Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

Exof Pediatr Sept-Oct 05

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¶·È‰È·ÙÚÈ΋ ñ TfiÌÔ˜ 68 ñ ∆‡¯Ô˜ 5 ñ ™Â٤̂ÚÈÔ˜-√ÎÙÒ‚ÚÈÔ˜ 2005

TfiÌÔ˜ 68 ñ ∆‡¯Ô˜ 5 ñ ™Â٤̂ÚÈÔ˜-√ÎÙÒ‚ÚÈÔ˜ 2005 www.paediatriki.edu.gr

Volume 68 ñ Number 5 ñ September-October 2005 ISSN 0377-2551

Pediatr Sept-Oct 05

17-10-05

16:50

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¶·È‰È·ÙÚÈ΋ ∆fiÌÔ˜ 68 ñ ∆‡¯Ô˜ 5 ñ ™Â٤̂ÚÈÔ˜-OÎÙÒ‚ÚÈÔ˜ 2005 ¢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 ¶. ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘ ∂. ∞ÓÙˆÓÔÔ‡ÏÔ˘ A. µ·˙·›Ô˘-°ÂÚ·ÛÈÌ›‰Ë °. µ·ÚÏ¿Ì˘ ª. ∫·Ó¿ÚÈÔ˘ ∂. ∫·ÙÛ·ÚÔ‡-¶ÂÎÙ·Û›‰Ë A. K·ÙÙ¿Ì˘ Ã. ∫ÒÛÙ·ÏÔ˜ ¡. ¶··‰fiÔ˘ÏÔ˜ ∞. ¶··‰ÔÔ‡ÏÔ˘ ∞. ™È·ÌÔÔ‡ÏÔ˘-ª·˘Ú›‰Ô˘ ª. ∆ÛÔÏÈ¿-¶·Û·Ú¿ÎË ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ· ∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈÌ¤ÓˆÓ I. K·Ú·‚Ú¿ÓÔ˘ EÈ̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ™. ¡¿ÎÔ˘

¶ÂÚȯfiÌÂÓ· ∂¶π™∆O§∏ ∞¶O ∆∏ ™À¡∆∞•∏

∂¡¢π∞º∂ƒOÀ™∂™ ¶∂ƒπ¶∆ø™∂π™

325 ∂Ó·ÚÎÙ‹ÚÈÔ˜ OÌÈÏ›· ÛÙÔ 43Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ °. ¢. ª·ÌÈÓÈÒÙ˘

366 ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË. ¶ÂÚÈÁÚ·Ê‹ ÚÒÙ˘ ÂÚ›ÙˆÛ˘ ÛÙËÓ ∂ÏÏ¿‰· O. ¶··Ó‰Ú¤Ô˘, ∞. ∫ÏËÌÂÓÙÔÔ‡ÏÔ˘, ¡. ¶ÔÓËÚfi˜, ∞. ∫ˆÓÛÙ·ÓÙÈÓ›‰Ô˘, ∞. •˘ÔÏ˘Ù¿-∑·¯·ÚÈ¿‰Ë, N. Rachman, °. ÃÚÔ‡ÛÔ˜ 372 ™‡Ó‰ÚÔÌÔ ÌÈÎÚÔÂÏÏ›ÌÌ·ÙÔ˜ 22q11.2 Û ÔÈÎÔÁ¤ÓÂÈ· Ì ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔÓ Ê·ÈÓfiÙ˘Ô. ¶ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ∞. ∞Ó‰Ú¤Ô˘, µ. ∞ÏÂÍ·Ó‰ÚÔÔ‡ÏÔ˘-∆ÛÈÎڛη, ∞. ™·ÎÂÏÏ·ÚÔÔ‡ÏÔ˘, ª. °Ô˘‰ÂÛ›‰Ô˘, §. ºÏˆÚÂÓÙ›Ó-∞Ú¿Ú

∞¡∞™∫O¶∏™∂π™ 329 ¶·ıÔÊ˘ÛÈÔÏÔÁ›· ÙˆÓ ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ ∂. ∫·Ú·Ù˙¿-•ÈÊÈÏ›‰Ô˘, ª. ∞ı·Ó·Û›Ô˘-ªÂÙ·Í¿ 337 ∞ÍÈÔÏfiÁËÛË Ù˘ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚfiÎÏËÛ˘ ÁÈ· ÙË ‰È¿ÁÓˆÛË Ê·Ú̷΢ÙÈ΋˜ ·ÏÏÂÚÁ›·˜ A. ™ÔÊÈ·ÓÔ‡-∫·ÙÛÔ‡ÏË, G. Du Toit, G. Lack 342 ∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ· µ. ∆Û·ÁÚ‹˜, ∞. ∫ÈÙÛÔÔ‡ÏÔ˘, ∫. ª›¯·˜, ¶. ¡ÈÎÔÏ·˝‰Ô˘-∫·Ú·ı›Ô˘

EΉfiÙ˘ K. °ÚÈ‚¤·˜

∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™

™˘ÓÙÔÓÈÛÙ‹˜ ∂ΉfiÛˆ˜

352 ™‡Ó‰ÚÔÌÔ Guillain-Barré. ŒÎ‚·ÛË Ù˘ ÓfiÛÔ˘ Û ·È‰È¿ Ô˘ ÓÔÛËχÙËÎ·Ó Û ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £ÂÚ·›·˜ ∞. ∆ÛÔ‡ÙÛÔ˘, ¢. °ÎÈÒÓ˘, ™. ª·ÛÙÚÔÁÈ¿ÓÓË, π. ¶··‰¿ÙÔ˜

∂¶π∫∞πƒO £∂ª∞ 377 ∏ Â›‰Ú·ÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ô˘ ¯ÔÚËÁÔ‡ÓÙ·È Î·Ù¿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô ÛÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ∂. ªÔ‡˙·, ∫. ¶··Á·ÚÔ˘Ê¿Ï˘

∂π¢π∫O ∞ƒ£ƒO E¶I™THMONIKE™ EK¢O™EI™ E.¶.E. ∞ÚÙ¤ÌȉԘ 3 151 25 M·ÚÔ‡ÛÈ TËÏ.: 210 87 78 810 Fax: 210 87 78 822 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

358 ∫›Ó‰˘ÓÔ˜ ·fi ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ·ÙfiÌˆÓ Ì ۇӉÚÔÌÔ Down Î·È ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ· Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡-§Ô˘Î›‰Ô˘, ª. ª·ÓÙÔ˘Ú¿ÎË, ¶. ∫·ÌÔ˘Ú›‰Ô˘, ∂. ™ˆÙËÚ›‰Ô˘

386 ¶ÚÔÏËÙÈÎfi˜ ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Î·È ı¤Ì·Ù· ËıÈ΋˜ Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡-§Ô˘Î›‰Ô˘

395 ∂¶π™∆√§∏ ¶ƒ√™ ∆∏ ™À¡∆∞•∏

396 ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√

398 µπµ§π√¶∞ƒ√À™π∞™∏

Pediatr Sept-Oct 05

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Paediatriki Volume 68 ñ Number 5 ñ September-October 2005 Bimonthly Publication of the Greek Paediatric Society President A. Constantopoulos Editorial Board Editor C. Stefanidis Members P. Avgoustidou-Savvopoulou H. Antonopoulou A. Vazeou-Gerasimidi G. Varlamis M. Kanariou ∂. Katsarou-Pectasides A. Kattamis Ch. Costalos N. Papadopoulos ∞. Papadopoulou A. Siamopoulou-Mavridou M. Tsolia-Pasparaki Manuscript Editing Greek Editing I. Karavranou English Editing S. Nakou Publisher

Contents LETTER FROM THE EDITOR

CASE REPORTS

325 Opening Lecture at the 43rd Panhellenic Paediatric Meeting G. D. Babiniotis

371 Juvenile hyaline fibromatosis.

The first case report in Greece O. Papandreou, A. Klimentopoulou, N. Poniros, A. Konstantinidou, A. Xypolita-Zachariadi, N. Rachman, G. Chroussos 376 22q11.2 micro-deletion syndrome in a family with variation in the phenotype.

Prenatal diagnosis A. Andreou, V. Alexandropoulou-Tsikrika, A. Sakellaropoulou, M. Goudesidou, L. Florentin-Arar

REVIEW ARTICLES 336 Pathophysiology of vaso-occlusive crises in sickle cell disease E. Karatza-Xifilidou, M. Athanasiou-Metaxa 341 Evaluation of oral challenges for the diagnosis of drug allergy A. Sofianou-Katsoulis, G. Du Toit, G. Lack

CURRENT ISSUE 351 Fibromyalgia in childhood V. Tsagris, A. Kitsopoulou, C. Mihas, P. Nikolaidou-Karpathiou

ORIGINAL ARTICLES

K. Griveas Publishing Coordinator SCIENTIFIC PUBLICATIONS Ltd 3 Artemidos str.

GR - 151 25, Maroussi Tel.: +30 210 87 78 810 Fax: +30 210 87 78 822 Owner Greek Paediatric Society© 92 Michalakopoulou str.

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

385 The effect of postnatal administration of steroids on the developing brain H. Bouza, C. Papagaroufalis

SPECIAL ARTICLE 357 Severe Guillain-Barré syndrome.

Outcome in children admitted to a PICU A. Tsoutsou, D. Gionis, S. Mastroyianni, J. Papadatos 365 Risks of sports activities in individuals with Down syndrome and atlantoaxial instability H. Hatzissevastou-Loukidou, M. Badouraki, P. Kambouridou, E. Sotiridou

394 Genetic preventive testing and ethical issues H. Hatzissevastou-Loukidou

395 LETTER TO THE EDITOR

396 NEWS FROM THE INTERNET

398 BOOK REVIEW

Pediatr Sept-Oct 05

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√¢∏°π∂™ ¶ƒ√™ ∆√À™ ™À°°ƒ∞º∂π™ A. °ÂÓÈΤ˜ ¶ÏËÚÔÊÔڛ˜ ∆Ô ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ΋” Â›Ó·È Ë ÂÈÛÙËÌÔÓÈ΋ ¤Î‰ÔÛË Î·È È‰ÈÔÎÙËÛ›· Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜, Ô˘ ‰È·Ó¤ÌÂÙ·È ÛÙ· ̤ÏË Ù˘. Œ¯ÂÈ ˆ˜ ‚·ÛÈÎÔ‡˜ ÛÙfi¯Ô˘˜ ÙËÓ ·ÔÙ‡ˆÛË ÙÔ˘ ·È‰È·ÙÚÈÎÔ‡ ÂÈÛÙËÌÔÓÈÎÔ‡ ¤ÚÁÔ˘ Î·È ÙË Û˘Ó¯‹ ÂÓË̤ڈÛË ÙˆÓ ¶·È‰È¿ÙÚˆÓ. °È· ÙÔ ÛÎÔfi ·˘Ùfi ‰¤¯ÂÙ·È ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÔÈÎÈÏ›· ¿ÚıÚˆÓ Î·È Û˘ÁÎÂÎÚÈ̤ӷ: 1. ÕÚıÚ· Û‡ÓÙ·Í˘ (ÌÂÙ¿ ·fi ÚfiÛÎÏËÛË Ù˘ ™˘ÓÙ·ÎÙÈ΋˜ EÈÙÚÔ‹˜).

2. ∞Ó·ÛÎÔ‹ÛÂȘ.

3. µÚ·‚Â˘Ì¤Ó˜ ÂÚÁ·Û›Â˜.

4. ∂Ú¢ÓËÙÈΤ˜ ÌÂϤÙ˜.

5. ∫ÏÈÓÈο ∫Ô˘›˙.

6. ∂ÈÏÂÁ̤Ó˜ Û˘˙ËÙ‹ÛÂȘ ÛÙÚÔÁÁ˘ÏÒÓ ÙÚ·Â˙ÒÓ.

7. ∂›Î·ÈÚ· ı¤Ì·Ù·.

8. £¤Ì·Ù· ÂÎ·›‰Â˘Û˘ Î·È ÔÚÁ¿ÓˆÛ˘ ˘Á›·˜.

9. ∂ӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ.

10. ™‡ÓÙÔÌ· Ó¤·.

11. µÚ·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ.

12. ∂ÈÛÙÔϤ˜ ÚÔ˜ ÙË Û‡ÓÙ·ÍË.

13. ¶ÂÚÈÏ‹„ÂȘ Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜.

14. ∞Ó·ÎÔÈÓÒÛÂȘ ÚÔÛ¯ÒÓ Û˘Ó‰ڛˆÓ Î·È ÂÈÛÙËÌÔÓÈÎÒÓ ÂΉËÏÒÛÂˆÓ 15. ∞Ó·ÊÔÚ¤˜ Î·È ÎÚÈÙÈ΋ Ó¤ˆÓ ÂΉfiÛˆÓ, ÂÏÏËÓÈÎÒÓ Î·È Í¤ÓˆÓ, ·È‰È·ÙÚÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜.

H ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ó· ‰ËÌÔÛȇÂÈ ¿ÚıÚ· Ì ȉȷ›ÙÂÚÔ ÂÈÛÙËÌÔÓÈÎfi ÂӉȷʤÚÔÓ, ηıÒ˜ Î·È ¿ÚıÚ· Ì Â›Î·ÈÚ· ı¤Ì·Ù· ¯ˆÚ›˜ Ó· ÙËÚÂ›Ù·È Ë ÛÂÈÚ¿ ˘Ô‚ÔÏ‹˜. ∂›Û˘, ÌÔÚ› Ó· ·ÔÊ·Û›˙ÂÈ ÁÈ· ÙË ‰ËÌÔÛ›Â˘ÛË ÂÚÁ·ÛÈÒÓ Ô˘ ¤¯Ô˘Ó ·ÚÔ˘ÛÈ·ÛÙ› ÛÙÔ ÂÙ‹ÛÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ÙËÓ ÔÏÈ΋ ‹ ÌÂÚÈ΋ ‰ËÌÔÛ›Â˘ÛË ·ÚÔ˘ÛÈ¿ÛÂˆÓ È‰È·›ÙÂÚÔ˘ ÂӉȷʤÚÔÓÙÔ˜ Î·È ÙËÓ ÔÏÈ΋ ‹ ÌÂÚÈ΋ ‰ËÌÔÛ›Â˘ÛË ÂÈÛÙÔÏÒÓ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ‰ËÌÔÛÈÂ˘Ì¤Ó· ÂÈÛÙËÌÔÓÈο ¿ÚıÚ· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ™ÙȘ ÂÚÁ·Û›Â˜ Ì Â›Î·ÈÚ· ı¤Ì·Ù· Ú¤ÂÈ Ó· ·Ó·ÁÚ¿ÊÂÙ·È ÛÙËÓ ÚÒÙË ÛÂÏ›‰· Ë ÚfiıÂÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ÁÈ· ÙËÓ ·Ó·ÁηÈfiÙËÙ· Ù˘ Ù·¯Â›·˜ ‰ËÌÔÛ›Â˘Û˘. ∏ ∂ÈÙÚÔ‹ ™‡ÓÙ·Í˘ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ù˘ ·Ô‰Ô¯‹˜ Ù˘ Ù·¯Â›·˜ ‰ËÌÔÛ›Â˘Û˘. ŸÏ˜ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ÌËÓ ¤¯Ô˘Ó ÂÓ Ì¤ÚÂÈ ‹ ÂÍ ÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚ÏËı› ÁÈ· ÎÚ›ÛË Û ¿ÏÏÔ ÂÚÈÔ‰ÈÎfi. ∂ÈϤÔÓ, ÛÙËÓ ÂÚÁ·Û›· ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢. ŸÏ˜ ÔÈ ÎÏÈÓÈΤ˜ ¤Ú¢Ó˜ Ú¤ÂÈ Ó· ¤¯Ô˘Ó Á›ÓÂÈ ÌÂÙ¿ ·fi ÏËÚÔÊÔÚË̤ÓË Û˘Ó·›ÓÂÛË ÙˆÓ ÌÂÙ¯fiÓÙˆÓ ‹ ÙˆÓ ÓÔÌ›ÌˆÓ ÂÎÚÔÛÒˆÓ ÙÔ˘˜, Û‡Ìʈӷ Ì ÙȘ ‰È·ÎËÚ‡ÍÂȘ ÙÔ˘ EÏÛ›ÓÎÈ Î·È ÙÔ˘ TfiÎÈÔ. E›Û˘, Ú¤ÂÈ Ó· ¤¯Ô˘Ó ÙËÚËı› ÔÈ Ô‰ËÁ›Â˜ ÁÈ· ÙË ÊÚÔÓÙ›‰· ÂÈÚ·Ì·Ùfi˙ˆˆÓ ÙÔ˘ ∂ıÓÈÎÔ‡ πÓÛÙÈÙÔ‡ÙÔ˘ ÀÁ›·˜ ÙˆÓ ∏¶∞ (DHEW Publication, NIH, 80-23). OÈ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Ú¤ÂÈ Ó· ¤¯Ô˘Ó ÂÁÎÚÈı› ·fi ÙËÓ ÂÈÙÚÔ‹ ∏ıÈ΋˜ Î·È ¢ÂÔÓÙÔÏÔÁ›·˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ.

OÈ ·fi„ÂȘ Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ Ô˘ ‰È·Ù˘ÒÓÔÓÙ·È ÛÙȘ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ‰ÂÓ ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ··Ú·›ÙËÙ· ·˘Ù¤˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. H ηٷ¯ÒÚÈÛË ‰È·ÊËÌ›ÛÂˆÓ ÛÙÔ ÂÚÈÔ‰ÈÎfi ‰ÂÓ ˘Ô‰ËÏÒÓÂÈ ÔˆÛ‰‹ÔÙ ¤ÁÎÚÈÛË ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘˜ ·fi ÙËÓ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·, ÙË ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‹ ·fi ÙÔÓ EΉfiÙË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ŸÏ˜ ÔÈ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ıˆÚÔ‡ÓÙ·È È‰ÈÔÎÙËÛ›· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ “¶·È‰È·ÙÚÈ΋” Î·È Ë ÔÏÈ΋ ‹ ÌÂÚÈ΋

xi

·Ó·‰ËÌÔÛ›Â˘Û‹ ÙÔ˘˜ ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ ÌÂÙ¿ ·fi ¤ÁÁÚ·ÊË Û˘ÁηٿıÂÛË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡.

µ. ™‡ÓÙ·ÍË ÙˆÓ ∂ÚÁ·ÛÈÒÓ ŸÏ˜ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ·ÎÔÏÔ˘ıÔ‡Ó “∫ÔÈÓ¤˜ ¶ÚԉȷÁڷʤ˜ ÁÈ· ÃÂÈÚfiÁÚ·Ê· Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È Û µÈÔ˚·ÙÚÈο ¶ÂÚÈÔ‰Èο” (Uniform Requirements for Manuscripts Submitted to Biomedical Journals), ÔÈ Ôԛ˜ ÙÚÔÔÔÈ‹ıËÎ·Ó ÚfiÛÊ·Ù· Î·È ‰ËÌÔÛȇÔÓÙ·È ÛÙȘ ‰È¢ı‡ÓÛÂȘ: http://www.icmje.org Î·È http://www.icmje.org/icmje.pdf TÔ Û‡ÓÔÏÔ Ù˘ ÂÚÁ·Û›·˜ (Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ ÙˆÓ ÈÓ¿ÎˆÓ Î·È ÙˆÓ Ù›ÙÏˆÓ ÙˆÓ ÂÈÎfiÓˆÓ) Ú¤ÂÈ Ó· Â›Ó·È Ù˘ˆÌ¤ÓÔ ÛÙË ÌÈ· fi„Ë Ï¢ÎÔ‡ ¯·ÚÙÈÔ‡ ÌÂÁ¤ıÔ˘˜ A4 (21x29,7 cm), Ì ‰ÈÏfi ‰È¿ÛÙËÌ· ÌÂٷ͇ ÙˆÓ ÁÚ·ÌÌÒÓ Î·È ÂÚÈıÒÚÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 2,5 cm ÛÙȘ ‰‡Ô Ï¢ڤ˜.

TÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ¤¯ÂÈ ÙËÓ ·ÎfiÏÔ˘ıË ‰È¿Ù·ÍË: ÛÂÏ›‰· Ù›ÙÏÔ˘, ‚Ú·¯‡˜ Ù›ÙÏÔ˜, ÂÚ›ÏË„Ë ÛÙ· ÂÏÏËÓÈο Î·È Ù· ·ÁÁÏÈο, ηٿÏÔÁÔ˜ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ, ΛÌÂÓÔ, ¢¯·ÚÈÛٛ˜ ηıÒ˜ Î·È ·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢, ‚È‚ÏÈÔÁÚ·Ê›·, ›Ó·Î˜, ÂÈÎfiÓ˜, Ù›ÙÏÔÈ ÂÈÎfiÓˆÓ. K¿ı ¤Ó· ·fi Ù· ·Ú·¿Óˆ ÛÙÔȯ›· Ú¤ÂÈ Ó· ·Ú¯›˙ÂÈ Û ¯ˆÚÈÛÙfi ʇÏÏÔ Î·È ÔÈ ÛÂÏ›‰Â˜ Ó· ·ÚÈıÌÔ‡ÓÙ·È ‰È·‰Ô¯Èο ·Ú¯›˙ÔÓÙ·˜ Ì ÙË ÛÂÏ›‰· Ù›ÙÏÔ˘.

H ¤ÎÙ·ÛË ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ÁÈ·: ñ ÙȘ ·Ó·ÛÎÔ‹ÛÂȘ Û 2000-3000 ϤÍÂȘ. ñ ÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ Û 1500-2500 ϤÍÂȘ. ñ ÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ Û 1000-1500 ϤÍÂȘ. ñ ÙȘ ÂÈÛÙÔϤ˜ Û 250-500 ϤÍÂȘ.

™ÂÏ›‰· Ù›ÙÏÔ˘ ¶Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ: ñ ÙÔÓ Ù›ÙÏÔ (<14 ϤÍÂȘ), ηıÒ˜ Î·È ÙÔÓ ‚Ú·¯‡ Ù›ÙÏÔ (<5 ϤÍÂȘ) ÙÔ˘ ¿ÚıÚÔ˘. ¢ÂÓ ÂÈÙÚ¤ÔÓÙ·È Û˘ÓÙÌ‹ÛÂȘ. ñ ÙÔ fiÓÔÌ· Î·È ÙÔ ÂÒÓ˘ÌÔ Î¿ıÂ Û˘ÁÁڷʤ· ÛÙËÓ ÔÓÔÌ·ÛÙÈ΋. ñ ÙÔ ÂÈÛÙËÌÔÓÈÎfi ΤÓÙÚÔ (›‰Ú˘Ì·, ÎÏÈÓÈ΋, ÂÚÁ·ÛÙ‹ÚÈÔ) ·fi fiÔ˘ ÚÔ¤Ú¯ÂÙ·È Ë ÂÚÁ·Û›·. ™Â ÂÚ›ÙˆÛË ¤ÏÏÂȄ˘ Û˘ÓÂÚÁ·Û›·˜ ÌÂ Û˘ÁÎÂÎÚÈ̤ӷ ΤÓÙÚ·, Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÂÙ·È Ë È‰ÈfiÙËÙ· ÙÔ˘/ÙˆÓ Û˘ÁÁڷʤ·/ˆÓ (.¯. ȉÈÒÙ˘ ·È‰›·ÙÚÔ˜) Î·È Ô ÙfiÔ˜ ‰È·ÌÔÓ‹˜ ÙÔ˘/ÙÔ˘˜. ñ ÙËÓ Ï‹ÚË ‰È‡ı˘ÓÛË, e-mail Î·È ÙÔ ÙËϤʈÓÔ ÙÔ˘ Û˘ÁÁڷʤ· Ì ÙÔÓ ÔÔ›Ô Á›ÓÂÙ·È Ë ·ÏÏËÏÔÁÚ·Ê›·.

¶ÂÚÈÏ‹„ÂȘ H ÂÚ›ÏË„Ë Ú¤ÂÈ Ó· ·Ó·ÎÂÊ·Ï·ÈÒÓÂÈ ÙÔ˘˜ ÛÙfi¯Ô˘˜ Ù˘ ÂÚÁ·Û›·˜, ÙË ÌÂıÔ‰ÔÏÔÁ›·, Ù· ΢ÚÈfiÙÂÚ· ·ÔÙÂϤÛÌ·Ù· Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ù˘ ÌÂϤÙ˘. ñ £· Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ 200 ϤÍÂȘ, fï˜ ‰ÂÓ ı· Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÂÈ ÙȘ 250 ϤÍÂȘ. ñ ∏ ÂÚ›ÏË„Ë ÙˆÓ ÂÚÁ·ÛÈÒÓ Ú¤ÂÈ Ó· Â›Ó·È ‰ÔÌË̤ÓË ÛÙȘ ÂÍ‹˜ ·Ú·ÁÚ¿ÊÔ˘˜: ÂÈÛ·ÁˆÁ‹, ̤ıÔ‰ÔÈ, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘ÌÂÚ¿ÛÌ·Ù·. ™ÙËÓ ÂÚ›ÏË„Ë ÛÙ· ·ÁÁÏÈο Ú¤ÂÈ Ó· ·Ó·ÁÚ¿ÊÔÓÙ·È ÛÙËÓ ·Ú¯‹ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Ô Ù›ÙÏÔ˜ Ù˘ ÂÚÁ·Û›·˜ Î·È Ù· ÔÓfiÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ ÛÙ· ·ÁÁÏÈο. TÔ ÂÚȯfiÌÂÓÔ ÙÔ˘ ·ÁÁÏÈÎÔ‡ ÎÂÈ̤ÓÔ˘ Ú¤ÂÈ Ó· Â›Ó·È ‰ÔÌË̤ÓÔ ÛÂ: ÂÈÛ·ÁˆÁ‹ (background), ̤ıÔ‰ÔÈ (methods), ·ÔÙÂϤÛÌ·Ù· (results) Î·È Û˘ÌÂÚ¿ÛÌ·Ù· (conclusions). H ÂÚ›ÏË„Ë ÛÙ· ·ÁÁÏÈο ‰ÂÓ Ú¤ÂÈ Ó· ‰È·Ê¤ÚÂÈ ·fi ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ÛÙ· ÂÏÏËÓÈο.

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K¿Ùˆ ·fi ÙËÓ ÂÏÏËÓÈ΋ Î·È ÙËÓ ·ÁÁÏÈ΋ ÂÚ›ÏË„Ë ÛËÌÂÈÒÓÔÓÙ·È ÙÚÂȘ ¤ˆ˜ ¤ÓÙ ϤÍÂȘ ÎÏÂȉȿ Ô˘ ı· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ÁÈ· ÙÔ ıÂÌ·ÙÈÎfi ¢ÚÂÙ‹ÚÈÔ.

K›ÌÂÓÔ OÈ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ÂÈÛ·ÁˆÁ‹, ÌÂıfi‰Ô˘˜, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘˙‹ÙËÛË. H ÂÈÛ·ÁˆÁ‹ ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· ÙÂÏÂ˘Ù·›· ‰Â‰Ô̤ӷ Ù˘ ¤Ú¢ӷ˜ ÛÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ı¤Ì· Ì ÙȘ ΢ÚÈfiÙÂÚ˜ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ Î·È ÙÔÓ ÛÎÔfi Ù˘ ÂÚÁ·Û›·˜. H ÂÚÈÁÚ·Ê‹ ÙˆÓ ÌÂıfi‰ˆÓ Ú¤ÂÈ Ó· Â›Ó·È ·ÎÚÈ‚‹˜ Î·È ·ÚÎÂÙ¿ ÏÂÙÔÌÂÚ‹˜, ÒÛÙ ӷ ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó··Ú·ÁˆÁ‹ ÙÔ˘˜ ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜. ∂›Û˘, Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ì ÏÂÙÔ̤ÚÂȘ ÔÈ ÛÙ·ÙÈÛÙÈΤ˜ ̤ıÔ‰ÔÈ ·Ó¿Ï˘Û˘ Î·È ·ÍÈÔÏfiÁËÛ˘ ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ. T· ·ÔÙÂϤÛÌ·Ù· Ú¤ÂÈ Ó· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ۷ʋÓÂÈ· Î·È Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ÙËÓ ··Ú·›ÙËÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË. H Û˘˙‹ÙËÛË Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙËÓ ÂÚÁ·Û›·, ÙË ÛËÌ·Û›· Ô˘ ÌÔÚ› Ó· ¤¯Ô˘Ó Î·È ÙËÓ Èı·Ó‹ Û˘Û¯¤ÙÈÛ‹ ÙÔ˘˜ Ì ·Ú·ÙËÚ‹ÛÂȘ ¿ÏÏˆÓ ÂÚ¢ÓËÙÒÓ.

OÈ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û‡ÓÙÔÌË ÂÈÛ·ÁˆÁ‹, ÂÚÈÁÚ·Ê‹ Ù˘ ÂÚ›ÙˆÛ˘ Î·È ‚Ú·¯Â›· Û˘˙‹ÙËÛË, Ì ¤ÌÊ·ÛË ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË.

T· ˘fiÏÔÈ· ı¤Ì·Ù· ¤¯Ô˘Ó ÂχıÂÚË ‰ÔÌ‹ ηٿ ÙËÓ ÎÚ›ÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ÙÔ˘˜. ∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢) ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Ù¤ÏÔ˜ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Î·È ÚÈÓ ·fi ÙȘ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜.

Editors/ Uniform Requirements for Manuscripts Sub-mitted to Biomedical Journals, (http://www.icmje.org Î·È http:// www.icmje.org/icmje.pdf). OÈ Û˘ÓÙÌ‹ÛÂȘ ÙˆÓ Ù›ÙÏˆÓ ÙˆÓ ÂÚÈÔ‰ÈÎÒÓ Á›ÓÔÓÙ·È Ì ‚¿ÛË ÙÔ Cumulated Index Medicus [List of Journals Indexed in Index Medicus (http://www.nlm.nih.gov/bsd/uniform_requirements.html)]. ¶·Ú·‰Â›ÁÌ·Ù· ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ I. ¶∂ƒπO¢π∫∞ AÓ ÔÈ Û˘ÁÁÚ·Ê›˜ Â›Ó·È ¤ˆ˜ ¤ÍÈ, ·Ó·ÁÚ¿ÊÔÓÙ·È fiÏÔÈ, ÂÓÒ ·Ó Â›Ó·È ÂÙ¿ ‹ ÂÚÈÛÛfiÙÂÚÔÈ, ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ ÚÒÙÔÈ ¤ÍÈ Î·È ÚÔÛÙ›ıÂÙ·È “et al” ‹ “Î·È Û˘Ó”.

T·ÎÙÈ΋ ¤Î‰ÔÛË ÂÚÈÔ‰ÈÎÔ‡: ∞ÓÙˆÓ›Ô˘ ¢, ª¿Ú· Ã, °È·ÓÓ¿ÙÔ˘ Ã. ∂Ì‚fiÏÈ·: Â›‰Ú·ÛË ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ÙˆÓ ÏÔÈ̈‰ÒÓ ÓÔÛËÌ¿ÙˆÓ Î·È ÙËÓ ·È‰È·ÙÚÈ΋ Ú¿ÍË. ¶·È‰È·ÙÚÈ΋ 1999;59:272-279.

Proesmans W. Bartter syndrome and its neonatal variant. Eur J Pediatr 1997;156:669-679. ™˘ÌÏËڈ̷ÙÈÎfi Ù‡¯Ô˜ ÂÚÈÔ‰ÈÎÔ‡: Flyvbjerg A. Role of growth hormone, insulin-like growth factors (IGFs) and IGF-binding proteins in the renal complications of diabetes. Kidney Int 1997;52 (60 Suppl):S12-S19. Èڛ˜ Û˘ÁÁڷʤ·: National Institutes of Health Consensus Development Conference. Neurofibromatosis conference statement. Arch Neurol 1988;45:575-578.

TÈ̤˜ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ OÈ ÙÈ̤˜ ÙˆÓ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ Ú¤ÂÈ Ó· ÂÎÊÚ¿˙ÔÓÙ·È ÛÙÔ ¢ÈÂıÓ¤˜ ™‡ÛÙËÌ· MÔÓ¿‰ˆÓ (SI Units) Î·È ÛÙÔ MÂÙÚÈÎfi (Conventional-™˘Ì‚·ÙÈÎfi) ™‡ÛÙËÌ· ̤۷ Û ·Ú¤ÓıÂÛË. ¶›Ó·Î˜ ÌÂÙ·ÙÚÔ‹˜ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙȘ ‰È¢ı‡ÓÛÂȘ: http://www.icmje.org Î·È http://www.icmje.org/icmje.pdf

™˘ÓÙÔÌÔÁڷʛ˜ OÈ Û˘ÓÙÔÌÔÁڷʛ˜ Ô˘ ¤¯Ô˘Ó ÂÈ‚ÏËı› ‰ÈÂıÓÒ˜, ‰ËÌÔÛȇÔÓÙ·È Û οı Ù‡¯Ô˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ™‡ÓıÂÙÔÈ Î·È Ì·ÎÚÔÛÎÂÏ›˜ fiÚÔÈ Ô˘ Â·Ó·Ï·Ì‚¿ÓÔÓÙ·È Û˘¯Ó¿ ÛÙÔ Î›ÌÂÓÔ ÌÔÚÔ‡Ó Ó· ·ÓÙÈηı›ÛÙ·ÓÙ·È ·fi Û˘ÓÙÔÌÔÁڷʛ˜ Ô˘ ÂÂÍËÁÔ‡ÓÙ·È ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ Û ÂȉÈÎfi ηٿÏÔÁÔ, Ô ÔÔ›Ô˜ ˘Ô‚¿ÏÏÂÙ·È Ì·˙› Ì ÙËÓ ÂÚÁ·Û›·.

OÈ Û˘ÓÙÔÌÔÁڷʛ˜ ·Ó·Ê¤ÚÔÓÙ·È Û ·Ú¤ÓıÂÛË ÌfiÓÔ ÛÙȘ ÂÚÈÏ‹„ÂȘ.

BÈ‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ ™ÙÔ ÙÌ‹Ì· Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ ηٷ¯ˆÚÔ‡ÓÙ·È fiϘ ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ™ÙÔ Î›ÌÂÓÔ, ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ ·Ó·Ê¤ÚÔÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Û ·Ú¤ÓıÂÛË. OÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ ‰ÂÓ Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÔ˘Ó:

¶ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù‡Ô˘ ¿ÚıÚÔ˘: Schreiner GF, Lange L. Ethanol modulation of macrophage influx in glomerulonephritis [Abstract]. J Am Soc Nephrol 1991;2:562.

Should antileukotriene therapies be used instead of inhaled corticosteroids in asthma? [Editorial]. Am J Respir Crit Care Med 1998;158:1697-1701.

Laux-End R, Inaebnit D, Gerber HA, Bianchetti MG. Vasculitis associated with levamisole and circulating autoantibodies [Letter]. Arch Dis Child 1996;75:355-356.

II. µπµ§π∞ ∫ÂÊ¿Ï·ÈÔ Û ‚È‚Ï›Ô: Clark AG, Barratt TM. Steroid-responsive nephrotic syndrome. In: Barratt TM, Arner ED, Harmon WE, editors.

Pediatric Nephrology. 4th ed. Baltimore: Lippincott William Wilkins; 1999. p. 742. ™‡ÁÁÚ·ÌÌ· ‹ ÌÔÓÔÁÚ·Ê›·: Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck. 3rd ed. New York: Oxford University Press; 1990.

ñ ÙȘ 70 ÛÙȘ ·Ó·ÛÎÔ‹ÛÂȘ. ñ ÙȘ 30 ÛÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜. ñ ÙȘ 12 ÛÙ· Â›Î·ÈÚ· ı¤Ì·Ù· Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ. ñ ÙȘ 5 ÛÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ Î·È ÙȘ ÂÈÛÙÔϤ˜.

¢ËÌÔÛ›Â˘ÛË Û ÙfiÌÔ Ú·ÎÙÈÎÒÓ: Bauer AW. The two definitions of bacterial resistance.

In: Smith AJ, Rogers CA, eds. Proceedings of the Third International Congress of Chemotherapy; 1962 May 29-31; New York: International Society of Chemotherapy; 1963. p.

484-500.

H Û‡ÓÙ·ÍË ÙˆÓ ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ Á›ÓÂÙ·È Û‡Ìʈӷ Ì ÙȘ ÚfiÛÊ·Ù· ÙÚÔÔÔÈË̤Ó˜ ÚԉȷÁڷʤ˜ Ù˘ International Committee of Medical Journal

¢È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹: ¶··‰fiÔ˘ÏÔ˜ Ã. ∏ ıÂÚ·›· ÙÔ˘ ÛÙÚ·‚ÈÛÌÔ‡ [‰È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹]. ∞ı‹Ó·: ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ; 1979.

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Kaplan SJ. Post hospital home health care: the elderly’s access and utilization [dissertation]. St. Louis (Mo): Washington Univ.; 1995. πππ. CD-ROM Anderson SC, Poulsen KB. Anderson’s electronic atlas of hematology [CD-ROM]. Philadelphia: Lippincott Williams & Wilkins; 2002.

IV. ™∆O ¢π∞¢π∫∆ÀO ÕÚıÚÔ Û ÂÚÈÔ‰ÈÎfi: Abood S. Quality improvement initiative in nursing homes: the ANA acts in an advisory role. Am J Nurs [Internet]. 2002 Jun: Webpage: http://www.nursingworld.org/AJN/2002/june/Wawatch.htm ªÔÓÔÁÚ·Ê›·: Foley KM, Gelband H, editors. Improving palliative care for cancer [Monograph, Internet]. Washington: National Academy Press; 2001. Webpage: http://www.nap.edu/books/0309074029/html

¶›Ó·Î˜ Î·È EÈÎfiÓ˜ AÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó (¤Ó· ÚˆÙfiÙ˘Ô Î·È ‰‡Ô ʈÙÔ·ÓÙ›ÁÚ·Ê·). ¶Ú¤ÂÈ Ó· ¤¯Ô˘Ó Ï¿ÙÔ˜ ›ÛÔ Ì ÙÔ Ï¿ÙÔ˜ ÙÔ˘ ÌÔÓfiÛÙËÏÔ˘ (7,5 cm) ‹ Ì ÙÔ Ï¿ÙÔ˜ fiÏ˘ Ù˘ ÛÂÏ›‰·˜ (15,5 cm). TÔ Ì¤ÁÈÛÙÔ Ì‹ÎÔ˜ ÙÔ˘˜, Ì·˙› Ì ÙÔ˘˜ Ù›ÙÏÔ˘˜, ‰ÂÓ Ú¤ÂÈ Ó· Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ·fi 22 cm.

OÈ ›Ó·Î˜ ·ÚÈıÌÔ‡ÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ¶ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‚Ú·¯‡ Ù›ÙÏÔ Î·È ÂÂÍ‹ÁËÛË ÙˆÓ Û˘ÓÙÌ‹ÛÂˆÓ ÛÙÔ Î¿Ùˆ ̤ÚÔ˜. ™ÙÔ˘˜ ›Ó·Î˜ Ú¤ÂÈ Ó· ·ÔʇÁÔÓÙ·È ÔÈ Î¿ıÂÙ˜ ÁÚ·Ì̤˜. ŸÏÔ ÙÔ ·ÂÈÎÔÓÈÛÙÈÎfi ˘ÏÈÎfi (Û¯‹Ì·Ù·, ‰È·ÁÚ¿ÌÌ·Ù·, ʈÙÔÁڷʛ˜, Î.Ï.) ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ÂÈÎfiÓ˜ Î·È ı· Ú¤ÂÈ Ó· Â›Ó·È ¿ÚÈÛÙ˘ ÔÈfiÙËÙ·˜. ™ÙÔ ›Ûˆ ̤ÚÔ˜ ÙˆÓ ÂÈÎfiÓˆÓ Ú¤ÂÈ Ó· ÛËÌÂÈÒÓÂÙ·È Ì ÌÔχ‚È Ô ·ÚÈıÌfi˜ Ù˘ ÂÈÎfiÓ·˜ Î·È ÙÔ fiÓÔÌ· ÙÔ˘ ÚÒÙÔ˘ Û˘ÁÁڷʤ·, ηıÒ˜ Î·È ¤Ó· ‚¤ÏÔ˜ ÙÔ ÔÔ›Ô Ó· ‰Â›¯ÓÂÈ ÙÔ ¿Óˆ ̤ÚÔ˜ Ù˘ ÂÈÎfiÓ·˜. ™ÙȘ ʈÙÔÁڷʛ˜ ·ÛıÂÓÒÓ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·ÁÓˆÚ›˙ÂÙ·È Ë Ù·˘ÙfiÙËÙ¿ ÙÔ˘˜. OÈ ·ÛıÂÓ›˜ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È ÔÓÔÌ·ÛÙÈο.

πÛÙÔÛÂÏ›‰Â˜: Cancer-Pain.org [Webpage, Internet]. New York: Association of Cancer Online Resources, Inc.; 2002: http://www.cancer-pain.org/

°. ÀÔ‚ÔÏ‹ Î·È ¢ËÌÔÛ›Â˘ÛË ÙˆÓ ∂ÚÁ·ÛÈÒÓ ŸÏ˜ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ˘Ô‚¿ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó Î·È Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ‰ÈÛΤٷ ‹ CD, ηıÒ˜ Î·È ·fi ÂÈÛÙÔÏ‹, Ë ÔÔ›· ı· Û˘Ó˘ÔÁÚ¿ÊÂÙ·È ·fi fiÏÔ˘˜ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜, ÛÙËÓ ÔÔ›· ı· ‰ËÏÒÓÂÙ·È fiÙÈ Ë ÂÚÁ·Û›· ‰ÂÓ ¤¯ÂÈ ÂÓ Ì¤ÚÂÈ ‹ ÂÍ ÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚ÏËı› ÁÈ· ÎÚ›ÛË Û ¿ÏÏÔ ÂÚÈÔ‰ÈÎfi Î·È fiÙÈ ÔÈ Û˘ÁÁÚ·Ê›˜ ÂÁÎÚ›ÓÔ˘Ó ÙË ‰ËÌÔÛ›Â˘Û‹ Ù˘ ÛÙÔ ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ΋”. ∂ÈϤÔÓ, ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢.

EÊfiÛÔÓ Ë ÂÚÁ·Û›· Á›ÓÂÈ ·Ô‰ÂÎÙ‹, ÙÔ ‰ÈÔÚıˆÌ¤ÓÔ Û‡Ìʈӷ Ì ÙȘ ˘ԉ›ÍÂȘ ÙˆÓ ÎÚÈÙÒÓ Î·È ÂÎ Ó¤Ô˘ ‰·ÎÙ˘ÏÔÁÚ·ÊË̤ÓÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ˘Ô‚¿ÏÂÙ·È ÛÙË ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹, Û˘Óԉ¢fiÌÂÓÔ ·fi ‰ÈÛΤٷ ‹ CD Ô˘ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÚÁ·Û›· Û ÚfiÁÚ·ÌÌ· Word, ηıÒ˜ Î·È ÂÈÛÙÔÏ‹ fiÔ˘ Ó· ·Ó·Ê¤ÚÔÓÙ·È ·Ó·Ï˘ÙÈο ÔÈ ÙÚÔÔÔÈ‹ÛÂȘ ‹ ÔÈ ·ÓÙÈÚÚ‹ÛÂȘ ÛÙȘ ÚÔÙ¿ÛÂȘ ÙˆÓ ÎÚÈÙÒÓ.

H ηı˘ÛÙ¤ÚËÛË Ù˘ Â·Ó˘Ô‚ÔÏ‹˜ ÙÔ˘ ÙÚÔÔÔÈË̤ÓÔ˘ ÎÂÈ̤ÓÔ˘ ¤Ú·Ó ÙˆÓ 30 ËÌÂÚÒÓ Û˘ÓÂ¿ÁÂÙ·È Ó¤· ˘Ô‚ÔÏ‹.

T· ¤ÍÔ‰· ÙˆÓ ÊÈÏÌ Î·È ÙˆÓ ·Ó·Ù‡ˆÓ ‚·Ú‡ÓÔ˘Ó ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ Î·È Ú¤ÂÈ Ó· ÏËÚÒÓÔÓÙ·È Ì ÙËÓ ·ÔÛÙÔÏ‹ ÙÔ˘ ÚÒÙÔ˘ ‰ÔÎÈÌ›Ô˘ ηÙ¢ı›·Ó ÛÙÔÓ Ù˘ÔÁÚ¿ÊÔ.

T· ΛÌÂÓ· ÙˆÓ ÂÚÁ·ÛÈÒÓ Ô˘ ‰ÂÓ Á›ÓÔÓÙ·È ·Ô‰ÂÎÙ¤˜ ÁÈ· ‰ËÌÔÛ›Â˘ÛË, ‰ÂÓ ÂÈÛÙÚ¤ÊÔÓÙ·È. MÔÚ› Ó· ÂÈÛÙÚ·ÊÔ‡Ó, ÂÊfiÛÔÓ ˙ËÙËı› ÂÓÙfi˜ ÂÍ·Ì‹ÓÔ˘, Ù· Û¯‹Ì·Ù· Î·È ÔÈ ÊˆÙÔÁڷʛ˜ Ô˘ Ù· Û˘Óԉ‡ԢÓ.

OÈ ÂÚÁ·Û›Â˜ Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È ÚÔ˜ ÎÚ›ÛË ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÛÙÔ ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ΋” ·ÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó ÛÙË ‰È‡ı˘ÓÛË: ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›· Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 115 28 Aı‹Ó·

¶ÚÈÓ ·ÔÛÙ›ÏÂÙ ÙËÓ ÂÚÁ·Û›· Û·˜, ‚‚·Èˆı›Ù fiÙÈ ÂÚÈÏ·Ì‚¿ÓÂÈ: 1. 3 ·ÓÙ›ÁÚ·Ê· ÙÔ˘ ÎÂÈ̤ÓÔ˘ Ù˘ ÂÚÁ·Û›·˜, Ù˘ˆÌ¤Ó· Û‡Ìʈӷ Ì ÙȘ Ô‰ËÁ›Â˜.

2. ¢ÈÛΤٷ ‹ CD Ì fiÏÔ ÙÔ ˘ÏÈÎfi Ù˘ ÂÚÁ·Û›·˜ (ΛÌÂÓÔ, ›Ó·Î˜, ÂÈÎfiÓ˜).

3. ∂ÈÛÙÔÏ‹ ˘Ô‚ÔÏ‹˜ Î·È ‰‹ÏˆÛË ÌË ÚÔÁÂÓ¤ÛÙÂÚ˘ ‰ËÌÔÛ›Â˘Û˘ Ù˘ ÂÚÁ·Û›·˜.

4. ™ÂÏ›‰· Ù›ÙÏÔ˘ (¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂÚÈÏ·Ì‚¿ÓÂÈ: ·.Ù›ÙÏÔ Î·È ‚Ú·¯‡ Ù›ÙÏÔ ÂÚÁ·Û›·˜ ‚.fiÓÔÌ· Î·È ÂÒÓ˘ÌÔ Û˘ÁÁڷʤˆÓ (ÔÏÔÁڿʈ˜) Á. ÂÈÛÙËÌÔÓÈÎfi/¿ ΤÓÙÚÔ/· fiÔ˘ ¤ÁÈÓÂ Ë ÂÚÁ·Û›· ‰.fiÓÔÌ·, ‰È‡ı˘ÓÛË Î·È ÙËϤʈÓÔ Û˘ÁÁڷʤ· ÁÈ· ·ÏÏËÏÔÁÚ·Ê›·.

5. ¶ÂÚ›ÏË„Ë ÂÏÏËÓÈ΋-·ÁÁÏÈ΋, ‰ÔÌË̤ÓË ÛÙȘ ÂÍ‹˜ ·Ú·ÁÚ¿ÊÔ˘˜: ÂÈÛ·ÁˆÁ‹, ̤ıÔ‰ÔÈ, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘ÌÂÚ¿ÛÌ·Ù·, ηıÒ˜ Î·È Ï¤ÍÂȘ ÎÏÂȉȿ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).

6. ∫·Ù¿ÏÔÁÔ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).

7. ∫›ÌÂÓÔ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).

8. ∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢).

9. µÈ‚ÏÈÔÁÚ·Ê›· (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).

10. ¶›Ó·Î˜ (Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó.

11. ∂ÈÎfiÓ˜, Ì ‚¤ÏÔ˜ ÛÙÔ ›Ûˆ ̤ÚÔ˜ ÙÔ ÔÔ›Ô Ó· ‰Â›¯ÓÂÈ ÚÔ˜ Ù· Â¿Óˆ, ·ÚÈıÌË̤Ó˜, ÂȘ ÙÚÈÏÔ‡Ó.

12. ∆›ÙÏÔ˘˜ ÂÈÎfiÓˆÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó.

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™À¡∆√ª√°ƒ∞ºπ∂™ o

A cal cm cm2 cm3 o C g h IU kg l m mg min mm mol n NS ‹ ª™ osm p SD SE sec U

angstrom ıÂÚÌ›‰· ÂηÙÔÛÙfi ÙÂÙÚ·ÁˆÓÈÎfi ÂηÙÔÛÙfi ΢‚ÈÎfi ÂηÙÔÛÙfi ‚·ıÌfi˜ ∫ÂÏÛ›Ô˘ ÁÚ·ÌÌ¿ÚÈÔ ÒÚ· ‰ÈÂıÓ‹˜ ÌÔÓ¿‰· ¯ÈÏÈfiÁÚ·ÌÌÔ Ï›ÙÚÔ Ì¤ÙÚÔ ¯ÈÏÈfiÁÚ·ÌÌÔ ÏÂÙfi ¯ÈÏÈÔÛÙfiÌÂÙÚÔ ÁÚ·ÌÌÔÌfiÚÈÔ ·ÚÈıÌfi˜ ÌË ÛËÌ·ÓÙÈÎfi ÔÛÌÒÏÈÔ Èı·ÓfiÙËÙ· ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË ÛÙ·ıÂÚfi ÛÊ¿ÏÌ· ‰Â˘ÙÂÚfiÏÂÙÔ ÌÔÓ¿‰·

™˘Ó‰˘·˙fiÌÂÓ· ÚÔı¤Ì·Ù· 12

tera- (10 ) giga- (109) mega- (106) kilo- (103) hector- (102) deca- (101) deci- (10-1) centi- (10-2) milli- (10-3) micro- (10-6) nano- (10-9) pico- (10-12) femto- (10-15) atto- (10-18)

ABBREVIATIONS

Combining prefixes T G M k h da d c m Ì n p f a

angstrom calorie centimeter square centimeter cubic centimeter degree Celsius gram hour international unit kilogram liter meter milligram minute millimeter mole number not significant osmole probability standard deviation standard error second unit

”

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INSTRUCTIONS TO AUTHORS A. General Information The Greek Paediatric Society is the owner of “Paediatriki”, its official scientific journal, which is distributed to its members. Its objectives are the publication of paediatric scientific work and the continuing education of paediatricians. For this purpose, it publishes a variety of articles, and in particular: 1. Editorials (upon invitation by the Editorial Board).

2. Review articles.

3. Award-winning articles.

4. Original articles.

5. Clinical Quiz.

6. Round tables.

7. Current issues.

8. Issues of healthcare management and education.

9. Case reports.

10. News.

11. Brief reports.

12. Letters to the editor.

13. Abstracts.

14. Future congresses and events.

15. Book reviews.

The Editorial Board reserves the right to publish articles of special scientific interest and articles on current issues without observing submission order. In addition it publishes upon decision original papers presented at the Annual Paediatric Conference, presentations of special interest - in whole or in part, and letters - in whole or in part - referring to scientific articles published in the journal.

Regarding papers on current issues, the author’s request for immediate publication should be quoted on the first page. The Editorial Board reserves the right to accept such papers for immediate publication.

All manuscripts should not have been published previously, in whole or in part, and not be under consideration by another publication. Manuscripts should acknowledge any funding, sponsorship or other financial support. All clinical research should have been conducted following informed consent of participants or of their legal representatives according to the Declarations of Helsinki and Tokyo.

In addition, the US National Institute of Health guide for the care and use of laboratory animals (DHEW Publication, NIH, 80-23) should have been observed. Clinical trials should have been approved by the Ethics Committee of the Hospital.

Authors’ opinions and conclusions expressed in the published papers do not necessarily reflect those of the journal. The Greek Paediatric Society, the Editorial Board and the Publisher of the journal do not necessarily approve the content of the advertisements appearing in the journal.

The copyright of all published papers is held by “Paediatriki” and their reproduction in whole or in part is authorized only following written consent of the journal.

B. Manuscript Preparation “Paediatriki” suggests compliance with the “Uniform Requirements for Manuscripts Submitted to Biomedical Journals”, recently modified and published on the websites: http://www.icmje.org and http://www.icmje.org/icmje.pdf The entire paper (including figure legends and tables)

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should be typed on one side of blank paper format A4 (21x29.7 cm), double line spacing and minimum indent 2.5 cm on both sides.

The paper should have the following structure: title page, short title, abstract in Greek and English, list of abbreviations, text, acknowledgements and quoting of grants, sponsorships or other financial support sources, references, tables, figures, figure legends. Each of these sections should be started on a new page. Pages should be numbered consecutively, beginning with the title page.

Text length shall be: ñ review articles 2000-3000 words; ñ original articles and case reports 1500-2500 words; ñ brief reports1000-1500 words; ñ letters 250-500 words.

The title page should include: ñ the title (<14 words) and the short title (<5 words) of the article. No abbreviations are permitted in the title; ñ the name and surname of all authors; ñ the centre (institution, clinic, laboratory) of origin of the paper. If there is no affiliation with a specific centre, the status of the author(s) should be cited (e.g., private paediatrician) and home address; ñ the complete address, e-mail and telephone number of the author to whom correspondence should be addressed.

Abstracts The abstract should summarize the objectives, methodology, main results and conclusions of the study. ñ It should contain at least 200 words, and not exceed 250 words. ñ It should consist of the following paragraphs: background, methods, results and conclusions.

The English abstract should cite at the beginning the title of the paper and the authors’ names in English. The content of the text should consist of the following paragraphs: background, methods, results and conclusions. The abstract in English should not differ in content from the corresponding Greek abstract.

Beneath the Greek and English abstracts, three to five key words in the respective language should be supplied, to be used in the thematic index.

Text Original articles include: introduction, methods, results and discussion. The introduction includes the latest research data on the subject and the main references and the objectives of the paper. The description of the methods should be precise and detailed so as to enable reproduction by other researchers. In addition, the statistical methods of analysis and evaluation of the results should be described.

Results should be presented clearly, together with the appropriate statistical analysis. Discussion should cover the results ensuing from the research, their significance and possible associations with the observations of other researchers.

Case reports comprise a short introduction, case description and brief discussion, with emphasis on differential diagnosis.

The structure of all other articles is free, according to the judgment of the authors.

Thanks or acknowledgements (reference to grants, sponsorships or other sources of financial support) should be quoted at the end of the text, before references.

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Units of measures of laboratory analyses Laboratory analyses should be expressed in the Système International (SI) units and in the metric (Conventional) system in parentheses. See conversion tables on the websites: http://www.icmje.org and http://www.icmje.org/icmje.pdf

II. BOOKS Chapter in book: Clark AG, Barratt TM. Steroid-responsive nephrotic syndrome. In: Barratt TM, Arner ED, Harmon WE, editors.

Pediatric Nephrology. 4th ed. Baltimore: Lippincott William Wilkins; 1999. p. 742.

Abbreviations All issues of the journal contain internationally established abbreviations. Complex or long terms often repeated in the text may be replaced by abbreviations explained by the authors in a list submitted with the paper. Abbreviations are reported in parentheses only in abstracts.

Book or monograph: Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck. 3rd ed. New York: Oxford University Press; 1990.

References The reference section contains all references numbered in the order in which they appear in the text. In the text, references are to be indicated by Arabic numerals in parentheses. References should be no more than: ñ 70 in review articles; ñ 30 in original articles; ñ 12 in current issues and case reports; ñ 5 in brief reports and letters.

In listing references follow the recently modified standards of the International Committee of Medical Journal Editors/Uniform Requirements for Manuscripts Submitted to Biomedical Journals, (http://www.icmje.org and http:// www.icmje.org/icmje.pdf). Abbreviated names of journals should conform to the Cumulated Index Medicus [List of Journals Indexed in Index Medicus (http://www.nlm.nih.gov/bsd/uniform_requirements.html)].

Examples of reference style I. JOURNALS All authors are cited if they are six or less; if they are 7 or more, the first six are cited, followed by “et al”.

Regular edition: Proesmans W. Bartter syndrome and its neonatal variant. Eur J Pediatr 1997;156:669-679.

Supplement issue: Flyvbjerg A. Role of growth hormone, insulin-like growth factors (IGFs) and IGF-binding proteins in the renal complications of diabetes. Kidney Int 1997;52 (60 Suppl):S12-S19.

No author: National Institutes of Health Consensus Development Conference. Neurofibromatosis conference statement. Arch Neurol 1988;45:575-578.

Article type specification: Schreiner GF, Lange L. Ethanol modulation of macrophage influx in glomerulonephritis [Abstract]. J Am Soc Nephrol 1991;2:562.

Should antileukotriene therapies be used instead of inhaled corticosteroids in asthma? [Editorial]. Am J Respir Crit Care Med 1998;158:1697-1701.

Laux-End R, Inaebnit D, Gerber HA, Bianchetti MG. Vasculitis associated with levamisole and circulating autoantibodies [Letter]. Arch Dis Child 1996;75:355-356.

Publication in a volume of proceedings: Bauer AW. The two definitions of bacterial resistance.

In: Smith AJ, Rogers CA, eds. Proceedings of the Third International Congress of Chemotherapy; 1962 May 29-31; New York: International Society of Chemotherapy; 1963. p.

484-500.

Doctoral dissertation: Kaplan SJ. Post hospital home health care: the elderly’s access and utilization [dissertation]. St. Louis (Mo): Washington Univ.; 1995. πππ. CD-ROM Anderson SC, Poulsen KB. Anderson’s electronic atlas of hematology [CD-ROM]. Philadelphia: Lippincott Williams & Wilkins; 2002.

IV. ON THE INTERNET Article in journal Abood S. Quality improvement initiative in nursing homes: the ANA acts in an advisory role. Am J Nurs [Internet]. 2002 Jun: Webpage: http://www.nursingworld.org/ AJN/2002/june/Wawatch.htm Monograph Foley KM, Gelband H, editors. Improving palliative care for cancer [Monograph, Internet]. Washington: National Academy Press; 2001. Webpage: http://www.nap.edu/books/0309074029/html Websites Cancer-Pain.org [Webpage, Internet]. New York: Association of Cancer Online Resources, Inc.; 2002: http://www.cancer-pain.org/ Tables and Figures Three copies should be submitted (original plus 2 copies). Their width should either be equal to the width of one column (7.5 cm) or to the width of the page (15.5 cm).

Their maximum length, titles included, should not exceed 22 cm.

Tables are numbered with Arabic numerals in the order in which they appear in the text. They should have a short title and abbreviations should be listed at the bottom. Vertical lines in tables should be avoided.

All illustration material is considered as figures (graphs, pictures, etc.). They should be of excellent quality. Also, at the back of every picture, the number of the picture and the name of the first author should be noted in pencil, with an arrow showing the top of the picture. The identity of patients should not be recognizable from their pictures nor should their names be stated.

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C. Manuscript Submission and Publication All manuscripts should be accompanied by a floppy disk or CD, as well as by a letter, signed by all the authors, in which it is stated that the paper has not been published in part or in whole, or is not under consideration by another journal and that the authors accept its publication in “Paediatriki”. Any funding, sponsorship or other financial support should be acknowledged.

Once the manuscript has been accepted, the corrected version, rewritten according to the reviewers’ recommendations should be submitted to the Editorial Board accompanied by a floppy disk or CD, containing the paper in Word format, along with a covering letter specifying in detail the modifications or objections to the reviewers’ suggestions.

Delay in submission of the modified paper exceeding 30 days entails new submission.

Authors will be charged film and reprint expenses, paid upon dispatch of the first proof directly to the printer.

Manuscripts of papers which have not been approved for publication are not returned to the author. The accompanying figures and photographs can be returned upon request within six months.

Manuscripts submitted for review and publication in “Paediatriki” should be sent in three copies to the following address: Editorial Board Greek Paediatric Society 92, Michalakopoulou Street 115 28 Athens, Greece

Before submitting your paper, make sure it contains: 1. 3 copies of the text of the paper, printed according to instructions.

2. A floppy disk or CD with the entire material of the paper (text, tables, pictures).

3. A covering letter and a statement that the paper has not been previously published.

4. The title page (on a separate page), which includes: a. the title and short title of the paper; b. the name and surname (full name)of the author(s); c. the academic centre(s) of origin; d. the corresponding authors’ name, address and telephone number; 5. English and Greek abstracts, with the following structure: background, methods, results and conclusions (double space, separate page) and keywords.

6. List of abbreviations (double space, separate page).

7. Text (double space, separate page).

8. Acknowledgements and reference to funding, sponsorships or other financial sources.

9. References (double space, separate page).

10. Tables (one per page) in three copies.

11. Figures with an arrow at the back showing the top, numbered, in two copies.

12. Figure titles (double space - on separate pages) in three copies.

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∂¶π™∆√§∏ ∞¶√ ∆∏ ™À¡∆∞•∏

325

™ÙÔ 43Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ Â›¯·Ì ÙËÓ ÙÈÌ‹ Î·È ÙË ¯·Ú¿ Ó· ·Ú·ÎÔÏÔ˘ı‹ÛÔ˘Ì ÙËÓ ÔÌÈÏ›· ÙÔ˘ ¶Ú‡Ù·ÓË ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ∫·ıËÁËÙ‹ °ÂˆÚÁ›Ô˘ ª·ÌÈÓÈÒÙË Ì ı¤Ì·: “∂ÏÏËÓÈ΋ ÁÏÒÛÛ·. ∏ Û˘Ì‚ÔÏ‹ Ù˘ Û ‚·ÛÈΤ˜ ¤ÓÓÔȘ ÙÔ‡ ∂˘Úˆ·˚ÎÔ‡ ÔÏÈÙÈÛÌÔ‡”. ∞ÓÙ·ÔÎÚÈÓfiÌÂÓÔÈ ÛÙÔ ·›ÙËÌ· ÔÏÏÒÓ Û˘Ó·‰¤ÏʈÓ, ‰ËÌÔÛȇԢÌ ¤Ó· ·fiÛ·ÛÌ· Ù˘ ÔÌÈÏ›·˜ ÙÔ˘ ∫·ıËÁËÙ‹, Ô˘ ÈÛÙ‡ԢÌ fiÙÈ ·ÔÙÂÏ› ¿ÓÙ· ¤Ó· Â›Î·ÈÚÔ Î·È ¯Ú‹ÛÈÌÔ ı¤Ì·. ªÂ ÙËÓ Â˘Î·ÈÚ›· ·˘Ù‹ ı· ı¤Ï·Ì ӷ ¢¯·ÚÈÛÙ‹ÛÔ˘Ì ÙÔÓ ∫·ıËÁËÙ‹, ÁÈ·Ù› Ì·˜ ‰È¤ıÂÛ ÙÔ Î›ÌÂÓÔ Ù˘ ÔÌÈÏ›·˜ ÙÔ˘. °È· ÙË ™˘ÓÙ·ÎÙÈ΋ ∂ÈÙÚÔ‹ ∫ˆÓÛÙ·ÓÙ›ÓÔ˜ ™ÙÂÊ·Ó›‰Ë˜ ¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘

∂¡∞ƒ∫∆∏ƒπO™ Oªπ§π∞ ÛÙÔ 43Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ ∂ÏÏËÓÈ΋ ÁÏÒÛÛ· ∏ Û˘Ì‚ÔÏ‹ Ù˘ Û ‚·ÛÈΤ˜ ¤ÓÓÔȘ ÙÔ‡ ∂˘Úˆ·˚ÎÔ‡ ÔÏÈÙÈÛÌÔ‡ °ÂÒÚÁÈÔ˜ ¢. ª·ÌÈÓÈÒÙ˘

∫¿ı ·ÓıÚÒÈÓË ÁÏÒÛÛ· Â›Ó·È ÌÈ· ÌÔÚÊ‹ Ù·ÍÈÓfiÌËÛ˘ ÙÔ‡ ÎfiÛÌÔ˘. ∫¿ı ÂıÓÈ΋ ÁÏÒÛÛ·, Ë ÁÏÒÛÛ· Ô˘ ÌÈÏ¿ÂÈ ¤Ó· ¤ıÓÔ˜ Â›Ó·È Ô ÙÚfiÔ˜ Ô˘ Û˘ÏÏ·Ì‚¿ÓÂÈ, ÂÂÍÂÚÁ¿˙ÂÙ·È Î·È Ù·ÍÈÓÔÌ› ÙÔÓ ÎfiÛÌÔ Ô Ï·fi˜ Ù‹˜ ¯ÒÚ·˜ Ô˘ ÌÈÏ¿ÂÈ ·˘Ù‹ ÙË ÁÏÒÛÛ·. ŒÙÛÈ, οı ÁÏÒÛÛ· - fiˆ˜ ÙÔ ¤¯ÂÈ ‰È‰¿ÍÂÈ Ô Saussure - Â›Ó·È Î·È ÌÈ· ¿ÏÏË Ù·ÍÈÓfiÌËÛË ÙÔ‡ ÎfiÛÌÔ˘. ŒÓ·˜ ¿ÏÏÔ˜ ÙÚfiÔ˜ Ó· ‚ϤÂȘ, Ó· ·ÈÛı¿ÓÂÛ·È Î·È Ó· ‚¿˙ÂȘ Û ٿÍË ÙÔÓ ÎfiÛÌÔ. ∫È ·˘Ùfi Á›ÓÂÙ·È Ì ÁψÛÛÈο ̤۷. ªÂ ÙÔÓ ÙÚfiÔ Ô˘ ¤Ó·˜ Ï·fi˜ Έ‰ÈÎÔÔÈ› ÁψÛÛÈο ÙȘ ¤ÓÓÔȘ Û ÛË̷ۛ˜, ·˘Ù¤˜ Ô˘ ÂÈϤÁÂÈ Î¿ı Ϸfi˜ ÁÈ· Ó· ÂÎÊÚ¿˙ÂÈ ÙȘ ¤ÓÓÔȘ Ô˘ ¯ÚÂÈ¿˙ÂÙ·È. ŒÙÛÈ, Ë ÁψÛÛÈ΋ ‰È·‰Èηۛ· Â›Ó·È ¿ÌÂÛË Û˘Ó¿ÚÙËÛË Ù‹˜ ÛΤ„˘ Î·È Ù‹˜ ÓÔÔÙÚÔ›·˜ οı Ϸԇ. ∞˜ ԇ̠·fi ÙÒÚ· ÌÈ· ¿ÏÏË ‚·ÛÈ΋ ı¤ÛË: O ¿ÓıÚˆÔ˜ ÛΤÙÂÙ·È Ì¤Û· ·fi ÙȘ ϤÍÂȘ Î·È Ì¤Û· ·fi ÙȘ ÁÚ·ÌÌ·ÙÈΤ˜ ‰Ô̤˜ Î·È ÛË̷ۛ˜ Ì ÙȘ Ôԛ˜ Â›Ó·È ÔÚÁ·ÓˆÌ¤ÓË Ë ÁÏÒÛÛ· ÙÔ˘. øÛÙfiÛÔ, ·˘Ùfi˜ Ô “ÁψÛÛÈÎfi˜ ÓÙÂÙÂÚÌÈÓÈÛÌfi˜”, ‰ÂÓ ÛËÌ·›ÓÂÈ fiÙÈ Ô ¿ÓıÚˆÔ˜ Â›Ó·È Î·È “‰Ô‡ÏÔ˜” Ù‹˜ ÁÏÒÛÛ·˜ ÙÔ˘, ÁÈ·Ù› ·ÎÚÈ‚Ò˜ Ë ÁÏÒÛÛ· ÙÔ‡ ·ÓıÚÒÔ˘ - fiˆ˜ ¤¯ÂÈ ‰È‰¿ÍÂÈ Ì ¤ÌÊ·ÛË Ô N. Chomsky - ¯·Ú·ÎÙËÚ›˙ÂÙ·È Î·Ù’ ÂÍÔ¯‹Ó ·fi ÌÈ· ȉÈfiÙËÙ·, ÙË ‰ËÌÈÔ˘ÚÁÈÎfiÙËÙ· (creativity). ¢˘Ó¿ÌÂÈ ‰ËÏ. ‰ÂÓ ˘¿Ú¯ÂÈ Î·Ó¤Ó·˜ ÊÚ·ÁÌfi˜ ÛÙË ÁψÛÛÈ΋ ¤ÎÊÚ·ÛË ÙÔ‡ ·ÓıÚÒÔ˘ , Ú¿ÁÌ· Ô˘ ÂȂ‚·ÈÒÓÂÈ Î·È Ô Wittgenstein ϤÁÔÓÙ·˜ ÙÔ ÂÚ›ÊËÌÔ “fi,ÙÈ ÌÔÚÒ Ó· Û˘ÏÏ¿‚ˆ ηı·Ú¿ Ì ÙÔÓ ÓÔ˘ ÌÔ˘, ÌÔÚÒ Ó· ÙÔ ÂÎÊÚ¿Ûˆ ηı·Ú¿ Î·È Ì ÙË ÁÏÒÛÛ· ÌÔ˘”, ÂÓÓÔÒÓÙ·˜ fiÙÈ ‰ÂÓ ÌÔÚԇ̠ӷ ÂÎÊÚ¿ÛÔ˘Ì ÁψÛÛÈο Ì ۷ʋÓÂÈ· ÌfiÓÔÓ fi,ÙÈ ‰ÂÓ ¤¯Ô˘Ì ÍÂηı·Ú›ÛÂÈ ÚÒÙ· ÛÙÔ Ì˘·Ïfi Ì·˜. ™˘Ì¤Ú·ÛÌ·: °ÏÒÛÛ· Î·È ‰È·ÓfiËÛË Û˘Ó‰¤ÔÓÙ·È ¿ÌÂÛ· Î·È ÛÙÂÓ¿ Ì ÌÈ· Û¯¤ÛË, Ô˘ ‰ÂÓ Â›Ó·È Û¯¤ÛË “˘ÔÙ·Á‹˜” Ù‹˜ Ì›·˜ ÛÙËÓ ¿ÏÏË (Ù‹˜ ÁÏÒÛÛ·˜ ÛÙË ‰È·ÓfiËÛË ‹ Ù‹˜ ‰È·ÓfiËÛ˘ ÛÙË ÁÏÒÛÛ·), ·ÏÏ¿ Û¯¤ÛË ·ÌÔÈ‚·ÈfiÙËÙ·˜, ÂÏ¢ıÂÚ›·˜ Î·È ‰ËÌÈÔ˘ÚÁÈÎfiÙËÙ·˜. ¢È·ÓÔÔ‡ÌÂı· ‰È¿ ÙˆÓ Ï¤ÍˆÓ, ·ÏÏ¿ Î·È ÚÈÓ Î·È ¤Ú· ·fi ÙȘ ϤÍÂȘ. ¢ÂÓ Â›Ó·È Ù˘¯·›Ô ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÈ ŒÏÏËÓ˜, Û˘ÓÂȉËÙÔÔÈÒÓÙ·˜ ÙË ÛÙÂÓ‹ Û¯¤ÛË ÓfiËÛ˘ Î·È ÁÏÒÛÛ·˜, ¤ÊÙ·Û·Ó Ó· ¯ÚËÛÈÌÔÔÈ‹ÛÔ˘Ó ÙË Ï¤ÍË ÏfiÁÔ˜ Ì ‰ÈÙÙ‹ ÛËÌ·Û›·: Ù‹˜ ÏÔÁÈ΋˜ ÛΤ„˘ (ratio) Î·È Ù‹˜ ¤ÏÏÔÁ˘ ÁÏÒÛÛ·˜ (oratio).

∂ÏÏËÓÈ΋ ÁÏÒÛÛ· ∂›Ó·È ÁÓˆÛÙfi Î·È Â˘Ú‡ÙÂÚ· ·Ú·‰ÂÎÙfi fiÙÈ ÔÈ ŒÏÏËÓ˜ ηٿ ÙËÓ ·Ú¯·ÈfiÙËÙ· (ȉ›ˆ˜ ÛÙÔ˘˜ ÏÂÁfiÌÂÓÔ˘˜ ÎÏ·ÛÈÎÔ‡˜ ¯ÚfiÓÔ˘˜) ·Ó¤Ù˘Í·Ó ¤Ó·Ó ÔÏÈÙÈÛÌfi, ¤Ó· Û‡ÛÙËÌ· ·Ú¯ÒÓ Î·È ·ÍÈÒÓ Ì ÂÈÙ‡ÁÌ·Ù· ÛÙÔ˘˜ ıÂÛÌÔ‡˜ Î·È ÛÙȘ Ù¤¯Ó˜, ÙȘ ÂÈÛً̘ Î·È Ù· ÁÚ¿ÌÌ·Ù·, Ô˘ ·ÂÙ¤ÏÂÛ·Ó ÙË ‚¿ÛË Î·È ÛÊÚ¿ÁÈÛ·Ó fi,ÙÈ ÔÓÔÌ¿˙Ô˘ÌÂ Â˘Úˆ·˚Îfi ÔÏÈÙÈÛÌfi. ∞˘Ù‹ Ë “¤ÎÚËÍË” ÛÙÔÓ ÔÏÈÙÈÛÌfi ÙˆÓ ∂ÏÏ‹ÓˆÓ ‹Ù·Ó ·fiÚÚÔÈ· ÌÈ·˜ ͯˆÚÈÛÙ‹˜ ‰È·ÓÔËÙÈ΋˜ ·Ó¿Ù˘Í˘ Ô˘ ÍÂΛÓËÛ ·fi ÙÔ ÂÚ¢ÓËÙÈÎfi ÙÔ˘˜ Ó‡̷, ·fi ÙËÓ ÂÈı˘Ì›· ÙÔ˘˜ Ó· ÁÓˆÚ›ÛÔ˘Ó ¶·È‰È·ÙÚÈ΋ 2005;68:325-328

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ÙÔÓ ÎfiÛÌÔ Î·È ÙÔÓ Â·˘Ùfi ÙÔ˘˜, Ó· ÚÔ‚ÏËÌ·ÙÈÛÙÔ‡Ó ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· ÙÔ‡ ÎfiÛÌÔ˘, ÁÈ· ÙÔÓ £Âfi, ÁÈ· Ù· Ú¿ÁÌ·Ù·, ÁÈ· ÙËÓ Ô˘Û›· Î·È ÙË ÌÔ›Ú· ÙÔ‡ ·ÓıÚÒÔ˘, ÁÈ· ÙȘ Û¯¤ÛÂȘ ÙˆÓ ·ÓıÚÒˆÓ ÌÂٷ͇ ÙÔ˘˜ Î.Ï. OÈ ·Ó·˙ËÙ‹ÛÂȘ ·˘Ù¤˜, ÚÔ¿ÓÙˆÓ Ë ÈηÓfiÙËÙ· Î·È Ë ÓÂ˘Ì·ÙÈ΋ ·ÓËÛ˘¯›· ÙÔ˘˜ Ó· ı¤ÙÔ˘Ó ÚÔ‚Ï‹Ì·Ù· Î·È ÂÚˆÙ‹Ì·Ù·, ÙÔ˘˜ ¤ÊÂÚ·Ó ÛÙËÓ ÂÈÛ‹Ì·ÓÛË ·Ú¯ÒÓ Î·È ·ÍÈÒÓ Ô˘ Ú¤ÂÈ Ó· ‰È¤Ô˘Ó ÙË ˙ˆ‹ ÙÔ‡ ·ÓıÚÒÔ˘ Î·È Ô˘ ‚Ú‹Î·Ó ÙËÓ ¤ÎÊÚ·Û‹ ÙÔ˘˜ ÛÙÔ˘˜ ÔÏÈÙÂÈ·ÎÔ‡˜ ıÂÛÌÔ‡˜ (ÙÈ fiÚÈ· ı¤ÙÂÈ ÛÙÔ Û˘ÓÂȉËÙfi ÔÏ›ÙË Ë ·›ÛıËÛË Â˘ı‡Ó˘ Ô˘ ÁÂÓÓ¿ Ë ‰ËÌÔÎÚ·Ù›·) ·ÏÏ¿ Î·È ÛÙÔ ‰Ú¿Ì· (ÔÈ· Â›Ó·È Ë Û¯¤ÛË Î·È ÔÈ· Â›Ó·È Ë ¿ÏË ÙÔ‡ ·ÓıÚÒÔ˘ Ì ÙË ÌÔ›Ú· ÙÔ˘). ∆Ô˘˜ ‰›‰·Í·Ó fiÙÈ Â›Ó·È ÛËÌ·ÓÙÈÎfi ÁÈ· ÙÔÓ ¿ÓıÚˆÔ Ó· ηÏÏÈÂÚÁ‹ÛÂÈ ÙÔ Ó‡̷ ÙÔ˘, Ó· ·ÔÎÙ‹ÛÂÈ ·È‰Â›·, ·Ú¿ÏÏËÏ· Ì ÙËÓ Î·ÏÏȤÚÁÂÈ· ÙÔ‡ ÛÒÌ·ÙÔ˜, ÙËÓ ¿ÛÎËÛË Û˘Ó‰˘·Ṳ̂ÓË Ì ÙËÓ ¿ÌÈÏÏ·, ‰ËÏ. ÙÔÓ ·ıÏËÙÈÛÌfi. ∂›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈ΋ Ë Û‡ÏÏË„Ë Î·È Î·ıȤڈÛË ÙˆÓ OÏ˘ÌÈ·ÎÒÓ ·ÁÒÓˆÓ, ÂÛÙÈ·ÛÌ¤ÓˆÓ ·ÔÎÏÂÈÛÙÈο ÛÙËÓ Â˘ÁÂÓ‹ ¿ÌÈÏÏ· Î·È Û˘Ó‰˘·ÛÌ¤ÓˆÓ Ì ÙËÓ ·fi‰ÔÛË ‡„ÈÛÙˆÓ ÙÈÌÒÓ ÛÙÔ˘˜ OÏ˘ÌÈÔӛΘ Î·È Ì ·‡ÛË ÙˆÓ Â¯ıÚÔÚ·ÍÈÒÓ, ‰ËÏ·‰‹ Ì ¤ÓÙÔÓ· ÓÂ˘Ì·ÙÈÎfi Ì‹Ó˘Ì·, ·ÓÂÏÏ‹ÓÈ· Û˘ÌÌÂÙÔ¯‹ Î·È Îϛ̷ ÊÈÏ›·˜.

OÈ ÌÂÁ¿ÏÔÈ ÛÙÔ¯·ÛÙ¤˜, ÊÈÏfiÛÔÊÔÈ ‹ ÂÈÛÙ‹ÌÔÓ˜ ‹ ÔÈËÙ¤˜ ‹ ‰Ú·Ì·ÙÔ˘ÚÁÔ›, ÁÈ· Ó· ÂÎÊÚ¿ÛÔ˘Ó ÙȘ ‰È·ÓÔËÙÈΤ˜ ÙÔ˘˜ Û˘ÏÏ‹„ÂȘ, ¯ÚÂÈ¿˙ÔÓÙ·Ó ÌÈ· ηÏÏÈÂÚÁË̤ÓË ÁÏÒÛÛ·, ÌÈ· ÁÏÒÛÛ· Ô˘ Ó· ÚÔÛʤÚÂÙ·È ÛÙË ‰‹ÏˆÛË ÙÔ‡ ÏÔ‡ÙÔ˘ Ó¤ˆÓ ÂÓÓÔÈÔÏÔÁÈÎÒÓ Û˘ÏÏ‹„ÂˆÓ Î·È ÙˆÓ ·Ô¯ÚÒÛÂÒÓ ÙÔ˘˜. O ÊÈÏÔÛÔÊÈÎfi˜ ÛÙÔ¯·ÛÌfi˜ ÚÔËÁÂ›Ù·È Î·È ‰ËÌÈÔ˘ÚÁ› ÙÔÓ ÊÈÏÔÛÔÊÈÎfi ÏfiÁÔ. OÈ ·Ó¿ÁΘ ÙˆÓ ÔÏÈÙÒÓ Ó· ·ÁÔÚ‡ÛÔ˘Ó ‰ËÌfiÛÈ· ÁÈ· Ù· ÎÔÈÓ¿ ‰ËÌÈÔ˘ÚÁÔ‡Ó ÙÔÓ ÔÏÈÙÈÎfi ÏfiÁÔ. OÈ ÂÈÛÙËÌÔÓÈΤ˜ ·Ó·˙ËÙ‹ÛÂȘ Î·È Ë ·Ó¿ÁÎË ÙˆÓ ÂÈÛÙËÌfiÓˆÓ Ó· ÙȘ ‰ËÏÒÛÔ˘Ó ‰ËÌÈÔ˘ÚÁÔ‡Ó ÙÔÓ ÂÈÛÙËÌÔÓÈÎfi ÏfiÁÔ. ∏ Ô›ËÛË Î·È ÙÔ ‰Ú¿Ì· ‰ËÌÈÔ˘ÚÁÔ‡Ó ÙÔÓ ˘„ËÏÒÓ ··ÈÙ‹ÛÂˆÓ ÏÔÁÔÙ¯ÓÈÎfi Î·È ı·ÙÚÈÎfi ÏfiÁÔ. ∏ ·È‰Â›·, Ô ÔÏÈÙÈÛÌfi˜, Ë ‰È·ÓÔËÙÈ΋ ·Ó¿Ù˘ÍË Â›Ó·È Ô˘ ‰ËÌÈÔ˘ÚÁÔ‡Ó ÙËÓ ·Ó¿ÁÎË Î·È ÙȘ ÚÔ¸Ôı¤ÛÂȘ ‡·Ú͢ ÌÈ·˜ ηÏÏÈÂÚÁË̤Ó˘ ÁÏÒÛÛ·˜, ÌÈ·˜ Ú·ÁÌ·ÙÈο ‰ËÌÈÔ˘ÚÁÈ΋˜ ÁÏÒÛÛ·˜ Ô˘ Ó· ÌÔÚ› Ó· ÂÎÊÚ¿˙ÂÈ ÙËÓ ÏÔ‡ÛÈ· ‰Ú·ÛÙËÚÈfiÙËÙ· ÛÙÔ˘˜ ÙÔÌ›˜ Ù‹˜ ÂÈÛÙ‹Ì˘ Î·È ÙÔ‡ ÔÏÈÙÈÛÌÔ‡. ∏ ÔÈÔÙÈ΋ Î·È ‰ËψÙÈ΋ ·˘Ù‹ ÁÏÒÛÛ·, ¿·Í Î·È Û˘ÓÙÂÏÂÛı›, ÙÚÔÊÔ‰ÔÙ› Ì ÙË ÛÂÈÚ¿ Ù˘ ÙË ‰È·ÓfiËÛË Î·È ÙËÓ ·Ú·ÎÈÓ› Ó· ÏÂÈÙÔ˘ÚÁ‹ÛÂÈ ‰ËÌÈÔ˘ÚÁÈο.

OÈ ÌÂÁ¿ÏÔÈ ÛÙÔ¯·ÛÙ¤˜ (ÊÈÏfiÛÔÊÔÈ, ÔÈËÙ¤˜, ‰Ú·Ì·ÙÔ˘ÚÁÔ›, ÈÛÙÔÚÈÎÔ›, ÂÈÛÙ‹ÌÔÓ˜ Î.Ï.) Â›Ó·È ÂÍ ·Ó¿Á΢ Î·È ÌÂÁ¿ÏÔÈ ‰ËÌÈÔ˘ÚÁÔ› ÁÏÒÛÛ·˜. ∆Ô ÏÂÙfi, Û‡ÓıÂÙÔ, ··ÈÙËÙÈÎfi, ÚˆÙfiÙ˘Ô Û ȉ¤Â˜ Î·È ÛΤ„ÂȘ ÂÚȯfiÌÂÓÔ ÂÓfi˜ ÎÂÈ̤ÓÔ˘ ··ÈÙ› Î·È ·Ó¿ÏÔÁË ÔÈfiÙËÙ· ÁÏÒÛÛ·˜ Ô˘ Ó· ÙÔ ‰ËÏÒÓÂÈ. ŒÙÛÈ Ï.¯. ÛÙÔ ÏÂÍÈÏfiÁÈÔ ÔÈ ŒÏÏËÓ˜ ‰È¤ıÂÙ·Ó ¤Ó·Ó ÏÂÍÈÏÔÁÈÎfi Ì˯·ÓÈÛÌfi Ô˘ ÂÍ·ÛÊ¿ÏÈ˙ ÛÙÔÓ ÔÌÈÏËÙ‹ Ù‹˜ ∂ÏÏËÓÈ΋˜ ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ÂÍÂȉÈ·ÂÈ ÙË ÛËÌ·Û›· ÙÔ‡ Ú‹Ì·ÙÔ˜ Û ÂÎÏËÎÙÈ΋ Îϛ̷η, ÙÚÔÔÔÈÒÓÙ·˜ ÏÈÁfiÙÂÚÔ ‹ ÂÚÈÛÛfiÙÂÚÔ ‹ ÌÂÙ·‚¿ÏÏÔÓÙ·˜ ÙË ‚·ÛÈ΋ ÛËÌ·Û›· Ù‹˜ Ϥ͢. ∞˘Ùfi ÂÈÙ˘Á¯·ÓfiÙ·Ó Ì ÙÔÓ Ì˯·ÓÈÛÌfi ÙˆÓ ÚÔÚÚËÌ·ÙÈÎÒÓ, ÙˆÓ ÚÔı¤ÛÂˆÓ Ô˘ Û˘ÓÙ›ıÂÓÙÔ Ì ٷ Ú‹Ì·Ù·. ÷ڷÎÙËÚÈÛÙÈÎfi ·Ú¿‰ÂÈÁÌ· Â›Ó·È ÙÔ Ú‹Ì· ‚¿Ïψ Ù‹˜ ·Ú¯·›·˜ ∂ÏÏËÓÈ΋˜. ™ÙÔ ÏÂÍÈÎfi ÙˆÓ Liddell-Scott-Jones ·Ú·‰›‰ÔÓÙ·È 104 Û‡ÓıÂÙ· Ú‹Ì·Ù· ÙÔ‡ ‚¿Ïψ Ì ÚÔÚÚËÌ·ÙÈο: Â΂¿Ïψ ·Ó·‚¿Ïψ ˘ÂÚ‚¿Ïψ

ÂÌ‚¿Ïψ ηٷ‚¿Ïψ ·Ú·‚¿Ïψ

ÂÈÛ‚¿Ïψ ‰È·‚¿Ïψ ˘Ô‚¿Ïψ

Û˘Ì‚¿Ïψ ÌÂÙ·‚¿Ïψ ·ÓÙÈ‚¿Ïψ

ÚÔ‚¿Ïψ ·Ô‚¿Ïψ ÂÈ‚¿Ïψ

ÚÔÛ‚¿Ïψ ÂÚÈ‚¿Ïψ Î.Ô.Î.

∞˜ ÛËÌÂȈı› fiÙÈ ÙÔ Ú‹Ì· ÙÔ Û‡ÓıÂÙÔ Ì ¤Ó· ÚÔÚÚËÌ·ÙÈÎfi ÌÔÚ› Ó· ÂÍÂȉÈ·ÂÙ·È ÂÚ·ÈÙ¤Úˆ Ì ÂÈϤÔÓ ÚÔÚÚËÌ·ÙÈο: ÂÈ-‚¿Ïψ: ‰È·-‚¿Ïψ:

Û˘Ó-ÂÈ‚¿Ïψ ÂÈ-‰È·‚¿Ïψ ˘Ô-‰È·‚¿Ïψ

ÚÔ-ÂÈ‚¿Ïψ ·ÓÙÈ-‰È·‚¿Ïψ ÚÔ-‰È·‚¿Ïψ

·Ú-ÂÈ‚¿Ïψ ÂÓ-‰È·‚¿Ïψ ÚÔÛ-‰È·‚¿Ïψ

ÚÔÛ-ÂÈ‚¿Ïψ Û˘Ó-‰È·‚¿Ïψ

∂Ó Û˘Ó¯›· ı· ÂÚÈÔÚ›Ûˆ ÙÔÓ ÏfiÁÔ ÌÔ˘ Û ÌÂÚÈο ‰Â›ÁÌ·Ù· Ϥ͈Ó-ÂÓÓÔÈÒÓ Ù‹˜ ∂ÏÏËÓÈ΋˜ Ô˘ ¤Ú·Û·Ó ÛÙȘ Â˘Úˆ·˚Τ˜ ÁÏÒÛÛ˜ (·ÁÁÏÈ΋, Á·ÏÏÈ΋, ÁÂÚÌ·ÓÈ΋, ÈÙ·ÏÈ΋, ÈÛ·ÓÈ΋ Î.¿.). ∂˘ÚÒË (Europe): ∆Ô fiÓÔÌ· Ù‹˜ ∂˘ÚÒ˘ Â›Ó·È Ë ÂÏÏËÓÈ΋ ÔÓÔÌ·Û›· ÂÓfi˜ Ì˘ıÔÏÔÁÈÎÔ‡ ÚÔÛÒÔ˘ Ô˘ Û‹Ì·ÈÓ Èı·Ófiٷٷ “·˘Ù‹Ó Ô˘ ¤¯ÂÈ Â˘Ú›˜ Ò·˜, ÌÂÁ¿Ï· Ì¿ÙÈ·”. ∏ Ú›˙· Ô- ‚Ú›ÛÎÂÙ·È Û ϤÍÂȘ fiˆ˜ ÔÙÈÎfi˜ (optical, optician), ÂÓÒ ·fi ÙÔ fiÓÔÌ· Ù‹˜ ∂˘ÚÒ˘ ÚÔ‹ÏıÂ Î·È Ë ÔÓÔÌ·Û›· ÙÔ‡ Â˘Úˆ·˚ÎÔ‡ ÓÔÌ›ÛÌ·ÙÔ˜ euro (Â˘Úˆ), ÙÔ ÔÔ›Ô Ì¿ÏÈÛÙ· ÁÚ¿ÊÂÙ·È ¿Óˆ ÛÙÔ ¯·ÚÙÔÓfiÌÈÛÌ· Î·È Ì ÙËÓ ÂÏÏËÓÈ΋ ÁÚ·Ê‹ Ù‹˜ Ϥ͢. ¶·È‰È·ÙÚÈ΋ 2005;68:325-328

Pediatr Sept-Oct 05

17-10-05

16:51

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ŒÓ·˜ ÂÓÓÔÈÔÏÔÁÈÎfi˜ ¯ÒÚÔ˜ fiÔ˘ ÔÈ ÂÏÏËÓÈΤ˜ ¤ÓÓÔȘ ¤Ú·Û·Ó Ì ÂÏÏËÓÈΤ˜ ϤÍÂȘ ÛÙȘ Â˘Úˆ·˚Τ˜ ÁÏÒÛÛ˜ Â›Ó·È ÙÔ ÂÓÓÔÈÔÏÔÁÈÎfi ‰›Ô Ù‹˜ ÔÏÈÙÈ΋˜, ÙˆÓ ıÂÛÌÒÓ Î·È Ù‹˜ ȉÂÔÏÔÁ›·˜: ÔÏÈÙÈ΋ (politics): ∏ ϤÍË ÚÔ¤Ú¯ÂÙ·È ·fi ÙÔ ÂÏÏËÓÈÎfi ÔÏÈÙÈÎfi˜: ÔÏÈÙÈ΋ Ù¤¯ÓË ‹ ÂÈÛÙ‹ÌË ‹Ù·Ó ÔÈ Ú¿ÍÂȘ Î·È ÔÈ ÁÓÒÛÂȘ ÙÔ‡ ˘‡ı˘ÓÔ˘ Î·È ÂÓÂÚÁÔ‡ ÔÏ›ÙË ÛÙËÓ ¿ÌÂÛË ‰ËÌÔÎÚ·Ù›· Ù‹˜ ·Ú¯·›·˜ ∞ı‹Ó·˜, ÙÔ‡ ÔÏ›ÙË, Ô˘ Â›Ó·È Ô Î¿ÙÔÈÎÔ˜ Ù‹˜ fiÏ˘ (Ù‹˜ fiÏ˘-ÎÚ¿ÙÔ˘˜ ÛÙËÓ ·Ú¯·›· ‰ËÌÔÎÚ·Ù›·). ‰ËÌÔÎÚ·Ù›· (democracy): ∞fi ÙÔ ‰‹ÌÔ˜ Î·È ÙÔ ÎÚ¿ÙÔ˜: ‰‹ÏˆÓ ÙË ‰‡Ó·ÌË, ÙËÓ ÂÍÔ˘Û›· ÙÔ‡ Ï·Ô‡, ÙˆÓ ÔÏÏÒÓ, ÙÔÓ ÔÏÈÙÒÓØ ·ÓÙÈÙÈı¤ÌÂÓÔ ÚÔ˜ ÙËÓ ·ÚÈÛÙÔÎÚ·Ù›· (aristocracy), ÙË ‰‡Ó·ÌË, ÙËÓ ÂÍÔ˘Û›· ÙˆÓ ·Ú›ÛÙˆÓ, Ù‹˜ ÂÏ›Ù, Ô˘ η٤ÏËÁ ӷ Â›Ó·È Ë ÂÍÔ˘Û›· ÙˆÓ Ï›ÁˆÓ. µ¿ÛË Ù‹˜ ‰ËÌÔÎÚ·Ù›·˜ ‹Ù·Ó Ô ‰È¿ÏÔÁÔ˜ (dialogue) ·fi ÙÔ ‰È·Ï¤ÁÔÌ·È, “Û˘ÓÔÌÈÏÒ, Û˘˙ËÙÒ Ì ÙÔ˘˜ ¿ÏÏÔ˘˜ ·Ó·˙ËÙÒÓÙ·˜ ÙËÓ ·Ï‹ıÂÈ·”. ∞fi ÙÔ ›‰ÈÔ Ú‹Ì· ÚÔ‹ÏıÂ Ë ‰È·ÏÂÎÙÈ΋ ̤ıÔ‰Ô˜ (dialectic) ÛÙË ÊÈÏÔÛÔÊ›·, Ë Ì¤ıÔ‰Ô˜ ·Ó·˙‹ÙËÛ˘ Ù‹˜ ·Ï‹ıÂÈ·˜ ̤۷ ·fi ÂÚˆÙ‹ÛÂȘ Î·È ··ÓÙ‹ÛÂȘ, ̤۷ ·fi ÙÔÓ ‰È¿ÏÔÁÔ. ∞ÚÁfiÙÂÚ·, Û ÌÂ۷ȈÓÈÎÔ‡˜ ¯ÚfiÓÔ˘˜, Ï¿ÛÙËÎÂ Î·È Ô ÌÔÓfiÏÔÁÔ˜ (ÌfiÓÔ˜ + ÏfiÁÔ˜, monologue) Ô˘ ›¯Â ÂÍ·Ú¯‹˜ ·ÚÓËÙÈ΋ ÛËÌ·Û›·. ŸÏ· ·˘Ù¿ ›ӷÈ, ‚‚·›ˆ˜, ı¤Ì·Ù· ÔÏÈÙÈ΋˜ ȉÂÔÏÔÁ›·˜/ideology), ÌÈ·˜ ¿ÏÏ˘ ÁÓˆÛÙ‹˜ Ϥ͢ ÙÔ‡ ÔÏÈÙÈÎÔ‡ ÏÂÍÈÏÔÁ›Ô˘, Ë ÔÔ›· ÍÂÎÈÓ¿ÂÈ ·fi ÙËÓ Ôχ ÛËÌ·ÓÙÈ΋ ÂÏÏËÓÈ΋ ϤÍË È‰¤·, ·˘Ù‹ Ô˘ ¤‰ˆÛ ÛÙȘ Â˘Úˆ·˚Τ˜ ÁÏÒÛÛ˜ fiϘ ÙȘ ·ÓÙ›ÛÙÔȯ˜ ϤÍÂȘ (·ÁÁÏ. idea, Á·ÏÏ. idée, ÁÂÚÌ. idee Î.Ï.). ∏ ·Ú¯È΋ ÛËÌ·Û›· ÙÔ‡ ȉ¤· Â›Ó·È “·˘Ùfi Ô˘ ‚Ϥˆ Ì ÙÔÓ ÓÔ˘ ÌÔ˘, Ô˘ Û˘ÏÏ·Ì‚¿Óˆ Ì ÙÔ Ì˘·Ïfi ÌÔ˘”, ÍÂÎÈÓ¿ÂÈ ‰ËÏ·‰‹ ·fi ÙËÓ fiÚ·ÛË ˆ˜ ËÁ‹ ÁÓÒÛˆ˜. ∞fi ÙËÓ ›‰È· Ú›˙· Ù‹˜ ∂ÏÏËÓÈ΋˜ ÚÔ¤Ú¯ÔÓÙ·È ‰‡Ô ¿ÏϘ ÛËÌ·ÓÙÈΤ˜ ϤÍÂȘ: Ë ÈÛÙÔÚ›· (< ›ÛÙˆÚ “Ô ÁÓÒÛÙ˘”), ·Ú¯Èο “·˘Ùfi˜ Ô˘ ‚ϤÂÈ Ì ٷ Ì¿ÙÈ· ÙÔ˘ Î·È ÁÓˆÚ›˙ÂÈ Î·Ï¿” (‚. ·ÁÁÏ. history Î·È story, Á·ÏÏ. histoire) ·ÏÏ¿ Î·È “·˘Ùfi Ô˘ Ê·›ÓÂÙ·È ¯ˆÚ›˜ Î·È Ó· Â›Ó·È ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ·”, ÙÔ Â›‰ˆÏÔ (·ÁÁÏ. idol, Á·ÏÏ. idole). ¶ÔÏϤ˜ ϤÍÂȘ ÂÏÏËÓÈΤ˜ ¤Ú·Û·Ó ·fi ÙËÓ ÂÈÛÙ‹ÌË ÛÙËÓ Î·ıËÌÂÚÈÓ‹ ÁÏÒÛÛ· ÌÈ·˜ ÈÔ ··ÈÙËÙÈ΋˜ ÌÔÚÊ‹˜ ÂÈÎÔÈÓˆÓ›·˜. ¶·Ú·‰Â›ÁÌ·Ù·: ıˆڛ· (theory), Ú·ÎÙÈ΋ (practice), ÂÌÂÈÚÈÎfi˜ (empirical), ÏÔÁÈ΋ (logic), Û‡ÛÙËÌ· (system), ̤ıÔ‰Ô˜ (method), ηÙËÁÔÚ›· (category), ÈÂÚ·Ú¯›· (hierarchy), ˘fiıÂÛȘ (hypothesis), ·Ó¿Ï˘ÛȘ-Û‡ÓıÂÛȘ (analysis-synthesis), ÎÚÈÙ‹ÚÈ· (criteria), Úfi‚ÏËÌ· (problem), ۇ̂ÔÏÔ, ·Í›ˆÌ·-·ÍȈ̷ÙÈÎfi˜ (axiom-axiomatic), fiÚÁ·ÓÔ (organ, organize, organism), ÚfiÁÚ·ÌÌ· (program), ı¤Ì· (theme), ı¤ÛȘ (thesis), ÙfiÔ˜/ÙÔÈÎfi˜ (topic), Ù‡Ô˜ (type), Ê¿ÛË (phase), ·Ú¿ÏÏËÏÔ˜ (parallel), ˙ÒÓË (zone), ÛÎÂÙÈÎfi˜ (skeptic/skepticism), ·ÓÈÎfi˜, Û‡Ìو̷ (symptom), ıÂÚ·›· (therapy), ÎÏ›ÓË/ÎÏÈÓÈÎfi˜ (clinic), ‰È¿ÁÓˆÛȘ (diagnosis), Ê¿ÚÌ·ÎÔ (pharmacy), ÂÓ‰ËÌÈÎfi˜ÂȉËÌÈÎfi˜ (endemic-epidemic). ∆¤ÏÔ˜ ˘¿Ú¯ÂÈ ¤Ó·˜ ·ÚÎÂÙ¿ ÌÂÁ¿ÏÔ˜ ·ÚÈıÌfi˜ ϤÍÂˆÓ Ô˘ Â›Ó·È ÂÏÏËÓÈΤ˜, ·ÏÏ¿ Ô˘ ‰ÂÓ ÙȘ ·Ó·ÁÓˆÚ›˙ÂÈ Ô ÌË ÂȉÈÎfi˜ ·Ì¤Ûˆ˜. ªÂÚÈο ·Ú·‰Â›ÁÌ·Ù·: butter Â›Ó·È Ô “‚Ô‡Ù˘ÚÔ˜”, paper Â›Ó·È Ô ¿˘ÚÔ˜, church/Kirche Â›Ó·È ÙÔ Î˘ÚÈ·ÎfiÓ (‰ÒÌ·), sketch Â›Ó·È ÙÔ Û¯¤‰ÈÔ (> schizzo > ÛΛÙÛÔ Î·È ÛÎÂÙ˜), scene Â›Ó·È Ë ÛÎËÓ‹ (> scenario> ÛÂÓ¿ÚÈÔ), pain Â›Ó·È Ë ÔÈÓ‹, calm ÙÔ Î·‡Ì· (> οÏÌ·), pirate Â›Ó·È Ô ÂÈÚ·Ù‹˜, priest/prêtre Â›Ó·È ÙÔ ÚÂÛ‚‡ÙÂÚÔ˜, clergy Î·È clerk Â›Ó·È ÙÔ ÎÏËÚÈÎfi˜, bishop ÙÔ Â›ÛÎÔÔ˜, monk Ô ÌÔÓ·¯fi˜, card Î·È chart Ô ¯¿ÚÙ˘, bomb Ô ‚fiÌ‚Ô˜, buffalo Ô ‚Ô‡‚·ÏÔ˜, diploma Î·È diplomat ÙÔ ‰›ψ̷ (< ‰ÈÏÒÓˆ). ∞˜ ÛËÌÂȈı› fiÙÈ ·ÎfiÌË Î·È ÂˆÓ˘Ì›Â˜ ÂÙ·ÈÚÂÈÒÓ ·Ó·ÙÚ¤¯Ô˘Ó Û ÂÏÏËÓÈο ÔÓfiÌ·Ù·: NIKE (Ó›ÎË) ·ıÏËÙÈο ›‰Ë, PEPSI-COLA (¤„Ș), FANTA (Ê·ÓÙ·Û›·), MONOPOLY, LEGO “·Ú¯. ϤÁˆ = Û˘Ó·ÚÌÔÏÔÁÒ”, IKEA (“Ô›ÎÔ˜”), XEROX (ÍËÚfi˜), ROLEX (ˆÚÔÏfiÁÈÔ), METRO. ªÂ ÂÏ¿¯ÈÛÙ· ·Ú·‰Â›ÁÌ·Ù· ÚÔÛ¿ıËÛ· Ó· ‰Â›Íˆ ÔÈ· Â›Ó·È Ë ·ÚÔ˘Û›· Ù‹˜ ÂÏÏËÓÈ΋˜ ÁÏÒÛÛ·˜ ̤۷ ÛÙÔÓ Â˘Úˆ·˚Îfi ÔÏÈÙÈÛÌfi Î·È ÙËÓ Â˘Úˆ·˚΋ ÛΤ„Ë, fiˆ˜ ÂÎÊÚ¿˙ÂÙ·È Ì ¤ÓÓÔȘ Î·È Ì ϤÍÂȘ ÙˆÓ Â˘Úˆ·˚ÎÒÓ ÁψÛÛÒÓ. £· ÌÔÚÔ‡Û· Ó· ·Ó·Ê¤Úˆ ¯ÈÏÈ¿‰Â˜ ÂÏÏËÓÈΤ˜ ϤÍÂȘ, Ô˘ Â›Ó·È ‹‰Ë Û˘ÁÎÂÓÙڈ̤Ó˜ Û ÂȉÈο ¤ÚÁ· ÁψÛÛÔÏfiÁˆÓ, ÊÈÏÔÏfiÁˆÓ Î·È ¿ÏÏˆÓ ÂÈÛÙËÌfiÓˆÓ Î·È Ô˘ ÌÔÚ› Ó· ‚ÚÂÈ Î·Ó›˜ Û fiÏ· Ù· ¤Á΢ڷ ÏÂÍÈο ÙˆÓ ÂÈ̤ÚÔ˘˜ Â˘Úˆ·˚ÎÒÓ ÁψÛÛÒÓ. £· ÛÙ·ıÒ ÌfiÓÔ Û ¤Ó· ¿ÚıÚÔ ÙÔ‡ Ôχ ÁÓˆÛÙÔ‡ ÕÁÁÏÔ˘ ηıËÁËÙ‹ Ù‹˜ °ÏˆÛÛÔÏÔÁ›·˜ David Crystal, Ì ÙÔÓ ·ÈÓÈÁÌ·ÙÈÎfi Ù›ÙÏÔ “∏ ∞ÁÁÏÈ΋ ˆ˜ ÎÏ·ÛÈ΋ ÁÏÒÛÛ·”. ™ÙÔ ¿ÚıÚÔ ·˘Ùfi Ô D. Crystal ‰Â›¯ÓÂÈ fiÙÈ Ë ∞ÁÁÏÈ΋ Â›Ó·È ÌÈ· “ÎÏ·ÛÈ΋ ÁÏÒÛÛ·” Ì ÙËÓ ¤ÓÓÔÈ· fiÙÈ ¿Óˆ ·fi Ù· 2/3 ÙÔ‡ ÏÂÍÈÏÔÁ›Ô˘ ¶·È‰È·ÙÚÈ΋ 2005;68:325-328

Pediatr Sept-Oct 05

17-10-05

16:51

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Ù˘ ÚÔ¤Ú¯ÂÙ·È ·fi ‰‡Ô ÎÏ·ÛÈΤ˜ ÁÏÒÛÛ˜, ÙËÓ ∂ÏÏËÓÈ΋ Î·È ÙË §·ÙÈÓÈ΋! O Crystal ˘ÔÛÙËÚ›˙ÂÈ Ì¿ÏÈÛÙ· ÙËÓ ¿Ô„Ë fiÙÈ Ë Â˘Ú‡Ù·ÙË Î˘ÚÈ·Ú¯›· Ù‹˜ ∞ÁÁÏÈ΋˜ ÔÊ›ÏÂÙ·È Î·Ù¿ Ôχ ÛÙÔ ÁÂÁÔÓfi˜ Ù‹˜ ÏÂÍÈÏÔÁÈ΋˜ Ù˘ ̛͈˜, ÙÔ‡ ‰·ÓÂÈÛÌÔ‡ ‰ËÏ·‰‹ ·fi ÙȘ ÎÏ·ÛÈΤ˜ ΢ڛˆ˜ ÁÏÒÛÛ˜, Ô˘ ηıÈÛÙ¿ ÙËÓ ∞ÁÁÏÈ΋ ÔÈΛ· Î·È ·Ô‰ÂÎÙ‹ ·fi ÔÌÈÏËÙ¤˜ ¿ÏÏˆÓ ÁψÛÛÒÓ. O Crystal ‰›ÓÂÈ ÛÙÔ ÎÏ·ÛÈÎfi ÏÂÍÈÏfiÁÈÔ ÌÈ· ¿ÏÏË ‰È¿ÛÙ·ÛË. ∆Ô ‚ϤÂÈ ˆ˜ “ÂÓÔÔÈËÙÈ΋ ÈÛÙÔÚÈ΋ ·Ú¿‰ÔÛË” Ù‹˜ ∂˘ÚÒ˘, ʤÚÂÈ ‰Â ÛÙÔ ÚÔÛ΋ÓÈÔ ÙÔÓ fiÚÔ “∂˘ÚˆÂÏÏËÓÈ΋”, ı¤ÏÔÓÙ·˜ Ì’ ·˘ÙfiÓ Ó· ‰ËÏÒÛÂÈ fiÙÈ Ë (·Ú¯·›·) ∂ÏÏËÓÈ΋ ÂÍ·ÎÔÏÔ˘ı› Ó· ÏÂÈÙÔ˘ÚÁ› ˆ˜ ˙ˆÓÙ·Ó‹ ÁÏÒÛÛ· Ù‹˜ ∂˘ÚÒ˘. π‰È·›ÙÂÚ· ÛËÌ·ÓÙÈ΋ Â›Ó·È Ë ·Ú·Ù‹ÚËÛË ÙÔ‡ Crystal fiÙÈ “ÔÈ ÎÏ·ÛÈΤ˜ ÁÏÒÛÛ˜ Û˘Ó¯›˙Ô˘Ó Ó· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙËÓ ∞ÁÁÏÈ΋ ˆ˜ ‰˘Ó·ÌÈ΋ ËÁ‹ Ó¤ˆÓ Ϥ͈Ó, ˆ˜ ËÁ‹ ÏÂÍÈÏÔÁÈ΋˜ ‰ËÌÈÔ˘ÚÁ›·˜ […] Â›Ó·È ÂÎÏËÎÙÈÎfi Ó· ‚ϤÂȘ fiÛÔ ÛÙËÚÈ˙fiÌ·ÛÙ ·ÎfiÌË ÛÙËÓ ∂ÏÏËÓÈ΋ Î·È ÙË §·ÙÈÓÈ΋, ÁÈ· Ó· ÌÈÏ‹ÛÔ˘Ì ÁÈ· ÔÓÙfiÙËÙ˜ Î·È ÁÂÁÔÓfiÙ· Ô˘ ‚Ú›ÛÎÔÓÙ·È ÛÙËÓ Î·Ú‰È¿ Ù‹˜ Û‡Á¯ÚÔÓ˘ ˙ˆ‹˜”. ∆ÂÏÂÈÒÓÔÓÙ·˜ ı¤Ïˆ Ó· ÂÈÛÙ‹Ûˆ ÙËÓ ÚÔÛÔ¯‹ Û·˜ Û ÌÈ· ‚·ÛÈ΋ ÁψÛÛÔÏÔÁÈ΋ ·Ú¯‹: Ë Û˘Ì‚ÔÏ‹ ÌÈ·˜ ÁÏÒÛÛ·˜, ÂÓ ÚÔÎÂÈ̤ӈ Ù‹˜ ∂ÏÏËÓÈ΋˜, Û ¿ÏÏË ‹ ¿ÏϘ ÁÏÒÛÛ˜ ‰ÂÓ Â›Ó·È ı¤Ì· ÔÛÔÙÈÎfi. ∂›Ó·È ı¤Ì· ÔÈÔÙÈÎfi. ∂›Ó·È ¿ÏÏÔ Ú¿ÁÌ· ‰ËÏ. - Û Â›Â‰Ô ÂÈÎÔÈÓˆÓ›·˜, ηÏÏȤÚÁÂÈ·˜, ÓÔËÙÈ΋˜ ‰ÈÂÚÁ·Û›·˜ - Ó· ‰›ÓÂȘ Ï.¯. ϤÍÂȘ-¤ÓÓÔȘ fiˆ˜ Ë ıˆڛ·, Ë È‰¤·, Ë Ô›ËÛË, Ë Ù¯ÓÔÏÔÁ›·, Ë ÔÏÈÙÈ΋, Ë ‰ËÌÔÎÚ·Ù›·, Ë Ê·ÓÙ·Û›·, Ô ‹Úˆ·˜, Ë ÌÔ˘ÛÈ΋, ÙÔ ı¤·ÙÚÔ, Ë ÈÛÙÔÚ›·, Î·È ¿ÏÏÔ Ó· ‰›ÓÂȘ ϤÍÂȘ-¤ÓÓÔȘ fiˆ˜ hamburger, fast-food, supermarket, kits, ÓÙÔÏÌ¿‰Â˜, Ì·ÚÌÔ‡ÙÈ ‹ Ï·ÌfiÁÈ·! °È·Ù› Ë ÔÈfiÙËÙ· Ù‹˜ ÛΤ„˘ Î·È Ù‹˜ ¤ÎÊÚ·Û˘ Â›Ó·È Û˘Ó¿ÚÙËÛË Ù‹˜ ÔÈfiÙËÙ·˜ Ù‹˜ ÁÏÒÛÛ·˜ Î·È ÙÔ‡ ÛËÌ·ÛÈÔÏÔÁÈÎÔ‡ ÊÔÚÙ›Ô˘ ÙˆÓ Ï¤ÍˆÓ. ∏ Û˘Ì‚ÔÏ‹ Ù‹˜ ÂÏÏËÓÈ΋˜ ÁÏÒÛÛ·˜ ÛÙÔÓ Â˘Úˆ·˚Îfi ÏfiÁÔ - ˆ˜ ÛΤ„Ë Î·È ˆ˜ ÁÏÒÛÛ· - Â›Ó·È fiÙÈ ÚÔÛ¤‰ˆÛ ÛÙÔÓ Â˘Úˆ·˚Îfi ÏfiÁÔ ¤Ó· ¿ÏÏÔ “ÁψÛÛÈÎfi ‹ıÔ˜”, ¤Ó·Ó ÏfiÁÔ Ì ÌÂÁ¿ÏË ‰ËψÙÈ΋ ‰‡Ó·ÌË, Ì ‰˘Ó·ÙfiÙËÙ˜ ÂÍ·ÈÚÂÙÈ΋˜ ·ÎÚ›‚ÂÈ·˜ ηıÒ˜ Î·È Ì ÚˆÙfiÁÓˆÚË ÂÎÊÚ·ÛÙÈ΋ ÔÈÎÈÏ›· Î·È Â‡ÚÔ˜ ÛËÌ·ÛÈÔÏÔÁÈÎÒÓ ·Ô¯ÚÒÛˆÓ. ∆¤ÏÔ˜, Ë ÂÓÓÔÈÔÏfiÁËÛË Î·È ÏÂÍÈ΋ Û‹Ì·ÓÛË ÙÔ‡ ÎfiÛÌÔ˘ ÙˆÓ Ú·ÁÌ¿ÙˆÓ Î·È ÙÔ‡ Ê¿ÛÌ·ÙÔ˜ ÙˆÓ ·ÊËÚËÌ¤ÓˆÓ ÂÓÓÔÈÒÓ Ô‰‹ÁËÛ ÛÙË ÁÓˆÛÙ‹ Î·È ÙÈÌËÙÈ΋ ÁÈ· ÙËÓ ÂÏÏËÓÈ΋ ÁÏÒÛÛ· Ú‹ÛË “The Greeks must have a word for it”! ∆Ô Ó· ¤¯ÂȘ fï˜ ϤÍË ÁÈ· fiÏ· Ù· Ú¿ÁÌ·Ù· Î·È ÁÈ· οı ¤ÓÓÔÈ· ÛËÌ·›ÓÂÈ Ó· ¤¯ÂȘ ‰È¢ڇÓÂÈ ÙfiÛÔ Ôχ Ù· fiÚÈ· Ù‹˜ ÁÓÒÛ˘ Î·È Ù‹˜ ÓfiËÛ‹˜ ÛÔ˘ Î·È Ó· ¤¯ÂȘ ·ÎÔÓ›ÛÂÈ Û ÙfiÛË ¤ÎÙ·ÛË ÙÔÓ ÏfiÁÔ ÛÔ˘, ÒÛÙ ӷ ÌÔÚ›˜ Ó· ‰ËÏÒÓÂȘ Ì ÙË ÁÏÒÛÛ· ÔÏfiÎÏËÚÔ ÙÔ Ê¿ÛÌ· ÙÔ‡ ÎfiÛÌÔ˘ Ì·˜, ·ÊÔ‡ - fiˆ˜ ¤¯ÂÈ ÂÈ Ôχ ·Ú·ÛÙ·ÙÈο Ô Wittgenstein - Ô ÎfiÛÌÔ˜ Ì·˜ Â›Ó·È Ë ÁÏÒÛÛ· Ì·˜. ªÈ· ÁÏÒÛÛ· fiˆ˜ Ë ∂ÏÏËÓÈ΋ Ô˘ Â˘Ù‡¯ËÛ ӷ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÚÔÊÔÚÈο Î·È ÁÚ·Ù¿ 4.000 ¯ÚfiÓÈ· Î·È Ô˘ ›¯Â ÙÔ ÚÔÓfiÌÈÔ Ó· Êı¿ÛÂÈ Û ÌÈ· ÚˆÙfiÁÓˆÚË Î·ÏÏȤÚÁÂÈ· ·fi Ù· ÁÈÁ¿ÓÙÂÈ· Ó‡̷ٷ Ô˘ ÂÎÊÚ¿ÛÙËÎ·Ó Û’ ·˘Ù‹, ÌÈ· ÁÏÒÛÛ· Ô˘ Â› 25 Î·È ϤÔÓ ·ÈÒÓ˜ ÛÌÈχÂÙ·È ·Î·Ù¿·˘ÛÙ· ÛÙÔÓ ¯ÒÚÔ Ù‹˜ ÂÈÛÙ‹Ì˘ Î·È Ù‹˜ Ô›ËÛ˘ - ‰ÂÓ ˘¿Ú¯ÂÈ Ô‡Ù ¤Ó·˜ ·ÈÒÓ·˜ Ô˘ Ó· ÌËÓ ¤¯ÂÈ ÁÚ·Ê› Ô›ËÛË Û ÂÏÏËÓÈ΋ ÁÏÒÛÛ·, ¤ÏÂÁÂ Ô ∂χÙ˘ - Â›Ó·È ·Ó·ÌÂÓfiÌÂÓÔ, fiˆ˜ ÚÔÛ¿ıËÛ· Ó· ‰Â›Íˆ, Ó· ¤¯ÂÈ ÏÔ˘Ù›ÛÂÈ ÙÔÓ Â˘Úˆ·˚Îfi ÏfiÁÔ Ì ıÂÌÂÏÈÒ‰ÂȘ ¤ÓÓÔȘ Î·È Ï¤ÍÂȘ. ∏ ÂÏÏËÓÈ΋ ÁÏÒÛÛ· ÙÂÏÈο Â›Ó·È Ë ‰‡Ó·ÌË Î·È Ë ·‰˘Ó·Ì›· Ì·˜: ¢‡Ó·ÌË ÁÈ· ÙÔÓ ÂÎÊÚ·ÛÙÈÎfi ÏÔ‡ÙÔ, ÙË ÁψÛÛÈ΋ ÈÛ¯‡ Î·È ÙËÓ ÂÓ‰˘Ó¿ÌˆÛË Ù‹˜ ÛΤ„˘ Ô˘ ÚÔÛÔÚ›˙ÂÈ Û’ ·˘ÙfiÓ Ô˘ ÙËÓ Î·Ù¤¯ÂÈ. ∞‰˘Ó·Ì›·, ÁÈ·Ù› ¯ÚÂÈ¿˙ÔÓÙ·È ÛÙ¤ÚÂË ·È‰Â›·, Ì·ÎÚfi˜ ¯ÚfiÓÔ˜ Î·È ÙÂÚ¿ÛÙÈÔ˜ Ìfi¯ıÔ˜ ÁÈ· Ó· ÙËÓ Î·Ù·ÎÙ‹ÛÂȘ. ∞ÊÔ‡ Ë Î·Ù¿ÎÙËÛË ÌÈ·˜ ÁÏÒÛÛ·˜, Î·È È‰›ˆ˜ Ù‹˜ ∂ÏÏËÓÈ΋˜, Â›Ó·È ¤ÚÁÔ ˙ˆ‹˜.

O ¶Ú‡Ù·Ó˘ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ∫·ıËÁËÙ‹˜ °ÂÒÚÁÈÔ˜ ¢. ª·ÌÈÓÈÒÙ˘

¶·È‰È·ÙÚÈ΋ 2005;68:325-328

Pediatr Sept-Oct 05

17-10-05

16:51

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329

¶·ıÔÊ˘ÛÈÔÏÔÁ›· ÙˆÓ ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ ∂. ∫·Ú·Ù˙¿ - •ÈÊÈÏ›‰Ô˘1, ª. ∞ı·Ó·Û›Ô˘ - ªÂÙ·Í¿2

¶ÂÚ›ÏË„Ë ∏ ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ˜ ÔÊ›ÏÂÙ·È Û ÌÂÙ¿ÏÏ·ÍË Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ·ÓÙÈηٿÛÙ·ÛË ÙÔ˘ ÁÏÔ˘Ù·ÌÈÓÈÎÔ‡ ÔͤԘ ·fi ÙÔ ·ÌÈÓÔ͇ ‚·Ï›ÓË ÛÙË ı¤ÛË 6 Ù˘ ‚-·Ï‡ÛÔ˘ Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ (‚6glu val) Î·È ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ S (a2b2S). ∞ÔÎÏÂÈÛÙÈ΋ ȉÈfiÙËÙ· Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ S Â›Ó·È Ó· ÔÏ˘ÌÂÚ›˙ÂÙ·È fiÙ·Ó ·Ô͢ÁÔÓÒÓÂÙ·È, Ì ·ÔÙ¤ÏÂÛÌ· ÙÔÓ Û¯ËÌ·ÙÈÛÌfi ·Ê˘‰·ÙˆÌ¤ÓˆÓ ¿Î·ÌÙˆÓ ‰ÚÂ·ÓÔ΢ÙÙ¿ÚˆÓ. Œˆ˜ ÙȘ ·Ú¯¤˜ Ù˘ ÙÂÏÂ˘Ù·›·˜ ‰ÂηÂÙ›·˜ ıˆÚÔ‡ÓÙ·Ó fiÙÈ Ù· ¿Î·ÌÙ· ‰ÚÂ·ÓÔ·ÙÙ·Ú· ¢ı‡ÓÔÓÙ·È ÁÈ· ÙËÓ ÚfiÎÏËÛË ÙˆÓ ÂÒ‰˘ÓˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÎÚ›ÛÂˆÓ Ù˘ ÓfiÛÔ˘. ¡ÂfiÙÂÚ˜ ÌÂϤÙ˜ ·¤‰ÂÈÍ·Ó fiÙÈ Ù· ıÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È· Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ÂÓÂÚÁÔÔ›ËÛ˘ ÂÓfi˜ ÔχÏÔÎÔ˘ ÔÏ˘·Ú·ÁÔÓÙÈÎÔ‡ Ì˯·ÓÈÛÌÔ‡. ∆· ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú· ÙÔ˘ stress ·Ú¯Èο ÚÔÛÎÔÏÏÒÓÙ·È ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ ÙˆÓ ·ÁÁ›ˆÓ Ù˘ ÌÈÎÚÔ΢ÎÏÔÊÔÚ›·˜ ηÈ, ·ÎÔÏÔ‡ıˆ˜, ·ÁÈ‰Â‡Ô˘Ó Ù· ¿Î·ÌÙ· ‰ÚÂ·ÓÔ·ÙÙ·Ú· ÁÈ· Ó· Ù· ·ÔÌ·ÎÚ‡ÓÔ˘Ó ·fi ÙËÓ Î˘ÎÏÔÊÔÚ›·. ™ÙËÓ Â‰Ú·›ˆÛË ÙÔ˘ ·Ú¯ÈÎÔ‡ ıÚfiÌ‚Ô˘ Û˘ÌÌÂÙ¤¯Ô˘Ó: ·) ·Ú¿ÁÔÓÙ˜ ·fi ÙËÓ ÂÚ˘ıÚÔ΢ÙÙ·ÚÈ΋ ÌÂÌ‚Ú¿ÓË Ô˘ ¤¯ÂÈ ˘ÔÛÙ› ‚Ï¿‚Ë ÏfiÁˆ Ù˘ ˘ÔÍ›·˜, Ù·˘Ùfi¯ÚÔÓ· Ì ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÔÚÈÛÌ¤ÓˆÓ ·Ú·ÁfiÓÙˆÓ ‹Í˘ ÙÔ˘ Ï¿ÛÌ·ÙÔ˜, ‚) ·Ú¿ÁÔÓÙ˜ ·fi ÙÔ ÂÓ‰Ôı‹ÏÈÔ ÙˆÓ ·ÁÁ›ˆÓ (·ÓÙ·ÁˆÓÈÛÌfi˜ ·ÁÁÂÈÔÛ˘ÛÙ·ÏÙÈÎÒÓ Î·È ·ÁÁÂÈԉȷÛÙ·ÏÙÈÎÒÓ Ô˘ÛÈÒÓ), Á) ÂÈÊ·ÓÂȷο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘, ÙfiÛÔ ÙˆÓ ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ fiÛÔ Î·È ÙˆÓ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ ÙÔ˘ stress Ô˘ Û˘Ì‚¿ÏÏÔ˘Ó ÛÙËÓ ·Ú¯È΋ ÚÔÛÎfiÏÏËÛË ÙˆÓ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ, ‰) Ë ÂÓÂÚÁÔÔ›ËÛË Î·È ÚÔÛÎfiÏÏËÛË Î·È ÙˆÓ ¿ÏÏˆÓ ¤ÌÌÔÚÊˆÓ ÛÙÔȯ›ˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ (·ÈÌÔÂÙ¿ÏÈ·-Ï¢ÎÔ·ÙÙ·Ú·). ∏ ÌÂϤÙË Ù˘ ·ÏÏËÏÂ›‰Ú·Û˘ fiÏˆÓ ·˘ÙÒÓ ÙˆÓ ·Ú·ÁfiÓÙˆÓ ‚Ô‹ıËÛ ÛÙËÓ Î·Ù·ÓfiËÛË Ù˘ ·ıÔÁ¤ÓÂÈ·˜ ÙˆÓ ·ÁÁÂÈÔıÚÔÌ‚ˆÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ Î·Ï‡ÙÂÚË ·Ú·ÎÔÏÔ‡ıËÛË, ıÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË, ÔÈfiÙËÙ· ˙ˆ‹˜ Î·È ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ. →

1 °’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£, πÔÎÚ¿ÙÂÈÔ °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢ 2 ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£, πÔÎÚ¿ÙÂÈÔ °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢ AÏÏËÏÔÁÚ·Ê›·: ∂Ï›˙· ∫·Ú·Ù˙¿-•ÈÊÈÏ›‰Ô˘ πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢ ∫ˆÓÛÙ·ÓÙÈÓÔ˘fiψ˜ 49 ∆.∫. 546 42, £ÂÛÛ·ÏÔÓ›ÎË ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 03-09-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 01-04-2005

§¤ÍÂȘ ÎÏÂȉȿ ¢ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ˜, ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú· ÙÔ˘ stress, ÂÓ‰Ôı‹ÏÈÔ, ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È·.

™˘ÓÙÔÌÔÁڷʛ˜ VCAM1

∞ÁÁÂȷο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ (Vascular cell adhesion molecules 1) ICAM ∂Ó‰Ô΢ÙÙ¿ÚÈ· ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ (Intercellular adhesion molecules) VLA4 πÓÙÂÁÎÚ›ÓË ·4b1 (Very late antigen-4 ‹ integrin alpha 4 beta 1 ‹ a4‚1) CD36 ‹ ªÂÌ‚Ú·ÓÈ΋ ·ÈÌÔÂÙ·Ïȷ΋ GPIV ÁÏ˘ÎÔÚˆÙ½ÓË πV (Platelet glycoprotein IV) GPIb °Ï˘ÎÔÚˆÙ½ÓË Ib (Glycoprotein Ib) GPIIb/IIIa °Ï˘ÎÔÚˆÙ½ÓË (b3 ÈÓÙÂÁÎÚ›ÓË) (Glycoprotein) VNR ∂Ó‰ÔıËÏÈ·Îfi˜ ˘Ô‰Ô¯¤·˜ Ù˘ ‚ÈÙÚÔÓÂÎÙ›Ó˘ (Vitronectin receptor) CD44 ¶ÚˆÙÂÔÁÏ˘Î¿ÓË Ì 5 ÈÛÔÌÂÚ‹ (Proteoglycan) FcR Àԉԯ‡˜ ÂÓ‰ÔıËÏÈ·ÎÔ‡ ΢ÙÙ¿ÚÔ˘ (Fragments counter receptor)

B-CAM/Lu ¶ÚˆÙÂ˚ÓÈÎfi ÌfiÚÈÔ ÚÔÛÎfiÏÏËÛ˘ ‚·ÛÈ΋˜ ÌÂÌ‚Ú¿Ó˘ (Basal cell adhesion molecule/Lutheran protein) ET1 ∂Ó‰ÔıËÏ›ÓË 1 (·ÁÁÂÈÔÛ˘ÛÙ·ÏÙÈ΋) (Enthothelin 1) PS ºˆÛÊ·ÙȉÈÏÔÛÂÚ›ÓË (Phosphatidylserine) SPLA2 ºˆÛÊÔÏÈ¿ÛË ∞2 (Phospholipase A2) IL1 πÓÙÂÚÏ¢ΛÓË 1 (Interleukin 1) IL4 πÓÙÂÚÏ¢ΛÓË 4 (Interleukin 4) TNFa ¶·Ú¿ÁˆÓ Ó¤ÎÚˆÛ˘ fiÁÎÔ˘ · (Tumor necrosis factor a) IFNÁ πÓÙÂÊÂÚfiÓË Á (Interferone Á) ∏ªW À„ËÏÔ‡ ÌÔÚÈ·ÎÔ‡ ‚¿ÚÔ˘˜ ÔÏ˘ÌÂÚ‹ multimer ÙÔ˘ ·Ú¿ÁÔÓÙ· Von Willebrand vWF (High molecular weight multimer Von Willebrand factor) NO ªÔÓÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘ (·ÁÁÂÈԉȷÛÙ·ÏÙÈÎfi) (Nitric oxide) MCHC ª¤ÛË Û˘ÁΤÓÙÚˆÛË ·ÈÌÔÛÊ·ÈÚ›Ó˘ ÛÙ· ÂÚ˘ıÚ¿ ¶·È‰È·ÙÚÈ΋ 2005;68:329-335

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·330

330

∂. ∫·Ú·Ù˙¿ - •ÈÊÈÏ›‰Ô˘ Î·È Û˘Ó.

∂ÈÛ·ÁˆÁ‹ ∏ ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ˜ Â›Ó·È Ì›· ·fi ÙȘ Û˘¯ÓfiÙÂÚ˜ ÎÏËÚÔÓÔÌÈΤ˜ ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ. O Ingram, ÙÔ 1956, ·¤‰ÂÈÍ fiÙÈ Ë ÌÔÚȷ΋ ‚Ï¿‚Ë ÔÊ›ÏÂÙ·È ÛÙËÓ ·ÓÙÈηٿÛÙ·ÛË ÙÔ˘ ÁÏÔ˘Ù·ÌÈÓÈÎÔ‡ ÔͤԘ ·fi ÙË ‚·Ï›ÓË ÛÙË ı¤ÛË 6 Ù˘ ‚-·Ï‡ÛÔ˘ Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘, Ô˘ ·ÚÁfiÙÂÚ· ·ԉ›¯ıËΠfiÙÈ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ÛËÌÂȷ΋˜ ÌÂÙ¿ÏÏ·Í˘ (ÛÙÔ Îˆ‰ÈÎfiÓÈÔ GAG Û GTG) ÛÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ı¤ÛË Ù˘ ÚÒÙ˘ ÂÎÊÚ·˙fiÌÂÓ˘ ·ÏÏËÏÔ˘¯›·˜ ÙÔ˘ ‚-ÁÔÓȉ›Ô˘ (1-4). ∞ÔÙ¤ÏÂÛÌ· Ù˘ ÌÂÙ¿ÏÏ·Í˘ ·˘Ù‹˜ Â›Ó·È Ô Û¯ËÌ·ÙÈÛÌfi˜ Ù˘ HbS, Ë ÔÔ›· ÛÂ Û˘Óı‹Î˜ ˘Ô͢ÁÔÓ·ÈÌ›·˜ ÂÌÊ·Ó›˙ÂÈ ÌÂȈ̤ÓË ‰È·Ï˘ÙfiÙËÙ·, ÔÏ˘ÌÂÚ›˙ÂÙ·È (ÂÏÈÎÔÂȉ›˜ ÓËÌ·ÙÔÂȉ›˜ Û¯ËÌ·ÙÈÛÌÔ›) Î·È Î·ıÈ˙¿ÓÂÈ ˘fi ÌÔÚÊ‹Ó ÂÓ‰Ô΢ÙÙ¿ÚÈˆÓ ÎÚ˘ÛÙ¿ÏÏˆÓ (1,2). À„ËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ÔÏ˘ÌÂÚÒÓ ÚÔηÏÔ‡Ó ·Ú·ÌfiÚʈÛË ÙˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ Î·È ÙÔÓ Û¯ËÌ·ÙÈÛÌfi ÙˆÓ ¿Î·ÌÙˆÓ ‰ÚÂ·ÓÔ΢ÙÙ¿ÚˆÓ (2,5,6). ∏ ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È Î˘Ú›ˆ˜ ·fi ÂÒ‰˘Ó· ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È·, Ô˘ fiÙ·Ó Û˘Ì‚Ô‡Ó ÛÙÔ˘˜ Ó‡ÌÔÓ˜ ‹ ÛÙÔ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· (∫¡™) Â›Ó·È ·ÂÈÏËÙÈο ÁÈ· ÙË ˙ˆ‹ ÙˆÓ ·Û¯fiÓÙˆÓ (1,2,5). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ë ·ıÔÊ˘ÛÈÔÏÔÁ›· ·˘ÙÒÓ ÙˆÓ ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ¤ÁÈÓ ·ÓÙÈΛÌÂÓÔ ÂÎÙÂÙ·Ì¤ÓˆÓ ÌÂÏÂÙÒÓ, ηı’ fiÛÔÓ ·ԉ›¯ıËΠfiÙÈ Ë Â¤ÏÂ˘Û‹ ÙÔ˘˜ ‰ÂÓ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ù˘ ·ÁÁÂȷ΋˜ ·fiÊڷ͢ ÌfiÓÔ ·fi Ù· ¿Î·ÌÙ· ·Ú·ÌÔÚʈ̤ӷ ‰ÚÂ·ÓÔ·ÙÙ·Ú·, ·ÏÏ¿ Î·È Ù˘ ÂÓÂÚÁÔÔ›ËÛ˘ Î·È ·ÏÏËÏÂ›‰Ú·Û˘ ÔÏÏÒÓ ¿ÏÏˆÓ ·Ú·ÁfiÓÙˆÓ, fiˆ˜ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ ÙÔ˘ stress, Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ, ·ÈÌÔÂÙ·Ï›ˆÓ, ÔÚÈÛÌ¤ÓˆÓ ·Ú·ÁfiÓÙˆÓ Ù˘ ‹Í˘ ÙÔ˘ Ï¿ÛÌ·ÙÔ˜, ÙÔ˘ ·ÁÁÂÈ·ÎÔ‡ ÂÓ‰ÔıËÏ›Ô˘ Î·È ÙˆÓ Î·ÏÔ‡ÌÂÓˆÓ ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ (4). OÈ ÏÔÈÌÒÍÂȘ Î·È Ë ˘ÔÍ›· ˘ÚÔ‰ÔÙÔ‡Ó ˆ˜ Â› ÙÔ Ï›ÛÙÔÓ ÙËÓ ¤Ó·ÚÍË ÙˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ Ô˘ Ô‰ËÁÔ‡Ó Û ÈÛÙÈ΋ ÈÛ¯·ÈÌ›· Î·È fiÓÔ ÛÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ÂÚÈÔ¯‹ (1,2,7-9).

¶·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ‰ÚÂ¿ÓˆÛ˘ ∆· ıÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È· ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ Û˘Ì‚·›ÓÔ˘Ó ˆ˜ Â› ÙÔ Ï›ÛÙÔÓ ÛÙÔ Â›Â‰Ô Ù˘ ÌÈÎÚÔ΢ÎÏÔÊÔÚ›·˜, Ì ·ÚÈÔ˘˜ ˘‡ı˘ÓÔ˘˜ ÛÙËÓ ¤Ó·ÚÍ‹ ÙÔ˘˜ Ù· ÏÈÁfiÙÂÚÔ ˘ÎÓ¿ Î·È ·Ú·ÌÔÚʈ̤ӷ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú·, Ù· ηÏÔ‡ÌÂÓ· ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú· ¶·È‰È·ÙÚÈ΋ 2005;68:329-335

(¢∂∫) ÙÔ˘ stress. ∆· ¢∂∫ ÙÔ˘ stress ÚÔÛÎÔÏÏÒÓÙ·È ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ ÙˆÓ ÌÂÙ·ÙÚȯÔÂȉÒÓ ÊÏ‚ȉ›ˆÓ, ÚÔηÏÒÓÙ·˜ ·Ú¯Èο Ì›ˆÛË Ù˘ ÚÔ‹˜ ÙÔ˘ ·›Ì·ÙÔ˜, ·ÏÏ¿ fi¯È Ï‹ÚË ·fiÊÚ·ÍË. ∞ÎÔÏÔ‡ıˆ˜, Â¤Ú¯ÂÙ·È Ì˯·ÓÈ΋ ·Á›‰Â˘ÛË ÙˆÓ ‰ÚÂ·ÓÔ΢ÙÙ¿ÚˆÓ Î·È Ï‹Ú˘ ·fiÊÚ·ÍË ÙÔ˘ ·ÁÁ›Ԣ (4,5,7,10). ¶ÂÈÚ·Ì·ÙÈΤ˜ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ Ë ÂÓ‰ÔıËÏȷ΋ ÚÔÛÎfiÏÏËÛË ÙˆÓ ¢∂∫ Î·È ÙˆÓ ‰ÚÂ·ÓÔ΢ÙÙ¿ÚˆÓ, Ô Û¯ËÌ·ÙÈÛÌfi˜ ıÚfiÌ‚Ô˘, Ë ·fiÊÚ·ÍË ÙÔ˘ ·ÁÁ›Ԣ Î·È Ë Â·ÎfiÏÔ˘ıË ÈÛÙÈ΋ ÈÛ¯·ÈÌ›· Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ·ÏÏËÏÂ›‰Ú·Û˘ ÔÏÏÒÓ ·Ú·ÁfiÓÙˆÓ (7,8).

OÈ ·Ú¿ÁÔÓÙ˜ ·˘ÙÔ› ›ӷÈ: ·) ∆· ÂÈÊ·ÓÂȷο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ ÙˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ (΢ڛˆ˜ ÙˆÓ ¢∂∫) (cell adhension molecules ‹ ICAM) Î·È ÙˆÓ ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ (vascular cell adhesion molecules ‹ VCAM1) (7,11,12). ∞fi ÙËÓ Î·ÙËÁÔÚ›· ·˘Ù‹, ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ Û˘ÌÌÂÙ¤¯Ô˘Ó: i) Ë ˘ÂÚÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ ÛÂÏÂÎÙÈÓÒÓ (E Selectin, L selectin, P selectin) (7), ii) Ë ˘ÂÚÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ (IgG gene), iii) ·fi ÙȘ ÈÓÙÂÁÎÚ›Ó˜, ΢ڛˆ˜ Ë ‚1 ÈÓÙÂÁÎÚ›ÓË (very late antigen 4, VLA4) ‹ ·4‚1, Ë ‚3 ÈÓÙÂÁÎÚ›ÓË (GPIIb/πππ·), Ë ÈÓÙÂÁÎÚ›ÓË GPπb Î·È Ô ˘Ô‰Ô¯¤·˜ Ù˘ ‚ÈÙÚÔÓÂÎÙ›Ó˘ (vitronectin receptror ‹ VNR) (7,12,13) Î·È iv) ‰È¿ÊÔÚ˜ ¿ÏϘ ÔÌ¿‰Â˜ ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ, fiˆ˜ Ù· CD36, CD44, ıÚÔÌ‚ÔÛÔÓ‰›ÓË Î·È ˘„ËÏÔ‡ ÌÔÚÈÎÔ‡ ‚¿ÚÔ˘˜ ÔÏ˘ÌÂÚ‹ ÙÔ˘ ·Ú¿ÁÔÓÙ· Von Willebrand (∏ªW multimer vWF) (7,11-13) (¶›Ó·Î·˜ 1). ‚) ∆Ô ÂÓ‰Ôı‹ÏÈÔ ÙˆÓ ·ÁÁ›ˆÓ. OÈ ·ÏÏËÏÂȉڿÛÂȘ ÙˆÓ ‰ÚÂ·ÓÔ΢ÙÙ¿ÚˆÓ Î·È ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ Ô‰ËÁÔ‡Ó Û ·ÏÏÔ›ˆÛË ÙÔ˘ ÂÓ‰ÔıËÏ›Ô˘, ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ Ù˘ ·ÁÁÂÈÔÛ˘ÛÙ·ÏÙÈ΋˜ ÂÓ‰ÔıËÏ›Ó˘ 1 (Et1) Î·È ÌÂȈ̤ÓË ÙÔ˘ ·ÁÁÂÈԉȷÛÙ·ÏÙÈÎÔ‡ ÌÔÓÔÍÂȉ›Ô˘ ÙÔ˘ ·˙ÒÙÔ˘ (¡O), ÂÈ‚Ú·‰‡ÓÔÓÙ·˜ Ì ·˘ÙfiÓ ÙÔÓ ÙÚfiÔ ÙË ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜ Î·È ÂȉÂÈÓÒÓÔÓÙ·˜ ÙÔ ıÚÔÌ‚ˆÙÈÎfi ÂÂÈÛfi‰ÈÔ (3,6,7,14,15). ™ÙËÓ Âȉ›ӈÛË ÙÔ˘ ıÚÔÌ‚ˆÙÈÎÔ‡ ÂÂÈÛÔ‰›Ô˘ Â›Ó·È Èı·Ófi Ó· Û˘Ì‚¿ÏÏÔ˘Ó Î·È Ù· ¯·ÌËÏ¿ Â›‰· Ù˘ ·ÁÁÂÈԉȷÛÙ·ÏÙÈ΋˜ ÚÔÛÙ·Î˘ÎÏ›Ó˘ (prostacyclin) Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È ÛÙÔ˘˜ ¿Û¯ÔÓÙ˜ ·fi ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ (5,10,14,15). ∏ ·‡ÍËÛË ÙˆÓ Î˘ÎÏÔÊÔÚÔ‡ÓÙˆÓ ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ, ·ÎfiÌË Î·È Û ʿÛÂȘ ‡ÊÂÛ˘ Ù˘ ÓfiÛÔ˘, ÂȂ‚·ÈÒÓÂÈ ÙËÓ ÂÓ‰ÔıËÏȷ΋ ‚Ï¿‚Ë ‹ ÙËÓ ·ÏÏÔ›ˆÛË Ù˘ ÂÓ‰ÔıËÏȷ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (5,10,16,17). Á) ¶·Ú¿ÁÔÓÙ˜ Ï¿ÛÌ·ÙÔ˜: ÙÔ ÈÓˆ‰ÔÁfiÓÔ, Ë ÊÈÌÚÔÓÂÎÙ›ÓË, Ë ıÚÔÌ‚ÔÛÔÓ‰›ÓË Î·È Ô

Pediatr Sept-Oct 05

17-10-05

16:51

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∞ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È· ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ

¶›Ó·Î·˜ 1. ∂ÈÊ·ÓÂȷο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ Ô˘ ÂÓÂÚÁÔÔÈÔ‡ÓÙ·È ÛÙ· ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È· Ù˘ ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋˜ ÓfiÛÔ˘ ™ÂÏÂÎÙ›Ó˜ (Selectin superfamily) ∂ ™ÂÏÂÎÙ›ÓË (ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ) L ™ÂÏÂÎÙ›ÓË (Ï¢ÎÔ΢ÙÙ¿ÚˆÓ) ƒ ™ÂÏÂÎÙ›ÓË (ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È ·ÈÌÔÂÙ·Ï›ˆÓ) ÀÂÚÔÈÎÔÁ¤ÓÂÈ· ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ: ηÙËÁÔÚ›· ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ [Immunoglobulin gene superfamily ‹ IgG gene (C2 type)] ÀÂÚÔÈÎÔÁ¤ÓÂÈ· ÈÓÙÂÁÎÚÈÓÒÓ (Integrine superfamily) ‚1 ÈÓÙÂÁÎÚ›ÓË ‹ alpha4 beta1 ‹ VLA4 ‚3 ÈÓÙÂÁÎÚ›ÓË ‹ ÁÏ˘ÎÔÚˆÙ½ÓË GPIIb/πππ· ‚3 ÈÓÙÂÁÎÚ›ÓË ‹ ÁÏ˘ÎÔÚˆÙ½ÓË GPIb Àԉԯ‡˜ Ù˘ ‚ÈÙÚÔÓÂÎÙ›Ó˘ ‹ VNR ¢È¿ÊÔÚ· (Miscellaneous group) CD36 ‹ GPIV (·ÈÌÔÂÙ·Ïȷ΋ ÁÏ˘ÎÔÚˆÙ½ÓË) CD44 ‹ ÚˆÙÂÔÁÏ˘Î¿ÓË CD44 µ-CAM/Lu (ÌfiÚÈÔ ÚÔÛÎfiÏÏËÛ˘ ‚·ÛÈ΋˜ ÌÂÌ‚Ú¿Ó˘) FcR (˘ԉԯ›˜ ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ) Thrombospondin (ıÚÔÌ‚ÔÛÔÓ‰›ÓË) À„ËÏÔ‡ ÌÔÚÈ·ÎÔ‡ ‚¿ÚÔ˘˜ ÔÏ˘ÌÂÚ‹ ÙÔ˘ ·Ú¿ÁÔÓÙ· Von Willebrand (HMW-vWF)

·Ú¿ÁˆÓ Von Willebrand (vWF). ™˘ÌϤÁÌ·Ù· ıÚÔÌ‚›Ó˘-·ÓÙÈıÚÔÌ‚›Ó˘ ‚Ú¤ıËÎ·Ó ·˘ÍË̤ӷ ÛÙÔ Ï¿ÛÌ· ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈÎÒÓ ·ÛıÂÓÒÓ, ·ÎfiÌË Î·È Û ʿÛÂȘ ËÚÂÌ›·˜ Ù˘ ÓfiÛÔ˘ (7,18,19). ‰) ∆· Ï¢ÎÔ·ÙÙ·Ú·, Â›Û˘, Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙ· ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È· ›Ù Ì ÙËÓ ÚÔÛÎfiÏÏËÛ‹ ÙÔ˘˜ ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ, ›Ù ÂÓÂÚÁÔÔÈÒÓÙ·˜ ÙËÓ ¤ÎÊÚ·ÛË ÙˆÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏËÛ˘ (20-22). ¶·Ú¿ÏÏËÏ· Ì ÙȘ ÂÓ‰Ô΢ÙÙ¿ÚȘ ÏÂÈÙÔ˘ÚÁÈΤ˜ ÌÂÙ·‚ÔϤ˜ (·ÏÏÔȈ̤ÓË ÔÏÈÎfiÙËÙ· HbS ÏfiÁˆ Ù˘ ˘‰ÚfiÊԂ˘ ‚·Ï›Ó˘ Î·È Û¯ËÌ·ÙÈÛÌfi˜ ÔÏ˘ÌÂÚÒÓ), Â¤Ú¯ÂÙ·È Î·È ‚Ï¿‚Ë Ù˘ ÌÂÌ‚Ú¿Ó˘ ÙÔ˘ ÂÚ˘ıÚÔ΢ÙÙ¿ÚÔ˘ Ì ·ÒÏÂÈ· ÈfiÓÙˆÓ ∫+ Î·È ‡‰·ÙÔ˜, ·Ê˘‰¿ÙˆÛË ÙÔ˘ ΢ÙÙ¿ÚÔ˘, ·‡ÍËÛË Ù˘ ÂÓ‰Ô΢ÙÙ¿ÚÈ·˜ Û˘ÁΤÓÙÚˆÛ˘ Hb (MCHC) Î·È Â›ÛÔ‰Ô˜ ÈfiÓÙˆÓ Ca++ Ô˘ ·ÔÙÂÏ› ÙÔÓ Î‡ÚÈÔ ·Ú¿ÁÔÓÙ· Ù˘ ÌÂȈ̤Ó˘ ¢ηÌÙfiÙËÙ·˜ ÙˆÓ ‰ÚÂ·ÓÔÌ¤ÓˆÓ ÂÚ˘ıÚÒÓ (2,4,7,11). ∆·˘Ùfi¯ÚÔÓ· Ì ÙË ‰È·Ù·Ú·¯‹ ÙˆÓ ·ÓÙÏÈÒÓ ∫+, Ca++ Î·È Ù˘ Û˘ÌÌÂÙ·ÊÔÚ¿˜ K-Cl, ‰È·Ù·Ú¿ÛÛÂÙ·È Î·È Ô ‰›·˘ÏÔ˜ Gardos (Û‡ÛÙËÌ· ·ÒÏÂÈ·˜ ∫+ Î·È H2O ·fi ÙÔ Î‡ÙÙ·ÚÔ Ô˘ ÂÓÂÚÁÔÔÈÂ›Ù·È ·fi ÙËÓ ÚfiÛηÈÚË ÂÈÛÚÔ‹ ÈfiÓÙˆÓ Ca++ ÂÓÙfi˜ ÙÔ˘ ΢ÙÙ¿ÚÔ˘) Î·È Â¤Ú¯ÂÙ·È ÂÚ·ÈÙ¤Úˆ Âȉ›ӈÛË Ù˘ ÂÓ‰Ô΢ÙÙ¿ÚÈ·˜ ·Ê˘‰¿ÙˆÛ˘ Î·È Ù˘ ‰ÚÂ¿ÓˆÛ˘ (2,4,7,23) (∂ÈÎfiÓ· 1). ¡ÂfiÙÂÚ˜ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ Î·È ¿ÏÏÔÈ ·Ú¿ÁÔÓÙ˜ ÂÎÙfi˜ ·fi ÙÔ˘˜ ·ÓˆÙ¤Úˆ Û˘Ì‚¿Ï-

O2

HbS ÔÏ˘ÌÂÚ‹

Ca++ Ca++ K+ K+

P∏ K+ K+ Cl- ClH2O H2O

H2O ¢›·˘ÏÔ˜ Gardos

™˘ÌÌÂÙ·ÊÔÚ¿ K-Cl

∂ÈÎfiÓ· 1. ªÂÙ·‚ÔϤ˜ Ù˘ ÂÚ˘ıÚÔ΢ÙÙ·ÚÈ΋˜ ÌÂÌ‚Ú¿Ó˘ Ô˘ Ô‰ËÁÔ‡Ó ÛÙË ‰ÚÂ¿ÓˆÛË (∆ÚÔÔÔÈË̤ÓÔ ·fi: The Molecular Basis of Blood Diseases. Stamatoyannopoulos G et al. 3rd ed. 2001. p. 247).

ÏÔ˘Ó ÛÙËÓ ·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ‰ÚÂ¿ÓˆÛ˘. ∆· Ó·ڿ ÂÚ˘ıÚÔ·ÙÙ·Ú· (ÏÈÁfiÙÂÚÔ ˘ÎÓ¿ Î·È ·Ú·ÌÔÚʈ̤ӷ), Ù· ÔÓÔÌ·˙fiÌÂÓ· ¢∂∫ ÙÔ˘ stress, ¤¯Ô˘Ó ÛÙËÓ ÂÈÊ¿ÓÂÈ¿ ÙÔ˘˜ ÚˆÙ½Ó˜ (ÂÈÊ·ÓÂȷο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘) Ô˘ ¯ÚËÛÈÌÂ‡Ô˘Ó ÁÈ· ÙË ÌÂٷ͇ ÙÔ˘˜ Û‡Ó‰ÂÛË ÛÙÔÓ Ì˘ÂÏfi Î·È Ù· ÔÔ›· ‰ÂÓ ·Ó¢ڛÛÎÔÓÙ·È ÛÙËÓ ÂÈÊ¿ÓÂÈ· ÙˆÓ ÒÚÈÌˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ (4,5,7,13). ™˘ÁÎÂÎÚÈ̤ӷ, Ë ÈÓÙÂÁÎÚ›ÓË alpha 4 beta 1 (·4‚1) ‹ VLA4 ·Ó·ÁÓˆÚ›˙ÂÈ ¿ÌÂÛ· Î·È ÚÔÛÎÔÏÏ¿Ù·È ÛÙÔ VCAM1 ÌfiÚÈÔ ÙÔ˘ ÂÓÂÚÁÔÔÈË̤ÓÔ˘ ·fi Ù· ÂÚ˘ıÚÔ·ÙÙ·Ú· ÂÓ‰ÔıËÏÈ·ÎÔ‡ ΢ÙÙ¿ÚÔ˘ (Û·Ó ¤Ó· ¤ÙÂÚÔ ‹ÌÈÛ˘). ∏ VLA-4 Â›Ó·È ÙÔ ÌfiÓÔ ÌfiÚÈÔ Ô˘ ·Ó·ÁÓˆÚ›˙ÂÙ·È ·fi Ù· VCAM1. ∆o ÌfiÚÈÔ CD36, Ô˘ ÔÓÔÌ¿˙ÂÙ·È Î·È ·ÈÌÔÂÙ·Ïȷ΋ ÁÏ˘ÎÔÚˆÙ½ÓË (GPIV), ÂÎÊÚ¿˙ÂÙ·È ÛÙ· ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú· ÙÔ˘ stress Î·È Û˘Ó‰¤ÂÙ·È Ì ¤Ó· ¿ÏÏÔ ÌfiÚÈÔ CD36 ÙÔ˘ ÂÓ‰ÔıËÏ›Ô˘ ‹ Ì ÙÔÓ ÂÓ‰ÔıËÏÈ·Îfi VNR, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ Û·Ó Á¤Ê˘Ú· ÙË ıÚÔÌ‚ÔÛÔÓ‰›ÓË Ô˘ ·Ú¿ÁÂÙ·È ·fi Ù· ÂÓÂÚÁÔÔÈË̤ӷ ·ÈÌÔÂÙ¿ÏÈ· (7,10,24). ∞˘Ù¤˜ ÔÈ ‰‡Ô ˘„ËÏ‹˜ Û˘Ó¿ÊÂÈ·˜ ·ÓÙȉڿÛÂȘ Â›Ó·È ÔÈ ϤÔÓ ˘‡ı˘Ó˜ ÁÈ· ÙËÓ ÚÔÛÎfiÏÏËÛË ÙˆÓ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ ÙˆÓ ÌÂÙ·ÙÚȯÔÂȉÈÎÒÓ ÊÏ‚ȉ›ˆÓ. ∂ÈϤÔÓ, ˘„ËÏÔ‡ ÌÔÚÈ·ÎÔ‡ ‚¿ÚÔ˘˜ ÌfiÚÈ· ÙÔ˘ vWF Û˘ÌÌÂÙ¤¯Ô˘Ó ˆ˜ Á¤Ê˘Ú˜ ÛÙËÓ ÚÔÛÎfiÏÏËÛË ÙÔ˘ GPππb/πππ· ÂÈÊ·ÓÂÈ·ÎÔ‡ ÌÔÚ›Ô˘ ÚÔÛÎfiÏÏËÛ˘ ÙÔ˘ ¢∂∫ Ì ÙÔ GP1b ‹ ÙÔÓ VNR (ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ ÙÔ˘ ÂÓ‰ÔıËÏÈ·ÎÔ‡ ΢ÙÙ¿ÚÔ˘) (7,13,15) (∂ÈÎfiÓ· 2). ∏ ÚÔÛÎfiÏÏËÛË ÙˆÓ ÒÚÈÌˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ù˘ ¿ıÚÔÈÛ˘ ·ÚÓËÙÈÎÒÓ ÊÔÚÙ›ˆÓ ÛÙËÓ ·ÏÏÔȈ̤ÓË Î˘ÙÙ·ÚÈ΋ ÌÂÌ‚Ú¿ÓË Î·È Û‡Ó‰ÂÛ‹˜ ÙÔ˘˜ ̤ۈ Ù˘ IgG ·ÓÔÛÔÛÊ·ÈÚ›Ó˘ Ì ÙÔ˘˜ (FcR) ˘ԉԯ›˜ ÙÔ˘ ÂÓ‰ÔıËÏÈ·ÎÔ‡ ΢ÙÙ¿ÚÔ˘, ¯ˆÚ›˜ fï˜ ·˘Ùfi Ó· ·›˙ÂÈ ·ÍÈfiÏÔÁÔ ÚfiÏÔ ÛÙËÓ ¤Ó·ÚÍË ÙÔ˘ ¶·È‰È·ÙÚÈ΋ 2005;68:329-335

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·332

332

GPIb ‹ VNR

HMW vWF

GPIIb/IIIa

∂ÓÂÚÁÔÔÈË̤ӷ ·ÈÌÔÂÙ¿ÏÈ· CD36 ‹ VNR

£ÚÔÌ‚ÔÛÔÓ‰›ÓË VCAM1

CD36

¢∂∫ Stress

∂Ó‰ÔıËÏÈ·Îfi ·ÙÙ·ÚÔ

∂. ∫·Ú·Ù˙¿ - •ÈÊÈÏ›‰Ô˘ Î·È Û˘Ó.

VLA4

∂ÈÎfiÓ· 2. ¶ÚÔÛÎfiÏÏËÛË ÙˆÓ ¢∂∫ stress ÛÙÔ ·ÁÁÂÈ·Îfi ÂÓ‰Ôı‹ÏÈÔ (ÚˆÙÔÁÂÓ›˜ ˘„ËÏ‹˜ Û˘Ó¿ÊÂÈ·˜ ·ÓÙȉڿÛÂȘ).

ıÚÔÌ‚ˆÙÈÎÔ‡ ÂÂÈÛÔ‰›Ô˘ (4,13,16). ∂ÈϤÔÓ, ‚Ú¤ıËÎÂ Û˘Ó¿ÊÂÈ· ·Ó¿ÌÂÛ· ÛÙÔ B-CAM/Lu ÂÈÊ·ÓÂÈ·Îfi ÌfiÚÈÔ ÙÔ˘ ÒÚÈÌÔ˘ ‰ÚÂ·ÓÔ΢ÙÙ¿ÚÔ˘ Î·È Ù˘ ˘ÂÓ‰ÔıËÏȷ΋˜ Ï·ÌÈÓ›Ó˘, ·Ô‰ÂÈÎÓ‡ÔÓÙ·˜ ÙËÓ ÂÌÏÔ΋ Ù˘ ÂÓ‰ÔıËÏȷ΋˜ ‚Ï¿‚˘ ÛÙËÓ ·ıÔÁ¤ÓÂÛË Ù˘ ıÚÔÌ‚ˆÙÈ΋˜ ÎÚ›Û˘ (7,25,26). OÈ ·Ó·ÊÂÚı›Û˜ ‰˘Ó¿ÌÂȘ ÚÔÛÎfiÏÏËÛ˘ ‰ÚÔ˘Ó Û˘ÓÂÚÁÈο, Ô‰ËÁÒÓÙ·˜ ÛÙËÓ ·Ú¯È΋ ÚÔÛÎfiÏÏËÛË ÙˆÓ ¢∂∫ stress Î·È ÛÙËÓ ·ÎfiÏÔ˘ıË ·Á›‰Â˘ÛË ÙˆÓ ‰ÚÂ·ÓÔ΢ÙÙ¿ÚˆÓ (4,7,13). ∆·˘Ùfi¯ÚÔÓ· Ì ÙËÓ ÚÔÛÎfiÏÏËÛË ÙˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ Î·È ÙËÓ ÂÓ‰ÔıËÏȷ΋ ‚Ï¿‚Ë, ÂÓÂÚÁÔÔÈÂ›Ù·È Î·È Ë ‰È·‰Èηۛ· Ù˘ ‹Í˘. ∂ÎÙfi˜ ·fi ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ·Ú¿ÁÔÓÙ· vWF, ·˘ÍË̤ӷ Â›‰· ÈÓˆ‰ÔÁfiÓÔ˘, ÊÈÌÚÔÓÂÎÙ›Ó˘, ÚÔıÚÔÌ‚›Ó˘ Î·È Û˘ÌÏÂÁÌ¿ÙˆÓ ıÚÔÌ‚›Ó˘-·ÓÙÈıÚÔÌ‚›Ó˘ πππ ‚Ú¤ıËÎ·Ó ÙfiÛÔ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÎÚ›ÛÂˆÓ fiÛÔ Î·È Î·Ù¿ ÙË Ê¿ÛË ‡ÊÂÛ˘ Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜ (7,18,27). ™ÙËÓ ˘ÂÚËÎÙÈ΋ ηٿÛÙ·ÛË Û˘Ì‚¿ÏÏÂÈ Ë Ù·˘Ùfi¯ÚÔÓË ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Î·È Ë ·˘ÍË̤ÓË ·ÂÏ¢ı¤ÚˆÛË ·fi ·˘Ù¿ ıÚÔÌ‚ÔÛÔÓ‰›Ó˘, ƒ ÛÂÏÂÎÙ›Ó˘, ‚-ıÚÔÌ‚ÔÁÏÔ‚Ô˘Ï›Ó˘ Î·È ÙÔ˘ ·ÈÌÔÂÙ·ÏÈ·ÎÔ‡ ·Ú¿ÁÔÓÙ· 4, Ô˘ ‰ÚÔ˘Ó Û˘ÓÂÚÁÈο ÛÙËÓ Âȉ›ӈÛË ÙÔ˘ ıÚÔÌ‚ˆÙÈÎÔ‡ ÂÂÈÛÔ‰›Ô˘ (4,27-30). ∆· ÂÓÂÚÁÔÔÈË̤ӷ Î·È Î˘ÎÏÔÊÔÚÔ‡ÓÙ· ÂÓ‰ÔıËÏȷο ·ÙÙ·Ú· ÛÙÔ˘˜ ¿Û¯ÔÓÙ˜ ·fi ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ, Ë ‰È·Ù·Ú·¯‹ Ù˘ ·Û˘ÌÌÂÙÚ›·˜ ÙˆÓ ÊˆÛÊÔÏÈȉ›ˆÓ Ù˘ ÌÂÌ‚Ú¿Ó˘ ÙˆÓ ‰ÚÂ·ÓÔ΢ÙÙ¿ÚˆÓ Î·È Ë ¤ÎıÂÛË Ù˘ ·ÓÈÔÓÈ΋˜ ʈÛÊ·ÙȉÈÏÔÛÂÚ›Ó˘ (phosphatidylserine PS) ·ÔÙÂÏÔ‡Ó ¤Ó·Ó ÂÈϤÔÓ ÈÛÙÈÎfi ·Ú¿ÁÔÓÙ·, ˘‡ı˘ÓÔ ÁÈ· ÙËÓ ÂÓÂÚÁÔÔ›ËÛË Ù˘ ‹Í˘ Î·È ÙË “ıÚÔÌ‚ÔÊÈÏÈ΋ ηٿÛÙ·ÛË” ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ (4,17,27,28). ¶·È‰È·ÙÚÈ΋ 2005;68:329-335

ŸÛÔÓ ·ÊÔÚ¿ ÛÙ· ¿ÏÏ· ¤ÌÌÔÚÊ· ÛÙÔȯ›· ÙÔ˘ ·›Ì·ÙÔ˜, ‚Ú¤ıËΠfiÙÈ ‰ÂÓ Ì¤ÓÔ˘Ó ·Ì¤ÙÔ¯· ÛÙËÓ ÂΉ‹ÏˆÛË Ù˘ ÂÒ‰˘Ó˘ ÎÚ›Û˘. ∆· ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· Ô˘‰ÂÙÂÚfiÊÈÏ· Î·È Ù· ÌÔÓÔ·ÙÙ·Ú· Â›Ó·È Â›Û˘ ÂÓÂÚÁÔÔÈË̤ӷ ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ (20,22,31). ªÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ Ù· Ï¢ÎÔ·ÙÙ·Ú· ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ ·Ú¿ÁÔ˘Ó ÙÔ˘˜ ‰È·Ï˘ÙÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÈÓÙÂÚÏ¢ΛÓË 1 Î·È ·Ú¿ÁÔÓÙ· Ó¤ÎÚˆÛ˘ fiÁÎÔ˘-a (IL1 Î·È ∆¡Fa), Ô˘ ‰ÈÂÁ›ÚÔ˘Ó ÙËÓ ¤ÎÊÚ·ÛË ÙˆÓ ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ηÈ, ÛÙË Û˘Ó¤¯ÂÈ·, Ù· ÂÓÂÚÁÔÔÈË̤ӷ Ô˘‰ÂÙÂÚfiÊÈÏ· ÚÔÛÎÔÏÏÒÓÙ·È ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ Ì ÙË ÌÂÛÔÏ¿‚ËÛË ÙˆÓ ÛÂÏÂÎÙÈÓÒÓ, ȉȷ›ÙÂÚ· Ù˘ ∂ ÛÂÏÂÎÙ›Ó˘, ÙˆÓ ÈÓÙÂÁÎÚÈÓÒÓ Î·È ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ (4,7,10, 21). ∂ÈϤÔÓ, ÔÈ ÈÓÙÂÁÎÚ›Ó˜ Â›Ó·È ˘‡ı˘Ó˜ ÙfiÛÔ ÁÈ· ÙË ÛÙ·ıÂÚ‹ ÚÔÛÎfiÏÏËÛË fiÛÔ Î·È ÁÈ· ÙË ‰È·ÂÓ‰ÔıËÏȷ΋ ÌÂÙ·Ó¿ÛÙ¢ÛË ÙˆÓ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ (14,32). ∏ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ Î·È Ë ÚÔÛÎfiÏÏËÛ‹ ÙÔ˘˜ ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ Ô‰ËÁ› Û ÂÈ‚Ú¿‰˘ÓÛË Ù˘ ÚÔ‹˜ ÙÔ˘ ·›Ì·ÙÔ˜ ÛÙË ÌÈÎÚÔ΢ÎÏÔÊÔÚ›· (4,32). §fiÁˆ ·˘Ù‹˜ Ù˘ Û˘ÌÌÂÙÔ¯‹˜ ÙˆÓ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ ÛÙ· ·ÁÁÂÈÔıÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È·, Ô ˘„ËÏfi˜ ·ÚÈıÌfi˜ ÙÔ˘˜ ·ÔÙÂÏ› ¤Ó·Ó ȉȷ›ÙÂÚ· ÂÈΛӉ˘ÓÔ ‰Â›ÎÙË ÛÙÔ˘˜ ¿Û¯ÔÓÙ˜ ·fi ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ (4,5,10,21,22). ™ÙÔ˘˜ ·ÓˆÙ¤Úˆ ·Ú¿ÁÔÓÙ˜ ÚÔÛÙ›ıÂÙ·È Ë ‰Ú¿ÛË Ù˘ ÂÓ‰ÔıËÏ›Ó˘ 1 (∂t1), Ô˘ ˘ÂÚ·Ú¿ÁÂÙ·È ·fi Ù· ·ÏÏÔȈ̤ӷ ÂÓ‰ÔıËÏȷο ·ÙÙ·Ú· Î·È Ú˘ıÌ›˙ÂÈ ÙÔÓ ÙfiÓÔ ÙˆÓ ·ÁÁ›ˆÓ, ‰ÚÒÓÙ·˜ ·ÁÁÂÈÔÛ˘Û·ÛÙÈο, Û ·ÓÙ›ıÂÛË Ì ÙË ÌÂȈ̤ÓË ·Ú·ÁˆÁ‹ ÙÔ˘ ·ÁÁÂÈԉȷÛÙ·ÏÙÈÎÔ‡ ÌÔÓÔÍÂȉ›Ô˘ ÙÔ˘ ·˙ÒÙÔ˘ (¡O) (5,7,10,33).

O ÚfiÏÔ˜ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÛÙ· ıÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È· ¶Ú¤ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ Ë ¤ÎÊÚ·ÛË ÙˆÓ ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ÙˆÓ ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È È‰È·›ÙÂÚ· ÙÔ˘ VCAM1, ·˘Í¿ÓÂÙ·È fiÙ·Ó ÙÔ ÂÓ‰Ôı‹ÏÈÔ ÂÓÂÚÁÔÔÈËı› ·fi ÙȘ ÚÔÊÏÂÁÌÔÓÒ‰ÂȘ ΢ÙÔΛÓ˜ IL1 Î·È TNFa, ÁÂÁÔÓfi˜ Ô˘ ‰ÈηÈÔÏÔÁ› ÙÔÓ ÌÂÁ¿ÏÔ ÚfiÏÔ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÛÙËÓ ·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋˜ ÎÚ›Û˘ (7,10,16,34).

OÈ Î˘ÙÔΛÓ˜ IL1 Î·È TNFa Ô˘ ·Ú¿ÁÔÓÙ·È ·fi Ù· Ï¢ÎÔ·ÙÙ·Ú· Î·È ‰ÈÂÁ›ÚÔ˘Ó ÙËÓ ¤ÎÊÚ·ÛË ÙˆÓ ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘, ‚Ú¤ıËÎ·Ó Û ·˘ÍË̤ӷ Â›‰· Û ·ÛıÂÓ›˜ Ì ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ, ·ÎfiÌË Î·È Û ʿÛÂȘ ‡ÊÂÛ˘ Ù˘ ÓfiÛÔ˘, ·Ô‰ÂÈÎÓ‡ÔÓÙ·˜ fiÙÈ

Pediatr Sept-Oct 05

17-10-05

16:51

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∞ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È· ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ

Ù· ·ÁÁ›· ÙˆÓ ·Û¯fiÓÙˆÓ ‚Ú›ÛÎÔÓÙ·È ÌfiÓÈÌ· Û ÚÔÊÏÂÁÌÔÓÒ‰Ë Î·Ù¿ÛÙ·ÛË (7,10). ªÂ ÙËÓ Â›ÛÔ‰Ô ÙÔ˘ ÌÈÎÚÔ‚È·ÎÔ‡ ·Ú¿ÁÔÓÙ· Î·È ÙËÓ ¤Ó·ÚÍË Ù˘ Ïԛ̈͢ Â¤Ú¯ÂÙ·È ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ Ì·ÎÚÔÊ¿ÁˆÓ ·fi Ù· ÌÈÎÚԂȷο ÚÔ˚fiÓÙ· Î·È ·ÂÏ¢ı¤ÚˆÛË Ù˘ πL1 Î·È ÙÔ˘ TNFa, ÂÓÒ Ù· ·ÙÙ·Ú· ÊÔÓ›˜ (killer cells) ·ÂÏ¢ıÂÚÒÓÔ˘Ó ÙËÓ ÈÓÙÂÚÊÂÚfiÓË Á (IFNÁ) fiÙ·Ó Û˘Ó·ÓÙ‹ÛÔ˘Ó ÂȉÈο ÌÈÎÚԂȷο ·ÓÙÈÁfiÓ·. ∞˘Ù¤˜ ÔÈ ÊÏÂÁÌÔÓÒ‰ÂȘ ΢ÙÔΛÓ˜ ‰ÈÂÁ›ÚÔ˘Ó, ÚÔ¿ÁÔ˘Ó Î·È Ú˘ıÌ›˙Ô˘Ó ÙËÓ ¤ÎÊÚ·ÛË ÙˆÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ̤۷ Û ϛÁ˜ ÒÚ˜ (4 ¤ˆ˜ 6) ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ Ïԛ̈͢ Î·È ÙËÓ ¤ÎÎÚÈÛ‹ ÙÔ˘˜ (7,32). ∏ ·ÏÏÂÚÁÈ΋ ÊÏÂÁÌÔÓ‹, Â›Û˘, ÚÔ¿ÁÂÈ ÙËÓ ÚÔÛÎfiÏÏËÛË ÙˆÓ ¢∂∫ ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ ÔÚÈÛÌ¤ÓˆÓ ·ÛıÂÓÒÓ Ì ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ. ∞˘Ùfi ÔÊ›ÏÂÙ·È ·ÊÂÓfi˜ ÛÙ· ·˘ÍË̤ӷ Â›‰· ÙˆÓ IgE ·ÓÙÈÛˆÌ¿ÙˆÓ Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜, ·ÊÂÙ¤ÚÔ˘ ÛÙËÓ ·˘ÍË̤ÓË ÂÓ‰ÔıËÏȷ΋ ¤ÎÊÚ·ÛË Î·È ÛÙ· ˘„ËÏ¿ Â›‰· ‰È·Ï˘ÙÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ÛÙËÓ ·ÙÔ›· (7). ™˘ÁÎÂÎÚÈ̤ӷ, Ë ÈÓÙÂÚÏ¢ΛÓË 4 (IL4) Ô˘ ·Ú¿ÁÂÙ·È Î·Ù¿ ÙËÓ ·ÏÏÂÚÁÈ΋ ÊÏÂÁÌÔÓ‹, Ú˘ıÌ›˙ÂÈ ÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ VCAM1 ÛÙ· ·ÁÁÂȷο ÂÓ‰ÔıËÏȷο ·ÙÙ·Ú· (31), ÂÓÒ Ù· ˘„ËÏ¿ Â›‰· IgE ÛÙËÓ ·ÙÔ›· Ô‰ËÁÔ‡Ó ÛÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Î·È ÛÙËÓ ·ÂÏ¢ı¤ÚˆÛË ıÚÔÌ‚ÔÛÔÓ‰›Ó˘ Ô˘ ÌÂÛÔÏ·‚› ÛÙËÓ ÚÔÛÎfiÏÏËÛË ÙˆÓ ¢∂∫ ÛÙ· ÂÓ‰ÔıËÏȷο ·ÙÙ·Ú· (7). Ÿˆ˜ ‹‰Ë ·Ó·Ê¤ÚıËÎÂ, ÔÈ ÛÔ‚·ÚfiÙÂÚ˜ ÂÈÏÔΤ˜ Ù˘ ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋˜ ÓfiÛÔ˘ ·ÊÔÚÔ‡Ó ÛÙ· ıÚÔÌ‚ÔÂÌ‚ÔÏÈο ÂÂÈÛfi‰È· ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (∫¡™) Î·È ÛÙÔ Ô͇ ıˆÚ·ÎÈÎfi Û‡Ó‰ÚÔÌÔ.

£ÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È· ÙÔ˘ ∫¡™ ∞ÁÁÂÈÔÁÚ·ÊÈΤ˜ Î·È ÓÂÎÚÔÙÔÌÈΤ˜ ÌÂϤÙ˜ Û ¿Û¯ÔÓÙ˜ ·fi ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ ·Ó·Ê¤ÚÔ˘Ó fiÙÈ ÛÙÔÓ ÂÁΤʷÏÔ Ù· ·ÁÁÂȷο ÂÂÈÛfi‰È· ·ÊÔÚÔ‡Ó ÛÙ· ÌÂÁ¿Ï· ·ÁÁ›· Î·È fi¯È ÛÙË ÌÈÎÚÔ΢ÎÏÔÊÔÚ›· (35). ™Ù· ÌÂÁ¿Ï· ·ÁÁ›· Â¤Ú¯ÂÙ·È ÛÙ¤ÓˆÛË ÙÔ˘ ·˘ÏÔ‡ ÏfiÁˆ ˘ÂÚÏ·Û›·˜ ÙÔ˘ ̤ÛÔ˘ ¯ÈÙÒÓ·, ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ Î·È ÙˆÓ Ï›ˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ. ∏ ‚Ï¿‚Ë ÙÔ˘ ̤ÛÔ˘ ¯ÈÙÒÓ· ¤¯ÂÈ ¿ÌÂÛË Û¯¤ÛË Ì ٷ ÚÔÛÎÔÏÏÒÌÂÓ· ‰ÚÂ·ÓÔ·ÙÙ·Ú·, Ô˘ ·ÏÏÔÈÒÓÔ˘Ó ÙÔ ÂÓ‰Ôı‹ÏÈÔ Î·È ‰Â˘ÙÂÚÔÁÂÓÒ˜ ÚÔηÏÔ‡Ó ÂÓ·fiıÂÛË ·ÈÌÔÂÙ·Ï›ˆÓ. ∆· ·ÈÌÔÂÙ¿ÏÈ· ·ÂÏ¢ıÂÚÒÓÔ˘Ó ·ÈÌÔÂÙ·ÏÈ·ÎÔ‡˜ ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ¢ı‡ÓÔÓÙ·È ÁÈ· ÙËÓ ˘ÂÚÏ·Û›· ÙÔ˘ ̤ÛÔ˘ ¯ÈÙÒÓ· Ô˘ Ô‰ËÁ› ÛÙËÓ ·ÁÁÂȷ΋ ÛÙ¤ÓˆÛË, ÙË ÛÙÚÔ‚ÈÏÒ‰Ë ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜, ÛÙËÓ ÂÈϤÔÓ

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‚Ï¿‚Ë ÙÔ˘ ̤ÛÔ˘ ¯ÈÙÒÓ· Î·È ÛÙËÓ Âȉ›ӈÛË Ù˘ ·fiÊڷ͢ (35-37).

O͇ ıˆÚ·ÎÈÎfi Û‡Ó‰ÚÔÌÔ ∏ ÂÌÊ¿ÓÈÛË ÙÔ˘ ÔͤԘ ıˆÚ·ÎÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ÔÊ›ÏÂÙ·È: ·) ÛÙËÓ È‰È·›ÙÂÚË Â˘·ÈÛıËÛ›· Ù˘ Ó¢ÌÔÓÈ΋˜ ÌÈÎÚÔ΢ÎÏÔÊÔÚ›·˜ ÛÙËÓ ˘ÔÍ›· Î·È ‚) ÛÙËÓ ·Ú·ÁˆÁ‹ ÚÔÊÏÂÁÌÔÓˆ‰ÒÓ Î˘ÙÔÎÈÓÒÓ Î·È ÙËÓ ·˘ÍË̤ÓË ¤ÎÊÚ·ÛË ÙˆÓ ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ÛÙ· Ó¢ÌÔÓÈο ÂÓ‰ÔıËÏȷο ·ÙÙ·Ú· ˆ˜ ·¿ÓÙËÛË ÛÙËÓ ˘ÔÍ›· (7,9,38-42). ∂ÎÏ˘ÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÁÈ· ÙËÓ ¤Ó·ÚÍË ÙÔ˘ ÔͤԘ ıˆÚ·ÎÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ Â›Ó·È Ë Ïԛ̈ÍË Ô˘ ·ÎÔÏÔ˘ıÂ›Ù·È ·fi Ï¢ÎÔ΢ÙÙ¿ÚˆÛË, Ë ·Ú·ÁˆÁ‹ ΢ÙÔÎÈÓÒÓ, Ë ÂÓ‰ÔıËÏȷ΋ ÚÔÛÎfiÏÏËÛË Î·È Ë ıÚfiÌ‚ˆÛË (9,42,43). ∞ÎÔÏÔ˘ı› Ó¢ÌÔÓÈ΋ ·ÁÁÂÈÔÛ˘ÛÙÔÏ‹, Ì›ˆÛË Ù˘ ·ÈÌ·ÙÈ΋˜ ÚÔ‹˜, ·Á›‰Â˘ÛË Î·È ·ÈÌfiÏ˘ÛË ‰ÚÂ·ÓÔ΢ÙÙ¿ÚˆÓ, ·Ú·ÁˆÁ‹ ÂχıÂÚˆÓ ÚÈ˙ÒÓ Î·È Âȉ›ӈÛË Ù˘ ıÚÔÌ‚ˆÙÈ΋˜ ÎÚ›Û˘ (9). ∏ ÔÚ›· ·˘Ù‹ ÂÈ‚·Ú‡ÓÂÙ·È ÏfiÁˆ ¤ÏÏÂȄ˘ ΢ÙÙ·ÚÔÚÔÛٷ٢ÙÈÎÒÓ ÌÂÛÔÏ·‚ËÙÒÓ, fiˆ˜ ÙÔ ¡√, ÙÔ˘ ÔÔ›Ô˘ Ë Û‡ÓıÂÛË ·fi ÙÔ ÂÓ‰Ôı‹ÏÈÔ ·Ó·ÛÙ¤ÏÏÂÙ·È ÏfiÁˆ Ù˘ ˘ÔÍ›·˜ (4448). ∆·˘Ùfi¯ÚÔÓ·, ˘¿Ú¯ÂÈ ·˘ÍË̤ÓË ‚ÚÔÁ¯È΋ ·ÓÙȉڷÛÙÈÎfiÙËÙ·, Ô˘ ıˆÚÂ›Ù·È fiÙÈ Â›Ó·È ‰Â˘ÙÂÚÔÁÂÓ‹˜ ÏfiÁˆ Ù˘ ˘ÔÎÏÈÓÈ΋˜ Ó¢ÌÔÓÈ΋˜ ‚Ï¿‚˘ ·fi Ù· ‰ÚÂ·ÓÔ·ÙÙ·Ú· (9). ¶ÚfiÛÊ·Ù· ˘ÔÛÙËÚ›¯ıËΠfiÙÈ, Û ¤Ó· ÈηÓÔÔÈËÙÈÎfi ÔÛÔÛÙfi, ÙÔ Ô͇ ıˆÚ·ÎÈÎfi Û‡Ó‰ÚÔÌÔ ÔÊ›ÏÂÙ·È Û Ó¢ÌÔÓÈÎfi ¤ÌÊÚ·ÎÙÔ ‹ ÂÌ‚ÔÏ‹ ·fi ÛÊ·ÈÚ›‰È· Ï›Ô˘˜ ‹ ÈÛÙÔÙÂÌ·¯›‰È· ·fi Ì˘ÂÏÈο ¤ÌÊÚ·ÎÙ· (40,41). ∂ÈϤÔÓ, ·Ó·Ê¤ÚÂÙ·È fiÙÈ ÔÈ ·ÛıÂÓ›˜ Ì ·ÁÁÂÈÔıÚÔÌ‚ˆÙÈ΋ ÎÚ›ÛË ‚Ú›ÛÎÔÓÙ·È Û ÚÔÛÙ¿‰ÈÔ ÏÈÒ‰Ô˘˜ ÂÌ‚ÔÏ‹˜, ‰ÈfiÙÈ ˘¿Ú¯ÂÈ ·˘ÍË̤ÓË ¤ÎÎÚÈÛË ÂÓfi˜ ÊÏÂÁÌÔÓÒ‰Ô˘˜ ÌÂÛÔÏ·‚ËÙ‹ Ù˘ ʈÛÊÔÏÈ¿Û˘ A2 (SPLA2) Ô˘ ·ÂÏ¢ıÂÚÒÓÂÈ ÂχıÂÚ· ÏÈ·Ú¿ Ôͤ·, ˘‡ı˘Ó· ÁÈ· ÙËÓ ÔÍ›· ‚Ï¿‚Ë ÙÔ˘ Ó‡ÌÔÓ· ÚÈÓ ÂÌÊ·ÓÈÛÙ› ÙÔ Ô͇ ıˆÚ·ÎÈÎfi Û‡Ó‰ÚÔÌÔ. ∏ ·Ó‡ÚÂÛË ·˘ÍËÌ¤ÓˆÓ ÂÈ¤‰ˆÓ SPLA2 Û ¿Û¯ÔÓÙ˜ ·ÔÙÂÏ› ¤Ó‰ÂÈÍË ÌÂÙ¿ÁÁÈÛ˘ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ ‹ ·Ê·ÈÌ·ÍÔÌÂÙ¿ÁÁÈÛ˘ ÚÔÎÂÈ̤ÓÔ˘ Ó· ÚÔÏËÊı› Ô͇ ıˆÚ·ÎÈÎfi Û‡Ó‰ÚÔÌÔ (49-51).

™˘ÌÂÚ¿ÛÌ·Ù· ∏ ÌÂϤÙË Ù˘ ·ıÔÊ˘ÛÈÔÏÔÁ›·˜ ÙˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ ÛÙԯ‡ÂÈ ÛÙËÓ ÚfiÏË„Ë Î·È Ì›ˆÛË Ù˘ ¶·È‰È·ÙÚÈ΋ 2005;68:329-335

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∂. ∫·Ú·Ù˙¿ - •ÈÊÈÏ›‰Ô˘ Î·È Û˘Ó.

Û˘¯ÓfiÙËÙ·˜ Î·È Ù˘ ‚·Ú‡ÙËÙ·˜ ÙˆÓ ÂÒ‰˘ÓˆÓ ÎÚ›ÛˆÓ, Ì ÛÎÔfi ÙË ‚ÂÏÙ›ˆÛË Ù˘ ÔÈfiÙËÙ·˜ ˙ˆ‹˜ Î·È ÙËÓ ·Ú¿Ù·ÛË Ù˘ ÂÈ‚›ˆÛ˘ ÙˆÓ ·Û¯fiÓÙˆÓ (3,23,42,52,53). π‰È·›ÙÂÚË ÛËÌ·Û›· ¤¯ÂÈ Ë ¤ÁηÈÚË ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ÔͤԘ ıˆÚ·ÎÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ Î·È ÙˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ÙÔ˘ ∫¡™, Ù· ÔÔ›· Â›Ó·È ‰˘Ó·ÙfiÓ Ó· Ô‰ËÁ‹ÛÔ˘Ó Û ÌfiÓÈÌË ·Ó·ËÚ›· ‹ ·ÎfiÌË Î·È ÛÙÔÓ ı¿Ó·ÙÔ.

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REVIEW ARTICLE

Pathophysiology of vaso-occlusive crises in sickle cell disease E. Karatza - Xifilidou1, M. Athanasiou - Metaxa2 1 3rd Paediatric Clinic, Aristotle University, Ippokration General Hospital, Thessaloniki 2 1st Paediatric Clinic, Aristotle University, Ippokration General Hospital, Thessaloniki Correspondence: ∂. Karatza-Xifilidou Ippokration General Hospital 49 Konstantinoupoleos Str. 546 42, Thessaloniki Date of submission: 03-09-2004 Date of approval: 01-04-2005

Abstract Sickle cell disease is caused by a mutation which is characterized by the replacement of glutaminic acid by valine in the 6th position of the ‚-chain of haemoglobin (‚6glu val) and the production of haemoglobin S (·2‚2S). The exclusive characteristic of haemoglobin S, when deoxygenated, is polymerization of the chains and, as a result, production of dehydrated rigid sickle cells. Until the beginning of the past decade, the rigid sickle cells were thought to play the exclusive role in the pathogenesis of the painful thrombotic crises of the disease. Newer studies have proved that thrombotic episodes are the result of the activation of a complex multifactorial mechanism. At the onset of these episodes, stress reticulocytes attach to the endothelium of the vessels in the microcirculatory system and the rigid sickle cells become trapped in an attempt at their removal from circulation. The establishment of the initial thrombus is controlled by: a) factors from the red blood cell membrane, which is damaged by hypoxia, and parallel activation of the plasma coagulation factors, b) factors from the endothelium of the vessels (antagonism between vasopressor and vasodilator factors), c) surface adhesion molecules of the endothelial cells and the stress reticulocytes, which contribute to the initial attachment of reticulocytes to the endothelium, and d) activation and attachment of other blood cells (platelets and white cells). Study of the interaction of all these factors has helped in the understanding of the pathogenesis of vaso-occlusive episodes, resulting in better monitoring, treatment approaches, quality of life and survival of patients suffering from sickle cell disease. →

Key words Sickle cell disease, stress reticulocytes, endothelium, vaso-occlusive crises.

Paediatriki 2005;68:336

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∞¡∞™∫O¶∏™∏

337

∞ÍÈÔÏfiÁËÛË Ù˘ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚfiÎÏËÛ˘ ÁÈ· ÙË ‰È¿ÁÓˆÛË Ê·Ú̷΢ÙÈ΋˜ ·ÏÏÂÚÁ›·˜ A. ™ÔÊÈ·ÓÔ‡ - ∫·ÙÛÔ‡ÏË1, G. Du Toit2, G. Lack2

¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: OÈ Ê·Ú̷΢ÙÈΤ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ ÛÙÔÓ ·È‰È·ÙÚÈÎfi ÏËı˘ÛÌfi Â›Ó·È ÛÔ‚·Ú¤˜ Î·È ‰˘ÓËÙÈÎÒ˜ ·ÂÈÏËÙÈΤ˜ ÁÈ· ÙË ˙ˆ‹ ÂÚÈÙÒÛÂȘ, Ô˘ Û˘¯Ó¿ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È Î·È ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È ÏËÌÌÂÏÒ˜. ∞˘Ùfi ÔÊ›ÏÂÙ·È ÛÙËÓ ¤ÏÏÂÈ„Ë ¤ÁÎ˘ÚˆÓ ‰È·ı¤ÛÈÌˆÓ ‰È·ÁÓˆÛÙÈÎÒÓ Ì¤ÛˆÓ, ηıÒ˜ Î·È ÚˆÙÔÎfiÏÏˆÓ ·ÓÙÈÌÂÙÒÈÛ˘. ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ: ¶ÚfiÎÂÈÙ·È ÁÈ· ·Ó·‰ÚÔÌÈ΋ ÌÂϤÙË 22 ·ÛıÂÓÒÓ Ô˘ ˘Ô‚Ï‹ıËÎ·Ó Û ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚfiÎÏËÛË, Ì ÛÎÔfi ÙË ‰È¿ÁÓˆÛË ·Ó·ÊÂÚfiÌÂÓ˘ Ê·Ú̷΢ÙÈ΋˜ ·ÏÏÂÚÁ›·˜. OÈ ÚÔÎÏ‹ÛÂȘ ¤Ï·‚·Ó ¯ÒÚ· ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô St. Mary’s ÙÔ˘ §ÔÓ‰›ÓÔ˘. OÈ ÚÔÎÏ‹ÛÂȘ ‹Ù·Ó ΢ڛˆ˜ ·fi ÙÔ ÛÙfiÌ·, Ì ÂÍ·›ÚÂÛË Ù· Ê¿Ú̷η ÏȉÔη˝ÓË Î·È ÚÔη˝ÓË, fiÔ˘ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó ‰ÂÚÌ·ÙÈο ÙÂÛÙ. OÈ ÂÚÈÛÛfiÙÂÚ˜ ‰ÔÎÈ̷ۛ˜ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó ÛÙËÓ ÂÓÈÎÈÏ›ÓË (40%). ¶ÔÛÔÛÙfi 75% ÙˆÓ ‰ÔÎÈÌ·ÛÈÒÓ ‹Ù·Ó Û ¤Ó· Ê¿ÚÌ·ÎÔ, ÂÓÒ 25% ‹Ù·Ó ÛÂ Û˘Ó‰˘·ÛÌfi Ê·Ú̿ΈÓ.

1 ∂ȉÈ΢fiÌÂÓË ¶·È‰È·ÙÚÈ΋˜, St. Mary’s Hospital, London, UK 2 ∆Ì‹Ì· ¶·È‰È·ÙÚÈ΋˜ ∞ÏÏÂÚÁÈÔÏÔÁ›·˜ Î·È ∞ÓÔÛÔÏÔÁ›·˜, St. Mary’s Hospital, London, UK AÏÏËÏÔÁÚ·Ê›·: A. ™ÔÊÈ·ÓÔ‡-∫·ÙÛÔ‡ÏË 42 Abbots Park, St. Albans Herts, UK AL1 1TW E-mail: katsofkat@hotmail.com ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 17-09-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 27-04-2005

∞ÔÙÂϤÛÌ·Ù·: ∞fi ÙÔ˘˜ 6 ·ÛıÂÓ›˜ Ô˘ ›¯·Ó οÔÈ· ·ÓÙ›‰Ú·ÛË ÛÙËÓ ÚfiÎÏËÛË, ÛÙÔ˘˜ 5 ‹Ù·Ó ¿ÌÂÛË Î·È ÛÙÔÓ ¤Ó·Ó ηı˘ÛÙÂÚË̤ÓË. OÈ ÁÔÓ›˜ ÙˆÓ ·È‰ÈÒÓ Ì ·ÚÓËÙÈ΋ ÚfiÎÏËÛË ¤‰ÂÈ¯Ó·Ó Û›ÁÔ˘ÚÔÈ ÌÂÙ¿ ÙÔ ·ÔÙ¤ÏÂÛÌ·. ™Â ·˘ÙÔ‡˜ Ô˘ Ù· ·È‰È¿ ÙÔ˘˜ ›¯·Ó ıÂÙÈ΋ ÚfiÎÏËÛË, ·˘Í‹ıËÎÂ Ë ·˘ÙÔÂÔ›ıËÛË ÁÈ· ÙË ¯Ú‹ÛË ÂÓ·ÏÏ·ÎÙÈÎÒÓ Û΢·ÛÌ¿ÙˆÓ. ™˘ÌÂÚ¿ÛÌ·Ù·: OÈ Ê·Ú̷΢ÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ Ú¤ÂÈ Ó· Ú·ÁÌ·ÙÔÔÈÔ‡ÓÙ·È Û ÂÍÂȉÈÎÂ˘Ì¤Ó· ΤÓÙÚ·, ȉȷ›ÙÂÚ· Â¿Ó ÙÔ ÈÛÙÔÚÈÎfi Â›Ó·È ·ÌÊ›‚ÔÏÔ. ¶ÔÏϤ˜ ·fi ÙȘ ˘¿Ú¯Ô˘Û˜ ‰È·ÁÓˆÛÙÈΤ˜ ‰ÔÎÈ̷ۛ˜ ¯ÚÂÈ¿˙ÔÓÙ·È ÂÈ·ڈÛË Ù˘ ·Í›·˜ ÙÔ˘˜, ÚÈÓ ÙË ÁÂÓ›ÎÂ˘Û‹ ÙÔ˘˜. ∂˘Ú‡ÙÂÚ˜ ÌÂϤÙ˜ Â›Ó·È ··Ú·›ÙËÙ˜, ÒÛÙ ӷ ‰ڷȈıÔ‡Ó Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ÁÈ· ÙËÓ ÚfiÁÓˆÛË ·˘ÙÒÓ ÙˆÓ ÂÚÈÙÒÛˆÓ.

§¤ÍÂȘ ÎÏÂȉȿ º·Ú̷΢ÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ, Ê·Ú̷΢ÙÈ΋ ·ÏÏÂÚÁ›·, ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ.

∂ÈÛ·ÁˆÁ‹ OÈ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ (∞∂) ·fi Ê¿Ú̷η Â›Ó·È ¤Ó· ÛËÌ·ÓÙÈÎfi Úfi‚ÏËÌ· ‰ËÌfiÛÈ·˜ ˘Á›·˜, ȉȷ›ÙÂÚ· ÛÙÔÓ ·È‰È·ÙÚÈÎfi ÏËı˘ÛÌfi. ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· (3,6), Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ∞∂ Û ·È‰È¿ Ô˘ ÂÍÂÙ¿ÛÙËÎ·Ó Û Â͈ÙÂÚÈο È·ÙÚ›· ‹Ù·Ó 1,46%, ÂÓÒ Û ÓÔÛËÏ¢fiÌÂÓ· ·È‰È¿ ÌÂٷ͇ 6,7% Î·È 9,53%, ÂÓÒ ÔÈ ∞∂ Ô˘ ·Ô‚·›ÓÔ˘Ó ÌÔÈÚ·›Â˜ ·Ó¤Ú¯ÔÓÙ·È Û 0,32%. ∆Ô 39% ÙˆÓ ∞∂ Ô˘ ··ÈÙÔ‡Ó ÓÔÛËÏ›·, Â›Ó·È ÛÔ‚·Ú¤˜ ‹ Î·È ·ÂÈÏËÙÈΤ˜ ÁÈ· ÙË ˙ˆ‹ (6). ™Â Ì›· ·Ï·ÈfiÙÂÚË ÌÂϤÙË (3), ÔÈ Û˘¯ÓfiÙÂÚ˜ ·ÓÙȉڿÛÂȘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙÔ˘˜ ·ÎfiÏÔ˘ıÔ˘˜ ·Ú¿ÁÔÓÙ˜: ·ÌÔ͢ÎÈÏ›ÓË (Û ÔÛÔÛÙfi 5,1%), ÙÚÈÌÂıÔÚ›ÌË-ÛÔ˘ÏÊÔÌÂıÔÍ·˙fiÏË

(3,4%), ·ÌÈÎÈÏ›ÓË (3,3%), ·Ú¿ÁˆÁ· ·›Ì·ÙÔ˜ (2,2%), ÎÂÊ·ÏÔÛÔÚ›Ó˜ (2,1%), ËÌÈÛ˘ÓıÂÙÈΤ˜ ÂÓÈÎÈϛӘ (2,1%), ÂÚ˘ıÚÔÌ˘Î›ÓË (2,0%) Î·È ÂÓÈÎÈÏ›ÓË G (1,8%) (1). ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ Â›Ó·È Ó· ·Ú·ı¤ÛÂÈ Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ Ê·Ú̷΢ÙÈÎÒÓ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚÔÎÏ‹ÛÂˆÓ ÛÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ÙÌ‹Ì·, ÒÛÙ ӷ ηٷ‰ÂȯÙÔ‡Ó ÔÈ Ê·Ú̷΢ÙÈΤ˜ Ô˘Û›Â˜ Ô˘ ÚÔηÏÔ‡Ó Û ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ Î·È Ë ÎÏÈÓÈ΋ ·ÓÙ·fiÎÚÈÛË ÙˆÓ ·È‰ÈÒÓ ÛÙȘ ‰È·ÁÓˆÛÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ. ∂›Û˘, Ë ÂÚÁ·Û›· ·ÔÛÎÔ› ÛÙË Û‡ÁÎÚÈÛË ·˘ÙÒÓ ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Ì ٷ ϤÔÓ ÚfiÛÊ·Ù· ‰Â‰Ô̤ӷ ÛÙË ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Ê·Ú̷΢ÙÈÎÒÓ ·ÏÏÂÚÁÈÒÓ Ô˘ ÂÓÙÔ›˙ÔÓÙ·È Î˘Ú›ˆ˜ ÛÙÔÓ ÙÔ̤· Ù˘ ·ÓÔÛԉȿÁÓˆÛ˘. ¶·È‰È·ÙÚÈ΋ 2005;68:337-340

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·338

338

A. ™ÔÊÈ·ÓÔ‡ - ∫·ÙÛÔ‡ÏË Î·È Û˘Ó.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ¶·ÚÔ˘ÛÈ¿˙ÂÙ·È Ì›· ·Ó·ÛÎfiËÛË ÙˆÓ Ê·Ú̷΢ÙÈÎÒÓ ÚÔÎÏ‹ÛÂˆÓ ÛÙÔ ∆Ì‹Ì· ¶·È‰È·ÙÚÈ΋˜ ∞ÏÏÂÚÁ›·˜ Î·È ∞ÓÔÛÔÏÔÁ›·˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ St. Mary’s, ÙÔ ÔÔ›Ô Â›Ó·È ÙÚÈÙÔ‚¿ıÌÈÔ Î¤ÓÙÚÔ ·Ó·ÊÔÚ¿˜ ÁÈ· ÙËÓ ·È‰È΋ ·ÏÏÂÚÁ›· ÛÙÔ ¢˘ÙÈÎfi §ÔÓ‰›ÓÔ Î·È ÙȘ ÁÂÈÙÔÓÈΤ˜ ÎÔÌËÙ›˜. ∂›ÎÔÛÈ ‰‡Ô ·È‰È¿ ·Ú·¤ÌÊıËÎ·Ó Ì ÈÛÙÔÚÈÎfi Ê·Ú̷΢ÙÈ΋˜ ·ÏÏÂÚÁ›·˜ ÁÈ· ‰ÈÂÚ‡ÓËÛË, ηٿ ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ·fi ÙÔÓ π·ÓÔ˘¿ÚÈÔ 2001 ¤ˆ˜ ÙÔÓ ª¿ÈÔ 2003. √ ÏËı˘ÛÌfi˜ ·˘Ùfi˜ ·ÔÙÂÏÔ‡ÓÙ·Ó ·fi ·È‰È¿ Ô˘ ÚÔÛ‹Ïı·Ó ›Ù ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô Ì ·ÏÏÂÚÁÈ΋ ·ÓÙ›‰Ú·ÛË ‹ ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ÌÂÙ¿ ·fi ·Ú·ÔÌ‹ ÙÔ˘ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ È·ÙÚÔ‡. ŸÏ· Ù· ·È‰È¿ ˘Ô‚Ï‹ıËÎ·Ó Û ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘ ÛÙÔ ‡ÔÙÔ Ê¿ÚÌ·ÎÔ Î·È/‹ ÛÙÔ ·ÛʷϤ˜ ˘ÔηٿÛÙ·Ùfi ÙÔ˘ (.¯. ÂÚ˘ıÚÔÌ˘Î›ÓË Û ÂÚ›ÙˆÛË ·ÏÏÂÚÁ›·˜ Û ÂÓÈÎÈÏ›ÓË ‹ ÂÓÈÎÈÏ›Ó˘ Û ÂÚ›ÙˆÛË ·ÏÏÂÚÁ›·˜ ÙÚÈÌÂıÔÚ›Ì˘-ÛÔ˘ÏÊÔÌÂıÔÍ·˙fiÏ˘). ŸÏ˜ ÔÈ ‰ÔÎÈ̷ۛ˜ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó Û ı¿Ï·ÌÔ ËÌÂÚ‹ÛÈ·˜ ÓÔÛËÏ›·˜ ˘fi ÙËÓ Â›‚ÏÂ„Ë ÂÍÂȉÈÎÂ˘Ì¤Ó˘ ÓÔÛÔÎfiÌ·˜, ÂÓfi˜ ÂȉÈ΢fiÌÂÓÔ˘ È·ÙÚÔ‡ Î·È ÂÓfi˜ ÂȉÈÎÂ˘Ì¤ÓÔ˘ ·È‰Ô·ÏÏÂÚÁÈÔÏfiÁÔ˘.

OÈ ÂÚÈÛÛfiÙÂÚ˜ ‰ÔÎÈ̷ۛ˜ ÍÂÎÈÓÔ‡Û·Ó Ì ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ¯ÔÚ‹ÁËÛË Û΢·ÛÌ¿ÙˆÓ Û ·˘Í·ÓfiÌÂÓ˜ ‰fiÛÂȘ: Ë ÚÒÙË ¯ÔÚËÁÔ‡ÌÂÓË ‰fiÛË ‹Ù·Ó ÙÔ 10% Ù˘ ÂÓ‰ÂÈÎÓ˘fiÌÂÓ˘ - ‚¿ÛÂÈ ÙÔ˘ ‚¿ÚÔ˘˜ Î·È Ù˘ ËÏÈΛ·˜ ‰fiÛ˘ Î·È ÛÙË Û˘Ó¤¯ÂÈ· ¯ÔÚËÁÔ‡ÓÙ·Ó ·Ó¿ ‰È·ÛÙ‹Ì·Ù· Ì›·˜ ÒÚ·˜ ÙÔ 25%, ÙÔ 50% Î·È ÙÂÏÈο, ÙÔ 100% Ù˘ ‰fiÛ˘. ∆Ô ·È‰› ¤·ÈÚÓ ÂÍÈÙ‹ÚÈÔ Ì ԉËÁ›Â˜ Ó· Ï¿‚ÂÈ ÙËÓ Î·ÓÔÓÈ΋ ‰fiÛË ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ÁÈ· ¤ÓÙ Ë̤Ú˜, ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔÎÏÂÈÛÙ› Ë ÂÚ›ÙˆÛË Î·ı˘ÛÙÂÚË̤Ó˘ ·ÓÙ›‰Ú·Û˘. ∞ÎÔÏÔ˘ıÔ‡Û Â›ÛÎÂ„Ë ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ÌÂÙ¿ ·fi 3-4 ‚‰ÔÌ¿‰Â˜. ™ÙËÓ ÂÚ›ÙˆÛË ÙˆÓ ÙÔÈÎÒÓ ·Ó·ÈÛıËÙÈÎÒÓ, Ú·ÁÌ·ÙÔÔÈÔ‡ÓÙ·Ó ‰ÂÚÌ·ÙÈΤ˜ ‰ÔÎÈ̷ۛ˜, ¿ÏÈ Ì ÛÙ·‰È·Î‹ ÂÊ·ÚÌÔÁ‹ ·˘Í·ÓfiÌÂÓˆÓ ‰fiÛˆÓ. OÈ ÚÔÎÏ‹ÛÂȘ Á›ÓÔÓÙ·Ó ˘fi ÙËÓ ÚÔ¸fiıÂÛË fiÙÈ Ù· ·È‰È¿ ‹Ù·Ó ˘ÁÈ‹ ·Ì¤Ûˆ˜ ÚÈÓ ÙËÓ ÂͤٷÛË Î·È ‰ÂÓ Â›¯·Ó Ï¿‚ÂÈ ·ÓÙÈÈÛÙ·ÌÈÓÈο Û΢¿ÛÌ·Ù· ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 48 ÒÚ˜. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ‰ÔÎÈÌ·Û›·˜ ÂÙ›ıÂÙÔ ÂÚÈÊÂÚÈ΋ ÊÏ‚È΋ ÁÚ·ÌÌ‹, ÂÓÒ Ù· ··Ú·›ÙËÙ· Ê¿Ú̷η ÁÈ· ·Ó¿Ù·ÍË Ù˘¯fiÓ ·ÏÏÂÚÁÈ΋˜ ·ÓÙ›‰Ú·Û˘ ‹Ù·Ó Û ¿ÌÂÛË ÚfiÛ‚·ÛË. ªÂÙ¿ ·fi ÙËÏÂʈÓÈ΋ ÂÈÎÔÈÓˆÓ›· Ì ÙÔ˘˜ ÁÔÓ›˜ ·˘ÙÒÓ ÙˆÓ ·È‰ÈÒÓ, ηٿ ÙËÓ ÔÔ›· ·¿ÓÙËÛ·Ó Û ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ, ÂÍÂÙ¿ÛÙËÎÂ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ·˘ÙÒÓ ÙˆÓ ‰ÔÎÈÌ·ÛÈÒÓ. ∂ÚˆÙ‹ıËÎ·Ó ÁÈ· ÙËÓ ‡·ÚÍË ‹ fi¯È ·ÓÙ›‰Ú·Û˘ Û ÌÂÙ¤ÂÈÙ· Â·Ê‹ Ì ʿÚÌ·ÎÔ ÛÙÔ ÔÔ›Ô Ë ÚfiÎÏËÛË ‹Ù·Ó ·ÚÓËÙÈ΋. ™Â ·ÛıÂÓ›˜ Ô˘ ·ԉ›¯ÙËΠʷÚ̷΢ÙÈ΋ ·ÏÏÂÚÁ›·, ‰ÈÂÚ¢ӋıËÎÂ Ù˘¯fiÓ ·ÓÙ›‰Ú·ÛË Û Û··ÛÌ· Ô˘ Û˘ÛÙ‹ıËΠ·ÓÙ› ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ Ô˘ ÚÔηÏÔ‡Û ·ÏÏÂÚÁ›·. ∂ÈϤÔÓ, ÂÍÂÙ¿ÛÙËÎ·Ó Ù· Èı·Ó¿ „˘¯ÔÏÔÁÈο ÔʤÏË ÁÈ· ÙËÓ ÔÈÎÔÁ¤ÓÂÈ·, ¤ÙÛÈ fiˆ˜ ηٷÁÚ¿ÊËÎ·Ó ·fi ÙËÓ ÏÂ˘Ú¿ ÙˆÓ ÁÔÓ¤ˆÓ.

∞ÔÙÂϤÛÌ·Ù· ∏ ÛÂÈÚ¿ ·ÔÙÂÏÔ‡ÓÙ·Ó ·fi 22 ·È‰È¿ (11 ·ÁfiÚÈ· Î·È 11 ÎÔÚ›ÙÛÈ·), ËÏÈΛ·˜ 3-16 ÂÙÒÓ (̤ÛË ÙÈÌ‹: 10 ¤ÙË, ‰È¿ÌÂÛÔ˜: 11 ¤ÙË). ∂ÓÓ¤· ¶·È‰È·ÙÚÈ΋ 2005;68:337-340

·fi ·˘Ù¿ ›¯·Ó οÔÈÔ ÈÛÙÔÚÈÎfi ·ÙÔ›·˜ (40,9%) Î·È 5 ›¯·Ó οÔÈÔ ÈÛÙÔÚÈÎfi ·ÏÏÂÚÁ›·˜, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Ù˘ ÙÚÔÊÈ΋˜ (22,7%). ∆· ·Ó·ÊÂÚfiÌÂÓ· Û˘ÌÙÒÌ·Ù· Î·È Ù· ÂÓÔ¯ÔÔÈÔ‡ÌÂÓ· Ê¿Ú̷η Ê·›ÓÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1 Î·È ÛÙËÓ ∂ÈÎfiÓ· 1, ·ÓÙ›ÛÙÔȯ·.

OÈ ÚÔÎÏ‹ÛÂȘ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó ÛÙ· ·ÎfiÏÔ˘ı· Ê¿Ú̷η: ÂÓÈÎÈÏ›ÓË V 9, ·ÌÔ͢ÎÈÏ›ÓË 5, ÂÚ˘ıÚÔÌ˘Î›ÓË 4, ·ÛÈÚ›ÓË 2, ÏȉÔη˝ÓË 1, ÚÔη˝ÓË 1, ÙÚÈÌÂıÔÚ›ÌË-ÛÔ˘ÏÊÔÌÂıÔÍ·˙fiÏË 1 Î·È ÎÏ·ÚÈıÚÔÌ˘Î›ÓË 1. ™˘Ó‰˘·˙fiÌÂÓ˜ ÚÔÎÏ‹ÛÂȘ (ÚÔη˝ÓË Î·È ÏȉÔη˝ÓË, ·ÌÔ͢ÎÈÏ›ÓË Î·È ÙÚÈÌÂıÔÚ›ÌË, ÂÓÈÎÈÏ›ÓË Î·È ÎÏ·ÚÈıÚÔÌ˘Î›ÓË ‹ ÂÚ˘ıÚÔÌ˘Î›ÓË) Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó Û 5 ·ÛıÂÓ›˜, Ì ‰‡Ô ·Ó·ÊÂÚfiÌÂÓ· Û˘ÌÙÒÌ·Ù· Ô˘ ηıÈÛÙÔ‡Û·Ó ÙËÓ ˘Ô„›· ·ÏÏÂÚÁÈ΋˜ ·ÓÙ›‰Ú·Û˘ ÈÛ¯˘ÚfiÙÂÚË Î·È ÛÙÔ˘˜ ÔÔ›Ô˘˜, ÙÂÏÈο, ·Ó·Ù‡¯ıËΠ·ÓÙ›‰Ú·ÛË. ™Â ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË, ÂÍÂÙ¿ÛÙËÎ·Ó ÙfiÛÔ ˆ˜ ÚÔ˜ ÙÔ ‡ÔÙÔ Û··ÛÌ· fiÛÔ Î·È Û ¤Ó· ¿ÏÏÔ Ô˘ ı· ÌÔÚÔ‡Û ӷ ¯ÚËÛÈÌÔÔÈËı› ÂÓ·ÏÏ·ÎÙÈο.

OÈ ÚÔÎÏ‹ÛÂȘ ‹Ù·Ó ıÂÙÈΤ˜ Û 6 ·È‰È¿ (28%), Û 5 ÂÎ ÙˆÓ ÔÔ›ˆÓ Ë ·ÓÙ›‰Ú·ÛË ‹Ù·Ó ¿ÌÂÛË Î·È Û 1 ÌÂÙ¿ ·fi ÙÔ ÂÓı‹ÌÂÚÔ Û¯‹Ì· ¯ÔÚ‹ÁËÛ˘. ™Â ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜ ˘‹Ú¯Â ۷ʤ˜ ÈÛÙÔÚÈÎfi ·ÏÏÂÚÁÈ΋˜ ·ÓÙ›‰Ú·Û˘, ›Ù ‹È·˜ (ÎÓȉˆÙÈÎfi ÂÍ¿ÓıËÌ·) ‹ ̤ÙÚÈ·˜ (·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·). ¶¤ÓÙ ·fi Ù· ·È‰È¿ ·˘Ù¿ ›¯·Ó ÈÛÙÔÚÈÎfi ·ÙÔ›·˜ Î·È ‹Ù·Ó fiÏ· ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 10 ÂÙÒÓ. ™Â ¤Ó· ·fi ·˘Ù¿ Ú·ÁÌ·ÙÔÔÈ‹ıËΠ‰ÂÚÌ·ÙÈ΋ ‰ÔÎÈÌ·Û›· ÛÙË ÏÈÁÓÔη˝ÓË Î·È ÚÔη˝ÓË, ÏfiÁˆ ÈÛÙÔÚÈÎÔ‡ ·ÓÙ›‰Ú·Û˘ ÌÂÙ¿ ·fi Â·Ê‹ Ì Á¿ÓÙÈ· Ì›·˜ ¯Ú‹Û˘ (latex). ™Â ÙÚÂȘ ÂÚÈÙÒÛÂȘ, Ë ¤Ó‰ÂÈÍË Ù˘ ·Ú¯È΋˜ ¯ÚËÛÈÌÔÔ›ËÛ˘ ÙÔ˘ ÂÓÔ¯ÔÔÈÔ‡ÌÂÓÔ˘ Ê·ÚÌ¿ÎÔ˘ ‹Ù·Ó Ë ·ÓÙÈÌÂÙÒÈÛË Ïԛ̈͢ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ‹ ÂÈÌÔÏ˘Ṳ̂ÓÔ˘ ÂÎ˙¤Ì·ÙÔ˜, ÂÓÒ Û ̛· ÂÚ›ÙˆÛË ‹Ù·Ó Ë ·ÓÙÈÌÂÙÒÈÛË ÌÔÏ˘ÛÌ·ÙÈÎÔ‡ ÎËÚ›Ô˘. ∫·Ì›· ·ÓÙ›‰Ú·ÛË ‰ÂÓ ‹Ù·Ó ÛÔ‚·Ú‹ Î·È fiϘ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Ì ¯ÏˆÚÊ·ÈÓÈÚ·Ì›ÓË ‹ Û·Ï‚Ô˘Ù·ÌfiÏË. ™Â ‰‡Ô ·ÛıÂÓ›˜ ¯ÔÚËÁ‹ıËÎ·Ó ÛÙÂÚÔÂȉ‹ ηٿ ÙË ‰ÔÎÈÌ·Û›· Î·È Û ¤Ó·Ó ¶›Ó·Î·˜ 1. ™˘¯ÓfiÙÂÚ· ·Ó·ÊÂÚfiÌÂÓ· Û˘ÌÙÒÌ·Ù· Û ʷÚ̷΢ÙÈΤ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ ∂Í¿ÓıËÌ·, Ô›‰ËÌ· ÚÔÛÒÔ˘ ∂Í¿ÓıËÌ· ∂Í¿ÓıËÌ·, ¤ÌÂÙÔ˜ ∫Ó›‰ˆÛË, Ô˘ÚÙÈοÚÈ·, ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· ∫·ı˘ÛÙÂÚË̤ÓË ·ÓÙ›‰Ú·ÛË ∫Ó›‰ˆÛË, Ô›‰ËÌ· ÚÔÛÒÔ˘ ∫Ó›‰ˆÛË

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Pediatr Sept-Oct 05

17-10-05

16:51

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∂ÓÔ¯ÔÔÈÔ‡ÌÂÓÔ Ê¿ÚÌ·ÎÔ Erythromycin: 4 Septrin/Penicillin: 2 Ibuprufen: 1 Lidocaine: 1 Aspirin: 3 Clarithromycin: 1

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Amoxicillin: 6 PenicillinV: 4 0

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3

4

5

6

7

∂ÈÎfiÓ· 1. ™˘¯ÓfiÙÂÚ· ÂÓÔ¯ÔÔÈÔ‡ÌÂÓ· Ê¿Ú̷η ÁÈ· ÙËÓ ÚfiÎÏËÛË ·ÏÏÂÚÁÈÎÒÓ ·ÓÙȉڿÛˆÓ.

·‰ÚÂÓ·Ï›ÓË ÁÈ· ¯Ú‹ÛË ÂÎÙfi˜ ÓÔÛÔÎÔÌ›Ԣ Û ÂÚ›ÙˆÛË ·Ó¿Á΢ (Epipen). ™Â ÔÎÙÒ ·fi Ù· ·È‰È¿ Ô˘ ›¯·Ó ·ÚÓËÙÈ΋ “ÚfiÎÏËÛË” ˘‹Ú¯Â ÈÛÙÔÚÈÎfi ¿ÛıÌ·ÙÔ˜ ‹ ÂÎ˙¤Ì·ÙÔ˜. ŒÓ· ·È‰› ÂÍÂÙ¿ÛÙËΠÁÈ· Èı·Ó‹ ·ÏÏÂÚÁ›· ÛÙËÓ ÂÓÈÎÈÏ›ÓË ÌÂÙ¿ ·fi ¤Ó·ÚÍË ÚÔÊ˘Ï·ÎÙÈ΋˜ ‰fiÛ˘ Ô˘ Ï¿Ì‚·Ó ÏfiÁˆ ·ÓÔÛÔηٷÛÙÔÏ‹˜. ™ÙÔ follow-up Ô˘ ¤ÁÈÓ Ì ÙËÏÂʈÓÈ΋ Û˘Ó¤ÓÙ¢ÍË ÙˆÓ ÁÔÓ¤ˆÓ, ·ÓÙ·ÔÎÚ›ıËΠÙÔ 73%. ¶¤ÓÙ ·È‰È¿ (22%) ¯ÚÂÈ¿ÛÙËÎ·Ó ·ÓÙÈ‚ÈÔÙÈο ÌÂÙ¿ ÙËÓ ÚfiÎÏËÛË Î·È Î·Ó¤Ó· ‰ÂÓ ÂÌÊ¿ÓÈÛ ·ÓÙ›‰Ú·ÛË. ∂Í¿ÏÏÔ˘, ÔÈ ÁÔÓ›˜ fiÙ·Ó ÚˆÙ‹ıËÎ·Ó - ‰‹ÏˆÛ·Ó ÂÚÈÛÛfiÙÂÚÔ ·Ó·ÎÔ˘ÊÈṲ̂ÓÔÈ ÌÂÙ¿ ÙȘ ‰ÔÎÈ̷ۛ˜ Î·È ÈÔ Û›ÁÔ˘ÚÔÈ ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙÔ ›‰ÈÔ Ê¿ÚÌ·ÎÔ, Â¿Ó ·ԉ›¯ÙËΠ“·ıÒÔ”, ‹ ÙÔ ·ÛʷϤ˜ ˘ÔηٿÛÙ·Ùfi ÙÔ˘ ÛÙËÓ ·ÓÙ›ıÂÙË ÂÚ›ÙˆÛË. ™Â Ù¤ÛÛÂÚȘ ÂÚÈÙÒÛÂȘ, ˆÛÙfiÛÔ, ·Ó Î·È Ë ‰ÔÎÈÌ·Û›· ‹Ù·Ó ·ÚÓËÙÈ΋, ÔÈ ÁÔÓ›˜ ·Ú·‰¤¯ÙËÎ·Ó fiÙÈ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ÌËÓ ¯ÚËÛÈÌÔÔÈÔ‡Ó ÙÔ Ê¿ÚÌ·ÎÔ, ΢ڛˆ˜ ÏfiÁˆ ÚÔËÁÔ‡ÌÂÓ˘ ÙÚ·˘Ì·ÙÈ΋˜ ÂÌÂÈÚ›·˜.

™˘˙‹ÙËÛË ∏ Ê·Ú̷΢ÙÈ΋ ·ÏÏÂÚÁ›· Â›Ó·È Ì›· ÛÔ‚·Ú‹ Î·È ‰˘ÓËÙÈÎÒ˜ ·ÂÈÏËÙÈ΋ ÁÈ· ÙË ˙ˆ‹ ηٿÛÙ·ÛË Î·È ÁÈ’ ·˘Ùfi ÙÔÓ ÏfiÁÔ ¯Ú‹˙ÂÈ Û˘ÛÙËÌ·ÙÈ΋˜ ÎÏÈÓÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘ Î·È ·ÓÙÈÌÂÙÒÈÛ˘. ∆Ô ÈÛÙÔÚÈÎfi Î·È Ë ÚÔÛÂÎÙÈ΋ ÎÏÈÓÈ΋ ÂͤٷÛË Â›Ó·È Ù· ÚÒÙ· ‚‹Ì·Ù· ÚÔ˜ ·˘Ù‹ ÙËÓ Î·Ù‡ı˘ÓÛË. ∂›Û˘, Ôχ ÛËÌ·ÓÙÈÎfi Â›Ó·È Ó· ·Ô‰ÂȯÙ› Ë Û¯¤ÛË ÌÂٷ͇ ÙÔ˘ ‡ÔÙÔ˘ Ê·ÚÌ¿ÎÔ˘ Î·È Ù˘ ·ÏÏÂÚÁ›·˜. ™ÙË ÛÂÈÚ¿ ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ·ÚÔ˘ÛÈ¿ÛÙËÎÂ, ÔÈ ·ÛıÂÓ›˜ Ô˘ ›¯·Ó ıÂÙÈ΋ “ÚfiÎÏËÛË” ·Ó¤ÊÂÚ·Ó Ôχ Û·Ê‹ Û˘ÌÙÒÌ·Ù·, Û˘Ì‚·Ù¿ Ì ·Ó·Ê˘Ï·ÎÙÈ΋ ·ÓÙ›‰Ú·ÛË.

∂›Ó·È ÁÂÁÔÓfi˜ fiÙÈ fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚÔ ÔÈ ÎÏÈÓÈÎÔ› È·ÙÚÔ› Û˘ÓÈÛÙÔ‡Ó ·ÔÊ˘Á‹ ÙˆÓ Ê·ÚÌ¿ÎˆÓ Ô˘ ˘ÔÙ›ıÂÙ·È fiÙÈ ÚÔοÏÂÛ·Ó “·ÏÏÂÚÁÈ΋ ·ÓÙ›‰Ú·ÛË”, ¯ˆÚ›˜ ÚÔËÁÔ˘Ì¤Óˆ˜ Ó· ¤¯Ô˘Ó ·ԉ›ÍÂÈ ÙËÓ ·Ú·¿Óˆ Û¯¤ÛË Î·È ¯ˆÚ›˜ Ó· ·Ú·¤ÌÔ˘Ó ÙÔ˘˜ ÂӉȷÊÂÚfiÌÂÓÔ˘˜ ÁÈ· ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛË. ŒÙÛÈ, ·Ú·ÙËÚÂ›Ù·È Ì›· Ì›ˆÛË ÛÙÔÓ ·ÚÈıÌfi ÙˆÓ ‰È·ÁÓˆÛÙÈÎÒÓ ‰ÔÎÈÌ·ÛÈÒÓ Û ۯ¤ÛË Ì ÚÔËÁÔ‡ÌÂÓ· ¯ÚfiÓÈ·, Ë ÔÔ›· Ê¿ÓËÎÂ Î·È ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. ∆Ô ·ÔÙ¤ÏÂÛÌ· Â›Ó·È ÔÏÏÔ› ¿ÓıÚˆÔÈ Ó· ÔÓÔÌ¿˙ÔÓÙ·È “·ÏÏÂÚÁÈÎÔ›” ÂÓÒ ‰ÂÓ Â›Ó·È, ÌÂ Û˘Ó¤ÂÈ· Ó· ÛÙÂÚÔ‡ÓÙ·È ÙˆÓ ıÂÚ·¢ÙÈÎÒÓ È‰ÈÔÙ‹ÙˆÓ ÛËÌ·ÓÙÈÎÒÓ Û΢·ÛÌ¿ÙˆÓ (9). ∂ÈϤÔÓ, Ë ¯Ú‹ÛË ·ÓÙÈ‚ÈÔÙÈÎÒÓ “‰Â‡ÙÂÚ˘ ÁÚ·ÌÌ‹˜” ÁÈ’ ·˘Ùfi ÙÔÓ ÛÎÔfi ·˘Í¿ÓÂÈ Î·È ÙÔ ÔÏÔ¤Ó· ‰ÈÔÁÎÔ‡ÌÂÓÔ Úfi‚ÏËÌ· Ù˘ “·ÓÙÔ¯‹˜” (3). ∏ ÛËÌ·Û›· Ù˘ Ï‹ÚÔ˘˜ ‰ÈÂÚ‡ÓËÛ˘ ·˘ÙÒÓ ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ê·›ÓÂÙ·È ·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ¤Ó·˜ ÛËÌ·ÓÙÈÎfi˜ ·ÚÈıÌfi˜ ÙˆÓ ·È‰ÈÒÓ Ô˘ ·Ú·¤ÌÊıËÎ·Ó ÁÈ· ‰ÈÂÚ‡ÓËÛË ¿Û¯ÂÈ ·fi ¿ÛıÌ·, ¤Î˙ÂÌ· Î·È ¿ÏϘ ÓfiÛÔ˘˜ Ô˘ Û˘¯Ó¿ ¯Ú‹˙Ô˘Ó ıÂÚ·›·˜ Ì ·ÓÙÈ‚ÈÔÙÈο.

OÈ ‰ÔÎÈ̷ۛ˜ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ Â›Ó·È ·ÛÊ·Ï›˜ fiÙ·Ó Ú·ÁÌ·ÙÔÔÈÔ‡ÓÙ·È Û ÂÍÂȉÈÎÂ˘Ì¤ÓÔ ÓÔÛÔÎÔÌÂÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ, ÂÊfiÛÔÓ ˘¿Ú¯ÂÈ Ë ÂÙÔÈÌfiÙËÙ· Ó· ·ÓÙÈÌÂÙˆÈÛÙÔ‡Ó ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ. ™ÙË ÛÂÈÚ¿ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‰ÂÓ ˘‹ÚÍ·Ó ÛÔ‚·Ú¤˜ ·Ó·Ê˘Ï·ÎÙÈΤ˜ ·ÓÙȉڿÛÂȘ. ∞fi ÙËÓ ¿ÏÏË ÏÂ˘Ú¿, ÔÈ ÁÔÓ›˜ ÙˆÓ ·È‰ÈÒÓ Ê·›ÓÂÙ·È Ó· ‰Â›¯ÓÔ˘Ó ÂÌÈÛÙÔÛ‡ÓË Û ·˘Ù¿ Ù· ·ÔÙÂϤÛÌ·Ù·. ∞˘Ùfi ÂÓÈÛ¯‡ÂÙ·È ·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ù· ·È‰È¿ Ô˘ ¤Ï·‚·Ó ÙÔ ›‰ÈÔ Ê¿ÚÌ·ÎÔ ÌÂÙ¿ ·fi Ì›· ·ÚÓËÙÈ΋ ·ÓÙ›‰Ú·ÛË, ‰ÂÓ Â›¯·Ó ÂÚ·ÈÙ¤Úˆ ÚÔ‚Ï‹Ì·Ù·. µ¤‚·È·, ˘‹ÚÍÂ Î·È ¤Ó· ÌÈÎÚfi ÔÛÔÛÙfi ‰˘ÛÈÛÙ›·˜, ÙÔ ÔÔ›Ô - fiˆ˜ Ê¿ÓËΠ·fi ÙȘ ··ÓÙ‹ÛÂȘ ÙˆÓ ÁÔÓ¤ˆÓ - ‰ÂÓ Â›¯Â Ó· οÓÂÈ Ì ÙËÓ ›‰È· ÙË ‰ÔÎÈÌ·Û›·, ·ÏÏ¿ Ì ÙËÓ ÚÔÛˆÈ΋ ÙÔ˘˜ ÛÙ¿ÛË. ¢ÈÂÚ¢ÓÒÓÙ·˜ ÙË Û‡Á¯ÚÔÓË ‚È‚ÏÈÔÁÚ·Ê›· Û¯ÂÙÈο Ì ÙȘ ˘¿Ú¯Ô˘Û˜ ‰È·ÁÓˆÛÙÈΤ˜ ‰ÔÎÈ̷ۛ˜, Ï›Á˜ Â›Ó·È ·˘Ù¤˜ Ô˘ Â›Ó·È Â˘Ú¤ˆ˜ ·Ô‰ÂÎÙ¤˜ Î·È ¤Á΢Ú˜. ª›· ·fi ·˘Ù¤˜ Â›Ó·È Ë ‰ÔÎÈÌ·Û›· ÂÓÈÎÈÏ›Ó˘ Ì ˘Ô‰fiÚÈ· ‰ÂÚÌ·ÙÈ΋ ‰ÔÎÈÌ·Û›· (3), Ô˘ ·ÎÔÏÔ˘ıÂ›Ù·È ·fi ÂÓ‰Ô‰ÂÚÌÈÎfi - Â¿Ó ÙÔ ÚÒÙÔ Â›Ó·È ·ÚÓËÙÈÎfi - Û ·˘Í·ÓfiÌÂÓ˜ ‰fiÛÂȘ. ∞˘Ù‹ Ë ‰ÔÎÈÌ·Û›· ¤¯ÂÈ Î·Ï‹ ÂȉÈÎfiÙËÙ· Î·È Ì¤ÙÚÈ· ¢·ÈÛıËÛ›· (5), Ô˘ ·˘Í¿ÓÂÙ·È Ì ÙË ¯Ú‹ÛË ÂÈϤÔÓ ·ÏÏÂÚÁÈÔÁfiÓˆÓ (9). °È· ÙȘ ÎÂÊ·ÏÔÛÔÚ›Ó˜ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ·ÊÂÓfi˜ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚÔÎÏ‹ÛÂȘ Û ·˘Í·ÓfiÌÂÓ˜ ‰fiÛÂȘ, ·ÊÂÙ¤ÚÔ˘ ¶·È‰È·ÙÚÈ΋ 2005;68:337-340

Pediatr Sept-Oct 05

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A. ™ÔÊÈ·ÓÔ‡ - ∫·ÙÛÔ‡ÏË Î·È Û˘Ó.

‰ÂÚÌ·ÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ (1,2). ª›· ÚfiÛÊ·ÙË ÂÚÁ·Û›· (10) ·Ó·Ê¤ÚÂÈ fiÙÈ ÔÈ ‰ÂÚÌ·ÙÈΤ˜ ‰ÔÎÈ̷ۛ˜ ÌÔÚ› Ó· Â›Ó·È ·ÚÓËÙÈΤ˜ Û ÂÚÈÙÒÛÂȘ ·ÏÏÂÚÁ›·˜ Û ‚-Ï·ÎÙ·ÌÈο ·ÓÙÈ‚ÈÔÙÈο ÏfiÁˆ ·ÓÙÔ¯‹˜. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, ÔÈ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚÔÎÏ‹ÛÂȘ ıˆÚÔ‡ÓÙ·È ÂÚÈÛÛfiÙÂÚÔ ·ÍÈfiÈÛÙ˜. ∏ ‰È¿ÁÓˆÛË Ù˘ ·ÏÏÂÚÁ›·˜ ÛÙËÓ ·ÛÈÚ›ÓË Î·È Ù· ÌË ÛÙÂÚÔÂȉ‹ ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë (ª™∞º) Á›ÓÂÙ·È Î˘Ú›ˆ˜ Ì ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚÔÎÏ‹ÛÂȘ, ÂÓÒ ·¢·ÈÛıËÙÔÔ›ËÛË ÚÔÙ›ÓÂÙ·È Û ÂÚÈÙÒÛÂȘ ÛÔ‚·ÚÒÓ ·ÓÙȉڿÛˆÓ. OÈ ‰È·ÛÙ·˘ÚÔ‡ÌÂÓ˜ ·ÓÙȉڿÛÂȘ ÌÂٷ͇ ·ÛÈÚ›Ó˘ Î·È ª™∞º ‰ÂÓ ¤¯Ô˘Ó ÂȂ‚·Èˆı›, ÁÈ’ ·˘Ùfi Ú¤ÂÈ Ó· ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ÂÈʇϷÍË (4). ∆¤ÏÔ˜, Ë ÚfiÎÏËÛË ÁÈ· ÙË ‰È¿ÁÓˆÛË ·ÏÏÂÚÁ›·˜ ÛÙ· ÙÔÈο ·Ó·ÈÛıËÙÈο ‰ÂÓ ıˆÚÂ›Ù·È ··Ú·›ÙËÙË, ÂÎÙfi˜ Â¿Ó ÙÔ Ê¿ÚÌ·ÎÔ ‰ÂÓ Â›Ó·È ÁÓˆÛÙfi (7). ™Â ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË Ú·ÁÌ·ÙÔÔÈÂ›Ù·È ‰ÂÚÌ·ÙÈ΋ ‰ÔÎÈÌ·Û›· Û ·˘Í·ÓfiÌÂÓ˜ ‰fiÛÂȘ (8). ™Â ÂÚ¢ÓËÙÈÎfi Â›Â‰Ô ‚Ú›ÛÎÂÙ·È Ë Ì¤ÙÚËÛË ÂÈ¤‰ˆÓ Ï¢ÎÔÙÚÈÂÓÒÓ ÌÂÙ¿ ·fi ‰È¤ÁÂÚÛË ÙˆÓ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ ·fi ·ÏÏÂÚÁÈÔÁfiÓÔ (Cellular antigen stimulation test - CAST) ÁÈ· ÙË ‰È¿ÁÓˆÛË ·ÏÏÂÚÁ›·˜ Û ª™∞º. ∆· RAST Î·È UniCAP ·ÓȯÓÂ‡Ô˘Ó ·ÓÙÈÛÒÌ·Ù· Û ʿÚ̷η. ∆Ô ÙÂÛÙ “ÌÂÙ·ÌfiÚʈÛ˘ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ” (Lymphocyte transformation test - LTT) ÂÊ·ÚÌfi˙ÂÙ·È ÛÙË ‰È¿ÁÓˆÛË ·ÓÙȉڿÛÂˆÓ Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi Ù‡Ô˘ πV ·ÓÔÛÔÏÔÁÈΤ˜ ·ÓÙȉڿÛÂȘ (2). ™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ “ÚÔÎÏ‹ÛÂȘ” ·ÔÙÂÏÔ‡Ó ·ÛÊ·Ï›˜ ‰ÔÎÈ̷ۛ˜, ÂÊfiÛÔÓ Ú·ÁÌ·ÙÔÔÈÔ‡ÓÙ·È Û ÂÍÂȉÈÎÂ˘Ì¤ÓÔ ÓÔÛÔÎÔÌÂÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ Î·È Ì ϋ„Ë Î·Ù¿ÏÏËÏˆÓ ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ ÁÈ· ÙÔ ÂӉ¯fiÌÂÓÔ ·Ó·Ê˘Ï·ÎÙÈ΋˜ ·ÓÙ›‰Ú·Û˘. ∞fi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ Ê¿ÓËΠfiÙÈ Ë ‰È¿ÁÓˆÛË ·ÏÏÂÚÁ›·˜ ÛÂ Û˘ÁÎÂÎÚÈ̤ӷ Û΢¿ÛÌ·Ù· Ì ·˘ÙfiÓ ÙÔÓ ÙÚfiÔ Â›Ó·È ·ÍÈfiÈÛÙË. ¶Ú¤ÂÈ Ó· ÂÈÛËÌ·Óı›, ˆÛÙfiÛÔ, fiÙÈ Ë ÛÂÈÚ¿ ·ÛıÂÓÒÓ ÛÙËÓ ÔÔ›· ‚·Û›˙ÂÙ·È Ë Û˘ÁÎÂÎÚÈ̤ÓË ÌÂϤÙË Â›Ó·È ÌÈÎÚ‹. ∂Ô̤ӈ˜, ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÈÎ˘Úˆı› Ë ÂÁ΢ÚfiÙËÙ· ·˘-

¶·È‰È·ÙÚÈ΋ 2005;68:337-340

ÙÒÓ ÙˆÓ ÚÔÎÏ‹ÛÂˆÓ Î·Ïfi ı· ‹Ù·Ó Ó· ˘¿ÚÍÂÈ Ì›· ÌÂÁ·Ï‡ÙÂÚË ÌÂϤÙË Ô˘ ı· ÂÚÈÏ·Ì‚¿ÓÂÈ ÙË Û‡ÁÎÚÈÛ‹ ÙÔ˘˜ Ì ̛· ¿ÏÏË ‰ÔÎÈÌ·Û›· Ô˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙË ‰È¿ÁÓˆÛË Ê·Ú̷΢ÙÈÎÒÓ ·ÏÏÂÚÁÈÒÓ. ™Â οı ÂÚ›ÙˆÛË Èı·Ó‹˜ Ê·Ú̷΢ÙÈ΋˜ ·ÏÏÂÚÁ›·˜ Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÂÙ·È ¤Ó· ηÏfi ÈÛÙÔÚÈÎfi, Ó· Ú·ÁÌ·ÙÔÔÈÂ›Ù·È ÚÔÛÂÎÙÈ΋ ÎÏÈÓÈ΋ ÂͤٷÛË Î·È Û ÂÚ›ÙˆÛË Ô˘ ·fi ·˘Ù¿ ÚÔ·ÙÂÈ ÈÛ¯˘Ú‹ ˘Ô„›·, Ó· Á›ÓÂÙ·È ·Ú·ÔÌ‹ ÙˆÓ ·È‰ÈÒÓ Û ÂȉÈÎfi ÁÈ· ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛË.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Gruchalla RS. Drug allergy. J Allergy Clin Immunol 2003;111 (Suppl 2):S548-S559.

2. Primeau MN, Adkinson NF Jr. Recent advances in the diagnosis of drug allergy. Curr Opin Allergy Clin Immunol 2001;1:337-341.

3. Gruchalla RS. Understanding drug allergies. J Allergy Clin Immunol 2000;105:637-644.

4. Grattan CE. Aspirin sensitivity and urticaria. Clin Exp Dermatol 2003;28:123-127.

5. Torres MJ, Blanca M, Fernandez J, Romano A, Weck A, Aberer W et al. EAACI Interest Group on Drug Hypersensitivity. Diagnosis of immediate allergic reactions to beta-lactam antibiotics. Allergy 2003;58:961-972.

6. Impicciatore P, Choonara I, Clarkson A, Provasi D, Pandolfini C, Bonati M. Incidence of adverse drug reactions in paediatric in/out-patients: a systematic review and meta-analysis of prospective studies. Br J Clin Pharmacol 2001;52:77-83.

7. deShazo RD, Kemp SF. Allergic reactions to drugs and biologic agents. JAMA 1997;278:1895-1906.

8. Cetinkaya F. Sensitivity to local anaesthetics among asthmatic children. Int J Paediatr Dent 2001;11:405408.

9. Macy E, Burchette RJ. Oral antibiotic adverse reactions after penicillin skin testing: multi-year followup. Allergy 2002;57:1151-1158.

10. Aberer W, Bircher A, Romano A, Blanca M, Campi P, Fernandez J et al. European Network for Drug Allergy (ENDA). EAACI Interest Group on Drug Hypersensitivity. Drug provocation testing in the diagnosis of drug hypersensitivity reactions: general considerations. Allergy 2003;58:854-863.

Pediatr Sept-Oct 05

17-10-05

16:51

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REVIEW ARTICLE

341

Evaluation of oral challenges for the diagnosis of drug allergy A. Sofianou - Katsoulis1, G. Du Toit2, G. Lack3

Abstract Background: Allergic reactions to drugs in the paediatric population are serious and potentially life threatening events that are often poorly diagnosed and managed. This is due to a lack of validated and readily available diagnostic tests, as well as of consensus guidelines for their management.

Methods: This was a retrospective study of 22 patients who underwent provocation tests for the diagnosis of reported drug allergy. The testing was made at St. Mary’s Hospital, London. The tests were predominantly oral challenges with the exception of lidocaine/procaine, for which a skin test was performed. Most of the tests were done to test penicillin allergy (40%). Three quarters were single compared to combined challenges.

Results: Six patients (28%) reacted, five of whom had an immediate and one a delayed reaction.

None of them had a reaction upon re-exposure to a suggested medication. The parents of the children who had a negative challenge felt that the outcome was reassuring. For those whose children had a positive outcome, it enhanced their confidence in the use of alternative drugs.

1 Paediatric Specialist Registrar, St. Mary’s Hospital, London, UK 2 Consultants in Paediatric Allergy, Department of Paediatric Allergology and Immunology, St. Mary’s Hospital, London, UK Correspondence: A. Sofianou-Katsoulis 42 Abbots Park, St. Albans Herts, UK AL1 1TW E-mail: katsofkat@hotmail.com Date of submission: 17-09-2004 Date of approval: 27-04-2005

Conclusions: Drug challenges need to be performed at a specialized centre particularly in patients where the history is equivocal. Many of the present diagnostic tests need validation prior to their generalization. Larger cohort studies are also required to establish the long-term prognosis in these cases.

Key words Drug challenges, drug allergy, adverse events.

Paediatriki 2005;68:341

Pediatr Sept-Oct 05

17-10-05

342

16:51

™ÂÏ›‰·342

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∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ· µ. ∆Û·ÁÚ‹˜1, ∞. ∫ÈÙÛÔÔ‡ÏÔ˘2, ∫. ª›¯·˜2, ¶. ¡ÈÎÔÏ·˝‰Ô˘ - ∫·Ú·ı›Ô˘1

1 °’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô Ã·˚‰·Ú›Ô˘ “∞ÙÙÈÎfiÓ”, ∞ı‹Ó· 2 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, “£ÚÈ¿ÛÈÔ” °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ∂ÏÂ˘Û›Ó·˜, ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: µ·Û›ÏÂÈÔ˜ ∆Û·ÁÚ‹˜ °’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô Ã·˚‰·Ú›Ô˘ “∞ÙÙÈÎfiÓ” ƒ›ÌÈÓÈ 1, ÷˚‰¿ÚÈ, ∞ı‹Ó· E-mail: billtsag@yahoo.com ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 22-10-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 13-04-2005

¶·È‰È·ÙÚÈ΋ 2005;68:342-350

¶ÂÚ›ÏË„Ë ∏ ÈÓÔÌ˘·ÏÁ›· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ·ÔÙÂÏ› Ì›· ·ÚÎÂÙ¿ Û˘Ó‹ıË, ÂÒ‰˘ÓË Ì˘ÔÛÎÂÏÂÙÈ΋ Û˘Ó‰ÚÔÌ‹, ¯·Ú·ÎÙËÚÈ˙fiÌÂÓË ·fi Â›ÌÔÓÔ ‰È¿¯˘ÙÔ fiÓÔ, ·ÚÔ˘Û›· ÔÏÏ·ÏÒÓ ‰È·ÎÚÈÙÒÓ Â˘·›ÛıËÙˆÓ ÛËÌ›ˆÓ ÛÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË, ‰˘Ûη̄›·, ‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘ Î·È ÎfiˆÛË. ∏ ÔÚ›· Ù˘ ÓfiÛÔ˘ Â›Ó·È ¯ÚÔÓ›˙Ô˘Û· Î·È ÂËÚ¿˙ÂÈ ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ÈηÓfiÙËÙ· Î·È ÙËÓ „˘¯ÔÎÔÈÓˆÓÈ΋ ·Ó¿Ù˘ÍË ÙˆÓ ·Û¯fiÓÙˆÓ ·È‰ÈÒÓ. ∞Ó Î·È Ë ·ÈÙÈÔÏÔÁ›· ·Ú·Ì¤ÓÂÈ ¿ÁÓˆÛÙË, ıˆÚÂ›Ù·È fiÙÈ Ì›· ÏÂÈ¿‰· ·Ú·ÁfiÓÙˆÓ ÌÔÚ› Ó· Û˘Ì‚¿ÏÏÂÈ ÛÙËÓ ÎÏÈÓÈ΋ ¤ÎÊÚ·ÛË Ù˘ ÓfiÛÔ˘. ∏ ıÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Â›Ó·È ÌË ÂȉÈ΋ Î·È ÔχÏ¢ÚË, ÂÚÈÏ·Ì‚¿ÓÔÓÙ·˜ Ê·ÚÌ·ÎÔÏÔÁÈο Î·È ÌË Ì¤ÙÚ·. §¤ÍÂȘ ÎÏÂȉȿ πÓÔÌ˘·ÏÁ›·, ·È‰È¿, fiÓÔ˜, ıÂÚ·›·, Ì˘ÔÛÎÂÏÂÙÈÎfi˜.

∂ÈÛ·ÁˆÁ‹ ∞ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ·ÈÙÈÔÏÔÁ›·, Ô fiÓÔ˜ ·ÔÙÂÏ› Ì›· Û˘¯Ó‹ ÂÌÂÈÚ›· ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜. ∞Ó Î·È ÛÙ· Ôͤ· ÂÒ‰˘Ó· Ì˘ÔÛÎÂÏÂÙÈο Û‡Ó‰ÚÔÌ· Ë ·ÈÙ›· Â›Ó·È Û˘Ó‹ıˆ˜ ÚÔÊ·Ó‹˜, ÛÙËÓ ÂÚ›ÙˆÛË ÙˆÓ ¯ÚfiÓÈˆÓ Û˘Ó‰ÚfiÌˆÓ Ë ‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË Û˘¯Ó¿ ·Ô‰ÂÈÎÓ‡ÂÙ·È ‰˘Û¯ÂÚ‹˜. O fiÓÔ˜ ·ÔÙÂÏ› Ì›· ÂÌÂÈÚ›· Ì ‰È·ÛÙ¿ÛÂȘ Ú·ÁÌ·ÙÈΤ˜ ÁÈ· ·˘ÙfiÓ Ô˘ ÙË ‚ÈÒÓÂÈ, ˘Ô‚·ıÌ›˙ÂÈ ÛËÌ·ÓÙÈο ÙËÓ ÔÈfiÙËÙ· ˙ˆ‹˜ ÙÔ˘ ¿Û¯ÔÓÙÔ˜ ·È‰ÈÔ‡ Î·È Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ Î·È Ô‰ËÁ› ÛÂ Û˘¯Ó¤˜ Î·È ÂӉ¯Ô̤ӈ˜ ·ÙÂϤÛÊÔÚ˜ ÂÈÛΤ„ÂȘ Û Â·ÁÁÂÏ̷ٛ˜ ˘Á›·˜ ‰È·ÊfiÚˆÓ ÂȉÈÎÔًوÓ. ∏ ÈÓÔÌ˘·ÏÁ›· ·ÔÙÂÏ› ‰È·Ù·Ú·¯‹ ÌË ÊÏÂÁÌÔÓÒ‰Ô˘˜ ·ÈÙÈÔÏÔÁ›·˜, Ë ÔÔ›· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÚÔÂÍ¿Ú¯Ô˘Û· Û˘Ìو̷ÙÔÏÔÁ›· ‰È¿¯˘ÙÔ˘ fiÓÔ˘ ‹/Î·È ‰˘Ûη̄›·˜ Ì˘˚ÎÒÓ ÔÌ¿‰ˆÓ ‹ Î·È ·ÚıÚÒÛÂˆÓ Î·È ·Ó‡ÚÂÛË Û˘ÁÎÂÎÚÈÌ¤ÓˆÓ ÛËÌ›ˆÓ ÂÓ Ùˆ ‚¿ıÂÈ Â˘·ÈÛıËÛ›·˜ ηٿ ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË. ∆·ÍÈÓÔÌÂ›Ù·È ÛÙË ‰È·ÁÓˆÛÙÈ΋ ÔÌ¿‰· ÙÔ˘ ‰È¿¯˘ÙÔ˘ ȉÈÔ·ı‹ Ì˘ÔÛÎÂÏÂÙÈÎÔ‡ fiÓÔ˘. ¶ÚfiÛÊ·Ù·, ÂÈÛ‹¯ıË Ô ÂÚÈÁÚ·ÊÈÎfiÙÂÚÔ˜ fiÚÔ˜ “Ó·ÓÈÎfi ÚˆÙÔ·ı¤˜ Û‡Ó‰ÚÔÌÔ ÈÓÔÌ˘·ÏÁ›·˜”, ÛÙËÓ ÚÔÛ¿ıÂÈ· ·ÓÙȉȷÛÙÔÏ‹˜ ÙˆÓ ‰È·ÊÔÚÒÓ Ù˘ ÎÏÈÓÈ΋˜ ¤ÎÊÚ·Û˘ Û ·È‰È¿ Î·È ÂÓ‹ÏÈΘ, ·ÏÏ¿ Î·È ÙˆÓ ‰È·ÁÓˆÛÙÈÎÒÓ È‰È·ÈÙÂÚÔًوÓ. OÈ ·Ï·ÈfiÙÂÚÔÈ fiÚÔÈ “Ì˘·ÏÁ›· ˘fi Ù¿ÛË”, “ÁÂÓÈÎÂ˘Ì¤ÓË ÙÂÓÔÓÙÔÌ˘Ô¿ıÂÈ·”, “ÈÓÔÌ˘ÔÛ›Ùȉ·” ‹ “ÈÓÔÛ›Ùȉ·”, ‡ÛÙÂÚ· ·fi ÂÈÛ‹ÁËÛË ÙÔ˘ ∞ÌÂÚÈηÓÈÎÔ‡ ∫ÔÏÂÁ›Ô˘ ƒÂ˘Ì·ÙÔÏÔÁ›·˜ (∞∫ƒ), ‰ÂÓ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ϤÔÓ (1). °È· ÏfiÁÔ˘˜ ·ÏfiÙËÙ·˜ Î·È ·ÔÊ˘Á‹˜ Û‡Á-

¯˘Û˘, ÔÈ Û˘ÁÁÚ·Ê›˜ ÙÔ˘ ·ÚfiÓÙÔ˜ ¿ÚıÚÔ˘ Â¤ÏÂÍ·Ó ÙË ¯Ú‹ÛË ÙÔ˘ ‰È·¯ÚÔÓÈÎfiÙÂÚÔ˘ fiÚÔ˘ “ÈÓÔÌ˘·ÏÁ›·”.

∏ ·ıÔÊ˘ÛÈÔÏÔÁ›· ÙÔ˘ fiÓÔ˘ O fiÓÔ˜ ‰È·ÎÚ›ÓÂÙ·È Û ‰‡Ô ·ÚÈÔ˘˜ Ù‡Ô˘˜: ÙÔÓ “Ù·¯‡” fiÓÔ (Ô͇˜ ‹ Ó˘ÁÌ҉˘) Î·È ÙÔÓ “‚Ú·‰‡” fiÓÔ (η˘ÛÙÈÎfi˜, ‚‡ıÈÔ˜, ¯ÚfiÓÈÔ˜ ‹ Ûʇ˙ˆÓ), Ô ÔÔ›Ô˜ Á›ÓÂÙ·È ·ÈÛıËÙfi˜ ÌÂÙ¿ ·fi 1 sec ‹ ÂÚÈÛÛfiÙÂÚÔ, Ì ‚·ıÌÈ·›· ·‡ÍËÛË Ù˘ ¤ÓÙ·Û‹˜ ÙÔ˘. O Ù·¯‡˜ fiÓÔ˜ ·ÊÔÚ¿ ΢ڛˆ˜ ÛÙÔ ‰¤ÚÌ· Î·È ‰ÂÓ Á›ÓÂÙ·È ·ÈÛıËÙfi˜ ·fi ÙÔ˘˜ ÂÓ Ùˆ ‚¿ıÂÈ ÈÛÙÔ‡˜ Î·È Ô ‚Ú·‰‡˜ fiÓÔ˜, Ô ÔÔ›Ô˜ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙËÓ ÈÓÔÌ˘·ÏÁ›·, ÌÔÚ› Ó· ÚÔ¤Ú¯ÂÙ·È ÙfiÛÔ ·fi ÙÔ ‰¤ÚÌ· fiÛÔ Î·È ·fi ÔÔÈ·‰‹ÔÙ ÂÓ Ùˆ ‚¿ıÂÈ ‰ÔÌ‹. ¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ fiÏÔÈ ÔÈ ˘ԉԯ›˜ fiÓÔ˘ Â›Ó·È ÂχıÂÚ˜ Ó¢ÚÈΤ˜ ·ÔÏ‹ÍÂȘ, ÁÈ· ÙËÓ ·ÁˆÁ‹ ÙˆÓ Ó¢ÚÈÎÒÓ ÒÛÂˆÓ ÚÔ˜ ÙÔ ∫¡™ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ‰‡Ô ͯˆÚÈÛÙ¤˜ Ó¢ÚÈΤ˜ Ô‰Ô›, Ì›· ÁÈ· ÙÔ Ù·¯‡, Ô͇ ¿ÏÁÔ˜ Î·È Ì›· ÁÈ· ÙÔ ‚Ú·‰‡, ¯ÚfiÓÈÔ ¿ÏÁÔ˜. ∆· Û‹Ì·Ù· ÁÈ· ÙÔ ‚Ú·‰‡ ¿ÏÁÔ˜ ÂÎχÔÓÙ·È ·fi ¯ËÌÈÎÔ‡ Ù‡Ô˘ ÂÚÂı›ÛÌ·Ù· (‹ ηٿ ÂÚ›ÙˆÛË ·fi Â›ÌÔÓ· Ì˯·ÓÈο ‹ ıÂÚÌÈο) Î·È ¿ÁÔÓÙ·È ÚÔ˜ ÙÔÓ ÓˆÙÈ·›Ô Ì˘ÂÏfi Ì ÂÚÈÊÂÚÈο Ó‡ڷ ·ÔÙÂÏÔ‡ÌÂÓ· ·fi ÙȘ ÛÙÂÚÔ‡ÌÂÓ˜ Ì˘ÂÏ›Ó˘ ›Ó˜ C. ™·Ó Ó¢Úԉȷ‚È‚·ÛÙ¤˜ ÏÂÈÙÔ˘ÚÁÔ‡Ó ÙÔ ˘fiÛÙڈ̷ ƒ Î·È ÂÙ›‰È· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔ ÁÔÓ›‰ÈÔ Ù˘ ηÏÛÈÙÔÓ›Ó˘. ªÂÙ¿ ÙËÓ Â›ÛÔ‰fi ÙÔ˘˜ ÛÙÔÓ ÓˆÙÈ·›Ô Ì˘ÂÏfi, Ù· ÂÚÂı›ÛÌ·Ù· fiÓÔ˘ ·ÎÔÏÔ˘ıÔ‡Ó ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜ Ô‰Ô‡˜ ÚÔ˜ ÙÔÓ ÂÁΤʷÏÔ: ÙË

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·343

343

∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ·

ÓÂÔÓˆÙÈ·ÈÔı·Ï·ÌÈ΋ Ô‰fi ÁÈ· ÙÔÓ Ù·¯‡ fiÓÔ Î·È ÙËÓ ·Ï·ÈÔÓˆÙÈ·ÈÔı·Ï·ÌÈ΋ Ô‰fi ÁÈ· ÙÔÓ ‚Ú·‰‡ fiÓÔ, Ô˘ ηٷϋÁÂÈ Î˘Ú›ˆ˜ ÛÙÔ ÂÁÎÂÊ·ÏÈÎfi Û٤ϯԘ (2).

O ‚·ıÌfi˜ ·ÓÙ›‰Ú·Û˘ ÛÙÔÓ fiÓÔ ÔÈΛÏÏÂÈ ÛËÌ·ÓÙÈο ·fi ¿ÙÔÌÔ Û ¿ÙÔÌÔ, ÁÂÁÔÓfi˜ ÙÔ ÔÔ›Ô ÂӉ¯Ô̤ӈ˜ ÔÊ›ÏÂÙ·È ÛÙËÓ ÈηÓfiÙËÙ· ÙÔ˘ ›‰ÈÔ˘ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Ó· ÂϤÁ¯ÂÈ ÙËÓ Â›ÛÔ‰Ô ÂÒ‰˘ÓˆÓ ÂÚÂıÈÛÌ¿ÙˆÓ ÛÙÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ·, ̤ۈ ÂÓfi˜ Û˘ÛÙ‹Ì·ÙÔ˜ ·Ó·ÏÁËÛ›·˜. ∂ÎÙfi˜ ·fi ÙȘ ·Ó·ÙÔÌÈΤ˜ ‰Ô̤˜, ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙË ÏÂÈÙÔ˘ÚÁ›· ·˘ÙÔ‡ ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ η٤¯Ô˘Ó Ù· ÂÓ‰ÔÁÂÓ‹ ÔÈÔÂȉ‹ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘. ∆· ÚÒÙ· ÔÈÔÂȉ‹, ÔÈ ÂÁÎÂʷϛӘ, ÂÚÈÁÚ¿ÊËÎ·Ó ÙÔ 1975, Ì ·ÚÈÔ ÂÎÚfiÛˆÔ ÙË ‚-ÂÓ‰ÔÚÊ›ÓË. ¶ÂÚÈÛÛfiÙÂÚ· ·fi 20 ÂÓ‰ÔÁÂÓ‹ ÔÈÔÂȉ‹ ÂÙ›‰È· ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ̤¯ÚÈ Û‹ÌÂÚ·, Ì ٷ ÓÂfiÙÂÚ· - ÂÓ‰ÔÌÔÚʛӘ Î·È ·ÏÁÔÛÙ·Ù›ÓË - Ó· ‚Ú›ÛÎÔÓÙ·È ÛÙÔ Â›ÎÂÓÙÚÔ ÙÔ˘ ÂӉȷʤÚÔÓÙÔ˜. ™Â ÔÏÏ¿ ÛËÌ›· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ÂÓÙÔ›ÛÙËÎÂ Ë ·ÚÔ˘Û›· ˘Ô‰Ô¯¤ˆÓ ÁÈ· ÔÈÔÂȉ‹, ȉȷ›ÙÂÚ· ÛÙȘ ÂÚÈÔ¯¤˜ Ô˘ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙÔ Û‡ÛÙËÌ· ·Ó·ÏÁËÛ›·˜. OÈ ˘ԉԯ›˜ ·˘ÙÔ› ‰È·ÎÚ›ÓÔÓÙ·È Û ÙÚÂȘ ηÙËÁÔڛ˜: ÙÔ˘˜ Ì, Î Î·È ‰. ∞Ó Î·È Ë ‰Ú¿ÛË ÙÔ˘ ‰ÂÓ ¤¯ÂÈ Â·ÎÚÈ‚Ò˜ ηٷÓÔËı›, Ë ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ·Ó·ÏÁËÛ›·˜ Â›Ó·È ‰˘Ó·Ùfi Ó· ηٷÛÙ›ÏÂÈ Ï‹Úˆ˜ ·ÚÎÂÙ¿ ÂÒ‰˘Ó· ÂÚÂı›ÛÌ·Ù· Ô˘ ÂÈÛ¤Ú¯ÔÓÙ·È ÛÙÔ ∫¡™ ·fi ÙËÓ ÂÚÈʤÚÂÈ· (3).

∂ȉËÌÈÔÏÔÁ›· ∏ ÈÓÔÌ˘·ÏÁ›· ·ÔÙÂÏ› Ì›· ·fi ÙȘ Û˘¯ÓfiÙÂÚ˜ ÚÂ˘Ì·ÙÈΤ˜ ·ı‹ÛÂȘ Û ÂÓ‹ÏÈΘ, Ì ÂÚ›Ô˘ 5 ÂηÙÔÌ̇ÚÈ· ¿ÙÔÌ· Ó· ÓÔÛÔ‡Ó ÌfiÓÔ ÛÙȘ ∏¶∞. ™Â ÏËı˘ÛÌȷ΋ ÌÂϤÙË 900 ÂÓËϛΈÓ, Ù˘¯·ÈÔÔÈË̤ÓÔ˘ ‰Â›ÁÌ·ÙÔ˜ ·fi ÙË ™Ô˘Ë‰›·, Ë Â›ÙˆÛË Ù˘ ÓfiÛÔ˘ ÚÔÛ‰ÈÔÚ›ÛÙËΠÛÙÔ 1% (4). ™Â ·ÓÙ›ÛÙÔȯ˜ ÌÂϤÙ˜ ·fi ÙÔ πÛÚ·‹Ï, ÙÔ ªÂÍÈÎfi Î·È ÙË ºÈÓÏ·Ó‰›·, ‰È·ÈÛÙÒıËΠfiÙÈ Ë Û˘¯ÓfiÙËÙ¿ Ù˘ Û ·È‰È¿ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ ·Ó¤Ú¯ÂÙ·È Û ÔÛÔÛÙfi 6,2%, 1,3% Î·È 1,3%, ·ÓÙ›ÛÙÔȯ· (5-7). ™Â ÌÂϤÙ˜ ÚÔÂÚ¯fiÌÂÓ˜ ·fi ·È‰ÔÚÂ˘Ì·ÙÔÏÔÁÈο ΤÓÙÚ· ÙˆÓ ∏¶∞, Ë Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ Ù˘ ÓfiÛÔ˘ ÌÂٷ͇ ÙˆÓ Ó¤ˆÓ ‰È·ÁÓÒÛÂˆÓ ·˘Í¿ÓÂÙ·È ÚÔԉ¢ÙÈο, ÂÎÚÔÛˆÒÓÙ·˜ ÔÛÔÛÙfi 7% Â› ÙÔ˘ Û˘ÓfiÏÔ˘ (8). OÈ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ÙˆÓ Ê‡ÏˆÓ Â›Ó·È ÛËÌ·ÓÙÈΤ˜, Ì ٷ ÎÔÚ›ÙÛÈ· Ó· ÚÔÛ‚¿ÏÏÔÓÙ·È Ôχ Û˘¯ÓfiÙÂÚ· ·fi Ù· ·ÁfiÚÈ· (·Ó·ÏÔÁ›· 4:1 ‹ ·ÎfiÌË Î·È 9:1 Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜). ∏ ÂÊ˂›· ·ÔÙÂÏ› ÙË Û˘ÓËı¤ÛÙÂÚË ËÏÈΛ·

¤Ó·Ú͢ ÙˆÓ Û˘Ìو̿وÓ, ÂÓÒ Ë ϤÔÓ ÚÒÈÌË ‰È¿ÁÓˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ·ÊÔÚ¿ Û ·È‰› ËÏÈΛ·˜ 5 ÂÙÒÓ (9).

∞ÈÙÈÔÏÔÁ›· ∞Ó Î·È Ë ·ÈÙÈÔÏÔÁ›· ·Ú·Ì¤ÓÂÈ ¿ÁÓˆÛÙË, Ë ‰È·ÊÔÚÂÙÈ΋ ÎÏÈÓÈ΋ ÚÔ‚ÔÏ‹ Î·È ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· ÛÙ· ·È‰È¿ ·’ fi,ÙÈ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, ·ÏÏ¿ Î·È Ë ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ÚÔÛ‚ÔÏ‹˜ ÙˆÓ ÎÔÚÈÙÛÈÒÓ ¤Ó·ÓÙÈ ÙˆÓ ·ÁÔÚÈÒÓ, ÂÈÙÚ¤Ô˘Ó ÙËÓ ·Ó¿Ù˘ÍË ÔÏÏÒÓ ÂÈηÛÈÒÓ Û¯ÂÙÈο Ì ÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ·.

∆Ô Ì˘˚Îfi Û‡ÛÙËÌ· Œ¯ÂÈ ÂÎÊÚ·ÛÙ› Ë ¿Ô„Ë fiÙÈ ‰È·Ù·Ú·¯¤˜ Ù˘ ·Ó·ÙÔÌÈ΋˜ ‰ÔÌ‹˜ Î·È ÙˆÓ ‰ÈÂÚÁ·ÛÈÒÓ ÂȉÈfiÚıˆÛ˘ ÙÔ˘ Ì˘˚ÎÔ‡ ÈÛÙÔ‡ Â›Ó·È Èı·Ófi Ó· Û˘ÓÂÈÛʤÚÔ˘Ó ÛËÌ·ÓÙÈο ÛÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ªÂϤÙ˜ Ì ̷ÁÓËÙÈ΋ Ê·ÛÌ·ÙÔÛÎÔ›· Û˘ÓËÁÔÚÔ‡Ó ˘¤Ú ‰È·Ù·Ú·¯ÒÓ ÛÙÔ Û·ÚÎfiÏ·ÛÌ· ÙˆÓ Ì˘˚ÎÒÓ Î˘ÙÙ¿ÚˆÓ, Ô˘ ÚÔηÏÔ‡Ó ÌÂȈ̤ÓË ¯ÚËÛÈÌÔÔ›ËÛË ÙˆÓ ÂÓÂÚÁÂÈ·ÎÒÓ ·ÔıÂÌ¿ÙˆÓ Î·È, ÙÂÏÈο, ΢ÙÙ·ÚÈ΋ ˘ÔÍ›· (10). ∏ ·ÒÏÂÈ· ÙÔ˘ ··Ú·›ÙËÙÔ˘ ÁÈ· ÙË ‰È·Ù‹ÚËÛË Ù˘ ÛÙ·ıÂÚfiÙËÙ·˜ ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ÌÂÌ‚Ú·ÓÒÓ ∞∆ƒ, Ë ÔÔ›· ÚÔηÏÂ›Ù·È ÛÙÔ Ï·›ÛÈÔ Ù˘ ˘ÔÍ›·˜, Ô‰ËÁ› ÛÂ Û˘Ó·ÎfiÏÔ˘ıË Î·Ù·ÛÙÚÔÊ‹ ÙˆÓ Û·ÚÎÔÌÂÚȉ›ˆÓ. ∞˘Ù‹, Ì ÙË ÛÂÈÚ¿ Ù˘, ÚÔηÏ› ·ÒÏÂÈ· ÂÓ‰Ô΢ÙÙ¿ÚÈÔ˘ Î·Ï›Ô˘, ÙÔ ÔÔ›Ô ÂÓÂÚÁÔÔÈ› ÙÔ˘˜ ˘ԉԯ›˜ fiÓÔ˘, ÚÔηÏÒÓÙ·˜ ÙÔÈΤ˜ Û˘Û¿ÛÂȘ Ì˘˚ÎÒÓ ÔÌ¿‰ˆÓ, ÔÈ Ôԛ˜ ÂÓÈÛ¯‡Ô˘Ó ÂÚ·ÈÙ¤Úˆ ÙËÓ ·ÒÏÂÈ· Î·Ï›Ô˘ Î·È Ô‰ËÁÔ‡Ó ÙÂÏÈο Û ʷ‡ÏÔ Î‡ÎÏÔ.

OÚÌÔÓÈΤ˜ ÌÂÙ·‚ÔϤ˜ Î·È ‰È·Ù·Ú·¯¤˜ ‚ÈÔÁÂÓÒÓ ·ÌÈÓÒÓ ∏ ·Ó‡ÚÂÛË ¯·ÌËÏÒÓ ÂÈ¤‰ˆÓ ÂχıÂÚ˘ ÎÔÚÙÈ˙fiÏ˘ Ô‡ÚˆÓ Î·È ‰È·Ù·Ú·¯‹˜ ÙÔ˘ ËÌÂÚ‹ÛÈÔ˘ Ú˘ıÌÔ‡ ¤ÎÎÚÈÛ˘ ÎÔÚÙÈ˙fiÏ˘, ηıÒ˜ Î·È Ë ¤ÓÙÔÓË ¤ÎÎÚÈÛË ACTH ÌÂÙ¿ ·fi ÚfiÎÏËÛË ˘ÔÁÏ˘Î·ÈÌ›·˜, ¯ˆÚ›˜ ˆÛÙfiÛÔ ÙËÓ ·Ó·ÌÂÓfiÌÂÓË ¤ÎÎÚÈÛË ÎÔÚÙÈ˙fiÏ˘ ·fi Ù· ÂÈÓÂÊÚ›‰È·, Û˘ÓËÁÔÚÔ‡Ó ˘¤Ú Èı·Ó‹˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ˘Ôı·Ï·ÌÈÎÔ‡-˘ÔÊ˘ÛÈ·ÎÔ‡-ÂÈÓÂÊÚȉÈÎÔ‡ ¿ÍÔÓ·. ªÂȈ̤ÓË ‚Ú¤ıËÎÂ, Â›Û˘, Ë ¤ÎÎÚÈÛË ÎÔÚÙÈ˙fiÏ˘ ÌÂÙ¿ ·fi ÚfiÎÏËÛË Ì ¯ÔÚ‹ÁËÛË CRH, ‰È·›ÛÙˆÛË Ô˘ ηıÈÛÙ¿ Èı·Ófi ÙÔ ÚˆÙÔ·ı¤˜ ·›ÙÈÔ Û ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›· Ó· Â›Ó·È Ë ‰È·Ù·Ú·¯‹ Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ˘Ôı·Ï¿ÌÔ˘ (11-13). O Ì˯·ÓÈÛÌfi˜, ˆÛÙfiÛÔ, Ì ÙÔÓ ¶·È‰È·ÙÚÈ΋ 2005;68:342-350

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µ. ∆Û·ÁÚ‹˜ Î·È Û˘Ó.

ÔÔ›Ô ÔÈ ‰ÈÂÚÁ·Û›Â˜ ·˘Ù¤˜ Û˘ÓÂÈÛʤÚÔ˘Ó ÛÙËÓ ÂΉ‹ÏˆÛË Ù˘ ÎÏÈÓÈ΋˜ Û˘Ó‰ÚÔÌ‹˜ ·Ú·Ì¤ÓÂÈ ·‰È¢ÎÚ›ÓÈÛÙÔ˜. OÈ Bennett Î·È Û˘Ó ‰È·›ÛÙˆÛ·Ó ÂÏ·Ùو̤ӷ Â›‰· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ (GH) Û ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›· (14,15). ¶ÚfiÎÂÈÙ·È ÁÈ· ÔÚÌfiÓË Ô˘ ÂÎÎÚ›ÓÂÙ·È Û ·˘ÍË̤ӷ Â›‰· ηٿ ÙÔ ÛÙ¿‰ÈÔ 4 ÙÔ˘ ‡ÓÔ˘, ‰È·‰Ú·Ì·Ù›˙ÔÓÙ·˜ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÔÌÔÈfiÛÙ·ÛË ÙˆÓ Ì˘ÒÓ Î·È Â˘Ô‰ÒÓÔÓÙ·˜ ÙȘ ·Ó·‚ÔÏÈΤ˜ ÙÔ˘˜ ‰ÈÂÚÁ·Û›Â˜. ŒÙÛÈ, ÂÎÊÚ¿ÛÙËÎÂ Ë ¿Ô„Ë ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ fiÙÈ Ë ÁÓˆÛÙ‹ ·fi ÙÔ ·ÚÂÏıfiÓ ‰È·Ù·Ú·¯‹ ÙÔ˘ ÛÙ·‰›Ô˘ 4 ÙÔ˘ ‡ÓÔ˘ Û ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›·, Â›Ó·È ‰˘Ó·Ùfi Ó· ÂËÚ¿ÛÂÈ ÙËÓ ¤ÎÎÚÈÛË Ù˘ GH Î·È ·˘Ù‹, Ì ÙË ÛÂÈÚ¿ Ù˘, ÙȘ ‰ÈÂÚÁ·Û›Â˜ ·Ó·‰fiÌËÛ˘ ÙˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ (16,17). øÛÙfiÛÔ, Ë ·Ú·¿Óˆ ·ÈÙÈÔÏÔÁÈ΋ Û˘Û¯¤ÙÈÛË ·Ú·Ì¤ÓÂÈ ·ÌÊ›‚ÔÏË, ηıÒ˜ ·ÔÙÂÏ› ·ÓÙÈΛÌÂÓÔ ‰È¯ÔÁӈ̛·˜ ÙÔ ·Ó Ë ‰È·Ù·Ú·¯‹ Ù˘ Ê˘ÛÈÔÏÔÁ›·˜ ÙÔ˘ ‡ÓÔ˘ ·ÔÙÂÏ› Ì›· ÚˆÙÔ·ı‹ ‰È·Ù·Ú·¯‹ ‹ ·Ï¿ ÂÈÛ˘Ì‚·›ÓÂÈ ˆ˜ ·ÓÙ›‰Ú·ÛË ÛÙÔÓ fiÓÔ Ô˘ ˘ÔÛ˘Ó›‰ËÙ· ‚ÈÒÓÂÈ Ô ·ÛıÂÓ‹˜ ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘. ∏ ÚfiÎÏËÛË ˘ÔÁÏ˘Î·ÈÌ›·˜ ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ÈÓÛÔ˘Ï›Ó˘ Û ¿Û¯ÔÓÙ˜, Ô‰‹ÁËÛ Û ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË Ù˘ ¤ÎÎÚÈÛ˘ GH, οÙÈ ÙÔ ÔÔ›Ô ‰ÂÓ ·Ú·ÙËÚ‹ıËΠÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ÎÏÔÓȉ›Ó˘, ‰È·›ÛÙˆÛË Ô˘ Ô‰‹ÁËÛ ÛÙËÓ ˘fiıÂÛË fiÙÈ Ë ‰È·Ù·Ú·¯‹ ÙÔ˘ ¿ÍÔÓ· ÂÓÙÔ›˙ÂÙ·È Î·È Â‰Ò ÛÙÔ Â›Â‰Ô ÙÔ˘ ˘Ôı·Ï¿ÌÔ˘ (18). ™Â ÌÂϤÙ˜ Û ∂¡À ·Û¯fiÓÙˆÓ ·fi ÈÓÔÌ˘·ÏÁ›·, ‰È·ÈÛÙÒıËÎ·Ó ÂÏ·Ùو̤ӷ Â›‰· ‚ÈÔÁÂÓÒÓ ·ÌÈÓÒÓ (5∏π∞∞, ÔÌÔ‚·Ó˘ÏÏÈÎfi Ô͇, 3-ÌÂı˘Ï-4-˘‰ÚÔ͢˚Ó‰ÔÏÈÎfi Ô͇) Û ۯ¤ÛË Ì ˘ÁÈ›˜ Ì¿ÚÙ˘Ú˜ (19,20). ™Â ¿ÏϘ ÌÂϤÙ˜ ‰È·ÈÛÙÒıËÎ·Ó ÂÏ·Ùو̤ӷ Â›‰· ÛÂÚÔÙÔÓ›Ó˘ Î·È ·˘ÍË̤ÓÔ˜ ·ÚÈıÌfi˜ ˘Ô‰Ô¯¤ˆÓ Â·Ó·ÚfiÛÏ˄˘ ÛÂÚÔÙÔÓ›Ó˘ ÛÙ· ·ÈÌÔÂÙ¿ÏÈ·. ™ËÌÂÈÒÓÂÙ·È fiÙÈ Ë ÛÂÚÔÙÔÓ›ÓË Â˘Ô‰ÒÓÂÈ ÙËÓ ·ÂÏ¢ı¤ÚˆÛË CRH ÛÙÔÓ ˘Ôı¿Ï·ÌÔ Î·È ·Ó·ÛÙ¤ÏÏÂÈ ÙËÓ ¤ÎÎÚÈÛË ˘ÔÛÙÚÒÌ·ÙÔ˜ ƒ ÛÙÔ ∂¡À, Ô˘Û›·˜ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙË ‰ÈÂÚÁ·Û›· ÚfiÎÏËÛ˘ ÙÔ˘ fiÓÔ˘ Î·È Ù˘ ÔÔ›·˜ ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ ÛÙÔ ∂¡À ·Û¯fiÓÙˆÓ ¤¯Ô˘Ó ‚ÚÂı› ·˘ÍË̤Ó˜ (21). ∞fi ·ÚÎÂÙÔ‡˜ Û˘ÁÁÚ·Ê›˜, Ù· ·Ú·¿Óˆ Â˘Ú‹Ì·Ù· ÂÓÔÔÈÔ‡ÓÙ·È ÛÙÔ Ï·›ÛÈÔ Ì›·˜ ¢ڇÙÂÚ˘ ıˆڛ·˜ ÌÂȈ̤Ó˘ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ·˜ ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (22).

∆Ô ·ÓÔÛÔÏÔÁÈÎfi ˘fi‚·ıÚÔ ∂Í·ÈÚÂÙÈÎfi ÂӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÂϤÙ˜ Ô˘ ÂÛÙÈ¿˙ÔÓÙ·È ÛÙËÓ Î·Ù·ÛÙÔÏ‹ Ù˘ ‰Ú¿Û˘ ÙˆÓ ÂÓ‰ÔÁÂÓÒÓ ÔÈÔÂȉÒÓ, ‹ Ú˘ıÌÈÛÙÒÓ Ù˘ ‰Ú¿Û˘ ÙÔ˘˜, ·fi ·˘ÙÔ¿ÓÔÛÔ˘˜ Ì˯·Óȶ·È‰È·ÙÚÈ΋ 2005;68:342-350

ÛÌÔ‡˜. Œ¯ÂÈ ‰È·Ù˘ˆı› Ë ¿Ô„Ë fiÙÈ ·˘ÙÔ·ÓÙÈÛÒÌ·Ù· Â›Ó·È ‰˘Ó·Ùfi Ó· ÛÙÚ¤ÊÔÓÙ·È Î·Ù¿ ÙˆÓ ÂÙȉ›ˆÓ ‹ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ ÙÔ˘˜ Î·È Ó· Ù· ηٷÛÙÚ¤ÊÔ˘Ó ‹ Ó· ‰È·Ù·Ú¿ÛÛÔ˘Ó ÙË ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘˜. ™ÙË ıˆڛ· fiÙÈ ÚˆÙ·Ú¯ÈÎfi ÚfiÏÔ ÛÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ· Ù˘ ÓfiÛÔ˘ ‰È·‰Ú·Ì·Ù›˙ÂÈ Ì›· ‰È·Ù·Ú·¯‹ Ù˘ Ô‰Ô‡ ÌÂÙ·‚›‚·Û˘ ÙÔ˘ fiÓÔ˘, ¤¯ÂÈ ÚfiÛÊ·Ù· ÂÚÈÏËÊı› Ë Èı·Ó‹ ‰Ú¿ÛË ·˘ÙÔ¿ÓÔÛˆÓ ·Ú·ÁfiÓÙˆÓ, ÔÈ ÔÔ›ÔÈ ÛÙÚ¤ÊÔÓÙ·È Î·Ù¿ Ó¢ÚÔÂÙȉ›ˆÓ (fiˆ˜ ÙÔ ˘fiÛÙڈ̷ ƒ) ‹ ÔÚÌÔÓÒÓ Ù˘ Ô‰Ô‡ ·˘Ù‹˜. ªÂϤÙ˜ ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË ·˘ÙÒÓ ÙˆÓ ·˘ÙÔ·ÓÙÈۈ̿وÓ, ÙfiÛÔ ÛÙÔÓ ÔÚfi fiÛÔ Î·È ÛÙÔ ∂¡À ·ÛıÂÓÒÓ Ì ÈÓÔÌ˘·ÏÁ›·, ‚Ú›ÛÎÔÓÙ·È Û ÂͤÏÈÍË (23-25).

°ÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ∏ Û˘¯ÓfiÙËÙ· Ù˘ ÈÓÔÌ˘·ÏÁ›·˜ ÛÂ Û˘ÁÁÂÓ›˜ ÚÒÙÔ˘ ‚·ıÌÔ‡ ÙˆÓ ·ÛıÂÓÒÓ ·Ó¤Ú¯ÂÙ·È Û ÔÛÔÛÙfi 28-52%, ÂÓÒ Û ÌËÙ¤Ú˜ ÓÔÛÔ‡ÓÙˆÓ ·È‰ÈÒÓ Û ÔÛÔÛÙfi 71% (26). ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó¿Ù˘ÍË ıˆÚÈÒÓ Û¯ÂÙÈο Ì ÙËÓ ‡·ÚÍË ÁÂÓÂÙÈÎÔ‡ ˘ÔÛÙÚÒÌ·ÙÔ˜ Ù˘ ÓfiÛÔ˘. ∆Ô ÂÚ¢ÓËÙÈÎfi ÂӉȷʤÚÔÓ, Û‹ÌÂÚ·, ÂÛÙÈ¿˙ÂÙ·È ÛÙË Û˘Û¯¤ÙÈÛË Ù˘ ÓfiÛÔ˘ ÌÂ Û˘ÁÎÂÎÚÈ̤ӷ ·ÓÙÈÁfiÓ· ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ HLA, ηıÒ˜ Î·È ÛÙË ‰ÈÂÚ‡ÓËÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ ÔÏ˘ÌÔÚÊÈÛÌÔ‡ (Ù˘ ·ÚÔ˘Û›·˜, ‰ËÏ·‰‹, ÂÚÈÛÛfiÙÂÚˆÓ Ù˘ Ì›·˜ ‰ÔÌÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ) ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ 5-∏∆∆, ˘‡ı˘ÓÔ˘ ÁÈ· ÙË ÌÂÙ·ÊÔÚ¿ Ù˘ ÛÂÚÔÙÔÓ›Ó˘ (27). ¢È·Ù·Ú·¯¤˜ ÙÔ˘ ‡ÓÔ˘ OÈ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ‡ÓÔ˘ Û ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›· ¤¯Ô˘Ó Â·ÚÎÒ˜ ÌÂÏÂÙËı›, ˆÛÙfiÛÔ Ë ‰È¿ÎÚÈÛË ÌÂٷ͇ ·ÈÙ›Ô˘ Î·È ·ÈÙÈ·ÙÔ‡ ·Ú·Ì¤ÓÂÈ Î·È Û ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË ‰˘Û¯ÂÚ‹˜. ∂›Ó·È ÁÓˆÛÙfi fiÙÈ Ë ‰È¤ÁÂÚÛË Û˘ÁÎÂÎÚÈÌ¤ÓˆÓ ÂÚÈÔ¯ÒÓ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ÌÔÚ› Ó· ÚÔηϤÛÂÈ ‡ÓÔ Ì ¯·Ú·ÎÙËÚÈÛÙÈο ·ÚfiÌÔÈ· Ì ÂΛӷ ÙÔ˘ Ê˘ÛÈÎÔ‡. ∆¤ÙÔÈ· ÂÚÈÔ¯‹ ·ÔÙÂÏ› ·˘Ù‹ ÙˆÓ ˘Ú‹ÓˆÓ Ù˘ Ú·Ê‹˜, ÛÙËÓ Î¿Ùˆ Á¤Ê˘Ú· Î·È ÙÔÓ ÚÔÌ‹ÎË, ·fi fiÔ˘ ÍÂÎÈÓÔ‡Ó Ó¢ÚÈΤ˜ ›Ó˜, ÔÈ Ôԛ˜ ÂÎÙfi˜ ·fi ÙË ÏÂÈÙÔ˘ÚÁ›· Ù˘ ·Ó·ÛÙÔÏ‹˜ ÙˆÓ ÂÚÂıÈÛÌ¿ÙˆÓ fiÓÔ˘ Ô˘ ÂÈÛ¤Ú¯ÔÓÙ·È ÛÙÔÓ ÓˆÙÈ·›Ô Ì˘ÂÏfi, ÂÈÙÂÏÔ‡Ó Î·È ·˘Ù‹ Ù˘ ¤ÎÎÚÈÛ˘ ÛÂÚÔÙÔÓ›Ó˘. ∏ ÛÂÚÔÙÔÓ›ÓË ·ÔÙÂÏ› ÙËÓ Î˘ÚÈfiÙÂÚË ·fi ÙȘ Ó¢Úԉȷ‚È‚·ÛÙÈΤ˜ Ô˘Û›Â˜ Ô˘ ¤¯Ô˘Ó Û¯¤ÛË Ì ÙËÓ ÚfiÎÏËÛË ÙÔ˘ ‡ÓÔ˘ (¯ÔÚ‹ÁËÛË ·Ó·ÛÙÔϤˆÓ Ù˘ Û ÂÈÚ·Ì·Ùfi˙ˆ· Û˘Óԉ‡ÂÙ·È ·fi ·¸Ó›· Ô˘ ‰È·ÚΛ Ë̤Ú˜) Î·È Ù· Â›‰¿ Ù˘ ¤¯Ô˘Ó ‰È·ÈÛÙˆı› ÂÏ·Ùو̤ӷ Û ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›·. ¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô ·ÛıÂÓ‹˜ ‰ÂÓ ·Ó·Ê¤ÚÂÈ

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∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ·

‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘, ÚÔÛÂÎÙÈ΋ ÚÔÛ¤ÁÁÈÛË ·ÔηχÙÂÈ fiÙÈ Ô ‡ÓÔ˜ ‰ÂÓ ÙÔÓ ÍÂÎÔ˘Ú¿˙ÂÈ (Ê·ÈÓfiÌÂÓÔ ÚˆÈÓ‹˜ ÎfiˆÛ˘), ÂÓÒ Ì·ÚÙ˘Ú›Â˜ ·fi ¿ÙÔÌ· Ô˘ ÎÔÈÌÔ‡ÓÙ·È ÏËÛ›ÔÓ ·Ó·Ê¤ÚÔ˘Ó fiÙÈ Ô ·ÛıÂÓ‹˜ “ÛÙÚÈÊÔÁ˘Ú›˙ÂÈ Û˘Ó¯Ҙ ÛÙÔ ÎÚ‚¿ÙÈ ÙÔ˘” (Û‡Ó‰ÚÔÌÔ ·Ó‹Û˘¯ˆÓ οو ¿ÎÚˆÓ - restless legs syndrome). ™ËÌ·ÓÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ ·Ú·ÙËÚÔ‡ÓÙ·È ÛÙÔ ÛÙ¿‰ÈÔ 4 ÙÔ˘ ‡ÓÔ˘ ‚Ú·‰¤ˆÓ Î˘Ì¿ÙˆÓ (‹ ÌË-REM ‡ÓÔ˘), ηıÒ˜ ÛÙÔ ∏∂° ‰È·ÈÛÙÒÓÂÙ·È fiÙÈ ÛÙ· Ê˘ÛÈÔÏÔÁÈο ·̷ٷ ‰ (·̷ٷ ¯·ÌËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ 2-3 ‹ Î·È 1 c/sec) ·ÚÂÌ‚¿ÏÏÔÓÙ·È Î‡Ì·Ù· · (Ú˘ıÌÈο ·̷ٷ Û˘¯ÓfiÙËÙ·˜ 8-13 c/sec Ô˘ Ê˘ÛÈÔÏÔÁÈο ¢ڛÛÎÔÓÙ·È Û ÂÁÚ‹ÁÔÚÛË Î·È Û ηٿÛÙ·ÛË ‹ÚÂÌ˘ ÂÁÎÂÊ·ÏÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜). ¶·Ú’ fiÏ· ·˘Ù¿, Ô Ù‡Ô˘ ·-‰ ‡ÓÔ˜ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÂÈ ˘„ËÏ‹ ÂȉÈÎfiÙËÙ· ÁÈ· ÙËÓ ÈÓÔÌ˘·ÏÁ›· (28-30).

æ˘¯ÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜ ∏ ηٿÛÙ·ÛË Ù˘ „˘¯È΋˜ ˘Á›·˜ ÙˆÓ ÓÔÛÔ‡ÓÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ ÙÔ˘˜ ¤¯ÂÈ ‰È·ÈÛÙˆı› fiÙÈ ‰È·‰Ú·Ì·Ù›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ. ∏ „˘¯ÔÏÔÁÈ΋ ÚÔÛ¤ÁÁÈÛË, ÙfiÛÔ ÙÔ˘ ·È‰ÈÔ‡ fiÛÔ Î·È Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ (Ë ÔÔ›· Û˘¯Ó¿ ÓÔÛ›), ·ÔÎÙ¿ ÎÂÊ·Ï·ÈÒ‰Ë ÛËÌ·Û›·. ∏ Û˘ÌÂÚÈÊÔÚ¿ Ù˘ ‰˘¿‰·˜ ÌËÙ¤Ú·˜-¿Û¯Ô˘Û·˜ ÎfiÚ˘ ÂÚÈÁÚ¿ÊÂÙ·È ˆ˜ ¯·Ú·ÎÙËÚÈÛÙÈ΋: Ë ˘ÂÚÚÔÛٷ٢ÙÈ΋ ÌËÙ¤Ú· “ÂÚÈ‚¿ÏÏÂÈ” Û˘Ó·ÈÛıËÌ·ÙÈο ÙËÓ ¿Û¯Ô˘Û· ÎfiÚË, ··ÓÙÒÓÙ·˜ Û˘¯Ó¿ ·ÓÙ’ ·˘Ù‹˜ Û ÂÚˆÙ‹ÛÂȘ Ô˘ ·¢ı‡ÓÔÓÙ·È ÛÙËÓ ÙÂÏÂ˘Ù·›· Î·È ·ÊÔÚÔ‡Ó ÛÂ Û˘Ó·ÈÛı‹Ì·Ù· ‹ ‚Ȉ̷ÙÈΤ˜ ÂÌÂÈڛ˜. ∆· ¿Û¯ÔÓÙ· ·È‰È¿ ·ÔÙÂÏÔ‡Ó Û˘Ó‹ıˆ˜ ¿ÙÔÌ· ȉȷ›ÙÂÚ· ·Ô‰ÔÙÈο, ÙfiÛÔ ÛÙÔ Û¯ÔÏÈÎfi ÂÚÈ‚¿ÏÏÔÓ fiÛÔ Î·È Û Â͈ۯÔÏÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜, Ù· ÔÔ›· ÂÈ˙ËÙÔ‡Ó ÌÂıÔ‰Èο - Î·È Û˘¯Ó¿ ÂÈÙ˘Á¯¿ÓÔ˘Ó - ÙÔÓ ¤·ÈÓÔ ÙˆÓ ÁÔÓ¤ˆÓ (31). ∞ÔÙÂÏ› ¢ڤˆ˜ ‰ڷȈ̤ÓË ÂÔ›ıËÛË fiÙÈ Ù· „˘¯È·ÙÚÈο ÓÔÛ‹Ì·Ù· ÂȉÚÔ‡Ó ÂÓÈÛ¯˘ÙÈο ÛÙËÓ ÚfiÛÏË„Ë Î·È ·ÓÙ›ÏË„Ë ÙˆÓ ÂÚÂıÈÛÌ¿ÙˆÓ fiÓÔ˘ ·fi ÙÔ ¿ÙÔÌÔ, ÛÙÔÈ¯Â›Ô Ô˘ Ô‰ËÁ› ÔÏÏÔ‡˜ Â·ÁÁÂÏ̷ٛ˜ ˘Á›·˜ Ó· ÚÔÛÂÁÁ›˙Ô˘Ó ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›· ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·˜ ÚˆÙ·Ú¯Èο ÙË Û˘¯Ó¿ Û˘Ó˘¿Ú¯Ô˘Û· ηٿıÏÈ„Ë, ıˆÚÒÓÙ·˜ ·˘Ù‹ ˆ˜ ÙËÓ ÚˆÙÔ·ı‹ ·ÈÙ›·. øÛÙfiÛÔ, Ë ÈÓÔÌ˘·ÏÁ›· ‰ÂÓ ·ÔÙÂÏ› „˘¯È·ÙÚÈ΋ ÔÓÙfiÙËÙ·. ∞Ó Î·È Ë Û˘Ó‡·ÚÍË ·˘Ù‹ Â›Ó·È Û˘¯Ó‹ Û ÂÓ‹ÏÈΘ, ·Ú·Ì¤ÓÂÈ Ì¤¯ÚÈ Û‹ÌÂÚ· ·‰È¢ÎÚ›ÓÈÛÙÔ Â¿Ó ·˘Ù‹ Ë Û˘Ó‡·ÚÍË ÔÊ›ÏÂÙ·È Û ·ÈÙÈÔ·ıÔÁÂÓÂÙÈ΋ Û˘Û¯¤ÙÈÛË ‹ ·Ï¿ ÂÈÛ˘Ì‚·›ÓÂÈ ÛÙÔ Ï·›ÛÈÔ ÙˆÓ ‰Â˘ÙÂÚÔ·ıÒÓ ÂΉËÏÒÛÂˆÓ Ù˘ ·Ú¯È΋˜ ÓfiÛÔ˘. ∂È-

ϤÔÓ, ‰ÂÓ ˘¿Ú¯Ô˘Ó ÌÂϤÙ˜ Û ·È‰È¿ Ô˘ Ó· Û˘Û¯ÂÙ›˙Ô˘Ó ÙËÓ ÈÓÔÌ˘·ÏÁ›· Ì ÙËÓ Î·Ù¿ıÏÈ„Ë. ¶ÚÔηٷÚÎÙÈο ÛÙÔȯ›· ·fi ÏÂÈÙÔ˘ÚÁÈΤ˜ ÌÂϤÙ˜ Ù˘ ÂÁÎÂÊ·ÏÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜ ̤ۈ ·ÂÈÎfiÓÈÛ˘ Ì ̷ÁÓËÙÈÎfi Û˘ÓÙÔÓÈÛÌfi, ηٷ‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Ë Î·Ù¿ıÏÈ„Ë Î·È Ë ‰ÈÂÚÁ·Û›· Ù˘ ÚfiÛÏ˄˘ Î·È ·ÓÙ›Ï˄˘ ÙÔ˘ fiÓÔ˘ ·ÔÙÂÏÔ‡Ó ‰È·‰Èηۛ˜ ‰È·ÊÔÚÂÙÈΤ˜ Î·È ·ÓÂÍ¿ÚÙËÙ˜ (32). ŒÙÛÈ, ·ÛıÂÓ›˜ ¿Û¯ÔÓÙ˜ ·fi ¯ÚfiÓÈÔ ¿ÏÁÔ˜ Î·È Î·Ù¿ıÏÈ„Ë ‰ÂÓ ‰È·Ê¤ÚÔ˘Ó fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÚfiÛÏË„Ë Î·È ÙËÓ ·ÓÙ›ÏË„Ë ÙÔ˘ fiÓÔ˘ ·fi ÂΛÓÔ˘˜ Ì ¯ÚfiÓÈÔ ¿ÏÁÔ˜, ·ÏÏ¿ ¯ˆÚ›˜ ηٿıÏÈ„Ë. ∆¤ÏÔ˜, Ë ‡·ÚÍË ÂÓfi˜ ÔÏ˘·Ú·ÁÔÓÙÈÎÔ‡ ·ÈÙÈÔ·ıÔÁÂÓÂÙÈÎÔ‡ ÌÔÓÙ¤ÏÔ˘, ÌÂ Û˘ÌÌÂÙÔ¯‹ ÙfiÛÔ ÂÓ‰ÔÁÂÓÒÓ (¯·ÌËÏfi˜ Ô˘‰fi˜ fiÓÔ˘, ı‹Ï˘ ʇÏÔ, ˘ÂÚÎÈÓËÙÈÎfiÙËÙ·) fiÛÔ Î·È Â͈ÁÂÓÒÓ ÌÂÙ·‚ÏËÙÒÓ (ÚÔËÁËı›Û˜ ÂÌÂÈڛ˜ fiÓÔ˘, ÂÏÏÂÈÌÌ·ÙÈ΋ ÎÔÈÓˆÓÈ΋ ·ÏÏËÏÂ›‰Ú·ÛË, Ê˘ÛÈ΋ ‹ ÛÂÍÔ˘·ÏÈ΋ ηÎÔÔ›ËÛË, ÚfiÙ˘· ÁÔÓÈ΋˜ Û˘ÌÂÚÈÊÔÚ¿˜ ·¤Ó·ÓÙÈ ÛÙÔÓ fiÓÔ, η΋ Ê˘ÛÈ΋ ηٿÛÙ·ÛË), ıˆÚÂ›Ù·È Èı·Ó‹. ∫·ıÒ˜ ÔÈ ·Ú·¿Óˆ ·Ú¿ÁÔÓÙ˜ ÌÔÚ› Ó· ‰ÚÔ˘Ó ÌÂÌÔӈ̤ӷ ‹ Î·È ÛÂ Û˘Ó‰˘·ÛÌfi, ÙÔ ¿ÙÔÌÔ ÌÔÚ› Ó· “‚ÈÒÓÂÈ” ÙËÓ ÂÌÂÈÚ›· ÙÔ˘ fiÓÔ˘ Ì ÌÔÓÔÛ‹Ì·ÓÙÔ ‹ ÌË ÙÚfiÔ. °È· ·Ú¿‰ÂÈÁÌ·, Ë ÂÌÊ¿ÓÈÛË fiÓÔ˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ‰È·Ù·Ú·¯‹ ÙÔ˘ ‡ÓÔ˘, Ë ÔÔ›· fi¯È ÌfiÓÔ ÂȉÂÈÓÒÓÂÈ ÂÚ·ÈÙ¤Úˆ ÙÔÓ fiÓÔ, ·ÏÏ¿ ÂÈϤÔÓ ÚÔηÏ› ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘ ÔÈÎÔÁÂÓÂȷ΋˜ ˙ˆ‹˜ (33,34).

∫ÏÈÓÈÎÔ› ¯·Ú·ÎÙ‹Ú˜ - ¢È¿ÁÓˆÛË ∆Ô ÁÂÁÔÓfi˜ fiÙÈ Ù· ·È‰È¿ Ì ÂÒ‰˘Ó· Ì˘ÔÛÎÂÏÂÙÈο Û‡Ó‰ÚÔÌ·, ÛÙËÓ ÏÂÈÔÓfiÙËÙ¿ ÙÔ˘˜, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ¯·ÚÔ‡ÌÂÓ·, ‹ÚÂÌ·, ·ÎfiÌ· Î·È ¯·ÌÔÁÂÏÒÓÙ·˜, ÂÓÒ Ù·˘Ùfi¯ÚÔÓ· ·Ó·Ê¤ÚÔ˘Ó ÛËÌ·ÓÙÈ΋˜ ¤ÓÙ·Û˘ fiÓÔ, ÚÔηÏ› ÂÓÙ‡ˆÛË. ∏ ¯·Ú·ÎÙËÚÈÛÙÈ΋ ·˘Ù‹ ·‰È·ÊÔÚ›· ÁÈ· ÙËÓ Î·Ù¿ÛÙ·Û‹ ÙÔ˘˜, ηÏÂ›Ù·È “la belle indifference” (“ˆÚ·›· ·‰È·ÊÔÚ›·”). ∆· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ ÂÌÊ·Ó›˙Ô˘Ó Î·È ·Ïψ‰˘Ó›·, Ë ÔÔ›· ‰È·ÈÛÙÒÓÂÙ·È ÌÂÙ¿ ·fi ··Ïfi ¿ÁÁÈÁÌ· ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ ‹ ‹È· ¤ÏÍË ‰ÂÚÌ·ÙÈ΋˜ Ù˘¯‹˜. ∏ ·‰˘Ó·Ì›· ÂÚÁ·ÛÙËÚȷ΋˜ ‹ ÈÛÙÔÏÔÁÈ΋˜ ÂȂ‚·›ˆÛ˘ Ù˘ ‰È¿ÁÓˆÛ˘ Ô‰‹ÁËÛ ÛÙË ı¤ÛÈÛË ÎÏÈÓÈÎÒÓ ‰È·ÁÓˆÛÙÈÎÒÓ ÎÚÈÙËÚ›ˆÓ ·fi ÙÔ ∞ÌÂÚÈηÓÈÎfi ∫ÔϤÁÈÔ ƒÂ˘Ì·ÙÔÏÔÁ›·˜, ÙÔ 1990, Ì ‚¿ÛË Ù· ·ÔÙÂϤÛÌ·Ù· ¢Ú›·˜ ÔÏ˘ÎÂÓÙÚÈ΋˜ ÌÂϤÙ˘ (1). ŒÙÛÈ, Û ÂÓ‹ÏÈΘ, Ë ‰È¿ÁÓˆÛË ÙÂÎÌËÚÈÒÓÂÙ·È Ì ÙËÓ ·ÚÔ˘Û›· ‰‡Ô ÌÂÈ˙fiÓˆÓ ¶·È‰È·ÙÚÈ΋ 2005;68:342-350

Pediatr Sept-Oct 05

17-10-05

16:51

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µ. ∆Û·ÁÚ‹˜ Î·È Û˘Ó.

ÎÚÈÙËÚ›ˆÓ: ·) ÙËÓ ‡·ÚÍË ‰È¿¯˘ÙÔ˘ fiÓÔ˘ ÁÈ· ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 3 ÌËÓÒÓ, Ô ÔÔ›Ô˜ ·ÊÔÚ¿ Î·È ÛÙ· ‰‡Ô ËÌÈÌfiÚÈ· ÙÔ˘ ÛÒÌ·ÙÔ˜, ¿Óˆ Î·È Î¿Ùˆ ·fi ÙË Ì¤ÛË Î·È ‚) ÙËÓ ‡·ÚÍË Î·Ù’ ÂÏ¿¯ÈÛÙÔ 11 ¢·›ÛıËÙˆÓ ÛËÌ›ˆÓ (·fi Û‡ÓÔÏÔ 18 ÚÔηıÔÚÈṲ̂ӈÓ) ηٿ ÙËÓ ÚÒÙË ÎÏÈÓÈ΋ ÂͤٷÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ (∂ÈÎfiÓ· 1 Î·È ¶›Ó·Î·˜ 1). O ¤ÏÂÁ¯Ô˜ ‰ÈÂÓÂÚÁÂ›Ù·È Ì ÙËÓ ÂÊ·ÚÌÔÁ‹ ›ÂÛ˘ ÈÛÔ‰‡Ó·Ì˘ Ì 4 kg ·fi ÙÔÓ ÂÍÂÙ·ÛÙ‹ ÛÙ· ¢·›ÛıËÙ· ÛËÌ›· ÙÔ˘ ·ÛıÂÓÔ‡˜, Ì ÙË ‚Ô‹ıÂÈ· ÂȉÈÎÔ‡ ‰È·ÁÓˆÛÙÈÎÔ‡ ÂÚÁ·Ï›Ԣ (·ÏÁËÛ›ÌÂÙÚÔ), ·Ó Î·È ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ Û˘ÁÁÚ·Ê›˜ Û˘ÌʈÓÔ‡Ó ÛÙÔ fiÙÈ ÂÍ›ÛÔ˘ ·ÍÈfiÈÛÙ· ·ÔÙÂϤÛÌ·Ù· Ï·Ì‚¿ÓÔÓÙ·È Î·È Ì ÙË ¯Ú‹ÛË ÙÔ˘ ·ÓÙ›¯ÂÈÚ· (Ú·ÎÙÈο ı· Ú¤ÂÈ Ó· ·ÛÚ›ÛÂÈ Ë ÔÓ˘¯ÔÊfiÚÔ˜ Ê¿Ï·ÁÁ· ÙÔ˘ ·ÓÙ›¯ÂÈÚ· ÙÔ˘ ÂÍÂÙ·ÛÙ‹). °È· ÙËÓ ÂÍ¿ÏÂÈ„Ë ÙÔ˘ ÛÙÔÈ¯Â›Ô˘ Ù˘ ˘ÔÎÂÈÌÂÓÈÎfiÙËÙ·˜, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ˆ˜ Ì¿ÚÙ˘Ú˜ 10 ÛËÌ›· (̤وÔ, ‰ÂÍÈfi Î·È ·ÚÈÛÙÂÚfi

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Ï¿ÁÈÔ, Ú·¯È·›· ÂÈÊ¿ÓÂÈ· ÂÚÈÊÂÚÈÎÔ‡ ÙÌ‹Ì·ÙÔ˜ ·ÓÙÈ‚Ú·¯›Ô˘ ·ÌÊÔÙÂÚfiÏ¢ڷ, ÔÓ˘¯ÔÊfiÚÔ˜ Ê¿Ï·ÁÁ· ÙˆÓ ·ÓÙȯ›ڈÓ, Ú·¯È·›· ÂÈÊ¿ÓÂÈ· ÙÔ˘ ÙÚ›ÙÔ˘ ÌÂٷٷÚÛ›Ô˘ ·ÌÊÔÙÂÚfiÏ¢ڷ, ÔÓ˘¯ÔÊfiÚÔ˜ Ê¿Ï·ÁÁ· 1Ô˘ ‰·ÎÙ‡ÏÔ˘ Ô‰ÈÔ‡ ·ÌÊÔÙÂÚfiÏ¢ڷ). ∏ ÙÂÎÌËÚ›ˆÛË Ù˘ ‰È¿ÁÓˆÛ˘ ıˆÚÂ›Ù·È ÂÈÛÊ·Ï‹˜ fiÙ·Ó ‰È·ÈÛÙÒÓÂÙ·È Â˘·ÈÛıËÛ›· Û ÂÚÈÛÛfiÙÂÚ· ·fi 3 ÛËÌ›·-Ì¿ÚÙ˘Ú˜. ™ËÌÂÈÒÓÂÙ·È Ë ·Ó¿ÁÎË ‰È¿ÎÚÈÛ˘ ÙˆÓ “¢·›ÛıËÙˆÓ” (tender) ·fi Ù· “˘ÚÔ‰ÔÙÔ‡ÓÙ·” (trigger) ÛËÌ›·, Ô˘ Â›Û˘ ·Ú·ÙËÚÔ‡ÓÙ·È ÛÙËÓ ÈÓÔÌ˘·ÏÁ›· Î·È Ù· ÔÔ›· ·ÓÙÈÛÙÔÈ¯Ô‡Ó Û ¢·›ÛıËÙ˜ ÂÚÈÔ¯¤˜ Ì˘ÒÓ, Ë ‰È¤ÁÂÚÛË ÙˆÓ ÔÔ›ˆÓ Ô‰ËÁ› Û ·ÓÙ·Ó¿ÎÏ·ÛË ÙÔ˘ fiÓÔ˘ Î·È ÛÙË ¯·Ú·ÎÙËÚÈÛÙÈ΋ Û‡Û·ÛË ÙÔ˘ Ì˘fi˜. ∆· ÛËÌ›· ·˘Ù¿, ˆÛÙfiÛÔ, ‰ÂÓ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙË ‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË, ηıÒ˜ ηٿ ÙËÓ ·ÍÈÔÏfiÁËÛ‹ ÙÔ˘˜ ˘ÂÈÛ¤Ú¯ÂÙ·È Û ÛËÌ·ÓÙÈÎfi ‚·ıÌfi Ô ·Ú¿ÁÔÓÙ·˜ Ù˘ ˘ÔÎÂÈÌÂÓÈÎfiÙËÙ·˜. ∞ÚÎÂÙÔ›

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∂ÈÎfiÓ· 1, ¶›Ó·Î·˜ 1. ∆· 18 ¢·›ÛıËÙ· ÛËÌ›· Ù˘ ÈÓÔÌ˘·ÏÁ›·˜ ηٿ ÙÔ ∞ÌÂÚÈηÓÈÎfi ∫ÔϤÁÈÔ ƒÂ˘Ì·ÙÔÏÔÁ›·˜, 1990 (¶ËÁ‹: National Fibromyalgia Partnership, Inc.) Î·È ÚÔÛ·ÚÌÔÁ‹ ÙÔ˘˜ ÛÙÔ ÎÏ·ÛÈÎfi ¤ÚÁÔ ÙÔ˘ ƒÔ‡ÌÂÓ˜ “∆ÚÂȘ ÿÚÈÙ˜” (ªÔ˘ÛÂ›Ô Prado, ª·‰Ú›ÙË. ÀÏÔÔ›ËÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ‚·ÛÈṲ̂ÓË Û ȉ¤· ‰ËÌÔÛÈ¢ı›۷ ÛÙÔ Arthritis and Rheumatism Journal, 1990) 1,2. πÓȷ΋ ÂÚÈÔ¯‹ 3,4. ∫·ÙÒÙÂÚÔ ÙÌ‹Ì· ·˘¯¤Ó· 5,6. ∆Ú·Â˙ÔÂȉ‹˜ Ì˘˜ 7,8. ÀÂڷοÓıÈÔ˜ Ì˘˜ 9,10. 2Ë ÏÂ˘Ú¿ 11,12. ŒÍˆ ÂÈÎfiÓ‰˘ÏÔ˜ 13,14. °ÏÔ˘ÙÔ› 15,16. ªÂ›˙ˆÓ ÙÚÔ¯·ÓÙ‹Ú·˜ 17,18. °fiÓ·ÙÔ

¶·È‰È·ÙÚÈ΋ 2005;68:342-350

∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙȘ ÚÔÛʇÛÂȘ ÙˆÓ ˘ÈÓÈ·ÎÒÓ Ì˘ÒÓ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙËÓ ÂÚÈÔ¯‹ ÙˆÓ ÂÁοÚÛÈˆÓ ·ÔʇÛÂˆÓ ÛÙÔ ‰È¿ÛÙËÌ· ∞5-∞7 ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙË ÌÂÛfiÙËÙ· ÙÔ˘ ·ÓÒÙÂÚÔ˘ ¯Â›ÏÔ˘˜ ÙÔ˘ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙËÓ ¤ÎÊ˘Û‹ ÙÔ˘, ÏËÛ›ÔÓ ÙÔ˘ ¤Ûˆ ¯Â›ÏÔ˘˜ Ù˘ ˆÌÔÏ·ÙÈ·›·˜ ¿Î·Óı·˜ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙË ÛÙÂÚÓÔÏ¢ÚÈ΋ Û˘Á¯fiÓ‰ÚˆÛË Ù˘ 2˘ ÏÂ˘Ú¿˜ ∞ÌÊÔÙÂÚfiÏ¢ڷ, 2 cm ÂÚÈÊÂÚÈÎfiÙÂÚ· ÙˆÓ ¤Íˆ ÂÈÎÔÓ‰‡ÏˆÓ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙÔ ¿Óˆ-¤Íˆ ÙÂÙ·ÚÙËÌfiÚÈfi ÙÔ˘˜, ηٿ ÙÔ ÚfiÛıÈÔ ¯Â›ÏÔ˜ ÙÔ˘ Ì›˙ÔÓÔ˜ ÁÏÔ˘ÙÈ·›Ô˘ Ì˘fi˜ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÂÏ¿¯ÈÛÙ· ÔÈÛı›ˆ˜ ÙÔ˘ Ì›˙ÔÓÔ˜ ÙÚÔ¯·ÓÙËÚÈÎÔ‡ Â¿ÚÌ·ÙÔ˜ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÏÈÒ‰Ô˘˜ ÛÒÌ·ÙÔ˜, ÏËÛ›ÔÓ Ù˘ ¤Ûˆ ·ÚıÚÈ΋˜ ÁÚ·ÌÌ‹˜

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·347

347

∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ·

Û˘ÁÁÚ·Ê›˜ Û˘ÓËÁÔÚÔ‡Ó ˘¤Ú Ù˘ ¿Ԅ˘ fiÙÈ ÛÙ· ·È‰È¿ Ë ‰È¿ÁÓˆÛË ÌÔÚ› Ó· ÙÂÎÌËÚȈı› ·ÎfiÌ· Î·È fiÙ·Ó ÙÔ ¿ıÚÔÈÛÌ· ÙˆÓ Â˘·›ÛıËÙˆÓ ÛËÌ›ˆÓ ÛÙÔ Û‡ÓÔÏÔ ÙˆÓ ÂÈÛΤ„ÂˆÓ ÛÙÔÓ ·È‰›·ÙÚÔ Â›Ó·È ÌÈÎÚfiÙÂÚÔ ÙÔ˘ 11 (≥5 ηٿ ÙÔ˘˜ Yunus Î·È Masi), Û ·ÓÙȉȷÛÙÔÏ‹ Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ, fiÔ˘ ··ÈÙÂ›Ù·È Î·Ù¿‰ÂÈÍË ÙÔ˘Ï¿¯ÈÛÙÔÓ 11 ÛËÌ›ˆÓ ηٿ ÙËÓ ÚÒÙË ÌfiÓÔ Â›ÛÎÂ„Ë (35). ∞˘Ùfi ÚÔÙ›ÓÂÙ·È ÏfiÁˆ Ù˘ ·ÙÂÏÔ‡˜ ¤ÎÊÚ·Û˘ Ù˘ ÓfiÛÔ˘ ÛÙ· ·È‰È¿, ηıÒ˜ Î·È Ù˘ ÈηÓÔÔÈËÙÈ΋˜ ·ÓÙ·fiÎÚÈÛ‹˜ ÙÔ˘˜ ÛÙË ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË, ˆÛÙfiÛÔ ‰ÂÓ Â›Ó·È Â·ÎÚÈ‚Ò˜ ÁÓˆÛÙfi Â¿Ó ÔÈ ·Ú·¿Óˆ ·ÈÙÈ¿ÛÂȘ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ· ‹ ·ÏÒ˜ ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ÌÂıÔ‰ÔÏÔÁÈΤ˜ ·‰˘Ó·Ì›Â˜ ÙˆÓ ÌÂÏÂÙÒÓ. ∆· Û˘ÓÔ‰¿ Û˘ÌÙÒÌ·Ù·, Û‡Ìʈӷ Ì ÙË ÌÂϤÙË ÙˆÓ Siegel Î·È Û˘Ó, ÙÔ 1998, Û 45 ·È‰È¿ Ì ÈÓÔÌ˘·ÏÁ›· Î·È ÙˆÓ Gedalia Î·È Û˘Ó, ÙÔ 2000, Û 59 ·È‰È¿ ·ÓÙ›ÛÙÔȯ·, ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 2 (8,9). ¶ÚÔηÏ› ÂӉȷʤÚÔÓ Ë ·Ú·ÙËÚÔ‡ÌÂÓË Û˘Û¯¤ÙÈÛË ÌÂٷ͇ Ù˘ ÈÓÔÌ˘·ÏÁ›·˜ Î·È ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ˘ÂÚÎÈÓËÙÈÎfiÙËÙ·˜, ηıÒ˜ Û ÛËÌ·ÓÙÈÎfi ·ÚÈıÌfi ·È‰ÈÒÓ Ì ÈÓÔÌ˘·ÏÁ›· ‰È·ÈÛÙÒÓÂÙ·È fiÙÈ ÏËÚÔ‡ÓÙ·È Î·È Ù· ÎÚÈÙ‹ÚÈ· ÁÈ· ÙË ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ˘ÂÚÎÈÓËÙÈÎfiÙËÙ·˜. ∞˘Ù‹ Ë ·ÏÏËÏÔÂÈÎ¿Ï˘„Ë Â›Ó·È Èı·Ófi Ó· ÔÊ›ÏÂÙ·È Û ·ÚÔ˘Û›· ÂÓfi˜ ÎÔÈÓÔ‡ ·ÈÙÈÔÏÔÁÈÎÔ‡ ˘Ô‚¿ıÚÔ˘. ∫·ıÒ˜, ˆÛÙfiÛÔ, ÔÈ ‰‡Ô ÎÏÈÓÈΤ˜ ÔÓÙfiÙËÙ˜ ÌÔÈ¿˙Ô˘Ó Û ·ÚÎÂÙÔ‡˜ ÎÏÈÓÈÎÔ‡˜ ¯·Ú·ÎÙ‹Ú˜ Î·È ‰È·Ê¤ÚÔ˘Ó ÌfiÓÔ fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ·ÚÔ˘Û›· ¢·›ÛıËÙˆÓ ÛËÌ›ˆÓ (·Ô˘ÛÈ¿˙Ô˘Ó ÛÙÔ Û‡Ó‰ÚÔÌÔ

˘ÂÚÎÈÓËÙÈÎfiÙËÙ·˜), Ë ·ÏÏËÏÔÂÈÎ¿Ï˘„Ë ·˘Ù‹ ı· ÌÔÚÔ‡Û ӷ ·Ô‰Ôı› ÛÙËÓ ·ÓÂ·Ú΋ ·ÍÈÔÏfiÁËÛË ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ ·fi ·ÚÎÂÙÔ‡˜ Û˘ÁÁÚ·Ê›˜.

¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ƒÂ˘Ì·ÙÈÎfi ÓfiÛËÌ·, ȉȷ›ÙÂÚ· ÚÂ˘Ì·ÙÔÂȉ‹˜ ·ÚıÚ›Ùȉ· ‹ Û˘ÛÙËÌ·ÙÈÎfi˜ ÂÚ˘ıËÌ·Ù҉˘ χÎÔ˜, Û˘Ó˘¿Ú¯ÂÈ Û ÔÛÔÛÙfi 6% ÙˆÓ ÂÓËÏ›ÎˆÓ Ì ÈÓÔÌ˘·ÏÁ›·. ∏ ÚÂ˘Ì·ÙÈ΋ ÔÏ˘Ì˘·ÏÁ›· Î·È ÔÈ ·ÁÁÂÈ›Ùȉ˜ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó Ì ÎÏÈÓÈ΋ ÂÈÎfiÓ· ·ÚfiÌÔÈ· Ì ÂΛÓË Ù˘ ÈÓÔÌ˘·ÏÁ›·˜, ·ÏÏ¿ ÙÔ ¿ıÚÔÈÛÌ· ÙˆÓ Â˘·›ÛıËÙˆÓ ÛËÌ›ˆÓ Î·È ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ ÔÙ¤ ‰ÂÓ ÚÔÛÂÁÁ›˙ÂÈ ·˘Ùfi Ù˘ ÈÓÔÌ˘·ÏÁ›·˜, ÂÎÙfi˜ Â¿Ó Ë ÙÂÏÂ˘Ù·›· Û˘Ó˘¿Ú¯ÂÈ ˆ˜ ͯˆÚÈÛÙ‹ ÔÓÙfiÙËÙ·. ∂ÈϤÔÓ, Ë ÚÂ˘Ì·ÙÈ΋ ÔÏ˘Ì˘·ÏÁ›· Â›Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈ· ÛÙ· ·È‰È¿. ™ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ë Û·ÚÎÔ›‰ˆÛË, ÔÈ Û˘ÛÙËÌ·ÙÈΤ˜ ÏÔÈÌÒÍÂȘ, Ù· ·Ú·ÓÂÔÏ·ÛÌ·ÙÈο Û‡Ó‰ÚÔÌ·, ÙÔ Û‡Ó‰ÚÔÌÔ ˘ÂÚÎÈÓËÙÈÎfiÙËÙ·˜, Ë ÚÒÈÌË ÛÔÓ‰˘ÏÔ·ÚıÚÔ¿ıÂÈ·, ÙÔ Û‡Ó‰ÚÔÌÔ ÙÔ˘ ÚfiÛıÈÔ˘ ıˆÚ·ÎÈÎÔ‡ ÙÔȯÒÌ·ÙÔ˜, Ë ·ÓÙ·Ó·ÎÏ·ÛÙÈ΋ Û˘Ì·ıËÙÈ΋ ‰˘ÛÙÚÔÊ›· (‹ ÂÓÙÔÈṲ̂ÓÔ ÂÚÈÔ¯ÈÎfi Û‡Ó‰ÚÔÌÔ fiÓÔ˘), Ë ÂÚÈÊÂÚÈ΋ ÌÔÓÔÓ¢ÚÔ¿ıÂÈ·, Ë ‰˘Û·˘ÙÔÓÔÌ›·, Ô ˘Ô- ‹ ˘ÂÚı˘ÚÂÔÂȉÈÛÌfi˜, Ô ˘ÂÚ·Ú·ı˘ÚÂÔÂȉÈÛÌfi˜ Î·È Ù· ÊÏÂÁÌÔÓÒ‰Ë ÓÔÛ‹Ì·Ù· ÙÔ˘ ÂÓÙ¤ÚÔ˘. ¶˘ÚÂÙfi˜, Ó˘ÎÙÂÚÈÓfi˜ ȉÚÒÙ·˜, ·ÒÏÂÈ· ‚¿ÚÔ˘˜, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ÔÚÁ·ÓÔÌÂÁ·Ï›·, Ê·ÈÓfiÌÂÓÔ

¶›Ó·Î·˜ 2. ∫ÏÈÓÈο Â˘Ú‹Ì·Ù· Û ·È‰È¿ Ì ÈÓÔÌ˘·ÏÁ›· ηٿ Siegel Î·È Gedalia (8,9) ∫ÏÈÓÈο Â˘Ú‹Ì·Ù· Û ·È‰È¿ Ì ÈÓÔÌ˘·ÏÁ›· ¢È·Ù·Ú·¯¤˜ ‡ÓÔ˘ ∫ÂÊ·Ï·ÏÁ›· °ÂÓÈÎÂ˘Ì¤ÓË ÎfiˆÛË ¶ÚˆÈÓ‹ ‰˘Ûη̄›· ¶ÚˆÈÓ‹ ÎfiˆÛË ∫·Ù¿ıÏÈ„Ë ∂ȉ›ӈÛË ÌÂÙ¿ ·fi ¿ÛÎËÛË O›‰ËÌ· ·ÚıÚÒÛÂˆÓ ∂˘ÂÚ¤ıÈÛÙÔ ¤ÓÙÂÚÔ ¢˘ÛÌËÓfiÚÚÔÈ· ªÂÙ·‚ÔϤ˜ Ù˘ ÓfiÛÔ˘ ·Ó¿ÏÔÁ· Ì ÙÔÓ Î·ÈÚfi ¶·Ú·ÈÛı‹ÛÂȘ ÕÁ¯Ô˜ “ ŒÏÏÂÈÌÌ· ÂÓ¤ÚÁÂÈ·˜” º·ÈÓfiÌÂÓÔ Raynaud ∫ÔÈÏÈ·Îfi ¿ÏÁÔ˜ ÀÂÚÎÈÓËÙÈÎfiÙËÙ· ·ÚıÚÒÛˆÓ

™˘¯ÓfiÙËÙ· % ηٿ Siegel Î·È Û˘Ó, 1998 (n=45)

™˘¯ÓfiÙËÙ· % ηٿ Gedalia Î·È Û˘Ó, 2000 (n=59)

96 71 62 53 49 43 42 40 38 36 36 24 22 18 13 40

70 76 20 30 7 24 17 14 ¶·È‰È·ÙÚÈ΋ 2005;68:342-350

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·348

348

µ. ∆Û·ÁÚ‹˜ Î·È Û˘Ó.

Raynaud, Ó¢ÚÔ¿ıÂÈ·, Ô›‰ËÌ· ‹ ÂÚÈÔÚÈÛÌfi˜ Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜ ÙˆÓ ·ÚıÚÒÛˆÓ, ηıÒ˜ Î·È ·ıÔÏÔÁÈο ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·, ·ÔÙÂÏÔ‡Ó ÛÙÔȯ›· Ô˘ ı· Ú¤ÂÈ Ó· ÛÙÚ¤ÊÔ˘Ó ÙË ‰È·ÁÓˆÛÙÈ΋ ÛΤ„Ë Û ÔÓÙfiÙËÙ˜ ÂÎÙfi˜ Ù˘ ÈÓÔÌ˘·ÏÁ›·˜. ™Â ·È‰È¿ ÂÊË‚È΋˜ ËÏÈΛ·˜ Ú¤ÂÈ, Â›Û˘, Ó· ·ÔÎÏ›ÂÙ·È Ë Â˘·ÈÛıËÛ›· Û ʿÚ̷η fiˆ˜ ‰ÈÔ˘ÚËÙÈο, ηʽÓË, ·ÏÎÔfiÏ ‹ ·ÌÊÂÙ·Ì›ÓË, Ô˘ ÌÔÚ› Ó· ÚÔ‚¿ÏÏÂÈ ÌÂ Û˘ÌÙÒÌ·Ù· ·ÚfiÌÔÈ· Ì ÂΛӷ Ù˘ ÈÓÔÌ˘·ÏÁ›·˜. ™ËÌÂÈÒÓÂÙ·È fiÙÈ Ë ÈÓÔÌ˘·ÏÁ›· ‰ÂÓ Â›Ó·È Û˘ÓÒÓ˘ÌË Ì ÙÔÓ „˘¯ÔÁÂÓ‹ ÚÂ˘Ì·ÙÈÛÌfi, ÛÙÔÓ ÔÔ›Ô Ô ·ÛıÂÓ‹˜ ·ÚÔ˘ÛÈ¿˙ÂÈ ÙË ¯·Ú·ÎÙËÚÈÛÙÈ΋ “ÌË ÌÔ˘ ¿ÙÔ˘” Û˘ÌÂÚÈÊÔÚ¿ Î·È ÂÌÊ·Ó›˙ÂÈ ıÂÙÈο ÙfiÛÔ Ù· ¢·›ÛıËÙ· fiÛÔ Î·È Ù· ÛËÌ›·-Ì¿ÚÙ˘Ú˜.

¶ÚfiÁÓˆÛË ¶ÔÏ˘¿ÚÈı̘ ÌÂϤÙ˜ Û ÂÓ‹ÏÈΘ ·fi ‰È¿ÊÔÚ˜ ¯ÒÚ˜, ηٷϋÁÔ˘Ó ÛÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ Û ÌÂÁ¿ÏÔ ÔÛÔÛÙfi ·ÛıÂÓÒÓ Ë ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ Â›Ó·È Ùˆ¯‹, Ì ٷ Û˘ÌÙÒÌ·Ù· Ó· ÂÈ̤ÓÔ˘Ó ‰È¿ ‚›Ô˘ (36,37). ∂Ï¿¯ÈÛÙ· Â›Ó·È ÁÓˆÛÙ¿ Û¯ÂÙÈο Ì ÙË Ê˘ÛÈ΋ ÈÛÙÔÚ›· Î·È ÙË Ì·ÎÚÔ¯ÚfiÓÈ· ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ Û ·È‰È¿, ηıÒ˜ ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ··ÓÙÒÓÙ·È ÙÚÂȘ Û˘ÓÔÏÈο ·Ó¿ÏÔÁ˜ ÌÂϤÙ˜, Ì ·ÓÙÈÎÚÔ˘fiÌÂÓ· ·ÔÙÂϤÛÌ·Ù· Ô˘ Èı·ÓfiÓ ÔÊ›ÏÔÓÙ·È Û ÌÂıÔ‰ÔÏÔÁÈΤ˜ ·‰˘Ó·Ì›Â˜. ¶·Ú·Ì¤ÓÂÈ, ˆÛÙfiÛÔ, ÈÛ¯˘Ú‹ Ë ·›ÛıËÛË fiÙÈ Ë ÚfiÁÓˆÛË Ù˘ Ó·ÓÈ΋˜ ÌÔÚÊ‹˜ Ù˘ ÓfiÛÔ˘ Â›Ó·È Â˘ÓÔ˚ÎfiÙÂÚË ·˘Ù‹˜ ÙˆÓ ÂÓËÏ›ÎˆÓ (38,39,4).

∞ÓÙÈÌÂÙÒÈÛË ªÂ ÙÔ ·ÈÙÈÔÏÔÁÈÎfi ˘fi‚·ıÚÔ Ù˘ ÓfiÛÔ˘ Ó· ‰È¢ڇÓÂÙ·È Û˘Ó¯Ҙ, Ë ÛÙÚ·ÙËÁÈ΋ ıÂÚ·¢ÙÈ΋˜ ·Ú¤Ì‚·Û˘ ÛÙËÓ ÈÓÔÌ˘·ÏÁ›· ·Ú·Ì¤ÓÂÈ ÔχÏ¢ÚË Î·È Ì ȉȷ›ÙÂÚ· ÂÎÙÂٷ̤ÓÔ Ê¿ÛÌ·. ∫·ıÒ˜ “Ì·ÁÈÎfi ¯¿È” ‰ÂÓ ˘¿Ú¯ÂÈ, ÚˆÙ·Ú¯ÈÎfi˜ ÛÙfi¯Ô˜ Â›Ó·È Ô ÂÚÈÔÚÈÛÌfi˜ ÛÙÔÓ Ì¤ÁÈÛÙÔ ‰˘Ó·Ùfi ‚·ıÌfi Ù˘ ¤ÎÊÚ·Û˘ ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Ù˘ ÓfiÛÔ˘. ∫·Ù’ ·Ó·ÏÔÁ›· Ì ÙË ‰È·›ÛÙˆÛË fiÙÈ “ηÓ›˜ ·ÛıÂÓ‹˜ ‰ÂÓ Â›Ó·È ›‰ÈÔ˜” fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÎÏÈÓÈ΋ ¤ÎÊÚ·ÛË Ù˘ ÓfiÛÔ˘, Ë ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË Ú¤ÂÈ Ó· ÂÍ·ÙÔÌÈ·ÂÙ·È, ·ÔÙÂÏÒÓÙ·˜ - Û οı ÂÚ›ÙˆÛË - ÚÔ˚fiÓ Ì›·˜ ÔÌ·‰È΋˜, ‰ÈÂÈÛÙËÌÔÓÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘. OÈ ‚·ÛÈΤ˜ ·Ú¯¤˜ ·˘Ù‹˜ Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ ·Ú·Ù›ıÂÓÙ·È ÛÙË Û˘Ó¤¯ÂÈ·. ¶·È‰È·ÙÚÈ΋ 2005;68:342-350

º·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ ∆· ÂÚÈÛÛfiÙÂÚ· ·fi Ù· ¯ÚËÛÈÌÔÔÈÔ‡ÌÂÓ· ÛÙËÓ ÈÓÔÌ˘·ÏÁ›· Û΢¿ÛÌ·Ù· Â›Ó·È “‰·ÓÂÈο” ·fi ¿ÏÏ· ÓÔÛ‹Ì·Ù·, ηıÒ˜ ηӤӷ Û··ÛÌ· ‰ÂÓ ¤¯ÂÈ ÂÁÎÚÈı› ·fi ÙÔÓ FDA Ì ÂȉÈ΋ ÁÈ· ÙË ÓfiÛÔ ¤Ó‰ÂÈÍË. ∏ Ù˘È΋ ·È‰È·ÙÚÈ΋ Ê·Ú̷΢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË ÂÚÈÏ·Ì‚¿ÓÂÈ ¯ÔÚ‹ÁËÛË Ì˘Ô¯·Ï·ÚˆÙÈÎÒÓ ÙˆÓ ÛÎÂÏÂÙÈÎÒÓ Ì˘ÒÓ (fiˆ˜ Ë Î˘ÎÏÔ‚ÂÓ˙·Ú›ÓË, Ë ÔÔ›· ‰ÂÓ Î˘ÎÏÔÊÔÚ› ÛÙËÓ ÂÏÏËÓÈ΋ ·ÁÔÚ¿. ∂Ó·ÏÏ·ÎÙÈο, Û ·È‰È¿ ËÏÈΛ·˜ >12 ÂÙÒÓ, ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó Ë Î·ÚÈÛÔÚÔ‰fiÏË ‹ Ë ÔÚÊÂÓ·‰Ú›ÓË) ‹/Î·È ·ÓÙÈηıÏÈÙÈÎÒÓ (ÙÚÈ΢ÎÏÈÎÒÓ fiˆ˜ Ë ·ÌÈÙÚÈÙ˘Ï›ÓË ‹ ÂÈÏÂÎÙÈÎÒÓ ·Ó·ÛÙÔϤˆÓ Â·Ó·ÚfiÛÏ˄˘ ÛÂÚÔÙÔÓ›Ó˘ - SSRIs - fiˆ˜ Ë ÊÏÔ˘ÔÍÂÙ›ÓË) Û ¯·ÌËÏ‹ ‰fiÛË. ™Â ÌÂϤÙË ÙÔ˘ 1996 Û ÂÓ‹ÏÈΘ Ì ÈÓÔÌ˘·ÏÁ›·, ‰È·ÈÛÙÒıËΠfiÙÈ Ë Û˘Ó‰˘·Ṳ̂ÓË ¯ÔÚ‹ÁËÛË ·ÓÙÈηٷıÏÈÙÈÎÒÓ, ÙfiÛÔ ÙÚÈ΢ÎÏÈÎÒÓ fiÛÔ Î·È SSRIs, ¤¯ÂÈ Â˘ÂÚÁÂÙÈο ·ÔÙÂϤÛÌ·Ù·, ηıÒ˜ ÂÌÊ·Ó›˙Ô˘Ó ‰˘Ó·ÌÈ΋ Û˘Ó¤ÚÁÂÈ· (9).

OÚÈṲ̂Ó˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó ıÂÙÈο ·ÔÙÂϤÛÌ·Ù· Ì ÙË ¯Ú‹ÛË ·Ó·ÏÁËÙÈÎÒÓ, fiˆ˜ Ë ·Ú·ÎÂÙ·ÌfiÏË, ‹ ÌË ÛÙÂÚÔÂȉÒÓ ·ÓÙÈÊÏÂÁÌÔÓˆ‰ÒÓ (NSAIDs - .¯. È‚Ô˘ÚÔʤÓË, ÈÓ‰ÔÌÂı·Î›ÓË), ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·ÓÙÈηٷıÏÈÙÈο Î·È Ì˘Ô¯·Ï·ÚˆÙÈο. ∏ ¯ÚËÛÈÌfiÙËÙ· ÙˆÓ NSAIDs ¤ÁÎÂÈÙ·È ÛÙËÓ ·Ó·ÏÁËÙÈ΋ ÙÔ˘˜ ‰Ú¿ÛË, ηıÒ˜ Ë ÈÓÔÌ˘·ÏÁ›· ‰ÂÓ ·ÔÙÂÏ› ÊÏÂÁÌÔÓ҉˜ ÓfiÛËÌ·. ¡ÂfiÙÂÚ· NSAIDs Ô˘ ÏÂÔÓÂÎÙÔ‡Ó fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÚfiÎÏËÛË Á·ÛÙÚÂÓÙÂÚÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ, fiˆ˜ ÔÈ ·Ó·ÛÙÔÏ›˜ ÙÔ˘ Cox II ÂÓ˙˘ÌÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ‰ÂÓ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û ·È‰È¿, ÂÓÒ ÙÂÏÂ˘Ù·›· ÂÎÊÚ¿˙ÔÓÙ·È ÂÈÊ˘Ï¿ÍÂȘ Û¯ÂÙÈο Ì ÙËÓ ·ÛÊ¿ÏÂÈ¿ ÙÔ˘˜ Î·È Û ÂÓ‹ÏÈΘ. ∏ ¯Ú‹ÛË Û ·È‰È¿ ·˘ÙÔÎfiÏÏËÙˆÓ ·Ó·ÏÁËÙÈÎÒÓ “ÂÌ‚·ÏˆÌ¿ÙˆÓ” (patches), Ù· ÔÔ›· ÂÚȤ¯Ô˘Ó ÏȉÔη˝ÓË Î·È Û˘Ó‰˘¿˙Ô˘Ó Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· Ù˘ ·˘ÍË̤Ó˘ Û˘ÌÌfiÚʈÛ˘, ÙˆÓ ÌÂÈˆÌ¤ÓˆÓ Û˘ÛÙËÌ·ÙÈÎÒÓ ·ÓÂÈı‡ÌËÙˆÓ ÂÓÂÚÁÂÈÒÓ Î·È Ù˘ ·˘ÍË̤Ó˘ ÙÔÈ΋˜ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜, ·ÚÔ˘ÛÈ¿˙ÂÈ ÂӉȷʤÚÔÓ. ¢È¯ÔÁӈ̛· ÂÈÎÚ·Ù› ˆ˜ ÚÔ˜ ÙÔ Û··ÛÌ· Ô˘ Ú¤ÂÈ Ó· ÂÈϤÁÂÙ·È ˆ˜ ÂÓ·ÚÎÙ‹ÚÈÔ˜ ·ÁˆÁ‹, ηıÒ˜ ÔÚÈṲ̂ÓÔÈ Û˘ÁÁÚ·Ê›˜ ÚÔÙ›ÓÔ˘Ó Ù· Ì˘Ô¯·Ï·ÚˆÙÈο, ÂÓÒ ¿ÏÏÔÈ Ù· ·ÓÙÈηٷıÏÈÙÈο Û ¯·ÌËÏ‹ ‰ÔÛÔÏÔÁ›· (9,34). ¶Ï‹ıÔ˜ ¿ÏÏˆÓ Û΢·ÛÌ¿ÙˆÓ Ô˘ ¤¯Ô˘Ó ‰ÔÎÈÌ·ÛÙ› Û ÂÓ‹ÏÈΘ, fiˆ˜ ‚ÂÓ˙ԉȷ˙Â›Ó˜, ηٷÛÙ·ÏÙÈο ÙÔ˘ ∫¡™, ·Ó·ÛÙÔÏ›˜ ÙÔ˘ ˘ÔÛÙÚÒÌ·ÙÔ˜ ƒ, ·ÓÙÈÂÈÏËÙÈο ‹ ·ÎfiÌ· Î·È ·ÓÙÈÌÈÎÚԂȷο, ‰ÂÓ ÌÔÚÔ‡Ó Û‡Ìʈӷ Ì ٷ ‰È·ı¤ÛÈÌ· ‰Â‰Ô̤ӷ Ó· ÂÓÙ·¯ıÔ‡Ó ÛÙËÓ Â›ÛËÌË ıÂÚ·¢ÙÈ΋ Ê·Ú¤ÙÚ·. ¡ÂfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜,

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∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ·

fiˆ˜ ·ÁˆÓÈÛÙ¤˜ ÓÙÔ·Ì›Ó˘, ÂÎÏÂÎÙÈÎÔ› ·Ó·ÛÙÔÏ›˜ Â·Ó·ÚfiÛÏ˄˘ ÛÂÚÔÙÔÓ›Ó˘ Î·È ÓÔÚ·‰ÚÂÓ·Ï›Ó˘ (fiˆ˜ Ë ÔÏÏ¿ ˘ÔÛ¯fiÌÂÓË ‰Ô˘ÏÔÍÂÙ›ÓË), ηıÒ˜ Î·È Û˘ÓıÂÙÈÎÔ› Ó¢ÚÔÌÂÙ·‚È‚·ÛÙ¤˜ Ô˘ ÌÈÌÔ‡ÓÙ·È ÙË ‰Ú¿ÛË ÙÔ˘ GABA, ‚Ú›ÛÎÔÓÙ·È Û ÂͤÏÈÍË.

æ˘¯ÔÏÔÁÈ΋ ÚÔÛ¤ÁÁÈÛË ∏ ¯Ú‹ÛË ÁÓˆÛȷ΋˜-Û˘ÌÂÚÈÊÔÚÈ΋˜ „˘¯ÔıÂÚ·›·˜ ¤¯ÂÈ Î·Ù¿ ÂÚÈÙÒÛÂȘ ·Ô‰Âȯı› ¯Ú‹ÛÈÌË, ·Ó Î·È ‰ÂÓ ˘¿Ú¯ÂÈ Ô ÈηÓfi˜ ·ÚÈıÌfi˜ ÌÂıÔ‰ÔÏÔÁÈο ÔÏÔÎÏËÚˆÌ¤ÓˆÓ ÌÂÏÂÙÒÓ Û ·È‰È¿ Ô˘ ı· ÌÔÚÔ‡Û·Ó Ó· ÙÂÎÌËÚÈÒÛÔ˘Ó ÙË ¯ÚËÛÈÌfiÙËÙ¿ Ù˘. ™Â Ì›· ÌÂϤÙË ·Ó·Ê¤ÚÂÙ·È fiÙÈ Ë ÂÊ·ÚÌÔÁ‹ ÁÓˆÛÈ·ÎÒÓ-Û˘ÌÂÚÈÊÔÚÈÎÒÓ Ù¯ÓÈÎÒÓ Ô‰‹ÁËÛ Û ‡ÊÂÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Û ‰È¿ÛÙËÌ· 4-24 ÌËÓÒÓ (40). º˘ÛÈ΋ Î·È ÎÔÈÓˆÓÈ΋ ·ÔηٿÛÙ·ÛË º˘ÛÈÎÔıÂÚ·›·, ¯ÂÈÚÔÌ·Ï¿ÍÂȘ, ÔÛÙÂÔ·ıËÙÈΤ˜ Ù¯ÓÈΤ˜, ¯Ú‹ÛË ËÏÂÎÙÚÈ΋˜ ‰È·‰ÂÚÌÈ΋˜ Ó¢ÚÈ΋˜ ‰È¤ÁÂÚÛ˘ (TENS), ηıÒ˜ Î·È ‰È·Ù·ÙÈΤ˜ Î·È ·ÂÚÔ‚ÈΤ˜ ·Û΋ÛÂȘ ¤¯Ô˘Ó ‰ÔÎÈÌ·ÛÙ› Ì ÂÈÙ˘¯›· Û ÂÓ‹ÏÈΘ, ‰ÂÓ ˘¿Ú¯ÂÈ fï˜ ÛËÌ·ÓÙÈ΋ ÂÌÂÈÚ›· Û ·È‰È¿. ∏ ÂÈÛÙÚÔÊ‹ ÛÙȘ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÙÔ˘ ·È‰ÈÔ‡ Ô˘ ‰È·ÎfiËÎ·Ó ÏfiÁˆ ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Ù˘ ÓfiÛÔ˘ (Ù·ÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË Û¯ÔÏ›Ԣ, ÎÔÈÓˆÓÈ΋ ·ÏÏËÏÂ›‰Ú·ÛË, ·È¯Ó›‰È, ‹È· ¿ıÏËÛË) η٤¯ÂÈ ÚˆÙ·Ú¯È΋ ÛËÌ·Û›·. ∫¿ÔÈÔÈ Û˘ÁÁÚ·Ê›˜, fiˆ˜ Ô Gedalia, ÚÔÙ›ÓÔ˘Ó ÚfiÁÚ·ÌÌ· ‹È·˜ ¿ÛÎËÛ˘ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ÎÔχÌÈ, ÂÚ¿ÙËÌ· ‹ Ô‰ËÏ·Û›·, ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ ÌÈÛ‹ ÒÚ· ËÌÂÚËÛ›ˆ˜ (9).

™˘ÌÂÚ¿ÛÌ·Ù· - ÙÔ Ì¤ÏÏÔÓ ¶·Ú¿ ÙË ÛËÌ·ÓÙÈ΋ ÚfiÔ‰Ô Ô˘ ¤¯ÂÈ ÛËÌÂȈı› ÛÙË ‰È·‰Èηۛ· ÚÔÛ¤ÁÁÈÛ˘ Î·È Î·Ù·ÓfiËÛ˘ Ù˘ ÈÓÔÌ˘·ÏÁ›·˜ ÛÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ ‰ÂηÂÙÈÒÓ, ÂÏ¿¯ÈÛÙ· ¤¯Ô˘Ó ÌÂÙ·‚ÏËı› fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ ‹ ÛÙË ‰˘Ó·ÙfiÙËÙ· ·ÔÙÂÏÂÛÌ·ÙÈ΋˜ Î·È ·ÛÊ·ÏÔ‡˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Ù˘. ¶·Ú·Ì¤ÓÂÈ, ¤ÙÛÈ, ۷ʤ˜ fiÙÈ Î·Ì›· ÛÙÚ·ÙËÁÈ΋ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ÓfiÛÔ˘ ‰ÂÓ ÚfiÎÂÈÙ·È Ó· ¢ԉˆı› fiÛÔ ÙÔ ·ÈÙÈÔ·ıÔÁÂÓÂÙÈÎfi ˘fiÛÙڈ̿ Ù˘ ·Ú·Ì¤ÓÂÈ ·‰È¢ÎÚ›ÓÈÛÙÔ. ∏ ÂÚ¢ÓËÙÈ΋ ÚÔÛ¿ıÂÈ· Û˘Ó¯›˙ÂÙ·È Ì ·Ì›ˆÙÔ ÂӉȷʤÚÔÓ, ÂÓÒ È‰È·›ÙÂÚË ÛËÌ·Û›· ‰›‰ÂÙ·È ÛÙȘ ÌÂϤÙ˜ Ô˘ ‰ÈÂÚ¢ÓÔ‡Ó ÙÔÓ ÚfiÏÔ ÙˆÓ ÁÂÓÂÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ÙˆÓ ·ÓÔÛÔÏÔÁÈÎÒÓ Ì˯·ÓÈÛÌÒÓ, ηıÒ˜

Î·È Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ÙˆÓ ÂÓ‰ÔÁÂÓÒÓ ÔÈÔÂȉÒÓ ÛÙËÓ ·ıÔÁ¤ÓÂÛË Ù˘ ÈÓÔÌ˘·ÏÁ›·˜.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Wolfe F, Smythe HA, Yunus MB, Bennett RM, Bombardier C, Goldenberg DL et al. The American College of Rheumatology 1990 criteria for the classification of fibromyalgia. Report of the Multicenter Criteria Committee. Arthritis Rheum 1990;33: 160-172.

2. Mart›nez-Lav›n M. The autonomic nervous system and fibromyalgia. The clinical neurobiology of fibromyalgia and myofascial pain: therapeutic implications. Binghamton, NY: The Haworth Press; 2002. p. 221-228.

3. Staud R, Robinson ME, Mauderli AP, Cannon RL, Vierck CJ, Price DD. Opioids modulate the enhanced temporal summation of second pain of fibromyalgia patients. Arthritis Rheum 2002;46 (9 Suppl):S396.

4. Jacobsson L, Lindgarde F, Manthorpe R. The commonest rheumatic complaints of over six weeks’ duration in a twelve-month period in a defined Swedish population. Prevalences and relationships.

Scand J Rheumatol 1989;18:353-360.

5. Buskila D, Press J, Gedalia A, Klein M, Neumann L, Boehm R et al. Assessment of nonarticular tenderness and prevalence of fibromyalgia in children. J Rheumatol 1993;20:368-370.

6. Clark P, Burgos-Vargas R, Medina-Palma C, Lavielle P, Marina FF. Prevalence of fibromyalgia in children: a clinical study of Mexican children. J Rheumatol 1998;25:2009-2014.

7. Mikkelsson M. One year outcome of preadolescents with fibromyalgia. J Rheumatol 1999;26:674-682.

8. Siegel DM, Janeway D, Baum J. Fibromyalgia syndrome in children and adolescents: clinical features at presentation and status at follow-up. Pediatrics 1998;101:377-382.

9. Gedalia A, Garcia CO, Molina JF, Bradford NJ, Espinoza LR. Fibromyalgia syndrome: experience in a pediatric rheumatology clinic. Clin Exp Rheumatol 2000;18:415-419.

10. Jubrias SA, Bennett RM, Klug GA. Increased incidence of a resonance in the phosphodiester region of 31P nuclear magnetic resonance spectra in the skeletal muscle of fibromyalgia patients. Arthritis Rheum 1994;37:801-807.

11. Abud-Mendoza C, Magana-Aquino M, Medina R, Grimaldo JI, Rodriguez-Rivera G, Gonsalez-Amaro R. Hypothalamus-hypophysis-thyroid axis dysfunction in patients with refractory fibromyalgia [Abstract Supplement]. Arthritis Rheum 1997;40:#9.

12. Riedel W, Schlapp U, Leck S, Netter P, Neeck G. Blunted ACTH and cortisol responses to systemic injection of corticotropin-releasing hormone (CRH) in fibromyalgia: role of somatostatin and CRH-binding protein. Ann NY Acad Sci 2002;966:483-490. ¶·È‰È·ÙÚÈ΋ 2005;68:342-350

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µ. ∆Û·ÁÚ‹˜ Î·È Û˘Ó.

13. Neeck G, Crofford LJ. Neuroendocrine perturbations in fibromyalgia and chronic fatigue syndrome.

Rheum Dis Clin North Am 2000;26:989-1002.

14. Bennett RM, Clark SR, Campbell SM, Burckhardt CS. Low levels of somatomedin C in patients with the fibromyalgia syndrome. A possible link between sleep and muscle pain. Arthritis Rheum 1992;35: 1113-1116.

15. Bennett RM, Cook DM, Clark SR, Burckhardt CS, Campbell SM. Hypothalamic-pituitary-insulin-like growth factor-I axis dysfunction in patients with fibromyalgia. J Rheumatol 1997;24:1384-1389.

16. Bennett RM, Clark SC, Walczyk J. A randomized, double-blind, placebo-controlled study of growth hormone in the treatment of fibromyalgia. Am J Med 1998;104:227-231.

17. Paiva ES, Deodhar A, Jones KD, Bennett R. Impaired growth hormone secretion in fibromyalgia patients: evidence for augmented hypothalamic somatostatin tone. Arthritis Rheum 2002;46:1344-1350.

18. Bennett RM, Clark SR, Burckhardt CS, Cook D. IGF1 assays and other GH tests in 500 fibromyalgia patients. J Musculoskeletal Pain 1995;3 (Suppl 1):109.

19. Russell IJ, Vaeroy H, Javors M, Nyberg F. Cerebrospinal fluid biogenic amine metabolites in fibromyalgia/fibrositis syndrome and rheumatoid arthritis. Arthritis Rheum 1992;35:550-556.

20. Larson AA, Giovengo SL, Russell IJ, Michalek JE. Changes in the concentrations of amino acids in the cerebrospinal fluid that correlate with pain in patients with fibromyalgia: implications for nitric oxide pathways. Pain 2000;87:201-211.

21. Russell IJ, Orr MD, Littman B, Vipraio GA, Alboukrek D, Michalek JE et al. Elevated cerebrospinal fluid levels of substance P in patients with the fibromyalgia syndrome. Arthritis Rheum 1994;37: 1593-1601.

22. Martinez-Lavin M, Hermosillo AG, Rosas M, Soto ME. Circadian studies of autonomic nervous balance in patients with fibromyalgia: a heart rate variability analysis. Arthritis Rheum 1998;41:1966-1971.

23. Wallace DJ, Linker-Israeli M, Hallegua D, Silverman S, Silver D, Weisman MH. Cytokines play an aetiopathogenetic role in fibromyalgia: a hypothesis and pilot study. Rheumatology 2001;40:743-749.

24. Salemi S, Rethage J, Wollina U, Michel BA, Gay RE, Gay S et al. Detection of interleukin 1beta (IL-1beta), IL-6, and tumor necrosis factor-alpha in skin of patients with fibromyalgia. J Rheumatol 2003;30: 146-150.

25. Gur A, Karakoc M, Nas K, Cevik R, Denli A, Sarac J. Cytokines and depression in cases with fibromyalgia. J Rheumatol 2002;29:358-361.

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26. Pellegrino MJ, Waylonis GW, Sommer A. Familial occurrence of primary fibromyalgia. Arch Phys Med Rehabil 1989;70:61-63.

27. Arnold LM, Iyengar SK, Khan MA, Kushner I, Russel IJ, Yunus MB et al. Genetic linkage of fibromyalgia to the serotonin receptor 2A region on chromosome 13 and the HLA region on chromosome 6.

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28. Roizenblatt S, Tufik S, Goldenberg J, Pinto LR, Hilario MO, Feldman D. Juvenile fibromyalgia: clinical and polysomnographic aspects. J Rheumatol 1997; 24:579-585.

29. Tayag-Kier CE, Keenan GF, Scalzi LV, Schultz B, Elliott J, Zhao RH et al. Sleep and periodic limb movement in sleep in juvenile fibromyalgia. Pediatrics 2000;106:E70.

30. Roizenblatt S, Moldofsky H, Benedito-Silva AA, Tufik S. Alpha sleep characteristics in fibromyalgia.

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31. Kashikar-Zuck S, Vaught MH, Goldschneider KR, Graham TB, Miller JC. Depression, coping, and functional disability in juvenile primary fibromyalgia syndrome. J Pain 2002;3:412-419.

32. Gracely RH, Petzke F, Wolf JM, Clauw DJ. Functional magnetic resonance imaging evidence of augmented pain processing in fibromyalgia. Arthritis Rheum 2002;46:1333-1343.

33. Russell IJ. Fibromyalgia syndrome sub-groups [editorial]. J Musculoskeletal Pain 2002;10:1-2.

34. Anthony KK, Schanberg LE. Juvenile primary fibromyalgia syndrome. Curr Rheumatol Rep 2001;3:165-171.

35. Yunus MB, Masi AT. Juvenile primary fibromyalgia syndrome. A clinical study of thirty-three patients and matched normal controls. Arthritis Rheum 1985;28:138-145.

36. Felson DT, Goldenberg DL. The natural history of fibromyalgia. Arthritis Rheum 1986;29:1522-1526.

37. Ledingham J, Doherty S, Doherty M. Primary fibromyalgia syndrome: an outcome study. Br J Rheumatol 1993;32:139-142.

38. ªalleson PN, al-Matar M, Petty RE. Idiopathic musculoskeletal pain syndromes in children. J Rheumatol 1992;19:1786-1789.

39. Buskila D, Neumann L, Hershman E, Gedalia A, Press J, Sukenik S. Fibromyalgia syndrome in children: an outcome study. J Rheumatol 1995;22:525528.

40. Walco GA, Ilowite NT. Cognitive-behavioral intervention for juvenile primary fibromyalgia syndrome. J Rheumatol 1992;19:1617-1619.

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Fibromyalgia in childhood V. Tsagris1, A. Kitsopoulou2, C. Mihas2, P. Nikolaidou - Karpathiou1

Abstract Fibromyalgia of childhood is a common musculoskeletal pain disorder characterized by widespread persistent pain, the presence of multiple discrete tender points on physical examination, stiffness, sleep disturbances and fatigue. Its course is unremitting and it has a restrictive effect on the functional ability and psychosocial development of the affected children. Although its exact aetiology remains unknown, it is perceived that a number of factors can contribute to the clinical expression of the disorder. The therapeutic intervention is non-specific and usually multidisciplinary.

Key words Fibromyalgia, children, pain, treatment, musculoskeletal.

1 3rd Paediatric Clinic of the University of Athens, “Attikon” University Hospital, Haidari, Athens 2 Paediatric Clinic, “Thriassio” General Hospital, Elefsina, Athens Correspondence: Vassilios Tsagris 3rd Paediatric Clinic of the University of Athens “Attikon” University Hospital 1 Rimini Str., Haidari, Athens E-mail: billtsag@yahoo.com Date of submission: 22-10-2004 Date of approval: 13-04-2005

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∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞

™‡Ó‰ÚÔÌÔ Guillain-Barré. ŒÎ‚·ÛË Ù˘ ÓfiÛÔ˘ Û ·È‰È¿ Ô˘ ÓÔÛËχÙËÎ·Ó Û ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £ÂÚ·›·˜ ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £ÂÚ·›·˜, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”, ∞ı‹Ó·

∞. ∆ÛÔ‡ÙÛÔ˘, ¢. °ÎÈÒÓ˘, ™. ª·ÛÙÚÔÁÈ¿ÓÓË, π. ¶··‰¿ÙÔ˜

AÏÏËÏÔÁÚ·Ê›·: ¢ËÌ‹ÙÚÈÔ˜ °ÎÈÒÓ˘ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”, £Ë‚ÒÓ & §Â‚·‰Â›·˜ ∆.∫. 115 27, ∞ı‹Ó·

∂ÈÛ·ÁˆÁ‹: ∆Ô Û‡Ó‰ÚÔÌÔ Guillain-Barré (GB) Â›Ó·È Ì›· ÚÔ˚Ô‡Û·, Û˘ÌÌÂÙÚÈ΋ ÔÏ˘Ó¢ÚÔ¿ıÂÈ·,

∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 10-12-2003 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 08-07-2005

ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ: ªÂÏÂÙ‹ıËÎ·Ó ·Ó·‰ÚÔÌÈο Ù· È·ÙÚÈο ·Ú¯Â›· fiÏˆÓ ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔ GB Ô˘ ÂÈÛ‹¯ıËÛ·Ó ÛÙË ª∂£ ·fi ÙÔÓ π·ÓÔ˘¿ÚÈÔ 1986 ¤ˆ˜ Î·È ÙÔÓ π·ÓÔ˘¿ÚÈÔ 2003 Î·È Î·Ù·ÁÚ¿ÊËÎ·Ó Ù· ÎÏÈÓÈο Î·È Ù· ÂÚÁ·ÛÙËÚȷο ÛÙÔȯ›·, Ë ·ÁˆÁ‹ Ô˘ ¯ÔÚËÁ‹ıËÎÂ, ηıÒ˜ Î·È Ë ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘. ∏ ÏÂÈÙÔ˘ÚÁÈ΋ ÈηÓfiÙËÙ· (Ì ÙËÓ Îϛ̷η Hughes) ÚÔÛ‰ÈÔÚ›ÛÙËΠηٿ ÙËÓ Â›ÛÔ‰Ô Î·È ÙËÓ ¤ÍÔ‰Ô ÙÔ˘ ·È‰ÈÔ‡ ·fi ÙÔ ÓÔÛÔÎÔÌ›Ô.

¶ÂÚ›ÏË„Ë Ì ÔÍ›· ÂÈÛ‚ÔÏ‹, ÂͤÏÈÍË Ô˘ ‰È·ÚΛ ̤Ú˜ ‹ ‚‰ÔÌ¿‰Â˜ Î·È Ì ηϋ ¤Î‚·ÛË ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ. ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ ‹Ù·Ó Ó· ÌÂÏÂÙËı› Ë ÔÚ›· Î·È Ë ¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔ GB Ô˘ ÓÔÛËχÙËÎ·Ó Û ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £ÂÚ·›·˜ (ª∂£).

∞ÔÙÂϤÛÌ·Ù·: ∫·Ù¿ ÙËÓ ÂÚ›Ô‰Ô ·fi ÙÔÓ π·ÓÔ˘¿ÚÈÔ 1986 ¤ˆ˜ ÙÔÓ π·ÓÔ˘¿ÚÈÔ 2003 ÓÔÛËχÙËηÓ

ÛÙË ª∂£ 12 ·È‰È¿ Ì ۇӉÚÔÌÔ GB (5 ·ÁfiÚÈ·, 7 ÎÔÚ›ÙÛÈ·), ËÏÈΛ·˜ 22 ÌËÓÒÓ ¤ˆ˜ 15 ÂÙÒÓ (̤ÛË ËÏÈΛ·: 8,04 ¤ÙË). ¶ÚÔËÁÔ‡ÌÂÓË ÈÔÁÂÓ‹˜ Ïԛ̈ÍË ·Ó·Ê¤ÚÂÙ·È Û 8 ·È‰È¿. ŒÏÂÁ¯Ô˜ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤ÁÈÓ Û 10/12 ·È‰È¿ Î·È Ô ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ‚Ú¤ıËΠ۠6/10 ·È‰È¿. ∂ÎÙfi˜ ·fi ÙË Ì˘˚΋ ·‰˘Ó·Ì›· Î·È ÙËÓ Î·Ù¿ÚÁËÛË ÙˆÓ ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ, Û 11/12 ·ÛıÂÓ›˜ ·ÚÔ˘ÛÈ¿ÛÙËΠÚÔÛ‚ÔÏ‹ ÎÚ·ÓÈ·ÎÒÓ ÓÂ‡ÚˆÓ Î·È Û 9/12 ·ÛıÂÓ›˜ ˘‹ÚÍ·Ó ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (΢ڛˆ˜ ˘¤ÚÙ·ÛË). ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ ›¯·Ó score ‚·Ú‡ÙËÙ·˜ 4 ‹ 5 Ì ÙËÓ Îϛ̷η Hughes. ∏ ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙËÛË (O¡¶) ¤ÁÈÓ Û fiÏ· Ù· ·È‰È¿ Î·È ‹Ù·Ó ‰È·ÁÓˆÛÙÈ΋ fiÙ·Ó ÂÎÙÂϤÛÙËΠÌÂÙ¿ ÙËÓ 7Ë Ë̤ڷ Ù˘ ÓfiÛÔ˘. ™Â Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi (ª∞) Ù¤ıËÎ·Ó 11/12 ·È‰È¿, Ì ̛· ̤ÛË ‰È¿ÚÎÂÈ· ˘ÔÛÙ‹ÚÈ͢ 18,4 Ë̤Ú˜ (·fi 2 ¤ˆ˜ 37 Ë̤Ú˜) Î·È Û 10/12 ·È‰È¿ ¯ÔÚËÁ‹ıËΠÁ-ÛÊ·ÈÚ›ÓË (IVIG). ∏ ̤ÛË ‰È¿ÚÎÂÈ· ·Ú·ÌÔÓ‹˜ ÛÙË ª∂£ ‹Ù·Ó 24,8 Ë̤Ú˜ (2-42 Ë̤Ú˜). ∫·Ó¤Ó· ·È‰› ‰ÂÓ ·‚›ˆÛ ·ÎfiÌË Î·È ÌÂÙ¿ ·fi 6 Ì‹Ó˜, ÂÓÒ 10/12 ·È‰È¿ ›¯·Ó Ï‹ÚË ·ÔηٿÛÙ·ÛË Î·È 2/12 ·ÚÔ˘Û›·˙·Ó Ó¢ÚÔÏÔÁÈο ˘ÔÏ›ÌÌ·Ù·. ™˘ÌÂÚ¿ÛÌ·Ù·: ∆Ô Û‡Ó‰ÚÔÌÔ Guillain-Barré ¤¯ÂÈ Î·Ï‡ÙÂÚË ¤Î‚·ÛË ÛÙ· ·È‰È¿ Û ۇÁÎÚÈÛË ÌÂ

ÙÔ˘˜ ÂÓ‹ÏÈΘ, ÂÓÒ Ë Ì˯·ÓÈ΋ ˘ÔÛÙ‹ÚÈÍË Ù˘ ·Ó·ÓÔ‹˜ ‰ÂÓ ˘‹ÚÍ ·Ú¿ÁÔÓÙ·˜ η΋˜ ÂͤÏÈ͢ ·˘ÙÒÓ ÙˆÓ ·È‰ÈÒÓ.

§¤ÍÂȘ ÎÏÂȉȿ ™‡Ó‰ÚÔÌÔ Guillain-Barré, Ì˯·ÓÈÎfi˜ ·ÂÚÈÛÌfi˜, ÂÓ‰ÔÊϤ‚È· Á-ÛÊ·ÈÚ›ÓË.

∂ÈÛ·ÁˆÁ‹ ∆Ô Û‡Ó‰ÚÔÌÔ Guillain-Barré (GB) Â›Ó·È Ì›· ÔÍ›· ·ÔÌ˘ÂÏÈÓˆÙÈ΋ Ó¢ÚÔ¿ıÂÈ· Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ÎÏÈÓÈο ·fi ÚÔԉ¢ÙÈο ¯·Ï·Ú‹ ·Ú¿Ï˘ÛË Î·È Î·Ù¿ÚÁËÛË ÙˆÓ ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ. ∏ ÚÔÛ‚ÔÏ‹ ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (∞¡™) Î·È ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ ÓÂ‡ÚˆÓ Â›Ó·È Û˘¯Ó‹. ∏ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÔÏÔ¶·È‰È·ÙÚÈ΋ 2005;68:352-356

ÎÏËÚÒÓÂÙ·È Û ‰È¿ÛÙËÌ· 3-21 ËÌÂÚÒÓ (̤ÛÔ˜ fiÚÔ˜: 15 Ë̤Ú˜). ∞ÎÔÏÔ˘ı› Ë Ê¿ÛË ÛÙ·ıÂÚÔÔ›ËÛ˘ Ô˘ Â›Ó·È ‰È¿ÚÎÂÈ·˜ ÂÚ›Ô˘ 15 ËÌÂÚÒÓ Î·È, Ù¤ÏÔ˜, Ë Ê¿ÛË ‚ÂÏÙ›ˆÛ˘, ‰È¿ÚÎÂÈ·˜ ËÌÂÚÒÓ, ‚‰ÔÌ¿‰ˆÓ ‹ ÌËÓÒÓ. ∆Ô Û‡Ó‰ÚÔÌÔ GB ¤¯ÂÈ Ì›· Û¯ÂÙÈο Ì·ÎÚ¿ ÔÚ›·, ·ÏÏ¿ ηϋ ¤Î‚·ÛË ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÊÔÚ¤˜. øÛÙfiÛÔ, ÔÛÔÛÙfi ÂÚ›Ô˘ 20-30% ÙˆÓ ·ÛıÂÓÒÓ ¯ÚÂÈ¿˙ÂÙ·È Ì˯·ÓÈ΋ ˘ÔÛÙ‹ÚÈÍË Ù˘

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™‡Ó‰ÚÔÌÔ Guillain-Barré

·Ó·ÓÔ‹˜, Ë ÔÔ›· Î·È ıˆÚÂ›Ù·È ÂÈ‚·Ú˘ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ (1) fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÚfiÁÓˆÛË. ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ ‹Ù·Ó Ó· ÌÂÏÂÙËıÔ‡Ó Ù· ÎÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο, Ë ÔÚ›· Î·È Ë ¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔ Guillain-Barré ÛÙË ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £ÂÚ·›·˜ (ª∂£).

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ªÂÏÂÙ‹ıËÎ·Ó ·Ó·‰ÚÔÌÈο Ù· È·ÙÚÈο ·Ú¯Â›· fiÏˆÓ ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔ GB Ô˘ ÓÔÛËχÙËÎ·Ó ÛÙË ª∂£ ·fi ÙÔÓ π·ÓÔ˘¿ÚÈÔ ÙÔ˘ 1986 ¤ˆ˜ ÙÔÓ π·ÓÔ˘¿ÚÈÔ ÙÔ˘ 2003. ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ ›¯·Ó ÂÍÂÙ·ÛÙ› ·fi ·È‰ÔÓ¢ÚÔÏfiÁÔ. ∆· ·È‰È¿ Ô˘ Û˘ÌÂÚÈÏ‹ÊıËÎ·Ó ÛÙË ÌÂϤÙË ÏËÚÔ‡Û·Ó Ù· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ GB Ô˘ ¤¯Ô˘Ó ıÂÛÈÛÙ› ·fi ÙÔ˘˜ Asbury Î·È Cornblath (2). ∫·Ù·ÁÚ¿ÊËÎ·Ó Ù· ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο ÛÙÔȯ›·, Ë ·ÁˆÁ‹ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ, ηıÒ˜ Î·È Ë ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘. °È· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ‚·Ú‡ÙËÙ·˜ Ù˘ ÓfiÛÔ˘ ηٿ ÙËÓ Â›ÛÔ‰Ô ÙˆÓ ·ÛıÂÓÒÓ ÛÙË ª∂£ ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Îϛ̷η Hughes (1). ™ËÌÂÈÒıËηÓ, Â›Û˘, Ë ·ÚÔ˘Û›· ‹ fi¯È ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ, Ë ÚÔÛ‚ÔÏ‹ ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ Ó‡ڈÓ, ÔÈ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ∞¡™, ηıÒ˜ Î·È ÙÔ ÈÛÙÔÚÈÎfi ÚÔËÁÔ‡ÌÂÓ˘ ÈÔÁÂÓÔ‡˜ Ïԛ̈͢, Ô ¤ÏÂÁ¯Ô˜ ·ÓÙÈÛˆÌ¿ÙˆÓ Î·È Ù· Â˘Ú‹Ì·Ù· ·fi ÙÔ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi (∂¡À). ∆¤ÏÔ˜, ηٷÁÚ¿ÊËÎ·Ó Ë ‰È¿ÚÎÂÈ· ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘, Ë ‰È¿ÚÎÂÈ· ÙÔ˘ Ì˯·ÓÈÎÔ‡ ·ÂÚÈÛÌÔ‡ (ª∞) Î·È Ë ‰È¿ÚÎÂÈ· Ù˘ ·Ú·ÌÔÓ‹˜ ÙÔ˘ ·È‰ÈÔ‡ ÛÙË ª∂£ Î·È ÛÙÔ ÓÔÛÔÎÔÌ›Ô. ∆· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Guillain-Barré ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1. £ÂˆÚ‹ıËΠ“ηϋ ¤Î‚·ÛË” Ë ÈηÓfiÙËÙ· ‚¿‰ÈÛ˘ ¯ˆÚ›˜ ‚Ô‹ıÂÈ· (score <2) Î·È “η΋ ¤Î‚·ÛË” Ë ·‰˘Ó·Ì›· ‚¿‰ÈÛ˘ ¯ˆÚ›˜ ‚Ô‹ıÂÈ· (score 3-5).

∞ÔÙÂϤÛÌ·Ù· ÷ڷÎÙËÚÈÛÙÈο ·ÛıÂÓÒÓ ∫·Ù¿ ÙËÓ ÂÚ›Ô‰Ô ·fi ÙÔÓ π·ÓÔ˘¿ÚÈÔ 1986 ¤ˆ˜ ÙÔÓ π·ÓÔ˘¿ÚÈÔ 2003 ÓÔÛËχÙËÎ·Ó ÛÙË ª∂£ 12 ·È‰È¿ Ì ۇӉÚÔÌÔ GB (5 ·ÁfiÚÈ·, 7 ÎÔÚ›ÙÛÈ·), ËÏÈΛ·˜ 22 ÌËÓÒÓ ¤ˆ˜ 15 ÂÙÒÓ (̤ÛË ËÏÈΛ·: 8,04 ¤ÙË). ¶ÚÔËÁÔ‡ÌÂÓË ÈÔÁÂÓ‹˜ Ïԛ̈ÍË ·Ó·Ê¤ÚÂÙ·È Û 8/12 ·È‰È¿ (Ïԛ̈ÍË ·Ó·Ó¢ÛÙÈÎÔ‡ Û 2, ÂÙÈÎÔ‡ Û 2, ¿ÏÏË ÂÌ‡ÚÂÙË Ïԛ̈ÍË Û 3, ÈÏ·Ú¿ Û 1). ™Â 10/12 ·È‰È¿ ÂÏ‹ÊıË ¤ÏÂÁ¯Ô˜ ·ÓÙÈÛˆÌ¿ÙˆÓ Î·È Ô ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ‚Ú¤ıËΠ۠6/10 ·È‰È¿ (Mycoplasma pneumoniae Û 2, Èfi˜ ÈÏ·Ú¿˜ Û 1, Campylobacter spp Û 1, Èfi˜ ·ÏÔ‡ ¤ÚËÙ· Û 1, Èfi˜ ·ÓÂÌ¢ÏÔÁÈ¿˜˙ˆÛÙ‹Ú· Û 1).

¶›Ó·Î·˜ 1. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ GuillainBarré ÷ڷÎÙËÚÈÛÙÈο Ô˘ Â›Ó·È ··Ú·›ÙËÙ· ÁÈ· ÙË ‰È¿ÁÓˆÛË ¶ÚÔԉ¢ÙÈ΋ ·‰˘Ó·Ì›· ÂÚÈÛÛfiÙÂÚˆÓ - ·fi ÙÔ ¤Ó· ¿ÎÚÔ - ÌÂÏÒÓ ªÂ›ˆÛË/ÂÍ¿ÏÂÈ„Ë ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ Ã·Ú·ÎÙËÚÈÛÙÈο Ô˘ ÂÓÈÛ¯‡Ô˘Ó ÙË ‰È¿ÁÓˆÛË ∂ͤÏÈÍË Û ÂÚ›Ô˘ 4 ‚‰ÔÌ¿‰Â˜ ™¯ÂÙÈο Û˘ÌÌÂÙÚÈ΋ ÚÔÛ‚ÔÏ‹ ¶ÂÚÈÔÚÈṲ̂ÓË ÚÔÛ‚ÔÏ‹ ·ÈÛıËÙÈÎfiÙËÙ·˜ ¶ÚÔÛ‚ÔÏ‹ ÎÚ·ÓÈ·ÎÒÓ ÓÂ‡ÚˆÓ ŒÓ·ÚÍË ‚ÂÏÙ›ˆÛ˘ 2-4 ‚‰ÔÌ¿‰Â˜ ÌÂÙ¿ ÙËÓ ÔÏÔÎÏ‹ÚˆÛË Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜ ¢È·Ù·Ú·¯¤˜ ·fi ÙÔ ·˘ÙfiÓÔÌÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ· ∞Ô˘Û›· ˘ÚÂÙÔ‡ ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘ ∞‡ÍËÛË Ï¢ÎÒÌ·ÙÔ˜ ∂¡À Ì›· ‚‰ÔÌ¿‰· ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘ ∫‡ÙÙ·Ú· ∂¡À <10 (ÌÔÓÔ·ÙÙ·Ú·) ∂Ï¿ÙÙˆÛË Ó¢ÚÈ΋˜ ·ÁˆÁÈÌfiÙËÙ·˜ ÷ڷÎÙËÚÈÛÙÈο Ô˘ ·ÌÊÈÛ‚ËÙÔ‡Ó ÙË ‰È¿ÁÓˆÛË ŒÓÙÔÓË, Â›ÌÔÓË ·Û˘ÌÌÂÙÚ›· Ù˘ Ì˘˚΋˜ ·‰˘Ó·Ì›·˜ ∂›ÌÔÓË ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ·ÛÙ˘ ‹ ÂÓÙ¤ÚÔ˘ OÚıÔ΢ÛÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ ÛÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘ ∫‡ÙÙ·Ú· ∂¡À >50 (ÌÔÓÔ·ÙÙ·Ú·) ‹ ·ÚÔ˘Û›· ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ Ã·Ú·ÎÙËÚÈÛÙÈο Ô˘ ·ÔÎÏÂ›Ô˘Ó ÙË ‰È¿ÁÓˆÛË ŒÓ‰ÂÈÍË ÌÂÙ·‚ÔÏÈÎÔ‡, ÏÔÈÌÒ‰Ô˘˜ ÓÔÛ‹Ì·ÙÔ˜ ‹ ‰ËÏËÙËÚ›·Û˘ ¶·ÚÔ˘Û›· ·ÌÈÁÒ˜ ·ÈÛıËÙÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ

¡Â˘ÚÔÏÔÁÈΤ˜ ÂΉËÏÒÛÂȘ ∫·Ù¿ ÙËÓ Â›ÛÔ‰fi ÙÔ˘˜ ÛÙË ª∂£, fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ ›¯·Ó ÂÎÛÂÛËÌ·Ṳ̂ÓË Ì˘˚΋ ·‰˘Ó·Ì›· (score ‚·Ú‡ÙËÙ·˜ 4 ‹ 5 Ì ÙËÓ Îϛ̷η Hughes). ∏ Ì˘˚΋ ·‰˘Ó·Ì›· ·ÊÔÚÔ‡ÛÂ Î·È ÛÙÔ˘˜ ·Ó·Ó¢ÛÙÈÎÔ‡˜ ̇˜. ∂›Û˘, fiÏ· Ù· ·È‰È¿ ·ÚÔ˘Û›·Û·Ó Ì›ˆÛË ‹ ηٿÚÁËÛË ÙˆÓ ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ. ÀÂÚ¢·ÈÛıËÛ›· ‹ fiÓÔ ·Ó¤ÊÂÚ·Ó 6/12 ·È‰È¿. ¶ÚÔÛ‚ÔÏ‹ ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ ÓÂ‡ÚˆÓ Â›¯·Ó 11/12 ·È‰È¿, ÂÓÒ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ∞¡™ ·ÚÔ˘Û›·Û·Ó 9/12 ·È‰È¿. ¶ÈÔ Û˘¯Ó‹ ÂΉ‹ÏˆÛË ·fi ÙÔ ∞¡™ ‹Ù·Ó Ë ˘¤ÚÙ·ÛË (8/12 ·È‰È¿) Ô˘ ‰‡ÛÎÔÏ· ÂÏÂÁ¯fiÙ·Ó Ì ÙËÓ ·ÁˆÁ‹. ÕÏϘ ÂΉËÏÒÛÂȘ ·fi ÙÔ ∞¡™ ‹Ù·Ó Ë Â›Û¯ÂÛË Ô‡ÚˆÓ (4/12), Ë ‰˘ÛÎÔÈÏÈfiÙËÙ· (2/12), Ë ¤ÓÙÔÓË ÂÊ›‰ÚˆÛË (4/12) Î·È Ë ‚Ú·‰˘Î·Ú‰›· (1/12). ∫·Ó¤Ó· ·È‰› ‰ÂÓ ·ÚÔ˘Û›·Û ÂÈΛӉ˘ÓË ÁÈ· ÙË ˙ˆ‹ ‰È·Ù·Ú·¯‹ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡. ∏ ‰È¿ÚÎÂÈ· Ù˘ Ê¿Û˘ Âȉ›ӈÛ˘ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘ ̤¯ÚÈ ÙË Ì¤ÁÈÛÙË ‚·Ú‡ÙËÙ· ‹Ù·Ó 1-20 Ë̤Ú˜ (̤ÛÔ˜ fiÚÔ˜: 7 Ë̤Ú˜). ¶·È‰È·ÙÚÈ΋ 2005;68:352-356

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·354

354

∞. ∆ÛÔ‡ÙÛÔ˘ Î·È Û˘Ó.

∏ O¡¶ ¤ÁÈÓ Û fiÏ· Ù· ·È‰È¿ Î·È Ô ¯·Ú·ÎÙËÚÈÛÙÈÎfi˜ ÏÂ˘ÎˆÌ·ÙÔ΢ÙÙ·ÚÈÎfi˜ ‰È¯·ÛÌfi˜ ‚Ú¤ıËΠ۠11/12 ·È‰È¿ (ÌÂÙ¿ ÙËÓ 7Ë Ë̤ڷ Ù˘ ÓfiÛÔ˘).

∞ÓÙÈÌÂÙÒÈÛË - £ÂÚ·›· ŒÓÙÂη ·È‰È¿ Ù¤ıËÎ·Ó Û ª∞ ÏfiÁˆ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜. ∏ ‰È¿ÚÎÂÈ· Ù˘ Ì˯·ÓÈ΋˜ ˘ÔÛÙ‹ÚÈ͢ Î˘Ì¿ÓıËΠ·fi 2 ¤ˆ˜ 37 Ë̤Ú˜ (̤ÛÔ˜ fiÚÔ˜: 18,4 Ë̤Ú˜). ™Â ÙÚ›· ·È‰È¿ ¤ÁÈÓ ÙÚ·¯ÂÈÔÛÙÔÌ›·, Ë ÔÔ›· ÎÚ›ıËΠÛÎfiÈÌË ÏfiÁˆ Ù˘ ·Ú·ÙÂٷ̤Ó˘ ‰È·ÛˆÏ‹ÓˆÛ˘ (22, 27 Î·È 37 Ë̤Ú˜). ∂Ó‰ÔÊϤ‚È· Á-ÛÊ·ÈÚ›ÓË (IVIG) ¯ÔÚËÁ‹ıËΠ۠10/12 ·È‰È¿, ‰ËÏ·‰‹ Û fiÏ· Ù· ·È‰È¿ Ô˘ ÓÔÛËχÙËÎ·Ó ÛÙË ª∂£ ÌÂÙ¿ ÙÔ 1990. ™Â 8 ·È‰È¿ ¯ÚËÛÈÌÔÔÈ‹ıËΠÙÔ Û¯‹Ì· 0,4 g/kg/Ë̤ڷ ÁÈ· ¤ÓÙÂ Û˘Ó¯›˜ Ë̤Ú˜ Î·È Û ‰‡Ô ·È‰È¿ ÙÔ Û¯‹Ì· 1 g/kg/Ë̤ڷ ÁÈ· ‰‡Ô Û˘Ó¯›˜ Ë̤Ú˜. ™Â ¤Ó· ·fi ·˘Ù¿ Ù· ‰‡Ô ·È‰È¿ ÙÔ Û¯‹Ì· Â·Ó·Ï‹ÊıËΠÌÂÙ¿ ·fi 15 Ë̤Ú˜, ¯ˆÚ›˜ ı·̷ÙÈο ·ÔÙÂϤÛÌ·Ù·. ∏ ˘fiÏÔÈË ·ÁˆÁ‹ ÂÚÈÂÏ¿Ì‚·Ó ·Ó·Ó¢ÛÙÈ΋ Î·È ÎÈÓËÙÈ΋ Ê˘ÛÈÔıÂÚ·›·, ÚfiÏË„Ë Î·È ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÏÔÈÌÒ͈Ó, ·ÓÙÈÌÂÙÒÈÛË fiÓÔ˘, „˘¯ÔÏÔÁÈ΋ ˘ÔÛÙ‹ÚÈÍË, ηıÒ˜ Î·È ÂÓÙÂÚÈ΋ Û›ÙÈÛË. ¶ÔÚ›· ÓfiÛÔ˘ - ŒÎ‚·ÛË - ∂ÈÏÔΤ˜ ∏ ‰È¿ÚÎÂÈ· ·Ú·ÌÔÓ‹˜ ÙˆÓ ·ÛıÂÓÒÓ ÛÙË ª∂£ Î˘Ì¿ÓıËΠ·fi 2 ¤ˆ˜ 140 Ë̤Ú˜. ∆Ô ·È‰› Ô˘ ·Ú¤ÌÂÈÓ 140 Ë̤Ú˜ ›¯Â ·ÚÔ˘ÛÈ¿ÛÂÈ Î·Ú‰ÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó·ÎÔ‹ Î·È ÂÈ‚›ˆÛ Ì ‚·ÚÈ¿ Ó¢ÚÔÏÔÁÈ΋ Û˘Ó‰ÚÔÌ‹ (‚·ÚÈ¿ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·). ∂¿Ó ÂÍ·ÈÚÂı› ·˘Ùfi ÙÔ ·È‰›, Ë Ì¤ÛË ‰È¿ÚÎÂÈ· ÓÔÛËÏ›·˜ ÛÙË ª∂£ ‹Ù·Ó 24,8 Ë̤Ú˜. ∫ÚÈÙ‹ÚÈ· ÁÈ· ÙËÓ ¤ÍÔ‰Ô ÙˆÓ ·ÛıÂÓÒÓ ·fi ÙË ª∂£ ·ÔÙÂÏÔ‡Û·Ó Ë ·Ó·Ó¢ÛÙÈ΋ Â¿ÚÎÂÈ·, Ô ·ÔÙÂÏÂÛÌ·ÙÈÎfi˜ ‚‹¯·˜ Î·È Ë ·Ô˘Û›· Ïԛ̈͢. ∏ ·ÔηٿÛÙ·ÛË Ù˘ Ì˘˚΋˜ ‰‡Ó·Ì˘ ‰ÂÓ ·ÔÙÂÏÔ‡Û ··Ú·›ÙËÙË ÚÔ¸fiıÂÛË. ™ÙË Û˘Ó¤¯ÂÈ·, Ù· ·È‰È¿ ÓÔÛËχÙËÎ·Ó ÛÙȘ ·È‰È·ÙÚÈΤ˜ ÎÏÈÓÈΤ˜. ∏ Û˘ÓÔÏÈ΋ ·Ú·ÌÔÓ‹ ÙÔ˘˜ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô Î˘Ì¿ÓıËΠ·fi 16 ¤ˆ˜ 75 Ë̤Ú˜. ªÂÙ¿ ÙËÓ ¤ÍÔ‰fi ÙÔ˘˜ ·fi ÙÔ ÓÔÛÔÎÔÌ›Ô, Ù· ·È‰È¿ ·Ú·ÎÔÏÔ˘ı‹ıËÎ·Ó ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ̤¯ÚÈ ÙËÓ Ï‹ÚË ·ÔηٿÛÙ·Û‹ ÙÔ˘˜. ™ÙÔ˘˜ 6 Ì‹Ó˜, Ù· 10/12 ·È‰È¿ ÂÚ·ÙÔ‡Û·Ó ¯ˆÚ›˜ ‚Ô‹ıÂÈ· (score <2). ∆· ˘fiÏÔÈ· 2 ·È‰È¿ ·ÚÔ˘Û›·˙·Ó Ó¢ÚÔÏÔÁÈο ˘ÔÏ›ÌÌ·Ù·. ∆Ô ¤Ó· ·È‰›, Ô˘ ›¯Â ˘ÔÛÙ› ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó·ÎÔ‹ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔ¶·È‰È·ÙÚÈ΋ 2005;68:352-356

ÛËÏ›·˜, ·Ú¤ÌÂÈÓ Ì ÂÈÎfiÓ· ‚·ÚÈ¿˜ ˘ÔÍ·ÈÌÈ΋˜-ÈÛ¯·ÈÌÈ΋˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜. ∆Ô ‰Â‡ÙÂÚÔ ·È‰› ›¯Â ‰È·Ù·Ú·¯¤˜ ‚¿‰ÈÛ˘ (·ÛÙ¿ıÂÈ· ‚¿‰ÈÛ˘). ∫·Ó¤Ó· ·È‰› ‰ÂÓ ·‚›ˆÛ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘ ÛÙË ª∂£.

™˘˙‹ÙËÛË ∆Ô Û‡Ó‰ÚÔÌÔ Guillain-Barré ıˆÚÂ›Ù·È fiÙÈ ÔÊ›ÏÂÙ·È Û ·ÓÔÛÔÏÔÁÈ΋ ‰È·Ù·Ú·¯‹, Ì ·ÔÙ¤ÏÂÛÌ· ÙË ÊÏÂÁÌÔÓÒ‰Ë ‰È‹ıËÛË ÙÔ˘ ÂÓ‰ÔÓ¢ڛԢ, ÙÔ Ô›‰ËÌ· Î·È ÙËÓ ÙÌËÌ·ÙÈ΋ ·ÔÌ˘ÂϛӈÛË (3). ™ÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÏÔÈÌÔÁfiÓÔÈ ·Ú¿ÁÔÓÙ˜, ηÎÔ‹ıÂȘ, ¯ÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ Î·È ‰È¿ÊÔÚ· Ê¿Ú̷η. ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ˘‹Ú¯Â ıÂÙÈÎfi ÈÛÙÔÚÈÎfi ÁÈ· ÚÔËÁÔ‡ÌÂÓË ÈÔÁÂÓ‹ Ïԛ̈ÍË Û 8/12 ·È‰È¿, ÂÓÒ Û 6/12 ·È‰È¿ ‚Ú¤ıËÎÂ Ô Èı·Ófi˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜. ∏ ·ÚÈ· ÎÏÈÓÈ΋ ÂΉ‹ÏˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ GB Â›Ó·È Ë Ì˘˚΋ ·‰˘Ó·Ì›·, Ô˘ Â›Ó·È Û˘ÌÌÂÙÚÈ΋ Î·È ÍÂÎÈÓ¿ÂÈ Û˘Ó‹ıˆ˜ ·fi Ù· οو ¿ÎÚ·, ¤¯ÔÓÙ·˜ ·ÓÈÔ‡Û· ÔÚ›·. ∏ Ì˘˚΋ ·‰˘Ó·Ì›· Û˘Óԉ‡ÂÙ·È ·fi ÙË Ì›ˆÛË/ηٿÚÁËÛË ÙˆÓ ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ (3). ∆· ·È‰È¿ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ›¯·Ó fiÏ· Ì˘˚΋ ·‰˘Ó·Ì›· (score ‚·Ú‡ÙËÙ·˜ 4-5 Ì ÙËÓ Îϛ̷η Hughes) Î·È Ï‹ÚË Î·Ù¿ÚÁËÛË ÙˆÓ ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ. ∂›Û˘, fiÏ· Ù· ·È‰È¿ ·ÚÔ˘Û›·Û·Ó ÚÔÛ‚ÔÏ‹ ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Ì˘ÒÓ, Ì Â·ÎfiÏÔ˘ıÔ ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ·. ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È fiÙÈ ÂÚ›Ô˘ 2030% ÙˆÓ ·ÛıÂÓÒÓ (·È‰È¿ Î·È ÂÓ‹ÏÈΘ) ·ÚÔ˘ÛÈ¿˙ÂÈ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ· Î·È ı· ¯ÚÂÈ·ÛÙ› Ì˯·ÓÈ΋ ˘ÔÛÙ‹ÚÈÍË Ù˘ ·Ó·ÓÔ‹˜ (4,5). ¢ÂÓ ˘¿Ú¯Ô˘Ó ·Ó¿ÏÔÁ˜ ·Ó·ÊÔÚ¤˜ ÁÈ· ·È‰È¿ Ô˘ ÓÔÛËχÙËÎ·Ó Û ª∂£. ∏ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ· Ì·˙› Ì ÙȘ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Â›Ó·È Ù· ·ÚÈ· ·›ÙÈ· ÂÈÛ·ÁˆÁÒÓ Û ª∂£ (5). ∞fi ÙÔ˘˜ ÂÚÈÛÛfiÙÂÚÔ˘˜ Û˘ÁÁÚ·Ê›˜ Û˘ÓÈÛÙ¿Ù·È ÔÈ ·ÛıÂÓ›˜ Ó· ‰È·ÛˆÏËÓÒÓÔÓÙ·È ÓˆÚ›˜, ÚÈÓ ÂÁηٷÛÙ·ı› Ë ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ· (3,5,6). ∏ ‰È·ÎÔ‹ ÙÔ˘ ª∞ ÂȯÂÈÚÂ›Ù·È ÛÙ·‰È·Î¿, fiÙ·Ó Ë ˙ˆÙÈ΋ ¯ˆÚËÙÈÎfiÙËÙ· Â›Ó·È >10 ml/kg, Ë ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋ Î·È ¤¯Ô˘Ó Â·Ó¤ÏıÂÈ Ô ‚‹¯·˜ Î·È Ù· ÚÔÛٷ٢ÙÈο ·ÓÙ·Ó·ÎÏ·ÛÙÈο ÙˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ (3). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, Ë ‰È¿ÚÎÂÈ· Ù˘ Ì˯·ÓÈ΋˜ ˘ÔÛÙ‹ÚÈ͢ Î˘Ì¿ÓıËΠ·fi 2 ¤ˆ˜ 37 Ë̤Ú˜ (̤ÛÔ˜ fiÚÔ˜: 18,4 Ë̤Ú˜). ™Â ·ÓÙ›ÛÙÔȯ˜

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·355

355

™‡Ó‰ÚÔÌÔ Guillain-Barré

ÌÂϤÙ˜ ·È‰ÈÒÓ ·Ó·Ê¤ÚÔÓÙ·È ·ÚfiÌÔÈ· ÛÙÔȯ›· (6), ÂÓÒ Ë ‰È¿ÚÎÂÈ· Ù˘ Ì˯·ÓÈ΋˜ ˘ÔÛÙ‹ÚÈ͢ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ ‰È·ÚΛ ÂÚÈÛÛfiÙÂÚÔ (1). ™ÙÔ Û‡ÓÔÏÔ ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔ GB, Ë ÚÔÛ‚ÔÏ‹ ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ ÓÂ‡ÚˆÓ ·Ó·Ê¤ÚÂÙ·È Û ÔÛÔÛÙfi ÂÚ›Ô˘ 35-45% (4). ∞fi Ù· ·È‰È¿ Ù˘ ÛÂÈÚ¿˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, ÚÔÛ‚ÔÏ‹ ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ ÓÂ‡ÚˆÓ ·ÚÔ˘Û›·Û ÙÔ 91,6% (11/12) Î·È ‹Ù·Ó ¿ÓÙ· ·Ó·ÛÙÚ¤„ÈÌË. ¢ÂÓ ˘¿Ú¯Ô˘Ó ·Ó¿ÏÔÁ˜ ·Ó·ÊÔÚ¤˜ ·fi ¿ÏϘ ª∂£. º·›ÓÂÙ·È fiÙÈ fiÛÔ ÌÂÁ·Ï‡ÙÂÚË Â›Ó·È Ë ‚·Ú‡ÙËÙ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, ÙfiÛÔ Û˘¯ÓfiÙÂÚË Â›Ó·È Î·È Ë ÚÔÛ‚ÔÏ‹ ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ Ó‡ڈÓ.

OÈ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ∞¡™ ‹Ù·Ó Û˘¯Ó¤˜ (9/12 ·È‰È¿) Î·È ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È ˆ˜ ‰Â‡ÙÂÚË ·ÈÙ›· ÂÈÛ·ÁˆÁ‹˜ ·ÛıÂÓÒÓ Ì ۇӉÚÔÌÔ GB ÛÙË ª∂£. ∏ ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó·ÎÔ‹ Ô˘ ·Ú·ÙËÚ‹ıËΠ۠¤Ó·Ó ·ÛıÂÓ‹ ‹Ù·Ó ·ÔÙ¤ÏÂÛÌ· ˘ÔÍ·ÈÌ›·˜ Î·È ‰ÂÓ ÔÊÂÈÏfiÙ·Ó Û ‰È·Ù·Ú·¯‹ ÙÔ˘ ∞¡™ (.¯. ·ÚÚ˘ıÌ›·), ·Ó Î·È ·˘Ùfi ·Ó·Ê¤ÚÂÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· (6). ∞fi ÙȘ ÂΉËÏÒÛÂȘ ·fi ÙÔ ∞¡™, Û˘¯ÓfiÙÂÚ˜ ‹Ù·Ó Ë ˘¤ÚÙ·ÛË, Ë ˘fiÙ·ÛË Î·È ÔÈ ÂÓ·ÏÏ·Á¤˜ ˘¤ÚÙ·Û˘-˘fiÙ·Û˘. ™·Ó ÂȉÈ΋ ıÂÚ·›· ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ GB ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› Ë ÎÔÚÙÈ˙fiÓË, Ë Ï·ÛÌ·Ê·›ÚÂÛË Î·È Ë IVIG. ∏ ¯ÔÚ‹ÁËÛË ÎÔÚÙÈ˙fiÓ˘ ¤¯ÂÈ ÂÁηٷÏÂÈÊı›, ηıÒ˜ ¤¯ÂÈ ·Ô‰ÂȯÙ› fiÙÈ ‰ÂÓ ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÚfiÁÓˆÛË. ∏ Ï·ÛÌ·Ê·›ÚÂÛË - ·Ó Î·È ·Ó·Ê¤ÚÔÓÙ·È Î·Ï¿ ·ÔÙÂϤÛÌ·Ù· - ‰ÂÓ ÚÔÙÈÌ¿Ù·È ÛÙ· ·È‰È¿ ÏfiÁˆ Ù¯ÓÈÎÒÓ ‰˘ÛÎÔÏÈÒÓ. ∏ IVIG ÂÊ·ÚÌfi˙ÂÙ·È ÂÎÙÂٷ̤ӷ ÙËÓ ÙÂÏÂ˘Ù·›· ‰ÂηÂÙ›·. OÈ ÂÚÈÛÛfiÙÂÚÔÈ ÂÚ¢ÓËÙ¤˜ ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ ¤¯ÂÈ Î·Ï¿ ·ÔÙÂϤÛÌ·Ù·, ‰ÈfiÙÈ ÂÏ·ÙÙÒÓÂÈ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÔͤԘ ÛÙ·‰›Ô˘, ‚ÂÏÙÈÒÓÂÈ ÙË Ì˘˚΋ ÈÛ¯‡ Î·È ‚Ú·¯‡ÓÂÈ ÙÔ ‰È¿ÛÙËÌ· ÙÔ˘ ª∞ Î·È Ù˘ ·Ú·ÌÔÓ‹˜ ÙÔ˘ ·ÛıÂÓ‹ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô (3,7). OÈ ÌÂϤÙ˜, fï˜, ·˘Ù¤˜ ·ÊÔÚÔ‡Ó Î˘Ú›ˆ˜ Û ÂÓ‹ÏÈΘ. ™Ù· ·È‰È¿ ‰ÂÓ ˘¿Ú¯Ô˘Ó ÌÂÁ¿Ï˜ ÛÂÈÚ¤˜ Ô˘ Ó· ÂȂ‚·ÈÒÓÔ˘Ó ÙËÓ ·Í›· Ù˘ ¯ÔÚ‹ÁËÛ˘ IVIG (8,9). ªÂÚÈÎÔ› Û˘ÁÁÚ·Ê›˜ ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ Ë ¯ÔÚ‹ÁËÛË IVIG Û ·È‰È¿ ÌÔÚ› Ó· Â›Ó·È ÏÈÁfiÙÂÚÔ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·fi ÙÔ˘˜ ÂÓ‹ÏÈΘ ‹ Ó· Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÌfiÓÔ fiÙ·Ó ¯ÔÚËÁËı› ÛÂ Û˘ÁÎÂÎÚÈ̤ÓÔ˘˜ ·ÛıÂÓ›˜ Î·È Ôχ ÓˆÚ›˜ ÛÙËÓ ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘ (8). O Û˘Ó‰˘·ÛÌfi˜ IVIG Î·È Ï·ÛÌ·Ê·›ÚÂÛ˘ ‰ÂÓ ¤¯ÂÈ ÛËÌ·ÓÙÈο ηχÙÂÚ· ·ÔÙÂϤÛÌ·Ù· (10). ™Â fi,ÙÈ ·ÊÔÚ¿ ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘, ·˘Ù‹ Â›Ó·È Î·Ï‹ ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛˆÓ. ∆Ô 80-85% ÙˆÓ ·ÛıÂÓÒÓ ¤¯ÂÈ Ï‹ÚË ·Ôηٿ-

ÛÙ·ÛË. ∆Ô ÔÛÔÛÙfi Ù˘ ıÓËÙfiÙËÙ·˜, ·Ú¿ ÙȘ ÚÔfi‰Ô˘˜ ÛÙËÓ ÂÓÙ·ÙÈ΋ ÓÔÛËÏ›·, ÂÍ·ÎÔÏÔ˘ı› Ó· Î˘Ì·›ÓÂÙ·È Û 2-12%, ÂÓÒ ÙÔ 10-15% ÙˆÓ ·ÛıÂÓÒÓ ÂÌÊ·Ó›˙ÂÈ ÌfiÓÈÌË ·Ó·ËÚ›· (1,3,4). ÀÔÙÚÔ‹ - ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓfiÛÔ˘ ‹ ·ÚÁfiÙÂÚ· - ·ÚÔ˘ÛÈ¿˙ÂÈ ÙÔ 3-10% ÙˆÓ ·ÛıÂÓÒÓ. ™Â ª∞ Ô‰ËÁÂ›Ù·È ÙÔ 20-30% ÙˆÓ ·ÛıÂÓÒÓ Î·È ıˆÚÂ›Ù·È ÂÈ‚·Ú˘ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ì ıÓËÙfiÙËÙ· Ô˘ ÊÙ¿ÓÂÈ ÙÔ 20% (1). ∆· ÛÙÔȯ›· ÚÔ¤Ú¯ÔÓÙ·È Î˘Ú›ˆ˜ ·fi ÛÂÈÚ¤˜ ÂÓËÏ›ÎˆÓ Î·È Â›Ó·È ÁÂÓÈÎÒ˜ ·Ô‰ÂÎÙfi fiÙÈ ÛÙ· ·È‰È¿ Ë ÚfiÁÓˆÛË Â›Ó·È Î·Ï‡ÙÂÚË. ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, Ô˘ ·ÊÔÚÔ‡Û Û ·È‰È¿ Ì ‚·Ú‡ Û‡Ó‰ÚÔÌÔ Guillain-Barré, ‰ÂÓ ˘‹Ú¯Â ηӤӷ˜ ı¿Ó·ÙÔ˜, fï˜ ‰‡Ô ·È‰È¿ ÂÌÊ¿ÓÈÛ·Ó ÌfiÓÈ̘ Ó¢ÚÔÏÔÁÈΤ˜ ÂÈÏÔΤ˜. ™ÙÔ ÚÒÙÔ ·fi ·˘Ù¿, Ë Ó¢ÚÔÏÔÁÈ΋ Û˘Ó‰ÚÔÌ‹ ‰ÂÓ ÔÊÂÈÏfiÙ·Ó ÛÙËÓ ›‰È· ÙËÓ ¿ıËÛË, ·ÏÏ¿ ÛÙËÓ Î·Ú‰ÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó·ÎÔ‹ Ô˘ ˘¤ÛÙË. ∆Ô ‰Â‡ÙÂÚÔ ·È‰› ›¯Â ·ÚÔ˘ÛÈ¿ÛÂÈ ‹‰Ë ‰‡Ô ˘ÔÙÚÔ¤˜ fiÙ·Ó ÓÔÛËχÙËΠÛÙÔ ÙÌ‹Ì·. O ¯ÚfiÓÔ˜ ·ÔηٿÛÙ·Û˘ ÙÔ˘ Ó¢ÚÔÏÔÁÈÎÔ‡ ˘ÔÏ›ÌÌ·ÙÔ˜ Â›Ó·È ÈÔ Û‡ÓÙÔÌÔ˜ ÛÙ· ·È‰È¿ ·fi fi,ÙÈ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ (1,8). ∏ ‰È¿ÚÎÂÈ· Ù˘ ·ÔηٿÛÙ·Û˘ ÛÙËÓ ÔÌ¿‰· ÙˆÓ ·È‰ÈÒÓ Ì ηϋ ¤Î‚·ÛË Î˘Ì¿ÓıËΠ·fi 24 ¤ˆ˜ 79 Ë̤Ú˜ (̤ÛÔ˜ fiÚÔ˜: 51,7 Ë̤Ú˜) Î·È ‹Ù·Ó ÈÔ Û‡ÓÙÔÌË ·fi ÂΛÓË Ô˘ ·Ó·Ê¤ÚÂÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· (4,8).

™˘ÌÂÚ¿ÛÌ·Ù· ∆Ô Û‡Ó‰ÚÔÌÔ Guillain-Barré ›¯Â Û¯ÂÙÈο ηϋ ¤Î‚·ÛË ÛÙ· ·È‰È¿ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ Ô˘ ÓÔÛËχÙËÎ·Ó ÛÙË ª∂£, ·ÎfiÌ· Î·È ÛÙȘ ÂÚÈÙÒÛÂȘ Ô˘ ··ÈÙ‹ıËΠÌ˯·ÓÈ΋ ˘ÔÛÙ‹ÚÈÍË Ù˘ ·Ó·ÓÔ‹˜ ÏfiÁˆ Ù˘ ‚·Ú‡ÙËÙ·˜ Ù˘ ÓfiÛÔ˘. ∏ ‰È·ÛˆÏ‹ÓˆÛË Ú¤ÂÈ Ó· ·ÔÊ·Û›˙ÂÙ·È ÂÁη›Úˆ˜, Ì ÙËÓ ÂÌÊ¿ÓÈÛË ·Ó·ÔÙÂÏÂÛÌ·ÙÈÎÔ‡ ‚‹¯·, Ì˘˚΋˜ ·‰˘Ó·Ì›·˜ ·Ó·Ó¢ÛÙÈÎÒÓ Ì˘ÒÓ Î·È ÚÔÛ‚ÔÏ‹ ÎÚ·ÓÈ·ÎÒÓ Ó‡ڈÓ. ∞Ó Î·È ‰ÂÓ ¤¯ÂÈ ·Ô‰ÂȯÙ› ·fi ÙȘ ÌÂϤÙ˜ fiÙÈ Ë ¯ÔÚ‹ÁËÛË IVIG ÛÙ· ·È‰È¿ Â›Ó·È ÙfiÛÔ ·ÔÙÂÏÂÛÌ·ÙÈ΋ fiÛÔ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, ·Ú·Ì¤ÓÂÈ Ë ÚÒÙË ÂÈÏÔÁ‹ Û·Ó ÂȉÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÓfiÛÔ˘.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Fletcher DD, Lawn ND, Wolter TD, Wijdicks EF. Long-term outcome in patients with Guillain-Barré ¶·È‰È·ÙÚÈ΋ 2005;68:352-356

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Bos AP, van der Meche FG, Witsenburg M, van der Voort E. Experiences with Guillain-Barré syndrome in a pediatric intensive care unit. Intensive Care Med 1987;13:328-331. van der Meche FG, Schmitz PI. Dutch Guillain-Barré Study Group. A randomized trial comparing intravenous immune globulin and plasma exchange in

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Severe Guillain-Barré syndrome. Outcome in children admitted to a PICU A. Tsoutsou, D. Gionis, S. Mastroyianni, J. Papadatos

Abstract Background: Guillain-Barré syndrome (GBS) is a progressive symmetrical polyneuritis with acute onset, evolution over days or weeks and a good outcome in most cases. The aim of this study was to analyze the clinical course and outcome in children with GBS requiring hospitalization in a Paediatric Intensive Care Unit (PICU).

Methods: The medical records of all children admitted to the PICU between January 1986 and January 2003 were reviewed. The clinical and laboratory features, therapy and outcome were recorded.

Intensive Care Unit, “P. & A. Kyriakou” Children’s Hospital, Athens Correspondence: Dimitrios Gionis “P. & A. Kyriakou” Children’s Hospital, Thivon & Levadias Str. 115 27, Athens Date of submission: 10-12-2003 Date of approval: 08-07-2005

Results: Twelve children, with GBS, 5 males, mean age 8.04 years (range 22 months - 15 years), were admitted to the PICU. In 8/12 patients a preceding viral illness was reported. In 10/12 patients serological investigations were performed and the aetiological factor was found in 6/10. In addition to weakness and loss of tendon reflexes, 11/12 children presented with cranial nerve palsy and 9/12 had autonomic dysfunction (mainly hypertension). All patients had a severity score (Hughes scale) of 4 or 5. A lumbar puncture was performed in all patients, with positive findings when it was done 7 days or more after the onset of symptoms. Eleven of the 12 patients required mechanical ventilation for a mean period of 18.4 days (range 2-37 days) and 10 were given intravenous immunoglobulin (IVIG). The mean period of PICU hospitalization was 24.8 days (range 2-42 days). None of the patients died, 10 had full recovery but two patients had neurological sequelae.

Conclusions: Guillain-Barré syndrome has a better outcome in children than in adults. Mechanical ventilation is not a risk factor for an adverse outcome.

Key words Guillain-Barré syndrome, mechanical ventilation, intravenous immunoglobulin.

Paediatriki 2005;68:357

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∫›Ó‰˘ÓÔ˜ ·fi ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ·ÙfiÌˆÓ Ì ۇӉÚÔÌÔ Down Î·È ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ· 1 ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£., “πÔÎÚ¿ÙÂÈÔ” °ÂÓÈÎfi ¡ÔÛÔÎÔÌ›Ô, £ÂÛÛ·ÏÔÓ›ÎË 2 ¶·È‰Ô·ÎÙÈÓÔÏÔÁÈÎfi ∆Ì‹Ì·, “πÔÎÚ¿ÙÂÈÔ” °ÂÓÈÎfi ¡ÔÛÔÎÔÌ›Ô, £ÂÛÛ·ÏÔÓ›ÎË AÏÏËÏÔÁÚ·Ê›·: Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡-§Ô˘Î›‰Ô˘ ∂ÚÁ·ÛÙ‹ÚÈÔ ∫˘ÙÙ·ÚÔÁÂÓÂÙÈ΋˜, ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£., “πÔÎÚ¿ÙÂÈÔ” °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ∫ˆÓÛÙ·ÓÙÈÓÔ˘fiψ˜ 49 ∆.∫. 546 42, £ÂÛÛ·ÏÔÓ›ÎË ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 03-09-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 29-03-2005

Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘1, ª. ª·ÓÙÔ˘Ú¿ÎË2, ¶. ∫·ÌÔ˘Ú›‰Ô˘1, ∂. ™ˆÙËÚ›‰Ô˘1

¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: ™ÎÔfi Ù˘ ÂÚÁ·Û›·˜ ·ÔÙ¤ÏÂÛÂ Ë ‰ÈÂÚ‡ÓËÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋˜ ·ÛÙ¿ıÂÈ·˜ (∞∞∞) Û ÌË È‰Ú˘Ì·ÙÈο ¿ÙÔÌ· Ì ۇӉÚÔÌÔ Down (DS) ÛÙË µfiÚÂÈ· ∂ÏÏ¿‰· Î·È Ë ÎÏÈÓÈ΋ ·Ú·ÎÔÏÔ‡ıËÛ‹ ÙÔ˘˜. ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ: ∆Ô ˘ÏÈÎfi ·ÔÙ¤ÏÂÛ·Ó 171 ¿ÙÔÌ· Ì DS ËÏÈΛ·˜ 0-40 ÂÙÒÓ. ŸÏ· ÂÍÂÙ¿ÛÙËÎ·Ó ÎÏÈÓÈο ·Ó¿ ¤ÙÔ˜ ÁÈ· ÛËÌÂÈÔÏÔÁ›· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∞fi Ù· ¿ÙÔÌ· ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 3 ÂÙÒÓ, ÔÛÔÛÙfi 81% ˘Ô‚Ï‹ıËΠ۠·ÎÙÈÓÔÁڷʛ˜ (·/·) ·˘¯ÂÓÈ΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ (∞ª™™), Û Ï¿ÁÈ· Ô˘‰¤ÙÂÚË ı¤ÛË Î·È ÂÈÏÂÎÙÈο Û Ï¿ÁÈ· ο̄Ë. ∏ ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·fiÛÙ·ÛË ÂÎÙÈÌ‹ıËΠˆ˜ ·ıÔÏÔÁÈ΋ fiÙ·Ó ‹Ù·Ó ¿Óˆ ÙˆÓ 4 mm ÁÈ· Ù· ·È‰È¿ ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 15 ÂÙÒÓ Î·È ¿Óˆ ÙˆÓ 3 mm ÁÈ· Ù· ¿ÙÔÌ· ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 15 ÂÙÒÓ. ŸÏ˜ ÔÈ ·ÎÙÈÓÔÁڷʛ˜ ·ÍÈÔÏÔÁ‹ıËÎ·Ó ·fi ÙËÓ ›‰È· ·ÎÙÈÓÔÏfiÁÔ. ∞ÔÙÂϤÛÌ·Ù·: ∏ Û˘¯ÓfiÙËÙ· Ù˘ ∞∞∞ ‹Ù·Ó Ôχ ˘„ËÏ‹ (42%) ÛÙ· ˘fi ¤Ú¢ӷ ¿ÙÔÌ· Ì DS, Û˘¯ÓfiÙÂÚË ‹Ù·Ó ÛÙ· ·ÁfiÚÈ· (56%) Î·È ÛÙ· ¿ÙÔÌ· ËÏÈΛ·˜ οو ÙˆÓ 15 ÂÙÒÓ (63%). ¶ÔÛÔÛÙfi 9,3% ›¯Â ˘ÔÎÂÈÌÂÓÈο Ó¢ÚÔÏÔÁÈο Û˘ÌÙÒÌ·Ù· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ÀÔ‚Ï‹ıËÎ·Ó Û ̷ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· (MRI) ∞ª™™ Î·È Û ηӤӷ ‰ÂÓ ‰È·ÈÛÙÒıËΠ›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ™˘ÌÂÚ¿ÛÌ·Ù·: ∆· Â˘Ú‹Ì·Ù· Ù˘ ¤Ú¢ӷ˜ ÂÓÈÛ¯‡Ô˘Ó ÙȘ Ô‰ËÁ›Â˜ Ù˘ ∞ıÏËÙÈ·ÙÚÈ΋˜ ∂ÈÙÚÔ‹˜ Ù˘ ∞ÌÂÚÈηÓÈ΋˜ ∞η‰ËÌ›·˜ Ù˘ ¶·È‰È·ÙÚÈ΋˜ (∞∞¶), Ô˘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ¤ÏÂÁ¯Ô ÙˆÓ ·È‰ÈÒÓ Ì DS ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 3 ÂÙÒÓ Ì ·/· ∞ª™™ ηٿ ÙËÓ ÚÒÙË Â›Û΄‹ ÙÔ˘˜ ÛÙÔÓ ·È‰›·ÙÚÔ, Û˘ÛÙ¿ÛÂȘ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÁÈ· Û˘ÌÌÂÙÔ¯‹ fiÏˆÓ ·˘ÙÒÓ ÙˆÓ ·È‰ÈÒÓ ÛÙ· ·ıÏ‹Ì·Ù· Ì ·ÔÊ˘Á‹ ˘¤Úη̄˘-˘ÂÚ¤ÎÙ·Û˘ ÙÔ˘ ·˘¯¤Ó·, Â·Ó¿ÏË„Ë Ù˘ ·ÎÙÈÓÔÁÚ·Ê›·˜ Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ· ÚÈÓ ÙË Û˘ÌÌÂÙÔ¯‹ Û ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ Î·È ÎÏÈÓÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË ÁÈ· Û˘ÌÙÒÌ·Ù· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡ (·È̈‰›Â˜, ÔÓÔÎÂÊ¿ÏÔ˘˜, fiÓÔ ÛÙÔÓ ·˘¯¤Ó·, ‰È·Ù·Ú·¯‹ ÏÂÈÙÔ˘ÚÁ›·˜ Ù˘ ·ÛÙ˘, ‰È·Ù·Ú·¯‹ ÛÙË ‚¿‰ÈÛË) ·fi ÙÔÓ ÎÏÈÓÈÎfi ÁÈ·ÙÚfi. ∏ ÛËÌÂÈÔÏÔÁ›· Ù˘ ÎÏÈÓÈ΋˜ ÂͤٷÛ˘ ·ÔÙÂÏ› ÙÔ ÎÚÈÙ‹ÚÈÔ Â·Ó¿Ï˄˘ Î·È ‰È‡ڢÓÛ˘ ÙÔ˘ ·ÂÈÎÔÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘.

§¤ÍÂȘ ÎÏÂȉȿ ™‡Ó‰ÚÔÌÔ Down, ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ·, ·ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ˘ÂÍ¿ÚıÚËÌ·, ·ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ÂÍ¿ÚıÚËÌ·, ·ÎÙÈÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜, Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·.

™˘ÓÙÔÌÔÁڷʛ˜ DS ∞∞∞ C-1 C-2 ∞ª™™ MRI CT ∞∞¶ ¶·È‰È·ÙÚÈ΋ 2005;68:358-364

™‡Ó‰ÚÔÌÔ Down ∞ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ· ¶ÚÒÙÔ˜ ·˘¯ÂÓÈÎfi˜ ÛfiÓ‰˘ÏÔ˜ ¢Â‡ÙÂÚÔ˜ ·˘¯ÂÓÈÎfi˜ ÛfiÓ‰˘ÏÔ˜ ∞˘¯ÂÓÈ΋ ÌÔ›Ú· ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ ª·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ∞ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· Ù˘ ¶·È‰È·ÙÚÈ΋˜

AAP ·/· AAI ™™

American Academy of Pediatrics ∞ÎÙÈÓÔÁÚ·Ê›· Atlantoaxial instability ™ÔÓ‰˘ÏÈ΋ ÛÙ‹ÏË

∂ÈÛ·ÁˆÁ‹ ∆· ·È‰È¿ Ì ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË ¤¯Ô˘Ó ·Ó¿ÁÎË ·fi ·˘ÍË̤ÓË ÚÔÛÔ¯‹, ÙfiÛÔ ÁÈ· ÙËÓ ÚÔ·ÁˆÁ‹ Ù˘ ˘Á›·˜ ÙÔ˘˜ fiÛÔ Î·È ÁÈ· ÙËÓ ÚfiÏË„Ë

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·359

359

™‡Ó‰ÚÔÌÔ Down Î·È ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ·

ۈ̷ÙÈÎÒÓ ·Ó·ËÚÈÒÓ. ¶ÈÔ Û˘ÁÎÂÎÚÈ̤ӷ, Ú¤ÂÈ Ó· ·ÔʇÁÔÓÙ·È ÓÔÛ‹Ì·Ù· ‹ ·Ó·Ëڛ˜, ÔÈ Ôԛ˜ ÌÔÚ› Ó· Â·˘Í‹ÛÔ˘Ó Ù· ÚÔ‚Ï‹Ì·Ù· Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙË ÓÔËÙÈ΋ ÙÔ˘˜ ˘ÛÙ¤ÚËÛË (1). ŒÓ· ÂȉÈÎfi Úfi‚ÏËÌ· Â›Ó·È Ô Î›Ó‰˘ÓÔ˜ ÙˆÓ ·ÙfiÌˆÓ Ì ۇӉÚÔÌÔ Down (DS) ·fi ‰È¿ÊÔÚ˜ ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÏfiÁˆ Ù˘ ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋˜ ·ÛÙ¿ıÂÈ·˜ (∞∞∞) (1,2). ø˜ ∞∞∞ ‹ ·ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ˘ÂÍ¿ÚıÚËÌ· ¯·Ú·ÎÙËÚ›˙ÂÙ·È Ë ·˘ÍË̤ÓË ÎÈÓËÙÈÎfiÙËÙ· ÛÙËÓ ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ¿ÚıÚˆÛË [¿ÚıÚˆÛË ÌÂٷ͇ ÙÔ˘ ÚÒÙÔ˘ (C1) Î·È ÙÔ˘ ‰Â‡ÙÂÚÔ˘ (C-2) ·˘¯ÂÓÈÎÔ‡ ÛÔÓ‰‡ÏÔ˘] (∂ÈÎfiÓ· 1) (3,4). ∆· ·›ÙÈ· Ù˘ ∞∞∞ ‰ÂÓ ¤¯Ô˘Ó Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈÛÙ›. ªÔÚ› Ó· ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ‰È·Ì·Úٛ˜ ÙˆÓ Û˘Ó‰¤ÛÌˆÓ Ô˘ ‰È·ÙËÚÔ‡Ó ÙËÓ ·ÎÂÚ·ÈfiÙËÙ· Ù˘ ¿ÚıÚˆÛ˘ ÌÂٷ͇ ÙÔ˘ C-1 ‹ C-2 ÛÔÓ‰‡ÏÔ˘ ‹ ÔÛÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ C-1 - C-2 ‹ Î·È Ù· ‰‡Ô (5-7). ¶Ôχ Û¿ÓÈ· Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÚÔÎÏËı› ·ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ÂÍ¿ÚıÚËÌ·, ÙÔ ÔÔ›Ô Â›Ó·È ·ÂÈÏËÙÈÎfi ÁÈ· ÙË ˙ˆ‹ Î·È ÂÌÊ·Ó›˙ÂÙ·È Ì ÎÏÈÓÈο ÛËÌ›· Î·È Û˘ÌÙÒÌ·Ù· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡, fiˆ˜ ·ÓÒÌ·ÏÔ ‚¿‰ÈÛÌ·, fiÓÔ Î·È ÂÚÈÔÚÈṲ̂ÓË ÎÈÓËÙÈÎfiÙËÙ· ÙÔ˘ ·˘¯¤Ó·, Ú·È‚fiÎÚ·ÓÔ, ·‰ÂÍÈfiÙËÙ·, ·Û˘Ó¤ÚÁÂÈ· Î·È ‰È·Ù·Ú·¯¤˜ ÛÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ÛÊÈÁÎÙ‹ÚˆÓ. ∏ Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË ÌÔÚ› Ó· ·Ôηχ„ÂÈ ·ÈÛıËÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, Û·ÛÙÈÎfiÙËÙ·, ·˘ÍË̤ӷ ·ÓÙ·Ó·ÎÏ·ÛÙÈο, ÎÏfiÓÔ Î·È ÙËÓ ·ÚÔ˘Û›· ÙÔ˘ ÛËÌ›Ԣ Babinski (3,8). ™ÎÔfi˜ Ù˘ ÂÚÁ·Û›·˜ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ ∞∞∞ Û ÌË È‰Ú˘Ì·ÙÈο ¿ÙÔÌ· Ì DS ÛÙË µfiÚÂÈ· ∂ÏÏ¿‰· Î·È Ë ÎÏÈÓÈ΋ ·Ú·ÎÔÏÔ‡ıËÛ‹ ÙÔ˘˜ ·Ó¿ ¤ÙÔ˜.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∆Ô ˘ÏÈÎfi Ù˘ ÌÂϤÙ˘ ·ÔÙ¤ÏÂÛ·Ó 171 ¿ÙÔÌ· Ì DS ËÏÈΛ·˜ 0-40 ÂÙÒÓ, Ù· ÂÚÈÛÛfiÙÂÚ· ·fi Ù· ÔÔ›· ÂÚ¢-

Ó‹ıËÎ·Ó ¯ÚˆÌÔÛˆÌÈο ÛÙÔ ∂ÚÁ·ÛÙ‹ÚÈÔ ∫˘ÙÙ·ÚÔÁÂÓÂÙÈ΋˜ Ù˘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ∞.¶.£. ¶ÚÔÛ‹Ïı·Ó Ì ÚÔÁÚ·ÌÌ·ÙÈṲ̂ӷ Ú·ÓÙ‚ԇ ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· °ÂÓÂÙÈ΋˜ ∫·ıÔ‰‹ÁËÛ˘ ÁÈ· ÙÔÓ ÂÙ‹ÛÈÔ ÚÔÏËÙÈÎfi ÙÔ˘˜ ¤ÏÂÁ¯Ô, Û‡Ìʈӷ Ì ÙȘ Û˘ÛÙ¿ÛÂȘ Ù˘ ∞ÌÂÚÈηÓÈ΋˜ ∞η‰ËÌ›·˜ Ù˘ ¶·È‰È·ÙÚÈ΋˜ (A∞¶). ∂ÍÂÙ¿ÛÙËÎ·Ó ÎÏÈÓÈο ÁÈ· ÙËÓ ·Ó·˙‹ÙËÛË ÛËÌÂÈÔÏÔÁ›·˜ ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ™‡Ìʈӷ Ì ÙȘ Ô‰ËÁ›Â˜ Ù˘ ∞ıÏËÙÈ·ÙÚÈ΋˜ ∂ÈÙÚÔ‹˜ Ù˘ A∞¶, ˘Ô‚Ï‹ıËÎ·Ó Û ·/· Ù˘ ·˘¯ÂÓÈ΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ (∞ª™™) Ù· ¿ÙÔÌ· ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 3 ÂÙÒÓ. OÈ ·Ϥ˜ ·ÎÙÈÓÔÁڷʛ˜ ¤ÁÈÓ·Ó Ì Ì˯¿ÓËÌ· Merate MMT90 Û Ï¿ÁÈ· ÚÔ‚ÔÏ‹, Û Ԣ‰¤ÙÂÚË ı¤ÛË Î·È ÂÏ·ÊÚ¿ ο̄Ë, Ì ·fiÛÙ·ÛË Ï˘¯Ó›·˜ ·fi ÙËÓ Î·Û¤Ù· ÂÚ›Ô˘ 1 m Î·È Ì ÂÈΤÓÙÚˆÛË ÛÙÔ Â›Â‰Ô ∞3∞4 (ÌÂÛfiÙËÙ· ÂÚ›Ô˘ ∞ª™™). ∞ÎÙÈÓÔÏÔÁÈο, Ë ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·˘ÍË̤ÓË ·fiÛÙ·ÛË ÌÂٷ͇ Ù˘ Ô›ÛıÈ·˜ ÂÈÊ¿ÓÂÈ·˜ ÙÔ˘ ÚfiÛıÈÔ˘ ÙfiÍÔ˘ ÙÔ˘ ¿ÙÏ·ÓÙ· Î·È Ù˘ Ô‰ÔÓÙÔÂȉԇ˜ ·fiÊ˘Û˘. ¶·ıÔÏÔÁÈ΋ ÂÎÙÈÌ‹ıËÎÂ Ë ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·fiÛÙ·ÛË fiÙ·Ó ‹Ù·Ó ¿Óˆ ·fi 4 mm ÁÈ· Ù· ·È‰È¿ ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 15 ÂÙÒÓ Î·È ¿Óˆ ·fi 3 mm ÁÈ· Ù· ¿ÙÔÌ· ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 15 ÂÙÒÓ. ŸÏ˜ ÔÈ ·ÎÙÈÓÔÁڷʛ˜ ·ÍÈÔÏÔÁ‹ıËÎ·Ó ·fi ÙËÓ ›‰È· ·ÎÙÈÓÔÏfiÁÔ. ¢ÂÓ ˘‹Ú¯Â ÔÌ¿‰· Ì·ÚÙ‡ÚˆÓ Ô˘ ı· Û˘ÁÎÚ›ÓÔÓÙ·Ó Ì ٷ ¿ÙÔÌ· Ì DS. ∂ÈÏÂÎÙÈο, ÔÚÈṲ̂ӷ ·È‰È¿ ˘Ô‚Ï‹ıËÎ·Ó Û CT Î·È MRI. OÈ ÂÍÂÙ¿ÛÂȘ MRI ¤ÁÈÓ·Ó Ì ̷ÁÓËÙÈÎfi ÙÔÌÔÁÚ¿ÊÔ Siemens Expert plus, 1T Î·È Ï‹ÊıËÎ·Ó ÂÁοÚÛȘ Î·È Ô‚ÂÏÈ·›Â˜ ÙÔ̤˜ Ì ÂÈ‚¿Ú˘ÓÛË ∆1 Î·È ∆2 ·Ú·Ì¤ÙÚˆÓ Û Ԣ‰¤ÙÂÚË ı¤ÛË ÁÈ· ¤ÏÂÁ¯Ô ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. °È· ÙË ‰ÈÂÓ¤ÚÁÂÈ· Ù˘ ·/· ˘‹Ú¯Â Ë ¤ÁÎÚÈÛË fiÏˆÓ ÙˆÓ ÁÔÓ¤ˆÓ. ™ÙÔ˘˜ ÁÔÓ›˜ Ô˘ Ë ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·fiÛÙ·ÛË ÛÙËÓ ·/· ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘˜ ˘ÂÚ¤‚·ÈÓ ٷ Ê˘ÛÈÔÏÔÁÈο fiÚÈ·, ‰fiıËÎ·Ó Ô‰ËÁ›Â˜ ·ÔÊ˘Á‹˜ ÔÚÈÛÌ¤ÓˆÓ ı¤ÛÂˆÓ ÙÔ˘ ÛÒÌ·ÙÔ˜ Î·È Û˘ÁÎÂÎÚÈÌ¤ÓˆÓ ·ıÏËÌ¿ÙˆÓ ÁÈ· ÙËÓ ÚfiÏË„Ë ·Ù˘¯‹Ì·ÙÔ˜.

∞ÔÙÂϤÛÌ·Ù· ∆· 126/171 ¿ÙÔÌ· (74%) ‹Ù·Ó ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 3 ÂÙÒÓ Î·È Ù· 102 (81%) ·fi ·˘Ù¿ ˘Ô‚Ï‹ıËÎ·Ó Û Ï¿ÁÈ· ·ÎÙÈÓÔÁÚ·Ê›· ∞ª™™. ™Â 43/102 (42%) ‰È·ÈÛÙÒıËΠ∞∞∞ (∂ÈÎfiÓ· 2).

∫·Ï˘Ù‹ÚÈÔ˜ ˘Ì¤Ó·˜

ª¤ÛÔ˜ ·ÍÔÓÔÂȉ‹˜ ‹ ÎÚÂÌ·ÛÙ‹ÚÈÔ˜ Û‡Ó‰ÂÛÌÔ˜ ÙÔ˘ ¿ÙÏ·ÓÙ·

¶ÚfiÛıÈ· ÙfiÍ· ÙÔ˘ ¿ÙÏ·ÓÙ· ∂ÁοÚÛÈÔ˜ Û‡Ó‰ÂÛÌÔ˜ ÙÔ˘ ¿ÙÏ·ÓÙ·

¶Ï¿ÁÈÔÈ ÙÂÚ˘ÁÔÂȉ›˜ Û‡Ó‰ÂÛÌÔÈ ÙÔ˘ Ô‰fiÓÙ· ·

‚

√›ÛıÈÔ˜ ÂÈÌ‹Î˘ Û‡Ó‰ÂÛÌÔ˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘

∂ÈÎfiÓ· 1·, ‚. ŒÛˆ ÎÚ·ÓÈÔ·˘¯ÂÓÈÎÔ› Û‡Ó‰ÂÛÌÔÈ: ·) Ô›ÛıÈ· fi„Ë: ‰Â›¯ÓÂÈ ÙÔ˘˜ Ï¿ÁÈÔ˘˜ ÙÂÚ˘ÁÔÂȉ›˜ Û˘Ó‰¤ÛÌÔ˘˜ Î·È ‚) Ï¿ÁÈ· fi„Ë: ‰Â›¯ÓÂÈ ÙËÓ Î·Ï˘Ù‹ÚÈ· ÌÂÌ‚Ú¿ÓË (4). ¶·È‰È·ÙÚÈ΋ 2005;68:358-364

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·360

360

Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘ Î·È Û˘Ó.

23% 40% ∞∞∞ 42% 21% 16%

∞ÁfiÚÈ· <15 ÂÙÒÓ ∞ÁfiÚÈ· >15 ÂÙÒÓ ∫ÔÚ›ÙÛÈ· >15 ÂÙÒÓ ∫ÔÚ›ÙÛÈ· <15 ÂÙÒÓ

∂ÈÎfiÓ· 2. ∫·Ù·ÓÔÌ‹ ÙˆÓ ·ÙfiÌˆÓ Ì DS Î·È ∞∞∞.

∞fi Ù· ¿ÙÔÌ· Ì ∞∞∞, 19/43 (44%) ‹Ù·Ó ÎÔÚ›ÙÛÈ· (¶›Ó·Î·˜ 1, ∂ÈÎfiÓ· 3) Î·È 24/43 (56%) ·ÁfiÚÈ· (¶›Ó·Î·˜ 2, ∂ÈÎfiÓ· 3). ∏ Û¯¤ÛË ÎÔÚÈÙÛÈÒÓ/·ÁÔÚÈÒÓ ‹Ù·Ó 5/6. ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ËÏÈΛ·, 27/43 (63%) ‹Ù·Ó ÌÈÎÚfiÙÂÚ· ÙˆÓ 15 ÂÙÒÓ Î·È 16/43 (37%) ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚ· ÙˆÓ 15 ÂÙÒÓ (∂ÈÎfiÓ· 3). ∆· 4/43 (9,3%) ¿ÙÔÌ· ·Ó¤ÊÂÚ·Ó Ó¢ÚÔÏÔÁÈο Û˘ÌÙÒÌ·Ù· ‡ÔÙ· ÁÈ· ›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∞˘Ù¿ ˘Ô‚Ï‹ıËÎ·Ó Û ÂÚ·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô Ù˘ ∞ª™™ Ì CT Î·È Û˘ÁÎÂÎÚÈ̤ӷ ÔÈ ·ÛıÂÓ›˜ Ì ·ÚÈıÌfi 7, 13, 19 ÙÔ˘ ¶›Ó·Î· 1 Î·È Ì ·ÚÈıÌfi 24 ÙÔ˘ ¶›Ó·Î· 2. ∞fi ·˘ÙÔ‡˜, ‰‡Ô ˘Ô‚Ï‹ıËÎ·Ó Û MRI (ÔÈ ·ÛıÂÓ›˜ Ì ·ÚÈıÌfi 13 ÙÔ˘ ¶›Ó·Î· 1 Î·È Ì ·ÚÈıÌfi 24 ¶›Ó·Î·˜ 1. ∫ÔÚ›ÙÛÈ· Ì DS Î·È AA∞ [(n=19) (44%)] OÌ¿‰·

No

ŸÓÔÌ·

<15 ¤ÙË

1 2 3 4 5 6 7* 8 9** 10 11 12 13 14 15 16 17 18 19

ª. ∂. ™. ª. π. ª. µ. ª. £. ™. £. ¡. ∆. ¢. °. ª. ™. ª. ¢. Ã. ∑. ¶. ª. °. §. ∂. ∑. ª. °. ∂. ª. °. ™. ™. ƒ. ª. °. ª.

>15 ¤ÙË

* ∞ÛıÂÓ‹˜ Ù˘ ∂ÈÎfiÓ·˜ 4 ** ∞ÛıÂÓ‹˜ Ù˘ ∂ÈÎfiÓ·˜ 5 ¶·È‰È·ÙÚÈ΋ 2005;68:358-364

∏ÏÈΛ· ∞ÙÏ·ÓÙÔ·ÍÔÓÈ΋ (¤ÙË) ·fiÛÙ·ÛË (mm) ı¤ÛË ·/· 8 6 4 4 4 11 11 10 8 7 26 23 30 29 17 17 18 19 30

5 Î¿Ì„Ë 5 Ô˘‰¤ÙÂÚË 7 Ô˘‰¤ÙÂÚË 5 Ô˘‰¤ÙÂÚË 7 Ô˘‰¤ÙÂÚË 5 Î¿Ì„Ë 9 Ô˘‰¤ÙÂÚË 5 Ô˘‰¤ÙÂÚË 6 Ô˘‰¤ÙÂÚË 5 Ô˘‰¤ÙÂÚË 6 Ô˘‰¤ÙÂÚË 6 Î¿Ì„Ë 4 Ô˘‰¤ÙÂÚË 7 Ô˘‰¤ÙÂÚË 8 Ô˘‰¤ÙÂÚË 4 Ô˘‰¤ÙÂÚË 5 Ô˘‰¤ÙÂÚË 5 Ô˘‰¤ÙÂÚË 4 Ô˘‰¤ÙÂÚË

∂ÈÎfiÓ· 3. ∫·Ù·ÓÔÌ‹ ηٿ ËÏÈΛ· Î·È Ê‡ÏÔ ·ÙfiÌˆÓ Ì DS Î·È ∞∞∞.

ÙÔ˘ ¶›Ó·Î· 2). O ¤ÏÂÁ¯Ô˜ ‰ÂÓ ¤‰ÂÈÍ Û ηӤӷ ¿ÙÔÌÔ ÛËÌ›· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡.

™˘˙‹ÙËÛË ∏ ¿ÚıÚˆÛË ÌÂٷ͇ ÙÔ˘ C-1 Î·È ÙÔ˘ C-2 ÛÔÓ‰‡ÏÔ˘, ÙÔ˘ ¿ÙÏ·ÓÙ· Î·È ÙÔ˘ ¿ÍÔÓ·, ·ÚÔ˘ÛÈ¿˙ÂÈ ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ·ÛÙ¿ıÂÈ·˜ ·ÎfiÌË Î·È ÂÍ·ÚıÚ‹Ì·ÙÔ˜, ÚÔηÏÒÓÙ·˜ ÙËÓ ÚÔ˜ Ù· ›Ûˆ ÌÂٷΛÓËÛË Ù˘ Ô‰ÔÓÙÔÂȉԇ˜ ·fiÊ˘Û˘ ÙÔ˘ ¿ÍÔÓ·, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡ (9,10). ∏ ·ÛÙ¿ıÂÈ· ÌÔÚ› Ó· ÚÔÎÏËı› ·fi ÙË ¯·Ï·ÚfiÙËÙ· ÙˆÓ Û˘Ó‰¤ÛÌˆÓ Ô˘ Û˘ÁÎÚ·ÙÔ‡Ó ÙËÓ ¿ÚıÚˆÛË ÛÂ Ê˘ÛÈÔÏÔÁÈ΋ ηٿÛÙ·ÛË, ·fi ÔÛÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ ·˘¯ÂÓÈÎÒÓ ÛÔÓ‰‡ÏˆÓ ‹ Î·È ·fi Ù· ‰‡Ô (1,3). ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È fiÙÈ Ë Û˘¯ÓfiÙËÙ· Ù˘ ∞∞∞ ÛÙ· ¿ÙÔÌ· Ì DS Î˘Ì·›ÓÂÙ·È ·fi 10% ¤ˆ˜ 40% Î·È Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ËÏÈΛ· Î·È ÙÔ Ê‡ÏÔ ÙÔ˘˜ (11-20). ∏ ∞∞∞ ÌÔÚ› Ó· ‰È·ÈÛÙˆı› fi¯È ÌfiÓÔ Û ¿ÙÔÌ· Ì DS, ·ÏÏ¿ Î·È Û ÔÚÈṲ̂ÓÔ˘˜ ·ÛıÂÓ›˜ Ì ÚÂ˘Ì·ÙÔÂȉ‹ ·ÚıÚ›Ùȉ·, Ì ‰È·Ì·Úٛ˜ Ù˘ Ô‰ÔÓÙÔÂȉԇ˜ ·fiÊ˘Û˘ ÙÔ˘ ¿ÍÔÓ· Î·È Û ‰È¿ÊÔÚÔ˘˜ Ù‡Ô˘˜ Ó·ÓÈÛÌÔ‡, ÂÓÒ Â›Ó·È Û˘¯ÓfiÙÂÚË ÛÙ· ·È‰È¿ ·’ fi,ÙÈ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ (10). ™Ù· ˘fi ¤Ú¢ӷ ¿ÙÔÌ· Ì DS Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, ‰È·ÈÛÙÒıËΠ·ÎÙÈÓÔÏÔÁÈο Û ÔÛÔÛÙfi 42% (43/102), ‹Ù·Ó Û˘¯ÓfiÙÂÚË ÛÙ· ·ÁfiÚÈ· (·ÁfiÚÈ·/ÎÔÚ›ÙÛÈ· 24/19, 56%) Î·È ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 15 ÂÙÒÓ (27/16, 63%). ∆· Â˘Ú‹Ì·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ Û˘ÌʈÓÔ‡Ó Ì ÂΛӷ ÙˆÓ ¿ÏÏˆÓ ÂÚ¢ÓËÙÒÓ. ∆Ô ·ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ÂÍ¿ÚıÚËÌ·, ·Ó Î·È Â›Ó·È Ôχ Û·ÓÈfiÙÂÚÔ ·fi ÙËÓ ·ÛÙ¿ıÂÈ·, ›ӷÈ

Pediatr Sept-Oct 05

17-10-05

16:51

™ÂÏ›‰·361

361

™‡Ó‰ÚÔÌÔ Down Î·È ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ·

¶›Ó·Î·˜ 2. ∞ÁfiÚÈ· Ì DS Î·È AA∞ [n=24 (56%)] OÌ¿‰·

No

ŸÓÔÌ·

<15 ¤ÙË

1 2 3 4 5 6 7 8* 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24

∆. ¢. µ. Ã. ∫. ∫. ¡. °. ∆. ™. ¶. ∫. º. ¢. ª. ª. ¶. °. µ. ª. ™. ™. §. Ã. ¡. °. ¶. ¢. µ. ™. ¶. °. ∞. Ã. ¡. Ã. ∫. ∞. ¶. ∞. ∫. Ã. π. ∞. ∞. ª. °. µ.

>15 ¤ÙË

∏ÏÈΛ· ∞ÙÏ·ÓÙÔ·ÍÔÓÈ΋ (¤ÙË) ·fiÛÙ·ÛË (mm) ı¤ÛË ·/· 4 4 4 3 1 6 6 12 10 12 11 11 11 10 7 7 14 40 25 20 17 17 16 22

6 Ô˘‰¤ÙÂÚË 7 Ô˘‰¤ÙÂÚË 5 Ô˘‰¤ÙÂÚË 6 Ô˘‰¤ÙÂÚË 5 Ô˘‰¤ÙÂÚË 5 Î¿Ì„Ë 5 Î¿Ì„Ë 5 Ô˘‰¤ÙÂÚË 7 Ô˘‰¤ÙÂÚË 5 Î¿Ì„Ë 5 Î¿Ì„Ë 6 Ô˘‰¤ÙÂÚË 7 Ô˘‰¤ÙÂÚË 5 Î¿Ì„Ë 5 Î¿Ì„Ë 5 Ô˘‰¤ÙÂÚË 5 Ô˘‰¤ÙÂÚË 4 Ô˘‰¤ÙÂÚË 4 Ô˘‰¤ÙÂÚË 7 Ô˘‰¤ÙÂÚË 8 Ô˘‰¤ÙÂÚË 4 Ô˘‰¤ÙÂÚË 4 Ô˘‰¤ÙÂÚË 4 Ô˘‰¤ÙÂÚË

∂ÈÎfiÓ· 5. ¶Ï¿ÁÈ· ·/· ∞ª™™ Ù˘ ·ÛıÂÓÔ‡˜ ™. ª., ËÏÈΛ·˜ 8 ÂÙÒÓ, Ì ·ÚÈıÌfi 9 ÙÔ˘ ¶›Ó·Î· 1 ÛÂ: ·) Ô˘‰¤ÙÂÚË ı¤ÛË ∞∞∞ 6 mm Î·È ‚) ÂÏ·ÊÚ¿ Î¿Ì„Ë ∞∞∞ 6 mm.

·ÂÈÏËÙÈÎfi ÁÈ· ÙË ˙ˆ‹. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi ··ÈÙÂ›Ù·È ˘„ËÏ‹ ¢·ÈÛıËÛ›· ÙˆÓ ÁÈ·ÙÚÒÓ ÁÈ· ÙËÓ ·ÔÙÚÔ‹ ηٷÛÙ¿ÛÂˆÓ Ô˘ ÚÔηÏÔ‡Ó ›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∞fi ÙÔ˘˜ ·ÛıÂÓ›˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, Ù¤ÛÛÂÚȘ ‰È·Ì·ÚÙ‡ÚÔÓÙ·Ó ÁÈ· Û˘ÌÙÒÌ·Ù· ‡ÔÙ· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∆· Û˘ÌÙÒÌ·Ù· ÂÚÈÂÏ¿Ì‚·Ó·Ó “Á‰Ô‡Ô” ·ÎÔ˘ÛÙfi Î·È ·fi ÙÔ˘˜ ÁÔÓ›˜ Î·È ·fi ÙÔ ÎÔÚ›ÙÛÈ Î·Ù¿ ÙËÓ ÚÔÛıÈÔ›ÛıÈ· Î¿Ì„Ë Ù˘

ÎÂÊ·Ï‹˜ ÙÔ˘ (·ÛıÂÓ‹˜ Ì ·ÚÈıÌfi 7 ÙÔ˘ ¶›Ó·Î· 1 Î·È Ì ∞∞∞ 9 mm Û Ï¿ÁÈ· Ô˘‰¤ÙÂÚË ı¤ÛË Î·È Û ο̄Ë, ∂ÈÎfiÓ· 4), ·˘¯ÂÓ·ÏÁ›· ηٿ ÙËÓ Î¿Ì„Ë Ù˘ ÎÂÊ·Ï‹˜ Î·È ·È̈‰›Â˜ ÙˆÓ ¿Óˆ ¿ÎÚˆÓ ¿Ìʈ (ÛÙȘ ·ÛıÂÓ›˜ Ì ·ÚÈıÌfi 13 Î·È 19 ÙÔ˘ ¶›Ó·Î· 1, ·ÓÙ›ÛÙÔȯ· - Ì ∞∞∞ 4 mm Û Ï¿ÁÈ· Ô˘‰¤ÙÂÚË ı¤ÛË, ·ÓÙ›ÛÙÔȯ·) ηÈ, Ù¤ÏÔ˜, ÌË ‰È·ÁÓˆṲ̂ӷ ÏÈÔı˘ÌÈο ÂÂÈÛfi‰È· ÛÙÔÓ ·ÛıÂÓ‹ Ì ·ÚÈıÌfi 24 ÙÔ˘ ¶›Ó·Î· 2 (Ì ∞∞∞ 4 mm Û Ï¿ÁÈ· Ô˘‰¤ÙÂÚË ı¤ÛË). ¢ÈÂÓÂÚÁ‹ıËΠCT Î·È MRI Ù˘ ∞ª™™ Î·È ‰ÂÓ Ê¿ÓËΠ›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ ۈϋӷ ÛÂ Î·Ó¤Ó·Ó ·fi ÙÔ˘˜ Ù¤ÛÛÂÚȘ ·ÛıÂÓ›˜. ∆· ¿ÙÔÌ· ·˘Ù¿ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÂ

∂ÈÎfiÓ· 4. ¶Ï¿ÁÈ· ·/· ∞ª™™ Ù˘ ·ÛıÂÓÔ‡˜ ∆. ¢., ËÏÈΛ·˜ 11 ÂÙÒÓ, Ì ·ÚÈıÌfi 7 ÙÔ˘ ¶›Ó·Î· 1 ÛÂ: ·) Ô˘‰¤ÙÂÚË ı¤ÛË AAA 9 mm Î·È ‚) ÂÏ·ÊÚ¿ Î¿Ì„Ë ∞∞∞ 9 mm.

∂ÈÎfiÓ· 6. ¶Ï¿ÁÈ· ·/· ∞ª™™ Ùo˘ ·ÛıÂÓÔ‡˜ ª. ª., ËÏÈΛ·˜ 12 ÂÙÒÓ, Ì ·ÚÈıÌfi 8 ÙÔ˘ ¶›Ó·Î· 2 ÛÂ: ·) Ô˘‰¤ÙÂÚË ı¤ÛË ∞∞∞ 5 mm Î·È ‚) Î¿Ì„Ë ∞∞∞ 6 mm.

* ∞ÛıÂÓ‹˜ Ù˘ ∂ÈÎfiÓ·˜ 6

¶·È‰È·ÙÚÈ΋ 2005;68:358-364

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Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘ Î·È Û˘Ó.

∂ÈÎfiÓ· 7·, ‚. π·ÙÚÈ΋ ‚‚·›ˆÛË Ô˘ ··ÈÙÂ›Ù·È ·fi ÙÔ˘˜ Special Olympics ÁÈ· Ù· ¿ÙÔÌ· Ì DS Î·È ∞∞∞.

·ıÏÔ·È‰È¤˜ Ô˘ ‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Î¿Ì„Ë Î·È ¤ÎÙ·ÛË Ù˘ ÎÂÊ·Ï‹˜ Î·È Â›Ó·È Û ȷÙÚÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË. ∆Ô 1984, Ë ∞∞¶ ‰ËÌÔÛ›Â˘Û ÁÈ· ÚÒÙË ÊÔÚ¿ Â›ÛËÌ· ÙË ı¤ÛË Ù˘ ÁÈ· ÙÔÓ ÚÔÏËÙÈÎfi ¤ÏÂÁ¯Ô Û¯ÂÙÈο Ì ÙËÓ ∞∞∞ ÙˆÓ ÂÊ‹‚ˆÓ Ì DS (8,20). ∆fiÙÂ, ˘ÔÛÙ‹ÚÈ˙ ÙËÓ ¿Ô„Ë Ô˘ ÂÈÛ‹Á·Á·Ó ÙÔ 1983 ÔÈ Special Olympics, fiÙÈ ‰ËÏ·‰‹ Ù· ¿ÙÔÌ· Ì DS ı· Ú¤ÂÈ Ó· ˘Ô‚¿ÏÏÔÓÙ·È Û Ï¿ÁÈ· ·/· ÙÔ˘ ·˘¯¤Ó· ÚÈÓ ·fi ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘˜ ÛÙÔ ‰ÈÂıÓ¤˜ ·ÓÙ·ÁˆÓÈÛÙÈÎfi ÚfiÁÚ·ÌÌ· ÙˆÓ Special Olympics (7). ∆· ¿ÙÔÌ· Ì ·ÎÙÈÓÔÏÔÁÈο ‰È·ÈÛو̤ÓË ∞∞∞ ·ÔÎÏ›ÔÓÙ·Ó ·fi οÔȘ ‰Ú·ÛÙËÚÈfiÙËÙ˜, ÔÈ Ôԛ˜ ÌÔÚ› Ó· Û˘Ó‰¤ÔÓÙ·È Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ Î¿ÎˆÛ˘ Ù˘ ∞ª™™. ¶ÚfiÛÊ·Ù· ·Ó·ıˆڋıËΠ·˘Ù‹ Ë ¿Ô„Ë, ‰ÈfiÙÈ Ë ∂ÈÙÚÔ‹ ∞ıÏËÙÈ·ÙÚÈ΋˜ Î·È Fitness Ù˘ AA¶ ·Ó·ÛÎfiËÛ ٷ ÛÙÔȯ›·, ‚¿ÛÂÈ ÙˆÓ ÔÔ›ˆÓ ÂÈÎÚ¿ÙËÛ ·˘Ù‹ Ë ı¤ÛË Î·È ·ÌÊÈÛ‚ËÙ‹ıËÎÂ Ë ·Í›· Ù˘ ·/· ˆ˜ screening test ÁÈ· Èı·Ó‹ οΈÛË ÙÔ˘ ·˘¯¤Ó· ÙˆÓ ·ıÏËÙÒÓ Ì DS (21). ∆· ÛÙÔȯ›· ÛÙ· ÔÔ›· ÛÙËÚ›¯ıËÎÂ Ë ·ÌÊÈÛ‚‹ÙËÛË Î·È Ë ·Ó·ıÂÒÚËÛË ‹Ù·Ó ÔÈ ·Û·Ê›˜ ÁÓÒÛÂȘ Û¯ÂÙÈο Ì ÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ·ÛÙ¿¶·È‰È·ÙÚÈ΋ 2005;68:358-364

ıÂÈ·˜ Î·È ÙÔ˘ Èı·ÓÔ‡ ÂÍ·ÚıÚ‹Ì·ÙÔ˜, ÁÈ· ÙÔ ·Ó Ë ·ÎÙÈÓÔÏÔÁÈο ÙÂÎÌËÚȈ̤ÓË ·Û˘Ìو̷ÙÈ΋ ∞∞∞ ˘ÔÎÚ‡ÙÂÈ Î›Ó‰˘ÓÔ ÁÈ· ÚfiÎÏËÛË Û˘Ìو̷ÙÈ΋˜ ·ÛÙ¿ıÂÈ·˜ ‹ Î·È ·ÎfiÌË ÛÔ‚·ÚfiÙÂÚ˘ ηٿÛÙ·Û˘ ÙÔ˘ ·ÙÏ·ÓÙÔ·ÍÔÓÈÎÔ‡ ÂÍ·ÚıÚ‹Ì·ÙÔ˜ Ô˘ Ô‰ËÁ› ÛÙÔÓ ÙÚ·˘Ì·ÙÈÛÌfi ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡ Î·È Î˘Ú›ˆ˜ Ù·, ·ÚÈıÌËÙÈο, ÂÍ·ÈÚÂÙÈο Ï›Á· ¿ÙÔÌ· Ì ۇӉÚÔÌÔ Down Î·È Û˘Ìو̷ÙÈ΋ ∞∞∞ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›·. ŒÓ· ÂÈϤÔÓ ÛÙÔÈ¯Â›Ô Ô˘ ÚÔηÏ› ·ÎfiÌË ÌÂÁ·Ï‡ÙÂÚË Û‡Á¯˘ÛË Â›Ó·È Ë ÊÙˆ¯‹ Â·Ó·ÏË„ÈÌfiÙËÙ· ÙˆÓ Â˘ÚËÌ¿ÙˆÓ Ù˘ ∞∞∞ ÛÙȘ ·ÎÙÈÓÔÁڷʛ˜ (21), ηıÒ˜ Ë ·ÎÙÈÓÔÏÔÁÈ΋ ÂÈÎfiÓ· ·ÏÏ¿˙ÂÈ ·fi ÒÚ· Û ÒÚ· ·fi ·ıÔÏÔÁÈ΋ ÛÂ Ê˘ÛÈÔÏÔÁÈ΋. ∏ ‡·ÚÍË Ù˘ ∞∞∞ ÌÔÚ› Ó· ÂÍ·Ê·ÓÈÛÙ› Û ÂfiÌÂÓ˜ ·ÎÙÈÓÔÁڷʛ˜ Ô˘ Ï·Ì‚¿ÓÔÓÙ·È ·fi ÙÔÓ ›‰ÈÔ ¯ÂÈÚÈÛÙ‹ ÛÙÔ ›‰ÈÔ ¿ÙÔÌÔ. ¢Â‰ÔÌ¤ÓˆÓ fiÏˆÓ ·˘ÙÒÓ ÙˆÓ ÚÔ‚ÏËÌ·ÙÈÛÌÒÓ, Ë ∞∞¶ ·Ó·Î¿ÏÂÛ ÙËÓ ·Ú¯È΋ Ù˘ Ô‰ËÁ›· ÁÈ· ÙÔÓ ÚÔÏËÙÈÎfi ¤ÏÂÁ¯Ô fiÏˆÓ ÙˆÓ ·ÙfiÌˆÓ Ì DS Ì ·ÎÙÈÓÔÁڷʛ˜ Ù˘ ∞ª™™ ÚÈÓ ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘˜ ÛÙ· ·ıÏ‹Ì·Ù·, ÙËÓ ÔÔ›· ÚfiÙÂÈÓ ÙÔ 1984. ∞ÓÙ›ıÂÙ·, ÙÒÚ· ÚÔÙ›ÓÂÈ ÔÈ ÎÏÈÓÈÎÔ› ÁÈ·ÙÚÔ› Ó· Ï·Ì‚¿ÓÔ˘Ó ÚÔÛÂÎÙÈο ·Ó¿ ¤ÙÔ˜ ÙÔ ÈÛÙÔÚÈÎfi Î·È Ì ÙË ÏÂÙÔÌÂÚ‹ ÎÏÈÓÈ΋ ÂͤٷÛË

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∂ÈÎfiÓ· 8. ŒÊË‚Ë Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ηٿ ÙË ‚Ú¿‚Â˘Û‹ Ù˘ ÛÙÔ˘˜ Special Olympics ÛÙËÓ πÚÏ·Ó‰›· ÙÔ 2003.

Ó· ·ÔÎÏÂ›Ô˘Ó ‹ Ó· ÚÔÛÂÁÁ›˙Ô˘Ó ÛÙË Ó¢ÚÔÏÔÁÈ΋ ÛËÌÂÈÔÏÔÁ›· Ù˘ ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∂ÈϤÔÓ, Û˘ÛÙ‹ÓÂÈ ÔÈ ÎÏÈÓÈÎÔ› ÁÈ·ÙÚÔ› Ó· ÂÎ·È‰Â‡Ô˘Ó ÙÔ˘˜ ÁÔÓ›˜ ÙˆÓ ·ÙfiÌˆÓ Ì DS Ó· ·Ó·ÁÓˆÚ›˙Ô˘Ó ¤ÁηÈÚ· Ù· Û˘ÌÙÒÌ·Ù· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡, fiˆ˜ ÙÔ ·ÓÒÌ·ÏÔ ‚¿‰ÈÛÌ·, Ë ·˘ÍË̤ÓË ·‰ÂÍÈfiÙËÙ· Î·È ÔÈ ‰È·Ù·Ú·¯¤˜ ÙˆÓ ÛÊÈÁÎÙ‹ÚˆÓ (21). ∆· ·Û˘Ìو̷ÙÈο ¿ÙÔÌ· Ì DS ÛÙ· ÔÔ›· ‰È·ÁÓÒÛÙËΠ·ÎÙÈÓÔÏÔÁÈο ∞∞∞, ı· Ú¤ÂÈ Ó· ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È Ó¢ÚÔÏÔÁÈο. ∂Λӷ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó Í·ÊÓÈο Ó¢ÚÔÏÔÁÈ΋ ÛËÌÂÈÔÏÔÁ›· ‹ Âȉ›ӈÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ı· Ú¤ÂÈ Ó· ˘Ô‚¿ÏÏÔÓÙ·È Û CT ‹ MRI ∞ª™™, ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰È¢ÎÚÈÓÈÛÙ› Ë ¤ÎÙ·ÛË Ù˘ ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∂¿Ó ·Ô‰Âȯı› ·ÂÈÎÔÓÈÛÙÈο ›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡, Ú¤ÂÈ Ó· ·Ú·ÂÌÊıÔ‡Ó Û Ó¢ÚÔ¯ÂÈÚÔ˘ÚÁfi ‹ ÔÚıÔ·È‰ÈÎfi Ô˘ ı· ÛÙ·ıÂÚÔÔÈ‹ÛÂÈ ¯ÂÈÚÔ˘ÚÁÈο ÙÔ ¿Óˆ ÙÌ‹Ì· Ù˘ ™™, ÒÛÙ ӷ ·ÔÊ¢¯ı› Ô ÙÚ·˘Ì·ÙÈÛÌfi˜ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡ (2,3,8,23). øÛÙfiÛÔ, Ë ∂ÈÙÚÔ‹ ÙˆÓ Special Olympics ÂÍ·ÎÔÏÔ˘ıÔ‡Û ̤¯ÚÈ ÚfiÛÊ·Ù· Ó· ıˆÚ› ÂÍ›ÛÔ˘ ··Ú·›ÙËÙÔ Ì ÙË Ê˘ÛÈ΋ ÂͤٷÛË ÙÔÓ ·ÎÙÈÓÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô Ù˘ ∞ª™™ fiÏˆÓ ÙˆÓ ·ÙfiÌˆÓ Ì DS ÚÈÓ ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘˜ ÛÙ· ·ıÏ‹Ì·Ù·. ∂¿Ó ‰È·ÈÛÙˆı› ∞∞∞ ‰ÂÓ ÂÈÙÚ¤ÂÈ Ó· Ï¿‚Ô˘Ó Ì¤ÚÔ˜ Û ‰Ú·ÛÙËÚÈfiÙËÙ˜ Ô˘ ÚÔηÏÔ‡Ó ˘ÂÚ¤ÎÙ·ÛË ÙÔ˘ ·˘¯¤Ó·, fiˆ˜ ηٿ‰˘ÛË, ÎÔχ̂ËÛË-ÂÙ·ÏÔ‡‰·, ηٿ‰˘ÛË ÛÙËÓ ¤Ó·ÚÍË Ù˘ ÎÔχ̂ËÛ˘, Á˘ÌÓ·ÛÙÈ΋, ¿ÏÌ·Ù· ÌÂÁ¿ÏÔ˘ ‡„Ô˘˜, ¤ÓÙ·ıÏÔ, Ô‰fiÛÊ·ÈÚÔ Î·È ·Û΋ÛÂȘ ÚÔı¤ÚÌ·ÓÛ˘, ηıÒ˜ ÚÔηÏÔ‡Ó ›ÂÛË ÙˆÓ Ì˘ÒÓ Ù˘ ÎÂÊ·Ï‹˜ Î·È ÙÔ˘ ·˘¯¤Ó·. ¢‡Ô ¤Ê˂˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, Ô˘ Û˘ÌÌÂÙ›¯·Ó ÛÙÔ˘˜ Special Olympics ÙÔ˘ 2003, ÌÂٷ͇ ÙˆÓ ¿ÏÏˆÓ ÂÓÙ‡ˆÓ ÁÈ· Û˘ÌÏ‹ÚˆÛË ÁÈ· ÙÔÓ È·ÙÚÈÎfi ÙÔ˘˜ ¤ÏÂÁ¯Ô, ÚÔÛÎfiÌÈÛ·Ó Î·È ÊfiÚÌ·

ÚÔ˜ Û˘ÌÏ‹ÚˆÛË Î·È ˘ÔÁÚ·Ê‹ ·fi ÙÔÓ È·ÙÚfi, ÙÔ ›‰ÈÔ ÙÔ ¿ÙÔÌÔ Ì DS Î·È ÙÔÓ ÁÔÓ¤· Û¯ÂÙÈο Ì ÙËÓ ∞∞∞ (∂ÈÎfiÓ˜ 7·, ‚ Î·È 8). ∆· ÂÚÈÛÛfiÙÂÚ· ¿ÙÔÌ· ·˘Ù‹˜ Ù˘ ¤Ú¢ӷ˜ Ì ∞∞∞ Â›Ó·È ·Û˘Ìو̷ÙÈο. ¢fiıËÎ·Ó Ô‰ËÁ›Â˜ ÛÙÔ˘˜ ÁÔÓ›˜ Ó· ÚÔˆıÔ‡Ó Ù· ·È‰È¿ ÙÔ˘˜ ÛÙÔÓ ·ıÏËÙÈÛÌfi, Û ·ıÏ‹Ì·Ù· Ô˘ ‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ˘ÂÚ¤ÎÙ·ÛË ÙÔ˘ ·˘¯¤Ó·, fiˆ˜ ÚԷӷʤÚıËÎÂ, ηıÒ˜ Â›Û˘ Î·È ·ÔÊ˘Á‹ ı¤ÛÂˆÓ ˘ÂÚ¤ÎÙ·Û˘ Ù˘ ÎÂÊ·Ï‹˜ Û ηıËÌÂÚÈÓ¤˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ fiˆ˜ Ë ˘ÂÚ¤ÎÙ·ÛË Ù˘ ÎÂÊ·Ï‹˜ ÛÙÔÓ ÏÔ˘Ù‹Ú· ÙÔ˘ ÎÔÌ̈ÙËÚ›Ô˘, Ó· Í·ÏÒÓÔ˘Ó fiÙ·Ó ÚfiÎÂÈÙ·È Ó· ‚¿ÏÏÔ˘Ó ÛÙ·ÁfiÓ˜ ÛÙ· Ì¿ÙÈ·, Ó· ˘¿Ú¯ÂÈ ÛÙÔ Î¿ıÈÛÌ· ÙÔ˘ ·˘ÙÔÎÈÓ‹ÙÔ˘ ÙÔ˘˜ ÚÔÛٷ٢ÙÈÎfi ÚÔÛΤʷÏÔ ÁÈ· Ó· ÚÔÛٷهÔÓÙ·È ·fi ·fiÙÔÌÔ ÊÚÂÓ¿ÚÈÛÌ· Î.Ï. ∆· Â˘Ú‹Ì·Ù· Ù˘ ¤Ú¢ӷ˜ ÂÓÈÛ¯‡Ô˘Ó ÙȘ Ô‰ËÁ›Â˜ Ù˘ ∞ıÏËÙÈ·ÙÚÈ΋˜ ∂ÈÙÚÔ‹˜ Ù˘ ∞∞¶, ‰ËÏ·‰‹ ÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ·È‰ÈÒÓ Ì DS ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 3 ÂÙÒÓ Ì ·/· Ù˘ ∞ª™™ ηٿ ÙËÓ ÚÒÙË Â›Û΄‹ ÙÔ˘˜ ÛÙÔÓ ·È‰›·ÙÚÔ, Û˘ÛÙ¿ÛÂȘ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÁÈ· ÙË Û˘ÌÌÂÙÔ¯‹ fiÏˆÓ ÙˆÓ ·È‰ÈÒÓ ÛÙ· ·ıÏ‹Ì·Ù· Î·È ·/· Ù˘ ∞ª™™ Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ· ÚÈÓ ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘˜ Û ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜. O ÎÏÈÓÈÎfi˜ ÁÈ·ÙÚfi˜, Ì ÙÔ ÏÂÙÔÌÂÚ¤˜ ÈÛÙÔÚÈÎfi Î·È ÙËÓ ÚÔÛÂÎÙÈ΋ ÎÏÈÓÈ΋ ÂͤٷÛË, Ú¤ÂÈ Ó· ·Ó·˙ËÙ¿ Û˘ÌÙÒÌ·Ù· Î·È Ó¢ÚÔÏÔÁÈο ÛËÌ›· Ù˘ ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡ (‡ÎÔÏË ÎfiˆÛË, ‰È·Ù·Ú·¯¤˜ ÛÙË ‚¿‰ÈÛË, fiÓÔ ‹ ÂÚÈÔÚÈÛÌfi Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜ ÙÔ˘ ·˘¯¤Ó·, Ú·È‚fiÎÚ·ÓÔ, ·Û˘Ó¤ÚÁÂÈ· Î·È ·‰ÂÍÈfiÙËÙ·, ·ÈÛıËÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, Û·ÛÙÈÎfiÙËÙ· ÙÔ˘ ·ÓÒÙÂÚÔ˘ ÎÈÓËÙÈÎÔ‡ Ó¢ÚÒÓ·, ÌÂÙ·‚ÔϤ˜ ÛÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ÛÊÈÁÎÙ‹ÚˆÓ Ù˘ ·ÛÙ˘ Î·È ÙÔ˘ ÚˆÎÙÔ‡). ∏ ÛËÌÂÈÔÏÔÁ›· Ô˘ ·ÔηχÙÂÙ·È ·fi ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ·ÔÙÂÏ› ÙÔ ÎÚÈÙ‹ÚÈÔ ÁÈ· ÙËÓ Â·Ó¿ÏË„Ë Ù˘ ·/· Ù˘ ∞ª™™ Î·È ÙÔ˘ ÂÚ·ÈÙ¤Úˆ ÂϤÁ¯Ô˘ Ì CT ‹ MRI Ù˘ ∞ª™™.

∂˘¯·ÚÈÛٛ˜ £ÂṲ́˜ ¢¯·ÚÈÛٛ˜ ÛÙȘ Ù¯ÓÔÏfiÁÔ˘˜ ·ÎÙÈÓÔÏfiÁÔ˘˜ Î. ¢. ∆ÛÔ˘Î·Ï¿ Î·È ∞. §ÂÔÓÙ·Ú›‰Ô˘ ÁÈ· ÙË Û˘Ì‚ÔÏ‹ ÙÔ˘˜ ÛÙÔ Ù¯ÓÈÎfi ̤ÚÔ˜ Ù˘ ¤Ú¢ӷ˜ Î·È ÁÈ· ÙÔ ÊÈÏÈÎfi Îϛ̷ Ô˘ ‰ËÌÈÔ˘ÚÁÔ‡Û·Ó ÛÙ· ¿ÙÔÌ· Ì DS Î·È ÛÙÔ˘˜ ÁÔÓ›˜ ÙÔ˘˜.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Cremers MJ, Bol E, de Roos F, van Gijn J. Risk of sports activities in children with Down’s syndrome ¶·È‰È·ÙÚÈ΋ 2005;68:358-364

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and atlantoaxial instability. Lancet 1993;342:511-514.

2. Pueschel SM, Scola FH. Atlantoaxial instability in individuals with Down syndrome: epidemiologic, radiographic, and clinical studies. Pediatrics 1987;80: 555-560.

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4. Stein SM, Kirchner SG, Horev G, Hernanz-Schulman M. Atlanto-Ôccipital subluxation in Down syndrome. Pediatr Radiol 1991;21:121-124.

5. Pueschel SM, Scola FH, Pezzullo JC. A longitudinal study of atlanto-dens relationships in asymptomatic individuals with Down syndrome. Pediatrics 1992; 89:1194-1198.

6. Selby KA, Newton RW, Gupta S, Hunt L. Clinical predictors and radiological reliability in atlantoaxial subluxation in Down’s syndrome. Arch Dis Child 1991;66:876-878.

7. Davidson RG. Atlantoaxial instability in individuals with Down syndrome: a fresh look at the evidence.

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10. Roche CJ, O’Malley M, Dorgan JC, Carty HM. A pictorial review of atlanto-axial rotatory fixation: key points for the radiologist. Clin Radiol 2001;56:947958.

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12. Herzka A, Sponseller PD, Pyeritz RE. Atlantoaxial rotatory subluxation in patients with Marfan syndrome.

A report of three cases. Spine 2000;25:524-526.

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13. Casey AT, Crockard HA, Geddes JF, Stevens J. Vertical translocation: the enigma of the disappearing atlantodens interval in patients with myelopathy and rheumatoid arthritis. Part I. Clinical, radiological, and neuropathological features. J Neurosurg 1997; 87:856-862.

14. Papadopoulos SM, Dickman CA, Sonntag VK. Atlantoaxial stabilization in rheumatoid arthritis. J Neurosurg 1991;74:1-7.

15. Roche CJ, Eyes BE, Whitehouse GH. The rheumatoid cervical spine: signs of instability on plain cervical radiographs. Clin Radiol 2002;57:241-249.

16. Fujiwara K, Owaki H, Fujimoto M, Yonenobu K, Ochi T. A long-term follow-up study of cervical lesions in rheumatoid arthritis. J Spinal Disord 2000; 13:519-526.

17. Neva MH, Isomaki P, Hannonen P, Kauppi M, Krishnan E, Sokka T. Early and extensive erosiveness in peripheral joints predicts atlantoaxial subluxations in patients with rheumatoid arthritis. Arthritis Rheum 2003;48:1808-1813.

18. ™Ô˘ÊÙ¿˜ µ, ÷Ù˙ËÛ‚·ÛÙÔ‡-§Ô˘Î›‰Ô˘ Ã, °ÚfiÏÈÔ˜ °, ∆Û›ÎÔ˘Ï·˜ π, ªÂÏ›‰Ë˜ ¢, µÂÚÁÔÔ‡ÏÔ˘ ™ Î·È Û˘Ó. ¶ÚÔÏËÙÈÎfi˜ ·ÎÙÈÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ Û ·È‰È¿ Ì ۇӉÚÔÌÔ Down. ¶·È‰È·ÙÚÈ΋ µÔÚ›Ԣ ∂ÏÏ¿‰Ô˜ 1991;3:191-200.

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Risks of sports activities in individuals with Down syndrome and atlantoaxial instability H. Hatzissevastou - Loukidou1, M. Badouraki2, P. Kambouridou1, E. Sotiridou1

Abstract Background: The prevalence of atlantoaxial instability (AAI) among persons with Down syndrome (DS) ranges from 10% to 40% and is related to age and sex. The aim of this study was to investigate AAI in non-institutional people with DS in Northern Greece and to monitor them annually.

Methods: A total of 171 persons with DS, aged 0-40 years, were enrolled in the study. The patients were examined clinically in relation to the presence or absence of discomfort in the neck area and spinal cord compression. Eighty one per cent of those aged 3 years and older were screened radiographically at the cervical spine. A lateral neck X-ray was made in the neutral position, and in flexion when necessary. An atlantoaxial distance of greater than 4 mm for children under 15 years of age and greater than 3 mm for people over 15 years is suggested as the cut off point for AAI. All the X-rays were examined by the same radiologist.

Results: The prevalence of AAI was high (42%) in the group of people with DS, and it was more frequent in boys (56%) and in those under 15 years (63%). Local symptoms were reported by 9.3%.

Investigation by computed tomography (CT) suggested that none of these had spinal cord compression.

1 1st Paediatric Clinic of Aristotle University, “Ippokration” General Hospital, Thessaloniki 2 Paediatric Department of Radiology, “Ippokration” General Hospital, Thessaloniki Correspondence: H. Hatzissevastou-Loukidou Laboratory of Cytogenetics, 1st Paediatric Clinic of Aristotle University, “Ippokration” General Hospital, 49 Konstantinoupoleos Str., 546 42, Thessaloniki Date of submission: 03-09-2004 Date of approval: 29-03-2005

Conclusions: The results of this study support the guidelines of Committee on Sports of American Academy of Pediatrics (AAP) which recommend screening of children over 3 years old with DS by cervical spine radiography at the initial visit to the paediatrician, and provision of guidance to the families about the participation of the children in sports (i.e., avoidance of extreme flexion or overstretching of the neck) and cervical X-ray screening before they start participation in sports activities. The physician should take a careful history and perform a thorough physical examination, looking for evidence of neurological signs and symptoms of spinal cord compression (easy fatiguability, difficulties in walking, abnormal gait, neck pain, limited neck mobility, torticollis or head tilt, incoordination and clumsiness, sensory deficits, spasticity, hyperreflexia, clonus, extensorplantar reflex, other upper motor neuron signs and changes in bowel or bladder control). Any findings on the physical examination should be investigated by repetition of cervical spine radiography, and further screening by MRI.

Key words Down syndrome, atlantoaxial instability, atlantoaxial subluxation, atlantoaxial dislocation, X-ray, magnetic resonance imaging (MRI).

Paediatriki 2005;68:365

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¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË. ¶ÂÚÈÁÚ·Ê‹ ÚÒÙ˘ ÂÚ›ÙˆÛ˘ ÛÙËÓ ∂ÏÏ¿‰· O. ¶··Ó‰Ú¤Ô˘1, ∞. ∫ÏËÌÂÓÙÔÔ‡ÏÔ˘1, ¡. ¶ÔÓËÚfi˜2, ∞. ∫ˆÓÛÙ·ÓÙÈÓ›‰Ô˘3, ∞. •˘ÔÏ˘Ù¿ - ∑·¯·ÚÈ¿‰Ë1, N. Rachman4, °. ÃÚÔ‡ÛÔ˜1 1 ∞’ ¶·ÓÂÈÛÙËÌȷ΋ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· 2 ∫ÏÈÓÈ΋ ¶Ï·ÛÙÈ΋˜ ÃÂÈÚÔ˘ÚÁÈ΋˜, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ ”∞Á›· ™ÔÊ›·”, ∞ı‹Ó· 3 ∂ÚÁ·ÛÙ‹ÚÈÔ ¶·ıÔÏÔÁÈ΋˜ ∞Ó·ÙÔÌÈ΋˜, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· 4 Institute of Cancer Research, Surrey, UK AÏÏËÏÔÁÚ·Ê›·: O˘Ú·Ó›· ¶··Ó‰Ú¤Ô˘ ∞’ ¶·ÓÂÈÛÙËÌȷ΋ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” £Ë‚ÒÓ & §Â‚·‰Â›·˜, ∆.∫. 115 27, ∞ı‹Ó· E-mail: alkliment@hotmail.com

¶ÂÚ›ÏË„Ë ∏ Ó·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË (juvenile hyaline fibromatosis) Â›Ó·È Û¿ÓÈÔ ÓfiÛËÌ·, Èı·ÓÒ˜ ÙÔ˘ Û˘Ó‰ÂÙÈÎÔ‡ ÈÛÙÔ‡, Ô˘ ÌÂÙ·‚È‚¿˙ÂÙ·È Ì ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú· Î·È ·Ô‰›‰ÂÙ·È Û ÁÔÓ›‰ÈÔ Ô˘ ¯·ÚÙÔÁÚ·ÊÂ›Ù·È ÛÙÔ 4q21 ¯ÚˆÌfiۈ̷. ™ÙËÓ Ù˘È΋ Ù˘ ÌÔÚÊ‹ ÂÌÊ·Ó›˙ÂÙ·È Ù· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÔÏÏ·Ï¿ ·ÓÒ‰˘Ó· ˘Ô‰fiÚÈ· Ô˙›‰È·, Ô˙Ò‰ÂȘ Î·È ˘·ÏÈÓÒ‰ÂȘ ‰ÂÚÌ·ÙÈΤ˜ ‚Ï¿‚˜ ‰È·ÊfiÚÔ˘ ÌÂÁ¤ıÔ˘˜, ˘ÂÚÙÚÔÊ›· ÙˆÓ Ô‡ÏˆÓ, Û˘Áο„ÂȘ ÙˆÓ ·ÚıÚÒÛÂˆÓ Î·È ÔÛÙÂÔÂÓ›·. ¢ÂÓ ˘¿Ú¯ÂÈ Û˘ÌÌÂÙÔ¯‹ ÙˆÓ ÛÏ·Á¯ÓÈÎÒÓ ÔÚÁ¿ÓˆÓ Î·È Ë ÓÔËÙÈ΋ ÂͤÏÈÍË Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋. ¢ÂÓ ˘¿Ú¯Ô˘Ó ¯·Ú·ÎÙËÚÈÛÙÈο ‚ÈÔ¯ËÌÈο Â˘Ú‹Ì·Ù· Î·È Ë ÎÏÈÓÈ΋ ‰È¿ÁÓˆÛË ÂȂ‚·ÈÒÓÂÙ·È Ì ÙËÓ ÈÛÙÔÏÔÁÈ΋ ÂͤٷÛË ÙˆÓ ‚Ï·‚ÒÓ. ¢ÂÓ ˘¿Ú¯ÂÈ ÂȉÈ΋ ıÂÚ·›·, ÂÓÒ Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÍ·›ÚÂÛË ÔÏÏÒÓ ÔÁÎȉ›ˆÓ Á›ÓÂÙ·È ÁÈ· ÏÂÈÙÔ˘ÚÁÈÎÔ‡˜ Î·È ·ÈÛıËÙÈÎÔ‡˜ ÏfiÁÔ˘˜. ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ÎÔÚÈÙÛÈÔ‡ ËÏÈΛ·˜ 4 ÂÙÒÓ, ÌÈÎÚfiÛˆÌÔ˘, ÌÂ Û˘Áο„ÂȘ ÙˆÓ ÌÂÁ¿ÏˆÓ Î·È ÌÈÎÚÒÓ ·ÚıÚÒÛˆÓ, ÂÎÛÂÛËÌ·Ṳ̂ÓË ‰˘Ûη̄›· ÎÔÚÌÔ‡, ˘ÂÚÙÚÔÊ›· ԇψÓ, ˘Ô‰fiÚÈ· Ô˙›‰È· Î·È Ô˙Ò‰ÂȘ-‚Ï·Ùȉ҉ÂȘ ‰ÂÚÌ·ÙÈΤ˜ ‚Ï¿‚˜ ȉ›ˆ˜ ÛÙËÓ ÎÂÊ·Ï‹ Î·È ÛÙ· ‰¿ÎÙ˘Ï· ÙˆÓ ¿ÎÚˆÓ ÌÂ Û˘ÓÔ‰fi ÎÈÓËÙÈ΋ ·Ó·ËÚ›·. ∏ ¯ÔÚ‹ÁËÛË Botox (Botulin toxinum A) ‚ÂÏÙ›ˆÛ ·ÈÛıËÙ¿ ÙË ‰È¿ÓÔÈÍË ÙÔ˘ ÛÙfiÌ·ÙÔ˜ Î·È ‰È¢ÎfiÏ˘Ó ÙË Ì¿ÛËÛË ÛÙË ÌÈÎÚ‹ ·ÛıÂÓ‹.

∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 26-07-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 01-04-2005

§¤ÍÂȘ ÎÏÂȉȿ O˙›‰È·, ‚Ï·Ù›‰Â˜, Èӈ̿وÛË.

∂ÈÛ·ÁˆÁ‹ ∏ Ó·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË Â›Ó·È ÎÏËÚÔÓÔÌÈÎfi ÓfiÛËÌ· Ô˘ ÌÂÙ·‚È‚¿˙ÂÙ·È Ì ÙÔÓ ˘ÔÏÂÈfiÌÂÓÔ ÛˆÌ·ÙÈÎfi ¯·Ú·ÎÙ‹Ú· Î·È ÙÔ ÁÔÓ›‰ÈÔ Â‰Ú¿˙ÂÙ·È ÛÙÔ 4q21 ¯ÚˆÌfiۈ̷ (1). ™˘Ó‹ıˆ˜ ÂΉËÏÒÓÂÙ·È Ù· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜, Ì ‰ÂÚÌ·ÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ ΢ڛˆ˜ ÛÙÔ ÎÚ·Ó›Ô, ÛÙ· ÒÙ·, Á‡Úˆ ·fi ÙË Ì‡ÙË Î·È ÛÙ· ¿ÎÚ·. OÈ ·ÏÏÔÈÒÛÂȘ Â›Ó·È ‚Ï·Ùȉ҉ÂȘ ‹ Î·È Ô˙Ò‰ÂȘ, Ì ٿÛË ·‡ÍËÛ˘ Û ·ÚÈıÌfi Î·È Ì¤ÁÂıÔ˜ Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘. ™˘¯Ó¿ ··ÈÙÂ›Ù·È ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ú¤Ì‚·ÛË ÁÈ· ÏÂÈÙÔ˘ÚÁÈÎÔ‡˜ Î·È ·ÈÛıËÙÈÎÔ‡˜ ÏfiÁÔ˘˜. ∏ ˘ÂÚÏ·Û›· ÙˆÓ Ô‡ÏˆÓ Ù˘Èο ·Ó·Ù‡ÛÛÂÙ·È Û˘Á¯ÚfiÓˆ˜ Ì ÙȘ ‰ÂÚÌ·ÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ Î·È ÂÓ›ÔÙ ··ÈÙÂ›Ù·È ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÂÚÈÔ‰ÔÓÙÈÎÔ‡ ÈÛÙÔ‡ ÁÈ· ‰È¢ÎfiÏ˘ÓÛË Ù˘ Û›ÙÈÛ˘ Î·È Ù˘ ÔÌÈÏ›·˜. OÈ Û˘Áο̄ÂȘ ÙˆÓ ·ÚıÚÒÛÂˆÓ ÂȉÂÈÓÒÓÔÓÙ·È Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ Î·È ÂÚÈÔÚ›˙Ô˘Ó ÙËÓ Î›ÓËÛË Û ٤ÙÔÈÔ ‚·ıÌfi ÒÛÙ ٷ ·È‰È¿ ·˘Ù¿ Ó· ηٷϋÁÔ˘Ó Û ·Ó·ËÚÈÎfi ·Ì·Í›‰ÈÔ. ∏ ÔÛÙÂÔÂÓ›· Â›Ó·È Û˘¯Ó‹. ∏ ÓÔËÙÈ΋ Î·È ¶·È‰È·ÙÚÈ΋ 2005;68:366-370

ÁψÛÛÈ΋ ·Ó¿Ù˘ÍË Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋. ∏ ‰È¿ÁÓˆÛË ÂȂ‚·ÈÒÓÂÙ·È ·fi ÙËÓ ÈÛÙÔÏÔÁÈ΋ ÂͤٷÛË ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ Î·È ÙˆÓ Ô˙ȉ›ˆÓ, fiÔ˘ ·Ó¢ڛÛÎÂÙ·È Û˘ÛÛÒÚ¢ÛË ÔÌÔÁÂÓÔ‡˜ ¿ÌÔÚÊÔ˘ ÔÍÂfiÊÈÏÔ˘ ÂÍˆÎ˘ÙÙ¿ÚÈÔ˘ ˘·ÏÈÓÈÎÔ‡ ˘ÏÈÎÔ‡. ∏ ʇÛË Î·È Ë ÚԤϢÛË ÙÔ˘ ˘·ÏÈÓÈÎÔ‡ ˘ÏÈÎÔ‡ ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈÛÙ›. º·›ÓÂÙ·È, ˆÛÙfiÛÔ, fiÙÈ Û˘ÌÌÂÙ¤¯Ô˘Ó ΢ڛˆ˜ ÙÔ ÎÔÏÏ·ÁfiÓÔ, ÔÈ ÁÏ˘ÎÔ˙·ÌÈÓÔÁÏ˘Î¿Ó˜ Î·È ÔÈ ÁÏ˘ÎÔÚˆÙ½Ó˜ (2).

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ∫ÔÚ›ÙÛÈ ËÏÈΛ·˜ 4 ÂÙÒÓ ·Ú·¤ÌÂÙ·È ÁÈ· Ó¢ÚÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË ÏfiÁˆ ·‰˘Ó·Ì›·˜ ÛÙ‹ÚÈ͢ Î·È ‚¿‰ÈÛ˘. ∂›Ó·È ÙÔ ÚÒÙÔ ·È‰› Ê·ÈÓÔÙ˘Èο ˘ÁÈÒÓ, ÌË Û˘ÁÁÂÓÒÓ ÁÔÓ¤ˆÓ, ÂÏÏËÓÈ΋˜ ÂıÓÈÎfiÙËÙ·˜, Ô˘ ηٿÁÔÓÙ·È ·fi ÙË £Ú¿ÎË. °ÂÓÓ‹ıËΠ- ÌÂÙ¿ ·fi ÙÂÏÂÈfiÌËÓË Î‡ËÛË Î·È Ê˘ÛÈÔÏÔÁÈ΋ ÂÁ΢ÌÔÛ‡ÓË - Ì ηÈÛ·ÚÈ΋ ÙÔÌ‹ ÏfiÁˆ ·ÓÒÌ·Ï˘ ı¤Û˘ Î·È ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ 3 kg. ∞ӷʤÚÔÓÙ·È ÙÚÂȘ ÚÔËÁËı›Û˜ ·˘ÙfiÌ·Ù˜ ·Ô‚ÔϤ˜ Ù˘

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¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË

ÌËÙ¤Ú·˜. ∞fi ÙË Á¤ÓÓËÛË ‰È·ÈÛÙÒıËΠۇÁÎ·Ì„Ë ÙˆÓ ÁÔÓ¿ÙˆÓ Î·È ÙˆÓ ·ÁÎÒÓˆÓ ÔfiÙÂ Î·È ÂÙ¤ıË Ë ‰È¿ÁÓˆÛË Ù˘ ·ÚıÚÔÁÚ‡ˆÛ˘. ∆Ô ·È‰› ˘Ô‚Ï‹ıËΠÛÂ Ê˘ÛÈÎÔıÂÚ·¢ÙÈ΋ ·ÁˆÁ‹ ¯ˆÚ›˜ ‚ÂÏÙ›ˆÛË. ™Â ËÏÈΛ· 12 ÌËÓÒÓ ¤ÁÈÓ ÔÚıÔ·È‰È΋ ·Ú¤Ì‚·ÛË Î·È ÛÙ· ‰‡Ô ÁfiÓ·Ù· Ì ı˘Ï·ÎÔÙÔÌ‹ Î·È ÙÂÓÔÓÙÔÙÔÌ‹ ÙˆÓ Ô›ÛıÈˆÓ ÌËÚÈ·›ˆÓ ¯ˆÚ›˜ ‚ÂÏÙ›ˆÛË. ªÂÙ¿ ·fi 6 Ì‹Ó˜ ÂȯÂÈÚ‹ıËΠÂÎ Ó¤Ô˘ ¯ÂÈÚÔ˘ÚÁÈ΋ ‰ÈfiÚıˆÛË ÙˆÓ Û˘Áο̄ˆÓ, Â›Û˘ ¯ˆÚ›˜ ·ÔÙ¤ÏÂÛÌ·. ∫·Ù¿ ÙËÓ ÎÏÈÓÈ΋ ÂÎÙ›ÌËÛË, Ë ÌÈÎÚ‹ ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·˙Â Ê˘ÛÈÔÏÔÁÈ΋, ÓÔËÙÈ΋ Î·È ÁψÛÛÈ΋ ·Ó¿Ù˘ÍË ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· Ù˘ (Peabody test), ·ÏÏ¿ ÎÈÓËÙÈ΋ ·Ó·ËÚ›· Ì ·‰˘Ó·Ì›· ÛÙ‹ÚÈ͢ Î·È ‚¿‰ÈÛ˘. O ÎÔÚÌfi˜ ‹Ù·Ó ¿Î·ÌÙÔ˜, Ë Î›ÓËÛË Ù˘ ÎÂÊ·Ï‹˜ ÂÚÈÔÚÈṲ̂ÓË (∂ÈÎfiÓ· 1), ÙÔ ÚÔÛˆÂ›Ô ·Ó¤ÎÊÚ·ÛÙÔ, Ì ÌÂÁ¿ÏË ˘ÂÚÙÚÔÊ›· ÙˆÓ Ô‡ÏˆÓ, ¤ÏÏÂÈ„Ë ÌÈÌËÙÈÎÒÓ ÎÈÓ‹ÛÂˆÓ Î·È Ôχ ÌÈÎÚfi ‡ÚÔ˜ ΛÓËÛ˘ Ù˘ ÎÚÔÙ·ÊÔÁÓ·ıÈ΋˜ ¿ÚıÚˆÛ˘ (∂È-

∂ÈÎfiÓ· 1. ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË Û ÎÔÚ›ÙÛÈ ËÏÈΛ·˜ 4 ÂÙÒÓ: ·ÂÈÎÔÓ›˙ÂÙ·È Ô ¿Î·ÌÙÔ˜ ÎÔÚÌfi˜, ÔÈ Û˘Áο̄ÂȘ Û ÔÚı‹ ÁˆÓ›· Î·È Ë Û˘Ó·ÎfiÏÔ˘ıË ·Ú·ÌfiÚʈÛË ÙˆÓ ¿ÎÚˆÓ. OÈ ·ÚıÚÒÛÂȘ ÙˆÓ ¿Óˆ ¿ÎÚˆÓ ‚Ú›ÛÎÔÓÙ·È ÛÂ Î¿Ì„Ë Î·È ¤Ûˆ ÛÙÚÔÊ‹.

ÎfiÓ· 2). ∞Ó¢ڛÛÎÔÓÙ·Ó ÔÈ ÓÂÔÁÈÏÔ› Ô‰fiÓÙ˜. ¶·Ú·ÙËÚ‹ıËΠÂÎÛÂÛËÌ·Ṳ̂ÓË Û‡ÁÎ·Ì„Ë ÙˆÓ ÁÔÓ¿ÙˆÓ Û ÔÚı‹ ÁˆÓ›·, ÂÓÒ fiϘ ÔÈ ·ÚıÚÒÛÂȘ ‹Ù·Ó ‰‡ÛηÌÙ˜ Î·È ÂÒ‰˘Ó˜ ÛÙËÓ ·ıËÙÈ΋ ΛÓËÛË (∂ÈÎfiÓ· 1). OÈ ·ÚıÚÒÛÂȘ ÙˆÓ ¿Óˆ ¿ÎÚˆÓ ‚Ú›ÛÎÔÓÙ·Ó, Â›Û˘, ÛÂ Î¿Ì„Ë Î·È ¤Ûˆ ÛÙÚÔÊ‹. ™ÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÙÚȯˆÙÔ‡ Ù˘ ÎÂÊ·Ï‹˜ÒÙˆÓ-̇Ù˘, ÛÙ· ‰¿ÎÙ˘Ï· ¯ÂÈÚÒÓ Î·È Ô‰ÒÓ Î·È ÛÙËÓ ÂÚÈÚˆÎÙÈ΋ ¯ÒÚ· ˘‹Ú¯·Ó ÔÏÏ·Ï¿ Ô˙›‰È· Î·È ‚Ï·Ù›‰Â˜ ‰È·ÊfiÚÔ˘ ÌÂÁ¤ıÔ˘˜ (¤ˆ˜ Î·È 10 cm), ·ÓÒ‰˘Ó· Î·È Ì·Ï·Î‹˜ ˘Ê‹˜ (∂ÈÎfiÓ· 3). ∞˘Ù¤˜ ÔÈ ·ÏÏÔÈÒÛÂȘ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜, ηıÒ˜ Î·È Ù· ˘Ô‰fiÚÈ· Ô˙›‰È· Ô˘ ‹Ù·Ó „ËÏ·ÊËÙ¿ Û fiÏÔ ÙÔ ÛÒÌ· ÂÌÊ·Ó›ÛÙËÎ·Ó ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 3 ÂÙÒÓ, Ì ٿÛË ·‡ÍËÛ˘ ÛÙÔÓ ·ÚÈıÌfi Î·È ÛÙÔ Ì¤ÁÂıÔ˜. ∆Ô ‰¤ÚÌ· ›¯Â ¯¿ÛÂÈ ÙË Û·ÚÁ‹ ÙÔ˘. ◊Ù·Ó ·ÓÙÈÎÂÈÌÂÓÈο ‰‡ÛÎÔÏÔ Ó· ÂÏÂÁ¯ı› Ë Ì˘˚΋ ÈÛ¯‡˜, fiˆ˜ Î·È Ù· ÙÂÓfiÓÙÈ· ·ÓÙ·Ó·ÎÏ·ÛÙÈο ÙÔ˘ ·È‰ÈÔ‡. ∞fi ÙÔÓ Ï‹ÚË ·ÈÌ·ÙÔÏÔÁÈÎfi, ‚ÈÔ¯ËÌÈÎfi Î·È ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ ·È‰ÈÔ‡ ‰ÂÓ ÚԤ΢„·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ¢ÂÓ ˘‹Ú¯·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙÔÓ Î·Ú‰ÈÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô, ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÎÔÈÏ›·˜, ÛÙËÓ ·ÍÔÓÈ΋ Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘, ÛÙÔÓ ÔÊı·ÏÌÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô Î·È ÛÙËÓ Ù·¯‡ÙËÙ· ·ÁˆÁ‹˜ Ó‡ڈÓ. O ηڢfiÙ˘Ô˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜ ı‹ÏÂÔ˜ (46ÃÃ).

∂ÈÎfiÓ· 2. ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË: Ê·›ÓÂÙ·È ÙÔ ·Ó¤ÎÊÚ·ÛÙÔ ÚÔÛˆ›Ô, Ë ÌÂÁ¿ÏË ˘ÂÚÙÚÔÊ›· ÙˆÓ Ô‡ÏˆÓ, ÔÈ ÂÚÈÛÙÔÌ·ÙÈΤ˜ ‚Ï·Ù›‰Â˜ Î·È ÙÔ Ô˙›‰ÈÔ ÛÙÔ ·ÎÚÔÚÚ›ÓÈÔ. ¶·È‰È·ÙÚÈ΋ 2005;68:366-370

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O. ¶··Ó‰Ú¤Ô˘ Î·È Û˘Ó.

∂ÈÎfiÓ· 4. ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË: ÛÙÔÓ ·ÎÙÈÓÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÙˆÓ ÔÛÙÒÓ ·Ó¢ڛÛÎÂÙ·È ÂÎÛÂÛËÌ·Ṳ̂ÓË ÔÛÙÂÔÂÓ›·.

ÓÂÈ· Ù˘ ·ÚÔ‡Û·˜ ÂÚ›ÙˆÛ˘ ÌÂÙ¿ÏÏ·ÍË ÛÙËÓ CMG-2 ÚˆÙ½ÓË. ∆Ô ·È‰› Â›Ó·È ÔÌfi˙˘ÁÔ ÁÈ· Ì›· ÌÂÙ¿ÏÏ·ÍË Ì·Ù›ÛÌ·ÙÔ˜ (Splice-site mutation IVS13+1 G>A), ÂÓÒ ÔÈ ÁÔÓ›˜ Â›Ó·È ÂÙÂÚÔ˙˘ÁÒÙ˜.

∂ÈÎfiÓ· 3. ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË: ÔÏÏ·Ï¿ Ô˙›‰È· ‰È·ÊfiÚˆÓ ÌÂÁÂıÒÓ ¿Óˆ Î·È Î¿Ùˆ ¿ÎÚˆÓ, ¯·Ú·ÎÙËÚÈÛÙÈο Â˘Ú‹Ì·Ù· Ù˘ ÓfiÛÔ˘.

™ÙÔÓ ·ÎÙÈÓÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÙˆÓ ÔÛÙÒÓ ‰È·ÈÛÙÒıËΠÂÎÛÂÛËÌ·Ṳ̂ÓË ÔÛÙÂÔÂÓ›· (∂ÈÎfiÓ· 4). ™ÙËÓ ËÏÂÎÙÚÔÊfiÚËÛË ÙˆÓ ‚ÏÂÓÓÔÔÏ˘Û·Î¯·ÚÈÙÒÓ Ô‡ÚˆÓ ‚Ú¤ıËΠÔÚȷ΋ ·‡ÍËÛË Ù˘ ·¤ÎÎÚÈÛ˘ Ù˘ ıÂÈÈ΋˜ ¯ÔÓ‰ÚÔ˚Ù›Ó˘. ™ÙË ‚ÈÔ„›· ÙˆÓ Ô˙ȉ›ˆÓ ‚Ú¤ıËΠÂÓ·fiıÂÛË ÎÔÏÏ·ÁÔÓÔÔÈË̤ÓÔ˘ ÈÓÒ‰Ô˘˜ ¿ÌÔÚÊÔ˘ ÔÍÂfiÊÈÏÔ˘ ÂÍˆÎ˘ÙÙ¿ÚÈÔ˘ ˘·ÏÈÓÈÎÔ‡ ÈÛÙÔ‡ Ì ÂÚÈÔ¯¤˜ ·˘ÍË̤Ó˘ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜. ¢ÂÓ ·Ú·ÙËÚ‹ıËΠÌÈÙˆÙÈ΋ ‹ ¿ÏÏË ·ÓÒÌ·ÏË ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ˘Ú‹ÓˆÓ. OÈ ‚Ï¿‚˜ (Ô˙›‰È·) ‹Ù·Ó ˘Ô‰fiÚȘ Ì Â¤ÎÙ·ÛË ˆ˜ ÙËÓ ÂÚÈÙÔÓ›·. ∏ Ó¢ÚÔÁÂÓ‹˜ ÚԤϢÛË ÙÔ˘ ÈÛÙÔ‡ ·ÔÎÏ›ÛÙËΠ̠ÙË ‚Ô‹ıÂÈ· ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈÎÒÓ ‰ÂÈÎÙÒÓ Ô˘ ‚Ú¤ıËÎ·Ó ·ÚÓËÙÈÎÔ› (S100, CD56, CD34). ∆· Â˘Ú‹Ì·Ù· ‹Ù·Ó Û˘Ì‚·Ù¿ ÌÂ Ù˘È΋ ÂÈÎfiÓ· ˘·ÏÈÓÈ΋˜ Èӈ̿وÛ˘ (∂ÈÎfiÓ· 5). °ÔÓȉȷÎfi˜ ¤ÏÂÁ¯Ô˜ Ô˘ ¤ÁÈÓ ÛÙÔ Institute of Cancer Research, Surrey, UK (Pr Dr Nazneen Rachman), ·Ó·ÁÓÒÚÈÛ ÛÙËÓ ÔÈÎÔÁ¤¶·È‰È·ÙÚÈ΋ 2005;68:366-370

™˘˙‹ÙËÛË ∞˘Ùfi ÙÔ Û‡Ó‰ÚÔÌÔ ÂÚÈÁÚ¿ÊËΠÚÒÙË ÊÔÚ¿ ·fi ÙÔÓ Murran, ÙÔ 1873, ˆ˜ molluscum fibrosum. ∏ ÓfiÛÔ˜ ÔÓÔÌ¿ÛÙËΠ·Ú¯Èο “Û‡Ó‰ÚÔÌÔ Murran” Î·È ÙÔ 1962 “Puretic syndrome” ·fi ÙÔ fiÓÔÌ· ÂΛÓÔ˘ Ô˘ ÙÔ ÂÚȤÁÚ·„ ‰Â‡ÙÂÚÔ˜. ∞fi ÙÔ˘˜ Kitano Î·È Û˘Ó, ÙÔ 1972, ηıÈÂÚÒıËÎÂ Ô fiÚÔ˜ Ù˘ Ó·ÓÈ΋˜ ˘·ÏÈÓÈ΋˜ Èӈ̿وÛ˘ (3). ∏ ÎÏËÚÔÓÔÌÈÎfiÙËÙ· Ù˘ ÓfiÛÔ˘ Ê·›ÓÂÙ·È Ó· ·ÎÔÏÔ˘ı› ·˘ÙÔۈ̷ÙÈÎfi ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú· Î·È ÁÈ· ÙÔÓ ÏfiÁÔ ·˘Ùfi Ù· ·ÁfiÚÈ· Î·È Ù· ÎÔÚ›ÙÛÈ· ÚÔÛ‚¿ÏÏÔÓÙ·È ÂÍ›ÛÔ˘. ∆Ô 1/3 ÙˆÓ ÚÔۂ‚ÏËÌ¤ÓˆÓ ·È‰ÈÒÓ Â›Ó·È ‰›‰˘Ì· (4) Î·È Û ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ ÔÈ ÁÔÓ›˜ Â›Ó·È Û˘ÁÁÂÓ›˜. ¢ÂÓ ·Ú·ÙËÚÔ‡ÓÙ·È ‰È·ÊÔÚ¤˜ ÛÙË Û˘¯ÓfiÙËÙ· Ù˘ ÓfiÛÔ˘ ·Ó¿ÏÔÁ· Ì ÙË Ê˘Ï‹, ÂÓÒ ˘¿Ú¯Ô˘Ó Ï›Á˜ ÌfiÓÔ ·Ó·ÊÔÚ¤˜ ÛÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· (5). ∞˘Ù‹ Â›Ó·È Ë ÚÒÙË ÂÚÈÁÚ·Ê‹ Ì ÁÔÓȉȷ΋ ÂȂ‚·›ˆÛË ÛÙËÓ ∂ÏÏ¿‰·. ∆· ÚÒÙ· Û˘ÌÙÒÌ·Ù· ÛÙËÓ Ù˘È΋ ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ ÂÌÊ·Ó›˙ÔÓÙ·È Û˘Ó‹ıˆ˜ ÛÙ· ÚÒÙ· ¯ÚfiÓÈ· ˙ˆ‹˜, ÌÂٷ͇ 3Ô˘ Ì‹Ó· Î·È 4Ô˘ ¤ÙÔ˘˜. ∆· Ù˘Èο ·ÓÙÈÎÂÈÌÂÓÈο Â˘Ú‹Ì·Ù· ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‚Ï·Ùȉ҉ÂȘ Î·È Ô˙Ò‰ÂȘ ‰ÂÚÌ·ÙÈΤ˜ ‚Ï¿‚˜, ΢ڛˆ˜ ÛÙËÓ ÎÂÊ·Ï‹ Î·È Ù·

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∂ÈÎfiÓ· 5. ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË: ÛÙË ‚ÈÔ„›· Ô˙ȉ›ˆÓ ·Ó¢ڛÛÎÂÙ·È ÂÓ·fiıÂÛË ÎÔÏÏ·ÁÔÓÔÔÈË̤ÓÔ˘ ÈÓÒ‰Ô˘˜ ¿ÌÔÚÊÔ˘ ÔÍÂfiÊÈÏÔ˘ ÂÍˆÎ˘ÙÙ¿ÚÈÔ˘ ˘·ÏÈÓÈÎÔ‡ ÈÛÙÔ‡ Ì ÂÚÈÔ¯¤˜ ·˘ÍË̤Ó˘ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜. ¢ÂÓ ·Ú·ÙËÚÂ›Ù·È ÌÈÙˆÙÈ΋ ‹ ¿ÏÏË ·ÓÒÌ·ÏË ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ˘Ú‹ÓˆÓ.

¿ÎÚ·, ÛÙ· ‰¿ÎÙ˘Ï· Î·È ÛÙËÓ ÂÚÈÓÂ˚΋ ÂÚÈÔ¯‹, ˘ÂÚÙÚÔÊ›· ÙˆÓ Ô‡ÏˆÓ, Û˘Áο̄ÂȘ, ·Ú¯Èο ΢ڛˆ˜ ÙˆÓ ÌÂÁ¿ÏˆÓ ·ÚıÚÒÛÂˆÓ Î·È ÂÎÛÂÛËÌ·Ṳ̂ÓË ÔÛÙÂÔÂÓ›·. ∞‡ÍËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ Î·È ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Ô˙ȉ›ˆÓ Ì ÙËÓ ¿ÚÔ‰Ô Ù˘ ËÏÈΛ·˜ Ô‰ËÁ› Û˘¯Ó¿ Û ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÍ·›ÚÂÛË ÁÈ· ·ÈÛıËÙÈÎÔ‡˜ Î·È ÏÂÈÙÔ˘ÚÁÈÎÔ‡˜ ÏfiÁÔ˘˜. ∏ ÚÔԉ¢ÙÈ΋ ‰˘Ûη̄›· Ô‰ËÁ› Û ·‰˘Ó·Ì›· ‚¿‰ÈÛ˘-ÛÙ‹ÚÈ͢ Î·È Î·ı‹ÏˆÛË Û ·Ó·ËÚÈÎfi ·Ì·Í›‰ÈÔ. ∏ ·ÈÙÈÔÏÔÁ›· ÙˆÓ Û˘ÁÎ¿Ì„ÂˆÓ ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈÛÙ›. ∞Ô‰›‰ÂÙ·È Èı·Ó¿ Û ‰È‹ıËÛË ÙˆÓ ı˘Ï¿ÎˆÓ ·fi Ô˙›‰È·. ∏ ÓÔËÙÈ΋ ·Ó¿Ù˘ÍË Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋, ÂÓÒ ‰ÂÓ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÏ·Á¯ÓÈο fiÚÁ·Ó·. ∏ ·ıÔÁ¤ÓÂÈ· Ù˘ ÓfiÛÔ˘ ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈÛÙ› Î·È ‰ÂÓ ˘¿Ú¯Ô˘Ó ¯·Ú·ÎÙËÚÈÛÙÈο ‚ÈÔ¯ËÌÈο Â˘Ú‹Ì·Ù·. £ÂˆÚÂ›Ù·È ˆ˜ ‰È·Ù·Ú·¯‹ ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙÔ˘ ÎÔÏÏ·ÁfiÓÔ˘, ÁÈ·Ù› Û οÔÈÔ˘˜ ·ÛıÂÓ›˜ ¤¯Ô˘Ó ‰È·ÈÛÙˆı› ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÙÔ˘ ÎÔÏÏ·ÁfiÓÔ˘ III (6) Î·È ÙÔ˘ ÎÔÏÏ·ÁfiÓÔ˘ VI (7). ∂ÓÙÔ‡ÙÔȘ, ·˘Ù¤˜ ÔÈ ‰È·Ù·Ú·¯¤˜ ‰ÂÓ ·ÔÙÂÏÔ‡Ó ÛÙ·ıÂÚfi ‡ÚËÌ·, ·ÊÔ‡ ¤¯Ô˘Ó ‚ÚÂı› Î·È Û ·ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó Ê˘ÛÈÔÏÔÁÈÎfi ÎÔÏÏ·ÁfiÓÔ. ÕÏÏÔÈ ÂÚ¢ÓËÙ¤˜ ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ ÔÈ ÌË Ê˘ÛÈÔÏÔÁÈΤ˜ ›Ó˜ ÎÔÏÏ·ÁfiÓÔ˘ ÔÊ›ÏÔÓÙ·È Û ˘ÔΛÌÂÓË ‰È·Ù·Ú·¯‹ ÛÙË ‚ÈÔÛ‡ÓıÂÛË ÙˆÓ ÁÏ˘ÎÔ˙·ÌÈÓÔÁÏ˘Î·ÓÒÓ (8). ∞ÎfiÌË, ÂÈÛËÌ·›ÓÔÓÙ·È ·ÓˆÌ·Ï›Â˜ ÛÙË ‚ÈÔÛ‡ÓıÂÛË Ù˘ ıÂÈÈ΋˜ ¯ÔÓ‰ÚÔ˚Ù›Ó˘ ηÈ/‹ ÙÔ˘ ˘·ÏÔ˘ÚÔÓÈÎÔ‡ ÔͤԘ, ÔÈ Ôԛ˜ ‰ÂÓ ·ÔÙÂÏÔ‡Ó ÛÙ·ıÂÚfi ‡ÚËÌ· Û fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ (3,9,10). ∆¤ÏÔ˜, Èı·ÓfiÙÂÚË ıˆÚÂ›Ù·È Ë ˘fiıÂÛË Ù˘ CMG-2 ÚˆÙ½Ó˘. ∏ ÚˆÙ½ÓË ·˘Ù‹, ·Ó Î·È Ô Ê˘ÛÈÔÏÔÁÈÎfi˜ ÚfiÏÔ˜ Ù˘ ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈÛÙ› (11), Ê·›ÓÂÙ·È fiÙÈ ·›˙ÂÈ ÚfiÏÔ ÛÙËÓ

ÙÚȯÔÂȉÈ΋ ÌÔÚÊÔÁ¤ÓÂÛË, ÛÙËÓ ÔÌÔÈÔÛÙ·Û›· Ù˘ ‰ÔÌ‹˜ Î·È ·Ú¯ÈÙÂÎÙÔÓÈ΋˜ Ù˘ ‚·ÛÈ΋˜ ÌÂÌ‚Ú¿Ó˘, ÛÙË Û‡Ó‰ÂÛË Ù˘ Ï·ÌÈÓ›Ó˘ Ì ÙÔ ÎÔÏÏ·ÁfiÓÔ IV, ÛÙË ‰Ú¿ÛË ÙÔ˘ von Willebrant A ·Ú¿ÁÔÓÙÔ˜ (vWA), ·ÏÏ¿ Î·È ˆ˜ ΢ÙÙ·ÚÈÎfi˜ ˘Ô‰Ô¯¤·˜ Ù˘ ·ÓıÚ·ÎÈ΋˜ ÙÔ͛Ӣ (11,12). ª¤¯ÚÈ Û‹ÌÂÚ· ¤¯Ô˘Ó ·Ó·ÁÓˆÚÈÛÙ› 8 ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ Ô˘ Έ‰ÈÎÔÔÈ› ÙË Û˘ÁÎÂÎÚÈ̤ÓË ÚˆÙ½ÓË Î·È Â‰Ú¿˙ÂÙ·È ÛÙÔ 4q21 ¯ÚˆÌfiۈ̷, Ì ÎÏÈÓÈ΋ ÂΉ‹ÏˆÛË Ó·ÓÈ΋˜ ˘·ÏÈÓÈ΋˜ Èӈ̿وÛ˘. ∞Ó¿ÏÔÁË ÌÂÙ¿ÏÏ·ÍË Ì ÙËÓ ·ÛıÂÓ‹ Ù˘ ·ÚÔ‡Û·˜ ÂÚ›ÙˆÛ˘ ¤¯ÂÈ ‚ÚÂı› Û ÙÚÂȘ ·ÎfiÌË ·ÛıÂÓ›˜ Ì Ó·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË (2 ÛÙËÓ ∆Ô˘ÚΛ· Î·È 1 ÛÙËÓ ∂˘ÚÒË) (12). ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ú¤ÂÈ Ó· Á›ÓÂÙ·È ·fi: ·) Û˘ÁÁÂÓ‹ ·ÚıÚÔÁÚ‡ˆÛË (‰ÂÓ ¤¯ÂÈ ‰ÂÚÌ·ÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ), ‚) Û‡Ó‰ÚÔÌÔ Winchester (ÌÈÎÚÔۈ̛·, Û˘Áο̄ÂȘ ·fi ÙË Á¤ÓÓËÛË, ¯ˆÚ›˜ ‰ÂÚÌ·ÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ, ÂÓÒ ˘Ê›ÛÙ·Ù·È ıfiψÛË ÙÔ˘ ÎÂÚ·ÙÔÂȉԇ˜), Á) ÓfiÛÔ Fabry (ÛÊÈÁÁÔÏÈ›‰ˆÛË, Û˘ÛÙËÌ·ÙÈÎfi ÓfiÛËÌ· ¯ˆÚ›˜ Û˘Áο̄ÂȘ, Ì ·ÁÁÂÈÔÎÂÚ·ÙÒÌ·Ù·, Ó¢ÚÔ¿ıÂÈ· Î·È ¤ÏÏÂÈ„Ë ÙÔ˘ ÂÓ˙‡ÌÔ˘ ÎÂÚ·Ìȉ¿ÛË) Î·È ‰) ‚ÚÂÊÈ΋ Û˘ÛÙËÌ·ÙÈ΋ ˘·Ï›ÓˆÛË - infantile systematic hyalinosis (ÚfiÎÂÈÙ·È Èı·ÓÒ˜ ÁÈ· ÙÔ ›‰ÈÔ ÓfiÛËÌ·, ·ÏÏ¿ Ì ‚·Ú‡Ù·ÙË ÎÏÈÓÈ΋ ÂÈÎfiÓ· ·fi ÙË Á¤ÓÓËÛË, ÌÂ Û˘ÌÌÂÙÔ¯‹ ÙˆÓ ÛÏ·Á¯ÓÈÎÒÓ ÔÚÁ¿ÓˆÓ Î·È Ù˘ ηډȿ˜, Ì ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÏÂÈÌÌ· Î·È Â·ÎfiÏÔ˘ı˜ ÏÔÈÌÒÍÂȘ Î·È ı¿Ó·ÙÔ Ù· ‰‡Ô ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜). ¢ÂÓ ˘¿Ú¯ÂÈ ·ÈÙÈÔÏÔÁÈ΋ ıÂÚ·›·. ™˘ÓÙËÚËÙÈο ¤¯Ô˘Ó ‰ÔÎÈÌ·ÛÙ› Ú·‰ÈÔıÂÚ·›·, ¯ËÌÂÈÔıÂÚ·›·, ÚˆÙÂÔÏ˘ÙÈο ¤Ó˙˘Ì· Î·È ÙÔÈΤ˜ ÂÁ¯‡ÛÂȘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ, ¯ˆÚ›˜ ·ÔÙ¤ÏÂÛÌ·. OÈ ¯ÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ ‰›ÓÔ˘Ó ÚÔÛˆÚÈÓ¤˜ χÛÂȘ Û ·ÈÛıËÙÈο Î·È ÏÂÈÙÔ˘ÚÁÈο ÚÔ‚Ï‹Ì·Ù·. ∏ ÚfiÁÓˆÛË Â›Ó·È È‰È·ÈÙ¤Úˆ˜ ÂÈÊ˘Ï·ÎÙÈ΋ ¤ˆ˜ ÂÈ‚·Ú˘Ì¤ÓË. ™ÙËÓ ·ÛıÂÓ‹ Ù˘ ·ÚÔ‡Û·˜ ÂÚ›ÙˆÛ˘ Ë ¯ÔÚ‹ÁËÛË Botulin toxinum A ÛÙËÓ ÎÚÔÙ·ÊÔÁÓ·ıÈ΋ ÂÚÈÔ¯‹ Î·È ÂÚÈÛÙÔÌ·ÙÈο ‚ÂÏÙ›ˆÛ ·ÈÛıËÙ¿ ÙË ‰È¿ÓÔÈÍË ÙÔ˘ ÛÙfiÌ·ÙÔ˜ Î·È ‰È¢ÎfiÏ˘Ó ÙË Ì¿ÛËÛË. ∆Ô Û‡ÌÏÔÎÔ Ù˘ Ó¢ÚÔÙÔ͛Ӣ Clostridium botulinum Ù‡Ô˘ ∞, ˆ˜ ÁÓˆÛÙfiÓ ·ÔÎÏ›ÂÈ ÙËÓ ÂÚÈÊÂÚÈ΋ ·ÂÏ¢ı¤ÚˆÛË Ù˘ ·ÎÂÙ˘ÏÔ¯ÔÏ›Ó˘ ÛÙȘ ÚÔÛ˘Ó·ÙÈΤ˜ Ó¢ÚÈΤ˜ ·ÔÏ‹ÍÂȘ ̤ۈ Ù˘ ·Ô‰fiÌËÛ˘ Ù˘ SNAP-25 ÚˆÙ½Ó˘, Ì ·ÔÙ¤ÏÂÛÌ· ÙÔÈ΋, ·ÚÔ‰È΋ Î·È Ì˘Ô¯·Ï·ÚˆÙÈ΋ ¢ÂÚÁÂÙÈ΋ ‰Ú¿ÛË ÁÈ· ÙËÓ ·ÛıÂÓ‹ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘. ªÂÙ¿ ÙË ÁÓÒÛË Ù˘ ÌÂÙ¿ÏÏ·Í˘, Ë ‰˘Ó·ÙfiÙËÙ· ÚÔÁÂÓÓËÙÈÎÔ‡ ¤ÏÂÁ¯Ô˘ Û ÂӉ¯fiÌÂÓË ¶·È‰È·ÙÚÈ΋ 2005;68:366-370

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O. ¶··Ó‰Ú¤Ô˘ Î·È Û˘Ó.

ÌÂÏÏÔÓÙÈ΋ ÂÁ΢ÌÔÛ‡ÓË, ·ÔÙÂÏ› ÚÔ¸fiıÂÛË ÁÈ· ÙËÓ ·fiÎÙËÛË ÂÓfi˜ ˘ÁÈÔ‡˜ ·È‰ÈÔ‡.

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2. Ishikawa H, Maeda H, Takamatsu H, Saito Y. Systemic hyalinosis (juvenile hyaline fibromatosis). Ultrastructure of the hyaline with particular reference to the cross-banded structure. Arch Dermatol Res 1979;265:195-206.

3. Kitano Y, Horiki M, Aoki T, Sagami S. Two cases of juvenile hyalin fibromatosis. Some histological, electron microscopic, and tissue culture observations.

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4. Keser G, Karabulut B, Oksel F, Calli C, Ustun EE, Akalin T et al. Two siblings with juvenile hyaline fibromatosis: case reports and review of the literature.

Clin Rheumatol 1999;18:248-252.

5. Allen PW. Selected case from the Arkadi M. Rywlin International Pathology Slide Seminar: hyaline fibromatosis. Adv Anat Pathol 2001;8:173-178.

6. Lubec B, Steinert I, Breier F, Jurecka W, Pillwein K, Fang-Kircher S. Skin collagen defects in a patient with juvenile hyaline fibromatosis. Arch Dis Child 1995;73:246-248.

¶·È‰È·ÙÚÈ΋ 2005;68:366-370

7. Kayashima K, Katagiri K, Shinkai H, Ono T. Is juvenile hyaline fibromatosis a disease of type VI collagenosis? In: Ishibashi Y, Nakagawa H, Suzuki H, editors. Electron microscopy in dermatology: basic and clinical research. Amsterdam: Excerpta Medica; 1994. p. 329-334.

8. Remberger K, Krieg T, Kunze D, Weinmann HM, Hubner G. Fibromatosis hyalinica multiplex (juvenile hyalin fibromatosis). Light microscopic, electron microscopic, immunohistochemical, and biochemical findings. Cancer 1985;56:614-624.

9. Iwata S, Horiuchi R, Maeda H, Ishikawa H. Systemic hyalinosis or juvenile hyaline fibromatosis. Ultrastructural and biochemical study of cultured skin fibroblasts. Arch Dermatol Res 1980;267:115-121.

10. Breier F, Fang-Kircher S, Wolff K, Jurecka W. Juvenile hyaline fibromatosis: impaired collagen metabolism in human skin fibroblasts. Arch Dis Child 1997;77:436-440.

11. Dowling O, Difeo A, Ramirez MC, Tukel T, Narla G, Bonafe L et al. Mutations in capillary morphogenesis gene-2 result in the allelic disorders juvenile hyaline fibromatosis and infantile systemic hyalinosis.

Am J Hum Genet 2003;73:957-966.

12. Hanks S, Adams S, Douglas J, Arbour L, Atherton DJ, Balci S et al. Mutations in the gene encoding capillary morphogenesis protein 2 cause juvenile hyaline fibromatosis and infantile systemic hyalinosis. Am J Hum Genet 2003;73:791-800.

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CASE REPORT

371

Juvenile hyaline fibromatosis.

The first case report in Greece O. Papandreou1, A. Klimentopoulou1, N. Poniros2, A. Konstantinidou3, A. Xypolita - Zachariadi1, N. Rachman4, G. Chroussos1

Abstract Juvenile hyaline fibromatosis is a rare connective tissue disease with autosomal recessive inheritance.

The gene is located on the 4q21 chromosome. It usually presents during the first years of life with multiple painless subcutaneous nodules, hyalinous cutaneous lesions of various sizes, gingival hypertrophy, limb contractures and low bone density. The visceral organs are not affected. Mental development is normal. The diagnosis is confirmed by biopsy of the lesions. There is no specific treatment other than numerous operations for functional and aesthetic reasons. The case is described of a 4 year-old female with failure to thrive, limb contractures, rigid torsus, gingival hypertrophy, subcutaneous nodules, and cutaneous nodules and macules especially on the head, fingers and toes, causing considerable disability. Administration of Botox injections (Botulin toxinum A) has temporarily improved the patient’s quality of life, rendering her able to chew and speak again, as before.

Key words Nodules, macules, fibromatosis.

1 1st Paediatric Clinic of the University of Athens, “Aghia Sophia” Children’s Hospital, Athens 2 Plastic Surgery Clinic, “Aghia Sophia” Children’s Hospital, Athens 3 Laboratory of PathologyAnatomy, “Aghia Sophia” Children’s Hospital, Athens 4 Institute of Cancer Research, Surrey, UK Correspondence: Ourania Papandreou 1st Paediatric Clinic of the University of Athens, “Aghia Sophia” Children’s Hospital Thivon & Levadias, 115 27, Athens E-mail: alkliment@hotmail.com Date of submission: 26-07-2004 Date of approval: 01-04-2005

Paediatriki 2005;68:371

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∞. ∞Ó‰Ú¤Ô˘1, µ. ∞ÏÂÍ·Ó‰ÚÔÔ‡ÏÔ˘ - ∆ÛÈÎڛη2, ∞. ™·ÎÂÏÏ·ÚÔÔ‡ÏÔ˘1, ª. °Ô˘‰ÂÛ›‰Ô˘2, §. ºÏˆÚÂÓÙ›Ó - ∞Ú¿Ú3

AÏÏËÏÔÁÚ·Ê›·: ∞ϤͷӉÚÔ˜ ∞Ó‰Ú¤Ô˘ ÃÂÈ̈ӛ‰Ô˘ 15 ∆.∫. 551 33, ∫·Ï·Ì·ÚÈ¿, £ÂÛÛ·ÏÔÓ›ÎË ∂-mail: aalex@the.forthnet.gr

¶ÂÚ›ÏË„Ë ¶ÂÚÈÁÚ¿ÊÂÙ·È Ì›· ÔÈÎÔÁ¤ÓÂÈ· Ì ۇӉÚÔÌÔ ÂÏÏ›ÌÌ·ÙÔ˜ ÙÔ˘ ¯ÚˆÌÔÛˆÌÈÎÔ‡ ÙÌ‹Ì·ÙÔ˜ 22q11.2 Û ÙÚ›· ̤ÏË Ù˘ Î·È ÌÂÁ¿ÏË ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔÓ Ê·ÈÓfiÙ˘Ô. ∫ÏÈÓÈο, ÙÔ Û‡Ó‰ÚÔÌÔ ÂΉËÏÒıËΠÛÙË ÌËÙ¤Ú· Ì ¤ÓÚÈÓË ÔÌÈÏ›· ÏfiÁˆ ˘ÂÚˆÈÔÊ·Ú˘ÁÁÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜, ÛÙÔ ÙÚ›ÙÔ ·È‰› Ì ÎÏ·ÛÈÎfi Û‡Ó‰ÚÔÌÔ DiGeorge, Ô˘ ÂÚÈÂÏ¿Ì‚·Ó ÎÔÈÓfi ·ÚÙËÚÈ·Îfi ÎÔÚÌfi Ù‡Ô˘ ππ, ˘·Û‚ÂÛÙÈ·ÈÌ›· Î·È ·Ï·Û›· ÙÔ˘ ı‡ÌÔ˘ Ì ÙÂÏÈ΋ ηٿÏËÍË ÙÔÓ ı¿Ó·ÙÔ ·fi ‚·ÚÈ¿ Û‹„Ë, ÂÓÒ ÛÙÔ Ù¤Ù·ÚÙÔ ·È‰› Ì ÌÈÎÚÔÁÓ·ı›· Î·È ·ÚÔ‰È΋ ˘·Û‚ÂÛÙÈ·ÈÌ›·. ∏ ‰È¿ÁÓˆÛË ¤ÁÈÓ ·Ú¯Èο ÛÙÔ ÙÚ›ÙÔ ·È‰› Î·È ÌÂÙ¿ ÛÙË ÌËÙ¤Ú·. ™ÙÔ Ù¤Ù·ÚÙÔ ·È‰› Ë ‰È¿ÁÓˆÛË ¤ÁÈÓ ÚÔÁÂÓÓËÙÈο ÌÂÙ¿ ·fi ·ÌÓÈÔ·Ú·Î¤ÓÙËÛË. ™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ Ë ‰È¿ÁÓˆÛË ¤ÁÈÓ Ì ÙËÓ Ù¯ÓÈ΋ ÙÔ˘ ÊıÔÚ›˙ÔÓÙ· in situ ˘‚ÚȉÈÛÌÔ‡ (FISH).

∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 27-09-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 29-03-2005

§¤ÍÂȘ ÎÏÂȉȿ ™‡Ó‰ÚÔÌÔ ÂÏÏ›ÌÌ·ÙÔ˜ 22q11, ÔÈÎÔÁÂÓ‹˜ ÂÌÊ¿ÓÈÛË, ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË, Ù¯ÓÈ΋ FISH.

∂ÈÛ·ÁˆÁ‹ ∆Ô Û‡Ó‰ÚÔÌÔ ÂÏÏ›ÌÌ·ÙÔ˜ ÛÙÔ ¯ÚˆÌfiۈ̷ 22q11 ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÌÂÁ¿ÏË ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔÓ Ê·ÈÓfiÙ˘Ô Î·È ÛÙȘ Û˘Ó˘¿Ú¯Ô˘Û˜ ·ÓˆÌ·Ï›Â˜ ·fi ÙËÓ Î·Ú‰È¿, ÙÔÓ ı‡ÌÔ Î·È ÙÔ˘˜ ·Ú·ı˘ÚÂÔÂȉ›˜ ·‰¤Ó˜, ·ÎfiÌË Î·È ·Ó¿ÌÂÛ· Û ¿ÙÔÌ· Ù˘ ›‰È·˜ ÔÈÎÔÁ¤ÓÂÈ·˜ (1-4). ∆· Ê·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο, fiˆ˜ ÎÚ·ÓÈÔÚÔÛˆÈΤ˜, ηډȷÁÁÂȷΤ˜, ˆÙÔÚÈÓÔÏ·Ú˘ÁÁÔÏÔÁÈΤ˜, Ì˘ÔÛÎÂÏÂÙÈΤ˜, ÂÓ‰ÔÎÚÈÓÔÏÔÁÈΤ˜ Î·È ·ÓÔÛÔÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜, ÌÔÚ› Ó· ‰È·Ê¤ÚÔ˘Ó ·ÎfiÌË Î·È ÌÂٷ͇ ÌÔÓÔˆÔÁÂÓÒÓ ‰È‰‡ÌˆÓ (5). ∏ ‚·Ú‡ÙËÙ· ̤۷ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÌÔÚ› Ó· ÔÈΛÏÏÂÈ ·fi ·Û˘Ìو̷ÙÈÎÔ‡˜ ÁÔÓ›˜ ̤¯ÚÈ ÛÔ‚·Ú¿ ηı˘ÛÙÂÚË̤ӷ Î·È ‰˘ÛÌÔÚÊÈο ·È‰È¿ (3). ∏ ÂÚÈÔ¯‹ ÙÔ˘ ÂÏÏ›ÌÌ·ÙÔ˜ ÛÙË ¯ÚˆÌÔÛˆÌÈ΋ ı¤ÛË 22q11.2 ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· ÂÚÈÁڷʤÓÙ· ·Ï·ÈfiÙÂÚ· Û‡Ó‰ÚÔÌ· DiGeorge Î·È ˘ÂÚˆÈÔ-ηډÈÔÚÔÛˆÈÎfi (velo-cardio-facial), ηıÒ˜ Î·È ¿ÙÔÌ· Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙÔ Û‡Ó‰ÚÔÌÔ conotruncal abnormal face (6). ¶ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ¤¯ÂÈ ·Ó·ÊÂÚı› Û ϛÁ· Û¯ÂÙÈο ÂÚÈÛÙ·ÙÈο (7,8). ¶ÂÚÈÁÚ¿ÊÂÙ·È, ÁÈ· ÚÒÙË ÊÔÚ¿ ÛÙËÓ ÂÏÏËÓÈ΋ ‚È‚ÏÈÔÁÚ·Ê›·, ¤Ó· ÔÈÎÔÁÂÓ¤˜ Û‡Ó‰ÚÔÌÔ ÌÈÎÚÔÂÏÏ›ÌÌ·ÙÔ˜ 22q11.2, Ô˘ ·ÊÔÚ¿ ÛÙË ÌËÙ¤Ú· Î·È ÛÙ· ‰‡Ô ·fi Ù· Ù¤ÛÛÂÚ· ·È‰È¿ Ù˘. ™ÙÔ ¶·È‰È·ÙÚÈ΋ 2005;68:372-375

‰Â‡ÙÂÚÔ ¿Û¯ÔÓ ·È‰› Ë ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ¤ÁÈÓ ÚÔÁÂÓÓËÙÈο.

¶ÂÚÈÁÚ·Ê‹ ÂÚÈÙÒÛÂˆÓ ¶ÚfiÎÂÈÙ·È ÁÈ· Ì›· ÔÈÎÔÁ¤ÓÂÈ· 3 ÁÔÓ¤ˆÓ (Ì›· ÌËÙ¤Ú·, ‰‡Ô ·Ù¤Ú˜) Î·È 4 ·È‰ÈÒÓ (‰‡Ô ·fi οı ·Ù¤Ú·). O ¤ÏÂÁ¯Ô˜ ÙˆÓ ¯ÚˆÌÔÛˆÌ¿ÙˆÓ Ì ÙË Ì¤ıÔ‰Ô FISH ¤‰ÂÈÍ fiÙÈ Ë ÌËÙ¤Ú· Î·È Ù· 2 ·È‰È¿ ·fi ÙÔÓ ›‰ÈÔ ·Ù¤Ú·, ÙÔ ÙÚ›ÙÔ Î·È ÙÔ Ù¤Ù·ÚÙÔ, ›¯·Ó ÌÈÎÚÔ¤ÏÏÂÈÌÌ· ÛÙÔ ¯ÚˆÌfiۈ̷ 22q11.2. ∏ ÌËÙ¤Ú· (ËÏÈΛ·˜ 37 ÂÙÒÓ) Î·È Ô ·Ù¤Ú·˜ (ËÏÈΛ·˜ 40 ÂÙÒÓ) ÙˆÓ ‰‡Ô ·Û¯fiÓÙˆÓ ·È‰ÈÒÓ ‰ÂÓ Â›¯·Ó Û˘ÁÁ¤ÓÂÈ· ÌÂٷ͇ ÙÔ˘˜. O ¤ÏÂÁ¯Ô˜ ÁÈ· ¤ÏÏÂÈÌÌ· 22q11 ÛÙÔÓ ·Ù¤Ú· ÙˆÓ ‰‡Ô ·Û¯fiÓÙˆÓ ·È‰ÈÒÓ ‹Ù·Ó ·ÚÓËÙÈÎfi˜. ∆· ¿ÏÏ· ‰‡Ô ·È‰È¿, ÙÔ ÚÒÙÔ Î·È ÙÔ ‰Â‡ÙÂÚÔ ·fi ‰È·ÊÔÚÂÙÈÎfi ·Ù¤Ú·, ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο Î·È Ô ¤ÏÂÁ¯Ô˜ Ì FISH ÛÙÔ ¤Ó· ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜, ÂÓÒ ÛÙÔ ¿ÏÏÔ ‰ÂÓ ¤¯ÂÈ Á›ÓÂÈ ·ÎfiÌ·. ªËÙ¤Ú·: ∏ ‰È¿ÁÓˆÛË ÛÙË ÌËÙ¤Ú· ¤ÁÈÓ ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÛÙÔ ÚÒÙÔ ¿Û¯ÔÓ ·È‰› Ù˘. ∏ ÌËÙ¤Ú· ›¯Â ¤ÓÚÈÓË ÔÌÈÏ›· Î·È ·Ó¤ÊÂÚÂ Û˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ ÛÙËÓ ·È‰È΋ ËÏÈΛ·. ∂›¯Â ÛÔ‚·Ú¤˜ Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜ Î·È ·Ú·ÎÔÏÔ‡ıËÛ ÌfiÓÔ ÙÔ ‰ËÌÔÙÈÎfi Û¯ÔÏ›Ô. ¢ÂÓ ·Ó¤ÊÂÚ ÈÛÙÔÚÈÎfi Û·ÛÌÒÓ ‹ ÙÚfiÌÔ. ∞fi

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373

™‡Ó‰ÚÔÌÔ ÌÈÎÚÔÂÏÏ›ÌÌ·ÙÔ˜ 22q11.2

fiÛÔ ı˘Ì¿Ù·È ‰ÂÓ ˘‹Ú¯·Ó ‰˘ÛÌÔÚʛ˜, Û·ÛÌÔ›, Û˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ, ˘·Û‚ÂÛÙÈ·ÈÌ›·, „˘¯ÔÎÔÈÓˆÓÈο ÚÔ‚Ï‹Ì·Ù·, ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË ‹ „˘¯È·ÙÚÈο ÚÔ‚Ï‹Ì·Ù· Û ¿ÏÏ· ̤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ¿˜ Ù˘. ∆Ú›ÙÔ ·È‰›: ¶ÚfiÎÂÈÙ·È ÁÈ· ı‹Ï˘ ÓÂÔÁÓfi, ÙÔ ÚÒÙÔ ·È‰› Ê·ÈÓÔÌÂÓÈο ˘ÁÈÒÓ ÁÔÓ¤ˆÓ Ô˘ ÌÂٷʤÚıËΠÛÙÔ ÓÔÛÔÎÔÌÂ›Ô Û ËÏÈΛ· 3 ËÌÂÚÒÓ ÌÂ Û˘Ìو̷ÙÔÏÔÁ›· ηډȷ΋˜ ο̄˘. ∏ ·ËÛË (40 ‚‰ÔÌ¿‰ˆÓ) Î·È Ô ÙÔÎÂÙfi˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. ¢ÂÓ ·Ú·ÙËÚ‹ıËΠ˘‰Ú¿ÌÓÈÔ Î·È Ë ÙÈÌ‹ Ù˘ ·-ÂÌ‚Ú˘˚΋˜ ÚˆÙ½Ó˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∏ ÂͤٷÛË ÁÈ· ÙËÓ ‡·ÚÍË ·˘ÛÙÚ·ÏÈ·ÓÔ‡ ·ÓÙÈÁfiÓÔ˘ ÛÙË ÌËÙ¤Ú· ‹Ù·Ó ıÂÙÈ΋. ∆Ô µ° ‹Ù·Ó 3200 g (~50‹ ∂£), Ë ¶∫ 34 cm (~50‹ ∂£) Î·È ÙÔ ª™ ‹Ù·Ó 49 cm (~50‹ ∂£). ∏ ‚·ıÌÔÏÔÁ›· Apgar ‹Ù·Ó 8 ÛÙÔ 1Ô ÏÂÙfi Î·È 10 ÛÙÔ 5Ô. ∞̤ۈ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ¯ÔÚËÁ‹ıËΠ˘ÂÚ¿ÓÔÛÔ˜ ÛÊ·ÈÚ›ÓË Î·È Ì›· ‰fiÛË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ηٿ Ù˘ Ë·Ù›Ùȉ·˜ µ. ∆Ë 2Ë Ë̤ڷ ˙ˆ‹˜ ÙÔ ÓÂÔÁÓfi ÂÌÊ¿ÓÈÛ ˆ¯ÚfiÙËÙ·, ÓˆıÚfiÙËÙ·, ¿ÚÓËÛË Ï‹„˘ ÙÚÔÊ‹˜, „ËÏ·ÊËÙfi ‹·Ú 3 cm Î·È Û˘ÛÙÔÏÈÎfi ʇÛËÌ· 1-2/6. O ˘ÂÚ˯ÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ù˘ ηډȿ˜ ¤‰ÂÈÍ ÙËÓ ‡·ÚÍË ÎÔÈÓÔ‡ ·ÚÙËÚÈ·ÎÔ‡ ÎÔÚÌÔ‡ Ù‡Ô˘ ππ Ì ÌÂÛÔÎÔÈÏȷ΋ ÂÈÎÔÈÓˆÓ›·. ∫·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÛÙÔ ÓÔÛÔÎÔÌ›Ô, ÙÔ ÓÂÔÁÓfi ›¯Â Úfi‰ÈÓÔ ¯ÚÒÌ·, Ù·¯‡ÓÔÈ·, Ù·¯˘Î·Ú‰›·, ¤ÓÙÔÓÔ ·’ ÙfiÓÔ, Û˘ÛÙÔÏÈÎfi ʇÛËÌ· ÂÍÒıËÛ˘ 1-2/6 ·ÎÔ˘ÛÙfi ÛÙȘ ÂÛٛ˜ ·ÎÚfi·Û˘ Ù˘ Ó¢ÌÔÓÈ΋˜ Î·È ·ÔÚÙÈ΋˜ ‚·Ï‚›‰·˜, ηϋ ÂÚÈÊÂÚÂȷ΋ ΢ÎÏÔÊÔÚ›· Î·È ¤ÓÙÔÓ˜ ÂÚÈÊÂÚÈΤ˜ ÛʇÍÂȘ. ∏ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη ¤‰ÂÈÍ ·˘ÍË̤ÓË Ó¢ÌÔÓÈ΋ ·ÁÁ›ˆÛË. O ·ÈÌ·ÙÔÏÔÁÈÎfi˜ Î·È ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ: ÏÂ˘Î¿ 13900, ¶ 83,1%, § 11,4%, ª.ª. 5,5%, Ca++=0,73 mmol/l, Mg=1,4 mg/dl, P=9,1 mg/dl Î·È ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË 142 IU/L. H PTH ‹Ù·Ó 71 pg/ml, Ì ÙÈÌ‹ Ca++=0,93 mmol/l. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ¤‰ÂÈÍ ˘ÔÏ·ÛÙÈÎfi ı‡ÌÔ, ÂÓÒ ÔÈ ·Ú·ı˘ÚÂÔÂȉ›˜ ‰ÂÓ ·ÂÈÎÔÓ›ÛÙËηÓ. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÙˆÓ ÓÂÊÚÒÓ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi. ™ÙÔÓ ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ‰È·ÈÛÙÒıËΠ·Ô˘Û›· ·ÓÔÛÔ˚ηÓÒÓ ∆-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ, ÏfiÁˆ ÌË ‰È·ÊÔÚÔÔ›ËÛ‹˜ ÙÔ˘˜ Û ÏÂÈÙÔ˘ÚÁÈÎÔ‡˜ ÏËı˘ÛÌÔ‡˜. O ¯ÚˆÌÔÛˆÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÛÙÔ ¯ÚˆÌfiۈ̷ 22 Ì ÙËÓ Ù¯ÓÈ΋ FISH Î·È ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÊıÔÚ›˙ÔÓÙ· DNA ·ÓȯÓÂ˘Ù‹ ¤‰ÂÈÍ ÌÈÎÚÔ¤ÏÏÂÈÌÌ· ÛÙË ı¤ÛË 22q11.2. ∏ ˘·Û‚ÂÛÙÈ·ÈÌ›· ·ÔηٷÛÙ¿ıËΠÙË 12Ë Ë̤ڷ ÓÔÛËÏ›·˜ ÌÂÙ¿ ·fi ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ÛÙÔȯÂÈ·ÎÔ‡ ·Û‚ÂÛÙ›Ô˘ Î·È ‚ÈÙ·Ì›Ó˘ D ·fi ÙÔ ÛÙfiÌ·. ™ÙÔ ÓÂÔÁÓfi ¤ÁÈÓ ÂÚÈÔÚÈÛÌfi˜ ÙˆÓ

¯ÔÚËÁÔ‡ÌÂÓˆÓ ˘ÁÚÒÓ Î·È ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ÊÔ˘ÚÔÛÂÌ›‰Ë˜. ™Â ËÏÈΛ· 18 ËÌÂÚÒÓ ¤ÁÈÓ ÌÂÙ·ÊÔÚ¿ Û ·È‰ÔηډÈÔ¯ÂÈÚÔ˘ÚÁÈÎfi ΤÓÙÚÔ ÁÈ· ¯ÂÈÚÔ˘ÚÁÈ΋ ‰ÈfiÚıˆÛË Ù˘ Û˘ÁÁÂÓÔ‡˜ ηډÈÔ¿ıÂÈ·˜. §›Á˜ Ë̤Ú˜ ·ÚÁfiÙÂÚ· Î·È ÚÈÓ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË ·Ó¤Ù˘Í ۋ„Ë ·fi „¢‰ÔÌÔÓ¿‰· Î·È ·‚›ˆÛÂ. ∆¤Ù·ÚÙÔ ·È‰›: ¶ÚfiÎÂÈÙ·È ÁÈ· ·ÁfiÚÈ, Ô˘ ÌÂٷʤÚıËΠÛÙËÓ ÎÏÈÓÈ΋ Û ËÏÈΛ· 28 ˆÚÒÓ ÏfiÁˆ ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Û˘Ó‰ÚfiÌÔ˘ ÌÈÎÚÔÂÏÏ›ÌÌ·ÙÔ˜ 22q11.2. ∞fi ÙÔ ÂÚÈÁÂÓÓËÙÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È fiÙÈ Ë ÌËÙ¤Ú· ›¯Â ÌÈÎÚÔ·ÈÌÔÚÚ·Á›Â˜ ÛÙÔÓ 3Ô-4Ô Ì‹Ó· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ¤ÁÈÓ Ì ¯ÚˆÌÔÛˆÌÈ΋ ·Ó¿Ï˘ÛË Ì ÙË Ì¤ıÔ‰Ô FISH Û ·ÌÓȷο ·ÙÙ·Ú· Ô˘ ÂÏ‹ÊıËÛ·Ó Ì ·ÌÓÈÔΤÓÙËÛË ÙËÓ 23Ë Â‚‰ÔÌ¿‰· ·ËÛ˘. ∏ Á¤ÓÓËÛË ¤ÁÈÓ ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ÙÔÎÂÙfi ÌÂÙ¿ ·fi 38 ‚‰ÔÌ¿‰Â˜ ·ËÛ˘. ∆Ô ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ ‹Ù·Ó 2630 g (75Ë ∂£ - 90‹ ∂£), Ë ¶∫ 36 cm (>90‹ ∂£) Î·È ÙÔ ª™ 48 cm (~50‹ ∂£). ∏ ‚·ıÌÔÏÔÁ›· Apgar ‹Ù·Ó 8 ÛÙÔ 1Ô ÏÂÙfi Î·È 9 ÛÙÔ 5Ô. ∞̤ۈ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ¯ÔÚËÁ‹ıËΠ˘ÂÚ¿ÓÔÛÔ˜ Á-ÛÊ·ÈÚ›ÓË Î·È ÂÌ‚fiÏÈÔ Î·Ù¿ Ù˘ Ë·Ù›Ùȉ·˜ µ. ∫·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹, Ë ÎÏÈÓÈ΋ ÂͤٷÛË - ÂÎÙfi˜ ·fi ‹È· ÌÈÎÚÔÁÓ·ı›· - ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. O ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ: ÏÂ˘Î¿ 12800, ¶ 67,1%, § 18,3%, ª.ª. 10,9%, Ca++=0,95 mmol/l. ∏ ˘·Û‚ÂÛÙÈ·ÈÌ›· ·ÓÙÈÌÂÙˆ›ÛÙËΠ̠¯ÔÚ‹ÁËÛË ·fi ÙÔ ÛÙfiÌ· ÁÏ˘ÎÔÓÈÎÔ‡ ·Û‚ÂÛÙ›Ô˘ ÁÈ· 14 Ë̤Ú˜. ™ÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη ‰ÂÓ ·ÂÈÎÔÓÈ˙fiÙ·Ó Ô ı‡ÌÔ˜ ·‰¤Ó·˜. ¢ÂÓ ¤ÁÈÓ ˘ÂÚ˯ÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ı‡ÌÔ˘ Î·È ÙˆÓ ·Ú·ı˘ÚÂÔÂȉÒÓ ·‰¤ÓˆÓ. O ˘ÂÚ˯ÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ù˘ ηډȿ˜, ÙˆÓ ÌÂÁ¿ÏˆÓ ·ÁÁ›ˆÓ Î·È ÙˆÓ ÓÂÊÚÒÓ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. O ·ÓÔÛÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍ ÔÚȷο ¯·ÌËÏfi ÔÛÔÛÙfi ·ÓÔÛÔ˚ηÓÒÓ ∆-΢ÙÙ¿ÚˆÓ Î·È ÙˆÓ ˘ÔÔÌ¿‰ˆÓ ÙÔ˘˜, ηıÒ˜ Î·È ÂÏ¿ÙÙˆÛË ÙˆÓ ÔÛÔÛÙÒÓ ¤ÎÊÚ·Û˘ ÙˆÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ÙˆÓ Ô˘‰ÂÙÂÚfiÊÈψÓ. ∆Ô ÓÂÔÁÓfi ·ÚÔ˘Û›·Û ‰˘ÛÎÔÏ›· ÛÙË Û›ÙÈÛË Ù· ‰‡Ô ÚÒÙ· 24ˆÚ· ˙ˆ‹˜, Ì ÂÏ·Ùو̤ÓË ÚfiÛÏË„Ë Á¿Ï·ÎÙÔ˜, ηıÒ˜ Î·È ÏÂÙfi ÙÚfiÌÔ ÙˆÓ ¿ÎÚˆÓ. ¡ÔÛËχÙËΠ17 Ë̤Ú˜ Î·È ÂÍ‹Ïı Ì ԉËÁ›Â˜ ÁÈ· ÚÔÛ‰ÈÔÚÈÛÌfi ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ·›Ì·ÙÔ˜ Î·È ÙˆÓ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ Û ٷÎÙ¿ ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù·, ηıÒ˜ Î·È Û ÂÚÈÙÒÛÂȘ ÛÙÚ˜, ÏÔÈÌÒ͈Ó, ÂÌÊ¿ÓÈÛ˘ ¢ÂÚÂıÈÛÙfiÙËÙ·˜, ÙÚfiÌÔ˘ ‹ Û·ÛÌÒÓ. ª¤¯ÚÈ Û‹ÌÂÚ· (ËÏÈΛ· 2 ÂÙÒÓ) ‰ÂÓ ·ÚÔ˘Û›·Û ÙÚfiÌÔ, Û·ÛÌÔ‡˜ ‹ Û˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ Î·È Ô Ù·ÎÙÈÎfi˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ Î·È ÙˆÓ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ ¶·È‰È·ÙÚÈ΋ 2005;68:372-375

Pediatr Sept-Oct 05

17-10-05

16:52

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374

∞. ∞Ó‰Ú¤Ô˘ Î·È Û˘Ó.

Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi˜. ∏ ·Ó¿Ù˘Í‹ ÙÔ˘ Â›Ó·È Â›Û˘ Ê˘ÛÈÔÏÔÁÈ΋.

™˘˙‹ÙËÛË ∏ Û˘¯ÓfiÙËÙ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÂÏÏ›ÌÌ·ÙÔ˜ ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ 22q11 ˘ÔÏÔÁ›˙ÂÙ·È Û 1:40006000 ÁÂÓÓ‹ÛÂȘ ˙ˆÓÙ·ÓÒÓ ÓÂÔÁÓÒÓ (4,9). £ÂˆÚÂ›Ù·È Ë ‰Â‡ÙÂÚË ÈÔ Û˘¯Ó‹ ¯ÚˆÌÔÛˆÌÈ΋ ·ÓˆÌ·Ï›· ÌÂÙ¿ ÙËÓ ÙÚÈۈ̛· 21 (4,7,10). OÈ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ (ÂÚ›Ô˘ 90%) ÚÔ·ÙÔ˘Ó de novo, ÂÓÒ ÔÛÔÛÙfi ÂÚ›Ô˘ 10% ÎÏËÚÔÓÔÌÂ›Ù·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú· (4). ∏ Û˘¯ÓfiÙËÙ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Û ÓÂÔÁÓ¿ ÌÂ Û˘ÁÁÂÓ‹ ηډÈÔ¿ıÂÈ· ÙÔ˘ ÛÙÂϯȷ›Ô˘ ÎÒÓÔ˘ (conotruncal) Â›Ó·È 48% (11). ™Â ÓÂÔÁÓ¿ Ì ÙÂÙÚ·ÏÔÁ›· Fallot Â›Ó·È 13% (8). ∏ ˘Ô„›· ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù›ıÂÙ·È ·fi ¤Ó· Ê¿ÛÌ· ¢ÚËÌ¿ÙˆÓ, Ô˘ ÌÔÚ› Ó· ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÔÚÈṲ̂ÓÔ˘˜ Û˘Ó‰˘·ÛÌÔ‡˜ ÙˆÓ ·ÎfiÏÔ˘ıˆÓ: Û˘ÁÁÂÓ‹˜ ηډÈÔ¿ıÂÈ· Ù‡Ô˘ conotruncal ·ÓˆÌ·Ï›·˜, ·ÓˆÌ·Ï›Â˜ Ù˘ ˘ÂÚÒ·˜, ˘·Û‚ÂÛÙÈ·ÈÌ›·, ·ÓÔÛÔ·ÓÂ¿ÚÎÂÈ·, Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜ Î·È ÌÂÚÈΤ˜ ÊÔÚ¤˜ ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ÌÔÚÊÔÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÚÔÛÒÔ˘ (4,6). ∏ ‚·Ú‡ÙËÙ· ̤۷ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÔÈΛÏÏÂÈ ·fi ·Û˘Ìو̷ÙÈ΋ ÂÌÊ¿ÓÈÛË ÛÙÔ˘˜ ÁÔÓ›˜ ̤¯ÚÈ ÛÔ‚·Ú¤˜ ÂΉËÏÒÛÂȘ Î·È ‰˘ÛÌÔÚʛ˜ ÛÙ· ·È‰È¿ (3,4). ∏ ÎÏËÚÔÓÔÌÈ΋ ÌÂÙ·‚›‚·ÛË Â›Ó·È ÈÔ Û˘¯Ó‹ ·fi ÙË ÌËÙ¤Ú· Î·È Á›ÓÂÙ·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ¯·Ú·ÎÙ‹Ú·. ™˘Ó‹ıˆ˜, ÔÈ ÌËÙ¤Ú˜ ÂÌÊ·Ó›˙Ô˘Ó ÂÏ¿ÛÛÔÓ˜ ÌÔÚÊÔÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÚÔÛÒÔ˘, ˘ÂÚˆ˚ÔÊ·Ú˘ÁÁÈ΋ ·ÓÂ¿ÚÎÂÈ· Î·È ‰È·Ù·Ú·¯¤˜ ÛÙËÓ „˘¯ÔÎÔÈÓˆÓÈ΋ ÙÔ˘˜ Û˘ÌÂÚÈÊÔÚ¿ Ô˘ ÔÈΛÏÏÔ˘Ó ·fi ‹Ș Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜ ̤¯ÚÈ ÛÔ‚·Ú‹ ·ÓÙÈÎÔÈÓˆÓÈ΋ ÚÔÛˆÈÎfiÙËÙ· Î·È ‚·ÚȤ˜ „˘¯È·ÙÚÈΤ˜ ηٷÛÙ¿ÛÂȘ. ∆Ô Ì¤ÁÂıÔ˜ Ù˘ ¤ÏÏÂȄ˘ ‰ÂÓ ıˆÚÂ›Ù·È ˘‡ı˘ÓÔ ÁÈ· ÙË Ê·ÈÓÔÙ˘È΋ ÔÈÎÈÏÔÌÔÚÊ›· (12). ¶Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ Î·È ¿ÏÏÔÈ Â͈ÁÂÓ›˜, ÌË ÁÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ·›˙Ô˘Ó ÚfiÏÔ ÛÙËÓ ÔÈÎÈÏÔÌÔÚÊ›· ÙˆÓ ÂΉËÏÒÛÂˆÓ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ (2). ™ÙËÓ ·ÚÔ‡Û· ÂÚ›ÙˆÛË, Ë ÌÂÙ·‚›‚·ÛË Ù˘ ¯ÚˆÌÔÛˆÌÈ΋˜ ·ÓˆÌ·Ï›·˜ ¤ÁÈÓ ·fi ÙË ÌËÙ¤Ú·, Ë ÔÔ›· ·ÚÔ˘Û›·˙ ¤ÓÚÈÓË ÔÌÈÏ›·, Ì·ıËÛȷο ÚÔ‚Ï‹Ì·Ù· Î·È Â˘¿ıÂÈ· Û ÏÔÈÌÒÍÂȘ. ∆Ô ¤Ó· ·È‰› ÂÌÊ¿ÓÈÛ ÙËÓ ÎÏ·ÛÈ΋ ÙÚÈ¿‰· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ DiGeorge Ì ·Ï·Û›· ÙÔ˘ ı‡ÌÔ˘ ·‰¤Ó·, ÛÔ‚·Ú‹ conotruncal ηډȷ΋ ·ÓˆÌ·Ï›· (·ÚÙËÚÈ·Îfi ÎÔÚÌfi Ù‡Ô˘ ππ) Î·È ‚·¶·È‰È·ÙÚÈ΋ 2005;68:372-375

ÚÈ¿ ·ÓÔÛÔ·ÓÂ¿ÚÎÂÈ· Î·È ·‚›ˆÛ ÌÂÙ¿ ·fi Û‹„Ë, ÂÓÒ ÙÔ ¿ÏÏÔ ·È‰› ›¯Â ÌfiÓÔ ÌÈÎÚÔÁÓ·ı›· Î·È ·ÚÔ‰È΋, ‹È· ˘·Û‚ÂÛÙÈ·ÈÌ›·.

OÈ Î·Ú‰È·Î¤˜ ·ÓˆÌ·Ï›Â˜ ··ÓÙÔ‡Ó ÛÙÔ 74% ÙˆÓ ·Û¯fiÓÙˆÓ (12) Î·È Â›Ó·È Ù‡Ô˘ conotruncal, ÌÂ Û˘¯ÓfiÙÂÚË ÙËÓ ÙÂÙÚ·ÏÔÁ›· Fallot (4). ¶ÔÛÔÛÙfi 69% ÙˆÓ ·Û¯fiÓÙˆÓ ¤¯ÂÈ ·ÓˆÌ·Ï›Â˜ Ù˘ ˘ÂÚÒ·˜, ΢ڛˆ˜ ˘ÂÚˆÈÔÊ·Ú˘ÁÁÈ΋ ·ÓÂ¿ÚÎÂÈ· Ô˘ ÂΉËÏÒÓÂÙ·È ÌfiÓÔ Ì ¤ÓÚÈÓË ÔÌÈÏ›· (4). ∆Ô Ê¿ÛÌ· Ù˘ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ ·Ú·ı˘ÚÂÔÂȉÒÓ ÛÙÔ 22q11 Û‡Ó‰ÚÔÌÔ ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ‰ÈÂÚ¢ÓËı›. Œ¯Ô˘Ó ·Ó·ÊÂÚı›: ·) ·Ô˘Û›· ·Ú·ı˘ÚÂÔÂȉÈÎÔ‡ ÈÛÙÔ‡ Ì ˘·Û‚ÂÛÙÈ·ÈÌ›·, ‚) ˘·Û‚ÂÛÙÈ·ÈÌÈÎfi˜ ˘Ô·Ú·ı˘ÚÂÔÂȉÈÛÌfi˜ Ô˘ ÂÌÊ·Ó›˙ÂÙ·È ÁÈ· ÚÒÙË ÊÔÚ¿ Û ÂÓ‹ÏÈΘ, Á) ˘·Û‚ÂÛÙÈ·ÈÌÈÎfi˜ ˘Ô·Ú·ı˘ÚÂÔÂȉÈÛÌfi˜ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· Ô˘ ‚ÂÏÙÈÒÓÂÙ·È ·˘ÙfiÌ·Ù· ÛÙËÓ ·È‰È΋ ËÏÈΛ· Î·È Â·Ó¤Ú¯ÂÙ·È ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ Î·È ‰) ÓÔÚÌ·Û‚ÂÛÙÈ·ÈÌ›· Ô˘ ÂÍÂÏ›ÛÛÂÙ·È Û ˘·Û‚ÂÛÙÈ·ÈÌÈÎfi ·Ú·ı˘ÚÂÔÂȉÈÛÌfi (8,12). ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ¤ÏÏÂÈÌÌ· 22q11 Î·È ÓÔÚÌ·Û‚ÂÛÙÈ·ÈÌ›· Û˘ÛÙ‹ÓÂÙ·È Ì·ÎÚÔ¯ÚfiÓÈ· ·Ú·ÎÔÏÔ‡ıËÛË, ÏfiÁˆ Ù˘ Ê˘ÛÈ΋˜ ÂͤÏÈ͢ Û ˘·Û‚ÂÛÙÈ·ÈÌÈÎfi ˘Ô·Ú·ı˘ÚÂÔÂȉÈÛÌfi (12). ∏ ·ÓÔÛÔ·ÓÂ¿ÚÎÂÈ· ÂÚÈÏ·Ì‚¿ÓÂÈ ÂÏ¿ÙÙˆÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙˆÓ ÚÔÂÚ¯ÔÌ¤ÓˆÓ ·fi ÙÔÓ ı‡ÌÔ ·‰¤Ó·, ηıÒ˜ Î·È ÏÂÈÙÔ˘ÚÁÈΤ˜ ·ÓÂ¿ÚÎÂȘ ÙˆÓ ∆ Î·È µ ΢ÙÙ¿ÚˆÓ (4). ∆Ô ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÚÔÛˆÂ›Ô ÂÚÈÏ·Ì‚¿ÓÂÈ ÌÈÎÚfi ÛÙfiÌ·, ÔȉËÌ·ÙÒ‰Ë Ì¿ÙÈ·, ÂÈ¤‰ˆÛË Ù˘ Ú¿¯Ë˜ Ù˘ ̇Ù˘, ˘ÂÚˆÈÔÛ¯ÈÛÙ›·, ÌÈÎÚÔÁÓ·ı›·, ˘ÂÚÙÂÏÔÚÈÛÌfi, ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ÙÂÚ˘Á›Ô˘ ÙˆÓ ·˘ÙÈÒÓ Î·È ÌÈÎÚ¤˜ ‚ÏÂÊ·ÚÈΤ˜ Û¯ÈṲ̂˜ (4,6,9). ™¯Â‰fiÓ fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ Ì ۇӉÚÔÌÔ ÂÏÏ›ÌÌ·ÙÔ˜ 22q11 ÂΉËÏÒÓÔ˘Ó ‹È·, ·ÏÏ¿ Ù˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο ÚÔÛÒÔ˘ (13), Ô˘ ·fi ÌfiÓ· ÙÔ˘˜ ‰ÂÓ Â›Ó·È Èηӿ Ó· ηÙ¢ı‡ÓÔ˘Ó ÛÙËÓ ˘ÔΛÌÂÓË ‰È¿ÁÓˆÛË (4). OÈ ÂÚÈÛÛfiÙÂÚÔÈ ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ¤ÏÏÂÈÌÌ· 22q11 ¤¯Ô˘Ó ‹ÈÔ˘ ÚÔ˜ ̤ÙÚÈÔ˘ ‚·ıÌÔ‡ ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË (4,8). ∂Ӊ›ÍÂȘ ÁÈ· ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ·ÔÙÂÏÔ‡Ó Ë ‡·ÚÍË ÂÓfi˜ ÁÔÓ¤· Ì ·ÒÏÂÈ· 22q11, Ë ÚÔËÁËı›۷ Á¤ÓÓËÛË ÂÓfi˜ ·È‰ÈÔ‡ Ì ¤ÏÏÂÈÌÌ· 22q11, ηıÒ˜ Î·È Ù· ¤Ì‚Ú˘· ÛÙ· ÔÔ›· Ù›ıÂÙ·È Ë ‰È¿ÁÓˆÛË Î·Ú‰ÈÔ¿ıÂÈ·˜ ÙÔ˘ ÛÙÂϯȷ›Ô˘ ÎÒÓÔ˘ (conotruncal) Î·È Û˘Ó‰ÚfiÌÔ˘ conotruncal abnormal face (8). ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ¤¯Ô˘Ó ·Ó·ÊÂÚı› Ï›Á˜ ÂÚÈÙÒÛÂȘ ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ 22q11 (3,7,9). ¶ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË

Pediatr Sept-Oct 05

17-10-05

16:52

™ÂÏ›‰·375

375

™‡Ó‰ÚÔÌÔ ÌÈÎÚÔÂÏÏ›ÌÌ·ÙÔ˜ 22q11.2

¤ÁÈÓ ÛÙÔ ‰Â‡ÙÂÚÔ ¿Û¯ÔÓ ·È‰› Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È, ·ÊÔ‡ ÚÒÙ· ¤ÁÈÓ ‰È¿ÁÓˆÛË ÛÙÔ ÚÒÙÔ ·È‰›. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ÂÈÙÚ¤ÂÈ ÙË ÛˆÛÙ‹ ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï‹. ∂›Ó·È ¯Ú‹ÛÈÌË ÛÙË Ï‹„Ë ·ÔÊ¿ÛÂˆÓ Î·È ÚÔÂÙÔÈÌ·ÛÈÒÓ ÁÈ· ÙËÓ ÂÂÚ¯fiÌÂÓË Á¤ÓÓ·, ÙËÓ ÂÚÈÁÂÓÓËÙÈ΋ ÊÚÔÓÙ›‰·, ÙÔÓ Û¯Â‰È·ÛÌfi Ù˘ ¿ÌÂÛ˘ È·ÙÚÈ΋˜ ÊÚÔÓÙ›‰·˜ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô Î·È ÙËÓ ÚÔÂÙÔÈÌ·Û›· ÁÈ· Â›ÁÔ˘Û· ηÈÛ·ÚÈ΋ ÙÔÌ‹ Û ÂÚ›ÙˆÛË ÂÌ‚Ú˘˚ÎÔ‡ ÛÙÚ˜ (8). ÃÚËÛÈ̇ÂÈ, Â›Û˘, ÁÈ· ÙÔÓ ÂÓÙÔÈÛÌfi ¿ÏÏˆÓ ·Û¯fiÓÙˆÓ ÌÂÏÒÓ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. ŸÌˆ˜, Ë ÚÔÁÂÓÓËÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋ ·Ú·Ì¤ÓÂÈ ‰‡ÛÎÔÏË ÏfiÁˆ Ù˘ ÎÏÈÓÈ΋˜ ÔÈÎÈÏÔÌÔÚÊ›·˜ Ô˘ ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÛÙÔ Û‡Ó‰ÚÔÌÔ, ηıfiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ Û˘Û¯¤ÙÈÛË Ê·ÈÓfiÙ˘Ô˘-ÁÔÓfiÙ˘Ô˘ Î·È Ù· Û˘ÌÙÒÌ·Ù· ÔÈΛÏÏÔ˘Ó ·Ó¿ÌÂÛ· ÛÙÔ Û‡Ó‰ÚÔÌÔ DiGeorge, ÙË ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË Î·È ÙȘ ‹Ș Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ÂÈÙÚ¤ÂÈ Î·È ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi ÙÔ˘ Û¯ÂÙÈÎÔ‡ ÎÈÓ‰‡ÓÔ˘ Â·ÓÂÌÊ¿ÓÈÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ™Â ÂÚÈÙÒÛÂȘ de novo ÂÌÊ¿ÓÈÛ˘ Û ¤Ó· ·È‰›, Ô Î›Ó‰˘ÓÔ˜ Â·ÓÂÌÊ¿ÓÈÛ˘ Û ÂfiÌÂÓÔ ·È‰› Â›Ó·È 1-3%. ¶Ú¤ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ ·ÎfiÌ· Î·È fiÙ·Ó Î·Ó¤Ó·˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ ¿Û¯ÔÓÙÔ˜ ·È‰ÈÔ‡ ‰ÂÓ Ê¤ÚÂÈ ÙÔ ¤ÏÏÂÈÌÌ·, ˘¿Ú¯ÂÈ ÌÈÎÚfi˜ ΛӉ˘ÓÔ˜ Â·ÓÂÌÊ¿ÓÈÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Û ÂfiÌÂÓÔ ·È‰›, ÂÂȉ‹ ‰ÂÓ ÌÔÚ› Ó· ·ÔÎÏÂÈÛÙ› Ë ÂÚ›ÙˆÛË ÌˆÛ·˚ÎÈÛÌÔ‡ ÙˆÓ ÁÔÓ¿‰ˆÓ (3,8,14). ∏ ÚÒÈÌË ‰È¿ÁÓˆÛË Â›Ó·È ÛËÌ·ÓÙÈ΋ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ·ÛıÂÓÔ‡˜. øÛÙfiÛÔ, Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÔÚÈÛÙÔ‡Ó ÎÚÈÙ‹ÚÈ· ÁÈ· ÙÔ fiÙ ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· ¤ÏÏÂÈÌÌ· 22q11. FISH ·Ó¿Ï˘ÛË ÁÈ· ÙÔ Û‡Ó‰ÚÔÌÔ Û˘ÛÙ‹ÓÂÙ·È Û fiÏ· Ù· ·È‰È¿ Ô˘ ¤¯Ô˘Ó ηډÈÔ¿ıÂÈ· Ù‡Ô˘ ‰È·ÎÔ‹˜ ·ÔÚÙÈÎÔ‡ ÙfiÍÔ˘, ÙÂÙÚ·ÏÔÁ›·˜ Fallot Î·È ·ÚÙËÚÈ·ÎÔ‡ ÎÔÚÌÔ‡ Ù‡Ô˘ II, ηıÒ˜ Î·È Û ‚Ú¤ÊË Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ·ÓÂÍ‹ÁËÙË, Â›ÌÔÓË ˘·Û‚ÂÛÙÈ·ÈÌ›· ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÂÏ·Ùو̤ÓÔ ·ÚÈıÌfi ∆-΢ÙÙ¿ÚˆÓ Î·È ‰˘ÛÌÔÚÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο. ŒÓ‰ÂÈÍË ÁÈ· FISH ·Ó¿Ï˘ÛË ·ÔÙÂÏ› Î·È Ë ·ÚÔ˘Û›· ¤ÓÚÈÓ˘ ÔÌÈÏ›·˜ Û ·È‰È¿ Ô˘ ·Ú¯›˙Ô˘Ó Ó· Û˘ÏÏ·‚›˙Ô˘Ó ¯ˆÚ›˜ Ó· Û˘Ó˘¿Ú¯Ô˘Ó ¿ÏϘ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ (4,10), ηıÒ˜ Î·È Û ·È‰È¿ Ì ÌË ÏÂÎÙÈΤ˜ (non-verbal) Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜ (4,10).

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7. Davidson A, Khandelwal M, Punnett HH. Prenatal diagnosis of the 22q11 deletion syndrome. Prenat Diagn 1997;17:380-383.

8. Oh DC, Min JY, Lee MH, Kim YM, Park SY, Won HS et al. Prenatal diagnosis of tetralogy of Fallot associated with chromosome 22q11 deletion. J Korean Med Sci 2002;17:125-128.

9. Driscoll DA, Salvin J, Sellinger B, Budarf ML, McDonald-McGinn DM, Zackai EH et al. Prevalence of 22q11 microdeletions in DiGeorge and velocardiofacial syndromes: implications for genetic counselling and prenatal diagnosis. J Med Genet 1993;30:813-817.

10. Goodship J, Cross I, LiLing J, Wren C. A population study of chromosome 22q11 deletions in infancy.

Arch Dis Child 1998;79:348-351.

11. Iserin L, de Lonlay P, Viot G, Sidi D, Kachaner J, Munnich A et al. Prevalence of the microdeletion 22q11 in newborn infants with congenital conotruncal cardiac anomalies. Eur J Pediatr 1998; 157:881-884.

12. Cuneo BF, Driscoll DA, Gidding SS, Langman CB. Evolution of latent hypoparathyroidism in familial 22q11 deletion syndrome. Am J Med Genet 1997; 69:50-55.

13. Digilio MC, Marino B, Giannotti A, Dallapiccola B. Search for 22q11 deletion in non-syndromic conotruncal cardiac defects. Eur J Pediatr 1996;155: 619-620.

14. Sitch FL, James RS, Cockwell AE, Hatchwell E. Gonadal mosaicism for a submicroscopic deletion of chromosome region 22q11. Eur J Hum Genet 1996;4:59.

¶·È‰È·ÙÚÈ΋ 2005;68:372-375

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CASE REPORT

22q11.2 micro-deletion syndrome in a family with variation in the phenotype.

Prenatal diagnosis 1 Neonatology Department ¡∏π, Hippokration General Hospital, Thessaloniki 2 Neonatology Department, University Hospital of Larissa 3 Molecular Biology and Cytogenetics Center, “Leto” Maternity Clinic, Athens Correspondence: Alexandros Andreou 15 Heimonidou Str. 551 33, Kalamaria, Thessaloniki ∂-mail: aalex@the.forthnet.gr Date of submission: 27-09-2004 Date of approval: 29-03-2005

Paediatriki 2005;68:376

A. Andreou1, V. Alexandropoulou - Tsikrika2, A. Sakellaropoulou1, M. Goudesidou2, L. Florentin - Arar3

Abstract A family is described in which chromosome deletion 22q11.2 was identified in three of its members, with a great heterogeneity in the phenotype. The mother had velo-pharyngeal insufficiency with nasal voice, the third child had DiGeorge syndrome with severe congenital cardiopathy, hypocalcaemia, and aplasia of the thymus gland and died of severe sepsis, and the fourth child had micrognathia and transient hypocalcaemia. The diagnosis was established first in the third child and subsequently in the mother. On the fourth child the diagnosis was made prenatally, after amniocentesis. In all cases the diagnosis was made with the FISH technique.

Key words 22q11 deletion syndrome, familial appearance, prenatal diagnosis, FISH technique.

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∏ Â›‰Ú·ÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ô˘ ¯ÔÚËÁÔ‡ÓÙ·È Î·Ù¿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô ÛÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ∂. ªÔ‡˙·, ∫. ¶··Á·ÚÔ˘Ê¿Ï˘

¶ÂÚ›ÏË„Ë ∆· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ¯ÔÚËÁÔ‡ÓÙ·È Ôχ Û˘¯Ó¿ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô ÁÈ· ÙËÓ ÚfiÏË„Ë Î·È ÙË ıÂÚ·›· Ù˘ ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ÓfiÛÔ˘ (‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋ ‰˘ÛÏ·Û›·) Ô˘ ·Ó·Ù‡ÛÛÂÙ·È Û ÚfiˆÚ· ÓÂÔÁÓ¿. ¶ÚfiÛÊ·Ù˜ ÌÂÙ·-·Ó·Ï‡ÛÂȘ Ù˘ ™˘ÓÂÚÁ·Û›·˜ Cochrane ‰Â›¯ÓÔ˘Ó fiÙÈ ÔÈ Î›Ó‰˘ÓÔÈ ·fi ÙË ıÂÚ·›· ÌÔÚ› Ó· ˘ÂÚ‚·›ÓÔ˘Ó Ù· ÔʤÏË, ÂȉÈο fiÙ·Ó Ë ıÂÚ·›· ·Ú¯›˙ÂÈ ÚÈÓ ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 96 ˆÚÒÓ. OÈ ÌÂÙ·-·Ó·Ï‡ÛÂȘ ·˘Ù¤˜ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ ÛÙËÓ ËÏÈΛ· ÙˆÓ 7-14 ËÌÂÚÒÓ Ê·›ÓÂÙ·È Ó· ¤¯ÂÈ ÙÔÓ Î·Ï‡ÙÂÚÔ ÏfiÁÔ ÔʤÏÔ˘˜-ÎÈÓ‰‡ÓÔ˘. ∂Âȉ‹, fï˜, Ù· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Ê·›ÓÂÙ·È fiÙÈ ¤¯Ô˘Ó Ó¢ÚÔÙÔÍÈ΋ ‰Ú¿ÛË ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ, ÔÈ ÓÂÔÁÓÔÏfiÁÔÈ Ú¤ÂÈ Ó· Û˘Ó¯›ÛÔ˘Ó ÙȘ ÂÚ¢ÓËÙÈΤ˜ ÙÔ˘˜ ÚÔÛ¿ıÂȘ ÁÈ· ÙËÓ ·Ó·Î¿Ï˘„Ë ÂÓ·ÏÏ·ÎÙÈÎÒÓ ıÂÚ·ÂÈÒÓ Ù˘ ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÔ¿ıÂÈ·˜ ÙˆÓ ÚfiˆÚˆÓ ÓÂÔÁÓÒÓ. ∂Ó Ùˆ ÌÂٷ͇, Ë ∂˘Úˆ·˚΋ ∂Ù·ÈÚ›· ¶ÂÚÈÁÂÓÓËÙÈ΋˜ π·ÙÚÈ΋˜, Ë ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ Î·È Ë ∫·Ó·‰È΋ ¶·È‰È·ÙÚÈ΋ ∂Ù·ÈÚ›·, ·ÊÔ‡ ÚÔÛ¿ıËÛ·Ó Ó· ‰ÈÂÚ¢ӋÛÔ˘Ó Ì›· Û˘Áί˘Ì¤ÓË Î·Ù¿ÛÙ·ÛË, Âͤ‰ˆÛ·Ó Ô‰ËÁ›Â˜, ÔÈ Ôԛ˜ ·Ú¿ ÙÔ˘˜ ÂÚÈÔÚÈÛÌÔ‡˜ ÙÔ˘˜, ·Ú¤¯Ô˘Ó ÙȘ ÈÔ Ú·ÏÈÛÙÈΤ˜ Î·È Î·ÏÔ˙˘ÁÈṲ̂Ó˜ Û˘Ì‚Ô˘Ï¤˜ ÁÈ· ÙÔÓ ÎÏÈÓÈÎfi ÓÂÔÁÓÔÏfiÁÔ.

µ’ ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ ¡ÔÛËÏ›·˜ ¡ÂÔÁÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: ∂ϤÓË ªÔ‡˙· µ’ ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ ¡ÔÛËÏ›·˜ ¡ÂÔÁÓÒÓ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” ∆.∫. 115 27, ∞ı‹Ó· E-mail: bnicu@paidon-agiasofia.gr ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 21-06-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 17-06-2005

§¤ÍÂȘ ÎÏÂȉȿ ∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, ÓÂÔÁÓfi, ÂÁΤʷÏÔ˜.

∂ÈÛ·ÁˆÁ‹ ∞Ó Î·È Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ì ÙË ¯Ú‹ÛË ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·, Ë ÂÈ‚›ˆÛË ÙˆÓ ÚfiˆÚˆÓ ÓÂÔÁÓÒÓ Ì ۇӉÚÔÌÔ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜ ¤¯ÂÈ ·˘ÍËı›, Ë ¯ÚfiÓÈ· ÓfiÛÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ ·Ú·Ì¤ÓÂÈ ¤Ó· ÛÔ‚·Úfi Úfi‚ÏËÌ· ÛÙȘ ÌÔÓ¿‰Â˜ ÂÓÙ·ÙÈ΋˜ ÓÔÛËÏ›·˜ ÓÂÔÁÓÒÓ. ∏ ¯ÚfiÓÈ· ÓfiÛÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ ‹ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋ ‰˘ÛÏ·Û›· Â›Ó·È Ì›· ÛËÌ·ÓÙÈ΋ ·ÈÙ›· ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÛÈÌfiÙËÙ·˜ ÙÔ˘ ÚfiˆÚÔ˘ ÓÂÔÁÓÔ‡. ∂ÈϤÔÓ, Â›Ó·È Ì›· ·fi ÙȘ ÈÔ Û˘¯Ó¤˜ ·Èٛ˜ Ì·ÎÚÔÚfiıÂÛÌˆÓ ‰˘ÛÌÂÓÒÓ Û˘ÓÂÂÈÒÓ ÛÙ· Ôχ ÌÈÎÚÔ‡ ‚¿ÚÔ˘˜ Á¤ÓÓËÛ˘ ÓÂÔÁÓ¿, Ô˘ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ú·ÙÂٷ̤ÓË ·Ú·ÌÔÓ‹ ÙÔ˘˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô, ÌÂ Û˘¯Ó¤˜ Â·ÓÂÈÛ·ÁˆÁ¤˜ Î·È ‰˘ÛÌÂÓ›˜ ÂÈÙÒÛÂȘ ÛÙË ÌÂÙ¤ÂÈÙ· ·Ó¿Ù˘ÍË Î·È „˘¯ÔÎÈÓËÙÈ΋ ÙÔ˘˜ ÂͤÏÈÍË. ø˜ ¯ÚfiÓÈ· ÓfiÛÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ ÔÚ›˙ÂÙ·È Ë ÂÍ¿ÚÙËÛË ·fi ÙÔ Ô͢ÁfiÓÔ ÛÙËÓ ËÏÈΛ· ÙˆÓ 36 ‚‰ÔÌ¿‰ˆÓ ‹ ÛÙȘ 28 Ë̤Ú˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, Û˘Á¯ÚfiÓˆ˜ Ì ÂÈ̤ÓÔÓÙ· ·Ó·Ó¢ÛÙÈο ÚÔ‚Ï‹Ì·Ù· Î·È Û˘Ì‚·Ù¤˜ ·ÓˆÌ·Ï›Â˜ ÛÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜.

∏ Û˘¯ÓfiÙËÙ· Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ ·Ó¿ÌÂÛ· ÛÙ· ÂÈ˙ÒÓÙ· ÓÂÔÁÓ¿ Ôχ ¯·ÌËÏÔ‡ ‚¿ÚÔ˘˜ Á¤ÓÓËÛ˘ (<1500 g) Â›Ó·È ÂÚ›Ô˘ 23% (1). À¿Ú¯Ô˘Ó ÔÏϤ˜ ÂӉ›ÍÂȘ fiÙÈ Ë ¯ÚfiÓÈ· ÓfiÛÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ Â›Ó·È ÙÔ ÙÂÏÈÎfi ·ÔÙ¤ÏÂÛÌ· Ì›·˜ Û˘Ó¯È˙fiÌÂÓ˘ ÊÏÂÁÌÔÓÒ‰Ô˘˜ ‰È·‰Èηۛ·˜ ÛÙÔÓ ÓÂÔÁÓÈÎfi Ó‡ÌÔÓ· Ô˘ ¤¯ÂÈ ˘ÔÛÙ› ‚Ï¿‚Ë ·fi Ì›· ‹ ÂÚÈÛÛfiÙÂÚ˜ ·Èٛ˜. ∆· ÓÂÔÁÓ¿ Ô˘ ·Ó·ÚÚÒÓÔ˘Ó ·fi ÙÔ Û‡Ó‰ÚÔÌÔ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜, Ì ·Ú·ÙÂٷ̤ÓË ‰È·ÛˆÏ‹ÓˆÛË Î·È ¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘, ¤¯Ô˘Ó ·˘ÍË̤ӷ Â›‰· ÚÔÊÏÂÁÌÔÓˆ‰ÒÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ ÛÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ÙÔ˘˜ ¤ÎÏ˘Ì· Î·È Ê·›ÓÂÙ·È fiÙÈ ·˘Ù‹ Ë ÊÏÂÁÌÔÓ҉˘ ·ÓÙ›‰Ú·ÛË ·›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ·Ó¿Ù˘ÍË Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ. ™Â ¿ÏÏ· ÚfiˆÚ· ÓÂÔÁÓ¿, Ô˘ ‰ÂÓ ¤¯Ô˘Ó ÛÔ‚·Úfi ·Ó·Ó¢ÛÙÈÎfi Úfi‚ÏËÌ·, ˘¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ ¤ÎıÂÛ˘ Û ÚÔÊÏÂÁÌÔÓÒ‰ÂȘ ΢ÙÙ·ÚÔΛÓ˜ ÂÓ‰ÔÌ‹ÙÚÈ·, ÚÔÂÚ¯fiÌÂÓ˜ ·fi ¯ÔÚÈÔ·ÌÓÈÔÓ›Ùȉ·. ™Â Ôχ ÚfiˆÚ· ÓÂÔÁÓ¿, ·ÎfiÌË Î·È Ë ·Ó¿ÁÎË ·Ó¿Ó˄˘ ÛÙË Á¤ÓÓËÛË ÌÔÚ› Ó· ÂÏ¢ıÂÚÒÛÂÈ Î˘ÙÙ·ÚÔΛÓ˜ ·fi ÙÔÓ Ó‡ÌÔÓ·, ˘ÚÔ‰ÔÙÒÓÙ·˜ Ì›· ¶·È‰È·ÙÚÈ΋ 2005;68:377-384

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∂. ªÔ‡˙· Î·È Û˘Ó.

ÊÏÂÁÌÔÓÒ‰Ë ·¿ÓÙËÛË Ô˘ Ô‰ËÁ› Û ¯ÚfiÓÈ· ÓfiÛÔ ÙˆÓ Ó¢ÌfiÓˆÓ. ∏ ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ - Î·È Î˘Ú›ˆ˜ Ë ‰ÂÍ·ÌÂı·˙fiÓË - ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› ¢ڤˆ˜ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô ÁÈ· ÙËÓ ÚfiÏË„Ë Î·È ÙË ıÂÚ·›· Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ, ÂÍ·ÈÙ›·˜ Ù˘ ÈÛ¯˘Ú‹˜ ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ô˘˜ ‰Ú¿Û˘ Ù˘. ¶Èı·ÓÔ› Ì˯·ÓÈÛÌÔ› Ù˘ ‰Ú¿Û˘ ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙÔÓ Ó‡ÌÔÓ· ÙÔ˘ ÓÂÔÁÓÔ‡ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙËÓ ·‡ÍËÛË Ù˘ Û‡ÓıÂÛ˘ ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·, ÙËÓ ·‡ÍËÛË Ù˘ ·ÓÙÈÔÍÂȉˆÙÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜, ÙËÓ ·Ó·ÛÙÔÏ‹ Ù˘ Û‡ÓıÂÛ˘ ÚÔÛÙ·ÁÏ·Ó‰ÈÓÒÓ Î·È Ï¢ÎÔÙÚÈÂÓÒÓ, ÙË Ì›ˆÛË ÙˆÓ ÊÏÂÁÌÔÓˆ‰ÒÓ Ê·ÈÓÔÌ¤ÓˆÓ Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi ÙȘ ΢ÙÙ·ÚÔΛÓ˜ ÛÙÔÓ Ó‡ÌÔÓ·, ÙË Ì›ˆÛË ÙÔ˘ ‡‰·ÙÔ˜ ÛÙÔÓ Ó‡ÌÔÓ· Î·È ÙË ÌÂȈ̤ÓË ‰È·‚·ÙfiÙËÙ· ÙÔ˘ Ó‡ÌÔÓ·. ∂›Û˘, ¤¯ÂÈ ‰Âȯı› fiÙÈ Ù· ÓÂÔÁÓ¿ Ô˘ ·Ó·Ù‡ÛÛÔ˘Ó ¯ÚfiÓÈ· ÓfiÛÔ ÙˆÓ Ó¢ÌfiÓˆÓ, ¤¯Ô˘Ó ¯·ÌËÏ¿ Â›‰· ÎÔÚÙÈ˙fiÏ˘ ÌÂÙ¿ ·fi ‰È¤ÁÂÚÛË Ì ACTH ηٿ ÙËÓ ÚÒÙË Â‚‰ÔÌ¿‰· ˙ˆ‹˜ (2).

OÈ ·Ú¯ÈΤ˜ ÌÂϤÙ˜ Ì ‰ÂÍ·ÌÂı·˙fiÓË, ¤Ó· ÈÛ¯˘Úfi Ì·ÎÚ¿˜ ‰È¿ÚÎÂÈ·˜ ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ¤˜, Û ÚfiˆÚ· ÓÂÔÁÓ¿ Ì ¯ÚfiÓÈ· ÓfiÛÔ ÙˆÓ Ó¢ÌfiÓˆÓ, ¤‰ÂÈÍ·Ó ¿ÌÂÛË ‚ÂÏÙ›ˆÛË Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ Ó¢ÌfiÓˆÓ Î·È Ù˘ ·ÓÙ·ÏÏ·Á‹˜ ÙˆÓ ·ÂÚ›ˆÓ Î·È Ì›ˆÛË Ù˘ ‰È¿ÚÎÂÈ·˜ ÂÍ¿ÚÙËÛ˘ ·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi. ªÂ ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ Î·È Ì ÙËÓ ·˘ÍË̤ÓË ÂÈ‚›ˆÛË ÙˆÓ ÂÍ·ÈÚÂÙÈο ÚfiˆÚˆÓ ÓÂÔÁÓÒÓ, ˘‹Ú¯Â Ì›· Ù¿ÛË ÁÈ· ÚÒÈÌË ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ Û ÓÂÔÁÓ¿ Ì fiÏÔ Î·È ÌÈÎÚfiÙÂÚÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ Î·È ‰È¿ÚÎÂÈ· ·ËÛ˘. ∆Ș ÙÂÏÂ˘Ù·›Â˜ ‰‡Ô ‰ÂηÂٛ˜, Ì›· ÏËıÒÚ· ÌÂÏÂÙÒÓ Î·Ù¤ÏËÍ ÛÙÔ Û˘Ì¤Ú·ÛÌ· Ù˘ ¢ÓÔ˚΋˜ ‰Ú¿Û˘ Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ (3,4). ∆ÂÏÂ˘Ù·›· ˘¿Ú¯ÂÈ ¤ÓÙÔÓË ·ÓËÛ˘¯›· ÏfiÁˆ ÙˆÓ ÎÏÈÓÈÎÒÓ ÌÂÏÂÙÒÓ Ô˘ ‰Â›¯ÓÔ˘Ó ¤Ó·Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ‚Ï·‚ÒÓ ·fi Ù· ÎÔÚÙÈÎÔÂȉ‹ Ô˘ ¯ÔÚËÁÔ‡ÓÙ·È Î·Ù¿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô, ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ Î·È ÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË. ∂Í·ÈÙ›·˜ ·˘ÙÒÓ ÙˆÓ ÌÂÏÂÙÒÓ, Ë ‰ÂÍ·ÌÂı·˙fiÓË ¤¯ÂÈ Á›ÓÂÈ ¤Ó· ·fi Ù· ÈÔ ·ÌÊÈÏÂÁfiÌÂÓ· Ê¿Ú̷η ÛÙȘ Û‡Á¯ÚÔÓ˜ ÌÔÓ¿‰Â˜ ÂÓÙ·ÙÈ΋˜ ÓÔÛËÏ›·˜ ÓÂÔÁÓÒÓ.

¶·È‰È·ÙÚÈ΋ 2005;68:377-384

ÂÚÈÂÏ¿Ì‚·Ó·Ó Ì›ˆÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜, ÙÔ˘ ‚¿ÚÔ˘˜ Î·È ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ DNA ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Û ÂÓ‹ÏÈη ÔÓÙ›ÎÈ·, ÛÙ· ÔÔ›· ›¯·Ó ¯ÔÚËÁËı› ÎÔÚÙÈÎÔÂȉ‹ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô (5). ∞ÚÁfiÙÂÚ·, ¿ÏϘ ÂÈÚ·Ì·ÙÈΤ˜ ÌÂϤÙ˜ Â›Û˘ Û ÔÓÙ›ÎÈ·, ¤‰ÂÈÍ·Ó ‚Ï¿‚Ë ÛÙË Ì˘ÂÏÈÓÔÔ›ËÛË, ‰È·›ÚÂÛË Î·È ‰È·ÊÔÚÔÔ›ËÛË ÙÔ˘ Ó¢ÚÈÎÔ‡ ΢ÙÙ¿ÚÔ˘ Î·È Î·ı˘ÛÙ¤ÚËÛË ÛÙË Û˘Ó·ÙÔÁ¤ÓÂÛË ÛÙÔÓ ÊÏÔÈfi, ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÂȉÒÓ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô (6,7). ¶ÚfiÛÊ·Ù·, Û ÂÈÚ·Ì·ÙÈΤ˜ ÌÂϤÙ˜, fiÔ˘ ÂÍÂÙ¿ÛÙËÎÂ Ë Ó¢ÚÔÙÔÍÈÎfiÙËÙ· ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ, ·Ú·ÙËÚ‹ıËΠfiÙÈ Ë ¤ÎıÂÛË ÓÂÔÁ¤ÓÓËÙˆÓ ÔÓÙÈÎÒÓ Û ˘„ËϤ˜ ‰fiÛÂȘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÚÔ‹Á·Á ÙÔÓ ı¿Ó·ÙÔ ÙÔ˘ Ó¢ÚÒÓ· Ì ÙÔÓ Ì˯·ÓÈÛÌfi Ù˘ ·fiÙˆÛ˘ (8).

∂Ӊ›ÍÂȘ ÛÙÔ ÓÂÔÁÓfi ªÂϤÙ˜ Û ÓÂÔÁÓ¿ ¤‰ÂÈÍ·Ó ÙËÓ Â›‰Ú·ÛË Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÛÙËÓ ·‡ÍËÛË Ù˘ ÂÚÈ̤ÙÚÔ˘ ÎÂÊ·Ï‹˜, ÛÙÔÓ fiÁÎÔ ÙÔ˘ ·Ó·Ù˘ÛÛfiÌÂÓÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È Û ‚Ï¿‚˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, fiˆ˜ Ê·›ÓÔÓÙ·È ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÂÁÎÂÊ¿ÏÔ˘. ∆ÚÂȘ ÌÂϤÙ˜ (9,10,21) ¤‰ÂÈÍ·Ó ÌÂȈ̤ÓË ·‡ÍËÛË Ù˘ ÂÚÈ̤ÙÚÔ˘ ÎÂÊ·Ï‹˜ ÌÂÙ¿ ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô. ∂ÈϤÔÓ, Û ‰‡Ô ¿ÏϘ ÌÂϤÙ˜ ·Ú·ÙËÚ‹ıËΠ̛· Ù¿ÛË ÚÔ˜ Û˘¯ÓfiÙÂÚ˜ ‚Ï¿‚˜ ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÂÁÎÂÊ¿ÏÔ˘ Û ÓÂÔÁÓ¿ Ô˘ ¤Ï·‚·Ó ‰ÂÍ·ÌÂı·˙fiÓË (11,12). ∆¤ÏÔ˜, Û ̛· ÚfiÛÊ·ÙË ÌÂϤÙË (13) Ì ÙË ¯Ú‹ÛË Ù˘ ÔÛÔÙÈ΋˜ ÔÁÎÔÌÂÙÚÈ΋˜ ÙÚÈۉȿÛÙ·Ù˘ Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ ÛÙËÓ ÙÂÏÂÈfiÌËÓË ˆÚÈÌfiÙËÙ·, ·Ú·ÙËÚ‹ıËΠÛËÌ·ÓÙÈ΋ Â›ÙˆÛË Ù˘ ÂÓ‰ÔÊϤ‚È·˜ ¯ÔÚ‹ÁËÛ˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ· ÛÙËÓ ·‡ÍËÛË ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, Ô˘ ·ÊÔÚÔ‡Û ΢ڛˆ˜ ÛÙË Ê·È¿ Ô˘Û›·. O fiÁÎÔ˜ Ù˘ ÊÏÔÈÒ‰Ô˘˜ Ê·È¿˜ Ô˘Û›·˜ ‹Ù·Ó ÌÂȈ̤ÓÔ˜ ηٿ 35% Î·È Ô ÔÏÈÎfi˜ fiÁÎÔ˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ‹Ù·Ó ÌÂȈ̤ÓÔ˜ ηٿ 30% Û ÚfiˆÚ· ÓÂÔÁÓ¿ ÛÙ· ÔÔ›· ›¯Â ¯ÔÚËÁËı› ‰ÂÍ·ÌÂı·˙fiÓË Û ۇÁÎÚÈÛË Ì ÚfiˆÚ· ÓÂÔÁÓ¿ Ô˘ ‰ÂÓ Â›¯·Ó Ï¿‚ÂÈ ‰ÂÍ·ÌÂı·˙fiÓË, ηıÒ˜ Î·È Ì ÙÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿ Ô˘ ÂÏ‹ÊıËÛ·Ó ˆ˜ Ì¿ÚÙ˘Ú˜.

¶ÂÈÚ·Ì·ÙÈο ‰Â‰Ô̤ӷ

∫ÏÈÓÈΤ˜ ÌÂϤÙ˜

ªÂϤÙ˜ Û ÂÈÚ·Ì·Ùfi˙ˆ· ¤‰ÂÈÍ·Ó Èı·Ó¤˜ ‰˘ÛÌÂÓ›˜ Ì·ÎÚÔÚfiıÂÛ̘ ÂȉڿÛÂȘ Ù˘ ¯ÔÚ‹ÁËÛ˘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ ·fi ÙÔ 1968. ∆· Â˘Ú‹Ì·Ù· ·˘Ù¿

∏ ÚÒÙË ÎÏÈÓÈ΋ ÌÂϤÙË ÛÙËÓ ÔÔ›· ‰È·ÈÛÙÒıËÎÂ Ë ‚Ï·ÙÈ΋ Â›‰Ú·ÛË Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË, ‰ËÌÔÛȇıËΠÌfiÏȘ ÙÔ 1998. ™ÙË ÌÂϤÙË ÙˆÓ Yeh ηÈ

Pediatr Sept-Oct 05

17-10-05

16:52

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379

∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Î·È ÓÂÔÁÓÈÎfi˜ ÂÁΤʷÏÔ˜

Û˘Ó (14), ¯ÔÚËÁ‹ıËΠ‰ÂÍ·ÌÂı·˙fiÓË ÛÙ· ÓÂÔÁÓ¿ ÓˆÚ›˜ (<12 ÒÚ˜ ˙ˆ‹˜) Î·È ÁÈ· 28 Ë̤Ú˜. ™ÙËÓ ËÏÈΛ· ÙˆÓ 2 ÂÙÒÓ ·ÚÔ˘Û›·Û·Ó ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÛÙË Û˘¯ÓfiÙËÙ· ÙˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ (ÔÛÔÛÙfi 40% ÛÙËÓ ÔÌ¿‰· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ¤Ó·ÓÙÈ 17% ÛÙÔ˘˜ Ì¿ÚÙ˘Ú˜). ¢‡Ô ¿ÏϘ ÌÂÁ¿Ï˜ Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ ÙˆÓ O’Shea Î·È Û˘Ó (15) Î·È Shinwell Î·È Û˘Ó (12) ¤‰ÂÈÍ·Ó ÙȘ ‰˘ÛÌÂÓ›˜ ÂÈÙÒÛÂȘ Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË. ™ÙË ÌÂϤÙË ÙˆÓ O’Shea Î·È Û˘Ó (15), ¯ÔÚËÁ‹ıËΠ‰ÂÍ·ÌÂı·˙fiÓË ÛÙ· ÓÂÔÁÓ¿ ·ÚÁ¿ (ÌÂÙ¿ ÙË 2Ë Â‚‰ÔÌ¿‰· ˙ˆ‹˜) Î·È ÁÈ· 42 Ë̤Ú˜. ™ÙËÓ ËÏÈΛ· ÙÔ˘ ÂÓfi˜ ¤ÙÔ˘˜ ·ÚÔ˘Û›·˙·Ó ÛËÌ·ÓÙÈο ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘ (ÔÛÔÛÙfi 25% ÛÙËÓ ÔÌ¿‰· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Û ·ÓÙ›ıÂÛË Ì 7% ÛÙÔ˘˜ Ì¿ÚÙ˘Ú˜). ∂›Û˘, ·ÚÔ˘Û›·˙·Ó ÛËÌ·ÓÙÈο ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ·ıÔÏÔÁÈ΋˜ Ó¢ÚÔÏÔÁÈ΋˜ ÂͤٷÛ˘ (ÔÛÔÛÙfi 45% ÛÙËÓ ÔÌ¿‰· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Û ·ÓÙ›ıÂÛË Ì 16% ÛÙÔ˘˜ Ì¿ÚÙ˘Ú˜). ™Â ·˘Ù‹ ÙË ÌÂϤÙË ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ‰È·ÊÔÚ¤˜ ÛÙËÓ „˘¯ÔÎÈÓËÙÈ΋ ÂͤÏÈÍË ÌÂٷ͇ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ. ™ÙË ÌÂϤÙË ÙˆÓ Shinwell Î·È Û˘Ó (12), Ë ‰ÂÍ·ÌÂı·˙fiÓË ¯ÔÚËÁ‹ıËΠÛÙ· ÓÂÔÁÓ¿ ÓˆÚ›˜ (<12 ÒÚ˜ ˙ˆ‹˜) Î·È ÁÈ· Ì›· ‚Ú·¯Â›· ÂÚ›Ô‰Ô 3 ËÌÂÚÒÓ. ™ÙËÓ ËÏÈΛ· ÙˆÓ 4ó ÂÙÒÓ, Ù· ·È‰È¿ ·˘Ù¿ ·ÚÔ˘Û›·˙·Ó ÛËÌ·ÓÙÈο ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘ (49% ÛÙËÓ ÔÌ¿‰· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È 15% ÛÙÔ˘˜ Ì¿ÚÙ˘Ú˜) Î·È „˘¯ÔÎÈÓËÙÈ΋˜ ηı˘ÛÙ¤ÚËÛ˘ (55% ÛÙËÓ ÔÌ¿‰· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È 29% ÛÙÔ˘˜ Ì¿ÚÙ˘Ú˜). ™Â η̛· ·fi ÙȘ ·Ú·¿Óˆ ÌÂϤÙ˜ ‰ÂÓ ‚Ú¤ıËΠÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÙˆÓ ·ıÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÂÁÎÂÊ¿ÏÔ˘ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô. ™Â ‰‡Ô, fï˜, ·fi ·˘Ù¤˜ ·Ú·ÙËÚ‹ıËΠÛËÌ·ÓÙÈ΋ Ù¿ÛË ÁÈ· ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ÂÚÈÎÔÈÏȷ΋˜ Ï¢ÎÔÌ·Ï¿Î˘ÓÛ˘ ÛÙ· ÓÂÔÁÓ¿ Ô˘ ¯ÔÚËÁ‹ıËΠ‰ÂÍ·ÌÂı·˙fiÓË (12,15). ™Â ·ÓÙ›ıÂÛË Ì ÙȘ ·Ú·¿Óˆ ÌÂϤÙ˜, ÔÈ Vincer (16) Î·È Jones (17) ¤‰ÂÈÍ·Ó Ì›· ÌË ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÛÙË Û˘¯ÓfiÙËÙ· Ù˘ ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘, ÂÓÒ ‰‡Ô ¿ÏϘ ÌÂϤÙ˜, ÙˆÓ Cummings (18) Î·È Romagnoli (19), ¤‰ÂÈÍ·Ó fiÙÈ ‰ÂÓ ˘¿Ú¯Ô˘Ó ÛËÌ·ÓÙÈΤ˜ Ì·ÎÚÔÚfiıÂÛ̘ ÂÈÙÒÛÂȘ ·fi ÙË ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô. OÈ Romagnoli Î·È Û˘Ó (19) ¯ÔÚ‹ÁËÛ·Ó ÙË ‰ÂÍ·ÌÂı·˙fiÓË ÓˆÚ›˜ (ÙËÓ 4Ë Ë̤ڷ ˙ˆ‹˜) Î·È ÁÈ· 7 Ë̤Ú˜. ™ÙËÓ ËÏÈΛ· ÙˆÓ 3 ÂÙÒÓ ·Ú·Ù‹ÚËÛ·Ó fiÙÈ Ë ÔÌ¿‰·

ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ ¤Ï·‚·Ó ‰ÂÍ·ÌÂı·˙fiÓË Î·È Ë ÔÌ¿‰· ÙˆÓ Ì·ÚÙ‡ÚˆÓ Â›¯·Ó ÙËÓ ›‰È· Û˘¯ÓfiÙËÙ· ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘, ÓÔËÙÈÎÔ‡ ËÏ›ÎÔ˘ Î·È ÛÔ‚·ÚÒÓ Ó¢ÚÔ·ÈÛıËÙËÚÈ·ÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ. ∂›Û˘, ÔÈ ‰‡Ô ÔÌ¿‰Â˜ ›¯·Ó ÙËÓ ›‰È· Û˘¯ÓfiÙËÙ· ÛÔ‚·ÚÒÓ ·ÓˆÌ·ÏÈÒÓ ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÂÁÎÂÊ¿ÏÔ˘. OÈ ‰‡Ô ÙÂÏÂ˘Ù·›Â˜ ÌÂϤÙ˜ ıˆڋıËΠfiÙÈ ‰ÂÓ Â›¯·Ó ·ÚÎÂÙ‹ ÛÙ·ÙÈÛÙÈ΋ ÈÛ¯‡, ÏfiÁˆ ÙÔ˘ ÌÈÎÚÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ Û˘ÌÌÂÙ›¯·Ó (20). ¡Â˘ÚÔ·Ó·Ù˘ÍȷΤ˜ ÂÎÙÈÌ‹ÛÂȘ ·È‰ÈÒÓ Ô˘ Û˘ÌÌÂÙ›¯·Ó Û ÌÂϤÙ˜ ¯ÔÚ‹ÁËÛ˘ ‰ÂÍ·ÌÂı·˙fiÓ˘, Û˘Ó¯›˙ÔÓÙ·È ·fi ‰È¿ÊÔÚ˜ ÔÌ¿‰Â˜ ÂÚ¢ÓËÙÒÓ. O Yeh, Ô˘ ‹Ù·Ó Ô ÚÒÙÔ˜ Ô˘ ‰È·›ÛÙˆÛ ÙȘ ÚÒÈ̘ ÂÈÙÒÛÂȘ ·fi ÙË ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô Û ·È‰È¿ ËÏÈΛ·˜ 2 ÂÙÒÓ, Û ̛· ÚfiÛÊ·ÙË ÌÂϤÙË ÂÎÙ›ÌËÛ ٷ ›‰È· ·È‰È¿ Û ۯÔÏÈ΋ ËÏÈΛ· (21). ∏ Û˘¯ÓfiÙËÙ· ·ıÔÏÔÁÈÎÒÓ Ó¢ÚÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ ÛÙË ÌÂϤÙË ·˘Ù‹ ‹Ù·Ó ·˘ÍË̤ÓË ÛÙËÓ ÔÌ¿‰· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Û ۯ¤ÛË Ì ÙÔ˘˜ Ì¿ÚÙ˘Ú˜. ∆· ·È‰È¿ ÛÙ· ÔÔ›· ›¯Â ¯ÔÚËÁËı› ‰ÂÍ·ÌÂı·˙fiÓË Â›¯·Ó ¯ÂÈÚfiÙÂÚË Â›‰ÔÛË ÛÙȘ ‰ÂÍÈfiÙËÙ˜ Ô˘ ·ÊÔÚÔ‡Û·Ó ÛÙË ¯Ú‹ÛË ÙˆÓ ¯ÂÚÈÒÓ, ÛÙÔ ·È¯Ó›‰È Ì ÙË Ì¿Ï· Î·È ÛÙËÓ ÈÛÔÚÚÔ›· ÛÙÔ Movement ABC test Û ۯ¤ÛË Ì ÙÔ˘˜ Ì¿ÚÙ˘Ú˜. ∂ÈϤÔÓ, ›¯·Ó ¯ÂÈÚfiÙÂÚÔ Û˘ÓÙÔÓÈÛÌfi ÙˆÓ ÎÈÓ‹ÛˆÓ, ÔÙÈ΋ ·ÓÙ›ÏË„Ë Î·È ÔÙÈÎÔÎÈÓËÙÈÎfi Û˘ÓÙÔÓÈÛÌfi, ηıÒ˜ Î·È ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚÔ ÔÏÈÎfi ÓÔËÙÈÎfi ËÏ›ÎÔ, ÏÂÎÙÈÎfi ÓÔËÙÈÎfi ËÏ›ÎÔ Î·È ÓÔËÙÈÎfi ËÏ›ÎÔ Ô˘ ·ÊÔÚÔ‡Û ÛÙËÓ ·fi‰ÔÛË. ∞˘Ù‹ Ë ‰È·ÊÔÚ¿ ÛÙÔ ÓÔËÙÈÎfi ËÏ›ÎÔ ‰ÂÓ Â›¯Â ‚ÚÂı› Û ÌÈÎÚfiÙÂÚË ËÏÈΛ·. ∆· ·È‰È¿ Ù˘ ÔÌ¿‰·˜ Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ›¯·Ó ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚË ‚·ıÌÔÏÔÁ›· ÛÙ· Ì·ıËÌ·ÙÈο Î·È ÙË ÁÚ·ÌÌ·ÙÈ΋ Û˘ÁÎÚÈÙÈο Ì ÙÔ˘˜ Ì¿ÚÙ˘Ú˜. ∏ ‰˘ÛÌÂÓ‹˜ ÂͤÏÈÍË ÛÙȘ ÁÓˆÛÙÈΤ˜ ÏÂÈÙÔ˘ÚÁ›Â˜ Û˘Û¯ÂÙ›ÛÙËΠ̠ÌÂȈ̤ÓË ·‡ÍËÛË Ù˘ ÂÚÈ̤ÙÚÔ˘ ÎÂÊ·Ï‹˜ (21).

∞Ó·ÛÎÔ‹ÛÂȘ Ù˘¯·ÈÔÔÈËÌ¤ÓˆÓ ÌÂÏÂÙÒÓ O Barrington (22), ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÎÙÈÌ‹ÛÂÈ Ì ÌÂÁ·Ï‡ÙÂÚË ·ÎÚ›‚ÂÈ· ÙÔ Èı·Ófi ̤ÁÂıÔ˜ ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ÙˆÓ ÂÈÙÒÛÂˆÓ Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË, Ú·ÁÌ·ÙÔÔ›ËÛ ̛· Û˘ÛÙËÌ·ÙÈ΋ ·Ó·ÛÎfiËÛË ÙˆÓ Ù˘¯·ÈÔÔÈËÌ¤ÓˆÓ ÌÂÏÂÙÒÓ Ô˘ ›¯·Ó ‰ËÌÔÛÈ¢ı› ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·. ∆Ô ·ÔÙ¤ÏÂÛÌ· Ù˘ ÌÂÙ·-·Ó¿Ï˘Û˘ ¤‰ÂÈÍ fiÙÈ Ë ıÂÚ·›· Ì ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô ¶·È‰È·ÙÚÈ΋ 2005;68:377-384

Pediatr Sept-Oct 05

17-10-05

16:52

™ÂÏ›‰·380

380

∂. ªÔ‡˙· Î·È Û˘Ó.

Û¯ÂÙ›˙ÂÙ·È Ì ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘ Î·È Ù˘ „˘¯ÔÎÈÓËÙÈ΋˜ ηı˘ÛÙ¤ÚËÛ˘.

O Û¯ÂÙÈÎfi˜ ΛӉ˘ÓÔ˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘ Ô˘ ÂÍ¿ÁÂÙ·È ·fi ÌÂϤÙ˜ Ì contamination <30% Â›Ó·È 2,86 (95% CI 1,95, 4,19). O Û¯ÂÙÈÎfi˜ ΛӉ˘ÓÔ˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Ó¢ÚÔ·Ó·Ù˘ÍÈ·ÎÔ‡ ÚÔ‚Ï‹Ì·ÙÔ˜ ·fi ÌÂϤÙ˜ Ì ÚfiÛÌÈÍË (contamination) <30% Â›Ó·È 1,66 (95%CI 1,26, 2,19). ∞fi ·˘Ù‹ ÙËÓ ˘ÔÔÌ¿‰· ÙˆÓ ÌÂÏÂÙÒÓ ÂÍ¿ÁÂÙ·È ÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ Ô ·ÚÈıÌfi˜ ÙˆÓ ÚfiˆÚˆÓ ÓÂÔÁÓÒÓ, ÛÙ· ÔÔ›· Ú¤ÂÈ Ó· ¯ÔÚËÁËı› ıÂÚ·¢ÙÈο ‰ÂÍ·ÌÂı·˙fiÓË ÁÈ· Ó· ¤¯Ô˘Ì ¤Ó· ·ÎfiÌË ·È‰› Ì ÂÁÎÂÊ·ÏÈ΋ ·Ú¿Ï˘ÛË (Number needed to harm - NNH), Â›Ó·È 7. °È· Ó· ¤¯Ô˘Ì ¤Ó· ·ÎfiÌË ·È‰› Ì Ó¢ÚÔ·Ó·Ù˘Íȷο ÚÔ‚Ï‹Ì·Ù·, Ô NNH Â›Ó·È 11. O Barrington ηٷϋÁÂÈ fiÙÈ Ë ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÁÈ· ÙËÓ ÚfiÏË„Ë Î·È ıÂÚ·›· Ù˘ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ‰˘ÛÏ·Û›·˜ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô Û˘Ó‰¤ÂÙ·È Ì ‰Ú·Ì·ÙÈ΋ ·‡ÍËÛË ÙˆÓ Ó¢ÚÔ·Ó·Ù˘ÍÈ·ÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ Î·È Û˘ÓÈÛÙ¿ Ó· ÂÁηٷÏÂÈÊı› Ë ¯Ú‹ÛË ÙÔ˘˜.

OÈ Halliday Î·È Û˘Ó ·Ó·ÛÎfiËÛ·Ó 37 Ù˘¯·ÈÔÔÈË̤Ó˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ÂÓ‰ÔÊϤ‚È·˜ ¯ÔÚ‹ÁËÛ˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Û 3 ‰È·ÊÔÚÂÙÈΤ˜ ÂÚÈfi‰Ô˘˜ ηٿ ÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·: ÓˆÚ›˜ (<96 ÒÚ˜ ˙ˆ‹˜) (23), Û¯ÂÙÈο ÓˆÚ›˜ (7Ë-14Ë Ë̤ڷ ˙ˆ‹˜) (24) Î·È ·ÚÁ¿ (>3 ‚‰ÔÌ¿‰Â˜ ˙ˆ‹˜) (25). ™Â ·˘Ù¤˜ ÙȘ ÙÚÂȘ ÌÂÙ·-·Ó·Ï‡ÛÂȘ ÂÎÙÈÌ‹ıËΠÙÔ fiÊÂÏÔ˜ Û ۯ¤ÛË Ì ÙÔ ÎfiÛÙÔ˜ Ù˘ ¯ÔÚ‹ÁËÛ˘ ‰ÂÍ·ÌÂı·˙fiÓ˘. ¶·Ú·ÙËÚ‹ıËΠ‚Ú·¯˘ÚfiıÂÛÌË ‚ÂÏÙ›ˆÛË Ù˘ ·ÓÙ·ÏÏ·Á‹˜ ÙˆÓ ·ÂÚ›ˆÓ Î·È Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ Ó¢ÌfiÓˆÓ Î·È Ù·¯‡ÙÂÚË ·Ô‰¤ÛÌ¢ÛË ·fi ÙÔÓ ·Ó·Ó¢ÛÙ‹Ú· ÌÂÙ¿ ÙË ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ Û fiϘ ÙȘ ÂÚÈfi‰Ô˘˜. ∂›Û˘, ‚Ú¤ıËΠ̛ˆÛË Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙË “ÓˆÚ›˜” Î·È ÙË “Û¯ÂÙÈο ÓˆÚ›˜” ıÂÚ·›·. ∆¤ÏÔ˜, ÛËÌÂÈÒıËΠ̛ˆÛË Ù˘ ÓÂÔÁÓÈ΋˜ ıÓËÛÈÌfiÙËÙ·˜ ÌfiÓÔ ÛÙË Û¯ÂÙÈο ÓˆÚ›˜ ¯ÔÚ‹ÁËÛË Ì ¤Ó·Ó ÂÈϤÔÓ ÂÈ˙ÒÓÙ· ÁÈ· οı 16 ÓÂÔÁÓ¿ ÛÙ· ÔÔ›· ¯ÔÚËÁ‹ıËΠ‰ÂÍ·ÌÂı·˙fiÓË. ∏ ‰ÂÍ·ÌÂı·˙fiÓË Ô˘ ¯ÔÚËÁÂ›Ù·È Â›Ù ӈڛ˜, ›Ù ·ÚÁ¿ ‰ÂÓ ÂËÚ¿˙ÂÈ ÙËÓ ÂÈ‚›ˆÛË. ÕÌÂÛ˜ ÂÈÏÔΤ˜, fiˆ˜ ·‡ÍËÛË Ù˘ ·ÈÌÔÚÚ·Á›·˜ ·fi ÙÔ ÂÙÈÎfi, ‰È¿ÙÚËÛË ÙÔ˘ ÂÓÙ¤ÚÔ˘ (Ô˘ Û˘Ì‚·›ÓÂÈ Î˘Ú›ˆ˜ ÛÙË ÓˆÚ›˜ ¯ÔÚ‹ÁËÛË), ˘ÂÚÁÏ˘Î·ÈÌ›·, ˘¤ÚÙ·ÛË, ˘ÂÚÙÚÔÊÈ΋ ηډÈÔÌ˘Ô¿ıÂÈ· Î·È Î·ı˘ÛÙ¤ÚËÛË Ù˘ ·Ó¿Ù˘Í˘ ‹Ù·Ó ÙÔ ÎfiÛÙÔ˜ Û fiϘ ÙȘ ÂÚÈfi‰Ô˘˜.

O ΛӉ˘ÓÔ˜ Ì·ÎÚÔÚfiıÂÛÌ˘ ·ıÔÏÔÁÈ΋˜ ¶·È‰È·ÙÚÈ΋ 2005;68:377-384

Ó¢ÚÔÏÔÁÈ΋˜ ÂͤٷÛ˘ Î·È ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘ ‹Ù·Ó ÛËÌ·ÓÙÈο ·˘ÍË̤ÓÔ˜, ·Ó Î·È Ë ÌÂıÔ‰ÔÏÔÁ›· ÛÙȘ ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ ‰ÂÓ ‹Ù·Ó ¿ÓÙ· ÈηÓÔÔÈËÙÈ΋ Î·È Ù· ·È‰È¿ ÂÎÙÈÌ‹ıËÎ·Ó Î˘Ú›ˆ˜ ÚÈÓ ·fi ÙË Û¯ÔÏÈ΋ ËÏÈΛ·. §fiÁˆ ÙÔ˘ ·˘ÍË̤ÓÔ˘ ÎÈÓ‰‡ÓÔ˘ ÁÈ· Èı·Ó¤˜ ‚Ú·¯˘ÚfiıÂÛ̘ Î·È Ì·ÎÚÔÚfiıÂÛ̘ ÂÈÏÔΤ˜ Û ۯ¤ÛË Ì ÙÔ Èı·Ófi ‚Ú·¯˘ÚfiıÂÛÌÔ fiÊÂÏÔ˜, ÔÈ Halliday Î·È Û˘Ó Û˘ÓÈÛÙÔ‡Ó fiÙÈ Ë ‰ÂÍ·ÌÂı·˙fiÓË ‰ÂÓ Ú¤ÂÈ ÔÙ¤ Ó· ¯ÔÚËÁÂ›Ù·È ÓˆÚ›˜ ÁÈ· ÙËÓ ÚfiÏË„Ë Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ. ¶Ú¤ÂÈ Ó· ¯ÔÚËÁÂ›Ù·È ÌfiÓÔ Û¯ÂÙÈο ÓˆÚ›˜, ‰ËÏ·‰‹ ÌÂٷ͇ 7˘-14˘ Ë̤ڷ˜ Î·È Ú¤ÂÈ Ó· ‰È·Ê˘Ï¿ÛÛÂÙ·È ÌfiÓÔ ÁÈ· ÓÂÔÁÓ¿ Ô˘ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ·Ô‰ÂÛÌ¢ÙÔ‡Ó ·fi ÙÔÓ ·Ó·Ó¢ÛÙ‹Ú·, ÂÓÒ Ë ‰fiÛË Î·È Ë ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ Ú¤ÂÈ Ó· Â›Ó·È ÔÈ ÂÏ¿¯ÈÛÙ˜ ‰˘Ó·Ù¤˜. ¶ÚfiÛÊ·Ù·, Ë American Academy of Pediatrics Î·È Ë Canadian Pediatric Society (26) ·Ó·ÛÎfiËÛ·Ó ÙȘ ÂӉ›ÍÂȘ Ù˘ Â›ÙˆÛ˘ Ù˘ ¯ÔÚ‹ÁËÛ˘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Î·Ù¿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô - Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ ÙˆÓ ·ÒÙÂÚˆÓ ÂÈÏÔÎÒÓ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË - Î·È ¤Î·Ó·Ó ÙȘ ·ÎfiÏÔ˘ı˜ Û˘ÛÙ¿ÛÂȘ: ·) ∏ Û˘ÛÙËÌ·ÙÈ΋ ¯ÔÚ‹ÁËÛË ÂÓ‰ÔÊϤ‚È·˜ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÁÈ· ÙËÓ ÚfiÏË„Ë ‹ ÙË ıÂÚ·›· Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙ· ÚfiˆÚ· ÓÂÔÁÓ¿ ‰ÂÓ Û˘ÓÈÛٿٷÈ. ‚) ∏ ¯ÔÚ‹ÁËÛË ÂÓ‰ÔÊϤ‚È·˜ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÁÈ· ÙËÓ ÚfiÏË„Ë ‹ ÙË ıÂÚ·›· Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ Ú¤ÂÈ Ó· ÂÚÈÔÚ›˙ÂÙ·È Û ηϿ ۯ‰ȷṲ̂Ó˜ Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ Ì ̷ÎÚÔ¯ÚfiÓÈ· ·Ú·ÎÔÏÔ‡ıËÛË. Á) ∏ Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂÎÙ›ÌËÛË ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ Û˘ÌÌÂÙ›¯·Ó ÛÂ Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÂÓı·ÚÚ‡ÓÂÙ·È ıÂÚÌ¿. ‰) ∂›Ó·È ·Ó·Áη›Ô Ó· Á›ÓÔ˘Ó ÎÏÈÓÈΤ˜ Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ Ì ¿ÏÏ· ÂÓ‰ÔÊϤ‚È· ‹ ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹. Â) ∂ÎÙfi˜ ·fi ÙȘ Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜, Ë ¯ÔÚ‹ÁËÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ú¤ÂÈ Ó· ÂÚÈÔÚ›˙ÂÙ·È Û ȉȷ›ÙÂÚ˜ ÎÏÈÓÈΤ˜ ÂÚÈÙÒÛÂȘ Î·È ÔÈ ÁÔÓ›˜ ı· Ú¤ÂÈ Ó· ÂÓËÌÂÚÒÓÔÓÙ·È Ï‹Úˆ˜ Î·È Ó· Û˘ÌʈÓÔ‡Ó ÁÈ· ÙË ıÂÚ·›·.

∂Ó·ÏÏ·ÎÙÈο ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Ÿˆ˜ ·Ó·Ê¤ÚıËÎÂ, ˘¿Ú¯Ô˘Ó ÈÛ¯˘Ú¤˜ ÂӉ›ÍÂȘ fiÙÈ ÛÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ Û˘ÌÌÂÙ¤¯Ô˘Ó ÊÏÂÁÌÔÓÒ‰ÂȘ

Pediatr Sept-Oct 05

17-10-05

16:52

™ÂÏ›‰·381

381

∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Î·È ÓÂÔÁÓÈÎfi˜ ÂÁΤʷÏÔ˜

·Ú¿ÁÔÓÙ˜. ø˜ ÂÎ ÙÔ‡ÙÔ˘, Ë ÂÓ‰ÂÈÎÓ˘fiÌÂÓË ıÂÚ·›· Â›Ó·È Ù· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹. ∏ ‰ÂÍ·ÌÂı·˙fiÓË Â›Ó·È ¤Ó· ÈÛ¯˘Úfi ·ÓÙÈÊÏÂÁÌÔÓ҉˜ ÎÔÚÙÈÎÔÂȉ¤˜. OÈ Ê·ÚÌ·ÎÔÏÔÁÈΤ˜ ‰fiÛÂȘ Ô˘ Û˘¯Ó¿ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙȘ ÌÂϤÙ˜ Î·È ÛÙËÓ Î·ı’ Ë̤ڷ Ú¿ÍË Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ·fi 1015 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚ˜ Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ËÌÂÚ‹ÛÈ·˜ ¤ÎÎÚÈÛ˘ Ù˘ ÎÔÚÙÈ˙fiÏ˘ ÛÙÔ ÓÂÔÁÓfi. ∂›Û˘, Ô ¯ÚfiÓÔ˜ ËÌ›ÛÂÈ·˜ ˙ˆ‹˜ Ù˘ Â›Ó·È ·Ú·ÙÂٷ̤ÓÔ˜ ÛÙ· ÂÍ·ÈÚÂÙÈο ¯·ÌËÏÔ‡ ‚¿ÚÔ˘˜ Á¤ÓÓËÛ˘ ÓÂÔÁÓ¿. À„ËÏ¿ Â›‰· ‰ÂÍ·ÌÂı·˙fiÓ˘ ÌÔÚ› Ó· ·˘Í¿ÓÔ˘Ó ÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÈÏÔÎÒÓ. ∂›Ó·È ÁÓˆÛÙfi fiÙÈ Ù· ‰È¿ÊÔÚ· ÌfiÚÈ· ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ¤¯Ô˘Ó ‰È·ÊÔÚÂÙÈ΋ ‰Ú¿ÛË. ∏ ‰È·ÊÔÚÂÙÈ΋ ‰Ú¿ÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÂÍËÁÂ›Ù·È Ì ÙË ıˆڛ· Ù˘ ÁÔÓȉȷ΋˜ - ÌË ÁÔÓȉȷ΋˜ ‰Ú¿Û˘. ∂›Û˘, ÔÈ ‰È·ÊÔÚÂÙÈΤ˜ ÂÈÙÒÛÂȘ ÙˆÓ ‰È·ÊfiÚˆÓ ÎÔÚÙÈÎÔÂȉÒÓ ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ ¤¯Ô˘Ó ‰Âȯı› ÂÈÚ·Ì·ÙÈο. ¶ÈÛÙ‡ÂÙ·È fiÙÈ Ù· ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ‹ ‰ÚÔ˘Ó Ì¤Ûˆ ÁÔÓȉȷÎÒÓ - ÌË ÁÔÓȉȷÎÒÓ Ì˯·ÓÈÛÌÒÓ (27). ªÂ ÙÔ˘˜ ÎÏ·ÛÈÎÔ‡˜ ÁÔÓȉȷÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜, Ù· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ÂÚÓÔ‡Ó Ì¤Û· ·fi ÙË ÌÂÌ‚Ú¿ÓË ÙÔ˘ ΢ÙÙ¿ÚÔ˘, Û˘Ó‰¤ÔÓÙ·È Û ÂȉÈΤ˜ ı¤ÛÂȘ Ì ÙÔ DNA, ·ÏÏËÏÂȉÚÔ‡Ó Ì ˘ÚËÓÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÌÂÙ·ÁÚ·Ê‹˜ (transcription factors) Î·È ÚÔηÏÔ‡Ó ÌÂÙ·ÁÚ·Ê‹ ÙÔ˘ ÁÔÓȉ›Ô˘ (gene transcription). ŸÙ·Ó Ù· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ‰ÚÔ˘Ó Ì¤Ûˆ ÁÔÓȉȷÎÒÓ Ì˯·ÓÈÛÌÒÓ, ¯ÚÂÈ¿˙ÔÓÙ·È ÙÔ˘Ï¿¯ÈÛÙÔÓ 30 min Î·È Û˘¯Ó¿ ·ÚÎÂÙ¤˜ ÒÚ˜ ÁÈ· Ó· ·Ú¯›ÛÔ˘Ó Ó· ‰ÚÔ˘Ó. ªÂ ÙÔ˘˜ ÌË ÁÔÓȉȷÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜, Ë ‰Ú¿ÛË ÙÔ˘˜ ·Ú¯›˙ÂÈ Ôχ ÈÔ ÁÚ‹ÁÔÚ·, ̤۷ Û ‰Â˘ÙÂÚfiÏÂÙ· ¤ˆ˜ Ï›Á· ÏÂÙ¿. ∆· ÎÔÚÙÈÎÔÂȉ‹ ÌÂÙ·‚¿ÏÏÔ˘Ó ÙȘ ȉÈfiÙËÙ˜ ÙˆÓ ÌÂÌ‚Ú·ÓÒÓ Î·È ·Ó·ÛÙ¤ÏÏÔ˘Ó ÙËÓ Â›ÛÔ‰Ô Na Î·È Ca ̤ۈ Ù˘ ÌÂÌ‚Ú¿Ó˘, Ô˘ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË Ì›ˆÛË ÙÔ˘ ÂÓ‰Ô΢ÙÙ¿ÚÈÔ˘ Ca. ∂›Ó·È ¯Ú‹ÛÈÌË Ë ÁÓÒÛË Ù˘ Û¯ÂÙÈ΋˜ ÈÛ¯‡Ô˜ ÙˆÓ ‰È·ÊfiÚˆÓ ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉÒÓ, fiÛÔÓ ·ÊÔÚ¿ ÛÙË ÁÔÓȉȷ΋ Î·È ÛÙË ÌË ÁÔÓȉȷ΋ ‰Ú¿ÛË. ∞fi Ù· Û˘Ó‹ıˆ˜ ¯ÚËÛÈÌÔÔÈÔ‡ÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, ÙË ÌÂÁ·Ï‡ÙÂÚË Û¯ÂÙÈ΋ ÈÛ¯‡ - Û ۯ¤ÛË Ì ·˘Ù‹ Ù˘ Ú‰ÓÈ˙ÔÏfiÓ˘ - ˆ˜ ÚÔ˜ ÙË ÁÔÓȉȷ΋ ‰Ú¿ÛË, ¤¯Ô˘Ó Ë ‰ÂÍ·ÌÂı·˙fiÓË Î·È Ë ‚ËÙ·ÌÂı·˙fiÓË, Ë ÔÔ›· Â›Ó·È 6,25 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË ·˘Ù‹˜ Ù˘ Ú‰ÓÈ˙ÔÏfiÓ˘. ∆Ë ÌÂÁ·Ï‡ÙÂÚË Û¯ÂÙÈ΋ ÈÛ¯‡ Û ۯ¤ÛË Ì ÂΛÓË Ù˘ Ú‰ÓÈ˙ÔÏfiÓ˘ ˆ˜ ÚÔ˜ ÙË ÌË ÁÔÓȉȷ΋ ‰Ú¿ÛË ¤¯ÂÈ Ë ‰ÂÍ·ÌÂı·˙fiÓË, Ë ÔÔ›· Â›Ó·È 2,9 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË, ÂÓÒ ÙË

ÌÈÎÚfiÙÂÚË ¤¯ÂÈ Ë ‚ËÙ·ÌÂı·˙fiÓË, Ë ÔÔ›· Â›Ó·È 0,56 ÊÔÚ¤˜ Ë ‰Ú¿ÛË Ù˘ Ú‰ÓÈ˙ÔÏfiÓ˘. ™‹ÌÂÚ·, ˘¿Ú¯ÂÈ Û¯ÂÙÈ΋ ·‚‚·ÈfiÙËÙ· ˆ˜ ÚÔ˜ ÙÔ Â›‰Ô˜ ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ô˘ ı· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó. °È· ÂÓ‰ÔÊϤ‚È· ıÂÚ·›· Ì ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Û ÌÂÁ¿Ï˜ ‰fiÛÂȘ ıˆÚËÙÈο Â›Ó·È ÚÔÙÈÌfiÙÂÚË Ì›· ÈÛÔÚÚÔ›· ÌÂٷ͇ ÁÔÓȉȷ΋˜ - ÌË ÁÔÓȉȷ΋˜ ‰Ú¿Û˘. ÀÂÈÛ¤Ú¯ÔÓÙ·È, fï˜, ·Ú¿ÁÔÓÙ˜ fiˆ˜ Ë ÂÈı˘ÌËÙ‹ Ù·¯‡ÙËÙ· ‰Ú¿Û˘, ÔfiÙ ÚÔÙÈÌÒÓÙ·È Ô˘Û›Â˜ Ì ÈÛ¯˘ÚfiÙÂÚË ÌË ÁÔÓȉȷ΋ ‰Ú¿ÛË ‹ ÔÈ ÂÈÏÔΤ˜ ÙˆÓ ‰È·ÊfiÚˆÓ Ô˘ÛÈÒÓ. ÕÏÏÔ˜ ÚÔ‚ÏËÌ·ÙÈÛÌfi˜ Û¯ÂÙÈο Ì ÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉԇ˜ Ô˘ ı· ¯ÚËÛÈÌÔÔÈËı›, ˘‹ÚÍ ÌÂÙ¿ ÙË ÌÂϤÙË ÙˆÓ Baud Î·È Û˘Ó (8), fiÛÔÓ ·ÊÔÚ¿ ÛÙË Ó¢ÚÔÙÔÍÈÎfiÙËÙ· ÙˆÓ ‰È·ÊfiÚˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ ÙÔ˘ ÔÓÙÈÎÔ‡. ™ÙȘ in vitro ÌÂϤÙ˜ ηÏÏÈÂÚÁ‹ıËÎ·Ó Ó¢ÚÒÓ˜ ÙÔ˘ ÊÏÔÈÔ‡ ·fi ¤Ì‚Ú˘· ÔÓÙ›ÎÈ·, ηıÒ˜ Î·È ·ÛÙÚÔ·ÙÙ·Ú· ·fi ÓÂÔÁ¤ÓÓËÙ· ÔÓÙ›ÎÈ·, Û ˘ÏÈÎfi Ô˘ ÂÚÈ›¯Â Û··ÛÌ· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ (Ô˘ ÂÚÈ›¯Â ÛÔ˘ÏÊ›‰È· ˆ˜ Û˘ÓÙËÚËÙÈÎfi), Û··ÛÌ· Ù˘ ‚ËÙ·ÌÂı·˙fiÓ˘, ηı·Ú‹ ‰ÂÍ·ÌÂı·˙fiÓË, ηı·Ú‹ ‚ËÙ·ÌÂı·˙fiÓË ‹ ÌfiÓÔ ÛÔ˘ÏÊ›‰È·. ∆· ·ÙÙ·Ú· ηÏÏÈÂÚÁ‹ıËÎ·Ó ÛÂ Ê˘ÛÈÔÏÔÁÈΤ˜ Û˘Óı‹Î˜, ηıÒ˜ Î·È ˘fi ÙËÓ Â›‰Ú·ÛË Ó¢ÚÔÙÔÍÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ‹ ˘ÔÍ›·˜. µÚ¤ıËΠfiÙÈ Ë ‚ȈÛÈÌfiÙËÙ· ÙˆÓ Ó¢ÚÒÓˆÓ ‰ÂÓ ÂËÚ·˙fiÙ·Ó ·fi ÙËÓ Î·ı·Ú‹ ‰ÂÍ·ÌÂı·˙fiÓË, ÙËÓ Î·ı·Ú‹ ‚ËÙ·ÌÂı·˙fiÓË ‹ ÙÔ Û··ÛÌ· Ù˘ ‚ËÙ·ÌÂı·˙fiÓ˘, ÂÓÒ ÙÔ Û··ÛÌ· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ‹ Ù· ÛÔ˘ÏÊ›‰È· ÂÈÙ¿¯˘Ó·Ó ÙÔÓ ı¿Ó·ÙÔ ÙÔ˘ Ó¢ÚÒÓ·. ∏ ηı·Ú‹ ‰ÂÍ·ÌÂı·˙fiÓË Î·È Ë Î·ı·Ú‹ ‚ËÙ·ÌÂı·˙fiÓË ÂÏ¿ÙÙˆÓ·Ó Î·Ù¿ 40-50% ÙÔÓ ı¿Ó·ÙÔ ÙˆÓ Ó¢ÚÒÓˆÓ Ô˘ ÚÔηÏÔ‡ÓÙ·Ó ·fi Ó¢ÚÔÙÔÍÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ ÙÔ ¡-methyl-D-asparate (NMDA) Î·È Ë ˘ÔÍ›·, ÂÓÒ ÙÔ Û··ÛÌ· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ‰ÂÓ Ì›ˆÓ ÙÔÓ ı¿Ó·ÙÔ ÙˆÓ Ó¢ÚÒÓˆÓ Ô˘ ÚÔηÏÔ‡ÓÙ·Ó ·fi ÙÔ˘˜ Ó¢ÚÔÙÔÍÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ‹ ÙËÓ ˘ÔÍ›·. ∆¤ÏÔ˜, Ù· ÛÔ˘ÏÊ›‰È· ·‡Í·Ó·Ó ÛËÌ·ÓÙÈο ÙËÓ ÙÔÍÈÎfiÙËÙ· ÙˆÓ Ó¢ÚÔÙÔÍÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ™ÙË Û˘Ó¤¯ÂÈ·, ÛÙȘ in vivo ÌÂϤÙ˜, ÔÈ ÂÚ¢ÓËÙ¤˜ ¤Î·Ó·Ó ¤Á¯˘ÛË Û΢¿ÛÌ·ÙÔ˜ Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘, Û΢¿ÛÌ·ÙÔ˜ Ù˘ ‚ËÙ·ÌÂı·˙fiÓ˘, ηı·Ú‹˜ ‰ÂÍ·ÌÂı·˙fiÓ˘, ηı·Ú‹˜ ‚ËÙ·ÌÂı·˙fiÓ˘ Î·È ÛÔ˘ÏÊȉ›ˆÓ ÂÓ‰ÔÂÚÈÙÔÓ·˚ο Û ÓÂÔÁ¤ÓÓËÙ· ÔÓÙ›ÎÈ·. ªÂ ÂȉÈΤ˜ Ù¯ÓÈΤ˜ ‰È·›ÛÙˆÛ·Ó ÙÔÓ ı¿Ó·ÙÔ ÙˆÓ Î˘ÙÙ¿ÚˆÓ Ì ÙÔÓ Ì˯·ÓÈÛÌfi Ù˘ ·fiÙˆÛ˘ ÛÙÔÓ ÂÁΤʷÏÔ ÙˆÓ ¶·È‰È·ÙÚÈ΋ 2005;68:377-384

Pediatr Sept-Oct 05

17-10-05

16:52

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382

∂. ªÔ‡˙· Î·È Û˘Ó.

ÔÓÙÈÎÒÓ, ΢ڛˆ˜ ÛÙÔÓ ÊÏÔÈfi Î·È ÙÔÓ ı¿Ï·ÌÔ. µÚ‹Î·Ó ·˘ÍË̤ÓÔ Î˘ÙÙ·ÚÈÎfi ı¿Ó·ÙÔ ÛÙÔÓ ÂÁΤʷÏÔ ÙˆÓ ÔÓÙÈÎÒÓ, ÛÙ· ÔÔ›· ›¯Â Á›ÓÂÈ ¤Á¯˘ÛË Û΢¿ÛÌ·ÙÔ˜ Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘, ÂÓÒ Ë Î·ı·Ú‹ ‰ÂÍ·ÌÂı·˙fiÓË, Ë Î·ı·Ú‹ ‚ËÙ·ÌÂı·˙fiÓË Î·È ÙÔ Û··ÛÌ· Ù˘ ‚ËÙ·ÌÂı·˙fiÓ˘ ‰ÂÓ ÂËÚ¤·Û·Ó ÙË ‰È¿ÚÎÂÈ· ˙ˆ‹˜ ÙˆÓ Î˘ÙÙ¿ÚˆÓ. ∆Ô Û˘Ì¤Ú·ÛÌ· ‹Ù·Ó fiÙÈ ›Ûˆ˜ Ù· ÛÔ˘ÏÊ›‰È· Ô˘ ÂÚȤ¯ÔÓÙ·È ÛÙÔ Û··ÛÌ· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘, ˆ˜ Û˘ÓÙËÚËÙÈÎfi, Â›Ó·È ‰˘ÓËÙÈο ÙÔÍÈο ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ. ∂Í·ÈÙ›·˜ Ù˘ ‰˘ÛÌÂÓÔ‡˜ Â›‰Ú·Û˘ Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ, ¤¯Ô˘Ó ÚÔÙ·ı› ÂÓ·ÏÏ·ÎÙÈο ÌfiÚÈ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ, Ù· ÔÔ›· ¤¯Ô˘Ó ¯ÔÚËÁËı› Û˘ÛÙËÌ·ÙÈο ‹ Ì ÂÈÛÓÔ¤˜. ¶Èı·Ó¿ ÂÓ·ÏÏ·ÎÙÈο ÛÙÂÚÔÂȉ‹, Ô˘ ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› Û˘ÛÙËÌ·ÙÈο, Â›Ó·È Ë ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfiÓË, Ë ˘‰ÚÔÎÔÚÙÈ˙fiÓË Î·È Ë ‚ËÙ·ÌÂı·˙fiÓË. ∏ ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfiÓË ¤¯ÂÈ ¯ÔÚËÁËı› ˆ˜ ıÂÚ·›· ÁÈ· Ó· ÂÈÙ·¯˘Óı› Ë ·Ô‰¤ÛÌ¢ÛË ·fi ÙÔÓ ·Ó·Ó¢ÛÙ‹Ú·. ∏ ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfiÓË Â›Ó·È ¤Ó· ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ¤˜ Ì ‚Ú·¯‡ÙÂÚË Î·È ÌÈÎÚfiÙÂÚË ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë ‰Ú¿ÛË ·fi ÙË ‰ÂÍ·ÌÂı·˙fiÓË, ·ÌÂÏËÙ¤· ·Ï·ÙÔÎÔÚÙÈÎÔÂȉ‹ ‰Ú¿ÛË, ÂÓÒ ÙÔ Û··ÛÌ¿ Ù˘ ‰ÂÓ ÂÚȤ¯ÂÈ ÛÔ˘ÏÊ›‰È·. Œ¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› Ì ۯÂÙÈ΋ ·ÛÊ¿ÏÂÈ· (28). ÷ÌËϤ˜ ‰fiÛÂȘ ˘‰ÚÔÎÔÚÙÈ˙fiÓ˘ ¯ÔÚËÁ‹ıËÎ·Ó Û ÓÂÔÁÓ¿ ÙȘ ÚÒÙ˜ Ë̤Ú˜ ˙ˆ‹˜ ÁÈ· ÙËÓ ÚfiÏË„Ë Ù˘ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ‰˘ÛÏ·Û›·˜. ªÂÙ¿ ·fi ¯·ÌËϤ˜ ‰fiÛÂȘ ˘‰ÚÔÎÔÚÙÈ˙fiÓ˘ (1 mg/kg/Ë̤ڷ) ‚Ú¤ıËΠfiÙÈ ÛÙ· ÓÂÔÁÓ¿ ·˘Ù¿ ·˘Í‹ıËΠηٿ ÙȘ 2 ÚÒÙ˜ ‚‰ÔÌ¿‰Â˜ ˙ˆ‹˜ Ë ÂÈ‚›ˆÛË ¯ˆÚ›˜ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋ ‰˘ÛÏ·Û›·, ÂÓÒ ‰ÂÓ ˘‹Ú¯·Ó ¿ÌÂÛ˜ ‹ ·ÒÙÂÚ˜ ÂÈÏÔΤ˜ (2). ∏ ‚ËÙ·ÌÂı·˙fiÓË Èı·ÓÒ˜ Â›Ó·È ·ÛʷϤÛÙÂÚË ·fi ÙË ‰ÂÍ·ÌÂı·˙fiÓË. ¶ÂÈÚ¿Ì·Ù· Û ÔÓÙ›ÎÈ· Î·È Ë ÂÌÂÈÚ›· ·fi ÙËÓ ÚÔÁÂÓÓËÙÈ΋ ¯ÔÚ‹ÁËÛ‹ Ù˘, ηıÈÛÙÔ‡Ó ÙË ‚ËÙ·ÌÂı·˙fiÓË ›Ûˆ˜ ÙÔ È‰·ÓÈÎfi ÂÓ·ÏÏ·ÎÙÈÎfi ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ¤˜ ·ÓÙ› ÁÈ· ÙË ‰ÂÍ·ÌÂı·˙fiÓË, ·ÏÏ¿ ‰ÂÓ ˘¿Ú¯Ô˘Ó ·ÚÎÂÙ¤˜ Û˘ÁÎÚÈÙÈΤ˜ ÌÂϤÙ˜ ÛÙ· ÓÂÔÁÓ¿. ™Â Ì›· ÌÈÎÚ‹ Ù˘¯·ÈÔÔÈË̤ÓË ÌÂϤÙË, Ë ¯ÔÚ‹ÁËÛË ‚ËÙ·ÌÂı·˙fiÓ˘ Û ÌÈÎÚ¤˜ ‰fiÛÂȘ Î·È ÁÈ· ‚Ú·¯‡ ‰È¿ÛÙËÌ· ‹Ù·Ó ÂÍ›ÛÔ˘ ·ÔÙÂÏÂÛÌ·ÙÈ΋ Ì ÙË ‰ÂÍ·ÌÂı·˙fiÓË, fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ·Ô‰¤ÛÌ¢ÛË ÙÔ˘ ÓÂÔÁÓÔ‡ ·fi ÙÔÓ ·Ó·Ó¢ÛÙ‹Ú· ¯ˆÚ›˜ ·ÓÂÈı‡ÌËÙ˜ ¿ÌÂÛ˜ ÂÈÏÔΤ˜ (29) ‹ ·ÒÙÂÚ˜ ÂÈÙÒÛÂȘ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË ÛÙËÓ ËÏÈΛ· ÙˆÓ 12 ÌËÓÒÓ (30). ¶·Ú’ fiÏ· ·˘Ù¿, Ë ›‰È· ¶·È‰È·ÙÚÈ΋ 2005;68:377-384

ÌÂϤÙË ‰ÂÓ ¤‰ÂÈÍ ·˘ÍË̤Ó˜ ·ÒÙÂÚ˜ ‰˘ÛÌÂÓ›˜ ÂÈÙÒÛÂȘ Ô‡Ù ÛÙËÓ ÔÌ¿‰· ÙˆÓ ÓÂÔÁÓÒÓ ÛÙ· ÔÔ›· ¯ÔÚËÁ‹ıËΠ‰ÂÍ·ÌÂı·˙fiÓË. ¶ÚfiÛÊ·Ù·, Ù· ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙË ıÂÚ·›· ‹ ÙËÓ ÚfiÏË„Ë Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ, ÂÂȉ‹ ıˆÚÂ›Ù·È fiÙÈ Ë ÙÔÈ΋ ‰Ú¿ÛË Û˘Ó‰¤ÂÙ·È Ì ÏÈÁfiÙÂÚ˜ ÂÈÏÔΤ˜. ª›· ÚfiÛÊ·ÙË ·Ó·ÛÎfiËÛË ÙˆÓ ÌÂÏÂÙÒÓ ÙˆÓ ÂÈÛÓÂfiÌÂÓˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Û˘Ì¤Ú·Ó fiÙÈ ÂÓÒ ˘‹Ú¯·Ó ‚Ú·¯˘ÚfiıÂÛÌ· ¢ÓÔ˚ο ·ÔÙÂϤÛÌ·Ù·, Ì ‚ÂÏÙ›ˆÛË ÛÙËÓ ·Ó·Ó¢ÛÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· Î·È ÌÂȈ̤ÓË ·Ó¿ÁÎË ÁÈ· ÌÂÙ¤ÂÈÙ· Û˘ÛÙËÌ·ÙÈο ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, ‰ÂÓ ‰È·ÈÛÙÒıËΠ̷ÎÚÔÚfiıÂÛÌ· fiÊÂÏÔ˜ ˆ˜ ÚÔ˜ ÙËÓ ÂÈ‚›ˆÛË ‹ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· ¯ÚfiÓÈ· ÓfiÛÔ ÙÔ˘ Ó‡ÌÔÓ· (31). ∆· ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Û˘Óԉ‡ÔÓÙ·È ·fi ÏÈÁfiÙÂÚ˜ ·ÚÂÓ¤ÚÁÂȘ (32), ·Ó Î·È ‰ÂÓ ˘¿Ú¯Ô˘Ó ·ÚÎÂÙ¤˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Ô˘ Ó· ÂϤÁ¯Ô˘Ó ÙȘ Ì·ÎÚÔÚfiıÂÛ̘ ÂÈÙÒÛÂȘ ÙÔ˘˜. ŒÙÛÈ, Ê·›ÓÂÙ·È fiÙÈ Ù· ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ÌÔÚ› Ó· ‚ÔËıÔ‡Ó Û ‹Ș ‹ ̤ÙÚȘ ÂÚÈÙÒÛÂȘ ¯ÚfiÓÈ·˜ ·Ó·Ó¢ÛÙÈ΋˜ ÓfiÛÔ˘, ·ÏÏ¿ ÛÙȘ ‚·ÚȤ˜ ÂÚÈÙÒÛÂȘ Â›Ó·È ··Ú·›ÙËÙ· Ù· ÂÓ‰ÔÊϤ‚È· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹.

™˘ÌÂÚ¿ÛÌ·Ù· ∆· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ¤¯Ô˘Ó ¢ÓÔ˚΋ ‰Ú¿ÛË ÛÙË ¯ÚfiÓÈ· ·Ó·Ó¢ÛÙÈ΋ ÓfiÛÔ, ·ÏÏ¿ Ë ‰ÂÍ·ÌÂı·˙fiÓË Û˘Ó‰¤ÂÙ·È Ì ÛÔ‚·Ú¤˜ ÂÈÏÔΤ˜. °›ÓÔÓÙ·È Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ ÁÈ· Ó· ‚ÚÂı› ÔÈÔ Â›Ó·È ÙÔ ÈÔ Î·Ù¿ÏÏËÏÔ ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ¤˜ Ô˘ Ú¤ÂÈ Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È Î·È ÔÈ· Â›Ó·È Ë ÌÈÎÚfiÙÂÚË ‰fiÛË Ô˘ ¤¯ÂÈ ıÂÙÈο ‚Ú·¯˘ÚfiıÂÛÌ· ·ÔÙÂϤÛÌ·Ù· Ì ÂÏ¿¯ÈÛÙ· ·ÚÓËÙÈο Ì·ÎÚÔÚfiıÂÛÌ·. ∏ American Academy of Pediatrics, Ë Canadian Pediatric Society Î·È Ë European Association of Perinatal Medicine Û˘ÓÈÛÙÔ‡Ó Ó· ÂÓÙ·¯ıÔ‡Ó Ù· ÓÂÔÁÓ¿ Ô˘ ›¯·Ó Ï¿‚ÂÈ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô, Û˘ÌÌÂÙ¤¯ÔÓÙ·˜ ÛÂ Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜, Û ÏÂÙÔÌÂÚ‹ Ó¢ÚÔÏÔÁÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË, ÒÛÙ ӷ ÂÎÙÈÌËı› Ë Ú·ÁÌ·ÙÈ΋ Û¯¤ÛË ÎÈÓ‰‡ÓÔ˘ ÚÔ˜ fiÊÂÏÔ˜ Ù˘ ıÂÚ·›·˜ Ì ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Jobe AH, Bancalari E. Bronchopulmonary dysplasia.

Am J Respir Crit Care Med 2001;163:1723-1729.

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2. Watterberg KL, Gerdes JS, Gifford KL, Lin HM. Prophylaxis against early adrenal insufficiency to prevent chronic lung disease in premature infants.

Pediatrics 1999;104:1258-1263.

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4. Bhuta T, Ohlsson A. Systematic review and metaanalysis of early postnatal dexamethasone for prevention of chronic lung disease. Arch Dis Child Fetal Neonatal Ed 1998;79:F26-F33.

5. Howard H. Reduction in size and total DNA of cerebrum and cerebellum in adult mice after corticosteroid treatment in infancy. Exp Neurol 1968;22: 191-208.

6. Howard E, Benjamins JA. DNA, ganglioside and sulfatide in brains of rats given corticosterone in infancy, with an estimate of cell loss during development.

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7. Weichsel ME Jr. The therapeutic use of glucocorticoid hormones in the perinatal period: potential neurological hazards. Ann Neurol 1977;2:364-366.

8. Baud O, Laudenbach V, Evrard P, Gressens P. Neurotoxic effects of fluorinated glucocorticoid preparations on the developing mouse brain: role of preservatives. Pediatr Res 2001;50:706-711.

9. Papile LA, Tyson JE, Stoll BJ, Wright LL, Donovan EF, Bauer CR et al. A multicenter trial of two dexamethasone regimens in ventilator-dependent premature infants. N Engl J Med 1998;338:1112-1118.

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16. Vincer MJ, Allen AC. Double blind controlled trial of 6-day pulse of dexamethasone for very low birth weight infants (VLBW <1500 grams) who are ventilator dependent at 4 weeks of age. Pediatr Res

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20. Halliday HL. Early postnatal dexamethasone and cerebral palsy. Pediatrics 2002;109:1168-1169.

21. Yeh TF, Lin YJ, Lin HC, Huang CC, Hsieh WS, Lin CH et al. Outcomes at school age after postnatal dexamethasone therapy for lung disease of prematurity. N Engl J Med 2004;350:1304-1313.

22. Barrington KJ. The adverse neurodevelopmental effects of postnatal steroids in the preterm infant: a systematic review of RCT’s. BMC Pediatrics 2001;1:1. Available at: http://www.biomedcentral.com/1471-2431/1/1 23. Halliday HL, Ehrenkranz RA. Early postnatal (<96 hours) corticosteroids for preventing chronic lung disease in preterm infants (Cochrane review). In: The Cochrane Library. Issue 1. Oxford: Update Software; 2003.

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In: The Cochrane Library. Issue 1. Oxford: Update Software; 2003.

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28. Andre P, Thebaud B, Odievre MH, Razafimahefa H, Zupan V, Dehan M et al. Methylprednisolone, an alternative to dexamethasone in very premature infants at risk of chronic lung disease. Intensive Care Med 2000;26:1496-1500.

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30. Hassan MS, Decastro MH, Dudhblai A, LaGamma EF. Twelve month neurodevelopmental outcome comparison between postnatal use of betamethasone vs dexamethasone for the treatment of evolving BPD in extremely low birth weight infants. Pediatr Res 2003;53:479-480A. ¶·È‰È·ÙÚÈ΋ 2005;68:377-384

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31. Halliday HL. Clinical trials of postnatal corticosteroids: inhaled and systemic. Biol Neonate 1999;76 (Suppl 1):S29-S40.

32. Jonsson B, Eriksson M, Soder O, Broberger U,

¶·È‰È·ÙÚÈ΋ 2005;68:377-384

Lagercrantz H. Budesonide delivered by dosimetric jet nebulization to preterm very low birthweight infants at high risk for development of chronic lung disease. Acta Paediatr 2000;89:1449-1455.

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CURRENT ISSUE

385

The effect of postnatal administration of steroids on the developing brain H. Bouza, C. Papagaroufalis

Abstract Postnatal steroid therapy has been widely used for the prevention and treatment of chronic lung disease (bronchopulmonary dysplasia) in preterm infants. Recently, the meta-analyses of the Cochrane Collaboration suggest that the risks of therapy may outweigh the possible benefits, especially if treatment is started before 96 hours of age. The meta-analyses show that treatment at 714 days of life seems to have the best benefit to risk ratio. However, since steroids may have neurotoxic effects on the developing brain, neonatologists must continue to search for alternative therapies for infants with chronic lung disease. Meanwhile, despite the limitations of steroid treatment, the European Association of Perinatal Medicine, the American Academy of Pediatrics and the Canadian Pediatric Society guidelines provide the most realistic and balanced advice on its use for the clinical neonatologist trying to clarify a confusing situation.

2nd Neonatal Intensive Care Unit, “Aghia Sophia” Children’s Hospital, Athens Correspondence: Helen Bouza 2nd Neonatal Intensive Care Unit, “Aghia Sophia” Children’s Hospital, 115 27, Athens E-mail: bnicu@paidon-agiasofia.gr Date of submission: 21-06-2004 Date of approval: 17-06-2005

Key words Corticosteroids, neonate, brain.

Paediatriki 2005;68:385

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¶ÚÔÏËÙÈÎfi˜ ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Î·È ı¤Ì·Ù· ËıÈ΋˜ Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘

∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô “πÔÎÚ¿ÙÂÈÔ”, £ÂÛÛ·ÏÔÓ›ÎË AÏÏËÏÔÁÚ·Ê›·: Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡-§Ô˘Î›‰Ô˘ ∂ÚÁ·ÛÙ‹ÚÈÔ ∫˘ÙÙ·ÚÔÁÂÓÂÙÈ΋˜ ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£. °.¶.¡. “πÔÎÚ¿ÙÂÈÔ” £ÂÛÛ·ÏÔӛ΢ ∫ˆÓÛÙ·ÓÙÈÓÔ˘fiψ˜ 49, ∆.∫. 546 42, £ÂÛÛ·ÏÔÓ›ÎË ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 05-11-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 29-03-2005

¶ÂÚ›ÏË„Ë §·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ fiÙÈ ÁÈ· ÔÚÈṲ̂ӷ ÁÂÓÂÙÈο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ‰ÂÓ ˘¿Ú¯ÂÈ ·ÎfiÌË ÚÈ˙È΋ ıÂÚ·›· ‹ ·ÔÙÂÏÂÛÌ·ÙÈΤ˜ ÛÙÚ·ÙËÁÈΤ˜ ÚfiÏ˄˘, Ë ÂÊ·ÚÌÔÁ‹ Ù˘ Ó¤·˜ ÁÂÓÂÙÈ΋˜ Ù¯ÓÔÏÔÁ›·˜ ÁÈ· Ù· ÓÂÔÁÓÈο ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· Î·È ÙȘ ÂÍÂÙ¿ÛÂȘ ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË ÊÔÚ¤ˆÓ ·ÏÏ¿ Î·È ÚԉȿıÂÛ˘ ηٷÛÙ¿ÛÂˆÓ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ·ÚÁfiÙÂÚ· ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹, ı· Ú¤ÂÈ Ó· ÌÂÏÂÙËı› Ì ÚÔÛÔ¯‹. OÈ ·È‰›·ÙÚÔÈ ı· ¯ÚÂÈ·ÛÙ› Ó· ·ÍÈÔÏÔÁ‹ÛÔ˘Ó ÙȘ ÂÈÙÒÛÂȘ ·˘ÙÒÓ ÙˆÓ Ó¤ˆÓ Ù¯ÓÔÏÔÁÈÒÓ ÛÙËÓ ÚfiÁÓˆÛË Î·È ÙË ‰È¿ÁÓˆÛË. ∂›Û˘, ı· Ú¤ÂÈ Ó· ÂÓı·ÚÚ˘Óı› Ë ÂÚ·ÈÙ¤Úˆ ¤Ú¢ӷ Î·È ÂÎ·›‰Â˘ÛË Û ·˘Ùfi ÙÔ ·Ó·Ù˘ÛÛfiÌÂÓÔ ‰›Ô Ù˘ ÁÂÓÂÙÈ΋˜. §¤ÍÂȘ ÎÏÂȉȿ ∂ÍÂÙ¿ÛÂȘ DNA, ı¤Ì·Ù· ËıÈ΋˜, ΢ÛÙÈ΋ ›ÓˆÛË, ·ÓÔÛÔÂÓÂÚÁfi˜ ıÚ˘„›ÓË, Ì˘˚΋ ‰˘ÛÙÚÔÊ›· Duchenne.

∂ÈÛ·ÁˆÁ‹ ∏ ÚfiÛÊ·ÙË ÂÚ¿ÙˆÛË Ù˘ ·Ó¿ÁÓˆÛ˘ ÙÔ˘ ·ÓıÚÒÈÓÔ˘ ÁÔÓȉÈÒÌ·ÙÔ˜ Î·È ÔÈ Û¯ÂÙÈΤ˜ ÚfiÔ‰ÔÈ Ô˘ ÚÔ·ÙÔ˘Ó ·fi ·˘Ù‹, ·‡ÍËÛ·Ó ÙÔ ÂӉȷʤÚÔÓ ÛÙË ÁÂÓÂÙÈ΋. ¶·Ú·Ì¤ÓÂÈ, fï˜, Ì›· Û‡Á¯˘ÛË ÌÂٷ͇ ÙˆÓ ÁÈ·ÙÚÒÓ Î·È Ù˘ ÎÔÈÓˆÓ›·˜ fiÛÔÓ ·ÊÔÚ¿ ÛÙÔÓ ÚfiÏÔ Ù˘ ÁÂÓÂÙÈ΋˜ ÏËÚÔÊÔÚ›·˜ ÛÙËÓ È·ÙÚÈ΋ Ú¿ÍË (1). ™Â ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ, fiˆ˜ ÛÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË (CF) Î·È ÛÙË ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ, ÔÈ Û˘ÁÎÂÎÚÈ̤ÓÔÈ ÌÔÚÈ·ÎÔ› Ì˯·ÓÈÛÌÔ› Â›Ó·È ·ÚÎÂÙ¿ ηٷÓÔËÙÔ›. ŸÌˆ˜, Û ·ÚÎÂÙ¿ ¿ÏÏ· ¯ÚfiÓÈ· ÓÔÛ‹Ì·Ù·, fiˆ˜ Ô Û·Î¯·Ú҉˘ ‰È·‚‹Ù˘ Î·È Ë ˘¤ÚÙ·ÛË, Ù· Û¯ÂÙÈο ÁÔÓ›‰È· - Ù· ÔÔ›· Û˘¯Ó¿ Â›Ó·È ÔÏÏ¿ - ·Ú¯›˙Ô˘Ó ÙÒÚ· Ó· ·Ó·ÁÓˆÚ›˙ÔÓÙ·È. §·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ ÙȘ ‰È·ÊÔÚÂÙÈΤ˜ ÂÈÙÒÛÂȘ ÙˆÓ ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ÁÔÓȉ›ˆÓ Î·È ÙËÓ ÂÚÈÔÚÈṲ̂ÓË ÁÓÒÛË, ‰ÂÓ ·ÔÙÂÏ› ¤ÎÏËÍË ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô fiÚÔ˜ “ÁÂÓÂÙÈ΋ ÏËÚÔÊÔÚ›·” ¯ÚËÛÈÌÔÔÈÂ›Ù·È Ì ‰È·ÊÔÚÂÙÈ΋ ¤ÓÓÔÈ· Û ‰È·ÊÔÚÂÙÈΤ˜ ÂÚÈÙÒÛÂȘ. ªÂÚÈΤ˜ ÊÔÚ¤˜, ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÂÓÓÔÒÓÙ·˜ ÙËÓ Â›‰Ú·ÛË fiÏÔ˘ ÙÔ˘ ÁÔÓȉÈÒÌ·ÙÔ˜, ÂÓÒ ÈÔ Û˘¯Ó¿ ·Ó·Ê¤ÚÂÙ·È Û ·Ó·ÁÓˆÚÈṲ̂ӷ ÌÔÓÔÁÔÓȉȷο ÓÔÛ‹Ì·Ù· ‹ - ÈÔ Û˘ÁÎÂÎÚÈ̤ӷ - ÛÙ· ·ÔÙÂϤÛÌ·Ù· ÂÍÂÙ¿ÛÂˆÓ ‚·ÛÈÛÌ¤ÓˆÓ ÛÙÔ DNA (2). ∏ ÚÒÙË Ú·ÎÙÈ΋ ÂÊ·ÚÌÔÁ‹ Ù˘ ·Ó¿ÁÓˆÛ˘ ÙÔ˘ ·ÓıÚÒÈÓÔ˘ ÁÔÓȉÈÒÌ·ÙÔ˜ ı· Â›Ó·È Ë ·Ó¿Ù˘ÍË ÈÔ ·ÔÙÂÏÂÛÌ·ÙÈÎÒÓ ÛÙÚ·ÙËÁÈÎÒÓ ÁÈ· ÙËÓ ÚfiÏË„Ë ÙˆÓ ÓÔÛËÌ¿ÙˆÓ Î·È ÙË ıÂÚ·›· ÙÔ˘˜, ηıÒ˜ Î·È Ë ‰È‡ڢÓÛË ÙˆÓ ‰˘Ó·ÙÔ¶·È‰È·ÙÚÈ΋ 2005;68:386-393

Ù‹ÙˆÓ ÁÈ· ÁÂÓÂÙÈΤ˜ ‰È·ÁÓˆÛÙÈΤ˜ ÂÍÂÙ¿ÛÂȘ (tests), ÙfiÛÔ Û ·ÙÔÌÈÎfi Â›Â‰Ô fiÛÔ Î·È Û ÏËı˘ÛÌȷο ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· (3). ™ÙËÓ ÂfiÌÂÓË ‰ÂηÂÙ›· ‹ ÂÈÎÔÛ·ÂÙ›· Â›Ó·È Ôχ Èı·Ófi Ó· “ÂÈÛ‚¿ÏÏÂÈ” Ë ÚÔÏËÙÈ΋ È·ÙÚÈ΋, ÂÈÙÚ¤ÔÓÙ·˜ ÙÔÓ Î·Ù¿ÏÏËÏÔ ¤ÏÂÁ¯Ô ÙÔ˘Ï¿¯ÈÛÙÔÓ ÂȉÈÎÒÓ ˘ÔÔÌ¿‰ˆÓ ÙÔ˘ ÏËı˘ÛÌÔ‡ ÁÈ· ÁÂÓÂÙÈΤ˜ ÏËÚÔÊÔڛ˜, ÔÈ Ôԛ˜ ı· ·ԂϤÔ˘Ó Û ÂÂÌ‚¿ÛÂȘ ÛÙÔ Î¿ı ¿ÙÔÌÔ Î·È ı· ‚ÂÏÙÈÒÓÔ˘Ó ÙËÓ ˘Á›· ÙÔ˘, ÚÔÏ·Ì‚¿ÓÔÓÙ·˜ Ì›· ÓfiÛÔ. ªÂ Ù· Ó¤· ÂÈÙ‡ÁÌ·Ù· Ù˘ ÁÂÓÂÙÈ΋˜ Ù¯ÓÔÏÔÁ›·˜, ı· ·Ó·ÁÓˆÚ›˙ÔÓÙ·È ¿ÙÔÌ· ¢·ı‹ ÛÂ Û˘¯Ó¿ ÓÔÛ‹Ì·Ù·, fiˆ˜ Ë Î·Ú‰È·Î‹ ÓfiÛÔ˜ (4), Ô ‰È·‚‹Ù˘ (5), Ô Î·ÚΛÓÔ˜ (6) Î·È Ë ÓfiÛÔ˜ Alzheimer (7-8), ÒÛÙ ӷ ÍÂÎÈÓ‹ÛÂÈ Ì›· ÚÔÛ¿ıÂÈ· ÚˆÙÔ‚¿ıÌÈ·˜ (.¯. ‰›·ÈÙ· Î·È ¿ÛÎËÛË) ‹ ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ ÚfiÏ˄˘ (ÚÒÈÌË ‰È¿ÁÓˆÛË ‹ Ê·Ú̷΢ÙÈ΋ ·Ú¤Ì‚·ÛË) (2). ∞Ó Î·È Ë ÁÂÓÂÙÈ΋ ¤Ú¢ӷ ˘fiÛ¯ÂÙ·È ÔÏÏ¿ fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÚÔ·ÁˆÁ‹ Ù˘ ˘Á›·˜ ÙˆÓ ·È‰ÈÒÓ, Ë ¯Ú‹ÛË ÙˆÓ Ó¤ˆÓ ÁÂÓÂÙÈÎÒÓ tests Û ·˘Ù¿ Ú¤ÂÈ Ó· ÌÂÏÂÙËı› Ì ÚÔÛÔ¯‹. §·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ fiÙÈ ·ÎfiÌË ‰ÂÓ ˘¿Ú¯ÂÈ ÚÈ˙È΋ Î·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ıÂÚ·›· ‹ ÛÙÚ·ÙËÁÈΤ˜ ÚfiÏ˄˘, ÌÔÚ› Ó· ÌËÓ ‰ÈηÈÔÏÔÁÔ‡ÓÙ·È Ù· ÁÂÓÂÙÈο tests ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ (3,9-11). ∂ÈϤÔÓ, ÂÂȉ‹ Ù· ÌÈÎÚ¿ ·È‰È¿ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ‰È·ÎÚ›ÓÔ˘Ó ÙËÓ ·Í›· Ù˘ ÁÂÓÂÙÈ΋˜ ÏËÚÔÊÔÚ›·˜ Û¯ÂÙÈο Ì ÙË ˙ˆ‹ ÙÔ˘˜, ÔÈ ÁÔÓ›˜ Î·È ÔÈ ·È‰›·ÙÚÔÈ Ô˘ ·›ÚÓÔ˘Ó ÙËÓ ·fiÊ·ÛË ÁÈ· Ù· ÁÂÓÂÙÈο tests ÙˆÓ ·È‰ÈÒÓ ÔÊ›ÏÔ˘Ó Ó· Â›Ó·È ÚÔÛÂÎÙÈÎÔ›. ∏ ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜

Pediatr Sept-Oct 05

17-10-05

16:52

™ÂÏ›‰·387

387

¶ÚfiÏË„Ë Î·È ËıÈ΋

(∞∞¶) ÂÎÙÈÌ¿ fiÙÈ ÔÈ ·È‰›·ÙÚÔÈ Â›Ó·È ÂΛÓÔÈ Ô˘ ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó - ηχÙÂÚ· ·fi fiÏÔ˘˜ ÙÔ˘˜ ÁÈ·ÙÚÔ‡˜ - ÙÔ ·È‰› Î·È ÙÔ˘˜ ÁÔÓ›˜ ÙÔ˘ Ó· ηٷÓÔ‹ÛÔ˘Ó ÙȘ ÁÂÓÂÙÈΤ˜ ÏËÚÔÊÔڛ˜, ‰È·ÛÊ·Ï›˙ÔÓÙ·˜ ¯Â̇ıÂÈ· ÁÈ· Ù· ·ÔÙÂϤÛÌ·Ù· ÙÔ˘ test. ∂›Û˘, ÂÎÙÈÌ¿ fiÙÈ Â›Ó·È ÔÈ Î·Ù·ÏÏËÏfiÙÂÚÔÈ Ó· ‰ÒÛÔ˘Ó ÚˆÙÔ‚¿ıÌÈ· Û˘Ì‚Ô˘Ï¢ÙÈ΋ ‹ Ó· ·Ú·¤Ì„Ô˘Ó ÙÔ ·È‰› ÁÈ· ‰Â˘ÙÂÚÔ‚¿ıÌÈ· ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋, ÌfiÓÔ fiÙ·Ó ÙÔ ÁÂÓÂÙÈÎfi test Â›Ó·È ÁÈ· ÙÔ Û˘ÌʤÚÔÓ ÙÔ˘ ‹ fiÙ·Ó ÚÔ¿ÁÂÙ·È ÙÔ Û˘ÌʤÚÔÓ ÙˆÓ ÁÔÓ¤ˆÓ ‹ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜, ¯ˆÚ›˜ Ó· ÚÔηÏÂ›Ù·È ÔÔÈ·‰‹ÔÙ ÂÈ‚¿Ú˘ÓÛË ÙÔ˘ ·È‰ÈÔ‡ (3). °›ÓÂÙ·È ·Ó·ÊÔÚ¿ Û ÙÚÂȘ ÂÊ·ÚÌÔÁ¤˜ ÙˆÓ ÁÂÓÂÙÈÎÒÓ tests Î·È ÙˆÓ ·ÓȯÓ¢ÙÈÎÒÓ ÚÔÁÚ·ÌÌ¿ÙˆÓ (screening) ÛÙ· ·È‰È¿: ÛÙ· ÓÂÔÁÓÈο ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù·, ÛÙ· ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· ÊÔÚ¤ˆÓ Î·È ÛÙÔÓ ÚÔÏËÙÈÎfi ¤ÏÂÁ¯Ô ÁÈ· ÓÔÛ‹Ì·Ù· Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ·ÚÁfiÙÂÚ· ÛÙË ˙ˆ‹. ™Â fi,ÙÈ ·ÊÔÚ¿ ÛÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘˜, ˘¿Ú¯ÂÈ ÛÔ‚·Úfi˜ ÚÔ‚ÏËÌ·ÙÈÛÌfi˜ ηıÒ˜ ÚÔ·ÙÔ˘Ó ËıÈο ı¤Ì·Ù·, Á‡Úˆ ·fi Ù· ÔÔ›· ·Ó·Ù‡ÛÛÂÙ·È ÌÂÁ¿ÏË Û˘˙‹ÙËÛË.

¡ÂÔÁÓÈο ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· πÓÔ΢ÛÙÈ΋ ÓfiÛÔ˜ ∏ ÚfiÎÏËÛË ·fi ÙËÓ ÚfiÔ‰Ô ÙˆÓ Ó¤ˆÓ ÁÂÓÂÙÈÎÒÓ tests ›¯Â ʤÚÂÈ ÛÙÔ ÚÔÛ΋ÓÈÔ ÙË Û˘˙‹ÙËÛË Û¯ÂÙÈο Ì ÙËÓ Î·Ù·ÏÏËÏfiÙËÙ· ÙÔ˘ ÓÂÔÁÓÈÎÔ‡ screening ÛÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË. ∆Ô 1983, ¤Ó· ÂÚÒÙËÌ·-‰›ÏËÌÌ· ‹Ù·Ó Â¿Ó Ë ‰È¿ÁÓˆÛË Ù˘ ΢ÛÙÈ΋˜ ›ÓˆÛ˘ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô Î·È Ë ¤ÁηÈÚË ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ ηٷϋÁÂÈ Û Ì›ˆÛË Ù˘ ÓÔÛËÚfiÙËÙ·˜ Î·È Û ÂÈÌ‹Î˘ÓÛË ÙÔ˘ ¯ÚfiÓÔ˘ ˙ˆ‹˜ ÙˆÓ ·È‰ÈÒÓ (12). ∞fi ÙÔ 1989 Ô˘ Ù·˘ÙÔÔÈ‹ıËΠÙÔ ÁÔÓ›‰ÈÔ Ù˘ ΢ÛÙÈ΋˜ ›ÓˆÛ˘ (cystic fibrosis transmembrane conductance regulator - CFTR) Î·È Ì¤¯ÚÈ Û‹ÌÂÚ· ¤¯Ô˘Ó Ù·˘ÙÔÔÈËı› ¿Óˆ ·fi 900 ‰È·ÊÔÚÂÙÈΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ‰ÈÂıÓÒ˜. ∆Ô ÁÂÓÂÙÈÎfi test Ù˘ ΢ÛÙÈ΋˜ ›ÓˆÛ˘ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô ÁÈ· ÙË ‰È¿ÁÓˆÛË ÔÌÔ˙˘ÁˆÙÒÓ ·Û¯fiÓÙˆÓ ÓÂÔÁÓÒÓ ‰ÂÓ ¤¯ÂÈ ÂÚÈÏËÊı› Û η̛· ¯ÒÚ· ÛÙ· ˘Ô¯ÚˆÙÈο screening tests. ™Â ÔÚÈṲ̂ӷ ΤÓÙÚ· ‰ÈÂÓÂÚÁÂ›Ù·È ÂÚ¢ÓËÙÈο Ë Ì¤ÙÚËÛË Ù˘ ·ÓÔÛÔÂÓÂÚÁÔ‡ ıÚ˘„›Ó˘ (immunoreactive trypsin - IRT) Û ÍËÚ¿ ÛÙ·ÁfiÓ· ·›Ì·ÙÔ˜ Û οÚÙ˜ Guthrie Î·È Ë ÂÎÙ›ÌËÛË Á›ÓÂÙ·È Ì ·ÓÔÛÔÌÂÙÚ‹ÛÂȘ. ∆· fiÚÈ· ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÂÈ¤‰ˆÓ Ù˘ IRT ÂÍ·ÚÙÒÓÙ·È ·fi ÙÔ kit Î·È Ù· ¯ÚËÛÈÌÔÔÈÔ‡ÌÂÓ· ·ÓÙÈÛÒÌ·Ù· (13).

™Ù· ÓÂÔÁÓ¿ Ô˘ ·Ó¢ڛÛÎÂÙ·È ·‡ÍËÛË Ù˘ IRT Ù›ıÂÙ·È ˘Ô„›· Ù˘ ÓfiÛÔ˘. ∏ ÂȂ‚·›ˆÛ‹ Ù˘ Á›ÓÂÙ·È Ì DNA ·Ó¿Ï˘ÛË ·fi ÙÔ ›‰ÈÔ ‰Â›ÁÌ· ‹ ·fi ‰Â‡ÙÂÚË ÍËÚ¿ ÛÙ·ÁfiÓ· ·›Ì·ÙÔ˜, ·Ó¿ÏÔÁ· Ì ÙÔ ÚˆÙfiÎÔÏÏÔ. ∆Ô ÔÛÔÛÙfi ‰È¿ÁÓˆÛ˘ ÔÌÔ˙˘ÁˆÙÒÓ ·Û¯fiÓÙˆÓ ÓÂÔÁÓÒÓ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ÂıÓÈÎfiÙËÙ· ÙÔ˘ ÏËı˘ÛÌÔ‡ Ô˘ ÂÚÂ˘Ó¿Ù·È Î·È ·fi ÙÔÓ ·ÚÈıÌfi ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ Ô˘ ‰ÈÂÚ¢ÓÒÓÙ·È (14). ™Â ·˘ÍË̤ÓË IRT: ·) ŒÓ· ÓÂÔÁÓfi ‰È·ÁÈÁÓÒÛÎÂÙ·È fiÙÈ ¤¯ÂÈ Î˘ÛÙÈ΋ ›ÓˆÛË Â¿Ó ‰È·ÈÛÙˆıÔ‡Ó ‰‡Ô ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ CFTR ÁÔÓȉ›Ô˘ Î·È Â¿Ó ÙÔ test ȉÚÒÙ· ‰Â›ÍÂÈ Û˘ÁΤÓÙÚˆÛË ¯ÏˆÚÈÔ‡¯ˆÓ >60 mEq/L. ‚) ¡ÂÔÁÓfi ÛÙÔ ÔÔ›Ô ‰È·ÈÛÙÒÓÂÙ·È ÌfiÓÔ Ì›· ÌÂÙ¿ÏÏ·ÍË, ı· Ú¤ÂÈ Ó· οÓÂÈ test ȉÚÒÙ·, ÂÂȉ‹ ÌÔÚ› Ó· ¤¯ÂÈ Î·È ‰Â‡ÙÂÚË ÌÂÙ¿ÏÏ·ÍË Ë ÔÔ›· ‰ÂÓ ¤¯ÂÈ ·Ó·ÁÓˆÚÈÛÙ› ̤¯ÚÈ Û‹ÌÂÚ·. ∂¿Ó ÙÔ test ȉÚÒÙ· Â›Ó·È ·ÚÓËÙÈÎfi, ÙÔ ÓÂÔÁÓfi ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ÊÔÚ¤·˜. Á) ∂¿Ó ‰ÂÓ ‰È·ÈÛÙˆı› η̛· ÌÂÙ¿ÏÏ·ÍË, ÙfiÙ Â·Ó·Ï·Ì‚¿ÓÂÙ·È Ë IRT. ∂¿Ó ÙÔ ·ÔÙ¤ÏÂÛÌ· Â›Ó·È ·ÚÓËÙÈÎfi, ÙÔ ·È‰› ¯·Ú·ÎÙËÚ›˙ÂÙ·È Ê˘ÛÈÔÏÔÁÈÎfi (13). ™ÙÔ˘˜ ÁÔÓ›˜ Ô˘ ¤¯Ô˘Ó ‹‰Ë ¤Ó· ¿Û¯ÔÓ ·È‰› Ì ΢ÛÙÈ΋ ›ÓˆÛË, Û˘ÓÈÛÙ¿Ù·È Î·È ÚÔÛʤÚÂÙ·È ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË. OÈ ÌÂÙ·ÏÏ¿ÍÂȘ Ô˘ ‰È·ÈÛÙÒıËÎ·Ó ‹‰Ë ÛÙÔ˘˜ ÁÔÓ›˜-ÊÔÚ›˜ ·Ó·˙ËÙÔ‡ÓÙ·È Û ‰Â›ÁÌ· ¯ÔÚȷ΋˜ Ï¿¯Ó˘ ÛÙȘ 10 ‚‰ÔÌ¿‰Â˜ Ù˘ ·ËÛ˘ ‹ Û ·ÌÓÈ·Îfi ˘ÁÚfi ÛÙȘ 17 ‚‰ÔÌ¿‰Â˜ Ù˘ ·ËÛ˘. ∂¿Ó ÙÔ ¤Ì‚Ú˘Ô ¤¯ÂÈ ‰‡Ô ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ CFTR ÁÔÓȉ›Ô˘, ÔÈ ÁÔÓ›˜ Û˘Ó‹ıˆ˜ ÂÈϤÁÔ˘Ó ÙË ‰È·ÎÔ‹. ∂ÈϤÔÓ, ÙÔ test ÚԂϤÂÈ ÙÔÓ ·ÓȯÓ¢ÙÈÎfi ¤ÏÂÁ¯Ô ÛÙÔ˘˜ Û˘ÁÁÂÓ›˜ ÙÔ˘ ¿Û¯ÔÓÙÔ˜ ·È‰ÈÔ‡ Ô˘ ‰È·ÈÛÙÒıËÎÂ Ë ÌÂÙ¿ÏÏ·ÍË. ∂¿Ó ‚ÚÂı› ¤Ó·˜ Û˘ÁÁÂÓ‹˜ ÊÔÚ¤·˜, ÙfiÙÂ Î·È Ô Û‡ÓÙÚÔÊfi˜ ÙÔ˘ Ú¤ÂÈ Ó· ÂÚ¢ÓËı› ÁÈ· ÙȘ ÈÔ Û˘¯Ó¤˜ ÌÂÙ·ÏÏ¿ÍÂȘ. ∆Ô test ·¢ı‡ÓÂÙ·È Û ÓÂÔÁÓ¿ ÔÈÎÔÁÂÓÂÈÒÓ ·fi ÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi Ì ÂχıÂÚÔ ÈÛÙÔÚÈÎfi ÁÈ· ÙË ÓfiÛÔ Î·È Ì ÙÔ ·ÈÙÈÔÏÔÁÈÎfi fiÙÈ Ë ¤ÁηÈÚË ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, ηٷϋÁÂÈ Û fiÊÂÏÔ˜ ÙÔ˘ ·È‰ÈÔ‡, Ì Ì›ˆÛË Ù˘ ÓÔÛËÚfiÙËÙ·˜ Î·È ÂÈÌ‹Î˘ÓÛË ÙÔ˘ ¯ÚfiÓÔ˘ ˙ˆ‹˜ ÙÔ˘ (13). ∞Ó·‰ÚÔÌÈΤ˜ ÌÂϤÙ˜ ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÔʤÏÔ˘˜ ÙÔ˘ ·È‰ÈÔ‡ ·fi ÙËÓ ÚÒÈÌË ‰È¿ÁÓˆÛË ¤‰ÂÈÍ·Ó fiÙÈ ÙÔ ÓÂÔÁÓÈÎfi screening ÁÈ· ÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË Ô‰‹ÁËÛ ÛÙËÓ Ô˘ÛÈ·ÛÙÈ΋ Ì›ˆÛË Ù˘ ËÏÈΛ·˜ ‰È¿ÁÓˆÛ˘ (·fi 72 Û 12 ‚‰ÔÌ¿‰Â˜, Û‡Ìʈӷ Ì ÙË ÌÂϤÙË Ù˘ ÔÏÈÙ›·˜ Wisconsin), Û ̛· ÂÈÌ‹Î˘ÓÛË ÙÔ˘ ¯ÚfiÓÔ˘ Î·È Ù˘ ÔÈfiÙËÙ·˜ Ù˘ ˙ˆ‹˜ ÙˆÓ ·È‰ÈÒÓ, ηıÒ˜ ¶·È‰È·ÙÚÈ΋ 2005;68:386-393

Pediatr Sept-Oct 05

17-10-05

16:52

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388

Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘

Î·È Û Ì›ˆÛË ÙÔ˘ ¯ÚfiÓÔ˘ ·Ú·ÌÔÓ‹˜ ÙÔ˘˜ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô Î·È Ù˘ ÓÔÛËÚfiÙËÙ·˜ ÛÙ· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜. ∏ ÙÂÏÂ˘Ù·›· ·Ô‰›‰ÂÙ·È ÛÙË ‰˘Ó·ÙfiÙËÙ· ‰ÈfiÚıˆÛ˘ Ù˘ ‰›·ÈÙ·˜ Î·È ÙˆÓ ‚ÈÔ¯ËÌÈÎÒÓ ÂÏÏÂÈÌÌ¿ÙˆÓ Ô˘ ˘¿Ú¯Ô˘Ó ÛÙ· ÓÂÔÁÓ¿ Ì ΢ÛÙÈ΋ ›ÓˆÛË. ™Â ÌÂϤÙ˜ ·fi ÙÔ ›‰ÈÔ ÂÚ¢ÓËÙÈÎfi ΤÓÙÚÔ Ù˘ ÔÏÈÙ›·˜ Wisconsin, Û Â·Ó¤ÏÂÁ¯Ô Û¯ÂÙÈο Ì ÙȘ ·ÓıÚˆÔÌÂÙÚÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜, Ê¿ÓËΠfiÙÈ Ù· ÓÂÔÁÓ¿ Ô˘ ‰È·ÁÓÒÛÙËÎ·Ó Ì ÙÔ ÓÂÔÁÓÈÎfi test ηٿ ÙÔÓ ÙÔÎÂÙfi ‹Ù·Ó Û ÌÂÁ·Ï‡ÙÂÚË ÂηÙÔÛÙÈ·›· ı¤ÛË fiÛÔÓ ·ÊÔÚ¿ ÛÙÔ ‡„Ô˜, ÙÔ ‚¿ÚÔ˜ Î·È ÙËÓ ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜, Û ۇÁÎÚÈÛË Ì ٷ ·È‰È¿ Ô˘ ‰ÂÓ Â›¯·Ó ‰È·ÁÓˆÛÙ› Ì screening (13,14). ¶¤Ú·Ó Ù˘ ‚ÂÏÙ›ˆÛ˘ ÙˆÓ ·ÓıÚˆÔÌÂÙÚÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ, Ë Â›‰Ú·ÛË Ù˘ ηχÙÂÚ˘ ıÚ¤„˘ ÛÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· Ê¿ÓËΠÛÙËÓ ¤Ú¢ӷ Ù˘ ¡¤·˜ ¡fiÙÈ·˜ O˘·Ï›·˜. ∏ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· ‹Ù·Ó ÛËÌ·ÓÙÈο ηχÙÂÚË ÛÙ· ·È‰È¿ Ô˘ ›¯·Ó ‰È·ÁÓˆÛÙ› Ì screening, ·fi ÂΛÓË ÙˆÓ ·È‰ÈÒÓ ¯ˆÚ›˜ screening ÛÙËÓ ËÏÈΛ· ÙˆÓ 5 Î·È 10 ÂÙÒÓ. ∏ ‰È·Ù‹ÚËÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Â›Ó·È ıÂÌÂÏÈ҉˘ ÁÈ· ÙËÓ ÔÈfiÙËÙ· Ù˘ ˙ˆ‹˜ ÙˆÓ ·È‰ÈÒÓ Î·È Ë ÚÒÈÌË ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË Û˘ÓÙÂÏ› ÛÙËÓ Î·ı˘ÛÙ¤ÚËÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ‚Ï¿‚˘. OÈ ÌÂϤÙ˜ Ù˘ πÙ·Ï›·˜, Ù˘ °ÂÚÌ·Ó›·˜ Î·È Ù˘ ∞˘ÛÙÚ·Ï›·˜ ·Ó¤ÊÂÚ·Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÛÙËÓ ÂÈ‚›ˆÛË Î·È ÛÙËÓ ÎÏÈÓÈ΋ ¤Î‚·ÛË ÌÂٷ͇ ‚ÚÂÊÒÓ ÌÂ Î·È ¯ˆÚ›˜ screening (15-17). ∆· ÏÂÔÓÂÎÙ‹Ì·Ù· ÙÔ˘ screening ‰ÂÓ ÂÚÈÔÚ›˙ÔÓÙ·È ÛÙËÓ ÚÒÈÌË ıÂÚ·›· ÙÔ˘ ¿Û¯ÔÓÙÔ˜ ·È‰ÈÔ‡. ∏ ÚÒÈÌË ‰È¿ÁÓˆÛË Î¿ÓÂÈ ‰˘Ó·Ù‹ ÙfiÛÔ ÙË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË ÛÙÔ˘˜ Û˘ÁÁÂÓ›˜ ÙÔ˘ ·È‰ÈÔ‡ Ô˘ ÂϤÁ¯ıËΠfiÛÔ Î·È ÙËÓ ÂÈÏÔÁ‹ ÁÈ· ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË, Ì ÛÎÔfi ÙËÓ ¤ÁηÈÚË ‰È¿ÁÓˆÛË ÂÓfi˜ ÚÔۂ‚ÏË̤ÓÔ˘ ÂÌ‚Ú‡Ô˘. ∆· ‰È¿ÊÔÚ· ΤÓÙÚ· ÂÚ¢ÓÔ‡Ó ‰È·ÊÔÚÂÙÈÎfi ·ÚÈıÌfi ÌÂÙ·ÏÏ¿ÍÂˆÓ Î·È Ì ‰È·ÊÔÚÂÙÈΤ˜ ÔÚȷΤ˜ ÙÈ̤˜ Ù˘ IRT, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ÚÔ·ÙÔ˘Ó ËıÈο ı¤Ì·Ù· ·fi ·È‰È¿ Ô˘ ‰ÂÓ ¤¯Ô˘Ó ‰È·ÁÓˆÛÙ›, ·fi ¿Ù˘Ô˘˜ Û˘Ó‰˘·ÛÌÔ‡˜ ÌÂÙ·ÏÏ¿ÍÂˆÓ Î·È ·fi „¢‰Ò˜ ıÂÙÈο ·ÔÙÂϤÛÌ·Ù·. ™ÙÔ ÚfiÁÚ·ÌÌ· Ù˘ Brittany Ù˘ °·ÏÏ›·˜, ÌÂÙ¿ ÙËÓ ÂÓÙfiÈÛË Ì›·˜ ÌÂÙ¿ÏÏ·Í˘, Á›ÓÂÙ·È ‰ÈÂÍÔ‰ÈÎfi˜ ÌÔÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜. ∞˘Ù‹ Ë Ú·ÎÙÈ΋, Ë ÔÔ›· Ô‰ËÁ› ÛÙÔ Ì¤ÏÏÔÓ ÛÙËÓ Èı·Ó‹ ÂÓۈ̿وÛË ÛÙËÓ Ù¯ÓÈ΋ ÙˆÓ DNA-chips, ¤¯ÂÈ ÏÂÔÓÂÎÙ‹Ì·Ù· Î·È ÌÂÈÔÓÂÎÙ‹Ì·Ù·, Ù· ÔÔ›· ‰ÂÓ ˘¿Ú¯Ô˘Ó ¿ÓÙ· Û ¿ÏÏ· ÚÔÁÚ¿ÌÌ·Ù· (13). ∏ ÂÎÙÂÓ‹˜ ·Ó¿Ï˘ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ CFTR Ô‰ËÁ› ÛÙË ‰È·›ÛÙˆÛË Û¿ÓÈˆÓ ÌÂÙ·ÏϿ͈Ó, ¶·È‰È·ÙÚÈ΋ 2005;68:386-393

ÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ ÌÂÚÈÎÒÓ Ó¤ˆÓ, Û ÌÂÚÈο ·È‰È¿ Ô˘ ¤¯Ô˘Ó Ì›· ÔÚȷ΋ Û˘ÁΤÓÙÚˆÛË ¯ÏˆÚÈÔ‡¯ˆÓ ȉÚÒÙ· Î·È ÁÈ· Ù· ÔÔ›· Ë ÎÏÈÓÈ΋ ¤Î‚·ÛË ·Ú·Ì¤ÓÂÈ ·ÎfiÌË ‰‡ÛÎÔÏÔ Ó· ÚÔ‚ÏÂÊı›. ŒÙÛÈ, ‰È·ÈÛÙÒÓÔÓÙ·È ·È‰È¿ Ì ‰‡Ô ÌÂÙ·ÏÏ¿ÍÂȘ CFTR Î·È Ì ¿Ù˘Ô ÁÔÓfiÙ˘Ô, Ô˘ Â›Ó·È ÂχıÂÚ· Û˘Ìو̿وÓ. ¢ÂÓ Â›Ó·È ÚÔ˜ ÙÔ ·ÚfiÓ ‰˘Ó·ÙfiÓ Ó· ·ÔÎÏÂÈÛÙ› ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ·˘ÙÔ› ÔÈ ÁÔÓfiÙ˘ÔÈ ··ÓÙÒÓÙ·È Û ‹È· ÌÔÚÊ‹ Ù˘ ΢ÛÙÈ΋˜ ›ÓˆÛ˘, Ë ÔÔ›· ı· ·Ó·Ù˘¯ı› ·ÚÁfiÙÂÚ· ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹. ∏ ·‚¤‚·ÈË ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘ ÌÔÚ› Ó· Â›Ó·È ÂÛÙ›· ¿Á¯Ô˘˜ ÁÈ· ÌÂÚÈο ˙¢Á¿ÚÈ·, Ù· ÔÔ›· ˙Ô˘Ó Ì ÙËÓ ·ÁˆÓ›· Ù˘ ÂΉ‹ÏˆÛ˘ Ù˘ ÓfiÛÔ˘, ηıÈÛÙÒÓÙ·˜ ÙË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË ˘¤ÚÙ·Ù˘ ÛËÌ·Û›·˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·˘ÙÒÓ ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ (18). ∂› ÙÔ˘ ·ÚfiÓÙÔ˜, Ë ÚÒÈÌË ‰È¿ÁÓˆÛË ÌÂÙ·ÏÏ¿ÍÂˆÓ ÙÔ˘ CFTR ηÈ, ÂÔ̤ӈ˜, ÂÓfi˜ ·È‰ÈÔ‡ Ì ÁÔÓfiÙ˘Ô Î˘ÛÙÈ΋˜ ›ÓˆÛ˘ ·ÏÏ¿ ÂχıÂÚÔ˘ Û˘Ìو̿وÓ, ÂÈÙÚ¤ÂÈ ÛÙ· ˙¢Á¿ÚÈ· Ó· ÚÔÁÚ·ÌÌ·Ù›˙Ô˘Ó ÙËÓ ·Ó··Ú·ÁˆÁ‹ Ù˘ ÔÈÎÔÁ¤ÓÂÈ¿˜ ÙÔ˘˜, ÌÂÙ¿ ·fi Ôχ ηϋ ÏËÚÔÊfiÚËÛË. ∞˘Ùfi Ô‰ËÁ› ÌÂÚÈο ˙¢Á¿ÚÈ· ÛÙË ‰ÈÂÓ¤ÚÁÂÈ· ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Û ÂfiÌÂÓË Î‡ËÛË, ÚÈÓ ·ÎfiÌË ÙÔ ·È‰› ÙÔ˘˜ Ô˘ ÂϤÁ¯ıËΠ̠screening test - ·ÚÔ˘ÛÈ¿ÛÂÈ Î¿ÔÈ· Û˘ÌÙÒÌ·Ù· Ù˘ ÓfiÛÔ˘. ∆¤ÏÔ˜, ÙÔ ÓÂÔÁÓÈÎfi screening ÂÈÙÚ¤ÂÈ Ó· Á›ÓÂÙ·È ¤ÏÂÁ¯Ô˜ ÛÙÔ˘˜ Û˘ÁÁÂÓ›˜ ÙÔ˘ ·È‰ÈÔ‡ Ô˘ ÂÍÂÙ¿ÛÙËÎÂ, ÒÛÙ ӷ ˆÊÂÏËıÔ‡Ó ·fi ÙËÓ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ‰ÂÓ ı· ÁÓÒÚÈ˙·Ó Ì ¿ÏÏÔ ÙÚfiÔ fiÙÈ ˘¿Ú¯ÂÈ Ô Î›Ó‰˘ÓÔ˜ Ó· ·ÔÎÙ‹ÛÔ˘Ó ·È‰› Ì ΢ÛÙÈ΋ ›ÓˆÛË (13). øÛÙfiÛÔ, ¤Ó· ·fi Ù· ÌÂÈÔÓÂÎÙ‹Ì·Ù· Ù˘ ÛÙÚ·ÙËÁÈ΋˜ Ù˘ ÂÎÙÂٷ̤Ó˘ ÁÔÓȉȷ΋˜ ¤Ú¢ӷ˜ Â›Ó·È Ë ‰È·›ÛÙˆÛË ÂÙÂÚÔ˙˘ÁˆÙÒÓ ÌÂٷ͇ ÙˆÓ ÂÏÂÁÌ¤ÓˆÓ ÓÂÔÁÓÒÓ. ∞˘Ù‹ Ë ÏËÚÔÊÔÚ›· ‰ËÌÈÔ˘ÚÁ› ÚÔ‚Ï‹Ì·Ù· ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ·, ‰ÈfiÙÈ ÔÈ ÁÔÓ›˜ ÌÔÚ› Ó· ·Ú·ÓÔ‹ÛÔ˘Ó ÙËÓ ¤ÓÓÔÈ· ÙÔ˘ Ó· Â›Ó·È Î¿ÔÈÔ˜ “ÂÙÂÚfi˙˘ÁÔ˜” (‰ËÏ·‰‹ ÊÔÚ¤·˜) Î·È ÙÔ˘ Ó· Â›Ó·È “¿Û¯ˆÓ”. ∂›Û˘, ÌÂÙ¿ ·fi ·˘Ù‹ ÙË ‰È·›ÛÙˆÛË ·ÎÔÏÔ˘ıÔ‡Ó screening ηٿÏÏËÏ· ÁÈ· ÙË Û˘ÁÎÂÎÚÈ̤ÓË ÔÈÎÔÁ¤ÓÂÈ·, ÁÂÁÔÓfi˜ Ô˘ ÚÔηÏ› ¿Á¯Ô˜ ÛÙ· ÂÏÂÁ¯fiÌÂÓ· ̤ÏË (15). ∏ ·ÎÚ›‚ÂÈ· ÙÔ˘ ÓÂÔÁÓÈÎÔ‡ screening ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ÂÈÏÔÁ‹ ÙˆÓ Î·ÙÒÙÂÚˆÓ ÙÈÌÒÓ Ù˘ ̤ÙÚËÛ˘ Ù˘ IRT. ∏ ·‡ÍËÛË ·˘ÙÔ‡ ÙÔ˘ ÔÚ›Ô˘ ¤¯ÂÈ Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙË Ì›ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ ·Ú·¤ÌÔÓÙ·È ÁÈ· DNA ·Ó¿Ï˘ÛË Î·È, ÂÔ̤ӈ˜, ÙË Ì›ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ‰È·ÁÓˆÛÌ¤ÓˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ. ªÔÚ›, ÏÔÈfiÓ, Ì ·˘Ùfi ÙÔ Â›Â‰Ô Ù˘ ̤ÙÚËÛ˘ Ó·

Pediatr Sept-Oct 05

17-10-05

16:52

™ÂÏ›‰·389

389

¶ÚfiÏË„Ë Î·È ËıÈ΋

ÌË ‰È·ÁÓˆÛÙÔ‡Ó ‹ Ó· ¯·ıÔ‡Ó ·È‰È¿, Ù· ÔÔ›· ¤¯Ô˘Ó ‰‡Ô ‹Ș ÌÔÚʤ˜ CFTR ÌÂÙ·ÏÏ¿ÍÂˆÓ Î·È Ô˘ ¤¯Ô˘Ó ÙËÓ Èı·ÓfiÙËÙ· Ó· ·Ó·Ù‡ÍÔ˘Ó ÙË ÓfiÛÔ ·ÚÁfiÙÂÚ· Û·Ó ¤ÊË‚ÔÈ ‹ Û·Ó ÂÓ‹ÏÈΘ (13). ∂ÈϤÔÓ, Ë ‰È¿ÁÓˆÛË Ù˘ ΢ÛÙÈ΋˜ ›ÓˆÛ˘ Û ·˘Ù¿ Ù· ·È‰È¿ ÌÔÚ› Ó· Â›Ó·È ·ÎfiÌË ÈÔ ‰‡ÛÎÔÏÔ Ó· Á›ÓÂÈ ÛÙÔ Ì¤ÏÏÔÓ, ÂÂȉ‹ ·˘Ù¿ ·Ó‹ÎÔ˘Ó ÛÙË ÁÂÓÈ¿ ÙˆÓ “screened children” Î·È oÈ ·È‰›·ÙÚÔÈ ÌÔÚ› ·ÚÁfiÙÂÚ· ÛÙË ˙ˆ‹ ÙÔ˘˜ Ó· ·ÔÚÚ›ÙÔ˘Ó ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘. ∞Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÔÈ ÔÈÎÔÁ¤ÓÂȘ ÙˆÓ ·È‰ÈÒÓ Ô˘ ¤¯Ô˘Ó „¢‰Ò˜ ıÂÙÈο ·ÔÙÂϤÛÌ·Ù· Ù˘ IRT ÌÔÚ› Ó· ˘Ê›ÛÙ·ÓÙ·È „˘¯ÔÏÔÁÈÎfi ÙÚ·‡Ì· Î·È Ó· ‰È·Î·Ù¤¯ÔÓÙ·È ·fi ·ÈÛı‹Ì·Ù· ¿Á¯Ô˘˜ (9-13). ∂ÈϤÔÓ, ¤Ó· ÌÈÎÚfi ÔÛÔÛÙfi ÙˆÓ ÁÔÓ¤ˆÓ ÌÔÚ› Ó· ¤¯ÂÈ Ì›· ÌfiÓÈÌË ·Ú·ÓfiËÛË fiÙÈ ÙÔ ·È‰› ÙÔ˘˜ Â›Ó·È Û ΛӉ˘ÓÔ Ó· ·Ó·Ù‡ÍÂÈ Î˘ÛÙÈ΋ ›ÓˆÛË ÌÂÙ¿ ·fi ¤Ó· „¢‰Ò˜ ıÂÙÈÎfi ·ÔÙ¤ÏÂÛÌ· ÓÂÔÁÓÈÎÔ‡ screening, ÂÓÒ Ù· „¢‰Ò˜ ıÂÙÈο ·ÔÙÂϤÛÌ·Ù· ÌÔÚ› Ó· ÂËÚ¿ÛÔ˘Ó ÙȘ ÂÈÏÔÁ¤˜ ·Ó··Ú·ÁˆÁ‹˜ ÙˆÓ ÁÔÓ¤ˆÓ (16). ŸÏ˜ ÔÈ ÚÔ·Ó·ÊÂÚı›Û˜ ηٷÛÙ¿ÛÂȘ ÂÍËÁÔ‡Ó ÙÔÓ ÏfiÁÔ ÁÈ· ÙÔÓ ÔÔ›Ô ÙÔ ÓÂÔÁÓÈÎfi screening ÁÈ· ÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË Â›Ó·È ·ÓÙÈΛÌÂÓÔ Û˘˙‹ÙËÛ˘.

¶ÚÔ‚Ï‹Ì·Ù· ·ÎÔ‹˜ ŒÓ· ¿ÏÏÔ ÓÂÔÁÓÈÎfi test ÛÙԯ‡ÂÈ ÛÙË ¤ÁηÈÚË ‰È¿ÁÓˆÛË ‚Ï·‚ÒÓ Ù˘ ·ÎÔ‹˜. ∏ ·ÎÔ˘ÔÌÂÙÚ›· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ˆ˜ ÓÂÔÁÓÈÎfi screening ÁÈ· ·˘Ù¤˜ ÙȘ ‚Ï¿‚˜. OÈ ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ÎÔÓÂ͛Ӣ 26 (connexin 26) ¢ı‡ÓÔÓÙ·È ÁÈ· ÙÔ 40% fiÏˆÓ ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ù˘ ·ÒÏÂÈ·˜ ·ÎÔ‹˜ ÛÙË Û¯ÔÏÈ΋ ËÏÈΛ·, ÌÂ Û˘¯ÓfiÙËÙ· ÙˆÓ ÊÔÚ¤ˆÓ 3% (19-21). ∆Ô test ¤¯ÂÈ ‰‡Ô ÛÙ¿‰È·: ÙËÓ ·ÎÔ˘ÔÌÂÙÚ›·, ·Ú¯Èο, ÙËÓ ÔÔ›· ·ÎÔÏÔ˘ı› DNA test ÁÈ· ¤ÏÂÁ¯Ô ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ÎÔÓÂ͛Ӣ 26. ∏ ÚÒÈÌË ‰È¿ÁÓˆÛË ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· Ù˘ ÂÈıÂÙÈ΋˜ ·Ú¤Ì‚·Û˘ ÁÈ· ÙË ‚ÂÏÙ›ˆÛË ÙˆÓ ‰ÂÍÈÔÙ‹ÙˆÓ ÙÔ˘ ÏfiÁÔ˘ ÂÓfi˜ ·È‰ÈÔ‡, ÙËÓ ÂÌʇÙ¢ÛË Îԯϛ· ‹ Î·È ÙˆÓ ‰‡Ô. ŒÓ· ÌfiÓÈÌÔ ËıÈÎfi ı¤Ì· ÛÙ· ÓÂÔÁÓÈο ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· Â›Ó·È Â¿Ó Ù· screening ı· Ú¤ÂÈ Ó· Â›Ó·È ÂıÂÏÔÓÙÈο ‹ ˘Ô¯ÚˆÙÈο. ª›· ÂıÂÏÔÓÙÈ΋ ÚÔÛ¤ÁÁÈÛË ··ÈÙ› ÙÂÏÈ΋ ·fiÊ·ÛË ·fi ÙÔ˘˜ ÁÔÓ›˜ ÌÂÙ¿ ·fi ÏËÚÔÊfiÚËÛË, ÂÓÒ Ë ˘Ô¯ÚˆÙÈ΋ ÚÔÛ¤ÁÁÈÛË ·Ó·ÌÊ›‚ÔÏ· ÙÔ˘˜ ˘Ô‚¿ÏÏÂÈ Û ¿ÚÓËÛË. ∏ ΢ÚÈfiÙÂÚË ËıÈ΋ ‰ÈηÈÔÏÔÁ›· Ô˘ ÚÔ‚¿ÏÏÂÙ·È ÁÈ· ÙÔÓ ˘Ô¯ÚˆÙÈÎfi ¤ÏÂÁ¯Ô Â›Ó·È ÙÔ ·Í›ˆÌ· fiÙÈ Î·ı‹ÎÔÓ Ù˘ ÎÔÈÓˆÓ›·˜ Â›Ó·È Ó· ÚÔ¿ÁÂÈ ÙËÓ ÔÈfiÙËÙ· Ù˘ ˙ˆ‹˜ ÙÔ˘ ·È‰ÈÔ‡

̤ۈ Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ Î·È ıÂÚ·›·˜ ÔÚÈÛÌ¤ÓˆÓ Î·Ù·ÛÙ¿ÛˆÓ, ˘ÔηıÈÛÙÒÓÙ·˜ ÙÔ ÁÔÓÂ˚Îfi ‰Èη›ˆÌ· ÁÈ· ¿ÚÓËÛË (3). ŒÓ· ·ÓÙ›ıÂÙÔ Âȯ›ÚËÌ· ˘ÔÛÙËÚ›˙ÂÈ fiÙÈ ·Ú·‰ÔÛȷο ÔÈ ÁÔÓ›˜ ¤¯Ô˘Ó ÙËÓ ·ÚÌÔ‰ÈfiÙËÙ· Ó· ÂÈϤÁÔ˘Ó ÙÔ˘˜ ¯ÂÈÚÈÛÌÔ‡˜ Û¯ÂÙÈο Ì ÙËÓ ˘Á›· ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘˜. ∞Ó Î·È ÔÈ ÁÔÓ›˜ ‰ÂÓ ¤¯Ô˘Ó ÙÔ ‰Èη›ˆÌ· Ó· ·ÚÓÔ‡ÓÙ·È Ì›· ·ÔÙÂÏÂÛÌ·ÙÈ΋ ıÂÚ·›· ÁÈ· ηٷÛÙ¿ÛÂȘ ·ÂÈÏËÙÈΤ˜ ÁÈ· ÙË ˙ˆ‹ ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘˜, ¤¯Ô˘Ó ˆÛÙfiÛÔ, ÙÔ ‰Èη›ˆÌ· Ó· ÂÈϤÁÔ˘Ó ÌÂٷ͇ ‰È·ÊfiÚˆÓ ÚÔÔÙÈÎÒÓ ÁÈ· ηٷÛÙ¿ÛÂȘ ÏÈÁfiÙÂÚÔ ·ÂÈÏËÙÈΤ˜ ÁÈ· ÙË ˙ˆ‹ ÙÔ˘. ªÂÚÈΤ˜ ÊÔÚ¤˜, Ì¿ÏÈÛÙ·, οÓÔ˘Ó Ù¤ÙÔȘ ÂÈÏÔÁ¤˜, ÙȘ Ôԛ˜ ÌÂÚÈÎÔ› Â·ÁÁÂÏ̷ٛ˜ ˘Á›·˜ ı· ıˆÚÔ‡Û·Ó ·Ú¿ÏÔÁ˜.

∞ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· ÊÔÚ¤ˆÓ ∏ ÚfiÔ‰Ô˜ Ù˘ È·ÙÚÈ΋˜ Ù¯ÓÔÏÔÁ›·˜ ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó·ÁÓÒÚÈÛË ·ÙfiÌˆÓ Ô˘ Â›Ó·È ÊÔÚ›˜ ÌÂÙ·ÏÏ¿ÍÂˆÓ Û ÁÔÓ›‰È· ‰È·ÊfiÚˆÓ ÓÔÛËÌ¿ÙˆÓ, fiˆ˜ Ù˘ ÓfiÛÔ˘ Tay-Sachs, Ù˘ Ì˘˚΋˜ ‰˘ÛÙÚÔÊ›·˜, Ù˘ ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋˜ ·Ó·ÈÌ›·˜ ‹ Ù˘ Ì›˙ÔÓÔ˜ ı·Ï·ÛÛ·ÈÌ›·˜. ∏ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· Duchenne Â›Ó·È Ô ÈÔ Û˘¯Ófi˜ Ù‡Ô˜ Ì˘˚΋˜ ‰˘ÛÙÚÔÊ›·˜ ÛÙ· ·È‰È¿. ∫ÏËÚÔÓÔÌÂ›Ù·È Ì ¤Ó·Ó Ê˘ÏÔÛ‡Ó‰ÂÙÔ ÙÚfiÔ Î·È - Ì ÌÂÚÈΤ˜ ÂÍ·ÈÚ¤ÛÂȘ - ÚÔÛ‚¿ÏÏÔÓÙ·È ÌfiÓÔ ·ÁfiÚÈ·. ∆Ô ÁÔÓ›‰ÈÔ Duchenne-Becker Â›Ó·È ÁÓˆÛÙfi fiÙÈ Â›Ó·È ¤Ó· ·fi Ù· ÌÂÁ·Ï‡ÙÂÚ· ÁÔÓ›‰È· ÙÔ˘ ·ÓıÚÒÔ˘ Î·È ÂÓÙÔ›˙ÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ à (Ãp21) (12,22,23). ∞˘Ùfi ÙÔ ÁÔÓ›‰ÈÔ Îˆ‰ÈÎÔÔÈ› ÙËÓ ÚˆÙ½ÓË ‰˘ÛÙÚÔÊ›ÓË, Ë ÔÔ›· ¤¯ÂÈ ¤Ó·Ó ÚfiÏÔ-ÎÏÂȉ› ÛÙËÓ ·ÎÂÚ·ÈfiÙËÙ· Ù˘ ‰ÔÌ‹˜ ÙˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ. ™ÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ ¤¯ÂÈ ·Ú·ÙËÚËı› ÌÂÁ¿ÏË ÔÈÎÈÏ›· ÌÂÙ·ÏϿ͈Ó, Ì Ï‹ÚË ‹ ÌÂÚÈ΋ ·ÒÏÂÈ· ÙÔ˘ ÁÔÓȉ›Ô˘. ŒÓ·˜ ·Ú·ÏÏ·Á̤ÓÔ˜ Ù‡Ô˜ Â›Ó·È Ë Ì˘˚΋ ‰˘ÛÙÚÔÊ›· Becker (BMD), Ë ÔÔ›· Â›Ó·È ÚÔԉ¢ÙÈ΋ ÓfiÛÔ˜ Ì ÌÂÁ·Ï‡ÙÂÚË ÂÈ‚›ˆÛË. ¢ÂÓ ˘¿Ú¯ÂÈ ÚÈ˙È΋ ıÂÚ·›· ÁÈ· ÙËÓ DMD, Ë ÔÔ›· ¤¯ÂÈ ÚÔԉ¢ÙÈ΋ Âȉ›ӈÛË, ÂÓÒ Ô Î·ÓfiÓ·˜ Â›Ó·È Ô ÚÒÈÌÔ˜ ı¿Ó·ÙÔ˜ ÛÙË 2Ë-3Ë ‰ÂηÂÙ›· Ù˘ ˙ˆ‹˜ (23). ™‹ÌÂÚ·, Û ÔÚÈṲ̂Ó˜ ¯ÒÚ˜ ÂÊ·ÚÌfi˙ÂÙ·È ÂıÂÏÔÓÙÈÎfi˜ ÓÂÔÁÓÈÎfi˜ ·ÓȯÓ¢ÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì DNA ·Ó¿Ï˘ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ÓfiÛÔ˘, fiÔ˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÍËÚ¿ ÛÙ·ÁfiÓ· ·›Ì·ÙÔ˜ Û ÚÔÛÚÔÊËÙÈÎfi ¯·ÚÙ›. ™ÙÔ ÂıÂÏÔÓÙÈÎfi ÚfiÁÚ·ÌÌ· ÛÙË °ÂÚÌ·Ó›· ÂÍÂÙ¿˙ÔÓÙ·È ‚Ú¤ÊË ËÏÈΛ·˜ ÌÂٷ͇ 4 ‚‰ÔÌ¿‰ˆÓ Î·È ÂÓfi˜ ¤ÙÔ˘˜. ∂›Û˘, ÓÂÔÁÓ¿ ÂϤÁ¯ÔÓÙ·È Ì ÈÏÔÙÈο ÚÔÁÚ¿ÌÌ·Ù· ¶·È‰È·ÙÚÈ΋ 2005;68:386-393

Pediatr Sept-Oct 05

17-10-05

16:52

™ÂÏ›‰·390

390

Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘

ÛÙË °·ÏÏ›· (Lyon), ÛÙÔ µ¤ÏÁÈÔ, ÛÙÔÓ ∫·Ó·‰¿ Î·È ÛÙË ªÂÁ¿ÏË µÚÂÙ·Ó›·.

O ¤ÏÂÁ¯Ô˜ ÛÙËÚ›˙ÂÙ·È, ·Ú¯Èο, ÛÙÔÓ Î·ıÔÚÈÛÌfi Ù˘ ÎÚ·ٛÓ˘ ÎÈÓ¿Û˘ (CK), Ì ÂÎÙ›ÌËÛË ÙÔ˘ ÊıÔÚÈÛÌÔ‡ Û ÍËÚ¿ ÛÙ·ÁfiÓ· ·›Ì·ÙÔ˜ ÙÔ˘ ÓÂÔÁÓÔ‡. º˘ÛÈÔÏÔÁÈο ‰Â›ÁÌ·Ù· ‰ÂÓ ÊıÔÚ›˙Ô˘Ó, ÂÓÒ ‰Â›ÁÌ·Ù· Ì Â›‰· CK >500 U/L ÂÌÊ·Ó›˙Ô˘Ó ÊıÔÚÈÛÌfi. ∞ÎÔÏÔ˘ı› ÂÎÙ›ÌËÛË Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ CK ÛÙÔÓ ÔÚfi Ì ·ÓÔÛÔÂÓ˙˘ÌÈ΋ ̤ıÔ‰Ô. ™Ù· ·ıÔÏÔÁÈο ‰Â›ÁÌ·Ù· ·ÎÔÏÔ˘ı› ÌÔÚȷ΋ ‰ÈÂÚ‡ÓËÛË ÁÈ· ÙËÓ ·Ó·˙‹ÙËÛË ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ (ÂÏÏ›„ˆÓ) ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ‰˘ÛÙÚÔÊ›Ó˘ Ì ÙËÓ Ù¯ÓÔÏÔÁ›· Ù˘ ·Ó¿Ï˘Û˘ ÙÔ˘ DNA. ∞ÚÓËÙÈο Â˘Ú‹Ì·Ù· ·Ú·ÙËÚÔ‡ÓÙ·È ÂÚ›Ô˘ ÛÙÔ 30% ÙˆÓ ·ÛıÂÓÒÓ Ì DMD, ÂÂȉ‹ οÔȘ ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ‰˘ÛÙÚÔÊ›Ó˘ ‰ÂÓ ·ÓȯÓ‡ÔÓÙ·È Ì¤¯ÚÈ Û‹ÌÂÚ· Ì ÙËÓ ·Ó¿Ï˘ÛË ÙÔ˘ DNA (23). ™Â ÂÚÈÙÒÛÂȘ ·ÛıÂÓÒÓ ¯ˆÚ›˜ ·Ó·ÁÓˆÚ›ÛÈ̘ ÌÂÙ·ÏÏ¿ÍÂȘ, ·ÎÔÏÔ˘ı› ‚ÈÔ„›· Ì˘ÒÓ ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ηٿÛÙ·Û˘ Ù˘ ‰˘ÛÙÚÔÊ›Ó˘. ∏ ‚ÈÔ„›· Û˘Ì‚¿ÏÏÂÈ ÛÙËÓ Ù·˘ÙÔÔ›ËÛË Ù˘ ÓfiÛÔ˘ ηÈ, ÂÈϤÔÓ, ηıÔÚ›˙ÂÈ ÙËÓ ÈÔ ‚·ÚÈ¿ ÌÔÚÊ‹ DMD ·fi ÙËÓ BMD, fiÙ·Ó Ë DNA ÌÂÙ¿ÏÏ·ÍË ‰ÂÓ Â›Ó·È ÏËÚÔÊÔÚȷ΋. ∆Ô fiÊÂÏÔ˜ ÙÔ˘ ·ÛıÂÓÔ‡˜, Ù˘ ÔÈÎÔÁ¤ÓÂÈ¿˜ ÙÔ˘ Î·È Ù˘ ÎÔÈÓˆÓ›·˜ ·Ú·Ì¤ÓÂÈ ¤Ó· ·ÌÊÈÏÂÁfiÌÂÓÔ ı¤Ì·, Ï·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ ÙËÓ ·Ô˘Û›· - ̤¯ÚÈ Û‹ÌÂÚ· - Ì›·˜ ·ÔÙÂÏÂÛÌ·ÙÈ΋˜ ıÂÚ·›·˜. Œ¯Ô˘Ó ÚÔ·„ÂÈ ÚÔ‚Ï‹Ì·Ù· Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙËÓ „˘¯ÔÏÔÁÈ΋ Î·È ÂÓ‰ÔÔÈÎÔÁÂÓÂȷ΋ ÂÈ‚¿Ú˘ÓÛË Û ÂÚÈÙÒÛÂȘ ÚÔÛ˘Ìو̷ÙÈÎÒÓ ‰È·ÁÓÒÛÂˆÓ (ÔÛÔÛÙfi ÂÚ›Ô˘ 40% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û˘Ì‚·›ÓÂÈ de novo, ‰ËÏ·‰‹ Û ÔÈÎÔÁ¤ÓÂȘ ¯ˆÚ›˜ ÚÔËÁÔ‡ÌÂÓ· ÚÔۂ‚ÏË̤ӷ ¿ÙÔÌ·). ∞fi ÙËÓ ¿ÏÏË ÏÂ˘Ú¿, fï˜, Ë ‰È·ıÂÛÈÌfiÙËÙ· Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ ÌÔÚ› Ó· ÂÈÙÚ¤„ÂÈ ÛÙȘ ÔÈÎÔÁ¤ÓÂȘ Ó· οÓÔ˘Ó Ú·ÎÙÈΤ˜ ÂÈÏÔÁ¤˜ Û¯ÂÙÈο Ì ÙË ˙ˆ‹ ÂÓfi˜ ·È‰ÈÔ‡ Ì ÂȉÈΤ˜ ·Ó¿ÁΘ Î·È Ó· ·ÔʇÁÔ˘Ó Ì›· ·Ú·ÙÂٷ̤ÓË “‰È·ÁÓˆÛÙÈ΋ Ô‰‡ÛÛÂÈ·”. ªÔÚ›, ·ÎfiÌË, ÁÈ· ÌÂÚÈΤ˜ ÔÈÎÔÁ¤ÓÂȘ Ó· Â›Ó·È ˆÊ¤ÏÈÌË Ë ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË Î·È Ô ¤ÏÂÁ¯Ô˜ ÌÂÏÒÓ Ù˘ Ô˘ ‚Ú›ÛÎÔÓÙ·È Û ΛӉ˘ÓÔ (22,23).

OÈ ÂÚ¢ÓËÙ¤˜, Ì ‚¿ÛË ÙËÓ ÂÌÂÈÚ›· ÙÔ˘˜, ÚÔÙ›ÓÔ˘Ó Ó· Â›Ó·È ÚÔ·ÈÚÂÙÈ΋ Ë ¯Ú‹ÛË Ù˘ ‰È·ÁÓˆÛÙÈ΋˜ ÁÂÓÂÙÈ΋˜ ·Ó¿Ï˘Û˘. ¶·Ú¿ÏÏËÏ·, ¯ÚÂÈ¿˙ÂÙ·È Ó· ˘¿Ú¯ÂÈ ¤Ó· ·˘ÛÙËÚfi ÚˆÙfiÎÔÏÏÔ ÁÈ· ÙËÓ ·ÚÔ¯‹ ˘ÔÛÙ‹ÚÈ͢ ÛÙÔ˘˜ ÁÔÓ›˜ Î·È ÛÙÔ ¿Û¯ÔÓ ·È‰›, ÙÔ ÔÔ›Ô ÂÈÙÚ¤ÂÈ ÙË ‰È·ÛÊ¿ÏÈÛË ÂÈÎÔÈÓˆÓ›·˜ Î·È Û˘ÓÂÚÁ·Û›·˜ ÌÂٷ͇ ÚˆÙÔ‚¿ıÌÈ·˜, ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ Î·È ¶·È‰È·ÙÚÈ΋ 2005;68:386-393

ÙÚÈÙÔ‚¿ıÌÈ·˜ ˘ËÚÂÛ›·˜. ∂ȉÈÎfiÙÂÚ·, Â›Ó·È ··Ú·›ÙËÙË Ë ÔÏÔÎÏËڈ̤ÓË ˘ËÚÂÛ›· Û˘Ì‚Ô˘Ï¢ÙÈ΋˜, Ë ·È‰È·ÙÚÈ΋ ˘ËÚÂÛ›· ÛÙ‹ÚÈ͢ Î·È ÂȉÈο ÂÎ·È‰Â˘Ì¤Ó· ¿ÙÔÌ· ÁÈ· ÙÔÓ Û˘ÓÙÔÓÈÛÌfi ÙÔ˘ ÓÂÔÁÓÈÎÔ‡ ·ÓȯÓ¢ÙÈÎÔ‡ ÂϤÁ¯Ô˘. ª¤¯ÚÈ Û‹ÌÂÚ·, Ô ¤ÏÂÁ¯Ô˜ ÊÔÚ¤ˆÓ ‹ Ô ÁÂÓÈÎfi˜ ÏËı˘ÛÌÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ‰ÂÓ ¤¯Ô˘Ó ÂÊ·ÚÌÔÛÙ› Û ÌÂÁ¿ÏÔ ‚·ıÌfi ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ÛÙȘ ∏.¶.∞. £ÂˆÚËÙÈο, Ë ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ¤Ï¤Á¯Ô˘ ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ÛÂÍÔ˘·ÏÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÙˆÓ ·È‰ÈÒÓ, ı· ·‡Í·Ó ÙȘ ÂÈÏÔÁ¤˜ ·Ó··Ú·ÁˆÁ‹˜ Û ÂΛÓÔ˘˜ Ô˘ ı· ‰È·ÈÛÙÒÓÔÓÙ·Ó ˆ˜ ÊÔÚ›˜, Û ۇÁÎÚÈÛË Ì ÂΛÓÔ˘˜ Ô˘ ı· ˘Ô‚ÏËıÔ‡Ó ÛÙÔÓ ¤ÏÂÁ¯Ô ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ·ËÛ˘. øÛÙfiÛÔ, Ù· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ Ô˘ ÁÓˆÚ›˙Ô˘Ó fiÙÈ Â›Ó·È ÊÔÚ›˜, ÌÔÚ› Ó· Â›Ó·È „˘¯ÔÏÔÁÈο ÈÔ Â˘¿ÏˆÙÔÈ Û ۇÁÎÚÈÛË Ì ÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜. ¶·Ú·Ì¤ÓÂÈ ‰Â ·ÌÊ›‚ÔÏÔ Â¿Ó Ô ¤ÏÂÁ¯Ô˜ Û Ó·ÚfiÙÂÚ˜ ËÏÈ˘ ı· ÂÈʤÚÂÈ ÛÙÔ Ì¤ÏÏÔÓ ·ÏÏ·Á¤˜ ÛÙË Û˘ÌÂÚÈÊÔÚ¿ Ù˘ ·Ó··Ú·ÁˆÁ‹˜ ÙÔ˘˜ (3). ∏ ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ ‰ÂÓ ˘ÔÛÙËÚ›˙ÂÈ ÙËÓ Â˘Ú›· ¯Ú‹ÛË ÙˆÓ ·ÓȯÓ¢ÙÈÎÒÓ ÂϤÁ¯ˆÓ ÊÔÚ¤ˆÓ ÛÙ· ·È‰È¿ ‹ ÙÔ˘˜ ÂÊ‹‚Ô˘˜, ·ÏÏ¿ Û˘ÓÈÛÙ¿ Ó· ‰ÈÂÓÂÚÁÔ‡ÓÙ·È ÛÙȘ ¤ÁÎ˘Â˜ ÂÊ‹‚Ô˘˜ ‹ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ Ô˘ ۯ‰ȿ˙Ô˘Ó Ì›· ·ËÛË Î·È ÔÈ ÔÔ›ÔÈ ¤¯Ô˘Ó Ï‹Úˆ˜ ÏËÚÔÊÔÚËı› ÁÈ· Ù· ÔʤÏË Î·È ÙÔ˘˜ ÎÈÓ‰‡ÓÔ˘˜ Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙÔÓ ¤ÏÂÁ¯Ô.

¶ÚÔÏËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· ÓÔÛ‹Ì·Ù· Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ·ÚÁfiÙÂÚ· ÛÙË ˙ˆ‹ ∏ ÁÂÓÂÙÈ΋ Ù¯ÓÔÏÔÁ›· ‰È·ÛÊ·Ï›˙ÂÈ Ù· ̤۷ ÁÈ· Ó· ‰È·ÁÓˆÛÙÔ‡Ó ÓÔÛ‹Ì·Ù· Ù· ÔÔ›· ÂΉËÏÒÓÔÓÙ·È ÌÂÙ¿ ÙË ‚ÚÂÊÈ΋ ËÏÈΛ· ‹ ÌfiÓÔ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹. ¶·Ú·‰Â›ÁÌ·Ù· ÓÔÛËÌ¿ÙˆÓ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹, Ì ˘„ËÏfi ‚·ıÌfi ÚÔ‚Ï„ÈÌfiÙËÙ·˜ Ì ·Ó¿Ï˘ÛË DNA, ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙË Ì˘ÔÙÔÓÈ΋ ‰˘ÛÙÚÔÊ›·, ÙËÓ ·ÈÌԯڈ̿وÛË, ÙÔ˘˜ ÔÏ˘Î˘ÛÙÈÎÔ‡˜ ÓÂÊÚÔ‡˜, ÙË ÓfiÛÔ Huntington Î·È ÌÂÚÈο ›‰Ë ηÚΛÓÔ˘ (2). ∂ÈϤÔÓ, Û‹ÌÂÚ· Â›Ó·È ÂÊÈÎÙfi Ó· ÂÓÙÔÈÛÙÔ‡Ó ÁÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜, ÔÈ ÔÔ›ÔÈ ·˘Í¿ÓÔ˘Ó ÙËÓ Èı·ÓfiÙËÙ· ÂΉ‹ÏˆÛ˘ Û˘¯ÓÒÓ ÓÔÛËÌ¿ÙˆÓ, fiˆ˜ Ë ÓfiÛÔ˜ ÙˆÓ ÛÙÂÊ·ÓÈ·›ˆÓ ·ÚÙËÚÈÒÓ, Ô ‰È·‚‹Ù˘, Ù· ÂÁÎÂÊ·ÏÈο ÂÂÈÛfi‰È·, ÔÈ ·ÁÁÂȷΤ˜ ıÚÔÌ‚ÒÛÂȘ ·fi ıÚÔÌ‚ÔÊÈÏ›· (ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÊÔÚ›·˜ ÙÔ˘ ›‰ÈÔ˘ ·ÓÙ›ÁÚ·ÊÔ˘ ÙÔ˘ ÁÔÓȉ›Ô˘ V Leiden) (4), Ë ˘¤ÚÙ·ÛË (2,3), Ë ÓfiÛÔ˜ Alzheimer (8), ÔÚÈṲ̂ÓÔÈ Ù‡ÔÈ Î·ÚΛÓÔ˘ ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘ (6) ηÈ

Pediatr Sept-Oct 05

17-10-05

16:52

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391

¶ÚfiÏË„Ë Î·È ËıÈ΋

ÙÔ˘ Ì·ÛÙÔ‡ (6), ÌÂÚÈΤ˜ „˘¯È·ÙÚÈΤ˜ ηٷÛÙ¿ÛÂȘ Î·È Î¿ÔÈ· ÚÂ˘Ì·ÙÈο ÓÔÛ‹Ì·Ù· (3). ™·Ó ·Ú¿‰ÂÈÁÌ· ·Ó·Ê¤ÚÂÙ·È Ë ÓfiÛÔ˜ Huntington Ô˘ ÌÂÙ·‚È‚¿˙ÂÙ·È Ì ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú·. ∂¿Ó ˘¿Ú¯ÂÈ Ë ÌÂÙ¿ÏÏ·ÍË Ù˘ ÓfiÛÔ˘, Ô Î›Ó‰˘ÓÔ˜ ÂΉ‹ÏˆÛ˘ ÁÈ· ÙË ÓfiÛÔ Â›Ó·È 100% Û ̛· ÂÈ‚›ˆÛË Ê˘ÛÈÔÏÔÁÈ΋˜ ‰È¿ÚÎÂÈ·˜. ª¤¯ÚÈ Û‹ÌÂÚ· ‰ÂÓ ˘¿Ú¯ÂÈ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ıÂÚ·›·, ·ÏÏ¿ Ô‡ÙÂ Î·È ÚÔÏËÙÈ΋. ∏ ·fiÊ·ÛË ÁÈ· ÙÔÓ ÚÔÎÏÈÓÈÎfi ¤ÏÂÁ¯Ô Â›Ó·È Î·ı·Ú¿ ÚÔÛˆÈ΋ Î·È Ù· ·ÔÙÂϤÛÌ·Ù¿ ÙÔ˘ ¤¯Ô˘Ó Èı·ÓfiÙËÙ· Ó· ÛÙÈÁÌ·Ù›ÛÔ˘Ó ‹ Ó· ηٷ‚¿ÏÏÔ˘Ó „˘¯ÔÏÔÁÈο ÙÔ ¿ÙÔÌÔ. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, Ë ÚÔÛÂÎÙÈ΋ ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË Â›Ó·È ··Ú·›ÙËÙË. ¢ÂηÂÙ‹˜ ÂÌÂÈÚ›· ·fi ÙË ª. µÚÂÙ·Ó›· ‰Â›¯ÓÂÈ fiÙÈ ÌfiÓÔ 20% ÂÚ›Ô˘ ÙˆÓ ·ÙfiÌˆÓ Û ΛӉ˘ÓÔ ÁÈ· ÓfiÛÔ Huntington ˙ËÙÔ‡Ó Ó· ÂÏÂÁ¯ıÔ‡Ó (23). °È· οÔȘ ·fi ·˘Ù¤˜ ÙȘ ηٷÛÙ¿ÛÂȘ, Ë ÁÓÒÛË ÙÔ˘ ÎÈÓ‰‡ÓÔ˘ ÌÔÚ› Ó· ‚ÔËı‹ÛÂÈ Ù· ¿ÙÔÌ· Ó· ÌÂÈÒÛÔ˘Ó ÙË ÓÔÛËÚfiÙËÙ· ‹ ÙÔÓ Î›Ó‰˘ÓÔ Ù˘ ıÓËÛÈÌfiÙËÙ·˜. ∂ÈϤÔÓ, ̤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ Û ΛӉ˘ÓÔ ÌÔÚ› Ó· ˆÊÂÏËıÔ‡Ó „˘¯ÔÏÔÁÈο ›Ù ̷ı·›ÓÔÓÙ·˜ fiÙÈ ‰ÂÓ Â›Ó·È ÊÔÚ›˜ Ù˘ ÌÂÙ¿ÏÏ·Í˘, ›Ù - ÂÂȉ‹ ı· ·‡ÛÂÈ Ë ·ÌÊÈ‚ÔÏ›· fiÙÈ ¤¯Ô˘Ó Èı·ÓÒ˜ ÙË ÓfiÛÔ - ·Ó ‰È·ÈÛÙˆı› fiÙÈ Â›Ó·È ÌfiÓÔ ÊÔÚ›˜. °È· ÔÏϤ˜ ηٷÛÙ¿ÛÂȘ, fï˜, ‰ÂÓ ¤¯ÂÈ ·Ô‰Âȯı› Ë Ì›ˆÛË Ù˘ ÓÔÛËÚfiÙËÙ·˜ ‹ Ù˘ ıÓËÛÈÌfiÙËÙ·˜ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÂÓfi˜ ÁÂÓÂÙÈÎÔ‡ ÂϤÁ¯Ô˘ Î·È ·Ú·Ì¤ÓÂÈ ·Û·Ê¤˜ Â¿Ó ÔÈ ·ÚÔ‡Û˜ ÚÔÙ¿ÛÂȘ ÁÈ· ÙËÓ ÚfiÏË„Ë ‹ ÙËÓ ÚÒÈÌË ‰È¿ÁÓˆÛË ı· Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈΤ˜ Û ·˘ÙfiÓ ÙÔÓ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÏËı˘ÛÌfi (3). ∂ÈϤÔÓ, Ë ÁÓˆÛÙÔÔ›ËÛË ÙÔ˘ ·˘ÍË̤ÓÔ˘ ÎÈÓ‰‡ÓÔ˘ ÌÔÚ› Ó· ÂÓÂÚÁÔÔÈ› Ì›· ·ÓÙ›ıÂÙË „˘¯ÔÏÔÁÈ΋ ·¿ÓÙËÛË Î·È ÂӉ¯Ô̤ӈ˜ ÎÔÈÓˆÓÈΤ˜ ‰È·ÎÚ›ÛÂȘ. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, Ô ÁÚ‹ÁÔÚÔ˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ÙˆÓ ÁÔÓȉ›ˆÓ BRCA 1/BRCA 2 (Ù· ÔÔ›· Úԉȷı¤ÙÔ˘Ó Û ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ηÚΛÓÔ ÙÔ˘ Ì·ÛÙÔ‡ Î·È ÙˆÓ ˆÔıËÎÒÓ) Î·È ÙÔ˘ ÁÔÓȉ›Ô˘ HNPCC (‹ ÁÔÓȉ›Ô˘ ÙÔ˘ ÌË ÔÏ˘ԉȷÛÈÎÔ‡ ÎÏËÚÔÓÔÌÈÎÔ‡ ηÚΛÓÔ˘ ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘) ÛÙËÓ ÎÏÈÓÈ΋ È·ÙÚÈ΋ ÙˆÓ ÂÓËÏ›ÎˆÓ ‹Ù·Ó ·Ôı·ÚÚ˘ÓÙÈÎfi˜ (6). ∏ ÔÏ˘ÏÔÎfiÙËÙ· ÙˆÓ ÁÂÓÂÙÈÎÒÓ tests Î·È Ë ·‚‚·ÈfiÙËÙ· ÁÈ· ÙÔ˘˜ ÎÈÓ‰‡ÓÔ˘˜ Î·È Ù· ÔʤÏË ·fi Ù· ·ÔÙÂϤÛÌ·Ù·, ÂȂ‚·ÈÒÓÔ˘Ó ÙËÓ ·Ó¿ÁÎË ‰ÈÂÍÔ‰È΋˜ ÁÂÓÂÙÈ΋˜ ηıÔ‰‹ÁËÛ˘ ÁÈ· ÙÔÓ ÚÔÏËÙÈÎfi ÁÂÓÂÙÈÎfi ¤ÏÂÁ¯Ô, fiÛÔÓ ·ÊÔÚ¿ ÛÙ· ÓÔÛ‹Ì·Ù· Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ·. ¶ÔÏÏÔ› ÂÓ‹ÏÈΘ ÂÈϤÁÔ˘Ó Ó· ÌËÓ ÂÏÂÁ¯ıÔ‡Ó, ÁÂÁÔÓfi˜ Ô˘ ‰Â›¯ÓÂÈ fiÙÈ ‰ÂÓ ÌÔÚ› Ó· ıˆÚÂ›Ù·È ‰Â‰Ô̤ÓÔ

fiÙÈ Ù· ·È‰È¿ ı· ‹ıÂÏ·Ó Ó· ÂÏÂÁ¯ıÔ‡Ó ‹ ı· ˆÊÂÏÔ‡ÓÙ·Ó (3). ¶ÚfiÛÊ·Ù·, ‰‡Ô ÂÈÙÚÔ¤˜ ·fi ÙËÓ ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ ¤¯Ô˘Ó ¿ÚÂÈ ı¤ÛË ÁÈ· ÙÔÓ ÁÂÓÂÙÈÎfi ¤ÏÂÁ¯Ô ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ÁÈ· Ù· ÓÔÛ‹Ì·Ù· Ô˘ ÂΉËÏÒÓÔÓÙ·È ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (9). ∏ ÂÈÙÚÔ‹ Ù˘ °ÂÓÂÙÈ΋˜ Û˘ÛÙ‹ÓÂÈ fiÙÈ ¿ÙÔÌ· ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 18 ÂÙÒÓ Ú¤ÂÈ Ó· ÂϤÁ¯ÔÓÙ·È ÁÈ· Ù· ÓÔÛ‹Ì·Ù· ·˘Ù¿ ÌfiÓÔ Â¿Ó Ô ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÚÔÛʤÚÂÈ ¿ÌÂÛ· È·ÙÚÈο ÔʤÏË ‹ Â¿Ó ¤Ó· ¿ÏÏÔ Ì¤ÏÔ˜ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ˆÊÂÏËı›, ÂÓÒ ‰ÂÓ ÚԂϤÂÙ·È ‚Ï¿‚Ë ÁÈ· ÙÔ ¿ÙÔÌÔ Ô˘ ÂϤÁ¯ÂÙ·È. ∏ ÂÈÙÚÔ‹ ıˆÚ› ‚·ÛÈÎfi ̤ÚÔ˜ Ù˘ ‰È·‰Èηۛ·˜ ÙË ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋ ÚÈÓ Î·È ÌÂÙ¿ ÙËÓ ÂͤٷÛË (3). ∏ ÂÈÙÚÔ‹ Ù˘ µÈÔËıÈ΋˜ ‰ÂÓ ˘ÔÛÙËÚ›˙ÂÈ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ÁÂÓÂÙÈÎÔ‡ ÂϤÁ¯Ô˘ ÊÔÚ¤ˆÓ ÁÈ· ÓÔÛ‹Ì·Ù· Ô˘ ÂΉËÏÒÓÔÓÙ·È ·ÚÁfiÙÂÚ· ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹, ÂÎÙfi˜ ·fi ÙËÓ ÂÚ›ÙˆÛË Ô˘ Ì›· ¤ÊË‚Ë Â›Ó·È ¤ÁÎ˘Ô˜ ‹ ۯ‰ȿ˙ÂÈ Ì›· ÂÁ΢ÌÔÛ‡ÓË, ÂÓÒ Â›Ó·È ·ÓÙ›ıÂÙË Ì ÙÔÓ ÚÔÏËÙÈÎfi ¤ÏÂÁ¯Ô Û ¿ÙÔÌ· ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 18 ÂÙÒÓ (3). ∆¤ÏÔ˜, Ë ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ÁÂÓÂÙÈÎÔ‡ ÂϤÁ¯Ô˘ ÌÔÚ› Ó· ‰ÒÛÂÈ ·ÚÔÛ‰fiÎËÙ˜ ÏËÚÔÊÔڛ˜ Û¯ÂÙÈο Ì ÙËÓ ·ÙÚfiÙËÙ·. ∏ ∞ÌÂÚÈηÓÈ΋ ∂Ù·ÈÚ›· ∞ÓıÚÒÈÓ˘ °ÂÓÂÙÈ΋˜ ¤¯ÂÈ ÙËÓ ¿Ô„Ë fiÙÈ Ì¤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ÏËÚÔÊÔÚÔ‡ÓÙ·È ÁÈ· ÙËÓ ·ÙÚfiÙËÙ·, ·Ú¿ ÌfiÓÔ Â¿Ó Ô Î·ıÔÚÈÛÌfi˜ Ù˘ ·ÙÚfiÙËÙ·˜ ‹Ù·Ó Ô ·ÔÎÏÂÈÛÙÈÎfi˜ ÛÎÔfi˜ ÙÔ˘ ÂϤÁ¯Ô˘ (5). ∂Í¿ÏÏÔ˘, ÛÙË ‰È·‰Èηۛ· ÙÔ˘ ÁÂÓÂÙÈÎÔ‡ ÂϤÁ¯Ô˘ ÁÈ· Ì›· ÓfiÛÔ, ÌÔÚ› Ó· ·ÔÎ·Ï˘Êı› ÏËÚÔÊÔÚ›· Û¯ÂÙÈο Ì ̛· ¿ÏÏË ÓfiÛÔ. ∏ ‰È¿ÁÓˆÛË Ù˘ ‰Â‡ÙÂÚ˘ ÓfiÛÔ˘ ÌÔÚ› Ó· Â›Ó·È ·Úfi‚ÏÂÙË Î·È Ó· Ô‰ËÁ‹ÛÂÈ Û ÔÈÎÔÁÂÓÂȷο Î·È ÎÔÈÓˆÓÈο ÚÔ‚Ï‹Ì·Ù·, ηıÒ˜ Î·È ÛÙÈÁÌ·ÙÈÛÌfi. ™ÙË Û˘ÁηٿıÂÛË ı· Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÙ·È ˘ÂÓı‡ÌÈÛË Û¯ÂÙÈο ÌÂ Ù˘¯fiÓ ·ÚÔÛ‰fiÎËÙ· Â˘Ú‹Ì·Ù· (5).

¶ÚÔÙ¿ÛÂȘ Ù˘ ∞ÌÂÚÈηÓÈ΋˜ ∞η‰ËÌ›·˜ ¶·È‰È·ÙÚÈ΋˜ ·) O ÁÂÓÂÙÈÎfi˜ ‰È·ÁÓˆÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜, fiˆ˜ ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ‰È·ÁÓˆÛÙÈΤ˜ ‹ ıÂÚ·¢ÙÈΤ˜ ·ÚÂÌ‚¿ÛÂȘ ÁÈ· Ù· ·È‰È¿, ··ÈÙ› Û˘ÁηٿıÂÛË ·fi ÙÔ˘˜ ÏËÚÔÊÔÚË̤ÓÔ˘˜ ÁÔÓ›˜ Î·È Û˘Ó·›ÓÂÛË ·fi Ù· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿. ‚) ¢ÂÓ ˘ÔÛÙËÚ›˙ÂÙ·È Ë Â˘Ú›· ¯Ú‹ÛË ÙÔ˘ ÂϤÁ¯Ô˘ ‹ Ô ·ÓȯÓ¢ÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÊÔÚ¤ˆÓ ÁÈ· ¶·È‰È·ÙÚÈ΋ 2005;68:386-393

Pediatr Sept-Oct 05

17-10-05

16:52

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Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘

·È‰È¿ Î·È ÂÊ‹‚Ô˘˜, ηıÒ˜ ¯ÚÂÈ¿˙ÂÙ·È ÂÈϤÔÓ ¤Ú¢ӷ. OÈ Î›Ó‰˘ÓÔÈ Î·È Ù· ÔʤÏË ÙÔ˘ ÂϤÁ¯Ô˘ ÊÔÚ¤ˆÓ ÛÙÔÓ ·È‰È·ÙÚÈÎfi ÏËı˘ÛÌfi ÚÈÓ ‰ÔıÔ‡Ó Û ¢Ú›· Îϛ̷η, ı· Ú¤ÂÈ Ó· ·ÍÈÔÏÔÁËıÔ‡Ó ÚÔÛÂÎÙÈο Ì ÂÏÂÁ¯fiÌÂÓ˜ ÎÏÈÓÈΤ˜ ‰ÔÎÈ̤˜. ∆· ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· ÂϤÁ¯Ô˘ ÊÔÚ¤ˆÓ ÌÔÚ› Ó· Â›Ó·È Î·Ù¿ÏÏËÏ· ÁÈ· ¤ÁÎ˘Â˜ ÂÊ‹‚Ô˘˜ ‹ ÁÈ· ÂΛӘ Ô˘ ÛΤÊÙÔÓÙ·È ÙËÓ ÙÂÎÓÔÔ›ËÛË. Á) O ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· ÓÔÛ‹Ì·Ù· Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ·ÚÁfiÙÂÚ· ÛÙË ˙ˆ‹, ÁÂÓÈο, ı· Ú¤ÂÈ Ó· ·Ó·‚ÏËı› ̤¯ÚÈ ÙËÓ ÂÓËÏÈΛˆÛË ‹ ̤¯ÚȘ fiÙÔ˘ Ô ¤ÊË‚Ô˜ Ô˘ ÂӉȷʤÚÂÙ·È ı· ¤¯ÂÈ ·Ó·Ù‡ÍÂÈ ÙËÓ ˆÚÈÌfiÙËÙ· Ó· ¿ÚÂÈ ÙËÓ ÙÂÏÈ΋ ·fiÊ·ÛË. £ÂˆÚÂ›Ù·È fiÙÈ Ô ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ ÁÈ· ÙËÓ ÚfiÏË„Ë ÙˆÓ ÓÔÛËÌ¿ÙˆÓ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ·ÚÁfiÙÂÚ· Â›Ó·È ·Î·Ù¿ÏÏËÏÔ˜, ·ÊÔ‡ Ë ÁÂÓÂÙÈ΋ ÏËÚÔÊÔÚ›· ÛÙËÓ ·Ú¯‹ Ù˘ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ ‰ÂÓ ¤‰ÂÈÍ fiÙÈ ÂÏ·ÙÙÒÓÂÈ ÙË ÓÔÛËÚfiÙËÙ· Î·È ÙË ıÓËÛÈÌfiÙËÙ· Ì ÙËÓ ·Ú¤Ì‚·ÛË. ‰) ∂Âȉ‹ Ù· ÁÂÓÂÙÈο ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· ÌÔÚ› Ó· ÌËÓ Á›ÓÔÓÙ·È Î·Ù·ÓÔËÙ¿, ÔÈ ·È‰›·ÙÚÔÈ ¯ÚÂÈ¿˙ÂÙ·È Ó· ‰›ÓÔ˘Ó ÛÙÔ˘˜ ÁÔÓ›˜ ÌfiÓÔ ÙËÓ ·Ó·Áη›· ÏËÚÔÊfiÚËÛË Û¯ÂÙÈο Ì ÙȘ ÂÚÈÔÚÈṲ̂Ó˜ ÁÂÓÂÙÈΤ˜ ÁÓÒÛÂȘ Î·È ıÂÚ·¢ÙÈΤ˜ ‰˘Ó·ÙfiÙËÙ˜ ÁÈ· ¤Ó·Ó ÌÂÁ¿ÏÔ ·ÚÈıÌfi ÁÂÓÂÙÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ. ∂›Û˘, ˘¿Ú¯ÂÈ Èı·ÓfiÙËÙ· ÂÈ‚¿Ú˘ÓÛ˘, Ë ÔÔ›· ÌÔÚ› Ó· ÚÔÎÏËı› ·fi ÙËÓ ÂÍÂȉÈÎÂ˘Ì¤ÓË ÁÂÓÂÙÈ΋ ÏËÚÔÊÔÚ›·, ÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Ù˘ Èı·ÓfiÙËÙ·˜ „˘¯ÔÏÔÁÈ΋˜ ‚Ï¿‚˘ Î·È ÛÙÈÁÌ·ÙÈÛÌÔ‡. OÈ ·È‰›·ÙÚÔÈ ÌÔÚ› Ó· ‚ÔËıÔ‡Ó ÛÙÔÓ ¯ÂÈÚÈÛÌfi ÔÏÏÒÓ ıÂÌ¿ÙˆÓ Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙÔÓ ÁÂÓÂÙÈÎfi ¤ÏÂÁ¯Ô, ÛÂ Û˘ÓÂÚÁ·Û›· Ì ÙÔ˘˜ ÁÂÓÂÙÈÛÙ¤˜, ÙÔ˘˜ ÁÂÓÂÙÈÛÙ¤˜ Û˘Ì‚Ô‡ÏÔ˘˜ Î·È fiÏÔ˘˜ fiÛÔ˘˜ ·Ú¤¯Ô˘Ó ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË. Â) ∏ ∞∞¶ Û˘ÓËÁÔÚ› ÛÙË ‰È‡ڢÓÛË ÙˆÓ ÂÎ·È‰Â˘ÙÈÎÒÓ Â˘Î·ÈÚÈÒÓ ÛÙË ÁÂÓÂÙÈ΋ ÙÔ˘ ·ÓıÚÒÔ˘ ÁÈ· ÙÔ˘˜ ÊÔÈÙËÙ¤˜ Ù˘ È·ÙÚÈ΋˜, ÙÔ˘˜ ÂȉÈ΢fiÌÂÓÔ˘˜ Î·È ÙÔ˘˜ Ú·ÎÙÈÎÔ‡˜ ÁÈ·ÙÚÔ‡˜ Î·È ÛÙË ‰È‡ڢÓÛË ÙˆÓ ÚÔÁÚ·ÌÌ¿ÙˆÓ ÂÈÌfiÚʈÛ˘ ÁÈ· ÙÔ˘˜ Â·ÁÁÂÏ̷ٛ˜ ÁÂÓÂÙÈÛÙ¤˜ (11).

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20. Cohn ES, Kelley PM. Clinical phenotype and mutations in connexin 26 (DFNB1/GJB2), the most common cause of childhood hearing loss. Am J Med Genet 1999;89:130-136.

21. Morell RJ, Kim HJ, Hood LJ, Goforth L, Friderici K, Fisher R et al. Mutations in the connexin 26 gene (GJB2) among Ashkenazi Jews with nonsyndromic

recessive deafness. N Engl J Med 1998;339:1500-1505.

22. Parsons EP, Clarke AJ, Hood K, Lycett E, Bradley DM. Newborn screening for Duchenne muscular dystrophy: a psychosocial study. Arch Dis Child Fetal Neonatal Ed 2002;86:F91-F95.

23. Burke W. Genetic testing. N Engl J Med 2002;347: 1867-1875.

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SPECIAL ARTICLE

Genetic preventive testing and ethical issues H. Hatzissevastou - Loukidou

1st Paediatric Clinic, Aristotle University of Thessaloniki, “Ippokration” General Hospital, Thessaloniki Correspondence: H. Hatzissevastou-Loukidou Laboratory of Cytogenetics, 1st Paediatric Clinic, Aristotle University of Thessaloniki, “Ippokration” General Hospital, Thessaloniki 49 Konstantinoupoleos Str., 546 42, Thessaloniki Date of submission: 05-11-2004 Date of approval: 29-03-2005

Paediatriki 2005;68:394

Abstract In the absence of clearly beneficial treatments or effective preventive strategies for many childhood diseases, the use of new genetic technology for newborn screening, carrier testing and testing for susceptibility to late-onset conditions must be considered carefully. Paediatricians will need to evaluate critically the diagnostic and prognostic implications of testing. Additional research and education in this developing area of medicine are encouraged.

Key words DNA tests, ethical issues, cystic fibrosis, immunoreactive trypsinogen, Duchenne muscular dystrophy.

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LETTER TO THE EDITOR

395

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µ‘ ¶·È‰È·ÙÚÈ΋ ¶·ÓÂÈÛÙËÌȷ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”, ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: ¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜ E-mail: ngp@allergy.gr

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¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜, ¶·È‰›·ÙÚÔ˜ - ∞ÏÏÂÚÁÈÔÏfiÁÔ˜

ª·ı·›ÓÔÓÙ·˜ ÙÔÓ ÎfiÛÌÔ ÙˆÓ ¡¤ˆÓ ∆¯ÓÔÏÔÁÈÒÓ - http://www.uptoten.com ª›· ÔÌ¿‰· ·fi ÂÓıÔ˘ÛÈÒ‰ÂȘ Â·ÁÁÂÏ̷ٛ˜ Ì ¤‰Ú· ÙÚ›· ÛËÌ›· Ù˘ Á˘ (∏¶∞, °·ÏÏ›· Î·È ª·˘Ú›ÎÈÔ) ÎÚ‡‚ÂÙ·È ›Ûˆ ·fi ÙÔÓ ‰ÈÎÙ˘·Îfi ÙfiÔ Up∆oTen, Ì ·ÓÙÈΛÌÂÓÔ ÙËÓ ÂÎÌ¿ıËÛË Î·È ÙËÓ „˘¯·ÁˆÁ›· ÙˆÓ ·È‰ÈÒÓ ËÏÈΛ·˜ ̤¯ÚÈ 10 ÂÙÒÓ. ¢È·ÎÚ›ÓÂÙ·È Û 2 ‚·ÛÈΤ˜ ηÙËÁÔڛ˜, “°È· °ÔÓ›˜” Î·È “°È· ¶·È‰È¿”, Ì ‰È·ÊÔÚÂÙÈÎfi ÂÚȯfiÌÂÓÔ ·Ó¿ÏÔÁ· Ì ÙÔ ÎÔÈÓfi ÛÙÔ ÔÔ›Ô ·¢ı‡ÓÂÙ·È. ∏ ÈÛÙÔÛÂÏ›‰· “°È· °ÔÓ›˜” ÚÔÛʤÚÂÈ Î˘Ú›ˆ˜ Ô‰ËÁ›Â˜ Î·È Û˘Ì‚Ô˘Ï¤˜ ÁÈ· ÙËÓ Î·Ï‡ÙÂÚË ‰È··È‰·ÁÒÁËÛË ÙˆÓ ·È‰ÈÒÓ. ∏ ÈÛÙÔÛÂÏ›‰· ¤¯ÂÈ Î·Ù¿ ‚¿ÛË ÏËÚÔÊÔÚÈ·Îfi ¯·Ú·ÎÙ‹Ú· Ì ‰È·ÏÂÎÙÈÎfi ÙÚfiÔ Î·È Ú·ÎÙÈο ˙ËÙ‹Ì·Ù· ÂÁÁÚ·Ê‹˜. ∏ ηÙËÁÔÚ›· “°È· ¶·È‰È¿” ·ÔÙÂÏ› Î·È ÙÔ ÎÂÓÙÚÈÎfi ÛÙÔÈ¯Â›Ô ÙÔ˘ Up∆oTen. ¢‡Ô ‚·ÛÈÎÔ› ¯·Ú·ÎÙ‹Ú˜, Ô Boowa - Ô ÔÔ›Ô˜ ·ÓÙÈÚÔÛˆ‡ÂÈ ÙÔÓ ÂÓ‹ÏÈÎÔ Ì ˘‡ı˘ÓË Î·È ÒÚÈÌË Û˘ÌÂÚÈÊÔÚ¿ - Î·È Ë ÌÈÎÚ‹ Kwala - Ë ÔÔ›· Â›Ó·È ÈÔ ˙ˆËÚ‹ Î·È ÂÈÚÚÂ‹˜ ÛÙȘ ÛηӉ·ÏȤ˜ - ÚˆÙ·ÁˆÓÈÛÙÔ‡Ó Û fiϘ ÙȘ ˘ÔηÙËÁÔڛ˜, ÔÈ Ôԛ˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‰È·‰ÈÎÙ˘·Î¿ ·È¯Ó›‰È·, ËÏÂÎÙÚÔÓÈÎfi ηٿÛÙËÌ· Ì ·È¯Ó›‰È·, ÚÔ‡¯·, ‚È‚Ï›·, ·Ê›Û˜ Î.¿. O ÙÔ̤·˜ ÙˆÓ ·È¯ÓȉÈÒÓ Â›Ó·È È‰È·›ÙÂÚ· ·ÓÂÙ˘Á̤ÓÔ˜ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ ÂÚÈÛÛfiÙÂÚ· ·fi 700 ÂÎ·È‰Â˘ÙÈο ·È¯Ó›‰È· Ì ‰ˆÚÂ¿Ó ÚfiÛ‚·ÛË, ηıÒ˜ Î·È ÚÔÙ¿ÛÂȘ ÁÈ· ‰Ú·ÛÙËÚÈfiÙËÙ˜, Ù· ÔÔ›· ·¢ı‡ÓÔÓÙ·È Û ‰È¿ÊÔÚÔ˘˜ ËÏÈÎÈ·ÎÔ‡˜ Û˘Ó‰˘·ÛÌÔ‡˜, ·Ó¿ÏÔÁ· Ì ÙȘ ··ÈÙ‹ÛÂȘ ÙÔ˘ οı ·È¯ÓȉÈÔ‡. ∏ ÏÔ‹ÁËÛË ÛÙÔÓ ÈÛÙfiÙÔÔ “°È· ¶·È‰È¿” Â›Ó·È ·Ï‹ Î·È ‰È·ÛΉ·ÛÙÈ΋, Ì ̛· ‡¯ÚËÛÙË ªË¯·Ó‹ ∞Ó·˙‹ÙËÛ˘ ÛÙȘ ÂÈ̤ÚÔ˘˜ ÂÓfiÙËÙ˜.

O ‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ Up∆oTen ¤¯ÂÈ ÌÂÁ¿ÏË ÂÈÛ΄ÈÌfiÙËÙ· (¿Óˆ ·fi 2 ÂηÙÔÌ̇ÚÈ· ¯Ú‹ÛÙ˜ ÌËÓÈ·›ˆ˜) Î·È Û˘ÛÙ‹ÓÂÙ·È ·fi ‰ÈÂıÓ›˜ ÂÎ·È‰Â˘ÙÈÎÔ‡˜ ÔÚÁ·ÓÈÛÌÔ‡˜ Î·È ·Ó·ÁÓˆÚÈṲ̂Ó˜ ÂÙ·ÈÚ›˜ ‰È·‰ÈÎÙ˘ÎÒÓ ÂÊ·ÚÌÔÁÒÓ.

Multimedia games ÁÈ· fiÏ· Ù· ÁÔ‡ÛÙ·! - http://www.shockwave.com O ‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ Shockwave.com ·ÔÙÂÏ› ¤Ó·Ó ·fi ÙÔ˘˜ ÚˆÙÔfiÚÔ˘˜ ÛÙÔÓ ÙÔ̤· ÙˆÓ ËÏÂÎÙÚÔÓÈÎÒÓ ·È¯ÓȉÈÒÓ (multimedia games) ̤ۈ ÙÔ˘ ‰È·‰ÈÎÙ‡Ô˘. ∞Ó‹ÎÂÈ ÛÙÔÓ ·ÌÂÚÈηÓÈÎfi fiÌÈÏÔ AtomShockwave Corp, Ì ̤ÛË ÌËÓÈ·›· ÂÈÛ΄ÈÌfiÙËÙ· ¿Óˆ ·fi 20 ÂηÙÔÌ̇ÚÈ· ¯Ú‹ÛÙ˜, ·ÔÙÂÏÒÓÙ·˜ ·ÁÎfiÛÌÈÔ ÛËÌÂ›Ô ·Ó·ÊÔÚ¿˜ ÁÈ· ÙÔÓ ¯ÒÚÔ Ù˘ „ËÊȷ΋˜ „˘¯·ÁˆÁ›·˜ Î·È ‰È·ÛΤ‰·Û˘. ∞¢ı‡ÓÂÙ·È Û ·È‰È¿ Î·È Ó¤Ô˘˜ οı ËÏÈΛ·˜ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ Ì›· ¢Ú›· Áο̷ ·È¯ÓȉÈÒÓ, Ù· ÔÔ›· ·Ó·ÓÂÒÓÔÓÙ·È Û ‚‰ÔÌ·‰È·›· ‚¿ÛË. ∆· ·È¯Ó›‰È· shockwave.com

¶·È‰È·ÙÚÈ΋ 2005;68:396-397

Pediatr Sept-Oct 05

17-10-05

16:52

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NEWS FROM THE INTERNET

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Î·È ÔÈ ‰È·‰ÈÎÙ˘·Î¤˜ ÂÊ·ÚÌÔÁ¤˜ ‰È·ÎÚ›ÓÔÓÙ·È ·Ó¿ ηÙËÁÔÚ›· Û ·È¯Ó›‰È· ‰Ú¿Û˘ (action games), ·È¯Ó›‰È· ÛÙÚ·ÙËÁÈ΋˜ (mind games), ·È¯Ó›‰È· Ì ÔÏÏ·ÏÔ‡˜ ¯Ú‹ÛÙ˜ (multiplayer games), ÂÓÒ ˘¿Ú¯ÂÈ Î·È ÚÔÙÂÈÓfiÌÂÓË Ï›ÛÙ· Ì ٷ 5 ÂÚÈÛÛfiÙÂÚÔ ‰ËÌÔÊÈÏ‹. ¶·Ú¿ÏÏËÏ·, ˘¿Ú¯ÂÈ Ë ‰˘Ó·ÙfiÙËÙ· ÏÔ‹ÁËÛ˘ ÛÂ Û˘Óԉ¢ÙÈÎfi ÔÙÈÎÔ·ÎÔ˘ÛÙÈÎfi ˘ÏÈÎfi, fiˆ˜ ʈÙÔÁڷʛ˜, ‚›ÓÙÂÔ, ÌÔ˘ÛÈ΋, ¢¯ÂÙ‹ÚȘ οÚÙ˜. ∏ ÚfiÛ‚·ÛË ÛÙ· ·È¯Ó›‰È· Â›Ó·È ‰ˆÚÂ¿Ó ÁÈ· ¯Ú‹ÛË Ì¤Ûˆ ÙÔ˘ ‰È·‰ÈÎÙ‡Ô˘, ÂÓÒ ·Ú¿ÏÏËÏ· Ô ¯Ú‹ÛÙ˘ ÌÔÚ› Ó· ÚÔÌËı¢Ù› Ù· ÂÚÈÛÛfiÙÂÚ· ·fi ·˘Ù¿ ·fi ÙÔ ÂȉÈÎfi ηٿÛÙËÌ· ·ÁÔÚÒÓ, Û ÏÔÁÈΤ˜ ÙÈ̤˜.

∏ÏÂÎÙÚÔÓÈÎfi “Û¯ÔÏ›Ԕ Ì·ıËÌ·ÙÈÎÒÓ ÁÈ· ÌÈÎÚÔ‡˜ Î·È ÌÂÁ¿ÏÔ˘˜ - ª·ıËÌ·ÙÈο ̤ۈ internet - http://www.primarygames.co.uk O ‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ Primary Games, ÚÔ˚fiÓ È‰ÈˆÙÈ΋˜ ÚˆÙÔ‚Ô˘Ï›·˜ Ì ¤‰Ú· ÙÔ ∏ӈ̤ÓÔ µ·Û›ÏÂÈÔ, ÚÔÛʤÚÂÈ ÏËıÒÚ· ‰˘Ó·ÙÔÙ‹ÙˆÓ ËÏÂÎÙÚÔÓÈ΋˜ ÂÎ·›‰Â˘Û˘ ÛÙȘ ‚·ÛÈΤ˜ ¤ÓÓÔȘ ÙˆÓ Ì·ıËÌ·ÙÈÎÒÓ Ô˘ ‰È‰¿ÛÎÔÓÙ·È ÛÙÔ Û¯ÔÏ›Ô, ·fi ÙȘ ÚÒÙ˜ Ù¿ÍÂȘ ÙÔ˘ ¢ËÌÔÙÈÎÔ‡ - Ì ·Ϥ˜ ÂÊ·ÚÌÔÁ¤˜ - ̤¯ÚÈ ÙȘ ÙÂÏÂ˘Ù·›Â˜ Ù¿ÍÂȘ - Ì ·È¯Ó›‰È· “·˘ÍË̤ӈӔ ··ÈÙ‹ÛˆÓ. ¢È·ÎÚ›ÓÂÙ·È Û 6 ‚·ÛÈΤ˜ ηÙËÁÔڛ˜, Ì ÂÊ·ÚÌÔÁ¤˜ ÂÎÌ¿ıËÛ˘ ÛÙȘ ‚·ÛÈΤ˜ ¤ÓÓÔȘ ÙˆÓ Ì·ıËÌ·ÙÈÎÒÓ, fiˆ˜ Â›Ó·È Ë ·ÚÈıÌËÙÈ΋, ÙÔ ¯Ú‹Ì· Î·È Ë Ì¤ÙÚËÛË Ù˘ ÒÚ·˜ ̤۷ ·fi “¤Í˘Ó·” Î·È ‰È·ÛΉ·ÛÙÈο ·È¯Ó›‰È·. ¶·Ú¿ÏÏËÏ·, ÚÔÛʤÚÂÈ ÛÙÔ˘˜ ÁÔÓ›˜ Î·È ÙÔ˘˜ ÂÎ·È‰Â˘ÙÈÎÔ‡˜ ‰È·‰ÈÎÙ˘·Î¿ “·Î¤Ù·” ÚÔ˜ ÒÏËÛË Ì ·ÓÙ›ÛÙÔȯ˜ ‰È·‰Ú·ÛÙÈΤ˜ ÌÂıfi‰Ô˘˜ ÂÎÌ¿ıËÛ˘. ∏ ÈÛÙÔÛÂÏ›‰· ÂÚÈÏ·Ì‚¿ÓÂÈ Ì›· ÌÂÁ¿ÏË ÔÈÎÈÏ›· ËÏÂÎÙÚÔÓÈÎÒÓ ·È¯ÓȉÈÒÓ, Ù· ÔÔ›· ÌÔÚÔ‡Ó Ó· ·ÔıË΢ÙÔ‡Ó Î·È Ó· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó Î·È ÂÎÙfi˜ ‰È·‰ÈÎÙ‡Ô˘. ¶ÚÈÓ ÙËÓ ÂÎΛÓËÛË ÂÓfi˜ Ó¤Ô˘ ·È¯ÓȉÈÔ‡, Ô ¯Ú‹ÛÙ˘ ‰È·‚¿˙ÂÈ ÙȘ Û‡ÓÙÔ̘ Ô‰ËÁ›Â˜ ¯Ú‹Û˘ Î·È ÂÓ Û˘Ó¯›· Ì·›ÓÂÈ Î·Ù¢ı›·Ó ÛÙÔ ·È¯Ó›‰È. ∆· ·È¯Ó›‰È· Â›Ó·È ·Ï¿ Î·È Û‡ÓÙÔÌ· Û ‰È¿ÚÎÂÈ·, ‰›ÓÔÓÙ·˜ ÙË ‰˘Ó·ÙfiÙËÙ· ÛÙÔÓ ¯Ú‹ÛÙË Ó· ‰ÔÎÈÌ¿ÛÂÈ fiÛÔ ÙÔ ‰˘Ó·ÙfiÓ ÂÚÈÛÛfiÙÂÚ·.

¶·È‰È·ÙÚÈ΋ 2005;68:396-397

Pediatr Sept-Oct 05

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17-10-05

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¢È·Ù·Ú·¯¤˜ Û¯ÔÏÈ΋˜ Ì¿ıËÛ˘ Î·È „˘¯Ô·ıÔÏÔÁ›· Àfi ¢.∫. ∞Ó·ÁÓˆÛÙfiÔ˘ÏÔ Î·È ∞.£. ™›ÓË ∂ΉfiÛÂȘ µ∏∆∞ ∞ı‹Ó·, 2004 ∆Ì‹Ì· ºÈÏÔÛÔÊ›·˜, ¶·È‰·ÁˆÁÈ΋˜ Î·È æ˘¯ÔÏÔÁ›·˜ ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ AÏÏËÏÔÁÚ·Ê›·: ∂˘ÊÚÔÛ‡ÓË ªfiÙÙË E-mail: stefanid@hol.gr

∆Ô ‚È‚Ï›Ô ÙˆÓ ¢.∫. ∞Ó·ÁÓˆÛÙfiÔ˘ÏÔ˘ Î·È ∞.£. ™›ÓË Ú·ÁÌ·Ù‡ÂÙ·È ÙÔ ı¤Ì· ÙˆÓ ‰È·Ù·Ú·¯ÒÓ Ù˘ Ì¿ıËÛ˘, ÁÓˆÛÙÒÓ ÛÙÔ Â˘Ú‡ ÎÔÈÓfi ˆ˜ ‰˘ÛÏÂÍ›·. ∆Ô ı¤Ì· ·˘Ùfi ¤¯ÂÈ Ï¿‚ÂÈ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ÛÔ‚·Ú¤˜ ÎÔÈÓˆÓÈΤ˜ ‰È·ÛÙ¿ÛÂȘ. ªÂÁ¿ÏÔ˜ ·ÚÈıÌfi˜ ÁÔÓ¤ˆÓ, ·È‰ÈÒÓ Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Û¯ÔÏÈ΋ ·ÔÙ˘¯›·, ηٷÎχ˙ÂÈ Ù· π·ÙÚÔ·È‰·ÁˆÁÈο ∫¤ÓÙÚ· Î·È Ù· ∫¤ÓÙÚ· ¢È¿ÁÓˆÛ˘, ∞ÍÈÔÏfiÁËÛ˘ Î·È ÀÔÛÙ‹ÚÈ͢ (∫.¢.∞.À.) Ì ·›ÙËÌ· Ó· ¿ÚÂÈ ÙÔ ·È‰› ÙÔ˘˜ ‚‚·›ˆÛË fiÙÈ ¤¯ÂÈ Úfi‚ÏËÌ· ‰˘ÛÏÂÍ›·˜, ÔfiÙ - ‚¿ÛÂÈ ÙÔ˘ ÓfiÌÔ˘ - ¤¯ÂÈ ÙÔ ‰Èη›ˆÌ· Ó· ˙ËÙ‹ÛÂÈ Ó· ÂÍÂÙ¿˙ÂÙ·È ÚÔÊÔÚÈο ÛÙ· Ì·ı‹Ì·Ù· ÙÔ˘ Û¯ÔÏ›Ԣ.

OÈ ‰È·Ù·Ú·¯¤˜ Ù˘ Ì¿ıËÛ˘ ÂΉËÏÒÓÔÓÙ·È Ì ÂȉÈΤ˜ Î·È ÛËÌ·ÓÙÈΤ˜ ‰˘ÛÎÔϛ˜ ÛÙËÓ ÂÎÌ¿ıËÛË Ù˘ ·Ó¿ÁÓˆÛ˘, Ù˘ ÁÚ·Ê‹˜, Ù˘ ÔÚıÔÁÚ·Ê›·˜ Î·È ÔÚÈṲ̂Ó˜ ÊÔÚ¤˜ Î·È Ù˘ ·ÚÈıÌËÙÈ΋˜. OÈ ‰˘ÛÎÔϛ˜ ·˘Ù¤˜ ‰ÂÓ ÔÊ›ÏÔÓÙ·È Û - ·Ó Î·È ÌÔÚ› Ó· Û˘Ó˘¿Ú¯Ô˘Ó - ÓÔËÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ·ÈÛıËÙËÚȷΤ˜ ‚Ï¿‚˜, Û˘Ó·ÈÛıËÌ·ÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, ¿ÏÏ· „˘¯È·ÙÚÈο ÚÔ‚Ï‹Ì·Ù·, ÂÚÈ‚·ÏÏÔÓÙÈ΋ ·ÔÛÙ¤ÚËÛË, ¤ÏÏÂÈ„Ë Â˘Î·ÈÚÈÒÓ ÁÈ· Ì¿ıËÛË ‹ ¿ÏÏÔ˘˜ ÔÏÈÙÈÛÌÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜. ∆· ·È‰È¿ Ô˘ ¤¯Ô˘Ó Úfi‚ÏËÌ· ‰˘ÛÏÂÍ›·˜, Â¿Ó ‰È·ÁÓˆÛÙÔ‡Ó ¤ÁηÈÚ· Î·È ‰Â¯ıÔ‡Ó ÙËÓ Î·Ù¿ÏÏËÏË „˘¯Ô·È‰·ÁˆÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË, ÌÔÚÔ‡Ó Ó· ÔÏÔÎÏËÚÒÛÔ˘Ó Ì ÂÈÙ˘¯›· ÙȘ Û¯ÔÏÈΤ˜ ÙÔ˘˜ ˘Ô¯ÚÂÒÛÂȘ.

OÈ Û˘ÁÁÚ·Ê›˜ ÙÔ˘ ‚È‚Ï›Ô˘ ¤¯Ô˘Ó ‚·ıÈ¿ ıˆÚËÙÈ΋ ÁÓÒÛË, ·ÏÏ¿ Î·È ÙÂÚ¿ÛÙÈ· ÎÏÈÓÈ΋ ÂÌÂÈÚ›· Û¯ÂÙÈο Ì ÙÔ ı¤Ì· ÙˆÓ ‰È·Ù·Ú·¯ÒÓ Ù˘ Ì¿ıËÛ˘. O ·ÚÈÔ˜ ∞Ó·ÁÓˆÛÙfiÔ˘ÏÔ˜ Â›Ó·È ·È‰Ô„˘¯›·ÙÚÔ˜, ˘‡ı˘ÓÔ˜ Ù˘ ÀËÚÂÛ›·˜ æ˘¯È΋˜ ÀÁ›·˜ ¶·È‰ÈÒÓ Î·È ∂Ê‹‚ˆÓ ÙÔ˘ ∫ÔÈÓÔÙÈÎÔ‡ ∫¤ÓÙÚÔ˘ æ˘¯È΋˜ ÀÁÈÂÈÓ‹˜ µ‡ÚˆÓ·∫·ÈÛ·ÚÈ·Ó‹˜ Ù˘ æ˘¯È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ. ∏ ‰È‰·ÎÙÔÚÈ΋ ÙÔ˘ ‰È·ÙÚÈ‚‹ ·ÊÔÚÔ‡Û ÛÙȘ ‰È·Ù·Ú·¯¤˜ Ù˘ Ì¿ıËÛ˘. ∏ ΢ڛ· ™›ÓË Â›Ó·È „˘¯Ô·È‰·ÁˆÁfi˜ Î·È ÂÚÁ¿˙ÂÙ·È ÛÙËÓ ›‰È· ˘ËÚÂÛ›· „˘¯È΋˜ ˘Á›·˜ Ì ÙÔÓ ÚÒÙÔ Û˘ÁÁڷʤ·. ∫‡ÚÈÔ ·ÓÙÈΛÌÂÓÔ Ù˘ ÂÚÁ·Û›·˜ Ù˘ Â›Ó·È ·ÎÚÈ‚Ò˜ Ë ‰È¿ÁÓˆÛË ÙˆÓ ‰È·Ù·Ú·¯ÒÓ Ù˘ Ì¿ıËÛ˘ Î·È Ë „˘¯Ô·È‰·ÁˆÁÈ΋ ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘˜. OÈ ‰‡Ô Û˘ÁÁÚ·Ê›˜ Û˘ÓÂÚÁ¿˙ÔÓÙ·È ÁÈ· ÙË ‰È¿ÁÓˆÛË Î·È ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·È‰ÈÒÓ Ì ÚÔ‚Ï‹Ì·Ù· ÛÙË Û¯ÔÏÈ΋ Ì¿ıËÛË. ∆Ô ‚È‚Ï›Ô ¯ˆÚ›˙ÂÙ·È Û ٤ÛÛÂÚ· ̤ÚË. ™Ù· ÚÒÙ· ‰‡Ô ̤ÚË Á›ÓÂÙ·È ‚È‚ÏÈÔÁÚ·ÊÈ΋ ·Ó·ÛÎfiËÛË Û¯ÂÙÈο Ì ÙȘ ‰È·Ù·Ú·¯¤˜ Ù˘ Ì¿ıËÛ˘ Î·È ÂÍÂÙ¿˙ÔÓÙ·È - ÌÂٷ͇ ¿ÏÏˆÓ - ı¤Ì·Ù· ÔÚÔÏÔÁ›·˜, ÂȉËÌÈÔÏÔÁ›·˜, ‰È¿ÁÓˆÛ˘ Î·È ÚfiÁÓˆÛ˘ ÙˆÓ ‰È·Ù·Ú·¯ÒÓ ·˘ÙÒÓ, Û˘ÓÓÔÛËÚfiÙËÙ·˜ Î.Ï. ™ÙÔ ÙÚ›ÙÔ Ì¤ÚÔ˜ Á›ÓÂÙ·È ·ÚÔ˘Û›·ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ ÌÈ·˜ ¤Ú¢ӷ˜ ÁÈ· ÙȘ Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜ Ô˘ ¤ÁÈÓ Ì ·È‰È¿ Ô˘ ›¯·Ó ·Ú·ÂÌÊı› ÛÙÔ ∫ÔÈÓÔÙÈÎfi ∫¤ÓÙÚÔ æ˘¯È΋˜ ÀÁÈÂÈÓ‹˜, fiÔ˘ ÂÚÁ¿˙ÔÓÙ·È ÔÈ Û˘ÁÁÚ·Ê›˜. µ·ÛÈÎÔ› ÛÙfi¯ÔÈ ·˘Ù‹˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ÂͤÏÈ͢ Ù˘ ‰È·Ù·Ú·¯‹˜ ÙÔ˘ ÁÚ·ÙÔ‡ ÏfiÁÔ˘ Î·È Ë ÂÌÊ¿ÓÈÛË Û˘ÓÓÔÛËÚfiÙËÙ·˜. ™ÙÔ Ù¤Ù·ÚÙÔ Î·È ÙÂÏÂ˘Ù·›Ô ̤ÚÔ˜ ÙÔ˘ ‚È‚Ï›Ô˘ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ù¤ÛÛÂÚȘ ÂÚÈÙÒÛÂȘ ·È‰ÈÒÓ Ì ‰È·Ù·Ú·¯¤˜ Ù˘ Û¯ÔÏÈ΋˜ Ì¿ıËÛ˘, ÔÈ Ôԛ˜ ‰Â›¯ÓÔ˘Ó ·Ó¿ÁÏ˘Ê· ÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ·, fiˆ˜ ÙË ‚ÈÒÓÔ˘Ó Ù· ›‰È· Ù· ·È‰È¿. ∆Ô ‚È‚Ï›Ô ·˘Ùfi οÓÂÈ Ì›· Û˘ÓÔÙÈ΋, ·ÏÏ¿ Î·È ÛÊ·ÈÚÈ΋ ·ÚÔ˘Û›·ÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ÙˆÓ ‰È·Ù·Ú·¯ÒÓ Ù˘ Ì¿ıËÛ˘, Û˘Ó‰˘¿˙ÔÓÙ·˜ ÌÈ· ηϋ ·Ó·ÛÎfiËÛË Ù˘ ‰ÈÂıÓÔ‡˜ Î·È ÂÏÏËÓÈ΋˜ Û¯ÂÙÈ΋˜ ‚È‚ÏÈÔÁÚ·Ê›·˜ Ì ÙË Ì·ÎÚfi¯ÚÔÓË ÎÏÈÓÈ΋ ÂÌÂÈÚ›· ÙˆÓ ‰‡Ô Û˘ÁÁڷʤˆÓ ÛÙË ‰È¿ÁÓˆÛË Î·È ÛÙËÓ „˘¯Ô·È‰·ÁˆÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Ì·ıËÛÈ·ÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ. ∆Ô Û‡ÁÁÚ·ÌÌ· ·˘Ùfi ı· ‰ÒÛÂÈ ÛÙÔÓ ·È‰›·ÙÚÔ Ì›· ÔÏÔÎÏËڈ̤ÓË ÂÈÎfiÓ· ÙˆÓ Û‡Á¯ÚÔÓˆÓ ÁÓÒÛÂˆÓ Û¯ÂÙÈο Ì ÙË ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Ì·ıËÛÈ·ÎÒÓ ‰È·Ù·Ú·¯ÒÓ. ∂˘ÊÚÔÛ‡ÓË ªfiÙÙË ∫·ıËÁ‹ÙÚÈ·, ∆Ì‹Ì· ºÈÏÔÛÔÊ›·˜, ¶·È‰·ÁˆÁÈ΋˜ Î·È æ˘¯ÔÏÔÁ›·˜ ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ

¶·È‰È·ÙÚÈ΋ 2005;68:398

Pediatr Sept-Oct 05

17-10-05

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22 OÎÙˆ‚Ú›Ô˘ 2005

1Ô ™˘ÌfiÛÈÔ ∂ıÓÈ΋˜ ∂ÈÙÚÔ‹˜ ∫·Ú‰ÈÔ·Ó·Ó¢ÛÙÈ΋˜ ∞Ó·˙ˆÔÁfiÓËÛ˘ (∂∂∫∞∞¡) ÙÔ˘ ∫ÂÓÙÚÈÎÔ‡ ™˘Ì‚Ô˘Ï›Ô˘ ÀÁ›·˜ (∫∂™À) ∞ÌÊÈı¤·ÙÚÔ £. ∫·Ú·Ù˙¿, ∂ıÓÈ΋ ∆Ú¿Â˙· ∂ÏÏ¿‰Ô˜, ¶Ï·Ù›· ∫ÔÙ˙È¿ ¶ÏËÚÔÊÔڛ˜: Congress World ∆ËÏ.: (+30) 210 7210 001, 210 7211 845 Fax: (+30) 210 7215 082 E-mail: info@congressworld.gr Website: www.congressworld.gr

∞ı‹Ó·

11-13 ¡ÔÂÌ‚Ú›Ô˘ 2005

15Ô ¢È··ÓÂÈÛÙËÌÈ·Îfi ™˘Ó¤‰ÚÈÔ ∞ÎÙÈÓÔÏÔÁ›·˜ ª¤Á·ÚÔ ªÔ˘ÛÈ΋˜ ∞ıËÓÒÓ ¶ÏËÚÔÊÔڛ˜: PRC Congress & Travel ∆ËÏ.: (+30) 210 7711 673, 210 7756 336 Fax: (+30) 210 7711 289 E-mail: secretariat@prctravel.gr

∞ı‹Ó·

26-28 π·ÓÔ˘·Ú›Ô˘ 2006 6Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ¶ÚÔÏËÙÈ΋˜ π·ÙÚÈ΋˜ •ÂÓÔ‰Ô¯Â›Ô Holiday Inn ¶ÏËÚÔÊÔڛ˜: PRC Congress & Travel ∆ËÏ.: (+30) 210 7711 673, 210 7756 336 Fax: (+30) 210 7711 289 E-mail: info@prctravel.gr

∞ı‹Ó·

18-19 ª·ÚÙ›Ô˘ 2006

4Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ÀÔÂȉÈÎÔÙ‹ÙˆÓ Ù˘ ¶·È‰È·ÙÚÈ΋˜ •ÂÓÔ‰Ô¯Â›Ô Divani Caravel ¶ÏËÚÔÊÔڛ˜: C&C International, Conventions and Congresses ∆ËÏ.: (+30) 210 6889 100 Fax: (+30) 210 6844 777 E-mail: ped-subspec2006@cnc.gr Website: www.ped-subspec2006.gr

∞ı‹Ó·

7-9 ∞ÚÈÏ›Ô˘ 2006

1Ô ¶·ÓÂÏÏ‹ÓÈÔ ¡ÂÔÁÓÔÏÔÁÈÎfi ™˘Ó¤‰ÚÈÔ •ÂÓÔ‰Ô¯Â›Ô Ledra Marriott ¶ÏËÚÔÊÔڛ˜: Triaena Tours ∆ËÏ.: (+30) 210 7499 300 Fax: (+30) 210 7705 752 E-mail: congress@triaenatours.gr

∞ı‹Ó·

26-29 ∞ÚÈÏ›Ô˘ 2006

XVIIth Annual Congress of the European Society for Paediatric Urology •ÂÓÔ‰Ô¯Â›Ô Athens Hilton ¶ÏËÚÔÊÔڛ˜: Erasmus Conferences Tours & Travel S.A. ∆ËÏ.: (+30) 210 7257 693 Fax: (+30) 210 7257 532 E-mail: info@espu2006.com Website: www.espu2006.com

∞ı‹Ó·

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2-5 ª·˝Ô˘ 2006

11o ¢ÈÂıÓ¤˜ ™˘Ó¤‰ÚÈÔ ∂Ù·ÈÚ›·˜ æ˘¯ÔÏÔÁÈ΋˜ æ˘¯È·ÙÚÈ΋˜ ∂ÓËÏ›ÎÔ˘ Î·È ¶·È‰ÈÔ‡ (∂ææ∂¶) “ºÈÏ›· & ∂ÓfiÙËÙ·, æ˘¯ÔÏÔÁ›· & ∂ÈÎÔÈÓˆÓ›·” •ÂÓÔ‰Ô¯Â›Ô Athens Hilton ¶ÏËÚÔÊÔڛ˜: ∂Ù·ÈÚ›· æ˘¯ÔÏÔÁÈ΋˜ æ˘¯È·ÙÚÈ΋˜ ∂ÓËÏ›ÎÔ˘ Î·È ¶·È‰ÈÔ‡ ∆ËÏ.: (+30) 210 6842 663, 210 6857 319 Fax: (+30) 210 6842 079 E-mail: appachellas@yahoo.gr Website: www.epsep.org.gr

∞ı‹Ó·

3-5 ª·˝Ô˘ 2006

24th Annual Meeting of the European Society for Paediatric Infectious Diseases - ESPID ¶ÏËÚÔÊÔڛ˜: Kenes International / ESPID 2006 ∆ËÏ.: +41 22 908 0488 Fax: +41 22 732 2850 E-mail: espid@kenes.com Website: www.kenes.com/espid/index2006.asp

Basel, Switzerland

10-14 πÔ˘Ó›Ô˘ 2006

XXV Congress of the European Academy of Allergology and Clinical Immunology ¶ÏËÚÔÊÔڛ˜: Congrex Sweden ∆ËÏ.: +46 8 459 66 00 Fax: +46 8 661 91 25 E-mail: eaaci2006@congrex.se Website: www.congrex.com/eaaci2006

Vienna, Austria

30 πÔ˘Ó›Ô˘3 πÔ˘Ï›Ô˘ 2006

45th Annual Meeting of the European Society for Paediatric Endocrinology ¶ÏËÚÔÊÔڛ˜: Congrex Sweden AB ∆ËÏ.: +46 8 459 66 00 Fax: +46 8 661 91 25 E-mail: espe2006@congrex.se Website: www.espe2006.org

8-11 πÔ˘Ï›Ô˘ 2006

7th International Congress on Pediatric Pulmonology ¶ÏËÚÔÊÔڛ˜: Annie Bidart, MD ∆ËÏ.: +33 0 497 038 597 Fax: +33 0 497 038 598 E-mail: cipp@cipp-meeting.com Website: www.cipp-meeting.com

Rotterdam, The Netherlands

Montreal, Canada