÔÙÂÏÔ‡Ó ÙÔ
ÈÔ Û‡Á¯ÚÔÓÔ ÂÚÁ·ÏÂ›Ô ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi Ù˘ ·-
¯˘Û·ÚΛ·˜ ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜, ÙfiÛÔ ÛÂ
Â›Â‰Ô Ì·˙È΋˜ ·Ó›¯Ó¢Û˘ ÙˆÓ ·È‰ÈÒÓ Ì ·˘ÍË-
̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ηډÈÔÌÂÙ·‚ÔÏÈΤ˜ ÂÈÏÔΤ˜
fiÛÔ Î·È ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÌÂÌÔӈ̤ӈÓ
·ÛıÂÓÒÓ. ™ÙË ÌÂϤÙË ·˘Ù‹ ÌÂÙÚ‹ıËÎ·Ó 10.925 ¿ÙÔ-
Ì· ËÏÈΛ·˜ 0-18 ÂÙÒÓ, 5.537 ·ÁfiÚÈ· Î·È 5.388 ÎÔÚ›-
ÙÛÈ·, Ì ÂÚ›Ô˘ ÈÛfiÙÈÌË Î·Ù·ÓÔÌ‹ ÛÙȘ ‰È¿ÊÔÚ˜
ËÏÈ˘. ∆· ¿ÙÔÌ· Ô˘ ÌÂÙÚ‹ıËÎ·Ó ‹Ù·Ó ·fi ÙËÓ
ÂÚÈÔ¯‹ Ù˘ ∞ı‹Ó·˜ Î·È ‹Ù·Ó ÂÏÏËÓÈ΋˜ ηٷÁˆÁ‹˜
Î·È ·fi ÙÔ˘˜ ‰‡Ô ÁÔÓ›˜. ∏ ÂÚÈÔ¯‹ Ù˘ ∞ı‹Ó·˜ ‰ÂÓ
ηχÙÂÈ fiÏË ÙËÓ ∂ÏÏ¿‰·, fï˜ ÙfiÛÔ ·fi ÏÂ˘Ú¿˜
·Ó·ÏÔÁ›·˜ ÏËı˘ÛÌÔ‡ (ÂÚ›Ô˘ Ô ÌÈÛfi˜ ÏËı˘-
ÛÌfi˜ Ù˘ ¯ÒÚ·˜) fiÛÔ Î·È ·fi ÏÂ˘Ú¿˜ ÁÂÓÂÙÈÎÔ‡
˘ÔÛÙÚÒÌ·ÙÔ˜ (¿ÙÔÌ· Ì ÔÈΛÏË ÚԤϢÛË),
Ê·›ÓÂÙ·È fiÙÈ ÔÈ Î·Ì‡Ï˜ Ì ÚԤϢÛË ÙËÓ Â˘Ú‡-
ÙÂÚË ÂÚÈÔ¯‹ Ù˘ ∞ı‹Ó·˜ ·ÔÙÂÏÔ‡Ó ÈηÓÔÔÈËÙÈ-
ο ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›ÁÌ· ÙÔ˘ ÏËı˘ÛÌÔ‡.
øÛÙfiÛÔ, ÙfiÛÔ Ë ÚԤϢÛË ·fi ÂıÓÈÎfi ÏËı˘ÛÌfi
fiÛÔ Î·È Ë ÚfiÛÊ·ÙË Î·Ù·ÁÚ·Ê‹ ·ÔÙÂÏÔ‡Ó ‰‡Ô
ÈÛ¯˘Ú¿ ÛÙÔȯ›· ˘¤Ú Ù˘ ¯Ú‹Û˘ ÙˆÓ Î·Ì˘ÏÒÓ
·Ó·ÊÔÚ¿˜ ÙÔ˘ 2004 ÁÈ· ÙÔÓ ÔÚÈÛÌfi Ù˘ ·È‰È΋˜ ·-
¯˘Û·ÚΛ·˜ ÛÙËÓ ∂ÏÏ¿‰·.
™‡Ìʈӷ Ì ·˘Ù¤˜, Ë 97Ë ∂£ Î·È Ë 50Ë ∂£ ÛÂ
·ÁfiÚÈ· 18 ÂÙÒÓ Ù˘ ÂÚÈÔ¯‹˜ Ù˘ ∞ı‹Ó·˜ ·ÓÙÈÛÙÔȯ›
Û‹ÌÂÚ· Û ‚¿ÚÔ˜ Ô˘ Â›Ó·È Î·Ù¿ 15 kg Î·È Î·Ù¿ 6 kg,
·ÓÙ›ÛÙÔȯ·, ÌÂÁ·Ï‡ÙÂÚÔ ·fi ÙÔ ‚¿ÚÔ˜ ÙˆÓ ı¤ÛˆÓ
·˘ÙÒÓ ÚÈÓ 20 ÂÚ›Ô˘ ¤ÙË. ŒÙÛÈ, Ë ¯ÚËÛÈÌÔÔ›ËÛË
Ù˘ ÛËÌÂÚÈÓ‹˜ 97˘ ∂£ ˆ˜ ÎÚÈÙËÚ›Ô˘ ÁÈ· ÙÔÓ ÔÚÈÛÌfi
Ù˘ ·¯˘Û·ÚΛ·˜ ˘ÔÂÎÙÈÌ¿ ÙfiÛÔ Ù· ÔÛÔÛÙ¿ ·¯˘-
Û·ÚΛ·˜ ÛÙÔÓ ÏËı˘ÛÌfi fiÛÔ Î·È ÙËÓ ·Ó›¯Ó¢ÛË ·È-
‰ÈÒÓ Û ΛӉ˘ÓÔ, Û ۇÁÎÚÈÛË Ì ٷ ‰Â‰Ô̤ӷ Ù˘
ÂÚÈfi‰Ô˘ 1978 Î·È Ì ٷ ‰Â‰Ô̤ӷ ¿ÏÏˆÓ ¯ˆÚÒÓ. °È·
ÙÔ ÏfiÁÔ ·˘Ùfi, Î·È Ì ÙÔ ÛÎÂÙÈÎfi Ù˘ ·Ó›¯Ó¢Û˘ Â-
ÚÈÛÛfiÙÂÚˆÓ ·È‰ÈÒÓ Û ΛӉ˘ÓÔ, ÛÙËÓ ›‰È· ‰ËÌÔÛ›Â˘-
ÛË ÚÔÙ›ÓÂÙ·È Ë ·Ó¿ÏÔÁË Ì ٷ ÎÚÈÙ‹ÚÈ· ÙÔ˘ IOTF
ÚÔ¤ÎÙ·ÛË ÙˆÓ ı¤ÛÂˆÓ 25 Î·È 30 ÙˆÓ ÂÓËÏ›ÎˆÓ (∂È-
ÎfiÓ· 1) ˆ˜ ı¤ÛË ÔÚ›Ô˘ ÁÈ· ÙÔÓ ÔÚÈÛÌfi ÙÔ˘ ˘¤Ú‚·-
ÚÔ˘ Î·È ÙÔ˘ ·¯‡Û·ÚÎÔ˘ ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹-
‚Ô˘˜. ∂›Ó·È ÚÔÊ·Ó¤˜ ˆ˜ Ô ÔÚÈÛÌfi˜ ·˘Ùfi˜ ÚÔÙ›-
ÓÂÙ·È ÁÈ· ÙËÓ ∂ÏÏ¿‰· ÙÔ˘ Û‹ÌÂÚ·, ÁÈ·Ù› Û ¿ÏÏÔ˘˜
ÂıÓÈÎÔ‡˜ ÏËı˘ÛÌÔ‡˜ Ë ·fiÎÏÈÛË ·fi ÙË Ì¤ÛË ÙÈÌ‹
ÌÔÚ› Ó· ¤¯ÂÈ Ê·Ó› ÂÚÈÛÛfiÙÂÚÔ ·ÓÙÈÚÔÛˆÂ˘ÙÈ-
΋ ÙÔ˘ ˘¤Ú‚·ÚÔ˘ Î·È Ù˘ ·¯˘Û·ÚΛ·˜.
™˘ÌÂÚ¿ÛÌ·Ù·
™˘ÌÂÚ·ÛÌ·ÙÈο, Ë Ú‹ÛË ÙˆÓ ÌÂıfi‰ˆÓ ̤ÙÚËÛ˘
Ù˘ ·¯˘Û·ÚΛ·˜ ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ÛÙËÓ
ÎÏÈÓÈ΋ Ú¿ÍË ÂÍ·ÚÙ¿Ù·È ·fi ÙÔ ÂÚÒÙËÌ· Ô˘ ¤¯ÂÈ
ηÓ›˜ Ó· ··ÓÙ‹ÛÂÈ. ™ÙÔ ÂÚÒÙËÌ· ·Ó Â›Ó·È ÙÔ ·È‰›
˘¤Ú‚·ÚÔ ‹ ·¯‡Û·ÚÎÔ (Ô˘ ¤¯ÂÈ Ú·ÎÙÈ΋ ÛËÌ·Û›·:
·Ó Î·È Û ÙÈ ‚·ıÌfi Û˘ÓÈÛÙ¿Ù·È Ó· ‰ÈÂÚ¢ÓËı› ÁÈ· Û¯Â-
ÙÈ˙fiÌÂÓË Ì ÙËÓ ·¯˘Û·ÚΛ· ÓÔÛËÚfiÙËÙ·), Ë ¯Ú‹ÛË
ÙˆÓ ÎÚÈÙËÚ›ˆÓ ÙÔ˘ π√∆F ÌÔÈ¿˙ÂÈ Ó· Â›Ó·È ÔÚıfiÙÂÚË ÁÈ·
Ù· ·È‰È¿ ÂÏÏËÓÈ΋˜ ÚÔ¤Ï¢Û˘ ÙÔ˘ Û‹ÌÂÚ· Î·È Á›ÓÂ-
Ù·È ÛÙËÓ Ú¿ÍË Ì¤Ûˆ ÙˆÓ ÚfiÙ˘ˆÓ ‰È·ÁÚ·ÌÌ¿ÙˆÓ
¢ª™ ÙÔ˘ ÂÏÏËÓÈÎÔ‡ ÏËı˘ÛÌÔ‡ ÙÔ˘ 2004 (11), Ì ÙȘ
ÚÔÂÎÙ¿ÛÂȘ ÙˆÓ ı¤ÛÂˆÓ 25 Î·È 30 ÙÔ˘ ÂÓ‹ÏÈη (∂ÈÎfi-
Ó· 1). ™ÙÔ ÂÚÒÙËÌ· fiÛÔ ·¯‡Û·ÚÎÔ Â›Ó·È ¤Ó· ·È‰›,
ÙÔ z-score, ·Ó Î·È ‚·Û›˙ÂÙ·È ÛÙËÓ ›‰È· ȉ¤· Ì ÙȘ Âη-
ÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ, ·ÔÙÂÏ› Û˘ÁÎÚÈÙÈο ·ÎÚÈ‚¤ÛÙÂÚË
̤ıÔ‰Ô. √ ˘ÔÏÔÁÈÛÌfi˜ ÙÔ˘ z-score ·Ú¤¯ÂÈ ¤Ó·Ó
·ÎÚÈ‚‹ ·ÚÈıÌfi Î·È ÁÈ· Ù· ·È‰È¿ Ô˘ ‚Ú›ÛÎÔÓÙ·È Ôχ
¿Óˆ ·fi ÙËÓ 97Ë ∂£ (ÂÎÙfi˜ ÙˆÓ Î·Ì˘ÏÒÓ) - Ù·
142 ¶. ¶ÂÚ‚·Ó›‰Ô˘
Paediatriki 2009;72:142-000
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·142
ÔÔ›· ‰˘ÛÙ˘¯Ò˜ Â›Ó·È ÔÏÏ¿. ∂›Û˘, ·Ú¤¯ÂÈ ·ÎÚÈ‚‹
Î·È Û˘ÁÎÚ›ÛÈÌ· ÌÂٷ͇ ÙÔ˘˜ ÛÙÔȯ›· ÁÈ· ÙËÓ ·Ú·ÎÔ-
ÏÔ‡ıËÛË ÙÔ˘ ¢ª™ ·È‰ÈÒÓ Û ·Ú¤Ì‚·ÛË. ∆¤ÏÔ˜, ÛÂ
ÂÚ¢ÓËÙÈÎfi ›‰Ô, ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ÔÛÔÙÈ΋˜
Û‡ÁÎÚÈÛ˘ ·Ó¿ÌÂÛ· Û ÔÌ¿‰Â˜. √È ›Ó·Î˜ Ì ÙȘ ̤-
Û˜ ÙÈ̤˜ Î·È ÙȘ ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ -·fi fiÔ˘ ÌÔ-
Ú› Ó· ˘ÔÏÔÁÈÛÙ› ÙÔ z-score- ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·
ÛÙËÓ ∂ÏÏ¿‰· ÙÔ˘ Û‹ÌÂÚ· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·-
η 2 (11). ¶·ÚfiÏ· ·˘Ù¿, ηٿ ÙË Ú‹ÛË ÙÔ˘ z-score, ı·
Ú¤ÂÈ Ô ·È‰›·ÙÚÔ˜ Ó· ¤¯ÂÈ ¿ÓÙ· ÛÙÔ ÓÔ˘, Î·È Î·Ù¿
ÂÓË̤ڈÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ, fiÙÈ ÌÈÏ¿ÂÈ ÁÈ· ¤Ó·Ó ·ÚÈı-
Ìfi Ô˘ Â›Ó·È Û¯ÂÙÈ˙fiÌÂÓÔ˜ Ì ÙË Ì¤ÛË ÙÈÌ‹ ÙÔ˘ ÏË-
ı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜, Ô ÔÔ›Ô˜ ÛÙËÓ ÚÔΛÌÂÓË ÂÚ›-
ÙˆÛË ¤¯ÂÈ ¿ÚÂÈ ·ÓÈÔ‡Û· ÔÚ›·.
µÈ‚ÏÈÔÁÚ·Ê›·
1. WHO: Obesity: Preventing and Managing the Global
Epidemic. Geneva, World Health Organization, 2000.
¶›Ó·Î·˜ 2. ∆È̤˜ ¢ª™ ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ z-score (11)
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·∏ÏÈΛ· ∞ÚÈıÌfi˜ ª¤ÛË ™Ù·ıÂÚ‹ ∏ÏÈΛ· ∞ÚÈıÌfi˜ ª¤ÛË ™Ù·ıÂÚ‹
(¯ÚfiÓÈ·) ÙÈÌ‹ ·fiÎÏÈÛË (¯ÚfiÓÈ·) ÙÈÌ‹ ·fiÎÏÈÛË
1 139 14,8 2,7 1 131 14,7 2,72 114 16,9 1,6 2 111 16,1 1,73 222 15,7 2,3 3 216 15,5 1,64 268 17,7 1,7 4 267 15,6 1,75 256 15,8 1,9 5 256 15,8 2,16 249 16,3 2,2 6 237 16,4 2,57 265 16,9 2,5 7 228 16,7 2,58 282 17,7 4,4 8 248 17,5 3,29 347 18,1 3,1 9 325 18,2 3,1
10 321 18,9 3,5 10 318 18,5 3,111 327 19,3 3,4 11 342 19,1 3,312 379 20,0 3,5 12 409 19,8 3,413 453 20,7 3,7 13 423 20,5 3,714 409 21,4 3,7 14 385 21,1 3,415 443 21,8 3,7 15 428 21,4 3,316 356 22,8 4,0 16 323 21,9 3,417 387 23,1 3,7 17 370 22,1 3,118 317 23,9 4,1 18 371 22,3 3,6
143√ÚÈÛÌfi˜ Ù˘ ·È‰È΋˜ Î·È ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜
¶·È‰È·ÙÚÈ΋ 2009;72:143-000
kgr
/m2
kgr
/m2
∏ÏÈΛ· (¤ÙË)0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
34333231302928272625242322212019181716151413
34333231302928272625242322212019181716151413
∞ÁfiÚÈ·
25
10
3
75
85
95
97
50
(µMI 25)
90 (µMI 30)
kgr
/m2
kgr
/m2
∏ÏÈΛ· (¤ÙË)0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
33323130292827262524232221201918171615141312
33323130292827262524232221201918171615141312
∫ÔÚ›ÙÛÈ·
25
(µMI 30)
75
50
3
10
90
97
(µMI 25)
∂ÈÎfiÓ· 1. ∂ηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ Ô˘ ÔÚ›˙Ô˘Ó ÙÔ ˘¤Ú‚·ÚÔ Î·È ÙÔ ·¯‡Û·ÚÎÔ ·È‰›, ·Ó¿ ʇÏÔ, ˆ˜ ÚÔ¤ÎÙ·ÛË ÙˆÓ ÔÚÈ·ÎÒÓ ÛËÌ›ˆÓ ÙˆÓÂÓËϛΈÓ, ÛÙ· ÂÏÏËÓÈο ÚfiÙ˘· ‰È·ÁÚ¿ÌÌ·Ù·. ∞Ó·Ê·›ÓÂÙ·È Ë ∂£ Ô˘ ‰È·¯ˆÚ›˙ÂÈ ÙÔ ·¯‡Û·ÚÎÔ (¢ª™ >30) Î·È ¤Ú‚·ÚÔ (¢ª™ >25)·È‰› (11).
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·143
2. Lee SY, Gallagher D. Assessment methods in human bodycomposition. Curr Opin Clin Nutr Metab Care 2008Sep;11(5):566-572.
3. Revicki DA, Israel RG. Relationship between body massindices and measures of body adiposity. Am J Publ Health1986;76:992-994.
4. Spiegelman D, Isrel RG, Bouchard C, Willett WC.Absolute fat mass, percent body fat, and body fatdistribution: Which is the real determinant of bloodpressure and serum glucose? Am J Clin Nutr 1992;55:1044-1044.
5. WHO: Physical Status: The use and interpretation ofAnthropometry. Geneva, World Health Organization, 1995.
6. Himes JH, Dietz WH. Guidelines for overweight inadolescent preventive services: recommendations from anexpert committee: The Expert Committee on ClinicalGuidelines for Overweigh in Adolescent PreventiveServices. Am J Clin Nutr 1994;59:307-316.
7. Guillaume M: defining obesity in childhood: currentpractice. Am J Clin Nutr 1999;70:126S-130S.
8. Speiser PW, Rudolf MCJ, Anhalt H, Camacho-Hubner C,Chiarelli F, Eliakim A, et al., on behalf of the ObesityConsensus Working Group. Consensus Statement:Childhood Obesity. JCEM 2005;90:1871-1887.
9. Lahti-Koski M, Gill T. Defining Childhood obesity. In:Obesity in childhood and adolescence / Volume Editors W.Kiess, C. Marcus, M. Wabitsch (Pediatric and adolescentMedicine, vol 9), Karger, 2004, Basel.
10. Bellizzi MC, Dietz WH. Workshop on childhood obesity:Summary of the discussion. Am J Clin Nutr 1999;70:173S-175S.
11. Cole TJ, Bellizzi MC, Flegal KM, Dietz WH. Establishing astandard definition for child overweight and obesityworldwide: International survey. BMJ 2000;320:1240-1243.
12. Pietrobelli A, Faith MS, Allison DB, et al. Body mass indexas a measure of adiposity among children and adolescents:A validation study. J Pediatr 1998;132:204-210.
13. Garn SM, Leonard WR, Hawthorne VM. Three limitationsof the body mass index. Am J Clin Nutr 1986;44:996-997.
14. Higgins PB, Gower BA, Hunter GR, Goran MI. Defininghealth-related obesity in prepubertal children. Obes Res2001;9:233-240.
15. Pervanidou P, Kanaka-Gantenbein C, Chrousos GP.Assessment of metabolic profile in a clinical setting. CurrOpin Clin Nutr Metab Care 2006;9:589-595.
16. Zimmet P, Alberti KG, Kaufman F, Tajima N, Silink M,Arslanian S, et al.; IDF Consensus Group. The metabolicsyndrome in children and adolescents - an IDF consensusreport. Pediatr Diabetes 2007;8:299-306.
17. §ÈÓ·Ú‰¿Î˘ ∂, µ·Ú‰·‚¿˜ ∫, ∫·Ê¿ÙÔ˜ ∞, Î·È Û˘Ó.Paediatriki 2007;70:300-307.
18. ¶ÂÚ‚·Ó›‰Ô˘ ¶, ∫·Ó·Î·-Gantenbein X, ¶··ÛˆÙËÚ›Ô˘ π,ÃÚÔ‡ÛÔ˜ °¶. ¢ÈÂÚ‡ÓËÛË ÙÔ˘ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ·: Ë ·¯˘Û·ÚΛ· ˆ˜ ΢-ÚÈfiÙÂÚÔ˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜. ¢ÂÏÙ ∞ã ¶·È‰È·ÙÚ∫ÏÈÓ ¶·Ó ∞ıËÓÒÓ 2007;54:41-52.
19. Chiotis D, Krikos X, Tsiftis G, Hatzisymeaon M, Maniati-Christidi M, Dacou-Voutetakis A. Body mass index andprevalence of obesity in subjects of Hellenic origin aged 0-18years, living in the Athens area. Ann Cli Pediatr UniveAtheniensis 2004;51:139-154.
144 ¶. ¶ÂÚ‚·Ó›‰Ô˘
Paediatriki 2009;72:23-28
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·144
™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek ηÈ
ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘
Ã. ∆۷ηϛ‰Ë˜1, µ. §·ÌÚfiÔ˘ÏÔ˜2, Œ. √˘ÚÁηÓÙ˙fiÁÏÔ˘3, ¢. ƒ¿ÏÏ˘4, ∞. ¶ÂÙÚfiÔ˘ÏÔ˜2, ¡. ¡ÈÎÔÏ·˝‰Ë˜1
¶ÂÚ›ÏË„Ë: ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Â›Ó·È ‰˘ÛÏ·Û›· ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È Â›ÛÔ‰Ô˜ ÂÓ-‰ÔÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ ̤۷ ÛÙÔÓ ıÒڷη, ‰È·Ì¤ÛÔ˘ ÂÓfi˜ ÂÏÏ›ÌÌ·ÙÔ˜ ÂÌ‚Ú˘ÔÁ¤ÓÂÛ˘ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·-ÙÔ˜, Î·È ÌÔÚ› Ó· ·ÊÔÚ¿ ‰È·ÊÔÚÂÙÈο ÙÌ‹Ì·Ù· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. √ ÈÔ ÎÔÈÓfi˜ Ù‡Ô˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋-Ï˘ Â›Ó·È Ë ÔÈÛıÔÏ¿ÁÈ· ΋ÏË ÙÔ˘ Bochdalek. √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ Â›Ó·È Û¿ÓÈ· ·ÓˆÌ·Ï›·Î·È ·ÓÙÈÚÔۈ‡ÂÈ ÏÈÁfiÙÂÚÔ ·fi 5% fiÏˆÓ ÙˆÓ ÓÂÊÚÈÎÒÓ ÂÎÙÔÈÒÓ. ªÔÚ› Ó· ÂÌÊ·Ó›˙ÂÙ·È ¯ˆÚ›˜ ¿ÏÏÂ˜Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ‹ Ó· Û¯ÂÙ›˙ÂÙ·È ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÌÂÙ¿ ·fi ÙÚ·˘Ì·-ÙÈÛÌfi. ¶ÂÚÈÁÚ¿ÊÔ˘Ì ÙËÓ ÂÚ›ÙˆÛË ÂÓfi˜ ¿ÚÚÂÓÔ˜ ÓÂÔÁÓÔ‡ ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Ù‡Ô˘Bochdalek Î·È Û˘ÓÔ‰fi ¤ÎÙÔÔ ÓÂÊÚfi ÛÙÔ ¿Óˆ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ.
§¤ÍÂȘ ÎÏÂȉȿ: ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÓÂÔÁÓfi.
Bochdalek congenital diaphragmatic hernia with intrathoracic
ectopic kidney - a case report
C. Tsakalidis1, V. Lampropoulos2, E. Ourgantzoglou3, D. Rallis4, A. Petropoulos2, N. Nikolaidis1
Abstract: Congenital diaphragmatic hernia (CDH) is an abnormality in which abdominal organsenter the thorax through a developmental defect of the diaphragm. The hernia may occur in differentparts of the diaphragm, but the most common type of diaphragmatic hernia is the posterolateralBochdalek hernia. Intrathoracic ectopic kidney is a rare abnormality accounting for less than 5% of allrenal ectopia. It can occur with no other malformation or can be associated with congenitaldiaphragmatic hernia or post-traumatic diaphragmatic hernia. The case is presented of a maleneonate with a congenital diaphragmatic Bochdalek hernia, associated with renal ectopy in the leftupper hemithorax.
Key worlds: Congenital diaphragmatic hernia, neonate.
145∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏ CASE REPORT
1 µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋∞¶£, ¡ÔÛÔÎÔÌ›Զ··ÁˆÚÁ›Ô˘,£ÂÛÛ·ÏÔÓ›ÎË
2 µ’ ¶·È‰Ô¯ÂÈÚÔ˘ÚÁÈ΋∫ÏÈÓÈ΋ ∞¶£, ¡ÔÛÔÎÔÌ›Զ··ÁˆÚÁ›Ô˘,£ÂÛÛ·ÏÔÓ›ÎË
3 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞¶£, ¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞,£ÂÛÛ·ÏÔÓ›ÎË
4 ¡ÔÛÔÎÔÌÂ›Ô ∂ȉÈÎÒÓ¶·ı‹ÛˆÓ, £ÂÛÛ·ÏÔÓ›ÎË
AÏÏËÏÔÁÚ·Ê›·:
ÃÚ‹ÛÙÔ˜ ∆۷ηϛ‰Ë˜[email protected]¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘»,£ÂÛÛ·ÏÔÓ›ÎË
1 2nd Department ofNeonatal Paediatrics,“Aristotelio” University ofThessaloniki,“Papageorgiou” Hospital,Thessaloniki, Greece
2 2nd Department ofPaediatric Surgery,“Aristotelio” University ofThessaloniki,“Papageorgiou” Hospital,Thessaloniki, Greece
3 2nd Department ofPaediatrics, “Aristotelio”University of Thessaloniki,“AHEPA” Hospital,Thessaloniki, Greece
4 Infectious DiseasesHospital, Thessaloniki,Greece
Correspondence:
Christos [email protected]“Papageorgiou” Hospital,Thessaloniki, Greece
¶·È‰È·ÙÚÈ΋ 2009;72:29-40
∂ÈÛ·ÁˆÁ‹
ŒÎÙÔÔ˜ ÓÂÊÚfi˜ ··ÓÙ¿Ù·È Û 1:1.000 ÁÂÓ-Ó‹ÛÂȘ, ·ÏÏ¿ ÌfiÓÔ 1 ÛÙȘ 10 ·fi ·˘Ù¤˜ ÙȘ ÂÚÈ-ÙÒÛÂȘ ‰È·ÁÈÁÓÒÛÎÂÙ·È (1). √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜¤ÎÙÔÔ˜ ÓÂÊÚfi˜ ÂÌÊ·Ó›˙ÂÙ·È ·ÎfiÌË ÈÔ Û¿ÓÈ·Î·È ¤¯ÂÈ ÙË ÌÈÎÚfiÙÂÚË Û˘¯ÓfiÙËÙ· Û ۯ¤ÛË ÌÂÙȘ ÏÔȤ˜ ÂÎÙԛ˜ ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ (2,3),Â›Ó·È Î·Ù¿ ηÓfiÓ· ·Û˘Ìو̷ÙÈÎfi˜ Î·È ·ÔÙÂ-Ï› Ù˘¯·›Ô ‡ÚËÌ· ÛÙËÓ ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›·ıÒÚ·ÎÔ˜. ∞ÎfiÌ· Û·ÓÈfiÙÂÚË Â›Ó·È Ë ‰È¿ÁÓˆÛËÙ˘ ‡·Ú͢ ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚԇηٿ ÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·. ¶ÂÚÈÁÚ¿ÊÔ˘Ì ÙËÓÂÚ›ÙˆÛË ÂÓfi˜ ¿ÚÚÂÓÔ˜ ÓÂÔÁÓÔ‡ ÌÂ Û˘ÁÁÂÓ‹‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Ù‡Ô˘ Bochdalek ηÈÛ˘ÓÔ‰fi ¤ÎÙÔÔ ÓÂÊÚfi ÛÙÔ ¿Óˆ ·ÚÈÛÙÂÚfi ËÌÈ-ıˆÚ¿ÎÈÔ, Ë ‰È¿ÁÓˆÛË ÙˆÓ ÔÔ›ˆÓ ¤ÁÈÓ ÚÔÁÂÓ-ÓËÙÈο, Û ËÏÈΛ· ·ËÛ˘ 32 ‚‰ÔÌ¿‰ˆÓ.
¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘
¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ¿ÚÚÂÓÔ˜ ÓÂÔ-
ÁÓÔ‡ Ô˘ ÁÂÓÓ‹ıËΠÌÂÙ¿ ·fi ·ËÛË ‰È¿ÚÎÂÈ·˜
34 ‚‰ÔÌ¿‰ˆÓ Ì ηÈÛ·ÚÈ΋ ÙÔÌ‹, ÏfiÁˆ ÚÔ-
ÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ™¢∫, ÓÂÊÚÈ΋˜ ÂÎÙÔ›·˜
Î·È ÔÏ˘·ÌÓ›Ô˘.
¶ÚfiÎÂÈÙ·È ÁÈ· ÙÔ ÚÒÙÔ ·È‰› Ê·ÈÓÔÙ˘Èο
˘ÁÈÒÓ, ÌË Û˘ÁÁÂÓÒÓ ÌÂٷ͇ ÙÔ˘˜ ÁÔÓ¤ˆÓ, ÌÂ
ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ÂχıÂÚÔ. ∆Ô ‚¿ÚÔ˜
Á¤ÓÓËÛ˘ ‹Ù·Ó 2770 g (50Ë-75Ë ∂£). ∞fi ÙÔ
ÂÚÈÁÂÓÓËÙÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È ˆ˜ ÛÙÔÓ
˘ÂÚ˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ ÂÌ‚Ú‡Ô˘, Ô˘
¤ÁÈÓ Û ËÏÈΛ·˜ ·ËÛ˘ 32 ‚‰ÔÌ¿‰ˆÓ, ‰È·È-
ÛÙÒıËÎÂ Ë ‡·ÚÍË Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ-
΋Ï˘ ·ÚÈÛÙÂÚ¿, Ì ›ÛÔ‰Ô ÙÔ˘ ÛÙÔÌ¿¯Ô˘ ηÈ
ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘, ÌÂ Û˘ÓÔ‰fi ·ÚÂÎÙfiÈ-
ÛË Ù˘ ηډȿ˜ ÚÔ˜ Ù· ‰ÂÍÈ¿, ·ÏÏ¿ Î·È Û˘Ó‡-
·ÚÍË ¤ÎÙÔÔ˘ ·ÚÈÛÙÂÚÔ‡ ÓÂÊÚÔ‡ ÛÙÔ Ô›ÛıÈÔ
ÙÌ‹Ì·. √È ‚ÈÔÊ˘ÛÈΤ˜ ·Ú¿ÌÂÙÚÔÈ ÙÔ˘ ÂÌ‚Ú‡-
Ô˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜.
∏ ÌËÙ¤Ú· Û fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔ-
Û‡Ó˘ ‚ÚÈÛÎfiÙ·Ó ˘fi ·ÁˆÁ‹ Ì Û΢¿ÛÌ·Ù·
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·145
Ûȉ‹ÚÔ˘, ·Û‚ÂÛÙ›Ô˘, Ê˘ÏÏÈÎÔ‡ ÔͤԘ Î·È Ì·ÁÓËÛ›Ô˘.
¢ÂÓ Î¿ÓÈ˙Â Î·È ‰ÂÓ ‹Ù·Ó ¯Ú‹ÛÙ˘ ·ÏÎÔfiÏ ‹ ¿ÏψÓ
Ô˘ÛÈÒÓ. ¶ÚÔËÁ‹ıËΠˆÚ›Ì·ÓÛË ÙˆÓ Ó¢ÌfiÓˆÓ ÙÔ˘
ÂÌ‚Ú‡Ô˘ Ì ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙË ÌË-
Ù¤Ú· Ì›· ‚‰ÔÌ¿‰· ÚÈÓ ·fi ÙÔÓ ÙÔÎÂÙfi.
∏ Ú‹ÍË ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ˘Ì¤ÓˆÓ ¤ÁÈÓ ηٿ ÙËÓ
¤̂·ÛË Î·È ÙÔ ·ÌÓÈ·Îfi ˘ÁÚfi ‹Ù·Ó ‰È·˘Á¤˜. ∆Ô ÓÂ-
ÔÁÓfi ÁÂÓÓ‹ıËÎÂ Î·È ¤ÎÏ·„ ·Ì¤Ûˆ˜, ·ÏÏ¿ ·Ì¤Ûˆ˜
ÂÁη٤ÛÙËÛ ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, Ì ÂÈÛÔÏΤ˜
ÌÂÛÔÏ¢ڛˆÓ ‰È·ÛÙËÌ¿ÙˆÓ, ÁÔÁÁ˘ÛÌfi Î·È Î˘¿Óˆ-
ÛË. ªÂ ÁÓˆÛÙ‹ ÙËÓ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Ù˘
™¢∫, ÙÔÔıÂÙ‹ıËΠ·Ì¤Ûˆ˜ ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎfi˜ η-
ıÂÙ‹Ú·˜ Î·È ¤ÁÈÓ ‰È·ÛˆÏ‹ÓˆÛË ÁÈ· ¿ÌÂÛË ˘ÔÛÙ‹-
ÚÈÍË ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡. ∆Ô Apgar score ‹Ù·Ó 17 ηÈ
58 (‰È·ÛˆÏËӈ̤ÓÔ). ∆· ·¤ÚÈ· ÙÔ˘ ÔÌÊ·Ï›Ô˘ ÏÒÚÔ˘
‹Ù·Ó: p∏=7,32, pO2=23,6 mmHg, pCO2=58,6
mmHg, SBE=2,3 mmol/L. ∆Ô ÓÂÔÁÓfi ÌÂٷʤÚıËÎÂ
‰È·ÛˆÏËӈ̤ÓÔ Ì ambu Û ÎÏÂÈÛÙ‹ ıÂÚÌÔÎÔÈÙ›‰·.
∆· ·¤ÚÈ· ·fi ÂÚÈÊÂÚÈ΋ ·ÚÙËÚ›· ÙÔ˘ ÓÂÔÁÓÔ‡ Ï›Á·
ÏÂÙ¿ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, ˘fi Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi,
‹Ù·Ó: pH=7,25, pO2=180,6 mmHg, pCO2=46,8
mmHg, SBE=-7 mmol/L.
∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒڷη ÂȂ‚·›ˆÛ ٷ
Â˘Ú‹Ì·Ù· ÙÔ˘ ÚÔÁÂÓÓËÙÈÎÔ‡ ÂϤÁ¯Ô˘ (∂ÈÎfiÓ· 1).
∏ ÛÙ·ıÂÚ‹ ÎÏÈÓÈ΋ Î·È ÂÚÁ·ÛÙËÚȷ΋ ÂÈÎfiÓ· ÙÔ˘ ÓÂ-
ÔÁÓÔ‡ ¤ÙÚ„ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË Ù˘
‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ÙÔ ÚÒÙÔ 24ˆÚÔ, Ì ÙÔÔı¤ÙË-
ÛË Ï¤ÁÌ·ÙÔ˜ Goretex Î·È ÌÂÙ·ÊÔÚ¿ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡
ÓÂÊÚÔ‡ ÛÙËÓ Ô›ÛıÈ· ¿Óˆ ·ÚÈÛÙÂÚ‹ ÎÔÈÏȷ΋ ¯ÒÚ·.
ÃÔÚËÁ‹ıËΠÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ‰‡Ô
146 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:146-000
∂ÈÎfiÓ· 1. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘. AÍÔÓÈ΋ ÙÔÌÔ-ÁÚ·Ê›· ıÒÚ·ÎÔ˜.
A B
° ¢
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·146
ÊÔÚ¤˜, ÙËÓ 1Ë Î·È ÙË 12Ë ÒÚ· ˙ˆ‹˜, ·Ì¤Ûˆ˜ ÌÂÙ¿
ÙÔ ¯ÂÈÚÔ˘ÚÁ›Ô. ∏ ¤ÎÙ˘ÍË ÙÔ˘ Ó‡ÌÔÓ· ‹Ù·Ó η-
Ï‹, fï˜ ÏfiÁˆ ‰È·Ù·Ú·¯ÒÓ Ù˘ Û¯¤Û˘ ·ÂÚÈÛÌÔ‡/
·ÈÌ¿ÙˆÛ˘ ·fi ÙË Û˘Ó˘¿Ú¯Ô˘Û· Ó¢ÌÔÓÈ΋
˘ÔÏ·Û›·, ÙÔ ·Ó·Ó¢ÛÙÈÎfi Û‡ÛÙËÌ· ˘ÔÛÙËÚ›-
¯ıËΠ̠˘„›Û˘¯ÓÔ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi, Ì ¯ÔÚ‹ÁË-
ÛË ÌÔÓÔÍÂȉ›Ô˘ ÙÔ˘ ·˙ÒÙÔ˘. ÃÔÚËÁ‹ıËÎ·Ó ÈÓfiÙÚÔ-
· ÁÈ· ÙËÓ ÂÓ›Û¯˘ÛË Ù˘ ÂÚÈÊÂÚÈ΋˜ ΢ÎÏÔÊÔÚ›·˜
Î·È ‰ÈÏ‹ ·ÓÙÈ‚ÈÔÙÈ΋ ·ÁˆÁ‹. ∏ ‰È¿ÁÓˆÛË Ù˘ ·-
ڷ̤ÓÔ˘Û·˜ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ ÂȂ‚·ÈÒ-
ıËΠ̠˘ÂÚ˯ÔÁÚ¿ÊËÌ· Doppler ηډȿ˜ ηÈ
Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ.
√ ·ӤÏÂÁ¯Ô˜ ÙË 12Ë Ë̤ڷ ˙ˆ‹˜ ¤‰ÂÈÍ ÛËÌ·-
ÓÙÈ΋ ‚ÂÏÙ›ˆÛË ÛÙȘ ·ÓÙÈÛÙ¿ÛÂȘ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ
·ÁÁ›ˆÓ. ∆Ë 13Ë Ë̤ڷ ÓÔÛËÏ›·˜ ÂΉ‹ÏˆÛ ÂÈÎfiÓ·
ÛË„·ÈÌ›·˜ Ì ·‡ÍËÛË Ù˘ CRP Î·È Ë Î·ÏÏȤÚÁÂÈ· ·›-
Ì·ÙÔ˜ ‹Ù·Ó ıÂÙÈ΋ ÁÈ· St. epidermidis. ∏ ¯ÔÚ‹ÁËÛË
·ÓÙÈ‚ÈÔÙÈ΋˜ ·ÁˆÁ‹˜ ‹Ù·Ó ·ÔÙÂÏÂÛÌ·ÙÈ΋. ∆Ë 17Ë
Ë̤ڷ ÓÔÛËÏ›·˜ ‰È·ÈÛÙÒıËΠÂÎÚÔ‹ ¯ÔÏÒ‰Ô˘˜
˘ÁÚÔ‡ ·fi ÙÔ ¯Â›ÏÔ˜ ÙÔ˘ ÎÔÈÏÈ·ÎÔ‡ ¯ÂÈÚÔ˘ÚÁÈÎÔ‡
ÙÚ·‡Ì·ÙÔ˜ ηÈ, ÏfiÁˆ ÂÌÌÔÓ‹˜, ·ÔÊ·Û›ÛÙËΠÂÚ¢-
ÓËÙÈ΋ Ï··ÚÔÙÔÌ›·, fiÔ˘ Î·È ‰È·ÈÛÙÒıËΠË
‡·ÚÍË, ·ÎÚÈ‚Ò˜ οو ·fi ÙÔ Ï¤ÁÌ·, ‰È·ÙÂٷ̤-
Ó˘ ¤ÏÈη˜ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘, Ô˘ ¤ÊÂÚ ÌÔÓ‹ÚË Ú‹-
ÍË ÛÙË ÌÂÛfiÙËÙ¿ Ù˘, ÌÂ Û˘Ú›ÁÁÈÔ ÚÔ˜ ÙÔ ÎÔÈÏÈ·Îfi
ÙÔ›¯ˆÌ·, ·fi fiÔ˘ ÂÍÂÚ¯fiÙ·Ó ÙÔ ˘ÎÓfiÚÚ¢ÛÙÔ
¯ÔÏ҉˜ ÂÚȯfiÌÂÓÔ. ¶Ú·ÁÌ·ÙÔÔÈ‹ıËΠÂÎÙÔÌ‹
ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘ Î·È ‰ÈÂÓ¤ÚÁÂÈ· ‰ÈÏ‹˜ ÛÙÔ-
Ì›·˜, ÙÔÔıÂÙ‹ıËΠ·ÚÔ¯¤Ù¢ÛË ÂÓÙfi˜ Ù˘ ÂÚÈ-
ÙÔÓ·˚΋˜ ÎÔÈÏfiÙËÙ·˜ Î·È ¤ÁÈÓ ۇÁÎÏÂÈÛË ÙÔ˘ ÙÚ·‡-
Ì·ÙÔ˜ Ì ηÈÓÔ‡ÚÁÈÔ Ï¤ÁÌ· Goretex. ∏ ÈÛÙÔ·ıÔ-
ÏÔÁÈ΋ ÂͤٷÛË ÙÔ˘ ÂÓÙ¤ÚÔ˘ ¤‰ÂÈÍ ÌfiÓÔ ÛËÌ›·
ÊÏÂÁÌÔÓ‹˜.
∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒڷη Ô˘ ‰ÈÂÓÂÚÁ‹ıË-
ΠÙËÓ ÂfiÌÂÓË Ë̤ڷ ¤‰ÂÈÍ ·ÙÂÏÂÎÙ·Û›· Î·È Ì›ˆ-
ÛË ÙÔ˘ fiÁÎÔ˘ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ Ó‡ÌÔÓ·. ™ÙÔÓ ·ÛıÂ-
Ó‹ ¯ÔÚËÁ‹ıËÎ·Ó ÂÈÛÓÂfiÌÂÓ· ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο
Î·È ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹. ∆Ô ÓÂÔÁÓfi ÂÙ¤ıË Û ·ÁˆÁ‹ ÌÂ
‰ÔÌÂÚȉfiÓË ÏfiÁˆ Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ΋˜ ·ÏÈÓ‰Úfi-
ÌËÛ˘. ∫ÂÓÙÚÈÎfi˜ ÊÏ‚ÈÎfi˜ ηıÂÙ‹Ú·˜ Ù‡Ô˘
Hickmann ÙÔÔıÂÙ‹ıËΠÛÙÔ ÓÂÔÁÓfi Î·È ·Ú¤ÌÂÈÓÂ
Û˘ÓÔÏÈο ÁÈ· 35 Ë̤Ú˜. √ ·ÛıÂÓ‹˜ ·Ú¤ÌÂÈÓ ÛÂ
Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi Û˘ÓÔÏÈο 46 Ë̤Ú˜. ªÂÙ¿ ÙËÓ
·Ô‰¤ÛÌ¢ÛË ·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ¯ÚÂÈ¿ÛıË-
Π¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ ÁÈ· 9 Ë̤Ú˜. ∆ËÓ 58Ë Ë̤ڷ
˙ˆ‹˜ ¤ÁÈÓ ¤̂·ÛË ·Ó·ÛÙfïÛ˘ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡
Î·È ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ÎÔÏÔ‚ÒÌ·ÙÔ˜ ÙÔ˘ ÏÂÙÔ‡
ÂÓÙ¤ÚÔ˘.
∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘, Ô ·ÛıÂÓ‹˜
ÂÙ¤ıË Û ÔÏÈ΋ ·ÚÂÓÙÂÚÈ΋ ‰È·ÙÚÔÊ‹ ÁÈ· 11 Ë̤-
Ú˜, ÛÙË Û˘Ó¤¯ÂÈ· Û ÌÈÎÙ‹ Û›ÙÈÛË Î·È ·fi ÙËÓ 72Ë
Ë̤ڷ ˙ˆ‹˜ ‚ÚÈÛÎfiÙ·Ó Û ϋÚË ÂÓÙÂÚÈ΋ Û›ÙÈÛË,
ηϿ ·ÓÂÎÙ‹. ∆· ˘ÂÚ˯ÔÁÚ·Ê‹Ì·Ù· ÂÁÎÂÊ¿ÏÔ˘
‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. √ ÔÊı·ÏÌÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ηٿ
ÙËÓ ¤ÍÔ‰fi ÙÔ˘ ·fi ÙËÓ ÎÏÈÓÈ΋ ‰ÂÓ ¤‰ÂÈÍ ·ıÔÏÔÁÈ-
ο Â˘Ú‹Ì·Ù·. √ ·ÛıÂÓ‹˜ ÂÍ‹Ïı ·fi ÙËÓ ÎÏÈÓÈ΋
ÙËÓ 85Ë Ë̤ڷ ˙ˆ‹˜ Ì ‚¿ÚÔ˜ 3.680 g (<10Ë ∂£),
Ì‹ÎÔ˜ 54 cm (25Ë ∂£) Î·È ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜ 38 cm
(50Ë ∂£). ŒÏ·‚ ˆ˜ ·ÁˆÁ‹ ÂÈÛÓÂfiÌÂÓ· ‚ÚÔÁ¯Ô‰È·-
ÛÙ·ÏÙÈο Î·È ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ÁÈ· ÙȘ ÂΉËÏÒÛÂȘ
Ù˘ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ÙÔ˘ ‰˘ÛÏ·Û›·˜ Î·È Û΢¿-
ÛÌ·Ù· Ûȉ‹ÚÔ˘ Î·È Û‡ÌÏÂÁÌ· ‚ÈÙ·ÌÈÓÒÓ ∞ Î·È D.
∆Ô ÛÙ·ÙÈÎfi (∂ÈÎfiÓ· 2) Î·È ‰˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿-
ÊËÌ· ÓÂÊÚÒÓ Ì Tc-99m Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËηÓ
Û ËÏÈΛ· 6 ÌËÓÒÓ ¤‰ÂÈÍ·Ó Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁ›·
Î·È ÙˆÓ ‰‡Ô ÓÂÊÚÒÓ.
™˘˙‹ÙËÛË
∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Â›Ó·È ‰˘-
ÛÏ·Û›· ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È ÚfiÙˆÛË ÂÓ‰Ô-
ÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ ̤۷ ÛÙÔ ıÒڷη, ‰È·Ì¤ÛÔ˘
ÂÓfi˜ ·Ó·ÙÔÌÈÎÔ‡ ÂÏÏ›ÌÌ·ÙÔ˜ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. ∏
‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÌÔÚ› Ó· ·ÊÔÚ¿ ‰È·ÊÔÚÂÙÈο
ÙÌ‹Ì·Ù· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. √ ÈÔ ÎÔÈÓfi˜ Ù‡Ô˜ ‰È·-
ÊÚ·ÁÌ·ÙÔ΋Ï˘ Â›Ó·È Ë Ï·ÁÈÔ›ÛıÈ· ΋ÏË ÙÔ˘
Bochdalek, Ë Û˘¯ÓfiÙËÙ· Ù˘ ÔÔ›·˜ ÂÎÙÈÌ¿Ù·È ÂÚ›-
Ô˘ ÛÙÔ 90-95% (4). ∏ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛÂ-
ˆÓ ™¢∫ Ù‡Ô˘ Bochdalek ·ÊÔÚ¿ ÙËÓ ·ÚÈÛÙÂÚ‹ Ï¢-
Ú¿ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ·ÏÏ¿ Ù· ÔÛÔÛÙ¿ ÙˆÓ ‰ÂÍÈ¿
Î·È ·ÚÈÛÙÂÚ¿ ÂÓÙÔÈ˙fiÌÂÓˆÓ ™¢∫ ÔÈΛÏÏÔ˘Ó. √È
™¢∫ ÂÓÙÔ›˙ÔÓÙ·È ·ÚÈÛÙÂÚ¿ Û ÔÛÔÛÙfi 73-90%,
147™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek
¶·È‰È·ÙÚÈ΋ 2009;72:29-40
∂ÈÎfiÓ· 2. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek Î·È ÂÓ‰Ô-ıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘. ™Ù·ÙÈÎfiÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ.
Anterior Posterior
Left Post Oblique Right Post Oblique
Left54.5%
Right 45.5%
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·147
‰ÂÍÈ¿ Û ÔÛÔÛÙfi 8-24% Î·È ·ÌÊÔÙÂÚfiÏ¢ڷ ‹ ÎÂ-
ÓÙÚÈο Û ÔÛÔÛÙfi 1,2-4,6% (5,6,7). ∏ ™¢∫ Ù‡Ô˘
Bochdalek ÂÚÈÁÚ¿ÊËÎÂ ÁÈ· ÚÒÙË ÊÔÚ¿ ÙÔ 1948 (8).
√ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ Â›Ó·È Û¿ÓÈ·
·ÓˆÌ·Ï›· Î·È ·ÓÙÈÚÔۈ‡ÂÈ ÏÈÁfiÙÂÚÔ ·fi 5%
fiÏˆÓ ÙˆÓ ÓÂÊÚÈÎÒÓ ÂÎÙÔÈÒÓ (2,9). ªÔÚ› Ó· ÂÌ-
Ê·Ó›˙ÂÙ·È ¯ˆÚ›˜ ¿ÏϘ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ‹ Ó· Û¯Â-
Ù›˙ÂÙ·È ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ‹ ‰È·ÊÚ·ÁÌ·-
ÙÔ΋ÏË ÌÂÙ¿ ·fi ÙÚ·˘Ì·ÙÈÛÌfi (10). ™Â 15.919 ·˘ÙÔ-
„›Â˜ ·È‰ÈÒÓ Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËηÓ, ÌfiÓÔ 1 ÂÚ›-
ÙˆÛË ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡ ·Ú·ÙËÚ‹-
ıËÎÂ, ·Ó¿ÌÂÛ· Û 22 ÂÚÈÙÒÛÂȘ ÓÂÊÚÈ΋˜ ÂÎÙÔ›·˜
(11). ∏ ÚÒÙË ÂÚ›ÙˆÛË ÎÏÈÓÈο ‰È·ÁÓˆṲ̂Ó˘ ÂÓ-
‰ÔıˆÚ·ÎÈ΋˜ ÓÂÊÚÈ΋˜ ÂÎÙÔ›·˜ ÂÚÈÁÚ¿ÊËΠ·fi
ÙÔÓ Wolfromm ÙÔ 1940 (12). ∆Ô 1987, ÔÈ Donat ηÈ
Donat ÌÂϤÙËÛ·Ó ÂÚÈÙÒÛÂȘ Ô˘ ‰ËÌÔÛȇıËηÓ
‰ÈÂıÓÒ˜ ·fi ÙÔ 1922 ¤ˆ˜ ÙÔ 1986 Î·È Î·Ù¤ÏËÍ·Ó ÛÙÔ
Û˘Ì¤Ú·ÛÌ· fiÙÈ Ë Û˘ÁÁÂÓ‹˜ ·˘Ù‹ ·ÓˆÌ·Ï›· ÂÌÊ·Ó›-
˙ÂÙ·È Û˘¯ÓfiÙÂÚ· ÛÙËÓ ·ÚÈÛÙÂÚ‹ (62%) ·fi fi,ÙÈ ÛÙË
‰ÂÍÈ¿ ÏÂ˘Ú¿ (36%). ∆Ô 2% ÙˆÓ ·ÛıÂÓÒÓ ¤¯ÂÈ ·ÌÊÔ-
ÙÂÚfiÏ¢ÚË ÂÓ‰ÔıˆÚ·ÎÈ΋ ÓÂÊÚÈ΋ ÂÎÙÔ›·. √ ·ÚÈ-
ÛÙÂÚfi˜ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÛÎÂ-
ÏÂÙÈΤ˜ Î·È Î·Ú‰È·Î¤˜ ·ÓˆÌ·Ï›Â˜ (13). ∂ÈÚfiÛıÂÙ·,
Ë Û˘ÁÁÂÓ‹˜ ·˘Ù‹ ·ÓˆÌ·Ï›· ··ÓÙ¿Ù·È Û˘¯ÓfiÙÂÚ· ÛÂ
¿ÚÚÂÓ˜ (63%) ·fi fi,ÙÈ ÛÙ· ı‹Ï· (37%). ∏ Û˘¯Ófi-
ÙËÙ· ÙÔ˘ ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ÓÂÊÚÔ‡ ÛÙË ™¢∫ Ù‡Ô˘
Bochdalek ·Ó·Ê¤ÚÂÙ·È ÏÈÁfiÙÂÚÔ ·fi 0,25% (2).
∆· ·Ó·ÙÔÌÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ÂÓ‰ÔıˆÚ·ÎÈ-
ÎÔ‡ ÓÂÊÚÔ‡ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ·ÓˆÌ·Ï›· ÛÙÚÔÊ‹˜ Ù˘
‡Ï˘ ÙÔ˘ ÓÂÊÚÔ‡ Á‡Úˆ ·fi ÙÔÓ ÂÈÌ‹ÎË ¿ÍÔÓ·,
Ì·ÎÚ‡ Ô˘ÚËÙ‹Ú·, ˘„ËÏ‹ ¤ÎÊ˘ÛË ÙˆÓ ÓÂÊÚÈÎÒÓ ·Á-
Á›ˆÓ Î·È ·fiÎÏÈÛË ÙÔ˘ οو fiÏÔ˘ ÙÔ˘ ÓÂÊÚÔ‡
·fi ÙË Ì¤ÛË ÁÚ·ÌÌ‹ (14,15). √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂ-
ÊÚfi˜ ÂÌÊ·Ó›˙ÂÈ Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ·, ‰ÂÓ
·ÚÔ˘ÛÈ¿˙ÂÈ ‰˘ÛÏ·Û›· ‹ ·fiÊÚ·ÍË Ù˘ ÂÎÎÚÈÙÈ΋˜
ÙÔ˘ ÌÔ›Ú·˜ Î·È ‰ÂÓ Û˘Óԉ‡ÂÙ·È ·fi ˘ÂÚÏ·Û›·
ÙÔ˘ ¿ÏÏÔ˘ ÓÂÊÚÔ‡ (16,22). ∏ ·ÈÙ›· Ù˘ ÂÓ‰ÔıˆÚ·ÎÈ-
΋˜ ÂÎÙÔ›·˜ Â›Ó·È ¿ÁÓˆÛÙË. ∏ ıˆڛ· Ù˘ ·ÔÙ˘-
¯Ë̤Ó˘ Û‡ÁÎÏÂÈÛ˘ Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘-
¯‹˜ ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· Ù˘ ™¢∫ Â›Ó·È Â˘Ú¤ˆ˜ ·Ô‰Â-
ÎÙ‹. ∏ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ·Ú¯›˙ÂÈ ·fi ÙÔ
ÚfiÛıÈÔ ÙÌ‹Ì· ÙÔ˘ ˆ˜ ¤Ó· ¯ÒÚÈÛÌ· ÌÂٷ͇ Ù˘ ηÚ-
‰È¿˜ Î·È ÙÔ˘ ‹·ÙÔ˜ Î·È ÚÔ¯ˆÚ¿ÂÈ ÚÔ˜ Ù· ›Ûˆ,
ÁÈ· Ó· ÎÏ›ÛÂÈ ÙÂÏÈο ÚÔ˜ ÙÔ ·ÚÈÛÙÂÚfi ÙÚ‹Ì· ÙÔ˘
Bochdalek, Á‡Úˆ ÛÙËÓ 8Ë Ì 10Ë Â‚‰ÔÌ¿‰· ·ËÛ˘.
∫·Ù¿ Ì›· ¿Ô„Ë, ıˆÚÂ›Ù·È fiÙÈ ÂÓ‰ÔıˆÚ·ÎÈ΋ ÓÂ-
ÊÚÈ΋ ÂÎÙÔ›· ÌÔÚ› Ó· ÚÔÎÏËı› ·fi ÙËÓ Î·ı˘-
ÛÙÂÚË̤ÓË Û‡ÁÎÏÂÈÛË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ‹ ÙËÓ ÂÈ-
Ù¿¯˘ÓÛË Ù˘ ·Ófi‰Ô˘ ÙÔ˘ ÓÂÊÚÔ‡ ÚÈÓ ·fi ÙËÓ Î·ÓÔ-
ÓÈ΋ Û‡ÁÎÏÂÈÛË (3,13). √ ÓÂÊÚfi˜ Û˘Ó‹ıˆ˜ ‚Ú›ÛÎÂÙ·È
ÛÙÔ Ï·ÁÈÔ›ÛıÈÔ ÙÌ‹Ì· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ Î·È Ô
Ô˘ÚËÙ‹Ú·˜ ÂͤگÂÙ·È Ì¤Û· ·fi ÙÔ ÛÙfiÌÈÔ Ù˘ ΋-
Ï˘. ∆Ô ‰È¿ÊÚ·ÁÌ· Û ·˘Ùfi ÙÔ ÛËÌÂ›Ô ·ÔÙÂÏ›ٷÈ
·fi ÌÈ· ÏÂÙ‹ ÌÂÌ‚Ú¿ÓË Ô˘ ÂÚÈ‚¿ÏÏÂÈ ÙÔ ÚÔ¤-
¯ÔÓ ÙÌ‹Ì· ÙÔ˘ ÓÂÊÚÔ‡ Î·È ¤ÙÛÈ Ô ÓÂÊÚfi˜ ‚Ú›ÛÎÂÙ·È
ÂÓÙfi˜ Ù˘ ıˆÚ·ÎÈ΋˜ ÎÔÈÏfiÙËÙ·˜. ¢Â˘ÙÂÚÔ·ıÒ˜
ÌÔÚ› Ó· ˘¿Ú¯ÂÈ Î·È ˘ÔÏ·Û›· ÙÔ˘ ηÙÒÙÂÚÔ˘
ÏÔ‚Ô‡ ÙÔ˘ Û‡ÛÙÔÈ¯Ô˘ Ó‡ÌÔÓ· (13). √È Hawass ND
et al., ÙÔ 1988, ÚfiÙÂÈÓ·Ó fiÙÈ Ë ÙÂÏÈ΋ ı¤ÛË ÙÔ˘ ÓÂ-
ÊÚÔ‡ ηıÔÚ›˙ÂÙ·È ·fi ÙÌ‹Ì· ÙÔ˘ ÁÂÓÓËÙÈÎÔ‡ ÙfiÓÔ˘
(ÙÔ Û˘Á¯ˆÓÂ˘Ì¤ÓÔ Ô˘Ú·›Ô ÙÌ‹Ì· ÙˆÓ ‰‡Ô Ô˘ÚÔÁÂÓ-
ÓËÙÈÎÒÓ ·ÎÚÔÏÔÊÈÒÓ, οı ̛· ·fi ÙȘ Ôԛ˜ ·Ú¤-
¯ÂÈ ¤Ó·Ó ÌÂÛÔÓÂÊÚÈÎfi Î·È ¤Ó·Ó ·Ú·ÌÂÛÔÓÂÊÚÈÎfi
fiÚÔ), ÙÔ ÔÔ›Ô ·ÔÚÚÔÊ¿Ù·È ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘
ÓÂÊÚÔÁ¤ÓÂÛ˘ (17). ™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ ÂÓ‰Ô-
ıˆÚ·ÎÈ΋˜ ÓÂÊÚÈ΋˜ ÂÎÙÔ›·˜, Ô ÓÂÊÚfi˜ ‚Ú›ÛÎÂÙ·È
ÂÓÙfi˜ Ù˘ ıˆÚ·ÎÈ΋˜ ÎÔÈÏfiÙËÙ·˜ Î·È fi¯È ÛÙËÓ ˘Â-
˙ˆÎÔÙÈ΋ ÎÔÈÏfiÙËÙ·. ∆· ÓÂÊÚÈο ·ÁÁ›· Î·È Ô Ô˘ÚË-
Ù‹Ú·˜ Ù˘Èο ÂͤگÔÓÙ·È ·fi ÙËÓ ˘Â˙ˆÎÔÙÈ΋ ÎÔÈ-
ÏfiÙËÙ· ‰È·Ì¤ÛÔ˘ ÙÔ˘ ÛÙÔÌ›Ô˘ Ù˘ ΋Ï˘ Î·È Û˘Ó‹-
ıˆ˜ ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚÔ Ì‹ÎÔ˜ ·fi ÙÔ Ê˘ÛÈÔÏÔÁÈÎfi.
√ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ Ô˘ ‰ÂÓ Û¯ÂÙ›˙ÂÙ·È ÌÂ
™¢∫ ‚Ú›ÛÎÂÙ·È ÛÙËÓ ·Ú·ÛÔÓ‰˘ÏÈ΋ ·‡Ï·Î·. ∆·
ËÌȉȷÊÚ¿ÁÌ·Ù· Â›Ó·È Ê˘ÛÈÔÏÔÁÈο Î·È ‚Ú›ÛÎÔÓÙ·È
ÛÙÔÓ Î·ÙÒÙÂÚÔ fiÏÔ ÙÔ˘ ÓÂÊÚÔ‡.
∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Â›Ó·È ÂÊÈÎÙ‹ ηٿ ÙÔÓ
˘ÂÚ˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ ÂÌ‚Ú‡Ô˘ (18). √ ÚÔÁÂÓ-
ÓËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì ¯Ú‹ÛË Ù˘ Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·-
Ê›·˜ Û ÂÚ›ÙˆÛË ˘Ô„›·˜ ‡·Ú͢ ™¢∫ Î·È Û˘ÓÔ-
‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ¤¯ÂÈ ÚfiÛÊ·Ù· ÚÔÙ·ı› (19) Î·È Û·-
ÊÒ˜ ÏÂÔÓÂÎÙ› ¤Ó·ÓÙÈ ÙÔ˘ ˘ÂÚ˯ÔÁÚ·Ê‹Ì·ÙÔ˜.
∞Ó Î·È Ë ™¢∫ ÂΉËÏÒÓÂÙ·È ÎÏÈÓÈο ·Ì¤Ûˆ˜ ÌÂÙ¿
ÙË Á¤ÓÓËÛË ‹ ÙȘ ÚÒÙ˜ ̤Ú˜ ˙ˆ‹˜, 10-20% ÙˆÓ
ÂÚÈÙÒÛÂˆÓ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ÌÂÙ¿ ÙË ÓÂÔÁÓÈ΋
ÂÚ›Ô‰Ô (20,21). ∏ ÓÂÊÚÈ΋ ÂÎÙÔ›·, fiÙ·Ó ‰ÂÓ Û˘ÓÔ-
‰Â‡ÂÙ·È ·fi ¿ÏϘ ·ÓˆÌ·Ï›Â˜, ‰ÂÓ ÚÔηÏ› Û˘-
ÌÙÒÌ·Ù·. √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ ·ÚÔ˘ÛÈ¿˙ÂÈ
Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁ›· ¯ˆÚ›˜ ÛËÌ›· ‰˘ÛÏ·Û›·˜,
·fiÊڷ͢ Ù˘ ·ÂÎÎÚÈÙÈ΋˜ ÌÔ›Ú·˜ ‹ Û˘ÓÔ‰fi
˘ÂÚÏ·Û›· ÙÔ˘ ¿ÏÏÔ˘ ÓÂÊÚÔ‡ (16,22). øÛÙfiÛÔ,
˘¿Ú¯Ô˘Ó ÂÚÈÔÚÈṲ̂Ó˜ ·Ó·ÊÔÚ¤˜ ÛÙË ‚È‚ÏÈÔÁÚ·-
Ê›· ÂÓ‰ÔıˆÚ·ÎÈÎÒÓ ¤ÎÙÔˆÓ ÓÂÊÚÒÓ Ì ·fiÊÚ·ÍË
Ù˘ ˘ÂÏÔÔ˘ÚËÙËÚÈ΋˜ Û˘Ì‚ÔÏ‹˜ ÙÔ˘ ÓÂÊÚÔ‡, ÌÂ
΢ÛÙÂÔÔ˘ÚËÙËÚÈ΋ ·ÏÈÓ‰ÚfiÌËÛË Î·È Ì ÔÏ˘Î˘-
ÛÙÈ΋ ‰˘ÛÏ·Û›· ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi (1). ™ÙȘ ÂÚÈ-
ÙÒÛÂȘ Û˘Ó‡·Ú͢ ™¢∫, ÏfiÁˆ Ù˘ Ó¢ÌÔÓÈ΋˜
˘ÔÏ·Û›·˜ Ô˘ ÔÊ›ÏÂÙ·È ÛÙËÓ ›ÂÛË ÙˆÓ Ó¢Ìfi-
ÓˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÌ‚Ú˘˚΋˜ ˙ˆ‹˜ ·fi Ù·
ÂÓ‰ÔÎÔÈÏȷο ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó ÂÓÙfi˜ ÙÔ˘
ıÒڷη, ÔÈ ·ÛıÂÓ›˜ ηٿ ÙË Á¤ÓÓËÛË ÂΉËÏÒÓÔ˘Ó
ÔÍ›· ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ·. ™Â ·˘Ù¤˜ ÙȘ ÂÚÈ-
ÙÒÛÂȘ ··ÈÙÂ›Ù·È ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË Ìfi-
ÏȘ ÛÙ·ıÂÚÔÔÈËı› Ô ·ÛıÂÓ‹˜. ™Â ·Û˘Ìو̷ÙÈ-
ÎÔ‡˜ ·ÛıÂÓ›˜, Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË ÌÔ-
Ú› Ó· Á›ÓÂÈ Ì¤Ú˜ ‹ Î·È ¯ÚfiÓÈ· ÌÂÙ¿, ·Ó¿ÏÔÁ· Ì ÙËÓ
ηٿÛÙ·Û‹ ÙÔ˘.
148 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.
Paediatriki 2009;72:29-40
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·148
¢È¿ÊÔÚ˜ ·ÂÈÎÔÓÈÛÙÈΤ˜ ̤ıÔ‰ÔÈ ¯ÚËÛÈÌÔÔÈ-Ô‡ÓÙ·È ÁÈ· ÙË ‰È¿ÁÓˆÛË ‡·Ú͢ ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ÓÂ-ÊÚÔ‡ Î·È ÁÈ· ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ·fi ˆÚÔηٷ-ÎÙËÙÈΤ˜ ÂÂÍÂÚÁ·Û›Â˜ ÙÔ˘ ıÒڷη ‹ ÙÔ˘ ÌÂÛÔıˆ-ڷΛԢ. ∏ ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη Û˘Ó‹ıˆ˜·ÔηχÙÂÈ ·Ú·ÛÔÓ‰˘ÏÈ΋ Ì¿˙· ÛÙÔ Ô›ÛıÈÔÌÂÛÔıˆÚ¿ÎÈÔ.
™ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ë Ó¢-ÚÔ˚ӈ̿وÛË, Ë ÌËÓÈÁÁÔ΋ÏË Î·È ÔÈ fiÁÎÔÈ ÙÔ˘ Ô›-ÛıÈÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘, fiˆ˜ ÙÔ Ó¢ÚÔ‚Ï¿Ûو̷, ÙÔÁ·ÁÁÏÈÔÓ‡ڈ̷ Î·È Ë Ó¢ÚÔÂÓÙÂÚÈ΋ ·ÛÙË (23). ™ÙȘÂÚÈÛÛfiÙÂÚ˜ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÈÙÒÛÂȘ, ¯ÚËÛÈÌÔ-ÔÈ‹ıËÎÂ Ë ÂÓ‰ÔÊϤ‚È· ˘ÂÏÔÁÚ·Ê›· ÁÈ· ÙË ‰È¿ÁÓˆ-ÛË (17,24). ∂›Û˘, ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· Î·È Ë CT ıÒ-ڷη ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó ÛÙË ‰È¿ÁÓˆÛË (18,25). ∏CT ıÒڷη ÌÔÚ› Ó· ÂȂ‚·ÈÒÛÂÈ ‚Ï¿‚˜ Ù˘ ˘¤-ÏÔ˘ Î·È ÙˆÓ Î·Ï‡ÎˆÓ Î·È Ó· ‰È·ÁÓÒÛÂÈ ¿ÏϘ ‚Ï¿‚Â˜Ô˘ ÌÈÌÔ‡ÓÙ·È ÂÓ‰ÔıˆÚ·ÎÈÎfi ¤ÎÙÔÔ ÓÂÊÚfi, fiˆ˜ ÔÈfiÁÎÔÈ ÙÔ˘ ÌÂÛ·˘Ï›Ô˘ Î·È Ë ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË.
£Âڷ¢ÙÈ΋ ·Ú¤Ì‚·ÛË ··ÈÙÂ›Ù·È ÌfiÓÔ Û Â-Ú›ÙˆÛË ·fiÊڷ͢ Ù˘ ·ÂÎÎÚÈÙÈ΋˜ ÌÔ›Ú·˜ ÙÔ˘ÓÂÊÚÔ‡ Î·È Û ÂÌÊ¿ÓÈÛË Î˘ÛÙÂÔÔ˘ÚËÙËÚÈ΋˜ ·ÏÈÓ-‰ÚfiÌËÛ˘. ∂¿Ó ηٿ ÙË ‰È¿ÁÓˆÛË Ô ÓÂÊÚfi˜ ‰ÂÓ Â›Ó·ÈηÙÂÛÙÚ·Ì̤ÓÔ˜, Ë ·fiÊÚ·ÍË Î·È Ë Î˘ÛÙÂÔÔ˘ÚËÙË-ÚÈ΋ ·ÏÈÓ‰ÚfiÌËÛË ÌÔÚ› Ó· ‰ÈÔÚıˆıÔ‡Ó Ì ¤Ì-‚·ÛË. ∂¿Ó fï˜ Ë ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÓÂÊÚÔ‡ Â›Ó·È ÛÔ-‚·Ú¿ ÂËÚ·Ṳ̂ÓË, ÙfiÙÂ Ë ·Ê·›ÚÂÛ‹ ÙÔ˘ ·ÔÙÂÏ›ÙË Ï‡ÛË ÂÈÏÔÁ‹˜ (1). ∂›Û˘, ¯ÂÈÚÔ˘ÚÁÈ΋ ‰ÈÂÚ‡-ÓËÛË ÂÈ‚¿ÏÏÂÙ·È fiÙ·Ó Û˘Ó˘¿Ú¯ÂÈ ™¢∫ Ù‡Ô˘Bochdalek. ™Â ·˘Ù¤˜ ÙȘ ÂÚÈÙÒÛÂȘ Ô ÓÂÊÚfi˜ Û˘-Ó‹ıˆ˜ ηχÙÂÙ·È ·fi ÙÔ ÔÈÛıÔÂÚÈÙfiÓ·ÈÔ Î·ÈÌÔÚ› ‡ÎÔÏ· Ó· ÙÔÔıÂÙËı› ÛÙËÓ ÎÔÈÏȷ΋ ¯ÒÚ·,¯ˆÚ›˜ ÛËÌ·ÓÙÈΤ˜ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÏÔΤ˜, fiˆ˜ÛÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜.
√ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ ·ÔÙÂÏ› Û¿-ÓÈ· ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ· Î·È ı¤ÙÂÈ ÔÏÏ¿ ‰È·ÁÓˆÛÙÈÎ¿Î·È ıÂڷ¢ÙÈο ‰ÈÏ‹ÌÌ·Ù· ÛÙÔ˘˜ ÎÏÈÓÈÎÔ‡˜ ÁÈ·-ÙÚÔ‡˜. ∏ Û˘Ó‡·ÚÍË ™¢∫ Ù‡Ô˘ Bochdalek Î·È ÂÓ‰Ô-ıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡ Â›Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈ·.¶Ú¤ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ Ë Û˘ÁÁÂÓ‹˜ ·˘Ù‹ ·ÓˆÌ·Ï›· ı·Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÂÙ·È ˘fi„Ë ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆ-ÛË Î¿ı ÊÔÚ¿ Ô˘ Ù›ıÂÙ·È ı¤Ì· ‡·Ú͢ ÂÓ‰ÔıˆÚ·-ÎÈ΋˜ Ì¿˙·˜. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È ÛˆÛÙ‹ ·ÓÙÈÌÂ-ÙÒÈÛË ÌÂÈÒÓÂÈ ÙË ÓÔÛËÚfiÙËÙ· Î·È ıÓËÙfiÙËÙ·.
µÈ‚ÏÈÔÁÚ·Ê›·
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149™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek
¶·È‰È·ÙÚÈ΋ 2009;72:29-40
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150 ∫§π¡π∫√ ∫√Àπ∑ CLINICAL QUIZ
Paediatriki 2009;72:66,71-73
∞ÁfiÚÈ 13 ÂÙÒÓ ÚÔÛÎÔÌ›ÛÙËΠÛÙ· Â͈ÙÂÚÈοȷÙÚ›· ·Ú·ÔÓÔ‡ÌÂÓÔ ÁÈ· ‰˘Ûη̄›· Ù˘ Ô‰Ô-ÎÓËÌÈ΋˜, ¿ÏÁÔ˜ Î·È ¯ˆÏfiÙËÙ·, ÌÂÙ¿ ·fi ηٷfi-ÓËÛË ÛÙË ‰È¿ÚÎÂÈ· ·ıÏԷȉȿ˜. ¢ÂÓ ÂÚȤÁÚ·„ÂÛ˘ÁÎÂÎÚÈ̤ÓË Î¿ÎˆÛË, ÏËÓ fï˜ ·Ó¤ÊÂÚ ˆ˜ ÙÔÙÂÏÂ˘Ù·›Ô ÂÍ¿ÌËÓÔ Â›¯Â ηْ ·ӿÏË„Ë ·Ú·ÙË-Ú‹ÛÂÈ Ô›‰ËÌ· ÛÙËÓ ¿ÚıÚˆÛË.
™ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË, ‰È·ÈÛÙÒıËΠ‰˘-Ûη̄›· Ù˘ ·ÚÈÛÙÂÚ‹˜ Ô‰ÔÎÓËÌÈ΋˜ Î·È ÙÔÈ΋¢·ÈÛıËÛ›·, ¯ˆÚ›˜ Ô›‰ËÌ·, ÛÙËÓ ÂÚÈÔ¯‹ ÙˆÓ ÛÊ˘-ÚÒÓ. √ ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‹Ù·Ó ·ÚÓËÙÈÎfi˜. √È ‰Â›-ÎÙ˜ Ïԛ̈͢ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎÔ›. √ Ï‹Ú˘ Ú¢-Ì·ÙÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‹Ù·Ó ›Û˘ ·ÚÓËÙÈÎfi˜. ∏ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË Û ۯÈÛÌÔÂȉ‹ Ï˘¯Ó›·, ÁÈ·ÙË ‰È·›ÛÙˆÛË Èı·Ó‹˜ ÈÚȉÔ΢ÎÏ›Ùȉ·˜, ‰ÂÓ ¤‰ÂÈ-Í ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·.
√È ·ÎÙÈÓÔÁڷʛ˜ ÛÙËÓ ÚÔÛıÈÔ›ÛıÈ· Î·È Ï¿-ÁÈ· ÚÔ‚ÔÏ‹ ¤‰ÂÈÍ·Ó ÔÛÙÈ΋ ·ÏÏÔ›ˆÛË ÛÙÔ Ô›-ÛıÈÔ-¤Ûˆ ÙÌ‹Ì· ÙÔ˘ ıfiÏÔ˘ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘ (EÈÎfi-Ó· 1). ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ¤‰ÂÈÍ ÌÈÎÚ‹ Û˘ÏÏÔÁ‹˘ÁÚÔ‡, ÂÓ‰ÂÈÎÙÈ΋ ‰Ú¿ÚıÚÔ˘. ∆Ô ÛÈÓıËÚÔÁÚ¿ÊË-Ì· ÙˆÓ ÔÛÙÒÓ Ì ڷ‰ÈÂÓÂÚÁfi ∆¯ӋÙÈÔ99 ¤‰ÂÈÍ ·˘-ÍË̤ÓÔ ÌÂÙ·‚ÔÏÈÛÌfi Î·È ÛÙȘ ÙÚÂȘ Ê¿ÛÂȘ. ∏ MRI(EÈÎfiÓ˜ 2 Î·È 3) ¤‰ÂÈÍ ÔÛÙÈ΋ ‚Ï¿‚Ë ÛÙÔ Ô›ÛıÈÔ-¤Ûˆ ÙÌ‹Ì· ÙÔ˘ ıfiÏÔ˘ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘, Ì ÌÈÎÚ‹Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡ Î·È Ô›‰ËÌ· ÙÔ˘ ˘ÔΛÌÂÓÔ˘ Ù˘‚Ï¿‚˘ ÔÛÙÔ‡, ¯ˆÚ›˜ ΢ÛÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ.
∆ÔÔıÂÙ‹ıËΠÁ‡„ÈÓÔ˜ Ó¿ÚıËη˜ ÎÓËÌÔÔ‰È-Îfi˜ Î·È ÌÂÙ¿ ·fi 3 ‚‰ÔÌ¿‰Â˜ ‰ÂÓ ‰È·ÈÛÙÒıËηÓÙ· ·Ú¯Èο Û˘ÌÙÒÌ·Ù· Î·È Ù· ÎÏÈÓÈο ÛËÌ›·. ∆Ô-ÔıÂÙ‹ıËΠÎÓËÌÔÔ‰ÈÎfi˜ ÎˉÂÌfiÓ·˜ ÁÈ· ¿ÏϘ 3‚‰ÔÌ¿‰Â˜, ÂÓÒ Û fiÏÔ ·˘Ùfi ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·ÙÔ ¿ÎÚÔ ‰ÂÓ ÊÔÚÙ›ÛÙËÎÂ. ∆ÚÂȘ Ì‹Ó˜ ÌÂÙ¿ ÙËÓ ·Ú-¯È΋ ÂÎÙ›ÌËÛË, Ô ·ÛıÂÓ‹˜ Â›Ó·È ··ÏÏ·Á̤ÓÔ˜ ·fiÙ· ÂÓԯϋ̷ٿ ÙÔ˘ Î·È ÔÈ ·ÎÙÈÓÔÁڷʛ˜ Â›Ó·È Ê˘-ÛÈÔÏÔÁÈΤ˜.
¶Èı·Ó¤˜ ‰È·ÁÓÒÛÂȘ:
1. MË ÙÚ·˘Ì·ÙÈ΋ ÌÔÓÔ·ÚıÚ›Ùȉ· Ô‰ÔÎÓËÌÈ΋˜ 2. OÛÙÂÔÓ¤ÎÚˆÛË3. ™¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· ·ÛÙÚ·Á¿ÏÔ˘4. √ÛÙÂÔÂȉ¤˜ ÔÛÙ¤ˆÌ· 5. ∞ÔÛ·ÛÙÈÎfi οٷÁÌ·
µ’ √ÚıԷȉÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
¡ÈÎfiÏ·Ô˜ ª·ÚΤ·˜[email protected]µ’ √ÚıԷȉÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
2nd Department ofOrthopaedics, “P. & A. Kyriakou” Children’sHospital, Athens, Greece
Correspondence:
Nikolaos [email protected] Department ofOrthopaedics, “P. & A. Kyriakou” Children’sHospital, Athens, Greece
∞ÁfiÚÈ 13 ÂÙÒÓ Ì ·ӷϷ̂·ÓfiÌÂÓ· ÂÂÈÛfi‰È· ÂÒ‰˘Ó˘
Ô‰ÔÎÓËÌÈ΋˜ ¿ÚıÚˆÛ˘
¡. ª·ÚΤ·˜, π. ¶··¯Ú‹ÛÙÔ˜
Thirteen-year-old boy with recurrent episodes of painful
ankle joint
¡. Markeas, π. Papachristos
∏ ·¿ÓÙËÛË ÛÙË ÛÂÏ›‰· 157
∂ÈÎfiÓ· 1. ∏ ·Ú¯È΋ ·ÎÙÈÓÔÁÚ·Ê›· ·Ó·‰ÂÈÎÓ‡ÂÈ ÔÛÙÈ΋·ÏÏÔ›ˆÛË ÛÙÔ ıfiÏÔ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘.
∂ÈÎfiÓ· 2. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·, ÛÙÔ ÛÙÂÊ·ÓÈ·›Ô ›-‰Ô, ·ÔηχÙÂÈ ÙËÓ ·ÏÏÔ›ˆÛË ÛÙÔ Ô›ÛıÈÔ-¤Ûˆ ÙÌ‹-Ì· ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘, ÙË ÌÈÎÚ‹ Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡ Î·È ÙÔ ÔÛÙÈ-Îfi Ô›‰ËÌ·.
∂ÈÎfiÓ· 3. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·, ÛÙÔ Ô‚ÂÏÈ·›Ô ›Â-‰Ô, ·Ó·‰ÂÈÎÓ‡ÂÈ Ù· ›‰È· Â˘Ú‹Ì·Ù·.
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·150
151™À¡∆√ª∞ ¶∞π¢π∞∆ƒπ∫∞ ¡∂∞ PAEDIATRIC NEWS IN BRIEF
¶·È‰È·ÙÚÈ΋ 2009;72:151-000
∂ËÚ¿˙ÂÈ Ë ıÂڷ›· Ù˘ ÔÍ›·˜ ̤Û˘ˆÙ›Ùȉ·˜ (√ªø) ÙËÓ Â›ÙˆÛË Ù˘̷ÛÙÔÂȉ›Ùȉ·˜ ÛÙ· ·È‰È¿;Paula Thompson et al.Pediatrics 2009;123:424-430
™ÙÔ Ù‡¯Ô˜ ºÂ‚ÚÔ˘·Ú›Ô˘ ÙÔ˘ Pediatrics ‰Ë-ÌÔÛȇıËΠ¤Ó· ÂӉȷʤÚÔÓ ¿ÚıÚÔ Ô˘ ·ÊÔÚ¿ÛÙË ¯Ú‹ÛË ÙˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒ-ÈÛË Ù˘ ÔÍ›·˜ ̤Û˘ ˆÙ›Ùȉ·˜ Î·È Ò˜ ·˘Ù‹ ËıÂڷ›· ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ ÙËÓ Â›ÙˆÛËÙ˘ Ì·ÛÙÔÂȉ›Ùȉ·˜, Ô˘ ·ÔÙÂÏ› Ì›· ·fi ÙȘÛÔ‚·ÚfiÙÂÚ˜ ÂÈÏÔΤ˜ Ù˘ ÓfiÛÔ˘. ∏ Û˘ÁÎÂ-ÎÚÈ̤ÓË ÌÂϤÙË ·ÌÊÈÛ‚ËÙ› Ì›· ÎÏ·ÛÈ΋ ıˆڛ·Ô˘ ηıÈÛÙ¿ ··Ú·›ÙËÙË ÙË ıÂڷ›· Ù˘√ªø. ™ÙËÓ ·Ó·‰ÚÔÌÈ΋ ·˘Ù‹ ÌÂϤÙË, Ë ÔÔ›·¤Ï·‚ ¯ÒÚ· ÛÙË ª. µÚÂÙ·Ó›·, ¯ÚËÛÈÌÔÔÈ‹ıË-Î·Ó ËÏÂÎÙÚÔÓÈÎÔ› Ê¿ÎÂÏÔÈ ·È‰ÈÒÓ ËÏÈΛ·˜ 3ÌËÓÒÓ ¤ˆ˜ 15 ÂÙÒÓ ÌÂٷ͇ ÙˆÓ ÂÙÒÓ 1990-2006,Ù· ÔÔ›· ·Ó¤Ù˘Í·Ó Ì·ÛÙÔÂȉ›Ùȉ·. ∞fi Ù· 854ÂÂÈÛfi‰È· Ì·ÛÙÔÂȉ›Ùȉ·˜, ÌfiÓÔ ÙÔ 1/3 (35,7%)›¯Â ÈÛÙÔÚÈÎfi √ªø. ∏ Èı·ÓfiÙËÙ· ·Ó¿Ù˘Í˘̷ÛÙÔÂȉ›Ùȉ·˜ ÌÂÙ¿ ·fi √ªø ÁÈ· ÙËÓ ÔÔ›·¯ÔÚËÁ‹ıËΠıÂڷ›· ‹Ù·Ó 1,8/100.000 ÂÂÈÛfi-‰È· √ªø, ÂÓÒ Ë Â›ÙˆÛË Û¯Â‰fiÓ ‰ÈÏ·ÛÈ¿˙Â-Ù·È (3,8/100.000) fiÙ·Ó ‰ÂÓ Â›¯Â ‰Ôı› ıÂڷ›·.∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ ÁÈ· Ó· ÚÔÏËÊı› 1 ÂÚ›Ùˆ-ÛË Ì·ÛÙÔÂȉ›Ùȉ·˜ Ú¤ÂÈ Ó· ıÂڷ‡ÛÔ˘ÌÂ4.831 ÂÚÈÙÒÛÂȘ √ªø. ™Â ·fiÏ˘ÙÔ˘˜ ·ÚÈı-ÌÔ‡˜, ·˘Ùfi ÌÂÙ·ÊÚ¿˙ÂÙ·È Û 255 ηÈÓÔ‡ÚÁÈ· Â-ÚÈÛÙ·ÙÈο Ì·ÛÙÔÂȉ›Ùȉ·˜ ÙÔ ¯ÚfiÓÔ ÁÈ· ÙË ª.µÚÂÙ·Ó›· Â¿Ó ÛÙ·Ì·ÙÔ‡ÛÂ Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛËÁÈ· ÙËÓ √ªø, ÂÓÒ Ë ¯Ú‹ÛË ÙˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ı·ÌÂȈÓfiÙ·Ó Î·Ù¿ 738.775 Û˘ÓÙ·Á¤˜.
™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ÂÈÛËÌ·›-ÓÔ˘Ó fiÙÈ Ù· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ Ô˘ ·Ó¤Ù˘Í·ÓÌ·ÛÙÔÂȉ›Ùȉ· ‰ÂÓ Â›¯·Ó ÂÈÛÎÂÊı› ÙÔÓ ÁÈ·ÙÚfiÙÔ˘˜. ∫·Ù¿ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜, Ë ·‡ÍËÛË Ù˘ ›-ÙˆÛ˘ Ù˘ Ì·ÛÙÔÂȉ›Ùȉ·˜ ÏfiÁˆ Ù˘ ÌË ¯Ú‹-Û˘ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ÁÈ· ÙË ıÂڷ›· √ªø ›ӷÈÌÈÎÚ‹ ÂÙËÛ›ˆ˜ Î·È ÙÔ fiÊÂÏÔ˜ ·fi ÙË Ì›ˆÛË Ù˘¯Ú‹Û˘ ·ÓÙÈ‚ÈÔÙÈÎÒÓ Ô˘ÛÈÒÓ ıˆÚÂ›Ù·È fiÙÈ Â›Ó·ÈÛËÌ·ÓÙÈÎfiÙÂÚÔ ·fi ÙÔ ÎfiÛÙÔ˜ Ù˘ ÂÈÏÔ΋˜.
£Âڷ›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·˜: ÊÂÏ› ËÌ·ÎÚÔ¯ÚfiÓÈ· ·ÚÂÓÙÂÚÈ΋ ıÂڷ›·;Theoklis Zaoutis et al. Pediatrics 2009:2;636-642
√ Zaoutis et al. ‰ËÌÔÛ›Â˘Û·Ó ÛÙÔ PediatricsÙ· ·ÔÙÂϤÛÌ·Ù· ÌÈ·˜ ÌÂÁ¿Ï˘ ·Ó·‰ÚÔÌÈ΋˜ÌÂϤÙ˘ Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËΠÛÙȘ ∏¶∞ ηȷÊÔÚÔ‡Û ÛÙË Û‡ÁÎÚÈÛË Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ÂÓ-‰ÔÊϤ‚È·˜ ıÂڷ›·˜ ÁÈ· ÔÍ›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·Ì ·˘Ù‹ Ù˘ Ù·¯Â›·˜ ÌÂÙ¿‚·Û˘ ·fi ÂÓ‰ÔÊϤ-‚È· ıÂڷ›· Û ıÂڷ›· ·fi ÙÔ ÛÙfiÌ·.
™Â ·˘Ù‹ ÙËÓ ÔÏ˘ÎÂÓÙÚÈ΋ ÌÂϤÙË (29 ÓÔÛÔ-ÎÔÌ›· ÙˆÓ ∏¶∞) Û˘ÌÂÚÈÂÏ‹ÊıËÛ·Ó ·È‰È¿ 2ÌËÓÒÓ ¤ˆ˜ 17 ¯ÚÔÓÒÓ Ì ÔÍ›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·Î·Ù¿ Ù· ¤ÙË 2000-2005. ™˘ÓÔÏÈο 1.969 ·È‰È¿ÏËÚÔ‡Û·Ó Ù· ÎÚÈÙ‹ÚÈ·, ÂÎ ÙˆÓ ÔÔ›ˆÓ 1.021¤Ï·‚·Ó Ì·ÎÚ¿˜ ‰È¿ÚÎÂÈ·˜ ÂÓ‰ÔÊϤ‚È· ıÂڷ›·(>2 ‚‰ÔÌ¿‰Â˜), ÂÓÒ 948 ¤Ï·‚·Ó ‚Ú·¯Â›· ÂÓ‰Ô-ÊϤ‚È· ıÂڷ›· (<2 ‚‰ÔÌ¿‰Â˜) Î·È ÛÙË Û˘Ó¤-¯ÂÈ· ıÂڷ›· ·fi ÙÔ ÛÙfiÌ·. ∂ÈÙ˘¯‹˜ ‹Ù·Ó ËıÂڷ›· ÛÙÔ 95% ÙˆÓ ·È‰ÈÒÓ Ô˘ ¤Ï·‚·Ó Ì·-ÎÚÔ¯ÚfiÓÈ· ÂӉԂϤ‚È· ıÂڷ›·, ¤Ó·ÓÙÈ ÙÔ˘ 96%ÙˆÓ ·È‰ÈÒÓ Ô˘ ¤Ï·‚·Ó ‚Ú·¯Â›· ÂÓ‰ÔÊϤ‚È·
¡¤· ·fi ÙÔÓ ÒÚÔ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÏÔÈÌÒ͈Ó
¡. ™˘Ú›‰Ë˜
News on paediatric infections
N. Spyridis
µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ
∞ÏÏËÏÔÁÚ·Ê›·:
¡›ÎÔ˜ ¶. ™˘Ú›‰Ë˜[email protected]µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ
2nd Department ofPaediatrics, University ofAthens, Athens, Greece
Correspondence:
Nikos P. [email protected] Department ofPaediatrics, University ofAthens, Athens, Greece
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·151
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152 ™À¡∆√ª∞ ¶∞π¢π∞∆ƒπ∫∞ ¡∂∞
Paediatriki 2009;72:152-000
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¤Ï·‚·Ó ·ÚÂÓÙÂÚÈ΋ ıÂڷ›· ›¯·Ó Û ÔÛÔ-
ÛÙfi 3,4% ÂÈÏÔΤ˜ Ô˘ ÔÊ›ÏÔÓÙ·Ó Î˘Ú›ˆ˜
ÛÙË ¯Ú‹ÛË ÙÔ˘ ÂÓ‰ÔÊϤ‚ÈÔ˘ ηıÂÙ‹Ú·.
™˘ÓÔ„›˙ÔÓÙ·˜, ÔÈ Û˘ÁÁÚ·Ê›˜ ˘ÔÛÙËÚ›˙Ô˘Ó
fiÙÈ ÁÈ· ÙË ıÂڷ›· Ù˘ ÔÍ›·˜ ÔÛÙÂÔÌ˘ÂÏ›ÙÈ-
‰·˜, Ë Ù·¯Â›· ÌÂÙ¿‚·ÛË ·fi ÙËÓ ÂÓ‰ÔÊϤ‚È·
ÛÙËÓ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ·ÁˆÁ‹ Û¯ÂÙ›˙ÂÙ·È ÌÂ
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§¿ıË Î·Ù¿ ÙË Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË ÛÙËÓ ·È‰È΋
ËÏÈΛ·
Ian Wong et al.
Archives of Diseases in Childhood 2009;94;161-164
√ Ian Wong et al. ‰ËÌÔÛ›Â˘Û·Ó ÛÙÔ Archives
of Diseases in Childhood ÌÈ· ÛÂÈÚ¿ ·fi„ÂˆÓ Ô˘
·ÊÔÚÔ‡Ó ÛÙȘ ÌÂıfi‰Ô˘˜ ·ÓÙÈÌÂÙÒÈÛ˘ Ï·ıÒÓ
ÛÙË Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË Ê·ÚÌ¿ÎˆÓ ÛÙËÓ ·È‰È΋
ËÏÈΛ·. ∆Ô Û˘ÓËı¤ÛÙÂÚÔ È·ÙÚÈÎfi ÛÊ¿ÏÌ· ·ÊÔÚ¿
ÛÙË Ï·Óı·Ṳ̂ÓË Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË. ∆· Ï¿ıË ·˘-
Ù¿ ·Ó¤Ú¯ÔÓÙ·È ÁÈ· ÙÔ ∏ӈ̤ÓÔ µ·Û›ÏÂÈÔ ÛÂ
500.000 ÂÚÈÙÒÛÂȘ ÂÙËÛ›ˆ˜. ∆· ΢ÚÈfiÙÂÚ· ·›-
ÙÈ· ›ӷÈ: 1) Ë ÔÏ˘ÏÔÎfiÙËÙ· ÛÙÔÓ ÔÏÔÁÈÛÌfi
Ù˘ ‰fiÛ˘, Ë ÔÔ›· ÚÔÛ‰ÈÔÚ›˙ÂÙ·È Ì ‚¿ÛË
ÔÏÏÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ËÏÈΛ·, ÙÔ ‚¿ÚÔ˜,
Ë ÂÈÊ¿ÓÂÈ· ÛÒÌ·ÙÔ˜, Ë ÚÔˆÚfiÙËÙ· Î·È Ë ÎÏÈ-
ÓÈ΋ ÂÈÎfiÓ·, 2) ·ÚÈıÌËÙÈο Ï¿ıË, ΢ڛˆ˜ ÛÙËÓ
ÙÔÔı¤ÙËÛË ‰Âη‰ÈÎÔ‡ „ËÊ›Ô˘, ÌˉÂÓÈÎÒÓ Î·È
ÌÔÓ¿‰ˆÓ ̤ÙÚËÛ˘, 3) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË ·fi
Ó¤Ô˘˜ ¿ÂÈÚÔ˘˜ ÁÈ·ÙÚÔ‡˜, ÔÈ ÔÔ›ÔÈ Û˘ÓÙ·ÁÔ-
ÁÚ·ÊÔ‡Ó Ì ‚¿ÛË ÙËÓ ÂÌÂÈÚ›· ÙÔ˘˜ ·fi ÙÔÓ
ÂÓ‹ÏÈÎÔ ÏËı˘ÛÌfi Î·È 4) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË
Ê·ÚÌ¿ÎˆÓ Ù· ÔÔ›· ‰ÂÓ ¤¯Ô˘Ó ¿‰ÂÈ· ¯Ú‹Û˘
ÛÙÔÓ ·È‰È·ÙÚÈÎfi ÏËı˘ÛÌfi Î·È ˆ˜ ÂÎ ÙÔ‡ÙÔ˘
‰ÂÓ ˘¿Ú¯Ô˘Ó ·È‰È·ÙÚÈΤ˜ ‰fiÛÂȘ.
°È· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·˘ÙÔ‡ ÙÔ˘ Ê·ÈÓÔ̤-
ÓÔ˘ Û˘ÓÈÛٿٷÈ: 1) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË Ó· Á›ÓÂ-
Ù·È Ì¤Ûˆ ËÏÂÎÙÚÔÓÈÎÒÓ ˘ÔÏÔÁÈÛÙÒÓ Ô˘ Ó·
ÂÚȤ¯Ô˘Ó ÂÓÙÔϤ˜ ·ÛÊ·Ï›·˜, 2) Ó· Á›ÓÂÙ·È
¤ÏÂÁ¯Ô˜ ÙˆÓ ‰fiÛÂˆÓ ·fi ÙÔ˘˜ Ê·ÚÌ·ÎÔÔÈÔ‡˜,
3) Ë Âη›‰Â˘ÛË ÙˆÓ È·ÙÚÒÓ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ
Î·È ÂÈÌÔÚʈÙÈο ÛÂÌÈÓ¿ÚÈ· Û˘ÓÙ·ÁÔÁÚ¿ÊË-
Û˘, 4) Ó· ÂÚÈÁÚ¿ÊÂÙ·È Ë Ì¤ıÔ‰Ô˜ ˘ÔÏÔÁÈ-
ÛÌÔ‡ Ù˘ ‰fiÛ˘ ÛÙË Û˘ÓÙ·Á‹, 5) fiÛÔÓ ·ÊÔÚ¿ ÙË
¯ÔÚ‹ÁËÛË ÂÓ‰ÔÊÏ‚›ˆÓ Ê·Ú̿ΈÓ, Ë ¯Ú‹ÛË
ËÏÂÎÙÚÔÓÈÎÒÓ Û˘Û΢ÒÓ ÌÔÚ› Ó· ·Ó·ÁÓˆÚ›-
ÛÂÈ Èı·Ófi ÛÊ¿ÏÌ· ÛÙË ‰ÔÛÔÏÔÁ›· ÙÔ˘ Ê·ÚÌ¿-
ÎÔ˘, 6) Ó· ÂÓÈÛ¯˘ı› Ë ·Ó·ÊÔÚ¿ Ï·ıÒÓ ÛÙË Û˘-
ÓÙ·ÁÔÁÚ¿ÊËÛË, ÁÂÁÔÓfi˜ Ô˘ ÌÔÚ› Ó· Ô‰ËÁ‹-
ÛÂÈ ÛÙË Ï‹„Ë ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ Î·È 7) Ó·
‚ÂÏÙȈı› Ë ÂÈÎÔÈÓˆÓ›· ÌÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ
ÂÌÏÂÎfiÌÂÓˆÓ Â·ÁÁÂÏÌ·ÙÈÒÓ ˘Á›·˜.
™˘Û¯¤ÙÈÛË ÙÔ˘ ÔÚfiÙ˘Ô˘ ÙÔ˘
Ó¢ÌÔÓÈfiÎÔÎÎÔ˘ Ì ÙË ÛÔ‚·ÚfiÙËÙ· Ù˘
Ïԛ̈͢ Î·È ÙË ı·Ó·ÙËÊfiÚÔ ¤Î‚·ÛË
Simon Ruckinger et al.
Pediatric Infectious Diseases Journal 2009;28:118-122
™ÙÔ ÙÂÏÂ˘Ù·›Ô Ù‡¯Ô˜ ÙÔ˘ Pediatric Infectious
Diseases Journal ‰ËÌÔÛȇÂÙ·È ·Ó·‰ÚÔÌÈ΋ ÌÂ-
ϤÙË Ô˘ ¤Ï·‚ ¯ÒÚ· ÛÙË °ÂÚÌ·Ó›· ÌÂٷ͇ ÙˆÓ
ÂÙÒÓ 1997-2003 Î·È ·ÊÔÚÔ‡Û ÛÙËÓ Ù·˘ÙÔÔ›Ë-
ÛË ÙÔ˘ Ó¢ÌÔÓÈfiÎÔÎÎÔ˘ Û ·È‰È¿ Ô˘ ¤ı·-
Ó·Ó ÏfiÁˆ Ù˘ Ïԛ̈͢ ‹ ·Ó¤Ù˘Í·Ó ‚·ÚÈ¿ ÏÔ›-
̈ÍË (ÛË„·ÈÌ›·, ÌËÓÈÁÁ›Ùȉ·) ‹ ÛÔ‚·Ú¤˜ ÌfiÓÈ-
̘ ‚Ï¿‚˜. ∞fi ÙȘ 559 ÂÚÈÙÒÛÂȘ fiÔ˘ Ô
ÔÚfiÙ˘Ô˜ Ù·˘ÙÔÔÈ‹ıËÎÂ, 494 ‹Ù·Ó ‰È·ı¤ÛÈ̘
ÁÈ· ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË. ∏ Û˘ÓÔÏÈ΋ ıÓËÙfiÙËÙ·
‹Ù·Ó 5,3%, ÂÓÒ Ë ‚·ÚÈ¿ Ïԛ̈ÍË ·ÓÂÚ¯fiÙ·Ó ÛÂ
17%. √ ÔÚfiÙ˘Ô˜ 7F Û˘Û¯ÂÙÈ˙fiÙ·Ó Ì ıÓËÙfiÙË-
Ù· Û ÔÛÔÛÙfi 14,8%, ·ÎÔÏÔ˘ıÔ‡ÌÂÓÔ˜ ·fi ÙÔÓ
23F (8,3%) Î·È ÙÔÓ ÔÚfiÙ˘Ô 3 Û ÔÛÔÛÙfi 3%.
µ·ÚÈ¿ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ·Ó¤Ù˘Í·Ó 17% ÙˆÓ ·È-
‰ÈÒÓ, ÂÎ ÙˆÓ ÔÔ›ˆÓ ÔÛÔÛÙfi 40,7% ÔÊÂÈÏfiÙ·Ó
ÛÙÔÓ ÔÚfiÙ˘Ô 7F.
™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ÛËÌÂÈÒ-
ÓÔ˘Ó fiÙÈ Ë ÌËÓÈÁÁ›Ùȉ· ·fi ÙÔÓ ÔÚfiÙ˘Ô 7F Û˘Ó-
‰¤ÂÙ·È Ì ·˘ÍË̤ÓË ıÓËÙfiÙËÙ· ‹ ÂÈÏÔΤ˜,
ÂÓÒ ÙÚÈÏ·ÛÈ¿˙ÂÙ·È Ë Èı·ÓfiÙËÙ· ·Ó¿Ù˘Í˘
‚·ÚÈ¿˜ ‰ÈÂÈÛ‰˘ÙÈ΋˜ Ïԛ̈͢.
∏ ·Í›· ·˘Ù‹˜ Ù˘ ÌÂϤÙ˘ ‚Ú›ÛÎÂÙ·È ÛÙÔ ÁÂ-
ÁÔÓfi˜ fiÙÈ Ô ÔÚfiÙ˘Ô˜ 7F ‰ÂÓ Û˘ÌÂÚÈÏ·Ì‚¿ÓÂ-
Ù·È ÛÙÔ ˘¿Ú¯ÔÓ Û˘˙¢Á̤ÓÔ Ó¢ÌÔÓÈÔÎÔÎÎÈ-
Îfi ÂÌ‚fiÏÈÔ, ÁÈ’ ·˘Ùfi Î·È Ë ·ÚÔ˘Û›· ÙÔ˘ ‹‰Ë
·ÚÔ˘ÛÈ¿˙ÂÈ ·‡ÍËÛË Û ¯ÒÚ˜ fiˆ˜ Ë ∞ÁÁÏ›·,
°·ÏÏ›·, °ÂÚÌ·Ó›· Î·È ∞Ï¿Ûη. ∆· ‰‡Ô Ó¤· Ó¢-
ÌÔÓÈÔÎÔÎÎÈο ÂÌ‚fiÏÈ· (10-‰‡Ó·ÌÔ Î·È 13-‰‡Ó·-
ÌÔ) Ô˘ ·Ó·Ì¤ÓÂÙ·È Ó· ΢ÎÏÔÊÔÚ‹ÛÔ˘Ó ÛÙÔ
ÚÔÛ¯¤˜ ̤ÏÏÔÓ ı· Û˘ÌÂÚÈÏ·Ì‚¿ÓÔ˘Ó Î·È ÙÔÓ
Û˘ÁÎÂÎÚÈ̤ÓÔ ÔÚfiÙ˘Ô.
¡fiÛÔ˜ Kawasaki ÛÙË ª. µÚÂÙ·Ó›·: ÂıÓÈÎfiÙËÙ·,
Û˘Óı‹Î˜ ‰È·‚›ˆÛ˘ Î·È ·Ó·Ó¢ÛÙÈÎÔ› ÈÔ›
Antony Hardnen et al.
Paediatric Infectious Diseases Journal 2009;28:21-24
™ÙËÓ ·Ó·‰ÚÔÌÈ΋ ·˘Ù‹ ÌÂϤÙË Ô˘ ¤Ï·‚ Ò-
Ú· ÛÙË ª. µÚÂÙ·Ó›·, ·Ó·˙ËÙ‹ıËÎÂ Ë Èı·Ó‹ Û˘-
Û¯¤ÙÈÛË Ù˘ ÓfiÛÔ˘ Kawasaki Ì ÙËÓ ÂıÓÈÎfiÙËÙ·
ÙÔ˘ ·ÛıÂÓÔ‡˜, ÙȘ Û˘Óı‹Î˜ ‰È·‚›ˆÛ˘ Î·È ÙËÓ
ÂȉËÌÈÔÏÔÁ›· ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÈÒÓ. ÃÚËÛÈ-
ÌÔÔÈ‹ıËÎ·Ó ËÏÂÎÙÚÔÓÈο ·Ú¯Â›· (Hospital
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·152
153PAEDIATRIC NEWS IN BRIEF
¶·È‰È·ÙÚÈ΋ 2009;72:153-000
Episode Statistics) Ù· ÔÔ›· ÂÚÈÁÚ¿ÊÔ˘Ó ÙÔ ·›ÙÈÔÂÈÛ·ÁˆÁ‹˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô, ‰ËÌÔÁÚ·ÊÈο ÛÙÔÈ-¯Â›· ÙÔ˘ ·ÛıÂÓÔ‡˜, ·ÓÙÈÌÂÙÒÈÛË Î·È ·Ú·ÎÏÈÓÈ-Τ˜ ÂÍÂÙ¿ÛÂȘ. ªÂٷ͇ 1998 Î·È 2003 ·ÓÙÈÌÂÙˆ-›ÛÙËÎ·Ó 2.432 ·È‰È¿ Ì ÓfiÛÔ Kawasaki, ÂÎ ÙˆÓÔÔ›ˆÓ 728 ÂÚÈÙÒÛÂȘ ·ÔÙÂÏÔ‡Û·Ó Â·ÓÂÈ-Û·ÁˆÁ‹. ∞fi ÙȘ 1.704 ηÈÓÔ‡ÚÁȘ ÂÚÈÙÒÛÂȘ,ÔÈ 1.228 (72%) ‹Ù·Ó ·È‰È¿ <5 ÂÙÒÓ Î·È ÔÈ 254·È‰È¿ οو ÙÔ˘ ¤ÙÔ˘˜. √ ·ÚÈıÌfi˜ ÙˆÓ Î·ÈÓÔ‡-ÚÈˆÓ ÂÚÈÙÒÛÂˆÓ Û ¤Ó· Ì‹Ó· ÁÈ· Ù· ·È‰È¿ ο-Ùˆ ÙˆÓ 5 ÂÙÒÓ Î˘Ì¿ÓıËΠ·fi 8 (™Â٤̂ÚÈÔ˜2001) ¤ˆ˜ 41 (π·ÓÔ˘¿ÚÈÔ˜ 1999). ∏ ÂÙ‹ÛÈ· ›-ÙˆÛË Ù˘ ÓfiÛÔ˘ ‹Ù·Ó 8,39/100.000 ·È‰È¿. ∏ ÎÈ-ÓÂ˙È΋ ÂıÓÈÎfiÙËÙ· (P=0,005), Ë ‰È·‚›ˆÛË Û ÌÂ-Á¿Ï˜ fiÏÂȘ (P=0,001) Î·È Ë ÔÈÎÔÓÔÌÈ΋ ·Ó¤¯ÂÈ·(P=0,016) ·ÔÙÂÏÔ‡Û·Ó ÈÛ¯˘ÚÔ‡˜ ÚԉȷıÂÛÈ-ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Ù˘ ÓfiÛÔ˘. ∏
˘„ËÏfiÙÂÚË Â›ÙˆÛË ·Ú·ÙËÚ‹ıËΠÙÔ˘˜ ¯ÂÈ-ÌÂÚÈÓÔ‡˜ Ì‹Ó˜, ¯ˆÚ›˜ fï˜ οÔÈÔ˜ ·fi ÙÔ˘˜Û˘Ó‹ıÂȘ ÈÔ‡˜ (RSV, adenovirus, influenza) Ó·ÂÌÊ·Ó›˙ÂÙ·È Û˘¯ÓfiÙÂÚ·.
™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ·Ó·Ê¤ÚÔ˘ÓfiÙÈ, fiˆ˜ ›¯Â ·Ú·ÙËÚËı› Î·È ÛÙËÓ π·ˆÓ›·,Ë ÌÂٷΛÓËÛË ÛÙȘ ÌÂÁ¿Ï˜ fiÏÂȘ Û˘Ó‰˘¿ÛÙË-Π̠¤Í·ÚÛË Ù˘ ÓfiÛÔ˘. √È Î·Î¤˜ Û˘Óı‹Î˜‰È·‚›ˆÛ˘ ·ÔÙÂÏÔ‡Ó ·ÚÓËÙÈÎfi ÚÔÁÓˆÛÙÈÎfi·Ú¿ÁÔÓÙ·, ÂÓÒ Ù· ÓÂԉȷÁÓˆÛı¤ÓÙ· ·È‰È¿ÂÌÊ·Ó›˙ÔÓÙ·È Î˘Ú›ˆ˜ ÙÔ˘˜ ¯ÂÈÌÂÚÈÓÔ‡˜ Ì‹Ó˜.¶·ÚfiÙÈ ‰È¿ÊÔÚÔÈ ÈÔÁÂÓ›˜ ·Ú¿ÁÔÓÙ˜ ¤¯Ô˘ÓÂÓÔ¯ÔÔÈËı›, ÔÈ ÂÚ¢ÓËÙ¤˜ ‰ÂÓ Î·Ù¿ÊÂÚ·Ó Ó·‚ÚÔ˘Ó Î¿ÔÈ· Û˘Û¯¤ÙÈÛË ÌÂ Û˘ÁÎÂÎÚÈ̤ÓÔ˘˜ÈÔ‡˜, ÂÓÈÛ¯‡ÔÓÙ·˜ ÙË ıˆڛ· fiÙÈ Ë ÁÂÓÂÙÈ΋ÚԉȿıÂÛË ·ÔÙÂÏ› ÙÔÓ ÈÛ¯˘ÚfiÙÂÚÔ Úԉȷ-ıÂÛÈÎfi ·Ú¿ÁÔÓÙ·.
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·153
154 ∂§§∏¡π∫∞ ∂ƒ∂À¡∞ ™∆∏ ¢π∂£¡∏ µπµ§π√°ƒ∞ºπ∞
Paediatriki 2009;72:154-000
∂ÏÏËÓÈ΋ ·È‰È·ÙÚÈ΋ ¤Ú¢ӷ Û ‰ÈÂıÓ‹ ÂÚÈÔ‰Èο, 2008
∂. °·Ï·Ó¿Î˘
Hellenic paediatric research in international journals, 2008
E. Galanakis
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘
AÏÏËÏÔÁÚ·Ê›·:
∂ÌÌ·ÓÔ˘‹Ï °·Ï·Ó¿Î˘[email protected]¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘
Department of Paediatrics,University of Crete,Heraklion, Greece
Correspondence:
Emmanouil [email protected] of Paediatrics,University of Crete,Heraklion, Greece
¶·ÚÔ˘ÛÈ¿˙ÔÓÙ·È, Ôχ Û˘ÓÔÙÈο, Ï›Á˜·fi ÙȘ ÔÏϤ˜ ÂÏÏËÓÈΤ˜ ÌÂϤÙ˜ ·È‰È·ÙÚÈ-ÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜ Ô˘ ‰ËÌÔÛȇıËÎ·Ó ÙÔ2008 Û ‰ÈÂıÓ‹ ÂÚÈÔ‰Èο Î·È Î·Ï‡ÙÔ˘Ó fiÏ·Ù· ‰›·, ·fi ÙÔ ÂÚÁ·ÛÙËÚÈ·Îfi ˆ˜ ÙÔ ÎÏÈÓÈÎfiÎ·È ÙÔ ÎÔÈÓˆÓÈÎfi, Î·È fiϘ ÙȘ ËÏÈ˘, ·fi ÙÔÓÂÔÁÓfi Î·È ÙÔ ‚Ú¤ÊÔ˜ ˆ˜ ÙÔ ·È‰› Î·È ÙÔÓ ¤ÊË-‚Ô. ªÂϤÙ˜ Ô˘ ›¯·Ó ‰ËÌÔÛÈ¢ı› ÙÔ˘˜ ÔχÚÒÙÔ˘˜ Ì‹Ó˜ ÙÔ˘ 2008 ¤¯Ô˘Ó ·ÚÔ˘ÛÈ·Ûı›ÛÙËÓ ¶∞π¢π∞∆ƒπ∫∏ 2008;71(3):248-250.
¡ÂÊÚÈ΋ ·Ó¿Ù˘ÍË ÛÙ· ÌÈÎÚ¿ ÓÂÔÁÓ¿.
¶ÚÔÔÙÈ΋ ÌÂϤÙË ·fi Ù· πˆ¿ÓÓÈÓ· (Drougiaet al., Nephrol Dial Transplant, Epub 2008 Aug4) ·Ú·ÎÔÏÔ‡ıËÛ ˘ÂÚ˯ÔÁÚ·ÊÈο ÙËÓ ·Ó¿-Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ Û 243 ÌÈÎÚ¿ ÁÈ· ÙËÓ ËÏÈ-Λ· ·ËÛ˘ ÓÂÔÁÓ¿ (SGA) Î·È 223 ÓÂÔÁÓ¿ ÌÂηٿÏÏËÏÔ ‚¿ÚÔ˜ ÁÈ· ÙËÓ ËÏÈΛ· ·ËÛ˘(AGA). ∏ ·Ú·ÎÔÏÔ‡ıËÛË ¤ÁÈÓ 5 ÊÔÚ¤˜ ÛÙ·2 ÚÒÙ· ¤ÙË ˙ˆ‹˜ (Û‡ÓÔÏÔ 1898 ˘ÂÚ˯ÔÁÚ·-ÊËÌ¿ÙˆÓ). ™Ù· ÙÂÏÂÈfiÌËÓ· SGA ÓÂÔÁÓ¿, Ë ÓÂ-ÊÚÈ΋ ·Ó¿Ù˘ÍË ‹Ù·Ó fiÌÔÈ· Ì ÂΛÓË ÙˆÓAGA, ˆÛÙfiÛÔ ÛÙ· ÌÈÎÚ¿ ÚfiˆÚ· ÓÂÔÁÓ¿, ÙÔ̤ÁÂıÔ˜ ÙˆÓ ÓÂÊÚÒÓ ·Ú¤ÌÂÓ ÌÈÎÚfiÙÂÚÔ·fi ÂΛÓÔ ÙˆÓ AGA ÓÂÔÁÓÒÓ ÁÈ· Ù· ‰‡Ô ÚÒ-Ù· ¤ÙË Ù˘ ˙ˆ‹˜.
°ÂÓÂÙÈ΋ ÚԉȿıÂÛË ÛÙË Ì¤ÛË ˆÙ›Ùȉ·.
ªÂϤÙË ·fi ÙÔ ∏Ú¿ÎÏÂÈÔ (Ilia S et al., PediatrInfect Dis J 2008;27:929-33) ·Ó·ÛÎÔ› Ù· ¤ˆ˜ÙÒÚ· ÁÓˆÛÙ¿ ÁÈ· ÙËÓ ·¿ÓÙËÛË ÙÔ˘ ÍÂÓÈÛÙ‹ ηÈÙË ÁÂÓÂÙÈ΋ ¢¿ıÂÈ· ÛÙËÓ ˆÙ›Ùȉ·. ∫·Ù·ÁÚ¿-ÊÂÙ·È ÌÈ· ÌÂÁ¿ÏË ÔÈÎÈÏ›· ·Ú·ÁfiÓÙˆÓ Ù˘ ÂÁ-ÁÂÓÔ‡˜ Î·È ÚÔÛ·ÚÌfiÛÈÌ˘ ·ÓÔÛ›·˜ Ô˘ ÚÔ-‰È·ı¤ÙÔ˘Ó Î¿ÔÈ· ‚Ú¤ÊË Î·È ·È‰È¿ Û ÔÏϤ˜‹ Û ÛÔ‚·Ú¤˜ ˆÙ›Ùȉ˜.
º˘Ì·Ù›ˆÛË ‚Ú¤ÊÔ˘˜ ÌÂÙ¿ ÂÚÈÙÔÌ‹. ¶Â-ÚÈÁÚ·Ê‹ ÚˆÙÔ·ıÔ‡˜ Ê˘Ì·Ù›ˆÛ˘ ¤Ô˘˜ ÌÂÛ˘ÓÔ‰fi ‚Ô˘‚ˆÓÈ΋ ÏÂÌÊ·‰ÂÓ›Ùȉ· Û ˘ÁȤ˜ η-Ù¿ Ù· ¿ÏÏ· ·ÁfiÚÈ, ËÏÈΛ·˜ 10 ÌËÓÒÓ, Ô˘ ›¯ÂÂÚÈÙÌËı› ÛÙÔ ¶·ÎÈÛÙ¿Ó ÚÔ 4Ì‹ÓÔ˘ (¡ÔÛÔ-
ÎÔÌÂ›Ô ¶·›‰ˆÓ ∞ıËÓÒÓ «∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡»,Papaevangelou V et al., J Infect Epub 2008 Dec18). ∏ ÂȂ‚·›ˆÛË ¤ÁÈÓ Ì ÔÍÂÔ¿ÓÙÔ¯Ë ¯ÚÒ-ÛË, ηÏÏȤÚÁÂÈ· Î·È PCR.
µÚԢΤÏψÛË Û ÌÈÎÚfi ‚Ú¤ÊÔ˜. ¶ÂÚÈÁÚ·-Ê‹ ÎÚÔ‡ÛÌ·ÙÔ˜ ‚ÚԢΤÏÏ·˜ Û ‚Ú¤ÊÔ˜ 2,5 ÌË-ÓÒÓ ·fi ÙËÓ ◊ÂÈÚÔ (Makis AC et al., EmergInfect Dis 2008;141:319-20). ∆Ô ‚Ú¤ÊÔ˜ ‰ÂÓ Â›¯Â·ʋ Ì ˙Ò·, ‰ÂÓ ı‹Ï·˙Â Î·È Ë ÌËÙ¤Ú· ÙÔ˘‰ÂÓ Â›¯Â ‚ÚԢΤÏψÛË, ¿Ú· ·Ú·Ì¤ÓÂÈ ·ÓÔÈÎÙfiÙÔ ı¤Ì· Ù˘ ÌÂÙ¿‰ÔÛ˘ ·fi ÌÔÏ˘Ṳ̂ӷ ·ÓÙÈ-ΛÌÂÓ·, οÙÈ ·Û˘Ó‹ıÈÛÙÔ ÁÈ· ÙË ÓfiÛÔ.
∞ÓıÂÎÙÈÎÔ› ÛÙ·Ê˘ÏfiÎÔÎÎÔÈ ÛÙËÓ ∞ı‹Ó·.
ªÂϤÙË 198 ÛÙÂϯÒÓ ÛÙ·Ê˘ÏÔÎfiÎÎˆÓ ·fi·È‰È¿ Ì ÏÔÈÌÒÍÂȘ Ù˘ ÎÔÈÓfiÙËÙ·˜ (¡ÔÛÔÎÔ-ÌÂ›Ô ¶·›‰ˆÓ ∞ıËÓÒÓ «∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡»,Niniou T et al., Eur J Clin Microbiol Infect Dis2008;27:831-7): 44% MRSA ·ÓÙÔ¯‹ (ÛÙË ÌÂıÈ-ÎÈÏÏ›ÓË).
∞ÓıÂÎÙÈÎÔ› ÛÙ·Ê˘ÏfiÎÔÎÎÔÈ ÛÙËÓ ¶¿ÙÚ·.
∞Ó¿ÏÔÁË ÌÂϤÙË ·fi ÙÔ ∫·Ú·Ì·Ó‰¿ÓÂÈÔ ¡ÔÛÔ-ÎÔÌÂ›Ô ¶·›‰ˆÓ ¶·ÙÚÒÓ (170 ·ÛıÂÓ›˜ Ì ÏÔ›-̈ÍË ·fi S. aureus Î·È ˘ÁÈ‹ ·È‰È¿ Ì ÚÈÓÈ΋ÊÔÚ›· S. aureus, Sdougkos G et al., ClinMicrobiol Infect 2008;14:995-1001): 62% MRSA·ÓÙÔ¯‹ ÁÈ· Ù· ÛÙÂϤ¯Ë ÙˆÓ ·ÛıÂÓÒÓ Î·È 5,5%MRSA ·ÓÙÔ¯‹ ÁÈ· Ù· ÚÈÓÈο ÛÙÂϤ¯Ë ÙˆÓ ˘ÁÈÒӷȉÈÒÓ.
¶Ó¢ÌÔÓÈfiÎÔÎÎÔ˜: ·ÓÙÔ¯‹. ªÂϤÙË 612ÛÙÂϯÒÓ Streptococcus pneumoniae, ‰ÈÂÈÛ‰˘ÙÈ-ÎÒÓ Î·È ÌË, ÚÈÓ ÙËÓ Â˘Ú›· ÂÊ·ÚÌÔÁ‹ ÙÔ˘Ó¢ÌÔÓÈÔÎÔÎÎÈÎÔ‡ ÂÌ‚ÔÏ›Ô˘ (¡ÔÛÔÎÔÌ›Զ·›‰ˆÓ ∞ıËÓÒÓ «∞Á›· ™ÔÊ›·», Daikos GL et al.,Diagn Microbiol Infect Dis 2008;60:393-8) ¤‰ÂÈÍ·‡ÍËÛË Ù˘ ·ÓÙÔ¯‹˜ ÛÙ· Ì·ÎÚÔÏ›‰È· (·fi 7,4%ÙÔ 1985-1996 Û 53,7% ÙÔ 2001-2004), ·Ú¿Ï-ÏËÏË Ì ÙËÓ ·‡ÍËÛË Ù˘ ηٷӿψÛ˘ Ì·ÎÚÔ-Ïȉ›ˆÓ Î·È ÏÈÓÎÔ˙·ÌȉÒÓ ÛÙËÓ ÎÔÈÓfiÙËÙ· (·fi
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·154
155HELLENIC RESEARCH IN THE INTERNATIONAL LITERATURE
¶·È‰È·ÙÚÈ΋ 2009;72:155-000
4,31 DI ‰fiÛÂȘ ÙÔ 1990-1996 Û 6,97 DI ‰fiÛÂȘ ÙÔ2001-2004).
¶Ó¢ÌÔÓÈfiÎÔÎÎÔ˜: ÌÂÙ¿ ÙÔ ÂÌ‚fiÏÈÔ. ªÂϤÙË·fi ÙË §¿ÚÈÛ· Û 1829 Ó‹È· (Grivea IN et al.,Pediatr Infect Dis J 2008;27:519-25) ¤‰ÂÈÍ Ì›ˆÛËÙ˘ ÚÈÓÔÊ·Ú˘ÁÁÈ΋˜ ÊÔÚ›·˜ ÙˆÓ ÔÚÔÙ‡ˆÓ ÙÔ˘ ÂÌ-‚ÔÏ›Ô˘ ÌÂÙ¿ ÙÔ ÂÙ·‰‡Ó·ÌÔ Û˘˙¢Á̤ÓÔ ÂÌ‚fiÏÈÔ,¯ˆÚ›˜ ·‡ÍËÛË Ù˘ ÊÔÚ›·˜ ÙÔ˘ ÔÚfiÙ˘Ô˘ 19∞. ™Ù·ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿ ·Ú·ÙËÚ‹ıËΠ̛ˆÛË ÙË˜Û˘¯ÓfiÙËÙ·˜ ÛÙÂϯÒÓ ˘„ËÏ‹˜ ·ÓÙÔ¯‹˜ ÛÙËÓ ÂÓÈ-ÎÈÏÏ›ÓË.
£Âڷ›· Ì˘ÎËÙÈ·ÛÈÎÒÓ ÏÔÈÌÒ͈Ó. ªÂϤÙË·fi ÙÔ πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢(Filioti I et al., Expert Opin Pharmacother2008;9:3179-96) ·Ó·ÛÎÔ› ÙȘ ıÂڷ¢ÙÈΤ˜ ÛÙÚ·-ÙËÁÈΤ˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ‰ÈÂÈÛ‰˘ÙÈÎÒÓ ÏÔÈ-ÌÒÍÂˆÓ ·fi ∫¿ÓÙÈÓÙ· Î·È ∞Û¤ÚÁÈÏÏÔ Û ÓÂÔÁÓ¿ ηȷȉȿ, Ì ¤ÌÊ·ÛË ÛÙ· ÓÂfiÙÂÚ· ·Ú¿ÁˆÁ·.
§Â˘¯·È̛˜ Î·È ÏÂÌÊÒÌ·Ù· ÛÙËÓ ∂ÏÏ¿‰·. ¶·-ÓÂÏÏ‹ÓÈ· ÌÂϤÙË 11ÂÙ›·˜ (1996-2006) η٤ÁÚ·„Â(Petridou ET et al., Arch Dis Child 2008;93:1027-32)863 ÂÚÈÙÒÛÂȘ Ï¢¯·ÈÌ›·˜ Î·È 311 ÏÂÌÊÒÌ·ÙÔ˜ Û·ȉȿ οو ÙˆÓ 15 ÂÙÒÓ. ∏ ›وÛË Ù˘ Ï¢¯·È-Ì›·˜ (46,6 ÂÚÈÙÒÛÂȘ ·Ó¿ 1.000.000 ·È‰È¿ ·Ó¿
¤ÙÔ˜) ‹Ù·Ó ·Ó¿ÌÂÛ· ÛÙȘ ˘„ËÏfiÙÂÚ˜ Ù˘ ∂˘Úˆ·˚-΋˜ ŒÓˆÛ˘ (19% ˘„ËÏfiÙÂÚË ·fi ÙË Ì¤ÛË Â˘Úˆ-·˚΋). ∏ ›وÛË ÏÂÌÊÒÌ·ÙÔ˜ (16,8 ÂÚÈÙÒÛÂȘ·Ó¿ 1.000.000 ·È‰È¿ ·Ó¿ ¤ÙÔ˜) ‰ÂÓ ‰È¤ÊÂÚ ·fi ÙË̤ÛË Â˘Úˆ·˚΋.
§ÈıÔÙÚÈ„›· ÛÙ· ·È‰È¿. ¶·ÚÔ˘Û›·ÛË Ù˘10ÂÙÔ‡˜ ÂÌÂÈÚ›·˜ ÂÓfi˜ ΤÓÙÚÔ˘ ÛÙËÓ ·ÓÙÈÌÂÙÒÈ-ÛË Ù˘ Ô˘ÚÔÏÈı›·Û˘ Û 125 ·È‰È¿ (πÔÎÚ¿ÙÂÈÔ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢, Charalambous S et al., JEndourol 2008;22:2169-74): Û 93 ·È‰È¿ ¤ÁÈÓ ÏÈıÔ-ÙÚÈ„›· Ì ˆÛÙÈο ·̷ٷ ˘ÂÚ‹¯ˆÓ (Û‡ÓÔÏÔ 108Û˘Ó‰ÚÈÒÓ), Û 12 Ô˘ÚËÙËÚÔÛÎfiËÛË Î·È ‰ÈÔ˘ÚË-ÙËÚÈ΋ ·Ê·›ÚÂÛË, Û 9 ‰È·‰ÂÚÌ·ÙÈ΋ ÓÂÊÚÔÏÈıÔÙÔ-Ì›· Î·È Û 11 ¯ÚÂÈ¿ÛÙËΠ·ÓÔÈÎÙfi ¯ÂÈÚÔ˘ÚÁ›Ô. ™‡Ì-ʈӷ Ì ÙË ÌÂϤÙË, Ë ÏÈıÔÙÚÈ„›· Î·È ÔÈ ÂÓ‰ÔÔ˘ÚË-ÙËÚÈΤ˜ Ù¯ÓÈΤ˜ Â›Ó·È ·ÛÊ·Ï›˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈ-Τ˜ ÛÙ· ·È‰È¿.
∆Ô ‡„Ô˜ ÙˆÓ Ó¤ˆÓ ∂ÏÏ‹ÓˆÓ. ªÂϤÙË (∞ÙÙÈÎfi¡ÔÛÔÎÔÌ›Ô, Papadimitriou ∞ et al., Acta Paediatr2008;97:1105-7) Û 3.982 ÓÂÔÛ‡ÏÏÂÎÙÔ˘˜ ËÏÈΛ·˜ 18-26 ÂÙÒÓ (2006-2007) ¤‰ÂÈÍ ̤ÛÔ ‡„Ô˜ ÛÒÌ·ÙÔ˜ Ù·178,1 cm. ∞ÓÙ›ÛÙÔÈ¯Ë ÌÂϤÙË ÙÔ˘ 1990 ›¯Â ηٷÁÚ¿-„ÂÈ Ì¤ÛÔ ‡„Ô˜ 175,7. º·›ÓÂÙ·È, Û˘ÓÂÒ˜, fiÙÈ Ô ·Ó-‰ÚÈÎfi˜ ÏËı˘ÛÌfi˜ Ù˘ ∂ÏÏ¿‰·˜ ‰ÂÓ ¤¯ÂÈ ·ÎfiÌË ÂÍ·-ÓÙÏ‹ÛÂÈ Ù· ÂÚÈıÒÚÈ· ·‡ÍËÛ˘ Û ‡„Ô˜.
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·155
πÛÙÔÛÂÏ›‰· Ù˘ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘
∞ıËÓÒÓ – http://www.2pediatric.gr/
™. ∫¯·ÁÈ¿˜, ¢. ∞Ó·ÁÓÒÛÙÔ˘
Site of the 2nd Department of Paediatrics of the
University of Athens – http://www.2pediatric.gr/
S. Kehagias, D. Anagnostou
√ ‰È·‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ Ù˘ µã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, Ô˘ ‰ڿ˙ÂÙ·È ÛÙÔ ¡ÔÛÔÎÔÌ›Զ·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ÏÂÈÙÔ˘ÚÁ› Î·È ·Ó·ÌÔÚÊÒÓÂÙ·ÈÛ˘Ó¯Ҙ Â‰Ò Î·È ‰‡Ô ¯ÚfiÓÈ·. ™Ùfi¯Ô˜ ÙÔ˘ Â›Ó·È Ó· ʤÚÂÈ Û·ʋ Ì ÙÔÓ ÒÚÔ Ù˘ ∫ÏÈÓÈ΋˜ fiÏÔ˘˜ fiÛÔ˘˜ ı· ıÂÏ‹ÛÔ˘Ó Ó·¯ÚËÛÈÌÔÔÈ‹ÛÔ˘Ó ÙȘ È·ÙÚÈΤ˜, ‰È‰·ÎÙÈΤ˜ Î·È ÂÚ¢ÓËÙÈΤ˜Ù˘ ‰Ú·ÛÙËÚÈfiÙËÙ˜. ™ÙȘ ÛÂÏ›‰Â˜ ÙÔ˘ ˘¿Ú¯Ô˘Ó ÏËÚÔÊÔ-ڛ˜ Ô˘ ı· ‰È¢ÎÔχÓÔ˘Ó ÙËÓ ÚfiÛ‚·ÛË ÛÙȘ ÂÍÂȉÈÎÂ˘Ì¤Ó˜˘ËÚÂۛ˜ Ô˘ ·Ú¤¯Ô˘Ó Ù· ÙÌ‹Ì·Ù· Ù˘ ∫ÏÈÓÈ΋˜ Î·È Ù· ÂÈ-‰Èο Â͈ÙÂÚÈο È·ÙÚ›·.
™ÙËÓ ·Ú¯È΋ ÛÂÏ›‰·, ÂÎÙfi˜ ·fi ÙËÓ Ù·¯˘‰ÚÔÌÈ΋ ‰È‡ı˘Ó-ÛË Ù˘ ÎÏÈÓÈ΋˜ Î·È Ù· ÙËϤʈӷ ÙˆÓ ÓÔÛËÏ¢ÙÈÎÒÓ ÌÔÓ¿‰ˆÓ,˘¿Ú¯Ô˘Ó ·Ó·ÚÙË̤Ó˜ Î·È ÔÈ ·Ó·ÎÔÈÓÒÛÂȘ ÙˆÓ Û˘Ó‰ڛˆÓ,ÙˆÓ ÛÂÌÈÓ·Ú›ˆÓ, ·ÏÏ¿ Î·È ÙˆÓ ÌÂÙÂÎ·È‰Â˘ÙÈÎÒÓ ÊÚÔÓÙÈÛÙË-
Ú›ˆÓ Ô˘ ÔÚÁ·ÓÒÓÂÈ Ë ∫ÏÈÓÈ΋ ÛÙÔ ·ÌÊÈı¤·ÙÚÔ «∫. ¶··‰¿ÙÔ˜» ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ Ì·˜. ™ÙËÓ ·Ú¯È΋ ÛÂÏ›‰· ›Û˘, ÛÙÔ˘˜ «ÃÚ‹ÛÈÌÔ˘˜ ™˘Ó‰¤ÛÌÔ˘˜», ¿Ú¯Ô˘Ó ÈÛÙfiÙÔÔÈ Ô˘ ÚÔÛʤÚÔ˘Ó
¢ηÈڛ˜ ÂÓË̤ڈÛ˘ ÁÈ· ÙË Û˘Ó¯Ҙ ÂÍÂÏÈÛÛfiÌÂÓË ÁÓÒÛË Û¯ÂÙÈο Ì ÙÔ ·È‰› Î·È ÙËÓ ˘Á›· ÙÔ˘, ·Ï-Ï¿ Î·È ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘, fiˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ ÈÛÙfiÙÔÔ ÙÔ˘ ∫¤ÓÙÚÔ˘ Ù˘ π·ÙÚÈ΋˜ Ô˘ µ·Û›˙ÂÙ·È Ûª·ÚÙ˘Ú›Â˜ (Center for Evidence Based Medicine). ™ÙËÓ ›‰È· ÛÂÏ›‰·, ·ÎÔÏÔ˘ı› Ô ¯ÒÚÔ˜ fiÔ˘ ÌÔÚ› Ó·Á›ÓÂÈ Ë ËÏÂÎÙÚÔÓÈ΋ ÂÁÁÚ·Ê‹ ÛÙÔ newsletter Ù˘ ∫ÏÈÓÈ΋˜.
∏ ÛÂÏ›‰· «¶ÚÔÛˆÈÎfi» ·Ú¤¯ÂÈ ÛÙÔȯ›· ÁÈ· ÙÔ ·ÓıÚÒÈ-ÓÔ ‰˘Ó·ÌÈÎfi Î·È ÙË ÛÙÂϤ¯ˆÛË Ù˘ ∫ÏÈÓÈ΋˜. ∏ ÛÂÏ›‰· «∆Ì‹-Ì·Ù·» ‰Â›¯ÓÂÈ ÙȘ ÎÏÈÓÈΤ˜ ‰˘Ó·ÙfiÙËÙ˜ Ù˘ ∫ÏÈÓÈ΋˜. ∞ӷʤ-ÚÔÓÙ·È, ÂÎÙfi˜ ÙˆÓ ‚·ÛÈÎÒÓ ÓÔÛËÏ¢ÙÈÎÒÓ ÌÔÓ¿‰ˆÓ, Ù· ÙÌ‹-Ì·Ù· Î·È Ù· ÂȉÈο Â͈ÙÂÚÈο È·ÙÚ›· ÁÈ· ÙȘ ˘ÔÂȉÈÎfiÙËÙ˜Ù˘ ·È‰È·ÙÚÈ΋˜. ™ÙËÓ ›‰È· ÛÂÏ›‰· ›Û˘, ·Ú¤¯ÔÓÙ·È ÏË-ÚÔÊÔڛ˜ ÁÈ· Ù· ˆÚ¿ÚÈ· ÏÂÈÙÔ˘ÚÁ›·˜, ·ÏÏ¿ Î·È ÙȘ ‰Ú·ÛÙËÚÈfi-ÙËÙ˜ ÙˆÓ ÙÌËÌ¿ÙˆÓ Î·È ÙˆÓ Â͈ÙÂÚÈÎÒÓ È·ÙÚ›ˆÓ.
∞ÎÔÏÔ˘ı› Ë ÛÂÏ›‰· «∂η›‰Â˘ÛË», Ô˘ ÂÚÈÁÚ¿ÊÂÈ ÙÔ ÂÎ-·È‰Â˘ÙÈÎfi ¤ÚÁÔ Ù˘ µã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙË-Ì›Ô˘ ∞ıËÓÒÓ. ™ÙËÓ ›‰È· ÛÂÏ›‰· ·ÎÔÏÔ˘ıÔ‡Ó ·ÓÙ›ÛÙÔȯÔÈ ÈÛÙfiÙÔ-ÔÈ, Ì ÏËÚÔÊÔڛ˜ Û¯ÂÙÈΤ˜ Ì ÙȘ ÂοÛÙÔÙ ‰Ú·ÛÙËÚÈfiÙËÙ˜.
∆Ô ÂÚ¢ÓËÙÈÎfi ¤ÚÁÔ Ù˘ ∫ÏÈÓÈ΋˜ ÂÚÈÁÚ¿ÊÂÙ·È ÛÙË ÛÂÏ›‰· «ŒÚ¢ӷ». ∏ ÂÚ¢ÓËÙÈ΋ ‰Ú·ÛÙËÚÈfi-ÙËÙ· Ô˘ ‚Ú›ÛÎÂÙ·È Û ÂͤÏÈÍË ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÎfiÓËÛË ‰È‰·ÎÙÔÚÈÎÒÓ ‰È·ÙÚÈ‚ÒÓ, ÔÏ˘ÎÂÓÙÚÈÎÒÓÌÂÏÂÙÒÓ, ÂȉËÌÈÔÏÔÁÈÎÒÓ ÌÂÏÂÙÒÓ, ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÌÂÏÂÙÒÓ, ÌÂÏÂÙÒÓ ÎÏÈÓÈ΋˜ ·Ú·Ù‹ÚË-Û˘, ·ÏÏ¿ Î·È ‰È·¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ ηٷÁÚ·Ê‹˜ ÓÔÛËÌ¿ÙˆÓ.
∆¤ÏÔ˜, ÛÙÔ Â‰›Ô «∞Ó·˙‹ÙËÛË», Ô ÈÛÙfiÙÔÔ˜ ÚÔÛʤÚÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ıÂÌ·ÙÈ΋˜ ·Ó·˙‹ÙËÛË˜ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘, Ì ¯Ú‹ÛË Ï¤ÍˆÓ-ÎÏÂȉÈÒÓ.
156 ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√ NEWS FROM THE INTERNET
Paediatriki 2009;72:156
¡ÂÔÁÓÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
™Ù¤Ê·ÓÔ˜ ∫¯·ÁÈ¿˜[email protected]¡ÂÔÁÓÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·
Neonatology Unit, 2nd Department of Paediatrics,University of Athens, “P. & A. Kyriakou” Children’sHospital, Athens, Greece
Correspondence:
Stefanos [email protected] Unit, 2nd Department of Paediatrics,University of Athens, “P. & A. Kyriakou” Children’sHospital, Athens, Greece
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·156
157∫§π¡π∫√ ∫√Àπ∑ CLINICAL QUIZ
¶·È‰È·ÙÚÈ΋ 2009;72:66,71-73
√È ÚÒÙ˜ ÂÚÈÁڷʤ˜ Ù˘ Û¯·ÏȉˆÙÈ΋˜ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ·˜ ÛÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›·Î¿ÓÔ˘Ó ÙËÓ ÂÌÊ¿ÓÈÛ‹ ÙÔ˘˜ ‹‰Ë ·fi Ù· Ù¤ÏËÙÔ˘ 19Ô˘ ·ÈÒÓ·, ·ÏÏ¿ ÌfiÏȘ ÙÔ 1959 ÔÈ BerndtÎ·È Harty (1) ÚÔ‚·›ÓÔ˘Ó ÛÙËÓ ÚÒÙË Ù·ÍÈÓfi-ÌËÛË. ∏ ÓfiÛÔ˜ ÚÔÛ‚¿ÏÏÂÈ Û˘Ó‹ıˆ˜ ÙÔ˘˜ ÌË-ÚÈ·›Ô˘˜ ÎÔÓ‰‡ÏÔ˘˜ Î·È Û·ÓÈfiÙÂÚ· ÙÔÓ ÎfiÓ‰˘-ÏÔ ÙÔ˘ ‚Ú·¯ÈfiÓÈÔ˘ ÔÛÙÔ‡. ∏ ÂÓÙfiÈÛ‹ Ù˘ÛÙÔÓ ıfiÏÔ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘ ıˆÚÂ›Ù·È È‰È·›ÙÂ-Ú· Û¿ÓÈ· (4% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ÔÛÙÂÔ¯fiÓ‰ÚÈ-ÓˆÓ ‚Ï·‚ÒÓ ÙÔ˘ ÛÎÂÏÂÙÔ‡). ∏ ÈÛ¯·ÈÌÈ΋ Ó¤-ÎÚˆÛË ÙÔ˘ ˘ÔΛÌÂÓÔ˘ ˘Ô¯fiÓ‰ÚÈÔ˘ ÔÛÙԇʷ›ÓÂÙ·È ˆ˜ ·ÈÙÈÔÏÔÁ› ¿ÚÈÛÙ· ÙËÓ ·ÔÎfiÏ-ÏËÛË ÙÔ˘ ÔÛÙÈÎÔ‡ ÙÂÌ·¯›Ô˘, Ì·˙› Ì ÙÔÓ ˘ÂÚ-ΛÌÂÓÔ ·ÚıÚÈÎfi ¯fiÓ‰ÚÔ.
πÛÙÔÚÈÎfi οΈÛ˘ ·Ó·Ê¤ÚÂÙ·È Û ¤Ó· Ô-ÛÔÛÙfi 85% ÙˆÓ ·ÛıÂÓÒÓ Ì ÔÛÙÂÔ¯fiÓ‰ÚÈÓË‚Ï¿‚Ë ÛÙÔÓ ·ÛÙÚ¿Á·ÏÔ. ∂ȉÈο, ÔÈ ‚Ï¿‚˜ ÛÙÔÔ›ÛıÈÔ-¤Ûˆ ÙÌ‹Ì· ÙÔ˘ ıfiÏÔ˘ ÙÔ˘ ·ÛÙÚ·Á¿-ÏÔ˘ Â›Ó·È ÙÔ ·ÔÙ¤ÏÂÛÌ· ·ӷϷ̂·ÓfiÌÂ-ÓÔ˘ ‚›·ÈÔ˘ ˘ÙÈ·ÛÌÔ‡ ÙÔ˘ ¿ÎÚÔ˘ Ô‰fi˜, Ì·˙›Ì ÂÏÌ·ÙÈ·›· Î¿Ì„Ë Î·È Â͈ÙÂÚÈ΋ ÛÙÚÔÊ‹ÙÔ˘ ÔÛÙÔ‡ ̤۷ ÛÙËÓ Ô‰ÔÎÓËÌÈ΋. ∏ ‚Ï¿‚ËÌÔÚ› Ó· ÚÔÎÏËı› ¯ˆÚ›˜ ··Ú·›ÙËÙ· Û˘-Ó˘¿Ú¯Ô˘Û· Ú‹ÍË ÙÔ˘ ¤Íˆ Ï·Á›Ô˘ Û˘Ó‰¤-ÛÌÔ˘, ÁÂÁÔÓfi˜ Ô˘ ‰ÈηÈÔÏÔÁ› ÙÔ ‡‰Ú·ÚıÚÔÙ˘ Ô‰ÔÎÓËÌÈ΋˜ (fiˆ˜ ÛÙËÓ ÂÚÈÁÚ·Ê›۷ÂÚ›ÙˆÛË), ‰›¯ˆ˜ ÙÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÙÔ˘‰È·ÛÙÚ¤ÌÌ·ÙÔ˜ Ô›‰ËÌ· ÛÙËÓ ÂÚÈÔ¯‹ ÙˆÓÛÊ˘ÚÒÓ.
™ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÔÓٷȤӷ Ï‹ıÔ˜ ·ı‹ÛˆÓ, Ì ÂÓÙfiÈÛË ÙËÓ Ô‰Ô-ÎÓËÌÈ΋ ‹ ÙÔÓ ›‰ÈÔ ÙÔÓ ·ÛÙÚ¿Á·ÏÔ. ∏ ÌË ÙÚ·˘-Ì·ÙÈ΋ ÌÔÓÔ·ÚıÚ›Ùȉ· Ù˘ Ô‰ÔÎÓËÌÈ΋˜ Û˘-Óԉ‡ÂÙ·È Û˘Ó‹ıˆ˜ ·fi ¤ÓÙÔÓ· ÂÓÔ¯Ï‹Ì·Ù·Î·È ÎÏÈÓÈο ÛËÌ›·, ÂÓÒ ÔÈ ‰Â›ÎÙ˜ ÊÏÂÁÌÔÓ‹˜Â›Ó·È ηٿ ηÓfiÓ· ·˘ÍË̤ÓÔÈ. ∏ ÔÛÙÂÔÓ¤ÎÚˆ-ÛË ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ·Ô˘Û›· ·ÁÁ›ˆ-Û˘ ÙÔ˘ ˘ÔΛÌÂÓÔ˘ Ù˘ ‚Ï¿‚˘ ÔÛÙÔ‡, ÂÓÒÛÙË Û¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· Ë ·ÁÁ›ˆÛËÂ›Ó·È Ê˘ÛÈÔÏÔÁÈ΋. ∂Í ¿ÏÏÔ˘, Ë ÔÛÙÂÔÓ¤ÎÚˆÛËÚÔÛ‚¿ÏÏÂÈ ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ˘. ∆Ô ÔÛÙÂÔÂÈ-‰¤˜ ÔÛÙ¤ˆÌ· ¤¯ÂÈ ÂÓÙÔÓfiÙÂÚ· Û˘ÌÙÒÌ·Ù· ηȯ·Ú·ÎÙËÚÈÛÙÈÎfi fiÓÔ, Ô˘ ÂΉËÏÒÓÂÙ·È Û˘Ó‹-ıˆ˜ ÙȘ Ó˘ÎÙÂÚÈÓ¤˜ ÒÚ˜ Î·È ˘Ê›ÂÙ·È Ì ÙË Ï‹-„Ë ÌË ÛÙÂÚÔÂȉÒÓ ·ÓÙÈÊÏÂÁÌÔÓˆ‰ÒÓ Ê·ÚÌ¿-ΈÓ. ∆Ô ·ÔÛ·ÛÙÈÎfi οٷÁÌ· Â›Ó·È ·Û˘Ì‚›-‚·ÛÙÔ Ì ÙËÓ ·Ó·ÙÔÌÈ΋ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘,
ÂÂȉ‹ Ô ıfiÏÔ˜ ÙÔ˘ ‰ÂÓ ‰¤¯ÂÙ·È ÚÔÛʇÛÂȘÈÛ¯˘ÚÒÓ Û˘Ó‰¤ÛÌˆÓ ‹ ÙÂÓfiÓÙˆÓ.
∏ ¿Ù˘Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· Î·È ÙÔ ·Û·Ê¤˜ ÈÛÙÔ-ÚÈÎfi ‰ÂÓ ÈηÓÔÔÈÔ‡Ó Û˘Ó‹ıˆ˜ ÙË ‰È¿ÁÓˆÛË Î·È··ÈÙÂ›Ù·È Û˘¯Ó¿ Ô ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜. √È·ÎÙÈÓÔÁڷʛ˜, ÔÈ Ôԛ˜ › ÛÂÈÚ¿ ÂÙÒÓ ‹Ù·Ó ËÚÒÙË ÚÔÛ¿ıÂÈ· ‰È·ÁÓˆÛÙÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜, ÛÙ· ·Ú¯Èο ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘‰ÂÓ ˆÊÂÏÔ‡Ó, ÁÈ·Ù› ·‰˘Ó·ÙÔ‡Ó Ó· ·ÂÈÎÔÓ›ÛÔ˘ÓÙËÓ ÔÛÙÈ΋ ‚Ï¿‚Ë. ∞˘Ùfi˜ ‹Ù·Ó Ô ÏfiÁÔ˜ Ù˘ η-ı˘ÛÙÂÚË̤Ó˘ ‰È¿ÁÓˆÛ˘ ÛÙË ‰È΋ Ì·˜ ÂÚ›-ÙˆÛË. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ¤¯ÂÈ ÙËÓ Èη-ÓfiÙËÙ· Ù˘ ¤ÁηÈÚ˘ ·ÂÈÎfiÓÈÛ˘ Ù˘ ‚Ï¿‚˘,Ù˘ ·ÎÚÈ‚Ô‡˜ ÂÓÙfiÈÛ˘ Î·È Ù˘ ¤ÎÙ·Û‹˜ Ù˘.∂›Û˘ ÌÔÚ› Ó· ·ÂÈÎÔÓ›ÛÂÈ Ì ·ÎÚ›‚ÂÈ· ÙËÓ‡·ÚÍË ÔÛÙÈÎÔ‡ Ôȉ‹Ì·ÙÔ˜. ™‡Ìʈӷ Ì ÙËÓÈÛ¯‡Ô˘Û· Ù·ÍÈÓfiÌËÛË (2-4) (Ô˘ ÛÙËÚ›˙ÂÙ·È ÛÙ·Â˘Ú‹Ì·Ù· Ù˘ Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜) ηȷӿÏÔÁ· Ì ÙÔ ·ÂÈÎÔÓÈ˙fiÌÂÓÔ ÛÙ¿‰ÈÔ, ·ÔÊ·-Û›˙ÂÙ·È Ë ÂÊ·ÚÌÔÛÙ¤· ıÂڷ›·.
∆Ô ÛÙ¿‰ÈÔ 1 ÂÚÈÏ·Ì‚¿ÓÂÈ ‚Ï¿‚Ë ÙÔ˘ ·Ú-ıÚÈÎÔ‡ ¯fiÓ‰ÚÔ˘ ÌfiÓÔ, Ô˘ ‰ÂÓ Á›ÓÂÙ·È ÔÚ·Ù‹ÛÙȘ ·ÎÙÈÓÔÁڷʛ˜ Î·È ‰ÂÓ ··ÈÙ› ¿ÏÏË ıÂ-ڷ›· ·fi ÙËÓ Ù·ÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË.
∆Ô ÛÙ¿‰ÈÔ 2 ÂÚÈÏ·Ì‚¿ÓÂÈ, ÂÎÙfi˜ ·fi ÙË‚Ï¿‚Ë ÙÔ˘ ·ÚıÚÈÎÔ‡ ¯fiÓ‰ÚÔ˘, οٷÁÌ· ÙÔ˘˘ÔΛÌÂÓÔ˘ ÔÛÙÔ‡, Ì ÔÛÙÈÎfi Ô›‰ËÌ· (ÛÙ¿‰ÈÔ2·, fiˆ˜ ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È)‹ ¯ˆÚ›˜ Ô›‰ËÌ· (2‚). ™Ù· ÛÙ¿‰È· ·˘Ù¿, Ë ÚÔ-ÙÂÈÓfiÌÂÓË ıÂڷ›· Â›Ó·È Û˘ÓÙËÚËÙÈ΋, Ì·ÎÈÓËÙÔÔ›ËÛË Û ӿÚıËη Î·È ·ÔÊ˘Á‹ Ï‹-ÚÔ˘˜ ÊfiÚÙÈÛ˘ ÙÔ˘ ÛΤÏÔ˘˜ ÁÈ· 6 ÙÔ˘Ï¿¯ÈÛÙÔÓ‚‰ÔÌ¿‰Â˜.
™ÙÔ ÛÙ¿‰ÈÔ 3, ·Ó·Ê·›ÓÂÙ·È ‰È·¯ˆÚÈÛÌfi˜,·ÏÏ¿ fi¯È ·ÚÂÎÙfiÈÛË, ÔÛÙÂÔ¯fiÓ‰ÚÈÓÔ˘ ÙÂÌ·-¯›Ô˘. ™ÙÔ ÛÙ¿‰ÈÔ 4, ÙÔ ‰È·¯ˆÚÈṲ̂ÓÔ ÙÂÌ¿¯ÈÔ¤¯ÂÈ ·ÔÎÔÏÏËı› Î·È Û˘ÌÂÚÈʤÚÂÙ·È ˆ˜ÂχıÂÚÔ ÛÒÌ· ÛÙÔ ÂÛˆÙÂÚÈÎfi Ù˘ ¿ÚıÚˆÛ˘.
™ÙÔ ÛÙ¿‰ÈÔ 5, ÂÎÙfi˜ ·fi Ù· ·ÓˆÙ¤Úˆ, ·Ó·-Ê·›ÓÔÓÙ·È Î˘ÛÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ ÛÙÔ ˘Ô¯fiÓ-‰ÚÈÔ ÔÛÙÔ‡Ó.
™Ù· ÛÙ¿‰È· 3-5, Ë ıÂڷ›· Â›Ó·È ¯ÂÈÚÔ˘Ú-ÁÈ΋. ¶ÚÔÙÈÌ¿Ù·È Ë Û˘ÁÎÚ¿ÙËÛË ÙÔ˘ ·ÔÎÔÏ-ÏË̤ÓÔ˘ ÙÂÌ·¯›Ô˘, ÂÊfiÛÔÓ Â›Ó·È ÌÂÁ¿ÏÔ Î·È·Ó‹ÎÂÈ Û ÊÔÚÙÈ˙fiÌÂÓË ·ÚıÚÈ΋ ÂÈÊ¿ÓÂÈ·, ‹Ë ·Ê·›ÚÂÛ‹ ÙÔ˘ ÂÊfiÛÔÓ Â›Ó·È ÌÈÎÚfi Î·È Ë·Ô˘Û›· ÙÔ˘ ‰ÂÓ ‰È·Ù·Ú¿ÛÛÂÈ ÙË ÏÂÈÙÔ˘ÚÁÈÎfi-ÙËÙ· Ù˘ ¿ÚıÚˆÛ˘ (5,6).
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¢È¿ÁÓˆÛË: ™¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· ·ÛÙÚ·Á¿ÏÔ˘
Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·157
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Paediatriki 2009;72:66,71-73
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