Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

Pediatri Mar-Apr 09

10-04-09

15:35

¢ÈÌËÓÈ·›· ¤Î‰ÔÛË Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜ ¶Úfi‰ÚÔ˜ ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘ ∫. ™ÙÂÊ·Ó›‰Ë˜ M¤ÏË ™. ∞Ó‰ÚÔÓ›ÎÔ˘ ª. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ ¶. ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘ °. µ·ÚÏ¿Ì˘ ∂. °·Ï·Ó¿Î˘ §. £ˆÌ·˝‰Ô˘ ª. ∫·Ó¿ÚÈÔ˘ A. K·ÙÙ¿Ì˘ ™. K›ÙÛÈÔ˘-∆˙¤ÏË ∞. ¶··‰ÔÔ‡ÏÔ˘ µ. ¶··Â˘·ÁÁ¤ÏÔ˘ ∞. ™È·ÌÔÔ‡ÏÔ˘-ª·˘Ú›‰Ô˘ ∞. ™˘Ú›ÁÔ˘-¶··‚·ÛÈÏ›Ԣ ∂. ÷ÚÌ·Ó‰¿ÚË ÀÔ‚ÔÏ‹ ∂ÚÁ·ÛÈÒÓ e-mail: hps@ath.forthnet.gr √‰ËÁ›Â˜ ÚÔ˜ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜: http://www.e-child.gr/Instructions_ to_Authors_GR.pdf ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ· ∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ∫. ¶··¯Ú‹ÛÙÔ˘ EÈ̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ™. ¡¿ÎÔ˘

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¶·È‰È·ÙÚÈ΋ ∆fiÌÔ˜ 72 ñ ∆‡¯Ô˜ 2 ñ ª¿ÚÙÈÔ˜-∞Ú›ÏÈÔ˜ 2009

¶ÂÚȯfiÌÂÓ· ∞¡∞™∫O¶∏™∂π™ 75 √Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹ 82 §ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ - ¡ÂfiÙÂÚ· ıÂÚ·¢ÙÈο ‰Â‰Ô̤ӷ 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 ¤ÎÙ˘ÍË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜ π. ¶ÂÏ·ÁÈ¿‰Ë˜, ∂. ¢ËÌËÙÚ›Ô˘, ∂. ™ÙÂȷοÎË, Ã. ¶ÂÚ‰ÈÎÔÁÈ¿ÓÓË, ∂. ÷Ù˙ˉ¿ÎË, ª. ∫·ÏÌ·ÓÙ‹

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117 ¶·ÓÂÏÏ‹ÓÈ· ÔÚÔÂȉËÌÈÔÏÔÁÈ΋ 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·ÓÙfiÔ˘ÏÔ˜

Communications In Practice ∞.∂. ¶ÈÂÚ›·˜ 1∞ 144 51 MÂÙ·ÌfiÚʈÛË TËÏ.: 210 87 78 884 Fax: 210 87 78 822

123 §¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿: ÂÌÂÈÚ›· 28 ÂÙÒÓ ∞. ¶Ô˘ÚÙÛ›‰Ë˜, ¢. ¢ÔÁ¿Ó˘, ª. ª¿Î·, ¢. ªÔ˘¯Ô‡ÙÛÔ˘, ª. µ·Ú‚Ô˘ÙÛ‹, ª. ™˘ÓÔ‰ÈÓÔ‡, ∞ÈÎ. ªÈ¯·‹Ï-™ÙÚ¿ÓÙ˙È·, ∂. ∫ÔÛÌ›‰Ë

EΉfiÙ˘ K. °ÚÈ‚¤·˜

¶ƒ∞∫∆π∫√ £∂ª∞ 132 √È ÂÈÙÒÛÂȘ Ù˘ ˘ÂÚÙÚÔÊ›·˜ ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ ÛÙËÓ Î·Ú‰È·Î‹ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ ∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁÌ¤ÏÔ˘, °. 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˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ Ã. ∆۷ηϛ‰Ë˜, µ. §·ÌÚfiÔ˘ÏÔ˜, Œ. √˘ÚÁηÓÙ˙fiÁÏÔ˘, ¢. ƒ¿ÏÏ˘, ∞. ¶ÂÙÚfiÔ˘ÏÔ˜, ¡. ¡ÈÎÔÏ·˝‰Ë˜ ∫§π¡π∫√ ∫√Àπ∑ 150 ∞ÁfiÚÈ 13 ÂÙÒÓ Ì Â·Ó·Ï·Ì‚·ÓfiÌÂÓ· ÂÂÈÛfi‰È· ÂÒ‰˘Ó˘ Ô‰ÔÎÓËÌÈ΋˜ ¿ÚıÚˆÛ˘ ¡. ª·ÚΤ·˜, π. ¶··¯Ú‹ÛÙÔ˜ ™À¡∆√ª∞ ¶∞π¢π∞∆ƒπ∫∞ ¡∂∞ 151 ¡¤· ·fi ÙÔÓ ¯ÒÚÔ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÏÔÈÌÒÍÂˆÓ ¡. ™˘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˘

I‰ÈÔÎÙ‹Ù˘ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·© Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 Aı‹Ó· 115 28 TËÏ.: 210 7771 140 210 7771 663 Fax: 210 7758 354 e-mail: hps@ath.forthnet.gr EÙ‹ÛÈ· ™˘Ó‰ÚÔÌ‹: 40 € EȉÈ΢fiÌÂÓÔÈ, ºÔÈÙËÙ¤˜: 20 € ∫ˆ‰ÈÎfi˜ ¢È‡ı˘ÓÛ˘ ∂ÔÙ›·˜ ªª∂: 3889

ISSN 0377-2551

¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√ 156 πÛÙÔÛÂÏ›‰· Ù˘ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ™. ∫¯·ÁÈ¿˜, ¢. ∞Ó·ÁÓÒÛÙÔ˘

Pediatri Mar-Apr 09

10-04-09

15:36

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Bimonthly Publication of the Greek Paediatric Society

Paediatriki

President A. Constantopoulos Editorial Board Editor-in-Chief C. Stefanidis Members S. Andronikou ª. ∞nthracopoulos P. Augoustides-Savvopoulou G. Varlamis ∂. Galanakis L. Thomaidou M. Kanariou A. Kattamis S. Kitsiou-Tzeli ∞. Papadopoulou V. Papaevagelou A. Siamopoulou-Mavridou A. Syrigou-Papavasiliou E. Charmandari Manuscript submission e-mail: hps@ath.forthnet.gr Instructions to authors: http://www.e-child.gr/paediatriki/ iae.pdf Manuscript Editing Greek Editing K. Papachristou English Editing S. Nakou Publisher K. Griveas Publishing Coordinator Communications In Practice S.∞.

1∞ Pierias St. GR - 144 51, Metamorfossi Tel.: +30 210 87 78 884 Fax: +30 210 87 78 822 Owner Greek Paediatric Society© 92 Michalakopoulou St. GR - 115 28, Athens Tel.: +30 210 7771 140 +30 210 7771 663 Fax: +30 210 7758 354 e-mail: hps@ath.forthnet.gr Annual Subscription All foreign countries: US $ 50

Volume 72 ñ Number 2 ñ March-April 2009

Contents REVIEW ARTICLES

PRACTICAL ISSUES

75 Acute lymphoblastic leukaemia in children and adolescents E. Stiakaki, M. Kalmanti

132 Cardiovascular consequences of adenotonsillar hypertrophy in children K. Papadopoulou-Legbelou, G. Varlamis

82 Childhood lymphomas - Novel therapeutic approaches V. Danilatou, M. Kalmanti

139 Defining obesity in children and adolescents: methodological dilemmas P. Pervanidou

89 Haemophagocytic lymphohistiocytosis - HLH C. Koutsaftiki, E. Mantziou, N. Myriokefalitakis

CASE REPORT

97 Current data on congenital diaphragmatic hernia C. Tsakalidis, E. Ourgantzoglou, N. Nikolaidis ORIGINAL ARTICLES

145 Bochdalek congenital diaphragmatic hernia with intrathoracic ectopic kidney a case report C. Takalidis, V. Lampropoulos, E. Ourgantzoglou, D. Rallis, A. Petropoulos, N. Nikolaidis

109 The effect of paediatric bone-marrowderived 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

CLINICAL QUIZ

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

151 News on paediatric infections N. Spyridis

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

150 Thirteen-year-old boy with recurrent episodes of painful ankle joint ¡. Markeas, π. Papachristos PAEDIATRIC NEWS IN BRIEF

HELLENIC RESEARCH IN THE INTERNATIONAL 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

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∞¡∞™∫√¶∏™∏

REVIEW ARTICLE

75

√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˜ ÛÙË ‰È¿ÁÓˆÛË Î·È Ë ıÂÚ·›· 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 ÚfiÏÔ ÛÙËÓ ÚÔÛ·ÚÌÔÁ‹ Ù˘ ıÂÚ·›·˜ Î·È ÛÙËÓ ·Ó·˙‹ÙËÛË Ó¤ˆÓ ıÂÚ·¢ÙÈÎÒÓ ÛÙfi¯ˆÓ. ∏ ηٿٷÍË Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ ‚·Û›˙ÂÙ·È Û ÎÏÈÓÈο Î·È Î˘ÙÙ·ÚÔÁÂÓÂÙÈο ‰Â‰Ô̤ӷ, ηıÒ˜ Î·È ÛÙËÓ ÂÎÙ›ÌËÛË Ù˘ ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ 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 ıÂÚ·›·. ∂ÈÚfiÛıÂÙ·, 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 ıÂÚ·‡ÔÓÙ·È Ì ·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ·, Û˘ÁÎÚÈÙÈο Ì ÂΛÓÔ˘˜ Ô˘ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ıÂÚ·¢ÙÈο Û¯‹Ì·Ù· ÙˆÓ ÂÓËϛΈÓ.

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘ AÏÏËÏÔÁÚ·Ê›·: ∂˘Ù˘¯›· ™ÙÂȷοÎË efstel@med.uoc.gr ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜, ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ, 71110 ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘

§¤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 in diagnosis 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 for specific treatment. Current risk classification schemes are based on clinical characteristics and cytogenetic features, and on minimal residual disease assessment, which appears to be among the strongest prognostic factors and provides an opportunity for more precise risk tailored treatments. Recent studies have shown that adolescents and young adults have a much better outcome when treated with paediatric than with adult protocols.

Department of Paediatric Haematology/Oncology, University of Crete, Greece Correspondence: Eftichia Stiakaki efstel@med.uoc.gr Department of Paediatric Haematology/Oncology, University Hospital of Heraklion, 71110 Heraklion, Crete, Greece

Key words: Acute lymphoblastic leukaemia, children, adolescents

™˘ÓÙÔÌÔÁڷʛ˜: ALL BFM CCG COG DFCI MRD NCI POG √§§ ∫¡™

acute lymphoblastic leukemia Berlin-Frankfurt-Muenster Children’s Cancer Group Children’s Oncology Group Dana-Farber Cancer Institute Minimal Residual Disease (ÂÏ¿¯ÈÛÙË ˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ˜) National Cancer Institute 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¿ Î·È ·ÔÙÂÏ› ÙÔ 75-80% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ Ó¤ˆÓ ÂÚÈÙÒÛÂˆÓ Ï¢¯·ÈÌ›·˜. ∏ Û˘¯ÓfiÙËÙ¿ Ù˘ Â›Ó·È 35 Ì 40 Ӥ˜ ÂÚÈÙÒÛÂȘ ÙÔ ¯ÚfiÓÔ ·Ó¿ 1000.000 ·È‰È¿ ËÏÈΛ·˜ <15 ÂÙÒÓ, Ì ̤ÁÈÛÙË Â›ÙˆÛË ÛÙËÓ ËÏÈΛ· ÙˆÓ 4 ÂÙÒÓ (1). ∏ ÚfiÔ‰Ô˜ ÛÙË ıÂÚ·›· Ù˘ √§§ ÛÙ· ·È-

‰È¿ ·ÔÙÂÏ› ÌÂÁ¿ÏÔ Â›Ù¢ÁÌ· Ù˘ Û‡Á¯ÚÔÓ˘ ÎÏÈÓÈ΋˜ ÔÁÎÔÏÔÁ›·˜, ηı’ fiÛÔÓ ÙÔ 1970 ÙÔ ÔÛÔÛÙfi ›·Û˘ ÌfiÏȘ ¿ÁÁÈ˙ ÙÔ 30%, ÂÓÒ Û‹ÌÂÚ· ¤¯ÂÈ ˘Âڂ› ÙÔ 80% (2). ∏ ÚfiÔ‰Ô˜ Ô˘ ¤¯ÂÈ ÂÈÙ¢¯ı› ÔÊ›ÏÂÙ·È ÛÙË ¯Ú‹ÛË ·ÔÙÂÏÂÛÌ·ÙÈÎÒÓ ¯ËÌÂÈÔıÂÚ·¢ÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÛÙ· Ï·›ÛÈ· Ù˘¯·ÈÔÔÈËÌ¤ÓˆÓ ÌÂÏÂÙÒÓ, ÛÙË ıÂÚ·›· Ù˘ ˘ÔÎÏÈÓÈ΋˜ ÓfiÛÔ˘ ÙÔ˘ ∫¡™, ÛÙËÓ ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂÚ·›·˜ ηÈ, ‚¤‚·È·, ÛÙËÓ Ù·ÍÈÓfiÌËÛË Î·È ıÂÚ·›· 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·ÎÔÁÂÓÂÙÈΤ˜ ÌÂϤÙ˜ Î·È ÛÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÚfiÏÔ˘ Ù˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘. ¶·Ú¿ ÙËÓ ÚfiÔ‰Ô Ô˘ ¤¯ÂÈ ÂÈÙ¢¯ı›, ¤Ó· ÔÛÔÛÙfi ·È‰ÈÒÓ Ï·Ì‚¿ÓÂÈ ›Ûˆ˜ ÂÚÈÛÛfiÙÂÚË ıÂÚ·›· ·fi ÂΛÓË Ô˘ Ú·ÁÌ·ÙÈο ¯ÚÂÈ¿˙ÂÙ·È ÁÈ· Ó· È·ı›, 10-20% ÙˆÓ ·È‰ÈÒÓ Ì ·Ú¯Èο ¢ÓÔ˚ÎÔ‡˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÛÙË ¶·È‰È·ÙÚÈ΋ 2009;72:75-000

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·76

76

∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹

Û˘Ó¤¯ÂÈ· ˘ÔÙÚÔÈ¿˙Ô˘Ó, ÂÓÒ ˘¿Ú¯ÂÈ Â›Û˘ ÌÈ· ÔÌ¿‰· ·ÛıÂÓÒÓ Ì ÓfiÛÔ ·ÓıÂÎÙÈ΋ ÛÙË ıÂÚ·›·. ∂Âȉ‹ Ë ÂÚ·ÈÙ¤Úˆ ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂÚ·›·˜ ‰ÂÓ Ê·›ÓÂÙ·È Ó· ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÚfiÁÓˆÛË, ÙÔ ÂӉȷʤÚÔÓ ¤¯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 ÂÈÚfiÛıÂÙˆÓ ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ¤ÙÛÈ ÒÛÙÂ Ë Ù·ÍÈÓfiÌËÛË Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ Ó· Â›Ó·È ÈÔ ·ÎÚÈ‚‹˜.

¶›Ó·Î·˜ 1. ∆·ÍÈÓfiÌËÛË Ù˘ Û˘ÌÌÂÙÔ¯‹˜ ÙÔ˘ ∫¡™ ÛÙËÓ √§§

∆·ÍÈÓfiÌËÛË Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ °È· ÔÏϤ˜ ‰ÂηÂٛ˜, Ë ıÂÚ·›· ‚·Û›ÛÙËΠ۠ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ 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˘ÙÙ·ÚÔÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ ‚Ï·ÛÙÒÓ, ηÈ, ÌÂÙ·ÁÂÓ¤ÛÙÂÚ·, Ë ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂÚ·›· (4,5). ™Ù· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ıÂÚ·›·˜ ¤¯ÂÈ ÚÔÛÙÂı› Ë ÂÎÙ›ÌËÛË Ù˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘. ∏ ËÏÈΛ· Î·È Ô ·ÚÈıÌfi˜ ÙˆÓ Ï¢ÎÒÓ ÛÙË ‰È¿ÁÓˆÛË ·Ú·Ì¤ÓÔ˘Ó ‰È·¯ÚÔÓÈο ÛÙ·ıÂÚÔ› Î·È ·ÓÂÍ¿ÚÙËÙÔÈ ÚÔÁÓˆÛÙÈÎÔ› ·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¿‰· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (4). ∞ÓÙ›ÛÙÔȯ·, Î·È ÛÙ· ÚˆÙfiÎÔÏÏ· BFM ̤¯ÚÈ Î·È ÙÔ 1995, Ë ËÏÈΛ· 1-6 ÂÙÒÓ Î·È Ô ·ÚÈıÌfi˜ Ï¢ÎÒÓ <20.000 Îί ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ÙËÓ Î·Ù¿Ù·ÍË 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 ıÂÚ·›· Ù˘ ˘ÔÎÏÈÓÈ΋˜ ÓfiÛÔ˘, ·fi Ù· Ù¤ÏË Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ 1960, Û˘Ó¤‚·Ï ٷ ̤ÁÈÛÙ· ÛÙË ‚ÂÏÙ›ˆÛË Ù˘ ÂÈ‚›ˆÛ˘/›·Û˘. ™‹ÌÂÚ·, Ë ıÂÚ·›· Ù˘ ˘ÔÎÏÈÓÈ΋˜ ÓfiÛÔ˘ ‚·Û›˙ÂÙ·È ÛÙËÓ ÂÈıÂÙÈ΋ Û˘ÛÙËÌ·ÙÈ΋ ¯ËÌÂÈÔıÂÚ·›· (HDMTX ) Î·È ÛÙËÓ ÂÓ‰ÔÚÚ·¯È·›· ¯ÔÚ‹ÁËÛË ÌÂıÔÙÚÂÍ¿Ù˘ ÛÙÔ 80-90% ÙˆÓ ·È‰ÈÒÓ. ™ÙÔ˘˜ ·ÛıÂÓ›˜ ˘„ËÏÔ‡ ‹ Ôχ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ¯ÔÚËÁÂ›Ù·È ÙÚÈÏ‹ ÂÓ‰ÔÚÚ·¯È·›· ¯ËÌÂÈÔıÂÚ·›· Î·È ·ÎÙÈÓÔ‚ÔÏ›· ÎÚ·Ó›Ô˘ (12Gy) Û fiÏ· ۯ‰fiÓ Ù· ΤÓÙÚ·, Ù·ÎÙÈ΋ Ô˘ ¤¯ÂÈ ÌÂÈÒÛÂÈ ÙÔ ÔÛÔÛÙfi ÙˆÓ ˘ÔÙÚÔÒÓ ÌÂ Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ∫¡™ Û <5% Î·È ÙˆÓ ÌÂÌÔÓˆÌ¤ÓˆÓ ˘ÔÙÚÔÒÓ ∫¡™ ÛÙÔ 1,1%. ∂Í·›ÚÂÛË ·ÔÙÂÏ› ÙÔ St. Jude, ÛÙ· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ÙÔ˘ ÔÔ›Ô˘ ‰ÂÓ ¯ÔÚËÁÂ›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¤ÓÙËÛË ÛÙË ‰È¿ÁÓˆÛË ÂÈ‚·Ú‡ÓÂÈ ÙËÓ ÙÂÏÈ΋ Â΂·ÛË ÙˆÓ

·ÛıÂÓÒÓ, Ì ÔÛÔÛÙfi ˘ÔÙÚÔÒÓ ÙÔ˘ ∫¡™ ÛÙ· ÂÚÈÛÙ·ÙÈο ·˘Ù¿ ÂÚ›Ô˘ 8% (8). ŒÙÛÈ, ‹‰Ë ÛÙ· ÚˆÙfiÎÔÏÏ· BFM -95 Î·È St. Jude XIII ·˘Í‹ıËÎÂ Ô ·ÚÈıÌfi˜ ÙˆÓ ÂÓ‰ÔÚÚ·¯È·›ˆÓ ¯ÔÚËÁ‹ÛÂˆÓ ÛÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜.

Paediatriki 2009;72:76-000

¡fiÛÔ˜ ÛÙÔ ∫¡™ ñ CNS 1 ∞Ô˘Û›· ‚Ï·ÛÙÒÓ ÛÙËÓ Î˘ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ÙÔ˘ ∂¡À ñ CNS 2 <5 WBC/Ìl ÛÙËÓ Î˘ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ÌÔÚÊÔÏÔÁÈο ‚Ï¿ÛÙ˜ ñ CNS 3 ≥5 WBC/Ìl ÌÔÚÊÔÏÔÁÈο ‚Ï¿ÛÙ˜

∞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 ·ÍÈfiÈÛÙ˘ ΢ÙÙ·ÚÔÌÂÙÚ›·˜ ÚÔ‹˜. ¢Â›ÎÙ˜ ȉȷ›ÙÂÚ· ¢·›ÛıËÙÔÈ ÁÈ· ÙË µ Î·È ∆ ÛÂÈÚ¿ ·ÓÙ›ÛÙÔȯ· Â›Ó·È Ù· CD19 Î·È CD7 Î·È ‰Â›ÎÙ˜ Ì ÌÂÁ¿ÏË ÂȉÈÎfiÙËÙ· Ù· ΢ÙÙ·ÚÔÏ·ÛÌ·ÙÈο CD79a, CD3 ÁÈ· µ Î·È ∆ ÏÂÌÊÔ·ÙÙ·Ú·, ·ÓÙ›ÛÙÔȯ·. ªÂ ÙÔÓ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô ÌfiÓÔ, Ë ‰È¿ÁÓˆÛË ÌÔÚ› Ó· ÙÂı› ÛÙÔ 99% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (9). ªÂ ‚¿ÛË ÙÔÓ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô ÙˆÓ ‚Ï·ÛÙÒÓ, Ë Î‡ÚÈ· ‰È¿ÎÚÈÛË Â›Ó·È ÌÂٷ͇ √§§ B-΢ÙÙ·ÚÈ΋˜ ÚÔ¤Ï¢Û˘ (µ-lineage ‹ µ-precursor ALL) (85% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÛÙ· ·È‰È¿) Î·È ∆√§§, Ô˘ ·ÔÙÂÏ› ÙÔ ˘fiÏÔÈÔ 15%. ∏ √§§ B΢ÙÙ·ÚÈ΋˜ ÚÔ¤Ï¢Û˘ ÌÔÚ› Ó· ‰È·ÎÚÈı› ÂÚ·ÈÙ¤Úˆ Û ÚÒÈÌË ÚÔ-µ, ÚÔ-µ, ÌÂÙ·‚·ÙÈ΋ ÚÔ-µ

∂ÈÎfiÓ· 1. §ÂÌÊÔ‚Ï¿ÛÙ˜ Û ΢ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ∂¡À.

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·77

77

√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·

Î·È µ-√§§ (10). ŸÛÔÓ ·ÊÔÚ¿ ÙÔÓ Û¯Â‰È·ÛÌfi Ù˘ ıÂÚ·›·˜ Ù˘ √§§ µ-ÛÂÈÚ¿˜, Ë ÌfiÓË ÛËÌ·ÓÙÈ΋ ‰È¿ÎÚÈÛË Â›Ó·È ÌÂٷ͇ ÒÚÈÌˆÓ µ Î·È Úfi‰ÚÔÌˆÓ µ-΢ÙÙ¿ÚˆÓ. ∞Ó¿ÏÔÁ·, Ë ∆-√§§ ÌÔÚ› Ó· Ù·ÍÈÓÔÌËı› Ì ‚¿ÛË ÙÔ ÛÙ¿‰ÈÔ ‰È·ÊÔÚÔÔ›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, ıÂÚ·›· Î·È ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ (11). ∫ÏÈÓÈο ÛËÌ·ÓÙÈΤ˜ ΢ÙÙ·ÚÔÁÂÓÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ Â›Ó·È Ô ˘ÂÚ‰ÈÏÔÂȉÈÛÌfi˜, Ô ˘Ô‰ÈÏÔÂȉÈÛÌfi˜, ÔÈ ¯ÚˆÌÔÛˆÌÈΤ˜ ÌÂÙ·ı¤ÛÂȘ fiˆ˜ t(12;21), t(1;19), t(9;22), Ì ·ÓÙ›ÛÙÔȯ· ÚÔ˚fiÓÙ· Û‡ÓÙË͢ ÙˆÓ ÁÔÓȉ›ˆÓ TEL-AML1, E2APBX1, BCR-ABL Î·È ÌÈ· ÔÈÎÈÏ›· ·Ó·‰È·Ù¿ÍÂˆÓ ÙÔ˘ MLL ÁÔÓȉ›Ô˘. √ ˘ÂÚ‰ÈÏÔÂȉÈÛÌfi˜ (>50 ¯ÚˆÌÔÛÒÌ·Ù· ‹ DNA index ≥1,16 ), ηıÒ˜ Î·È ÔÈ ÙÚÈۈ̛˜ 4,10,17 ·ÏÏ¿ ΢ڛˆ˜ Ë ÙÚÈÏ‹ ÙÚÈۈ̛· 4,10,17, ¤¯Ô˘Ó ıÂÙÈ΋ Â›ÙˆÛË 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 ıˆڋıËΠ¢ÓÔ˚Îfi˜, Ì ÂÈ‚›ˆÛË >90% ÛÙËÓ ÔÌ¿‰· ·˘Ù‹, ÂÓÒ ·ÎÔÏÔ‡ıËÛ·Ó ·Ó·ÊÔÚ¤˜ ÁÈ· ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· fi„ÈÌˆÓ ˘ÔÙÚÔÒÓ, ¤ÙÛÈ ÒÛÙÂ Ë ÂÈ‚›ˆÛË Ó· ÌË ‰È·Ê¤ÚÂÈ ÌÂÙ¿ ·fi Ì·ÎÚ¿ ÂÚ›Ô‰Ô ·Ú·ÎÔÏÔ‡ıËÛ˘ (15,16). ¶ÚfiÛÊ·Ù·, ÔÈ ‰È·ÊÔÚ¤˜ ÛÙËÓ ¤Î‚·ÛË ·Ô‰›‰ÔÓÙ·È ÛÙÔ Â›‰Ô˜ Î·È ÙËÓ ÂÈıÂÙÈÎfiÙËÙ· ÙˆÓ ıÂÚ·¢ÙÈÎÒÓ ÚˆÙÔÎfiÏψÓ. ∫·Ï‡ÙÂÚË ¤Î‚·ÛË Û¯ÂÙ›˙ÂÙ·È Ì ˘„ËϤ˜ ‰fiÛÂȘ ÌÂıÔÙÚÂÍ¿Ù˘ Î·È L-·Û·Ú·ÁÈÓ¿Û˘. ¶ÚÔÔÙÈ΋ ÌÂϤÙË ¤‰ÂÈÍ fiÙÈ Ì ÂÈıÂÙÈ΋ ¯ËÌÂÈÔıÂÚ·›· ÛÙ· ·È‰È¿ ·˘Ù¿, Ë 5ÂÙ‹˜ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÊÙ¿ÓÂÈ ÙÔ 97%, ¯ˆÚ›˜ fï˜ Ë ÌÂÙ¿ıÂÛË TEL-AML1 Ó· ·Ô‰ÂÈÎÓ‡ÂÙ·È ·ÓÂÍ¿ÚÙËÙÔ˜ ÚÔÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ fiÙ·Ó Ï·Ì‚¿ÓÔÓÙ·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 ıˆÚËı› ‰˘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¿ Â›Ù¢Í˘ ‡ÊÂÛ˘, Û˘¯Ó¤˜ Î·È ÚÒÈ̘ ˘ÔÙÚÔ¤˜ Î·È ÊÙˆ¯‹ Û˘ÓÔÏÈ΋ ÂÈ‚›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 ıÂÚ·›· ÂÎÏÔÁ‹˜ ÁÈ· ›·ÛË, ÂÓÒ Ë ¯ÔÚ‹ÁËÛË ÈÌ·ÙÈÓ›Ì˘ Û˘ÓÈÛÙ¿Ù·È Û˘Á¯ÚfiÓˆ˜ Ì ÙË ¯ËÌÂÈÔıÂÚ·›· (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. ∏ ÌÂÙ¿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 ÂÈıÂÙÈÎÒÓ 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 ∆- ÛÂÈÚ¿˜. ∏ ∆-√§§ Â›Ó·È ÈÔ Û˘¯Ó‹ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ Ì ˘„ËÏfi ·ÚÈıÌfi Ï¢ÎÒÓ Î·È Â›Û˘ Û˘Óԉ‡ÂÙ·È Û˘¯ÓfiÙÂÚ· ·fi ‰È‡ڢÓÛË ÌÂÛÔıˆÚ·Î›Ô˘, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›· Î·È ‰È‹ıËÛË ∫¡™. ªÂ ÙË ¯ÔÚ‹ÁËÛË ÂÈıÂÙÈ΋˜ ¯ËÌÂÈÔıÂÚ·›·˜, 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‹ ÚÔÁÓˆÛÙÈ΋ ·Í›·, fiˆ˜ Â›Û˘ ‰ÂÓ Â›Ó·È Û·Ê‹˜ Ô ÚÔÁÓˆÛÙÈÎfi˜ ÚfiÏÔ˜ ÙˆÓ Î˘ÙÙ·ÚÔÁÂÓÂÙÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ (24). ªÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ NOTCH ¤¯Ô˘Ó ·Ú·ÙËÚËı› ÛÙÔ 50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ∆-√§§, ·ÏÏ¿ Ë ÚÔÁÓˆÛÙÈ΋ ÙÔ˘˜ ÛËÌ·Û›· Â›Ó·È ·Û·Ê‹˜ (27-28).

¶ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂÚ·›· Î·È ÂÏ¿¯ÈÛÙË ˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ˜ ∏ ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂÚ·›· ·ÔÙÂÏ› ÈÛ¯˘Úfi ÚÔÁÓˆÛÙÈÎfi ·Ú¿ÁÔÓÙ·, ÁÈ·Ù› Û¯ÂÙ›˙ÂÙ·È Ì 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 ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂÚ·›· Â›Ó·È Ô ·ÚÈıÌfi˜ ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙÔ ·›Ì· ÙËÓ 8Ë Ë̤ڷ ıÂÚ·›·˜, Ô Ì˘ÂÏfi˜ ÙËÓ 7Ë ‹ 14Ë Ë̤ڷ ηÈ, ÚfiÛÊ·Ù·, Ë ÂÎÙ›ÌËÛË Ù˘ ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ (30-34). ∏ ÔÌ¿‰· ÙÔ˘ BFM ÂÈÛ‹Á·Á ·fi ÙÔ 1983 ÙËÓ ÚÔÁÓˆÛÙÈ΋ ·Í›· Ù˘ ·¿ÓÙËÛ˘ ÛÙËÓ Ú‰ÓÈ˙fiÓË. ∆· ÚˆÙfiÎÔÏÏ· BFM ¯ÚËÛÈÌÔÔÈÔ‡Ó ÙË Ì›ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ‚Ï·ÛÙÒÓ Û <1000/Ìl ÙËÓ 8Ë Ë̤ڷ, ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË Ú‰ÓÈ˙fiÓ˘ Î·È ÌÈ·˜ ÂÓ‰ÔÚÚ·¯È·›·˜ ¤Á¯˘Û˘ MTX, ˆ˜ ÎÚÈÙ‹ÚÈÔ ÁÈ· ¢ÓÔ˚΋ ÚfiÁÓˆÛË (35). √ Ú˘ıÌfi˜ οı·ÚÛ˘ ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· ¤¯ÂÈ ÚÔÁÓˆÛÙÈ΋ ·Í›· Û ∆- Î·È µÛÂÈÚ¿˜ √§§. √ ·ÚÈıÌfi˜ ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙÔ ·›Ì· 7 Ë̤Ú˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ÔÏ˘·Ú·ÁÔÓÙÈ΋˜ ıÂÚ·›·˜ ¶·È‰È·ÙÚÈ΋ 2009;72:10-15

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·78

78

∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹

¶›Ó·Î·˜ 2. ∞ÓÔÛÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË √§§ ÀÔÔÌ¿‰·

ŒÎÊÚ·ÛË ·ÓÙÈÁfiÓˆÓ

™˘¯ÓfiÙËÙ· (%)

¶ÚÒÈÌË ÚÔ-B

CD19+, CD22+, CD79a+, CD10+/-, CD7-, CD3-a, cIgÌ-, sIgÎ-, sIgÏCD19+, CD22+, CD79a+,CD10+/-, CD7-,CD3-a, cIgÌ+, sIgÌ-, sIgÎ-, sIgÏCD19+, CD22+, CD79a+, CD10+/-b, CD7-, CD3-a, cIgÌ+, sIgÌ+, sIgÎ-, sIgÏCD19+, CD22+, CD79a+, CD10+/-b, CD7-, CD3-a, cIgÌ+, sIgÌ+, sIgÎ+, sIgÏ+ CD19+/-c, CD22-, CD79a-, CD10+/-b, CD7+, CD3+a, cIgÌ-, sIgÌ-, sIgÎ-, sIgÏ-

57-65%

¶ÚÔ-µ ªÂÙ·‚·ÙÈ΋ ÚÔ-µ B- √§§ T- √§§

·ÔÙÂÏ› Â›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΋˜ ¯ËÌÂÈÔıÂÚ·›·˜ ¤¯Ô˘Ó ȉȷ›ÙÂÚ· ¢ÓÔ˚΋ ÚfiÁÓˆÛË. ∏ ÌÔÚÊÔÏÔÁÈ΋ ·¿ÓÙËÛË ÛÙË ıÂÚ·›· ÂÍ·ÎÔÏÔ˘ı› Ó· ¤¯ÂÈ ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· Î·È ÛÙ· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ÙÔ˘ COG (37). ∏ ÂÎÙ›ÌËÛË Ù˘ ÚÒÈÌ˘ ·¿ÓÙËÛ˘ ÛÙË ıÂÚ·›·, 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 Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ Î·È ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂÚ·›·˜ (34,38-40). √È ·ÛıÂÓ›˜ Ô˘ ÂÈÙ˘Á¯¿ÓÔ˘Ó ·ÓÔÛÔÏÔÁÈ΋ ‹ ÌÔÚȷ΋ ‡ÊÂÛË, Ô˘ ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ˆ˜ Ì›ˆÛË ÙˆÓ ‚Ï·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Û <0,01% ÙˆÓ ÂÌ‡ÚËÓˆÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ Ì˘ÂÏÔ‡ ÛÙÔ Ù¤ÏÔ˜ Ù˘ Â·ÁˆÁ‹˜ ÛÙËÓ ‡ÊÂÛË, ¤¯Ô˘Ó ÂÍ·ÈÚÂÙÈ΋ ÚfiÁÓˆÛË (41,42).

20-25% 2-3% 2-3% 13-15%

√§§ Û ÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ πÛÙÔÚÈο, Ù· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ Ì √§§ ¤¯Ô˘Ó ¯ÂÈÚfiÙÂÚË ÚfiÁÓˆÛË, Û ۯ¤ÛË Ì ٷ ÌÈÎÚfiÙÂÚ· ·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, fiˆ˜ ˘„ËÏfi˜ ·ÚÈıÌfi˜ Ï¢ÎÒÓ, ∆ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô˜ Î·È ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· ‰˘ÛÌÂÓÒÓ Î˘ÙÙ·ÚÔÁÂÓÂÙÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ (43). √È ‰È·ÊÔÚ¤˜, fï˜, ÛÙËÓ ¤Î‚·ÛË ·Ô‰›‰ÔÓÙ·È Î·È ÛÙË ÏÈÁfiÙÂÚÔ ÂÈıÂÙÈ΋ ıÂÚ·›· Ô˘ Ï·Ì‚¿ÓÔ˘Ó fiÙ·Ó ıÂÚ·‡ÔÓÙ·È Ì ÚˆÙfiÎÔÏÏ· ÂÓËϛΈÓ. ¶ÚfiÛÊ·Ù· ‰ËÌÔÛȇÙËÎ·Ó 3 ·Ó·‰ÚÔÌÈΤ˜ ÌÂϤÙ˜ ·fi ÙËÓ ∂˘ÚÒË 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 ıÂÚ·‡ÔÓÙ·È Ì ·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ· (44-47). ∏ Û‡ÁÎÚÈÛË Ù˘ 5ÂÙÔ‡˜ ÂÈ‚›ˆÛ˘ ·ÛıÂÓÒÓ 15-21 ÂÙÒÓ ¤‰ÂÈÍ ̤¯ÚÈ Î·È 30% ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ›·Û˘ Ì ٷ

100 E2A-PXB1 (n=19)

∂È‚›ˆÛË ÂχıÂÚË Û˘Ì‚·Ì¿ÙˆÓ (%)

90 80 70

Hyperdiploidy >50 chromosomes (n=112) TEL-AML1 (n=81) Other subtypes (n=148)

T-cell ALL (n=78)

60 50 40 30

MLL-AF4 (n=15) BCR-AµL (n=14)

20 10 0 0

2

4

6

8

10

12

ŒÙË ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË ∂ÈÎfiÓ· 2. ∫·Ì‡Ï˜ ÂÈ‚›ˆÛ˘ ˘ÔÔÌ¿‰ˆÓ Ì ‚¿ÛË Ù· ΢ÙÙ·ÚÔÁÂÓÂÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο (∞fi Pui C et al. NEJM 2004).

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√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·

·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ· ıÂÚ·›·˜. ∞Ó Î·È ÔÈ ‰È·ÊÔÚ¤˜ ·˘Ù¤˜ ‰ÂÓ ÌÔÚÔ‡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 Û ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Î·È ıÂÚ·‡ÔÓÙ·È Ì ·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ·, ÌÔÚ› Ó· ÂÈÙ‡¯Ô˘Ó ÔÛÔÛÙ¿ ›·Û˘ Ô˘ Êı¿ÓÔ˘Ó ÙÔ 70%.

∆Ú¤¯Ô˘Û· ıÂÚ·¢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˘ ıÂÚ·›·˜, ¤ÙÛÈ ÒÛÙ ÈÔ ÂÈıÂÙÈ΋ ηÈ, ÂÔ̤ӈ˜, ÈÔ ÙÔÍÈ΋ ıÂÚ·›· Ó· Ï·Ì‚¿ÓÔ˘Ó ÌfiÓÔ Ù· ·È‰È¿ Ô˘ ÙË ¯ÚÂÈ¿˙ÔÓÙ·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 ıÂÚ·¢ÙÈÎÒÓ ÛÙfi¯ˆÓ (48). ™ËÌ·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 ÚˆÙfiÎÔÏÏÔ ALL BFM 2000, ÔÈ ·ÛıÂÓ›˜ Ù·ÍÈÓÔÌÔ‡ÓÙ·È Ì ‚¿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, ˆ˜ 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¿ Ì ÊÙˆ¯‹ ·¿ÓÙËÛË ÛÙËÓ Ú‰ÓÈ˙fiÓË ıˆÚÔ‡ÓÙ·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ MRD, ÂÓÒ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Â›Û˘ ıˆÚÔ‡ÓÙ·È ÔÈ ·ÛıÂÓ›˜ Ì ÌÂÙ¿ıÂÛË t(9;22) Î·È t(4;11), ·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ·¿ÓÙËÛË ÛÙË ıÂÚ·›· (49). ∞fi ÙÔ DFCI ¤¯ÂÈ ÚÔÙ·ı› Â›Û˘ Ó¤Ô Û‡ÛÙËÌ· Ù·ÍÈÓfiÌËÛ˘ ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì √§§ µ-ÛÂÈÚ¿˜. √È ·ÛıÂÓ›˜ ·Ú¯Èο Ù·ÍÈÓÔÌÔ‡ÓÙ·È ˆ˜ ÛÙ·ıÂÚÔ‡ ‹ ˘„ËÏÔ‡ 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˘ ıÂÚ·›·˜ Â·ÁˆÁ‹˜ ÛÙËÓ ‡ÊÂÛË, ÔÈ ·ÛıÂÓ›˜ Ì MRD ≥0,1% Ù·ÍÈÓÔÌÔ‡ÓÙ·È ˆ˜ Ôχ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÂÓÒ ÂΛÓÔÈ Ì MRD <0,1% Û˘Ó¯›˙Ô˘Ó fiˆ˜ Ù·ÍÈÓÔÌ‹ıËÎ·Ó ·Ú¯Èο. ™Ùfi¯Ô˜ Â›Ó·È Ó· ÚÔÛ‰ÈÔÚÈÛı› Â¿Ó Ë ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂÚ·›·˜ ı· ‚ÔËı‹ÛÂÈ ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ ÛÙÔ Ù¤ÏÔ˜ Ù˘ Â·ÁˆÁ‹˜ ÛÙËÓ ‡ÊÂÛË. √È ·ÛıÂÓ›˜ Ì ∆-√§§ Ï·Ì‚¿ÓÔ˘Ó ıÂ-

Ú·›· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ MRD ÛÙË Ê¿ÛË ·˘Ù‹. ø˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Â›Û˘ ÔÈ ·ÛıÂÓ›˜ Ì ˘Ô‰ÈÏÔÂȉÈÛÌfi, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔÓ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô ‹ ÙËÓ ÂÏ¿¯ÈÛÙË ˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ. ™ÙÔ St. Jude Children’s Research Hospital, Ë Ù·ÍÈÓfiÌËÛË Â›Û˘ ‚·Û›˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙ· Â›‰· Ù˘ MRD ÌÂÙ¿ ·fi 6 ‚‰ÔÌ¿‰Â˜ ıÂÚ·›·˜ Â·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¤ÓˆÛË ÙÔ˘ CCG Î·È ÙÔ˘ POG ¤‰ˆÛ ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ·Ó·Ï˘ıÔ‡Ó ·Ó·‰ÚÔÌÈο Ù· ÎÏÈÓÈο ‚ÈÔÏÔÁÈο ‰Â‰Ô̤ӷ Î·È Ë ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂÚ·›· 18.000 ·ÛıÂÓÒÓ Ì √§§, ËÏÈΛ·˜ 1-21.99 ÂÙÒÓ, Î·È Ó· ÚÔÙ·ı› ¤Ó· Ó¤Ô Û‡ÛÙËÌ· Ù·ÍÈÓfiÌËÛ˘ (37). ªÂ ‚¿ÛË ÙÔ ÚÔÙÂÈÓfiÌÂÓÔ Û‡ÛÙËÌ·, ÔÈ ·ÛıÂÓ›˜ Ì √§§ µÛÂÈÚ¿˜ ‰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˘ Â·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) ‹ ÙÚÈۈ̛˜ 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 ıÂÚ·›· Î·È ÛÙ· ΢ÙÙ·ÚÔÁÂÓÂÙÈο ¯·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·ÙÈÎÔÔ›ËÛË Ù˘ ıÂÚ·›·˜ ÙÔ˘˜ Î·È Ó· ÂÚÈÔÚ›ÛÔ˘Ó ÙËÓ ÙÔÍÈÎfiÙËÙ· Ù˘ ıÂÚ·›·˜ Û ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÌÂÈÒÓÔÓÙ·˜ ÂÚ·ÈÙ¤Úˆ ÙË ¯ËÌÂÈÔıÂÚ·›·.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Carrol WL, Bhojwani DJ, et al. Pediatric acute lymphoblastic leukemia. Hematology (Am Soc ∏ematol Educ Program) 2003;102-131.

2. Pui CH, Evans WE. Treatment of acute lymphoblastic leukemia. N Engl J Med 2006;354:166-178.

3. Winick NJ, Carroll WL, Hunger SP. Childhood leukemianew advances and challenges. N Engl J Med 2004;351: 601-603. ¶·È‰È·ÙÚÈ΋ 2009;72:10-15

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4. Smith M, Arthur D, Camitta B, et al. Uniform approach to risk classification and treatment assignment for children with acute lymphoblastic leukemia. J Clin Oncol 1996;14: 18-24.

5. Moricke A, Zimmermann M, Reiter A, et al. Prognostic impact of age in children and adolescents with acute lymphoblastic leukemia: Data from the trials ALL- BFM 86, 90 and 95. Klin Paediatr 2005;217:310-320.

6. Mahmoud HH, Rivera GK, Hancock ML, et al. Low leukocyte counts with blast cells in cerebrospinal fluid of children with newly diagnosed acute lymphoblastic leukemia. N Engl J Med 1993;329:314-319.

7. Pui CH, Mahmoud HH, Rivera GK, et al. Early intensification of intrathecal chemotherapy virtually eliminates central nervous system relapse in children with acute lymphoblastic leukemia. Blood 1998;92:411-415.

8. Gajjar A, Harrison PL, Sandlund JT, et al. Traumatic lumbar puncture at diagnosis adversely affects outcome in childhood acute lymphoblastic leukemia. Blood 2000;96: 3381-3384.

9. Borowitz MJ, Bray R, Gascoyne R, et al. US-Canadian Consensus recommendations on the immunophenotypic analysis of hematologic neoplasia by flow cytometry: data analysis and interpretation. Consensus Development Conference. Cytometry 1997;30:236-244.

10. Pui CH, Behm FG, Crist WM. Clinical and biologic relevance of immunologic marker studies in childhood acute lymphoblastic leukemia. Blood 1993;82:343-362.

11. Trueworthy R, Shuster J, Look T, et al. Ploidy of lymphoblasts is the strongest predictor of treatment outcome in B-progenitor cell acute lymphoblastic leukemia of childhood: a Pediatric Oncology Group study.

J Clin Oncol 1992;10:606-613.

12. Arico` M, Valsecchi MG, Rizzari C, et al. Long-term results of the AIEOP-ALL-95 trial for childhood acute lymphoblastic leukemia: Insight on the prognostic value of DNA index in the framework of Berlin-Frankfurt-Muenster-based chemotherapy. J Clin Oncol 2008;26:283-289.

13. Sutcliffe MJ, Shuster JJ, Sather HN, et al.: High concordance from independent studies by the Children's Cancer Group (CCG) and Pediatric Oncology Group (POG) associating favorable prognosis with combined trisomies 4, 10, and 17 in children with NCI Standard-Risk B-precursor Acute Lymphoblastic Leukemia: a COG initiative. Leukemia 2005;19:734-740.

14. Nachman JB, Heerema NA, Sather H, et al. Outcome of treatment in children with hypodiploid acute lymphoblastic leukaemia. Blood 2007;110:1112-1115.

15. Loh ML, Rubnitz JE. TEL/AML1-positive pediatric leukemia: prognostic significance and therapeutic approaches. Curr Opin Hematol 2002;9:345-352.

16. Seeger K, Adams HP, Buchwald D, et al. TEL-AML1 fusion transcript in relapsed childhood acute lymphoblastic leukemia: the BFM Study Group. Blood 1998;91:17161722.

17. Loh ML, Goldwasser MA, Silverman LB, et al. Prospective analysis of TEL/AML1-positive patients treated on DanaFarber Cancer Institute Consortium Protocol 95-01. Blood 2006;107:4508-4513.

18. Uckun FM, Sensel MG, Sather HN, et al. Clinical significance of translocation t(1;19) in childhood acute lymphoblastic leukemia in the context of contemporary Paediatriki 2009;72:10-15

therapies: a report from the Children's Cancer Group. J Clin Oncol 1998;16:527-535.

19. Arico` M, Valsecchi MG, Camitta B, et al. Outcome of treatment in children with Philadelphia chromosomepositive acute lymphoblastic leukemia. N Engl J Med 2000;342:998-1006.

20. Jones LK, Saha V. Philadelphia positive acute lymphoblastic leukaemia of childhood. Br J Haematol 2005;130: 489-500.

21. Pui CH, Gaynon PS, Boyett JM, et al. Outcome of treatment in childhood acute lymphoblastic leukaemia with rearrangements of the 11q23 chromosomal region.

Lancet 2002;359:1909-1915.

22. Kosaka Y, Koh K, Kinukawa N, et al. Infant acute lymphoblastic leukemia with MLL gene rearrangements: outcome following intensive chemotherapy and hematopoietic stem cell transplantation. Blood 2004;104:3527-3534.

23. Hilden JM, Dinndorf PA, Meerbaum SO, et al. Analysis of prognostic factors of acute lymphoblastic leukemia in infants: report on CCG 1953 from the Children's Oncology Group. Blood 2006;108:441-451.

24. Uckun FM, Sensel MG, Sun L, et al. Biology and treatment of childhood T-lineage acute lymphoblastic leukemia.

Blood 1998;91:735-746.

25. Goldberg JM, Silverman LB, Levy DE, et al. Childhood Tcell acute lymphoblastic leukemia: the Dana-Farber Cancer Institute acute lymphoblastic leukemia consortium experience. J Clin Oncol 2003;21:3616-3622.

26. van Grotel M, Meijerink JP, van Wering ER, et al. Prognostic significance of molecular-cytogenetic abnormalities in pediatric T-ALL is not explained by immunophenotypic differences. Leukemia 2008;22:124-131.

27. Weng AP, Ferrando AA, Lee W, et al. Activating mutations of NOTCH1 in human T cell acute lymphoblastic leukemia. Science 2004;306:269-271.

28. Breit S, Stanulla M, Flohr T, et al. Activating NOTCH1 mutations predict favorable early treatment response and long-term outcome in childhood precursor T-cell lymphoblastic leukemia. Blood 2006;108:1151-1157.

29. Gaynon PS, Desai AA, Bostrom BC, et al. Early response to therapy and outcome in childhood acute lymphoblastic leukemia: a review. Cancer 1997;80:1717-1726.

30. Rautonen J, Hovi L, Siimes MA. Slow disappearance of peripheral blast cells: an independent risk factor indicating poor prognosis in children with acute lymphoblastic leukemia. Blood 1988;71: 989-991.

31. Steinherz PG, Gaynon PS, Breneman JC, et al. Cytoreduction and prognosis in acute lymphoblastic leukaemia - the importance of early marrow response: report from the Childrens Cancer Group. J Clin Oncol 1996;14:389-398.

32. Gajjar A, Ribeiro R, Hancock ML, et al. Persistence of circulating blasts after 1 week of multiagent chemotherapy confers a poor prognosis in childhood acute lymphoblastic leukemia. Blood 1995;86:1292-1295.

33. Griffin TC, Shuster JJ, Buchanan GR, et al. Slow disappearance of peripheral blood blasts is an adverse prognostic factor in childhood T cell acute lymphoblastic leukemia: a Pediatric Oncology Group study. Leukemia 2000;14:792-795.

34. Cavé H, van der Werff ten Bosch J, Suciu S, et al. Clinical significance of minimal residual disease in childhood acute lymphoblastic leukemia. European Organization for

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·81

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√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·

Research and Treatment of Cancer - Childhood Leukemia Cooperative Group. N Engl J Med 1998;339:591-598.

35. Reiter A, Schrappe M, Ludwig W-D, et al. Chemotherapy in 998 unselected childhood acute lymphoblastic leukemia patients. Results and conclusions of the multicenter trial ALL-BFM 86. Blood 1994;84:3122-3133.

36. Schrappe M, Reiter A, Zimmermann M, et al. Long term results of four consecutive trials in childhood ALL performed by the ALL-BFM study group from 1981 to 1995. Leukemia 2000;14:2205-2222.

37. Schultz KR, Pullen DJ, Sather HN, et al. Risk- and response-based classification of childhood B-precursor acute lymphoblastic leukemia: a combined analysis of prognostic markers from the POG and CCG. Blood 2007; 109:926-935.

38. van Dongen JJ, Seriu T, Panzer-Gruemayer ER, et al.

Prognostic value of minimal residual disease in acute lymphoblastic leukaemia in childhood. Lancet 1998;352: 1731-1738.

39. Coustan-Smith E, Sancho J, Hancock ML, et al. Clinical importance of minimal residual disease in childhood acute lymphoblastic leukemia. Blood 2000;96:2691-2696.

40. Björklund E, Mazur J, Söderhäll S, et al. Flow cytometric follow-up of minimal residual disease in bone marrow gives prognostic information in children with acute lymphoblastic leukemia. Leukemia 2003;17:138-148.

41. Panzer-Grümayer ER, Schneider M, Panzer S, et al. Rapid molecular response during early induction chemotherapy predicts a good outcome in childhood acute lymphoblastic leukemia. Blood 2000;95:790-794.

42. Zhou J, Goldwasser MA, Li A, et al. Quantitative analysis of

minimal residual disease predicts relapse in children with B-lineage acute lymphoblastic leukemia in DFCI ALL Consortium Protocol 95-01. Blood 2007;110:1607-1611.

43. Santana VM, Dodge RK, Crist WM, et al. Presenting features and treatment outcome of adolescents with acute lymphoblastic leukemia. Leukemia 1990;4:87-90.

44. Boissel N, Auclerc MF, Lhéritier V, et al. Should adolescents with acute lymphoblastic leukemia be treated as old children or young adults? Comparison of the French FRALLE-93 and LALA-94 trials. J Clin Oncol 2003;21:774-780.

45. de Bont JM, Holt B, Dekker AW, et al. Significant difference in outcome for adolescents with acute lymphoblastic leukemia treated on pediatric vs adult protocols in the Netherlands. Leukemia 2004;18:2032-2035.

46. Ramanujachar R, Richards S, Hann I, et al. Adolescents with acute lymphoblastic leukaemia: outcome on UK national paediatric (ALL97) and adult (UKALLXII/E2993) trials. Pediatr Blood Cancer 2007;48:254-261.

47. Barry E, Deangelo DJ, Neuberg D, et al. Favorable outcome for adolescents with acute lymphoblastic leukemia treated on Dana-Farber Cancer Institute acute lymphoblastic leukemia Concortium Protocols. J Clin Oncol 2007;25:813-819.

48. Bhojwani D, Kang H, Moskowitz NP, et al. Biologic pathways associated with relapse in childhood acute lymphoblastic leukemia: a Children's Oncology Group study. Blood 2006;108:711-717.

49. Moricke A, Reiter A, Zimmermann M, et al. Risk-adjusted therapy of acute lymphoblastic leukemia can decrease treatment burden and improve survival: treatment results of 2169 unselected pediatric and adolescent patients enrolled in the trial ALL-BFM 95. Blood 2008;111:4477-4489.

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§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ - ¡ÂfiÙÂÚ· ıÂÚ·¢ÙÈο ‰Â‰Ô̤ӷ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ÈÌ·ÙÔÏÔÁ›·˜/√ÁÎÔÏÔÁ›·˜, ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ ∫Ú‹Ù˘ AÏÏËÏÔÁÚ·Ê›·: µ·ÛÈÏÈ΋ ¢·ÓËÏ¿ÙÔ˘ pedhem@med.uoc.gr vdanilatou@gmail.com ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ÈÌ·ÙÔÏÔÁ›·˜/√ÁÎÔÏÔÁ›·˜, ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ ∫Ú‹Ù˘, ∆.∫. 71110

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·. ∏ ıÂÚ·›· ÙÔ˘˜ ÂÚÈÏ·Ì‚¿ÓÂÈ Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂÚ·¢ÙÈÎÒÓ Ê·ÚÌ¿ÎˆÓ Î·È ·ÎÙÈÓÔıÂÚ·›·˜ Î·È Ë ÚfiÁÓˆÛË Û˘Ó‹ıˆ˜ Â›Ó·È ÂÍ·ÈÚÂÙÈο ηϋ, Û ·ÓÙ›ıÂÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ. ¶·ÚfiÏ· ·˘Ù¿, οÔÈ· ·È‰È¿ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ˘ÔÙÚÔÈ¿˙Ô˘Ó ‹ Ó· ÂÌÊ·Ó›˙Ô˘Ó ·ÒÙÂÚ˜ ÂÈÏÔΤ˜ ·fi ÙË ıÂÚ·›·. √È ÓÂfiÙÂÚ˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô ÙËÓ ·Ó·ÁÓÒÚÈÛË ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ÙËÓ ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂÚ·¢ÙÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ Î·È ÙËÓ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· οı ·ÛıÂÓÔ‡˜, ¤ÙÛÈ ÒÛÙ ӷ ‚ÂÏÙȈı› Ë ÂÈ‚›ˆÛË, Ì ٷ˘Ùfi¯ÚÔÓË ÂÏ¿ÙÙˆÛË Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ÙÔÍÈÎfiÙËÙ·˜ Ù˘ ıÂÚ·›·˜. ∏ ·Ó·Î¿Ï˘„Ë Ó¤ˆÓ Ê·ÚÌ¿ÎˆÓ Ô˘ ÛÙÔ¯Â‡Ô˘Ó ÂȉÈο Û ÌÔÚÈ·Îfi Â›Â‰Ô ·Ó·Ì¤ÓÂÙ·È Ó· Û˘Ì‚¿ÏÂÈ ÛËÌ·ÓÙÈο ÛÙÔÓ ÙÔ̤· ·˘Ùfi. ¶·Ú¿ÏÏËÏ·, Ë ·Ó¿Ù˘ÍË Â˘·›ÛıËÙˆÓ ÌÂıfi‰ˆÓ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂÚ·›· Î·È Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ Ù˘ ˘ÔÙÚÔ‹˜ Î·È Ë ·Ó›¯Ó¢ÛË ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ ı· ‚ÔËı‹ÛÔ˘Ó ÛÙËÓ ÚÒÈÌË ·Ó·ÁÓÒÚÈÛË Ù˘ ¯ËÌÂÈÔ-¢·ÈÛıËÛ›·˜ ‹ ·ÓıÂÎÙÈÎfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Î·È ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ηٷÏÏËÏfiÙÂÚÔ˘ ıÂÚ·¢ÙÈÎÔ‡ Û¯‹Ì·ÙÔ˜. ∏ ¯Ú‹ÛË Û‡Á¯ÚÔÓˆÓ ‚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 Ì˯·ÓÈÛÌÒÓ, ÛÙËÓ Ù·ÍÈÓfiÌËÛË ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Î·È ÛÙË ‚ÂÏÙÈÛÙÔÔ›ËÛË ÙˆÓ ıÂÚ·¢ÙÈÎÒÓ ÌÂıfi‰ˆÓ. §¤ÍÂȘ ÎÏÂȉȿ: §¤Ìʈ̷, ·È‰È΋ ËÏÈΛ·, Hodgkin, non-Hodgkin, ¯ËÌÂÈÔıÂÚ·›·, ·ÎÙÈÓÔıÂÚ·›·.

Childhood lymphomas - Novel therapeutic approaches Department of Paediatric Hematology/Oncology, University Hospital of Heraklion, Crete, Greece Correspondence: Vassiliki Danilatou pedhem@med.uoc.gr vdanilatou@gmail.com Department of Paediatric Hematology/Oncology, University Hospital of Heraklion, 71110 Crete, Greece

V. Danilatou, M. Kalmanti Abstract: Childhood lymphoma is the third most common paediatric malignancy. Non-Hodgkin lymphoma 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 for children and adolescents, in contrast with that for adults. However, some patients relapse or suffer from long-term toxicity. πn order to improve the overall survival, the aims of current clinical trials are the identification of prognostic markers and the individualization of treatment according to risk group and treatment response. Targeted therapies directed against tumour-characteristic molecules will probably contribute to the reduction of toxicity. The development of sensitive methods for monitoring minimal residual disease and early diagnosis of relapse will assist in choosing the appropriate treatment schedule for each patient. Genomic and proteomic analysis will provide new dimensions in the understanding of the pathogenetic mechanisms, in disease classification and in the optimization of treatment strategies.

Key words: Lymphoma, childhood, Hodgkin, non-Hodgkin, chemotherapy, radiotherapy.

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∂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 ÈÔ Û˘¯Ó¿ Î·È ·ÔÙÂÏÔ‡Ó ÙÔ 60% ÙÔ˘ Û˘ÓfiÏÔ˘, Î·È Ù· Hodgkin ÏÂÌÊÒÌ·Ù· (∏§). ¶ÚfiÎÂÈÙ·È ÁÈ· ÎψÓÈΤ˜ ηÎÔ‹ıÂȘ ˘ÂÚϷۛ˜ 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·,

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¯·ÌËÏÔ‡ ‚·ıÌÔ‡ ηÎÔ‹ıÂÈ·˜ ÏÂÌÊÒÌ·Ù·, Ù· ª∏§ ÛÙ· ·È‰È¿ Â›Ó·È ÈÔ Û˘¯Ó¿ ÂÈıÂÙÈο ÏÂÌÊÒÌ·Ù· ˘„ËÏÔ‡ ‚·ıÌÔ‡ ηÎÔ‹ıÂÈ·˜ (1). ∆Ô ÈÔ Û˘¯Ófi ϤÌʈ̷ ÛÙ· ·È‰È¿ Â›Ó·È ÙÔ Ï¤Ìʈ̷ Burkitt (µ§) Î·È ·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›˙ÔÓÙ·È ·fi ‰È·ÊÔÚÂÙÈΤ˜ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ, fiˆ˜ ÔÈΛÏÔ˘ ‚·ıÌÔ‡ ÚԉȿıÂÛË ÁÈ· ‰È‹ıËÛË ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ Î·È/‹ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (∫¡™), ηıÒ˜ Î·È ÌÔÚȷ΋ Î·È Î˘ÙÙ·ÚÔÁÂÓÂÙÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ·, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ··ÈÙÔ‡Ó ‰È·ÊÔÚÂÙÈΤ˜ ıÂÚ·¢ÙÈΤ˜ ÚÔÛÂÁÁ›ÛÂȘ (3). ª¤¯ÚÈ ÚfiÛÊ·Ù·, ˘‹Ú¯·Ó ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜ ÚÔÛÂÁÁ›ÛÂȘ ÁÈ· ÙÔÓ ‰È·¯ˆÚÈÛÌfi ÙˆÓ ıÂÚ·¢ÙÈÎÒÓ ÔÌ¿‰ˆÓ: ·Ó¿ÏÔÁ· Ì ÙËÓ ÈÛÙÔÏÔÁÈ΋ ÔÌ¿‰· Î·È ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ (4). ¶Ùˆ¯Ô› ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Â›Ó·È Ë ˘„ËÏ‹ LDH ÔÚÔ‡, Ë ËÏÈΛ· >15 ÂÙÒÓ, Ë ÌË ·¿ÓÙËÛË ÛÙË ıÂÚ·›· ÚfiÊ·Û˘ Ì ΢ÎÏÔʈÛÊ·Ì›‰Ë, ‚ÈÓÎÚÈÛÙ›ÓË Î·È Ú‰ÓÈ˙ÔÏfiÓË 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 ÏÂÌÊÔÙÔ͛Ӣ-· (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˜ ıÂÚ·¢ÙÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘. ∏ ÚÒÙË ÔÌ¿‰· ·ÊÔÚ¿ Ù· §§, Ô˘ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ÚˆÙfiÎÔÏÏ· Ù‡Ô˘ ÔÍ›·˜ ÏÂÌÊÔ‚Ï·ÛÙÈ΋˜ Ï¢¯·ÈÌ›·˜ Î·È ‚·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›˙ÔÓÙ·È Ì ‚Ú·¯Â›·˜ ‰È¿ÚÎÂÈ·˜ Â·Ó·Ï·Ì‚·ÓfiÌÂÓÔ˘˜ ·ÎÏÔ˘˜ ÂÓÙ·ÙÈ΋˜ Û˘Ó‰˘·Ṳ̂Ó˘ ¯ËÌÂÈÔıÂÚ·›·˜. ∆¤ÏÔ˜, Ë ÙÚ›ÙË ÔÌ¿‰· ·ÊÔÚ¿ Ù· ∞§ª∫, ÁÈ· Ù· ÔÔ›·, ·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ·Ú¯Èο ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó ÂÈÙ˘¯Ò˜ Ì ÚˆÙfiÎÔÏÏ· Î·È ·fi ÙȘ ‰‡Ô ÔÌ¿‰Â˜, ÙÂÏÈο ·Ó·Ù‡¯ıËÎ·Ó Í¯ˆÚÈÛÙ¿ ıÂÚ·¢ÙÈο ÚˆÙfiÎÔÏÏ· (4). ∂Âȉ‹ Ë ÚÒÙË ÔÌ¿‰· ¯ÚËÛÈÌÔÔÈ› ÚˆÙfiÎÔÏÏ· ıÂÚ·›·˜ 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¿ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÒÚÈÌÔ µ-Ê·ÈÓfiÙ˘Ô, Ì Tdt-, CD10+, CD19+, CD20+, sIg+, CD22+ (4). ™Â ÔÛÔÛÙfi 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), Ì ·Ó·‰È¿Ù·ÍË ÙÔ˘ c-myc. ∏ ÓfiÛÔ˜ Û˘Ó‹ıˆ˜ ÂÓÙÔ›˙ÂÙ·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˘ ıÂÚ·›·˜, ¤¯ÂÈ ÂÏ·¯ÈÛÙÔÔÈËı› ÌÂÙ¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÚfiÊ·Û˘ ÛÙË ıÂÚ·›· (Ì ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Î·È ¯·ÌËÏ‹ ‰fiÛË Î˘ÎÏÔʈÛÊ·Ì›‰Ë˜) Î·È ÙË ¯Ú‹ÛË Ù˘ Ú·Û‚Ô˘ÚÈοÛ˘ Ì ÂÓÙ·ÙÈ΋ ÂÓ˘‰¿ÙˆÛË. ∏ ·ÎÙÈÓÔıÂÚ·›· Î·È Ë ÂÎÙÂٷ̤ÓË ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ‰ÂÓ ¤¯Ô˘Ó ϤÔÓ ı¤ÛË ÛÙË ıÂÚ·¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË 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 ÂÓÙÔÈṲ̂ӷ Î·È Û·ÓÈfiÙÂÚ· 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΋ ÌÂÙ¿ıÂÛË t(14;18) [IgH/bcl-2] Ì ·Ó·‰È¿Ù·ÍË ÙÔ˘ bcl-2 ÛÙËÓ ÂÚÈÔ¯‹ Ù˘ ‚·ÚÈ¿˜ ·Ï‡ÛÔ˘ ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ, ÂÓÒ ÈÔ Û˘¯Ó¿ ·ÓȯÓ‡ÔÓÙ·È ·Ó·‰È·Ù¿ÍÂȘ ÙÔ˘ c-myc. ∏ ÛËÌ·Û›· ÙˆÓ ÛˆÌ·ÙÈÎÒÓ ˘ÂÚÌÂÙ·ÏÏ¿ÍÂˆÓ ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ, Ô˘ Â›Ó·È ÁÓˆÛÙ‹ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, ÛÙ· ·È‰È¿ ‰ÂÓ ¤¯ÂÈ ·ÔÛ·ÊËÓÈÛÙ›. ∏ ÂÓÙ·ÂÙ‹˜ ÂÈ‚›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 ıÂÚ·¢ÙÈΤ˜ ÂÈÏÔÁ¤˜ ÁÈ· Ù· B§ Î·È ¢§ªµ§ Â›Ó·È ‚Ú·¯Â›·˜ ‰È¿ÚÎÂÈ·˜ ÂÓÙ·ÙÈο ¯ËÌÂÈÔıÂÚ·¢ÙÈο ÚˆÙfiÎÔÏÏ· (8). ∆· ÚˆÙfiÎÔÏÏ· ¯ÚËÛÈÌÔÔÈÔ‡Ó ¤Ó· Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂÚ·¢ÙÈÎÒÓ Ê·ÚÌ¿ÎˆÓ Ì ‰È·ÊÔÚÂÙÈ΋ ΢ÙÙ·ÚÔÙÔÍÈ΋ ‰Ú¿ÛË Î·È ‰È·ÊÔÚÂÙÈ΋ ÙÔÍÈÎfiÙËÙ· Î·È ¤¯Ô˘Ó ˆ˜ ÊÈÏÔÛÔÊ›· ÙË Û˘Ó¯‹ ‹ ÎÏ·ÛÌ·ÙÔÔÈË̤ÓË ¤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, ‚ÈÓÎÚÈÛÙ›Ó˘, ΢ÎÏÔʈÛÊ·Ì›‰Ë˜ ‹ ÈʈÛÊ·Ì›‰Ë˜, ˘„ËÏ‹˜ ‰fiÛ˘ ÌÂıÔÙÚÂÍ¿Ù˘, Î˘Ù·Ú·‚›Ó˘, ·‰ÚÈ·Ì˘Î›Ó˘ Î·È ÂÙÔÔÛ›‰Ë˜, Û˘Ó‹ıˆ˜ Û ·ÎÏÔ˘˜ ÙˆÓ 3 ‚‰ÔÌ¿‰ˆÓ, Ì Ôχ ηϿ ·ÔÙÂϤÛÌ·Ù· (ÂÈ‚›ˆÛË ÂχıÂÚË Û˘Ì‚·Ì¿ÙˆÓ ÛÙ· 3-5 ¤ÙË 77-91%). ∂ÈϤÔÓ, 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 ÂÈ‚›ˆÛË, Û‡Á¯ÚÔÓ˜ ÔÏ˘ÎÂÓÙÚÈΤ˜ ÌÂϤÙ˜ ÚÔÛ¿ıËÛ·Ó Ó· ÂÏ·ÙÙÒÛÔ˘Ó ÙË ‰fiÛË Î·È ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜. ŒÙÛÈ, ¤ÁÈÓ ÚÔÛ¿ıÂÈ· Ó· ÂÏ·ÙÙˆı› Ë ‰fiÛË Ù˘ ¯ËÌÂÈÔıÂÚ·›·˜ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, Ì ·ÚÓËÙÈÎfi fï˜ ·ÔÙ¤ÏÂÛÌ· ÛÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË, ÂÓÒ Û ÂӉȷ̤ÛÔ˘ ÎÈÓ‰‡ÓÔ˘ ·ÛıÂÓ›˜ ÂÏ·ÙÙÒıËΠ̠ÂÈÙ˘¯›· Ë ‰fiÛË Ù˘ ¯ËÌÂÈÔıÂÚ·›·˜, ¯ˆÚ›˜ ·ÚÓËÙÈο ·ÔÙÂϤÛÌ·Ù· ÛÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË (11-13). Ÿˆ˜ ·Ó·Ê¤ÚıËÎÂ, ÔÈ ·ÛıÂÓ›˜ Ì µ§ ¤¯Ô˘Ó ·˘ÍË̤ÓË Èı·ÓfiÙËÙ· ÓfiÛÔ˘ ÛÙÔ ∫¡™. ∏ Û˘ÛÙËÌ·ÙÈ΋ ¶·È‰È·ÙÚÈ΋ 2009;72:83-000

Pediatri Mar-Apr 09

10-04-09

15:36

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84

µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹

¯ÔÚ‹ÁËÛË ˘„ËÏ‹˜ ‰fiÛ˘ ÌÂıÔÙÚÂÍ¿Ù˘ Î·È ÙÚÈÏ‹˜ ÂÓ‰ÔÚÚ·¯È·›·˜ ıÂÚ·›·˜ ÂÏ·ÙÙÒÓÂÈ ÙËÓ Èı·ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ ÛÙÔ ∫¡™ Û ·ÛıÂÓ›˜ Ô˘ ‹Ù·Ó ·ÚÓËÙÈÎÔ› 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˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ Ô˘ Ó· Û˘ÁÎÚ›ÓÔ˘Ó ÙÔÓ ÚfiÏÔ Ù˘ ·ÎÙÈÓÔıÂÚ·›·˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÚÔÛ‚ÔÏ‹ ∫¡™ (4). ∞Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÙÂÏÂ˘Ù·›· ¤¯ÂÈ ÂÓۈ̷وı› Ë ÌÂϤÙË ÙÔ˘ ÁÔÓȉȷÎÔ‡ ÚÔÊ›Ï ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ ÛÙ· ·È‰È¿ Ì ¢§ªµ§ Î·È B§ (8). Ÿˆ˜ Â›Ó·È ÁÓˆÛÙfi, Ù· ¢§ªµ§ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÂÁ¿ÏË ÎÏÈÓÈ΋ Î·È ‚ÈÔÏÔÁÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, Ë ÔÔ›· ·ÓÙ·Ó·ÎÏ¿Ù·È Û ‰È·ÊÔÚÂÙÈ΋ ÚfiÁÓˆÛË Î·È ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›·. √È Alizadeh Î·È Û˘Ó., ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ 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-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ» Î·È ÙËÓ «ÂÓÂÚÁÔÔÈË̤ÓË», ÂÓÒ ·ÚÁfiÙÂÚ· ÔÈ Rosenwald Î·È Û˘Ó. ÂȂ‚·›ˆÛ·Ó ÙËÓ ‡·ÚÍË 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¿ Ì ¢§ªµ§. ¶·ÚfiÏ· ·˘Ù¿, ÚfiÛÊ·ÙË ÌÂϤÙË, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ·ÓÔÛÔ˚ÛÙÔ¯ËÌ›· Î·È «Â› ÙfiÔ˘» ˘‚ÚȉÈÛÌfi ÊıÔÚÈÛÌÔ‡ (FISH), η٤‰ÂÈÍ fiÙÈ ÛÙ· ·È‰È¿ Ù· ¢§ªµ§ Â›Ó·È ÈÔ Û˘¯Ó¿ «Ù‡Ô˘ ‚Ï·ÛÙÈÎÔ‡ ΤÓÙÚÔ˘ µ-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ» Î·È ÁÈ’ ·˘Ùfi ¤¯Ô˘Ó Î·È Î·Ï‡ÙÂÚË ¤Î‚·ÛË (17). ™Â ÂÚÈÙÒÛÂȘ ˘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‡˜ ıÂÚ·›· ÂÎÏÔÁ‹˜ Ô˘ ›Ûˆ˜ ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÂÈ‚›ˆÛË, ÂÓÒ ·Ú¿ÏÏËÏ· Á›ÓÔÓÙ·È Î·È ÂÈÚ·Ì·ÙÈΤ˜ ÛÙÔ¯Â˘Ì¤Ó˜ ıÂÚ·›˜ (18). ¶Ôχ ÛËÌ·ÓÙÈÎfi˜ ÚÔÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Û ÂÚ›ÙˆÛË ˘ÔÙÚÔ‹˜ Â›Ó·È Ë Â›Ù¢ÍË ‰Â‡ÙÂÚ˘ ‡ÊÂÛ˘ Î·È Ë ·¿ÓÙËÛË ÛÙË ıÂÚ·›· ‰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 Ì ÁÂÓÈÎÂ˘Ì¤ÓË ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· Î·È µ-Û˘ÌÙÒÌ·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 ¯ÚˆÌÔÛˆÌÈ΋ ÌÂÙ¿ıÂÛË t(2;5)(p23;q35) Ô˘ Ô‰ËÁ› ÛÙÔ ÚˆÙÂ˚ÓÈÎfi Û‡ÌÏÂÁÌ· NPM/ALK Ì ‰Ú·ÛÙËÚÈfiÙËÙ· Ù˘ÚÔÛÈÓÈ΋˜ ÎÈÓ¿Û˘ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ¢ÓÔ˚ÎfiÙÂÚË ÚfiÁÓˆÛË, Û ·ÓÙ›ıÂÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ. ∆Ô Û‡ÌÏÂÁÌ· ·˘Ùfi Ô‰ËÁ› Û ÂÏ·Ùو̤ÓË ·fiÙˆÛË, ̤ۈ ÙˆÓ ÌÔÓÔ·ÙÈÒÓ PI3K Î·È PKB/AKT, ηıÒ˜ Î·È Paediatriki 2009;72:1-9

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· ¯ËÌÂÈÔıÂÚ·¢ÙÈο ÚˆÙfiÎÔÏÏ· ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› Ì Ôχ ηϿ ·ÔÙÂϤÛÌ·Ù· (ÂÈ‚›ˆÛË ÂχıÂÚË ÓfiÛÔ˘ 65-75%) (21-23). ∂›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈÎfi fiÙÈ Ù· ıÂÚ·¢ÙÈο ÚˆÙfiÎÔÏÏ· Â›Ó·È ÂÙÂÚÔÁÂÓ‹ Î·È ÂÔ̤ӈ˜ Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÍ·¯ıÔ‡Ó Û˘ÌÂÚ¿ÛÌ·Ù· Û¯ÂÙÈο Ì ÙÔÓ ÚfiÏÔ Î¿ı ʷÚ̷΢ÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ͯˆÚÈÛÙ¿. ¶Èı·ÓÒ˜ Ë ‰ÔÍÔÚÔ˘ÌÈΛÓË, Ë ‚ÈÓÎÚÈÛÙ›ÓË Î·È Ù· ÛÙÂÚÔÂȉ‹ Ó· ·ÔÙÂÏÔ‡Ó ÛËÌ·ÓÙÈο Ê¿Ú̷η ÛÙË ıÂÚ·›· (4). ∂›Û˘, Û ·ÓÙ›ıÂÛË Ì ٷ ¿ÏÏ· ª∏§, Ë ‰È·ÛÙڈ̿وÛË Ù˘ ıÂÚ·›·˜ ·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 Ï‹Úˆ˜ ¯ÂÈÚÔ˘ÚÁÈο ÂÍ·ÈÚ¤ÛÈÌÔ fiÁÎÔ, Ô˘ Ê·›ÓÂÙ·È Ó· ¯ÚÂÈ¿˙ÔÓÙ·È ÏÈÁfiÙÂÚË ıÂÚ·›·, Î·È Ë ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂÚ·›·˜ Û ·ÚÔ˘Û›· ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ (.¯. ÚÔÛ‚ÔÏ‹ ‰¤ÚÌ·ÙÔ˜, ÌÂÛÔıˆÚ·Î›Ô˘, ÛÏ¿¯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˘˜, fiˆ˜ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô, Â›‰· ΢ÙÔÎÈÓÒÓ, ÚÔÛ‰ÈÔÚÈÛÌfi ÙÔ˘ CD30 ÛÙÔ Ï¿ÛÌ·, ÌÂϤÙË ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ Î·È ÁÔÓȉȷÎÒÓ ÌÈÎÚÔÛ˘ÛÙÔȯÈÒÓ DNA, Ì ÛÙfi¯Ô ÙËÓ ·ÍÈÔÏfiÁËÛ‹ ÙÔ˘˜ ˆ˜ ‰È·ÁÓˆÛÙÈÎÔ‡˜ ‹ ÚÔÁÓˆÛÙÈÎÔ‡˜ ‰Â›ÎÙ˜ (24,25). ¶Úfi‰ÚÔÌ· ·ÔÙÂϤÛÌ·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¯ÂÙÈο 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˜ ÂÚÈÙÒÛÂȘ Á›ÓÂÙ·È Î·È ‰È¿ÛˆÛË Ì ·˘ÙfiÏÔÁË ‹ ·ÏÏÔÁÂÓ‹ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (4,27).

µ. Hodgkin §ÂÌÊÒÌ·Ù· (∏§) H ÓfiÛÔ˜ ÙÔ˘ Hodgkin ÂÚÈÁÚ¿ÊËΠÁÈ· ÚÒÙË ÊÔÚ¿ ·fi ÙÔÓ Thomas Hodgkin (1832). ∆Ô ¯·Ú·ÎÙËÚÈÛÙÈÎfi ·ıÔÁÓˆÌÔÓÈÎfi ·ÙÙ·ÚÔ Â›Ó·È ÙÔ ReedSternberg (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 ÂÓÂÚÁÔÔ›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˘ ÂÈ‚›ˆÛ˘ ÙˆÓ ÓÂÔÏ·ÛÌ·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ R-S. ™ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÌÂÙ·ÁÚ·ÊÈÎÒÓ ·˘ÙÒÓ ·Ú·ÁfiÓÙˆÓ Û˘ÌÌÂÙ¤¯Ô˘Ó ‰È¿ÊÔÚ˜ ΢ÙÙ·ÚÔΛÓ˜, fiˆ˜ Ë IL-13 Î·È Ô TNF-·, ÂÓÒ ˘¿Ú¯ÂÈ ·ÏÏËÏÂ›‰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›‰·85

85

§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿

¶›Ó·Î·˜ 1. ∏ ÈÛÙÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Hodgkin 1. Hodgkin ÎÏ·ÛÈÎfi ϤÌʈ̷ ñ √˙҉˘ ÛÎÏ‹Ú˘ÓÛË - √™ [nodular sclerosis (NS)] ñ §ÂÌÊÔÂÈÎÚ·Ù¤˜ - §∂ [lymphocyte rich classical Hodgkin’s lymphoma (LRCHD)] ñ §ÂÌÊÔÂÓÈÎfi - §¶ [lymphocyte depleted subtypes (LD)] ñ ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ - ª∫ [mixed cellularity (MC)] 2. √˙҉˜ §ÂÌÊÔÂÈÎÚ·Ù¤˜ - √§∂∏§ [nodular lymphocyte predominance type (¡LPHD)] 3. ∞Ù·ÍÈÓfiÌËÙ· [unclassifiable cases (UC)]

∏ ÚˆÙ½ÓË LMP1ÙÔ˘ ÈÔ‡ EB ÂÓÂÚÁÔÔÈ› ·¢ı›·˜ ÙÔÓ ÌÂÙ·ÁÚ·ÊÈÎfi ·Ú¿ÁÔÓÙ· NfkB (28-30). ∏ ÈÛÙÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË ÙˆÓ ∏§ Û‡Ìʈӷ Ì ÙËÓ International Lymphoma Study Group, REAL Î·È 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 ÏÂÌÊÔÂÈÎÚ·ÙÔ‡ÓÙÔ˜ Î·È ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ ¤¯Ô˘Ó ÙËÓ Î·Ï‡ÙÂÚË ÚfiÁÓˆÛË, ÂÓÒ Ù· ÏÂÌÊÔÂÓÈο ¤¯Ô˘Ó ÙË ¯ÂÈÚfiÙÂÚË ÚfiÁÓˆÛË. ∏ ‰È¿ÁÓˆÛË Ù›ıÂÙ·È Ì ‚ÈÔ„›· Ù˘ Ì¿˙·˜. ∞ÂÈÎÔÓÈÛÙÈΤ˜ ÂÍÂÙ¿ÛÂȘ Ô˘ Û˘ÓÂÈÎÔ˘ÚÔ‡Ó Â›Ó·È Ë ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜, ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ·, Ë ·ÍÔÓÈ΋ (CT) Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· (MRI), ÙÔ ÛÈÓıËÚÔÁÚ¿ÊËÌ· Ì Á¿ÏÏÈÔ Î·È Ë ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· (FDP-PET). H ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· Ù›ÓÂÈ Ó· ·ÓÙÈηٷÛÙ‹ÛÂÈ ÙÔ ÛÈÓıËÚÔÁÚ¿ÊËÌ· Ì Á¿ÏÏÈÔ, ηıÒ˜ ‰È·ı¤ÙÂÈ ˘„ËÏfiÙÂÚË ‰È·ÎÚÈÙÈ΋ ÈηÓfiÙËÙ· Î·È Â˘·ÈÛıËÛ›·, ¯·ÌËÏfiÙÂÚË ‰fiÛË ·ÎÙÈÓÔ‚ÔÏ›·˜ ÁÈ· ÙÔÓ ·ÛıÂÓ‹, ÌÈÎÚfiÙÂÚÔ ¯ÚfiÓÔ ÂͤٷÛ˘ Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ·Ó›¯Ó¢ÛË Ù˘ ÛÏËÓÈ΋˜ ÚÔÛ‚ÔÏ‹˜. ∏ ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÌÔÚ› 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 ÌÔÚ› Ó· ·ÓÙÈηٷÛÙ‹ÛÂÈ ÙË ‚ÈÔ„›· Ì˘ÂÏÔ‡. À¿Ú¯Ô˘Ó, fï˜, ÂÚÈÙÒÛÂȘ Ì ıÂÙÈÎfi PET Î·È ·ÚÓËÙÈ΋ ‚ÈÔ„›· Ì˘ÂÏÔ‡. ∆Ô PET ‰ÂÓ ÌÔÚ› Ó· ·ÓÙÈηٷÛÙ‹ÛÂÈ ÙË CT. √ Û˘Ó‰˘·ÛÌfi˜ ƒ∂∆/CT ¤¯ÂÈ ÌÂÁ·Ï‡ÙÂÚË ‰È·ÁÓˆÛÙÈ΋ ·Í›·. ¢ÂÓ ¯ÚÂÈ¿˙ÂÙ·È ÁÈ· ÙË ÛÙ·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘ ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, ηıÒ˜ ¤¯ÂÈ Ù¿ÛË ÁÈ· ˘ÂÚ-ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ·ÛıÂÓÒÓ (28,32-37). ∏ ÛÙ·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘ ηٿ Ann-Arbor Á›ÓÂÙ·È Û ٤ÛÛÂÚ· ÛÙ¿‰È· I-IV, ·Ó¿ÏÔÁ· Ì ÙËÓ ¤ÎÙ·ÛË Ù˘ ÓfiÛÔ˘, Î·È Î·ÙËÁÔÚÈÔÔÈÂ›Ù·È Û A Î·È µ ·Ó¿ÏÔÁ· Ì ÙËÓ ·ÚÔ˘Û›· ‹ fi¯È µ-Û˘ÌÙˆÌ¿ÙˆÓ (ηÎÔ˘¯›·, ˘ÚÂÙfi˜), Û à Û ÂÚÈÙÒÛÂȘ ÌÂÁ¿Ï˘ Ì¿˙·˜ (>10 cm), Û ∂ Û ·ÚÔ˘Û›· Â͈ÏÂÌÊ·‰ÂÓÈÎÒÓ Ì·˙ÒÓ Î·È Û S Û ÂÚÈÙÒÛÂȘ ÛÏËÓÈ΋˜ ÚÔÛ‚ÔÏ‹˜ (38). ∏ ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ ¤¯ÂÈ ‚ÂÏÙȈı› ÛËÌ·ÓÙÈο Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· Ì ÙË ¯Ú‹ÛË Û˘Ó‰˘·Ṳ̂Ó˘ ¯ËÌÂÈÔıÂÚ·›·˜ Î·È ·ÎÙÈÓÔıÂÚ·›·˜ Î·È ÙËÓ ÚÔ-

Û·ÚÌÔÁ‹ Ù˘ ıÂÚ·›·˜ ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘. ∆· Û˘Ó‹ıË ¯ËÌÂÈÔıÂÚ·¢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˜. ªÂÚÈο ·Ú·‰Â›ÁÌ·Ù· ›ӷÈ: ABVD (‰ÔÍÔÚÔ˘ÌÈΛÓË, ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÌÏ·ÛÙ›ÓË Óٷηڂ·˙›ÓË), OPPA±COPP Û ı‹Ï· (‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ˙fiÓË, ÚÔηڂ·˙›ÓË, ‰ÔÍÔÚÔ˘ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë, Ú‰ÓÈ˙fiÓË, ÚÔηڂ·˙›ÓË), OEPA±COPP Û ¿ÚÚÂÓ˜ (‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ˙fiÓË, ÂÙÔÔÛ›‰Ë, ‰ÔÍÔÚÔ˘ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë, Ú‰ÓÈ˙fiÓË, ÚÔηڂ·˙›ÓË), BEACOPP (ÌÏÂÔÌ˘Î›ÓË, ÂÙÔÔÛ›‰Ë, ‰ÔÍÔÚÔ˘ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë, ‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ˙fiÓË, ÚÔηڂ·˙›ÓË), DBVE-PC (‰ÔÍÔÚÔ˘ÌÈΛÓË, ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÎÚÈÛÙ›ÓË, ÂÙÔÔÛ›‰Ë, Ú‰ÓÈ˙fiÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë), MOPP/ABV (̯ψڷÈı·Ì›ÓË, ‚ÈÓÎÚÈÛÙ›ÓË, ÚÔηڂ·˙›ÓË, Ú‰ÓÈ˙fiÓË, ‰ÔÍÔÚÔ˘ÌÈΛÓË, ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÌÏ·ÛÙ›ÓË) (39). ∏ ıÂÚ·›· Á›ÓÂÙ·È ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ Î·È ÌÔÚ› Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Û˘Ó‰˘·ÛÌfi ·ÎÙÈÓÔıÂÚ·›·˜ ÚÔۂ‚ÏËÌ¤ÓˆÓ ‰›ˆÓ, Ë ‰Â ÂÈ‚›ˆÛË Â›Ó·È Ôχ ˘„ËÏ‹ Î·È ÔÈΛÏÏÂÈ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ (¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘: ÔÏÈ΋ ÂÈ‚›ˆÛË 96-100%, ÂӉȷ̤ÛÔ˘ Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘: 85-100%). ¶·ÚfiÏÔ Ô˘ Ë ÂÈ‚›ˆÛË Â›Ó·È Ôχ ηϋ, Ë ÙÔÍÈÎfiÙËÙ· ÙfiÛÔ ·fi ÙË ¯ËÌÂÈÔıÂÚ·›· fiÛÔ Î·È ·fi ÙËÓ ·ÎÙÈÓÔıÂÚ·›· ·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 ÂÙÔÔÛ›‰Ë ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ· ÂÌÊ¿ÓÈÛË ‰Â˘ÙÂÚÔ·ıÔ‡˜ Ï¢¯·ÈÌ›·˜, Ë ÌÏÂÔÌ˘Î›ÓË Ô‰ËÁ› Û ‰Â˘ÙÂÚÔ·ı‹ ›ÓˆÛË Î·È ÔÈ ·Óıڷ΢ÎϛӘ Û ηډÈÔÌ˘Ô¿ıÂÈ·. ∏ ·ÎÙÈÓÔıÂÚ·›· ¢ı‡ÓÂÙ·È ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Û˘Ì·ÁÒÓ fiÁΈÓ, Ï¢¯·ÈÌÈÒÓ Î·È Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎÒÓ Û˘Ó‰ÚfïÓ, ·ıËÚÔÛÎÏËÚ˘ÓÙÈ΋˜ ηډȷ΋˜ ÓfiÛÔ˘, ‰˘ÛÏÂÈÙÔ˘ÚÁÈÒÓ ÙˆÓ ‚·Ï‚›‰ˆÓ, Ó¢ÌÔÓÈ΋˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜, ÛÙ›ڈÛ˘, ˘ÔÁÔÓÈÌfiÙËÙ·˜ Î·È ˘Ôı˘ÚÂÔÂȉÈÛÌÔ‡/ Ca ı˘ÚÂÔÂȉԇ˜ (39). °È· ÙÔ ÏfiÁÔ ·˘Ùfi, Ù· Û‡Á¯ÚÔÓ· ıÂÚ·¢ÙÈο ÚˆÙfiÎÔÏÏ· ÚÔÛ·ıÔ‡Ó Ó· ÚÔÛ‰ÈÔÚ›ÛÔ˘Ó ÙË ‚¤ÏÙÈÛÙË ‰fiÛË ¯ËÌÂÈÔıÂÚ·›·˜ ηÈ/‹ ·ÎÙÈÓÔıÂÚ·›·˜ ‹ Ó· ·ÔʇÁÔ˘Ó ÙËÓ ·ÎÙÈÓÔıÂÚ·›· ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÏ·¯ÈÛÙÔÔÈ‹ÛÔ˘Ó ÙËÓ ÙÔÍÈÎfiÙËÙ·. ŒÙÛÈ, ÔÈ Nachman Î·È Û˘Ó. ¯ÔÚ‹ÁËÛ·Ó ¯ËÌÂÈÔıÂÚ·›· ·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 Ï‹ÚË ·ÓÙ·fiÎÚÈÛË ÛÙË ¯ËÌÂÈÔıÂÚ·›· Ô˘ ¤Ï·‚·Ó ·ÎÙÈÓÔıÂÚ·›· ·ÎÔÏÔ‡ıˆ˜ ‰ÂÓ Â›¯·Ó ηӤӷ ÏÂÔÓ¤ÎÙËÌ· ÛÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË. ¶·ÚÔÌÔ›ˆ˜, ÔÈ Rühl Î·È Û˘Ó. ‰ÂÓ ¶·È‰È·ÙÚÈ΋ 2009;72:1-9

Pediatri Mar-Apr 09

10-04-09

15:36

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86

µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹

‰È·›ÛÙˆÛ·Ó ˘ÂÚÔ¯‹ ÛÙËÓ ÂÈ‚›ˆÛË ÌÂÙ¿ ·fi Û˘ÌÏËڈ̷ÙÈ΋ ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔıÂÚ·›·˜ Û ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÛıÂÓ›˜ (41). ŸÌˆ˜, ÛÙÔ˘˜ ·ÛıÂÓ›˜ ÂӉȷ̤ÛÔ˘ Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, Ë ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔıÂÚ·›·˜ ÚÔۂ‚ÏË̤ÓÔ˘ ‰›Ô˘ Û˘ÌÏËڈ̷ÙÈο ÌÂÙ¿ ÙË ¯ÔÚ‹ÁËÛË ¯ËÌÂÈÔıÂÚ·›·˜ ‚ÂÏÙ›ˆÛ ÛËÌ·ÓÙÈο ÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂÓÒÓ. ∞ÓÙ›ıÂÙ·, Ì›· ÈÔ ÚfiÛÊ·ÙË ÌÂϤÙË ·Ó·Ê¤ÚÂÈ ·ÚfiÌÔÈ· ÂÈ‚›ˆÛË ÙfiÛÔ ÛÙÔ˘˜ ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡ fiÛÔ Î·È ÛÙÔ˘˜ ·ÛıÂÓ›˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (42). ∞fi ÙȘ ÌÂϤÙ˜ ·˘Ù¤˜ ÚÔ·ÙÂÈ fiÙÈ Ë ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔıÂÚ·›·˜ ÚÔۂ‚ÏË̤ÓÔ˘ ‰›Ô˘ ÌÂÙ¿ ·fi ¯ËÌÂÈÔıÂÚ·›·, Ô˘ ÚÔÛ·ÚÌfi˙ÂÙ·È ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘, ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÂÈ‚›ˆÛË ÂχıÂÚË Û˘Ì‚·Ì¿ÙˆÓ, fi¯È fï˜ Î·È ÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË. ∏ ‰Â ¯ÔÚ‹ÁËÛË ÈÛ¯˘ÚfiÙÂÚ˘ ¯ËÌÂÈÔıÂÚ·›·˜ ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔÊ¢¯ı› Ë ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔıÂÚ·›·˜ ‰ÂÓ ÛËÌ·›ÓÂÈ fiÙÈ ¤¯ÂÈ Î·È ÏÈÁfiÙÂÚË ÙÔÍÈÎfiÙËÙ·. ¶¿ÓÙˆ˜, Ë ¯ÔÚ‹ÁËÛË ¯·ÌËÏfiÙÂÚ˘ ‰fiÛ˘ ¯ËÌÂÈÔıÂÚ·›·˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·ÎÙÈÓÔıÂÚ·›· ÚÔۂ‚ÏË̤ÓÔ˘ ‰›Ô˘ ¤¯ÂÈ ·ÚfiÌÔÈ· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ì ÙË ¯ÔÚ‹ÁËÛË ÌfiÓÔ ¯ËÌÂÈÔıÂÚ·›·˜ ˘„ËÏfiÙÂÚ˘ ‰fiÛ˘, ·ÏÏ¿ ·ÚÔ˘ÛÈ¿˙ÂÈ ÏÈÁfiÙÂÚË ÙÔÍÈÎfiÙËÙ·. πÛÙÔÚÈο, Ô Û¯Â‰È·ÛÌfi˜ Ù˘ ¯ËÌÂÈÔıÂÚ·›·˜ ·Ï·ÈfiÙÂÚ· ÂÚÈÂÏ¿Ì‚·Ó ¢ڇÙÂÚ· ‰›· ·ÎÙÈÓÔ‚fiÏËÛ˘ Ù‡Ô˘ Ì·Ó‰‡·, Ô˘ ¤¯Ô˘Ó ηٷÚÁËı› Î·È ·ÓÙÈηٷÛÙ·ı› ·fi ·ÎÙÈÓÔ‚fiÏËÛË ÚÔۂ‚ÏË̤ÓÔ˘ ‰›Ô˘. ª¿ÏÈÛÙ·, Ë Û‡Á¯ÚÔÓË Ù¿ÛË ÙˆÓ ·ÎÙÈÓÔıÂÚ·¢ÙÒÓ Â›Ó·È Ó· ·ÎÙÈÓÔ‚ÔÏÔ‡Ó ÌfiÓÔ ÙÔ˘˜ ÚÔۂ‚ÏË̤ÓÔ˘˜ ÏÂÌÊ·‰¤Ó˜, Ì ·ÔÙ¤ÏÂÛÌ· Ë ‰fiÛË ·ÎÙÈÓÔ‚fiÏËÛ˘ ÛÙÔ˘˜ Ê˘ÛÈÔÏÔÁÈÎÔ‡˜ ÈÛÙÔ‡˜ Ó· Â›Ó·È ÂÚÈÔÚÈṲ̂ÓË (43). ∏ Û‡Á¯ÚÔÓË Ù¿ÛË ÙˆÓ ÓÂfiÙÂÚˆÓ ıÂÚ·¢ÙÈÎÒÓ ÚˆÙÔÎfiÏÏˆÓ Â›Ó·È Ë ÚÔÛ·ÚÌÔÁ‹ Ù˘ ıÂÚ·›·˜ ·Ó¿ÏÔÁ· Ì ÙËÓ Ù·¯Â›· ‹ ‚Ú·‰Â›· ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂÚ·›·. ™Ùfi¯Ô˜ Â›Ó·È Ë ·Ó·ÁÓÒÚÈÛË ·ÛıÂÓÒÓ Ì ¢ÓÔ˚ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Î·È Ë ÂÏ¿ÙÙˆÛË Ù˘ ¤ÓÙ·Û˘ Ù˘ ıÂÚ·›·˜ Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ª›· ·fi ÙȘ ÚÒÙ˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÂÈ Î·Ï¿ ·ÔÙÂϤÛÌ·Ù· Û ·È‰È¿ Ì ÂÓÙÔÈṲ̂ÓÔ ∏§ Ô˘ ··ÓÙÔ‡Ó Î·Ï¿ ÛÙË ¯ËÌÂÈÔıÂÚ·›·, Ì ¯ÔÚ‹ÁËÛË ¯ËÌÂÈÔıÂÚ·›·˜ ¯ˆÚ›˜ ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜ Î·È ·Óıڷ΢ÎϛӘ Î·È ·ÎÔÏÔ‡ıˆ˜ ¯·ÌËÏ‹ ‰fiÛË ·ÎÙÈÓÔıÂÚ·›·˜ (44). ¶·ÚÔÌÔ›ˆ˜, Ì›· ¿ÏÏË ÌÂϤÙË ÚÔÛ¿ıËÛ ӷ ÂÍ·ÙÔÌÈ·ÛÂÈ ÙË ıÂÚ·›· ·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 ¯ÚËÛÈÌÔÔÈËı› ÌÈ· ̤ıÔ‰Ô˜ ÂÎÙÂٷ̤Ó˘ ÌÂϤÙ˘ ÚˆÙÂ˚ÓÈÎÒÓ ‰ÂÈÎÙÒÓ ÛÙÔ Paediatriki 2009;72:1-9

·›Ì· (surface enhanced laser desorption/ionization, SELDI), Û ÌÈ· ÚÔÛ¿ıÂÈ· ·Ó·ÁÓÒÚÈÛ˘ ÌÈ·˜ ÚˆÙˆÌÈ΋˜ ˘ÔÁÚ·Ê‹˜ Ô˘ ‰È·¯ˆÚ›˙ÂÈ Ù· ‰È¿ÊÔÚ· ÎÏÈÓÈο ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘ (47). ∏ ÌÂϤÙË ÙˆÓ ÁÔÓȉȷÎÒÓ ˘ÔÁÚ·ÊÒÓ Û 29 ·ÛıÂÓ›˜ Ì ÎÏ·ÛÛÈÎfi ∏§ ·Ó¤‰ÂÈÍÂ Û˘ÁÎÂÎÚÈ̤ӷ ÁÔÓ›‰È· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ·¿ÓÙËÛË ÛÙË ıÂÚ·›· (48). ªÂÏÏÔÓÙÈο, Ë ·Ó·ÁÓÒÚÈÛË ‚ÈÔÏÔÁÈÎÒÓ ‰ÂÈÎÙÒÓ ÂÓ‰¤¯ÂÙ·È Ó· ·ÔÙÂϤÛÂÈ ÛËÌ·ÓÙÈÎfi ÂÚÁ·ÏÂ›Ô ÁÈ· ÙËÓ ¤ÁηÈÚË ÚfiÁÓˆÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂÚ·›·, ÙËÓ ·Ó›¯Ó¢ÛË ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ Î·È ÙËÓ Úfi‚ÏÂ„Ë Èı·ÓÒÓ ÌÂÏÏÔÓÙÈÎÒÓ ˘ÔÙÚÔÒÓ. ∆Ô √§∂∏§ Â›Ó·È Í¯ˆÚÈÛÙ‹ ÔÓÙfiÙËÙ· ·fi ÙÔ ÎÏ·ÛÈÎfi ∏§, Ì ‰È·ÊÔÚÂÙÈο ÈÛÙÔ·ıÔÏÔÁÈο Î·È ·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‰ÈÎÔÔÈÔ‡Ó ¤Ó˙˘Ì· ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙˆÓ Ê·Ú̿ΈÓ, fiˆ˜ ÙÔ Î˘Ùfi¯ÚˆÌ· P450, Î·È ·ÔÙÔ͛ӈÛ˘ ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡, fiˆ˜ 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 ıÂÚ·›·. ∆Ú¤¯Ô˘Û˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ÂÈÛ·Á¿ÁÂÈ ÙËÓ ÂÊ·ÚÌÔÁ‹ Ê·ÚÌ·ÎÔÁÂÓˆÌÈÎÒÓ ÌÂıfi‰ˆÓ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ·Ó·ÁÓˆÚ›ÛÔ˘Ó ‚ÈÔÏÔÁÈÎÔ‡˜ ‰Â›ÎÙ˜ Ô˘ ı· ÂÈÙÚ¤„Ô˘Ó ÌÂÏÏÔÓÙÈο ÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ηٿÏÏËÏÔ˘ ıÂÚ·¢ÙÈÎÔ‡ Û¯‹Ì·ÙÔ˜ Î·È ı· ÂÏ·ÙÙÒÛÔ˘Ó ÙËÓ ÂÌÊ¿ÓÈÛË ÂÈÏÔÎÒÓ (50). ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÙˆÓ ·È‰ÈÒÓ Ì ÏÂÌÊÒÌ·Ù· Â›Ó·È Ôχ ηϋ, fï˜ οÔÈ· ·È‰È¿ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ˘ÔÙÚÔÈ¿˙Ô˘Ó ‹ Ó· ÂÌÊ·Ó›˙Ô˘Ó ·ÒÙÂÚ˜ ÂÈÏÔΤ˜. √È ÓÂfiÙÂÚ˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô ÙËÓ ·Ó·ÁÓÒÚÈÛË ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ÙËÓ ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂÚ·¢ÙÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ Î·È ÙËÓ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· οı ·ÛıÂÓÔ‡˜, ¤ÙÛÈ ÒÛÙ ӷ ‚ÂÏÙȈı› Ë ÂÈ‚›ˆÛË, Ì ٷ˘Ùfi¯ÚÔÓË ÂÏ¿ÙÙˆÛË Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ÙÔÍÈÎfiÙËÙ·˜ Ù˘ ıÂÚ·›·˜. ∏ ·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¿ÏÏËÏ·, Ë ·Ó¿Ù˘ÍË Â˘·›ÛıËÙˆÓ ÌÂıfi‰ˆÓ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂÚ·›· Î·È Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ Ù˘ ˘ÔÙÚÔ‹˜ Î·È Ë ·Ó›¯Ó¢ÛË ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ ı· ‚ÔËı‹ÛÔ˘Ó ÛÙËÓ ÚÒÈÌË ·Ó·ÁÓÒÚÈÛË Ù˘ ¯ËÌÂÈÔ-¢·ÈÛıËÛ›·˜ ‹ ·ÓıÂÎÙÈÎfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Î·È ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ηٷÏÏËÏfiÙÂÚÔ˘ ıÂÚ·¢ÙÈÎÔ‡ Û¯‹Ì·ÙÔ˜. ∏ ¯Ú‹ÛË Û‡Á¯ÚÔÓˆÓ ‚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

Pediatri Mar-Apr 09

10-04-09

15:36

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87

§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿

Ì˯·ÓÈÛÌÒÓ, ÛÙËÓ Ù·ÍÈÓfiÌËÛË ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Î·È ÛÙË ‚ÂÏÙÈÛÙÔÔ›ËÛË ÙˆÓ ıÂÚ·¢ÙÈÎÒÓ ÌÂıfi‰ˆÓ.

∂˘¯·ÚÈÛٛ˜ ∂˘¯·ÚÈÛÙԇ̠ȉȷÈÙ¤Úˆ˜ ÙÔÓ ∂›ÎÔ˘ÚÔ ∫·ıËÁËÙ‹ ¶·È‰È·ÙÚÈ΋˜ ÙÔ˘ ¢ËÌÔÎÚ›ÙÂÈÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ £Ú¿Î˘ Î. ∂ÏȉÔÊfiÚÔ ª·ÓÙ·‰¿ÎË ÁÈ· ÙȘ ÏÂÙÔÌÂÚ›˜ ÏËÚÔÊÔڛ˜ Ô˘ Ì·˜ ¤‰ˆÛ ۯÂÙÈο Ì ٷ ÙÚ¤¯ÔÓÙ· ·ÌÂÚÈηÓÈο ıÂÚ·¢ÙÈο ÚˆÙfiÎÔÏÏ·.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Sandlund JT, Downing JR, Crist WM. Non-Hodgkin's lymphoma in childhood. N Engl J Med 1996;334:1238-1248.

2. Anagnostopoulos I. Individualized target therapy of malignant lymphomas: an outlook. Recent Results Cancer Res 2007;176:177-188.

3. Cairo MS, Raetz E, Lim MS, Davenport V, Perkins SL. Childhood and adolescent non-Hodgkin lymphoma: new insights in biology and critical challenges for the future.

Pediatr Blood Cancer 2005;45:753-769.

4. Reiter A. Diagnosis and treatment of childhood nonHodgkin lymphoma. Hematology Am Soc Hematol Educ Program 2007;2007:285-296.

5. Reiter A, Schrappe M, Tiemann M, Ludwig WD, Yakisan E, Zimmermann M et al. Improved treatment results in childhood B-cell neoplasms with tailored intensification of therapy: A report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90. Blood 1999;94:3294-3306.

6. Seidemann K, Zimmermann M, Book M, Meyer U, Burkhardt B, Welte K, et al. Tumor necrosis factor and lymphotoxin alfa genetic polymorphisms and outcome in pediatric patients with non-Hodgkin's lymphoma: results from Berlin-Frankfurt-Münster Trial NHL-BFM 95. Clin Oncol 2005;23:8414-8421.

7. National Cancer Institute, U.S. National Institutes of Health [Webpage, Internet]. http://www.cancer.gov/cancertopics/ pdq/treatment/child-non-hodgkins/ healthprofessional.

8. Raetz E, Perkins S, Davenport V, Cairo MS. B large-cell lymphoma in children and adolescents. Cancer Treat Rev 2003;29:91-98.

9. Heerema NA, Bernheim A, Lim MS, Look AT, Pasqualucci L, Raetz E, et al. State of the Art and Future Needs in Cytogenetic/Molecular Genetics/Arrays in childhood lymphoma: summary report of workshop at the First International Symposium on childhood and adolescent non-Hodgkin lymphoma, April 9, 2003, New York City, NY. Pediatr Blood Cancer 2005;45:616-622.

10. Patte C. Treatment of mature B-ALL and high grade B-NHL in children. Best Pract Res Clin Haematol 2002;15: 695-711.

11. Cairo MS, Gerrard M, Sposto R, Auperin A, Pinkerton CR, Michon J, et al. FAB LMB96 International Study Committee.

Results of a randomized international study of high-risk central nervous system B non-Hodgkin lymphoma and B acute lymphoblastic leukemia in children and adolescents.

Blood 2007;109:2736-2743.

12. Patte C, Auperin A, Gerrard M, Michon J, Pinkerton R, Sposto R, et al. FAB/LMB96 International Study Committee.

Results of the randomized international FAB/LMB96 trial for intermediate risk B-cell non-Hodgkin lymphoma in children and adolescents: it is possible to reduce treatment for the early responding patients. Blood 2007;109:2773-2780.

13. Woessmann W, Seidemann K, Mann G, Zimmermann M, Burkhardt B, Oschlies I, et al. BFM Group. The impact of the methotrexate administration schedule and dose in the treatment of children and adolescents with B-cell neoplasms: a report of the BFM Group Study NHLBFM95. Blood 2005;105:948-958.

14. Patte C, Auperin A, Michon J, Behrendt H, Leverger G, Frappaz D, et al. The Société Française d'Oncologie Pédiatrique LMB89 protocol: highly effective multiagent chemotherapy tailored to the tumor burden and initial response in 561 unselected children with B-cell lymphomas and L3 leukemia. Blood 2001;97:3370-3379.

15. Alizadeh AA, Eisen MB, Davis RE, Ma C, Lossos IS, Rosenwald A, et al. Distinct types of diffuse large B-cell lymphoma identified by gene expression profiling. Nature 2000;403:503-511.

16. Rosenwald A, Wright G, Chan WC, Connors JM, Campo E, Fisher RI, et al. The use of molecular profiling to predict survival after chemotherapy for diffuse large-B-cell lymphoma. N Engl J Med 2002;346:1937-1947.

17. Oschlies I, Klapper W, Zimmermann M, Krams M, Wacker HH, Burkhardt B, et al. Diffuse large B-cell lymphoma in pediatric patients belongs predominantly to the germinal-center type B-cell lymphomas: a clinicopathologic analysis of cases included in the German BFM (Berlin-Frankfurt-Munster) Multicenter Trial. Blood 2006;107:4047-4052.

18. Cooney-Qualter E, Krailo M, Angiolillo A, Fawwaz RA, Wiseman G, Harrison L, et al. Children's Oncology Group.

A phase I study of 90yttrium-ibritumomab-tiuxetan in children and adolescents with relapsed/refractory CD20positive non-Hodgkin's lymphoma: a Children's Oncology Group study. Clin Cancer Res. 2007;13:5652s-5660s.

19. Fujita N, Mori T, Mitsui T, Inada H, Horibe K, Tsurusawa M; for the Lymphoma Committee of the Japanese Pediatric Leukemia/Lymphoma Study Group. The role of hematopoietic stem cell transplantation with relapsed or primary refractory childhood B-cell non-Hodgkin lymphoma and mature B-cell leukemia: A retrospective analysis of enrolled cases in Japan. Pediatr Blood Cancer.

2008 Apr 21. [Epub ahead of print].

20. Attarbaschi A, Dworzak M, Steiner M, Urban C, Fink FM, Reiter A, et al. Outcome of children with primary resistant or relapsed non-Hodgkin lymphoma and mature B-cell leukemia after intensive first-line treatment: a populationbased analysis of the Austrian Cooperative Study Group.

Pediatr Blood Cancer 2005;44:70-76.

21. Seidemann K, Tiemann M, Schrappe M, Yakisan E, Simonitsch I, Janka-Schaub G, Dörffel W, Zimmermann M, Mann G, Gadner H, Parwaresch R, Riehm H, Reiter A. Short-pulse B-non-Hodgkin lymphoma-type chemotherapy is efficacious treatment for pediatric anaplastic large cell lymphoma: a report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90. Blood 2001;97:3699-3706.

22. Brugières L, Deley MC, Pacquement H, Meguerian-Bedoyan Z, Terrier-Lacombe MJ, Robert A, Pondarré C, Leverger G, Devalck C, Rodary C, Delsol G, Hartmann O. CD30(+) anaplastic large-cell lymphoma in children: analysis of 82 patients enrolled in two consecutive studies of the French Society of Pediatric Oncology. Blood 1998;92:3591-3598.

23. Laver JH, Kraveka JM, Hutchison RE, Chang M, Kepner J, Schwenn M, Tarbell N, Desai S, Weitzman S, Weinstein ¶·È‰È·ÙÚÈ΋ 2009;72:1-9

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·88

88

µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹

HJ, Murphy SB. Advanced-stage large-cell lymphoma in children and adolescents: results of a randomized trial incorporating intermediate-dose methotrexate and highdose cytarabine in the maintenance phase of the APO regimen: a Pediatric Oncology Group phase III trial. J Clin Oncol 2005;23:541-547.

24. Nadali G, Vinante F, Stein H, Todeschini G, Tecchio C, Morosato L, et al. Serum levels of the soluble form of CD30 molecule as a tumor marker in CD30+ anaplastic large-cell lymphoma. J Clin Oncol 1995;13:1355-1360.

25. Franchina M, Woo AJ, Dods J, Karimi M, Ho D, Watanabe T, et al. The CD30 gene promoter microsatellite binds transcription factor Yin Yang 1 (YY1) and shows genetic instability in anaplastic large cell lymphoma. J Pathol 2008;214:65-74.

26. Wellmann A, Thieblemont C, Pittaluga S, Sakai A, Jaffe ES, Siebert P, et al. Detection of differentially expressed genes in lymphomas using cDNA arrays: identification of clusterin as a new diagnostic marker for anaplastic largecell lymphomas. Blood 2000;96:398-404.

27. Brugières L, Quartier P, Le Deley MC, Pacquement H, Perel Y, Bergeron C, et al. Relapses of childhood anaplastic large-cell lymphoma: treatment results in a series of 41 children - a report from the French Society of Pediatric Oncology. Ann Oncol 2000;11:53-58.

28. Hodgson DC, Hudson MM, Constine LS. Pediatric hodgkin lymphoma: maximizing efficacy and minimizing toxicity. Semin Radiat Oncol 2007;17:230-242.

29. Skinnider BF, Mak TW. The role of cytokines in classical Hodgkin lymphoma. Blood 2002;99:4283-4297.

30. Re D, Küppers R, Diehl V. Molecular pathogenesis of Hodgkin's lymphoma. J Clin Oncol 2005;23:6379-6386.

31. Harris NL, Jaffe ES, Diebold J, Flandrin G, MullerHermelink HK, Vardiman J, et al. The World Health Organization classification of neoplasms of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting - Airlie House, Virginia, November, 1997. Hematol J 2000;1:53-66.

32. Bar-Shalom R. Gallium SPECT/CT in lymphoma: the ups and downs of functional imaging. Eur J Nucl Med Mol Imaging 2005;32:1247-1249.

33. Kaste SC, Howard SC, McCarville EB, Krasin MJ, Kogos PG, Hudson MM. 18F-FDG-avid sites mimicking active disease in pediatric Hodgkin's. Pediatr Radiol 2005;35:141-154.

34. Furth C, Denecke T, Steffen I, Ruf J, Voelker T, Misch D, et al. Correlative imaging strategies implementing CT, MRI, and PET for staging of childhood Hodgkin disease. J Pediatr Hematol Oncol 2006;28:501-512.

35. Hutchings M, Loft A, Hansen M, Berthelsen AK, Specht L. Clinical impact of FDG-PET/CT in the planning of radiotherapy for early-stage Hodgkin lymphoma. Eur J Haematol 2007;78:206-212.

36. Montravers F, McNamara D, Landman-Parker J, Grahek D, Kerrou K, Younsi N, et al. [(18)F]FDG in childhood lymphoma: clinical utility and impact on management.

Eur J Nucl Med Mol Imaging 2002;29:1155-1165.

37. Menzel C, Döbert N, Mitrou P, Mose S, Diehl M, Berner U, Grünwald F. Positron emission tomography for the staging of Hodgkin's lymphoma - increasing the body of evidence in favor of the method. Acta Oncol. 2002;41:430-436.

38. Prosnitz LR. The Ann Arbor staging system for Hodgkin's

Paediatriki 2009;72:1-9

disease: does E stand for error? Int J Radiat Oncol Biol Phys 1977;2:1039.

39. Schwartz CL. The management of Hodgkin disease in the young child. Curr Opin Pediatr 2003;15:10-16.

40. Nachman JB, Sposto R, Herzog P, Gilchrist GS, Wolden SL, Thomson J, et al; Children's Cancer Group.

Randomized comparison of low-dose involved-field radiotherapy and no radiotherapy for children with Hodgkin's disease who achieve a complete response to chemotherapy. J Clin Oncol 2002;20:3765-3771.

41. Rühl U, Albrecht M, Dieckmann K, Lüders H, Marciniak H, Schellenberg D, et al. Response-adapted radiotherapy in the treatment of pediatric Hodgkin's disease: an interim report at 5 years of the German GPOH-HD 95 trial. Int J Radiat Oncol Biol Phys 2001;51:1209-1218.

42. Kung FH, Schwartz CL, Ferree CR, London WB, Ternberg JL, Behm FG, et al; Children's Oncology Group. POG 8625: a randomized trial comparing chemotherapy with chemoradiotherapy for children and adolescents with Stages I, IIA, IIIA1 Hodgkin Disease: a report from the Children’s Oncology Group. J Pediatr Hematol Oncol 2006;28:362-368.

43. Girinsky T, van der Maazen R, Specht L, Aleman B, Poortmans P, Lievens Y, et al. Involved-node radiotherapy (INRT) in patients with early Hodgkin lymphoma: concepts and guidelines. Radiother Oncol 2006;79:270-277.

44. Landman-Parker J, Pacquement H, Leblanc T, Habrand JL, Terrier-Lacombe MJ, Bertrand Y, et al. Localized childhood Hodgkin's disease: response-adapted chemotherapy with etoposide, bleomycin, vinblastine, and prednisone before low-dose radiation therapy-results of the French Society of Pediatric Oncology Study MDH90. J Clin Oncol 2000;18:1500-1507.

45. Schwartz CL, Constine LS, London W, Marcus R, Kamps W, Hutchinson R, et al. POG-9425: response-based, intensively timed therapy for intermediate/high stage (IS/HS) pediatric Hodgkin’s disease. Proc Am Soc Clin Oncol 2002;21:abstr 1555.

46. Hudson MM, Krasin M, Link MP, Donaldson SS, Billups C, Merchant TE, et al. Risk-adapted, combined-modality therapy with VAMP/COP and response-based, involvedfield radiation for unfavorable pediatric Hodgkin's disease.

J Clin Oncol 2004;22:4541-4550.

47. Qi L, Cazares L, Johnson C, de Alarcon P, Kupfer GM, Semmes OJ. Serum protein expression profiling in pediatric Hodgkin lymphoma: A report from the Children's Oncology Group. Pediatr Blood Cancer. 2008.

[Epub ahead of print].

48. Saãnchez-Aguilera A, Montalbaãn C, de la Cueva P, SaãnchezVerde L, Morente MM, Garc›a-Cos›o M, et al. Tumor microenvironment and mitotic checkpoint are key factors in the outcome of classic Hodgkin lymphoma. Blood.

2006;108:662-668.

49. Cincinati Children’s Hospital Medical Center [Webpage, Internet]. http://www.cincinnatichildrens.org/svc/alpha/c/ cancer/trials/hodgkins/ahod03p1-pf.htm.

50. Kelly KM, Perentesis JP; Children's Oncology Group.

Polymorphisms of drug metabolizing enzymes and markers of genotoxicity to identify patients with Hodgkin's lymphoma at risk of treatment-related complications. Ann Oncol. 2002;13(Suppl 1):34-39.

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∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË Ã. ∫Ô˘ÙÛ·˘Ù›ÎË, ∂. ª¿ÓÙ˙ÈÔ˘, ¡. ª˘ÚÈÔÎÂÊ·ÏÈÙ¿Î˘ ¶ÂÚ›ÏË„Ë: ∏ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË ·ÔÙÂÏ› Û¿ÓÈÔ Û‡Ó‰ÚÔÌÔ, fiÔ˘ ÙÔ ÚÔÂÍ¿Ú¯ÔÓ ¯·Ú·ÎÙËÚÈÛÙÈÎfi Â›Ó·È Ë ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÌÔÓÔ˘ÚËÓÈÎÔ‡ Ê·ÁÔ΢ÙÙ·ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ Î·ÏÔ‹ıË ÁÂÓÈÎÂ˘Ì¤ÓË ÈÛÙÈÔ΢ÙÙ·ÚÈ΋ ‰È‹ıËÛË ‰È·ÊfiÚˆÓ ÔÚÁ¿ÓˆÓ Ì ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË. ∆· Û˘ÌÙÒÌ·Ù· Ô˘ ·Ú·¤ÌÔ˘Ó ÛÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ Â›Ó·È Ô Â›ÌÔÓÔ˜ ˘ÚÂÙfi˜ (100%), ÔÈ Î˘ÙÙ·ÚÔÂӛ˜ (95%), Ë Ë·ÙÈ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· (96%), Ë Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›· (84%), Ô ›ÎÙÂÚÔ˜ (55%), Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· (43%), ÔÈ ‰È·Ù·Ú·¯¤˜ Ù˘ ‹Í˘ (75%) Î·È ÙÔ˘ ∫¡™ (32%) Î·È Ë ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË Ì ·ÈÌÔÊ·ÁÔ·ÙÙ·Ú· ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Î·È Û fiÚÁ·Ó· ÙÔ˘ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Û fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ∆· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ˘ÂÚÊÏÂÁÌÔÓÒ‰Ô˘˜ ‰È·‰Èηۛ·˜ Î·È Û˘Ó›ÛÙ·ÓÙ·È Û ·‡ÍËÛË ÛÙÔ ·›Ì· ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ, Ù˘ ÊÂÚÚÈÙ›Ó˘, Ù˘ Á·Ï·ÎÙÈ΋˜ ·Ê˘‰ÚÔÁoÓ¿Û˘, ÙˆÓ ÙÚÈÁÏ˘ÎÂÚȉ›ˆÓ, ÙˆÓ ÙÚ·ÓÛ·ÌÈÓ·ÛÒÓ Î·È Ù˘ ¯ÔÏÂÚ˘ıÚ›Ó˘, ηıÒ˜ Î·È Û ÂÏ¿ÙÙˆÛË ÙÔ˘ ÈÓˆ‰ÔÁfiÓÔ˘. ¢È·ÎÚ›ÓÂÙ·È Û ÔÈÎÔÁÂÓ¤˜ ‹ ÚˆÙÔ·ı¤˜ Î·È Û ‰Â˘ÙÂÚÔ·ı¤˜, Ô˘ ‰È·ÎÚ›ÓÔÓÙ·È ‰‡ÛÎÔÏ· ÌÂٷ͇ ÙÔ˘˜ ÂÂȉ‹ Î·È ÛÙȘ ‰‡Ô ÌÔÚʤ˜ ÌÔÚÔ‡Ó Ó· ·Ó¢ÚÂıÔ‡Ó ÔÈ ›‰È˜ ÌÂÙ·ÏÏ¿ÍÂȘ, ÂÓÒ ¤¯Ô˘Ó ·ÚfiÌÔÈ· ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Î·È ÚfiÁÓˆÛË. ∂›Û˘, ·ÊÔÚÌ‹ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Î·È ÙˆÓ ‰‡Ô ÌÔÚ› Ó· ·ÔÙÂÏ› οÔÈ· Ïԛ̈ÍË. ™˘¯Ó¿ Ë ‰È¿ÁÓˆÛË ‰È·Ê‡ÁÂÈ, ηıÒ˜ Ë ÓfiÛÔ˜ ÌÈÌÂ›Ù·È ÔÈΛϘ ÓÔÛÔÏÔÁÈΤ˜ ÔÓÙfiÙËÙ˜, οÙÈ Ô˘ ÌÔÚ› Ó· ·Ԃ› ÌÔÈÚ·›Ô, ·ÊÔ‡ ¯ˆÚ›˜ ıÂÚ·›· Â›Ó·È Ôχ Èı·Ó‹ Ë Î·Ù¿ÏËÍË ÙÔ˘ ·ÛıÂÓÔ‡˜, ÂÓÒ Ì ÙËÓ Î·Ù¿ÏÏËÏË ·ÁˆÁ‹ ηÈ, ΢ڛˆ˜, Ì ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ¤¯ÂÈ ÂÈÙ¢¯ı› ›·ÛË Û ˘„ËÏ¿ ÔÛÔÛÙ¿.

∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ ¶ÂÓÙ¤Ï˘ AÏÏËÏÔÁÚ·Ê›·: ÃÚ˘Û‹ ∫Ô˘ÙÛ·˘Ù›ÎË kochrysa@yahoo.com ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ ¶ÂÓÙ¤Ï˘, 152 36, ¶. ¶ÂÓÙ¤ÏË

§¤ÍÂȘ ÎÏÂȉȿ: ∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË, ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ, ·È‰È¿.

Haemophagocytic lymphohistiocytosis - HLH C. Koutsaftiki, E. Mantziou, N. Myriokefalitakis Abstract: Haemophagocytic lymphÔhistiocytosis is a rare syndrome characterized by a reactive disorder of the mononuclear phagocytic system which gives rise to benign, generalized histiocytic proliferation in many organs, with marked haemophagocytosis. The symptoms and signs that are associated with the diagnosis, along with their frequency of appearance, are: persistent fever (100%), cytopenia (96%), liver dysfunction (96%), hepatosplenomegaly (84%), jaundice (55%), lymphadenopathy (43%), coagulopathy (75%) and central nervous system symptoms (32%). Lymphohistiocytosis, with the presence of haemophagocytes in the bone marrow and the reticuloendothelial system, is observed in all cases. The laboratory findings that are usually present are due to the associated hyperinflammatory process and usually consist of hypercytokinaemia, hyperferritinaemia, elevated blood levels of lactate dehydrogenase, hypertriglyceridaemia, hypofibrinogenaemia, transaminasaemia, hyperbilirubinaemia, etc. Two types of haemophagocytic lymphÔhistiocytosis have been identified, the familial or primary syndrome and the secondary, but there is difficulty in distinguishing between them, as similar mutations and 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 to be delayed, because the syndrome can mimic a variety of diseases. Delay in diagnosis can be fatal, since prompt treatment, specifically bone marrow transplantation, can achieve a cure in most cases.

1st Department of Paediatrics, General Children’s Hospital of Penteli, Athens, Greece Correspondence: Chryssie Koutsaftiki kochrysa@yahoo.com 1st Department of Paediatrics, General Children’s Hospital of Penteli, Palaia Penteli 152 36, Athens, Greece

Key words: ∏aemophagocytic lymphÔhistiocytosis, haemophagocytic syndrome, children.

∂ÈÛ·ÁˆÁ‹ - √ÚÈÛÌÔ› ∏ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË ‹ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ (∞º™) ·ÓÙÈÚÔÛˆ‡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, Ì ÎÔÈÓ¿ ÎÏÈÓÈο Î·È ·ıÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο:

ÙÔ ÚˆÙÔ·ı¤˜ ‹ ÔÈÎÔÁÂÓ¤˜ ∞º™ (Familial Haemophagocytic Lymphoistiocytosis, FHL) Î·È ÙÔ ‰Â˘ÙÂÚÔ·ı¤˜ ∞º™ (secondary Haemophagocytic Lymphoistiocytosis, HLH) (1). ∏ ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ·ÔÙÂÏ› ·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎfi ‡ÚËÌ·, fiÔ˘ ÂÓÂÚÁÔÔÈË̤ӷ 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). ¶·È‰È·ÙÚÈ΋ 2009;72:89-000

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Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

∆Ô ∏LH, ÚˆÙÔ·ı¤˜ Î·È ‰Â˘ÙÂÚÔ·ı¤˜, ÌÔÚ› 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 ·ÓÙÈÌÂÙˆÈÛÙ› (3).

™˘¯ÓfiÙËÙ· ∏ Û˘¯ÓfiÙËÙ· Ù˘ ÓfiÛÔ˘ ‰È·Ê¤ÚÂÈ ·fi ¯ÒÚ· Û ¯ÒÚ·, ÂÓÒ, ÂÍ ·ÈÙ›·˜ ÙˆÓ ‰˘ÛÎÔÏÈÒÓ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ÛÙË ‰È¿ÁÓˆÛË, Â›Ó·È Èı·Ófi ÔÈ ·ÚÈıÌÔ› Ô˘ ·Ó·Ê¤ÚÔÓÙ·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 ÂÚÈÙÒÛÂȘ:1.000.000 ·È‰È¿ ÙÔ ¯ÚfiÓÔ (1,4). ªÂÁ¿Ï˜ ÛÂÈÚ¤˜ ÂÚÈÙÒÛÂˆÓ Ì ∞º™ ¤¯Ô˘Ó ·Ó·ÎÔÈÓˆı› ·fi ÙËÓ π·ˆÓ›· Î·È 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˘ ÓÔÛËχÙËÎ·Ó ÛÙÔ ›‰ÈÔ ‰È¿ÛÙËÌ· (0,052% Î·È 0,055% ·ÓÙÈÛÙÔ›¯ˆ˜) (6). °ÂÓÈο ÁÈ· ÙÔ FHL ¶ÂÚ›Ô˘ 25% fiÏˆÓ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ∞º™ Â›Ó·È 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΋ ˘ÔÏÂÈfiÌÂÓË ‰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 ÛÔÚ·‰ÈΤ˜ ÂÚÈÙÒÛÂȘ. ∂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 ·ÓÙÈÌÂÙˆÈÛÙ›. ∏ ̤ÛË ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ‰ÂÓ ˘Ô‚¿ÏÏÔÓÙ·È Û ıÂÚ·›· Â›Ó·È 2 Ì‹Ó˜, ÁÂÁÔÓfi˜ Ô˘ ‰Â›¯ÓÂÈ ÙËÓ ¤ÌÊ·ÛË Ô˘ Ú¤ÂÈ Ó· ‰Ôı› ÛÙËÓ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È ıÂÚ·›· (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›· ÌÔÚ› Ó· ·ÊÔÚ¿ ÙËÓ ÚˆÙ½ÓË ÂÚÊÔÚ›ÓË, Ë ÔÔ›· 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). √ ˘‡ı˘ÓÔ˜ ÁÔÓȉȷÎfi˜ ÙfiÔ˜ ‚Ú›ÛÎÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 10q21-22, ¯·Ú·ÎÙËÚ›˙ÂÈ ÙËÓ FHL-2 Î·È ··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 ÂΉ‹ÏˆÛË Ù˘ FHL-3, ÛÙËÓ ÔÔ›· ˘¿Ú¯ÂÈ ·ÓÂ¿ÚÎÂÈ· Paediatriki 2009;72:90-000

Ù˘ ÚˆÙ½Ó˘ Munc 13-4, Ë ÔÔ›· ÂÌϤÎÂÙ·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 ÂÚÈÙÒÛÂȘ ÛÙȘ Ôԛ˜ ‰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˜ ÌÂÙ·ÏÏ¿ÍÂȘ (10). ∆ÂÏÂ˘Ù·›·, Á›ÓÂÙ·È ÏfiÁÔ˜ ÁÈ· ÙËÓ FHL-4, fiÔ˘ ˘¿Ú¯ÂÈ ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ¯ÚˆÌfiۈ̷ 6q24, ‰ËÏ·‰‹ ÛÙÔ ÁÔÓ›‰ÈÔ Ù˘ Û˘ÓÙ·Í›Ó˘ 11 Ô˘ ÂËÚ¿˙ÂÈ ÙÔ Û‡ÛÙËÌ· Ê·ÁÔ΢ÙÙ¿ÚˆÛ˘, Ë ÔÔ›· ÂÓÙÔ›ÛÙËΠ۠ÌÈÎÚ‹ 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˘, fiÔ˘ Ë ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ÂÈÎfiÓ· 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 ÌÔÚ› Ó· ·Ó·Î¿Ì„ÂÈ ·˘ÙfiÌ·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¯ÔÔÈÔ‡ÓÙ·È ÁÈ· ÙËÓ ÚfiÎÏËÛË HLH, ·ÏÏ¿ ÂÓ›ÔÙ ˘ÚÔ‰ÔÙÔ‡Ó Î·È ÂÓÂÚÁÔÔÈÔ‡Ó ÙËÓ FLH, Â›Ó·È Ô EBV (ηٿ ·ÚÈÔ ÏfiÁÔ), Ô CMV, ¿ÏÏÔÈ ÂÚËÙÔ˚Ô›, Ô Èfi˜ ·ÓÂÌ¢ÏÔÁÈ¿˜¤ÚËÙ· ˙ˆÛÙ‹Ú·, Ô Èfi˜ Ù˘ ÈÏ·Ú¿˜, Ù˘ ÂÚ˘ıÚ¿˜, Ô ·Ú‚Ô˚fi˜ µ-19, Ô Èfi˜ Ù˘ ÁÚ›˘ ∞, ÔÈ ÈÔ› ∂CHO, Arbo, ηıÒ˜ Î·È ÙÔ Ì˘ÎÔ‚·ÎÙËÚ›‰ÈÔ Ù˘ Ê˘Ì·Ù›ˆÛ˘, ÙÔ Ì˘ÎfiÏ·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 ÌÔÚ› Ó· ÂΉËψı› ‡ÛÙÂÚ· ·fi HIV Ïԛ̈ÍË ‹/Î·È ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÔÓ ·ÓıÚÒÈÓÔ ÂÚËÙÔ˚fi 8, ·ÏÏ¿ Î·È Ï›ÁÔ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÓÙÈÚÂÙÚÔ˚΋˜ ıÂÚ·›·˜ ηٿ ÙÔ˘ ∏πV-AIDS (26). ∏ ÏËıÒÚ· ÙˆÓ ˘‡ı˘ÓˆÓ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ Î¿ÓÂÈ ÈÔ Â‡ÎÔÏ· ηٷÓÔËÙfi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô ·Ú¯ÈÎfi˜ fiÚÔ˜ (1979) «HLH Û¯ÂÙÈ˙fiÌÂÓÔ Ì ›ˆÛË» (virusassociated haemophagocytic syndrome, VAHS) ¿ÏÏ·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 ÌÔÚ› Ó· ÂΉËψı› Î·È ‡ÛÙÂÚ· ·fi ¯ÔÚ‹ÁËÛË Ê·Ú̿ΈÓ, fiˆ˜ ·ÓÙÈÂÈÏËÙÈÎÒÓ (Ê·ÈÓ˘ÙÔ˝ÓË, Ï·ÌÔÙÚÈÁ›ÓË), ·ÓÙÈ‚ÈÔÙÈÎÒÓ (ÁÏ˘ÎÔÂÙ›‰È·, ÎÔÙÚÈÌÔÍ·˙fiÏË) Î·È ÂӉ¯Ô̤ӈ˜ ÌÂÊ·ÈÓ·ÌÈÎÔ‡ ÔͤԘ (2,30,31).

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·91

91

∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË

√È Î·ÎÔ‹ıÂȘ Ì ÙȘ Ôԛ˜ Û˘Ó‰¤ÂÙ·È ÙÔ HLH ·ÊÔÚÔ‡Ó Î˘Ú›ˆ˜ ÙÔ ·ÈÌÔÔÈËÙÈÎfi Û‡ÛÙËÌ· (Ï¢¯·È̛˜-ÏÂÌÊÒÌ·Ù·) Î·È ÌÔÚ› Ó· Â¤ÏıÂÈ ÚÈÓ ‹ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜, Ó· Û¯ÂÙ›˙ÂÙ·È Ì Ïԛ̈ÍË ‹ Ó· Â¤Ú¯ÂÙ·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·ÓÂ¿ÚÎÂȘ, fiˆ˜ ÙÔ Ê˘ÏÔÛ‡Ó‰ÂÙÔ ÏÂÌÊÔÔÏÏ·Ï·ÛÈ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ (X-linked lymphoproliferative syndrome: XLP), ÙÔ Û‡Ó‰ÚÔÌÔ Griscelli, ÙÔ Û‡Ó‰ÚÔÌÔ HermanskyPudlak type II, Ë ÓfiÛÔ˜ Kawasaki Î·È ÙÔ Û‡Ó‰ÚÔÌÔ Chediak- Higashi. ∆¤ÏÔ˜, HLH ÌÔÚ› Ó· ÂΉËψı› ‡ÛÙÂÚ· ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ÓÂÊÚÒÓ-‹·ÙÔ˜ Î·È ÌÔÚ› Ó· Û˘Óԉ‡ÂÈ ÏÂÌÊÔ¸ÂÚÏ·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·ÙËÚÂ›Ù·È 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 Ì HLH (43,44). π‰È·›ÙÂÚÔ˜ ÏfiÁÔ˜ Ú¤ÂÈ Ó· Á›ÓÂÈ ÁÈ· ÙÔ Û‡Ó‰ÚÔÌÔ ÙˆÓ ÂÓÂÚÁÔÔÈËÌ¤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∞) Î·È Û·ÓÈfiÙÂÚ· Ì ¿ÏÏ· ÓÔÛ‹Ì·Ù· ÙÔ˘ Û˘Ó‰ÂÙÈÎÔ‡ ÈÛÙÔ‡ ÛÙ· ·È‰È¿, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤ÓÔ˘ ÙÔ˘ Û˘ÛÙËÌ·ÙÈÎÔ‡ ÂÚ˘ıËÌ·ÙÒ‰Ô˘˜ χÎÔ˘ (15). ∏ Û˘¯ÓfiÙËÙ· ÙÔ˘ MAS ÛÙË ™¡ƒ∞ ·Ó¤Ú¯ÂÙ·È Û 6,7%-13% (45,46,47). ªÔÚ› Ó· ÂΉËψı› ÔÔÙ‰‹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˘ÙÂÚÔ·ıÔ‡˜ HLH. £ÂˆÚÂ›Ù·È fiÙÈ ı· ˘¿ÚÍÂÈ ÂÈÙ˘¯‹˜ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÌfiÓÔ fiÙ·Ó Á›ÓÂÈ ¯Ú‹ÛË ÙˆÓ ‰È·ı¤ÛÈÌˆÓ ıÂÚ·¢ÙÈÎÒÓ Û¯ËÌ¿ÙˆÓ Ô˘ ˘¿Ú¯Ô˘Ó ÁÈ· ÙÔ HLH (15). ¶·Ú¿ÁÔÓÙ˜ Ô˘ ÌÔÚÔ‡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 (·ÛÈÚ›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 ·ÓıÂÎÙÈ΋ ÌÔÚÊ‹ ™¡ƒ∞ (15,45,48).

∫ÏÈÓÈ΋ ÂÈÎfiÓ· ∏ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ HLH (ÚˆÙÔ·ıÔ‡˜ Î·È ‰Â˘ÙÂÚÔ·ıÔ‡˜) ÂÚÈÏ·Ì‚¿ÓÂÈ Â›ÌÔÓÔ ˘ÚÂÙfi, Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ÂÍ¿ÓıËÌ· Î·È ÔÈÎÈÏ›· Ó¢ÚÔÏÔÁÈÎÒÓ Û˘Ìو̿وÓ, fiˆ˜ ¢ÂÚÂıÈÛÙfiÙËÙ·, Û·ÛÌÔ‡˜, ·Ú¿Ï˘ÛË ÎÚ·ÓÈ·ÎÒÓ Ó‡ڈÓ, ·Ù·Í›·, Ó˘ÛÙ·ÁÌfi, ‰˘Û¯¤ÚÂÈ· ÛÙË ‚¿‰ÈÛË, „˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ËÌÈÏËÁ›·, ÙÂÙÚ·ÏËÁ›·, ÔÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, Â̤ÙÔ˘˜, ·˘¯ÂÓÈ΋ ‰˘Ûη̄›· Î·È ÛËÌ›· ·˘ÍË̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›ÂÛ˘ (8,49). ∏ Ó¢ÌÔÓÈ΋ Û˘ÌÌÂÙÔ¯‹ Â›Ó·È Â›Û˘ Û˘¯Ó‹ (4). ∂ȉÈÎfiÙÂÚ· ÁÈ· ÙËÓ ÂÈÓ¤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˘˜. ∂ÈϤÔÓ, Û οÔÈÔ˘˜ ·fi ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜, Ù· Â˘Ú‹Ì·Ù· ÛÙÔÓ ÂÁΤʷÏÔ Â›¯·Ó ·Ô‰oı› Û ÚÔÛ‚ÔÏ‹ ·fi οÔÈÔÓ Èfi ‹ Û ÌË ÂȉÈ΋ ÌËÓÈÁÁÔÂÁÎÂÊ·Ï›Ùȉ· (8). ∏ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ‰È·Ù·Ú·¯ÒÓ ·fi ÙÔ ∫¡™ ·Ó¤Ú¯ÂÙ·È Û 20100% (8). ªÔÚ› 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˘ ηÎÔÔ›ËÛ˘, ÚÔηÏÒÓÙ·˜ ‰˘ÛÌÂÓ›˜ Û˘Ó¤ÂȘ ÁÈ· ÙËÓ ÔÈÎÔÁ¤ÓÂÈ·, ·ÏÏ¿ ΢ڛˆ˜ ηı˘ÛÙÂÚÒÓÙ·˜ ÂÈΛӉ˘Ó· ÙË ÛˆÛÙ‹ ‰È¿ÁÓˆÛË Î·È ıÂÚ·›· (50). ¢ÂÓ ˘¿Ú¯ÂÈ Û·Ê‹˜ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙˆÓ Ó¢ÚÔ·ıÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ Î·È ÙˆÓ ÈÛÙÔ·ıÔÏÔÁÈÎÒÓ ‚Ï·‚ÒÓ Û ¿ÏÏ· fiÚÁ·Ó·, ÂÓÒ ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÂÚ›ÙˆÛË HLH Ì ·ÔÎÏÂÈÛÙÈ΋ ÂÓÙfiÈÛË ÛÙÔ ∫¡™ (8,11). ¶·ıÔÊ˘ÛÈÔÏÔÁ›· √È ˘‡ı˘ÓÔÈ ·ıÔÊ˘ÛÈÔÏÔÁÈÎÔ› Ì˯·ÓÈÛÌÔ› ‰ÂÓ Â›Ó·È ·Ôχو˜ ÁÓˆÛÙÔ› Î·È Î·Ù·ÓÔËÙÔ›, ¤¯Ô˘Ó ·ÔÎ·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‹˜, fiˆ˜ Û·ÊÒ˜ ÚÔ·ÙÂÈ ·fi ÙË ‰È·›ÛÙˆÛË ˘ÂÚ΢ÙÙ·ÚÔÎÈÓ·ÈÌ›·˜, ηıÒ˜ Î·È ÙËÓ ·‡ÍËÛË ¿ÏÏˆÓ ÚÔÊÏÂÁÌÔÓˆ‰ÒÓ ÌÂÛÔÏ·‚ËÙÒÓ, fiˆ˜ Ô ·Ú¿ÁÔÓÙ·˜ Ó¤ÎÚˆÛ˘ 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˘ ÚˆÙ½ÓË ÙˆÓ Ì·ÎÚÔÊ¿ÁˆÓ (macrophage inflammatory protein: MIP-·), Ë ÈÓÙÂÚÊÂÚfiÓË Á (INF-Á) Î·È Ï‹ıÔ˜ ·ÈÌÔÔÈËÙÈÎÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Ô˘ ·ÂÏ¢ıÂÚÒÓÔÓÙ·È ·fi ÂÓÂÚÁÔÔÈË̤ӷ ÏÂÌÊÔ·ÙÙ·Ú· Î·È ÈÛÙÈÔ·ÙÙ·Ú· Û ÔÏÏ¿ fiÚÁ·Ó· (51-52). ™ËÌ·ÓÙÈÎfi˜ ÚfiÏÔ˜ ·Ô‰›‰ÂÙ·È Î·È Û ·ıÔÏÔÁÈΤ˜ ÚˆÙ½Ó˜ Ô˘ Έ‰ÈÎÔÔÈÔ‡ÓÙ·È ·fi Ù· ¶·È‰È·ÙÚÈ΋ 2009;72:16-22

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·92

92

Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

ÌÂÙ·ÏÏ·Á̤ӷ ÁÔÓ›‰È· Ù· ÔÔ›· ˘¿Ú¯Ô˘Ó Û ¿ÙÔÌ· Ì FHL, ·ÏÏ¿ Î·È ÛÙÔ HLH, Ì ΢ÚÈfiÙÂÚÔ ÂÎÚfiÛˆÔ ÙËÓ ÂÚÊÔÚ›ÓË. ∏ INF-Á ·ÚÔ˘ÛÈ¿˙ÂÈ ·ÚÎÂÙ¿ ˘„ËÏfiÙÂÚ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ÛÙÔ Ï¿ÛÌ· ηٿ ÙËÓ ÂÓÂÚÁfi Ê¿ÛË Ù˘ ÓfiÛÔ˘, ·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˘, fiˆ˜ ÔÈ Î˘ÙÙ·ÚÔÂӛ˜, 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˜ ÂÚÈÙÒÛÂȘ HLH, ÂÓÒ ·ÓÙÈı¤Ùˆ˜ Ôχ ·˘ÍË̤ӷ Â›‰· sCD-25 (>10000 U/ml) ‰ËÏÒÓÔ˘Ó ‰˘ÛÌÂÓ‹ ÚfiÁÓˆÛË ÁÈ· fiϘ ÙȘ ηÙËÁÔڛ˜ ·ÛıÂÓÒÓ (13,10,60). ∏ ÊÂÚÚÈÙ›ÓË, Â›Û˘ ÛËÌ·ÓÙÈÎfi˜ ‰Â›ÎÙ˘, ·˘Í¿ÓÂÙ·È ÏfiÁˆ ÙÔ˘ ÔÍÂȉˆÙÈÎÔ‡ stress Î·È ÌÔÚ› Ó· Û¯ÂÙ›˙ÂÙ·È Ì ·ÓÙÈÛÙ·ıÌÈÛÙÈ΋ ·ÓÔÛÔÏÔÁÈ΋ ·¿ÓÙËÛË ÛÙÔ HLH. ∏ ·ÓÂͤÏÂÁÎÙË ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÓÔÛÔÏÔÁÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Á›ÓÂÙ·È Èı·ÓÒ˜ ÏfiÁˆ ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋˜ ·ÓÔÛÔÏÔÁÈ΋˜ ·¿ÓÙËÛ˘ Ô˘ ˘ÚÔ‰ÔÙÂ›Ù·È ·fi Â͈ÁÂÓ‹ ÂÚÂı›ÛÌ·Ù·, fiˆ˜ Â›Ó·È ÔÈ ÏÔÈÌÔÁfiÓÔÈ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ› Î·È ÔÈ ÙÔ͛Ә, ‹ ·fi ÂÓ‰ÔÁÂÓ›˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ ÙÔ ÔÍÂȉˆÙÈÎfi stress, Ë ÈÛÙÈ΋ ηٷÛÙÚÔÊ‹ ‹ ÚÔ˚fiÓÙ· ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ (13). ∆Ô ˘ÔΛÌÂÓÔ ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÏÂÈÌÌ· Ô˘ Û˘Ì‚¿ÏÏÂÈ ÛÙËÓ ·Ó¿Ù˘ÍË HLH ÌÔÚ› Ó· Â›Ó·È ÎÏËÚÔÓÔÌÔ‡ÌÂÓË ‰È·Ù·Ú·¯‹ ÙˆÓ ·ÓÔÛÔ˚ηÓÒÓ Î˘ÙÙ¿ÚˆÓ ‹ Â›ÎÙËÙË, fiˆ˜ .¯. È·ÙÚÔÁÂÓ¤˜ ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÏÂÈÌÌ· ηÙfiÈÓ ÌÂÙ·ÌfiÛ¯Â˘Û˘ ‹ ΢ÙÙ·ÚÔÙÔÍÈ΋˜ ıÂÚ·›·˜. ∆Ô Â›ÎÙËÙÔ ¤ÏÏÂÈÌÌ· ÛÙËÓ ·ÓÔÛ›· ÌÔÚ› Â›Û˘ Ó· Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ïԛ̈͢ ÙˆÓ ·ÓÔÛÔ˚ηÓÒÓ Î˘ÙÙ¿ÚˆÓ ‹ Ó· ÚÔηÏÂ›Ù·È ¤ÌÌÂÛ· ̤ۈ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ‰Ú¿Û˘ ÙˆÓ ÊÏÂÁÌÔÓˆ‰ÒÓ ÌÂÛÔÏ·‚ËÙÒÓ. °ÂÓÈο, ÙÔ HLH Â›Ó·È ˘ÂÚÊÏÂÁÌÔÓ҉˜ Û‡Ó‰ÚÔÌÔ, Ô˘ ˘ԉȷÁÈÁÓÒÛÎÂÙ·È Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ˘ÂÚ‰Ú·ÛÙ‹ÚÈ· Ê·ÁÔ·ÙÙ·Ú·, ÌË ·ÔÙÂÏÂÛÌ·ÙÈ΋ ¤Ó·ÚÍË Ù˘ ·fiÙˆÛ˘ Î·È ÌÂȈ̤ÓË ‰Ú·ÛÙËÚÈfiÙËÙ· 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˘ ΢ÙÙ·ÚÔÙÔÍÈÎfiÙËÙ·˜ ÙˆÓ ∆, Û‡ÌÊ˘ÙË ‹ Â›ÎÙËÙË, ÌÔÚ› Ó· Â›Ó·È ÎÔÈÓfi˜ Ì˯·ÓÈÛÌfi˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì HLH, Ô˘ Ô‰ËÁ› Û ·ÚfiÌÔȘ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ (13). ¢˘ÛÙ˘¯Ò˜, ÔÈ ÂÍÂÙ¿ÛÂȘ Ô˘ ÌÔÚÔ‡Ó Ó· ·Ôηχ„Ô˘Ó ÙÔ˘˜ ÂοÛÙÔÙ ÂÌÏÂÎfiÌÂÓÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜, Á›ÓÔÓÙ·È ÌfiÓÔ Û ΤÓÙÚ· Ì ˘„ËÏ‹ ÂÍÂȉ›Î¢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 ıÂÚ·›· Î·È ÙËÓ ÂÚ·ÈÙ¤Úˆ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ (53).

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·ÚÔÂӛ˜ (Ó· ÂËÚ¿˙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) - ∞ÈÌÔÂÙ¿ÏÈ· <100x109/L - √˘‰ÂÙÂÚfiÊÈÏ· <1,0x109/L iv) ÀÂÚÙÚÈÁÏ˘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, ÛÏ‹Ó·, ÏÂÌÊ·‰¤Ó˜ ‹ ∂¡À: ¯ˆÚ›˜ ÂӉ›ÍÂȘ ηÎÔ‹ıÂÈ·˜ vi) ∂Ï·Ùو̤ÓË ‹ Ï‹Ú˘ ¤ÏÏÂÈ„Ë ‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¡∫ (Û‡Ìʈӷ Ì ÙȘ ÙÈ̤˜ ·Ó·ÊÔÚ¿˜ ÙÔ˘ ÂοÛÙÔÙ ÂÚÁ·ÛÙËÚ›Ô˘) vii) ∞˘ÍË̤ӷ Â›‰· 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 ™˘ÌÙÒÌ·Ù· ∫¡™ Î·È ∂˘Ú‹Ì·Ù· ∂¡À: ·)̤ÙÚÈ· ÏÂÈÔ΢ÙÙ¿ÚˆÛË 20-80 ÏÂÌÊÔ·ÙÙ·Ú·/ÌL ENY (΢ڛˆ˜ ∆ ÏÂÌÊÔ·ÙÙ·Ú·) ‚) ·‡ÍËÛË Ï¢ÎÒÌ·ÙÔ˜ Û 0,5-1 g/L ENY Á) ·ÈÌÔÊ·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

∂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· Â›‰· 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·ÚÔΛÓ˜, 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 ·fiÚÚÔÈ· ÙÔ˘ ÂÈıÂÙÈÎÔ‡ 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 ·ÈÌÔÛÊ·ÈÚ›ˆÓ ‹ ·ÈÌÔÂÙ·Ï›ˆÓ ̤۷ ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ, ÛÙÔÓ ÛÏ‹Ó· Î·È ÛÙÔ˘˜ ÏÂÌÊ·‰¤Ó˜, Ô˘ ÌÔÚ› 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›·, ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ‹Ù·Ó ·ÚÔ‡Û· ÌfiÓÔ ÛÙÔ 32% ÙˆÓ

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·93

93

∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË

¶›Ó·Î·˜ 2. ∫ÏÈÓÈ΋ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ Û˘ÌÙˆÌ¿ÙˆÓ ÛÙÔ ∫¡™-∞º™ ™Ù¿‰ÈÔ 0: ™Ù¿‰ÈÔ 1: ™Ù¿‰ÈÔ 2: ™Ù¿‰ÈÔ 3:

¶Ï‹Ú˘ ¤ÏÏÂÈ„Ë Ó¢ÚÔÏÔÁÈÎÒÓ ÂΉËÏÒÛÂˆÓ ‹ ÌË ÂȉÈΤ˜ Ó¢ÚÔÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜ ªË ÂȉÈο Ó¢ÚÔÏÔÁÈο ÛËÌ›·, fiˆ˜ Ûʇ˙Ô˘Û· ËÁ‹ ‹ ˘ÔÙÔÓ›· ∂ȉÈο Ó¢ÚÔÏÔÁÈο ÛËÌ›·, fiˆ˜ ·˘¯ÂÓÈ΋ ‰˘Ûη̄›·, Û·ÛÌÔ›, ·Ú¤ÛÂȘ ™Ô‚·Ú‹ Ó¢ÚÔÏÔÁÈ΋ ÓfiÛÔ˜, fiˆ˜ ÚÔԉ¢ÙÈ΋ „˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË ‹ ·ÒÏÂÈ· Û˘Ó›‰ËÛ˘ (ÎÒÌ·)

¶›Ó·Î·˜ 3. πÛÙÔÏÔÁÈ΋ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ Â˘ÚËÌ¿ÙˆÓ ÛÙÔ ∫¡™∞º™ (8) ™Ù¿‰ÈÔ 0: ™Ù¿‰ÈÔ π:

™Ù¿‰ÈÔ ππ:

™Ù¿‰ÈÔ πππ:

ÂÚÈÙÒÛÂˆÓ ÛÙËÓ ÚÒÙË Ï‹„Ë Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ Î·È ÛÙÔ 85% ÙË ÛÙÈÁÌ‹ Ù˘ ‰È¿ÁÓˆÛ˘ (13). ∏ Ë·ÙÈ΋ ·ÓÂ¿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˘ ÌÔÚ› Ó· ÂÌÊ·Ó›˙Ô˘Ó È‰È·ÈÙ¤Úˆ˜ ·˘ÍË̤ӷ Â›‰· Ë·ÙÈÎÒÓ 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 ÂÚÈÙÒÛˆÓ, Â›Ó·È ‰‡ÛÎÔÏË ‹ Î·È ·‰‡Ó·ÙË, fï˜ ÙÔ ÁÂÁÔÓfi˜ ·˘Ùfi ‰ÂÓ ı· Ú¤ÂÈ Ó· ·ÔÙÂÏ› ÂÌfi‰ÈÔ ÛÙËÓ ¿ÌÂÛË ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, 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·. ∂ÈϤÔÓ, ˘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. ∆· Â›‰¿ ÙÔ˘ Û¯ÂÙ›˙ÔÓÙ·È ÛÙÂÓ¿ Ì ÙË ‰Ú·ÛÙËÚÈfiÙËÙ· Ù˘ ÓfiÛÔ˘ Î·È Â›Û˘ Ì ÙË ÊÂÚÚÈÙ›ÓË ÙÔ˘ ÔÚÔ‡. ∏ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ÎÏÈÓÈÎÒÓ Î·È ÈÛÙÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ Û˘ÓÔ„›˙ÂÙ·È ÛÙÔ˘˜ ¶›Ó·Î˜ 2 Î·È 3 (8).

¶ÚÔÛ‚ÔÏ‹ ÙÔ˘ ∫¡™ ŸÙ·Ó ˘¿Ú¯ÂÈ ÂÈÓ¤ÌÂÛË ÙÔ˘ ∫¡™, ÙÔ Î˘ÚÈfiÙÂÚÔ ÈÛÙÔ·ıÔÏÔÁÈÎfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi Â›Ó·È Ë ÂÎÙÂٷ̤ÓË, ÌË Î·ÎÔ‹ı˘ ‰È‹ıËÛË ·fi ÏÂÌÊÔ·ÙÙ·Ú· Î·È Ì·ÎÚÔÊ¿Á·, Ù˘ÈÎfiÙÂÚ· Ì ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË, Û˘Ó‹ıˆ˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ‰È‹ıËÛË Î·È Û ¿ÏÏ· fiÚÁ·Ó·, fiˆ˜ ÛÏ¿¯Ó·, ÏÂÌÊ·‰¤Ó˜, Ì˘ÂÏfi˜ ÙˆÓ ÔÛÙÒÓ Î.¿. (55). ªÔÚ› Ó· ˘¿Ú¯ÂÈ ÂÈÓ¤ÌËÛË Î·È ÙˆÓ ÂÚÈÊÂÚÈÎÒÓ ÓÂ‡ÚˆÓ (8). ∂›Û˘, Â›Ó·È ÂӉȷʤÚÔÓ fiÙÈ ¤¯ÂÈ ‰È·ÈÛÙˆı›

¶Ï‹Ú˘ ¤ÏÏÂÈ„Ë Ó¢ÚÔ-·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ ªfiÓÔ ÏÂÙÔÌËÓÈÁÁÈΤ˜ ‰ÈËı‹ÛÂȘ ·fi ÏÂÌÊÔ·ÙÙ·Ú· Î·È ÈÛÙÈÔ·ÙÙ·Ú· /Ì·ÎÚÔÊ¿Á·. ¶ÂÚÈÛÙ·Ûȷο ÂÚÈ·ÁÁÂȷ΋ ‰È‹ıËÛË. ◊È· ·ÚÂÁ¯˘Ì·ÙÈ΋ Û˘ÌÌÂÙÔ¯‹ Ì ÂÓÙÔÓfiÙÂÚË ÂÚÈ·ÁÁÂȷ΋ ‰È‹ıËÛË, ·ÏÏ¿ Î·È ÂÓÙÔÓfiÙÂÚË ‰È‹ıËÛË ÙˆÓ ÌËÓ›ÁÁˆÓ, Ô˘ ÂÓ›ÔÙ ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ÙÔÓ ˘ÔΛÌÂÓÔ ÂÁÎÂÊ·ÏÈÎfi ÈÛÙfi. ∂ÈϤÔÓ Ù˘ ¤ÓÙÔÓ˘ Ì·˙È΋˜ ÈÛÙÈ΋˜ ‰È‹ıËÛ˘ ·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·È Û¯ÂÙÈο Ï›Á· ·ÊÚÒ‰Ë Ì·ÎÚÔÊ¿Á·. ªÔÚ› Ó· ˘¿Ú¯Ô˘Ó Â·Û‚ÂÛÙÒÛÂȘ.

Û˘Û¯¤ÙÈÛË Ù˘ ÎÏÈÓÈ΋˜ ‚·Ú‡ÙËÙ·˜ Ù˘ ÚÔÛ‚ÔÏ‹˜ ÙÔ˘ ∫¡™ Î·È ÙˆÓ Ó¢ÚÔ·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎÒÓ ·ÏÏÔÈÒÛÂˆÓ (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˘ ∫¡™ ÌÔÚÔ‡Ó Ó· Ù·ÍÈÓÔÌËıÔ‡Ó ˆ˜ ¿Û¯ÔÓÙ˜ ›Ù ·fi ÏÂÌÊÔ΢ÙÙ·ÚÈ΋ ÌËÓÈÁÁ›Ùȉ·/ÂÁÎÂÊ·Ï›Ùȉ·, fiÔ˘ ‰ÂÓ ˘¿Ú¯ÂÈ Î·Ó¤Ó· ¿ÏÏÔ Ó¢ÚÔÏÔÁÈÎfi Û‡Ìو̷ ÂÎÙfi˜ ·fi ÌËÓÈÁÁÈÛÌfi Î·È ·ıÔÏÔÁÈÎfi ∂¡À, ›Ù ·fi «Ó¢ÚÔÏÔÁÈ΋ Û˘Ìو̷ÙÔÏÔÁ›·», ‰ËÏ·‰‹ ÔÔÈÔ‰‹ÔÙ Ó¢ÚÔÏÔÁÈÎfi Úfi‚ÏËÌ· ‹ Û·ÛÌÔ‡˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ‰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 ÓÂÔÙÂÚ›Ó˘ ÛÙÔ ∂¡À (8,55). ∆Ô Î˘ÙÙ·ÚÈÎfi ÂÚȯfiÌÂÓÔ ÙÔ˘ ∂¡À ‰ÂÓ Ê·›ÓÂÙ·È fiÙÈ ·ÔÙÂÏ› ȉȷ›ÙÂÚ· ¯Ú‹ÛÈÌÔ ‰Â›ÎÙË Ù˘ ‚·Ú‡ÙËÙ·˜ ÂÈÓ¤ÌËÛ˘ ÙÔ˘ ∫¡™, Û ·ÓÙ›ıÂÛË Ì ÙÔ Ï‡Έ̷, ÙÔ ÔÔ›Ô ‹Ù·Ó ȉȷ›ÙÂÚ· ·˘ÍË̤ÓÔ Û ·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·‰ÈÔÔ›ËÛË (ÛÙ¿‰ÈÔ πππ) (8). ¶·ÚfiÏ· ·˘Ù¿, ÙÔ ∂¡À ÌÔÚ› Ó· Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi, ·ÎfiÌ· Î·È ¶›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· ÙË ÓfiÛÔ) ¶·È‰È·ÙÚÈ΋ 2009;72:16-22

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·94

94

Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

ηٿ ÙË ‰È¿ÚÎÂÈ· ÎÏÈÓÈο ÚÔ¯ˆÚË̤Ó˘ ÓfiÛÔ˘ ÙÔ˘ ∫¡™ (8).

∞ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù· ÀÂÚ˯ÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ηٿ ÙËÓ ·Ú¯È΋ ÚÔۤϢÛË ÙˆÓ ·ÛıÂÓÒÓ Â›Ó·È (Ì Êı›ÓÔ˘Û· ÛÂÈÚ¿ Û˘¯ÓfiÙËÙ·˜): ·) ÛÏËÓÔÌÂÁ·Ï›·, ‚) Ë·ÙÔÌÂÁ·Ï›·, Á) ·ÛΛÙ˘, ‰) ¿¯˘ÓÛË ÙÔ˘ ÙÔȯÒÌ·ÙÔ˜ Ù˘ ¯ÔÏˉfi¯Ô˘ ·ÛÙ˘, Â) ·˘ÍË̤ÓË ÂÚÈ˘Ï·›· ˯ÔÁ¤ÓÂÈ·, ÛÙ) ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ˙) Ï¢ÚÈÙÈÎfi ÂÍ›‰ÚˆÌ· ηÈ, Û·ÓÈfiÙÂÚ·, Ë) ÔÏÏ·Ϥ˜ ˘fi˯˜ ÂÚÈÔ¯¤˜ ÛÙÔÓ ÛÏ‹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˜ Ì Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹, Û˘¯Ó¿ Ì ٷ¯Â›· ÂͤÏÈÍË Î·È ·ÔηٿÛÙ·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 Â˘Ú‹Ì·Ù· ÌÔÚ› Ó· ·Ú·¤ÌÔ˘Ó Û SARS ‹ ARDS (57). ™ÙËÓ ˘ÔÏÔÁÈÛÙÈ΋ Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·, Ù· Â˘Ú‹Ì·Ù· ÔÈΛÏÏÔ˘Ó Î·È ÌÔÚ› Ó· Â›Ó·È ·ÎfiÌ· Î·È Ê˘ÛÈÔÏÔÁÈο, ·Ú¿ Ù· ¤ÓÙÔÓ· Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ∫¡™ (8). ÕÏÏÔÙ ÌÔÚ› Ó· ˘¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ ·˘ÍË̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›ÂÛ˘, ·ÓÒÌ·ÏË ÏÂÙÔÌËÓÈÁÁÈ΋ ÂÓ›Û¯˘ÛË, ÂÛÙȷ΋ Ó¤ÎÚˆÛË Î·È ˘fi˘ÎÓ˜ ÓÂÎÚˆÙÈΤ˜ ÂÚÈÔ¯¤˜, Ì ·ÒÏÂÈ· ·ÚÂÁ¯˘Ì·ÙÈÎÔ‡ fiÁÎÔ˘ Î·È ·ÙÚÔÊ›· (8,50). ™ÙË Û˘Ó¤¯ÂÈ·, ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ÂÚÈÔ¯¤˜ Ì ӤÎÚˆÛË Î·È ÂÛÙȷ΋ ‹ ‰È¿¯˘ÙË ·ÔÌ˘ÂϛӈÛË. ∂›Û˘, ÂÚÈÁÚ¿ÊÔÓÙ·È ÂÛÙȷΤ˜ ·ÈÌÔÚÚ·Á›Â˜ Î·È ‰È¿Ù·ÛË ÙÔ˘ ˘ÔÛÎÏËÚ›‰ÈÔ˘ ¯ÒÚÔ˘ (50). ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÌËÓÈÁÁ›Ùȉ· ÌfiÓÔ, ÌÔÚ› Ó· ·ÓȯÓ‡ÔÓÙ·È ÌfiÓÔ ·ÓˆÌ·Ï›Â˜ ÛÙË Ï¢΋ Ô˘Û›· (55). ¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ∆Ô ∞º™ ÌÔÚ› Ó· ÌÈÌÂ›Ù·È ‰È¿ÊÔÚ· ÓÔÛ‹Ì·Ù· Î·È ÁÈ’ ·˘Ùfi Ú¤ÂÈ Ó· ‰È·ÊÔÚԉȷÁÓˆÛı› ·fi ÔÏÏ·Ï‹ ·ÓÂ¿ÚÎÂÈ· ÔÚÁ¿ÓˆÓ, ˘ÚÂÙfi ·ÁÓÒÛÙÔ˘ ·ÈÙÈÔÏÔÁ›·˜, 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, ηÎfiËı˜ ÁÏÔ›ˆÌ·, ÔÍ›· ·ÈÌÔÚÚ·ÁÈ΋ Ï¢ÎÔÂÁÎÂÊ·Ï›Ùȉ·, ۈ̷ÙÈ΋ ηÎÔÔ›ËÛË, ·ÏÏÂÚÁÈ΋ ÔÚʇڷ, ÈÛÙÈÔ΢ÙÙ¿ÚˆÛË ·fi ·ÙÙ·Ú· ÙÔ˘ Langerhans, ϤÌʈ̷ ‹ ¿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˜ ÌÂÙ¿ ÙÔ ı¿Ó·ÙÔ. £ÂÚ·›· √ ¿ÌÂÛÔ˜ ÛÙfi¯Ô˜ Ù˘ ıÂÚ·›·˜ Â›Ó·È Ë Î·Ù·ÛÙÔPaediatriki 2009;72:16-22

Ï‹ Ù˘ ÛÔ‚·Ú‹˜ ˘ÂÚÊÏÂÁÌÔÓ‹˜, Ô˘ ıˆÚÂ›Ù·È ˘‡ı˘ÓË ÁÈ· Ù· ·ÂÈÏËÙÈο ÁÈ· ÙË ˙ˆ‹ Û˘ÌÙÒÌ·Ù·. ¢Â˘ÙÂÚ¢fiÓÙˆ˜, ÛÎÔfi˜ 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 ÙÚfiÔ ·˘Ùfi ÙÔ˘ ÂÚÂı›ÛÌ·ÙÔ˜ ÁÈ· ¤ÓÙÔÓË ·ÏÏ¿ ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ‰Ú·ÛÙËÚÈÔÔ›ËÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¡∫ Î·È Î·Ù·ÛÙ·ÏÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ∆. ™ÙȘ ÂÚÈÙÒÛÂȘ Ì ÁÂÓÂÙÈÎfi ˘fiÛÙڈ̷, Ô ·fiÏ˘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 ÚÔÔÙÈ΋ ıÂÚ·¢ÙÈ΋ ¤Ú¢ӷ Û˘ÓÂÚÁ·Û›·˜ (ÙÔ ÚˆÙfiÎÔÏÏÔ HLH-94), Ì ÛÙfi¯Ô ÙË ‚ÂÏÙ›ˆÛË ÙÔ˘ ÔÛÔÛÙÔ‡ ÂÈ‚›ˆÛ˘ (1,28). ª¤¯ÚÈ ÙfiÙÂ, Ë ıÓËÙfiÙËÙ· ·ÓÂÚ¯fiÙ·Ó Û >90%. ∆Ô 2004 ÍÂΛÓËÛÂ Ë ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ÚˆÙÔÎfiÏÏÔ˘ HLH2004, ÌÈ·˜ Ó¤·˜ ¤Î‰ÔÛ˘ ÙÔ˘ ·Ï·ÈfiÙÂÚÔ˘ (58). ™Ù· ÚˆÙfiÎÔÏÏ· ·˘Ù¿ Û˘Ó‰˘¿˙ÔÓÙ·È Ë ¯ËÌÂÈÔıÂÚ·›· Î·È Ë ·ÓÔÛÔıÂÚ·›·, ÌÂ Û˘Ó·ÎfiÏÔ˘ıË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ÁÈ· ÙȘ Â›ÌÔÓ˜, ˘ÔÙÚÔÈ¿˙Ô˘Û˜ ‹/Î·È ÔÈÎÔÁÂÓ›˜ ÌÔÚʤ˜ (1). ∏ ıÂÚ·›· ¤Ó·Ú͢ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙË ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È ÂÙÔÔÛ›‰Ë˜, Ô˘ ·ÎÔÏÔ˘ıÔ‡ÓÙ·È ·fi Û˘Ó¯‹ ıÂÚ·›· Ì ΢ÎÏÔÛÔÚ›ÓË ∞, Ì·˙› Ì ÒÛÂȘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È ÂÙÔÔÛ›‰Ë˜, ÁÈ· ÂÚ›Ô˘ 1 ¯ÚfiÓÔ. ∞˘Ù‹ Ë ıÂÚ·›· ÌÔÚ› 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). ∏ ÂÙÔÔÛ›‰Ë Â›Ó·È ¤Ó· ÂÍ·ÈÚÂÙÈÎfi Ê¿ÚÌ·ÎÔ ÁÈ· Ó· ÚÔηϤÛÂÈ ¤Ó·ÚÍË Ù˘ ·fiÙˆÛ˘, Ì ·ÚÂÓ¤ÚÁÂȘ ÙË ÛÔ‚·Ú‹ Ô˘‰ÂÙÂÚÔÂÓ›· ηÈ, Û·ÓÈfiÙÂÚ·, ÙË ‰ËÌÈÔ˘ÚÁ›· ÔÍ›·˜ Ì˘ÂÏÔÁÂÓÔ‡˜ Ï¢¯·ÈÌ›·˜ Î·È Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ (1). ∏ ‰ÂÍ·ÌÂı·˙fiÓË ‰ÈÂÈÛ‰‡ÂÈ ÛÙÔÓ ·ÈÌ·ÙÂÁÎÂÊ·ÏÈÎfi ÊÚ·ÁÌfi Ì ηϤ˜ ·ÓÙÈÊÏÂÁÌÔÓÒ‰ÂȘ Î·È ÚÔ·ÔÙˆÙÈΤ˜ ȉÈfiÙËÙ˜, ÂÓÒ Ë Î˘ÎÏÔÛÔÚ›ÓË ∞ ÌÂÈÒÓÂÈ ÙË ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆, Ô˘ Â›Ó·È ·˘ÍË̤ÓË ÛÙÔ HLH Î·È ÌÔÚ› Ó· ÌÂÈÒÛÂÈ ÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ı·Ó·ÙËÊfiÚˆÓ ÏÔÈÌÒÍÂˆÓ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ Ô˘‰ÂÙÂÚÔÂÓ›· (1,60). ™ÙË ÁÂÓÈÎfiÙÂÚË ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÓfiÛÔ˘ ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› Ï·ÛÌ·Ê·›ÚÂÛË, ·Ê·ÈÌ·ÍÔÌÂÙ¿ÁÁÈÛË, ·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 ‹Ș ÌÔÚʤ˜ HLH ·fi ÏÔÈÌ҉˜ ·›ÙÈÔ, ·ÚΛ ÌfiÓÔ ·ÓÙÈÏÔÈÌ҉˘ ·ÁˆÁ‹, fiˆ˜ Û ·ÛıÂÓ›˜ Ì ÏÂ˚ÛÌ·Ó›·ÛË. ∏ ÂÙÂÚfiÏÔÁË ÌÂÙ·ÌfiÛ¯Â˘ÛË ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·Ú¤¯ÂÈ Ù· ηχÙÂÚ· ıÂÚ·¢ÙÈο ·ÔÙÂϤÛÌ·Ù· (3,11,50,55,56). ∞ӷʤÚÂÙ·È ÂÈ‚›ˆÛË 62% ÛÂ

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·95

95

∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË

‰È¿ÊÔÚ˜ ÌÂϤÙ˜, ÔÛÔÛÙfi ۯ‰fiÓ ·ÓÔÌÔÈfiÙ˘Ô (62%±12%) Ì ÙËÓ ÙÚÈÂÙ‹ ÂÈ‚›ˆÛË ·È‰ÈÒÓ (¿Û¯ÔÓÙ· ·fi FHL ‹ Â›ÌÔÓ˜-˘ÔÙÚÔÈ¿˙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¿ ÂÈıÂÙÈ΋, ·ÚÎÂÙÔ› ·ÛıÂÓ›˜ ηٷϋÁÔ˘Ó ÚÈÓ Ï¿‚Ô˘Ó HCT. ∞˘Ù‹ Ë ıÂÚ·›· ·ÊÔÚ¿ ÔÌÔ˙˘ÁÒÙ˜ ÁÈ· ÌÂÙ·ÏÏ¿ÍÂȘ ‰È·ÊfiÚˆÓ ÁÔÓȉ›ˆÓ Û¯ÂÙÈο Ì ÙÔ ∞º™, ·ÛıÂÓ›˜ Ô˘ ··ÓÙÔ‡Ó ·ÓÂ·ÚÎÒ˜ ÛÙËÓ ·Ú¯È΋ ıÂÚ·›· Î·È ÂΛÓÔ˘˜ Ì ÓfiÛÔ ÙÔ˘ ∫¡™. √È ÂÚÈÛÛfiÙÂÚÔÈ ÂȉÈÎÔ› ıˆÚÔ‡Ó fiÙÈ Ë ¿ÌÂÛË ¤Ó·ÚÍË ·ÓÔÛÔ¯ËÌÂÈÔıÂÚ·›·˜, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÚԷӷʤÚıËηÓ, ·ÎÔÏÔ˘ıÔ‡ÌÂÓË ·fi HCT, Ê·›ÓÂÙ·È ˆ˜ Ë ÌfiÓË ıÂÚ·¢ÙÈ΋ ‰È·‰Èηۛ· Ô˘ Â›Ó·È ÈηӋ Ó· ·Ó·ÛÙ›ÏÂÈ ÙËÓ ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘ ÙÔ˘ ∫¡™ Î·È Ó· ÂÈʤÚÂÈ ÙËÓ ›·ÛË, ·Ó Á›ÓÂÈ Û¯ÂÙÈο ÓˆÚ›˜, ·ÊÔ‡ ÚÒÙ· ÂÈÙ¢¯ı› ‡ÊÂÛË (12,55). ∆ÂÏÂÈÒÓÔÓÙ·˜, ˘ÔÁÚ·ÌÌ›˙ÂÙ·È Ë ·Ó¿ÁÎË ÁÈ· ÙËÓ Â˘·ÈÛıËÙÔÔ›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› ıÂÚ·›·.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Henter JI, Samuelsson-Horne AC, Arico M, Egeler M, Elinder G, Filipovich A, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunotherapy and bone marrow transplantation. Blood 2002;100: 2367-2373.

2. Yang Y-C, Jou S-T, Chang Y-H, Liang J-S, Lee W-T. Hemophagocytic syndrome associated with antiepileptic drug. Pediatr Neurol 2004;30:358-356.

3. DiCarlo J, Lui W, Frankel L, Howell W, Schiffman J, Alexander S. The hemophagocytic syndrome: titrating continuous hemofiltration to the degree of lactic acidosis. J Pediatr Hematol Oncol 2006;23:599-610.

4. Henter JI, Elinder G, Soder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand 1991;80:428-435.

5. van der Woude HJ, van der Werf TS, Verschuuren EAM, Tamminga RYJ, Rosati S, Meertens JHJM, et al. An 18year-old man with rapidly progressive multiorgan failure after a positive mononucleosis spot test result. Chest 2006;130:291-295.

6. Gurgey A, Secmeer G, Tavil B, Ceyhan M, Kuskonmaz B, Cengiz B, et al. Secondary hemophagocytic lymphohistiocytosis in Turkish children. J Pediatr Infect Dis 2005; 24:1116-1117.

7. Farquhar JW, Claireaux AE. Familial hemophagocytic reticulosis. Arch Dis Child 1952;27:519-525.

8. Henter JI, Nennesmo I. Neurophathologic findings and neurologic symptoms in twenty-three children with hemophagocytic lymphohistiocytosis. J Pediatr 1997;130:358-365.

9. Henter JI, Ehrnst A, Andersson J, Elinder G. Familial hemophagocytic lymphohistiocytosis and viral infections.

Acta Paediatr 1993;82:369-372.

10. Ueda I, Ishii E, Morimoto A, Ohga S, Sako M, Imashuku S. Correlation between phenotypic heterogeneity and gene

mutational characteristics in familial hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2006;46:482-488.

11. Shinoda J, Murase S, Takenaka K, Sakai N. Isolated central nervous system hemophagocytic lymphohistiocytosis: case report. Neurosurgery 2005;56:187-190.

12. Schmidt MH, Sung L, Shuckett BM. Hemophagocytic lymphohistiocytosis in children: abdominal US findings within 1 week of presentation. Radiology 2004;230:685-689.

13. Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 2004;124:4-14 14. Marcenaro S, Gallo F, Martini S, Santoro A, Griffiths GM, AricÔ` M, et al. Analysis of natural killer-cell function in familial hemophagocytic lymphohistiocytosis (FHL): defective CD 107a surface expression heralds Munc 13-4 defect and discriminates between genetic subtypes of the disease. Blood 2006;108:2316-2323.

15. Kelly A, Ramanan AV. Recognition and management of macrophage activation syndrome in juvenile arthritis.

Curr Opin Rheumatol 2007;19:477-481.

16. Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis: The FHL Study Group of the Histiocyte Society. Semin Oncol 1991;18;29-33.

17. Arico M, Janka G, Fischer A, Henter JI, Blanche S, Elinder J, et al Hemophagic histiocytosis. Report of 122 children from the International Registry. Leukemia 1996;10:197-203.

18. Reiner AP, Spivak JL. Hematophagic histiocytosis. A report of 23 new patients and a review of the literature.

Medicine 1988;67:369-388.

19. Clementi R, Emmi L, Maccario R, Liotta F, Moretta L, Danesino C, et al. Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Blood 2002;100:2266-2267.

20. Ishida Y, Hiroi K, Tauchi H, Oto Y, Tokuda K, Kida K. Hemophagocytic lymphohistiocytosis secondary to Mycoplasma pneumoniae infection. Pediatrics International 2004;46:174-177.

21. McClain K, Gehrz R, Grierson H, Purtilo D, Filipovich A. Virus-associated histiocytic proliferations in children.

Frequent association with Epstein-Barr virus and congenital or acquired immunodeficiencies. Am J Pediatr Hematol Oncol 1988;10:196-205.

22. Chen TL, Wong WW, Chiou TJ. Hematophagocytic syndrome: an unusual manifestation of acute human immunodeficiency virus infection. Int J Hematol 2003;78: 450-452.

23. Fardet L, Blum L, Kerob D, Aqbalika F, Galicier L, Dupuy A, et al. Human herpesvirus 8-associated lymphohistiocytosis in human immunodeficiency virus-infected patients. Clin Infect Dis 2003;37:285-291.

24. Grossman WJ, Radhi M, Schauer D, Gerday E, Grose C, Goldman FD. Development of hemophagocytic lymphohistiocytosis in triplets infected with HHV-8. Blood 2005;106:1203-1206.

25. Mou SS, Nakagawa TA, Riemer EC, McLean TW, Hines MH, Shetty AK. Hemophagocytic lymphohistiocytosis complicating influenza A infection. Pediatrics 2006;118: e216-e219.

26. Huang DB, Wu JJ, Hamill RJ. Reactive hemophagocytosis associated the initiation of highly active antiretroviral therapy (HAART) in a patient with AIDS. Scand J Infect Dis 2004;36:516-519. ¶·È‰È·ÙÚÈ΋ 2009;72:16-22

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·96

96

Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

27. Risdall RJ, McKenna RW, Nesbit ME, Krivit W, Balfour HH, Simmons RL, et al. Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer 1979;44:993-1002.

28. Filipovich AH. Hemophagocytic lymphohistiocytosis and related disorders. Cur Opin Allergy Clin Immunol 2006; 6:410-415.

29. Janka G, Imashuku S, Elinder G, Schneider M, Henter JL. Infection- and malignancy- associated hematophagocytic syndromes. Secondary hematophagocytic lymphohistiocytosis. Hematol/Oncol Clin North Am 1998;12:435-444.

30. Stanley J, Fallon-Pelicci V. Phenytoin hypersensitivity reaction. Arch Dermatol 1978;114:1350-1353.

31. Akiyoshi K, Hamada Y, Yamada H, Kojo M, Izumi T. Acute necrotizing encephalopathy associated with hemophagocytic syndrome. Pediatr Neurol 2006;34:315-318.

32. Falini B, Pileri S, De Solas I, Martelli MF, Mason DY, Delson G, et al. Peripheral T-Cell lymphoma associated with hemophagocytic syndrome. Blood 1990;75:434-444.

33. Okuda T, Sakamoto S, Deguchi T, Misawa S, Kashima K, Yoshihara T, et al. Hemophagocytic syndrome associated with agressive natular killer cell leukemia. Am J Hematol 1991;38:321-323.

34. Miyahara M, Sano M, Shibata K, Matsuzaki M, Ibaraki K, Shimamoto Y, et al. B-cell lymphoma-associated hemophagocytic syndrome: clinicopathological characteristics.

Ann Hematol 2000;79:378-388.

35. Shimazaki C, Inaba T, Nakagawa M. B-cell lymphomaassociated hemophagocytic syndrome. Leuk Lymphoma 2000;38:121-130.

36. Chuang HC, Lay JD, Hsieh WC, Su IJ. Pathogenesis and mechanism of disease progression from hemophagocytic lymphohistiocytosis to Epstein-Barr Virus-associated T-cell lymphoma: nuclear factor-kappa B pathway as a potential therapeutic target. Cancer Sci 2007;98:1281-1287.

37. Ezdinli EZ, Kucuk O, Chedid A, Sinclair TF, Thomas K, Singh S, et al. Hypogammaglobulinemia and hemophagocytic syndrome associated with lymphoproliferative disorders. Cancer 1986;57:1024-1037.

38. Janka G , Zur Stadt U. Familial and aquired hemophagocytic lymphohistiocytosis. Hamatology Am Soc Hematol Educ Program 2005:82-8.

39. Enders A, Zieger B, Schwarz K, Yoshimi A, Speckmann C, Knoepfle EM, et al. Lethal hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type II. Blood 2006;108:81-87.

40. Karras A, Thervet E, Legendre C. Hemophagocytic syndrome in renal transplant recipients: report of 17 cases and review of the literature. Transplantation 2004;77:238-243.

41. George TI, Jeng M, Berquist W, Cherry AM, Link MP, Arber DA. Epstein-Barr virus-associated peripheral T-cell lymphoma and hemophagocytic syndrome arising after liver transplantation: case report and review of the literature. Pediatr Blood Cancer 2005;44:270-276.

42. Dufourcq-Lagelouse R, Pastural E, Barrat FJ, Feldmann J, Le Deist F, Fisher A, et al. Genetic basis of hemophagocytic lymphohistiocytosis syndrome. Int J Mol Med 1999;4: 127-133.

43. Duval M, Fenneteau O, Doireau V, Faye A, Emilie D, Yotnda P, et al. Intermittent hemophagocytic lymphohistiocytosis is a regular feature of lysinuric protein intolerance. J Pediatr 1999;134:236-239.

44. Wu S, Gonzalez-Gomez I, Coates T, Yano S. Cobalamin C Paediatriki 2009;72:16-22

disease presenting with hemophagocytic lymphohistiocytosis. Pediatr Hematol Oncol 2005;22:717-721.

45. Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child 2001;85:421-426.

46. Behrens EM, Beukelman T, Paessler M, Cron RQ. Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol 2007;34:1133-1138.

47. Katoh N, Gono T, Mitsuhashi S, Fukushima K, Takei Y, Matsuda M, et al. Hemophagocytic syndrome associated with rheumatoid arthritis. Intern Med 2007;46:1809-1813.

48. Brinkman DM, de Kleer IM, ten Cate R, van Rossum Ma, Bekkering WP, Fasth A, et al. Autologus stem cell transplantation in children with severe progressive systemic or polyarticular juvenile idiopathic arthritis: longterm follow-up of a prospective clinical trial. Arthritis Rheum 2007;56:2410-2421.

49. Henter JI, Elinder G. Cerebromeningeal haemophagocytic lymphohistiocytosis. Lancet 1992;339:104-107.

50. Rooms L, Fitzgerald N, McClain KL. Haemophagocytic lymphohistiocytosis masquerading as child abuse: presentation of three cases and review of central nervous system findings in haemophagocytic lymphohistiocytosis.

Pediatrics 2003;111:e636-e640.

51. Henter JI, Elinder G, Söder O, Hansson M, Andersson B, Andersson U. Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood 1991;78:2918-2922.

52. Osugi Y, Hara J, Tagawa S, Takai K, Hosoi G, Matsuda Y, et al. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood 1997;89:4100-4103.

53. Astigarraga I, Prats JM, Navajas A, Fernandez-Teijeiro A, Urberuaga A. Near fatal cerebellar swelling in familial hemophagocytic lymphohistiocytosis. Pediatr Neurol 2004;30:361-364.

54. de Kerguenec C, Hillaire S, Molinie V, Gardin C, Deqott C, Erlinger S, et al. Hepatic manifestations of hemophagocytic syndrome: a study of 30 cases. Am J Gastroenterol 2001; 96:852-857.

55. Haddad E, Sulis ML, Jabado N, Blanche S, Fisher A, Tardieu M. Frequency and severity of central nervous system lesions in haemophagocytic lymphohistiocytosis.

Blood 1997;89:794-800.

56. Imashuku S, Iwai A. Isolated central nervous system haemophagocytic lymphohistiocytosis: case report. Neurosurgery 2006;58:e590.

57. Ishii H, Asai S, Suzuki T, Eguchi K, Miyachi H. Virusassociated hemophagocytic syndrome in an international traveler as a differential diagnosis of SARS. Internal Medicine 2005;44:342-345.

58. Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

Pediatr Blood Cancer 2007;48:124-131.

59. Imashuku S, Hibi S, Todo S. Hemophagocytic lymphohistiocytosis in infancy and childhood. J Pediatr 1997;130: 352-357.

60. Imashuku S, Kuriyama K, Teramura T, Ishii E, Kinugawa N, Kato M, et al. Requirement for etoposide in the treatement of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. J Clin Oncol 2001;19:2665-2673.

61. Tardieu M, Mikaeloff Y. Multiple Sclerosis in children. The International MS Journal 2004;11:36-42.

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¡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 ÙÂÏÂ˘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΋ ·ÓÙÈÌÂÙÒÈÛË ¤¯ÂÈ ‚ÂÏÙȈı›. ∆· ÓÂÔÁÓ¿ Ì ™¢∫ Û˘¯Ó¿ ¤¯Ô˘Ó ·Ó¿ÁÎË ÂÓÙ·ÙÈ΋˜ ıÂÚ·›·˜ 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 ˘‡ı˘ÓÔÈ ÁÈ· ÙËÓ Î·Î‹ ÚfiÁÓˆÛË Â›Ó·È Ë ˘ÔÏ·Û›· ÙˆÓ Ó¢ÌfiÓˆÓ Î·È Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÔ˘˜ È·ÙÚÔÁÂÓ›˜ ÙÚ·˘Ì·ÙÈÛÌÔ‡˜ Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi. ∂ÓÙÔ‡ÙÔȘ, ÓÂfiÙÂÚ· ‰Â‰Ô̤ӷ Ù˘ ·ıÔÊ˘ÛÈÔÏÔÁ›·˜ Ù˘ ÓfiÛÔ˘ Î·È ÙˆÓ Ì˯·ÓÈÛÌÒÓ Ô˘ ÂÚÌËÓÂ‡Ô˘Ó ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ· ¤¯Ô˘Ó Ô‰ËÁ‹ÛÂÈ Û ÛËÌ·ÓÙÈΤ˜ ·ÏÏ·Á¤˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÓÂÔÁÓÒÓ Ì ™¢∫.

§¤ÍÂȘ ÎÏÂȉȿ: ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÓÂÔÁÓfi, ıÂÚ·›·.

1 µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋, ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘», £ÂÛÛ·ÏÔÓ›ÎË 2 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌÂ›Ô «∞Ã∂¶∞», £ÂÛÛ·ÏÔÓ›ÎË AÏÏËÏÔÁÚ·Ê›·: ÃÚ‹ÛÙÔ˜ ∆۷ηϛ‰Ë˜ zoichr@otenet.gr µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋, ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘», £ÂÛÛ·ÏÔÓ›ÎË

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 2,000 of living births. Over the past two decades, antenatal diagnosis rates have increased, the pathophysiology 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 are persistent pulmonary hypertension and lung hypoplasia in combination with iatrogenic injuries caused by the mechanical ventilation. However, newer data of pathophysiology of the disease and the mechanisms of the respiratory distress have resulted in improving the treatment of newborns with CDH. Key words: Congenital diaphragmatic hernia, neonate, treatment.

∂ÈÛ·ÁˆÁ‹ ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) ·Ú·Ì¤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 ·ÓÙÈÌÂÙÒÈÛ‹ Ù˘, ÂÍ·ÎÔÏÔ˘ı› Ó· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ˘„ËÏ‹ ÓÔÛËÚfiÙËÙ· Î·È ıÓËÙfiÙËÙ·. ∏ ˘ÔÏ·Û›· ÙˆÓ Ó¢ÌfiÓˆÓ, Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË, Ë ‚ÈÔ¯ËÌÈ΋ Î·È ‰ÔÌÈ΋ ·ÓˆÚÈÌfiÙËÙ· 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 ÛËÌ·ÓÙÈÎfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ Â›Ó·È ˘‡ı˘ÓÔÈ ÁÈ· ÙË ÊÙˆ¯‹ ÚfiÁÓˆÛË. ∂ÓÙÔ‡ÙÔȘ, Ù· ÓÂfiÙÂÚ· ‰Â‰Ô̤ӷ Ù˘ ·ıÔÊ˘ÛÈÔÏÔÁ›·˜ Ù˘ ÓfiÛÔ˘ Î·È ÙˆÓ Ì˯·ÓÈÛÌÒÓ Ô˘ ÂÚÌËÓÂ‡Ô˘Ó ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿Ú-

1 2nd Department of Neonatal Paediatrics, “Aristotelio” University of Thessaloniki, “Papageorgiou” Hospital, Thessaloniki, Greece 2 2nd Department of Paediatrics, “Aristotelio” University of Thessaloniki, “AHEPA” Hospital, Thessaloniki, Greece Correspondence: Christos Tsakalidis zoichr@otenet.gr 2nd Department of Neonatal Paediatrics, “Aristotelio” University of Thessaloniki, “Papageorgiou” Hospital, Thessaloniki, Greece

ÎÂÈ· ¤¯Ô˘Ó Ô‰ËÁ‹ÛÂÈ Û ÛËÌ·ÓÙÈΤ˜ ·ÏÏ·Á¤˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÓÂÔÁÓÒÓ Ì ™¢∫.

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∂ÈÎfiÓ· 1. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË: ·) Ù‡Ô˘ Bochdalec, ‚) Ù‡Ô˘ Morgagni Î·È ÚfiÛıÈ· ‹ ÂÁοÚÛÈ· ™¢∫, Á) ÎÂÓÙÚÈ΋ ™¢∫.

√˘ÚÔÁÂÓÓËÙÈÎfi

Ì ÙËÓ ·ÚÈÛÙÂÚ‹ Ó· ·Ó¤Ú¯ÂÙ·È ÛÙÔ 73-90%, ÙË ‰ÂÍÈ¿ ÛÙÔ 8-24% Î·È ÙËÓ ·ÌÊÔÙÂÚfiÏ¢ÚË ‹ ÎÂÓÙÚÈ΋ ÛÙÔ 1,2-4,6% (3-5). √È ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ Ô˘ ‰ÂÓ ÂÓÙÔ›˙ÔÓÙ·È ÛÙ· Ï¿ÁÈ· Â›Ó·È Û¿ÓȘ Î·È ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 1-6% ÙˆÓ ·ÛıÂÓÒÓ. √È ÏÈÁfiÙÂÚÔ Û˘¯ÓÔ› Ù‡ÔÈ ™¢∫ ·ÊÔÚÔ‡Ó ÙËÓ ÔÈÛıÔÛÙÂÚÓÈ΋ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ‹ ΋ÏË ÙÔ˘ Morgagni, ÙËÓ ÚfiÛıÈ· ‹ ÂÁοÚÛÈ· ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÙËÓ ÎÂÓÙÚÈ΋ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÙËÓ ·Ó‡„ˆÛË ‹ ϤÙ˘ÓÛË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ Î·È ÙËÓ ·ÁÂÓÂÛ›· ·˘ÙÔ‡ (1) (∂ÈÎfiÓ· 1). ∫ËÏÈÎfi˜ Û¿ÎÔ˜ ·ÔÙÂÏÔ‡ÌÂÓÔ˜ ·fi ÏÂÙ‹ ÌÂÛÔıËÏȷ΋ ÌÂÌ‚Ú¿ÓË ÂÌÊ·Ó›˙ÂÙ·È Û ÏÈÁfiÙÂÚÔ ·fi 10% ÙˆÓ ÂÚÈÙÒÛˆÓ. ™ÙËÓ ·ÚÈÛÙÂÚ‹ ™¢∫, Ù· ÎÔÈÏȷο ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó ÛÙÔÓ ıÒڷη Â›Ó·È ÙÔ ÛÙÔÌ¿¯È, ÙÔ ÏÂÙfi ¤ÓÙÂÚÔ, ÙÔ ÎfiÏÔÓ, Ô ÛÏ‹Ó·˜ Î·È Ô ·ÚÈÛÙÂÚfi˜ ÏÔ‚fi˜ ÙÔ˘ ‹·ÙÔ˜. ¶ÂÚÈÛÙ·Ûȷο ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÙÔ ¿ÁÎÚ·˜, Ô ÓÂÊÚfi˜ Î·È ÙÔ ·ÚÈÛÙÂÚfi ÂÈÓÂÊÚ›‰ÈÔ. ™ÙË ‰ÂÍÈ¿ ™¢∫, Ôχ ÏÈÁfiÙÂÚÔ ¤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 ·ÔÙÂÏ› ÙÔ 8% fiÏˆÓ ÙˆÓ Û˘ÁÁÂÓÒÓ ‰˘ÛÏ·ÛÈÒÓ (6). ∆· ÂÚÈÛÛfiÙÂÚ· ÓÂÔÁÓ¿ Â›Ó·È ÙÂÏÂÈfiÌËÓ·. §›Á˜ ‰ËÌÔÛȇÛÂȘ ·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·ÊÔÚ¿ fiÛÔÓ ·ÊÔÚ¿ ÙÔ Ê‡ÏÔ (9). √È ·Ó·ÊÔÚ¤˜ ÂÚÈÛÙ·ÙÈÎÒÓ ÂÌÊ¿ÓÈÛ˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ Û ‰È‰‡ÌÔ˘˜, Û˘ÁÁÂÓ›˜ Î·È ·ÔÁfiÓÔ˘˜ ÙˆÓ ·ÛıÂÓÒÓ Â›Ó·È ÛÔÚ·‰ÈΤ˜. ™Â ÔÈÎÔÁ¤ÓÂȘ Ô˘ ·Ú·ÙËÚ‹ıËΠÏ‹Ú˘ ·ÁÂÓÂÛ›· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ‚Ú¤ıËΠÎÏËÚÔÓÔÌÈÎfi˜ ·˘ÙÔۈ̷ÙÈÎfi˜ ˘ÔÏÂÈfiÌÂÓÔ˜ ¯·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΋ ˘ÔÏ·Û›·, ·ÓÔȯÙfi˜ ·ÚÙËÚÈ·Îfi˜ fiÚÔ˜, ·ÓÔȯÙfi ˆÔÂȉ¤˜ ÙÚ‹Ì· Î·È ·ÙÂÏ‹˜ Û˘ÛÙÚÔÊ‹ ÙÔ˘ ÂÓÙ¤ÚÔ˘), ÂÚ›Ô˘ ÙÔ 40% ÙˆÓ ˙ÒÓÙˆÓ Paediatriki 2009;72:98-000

∫¡™ ™ÎÂÏÂÙÔ‡-ÚÔÛÒÔ˘

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·Î·˜ 1) (10,2). ™ÙÔ 60% ÙˆÓ ÂÚÈÙÒÛÂˆÓ 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 ÛÔÚ·‰ÈÎÒÓ Î·È ÛÙÔ 18% ÙˆÓ ÔÈÎÔÁÂÓÒÓ ÂÚÈÙÒÛÂˆÓ (11). ∞ӈ̷ϛ˜ ÙÔ˘ Á·ÛÙÚÂÓÙÂÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 17% ÙˆÓ ÂÚÈÙÒÛˆÓ, ÙÔ˘ ∫¡™ ÛÙÔ 14%, ÂÓÒ ÛÙÔ 10% Û˘Ó˘¿Ú¯Ô˘Ó ¯ÚˆÌÔۈ̷ÙÈΤ˜ ·ÓˆÌ·Ï›Â˜. ∆Ô Û‡Ó‰ÚÔÌÔ Ù˘ ˘ÔÏ·ÛÙÈ΋˜ ·ÚÈÛÙÂÚ‹˜ ηډ›·˜ ·ÚÔ˘ÛÈ¿˙ÂÙ·È Ì ¤Ó· ¢ڇ Ê¿ÛÌ· ·ÓˆÌ·ÏÈÒÓ, Ô˘ Î˘Ì·›ÓÂÙ·È ·fi ÙËÓ ˘ÔÏ·ÛÙÈ΋ ·ÚÈÛÙÂÚ‹ ηډȿ ¤ˆ˜ ÙËÓ ·Ó·ÙÔÌÈο Ê˘ÛÈÔÏÔÁÈ΋ ηډȿ, Ë ÔÔ›· fï˜ ÛÙÔ Û‡ÓÔÏfi Ù˘ Â›Ó·È ˘ÔÏ·ÛÙÈ΋ (3). ¢ÂÓ ¤¯ÂÈ ‰È¢ÎÚÈÓÈÛÙ› ÁÈ·Ù› Û˘Ì‚·›ÓÂÈ ·˘Ùfi. ª›· ıˆڛ· Â›Ó·È fiÙÈ Ë ÂÈÛÙÚÔÊ‹ ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡ ÊÏ‚ÈÎÔ‡ ·›Ì·ÙÔ˜ ‰È·Ì¤ÛÔ˘ ÙÔ˘ ˆÔÂȉԇ˜ ÙÚ‹Ì·ÙÔ˜ ÛÙËÓ ·ÚÈÛÙÂÚ‹ ÏÂ˘Ú¿ Ù˘ ηډȿ˜ ÌÔÚ› Ó· ÂÏ·ÙÙˆı› ·fi ÙË ÌÂÙ·ÙfiÈÛË Ù˘ ηډȿ˜ ̤۷ ÛÙÔÓ ıÒڷη, ÔfiÙÂ Ë ·ÏÏ·Á‹ ÛÙË ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜ ÌÔÚ› Ó· ÚÔηϤÛÂÈ ˘ÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ (12). ¢˘ÛÌÔÚÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο Î·È ·ÓˆÌ·Ï›Â˜ ÙˆÓ ¿ÎÚˆÓ Â›Ó·È Û˘¯ÓfiÙÂÚ· ÛÙËÓ ·ÚÈÛÙÂÚ¿ ÂÓÙÔÈ˙fiÌÂÓË ™¢∫ (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˜ ÌÂÈÒıËΠÛËÌ·ÓÙÈο Î·È ·˘Ùfi ÌÔÚ› 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›ÙˆÛË ÙˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ¤¯ÂÈ ·ÏÏ¿ÍÂÈ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· (9).

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·99

99

™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

¶›Ó·Î·˜ 2. ™‡Ó‰ÚÔÌ· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ñ ™‡Ó‰ÚÔÌÔ Fryns ñ ™‡Ó‰ÚÔÌÔ Beckwith-Wiedemann ñ ™‡Ó‰ÚÔÌÔ Brachman-de Lange ñ ™‡Ó‰ÚÔÌÔ Simpson-Golabi-Behmel ñ ™‡Ó‰ÚÔÌÔ Donnai ñ ™‡Ó‰ÚÔÌÔ Denys-Drash ñ ™‡Ó‰ÚÔÌÔ Perlman ñ ™‡Ó‰ÚÔÌÔ Craniofrontonasal ñ ™‡Ó‰ÚÔÌÔ Spondylocostal ñ ™‡Ó‰ÚÔÌÔ Marfan ñ ™‡Ó‰ÚÔÌÔ ™‡Ó‰ÚÔÌÔ Donnai-Barrow ñ ™‡Ó‰ÚÔÌÔ Gershoni-Baruch ñ ™‡Ó‰ÚÔÌÔ Matthew-Wood

°ÂÓÂÙÈ΋ ∏ ™¢∫ ¯ˆÚ›˜ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ÂÌÊ·Ó›˙ÂÙ·È ÛÔÚ·‰Èο. √ÈÎÔÁÂÓ›˜ ÂÚÈÙÒÛÂȘ ··ÓÙÔ‡Ó ÛÙÔ 2% (14,15), Û˘Ó‹ıˆ˜ ‰ÂÓ Û˘Óԉ‡ÔÓÙ·È ·fi ¿ÏϘ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ Î·È ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· ·ÌÊÔÙÂÚfiÏ¢Ú˘ ÂÓÙfiÈÛ˘. √ ΛӉ˘ÓÔ˜ Â·ÓÂÌÊ¿ÓÈÛ˘ ˘ÔÏÔÁ›˙ÂÙ·È Î·Ù¿ ÚÔÛ¤ÁÁÈÛË Û 2% Â› ·Ô˘Û›·˜ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ ÈÛÙÔÚÈÎÔ‡ (16). ∏ ™¢∫ ÌÔÚ› Ó· ··ÓÙ¿Ù·È ˆ˜ ̤ÚÔ˜ ÂÓfi˜ Û˘Ó‰ÚfiÌÔ˘ (17). ∆Ô˘Ï¿¯ÈÛÙÔÓ ÙÔ 10% ÙˆÓ ·ÛıÂÓÒÓ Ì ™¢∫ Î·È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ·Ó‹ÎÂÈ Û οÔÈÔ ÁÓˆÛÙfi Û‡Ó‰ÚÔÌÔ (18) (¶›Ó·Î·˜ 2), fiˆ˜ ÙÔ Û‡Ó‰ÚÔÌÔ Fryns (19,20). ¶Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ÛÙÔ ¯ÚˆÌfiۈ̷ 1q41-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˘Ó fiÙÈ ÛÙÔ ÛËÌÂ›Ô Û‡Ó‰ÂÛ˘ ÙˆÓ ÂÚÈÔ¯ÒÓ 1q41 Î·È 1q42.11 ‚Ú›ÛÎÂÙ·È Ô ÁÂÓÂÙÈÎfi˜ ÙfiÔ˜ Ô˘ Â›Ó·È ˘‡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 ‰ÈÏ·ÛÈ·ÛÌfi ÙÔ˘ 1q24q31.2, ÂÍ¿ÏÂÈ„Ë ÙÔ˘ ÙÂÏÈÎÔ‡ ÙÌ‹Ì·ÙÔ˜ ÙˆÓ 6q, 8p23.1, 15q26 ¯ÚˆÌ·ÙÔÛˆÌ¿ÙˆÓ Î·È ÌÂÚÈ΋ ÙÚÈۈ̛· 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·È Ì ™¢∫ ÂÌÊ·Ó›˙Ô˘Ó ÌÂÓ‰¤ÏÂÈ· ÎÏËÚÔÓÔÌÈÎfiÙËÙ· Î·È Û ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ Ë ÂÚÈÔ¯‹ Ô˘ ‰ڿ˙ÔÓÙ·È Ù· ÁÔÓ›‰È· ·˘Ù¿ ‹ Î·È Ë Ù·˘ÙfiÙËÙ· ÙˆÓ ÁÔÓȉ›ˆÓ ·˘ÙÒÓ Â›Ó·È ÁÓˆÛÙ¤˜. ∏ ‡·ÚÍË ÁÂÓÂÙÈÎÒÓ Û˘Ó‰ÚfiÌˆÓ 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· ÙȘ ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ WT1 ‚Ú¤ıËΠfiÙÈ ÔÈ ÌÂÙ·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 ‰ËÌÈÔ˘ÚÁ›· ™¢∫. ∫¿ÙÈ ·ÓÙ›ÛÙÔÈ¯Ô ‰ÂÓ ·ԉ›¯ıËΠ۠·ÓıÚÒÔ˘˜ (24). À¿Ú¯Ô˘Ó ·˘Í·ÓfiÌÂÓ· ÛÙÔȯ›· Ô˘ ‰Â›¯ÓÔ˘Ó fiÙÈ ÙÔ Ì·ÎÚ‡ ÛΤÏÔ˜ ÙÔ˘ ¯ÚˆÌ·ÙÔÛÒÌ·ÙÔ˜ 15 ‰È·‰Ú·Ì·Ù›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÂÌÊ¿ÓÈÛË ™¢∫ Î·È ÔÚÈÛÌ¤ÓˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ. ∏ ÂÚÈÔ¯‹ ·˘Ù‹ ÙÔ˘ ¯ÚˆÌ·ÙÔÛÒÌ·ÙÔ˜ 15 Έ‰ÈÎÔÔÈ› ÙËÓ Î˘ÙÙ·ÚÈ΋ ÚˆÙ½ÓË-1 Ô˘ ‰ÂÛ̇ÂÈ ÙÔ ÚÂÙÈÓÔ˚Îfi Ô͇ (25,26,27). √È ÙÚÈۈ̛˜ 13, 18, 21 Î·È 45Ã Â›Ó·È ÔÈ ÈÔ Û˘¯Ó¤˜ ·Ó¢ÏÔÂȉ›Â˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ™¢∫ (9). ∞ӈ̷ϛ˜ fiˆ˜ ÂÍ·Ï›„ÂȘ, ‰ÈÏ·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˜ ÛÔ˘‰·›Ô˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜, ÌÂ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ Ô˘ ··ÓÙÔ‡Ó ÛÙÔ 33% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (18). ∫·Ú˘fiÙ˘Ô˜ ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È Û οı ·È‰› Ì ™¢∫ 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· ηٷÓÔËÙ‹ ¤ˆ˜ Û‹ÌÂÚ·. ∏ ıˆڛ· Ù˘ ·ÔÙ˘¯Ë̤Ó˘ Û‡ÁÎÏÂÈÛ˘ Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ 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· ÌÂٷ͇ 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 ÏÂÎÈıÈÎfi Û¿ÎÔ ÂÚ›Ô˘ ÙË 10Ë Â‚‰ÔÌ¿‰· ·ËÛ˘ ηÈ, ·Ó ÊÙ¿ÛÂÈ ÚÈÓ ÙÔ ÎÏ›ÛÈÌÔ ÙÔ˘ ÙÚ‹Ì·ÙÔ˜, ‰ËÌÈÔ˘ÚÁÂ›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 ÂÈÙÚ¤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¢ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘¯‹˜. ¢Â‰Ô̤ӷ ·fi ¤Ú¢Ó˜ Û ˙Ò· Ô‰ËÁÔ‡Ó ÛÙËÓ ˘fiıÂÛË ˆ˜ ¤Ó· ÌË Ì˘˚Îfi, ÌÂÛÂÁ¯˘Ì·ÙÈÎfi ÛÙÔÈ¯Â›Ô Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ 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 ·ÓÂ¿ÚÎÂÈ· 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΋˜ ˘ÔÏ·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· Â›‰· ÚÂÙÈÓfiÏ˘ ÛÙÔ Ï¿ÛÌ·, ¶·È‰È·ÙÚÈ΋ 2009;72:99-000

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·100

100

Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

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 (FGF18) Ù˘ ÊÈÌÚÔÏ·ÛÙ›Ó˘, Ô˘ ÂÎÎÚ›ÓÔÓÙ·È ·fi ÙÔ ¿ˆ Ó¢ÌÔÓÈÎfi ÌÂÛ¤Á¯˘Ì·. √ ·Ú¿ÁÔÓÙ·˜ FGF-10 ÚÔ¿ÁÂÈ ÙË ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘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). ∏ ·ÓÂ¿ÚÎÂÈ¿ ÙÔ˘ ÌÔÚ› Ó· ·˘Í‹ÛÂÈ ÙËÓ Â˘¿ıÂÈ· ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙÔÓ ÙÚ·˘Ì·ÙÈÛÌfi ·fi Ù· ÂÓ‰ÔÎÔÈÏȷο ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó. √ ·Ú¿ÁÔÓÙ·˜ FGF-18 ÚÔ¿ÁÂÈ ÙËÓ ·Ú·ÁˆÁ‹ ÂÏ·ÛÙÈÎÒÓ ÈÓÒÓ Î·È Ë ·ÓÂ¿ÚÎÂÈ¿ ÙÔ˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ÂÏ·Ùو̤ÓË ·Ú·ÁˆÁ‹ ·˘ÙÒÓ (34). √ Ì˯·ÓÈÛÌfi˜ Ù˘ Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜ ÛÙË ™¢∫ ·ÔÙÂÏ› ÛËÌÂ›Ô ‰È¯ÔÁӈ̛·˜. ™‡Ìʈӷ Ì ÙËÓ ÈÔ ·ԉ¯ً ¿Ô„Ë, ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÓ‰ÔÌ‹ÙÚÈ·˜ ·Ó¿Ù˘Í˘, Ù· ÂÓÙfi˜ ÙÔ˘ ıÒڷη ÎÔÈÏȷο ÛÏ¿¯Ó· Ȥ˙Ô˘Ó ÙÔÓ Û‡ÛÙÔÈ¯Ô Ó‡ÌÔÓ·, ÁÂÁÔÓfi˜ Ô˘ ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ Û ÌÈÎÚfiÙÂÚÔ ‚·ıÌfi Î·È ÙÔÓ ÂÙÂÚfiÏ¢ÚÔ Ó‡ÌÔÓ·, ÏfiÁˆ Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜ ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘ ÛÙÔ ¤Ì‚Ú˘Ô. ∞˘Ù‹ Ë ¿Ô„Ë ¤¯ÂÈ ÂȂ‚·Èˆı› ÂÈÚ·Ì·ÙÈο Û ÔÓÙÈÎÔ‡˜ Î·È ·ÌÓÔ‡˜ (35). ™‡Ìʈӷ Ì ÙËÓ ˘fiıÂÛË ÙˆÓ «‰‡Ô ¯Ù˘ËÌ¿ÙˆÓ», ÙÔ «ÚÒÙÔ ¯Ù‡ËÌ·» Â¤Ú¯ÂÙ·È ÓˆÚ›˜ ÛÙËÓ ·Ó¿Ù˘ÍË Î·È ÂËÚ¿˙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·ÁÌ·, Ô˘ Ì·˙› Ì ٷ ÂÓ‰ÔÎÔÈÏȷο ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó ÂËÚ¿˙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˘ÌÙÒÌ·Ù· ÂÌÊ·Ó›˙ÔÓÙ·È ÓˆÚ›˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, Ë ˘ÔÏ·Û›· Î·È ÙˆÓ ‰‡Ô Ó¢ÌfiÓˆÓ Â›Ó·È ¤Ó· ÛÙ·ıÂÚfi ‡ÚËÌ·. ∏ ·ÌÊÔÙÂÚfiÏ¢ÚË Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· Â›Ó·È ÛËÌ·ÓÙÈο ·˘ÍË̤ÓË Û ·ÛıÂÓ›˜ Ì ‰ÂÍÈ¿ ™¢∫. √È Ó‡ÌÔÓ˜ ¤¯Ô˘Ó ÂÏ·Ùو̤ÓÔ ·ÚÈıÌfi ‚ÚfiÁ¯ˆÓ, ÏÈÁfiÙÂÚ· ‚ÚÔÁ¯ÈfiÏÈ· (Ù˘Èο 6-8 ÁÂÓ¤˜ ‚ÚfiÁ¯ˆÓ, ¤Ó·ÓÙÈ ÙˆÓ 15 ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÓÂÔÁ¤ÓÓËÙˆÓ ‚ÚÂÊÒÓ) Î·È ÂÏ·Ùو̤ÓË Î˘„ÂÏȉÈ΋ ÂÈÊ¿ÓÂÈ· (37). ∏ ·ÁÁ›ˆÛË ÙˆÓ Ó¢ÌfiÓˆÓ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·‡ÍËÛË ÙÔ˘ ¿¯Ô˘˜ ÙÔ˘ Ï›Ԣ Ì˘˚ÎÔ‡ ¯ÈÙÒÓ· ÙˆÓ ·ÁÁ›ˆÓ, Ë ÔÔ›· ÂÂÎÙ›ÓÂÙ·È ¤ˆ˜ Î·È ÙÔ Â›Â‰Ô ÙˆÓ ÙÚȯÔÂȉÒÓ ÙˆÓ Î˘„ÂÏ›‰ˆÓ. √ ·ÚÈıÌfi˜ ÙˆÓ ·ÚÙËÚȉ›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 ¿ÌÂPaediatriki 2009;72:100-000

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΋ ˘ÔÏ·Û›· (39,40). ∏ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÂÏ·ÙÙÒÓÂÙ·È ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ ·fi ÙËÓ ·˘Í·ÓfiÌÂÓË ›ÂÛË ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›·, Ì ·ÔÙ¤ÏÂÛÌ· ÙË ‰ÂÍÈÔ-·ÚÈÛÙÂÚ‹ ‰È·Ê˘Á‹ (41). ™Ù· ÓÂÔÁÓ¿ ·˘Ù¿, Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Â›Ó·È Û¯Â‰fiÓ Î·ıÔÏÈÎfi ‡ÚËÌ· Î·È Ë ·Ó·Ó¢ÛÙÈ΋ ‹ ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË ÙËÓ ÂȉÂÈÓÒÓÂÈ. ∏ 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 ˘ÔÏ·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 ÚfiÛÊ·Ù·, ÛÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· (45). ™ÙÔÓ Ó¢ÌÔÓÈÎfi ÈÛÙfi ÓÂÔÁÓÒÓ Ì ™¢∫, Ë Û˘ÁΤÓÙÚˆÛË ÊˆÛÊ·ÙȉԯÔÏ›Ó˘ Â›Ó·È ¯·ÌËÏfiÙÂÚË ÛÙ· ÂÚÈÛÛfiÙÂÚ· ¿ÙÔÌ· (44). ™ÙÔ ·ÌÓÈ·Îfi ˘ÁÚfi ¤¯Ô˘Ó ·Ó·ÊÂÚı› ÌÂȈ̤ÓË ·Ó·ÏÔÁ›· ÏÂÎÈı›Ó˘/ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ Î·È ÂÏ·Ùو̤Ó˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ʈÛÊ·ÙȉԯÔÏ›Ó˘ Î·È ÚˆÙ½Ó˘ ∞ ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· (46,47), ÂÓÒ ¿ÏÏÔÈ ÂÚ¢ÓËÙ¤˜ ·Ó·Ê¤ÚÔ˘Ó Ê˘ÛÈÔÏÔÁÈΤ˜ ·Ó·ÏÔÁ›Â˜ ÏÂÎÈı›Ó˘/ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ Î·È Ê˘ÛÈÔÏÔÁÈ΋ Û˘ÁΤÓÙÚˆÛË Ù˘ ʈÛÊ·ÙȉÔÁÏ˘ÎÂÚfiÏ˘ (48). ™ÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· ÓÂÔÁÓÒÓ Ì ™¢∫, 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·ÚÙ‡ÚˆÓ (45) Î·È ÚÔÙ›ÓÂÈ fiÙÈ ÌÈ· ÚfiˆÚË ·ÓÂ¿ÚÎÂÈ· ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· Â›Ó·È Ì¿ÏÏÔÓ ·›ı·ÓË ÛÙ· ‚Ú¤ÊË Ì ™¢∫. ÕÏÏË ÌÂϤÙË (49) ·Ó·Ê¤ÚÂÈ fiÙÈ Ù· ÓÂÔÁÓ¿ Ì ™¢∫ ¤¯Ô˘Ó ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ· Â›‰· ʈÛÊ·ÙȉÔÁÏ˘ÎÂÚfiÏ˘ Î·È ÚˆÙ½Ó˘ ∞ ÛÙÔ ˘ÏÈÎfi ·fi ·Ó·ÚÚfiÊËÛË Ù˘ ÙÚ·¯Â›·˜. ∞˘Ù¿ Ù· Â›‰· ÌÂÈÒÓÔÓÙ·È ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘, Û ۇÁÎÚÈÛË Ì ·˘Ù¿ ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÓÂÔÁÓÒÓ ›‰È·˜ ËÏÈΛ·˜ Ô˘ ·ÔÙ¤ÏÂÛ·Ó ÙËÓ ÔÌ¿‰· ÙˆÓ Ì·ÚÙ‡ÚˆÓ. ∞˘Ù¿ Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ‰ËÏÒÓÔ˘Ó ÙËÓ ‡·ÚÍË ·ÓÂ¿ÚÎÂÈ·˜ ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· Û ·ÛıÂÓ›˜ Ì ™¢∫, Ë ÔÔ›· ı· ÌÔÚÔ‡Û ӷ ÔÊ›ÏÂÙ·È ÛÙËÓ ‡·ÚÍË ÌÈÎÚfiÙÂÚ˘ ΢„ÂÏȉÈ΋˜ ÂÈÊ¿ÓÂÈ·˜ ÛÙÔ˘˜ ÂËÚ·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¿ÓÈÛË ™¢∫, fiˆ˜ Ë ı·ÏȉÔÌ›‰Ë, Ë ÎÈÓ›ÓË Î·È Ù· ·ÓÙÈÂÈÏËÙÈο (51,52). ∂›Û˘, ™¢∫ ¤¯ÂÈ ·Ó·ÊÂÚı› Û ÓÂÔÁÓfi ‰È·‚ËÙÈ΋˜ ÌËÙ¤Ú·˜ (53). ∏ ¯ÔÚ‹ÁËÛË ÔÚÌÔÓÒÓ ÌÔÚ› Ó· Â›Ó·È ÛËÌ·ÓÙÈ΋. ∆· ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ‹

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·101

101

™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

ÚÔ¿ÁÔ˘Ó ÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ Ó¢ÌfiÓˆÓ Î·È ÛÙ·ıÂÚÔÔÈÔ‡Ó ÙÔ Ó¢ÌÔÓÈÎfi ·ÁÁÂÈ·Îfi ÙÔ›¯ˆÌ·, ÚÔηÏÒÓÙ·˜ ·‡ÍËÛË ÙÔ˘ ¿¯Ô˘˜ ÙÔ˘ ÙÔȯÒÌ·Ùfi˜ ÙÔ˘˜ ÛÙ· ˙Ò· Ì ™¢∫ Î·È ·˘Í¿ÓÔ˘Ó ÙÔ˘˜ ˘ԉԯ›˜ 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 ˘ÂÚ˯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·›ÓÂÙ·È ·fi 29-100% (56,57).∆Ô ÔÛÔÛÙfi ·˘Ùfi ·˘Í¿ÓÂÙ·È ÛÙÔ 72% fiÙ·Ó Û˘Ó˘¿Ú¯Ô˘Ó ÂÈÚfiÛıÂÙ˜ ·ÓˆÌ·Ï›Â˜ ‹ ·ıÔÏÔÁÈÎfi˜ ηڢfiÙ˘Ô˜ (56). ŒÓ· ÛËÌ·ÓÙÈÎfi˜ ·ÚÈıÌfi˜ ™¢∫ ‰È·ÁÈÁÓÒÛÎÂÙ·È ÌÂÙ¿ ÙËÓ 24Ë Â‚‰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 ·ÌÊÔÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË ·ÚÔ˘ÛÈ¿˙Ô˘Ó Û˘¯ÓfiÙÂÚ· Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜, Ô˘ Ô‰ËÁÔ‡Ó Û ˘„ËÏfiÙÂÚÔ ÔÛÔÛÙfi ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Î·È Ùˆ¯‹ ¤Î‚·ÛË (55). ∏ ™¢∫ ‰È·ÁÈÁÓÒÛÎÂÙ·È ˘ÂÚ˯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˜ ηډȷÎfi˜ ¿ÍÔÓ·˜ Î·È Ë ÌÂÙ·ÙfiÈÛË ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘. ¶ÂÚÈÛÙ·ÏÙÈΤ˜ ÎÈÓ‹ÛÂȘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Î·È ÙÔ˘ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘ ÂÓÙfi˜ ÙÔ˘ ıÒڷη ÌÔÚ› Ó· Á›ÓÔ˘Ó ·ÓÙÈÏËÙ¤˜ ˘ÂÚ˯ÔÁÚ·ÊÈο ÛÙ· ÙÂÏÈο ÛÙ¿‰È· Ù˘ ·ËÛ˘. √È ‰È·ÊÚ·ÁÌ·ÙÔ΋Ϙ Ô˘ ÂÓÙÔ›˙ÔÓÙ·È ‰ÂÍÈ¿ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ÚÔÁÂÓÓËÙÈο ÈÔ ‰‡ÛÎÔÏ·, ‰ÈfiÙÈ ÙÔ ‹·Ú ¤¯ÂÈ ·ÚfiÌÔÈ· ˯ÔÁ¤ÓÂÈ· Ì ÙÔ˘˜ ÂÌ‚Ú˘˚ÎÔ‡˜ Ó‡ÌÔÓ˜ Î·È ÌÔÚ› 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˜ ÌÔÚ› 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˘ ÓÂÔÁÓÈ΋˜ ÂÚÈfi‰Ô˘ (58,59) Î·È ÌÔÚ› 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 ÂȉÂÈÓÒÓÂÙ·È. ∏ ÚfiÁÓˆÛË ÂÍ·ÚÙ¿Ù·È ·fi ÙÔÓ ‚·ıÌfi Ù˘ Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜ (61) Î·È ÙË Û˘Ó˘¿Ú¯Ô˘Û· ÂÏ¿ÙÙˆÛË ÙˆÓ Î˘„ÂÏȉÈÎÒÓ Î·È ·ÁÁÂÈ·ÎÒÓ ÂÈÊ·ÓÂÈÒÓ. ªÔÚ› Ó· Û˘Ó˘¿Ú¯ÂÈ ÛÔ‚·Ú‹ ˘ÂÚηÓ›· Î·È ˘ÔÍ·ÈÌ›·, Ì·˙› ÌÂ

‚·ÚÈ¿ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË, Ì ·ÔÙ¤ÏÂÛÌ· ‰ÂÍÈÔ-·ÚÈÛÙÂÚ‹ ‰È·Ê˘Á‹ ÛÙÔ Â›Â‰Ô ÙˆÓ ÎfiÏˆÓ Î·È ÙÔ˘ ·ÚÙËÚÈ·ÎÔ‡ fiÚÔ˘. ∆· Û˘¯ÓfiÙÂÚ· Û˘ÌÙÒÌ·Ù· ·ÊÔÚÔ‡Ó ÙÔ ·Ó·Ó¢ÛÙÈÎfi (43%), ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi (33%), ÙÔ ·Ó·Ó¢ÛÙÈÎfi Î·È Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ· (13%), ÂÓÒ ·Û˘Ìو̷ÙÈΤ˜ ÂÚÈÙÒÛÂȘ ·Ó·Ê¤ÚÔÓÙ·È Û ÔÛÔÛÙfi 11%. °È· Ù· ÓÂÔÁÓ¿ ÛÙ· ÔÔ›· ‰ÂÓ Á›ÓÂÙ·È Ë ‰È¿ÁÓˆÛË ÂÓ‰ÔÌ‹ÙÚÈ·, Ë ¤Ó·ÚÍË ÙˆÓ Û˘ÌÙˆÌ¿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˘Ìو̷ÙÔÏÔÁ›· ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙËÓ ·›ıÔ˘Û· ÙÔÎÂÙÔ‡ Ì ‰˘Û¯¤ÚÂÈ· ÛÙËÓ ·Ó¿ÓË„Ë Î·È Ì ˘„ËÏ¿ ÔÛÔÛÙ¿ Ó¢ÌÔıÒڷη. √È ·Ó·Ó¢ÛÙÈÎÔ› ‹¯ÔÈ Â›Ó·È ·fiÓÙ˜ ÛÙËÓ ¿Û¯Ô˘Û· ÏÂ˘Ú¿, Ô ıÒڷη˜ ¤¯ÂÈ Î˘ÏÈÓ‰ÚÈÎfi Û¯‹Ì·, Ë ÎÔÈÏÈ¿ Â›Ó·È ÛηÊÔÂȉ‹˜ Î·È ÔÈ Î·Ú‰È·ÎÔ› ÙfiÓÔÈ ÌÂÙ·ÙÔ›˙ÔÓÙ·È ‰ÂÍÈ¿. ∏ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ‰ÂÍÈ¿ ÂÓÙÔÈ˙fiÌÂÓˆÓ ™¢∫ ÂΉËÏÒÓÂÙ·È ÌÂ Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi (62). ∏ Û‡ÁÎÏÂÈÛË ÙÔ˘ ·Ó·ÙÔÌÈÎÔ‡ ÂÏÏ›ÌÌ·ÙÔ˜ ·fi ÙÔ ‹·Ú ·ÔÙÚ¤ÂÈ ÙË ÌÂÙ¤ÂÈÙ· ÚfiÙˆÛË ÎÔ›ÏˆÓ ÔÚÁ¿ÓˆÓ Î·È ÙËÓ ÂÌÊ¿ÓÈÛË Û˘ÌÙˆÌ¿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˘ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi ‹ Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ· (60,62). ¢˘ÛÎÔÏ›· ÛÙËÓ ·ÎÙÈÓÔÏÔÁÈ΋ ‰È¿ÁÓˆÛË ··ÓÙ¿ Û ÔÛÔÛÙfi 25%, Ì ÙÔÓ Ó¢ÌÔıÒڷη Î·È ÙÔÓ ˘‰ÚÔıÒڷη ˆ˜ ÈÔ ÎÔÈÓ¤˜ Ï·Óı·Ṳ̂Ó˜ ‰È·ÁÓÒÛÂȘ (62). ∏ ÙÔÔı¤ÙËÛË ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎÔ‡ ηıÂÙ‹Ú· ÁÈ· ÙË ‰È·›ÛÙˆÛË Ù˘ ı¤Û˘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Î·È Ô ·ÎÙÈÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì ÛÎÈ·ÛÙÈÎfi ÙÔ˘ ·ÓÒÙÂÚÔ˘ ÂÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ‚ÔËıÔ‡Ó ÛÙË ÛˆÛÙ‹ ‰È¿ÁÓˆÛË Ù˘ ·ÚÈÛÙÂÚ‹˜ ™¢∫. ∏ CT ıÒڷη Â›Ó·È ¯Ú‹ÛÈÌË 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‹˜ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ ÛÙËÓ ÂÌÊ¿ÓÈÛË Û˘ÁÁÂÓÒÓ ·ÓˆÌ·ÏÈÒÓ, fiˆ˜ Û˘ÁÁÂÓ‹ ·‰Âӈ̷ÙÒ‰Ë Î˘ÛÙÈ΋ ‰˘ÛÏ·Û›·, Û˘ÁÁÂÓ¤˜ ÏԂ҉˜ 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 ÌÔÚ› 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. ¶·È‰È·ÙÚÈ΋ 2009;72:101-000

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·102

102

Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

£ÂÚ·›· ∏ ‡·ÚÍË Â͈ۈ̷ÙÈ΋˜ ÌÂÌ‚Ú¿Ó˘ Ô͢ÁfiÓˆÛ˘ (ECMO), Ë ‰˘Ó·ÙfiÙËÙ· ÚÔÂÁ¯ÂÈÚËÙÈ΋˜ ÛÙ·ıÂÚÔÔ›ËÛ˘ ÙÔ˘ ÓÂÔÁÓÔ‡ Î·È Ë ÚfiÔ‰Ô˜ fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÂÓ‰ÔÌ‹ÙÚÈÔ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ÛÙËÚ›˙Ô˘Ó ÙËÓ ÚÔÛ¿ıÂÈ· ÌË ÂÈıÂÙÈ΋˜ ·ÔηٿÛÙ·Û˘ ÙˆÓ ‚Ï·‚ÒÓ. ¶·Ï·ÈfiÙÂÚ·, Ë ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ıˆÚÔ‡ÓÙ·Ó Â›ÁÔ˘Û· ηٿÛÙ·ÛË, ÛÙËÓ ÔÔ›· ›¯Â ·fiÏ˘ÙË ¤Ó‰ÂÈÍË Ë ¿ÌÂÛË ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ú¤Ì‚·ÛË, ÒÛÙ ӷ ÂÈÙ¢¯ı› ÙÔ Î·Ï‡ÙÂÚÔ ‰˘Ó·Ùfi ·ÔÙ¤ÏÂÛÌ·. ∞˘Ù‹ Ë ÛÙÚ·ÙËÁÈ΋ ¤¯ÂÈ Â·ÓÂÎÙÈÌËı›, ÏfiÁˆ Ù˘ ηٷÓfiËÛ˘ ÙÔ˘ ÚfiÏÔ˘ Ù˘ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· Î·È ÙËÓ Â›‰Ú·ÛË Ù˘ ¯ÂÈÚÔ˘ÚÁÈ΋˜ ·Ú¤Ì‚·Û˘ ÛÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›·. ∂›Ó·È ϤÔÓ ÍÂοı·ÚÔ fiÙÈ ÙÔ Î‡ÚÈÔ Úfi‚ÏËÌ· Ô˘ ÚÔηÏ› ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ· Â›Ó·È Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Î·È Ë ˘ÔÏ·Û›· ÙˆÓ Ó¢ÌfiÓˆÓ Î·È fi¯È Ë Û˘Ì›ÂÛË Ô˘ ÚÔηÏÔ‡Ó Ù· ÂÓ‰ÔÂÚÈÙÔÓ·˚ο ÛÏ¿¯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˘ ÙÔÔıÂÙÂ›Ù·È ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎfi˜ ηıÂÙ‹Ú·˜ Î·È 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˜ ·ÔÙÂÏ› ÙË ‚¿ÛË Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ™¢∫. ™ÙËÓ ÚÔÛ¿ıÂÈ· ÂÏ¿ÙÙˆÛ˘ ÙÔ˘ ‚·ÚÔÙÚ·‡Ì·ÙÔ˜ Î·È ÙÔ˘ Ó¢ÌÔıÒڷη ÚÔÙÈÌ¿Ù·È Ô ‹ÈÔ˜ ·ÂÚÈÛÌfi˜ (63,65). ™Â ÂÚÈÙÒÛÂȘ ·ÓıÂÎÙÈ΋˜ ·Ó·Ó¢ÛÙÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜ Î·È ·Ú·Ì¤ÓÔ˘Û·˜ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ ÂӉ›ÎÓ˘Ù·È Ë ÂÊ·ÚÌÔÁ‹ ˘„›Û˘¯ÓÔ˘ ·ÂÚÈÛÌÔ‡, ÌfiÓÔ˘ ‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÂÈÛÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘ (65). °È· Ù· ÓÂÔÁÓ¿ Ì ‚·ÚÈ¿ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ·, Ì ÌÂÁ¿ÏË ˘ÔÛÙ‹ÚÈÍË ÛÙÔÓ Û˘Ì‚·ÙÈÎfi Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi, Ô˘ ÏËÚÔ‡Ó Ù· ÎÚÈÙ‹ÚÈ· ÁÈ· ECMO, Ô ‚·ıÌfi˜ ÂÈÙ˘¯›·˜ ÙÔ˘ ˘„›Û˘¯ÓÔ˘ ·ÂÚÈÛÌÔ‡ ÚÈÓ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ECMO ÊÙ¿ÓÂÈ ÙÔ 33% (67). ∆Ô ÂÈÛÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘ ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› Â›Û˘ Ì ÂÈÙ˘¯›· ÛÙË ıÂÚ·›· Ù˘ ‚·ÚÈ¿˜ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ ÙÔ˘ ÓÂÔÁÓÔ‡ Î·È ‰È¿ÊÔÚÔÈ ÂÚ¢ÓËÙ¤˜ ‰ÔÎ›Ì·Û·Ó ·˘Ù‹Ó ÙË ıÂÚ·›· Û ÓÂÔÁÓ¿ Ì ÛÔ‚·Ú‹ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Ô˘ ÂÈϤÎÂÈ ÙËÓ ÔÚ›· Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ (64). ∏ ¯ÔÚ‹ÁËÛË ÓÙÔ·Ì›Ó˘ ‹ ÓÙÔÌÔ˘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˘ ÌÔÚ› Ó· Ê·Ó› ¯Ú‹ÛÈÌË. ¶¿ÓÙˆ˜, Ô Â·ÚPaediatriki 2009;72:102-000

΋˜ ·ÂÚÈÛÌfi˜, Ë Ô͢ÁfiÓˆÛË Î·È ÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ·˘Í¿ÓÔ˘Ó ÙË Û˘ÛÙËÌ·ÙÈ΋ ›ÂÛË ·ÔÙÂÏÔ‡Ó ÙËÓ Â·Ú΋ ıÂÚ·›·. ∏ ¯ÔÚ‹ÁËÛË ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ÌÔÚ›, fi¯È ÌfiÓÔ Ó· ·ÓÙÈÌÂÙˆ›ÛÂÈ ÙËÓ ·ÓÂ¿ÚÎÂÈ¿ ÙÔ˘, ·ÏÏ¿ Î·È Ó· ‚ÂÏÙÈÒÛÂÈ ÙËÓ Â˘ÂÓ‰ÔÙfiÙËÙ·, Ó· ÂÏ·ÙÙÒÛÂÈ ÙȘ Ó¢ÌÔÓÈΤ˜ ·ÁÁÂȷΤ˜ ·ÓÙÈÛÙ¿ÛÂȘ Î·È Ó· ‚ÂÏÙÈÒÛÂÈ ÙË ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜ ÛÙÔÓ Ó‡ÌÔÓ· (68,69). ∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ™¢∫ ‰ÂÓ Â›Ó·È ¿ÌÔÈÚË ÂÈÏÔÎÒÓ. ¶Ó¢ÌÔıÒڷη˜ ÛÙËÓ ·ÓÙ›ıÂÙË ÏÂ˘Ú¿ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙ› Ï›Á· ÏÂÙ¿ ÌÂÙ¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ·ÂÚÈÛÌÔ‡ Ì ˘„ËϤ˜ ȤÛÂȘ. ∆· ÓÂÔÁÓ¿ Ì ˘ÔÏ·ÛÙÈÎÔ‡˜ Ó‡ÌÔÓ˜ Â›Ó·È ÈÔ ÂÈÚÚÂ‹ ÛÙËÓ ·Ó¿Ù˘ÍË Ó¢ÌÔıÒڷη. ÕÌÂÛË ·Ó Î·È Û¿ÓÈ· ÌÂÙÂÁ¯ÂÈÚËÙÈ΋ ÂÈÏÔ΋ ·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 Â˘Ú‹Ì·Ù·, Ù· ÔÔ›· ÔÚÈÔıÂÙÔ‡Ó ÙË ÛÔ‚·ÚfiÙËÙ· Ù˘ ™¢∫ Î·È ·ÔÙÂÏÔ‡Ó ÙË ‚¿ÛË Ù˘ ÂÓË̤ڈÛ˘ Î·È ÚÔÁÂÓÓËÙÈ΋˜ ηıÔ‰‹ÁËÛ˘ ÙˆÓ ÁÔÓ¤ˆÓ. ∏ ¤Î‚·ÛË ÂÍ·ÚÙ¿Ù·È ·fi ·Ú¿ÁÔÓÙ˜ fiˆ˜ Ë ·ÚÔ˘Û›· Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ, ÙÔ ÛÙ¿‰ÈÔ Î‡ËÛ˘ ÛÙÔ ÔÔ›Ô Û˘Ó¤‚Ë Ë ÚfiÙˆÛË ÙˆÓ ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÔÚÁ¿ÓˆÓ ÂÓÙfi˜ ÙÔ˘ ıÒڷη, Ô fiÁÎÔ˜ ÙˆÓ ÛÏ¿Á¯ÓˆÓ Ô˘ ÚÔÛÙ›ÙÔ˘Ó ÂÓÙfi˜ ÙÔ˘ ıÒڷη Î·È Ô ‚·ıÌfi˜ Ù˘ Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜ Î·È ÙˆÓ Î·Ú‰È·ÁÁÂÈ·ÎÒÓ ·ÏÏ·ÁÒÓ Ô˘ ¤¯Ô˘Ó ÂÈÛ˘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› ÚÔÁÓˆÛÙÈÎÔ› ‰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 ÂÈ‚›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‰ÔıˆÚ·ÎÈÎfi fiÁÎÔ), Ë ÚfiÙˆÛË 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 ÂÚÈÙÒÛÂˆÓ ¤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 Ùˆ¯‹ ÚfiÁÓˆÛË) Î·È Ô ÏfiÁÔ˜ Ó‡ÌÔÓ· ÚÔ˜ ÎÂÊ·Ï‹ (lung to head ratio, LHR) ÌÈÎÚfiÙÂÚÔ˜ ·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˘ ÂÈÊ¿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›˙ÂÙ·È Ó· Â›Ó·È Ô Î·Ï‡ÙÂÚÔ˜ ÚÔÁÓˆÛÙÈÎfi˜ ‰Â›ÎÙ˘.

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·103

103

™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

¶›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¿ÊËÌ· Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ñ ¶ÚÔÁÂÓÓËÙÈ΋ ÂÎÙ›ÌËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ - ∂Ó‰ÔıˆÚ·ÎÈ΋ ÂÓÙfiÈÛË ÙÔ˘ ÛÙÔÌ¿¯Ô˘ ÛÙËÓ ·ÚÈÛÙÂÚ‹ ™¢∫ - ¶ÚfiÙˆÛË ÙÔ˘ ‹·ÙÔ˜ - ¶ÚfiÙˆÛË ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ - ¶ÚÒÈÌË ‰È¿ÁÓˆÛË Ù˘ ™¢∫ (<25Ë Â‚‰ÔÌ¿‰· ·ËÛ˘) - ªÂÙ·ÙfiÈÛË ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘ - ¢È¿ÌÂÙÚÔ˜ Ù˘ ÎÔÈÏÈ¿˜ ñ ™˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ñ ÃÚˆÌÔۈ̷ÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ñ ¶ÔÏ˘˘‰Ú¿ÌÓÈÔ ñ À‰ÚÔıÒڷη˜ ñ ÀÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ηډ›·˜ ñ ¶Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·

LHR <1 Û¯ÂÙ›˙ÂÙ·È Ì ıÓËÙfiÙËÙ· 100%, ÂÓÒ LHR >1,4 Û¯ÂÙ›˙ÂÙ·È Ì ÂÈ‚›ˆÛË 100% (75,76). ∆Ô ÚÔÁÂÓÓËÙÈÎfi ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜ ÌÔÚ› Ó· ·Ó·ÁÓˆÚ›ÛÂÈ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ Ù˘ ηډȿ˜. ¶·Ú¿ÁÔÓÙ˜ Ùˆ¯‹˜ ¤Î‚·Û˘ ıˆÚÔ‡ÓÙ·È Ë ˘ÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ (77) Î·È Ô ·˘ÍË̤ÓÔ˜ 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 ·ÂÈÎfiÓÈÛË ÙˆÓ Ì·Ï·ÎÒÓ ÈÛÙÒÓ, ÌÔÚ› Ó· ÂȂ‚·ÈÒÛÂÈ ÙËÓ ·ÚÔ˘Û›· Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ (79) Î·È Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈÎfi˜ ÛÙË ‰È¿ÁÓˆÛË ‰ÂÍÈ¿˜ ™¢∫ Ì ÚfiÙˆÛË ÙÔ˘ ‹·ÙÔ˜. ∞ÚÎÂÙÔ› ÌÂÙ·ÁÂÓÓËÙÈÎÔ› ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ¤¯Ô˘Ó ÂÚ¢ÓËı›, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤ÓÔ˘ Î·È ÙÔ˘ Ùˆ¯Ô‡ ·ÂÚÈÛÌÔ‡ ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη (80).

¡ÔÛËÚfiÙËÙ· ∂ÎÙÈÌ¿Ù·È fiÙÈ ÏÈÁfiÙÂÚÔ ·fi ÙÔ 1/3 ÙˆÓ ÂÌ‚Ú‡ˆÓ Ì ™¢∫ Ô˘ ‰ÈÂÁÓÒÛıË ÚÔÁÂÓÓËÙÈο ı· ÂÈ‚ÈÒÛÂÈ ¯ˆÚ›˜ ·˘ÍË̤ÓË ÓÔÛËÚfiÙËÙ·, Ë ÔÔ›· Û ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ ÔÊ›ÏÂÙ·È Û ·Ó·ÙÔÌÈο ·›ÙÈ· Î·È Â›Ó·È ·Ó·fiÊ¢ÎÙË, ÂÓÒ Û ¿ÏϘ ·ÔÙÂÏ› Û˘Ó¤ÂÈ· ÙˆÓ ıÂÚ·¢ÙÈÎÒÓ ¯ÂÈÚÈÛÌÒÓ Î·È Â›Ó·È ‰˘Ó·Ùfi Ó· ÂÚÈÔÚÈÛÙ› ÛÙÔ Ì¤ÏÏÔÓ. ∞Ó·Ó¢ÛÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· ∏ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ· ·ÔÙÂÏ› ÙËÓ ÈÔ ÛËÌ·ÓÙÈ΋ ·ÈÙ›· ÓÔÛËÚfiÙËÙ·˜.

™ÙȘ ·ÒÙÂÚ˜ ÂÈÏÔΤ˜ Ù˘ ™¢∫ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ë ÂÚÈÔÚÈÛÙÈ΋ ÓfiÛÔ˜ ÙÔ˘ Ó‡ÌÔÓ·, Ô˘ ·ÓÙ·Ó·ÎÏ¿ ÙËÓ Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·, fiˆ˜ Ê·›ÓÂÙ·È ·fi ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ˘ÔÏÂÈfiÌÂÓË ¯ˆÚËÙÈÎfiÙËÙ·, 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 ·ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ‰˘ÛÏ·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 Ì ¯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˘ÚÁ›·˜ ÙˆÓ Ó¢ÌfiÓˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ¿ÌÂÛ˘ ÌÂÙ·ÁÂÓÓËÙÈ΋˜ ÂÚÈfi‰Ô˘ ·ÎÔÏÔ˘ıÔ‡ÓÙ·È ·fi ηÓÔÓÈ΋ ‹ 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 ÓÂÔÁÓ¿ ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, Èı·ÓÒ˜ ÂÂȉ‹ ÙÔ ·˘Í·ÓfiÌÂÓÔ ¤ÚÁÔ ·Ó·ÓÔ‹˜ ··ÈÙ› ˘„ËÏ‹ ηٷӿψÛË ÂÓ¤ÚÁÂÈ·˜, ÂÓÒ ÂÚÈÔÚ›˙ÂÈ ÙËÓ ÈηÓfiÙËÙ· ÙÔ˘ ·ÛıÂÓÔ‡˜ Ó· ‰È·ÙÚ·Ê› Â·ÚÎÒ˜. ∞ÓÙ›ıÂÙ·, Ë ‚ÂÏÙ›ˆÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÂÈÙÚ¤ÂÈ Î·Ï‡ÙÂÚË ·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¢ÌÔÓÈ΋ ˘ÔÏ·Û›· (84).

∞ÎÔ‹ ∂ËÚ·ÛÌfi˜ Ù˘ ·ÈÛıËÙËÚȷ΋˜ ·ÎÔ‹˜ ¤¯ÂÈ ÂÚÈÁÚ·Ê› Û ÓÂÔÁÓ¿ Ì ™¢∫ Î·È ·ÚÔ˘ÛÈ¿˙ÂÈ ¤Ó· ¢ڇ Ê¿ÛÌ· Â›ÙˆÛ˘. ¢˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘ ·ÎÔ‹˜ ·Ó·Ê¤ÚıËΠ۠ÓÂÔÁÓ¿, ›Ù ıÂÚ·‡ÙËÎ·Ó Ì ECMO ›Ù fi¯È (85). ∏ ·Ú·ÙÂٷ̤ÓË ¯Ú‹ÛË ÙÔ˘ ·ÓÎÔ˘Ú·Ó›Ô˘ (86) Î·È ‰ÈÔ˘ÚËÙÈÎÒÓ Û ÓÂÔÁÓ¿ Ì ¯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· ıÂÚ·›·˜ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ECMO, Î·È Ë ‰È¿ÚÎÂÈ· ¯ÔÚ‹ÁËÛ˘ ˆÙÔÙÔÍÈÎÒÓ Ê·Ú̿ΈÓ, fiˆ˜ Â›Ó·È Ù· ‰ÈÔ˘ÚËÙÈο Ù˘ ·Á·Ï˘ Î·È ÙÔ ·ÓÎÔ˘Ú¿ÓÈÔ, Ô˘ ÂËÚ¿˙ÂÈ ÙË ÓÂÊÚÈ΋ ÏÂÈÙÔ˘ÚÁ›· (87). √È Ù·ÎÙÈÎÔ› ¤ÏÂÁ¯ÔÈ Ù˘ ·ÎÔ‹˜ ˆ˜ ÙËÓ ËÏÈΛ· ÙˆÓ 3 ÂÙÒÓ ıˆÚÔ‡ÓÙ·È ··Ú·›ÙËÙÔÈ. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Ù˘ ÌÂȈ̤Ó˘ ·ÎÔ‹˜ ·ÔÙÚ¤ÂÈ ÙËÓ Î·ı˘ÛÙ¤ÚËÛË ¤Ó·Ú͢ Ù˘ ÔÌÈÏ›·˜ Î·È ÙËÓ ÂÌÊ¿ÓÈÛË ‰È·Ù·Ú·¯ÒÓ ÂÈÎÔÈÓˆÓ›·˜. ¶·È‰È·ÙÚÈ΋ 2009;72:103-000

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·104

104

Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

¶ÚÔ‚Ï‹Ì·Ù· ÔÚıÔ‰Èο √È ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ÛÎÂÏÂÙÔ‡, fiˆ˜ Â›Ó·È Ë ·Û˘ÌÌÂÙÚ›·, ÔÈ ·Ú·ÌÔÚÊÒÛÂȘ ÙÔ˘ ıÒڷη Î·È Ë ÛÎÔÏ›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 ÌÂÁ¿Ï˜ ™¢∫ Î·È Â›Ó·È Èı·ÓfiÓ Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ·ÚÁfiÙÂÚ· Ì ÙËÓ ·Ó¿Ù˘ÍË. Ÿˆ˜ ·Ó·Ê¤ÚÂÙ·È ·fi ÙÔÓ Lund, Ë ÛÎÔÏ›ˆÛË Î·È ÔÈ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ıÒڷη ÌÔÚ› Ó· Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ·‡ÍËÛË ÙˆÓ ÂÓ‰ÔıˆÚ·ÎÈÎÒÓ Î·È ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ȤÛÂˆÓ Î·Ù¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË. √È ÂÚÈÛÛfiÙÂÚ˜ ·fi ÙȘ ·Ú·ÌÔÚÊÒÛÂȘ ·˘Ù¤˜ Â›Ó·È ‹Ș 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 ·˘ÍË̤Ó˜ 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˘¯ÓfiÙÂÚ· Û ·ÛıÂÓ›˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (Ô˘ ·ÚÔ˘Û›·Û·Ó Û˘ÌÙÒÌ·Ù· ÂÓÙfi˜ ÙˆÓ ÚÒÙˆÓ 6 ˆÚÒÓ ·fi ÙË Á¤ÓÓËÛË) Ì ‰È¿Ù·ÛË ÔÈÛÔÊ¿ÁÔ˘ Î·È Û ·ÛıÂÓ›˜ ÛÙÔ˘˜ ÔÔ›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·ÎÚÔÚfiıÂÛÌ· ÚÔ‚Ï‹Ì·Ù· ·fi ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi, fiˆ˜ ¯ÚfiÓÈ· ÔÈÛÔÊ·Á›Ùȉ· (Û¯ÂÙÈ˙fiÌÂÓË Ì °√¶) Î·È Î·ı˘ÛÙÂÚË̤ÓË ÂÓÙÂÚÈ΋ ΤӈÛË. ∏ ˘„ËÏ‹ Â›ÙˆÛË Ù˘ ÔÈÛÔÊ·Á›Ùȉ·˜ Î·È ÙÔ˘ ÔÈÛÔÊ¿ÁÔ˘ Barrett ÛÙÔ˘˜ ÂÓ‹ÏÈÎÔ˘˜ ·ÛıÂÓ›˜ Ì ÈÛÙÔÚÈÎfi Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ηıÈÛÙ¿ ·Ó·Áη›· ÙËÓ ÂÊ·ÚÌÔÁ‹ ÚÔÁÚ¿ÌÌ·ÙÔ˜ ÂÚ·ÈÙ¤Úˆ ·Ú·ÎÔÏÔ‡ıËÛ‹˜ ÙÔ˘˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÓ‹ÏÈ΢ ˙ˆ‹˜ ÙÔ˘˜ (90). ¶ÚÔÙ›ÓÔÓÙ·È ‰È·ÙÚÔÊ‹ ÏÔ‡ÛÈ· Û ıÂÚÌ›‰Â˜ Î·È Ê·Ú̷΢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘ Ì ∏2-·ÔÎÏÂÈÛÙ¤˜ ˆ˜ ıÂÚ·¢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·˜. ∆· ÓÂÔÁÓ¿ Ì ÛÔ‚·Ú‹ Ó¢ÌÔÓÈ΋ ˘ÔÏ·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˘ÍȷΤ˜ ÂÈÏÔΤ˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û·ÛÌÔ‡˜, Ì·ıËÛȷΤ˜ ‰˘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·Paediatriki 2009;72:104-000

ÊÚ·ÁÌ·ÙÔ΋ÏË ˙Ô˘Ó ÌÈ· Ê˘ÛÈÔÏÔÁÈ΋ ˙ˆ‹. øÛÙfiÛÔ, Û¯ÂÙÈΤ˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›Â˜, fiˆ˜ 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 ·ÚÎÂÙÔ‡˜ ·fi ·˘ÙÔ‡˜ (88,61,94). ∏ ·Ô˘Û›· Û˘ÓÙÔÓÈṲ̂ÓÔ˘ Ï¿ÓÔ˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ Ô‰ËÁ› Û ˘ÔÙ›ÌËÛË Ù˘ ÂÈÎÚ¿ÙËÛ˘ Û˘ÁÎÂÎÚÈÌ¤ÓˆÓ ÂÈÏÔÎÒÓ Û ·ÛıÂÓ›˜ ÌÂÁ·Ï‡ÙÂÚ˘ ËÏÈΛ·˜ Ì ÈÛÙÔÚÈÎfi Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ (61). ™Â ÚfiÛÊ·ÙË ¤Ú¢ӷ ‚Ú¤ıËΠˆ˜ Ù· ÓÂÔÁÓ¿ Ù· ÔÔ›· ¯ÚÂÈ¿˙ÔÓÙ·Ó ıÂÚ·›· ̤۷ ÛÙȘ ÚÒÙ˜ 6 ÒÚ˜ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ıÓËÙfiÙËÙ· Û ÔÛÔÛÙfi 44%, ÂÓÒ ÌˉÂÓÈ΋ ıÓËÙfiÙËÙ· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·˘Ù¿ Ô˘ ¯ÚÂÈ¿˙ÔÓÙ·Ó ıÂÚ·›· ÌÂÙ¿ ÙȘ ÚÒÙ˜ 6 ÒÚ˜ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ (98,99). √È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ‰ÂÓ Â›Ó·È ˘Ô¯ÚˆÙÈο ‰Â˘ÙÂÚ‡ԢÛ˜ Î·È Ë Û˘Ó‡·ÚÍ‹ ÙÔ˘˜ ÌÔÚ› Ó· ¯ÂÈÚÔÙÂÚ‡ÂÈ ÙËÓ ÚfiÁÓˆÛË. ∫·ıÔÚÈÛÙÈ΋˜ ÛËÌ·Û›·˜ ÁÈ· ÙËÓ ¤Î‚·ÛË ÙˆÓ ÓÂÔÁÓÒÓ ·˘ÙÒÓ Â›Ó·È Ë Û˘Ó‡·ÚÍË ÌÂÈ˙fiÓˆÓ ·Ó·ÙÔÌÈÎÒÓ ‹ ¯ÚˆÌÔۈ̷ÙÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ, Ô˘ ·˘Í¿ÓÂÈ ÙË ıÓËÙfiÙËÙ· ÛÙÔ 89% (36). ™ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›·, Ù· ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘ Û ۯ¤ÛË Ì ÙËÓ ÂÓÙfiÈÛË Ù˘ ™¢∫ ÔÈΛÏÏÔ˘Ó. ŸÙ·Ó Ë ‚Ï¿‚Ë ÂÓÙÔ›˙ÂÙ·È ÛÙÔ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ Ë ÂÈ‚›ˆÛË ·Ó¤Ú¯ÂÙ·È Û 78%, fiÙ·Ó ÂÓÙÔ›˙ÂÙ·È ÛÙÔ ‰ÂÍÈfi Û 21%, ÂÓÒ Û ·ÌÊÔÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË ·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) ÛÙË ‰ÂÍÈ¿ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÂÓÒ ¿ÏϘ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë ÂÓÙfiÈÛË Ù˘ ·ÓˆÌ·Ï›·˜ ‰ÂÓ ÂËÚ¿˙ÂÈ ÙËÓ ¤Î‚·ÛË (55). ∆· ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘ ÁÈ· ·Ï‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Â›Ó·È ÂÚ›Ô˘ 47% Î·È ·˘Í¿ÓÔÓÙ·È ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ¯ÚfiÓÔ˘, ·Ú·Ì¤ÓÔ˘Ó fï˜ ¯·ÌËÏ¿ ÁÈ· ÙȘ ÂÚÈÙÒÛÂȘ fiÔ˘ Û˘Ó˘¿Ú¯Ô˘Ó Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ (55). ÕÏϘ ÌÂϤÙ˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÂÈ‚›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 ÂÈÙÚ¤ÂÈ ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÁÈ· Èı·Ó¤˜ ÂÈÚfiÛıÂÙ˜ ·ÓˆÌ·Ï›Â˜, ηıÒ˜ Î·È ÙËÓ ÂÓË̤ڈÛË ÙˆÓ ÁÔÓ¤ˆÓ ÁÈ· ÙȘ Èı·Ó¤˜ ıÂÚ·¢ÙÈΤ˜ ÚÔÛÂÁÁ›ÛÂȘ Î·È ÙËÓ ÚfiÁÓˆÛË. ∏ ÂÈÎÔÈÓˆÓ›· Ì ÙÔ˘˜ ÁÔÓ›˜ Ú¤ÂÈ Ó· ·Ú¯›˙ÂÈ ·fi ÙË ÛÙÈÁÌ‹ Ù˘ ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Î·È Ó· Û˘Ó¯›˙ÂÙ·È Û fiÏË ÙËÓ ·È‰È΋ ËÏÈΛ·.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Pober BR, Lin A, Russell M, Ackerman KG, Chakravorty S, Strauss B et al. Infants with Bochdalek diaphragmatic hernia: Sibling precurrence and monozygotic twin discordance in a hospital-based malformation surveillance program. Am J Med Genet Part A 2005;138A:81-88.

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·105

105

™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

2. Pober BR. Overview of epidemiology, genetics, birth defects and chromosome abnormalities associated with CDH. Am J Med Part C Semin Med Genet 2007;145C: 158-171.

3. Skari H, Bjornland K, Frenckner B, Friberg LG, Heikkinen M, Hurme T et al. Congenital diaphragmatic hernia in Scandinavia from 1995 to 1998: predictors of mortality. J Pediatr Surg 2002;37:1269-1275.

4. Dott MM, Wong LY, Rasmussen SA. Population-based study of congenital diaphragmatic hernia: Risk factors and survival in Metropolitan Atlanta, 1968-1999. Birth Defects Res A Clin Mol Teratol 2003;67:261-267.

5. Yang W, Carmichael SL, Harris JA, Shaw GM. Epidemiologic characteristics of congenital diaphragmatic hernia among 2.5 million California births, 1989-1997.

Birth defects Res 2006;76:170-174.

6. Langham MRJ, Kays DW, Ledbetter DJ, Frentzen B, Sanford LL, Richards DS. Congenital diaphragmatic hernia. Epidemiology and outcome. Clin Perinatol 1996;23:671-688.

7. Arioni C, Bellini C, Mazzella M, Zullino E, Serra G, Toma P. Congenital right diaphragmatic hernia. Pediatr Ratiol 2003;33:807-808.

8. Devine PC, Malone FD. Noncardiac thoracic anomalies.

Clin Perinatol 2000;4:865-899.

9. Harmath A, Hajdu J, Csaba A, Hauzman E, Pete B, Görbe E, et al. Associated malformation in congenital diaphragmatic hernia cases in the last 15 years in a tertiary referral institute.

A J Med Genet 2006;A:2298-2304.

10. Torfs CP, Curry CJ, Bateson TF, Honore LH. A population-based study of congenital diaphragmatic hernia. Teratology 1992;46:555-565.

11. Crane JP. Familian congenital diaphragmatic hernia: Prenatal diagnostic approach and analysis of twelve families. Clin Genet 1979:16:244-252.

12. Allan DW, Greer JJ. Embryogenesis of the phrenic nerve and diaphragm in the fetal. J Comp Neurol 1997;382:459-468.

13. Slavotinek A, Warmerdam B, Lin A, Shaw G. Populationbased analysis of left and right-sided diaphragmatic hernia demonstrates different frequencies of selected additional anomalies. A J Med Genet Part A. 2007;143A:3127-3136.

14. Wolff G. Familiar congenital diaphragmatic defect: Review and conclusions. Hum Genet 1980;54:1-5.

15. Tibboel D, Gaag AV. Etiologic and genetic factors in congenital diaphragmatic hernia. Clin Perinatol 1996;23: 689-699.

16. Norio R, Kaariainen H, Rapola J, Herva R, Kekomari M. Familial congenital diaphragmatic defects: aspects of etiology, prenatal diagnosis and treatment. Am J Med Genet 1984;17:471-483.

17. Smith DW. Recognizable Patterns of Human Malformation. Genetic, Embryologic and Clinical Aspects. 3 Philadelphia PA: W.B Saunders. 1982;7:1-653.

18. Enns GM, Cox VA, Goldstein RB, Gibbs DL, Harrison MR, Golabi M. Congenital diaphragmatic defects and associated syndromes, malformations and chromosome anomalies: a retrospective study of 60 patients and literature review. Am J Med Genet 1998;79:215-225.

19. Kantarci S, Casavant D, Prada C, Russell M, Byrne J, Haug L, et al. Findings from aCGH in patients with congenital diaphragmatic hernia: a possible locus for Fryns syndrome.

A J Med Genet 2006;140A:17-23.

20. Slavotinek A, Lee SS, Davis R, Shrit A, Leppig KA, Rhim J, et al. Fryns syndrome phenotype caused by chromosome microdeletions at 15q26.2 and 8p23.1. J Med Genet 2005;42:730-736.

21. Philip N, Gambarelli D, Guys JM, Camboulives J, Ayme S. Epidemiological study of congenital diaphragmatic defects with special reference to etiology. Eur J Pediatr 1991;150: 726-729.

22. Clerk RD, Fenner-Gonzales M. Apparent Fryns syndrome in a boy with a tandem duplication of 1q24-31.2. Am J Genet 1989;34:422-426.

23. Dean JC, Couzin DA, Gray ES, Lioyd DJ, Stephen GS. Apparent Fryns syndrome and aneuploidy: evidence for a disturbance of the midline developmental field. Clin Genet 1991;40:349-352.

24. Nordenskjold A, Tapper-Persson M, Anvret M. No evidence of WT1 gene mutations in children with congenital diaphragmatic hernia. J Pediatr Surg 1996; 31:925-927.

25. Biggio JR, Descartes MD, Carroll AJ, Holt RL. Congenital diaphragmatic hernia: Is 15q26.1-26.2 a candidate locus?

Am J Med Genet Part A 2004;126A:183-185.

26. Greer JJ, Babiuk P, Thebaud B. Etiology of congenital diaphragmatic hernia: the retinoid hypothesis. Pediatr Res 2003;53:726-730.

27. Cushman L, Martinez W, Cherry A, Manning M, AbdulRahman O, Anderson C, et al. A J Med Genet 2005;137A: 65-71.

28. Fauza DO, Wilson JM. Congenital diaphragmatic hernia and associated anomalies: their incidence, identification and impact on prognosis. J Pediatr Surg 1994;29:11131117.

29. Lurie IW. Where to look for the genes related to diaphragmatic hernia? Genet Couns 2003;14:75-93.

30. Mendelsohn C, Lohnes D, Decimo D, Lufkin T, LeMeur M, Chambon P, et al. Function of the retinoic acid receptors (RARs) during development: (ππ) multiple abnormalities at various stages of organogenesis in RAR double mutants. Development 1994;120:2749-2771.

31. Chen MH, McGowan A, Ward S, Bsvik C, Greer JJ. The activation of the retinoic acid response element is inhibited in an animal model of congenital diaphragmatic hernia. Biol Neonate 2003;83:157-161.

32. Major D, Cadenas M, Fournier L, Leclerc S, Lefebre M, Cloutier R. Retinal status of newborn infants with congenital diaphragmatic hernia. Pediatr Surg Int 1998;13:547-549.

33. Jesudason EC, Smith NP, Connell, Spiller D, White M, Fernig D, et al. Peristalsis of airway smooth muscle is developmentally regulated and uncoupled from hypoplastic lung growth. Am J Physiol Lung Cell Mol Physiol 2006;291:559-565.

34. Boucherat O, Benachi A, Barlier-Mur A, Franco-Montoya M, Martinovic J, Thebaud B, et al. Decreased lung fibroblast growth factor 18 and elastin in human congenital diaphragmatic hernia and animal models. Am J Respir Crit Care Med 2004;175:1066-1077.

35. Wilcox DT, Irish MS, Holm BA, Glick PL. Animal models in congenital diaphragmatic hernia. Clin Perinatol 1996;23:813-822.

36. Keijzer R, Liu J, Deimling J, Tibboel D, Post M. Dual-hit hypothesis explains pulmonary hypoplasia in the nitrofen ¶·È‰È·ÙÚÈ΋ 2009;72:105-000

Pediatri Mar-Apr 09

10-04-09

15:36

™ÂÏ›‰·106

106

Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

model of congenital diaphragmatic hernia. Am J Pathol 2000;156:1299-1306.

37. Glick PL, Leach CL, Besner GE, Equan EA, Morin FC,Robinson LK, et al. Pathophysiology of congenital diaphragmatic hernia III: exogenous surfactant therapy for the high-risk neonate with CDH. J Pediatr Surg 1992;27:866.

38. Bohn DJ, Lames I, Filler RM, Ein SH, Wesson DE, Shandling B, et al. The relationship between PaCO2 and ventilation parameters in predicting survival in congenital diaphragmatic hernia. J Pediatr Surg 1984;19:666-671.

39. Weber TR, Tracy T, Bailey PV, Lewis JE. Congenital diaphragmatic hernia beyond infancy. Am J Surg 1991; 162:643-646.

40. Adzick NS, Harrison MR, Glick PL, Villa RL, Finkbeiner W. Experimental pulmonary hypoplasia and oligohydramnios: relative contributions of lung fluid and fetal breathing movements. J Pediatr Surg 1984;19:658-665.

41. Shochat SJ. Pulmonary vascular pathology in congenital diaphragmatic hernia. Pediatr Surg Int 1987;2:331-334.

42. Crawford DC, Wright VM, Drake DP, Allan LD. Fetal diaphragmatic hernia: the value of fetal echocardiography in the prediction of postnatal outcome. Br J Obstet Gynaecol 1989;96:705-710.

43. Schwartz SM, Vermilion RP, Hirschl RB. Evaluation of left ventricular mass in children with left-sided congenital diaphragmatic hernia. J Pediatr 1994;125:447-451.

44. Nakamura Y, Yamamoto I, Fukuda S, Hashimoto T. Pulmonary acinar development in diaphragmatic hernia.

Arch Pathol Lab Med 1991;115:372-376.

45. Ijsslstijn H, Zimmermann LJ, Bunt JE, de Jongste JC, Tibboel D. Prospective evaluation of surfactant composition in bronchoalveolar lavage fluid of infants with congenital diaphragmatic hernia and of age-matched controls. Crit Care Med 1998;26:573-580.

46. Berk C, Grundy M. High risk lecithin/sphingomyelin ratios associated with neonatal diaphragmatic hernia: case report. Br J Obstet Gynaecol 1982;89:250-251.

47. Moya FR, Thomas VL, Romaguera J, Mysore MR, Maberry M, Bernard A, et al. Fetal lung maturation in congenital diaphragmatic hernia. Am J Obstet Gynecol 1995;173: 1401-1405.

48. Sullivan KM, Hawgood S, Flake AW, Harrison MR, Adzick NS. Amniotic fluid phospholipid analysis in the fetus with congenital diaphragmatic hernia. J Pediatr Surg 1994;29: 1020-1023.

49. Cogo P, Zimmermann L, Rosso F, Tormera F, Gamba P, Verlato G, et al. Surfactant synthesis and kinetics in infants with congenital diaphragmatic hernia. Am Respir Crit Care Med 2002;40:154-158.

50. Godinez MH, Godinez RI. Inspired oxygen concentration alters the phospholipid and protein content in the bronchoalveolar lavage-accessible space. Crit Care Med 1996;24:862-869.

51. Hagen EO. Drugs and congenital abnormalities. Lancet.

1963; I:501.

52. Sievers G, Schradle-Beielstein H. Drugs and congenital abnormalities. Lancet. 1963; I:330.

53. Higuchi R, Minami T, Shimoyamada Y, Kamisako H, Koike M, Takigawa H, et al. Diaphragmatic hernia in an infant of a diabetic mother: an unusual association in diabetic embryopathy. Pediatr Int 1999;41:581-583.

Paediatriki 2009;72:106-000

54. Solari V, Puri P. Glucocorticoid receptor gene expression in the hypoplastic lung of newborns with congenital diaphragmatic hernia. J Pediatr Surg 2002;37:715-718.

55. Gallot D, Boda C, Ughetto S, Perthus I, Robert-Gnansia E, Francannet C, et al. Prenatal detection and outcome of congenital diaphragmatic hernia: a French registry-based study. Ultrasound Obstet Gynecol 2007;29:276-283.

56. Game E, Haeusler M, Barisic I, Gjergia R, Stoll C, Clementi M. Congenital diaphragmatic hernia; evaluation of prenatal diagnosis in 20 European regions. Ultrasound Obstet Gynecol 2002;19:329-333.

57. Grandjean H, Larroque D, Levi S. The performance of routine ultrasonographic screening of pregnancies in the Eurofetus Study. Am J Ostet Gynecol 1999;181:446-454.

58. Newman BM, Afshari E, Karp,MP, Jewett TC Jr, Cooney DR. Presentation of congenital diaphragmatic hernia post the neonatal period. Arch Surg 1986;121:813-816.

59. Snyder HS, Salo DF, Kelly PH. Congenital diaphragmatic hernia presenting as massive gastrothorax. Ann Emerg Med 1990;19:562-56.

60. Mei-Zahav M, Solomon M, Trachsel D, Langer JC. Bochdalek diaphragmatic hernia: not only a neonatal disease. Arch Dis Child 2003;88:532-535.

61. Muratore C, Utter S, Jaksic T, Lund D, Wilson J. Nutritional morbidity in survivors of congenital diaphragmatic hernia. J Pediatr Surg 2001;36:1171-1176.

62. Baglaj M, Dorobisz U. Late presenting congenital diaphragmatic hernia in children: a literature review.

Pediatr Radiol 2005;35:478-488.

63. Katz AL, Wiswell TE, Baumgact S. Contemporary controversies in the management of congenital diaphragmatic hernia. Clin Perinatol 1998;25:219-248.

64. Finer NN, Tierney A, Etchers PC, Peliowski A, Ainsworth W. Congenital diaphragmatic hernia: developing a protocolixed approach. J Pediatr Surg 1998;33:1331-1337.

65. Kinsalla LP, Abma SH. Recent developments in the pathophysiology and treatment of persistent pulmonary hypertension of the newborn. J Pediatr 1995;126:853-64.

66. Paranka MS, Walsh WF, Stancombe BB. Surfactant lavage in a piglet model of meconium aspiration syndrome.

Pediatr Res 1992;31:625.

67. Baumgart S, Hirschl RB, Butler SZ, Coburn CE, Spitzer AR. Diagnosis-related criteria in the consideration of extracorporeal membrane oxygenation in neonates previously treated with high frequency jet ventilation.

Pediatr 1992;89:491-494.

68. O’Tool SJ, Karamnukian HL, Morin FC, Holm BA, Egan EA, Azizkhan RG, et al. Surfactant decreases pulmonary vascular resistant and increases pulmonary blood flow in the fetal lamb model of congenital diaphragmatic hernia.

J Pediatr Surg 1996;31:507-511.

69. Lotze A, Mitchell BR, Bulas DI, Zola EM, Shalwitz RA, Gunkel JH. Multicenter study of surfactant (beractant) use in the treatment of term infants with severe respiratory failure. Survanta in Term Infants Study Group. J Pediatr 1998;132:40-47.

70. Merces S. Factors involved in chylothorax following repair of congenital diaphragmatic hernia. J Pediatr Surg 1986;21:809-811.

71. Naik S, Greenough A, Zhang Y, Davenport M. Prediction of morbidity during infancy after repair of congenital diaphragmatic hernia. J Pediatr 1996;31:1651-1654.

Pediatri Mar-Apr 09

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™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

72. Hatch EL Jr, Kendall J, Blumhagen J. Stomach position as an in utero predictor of neonatal outcome in left-sided diaphragmatic hernia. J Pediatr Surg 1992;27:778-779.

73. Doyle NM, Lally KP. The CDH Study Group and advances in the clinical care of the patient with congenital diaphragmatic hernia. Semin Perinatol 2004;28:174-184.

74. Bath ME, Jesudason EC, Losty PD. How useful is the lungto-head ratio in predicting outcome in the fetus with congenital diaphragmatic hernia? ∞ systematic review and meta-analysis. Ultrasound Obstet Gynecol 2007;30:897-906.

75. Laudy JA, Van Gucht M, Van Dooren MF, Wladimiroff JW, Tibboel D. Congenital diaphragmatic hernia; an evaluation of the prognostic value of the lung-to-head ratio and other prenatal parameters. Prenat Diagn 2003;23:634-639.

76. Lipshutz GS, Albanese CT, Feldstein VA, Jennings RW, Housley HT, Beech R, et al. Prospective analysis of lungto-head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia. J Pediatr Surg 1997;32:1634-1636.

77. Thebaud B, Azancot A, de Lagaisie P, Vuillard E, Ferkadji L, Benali K, et al. Congenital diaphragmatic hernia: antenatal prognostic factor. Does cardiac ventricular disproportion in utero predict outcome and pulmonary hypoplasia? Intensive Care Med 1997;23:10062-10069.

78. Chaoui R, Kalache K, Tennstedt C, Lenz F, Vogel M. Pulmonary arterial Doppler velocimetry in fetuses with lung hypoplasia. Eur J Obstet Gynocol Reprod Biol 1999; 84:179-185.

79. Levine D, Barnewolt CE, Mehta TS, Trop I, Estroff J, Wong G. Fetal thoracic abnormalities: MR imaging.

Radiology 2003;228:379-388.

80. Donnelly LF, Sakurai M, Klosterman LA, Delong DM, Strife JL. Correlation between findings on chest radiography and survival in neonates with congenital diaphragmatic hernia. AJR Am J Proentgenol 1999;173:1589-1593.

81. Bos AP, Hussain SM, Hazebroek FWJ, Tibboel D, Meradji M, Molenaar. Radiographic evidence of bronchopulmonary dyplasia in high-risk congenital diaphragmatic hernia survivors. Pediatr Pulmonol 1993;15:231-234.

82. Koumbourlis A, Wung J, Stolar C. Lung function in infants after repair of congenital diaphragmatic hernia. J Pediatr Surg 2006;41:1716-1721.

83. Marven SS, Smith CM, Claxton D,Chapman J, Davies HA, Primhak PA, et al. Pulmonary function, exercise performance, and growth in survivors of congenital diaphragmatic hernia. Arch Dis Child 1998;78:137-142.

84. Wischermann A, Holschneider AM, Hubner U. Longterm follow-up of children with diaphragmatic hernia.

Eur J Pediatr Surg 1995;5:13-18.

85. Rasheed A, Tindall S, Cueny DL, Klein MD, DelaneyBlack V. Neurodevelopmental outcome after congenital diaphragmatic hernia: extracorporeal membrane oxygenation before and after surgery. J Pediatr Surg 2001;36: 539-544.

86. Cheuhg PY, Tyebkhan LM, Peliowski A, Ainsworth W, Robertson CMT. Prolonged use of pancuronium bromide and sensorineural hearing loss in childhood survivors of congenital diaphragmatic hernia. J Pediatr 1999;135: 233-239.

87. ªasumoto K, Nagata K, Uesugi T, Yamada T, Taguchi T. Risk factors for sensorineural hearing loss in survivors with severe congenital diaphragmatic hernia. Eur J Pediatr 2007;166:607-612.

88. Lund DP, Mitchell J, Kharasch V, Quigley S, Kuehn M, Wilson JM. Congenital diaphragmatic hernia: the hidden morbidity. J Pediatr Surg 1994;29:258-264.

89. Jaillard S, Pierrat V, Dubois A, Truffert P, Lequien P, Wurtz A, et al. Outcome at 2 years of infants with congenital diaphragmatic hernia: a population-based study. Ann Thorac Surg 2003;75:250-256.

90. Vanamo K, Peltonen J, Rintala R, Lindahl H, Jaaskelainen J, Louhimoo I. Chest wall and spinal deformities in adults with congenital diaphragmatic defects. J Pediatr Surg 1996; 31:851-854.

91. Van Meurs KP, Robbins ST, Reed VL, Karr SS, Wagner AE, Glass P, et al. Congenital diaphragmatic hernia; longterm outcome in neonates treated with extracorporeal membrane oxygenation. J Pediatr 1993;122:893-899.

92. Cohen MD, Beck LM. Hiatus hernia: a complication of postero-lateral diaphragmatic hernia (Bochdalek hernia) in infants. Clin Ratiol 1980;31:215-219.

93. Koot VCM, Bergmeijer JH, Bos AP, Molennar JC. Incidence and management of gastroesophageal reflux after repair of congenital diaphragmatic hernia. J Pediatr Surg 1993;28:48-52.

94. Stolar CJ. What do survivors of congenital diaphragmatic hernia look kike when they grow up? Semin Pediatr Surg 1996;5:275-279.

95. Glass P, Wagner AE, Papero PH, Rajasingham SR, Civitello LA, Kjaer MS, et al. Neurodevelopmental status at age five years of neonates treated with extracorporeal membrane oxygenation. J Pediatr 1995;127:447-457.

96. Graziani LJ, Gringlas M, Baumgart S. Celebrovascular complications and neurodavelopment sequelae of neonatal ECMO. Clin Perinatol 1997;24:655-674.

97. McGahren ED, Mallik K, Rodgers BM. Neurological outcome is diminished in survivors of congenital diaphragmatic hernia requiring exrtacorporeal membrane oxygenation. J Pediatr Surg 1997;32:1216-1220.

98. Collins DL, Pomerance JJ, Travis KW, Turner SW, Pappelbaum SJ. A new approach to congenital posterolateral diaphragmatic hernia. J Pediatr Surg 1977; 12:149156.

99. Skari H, Bjornland K, Haugen G, Egeland T, Emblem R. Congenital diaphragmatic hernia: A meta-analysis of mortality factors. J Pediatr Surg 2000;35:1187-1197.

100. Wung JT, Sahni R, Moffitt ST, Lipsitz E, Stolar CJ. Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration and no chest tube. J Pediatr Surg 1995;30:406-409.

101. Clark WR, Nieman GF, Thomposon JE, Paskanik AM, Rokhar JE, Bredenberg CE. Surfactant displacement by mecomium free fatty acids: an alternative explanation for atelectasis in meconiun aspiration syndrome. J Pediatr 1987;110:765-770.

102. Geary M. Management of congenital diaphragmatic hernia diagnosed prenatally: an update. Prenat Diagn 1998;18:1155-1158.

103. Macken MB, Thompson DL. Antenatal diagnosis of congenital diaphragmatic hernia. Can Assoc Radiol J 1993;44:439-442.

104. Sreenan C, Etches P, Osiovich H. The western Canadial experience with congenital diaphragmatic hernia: perinatal factors predictive of extracorporeal membrane oxygenation and death. Pediatr Surg Int 2001;17:196-200.

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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‹¯ˆÓ Á. °›ÓÂÙ·È ÌfiÓÔ ÛÙÔ 10% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ‰. √È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ‰˘ÛÎÔÏÂ‡Ô˘Ó ÙË ‰È¿ÁÓˆÛË 2. ∏ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ· ÓÂÔÁÓÔ‡ ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ÔÊ›ÏÂÙ·È ÛÂ: ·. ¶Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· ‚. §Ô›ÌˆÍË Á. ¶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. ∞ÌÊÔÙÂÚfiÏ¢ڷ ‰. ∫ÂÓÙÚÈο 4. ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÂΉËÏÒÓÂÙ·È Û˘¯ÓfiÙÂÚ· ÌÂ Û˘ÌÙÒÌ·Ù· ·fi: ·. ∆Ô Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ· ‚. ∆Ô ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· Á. ∂›Ó·È ·Û˘Ìو̷ÙÈ΋ ‰. ∆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

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∂›‰Ú·ÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì˘ÂÏÔ‡ ÔÛÙÒÓ ÛÙËÓ ex vivo ¤ÎÙ˘ÍË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÔÌÊ·ÏÔÏ·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‡ ÌÈÎÚÔÂÚÈ‚¿ÏÏÔÓÙÔ˜, ı· ÌÔÚÔ‡Û·Ó Ó· ÚÔ¿ÁÔ˘Ó ÙË ‰È·‰Èηۛ· ¤ÎÙ˘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 ¤ÎÙ˘Í‹ ÙÔ˘˜ ÁÈ· ¯Ú‹ÛË Û ·È‰È¿ ÌÂÁ·Ï‡ÙÂÚÔ˘ ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜ Î·È Û ÂÓ‹ÏÈΘ. ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ·ÏÏËÏÂ›‰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·ÛË Û˘Ó‰˘·ÛÌÔ‡ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ÃÚËÛÈÌÔÔÈ‹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¤ıËÎ·Ó Û ‰È·ÊÔÚÂÙÈΤ˜ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜, ·ÚÔ˘Û›· ‹ ÌË Û˘Ó‰˘·ÛÌÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, Î·È ¤ÁÈÓ ·ÓÔÛÔÊ·ÈÓÔÙ˘ÈÎfi˜ ¯·Ú·ÎÙËÚÈÛÌfi˜, ·ÍÈÔÏfiÁËÛË Ù˘ ¤ÎÙ˘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¿ÁÔÓÙ˜, ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË ÌÂÁ·Ï‡ÙÂÚË ¤ÎÙ˘ÍË ÙfiÛÔ ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÙÙ¿ÚˆÓ fiÛÔ Î·È ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ CD34+. ∏ ·ÚÔ˘Û›· ª∫ ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ¤‰ˆÛ ÙÔ ˘„ËÏfiÙÂÚÔ ÔÛÔÛÙfi ÙˆÓ ˘ÔÏËı˘ÛÌÒÓ CD34+CD38-, CD34+CD33-, ηıÒ˜ Î·È CD34 Ô˘ ıˆÚÔ‡ÓÙ·È Î·È ÔÈ ϤÔÓ ·Ú¯¤ÁÔÓÔÈ. ∂ÈϤÔÓ, Û ·˘Ù‹ ÙË Û˘Óı‹ÎË Ù· ÂÎÙ˘Á̤ӷ ·ÙÙ·Ú· ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ˘„ËÏ‹ ÎψÓÔÁÔÓÈÎfiÙËÙ·, ·ÊÔ‡ ¤‰ˆÛ·Ó ÙÔÓ ÌÂÁ·Ï‡ÙÂÚÔ ·ÚÈıÌfi ·ÔÈÎÈÒÓ ÙfiÛÔ ÎÔÎÎÈÒ‰Ô˘˜ fiÛÔ Î·È ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜. ™˘ÌÂÚ¿ÛÌ·Ù·: ™˘ÌÂÚ·ÛÌ·ÙÈο, Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· ª√ ˘ÔÛÙËÚ›˙Ô˘Ó ÙËÓ ¤ÎÙ˘ÍË Î·È Û˘Ì‚¿ÏÏÔ˘Ó ÛÙË ‰È·Ù‹ÚËÛË Ù˘ ·ˆÚfiÙËÙ·˜ ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ ÛÂ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜, ·Ú¤¯ÔÓÙ·˜ 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.

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜. ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ, ∆Ì‹Ì· π·ÙÚÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘, ∏Ú¿ÎÏÂÈÔ, ∫Ú‹ÙË AÏÏËÏÔÁÚ·Ê›·: ª·Ú›· ∫·ÏÌ·ÓÙ‹ pedhem@med.uoc.gr ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜, ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ, 71110 ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘

§¤ÍÂȘ ÎÏÂȉȿ: CD34+, ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ex vivo ¤ÎÙ˘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 overcome this 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 the regulation 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 growth factor (GF) combinations on the expansion of HSC. Methods: CD34+ HSC from the UCB of full term deliveries (n=10) and MSC isolated and expanded from the BM of children were co-cultured under different conditions in the presence or absence of GF combinations. The expansion capability was evaluated, the various populations were immunophenotypically characterized and HSC clonogenicity (expressed as the development-formation of CFUGM/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 a higher expansion of both total cell number and CD34+ cells. Those cultured on the MSC feeder layer only yielded a higher percentage of CD34+, CD34+CD38- and CD34+CD33- subpopulations, and enhanced CFUGM and BFU-E colony development.

Department of Paediatric Haematology-Oncology, University Hospital of Heraklion, University of Crete Medical School, Heraklion, Crete, Greece Correspondence: Maria Kalmanti pedhem@med.uoc.gr Department of Paediatric Haematology-Oncology, University Hospital of Heraklion, 71110, Heraklion, Crete, Greece

* ∏ ÂÚÁ·Û›· ·˘Ù‹ ‚Ú·‚‡ıËΠ̠ÙÔ A’ XˆÚ¤ÌÂÈÔ Œ·ıÏÔ, ηٿ ÙÔ 46Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ∫¤Ú΢ڷ 2008 ¶·È‰È·ÙÚÈ΋ 2009;72:109-000

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π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.

Conclusions: MSC derived from BM of children, used as a feeder layer, supports the ex-vivo expansion and preserves the characteristics of ‘stemness’ of CD34+ HSC in co-culture conditions, providing a population large enough to overcome the problems caused by limited HSC numbers for CB transplants.

Key words: CD34+, mesenchymal stromal cells, ex vivo expansion, cord blood, children bone marrow.

∂ÈÛ·ÁˆÁ‹ ∆Ô ÔÌÊ·ÏÈÔÏ·ÎÔ˘ÓÙÈ·Îfi ·›Ì· (√§) ·ÔÙÂÏ› ‡ÎÔÏ· ÚÔÛ‚¿ÛÈÌË ËÁ‹ ÁÈ· ÌÂÙ·ÌfiÛ¯Â˘ÛË Î·È ‰È·ı¤ÙÂÈ ÛËÌ·ÓÙÈο ÏÂÔÓÂÎÙ‹Ì·Ù·, ‰ËÏ. ÌÂÁ·Ï‡ÙÂÚË ÂÚÈÂÎÙÈÎfiÙËÙ· Û ÚÔÁÔÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú·, ÌÈÎÚfiÙÂÚÔ ÔÛÔÛÙfi ÂÌÊ¿ÓÈÛ˘ GVHD Î·È ‰˘Ó·ÙfiÙËÙ· ÌÂÙ·ÌfiÛ¯Â˘Û˘ Û ÌË Û˘Ì‚·Ùfi ‰¤ÎÙË (1-2 HLA ·ÓÙÈÁfiÓ·) (1-3). ∆·˘Ùfi¯ÚÔÓ·, fï˜, Ô Û¯ÂÙÈο ÌÈÎÚfi˜ ·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 ÛÎÔfi ÙËÓ Â˘Ú‡ÙÂÚË ¯Ú‹ÛË ·›Ì·ÙÔ˜ √§ ÛÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË, Á›ÓÔÓÙ·È ÚÔÛ¿ıÂȘ ÁÈ· ex vivo ¤ÎÙ˘ÍË Î˘ÙÙ¿ÚˆÓ. √È ‰È·‰Èηۛ˜ ¤ÎÙ˘Í˘ ̤¯ÚÈ ÚfiÛÊ·Ù· ÂÚÈÏ¿Ì‚·Ó·Ó ÙË ¯Ú‹ÛË ‰È·ÊÔÚÂÙÈÎÒÓ Û˘Ó‰˘·ÛÌÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ÔÈ Ôԛ˜ fï˜ ηٷϋÁÔ˘Ó Û ۯÂÙÈο ‰È·ÊÔÚÔÔÈË̤ÓÔ˘˜ ΢ÙÙ·ÚÈÎÔ‡˜ ˘ÔÏËı˘ÛÌÔ‡˜ (6-8). ¶ÚfiÛÊ·Ù· ÍÂΛÓËÛÂ Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ‰˘Ó·ÙfiÙËÙ·˜ ¯Ú‹Û˘ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿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 ¤ÎÙ˘Í˘ (9-13). ∆· ª∫ ·ÔÙÂÏÔ‡Ó Ì¤ÚÔ˜ ÙÔ˘ ÌÈÎÚÔÂÚÈ‚¿ÏÏÔÓÙÔ˜ ÙÔ˘ ª√, ÙÔ ÔÔ›Ô Û˘ÌÌÂÙ¤¯ÂÈ ÛÙË Ú‡ıÌÈÛË Ù˘ ·ÈÌÔÔ›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 ÚÔÁÔÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· Û ·ÛıÂÓ›˜ Ì ηÚΛÓÔ ÙÔ˘ Ì·ÛÙÔ‡ Î·È ·ÈÌ·ÙÔÏÔÁÈΤ˜ ηÎÔ‹ıÂȘ ¤‰ÂÈÍ ٷ¯‡ÙÂÚË ·ÔηٿÛÙ·ÛË ÙˆÓ ÂÈ¤‰ˆÓ ÙˆÓ Ô˘‰ÂÙÂÚfiÊÈÏˆÓ Î·È ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ, ÂÓÒ ·Ú¿ÏÏËÏ· Ë Ì¤ıÔ‰Ô˜ Ê¿ÓËΠӷ Â›Ó·È ·ÛÊ·Ï‹˜, ¯ˆÚ›˜ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ (16,17). ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ Â›‰Ú·Û˘ ÙˆÓ ª∫ ÛÙÚÒÌ·ÙÔ˜ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ÛÙË ‰˘Ó·ÙfiÙËÙ· ¤ÎÙ˘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.

Paediatriki 2009;72:110-000

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‹ÚˆÛË ÙÔ˘ ÙÔÎÂÙÔ‡ (ŒÁÎÚÈÛË ∂ÈÙÚÔ‹˜ ¢ÂÔÓÙÔÏÔÁ›·˜ ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ Ì·˜). ∆· ÌÔÓÔ‡ÚËÓ· ·ÙÙ·Ú· (ª¡¶) ‰È·¯ˆÚ›ÛÙËÎ·Ó Ì Â›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˜.

AÔÌfiÓˆÛË 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‚ÔÂÓÈ΋ ÔÚʇڷ, n=9). ∆· MN¶ ηÏÏÈÂÚÁ‹ıËÎ·Ó Û a-MEM, ÂÌÏÔ˘ÙÈṲ̂ÓÔ Ì ÂÈÏÂÁ̤ÓÔ ÔÚfi ÂÌ‚Ú‡Ô˘ ‚Ôfi˜ (FCS), ÂÓÈÎÈÏÏ›ÓË/ÛÙÚÂÙÔÌ˘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˘„ÈÓÔÔ›ËÛË (ıÚ˘„›ÓË 0,25% + EDTA) Î·È Ù· ·ÙÙ·Ú· Â·Ó·Î·ÏÏÈÂÚÁ‹ıËÎ·Ó Û ηٿÏÏËÏË Û˘ÁΤÓÙÚˆÛË (P1). ∏ ‰È·‰Èηۛ· Â·Ó·Ï‹ÊıËΠ̤¯ÚÈ ƒ4. ∞Ó¿Ù˘ÍË CFU-F ·ÔÈÎÈÒÓ - ¯ÚfiÓÔ˜ ΢ÙÙ·ÚÈÎÔ‡ ‰ÈÏ·ÛÈ·ÛÌÔ‡: °È· ÙËÓ ·Ó¿Ù˘ÍË CFU-F ·ÔÈÎÈÒÓ 10 ª∫/cm2 ÙÔÔıÂÙ‹ıËÎ·Ó Û ÙÚ˘‚Ï›· Petri 20 cm2 Û ·-ª∂ª (ÂȘ ‰ÈÏÔ‡Ó). ªÂÙ¿ ·fi 14 Ë̤Ú˜ ηÏÏȤÚÁÂÈ·˜, ¤ÁÈÓ ¯ÚÒÛË Ì Giemsa Î·È Î·Ù·ÌÂÙÚ‹ıËÎ·Ó ÔÈ ·ÔÈ˘ Û ·Ó¿ÛÙÚÔÊÔ ÌÈÎÚÔÛÎfiÈÔ. √ ¯ÚfiÓÔ˜ ΢ÙÙ·ÚÈÎÔ‡ ‰ÈÏ·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˘„ÈÓÔÔ›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˘„ÈÓÔÔ›ËÛË). ∫·ÏÏȤÚÁÂȘ ‰È·ÊÔÚÔÔ›ËÛ˘ ª∫: ™ÙËÓ ƒ2 ‹ ƒ3 ¤ÁÈÓ Â·ÁˆÁ‹ ÙˆÓ ª∫ ÚÔ˜ ÏÈÔ·ÙÙ·Ú· (§), ÔÛÙÂÔ·ÙÙ·Ú· (√) Î·È ¯ÔÓ‰ÚÔ·ÙÙ·Ú· (Ã) Û ÂȉÈÎfi ıÚÂÙÈÎfi ˘ÏÈÎfi. ªÂ ÈÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË ÂϤÁ¯ıËÎÂ Ë ‰È·ÊÔÚÔÔ›ËÛË ÚÔ˜ § (Oil red O), √: (Von Kossa) Î·È Ã (Alcian Blue). ªÂ RT-PCR ·ÓȯÓ‡ıËÎÂ Ë ¤ÎÊÚ·ÛË ÁÔÓȉ›ˆÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ ÁÈ· § (LPL, aP2), ÁÈ· √ (Osteocalcin, Osteoprotegerin) Î·È ÁÈ· à (Aggrecan, Collagen II). ∫·ÏÏȤÚÁÂÈ· CD34+ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·ÚÔ˘Û›· ª∫ °È· ÙË Û˘ÁηÏÏȤÚÁÂÈ· ÙˆÓ ª∫ Ì ٷ ·Ú¯¤ÁÔÓ·

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∂ÈÎfiÓ· 1. ·) ªÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ‚) ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ÌÂÙ¿ ·fi ‰¤Î· Ë̤Ú˜ ηÏÏȤÚÁÂÈ·˜ ·ÚÔ˘Û›· ÌfiÓÔ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, Á) ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· Û ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· ÛÙÚÒÌ·ÙÔ˜ ÙË ‰¤Î·ÙË Ë̤ڷ Û˘ÁηÏÏȤÚÁÂÈ·˜.

CD34+ ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ª∫ ƒ3 ‹ ƒ4 Ô˘ ÙÔÔıÂÙ‹ıËÎ·Ó Û ÙÚ˘‚Ï›· 24 ‚ÔıÚ›ˆÓ ÂÈÛÙڈ̤ӷ Ì ˙ÂÏ·Ù›ÓË, ÛÂ Û˘ÁΤÓÙÚˆÛË 3x104/‚ÔıÚ›Ô. ŸÙ·Ó Ù· ª∫ ‰ËÌÈÔ‡ÚÁËÛ·Ó ÛÙÚÒÌ·, ÚÔÛÙ¤ıËÎ·Ó CD34+ ·ÙÙ·Ú· (2,5x103/‚ÔıÚ›Ô ) Î·È Âˆ¿ÛÙËÎ·Ó ÁÈ· 10 Ë̤Ú˜ Û 37ÔC 5% CO2, Ì ÚÔÛı‹ÎË ıÚÂÙÈÎÔ‡ ˘ÏÈÎÔ‡ Î·È ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÙËÓ 5Ë Ë̤ڷ. ∆Ô Î·ÏÏÈÂÚÁËÙÈÎfi ˘ÏÈÎfi ·ÔÙÂÏ›ÙÔ ·fi a-MEM + 10% FCS + 10% ÂÓÈÎÈÏÏ›ÓË /ÛÙÚÂÙÔÌ˘Î›ÓË + 10% ÁÏÔ˘Ù·Ì›ÓË, ·ÚÔ˘Û›· ‹ ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ÃÚËÛÈÌÔÔÈ‹ıËÎ·Ó ‰‡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/ml Î·È GM-CSF: 1 ng/ml Î·È GFmix2: SCF: 20 ng/ml, TPO: 25 ng/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· ·ÙÙ·Ú· ÙÔ˘ ˘ÂÚÎÂÈ̤ÓÔ˘, ÂÓÒ Ù· ÚÔÛÎÔÏÏË̤ӷ ·ÙÙ·Ú· ·ÔÌ·ÎÚ‡ÓıËÎ·Ó Ì ıÚ˘„ÈÓÔÔ›ËÛË. ∏ ¤ÎÙ˘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 ·ÈÌÔÔÈËÙÈÎÒÓ 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· ·ÈÌÔÔÈËÙÈο ·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˘ (STEM CELL 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˘ıÚÔÔÈËÙ›ÓË (EPO): 4 πU/ml. √È Î˘ÙÙ·ÚÔηÏÏȤÚÁÂȘ Âˆ¿ÛÙËÎ·Ó ÁÈ· 14 Ë̤Ú˜ Û 37ÔC 5% CO2 Î·È Ë Î·Ù·Ì¤ÙÚËÛË ·ÔÈÎÈÒÓ ¤ÁÈÓ Û ·Ó¿ÛÙÚÔÊÔ ÌÈÎÚÛÎfiÈÔ. ŸÏ· Ù· ÂÈÚ¿Ì·Ù· ¤ÁÈÓ·Ó ÂȘ ‰ÈÏÔ‡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·ÓÔÓÈ΋ ηٷÓÔÌ‹ Û˘ÁÎÚ›ıËÎ·Ó Ì Mann-Whitney U test. ŸÏ˜ ÔÈ Û˘ÁÎÚ›ÛÂȘ Â›Ó·È ‰ÈÏ‹˜ ηÙ‡ı˘ÓÛ˘. ™Ù·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ıˆڋıËÎ·Ó Ù· ·ÔÙÂϤÛÌ·Ù· Ì p<0,05. ∏ ·Ó¿Ï˘ÛË ¤ÁÈÓ 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· Î·È ‰È·Ù‹ÚËÛ·Ó ÛÙȘ ÂfiÌÂÓ˜ Â·Ó·Î·ÏÏȤÚÁÂȘ ÙÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ·ÙÚ·ÎÙÔÂȉ¤˜ Û¯‹Ì· (∂ÈÎfiÓ· 1). ∞ÓÔÛÔÊ·ÈÓÔÙ˘Èο, ‹‰Ë ·fi ÙËÓ ÚÒÙË Â·Ó·Î·ÏÏȤÚÁÂÈ· ‰ÂÓ ·Ú·ÙËÚ‹ıËΠ¤ÎÊÚ·ÛË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ ‰ÂÈÎÙÒÓ CD34 Î·È CD45, ÂÓÒ ÔÈ Û¯ÂÙÈ˙fiÌÂÓÔÈ Ì ٷ MK ‰Â›ÎÙ˜, Ô˘ ÂÎÊÚ¿˙ÔÓÙ·Ó Û Ôχ ÌÈÎÚfi ÔÛÔÛÙfi ÛÙÔÓ ÏËı˘ÛÌfi ÙˆÓ M¶¡ (CD105: 1,85±0,3%, CD146: 0,93±0,3%), ÂÎÊÚ¿˙ÔÓÙ·Ó Û ˘„ËÏfi ÔÛÔÛÙfi ÛÙȘ Â·Ó·Î·ÏÏȤÚÁÂȘ ÙˆÓ ª∫ (CD105: 99,4±0,23% Î·È CD146: 94,05±1,45%). ™ÙËÓ ƒ2 ÙÔ Û‡ÓÔÏÔ ÙˆÓ ·ÔÈÎÈÒÓ CFU-F ‹Ù·Ó 38,05±3,7 Î·È ÛÙËÓ P4 ‹Ù·Ó 24,03±3. √ ¯ÚfiÓÔ˜ ΢ÙÙ·ÚÈÎÔ‡ ‰ÈÏ·ÛÈ·ÛÌÔ‡ ÛÙËÓ ƒ2 ‹Ù·Ó 1,93±0,1 Ë̤Ú˜ Î·È ÛÙËÓ ƒ4 2,6±0,2 Ë̤Ú˜. ∞fi ÙËÓ ÈÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË Ê¿ÓËΠfiÙÈ Ù· ª∫ ¤¯Ô˘Ó ÈηÓfiÙËÙ· ‰È·ÊÔÚÔÔ›ËÛ˘ ÚÔ˜ ÔÛÙÂÔ·ÙÙ·Ú·, ÏÈÔ·ÙÙ·Ú·, ¯ÔÓ‰ÚÔ·ÙÙ·Ú· (∂ÈÎfiÓ· 2). ∏ ·Ó¿Ï˘ÛË Ì RT-PCR ¤‰ÂÈÍ fiÙÈ Ù· ·ÙÙ·Ú· ·˘Ù¿ ÂÎÊÚ¿˙Ô˘Ó ÌÔÚÈ·ÎÔ‡˜ ‰Â›ÎÙ˜ ÂȉÈÎÔ‡˜ ÁÈ· ÔÛÙÂÔ·ÙÙ·Ú·, ÏÈÔ·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 ·ÔÌÔÓÒıËÎ·Ó 2,9±0,5x108 ª¡¶. ªÂÙ¿ ÙÔÓ ·ÓÔÛÔÌ·ÁÓËÙÈÎfi ‰È·¯ˆÚÈÛÌfi, Ù· ·ÙÙ·Ú· ‹Ù·Ó 54,3±2,1x104 Î·È ÂͤÊÚ·˙·Ó CD34 Û ÔÛÔÛÙfi 93±1,43%. ∂›‰Ú·ÛË Ù˘ ·ÚÔ˘Û›·˜ ª∫ ÛÙËÓ ¤ÎÙ˘ÍË ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ΢ÙÙ¿ÚˆÓ ∏ Ù·˘Ùfi¯ÚÔÓË ·ÚÔ˘Û›· ª∫ Î·È ·˘ÍËÙÈÎÒÓ ¶·È‰È·ÙÚÈ΋ 2009;72:111-000

Pediatri Mar-Apr 09

10-04-09

15:37

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∂ÈÎfiÓ· 2. πÛÙÔ¯ËÌÈΤ˜ ¯ÚÒÛÂȘ ·fi ‰È·ÊÔÚÔÔÈË̤ӷ ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·: ·) ÏÈÔ·ÙÙ·Ú·, ‚) ÔÛÙÂÔ·ÙÙ·Ú·, Á) ¯ÔÓ‰ÚÔ·ÙÙ·Ú·.

·Ú·ÁfiÓÙˆÓ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË ÌÂÁ·Ï‡ÙÂÚË ¤ÎÙ˘ÍË ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÙÙ¿ÚˆÓ (Û˘Óı‹ÎË B/° Û ۇÁÎÚÈÛË ÌÂ Û˘Óı‹Î˜ ¢/∂) (∂ÈÎfiÓ· 1), ÂÓÒ ÔÈ Û˘Ó‰˘·ÛÌÔ› ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ‰ÂÓ ‰È¤ÊÂÚ·Ó ÛÙËÓ Â›‰Ú·ÛË Ô˘ ›¯·Ó ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ Î˘ÙÙ¿ÚˆÓ (¶›Ó·Î·˜ 1, Û˘Óı‹ÎË µ Î·È °). ∆· ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· Ô˘ ηÏÏÈÂÚÁ‹ıËÎ·Ó ·ÔÎÏÂÈÛÙÈο ·ÚÔ˘Û›· ΢ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ (Û˘Óı‹ÎË ∞) ·˘Í‹ıËÎ·Ó Î·Ù¿ 10,9±5,73 ÊÔÚ¤˜ Î·È ·˘Ù‹ Ë Û˘Óı‹ÎË ·¤ÊÂÚ ÙË ÌÈÎÚfiÙÂÚË ¤ÎÙ˘ÍË (p≤0,05) Û ۇÁÎÚÈÛË Ì fiϘ ÙȘ ˘fiÏÔÈ˜ (¶›Ó·Î·˜ 1). ∂›‰Ú·ÛË Ù˘ ·ÚÔ˘Û›·˜ ª∫ ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ ·Ú¯¤ÁÔÓˆÓ/ÚÔÁÔÓÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ 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) Â¤ÊÂÚ ÙË ÌÂÁ·Ï‡ÙÂÚË ¤ÎÙ˘ÍË ÛÙ· CD34+ ·ÙÙ·Ú·, ÂÓÒ ÙÔ Ùˆ¯fiÙÂÚÔ ÔÛÔÙÈο ·ÔÙ¤ÏÂÛÌ· ›¯Â Ë Û˘Óı‹ÎË ∞ (p=0,025). ∏ ηÏÏȤÚÁÂÈ· ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÌfiÓÔ (Û˘Óı‹Î˜ ¢ Î·È ∂) Â¤ÊÂÚ ÈηÓÔÔÈËÙÈ΋ ¤ÎÙ˘Í‹ ÙÔ˘˜, Ë ÔÔ›·, ·Ó Î·È ·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 ÂÎÙ˘ÁÌ¤ÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ √ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ¤ÎÊÚ·Û˘ ÙÔ˘ CD34 ¤‰ÂÈÍÂ

ˆ˜ Ë ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ·fi ÙËÓ Î·ÏÏȤÚÁÂÈ· ¤¯ÂÈ ˆ˜ Û˘Ó¤ÂÈ· ÌÂÁ·Ï‡ÙÂÚÔ ÂηÙÔÛÙÈ·›Ô ÔÛÔÛÙfi ΢ÙÙ¿ÚˆÓ Ô˘ ÙÔ ÂÎÊÚ¿˙Ô˘Ó (¶›Ó·Î·˜ 1). ∆· ÔÛÔÛÙ¿ ÙˆÓ ÏËı˘ÛÌÒÓ CD34+38- Î·È CD34+33- Ô˘ ˘Ô‰ÂÈÎÓ‡Ô˘Ó Î‡ÙÙ·Ú· Ì ¯·Ú·ÎÙËÚÈÛÙÈο ·ˆÚfiÙËÙ·˜ ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚ· ÛÙË Û˘Óı‹ÎË ∞ (·ÚÔ˘Û›· ÌfiÓÔ ª∫), Û ۇÁÎÚÈÛË Ì ÙȘ ˘fiÏÔÈ˜ Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ (µ/°/¢ Î·È ∂). ∆Ô ÔÛÔÛÙfi ÙˆÓ 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˘Óı‹ÎË ¢. (∂ÈÎfiÓ· 3). ∆· ·ÓÙ›ÛÙÔȯ· ÔÛÔÛÙ¿ ÁÈ· Ù· CD34+38- ·ÙÙ·Ú· ‹Ù·Ó 5,6±3,4%, 1,3±0,9% Î·È 4,2±3,8%. ¢ÂÓ ·Ú·ÙËÚ‹ıËΠ‰È·ÊÔÚ¿ ·˘ÙÒÓ ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ˘ÔÏËı˘ÛÌÒÓ ÂÍ·ÚÙÒÌÂÓË ·fi ÙÔÓ Û˘Ó‰˘·ÛÌfi ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (Û˘Óı‹Î˜ µ ÚÔ˜ ° Î·È ¢ ÚÔ˜ ∂) (∂ÈÎfiÓ· 3). ∞Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ ÎÔÎÎÈÒ‰Ô˘˜ (CFU-GM) Î·È ÂÚ˘ıÚ¿˜ (BFU-E) ÛÂÈÚ¿˜ ∞Ó¿Ù˘ÍË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÔÈÎÈÒÓ CFUGM (186,81±9,9) ·Ú·ÙËÚ‹ıËΠ·ÚÔ˘Û›· ÌfiÓÔ Î˘ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ (∂ÈÎfiÓ· 4). ™˘ÁÎÚ›ÓÔÓÙ·˜ Ì fiϘ ÙȘ ˘fiÏÔÈ˜ Û˘Óı‹Î˜, Ê·›ÓÂÙ·È ˆ˜ Ë ·ÚÔ˘Û›· ÌfiÓÔ ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ·, ÌÔÏÔÓfiÙÈ ‰ÂÓ ÚÔ¿ÁÂÈ ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ Î˘ÙÙ¿ÚˆÓ (ÌÈÎÚfiÙÂÚË ¤ÎÙ˘ÍË ÙÔ˘ ÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ Î·È ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ CD34+), ÂÓ ÙÔ‡ÙÔȘ ·Ú¤¯ÂÈ Î‡ÙÙ·Ú· Ì ·˘ÍË̤ÓË ÎψÓÔÁÔÓÈ΋ ÈηÓfiÙËÙ·. ∂›Û˘, ·Ú·ÙËÚÂ›Ù·È ‰È·ÊÔÚÂÙÈÎfi˜ Ú˘ıÌfi˜ ·Ó¿Ù˘Í˘ ·ÔÈÎÈÒÓ ·Ó¿ÏÔÁ· Ì ÙÔÓ Û˘Ó‰˘·ÛÌfi ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Î·È Ô GFmix1 (Û˘Óı‹ÎË ¢) ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ·Ó¿Ù˘ÍË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÔÈÎÈÒÓ (147,0±12,9), ÛÂ

¶›Ó·Î·˜ 1. ∞ÔÙÂϤÛÌ·Ù· ¤ÎÙ˘Í˘ ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÙÙ¿ÚˆÓ Î·È ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ Î·È ÔÛÔÛÙfi ¤ÎÊÚ·Û˘ ÙÔ˘ CD34 ·ÓÙÈÁfiÓÔ˘ ÂÈÊ·Ó›·˜ ÌÂÙ¿ ·fi ‰¤Î· Ë̤Ú˜ Û˘ÁηÏÏȤÚÁÂÈ·˜

∞ µ ° ¢ ∂

™˘Óı‹ÎË

ŒÎÙ˘ÍË ÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ΢ÙÙ¿ÚˆÓ (x)

ŒÎÙ˘ÍË CD34+

% CD34

CD34∆ + ª∫ CD34∆ + ª∫ + GFmix1 CD34∆ + ª∫ + GFmix2 CD34∆ + + GFmix1 CD34∆ + + GFmix2

10,9 ± 5,7 164,4 ± 42,7* 165,8 ± 35,8* 101, 9 ± 36,3* 134,2 ± 10,8*

2,2 ± 0 ,7 20,9 ± 5,9* 16,8 ± 0,5* 13,7 ± 9,7 14,2 ± 1,7*

50,6 ± 5,5 13,1± 0,9* 11,4 ± 2,0* 10,9 ± 5,5 10,5 ± 0,4

*ηٷ‰ÂÈÎÓ‡ÂÈ ÛÙ·ÙÈÛÙÈ΋ ÛËÌ·ÓÙÈÎfiÙËÙ·, p<0,05 Û ۇÁÎÚÈÛË Ì ÙË Û˘Óı‹ÎË ∞ Paediatriki 2009;72:112-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·113

113

ªÂÛÂÁ¯˘Ì·ÙÈο Î·È ¤ÎÙ˘ÍË ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜

CD33 PC5

A

40,48%

°

1,40%

AA

B

4,15%

27,45%

¢

¢¢

4,00%

BB

15,20%

E

17,95%

0,45%

B

BB

CD38 PC5

CD34 PE

A

AA

5,63%

7,15%

°

0,40%

1,35%

¢

¢¢

4,20%

20,18%

2,83%

E

0,45%

CD34 PE ∂ÈÎfiÓ· 3. ∞ÓÔÛÔÊ·ÈÓÔÙ˘È΋ ·Ó¿Ï˘ÛË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ 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.

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. ™˘ÁÎÂÎÚÈ̤ӷ, ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ·ÔÈ˘ ‰ËÌÈÔ˘ÚÁ‹ıËÎ·Ó ·fi Ù· ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ·ÚÔ˘Û›· ÌfiÓÔ ª∫ (Û˘Óı‹ÎË ∞), ÂÓÒ ÔÈ ÏÈÁfiÙÂÚ˜ ÛÙȘ Û˘Óı‹Î˜ ¢ Î·È ∂ fiÔ˘ ·Ô˘Û›·˙·Ó ÙÂÏ›ˆ˜ Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο (p=0,03 Î·È p=0,01, ·ÓÙ›ÛÙÔȯ·, Û ۇÁÎÚÈÛË Ì ™˘Óı‹ÎË ∞) (∂ÈÎfiÓ· 4). ¶·ÚÔÌÔ›ˆ˜, fiˆ˜ 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˘ ÌÈÎÚÔÂÚÈ‚¿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·ÊÔÚÔÔ›ËÛ˘ ÙˆÓ ·Ú¯¤ÁÔÓˆÓ/ÚÔÁÔÓÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. √ ·ÎÚÈ‚‹˜ Ì˯·ÓÈÛÌfi˜ ·˘Ù‹˜ Ù˘ ‰È·‰Èηۛ·˜ ‰ÂÓ ¤¯ÂÈ ·ÔÛ·ÊËÓÈÛÙ›, ·ÏÏ¿ ¶·È‰È·ÙÚÈ΋ 2009;72:113-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·114

114

π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.

∞ÚÈıÌfi˜ ·ÔÈÎÈÒÓ/ 5x103 ·ÙÙ·Ú·

180

CFU-GM BFU-E

150 120 90 60 30 0 A

B

°

¢

∂

™˘Óı‹ÎË ∂ÈÎfiÓ· 4. ∞Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ CFU-GM Î·È BFU-E ÛÙȘ ‰È·ÊÔÚÂÙÈΤ˜ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜.

Èı·ÓÒ˜ ÂÈÙ˘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 ¤ÎÙ˘ÍË ·›Ì·ÙÔ˜ √§, ÛÙËÓ ÚÔÛ¿ıÂÈ· ·ÓÙÈÌÂÙÒÈÛ˘ 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 ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ™Ù· Ï·›ÛÈ· ·˘Ù¿ ‰ÔÎÈÌ¿ÛÙËÎ·Ó ‰È¿ÊÔÚ˜ Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜, ÔÈ Ôԛ˜ ÂÚÈÂÏ¿Ì‚·Ó·Ó ΢ڛˆ˜ ‰È·ÊÔÚÂÙÈÎÔ‡˜ Û˘Ó‰˘·ÛÌÔ‡˜ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (6-8,19). ™ÎÔfi˜ ‹Ù·Ó Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ È‰·ÓÈÎÒÓ Û˘ÓıËÎÒÓ Î·È Ù˘ Â›‰Ú·Û˘ Û˘Ó‰˘·ÛÌÔ‡ ·Ú·ÁfiÓÙˆÓ Ô˘, ÌÂÙ¿ ·fi Û¯ÂÙÈο Û‡ÓÙÔÌË Î·ÏÏȤÚÁÂÈ·, ı· ÌÔÚÔ‡Û ӷ ·Ú¤¯ÂÈ ·Ú¯¤ÁÔÓ· ·ÈÌÔÔÈËÙÈο ·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 ÌÔÚ› Ó· ıˆÚËı› fiÙÈ ·ÓÙÈÌÂÙÒÈÛ·Ó ÈηÓÔÔÈËÙÈο ÙÔ Úfi‚ÏËÌ·, ·ÊÔ‡, Û‡Ìʈӷ Ì ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Ê¿Û˘ π, ‰ÂÓ ·Ú·ÙËÚ‹ıËΠÛÙ·ıÂÚ‹ ·ÔηٿÛÙ·ÛË Ù˘ ·ÈÌÔÔ›ËÛ˘, Èı·ÓÒ˜ ÁÈ·Ù› ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ¤ÎÙ˘Í˘ ÔÈ Î·ÏÏÈÂÚÁËÙÈΤ˜ Û˘Óı‹Î˜ Ô‰ËÁÔ‡Û·Ó Û ÈÔ ‰È·ÊÔÚÔÔÈË̤ӷ ·ÙÙ·Ú· (20-21). ¶ÈÔ ÚfiÛÊ·Ù·, ÂÍÂÙ¿ÛÙËÎÂ Â¿Ó Ù· ª∫ ı· ÌÔÚÔ‡Û·Ó Ó· Û˘ÁηÏÏÈÂÚÁËıÔ‡Ó Ì ·Ú¯¤ÁÔÓ· ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ÁÈ· Ó· ÂÈÙ¢¯ı› ηχÙÂÚË ex vivo ¤ÎÙ˘ÍË ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ (22). ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ ‹Ù·Ó Ë in vitro ÌÂϤÙË Ù˘ Â›‰Ú·Û˘ ÙˆÓ ª∫ ·fi ª√ ·È‰ÈÒÓ Ì ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· Û CD34+ ·ÙÙ·Ú· ·›Ì·ÙÔ˜ √§ Î·È Ë Û‡ÁÎÚÈÛË ÙˆÓ ·ÔÙÂϤÛÌ·ÙˆÓ Ì Paediatriki 2009;72:114-000

·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 ÂÚÈÔÚÈṲ̂ÓË ¤ÎÙ˘ÍË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ/ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ, ·ÏÏ¿ ·ÔʤÚÂÈ ÏËı˘ÛÌfi Ô˘ ÂÎÊÚ¿˙ÂÈ Û ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÙÔ ·ÓÙÈÁfiÓÔ ÂÈÊ·Ó›·˜ CD34. ∆· ·ÔÙÂϤÛÌ·Ù· ·˘Ù¿ Û˘ÁÎÏ›ÓÔ˘Ó Ì ·˘Ù¿ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ·fi ÙÔ˘˜ Jang et al. (23), ÔÈ ÔÔ›ÔÈ fï˜ ¯ÚËÛÈÌÔÔ›ËÛ·Ó ª∫ ·fi ·›Ì· √§. ∞Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ Ë ÂÈÙ˘¯‹˜ ·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 Â›Û˘ Û‡ÁÎÚÈÛË Ù˘ ¤ÎÙ˘Í˘ CD34+ Ì ٷ ·ÔÙÂϤÛÌ·Ù· Ô˘ ÚԤ΢„·Ó ·fi ÙËÓ ¤ÎÙ˘ÍË CD34+ ÛÂ Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ Ì ª∫ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·fi ˘ÁÈ›˜ ÂÓ‹ÏÈΘ. ∞fi Ù· ·ÔÙÂϤÛÌ·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˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. √È Yamaguchi et al. (10) Â›Û˘ ·Ó·Ê¤ÚÔ˘Ó ·Ó¿ÏÔÁË ¤ÎÙ˘ÍË CD34+ Ì ÙË ¯Ú‹ÛË ÌfiÓÔ Î˘ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ ·fi ª√ ÂÓ‹ÏÈÎˆÓ ‰ÔÙÒÓ ‹ ÂÓ‹ÏÈÎˆÓ ·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 ·ÔÙÂÏÂÛÌ¿ÙˆÓ. ∂ÈϤÔÓ, ÛÙË ÌÂϤÙË ·˘Ù‹ ‰ÂÓ ·Ó·Ê¤ÚÂÙ·È ·Ó ˘‹ÚÍ·Ó ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ˘ÁÈÒÓ Î·È ·ÛıÂÓÒÓ ÛÙËÓ ¤ÎÙ˘ÍË ÙÔ˘ ˘ÔÏËı˘ÛÌÔ‡ ÙˆÓ ÚÔÁÔÓÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ (10).

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·115

115

ªÂÛÂÁ¯˘Ì·ÙÈο Î·È ¤ÎÙ˘ÍË ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜

°È· Ó· ÂÏÂÁ¯ıÔ‡Ó ·ÊÂÓfi˜ Ë ÈηÓfiÙËÙ· ÙˆÓ ª∫ Ó· ÂÓÈÛ¯‡ÛÔ˘Ó ÙËÓ ¤ÎÙ˘ÍË ÙˆÓ ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ 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 CFUGM Î·È BFU-E ·ÔÈÎÈÒÓ. ¶·Ú·ÙËÚ‹ıËΠÌÂÁ·Ï‡ÙÂÚÔ˜ ·ÚÈıÌfi˜ Î·È ÙˆÓ ‰‡Ô Ù‡ˆÓ ·ÔÈÎÈÒÓ ÛÙȘ Û˘Óı‹Î˜ fiÔ˘ ˘‹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›ÏÂÙ·È ÛÙÔ fiÙÈ Ù· ·ÙÙ·Ú· ˘fi ÙȘ Û˘ÁÎÂÎÚÈ̤Ó˜ Û˘Óı‹Î˜ ˆı‹ıËÎ·Ó Û ‰È·ÊÔÚÔÔ›ËÛË ·ÊÔ‡ Â‹Ïı ηٷӿψÛË ‹ ·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‹ıËΠÂ›Û˘ Î·È ·ÚÔ˘Û›· ÌfiÓÔ Î˘ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ ÌÂÙ¿ ÙËÓ 7Ë Ë̤ڷ ηÏÏȤÚÁÂÈ·˜ (23). ∞Í›˙ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ ÔÈ Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ ‰ÂÓ Â›Ó·È Ù·˘ÙfiÛË̘ ÛÙȘ Û˘ÁÎÚÈÓfiÌÂÓ˜ ÌÂϤÙ˜, ηıÒ˜, ¤Ú·Ó Ù˘ ÌÂÁ·Ï‡ÙÂÚ˘ ‰È¿ÚÎÂÈ·˜ Ù˘ ηÏÏȤÚÁÂÈ·˜, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÔÈÔÙÈο Î·È ÔÛÔÙÈο ‰È·ÊÔÚÂÙÈÎÔ› Û˘Ó‰˘·ÛÌÔ› ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ηıÒ˜ Î·È ª∫ ΢ڛˆ˜ √§, ·ÏÏ¿ Î·È ÂÓËϛΈÓ. ∞fi Ù· ‰Â‰Ô̤ӷ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ Ê·›ÓÂÙ·È, ÏÔÈfiÓ, fiÙÈ Ë ¯Ú‹ÛË ª∫ ÛÙËÓ ¤ÎÙ˘ÍË ·¤‰ˆÛ ÙÔÓ ÌÂÁ·Ï‡ÙÂÚÔ ·ÚÈıÌfi ·ÔÈÎÈÒÓ ÙfiÛÔ Ù˘ ÎÔÎÎÈÒ‰Ô˘˜ fiÛÔ Î·È Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ ·fi ÔÔÈ·‰‹ÔÙ ¿ÏÏË Û˘Óı‹ÎË Î·ÏÏȤÚÁÂÈ·˜ Ô˘ ‰ÔÎÈÌ¿ÛÙËÎÂ, 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 ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ, ·ÚÔ˘Û›·Û·Ó ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ¤ÎÊÚ·Û˘ Â›Û˘ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· fiÔ˘ Ù· CD34+ ·ÈÌÔÔÈËÙÈο Û˘Ó˘‹Ú¯·Ó Ì ª∫ ÌfiÓÔ. √È Chute et al. (27) ¤‰ÂÈÍ·Ó ÂȉÈÎfiÙÂÚ· fiÙÈ Ù· CD34+CD38- ÌÔÚÔ‡Ó, Â·ÁfiÌÂÓ· Û ΢ÙÙ·ÚÈÎfi ·ÎÏÔ, Ó· ÂÎÙ˘¯ıÔ‡Ó in vitro. T· ·ÙÙ·Ú· Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙËÓ ¤ÎÙ˘ÍË ‰È·ÙËÚÔ‡Ó ÙËÓ ¤ÎÊÚ·ÛË ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘, Ù· ÔÔ›· Èı·ÓÒ˜ ¤¯Ô˘Ó ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÂÁηٿÛÙ·ÛË ÙˆÓ ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ Î·È ÛÙËÓ ·ÔηٿÛÙ·ÛË Ù˘ ·ÈÌÔÔ›ËÛ˘. ∏ ȉÈfiÙËÙ· ·˘Ù‹ ÙˆÓ ª∫ ÌÔÚ› Ó· ·ÍÈÔÔÈËı› ÎÏÈÓÈο ÛÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË. ∞Ó Î·È Ô Ì˯·ÓÈÛÌfi˜ ·ÏÏËÏÂ›‰Ú·Û˘ ÙˆÓ ª∫ Ì ٷ ÚÔÁÔÓÈο ·ÈÌÔÔÈËÙÈο ·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 ÔÔ›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˘ ¯ÚËÛÈÌÔÔÈ‹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‹ıËΠ·Ó¿Ù˘ÍË-‰È·ÊÔÚÔÔ›ËÛË Û ϤÔÓ ÒÚÈÌ· ÛÙÔȯ›· Ù˘ ηÏÏȤÚÁÂÈ·˜ (ÔÏÈÎfi˜ ·ÚÈıÌfi˜ ΢ÙÙ¿ÚˆÓ, CD34+ Ì ÌÈÎÚfiÙÂÚË ÎψÓÔÁÔÓÈÎfiÙËÙ· Î.Ï.), ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ Â¿Ó Ô Û˘Ó‰˘·ÛÌfi˜ ΢ÙÙ·ÚÔÎÈÓÒÓ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËΠÂȉڿ ΢ڛˆ˜ Û ·Ú¯¤ÁÔÓ· ‹ ÈÔ ÒÚÈÌ· ‰ÂÛÌÂ˘Ì¤Ó· ÚÔÁÔÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· (30). ™˘ÌÂÚ·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 ¤ÎÙ˘ÍË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·fi ·›Ì· √§, Û ۇÁÎÚÈÛË Ì ÙËÓ ¤ÎÙ˘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˘ ·ÈÌÔÔ›ËÛ˘ ÌÂÙ¿ ·fi ÌÂÙ·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 reconstitution. Exp Hematol 2003;31:261-270.

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 cell for self-renewal and amplification vs. proliferation and differentiation. Leukemia 1998;12:718-727.

9. Ueda T, Tsuji K, Yoshino H, Ebihara Y, Yagasaki H, Hisakawa H, et al. Expansion of human NOD/ SCIDrepopulating 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, ¶·È‰È·ÙÚÈ΋ 2009;72:115-000

Pediatri Mar-Apr 09

10-04-09

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™ÂÏ›‰·116

116

π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.

Yamashita K, et al. Serum-free co-culture system for ex vivo expansion of human cord blood primitive progenitors and SCID mouse-reconstituting cells using human bone marrow primary stromal cells. Exp Hematol 2001;29: 174-182.

11. Shih CC, Hu MC, Hu J, Medeiros J, Forman SJ. Long-term ex vivo maintenance and expansion of transplantable human hematopoietic stem cells. Blood 1999;94:16231636.

12. Thiemann FT, Moore KA, Smogorzewska EM, Lemischka IR, Crooks GM. The murine stromal cell line AFT024 acts specifically on human CD34-CD38- progenitors to maintain primitive function and immunophenotype in vitro. 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 human umbilical cord hematopoietic progenitors using a novel culture system. Exp Hematol 1999;27:904-915.

14. Woodbury D, Reynolds K, Black IB. Adult bone marrow stromal stem cells express germline, ectodermal, endodermal, and mesodermal genes prior to neurogenesis.

J Neurosci Res 2002;69:908-917.

15. Campagnoli C, Roberts IA, Kumar S, Bennett PR, Bellantuono I, Fisk NM. Identification of mesenchymal stem/progenitor cells in human first-trimester fetal blood, liver, and bone marrow. Blood 2001;98:2396-2402.

16. Koc ON, Gerson SL, Cooper BW. Rapid hematopoietic recovery after coinfusion of autologous-blood stem cells and culture-expanded marrow mesenchymal stem cells in advanced breast cancer patients receiving high-dose chemotherapy. J Clin Oncol 2000;18:307-316.

17. Lazarus HM., Koc ON, Devine SM. Cotransplantation of HLA-identical sibling culture-expanded mesenchymal stem cells and hematopoietic stem cells in hematologic malignancy patients. Biol Blood Marrow Transplant 2005; 11:389-398.

18. Breems DA, Blokland EA, Siebel KE, Mayen AE, Engels LJ, Ploemacher RE. Stroma-contact prevents loss of hematopoietic stem cell quality during ex vivo expansion of CD34+ mobilized peripheral blood stem cells. Blood.

1998;91:111-117.

19. Dimitriou H, Vorgia P, Stiakaki E, Mavroudis D, Markaki EA, Koumantakis E, et al. In vitro proliferative and differentiating characteristics of CD133(+) and CD34(+) cord blood cells in the presence of thrombopoietin (TPO) or erythropoietin (EPO). Potential implications for hematopoietic cell transplantation. Leuk Res. 2003;27:1143-1151.

20. Kögler G, Nürnberger W, Fischer J, Niehues T, Somville T,

Paediatriki 2009;72:116-000

Göbel U, et al. Simultaneous cord blood transplantation of ex vivo expanded together with nonexpanded cells for high risk leukemia. Bone Marrow Transplant 1999;24:397.

21. Koller MR, Manchel I, Maher RJ, Goltry KL, Armstrong RD, Smith AK. Clinical-scale human umbilical cord blood cell expansion in a novel automated perfusion culture system. Bone Marrow Transplant 1998;21:653.

22. XM Fei, YJ Wu, Z Chang, KR Miao, YH Tang, XY Zhou, et al. Co-culture of cord blood CD34 cells with human BM mesenchymal stromal cells enhance short-term engraftment of cord blood cells in NOD/SCID mice.

Cytotherapy 2007;9:338-347.

23. Jang YK, Jung DH, Jung MH, Kim DH, Yoo KH, Sung KW, et al. Mesenchymal stem cells feeder layer from human umbilical cord blood for ex vivo expanded growth and proliferation of hematopoietic progenitor cells. Ann Hematol 2006;85:212-225.

24. Wexler SA, Donaldson C, Denning-Kendall P, Rice C, Bradley B, Hows JM. Adult bone marrow is a rich source of human mesenchymal stem cells but umbilical cord and mobilized adult blood are not. Br J Haematol 2003;121: 368-374.

25. Yu M, Xiao Z, Shen L, Li L. Mid-trimester fetal bloodderived adherent cells share characteristics similar to mesenchymal stem cells but full-term umbilical cord blood does 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. Haematologica 2001;86:1099-1100.

27. Chute JP, Saini AA, Kampen RL, Wells MR, Davis TA. A comparative study of the cell cycle status and primitive cell adhesion molecule profile of human CD34+ cells cultured in stromal-free versus porcine microvascular endothelial cell cultures. Exp Hematol 1999;27:370-379.

28. Haynesworth SE, Baber MA, Caplan AI. Cytokine expression by human marrow-derived mesenchymal progenitor cells in vitro: effects of dexamethasone and IL-1 alpha. J Cell Physiol 1996;166:585-592.

29. Dormady SP, Bashayan O, Dougherty R, Zhang XM, Basch RS. Immortalized multipotential mesenchymal cells and the hematopoietic microenvironment. J Hematother Stem Cell Res 2001;10:125-140.

30. Ueda T, Tsuji K, Yoshino H, Ebihara Y, Yagasaki H, Hisakawa H, et al. Expansion of human NOD/SCIDrepopulating cells by stem cell factor, Flk2/Flt3 ligand, thrombopoietin, IL-6, and soluble IL-6 receptor. J Clin Invest 2000;105:1013-1021.

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ORIGINAL ARTICLE

117

¶·ÓÂÏÏ‹ÓÈ· ÔÚÔÂȉËÌÈÔÏÔÁÈ΋ 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·ÓÙfiÔ˘ÏÔ˜7 ¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: ∏ Ë·Ù›Ùȉ· ∞ ·ÔÙÂÏ› ÂÓ‰ËÌÈÎfi ÓfiÛËÌ· Û ÔÏϤ˜ ÂÚÈÔ¯¤˜ ÙÔ˘ ÎfiÛÌÔ˘. ∏ ∂ÏÏ¿‰· ηٷٿÛÛÂÙ·È ÛÙȘ ¯ÒÚ˜ ÂӉȿÌÂÛ˘ ÂÓ‰ËÌÈÎfiÙËÙ·˜, ·ÏÏ¿ ‰ÂÓ ˘¿Ú¯Ô˘Ó Â·Ú΋ ÚfiÛÊ·Ù· ÔÚÔÂȉËÌÈÔÏÔÁÈο ‰Â‰Ô̤ӷ ÁÈ· ÙË ÓfiÛÔ. ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ·ÍÈÔÏfiÁËÛË Ù˘ ÔÚÔÂȉËÌÈÔÏÔÁ›·˜ ÙÔ˘ ÈÔ‡ Ù˘ Ë·Ù›Ùȉ·˜ ∞, ·Ó¿ ËÏÈΛ· Î·È ÁˆÁÚ·ÊÈÎfi ‰È·Ì¤ÚÈÛÌ· Ù˘ ¯ÒÚ·˜, Û ·ÓÙÈÚÔÛˆ¢ÙÈÎfi ‰Â›ÁÌ· ·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‰Ô̤ӈÓ, ·fi fiÏ· Ù· ÁˆÁÚ·ÊÈο ‰È·ÌÂÚ›ÛÌ·Ù· Ù˘ ¯ÒÚ·˜, Û‡Ìʈӷ Ì ÙÔ Û¯‹Ì· Û˘ÏÏÔÁ‹˜ ÂÓ·ÔÌÂÈÓ¿ÓÙˆÓ ÔÚÒÓ Ô˘ ¯ÚËÛÈÌÔÔÈ› ÙÔ Â˘Úˆ·˚Îfi ‰›ÎÙ˘Ô ÔÚÔÂÈÙ‹ÚËÛ˘ ÓÔÛËÌ¿ÙˆÓ Ô˘ ÚÔÏ·Ì‚¿ÓÔÓÙ·È Ì ÂÌ‚ÔÏÈ·ÛÌfi (∂SEN II), ÛÙÔ˘˜ ÔÔ›Ô˘˜ ¤ÁÈÓ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ IgG ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ Ù˘ Ë·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¯¤˜, ›¯·Ó ÛÙ·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›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 ÔÚÔıÂÙÈÎfiÙËÙ·˜ ÏfiÁˆ Ê˘ÛÈ΋˜ ÓfiÛËÛ˘, Û ۇÁÎÚÈÛË Ì ٷ ∂ÏÏËÓfiÔ˘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. ™˘ÌÂÚ¿ÛÌ·Ù·: ∞fi Ù· ÛÙÔȯ›· Ù˘ ÌÂϤÙ˘ Ì·˜ ÚÔ·ÙÂÈ fiÙÈ ÂÈ‚¿ÏÏÂÙ·È Ë ÂÊ·ÚÌÔÁ‹ ηıÔÏÈÎÔ‡ ÂÌ‚ÔÏÈ·ÛÌÔ‡ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ ÛÙË ¯ÒÚ· Ì·˜.

1 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó· 2 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£, ¡ÔÛÔÎÔÌÂ›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΋ ¶·ÓÂÈÛÙËÌ›Ô˘ πˆ·ÓÓ›ÓˆÓ 6 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, «µÂÓÈ˙¤ÏÂÈÔ» ¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ 7 ™¯ÔÏ‹ ÀÁÈÂÈÓ‹˜ Î·È ∂ȉËÌÈÔÏÔÁ›·˜ ∂∫¶∞ AÏÏËÏÔÁÚ·Ê›·: µ·ÛÈÏÈ΋ ¶··Â˘·ÁÁ¤ÏÔ˘ atriagia@auth.gr, vpapaev@med.uoa.gr µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

§¤ÍÂȘ ÎÏÂȉȿ: ∏·Ù›Ùȉ· ∞, ÂÌ‚fiÏÈÔ, ÔÚÔÂȉËÌÈÔÏÔÁÈ΋.

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 country of intermediate endemicity, but recent data are lacking. A national cross-sectional seroprevalence survey was conducted to evaluate the current seroepidemiology of hepatitis A in children in the age group 0-14 years living in Greece.

Methods: Using the ESSEN II methodology, 100 sera per year of age, stratified by geographic region, were collected and tested for the presence of anti-HAV IgG antibodies (AXSYM, Abbott Laboratories). Data from 1,393 children were analyzed.

Results: Immigrant children and children residing in rural areas were significantly less likely to be immunized. Among unvaccinated children, the seroprevalence was 17.07%. Nationality had a marginally significant 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 in both vaccination coverage and natural immunity.

Conclusions: These data indicate that hepatitis A continues to prevail in Greece and therefore universal infant immunization should be considered.

Key words: Hepatitis A, vaccine, seroepidemiology.

1 2nd Department of Paediatrics, General Children’s Hospital “P. & A. Kyriakou”, University of Athens, Athens, Greece 2 2nd Department of Paediatrics, “AHEPA” Hospital, “Aristotelio” University of Thessaloniki, Thessaloniki, Greece 3 Department of Paediatrics, “Dimokritio” University of Thrace, Alexandroupolis, Greece 4 Laboratory for Blood Donation, “Agia Sophia” General Children’s Hospital, Athens, Greece 5 Department of Paediatrics, University of Ioannina, Ioannina, Greece 6 Department of Paediatrics, “Venizelio” Hospital of Heraklion, Heraklion, Greece 7 School of Hygiene and Epidemiology, EKPA Correspondence: Vassiliki Papaevagelou atriagia@auth.gr, vpapaev@med.uoa.gr 2nd Department of Paediatrics, General Children’s Hospital “P. & A. Kyriakou”, University of Athens, Athens, Greece

* ∏ ÂÚÁ·Û›· ·˘Ù‹ ‚Ú·‚‡ıËΠ̠ÙÔ Œ·ıÏÔ ¢ÔÍÈ¿‰Ë, ηٿ ÙÔ 46Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ∫¤Ú΢ڷ 2008

¶·È‰È·ÙÚÈ΋ 2009;72:117-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·118

118

µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.

∂ÈÛ·ÁˆÁ‹ ∏ Ë·Ù›Ùȉ· ∞ ·ÔÙÂÏ› Û˘¯Ófi ÏÔÈÌ҉˜ ÓfiÛËÌ·, ·Ú¿ ÙËÓ Î˘ÎÏÔÊÔÚ›· ·ÔÙÂÏÂÛÌ·ÙÈÎÔ‡ ÂÌ‚ÔÏ›Ô˘ (1). ∞Ó Î·È Ù· ÔÛÔÛÙ¿ Ù˘ Â›ÙˆÛ‹˜ 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 ÂȉËÌÈÔÏÔÁ›· ¤¯ÂÈ ·Ú·ÙËÚËı› ÛÙȘ ÂÚÈÔ¯¤˜ fiÔ˘ ÂÊ·ÚÌfiÛÙËΠηıÔÏÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ (1-3). ™ÙËÓ ∂ÏÏ¿‰· ˘¿Ú¯Ô˘Ó ÛÙÔȯ›· ·fi ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Û ÔÚÈṲ̂Ó˜ ÂÚÈÔ¯¤˜ Ù˘ ¯ÒÚ·˜ Ô˘ ·Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Ù· ÔÛÔÛÙ¿ Â›ÙˆÛ˘ Ù˘ ÓfiÛÔ˘ ¤¯Ô˘Ó ÌÂȈı› ÛËÌ·ÓÙÈο Î·È Ë Ì¤ÛË ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ Ì Ë·Ù›Ùȉ· ∞ ¤¯ÂÈ ·˘ÍËı›, fiˆ˜ ÂÚÈÁÚ¿ÊÂÙ·È Î·È Û ¿ÏϘ Â˘Úˆ·˚Τ˜ ¯ÒÚ˜ (4-9). ∂ÓÙÔ‡ÙÔȘ, ÂıÓÈο ÔÚÔÂȉËÌÈÔÏÔÁÈο 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 ÛÎfiÈÌË Ë Û˘ÏÏÔÁ‹ ‰Â‰ÔÌ¤ÓˆÓ ÁÈ· ÙÔÓ ÂÈÔÏ·ÛÌfi Ù˘ ÓfiÛÔ˘ Û ÌÈ· ÏËı˘ÛÌȷ΋ ÔÌ¿‰·, ηıÒ˜ Î·È Ë Î·Ù·ÁÚ·Ê‹ ÙˆÓ ÂÈÏÔÎÒÓ Ù˘. ¢˘ÛÙ˘¯Ò˜, Ù· ‰Â‰Ô̤ӷ ·fi ÙÔÓ ÂÏÏËÓÈÎfi ¯ÒÚÔ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÛÙÔȯ›· Û ÙÔÈÎfi ΢ڛˆ˜ Â›Â‰Ô Î·È ÂӉ¯Ô̤ӈ˜ ı· ÌÔÚÔ‡Û ӷ Á›ÓÂÈ ÂÎÙ›ÌËÛË Ù˘ ÓfiÛÔ˘ ÌfiÓÔ Ì ·Ó·‰ÚÔÌÈ΋ Û˘ÏÏÔÁ‹, ‚¿ÛÂÈ ÛÙÔȯ›ˆÓ ÙˆÓ ÂÈÛ·ÁˆÁÒÓ Û ·È‰È·ÙÚÈΤ˜ ÎÏÈÓÈΤ˜. ™ÎÔfi˜, ÂÔ̤ӈ˜, Ù˘ ·ÚÔ‡Û·˜ ÔÚÔÂȉËÌÈÔÏÔÁÈ΋˜ 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 ·ÓÙÈÚÔÛˆ¢ÙÈÎfi ‰Â›ÁÌ· ·È‰ÈÒÓ 0-14 ÂÙÒÓ ·fi fiÏË ÙËÓ ∂ÏÏ¿‰·. ∆· ·ÔÙÂϤÛÌ·Ù· Ù¤ÙÔÈ·˜ ÌÂϤÙ˘ ı· ·Ú¿Û¯Ô˘Ó ÂӉ¯Ô̤ӈ˜ ¯Ú‹ÛÈÌ· ÛÙÔȯ›· ÛÙËÓ ∂ıÓÈ΋ ∂ÈÙÚÔ‹ ∂Ì‚ÔÏÈ·ÛÌÒÓ. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∞ÛıÂÓ›˜ √Úfi˜ ·›Ì·ÙÔ˜ ·ÓÙÈÚÔÛˆ¢ÙÈÎÔ‡ ‰Â›ÁÌ·ÙÔ˜ ·È‰ÈÒÓ 014 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˘ ¯ÚËÛÈÌÔÔÈ› ÙÔ Â˘Úˆ·˚Îfi ‰›ÎÙ˘Ô ÔÚÔÂÈÙ‹ÚËÛ˘ ÙˆÓ ÓÔÛËÌ¿ÙˆÓ Ô˘ ÚÔÏ·Ì‚¿ÓÔÓÙ·È Ì ÂÌ‚fiÏÈ· (∂SEN II), Ì ÛÎÔfi Ó· ¤¯Ô˘ÌÂ Û˘ÁÎÚ›ÛÈÌ· ·ÔÙÂϤÛÌ·Ù· Ì ·ÓÙ›ÛÙÔȯ˜ Â˘Úˆ·˚Τ˜ ÌÂϤÙ˜. ∆Ô ÔÛÔÛÙfi ÙˆÓ ·È‰ÈÒÓ ·fi οı ÁˆÁÚ·ÊÈ΋ ÂÚÈÔ¯‹ ˘ÔÏÔÁ›ÛÙËΠ̠‚¿ÛË ÙËÓ ÂıÓÈ΋ ·ÔÁÚ·Ê‹ ÙÔ˘ 2001. ∂ÈϤÔÓ, ·fi ÙË ÌÂϤÙË ·ÔÎÏ›ÛÙËÎ·Ó Ù· ·È‰È¿ Ì ¯ÚfiÓÈ· ÓÔÛ‹Ì·Ù·, ÚˆÙÔ·ı›˜, ‰Â˘ÙÂÚÔ·ı›˜ ·ÓÔÛÔ·ÓÂ¿ÚÎÂȘ, ηıÒ˜ Î·È ÂΛӷ Ì ÚfiÛÊ·ÙË Ï‹„Ë ÎÔÈÓ‹˜ Á-ÛÊ·ÈÚ›Ó˘. ∆· ‰Â›ÁÌ·Ù· ÙˆÓ ÔÚÒÓ ÛÙ¿ÏıËÎ·Ó Ù·¯˘‰ÚÔPaediatriki 2009;72:118-000

ÌÈο ÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∞Á›· ™ÔÊ›·». ∆ËÚ‹ıËΠÙÔ ·fiÚÚËÙÔ ÙˆÓ ÚÔÛˆÈÎÒÓ ‰Â‰ÔÌ¤ÓˆÓ ÙˆÓ ·ÛıÂÓÒÓ Ô˘ Û˘ÌÌÂÙ›¯·Ó ÛÙË ÌÂϤÙË. ∆· ‰Â›ÁÌ·Ù· ‰È·ÙËÚ‹ıËÎ·Ó Û ıÂÚÌÔÎÚ·Û›· -20ÔC ̤¯ÚÈ ÙËÓ Âͤٷۋ ÙÔ˘˜. ∆· ‰Â‰Ô̤ӷ ÙˆÓ ·ÛıÂÓÒÓ ÂÈÛ‹¯ıËÛ·Ó Û ÂȉÈο ‰È·ÌÔÚʈ̤ÓË ‚¿ÛË ‰Â‰ÔÌ¤ÓˆÓ Ì Έ‰ÈÎfi ·ÚÈıÌfi, ÒÛÙ ӷ ÂÍ·ÛÊ·ÏÈÛÙ› ·ÊÂÓfi˜ Ë ·ÓˆÓ˘Ì›· Î·È ·ÊÂÙ¤ÚÔ˘ Ë Ï‹Ú˘ ÚÔÛÙ·Û›· ÙˆÓ ÚÔÛˆÈÎÒÓ ‰Â‰ÔÌ¤ÓˆÓ Î¿ı ·ÛıÂÓÔ‡˜. °È· οı ·È‰› ¤ÁÈÓ ηٷÁÚ·Ê‹ Ù˘ ËÏÈΛ·˜, ÙÔ˘ ʇÏÔ˘, ÙÔ˘ ÙfiÔ˘ ‰È·ÌÔÓ‹˜, Ù˘ ˘ËÎÔfiÙËÙ·˜ 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 Ì ¯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·ÓÙÈÎfiÙËÙ·˜ ıˆڋıËÎÂ Ë ÙÈÌ‹ p<0,05. ªÂ ¯Ú‹ÛË ÌÔÓÔÌÂÙ·‚ÏËÙ‹˜ ÏÔÁÈÛÙÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘, ÂϤÁÍ·Ì ÙËÓ Â›‰Ú·ÛË Ù˘ ËÏÈΛ·˜, Ù˘ ÂıÓÈÎfiÙËÙ·˜ Î·È ÙÔ˘ ÙfiÔ˘ ‰È·ÌÔÓ‹˜ ÙˆÓ ·ÛıÂÓÒÓ 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· ·ÍÈÔÈÛÙ›·˜. ∏ ÛÙ·ÙÈÛÙÈ΋ ÛËÌ·ÓÙÈÎfiÙËÙ· (pvalue) ˘ÔÏÔÁ›ÛÙËΠ̠ÙÔ Wald test.

∞ÔÙÂϤÛÌ·Ù· ∏ Û˘ÏÏÔÁ‹ ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ Ù˘ ÌÂϤÙ˘ ¤ÁÈÓ ÌÂٷ͇ 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˘ÛË ÙˆÓ ‰ËÌÔÁÚ·ÊÈÎÒÓ ÛÙÔȯ›ˆÓ 1.393 ‰ÂÈÁÌ¿ÙˆÓ ÚԤ΢„ fiÙÈ Ù· ·È‰È¿ ·fi ÔÈÎÔÁ¤ÓÂȘ ÌÂÙ·Ó·ÛÙÒÓ ·ÓÙÈÚÔÛÒ¢·Ó ÙÔ 12,6% (175/1383), ÂÓÒ Û‡Ìʈӷ Ì ÚfiÛÊ·Ù· ·ÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ (2001), ÔÈ ÌÂÙ·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 ÂÔ̤ӈ˜ ·ÔÎÏ›ÛıËÎ·Ó ·fi ÙË ÌÂϤÙË. ∞fi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ Ì·˜ ÚԤ΢„ fiÙÈ ÙÔ 38% (438/1152) ÙˆÓ ·È‰ÈÒÓ ËÏÈΛ·˜ >24 ÌËÓÒÓ Â›¯Â ÂÌ‚ÔÏÈ·ÛÙ› ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞. ∆Ô ÔÛÔÛÙfi 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 EÏÏËÓfiÔ˘ÏˆÓ (40,5%, 403/995) (OR=0,42, 95%CI: 0,28-0.63, p<0,001) (∂ÈÎfiÓ· 1). ∂ÈϤÔÓ, Ù· ·È‰È¿ Ô˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ·ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜ ÂÌÊ¿ÓÈ˙·Ó ¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ Î¿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· ·ÓÔÛÔÔÈË̤ӷ ·È‰È¿, Ë ÔÚÔıÂÙÈÎfiÙËÙ· ·Ó‹Ïı ÛÙÔ 90,1% (393/436) ÁÈ· ·È‰È¿ Ô˘ ¤Ï·‚·Ó

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·119

119

√ÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞

40

45 40

p<0,001

35

%

30

35

p=0,002

30 40,8

40,5

25

25 20

31

20

15

22,3

10

15 10

5 0

5 ∂ÏÏËÓfiÔ˘Ï·

∞ÛÙÈΤ˜ ÂÚÈÔ¯¤˜ ∞ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜

¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ

∂ÈÎfiÓ· 1. ¶ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›ËÛ˘ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ Û ·È‰È¿ ËÏÈΛ·˜ >24 ÌËÓÒÓ, Û ۯ¤ÛË Ì ÙËÓ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· Î·È ÙÔÓ ÙfiÔ ‰È·ÌÔÓ‹˜.

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 ËÏÈΛ·˜ 0-12 ÌËÓÒÓ Ù˘ ÌÂϤÙ˘, 41 (31,29%) ‹Ù·Ó ÔÚÔıÂÙÈο, ÁÂÁÔÓfi˜ ÂÓ‰ÂÈÎÙÈÎfi ·ıËÙÈ΋˜ ·ÓÔÛ›·˜ ÏfiÁˆ ÌËÙÚÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞. Ÿˆ˜ ‹Ù·Ó ·Ó·ÌÂÓfiÌÂÓÔ, Ù· ·È‰È¿ ËÏÈΛ·˜ <12 ÌËÓÒÓ Ô˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ÔÈÎÔÁ¤ÓÂȘ ÌÂÙ·Ó·ÛÙÒÓ Â›¯·Ó ÛÙ·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÔÚÔıÂÙÈÎfiÙËÙ·˜ (23/41, 56,1%), Û ۇÁÎÚÈÛË Ì ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô ÔÛÔÛÙfi ÙˆÓ ∂ÏÏËÓfiÔ˘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 ˘„ËÏ¿ ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›ËÛ˘ Û ∂ÏÏËÓfiÔ˘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˘ ∂ÏÏËÓfiÔ˘ÏˆÓ ‰È¤ÊÂÚ·Ó ÛËÌ·ÓÙÈο ÌÂٷ͇ ÙˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ, fiˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ ¶›Ó·Î· 1.

∆Ô ÔÛÔÛÙfi ÔÚÔıÂÙÈÎfiÙËÙ·˜ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ Û ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰ÈÒÓ ËÏÈΛ·˜ >12 ÌËÓÒÓ, ÏfiÁˆ Ê˘ÛÈ΋˜ ÓfiÛËÛ˘, ·Ó‹Ïı Û 17,07% (136/797). ∆Ô ÔÛÔÛÙfi ÙˆÓ ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ÔÚÔıÂÙÈÎÒÓ ∂ÏÏËÓfiÔ˘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¿‰· Î·È 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·ÙÈÛÙÈο 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˘ ·ÓÙÈÚÔÛˆ‡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 ÙfiÔ ‰È·ÌÔÓ‹˜ Î·È ÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞, ÌÂٷ͇ ∂ÏÏËÓfiÔ˘ÏˆÓ Î·È ÌÂÙ·Ó·ÛÙÒÓ N (%) ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· µfiÚÂÈ· ∂ÏÏ¿‰· ¶ÂÏÔfiÓÓËÛÔ˜ Î·È ÓËÛÈ¿ ™‡ÓÔÏÔ †

226/418 (54,07%) 79/362 (21,82%) 108/294 (36,73%) 413/1074 (38,45%)

∂ÏÏËÓfiÔ˘Ï· OR (95% CI)

∆ÈÌ‹ p

Ref† 0,24 (0,17-0,32) 0,49 (0,36-0,67)

<0,001 <0,001

N (%)

¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ OR ∆ÈÌ‹ p (95% CI)

26/102 (25,49%) 5/44 (11,36%) 4/32 (12,50%) 35/178 (19,66%)

Ref† 0,37 (0,13-1,05) 0,42 (0,13-1,30)

0,062 0,133

Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜ ¶·È‰È·ÙÚÈ΋ 2009;72:119-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·120

120

µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.

¶›Ó·Î·˜ 2. ¢È·ÊÔÚ¤˜, ·Ó¿ÏÔÁ· Ì ÙÔÓ ÙfiÔ ‰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 ∂ÏÏËÓfiÔ˘ÏˆÓ Î·È ÌÂÙ·Ó·ÛÙÒÓ N (%) ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰·

9/191 (4,71%) 75/278 (26,98%) 20/185 (10,81%) 104/654 (15,90%)

µfiÚÂÈ· ∂ÏÏ¿‰· ¶ÂÏÔfiÓÓËÛÔ˜ Î·È ÓËÛÈ¿ ™‡ÓÔÏÔ †

∂ÏÏËÓfiÔ˘Ï· OR (95% CI)

∆ÈÌ‹ p

Ref† 7,47 (3,64-15,35) 2,45 (1,09-5,53)

<0,001 0,031

N (%)

¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ OR ∆ÈÌ‹ p (95% CI)

14/76 (18,42%) 16/39 (41,03%) 2/28 (7,14%) 32/143 (22,38%)

Ref† 3,08 (1,30-7,30) 0,34 (0,07-1,61)

0,011 0,174

Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜

∂ÏÏËÓfiÔ˘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 Î·Ù¿ÔÛË ÌÔÏ˘ÛÌ¤ÓˆÓ ÙÚÔÊ›ÌˆÓ ‹ ‡‰·ÙÔ˜. ∏ ÌfiÏ˘ÓÛË, fiˆ˜ Â›Û˘ Î·È Ô ÂÌ‚ÔÏÈ·ÛÌfi˜, ·Ú¤¯Ô˘Ó ÈÛfi‚È· ·ÓÔÛ›· (1,9). ∏ Èı·ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ Û˘ÌÙˆÌ¿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˘Ìو̷ÙÈΤ˜ ‹ ·ÓÈÎÙÂÚÈΤ˜ (1,2). ∆· ·È‰È¿ Ì ·Û˘Ìو̷ÙÈ΋ ÌfiÏ˘ÓÛË ÌÔÚÔ‡Ó Ó· ·ÔÙÂϤÛÔ˘Ó ËÁ‹ ÌfiÏ˘ÓÛ˘ ÁÈ· Ù· ËÏÈÎȈ̤ӷ ¿ÙÔÌ· (3). ∆Ș ÙÂÏÂ˘Ù·›Â˜ ‰ÂηÂٛ˜, ÙÔ ÔÛÔÛÙfi ÙˆÓ ·ÙfiÌˆÓ 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˜ ÌÔχÓÛÂȘ ·fi ÙÔÓ Èfi ·Ó·Ì¤ÓÂÙ·È Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ·ÁÎÔÛÌ›ˆ˜ οı ¯ÚfiÓÔ (8). ¶·Ú¿ ÙË Ì›ˆÛË Ô˘ ηٷÁÚ¿ÊÂÙ·È ÛÙ· ÔÚÔÂȉËÌÈÔÏÔÁÈο ÔÛÔÛÙ¿, Ë Ë·Ù›Ùȉ· ∞ Û˘Ó¯›˙ÂÈ Ó· Â›Ó·È Ì›· ·fi ÙȘ Ôχ Û˘¯Ó¿ ·Ó·ÊÂÚfiÌÂÓ˜ ·Ûı¤ÓÂȘ Ô˘ ÌÔÚÔ‡Ó Ó· ÚÔÏËÊıÔ‡Ó Ì ÂÌ‚ÔÏÈ·ÛÌfi ·ÁÎÔÛÌ›ˆ˜ (8).

∏ ∂ÏÏ¿‰· ıˆÚÂ›Ù·È ¯ÒÚ· Ì ÂӉȿÌÂÛË ÂÓ‰ËÌÈÎfiÙËÙ·, ·ÚfiÏÔ Ô˘ ‰ÂÓ ¤¯Ô˘Ì ÚfiÛÊ·Ù· ‰Â‰Ô̤ӷ. ∫¿ÔȘ ÌÈÎÚ¤˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó Ì›ˆÛË ÙÔ˘ ÂÈÔÏ·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 ·ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ (5-7). ™Â ÌÈ· ·Ó·‰ÚÔÌÈ΋ ÌÂϤÙË Ô˘ ‰ÈÂÓÂÚÁ‹ıËΠÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ∂ȉÈÎÒÓ ¶·ı‹ÛÂˆÓ £ÂÛÛ·ÏÔӛ΢, ÌÂٷ͇ ÙˆÓ ÂÙÒÓ 19851992 ηٷÁÚ¿ÊËΠÛÙ·ıÂÚ‹ Ì›ˆÛË ÙˆÓ ÂÈÛ·ÁˆÁÒÓ (6). ∂ÈϤÔÓ, ÛËÌÂÈÒıËΠ·‡ÍËÛË Ù˘ ̤Û˘ ËÏÈΛ·˜ ÙˆÓ ·ÛıÂÓÒÓ, ·fi 0-5 ¤ÙË ÙÔ 1985 Û 21-25 ¤ÙË ÙÔ 1992 (6). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÚÔËÁÔ‡ÌÂÓ˘ ‰ÂηÂÙ›·˜, ˘‹ÚÍ ÌÂÁ¿ÏË ÏËı˘ÛÌȷ΋ ·ÏÏ·Á‹ ÛÙËÓ ∂ÏÏ¿‰·, ÏfiÁˆ Ù˘ ÂÈÛfi‰Ô˘ ÛÙË ¯ÒÚ· ÔÈÎÔÓÔÌÈÎÒÓ ÚÔÛʇÁˆÓ ·fi ÙËÓ ∞Ó·ÙÔÏÈ΋ ∂˘ÚÒË Î·È Ù· µ·ÏοÓÈ·. ™‡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 ·ÓÙÈÛˆÌ¿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 N (%) ∂ÏÏËÓfiÔ˘Ï· ªÂÙ·Ó¿ÛÙ˜ ™‡ÓÔÏÔ †

9/191 Ref† (4,71%) 14/76 4,57 0,001 (18,42%) (1,88-11,07) 23/267 (8,61%)

Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜

Paediatriki 2009;72:120-000

∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· OR p (95% CI)

N (%)

µfiÚÂÈ· ∂ÏÏ¿‰· OR (95% CI)

75/278 Ref† (26,98%) 16/39 1,88 (41,03%) (0,94-3,76) 91/317 (28,71%)

p

0,073

¶ÂÏÔfiÓÓËÛÔ˜ Î·È ÓËÛÈ¿ N (%) OR p (95% CI) 20/185 (10,81%) 2/28 (7,14%) 22/213 (10,33%)

Ref† 0,63 (0,14-2,88)

0,555

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™ÂÏ›‰·121

121

√ÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞

·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 ÂÚÈÙÒÛÂˆÓ Ô˘ ‰ËÏÒÓÔÓÙ·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‚ÔÏÈ·ÛÌfi˜ Û˘ÛÙ‹ıËΠÁÈ· ÙȘ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÔÌ¿‰Â˜ ÌfiÓÔ, ¤¯Ô˘Ó Á›ÓÂÈ ÚÔÁÚ¿ÌÌ·Ù· Ì·˙ÈÎÔ‡ ÂÌ‚ÔÏÈ·ÛÌÔ‡ ÛÙȘ ÂÚÈÔ¯¤˜ Ì ÂӉȿÌÂÛË ÂÓ‰ËÌÈÎfiÙËÙ· ·fi ÙÔ 1997, fiˆ˜ 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‡ Â›ÙˆÛ˘ Ù˘ Ë·Ù›Ùȉ·˜ ∞, 5 ¯ÚfiÓÈ· ÌÂÙ¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ˘Ô¯ÚˆÙÈÎÔ‡ ÂÌ‚ÔÏÈ·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 ∏¶∞ fiÔ˘ 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·˜ ÌÂȈ̤ÓË 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‚ÔÏÈ·ÛÌÒÓ. ∂Ô̤ӈ˜, Ë ·ÁÔÚ¿ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ÂÈ‚·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‰Ô̤ӷ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë. ∞fi Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜ ÚԤ΢„Â, fiˆ˜ ¿ÏψÛÙ ·Ó·ÌÂÓfiÙ·Ó, fiÙÈ Ù· ·È‰È¿ ÂÏÏËÓÈ΋˜ ηٷÁˆÁ‹˜, ηıÒ˜ Î·È ÂΛӷ ·ÛÙÈÎÒÓ ÂÚÈÔ¯ÒÓ, ›¯·Ó ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ Î¿Ï˘„˘, Û ۇÁÎÚÈÛË 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‰ËÏÒÓÂÈ ·ÓÔÛ›· ÌÂÙ¿ ·fi Ê˘ÛÈ΋ Ïԛ̈ÍË. ™‡Ìʈӷ Ì ٷ ·ÔÙÂϤÛÌ·Ù¿ Ì·˜, 17% ÙˆÓ ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ·È‰ÈÒÓ Â›¯·Ó ÓÔÛ‹ÛÂÈ ·fi Ë·Ù›Ùȉ· ∞. ∆Ô ÔÛÔÛÙfi ·˘Ùfi Â›Ó·È ÂÍ·ÈÚÂÙÈο ·˘ÍË̤ÓÔ Î·È ‰ÂÓ ÌÔÚ› Ó· ·Ô‰Ôı› ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜. ™ËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÙfiÛÔ ÛÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë fiÛÔ Î·È ÛÙË Ê˘ÛÈ΋ ·ÓÔÛ›· ‚Ú¤ıËΠÌÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ Ù˘ ¯ÒÚ·˜ (¶›Ó·Î·˜ 3). ™˘ÁÎÂÎÚÈ̤ӷ, ÛÙËÓ ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· ηٷÁÚ¿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· ∂ÏÏËÓfiÔ˘Ï·, ÏfiÁˆ Ù˘ ¯·ÌËÏ‹˜ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ Î¿Ï˘„˘ ÙˆÓ ·È‰ÈÒÓ ÌÂÙ·Ó·ÛÙÒÓ. ∞Í›˙ÂÈ ÂÈϤÔÓ Ó· ÙÔÓÈÛÙ› fiÙÈ, ÂÓÒ ÛÙÔ Û˘ÓÔÏÈÎfi ‰Â›ÁÌ· Ë ‰È·ÊÔÚ¿ ÔÚÔıÂÙÈÎfiÙËÙ·˜ ÌÂٷ͇ ∂ÏÏËÓfiÔ˘ÏˆÓ Î·È ·È‰ÈÒÓ ÌÂÙ·Ó·ÛÙÒÓ ‰ÂÓ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋, ÛÙ· ·È‰È¿ Ô˘ ‰È·‚ÈÔ‡Ó ÛÙËÓ ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· (fiÔ˘ ηٷÁÚ¿ÊËÎÂ Ë ˘„ËÏfiÙÂÚË ÂÌ‚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¿ÏÔÁ· 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·ÛÔÚ¿ 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 Ì ÂΛӷ ÌÈ·˜ ÚfiÛÊ·Ù˘ ÔÚÔÂȉËÌÈÔÏÔÁÈ΋˜ ÌÂϤÙ˘ Ô˘ ·ÊÔÚÔ‡Û ÙËÓ Ë·Ù›Ùȉ· µ, ÁÈ· ÙËÓ ÔÔ›· ÂÊ·ÚÌfi˙ÂÙ·È Ì·˙ÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ ÙˆÓ ‚ÚÂÊÒÓ ·fi ÙÔ 1998 (15). ™ÙË Û˘ÁÎÂÎÚÈ̤ÓË ÌÂϤÙË, ‰ÂÓ ‚Ú¤ıËΠÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÌÂٷ͇ ÙˆÓ ‰È¿ÊÔÚˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ, ÏfiÁˆ Ù˘ ÂÓÈ·›·˜ ÔÏÈÙÈ΋˜ ˘Á›·˜ Ô˘ ÂÊ·ÚÌfi˙ÂÙ·È Û fiÏË ÙË ¯ÒÚ· (15). ∂Ô̤ӈ˜, ·fi Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜, ÛÂ Û˘Ó‰˘·ÛÌfi Ì 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· ∞, fiˆ˜ Û˘Ó¤‚Ë Î·È Û ¿ÏϘ ¯ÒÚ˜, Î·È Ì¿ÏÈÛÙ· ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 12 ÌËÓÒÓ (11-14). ª¿ÏÈÛÙ·, Ì ‚¿ÛË Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜, Ë ∂ıÓÈ΋ ∂ÈÙÚÔ‹ ∂Ì‚ÔÏÈ·ÛÌÒÓ ÂÈÛËÁ‹ıËΠÙËÓ ¤ÓÙ·ÍË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ Ù˘ Ë·Ù›Ùȉ·˜ ∞ ÛÙÔ˘˜ ˘Ô¯ÚˆÙÈÎÔ‡˜ ÂÌ‚ÔÏÈ·ÛÌÔ‡˜, Ì ·ÔÙ¤ÏÂÛÌ· 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 Committee on Infectious Diseases. 26th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2003:309-318.

2. CDC: Prevention of Hepatitis A through Active or Passive Immunization: Recommendations of the advisory Committee on Immunization Practices (ACIP). MMWR 1999,48(No. RR-12).

3. Staes C, Schlenker T, Risk I, et al. Source of infection among persons with acute hepatitis A and no identificatied risk 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 defined area in rural Krete, Greece. J Viral Hepat 1997;4:55-61.

5. Kremastinou J, Kalapothaki V, Trichopoulos D. The changing epidemiologic pattern of hepatitis A infection in urban Greece. Am J Epidemiol 1984;120:703-706. ¶·È‰È·ÙÚÈ΋ 2009;72:121-000

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10-04-09

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µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.

6. Papadopoulou V, Stavrati A, Patsialis T, et al. Changes in the Epidemiology of acute hepatitis A during 1985-1992 period. Pediatr N Gr 1995;7:30-34.

7. Dalekos GN, Zervou E, Karabini F, Tsianos EV. Prevalence of viral markers among refugees from southern Albania: increased incidence of infection with hepatitis A, B and D viruses. Eur J Gastrenterol Hepatol 1995;7:553-558.

8. Jacobsen KH, Koopman JS. Declining hepatitis A seroprevalance: a global review and analysis Epidemiol Infect 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 against hepatitis A in seven European countries I: comparison of prevalence data in different age groups. Am J Epidemiol 1979;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 A Vaccination Programs. JAMA 2005;294:246-248.

13. Dagan R, Leventha A, Anis E, Slatter P, Ashur Y, Shouval D. Incidence of Hepatitis A in Israel Following Universal Immunization of Toddlers. JAMA 2005;294:202-210.

14. Wasley A, Samandari T, Bell BP. Incidence of Hepatitis A in the United States in the era of vaccination. JAMA 2005;294:194-201.

15. Papaevangelou V, Hadjichristodoulou C, Cassimos D, Pantelaki K, Tzivaras A, Hatzimichael et al. Seroepidemiology of hepatitis B in Greek children six years after the implementation of universal vaccination. Infection 2008;36:135-139

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123

§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿: ÂÌÂÈÚ›· 28 ÂÙÒÓ ∞. ¶Ô˘ÚÙÛ›‰Ë˜1, ¢. ¢ÔÁ¿Ó˘1, ª. ª¿Î·1, ¢. ªÔ˘¯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‡ ¯ËÌÂÈÔıÂÚ·¢ÙÈÎÒÓ ÚˆÙÔÎfiÏψÓ, Ì ‹ ¯ˆÚ›˜ ·ÎÙÈÓÔıÂÚ·›·, 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 ÓfiÛÔ Hodgkin. ™Ù· ·È‰È¿ ·˘Ù¿ ÌÂÏÂÙ‹ıËÎ·Ó ÂȉËÌÈÔÏÔÁÈο, ÎÏÈÓÈο Î·È ÈÛÙÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο, Ô ÙÚfiÔ˜ ıÂÚ·›·˜ Î·È Ë ¤Î‚·ÛË Î·È ·ÍÈÔÏÔÁ‹ıËÎ·Ó ‰˘ÓËÙÈÎÔ› ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ ÂȉÚÔ‡Ó ÛÙËÓ ÚfiÁÓˆÛË.

AÔÙÂϤÛÌ·Ù·: ∏ ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ (46 ·ÁfiÚÈ·, 27 ÎÔÚ›ÙÛÈ·) Î˘Ì·ÈÓfiÙ·Ó ·fi 3,5 ¤ˆ˜ 15 ¤ÙË. ™ÙË Û˘ÓÙÚÈÙÈ΋ ÏÂÈÔ„ËÊ›·, Ë ·ÈÙ›· Ù˘ ·Ú¯È΋˜ ÚÔÛ¤Ï¢Û˘ ‹Ù·Ó Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· (81%), ÂÓÒ Ë ‰È¿ÚÎÂÈ· ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ÚÈÓ ÙË ‰È¿ÁÓˆÛË Î˘Ì·ÈÓfiÙ·Ó ·fi Ï›Á˜ ̤Ú˜ ¤ˆ˜ Î·È 12 Ì‹Ó˜. ªÂٷ͇ ÙˆÓ ÈÛÙÔÏÔÁÈÎÒÓ ˘fiÙ˘ˆÓ ˘ÂÚ›¯Â Ë Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË (63%), ÂÓÒ 44% ÙˆÓ ·ÛıÂÓÒÓ Â›¯·Ó ÚÔ¯ˆÚË̤ÓË ÓfiÛÔ (ÛÙ·‰›Ô˘ πππ-IV). ∏ ıÂÚ·›· ÂÚÈÂÏ¿Ì‚·Ó ÌfiÓÔ ¯ËÌÂÈÔıÂÚ·›· Û 5 ·ÛıÂÓ›˜, ÌfiÓÔ ·ÎÙÈÓÔıÂÚ·›· Û 9 Î·È Û˘Ó‰˘·ÛÌfi Û 59. ∏ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ·Ó¤Ú¯ÂÙ·È Û 97,3%. OÎÙÒ ·È‰È¿ ˘ÔÙÚÔ›·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· ÔÔ›· ˙Ô˘Ó Ù· 3. ¢ÂÓ ‰È·ÈÛÙÒıËÎ·Ó ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ Ó· Û˘Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ. ™˘ÌÂÚ¿ÛÌ·Ù·: ∏ ¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ì ÓfiÛÔ Hodgkin Â›Ó·È ÂÍ·ÈÚÂÙÈ΋ ÌÂ Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂÚ·›·˜ Î·È ·ÎÙÈÓÔıÂÚ·›·˜. ∏ Û˘Ó¯È˙fiÌÂÓË ·Ú·ÎÔÏÔ‡ıËÛË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·È‰ÈÒÓ ÎÚ›ÓÂÙ·È ··Ú·›ÙËÙË ÁÈ· Ó· ‰È¢ÎÚÈÓÈÛÙ› ·Ó ˘¿Ú¯Ô˘Ó ‰˘ÓËÙÈο ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ù˘ ¤Î‚·Û˘ ÙˆÓ ·È‰ÈÒÓ Ì ÓfiÛÔ Hodgkin.

1 √ÁÎÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó· 2 ∞ÎÙÈÓÔıÂÚ·¢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˜ pedonca@aglaiakyriakou.gr √ÁÎÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

§¤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 without radiotherapy, 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 centre over 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 with lymphadenopathy, with a time interval of 3 days to 12 months between the first appearance of clinical signs and the diagnosis. Nodular sclerosis was the predominant histological subtype (63%), and 44% of patients had advanced disease (stage III or IV). Chemotherapy alone was used in 5 patients, radiation in 9 and combined treatment in 59. Relapses occurred in 8 children and 4 developed second or third malignancies. The overall survival was 97.3%. One patient died from the disease and one after a third malignancy. No specific factors related to the outcome were detected.

Conclusions: The outcome of children with Hodgkin’s disease is excellent with chemotherapy combined with low dose radiation. A longer follow-up of a greater number of patients is necessary to detect prognostic factors related to the outcome of children and adolescents with Hodgkin’s disease.

1 Department of Oncology, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece 2 Department of Radiotherapy, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece 3 Laboratory of Pathology, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece Correspondence: Apostolos Pourtsidis pedonca@aglaiakyriakou.gr Department of Oncology, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece

Key words: Hodgkin’s lymphoma, children, prognostic factors.

¶·È‰È·ÙÚÈ΋ 2009;72:123-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·124

124

∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.

∂ÈÛ·ÁˆÁ‹ ∆Ô Ï¤Ìʈ̷ Hodgkin ·ÔÙÂÏ› ÙÔ 4-5% ÙˆÓ Î·ÎÔËıÂÈÒÓ ÛÙ· ·È‰È¿ Î·È ÙÔ ¤ÎÙÔ Î·Ù¿ ÛÂÈÚ¿ Û˘¯ÓfiÙËÙ·˜ ÓÂÔÏ·ÛÌ·ÙÈÎfi ÓfiÛËÌ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ÛÙËÓ ∂˘ÚÒË Î·È ÛÙȘ ∏.¶.∞., ÌÂÙ¿ ÙË Ï¢¯·ÈÌ›·, ÙÔ˘˜ fiÁÎÔ˘˜ ÂÁÎÂÊ¿ÏÔ˘, ÙÔ Ó¢ÚÔ‚Ï¿Ûو̷, ÙÔ ÓÂÊÚÔ‚Ï¿Ûو̷ Î·È ÙÔ ÌË Hodgkin ϤÌʈ̷. ∏ Â›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· Â›ÙˆÛË ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin Û ·È‰È¿ ËÏÈΛ·˜ οو ÙˆÓ 15 ¯ÚfiÓˆÓ ˘ÔÏÔÁ›˙ÂÙ·È Û 5,5 ÂÚÈÙÒÛÂȘ ·Ó¿ ÂηÙÔÌ̇ÚÈÔ ÛÙÔÓ ·ÓÂÙ˘Á̤ÓÔ ÎfiÛÌÔ, ·ÏÏ¿ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ ËÏÈΛ·˜ 15-20 ¯ÚfiÓˆÓ Êı¿ÓÂÈ ÙȘ 12,1. ∞ÓÙÈı¤Ùˆ˜, ÛÙË ¢˘ÙÈ΋ ∞Û›· Ë Â›ÙˆÛË ÛÙ· ·È‰È¿ Â›Ó·È ÛÙ·ıÂÚ¿ ¿Óˆ ·fi ÙȘ 7 ÂÚÈÙÒÛÂȘ ·Ó¿ ÂηÙÔÌ̇ÚÈÔ (1). ™ÙËÓ ∂ÏÏ¿‰·, Ë Â›ÙˆÛË ÛÙ· ·È‰È¿ ·Ó¤Ú¯ÂÙ·È Û 7,2 ·Ó¿ ÂηÙÔÌ̇ÚÈÔ ¿ÓıÚˆÔ-¤ÙË. ÀÔÏÔÁ›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˘ÁÎÂÓÙÚÒÓÂÈ 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 ÂÓËÏ›ÎˆÓ Î·È ·˘Ùfi˜ ÙˆÓ ÌÂÁ·Ï‡ÙÂÚˆÓ ÂÓËÏ›ÎˆÓ (·ÛıÂÓ›˜ ¿Óˆ ÙˆÓ 55 ÂÙÒÓ). ∏ ÓfiÛÔ˜ ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÈ· ‰ÈÎfiÚ˘ÊË ËÏÈÎȷ΋ ηٷÓÔÌ‹, Ì ̛· ·È¯Ì‹ ÛÙËÓ ÂÊ˂›· 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 ¯ËÌÂÈÔıÂÚ·¢ÙÈÎÒÓ Û¯ËÌ¿ÙˆÓ, Ì ‹ ¯ˆÚ›˜ ·ÎÙÈÓÔıÂÚ·›·, Ô‰‹ÁËÛ Û ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÚfiÁÓˆÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì ϤÌʈ̷ Hodgkin, ·‡ÍËÛË ÙˆÓ ÔÛÔÛÙÒÓ ›·Û˘ Î·È Û˘Á¯ÚfiÓˆ˜ Ì›ˆÛË Ù˘ ·ÒÙÂÚ˘ ÙÔÍÈÎfiÙËÙ·˜. H ÂÓÙ·ÂÙ‹˜ ÂχıÂÚË Û˘Ì‚¿Ì·ÙÔ˜ ÂÈ‚›ˆÛË (EFS) ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ· ˘ÂÚ‚·›ÓÂÈ ÙÔ 90% ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡ Î·È ÂӉȿÌÂÛÔ˘ ÎÈÓ‰‡ÓÔ˘ Ì ÂÓÙÔÈṲ̂ÓË 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 ÂȉËÌÈÔÏÔÁÈο, ÂÚÁ·ÛÙËÚȷο, ÈÛÙÔÏÔÁÈο Î·È ·ÂÈÎÔÓÈÛÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο, ηıÒ˜ Â›Û˘ ÙÔ Â›‰Ô˜ ıÂÚ·›·˜ 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›˙ÔÓÙ·È Ì ÙËÓ ÙÂÏÈ΋ ¤Î‚·ÛË Î·È ÙȘ ·ÒÙÂÚ˜ Û˘Ó¤ÂȘ Ù˘ ıÂÚ·›·˜ ÙˆÓ ·È‰ÈÒÓ Ì ϤÌʈ̷ Hodgkin.

Paediatriki 2009;72:124-000

¶›Ó·Î·˜ 1. £ÂÚ·›· ÙˆÓ ·ÛıÂÓÒÓ Ì ÓfiÛÔ Hodgkin 1978-1988

A£ ª√¡√ (9*) ã ª√¡√ (5)

X£ & ∞£ (5) 1989-1993

ã & ∞£ (26)

1994-2005

X£ & ∞£ (28)

ª√PP:2* ABVD:1 ∞§§√:2 ª√PP:2 ABVD:3 ª√PP & ABVD: 15 ABVD: 10 A§§√: 1 ª√PP & ABVP:8 VBVP:11 √PPA & COPP: 8 ABVP/COP: 1

* ·ÚÈıÌfi˜ ·ÛıÂÓÒÓ MOPP (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)

∞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 √ÁÎÔÏÔÁÈÎfi ÙÌ‹Ì· ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡» ·fi ÙÔ 1978 ¤ˆ˜ ÙÔ 2005. ∏ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ·ÛıÂÓÒÓ ¤ÁÈÓ 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· ¯ËÌÂÈÔıÂÚ·›·˜ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1. ∏ ıÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË ‰È¤ÊÂÚ ·Ó¿ÏÔÁ· Ì ÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô. ¶ÂÚ›Ô‰Ô˜ ∞ (1979-1988, 19 ·ÛıÂÓ›˜): ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó Ì ·ÎÙÈÓÔıÂÚ·›· ÌfiÓÔ, ¯ËÌÂÈÔıÂÚ·›· ÌfiÓÔ (MOPP ‹ ABVD) ‹ Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂÚ·›·˜ (ªOPP ‹ ABVD) Î·È ·ÎÙÈÓÔıÂÚ·›·˜ 30-40 Gy. ¶ÂÚ›Ô‰Ô˜ µ (19891993, 26 ·ÛıÂÓ›˜): ÔÈ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ π ‹ ππ∞ ¤Ï·‚·Ó 4 ·ÎÏÔ˘˜ ∞µVD Î·È ·ÎÔÏÔ‡ıˆ˜ ·ÎÙÈÓÔıÂÚ·›· 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· ·ÎÙÈÓÔıÂÚ·›· 20 Gy ÛÙȘ ·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜, ÙÔ ÛÏ‹Ó· Î·È ÙÔ˘˜ ·Ú·ÔÚÙÈÎÔ‡˜ ÏÂÌÊ·‰¤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˜ ÂÚÈÔ¯¤˜. √È ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ πππ ¤Ï·‚·Ó ·fi 2 ·ÎÏÔ˘˜ MOPP Î·È ABVP ÂÓ·ÏÏ¿Í Î·È ·ÎÙÈÓÔıÂÚ·›· Ì 20 Gy ÛÙȘ ·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜, ÙÔ ÛÏ‹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· ·ÎÙÈÓÔıÂÚ·›· Ì 20 Gy ÛÙÔ˘˜ ·Ú¯Èο ÚÔۂ‚ÏË̤ÓÔ˘˜ ÏÂÌÊ·‰¤Ó˜ Î·È Ù· ·Ú¯Èο ÚÔۂ‚ÏË̤ӷ fiÚÁ·Ó·. ∏ ‰fiÛË Ù˘ ·ÎÙÈÓÔıÂÚ·›·˜ ‹Ù·Ó Û fiϘ ÙȘ ÂÚÈÙÒÛÂȘ 20 Gy, ÂÊfiÛÔÓ Ë ·ÂÈÎÔÓÈÛÙÈ΋ ·¿ÓÙËÛË ˘ÂÚ¤‚·ÈÓ ÙÔ 70%. ∂ȉÈο ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ IV, ÁÈ· ÙÔ ‹·Ú ¯ÔÚËÁ‹ıËÎ·Ó 12

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·125

125

§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿

20

∫ÔÚ›ÙÛÈ· ∞ÁfiÚÈ·

%

6

10

2

10

7

5 8 6

3 3 4 3

0

3

1 4

5

6

7

1 1

3

3

2

2

8 9 10 11 12 13 14 15 ∏ÏÈΛ· (¤ÙË)

∂ÈÎfiÓ· 1. ¢ËÌÔÁÚ·ÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο.

Gy, ÁÈ· Ù· ÔÛÙ¿ 20-30 Gy, ÂÓÒ ÁÈ· ÙÔ˘˜ Ó‡ÌÔÓ˜ ¯ÔÚËÁ‹ıËÎ·Ó 12 Gy ÌfiÓÔ ·Ó ‰ÂÓ ˘‹Ú¯Â Ï‹Ú˘ ‡ÊÂÛË ÌÂÙ¿ ·fi ‰‡Ô Û¯‹Ì·Ù· √PPA. ÃÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Ì¤ıÔ‰Ô˜ Kaplan-Meier ÁÈ· ÙÔÓ Î·ıÔÚÈÛÌfi Ù˘ Û˘ÓÔÏÈ΋˜ ÂÈ‚›ˆÛ˘ Î·È Ù˘ ¯ˆÚ›˜ ÓfiÛÔ ‹ ۇ̂·Ì· ÂÈ‚›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 ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÔÈ ÂÍ‹˜ ÛÙ·ÙÈÛÙÈΤ˜ ‰ÔÎÈ̷ۛ˜: (1) ÌË ·Ú·ÌÂÙÚÈ΋ ‰ÔÎÈÌ·Û›· MannWitney, (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˘ÌÙÒÌ·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˘Ìو̷ÙÔÏÔÁ›·˜ ‹ ÛËÌÂÈÔÏÔÁ›·˜ (Û ‰‡Ô ·È‰È¿ Ë ‰È¿ÁÓˆÛË ÂÙ¤ıË ÛÙ· Ï·›ÛÈ· ‰ÈÂÚ‡ÓËÛ˘ ÓÂÊÚˆÛÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘, Ô˘ ·ÔÙÂÏ› ·Ú·ÓÂÔÏ·ÛÌ·ÙÈ΋ ÂΉ‹ÏˆÛË Ù˘ ÓfiÛÔ˘ Hodgkin). ™Â 50 ·È‰È¿ (68,5%) ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó Û˘ÓÔ‰¿ Û˘ÌÙÒÌ·Ù·, ÂÓÒ ÛÙ· ˘fiÏÔÈ·, ÂÎÙfi˜ ÙÔ˘ ·ÚÈÔ˘ Û˘ÌÙÒÌ·ÙÔ˜/ÛËÌ›Ԣ, Û˘Ó˘‹Ú¯·Ó ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, ‚‹¯·˜-Ï¢ÚÔ‰˘Ó›·, ÂÌ‡ÚÂÙÔ, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· ‹ Û˘Ó‰˘·ÛÌfi˜ ÙˆÓ ·ÓˆÙ¤Úˆ. ™˘ÌÙÒÌ·Ù· Ù‡Ô˘ µ ‰È·ÈÛÙÒıËÎ·Ó Û 21 ·È‰È¿ (28,8%) Î·È ÂȉÈÎfiÙÂÚ· ·Ú·ÙÂÈÓfiÌÂÓÔ ˘Ú¤ÙÈÔ (¿Óˆ ÙˆÓ 10 ËÌÂÚÒÓ) ‰È·ÈÛÙÒıËΠ۠10, ·ÒÏÂÈ· ‚¿ÚÔ˘˜ >10% Û 10, ÂÊȉÚÒÛÂȘ Û 7 Î·È ÎÓËÛÌfi˜ Û ¿ÏÏ· 2. §ÂÌÊ·‰ÂÓÔ¿ıÂÈ· ηٿ ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠ۠68 ·ÛıÂÓ›˜ (93%). ™Â 12 ÂÍ ·˘ÙÒÓ ‹Ù·Ó ·ÌÊÔÙÂÚfiÏ¢ÚË Î·È Û 56 ÂÙÂÚfiÏ¢ÚË. ∂ÓÙfiÈÛË ÛÙÔÓ ÙÚ¿¯ËÏÔ ‰È·ÈÛÙÒıËΠ۠55 ·È‰È¿, ÛÙÔ˘˜ ˘ÂÚÎÏ›‰ÈÔ˘˜ ‚fiıÚÔ˘˜ Û 41, ÛÙȘ Ì·Û¯·ÏÈ·›Â˜ ÎÔÈÏfiÙËÙ˜ Û 13 Î·È ÛÙË ‚Ô˘‚ˆÓÈ΋ ¯ÒÚ· Û 4. ∫·Ù¿ ÙË Ê˘ÛÈ΋ ÂͤٷÛË, Ë·ÙÔÌÂÁ·Ï›· ‰È·ÈÛÙÒıËΠ۠2 ·È‰È¿ Î·È ÛÏËÓÔÌÂÁ·Ï›· Û 5. ∆· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹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·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘. ∆Ô ˘ÂÚ˯ÔÁÚ¿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˘

∏ÏÈΛ· (¤ÙË) WBC (x 103/mm3) ¶ÔÏ˘ (%) ∏ˆÛ (%) Hb (g/dl) ∆∫∂ (mm 1˘ ÒÚ·˜) PLT (x 103/mm3) CRP (mg/l) LDH (mg/dl)

º‡ÏÔ

¢∆*

ŸÚÈ·

∞ÁfiÚÈ· ¢∆*

12 10,2 66 3 11,1 61 383 72 380

3,5-12 4,2-25 39-91 0-11 7,6-15 5-150 200-820 0-173 206-1300

10,3 9,3 64,3 3 11,1 52 367 51,8 381

19

19

¶›Ó·Î·˜ 2. ∂ÚÁ·ÛÙËÚȷΤ˜ ·Ú¿ÌÂÙÚÔÈ ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÌÂϤÙ˘ ηٿ ÙË ‰È¿ÁÓˆÛË ™‡ÓÔÏÔ

¶›

19

∫ÔÚ›ÙÛÈ· ¢∆*

ƒ

13 11,25 74 2,5 11,1 82 387 81 335

0,001 0,183 0,044 0,768 0,35 0,123 0,614 0,577 0,672

*¢∆: ‰È¿ÌÂÛË ÙÈÌ‹ ¶·È‰È·ÙÚÈ΋ 2009;72:125-000

*· M AB VB OP CO

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·126

126

∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.

¶›Ó·Î·˜ 3. πÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜ Î·È ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ ™‡ÓÔÏÔ ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ §ÂÌÊÔÂÓÈÎfi˜ §ÂÌÊÔÂÈÎÚ·ÙÒÓ √˙҉˘ ÛÎÏ‹Ú˘ÓÛË ™‡ÓÔÏÔ

™Ù¿‰ÈÔ

19* (26)** 2 (2,7) 6 (8,2) 46 (63) 73 (100)

π

ππ

πππ

IV

6 (37,5) 4 (25) 6 (37,5) 16 (22)

4 (16) 1 (4) 20 (80) 25 (34)

7 (35) 1 (5) 12 (60) 20 (27,5)

2 (16,7) 2 (16,7) 8 (66,6) 12 (16,5)

*·ÚÈıÌfi˜ ·ÛıÂÓÒÓ, **(%) ·Ó·ÏÔÁ›· ·ÛıÂÓÒÓ

‰È·ÈÛÙÒıËΠÛÂ Û˘¯ÓfiÙËÙ· 37% (7/19) Î·È 78% (36/46) ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÈÛÙÔÏÔÁÈÎfi ˘fiÙ˘Ô ÌÈÎÙ‹˜ ΢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‡ڢÓÛË 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¿, ÛÏËÓÔÌÂÁ·Ï›· Û 13, Ô˙Ò‰ÂȘ ‰ÈËı‹ÛÂȘ ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ÏÂÌÊÒÌ·ÙÔ˜ ∏odgkin ‰È·ÈÛÙÒıËÎ·Ó ÛÙÔ ‹·Ú Û 3 ·È‰È¿, ÛÙÔ ÛÏ‹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˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·˜. ∆¤ÏÔ˜, ÏÂÌÊ·ÁÁÂÈÔÁÚ·Ê›· (ÎÏ·ÛÈ΋ ‹ Ú·‰ÈÔ˚ÛÔÙÔÈ΋) Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠17 ·È‰È¿ Î·È ‹Ù·Ó ·ıÔÏÔÁÈ΋ Û 6. ™Ù·‰ÈÔÔ›ËÛË ªÂ ‚¿ÛË ÎÏÈÓÈο Î·È ·ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù·, 16 (22%) ·È‰È¿ Ù·ÍÈÓÔÌ‹ıËÎ·Ó ˆ˜ 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·‰ÈÔÔ›ËÛË Ì ÛÏËÓÂÎÙÔÌ‹ ˘Ô‚Ï‹ıËÎ·Ó 3 ·È‰È¿ ÛÙ· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ÌÂϤÙ˘. ¢È‹ıËÛË ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ‰È·ÈÛÙÒıËΠ̠ÔÛÙÂÔÌ˘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·ÈÛÙÒıËΠ̠ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÔÛÙÒÓ Û 4/73 ·È‰È¿ Î·È ·ÂÈÎÔÓÈÛÙÈο ÔÚ·Ù¤˜ Ó¢ÌÔÓÈΤ˜ ‰ÈËı‹ÛÂȘ Û 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 ÏÂÌÊÔÂÈÎÚ·ÙÒÓ Û ·ÛıÂÓ›˜ ÛÙ·‰›Ô˘ π, ÂÓÒ Ù· ‰‡Ô ·È‰È¿ Ì ÏÂÌÊÔÂÓÈÎfi ˘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 ÂȉËÌÈÔÏÔÁÈο Î·È ÂÚÁ·ÛÙËÚȷο ‰Â‰Ô̤ӷ ÂÚÈÁÚ¿ÊÂÙ·È ÛÙÔÓ ¶›Ó·Î· 4.

¶›Ó·Î·˜ 4. ™˘Û¯¤ÙÈÛË ÂȉËÌÈÔÏÔÁÈÎÒÓ Î·È ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ·Ú·Ì¤ÙÚˆÓ Ì ÙÔ ÛÙ¿‰ÈÔ Î·È ÙËÓ ÈÛÙÔÏÔÁ›·

º‡ÏÔ (·Á:ÎÔ)* ∏ÏÈΛ· (¤ÙË)** WBC (x 103/mm3)** ¶ÔÏ˘ (%)** ∏ˆÛ (%)** Hb (g/dl)** PLT (x 103/mm3)** ∆∫∂ (mm 1˘ ÒÚ·˜)** CRP (mg/l)** LDH (mg/dl)**

π

ππ

™Ù¿‰ÈÔ πππ

πV

P

ª

§¶

13:3 9,3 8,75 55 3 12,9 200 16,5 20 498

13:12 12,1 12,19 74 2 11,1 400 70 57 330

12:8 12,1 9 62 3 11,1 403 75 80 380

8:4 13 10,4 77 2,4 10,9 383 115 110 404

0,393 0,102 0,027 0,002 0,173 0,014 0,022 0,001 0,398 0,44

18:1 10 8,4 63 2,8 11,1 367 59 57 323

2:0 11,6 4,7 60,5 2,5 10,6 384 65 -

πÛÙÔÏÔÁ›·*** §∂ √™

* ·Á: ·ÁfiÚÈ·, ÎÔ: ÎÔÚ›ÙÛÈ· ** ‰È¿ÌÂÛ˜ ÙÈ̤˜ *** ª: ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜, §¶: ÏÂÌÊÔÂÓÈÎfi˜, §∂: ÏÂÌÊÔÂÈÎÚ·ÙÒÓ, √™: Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘ # ÌË ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿, ¶√§À: ÔÏ˘ÌÔÚÊÔ‡ÚËÓ·, ∏ø™: ˈÛÈÓfiÊÈÏ· Paediatriki 2009;72:126-000

6:0 10,5 8,5 57 3 13,8 301 16 27 380

20:26 12,7 11,4 70 3 11,1 393 70 74 383

ƒ 0,000 0,017 0,007 ns# ns ns ns ns ns ns

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·127

127

§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿

¶›Ó·Î·˜ 5. ÷ڷÎÙËÚÈÛÙÈο ·ÛıÂÓÒÓ Ì ˘ÔÙÚÔ‹ Ã√

ºÀ

∏§π

™∆

π™∆

£ÂÚ·›· ∂›‰Ô˜ Â·Ú΋˜ ıÂÚ·›·˜

1

20*

∫√

13.5**

πV

ê

¡∞π

2 3 4 5 6

30 24 40 40 30

∞° A° ∫√ ∫√ ∞°

4.5 8 14.2 14 13.2

π ππ πππ πππ πππ

ª∫ ª∫ √™ √™ √™

¡∞π ¡∞π √Ãπ √Ãπ ¡∞π

7

25

∞°

6

πV

ê

√Ãπ

8

65

∞°

14

πV

ê

√Ãπ

∂›‰Ô˜ ã

ã & ∞£ OPPA + COPP ∞£ ã & ∞£ VBVP ã §√π¶∞ ã MOPP ã & ∞£ ABVD + MOPP ã & ∞£ ABVD + MOPP ã & ∞£ OPPA + COPP

∞£ ŒÎ‚·ÛË ÃÚfiÓÔ˜ ∂·Ú΋˜ ¢fiÛË ∂ÓÙfiÈÛË ¡∞π

20 Gy

∂ÛÙȷ΋

∑ÂÈ

32*

¡∞π ¡∞π

40 Gy 20 Gy

∂ÛÙȷ΋ ∂ÛÙȷ΋

253 90

¡∞π

20 Gy

∂ÛÙȷ΋

∑ÂÈ ∑ÂÈ ¢ÂÓ ˙ÂÈ ∑ÂÈ ∑ÂÈ

310 125

¡∞π

20 Gy

∂ÛÙȷ΋

∑ÂÈ

223

√Ãπ

20 Gy

∂ÛÙȷ΋

∑ÂÈ

78

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·˜, §∂: ÏÂÌÊÔÂÈÎÚ·ÙÒÓ *Ì‹Ó˜ ·fi ‰È¿ÁÓˆÛË, **¤ÙË

Î·È 32-310 Ì‹Ó˜ (¢∆ 125) ·fi ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ (ÂÈ‚›ˆÛË Û 2Ë ‡ÊÂÛË: 89%). ¢Â‡ÙÂÚË ‹ ÙÚ›ÙË Î·ÎÔ‹ıÂÈ· ‰È·ÈÛÙÒıËΠ۠4 ·È‰È¿, ·fi Ù· ÔÔ›· ˙Ô˘Ó Ù· 3. ∆· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ÂÌÊ¿ÓÈÛ·Ó ‰Â‡ÙÂÚË ‹ ÙÚ›ÙË Î·ÎÔ‹ıÂÈ· ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 6. ™˘ÓÔÏÈο ˙Ô˘Ó 71/73 ·ÛıÂÓ›˜ (Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË: 97,3%), 24-366 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË (¢∆ 156 Ì‹Ó˜) (ÂÈ‚›ˆÛË ÂχıÂÚË Û˘Ì‚¿Ì·ÙÔ˜: 83,6% - 61/73) (∂ÈÎfiÓ˜ 2, 3). ∆· 64 ·fi Ù· 73 ·È‰È¿ (87,7%) ˙Ô˘Ó Û Ï‹ÚË ÚÒÙË ‡ÊÂÛË.

£ÂÚ·›· ∏ ıÂÚ·›· ÎÚ›ıËΠˆ˜ Â·Ú΋˜ Û 68 ·ÛıÂÓ›˜ Î·È ·ÓÂ·Ú΋˜ Û 5. ™Â 68 ·È‰È¿ Ô˘ ¤Ï·‚·Ó ·ÎÙÈÓÔıÂÚ·›·, ·˘Ù‹ ‹Ù·Ó Â·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΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ˘ÔÙÚÔ‹˜ ÂÚÈÂÏ¿Ì‚·Ó ÌfiÓÔ Ã£ ‰Â‡ÙÂÚ˘ ÁÚ·ÌÌ‹˜ Û 3 ÂÚÈÙÒÛÂȘ Î·È Û˘Ó‰˘·ÛÌfi ã & ∞£ Û 5. ™Â 6/8 ·È‰È¿ ¤ÁÈÓÂ Î·È ·˘ÙfiÏÔÁË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ. ∞fi Ù· 8 ·È‰È¿ Ô˘ ·ÚÔ˘Û›·Û·Ó ˘ÔÙÚÔ‹ ˙Ô˘Ó Ù· 7, 12270 Ì‹Ó˜ (¢∆ 95) ·fi ÙË ‰È¿ÁÓˆÛË Ù˘ ˘ÔÙÚÔ‹˜

¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ∏ Û˘Û¯¤ÙÈÛË ÂȉËÌÈÔÏÔÁÈÎÒÓ, ÎÏÈÓÈÎÒÓ, ·ÂÈÎÔÓÈÛÙÈÎÒÓ, ÂÚÁ·ÛÙËÚÈ·ÎÒÓ Î·È ıÂÚ·¢ÙÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ 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·

1 2 3 4

Ã√

ºÀ

∏§π

™∆

π™∆ £ÂÚ·›· ∂›‰Ô˜ Â·Ú΋˜ ıÂÚ·›·˜

204* 144 48 71

∞° ∞° ∫√ ∫√

13** 4,9 12,2 11,2

π π ππ ππ

§∂ √™ √™ √™

¡∞π ¡∞π ¡∞π ¡∞π

∞£ ã & ∞£ ã & ∞£ ã & ∞£

∂›‰Ô˜ ã ∞µVD VBVP + OPPA ABVD + MOPP

∞£ ŒÎ‚·ÛË ÃÚfiÓÔ˜ ∂·Ú΋˜ ¢fiÛË ∂ÓÙfiÈÛË ¡∞π ¡∞π ¡∞π ¡∞π

40Gy 20 Gy 20 Gy 20 Gy

∂ÛÙȷ΋ ∂ÛÙȷ΋ ∂ÛÙȷ΋ ∂ÛÙȷ΋

¢ÂÓ ˙ÂÈ ∑ÂÈ ∑ÂÈ ∑ÂÈ

178* 61 175

Ã√: ¯ÚfiÓÔ˜ ·fi ÙË ‰È¿ÁÓˆÛË, ºÀ: ʇÏÔ, ∞°: ·ÁfiÚÈ·, ∫√: ÎÔÚ›ÙÛÈ·, ∏§π: ËÏÈΛ·, ™∆: ÛÙ¿‰ÈÔ, I™∆: ÈÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜, §∂: ÏÂÌÊÔÂÈÎÚ·ÙÒÓ, √™: Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË *Ì‹Ó˜ ·fi ‰È¿ÁÓˆÛË, **¤ÙË 1. ∞‰ÂÓÔηÚΛӈ̷ ÛÙÔÌ¿¯Ô˘ (2Ë) Î·È Î·ÚΛÓÔ˜ Ì·ÛÙÔ‡ (3Ë) 228 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË, ÏfiÁˆ ÙÔ˘ ÔÔ›Ô˘ η٤ÏËÍ 2. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜ 3. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜ 4. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜ (2Ë) Î·È ÔÛÙÂÔÛ¿ÚΈ̷ (3Ë) 94 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË ¶·È‰È·ÙÚÈ΋ 2009;72:127-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·128

128

1,00

1,00

0,80

0,80

0,60

0,60

¯100%

¯100%

∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.

0,40

0,20

0,20 0,00

0,40

0,00

0

100

200 ª‹Ó˜

300

400

0

100

200 ª‹Ó˜

300

400

∂ÈÎfiÓ· 2. ∫·Ì‡ÏË Û˘ÓÔÏÈ΋˜ ÂÈ‚›ˆÛ˘.

∂ÈÎfiÓ· 3. ∫·Ì‡ÏË ÂÈ‚›ˆÛ˘ ÂχıÂÚ˘ Û˘Ì‚¿Ì·ÙÔ˜.

ÁÈ· ÚÒÙË ÊÔÚ¿ ·fi ÙÔÓ Thomas Hodgkin ÙÔ 1932. ∏ ·ÈÙÈÔÏÔÁ›· Ù˘ ÓfiÛÔ˘ ·Ú·Ì¤ÓÂÈ ·‰È¢ÎÚ›ÓÈÛÙË. ∞ÔÙ¤ÏÂÛ ÁÈ· ÔÏϤ˜ ‰ÂηÂٛ˜ ·ÓÙÈΛÌÂÓÔ ÂȉËÌÈÔÏÔÁÈÎÒÓ ÂÚ¢ÓÒÓ, Ù· Â˘Ú‹Ì·Ù· ÙˆÓ ÔÔ›ˆÓ Ê·›ÓÂÙ·È Ó· ˘Ô‰ÂÈÎÓ‡Ô˘Ó ‰È·ÊÔÚÂÙÈÎÔ‡˜ ·ÈÙÈÔÏÔÁÈÎÔ‡˜ ·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˘˜ ÔÔ›Ô˘˜ ¤¯ÂÈ ÂȉÈο ÎÏÈÓÈο Î·È ÚÔÁÓˆÛÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο: Ë ÎÏ·ÛÈ΋ ÓfiÛÔ˜ (Classical Hodgkin’s lymphoma, CHL) Î·È Ô Ù‡Ô˜ Ô˙Ò‰Ô˘˜ ÏÂÌÊÔ΢ÙÙ·ÚÈ΋˜ ÂÈÎÚ¿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›· Î·È 62% ÛÙË ÌÂϤÙË Ì·˜). O Ù‡Ô˜ Ù˘ Ô˙Ò‰Ô˘˜ ÏÂÌÊÔ΢ÙÙ·ÚÈ΋˜ ÂÈÎÚ¿ÙËÛ˘ ··ÓÙ¿Ù·È Û ËÏÈ˘ 2545 ÂÙÒÓ, Ì ÂÈÎÚ¿ÙËÛË ÙÔ˘ ¿ÚÚÂÓÔ˜ ʇÏÔ˘, Î·È ·ÔÙÂÏ› ÙÔ 5% ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Hodgkin (12). ∞fi ÙÔ ÎÏ·ÛÈÎfi ϤÌʈ̷ Hodgkin, Ô ÏÂÌÊÔÂÈÎÚ·ÙÒÓ ˘fiÙ˘Ô˜ ‰È·ÈÛÙÒÓÂÙ·È ÛÙÔ 10-15% ÙˆÓ ·ÛıÂÓÒÓ, Â›Ó·È ÈÔ Û˘¯Ófi˜ ÛÙÔ ¿ÚÚÂÓ Ê‡ÏÔ Î·È ÙÔ˘˜ ÌÈÎÚfiÙÂÚÔ˘˜ Û ËÏÈΛ· ·ÛıÂÓ›˜, ÂÓÒ Û˘Ó‰¤ÂÙ·È Û˘¯ÓfiÙÂÚ· Ì ÂÓÙÔÈṲ̂ÓË ÓfiÛÔ (3). √ ÏÂÌÊÔÂÓÈÎfi˜ ˘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˘ÌÙÒÌ·Ù· Ù‡Ô˘ µ, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ٷ¯¤ˆ˜ ÂÍÂÏÈÛÛfiÌÂÓË 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). √ ˘fiÙ˘Ô˜ Ù˘ ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ ÂÌÊ·Ó›˙ÂÙ·È ÛÙÔ 14-38% ÙˆÓ ·ÛıÂÓÒÓ (5,6,11), ‰È·ÈÛÙÒÓÂÙ·È Û˘¯ÓfiÙÂÚ· Û ·È‰È¿ 10 ÂÙÒÓ ‹ ÌÈÎÚfiÙÂÚ· Î·È ÂÌÊ·Ó›˙ÂÙ·È Û˘¯Ó¿ ˆ˜ ÚÔ¯ˆÚË̤ÓË ÓfiÛÔ˜ Ì Â͈ÏÂÌÊ·‰ÂÓÈ΋

ÂÓÙfiÈÛË. ∏ Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË ·ÔÙÂÏ› ÙÔÓ ÈÔ Û˘¯Ófi ˘fiÙ˘Ô ÛÙȘ ·ÓÂÙ˘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 ÔÔ›Ô ‰ÂÓ ÂÈÎÚ·Ù› ÙÔ ¿ÚÚÂÓ Ê‡ÏÔ, ηıÒ˜ ‰È·ÈÛÙÒÓÂÙ·È Ì ›ÛË Û˘¯ÓfiÙËÙ· Û ¿ÚÚÂÓ· Î·È ı‹Ï· (13). °ÂÓÈο, Ô ÏÂÌÊÔÂÈÎÚ·ÙÒÓ ˘fiÙ˘Ô˜ Î·È ·˘Ùfi˜ Ù˘ Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÓfiÛÔ ¯·ÌËÏÔ‡ ÛÙ·‰›Ô˘, Ô ÏÂÌÊÔÂÓÈÎfi˜ Ì ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È·, ÂÓÒ Ô ˘fiÙ˘Ô˜ Ù˘ ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ Ì ÂӉȿÌÂÛ·. ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ÂÈÎÚ·Ù› Ë Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË (63%), ÂÓÒ ÌfiÓÔ Û ‰‡Ô ·È‰È¿ ‰È·ÈÛÙÒıËΠÏÂÌÊÔÂÓÈÎfi˜ ˘fiÙ˘Ô˜. ∆¤ÏÔ˜, fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ¤Î‚·ÛË ‰ÂÓ ‰È·ÈÛÙÒıËΠÂ›‰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 ¯ËÌÂÈÔıÂÚ·›·˜, Ù· ÈÛÙÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο ¤¯Ô˘Ó ÌÈÎÚfiÙÂÚË ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· (14). ∏ ‰È¿ÌÂÛË ÙÈÌ‹ ËÏÈΛ·˜ ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÌÂϤÙ˘ 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 ·ÓÂÙ˘Á̤Ó˜ ¯ÒÚ˜ (4), ·ÏÏ¿ ÚÔÛÂÁÁ›˙ÂÈ ‹ Û·ÊÒ˜ ˘ÔÏ›ÂÙ·È ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô ÔÛÔÛÙfi ¯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¿ ÂÈÎÚ¿ÙËÛË ÙˆÓ ·ÚÚ¤ÓˆÓ (1.5:1), Ô˘ fï˜ Êı›ÓÂÈ Ì ÙËÓ ¿ÚÔ‰Ô Ù˘ ËÏÈΛ·˜, Ì ÙËÓ ·Ó·ÏÔÁ›· Ó· Â›Ó·È ·ÚfiÌÔÈ· ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ (3). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ‰È·ÈÛÙÒÓÂÙ·È Û·Ê‹˜ ÂÈÎÚ¿ÙËÛË ÙÔ˘ ¿ÚÚÂÓÔ˜ ʇÏÔ˘, Ì ٷ ÎÔÚ›ÙÛÈ· Ó· ¤¯Ô˘Ó Û ÛËÌ·ÓÙÈÎfi ‚·ıÌfi ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ·. ÷ڷÎÙËÚÈÛÙÈο ‰È·ÈÛÙÒÓÔ˘Ì ˆ˜

Paediatriki 2009;72:128-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·129

129

§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿

¶›Ó·Î·˜ 7. ¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ˆ˜ ÚÔ˜ ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘

∏ÏÈΛ· (¤ÙË ≤8 >8 º‡ÏÔ ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ· ™Ù¿‰ÈÔ π ππ πππ IV πÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜ ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ §ÂÌÊÔÂÓÈÎfi˜ §ÂÌÊÔÂÈÎÚ·ÙÒÓ √˙҉˘ ÛÎÏ‹Ú˘ÓÛË µ-Û˘ÌÙÒÌ·Ù· ¶·ÚfiÓÙ· ∞fiÓÙ· ªÂÛÔıˆÚ¿ÎÈÔ ªÂÁ¿ÏË ‰È‡ڢÓÛË ª¤ÙÚÈ· ‰È‡ڢÓÛË Ÿ¯È ‰È‡ڢÓÛË §Â˘Î¿ ·ÈÌÔÛÊ·›ÚÈ· (x103/mm3) ≤10,2 >10,2 Hb (g/dl) ≤11,1 >11,1 TKE (mm 1˘ ÒÚ·˜) ≤61 >61

∞ÚÈıÌfi˜ ·ÛıÂÓÒÓ

%

DFS

% EFS

OS

DFS

ƒ EFS

OS

15 58

20,5 79,5

80 91,4

73,3 86,2

100 96,6

ns

ns

ns

46 27

63 37

89,1 88,9

84,8 81,5

97,8 96,3

ns

ns

ns

16 25 20 12

22 34,2 27,4 16,4

93,8 96 85 75

81,3 92 80 75

93,8 100 95 100

ns

ns

ns

19 2 6 46

26,1 2,7 8,2 63

89,5 100 100 87

89,5 100 83,3 80,4

100 100 83,3 97,8

ns

ns

ns

21 52

29 71

90,5 88,5

90,5 80,8

95,2 98,1

ns

ns

ns

13 33 27

17,8 45,2 37

92,3 87,9 88,9

84,6 84,8 81,5

100 97 96,3

ns

ns

ns

34 33

50,7 49,3

88,2 90,9

82,4 84,8

94,1 100

ns

ns

ns

36 32

53 47

88,9 90,6

80,6 87,5

100 93,8

ns

ns

ns

34 34

50 50

88,2 91,2

82,4 85,3

94,1 100

ns

ns

ns

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 ‚ÈÔÌ˯·ÓÈΤ˜ ¯ÒÚ˜), ·ÏÏ¿ Î·È Ì ¤ÎıÂÛË Û ÏÔÈÌÒ‰ÂȘ ·Ú¿ÁÔÓÙ˜ (EBV, ÂÚËÙÔ˚fi˜ 6, CMV) ‹ Ì ·ÓÔÛÔ·ÓÂ¿ÚÎÂÈ· (3). ™ËÌ·ÓÙÈ΋ ·Ó·ÏÔÁ›· ÙˆÓ ·ÛıÂÓÒÓ Ì ÓfiÛÔ ∏odgkin ¤¯Ô˘Ó ˘„ËÏÔ‡˜ Ù›ÙÏÔ˘˜ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ EBV (17). ™ÙËÓ ÂÚ›ÙˆÛË Ù˘ ÎÏ·ÛÈ΋˜ ÓfiÛÔ˘ Hodgkin, ÛÙȘ ¢˘ÙÈΤ˜ ¯ÒÚ˜ Ë Û‡Ó‰ÂÛË Ì 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 ÌÔÚ› Ó· ÚÔÛ‚ÏËı›. ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ‰È·È-

ÛÙÒıËΠÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· Û ÔÛÔÛÙfi 93%. ªË ÂȉÈο Û˘ÌÙÒÌ·Ù· Ù˘ ÓfiÛÔ˘ ·ÔÙÂÏÔ‡Ó Ë Î·ÎÔ˘¯›·, Ë ·ÓÔÚÂÍ›·, ÙÔ ·Ú·ÙÂÈÓfiÌÂÓÔ ˘Ú¤ÙÈÔ Î·È Ë ·ÒÏÂÈ· ‚¿ÚÔ˘˜ (3). ∂›Û˘, ÌÔÚ› Ó· Û˘Ó˘¿Ú¯ÂÈ ÌË ·Ú·ÁˆÁÈÎfi˜ ¯ÚfiÓÈÔ˜ ‚‹¯·˜ ‹ ¿ÏÏ· Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi, ÏfiÁˆ Ù˘ ‰È‡ڢÓÛ˘ ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘, Ô˘ ÚÔηÏ› ›ÂÛË Ù˘ ÙÚ·¯Â›·˜ Î·È ÙˆÓ ‚ÚfiÁ¯ˆÓ. ∏ ·ÚÔ˘Û›· µ-Û˘Ìو̿وÓ, fiˆ˜ ·Ó·Ê¤ÚıËΠ‹‰Ë, ıˆÚÂ›Ù·È ˆ˜ ¤¯ÂÈ ÛËÌ·ÓÙÈ΋ ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· Î·È Û˘Ó‰¤ÂÙ·È Ì ÂÈıÂÙÈÎfiÙÂÚË ÓfiÛÔ (21). ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ·Ó·Ê¤ÚıËÎ·Ó Û ·Ó·ÏÔÁ›· 29%, Ì ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚË ·Ó·ÏÔÁ›· 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˘ÌÙˆÌ¿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, fiˆ˜ Ï¢ÎÔ΢ÙÙ¿ÚˆÛË, ˈÛÈÓÔÊÈÏ›·, ¶·È‰È·ÙÚÈ΋ 2009;72:129-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·130

130

∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.

·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·ÈÛÙÒıËΠӷ ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ·ÚÔ˘Û›· ‰È‡ڢÓÛ˘ ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘, ·ÎfiÌË Î·È ÂÎÙÂٷ̤Ó˘, ·Ó Î·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È ˆ˜ Ì¿˙· ÌÂÛÔıˆÚ·Î›Ô˘ Ô˘ ˘ÂÚ‚·›ÓÂÈ ÙÔ 1/3 Ù˘ ÌÂÁ·Ï‡ÙÂÚ˘ ÂÓ‰ÔıˆÚ·ÎÈ΋˜ ‰È·Ì¤ÙÚÔ˘ Û˘Ó‰¤ÂÙ·È Ì ÚÔ¯ˆÚË̤ÓË ÓfiÛÔ, Ùˆ¯fiÙÂÚË ÚfiÁÓˆÛË Î·È ·Ó¿ÁÎË ÂÈıÂÙÈÎfiÙÂÚ˘ ıÂÚ·›·˜ (22). ™˘ÓÔÏÈο, Ë ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ ‹Ù·Ó ÂÍ·ÈÚÂÙÈ΋ Û fiϘ ÙȘ ¯ÚÔÓÈΤ˜ ÂÚ›Ô‰Ô˘˜ ·ÓÙÈÌÂÙÒÈÛ˘ ·˘ÙÒÓ Î·È ·ÓÂÍ¿ÚÙËÙË ·fi ÙÔ Â›‰Ô˜ Ù˘ ıÂÚ·›·˜. ∏ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË Êı¿ÓÂÈ ÙÔ 97,3%, ÂÓÒ ÂÍ·ÈÚÂÙÈ΋ Â›Ó·È Î·È Ë ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ˘ÔÙÚÔ‹ - ÂÈ‚ÈÒÓÔ˘Ó ÔÈ 7/8. ∏ ÂÈ‚›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¿ÁÓˆÛË. √È ˘ÔÙÚÔ¤˜ Û˘Ì‚·›ÓÔ˘Ó Û˘Ó‹ıˆ˜ Ù· ÚÒÙ· 3 ¤ÙË, ·ÏÏ¿ ÔÚÈṲ̂ÓÔÈ ·ÛıÂÓ›˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Î·È fi„È̘ ˘ÔÙÚÔ¤˜ (¿Óˆ ·fi 10 ¤ÙË) (23). √È ·ÏÏ·Á¤˜ ÛÙÔÓ ÙÚfiÔ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ÓfiÛÔ˘ ‰È·¯ÚÔÓÈο ÔÊ›ÏÔÓÙ·È ÛÙËÓ ÂÌÂÈÚ›· ·fi ÙËÓ ÂÊ·ÚÌÔÁ‹ ·ÚfiÌÔÈˆÓ ıÂÚ·¢ÙÈÎÒÓ ÚÔÛÂÁÁ›ÛˆÓ. ™ÙËÓ ÚÒÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô, Ù· ·È‰È¿ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó ˆ˜ ÂÓ‹ÏÈΘ Î·È ÛÙË ıÂÚ·›· ΢ÚÈ¿Ú¯ËÛ·Ó ÔÈ ˘„ËϤ˜ ‰fiÛÂȘ ·ÎÙÈÓÔ‚ÔÏ›·˜ (̤¯ÚÈ Î·È 40 Gy) ‹ Ë ·ÓÙÈÌÂÙÒÈÛË Ì ÔÏÏ¿ Û¯‹Ì·Ù· ¯ËÌÂÈÔıÂÚ·›·˜ ¯ˆÚ›˜ ·ÎÙÈÓÔıÂÚ·›·. 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‰ÔÎÚÈÓÔÏÔÁÈο, ˘‹ÚÍ ·ÏÏ·Á‹ ÛÙË ıÂÚ·¢ÙÈ΋ ÛÙÚ·ÙËÁÈ΋ Î·È Ù· ·È‰È¿-·ÛıÂÓ›˜ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Ì Ì›ˆÛË Ù˘ ¯ËÌÂÈÔıÂÚ·›·˜ ÛÙËÓ ÂÓÙÔÈṲ̂ÓË ÓfiÛÔ Î·È, ‚¤‚·È·, Ì ηıȤڈÛË ¯·ÌËÏÒÓ ‰fiÛÂˆÓ ·ÎÙÈÓÔ‚ÔÏ›·˜ (20 Gy) ÌfiÓÔ ÛÙȘ ·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜. ∆¤ÏÔ˜, ÛÙËÓ ÙÚ›ÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô, Ë ıÂÚ·¢ÙÈ΋ ÔÌ¿‰·, ‰È·ÙËÚÒÓÙ·˜ ÙȘ ¯·ÌËϤ˜ ‰fiÛÂȘ ·ÎÙÈÓÔ‚ÔÏ›·˜, ÚÔ¯ÒÚËÛ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·È‰ÈÒÓ Ì ÂÓÙÔÈṲ̂ÓË ¯·ÌËÏÔ‡ Î·È ÂӉȿÌÂÛÔ˘ ÎÈÓ‰‡ÓÔ˘ ÓfiÛÔ Ì ¯ËÌÂÈÔıÂÚ·¢ÙÈο Û¯‹Ì·Ù· ¯ˆÚ›˜ ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜ Î·È ·Óıڷ΢ÎϛӘ, Ô˘ Â›Ó·È Ê¿Ú̷η ˘‡ı˘Ó· ÁÈ· ÛËÌ·ÓÙÈ΋ ·ÒÙÂÚË ÙÔÍÈÎfiÙËÙ·. °ÂÓÈο, fiÏ· ·˘Ù¿ Ù· ¯ÚfiÓÈ· ÔÈ ÛÙfi¯ÔÈ Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ Ì ÓfiÛÔ ∏odgkin ‹Ù·Ó Ë ‰È·Ù‹ÚËÛË ÙˆÓ ˘„ËÏÒÓ ÔÛÔÛÙÒÓ ÂÈ‚›ˆÛ˘, Ì ٷ˘Ùfi¯ÚÔÓË Ì›ˆÛË Ù˘ Ì·ÎÚÔÚfiıÂÛÌ˘ ÙÔÍÈÎfiÙËÙ·˜ (ηډÈÔÙÔÍÈÎfiÙËÙ·, Ó¢ÌÔÓÔ¿ıÂÈ·, ÛÎÂÏÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÂÓ‰ÔÎÚÈÓÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜, ‰Â‡ÙÂÚ˜ ηÎÔ‹ıÂȘ) (24). ∞fi ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜, Ù¤ÛÛÂÚȘ ·ÚÔ˘Û›·Û·Ó ‰Â‡ÙÂÚË ‹ Î·È ÙÚ›ÙË Î·ÎÔ‹ıÂÈ·. ∏ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ‰Â‡ÙÂÚ˘ ηÎÔ‹ıÂÈ·˜ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ÂÊ·ÚÌÔÁ‹ ‹ fi¯È ·ÎÙÈÓÔPaediatriki 2009;72:130-000

ıÂÚ·›·˜ Î·È ÙË ‰fiÛË ·˘Ù‹˜, ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ ‹ ÙËÓ ‡·ÚÍË ˘ÔÙÚÔ‹˜ Î·È ÙËÓ ·ÚÔ˘Û›· ·ÓÔÛÔηٷÛÙÔÏ‹˜. π‰È·›ÙÂÚÔ ÚfiÏÔ Ê·›ÓÂÙ·È Ó· ·›˙ÂÈ Ë ¯Ú‹ÛË ·Ï΢ÏȈÙÈÎÒÓ ·Ú·ÁÒÁˆÓ (ª√PP), ηıÒ˜ Î·È ÔÈ ‰fiÛÂȘ Ù˘ ¯ËÌÂÈÔıÂÚ·›·˜ (25). √ ΛӉ˘ÓÔ˜ ÁÈ· ÂÌÊ¿ÓÈÛË ÌË ÏÂÌÊÔ‚Ï·ÛÙÈ΋˜ Ï¢¯·ÈÌ›·˜ ‹ ÌË ∏odgkin ÏÂÌÊÒÌ·ÙÔ˜ Êı¿ÓÂÈ ÙÔ 8,1% ÌÂÙ¿ ·fi ıÂÚ·›· 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 ıÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Ù˘ ÓfiÛÔ˘ ÛÙËÚ›˙ÂÙ·È Û ÚÔÛÂÁÁ›ÛÂȘ ÚÔÛ·ÚÌÔṲ̂Ó˜ ÛÙËÓ ·¿ÓÙËÛË ÛÙËÓ ·Ú¯È΋ ıÂÚ·›·, ÛÙÔ Ê‡ÏÔ Î·È ÙËÓ ËÏÈΛ·. √ Û‡Á¯ÚÔÓÔ˜ ÙÚfiÔ˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ·ÛıÂÓÒÓ Ì ÂÓÙÔÈṲ̂ÓË ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÓfiÛÔ ÂÚÈÏ·Ì‚¿ÓÂÈ ÏÈÁfiÙÂÚÔ ÙÔÍÈ΋ ıÂÚ·›· Ì 2-4 ·ÎÏÔ˘˜ ¯ËÌÂÈÔıÂÚ·›·˜ Î·È ¯·ÌËϤ˜ ‰fiÛÂȘ ·ÎÙÈÓÔ‚ÔÏ›·˜, ÂÓÒ ÁÈ· ÙÔ˘˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Î·È Ì ÚÔ¯ˆÚË̤ÓË ÓfiÛÔ ·ÛıÂÓ›˜ ‰ÔÎÈÌ¿˙ÔÓÙ·È ÂÚÈÛÛfiÙÂÚÔ ÂÓÙ·ÙÈο Û¯‹Ì·Ù· ıÂÚ·›·˜ (27). ∆ÂÏÂ˘Ù·›·, ÙÔ Î˘Ú›·Ú¯Ô ÂÚÒÙËÌ· ÙˆÓ ÌÂÁ¿ÏˆÓ Û˘ÓÂÚÁ·ÙÈÎÒÓ ÌÂÏÂÙÒÓ Â›Ó·È «ÔÈÔÈ ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ ÌÔÚÔ‡Ó Ó· È·ıÔ‡Ó ¯ˆÚ›˜ ÙË ¯Ú‹ÛË ·ÎÙÈÓÔıÂÚ·›·˜;». √ Â·Ó·ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ ÔÌ¿‰ˆÓ ÎÈÓ‰‡ÓÔ˘ Ì ӤԢ˜ ‚ÈÔÏÔÁÈÎÔ‡˜ Î·È ÌÔÚÈ·ÎÔ‡˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ‰˘ÓËÙÈο ı· Ô‰ËÁ‹ÛÂÈ Û ÂÚ·ÈÙ¤Úˆ ÂÚÈÔÚÈÛÌfi Ù˘ ıÂÚ·›·˜ ‹ Û ÂÓÙ·ÙÈÎÔÔ›ËÛË ·˘Ù‹˜. √È ÚfiÔ‰ÔÈ ÛÙË ıÂÚ·›· ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin ¤¯Ô˘Ó ÌÂÈÒÛÂÈ ÙËÓ ÚÔÁÓˆÛÙÈ΋ ·Í›· ·Ú·‰ÔÛÈ·ÎÒÓ ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, fiˆ˜ Ë ËÏÈΛ·, ÙÔ Ê‡ÏÔ, Ë ÈÛÙÔÏÔÁ›·, ÙÔ ÛÙ¿‰ÈÔ Î·È Ù· Û˘ÌÙÒÌ·Ù· Ù‡Ô˘ µ. ∏ ·¿ÓÙËÛË ÛÙËÓ ·Ú¯È΋ ıÂÚ·›· ıˆÚÂ›Ù·È Û‹ÌÂÚ· ÚÔÛ‰ÈÔÚÈÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÁÈ· ÙËÓ Â·ÎfiÏÔ˘ıË ıÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË. √È ÚfiÔ‰ÔÈ ÛÙȘ ·ÂÈÎÔÓÈÛÙÈΤ˜ ÌÂıfi‰Ô˘˜, fiˆ˜ Ë ¯Ú‹ÛË ˘ÔÏÔÁÈÛÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ ˘„ËÏ‹˜ ¢ÎÚ›ÓÂÈ·˜ (HRCT), Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ (MRI), ·ÏÏ¿, ÙÂÏÂ˘Ù·›·, Î·È Ë ¯Ú‹ÛË ÙÔÌÔÁÚ·Ê›·˜ ÂÎÔÌ‹˜ Ô˙ÈÙÚÔÓ›ˆÓ (PET) [ÂÎÙÈÌ¿ ÙËÓ ˘ÂÚÏ·ÛÙÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ ÏÂÌÊ·‰¤ÓˆÓ ‰È· ̤ÛÔ˘ Ù˘ ·˘ÍË̤Ó˘ ÚfiÛÏ˄˘ ÙÔ˘ 18-fluoro-2-deoxyglucose (FDG)] ¤¯Ô˘Ó ʤÚÂÈ Â·Ó¿ÛÙ·ÛË ÛÙË ÛÙ·‰ÈÔÔ›ËÛË ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin. ∏ ÙÔÌÔÁÚ·Ê›· ÂÎÔÌ‹˜ Ô˙ÈÙÚÔÓ›ˆÓ Î·È ÂȉÈο Ô Û˘Ó‰˘·ÛÌfi˜ Ù˘ Ì ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· (PET-CT) ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂÁ¿ÏË Â˘·ÈÛıËÛ›· ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·ÓÙ·fiÎÚÈÛ˘ Î·È ÙËÓ ÔÚıfiÙÂÚË ¤ˆ˜ Î·È ÂÍ·ÙÔÌÈÎÂ˘Ì¤ÓË ıÂÚ·›·, Ì ÚÔÔÙÈ΋ ÙËÓ ·ÔÊ˘Á‹ Ù˘ ·ÎÙÈÓÔıÂÚ·›·˜ Û ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ·ÛıÂÓÒÓ (28).

µÈ‚ÏÈÔÁÚ·Ê›· 1. Gurney J, Bondy M. Epidemiology of childhood cancer. In: Pizzo AP, Poplack DG (eds). Principles and Practice of Pediatric Oncology, 5th Edt. Philadelphia, PA: LippincottRaven Publishers; 2006, Chapter 1, pp. 1-13.

2. Petridou E, Pourtsidis A, Dessypris N, Katsiardanis K, Baka

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131

§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿

M, Moschovi M, et al. Childhood leukaemias and lymphomas in Greece (1996-2006): a nationwide registration study. Archives of Disease in Childhood. 2008;93: 1027-1032.

3. Hudson M, Onciu M, Donaldson S. Hodgkin’s Disease. In: Pizzo P, Poplack D, editors. Principles and Practise of Pediatric Oncology, 5th Edt. Philadelphia; LipincottRaven Publishers; 2006, pp. 695-721.

4. Donaldson SS, Link MP, Weinstein HJ, Rai SN, Brain S, Billett AL, et al. Final results of a prospective clinical trial with VAMP and low-dose involved-field radiation for children with low-risk Hodgkin's disease. J Clin Oncol 2007;25:332-337.

5. Oguz A, Karadeniz C, Okur FV, Citak EC, Pinarli FG, Bora H, et al. Prognostic factors and treatment outcome in childhood Hodgkin disease. Pediatr Blood Cancer 2005;45: 670-675.

6. Hudson MM, Krasin M, Link MP, Donaldson SS, Billups C, Merchant TE, et al. Risk-adapted, combined-modality therapy with VAMP/COP and response-based, involvedfield radiation for unfavorable pediatric Hodgkin's disease.

J Clin Oncol 2004;22:4541-4550.

7. Lister TA, Crowther D, Sutcliffe SB. Report of a committee convened to discuss the evaluation and staging of patients with Hodgkin’s disease: Cotswolds meeting. J Clin Oncol 1989;7:1630-1636.

8. Weinshel EL, Peterson BA. Hodgkin’s disease. CA: Cancer J Clin. 1993;43:327-372.

9. Oberlin O, Leverger G, Pacquement H, Raquin MA, Chompret A, Habrand JL, et al. Low-dose radiation therapy and reduced chemotherapy in childhood Hodgkin’s disease: the experience of the French Society of Pediatric Oncology. J Clin Oncol 1992;10:1602-1608.

10. Landman-Parker J, Pacquement H, Leblanc T, Habrand JL, Terrier-Lacombe MJ, et al. Localized Childhood Hodgkin’s Disease: Response-Adapted Chemotherapy With Etoposide, Bleomycin, Vinblastine, and Prednisone Before Low-Dose Radiation Therapy - Results of the French Society of Pediatric Oncology Study MDH90. J Clin Oncol 2000;18:1500-1507.

11. Feldman AL, Pittaluga S, Jaffe ES. Classification and histopathology of the lymphomas. In: Canellos GP, Lister TA, Young B (eds) The lymphomas. 2nd ed., Saunders Elsevier, Philadelphia; 2006, pp. 2-25.

12. Nogovaã L, Rudiger T, Engert A. Biology, clinical course and management of nodular lymphocyte-predominant hodgkin lymphoma. Hematology Am Soc Hematol Educ Program 2006;1:266-272.

13. Smith RS, Chen Q, Hudson MM, Link MP, Kun L, Weinstein H et al. Prognostic factors for children with Hodgkin’s disease treated with combined-modality therapy.

J Clin Oncol. 2003;21:2026-2033.

14. Schwartz CL. Prognostic Factors in Pediatric Hodgkin Disease. 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: Clinical characteristics and outcome of treatment. Leuk Lymphoma 2008;49:910-916.

16. Viani GA, Castilho MS, Novaes PE, Antonelli CG, Ferrigno R, Pellizzon CA, et al. Chemotherapy followed by low dose radiotherapy in childhood Hodgkin’s disease: Retrospective 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: Prevalence and significance of Epstein-Barr virus detection in Hodgkin’s and Reed-Sternberg cells. European Journal of Cancer 2007;43:161-168.

18. Portis T, Dyck P, Longnecker R. Epstein-Barr Virus (EBV) LMP2A induces alterations in gene transcription similar to those observed in Reed-Sternberg cells of Hodgkin lymphoma. Blood 2003;102:4166-4178.

19. Landgren O, Engels EA, Pfeiffer RM, Gridley G, Mellemkjaer L, Olsen JH, et al. Autoimmunity and susceptibility to Hodgkin lymphoma: a population-based case-control study in 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 the young 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 for primary, refractory, and relapsed disease. Hematology. Am Soc Hematol Educ Program 2003;1:225-247.

23. Cashen ∞, Bartlett ¡. Therapy of Relapsed Hodgkin Lymphoma. 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 of second malignancy after Hodgkin's disease. Ann Oncol 2006;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-year follow-up study. J Clin Oncol 1994;12:312-325.

27. Olson MR, Donaldson SS. Treatment of pediatric Hodgkin lymphoma. Curr Treat Options Oncol 2008;9:81-94.

28. Malik E. Juweid. Utility of Positron Emission Tomography (PET) Scanning in Managing Patients with Hodgkin Lymphoma. Hematology, Am Soc Hematol Educ Program 2006;1:259-265.

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√È ÂÈÙÒÛÂȘ Ù˘ ˘ÂÚÙÚÔÊ›·˜ ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ ÛÙËÓ Î·Ú‰È·Î‹ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ÚÈÛÙÔÙ¤ÏÂÈÔ˘ ¶·ÓÂÈÛÙ‹ÌÈÔ˘ £ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘» AÏÏËÏÔÁÚ·Ê›·: ∫˘Úȷ΋ ¶··‰ÔÔ‡ÏÔ˘§ÂÁÌ¤ÏÔ˘ kelipap@gmail.com ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£, ¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘» ¶ÂÚÈÊÂÚÂȷ΋ Ô‰fi˜ £ÂÛ/ӛ΢, ¡. ∂˘Î·Ú›·, 56403, £ÂÛÛ·ÏÔÓ›ÎË

∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁÌ¤ÏÔ˘, °. 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˘ ÌÔÚ› Ó· ¤¯ÂÈ ÛÔ‚·Ú¤˜ ÂÈÙÒÛÂȘ ÛÙËÓ Î·Ú‰ÈÔÓ¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ. ∏ ·fiÊÚ·ÍË ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ÏfiÁˆ ÂÏ¿ÙÙˆÛ˘ ÙÔ˘ Ó¢ÚÔÌ˘˚ÎÔ‡ ÙfiÓÔ˘, Î·È Ô‰ËÁ› Û ˘fiÓÔÈ· ‹ ¿ÓÔȘ ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘. √È ÙÂÏÂ˘Ù·›Â˜ Û˘Óԉ‡ÔÓÙ·È ·fi ÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜, ˘ÂÚηÓ›·˜ Î·È ·Ó·Ó¢ÛÙÈ΋˜ ÔͤˆÛ˘ (Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘). ∆· ˘ÔÙÚÔÈ¿˙ÔÓÙ· ÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜ Î·È ˘ÂÚηÓ›·˜ ÚÔηÏÔ‡Ó Ó¢ÌÔÓÈ΋ ·ÁÁÂÈÔÛ‡Û·ÛË, Ë ÔÔ›· Ô‰ËÁ› Û ÌfiÓÈ̘ ·ÏÏÔÈÒÛÂȘ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ. √È ÂÈÙÒÛÂȘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÂÂÈÛfi‰È· ˘ÂÚÙ·ÛÈÎÒÓ ÎÚ›ÛÂˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ ηÈ, Û ·Ú·ÌÂÏË̤Ó˜ ÂÚÈÙÒÛÂȘ, 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·, ÌÔÚ› Ó· ˘¿Ú¯ÂÈ ·Û˘Ìو̷ÙÈ΋ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ª¤¯ÚÈ Û‹ÌÂÚ·, ˆ˜ ıÂÚ·›· ÚÒÙ˘ ÂÎÏÔÁ‹˜ Û ·È‰È¿ Ì ۇӉÚÔÌÔ ·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‹ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ¤¯Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ¿ÌÂÛ˜ ÌÂÙÂÁ¯ÂÈÚËÙÈΤ˜ ÂÈÏÔΤ˜, fiˆ˜ Âȉ›ӈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‹ ÂΉ‹ÏˆÛË Ó¢ÌÔÓÈÎÔ‡ Ôȉ‹Ì·ÙÔ˜. ŸÛÔÓ ·ÊÔÚ¿ ÛÙȘ ·ÒÙÂÚ˜ ÌÂÙÂÁ¯ÂÈÚËÙÈΤ˜ ÂÈÏÔΤ˜, ·˘Ù¤˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ·Ú·ÌÔÓ‹ ΢„ÂÏȉÈÎÔ‡ ˘Ô·ÂÚÈÛÌÔ‡ (Ô˘ ÌÔÚ› Ó· ¯ÚÂÈ·Ûı› ̤¯ÚÈ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ‹ ÙÚ·¯ÂÈÔÛÙÔÌ›·), Â›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˯ÔηډÈÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÚÈÓ ·fi ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË. ÃÚÂÈ¿˙ÂÙ·È Â›Û˘ ÎÏÈÓÈ΋ Â·ÓÂÎÙ›ÌËÛË ·˘ÙÒÓ ÙˆÓ ·ÛıÂÓÒÓ ÌÂÙ¿ ·fi 6-8 ‚‰ÔÌ¿‰Â˜ ·fi ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔÌ‹-·Ì˘Á‰·ÏÂÎÙÔÌ‹, ÁÈ· Ó· ‰È·ÈÛÙˆı› ·Ó ··ÈÙÂ›Ù·È ÂÈÚfiÛıÂÙË ıÂÚ·›·. §¤ÍÂȘ ÎÏÂȉȿ: ÀÂÚÙÚÔÊ›· ·‰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 4th Department of Paediatrics, “Aristotelio” University of Thessaloniki, “PapageÔrgiou” Hospital, Thessaloniki, Greece Correspondence: Kyriaki PapadopoulouLegbelou kelipap@gmail.com 4th Department of Paediatrics, “Aristotelio” University of Thessaloniki, “PapageÔrgiou” Hospital Ring Road, N. Efkarpia, 56403, Thessaloniki, Greece

K. Papadopoulou-Legbelou, G. Varlamis Abstract: Adenotonsillar hypertrophy is the most common cause of upper airway obstruction, which may lead to serious cardiopulmonary complications in children. Upper airway obstruction can be exacerbated by the decrease in neuromuscular tone that occurs during sleep, leading to hypopnoea or obstructive sleep apnoea. Obstructive sleep apnoea produces episodic hypoxia, hypercapnia and respiratory acidosis (obstructive sleep apnoea syndrome). Episodes of recurrent hypoxia and hypercapnia are potent mediators of pulmonary vasoconstriction that can lead to chronic changes in the pulmonary vasculature.

Cardiovascular consequences of adenotonsillar hypertrophy include repetitive nocturnal surges in blood pressure, breathing rhythm disturbances and, particularly in severe cases, pulmonary hypertension and cor pulmonale. Although overt right heart failure now occurs less often, asymptomatic degrees of pulmonary hypertension can occur. To date, adenotonsillectomy is the first line of treatment for children with obstructive sleep apnoea syndrome. Children with significant airway obstruction associated with pulmonary hypertension and/or cardiac dysfunction are at greater risk for perioperative complications, such as worsening of their obstructive sleep apnoea, and pulmonary oedema. Potential postoperative complications are persistent airway obstruction (including the need for reintubation or tracheostomy), desaturation, sleep disturbances, or a combination of these. Thus, patients with severe obstruction should be screened by echocardiography preoperatively. Clinical reevaluation is also recommended 6 to 8 weeks after adenotonsillectomy, to determine whether additional treatment is required.

Key words: Adenotonsillar hypertrophy, obstructive sleep apnoea syndrome, pulmonary hypertension, cor pulmonale.

Paediatriki 2009;72:132-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·133

133

∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ

™˘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·˜ ·fiÊڷ͢ ÙˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ, Ô˘ ÌÔÚ› Ó· ¤¯ÂÈ ÛÔ‚·Ú¤˜ ÂÈÙÒÛÂȘ ÛÙËÓ Î·Ú‰ÈÔÓ¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ. ∏ ·fiÊÚ·ÍË ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, fiÔ˘ ¯·Ï·ÚÒÓÔ˘Ó ÔÈ Ê·Ú˘ÁÁÈÎÔ› ̇˜, ηıÒ˜ Â›Û˘ ·fi Û˘Ó˘¿Ú¯Ô˘Û˜ ηٷÛÙ¿ÛÂȘ, fiˆ˜ ˘ÔÙÚÔÈ¿˙Ô˘Û˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·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 Ôԛ˜ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ·‰˘Ó·Ì›· ÂÈÛfi‰Ô˘ ·¤Ú· ·Ú¿ ÙËÓ ‡·ÚÍË ·Ó·Ó¢ÛÙÈ΋˜ ÚÔÛ¿ıÂÈ·˜ Î·È ‰È·ÎÔ‹ ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ·ÂÚÈÛÌÔ‡ Î·È Ù˘ ·Ú¯ÈÙÂÎÙÔÓÈ΋˜ 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, fiÔ˘ ˘¿Ú¯ÂÈ Î·È Ë ÌÂÁ·Ï‡ÙÂÚË ˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ÙˆÓ ·Ì˘Á‰·ÏÒÓ (2,3). ¶Ú¤ÂÈ Ó· ‰È·ÊÔÚԉȷÁÓˆÛÙ› ·fi ÙÔ ÚˆÙÔ·ı¤˜ ÚÔ¯·ÏËÙfi, ÛÙÔ ÔÔ›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). ªÂϤÙ˜ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ Î·È Ù˘ ‡·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 Ôχ ÌÂÁ·Ï‡ÙÂÚË Î·È Î˘Ì·›ÓÂÙ·È Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ ÌÂٷ͇ 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· ÙÂÏÂ˘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 ÔÔ›· ·Ú·ÙËÚÂ›Ù·È ÛÔ‚·Ú‹ ˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ, Ù‡Ô˘ ππ, ÛÙËÓ ÔÔ›· ˘¿Ú¯ÂÈ ËÈfiÙÂÚË ·fiÊÚ·ÍË Ù˘ ·ÓÒÙÂÚ˘ ·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·¯˘Û·ÚΛ·, Î·È Ù‡Ô˘ πππ, ÛÙËÓ ÔÔ›· ÙÔ ™∞∞À ÔÊ›ÏÂÙ·È Û ÎÚ·ÓÈÔÚÔÛˆÈΤ˜ ·ÓˆÌ·Ï›Â˜ ‹ Ó¢ÚÔÌ˘˚Τ˜ ‰È·Ù·Ú·¯¤˜ (Û‡Ó‰ÚÔÌ· Crouzon, Apert, Teacher Collins, Pierre Robin, Down, ·¯ÔÓ‰ÚÔÏ·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· Â›‰· ·ÚÙËÚȷ΋˜ ›ÂÛ˘, ·‡ÍËÛË Ù˘ C ·ÓÙȉÚÒÛ·˜ ÚˆÙ½Ó˘ (Ë ÔÔ›· Û˘Û¯ÂÙ›˙ÂÙ·È Ì ηډȷÁÁÂȷΤ˜ ÓfiÛÔ˘˜), ·ÓÙ›ÛÙ·ÛË ÛÙËÓ ÈÓÛÔ˘Ï›ÓË Î·È ˘ÂÚÙÚÔÊ›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ (13). ŸÛÔÓ ·ÊÔÚ¿ ÛÙÔÓ Ù‡Ô π (ÌÂÌÔӈ̤ÓË ˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ), ¤¯ÂÈ ‰È·ÈÛÙˆı› fiÙÈ Ë ¯ÚfiÓÈ· ‰È·Ï›Ô˘Û· ˘ÔÍ·ÈÌ›· Î·È ÙÔ ÔÍÂȉˆÙÈÎfi 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 ÂȉÂÈÓÒÓÂÈ ÙÔ ÔÍÂȉˆÙÈÎfi stress Î·È ÙË Û˘ÛÙËÌ·ÙÈ΋ ÊÏÂÁÌÔÓÒ‰Ë ·ÓÙ›‰Ú·ÛË Î·È ÂÈÙ·¯‡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˜ ÂÈÙÒÛÂȘ ÙÔ˘ ™∞∞À ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, Ù· Â·ÓÂÈÏËÌ̤ӷ ÂÂÈÛfi‰È· ÌÂÚÈ΋˜ ‹ Ï‹ÚÔ˘˜ ·fiÊڷ͢ Ù˘ ·ÓÒÙÂÚ˘ ·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡ ÚÔηÏÔ‡Ó ˘Ô·ÂÚÈÛÌfi (˘fiÓÔÈ·) ‹ ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘ ¿ÓÔȘ. √È ÙÂÏÂ˘Ù·›Â˜ ÚÔηÏÔ‡Ó ÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜, ÌÂ Û˘ÓÔ‰fi ˘ÂÚηÓ›· Î·È ·Ó·Ó¢ÛÙÈ΋ ÔͤˆÛË (15).

1) √Í›˜ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÙÒÛÂȘ: √È ·ÔÊÚ·ÎÙÈΤ˜ ¿ÓÔȘ ÚÔηÏÔ‡Ó Â·Ó·Ï·Ì‚·ÓfiÌÂÓ· ÂÂÈÛfi‰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΋ ÂÈÛÙÚÔÊ‹ ·›Ì·ÙÔ˜, ÏfiÁˆ ÂÏ¿ÙÙˆÛ˘ Ù˘ ÎÂÓÙÚÈ΋˜ ÊÏ‚È΋˜ ›ÂÛ˘, Ì Â·ÎfiÏÔ˘ıÔ ÙËÓ ·‡ÍËÛË ÙÔ˘ ÙÂÏԉȷÛÙÔÏÈÎÔ‡ fiÁÎÔ˘ Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜. ªÂ ÙËÓ ·Ê‡ÓÈÛË ‰È·ÎfiÙÂÙ·È Ë ¿ÓÔÈ· Î·È ·˘Í¿ÓÂÙ·È Ë Û˘ÛÙËÌ·ÙÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË Î·È Ë Î·Ú‰È·Î‹ ·ÚÔ¯‹ (15).

2) ÃÚfiÓȘ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÙÒÛÂȘ: ∏ ¯ÚfiÓÈ· ˘ÔÍ·ÈÌ›· ÚÔηÏ› ·ÁÁÂÈÔÛ‡Û·ÛË ÙˆÓ ÎÏ¿‰ˆÓ Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜. ∆· Â·ÓÂÈÏËÌ̤ӷ ÂÂÈÛfi‰È· ·ÁÁÂÈÔÛ‡Û·Û˘ ηٷÛÙÚ¤ÊÔ˘Ó ÙÔ Ó¢ÌÔÓÈÎfi ·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˘Ìو̷ÙÔÏÔÁ›· ÙÔ˘ ™∞∞À ™Â ·Ú¯ÈÎfi ÛÙ¿‰ÈÔ ·Ú·ÙËÚÂ›Ù·È ·‰ÂÓÔÂȉ¤˜ ÚÔÛˆ›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, ¶·È‰È·ÙÚÈ΋ 2009;72:133-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·134

134

∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁÌ¤ÏÔ˘, °. µ·ÚÏ¿Ì˘

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˘ÌÙÒÌ·Ù· ‰ÂÍÈ¿˜ ‹ ·ÚÈÛÙÂÚ‹˜ ηډȷ΋˜ ·ÓÂ¿ÚÎÂÈ·˜ (2,3). ™ËÌ·ÓÙÈ΋ ËÌÂÚ‹ÛÈ· ˘ÓËÏ›· ‰ÂÓ ·Ú·ÙËÚÂ›Ù·È Û˘¯Ó¿ Û ·È‰È¿ Ô˘ ¤¯Ô˘Ó ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘ ¿ÓÔȘ (16). ¶·ÚfiÏ· ·˘Ù¿, ·ÎfiÌË Î·È ·È‰È¿ Ì ·Ïfi ÚÔ¯·ÏËÙfi ÌÔÚ› Ó· ¤¯Ô˘Ó ‰È·Ù·Ú·¯¤˜ Û˘ÌÂÚÈÊÔÚ¿˜ Î·È Ì·ıËÛȷΤ˜ ‰È·Ù·Ú·¯¤˜ (17,18), ÔÈ Ôԛ˜ 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 ÔÔ›· Ô‰ËÁ› Û ·˘ÍË̤Ó˜ ÌÂÙ·‚ÔÏÈΤ˜ ·Ó¿ÁΘ, ÂÓÒ Û ÌÈ· ¿ÏÏË ÌÂϤÙË ıˆڋıËΠˆ˜ Èı·Ófi˜ ÔÚÌÔÓÈÎfi˜ Ì˯·ÓÈÛÌfi˜ (ÂÏ¿ÙÙˆÛË ÙÔ˘ ·Ú¿ÁÔÓÙ· IGF-I) (22). ∞Ó Î·È Ë ÌÂȈ̤ÓË ÛˆÌ·ÙÈ΋ ·Ó¿Ù˘ÍË ‰ÂÓ Â›Ó·È Û˘¯Ó‹ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ù· ·È‰È¿ Ô˘ οÓÔ˘Ó ·‰ÂÓÔÂȉÂÎÙÔÌ‹ Î·È ·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΋ ·ÓÂ¿ÚÎÂÈ· Â›Ó·È Û¿ÓÈ· Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·. ¶·ÚfiÏ· ·˘Ù¿, Û˘¯Ó¿ ˘¿Ú¯ÂÈ ·Û˘Ìو̷ÙÈ΋ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË (3). ¢È¿ÁÓˆÛË ÙÔ˘ ™∞∞À ∂ÎÙfi˜ ·fi ÙÔ ÈÛÙÔÚÈÎfi Î·È ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË, ÔÈ ‰È·ÁÓˆÛÙÈΤ˜ ̤ıÔ‰ÔÈ Ô˘ ÌÔÚÔ‡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˘ ‡·Ú͢ ™∞∞À Â›Ó·È Ë ·Ó›¯Ó¢ÛË ·ÛıÂÓÒÓ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙËÓ ÂΉ‹ÏˆÛË ÛÔ‚·ÚÒÓ ÂÈÏÔÎÒÓ. øÛÙfiÛÔ ÙÔ ÈÛÙÔÚÈÎfi ‰ÂÓ ÌÔÚ› Ó· ‰ÒÛÂÈ ·ÍÈfiÈÛÙ˜ ÏËÚÔÊÔڛ˜ ÁÈ· ÙËÓ ‡·ÚÍË ‹ fi¯È ·ÔÊÚ·ÎÙÈÎÔ‡ 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˘, fiÔ˘ 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 ·ÓÙÈÏËÙ¤˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ (2). ∏ ÔÏÔÓ‡ÎÙÈ· ηٷÁÚ·Ê‹ Ù˘ Ô͢ÌÂÙÚ›·˜ ÌÔÚ› Â›Û˘ Ó· ‰Â›ÍÂÈ ÂÂÈÛfi‰È· ΢ÎÏÈ΋˜ ÙÒÛ˘ ÙÔ˘ ÎÔÚÂÛÌÔ‡, ·ÏÏ¿ ÌÔÚ› Ó· ¤¯ÂÈ „¢‰Ò˜ ıÂÙÈο ·ÔÙÂϤÛÌ·Ù· Û ·È‰È¿ Ì ·¯˘Û·ÚΛ· ‹ ¿ÛıÌ·. ∏ ÌfiÓË Û›ÁÔ˘ÚË ‰È·ÁÓˆÛÙÈ΋ ̤ıÔ‰Ô˜, ÏÔÈfiÓ, Â›Ó·È Ë ÔÏ˘Î·Ù·ÁÚ·ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘, Ë ÔÔ›· ÌÔÚ› Ó· Paediatriki 2009;72:134-000

‰È·ÎÚ›ÓÂÈ ÙÔ ·Ïfi ÚÔ¯·ÏËÙfi ·fi ÙÔ Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ (2). ø˜ ÚÔ˜ ÙÔ fiÙ Ú¤ÂÈ Ó· ıÂÚ·‡ÂÙ·È ¤Ó· ·È‰› Ì ۇӉÚÔÌÔ Ù˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜, ·˘Ùfi Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÔˆÛ‰‹ÔÙ fiÙ·Ó ˘¿Ú¯Ô˘Ó ¿Óˆ ·fi 10 ·ÔÊÚ·ÎÙÈο ·ÓÔ˚ο ÂÂÈÛfi‰È· ·Ó¿ ÒÚ· (23). ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÙÔ˘ ™∞∞À ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· √È ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙËÓ ÂΉ‹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΋˜ ·ÓÂ¿ÚÎÂÈ·˜.

™∞∞À Î·È ˘¤ÚÙ·ÛË ∏ ·fiÊÚ·ÍË Ô‰ËÁ› Û Â·Ó·Ï·Ì‚·ÓfiÌÂÓ· ÂÂÈÛfi‰È· ˘ÂÚÙ·ÛÈÎÒÓ ÎÚ›ÛÂˆÓ Î·Ù¿ ÙÔÓ ‡ÓÔ, Ô˘ Û˘Ó‰˘¿˙ÔÓÙ·È Ì ٷ ·ÓÔ˚ο ÂÂÈÛfi‰È·. ∂›Û˘ ˘¿Ú¯ÂÈ ·˘ÍË̤ÓÔ˜ ΛӉ˘ÓÔ˜ Û˘ÛÙËÌ·ÙÈ΋˜ ˘¤ÚÙ·Û˘ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (24). √È ·ıÔÁÂÓÂÙÈÎÔ› Ì˯·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¯ÔÏ·ÌÈÓÒÓ (25), ‚) ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ·ÁÁÂÈÔÛ˘Û·ÛÙÈÎÒÓ Ô˘ÛÈÒÓ ·fi ÙÔ ÂÓ‰Ôı‹ÏÈÔ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ, fiˆ˜ 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 ·˘ÍË̤ÓË 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¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, Ë ÔÔ›· Â¤ÌÂÓÂ Î·È ÌÂÙ¿ ÙËÓ ¤ÁÂÚÛË. √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 Û 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 ÂȉÂÈÓÔ‡ÌÂÓË ‚Ú·‰˘Î·Ú‰›· ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·ÓÔ˚ÎÔ‡ ÂÂÈÛÔ‰›Ô˘,

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·135

135

∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ

Ô˘ ·ÎÔÏÔ˘ıÂ›Ù·È ·fi Ù·¯˘Î·Ú‰›·. ∂ÈÚfiÛıÂÙ·, ÌÔÚ› Ó· ·Ú·ÙËÚËıÔ‡Ó ¤ÎÙ·ÎÙ˜ ÎÔÈÏȷΤ˜ Û˘ÛÙÔϤ˜, ηډȷΤ˜ ·‡ÛÂȘ >3 sec Î·È 2Ô˘ ‚·ıÌÔ‡ ÎÔÏÔÎÔÈÏÈ·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 Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·ÓÔ˚ÎÔ‡ ÂÂÈÛÔ‰›Ô˘ Î·È Ô‰ËÁÔ‡Ó Û ÈÛ¯·ÈÌ›· ÙÔ˘ Ì˘Ôηډ›Ô˘ (34) Î·È ‚) Ë ¯ÚfiÓÈ· ˘ÔÍ·ÈÌ›· Î·È Ë ˘ÂÚηÓ›·, ÔÈ Ôԛ˜ ÚÔηÏÔ‡Ó ‰È¤ÁÂÚÛË ÙÔ˘ Û˘Ì·ıËÙÈÎÔ‡ ¡™ Î·È ·ÂÏ¢ı¤ÚˆÛË Î·Ù¯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 ÔÔ›· Â›Ó·È ˘‡ı˘ÓË ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ˘ÂÚÙÚÔÊ›·˜ Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È ÙËÓ Î·Ú‰È·Î‹ ·ÓÂ¿ÚÎÂÈ· Ù˘ ‰ÂÍÈ¿˜ (36). ø˜ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË ¯·Ú·ÎÙËÚ›˙ÂÙ·È Ë ·‡ÍËÛË Ù˘ ̤Û˘ ›ÂÛ˘ ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›· >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· Û ϛÁ· ÏÂÙ¿ Î·È Â›Ó·È ·Ó·ÛÙÚ¤„ÈÌË Ì ÙËÓ ¿ÚÛË Ù˘ ·fiÊڷ͢. ∏ ¯ÚfiÓÈ· ÔÊ›ÏÂÙ·È Û ‰ÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ (˘ÂÚÙÚÔÊ›· ÙÔ˘ Ì˘˚ÎÔ‡ ¯ÈÙÒÓ· ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÚÙËÚÈÒÓ Ì¤ÛÔ˘ Î·È 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 ·‡ÍËÛË ÙÔ˘ ηډȷÎÔ‡ ÌÂÙ·ÊÔÚÙ›Ô˘ Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜. ∏ ÙÂÏÂ˘Ù·›· Ô‰ËÁ› Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ Û ˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È Û ‰ÂÍÈ¿ ηډȷ΋ ·ÓÂ¿ÚÎÂÈ· (1). ™ÙË Û˘Ó¤¯ÂÈ·, Â¿Ó ‰ÂÓ ·ÓÙÈÌÂÙˆÈÛÙ› Ë Î·Ù¿ÛÙ·ÛË, ÌÔÚ› Ó· ·ÎÔÏÔ˘ı‹ÛÂÈ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Î·È Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜, Ì ÂÏ¿ÙÙˆÛË ÙÔ˘ fiÁÎÔ˘ ·ÏÌÔ‡ Î·È Û˘ÌÊÔÚËÙÈ΋ ηډȷ΋ ·ÓÂ¿ÚÎÂÈ·. √ Û˘Ó‰˘·ÛÌfi˜ Ù˘ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ Ù˘ ·ÚÈÛÙÂÚ‹˜ Î·È ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ ÌÔÚ› Ó· ÂÈ‚·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·ÈÌ›·˜ ÌÔÚ› Ó· ÚÔÎÏËı› ÌË ·Ó·ÛÙÚ¤„ÈÌË Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË. ∞ÓÙ›ıÂÙ·, Ë Û˘Ó‡·ÚÍË ÛÔ‚·Ú‹˜ Ó˘-

¯ÙÂÚÈÓ‹˜ ˘ÔÍ·ÈÌ›·˜ Ì ‹È· ¤ˆ˜ ̤ÙÚÈ· ËÌÂÚ‹ÛÈ· ˘ÔÍ·ÈÌ›· ‹/Î·È ˘ÂÚηÓ›· ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ ÛÙËÓ ÂÁηٿÛÙ·ÛË Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ (38). ™˘Ìو̷ÙÔÏÔÁ›· ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜ ∆· Û˘ÌÙÒÌ·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 ÂÚÈÙÒÛÂȘ Ô˘ ¤¯ÂÈ Î·Ù·Ï‹ÍÂÈ Û ¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ηډȿ, ÂÌÊ·Ó›˙ÂÙ·È ‰‡ÛÓÔÈ· (·fi ÂÏ¿ÙÙˆÛË Ù˘ ‰È·Ù·ÛÈÌfiÙËÙ·˜ ÙˆÓ Ó¢ÌfiÓˆÓ), ‡ÎÔÏË ÎfiˆÛË (·fi ÂÏ¿ÙÙˆÛË Ù˘ ηډȷ΋˜ ·ÚÔ¯‹˜), Û˘ÁÎÔÈο ÂÂÈÛfi‰È· (·fi ·ÚÔ‰È΋ ÂÁÎÂÊ·ÏÈ΋ ÈÛ¯·ÈÌ›·), ÚÔοډȷ ¿ÏÁË ÛÙËı·Á¯ÈÎÔ‡ ¯·Ú·ÎÙ‹Ú· Î·È ·›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·Î‹ ·ÓÂ¿ÚÎÂÈ· Î·È ¤¯ÂÈ ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ (36). ∫ÏÈÓÈο Â˘Ú‹Ì·Ù· ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜ ∫·Ù¿ ÙËÓ ·ÎÚfi·ÛË Ù˘ ηډȿ˜ ·Ú·ÙËÚÂ›Ù·È ·˘ÍË̤ÓÔ ÙÔ Ó¢ÌÔÓÈÎfi ÛÙÔÈ¯Â›Ô ÙÔ˘ 2Ô˘ ÙfiÓÔ˘, Û˘ÛÙÔÏÈÎfi ʇÛËÌ· ·ÓÂ¿ÚÎÂÈ·˜ Ù˘ ÙÚÈÁÏÒ¯ÈÓ·˜ ‹ ‰È·ÛÙÔÏÈÎfi ʇÛËÌ· ·ÓÂ¿ÚÎÂÈ·˜ Ù˘ Ó¢ÌÔÓÈ΋˜, 3Ô˜ ‹ 4Ô˜ ÙfiÓÔ˜. ∂›Û˘, ÌÔÚ› Ó· ˘¿Ú¯ÂÈ ‰È¿Ù·ÛË ÙˆÓ ÊÏ‚ÒÓ ÙÔ˘ ÙÚ·¯‹ÏÔ˘, Ë·ÙÔÌÂÁ·Ï›· Î·È ÂÚÈÊÂÚÈο 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‡ ÎfiÏÔ˘, ‰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·, fiÔ˘ ˘¿Ú¯ÂÈ ˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È Î·Ú‰È·Î‹ ·ÓÂ¿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˘ ‰ÂÍÈÔ‡ ÎfiÏÔ˘), ˘„ËÏ¿ R ÛÙȘ ‰ÂÍȤ˜ ÚÔοډȘ ··ÁˆÁ¤˜ Î·È ‚·ıÈ¿ S ÛÙȘ ·ÚÈÛÙÂÚ¤˜ ÚÔοډȘ ··ÁˆÁ¤˜ (·fi ˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜). ªÔÚ› Â›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‡ ÎfiÏÔ˘ Î·È ‰È¿Ù·ÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜. ªÂ ÙË ÌÂϤÙË ¶·È‰È·ÙÚÈ΋ 2009;72:135-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·136

136

∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁÌ¤ÏÔ˘, °. µ·ÚÏ¿Ì˘

Doppler ‰È·ÈÛÙÒÓÂÙ·È ·ÓÂ¿ÚÎÂÈ· ÙÚÈÁÏÒ¯ÈÓ·˜ Î·È Ó¢ÌÔÓÈ΋˜ ‚·Ï‚›‰·˜ Î·È ÌÂÙÚ¿Ù·È Ë ›ÂÛË ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›·, Ë ÔÔ›· Â›Ó·È ·˘ÍË̤ÓË (1). ™Â ÈÔ ÚÔ¯ˆÚË̤Ó˜ ηٷÛÙ¿ÛÂȘ ÌÔÚ› ·ÎfiÌË Ó· ·Ú·ÙËÚËı› ‰È·ÛÙÔÏÈ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ ‹/Î·È ˘ÂÚÙÚÔÊ›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ (40,41).

£ÂÚ·›· ÙÔ˘ ™∞∞À ∞‰ÂÓÔÂȉÂÎÙÔÌ‹ Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹ ∏ ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ ·Ú·Ì¤ÓÂÈ Ë ıÂÚ·›· 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 Î·È Berkovitz ÌÂϤÙËÛ·Ó ·Û˘Ìو̷ÙÈο ·È‰È¿ Ì ˘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 ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË (48). ŸÛÔÓ ·ÊÔÚ¿ ÛÙË ÌÂÌÔӈ̤ÓË ·‰ÂÓÔÂȉÂÎÙÔÌ‹, οÔȘ 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˘ Â¤Ì‚·Û˘ Â›Ó·È Ë ‚·Ú‡ÙËÙ· Ù˘ ·fiÊڷ͢. ªÈ· ÌÂϤÙË Ô˘ Û˘ÁÎÚ›ÓÂÈ ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ Â¤Ì‚·Û˘ Ì ÙË ‚·Ú‡ÙËÙ· 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¿ÁÔÓÙ˜ Ô˘ Úԉȷı¤ÙÔ˘Ó Û ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À ÌÂÙ¿ 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 ÌÔÚ› 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 ÂÈÏÔΤ˜ ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË. √È ÂÈÏÔΤ˜ ÌÔÚ› Ó· Â›Ó·È ¿ÌÂÛ˜, fiˆ˜ Âȉ›ӈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ ‹ Ó¢ÌÔÓÈÎfi Ô›‰ËÌ·, Î·È ·ÒÙÂÚ˜, fiˆ˜ ·Ú·ÌÔÓ‹ ·ÚÔ‰ÈÎÔ‡ ΢„ÂÏȉÈÎÔ‡ ˘Ô·ÂÚÈÛÌÔ‡ (Ô˘ ÌÔÚ› Ó· ¯ÚÂÈ·Ûı› ̤¯ÚÈ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ‹ ÙÚ·¯ÂÈÔÛÙÔÌ›·) ‹ Â›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 ÂÈÛٷ̤ÓË ÌÂÙÂÁ¯ÂÈÚËÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜.

Paediatriki 2009;72:136-000

ÕÏϘ ıÂÚ·›˜ ™Â ·È‰È¿ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË Û˘ÓÈÛÙ¿Ù·È Ë ¯Ú‹ÛË ÙÔ˘ ÚÈÓÈÎÔ‡ CPAP. ∂›Û˘, ¤¯ÂÈ ·Ú·ÙËÚËı› ‚ÂÏÙ›ˆÛË ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÂȉÒÓ ÂÓ‰ÔÚÚÈÓÈο Û ·È‰È¿ Ì ÂÏ·ÊÚ¿ ¤ˆ˜ ̤ÙÚÈ· ·fiÊÚ·ÍË Ô˘ ‰ÂÓ ¤¯Ô˘Ó ¯ÂÈÚÔ˘ÚÁËı› (53), ηıÒ˜ Â›Û˘ Û ·È‰È¿ Ì ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À ÌÂÙ¿ ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔÌ‹ 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 ÂÈϤÔÓ ÌÔÚ› Ó· ÂȉÂÈÓÒÛÂÈ ÙÔÓ ˘Ô·ÂÚÈÛÌfi (55). ™˘ÓÈÛÙ¿Ù·È fï˜ Ë ¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ ÛÙÔ ™∞∞À Ù‡Ô˘ ππ, ·Ú¿ÏÏËÏ· Ì ÙÔ ÚÈÓÈÎfi CPAP, ÂÓÒ Ë ¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ >18 ÒÚ˜ ËÌÂÚËÛ›ˆ˜ Û˘ÓÈÛÙ¿Ù·È Û ·ÛıÂÓ›˜ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó Î·È ËÌÂÚ‹ÛÈ· ˘ÔÍ·ÈÌ›· (38). ™Â ηډȷ΋ ·ÓÂ¿ÚÎÂÈ· ÌÔÚ› Â›Û˘ Ó· ¯ÔÚËÁËıÔ‡Ó ‰·ÎÙ˘Ï›Ùȉ· Î·È ‰ÈÔ˘ÚËÙÈο ̤¯ÚÈ ÙËÓ ¿ÚÛË Ù˘ ·fiÊڷ͢ (36). ∆· ·È‰È¿ Ô˘ ¤¯Ô˘Ó ˘ÔÛÙ› ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË ÁÈ· ™∞∞À Ú¤ÂÈ Ó· Â·ÓÂÎÙÈÌËıÔ‡Ó ÌÂÙ¿ ·fi 6-8 ‚‰ÔÌ¿‰Â˜ ÁÈ· Ó· Ê·Ó› Â¿Ó ¤¯Ô˘Ó ‚ÂÏÙȈı› ‹ ¯ÚÂÈ¿˙ÔÓÙ·È ÂÈÚfiÛıÂÙË ıÂÚ·›·, fiˆ˜ ÙÔ ÚÈÓÈÎfi CPAP (1,2).

µÈ‚ÏÈÔÁÚ·Ê›· 1. Blum RH, McGowan FX Jr. Chronic upper airway obstruction and cardiac dysfunction: anatomy, pathophysiology and anesthetic implications. Paediatr Anaesth 2004;14:75-83.

2. American Academy of Pediatrics. Clinical Practice Guideline: Diagnosis and Management of Childhood Obstructive Sleep Apnea Syndrome. Pediatrics 2002;109: 704-712.

3. Marcus CL. Sleep-disordered breathing in children. Am J Respir Crit Care Med 2001;164:16-30.

4. Laurikainen E, Erkinjuntti M, Alihanka J, Rikalainen H, Suonpaa J. Radiological parameters of the bony nasopharynx and the adenotonsillar size compared with sleep apnea episodes in children. Int J Pediatr Otorhinolaryngol, 1987;12:303-310.

5. Brooks LJ, Stephens BM, Bacevice AM. Adenoid size is related to severity but not the number of episodes of obstructive apnea in children. J Pediatr 1998;132:682-86.

6. Ali NJ, Pitson DJ, Stradling JR. Snoring, sleep disturbance and behaviour in 4-5 years olds. Arch Dis Child 1993;68: 360-366.

7. Gislason T, Benediktsdottir B. Snoring, apneic episodes and nocturnal hypoxemia among children 6 months to 6 years old. An epidemiologic study of lower limit of prevalence. Chest 1995;107:963-966.

8. Lipton AJ, Gozal D. Treatment of obstructive sleep apnea in children: do we really know how? Sleep Med Rev 2003; 7:61-80.

9. Hultcrantz E, Lofstrand-Tidestrom B, Ahlquist-Rastad J.

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·137

137

∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ

The epidemiology of sleep related breathing disorder in children. Int J Pediatr Otorhinolaryngol 1995;32(suppl): S63-S66.

10. Owen GO, Canter RJ, Robinson A. Snoring, apnoea and ENT symptoms in the pediatric community. Clin Otolaryngol Allied Sci 1996;21:130-134.

11. Ferreira AM, Clemente V, Gozal D, Gomes A, Pissara C, Cesar H, et al. Snoring in Portuguese primary school children. Pediatrics 2000;106:E64.

12. Capdevila OS, Kheirandish-Gozal L, Dayyat E, Gozal D. Pediatric obstructive sleep apnea: complications, management and long-term outcomes. Proc Am Thorac Soc 2008; 5:274-282.

13. Ievers-Landis CE, Redline S. Pediatric sleep apnea: implications of the epidemic of childhood overweight. Am J Respir Crit Care Med 2007;175:436-441.

14. Redline S, Storfer-Isser A, Rosen CL, Johnson NL, Kirchner HL, Emancipator J, et al. Association between metabolic syndrome and sleep-disordered breathing in adolescents. Am J Respir Crit Care Med 2007;176:401-408.

15. Mansfield DR, Naughton MT. Sleep apnea and congestive heart failure. Minerva Med 2004;95:257-280.

16. Gozal D, Wang M, Pope DW Jr. Objective sleepiness measures in pediatric obstructive sleep apnea. Pediatrics 2001;108:693-697.

17. Chervin RD, Dillon JE, Bassetti C, Ganoczy D, Pituch KJ. Symptoms of sleep disorders, inattention and hyperactivity in children. Sleep 1997;20:1185-1192.

18. Owens J, Opipari L, Nobile C, Spirito A. Sleep and daytime behavior in children with obstructive sleep apnea and behavioral sleep disorders. Pediatrics 1998;102:1178-1184.

19. Ali NJ, Pitson D, Stradling JR. Sleep disordered breathing: effects of adenotonsillectomy on behaviour and psychological functioning. Eur J Pediatr 1996;155:56-62.

20. Gozal D. Sleep-disordered breathing and school performance in children. Pediatrics 1998;102:616-620.

21. Marcus CL, Carroll JL, Koerner CB, Hamer A, Lutz J, Loughlin GM. Determinants of growth in children with the obstructive sleep apnea syndrome. J Pediatr 1994;125: 556-562.

22. Bar A, Tarasiuk A, Segev Y, Phillip M, Tal A. The effect of adenotonsillectomy on serum insulin-like growth factor-I and growth in children with obstructive sleep apnea syndrome. J Pediatr 1999;135:76-80.

23. Marcus CL, Omlin KJ, Basinski DJ, Bailey SL, Rachal AB, Von Pechmann WS. Normal polysomnographic values for children and adolescents. Am Rev Resp Dis 1992;146:12351239.

24. Bixler EO, Vgontzas AN, Lin HM, Ten Have T, Leiby BE, Vela-Bueno A, et al. Association of hypertension and sleepdisordered breathing. Arch Intern Med 2000;160:2289-2295.

25. Somers VK, Dyken ME, Clary MP, Abboud FM. Sympathetic neural mechanisms in obstructive sleep apnea. J Clin Invest 1995;96:1897-1904.

26. Phillips BG, Narkiewich K, Pesek CA, Haynes WG, Dyken ME, Somers VK. Effects of obstructive sleep apnea on endothelin-1 and blood pressure. J Hypertens 1999;17:61-66.

27. Quan SF, Gersh BJ. Cardiovascular consequences of sleepdisordered breathing: past, present and future. Report of a workshop from the national center on sleep disorders research and the national heart, lung and blood institute.

Circulation 2004,109:951-957.

28. Peled N, Greenberg A, Pillar G, Zinder O, LevI N, Lavie P. Contributions of hypoxia and respiratory disturbance index to sympathetic activation and blood pressure in obstructive sleep apnea syndrome. Am J Hypertens 1998;11:1284-1289.

29. Fletcher EC. Effect of episodic hypoxia on sympathetic activity and blood pressure. Respir Physiol 2000;119: 189-197.

30. Marcus CL, Green MG, Carroll JL. Blood pressure in children with obstructive sleep apnea. Am J Respir Crit Care Med 1998;157:1098-1103.

31. Amin RS, Carroll JL, Jeffries JL, Grone C, Bean JA, Chini B, et al. Twenty-four-hour ambulatory blood pressure in children with sleep disordered breathing. Am J Respir Crit Care Med 2004;169:950-956.

32. Amin RS, Daniels S, Kimball T, Willging P, Cotton R. Echocardiographic changes in children with obstructive sleep apnea. Sleep 2000;23:A99.

33. Gula LJ, Krahn AD, Skanes AC, Yee R, Klein GJ. Clinical relevance of arrhythmias during sleep: guidance for clinicians. Heart 2004;90:347-352.

34. Leung RS, Bradley TD. Sleep apnea and cardiovascular disease. Am J Respir Crit Care Med 2001;164:2147-2165.

35. Ziegler MG, Mills PJ, Loredo JS, Ancoli-Israel S, Dimsdale JE. Effect of continuous positive airway pressure and placebo treatment on sympathetic nervous activity in patients with obstructive sleep apnea. Chest 2001;120:887-893.

36. Noonan JA. Cor pulmonale. In Chernick V, Boat TF, editors. Kendig’s Disorders of the Respiratory Tract in Children, 6th ed. Philadelphia, Pensylvania: WB Saunders Company; 1998. p. 826.

37. Widlitz A, Barst RJ. Pulmonary arterial hypertension in children. Eur Respir J 2003,21:155-176.

38. Kessler R, Chaouat A, Weitzenblum E, Oswald M, Ehrhart M, Apprill M, et al. Pulmonary hypertension in the obstructive sleep apnoea syndrome: prevalence, causes and therapeutic consequences. Eur Respir J 1996;9:787-794.

39. Pac A, Karadag A, Kurtaran J, Aktas D. Comparison of cardiac function and valvular damage in children with and without adenotonsillar hypertrophy. Int J Pediatr Otorhinolaryngol 2005;69:527-532.

40. Amin RS, Kimball TR, Bean JA, Jeffries JL, Willging JP, Cotton RT, et al. Left ventricular hypertrophy and abnormal ventricular geometry in children and adolescents with obstructive sleep apnea. Am J Respir Crit Care Med 2002;165:1395-1399.

41. Amin RS, Kimball TR, Kalra M, Jeffries JL, Carroll JL, Bean JA, et al. Left ventricular function in children with sleepdisordered breathing. Am J Cardiol 2005;95:801-804.

42. Zucconi M, Strambi LF, Pestalozza G, Tessitore E, Smirne S. Habitual snoring and obstructive sleep apnea syndrome in children: effects of early tonsil surgery. Int J Pediatr Otorhinolaryngol 1993;26:235-243.

43. Nieminen P, Tolonen U, Lopponen H. Snoring and obstructive sleep apnea in children: a 6-month follow-up study. Arch Otolaryngol Head Neck Surg 2000;126: 481-486.

44. Shintani T, Asakura K, Kataura A. The effect of adenotonsillectomy in children with OSA. Int J Pediatr Otorhinolaryngol 1998;44:51-58.

45. Gorur K, Doven O, Unal M, Akkus N, Ozcan C. Preoperative and postoperative cardiac and clinical ¶·È‰È·ÙÚÈ΋ 2009;72:137-000

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findings of patients with adenotonsillar hypertrophy. Int J Pediatr Otorhinolaryngol 2001;59:41-46.

46. Jain A, Sahni JK. Polysomnographic studies in children undergoing adenoindectomy and/or tonsillectomy. J Laryngol Otol 2002;116:711-715.

47. Tal A, Bar A, Leiberman A, Tarasiuk A. Sleep characteristics following adenotonsillectomy in children with obstructive sleep apnea syndrome. Chest 2003;124:948-953.

48. Sdralis T, Berkowitz RG. Early adenotonsillectomy for relief of acute upper airway obstruction due to acute tonsillitis in children. Int J Pediatr Otorhinolaryngol 1996;35:25-29.

49. Nieminen P, Tolonen U, Lopponen H, Lopponen T, Luotonen J, Jokinen K. Snoring children: factors predicting sleep apnea. Acta Otolaryngol Suppl 1997;529:190-194.

50. Suen JS, Arnold JE, Brooks LJ. Adenotonsillectomy for treatment of obstructive sleep apnea in children. Arch Otolaryngol Head Neck Surg 1995;121:525-530.

51. Redline S, Tishler PV, Schluchter M, Aylor J, Clark K,

Paediatriki 2009;72:138-000

Graham G. Risk factors for sleep-disordered breathing in children. Associations with obesity, race and respiratory problems. Am J Respir Crit Care Med 1999;159:15271532.

52. Rosen GM, Muckle RP, Mahowald MW, Goding GS, Ulleviq C. Postoperative respiratory compromise in children with obstructive sleep apnea syndrome: can it be anticipated? Pediatrics 1994;93:784-788.

53. Alexopoulos EI, Kaditis AG, Kalampouka E, Kostadima E, Angelopoulos NV, Mikraki V, et al. Nasal corticosteroids for children with snoring. Pediatr Pulmonol 2004;38:161-167.

54. Kheiradish L, Goldbart AD, Gozal D. Intranasal steroids and oral leukotriene modifier therapy in residual sleepdisordered breathing after tonsillectomy and adenoidectomy in children. Pediatrics 2006;117:e61-e66.

55. Marcus CL, Carroll JL, Bamford O, Pyzik P, Loughlin GM. Supplemental oxygen during sleep in children with sleepdisordered breathing. Am J Respir Crit Care Med 1995; 152:1297-1301.

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PRACTICAL ISSUE

139

√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 ÌÂÁ¿ÏÔ ‚·ıÌfi ÙÔÓ ·È‰›·ÙÚÔ Û‹ÌÂÚ·. √È ‰ÈÂıÓ›˜ ÔÚÈÛÌÔ› Ù˘ ·¯˘Û·ÚΛ·˜, Ô˘ ‚·Û›˙ÔÓÙ·È Î·Ù¿ ·ÚÈÔ ÏfiÁÔ ÛÙÔÓ ¢Â›ÎÙË ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™) Î·È ÛÙ· ÔÚȷο ÛËÌ›· ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙÔ˘˜ ÂÓ‹ÏÈΘ, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û ÂȉËÌÈÔÏÔÁÈΤ˜ 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 ÙÔÔı¤ÙËÛË Ù˘ ÙÈÌ‹˜ ÙÔ˘ ¢Â›ÎÙË ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™) ÛÙ· ÈÔ Û‡Á¯ÚÔÓ· ·ÓıÚˆÔÌÂÙÚÈο ÚfiÙ˘· ‰È·ÁÚ¿ÌÌ·Ù· ÙÔ˘ ¢ª™ ·Ó¿ ʇÏÔ Î·È ·Ó¿ ¯ÒÚ·. ∏ ÚÔ¤ÎÙ·ÛË ÙˆÓ ÔÚÈ·ÎÒÓ ÛËÌ›ˆÓ ÙÔ˘ ˘¤Ú‚·ÚÔ˘ Î·È ÙÔ˘ ·¯‡Û·ÚÎÔ˘ ÁÈ· ÙÔ˘˜ ÂÓ‹ÏÈΘ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë Î·Ù¿Ù·ÍË ÙˆÓ ·È‰ÈÒÓ ÂÏÏËÓÈ΋˜ ηٷÁˆÁ‹˜, ÂÓÒ ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÔÛÔÛÙÔ‡ ·¯˘Û·ÚΛ·˜ ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 18 ¯ÚfiÓˆÓ, Û ¤Ó· Û˘ÁÎÂÎÚÈ̤ÓÔ ÏËı˘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·.

π·ÙÚÂ›Ô ¶·È‰È΋˜ & ∂ÊË‚È΋˜ ¶·¯˘Û·ÚΛ·˜, ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∏ ∞Á›· ™ÔÊ›·» AÏÏËÏÔÁÚ·Ê›·: ¶·Ó·ÁÈÒÙ· ¶ÂÚ‚·Ó›‰Ô˘ ppervanid@med.uoa.gr ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∏ ∞Á›· ™ÔÊ›·», ∞ı‹Ó·

§¤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 those individuals with excessive adiposity. However, the accurate estimation of body fat content and of the consequent health implications is not an easy task. International definitions of childhood obesity, based on Body Mass Index (BMI) and adult cut-off points, which are used in large epidemiologic studies are less valuable in clinical practice.

Definitions dependent upon a reference population, based on the concept of a certain level of deviation from 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 the same patient and a comparison measure of obesity status between groups.

Key words: Obesity, child, body mass index, percentiles, z-score.

∏ ·¯˘Û·ÚΛ· ÁÂÓÈο ÔÚ›˙ÂÙ·È ˆ˜ Ë ˘ÂÚ‚ÔÏÈ΋ Û˘ÛÛÒÚ¢ÛË Ï›Ô˘˜ ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi, Û ‚·ıÌfi Ô˘ ÂËÚ¿˙ÂÙ·È Ë ˘Á›· ÙÔ˘ ·ÙfiÌÔ˘ (1). ∏ ÔÛÔÙÈÎÔÔ›ËÛË, fï˜, ÙÔ˘ ÏÈÒ‰Ô˘˜ ÈÛÙÔ‡, ηıÒ˜ Î·È Ô ‚·ıÌfi˜ Â›‰Ú·Û˘ Ù˘ ÂÚ›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¤ÙÚËÛ˘ Ù˘ Ì¿˙·˜ ÙÔ˘ ÏÈÒ‰Ô˘˜ ÈÛÙÔ‡, fiˆ˜ Ë Ì¤ÙÚËÛË Ù˘ ۈ̷ÙÈ΋˜ ˘ÎÓfiÙËÙ·˜ οو ·fi ÙÔ ÓÂÚfi, Ë ÂÚÈÂÎÙÈÎfiÙËÙ· ۈ̷ÙÈÎÔ‡ Ï›Ô˘˜ fiˆ˜ ÂÎÙÈÌ¿Ù·È Ì¤Ûˆ ÙÔ˘ ·ÔÚÚÔÊËÛÈÔÌÂÙÚËÙ‹ ‰ÈÏ‹˜ ÂÓ¤ÚÁÂÈ·˜ ·ÎÙ›ÓˆÓ Ã (dual-energy Xry absorptiometer, DEXA), ηıÒ˜ Î·È ÔÈ Ó¤Â˜

Childhood and Adolescence Obesity Clinic, First Department of Pediatrics, Athens University Medical School, “Aghia Sophia” Children’s Hospital, Athens, Greece Correspondence: Panagiota Pervanidou ppervanid@med.uoa.gr Unit for Developmental and Behavioral Paediatrics, First Department of Paediatrics, Athens University Medical School, “Aghia Sophia” Children’s Hospital, Athens, Greece

Ù¯ÓÈΤ˜, fiˆ˜ Â›Ó·È Ë ·ÂÈÎfiÓÈÛË Ì·ÁÓËÙÈÎÔ‡ Û˘ÓÙÔÓÈÛÌÔ‡ (ªagnetic Resonance Imaging, MRI) Î·È Ë ˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›· (Computed Tomography, CT), Â¤ÙÚ„·Ó Ó· ÂÚÈÁÚ·Ê› Ì ·ÎÚ›‚ÂÈ· Ô ÏÈ҉˘ ÈÛÙfi˜, ·ÏÏ¿ ·ÔÙÂÏÔ‡Ó ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙÔ˘˜ ‰··ÓËÚ¤˜ Î·È ¯ÚÔÓÔ‚fiÚ˜ ÂÍÂÙ¿ÛÂȘ, ÁÂÁÔÓfi˜ Ô˘ ÂÚÈÔÚ›˙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 ¯ÚËÛÈÌÔÔÈËı› ‰Â›ÎÙ˜ ‚¿ÚÔ˘˜ ‹ Ì¿˙·˜ ÛÒÌ·ÙÔ˜, ÔÚÈ˙fiÌÂÓÔÈ ˆ˜ ‰È¿ÊÔÚÔÈ Û˘Ó‰˘·ÛÌÔ› ÙÔ˘ ‚¿ÚÔ˘˜ ¶·È‰È·ÙÚÈ΋ 2009;72:23-28

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·140

140

¶. ¶ÂÚ‚·Ó›‰Ô˘

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˘ ÙÂÙÚ·ÁÒÓÔ˘ ÙÔ˘ ‡„Ô˘˜ Û ̤ÙÚ· (m2), ˆ˜ ¤Ó· ·Ïfi Î·È ·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 Ï›Ô˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ (3,4). √ ¢ª™ ¯ÚËÛÈÌÔÔÈÂ›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·ÓÔÓÈÎÔ‡ ‚¿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›Ù ̤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˜ ¯ÒÚ˜ ‚·Û›˙ÔÓÙ·È ÛÂ Û˘Á¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ ÙÔÌ‹˜ (cross sectional) ·ÓÙÈÚÔÛˆ¢ÙÈÎÔ‡ ‰Â›ÁÌ·ÙÔ˜ ·È‰ÈÒÓ, ·fi ÙË Á¤ÓÓËÛË ˆ˜ ÙËÓ ÂÓËÏÈΛˆÛË. ∆· ÂÚÈÛÛfiÙÂÚ· 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 ÔÔ›Ô ¤Ó· ·È‰› ‹ ¤ÊË‚Ô˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ˘¤Ú‚·ÚÔ˜ Î·È ÙËÓ 95Ë (‹ 97Ë) ∂£ ˆ˜ ÙÔ fiÚÈÔ ¿Óˆ ·fi ÙÔ ÔÔ›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 ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Ù· ÏËÛȤÛÙÂÚ· ‰È·ı¤ÛÈÌ·. ∞˘Ùfi˜ Ô ÙÚfiÔ˜ 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˘ ÌÂÏÂٿٷÈ. √ ̤ÛÔ˜ ·˘Ùfi˜ fiÚÔ˜, fï˜, ÌÔÚ› Ó· ÔÈΛÏÏÂÈ Û ‰È¿ÊÔÚ˜ ¯ÒÚ˜, ηıÒ˜ Î·È ÛÙËÓ ›‰È· ¯ÒÚ·, fiÙ·Ó Ì ÙËÓ ¿ÚÔ‰Ô ÙˆÓ ¯ÚfiÓˆÓ ÌÂÙ·‚¿ÏÏÔÓÙ·È Ù· ·ÓıÚˆÔÌÂÙÚÈο ¯·Ú·ÎÙËÚÈÛÙÈο ·˘Ù‹˜. ∏ ¯Ú‹ÛË ÙˆÓ ‰È·ÁÚ·ÌÌ¿ÙˆÓ ∂£ ÂÈÙÚ¤ÂÈ Â›Û˘ ÙÔÓ ¿ÌÂÛÔ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ ËÏÈΛ·˜ ·Ó·ÛÙÚÔPaediatriki 2009;72:140-000

Ê‹˜ ÙÔ˘ ¢ª™ (adiposity rebound), ‰ËÏ·‰‹ Ù˘ ËÏÈΛ·˜ ‰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 ÔχÏÔÎË Ì¤ıÔ‰Ô˜ ÁÈ· ÙË Ì¤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 ÔÛÔÙÈÎÔÔ›ËÛ˘ ÙÔ˘ ¢ª™ Î·È Û˘ÓÈÛÙ¿Ù·È Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È Û ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ Ô˘ ··ÈÙÔ‡Ó ÙË Û‡ÁÎÚÈÛË ÔÌ¿‰ˆÓ Ô˘ ÚÔ¤Ú¯ÔÓÙ·È fï˜ ·fi ÙÔÓ ›‰ÈÔ ÏËı˘ÛÌfi (¤¯Ô˘Ó ÂÎÙÈÌËı› Ì ‚¿ÛË ÙȘ ›‰È˜ ηÌ‡Ï˜) ‹ Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙËÓ ÈÔ ·ÎÚÈ‚‹ ·Ú·ÎÔÏÔ‡ıËÛË ·ÛıÂÓÒÓ Ô˘ Â›Ó·È Û ÚfiÁÚ·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›˙ÂÙ·È ÛÙËÓ ›‰È· ȉ¤·: ·fiÛÙ·ÛË ·fi ÙË Ì¤ÛË ÙÈÌ‹ ÂÓfi˜ Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ÏËı˘ÛÌÔ‡, ÌÈ· Û˘ÁÎÂÎÚÈ̤ÓË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ Ë ÚÔÛ¤ÁÁÈÛË ·˘Ù‹ ‰ÂÓ ÌÔÚ› Ó· ‰Â›ÍÂÈ ÙËÓ ·‡ÍËÛË ÙÔ˘ ÂÈÔÏ·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·ÊÔÚ¿˜ ‰È·Ê¤ÚÔ˘Ó ÌÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ ¯ˆÚÒÓ Î·È ¿Ú· ÔÈ ÌÂÙÚ‹ÛÂȘ ÙÔ˘ z-score, fiˆ˜ 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 ÂÓËÏ›ÎˆÓ ∏ ·Ó¿ÁÎË ÁÈ· ÂÓÔÔ›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 ForceIOTF) 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΋˜ Î·È ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜. ∂›Û˘, Ì ‚¿ÛË ÙÔÓ ÔÚÈÛÌfi

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·141

141

√ÚÈÛÌfi˜ Ù˘ ·È‰È΋˜ Î·È ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜

¶›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 ÙȘ ÔÚȷΤ˜ ÙÈ̤˜ ¢ª™ ÙˆÓ 25 kg/m2 Î·È ÙˆÓ 30 kg/m2 Ô˘ ¤¯Ô˘Ó ÔÚÈÛı› ÁÈ· ÙÔ˘˜ ÂÓ‹ÏÈΘ (11). ∏ÏÈΛ· (¯ÚfiÓÈ·) 2 2,5 3 3,5 4 4,5 5 5,5 6 6,5 7 7,5 8 8,5 9 9,5 10 10,5 11 11,5 12 12,5 13 13,5 14 14,5 15 15,5 16 16,5 17 17,5 18

À¤Ú‚·ÚoÈ ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ· 18,4 18,1 17,9 17,7 17,6 17,5 17,4 17,5 17,6 17,7 17,9 18,2 18,4 18,8 19 19,5 19,8 20,2 20,6 20,9 21,2 21,6 21,9 22,3 22,6 23 23,3 23,6 23,9 24,2 24,5 24,7 25

18,0 17,8 17,6 17,4 17,3 17,2 17,2 17,2 17,3 17,5 17,8 18,0 18,4 18,7 19,1 19,5 19,9 20,3 20,7 21,2 21,7 22,1 22,6 23,0 23,3 23,7 23,9 24,2 24,4 24,5 24,7 24,9 25,0

¶·¯‡Û·ÚÎÔÈ ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ· 20,1 19,8 19,6 19,4 19,3 19,3 19,3 19,5 19,8 20,2 20,6 21,1 21,6 22,2 22,8 23,4 24,0 24,6 25,1 25,6 26,0 26,4 26,8 27,3 27,6 28,0 28,3 28,6 28,9 29,1 29,4 29,7 30,0

19,8 19,6 19,4 19,2 19,2 19,1 19,2 19,3 19,7 20,1 20,5 21,0 21,6 22,2 22,8 23,5 24,1 24,8 25,4 26,1 26,7 27,2 27,8 28,2 28,6 28,9 29,1 29,3 29,4 29,6 29,7 29,8 30,0

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· ÔÔ›· ÚԤ΢„·Ó ·fi Ù· ‰Â‰Ô̤ӷ ¤ÍÈ ¯ˆÚÒÓ (µÚ·˙ÈÏ›·, ªÂÁ¿ÏË µÚÂÙ·Ó›·, Hong Kong, √ÏÏ·Ó‰›·, ™ÈÁηÔ‡ÚË Î·È ∏ӈ̤Ó˜ ¶ÔÏÈÙ›˜ ∞ÌÂÚÈ΋˜). ∆· ÎÚÈÙ‹ÚÈ· ÙÔ˘ Cole ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1. ªÂ ÙÔÓ ÙÚfiÔ ·˘Ùfi, Ù· ÔÚȷο ÛËÌ›· ÔÚÈÛÌÔ‡ Ù˘ ·È‰È΋˜ ·¯˘Û·ÚΛ·˜ ‚·Û›˙ÔÓÙ·È ÛÙÔÓ ˘„ËÏfi ΛӉ˘ÓÔ ÓÔÛËÚfiÙËÙ·˜ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ Î·È fi¯È ÛÙÔÓ Ì¤ÛÔ fiÚÔ ÙÔ˘ ˘fi ÌÂϤÙË ÏËı˘ÛÌÔ‡, ÁÂÁÔÓfi˜ Ô˘ ÂÈÙÚ¤ÂÈ Î·È ÙË 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΋˜ Î·È ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜ ÛÂ Û˘ÁÎÚÈÙÈΤ˜ ÂȉËÌÈÔÏÔÁÈΤ˜ 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 ÌÔÚ› Ó· ‰È·¯ˆÚ›ÛÂÈ ÙË ÏÈÒ‰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 ÂÚÈÛÛfiÙÂÚÔ, 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˜. ∂ÈÚfiÛıÂÙ·, Ë ËÏÈΛ· ¤Ó·Ú͢ Ù˘ ÂÊ˂›·˜ Î·È ÔÈ Û¯ÂÙÈ˙fiÌÂÓ˜ Ì ·˘Ù‹ ÌÂÙ·‚ÔϤ˜ ÔÈΛÏÏÔ˘Ó ÌÂٷ͇ ‰È·ÊfiÚˆÓ ÂıÓÔÏÔÁÈÎÒÓ 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 ÂÈÏÔÎÒÓ Û¯ÂÙÈ˙fiÌÂÓˆÓ Ì 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¤˜ ˆ˜ ¤Ó·˜ ÂÚÈÛÛfiÙÂÚÔ ·ÍÈfiÈÛÙÔ˜ ‰Â›ÎÙ˘ ÙÔ˘ ÂÈ¤‰Ô˘ ÙÔ˘ ÂÓ‰ÔÎÔÈÏÈ·ÎÔ‡ Ï›Ô˘˜ Î·È ·ÔÙÂÏ› ·Ú¿ÁÔÓÙ· ηډÈÔÌÂÙ·‚ÔÏÈÎÔ‡ ÎÈÓ‰‡ÓÔ˘ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ. ∏ ÛÔ˘‰·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· √ÌÔÛÔÓ‰›· ¢È·‚‹ÙË (International Diabetes Federation, IDF) (15). AÎfiÌË ÂÚÈÛÛfiÙÂÚÔ, Ë ¯Ú‹ÛË Ù˘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘, ·ÓÙ› ÙÔ˘ ¢ª™, ÂÂÎÙ¿ıËΠÚfiÛÊ·Ù· ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ·, Ì ·ÔÙ¤ÏÂÛÌ· ÙË Û‡ÌʈÓË ‰‹ÏˆÛË (consensus statement) ÂÚ¢ÓËÙÒÓ Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÛÙËÓ ·È‰È΋ ·¯˘Û·ÚΛ· Ë ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ ≥90˘ ∂£, ·ÓÙ› ÙÔ˘ ¢ª™, ÛÙÔÓ ÔÚÈÛÌfi ÙÔ˘ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ÛÙ· ·È‰È¿, Û‡Ìʈӷ Ì ÙËÓ ÚfiÙ·ÛË ÙÔ˘ πDF (16). ¶·ÚfiÙÈ ‰ÂÓ Â›Ó·È ··ÁÔÚ¢ÙÈÎfi Ó· ¯ÚËÛÈÌÔÔÈ‹ÛÂÈ Î·Ó›˜ ÙȘ ηÌ‡Ï˜ Ô˘ ¤¯Ô˘Ó ·Ó·Ù˘¯ı› Û ¿ÏϘ ¯ÒÚ˜, Ë ÂÈÏÔÁ‹ ·˘Ù‹ Ú¤ÂÈ Ó· Â›Ó·È Ë ÙÂÏÂ˘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· ¶·È‰È·ÙÚÈ΋ 2009;72:141-000

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·142

142

¶. ¶ÂÚ‚·Ó›‰Ô˘

ηÌ‡Ï˜ ÁÈ· ÙËÓ ÂÚ›ÌÂÙÚÔ Ì¤Û˘ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ù˘ ∫Ú‹Ù˘ (17). ™ÙÔ ¿ÚıÚÔ ·˘Ùfi, ·Ú¤¯ÔÓÙ·È ›Ó·Î˜ Ì ÙȘ ∂£ ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, Ô˘ ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó Û ÎÏÈÓÈ΋ ¯Ú‹ÛË, Ì·˙› Ì ÙÔÓ ¢ª™, ÁÈ· ·È‰È¿ Ù˘ ∫Ú‹Ù˘. ¶·Ú¤¯ÂÙ·È Â›Û˘ ›Ó·Î·˜ Ì ÙË Ì¤ÛË ÙÈÌ‹ Î·È ÙË ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, Ô˘ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙÔÓ ÈÔ ·ÎÚÈ‚‹ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ zscore 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·ÙÔÙ˘¯ÒÓ ·ÔÙÂÏ› ÌÈ· ·Ï‹ ̤ıÔ‰Ô ·ÍÈÔÏfiÁËÛ˘ Ù˘ ηٷÓÔÌ‹˜ ÙÔ˘ Ï›Ô˘˜, ÁÈ·Ù› ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› Û ÔÏÏ¿ ̤ÚË ÙÔ˘ ÛÒÌ·ÙÔ˜. ∏ ̤ÙÚËÛË Ù˘ ‰ÂÚÌ·ÙÔÙ˘¯‹˜ ÙÔ˘ ‰ÈÎÂÊ¿ÏÔ˘ ıˆÚÂ›Ù·È fiÙÈ Û¯ÂÙ›˙ÂÙ·È Î·Ï¿ Ì ÙË ÏÈÒ‰Ë Ì¿˙·. ∏ ̤ıÔ‰Ô˜ ·˘Ù‹ ÌÔÚ› 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¤˜ Î·È ‰ÂÓ ıˆÚÂ›Ù·È ·ÎÚÈ‚‹˜ (18). √ ÏfiÁÔ˜ Ù˘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘ ÚÔ˜ ÙËÓ ÂÚ›ÌÂÙÚÔ ÁÔÊÒÓ (Waist to Hip Ratio, W/H) ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙËÓ ·ÍÈÔÏfiÁËÛË Ù˘ ηٷÓÔÌ‹˜ ÙÔ˘ ۈ̷ÙÈÎÔ‡ Ï›Ô˘˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ. ∏ ¯ÚËÛÈÌfiÙËÙ· ÙÔ˘ ‰Â›ÎÙË ·˘ÙÔ‡ Î·È Ë Û¯¤ÛË ÙÔ˘ Ì ÙÔÓ ÌÂÏÏÔÓÙÈÎfi ηډȷÁÁÂÈ·Îfi ΛӉ˘ÓÔ ‰ÂÓ Â›Ó·È ÍÂοı·Ú˜ ÛÙËÓ ·È‰È΋ ËÏÈΛ·. ∂›Û˘, Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÎÙÈÌËıÔ‡Ó ÌÂÙ·‚ÔϤ˜ ÛÙÔ˘˜ ‰Â›ÎÙ˜ ·˘ÙÔ‡˜ ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ·, ÏfiÁˆ ÙˆÓ ·ÏÏ·ÁÒÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó Î·Ù¿ ÙËÓ ·‡ÍËÛË Î·È ÙËÓ ÂÊ˂›·. ∆¤ÏÔ˜, ‰ÂÓ ¤¯Ô˘Ó ·Ó·Ù˘¯ı› ηÌ‡Ï˜ ÁÈ· ÙËÓ ÂÚ›ÌÂÙÚÔ ÁÔÊÒÓ ‹ ÁÈ· ÙÔÓ ÏfiÁÔ W/H ÛÙ· ·È‰È¿ (18). √È ÔÚÈÛÌÔ› Â‰Ò Î·È ÙÒÚ·: ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ 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 Û Â›Â‰Ô Ì·˙È΋˜ ·Ó›¯Ó¢Û˘ ÙˆÓ ·È‰ÈÒÓ Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ηډÈÔÌÂÙ·‚ÔÏÈΤ˜ ÂÈÏÔΤ˜ fiÛÔ Î·È ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÌÂÌÔÓˆÌ¤ÓˆÓ ·ÛıÂÓÒÓ. ™ÙË ÌÂϤÙË ·˘Ù‹ ÌÂÙÚ‹ıËÎ·Ó 10.925 ¿ÙÔÌ· 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 Paediatriki 2009;72:142-000

ηχÙÂÈ 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 ÈηÓÔÔÈËÙÈο ·ÓÙÈÚÔÛˆ¢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 ∂£ Û ·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¤ÛÂˆÓ ·˘ÙÒÓ ÚÈÓ 20 ÂÚ›Ô˘ ¤ÙË. ŒÙÛÈ, Ë ¯ÚËÛÈÌÔÔ›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 ÛÎÂÙÈÎfi Ù˘ ·Ó›¯Ó¢Û˘ ÂÚÈÛÛfiÙÂÚˆÓ ·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 (∂ÈÎfiÓ· 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‹ ÌÔÚ› Ó· ¤¯ÂÈ Ê·Ó› ÂÚÈÛÛfiÙÂÚÔ ·ÓÙÈÚÔÛˆ¢ÙÈ΋ ÙÔ˘ ˘¤Ú‚·ÚÔ˘ Î·È Ù˘ ·¯˘Û·ÚΛ·˜.

™˘ÌÂÚ¿ÛÌ·Ù· ™˘ÌÂÚ·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 Î·Ó›˜ 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¯ÂÙÈ˙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›ÓÂÙ·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 (∂ÈÎfiÓ· 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 ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ, ·ÔÙÂÏ› Û˘ÁÎÚÈÙÈο ·ÎÚÈ‚¤ÛÙÂÚË Ì¤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·

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·143

143

97

33 33 97 32 32 ∫ÔÚ›ÙÛÈ· 31 31 30 (µMI 30) 30 29 29 28 28 90 27 27 26 26 (µMI 25) 25 25 75 24 24 23 23 50 22 22 21 21 25 20 20 10 19 19 3 18 18 17 17 16 16 15 15 14 14 13 13 12 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 1812 ∏ÏÈΛ· (¤ÙË)

kgr/m2

34 33 95 32 31 90 (µMI 30) 30 29 85 28 27 75 26 (µMI 25) 25 50 24 23 22 25 21 10 20 19 3 18 17 16 15 14 13 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 ∏ÏÈΛ· (¤ÙË)

∞ÁfiÚÈ·

kgr/m2

34 33 32 31 30 29 28 27 26 25 24 23 22 21 20 19 18 17 16 15 14 13

kgr/m2

kgr/m2

√ÚÈÛÌfi˜ Ù˘ ·È‰È΋˜ Î·È ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜

∂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 ÙˆÓ ÂÓËϛΈÓ, 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).

ÔÔ›· ‰˘ÛÙ˘¯Ò˜ Â›Ó·È ÔÏÏ¿. ∂›Û˘, ·Ú¤¯ÂÈ ·ÎÚÈ‚‹ Î·È Û˘ÁÎÚ›ÛÈÌ· ÌÂٷ͇ ÙÔ˘˜ ÛÙÔȯ›· ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÙÔ˘ ¢ª™ ·È‰ÈÒÓ Û ·Ú¤Ì‚·ÛË. ∆¤ÏÔ˜, Û ÂÚ¢ÓËÙÈÎfi Â›‰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 fiÔ˘ ÌÔÚ› Ó· ˘ÔÏÔÁÈÛÙ› ÙÔ z-score- ·Ó¿ ʇÏÔ Î·È ËÏÈΛ· ÛÙËÓ ∂ÏÏ¿‰· ÙÔ˘ Û‹ÌÂÚ· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 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 ·ÚÈıÌfi Ô˘ Â›Ó·È Û¯ÂÙÈ˙fiÌÂÓÔ˜ Ì ÙË Ì¤ÛË ÙÈÌ‹ ÙÔ˘ ÏËı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜, Ô ÔÔ›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) ∏ÏÈΛ· (¯ÚfiÓÈ·) 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18

∞ÁfiÚÈ· ∞ÚÈıÌfi˜ ª¤ÛË ÙÈÌ‹ 139 114 222 268 256 249 265 282 347 321 327 379 453 409 443 356 387 317

14,8 16,9 15,7 17,7 15,8 16,3 16,9 17,7 18,1 18,9 19,3 20,0 20,7 21,4 21,8 22,8 23,1 23,9

™Ù·ıÂÚ‹ ·fiÎÏÈÛË

∏ÏÈΛ· (¯ÚfiÓÈ·)

2,7 1,6 2,3 1,7 1,9 2,2 2,5 4,4 3,1 3,5 3,4 3,5 3,7 3,7 3,7 4,0 3,7 4,1

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18

∫ÔÚ›ÙÛÈ· ∞ÚÈıÌfi˜ ª¤ÛË ÙÈÌ‹ 131 111 216 267 256 237 228 248 325 318 342 409 423 385 428 323 370 371

14,7 16,1 15,5 15,6 15,8 16,4 16,7 17,5 18,2 18,5 19,1 19,8 20,5 21,1 21,4 21,9 22,1 22,3

™Ù·ıÂÚ‹ ·fiÎÏÈÛË 2,7 1,7 1,6 1,7 2,1 2,5 2,5 3,2 3,1 3,1 3,3 3,4 3,7 3,4 3,3 3,4 3,1 3,6 ¶·È‰È·ÙÚÈ΋ 2009;72:143-000

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144

¶. ¶ÂÚ‚·Ó›‰Ô˘

2. Lee SY, Gallagher D. Assessment methods in human body composition. Curr Opin Clin Nutr Metab Care 2008 Sep;11(5):566-572.

3. Revicki DA, Israel RG. Relationship between body mass indices and measures of body adiposity. Am J Publ Health 1986;76:992-994.

4. Spiegelman D, Isrel RG, Bouchard C, Willett WC. Absolute fat mass, percent body fat, and body fat distribution: Which is the real determinant of blood pressure and serum glucose? Am J Clin Nutr 1992;55:10441044.

5. WHO: Physical Status: The use and interpretation of Anthropometry. Geneva, World Health Organization, 1995.

6. Himes JH, Dietz WH. Guidelines for overweight in adolescent preventive services: recommendations from an expert committee: The Expert Committee on Clinical Guidelines for Overweigh in Adolescent Preventive Services. Am J Clin Nutr 1994;59:307-316.

7. Guillaume M: defining obesity in childhood: current practice. 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 Obesity Consensus 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 adolescent Medicine, vol 9), Karger, 2004, Basel.

10. Bellizzi MC, Dietz WH. Workshop on childhood obesity: Summary of the discussion. Am J Clin Nutr 1999;70:173S175S.

Paediatriki 2009;72:23-28

11. Cole TJ, Bellizzi MC, Flegal KM, Dietz WH. Establishing a standard definition for child overweight and obesity worldwide: International survey. BMJ 2000;320:12401243.

12. Pietrobelli A, Faith MS, Allison DB, et al. Body mass index as 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 limitations of the body mass index. Am J Clin Nutr 1986;44:996-997.

14. Higgins PB, Gower BA, Hunter GR, Goran MI. Defining health-related obesity in prepubertal children. Obes Res 2001;9:233-240.

15. Pervanidou P, Kanaka-Gantenbein C, Chrousos GP. Assessment of metabolic profile in a clinical setting. Curr Opin 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 metabolic syndrome in children and adolescents - an IDF consensus report. 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 ¶·ÓÂ ∞ıËÓÒÓ 2007;54:41-52.

19. Chiotis D, Krikos X, Tsiftis G, Hatzisymeaon M, ManiatiChristidi M, Dacou-Voutetakis A. Body mass index and prevalence of obesity in subjects of Hellenic origin aged 018years, living in the Athens area. Ann Cli Pediatr Unive Atheniensis 2004;51:139-154.

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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

CASE REPORT

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™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ Ã. ∆۷ηϛ‰Ë˜1, µ. §·ÌÚfiÔ˘ÏÔ˜2, Œ. √˘ÚÁηÓÙ˙fiÁÏÔ˘3, ¢. ƒ¿ÏÏ˘4, ∞. ¶ÂÙÚfiÔ˘ÏÔ˜2, ¡. ¡ÈÎÔÏ·˝‰Ë˜1 ¶ÂÚ›ÏË„Ë: ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Â›Ó·È ‰˘ÛÏ·Û›· ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È Â›ÛÔ‰Ô˜ ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ ̤۷ ÛÙÔÓ ıÒڷη, ‰È·Ì¤ÛÔ˘ ÂÓfi˜ ÂÏÏ›ÌÌ·ÙÔ˜ ÂÌ‚Ú˘ÔÁ¤ÓÂÛ˘ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, Î·È ÌÔÚ› 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 ÔÈÛıÔÏ¿ÁÈ· ΋ÏË ÙÔ˘ Bochdalek. √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ Â›Ó·È Û¿ÓÈ· ·ÓˆÌ·Ï›· Î·È ·ÓÙÈÚÔÛˆ‡ÂÈ ÏÈÁfiÙÂÚÔ ·fi 5% fiÏˆÓ ÙˆÓ ÓÂÊÚÈÎÒÓ ÂÎÙÔÈÒÓ. ªÔÚ› Ó· ÂÌÊ·Ó›˙ÂÙ·È ¯ˆÚ›˜ ¿ÏϘ 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 ¤ÎÙÔÔ ÓÂÊÚfi ÛÙÔ ¿Óˆ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ.

1 µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋ ∞¶£, ¡ÔÛÔÎÔÌÂ›Ô ¶··ÁˆÚÁ›Ô˘, £ÂÛÛ·ÏÔÓ›ÎË 2 µ’ ¶·È‰Ô¯ÂÈÚÔ˘ÚÁÈ΋ ∫ÏÈÓÈ΋ ∞¶£, ¡ÔÛÔÎÔÌÂ›Ô ¶··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˜ zoichr@otenet.gr ¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘», £ÂÛÛ·ÏÔÓ›ÎË

§¤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 organs enter the thorax through a developmental defect of the diaphragm. The hernia may occur in different parts of the diaphragm, but the most common type of diaphragmatic hernia is the posterolateral Bochdalek hernia. Intrathoracic ectopic kidney is a rare abnormality accounting for less than 5% of all renal ectopia. It can occur with no other malformation or can be associated with congenital diaphragmatic hernia or post-traumatic diaphragmatic hernia. The case is presented of a male neonate with a congenital diaphragmatic Bochdalek hernia, associated with renal ectopy in the left upper hemithorax.

Key worlds: Congenital diaphragmatic hernia, neonate.

∂ÈÛ·ÁˆÁ‹ ŒÎÙÔÔ˜ ÓÂÊÚfi˜ ··ÓÙ¿Ù·È Û 1:1.000 ÁÂÓÓ‹ÛÂȘ, ·ÏÏ¿ ÌfiÓÔ 1 ÛÙȘ 10 ·fi ·˘Ù¤˜ ÙȘ ÂÚÈÙÒÛÂȘ ‰È·ÁÈÁÓÒÛÎÂÙ·È (1). √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ ÂÌÊ·Ó›˙ÂÙ·È ·ÎfiÌË ÈÔ Û¿ÓÈ· Î·È ¤¯ÂÈ ÙË ÌÈÎÚfiÙÂÚË Û˘¯ÓfiÙËÙ· Û ۯ¤ÛË Ì ÙȘ ÏÔÈ¤˜ ÂÎÙÔ›Â˜ ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ (2,3), Â›Ó·È Î·Ù¿ ηÓfiÓ· ·Û˘Ìو̷ÙÈÎfi˜ Î·È ·ÔÙÂÏ› Ù˘¯·›Ô ‡ÚËÌ· ÛÙËÓ ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜. ∞ÎfiÌ· Û·ÓÈfiÙÂÚË Â›Ó·È Ë ‰È¿ÁÓˆÛË Ù˘ ‡·Ú͢ ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡ ηٿ ÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·. ¶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 ¤ÎÙÔÔ ÓÂÊÚfi ÛÙÔ ¿Óˆ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ, Ë ‰È¿ÁÓˆÛË ÙˆÓ ÔÔ›ˆÓ ¤ÁÈÓ ÚÔÁÂÓÓËÙÈο, 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 ÚÔÁÂÓÓËÙÈ΋˜ ‰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, Ì 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·ÈÛÙÒıËÎÂ Ë ‡·ÚÍË Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ·ÚÈÛÙÂÚ¿, Ì ›ÛÔ‰Ô ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Î·È ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘, ÌÂ Û˘ÓÔ‰fi ·ÚÂÎÙfiÈÛË Ù˘ ηډȿ˜ ÚÔ˜ Ù· ‰ÂÍÈ¿, ·ÏÏ¿ Î·È Û˘Ó‡·ÚÍË ¤ÎÙÔÔ˘ ·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΢ÌÔÛ‡Ó˘ ‚ÚÈÛÎfiÙ·Ó ˘fi ·ÁˆÁ‹ Ì Û΢¿ÛÌ·Ù·

1 2nd Department of Neonatal Paediatrics, “Aristotelio” University of Thessaloniki, “Papageorgiou” Hospital, Thessaloniki, Greece 2 2nd Department of Paediatric Surgery, “Aristotelio” University of Thessaloniki, “Papageorgiou” Hospital, Thessaloniki, Greece 3 2nd Department of Paediatrics, “Aristotelio” University of Thessaloniki, “AHEPA” Hospital, Thessaloniki, Greece 4 Infectious Diseases Hospital, Thessaloniki, Greece Correspondence: Christos Tsakalidis zoichr@otenet.gr “Papageorgiou” Hospital, Thessaloniki, Greece

¶·È‰È·ÙÚÈ΋ 2009;72:29-40

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∂ÈÎfiÓ· 1. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘. AÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜.

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 ‹ ¿ÏÏˆÓ Ô˘ÛÈÒÓ. ¶ÚÔËÁ‹ıËΠˆÚ›Ì·ÓÛË ÙˆÓ Ó¢ÌfiÓˆÓ ÙÔ˘ ÂÌ‚Ú‡Ô˘ Ì ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙË ÌËÙ¤Ú· Ì›· ‚‰ÔÌ¿‰· ÚÈÓ ·fi ÙÔÓ ÙÔÎÂÙfi. ∏ Ú‹ÍË ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ˘Ì¤ÓˆÓ ¤ÁÈÓ ηٿ ÙËÓ Â¤Ì‚·ÛË Î·È ÙÔ ·ÌÓÈ·Îfi ˘ÁÚfi ‹Ù·Ó ‰È·˘Á¤˜. ∆Ô ÓÂÔÁÓfi ÁÂÓÓ‹ıËÎÂ Î·È ¤ÎÏ·„ ·Ì¤Ûˆ˜, ·ÏÏ¿ ·Ì¤Ûˆ˜ ÂÁη٤ÛÙËÛ ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, Ì ÂÈÛÔÏΤ˜ ÌÂÛÔÏ¢ڛˆÓ ‰È·ÛÙËÌ¿ÙˆÓ, ÁÔÁÁ˘ÛÌfi Î·È Î˘¿ÓˆÛË. ªÂ ÁÓˆÛÙ‹ ÙËÓ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Ù˘ ™¢∫, ÙÔÔıÂÙ‹ıËΠ·Ì¤Ûˆ˜ ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎfi˜ ηıÂÙ‹Ú·˜ Î·È ¤ÁÈÓ ‰È·ÛˆÏ‹ÓˆÛË ÁÈ· ¿ÌÂÛË ˘ÔÛÙ‹ÚÈÍË ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡. ∆Ô Apgar score ‹Ù·Ó 17 Î·È 58 (‰È·ÛˆÏËӈ̤ÓÔ). ∆· ·¤ÚÈ· ÙÔ˘ ÔÌÊ·Ï›Ô˘ ÏÒÚÔ˘ Paediatriki 2009;72:146-000

‹Ù·Ó: p∏=7,32, pO2=23,6 mmHg, pCO2=58,6 mmHg, SBE=2,3 mmol/L. ∆Ô ÓÂÔÁÓfi ÌÂٷʤÚıËΠ‰È·ÛˆÏËӈ̤ÓÔ Ì ambu Û ÎÏÂÈÛÙ‹ ıÂÚÌÔÎÔÈÙ›‰·. ∆· ·¤ÚÈ· ·fi ÂÚÈÊÂÚÈ΋ ·ÚÙËÚ›· ÙÔ˘ ÓÂÔÁÓÔ‡ Ï›Á· ÏÂÙ¿ 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 ÂȂ‚·›ˆÛ ٷ Â˘Ú‹Ì·Ù· ÙÔ˘ ÚÔÁÂÓÓËÙÈÎÔ‡ ÂϤÁ¯Ô˘ (∂ÈÎfiÓ· 1). ∏ ÛÙ·ıÂÚ‹ ÎÏÈÓÈ΋ Î·È ÂÚÁ·ÛÙËÚȷ΋ ÂÈÎfiÓ· ÙÔ˘ ÓÂÔÁÓÔ‡ Â¤ÙÚ„ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ÙÔ ÚÒÙÔ 24ˆÚÔ, Ì ÙÔÔı¤ÙËÛË ϤÁÌ·ÙÔ˜ Goretex Î·È ÌÂÙ·ÊÔÚ¿ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ÓÂÊÚÔ‡ ÛÙËÓ Ô›ÛıÈ· ¿Óˆ ·ÚÈÛÙÂÚ‹ ÎÔÈÏȷ΋ ¯ÒÚ·. ÃÔÚËÁ‹ıËΠÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ‰‡Ô

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147

™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek

ÊÔÚ¤˜, ÙËÓ 1Ë Î·È ÙË 12Ë ÒÚ· ˙ˆ‹˜, ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙÔ ¯ÂÈÚÔ˘ÚÁ›Ô. ∏ ¤ÎÙ˘ÍË ÙÔ˘ Ó‡ÌÔÓ· ‹Ù·Ó ηϋ, fï˜ ÏfiÁˆ ‰È·Ù·Ú·¯ÒÓ Ù˘ Û¯¤Û˘ ·ÂÚÈÛÌÔ‡/ ·ÈÌ¿ÙˆÛ˘ ·fi ÙË Û˘Ó˘¿Ú¯Ô˘Û· Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·, ÙÔ ·Ó·Ó¢ÛÙÈÎfi Û‡ÛÙËÌ· ˘ÔÛÙËÚ›¯ıËΠ̠˘„›Û˘¯ÓÔ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi, Ì ¯ÔÚ‹ÁËÛË ÌÔÓÔÍÂȉ›Ô˘ ÙÔ˘ ·˙ÒÙÔ˘. ÃÔÚËÁ‹ıËÎ·Ó ÈÓfiÙÚÔ· ÁÈ· ÙËÓ ÂÓ›Û¯˘ÛË Ù˘ ÂÚÈÊÂÚÈ΋˜ ΢ÎÏÔÊÔÚ›·˜ Î·È ‰ÈÏ‹ ·ÓÙÈ‚ÈÔÙÈ΋ ·ÁˆÁ‹. ∏ ‰È¿ÁÓˆÛË Ù˘ ·Ú·Ì¤ÓÔ˘Û·˜ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ ÂȂ‚·ÈÒıËΠ̠˘ÂÚ˯ÔÁÚ¿ÊËÌ· Doppler ηډȿ˜ Î·È Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ. √ Â·Ó¤ÏÂÁ¯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›·, fiÔ˘ Î·È ‰È·ÈÛÙÒıËÎÂ Ë ‡·ÚÍË, ·ÎÚÈ‚Ò˜ οو ·fi ÙÔ ϤÁÌ·, ‰È·ÙÂٷ̤Ó˘ ¤ÏÈη˜ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘, Ô˘ ¤ÊÂÚ ÌÔÓ‹ÚË Ú‹ÍË ÛÙË ÌÂÛfiÙËÙ¿ Ù˘, ÌÂ Û˘Ú›ÁÁÈÔ ÚÔ˜ ÙÔ ÎÔÈÏÈ·Îfi ÙÔ›¯ˆÌ·, ·fi fiÔ˘ ÂÍÂÚ¯fiÙ·Ó ÙÔ ˘ÎÓfiÚÚ¢ÛÙÔ ¯ÔÏ҉˜ ÂÚȯfiÌÂÓÔ. ¶Ú·ÁÌ·ÙÔÔÈ‹ıËΠÂÎÙÔÌ‹ ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘ Î·È ‰ÈÂÓ¤ÚÁÂÈ· ‰ÈÏ‹˜ ÛÙÔÌ›·˜, ÙÔÔıÂÙ‹ıËΠ·ÚÔ¯¤Ù¢ÛË ÂÓÙfi˜ Ù˘ ÂÚÈÙÔÓ·˚΋˜ ÎÔÈÏfiÙËÙ·˜ Î·È ¤ÁÈÓ ۇÁÎÏÂÈÛË ÙÔ˘ ÙÚ·‡Ì·ÙÔ˜ Ì ηÈÓÔ‡ÚÁÈÔ ϤÁÌ· Goretex. ∏ ÈÛÙÔ·ıÔÏÔÁÈ΋ ÂͤٷÛË ÙÔ˘ ÂÓÙ¤ÚÔ˘ ¤‰ÂÈÍ ÌfiÓÔ ÛËÌ›· ÊÏÂÁÌÔÓ‹˜. ∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒڷη Ô˘ ‰ÈÂÓÂÚÁ‹ıËΠÙËÓ ÂfiÌÂÓË Ë̤ڷ ¤‰ÂÈÍ ·ÙÂÏÂÎÙ·Û›· Î·È Ì›ˆÛË ÙÔ˘ fiÁÎÔ˘ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ Ó‡ÌÔÓ·. ™ÙÔÓ ·ÛıÂÓ‹ ¯ÔÚËÁ‹ıËÎ·Ó ÂÈÛÓÂfiÌÂÓ· ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο Î·È ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹. ∆Ô ÓÂÔÁÓfi ÂÙ¤ıË Û ·ÁˆÁ‹ Ì ‰ÔÌÂÚȉfiÓË ÏfiÁˆ Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘. ∫ÂÓÙÚÈÎfi˜ ÊÏ‚ÈÎfi˜ ηıÂÙ‹Ú·˜ Ù‡Ô˘ Hickmann ÙÔÔıÂÙ‹ıËΠÛÙÔ ÓÂÔÁÓfi Î·È ·Ú¤ÌÂÈÓÂ Û˘ÓÔÏÈο ÁÈ· 35 Ë̤Ú˜. √ ·ÛıÂÓ‹˜ ·Ú¤ÌÂÈÓ Û Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi Û˘ÓÔÏÈο 46 Ë̤Ú˜. ªÂÙ¿ ÙËÓ ·Ô‰¤ÛÌ¢ÛË ·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ¯ÚÂÈ¿ÛıËΠ¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ ÁÈ· 9 Ë̤Ú˜. ∆ËÓ 58Ë Ë̤ڷ ˙ˆ‹˜ ¤ÁÈÓ Â¤Ì‚·ÛË ·Ó·ÛÙfïÛ˘ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Î·È ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ÎÔÏÔ‚ÒÌ·ÙÔ˜ ÙÔ˘ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘, Ô ·ÛıÂÓ‹˜ ÂÙ¤ıË Û ÔÏÈ΋ ·ÚÂÓÙÂÚÈ΋ ‰È·ÙÚÔÊ‹ ÁÈ· 11 Ë̤Ú˜, ÛÙË Û˘Ó¤¯ÂÈ· Û ÌÈÎÙ‹ Û›ÙÈÛË Î·È ·fi ÙËÓ 72Ë Ë̤ڷ ˙ˆ‹˜ ‚ÚÈÛÎfiÙ·Ó Û Ï‹ÚË ÂÓÙÂÚÈ΋ Û›ÙÈÛË, ηϿ ·ÓÂÎÙ‹. ∆· ˘ÂÚ˯ÔÁÚ·Ê‹Ì·Ù· ÂÁÎÂÊ¿ÏÔ˘

Anterior

Posterior

Left Post Oblique Left 54.5%

Right Post Oblique Right 45.5%

∂ÈÎfiÓ· 2. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘. ™Ù·ÙÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ.

‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. √ ÔÊı·ÏÌÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ηٿ ÙËÓ ¤ÍÔ‰fi ÙÔ˘ ·fi ÙËÓ ÎÏÈÓÈ΋ ‰ÂÓ ¤‰ÂÈÍ ·ıÔÏÔÁÈο 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‹ ÂÈÛÓÂfiÌÂÓ· ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο Î·È ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ÁÈ· ÙȘ ÂΉËÏÒÛÂȘ Ù˘ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ÙÔ˘ ‰˘ÛÏ·Û›·˜ Î·È Û΢¿ÛÌ·Ù· Ûȉ‹ÚÔ˘ Î·È Û‡ÌÏÂÁÌ· ‚ÈÙ·ÌÈÓÒÓ ∞ Î·È D. ∆Ô ÛÙ·ÙÈÎfi (∂ÈÎfiÓ· 2) Î·È ‰˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ Ì Tc-99m Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó 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 ‰˘ÛÏ·Û›· ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È ÚfiÙˆÛË ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ ̤۷ ÛÙÔ ıÒڷη, ‰È·Ì¤ÛÔ˘ ÂÓfi˜ ·Ó·ÙÔÌÈÎÔ‡ ÂÏÏ›ÌÌ·ÙÔ˜ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. ∏ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÌÔÚ› 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˘ ÔÔ›·˜ ÂÎÙÈÌ¿Ù·È ÂÚ›Ô˘ ÛÙÔ 90-95% (4). ∏ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛÂˆÓ ™¢∫ Ù‡Ô˘ Bochdalek ·ÊÔÚ¿ ÙËÓ ·ÚÈÛÙÂÚ‹ ÏÂ˘Ú¿ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ·ÏÏ¿ Ù· ÔÛÔÛÙ¿ ÙˆÓ ‰ÂÍÈ¿ Î·È ·ÚÈÛÙÂÚ¿ ÂÓÙÔÈ˙fiÌÂÓˆÓ ™¢∫ ÔÈΛÏÏÔ˘Ó. √È ™¢∫ ÂÓÙÔ›˙ÔÓÙ·È ·ÚÈÛÙÂÚ¿ Û ÔÛÔÛÙfi 73-90%, ¶·È‰È·ÙÚÈ΋ 2009;72:29-40

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·148

148

Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

‰ÂÍÈ¿ Û ÔÛÔÛÙfi 8-24% Î·È ·ÌÊÔÙÂÚfiÏ¢ڷ ‹ ÎÂÓÙÚÈο Û ÔÛÔÛÙfi 1,2-4,6% (5,6,7). ∏ ™¢∫ Ù‡Ô˘ Bochdalek ÂÚÈÁÚ¿ÊËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÙÔ 1948 (8). √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ Â›Ó·È Û¿ÓÈ· ·ÓˆÌ·Ï›· Î·È ·ÓÙÈÚÔÛˆ‡ÂÈ ÏÈÁfiÙÂÚÔ ·fi 5% fiÏˆÓ ÙˆÓ ÓÂÊÚÈÎÒÓ ÂÎÙÔÈÒÓ (2,9). ªÔÚ› Ó· ÂÌÊ·Ó›˙ÂÙ·È ¯ˆÚ›˜ ¿ÏϘ 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 (10). ™Â 15.919 ·˘ÙÔ„›Â˜ ·È‰ÈÒÓ Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËηÓ, ÌfiÓÔ 1 ÂÚ›ÙˆÛË ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡ ·Ú·ÙËÚ‹ıËÎÂ, ·Ó¿ÌÂÛ· Û 22 ÂÚÈÙÒÛÂȘ 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 Î·È Donat ÌÂϤÙËÛ·Ó ÂÚÈÙÒÛÂȘ Ô˘ ‰ËÌÔÛȇıËÎ·Ó ‰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 ·ÌÊÔÙÂÚfiÏ¢ÚË ÂÓ‰ÔıˆÚ·ÎÈ΋ ÓÂÊÚÈ΋ ÂÎÙÔ›·. √ ·ÚÈÛÙÂÚfi˜ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÛÎÂÏÂÙÈΤ˜ Î·È Î·Ú‰È·Î¤˜ ·ÓˆÌ·Ï›Â˜ (13). ∂ÈÚfiÛıÂÙ·, Ë Û˘ÁÁÂÓ‹˜ ·˘Ù‹ ·ÓˆÌ·Ï›· ··ÓÙ¿Ù·È Û˘¯ÓfiÙÂÚ· Û ¿ÚÚÂÓ˜ (63%) ·fi fi,ÙÈ ÛÙ· ı‹Ï· (37%). ∏ Û˘¯ÓfiÙËÙ· 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·ÎÈÎÔ‡ ÓÂÊÚÔ‡ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ·ÓˆÌ·Ï›· ÛÙÚÔÊ‹˜ Ù˘ ‡Ï˘ ÙÔ˘ ÓÂÊÚÔ‡ Á‡Úˆ ·fi ÙÔÓ ÂÈÌ‹ÎË ¿ÍÔÓ·, Ì·ÎÚ‡ Ô˘ÚËÙ‹Ú·, ˘„ËÏ‹ ¤ÎÊ˘ÛË ÙˆÓ ÓÂÊÚÈÎÒÓ ·ÁÁ›ˆÓ Î·È ·fiÎÏÈÛË ÙÔ˘ οو fiÏÔ˘ ÙÔ˘ ÓÂÊÚÔ‡ ·fi ÙË Ì¤ÛË ÁÚ·ÌÌ‹ (14,15). √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ ÂÌÊ·Ó›˙ÂÈ Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ·, ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÂÈ ‰˘ÛÏ·Û›· ‹ ·fiÊÚ·ÍË Ù˘ ÂÎÎÚÈÙÈ΋˜ ÙÔ˘ ÌÔ›Ú·˜ Î·È ‰ÂÓ Û˘Óԉ‡ÂÙ·È ·fi ˘ÂÚÏ·Û›· ÙÔ˘ ¿ÏÏÔ˘ ÓÂÊÚÔ‡ (16,22). ∏ ·ÈÙ›· Ù˘ ÂÓ‰ÔıˆÚ·ÎÈ΋˜ ÂÎÙÔ›·˜ Â›Ó·È ¿ÁÓˆÛÙË. ∏ ıˆڛ· Ù˘ ·ÔÙ˘¯Ë̤Ó˘ Û‡ÁÎÏÂÈÛ˘ Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘¯‹˜ ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· 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· ÌÂٷ͇ 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˘. ∫·Ù¿ Ì›· ¿Ô„Ë, ıˆÚÂ›Ù·È fiÙÈ ÂÓ‰ÔıˆÚ·ÎÈ΋ ÓÂÊÚÈ΋ ÂÎÙÔ›· ÌÔÚ› Ó· ÚÔÎÏËı› ·fi ÙËÓ Î·ı˘ÛÙÂÚË̤ÓË Û‡ÁÎÏÂÈÛË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ‹ ÙËÓ ÂÈÙ¿¯˘ÓÛË Ù˘ ·Ófi‰Ô˘ ÙÔ˘ ÓÂÊÚÔ‡ ÚÈÓ ·fi ÙËÓ Î·ÓÔÓÈ΋ 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 ·ÔÙÂÏÂ›Ù·È Paediatriki 2009;72:29-40

·fi ÌÈ· ÏÂÙ‹ 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˜ ÌÔÚ› Ó· ˘¿Ú¯ÂÈ Î·È ˘ÔÏ·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˘ ÓÂÊÚÔ‡ ηıÔÚ›˙ÂÙ·È ·fi ÙÌ‹Ì· ÙÔ˘ ÁÂÓÓËÙÈÎÔ‡ ÙfiÓÔ˘ (ÙÔ Û˘Á¯ˆÓÂ˘Ì¤ÓÔ Ô˘Ú·›Ô ÙÌ‹Ì· ÙˆÓ ‰‡Ô Ô˘ÚÔÁÂÓÓËÙÈÎÒÓ ·ÎÚÔÏÔÊÈÒÓ, οı ̛· ·fi ÙȘ Ôԛ˜ ·Ú¤¯ÂÈ ¤Ó·Ó ÌÂÛÔÓÂÊÚÈÎfi Î·È ¤Ó·Ó ·Ú·ÌÂÛÔÓÂÊÚÈÎfi fiÚÔ), ÙÔ ÔÔ›Ô ·ÔÚÚÔÊ¿Ù·È ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ÓÂÊÚÔÁ¤ÓÂÛ˘ (17). ™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ ÂÓ‰ÔıˆÚ·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˘ÚËÙ‹Ú·˜ Ù˘Èο ÂͤگÔÓÙ·È ·fi ÙËÓ ˘Â˙ˆÎÔÙÈ΋ ÎÔÈÏfiÙËÙ· ‰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 Ì ™¢∫ ‚Ú›ÛÎÂÙ·È ÛÙËÓ ·Ú·ÛÔÓ‰˘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). √ ÚÔÁÂÓÓËÙÈÎfi˜ ¤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 ÂÚÈÙÒÛÂˆÓ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ÌÂÙ¿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô (20,21). ∏ ÓÂÊÚÈ΋ ÂÎÙÔ›·, fiÙ·Ó ‰ÂÓ Û˘Óԉ‡ÂÙ·È ·fi ¿ÏϘ ·ÓˆÌ·Ï›Â˜, ‰ÂÓ ÚÔηÏ› Û˘ÌÙÒÌ·Ù·. √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ ·ÚÔ˘ÛÈ¿˙ÂÈ Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁ›· ¯ˆÚ›˜ ÛËÌ›· ‰˘ÛÏ·Û›·˜, ·fiÊڷ͢ Ù˘ ·ÂÎÎÚÈÙÈ΋˜ ÌÔ›Ú·˜ ‹ Û˘ÓÔ‰fi ˘ÂÚÏ·Û›· ÙÔ˘ ¿ÏÏÔ˘ ÓÂÊÚÔ‡ (16,22). øÛÙfiÛÔ, ˘¿Ú¯Ô˘Ó ÂÚÈÔÚÈṲ̂Ó˜ ·Ó·ÊÔÚ¤˜ ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ÂÓ‰ÔıˆÚ·ÎÈÎÒÓ ¤ÎÙÔˆÓ ÓÂÊÚÒÓ Ì ·fiÊÚ·ÍË Ù˘ ˘ÂÏÔÔ˘ÚËÙËÚÈ΋˜ Û˘Ì‚ÔÏ‹˜ ÙÔ˘ ÓÂÊÚÔ‡, Ì ΢ÛÙÂÔÔ˘ÚËÙËÚÈ΋ ·ÏÈÓ‰ÚfiÌËÛË Î·È Ì ÔÏ˘Î˘ÛÙÈ΋ ‰˘ÛÏ·Û›· ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi (1). ™ÙȘ ÂÚÈÙÒÛÂȘ Û˘Ó‡·Ú͢ ™¢∫, ÏfiÁˆ Ù˘ Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜ Ô˘ ÔÊ›ÏÂÙ·È ÛÙËÓ ›ÂÛË ÙˆÓ Ó¢ÌfiÓˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÌ‚Ú˘˚΋˜ ˙ˆ‹˜ ·fi Ù· ÂÓ‰ÔÎÔÈÏȷο ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó ÂÓÙfi˜ ÙÔ˘ ıÒڷη, ÔÈ ·ÛıÂÓ›˜ ηٿ ÙË Á¤ÓÓËÛË ÂΉËÏÒÓÔ˘Ó ÔÍ›· ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ·. ™Â ·˘Ù¤˜ ÙȘ ÂÚÈÙÒÛÂȘ ··ÈÙÂ›Ù·È ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË ÌfiÏȘ ÛÙ·ıÂÚÔÔÈËı› Ô ·ÛıÂÓ‹˜. ™Â ·Û˘Ìو̷ÙÈÎÔ‡˜ ·ÛıÂÓ›˜, Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË ÌÔÚ› Ó· Á›ÓÂÈ Ì¤Ú˜ ‹ Î·È ¯ÚfiÓÈ· ÌÂÙ¿, ·Ó¿ÏÔÁ· Ì ÙËÓ Î·Ù¿ÛÙ·Û‹ ÙÔ˘.

Pediatri Mar-Apr 09

10-04-09

15:37

™ÂÏ›‰·149

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™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek

¢È¿ÊÔÚ˜ ·ÂÈÎÔÓÈÛÙÈΤ˜ ̤ıÔ‰ÔÈ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ÙË ‰È¿ÁÓˆÛË ‡·Ú͢ ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ÓÂÊÚÔ‡ Î·È ÁÈ· ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ·fi ¯ˆÚÔηٷÎÙËÙÈΤ˜ ÂÂÍÂÚÁ·Û›Â˜ ÙÔ˘ ıÒڷη ‹ ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘. ∏ ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη Û˘Ó‹ıˆ˜ ·ÔηχÙÂÈ ·Ú·ÛÔÓ‰˘ÏÈ΋ Ì¿˙· ÛÙÔ Ô›ÛıÈÔ ÌÂÛÔıˆÚ¿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˘, fiˆ˜ ÙÔ Ó¢ÚÔ‚Ï¿Ûو̷, ÙÔ Á·ÁÁÏÈÔÓ‡ڈ̷ Î·È Ë Ó¢ÚÔÂÓÙÂÚÈ΋ ·ÛÙË (23). ™ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÈÙÒÛÂȘ, ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Ë ÂÓ‰ÔÊϤ‚È· ˘ÂÏÔÁÚ·Ê›· ÁÈ· ÙË ‰È¿ÁÓˆÛË (17,24). ∂›Û˘, ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· Î·È Ë CT ıÒڷη ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó ÛÙË ‰È¿ÁÓˆÛË (18,25). ∏ CT ıÒڷη ÌÔÚ› Ó· ÂȂ‚·ÈÒÛÂÈ ‚Ï¿‚˜ Ù˘ ˘¤ÏÔ˘ Î·È ÙˆÓ Î·Ï‡ÎˆÓ Î·È Ó· ‰È·ÁÓÒÛÂÈ ¿ÏϘ ‚Ï¿‚˜ Ô˘ ÌÈÌÔ‡ÓÙ·È ÂÓ‰ÔıˆÚ·ÎÈÎfi ¤ÎÙÔÔ ÓÂÊÚfi, fiˆ˜ ÔÈ fiÁÎÔÈ ÙÔ˘ ÌÂÛ·˘Ï›Ô˘ Î·È Ë ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË. £ÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË ··ÈÙÂ›Ù·È ÌfiÓÔ Û ÂÚ›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·È Î·ÙÂÛÙÚ·Ì̤ÓÔ˜, Ë ·fiÊÚ·ÍË Î·È Ë Î˘ÛÙÂÔÔ˘ÚËÙËÚÈ΋ ·ÏÈÓ‰ÚfiÌËÛË ÌÔÚ› Ó· ‰ÈÔÚıˆıÔ‡Ó Ì Â¤Ì‚·ÛË. ∂¿Ó fï˜ Ë ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÓÂÊÚÔ‡ Â›Ó·È ÛÔ‚·Ú¿ ÂËÚ·Ṳ̂ÓË, ÙfiÙÂ Ë ·Ê·›ÚÂÛ‹ ÙÔ˘ ·ÔÙÂÏ› ÙË Ï‡ÛË ÂÈÏÔÁ‹˜ (1). ∂›Û˘, ¯ÂÈÚÔ˘ÚÁÈ΋ ‰ÈÂÚ‡ÓËÛË ÂÈ‚¿ÏÏÂÙ·È fiÙ·Ó Û˘Ó˘¿Ú¯ÂÈ ™¢∫ Ù‡Ô˘ Bochdalek. ™Â ·˘Ù¤˜ ÙȘ ÂÚÈÙÒÛÂȘ Ô ÓÂÊÚfi˜ Û˘Ó‹ıˆ˜ ηχÙÂÙ·È ·fi ÙÔ ÔÈÛıÔÂÚÈÙfiÓ·ÈÔ Î·È ÌÔÚ› ‡ÎÔÏ· Ó· ÙÔÔıÂÙËı› ÛÙËÓ ÎÔÈÏȷ΋ ¯ÒÚ·, ¯ˆÚ›˜ ÛËÌ·ÓÙÈΤ˜ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÏÔΤ˜, fiˆ˜ ÛÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜. √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ ·ÔÙÂÏ› Û¿ÓÈ· ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ· Î·È ı¤ÙÂÈ ÔÏÏ¿ ‰È·ÁÓˆÛÙÈο Î·È ıÂÚ·¢ÙÈο ‰ÈÏ‹ÌÌ·Ù· ÛÙÔ˘˜ ÎÏÈÓÈÎÔ‡˜ ÁÈ·ÙÚÔ‡˜. ∏ Û˘Ó‡·ÚÍË ™¢∫ Ù‡Ô˘ Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ 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¿ÁÓˆÛË Î¿ı ÊÔÚ¿ Ô˘ Ù›ıÂÙ·È ı¤Ì· ‡·Ú͢ ÂÓ‰ÔıˆÚ·ÎÈ΋˜ Ì¿˙·˜. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È ÛˆÛÙ‹ ·ÓÙÈÌÂÙÒÈÛË ÌÂÈÒÓÂÈ ÙË ÓÔÛËÚfiÙËÙ· Î·È ıÓËÙfiÙËÙ·.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Bauerb SB. Anomalies of the kidney and ureteropelvic junction. Campbell’s urology. Saunders, Philadelphia 1998;1708-1755.

2. Donat SM, Donat PE. Intrathoracic kidney: a case report with a review of the world literature. J Urol 1987;140: 131-133.

3. Franciskovic V, Martincic N. Intrathoracic kidney. Br J Urol 1959;31:156.

4. Pober BR. Overview of epidemiology, genetics, birth defects and chromosome abnormalities associated with CDH. Am J Med Part C Semin Med Genet 2007;145C:158-171.

5. Skari H, Bjornland K, Haugen G, Egeland T, Emblem R. Congenital diaphragmatic hernia: A meta-analysis of mortality factors. J Pediatr Surg 2000;35:1187-1197.

6. Dott MM, Wong LY, Rasmussen SA. Population-based study of congenital diaphragmatic hernia: Risk factors and survival in Metropolitan Atlanta, 1968-1999. Birth Defects Res A Clin Mol Teratol 2003;67:261-267.

7. Yang W, Carmichael SL, Harris JA, Shaw GM. Epidemiologic characteristics of congenital diaphragmatic hernia among 2.5 million California births, 1989-1997.

Birth defects Res 2006;76:170-174.

8. Haller SA. Professor Bochdalek and his hernia: then and now. Prog Paediatr Surg 1986;20:252-255.

9. Summer TE, Volberg FM, Smolen PM. Intrathoracic kidney-diagnosis by ultrasound. Pediatr Radiol 1982;12: 78-80.

10. Karaoglanoglu N, Turkyilmaz A, Eroglu A, Alici A. Rightsided Bochdalek hernia with intrathoracic kidney. Pediatr Surg Int 2006;22:1029-1031.

11. Campbell MF. Renal ectopy. J Urol 1930;24:187-189.

12. Wolfromm MG. Situation du rein dans l’eventration diaphragmatique droite. Mem Acad Chir 1940;60:41-47.

13. Yalcinbas À, Sasmaz H, Canbaz S. Thoracic left kidney: differential diagnostic dilemma for thoracic Surgeons. Ann Thorac Surg 2001;72:281-283.

14. Spillane RJ, Prather GC. Right diaphragmatic eventration with renal displacement. J Urol 1952;68:804.

15. Gondos B. High ectopy of left kidney. Am J Roent-genol 1955;74:295-298.

16. Obatake M, Nakata T, Nomura M, Nanashina A, Inamura Y, Tanaka K, et al. Congenital intrathoracic kidney with right Bochdalek defect. Pediatr Surg Int 2006;22:861-863.

17. Hawass ND, Kolawole TM, EI Badawi MG, Patel PJ, Malabarey T. Intrathoracic kidneys: report of 6 cases and a review of the literature. Eur Urol 1988;14:83-87.

18. Mastrurzo B, Kalache KD, Cockell A, Pierro A, Rodeck CH. Prenatal diagnosis of an ectopic intrathoracic kidney in right-sided congenital diaphragmatic hernia using color Doppler ultrasonography. Ultrasound Obstet Gynecol 2001;18:173-174.

19. Hubbard AM, Crombleholme TM, Adzick NS, Coleman BG, Howell LJ, Meyer JS, et al. Prenatal MRI evaluation of congenital diaphragmatic hernia. Am J Perinatol 1999; 16:407-413.

20. Baglaj ª. Late-presenting congenital diaphragmatic hernia. Journal of Pediatric Surgery 2004;40:1839-1843.

21. Keles S, Artac H, Elmaci M, Reishi I, Dilsiz A. Late presenting congenital diaphragmatic hernia associated with ectopic thoracic kidney. Eur J Pediatr 2006;165:571-572.

22. Ramos A, Slovis T, Reed J. Intrathoracic kidney. Urology vol. XIII. 1979;1:14-19.

23. Vasquez J, Lupi H. Kasep J. Congenital intrathoracic right kidney. Arch Inst Cardiol Mex 1984;54:377-379.

24. Kirshenbaum AS, Puri HC, Rao BR. Congenital intrathorax kidney. J Urol 1981;125:412-413.

25. Grenadin DS, Larsen CR. Computed tomography of intrathoracic kidney. Urology 1983;22:85-87.

¶·È‰È·ÙÚÈ΋ 2009;72:29-40

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CLINICAL QUIZ

∞ÁfiÚÈ 13 ÂÙÒÓ Ì Â·Ó·Ï·Ì‚·ÓfiÌÂÓ· ÂÂÈÛfi‰È· ÂÒ‰˘Ó˘ Ô‰ÔÎÓËÌÈ΋˜ ¿ÚıÚˆÛ˘ µ’ √ÚıÔ·È‰ÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

¡. ª·ÚΤ·˜, π. ¶··¯Ú‹ÛÙÔ˜

AÏÏËÏÔÁÚ·Ê›·: ¡ÈÎfiÏ·Ô˜ ª·ÚΤ·˜ markeasn@otenet.gr µ’ √ÚıÔ·È‰ÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

Thirteen-year-old boy with recurrent episodes of painful ankle joint

2nd Department of Orthopaedics, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece

∞ÁfiÚÈ 13 ÂÙÒÓ ÚÔÛÎÔÌ›ÛÙËΠÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ·Ú·ÔÓÔ‡ÌÂÓÔ ÁÈ· ‰˘Ûη̄›· Ù˘ Ô‰ÔÎÓËÌÈ΋˜, ¿ÏÁÔ˜ Î·È ¯ˆÏfiÙËÙ·, ÌÂÙ¿ ·fi ηٷfiÓËÛË ÛÙË ‰È¿ÚÎÂÈ· ·ıÏÔ·È‰È¿˜. ¢ÂÓ ÂÚȤÁÚ·„Â Û˘ÁÎÂÎÚÈ̤ÓË Î¿ÎˆÛË, ÏËÓ fï˜ ·Ó¤ÊÂÚ ˆ˜ ÙÔ ÙÂÏÂ˘Ù·›Ô ÂÍ¿ÌËÓÔ Â›¯Â ηْ Â·Ó¿ÏË„Ë ·Ú·ÙËÚ‹ÛÂÈ Ô›‰ËÌ· ÛÙËÓ ¿ÚıÚˆÛË. ™ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË, ‰È·ÈÛÙÒıËΠ‰˘Ûη̄›· Ù˘ ·ÚÈÛÙÂÚ‹˜ Ô‰ÔÎÓËÌÈ΋˜ Î·È ÙÔÈ΋ ¢·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˘ ÚÂ˘Ì·ÙÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‹Ù·Ó Â›Û˘ ·ÚÓËÙÈÎfi˜. ∏ ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË Û ۯÈÛÌÔÂȉ‹ Ï˘¯Ó›·, ÁÈ· ÙË ‰È·›ÛÙˆÛË Èı·Ó‹˜ ÈÚȉÔ΢ÎÏ›Ùȉ·˜, ‰ÂÓ ¤‰ÂÈÍ ·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˘ (EÈÎfiÓ· 1). ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ¤‰ÂÈÍ ÌÈÎÚ‹ Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡, ÂÓ‰ÂÈÎÙÈ΋ ˘‰Ú¿ÚıÚÔ˘. ∆Ô ÛÈÓıËÚÔÁÚ¿ÊËÌ· 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. ∆ÔÔıÂÙ‹ıËΠÁ‡„ÈÓÔ˜ Ó¿ÚıËη˜ ÎÓËÌÔÔ‰ÈÎfi˜ Î·È ÌÂÙ¿ ·fi 3 ‚‰ÔÌ¿‰Â˜ ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó Ù· ·Ú¯Èο Û˘ÌÙÒÌ·Ù· Î·È Ù· ÎÏÈÓÈο ÛËÌ›·. ∆ÔÔıÂÙ‹ıËΠÎÓËÌÔÔ‰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˜ ·fi Ù· ÂÓԯϋ̷ٿ ÙÔ˘ Î·È ÔÈ ·ÎÙÈÓÔÁڷʛ˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈΤ˜.

Correspondence: Nikolaos Markeas markeasn@otenet.gr 2nd Department of Orthopaedics, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece

¡. Markeas, π. Papachristos

∂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 Â›‰Ô, ·ÔηχÙÂÈ ÙËÓ ·ÏÏÔ›ˆÛË ÛÙÔ Ô›ÛıÈÔ-¤Ûˆ ÙÌ‹Ì· ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘, ÙË ÌÈÎÚ‹ Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡ Î·È ÙÔ ÔÛÙÈÎfi Ô›‰ËÌ·.

¶Èı·Ó¤˜ ‰È·ÁÓÒÛÂȘ: 1. MË ÙÚ·˘Ì·ÙÈ΋ ÌÔÓÔ·ÚıÚ›Ùȉ· Ô‰ÔÎÓËÌÈ΋˜ 2. OÛÙÂÔÓ¤ÎÚˆÛË 3. ™¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· ·ÛÙÚ·Á¿ÏÔ˘ 4. √ÛÙÂÔÂȉ¤˜ ÔÛÙ¤ˆÌ· 5. ∞ÔÛ·ÛÙÈÎfi οٷÁÌ· Paediatriki 2009;72:66,71-73

∂ÈÎfiÓ· 3. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·, ÛÙÔ Ô‚ÂÏÈ·›Ô Â›‰Ô, ·Ó·‰ÂÈÎÓ‡ÂÈ Ù· ›‰È· Â˘Ú‹Ì·Ù·.

∏ ·¿ÓÙËÛË ÛÙË ÛÂÏ›‰· 157

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PAEDIATRIC NEWS IN BRIEF

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µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ

¡. ™˘Ú›‰Ë˜

∞ÏÏËÏÔÁÚ·Ê›·: ¡›ÎÔ˜ ¶. ™˘Ú›‰Ë˜ nspyridis@hotmail.co.uk µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ

News on paediatric infections N. Spyridis

∂ËÚ¿˙ÂÈ Ë ıÂÚ·›· Ù˘ ÔÍ›·˜ ̤Û˘ ˆÙ›Ùȉ·˜ (√ªø) ÙËÓ Â›ÙˆÛË Ù˘ Ì·ÛÙÔÂȉ›Ùȉ·˜ ÛÙ· ·È‰È¿; Paula Thompson et al.

Pediatrics 2009;123:424-430 ™ÙÔ Ù‡¯Ô˜ ºÂ‚ÚÔ˘·Ú›Ô˘ ÙÔ˘ Pediatrics ‰ËÌÔÛȇıËΠ¤Ó· ÂӉȷʤÚÔÓ ¿ÚıÚÔ Ô˘ ·ÊÔÚ¿ ÛÙË ¯Ú‹ÛË ÙˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ̤Û˘ ˆÙ›Ùȉ·˜ Î·È Ò˜ ·˘Ù‹ Ë ıÂÚ·›· ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ ÙËÓ Â›ÙˆÛË Ù˘ Ì·ÛÙÔÂȉ›Ùȉ·˜, Ô˘ ·ÔÙÂÏ› Ì›· ·fi ÙȘ ÛÔ‚·ÚfiÙÂÚ˜ ÂÈÏÔΤ˜ Ù˘ ÓfiÛÔ˘. ∏ Û˘ÁÎÂÎÚÈ̤ÓË ÌÂϤÙË ·ÌÊÈÛ‚ËÙ› Ì›· ÎÏ·ÛÈ΋ ıˆڛ· Ô˘ ηıÈÛÙ¿ ··Ú·›ÙËÙË ÙË ıÂÚ·›· Ù˘ √ªø. ™ÙËÓ ·Ó·‰ÚÔÌÈ΋ ·˘Ù‹ ÌÂϤÙË, Ë ÔÔ›· ¤Ï·‚ ¯ÒÚ· ÛÙË ª. µÚÂÙ·Ó›·, ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ËÏÂÎÙÚÔÓÈÎÔ› Ê¿ÎÂÏÔÈ ·È‰ÈÒÓ ËÏÈΛ·˜ 3 ÌËÓÒÓ ¤ˆ˜ 15 ÂÙÒÓ ÌÂٷ͇ ÙˆÓ ÂÙÒÓ 1990-2006, Ù· ÔÔ›· ·Ó¤Ù˘Í·Ó Ì·ÛÙÔÂȉ›Ùȉ·. ∞fi Ù· 854 ÂÂÈÛfi‰È· Ì·ÛÙÔÂȉ›Ùȉ·˜, ÌfiÓÔ ÙÔ 1/3 (35,7%) ›¯Â ÈÛÙÔÚÈÎfi √ªø. ∏ Èı·ÓfiÙËÙ· ·Ó¿Ù˘Í˘ Ì·ÛÙÔÂȉ›Ùȉ·˜ ÌÂÙ¿ ·fi √ªø ÁÈ· ÙËÓ ÔÔ›· ¯ÔÚËÁ‹ıËΠıÂÚ·›· ‹Ù·Ó 1,8/100.000 ÂÂÈÛfi‰È· √ªø, ÂÓÒ Ë Â›ÙˆÛË Û¯Â‰fiÓ ‰ÈÏ·ÛÈ¿˙ÂÙ·È (3,8/100.000) fiÙ·Ó ‰ÂÓ Â›¯Â ‰Ôı› ıÂÚ·›·. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ ÁÈ· Ó· ÚÔÏËÊı› 1 ÂÚ›ÙˆÛË Ì·ÛÙÔÂȉ›Ùȉ·˜ Ú¤ÂÈ Ó· ıÂÚ·‡ÛÔ˘Ì 4.831 ÂÚÈÙÒÛÂȘ √ªø. ™Â ·fiÏ˘ÙÔ˘˜ ·ÚÈıÌÔ‡˜, ·˘Ùfi ÌÂÙ·ÊÚ¿˙ÂÙ·È Û 255 ηÈÓÔ‡ÚÁÈ· ÂÚÈÛÙ·ÙÈο Ì·ÛÙÔÂȉ›Ùȉ·˜ ÙÔ ¯ÚfiÓÔ ÁÈ· ÙË ª. µÚÂÙ·Ó›· Â¿Ó ÛÙ·Ì·ÙÔ‡ÛÂ Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË ÁÈ· ÙËÓ √ªø, ÂÓÒ Ë ¯Ú‹ÛË ÙˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ı· ÌÂȈÓfiÙ·Ó Î·Ù¿ 738.775 Û˘ÓÙ·Á¤˜.

2nd Department of Paediatrics, University of Athens, Athens, Greece

™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ÂÈÛËÌ·›ÓÔ˘Ó fiÙÈ Ù· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ Ô˘ ·Ó¤Ù˘Í·Ó Ì·ÛÙÔÂȉ›Ùȉ· ‰ÂÓ Â›¯·Ó ÂÈÛÎÂÊı› ÙÔÓ ÁÈ·ÙÚfi ÙÔ˘˜. ∫·Ù¿ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜, Ë ·‡ÍËÛË Ù˘ Â›ÙˆÛ˘ Ù˘ Ì·ÛÙÔÂȉ›Ùȉ·˜ ÏfiÁˆ Ù˘ ÌË ¯Ú‹Û˘ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ÁÈ· ÙË ıÂÚ·›· √ªø Â›Ó·È ÌÈÎÚ‹ ÂÙËÛ›ˆ˜ Î·È ÙÔ fiÊÂÏÔ˜ ·fi ÙË Ì›ˆÛË Ù˘ ¯Ú‹Û˘ ·ÓÙÈ‚ÈÔÙÈÎÒÓ Ô˘ÛÈÒÓ ıˆÚÂ›Ù·È fiÙÈ Â›Ó·È ÛËÌ·ÓÙÈÎfiÙÂÚÔ ·fi ÙÔ ÎfiÛÙÔ˜ Ù˘ ÂÈÏÔ΋˜.

Correspondence: Nikos P. Spyridis nspyridis@hotmail.co.uk 2nd Department of Paediatrics, University of Athens, Athens, Greece

£ÂÚ·›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·˜: ˆÊÂÏ› Ë Ì·ÎÚÔ¯ÚfiÓÈ· ·ÚÂÓÙÂÚÈ΋ ıÂÚ·›·; Theoklis Zaoutis et al.

Pediatrics 2009:2;636-642 √ Zaoutis et al. ‰ËÌÔÛ›Â˘Û·Ó ÛÙÔ Pediatrics Ù· ·ÔÙÂϤÛÌ·Ù· ÌÈ·˜ ÌÂÁ¿Ï˘ ·Ó·‰ÚÔÌÈ΋˜ ÌÂϤÙ˘ Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËΠÛÙȘ ∏¶∞ Î·È ·ÊÔÚÔ‡Û ÛÙË Û‡ÁÎÚÈÛË Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ÂÓ‰ÔÊϤ‚È·˜ ıÂÚ·›·˜ ÁÈ· ÔÍ›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ· Ì ·˘Ù‹ Ù˘ Ù·¯Â›·˜ ÌÂÙ¿‚·Û˘ ·fi ÂÓ‰ÔÊϤ‚È· ıÂÚ·›· Û ıÂÚ·›· ·fi ÙÔ ÛÙfiÌ·. ™Â ·˘Ù‹ ÙËÓ ÔÏ˘ÎÂÓÙÚÈ΋ ÌÂϤÙË (29 ÓÔÛÔÎÔÌ›· ÙˆÓ ∏¶∞) Û˘ÌÂÚÈÂÏ‹ÊıËÛ·Ó ·È‰È¿ 2 ÌËÓÒÓ ¤ˆ˜ 17 ¯ÚÔÓÒÓ Ì ÔÍ›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ· ηٿ Ù· ¤ÙË 2000-2005. ™˘ÓÔÏÈο 1.969 ·È‰È¿ ÏËÚÔ‡Û·Ó Ù· ÎÚÈÙ‹ÚÈ·, ÂÎ ÙˆÓ ÔÔ›ˆÓ 1.021 ¤Ï·‚·Ó Ì·ÎÚ¿˜ ‰È¿ÚÎÂÈ·˜ ÂÓ‰ÔÊϤ‚È· ıÂÚ·›· (>2 ‚‰ÔÌ¿‰Â˜), ÂÓÒ 948 ¤Ï·‚·Ó ‚Ú·¯Â›· ÂÓ‰ÔÊϤ‚È· ıÂÚ·›· (<2 ‚‰ÔÌ¿‰Â˜) Î·È ÛÙË Û˘Ó¤¯ÂÈ· ıÂÚ·›· ·fi ÙÔ ÛÙfiÌ·. ∂ÈÙ˘¯‹˜ ‹Ù·Ó Ë ıÂÚ·›· ÛÙÔ 95% ÙˆÓ ·È‰ÈÒÓ Ô˘ ¤Ï·‚·Ó Ì·ÎÚÔ¯ÚfiÓÈ· ÂӉԂϤ‚È· ıÂÚ·›·, ¤Ó·ÓÙÈ ÙÔ˘ 96% ÙˆÓ ·È‰ÈÒÓ Ô˘ ¤Ï·‚·Ó ‚Ú·¯Â›· ÂÓ‰ÔÊϤ‚È· ¶·È‰È·ÙÚÈ΋ 2009;72:151-000

Pediatri Mar-Apr 09

10-04-09

152

15:37

™ÂÏ›‰·152

™À¡∆√ª∞ ¶∞π¢π∞∆ƒπ∫∞ ¡∂∞ ıÂÚ·›·. Ÿˆ˜ ·Ó·ÌÂÓfiÙ·Ó, Ù· ·È‰È¿ Ô˘ ¤Ï·‚·Ó ·ÚÂÓÙÂÚÈ΋ ıÂÚ·›· ›¯·Ó Û ÔÛÔÛÙfi 3,4% ÂÈÏÔΤ˜ 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 ıÂÚ·›· Ù˘ ÔÍ›·˜ ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·˜, Ë Ù·¯Â›· ÌÂÙ¿‚·ÛË ·fi ÙËÓ ÂÓ‰ÔÊϤ‚È· ÛÙËÓ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ·ÁˆÁ‹ Û¯ÂÙ›˙ÂÙ·È Ì ¿ÚÈÛÙ· ·ÔÙÂϤÛÌ·Ù·, ÂÓÒ ·Ú¿ÏÏËÏ· ·ÔʇÁÔÓÙ·È ÔÈ ·ÚÂÓ¤ÚÁÂȘ ·fi ÙË ¯Ú‹ÛË Î·ıÂÙ‹ÚˆÓ.

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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

˘Ù¤˜ ÂÈ ÙȘ Ô˘,

√ Ian Wong et al. ‰ËÌÔÛ›Â˘Û·Ó ÛÙÔ Archives of Diseases in Childhood ÌÈ· ÛÂÈÚ¿ ·fi„ÂˆÓ Ô˘ ·ÊÔÚÔ‡Ó ÛÙȘ ÌÂıfi‰Ô˘˜ ·ÓÙÈÌÂÙÒÈÛ˘ 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 Û 500.000 ÂÚÈÙÒÛÂȘ ÂÙËÛ›ˆ˜. ∆· ΢ÚÈfiÙÂÚ· ·›ÙÈ· ›ӷÈ: 1) Ë ÔÏ˘ÏÔÎfiÙËÙ· ÛÙÔÓ ˘ÔÏÔÁÈÛÌfi Ù˘ ‰fiÛ˘, Ë ÔÔ›· ÚÔÛ‰ÈÔÚ›˙ÂÙ·È Ì ‚¿ÛË ÔÏÏÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ËÏÈΛ·, ÙÔ ‚¿ÚÔ˜, Ë ÂÈÊ¿ÓÂÈ· ÛÒÌ·ÙÔ˜, Ë ÚÔˆÚfiÙËÙ· Î·È Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ·, 2) ·ÚÈıÌËÙÈο Ï¿ıË, ΢ڛˆ˜ ÛÙËÓ ÙÔÔı¤ÙËÛË ‰Âη‰ÈÎÔ‡ „ËÊ›Ô˘, ÌˉÂÓÈÎÒÓ Î·È ÌÔÓ¿‰ˆÓ ̤ÙÚËÛ˘, 3) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË ·fi Ó¤Ô˘˜ ¿ÂÈÚÔ˘˜ ÁÈ·ÙÚÔ‡˜, ÔÈ ÔÔ›ÔÈ Û˘ÓÙ·ÁÔÁÚ·ÊÔ‡Ó Ì ‚¿ÛË ÙËÓ ÂÌÂÈÚ›· ÙÔ˘˜ ·fi ÙÔÓ ÂÓ‹ÏÈÎÔ ÏËı˘ÛÌfi Î·È 4) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË Ê·ÚÌ¿ÎˆÓ Ù· ÔÔ›· ‰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 ·ÓÙÈÌÂÙÒÈÛË ·˘ÙÔ‡ ÙÔ˘ Ê·ÈÓÔ̤ÓÔ˘ Û˘ÓÈÛٿٷÈ: 1) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË Ó· Á›ÓÂÙ·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·ÎÔÔÈÔ‡˜, 3) Ë ÂÎ·›‰Â˘ÛË ÙˆÓ È·ÙÚÒÓ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ÂÈÌÔÚʈÙÈο ÛÂÌÈÓ¿ÚÈ· Û˘ÓÙ·ÁÔÁÚ¿ÊËÛ˘, 4) Ó· ÂÚÈÁÚ¿ÊÂÙ·È Ë Ì¤ıÔ‰Ô˜ ˘ÔÏÔÁÈÛÌÔ‡ Ù˘ ‰fiÛ˘ ÛÙË Û˘ÓÙ·Á‹, 5) fiÛÔÓ ·ÊÔÚ¿ ÙË ¯ÔÚ‹ÁËÛË ÂÓ‰ÔÊÏ‚›ˆÓ Ê·Ú̿ΈÓ, Ë ¯Ú‹ÛË ËÏÂÎÙÚÔÓÈÎÒÓ Û˘Û΢ÒÓ ÌÔÚ› 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˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ ÛÙË Ï‹„Ë ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ Î·È 7) Ó·

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Paediatriki 2009;72:152-000

‚ÂÏÙȈı› Ë ÂÈÎÔÈÓˆÓ›· ÌÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ ÂÌÏÂÎfiÌÂÓˆÓ 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΋ ÌÂϤÙË Ô˘ ¤Ï·‚ ¯ÒÚ· ÛÙË °ÂÚÌ·Ó›· ÌÂٷ͇ ÙˆÓ ÂÙÒÓ 1997-2003 Î·È ·ÊÔÚÔ‡Û ÛÙËÓ Ù·˘ÙÔÔ›ËÛË ÙÔ˘ Ó¢ÌÔÓÈfiÎÔÎÎÔ˘ Û ·È‰È¿ Ô˘ ¤ı·Ó·Ó ÏfiÁˆ Ù˘ Ïԛ̈͢ ‹ ·Ó¤Ù˘Í·Ó ‚·ÚÈ¿ Ïԛ̈ÍË (ÛË„·ÈÌ›·, ÌËÓÈÁÁ›Ùȉ·) ‹ ÛÔ‚·Ú¤˜ ÌfiÓÈ̘ ‚Ï¿‚˜. ∞fi ÙȘ 559 ÂÚÈÙÒÛÂȘ fiÔ˘ Ô ÔÚfiÙ˘Ô˜ Ù·˘ÙÔÔÈ‹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 Û 17%. √ ÔÚfiÙ˘Ô˜ 7F Û˘Û¯ÂÙÈ˙fiÙ·Ó Ì ıÓËÙfiÙËÙ· 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 ÔÔ›ˆÓ ÔÛÔÛÙfi 40,7% ÔÊÂÈÏfiÙ·Ó ÛÙÔÓ ÔÚfiÙ˘Ô 7F. ™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ÛËÌÂÈÒÓÔ˘Ó fiÙÈ Ë ÌËÓÈÁÁ›Ùȉ· ·fi ÙÔÓ ÔÚfiÙ˘Ô 7F Û˘Ó‰¤ÂÙ·È Ì ·˘ÍË̤ÓË ıÓËÙfiÙËÙ· ‹ ÂÈÏÔΤ˜, ÂÓÒ ÙÚÈÏ·ÛÈ¿˙ÂÙ·È Ë Èı·ÓfiÙËÙ· ·Ó¿Ù˘Í˘ ‚·ÚÈ¿˜ ‰ÈÂÈÛ‰˘ÙÈ΋˜ Ïԛ̈͢. ∏ ·Í›· ·˘Ù‹˜ Ù˘ ÌÂϤÙ˘ ‚Ú›ÛÎÂÙ·È ÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô ÔÚfiÙ˘Ô˜ 7F ‰ÂÓ Û˘ÌÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙÔ ˘¿Ú¯ÔÓ Û˘˙¢Á̤ÓÔ Ó¢ÌÔÓÈÔÎÔÎÎÈÎfi ÂÌ‚fiÏÈÔ, ÁÈ’ ·˘Ùfi Î·È Ë ·ÚÔ˘Û›· ÙÔ˘ ‹‰Ë ·ÚÔ˘ÛÈ¿˙ÂÈ ·‡ÍËÛË Û ¯ÒÚ˜ fiˆ˜ 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˘ÌÂÚÈÏ·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·‚ ¯ÒÚ· 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 ÂȉËÌÈÔÏÔÁ›· ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÈÒÓ. ÃÚËÛÈÌÔÔÈ‹ıËÎ·Ó ËÏÂÎÙÚÔÓÈο ·Ú¯Â›· (Hospital

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PAEDIATRIC NEWS IN BRIEF Episode Statistics) Ù· ÔÔ›· ÂÚÈÁÚ¿ÊÔ˘Ó ÙÔ ·›ÙÈÔ ÂÈÛ·ÁˆÁ‹˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô, ‰ËÌÔÁÚ·ÊÈο ÛÙÔȯ›· ÙÔ˘ ·ÛıÂÓÔ‡˜, ·ÓÙÈÌÂÙÒÈÛË Î·È ·Ú·ÎÏÈÓÈΤ˜ ÂÍÂÙ¿ÛÂȘ. ªÂٷ͇ 1998 Î·È 2003 ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó 2.432 ·È‰È¿ Ì ÓfiÛÔ Kawasaki, ÂÎ ÙˆÓ ÔÔ›ˆÓ 728 ÂÚÈÙÒÛÂȘ ·ÔÙÂÏÔ‡Û·Ó Â·ÓÂÈÛ·ÁˆÁ‹. ∞fi ÙȘ 1.704 ηÈÓÔ‡ÚÁȘ ÂÚÈÙÒÛÂȘ, ÔÈ 1.228 (72%) ‹Ù·Ó ·È‰È¿ <5 ÂÙÒÓ Î·È ÔÈ 254 ·È‰È¿ οو ÙÔ˘ ¤ÙÔ˘˜. √ ·ÚÈıÌfi˜ ÙˆÓ Î·ÈÓÔ‡ÚÈˆÓ ÂÚÈÙÒÛÂˆÓ Û ¤Ó· Ì‹Ó· ÁÈ· Ù· ·È‰È¿ οو ÙˆÓ 5 ÂÙÒÓ Î˘Ì¿ÓıËΠ·fi 8 (™Â٤̂ÚÈÔ˜ 2001) ¤ˆ˜ 41 (π·ÓÔ˘¿ÚÈÔ˜ 1999). ∏ ÂÙ‹ÛÈ· Â›ÙˆÛË Ù˘ ÓfiÛÔ˘ ‹Ù·Ó 8,39/100.000 ·È‰È¿. ∏ ÎÈÓÂ˙È΋ ÂıÓÈÎfiÙËÙ· (P=0,005), Ë ‰È·‚›ˆÛË Û ÌÂÁ¿Ï˜ fiÏÂȘ (P=0,001) Î·È Ë ÔÈÎÔÓÔÌÈ΋ ·Ó¤¯ÂÈ· (P=0,016) ·ÔÙÂÏÔ‡Û·Ó ÈÛ¯˘ÚÔ‡˜ ÚԉȷıÂÛÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Ù˘ ÓfiÛÔ˘. ∏

153

˘„ËÏfiÙÂÚË Â›ÙˆÛË ·Ú·ÙËÚ‹ıËΠÙÔ˘˜ ¯ÂÈÌÂÚÈÓÔ‡˜ Ì‹Ó˜, ¯ˆÚ›˜ fï˜ οÔÈÔ˜ ·fi ÙÔ˘˜ Û˘Ó‹ıÂȘ ÈÔ‡˜ (RSV, adenovirus, influenza) Ó· ÂÌÊ·Ó›˙ÂÙ·È Û˘¯ÓfiÙÂÚ·. ™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ·Ó·Ê¤ÚÔ˘Ó fiÙÈ, fiˆ˜ ›¯Â ·Ú·ÙËÚËı› Î·È ÛÙËÓ π·ˆÓ›·, Ë ÌÂٷΛÓËÛË ÛÙȘ ÌÂÁ¿Ï˜ fiÏÂȘ Û˘Ó‰˘¿ÛÙËΠ̠¤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˘Ó ÂÓÔ¯ÔÔÈËı›, 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 ÚԉȷıÂÛÈÎfi ·Ú¿ÁÔÓÙ·.

¶·È‰È·ÙÚÈ΋ 2009;72:153-000

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∂§§∏¡π∫∞ ∂ƒ∂À¡∞ ™∆∏ ¢π∂£¡∏ µπµ§π√°ƒ∞ºπ∞

∂ÏÏËÓÈ΋ ·È‰È·ÙÚÈ΋ ¤Ú¢ӷ Û ‰ÈÂıÓ‹ ÂÚÈÔ‰Èο, 2008 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘ AÏÏËÏÔÁÚ·Ê›·: ∂ÌÌ·ÓÔ˘‹Ï °·Ï·Ó¿Î˘ emmgalan@med.uoc.gr ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘

∂. °·Ï·Ó¿Î˘

Hellenic paediatric research in international journals, 2008 E. Galanakis

Department of Paediatrics, University of Crete, Heraklion, Greece Correspondence: Emmanouil Galanakis emmgalan@med.uoc.gr Department of Paediatrics, University of Crete, Heraklion, Greece

¶·ÚÔ˘ÛÈ¿˙ÔÓÙ·È, Ôχ Û˘ÓÔÙÈο, Ï›Á˜ ·fi ÙȘ ÔÏϤ˜ ÂÏÏËÓÈΤ˜ ÌÂϤÙ˜ ·È‰È·ÙÚÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜ Ô˘ ‰ËÌÔÛȇıËÎ·Ó ÙÔ 2008 Û ‰ÈÂıÓ‹ ÂÚÈÔ‰Èο Î·È Î·Ï‡ÙÔ˘Ó fiÏ· Ù· ‰›·, ·fi ÙÔ ÂÚÁ·ÛÙËÚÈ·Îfi ˆ˜ ÙÔ ÎÏÈÓÈÎfi Î·È ÙÔ ÎÔÈÓˆÓÈÎfi, Î·È fiϘ ÙȘ ËÏÈ˘, ·fi ÙÔ ÓÂÔÁÓfi Î·È ÙÔ ‚Ú¤ÊÔ˜ ˆ˜ ÙÔ ·È‰› Î·È ÙÔÓ ¤ÊË‚Ô. ªÂϤÙ˜ Ô˘ ›¯·Ó ‰ËÌÔÛÈ¢ı› ÙÔ˘˜ Ôχ ÚÒÙÔ˘˜ 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¿. ¶ÚÔÔÙÈ΋ ÌÂϤÙË ·fi Ù· πˆ¿ÓÓÈÓ· (Drougia et al., Nephrol Dial Transplant, Epub 2008 Aug 4) ·Ú·ÎÔÏÔ‡ıËÛ ˘ÂÚ˯ÔÁÚ·ÊÈο ÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ Û 243 ÌÈÎÚ¿ ÁÈ· ÙËÓ ËÏÈΛ· ·ËÛ˘ ÓÂÔÁÓ¿ (SGA) Î·È 223 ÓÂÔÁÓ¿ Ì ηٿÏÏËÏÔ ‚¿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 ÓÂÊÚÈ΋ ·Ó¿Ù˘ÍË ‹Ù·Ó fiÌÔÈ· Ì ÂΛÓË ÙˆÓ AGA, ˆÛÙfiÛÔ ÛÙ· ÌÈÎÚ¿ ÚfiˆÚ· ÓÂÔÁÓ¿, ÙÔ Ì¤ÁÂıÔ˜ ÙˆÓ ÓÂÊÚÒÓ ·Ú¤ÌÂÓ ÌÈÎÚfiÙÂÚÔ ·fi ÂΛÓÔ ÙˆÓ AGA ÓÂÔÁÓÒÓ ÁÈ· Ù· ‰‡Ô ÚÒÙ· ¤ÙË Ù˘ ˙ˆ‹˜. °ÂÓÂÙÈ΋ ÚԉȿıÂÛË ÛÙË Ì¤ÛË ˆÙ›Ùȉ·. ªÂϤÙË ·fi ÙÔ ∏Ú¿ÎÏÂÈÔ (Ilia S et al., Pediatr Infect Dis J 2008;27:929-33) ·Ó·ÛÎÔ› Ù· ¤ˆ˜ ÙÒÚ· ÁÓˆÛÙ¿ ÁÈ· ÙËÓ ·¿ÓÙËÛË ÙÔ˘ ÍÂÓÈÛÙ‹ Î·È ÙË ÁÂÓÂÙÈ΋ ¢¿ıÂÈ· ÛÙËÓ ˆÙ›Ùȉ·. ∫·Ù·ÁÚ¿ÊÂÙ·È ÌÈ· ÌÂÁ¿ÏË ÔÈÎÈÏ›· ·Ú·ÁfiÓÙˆÓ Ù˘ ÂÁÁÂÓÔ‡˜ Î·È ÚÔÛ·ÚÌfiÛÈÌ˘ ·ÓÔÛ›·˜ Ô˘ Úԉȷı¤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‹. ¶ÂÚÈÁÚ·Ê‹ ÚˆÙÔ·ıÔ‡˜ Ê˘Ì·Ù›ˆÛ˘ ¤Ô˘˜ ÌÂ Û˘ÓÔ‰fi ‚Ô˘‚ˆÓÈ΋ ÏÂÌÊ·‰ÂÓ›Ùȉ· Û ˘ÁȤ˜ ηٿ Ù· ¿ÏÏ· ·ÁfiÚÈ, ËÏÈΛ·˜ 10 ÌËÓÒÓ, Ô˘ ›¯Â ÂÚÈÙÌËı› ÛÙÔ ¶·ÎÈÛÙ¿Ó ÚÔ 4Ì‹ÓÔ˘ (¡ÔÛÔ-

Paediatriki 2009;72:154-000

ÎÔÌÂ›Ô ¶·›‰ˆÓ ∞ıËÓÒÓ «∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡», Papaevangelou V et al., J Infect Epub 2008 Dec 18). ∏ ÂȂ‚·›ˆÛË ¤ÁÈÓ Ì ÔÍÂÔ¿ÓÙÔ¯Ë ¯ÚÒÛË, ηÏÏȤÚÁÂÈ· Î·È PCR. µÚԢΤÏψÛË Û ÌÈÎÚfi ‚Ú¤ÊÔ˜. ¶ÂÚÈÁÚ·Ê‹ ÎÚÔ‡ÛÌ·ÙÔ˜ ‚ÚԢΤÏÏ·˜ Û ‚Ú¤ÊÔ˜ 2,5 ÌËÓÒÓ ·fi ÙËÓ ◊ÂÈÚÔ (Makis AC et al., Emerg Infect Dis 2008;141:319-20). ∆Ô ‚Ú¤ÊÔ˜ ‰ÂÓ Â›¯Â Â·Ê‹ Ì ˙Ò·, ‰ÂÓ ı‹Ï·˙Â Î·È Ë ÌËÙ¤Ú· ÙÔ˘ ‰ÂÓ Â›¯Â ‚ÚԢΤÏψÛË, ¿Ú· ·Ú·Ì¤ÓÂÈ ·ÓÔÈÎÙfi ÙÔ ı¤Ì· Ù˘ ÌÂÙ¿‰ÔÛ˘ ·fi ÌÔÏ˘Ṳ̂ӷ ·ÓÙÈΛÌÂÓ·, 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·˜ (¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ ∞ıËÓÒÓ «∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡», Niniou T et al., Eur J Clin Microbiol Infect Dis 2008;27:831-7): 44% MRSA ·ÓÙÔ¯‹ (ÛÙË ÌÂıÈÎÈÏÏ›ÓË). ∞ÓıÂÎÙÈÎÔ› ÛÙ·Ê˘ÏfiÎÔÎÎÔÈ ÛÙËÓ ¶¿ÙÚ·. ∞Ó¿ÏÔÁË ÌÂϤÙË ·fi ÙÔ ∫·Ú·Ì·Ó‰¿ÓÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ ¶·ÙÚÒÓ (170 ·ÛıÂÓ›˜ Ì Ïԛ̈ÍË ·fi S. aureus Î·È ˘ÁÈ‹ ·È‰È¿ Ì ÚÈÓÈ΋ ÊÔÚ›· S. aureus, Sdougkos G et al., Clin Microbiol 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 ˘ÁÈÒÓ ·È‰ÈÒÓ. ¶Ó¢ÌÔÓÈfiÎÔÎÎÔ˜: ·ÓÙÔ¯‹. ªÂϤÙË 612 ÛÙÂϯÒÓ Streptococcus pneumoniae, ‰ÈÂÈÛ‰˘ÙÈÎÒÓ Î·È ÌË, ÚÈÓ ÙËÓ Â˘Ú›· ÂÊ·ÚÌÔÁ‹ ÙÔ˘ Ó¢ÌÔÓÈÔÎÔÎÎÈÎÔ‡ ÂÌ‚ÔÏ›Ô˘ (¡ÔÛÔÎÔÌÂ›Ô ¶·›‰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¿ÏÏËÏË Ì ÙËÓ ·‡ÍËÛË Ù˘ ηٷӿψÛ˘ Ì·ÎÚÔÏȉ›ˆÓ Î·È ÏÈÓÎÔ˙·ÌȉÒÓ ÛÙËÓ ÎÔÈÓfiÙËÙ· (·fi

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HELLENIC RESEARCH IN THE INTERNATIONAL LITERATURE 4,31 DI ‰fiÛÂȘ ÙÔ 1990-1996 Û 6,97 DI ‰fiÛÂȘ ÙÔ 2001-2004). ¶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›ˆÛË Ù˘ ÚÈÓÔÊ·Ú˘ÁÁÈ΋˜ ÊÔÚ›·˜ ÙˆÓ ÔÚÔÙ‡ˆÓ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ÌÂÙ¿ ÙÔ ÂÙ·‰‡Ó·ÌÔ Û˘˙¢Á̤ÓÔ ÂÌ‚fiÏÈÔ, ¯ˆÚ›˜ ·‡ÍËÛË Ù˘ ÊÔÚ›·˜ ÙÔ˘ ÔÚfiÙ˘Ô˘ 19∞. ™Ù· ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿ ·Ú·ÙËÚ‹ıËΠ̛ˆÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ ÛÙÂϯÒÓ ˘„ËÏ‹˜ ·ÓÙÔ¯‹˜ ÛÙËÓ ÂÓÈÎÈÏÏ›ÓË. £ÂÚ·›· Ì˘ÎËÙÈ·ÛÈÎÒÓ ÏÔÈÌÒ͈Ó. ªÂϤÙË ·fi ÙÔ πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢ (Filioti I et al., Expert Opin Pharmacother 2008;9:3179-96) ·Ó·ÛÎÔ› ÙȘ ıÂÚ·¢ÙÈΤ˜ ÛÙÚ·ÙËÁÈΤ˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ‰ÈÂÈÛ‰˘ÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ ·fi ∫¿ÓÙÈÓÙ· Î·È ∞Û¤ÚÁÈÏÏÔ Û ÓÂÔÁÓ¿ Î·È ·È‰È¿, Ì ¤ÌÊ·ÛË ÛÙ· ÓÂfiÙÂÚ· ·Ú¿ÁˆÁ·. §Â˘¯·È̛˜ Î·È ÏÂÌÊÒÌ·Ù· ÛÙËÓ ∂ÏÏ¿‰·. ¶·ÓÂÏÏ‹ÓÈ· ÌÂϤÙË 11ÂÙ›·˜ (1996-2006) η٤ÁÚ·„ (Petridou ET et al., Arch Dis Child 2008;93:1027-32) 863 ÂÚÈÙÒÛÂȘ Ï¢¯·ÈÌ›·˜ Î·È 311 ÏÂÌÊÒÌ·ÙÔ˜ Û ·È‰È¿ οو ÙˆÓ 15 ÂÙÒÓ. ∏ Â›ÙˆÛË Ù˘ Ï¢¯·ÈÌ›·˜ (46,6 ÂÚÈÙÒÛÂȘ ·Ó¿ 1.000.000 ·È‰È¿ ·Ó¿

155

¤ÙÔ˜) ‹Ù·Ó ·Ó¿ÌÂÛ· ÛÙȘ ˘„ËÏfiÙÂÚ˜ Ù˘ ∂˘Úˆ·˚΋˜ ŒÓˆÛ˘ (19% ˘„ËÏfiÙÂÚË ·fi ÙË Ì¤ÛË Â˘Úˆ·˚΋). ∏ Â›ÙˆÛË ÏÂÌÊÒÌ·ÙÔ˜ (16,8 ÂÚÈÙÒÛÂȘ ·Ó¿ 1.000.000 ·È‰È¿ ·Ó¿ ¤ÙÔ˜) ‰ÂÓ ‰È¤ÊÂÚ ·fi ÙË Ì¤ÛË Â˘Úˆ·˚΋. §ÈıÔÙÚÈ„›· ÛÙ· ·È‰È¿. ¶·ÚÔ˘Û›·ÛË Ù˘ 10ÂÙÔ‡˜ ÂÌÂÈÚ›·˜ ÂÓfi˜ ΤÓÙÚÔ˘ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Ô˘ÚÔÏÈı›·Û˘ Û 125 ·È‰È¿ (πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢, Charalambous S et al., J Endourol 2008;22:2169-74): Û 93 ·È‰È¿ ¤ÁÈÓ ÏÈıÔÙÚÈ„›· Ì ˆÛÙÈο ·̷ٷ ˘ÂÚ‹¯ˆÓ (Û‡ÓÔÏÔ 108 Û˘Ó‰ÚÈÒÓ), Û 12 Ô˘ÚËÙËÚÔÛÎfiËÛË Î·È ‰ÈÔ˘ÚËÙËÚÈ΋ ·Ê·›ÚÂÛË, Û 9 ‰È·‰ÂÚÌ·ÙÈ΋ ÓÂÊÚÔÏÈıÔÙÔÌ›· Î·È Û 11 ¯ÚÂÈ¿ÛÙËΠ·ÓÔÈÎÙfi ¯ÂÈÚÔ˘ÚÁ›Ô. ™‡Ìʈӷ Ì ÙË ÌÂϤÙË, Ë ÏÈıÔÙÚÈ„›· Î·È ÔÈ ÂÓ‰ÔÔ˘ÚËÙËÚÈΤ˜ Ù¯ÓÈΤ˜ Â›Ó·È ·ÛÊ·Ï›˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈΤ˜ ÛÙ· ·È‰È¿. ∆Ô ‡„Ô˜ ÙˆÓ Ó¤ˆÓ ∂ÏÏ‹ÓˆÓ. ªÂϤÙË (∞ÙÙÈÎfi ¡ÔÛÔÎÔÌ›Ô, Papadimitriou ∞ et al., Acta Paediatr 2008;97:1105-7) Û 3.982 ÓÂÔÛ‡ÏÏÂÎÙÔ˘˜ ËÏÈΛ·˜ 1826 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˘ÓÂÒ˜, fiÙÈ Ô ·Ó‰ÚÈÎfi˜ ÏËı˘ÛÌfi˜ Ù˘ ∂ÏÏ¿‰·˜ ‰ÂÓ ¤¯ÂÈ ·ÎfiÌË ÂÍ·ÓÙÏ‹ÛÂÈ Ù· ÂÚÈıÒÚÈ· ·‡ÍËÛ˘ Û ‡„Ô˜.

¶·È‰È·ÙÚÈ΋ 2009;72:155-000

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¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√

NEWS FROM THE INTERNET

¡ÂÔÁÓÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

πÛÙÔÛÂÏ›‰· Ù˘ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ – http://www.2pediatric.gr/

AÏÏËÏÔÁÚ·Ê›·: ™Ù¤Ê·ÓÔ˜ ∫¯·ÁÈ¿˜ kechstef@otenet.gr ¡ÂÔÁÓÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

™. ∫¯·ÁÈ¿˜, ¢. ∞Ó·ÁÓÒÛÙÔ˘

Site of the 2nd Department of Paediatrics of the University of Athens – http://www.2pediatric.gr/ S. Kehagias, D. Anagnostou

Neonatology Unit, 2nd Department of Paediatrics, University of Athens, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece Correspondence: Stefanos Kehagias kechstef@otenet.gr Neonatology Unit, 2nd Department of Paediatrics, University of Athens, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece

Paediatriki 2009;72:156

√ ‰È·‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ Ù˘ µã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, Ô˘ ‰ڿ˙ÂÙ·È ÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ÏÂÈÙÔ˘ÚÁ› Î·È ·Ó·ÌÔÚÊÒÓÂÙ·È Û˘Ó¯Ҙ Â‰Ò Î·È ‰‡Ô ¯ÚfiÓÈ·. ™Ùfi¯Ô˜ ÙÔ˘ Â›Ó·È Ó· ʤÚÂÈ Û Â·Ê‹ Ì ÙÔÓ ¯ÒÚÔ Ù˘ ∫ÏÈÓÈ΋˜ fiÏÔ˘˜ fiÛÔ˘˜ ı· ıÂÏ‹ÛÔ˘Ó Ó· ¯ÚËÛÈÌÔÔÈ‹ÛÔ˘Ó ÙȘ È·ÙÚÈΤ˜, ‰È‰·ÎÙÈΤ˜ Î·È ÂÚ¢ÓËÙÈΤ˜ Ù˘ ‰Ú·ÛÙËÚÈfiÙËÙ˜. ™ÙȘ ÛÂÏ›‰Â˜ ÙÔ˘ ˘¿Ú¯Ô˘Ó ÏËÚÔÊÔڛ˜ 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· ÂȉÈο Â͈ÙÂÚÈο È·ÙÚ›·. ™ÙËÓ ·Ú¯È΋ ÛÂÏ›‰·, ÂÎÙfi˜ ·fi ÙËÓ Ù·¯˘‰ÚÔÌÈ΋ ‰È‡ı˘ÓÛË 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 ÊÚÔÓÙÈÛÙËÚ›ˆÓ Ô˘ ÔÚÁ·ÓÒÓÂÈ Ë ∫ÏÈÓÈ΋ ÛÙÔ ·ÌÊÈı¤·ÙÚÔ «∫. ¶··‰¿ÙÔ˜» ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ Ì·˜. ™ÙËÓ ·Ú¯È΋ ÛÂÏ›‰· Â›Û˘, ÛÙÔ˘˜ «ÃÚ‹ÛÈÌÔ˘˜ ™˘Ó‰¤ÛÌÔ˘˜», ˘¿Ú¯Ô˘Ó ÈÛÙfiÙÔÔÈ Ô˘ ÚÔÛʤÚÔ˘Ó Â˘Î·Èڛ˜ ÂÓË̤ڈÛ˘ ÁÈ· ÙË Û˘Ó¯Ҙ ÂÍÂÏÈÛÛfiÌÂÓË ÁÓÒÛË Û¯ÂÙÈο Ì ÙÔ ·È‰› Î·È ÙËÓ ˘Á›· ÙÔ˘, ·ÏÏ¿ Î·È ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘, fiˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ ÈÛÙfiÙÔÔ ÙÔ˘ ∫¤ÓÙÚÔ˘ Ù˘ π·ÙÚÈ΋˜ Ô˘ µ·Û›˙ÂÙ·È Û ª·ÚÙ˘Ú›Â˜ (Center for Evidence Based Medicine). ™ÙËÓ ›‰È· ÛÂÏ›‰·, ·ÎÔÏÔ˘ı› Ô ¯ÒÚÔ˜ fiÔ˘ ÌÔÚ› Ó· Á›ÓÂÈ Ë ËÏÂÎÙÚÔÓÈ΋ ÂÁÁÚ·Ê‹ ÛÙÔ newsletter Ù˘ ∫ÏÈÓÈ΋˜. ∏ ÛÂÏ›‰· «¶ÚÔÛˆÈÎfi» ·Ú¤¯ÂÈ ÛÙÔȯ›· ÁÈ· ÙÔ ·ÓıÚÒÈÓÔ ‰˘Ó·ÌÈÎfi Î·È ÙË ÛÙÂϤ¯ˆÛË Ù˘ ∫ÏÈÓÈ΋˜. ∏ ÛÂÏ›‰· «∆Ì‹Ì·Ù·» ‰Â›¯ÓÂÈ ÙȘ ÎÏÈÓÈΤ˜ ‰˘Ó·ÙfiÙËÙ˜ Ù˘ ∫ÏÈÓÈ΋˜. ∞ӷʤÚÔÓÙ·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›‰· Â›Û˘, ·Ú¤¯ÔÓÙ·È ÏËÚÔÊÔڛ˜ ÁÈ· Ù· ˆÚ¿ÚÈ· ÏÂÈÙÔ˘ÚÁ›·˜, ·ÏÏ¿ Î·È ÙȘ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÙˆÓ ÙÌËÌ¿ÙˆÓ Î·È ÙˆÓ Â͈ÙÂÚÈÎÒÓ È·ÙÚ›ˆÓ. ∞ÎÔÏÔ˘ı› Ë ÛÂÏ›‰· «∂Î·›‰Â˘ÛË», Ô˘ ÂÚÈÁÚ¿ÊÂÈ ÙÔ ÂÎ·È‰Â˘ÙÈÎfi ¤ÚÁÔ Ù˘ µã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›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·ÛÙËÚÈfiÙËÙ· Ô˘ ‚Ú›ÛÎÂÙ·È Û ÂͤÏÈÍË ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÎfiÓËÛË ‰È‰·ÎÙÔÚÈÎÒÓ ‰È·ÙÚÈ‚ÒÓ, ÔÏ˘ÎÂÓÙÚÈÎÒÓ ÌÂÏÂÙÒÓ, ÂȉËÌÈÔÏÔÁÈÎÒÓ ÌÂÏÂÙÒÓ, ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÌÂÏÂÙÒÓ, ÌÂÏÂÙÒÓ ÎÏÈÓÈ΋˜ ·Ú·Ù‹ÚËÛ˘, ·ÏÏ¿ Î·È ‰È·¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ ηٷÁÚ·Ê‹˜ ÓÔÛËÌ¿ÙˆÓ. ∆¤ÏÔ˜, ÛÙÔ ‰›Ô «∞Ó·˙‹ÙËÛË», Ô ÈÛÙfiÙÔÔ˜ ÚÔÛʤÚÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ıÂÌ·ÙÈ΋˜ ·Ó·˙‹ÙËÛ˘ ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘, Ì ¯Ú‹ÛË Ï¤ÍˆÓ-ÎÏÂȉÈÒÓ.

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∫§π¡π∫√ ∫√Àπ∑

CLINICAL QUIZ

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∞¶∞¡∆∏™∏ ¢È¿ÁÓˆÛË: ™¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· ·ÛÙÚ·Á¿ÏÔ˘

√È ÚÒÙ˜ ÂÚÈÁڷʤ˜ Ù˘ Û¯·ÏȉˆÙÈ΋˜ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ·˜ ÛÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· οÓÔ˘Ó ÙËÓ ÂÌÊ¿ÓÈÛ‹ ÙÔ˘˜ ‹‰Ë ·fi Ù· Ù¤ÏË ÙÔ˘ 19Ô˘ ·ÈÒÓ·, ·ÏÏ¿ ÌfiÏȘ ÙÔ 1959 ÔÈ Berndt Î·È Harty (1) ÚÔ‚·›ÓÔ˘Ó ÛÙËÓ ÚÒÙË Ù·ÍÈÓfiÌËÛË. ∏ ÓfiÛÔ˜ ÚÔÛ‚¿ÏÏÂÈ Û˘Ó‹ıˆ˜ ÙÔ˘˜ ÌËÚÈ·›Ô˘˜ ÎÔÓ‰‡ÏÔ˘˜ Î·È Û·ÓÈfiÙÂÚ· ÙÔÓ ÎfiÓ‰˘ÏÔ ÙÔ˘ ‚Ú·¯ÈfiÓÈÔ˘ ÔÛÙÔ‡. ∏ ÂÓÙfiÈÛ‹ Ù˘ ÛÙÔÓ ıfiÏÔ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘ ıˆÚÂ›Ù·È È‰È·›ÙÂÚ· Û¿ÓÈ· (4% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ÔÛÙÂÔ¯fiÓ‰ÚÈÓˆÓ ‚Ï·‚ÒÓ ÙÔ˘ ÛÎÂÏÂÙÔ‡). ∏ ÈÛ¯·ÈÌÈ΋ Ó¤ÎÚˆÛË ÙÔ˘ ˘ÔΛÌÂÓÔ˘ ˘Ô¯fiÓ‰ÚÈÔ˘ ÔÛÙÔ‡ Ê·›ÓÂÙ·È ˆ˜ ·ÈÙÈÔÏÔÁ› ¿ÚÈÛÙ· ÙËÓ ·ÔÎfiÏÏËÛË ÙÔ˘ ÔÛÙÈÎÔ‡ ÙÂÌ·¯›Ô˘, Ì·˙› Ì ÙÔÓ ˘ÂÚΛÌÂÓÔ ·ÚıÚÈÎfi ¯fiÓ‰ÚÔ. πÛÙÔÚÈÎfi οΈÛ˘ ·Ó·Ê¤ÚÂÙ·È Û ¤Ó· ÔÛÔÛÙfi 85% ÙˆÓ ·ÛıÂÓÒÓ Ì ÔÛÙÂÔ¯fiÓ‰ÚÈÓË ‚Ï¿‚Ë ÛÙÔÓ ·ÛÙÚ¿Á·ÏÔ. ∂ȉÈο, ÔÈ ‚Ï¿‚˜ ÛÙÔ Ô›ÛıÈÔ-¤Ûˆ ÙÌ‹Ì· ÙÔ˘ ıfiÏÔ˘ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘ Â›Ó·È ÙÔ ·ÔÙ¤ÏÂÛÌ· Â·Ó·Ï·Ì‚·ÓfiÌÂÓÔ˘ ‚›·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¿‚Ë ÌÔÚ› 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·ÚıÚÔ Ù˘ Ô‰ÔÎÓËÌÈ΋˜ (fiˆ˜ ÛÙËÓ ÂÚÈÁÚ·Ê›۷ ÂÚ›ÙˆÛË), ‰›¯ˆ˜ ÙÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÙÔ˘ ‰È·ÛÙÚ¤ÌÌ·ÙÔ˜ Ô›‰ËÌ· ÛÙËÓ ÂÚÈÔ¯‹ ÙˆÓ ÛÊ˘ÚÒÓ. ™ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ¤Ó· Ï‹ıÔ˜ ·ı‹ÛˆÓ, Ì ÂÓÙfiÈÛË ÙËÓ Ô‰ÔÎÓËÌÈ΋ ‹ 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· ÂÓԯϋ̷ٷ Î·È ÎÏÈÓÈο ÛËÌ›·, ÂÓÒ ÔÈ ‰Â›ÎÙ˜ ÊÏÂÁÌÔÓ‹˜ Â›Ó·È Î·Ù¿ ηÓfiÓ· ·˘ÍË̤ÓÔÈ. ∏ ÔÛÙÂÔÓ¤ÎÚˆÛË ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ·Ô˘Û›· ·ÁÁ›ˆÛ˘ ÙÔ˘ ˘ÔΛÌÂÓÔ˘ Ù˘ ‚Ï¿‚˘ ÔÛÙÔ‡, ÂÓÒ ÛÙË Û¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· Ë ·ÁÁ›ˆÛË Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋. ∂Í ¿ÏÏÔ˘, Ë ÔÛÙÂÔÓ¤ÎÚˆÛË ÚÔÛ‚¿ÏÏÂÈ ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ˘. ∆Ô ÔÛÙÂÔÂȉ¤˜ ÔÛÙ¤ˆÌ· ¤¯ÂÈ ÂÓÙÔÓfiÙÂÚ· Û˘ÌÙÒÌ·Ù· Î·È ¯·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·Ú̿ΈÓ. ∆Ô ·ÔÛ·ÛÙÈÎfi οٷÁÌ· Â›Ó·È ·Û˘Ì‚›‚·ÛÙÔ Ì ÙËÓ ·Ó·ÙÔÌÈ΋ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘,

ÂÂȉ‹ Ô ıfiÏÔ˜ ÙÔ˘ ‰ÂÓ ‰¤¯ÂÙ·È ÚÔÛʇÛÂȘ ÈÛ¯˘ÚÒÓ Û˘Ó‰¤ÛÌˆÓ ‹ ÙÂÓfiÓÙˆÓ. ∏ ¿Ù˘Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· Î·È ÙÔ ·Û·Ê¤˜ ÈÛÙÔÚÈÎfi ‰ÂÓ ÈηÓÔÔÈÔ‡Ó Û˘Ó‹ıˆ˜ ÙË ‰È¿ÁÓˆÛË Î·È ··ÈÙÂ›Ù·È Û˘¯Ó¿ Ô ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜. √È ·ÎÙÈÓÔÁڷʛ˜, ÔÈ Ôԛ˜ Â› ÛÂÈÚ¿ ÂÙÒÓ ‹Ù·Ó Ë ÚÒÙË 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˘Ó ÙËÓ ÔÛÙÈ΋ ‚Ï¿‚Ë. ∞˘Ùfi˜ ‹Ù·Ó Ô ÏfiÁÔ˜ Ù˘ ηı˘ÛÙÂÚË̤Ó˘ ‰È¿ÁÓˆÛ˘ ÛÙË ‰È΋ Ì·˜ ÂÚ›ÙˆÛË. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ¤¯ÂÈ ÙËÓ ÈηÓfiÙËÙ· Ù˘ ¤ÁηÈÚ˘ ·ÂÈÎfiÓÈÛ˘ Ù˘ ‚Ï¿‚˘, Ù˘ ·ÎÚÈ‚Ô‡˜ ÂÓÙfiÈÛ˘ Î·È Ù˘ ¤ÎÙ·Û‹˜ Ù˘. ∂›Û˘ ÌÔÚ› Ó· ·ÂÈÎÔÓ›ÛÂÈ Ì ·ÎÚ›‚ÂÈ· ÙËÓ ‡·ÚÍË ÔÛÙÈÎÔ‡ Ôȉ‹Ì·ÙÔ˜. ™‡Ìʈӷ Ì ÙËÓ ÈÛ¯‡Ô˘Û· Ù·ÍÈÓfiÌËÛË (2-4) (Ô˘ ÛÙËÚ›˙ÂÙ·È ÛÙ· Â˘Ú‹Ì·Ù· Ù˘ Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜) Î·È ·Ó¿ÏÔÁ· Ì ÙÔ ·ÂÈÎÔÓÈ˙fiÌÂÓÔ ÛÙ¿‰ÈÔ, ·ÔÊ·Û›˙ÂÙ·È Ë ÂÊ·ÚÌÔÛÙ¤· ıÂÚ·›·. ∆Ô ÛÙ¿‰ÈÔ 1 ÂÚÈÏ·Ì‚¿ÓÂÈ ‚Ï¿‚Ë ÙÔ˘ ·ÚıÚÈÎÔ‡ ¯fiÓ‰ÚÔ˘ ÌfiÓÔ, Ô˘ ‰ÂÓ Á›ÓÂÙ·È ÔÚ·Ù‹ ÛÙȘ ·ÎÙÈÓÔÁڷʛ˜ Î·È ‰ÂÓ ··ÈÙ› ¿ÏÏË ıÂÚ·›· ·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¿‰ÈÔ 2·, fiˆ˜ ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È) ‹ ¯ˆÚ›˜ Ô›‰ËÌ· (2‚). ™Ù· ÛÙ¿‰È· ·˘Ù¿, Ë ÚÔÙÂÈÓfiÌÂÓË ıÂÚ·›· Â›Ó·È Û˘ÓÙËÚËÙÈ΋, Ì ·ÎÈÓËÙÔÔ›ËÛË Û ӿÚıËη Î·È ·ÔÊ˘Á‹ Ï‹ÚÔ˘˜ ÊfiÚÙÈÛ˘ ÙÔ˘ ÛΤÏÔ˘˜ ÁÈ· 6 ÙÔ˘Ï¿¯ÈÛÙÔÓ Â‚‰ÔÌ¿‰Â˜. ™ÙÔ ÛÙ¿‰ÈÔ 3, ·Ó·Ê·›ÓÂÙ·È ‰È·¯ˆÚÈÛÌfi˜, ·ÏÏ¿ fi¯È ·ÚÂÎÙfiÈÛË, ÔÛÙÂÔ¯fiÓ‰ÚÈÓÔ˘ ÙÂÌ·¯›Ô˘. ™ÙÔ ÛÙ¿‰ÈÔ 4, ÙÔ ‰È·¯ˆÚÈṲ̂ÓÔ ÙÂÌ¿¯ÈÔ ¤¯ÂÈ ·ÔÎÔÏÏËı› Î·È Û˘ÌÂÚÈʤÚÂÙ·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 ıÂÚ·›· Â›Ó·È ¯ÂÈÚÔ˘ÚÁÈ΋. ¶ÚÔÙÈÌ¿Ù·È Ë Û˘ÁÎÚ¿ÙËÛË ÙÔ˘ ·ÔÎÔÏÏË̤ÓÔ˘ ÙÂÌ·¯›Ô˘, ÂÊfiÛÔÓ Â›Ó·È ÌÂÁ¿ÏÔ Î·È ·Ó‹ÎÂÈ Û ÊÔÚÙÈ˙fiÌÂÓË ·ÚıÚÈ΋ ÂÈÊ¿ÓÂÈ·, ‹ Ë ·Ê·›ÚÂÛ‹ ÙÔ˘ ÂÊfiÛÔÓ Â›Ó·È ÌÈÎÚfi Î·È Ë ·Ô˘Û›· ÙÔ˘ ‰ÂÓ ‰È·Ù·Ú¿ÛÛÂÈ ÙË ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ· Ù˘ ¿ÚıÚˆÛ˘ (5,6).

¶·È‰È·ÙÚÈ΋ 2009;72:66,71-73

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∫§π¡π∫√ ∫√Àπ∑ µÈ‚ÏÈÔÁÚ·Ê›· 1. Berndt AL, Harty M. Transchondral fractures (osteochondritis dissecans) of the talus. J Bone Joint Surg 1959;41A:988-1020.

2. Stroud CC, Marks RM. Imaging of osteochondral lesions of the talus. Foot Ankle Clin Mar 2000;5:119-133.

3. Elias I, Jung JW, Raikin SM, Schweitzer MW, Carrino JA, Morrison WB. Osteochondral lesions of the talus: change in MRI findings over time in talar lesions without operative intervention and implications for staging systems. Foot Ankle Int 2006;27:157-166.

Paediatriki 2009;72:66,71-73

4. Hepple S, Winson IG, Glew D. Osteochondral lesions of the talus: a revised classification. Foot Ankle Int 1999;20:789-793.

5. Tol JL, Struijs PA, Bossuyt PM, Verhagen RA, van Dijk CN. Treatment strategies in osteochondral defects of the talar dome: a systematic review. Foot Ankle Int 2000;21:119-126.

6. Giannini S, Buda R, Faldini C, Vannini F, Bevoni R, Grandi G. Surgical treatment of osteochondral lesions of the talus in young active patients. J Bone Joint Surg Am 2005;87(Suppl 2):28-41.