Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος -...

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TfiÌÔ˜ 68 ñ ∆‡¯Ô˜ 5 ñ ™Â٤̂ÚÈÔ˜-√ÎÙÒ‚ÚÈÔ˜ 2005 TfiÌÔ˜ 68 ñ ∆‡¯Ô˜ 5 ñ ™Â٤̂ÚÈÔ˜-√ÎÙÒ‚ÚÈÔ˜ 2005 ¶·È‰È·ÙÚÈ΋ www.paediatriki.edu.gr www.paediatriki.edu.gr ISSN 0377-2551 ISSN 0377-2551 Volume 68 ñ Number 5 ñ September-October 2005 Volume 68 ñ Number 5 ñ September-October 2005

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Τριμηνιαία έκδοση της Ελληνικής Παιδιατρικής Εταιρείας

Transcript of Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος -...

Page 1: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

TfiÌÔ˜ 68 ñ ∆‡¯Ô˜ 5 ñ ™Â٤̂ÚÈÔ˜-√ÎÙÒ‚ÚÈÔ˜ 2005TfiÌÔ˜ 68 ñ ∆‡¯Ô˜ 5 ñ ™Â٤̂ÚÈÔ˜-√ÎÙÒ‚ÚÈÔ˜ 2005¶·

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www.paediatriki.edu.grwww.paediatriki.edu.gr

ISSN 0377-2551ISSN 0377-2551

Volume 68 ñ Number 5 ñ September-October 2005Volume 68 ñ Number 5 ñ September-October 2005

Exof Pediatr Sept-Oct 05 17-10-05 14:01 ™ÂÏ›‰·1

Page 2: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

¢ÈÌËÓÈ·›· ¤Î‰ÔÛË Ù˘

EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜

¶Úfi‰ÚÔ˜

∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜

™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹

¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘

∫. ™ÙÂÊ·Ó›‰Ë˜

M¤ÏË

¶. ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘

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°. µ·ÚÏ¿Ì˘

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A. K·ÙÙ¿Ì˘

Ã. ∫ÒÛÙ·ÏÔ˜

¡. ¶··‰fiÔ˘ÏÔ˜

∞. ¶··‰ÔÔ‡ÏÔ˘

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ª. ∆ÛÔÏÈ¿-¶·Û·Ú¿ÎË

ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ·

∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈ̤ӈÓ

I. K·Ú·‚Ú¿ÓÔ˘

EÈ̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈ̤ӈÓ

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E¶I™THMONIKE™ EK¢O™EI™ E.¶.E.

∞ÚÙ¤ÌȉԘ 3

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325 ∂Ó·ÚÎÙ‹ÚÈÔ˜ OÌÈÏ›· ÛÙÔ

43Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ

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329 ¶·ıÔÊ˘ÛÈÔÏÔÁ›· ÙˆÓ ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈÎÒÓ

ÂÂÈÛÔ‰›ˆÓ ÛÙË ‰Ú·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‹˜, ∞. ∫ÈÙÛÔÔ‡ÏÔ˘, ∫. ª›¯·˜, ¶. ¡ÈÎÔÏ·˝‰Ô˘-∫·Ú·ı›Ô˘

∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™

352 ™‡Ó‰ÚÔÌÔ Guillain-Barré. ŒÎ‚·ÛË Ù˘

ÓfiÛÔ˘ Û ·È‰È¿ Ô˘ ÓÔÛËχÙËÎ·Ó ÛÂ

ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £Âڷ›·˜

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

358 ∫›Ó‰˘ÓÔ˜ ·fi ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜

·ÙfiÌˆÓ Ì ۇӉÚÔÌÔ Down ηÈ

·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ·

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

∂¡¢π∞º∂ƒOÀ™∂™ ¶∂ƒπ¶∆ø™∂π™

366 ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË. ¶ÂÚÈÁÚ·Ê‹

ÚÒÙ˘ ÂÚ›ÙˆÛ˘ ÛÙËÓ ∂ÏÏ¿‰·

O. ¶··Ó‰Ú¤Ô˘, ∞. ∫ÏËÌÂÓÙÔÔ‡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

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∞. ∞Ó‰Ú¤Ô˘, µ. ∞ÏÂÍ·Ó‰ÚÔÔ‡ÏÔ˘-∆ÛÈÎڛη, ∞. ™·ÎÂÏÏ·ÚÔÔ‡ÏÔ˘, ª. °Ô˘‰ÂÛ›‰Ô˘, §. ºÏˆÚÂÓÙ›Ó-∞Ú¿Ú

∂¶π∫∞πƒO £∂ª∞

377 ∏ ›‰Ú·ÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ô˘

¯ÔÚËÁÔ‡ÓÙ·È Î·Ù¿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô

ÛÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘

∂. ªÔ‡˙·, ∫. ¶··Á·ÚÔ˘Ê¿Ï˘

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386 ¶ÚÔÏËÙÈÎfi˜ ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ηÈ

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∫ˆ‰ÈÎfi ̃¢È‡ı˘ÓÛË ̃∂ÔÙ›· ̃ªª∂: 3889

ISSN 0377-2551

¶·È‰È·ÙÚÈ΋∆fiÌÔ˜ 68 ñ ∆‡¯Ô˜ 5 ñ ™Â٤̂ÚÈÔ˜-OÎÙÒ‚ÚÈÔ˜ 2005

Pediatr Sept-Oct 05 17-10-05 16:50 ™ÂÏ›‰·1

Page 3: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 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

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English Editing

S. Nakou

Publisher

K. Griveas

Publishing Coordinator

SCIENTIFIC PUBLICATIONS Ltd

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Contents

LETTER FROM THE EDITOR

325 Opening Lecture at the

43rd Panhellenic Paediatric Meeting

G. D. Babiniotis

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

351 Fibromyalgia in childhood

V. Tsagris, A. Kitsopoulou, C. Mihas, P. Nikolaidou-Karpathiou

ORIGINAL ARTICLES

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

CASE REPORTS

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

CURRENT ISSUE

385 The effect of postnatal administration of

steroids on the developing brain

H. Bouza, C. Papagaroufalis

SPECIAL ARTICLE

394 Genetic preventive testing and ethical

issues

H. Hatzissevastou-Loukidou

395 LETTER TO THE EDITOR

396 NEWS FROM THE INTERNET

398 BOOK REVIEW

PaediatrikiVolume 68 ñ Number 5 ñ September-October 2005

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·3

Page 4: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

xi√¢∏°π∂™ ¶ƒ√™ ∆√À™ ™À°°ƒ∞º∂π™

A. °ÂÓÈΤ˜ ¶ÏËÚÔÊÔڛ˜∆Ô ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ΋” Â›Ó·È Ë ÂÈÛÙËÌÔÓÈ΋ ¤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‡ ÂÈÛÙËÌÔÓÈÎÔ‡¤ÚÁÔ˘ Î·È ÙË Û˘Ó¯‹ ÂÓË̤ڈÛË ÙˆÓ ¶·È‰È¿ÙÚˆÓ. °È· ÙÔÛÎÔfi ·˘Ùfi ‰¤¯ÂÙ·È ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÔÈÎÈÏ›· ¿ÚıÚˆÓ Î·ÈÛ˘ÁÎÂÎÚÈ̤ӷ:

1. ÕÚıÚ· Û‡ÓÙ·Í˘ (ÌÂÙ¿ ·fi ÚfiÛÎÏËÛË Ù˘ ™˘ÓÙ·-ÎÙÈ΋˜ EÈÙÚÔ‹˜).

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· Âη›‰Â˘Û˘ Î·È ÔÚÁ¿ÓˆÛ˘ ˘Á›·˜.9. ∂ӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ.

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 ÂÈÛÙËÌÔÓÈ-

ÎÒÓ ÂΉËÏÒÛˆÓ15. ∞Ó·ÊÔÚ¤˜ Î·È ÎÚÈÙÈ΋ Ó¤ˆÓ ÂΉfiÛˆÓ, ÂÏÏËÓÈÎÒÓ

Î·È Í¤ÓˆÓ, ·È‰È·ÙÚÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜.

H ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ó· ‰Ë-ÌÔÛȇÂÈ ¿ÚıÚ· Ì ȉȷ›ÙÂÚÔ ÂÈÛÙËÌÔÓÈÎfi ÂӉȷʤÚÔÓ,ηıÒ˜ Î·È ¿ÚıÚ· Ì ›ηÈÚ· ı¤Ì·Ù· ¯ˆÚ›˜ Ó· ÙËÚÂ›Ù·È ËÛÂÈÚ¿ ˘Ô‚ÔÏ‹˜. ∂›Û˘, ÌÔÚ› Ó· ·ÔÊ·Û›˙ÂÈ ÁÈ· ÙË ‰Ë-ÌÔÛ›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 ÂÈÛÙÔÏÒÓ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ‰ËÌÔ-ÛÈÂ˘Ì¤Ó· ÂÈÛÙËÌÔÓÈο ¿ÚıÚ· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡.

™ÙȘ ÂÚÁ·Û›Â˜ Ì ›ηÈÚ· ı¤Ì·Ù· Ú¤ÂÈ Ó· ·Ó·ÁÚ¿-ÊÂÙ·È ÛÙËÓ ÚÒÙË ÛÂÏ›‰· Ë ÚfiıÂÛË ÙˆÓ Û˘ÁÁڷʤˆÓÁÈ· ÙËÓ ·Ó·ÁηÈfiÙËÙ· Ù˘ Ù·¯Â›·˜ ‰ËÌÔÛ›Â˘Û˘. ∏ ∂È-ÙÚÔ‹ ™‡ÓÙ·Í˘ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ù˘ ·Ô‰Ô¯‹˜ Ù˘ٷ¯Â›·˜ ‰ËÌÔÛ›Â˘Û˘.

ŸÏ˜ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ÌËÓ ¤¯Ô˘Ó ÂÓ Ì¤ÚÂÈ ‹ ÂÍÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚ÏËı› ÁÈ· ÎÚ›ÛË Û ¿ÏÏÔÂÚÈÔ‰ÈÎfi. ∂ÈϤÔÓ, ÛÙËÓ ÂÚÁ·Û›· ı· Ú¤ÂÈ Ó· ·Ó·Ê¤-ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘Ô-ÛÙ‹ÚÈ͢. ŸÏ˜ ÔÈ ÎÏÈÓÈΤ˜ ¤Ú¢Ó˜ Ú¤ÂÈ Ó· ¤¯Ô˘Ó Á›ÓÂÈÌÂÙ¿ ·fi ÏËÚÔÊÔÚË̤ÓË Û˘Ó·›ÓÂÛË ÙˆÓ ÌÂÙ¯fiÓÙˆÓ ‹ÙˆÓ ÓÔÌ›ÌˆÓ ÂÎÚÔÛÒˆÓ ÙÔ˘˜, Û‡Ìʈӷ Ì ÙȘ ‰È·ÎËÚ‡-ÍÂȘ ÙÔ˘ EÏÛ›ÓÎÈ Î·È ÙÔ˘ TfiÎÈÔ. E›Û˘, Ú¤ÂÈ Ó· ¤¯Ô˘ÓÙËÚËı› ÔÈ Ô‰ËÁ›Â˜ ÁÈ· ÙË ÊÚÔÓÙ›‰· ÂÈÚ·Ì·Ùfi˙ˆˆÓ ÙÔ˘∂ıÓÈÎÔ‡ πÓÛÙÈÙÔ‡ÙÔ˘ ÀÁ›·˜ ÙˆÓ ∏¶∞ (DHEWPublication, NIH, 80-23). OÈ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Ú¤ÂÈ Ó·¤¯Ô˘Ó ÂÁÎÚÈı› ·fi ÙËÓ ÂÈÙÚÔ‹ ∏ıÈ΋˜ Î·È ¢ÂÔÓÙÔÏÔ-Á›·˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ.

OÈ ·fi„ÂȘ Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓÔ˘ ‰È·Ù˘ÒÓÔÓÙ·È ÛÙȘ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ‰ÂÓ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ··Ú·›ÙËÙ· ·˘Ù¤˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. Hηٷ¯ÒÚÈÛË ‰È·ÊËÌ›ÛÂˆÓ ÛÙÔ ÂÚÈÔ‰ÈÎfi ‰ÂÓ ˘Ô‰ËÏÒÓÂÈÔˆÛ‰‹ÔÙ ¤ÁÎÚÈÛË ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘˜ ·fi ÙËÓEÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·, ÙË ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹‹ ·fi ÙÔÓ EΉfiÙË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡.

ŸÏ˜ ÔÈ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ıˆÚÔ‡ÓÙ·È È‰ÈÔÎÙË-Û›· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ “¶·È‰È·ÙÚÈ΋” Î·È Ë ÔÏÈ΋ ‹ ÌÂÚÈ΋

·Ó·‰ËÌÔÛ›Â˘Û‹ ÙÔ˘˜ ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ ÌÂÙ¿ ·fi ¤ÁÁÚ·ÊË

Û˘ÁηٿıÂÛË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡.

µ. ™‡ÓÙ·ÍË ÙˆÓ ∂ÚÁ·ÛÈÒӟϘ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ·ÎÔÏÔ˘ıÔ‡Ó “∫ÔÈÓ¤˜ ¶ÚÔ-

‰È·Áڷʤ˜ ÁÈ· ÃÂÈÚfiÁÚ·Ê· Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È Û µÈÔ˚·-

ÙÚÈο ¶ÂÚÈÔ‰Èο” (Uniform Requirements for Manuscripts

Submitted to Biomedical Journals), ÔÈ Ôԛ˜ ÙÚÔÔÔÈ‹-

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˘ÌÂÚÈÏ·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 ‰ÈÏfi ‰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: ÛÂ-

Ï›‰· Ù›ÙÏÔ˘, ‚Ú·¯‡˜ Ù›ÙÏÔ˜, ÂÚ›ÏË„Ë ÛÙ· ÂÏÏËÓÈο Î·È Ù·

·ÁÁÏÈο, ηٿÏÔÁÔ˜ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ, ΛÌÂÓÔ, ¢¯·ÚÈ-

Ûٛ˜ ηıÒ˜ Î·È ·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿Ï-

Ϙ 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· ·ÚÈı-

ÌÔ‡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 ÂÈÛÙÔϤ˜ 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 ÂÈÙÚ¤ÔÓÙ·È Û˘ÓÙÌ‹ÛÂȘ.

ñ ÙÔ fiÓÔÌ· Î·È ÙÔ ÂÒÓ˘ÌÔ Î¿ıÂ Û˘ÁÁڷʤ· ÛÙËÓ ÔÓÔ-

Ì·ÛÙÈ΋.

ñ ÙÔ ÂÈÛÙËÌÔÓÈÎfi ΤÓÙÚÔ (›‰Ú˘Ì·, ÎÏÈÓÈ΋, ÂÚÁ·ÛÙ‹-

ÚÈÔ) ·fi fiÔ˘ 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 ÙfiÔ˜ ‰È·ÌÔÓ‹˜

ÙÔ˘/ÙÔ˘˜.

ñ ÙËÓ Ï‹ÚË ‰È‡ı˘ÓÛË, e-mail Î·È ÙÔ ÙËϤʈÓÔ ÙÔ˘

Û˘ÁÁڷʤ· Ì ÙÔÓ ÔÔ›Ô Á›ÓÂÙ·È Ë ·ÏÏËÏÔÁÚ·Ê›·.

¶ÂÚÈÏ‹„ÂȘH 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˘ÌÂÚ¿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, ·ÔÙÂϤ-

ÛÌ·Ù· Î·È Û˘ÌÂÚ¿ÛÌ·Ù·.

™ÙËÓ ÂÚ›ÏË„Ë ÛÙ· ·ÁÁÏÈο Ú¤ÂÈ Ó· ·Ó·ÁÚ¿ÊÔ-

ÓÙ·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: ÂÈ-

Û·ÁˆÁ‹ (background), ̤ıÔ‰ÔÈ (methods), ·ÔÙÂϤÛÌ·-

Ù· (results) Î·È Û˘ÌÂÚ¿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 ÛË-ÌÂÈÒÓÔÓÙ·È ÙÚÂȘ ¤ˆ˜ ¤ÓÙ ϤÍÂȘ ÎÏÂȉȿ Ô˘ ı· ¯ÚËÛÈ-ÌÔÔÈËıÔ‡Ó ÁÈ· ÙÔ ıÂÌ·ÙÈÎfi ¢ÚÂÙ‹ÚÈÔ.

K›ÌÂÓÔOÈ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ÂÈÛ·ÁˆÁ‹,

ÌÂıfi‰Ô˘˜, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘˙‹ÙËÛË. H ÂÈÛ·ÁˆÁ‹ Â-ÚÈÏ·Ì‚¿ÓÂÈ Ù· ÙÂÏÂ˘Ù·›· ‰Â‰Ô̤ӷ Ù˘ ¤Ú¢ӷ˜ ÛÙÔ Û˘-ÁÎÂÎÚÈ̤ÓÔ ı¤Ì· Ì ÙȘ ΢ÚÈfiÙÂÚ˜ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·-Ú·Ô̤˜ Î·È ÙÔÓ ÛÎÔfi Ù˘ ÂÚÁ·Û›·˜. H ÂÚÈÁÚ·Ê‹ ÙˆÓÌÂıfi‰ˆÓ Ú¤ÂÈ Ó· Â›Ó·È ·ÎÚÈ‚‹˜ Î·È ·ÚÎÂÙ¿ ÏÂÙÔÌÂ-Ú‹˜, ÒÛÙ ӷ ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó··Ú·ÁˆÁ‹ ÙÔ˘˜ ·fi ¿Ï-ÏÔ˘˜ ÂÚ¢ÓËÙ¤˜. ∂›Û˘, Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ì ÏÂ-ÙÔ̤ÚÂȘ ÔÈ ÛÙ·ÙÈÛÙÈΤ˜ ̤ıÔ‰ÔÈ ·Ó¿Ï˘Û˘ Î·È ·ÍÈÔÏfi-ÁËÛ˘ ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ. T· ·ÔÙÂϤÛÌ·Ù· Ú¤ÂÈÓ· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ۷ʋÓÂÈ· Î·È Ó· Û˘Óԉ‡ÔÓÙ·È·fi ÙËÓ ··Ú·›ÙËÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË. H Û˘˙‹ÙËÛËÚ¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ô˘ ÚÔ·-ÙÔ˘Ó ·fi ÙËÓ ÂÚÁ·Û›·, ÙË ÛËÌ·Û›· Ô˘ ÌÔÚ› Ó· ¤¯Ô˘ÓÎ·È ÙËÓ Èı·Ó‹ Û˘Û¯¤ÙÈÛ‹ ÙÔ˘˜ Ì ·Ú·ÙËÚ‹ÛÂȘ ¿ÏψÓÂÚ¢ÓËÙÒÓ.

OÈ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ 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 ÙËÓÎÚ›ÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ÙÔ˘˜.

∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹-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·Ô̤˜.

TÈ̤˜ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛˆÓOÈ ÙÈ̤˜ ÙˆÓ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ Ú¤ÂÈ Ó· ÂÎ-

ÊÚ¿˙ÔÓÙ·È ÛÙÔ ¢ÈÂıÓ¤˜ ™‡ÛÙËÌ· MÔÓ¿‰ˆÓ (SI Units) ηÈÛÙÔ MÂÙÚÈÎfi (Conventional-™˘Ì‚·ÙÈÎfi) ™‡ÛÙËÌ· ̤۷ Û·ڤÓıÂÛË. ¶›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 ÂÈ‚ÏËı› ‰ÈÂıÓÒ˜,

‰ËÌÔÛȇÔÓÙ·È Û οı Ù‡¯Ô˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ™‡ÓıÂÙÔÈÎ·È Ì·ÎÚÔÛÎÂÏ›˜ fiÚÔÈ Ô˘ ·ӷϷ̂¿ÓÔÓÙ·È Û˘¯Ó¿ ÛÙÔ Î›ÌÂÓÔ ÌÔÚÔ‡Ó Ó· ·ÓÙÈηı›ÛÙ·ÓÙ·È ·fi Û˘ÓÙÔÌÔ-Áڷʛ˜ Ô˘ ÂÂÍËÁÔ‡ÓÙ·È ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ Û ÂȉÈ-Îfi ηٿÏÔÁÔ, Ô ÔÔ›Ô˜ ˘Ô‚¿ÏÏÂÙ·È Ì·˙› Ì ÙËÓ ÂÚÁ·Û›·. OÈ Û˘ÓÙÔÌÔÁڷʛ˜ ·Ó·Ê¤ÚÔÓÙ·È Û ·Ú¤ÓıÂÛË ÌfiÓÔÛÙȘ ÂÚÈÏ‹„ÂȘ.

BÈ‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜™ÙÔ ÙÌ‹Ì· Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ ηٷ¯ˆÚÔ‡ÓÙ·È fiϘ

ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ·Ó·Ê¤-ÚÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ™ÙÔ Î›ÌÂÓÔ, ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·-Ú·Ô̤˜ ·Ó·Ê¤ÚÔÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Û ·-Ú¤ÓıÂÛË. OÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ ‰ÂÓ Ú¤ÂÈ Ó·˘ÂÚ‚·›ÓÔ˘Ó:

ñ ÙȘ 70 ÛÙȘ ·Ó·ÛÎÔ‹ÛÂȘ. ñ ÙȘ 30 ÛÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜. ñ ÙȘ 12 ÛÙ· ›ηÈÚ· ı¤Ì·Ù· Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜

ÂÚÈÙÒÛÂȘ. ñ ÙȘ 5 ÛÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ Î·È ÙȘ ÂÈÛÙÔϤ˜.

H Û‡ÓÙ·ÍË ÙˆÓ ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ Á›ÓÂÙ·ÈÛ‡Ìʈӷ Ì ÙȘ ÚfiÛÊ·Ù· ÙÚÔÔÔÈË̤Ó˜ ÚԉȷÁÚ·-ʤ˜ Ù˘ International Committee of Medical Journal

Editors/ Uniform Requirements for Manuscripts Sub-mittedto Biomedical Journals, (http://www.icmje.org Î·È http://www.icmje.org/icmje.pdf). OÈ Û˘ÓÙÌ‹ÛÂȘ ÙˆÓ Ù›ÙÏˆÓ ÙˆÓÂÚÈÔ‰ÈÎÒÓ Á›ÓÔÓÙ·È Ì ‚¿ÛË ÙÔ Cumulated IndexMedicus [List of Journals Indexed in Index Medicus(http://www.nlm.nih.gov/bsd/uniform_requirements.html)].

¶·Ú·‰Â›ÁÌ·Ù· ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ

I. ¶∂ƒπO¢π∫∞

AÓ ÔÈ Û˘ÁÁÚ·Ê›˜ Â›Ó·È ¤ˆ˜ ¤ÍÈ, ·Ó·ÁÚ¿ÊÔÓÙ·È fiÏÔÈ,ÂÓÒ ·Ó Â›Ó·È ÂÙ¿ ‹ ÂÚÈÛÛfiÙÂÚÔÈ, ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ ÚÒ-ÙÔÈ ¤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·: ›‰Ú·-ÛË ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ÙˆÓ ÏÔÈ̈‰ÒÓ ÓÔÛËÌ¿ÙˆÓ Î·È ÙËӷȉȷÙÚÈ΋ Ú¿ÍË. ¶·È‰È·ÙÚÈ΋ 1999;59:272-279.

Proesmans W. Bartter syndrome and its neonatalvariant. Eur J Pediatr 1997;156:669-679.

™˘ÌÏËڈ̷ÙÈÎfi Ù‡¯Ô˜ ÂÚÈÔ‰ÈÎÔ‡:

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

Èڛ˜ Û˘ÁÁڷʤ·:

National Institutes of Health Consensus DevelopmentConference. Neurofibromatosis conference statement. ArchNeurol 1988;45:575-578.

¶ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù‡Ô˘ ¿ÚıÚÔ˘:

Schreiner GF, Lange L. Ethanol modulation ofmacrophage influx in glomerulonephritis [Abstract]. J AmSoc Nephrol 1991;2:562.

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

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

II. µπµ§π∞

∫ÂÊ¿Ï·ÈÔ Û ‚È‚Ï›Ô:

Clark AG, Barratt TM. Steroid-responsive nephroticsyndrome. In: Barratt TM, Arner ED, Harmon WE, editors.Pediatric Nephrology. 4th ed. Baltimore: Lippincott Wil-liam Wilkins; 1999. p. 742.

™‡ÁÁÚ·ÌÌ· ‹ ÌÔÓÔÁÚ·Ê›·:

Gorlin RJ, Cohen MM, Levin LS. Syndromes of the headand neck. 3rd ed. New York: Oxford University Press; 1990.

¢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 In-ternational Congress of Chemotherapy; 1962 May 29-31;New York: International Society of Chemotherapy; 1963. p.484-500.

¢È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹:

¶··‰fiÔ˘ÏÔ˜ Ã. ∏ ıÂڷ›· ÙÔ˘ ÛÙÚ·‚ÈÛÌÔ‡ [‰È‰·-ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹]. ∞ı‹Ó·: ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ; 1979.

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xiii

Kaplan SJ. Post hospital home health care: the elderly’saccess and utilization [dissertation]. St. Louis (Mo): Wash-ington Univ.; 1995.

πππ. CD-ROMAnderson SC, Poulsen KB. Anderson’s electronic atlas

of hematology [CD-ROM]. Philadelphia: LippincottWilliams & Wilkins; 2002.

IV. ™∆O ¢π∞¢π∫∆ÀOÕÚıÚÔ Û ÂÚÈÔ‰ÈÎfi:

Abood S. Quality improvement initiative in nursinghomes: 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

πÛÙÔÛÂÏ›‰Â˜:Cancer-Pain.org [Webpage, Internet]. New York:

Association of Cancer Online Resources, Inc.; 2002:http://www.cancer-pain.org/

¶›Ó·Î˜ Î·È EÈÎfiÓ˜AÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡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˘ ÛÂ-Ï›‰·˜ (15,5 cm). TÔ Ì¤ÁÈÛÙÔ Ì‹ÎÔ˜ ÙÔ˘˜, Ì·˙› Ì ÙÔ˘˜ Ù›Ù-ÏÔ˘˜, ‰ÂÓ Ú¤ÂÈ Ó· Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ·fi 22 cm.

OÈ ›Ó·Î˜ ·ÚÈıÌÔ‡ÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Ì ÙËÛÂÈÚ¿ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ¶ÂÚÈÏ·Ì‚¿ÓÔ˘Ó‚Ú·¯‡ Ù›ÙÏÔ Î·È ÂÂÍ‹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˜ ÙˆÓÂÈÎfiÓˆÓ Ú¤ÂÈ Ó· ÛËÌÂÈÒÓÂÙ·È Ì ÌÔχ‚È Ô ·ÚÈıÌfi˜ Ù˘ÂÈÎfiÓ·˜ Î·È ÙÔ fiÓÔÌ· ÙÔ˘ ÚÒÙÔ˘ Û˘ÁÁڷʤ·, ηıÒ˜ ηȤӷ ‚¤ÏÔ˜ ÙÔ ÔÔ›Ô Ó· ‰Â›¯ÓÂÈ ÙÔ ¿Óˆ ̤ÚÔ˜ Ù˘ ÂÈÎfiÓ·˜.™ÙȘ ʈÙÔÁڷʛ˜ ·ÛıÂÓÒÓ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·ÁÓˆÚ›˙ÂÙ·ÈË Ù·˘ÙfiÙËÙ¿ ÙÔ˘˜. OÈ ·ÛıÂÓ›˜ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·ÈÔÓÔÌ·ÛÙÈο.

°. ÀÔ‚ÔÏ‹ Î·È ¢ËÌÔÛ›Â˘ÛË ÙˆÓ ∂ÚÁ·ÛÈÒӟϘ 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˜ ηȷfi ÂÈÛÙÔÏ‹, Ë ÔÔ›· ı· Û˘Ó˘ÔÁÚ¿ÊÂÙ·È ·fi fiÏÔ˘˜ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜, ÛÙËÓ ÔÔ›· ı· ‰ËÏÒÓÂÙ·È fiÙÈ Ë ÂÚÁ·Û›·‰ÂÓ ¤¯ÂÈ ÂÓ Ì¤ÚÂÈ ‹ ÂÍ ÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚ÏË-ı› 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-΋”. ∂ÈϤÔÓ, ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹-ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢.

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

H ηı˘ÛÙ¤ÚËÛË Ù˘ ·ӢԂÔÏ‹˜ ÙÔ˘ ÙÚÔÔÔÈ-Ë̤ÓÔ˘ ÎÂÈ̤ÓÔ˘ ¤Ú·Ó ÙˆÓ 30 ËÌÂÚÒÓ Û˘Ó¿ÁÂÙ·ÈÓ¤· ˘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 ÂÈÛÙÚ¤ÊÔÓÙ·È. MÔÚ› Ó· ÂÈÛÙÚ·-ÊÔ‡Ó, ÂÊfiÛÔÓ ˙ËÙËı› ÂÓÙfi˜ ÂÍ·Ì‹ÓÔ˘, Ù· Û¯‹Ì·Ù· Î·È ÔÈʈÙÔÁڷʛ˜ Ô˘ Ù· Û˘Óԉ‡ԢÓ.

OÈ ÂÚÁ·Û›Â˜ Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È ÚÔ˜ ÎÚ›ÛË ÁÈ· ‰ËÌÔ-Û›Â˘ÛË ÛÙÔ ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ΋” ·ÔÛÙ¤ÏÏÔÓÙ·È ÂȘÙÚÈÏÔ‡Ó ÛÙË ‰È‡ı˘ÓÛË:

™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92115 28 Aı‹Ó·

¶ÚÈÓ ·ÔÛÙ›ÏÂÙ ÙËÓ ÂÚÁ·Û›· Û·˜, ‚‚·Èˆı›Ù fiÙÈÂÚÈÏ·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 ÂÒÓ˘ÌÔ Û˘ÁÁڷʤˆÓ (ÔÏÔÁڿʈ˜) Á. ÂÈÛÙËÌÔÓÈÎfi/¿ ΤÓÙÚÔ/· fiÔ˘ ¤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˘ÌÂÚ¿ÛÌ·Ù·, ηıÒ˜ Î·È Ï¤ÍÂȘ ÎÏÂȉȿ (‰ÈÏfi‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).

6. ∫·Ù¿ÏÔÁÔ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈ-ÛÙ‹ ÛÂÏ›‰·).

7. ∫›ÌÂÓÔ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).8. ∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹-

ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢).9. µÈ‚ÏÈÔÁÚ·Ê›· (‰ÈÏfi ‰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 ÔÔ›Ô Ó· ‰Â›-¯ÓÂÈ ÚÔ˜ Ù· ¿ӈ, ·ÚÈıÌË̤Ó˜, ÂȘ ÙÚÈÏÔ‡Ó.

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

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·13

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xiv ™À¡∆√ª√°ƒ∞ºπ∂™ ABBREVIATIONS

Ao

angstrom angstromcal ıÂÚÌ›‰· caloriecm ÂηÙÔÛÙfi centimetercm2 ÙÂÙÚ·ÁˆÓÈÎfi ÂηÙÔÛÙfi square centimetercm3 ΢‚ÈÎfi ÂηÙÔÛÙfi cubic centimeteroC ‚·ıÌfi˜ ∫ÂÏÛ›Ô˘ degree Celsiusg ÁÚ·ÌÌ¿ÚÈÔ gramh ÒÚ· hourIU ‰ÈÂıÓ‹˜ ÌÔÓ¿‰· international unitkg ¯ÈÏÈfiÁÚ·ÌÌÔ kilograml Ï›ÙÚÔ literm ̤ÙÚÔ metermg ¯ÈÏÈfiÁÚ·ÌÌÔ milligrammin ÏÂÙfi minutemm ¯ÈÏÈÔÛÙfiÌÂÙÚÔ millimetermol ÁÚ·ÌÌÔÌfiÚÈÔ molen ·ÚÈıÌfi˜ numberNS ‹ ª™ ÌË ÛËÌ·ÓÙÈÎfi not significantosm ÔÛÌÒÏÈÔ osmole

p Èı·ÓfiÙËÙ· probabilitySD ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË standard deviationSE ÛÙ·ıÂÚfi ÛÊ¿ÏÌ· standard errorsec ‰Â˘ÙÂÚfiÏÂÙÔ secondU ÌÔÓ¿‰· unit

™˘Ó‰˘·˙fiÌÂÓ· ÚÔı¤Ì·Ù· Combining prefixes

tera- (1012) Tgiga- (109) Gmega- (106) Mkilo- (103) khector- (102) hdeca- (101) dadeci- (10-1) dcenti- (10-2) cmilli- (10-3) mmicro- (10-6) Ìnano- (10-9) npico- (10-12) pfemto- (10-15) fatto- (10-18) a

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xviiINSTRUCTIONS TO AUTHORS

A. General InformationThe Greek Paediatric Society is the owner of “Paedia-

triki”, its official scientific journal, which is distributed to

its members. Its objectives are the publication of paediatric

scientific work and the continuing education of paediatri-

cians. 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 publish-

es 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 previ-

ously, 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 in-

formed consent of participants or of their legal representa-

tives 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 jour-

nal. 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 “Paedi-

atriki” and their reproduction in whole or in part is autho-

rized 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 web-

sites:

http://www.icmje.org and http://www.icmje.org/icmje.pdf

The entire paper (including figure legends and tables)

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

The paper should have the following structure: title page,short title, abstract in Greek and English, list of abbrevia-tions, text, acknowledgements and quoting of grants, spon-sorships or other financial support sources, references, ta-bles, figures, figure legends. Each of these sections should bestarted on a new page. Pages should be numbered consecu-tively, 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., privatepaediatrician) and home address;

ñ the complete address, e-mail and telephone number ofthe author to whom correspondence should be addressed.

Abstracts

The abstract should summarize the objectives, method-ology, main results and conclusions of the study.

ñ It should contain at least 200 words, and not exceed 250words.

ñ It should consist of the following paragraphs: back-ground, methods, results and conclusions.

The English abstract should cite at the beginning the ti-tle of the paper and the authors’ names in English. The con-tent of the text should consist of the following paragraphs:background, methods, results and conclusions. The abstractin English should not differ in content from the correspond-ing Greek abstract.

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

Text

Original articles include: introduction, methods, resultsand discussion. The introduction includes the latest re-search data on the subject and the main references and theobjectives of the paper. The description of the methods

should be precise and detailed so as to enable reproductionby other researchers. In addition, the statistical methods ofanalysis and evaluation of the results should be described.Results should be presented clearly, together with the ap-propriate statistical analysis. Discussion should cover theresults ensuing from the research, their significance andpossible associations with the observations of other re-searchers.

Case reports comprise a short introduction, case de-scription and brief discussion, with emphasis on differentialdiagnosis.

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

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

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xviii

Units of measures of laboratory analyses

Laboratory analyses should be expressed in the Sys-tème International (SI) units and in the metric (Conven-tional) system in parentheses. See conversion tables on thewebsites: http://www.icmje.org and http://www.icmje.org/icmje.pdf

Abbreviations

All issues of the journal contain internationally estab-lished abbreviations. Complex or long terms often repeatedin the text may be replaced by abbreviations explained bythe authors in a list submitted with the paper. Abbreviationsare reported in parentheses only in abstracts.

References

The reference section contains all references numberedin the order in which they appear in the text. In the text, ref-erences are to be indicated by Arabic numerals in parenthe-ses. 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 stan-dards of the International Committee of Medical Journal Edi-tors/Uniform Requirements for Manuscripts Submitted toBiomedical Journals, (http://www.icmje.org and http://www.icmje.org/icmje.pdf). Abbreviated names of journalsshould conform to the Cumulated Index Medicus [List ofJournals 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 7or more, the first six are cited, followed by “et al”.

Regular edition:

Proesmans W. Bartter syndrome and its neonatal vari-ant. Eur J Pediatr 1997;156:669-679.

Supplement issue:

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

No author:

National Institutes of Health Consensus DevelopmentConference. Neurofibromatosis conference statement. ArchNeurol 1988;45:575-578.

Article type specification:

Schreiner GF, Lange L. Ethanol modulation ofmacrophage influx in glomerulonephritis [Abstract]. J AmSoc Nephrol 1991;2:562.

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

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

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 Wil-

liam Wilkins; 1999. p. 742.

Book or monograph:

Gorlin RJ, Cohen MM, Levin LS. Syndromes of the

head and neck. 3rd ed. New York: Oxford University Press;

1990.

Publication in a volume of proceedings:

Bauer AW. The two definitions of bacterial resistance.

In: Smith AJ, Rogers CA, eds. Proceedings of the Third In-

ternational 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): Wash-

ington Univ.; 1995.

πππ. CD-ROM

Anderson SC, Poulsen KB. Anderson’s electronic atlas

of hematology [CD-ROM]. Philadelphia: Lippincott Wil-

liams & 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 [Inter-

net]. 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: Na-

tional Academy Press; 2001. Webpage:

http://www.nap.edu/books/0309074029/html

Websites

Cancer-Pain.org [Webpage, Internet]. New York: Asso-

ciation 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. Ver-

tical 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 pa-

tients should not be recognizable from their pictures nor

should their names be stated.

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xix

C. Manuscript Submission and PublicationAll 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 inpart or in whole, or is not under consideration by anotherjournal and that the authors accept its publication in “Pae-diatriki”. Any funding, sponsorship or other financial sup-port should be acknowledged.

Once the manuscript has been accepted, the correctedversion, rewritten according to the reviewers’ recommenda-tions should be submitted to the Editorial Board accompa-nied by a floppy disk or CD, containing the paper in Wordformat, along with a covering letter specifying in detail themodifications or objections to the reviewers’ suggestions.

Delay in submission of the modified paper exceeding30 days entails new submission.

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

Manuscripts of papers which have not been approvedfor publication are not returned to the author. The accom-panying figures and photographs can be returned upon re-quest within six months.

Manuscripts submitted for review and publication in“Paediatriki” should be sent in three copies to the followingaddress:

Editorial BoardGreek Paediatric Society92, Michalakopoulou Street115 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 pa-

per (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 tele-

phone number; 5. English and Greek abstracts, with the following struc-

ture: 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, sponsor-

ships 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.

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·19

Page 11: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

325∂¶π™∆√§∏ ∞¶√ ∆∏ ™À¡∆∞•∏

¶·È‰È·ÙÚÈ΋ 2005;68:325-328

™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˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ∫·ıËÁËÙ‹ °ÂˆÚÁ›Ô˘ ª·ÌÈÓÈÒÙË Ì ı¤Ì·: “∂ÏÏË-

ÓÈ΋ 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· ›ηÈÚÔ 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·ÌÈÓÈÒÙ˘

∫¿ı ·ÓıÚÒÈÓË ÁÏÒÛÛ· Â›Ó·È ÌÈ· ÌÔÚÊ‹ Ù·ÍÈÓfiÌËÛ˘ ÙÔ‡ ÎfiÛÌÔ˘. ∫¿ı ÂıÓÈ΋ ÁÏÒÛÛ·, Ë

ÁÏÒÛÛ· Ô˘ ÌÈÏ¿ÂÈ ¤Ó· ¤ıÓÔ˜ Â›Ó·È Ô ÙÚfiÔ˜ Ô˘ Û˘ÏÏ·Ì‚¿ÓÂÈ, ÂÂÍÂÚÁ¿˙ÂÙ·È Î·È Ù·ÍÈÓÔÌ› ÙÔÓ

ÎfiÛÌÔ Ô Ï·fi˜ Ù‹˜ ¯ÒÚ·˜ Ô˘ ÌÈÏ¿ÂÈ ·˘Ù‹ ÙË ÁÏÒÛÛ·. ŒÙÛÈ, οı ÁÏÒÛÛ· - fiˆ˜ ÙÔ ¤¯ÂÈ ‰È‰¿ÍÂÈ Ô

Saussure - Â›Ó·È Î·È ÌÈ· ¿ÏÏË Ù·ÍÈÓfiÌËÛË ÙÔ‡ ÎfiÛÌÔ˘. ŒÓ·˜ ¿ÏÏÔ˜ ÙÚfiÔ˜ Ó· ‚ϤÂȘ, Ó· ·ÈÛı¿-

ÓÂÛ·È Î·È Ó· ‚¿˙ÂȘ Û ٿÍË ÙÔÓ ÎfiÛÌÔ. ∫È ·˘Ùfi Á›ÓÂÙ·È Ì ÁψÛÛÈο ̤۷. ªÂ ÙÔÓ ÙÚfiÔ Ô˘ ¤Ó·˜

Ï·fi˜ Έ‰ÈÎÔÔÈ› ÁψÛÛÈο ÙȘ ¤ÓÓÔȘ Û ÛË̷ۛ˜, ·˘Ù¤˜ Ô˘ ÂÈϤÁÂÈ 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„˘ ηÈ

Ù‹˜ ÓÔÔÙÚÔ›·˜ οı Ϸԇ.

∞˜ ԇ̠·fi ÙÒÚ· ÌÈ· ¿ÏÏË ‚·ÛÈ΋ ı¤ÛË: O ¿ÓıÚˆÔ˜ ÛΤÙÂÙ·È Ì¤Û· ·fi ÙȘ ϤÍÂȘ Î·È Ì¤-

Û· ·fi ÙȘ ÁÚ·ÌÌ·ÙÈΤ˜ ‰Ô̤˜ Î·È ÛË̷ۛ˜ Ì ÙȘ Ôԛ˜ Â›Ó·È ÔÚÁ·ÓˆÌ¤ÓË Ë ÁÏÒÛÛ· ÙÔ˘. øÛÙfi-

ÛÔ, ·˘Ùfi˜ Ô “ÁψÛÛÈÎfi˜ ÓÙÂÙÂÚÌÈÓÈÛÌfi˜”, ‰ÂÓ ÛËÌ·›ÓÂÈ fiÙÈ Ô ¿ÓıÚˆÔ˜ Â›Ó·È Î·È “‰Ô‡ÏÔ˜” Ù‹˜

ÁÏÒÛÛ·˜ ÙÔ˘, ÁÈ·Ù› ·ÎÚÈ‚Ò˜ Ë ÁÏÒÛÛ· ÙÔ‡ ·ÓıÚÒÔ˘ - fiˆ˜ ¤¯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‡ ·ÓıÚÒÔ˘ , Ú¿ÁÌ· Ô˘ ÂȂ‚·ÈÒÓÂÈ Î·È

Ô Wittgenstein ϤÁÔÓÙ·˜ ÙÔ ÂÚ›ÊËÌÔ “fi,ÙÈ ÌÔÚÒ Ó· Û˘ÏÏ¿‚ˆ ηı·Ú¿ Ì ÙÔÓ ÓÔ˘ ÌÔ˘, ÌÔÚÒ Ó· ÙÔ ÂÎ-

ÊÚ¿Ûˆ ηı·Ú¿ Î·È Ì ÙË ÁÏÒÛÛ· ÌÔ˘”, ÂÓÓÔÒÓÙ·˜ fiÙÈ ‰ÂÓ ÌÔÚԇ̠ӷ ÂÎÊÚ¿ÛÔ˘Ì ÁψÛÛÈο Ì ۷-

Ê‹ÓÂÈ· 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˘ÓÂȉËÙÔÔÈÒÓÙ·˜

ÙË ÛÙÂÓ‹ Û¯¤ÛË ÓfiËÛ˘ Î·È ÁÏÒÛÛ·˜, ¤ÊÙ·Û·Ó Ó· ¯ÚËÛÈÌÔÔÈ‹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˘˜ ÏÂÁfi-

ÌÂÓÔ˘˜ ÎÏ·ÛÈÎÔ‡˜ ¯ÚfiÓÔ˘˜) ·Ó¤Ù˘Í·Ó ¤Ó·Ó ÔÏÈÙÈÛÌfi, ¤Ó· Û‡ÛÙËÌ· ·Ú¯ÒÓ Î·È ·ÍÈÒÓ Ì ÂÈÙ‡Á-

Ì·Ù· ÛÙÔ˘˜ ıÂÛÌÔ‡˜ Î·È ÛÙȘ Ù¤¯Ó˜, ÙȘ ÂÈÛً̘ 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 ÂÈı˘Ì›· ÙÔ˘˜ Ó· ÁÓˆÚ›ÛÔ˘Ó

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·325

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326 ∂Ó·ÚÎÙ‹ÚÈÔ˜ ÔÌÈÏ›·

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‡ ·ÓıÚÒÔ˘, ÁÈ· ÙȘ Û¯¤ÛÂȘ ÙˆÓ ·ÓıÚÒˆÓ 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 ÂÈÛ‹Ì·ÓÛË ·Ú¯ÒÓ Î·È ·ÍÈÒÓ Ô˘ Ú¤ÂÈ Ó·

‰È¤Ô˘Ó ÙË ˙ˆ‹ ÙÔ‡ ·ÓıÚÒÔ˘ 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‡ ·ÓıÚÒÔ˘ Ì ÙË ÌÔ›Ú· ÙÔ˘). ∆Ô˘˜ ‰›‰·Í·Ó fiÙÈ Â›-

Ó·È ÛËÌ·ÓÙÈÎfi ÁÈ· ÙÔÓ ¿ÓıÚˆÔ Ó· ηÏÏÈÂÚÁ‹ÛÂÈ ÙÔ Ó‡̷ ÙÔ˘, Ó· ·ÔÎÙ‹ÛÂÈ ·È‰Â›·, ·Ú¿ÏÏË-

Ï· 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˘ÌÈ·ÎÒÓ ·ÁÒÓˆÓ, ÂÛÙÈ·ÛÌ¤ÓˆÓ ·Ô-

ÎÏÂÈÛÙÈο ÛÙËÓ Â˘ÁÂÓ‹ ¿ÌÈÏÏ· Î·È Û˘Ó‰˘·ÛÌ¤ÓˆÓ Ì ÙËÓ ·fi‰ÔÛË ‡„ÈÛÙˆÓ ÙÈÌÒÓ ÛÙÔ˘˜ OÏ˘-

ÌÈÔӛΘ Î·È Ì ·‡ÛË ÙˆÓ Â¯ıÚÔÚ·ÍÈÒÓ, ‰ËÏ·‰‹ Ì ¤ÓÙÔÓ· ÓÂ˘Ì·ÙÈÎfi Ì‹Ó˘Ì·, ·ÓÂÏÏ‹ÓÈ·

Û˘ÌÌÂÙÔ¯‹ Î·È Îϛ̷ ÊÈÏ›·˜.

OÈ ÌÂÁ¿ÏÔÈ ÛÙÔ¯·ÛÙ¤˜, ÊÈÏfiÛÔÊÔÈ ‹ ÂÈÛÙ‹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 ÂÈÛÙËÌÔÓÈΤ˜

·Ó·˙ËÙ‹ÛÂȘ Î·È Ë ·Ó¿ÁÎË ÙˆÓ ÂÈÛÙËÌfiÓˆÓ Ó· ÙȘ ‰ËÏÒÛÔ˘Ó ‰ËÌÈÔ˘ÚÁÔ‡Ó ÙÔÓ ÂÈÛÙËÌÔÓÈÎfi 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· ÌÔÚ› Ó· ÂÎÊÚ¿˙ÂÈ ÙËÓ ÏÔ‡ÛÈ· ‰Ú·ÛÙËÚÈfiÙËÙ· ÛÙÔ˘˜ ÙÔÌ›˜ Ù‹˜ ÂÈÛÙ‹Ì˘ Î·È ÙÔ‡ ÔÏÈ-

ÙÈÛÌÔ‡. ∏ 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›, ÂÈÛÙ‹ÌÔÓ˜ Î.Ï.) Â›Ó·È ÂÍ

·Ó¿Á΢ Î·È ÌÂÁ¿ÏÔÈ ‰ËÌÈÔ˘ÚÁÔ› ÁÏÒÛÛ·˜. ∆Ô ÏÂÙfi, Û‡ÓıÂÙÔ, ··ÈÙËÙÈÎfi, ÚˆÙfiÙ˘Ô Û ȉ¤Â˜ ηÈ

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 ÂÎÏËÎÙÈ΋ ÎÏ›-

̷η, ÙÚÔÔÔÈÒÓÙ·˜ ÏÈÁfiÙÂÚÔ ‹ ÂÚÈÛÛfiÙÂÚÔ ‹ ÌÂÙ·‚¿ÏÏÔÓÙ·˜ ÙË ‚·ÛÈ΋ ÛËÌ·Û›· Ù‹˜ Ϥ͢.

∞˘Ùfi ÂÈÙ˘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 ÂÌ‚¿Ïψ ÂÈÛ‚¿Ïψ Û˘Ì‚¿Ïψ ÚÔ‚¿Ïψ ÚÔÛ‚¿Ïψ

·Ó·‚¿Ïψ ηٷ‚¿Ïψ ‰È·‚¿Ïψ ÌÂÙ·‚¿Ïψ ·Ô‚¿Ïψ ÂÚÈ‚¿Ïψ

˘ÂÚ‚¿Ïψ ·Ú·‚¿Ïψ ˘Ô‚¿Ïψ ·ÓÙÈ‚¿Ïψ ÂÈ‚¿Ïψ Î.Ô.Î.

∞˜ ÛËÌÂȈı› fiÙÈ ÙÔ Ú‹Ì· ÙÔ Û‡ÓıÂÙÔ Ì ¤Ó· ÚÔÚÚËÌ·ÙÈÎfi ÌÔÚ› Ó· ÂÍÂȉÈ·ÂÙ·È ÂÚ·ÈÙ¤-

Úˆ Ì ÂÈϤÔÓ ÚÔÚÚËÌ·ÙÈο:

ÂÈ-‚¿Ïψ: Û˘Ó-ÂÈ‚¿Ïψ ÚÔ-ÂÈ‚¿Ïψ ·Ú-ÂÈ‚¿Ïψ ÚÔÛ-ÂÈ‚¿Ïψ

‰È·-‚¿Ïψ: ÂÈ-‰È·‚¿Ïψ ·ÓÙÈ-‰È·‚¿Ïψ ÂÓ-‰È·‚¿Ïψ Û˘Ó-‰È·‚¿Ïψ

˘Ô-‰È·‚¿Ïψ ÚÔ-‰È·‚¿Ïψ ÚÔÛ-‰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.¿.).

∂˘ÚÒË (Europe): ∆Ô fiÓÔÌ· Ù‹˜ ∂˘ÚÒ˘ Â›Ó·È Ë ÂÏÏËÓÈ΋ ÔÓÔÌ·Û›· ÂÓfi˜ Ì˘ıÔÏÔÁÈÎÔ‡ ÚÔ-

ÛÒÔ˘ Ô˘ Û‹Ì·ÈÓ Èı·Ófiٷٷ “·˘Ù‹Ó Ô˘ ¤¯ÂÈ Â˘Ú›˜ Ò·˜, ÌÂÁ¿Ï· Ì¿ÙÈ·”. ∏ Ú›˙· Ô- ‚Ú›ÛÎÂÙ·È

Û ϤÍÂȘ fiˆ˜ ÔÙÈÎfi˜ (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 ÔÔ›Ô Ì¿ÏÈÛÙ· ÁÚ¿ÊÂÙ·È ¿Óˆ ÛÙÔ ¯·ÚÙÔÓfiÌÈÛÌ· ηÈ

Ì ÙËÓ ÂÏÏËÓÈ΋ ÁÚ·Ê‹ Ù‹˜ Ϥ͢.

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327∂Ó·ÚÎÙ‹ÚÈÔ˜ ÔÌÈÏ›·

ŒÓ·˜ ÂÓÓÔÈÔÏÔÁÈÎfi˜ ¯ÒÚÔ˜ fiÔ˘ 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 ‹ ÂÈÛÙ‹ÌË

‹Ù·Ó ÔÈ Ú¿ÍÂȘ Î·È ÔÈ ÁÓÒÛÂȘ ÙÔ‡ ˘Â‡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¯·›· ‰Ë-

ÌÔÎÚ·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 ÔÔ›· 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˘ ‚Ϥˆ 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 ÁÓÒ-

ÛÙ˘”), ·Ú¯Èο “·˘Ùfi˜ Ô˘ ‚ϤÂÈ 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 ÂÈÛÙ‹ÌË ÛÙËÓ Î·ıËÌÂÚÈÓ‹ ÁÏÒÛÛ· ÌÈ·˜ ÈÔ ··È-

ÙËÙÈ΋˜ ÌÔÚÊ‹˜ ÂÈÎÔÈÓˆÓ›·˜. ¶·Ú·‰Â›ÁÌ·Ù·:

ıˆڛ· (theory), Ú·ÎÙÈ΋ (practice), ÂÌÂÈÚÈÎfi˜ (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›ˆ-

Ì·-·ÍȈ̷ÙÈÎfi˜ (axiom-axiomatic), fiÚÁ·ÓÔ (organ, organize, organism), ÚfiÁÚ·ÌÌ· (program),

ı¤Ì· (theme), ı¤ÛȘ (thesis), ÙfiÔ˜/ÙÔÈÎfi˜ (topic), Ù‡Ô˜ (type), Ê¿ÛË (phase), ·Ú¿ÏÏËÏÔ˜

(parallel), ˙ÒÓË (zone), ÛÎÂÙÈÎfi˜ (skeptic/skepticism), ·ÓÈÎfi˜, Û‡Ìو̷ (symptom), ıÂÚ·-

›· (therapy), ÎÏ›ÓË/ÎÏÈÓÈÎfi˜ (clinic), ‰È¿ÁÓˆÛȘ (diagnosis), Ê¿ÚÌ·ÎÔ (pharmacy), ÂÓ‰ËÌÈÎfi˜-

ÂȉËÌÈÎfi˜ (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›ÛÎÔÔ˜, monk Ô ÌÔÓ·¯fi˜, card Î·È chart Ô ¯¿ÚÙ˘, bomb Ô ‚fiÌ‚Ô˜, buffalo Ô ‚Ô‡‚·ÏÔ˜,

diploma Î·È diplomat ÙÔ ‰›ÏˆÌ· (< ‰ÈÏÒÓˆ). ∞˜ ÛËÌÂȈı› fiÙÈ ·ÎfiÌË Î·È ÂˆÓ˘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΋˜ ÁÏÒÛ-

Û·˜ ̤۷ ÛÙÔÓ Â˘Úˆ·˚Îfi ÔÏÈÙÈÛÌfi Î·È ÙËÓ Â˘Úˆ·˚΋ ÛΤ„Ë, fiˆ˜ ÂÎÊÚ¿˙ÂÙ·È Ì ¤ÓÓÔȘ ηÈ

Ì ϤÍÂȘ ÙˆÓ Â˘Úˆ·˚ÎÒÓ ÁψÛÛÒÓ. £· ÌÔÚÔ‡Û· Ó· ·Ó·Ê¤Úˆ ¯ÈÏÈ¿‰Â˜ ÂÏÏËÓÈΤ˜ ϤÍÂȘ, Ô˘ ›-

Ó·È ‹‰Ë Û˘ÁÎÂÓÙڈ̤Ó˜ Û ÂȉÈο ¤ÚÁ· ÁψÛÛÔÏfiÁˆÓ, ÊÈÏÔÏfiÁˆÓ Î·È ¿ÏÏˆÓ ÂÈÛÙËÌfiÓˆÓ Î·È Ô˘

ÌÔÚ› Ó· ‚ÚÂÈ Î·Ó›˜ Û fiÏ· Ù· ¤Á΢ڷ ÏÂÍÈο ÙˆÓ ÂÈ̤ÚÔ˘˜ 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˘

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328 ∂Ó·ÚÎÙ‹ÚÈÔ˜ ÔÌÈÏ›·

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 ÁÂÁÔ-Ófi˜ Ù‹˜ ÏÂÍÈÏÔÁÈ΋˜ Ù˘ ̛͈˜, ÙÔ‡ ‰·ÓÂÈÛÌÔ‡ ‰ËÏ·‰‹ ·fi ÙȘ ÎÏ·ÛÈΤ˜ ΢ڛˆ˜ ÁÏÒÛÛ˜, ԢηıÈÛÙ¿ ÙËÓ ∞ÁÁÏÈ΋ ÔÈΛ· Î·È ·Ô‰ÂÎÙ‹ ·fi ÔÌÈÏËÙ¤˜ ¿ÏÏˆÓ ÁψÛÛÒÓ. O Crystal ‰›ÓÂÈ ÛÙÔÎÏ·ÛÈÎfi ÏÂÍÈÏfiÁÈÔ ÌÈ· ¿ÏÏË ‰È¿ÛÙ·ÛË. ∆Ô ‚ϤÂÈ ˆ˜ “ÂÓÔÔÈËÙÈ΋ 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΋ ›-Ó·È Ë ·Ú·Ù‹ÚËÛË ÙÔ‡ Crystal fiÙÈ “ÔÈ ÎÏ·ÛÈΤ˜ ÁÏÒÛÛ˜ Û˘Ó¯›˙Ô˘Ó Ó· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙËÓ ∞ÁÁÏÈ΋ˆ˜ ‰˘Ó·ÌÈ΋ ËÁ‹ Ó¤ˆÓ Ϥ͈Ó, ˆ˜ ËÁ‹ ÏÂÍÈÏÔÁÈ΋˜ ‰ËÌÈÔ˘ÚÁ›·˜ […] Â›Ó·È ÂÎÏËÎÙÈÎfi Ó· ‚ϤÂȘ fi-ÛÔ 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· ÂÈÛÙ‹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· Ô-ÛÔÙÈÎfi. ∂›Ó·È ı¤Ì· ÔÈÔÙÈÎfi. ∂›Ó·È ¿ÏÏÔ Ú¿ÁÌ· ‰ËÏ. - ÛÂ Â›Â‰Ô ÂÈÎÔÈÓˆÓ›·˜, ηÏÏȤÚÁÂÈ·˜,ÓÔËÙÈ΋˜ ‰ÈÂÚÁ·Û›·˜ - Ó· ‰›ÓÂȘ Ï.¯. ϤÍÂȘ-¤ÓÓÔȘ fiˆ˜ Ë ıˆڛ·, Ë È‰¤·, Ë Ô›ËÛË, Ë Ù¯ÓÔÏÔÁ›·, ËÔÏÈÙÈ΋, Ë ‰ËÌÔÎÚ·Ù›·, Ë Ê·ÓÙ·Û›·, Ô ‹Úˆ·˜, Ë ÌÔ˘ÛÈ΋, ÙÔ ı¤·ÙÚÔ, Ë ÈÛÙÔÚ›·, Î·È ¿ÏÏÔ Ó· ‰›ÓÂȘ Ϥ-ÍÂȘ-¤ÓÓÔȘ fiˆ˜ hamburger, fast-food, supermarket, kits, ÓÙÔÏÌ¿‰Â˜, Ì·ÚÌÔ‡ÙÈ ‹ Ï·ÌfiÁÈ·! °È·Ù› ËÔÈfiÙËÙ· Ù‹˜ ÛΤ„˘ Î·È Ù‹˜ ¤ÎÊÚ·Û˘ Â›Ó·È Û˘Ó¿ÚÙËÛË Ù‹˜ ÔÈfiÙËÙ·˜ Ù‹˜ ÁÏÒÛÛ·˜ Î·È ÙÔ‡ ÛË-Ì·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 ÌÂÚˆÙfiÁÓˆÚË ÂÎÊÚ·ÛÙÈ΋ ÔÈÎÈÏ›· Î·È Â‡ÚÔ˜ ÛËÌ·ÛÈÔÏÔÁÈÎÒÓ ·Ô¯ÚÒÛˆÓ. ∆¤ÏÔ˜, Ë ÂÓÓÔÈÔÏfi-ÁËÛË Î·È ÏÂÍÈ΋ Û‹Ì·ÓÛË ÙÔ‡ ÎfiÛÌÔ˘ ÙˆÓ Ú·ÁÌ¿ÙˆÓ Î·È ÙÔ‡ Ê¿ÛÌ·ÙÔ˜ ÙˆÓ ·ÊËÚËÌ¤ÓˆÓ ÂÓÓÔÈ-ÒÓ Ô‰‹ÁËÛ ÛÙË ÁÓˆÛÙ‹ Î·È ÙÈÌËÙÈ΋ ÁÈ· ÙËÓ ÂÏÏËÓÈ΋ ÁÏÒÛÛ· Ú‹ÛË “The Greeks must have a wordfor it”! ∆Ô Ó· ¤¯ÂȘ fï˜ ϤÍË ÁÈ· fiÏ· Ù· Ú¿ÁÌ·Ù· Î·È ÁÈ· οı ¤ÓÓÔÈ· ÛËÌ·›ÓÂÈ Ó· ¤¯ÂȘ ‰È¢ڇ-ÓÂÈ ÙfiÛÔ Ôχ Ù· fiÚÈ· Ù‹˜ ÁÓÒÛ˘ Î·È Ù‹˜ ÓfiËÛ‹˜ ÛÔ˘ Î·È Ó· ¤¯ÂȘ ·ÎÔÓ›ÛÂÈ Û ÙfiÛË ¤ÎÙ·ÛË ÙÔÓÏfiÁÔ ÛÔ˘, ÒÛÙ ӷ ÌÔÚ›˜ Ó· ‰ËÏÒÓÂȘ Ì ÙË ÁÏÒÛÛ· ÔÏfiÎÏËÚÔ ÙÔ Ê¿ÛÌ· ÙÔ‡ ÎfiÛÌÔ˘ Ì·˜, ·ÊÔ‡- fiˆ˜ ¤¯ÂÈ ÂÈ Ôχ ·Ú·ÛÙ·ÙÈο Ô Wittgenstein - Ô ÎfiÛÌÔ˜ Ì·˜ Â›Ó·È Ë ÁÏÒÛÛ· Ì·˜.

ªÈ· ÁÏÒÛÛ· fiˆ˜ Ë ∂ÏÏËÓÈ΋ Ô˘ Â˘Ù‡¯ËÛ ӷ ¯ÚËÛÈÌÔÔÈÂ›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˘ › 25 Î·È Ï¤ÔÓ ·ÈÒÓ˜ ÛÌÈχÂÙ·È ·Î·Ù¿-·˘ÛÙ· ÛÙÔÓ ¯ÒÚÔ Ù‹˜ ÂÈÛÙ‹Ì˘ Î·È Ù‹˜ Ô›ËÛ˘ - ‰ÂÓ ˘¿Ú¯ÂÈ Ô‡Ù ¤Ó·˜ ·ÈÒÓ·˜ Ô˘ Ó· ÌËÓ ¤¯ÂÈÁÚ·Ê› Ô›ËÛË Û ÂÏÏËÓÈ΋ ÁÏÒÛÛ·, ¤ÏÂÁÂ Ô ∂χÙ˘ - Â›Ó·È ·Ó·ÌÂÓfiÌÂÓÔ, fiˆ˜ ÚÔÛ¿ıËÛ· Ó·‰Â›Íˆ, Ó· ¤¯ÂÈ ÏÔ˘Ù›ÛÂÈ ÙÔÓ Â˘Úˆ·˚Îfi ÏfiÁÔ Ì ıÂÌÂÏÈÒ‰ÂȘ ¤ÓÓÔȘ Î·È Ï¤ÍÂȘ. ∏ ÂÏÏËÓÈ΋ ÁÏÒÛ-Û· 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˘ ÚÔÛÔÚ›˙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˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ∫·ıËÁËÙ‹˜ °ÂÒÚÁÈÔ˜ ¢. ª·ÌÈÓÈÒÙ˘

¶·È‰È·ÙÚÈ΋ 2005;68:325-328

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·328

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329∞¡∞™∫O¶∏™∏

¶·ıÔÊ˘ÛÈÔÏÔÁ›· ÙˆÓ ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈÎÒÓ

ÂÂÈÛÔ‰›ˆÓ ÛÙË ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ

∂. ∫·Ú·Ù˙¿ - •ÈÊÈÏ›‰Ô˘1, ª. ∞ı·Ó·Û›Ô˘ - ªÂÙ·Í¿2

¶ÂÚ›ÏË„Ë

∏ ‰Ú·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 (a2b2

S). ∞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·ÌÙˆÓ ‰Ú·ÓÔ΢ÙÙ¿ÚˆÓ. Œˆ˜ ÙȘ ·Ú¯¤˜ Ù˘ ÙÂÏÂ˘Ù·›·˜ ‰ÂηÂÙ›·˜ ıˆÚÔ‡ÓÙ·Ó fiÙÈ Ù· ¿Î·-ÌÙ· ‰Ú·ÓÔ·ÙÙ·Ú· ¢ı‡ÓÔÓÙ·È ÁÈ· ÙËÓ ÚfiÎÏËÛË ÙˆÓ ÂÒ‰˘ÓˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÎÚ›ÛÂˆÓ Ù˘ÓfiÛÔ˘. ¡ÂfiÙÂÚ˜ ÌÂϤÙ˜ ·¤‰ÂÈÍ·Ó fiÙÈ Ù· ıÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È· Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ÂÓÂÚÁÔÔ›-ËÛ˘ ÂÓfi˜ ÔχÏÔÎÔ˘ 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·ÌÙ· ‰Ú·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 ÂÓÂÚÁÔÔ›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) ÂÈÊ·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 ÂÓÂÚÁÔÔ›ËÛË Î·È ÚÔÛÎfiÏ-ÏËÛË Î·È ÙˆÓ ¿ÏÏˆÓ ¤ÌÌÔÚÊˆÓ ÛÙÔȯ›ˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ (·ÈÌÔÂÙ¿ÏÈ·-Ï¢ÎÔ·ÙÙ·Ú·). ∏ ÌÂϤÙËÙ˘ ·ÏÏËÏ›‰Ú·Û˘ fiÏˆÓ ·˘ÙÒÓ ÙˆÓ ·Ú·ÁfiÓÙˆÓ ‚Ô‹ıËÛ ÛÙËÓ Î·Ù·ÓfiËÛË Ù˘ ·ıÔÁ¤ÓÂÈ·˜ÙˆÓ ·ÁÁÂÈÔıÚÔÌ‚ˆÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ Î·Ï‡ÙÂÚË ·Ú·ÎÔÏÔ‡ıËÛË, ıÂڷ¢ÙÈ-΋ ÚÔÛ¤ÁÁÈÛË, ÔÈfiÙËÙ· ˙ˆ‹˜ Î·È ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ.

§¤ÍÂȘ ÎÏÂȉȿ

¢Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ˜, ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú· ÙÔ˘ stress, ÂÓ‰Ôı‹ÏÈÔ, ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈοÂÂÈÛfi‰È·.

1 °’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞¶£, πÔÎÚ¿ÙÂÈÔ °ÂÓÈÎfi¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢

2 ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞¶£, πÔÎÚ¿ÙÂÈÔ °ÂÓÈÎfi¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢

AÏÏËÏÔÁÚ·Ê›·:

∂Ï›˙· ∫·Ú·Ù˙¿-•ÈÊÈÏ›‰Ô˘ πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌ›ԣÂÛÛ·ÏÔӛ΢ ∫ˆÓÛÙ·ÓÙÈÓÔ˘fiψ˜ 49 ∆.∫. 546 42, £ÂÛÛ·ÏÔÓ›ÎË

∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 03-09-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 01-04-2005

¶·È‰È·ÙÚÈ΋ 2005;68:329-335

™˘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΋ ·ÈÌÔÂÙ·Ïȷ΋

GPIV ÁÏ˘ÎÔÚˆÙ½ÓË πV (Platelet

glycoprotein IV)

GPIb °Ï˘ÎÔÚˆÙ½ÓË Ib (Glycoprotein Ib)

GPIIb/IIIa °Ï˘ÎÔÚˆÙ½ÓË (b3 ÈÓÙÂÁÎÚ›ÓË)

(Glycoprotein)

VNR ∂Ó‰ÔıËÏÈ·Îfi˜ ˘Ô‰Ô¯¤·˜ Ù˘

‚ÈÙÚÔÓÂÎÙ›Ó˘ (Vitronectin receptor)

CD44 ¶ÚˆÙÂÔÁÏ˘Î¿ÓË Ì 5 ÈÛÔÌÂÚ‹

(Proteoglycan)

FcR Àԉԯ‡˜ ÂÓ‰ÔıËÏÈ·ÎÔ‡ ΢ÙÙ¿ÚÔ˘

(Fragments counter receptor)

B-CAM/Lu ¶ÚˆÙÂ˚ÓÈÎfi ÌfiÚÈÔ ÚÔÛÎfiÏÏËÛ˘ ‚·ÛÈ΋˜ ÌÂÌ‚Ú¿Ó˘ (Basal celladhesion 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¿

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·329

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330 ∂. ∫·Ú·Ù˙¿ - •ÈÊÈÏ›‰Ô˘ Î·È Û˘Ó.

∂ÈÛ·ÁˆÁ‹

∏ ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ˜ Â›Ó·È Ì›· ·fi

ÙȘ Û˘¯ÓfiÙÂÚ˜ ÎÏËÚÔÓÔÌÈΤ˜ ·ÈÌÔÛÊ·ÈÚÈÓÔ¿-

ıÂȘ. O Ingram, ÙÔ 1956, ·¤‰ÂÈÍ fiÙÈ Ë ÌÔÚÈ·-

΋ ‚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˘ ·ÚÁfiÙÂ-

Ú· ·Ô‰Â›¯ıËΠfiÙÈ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ÛËÌÂÈ·-

΋˜ ÌÂÙ¿ÏÏ·Í˘ (ÛÙÔ Îˆ‰ÈÎfiÓÈÔ GAG Û GTG)

ÛÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ı¤ÛË Ù˘ ÚÒÙ˘ ÂÎÊÚ·˙fiÌÂ-

Ó˘ ·ÏÏËÏÔ˘¯›·˜ ÙÔ˘ ‚-ÁÔÓȉ›Ô˘ (1-4). ∞ÔÙ¤-

ÏÂÛÌ· Ù˘ ÌÂÙ¿ÏÏ·Í˘ ·˘Ù‹˜ Â›Ó·È Ô Û¯ËÌ·ÙÈ-

ÛÌfi˜ Ù˘ HbS, Ë ÔÔ›· ÛÂ Û˘Óı‹Î˜ ˘Ô͢ÁÔ-

Ó·ÈÌ›·˜ ÂÌÊ·Ó›˙ÂÈ ÌÂȈ̤ÓË ‰È·Ï˘ÙfiÙËÙ·, Ô-

Ï˘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·-

ÌÙˆÓ ‰Ú·ÓÔ΢ÙÙ¿ÚˆÓ (2,5,6).

∏ ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È

΢ڛˆ˜ ·fi ÂÒ‰˘Ó· ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈ-

Ûfi‰È·, Ô˘ fiÙ·Ó Û˘Ì‚Ô‡Ó ÛÙÔ˘˜ Ó‡ÌÔÓ˜ ‹

ÛÙÔ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· (∫¡™) ›ӷÈ

·ÂÈÏËÙÈο ÁÈ· ÙË ˙ˆ‹ ÙˆÓ ·Û¯fiÓÙˆÓ (1,2,5).

∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ë ·ıÔÊ˘ÛÈÔÏÔÁ›·

·˘ÙÒÓ ÙˆÓ ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ

¤ÁÈÓ ·ÓÙÈΛÌÂÓÔ ÂÎÙÂÙ·Ì¤ÓˆÓ ÌÂÏÂÙÒÓ, ηı’

fiÛÔÓ ·Ô‰Â›¯ıËΠfiÙÈ Ë Â¤ÏÂ˘Û‹ ÙÔ˘˜ ‰ÂÓ Â›-

Ó·È ·ÔÙ¤ÏÂÛÌ· Ù˘ ·ÁÁÂȷ΋˜ ·fiÊڷ͢ Ìfi-

ÓÔ ·fi Ù· ¿Î·ÌÙ· ·Ú·ÌÔÚʈ̤ӷ ‰Ú·ÓÔ-

·ÙÙ·Ú·, ·ÏÏ¿ Î·È Ù˘ ÂÓÂÚÁÔÔ›ËÛ˘ Î·È ·Ï-

ÏËÏ›‰Ú·Û˘ ÔÏÏÒÓ ¿ÏÏˆÓ ·Ú·ÁfiÓÙˆÓ,

fiˆ˜ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ ÙÔ˘ stress, Ï¢-

ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ, ·ÈÌÔÂÙ·Ï›ˆÓ, ÔÚÈṲ̂ӈÓ

·Ú·ÁfiÓÙˆÓ Ù˘ ‹Í˘ ÙÔ˘ Ï¿ÛÌ·ÙÔ˜, ÙÔ˘

·ÁÁÂÈ·ÎÔ‡ ÂÓ‰ÔıËÏ›Ô˘ Î·È ÙˆÓ Î·ÏÔ‡ÌÂÓˆÓ

ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ (4). OÈ

ÏÔÈÌÒÍÂȘ Î·È Ë ˘ÔÍ›· ˘ÚÔ‰ÔÙÔ‡Ó ˆ˜ › ÙÔ

Ï›ÛÙÔÓ ÙËÓ ¤Ó·ÚÍË ÙˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÂÂÈ-

ÛÔ‰›ˆÓ Ô˘ Ô‰ËÁÔ‡Ó Û ÈÛÙÈ΋ ÈÛ¯·ÈÌ›· Î·È fi-

ÓÔ ÛÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ÂÚÈÔ¯‹ (1,2,7-9).

¶·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ‰Ú¿ӈÛ˘

∆· ıÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È· ÛÙË ‰Ú·ÓÔ΢Ù-

Ù·ÚÈ΋ ÓfiÛÔ Û˘Ì‚·›ÓÔ˘Ó ˆ˜ › ÙÔ Ï›ÛÙÔÓ

ÛÙÔ Â›Â‰Ô Ù˘ ÌÈÎÚÔ΢ÎÏÔÊÔÚ›·˜, Ì ·ÚÈ-

Ô˘˜ ˘Â‡ı˘ÓÔ˘˜ ÛÙËÓ ¤Ó·ÚÍ‹ ÙÔ˘˜ Ù· ÏÈÁfiÙÂÚÔ

˘ÎÓ¿ Î·È ·Ú·ÌÔÚʈ̤ӷ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·Ù-

Ù·Ú·, Ù· ηÏÔ‡ÌÂÓ· ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú·

(¢∂∫) ÙÔ˘ stress. ∆· ¢∂∫ ÙÔ˘ stress ÚÔÛÎÔÏ-

ÏÒÓÙ·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˜, ¤گÂÙ·È Ì˯·ÓÈ΋ ·Á›‰Â˘ÛË

ÙˆÓ ‰Ú·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‰Ô-

ıËÏȷ΋ 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· ·ÏÏËÏ›‰Ú·Û˘

ÔÏÏÒÓ ·Ú·ÁfiÓÙˆÓ (7,8).

OÈ ·Ú¿ÁÔÓÙ˜ ·˘ÙÔ› ›ӷÈ:

·) ∆· ÂÈÊ·ÓÂȷο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘

ÙˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ (΢ڛˆ˜ ÙˆÓ ¢∂∫) (cell

adhension molecules ‹ ICAM) Î·È ÙˆÓ ÂÓ‰ÔıË-

ÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ (vascular cell adhesion

molecules ‹ VCAM1) (7,11,12). ∞fi ÙËÓ Î·ÙË-

ÁÔÚ›· ·˘Ù‹, ÛÙË ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ Û˘Ì-

ÌÂÙ¤¯Ô˘Ó: i) Ë ˘ÂÚÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ ÛÂÏÂÎÙÈÓÒÓ

(E Selectin, L selectin, P selectin) (7), ii) Ë ˘Â-

ÚÔÈÎÔÁ¤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 ÈÓÙÂ-

ÁÎÚ›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˜ ¿Ï-

Ϙ ÔÌ¿‰Â˜ ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ, fiˆ˜ Ù·

CD36, CD44, ıÚÔÌ‚ÔÛÔÓ‰›ÓË Î·È ˘„ËÏÔ‡ ÌÔ-

ÚÈÎÔ‡ ‚¿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 ·ÏÏËÏÂÈ-

‰Ú¿ÛÂȘ ÙˆÓ ‰Ú·ÓÔ΢ÙÙ¿ÚˆÓ Î·È ÂÓ‰ÔıËÏÈ·-

ÎÒÓ Î˘ÙÙ¿ÚˆÓ Ô‰ËÁÔ‡Ó Û ·ÏÏÔ›ˆÛË ÙÔ˘ ÂÓ‰Ô-

ıËÏ›Ô˘, ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ Ù˘ ·ÁÁÂÈÔÛ˘ÛÙ·Ï-

ÙÈ΋˜ ÂÓ‰ÔıËÏ›Ó˘ 1 (Et1) Î·È ÌÂȈ̤ÓË ÙÔ˘ ·Á-

ÁÂÈԉȷÛÙ·ÏÙÈÎÔ‡ ÌÔÓÔÍÂȉ›Ô˘ ÙÔ˘ ·˙ÒÙÔ˘

(¡O), ÂÈ‚Ú·‰‡ÓÔÓÙ·˜ Ì ·˘ÙfiÓ ÙÔÓ ÙÚfiÔ ÙË

ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜ Î·È ÂȉÂÈÓÒÓÔÓÙ·˜ ÙÔ ıÚÔÌ-

‚ˆÙÈÎfi ÂÂÈÛfi‰ÈÔ (3,6,7,14,15). ™ÙËÓ Âȉ›ӈ-

ÛË ÙÔ˘ ıÚÔÌ‚ˆÙÈÎÔ‡ ÂÂÈÛÔ‰›Ô˘ Â›Ó·È Èı·Ófi Ó·

Û˘Ì‚¿ÏÏÔ˘Ó Î·È Ù· ¯·ÌËÏ¿ ›‰· Ù˘ ·ÁÁÂÈÔ-

‰È·ÛÙ·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˘, ÂÈ‚Â-

‚·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 ÊÈÌÚÔÓÂÎÙ›ÓË, Ë ıÚÔÌ‚ÔÛÔÓ‰›ÓË Î·È Ô

¶·È‰È·ÙÚÈ΋ 2005;68:329-335

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·330

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331∞ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È· ÛÙË ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ

·Ú¿ÁˆÓ Von Willebrand (vWF). ™˘ÌϤÁÌ·Ù·

ıÚÔÌ‚›Ó˘-·ÓÙÈıÚÔÌ‚›Ó˘ ‚Ú¤ıËÎ·Ó ·˘ÍË̤ӷ

ÛÙÔ Ï¿ÛÌ· ‰Ú·ÓÔ΢ÙÙ·ÚÈÎÒÓ ·ÛıÂÓÒÓ, ·Îfi-

ÌË Î·È Û ʿÛÂȘ ËÚÂÌ›·˜ Ù˘ ÓfiÛÔ˘ (7,18,19).

‰) ∆· Ï¢ÎÔ·ÙÙ·Ú·, ›Û˘, Û˘ÌÌÂÙ¤¯Ô˘Ó

ÛÙ· ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È· ›Ù Ì ÙËÓ

ÚÔÛÎfiÏÏËÛ‹ ÙÔ˘˜ ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ, ›Ù ÂÓÂÚ-

ÁÔÔÈÒÓÙ·˜ ÙËÓ ¤ÎÊÚ·ÛË ÙˆÓ ÌÔÚ›ˆÓ ÚÔÛÎfi-

ÏËÛ˘ (20-22).

¶·Ú¿ÏÏËÏ· Ì ÙȘ ÂÓ‰Ô΢ÙÙ¿ÚȘ ÏÂÈÙÔ˘Ú-

ÁÈΤ˜ ÌÂÙ·‚ÔϤ˜ (·ÏÏÔȈ̤ÓË ÔÏÈÎfiÙËÙ· HbS

ÏfiÁˆ Ù˘ ˘‰ÚfiÊԂ˘ ‚·Ï›Ó˘ Î·È Û¯ËÌ·ÙÈÛÌfi˜

ÔÏ˘ÌÂÚÒÓ), ¤گÂÙ·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˘ ¢ηÌÙfiÙËÙ·˜

ÙˆÓ ‰Ú·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˘ ÂÓÂÚÁÔÔÈ›ٷÈ

·fi ÙËÓ ÚfiÛηÈÚË ÂÈÛÚÔ‹ ÈfiÓÙˆÓ Ca++ ÂÓÙfi˜

ÙÔ˘ ΢ÙÙ¿ÚÔ˘) Î·È Â¤Ú¯ÂÙ·È ÂÚ·ÈÙ¤Úˆ ÂÈ-

‰Â›ÓˆÛË Ù˘ ÂÓ‰Ô΢ÙÙ¿ÚÈ·˜ ·Ê˘‰¿ÙˆÛ˘ ηÈ

Ù˘ ‰Ú¿ӈÛ˘ (2,4,7,23) (∂ÈÎfiÓ· 1).

¡ÂfiÙÂÚ˜ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ Î·È ¿ÏÏÔÈ ·-

Ú¿ÁÔÓÙ˜ ÂÎÙfi˜ ·fi ÙÔ˘˜ ·ÓˆÙ¤Úˆ Û˘Ì‚¿Ï-

ÏÔ˘Ó ÛÙËÓ ·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ‰Ú¿ӈÛ˘.∆· Ó·ڿ ÂÚ˘ıÚÔ·ÙÙ·Ú· (ÏÈÁfiÙÂÚÔ ˘ÎÓ¿ ηȷڷÌÔÚʈ̤ӷ), Ù· ÔÓÔÌ·˙fiÌÂÓ· ¢∂∫ ÙÔ˘stress, ¤¯Ô˘Ó ÛÙËÓ ÂÈÊ¿ÓÂÈ¿ ÙÔ˘˜ ÚˆÙ½Ó˜(ÂÈÊ·ÓÂȷο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘) Ô˘ ¯ÚË-ÛÈÌÂ‡Ô˘Ó ÁÈ· ÙË ÌÂٷ͇ ÙÔ˘˜ Û‡Ó‰ÂÛË ÛÙÔÓÌ˘ÂÏfi Î·È Ù· ÔÔ›· ‰ÂÓ ·Ó¢ڛÛÎÔÓÙ·È ÛÙËÓÂÈÊ¿ÓÂÈ· ÙˆÓ ÒÚÈÌˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ(4,5,7,13). ™˘ÁÎÂÎÚÈ̤ӷ, Ë ÈÓÙÂÁÎÚ›ÓË alpha 4beta 1 (·4‚1) ‹ VLA4 ·Ó·ÁÓˆÚ›˙ÂÈ ¿ÌÂÛ· ηÈÚÔÛÎÔÏÏ¿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-4Â›Ó·È ÙÔ ÌfiÓÔ ÌfiÚÈÔ Ô˘ ·Ó·ÁÓˆÚ›˙ÂÙ·È ·fi Ù·VCAM1. ∆o ÌfiÚÈÔ CD36, Ô˘ ÔÓÔÌ¿˙ÂÙ·È Î·È·ÈÌÔÂÙ·Ïȷ΋ ÁÏ˘ÎÔÚˆÙ½ÓË (GPIV), ÂÎ-ÊÚ¿˙ÂÙ·È ÛÙ· ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú· ÙÔ˘ stressÎ·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‚ÔÛÔÓ‰›ÓË Ô˘·Ú¿ÁÂÙ·È ·fi Ù· ÂÓÂÚÁÔÔÈË̤ӷ ·ÈÌÔÂÙ¿-ÏÈ· (7,10,24). ∞˘Ù¤˜ ÔÈ ‰‡Ô ˘„ËÏ‹˜ Û˘Ó¿ÊÂÈ·˜·ÓÙȉڿÛÂȘ Â›Ó·È ÔÈ Ï¤ÔÓ ˘Â‡ı˘Ó˜ ÁÈ· ÙËÓÚÔÛÎfiÏÏËÛË ÙˆÓ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ ÛÙÔÂÓ‰Ôı‹ÏÈÔ ÙˆÓ ÌÂÙ·ÙÚȯÔÂȉÈÎÒÓ ÊÏ‚ȉ›ˆÓ.∂ÈϤÔÓ, ˘„ËÏÔ‡ ÌÔÚÈ·ÎÔ‡ ‚¿ÚÔ˘˜ ÌfiÚÈ· ÙÔ˘vWF Û˘ÌÌÂÙ¤¯Ô˘Ó ˆ˜ Á¤Ê˘Ú˜ ÛÙËÓ ÚÔÛÎfiÏ-ÏËÛË ÙÔ˘ GPππb/πππ· ÂÈÊ·ÓÂÈ·ÎÔ‡ ÌÔÚ›Ô˘ÚÔÛÎfiÏÏËÛ˘ ÙÔ˘ ¢∂∫ Ì ÙÔ GP1b ‹ ÙÔÓ VNR(ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ ÙÔ˘ ÂÓ‰ÔıËÏÈ·ÎÔ‡ ΢Ù-Ù¿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΢ÙÙ·ÚÈ΋ ÌÂÌ‚Ú¿ÓË Î·È Û‡Ó‰ÂÛ‹˜ ÙÔ˘˜ ̤ۈÙ˘ IgG ·ÓÔÛÔÛÊ·ÈÚ›Ó˘ Ì ÙÔ˘˜ (FcR) ˘Ô‰Ô-¯Â›˜ ÙÔ˘ ÂÓ‰ÔıËÏÈ·ÎÔ‡ ΢ÙÙ¿ÚÔ˘, ¯ˆÚ›˜ f˘Ùfi Ó· ·›˙ÂÈ ·ÍÈfiÏÔÁÔ ÚfiÏÔ ÛÙËÓ ¤Ó·ÚÍË ÙÔ˘

¶·È‰È·ÙÚÈ΋ 2005;68:329-335

¶›Ó·Î·˜ 1. ∂ÈÊ·ÓÂȷο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ Ô˘ ÂÓÂÚÁÔ-ÔÈÔ‡ÓÙ·È ÛÙ· ·ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È· Ù˘ ‰Ú·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 ·ÈÌÔÂÙ·-Ï›ˆÓ) ÀÂÚÔÈÎÔÁ¤ÓÂÈ· ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ: ηÙËÁÔÚ›· ÌÔÚ›ˆÓÚÔÛÎfiÏÏËÛ˘ [Immunoglobulin gene superfamily ‹ IgG gene (C2

type)] ÀÂÚÔÈÎÔÁ¤ÓÂÈ· ÈÓÙÂÁÎÚÈÓÒÓ (Integrine superfamily)‚1 ÈÓÙÂÁÎÚ›ÓË ‹ alpha4 beta1 ‹ VLA4

‚3 ÈÓÙÂÁÎÚ›ÓË ‹ ÁÏ˘ÎÔÚˆÙ½ÓË GPIIb/πππ· ‚3 ÈÓÙÂÁÎÚ›ÓË ‹ ÁÏ˘ÎÔÚˆÙ½ÓË GPIb Àԉԯ‡˜ Ù˘ ‚ÈÙÚÔÓÂÎÙ›Ó˘ ‹ VNR ¢È¿ÊÔÚ· (Miscellaneous group) CD36 ‹ GPIV (·ÈÌÔÂÙ·Ïȷ΋ ÁÏ˘ÎÔÚˆÙ½ÓË) CD44 ‹ ÚˆÙÂÔÁÏ˘Î¿ÓË CD44

µ-CAM/Lu (ÌfiÚÈÔ ÚÔÛÎfiÏÏËÛ˘ ‚·ÛÈ΋˜ ÌÂÌ‚Ú¿Ó˘) FcR (˘Ô‰Ô¯Â›˜ ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ)Thrombospondin (ıÚÔÌ‚ÔÛÔÓ‰›ÓË)À„ËÏÔ‡ ÌÔÚÈ·ÎÔ‡ ‚¿ÚÔ˘˜ ÔÏ˘ÌÂÚ‹ ÙÔ˘ ·Ú¿ÁÔÓÙ·Von Willebrand (HMW-vWF)

�O2 �P∏HbS ÔÏ˘ÌÂÚ‹

H2O

¢›·˘ÏÔ˜ Gardos

K+Ca++� �

��

� K+Ca++

H2O H2O

™˘ÌÌÂÙ·ÊÔÚ¿ K-Cl

Cl-K+

Cl-K+

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

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·331

Page 18: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

332 ∂. ∫·Ú·Ù˙¿ - •ÈÊÈÏ›‰Ô˘ Î·È Û˘Ó.

ıÚÔÌ‚ˆÙÈÎÔ‡ ÂÂÈÛÔ‰›Ô˘ (4,13,16). ∂ÈϤÔÓ,

‚Ú¤ıËÎÂ Û˘Ó¿ÊÂÈ· ·Ó¿ÌÂÛ· ÛÙÔ B-CAM/Lu

ÂÈÊ·ÓÂÈ·Îfi ÌfiÚÈÔ ÙÔ˘ ÒÚÈÌÔ˘ ‰Ú·ÓÔ΢ÙÙ¿-

ÚÔ˘ Î·È Ù˘ ˘ÂÓ‰ÔıËÏȷ΋˜ Ï·ÌÈÓ›Ó˘, ·Ô-

‰ÂÈÎÓ‡ÔÓÙ·˜ ÙËÓ ÂÌÏÔ΋ 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 ηÈ

ÛÙËÓ ·ÎfiÏÔ˘ıË ·Á›‰Â˘ÛË ÙˆÓ ‰Ú·ÓÔ΢ÙÙ¿-

ÚˆÓ (4,7,13).

∆·˘Ùfi¯ÚÔÓ· ÌÂ ÙËÓ ÚÔÛÎfiÏÏËÛË ÙˆÓ ÂÚ˘-

ıÚÔ΢ÙÙ¿ÚˆÓ Î·È ÙËÓ ÂÓ‰ÔıËÏȷ΋ ‚Ï¿‚Ë,

ÂÓÂÚÁÔÔÈÂ›Ù·È Î·È Ë ‰È·‰Èηۛ· Ù˘ ‹Í˘.

∂ÎÙfi˜ ·fi ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ·Ú¿ÁÔÓÙ· vWF,

·˘ÍË̤ӷ ›‰· ÈÓˆ‰ÔÁfiÓÔ˘, ÊÈÌÚÔÓÂÎÙ›-

Ó˘, ÚÔıÚÔÌ‚›Ó˘ Î·È Û˘ÌÏÂÁÌ¿ÙˆÓ ıÚÔÌ‚›-

Ó˘-·ÓÙÈıÚÔÌ‚›Ó˘ πππ ‚Ú¤ıËÎ·Ó ÙfiÛÔ Î·Ù¿ ÙË

‰È¿ÚÎÂÈ· ÙˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÎÚ›ÛÂˆÓ fiÛÔ Î·È

ηٿ ÙË Ê¿ÛË ‡ÊÂÛ˘ Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜

(7,18,27). ™ÙËÓ ˘ÂÚËÎÙÈ΋ ηٿÛÙ·ÛË Û˘Ì-

‚¿ÏÏÂÈ Ë Ù·˘Ùfi¯ÚÔÓË ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÈÌÔ-

ÂÙ·Ï›ˆÓ Î·È Ë ·˘ÍË̤ÓË ·ÂÏ¢ı¤ÚˆÛË ·fi

·˘Ù¿ ıÚÔÌ‚ÔÛÔÓ‰›Ó˘, ƒ ÛÂÏÂÎÙ›Ó˘, ‚-ıÚÔÌ-

‚ÔÁÏÔ‚Ô˘Ï›Ó˘ Î·È ÙÔ˘ ·ÈÌÔÂÙ·ÏÈ·ÎÔ‡ ·Ú¿-

ÁÔÓÙ· 4, Ô˘ ‰ÚÔ˘Ó Û˘ÓÂÚÁÈο ÛÙËÓ Âȉ›ӈ-

ÛË ÙÔ˘ ıÚÔÌ‚ˆÙÈÎÔ‡ ÂÂÈÛÔ‰›Ô˘ (4,27-30). ∆·

ÂÓÂÚÁÔÔÈË̤ӷ Î·È Î˘ÎÏÔÊÔÚÔ‡ÓÙ· ÂÓ‰ÔıË-

Ïȷο ·ÙÙ·Ú· ÛÙÔ˘˜ ¿Û¯ÔÓÙ˜ ·fi ‰Ú·ÓÔ-

΢ÙÙ·ÚÈ΋ ÓfiÛÔ, Ë ‰È·Ù·Ú·¯‹ Ù˘ ·Û˘ÌÌÂÙÚ›·˜

ÙˆÓ ÊˆÛÊÔÏÈȉ›ˆÓ Ù˘ ÌÂÌ‚Ú¿Ó˘ ÙˆÓ ‰ÚÂ-

·ÓÔ΢ÙÙ¿ÚˆÓ Î·È Ë ¤ÎıÂÛË Ù˘ ·ÓÈÔÓÈ΋˜ ʈ-

ÛÊ·ÙȉÈÏÔÛÂÚ›Ó˘ (phosphatidylserine PS)

·ÔÙÂÏÔ‡Ó ¤Ó·Ó ÂÈϤÔÓ ÈÛÙÈÎfi ·Ú¿ÁÔÓÙ·,

˘Â‡ı˘ÓÔ ÁÈ· ÙËÓ ÂÓÂÚÁÔÔ›ËÛË Ù˘ ‹Í˘ ηÈ

ÙË “ıÚÔÌ‚ÔÊÈÏÈ΋ ηٿÛÙ·ÛË” ÛÙË ‰Ú·ÓÔ-

΢ÙÙ·ÚÈ΋ ÓfiÛÔ (4,17,27,28).

ŸÛÔÓ ·ÊÔÚ¿ ÛÙ· ¿ÏÏ· ¤ÌÌÔÚÊ· ÛÙÔȯ›· ÙÔ˘

·›Ì·ÙÔ˜, ‚Ú¤ıËΠfiÙÈ ‰ÂÓ Ì¤ÓÔ˘Ó ·Ì¤ÙÔ¯· ÛÙËÓ

ÂΉ‹ÏˆÛË Ù˘ ÂÒ‰˘Ó˘ ÎÚ›Û˘. ∆· ÔÏ˘ÌÔÚ-

ÊÔ‡ÚËÓ· Ô˘‰ÂÙÂÚfiÊÈÏ· Î·È Ù· ÌÔÓÔ·ÙÙ·Ú·

Â›Ó·È Â›Û˘ ÂÓÂÚÁÔÔÈË̤ӷ ÛÙË ‰Ú·ÓÔ΢Ù-

Ù·ÚÈ΋ ÓfiÛÔ (20,22,31). ªÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ Ù·

Ï¢ÎÔ·ÙÙ·Ú· ÛÙË ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ ·-

Ú¿ÁÔ˘Ó ÙÔ˘˜ ‰È·Ï˘ÙÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÈÓÙÂÚÏ¢Λ-

ÓË 1 Î·È ·Ú¿ÁÔÓÙ· Ó¤ÎÚˆÛ˘ fiÁÎÔ˘-a (IL1 ηÈ

∆¡Fa), Ô˘ ‰ÈÂÁ›ÚÔ˘Ó ÙËÓ ¤ÎÊÚ·ÛË ÙˆÓ ÂÈÊ·-

ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ηÈ, ÛÙË Û˘Ó¤-

¯ÂÈ·, Ù· ÂÓÂÚÁÔÔÈË̤ӷ Ô˘‰ÂÙÂÚfiÊÈÏ· ÚÔ-

ÛÎÔÏÏÒÓÙ·È ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ ÌÂ ÙË ÌÂÛÔÏ¿‚ËÛË

ÙˆÓ ÛÂÏÂÎÙÈÓÒÓ, ȉȷ›ÙÂÚ· Ù˘ ∂ ÛÂÏÂÎÙ›Ó˘, ÙˆÓ

ÈÓÙÂÁÎÚÈÓÒÓ Î·È ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ (4,7,10,

21). ∂ÈϤÔÓ, ÔÈ ÈÓÙÂÁÎÚ›Ó˜ Â›Ó·È ˘Â‡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). ∏ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÔÏ˘-

ÌÔÚÊÔ‡ÚËÓˆÓ Î·È Ë ÚÔÛÎfiÏÏËÛ‹ ÙÔ˘˜ ÛÙÔ

ÂÓ‰Ôı‹ÏÈÔ Ô‰ËÁ› Û ÂÈ‚Ú¿‰˘ÓÛË Ù˘ ÚÔ‹˜ ÙÔ˘

·›Ì·ÙÔ˜ ÛÙË ÌÈÎÚÔ΢ÎÏÔÊÔÚ›· (4,32). §fiÁˆ ·˘-

Ù‹˜ Ù˘ Û˘ÌÌÂÙÔ¯‹˜ ÙˆÓ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ

ÛÙ· ·ÁÁÂÈÔıÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È·, Ô ˘„ËÏfi˜

·ÚÈıÌfi˜ ÙÔ˘˜ ·ÔÙÂÏ› ¤Ó·Ó ȉȷ›ÙÂÚ· ÂÈΛӉ˘-

ÓÔ ‰Â›ÎÙË ÛÙÔ˘˜ ¿Û¯ÔÓÙ˜ ·fi ‰Ú·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 ÌÂȈ̤-

ÓË ·Ú·ÁˆÁ‹ ÙÔ˘ ·ÁÁÂÈԉȷÛÙ·ÏÙÈÎÔ‡ ÌÔÓÔÍÂÈ-

‰›Ô˘ ÙÔ˘ ·˙ÒÙÔ˘ (¡O) (5,7,10,33).

O ÚfiÏÔ˜ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÛÙ· ıÚÔÌ‚ˆÙÈο

ÂÂÈÛfi‰È·

¶Ú¤ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ Ë ¤ÎÊÚ·ÛË ÙˆÓ ÂÈ-

Ê·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ÙˆÓ ÂÓ‰Ô-

ıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È È‰È·›ÙÂÚ· ÙÔ˘ VCAM1,

·˘Í¿ÓÂÙ·È fiÙ·Ó ÙÔ ÂÓ‰Ôı‹ÏÈÔ ÂÓÂÚÁÔÔÈËı›

·fi ÙȘ ÚÔÊÏÂÁÌÔÓÒ‰ÂȘ ΢ÙÔΛÓ˜ IL1 ηÈ

TNFa, ÁÂÁÔÓfi˜ Ô˘ ‰ÈηÈÔÏÔÁ› ÙÔÓ ÌÂÁ¿ÏÔ

ÚfiÏÔ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÛÙËÓ ·ıÔÊ˘ÛÈÔÏÔÁ›·

Ù˘ ‰Ú·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 ¤Î-

ÊÚ·ÛË ÙˆÓ ÂÈÊ·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏË-

Û˘, ‚Ú¤ıËÎ·Ó Û ·˘ÍË̤ӷ ›‰· Û ·ÛıÂ-

Ó›˜ Ì ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ, ·ÎfiÌË Î·È ÛÂ

Ê¿ÛÂȘ ‡ÊÂÛ˘ Ù˘ ÓfiÛÔ˘, ·Ô‰ÂÈÎÓ‡ÔÓÙ·˜ fiÙÈ

¶·È‰È·ÙÚÈ΋ 2005;68:329-335

∂Ó‰

Ôı

ËÏ

È·Î

fi Î

‡ÙÙ

·Ú

Ô

¢∂

∫ S

tres

s

GPIb‹

VNR

CD36

‹VNR

GPIIb/IIIa

CD36

VCAM1 VLA4

HMWvWF

∂ÓÂÚÁÔÔÈË̤ӷ·ÈÌÔÂÙ¿ÏÈ·

£ÚÔÌ‚ÔÛÔÓ‰›ÓË

∂ÈÎfiÓ· 2. ¶ÚÔÛÎfiÏÏËÛË ÙˆÓ ¢∂∫ stress ÛÙÔ ·ÁÁÂÈ·ÎfiÂÓ‰Ôı‹ÏÈÔ (ÚˆÙÔÁÂÓ›˜ ˘„ËÏ‹˜ Û˘Ó¿ÊÂÈ·˜ ·ÓÙȉڿ-ÛÂȘ).

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·332

Page 19: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

333∞ÁÁÂÈÔ·ÔÊÚ·ÎÙÈο ÂÂÈÛfi‰È· ÛÙË ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ

Ù· ·ÁÁ›· ÙˆÓ ·Û¯fiÓÙˆÓ ‚Ú›ÛÎÔÓÙ·È ÌfiÓÈÌ·

Û ÚÔÊÏÂÁÌÔÓÒ‰Ë Î·Ù¿ÛÙ·ÛË (7,10). ªÂ ÙËÓ

›ÛÔ‰Ô ÙÔ˘ ÌÈÎÚÔ‚È·ÎÔ‡ ·Ú¿ÁÔÓÙ· Î·È ÙËÓ

¤Ó·ÚÍË Ù˘ Ïԛ̈͢ ¤گÂÙ·È ÂÓÂÚÁÔÔ›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¢ıÂÚÒ-

ÓÔ˘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‹, ›Û˘, ÚÔ¿ÁÂÈ

ÙËÓ ÚÔÛÎfiÏÏËÛË ÙˆÓ ¢∂∫ ÛÙÔ ÂÓ‰Ôı‹ÏÈÔ ÔÚÈ-

ÛÌ¤ÓˆÓ ·ÛıÂÓÒÓ Ì ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ.

∞˘Ùfi ÔÊ›ÏÂÙ·È ·ÊÂÓfi˜ ÛÙ· ·˘ÍË̤ӷ ›‰·

ÙˆÓ IgE ·ÓÙÈÛˆÌ¿ÙˆÓ Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜,

·ÊÂÙ¤ÚÔ˘ ÛÙËÓ ·˘ÍË̤ÓË ÂÓ‰ÔıËÏȷ΋ ¤ÎÊÚ·-

ÛË Î·È ÛÙ· ˘„ËÏ¿ ›‰· ‰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¿ ›‰· IgE ÛÙËÓ ·ÙÔ-

›· Ô‰ËÁÔ‡Ó ÛÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÈÌÔÂ-

Ù·Ï›ˆÓ Î·È ÛÙËÓ ·ÂÏ¢ı¤ÚˆÛË ıÚÔÌ‚ÔÛÔÓ-

‰›Ó˘ Ô˘ ÌÂÛÔÏ·‚› ÛÙËÓ ÚÔÛÎfiÏÏËÛË ÙˆÓ

¢∂∫ ÛÙ· ÂÓ‰ÔıËÏȷο ·ÙÙ·Ú· (7).

Ÿˆ˜ ‹‰Ë ·Ó·Ê¤ÚıËÎÂ, ÔÈ ÛÔ‚·ÚfiÙÂÚ˜

ÂÈÏÔΤ˜ Ù˘ ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋˜ ÓfiÛÔ˘

·ÊÔÚÔ‡Ó ÛÙ· ıÚÔÌ‚ÔÂÌ‚ÔÏÈο ÂÂÈÛfi‰È· ÙÔ˘

ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (∫¡™) ηÈ

ÛÙÔ Ô͇ ıˆÚ·ÎÈÎfi Û‡Ó‰ÚÔÌÔ.

£ÚÔÌ‚ˆÙÈο ÂÂÈÛfi‰È· ÙÔ˘ ∫¡™

∞ÁÁÂÈÔÁÚ·ÊÈΤ˜ Î·È ÓÂÎÚÔÙÔÌÈΤ˜ ÌÂϤÙ˜

Û ¿Û¯ÔÓÙ˜ ·fi ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ ÓfiÛÔ

·Ó·Ê¤ÚÔ˘Ó fiÙÈ ÛÙÔÓ ÂÁΤʷÏÔ Ù· ·ÁÁÂȷο

ÂÂÈÛfi‰È· ·ÊÔÚÔ‡Ó ÛÙ· ÌÂÁ¿Ï· ·ÁÁ›· Î·È fi¯È

ÛÙË ÌÈÎÚÔ΢ÎÏÔÊÔÚ›· (35). ™Ù· ÌÂÁ¿Ï· ·ÁÁ›·

¤گÂÙ·È ÛÙ¤ÓˆÛË ÙÔ˘ ·˘ÏÔ‡ ÏfiÁˆ ˘ÂÚÏ·-

Û›·˜ ÙÔ˘ ̤ÛÔ˘ ¯ÈÙÒÓ·, ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ Î·È

ÙˆÓ Ï›ˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ. ∏ ‚Ï¿‚Ë ÙÔ˘ ̤ÛÔ˘ ¯È-

ÙÒÓ· ¤¯ÂÈ ¿ÌÂÛË Û¯¤ÛË Ì ٷ ÚÔÛÎÔÏÏÒÌÂÓ·

‰Ú·ÓÔ·ÙÙ·Ú·, Ô˘ ·ÏÏÔÈÒÓÔ˘Ó ÙÔ ÂÓ‰Ôı‹-

ÏÈÔ Î·È ‰Â˘ÙÂÚÔÁÂÓÒ˜ ÚÔηÏÔ‡Ó ÂÓ·fiıÂÛË

·ÈÌÔÂÙ·Ï›ˆÓ. ∆· ·ÈÌÔÂÙ¿ÏÈ· ·ÂÏ¢ıÂÚÒ-

ÓÔ˘Ó ·ÈÌÔÂÙ·ÏÈ·ÎÔ‡˜ ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜

Ô˘ ¢ı‡ÓÔÓÙ·È ÁÈ· ÙËÓ ˘ÂÚÏ·Û›· ÙÔ˘ ̤ÛÔ˘

¯ÈÙÒÓ· Ô˘ Ô‰ËÁ› ÛÙËÓ ·ÁÁÂȷ΋ ÛÙ¤ÓˆÛË, ÙË

ÛÙÚÔ‚ÈÏÒ‰Ë ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜, ÛÙËÓ ÂÈϤÔÓ

‚Ï¿‚Ë ÙÔ˘ ̤ÛÔ˘ ¯ÈÙÒÓ· Î·È ÛÙËÓ Âȉ›ӈÛË

Ù˘ ·fiÊڷ͢ (35-37).

O͇ ıˆÚ·ÎÈÎfi Û‡Ó‰ÚÔÌÔ

∏ ÂÌÊ¿ÓÈÛË ÙÔ˘ ÔͤԘ ıˆÚ·ÎÈÎÔ‡ Û˘Ó‰Úfi-

ÌÔ˘ ÔÊ›ÏÂÙ·È: ·) ÛÙËÓ È‰È·›ÙÂÚË Â˘·ÈÛıËÛ›·

Ù˘ Ó¢ÌÔÓÈ΋˜ ÌÈÎÚÔ΢ÎÏÔÊÔÚ›·˜ ÛÙËÓ ˘Ô-

Í›· Î·È ‚) ÛÙËÓ ·Ú·ÁˆÁ‹ ÚÔÊÏÂÁÌÔÓˆ‰ÒÓ

΢ÙÔÎÈÓÒÓ Î·È ÙËÓ ·˘ÍË̤ÓË ¤ÎÊÚ·ÛË ÙˆÓ ÂÈ-

Ê·ÓÂÈ·ÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘ ÛÙ· Ó¢-

ÌÔÓÈο ÂÓ‰ÔıËÏȷο ·ÙÙ·Ú· ˆ˜ ·¿ÓÙËÛË

ÛÙËÓ ˘ÔÍ›· (7,9,38-42). ∂ÎÏ˘ÙÈÎfi˜ ·Ú¿ÁÔ-

ÓÙ·˜ 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˘ÛÙÔ-

Ï‹, Ì›ˆÛË Ù˘ ·ÈÌ·ÙÈ΋˜ ÚÔ‹˜, ·Á›‰Â˘ÛË Î·È

·ÈÌfiÏ˘ÛË ‰Ú·ÓÔ΢ÙÙ¿ÚˆÓ, ·Ú·ÁˆÁ‹ Âχ-

ıÂÚˆÓ ÚÈ˙ÒÓ Î·È Âȉ›ӈÛË Ù˘ ıÚÔÌ‚ˆÙÈ΋˜

ÎÚ›Û˘ (9).

∏ ÔÚ›· ·˘Ù‹ ÂÈ‚·Ú‡ÓÂÙ·È ÏfiÁˆ ¤ÏÏÂÈ-

„˘ ΢ÙÙ·ÚÔÚÔÛٷ٢ÙÈÎÒÓ ÌÂÛÔÏ·‚ËÙÒÓ,

fiˆ˜ ÙÔ ¡√, ÙÔ˘ ÔÔ›Ô˘ Ë Û‡ÓıÂÛË ·fi ÙÔ ÂÓ-

‰Ôı‹ÏÈÔ ·Ó·ÛÙ¤ÏÏÂÙ·È ÏfiÁˆ Ù˘ ˘ÔÍ›·˜ (44-

48). ∆·˘Ùfi¯ÚÔÓ·, ˘¿Ú¯ÂÈ ·˘ÍË̤ÓË ‚ÚÔÁ¯È΋

·ÓÙȉڷÛÙÈÎfiÙËÙ·, Ô˘ ıˆÚÂ›Ù·È fiÙÈ Â›Ó·È

‰Â˘ÙÂÚÔÁÂÓ‹˜ ÏfiÁˆ Ù˘ ˘ÔÎÏÈÓÈ΋˜ Ó¢ÌÔ-

ÓÈ΋˜ ‚Ï¿‚˘ ·fi Ù· ‰Ú·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). ∂ÈϤÔÓ, ·Ó·Ê¤-

ÚÂÙ·È fiÙÈ ÔÈ ·ÛıÂÓ›˜ Ì ·ÁÁÂÈÔıÚÔÌ‚ˆÙÈ΋ ÎÚ›-

ÛË ‚Ú›ÛÎÔÓÙ·È Û ÚÔÛÙ¿‰ÈÔ ÏÈÒ‰Ô˘˜ ÂÌ‚Ô-

Ï‹˜, ‰ÈfiÙÈ ˘¿Ú¯ÂÈ ·˘ÍË̤ÓË ¤ÎÎÚÈÛË ÂÓfi˜

ÊÏÂÁÌÔÓÒ‰Ô˘˜ ÌÂÛÔÏ·‚ËÙ‹ Ù˘ ʈÛÊÔÏÈ¿-

Û˘ A2 (SPLA2) Ô˘ ·ÂÏ¢ıÂÚÒÓÂÈ ÂχıÂÚ·

ÏÈ·Ú¿ Ôͤ·, ˘Â‡ı˘Ó· ÁÈ· ÙËÓ ÔÍ›· ‚Ï¿‚Ë

ÙÔ˘ Ó‡ÌÔÓ· ÚÈÓ ÂÌÊ·ÓÈÛÙ› ÙÔ Ô͇ ıˆÚ·ÎÈ-

Îfi Û‡Ó‰ÚÔÌÔ. ∏ ·Ó‡ÚÂÛË ·˘ÍËÌ¤ÓˆÓ ÂȤ-

‰ˆÓ SPLA2 Û ¿Û¯ÔÓÙ˜ ·ÔÙÂÏ› ¤Ó‰ÂÈÍË ÌÂ-

Ù¿ÁÁÈÛ˘ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ ‹ ·Ê·ÈÌ·ÍÔ-

ÌÂÙ¿ÁÁÈÛ˘ ÚÔÎÂÈ̤ÓÔ˘ Ó· ÚÔÏËÊı› Ô͇

ıˆÚ·ÎÈÎfi Û‡Ó‰ÚÔÌÔ (49-51).

™˘ÌÂÚ¿ÛÌ·Ù·

∏ ÌÂϤÙË Ù˘ ·ıÔÊ˘ÛÈÔÏÔÁ›·˜ ÙˆÓ ıÚÔÌ-

‚ˆÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ÛÙË ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋

ÓfiÛÔ ÛÙԯ‡ÂÈ ÛÙËÓ ÚfiÏË„Ë Î·È Ì›ˆÛË Ù˘

¶·È‰È·ÙÚÈ΋ 2005;68:329-335

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·333

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334 ∂. ∫·Ú·Ù˙¿ - •ÈÊÈÏ›‰Ô˘ Î·È Û˘Ó.

Û˘¯ÓfiÙËÙ·˜ Î·È Ù˘ ‚·Ú‡ÙËÙ·˜ ÙˆÓ ÂÒ‰˘ÓˆÓ

ÎÚ›ÛˆÓ, Ì ÛÎÔfi ÙË ‚ÂÏÙ›ˆÛË Ù˘ ÔÈfiÙËÙ·˜

˙ˆ‹˜ Î·È ÙËÓ ·Ú¿Ù·ÛË Ù˘ ÂÈ‚›ˆÛ˘ ÙˆÓ ·-

Û¯fiÓÙˆÓ (3,23,42,52,53). π‰È·›ÙÂÚË ÛËÌ·Û›·

¤¯ÂÈ Ë ¤ÁηÈÚË ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ÔͤԘ ıˆÚ·-

ÎÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ Î·È ÙˆÓ ıÚÔÌ‚ˆÙÈÎÒÓ ÂÂÈ-

ÛÔ‰›ˆÓ ÙÔ˘ ∫¡™, Ù· ÔÔ›· Â›Ó·È ‰˘Ó·ÙfiÓ Ó·

Ô‰ËÁ‹ÛÔ˘Ó Û ÌfiÓÈÌË ·Ó·ËÚ›· ‹ ·ÎfiÌË Î·È

ÛÙÔÓ ı¿Ó·ÙÔ.

µÈ‚ÏÈÔÁÚ·Ê›·

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48. Hammerman SI, Kourembanas S, Conca TJ, TucciM, Brauer M, Farber HW. Endothelin-1 productionduring the acute chest syndrome in sickle cell dis-ease. Am J Respir Crit Care Med 1997;156:280-285.

49. Styles LA, Schalkwijk CG, Aarsman AJ, VichinskyEP, Lubin BH, Kuypers FA. Phospholipase A2 levelsin acute chest syndrome of sickle cell disease. Blood1996;87:2573-2578.

50. Styles LA, Aarsman AJ, Vichinsky EP, Kuypers FA.Secretory phospholipase A(2) predicts impendingacute chest syndrome in sickle cell disease. Blood2000;96:3276-3278.

51. Haynes J Jr, Obiako B. Activated polymorphonu-clear cells increase sickle red blood cell retention inlung: role of phospholipids. Am J Physiol Heart CircPhysiol 2002;282:122-130.

52. Morris CR, Kuypers FA, Larkin S, Sweeters N, Si-mon J, Vichinsky EP et al. Arginine therapy: a novelstrategy to induce nitric oxide production in sicklecell disease. Br J Haematol 2000;111:498-500.

53. Kanakoudi-Tsakalidou F, Athanasiou-Metaxa M,Tzimouli V, Koussi A, Taparkou M, Trachana M etal. Effect of cetrizin on circulating indicators of per-sistent inflammation and endothelial cell activationin patients with sickle cell disease. Ped Res1999;45:755.

¶·È‰È·ÙÚÈ΋ 2005;68:329-335

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336 REVIEW ARTICLE

1 3rd Paediatric Clinic,Aristotle University,Ippokration GeneralHospital, Thessaloniki

2 1st Paediatric Clinic,Aristotle University,Ippokration GeneralHospital, Thessaloniki

Correspondence:

∂. Karatza-Xifilidou Ippokration General Hospital 49 Konstantinoupoleos Str. 546 42, Thessaloniki

Date of submission: 03-09-2004Date of approval: 01-04-2005

Paediatriki 2005;68:336

Pathophysiology of vaso-occlusive crises in

sickle cell disease

E. Karatza - Xifilidou1, M. Athanasiou - Metaxa2

Abstract

Sickle cell disease is caused by a mutation which is characterized by the replacement of glutaminicacid by valine in the 6th position of the ‚-chain of haemoglobin (‚6glu→val) and the production ofhaemoglobin S (·2‚2

S). The exclusive characteristic of haemoglobin S, when deoxygenated, ispolymerization of the chains and, as a result, production of dehydrated rigid sickle cells. Until thebeginning of the past decade, the rigid sickle cells were thought to play the exclusive role in thepathogenesis of the painful thrombotic crises of the disease. Newer studies have proved thatthrombotic episodes are the result of the activation of a complex multifactorial mechanism. At theonset of these episodes, stress reticulocytes attach to the endothelium of the vessels in themicrocirculatory system and the rigid sickle cells become trapped in an attempt at their removalfrom circulation. The establishment of the initial thrombus is controlled by: a) factors from the redblood cell membrane, which is damaged by hypoxia, and parallel activation of the plasmacoagulation factors, b) factors from the endothelium of the vessels (antagonism between vasopressorand vasodilator factors), c) surface adhesion molecules of the endothelial cells and the stressreticulocytes, 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 ofall 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 sufferingfrom sickle cell disease.

Key words

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

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·336

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337∞¡∞™∫O¶∏™∏

∞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‹ ÂÚÈÙÒÛÂȘ, 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, η-ıÒ˜ Î·È ÚˆÙÔÎfiÏÏˆÓ ·ÓÙÈÌÂÙÒÈÛ˘.

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 ÛÎÔfi ÙË ‰È¿ÁÓˆÛË ·Ó·ÊÂÚfiÌÂÓ˘ Ê·Ú̷΢ÙÈ΋˜ ·ÏÏÂÚÁ›·˜. OÈ ÚÔ-ÎÏ‹ÛÂȘ ¤Ï·‚·Ó ¯ÒÚ· ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô St. Mary’s ÙÔ˘ §ÔÓ‰›ÓÔ˘. OÈ ÚÔÎÏ‹ÛÂȘ ‹Ù·Ó ΢ڛˆ˜ ·fi ÙÔÛÙfiÌ·, Ì ÂÍ·›ÚÂÛË Ù· Ê¿Ú̷η ÏȉÔη˝ÓË Î·È ÚÔη˝ÓË, fiÔ˘ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó ‰ÂÚÌ·ÙÈοÙÂÛÙ. OÈ ÂÚÈÛÛfiÙÂÚ˜ ‰ÔÎÈ̷ۛ˜ Ú·ÁÌ·ÙÔÔÈ‹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.

∞ÔÙÂϤÛÌ·Ù·: ∞fi ÙÔ˘˜ 6 ·ÛıÂÓ›˜ Ô˘ ›¯·Ó οÔÈ· ·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‹ıËΠ˷˘ÙÔÂÔ›ıËÛË ÁÈ· ÙË ¯Ú‹ÛË ÂÓ·ÏÏ·ÎÙÈÎÒÓ Û΢·ÛÌ¿ÙˆÓ.

™˘ÌÂÚ¿ÛÌ·Ù·: OÈ Ê·Ú̷΢ÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ Ú¤ÂÈ Ó· Ú·ÁÌ·ÙÔÔÈÔ‡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 ÂÈ·ڈÛË Ù˘ ·Í›·˜ ÙÔ˘˜, ÚÈÓ ÙË ÁÂÓ›ÎÂ˘Û‹ ÙÔ˘˜. ∂˘Ú‡ÙÂÚ˜ ÌÂϤÙÂ˜Â›Ó·È ··Ú·›ÙËÙ˜, ÒÛÙ ӷ ‰ڷȈıÔ‡Ó Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ÁÈ· ÙËÓ ÚfiÁÓˆÛË ·˘ÙÒÓ ÙˆÓ ÂÚÈ-ÙÒÛˆÓ.

§¤ÍÂȘ ÎÏÂȉȿ

º·Ú̷΢ÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ, Ê·Ú̷΢ÙÈ΋ ·ÏÏÂÚÁ›·, ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ.

1 ∂ȉÈ΢fiÌÂÓË ¶·È‰È·ÙÚÈ΋˜,St. Mary’s Hospital, London, UK

2 ∆Ì‹Ì· ¶·È‰È·ÙÚÈ΋˜∞ÏÏÂÚÁÈÔÏÔÁ›·˜ ηÈ∞ÓÔÛÔÏÔÁ›·˜, St. Mary’sHospital, London, UK

AÏÏËÏÔÁÚ·Ê›·:

A. ™ÔÊÈ·ÓÔ‡-∫·ÙÛÔ‡ÏË 42 Abbots Park, St. Albans Herts, UK AL1 1TW E-mail: [email protected]

∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 17-09-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 27-04-2005

¶·È‰È·ÙÚÈ΋ 2005;68:337-340

∂ÈÛ·ÁˆÁ‹

OÈ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ (∞∂) ·fi Ê¿Ú-̷η Â›Ó·È ¤Ó· ÛËÌ·ÓÙÈÎfi Úfi‚ÏËÌ· ‰ËÌfi-ÛÈ·˜ ˘Á›·˜, ȉȷ›ÙÂÚ· ÛÙÔÓ ·È‰È·ÙÚÈÎfi ÏË-ı˘ÛÌfi. ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· (3,6), Ë Û˘¯ÓfiÙËÙ·ÙˆÓ ∞∂ Û ·È‰È¿ Ô˘ ÂÍÂÙ¿ÛÙËÎ·Ó Û Â͈ÙÂ-ÚÈο È·ÙÚ›· ‹Ù·Ó 1,46%, ÂÓÒ Û ÓÔÛËÏ¢fiÌÂ-Ó· ·È‰È¿ ÌÂٷ͇ 6,7% Î·È 9,53%, ÂÓÒ ÔÈ ∞∂Ô˘ ·Ô‚·›ÓÔ˘Ó ÌÔÈÚ·›Â˜ ·Ó¤Ú¯ÔÓÙ·È ÛÂ0,32%. ∆Ô 39% ÙˆÓ ∞∂ Ô˘ ··ÈÙÔ‡Ó ÓÔÛË-Ï›·, Â›Ó·È ÛÔ‚·Ú¤˜ ‹ Î·È ·ÂÈÏËÙÈΤ˜ ÁÈ· ÙË˙ˆ‹ (6).

™Â Ì›· ·Ï·ÈfiÙÂÚË ÌÂϤÙË (3), ÔÈ Û˘¯ÓfiÙÂ-Ú˜ ·ÓÙȉڿÛÂȘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙÔ˘˜ ·Îfi-ÏÔ˘ıÔ˘˜ ·Ú¿ÁÔÓÙ˜: ·ÌÔ͢ÎÈÏ›ÓË (Û ÔÛÔ-ÛÙfi 5,1%), ÙÚÈÌÂıÔÚ›ÌË-ÛÔ˘ÏÊÔÌÂıÔÍ·˙fiÏË

(3,4%), ·ÌÈÎÈÏ›ÓË (3,3%), ·Ú¿ÁˆÁ· ·›Ì·ÙÔ˜(2,2%), ÎÂÊ·ÏÔÛÔÚ›Ó˜ (2,1%), ËÌÈÛ˘ÓıÂÙÈ-Τ˜ ÂÓÈÎÈϛӘ (2,1%), ÂÚ˘ıÚÔÌ˘Î›ÓË (2,0%)Î·È ÂÓÈÎÈÏ›ÓË G (1,8%) (1).

™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ Â›Ó·È Ó··Ú·ı¤ÛÂÈ Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ Ê·Ú̷΢ÙÈ-ÎÒÓ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚÔÎÏ‹ÛÂˆÓ ÛÙÔ Û˘-ÁÎÂÎÚÈ̤ÓÔ ÙÌ‹Ì·, ÒÛÙ ӷ ηٷ‰ÂȯÙÔ‡Ó ÔÈÊ·Ú̷΢ÙÈΤ˜ Ô˘Û›Â˜ Ô˘ ÚÔηÏÔ‡Ó Û ÌÂ-Á·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂÈ˜Î·È Ë ÎÏÈÓÈ΋ ·ÓÙ·fiÎÚÈÛË ÙˆÓ ·È‰ÈÒÓ ÛÙȘ‰È·ÁÓˆÛÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ. ∂›Û˘, Ë ÂÚÁ·Û›··ÔÛÎÔ› ÛÙË Û‡ÁÎÚÈÛË ·˘ÙÒÓ ÙˆÓ ·ÔÙÂÏÂ-ÛÌ¿ÙˆÓ Ì ٷ ϤÔÓ ÚfiÛÊ·Ù· ‰Â‰Ô̤ӷ ÛÙˉȿÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Ê·Ú̷΢ÙÈ-ÎÒÓ ·ÏÏÂÚÁÈÒÓ Ô˘ ÂÓÙÔ›˙ÔÓÙ·È Î˘Ú›ˆ˜ ÛÙÔÓÙÔ̤· Ù˘ ·ÓÔÛԉȿÁÓˆÛ˘.

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·337

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338 A. ™ÔÊÈ·ÓÔ‡ - ∫·ÙÛÔ‡ÏË Î·È Û˘Ó.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ

¶·ÚÔ˘ÛÈ¿˙ÂÙ·È Ì›· ·Ó·ÛÎfiËÛË ÙˆÓ Ê·Ú̷΢ÙÈ-

ÎÒÓ ÚÔÎÏ‹ÛÂˆÓ ÛÙÔ ∆Ì‹Ì· ¶·È‰È·ÙÚÈ΋˜ ∞ÏÏÂÚÁ›·˜

Î·È ∞ÓÔÛÔÏÔÁ›·˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ St. Mary’s, ÙÔ ÔÔ›Ô

Â›Ó·È ÙÚÈÙÔ‚¿ıÌÈÔ Î¤ÓÙÚÔ ·Ó·ÊÔÚ¿˜ ÁÈ· ÙËÓ ·È‰È΋ ·Ï-

ÏÂÚÁ›· 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 ¯ÚÔ-

ÓÈÎfi ‰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·Ô-

Ì‹ ÙÔ˘ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ È·ÙÚÔ‡. ŸÏ· Ù· ·È‰È¿ ˘Ô‚Ï‹-

ıËÎ·Ó Û ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘ ÛÙÔ ‡ÔÙÔ Ê¿ÚÌ·ÎÔ

ηÈ/‹ ÛÙÔ ·ÛʷϤ˜ ˘ÔηٿÛÙ·Ùfi ÙÔ˘ (.¯. ÂÚ˘ıÚÔÌ˘-

ΛÓË Û ÂÚ›ÙˆÛË ·ÏÏÂÚÁ›·˜ Û ÂÓÈÎÈÏ›ÓË ‹ ÂÓÈÎÈÏ›-

Ó˘ Û ÂÚ›ÙˆÛË ·ÏÏÂÚÁ›·˜ ÙÚÈÌÂıÔÚ›Ì˘-ÛÔ˘ÏÊÔ-

ÌÂıÔÍ·˙fiÏ˘). ŸÏ˜ ÔÈ ‰ÔÎÈ̷ۛ˜ Ú·ÁÌ·ÙÔÔÈ‹ıË-

Î·Ó Û ı¿Ï·ÌÔ ËÌÂÚ‹ÛÈ·˜ ÓÔÛËÏ›·˜ ˘fi ÙËÓ Â›‚Ï„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˘ ‰fi-

Û˘. ∆Ô ·È‰› ¤·ÈÚÓ ÂÍÈÙ‹ÚÈÔ Ì ԉËÁ›Â˜ Ó· Ï¿‚ÂÈ ÙËÓ

ηÓÔÓÈ΋ ‰fiÛË ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ÁÈ· ¤ÓÙ Ë̤Ú˜, ÚÔ-

ÎÂÈ̤ÓÔ˘ Ó· ·ÔÎÏÂÈÛÙ› Ë ÂÚ›ÙˆÛË Î·ı˘ÛÙÂÚË̤-

Ó˘ ·ÓÙ›‰Ú·Û˘. ∞ÎÔÏÔ˘ıÔ‡Û ›ÛÎÂ„Ë ÛÙ· Â͈ÙÂÚÈ-

ο È·ÙÚ›· ÌÂÙ¿ ·fi 3-4 ‚‰ÔÌ¿‰Â˜. ™ÙËÓ ÂÚ›ÙˆÛË

ÙˆÓ ÙÔÈÎÒÓ ·Ó·ÈÛıËÙÈÎÒÓ, Ú·ÁÌ·ÙÔÔÈÔ‡ÓÙ·Ó ‰ÂÚ-

Ì·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˘ ‰Ô-

ÎÈÌ·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΋ ÂÈÎÔÈÓˆÓ›· Ì ÙÔ˘˜ ÁÔÓ›˜

·˘ÙÒÓ ÙˆÓ ·È‰ÈÒÓ, ηٿ ÙËÓ ÔÔ›· ·¿ÓÙËÛ·Ó Û ÂÚˆ-

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ÙÒÓ ÙˆÓ ‰ÔÎÈÌ·ÛÈÒÓ. ∂ÚˆÙ‹ıËÎ·Ó ÁÈ· ÙËÓ ‡·ÚÍË ‹ fi¯È

·ÓÙ›‰Ú·Û˘ Û ÌÂÙ¤ÂÈÙ· ·ʋ Ì ʿÚÌ·ÎÔ ÛÙÔ ÔÔ›Ô

Ë ÚfiÎÏËÛË ‹Ù·Ó ·ÚÓËÙÈ΋. ™Â ·ÛıÂÓ›˜ Ô˘ ·Ô‰Â›-

¯ÙËΠʷÚ̷΢ÙÈ΋ ·ÏÏÂÚÁ›·, ‰ÈÂÚ¢ӋıËÎÂ Ù˘¯fiÓ

·ÓÙ›‰Ú·ÛË Û Û··ÛÌ· Ô˘ Û˘ÛÙ‹ıËΠ·ÓÙ› ÙÔ˘ Ê·Ú-

Ì¿ÎÔ˘ Ô˘ ÚÔηÏÔ‡Û ·ÏÏÂÚÁ›·. ∂ÈϤÔÓ, ÂÍÂÙ¿ÛÙË-

Î·Ó Ù· Èı·Ó¿ „˘¯ÔÏÔÁÈο ÔʤÏË ÁÈ· ÙËÓ ÔÈÎÔÁ¤ÓÂÈ·,

¤ÙÛÈ fiˆ˜ 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¤·

·fi ·˘Ù¿ ›¯·Ó οÔÈÔ ÈÛÙÔÚÈÎfi ·ÙÔ›·˜

(40,9%) Î·È 5 ›¯·Ó οÔÈÔ ÈÛÙÔÚÈÎfi ·ÏÏÂÚ-

Á›·˜, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Ù˘ ÙÚÔÊÈ΋˜

(22,7%). ∆· ·Ó·ÊÂÚfiÌÂÓ· Û˘ÌÙÒÌ·Ù· Î·È Ù·

ÂÓÔ¯ÔÔÈÔ‡ÌÂÓ· Ê¿Ú̷η Ê·›ÓÔÓÙ·È ÛÙÔÓ ¶›-

ӷη 1 Î·È ÛÙËÓ ∂ÈÎfiÓ· 1, ·ÓÙ›ÛÙÔȯ·.

OÈ ÚÔÎÏ‹ÛÂȘ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó ÛÙ·

·ÎfiÏÔ˘ı· Ê¿Ú̷η: ÂÓÈÎÈÏ›ÓË V 9, ·ÌÔ͢ÎÈÏ›-

ÓË 5, ÂÚ˘ıÚÔÌ˘Î›ÓË 4, ·ÛÈÚ›ÓË 2, ÏȉÔη˝ÓË 1,

ÚÔη˝ÓË 1, ÙÚÈÌÂıÔÚ›ÌË-ÛÔ˘ÏÊÔÌÂıÔÍ·˙fiÏË

1 Î·È ÎÏ·ÚÈıÚÔÌ˘Î›ÓË 1. ™˘Ó‰˘·˙fiÌÂÓ˜ ÚÔ-

ÎÏ‹ÛÂȘ (ÚÔη˝ÓË Î·È ÏȉÔη˝ÓË, ·ÌÔ͢ÎÈÏ›ÓË

Î·È ÙÚÈÌÂıÔÚ›ÌË, ÂÓÈÎÈÏ›ÓË Î·È ÎÏ·ÚÈıÚÔÌ˘-

ΛÓË ‹ ÂÚ˘ıÚÔÌ˘Î›ÓË) Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó ÛÂ

5 ·ÛıÂÓ›˜, Ì ‰‡Ô ·Ó·ÊÂÚfiÌÂÓ· Û˘ÌÙÒÌ·Ù·

Ô˘ ηıÈÛÙÔ‡Û·Ó ÙËÓ ˘Ô„›· ·ÏÏÂÚÁÈ΋˜ ·ÓÙ›-

‰Ú·Û˘ ÈÛ¯˘ÚfiÙÂÚË Î·È ÛÙÔ˘˜ ÔÔ›Ô˘˜, ÙÂÏÈο,

·Ó·Ù‡¯ıËΠ·ÓÙ›‰Ú·ÛË. ™Â ·˘Ù‹ ÙËÓ ÂÚ›-

ÙˆÛË, ÂÍÂÙ¿ÛÙËÎ·Ó ÙfiÛÔ ˆ˜ ÚÔ˜ ÙÔ ‡ÔÙÔ

Û··ÛÌ· fiÛÔ Î·È Û ¤Ó· ¿ÏÏÔ Ô˘ ı· ÌÔÚÔ‡-

Û ӷ ¯ÚËÛÈÌÔÔÈËı› ÂÓ·ÏÏ·ÎÙÈο.

OÈ ÚÔÎÏ‹ÛÂȘ ‹Ù·Ó ıÂÙÈΤ˜ Û 6 ·È‰È¿

(28%), Û 5 ÂÎ ÙˆÓ ÔÔ›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˘, ›ÙÂ

‹È·˜ (ÎÓȉˆÙÈÎfi ÂÍ¿ÓıËÌ·) ‹ ̤ÙÚÈ·˜ (·Ó·-

Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·). ¶¤ÓÙ ·fi Ù· ·È‰È¿

·˘Ù¿ ›¯·Ó ÈÛÙÔÚÈÎfi ·ÙÔ›·˜ Î·È ‹Ù·Ó fiÏ· ËÏÈ-

Λ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 10 ÂÙÒÓ. ™Â ¤Ó· ·fi

·˘Ù¿ Ú·ÁÌ·ÙÔÔÈ‹ıËΠ‰ÂÚÌ·ÙÈ΋ ‰ÔÎÈÌ·-

Û›· ÛÙË ÏÈÁÓÔη˝ÓË Î·È ÚÔη˝ÓË, ÏfiÁˆ ÈÛÙÔ-

ÚÈÎÔ‡ ·ÓÙ›‰Ú·Û˘ ÌÂÙ¿ ·fi ·ʋ Ì Á¿ÓÙÈ·

Ì›·˜ ¯Ú‹Û˘ (latex). ™Â ÙÚÂȘ ÂÚÈÙÒÛÂȘ, Ë

¤Ó‰ÂÈÍË Ù˘ ·Ú¯È΋˜ ¯ÚËÛÈÌÔÔ›ËÛ˘ ÙÔ˘ ÂÓÔ-

¯ÔÔÈÔ‡ÌÂÓÔ˘ Ê·ÚÌ¿ÎÔ˘ ‹Ù·Ó Ë ·ÓÙÈÌÂÙÒÈ-

ÛË Ïԛ̈͢ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ‹ ÂÈÌÔÏ˘-

Ṳ̂ÓÔ˘ ÂÎ˙¤Ì·ÙÔ˜, ÂÓÒ Û ̛· ÂÚ›ÙˆÛË

‹Ù·Ó Ë ·ÓÙÈÌÂÙÒÈÛË 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‹ıË-

Î·Ó ÛÙÂÚÔÂȉ‹ ηٿ ÙË ‰ÔÎÈÌ·Û›· Î·È Û ¤Ó·Ó

¶·È‰È·ÙÚÈ΋ 2005;68:337-340

¶›Ó·Î·˜ 1. ™˘¯ÓfiÙÂÚ· ·Ó·ÊÂÚfiÌÂÓ· Û˘ÌÙÒÌ·Ù· Û ʷÚ-̷΢ÙÈΤ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ

∂Í¿ÓıËÌ·, Ô›‰ËÌ· ÚÔÛÒÔ˘ 1∂Í¿ÓıËÌ· 1∂Í¿ÓıËÌ·, ¤ÌÂÙÔ˜ 7∫Ó›‰ˆÛË, Ô˘ÚÙÈοÚÈ·, ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· 1∫·ı˘ÛÙÂÚË̤ÓË ·ÓÙ›‰Ú·ÛË 1∫Ó›‰ˆÛË, Ô›‰ËÌ· ÚÔÛÒÔ˘ 5∫Ó›‰ˆÛË 4

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·338

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339º·Ú̷΢ÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ Û ·È‰È¿

·‰ÚÂÓ·Ï›ÓË ÁÈ· ¯Ú‹ÛË ÂÎÙfi˜ ÓÔÛÔÎÔÌ›Ԣ ÛÂÂÚ›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‹ ·Ï-ÏÂÚÁ›· ÛÙËÓ ÂÓÈÎÈÏ›ÓË ÌÂÙ¿ ·fi ¤Ó·ÚÍË ÚÔ-Ê˘Ï·ÎÙÈ΋˜ ‰fiÛ˘ Ô˘ Ï¿Ì‚·Ó ÏfiÁˆ ·ÓÔÛÔ-ηٷÛÙÔÏ‹˜.

™ÙÔ follow-up Ô˘ ¤ÁÈÓ Ì ÙËÏÂʈÓÈÎ‹Û˘Ó¤ÓÙ¢ÍË ÙˆÓ ÁÔÓ¤ˆÓ, ·ÓÙ·ÔÎÚ›ıËΠÙÔ73%. ¶¤ÓÙ ·È‰È¿ (22%) ¯ÚÂÈ¿ÛÙËÎ·Ó ·ÓÙÈ-‚ÈÔÙÈο ÌÂÙ¿ ÙËÓ ÚfiÎÏËÛË Î·È Î·Ó¤Ó· ‰ÂÓÂÌÊ¿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 ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙÔ ›‰ÈÔ Ê¿ÚÌ·ÎÔ,Â¿Ó ·Ô‰Â›¯ÙËΠ“·ıÒÔ”, ‹ ÙÔ ·ÛʷϤ˜ ˘Ô-ηٿÛÙ·Ùfi ÙÔ˘ ÛÙËÓ ·ÓÙ›ıÂÙË ÂÚ›ÙˆÛË. ™ÂÙ¤ÛÛÂÚȘ ÂÚÈÙÒÛÂȘ, ˆÛÙfiÛÔ, ·Ó Î·È Ë ‰ÔÎÈ-Ì·Û›· ‹Ù·Ó ·ÚÓËÙÈ΋, ÔÈ ÁÔÓ›˜ ·Ú·‰¤¯ÙËηÓfiÙÈ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ÌËÓ ¯ÚËÛÈÌÔÔÈÔ‡Ó ÙÔÊ¿ÚÌ·ÎÔ, ΢ڛˆ˜ ÏfiÁˆ ÚÔËÁÔ‡ÌÂÓ˘ ÙÚ·˘-Ì·ÙÈ΋˜ ÂÌÂÈÚ›·˜.

™˘˙‹ÙËÛË

∏ Ê·Ú̷΢ÙÈ΋ ·ÏÏÂÚÁ›· Â›Ó·È Ì›· ÛÔ‚·-Ú‹ Î·È ‰˘ÓËÙÈÎÒ˜ ·ÂÈÏËÙÈ΋ ÁÈ· ÙË ˙ˆ‹ η-Ù¿ÛÙ·ÛË Î·È ÁÈ’ ·˘Ùfi ÙÔÓ ÏfiÁÔ ¯Ú‹˙ÂÈ Û˘ÛÙË-Ì·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 ›-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 Ôχ۷ʋ Û˘ÌÙÒÌ·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·Ú-

Ì¿ÎˆÓ Ô˘ ˘ÔÙ›ıÂÙ·È fiÙÈ ÚÔοÏÂÛ·Ó “·Ï-

ÏÂÚÁÈ΋ ·ÓÙ›‰Ú·ÛË”, ¯ˆÚ›˜ ÚÔËÁÔ˘Ì¤Óˆ˜ Ó·

¤¯Ô˘Ó ·Ô‰Â›ÍÂÈ ÙËÓ ·Ú·¿Óˆ Û¯¤ÛË Î·È ¯ˆ-

Ú›˜ Ó· ·Ú·¤ÌÔ˘Ó ÙÔ˘˜ ÂӉȷÊÂÚfiÌÂÓÔ˘˜

ÁÈ· ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛË. ŒÙÛÈ, ·Ú·ÙËÚ›-

Ù·È Ì›· Ì›ˆÛË ÛÙÔÓ ·ÚÈıÌfi ÙˆÓ ‰È·ÁÓˆÛÙÈÎÒÓ

‰ÔÎÈÌ·ÛÈÒÓ Û ۯ¤ÛË Ì ÚÔËÁÔ‡ÌÂÓ· ¯ÚfiÓÈ·,

Ë ÔÔ›· Ê¿ÓËÎÂ Î·È ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. ∆Ô

·ÔÙ¤ÏÂÛÌ· Â›Ó·È ÔÏÏÔ› ¿ÓıÚˆÔÈ Ó· ÔÓÔÌ¿-

˙ÔÓÙ·È “·ÏÏÂÚÁÈÎÔ›” ÂÓÒ ‰ÂÓ Â›Ó·È, ÌÂ Û˘Ó¤-

ÂÈ· Ó· ÛÙÂÚÔ‡ÓÙ·È ÙˆÓ ıÂڷ¢ÙÈÎÒÓ È‰ÈÔÙ‹-

ÙˆÓ ÛËÌ·ÓÙÈÎÒÓ Û΢·ÛÌ¿ÙˆÓ (9). ∂ÈϤÔÓ,

Ë ¯Ú‹ÛË ·ÓÙÈ‚ÈÔÙÈÎÒÓ “‰Â‡ÙÂÚ˘ ÁÚ·ÌÌ‹˜” ÁÈ’

·˘Ùfi ÙÔÓ ÛÎÔfi ·˘Í¿ÓÂÈ Î·È ÙÔ ÔÏÔ¤Ó· ‰ÈÔ-

ÁÎÔ‡ÌÂÓÔ Úfi‚ÏËÌ· Ù˘ “·ÓÙÔ¯‹˜” (3). ∏ ÛË-

Ì·Û›· Ù˘ Ï‹ÚÔ˘˜ ‰ÈÂÚ‡ÓËÛ˘ ·˘ÙÒÓ ÙˆÓ

ÂÚÈÙÒÛÂˆÓ 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 ıÂڷ›·˜ Ì ·ÓÙÈ‚ÈÔÙÈο.

OÈ ‰ÔÎÈ̷ۛ˜ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ›ӷÈ

·ÛÊ·Ï›˜ fiÙ·Ó Ú·ÁÌ·ÙÔÔÈÔ‡ÓÙ·È Û ÂÍÂȉÈ-

ÎÂ˘Ì¤ÓÔ ÓÔÛÔÎÔÌÂÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ, ÂÊfiÛÔÓ

˘¿Ú¯ÂÈ Ë ÂÙÔÈÌfiÙËÙ· Ó· ·ÓÙÈÌÂÙˆÈÛÙÔ‡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 ÂÌÈÛÙÔÛ‡ÓË ÛÂ

·˘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 ‰˘ÛÈÛÙ›·˜, ÙÔ

ÔÔ›Ô - fiˆ˜ Ê¿ÓËΠ·fi ÙȘ ··ÓÙ‹ÛÂȘ ÙˆÓ

ÁÔÓ¤ˆÓ - ‰ÂÓ Â›¯Â Ó· οÓÂÈ Ì ÙËÓ ›‰È· ÙË ‰Ô-

ÎÈÌ·Û›·, ·ÏÏ¿ Ì ÙËÓ ÚÔÛˆÈ΋ ÙÔ˘˜ ÛÙ¿ÛË.

¢ÈÂÚ¢ÓÒÓÙ·˜ ÙË Û‡Á¯ÚÔÓË ‚È‚ÏÈÔÁÚ·Ê›·

Û¯ÂÙÈο Ì ÙȘ ˘¿Ú¯Ô˘Û˜ ‰È·ÁÓˆÛÙÈΤ˜ ‰Ô-

ÎÈ̷ۛ˜, 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 - ÛÂ

·˘Í·ÓfiÌÂÓ˜ ‰fiÛÂȘ. ∞˘Ù‹ Ë ‰ÔÎÈÌ·Û›· ¤¯ÂÈ

ηϋ ÂȉÈÎfiÙËÙ· Î·È Ì¤ÙÚÈ· ¢·ÈÛıËÛ›· (5),

Ô˘ ·˘Í¿ÓÂÙ·È Ì ÙË ¯Ú‹ÛË ÂÈϤÔÓ ·ÏÏÂÚ-

ÁÈÔÁfiÓˆÓ (9). °È· ÙȘ ÎÂÊ·ÏÔÛÔÚ›Ó˜ ¯ÚËÛÈ-

ÌÔÔÈÔ‡ÓÙ·È ·ÊÂÓfi˜ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÚÔ-

ÎÏ‹ÛÂȘ Û ·˘Í·ÓfiÌÂÓ˜ ‰fiÛÂȘ, ·ÊÂÙ¤ÚÔ˘

¶·È‰È·ÙÚÈ΋ 2005;68:337-340

∂ÓÔ¯ÔÔÈÔ‡ÌÂÓÔ Ê¿ÚÌ·ÎÔ

Erythromycin: 4

Septrin/Penicillin: 2

Ibuprufen: 1

Lidocaine: 1

Aspirin: 3

Clarithromycin: 1

PenicillinV: 4

Amoxicillin: 6

0

8

1 2 3 4 5 6 7

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

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·339

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340 A. ™ÔÊÈ·ÓÔ‡ - ∫·ÙÛÔ‡ÏË Î·È Û˘Ó.

‰ÂÚÌ·ÙÈΤ˜ ÚÔÎÏ‹ÛÂȘ (1,2). ª›· ÚfiÛÊ·ÙËÂÚÁ·Û›· (10) ·Ó·Ê¤ÚÂÈ fiÙÈ ÔÈ ‰ÂÚÌ·ÙÈΤ˜ ‰ÔÎÈ-̷ۛ˜ ÌÔÚ› Ó· Â›Ó·È ·ÚÓËÙÈΤ˜ Û ÂÚÈÙÒ-ÛÂȘ ·ÏÏÂÚÁ›·˜ Û ‚-Ï·ÎÙ·ÌÈο ·ÓÙÈ‚ÈÔÙÈοÏfiÁˆ ·ÓÙÔ¯‹˜. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, ÔÈ ·fi ÙÔ˘ÛÙfiÌ·ÙÔ˜ ÚÔÎÏ‹ÛÂȘ ıˆÚÔ‡ÓÙ·È ÂÚÈÛÛfiÙÂ-ÚÔ ·ÍÈfiÈÛÙ˜. ∏ ‰È¿ÁÓˆÛË Ù˘ ·ÏÏÂÚÁ›·˜ÛÙËÓ ·ÛÈÚ›ÓË Î·È Ù· ÌË ÛÙÂÚÔÂȉ‹ ·ÓÙÈÊÏÂÁ-ÌÔÓÒ‰Ë (ª™∞º) Á›ÓÂÙ·È Î˘Ú›ˆ˜ Ì ·fi ÙÔ˘ÛÙfiÌ·ÙÔ˜ ÚÔÎÏ‹ÛÂȘ, ÂÓÒ ·Â˘·ÈÛıËÙÔÔ›Ë-ÛË ÚÔÙ›ÓÂÙ·È Û ÂÚÈÙÒÛÂȘ ÛÔ‚·ÚÒÓ ·ÓÙÈ-‰Ú¿ÛˆÓ. OÈ ‰È·ÛÙ·˘ÚÔ‡ÌÂÓ˜ ·ÓÙȉڿÛÂȘÌÂٷ͇ ·ÛÈÚ›Ó˘ Î·È ª™∞º ‰ÂÓ ¤¯Ô˘Ó ÂÈ-‚‚·Èˆı›, ÁÈ’ ·˘Ùfi Ú¤ÂÈ Ó· ·ÓÙÈÌÂÙˆ›˙Ô-ÓÙ·È Ì ÂÈʇϷÍË (4). ∆¤ÏÔ˜, Ë ÚfiÎÏËÛË ÁÈ·ÙË ‰È¿ÁÓˆÛË ·ÏÏÂÚÁ›·˜ ÛÙ· ÙÔÈο ·Ó·ÈÛıËÙÈ-ο ‰ÂÓ ıˆÚÂ›Ù·È ··Ú·›ÙËÙË, ÂÎÙfi˜ Â¿Ó ÙÔÊ¿ÚÌ·ÎÔ ‰ÂÓ Â›Ó·È ÁÓˆÛÙfi (7). ™Â ·˘Ù‹ ÙËÓÂÚ›ÙˆÛË Ú·ÁÌ·ÙÔÔÈÂ›Ù·È ‰ÂÚÌ·ÙÈ΋ ‰Ô-ÎÈÌ·Û›· Û ·˘Í·ÓfiÌÂÓ˜ ‰fiÛÂȘ (8).

™Â ÂÚ¢ÓËÙÈÎfi Â›Â‰Ô ‚Ú›ÛÎÂÙ·È Ë Ì¤ÙÚË-ÛË ÂȤ‰ˆÓ Ï¢ÎÔÙÚÈÂÓÒÓ ÌÂÙ¿ ·fi ‰È¤ÁÂÚ-ÛË ÙˆÓ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ ·fi ·ÏÏÂÚÁÈÔÁfiÓÔ(Cellular antigen stimulation test - CAST) ÁÈ·ÙË ‰È¿ÁÓˆÛË ·ÏÏÂÚÁ›·˜ Û ª™∞º. ∆· RASTÎ·È UniCAP ·ÓȯÓÂ‡Ô˘Ó ·ÓÙÈÛÒÌ·Ù· Û ʿÚ-̷η. ∆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).

™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜“ÚÔÎÏ‹ÛÂȘ” ·ÔÙÂÏÔ‡Ó ·ÛÊ·Ï›˜ ‰ÔÎÈÌ·-ۛ˜, ÂÊfiÛÔÓ Ú·ÁÌ·ÙÔÔÈÔ‡ÓÙ·È Û ÂÍÂȉÈ-ÎÂ˘Ì¤ÓÔ ÓÔÛÔÎÔÌÂÈ·Îfi ÂÚÈ‚¿ÏÏÔÓ Î·È Ì ϋ-„Ë Î·Ù¿ÏÏËÏˆÓ ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ ÁÈ· ÙÔÂӉ¯fiÌÂÓÔ ·Ó·Ê˘Ï·ÎÙÈ΋˜ ·ÓÙ›‰Ú·Û˘. ∞fiÙ· ·ÔÙÂϤÛÌ·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ Ê¿-ÓËΠfiÙÈ Ë ‰È¿ÁÓˆÛË ·ÏÏÂÚÁ›·˜ ÛÂ Û˘ÁÎÂÎÚÈ-̤ӷ Û΢¿ÛÌ·Ù· Ì ·˘ÙfiÓ ÙÔÓ ÙÚfiÔ Â›Ó·È·ÍÈfiÈÛÙË. ¶Ú¤ÂÈ Ó· ÂÈÛËÌ·Óı›, ˆÛÙfiÛÔ,fiÙÈ Ë ÛÂÈÚ¿ ·ÛıÂÓÒÓ ÛÙËÓ ÔÔ›· ‚·Û›˙ÂÙ·È ËÛ˘ÁÎÂÎÚÈ̤ÓË ÌÂϤÙË Â›Ó·È ÌÈÎÚ‹. ∂Ô̤ӈ˜,ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÈÎ˘Úˆı› Ë ÂÁ΢ÚfiÙËÙ· ·˘-

ÙÒÓ ÙˆÓ ÚÔÎÏ‹ÛÂˆÓ Î·Ïfi ı· ‹Ù·Ó Ó· ˘¿Ú-

ÍÂÈ Ì›· ÌÂÁ·Ï‡ÙÂÚË ÌÂϤÙË Ô˘ ı· ÂÚÈÏ·Ì-

‚¿ÓÂÈ ÙË Û‡ÁÎÚÈÛ‹ ÙÔ˘˜ Ì ̛· ¿ÏÏË ‰ÔÎÈÌ·Û›·

Ô˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙË ‰È¿ÁÓˆÛË Ê·ÚÌ·-

΢ÙÈÎÒÓ ·ÏÏÂÚÁÈÒÓ. ™Â οı ÂÚ›ÙˆÛË È-

ı·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 ÛÂ

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 Aller-

gy 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 al-

lergic 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;11:405-

408.

9. Macy E, Burchette RJ. Oral antibiotic adverse reac-

tions after penicillin skin testing: multi-year follow-

up. Allergy 2002;57:1151-1158.

10. Aberer W, Bircher A, Romano A, Blanca M, Campi

P, Fernandez J et al. European Network for Drug Al-

lergy (ENDA). EAACI Interest Group on Drug

Hypersensitivity. Drug provocation testing in the di-

agnosis of drug hypersensitivity reactions: general

considerations. Allergy 2003;58:854-863.

¶·È‰È·ÙÚÈ΋ 2005;68:337-340

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·340

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341REVIEW ARTICLE

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 lifethreatening events that are often poorly diagnosed and managed. This is due to a lack of validatedand 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 thediagnosis of reported drug allergy. The testing was made at St. Mary’s Hospital, London. The testswere predominantly oral challenges with the exception of lidocaine/procaine, for which a skin test wasperformed. Most of the tests were done to test penicillin allergy (40%). Three quarters were singlecompared 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 childrenwho had a negative challenge felt that the outcome was reassuring. For those whose children had apositive outcome, it enhanced their confidence in the use of alternative drugs.

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

Key words

Drug challenges, drug allergy, adverse events.

1 Paediatric SpecialistRegistrar, St. Mary’sHospital, London, UK

2 Consultants in PaediatricAllergy, Department ofPaediatric Allergology andImmunology, St. Mary’sHospital, London, UK

Correspondence:

A. Sofianou-Katsoulis 42 Abbots Park, St. Albans Herts, UK AL1 1TWE-mail: [email protected]

Date of submission: 17-09-2004 Date of approval: 27-04-2005

Paediatriki 2005;68:341

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·341

Page 28: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

342 ∞¡∞™∫O¶∏™∏

1 °’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¶·ÓÂÈÛÙËÌÈ·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΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô÷˚‰·Ú›Ô˘ “∞ÙÙÈÎfiÓ”ƒ›ÌÈÓÈ 1, ÷˚‰¿ÚÈ, ∞ı‹Ó· E-mail: [email protected]

∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 22-10-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 13-04-2005

¶·È‰È·ÙÚÈ΋ 2005;68:342-350

∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ·

µ. ∆Û·ÁÚ‹˜1, ∞. ∫ÈÙÛÔÔ‡ÏÔ˘2, ∫. ª›¯·˜2, ¶. ¡ÈÎÔÏ·˝‰Ô˘ - ∫·Ú·ı›Ô˘1

¶ÂÚ›ÏË„Ë

∏ ÈÓÔÌ˘·ÏÁ›· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ·ÔÙÂÏ› Ì›· ·ÚÎÂÙ¿ Û˘Ó‹ıË, ÂÒ‰˘ÓË Ì˘ÔÛÎÂÏÂÙÈ΋ Û˘Ó‰ÚÔ-Ì‹, ¯·Ú·ÎÙËÚÈ˙fiÌÂÓË ·fi ›ÌÔÓÔ ‰È¿¯˘ÙÔ fiÓÔ, ·ÚÔ˘Û›· ÔÏÏ·ÏÒÓ ‰È·ÎÚÈÙÒÓ Â˘·›ÛıËÙˆÓÛËÌ›ˆÓ ÛÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË, ‰˘Ûη̄›·, ‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘ Î·È ÎfiˆÛË. ∏ ÔÚ›· Ù˘ ÓfiÛÔ˘ ›-Ó·È ¯ÚÔÓ›˙Ô˘Û· Î·È ÂËÚ¿˙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·ÁfiÓÙˆÓÌÔÚ› Ó· Û˘Ì‚¿ÏÏÂÈ ÛÙËÓ ÎÏÈÓÈ΋ ¤ÎÊÚ·ÛË Ù˘ ÓfiÛÔ˘. ∏ ıÂڷ¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Â›Ó·È ÌË ÂȉÈ-΋ Î·È ÔχÏ¢ÚË, ÂÚÈÏ·Ì‚¿ÓÔÓÙ·˜ Ê·ÚÌ·ÎÔÏÔÁÈο Î·È ÌË Ì¤ÙÚ·.

§¤ÍÂȘ ÎÏÂȉȿ

πÓÔÌ˘·ÏÁ›·, ·È‰È¿, fiÓÔ˜, ıÂڷ›·, Ì˘ÔÛÎÂÏÂÙÈÎfi˜.

∂ÈÛ·ÁˆÁ‹

∞ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ·ÈÙÈÔÏÔÁ›·, Ô fiÓÔ˜·ÔÙÂÏ› Ì›· Û˘¯Ó‹ ÂÌÂÈÚ›· ÛÙË ‰È¿ÚÎÂÈ· Ù˘·È‰È΋˜ ËÏÈΛ·˜. ∞Ó Î·È ÛÙ· Ôͤ· ÂÒ‰˘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›·ÂÌÂÈÚ›· Ì ‰È·ÛÙ¿ÛÂȘ Ú·ÁÌ·ÙÈΤ˜ ÁÈ· ·˘ÙfiÓÔ˘ ÙË ‚ÈÒÓÂÈ, ˘Ô‚·ıÌ›˙ÂÈ ÛËÌ·ÓÙÈο ÙËÓÔÈfiÙËÙ· ˙ˆ‹˜ ÙÔ˘ ¿Û¯ÔÓÙÔ˜ ·È‰ÈÔ‡ Î·È Ù˘ÔÈÎÔÁ¤ÓÂÈ·˜ Î·È Ô‰ËÁ› ÛÂ Û˘¯Ó¤˜ Î·È ÂӉ¯Ô-̤ӈ˜ ·ÙÂϤÛÊÔÚ˜ ÂÈÛΤ„ÂȘ Û ·ÁÁÂÏÌ·-ٛ˜ ˘Á›·˜ ‰È·ÊfiÚˆÓ ÂȉÈÎÔًوÓ.

∏ ÈÓÔÌ˘·ÏÁ›· ·ÔÙÂÏ› ‰È·Ù·Ú·¯‹ ÌË ÊÏÂÁ-ÌÔÓÒ‰Ô˘˜ ·ÈÙÈÔÏÔÁ›·˜, Ë ÔÔ›· ¯·Ú·ÎÙËÚ›˙ÂÙ·È·fi ÚÔÂÍ¿Ú¯Ô˘Û· Û˘Ìو̷ÙÔÏÔÁ›· ‰È¿¯˘-ÙÔ˘ fiÓÔ˘ ‹/Î·È ‰˘Ûη̄›·˜ Ì˘˚ÎÒÓ ÔÌ¿‰ˆÓ ‹Î·È ·ÚıÚÒÛÂˆÓ Î·È ·Ó‡ÚÂÛË Û˘ÁÎÂÎÚÈ̤ӈÓÛËÌ›ˆÓ ÂÓ Ùˆ ‚¿ıÂÈ Â˘·ÈÛıËÛ›·˜ ηٿ ÙËÓ ÎÏÈ-ÓÈ΋ ÂͤٷÛË. ∆·ÍÈÓÔÌÂ›Ù·È ÛÙË ‰È·ÁÓˆÛÙÈ΋ÔÌ¿‰· ÙÔ˘ ‰È¿¯˘ÙÔ˘ ȉÈÔ·ı‹ Ì˘ÔÛÎÂÏÂÙÈÎÔ‡fiÓÔ˘. ¶ÚfiÛÊ·Ù·, ÂÈÛ‹¯ıË Ô ÂÚÈÁÚ·ÊÈÎfiÙÂ-ÚÔ˜ fiÚÔ˜ “Ó·ÓÈÎfi ÚˆÙÔ·ı¤˜ Û‡Ó‰ÚÔÌÔ ÈÓÔ-Ì˘·ÏÁ›·˜”, ÛÙËÓ ÚÔÛ¿ıÂÈ· ·ÓÙȉȷÛÙÔÏ‹˜ÙˆÓ ‰È·ÊÔÚÒÓ Ù˘ ÎÏÈÓÈ΋˜ ¤ÎÊÚ·Û˘ Û ·È-‰È¿ Î·È ÂÓ‹ÏÈΘ, ·ÏÏ¿ Î·È ÙˆÓ ‰È·ÁÓˆÛÙÈÎÒÓȉȷÈÙÂÚÔًوÓ. OÈ ·Ï·ÈfiÙÂÚÔÈ fiÚÔÈ “Ì˘·ÏÁ›·˘fi Ù¿ÛË”, “ÁÂÓÈÎÂ˘Ì¤ÓË ÙÂÓÔÓÙÔÌ˘Ô¿ıÂÈ·”,“ÈÓÔÌ˘ÔÛ›Ùȉ·” ‹ “ÈÓÔÛ›Ùȉ·”, ‡ÛÙÂÚ· ·fi ÂÈ-Û‹ÁËÛË ÙÔ˘ ∞ÌÂÚÈηÓÈÎÔ‡ ∫ÔÏÂÁ›Ô˘ ƒÂ˘Ì·ÙÔ-ÏÔÁ›·˜ (∞∫ƒ), ‰ÂÓ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Ï¤ÔÓ(1). °È· ÏfiÁÔ˘˜ ·ÏfiÙËÙ·˜ Î·È ·ÔÊ˘Á‹˜ Û‡Á-

¯˘Û˘, ÔÈ Û˘ÁÁÚ·Ê›˜ ÙÔ˘ ·ÚfiÓÙÔ˜ ¿ÚıÚԢ¤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˘) ηÈÙÔÓ “‚Ú·‰‡” fiÓÔ (η˘ÛÙÈÎfi˜, ‚‡ıÈÔ˜, ¯ÚfiÓÈÔ˜‹ Ûʇ˙ˆÓ), Ô ÔÔ›Ô˜ Á›ÓÂÙ·È ·ÈÛıËÙfi˜ ÌÂÙ¿·fi 1 sec ‹ ÂÚÈÛÛfiÙÂÚÔ, Ì ‚·ıÌÈ·›· ·‡ÍËÛËÙ˘ ¤ÓÙ·Û‹˜ ÙÔ˘. O Ù·¯‡˜ fiÓÔ˜ ·ÊÔÚ¿ ΢-Ú›ˆ˜ ÛÙÔ ‰¤ÚÌ· Î·È ‰ÂÓ Á›ÓÂÙ·È ·ÈÛıËÙfi˜ ·fiÙÔ˘˜ ÂÓ Ùˆ ‚¿ıÂÈ ÈÛÙÔ‡˜ Î·È Ô ‚Ú·‰‡˜ fiÓÔ˜, ÔÔÔ›Ô˜ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙËÓ ÈÓÔÌ˘·ÏÁ›·, ÌÔÚ›ӷ ÚÔ¤Ú¯ÂÙ·È ÙfiÛÔ ·fi ÙÔ ‰¤ÚÌ· fiÛÔ Î·È ·fiÔÔÈ·‰‹ÔÙ ÂÓ Ùˆ ‚¿ıÂÈ ‰ÔÌ‹. ¶·Ú¿ ÙÔ ÁÂÁÔ-Ófi˜ fiÙÈ fiÏÔÈ ÔÈ ˘Ô‰Ô¯Â›˜ fiÓÔ˘ Â›Ó·È ÂχıÂ-Ú˜ Ó¢ÚÈΤ˜ ·ÔÏ‹ÍÂȘ, ÁÈ· ÙËÓ ·ÁˆÁ‹ ÙˆÓÓ¢ÚÈÎÒÓ ÒÛÂˆÓ ÚÔ˜ ÙÔ ∫¡™ ¯ÚËÛÈÌÔÔÈÔ‡-ÓÙ·È ‰‡Ô ͯˆÚÈÛÙ¤˜ Ó¢ÚÈΤ˜ Ô‰Ô›, Ì›· ÁÈ· ÙÔÙ·¯‡, Ô͇ ¿ÏÁÔ˜ Î·È Ì›· ÁÈ· ÙÔ ‚Ú·‰‡, ¯ÚfiÓÈÔ¿ÏÁÔ˜. ∆· Û‹Ì·Ù· ÁÈ· ÙÔ ‚Ú·‰‡ ¿ÏÁÔ˜ ÂÎχÔ-ÓÙ·È ·fi ¯ËÌÈÎÔ‡ Ù‡Ô˘ ÂÚÂı›ÛÌ·Ù· (‹ ηٿÂÚ›ÙˆÛË ·fi ›ÌÔÓ· Ì˯·ÓÈο ‹ ıÂÚÌÈο)Î·È ¿ÁÔÓÙ·È ÚÔ˜ ÙÔÓ ÓˆÙÈ·›Ô Ì˘ÂÏfi Ì ÂÚÈ-ÊÂÚÈο 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 ÂÙ›‰È·Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔ ÁÔÓ›‰ÈÔ Ù˘ ηÏÛÈÙÔÓ›-Ó˘. ªÂÙ¿ ÙËÓ Â›ÛÔ‰fi ÙÔ˘˜ ÛÙÔÓ ÓˆÙÈ·›Ô Ì˘Â-Ïfi, 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›‰·342

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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 ÂÁÎÂ-

Ê·ÏÈÎfi Û٤ϯԘ (2).

O ‚·ıÌfi˜ ·ÓÙ›‰Ú·Û˘ ÛÙÔÓ fiÓÔ ÔÈΛÏÏÂÈ

ÛËÌ·ÓÙÈο ·fi ¿ÙÔÌÔ Û ¿ÙÔÌÔ, ÁÂÁÔÓfi˜ ÙÔ

ÔÔ›Ô ÂӉ¯Ô̤ӈ˜ ÔÊ›ÏÂÙ·È ÛÙËÓ ÈηÓfiÙËÙ·

ÙÔ˘ ›‰ÈÔ˘ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Ó· ÂϤÁ¯ÂÈ ÙËÓ Â›ÛÔ‰Ô

ÂÒ‰˘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 ÏÂÈ-

ÙÔ˘ÚÁ›· ·˘ÙÔ‡ ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ η٤¯Ô˘Ó Ù·

ÂÓ‰ÔÁÂÓ‹ ÔÈÔÂȉ‹ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘. ∆· ÚÒÙ·

ÔÈÔÂȉ‹, ÔÈ ÂÁÎÂʷϛӘ, ÂÚÈÁÚ¿ÊËÎ·Ó ÙÔ

1975, Ì ·ÚÈÔ ÂÎÚfiÛˆÔ ÙË ‚-ÂÓ‰ÔÚÊ›ÓË.

¶ÂÚÈÛÛfiÙÂÚ· ·fi 20 ÂÓ‰ÔÁÂÓ‹ ÔÈÔÂȉ‹ ÂÙ›-

‰È· ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ̤¯ÚÈ Û‹ÌÂÚ·, Ì ٷ ÓÂfi-

ÙÂÚ· - ÂÓ‰ÔÌÔÚʛӘ Î·È ·ÏÁÔÛÙ·Ù›ÓË - Ó· ‚Ú›-

ÛÎÔÓÙ·È ÛÙÔ Â›ÎÂÓÙÚÔ ÙÔ˘ ÂӉȷʤÚÔÓÙÔ˜. ™Â

ÔÏÏ¿ ÛËÌ›· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ÂÓÙÔ›ÛÙËΠË

·ÚÔ˘Û›· ˘Ô‰Ô¯¤ˆÓ ÁÈ· ÔÈÔÂȉ‹, ȉȷ›ÙÂÚ·

ÛÙȘ ÂÚÈÔ¯¤˜ Ô˘ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙÔ Û‡-

ÛÙËÌ· ·Ó·ÏÁËÛ›·˜. OÈ ˘Ô‰Ô¯Â›˜ ·˘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˜ ηٷÓÔË-

ı›, Ë ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ·Ó·ÏÁË-

Û›·˜ Â›Ó·È ‰˘Ó·Ùfi Ó· ηٷÛÙ›ÏÂÈ Ï‹Úˆ˜ ·ÚÎÂ-

Ù¿ ÂÒ‰˘Ó· ÂÚÂı›ÛÌ·Ù· Ô˘ ÂÈÛ¤Ú¯ÔÓÙ·È ÛÙÔ

∫¡™ ·fi ÙËÓ ÂÚÈʤÚÂÈ· (3).

∂ȉËÌÈÔÏÔÁ›·

∏ ÈÓÔÌ˘·ÏÁ›· ·ÔÙÂÏ› Ì›· ·fi ÙȘ Û˘¯Ófi-

ÙÂÚ˜ ÚÂ˘Ì·ÙÈΤ˜ ·ı‹ÛÂȘ Û ÂÓ‹ÏÈΘ, Ì Â-

Ú›Ô˘ 5 ÂηÙÔÌ̇ÚÈ· ¿ÙÔÌ· Ó· ÓÔÛÔ‡Ó ÌfiÓÔ

ÛÙȘ ∏¶∞. ™Â ÏËı˘ÛÌȷ΋ ÌÂϤÙË 900 ÂÓËÏ›-

ΈÓ, Ù˘¯·ÈÔÔÈË̤ÓÔ˘ ‰Â›ÁÌ·ÙÔ˜ ·fi ÙË ™Ô˘-

ˉ›·, Ë Â›ÙˆÛË Ù˘ ÓfiÛÔ˘ ÚÔÛ‰ÈÔÚ›ÛÙËÎÂ

ÛÙÔ 1% (4). ™Â ·ÓÙ›ÛÙÔȯ˜ ÌÂϤÙ˜ ·fi ÙÔ

πÛÚ·‹Ï, ÙÔ ªÂÍÈÎfi Î·È ÙË ºÈÓÏ·Ó‰›·, ‰È·È-

ÛÙÒıËΠfiÙÈ 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˘ ÌÂÙ·-

͇ ÙˆÓ Ó¤ˆÓ ‰È·ÁÓÒÛÂˆÓ ·˘Í¿ÓÂÙ·È ÚÔԉ¢-

ÙÈο, ÂÎÚÔÛˆÒÓÙ·˜ ÔÛÔÛÙfi 7% › ÙÔ˘ Û˘-

ÓfiÏÔ˘ (8). OÈ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ÙˆÓ Ê‡ÏˆÓ Â›-

Ó·È ÛËÌ·ÓÙÈΤ˜, Ì ٷ ÎÔÚ›ÙÛÈ· Ó· ÚÔÛ‚¿ÏÏÔ-

ÓÙ·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˘Ìو̿وÓ, ÂÓÒ Ë Ï¤ÔÓ ÚÒ-

ÈÌË ‰È¿ÁÓˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È ÛÙË ‚È‚ÏÈÔ-

ÁÚ·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·fiÎÚÈ-

ÛË ÛÙË ıÂڷ›· ÛÙ· ·È‰È¿ ·’ fi,ÙÈ ÛÙÔ˘˜ ÂÓ‹-

ÏÈΘ, ·ÏÏ¿ Î·È Ë ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ÚÔ-

Û‚ÔÏ‹˜ ÙˆÓ ÎÔÚÈÙÛÈÒÓ ¤Ó·ÓÙÈ ÙˆÓ ·ÁÔÚÈÒÓ,

ÂÈÙÚ¤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 ÂȉÈfiÚ-

ıˆÛ˘ ÙÔ˘ Ì˘˚ÎÔ‡ ÈÛÙÔ‡ Â›Ó·È Èı·Ófi Ó· Û˘ÓÂÈ-

ÛʤÚÔ˘Ó ÛËÌ·ÓÙÈο ÛÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ· ÙÔ˘

Û˘Ó‰ÚfiÌÔ˘. ªÂϤÙ˜ Ì ̷ÁÓËÙÈ΋ Ê·ÛÌ·ÙÔ-

ÛÎÔ›· Û˘ÓËÁÔÚÔ‡Ó ˘¤Ú ‰È·Ù·Ú·¯ÒÓ ÛÙÔ Û·Ú-

ÎfiÏ·ÛÌ· ÙˆÓ Ì˘˚ÎÒÓ Î˘ÙÙ¿ÚˆÓ, Ô˘ ÚÔη-

ÏÔ‡Ó ÌÂȈ̤ÓË ¯ÚËÛÈÌÔÔ›ËÛË ÙˆÓ ÂÓÂÚÁÂÈ·-

ÎÒÓ ·ÔıÂÌ¿ÙˆÓ Î·È, ÙÂÏÈο, ΢ÙÙ·ÚÈ΋ ˘Ô-

Í›· (10). ∏ ·ÒÏÂÈ· ÙÔ˘ ··Ú·›ÙËÙÔ˘ ÁÈ· ÙË

‰È·Ù‹ÚËÛË Ù˘ ÛÙ·ıÂÚfiÙËÙ·˜ ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ

ÌÂÌ‚Ú·ÓÒÓ ∞∆ƒ, Ë ÔÔ›· ÚÔηÏÂ›Ù·È ÛÙÔ

Ï·›ÛÈÔ Ù˘ ˘ÔÍ›·˜, Ô‰ËÁ› ÛÂ Û˘Ó·ÎfiÏÔ˘ıË

ηٷÛÙÚÔÊ‹ ÙˆÓ Û·ÚÎÔÌÂÚȉ›ˆÓ. ∞˘Ù‹, Ì ÙË

ÛÂÈÚ¿ Ù˘, ÚÔηÏ› ·ÒÏÂÈ· ÂÓ‰Ô΢ÙÙ¿ÚÈÔ˘

Î·Ï›Ô˘, ÙÔ ÔÔ›Ô ÂÓÂÚÁÔÔÈ› ÙÔ˘˜ ˘Ô‰Ô¯Â›˜

fiÓÔ˘, ÚÔηÏÒÓÙ·˜ ÙÔÈΤ˜ Û˘Û¿ÛÂȘ Ì˘˚-

ÎÒÓ ÔÌ¿‰ˆÓ, ÔÈ Ôԛ˜ ÂÓÈÛ¯‡Ô˘Ó ÂÚ·ÈÙ¤Úˆ

ÙËÓ ·ÒÏÂÈ· Î·Ï›Ô˘ Î·È Ô‰ËÁÔ‡Ó ÙÂÏÈο ÛÂ

Ê·‡ÏÔ Î‡ÎÏÔ.

OÚÌÔÓÈΤ˜ ÌÂÙ·‚ÔϤ˜ Î·È ‰È·Ù·Ú·¯¤˜

‚ÈÔÁÂÓÒÓ ·ÌÈÓÒÓ

∏ ·Ó‡ÚÂÛË ¯·ÌËÏÒÓ ÂȤ‰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·ÌÂÓfiÌÂ-

ÓË ¤ÎÎÚÈÛË ÎÔÚÙÈ˙fiÏ˘ ·fi Ù· ÂÈÓÂÊÚ›‰È·, Û˘-

ÓËÁÔÚÔ‡Ó ˘¤Ú Èı·Ó‹˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘

˘Ôı·Ï·ÌÈÎÔ‡-˘ÔÊ˘ÛÈ·ÎÔ‡-ÂÈÓÂÊÚȉÈÎÔ‡

¿ÍÔÓ·. ªÂȈ̤ÓË ‚Ú¤ıËÎÂ, ›Û˘, Ë ¤ÎÎÚÈÛË

ÎÔÚÙÈ˙fiÏ˘ ÌÂÙ¿ ·fi ÚfiÎÏËÛË Ì ¯ÔÚ‹ÁËÛË

CRH, ‰È·›ÛÙˆÛË Ô˘ ηıÈÛÙ¿ Èı·Ófi ÙÔ Úˆ-

ÙÔ·ı¤˜ ·›ÙÈÔ Û ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›· Ó· ›-

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

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·343

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344 µ. ∆Û·ÁÚ‹˜ Î·È Û˘Ó.

ÔÔ›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·›ÛÙˆ-

Û·Ó ÂÏ·Ùو̤ӷ ›‰· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘

(GH) Û ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›· (14,15). ¶Úfi-

ÎÂÈÙ·È ÁÈ· ÔÚÌfiÓË Ô˘ ÂÎÎÚ›ÓÂÙ·È Û ·˘ÍË̤ӷ

›‰· 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˘ ÛÂ

·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›·, Â›Ó·È ‰˘Ó·Ùfi Ó· ÂË-

Ú¿ÛÂÈ ÙËÓ ¤ÎÎÚÈÛË Ù˘ GH Î·È ·˘Ù‹, Ì ÙË ÛÂÈ-

Ú¿ 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˜ ·ÔÙÂ-

Ï› ·ÓÙÈΛÌÂÓÔ ‰È¯ÔÁӈ̛·˜ ÙÔ ·Ó Ë ‰È·Ù·Ú·¯‹

Ù˘ Ê˘ÛÈÔÏÔÁ›·˜ ÙÔ˘ ‡ÓÔ˘ ·ÔÙÂÏ› Ì›· Úˆ-

ÙÔ·ı‹ ‰È·Ù·Ú·¯‹ ‹ ·Ï¿ ÂÈÛ˘Ì‚·›ÓÂÈ ˆ˜

·ÓÙ›‰Ú·ÛË ÛÙÔÓ fiÓÔ Ô˘ ˘ÔÛ˘Ó›‰ËÙ· ‚ÈÒ-

ÓÂÈ Ô ·ÛıÂÓ‹˜ ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘. ∏ Úfi-

ÎÏËÛË ˘ÔÁÏ˘Î·ÈÌ›·˜ ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ÈÓ-

ÛÔ˘Ï›Ó˘ Û ¿Û¯ÔÓÙ˜, Ô‰‹ÁËÛ Û ÛËÌ·ÓÙÈ΋

·‡ÍËÛË Ù˘ ¤ÎÎÚÈÛ˘ GH, οÙÈ ÙÔ ÔÔ›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·ÈÛÙÒ-

ıËÎ·Ó ÂÏ·Ùو̤ӷ ›‰· ‚ÈÔÁÂÓÒÓ ·ÌÈÓÒÓ

(5∏π∞∞, ÔÌÔ‚·Ó˘ÏÏÈÎfi Ô͇, 3-ÌÂı˘Ï-4-˘‰ÚÔ-

͢˚Ó‰ÔÏÈÎfi Ô͇) Û ۯ¤ÛË Ì ˘ÁÈ›˜ Ì¿ÚÙ˘Ú˜

(19,20). ™Â ¿ÏϘ ÌÂϤÙ˜ ‰È·ÈÛÙÒıËηÓ

ÂÏ·Ùو̤ӷ ›‰· ÛÂÚÔÙÔÓ›Ó˘ Î·È ·˘ÍË̤-

ÓÔ˜ ·ÚÈıÌfi˜ ˘Ô‰Ô¯¤ˆÓ ·ӷÚfiÛÏ˄˘ ÛÂ-

ÚÔÙÔÓ›Ó˘ ÛÙ· ·ÈÌÔÂÙ¿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˘ ÔÔ›·˜ ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ ÛÙÔ ∂¡À ·-

Û¯fiÓÙˆÓ ¤¯Ô˘Ó ‚ÚÂı› ·˘ÍË̤Ó˜ (21). ∞fi ·Ú-

ÎÂÙÔ‡˜ Û˘ÁÁÚ·Ê›˜, Ù· ·Ú·¿Óˆ Â˘Ú‹Ì·Ù·

ÂÓÔÔÈÔ‡ÓÙ·È ÛÙÔ Ï·›ÛÈÔ Ì›·˜ ¢ڇÙÂÚ˘ ıˆ-

Ú›·˜ ÌÂȈ̤Ó˘ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ·˜ ÙÔ˘ ·˘ÙfiÓÔ-

ÌÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (22).

∆Ô ·ÓÔÛÔÏÔÁÈÎfi ˘fi‚·ıÚÔ

∂Í·ÈÚÂÙÈÎfi ÂӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÂϤ-

Ù˜ Ô˘ ÂÛÙÈ¿˙ÔÓÙ·È ÛÙËÓ Î·Ù·ÛÙÔÏ‹ Ù˘ ‰Ú¿-

Û˘ ÙˆÓ ÂÓ‰ÔÁÂÓÒÓ ÔÈÔÂȉÒÓ, ‹ Ú˘ıÌÈÛÙÒÓ

Ù˘ ‰Ú¿Û˘ ÙÔ˘˜, ·fi ·˘ÙÔ¿ÓÔÛÔ˘˜ Ì˯·ÓÈ-

ÛÌÔ‡˜. Œ¯ÂÈ ‰È·Ù˘ˆı› Ë ¿Ô„Ë fiÙÈ ·˘ÙÔ·ÓÙÈ-

ÛÒÌ·Ù· Â›Ó·È ‰˘Ó·Ùfi Ó· ÛÙÚ¤ÊÔÓÙ·È Î·Ù¿ ÙˆÓ

ÂÙȉ›ˆÓ ‹ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ ÙÔ˘˜ Î·È Ó· Ù· η-

Ù·ÛÙÚ¤ÊÔ˘Ó ‹ Ó· ‰È·Ù·Ú¿ÛÛÔ˘Ó ÙË ÏÂÈÙÔ˘ÚÁ›·

ÙÔ˘˜. ™ÙË ıˆڛ· fiÙÈ ÚˆÙ·Ú¯ÈÎfi ÚfiÏÔ ÛÙËÓ ·È-

ÙÈÔ·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 ÔÔ›ÔÈ ÛÙÚ¤ÊÔÓÙ·È

ηٿ Ó¢ÚÔÂÙȉ›ˆÓ (fiˆ˜ 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 ÈÓÔ-

Ì˘·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 Ô-

ÛÔÛÙfi 28-52%, ÂÓÒ Û ÌËÙ¤Ú˜ ÓÔÛÔ‡ÓÙˆÓ ·È-

‰ÈÒÓ Û ÔÛÔÛÙfi 71% (26). ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi

ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó¿Ù˘ÍË ıˆÚÈÒÓ Û¯ÂÙÈο ÌÂ

ÙËÓ ‡·ÚÍË ÁÂÓÂÙÈÎÔ‡ ˘ÔÛÙÚÒÌ·ÙÔ˜ Ù˘ Ófi-

ÛÔ˘. ∆Ô ÂÚ¢ÓËÙÈÎfi ÂӉȷʤÚÔÓ, Û‹ÌÂÚ·,

ÂÛÙÈ¿˙ÂÙ·È ÛÙË Û˘Û¯¤ÙÈÛË Ù˘ ÓfiÛÔ˘ ÌÂ Û˘ÁÎÂ-

ÎÚÈ̤ӷ ·ÓÙÈÁfiÓ· ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ HLA, ηıÒ˜

Î·È ÛÙË ‰ÈÂÚ‡ÓËÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ ÔÏ˘ÌÔÚ-

ÊÈÛÌÔ‡ (Ù˘ ·ÚÔ˘Û›·˜, ‰ËÏ·‰‹, ÂÚÈÛÛfiÙÂ-

ÚˆÓ Ù˘ Ì›·˜ ‰ÔÌÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ) ÙÔ˘ ÁÔÓȉ›-

Ô˘ Ù˘ 5-∏∆∆, ˘Â‡ı˘ÓÔ˘ ÁÈ· ÙË ÌÂÙ·ÊÔÚ¿ Ù˘

ÛÂÚÔÙÔÓ›Ó˘ (27).

¢È·Ù·Ú·¯¤˜ ÙÔ˘ ‡ÓÔ˘

OÈ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ‡ÓÔ˘ Û ·ÛıÂÓ›˜ ÌÂ

ÈÓÔÌ˘·ÏÁ›· ¤¯Ô˘Ó ·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˘ ÌÔÚ› 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 fiÔ˘ ÍÂÎÈÓÔ‡Ó Ó¢ÚÈΤ˜

›Ó˜, ÔÈ Ôԛ˜ ÂÎÙfi˜ ·fi ÙË ÏÂÈÙÔ˘ÚÁ›· Ù˘

·Ó·ÛÙÔÏ‹˜ ÙˆÓ ÂÚÂıÈÛÌ¿ÙˆÓ fiÓÔ˘ Ô˘ ÂÈÛ¤Ú-

¯ÔÓÙ·È ÛÙÔÓ ÓˆÙÈ·›Ô Ì˘ÂÏfi, ÂÈÙÂÏÔ‡Ó Î·È ·˘Ù‹

Ù˘ ¤ÎÎÚÈÛ˘ ÛÂÚÔÙÔÓ›Ó˘. ∏ ÛÂÚÔÙÔÓ›ÓË ·Ô-

ÙÂÏ› ÙËÓ Î˘ÚÈfiÙÂÚË ·fi ÙȘ Ó¢Úԉȷ‚È‚·ÛÙÈ-

Τ˜ Ô˘Û›Â˜ Ô˘ ¤¯Ô˘Ó Û¯¤ÛË Ì ÙËÓ ÚfiÎÏËÛË

ÙÔ˘ ‡ÓÔ˘ (¯ÔÚ‹ÁËÛË ·Ó·ÛÙÔϤˆÓ Ù˘ Û ÂÈ-

Ú·Ì·Ùfi˙ˆ· Û˘Óԉ‡ÂÙ·È ·fi ·¸Ó›· Ô˘ ‰È·Ú-

Λ Ë̤Ú˜) Î·È Ù· ›‰¿ Ù˘ ¤¯Ô˘Ó ‰È·È-

ÛÙˆı› ÂÏ·Ùو̤ӷ Û ·ÛıÂÓ›˜ Ì ÈÓÔÌ˘·ÏÁ›·.

¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô ·ÛıÂÓ‹˜ ‰ÂÓ ·Ó·Ê¤ÚÂÈ

¶·È‰È·ÙÚÈ΋ 2005;68:342-350

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·344

Page 31: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

345∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ·

‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘, ÚÔÛÂÎÙÈ΋ ÚÔÛ¤ÁÁÈÛË

·ÔηχÙÂÈ fiÙÈ Ô ‡ÓÔ˜ ‰ÂÓ ÙÔÓ ÍÂÎÔ˘Ú¿˙ÂÈ

(Ê·ÈÓfiÌÂÓÔ ÚˆÈÓ‹˜ ÎfiˆÛ˘), 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˘ÛÈÔ-

ÏÔÁÈο ·̷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 ÛÂ

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 ÓÔ-

ÛÔ‡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 ÔÔ›· Û˘-

¯Ó¿ ÓÔÛ›), ·ÔÎÙ¿ ÎÂÊ·Ï·ÈÒ‰Ë ÛËÌ·Û›·. ∏

Û˘ÌÂÚÈÊÔÚ¿ Ù˘ ‰˘¿‰·˜ ÌËÙ¤Ú·˜-¿Û¯Ô˘Û·˜

ÎfiÚ˘ ÂÚÈÁÚ¿ÊÂÙ·È ˆ˜ ¯·Ú·ÎÙËÚÈÛÙÈ΋: Ë

˘ÂÚÚÔÛٷ٢ÙÈ΋ ÌËÙ¤Ú· “ÂÚÈ‚¿ÏÏÂÈ” Û˘-

Ó·ÈÛıËÌ·ÙÈο ÙËÓ ¿Û¯Ô˘Û· ÎfiÚË, ··ÓÙÒ-

ÓÙ·˜ Û˘¯Ó¿ ·ÓÙ’ ·˘Ù‹˜ Û ÂÚˆÙ‹ÛÂȘ Ô˘ ·Â˘-

ı‡ÓÔÓÙ·È ÛÙËÓ ÙÂÏÂ˘Ù·›· Î·È ·ÊÔÚÔ‡Ó ÛÂ Û˘-

Ó·ÈÛı‹Ì·Ù· ‹ ‚Ȉ̷ÙÈΤ˜ ÂÌÂÈڛ˜. ∆· ¿-

Û¯ÔÓÙ· ·È‰È¿ ·ÔÙÂÏÔ‡Ó Û˘Ó‹ıˆ˜ ¿ÙÔÌ· ȉÈ-

·›ÙÂÚ· ·Ô‰ÔÙÈο, ÙfiÛÔ ÛÙÔ Û¯ÔÏÈÎfi ÂÚÈ‚¿Ï-

ÏÔÓ fiÛÔ Î·È Û Â͈ۯÔÏÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜,

Ù· ÔÔ›· ÂÈ˙ËÙÔ‡Ó ÌÂıÔ‰Èο - Î·È Û˘¯Ó¿ ÂÈ-

Ù˘Á¯¿ÓÔ˘Ó - ÙÔÓ ¤·ÈÓÔ ÙˆÓ ÁÔÓ¤ˆÓ (31). ∞Ô-

ÙÂÏ› ¢ڤˆ˜ ‰ڷȈ̤ÓË ÂÔ›ıËÛË fiÙÈ Ù·

„˘¯È·ÙÚÈο ÓÔÛ‹Ì·Ù· ÂȉÚÔ‡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‡˜ ·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¿ ÂÈÛ˘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˜ ÌÂ-

ϤÙ˜ 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˘ ·ÔÙÂ-

ÏÔ‡Ó ‰È·‰Èηۛ˜ ‰È·ÊÔÚÂÙÈΤ˜ Î·È ·ÓÂÍ¿ÚÙË-

Ù˜ (32). ŒÙÛÈ, ·ÛıÂÓ›˜ ¿Û¯ÔÓÙ˜ ·fi ¯ÚfiÓÈÔ

¿ÏÁÔ˜ Î·È Î·Ù¿ıÏÈ„Ë ‰ÂÓ ‰È·Ê¤ÚÔ˘Ó fiÛÔÓ ·ÊÔ-

Ú¿ ÛÙËÓ ÚfiÛÏË„Ë Î·È ÙËÓ ·ÓÙ›ÏË„Ë ÙÔ˘ fi-

ÓÔ˘ ·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˜ ÂÌÂÈڛ˜ fiÓÔ˘,

ÂÏÏÂÈÌÌ·ÙÈ΋ ÎÔÈÓˆÓÈ΋ ·ÏÏËÏ›‰Ú·ÛË, Ê˘-

ÛÈ΋ ‹ ÛÂÍÔ˘·ÏÈ΋ ηÎÔÔ›ËÛË, ÚfiÙ˘· ÁÔ-

ÓÈ΋˜ Û˘ÌÂÚÈÊÔÚ¿˜ ·¤Ó·ÓÙÈ ÛÙÔÓ fiÓÔ, η-

΋ Ê˘ÛÈ΋ ηٿÛÙ·ÛË), ıˆÚÂ›Ù·È Èı·Ó‹. ∫·-

ıÒ˜ ÔÈ ·Ú·¿Óˆ ·Ú¿ÁÔÓÙ˜ ÌÔÚ› Ó·

‰ÚÔ˘Ó ÌÂÌÔӈ̤ӷ ‹ Î·È ÛÂ Û˘Ó‰˘·ÛÌfi, ÙÔ

¿ÙÔÌÔ ÌÔÚ› Ó· “‚ÈÒÓÂÈ” ÙËÓ ÂÌÂÈÚ›· ÙÔ˘

fiÓÔ˘ ÌÂ ÌÔÓÔÛ‹Ì·ÓÙÔ ‹ ÌË ÙÚfiÔ. °È· ·Ú¿-

‰ÂÈÁÌ·, Ë ÂÌÊ¿ÓÈÛË fiÓÔ˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ

Û ‰È·Ù·Ú·¯‹ ÙÔ˘ ‡ÓÔ˘, Ë ÔÔ›· fi¯È ÌfiÓÔ

ÂȉÂÈÓÒÓÂÈ ÂÚ·ÈÙ¤Úˆ ÙÔÓ fiÓÔ, ·ÏÏ¿ ÂÈ-

ϤÔÓ ÚÔηÏ› ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘ ÔÈÎÔÁÂÓÂÈ-

·Î‹˜ ˙ˆ‹˜ (33,34).

∫ÏÈÓÈÎÔ› ¯·Ú·ÎÙ‹Ú˜ - ¢È¿ÁÓˆÛË

∆Ô ÁÂÁÔÓfi˜ fiÙÈ Ù· ·È‰È¿ Ì ÂÒ‰˘Ó· Ì˘Ô-

ÛÎÂÏÂÙÈο Û‡Ó‰ÚÔÌ·, ÛÙËÓ ÏÂÈÔÓfiÙËÙ¿ ÙÔ˘˜,

·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ¯·ÚÔ‡ÌÂÓ·, ‹ÚÂÌ·, ·ÎfiÌ· ηÈ

¯·ÌÔÁÂÏÒÓÙ·˜, ÂÓÒ Ù·˘Ùfi¯ÚÔÓ· ·Ó·Ê¤ÚÔ˘Ó ÛË-

Ì·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 ÔÔ›· ‰È·ÈÛÙÒ-

ÓÂÙ·È ÌÂÙ¿ ·fi ··Ïfi ¿ÁÁÈÁÌ· ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ ‹

‹È· ¤ÏÍË ‰ÂÚÌ·ÙÈ΋˜ Ù˘¯‹˜.

∏ ·‰˘Ó·Ì›· ÂÚÁ·ÛÙËÚȷ΋˜ ‹ ÈÛÙÔÏÔÁÈ΋˜

ÂȂ‚·›ˆÛ˘ Ù˘ ‰È¿ÁÓˆÛ˘ Ô‰‹ÁËÛ ÛÙË ı¤-

ÛÈÛË 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

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346 µ. ∆Û·ÁÚ‹˜ Î·È Û˘Ó.

ÎÚÈÙËÚ›ˆÓ: ·) ÙËÓ ‡·ÚÍË ‰È¿¯˘ÙÔ˘ fiÓÔ˘ ÁÈ·

¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 3 ÌËÓÒÓ, Ô

ÔÔ›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˘ ·ÍÈfiÈÛÙ· ·Ô-

ÙÂϤÛÌ·Ù· Ï·Ì‚¿ÓÔÓÙ·È Î·È Ì ÙË ¯Ú‹ÛË ÙÔ˘

·ÓÙ›¯ÂÈÚ· (Ú·ÎÙÈο ı· Ú¤ÂÈ Ó· ·ÛÚ›ÛÂÈ Ë

ÔÓ˘¯ÔÊfiÚÔ˜ Ê¿Ï·ÁÁ· ÙÔ˘ ·ÓÙ›¯ÂÈÚ· ÙÔ˘ ÂÍÂÙ·-

ÛÙ‹). °È· ÙËÓ ÂÍ¿ÏÂÈ„Ë ÙÔ˘ ÛÙÔÈ¯Â›Ô˘ Ù˘ ˘Ô-

ÎÂÈÌÂÓÈÎfiÙËÙ·˜, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ˆ˜ Ì¿ÚÙ˘-

Ú˜ 10 ÛËÌ›· (̤وÔ, ‰ÂÍÈfi Î·È ·ÚÈÛÙÂÚfi

Ï¿ÁÈÔ, Ú·¯È·›· ÂÈÊ¿ÓÂÈ· ÂÚÈÊÂÚÈÎÔ‡ ÙÌ‹Ì·-

ÙÔ˜ ·ÓÙÈ‚Ú·¯›Ô˘ ·ÌÊÔÙÂÚfiÏ¢ڷ, ÔÓ˘¯ÔÊfiÚÔ˜

Ê¿Ï·ÁÁ· ÙˆÓ ·ÓÙȯ›ڈÓ, Ú·¯È·›· ÂÈÊ¿ÓÂÈ·

ÙÔ˘ ÙÚ›ÙÔ˘ ÌÂٷٷÚÛ›Ô˘ ·ÌÊÔÙÂÚfiÏ¢ڷ, ÔÓ˘-

¯ÔÊfiÚÔ˜ Ê¿Ï·ÁÁ· 1Ô˘ ‰·ÎÙ‡ÏÔ˘ Ô‰ÈÔ‡ ·ÌÊÔ-

ÙÂÚfiÏ¢ڷ). ∏ ÙÂÎÌËÚ›ˆÛË Ù˘ ‰È¿ÁÓˆÛ˘ ıÂ-

ˆÚÂ›Ù·È ÂÈÛÊ·Ï‹˜ fiÙ·Ó ‰È·ÈÛÙÒÓÂÙ·È Â˘·È-

ÛıËÛ›· 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˘ ›Û˘ ·Ú·ÙËÚÔ‡ÓÙ·È

ÛÙËÓ ÈÓÔÌ˘·ÏÁ›· Î·È Ù· ÔÔ›· ·ÓÙÈÛÙÔÈ¯Ô‡Ó ÛÂ

¢·›ÛıËÙ˜ ÂÚÈÔ¯¤˜ Ì˘ÒÓ, Ë ‰È¤ÁÂÚÛË ÙˆÓ

ÔÔ›ˆÓ Ô‰ËÁ› Û ·ÓÙ·Ó¿ÎÏ·ÛË ÙÔ˘ fiÓÔ˘ ηÈ

ÛÙË ¯·Ú·ÎÙËÚÈÛÙÈ΋ Û‡Û·ÛË ÙÔ˘ Ì˘fi˜. ∆· ÛË-

Ì›· ·˘Ù¿, ˆÛÙfiÛÔ, ‰ÂÓ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙË

‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË, ηıÒ˜ ηٿ ÙËÓ ·ÍÈÔ-

ÏfiÁËÛ‹ ÙÔ˘˜ ˘ÂÈÛ¤Ú¯ÂÙ·È Û ÛËÌ·ÓÙÈÎfi ‚·ıÌfi

Ô ·Ú¿ÁÔÓÙ·˜ Ù˘ ˘ÔÎÂÈÌÂÓÈÎfiÙËÙ·˜. ∞ÚÎÂÙÔ›

¶·È‰È·ÙÚÈ΋ 2005;68:342-350

3 4

12

1817

11

9 10

1 26

875

15 16

1413

∂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‡ÌÂÓ˜ “∆ÚÂȘ ÿÚÈÙ˜” (ªÔ˘Û›ÔPrado, ª·‰Ú›ÙË. ÀÏÔÔ›ËÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ‚·ÛÈṲ̂ÓË Û ȉ¤· ‰ËÌÔÛÈ¢ı›۷ ÛÙÔ Arthritis and Rheumatism Journal,1990)

1,2. πÓȷ΋ ÂÚÈÔ¯‹ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙȘ ÚÔÛʇÛÂȘ ÙˆÓ ˘ÈÓÈ·ÎÒÓ Ì˘ÒÓ3,4. ∫·ÙÒÙÂÚÔ ÙÌ‹Ì· ·˘¯¤Ó· ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙËÓ ÂÚÈÔ¯‹ ÙˆÓ ÂÁοÚÛÈˆÓ ·ÔʇÛÂˆÓ ÛÙÔ ‰È¿ÛÙËÌ· ∞5-∞75,6. ∆Ú·Â˙ÔÂȉ‹˜ Ì˘˜ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙË ÌÂÛfiÙËÙ· ÙÔ˘ ·ÓÒÙÂÚÔ˘ ¯Â›ÏÔ˘˜ ÙÔ˘7,8. ÀÂڷοÓıÈÔ˜ Ì˘˜ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙËÓ ¤ÎÊ˘Û‹ ÙÔ˘, ÏËÛ›ÔÓ ÙÔ˘ ¤Ûˆ ¯Â›ÏÔ˘˜ Ù˘ ˆÌÔÏ·ÙÈ·›·˜

¿Î·Óı·˜9,10. 2Ë ÏÂ˘Ú¿ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙË ÛÙÂÚÓÔÏ¢ÚÈ΋ Û˘Á¯fiÓ‰ÚˆÛË Ù˘ 2˘ ÏÂ˘Ú¿˜11,12. ŒÍˆ ÂÈÎfiÓ‰˘ÏÔ˜ ∞ÌÊÔÙÂÚfiÏ¢ڷ, 2 cm ÂÚÈÊÂÚÈÎfiÙÂÚ· ÙˆÓ ¤Íˆ ÂÈÎÔÓ‰‡ÏˆÓ 13,14. °ÏÔ˘ÙÔ› ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙÔ ¿Óˆ-¤Íˆ ÙÂÙ·ÚÙËÌfiÚÈfi ÙÔ˘˜, ηٿ ÙÔ ÚfiÛıÈÔ ¯Â›ÏÔ˜ ÙÔ˘

Ì›˙ÔÓÔ˜ ÁÏÔ˘ÙÈ·›Ô˘ Ì˘fi˜ 15,16. ªÂ›˙ˆÓ ÙÚÔ¯·ÓÙ‹Ú·˜ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÂÏ¿¯ÈÛÙ· ÔÈÛı›ˆ˜ ÙÔ˘ Ì›˙ÔÓÔ˜ ÙÚÔ¯·ÓÙËÚÈÎÔ‡ ¿ÚÌ·ÙÔ˜ 17,18. °fiÓ·ÙÔ ∞ÌÊÔÙÂÚfiÏ¢ڷ, ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÏÈÒ‰Ô˘˜ ÛÒÌ·ÙÔ˜, ÏËÛ›ÔÓ Ù˘ ¤Ûˆ ·ÚıÚÈ΋˜

ÁÚ·ÌÌ‹˜

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·346

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347∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ·

Û˘ÁÁÚ·Ê›˜ Û˘ÓËÁÔÚÔ‡Ó ˘¤Ú Ù˘ ¿Ô„˘ fiÙÈ

ÛÙ· ·È‰È¿ Ë ‰È¿ÁÓˆÛË ÌÔÚ› Ó· ÙÂÎÌËÚȈı›

·ÎfiÌ· Î·È fiÙ·Ó ÙÔ ¿ıÚÔÈÛÌ· ÙˆÓ Â˘·›ÛıËÙˆÓ

ÛËÌ›ˆÓ ÛÙÔ Û‡ÓÔÏÔ ÙˆÓ ÂÈÛΤ„ÂˆÓ ÛÙÔÓ ·È-

‰›·ÙÚÔ Â›Ó·È ÌÈÎÚfiÙÂÚÔ ÙÔ˘ 11 (≥5 ηٿ ÙÔ˘˜

Yunus Î·È Masi), Û ·ÓÙȉȷÛÙÔÏ‹ Ì ÙÔ˘˜ ÂÓ‹-

ÏÈΘ, fiÔ˘ ··ÈÙÂ›Ù·È Î·Ù¿‰ÂÈÍË ÙÔ˘Ï¿¯ÈÛÙÔÓ

11 ÛËÌ›ˆÓ ηٿ ÙËÓ ÚÒÙË ÌfiÓÔ Â›Û΄Ë

(35). ∞˘Ùfi ÚÔÙ›ÓÂÙ·È ÏfiÁˆ Ù˘ ·ÙÂÏÔ‡˜ ¤Î-

ÊÚ·Û˘ Ù˘ ÓfiÛÔ˘ ÛÙ· ·È‰È¿, ηıÒ˜ Î·È Ù˘

ÈηÓÔÔÈËÙÈ΋˜ ·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˜

·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ÌÂıÔ‰ÔÏÔÁÈΤ˜ ·‰˘Ó·Ì›Â˜

ÙˆÓ ÌÂÏÂÙÒÓ.

∆· Û˘ÓÔ‰¿ Û˘ÌÙÒÌ·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˘ ÈÓÔ-

Ì˘·ÏÁ›·˜ Î·È ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ˘ÂÚÎÈÓËÙÈÎfiÙË-

Ù·˜, ηıÒ˜ Û ÛËÌ·ÓÙÈÎfi ·ÚÈıÌfi ·È‰ÈÒÓ ÌÂ

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 ·ÏÏËÏÔÂÈÎ¿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 ·ÏÏËÏÔÂÈÎ¿Ï˘„Ë ·˘-

Ù‹ ı· ÌÔÚÔ‡Û ӷ ·Ô‰Ôı› ÛÙËÓ ·Ó·Ú΋

·ÍÈÔÏfiÁËÛË ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ ·fi ·ÚÎÂÙÔ‡˜

Û˘ÁÁÚ·Ê›˜.

¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË

ƒÂ˘Ì·ÙÈÎfi ÓfiÛËÌ·, ȉȷ›ÙÂÚ· ÚÂ˘Ì·ÙÔÂÈ-

‰‹˜ ·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˘·Ï-

Á›· Î·È ÔÈ ·ÁÁÂÈ›Ùȉ˜ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó

Ì ÎÏÈÓÈ΋ ÂÈÎfiÓ· ·ÚfiÌÔÈ· Ì ÂΛÓË Ù˘ ÈÓÔ-

Ì˘·ÏÁ›·˜, ·ÏÏ¿ ÙÔ ¿ıÚÔÈÛÌ· ÙˆÓ Â˘·›ÛıËÙˆÓ

ÛËÌ›ˆÓ Î·È ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ ÔÙ¤ ‰ÂÓ

ÚÔÛÂÁÁ›˙ÂÈ ·˘Ùfi Ù˘ ÈÓÔÌ˘·ÏÁ›·˜, ÂÎÙfi˜ ¿Ó

Ë ÙÂÏÂ˘Ù·›· Û˘Ó˘¿Ú¯ÂÈ ˆ˜ ͯˆÚÈÛÙ‹ ÔÓÙfi-

ÙËÙ·. ∂ÈϤÔÓ, Ë ÚÂ˘Ì·ÙÈ΋ ÔÏ˘Ì˘·ÏÁ›· ›-

Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈ· ÛÙ· ·È‰È¿. ™ÙË ‰È·ÊÔ-

ÚÈ΋ ‰È¿ÁÓˆÛË Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ë

Û·ÚÎÔ›‰ˆÛË, ÔÈ Û˘ÛÙËÌ·ÙÈΤ˜ ÏÔÈÌÒÍÂȘ, Ù·

·Ú·ÓÂÔÏ·ÛÌ·ÙÈο Û‡Ó‰ÚÔÌ·, ÙÔ Û‡Ó‰ÚÔÌÔ

˘ÂÚÎÈÓËÙÈÎfiÙËÙ·˜, Ë ÚÒÈÌË ÛÔÓ‰˘ÏÔ·Ú-

ıÚÔ¿ıÂÈ·, ÙÔ Û‡Ó‰ÚÔÌÔ ÙÔ˘ ÚfiÛıÈÔ˘ ıˆÚ·-

ÎÈÎÔ‡ ÙÔȯÒÌ·ÙÔ˜, Ë ·ÓÙ·Ó·ÎÏ·ÛÙÈ΋ Û˘Ì·-

ıËÙÈ΋ ‰˘ÛÙÚÔÊ›· (‹ ÂÓÙÔÈṲ̂ÓÔ ÂÚÈÔ¯ÈÎfi

Û‡Ó‰ÚÔÌÔ fiÓÔ˘), Ë ÂÚÈÊÂÚÈ΋ ÌÔÓÔÓ¢ÚÔ-

¿ıÂÈ·, Ë ‰˘Û·˘ÙÔÓÔÌ›·, Ô ˘Ô- ‹ ˘ÂÚı˘ÚÂ-

ÔÂȉÈÛÌfi˜, Ô ˘ÂÚ·Ú·ı˘ÚÂÔÂȉÈÛÌfi˜ Î·È Ù·

ÊÏÂÁÌÔÓÒ‰Ë ÓÔÛ‹Ì·Ù· ÙÔ˘ ÂÓÙ¤ÚÔ˘. ¶˘ÚÂ-

Ùfi˜, Ó˘ÎÙÂÚÈÓfi˜ ȉÚÒÙ·˜, ·ÒÏÂÈ· ‚¿ÚÔ˘˜,

ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ÔÚÁ·ÓÔÌÂÁ·Ï›·, Ê·ÈÓfiÌÂÓÔ

¶·È‰È·ÙÚÈ΋ 2005;68:342-350

¶›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¿ ™˘¯ÓfiÙËÙ· % ηٿ ™˘¯ÓfiÙËÙ· % ηٿ Ì ÈÓÔÌ˘·ÏÁ›· Siegel Î·È Û˘Ó, 1998 (n=45) Gedalia Î·È Û˘Ó, 2000 (n=59)

¢È·Ù·Ú·¯¤˜ ‡ÓÔ˘ 96 70∫ÂÊ·Ï·ÏÁ›· 71 76°ÂÓÈÎÂ˘Ì¤ÓË ÎfiˆÛË 62 20¶ÚˆÈÓ‹ ‰˘Ûη̄›· 53 30¶ÚˆÈÓ‹ ÎfiˆÛË 49 -∫·Ù¿ıÏÈ„Ë 43 7∂ȉ›ӈÛË ÌÂÙ¿ ·fi ¿ÛÎËÛË 42 -O›‰ËÌ· ·ÚıÚÒÛÂˆÓ 40 24∂˘ÂÚ¤ıÈÛÙÔ ¤ÓÙÂÚÔ 38 -¢˘ÛÌËÓfiÚÚÔÈ· 36 -ªÂÙ·‚ÔϤ˜ Ù˘ ÓfiÛÔ˘ ·Ó¿ÏÔÁ· Ì ÙÔÓ Î·ÈÚfi 36 -¶·Ú·ÈÛı‹ÛÂȘ 24 -ÕÁ¯Ô˜ 22 -“ ŒÏÏÂÈÌÌ· ÂÓ¤ÚÁÂÈ·˜” 18 -º·ÈÓfiÌÂÓÔ Raynaud 13 -∫ÔÈÏÈ·Îfi ¿ÏÁÔ˜ - 17ÀÂÚÎÈÓËÙÈÎfiÙËÙ· ·ÚıÚÒÛÂˆÓ 40 14

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·347

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348 µ. ∆Û·ÁÚ‹˜ Î·È Û˘Ó.

Raynaud, Ó¢ÚÔ¿ıÂÈ·, Ô›‰ËÌ· ‹ ÂÚÈÔÚÈÛÌfi˜

Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜ ÙˆÓ ·ÚıÚÒÛˆÓ, ηıÒ˜ ηÈ

·ıÔÏÔÁÈο ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·, ·ÔÙÂ-

ÏÔ‡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, ›Û˘, Ó· ·ÔÎÏ›ÂÙ·È Ë Â˘·ÈÛıËÛ›· ÛÂ

Ê¿Ú̷η fiˆ˜ ‰ÈÔ˘ÚËÙÈο, ηʽÓË, ·ÏÎÔfiÏ

‹ ·ÌÊÂÙ·Ì›ÓË, Ô˘ ÌÔÚ› Ó· ÚÔ‚¿ÏÏÂÈ ÌÂ

Û˘ÌÙÒÌ·Ù· ·ÚfiÌÔÈ· Ì ÂΛӷ Ù˘ ÈÓÔ-

Ì˘·ÏÁ›·˜. ™ËÌÂÈÒÓÂÙ·È fiÙÈ Ë ÈÓÔÌ˘·ÏÁ›· ‰ÂÓ

Â›Ó·È Û˘ÓÒÓ˘ÌË Ì ÙÔÓ „˘¯ÔÁÂÓ‹ ÚÂ˘Ì·ÙÈÛÌfi,

ÛÙÔÓ ÔÔ›Ô Ô ·ÛıÂÓ‹˜ ·ÚÔ˘ÛÈ¿˙ÂÈ ÙË ¯·Ú·-

ÎÙËÚÈÛÙÈ΋ “ÌË ÌÔ˘ ¿ÙÔ˘” Û˘ÌÂÚÈÊÔÚ¿ ηÈ

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˘ÌÙÒÌ·Ù· Ó· ÂÈ-

̤ÓÔ˘Ó ‰È¿ ‚›Ô˘ (36,37). ∂Ï¿¯ÈÛÙ· Â›Ó·È ÁÓˆÛÙ¿

Û¯ÂÙÈο Ì ÙË Ê˘ÛÈ΋ ÈÛÙÔÚ›· Î·È ÙË Ì·ÎÚÔ¯Úfi-

ÓÈ· 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).

∞ÓÙÈÌÂÙÒÈÛË

ªÂ ÙÔ ·ÈÙÈÔÏÔÁÈÎfi ˘fi‚·ıÚÔ Ù˘ ÓfiÛÔ˘ Ó·

‰È¢ڇÓÂÙ·È Û˘Ó¯Ҙ, Ë ÛÙÚ·ÙËÁÈ΋ ıÂڷ¢-

ÙÈ΋˜ ·Ú¤Ì‚·Û˘ ÛÙËÓ ÈÓÔÌ˘·ÏÁ›· ·Ú·Ì¤ÓÂÈ

ÔχÏ¢ÚË Î·È Ì ȉȷ›ÙÂÚ· ÂÎÙÂٷ̤ÓÔ Ê¿-

ÛÌ·. ∫·ıÒ˜ “Ì·ÁÈÎfi ¯¿È” ‰ÂÓ ˘¿Ú¯ÂÈ, Úˆ-

Ù·Ú¯ÈÎfi˜ ÛÙfi¯Ô˜ Â›Ó·È Ô ÂÚÈÔÚÈÛÌfi˜ ÛÙÔÓ Ì¤-

ÁÈÛÙÔ ‰˘Ó·Ùfi ‚·ıÌfi Ù˘ ¤ÎÊÚ·Û˘ ÙˆÓ Û˘-

ÌÙˆÌ¿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 ıÂڷ¢ÙÈ΋ ·Ú¤Ì‚·ÛË Ú¤ÂÈ Ó·

ÂÍ·ÙÔÌÈ·ÂÙ·È, ·ÔÙÂÏÒÓÙ·˜ - Û οı ÂÚ›-

ÙˆÛË - ÚÔ˚fiÓ Ì›·˜ ÔÌ·‰È΋˜, ‰ÈÂÈÛÙËÌÔÓÈ-

΋˜ ÚÔÛ¤ÁÁÈÛ˘. OÈ ‚·ÛÈΤ˜ ·Ú¯¤˜ ·˘Ù‹˜ Ù˘

·ÓÙÈÌÂÙÒÈÛ˘ ·Ú·Ù›ıÂÓÙ·È ÛÙË Û˘Ó¤¯ÂÈ·.

º·Ú̷΢ÙÈ΋ ·ÁˆÁ‹

∆· ÂÚÈÛÛfiÙÂÚ· ·fi Ù· ¯ÚËÛÈÌÔÔÈÔ‡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·ÚÌ·-

΢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË ÂÚÈÏ·Ì‚¿ÓÂÈ ¯ÔÚ‹ÁËÛË

Ì˘Ô¯·Ï·ÚˆÙÈÎÒÓ ÙˆÓ ÛÎÂÏÂÙÈÎÒÓ Ì˘ÒÓ (fiˆ˜

Ë Î˘ÎÏÔ‚ÂÓ˙·Ú›ÓË, Ë ÔÔ›· ‰ÂÓ Î˘ÎÏÔÊÔÚ›

ÛÙËÓ ÂÏÏËÓÈ΋ ·ÁÔÚ¿. ∂Ó·ÏÏ·ÎÙÈο, Û ·È‰È¿

ËÏÈΛ·˜ >12 ÂÙÒÓ, ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈË-

ıÔ‡Ó Ë Î·ÚÈÛÔÚÔ‰fiÏË ‹ Ë ÔÚÊÂÓ·‰Ú›ÓË) ‹/ηÈ

·ÓÙÈηıÏÈÙÈÎÒÓ (ÙÚÈ΢ÎÏÈÎÒÓ fiˆ˜ Ë ·ÌÈÙÚÈ-

Ù˘Ï›ÓË ‹ ÂÈÏÂÎÙÈÎÒÓ ·Ó·ÛÙÔϤˆÓ ·ӷ-

ÚfiÛÏ˄˘ ÛÂÚÔÙÔÓ›Ó˘ - SSRIs - fiˆ˜ Ë

ÊÏÔ˘ÔÍÂÙ›ÓË) Û ¯·ÌËÏ‹ ‰fiÛË. ™Â ÌÂϤÙË ÙÔ˘

1996 Û ÂÓ‹ÏÈΘ Ì ÈÓÔÌ˘·ÏÁ›·, ‰È·ÈÛÙÒıËÎÂ

fiÙÈ Ë Û˘Ó‰˘·Ṳ̂ÓË ¯ÔÚ‹ÁËÛË ·ÓÙÈηٷıÏÈÙÈ-

ÎÒÓ, 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 ·Ô-

ÙÂϤÛÌ·Ù· Ì ÙË ¯Ú‹ÛË ·Ó·ÏÁËÙÈÎÒÓ, fiˆ˜ Ë

·Ú·ÎÂÙ·ÌfiÏË, ‹ ÌË ÛÙÂÚÔÂȉÒÓ ·ÓÙÈÊÏÂÁÌÔ-

Óˆ‰ÒÓ (NSAIDs - .¯. È‚Ô˘ÚÔʤÓË, ÈÓ‰ÔÌÂı·-

ΛÓË), ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·ÓÙÈηٷıÏÈÙÈο ηÈ

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,

fiˆ˜ ÔÈ ·Ó·ÛÙÔÏ›˜ ÙÔ˘ Cox II ÂÓ˙˘ÌÈÎÔ‡ Û˘-

ÛÙ‹Ì·ÙÔ˜, ‰ÂÓ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û ·È‰È¿, ÂÓÒ

ÙÂÏÂ˘Ù·›· ÂÎÊÚ¿˙ÔÓÙ·È ÂÈÊ˘Ï¿ÍÂȘ Û¯ÂÙÈο ÌÂ

ÙËÓ ·ÛÊ¿ÏÂÈ¿ ÙÔ˘˜ Î·È Û ÂÓ‹ÏÈΘ. ∏ ¯Ú‹ÛË ÛÂ

·È‰È¿ ·˘ÙÔÎfiÏÏËÙˆÓ ·Ó·ÏÁËÙÈÎÒÓ “ÂÌ‚·Ïˆ-

Ì¿ÙˆÓ” (patches), Ù· ÔÔ›· ÂÚȤ¯Ô˘Ó ÏȉÔη˝ÓË

Î·È Û˘Ó‰˘¿˙Ô˘Ó Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· Ù˘ ·˘ÍË̤-

Ó˘ Û˘ÌÌfiÚʈÛ˘, ÙˆÓ ÌÂÈˆÌ¤ÓˆÓ Û˘ÛÙËÌ·ÙÈ-

ÎÒÓ ·ÓÂÈı‡ÌËÙˆÓ ÂÓÂÚÁÂÈÒÓ Î·È Ù˘ ·˘ÍË̤-

Ó˘ ÙÔÈ΋˜ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜, ·ÚÔ˘ÛÈ¿-

˙ÂÈ ÂӉȷʤÚÔÓ. ¢È¯ÔÁӈ̛· ÂÈÎÚ·Ù› ˆ˜ ÚÔ˜

ÙÔ Û··ÛÌ· Ô˘ Ú¤ÂÈ Ó· ÂÈϤÁÂÙ·È ̂ ˜ ÂÓ·Ú-

ÎÙ‹ÚÈÔ˜ ·ÁˆÁ‹, ηıÒ˜ ÔÚÈṲ̂ÓÔÈ Û˘ÁÁÚ·Ê›˜

ÚÔÙ›ÓÔ˘Ó Ù· Ì˘Ô¯·Ï·ÚˆÙÈο, ÂÓÒ ¿ÏÏÔÈ Ù·

·ÓÙÈηٷıÏÈÙÈο Û ¯·ÌËÏ‹ ‰ÔÛÔÏÔÁ›· (9,34).

¶Ï‹ıÔ˜ ¿ÏÏˆÓ Û΢·ÛÌ¿ÙˆÓ Ô˘ ¤¯Ô˘Ó ‰Ô-

ÎÈÌ·ÛÙ› Û ÂÓ‹ÏÈΘ, fiˆ˜ ‚ÂÓ˙ԉȷ˙›Ó˜,

ηٷÛÙ·ÏÙÈο ÙÔ˘ ∫¡™, ·Ó·ÛÙÔÏ›˜ ÙÔ˘ ˘Ô-

ÛÙÚÒÌ·ÙÔ˜ ƒ, ·ÓÙÈÂÈÏËÙÈο ‹ ·ÎfiÌ· Î·È ·ÓÙÈ-

ÌÈÎÚԂȷο, ‰ÂÓ ÌÔÚÔ‡Ó Û‡Ìʈӷ Ì ٷ ‰È·-

ı¤ÛÈÌ· ‰Â‰Ô̤ӷ Ó· ÂÓÙ·¯ıÔ‡Ó ÛÙËÓ Â›ÛËÌË

ıÂڷ¢ÙÈ΋ Ê·Ú¤ÙÚ·. ¡ÂfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜,

¶·È‰È·ÙÚÈ΋ 2005;68:342-350

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·348

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349∏ ÈÓÔÌ˘·ÏÁ›· ÛÙËÓ ·È‰È΋ ËÏÈΛ·

fiˆ˜ ·ÁˆÓÈÛÙ¤˜ ÓÙÔ·Ì›Ó˘, ÂÎÏÂÎÙÈÎÔ› ·Ó·-

ÛÙÔÏ›˜ ·ӷÚfiÛÏ˄˘ ÛÂÚÔÙÔÓ›Ó˘ Î·È ÓÔ-

Ú·‰ÚÂÓ·Ï›Ó˘ (fiˆ˜ 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˘ÌÂÚÈÊÔÚÈ΋˜ „˘-

¯ÔıÂڷ›·˜ ¤¯ÂÈ Î·Ù¿ ÂÚÈÙÒÛÂȘ ·Ô‰ÂÈ-

¯ı› ¯Ú‹ÛÈÌË, ·Ó Î·È ‰ÂÓ ˘¿Ú¯ÂÈ Ô ÈηÓfi˜

·ÚÈıÌfi˜ ÌÂıÔ‰ÔÏÔÁÈο ÔÏÔÎÏËÚˆÌ¤ÓˆÓ ÌÂÏÂ-

ÙÒÓ Û ·È‰È¿ Ô˘ ı· ÌÔÚÔ‡Û·Ó Ó· ÙÂÎÌËÚÈÒ-

ÛÔ˘Ó ÙË ¯ÚËÛÈÌfiÙËÙ¿ Ù˘. ™Â Ì›· ÌÂϤÙË ·Ó·-

ʤÚÂÙ·È fiÙÈ Ë ÂÊ·ÚÌÔÁ‹ ÁÓˆÛÈ·ÎÒÓ-Û˘ÌÂÚÈ-

ÊÔÚÈÎÒÓ Ù¯ÓÈÎÒÓ Ô‰‹ÁËÛ Û ‡ÊÂÛË ÙˆÓ Û˘-

ÌÙˆÌ¿ÙˆÓ Û ‰È¿ÛÙËÌ· 4-24 ÌËÓÒÓ (40).

º˘ÛÈ΋ Î·È ÎÔÈÓˆÓÈ΋ ·ÔηٿÛÙ·ÛË

º˘ÛÈÎÔıÂڷ›·, ¯ÂÈÚÔÌ·Ï¿ÍÂȘ, ÔÛÙÂÔ·-

ıËÙÈΤ˜ Ù¯ÓÈΤ˜, ¯Ú‹ÛË ËÏÂÎÙÚÈ΋˜ ‰È·‰ÂÚÌÈ-

΋˜ Ó¢ÚÈ΋˜ ‰È¤ÁÂÚÛ˘ (TENS), ηıÒ˜ ηÈ

‰È·Ù·ÙÈΤ˜ Î·È ·ÂÚÔ‚ÈΤ˜ ·Û΋ÛÂȘ ¤¯Ô˘Ó ‰ÔÎÈ-

Ì·ÛÙ› Ì ÂÈÙ˘¯›· Û ÂÓ‹ÏÈΘ, ‰ÂÓ ˘¿Ú¯ÂÈ

fï˜ ÛËÌ·ÓÙÈ΋ ÂÌÂÈÚ›· Û ·È‰È¿. ∏ ÂÈ-

ÛÙÚÔÊ‹ ÛÙȘ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÙÔ˘ ·È‰ÈÔ‡ Ô˘

‰È·ÎfiËÎ·Ó ÏfiÁˆ ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Ù˘ Ófi-

ÛÔ˘ (Ù·ÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË Û¯ÔÏ›Ԣ, ÎÔÈ-

ÓˆÓÈ΋ ·ÏÏËÏ›‰Ú·ÛË, ·È¯Ó›‰È, ‹È· ¿ıÏË-

ÛË) η٤¯ÂÈ ÚˆÙ·Ú¯È΋ ÛËÌ·Û›·. ∫¿ÔÈÔÈ

Û˘ÁÁÚ·Ê›˜, fiˆ˜ Ô Gedalia, ÚÔÙ›ÓÔ˘Ó Úfi-

ÁÚ·ÌÌ· ‹È·˜ ¿ÛÎËÛ˘ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ÎÔ-

χÌÈ, ÂÚ¿ÙËÌ· ‹ Ô‰ËÏ·Û›·, ÁÈ· ÙÔ˘Ï¿¯È-

ÛÙÔÓ ÌÈÛ‹ ÒÚ· ËÌÂÚËÛ›ˆ˜ (9).

™˘ÌÂÚ¿ÛÌ·Ù· - ÙÔ Ì¤ÏÏÔÓ

¶·Ú¿ ÙË ÛËÌ·ÓÙÈ΋ ÚfiÔ‰Ô Ô˘ ¤¯ÂÈ ÛËÌÂÈ-

ˆı› ÛÙË ‰È·‰Èηۛ· ÚÔÛ¤ÁÁÈÛ˘ Î·È Î·Ù·ÓfiË-

Û˘ Ù˘ ÈÓÔÌ˘·ÏÁ›·˜ ÛÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÂÏ¢-

Ù·›ˆÓ ‰ÂηÂÙÈÒÓ, ÂÏ¿¯ÈÛÙ· ¤¯Ô˘Ó ÌÂÙ·‚ÏËı›

fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ ‹ ÛÙË

‰˘Ó·ÙfiÙËÙ· ·ÔÙÂÏÂÛÌ·ÙÈ΋˜ Î·È ·ÛÊ·ÏÔ‡˜

·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Ù˘. ¶·Ú·-

̤ÓÂÈ, ¤ÙÛÈ, ۷ʤ˜ fiÙÈ Î·Ì›· ÛÙÚ·ÙËÁÈ΋ ·ÓÙÈ-

ÌÂÙÒÈÛ˘ Ù˘ ÓfiÛÔ˘ ‰ÂÓ ÚfiÎÂÈÙ·È Ó· ¢ԉˆ-

ı› fiÛÔ ÙÔ ·ÈÙÈÔ·ıÔÁÂÓÂÙÈÎfi ˘fiÛÙڈ̿ Ù˘

·Ú·Ì¤ÓÂÈ ·‰È¢ÎÚ›ÓÈÛÙÔ. ∏ ÂÚ¢ÓËÙÈ΋ ÚÔ-

Û¿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‰Ô-ÁÂÓÒÓ ÔÈÔÂȉÒÓ ÛÙËÓ ·ıÔÁ¤ÓÂÛË Ù˘ ÈÓÔ-Ì˘·ÏÁ›·˜.

µÈ‚ÏÈÔÁÚ·Ê›·

1. Wolfe F, Smythe HA, Yunus MB, Bennett RM,Bombardier C, Goldenberg DL et al. The AmericanCollege of Rheumatology 1990 criteria for the clas-sification of fibromyalgia. Report of the Multicen-ter Criteria Committee. Arthritis Rheum 1990;33:160-172.

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

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350 µ. ∆Û·ÁÚ‹˜ Î·È Û˘Ó.

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

29. Tayag-Kier CE, Keenan GF, Scalzi LV, Schultz B, El-liott J, Zhao RH et al. Sleep and periodic limbmovement in sleep in juvenile fibromyalgia. Pedi-atrics 2000;106:E70.

30. Roizenblatt S, Moldofsky H, Benedito-Silva AA, Tu-fik S. Alpha sleep characteristics in fibromyalgia.Arthritis Rheum 2001;44:222-230.

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

32. Gracely RH, Petzke F, Wolf JM, Clauw DJ. Func-tional magnetic resonance imaging evidence of aug-mented pain processing in fibromyalgia. ArthritisRheum 2002;46:1333-1343.

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38. ªalleson PN, al-Matar M, Petty RE. Idiopathicmusculoskeletal pain syndromes in children. JRheumatol 1992;19:1786-1789.

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351REVIEW ARTICLE

Fibromyalgia in childhood

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

Abstract

Fibromyalgia of childhood is a common musculoskeletal pain disorder characterized by widespreadpersistent 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 functionalability and psychosocial development of the affected children. Although its exact aetiology remainsunknown, it is perceived that a number of factors can contribute to the clinical expression of thedisorder. The therapeutic intervention is non-specific and usually multidisciplinary.

Key words

Fibromyalgia, children, pain, treatment, musculoskeletal.

1 3rd Paediatric Clinic of theUniversity of Athens,“Attikon” UniversityHospital, Haidari, Athens

2 Paediatric Clinic,“Thriassio” GeneralHospital, Elefsina, Athens

Correspondence:

Vassilios Tsagris 3rd Paediatric Clinic of theUniversity of Athens“Attikon” University Hospital1 Rimini Str., Haidari, Athens E-mail: [email protected]

Date of submission: 22-10-2004 Date of approval: 13-04-2005

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ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ: ªÂÏÂÙ‹ıËÎ·Ó ·Ó·‰ÚÔÌÈο Ù· È·ÙÚÈο ·Ú¯Â›· fiÏˆÓ ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔGB 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·ÈÙËÓ ¤ÍÔ‰Ô ÙÔ˘ ·È‰ÈÔ‡ ·fi ÙÔ ÓÔÛÔÎÔÌ›Ô.

∞ÔÙÂϤÛÌ·Ù·: ∫·Ù¿ ÙËÓ ÂÚ›Ô‰Ô ·fi ÙÔÓ π·ÓÔ˘¿ÚÈÔ 1986 ¤ˆ˜ ÙÔÓ π·ÓÔ˘¿ÚÈÔ 2003 ÓÔÛËχÙËηÓÛÙË ª∂£ 12 ·È‰È¿ Ì ۇӉÚÔÌÔ GB (5 ·ÁfiÚÈ·, 7 ÎÔÚ›ÙÛÈ·), ËÏÈΛ·˜ 22 ÌËÓÒÓ ¤ˆ˜ 15 ÂÙÒÓ (̤ÛËËÏÈΛ·: 8,04 ¤ÙË). ¶ÚÔËÁÔ‡ÌÂÓË ÈÔÁÂÓ‹˜ Ïԛ̈ÍË ·Ó·Ê¤ÚÂÙ·È Û 8 ·È‰È¿. ŒÏÂÁ¯Ô˜ ·ÓÙÈۈ̿وӤÁÈÓ 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¿ÛÙËÎÂÚÔÛ‚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 Îϛ̷ηHughes. ∏ ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙËÛË (O¡¶) ¤ÁÈÓ Û fiÏ· Ù· ·È‰È¿ Î·È ‹Ù·Ó ‰È·ÁÓˆÛÙÈ΋ fiÙ·ÓÂÎÙÂϤÛÙËΠÌÂÙ¿ ÙËÓ 7Ë Ë̤ڷ Ù˘ ÓfiÛÔ˘. ™Â Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi (ª∞) Ù¤ıËÎ·Ó 11/12 ·È‰È¿, ÌÂÌ›· ̤ÛË ‰È¿ÚÎÂÈ· ˘ÔÛÙ‹ÚÈ͢ 18,4 Ë̤Ú˜ (·fi 2 ¤ˆ˜ 37 Ë̤Ú˜) Î·È Û 10/12 ·È‰È¿ ¯ÔÚËÁ‹ıË-ΠÁ-ÛÊ·ÈÚ›ÓË (IVIG). ∏ ̤ÛË ‰È¿ÚÎÂÈ· ·Ú·ÌÔÓ‹˜ ÛÙË ª∂£ ‹Ù·Ó 24,8 Ë̤Ú˜ (2-42 Ë̤Ú˜). ∫·-Ó¤Ó· ·È‰› ‰ÂÓ ·Â‚›ˆÛ ·ÎfiÌË Î·È ÌÂÙ¿ ·fi 6 Ì‹Ó˜, ÂÓÒ 10/12 ·È‰È¿ ›¯·Ó Ï‹ÚË ·Ôηٿ-ÛÙ·ÛË Î·È 2/12 ·ÚÔ˘Û›·˙·Ó Ó¢ÚÔÏÔÁÈο ˘ÔÏ›ÌÌ·Ù·.

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∆Ô Û‡Ó‰ÚÔÌÔ Guillain-Barré (GB) Â›Ó·È Ì›·

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353™‡Ó‰ÚÔÌÔ Guillain-Barré

·Ó·ÓÔ‹˜, Ë ÔÔ›· Î·È ıˆÚÂ›Ù·È ÂÈ‚·Ú˘ÓÙÈ-Îfi˜ ·Ú¿ÁÔÓÙ·˜ (1) fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ Úfi-ÁÓˆÛË.

™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ ‹Ù·Ó Ó· ÌÂ-ÏÂÙËıÔ‡Ó Ù· ÎÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο, Ë ÔÚ›·Î·È Ë ¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔGuillain-Barré ÛÙË ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £ÂÚ·-›·˜ (ª∂£).

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ

ªÂÏÂÙ‹ıËÎ·Ó ·Ó·‰ÚÔÌÈο Ù· È·ÙÚÈο ·Ú¯Â›· fiψÓÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔ GB Ô˘ ÓÔÛËχÙËÎ·Ó ÛÙ˪∂£ ·fi ÙÔÓ π·ÓÔ˘¿ÚÈÔ ÙÔ˘ 1986 ¤ˆ˜ ÙÔÓ π·ÓÔ˘¿ÚÈÔÙÔ˘ 2003. ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ ›¯·Ó ÂÍÂÙ·ÛÙ› ·fi ·È‰Ô-Ó¢ÚÔÏfiÁÔ. ∆· ·È‰È¿ Ô˘ Û˘ÌÂÚÈÏ‹ÊıËÎ·Ó ÛÙË ÌÂ-ϤÙË ÏËÚÔ‡Û·Ó Ù· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰Úfi-ÌÔ˘ GB Ô˘ ¤¯Ô˘Ó ıÂÛÈÛÙ› ·fi ÙÔ˘˜ Asbury ηÈCornblath (2).

∫·Ù·ÁÚ¿ÊËÎ·Ó Ù· ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο ÛÙÔÈ-¯Â›·, Ë ·ÁˆÁ‹ Ô˘ ¯ÚËÛÈÌÔÔÈ‹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∂£¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Îϛ̷η Hughes (1). ™ËÌÂÈÒıË-ηÓ, ›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· ¢-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¿Ú-ÎÂÈ· Ù˘ ·Ú·ÌÔÓ‹˜ ÙÔ˘ ·È‰ÈÔ‡ ÛÙË ª∂£ Î·È ÛÙÔ ÓÔ-ÛÔÎÔÌ›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 ÛÙ˪∂£ 12 ·È‰È¿ Ì ۇӉÚÔÌÔ GB (5 ·ÁfiÚÈ·, 7ÎÔÚ›ÙÛÈ·), ËÏÈΛ·˜ 22 ÌËÓÒÓ ¤ˆ˜ 15 ÂÙÒÓ (̤ÛËËÏÈΛ·: 8,04 ¤ÙË).

¶ÚÔËÁÔ‡ÌÂÓË ÈÔÁÂÓ‹˜ Ïԛ̈ÍË ·Ó·Ê¤ÚÂÙ·ÈÛ 8/12 ·È‰È¿ (Ïԛ̈ÍË ·Ó·Ó¢ÛÙÈÎÔ‡ Û 2,ÂÙÈÎÔ‡ Û 2, ¿ÏÏË Â̇ÚÂÙË 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 pneumo-niae Û 2, Èfi˜ ÈÏ·Ú¿˜ Û 1, Campylobacter spp ÛÂ1, Èfi˜ ·ÏÔ‡ ¤ÚËÙ· Û 1, Èfi˜ ·ÓÂÌ¢ÏÔÁÈ¿˜-˙ˆÛÙ‹Ú· Û 1).

¡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‡Û ηÈ

ÛÙÔ˘˜ ·Ó·Ó¢ÛÙÈÎÔ‡˜ ̇˜.

∂›Û˘, fiÏ· Ù· ·È‰È¿ ·ÚÔ˘Û›·Û·Ó Ì›ˆ-

ÛË ‹ ηٿÚÁËÛË ÙˆÓ ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈ-

ÎÒÓ. ÀÂÚ¢·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‹Ïˆ-

ÛË ·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 ÂÈΛÓ-

‰˘ÓË ÁÈ· ÙË ˙ˆ‹ ‰È·Ù·Ú·¯‹ ÙÔ˘ ηډȷÎÔ‡

Ú˘ıÌÔ‡. ∏ ‰È¿ÚÎÂÈ· Ù˘ Ê¿Û˘ Âȉ›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

¶›Ó·Î·˜ 1. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Guillain-Barré

÷ڷÎÙËÚÈÛÙÈο Ô˘ Â›Ó·È ··Ú·›ÙËÙ· ÁÈ· ÙˉȿÁÓˆÛË ¶ÚÔԉ¢ÙÈ΋ ·‰˘Ó·Ì›· ÂÚÈÛÛfiÙÂÚˆÓ - ·fi ÙÔ ¤Ó·¿ÎÚÔ - ÌÂÏÒÓ ªÂ›ˆÛË/ÂÍ¿ÏÂÈ„Ë ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ

÷ڷÎÙËÚÈÛÙÈο 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¿ ÙËÓÔÏÔÎÏ‹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, ›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

÷ڷÎÙËÚÈÛÙÈο 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

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·353

Page 40: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

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˘).

∞ÓÙÈÌÂÙÒÈÛË - £Âڷ›·

ŒÓÙÂη ·È‰È¿ Ù¤ıËÎ·Ó Û ª∞ ÏfiÁˆ Ù˘

·Ó·Ó¢ÛÙÈ΋˜ ·Ó¿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 ÔÔ›· ÎÚ›ıËÎÂ

ÛÎfiÈÌË ÏfiÁˆ Ù˘ ·Ú·ÙÂٷ̤Ó˘ ‰È·ÛˆÏ‹Óˆ-

Û˘ (22, 27 Î·È 37 Ë̤Ú˜).

∂Ó‰ÔÊϤ‚È· Á-ÛÊ·ÈÚ›ÓË (IVIG) ¯ÔÚËÁ‹ıËÎÂ

Û 10/12 ·È‰È¿, ‰ËÏ·‰‹ Û fiÏ· Ù· ·È‰È¿ Ô˘

ÓÔÛËχÙËÎ·Ó ÛÙË ª∂£ ÌÂÙ¿ ÙÔ 1990. ™Â 8

·È‰È¿ ¯ÚËÛÈÌÔÔÈ‹ıËΠÙÔ Û¯‹Ì· 0,4

g/kg/Ë̤ڷ ÁÈ· ¤ÓÙÂ Û˘Ó¯›˜ Ë̤Ú˜ Î·È ÛÂ

‰‡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· ·ӷϋÊıËΠÌÂÙ¿ ·fi 15 Ë̤Ú˜,

¯ˆÚ›˜ ı·̷ÙÈο ·ÔÙÂϤÛÌ·Ù·. ∏ ˘fiÏÔÈË

·ÁˆÁ‹ ÂÚÈÂÏ¿Ì‚·Ó ·Ó·Ó¢ÛÙÈ΋ Î·È ÎÈÓËÙÈ-

΋ Ê˘ÛÈÔıÂڷ›·, ÚfiÏË„Ë Î·È ·ÓÙÈÌÂÙÒÈÛË

ÙˆÓ ÏÔÈÌÒ͈Ó, ·ÓÙÈÌÂÙÒÈÛË fiÓÔ˘, „˘¯ÔÏÔ-

ÁÈ΋ ˘ÔÛÙ‹ÚÈÍË, ηıÒ˜ Î·È ÂÓÙÂÚÈ΋ Û›ÙÈÛË.

¶ÔÚ›· ÓfiÛÔ˘ - ŒÎ‚·ÛË - ∂ÈÏÔΤ˜

∏ ‰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 ÂÈ‚›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΋ ¿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 ÓÔÛÔ-

ÎÔÌ›Ô, Ù· ·È‰È¿ ·Ú·ÎÔÏÔ˘ı‹ıËÎ·Ó ÛÙ·

Â͈ÙÂÚÈο È·ÙÚ›· ̤¯ÚÈ ÙËÓ Ï‹ÚË ·Ôηٿ-

ÛÙ·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-

ÛËÏ›·˜, ·Ú¤ÌÂÈÓ Ì ÂÈÎfiÓ· ‚·ÚÈ¿˜ ˘ÔÍ·ÈÌÈ-

΋˜-ÈÛ¯·ÈÌÈ΋˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜. ∆Ô ‰Â‡ÙÂÚÔ

·È‰› ›¯Â ‰È·Ù·Ú·¯¤˜ ‚¿‰ÈÛ˘ (·ÛÙ¿ıÂÈ· ‚¿‰È-

Û˘). ∫·Ó¤Ó· ·È‰› ‰ÂÓ ·Â‚›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 ·Ô-

Ù¤ÏÂÛÌ· ÙË ÊÏÂÁÌÔÓÒ‰Ë ‰È‹ıËÛË ÙÔ˘ ÂÓ‰ÔÓ¢-

Ú›Ô˘, ÙÔ Ô›‰ËÌ· Î·È ÙËÓ ÙÌËÌ·ÙÈ΋ ·ÔÌ˘ÂÏ›-

ÓˆÛË (3). ™ÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ· ÙÔ˘ Û˘Ó‰Úfi-

ÌÔ˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÏÔÈÌÔÁfiÓÔÈ ·Ú¿ÁÔÓÙ˜,

ηÎÔ‹ıÂȘ, ¯ÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ Î·È ‰È¿-

ÊÔÚ· Ê¿Ú̷η. ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ˘‹Ú¯Â

ıÂÙÈÎfi ÈÛÙÔÚÈÎfi ÁÈ· ÚÔËÁÔ‡ÌÂÓË ÈÔÁÂÓ‹ ÏÔ›-

̈ÍË 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˘ÌÌÂ-

ÙÚÈ΋ 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, ÌÂ

·ÎfiÏÔ˘ıÔ ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ·.

™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È fiÙÈ ÂÚ›Ô˘ 20-

30% ÙˆÓ ·ÛıÂÓÒÓ (·È‰È¿ Î·È ÂÓ‹ÏÈΘ) ·-

ÚÔ˘ÛÈ¿˙ÂÈ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿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΋ ·Ó¿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˘ÓÈ-

ÛÙ¿Ù·È ÔÈ ·ÛıÂÓ›˜ Ó· ‰È·ÛˆÏËÓÒÓÔÓÙ·È ÓˆÚ›˜,

ÚÈÓ ÂÁηٷÛÙ·ı› Ë ·Ó·Ó¢ÛÙÈ΋ ·Ó¿Ú-

ÎÂÈ· (3,5,6).

∏ ‰È·ÎÔ‹ ÙÔ˘ ª∞ ÂȯÂÈÚÂ›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΋ ηÈ

¤¯Ô˘Ó ·ӤÏıÂÈ 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 Ë̤-

Ú˜ (̤ÛÔ˜ fiÚÔ˜: 18,4 Ë̤Ú˜). ™Â ·ÓÙ›ÛÙÔȯ˜

¶·È‰È·ÙÚÈ΋ 2005;68:352-356

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355™‡Ó‰ÚÔÌÔ Guillain-Barré

ÌÂϤÙ˜ ·È‰ÈÒÓ ·Ó·Ê¤ÚÔÓÙ·È ·ÚfiÌÔÈ· ÛÙÔÈ-

¯Â›· (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

ÛÂ ÔÛÔÛÙfi ÂÚ›Ô˘ 35-45% (4). ∞fi Ù· ·È-

‰È¿ Ù˘ ÛÂÈÚ¿˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, ÚÔÛ‚Ô-

Ï‹ 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‚ÏÈÔ-

ÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È ˆ˜ ‰Â‡ÙÂÚË ·ÈÙ›· ÂÈÛ·Áˆ-

Á‹˜ ·ÛıÂÓÒÓ Ì ۇӉÚÔÌÔ GB ÛÙË ª∂£. ∏

ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó·ÎÔ‹ Ô˘ ·Ú·ÙËÚ‹-

ıËΠ۠¤Ó·Ó ·ÛıÂÓ‹ ‹Ù·Ó ·ÔÙ¤ÏÂÛÌ· ˘ÔÍ·È-

Ì›·˜ 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΋ ıÂڷ›· ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË

ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ GB ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› Ë

ÎÔÚÙÈ˙fiÓË, Ë Ï·ÛÌ·Ê·›ÚÂÛË Î·È Ë IVIG. ∏

¯ÔÚ‹ÁËÛË ÎÔÚÙÈ˙fiÓ˘ ¤¯ÂÈ ÂÁηٷÏÂÈÊı›, η-

ıÒ˜ ¤¯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·›· ‰Âη-

ÂÙ›·. OÈ ÂÚÈÛÛfiÙÂÚÔÈ ÂÚ¢ÓËÙ¤˜ ˘ÔÛÙËÚ›-

˙Ô˘Ó fiÙÈ ¤¯ÂÈ Î·Ï¿ ·ÔÙÂϤÛÌ·Ù·, ‰ÈfiÙÈ ÂÏ·Ù-

ÙÒÓÂÈ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÔͤԘ ÛÙ·‰›Ô˘, ‚ÂÏÙÈÒ-

ÓÂÈ 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· ÂȂ‚·ÈÒ-

ÓÔ˘Ó ÙËÓ ·Í›· Ù˘ ¯ÔÚ‹ÁËÛ˘ IVIG (8,9). ªÂ-

ÚÈÎÔ› Û˘ÁÁÚ·Ê›˜ ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ Ë ¯ÔÚ‹ÁË-

ÛË IVIG Û ·È‰È¿ ÌÔÚ› Ó· Â›Ó·È ÏÈÁfiÙÂÚÔ

·ÔÙÂÏÂÛÌ·ÙÈ΋ ·fi ÙÔ˘˜ ÂÓ‹ÏÈΘ ‹ Ó· ›ӷÈ

·ÔÙÂÏÂÛÌ·ÙÈ΋ ÌfiÓÔ fiÙ·Ó ¯ÔÚËÁËı› ÛÂ Û˘-

ÁÎÂÎÚÈ̤ÓÔ˘˜ ·ÛıÂÓ›˜ Î·È Ôχ ÓˆÚ›˜ ÛÙËÓ

ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘ (8). O Û˘Ó‰˘·ÛÌfi˜ IVIG ηÈ

Ï·ÛÌ·Ê·›ÚÂÛ˘ ‰ÂÓ ¤¯ÂÈ ÛËÌ·ÓÙÈο ηχÙÂ-

Ú· ·ÔÙÂϤÛÌ·Ù· (10).

™Â fi,ÙÈ ·ÊÔÚ¿ ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘, ·˘Ù‹

Â›Ó·È Î·Ï‹ ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛˆÓ.

∆Ô 80-85% ÙˆÓ ·ÛıÂÓÒÓ ¤¯ÂÈ Ï‹ÚË ·Ôηٿ-

ÛÙ·ÛË. ∆Ô ÔÛÔÛÙfi Ù˘ ıÓËÙfiÙËÙ·˜, ·Ú¿ ÙȘÚÔfi‰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). ÀÔÙÚÔ‹ - ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ófi-ÛÔ˘ ‹ ·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 ÂÈ‚·Ú˘ÓÙÈÎfi˜ ·Ú¿ÁÔ-ÓÙ·˜ 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 ηӤ-Ó·˜ ı¿Ó·ÙÔ˜, fï˜ ‰‡Ô ·È‰È¿ ÂÌÊ¿ÓÈÛ·Ó ÌfiÓÈ-̘ Ó¢ÚÔÏÔÁÈΤ˜ ÂÈÏÔΤ˜. ™ÙÔ ÚÒÙÔ ·fi·˘Ù¿, Ë Ó¢ÚÔÏÔÁÈ΋ Û˘Ó‰ÚÔÌ‹ ‰ÂÓ ÔÊÂÈÏfiÙ·ÓÛÙËÓ ›‰È· ÙËÓ ¿ıËÛË, ·ÏÏ¿ ÛÙËÓ Î·Ú‰ÈÔ·Ó·-Ó¢ÛÙÈ΋ ·Ó·ÎÔ‹ Ô˘ ˘¤ÛÙË. ∆Ô ‰Â‡ÙÂÚԷȉ› ›¯Â ·ÚÔ˘ÛÈ¿ÛÂÈ ‹‰Ë ‰‡Ô ˘ÔÙÚÔ¤˜fiÙ·Ó ÓÔÛËχÙËΠÛÙÔ ÙÌ‹Ì·. O ¯ÚfiÓÔ˜ ·Ô-ηٿÛÙ·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·fi24 ¤ˆ˜ 79 Ë̤Ú˜ (̤ÛÔ˜ fiÚÔ˜: 51,7 Ë̤Ú˜) Î·È‹Ù·Ó ÈÔ Û‡ÓÙÔÌË ·fi ÂΛÓË Ô˘ ·Ó·Ê¤ÚÂÙ·ÈÛÙË ‚È‚ÏÈÔÁÚ·Ê›· (4,8).

™˘ÌÂÚ¿ÛÌ·Ù·

∆Ô Û‡Ó‰ÚÔÌÔ Guillain-Barré ›¯Â Û¯ÂÙÈοηϋ ¤Î‚·ÛË ÛÙ· ·È‰È¿ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤ-Ù˘ Ô˘ ÓÔÛËχÙËÎ·Ó ÛÙË ª∂£, ·ÎfiÌ· ηÈÛÙȘ ÂÚÈÙÒÛÂȘ Ô˘ ··ÈÙ‹ıËΠÌ˯·ÓÈ΋˘ÔÛÙ‹ÚÈÍË Ù˘ ·Ó·ÓÔ‹˜ ÏfiÁˆ Ù˘ ‚·Ú‡ÙË-Ù·˜ Ù˘ ÓfiÛÔ˘.

∏ ‰È·ÛˆÏ‹ÓˆÛË Ú¤ÂÈ Ó· ·ÔÊ·Û›˙ÂÙ·ÈÂÁη›Úˆ˜, Ì ÙËÓ ÂÌÊ¿ÓÈÛË ·Ó·ÔÙÂÏÂÛÌ·ÙÈ-ÎÔ‡ ‚‹¯·, Ì˘˚΋˜ ·‰˘Ó·Ì›·˜ ·Ó·Ó¢ÛÙÈÎÒÓÌ˘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 ÂÈÏÔÁ‹ Û·Ó ÂȉÈ΋ ·ÓÙÈÌÂÙÒÈÛËÙ˘ 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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356 ∞. ∆ÛÔ‡ÙÛÔ˘ Î·È Û˘Ó.

syndrome requiring mechanical ventilation. Neu-rology 2000;54:2311-2315.

2. Asbury AK, Cornblath DR. Assessment of currentdiagnostic criteria for Guillain-Barré syndrome.Ann Neurol 1990;27 (Suppl):S21-S24.

3. Ropper AH. Critical care of Guillain-Barré syn-drome. In: Ropper AH, editor. Neurological andneurosurgical intensive care. 3rd ed. New York:Raven Press; 1993. p. 363.

4. Korinthenberg R, Monting JS. Natural history andtreatment effects in Guillain-Barré syndrome: amulticentre study. Arch Dis Child 1996;74:281-287.

5. Hund EF, Borel CO, Cornblath DR, Hanley DF,McKhann GM. Intensive management and treat-ment of severe Guillain-Barré syndrome. Crit CareMed 1993;21:433-446.

6. Bos AP, van der Meche FG, Witsenburg M, van derVoort E. Experiences with Guillain-Barré syndromein a pediatric intensive care unit. Intensive CareMed 1987;13:328-331.

7. van der Meche FG, Schmitz PI. Dutch Guillain-Bar-ré Study Group. A randomized trial comparing in-travenous immune globulin and plasma exchange in

Guillain-Barré syndrome. N Engl J Med 1992;326:1123-1129.

8. Graf WD, Katz JS, Eder DN, Smith AJ, Chun MR.Outcome in severe pediatric Guillain-Barré syn-drome after immunotherapy or supportive care.Neurology 1999;52:1494-1497.

9. Gurses N, Uysal S, Cetinkaya F, Islek I, Kalayci AG.Intravenous immunoglobulin treatment in childrenwith Guillain-Barré syndrome. Scand J Infect Dis1995;27:241-243.

10. Plasma Exchange/Sandoglobulin Guillain-BarréSyndrome Trial Group. Randomised trial of plasmaexchange, intravenous immunoglobulin, and com-bined treatments in Guillain-Barré syndrome.Lancet 1997;349:225-230.

11. Henderson RD, Lawn ND, Fletcher DD, McClellandRL, Wijdicks EF. The morbidity of Guillain-Barrésyndrome admitted to the intensive care unit. Neu-rology 2003;60:17-21.

12. Lawn ND, Fletcher DD, Henderson RD, WolterTD, Wijdicks EF. Anticipating mechanical ventila-tion in Guillain-Barré syndrome. Arch Neurol2001;58:893-898.

¶·È‰È·ÙÚÈ΋ 2005;68:352-356

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357ORIGINAL ARTICLE

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 acuteonset, evolution over days or weeks and a good outcome in most cases. The aim of this study was toanalyze the clinical course and outcome in children with GBS requiring hospitalization in a PaediatricIntensive Care Unit (PICU).

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

Results: Twelve children, with GBS, 5 males, mean age 8.04 years (range 22 months - 15 years), wereadmitted to the PICU. In 8/12 patients a preceding viral illness was reported. In 10/12 patientsserological investigations were performed and the aetiological factor was found in 6/10. In additionto weakness and loss of tendon reflexes, 11/12 children presented with cranial nerve palsy and 9/12had autonomic dysfunction (mainly hypertension). All patients had a severity score (Hughes scale) of4 or 5. A lumbar puncture was performed in all patients, with positive findings when it was done 7days or more after the onset of symptoms. Eleven of the 12 patients required mechanical ventilationfor 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 thepatients died, 10 had full recovery but two patients had neurological sequelae.

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

Key words

Guillain-Barré syndrome, mechanical ventilation, intravenous immunoglobulin.

Intensive Care Unit, “P. & A. Kyriakou” Children’sHospital, Athens

Correspondence:

Dimitrios Gionis “P. & A. Kyriakou” Children’sHospital, Thivon & Levadias Str. 115 27, Athens

Date of submission: 10-12-2003 Date of approval: 08-07-2005

Paediatriki 2005;68:357

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·357

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¶·È‰È·ÙÚÈ΋ 2005;68:358-364

∫›Ó‰˘ÓÔ˜ ·fi ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜

·ÙfiÌˆÓ Ì ۇӉÚÔÌÔ Down Î·È ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋

·ÛÙ¿ıÂÈ·

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

¶ÂÚ›ÏË„Ë

∂ÈÛ·ÁˆÁ‹: ™ÎÔfi 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 ÎÏÈ-ÓÈ΋ ·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¿ÛÙËηÓÎÏÈÓÈο ·Ó¿ ¤ÙÔ˜ ÁÈ· ÛËÌÂÈÔÏÔÁ›· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∞fi Ù· ¿ÙÔÌ· ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂ-Ú˘ ÙˆÓ 3 ÂÙÒÓ, ÔÛÔÛÙfi 81% ˘Ô‚Ï‹ıËΠ۠·ÎÙÈÓÔÁڷʛ˜ (·/·) ·˘¯ÂÓÈ΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘-ÏÈ΋˜ ÛÙ‹Ï˘ (∞ª™™), Û ϿÁÈ· Ô˘‰¤ÙÂÚË ı¤ÛË Î·È ÂÈÏÂÎÙÈο Û ϿÁÈ· ο̄Ë. ∏ ·ÙÏ·ÓÙÔ·ÍÔ-ÓÈ΋ ·fiÛÙ·ÛË ÂÎÙÈÌ‹ıËΠˆ˜ ·ıÔÏÔÁÈ΋ fiÙ·Ó ‹Ù·Ó ¿Óˆ ÙˆÓ 4 mm ÁÈ· Ù· ·È‰È¿ ËÏÈΛ·˜ ÌÈÎÚfi-ÙÂÚ˘ ÙˆÓ 15 ÂÙÒÓ Î·È ¿Óˆ ÙˆÓ 3 mm ÁÈ· Ù· ¿ÙÔÌ· ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 15 ÂÙÒÓ. ŸÏ˜ ÔÈ ·ÎÙÈ-ÓÔÁڷʛ˜ ·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˘ÌÙÒÌ·Ù· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ÀÔ‚Ï‹ıËÎ·Ó Û ̷ÁÓË-ÙÈ΋ ÙÔÌÔÁÚ·Ê›· (MRI) ∞ª™™ Î·È Û ηӤӷ ‰ÂÓ ‰È·ÈÛÙÒıËΠ›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡.

™˘ÌÂÚ¿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‰ÈÒÓÌ 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‹ ˘¤Ú-η̄˘-˘ÂÚ¤ÎÙ·Û˘ ÙÔ˘ ·˘¯¤Ó·, ·ӿÏË„Ë Ù˘ ·ÎÙÈÓÔÁÚ·Ê›·˜ Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ· ÚÈÓ ÙËÛ˘ÌÌÂÙÔ¯‹ Û ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ Î·È ÎÏÈÓÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË ÁÈ· Û˘ÌÙÒÌ·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˘ ·Ô-ÙÂÏ› 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·ÍÔÓÈÎfiÂÍ¿ÚıÚËÌ·, ·ÎÙÈÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜, Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·.

™˘ÓÙÔÌÔÁڷʛ˜

DS ™‡Ó‰ÚÔÌÔ Down

∞∞∞ ∞ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·ÛÙ¿ıÂÈ·

C-1 ¶ÚÒÙÔ˜ ·˘¯ÂÓÈÎfi˜ ÛfiÓ‰˘ÏÔ˜

C-2 ¢Â‡ÙÂÚÔ˜ ·˘¯ÂÓÈÎfi˜ ÛfiÓ‰˘ÏÔ˜

∞ª™™ ∞˘¯ÂÓÈ΋ ÌÔ›Ú· ÛÔÓ‰˘ÏÈ΋˜

ÛÙ‹Ï˘

MRI ª·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·

CT ∞ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›·

∞∞¶ ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· Ù˘ ¶·È‰È·ÙÚÈ΋˜

AAP American Academy of Pediatrics

·/· ∞ÎÙÈÓÔÁÚ·Ê›·

AAI 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›‰·358

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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·Ë-ڛ˜, ÔÈ Ôԛ˜ ÌÔÚ› Ó· ·˘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¿ÊÔ-Ú˜ ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÏfiÁˆ Ù˘ ·ÙÏ·-ÓÙÔ·ÍÔÓÈ΋˜ ·ÛÙ¿ıÂÈ·˜ (∞∞∞) (1,2). ø˜ ∞∞∞ ‹·ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ˘ÂÍ¿ÚıÚËÌ· ¯·Ú·ÎÙËÚ›˙ÂÙ·ÈË ·˘ÍË̤ÓË ÎÈÓËÙÈÎfiÙËÙ· ÛÙËÓ ·ÙÏ·ÓÙÔ·ÍÔÓÈ-΋ ¿ÚıÚˆÛË [¿ÚıÚˆÛË ÌÂٷ͇ ÙÔ˘ ÚÒÙÔ˘ (C-1) Î·È ÙÔ˘ ‰Â‡ÙÂÚÔ˘ (C-2) ·˘¯ÂÓÈÎÔ‡ ÛÔÓ‰‡-ÏÔ˘] (∂ÈÎfiÓ· 1) (3,4). ∆· ·›ÙÈ· Ù˘ ∞∞∞ ‰ÂÓ¤¯Ô˘Ó Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈÛÙ›. ªÔÚ› Ó· ÂÓÔ¯Ô-ÔÈÔ‡ÓÙ·È ‰È·Ì·Úٛ˜ ÙˆÓ Û˘Ó‰¤ÛÌˆÓ Ô˘ ‰È·-ÙËÚÔ‡Ó ÙËÓ ·ÎÂÚ·ÈfiÙËÙ· Ù˘ ¿ÚıÚˆÛ˘ ÌÂٷ͇ÙÔ˘ C-1 ‹ C-2 ÛÔÓ‰‡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 ÔÔ›Ô Â›Ó·È·ÂÈÏËÙÈÎfi ÁÈ· ÙË ˙ˆ‹ Î·È ÂÌÊ·Ó›˙ÂÙ·È Ì ÎÏÈ-ÓÈο ÛËÌ›· Î·È Û˘ÌÙÒÌ·Ù· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ-·›Ô˘ Ì˘ÂÏÔ‡, fiˆ˜ ·ÓÒÌ·ÏÔ ‚¿‰ÈÛÌ·, fiÓÔ Î·ÈÂÚÈÔÚÈṲ̂ÓË ÎÈÓËÙÈÎfiÙËÙ· ÙÔ˘ ·˘¯¤Ó·, Ú·È‚fi-ÎÚ·ÓÔ, ·‰ÂÍÈfiÙËÙ·, ·Û˘Ó¤ÚÁÂÈ· Î·È ‰È·Ù·Ú·¯¤˜ÛÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ÛÊÈÁÎÙ‹ÚˆÓ. ∏ Ó¢ÚÔÏÔÁÈ΋ÂͤٷÛË ÌÔÚ› Ó· ·Ôηχ„ÂÈ ·ÈÛıËÙÈΤ˜‰È·Ù·Ú·¯¤˜, Û·ÛÙÈÎfiÙËÙ·, ·˘ÍË̤ӷ ·ÓÙ·Ó·-ÎÏ·ÛÙÈο, ÎÏfiÓÔ Î·È ÙËÓ ·ÚÔ˘Û›· ÙÔ˘ ÛËÌ›ԢBabinski (3,8).

™ÎÔfi˜ Ù˘ ÂÚÁ·Û›·˜ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛËÙ˘ 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 DSËÏÈΛ·˜ 0-40 ÂÙÒÓ, Ù· ÂÚÈÛÛfiÙÂÚ· ·fi Ù· ÔÔ›· ÂÚ¢-

Ó‹ıËÎ·Ó ¯ÚˆÌÔÛˆÌÈο ÛÙÔ ∂ÚÁ·ÛÙ‹ÚÈÔ ∫˘ÙÙ·ÚÔÁÂÓÂ-

ÙÈ΋˜ Ù˘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ∞.¶.£. ¶ÚÔ-

Û‹Ïı·Ó Ì ÚÔÁÚ·ÌÌ·ÙÈṲ̂ӷ Ú·ÓÙ‚ԇ ÛÙ· Â͈ÙÂÚÈ-

ο È·ÙÚ›· °ÂÓÂÙÈ΋˜ ∫·ıÔ‰‹ÁËÛ˘ ÁÈ· ÙÔÓ ÂÙ‹ÛÈÔ

ÚÔÏËÙÈÎfi 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 ÛË-

ÌÂÈÔÏÔÁ›·˜ ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ™‡Ìʈӷ ÌÂ

ÙȘ Ô‰ËÁ›Â˜ Ù˘ ∞ıÏËÙÈ·ÙÚÈ΋˜ ∂ÈÙÚÔ‹˜ Ù˘ A∞¶,

˘Ô‚Ï‹ıËÎ·Ó Û ·/· Ù˘ ·˘¯ÂÓÈ΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ-

‰˘ÏÈ΋˜ ÛÙ‹Ï˘ (∞ª™™) Ù· ¿ÙÔÌ· ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 3

ÂÙÒÓ. OÈ ·Ï¤˜ ·ÎÙÈÓÔÁڷʛ˜ ¤ÁÈÓ·Ó Ì Ì˯¿ÓËÌ·

Merate MMT90 Û ϿÁÈ· 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 ÂÈΤÓÙÚˆÛË 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·˜ ÂÈÊ¿ÓÂÈ·˜ ÙÔ˘ Úfi-

ÛıÈÔ˘ ÙfiÍÔ˘ ÙÔ˘ ¿ÙÏ·ÓÙ· Î·È Ù˘ Ô‰ÔÓÙÔÂȉԇ˜ ·fi-

Ê˘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 ·ÎÙÈÓÔ-

Áڷʛ˜ ·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‹ıË-

Î·Ó Û CT Î·È MRI. OÈ ÂÍÂÙ¿ÛÂȘ MRI ¤ÁÈÓ·Ó Ì ̷-

ÁÓËÙÈÎfi ÙÔÌÔÁÚ¿ÊÔ Siemens Expert plus, 1T Î·È Ï‹ÊıË-

Î·Ó ÂÁοÚÛȘ Î·È Ô‚ÂÏÈ·›Â˜ ÙÔ̤˜ Ì ÂÈ‚¿Ú˘ÓÛË ∆1

Î·È ∆2 ·Ú·Ì¤ÙÚˆÓ Û Ԣ‰¤ÙÂÚË ı¤ÛË ÁÈ· ¤ÏÂÁ¯Ô ›Â-

Û˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡.

°È· ÙË ‰ÈÂÓ¤ÚÁÂÈ· Ù˘ ·/· ˘‹Ú¯Â Ë ¤ÁÎÚÈÛË fiψÓ

ÙˆÓ ÁÔÓ¤ˆÓ. ™ÙÔ˘˜ ÁÔÓ›˜ Ô˘ Ë ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ·fi-

ÛÙ·ÛË ÛÙËÓ ·/· ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘˜ ˘ÂÚ¤‚·ÈÓ ٷ Ê˘-

ÛÈÔÏÔÁÈο fiÚÈ·, ‰fiıËÎ·Ó Ô‰ËÁ›Â˜ ·ÔÊ˘Á‹˜ ÔÚÈṲ̂ӈÓ

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 ϿÁÈ· ·ÎÙÈÓÔÁÚ·Ê›· ∞ª™™. ™Â

43/102 (42%) ‰È·ÈÛÙÒıËΠ∞∞∞ (∂ÈÎfiÓ· 2).

¶·È‰È·ÙÚÈ΋ 2005;68:358-364

∂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).

·

ª¤ÛÔ˜ ·ÍÔÓÔÂȉ‹˜ ‹ÎÚÂÌ·ÛÙ‹ÚÈÔ˜ Û‡Ó‰ÂÛÌÔ˜

ÙÔ˘ ¿ÙÏ·ÓÙ·

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

∫·Ï˘Ù‹ÚÈÔ˜ ˘Ì¤Ó·˜

¶ÚfiÛıÈ· ÙfiÍ· ÙÔ˘ ¿ÙÏ·ÓÙ·

∂ÁοÚÛÈÔ˜ Û‡Ó‰ÂÛÌÔ˜ ÙÔ˘ ¿ÙÏ·ÓÙ·

√›ÛıÈÔ˜ ÂÈÌ‹Î˘ Û‡Ó‰ÂÛÌÔ˜Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘

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360 Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘ Î·È Û˘Ó.

∞fi Ù· ¿ÙÔÌ· ÌÂ ∞∞∞, 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 ÎÔÚÈ-

ÙÛÈÒÓ/·ÁÔÚÈÒÓ ‹Ù·Ó 5/6. ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ËÏÈ-

Λ·, 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˘ÌÙÒÌ·Ù· ‡ÔÙ· ÁÈ· ›ÂÛË

ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∞˘Ù¿ ˘Ô‚Ï‹ıËÎ·Ó ÛÂ

ÂÚ·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô Ù˘ ∞ª™™ Ì CT Î·È Û˘ÁÎÂ-

ÎÚÈ̤ӷ ÔÈ ·ÛıÂÓ›˜ Ì ·ÚÈıÌfi 7, 13, 19 ÙÔ˘ ¶›-

ӷη 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

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-2ÛÔÓ‰‡ÏÔ˘, ÙÔ˘ ¿ÙÏ·ÓÙ· Î·È ÙÔ˘ ¿ÍÔÓ·, ·-ÚÔ˘ÛÈ¿˙ÂÈ ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ·ÛÙ¿ıÂÈ·˜ ·ÎfiÌËÎ·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· ÌÔÚ› Ó· ÚÔ-ÎÏËı› ·fi ÙË ¯·Ï·ÚfiÙËÙ· ÙˆÓ Û˘Ó‰¤Û̈ÓÔ˘ Û˘ÁÎÚ·ÙÔ‡Ó ÙËÓ ¿ÚıÚˆÛË ÛÂ Ê˘ÛÈÔÏÔÁÈ΋ηٿÛÙ·ÛË, ·fi ÔÛÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ ·˘¯Â-ÓÈÎÒÓ ÛÔÓ‰‡ÏˆÓ ‹ Î·È ·fi Ù· ‰‡Ô (1,3).

™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È fiÙÈ Ë Û˘¯Ófi-ÙËÙ· Ù˘ ∞∞∞ ÛÙ· ¿ÙÔÌ· Ì DS Î˘Ì·›ÓÂÙ·È ·fi10% ¤ˆ˜ 40% Î·È Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ËÏÈΛ· ηÈÙÔ Ê‡ÏÔ ÙÔ˘˜ (11-20). ∏ ∞∞∞ ÌÔÚ› Ó· ‰È·È-ÛÙˆı› fi¯È ÌfiÓÔ Û ¿ÙÔÌ· Ì DS, ·ÏÏ¿ Î·È ÛÂÔÚÈṲ̂ÓÔ˘˜ ·ÛıÂÓ›˜ Ì ÚÂ˘Ì·ÙÔÂȉ‹ ·ÚıÚ›ÙÈ-‰·, 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 DSÙ˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, ‰È·ÈÛÙÒıËΠ·ÎÙÈÓÔ-ÏÔÁÈο Û ÔÛÔÛÙfi 42% (43/102), ‹Ù·Ó Û˘¯Ófi-ÙÂÚË ÛÙ· ·ÁfiÚÈ· (·ÁfiÚÈ·/ÎÔÚ›ÙÛÈ· 24/19, 56%)Î·È ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ËÏÈΛ·˜ ÌÈ-ÎÚfiÙÂÚ˘ ÙˆÓ 15 ÂÙÒÓ (27/16, 63%). ∆· ¢ڋ-Ì·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ Û˘ÌʈÓÔ‡Ó ÌÂÂΛӷ ÙˆÓ ¿ÏÏˆÓ ÂÚ¢ÓËÙÒÓ.

∆Ô ·ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ÂÍ¿ÚıÚËÌ·, ·Ó Î·È Â›Ó·ÈÔχ Û·ÓÈfiÙÂÚÔ ·fi ÙËÓ ·ÛÙ¿ıÂÈ·, ›ӷÈ

¶·È‰È·ÙÚÈ΋ 2005;68:358-364

23%

21%16%

40%

∞ÁfiÚÈ· <15 ÂÙÒÓ

∞ÁfiÚÈ· >15 ÂÙÒÓ

∫ÔÚ›ÙÛÈ· >15 ÂÙÒÓ

∫ÔÚ›ÙÛÈ· <15 ÂÙÒÓ

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

∞∞∞42%

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

¶›Ó·Î·˜ 1. ∫ÔÚ›ÙÛÈ· Ì DS Î·È AA∞ [(n=19) (44%)]

OÌ¿‰· No ŸÓÔÌ· ∏ÏÈΛ· ∞ÙÏ·ÓÙÔ·ÍÔÓÈ΋(¤ÙË) ·fiÛÙ·ÛË (mm) -

ı¤ÛË ·/·

<15 ¤ÙË 1 ª. ∂. 8 5 ο̄Ë2 ™. ª. 6 5 Ô˘‰¤ÙÂÚË3 π. ª. 4 7 Ô˘‰¤ÙÂÚË4 µ. ª. 4 5 Ô˘‰¤ÙÂÚË5 £. ™. 4 7 Ô˘‰¤ÙÂÚË6 £. ¡. 11 5 ο̄Ë7* ∆. ¢. 11 9 Ô˘‰¤ÙÂÚË8 °. ª. 10 5 Ô˘‰¤ÙÂÚË

9** ™. ª. 8 6 Ô˘‰¤ÙÂÚË10 ¢. Ã. 7 5 Ô˘‰¤ÙÂÚË

>15 ¤ÙË 11 ∑. ¶. 26 6 Ô˘‰¤ÙÂÚË12 ª. °. 23 6 ο̄Ë13 §. ∂. 30 4 Ô˘‰¤ÙÂÚË14 ∑. ª. 29 7 Ô˘‰¤ÙÂÚË15 °. ∂. 17 8 Ô˘‰¤ÙÂÚË16 ª. °. 17 4 Ô˘‰¤ÙÂÚË17 ™. ™. 18 5 Ô˘‰¤ÙÂÚË18 ƒ. ª. 19 5 Ô˘‰¤ÙÂÚË19 °. ª. 30 4 Ô˘‰¤ÙÂÚË

* ∞ÛıÂÓ‹˜ Ù˘ ∂ÈÎfiÓ·˜ 4 ** ∞ÛıÂÓ‹˜ Ù˘ ∂ÈÎfiÓ·˜ 5

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

·ÂÈÏËÙÈÎfi ÁÈ· ÙË ˙ˆ‹. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi ··È-ÙÂ›Ù·È ˘„ËÏ‹ ¢·ÈÛıËÛ›· ÙˆÓ ÁÈ·ÙÚÒÓ ÁÈ· ÙËÓ·ÔÙÚÔ‹ ηٷÛÙ¿ÛÂˆÓ Ô˘ ÚÔηÏÔ‡Ó ›ÂÛËÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∞fi ÙÔ˘˜ ·ÛıÂÓ›˜ Ù˘·ÚÔ‡Û·˜ ÌÂϤÙ˘, Ù¤ÛÛÂÚȘ ‰È·Ì·ÚÙ‡ÚÔÓÙ·ÓÁÈ· Û˘ÌÙÒÌ·Ù· ‡ÔÙ· ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘Ì˘ÂÏÔ‡. ∆· Û˘ÌÙÒÌ·Ù· ÂÚÈÂÏ¿Ì‚·Ó·Ó“Á‰Ô‡Ô” ·ÎÔ˘ÛÙfi Î·È ·fi ÙÔ˘˜ ÁÔÓ›˜ Î·È ·fiÙÔ 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 ϿÁÈ· Ô˘‰¤ÙÂÚË ı¤ÛËÎ·È Û ο̄Ë, ∂ÈÎfiÓ· 4), ·˘¯ÂÓ·ÏÁ›· ηٿ ÙËÓÎ¿Ì„Ë Ù˘ ÎÂÊ·Ï‹˜ Î·È ·È̈‰›Â˜ ÙˆÓ ¿Óˆ¿ÎÚˆÓ ¿Ìʈ (ÛÙȘ ·ÛıÂÓ›˜ Ì ·ÚÈıÌfi 13 Î·È 19ÙÔ˘ ¶›Ó·Î· 1, ·ÓÙ›ÛÙÔȯ· - Ì ∞∞∞ 4 mm ÛÂÏ¿ÁÈ· Ô˘‰¤ÙÂÚË ı¤ÛË, ·ÓÙ›ÛÙÔȯ·) ηÈ, Ù¤ÏÔ˜,ÌË ‰È·ÁÓˆṲ̂ӷ ÏÈÔı˘ÌÈο ÂÂÈÛfi‰È· ÛÙÔÓ·ÛıÂÓ‹ Ì ·ÚÈıÌfi 24 ÙÔ˘ ¶›Ó·Î· 2 (Ì ∞∞∞ 4mm Û ϿÁÈ· Ô˘‰¤ÙÂÚË ı¤ÛË). ¢ÈÂÓÂÚÁ‹ıËÎÂCT Î·È MRI Ù˘ ∞ª™™ Î·È ‰ÂÓ Ê¿ÓËΠ›ÂÛËÙÔ˘ ÓˆÙÈ·›Ô˘ ۈϋӷ ÛÂ Î·Ó¤Ó·Ó ·fi ÙÔ˘˜ Ù¤Û-ÛÂÚȘ ·ÛıÂÓ›˜. ∆· ¿ÙÔÌ· ·˘Ù¿ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÂ

¶·È‰È·ÙÚÈ΋ 2005;68:358-364

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

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

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

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

OÌ¿‰· No ŸÓÔÌ· ∏ÏÈΛ· ∞ÙÏ·ÓÙÔ·ÍÔÓÈ΋(¤ÙË) ·fiÛÙ·ÛË (mm) -

ı¤ÛË ·/·

<15 ¤ÙË 1 ∆. ¢. 4 6 Ô˘‰¤ÙÂÚË2 µ. Ã. 4 7 Ô˘‰¤ÙÂÚË3 ∫. ∫. 4 5 Ô˘‰¤ÙÂÚË4 ¡. °. 3 6 Ô˘‰¤ÙÂÚË5 ∆. ™. 1 5 Ô˘‰¤ÙÂÚË6 ¶. ∫. 6 5 ο̄Ë7 º. ¢. 6 5 ο̄Ë8* ª. ª. 12 5 Ô˘‰¤ÙÂÚË9 ¶. °. 10 7 Ô˘‰¤ÙÂÚË

10 µ. ª. 12 5 ο̄Ë11 ™. ™. 11 5 ο̄Ë12 §. Ã. 11 6 Ô˘‰¤ÙÂÚË13 ¡. °. 11 7 Ô˘‰¤ÙÂÚË14 ¶. ¢. 10 5 ο̄Ë15 µ. ™. 7 5 ο̄Ë16 ¶. °. 7 5 Ô˘‰¤ÙÂÚË17 ∞. Ã. 14 5 Ô˘‰¤ÙÂÚË

>15 ¤ÙË 18 ¡. Ã. 40 4 Ô˘‰¤ÙÂÚË19 ∫. ∞. 25 4 Ô˘‰¤ÙÂÚË20 ¶. ∞. 20 7 Ô˘‰¤ÙÂÚË21 ∫. Ã. 17 8 Ô˘‰¤ÙÂÚË22 π. ∞. 17 4 Ô˘‰¤ÙÂÚË23 ∞. ª. 16 4 Ô˘‰¤ÙÂÚË24 °. µ. 22 4 Ô˘‰¤ÙÂÚË

* ∞ÛıÂÓ‹˜ Ù˘ ∂ÈÎfiÓ·˜ 6

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·ıÏԷȉȤ˜ 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 ÊÔ-

Ú¿ ›ÛËÌ· ÙË ı¤ÛË Ù˘ ÁÈ· ÙÔÓ ÚÔÏËÙÈÎfi

¤ÏÂÁ¯Ô Û¯ÂÙÈο Ì ÙËÓ ∞∞∞ ÙˆÓ ÂÊ‹‚ˆÓ Ì DS

(8,20). ∆fiÙÂ, ˘ÔÛÙ‹ÚÈ˙Â 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 ÛÂ

Ï¿ÁÈ· ·/· ÙÔ˘ ·˘¯¤Ó· ÚÈÓ ·fi ÙË Û˘ÌÌÂÙÔ¯‹

ÙÔ˘˜ ÛÙÔ ‰ÈÂıÓ¤˜ ·ÓÙ·ÁˆÓÈÛÙÈÎfi ÚfiÁÚ·ÌÌ·

ÙˆÓ Special Olympics (7). ∆· ¿ÙÔÌ· ÌÂ ·ÎÙÈÓÔ-

ÏÔÁÈο ‰È·ÈÛو̤ÓË ∞∞∞ ·ÔÎÏ›ÔÓÙ·Ó ·fi

οÔȘ ‰Ú·ÛÙËÚÈfiÙËÙ˜, ÔÈ Ôԛ˜ ÌÔÚ› 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΋˜ ηÈ

Fitness Ù˘ AA¶ ·Ó·ÛÎfiËÛ ٷ ÛÙÔȯ›·, ‚¿-

ÛÂÈ ÙˆÓ ÔÔ›ˆÓ ÂÈÎÚ¿ÙËÛ ·˘Ù‹ Ë ı¤ÛË Î·È

·ÌÊÈÛ‚ËÙ‹ıËÎÂ Ë ·Í›· Ù˘ ·/· ˆ˜ screening test

ÁÈ· Èı·Ó‹ οΈÛË ÙÔ˘ ·˘¯¤Ó· ÙˆÓ ·ıÏËÙÒÓ

ÌÂ DS (21).

∆· ÛÙÔȯ›· ÛÙ· ÔÔ›· ÛÙËÚ›¯ıËÎÂ Ë ·ÌÊÈ-

Û‚‹ÙËÛË Î·È Ë ·Ó·ıÂÒÚËÛË ‹Ù·Ó ÔÈ ·Û·Ê›˜

ÁÓÒÛÂȘ Û¯ÂÙÈο Ì ÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ·ÛÙ¿-

ıÂÈ·˜ Î·È ÙÔ˘ Èı·ÓÔ‡ ÂÍ·ÚıÚ‹Ì·ÙÔ˜, ÁÈ· ÙÔ ·Ó

Ë ·ÎÙÈÓÔÏÔÁÈο ÙÂÎÌËÚȈ̤ÓË ·Û˘Ìو̷ÙÈ΋

∞∞∞ ˘ÔÎÚ‡ÙÂÈ Î›Ó‰˘ÓÔ ÁÈ· ÚfiÎÏËÛË Û˘-

Ìو̷ÙÈ΋˜ ·ÛÙ¿ıÂÈ·˜ ‹ Î·È ·ÎfiÌË ÛÔ‚·ÚfiÙÂ-

Ú˘ 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·ÈÚÂ-

ÙÈο Ï›Á· ¿ÙÔÌ· Ì ۇӉÚÔÌÔ Down Î·È Û˘-

Ìو̷ÙÈ΋ ∞∞∞ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙË ‰ÈÂıÓ‹

‚È‚ÏÈÔÁÚ·Ê›·. ŒÓ· ÂÈϤÔÓ ÛÙÔÈ¯Â›Ô Ô˘ ÚÔ-

ηÏ› ·ÎfiÌË ÌÂÁ·Ï‡ÙÂÚË Û‡Á¯˘ÛË Â›Ó·È Ë ÊÙˆ-

¯‹ ·ӷÏË„ÈÌfiÙËÙ· ÙˆÓ Â˘ÚËÌ¿ÙˆÓ Ù˘ ∞∞∞

ÛÙȘ ·ÎÙÈÓÔÁڷʛ˜ (21), ηıÒ˜ Ë ·ÎÙÈÓÔÏÔÁÈ΋

ÂÈÎfiÓ· ·ÏÏ¿˙ÂÈ ·fi ÒÚ· ÛÂ ÒÚ· ·fi ·ıÔÏÔÁÈ-

΋ ÛÂ Ê˘ÛÈÔÏÔÁÈ΋. ∏ ‡·ÚÍË Ù˘ ∞∞∞ ÌÔÚ›

Ó· ÂÍ·Ê·ÓÈÛÙ› Û ÂfiÌÂÓ˜ ·ÎÙÈÓÔÁڷʛ˜ Ô˘

Ï·Ì‚¿ÓÔÓÙ·È ·fi ÙÔÓ ›‰ÈÔ ¯ÂÈÚÈÛÙ‹ ÛÙÔ ›‰ÈÔ ¿ÙÔ-

ÌÔ. ¢Â‰ÔÌ¤ÓˆÓ fiÏˆÓ ·˘ÙÒÓ ÙˆÓ ÚÔ‚ÏËÌ·ÙÈ-

ÛÌÒÓ, Ë ∞∞¶ ·Ó·Î¿ÏÂÛ ÙËÓ ·Ú¯È΋ Ù˘ Ô‰ËÁ›·

ÁÈ· ÙÔÓ ÚÔÏËÙÈÎfi ¤ÏÂÁ¯Ô fiÏˆÓ ÙˆÓ ·ÙfiÌˆÓ ÌÂ

DS Ì ·ÎÙÈÓÔÁڷʛ˜ Ù˘ ∞ª™™ ÚÈÓ ÙË Û˘ÌÌÂ-

ÙÔ¯‹ ÙÔ˘˜ ÛÙ· ·ıÏ‹Ì·Ù·, ÙËÓ ÔÔ›· 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 ÏÂÙÔÌÂÚ‹ ÎÏÈÓÈ΋ ÂͤٷÛË

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

362 Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘ Î·È Û˘Ó.

¶·È‰È·ÙÚÈ΋ 2005;68:358-364

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·362

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

Ó· ·ÔÎÏÂ›Ô˘Ó ‹ Ó· ÚÔÛÂÁÁ›˙Ô˘Ó ÛÙË Ó¢ÚÔÏÔ-ÁÈ΋ ÛËÌÂÈÔÏÔÁ›· Ù˘ ›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘Â-ÏÔ‡. ∂ÈϤÔÓ, 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˘ÌÙÒÌ·Ù· ›ÂÛ˘ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡, fiˆ˜ ÙÔ ·ÓÒÌ·ÏÔ ‚¿‰È-ÛÌ·, Ë ·˘ÍË̤ÓË ·‰ÂÍÈfiÙËÙ· Î·È ÔÈ ‰È·Ù·Ú·¯¤˜ÙˆÓ ÛÊÈÁÎÙ‹ÚˆÓ (21).

∆· ·Û˘Ìو̷ÙÈο ¿ÙÔÌ· Ì DS ÛÙ· ÔÔ›·‰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›·‹ Âȉ›ӈÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ı· Ú¤ÂÈ Ó·˘Ô‚¿ÏÏÔÓÙ·È Û CT ‹ MRI ∞ª™™, ÚÔÎÂÈ̤-ÓÔ˘ Ó· ‰È¢ÎÚÈÓÈÛÙ› Ë ¤ÎÙ·ÛË Ù˘ ›ÂÛ˘ ÙÔ˘ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ∂¿Ó ·Ô‰Âȯı› ·ÂÈÎÔÓÈÛÙÈ-ο ›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡, Ú¤ÂÈ Ó· ·-Ú·ÂÌÊıÔ‡Ó Û Ó¢ÚÔ¯ÂÈÚÔ˘ÚÁfi ‹ ÔÚıԷȉÈ-Îfi Ô˘ ı· ÛÙ·ıÂÚÔÔÈ‹ÛÂÈ ¯ÂÈÚÔ˘ÚÁÈο ÙÔ ¿ÓˆÙÌ‹Ì· Ù˘ ™™, ÒÛÙ ӷ ·ÔÊ¢¯ı› Ô ÙÚ·˘Ì·-ÙÈÛÌfi˜ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡ (2,3,8,23).

øÛÙfiÛÔ, Ë ∂ÈÙÚÔ‹ ÙˆÓ Special OlympicsÂÍ·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 ·Ùfi-ÌˆÓ Ì DS ÚÈÓ ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘˜ ÛÙ· ·ıÏ‹-Ì·Ù·. ∂¿Ó ‰È·ÈÛÙˆı› ∞∞∞ ‰ÂÓ ÂÈÙÚ¤ÂÈ Ó·Ï¿‚Ô˘Ó Ì¤ÚÔ˜ Û ‰Ú·ÛÙËÚÈfiÙËÙ˜ Ô˘ ÚÔη-ÏÔ‡Ó ˘ÂÚ¤ÎÙ·ÛË ÙÔ˘ ·˘¯¤Ó·, fiˆ˜ ηٿ‰˘ÛË,ÎÔχ̂ËÛË-ÂÙ·ÏÔ‡‰·, ηٿ‰˘ÛË ÛÙËÓ ¤Ó·Ú-ÍË 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 ›ÂÛËÙˆÓ Ì˘ÒÓ Ù˘ ÎÂÊ·Ï‹˜ Î·È ÙÔ˘ ·˘¯¤Ó·. ¢‡Ô¤Ê˂˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, Ô˘ Û˘ÌÌÂÙ›-¯·Ó ÛÙÔ˘˜ Special Olympics ÙÔ˘ 2003, ÌÂÙ·Í‡ÙˆÓ ¿ÏÏˆÓ ÂÓÙ‡ˆÓ ÁÈ· Û˘ÌÏ‹ÚˆÛË ÁÈ· ÙÔÓÈ·ÙÚÈÎfi ÙÔ˘˜ ¤ÏÂÁ¯Ô, ÚÔÛÎfiÌÈÛ·Ó Î·È ÊfiÚÌ·

ÚÔ˜ Û˘ÌÏ‹ÚˆÛË Î·È ˘ÔÁÚ·Ê‹ ·fi ÙÔÓ È·-ÙÚfi, ÙÔ ›‰ÈÔ ÙÔ ¿ÙÔÌÔ Ì DS Î·È ÙÔÓ ÁÔÓ¤· Û¯Â-ÙÈο Ì ÙËÓ ∞∞∞ (∂ÈÎfiÓ˜ 7·, ‚ Î·È 8).

∆· ÂÚÈÛÛfiÙÂÚ· ¿ÙÔÌ· ·˘Ù‹˜ Ù˘ ¤Ú¢ӷ˜Ì ∞∞∞ Â›Ó·È ·Û˘Ìو̷ÙÈο. ¢fiıËÎ·Ó Ô‰Ë-Á›Â˜ ÛÙÔ˘˜ ÁÔÓ›˜ Ó· ÚÔˆıÔ‡Ó Ù· ·È‰È¿ ÙÔ˘˜ÛÙÔÓ ·ıÏËÙÈÛÌfi, Û ·ıÏ‹Ì·Ù· Ô˘ ‰ÂÓ ÂÚÈ-Ï·Ì‚¿ÓÔ˘Ó ˘ÂÚ¤ÎÙ·ÛË ÙÔ˘ ·˘¯¤Ó·, fiˆ˜ÚԷӷʤÚıËÎÂ, ηıÒ˜ ›Û˘ Î·È ·ÔÊ˘Á‹ı¤ÛÂˆÓ ˘ÂÚ¤ÎÙ·Û˘ Ù˘ ÎÂÊ·Ï‹˜ Û ηıËÌÂ-ÚÈÓ¤˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ fiˆ˜ 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·Áfi-Ó˜ ÛÙ· Ì¿ÙÈ·, Ó· ˘¿Ú¯ÂÈ ÛÙÔ Î¿ıÈÛÌ· ÙÔ˘ ·˘-ÙÔÎÈÓ‹ÙÔ˘ ÙÔ˘˜ ÚÔÛٷ٢ÙÈÎfi ÚÔÛΤʷÏÔÁÈ· Ó· ÚÔÛٷهÔÓÙ·È ·fi ·fiÙÔÌÔ ÊÚÂÓ¿ÚÈ-ÛÌ· Î.Ï.

∆· Â˘Ú‹Ì·Ù· Ù˘ ¤Ú¢ӷ˜ ÂÓÈÛ¯‡Ô˘Ó ÙȘԉËÁ›Â˜ Ù˘ ∞ıÏËÙÈ·ÙÚÈ΋˜ ∂ÈÙÚÔ‹˜ Ù˘∞∞¶, ‰ËÏ·‰‹ ÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ·È‰ÈÒÓ Ì DSËÏÈΛ·˜ ¿Óˆ ÙˆÓ 3 ÂÙÒÓ Ì ·/· Ù˘ ∞ª™™ ηٿÙËÓ 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 ÎÏÈÓÈ-Îfi˜ ÁÈ·ÙÚfi˜, Ì ÙÔ ÏÂÙÔÌÂÚ¤˜ ÈÛÙÔÚÈÎfi Î·È ÙËÓÚÔÛÂÎÙÈ΋ ÎÏÈÓÈ΋ ÂͤٷÛË, Ú¤ÂÈ Ó· ·Ó·˙Ë-Ù¿ Û˘ÌÙÒÌ·Ù· Î·È Ó¢ÚÔÏÔÁÈο ÛËÌ›· Ù˘›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡ (‡ÎÔÏË ÎfiˆÛË,‰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‡). ∏ ÛËÌÂÈÔ-ÏÔÁ›· Ô˘ ·ÔηχÙÂÙ·È ·fi ÙËÓ ÎÏÈÓÈ΋ Âͤ-Ù·ÛË ·ÔÙÂÏ› ÙÔ ÎÚÈÙ‹ÚÈÔ ÁÈ· ÙËÓ Â·Ó¿ÏË„ËÙ˘ ·/· Ù˘ ∞ª™™ Î·È ÙÔ˘ ÂÚ·ÈÙ¤Úˆ ÂϤÁ¯Ô˘Ì CT ‹ MRI Ù˘ ∞ª™™.

∂˘¯·ÚÈÛٛ˜

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

µÈ‚ÏÈÔÁÚ·Ê›·

1. Cremers MJ, Bol E, de Roos F, van Gijn J. Risk ofsports activities in children with Down’s syndrome

¶·È‰È·ÙÚÈ΋ 2005;68:358-364

∂ÈÎfiÓ· 8. ŒÊË‚Ë Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ηٿ ÙË ‚Ú¿-‚Â˘Û‹ Ù˘ ÛÙÔ˘˜ Special Olympics ÛÙËÓ πÚÏ·Ó‰›· ÙÔ2003.

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·363

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and atlantoaxial instability. Lancet 1993;342:511-514. 2. Pueschel SM, Scola FH. Atlantoaxial instability in in-

dividuals with Down syndrome: epidemiologic, ra-diographic, and clinical studies. Pediatrics 1987;80:555-560.

3. American Academy of Pediatrics. Committee onSports Medicine and Fitness. Atlantoaxial instabilityin Down syndrome: subject review. Pediatrics1995;96:151-154.

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

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

6. Selby KA, Newton RW, Gupta S, Hunt L. Clinicalpredictors and radiological reliability in atlantoaxialsubluxation in Down’s syndrome. Arch Dis Child1991;66:876-878.

7. Davidson RG. Atlantoaxial instability in individualswith Down syndrome: a fresh look at the evidence.Pediatrics 1988;81:857-865.

8. American Academy of Pediatrics. Committee onSports Medicine. Atlantoaxial instability in Downsyndrome. Pediatrics 1984;74:152-154.

9. Cremers MJ, Ramos L, Bol E, van Gijn J. Radiologi-cal assessment of the atlantoaxial distance in Down’ssyndrome. Arch Dis Child 1993;69:347-350.

10. Roche CJ, O’Malley M, Dorgan JC, Carty HM. A pic-torial review of atlanto-axial rotatory fixation: keypoints for the radiologist. Clin Radiol 2001;56:947-958.

11. Kauppi M, Leppanen L, Heikkila S, Lahtinen T, Kau-tiainen H. Active conservative treatment of at-lantoaxial subluxation in rheumatoid arthritis. Br JRheumatol 1998;37:417-420.

12. Herzka A, Sponseller PD, Pyeritz RE. Atlantoaxial ro-tatory subluxation in patients with Marfan syndrome.A report of three cases. Spine 2000;25:524-526.

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

14. Papadopoulos SM, Dickman CA, Sonntag VK. At-lantoaxial stabilization in rheumatoid arthritis. JNeurosurg 1991;74:1-7.

15. Roche CJ, Eyes BE, Whitehouse GH. The rheuma-toid cervical spine: signs of instability on plain cervi-cal 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 le-sions in rheumatoid arthritis. J Spinal Disord 2000;13:519-526.

17. Neva MH, Isomaki P, Hannonen P, Kauppi M, Kr-ishnan E, Sokka T. Early and extensive erosiveness inperipheral joints predicts atlantoaxial subluxationsin patients with rheumatoid arthritis. ArthritisRheum 2003;48:1808-1813.

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

19. Riise T, Jacobsen BK, Gran JT. High mortality in pa-tients with rheumatoid arthritis and atlantoaxialsubluxation. J Rheumatol 2001;28:2425-2429.

20. Pueschel SM, Sustrova M. Adolescents with Downsyndrome: Toward a more fulfilling life. 2nd ed. Bal-timore: Paul H. Brookes Publishing Co; 2002.

21. Braganza SF. Atlantoaxial dislocation. Pediatr Rev2003;24:106-107.

22. Saenz RB. Primary care of infants and young chil-dren with Down syndrome. Am Fam Physician1999;59:381-390, 392, 395-396.

23. American Academy of Pediatrics. Committee on Ge-netics. Health supervision for children with Downsyndrome. Pediatrics 2001;107:442-449.

364 Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘ Î·È Û˘Ó.

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365ORIGINAL ARTICLE

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 investigateAAI 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 patientswere examined clinically in relation to the presence or absence of discomfort in the neck area andspinal cord compression. Eighty one per cent of those aged 3 years and older were screenedradiographically at the cervical spine. A lateral neck X-ray was made in the neutral position, and inflexion when necessary. An atlantoaxial distance of greater than 4 mm for children under 15 years ofage and greater than 3 mm for people over 15 years is suggested as the cut off point for AAI. All theX-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 morefrequent 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 cordcompression.

Conclusions: The results of this study support the guidelines of Committee on Sports of AmericanAcademy of Pediatrics (AAP) which recommend screening of children over 3 years old with DS bycervical spine radiography at the initial visit to the paediatrician, and provision of guidance to thefamilies about the participation of the children in sports (i.e., avoidance of extreme flexion oroverstretching of the neck) and cervical X-ray screening before they start participation in sportsactivities. 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 (easyfatiguability, difficulties in walking, abnormal gait, neck pain, limited neck mobility, torticollis orhead tilt, incoordination and clumsiness, sensory deficits, spasticity, hyperreflexia, clonus, extensor-plantar reflex, other upper motor neuron signs and changes in bowel or bladder control). Anyfindings on the physical examination should be investigated by repetition of cervical spineradiography, and further screening by MRI.

Key words

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

1 1st Paediatric Clinic ofAristotle University,“Ippokration” GeneralHospital, Thessaloniki

2 Paediatric Department ofRadiology, “Ippokration”General Hospital,Thessaloniki

Correspondence:

H. Hatzissevastou-LoukidouLaboratory 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

Paediatriki 2005;68:365

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366 ∂¡¢π∞º∂ƒOÀ™∞ ¶∂ƒπ¶∆ø™∏

1 ∞’ ¶·ÓÂÈÛÙËÌȷ΋¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó·

2 ∫ÏÈÓÈ΋ ¶Ï·ÛÙÈ΋˜ÃÂÈÚÔ˘ÚÁÈ΋˜, ¡ÔÛÔÎÔÌ›Զ·›‰ˆÓ ”∞Á›· ™ÔÊ›·”,∞ı‹Ó·

3 ∂ÚÁ·ÛÙ‹ÚÈÔ ¶·ıÔÏÔÁÈ΋˜∞Ó·ÙÔÌÈ΋˜, ¡ÔÛÔÎÔÌ›Զ·›‰ˆÓ “∞Á›· ™ÔÊ›·”,∞ı‹Ó·

4 Institute of Cancer Research, Surrey, UK

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O˘Ú·Ó›· ¶··Ó‰Ú¤Ô˘ ∞’ ¶·ÓÂÈÛÙËÌȷ΋¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” £Ë‚ÒÓ & §Â‚·‰Â›·˜, ∆.∫. 115 27, ∞ı‹Ó· E-mail: [email protected]

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

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O. ¶··Ó‰Ú¤Ô˘1, ∞. ∫ÏËÌÂÓÙÔÔ‡ÏÔ˘1, ¡. ¶ÔÓËÚfi˜2, ∞. ∫ˆÓÛÙ·ÓÙÈÓ›‰Ô˘3, ∞. •˘ÔÏ˘Ù¿ - ∑·¯·ÚÈ¿‰Ë1,

N. Rachman4, °. ÃÚÔ‡ÛÔ˜1

¶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 ˘ÔÏÂÈfiÌÂÓÔ ¯·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 ‰ÂÚ-Ì·ÙÈΤ˜ ‚Ï¿‚˜ ‰È·ÊfiÚÔ˘ ÌÂÁ¤ıÔ˘˜, ˘ÂÚÙÚÔÊ›· ÙˆÓ Ô‡ÏˆÓ, Û˘Áο„ÂȘ ÙˆÓ ·ÚıÚÒÛÂˆÓ Î·È ÔÛÙÂ-ÔÂÓ›·. ¢ÂÓ ˘¿Ú¯ÂÈ Û˘ÌÌÂÙÔ¯‹ ÙˆÓ ÛÏ·Á¯ÓÈÎÒÓ ÔÚÁ¿ÓˆÓ Î·È Ë ÓÔËÙÈ΋ ÂͤÏÈÍË Â›Ó·È Ê˘ÛÈÔÏÔ-ÁÈ΋. ¢ÂÓ ˘¿Ú¯Ô˘Ó ¯·Ú·ÎÙËÚÈÛÙÈο ‚ÈÔ¯ËÌÈο Â˘Ú‹Ì·Ù· Î·È Ë ÎÏÈÓÈ΋ ‰È¿ÁÓˆÛË ÂȂ‚·ÈÒÓÂÙ·ÈÌ ÙËÓ ÈÛÙÔÏÔÁÈ΋ ÂͤٷÛË ÙˆÓ ‚Ï·‚ÒÓ. ¢ÂÓ ˘¿Ú¯ÂÈ ÂȉÈ΋ ıÂڷ›·, ÂÓÒ Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÍ·›ÚÂ-ÛË ÔÏÏÒÓ ÔÁÎȉ›ˆÓ Á›ÓÂÙ·È ÁÈ· ÏÂÈÙÔ˘ÚÁÈÎÔ‡˜ Î·È ·ÈÛıËÙÈÎÔ‡˜ ÏfiÁÔ˘˜. ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›Ùˆ-ÛË ÎÔÚÈÙÛÈÔ‡ ËÏÈΛ·˜ 4 ÂÙÒÓ, ÌÈÎÚfiÛˆÌÔ˘, ÌÂ Û˘Áο„ÂȘ ÙˆÓ ÌÂÁ¿ÏˆÓ Î·È ÌÈÎÚÒÓ ·ÚıÚÒÛˆÓ, ÂÎ-ÛÂÛËÌ·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¢Îfi-Ï˘Ó ÙË Ì¿ÛËÛË ÛÙË ÌÈÎÚ‹ ·ÛıÂÓ‹.

§¤ÍÂȘ ÎÏÂȉȿ

O˙›‰È·, ‚Ï·Ù›‰Â˜, Èӈ̿وÛË.

∂ÈÛ·ÁˆÁ‹

∏ Ó·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË Â›Ó·È ÎÏË-ÚÔÓÔÌÈÎfi ÓfiÛËÌ· Ô˘ ÌÂÙ·‚È‚¿˙ÂÙ·È Ì ÙÔÓ˘ÔÏÂÈfiÌÂÓÔ ÛˆÌ·ÙÈÎfi ¯·Ú·ÎÙ‹Ú· Î·È ÙÔ ÁÔ-Ó›‰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, ÌÂÙ¿ÛË ·‡ÍËÛ˘ Û ·ÚÈıÌfi Î·È Ì¤ÁÂıÔ˜ Ì ÙËÓ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘. ™˘¯Ó¿ ··ÈÙÂ›Ù·È ¯ÂÈÚÔ˘Ú-ÁÈ΋ ·Ú¤Ì‚·ÛË ÁÈ· ÏÂÈÙÔ˘ÚÁÈÎÔ‡˜ Î·È ·ÈÛıË-ÙÈÎÔ‡˜ ÏfiÁÔ˘˜.

∏ ˘ÂÚÏ·Û›· ÙˆÓ Ô‡ÏˆÓ Ù˘Èο ·Ó·Ù‡Û-ÛÂÙ·È Û˘Á¯ÚfiÓˆ˜ Ì ÙȘ ‰ÂÚÌ·ÙÈΤ˜ ·ÏÏÔÈÒÛÂÈ˜Î·È ÂÓ›ÔÙ ··ÈÙÂ›Ù·È ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛËÂÚÈÔ‰ÔÓÙÈÎÔ‡ ÈÛÙÔ‡ ÁÈ· ‰È¢ÎfiÏ˘ÓÛË Ù˘ Û›ÙÈ-Û˘ Î·È Ù˘ ÔÌÈÏ›·˜. OÈ Û˘Áο̄ÂȘ ÙˆÓ ·Ú-ıÚÒÛÂˆÓ ÂȉÂÈÓÒÓÔÓÙ·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·ӷËÚÈÎfi ·Ì·Í›‰ÈÔ.

∏ ÔÛÙÂÔÂÓ›· Â›Ó·È Û˘¯Ó‹. ∏ ÓÔËÙÈ΋ ηÈ

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

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘

∫ÔÚ›ÙÛÈ ËÏÈΛ·˜ 4 ÂÙÒÓ ·Ú·¤ÌÂÙ·È ÁÈ·Ó¢ÚÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË ÏfiÁˆ ·‰˘Ó·Ì›·˜ ÛÙ‹ÚÈ-͢ Î·È ‚¿‰ÈÛ˘.

∂›Ó·È ÙÔ ÚÒÙÔ ·È‰› Ê·ÈÓÔÙ˘Èο ˘ÁÈÒÓ, ÌËÛ˘ÁÁÂÓÒÓ ÁÔÓ¤ˆÓ, ÂÏÏËÓÈ΋˜ ÂıÓÈÎfiÙËÙ·˜, Ô˘Î·Ù¿ÁÔÓÙ·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·ÈÙÚÂȘ ÚÔËÁËı›Û˜ ·˘ÙfiÌ·Ù˜ ·Ô‚ÔϤ˜ Ù˘

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367¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË

ÌËÙ¤Ú·˜. ∞fi ÙË Á¤ÓÓËÛË ‰È·ÈÛÙÒıËΠۇ-

ÁÎ·Ì„Ë ÙˆÓ ÁÔÓ¿ÙˆÓ Î·È ÙˆÓ ·ÁÎÒÓˆÓ ÔfiÙÂ

Î·È ÂÙ¤ıË Ë ‰È¿ÁÓˆÛË Ù˘ ·ÚıÚÔÁÚ‡ˆÛ˘. ∆Ô

·È‰› ˘Ô‚Ï‹ıËΠÛÂ Ê˘ÛÈÎÔıÂڷ¢ÙÈ΋ ·Áˆ-

Á‹ ¯ˆÚ›˜ ‚ÂÏÙ›ˆÛË. ™Â ËÏÈΛ· 12 ÌËÓÒÓ ¤ÁÈÓÂ

ÔÚıԷȉÈ΋ ·Ú¤Ì‚·ÛË Î·È ÛÙ· ‰‡Ô ÁfiÓ·Ù· ÌÂ

ı˘Ï·ÎÔÙÔÌ‹ Î·È ÙÂÓÔÓÙÔÙÔÌ‹ ÙˆÓ Ô›ÛıÈˆÓ ÌË-

ÚÈ·›ˆÓ ¯ˆÚ›˜ ‚ÂÏÙ›ˆÛË. ªÂÙ¿ ·fi 6 Ì‹Ó˜ ÂÈ-

¯ÂÈÚ‹ıËΠÂÎ Ó¤Ô˘ ¯ÂÈÚÔ˘ÚÁÈ΋ ‰ÈfiÚıˆÛË ÙˆÓ

Û˘Áο̄ˆÓ, ›Û˘ ¯ˆÚ›˜ ·ÔÙ¤ÏÂÛÌ·.

∫·Ù¿ ÙËÓ ÎÏÈÓÈ΋ ÂÎÙ›ÌËÛË, Ë ÌÈÎÚ‹ ·ÛıÂ-

Ó‹˜ ·ÚÔ˘Û›·˙Â Ê˘ÛÈÔÏÔÁÈ΋, ÓÔËÙÈ΋ ηÈ

ÁψÛÛÈ΋ ·Ó¿Ù˘ÍË ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· Ù˘

(Peabody test), ·ÏÏ¿ ÎÈÓËÙÈ΋ ·Ó·ËÚ›· ÌÂ

·‰˘Ó·Ì›· ÛÙ‹ÚÈ͢ Î·È ‚¿‰ÈÛ˘. O ÎÔÚÌfi˜

‹Ù·Ó ¿Î·ÌÙÔ˜, Ë Î›ÓËÛË Ù˘ ÎÂÊ·Ï‹˜ ÂÚÈÔ-

ÚÈṲ̂ÓË (∂ÈÎfiÓ· 1), ÙÔ ÚÔÛˆÂ›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-

ÎfiÓ· 2). ∞Ó¢ڛÛÎÔÓÙ·Ó ÔÈ ÓÂÔÁÈÏÔ› Ô‰fiÓÙ˜.¶·Ú·ÙËÚ‹ıËΠÂÎÛÂÛËÌ·Ṳ̂ÓË Û‡Áη̄ËÙˆÓ ÁÔÓ¿ÙˆÓ Û ÔÚı‹ ÁˆÓ›·, ÂÓÒ fiϘ ÔÈ ·Ú-ıÚÒÛÂȘ ‹Ù·Ó ‰‡ÛηÌÙ˜ Î·È ÂÒ‰˘Ó˜ ÛÙËÓ·ıËÙÈ΋ ΛÓËÛË (∂ÈÎfiÓ· 1). OÈ ·ÚıÚÒÛÂȘ ÙˆÓ¿Óˆ ¿ÎÚˆÓ ‚Ú›ÛÎÔÓÙ·Ó, ›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‰ÒÓÎ·È ÛÙËÓ ÂÚÈÚˆÎÙÈ΋ ¯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 ÙËÓËÏÈΛ· ÙˆÓ 3 ÂÙÒÓ, Ì ٿÛË ·‡ÍËÛ˘ ÛÙÔÓ ·ÚÈı-Ìfi Î·È ÛÙÔ Ì¤ÁÂıÔ˜. ∆Ô ‰¤ÚÌ· ›¯Â ¯¿ÛÂÈ ÙËÛ·ÚÁ‹ ÙÔ˘. ◊Ù·Ó ·ÓÙÈÎÂÈÌÂÓÈο ‰‡ÛÎÔÏÔ Ó·ÂÏÂÁ¯ı› Ë Ì˘˚΋ ÈÛ¯‡˜, fiˆ˜ Î·È Ù· ÙÂÓfiÓÙÈ··ÓÙ·Ó·ÎÏ·ÛÙÈο ÙÔ˘ ·È‰ÈÔ‡.

∞fi ÙÔÓ Ï‹ÚË ·ÈÌ·ÙÔÏÔÁÈÎfi, ‚ÈÔ¯ËÌÈÎfiÎ·È ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ ·È‰ÈÔ‡ ‰ÂÓ ÚÔ¤-΢„·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·¯‡ÙË-Ù· ·ÁˆÁ‹˜ Ó‡ڈÓ. O ηڢfiÙ˘Ô˜ ‹Ù·Ó Ê˘ÛÈÔ-ÏÔÁÈÎfi˜ ı‹ÏÂÔ˜ (46ÃÃ).

¶·È‰È·ÙÚÈ΋ 2005;68:366-370

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

∂ÈÎfiÓ· 2. ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË: Ê·›ÓÂÙ·È ÙÔ·Ó¤ÎÊÚ·ÛÙÔ ÚÔۈ›Ô, Ë ÌÂÁ¿ÏË ˘ÂÚÙÚÔÊ›· ÙˆÓ Ô‡-ψÓ, ÔÈ ÂÚÈÛÙÔÌ·ÙÈΤ˜ ‚Ï·Ù›‰Â˜ Î·È ÙÔ Ô˙›‰ÈÔ ÛÙÔ·ÎÚÔÚÚ›ÓÈÔ.

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368 O. ¶··Ó‰Ú¤Ô˘ Î·È Û˘Ó.

™ÙÔÓ ·ÎÙÈÓÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÙˆÓ ÔÛÙÒÓ ‰È·-

ÈÛÙÒıËΠÂÎÛÂÛËÌ·Ṳ̂ÓË ÔÛÙÂÔÂÓ›· (∂ÈÎfi-

Ó· 4). ™ÙËÓ ËÏÂÎÙÚÔÊfiÚËÛË ÙˆÓ ‚ÏÂÓÓÔÔÏ˘-

۷ί·ÚÈÙÒÓ Ô‡ÚˆÓ ‚Ú¤ıËΠÔÚȷ΋ ·‡ÍËÛË

Ù˘ ·¤ÎÎÚÈÛ˘ Ù˘ ıÂÈÈ΋˜ ¯ÔÓ‰ÚÔ˚Ù›Ó˘. ™ÙË

‚ÈÔ„›· ÙˆÓ Ô˙ȉ›ˆÓ ‚Ú¤ıËΠÂÓ·fiıÂÛË ÎÔÏ-

Ï·ÁÔÓÔÔÈË̤ÓÔ˘ ÈÓÒ‰Ô˘˜ ¿ÌÔÚÊÔ˘ ÔÍÂfiÊÈ-

ÏÔ˘ ÂÍˆÎ˘ÙÙ¿ÚÈÔ˘ ˘·ÏÈÓÈÎÔ‡ ÈÛÙÔ‡ Ì ÂÚÈÔ-

¯¤˜ ·˘ÍË̤Ó˘ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜. ¢ÂÓ ·Ú·ÙË-

Ú‹ıËΠÌÈÙˆÙÈ΋ ‹ ¿ÏÏË ·ÓÒÌ·ÏË ‰Ú·ÛÙËÚÈfi-

ÙËÙ· ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ˘Ú‹ÓˆÓ. OÈ ‚Ï¿‚˜

(Ô˙›‰È·) ‹Ù·Ó ˘Ô‰fiÚȘ Ì ¤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¤-

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

™˘˙‹ÙËÛË

∞˘Ùfi ÙÔ Û‡Ó‰ÚÔÌÔ ÂÚÈÁÚ¿ÊËΠÚÒÙË ÊÔ-Ú¿ ·fi ÙÔÓ Murran, ÙÔ 1873, ˆ˜ molluscumfibrosum. ∏ 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, η-ıÈÂÚÒıËÎÂ Ô fiÚÔ˜ Ù˘ Ó·ÓÈ΋˜ ˘·ÏÈÓÈ΋˜ ÈÓˆ-Ì¿ÙˆÛ˘ (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¤˜ ÂÚÈÙÒÛÂȘ 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¤˜ ÛÙˉÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· (5). ∞˘Ù‹ Â›Ó·È Ë ÚÒÙËÂÚÈÁÚ·Ê‹ Ì ÁÔÓȉȷ΋ ÂȂ‚·›ˆÛË ÛÙËÓ∂ÏÏ¿‰·. ∆· ÚÒÙ· Û˘ÌÙÒÌ·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· Â-ÚÈÏ·Ì‚¿ÓÔ˘Ó ‚Ï·Ùȉ҉ÂȘ Î·È Ô˙Ò‰ÂȘ ‰ÂÚÌ·-ÙÈΤ˜ ‚Ï¿‚˜, ΢ڛˆ˜ ÛÙËÓ ÎÂÊ·Ï‹ Î·È Ù·

¶·È‰È·ÙÚÈ΋ 2005;68:366-370

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

∂ÈÎfiÓ· 4. ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË: ÛÙÔÓ ·ÎÙÈÓÔ-ÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÙˆÓ ÔÛÙÒÓ ·Ó¢ڛÛÎÂÙ·È ÂÎÛÂÛËÌ·Ṳ̂-ÓË ÔÛÙÂÔÂÓ›·.

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369¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË

¿ÎÚ·, ÛÙ· ‰¿ÎÙ˘Ï· Î·È ÛÙËÓ ÂÚÈÓÂ˚΋ ÂÚÈÔ-¯‹, ˘ÂÚÙÚÔÊ›· ÙˆÓ Ô‡ÏˆÓ, Û˘Áο̄ÂȘ, ·Ú¯È-ο ΢ڛˆ˜ ÙˆÓ ÌÂÁ¿ÏˆÓ ·ÚıÚÒÛÂˆÓ Î·È ÂÎÛÂ-ÛËÌ·Ṳ̂ÓË ÔÛÙÂÔÂÓ›·. ∞‡ÍËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜Î·È ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Ô˙ȉ›ˆÓ Ì ÙËÓ ¿ÚÔ‰ÔÙ˘ ËÏÈΛ·˜ Ô‰ËÁ› Û˘¯Ó¿ Û ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÍ·›-ÚÂÛË ÁÈ· ·ÈÛıËÙÈÎÔ‡˜ Î·È ÏÂÈÙÔ˘ÚÁÈÎÔ‡˜ Ïfi-ÁÔ˘˜. ∏ ÚÔԉ¢ÙÈ΋ ‰˘Ûη̄›· Ô‰ËÁ› Û·‰˘Ó·Ì›· ‚¿‰ÈÛ˘-ÛÙ‹ÚÈ͢ Î·È Î·ı‹ÏˆÛË Û·ӷËÚÈÎfi ·Ì·Í›‰ÈÔ. ∏ ·ÈÙÈÔÏÔÁ›· ÙˆÓ Û˘ÁοÌ-„ÂˆÓ ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈÛÙ›. ∞Ô‰›‰Â-Ù·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 ÛÏ·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› ÛÂοÔÈÔ˘˜ ·ÛıÂÓ›˜ ¤¯Ô˘Ó ‰È·ÈÛÙˆı› ·ıÔÏÔ-ÁÈο 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˘ÛÈÔ-ÏÔÁÈÎfi 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 ‚ÈÔ-Û‡ÓıÂÛË ÙˆÓ ÁÏ˘ÎÔ˙·ÌÈÓÔÁÏ˘Î·ÓÒÓ (8). ∞Îfi-ÌË, ÂÈÛËÌ·›ÓÔÓÙ·È ·ÓˆÌ·Ï›Â˜ ÛÙË ‚ÈÔÛ‡ÓıÂÛËÙ˘ ıÂÈÈ΋˜ ¯ÔÓ‰ÚÔ˚Ù›Ó˘ ηÈ/‹ ÙÔ˘ ˘·ÏÔ˘ÚÔÓÈ-ÎÔ‡ ÔͤԘ, ÔÈ Ôԛ˜ ‰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 ÔÊ˘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·Ï-

Ï¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ Ô˘ Έ‰ÈÎÔÔÈ› ÙË Û˘ÁÎÂ-

ÎÚÈ̤ÓË ÚˆÙ½ÓË Î·È Â‰Ú¿˙ÂÙ·È ÛÙÔ 4q21 ¯Úˆ-

Ìfiۈ̷, Ì ÎÏÈÓÈ΋ ÂΉ‹ÏˆÛË Ó·ÓÈ΋˜ ˘·ÏÈ-

ÓÈ΋˜ 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 ∂˘ÚÒË) (12).

∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ú¤ÂÈ Ó· Á›ÓÂÙ·È

·fi: ·) Û˘ÁÁÂÓ‹ ·ÚıÚÔÁÚ‡ˆÛË (‰ÂÓ ¤¯ÂÈ ‰ÂÚ-

Ì·ÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ), ‚) Û‡Ó‰ÚÔÌÔ Winchester

(ÌÈÎÚÔۈ̛·, Û˘Áο̄ÂȘ ·fi ÙË Á¤ÓÓËÛË, ¯ˆ-

Ú›˜ ‰ÂÚÌ·ÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ, ÂÓÒ ˘Ê›ÛÙ·Ù·È ıfi-

ψÛË ÙÔ˘ ÎÂÚ·ÙÔÂȉԇ˜), Á) ÓfiÛÔ Fabry (ÛÊÈÁ-

ÁÔÏÈ›‰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 ÛÏ·Á¯ÓÈ-

ÎÒÓ 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΋ ıÂڷ›·. ™˘ÓÙË-

ÚËÙÈο ¤¯Ô˘Ó ‰ÔÎÈÌ·ÛÙ› Ú·‰ÈÔıÂڷ›·, ¯Ë-

ÌÂÈÔıÂڷ›·, ÚˆÙÂÔÏ˘ÙÈο ¤Ó˙˘Ì· Î·È ÙÔÈ-

Τ˜ ÂÁ¯‡ÛÂȘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ, ¯ˆÚ›˜ ·ÔÙ¤-

ÏÂÛÌ·. OÈ ¯ÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ ‰›ÓÔ˘Ó

ÚÔÛˆÚÈÓ¤˜ χÛÂȘ Û ·ÈÛıËÙÈο Î·È ÏÂÈÙÔ˘ÚÁÈ-

ο ÚÔ‚Ï‹Ì·Ù·. ∏ ÚfiÁÓˆÛË Â›Ó·È È‰È·ÈÙ¤Úˆ˜

ÂÈÊ˘Ï·ÎÙÈ΋ ¤ˆ˜ ÂÈ‚·Ú˘Ì¤ÓË.

™ÙËÓ ·ÛıÂÓ‹ Ù˘ ·ÚÔ‡Û·˜ ÂÚ›ÙˆÛ˘ Ë

¯ÔÚ‹ÁËÛË Botulin toxinum A ÛÙËÓ ÎÚÔÙ·ÊÔ-

ÁÓ·ıÈ΋ ÂÚÈÔ¯‹ Î·È ÂÚÈÛÙÔÌ·ÙÈο ‚ÂÏÙ›ˆÛÂ

·ÈÛıËÙ¿ ÙË ‰È¿ÓÔÈÍË ÙÔ˘ ÛÙfiÌ·ÙÔ˜ Î·È ‰È¢Îfi-

Ï˘Ó ÙË Ì¿ÛËÛË. ∆Ô Û‡ÌÏÔÎÔ Ù˘ Ó¢ÚÔÙÔÍ›-

Ó˘ Clostridium botulinum Ù‡Ô˘ ∞, ˆ˜ ÁÓˆ-

ÛÙfiÓ ·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· ÙÔÈ΋,

·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·Ùfi-

ÙËÙ· ÚÔÁÂÓÓËÙÈÎÔ‡ ¤ÏÂÁ¯Ô˘ Û ÂӉ¯fiÌÂÓË

¶·È‰È·ÙÚÈ΋ 2005;68:366-370

∂ÈÎfiÓ· 5. ¡Â·ÓÈ΋ ˘·ÏÈÓÈ΋ Èӈ̿وÛË: ÛÙË ‚ÈÔ„›·Ô˙ȉ›ˆÓ ·Ó¢ڛÛÎÂÙ·È ÂÓ·fiıÂÛË ÎÔÏÏ·ÁÔÓÔÔÈË̤ÓÔ˘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·ÛÙËÚÈfiÙË-Ù· ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ˘Ú‹ÓˆÓ.

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370 O. ¶··Ó‰Ú¤Ô˘ Î·È Û˘Ó.

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

µÈ‚ÏÈÔÁÚ·Ê›·

1. Rahman N, Dunstan M, Teare MD, Hanks S, EdkinsSJ, Hughes J et al. The gene for juvenile hyaline fi-bromatosis maps to chromosome 4q21. Am J HumGenet 2002;71:975-980.

2. Ishikawa H, Maeda H, Takamatsu H, Saito Y. Sys-temic hyalinosis (juvenile hyaline fibromatosis). Ul-trastructure of the hyaline with particular referenceto the cross-banded structure. Arch Dermatol Res1979;265:195-206.

3. Kitano Y, Horiki M, Aoki T, Sagami S. Two cases ofjuvenile hyalin fibromatosis. Some histological, elec-tron microscopic, and tissue culture observations.Arch Dermatol 1972;106:877-883.

4. Keser G, Karabulut B, Oksel F, Calli C, Ustun EE,Akalin T et al. Two siblings with juvenile hyaline fi-bromatosis: case reports and review of the literature.Clin Rheumatol 1999;18:248-252.

5. Allen PW. Selected case from the Arkadi M. RywlinInternational Pathology Slide Seminar: hyaline fi-bromatosis. 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 patientwith juvenile hyaline fibromatosis. Arch Dis Child1995;73:246-248.

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

8. Remberger K, Krieg T, Kunze D, Weinmann HM,Hubner G. Fibromatosis hyalinica multiplex (juve-nile hyalin fibromatosis). Light microscopic, elec-tron microscopic, immunohistochemical, and bio-chemical findings. Cancer 1985;56:614-624.

9. Iwata S, Horiuchi R, Maeda H, Ishikawa H. Systemichyalinosis or juvenile hyaline fibromatosis. Ul-trastructural and biochemical study of cultured skinfibroblasts. Arch Dermatol Res 1980;267:115-121.

10. Breier F, Fang-Kircher S, Wolff K, Jurecka W. Juve-nile hyaline fibromatosis: impaired collagen metab-olism in human skin fibroblasts. Arch Dis Child1997;77:436-440.

11. Dowling O, Difeo A, Ramirez MC, Tukel T, Narla G,Bonafe L et al. Mutations in capillary morphogene-sis gene-2 result in the allelic disorders juvenile hya-line fibromatosis and infantile systemic hyalinosis.Am J Hum Genet 2003;73:957-966.

12. Hanks S, Adams S, Douglas J, Arbour L, AthertonDJ, Balci S et al. Mutations in the gene encodingcapillary morphogenesis protein 2 cause juvenilehyaline fibromatosis and infantile systemic hyali-nosis. Am J Hum Genet 2003;73:791-800.

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371CASE REPORT

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 withmultiple painless subcutaneous nodules, hyalinous cutaneous lesions of various sizes, gingivalhypertrophy, limb contractures and low bone density. The visceral organs are not affected. Mentaldevelopment is normal. The diagnosis is confirmed by biopsy of the lesions. There is no specifictreatment other than numerous operations for functional and aesthetic reasons. The case is describedof 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) hastemporarily improved the patient’s quality of life, rendering her able to chew and speak again, asbefore.

Key words

Nodules, macules, fibromatosis.

1 1st Paediatric Clinic of theUniversity of Athens,“Aghia Sophia” Children’sHospital, Athens

2 Plastic Surgery Clinic,“Aghia Sophia” Children’sHospital, Athens

3 Laboratory of Pathology-Anatomy, “Aghia Sophia”Children’s Hospital, Athens

4 Institute of CancerResearch, Surrey, UK

Correspondence:

Ourania Papandreou 1st Paediatric Clinic of theUniversity of Athens, “Aghia Sophia” Children’sHospital Thivon & Levadias, 115 27, Athens E-mail: [email protected]

Date of submission: 26-07-2004 Date of approval: 01-04-2005

Paediatriki 2005;68:371

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·371

Page 58: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

372 ∂¡¢π∞º∂ƒOÀ™∞ ¶∂ƒπ¶∆ø™∏

1 ¡ÂÔÁÓÔÏÔÁÈÎfi ∆Ì‹Ì· ∂™À,πÔÎÚ¿ÙÂÈÔ °¶¡£ÂÛÛ·ÏÔӛ΢

2 ¡ÂÔÁÓÔÏÔÁÈÎfi ∆Ì‹Ì·,¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô §¿ÚÈÛ·˜

3 ∫¤ÓÙÚÔ ªÔÚȷ΋˜µÈÔÏÔÁ›·˜ ηÈ∫˘ÙÙ·ÚÔÁÂÓÂÙÈ΋˜,ª·ÈÂ˘Ù‹ÚÈÔ “§ËÙÒ”,∞ı‹Ó·

AÏÏËÏÔÁÚ·Ê›·:

∞ϤͷӉÚÔ˜ ∞Ó‰Ú¤Ô˘ÃÂÈ̈ӛ‰Ô˘ 15∆.∫. 551 33, ∫·Ï·Ì·ÚÈ¿,£ÂÛÛ·ÏÔÓ›ÎË ∂-mail: [email protected]

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

¶·È‰È·ÙÚÈ΋ 2005;68:372-375

™‡Ó‰ÚÔÌÔ ÌÈÎÚÔÂÏÏ›ÌÌ·ÙÔ˜ 22q11.2 ÛÂ

ÔÈÎÔÁ¤ÓÂÈ· Ì ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔÓ Ê·ÈÓfiÙ˘Ô.

¶ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË

∞. ∞Ó‰Ú¤Ô˘1, µ. ∞ÏÂÍ·Ó‰ÚÔÔ‡ÏÔ˘ - ∆ÛÈÎڛη2, ∞. ™·ÎÂÏÏ·ÚÔÔ‡ÏÔ˘1, ª. °Ô˘‰ÂÛ›‰Ô˘2,

§. ºÏˆÚÂÓÙ›Ó - ∞Ú¿Ú3

¶ÂÚ›ÏË„Ë

¶ÂÚÈÁÚ¿ÊÂÙ·È Ì›· ÔÈÎÔÁ¤ÓÂÈ· Ì ۇӉÚÔÌÔ ÂÏÏ›ÌÌ·ÙÔ˜ ÙÔ˘ ¯ÚˆÌÔÛˆÌÈÎÔ‡ ÙÌ‹Ì·ÙÔ˜ 22q11.2 ÛÂÙÚ›· ̤ÏË Ù˘ Î·È ÌÂÁ¿ÏË ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔÓ Ê·ÈÓfiÙ˘Ô. ∫ÏÈÓÈο, ÙÔ Û‡Ó‰ÚÔÌÔ ÂΉËÏÒıËΠÛÙË ÌË-Ù¤Ú· Ì ¤ÓÚÈÓË ÔÌÈÏ›· ÏfiÁˆ ˘ÂÚˆÈÔÊ·Ú˘ÁÁÈ΋˜ ·Ó¿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 ÌÈÎÚÔ-ÁÓ·ı›· Î·È ·ÚÔ‰È΋ ˘·Û‚ÂÛÙÈ·ÈÌ›·. ∏ ‰È¿ÁÓˆÛË ¤ÁÈÓ ·Ú¯Èο ÛÙÔ ÙÚ›ÙÔ ·È‰› Î·È ÌÂÙ¿ ÛÙË ÌË-Ù¤Ú·. ™ÙÔ Ù¤Ù·ÚÙÔ ·È‰› Ë ‰È¿ÁÓˆÛË ¤ÁÈÓ ÚÔÁÂÓÓËÙÈο ÌÂÙ¿ ·fi ·ÌÓÈԷڷΤÓÙËÛË. ™Â fiϘ ÙȘÂÚÈÙÒÛÂȘ Ë ‰È¿ÁÓˆÛË ¤ÁÈÓ Ì ÙËÓ Ù¯ÓÈ΋ ÙÔ˘ ÊıÔÚ›˙ÔÓÙ· in situ ˘‚ÚȉÈÛÌÔ‡ (FISH).

§¤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˜ ·Óˆ-

̷ϛ˜ ·fi ÙËÓ Î·Ú‰È¿, ÙÔÓ ı‡ÌÔ Î·È ÙÔ˘˜ ·-

Ú·ı˘ÚÂÔÂȉ›˜ ·‰¤Ó˜, ·ÎfiÌË Î·È ·Ó¿ÌÂÛ· ÛÂ

¿ÙÔÌ· Ù˘ ›‰È·˜ ÔÈÎÔÁ¤ÓÂÈ·˜ (1-4). ∆· Ê·ÈÓÔÙ˘-

Èο ¯·Ú·ÎÙËÚÈÛÙÈο, fiˆ˜ ÎÚ·ÓÈÔÚÔÛˆÈΤ˜,

ηډȷÁÁÂȷΤ˜, ˆÙÔÚÈÓÔÏ·Ú˘ÁÁÔÏÔÁÈΤ˜, Ì˘Ô-

ÛÎÂÏÂÙÈΤ˜, ÂÓ‰ÔÎÚÈÓÔÏÔÁÈΤ˜ Î·È ·ÓÔÛÔÏÔÁÈΤ˜

·ÓˆÌ·Ï›Â˜, ÌÔÚ› Ó· ‰È·Ê¤ÚÔ˘Ó ·ÎfiÌË Î·È ÌÂ-

ٷ͇ ÌÔÓÔˆÔÁÂÓÒÓ ‰È‰‡ÌˆÓ (5). ∏ ‚·Ú‡ÙËÙ·

̤۷ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÌÔÚ› Ó· ÔÈΛÏÏÂÈ ·fi

·Û˘Ìو̷ÙÈÎÔ‡˜ 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·Èfi-

ÙÂÚ· Û‡Ó‰ÚÔÌ· DiGeorge Î·È ˘ÂÚˆÈÔ-ηډÈÔ-

ÚÔÛˆÈÎfi (velo-cardio-facial), ηıÒ˜ Î·È ¿ÙÔ-

Ì· 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 Â-

ÚÈÛÙ·ÙÈο (7,8).

¶ÂÚÈÁÚ¿ÊÂÙ·È, ÁÈ· ÚÒÙË ÊÔÚ¿ ÛÙËÓ ÂÏÏË-

ÓÈ΋ ‚È‚ÏÈÔÁÚ·Ê›·, ¤Ó· ÔÈÎÔÁÂÓ¤˜ Û‡Ó‰ÚÔÌÔ ÌÈ-

ÎÚÔÂÏÏ›ÌÌ·ÙÔ˜ 22q11.2, Ô˘ ·ÊÔÚ¿ ÛÙË ÌËÙ¤-

Ú· Î·È ÛÙ· ‰‡Ô ·fi Ù· Ù¤ÛÛÂÚ· ·È‰È¿ Ù˘. ™ÙÔ

‰Â‡ÙÂÚÔ ¿Û¯ÔÓ ·È‰› Ë ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰Úfi-

ÌÔ˘ ¤ÁÈÓ ÚÔÁÂÓÓËÙÈο.

¶ÂÚÈÁÚ·Ê‹ ÂÚÈÙÒÛˆÓ

¶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 ¯ÚˆÌÔۈ̿وÓ

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

٤ٷÚÙÔ, ›¯·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 ÌÂ-

Ù¿ 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

Pediatr Sept-Oct 05 17-10-05 16:51 ™ÂÏ›‰·372

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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˘ ÔÈ-

ÎÔÁ¤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˘Ìو̷ÙÔÏÔÁ›· ηډȷ΋˜ οÌ-

„˘. ∏ ·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 ıÂ-

ÙÈ΋. ∆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›· ‰fi-

ÛË 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·ÊË-

Ùfi ‹·Ú 3 cm Î·È Û˘ÛÙÔÏÈÎfi ʇÛËÌ· 1-2/6. O

˘ÂÚ˯ÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ù˘ ηډȿ˜ ¤‰ÂÈÍÂ

ÙËÓ ‡·ÚÍË ÎÔÈÓÔ‡ ·ÚÙËÚÈ·ÎÔ‡ ÎÔÚÌÔ‡ Ù‡Ô˘

ππ Ì ÌÂÛÔÎÔÈÏȷ΋ ÂÈÎÔÈÓˆÓ›·. ∫·Ù¿ ÙËÓ ÂÈ-

Û·ÁˆÁ‹ ÛÙÔ ÓÔÛÔÎÔÌ›Ô, ÙÔ ÓÂÔÁÓfi ›¯Â Úfi‰È-

ÓÔ ¯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 ÂÚÈÊÂÚÂÈ·-

΋ ΢ÎÏÔÊÔÚ›· Î·È ¤ÓÙÔÓ˜ ÂÚÈÊÂÚÈΤ˜ Ûʇ-

ÍÂȘ. ∏ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη ¤‰ÂÈÍ ·˘ÍË̤-

ÓË 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 ·Ïη-

ÏÈ΋ ʈÛÊ·Ù¿ÛË 142 IU/L. H PTH ‹Ù·Ó 71

pg/ml, Ì ÙÈÌ‹ Ca++=0,93 mmol/l. ∆Ô ˘ÂÚË-

¯ÔÁÚ¿ÊËÌ· ¤‰ÂÈÍ ˘ÔÏ·ÛÙÈÎfi ı‡ÌÔ, ÂÓÒ ÔÈ

·Ú·ı˘ÚÂÔÂȉ›˜ ‰ÂÓ ·ÂÈÎÔÓ›ÛÙËηÓ. ∆Ô

˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÙˆÓ ÓÂÊÚÒÓ ‹Ù·Ó Ê˘ÛÈÔÏÔ-

ÁÈÎfi. ™ÙÔÓ ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ‰È·ÈÛÙÒıËÎÂ

·Ô˘Û›· ·ÓÔÛÔ˚ηÓÒÓ ∆-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ, Ïfi-

Áˆ ÌË ‰È·ÊÔÚÔÔ›ËÛ‹˜ ÙÔ˘˜ Û ÏÂÈÙÔ˘ÚÁÈÎÔ‡˜

ÏËı˘ÛÌÔ‡˜. O ¯ÚˆÌÔÛˆÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÛÙÔ

¯ÚˆÌfiۈ̷ 22 Ì ÙËÓ Ù¯ÓÈ΋ FISH Î·È ¯ÚË-

ÛÈÌÔÔÈÒÓÙ·˜ ÊıÔÚ›˙ÔÓÙ· DNA ·ÓȯÓÂ˘Ù‹

¤‰ÂÈÍ ÌÈÎÚÔ¤ÏÏÂÈÌÌ· ÛÙË ı¤ÛË 22q11.2. ∏

˘·Û‚ÂÛÙÈ·ÈÌ›· ·ÔηٷÛÙ¿ıËΠÙË 12Ë Ë̤-

Ú· 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 ¤ÁÈ-

Ó ÌÂÙ·ÊÔÚ¿ Û ·È‰ÔηډÈÔ¯ÂÈÚÔ˘ÚÁÈÎfi Τ-

ÓÙÚÔ ÁÈ· ¯ÂÈÚÔ˘ÚÁÈ΋ ‰ÈfiÚıˆÛË Ù˘ Û˘ÁÁÂÓÔ‡˜

ηډÈÔ¿ıÂÈ·˜. §›Á˜ Ë̤Ú˜ ·ÚÁfiÙÂÚ· ηÈ

ÚÈÓ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË ·Ó¤Ù˘Í ۋ-

„Ë ·fi „¢‰ÔÌÔÓ¿‰· Î·È ·Â‚›ˆÛÂ.

∆¤Ù·ÚÙÔ ·È‰›: ¶ÚfiÎÂÈÙ·È ÁÈ· ·ÁfiÚÈ, Ô˘ ÌÂ-

ٷʤÚıËΠÛÙËÓ ÎÏÈÓÈ΋ Û ËÏÈΛ· 28 ˆÚÒÓ Ïfi-

Áˆ ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Û˘Ó‰ÚfiÌÔ˘ ÌÈ-

ÎÚÔÂÏÏ›ÌÌ·ÙÔ˜ 22q11.2. ∞fi ÙÔ ÂÚÈÁÂÓÓËÙÈ-

Îfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È fiÙÈ Ë ÌËÙ¤Ú· ›¯Â ÌÈ-

ÎÚÔ·ÈÌÔÚÚ·Á›Â˜ ÛÙÔÓ 3Ô-4Ô Ì‹Ó· Ù˘ ÂÁ΢ÌÔ-

Û‡Ó˘. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ¤ÁÈÓ ÌÂ

¯ÚˆÌÔÛˆÌÈ΋ ·Ó¿Ï˘ÛË Ì ÙË Ì¤ıÔ‰Ô FISH ÛÂ

·ÌÓȷο ·ÙÙ·Ú· Ô˘ ÂÏ‹ÊıËÛ·Ó Ì ·ÌÓÈÔΤ-

ÓÙËÛË 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‹ ∂£). ∏ ‚·ıÌÔÏÔ-

Á›· Apgar ‹Ù·Ó 8 ÛÙÔ 1Ô ÏÂÙfi 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˘ÛÈÔÏÔ-

ÁÈ΋. 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›· ·ÓÙÈÌÂ-

Ùˆ›ÛÙËΠ̠¯ÔÚ‹ÁËÛË ·fi ÙÔ ÛÙfiÌ· ÁÏ˘ÎÔ-

ÓÈÎÔ‡ ·Û‚ÂÛÙ›Ô˘ ÁÈ· 14 Ë̤Ú˜. ™ÙËÓ ·ÎÙÈÓÔ-

ÁÚ·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˘ÛÈÔ-

ÏÔÁÈÎfi˜. O ·ÓÔÛÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍ ÔÚÈ·-

ο ¯·ÌËÏfi ÔÛÔÛÙfi ·ÓÔÛÔ˚ηÓÒÓ ∆-΢ÙÙ¿ÚˆÓ

Î·È ÙˆÓ ˘ÔÔÌ¿‰ˆÓ ÙÔ˘˜, ηıÒ˜ Î·È ÂÏ¿ÙÙˆÛË

ÙˆÓ ÔÛÔÛÙÒÓ ¤ÎÊÚ·Û˘ ÙˆÓ ÌÔÚ›ˆÓ ÚÔ-

ÛÎfiÏÏËÛ˘ 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 ÏÂÙfi ÙÚfiÌÔ ÙˆÓ ¿ÎÚˆÓ. ¡Ô-

ÛËχÙËΠ17 Ë̤Ú˜ Î·È ÂÍ‹Ïı Ì ԉËÁ›Â˜

ÁÈ· ÚÔÛ‰ÈÔÚÈÛÌfi ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ·›Ì·ÙÔ˜ ηÈ

ÙˆÓ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ Û ٷÎÙ¿ ¯ÚÔÓÈο

‰È·ÛÙ‹Ì·Ù·, ηıÒ˜ Î·È Û ÂÚÈÙÒÛÂȘ ÛÙÚ˜,

ÏÔÈÌÒ͈Ó, ÂÌÊ¿ÓÈÛ˘ ¢ÂÚÂıÈÛÙfiÙËÙ·˜, ÙÚfi-

ÌÔ˘ ‹ Û·ÛÌÒÓ. ª¤¯ÚÈ Û‹ÌÂÚ· (ËÏÈΛ· 2 ÂÙÒÓ)

‰ÂÓ ·ÚÔ˘Û›·Û ÙÚfiÌÔ, Û·ÛÌÔ‡˜ ‹ Û˘¯Ó¤˜ ÏÔÈ-

ÌÒÍÂȘ 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

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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 ÛÂ 1:4000-

6000 ÁÂÓÓ‹ÛÂȘ ˙ˆÓÙ·ÓÒÓ ÓÂÔÁÓÒÓ (4,9). £Âˆ-

ÚÂ›Ù·È Ë ‰Â‡ÙÂÚË ÈÔ Û˘¯Ó‹ ¯ÚˆÌÔÛˆÌÈ΋ ·Óˆ-

Ì·Ï›· ÌÂÙ¿ ÙËÓ ÙÚÈۈ̛· 21 (4,7,10). OÈ ÂÚÈÛ-

ÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ (ÂÚ›Ô˘ 90%) ÚÔ·-

ÙÔ˘Ó de novo, ÂÓÒ ÔÛÔÛÙfi ÂÚ›Ô˘ 10% ÎÏË-

ÚÔÓÔÌÂ›Ù·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡-

ÓÙ· ¯·Ú·ÎÙ‹Ú· (4). ∏ Û˘¯ÓfiÙËÙ· ÙÔ˘ Û˘Ó‰Úfi-

ÌÔ˘ 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˘ ÌÔÚ› 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‚Â-

ÛÙÈ·ÈÌ›·, ·ÓÔÛÔ·Ó¿ÚÎÂÈ·, Ì·ıËÛȷΤ˜ ‰˘ÛÎÔ-

ϛ˜ Î·È ÌÂÚÈΤ˜ ÊÔÚ¤˜ ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ÌÔÚÊÔ-

ÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÚÔÛÒÔ˘ (4,6).

∏ ‚·Ú‡ÙËÙ· ̤۷ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÔÈΛÏ-

ÏÂÈ ·fi ·Û˘Ìو̷ÙÈ΋ ÂÌÊ¿ÓÈÛË ÛÙÔ˘˜ ÁÔ-

Ó›˜ ̤¯ÚÈ ÛÔ‚·Ú¤˜ ÂΉËÏÒÛÂȘ Î·È ‰˘ÛÌÔÚ-

ʛ˜ ÛÙ· ·È‰È¿ (3,4). ∏ ÎÏËÚÔÓÔÌÈ΋ ÌÂÙ·‚›-

‚·ÛË Â›Ó·È ÈÔ Û˘¯Ó‹ ·fi ÙË ÌËÙ¤Ú· Î·È Á›ÓÂ-

Ù·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ¯·Ú·-

ÎÙ‹Ú·. ™˘Ó‹ıˆ˜, ÔÈ ÌËÙ¤Ú˜ ÂÌÊ·Ó›˙Ô˘Ó ÂÏ¿Û-

ÛÔÓ˜ ÌÔÚÊÔÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÚÔÛÒÔ˘,

˘ÂÚˆ˚ÔÊ·Ú˘ÁÁÈ΋ ·Ó¿ÚÎÂÈ· Î·È ‰È·Ù·Ú·¯¤˜

ÛÙËÓ „˘¯ÔÎÔÈÓˆÓÈ΋ ÙÔ˘˜ Û˘ÌÂÚÈÊÔÚ¿ Ô˘

ÔÈΛÏÏÔ˘Ó ·fi ‹È˜ Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜

̤¯ÚÈ ÛÔ‚·Ú‹ ·ÓÙÈÎÔÈÓˆÓÈ΋ ÚÔÛˆÈÎfiÙËÙ·

Î·È ‚·ÚȤ˜ „˘¯È·ÙÚÈΤ˜ ηٷÛÙ¿ÛÂȘ. ∆Ô Ì¤ÁÂ-

ıÔ˜ Ù˘ ¤ÏÏÂȄ˘ ‰ÂÓ ıˆÚÂ›Ù·È ˘Â‡ı˘ÓÔ ÁÈ·

ÙË Ê·ÈÓÔÙ˘È΋ ÔÈÎÈÏÔÌÔÚÊ›· (12). ¶Èı·ÓÔ-

ÏÔÁÂ›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 ÔÔ›· ·ÚÔ˘Û›·˙Â ¤ÓÚÈÓË ÔÌÈÏ›·, Ì·-

ıËÛȷο ÚÔ‚Ï‹Ì·Ù· Î·È Â˘¿ıÂÈ· Û ÏÔÈÌÒ-

ÍÂȘ. ∆Ô ¤Ó· ·È‰› ÂÌÊ¿ÓÈÛ ÙËÓ ÎÏ·ÛÈ΋ ÙÚÈ¿-

‰· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ DiGeorge ÌÂ ·Ï·Û›· ÙÔ˘

ı‡ÌÔ˘ ·‰¤Ó·, ÛÔ‚·Ú‹ conotruncal ηډȷ΋

·ÓˆÌ·Ï›· (·ÚÙËÚÈ·Îfi ÎÔÚÌfi Ù‡Ô˘ ππ) Î·È ‚·-

ÚÈ¿ ·ÓÔÛÔ·Ó¿ÚÎÂÈ· Î·È ·Â‚›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˘ cono-

truncal, ÌÂ Û˘¯ÓfiÙÂÚË ÙËÓ ÙÂÙÚ·ÏÔÁ›· Fallot

(4). ¶ÔÛÔÛÙfi 69% ÙˆÓ ·Û¯fiÓÙˆÓ ¤¯ÂÈ ·ÓˆÌ·-

ϛ˜ Ù˘ ˘ÂÚÒ·˜, ΢ڛˆ˜ ˘ÂÚˆÈÔÊ·Ú˘ÁÁÈ΋

·Ó¿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·È-

Ì›·, ‚) ˘·Û‚ÂÛÙÈ·ÈÌÈÎfi˜ ˘Ô·Ú·ı˘ÚÂÔÂȉÈ-

ÛÌfi˜ Ô˘ ÂÌÊ·Ó›˙ÂÙ·È ÁÈ· ÚÒÙË ÊÔÚ¿ ÛÂ ÂÓ‹-

ÏÈΘ, Á) ˘·Û‚ÂÛÙÈ·ÈÌÈÎfi˜ ˘Ô·Ú·ı˘ÚÂÔÂÈ-

‰ÈÛÌfi˜ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· Ô˘ ‚ÂÏÙÈÒÓÂÙ·È

·˘ÙfiÌ·Ù· ÛÙËÓ ·È‰È΋ ËÏÈΛ· Î·È Â·Ó¤Ú¯Â-

Ù·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˘ÚÂÔ-

ÂȉÈÛÌfi (12).

∏ ·ÓÔÛÔ·Ó¿ÚÎÂÈ· ÂÚÈÏ·Ì‚¿ÓÂÈ ÂÏ¿ÙÙˆ-

ÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙˆÓ ÚÔÂÚ¯ÔÌ¤ÓˆÓ ·fi ÙÔÓ

ı‡ÌÔ ·‰¤Ó·, ηıÒ˜ Î·È ÏÂÈÙÔ˘ÚÁÈΤ˜ ·Ó¿Ú-

ÎÂȘ ÙˆÓ ∆ Î·È µ ΢ÙÙ¿ÚˆÓ (4).

∆Ô ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÚÔÛˆÂ›Ô ÂÚÈÏ·Ì‚¿-

ÓÂÈ ÌÈÎÚfi ÛÙfiÌ·, ÔȉËÌ·ÙÒ‰Ë Ì¿ÙÈ·, ÂȤ‰ˆ-

ÛË Ù˘ Ú¿¯Ë˜ Ù˘ ̇Ù˘, ˘ÂÚˆÈÔÛ¯ÈÛÙ›·, ÌÈ-

ÎÚÔÁÓ·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›˜ ÌÂ

Û‡Ó‰ÚÔÌÔ ÂÏÏ›ÌÌ·ÙÔ˜ 22q11 ÂΉËÏÒÓÔ˘Ó

‹È·, ·ÏÏ¿ Ù˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο ÚÔÛÒÔ˘

(13), Ô˘ ·fi ÌfiÓ· ÙÔ˘˜ ‰ÂÓ Â›Ó·È Èηӿ Ó· η-

Ù¢ı‡ÓÔ˘Ó ÛÙËÓ ˘ÔΛÌÂÓË ‰È¿ÁÓˆÛË (4). OÈ

ÂÚÈÛÛfiÙÂÚÔÈ ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ¤ÏÏÂÈÌÌ·

22q11 ¤¯Ô˘Ó ‹ÈÔ˘ ÚÔ˜ ̤ÙÚÈÔ˘ ‚·ıÌÔ‡ ÓÔË-

ÙÈ΋ ˘ÛÙ¤ÚËÛË (4,8).

∂Ӊ›ÍÂȘ ÁÈ· ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ·Ô-

ÙÂÏÔ‡Ó Ë ‡·ÚÍË ÂÓfi˜ ÁÔÓ¤· ÌÂ ·ÒÏÂÈ·

22q11, Ë ÚÔËÁËı›۷ Á¤ÓÓËÛË ÂÓfi˜ ·È‰ÈÔ‡

Ì ¤ÏÏÂÈÌÌ· 22q11, ηıÒ˜ Î·È Ù· ¤Ì‚Ú˘· ÛÙ·

ÔÔ›· Ù›ıÂÙ·È Ë ‰È¿ÁÓˆÛË Î·Ú‰ÈÔ¿ıÂÈ·˜ ÙÔ˘

ÛÙÂϯȷ›Ô˘ ÎÒÓÔ˘ (conotruncal) Î·È Û˘Ó‰Úfi-

ÌÔ˘ conotruncal abnormal face (8).

™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ¤¯Ô˘Ó ·Ó·ÊÂÚı› Ï›Á˜ Â-

ÚÈÙÒÛÂȘ ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ÙÔ˘ Û˘Ó-

‰ÚfiÌÔ˘ 22q11 (3,7,9). ¶ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË

¶·È‰È·ÙÚÈ΋ 2005;68:372-375

Pediatr Sept-Oct 05 17-10-05 16:52 ™ÂÏ›‰·374

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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‰ȿÁÓˆÛË ÛÙÔ ÚÒÙÔ ·È‰›. ∏ ÚÔÁÂÓÓËÙÈ΋‰È¿ÁÓˆÛË ÂÈÙÚ¤ÂÈ ÙË ÛˆÛÙ‹ ÁÂÓÂÙÈ΋ Û˘Ì-‚Ô˘Ï‹. ∂›Ó·È ¯Ú‹ÛÈÌË ÛÙË Ï‹„Ë ·ÔÊ¿ÛˆÓÎ·È ÚÔÂÙÔÈÌ·ÛÈÒÓ ÁÈ· ÙËÓ ÂÂÚ¯fiÌÂÓË Á¤ÓÓ·,ÙËÓ ÂÚÈÁÂÓÓËÙÈ΋ ÊÚÔÓÙ›‰·, ÙÔÓ Û¯Â‰È·ÛÌfiÙ˘ ¿ÌÂÛ˘ È·ÙÚÈ΋˜ ÊÚÔÓÙ›‰·˜ ÛÙË ÓÂÔÁÓÈ΋ÂÚ›Ô‰Ô Î·È ÙËÓ ÚÔÂÙÔÈÌ·Û›· ÁÈ· ›ÁÔ˘Û·Î·ÈÛ·ÚÈ΋ ÙÔÌ‹ Û ÂÚ›ÙˆÛË ÂÌ‚Ú˘˚ÎÔ‡ÛÙÚ˜ (8). ÃÚËÛÈ̇ÂÈ, ›Û˘, ÁÈ· ÙÔÓ ÂÓÙÔÈ-ÛÌfi ¿ÏÏˆÓ ·Û¯fiÓÙˆÓ ÌÂÏÒÓ Ù˘ ÔÈÎÔÁ¤ÓÂÈ-·˜. ŸÌˆ˜, Ë ÚÔÁÂÓÓËÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋·Ú·Ì¤ÓÂÈ ‰‡ÛÎÔÏË ÏfiÁˆ Ù˘ ÎÏÈÓÈ΋˜ ÔÈÎÈ-ÏÔÌÔÚÊ›·˜ Ô˘ ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÛÙÔ Û‡Ó‰ÚÔ-ÌÔ, ηıfiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ Û˘Û¯¤ÙÈÛË Ê·ÈÓfiÙ˘-Ô˘-ÁÔÓfiÙ˘Ô˘ Î·È Ù· Û˘ÌÙÒÌ·Ù· ÔÈΛÏ-ÏÔ˘Ó ·Ó¿ÌÂÛ· ÛÙÔ Û‡Ó‰ÚÔÌÔ DiGeorge, ÙËÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË Î·È ÙȘ ‹È˜ Ì·ıËÛȷΤ˜‰˘ÛÎÔϛ˜. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ÂÈÙÚ¤-ÂÈ Î·È ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi ÙÔ˘ Û¯ÂÙÈÎÔ‡ ÎÈÓ‰‡-ÓÔ˘ ·ÓÂÌÊ¿ÓÈÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ™Â ÂÚÈ-ÙÒÛÂȘ de novo ÂÌÊ¿ÓÈÛ˘ Û ¤Ó· ·È‰›, ÔΛӉ˘ÓÔ˜ ·ÓÂÌÊ¿ÓÈÛ˘ Û ÂfiÌÂÓÔ ·È‰› ›-Ó·È 1-3%. ¶Ú¤ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ ·ÎfiÌ· ηÈfiÙ·Ó Î·Ó¤Ó·˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ ¿Û¯ÔÓÙÔ˜ ·È-‰ÈÔ‡ ‰ÂÓ Ê¤ÚÂÈ ÙÔ ¤ÏÏÂÈÌÌ·, ˘¿Ú¯ÂÈ ÌÈÎÚfi˜Î›Ó‰˘ÓÔ˜ ·ÓÂÌÊ¿ÓÈÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÛÂÂfiÌÂÓÔ ·È‰›, ÂÂȉ‹ ‰ÂÓ ÌÔÚ› Ó· ·Ô-ÎÏÂÈÛÙ› Ë ÂÚ›ÙˆÛË ÌˆÛ·˚ÎÈÛÌÔ‡ ÙˆÓ ÁÔÓ¿-‰ˆÓ (3,8,14).

∏ ÚÒÈÌË ‰È¿ÁÓˆÛË Â›Ó·È ÛËÌ·ÓÙÈ΋ ÁÈ·ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ·ÛıÂÓÔ‡˜. øÛÙfiÛÔ, ›-Ó·È ‰‡ÛÎÔÏÔ Ó· ÔÚÈÛÙÔ‡Ó ÎÚÈÙ‹ÚÈ· ÁÈ· ÙÔ fiÙÂı· 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˘ÓηډÈÔ¿ıÂÈ· Ù‡Ô˘ ‰È·ÎÔ‹˜ ·ÔÚÙÈÎÔ‡ Ùfi-ÍÔ˘, ÙÂÙÚ·ÏÔÁ›·˜ Fallot Î·È ·ÚÙËÚÈ·ÎÔ‡ ÎÔÚ-ÌÔ‡ Ù‡Ô˘ II, ηıÒ˜ Î·È Û ‚Ú¤ÊË Ô˘ ÂÌÊ·-Ó›˙Ô˘Ó ·ÓÂÍ‹ÁËÙË, ›ÌÔÓË ˘·Û‚ÂÛÙÈ·ÈÌ›· ÛÂÛ˘Ó‰˘·ÛÌfi Ì ÂÏ·Ùو̤ÓÔ ·ÚÈıÌfi ∆-΢ÙÙ¿-ÚˆÓ Î·È ‰˘ÛÌÔÚÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο. ŒÓ‰ÂÈÍËÁÈ· 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 ¿ÏÏÂ˜Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ (4,10), ηıÒ˜ Î·È Û ·È-‰È¿ Ì ÌË ÏÂÎÙÈΤ˜ (non-verbal) Ì·ıËÛȷΤ˜‰˘ÛÎÔϛ˜ (4,10).

µÈ‚ÏÈÔÁÚ·Ê›·

1. Kasprzak L, Der Kaloustian VM, Elliott AM, ShevellM, Lejtenyi C, Eydoux P. Deletion of 22q11 in twobrothers with different phenotype. Am J Med Genet1998;75:288-291.

2. Hillebrand G, Siebert R, Simeoni E, Santer R. DiGe-orge syndrome with discordant phenotype inmonozygotic twins. J Med Genet 2000;37:E23.

3. Levy-Mozziconacci A, Piquet C, Heurtevin PC,Philip N. Prenatal diagnosis of 22q11 microdeletion.Prenat Diagn 1997;17:1033-1037.

4. McDonald-McGinn DM, Kirschner R, GoldmuntzE, Sullivan K, Eicher P, Gerdes M et al. The Philadel-phia story: the 22q11.2 deletion: report on 250 pa-tients. Genet Couns 1999;10:11-24.

5. Goodship J, Cross I, Scambler P, Burn J. Monozygot-ic twins with chromosome 22q11 deletion and dis-cordant phenotype. J Med Genet 1995;32:746-748.

6. Matsuoka R, Takao A, Kimura M, Imamura S, Kon-do C, Joh-o K et al. Confirmation that the conotrun-cal anomaly face syndrome is associated with a dele-tion within 22q11.2. Am J Med Genet 1994;53:285-289.

7. Davidson A, Khandelwal M, Punnett HH. Prenataldiagnosis of the 22q11 deletion syndrome. PrenatDiagn 1997;17:380-383.

8. Oh DC, Min JY, Lee MH, Kim YM, Park SY, WonHS et al. Prenatal diagnosis of tetralogy of Fallot as-sociated with chromosome 22q11 deletion. J KoreanMed Sci 2002;17:125-128.

9. Driscoll DA, Salvin J, Sellinger B, Budarf ML,McDonald-McGinn DM, Zackai EH et al. Preva-lence of 22q11 microdeletions in DiGeorge and ve-locardiofacial syndromes: implications for geneticcounselling and prenatal diagnosis. J Med Genet1993;30:813-817.

10. Goodship J, Cross I, LiLing J, Wren C. A populationstudy 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 microdeletion22q11 in newborn infants with congenitalconotruncal cardiac anomalies. Eur J Pediatr 1998;157:881-884.

12. Cuneo BF, Driscoll DA, Gidding SS, Langman CB.Evolution of latent hypoparathyroidism in familial22q11 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-syndromicconotruncal cardiac defects. Eur J Pediatr 1996;155:619-620.

14. Sitch FL, James RS, Cockwell AE, Hatchwell E. Gon-adal mosaicism for a submicroscopic deletion ofchromosome region 22q11. Eur J Hum Genet1996;4:59.

¶·È‰È·ÙÚÈ΋ 2005;68:372-375

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376 CASE REPORT

1 Neonatology Department¡∏π, Hippokration GeneralHospital, Thessaloniki

2 Neonatology Department,University Hospital ofLarissa

3 Molecular Biology andCytogenetics Center,“Leto” Maternity Clinic,Athens

Correspondence:

Alexandros Andreou 15 Heimonidou Str. 551 33, Kalamaria, Thessaloniki ∂-mail: [email protected]

Date of submission: 27-09-2004 Date of approval: 29-03-2005

Paediatriki 2005;68:376

22q11.2 micro-deletion syndrome in a family

with variation in the phenotype.

Prenatal diagnosis

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 nasalvoice, 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 andtransient hypocalcaemia. The diagnosis was established first in the third child and subsequently in themother. On the fourth child the diagnosis was made prenatally, after amniocentesis. In all cases thediagnosis was made with the FISH technique.

Key words

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

Pediatr Sept-Oct 05 17-10-05 16:52 ™ÂÏ›‰·376

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377∂¶π∫∞πƒO £∂ª∞

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§¤ÍÂȘ ÎÏÂȉȿ

∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, ÓÂÔÁÓfi, ÂÁΤʷÏÔ˜.

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AÏÏËÏÔÁÚ·Ê›·:

∂ϤÓË ªÔ‡˙· µ’ ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜¡ÔÛËÏ›·˜ ¡ÂÔÁÓÒÓ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” ∆.∫. 115 27, ∞ı‹Ó· E-mail: [email protected]

∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 21-06-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 17-06-2005

¶·È‰È·ÙÚÈ΋ 2005;68:377-384

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∞Ó Î·È Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ì ÙË ¯Ú‹ÛË ÙÔ˘ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·, Ë ÂÈ‚›ˆÛËÙˆÓ ÚfiˆÚˆÓ ÓÂÔÁÓÒÓ Ì ۇӉÚÔÌÔ ·Ó·Ó¢-ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜ ¤¯ÂÈ ·˘ÍËı›, Ë ¯ÚfiÓÈ· ÓfiÛÔ˜ÙˆÓ Ó¢ÌfiÓˆÓ ·Ú·Ì¤ÓÂÈ ¤Ó· ÛÔ‚·Úfi Úfi‚ÏË-Ì· ÛÙȘ ÌÔÓ¿‰Â˜ ÂÓÙ·ÙÈ΋˜ ÓÔÛËÏ›·˜ ÓÂÔÁÓÒÓ.

∏ ¯ÚfiÓÈ· ÓfiÛÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ ‹ ‚ÚÔÁ¯Ô-Ó¢ÌÔÓÈ΋ ‰˘ÛÏ·Û›· Â›Ó·È Ì›· ÛËÌ·ÓÙÈ΋ ·È-Ù›· ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÛÈÌfiÙËÙ·˜ ÙÔ˘ Úfiˆ-ÚÔ˘ ÓÂÔÁÓÔ‡. ∂ÈϤÔÓ, Â›Ó·È Ì›· ·fi ÙȘ ÈÔ Û˘-¯Ó¤˜ ·Èٛ˜ Ì·ÎÚÔÚfiıÂÛÌˆÓ ‰˘ÛÌÂÓÒÓ Û˘ÓÂ-ÂÈÒÓ ÛÙ· Ôχ ÌÈÎÚÔ‡ ‚¿ÚÔ˘˜ Á¤ÓÓËÛ˘ ÓÂÔ-ÁÓ¿, Ô˘ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ú·ÙÂٷ̤ÓË·Ú·ÌÔÓ‹ ÙÔ˘˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô, ÌÂ Û˘¯Ó¤˜ ·-ÓÂÈÛ·ÁˆÁ¤˜ Î·È ‰˘ÛÌÂÓ›˜ ÂÈÙÒÛÂȘ ÛÙË ÌÂÙ¤-ÂÈÙ· ·Ó¿Ù˘ÍË Î·È „˘¯ÔÎÈÓËÙÈ΋ ÙÔ˘˜ ÂͤÏÈÍË.

ø˜ ¯ÚfiÓÈ· ÓfiÛÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ ÔÚ›˙ÂÙ·È ËÂÍ¿ÚÙËÛË ·fi ÙÔ Ô͢ÁfiÓÔ ÛÙËÓ ËÏÈΛ· ÙˆÓ 36‚‰ÔÌ¿‰ˆÓ ‹ ÛÙȘ 28 Ë̤Ú˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË,Û˘Á¯ÚfiÓˆ˜ Ì ÂÈ̤ÓÔÓÙ· ·Ó·Ó¢ÛÙÈο ÚÔ-‚Ï‹Ì·Ù· Î·È Û˘Ì‚·Ù¤˜ ·ÓˆÌ·Ï›Â˜ ÛÙËÓ ·ÎÙÈÓÔ-ÁÚ·Ê›· ıÒÚ·ÎÔ˜.

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ηۛ·˜ ÛÙÔÓ ÓÂÔÁÓÈÎfi Ó‡ÌÔÓ· Ô˘ ¤¯ÂÈ ˘Ô-

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Î·È Ê·›ÓÂÙ·È fiÙÈ ·˘Ù‹ Ë ÊÏÂÁÌÔÓ҉˘ ·ÓÙ›‰Ú·-

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¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ. ™Â ¿ÏÏ· Úfiˆ-

Ú· ÓÂÔÁÓ¿, Ô˘ ‰ÂÓ ¤¯Ô˘Ó ÛÔ‚·Úfi ·Ó·Ó¢ÛÙÈ-

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ΛÓ˜ ·fi ÙÔÓ Ó‡ÌÔÓ·, ˘ÚÔ‰ÔÙÒÓÙ·˜ Ì›·

Pediatr Sept-Oct 05 17-10-05 16:52 ™ÂÏ›‰·377

Page 64: Παιδιατρική | Τόμος 68 • Τεύχος 5 • Σεπτέμβριος - Οκτώβριος 2005

378 ∂. ªÔ‡˙· Î·È Û˘Ó.

ÊÏÂÁÌÔÓÒ‰Ë ·¿ÓÙËÛË Ô˘ Ô‰ËÁ› Û ¯ÚfiÓÈ·

ÓfiÛÔ ÙˆÓ Ó¢ÌfiÓˆÓ.

∏ ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂÈ-

‰ÒÓ - Î·È Î˘Ú›ˆ˜ Ë ‰ÂÍ·ÌÂı·˙fiÓË - ¤¯ÂÈ ¯ÚËÛÈ-

ÌÔÔÈËı› ¢ڤˆ˜ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô ÁÈ·

ÙËÓ ÚfiÏË„Ë Î·È ÙË ıÂڷ›· Ù˘ ¯ÚfiÓÈ·˜ Ófi-

ÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ, ÂÍ·ÈÙ›·˜ Ù˘ ÈÛ¯˘Ú‹˜ ·ÓÙÈ-

ÊÏÂÁÌÔÓÒ‰Ô˘˜ ‰Ú¿Û˘ Ù˘.

¶Èı·ÓÔ› Ì˯·ÓÈÛÌÔ› Ù˘ ‰Ú¿Û˘ ÙˆÓ ÎÔÚÙÈ-

ÎÔÛÙÂÚÔÂȉÒÓ ÛÙÔÓ Ó‡ÌÔÓ· ÙÔ˘ ÓÂÔÁÓÔ‡ Â-

ÚÈÏ·Ì‚¿ÓÔ˘Ó ÙËÓ ·‡ÍËÛË Ù˘ Û‡ÓıÂÛ˘ ÙÔ˘ ÂÈ-

Ê·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·, ÙËÓ ·‡ÍËÛË Ù˘

·ÓÙÈÔÍÂȉˆÙÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜, ÙËÓ ·Ó·ÛÙÔÏ‹

Ù˘ Û‡ÓıÂÛ˘ ÚÔÛÙ·ÁÏ·Ó‰ÈÓÒÓ Î·È Ï¢ÎÔÙÚÈÂ-

ÓÒÓ, ÙË Ì›ˆÛË ÙˆÓ ÊÏÂÁÌÔÓˆ‰ÒÓ Ê·ÈÓÔ̤ӈÓ

Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi ÙȘ ΢ÙÙ·ÚÔΛÓ˜ ÛÙÔÓ

Ó‡ÌÔÓ·, ÙË Ì›ˆÛË ÙÔ˘ ‡‰·ÙÔ˜ ÛÙÔÓ Ó‡ÌÔ-

Ó· 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¿ ›‰· 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¢Ìfi-

ÓˆÓ, ¤‰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 ÂÈ‚›ˆÛË ÙˆÓ ÂÍ·ÈÚÂÙÈο

ÚfiˆÚˆÓ ÓÂÔÁÓÒÓ, ˘‹Ú¯Â Ì›· Ù¿ÛË ÁÈ· ÚÒÈ-

ÌË ¯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›· ÏË-

ıÒÚ· 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 ÌÂÏÂ-

ÙÒÓ, Ë ‰ÂÍ·ÌÂı·˙fiÓË ¤¯ÂÈ Á›ÓÂÈ ¤Ó· ·fi Ù· ÈÔ

·ÌÊÈÏÂÁfiÌÂÓ· Ê¿Ú̷η ÛÙȘ Û‡Á¯ÚÔÓ˜ ÌÔÓ¿-

‰Â˜ ÂÓÙ·ÙÈ΋˜ ÓÔÛËÏ›·˜ ÓÂÔÁÓÒÓ.

¶ÂÈÚ·Ì·ÙÈο ‰Â‰Ô̤ӷ

ªÂϤÙ˜ Û ÂÈÚ·Ì·Ùfi˙ˆ· ¤‰ÂÈÍ·Ó Èı·Ó¤˜

‰˘ÛÌÂÓ›˜ Ì·ÎÚÔÚfiıÂÛ̘ ÂȉڿÛÂȘ Ù˘ ¯Ô-

Ú‹ÁËÛ˘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙÔÓ ·Ó·Ù˘ÛÛfi-

ÌÂÓÔ ÂÁΤʷÏÔ ·fi ÙÔ 1968. ∆· Â˘Ú‹Ì·Ù· ·˘Ù¿

ÂÚÈÂÏ¿Ì‚·Ó·Ó Ì›ˆÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜, ÙÔ˘ ‚¿-ÚÔ˘˜ Î·È ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ DNA ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ÛÂÂÓ‹ÏÈη ÔÓÙ›ÎÈ·, ÛÙ· ÔÔ›· ›¯·Ó ¯ÔÚËÁËı›ÎÔÚÙÈÎÔÂȉ‹ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô (5).

∞ÚÁfiÙÂÚ·, ¿ÏϘ ÂÈÚ·Ì·ÙÈΤ˜ ÌÂϤÙ˜ ›-Û˘ Û ÔÓÙ›ÎÈ·, ¤‰ÂÈÍ·Ó ‚Ï¿‚Ë ÛÙË Ì˘ÂÏÈÓÔ-Ô›ËÛË, ‰È·›ÚÂÛË Î·È ‰È·ÊÔÚÔÔ›ËÛË ÙÔ˘ Ó¢-ÚÈÎÔ‡ ΢ÙÙ¿ÚÔ˘ Î·È Î·ı˘ÛÙ¤ÚËÛË ÛÙË Û˘Ó·-ÙÔÁ¤ÓÂÛË ÛÙÔÓ ÊÏÔÈfi, ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛËÎÔÚÙÈÎÔÂȉÒÓ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô (6,7).

¶ÚfiÛÊ·Ù·, Û ÂÈÚ·Ì·ÙÈΤ˜ ÌÂϤÙ˜, fiÔ˘ÂÍÂÙ¿ÛÙËÎÂ Ë Ó¢ÚÔÙÔÍÈÎfiÙËÙ· ÙˆÓ ÎÔÚÙÈÎÔ-ÛÙÂÚÔÂȉÒÓ, ·Ú·ÙËÚ‹ıËΠfiÙÈ Ë ¤ÎıÂÛË ÓÂÔ-Á¤ÓÓËÙˆÓ ÔÓÙÈÎÒÓ Û ˘„ËϤ˜ ‰fiÛÂȘ ‰ÂÍ·ÌÂ-ı·˙fiÓ˘ ÚÔ‹Á·Á ÙÔÓ ı¿Ó·ÙÔ ÙÔ˘ Ó¢ÚÒÓ· ÌÂÙÔÓ Ì˯·ÓÈÛÌfi Ù˘ ·fiÙˆÛ˘ (8).

∂Ӊ›ÍÂȘ ÛÙÔ ÓÂÔÁÓfi

ªÂϤÙ˜ Û ÓÂÔÁÓ¿ ¤‰ÂÈÍ·Ó ÙËÓ Â›‰Ú·ÛËÙ˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÛÙËÓ ·‡ÍËÛË Ù˘ ÂÚÈ̤-ÙÚÔ˘ ÎÂÊ·Ï‹˜, ÛÙÔÓ fiÁÎÔ ÙÔ˘ ·Ó·Ù˘ÛÛfiÌÂÓÔ˘ÂÁÎÂÊ¿ÏÔ˘ Î·È Û ‚Ï¿‚˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, fiˆ˜Ê·›ÓÔÓÙ·È ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÂÁÎÂÊ¿ÏÔ˘.

∆ÚÂȘ ÌÂϤÙ˜ (9,10,21) ¤‰ÂÈÍ·Ó ÌÂȈ̤ÓË·‡ÍËÛË Ù˘ ÂÚÈ̤ÙÚÔ˘ ÎÂÊ·Ï‹˜ ÌÂÙ¿ ¯ÔÚ‹ÁË-ÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô.

∂ÈϤÔÓ, 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 Ù˯ڋÛË Ù˘ ÔÛÔÙÈ΋˜ ÔÁÎÔÌÂÙÚÈ΋˜ ÙÚÈۉȿ-ÛÙ·Ù˘ Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ ÛÙËÓ ÙÂÏÂÈfi-ÌËÓË ˆÚÈÌfiÙËÙ·, ·Ú·ÙËÚ‹ıËΠÛËÌ·ÓÙÈ΋›وÛË Ù˘ ÂÓ‰ÔÊϤ‚È·˜ ¯ÔÚ‹ÁËÛ˘ ‰ÂÍ·ÌÂ-ı·˙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·ÓÌÂȈ̤ÓÔ˜ ηٿ 35% Î·È Ô ÔÏÈÎfi˜ fiÁÎÔ˜ ÙÔ˘ÂÁÎÂÊ¿ÏÔ˘ ‹Ù·Ó ÌÂȈ̤ÓÔ˜ ηٿ 30% Û Úfiˆ-Ú· ÓÂÔÁÓ¿ ÛÙ· ÔÔ›· ›¯Â ¯ÔÚËÁËı› ‰ÂÍ·ÌÂı·-˙fiÓË Û ۇÁÎÚÈÛË Ì ÚfiˆÚ· ÓÂÔÁÓ¿ Ô˘ ‰ÂÓ›¯·Ó Ï¿‚ÂÈ ‰ÂÍ·ÌÂı·˙fiÓË, ηıÒ˜ Î·È Ì ÙÂÏÂÈ-fiÌËÓ· ÓÂÔÁÓ¿ Ô˘ ÂÏ‹ÊıËÛ·Ó ˆ˜ Ì¿ÚÙ˘Ú˜.

∫ÏÈÓÈΤ˜ ÌÂϤÙ˜

∏ ÚÒÙË ÎÏÈÓÈ΋ ÌÂϤÙË ÛÙËÓ ÔÔ›· ‰È·È-ÛÙÒıËÎÂ Ë ‚Ï·ÙÈ΋ ›‰Ú·ÛË Ù˘ ‰ÂÍ·ÌÂı·-˙fiÓ˘ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË, ‰ËÌÔÛÈ-‡ıËΠÌfiÏȘ ÙÔ 1998. ™ÙË ÌÂϤÙË ÙˆÓ Yeh ηÈ

¶·È‰È·ÙÚÈ΋ 2005;68:377-384

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379∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Î·È ÓÂÔÁÓÈÎfi˜ ÂÁΤʷÏÔ˜

Û˘Ó (14), ¯ÔÚËÁ‹ıËΠ‰ÂÍ·ÌÂı·˙fiÓË ÛÙ· ÓÂÔ-

ÁÓ¿ 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˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤ-

Ù˜ ÙˆÓ O’Shea Î·È Û˘Ó (15) Î·È Shinwell Î·È Û˘Ó

(12) ¤‰ÂÈÍ·Ó ÙȘ ‰˘ÛÌÂÓ›˜ ÂÈÙÒÛÂȘ Ù˘ ‰Â-

Í·ÌÂı·˙fiÓ˘ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË.

™ÙË ÌÂϤÙË ÙˆÓ O’Shea Î·È Û˘Ó (15), ¯ÔÚË-

Á‹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·˙fi-

Ó˘ 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·˙fi-

Ó˘ 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 ‰Â-

Í·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¿Ú-

Ù˘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¿ÊË-

Ì· 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‹ıË-

Π‰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·ÎÚÔÚfiıÂ-

Û̘ ÂÈÙÒÛÂȘ ·fi ÙË ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·-

˙fiÓ˘ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô. OÈ Romagnoli ηÈ

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˯Ô-

ÁÚ¿ÊËÌ· ÂÁÎÂÊ¿ÏÔ˘. OÈ ‰‡Ô ÙÂÏÂ˘Ù·›Â˜ ÌÂϤ-

Ù˜ 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 ÂÈÙÒÛÂȘ ·fi ÙË ¯Ô-

Ú‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ ηٿ ÙË ÓÂÔÁÓÈ΋ Â-

Ú›Ô‰Ô Û ·È‰È¿ ËÏÈΛ·˜ 2 ÂÙÒÓ, Û ̛· Úfi-

ÛÊ·ÙË ÌÂϤÙË ÂÎÙ›ÌËÛ ٷ ›‰È· ·È‰È¿ Û ۯÔ-

ÏÈ΋ ËÏÈΛ· (21). ∏ Û˘¯ÓfiÙËÙ· ·ıÔÏÔÁÈÎÒÓ

Ó¢ÚÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ ÛÙË ÌÂϤÙË ·˘Ù‹

‹Ù·Ó ·˘ÍË̤ÓË ÛÙËÓ ÔÌ¿‰· Ù˘ ‰ÂÍ·ÌÂı·˙fi-

Ó˘ Û ۯ¤ÛË Ì ÙÔ˘˜ Ì¿ÚÙ˘Ú˜. ∆· ·È‰È¿ ÛÙ·

ÔÔ›· ›¯Â ¯ÔÚËÁËı› ‰ÂÍ·ÌÂı·˙fiÓË Â›¯·Ó ¯ÂÈ-

ÚfiÙÂÚË 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˜. ∂ÈϤÔÓ, ›-

¯·Ó ¯ÂÈÚfiÙÂÚÔ Û˘ÓÙÔÓÈÛÌfi ÙˆÓ ÎÈÓ‹ÛˆÓ, ÔÙÈ-

΋ ·ÓÙ›ÏË„Ë Î·È ÔÙÈÎÔÎÈÓËÙÈÎfi Û˘ÓÙÔÓÈÛÌfi,

ηıÒ˜ Î·È ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚÔ ÔÏÈÎfi ÓÔËÙÈ-

Îfi 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› ÛÂ

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˘ ÎÂ-

Ê·Ï‹˜ (21).

∞Ó·ÛÎÔ‹ÛÂȘ Ù˘¯·ÈÔÔÈËÌ¤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 ÂÈÙÒÛÂˆÓ Ù˘ ‰ÂÍ·-

ÌÂı·˙fiÓ˘ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË,

Ú·ÁÌ·ÙÔÔ›ËÛ ̛· Û˘ÛÙËÌ·ÙÈ΋ ·Ó·ÛÎfiË-

ÛË ÙˆÓ Ù˘¯·ÈÔÔÈËÌ¤ÓˆÓ ÌÂÏÂÙÒÓ Ô˘ ›¯·Ó

‰ËÌÔÛÈ¢ı› ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·. ∆Ô ·ÔÙ¤ÏÂÛÌ·

Ù˘ ÌÂÙ·-·Ó¿Ï˘Û˘ ¤‰ÂÈÍ fiÙÈ Ë ıÂڷ›· ÌÂ

ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô

¶·È‰È·ÙÚÈ΋ 2005;68:377-384

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380 ∂. ªÔ‡˙· Î·È Û˘Ó.

Û¯ÂÙ›˙ÂÙ·È Ì ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË Ù˘ Û˘¯ÓfiÙË-

Ù·˜ Ù˘ ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘ Î·È Ù˘ „˘¯Ô-

ÎÈÓËÙÈ΋˜ ηı˘ÛÙ¤ÚËÛ˘.

O Û¯ÂÙÈÎfi˜ ΛӉ˘ÓÔ˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË

ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿Ï˘Û˘ Ô˘ ÂÍ¿ÁÂÙ·È ·fi ÌÂ-

ϤÙ˜ Ì contamination <30% Â›Ó·È 2,86 (95%

CI 1,95, 4,19). O Û¯ÂÙÈÎfi˜ ΛӉ˘ÓÔ˜ ÁÈ· ÙËÓ ÂÌ-

Ê¿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 Úfiˆ-

ÚˆÓ ÓÂÔÁÓÒÓ, ÛÙ· ÔÔ›· Ú¤ÂÈ Ó· ¯ÔÚËÁËı›

ıÂڷ¢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˘ÌÂ

¤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 Úfi-

ÏË„Ë Î·È ıÂڷ›· Ù˘ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ‰˘-

ÛÏ·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·ÛÎfiËÛ·Ó 37 Ù˘¯·È-

ÔÔÈË̤Ó˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ÂÓ‰ÔÊϤ‚È·˜ ¯Ô-

Ú‹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‹ıË-

Π‚Ú·¯˘ÚfiıÂÛÌË ‚ÂÏÙ›ˆÛË Ù˘ ·ÓÙ·ÏÏ·Á‹˜

ÙˆÓ ·ÂÚ›ˆÓ Î·È Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ Ó¢Ìfi-

ÓˆÓ 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¤ıËΠ̛ˆ-

ÛË Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙË

“ÓˆÚ›˜” Î·È ÙË “Û¯ÂÙÈο ÓˆÚ›˜” ıÂڷ›·. ∆¤-

ÏÔ˜, ÛËÌÂÈÒıËΠ̛ˆÛË Ù˘ ÓÂÔÁÓÈ΋˜ ıÓËÛÈ-

ÌfiÙËÙ·˜ ÌfiÓÔ ÛÙË Û¯ÂÙÈο ÓˆÚ›˜ ¯ÔÚ‹ÁËÛË ÌÂ

¤Ó·Ó ÂÈϤÔÓ ÂÈ˙ÒÓÙ· ÁÈ· οı 16 ÓÂÔÁÓ¿

ÛÙ· ÔÔ›· ¯ÔÚËÁ‹ıËΠ‰ÂÍ·ÌÂı·˙fiÓË. ∏ ‰Â-

Í·ÌÂı·˙fiÓË Ô˘ ¯ÔÚËÁÂ›Ù·È Â›Ù ӈڛ˜, ›ÙÂ

·ÚÁ¿ ‰ÂÓ ÂËÚ¿˙ÂÈ ÙËÓ ÂÈ‚›ˆÛË. ÕÌÂÛ˜ ÂÈ-

ÏÔΤ˜, fiˆ˜ ·‡ÍËÛË Ù˘ ·ÈÌÔÚÚ·Á›·˜ ·fi ÙÔ

ÂÙÈÎfi, ‰È¿ÙÚËÛË ÙÔ˘ ÂÓÙ¤ÚÔ˘ (Ô˘ Û˘Ì‚·›-

ÓÂÈ Î˘Ú›ˆ˜ ÛÙË ÓˆÚ›˜ ¯ÔÚ‹ÁËÛË), ˘ÂÚÁÏ˘Î·È-

Ì›·, ˘¤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·ÎÚÔÚfiıÂÛÌ˘ ·ıÔÏÔÁÈ΋˜

Ó¢ÚÔÏÔÁÈ΋˜ ÂͤٷÛ˘ Î·È ÂÁÎÂÊ·ÏÈ΋˜ ·Ú¿-

Ï˘Û˘ ‹Ù·Ó ÛËÌ·ÓÙÈο ·˘ÍË̤ÓÔ˜, ·Ó Î·È Ë ÌÂ-

ıÔ‰ÔÏÔÁ›· ÛÙȘ ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ ‰ÂÓ ‹Ù·Ó ¿-

ÓÙ· ÈηÓÔÔÈËÙÈ΋ Î·È Ù· ·È‰È¿ ÂÎÙÈÌ‹ıËηÓ

΢ڛˆ˜ ÚÈÓ ·fi ÙË Û¯ÔÏÈ΋ ËÏÈΛ·.

§fiÁˆ ÙÔ˘ ·˘ÍË̤ÓÔ˘ ÎÈÓ‰‡ÓÔ˘ ÁÈ· Èı·Ó¤˜

‚Ú·¯˘ÚfiıÂÛ̘ Î·È Ì·ÎÚÔÚfiıÂÛ̘ ÂÈ-

ÏÔΤ˜ Û ۯ¤ÛË Ì ÙÔ Èı·Ófi ‚Ú·¯˘ÚfiıÂ-

ÛÌÔ fiÊÂÏÔ˜, ÔÈ Halliday Î·È Û˘Ó Û˘ÓÈÛÙÔ‡Ó fiÙÈ

Ë ‰ÂÍ·ÌÂı·˙fiÓË ‰ÂÓ Ú¤ÂÈ ÔÙ¤ Ó· ¯ÔÚËÁ›-

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˘ Ë̤-

Ú·˜ Î·È Ú¤ÂÈ Ó· ‰È·Ê˘Ï¿ÛÛÂÙ·È ÌfiÓÔ ÁÈ· ÓÂ-

ÔÁÓ¿ Ô˘ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ·Ô‰ÂÛÌ¢ÙÔ‡Ó ·fi

ÙÔÓ ·Ó·Ó¢ÛÙ‹Ú·, ÂÓÒ Ë ‰fiÛË Î·È Ë ‰È¿ÚÎÂÈ·

Ù˘ ıÂڷ›·˜ Ú¤ÂÈ Ó· Â›Ó·È ÔÈ ÂÏ¿¯ÈÛÙ˜

‰˘Ó·Ù¤˜.

¶ÚfiÛÊ·Ù·, Ë American Academy of Pedia-

trics Î·È Ë Canadian Pediatric Society (26) ·Ó·-

ÛÎfiËÛ·Ó ÙȘ ÂӉ›ÍÂȘ Ù˘ ›وÛ˘ Ù˘ ¯Ô-

Ú‹ÁËÛ˘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Î·Ù¿ ÙË ÓÂÔÁÓÈ΋

ÂÚ›Ô‰Ô - Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ ÙˆÓ ·ÒÙÂ-

ÚˆÓ ÂÈÏÔÎÒÓ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈ-

ÍË - 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·˙fi-

Ó˘ ÁÈ· ÙËÓ ÚfiÏË„Ë ‹ ÙË ıÂڷ›· Ù˘ ¯Úfi-

ÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ Ú¤ÂÈ Ó· ÂÚÈÔÚ›-

˙ÂÙ·È Û ηϿ ۯ‰ȷṲ̂Ó˜ Ù˘¯·ÈÔÔÈË̤Ó˜

ÌÂϤÙ˜ Ì ̷ÎÚÔ¯ÚfiÓÈ· ·Ú·ÎÔÏÔ‡ıËÛË.

Á) ∏ Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂÎÙ›ÌËÛË ÙˆÓ ÓÂÔ-

ÁÓÒÓ Ô˘ Û˘ÌÌÂÙ›¯·Ó ÛÂ Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂ-

ϤÙ˜ ‰ÂÍ·ÌÂı·˙fiÓ˘ ÂÓı·ÚÚ‡ÓÂÙ·È ıÂÚÌ¿.

‰) ∂›Ó·È ·Ó·Áη›Ô Ó· Á›ÓÔ˘Ó ÎÏÈÓÈΤ˜ Ù˘¯·È-

ÔÔÈË̤Ó˜ ÌÂϤÙ˜ Ì ¿ÏÏ· ÂÓ‰ÔÊϤ‚È· ‹ ÂÈ-

ÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹.

Â) ∂ÎÙfi˜ ·fi ÙȘ Ù˘¯·ÈÔÔÈË̤Ó˜ 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· ÂÓËÌÂÚÒ-

ÓÔÓÙ·È Ï‹Úˆ˜ Î·È Ó· Û˘ÌʈÓÔ‡Ó ÁÈ· ÙË ıÂ-

ڷ›·.

∂Ó·ÏÏ·ÎÙÈο ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹

Ÿˆ˜ ·Ó·Ê¤ÚıËÎÂ, ˘¿Ú¯Ô˘Ó ÈÛ¯˘Ú¤˜ ÂÓ-

‰Â›ÍÂȘ fiÙÈ ÛÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘

ÙˆÓ Ó¢ÌfiÓˆÓ Û˘ÌÌÂÙ¤¯Ô˘Ó ÊÏÂÁÌÔÓÒ‰ÂȘ

¶·È‰È·ÙÚÈ΋ 2005;68:377-384

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381∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Î·È ÓÂÔÁÓÈÎfi˜ ÂÁΤʷÏÔ˜

·Ú¿ÁÔÓÙ˜. ø˜ ÂÎ ÙÔ‡ÙÔ˘, Ë ÂÓ‰ÂÈÎÓ˘fiÌÂÓË

ıÂڷ›· Â›Ó·È Ù· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹. ∏ ‰ÂÍ·ÌÂ-

ı·˙fiÓË Â›Ó·È ¤Ó· ÈÛ¯˘Úfi ·ÓÙÈÊÏÂÁÌÔÓ҉˜ ÎÔÚ-

ÙÈÎÔÂȉ¤˜. OÈ Ê·ÚÌ·ÎÔÏÔÁÈΤ˜ ‰fiÛÂȘ Ô˘ Û˘-

¯Ó¿ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙȘ ÌÂϤÙ˜ Î·È ÛÙËÓ

ηı’ Ë̤ڷ Ú¿ÍË Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ·fi 10-

15 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚ˜ Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ËÌÂ-

Ú‹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¿ ›‰· ‰ÂÍ·ÌÂı·˙fi-

Ó˘ ÌÔÚ› Ó· ·˘Í¿ÓÔ˘Ó ÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÈ-

ÏÔÎÒÓ.

∂›Ó·È ÁÓˆÛÙfi fiÙÈ Ù· ‰È¿ÊÔÚ· ÌfiÚÈ· ÙˆÓ ÎÔÚ-

ÙÈÎÔÛÙÂÚÔÂȉÒÓ ¤¯Ô˘Ó ‰È·ÊÔÚÂÙÈ΋ ‰Ú¿ÛË. ∏

‰È·ÊÔÚÂÙÈ΋ ‰Ú¿ÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ

ÂÍËÁÂ›Ù·È Ì ÙË ıˆڛ· Ù˘ ÁÔÓȉȷ΋˜ - ÌË ÁÔ-

Óȉȷ΋˜ ‰Ú¿Û˘. ∂›Û˘, ÔÈ ‰È·ÊÔÚÂÙÈΤ˜ ÂÈ-

ÙÒÛÂȘ ÙˆÓ ‰È·ÊfiÚˆÓ ÎÔÚÙÈÎÔÂȉÒÓ ÛÙÔÓ

·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ ¤¯Ô˘Ó ‰Âȯı› ÂÈ-

Ú·Ì·ÙÈο.

¶ÈÛÙ‡ÂÙ·È fiÙÈ Ù· ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ‹ ‰ÚÔ˘Ó

̤ۈ ÁÔÓȉȷÎÒÓ - ÌË ÁÔÓȉȷÎÒÓ Ì˯·ÓÈÛÌÒÓ

(27). ªÂ ÙÔ˘˜ ÎÏ·ÛÈÎÔ‡˜ ÁÔÓȉȷÎÔ‡˜ Ì˯·ÓÈ-

ÛÌÔ‡˜, Ù· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ÂÚÓÔ‡Ó Ì¤Û· ·fi

ÙË ÌÂÌ‚Ú¿ÓË ÙÔ˘ ΢ÙÙ¿ÚÔ˘, Û˘Ó‰¤ÔÓÙ·È Û ÂȉÈ-

Τ˜ ı¤ÛÂȘ Ì ÙÔ DNA, ·ÏÏËÏÂȉÚÔ‡Ó Ì ˘ÚË-

ÓÈÎÔ‡˜ ·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 ÁÔ-

ÓȉȷÎÒÓ Ì˯·ÓÈÛÌÒÓ, ¯ÚÂÈ¿˙ÔÓÙ·È ÙÔ˘Ï¿¯È-

ÛÙÔÓ 30 min Î·È Û˘¯Ó¿ ·ÚÎÂÙ¤˜ ÒÚ˜ ÁÈ·

Ó· ·Ú¯›ÛÔ˘Ó Ó· ‰ÚÔ˘Ó. ªÂ ÙÔ˘˜ ÌË ÁÔÓȉȷ-

ÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜, Ë ‰Ú¿ÛË ÙÔ˘˜ ·Ú¯›˙ÂÈ Ôχ

ÈÔ ÁÚ‹ÁÔÚ·, ̤۷ Û ‰Â˘ÙÂÚfiÏÂÙ· ¤ˆ˜ Ï›Á·

ÏÂÙ¿. ∆· ÎÔÚÙÈÎÔÂȉ‹ ÌÂÙ·‚¿ÏÏÔ˘Ó ÙȘ ȉÈfi-

ÙËÙ˜ ÙˆÓ ÌÂÌ‚Ú·ÓÒÓ Î·È ·Ó·ÛÙ¤ÏÏÔ˘Ó ÙËÓ

›ÛÔ‰Ô Na Î·È Ca ̤ۈ Ù˘ ÌÂÌ‚Ú¿Ó˘, Ô˘

¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË Ì›ˆÛË ÙÔ˘ ÂÓ‰Ô΢Ù-

Ù¿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˜ ̄ ÚËÛÈÌÔÔÈÔ‡ÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂÈ-

‰‹, ÙË ÌÂÁ·Ï‡ÙÂÚË Û¯ÂÙÈ΋ ÈÛ¯‡ - Û ۯ¤ÛË ÌÂ

·˘Ù‹ Ù˘ Ú‰ÓÈ˙ÔÏfiÓ˘ - ˆ˜ ÚÔ˜ ÙË ÁÔÓȉȷ΋

‰Ú¿ÛË, ¤¯Ô˘Ó Ë ‰ÂÍ·ÌÂı·˙fiÓË Î·È Ë ‚ËÙ·ÌÂı·-

˙fiÓË, Ë ÔÔ›· 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·˙fi-

ÓË, Ë ÔÔ›· Â›Ó·È 2,9 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË, ÂÓÒ ÙË

ÌÈÎÚfiÙÂÚË ¤¯ÂÈ Ë ‚ËÙ·ÌÂı·˙fiÓË, Ë ÔÔ›· ›ӷÈ

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·

¯ÚËÛÈÌÔÔÈËıÔ‡Ó. °È· ÂÓ‰ÔÊϤ‚È· ıÂڷ›· ÌÂ

ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Û ÌÂÁ¿Ï˜ ‰fiÛÂȘ ıˆÚËÙÈ-

ο Â›Ó·È ÚÔÙÈÌfiÙÂÚË Ì›· ÈÛÔÚÚÔ›· ÌÂٷ͇ ÁÔ-

Óȉȷ΋˜ - ÌË ÁÔÓȉȷ΋˜ ‰Ú¿Û˘. ÀÂÈÛ¤Ú¯Ô-

ÓÙ·È, fï˜, ·Ú¿ÁÔÓÙ˜ fiˆ˜ Ë ÂÈı˘ÌËÙ‹ Ù·-

¯‡ÙËÙ· ‰Ú¿Û˘, ÔfiÙ ÚÔÙÈÌÒÓÙ·È Ô˘Û›Â˜ ÌÂ

ÈÛ¯˘ÚfiÙÂÚË ÌË ÁÔÓȉȷ΋ ‰Ú¿ÛË ‹ ÔÈ ÂÈÏÔΤ˜

ÙˆÓ ‰È·ÊfiÚˆÓ Ô˘ÛÈÒÓ.

ÕÏÏÔ˜ ÚÔ‚ÏËÌ·ÙÈÛÌfi˜ Û¯ÂÙÈο Ì ÙËÓ ÂÈ-

ÏÔÁ‹ ÙÔ˘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉԇ˜ Ô˘ ı· ¯ÚËÛÈÌÔ-

ÔÈËı›, ˘‹ÚÍ ÌÂÙ¿ ÙË ÌÂϤÙË ÙˆÓ Baud ηÈ

Û˘Ó (8), fiÛÔÓ ·ÊÔÚ¿ ÛÙË Ó¢ÚÔÙÔÍÈÎfiÙËÙ· ÙˆÓ

‰È·ÊfiÚˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙÔÓ ·Ó·Ù˘Û-

ÛfiÌÂÓÔ ÂÁΤʷÏÔ ÙÔ˘ ÔÓÙÈÎÔ‡.

™ÙȘ in vitro ÌÂϤÙ˜ ηÏÏÈÂÚÁ‹ıËÎ·Ó Ó¢-

ÚÒÓ˜ ÙÔ˘ ÊÏÔÈÔ‡ ·fi ¤Ì‚Ú˘· ÔÓÙ›ÎÈ·, η-

ıÒ˜ Î·È ·ÛÙÚÔ·ÙÙ·Ú· ·fi ÓÂÔÁ¤ÓÓËÙ· ÔÓÙ›-

ÎÈ·, Û ˘ÏÈÎfi Ô˘ ÂÚÈ›¯Â Û··ÛÌ· Ù˘ ‰Â-

Í·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‹ıË-

Î·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 ÂËÚ·˙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· ÂÈÙ¿¯˘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˘ ÚÔη-

ÏÔ‡ÓÙ·Ó ·fi Ó¢ÚÔÙÔÍÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜

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 ÛË-

Ì·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‰ÔÂÚÈÙÔÓ·˚ο Û ÓÂÔ-

Á¤ÓÓËÙ· ÔÓÙ›ÎÈ·. ªÂ ÂȉÈΤ˜ Ù¯ÓÈΤ˜ ‰È·›-

ÛÙˆÛ·Ó ÙÔÓ ı¿Ó·ÙÔ ÙˆÓ Î˘ÙÙ¿ÚˆÓ Ì ÙÔÓ ÌË-

¯·ÓÈÛÌfi Ù˘ ·fiÙˆÛ˘ ÛÙÔÓ ÂÁΤʷÏÔ ÙˆÓ

¶·È‰È·ÙÚÈ΋ 2005;68:377-384

Pediatr Sept-Oct 05 17-10-05 16:52 ™ÂÏ›‰·381

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

382 ∂. ªÔ‡˙· 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· ÔÔ›· ›¯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

ÂËÚ¤·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‡˜ ›‰Ú·Û˘ Ù˘ ‰Â-

Í·ÌÂı·˙fiÓ˘ ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ,

¤¯Ô˘Ó ÚÔÙ·ı› ÂÓ·ÏÏ·ÎÙÈο ÌfiÚÈ· ÎÔÚÙÈÎÔ-

ÛÙÂÚÔÂȉÒÓ, Ù· ÔÔ›· ¤¯Ô˘Ó ¯ÔÚËÁËı› Û˘ÛÙË-

Ì·ÙÈο ‹ Ì ÂÈÛÓÔ¤˜.

¶Èı·Ó¿ ÂÓ·ÏÏ·ÎÙÈο ÛÙÂÚÔÂȉ‹, Ô˘ ¤¯Ô˘Ó

¯ÚËÛÈÌÔÔÈËı› Û˘ÛÙËÌ·ÙÈο, Â›Ó·È Ë ÌÂı˘Ï-

Ú‰ÓÈ˙ÔÏfiÓË, Ë ˘‰ÚÔÎÔÚÙÈ˙fiÓË Î·È Ë ‚ËÙ·-

ÌÂı·˙fiÓË.

∏ ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfiÓË ¤¯ÂÈ ¯ÔÚËÁËı› ˆ˜

ıÂڷ›· ÁÈ· Ó· ÂÈÙ·¯˘Óı› Ë ·Ô‰¤ÛÌ¢ÛË

·fi ÙÔÓ ·Ó·Ó¢ÛÙ‹Ú·. ∏ ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfi-

ÓË 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 ¯ÚËÛÈÌÔÔÈËı› Ì ۯÂÙÈ΋

·ÛÊ¿ÏÂÈ· (28).

÷ÌËϤ˜ ‰fiÛÂȘ ˘‰ÚÔÎÔÚÙÈ˙fiÓ˘ ¯ÔÚËÁ‹-

ıËÎ·Ó Û ÓÂÔÁÓ¿ ÙȘ ÚÒÙ˜ Ë̤Ú˜ ˙ˆ‹˜ ÁÈ·

ÙËÓ ÚfiÏË„Ë Ù˘ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ‰˘-

ÛÏ·Û›·˜. ªÂÙ¿ ·fi ¯·ÌËϤ˜ ‰fiÛÂȘ ˘‰ÚÔÎÔÚ-

ÙÈ˙fiÓ˘ (1 mg/kg/Ë̤ڷ) ‚Ú¤ıËΠfiÙÈ ÛÙ· ÓÂÔ-

ÁÓ¿ ·˘Ù¿ ·˘Í‹ıËΠηٿ ÙȘ 2 ÚÒÙ˜ ‚‰ÔÌ¿-

‰Â˜ ˙ˆ‹˜ Ë ÂÈ‚›ˆÛË ¯ˆÚ›˜ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋

‰˘ÛÏ·Û›·, ÂÓÒ ‰ÂÓ ˘‹Ú¯·Ó ¿ÌÂÛ˜ ‹ ·ÒÙÂ-

Ú˜ ÂÈÏÔΤ˜ (2).

∏ ‚ËÙ·ÌÂı·˙fiÓË Èı·ÓÒ˜ Â›Ó·È ·ÛʷϤÛÙÂ-

ÚË ·fi ÙË ‰ÂÍ·ÌÂı·˙fiÓË. ¶ÂÈÚ¿Ì·Ù· Û ÔÓÙ›-

ÎÈ· Î·È Ë ÂÌÂÈÚ›· ·fi ÙËÓ ÚÔÁÂÓÓËÙÈ΋ ¯ÔÚ‹-

ÁËÛ‹ Ù˘, ηıÈÛÙÔ‡Ó ÙË ‚ËÙ·ÌÂı·˙fiÓË ›Ûˆ˜ ÙÔ

ȉ·ÓÈÎfi ÂÓ·ÏÏ·ÎÙÈÎfi ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ¤˜ ·ÓÙ›

ÁÈ· ÙË ‰ÂÍ·ÌÂı·˙fiÓË, ·ÏÏ¿ ‰ÂÓ ˘¿Ú¯Ô˘Ó ·ÚÎÂ-

Ù¤˜ Û˘ÁÎÚÈÙÈΤ˜ ÌÂϤÙ˜ ÛÙ· ÓÂÔÁÓ¿. ™Â Ì›· ÌÈ-

ÎÚ‹ Ù˘¯·ÈÔÔÈË̤ÓË 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›˜ ·ÓÂÈı‡-

ÌËÙ˜ ¿ÌÂÛ˜ ÂÈÏÔΤ˜ (29) ‹ ·ÒÙÂÚ˜ ÂÈ-

ÙÒÛÂȘ ÛÙË Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË ÛÙËÓ

ËÏÈΛ· ÙˆÓ 12 ÌËÓÒÓ (30). ¶·Ú’ fiÏ· ·˘Ù¿, Ë ›‰È·

ÌÂϤÙË ‰ÂÓ ¤‰ÂÈÍ ·˘ÍË̤Ó˜ ·ÒÙÂÚ˜ ‰˘ÛÌÂ-Ó›˜ ÂÈÙÒÛÂȘ Ô‡Ù ÛÙËÓ ÔÌ¿‰· ÙˆÓ ÓÂÔÁÓÒÓÛÙ· ÔÔ›· ¯ÔÚËÁ‹ıËΠ‰ÂÍ·ÌÂı·˙fiÓË.

¶ÚfiÛÊ·Ù·, Ù· ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔ-Âȉ‹ ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙË ıÂڷ›· ‹ÙËÓ ÚfiÏË„Ë Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÙˆÓ Ó¢Ìfi-ÓˆÓ, ÂÂȉ‹ ıˆÚÂ›Ù·È fiÙÈ Ë ÙÔÈ΋ ‰Ú¿ÛË Û˘Ó-‰¤ÂÙ·È Ì ÏÈÁfiÙÂÚ˜ ÂÈÏÔΤ˜.

ª›· ÚfiÛÊ·ÙË ·Ó·ÛÎfiËÛË ÙˆÓ ÌÂÏÂÙÒÓÙˆÓ ÂÈÛÓÂfiÌÂÓˆÓ 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·-ÈÛÙÒıËΠ̷ÎÚÔÚfiıÂÛÌ· fiÊÂÏÔ˜ ˆ˜ ÚÔ˜ÙËÓ ÂÈ‚›ˆÛË ‹ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· ¯ÚfiÓÈ· ÓfiÛÔÙÔ˘ Ó‡ÌÔÓ· (31). ∆· ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔ-ÛÙÂÚÔÂȉ‹ Û˘Óԉ‡ÔÓÙ·È ·fi ÏÈÁfiÙÂÚ˜ ·ÚÂ-Ó¤ÚÁÂȘ (32), ·Ó Î·È ‰ÂÓ ˘¿Ú¯Ô˘Ó ·ÚÎÂÙ¤˜ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Ô˘ Ó· ÂϤÁ¯Ô˘Ó ÙȘ Ì·ÎÚÔ-ÚfiıÂÛ̘ ÂÈÙÒÛÂȘ ÙÔ˘˜. ŒÙÛÈ, Ê·›ÓÂÙ·È fiÙÈÙ· ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ÌÔÚ› Ó·‚ÔËıÔ‡Ó Û ‹È˜ ‹ ̤ÙÚȘ ÂÚÈÙÒÛÂȘ ¯Úfi-ÓÈ·˜ ·Ó·Ó¢ÛÙÈ΋˜ ÓfiÛÔ˘, ·ÏÏ¿ ÛÙȘ ‚·ÚȤ˜ÂÚÈÙÒÛÂȘ Â›Ó·È ··Ú·›ÙËÙ· Ù· ÂÓ‰ÔÊϤ‚È·ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹.

™˘ÌÂÚ¿ÛÌ·Ù·

∆· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ¤¯Ô˘Ó ¢ÓÔ˚΋ ‰Ú¿ÛËÛÙË ¯ÚfiÓÈ· ·Ó·Ó¢ÛÙÈ΋ ÓfiÛÔ, ·ÏÏ¿ Ë ‰ÂÍ·-ÌÂı·˙fiÓË Û˘Ó‰¤ÂÙ·È Ì ÛÔ‚·Ú¤˜ ÂÈÏÔΤ˜.

°›ÓÔÓÙ·È Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ ÁÈ· Ó·‚ÚÂı› ÔÈÔ Â›Ó·È ÙÔ ÈÔ Î·Ù¿ÏÏËÏÔ ÁÏ˘ÎÔÎÔÚ-ÙÈÎÔÂȉ¤˜ Ô˘ Ú¤ÂÈ Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È Î·ÈÔÈ· Â›Ó·È Ë ÌÈÎÚfiÙÂÚË ‰fiÛË Ô˘ ¤¯ÂÈ ıÂÙÈο‚Ú·¯˘ÚfiıÂÛÌ· ·ÔÙÂϤÛÌ·Ù· Ì ÂÏ¿¯ÈÛÙ··ÚÓËÙÈο Ì·ÎÚÔÚfiıÂÛÌ·.

∏ American Academy of Pediatrics, ËCanadian Pediatric Society Î·È Ë EuropeanAssociation of Perinatal Medicine Û˘ÓÈÛÙÔ‡Ó Ó·ÂÓÙ·¯ıÔ‡Ó Ù· ÓÂÔÁÓ¿ Ô˘ ›¯·Ó Ï¿‚ÂÈ ÎÔÚÙÈÎÔ-ÛÙÂÚÔÂȉ‹ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô, Û˘ÌÌÂÙ¤-¯ÔÓÙ·˜ ÛÂ Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜, Û ÏÂÙÔ-ÌÂÚ‹ Ó¢ÚÔÏÔÁÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË, ÒÛÙ ӷÂÎÙÈÌËı› Ë Ú·ÁÌ·ÙÈ΋ Û¯¤ÛË ÎÈÓ‰‡ÓÔ˘ ÚÔ˜fiÊÂÏÔ˜ Ù˘ ıÂڷ›·˜ 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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383∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Î·È ÓÂÔÁÓÈÎfi˜ ÂÁΤʷÏÔ˜

2. Watterberg KL, Gerdes JS, Gifford KL, Lin HM.Prophylaxis against early adrenal insufficiency toprevent chronic lung disease in premature infants.Pediatrics 1999;104:1258-1263.

3. Halliday HL. A review of postnatal corticosteroids fortreatment and prevention of chronic lung disease inthe preterm infant. Prenat Neonat Med 1997; 2:1-12.

4. Bhuta T, Ohlsson A. Systematic review and meta-analysis of early postnatal dexamethasone for pre-vention of chronic lung disease. Arch Dis Child Fe-tal Neonatal Ed 1998;79:F26-F33.

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

6. Howard E, Benjamins JA. DNA, ganglioside and sul-fatide in brains of rats given corticosterone in infan-cy, with an estimate of cell loss during development.Brain Res 1975;92:73-87.

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

8. Baud O, Laudenbach V, Evrard P, Gressens P. Neu-rotoxic effects of fluorinated glucocorticoid prepa-rations on the developing mouse brain: role ofpreservatives. Pediatr Res 2001;50:706-711.

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

10. Romagnoli C, Zecca E, Vento G, Maggio L, PapacciP, Tortorolo G. Effect on growth of two differentdexamethasone courses for preterm infants at risk ofchronic lung disease. A randomized trial. Pharma-cology 1999;59:266-274.

11. Noble-Jamieson CM, Regev R, Silverman M. Dex-amethasone in neonatal chronic lung disease: pul-monary effects and intracranial complications. Eur JPediatr 1989;148:365-367.

12. Shinwell ES, Karplus M, Reich D, Weintraub Z,Blazer S, Yurman S et al. Early postnatal dexametha-sone treatment and increased incidence of cerebralpalsy. Arch Dis Child Fetal Neonatal Ed 2000;83:F177-F181.

13. Murphy BP, Inder TE, Huppi PS, Warfield S, Zien-tara GP, Kikinis R et al. Impaired cerebral corticalgray matter growth after treatment with dexametha-sone for neonatal chronic lung disease. Pediatrics2001;107:217-221.

14. Yeh TF, Lin YJ, Huang CC, Chen YJ, Lin CH, LinHC et al. Early dexamethasone therapy in preterminfants: a follow-up study. Pediatrics 1998;101:E7.

15. O’Shea TM, Kothadia JM, Klinepeter KL, GoldsteinDJ, Jackson BG, Weaver RG 3rd et al. Randomizedplacebo-controlled trial of a 42-day tapering courseof dexamethasone to reduce the duration of ventila-tor dependency in very low birth weight infants:outcome of study participants at 1-year adjustedage. Pediatrics 1999;104:15-21.

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

1998;43:201A. 17. Jones R, Wincott E, Elbourne D, Grant A. Con-

trolled trial of dexamethasone in neonatal chroniclung disease: a 3-year follow-up. Pediatrics 1995;96:897-906.

18. Cummings JJ, D’ Eugenio DB, Gross SJ. A con-trolled trial of dexamethasone in preterm infants athigh risk for bronchopulmonary dysplasia. N Engl JMed 1989;320:1505-1510.

19. Romagnoli C, Zecca E, Luciano R, Torrioli G, Tor-torolo G. Controlled trial of early dexamethasonetreatment for the prevention of chronic lung diseasein preterm infants: a 3-year follow-up. Pediatrics2002;109:e85.

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

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

22. Barrington KJ. The adverse neurodevelopmental ef-fects of postnatal steroids in the preterm infant: asystematic review of RCT’s. BMC Pediatrics2001;1:1. Available at:http://www.biomedcentral.com/1471-2431/1/1

23. Halliday HL, Ehrenkranz RA. Early postnatal (<96hours) corticosteroids for preventing chronic lungdisease in preterm infants (Cochrane review). In:The Cochrane Library. Issue 1. Oxford: Update Soft-ware; 2003.

24. Halliday HL, Ehrenkranz RA. Moderately early (7-14 days) postnatal corticosteroids for preventinglung disease in preterm infants (Cochrane review).In: The Cochrane Library. Issue 1. Oxford: UpdateSoftware; 2003.

25. Halliday HL, Ehrenkranz RA. Delayed (>3 weeks)postnatal corticosteroids for chronic lung disease inpreterm infants (Cochrane review). In: The CochraneLibrary. Issue 1. Oxford: Update Software; 2003.

26. American Academy of Pediatrics, Committee on Fe-tus and Newborn and Canadian Paediatric Society,Fetus and Newborn Committee. Postnatal corticos-teroids to treat or prevent chronic lung disease inpreterm infants. Pediatrics 2002;109:330-338.

27. Lipworth BJ. Therapeutic implications of non-ge-nomic glucocorticoid activity. Lancet 2000;356:87-89.

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

29. Decastro MH, LaGamma EF. Postnatal use of be-tamethasone vs dexamethasone for treatment ofevolving bronchopulmonary dysplasia in extremelylow birth weight neonates. 17th InternationalWorkshop on Surfactant Replacement. Cagliari,Italy; May 2002.

30. Hassan MS, Decastro MH, Dudhblai A, LaGammaEF. Twelve month neurodevelopmental outcomecomparison between postnatal use of betametha-sone vs dexamethasone for the treatment of evolvingBPD in extremely low birth weight infants. PediatrRes 2003;53:479-480A.

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384 ∂. ªÔ‡˙· Î·È Û˘Ó.

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

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

Lagercrantz H. Budesonide delivered by dosimetricjet nebulization to preterm very low birthweight in-fants at high risk for development of chronic lungdisease. Acta Paediatr 2000;89:1449-1455.

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385CURRENT ISSUE

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 lungdisease (bronchopulmonary dysplasia) in preterm infants. Recently, the meta-analyses of theCochrane 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 7-14 days of life seems to have the best benefit to risk ratio. However, since steroids may haveneurotoxic effects on the developing brain, neonatologists must continue to search for alternativetherapies for infants with chronic lung disease. Meanwhile, despite the limitations of steroidtreatment, the European Association of Perinatal Medicine, the American Academy of Pediatrics andthe Canadian Pediatric Society guidelines provide the most realistic and balanced advice on its use forthe clinical neonatologist trying to clarify a confusing situation.

Key words

Corticosteroids, neonate, brain.

2nd Neonatal Intensive CareUnit, “Aghia Sophia”Children’s Hospital, Athens

Correspondence:

Helen Bouza 2nd Neonatal Intensive Care Unit, “Aghia Sophia” Children’sHospital, 115 27, Athens E-mail: [email protected]

Date of submission: 21-06-2004 Date of approval: 17-06-2005

Paediatriki 2005;68:385

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∂ÍÂÙ¿ÛÂȘ DNA, ı¤Ì·Ù· ËıÈ΋˜, ΢ÛÙÈ΋ ›ÓˆÛË, ·ÓÔÛÔÂÓÂÚÁfi˜ ıÚ˘„›ÓË, Ì˘˚΋ ‰˘ÛÙÚÔÊ›·Duchenne.

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∏ ÚfiÛÊ·ÙË ÂÚ¿ÙˆÛË Ù˘ ·Ó¿ÁÓˆÛ˘ ÙÔ˘·ÓıÚÒÈÓÔ˘ ÁÔÓȉÈÒÌ·ÙÔ˜ Î·È ÔÈ Û¯ÂÙÈΤ˜ Úfi-Ô‰ÔÈ Ô˘ ÚÔ·ÙÔ˘Ó ·fi ·˘Ù‹, ·‡ÍËÛ·Ó ÙÔÂӉȷʤÚÔÓ ÛÙË ÁÂÓÂÙÈ΋. ¶·Ú·Ì¤ÓÂÈ, fï˜,Ì›· Û‡Á¯˘ÛË ÌÂٷ͇ ÙˆÓ ÁÈ·ÙÚÒÓ Î·È Ù˘ ÎÔÈ-ÓˆÓ›·˜ fiÛÔÓ ·ÊÔÚ¿ ÛÙÔÓ ÚfiÏÔ Ù˘ ÁÂÓÂÙÈ΋˜ÏËÚÔÊÔÚ›·˜ ÛÙËÓ È·ÙÚÈ΋ Ú¿ÍË (1).

™Â ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ, fiˆ˜ ÛÙËÓ Î˘ÛÙÈ-΋ ›ÓˆÛË (CF) Î·È ÛÙË ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋ Ófi-ÛÔ, ÔÈ Û˘ÁÎÂÎÚÈ̤ÓÔÈ ÌÔÚÈ·ÎÔ› Ì˯·ÓÈÛÌÔ› ›ӷȷÚÎÂÙ¿ ηٷÓÔËÙÔ›. ŸÌˆ˜, Û ·ÚÎÂÙ¿ ¿ÏÏ·¯ÚfiÓÈ· ÓÔÛ‹Ì·Ù·, fiˆ˜ Ô Û·Î¯·Ú҉˘ ‰È·‚‹-Ù˘ Î·È Ë ˘¤ÚÙ·ÛË, Ù· Û¯ÂÙÈο ÁÔÓ›‰È· - Ù·ÔÔ›· Û˘¯Ó¿ Â›Ó·È ÔÏÏ¿ - ·Ú¯›˙Ô˘Ó ÙÒÚ· Ó··Ó·ÁÓˆÚ›˙ÔÓÙ·È. §·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ ÙȘ‰È·ÊÔÚÂÙÈΤ˜ ÂÈÙÒÛÂȘ ÙˆÓ ÌÂÙ·ÏÏ·Á̤ӈÓÁÔÓȉ›ˆÓ Î·È ÙËÓ ÂÚÈÔÚÈṲ̂ÓË ÁÓÒÛË, ‰ÂÓ·ÔÙÂÏ› ¤ÎÏËÍË ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô fiÚÔ˜ “ÁÂÓÂ-ÙÈ΋ ÏËÚÔÊÔÚ›·” ¯ÚËÛÈÌÔÔÈÂ›Ù·È Ì ‰È·ÊÔ-ÚÂÙÈ΋ ¤ÓÓÔÈ· Û ‰È·ÊÔÚÂÙÈΤ˜ ÂÚÈÙÒÛÂȘ.ªÂÚÈΤ˜ ÊÔÚ¤˜, ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÂÓÓÔÒÓÙ·˜ÙËÓ Â›‰Ú·ÛË fiÏÔ˘ ÙÔ˘ ÁÔÓȉÈÒÌ·ÙÔ˜, ÂÓÒ ÈÔÛ˘¯Ó¿ ·Ó·Ê¤ÚÂÙ·È Û ·Ó·ÁÓˆÚÈṲ̂ӷ ÌÔÓÔÁÔ-Óȉȷο ÓÔÛ‹Ì·Ù· ‹ - ÈÔ Û˘ÁÎÂÎÚÈ̤ӷ - ÛÙ··ÔÙÂϤÛÌ·Ù· ÂÍÂÙ¿ÛÂˆÓ ‚·ÛÈÛÌ¤ÓˆÓ ÛÙÔDNA (2).

∏ ÚÒÙË Ú·ÎÙÈ΋ ÂÊ·ÚÌÔÁ‹ Ù˘ ·Ó¿ÁÓˆ-Û˘ ÙÔ˘ ·ÓıÚÒÈÓÔ˘ ÁÔÓȉÈÒÌ·ÙÔ˜ ı· Â›Ó·È Ë·Ó¿Ù˘ÍË ÈÔ ·ÔÙÂÏÂÛÌ·ÙÈÎÒÓ ÛÙÚ·ÙËÁÈÎÒÓÁÈ· ÙËÓ ÚfiÏË„Ë ÙˆÓ ÓÔÛËÌ¿ÙˆÓ Î·È ÙË ıÂÚ·-›· ÙÔ˘˜, ηıÒ˜ Î·È Ë ‰È‡ڢÓÛË ÙˆÓ ‰˘Ó·ÙÔ-

Ù‹ÙˆÓ ÁÈ· ÁÂÓÂÙÈΤ˜ ‰È·ÁÓˆÛÙÈΤ˜ ÂÍÂÙ¿ÛÂȘ(tests), ÙfiÛÔ Û ·ÙÔÌÈÎfi Â›Â‰Ô fiÛÔ Î·È ÛÂÏËı˘ÛÌȷο ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· (3).™ÙËÓ ÂfiÌÂÓË ‰ÂηÂÙ›· ‹ ÂÈÎÔÛ·ÂÙ›· Â›Ó·È Ô-χ Èı·Ófi Ó· “ÂÈÛ‚¿ÏÏÂÈ” Ë ÚÔÏËÙÈ΋ È·ÙÚÈ-΋, ÂÈÙÚ¤ÔÓÙ·˜ ÙÔÓ Î·Ù¿ÏÏËÏÔ ¤ÏÂÁ¯Ô ÙÔ˘-Ï¿¯ÈÛÙÔÓ ÂȉÈÎÒÓ ˘ÔÔÌ¿‰ˆÓ ÙÔ˘ ÏËı˘ÛÌÔ‡ÁÈ· ÁÂÓÂÙÈΤ˜ ÏËÚÔÊÔڛ˜, ÔÈ Ôԛ˜ ı· ·Ô-‚Ï¤Ô˘Ó Û ÂÂÌ‚¿ÛÂȘ ÛÙÔ Î¿ı ¿ÙÔÌÔ Î·È ı·‚ÂÏÙÈÒÓÔ˘Ó ÙËÓ ˘Á›· ÙÔ˘, ÚÔÏ·Ì‚¿ÓÔÓÙ·˜Ì›· ÓfiÛÔ. ªÂ Ù· Ó¤· ÂÈÙ‡ÁÌ·Ù· Ù˘ ÁÂÓÂÙÈ-΋˜ Ù¯ÓÔÏÔÁ›·˜, ı· ·Ó·ÁÓˆÚ›˙ÔÓÙ·È ¿ÙÔÌ· ¢-·ı‹ ÛÂ Û˘¯Ó¿ ÓÔÛ‹Ì·Ù·, fiˆ˜ Ë Î·Ú‰È·Î‹ÓfiÛÔ˜ (4), Ô ‰È·‚‹Ù˘ (5), Ô Î·ÚΛÓÔ˜ (6) Î·È ËÓfiÛÔ˜ Alzheimer (7-8), ÒÛÙ ӷ ÍÂÎÈÓ‹ÛÂÈ Ì›·ÚÔÛ¿ıÂÈ· ÚˆÙÔ‚¿ıÌÈ·˜ (.¯. ‰›·ÈÙ· ηȿÛÎËÛË) ‹ ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ ÚfiÏ˄˘ (ÚÒÈ-ÌË ‰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›·˜ وӷȉÈÒÓ, 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˙ÈÎ‹Î·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ıÂڷ›· ‹ ÛÙÚ·ÙËÁÈΤ˜ÚfiÏ˄˘, ÌÔÚ› Ó· ÌËÓ ‰ÈηÈÔÏÔÁÔ‡ÓÙ·È Ù·ÁÂÓÂÙÈο tests ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜(3,9-11). ∂ÈϤÔÓ, ÂÂȉ‹ Ù· ÌÈÎÚ¿ ·È‰È¿ ‰ÂÓÌÔÚÔ‡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·ÛËÁÈ· Ù· ÁÂÓÂÙÈο tests ÙˆÓ ·È‰ÈÒÓ ÔÊ›ÏÔ˘Ó Ó·Â›Ó·È ÚÔÛÂÎÙÈÎÔ›.

∏ ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜

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387¶ÚfiÏË„Ë Î·È ËıÈ΋

(∞∞¶) ÂÎÙÈÌ¿ fiÙÈ ÔÈ ·È‰›·ÙÚÔÈ Â›Ó·È ÂΛÓÔÈ

Ô˘ ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó - ηχÙÂÚ· ·fi

fiÏÔ˘˜ ÙÔ˘˜ ÁÈ·ÙÚÔ‡˜ - ÙÔ ·È‰› Î·È ÙÔ˘˜ ÁÔÓ›˜

ÙÔ˘ Ó· ηٷÓÔ‹ÛÔ˘Ó ÙȘ ÁÂÓÂÙÈΤ˜ ÏËÚÔÊÔ-

ڛ˜, ‰È·ÛÊ·Ï›˙ÔÓÙ·˜ ¯Â̇ıÂÈ· ÁÈ· Ù· ·ÔÙÂ-

ϤÛÌ·Ù· ÙÔ˘ test. ∂›Û˘, ÂÎÙÈÌ¿ fiÙÈ Â›Ó·È ÔÈ

ηٷÏÏËÏfiÙÂÚÔÈ 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 ÔÔÈ·-

‰‹ÔÙ ÂÈ‚¿Ú˘ÓÛË ÙÔ˘ ·È‰ÈÔ‡ (3).

°›ÓÂÙ·È ·Ó·ÊÔÚ¿ Û ÙÚÂȘ ÂÊ·ÚÌÔÁ¤˜ ÙˆÓ

ÁÂÓÂÙÈÎÒÓ tests Î·È ÙˆÓ ·ÓȯÓ¢ÙÈÎÒÓ ÚÔÁÚ·Ì-

Ì¿ÙˆÓ (screening) ÛÙ· ·È‰È¿: ÛÙ· ÓÂÔÁÓÈο

·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù·, ÛÙ· ·ÓȯÓ¢ÙÈο

ÚÔÁÚ¿ÌÌ·Ù· ÊÔÚ¤ˆÓ Î·È ÛÙÔÓ ÚÔÏËÙÈÎfi

¤ÏÂÁ¯Ô ÁÈ· ÓÔÛ‹Ì·Ù· Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ·ÚÁfi-

ÙÂÚ· ÛÙË ˙ˆ‹. ™Â fi,ÙÈ ·ÊÔÚ¿ ÛÙËÓ ÂÊ·ÚÌÔÁ‹

ÙÔ˘˜, ˘¿Ú¯ÂÈ ÛÔ‚·Úfi˜ ÚÔ‚ÏËÌ·ÙÈÛÌfi˜ η-

ıÒ˜ ÚÔ·ÙÔ˘Ó ËıÈο ı¤Ì·Ù·, Á‡Úˆ ·fi Ù·

ÔÔ›· ·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 ÁÂ-

ÓÂÙÈÎÒÓ tests ›¯Â ʤÚÂÈ ÛÙÔ ÚÔÛ΋ÓÈÔ ÙË Û˘-

˙‹ÙËÛË Û¯ÂÙÈο Ì ÙËÓ Î·Ù·ÏÏËÏfiÙËÙ· ÙÔ˘ ÓÂ-

ÔÁÓÈÎÔ‡ screening ÛÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË. ∆Ô

1983, ¤Ó· ÂÚÒÙËÌ·-‰›ÏËÌÌ· ‹Ù·Ó Â¿Ó Ë ‰È¿-

ÁÓˆÛË Ù˘ ΢ÛÙÈ΋˜ ›ÓˆÛ˘ ÛÙË ÓÂÔÁÓÈ΋ Â-

Ú›Ô‰Ô Î·È Ë ¤ÁηÈÚË ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜ η-

Ù·Ï‹ÁÂÈ Û Ì›ˆÛË Ù˘ ÓÔÛËÚfiÙËÙ·˜ Î·È Û ÂÈ-

Ì‹Î˘ÓÛË ÙÔ˘ ¯ÚfiÓÔ˘ ˙ˆ‹˜ ÙˆÓ ·È‰ÈÒÓ (12).

∞fi ÙÔ 1989 Ô˘ Ù·˘ÙÔÔÈ‹ıËΠÙÔ ÁÔÓ›‰ÈÔ

Ù˘ ΢ÛÙÈ΋˜ ›ÓˆÛ˘ (cystic fibrosis trans-

membrane conductance regulator - CFTR) ηÈ

̤¯ÚÈ Û‹ÌÂÚ· ¤¯Ô˘Ó Ù·˘ÙÔÔÈËı› ¿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˙˘Áˆ-

ÙÒÓ ·Û¯fiÓÙˆÓ ÓÂÔÁÓÒÓ ‰ÂÓ ¤¯ÂÈ ÂÚÈÏËÊı›

Û η̛· ¯ÒÚ· ÛÙ· ˘Ô¯ÚˆÙÈο screening

tests. ™Â ÔÚÈṲ̂ӷ ΤÓÙÚ· ‰ÈÂÓÂÚÁÂ›Ù·È ÂÚ¢ÓË-

ÙÈο Ë Ì¤ÙÚËÛË Ù˘ ·ÓÔÛÔÂÓÂÚÁÔ‡ ıÚ˘„›Ó˘

(immunoreactive trypsin - IRT) ÛÂ ÍËÚ¿ ÛÙ·Áfi-

Ó· ·›Ì·ÙÔ˜ Û οÚÙ˜ Guthrie Î·È Ë ÂÎÙ›ÌËÛË

Á›ÓÂÙ·È Ì ·ÓÔÛÔÌÂÙÚ‹ÛÂȘ. ∆· fiÚÈ· ÙˆÓ Ê˘ÛÈÔ-

ÏÔÁÈÎÒÓ ÂȤ‰ˆÓ Ù˘ IRT ÂÍ·ÚÙÒÓÙ·È ·fi ÙÔ

kit Î·È Ù· ¯ÚËÛÈÌÔÔÈÔ‡ÌÂÓ· ·ÓÙÈÛÒÌ·Ù· (13).

™Ù· ÓÂÔÁÓ¿ Ô˘ ·Ó¢ڛÛÎÂÙ·È ·‡ÍËÛË Ù˘ IRT

Ù›ıÂÙ·È ˘Ô„›· Ù˘ ÓfiÛÔ˘. ∏ ÂȂ‚·›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·ÚٿٷÈ

·fi ÙËÓ ÂıÓÈÎfiÙËÙ· ÙÔ˘ ÏËı˘ÛÌÔ‡ Ô˘ ÂÚ¢-

Ó¿Ù·È Î·È ·fi ÙÔÓ ·ÚÈıÌfi ÙˆÓ ÌÂÙ·ÏϿ͈Ó

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 ÔÔ›Ô ‰È·ÈÛÙÒÓÂÙ·È ÌfiÓÔ

Ì›· ÌÂÙ¿ÏÏ·ÍË, ı· Ú¤ÂÈ Ó· οÓÂÈ test ȉÚÒÙ·,

ÂÂȉ‹ ÌÔÚ› Ó· ¤¯ÂÈ Î·È ‰Â‡ÙÂÚË ÌÂÙ¿ÏÏ·ÍË

Ë ÔÔ›· ‰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 ·ӷϷ̂¿ÓÂÙ·È Ë IRT. ∂¿Ó ÙÔ ·ÔÙ¤ÏÂ-

ÛÌ· 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 ÚÔ-

ÛʤÚÂÙ·È ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË. OÈ ÌÂÙ·ÏÏ¿-

ÍÂȘ Ô˘ ‰È·ÈÛÙÒıËÎ·Ó ‹‰Ë ÛÙÔ˘˜ ÁÔÓ›˜-ÊÔ-

Ú›˜ ·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˜ ÂÈϤÁÔ˘Ó ÙË ‰È·ÎÔ‹.

∂ÈϤÔÓ, ÙÔ test ÚԂϤÂÈ ÙÔÓ ·ÓȯÓ¢ÙÈ-

Îfi ¤ÏÂÁ¯Ô ÛÙÔ˘˜ Û˘ÁÁÂÓ›˜ ÙÔ˘ ¿Û¯ÔÓÙÔ˜ ·È-

‰ÈÔ‡ Ô˘ ‰È·ÈÛÙÒıËÎÂ Ë ÌÂÙ¿ÏÏ·ÍË. ∂¿Ó ‚ÚÂ-

ı› ¤Ó·˜ Û˘ÁÁÂÓ‹˜ ÊÔÚ¤·˜, ÙfiÙÂ Î·È Ô Û‡ÓÙÚÔ-

Êfi˜ ÙÔ˘ Ú¤ÂÈ Ó· ÂÚ¢ÓËı› ÁÈ· ÙȘ ÈÔ Û˘¯Ó¤˜

ÌÂÙ·ÏÏ¿ÍÂȘ.

∆Ô test ·Â˘ı‡ÓÂÙ·È Û ÓÂÔÁÓ¿ ÔÈÎÔÁÂÓÂÈÒÓ

·fi ÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi Ì ÂχıÂÚÔ ÈÛÙÔÚÈ-

Îfi ÁÈ· ÙË ÓfiÛÔ Î·È Ì ÙÔ ·ÈÙÈÔÏÔÁÈÎfi fiÙÈ Ë

¤ÁηÈÚË ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ηٷϋÁÂÈ ÛÂ

fiÊÂÏÔ˜ ÙÔ˘ ·È‰ÈÔ‡, Ì Ì›ˆÛË Ù˘ ÓÔÛËÚfiÙË-

Ù·˜ Î·È ÂÈÌ‹Î˘ÓÛË ÙÔ˘ ¯ÚfiÓÔ˘ ˙ˆ‹˜ ÙÔ˘ (13).

∞Ó·‰ÚÔÌÈΤ˜ ÌÂϤÙ˜ ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘

ÔʤÏÔ˘˜ ÙÔ˘ ·È‰ÈÔ‡ ·fi ÙËÓ ÚÒÈÌË ‰È¿ÁÓˆ-

ÛË ¤‰ÂÈÍ·Ó fiÙÈ ÙÔ ÓÂÔÁÓÈÎfi screening ÁÈ· ÙËÓ

΢ÛÙÈ΋ ›ÓˆÛË Ô‰‹ÁËÛ ÛÙËÓ Ô˘ÛÈ·ÛÙÈ΋ Ì›ˆ-

ÛË Ù˘ ËÏÈΛ·˜ ‰È¿ÁÓˆÛ˘ (·fi 72 Û 12 ‚‰Ô-

Ì¿‰Â˜, Û‡Ìʈӷ Ì ÙË ÌÂϤÙË Ù˘ ÔÏÈÙ›·˜

Wisconsin), Û ̛· ÂÈÌ‹Î˘ÓÛË ÙÔ˘ ¯ÚfiÓÔ˘ ηÈ

Ù˘ ÔÈfiÙËÙ·˜ Ù˘ ˙ˆ‹˜ ÙˆÓ ·È‰ÈÒÓ, ηıÒ˜

¶·È‰È·ÙÚÈ΋ 2005;68:386-393

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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· ÓÂÔ-

ÁÓ¿ Ì ΢ÛÙÈ΋ ›ÓˆÛË. ™Â ÌÂϤÙ˜ ·fi ÙÔ ›‰ÈÔ

ÂÚ¢ÓËÙÈÎfi ΤÓÙÚÔ Ù˘ ÔÏÈÙ›·˜ Wisconsin, ÛÂ

·ӤÏÂÁ¯Ô Û¯ÂÙÈο Ì ÙȘ ·ÓıÚˆÔÌÂÙÚÈΤ˜

·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 ·ÓıÚˆÔÌÂÙÚÈ-

ÎÒÓ ·Ú·Ì¤ÙÚˆÓ, Ë Â›‰Ú·ÛË Ù˘ ηχÙÂÚ˘

ıÚ¤„˘ ÛÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· Ê¿ÓËÎÂ

ÛÙËÓ ¤Ú¢ӷ Ù˘ ¡¤·˜ ¡fiÙÈ·˜ O˘·Ï›·˜. ∏

Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· ‹Ù·Ó ÛËÌ·ÓÙÈο ηχ-

ÙÂÚË ÛÙ· ·È‰È¿ Ô˘ ›¯·Ó ‰È·ÁÓˆÛÙ› ÌÂ

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 ıÂ-

ÌÂÏÈ҉˘ ÁÈ· ÙËÓ ÔÈfiÙËÙ· Ù˘ ˙ˆ‹˜ ÙˆÓ ·È-

‰ÈÒÓ Î·È Ë ÚÒÈÌË ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË

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 ÂÈ‚›ˆÛË Î·È ÛÙËÓ ÎÏÈÓÈ΋ ¤Î‚·ÛË

ÌÂٷ͇ ‚ÚÂÊÒÓ ÌÂ Î·È ¯ˆÚ›˜ screening (15-17).

∆· ÏÂÔÓÂÎÙ‹Ì·Ù· ÙÔ˘ screening ‰ÂÓ Â-

ÚÈÔÚ›˙ÔÓÙ·È ÛÙËÓ ÚÒÈÌË ıÂڷ›· ÙÔ˘ ¿Û¯Ô-

ÓÙÔ˜ ·È‰ÈÔ‡. ∏ ÚÒÈÌË ‰È¿ÁÓˆÛË Î¿ÓÂÈ ‰˘Ó·-

Ù‹ ÙfiÛÔ ÙË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË ÛÙÔ˘˜ Û˘ÁÁÂ-

Ó›˜ ÙÔ˘ ·È‰ÈÔ‡ Ô˘ ÂϤÁ¯ıËΠfiÛÔ Î·È ÙËÓ

ÂÈÏÔÁ‹ ÁÈ· ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË, Ì ÛÎÔfi

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˜ ÔÚÈ·-

Τ˜ 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·Ï-

Ï¿ÍÂˆÓ Î·È ·fi „¢‰Ò˜ ıÂÙÈο ·ÔÙÂϤÛÌ·Ù·.

™ÙÔ ÚfiÁÚ·ÌÌ· Ù˘ Brittany Ù˘ °·ÏÏ›·˜,

ÌÂÙ¿ ÙËÓ ÂÓÙfiÈÛË Ì›·˜ ÌÂÙ¿ÏÏ·Í˘, Á›ÓÂÙ·È

‰ÈÂÍÔ‰ÈÎfi˜ ÌÔÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜. ∞˘Ù‹ Ë Ú·ÎÙÈ-

΋, Ë ÔÔ›· Ô‰ËÁ› ÛÙÔ Ì¤ÏÏÔÓ ÛÙËÓ Èı·Ó‹ ÂÓ-

ۈ̿وÛË ÛÙËÓ Ù¯ÓÈ΋ ÙˆÓ DNA-chips, ¤¯ÂÈ

ÏÂÔÓÂÎÙ‹Ì·Ù· Î·È ÌÂÈÔÓÂÎÙ‹Ì·Ù·, Ù· ÔÔ›·

‰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,

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· ÔÔ›· Ë ÎÏÈÓÈ΋

¤Î‚·ÛË ·Ú·Ì¤ÓÂÈ ·ÎfiÌË ‰‡ÛÎÔÏÔ Ó· ÚÔ‚ÏÂ-

Êı›. ŒÙÛÈ, ‰È·ÈÛÙÒÓÔÓÙ·È ·È‰È¿ Ì ‰‡Ô ÌÂ-

Ù·ÏÏ¿ÍÂȘ CFTR Î·È Ì ¿Ù˘Ô ÁÔÓfiÙ˘Ô, Ô˘

Â›Ó·È ÂχıÂÚ· Û˘Ìو̿وÓ. ¢ÂÓ Â›Ó·È ÚÔ˜

ÙÔ ·ÚfiÓ ‰˘Ó·ÙfiÓ Ó· ·ÔÎÏÂÈÛÙ› ÙÔ ÁÂÁÔÓfi˜

fiÙÈ ·˘ÙÔ› ÔÈ ÁÔÓfiÙ˘ÔÈ ··ÓÙÒÓÙ·È ÛÂ ‹È·

ÌÔÚÊ‹ Ù˘ ΢ÛÙÈ΋˜ ›ÓˆÛ˘, Ë ÔÔ›· ı· ·Ó·-

Ù˘¯ı› ·ÚÁfiÙÂÚ· ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹. ∏ ·‚¤-

‚·ÈË ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘ ÌÔÚ› Ó· Â›Ó·È ÂÛÙ›·

¿Á¯Ô˘˜ ÁÈ· ÌÂÚÈο ˙¢Á¿ÚÈ·, Ù· ÔÔ›· ˙Ô˘Ó ÌÂ

ÙËÓ ·ÁˆÓ›· Ù˘ ÂΉ‹ÏˆÛ˘ Ù˘ ÓfiÛÔ˘, ηıÈ-

ÛÙÒÓÙ·˜ ÙË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË ˘¤ÚÙ·Ù˘

ÛËÌ·Û›·˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·˘ÙÒÓ ÙˆÓ ÔÈÎÔ-

ÁÂÓÂÈÒÓ (18). ∂› ÙÔ˘ ·ÚfiÓÙÔ˜, Ë ÚÒÈÌË ‰È¿-

ÁÓˆÛË ÌÂÙ·ÏÏ¿ÍÂˆÓ ÙÔ˘ CFTR ηÈ, ÂÔ̤ӈ˜,

ÂÓfi˜ ·È‰ÈÔ‡ Ì ÁÔÓfiÙ˘Ô Î˘ÛÙÈ΋˜ ›ÓˆÛ˘ ·Ï-

Ï¿ ÂχıÂÚÔ˘ Û˘Ìو̿وÓ, ÂÈÙÚ¤ÂÈ ÛÙ·

˙¢Á¿ÚÈ· Ó· ÚÔÁÚ·ÌÌ·Ù›˙Ô˘Ó ÙËÓ ·Ó··Ú·-

ÁˆÁ‹ Ù˘ ÔÈÎÔÁ¤ÓÂÈ¿˜ ÙÔ˘˜, ÌÂÙ¿ ·fi Ôχ η-

Ï‹ ÏËÚÔÊfiÚËÛË. ∞˘Ùfi Ô‰ËÁ› ÌÂÚÈο ˙¢Á¿-

ÚÈ· ÛÙË ‰ÈÂÓ¤ÚÁÂÈ· ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘

Û ÂfiÌÂÓË Î‡ËÛË, ÚÈÓ ·ÎfiÌË ÙÔ ·È‰› ÙÔ˘˜ -

Ô˘ ÂϤÁ¯ıËΠ̠screening test - ·ÚÔ˘ÛÈ¿ÛÂÈ

οÔÈ· Û˘ÌÙÒÌ·Ù· Ù˘ ÓfiÛÔ˘. ∆¤ÏÔ˜, ÙÔ ÓÂÔ-

ÁÓÈÎfi screening ÂÈÙÚ¤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 ÌÂ

¿ÏÏÔ ÙÚfiÔ 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 ÌÂ-

ٷ͇ ÙˆÓ ÂÏÂÁÌ¤ÓˆÓ ÓÂÔÁÓÒÓ. ∞˘Ù‹ Ë ÏËÚÔ-

ÊÔÚ›· ‰ËÌÈÔ˘ÚÁ› ÚÔ‚Ï‹Ì·Ù· ÛÙËÓ ÔÈÎÔÁ¤-

ÓÂÈ·, ‰ÈfiÙÈ ÔÈ ÁÔÓ›˜ ÌÔÚ› 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 ÂËÚÂ-

¿˙ÂÙ·È ·fi ÙËÓ ÂÈÏÔÁ‹ ÙˆÓ Î·ÙÒÙÂÚˆÓ ÙÈÌÒÓ

Ù˘ ̤ÙÚËÛ˘ Ù˘ IRT. ∏ ·‡ÍËÛË ·˘ÙÔ‡ ÙÔ˘

ÔÚ›Ô˘ ¤¯ÂÈ Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙË Ì›ˆÛË ÙÔ˘ ·ÚÈı-

ÌÔ‡ ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ ·Ú·¤ÌÔÓÙ·È ÁÈ· DNA

·Ó¿Ï˘ÛË Î·È, ÂÔ̤ӈ˜, ÙË Ì›ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡

ÙˆÓ ‰È·ÁÓˆÛÌ¤ÓˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ. ªÔÚ›,

ÏÔÈfiÓ, Ì ·˘Ùfi ÙÔ Â›Â‰Ô Ù˘ ̤ÙÚËÛ˘ Ó·

¶·È‰È·ÙÚÈ΋ 2005;68:386-393

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389¶ÚfiÏË„Ë Î·È ËıÈ΋

ÌË ‰È·ÁÓˆÛÙÔ‡Ó ‹ Ó· ¯·ıÔ‡Ó ·È‰È¿, Ù· ÔÔ›·

¤¯Ô˘Ó ‰‡Ô ‹È˜ ÌÔÚʤ˜ CFTR ÌÂÙ·ÏϿ͈Ó

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). ∂ÈϤÔÓ, Ë ‰È¿ÁÓˆÛË Ù˘ ΢ÛÙÈ΋˜ ›Óˆ-

Û˘ Û ·˘Ù¿ Ù· ·È‰È¿ ÌÔÚ› Ó· Â›Ó·È ·ÎfiÌË

ÈÔ ‰‡ÛÎÔÏÔ Ó· Á›ÓÂÈ ÛÙÔ Ì¤ÏÏÔÓ, ÂÂȉ‹ ·˘Ù¿

·Ó‹ÎÔ˘Ó ÛÙË ÁÂÓÈ¿ ÙˆÓ “screened children” ηÈ

oÈ ·È‰›·ÙÚÔÈ ÌÔÚ› ·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 ·ÔÙÂϤ-

ÛÌ·Ù· Ù˘ IRT ÌÔÚ› Ó· ˘Ê›ÛÙ·ÓÙ·È „˘¯ÔÏÔ-

ÁÈÎfi ÙÚ·‡Ì· Î·È Ó· ‰È·Î·Ù¤¯ÔÓÙ·È ·fi ·ÈÛı‹-

Ì·Ù· ¿Á¯Ô˘˜ (9-13). ∂ÈϤÔÓ, ¤Ó· ÌÈÎÚfi Ô-

ÛÔÛÙfi ÙˆÓ ÁÔÓ¤ˆÓ ÌÔÚ› 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· ÌÔÚ› Ó· ÂËÚ¿ÛÔ˘Ó ÙȘ ÂÈÏÔÁ¤˜ ·Ó··-

Ú·ÁˆÁ‹˜ ÙˆÓ ÁÔÓ¤ˆÓ (16). ŸÏ˜ ÔÈ ÚÔ·Ó·ÊÂÚ-

ı›Û˜ ηٷÛÙ¿ÛÂȘ ÂÍËÁÔ‡Ó ÙÔÓ ÏfiÁÔ ÁÈ· ÙÔÓ

ÔÔ›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 ¤ÁηÈ-

ÚË ‰È¿ÁÓˆÛË ‚Ï·‚ÒÓ Ù˘ ·ÎÔ‹˜. ∏ ·ÎÔ˘ÔÌÂ-

ÙÚ›· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ˆ˜ ÓÂÔÁÓÈÎfi screening

ÁÈ· ·˘Ù¤˜ ÙȘ ‚Ï¿‚˜. OÈ ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓÈ-

‰›Ô˘ Ù˘ ÎÔÓÂ͛Ӣ 26 (connexin 26) ¢ı‡ÓÔ-

ÓÙ·È ÁÈ· ÙÔ 40% fiÏˆÓ ÙˆÓ ÂÚÈÙÒÛÂˆÓ 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

ÔÔ›· ·ÎÔÏÔ˘ı› DNA test ÁÈ· ¤ÏÂÁ¯Ô ÙˆÓ ÌÂ-

Ù·ÏÏ¿ÍÂˆÓ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ÎÔÓÂ͛Ӣ 26. ∏

ÚÒÈÌË ‰È¿ÁÓˆÛË ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· Ù˘

ÂÈıÂÙÈ΋˜ ·Ú¤Ì‚·Û˘ ÁÈ· ÙË ‚ÂÏÙ›ˆÛË ÙˆÓ

‰ÂÍÈÔÙ‹ÙˆÓ ÙÔ˘ ÏfiÁÔ˘ ÂÓfi˜ ·È‰ÈÔ‡, ÙËÓ ÂÌ-

ʇÙ¢ÛË Îԯϛ· ‹ Î·È ÙˆÓ ‰‡Ô.

ŒÓ· ÌfiÓÈÌÔ ËıÈÎfi ı¤Ì· ÛÙ· ÓÂÔÁÓÈο ·ÓÈ-

¯Ó¢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 ÏËÚÔÊfi-

ÚËÛË, 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 ıÂڷ›·˜

ÔÚÈÛÌ¤ÓˆÓ Î·Ù·ÛÙ¿ÛˆÓ, ˘ÔηıÈÛÙÒÓÙ·˜ ÙÔ

ÁÔÓÂ˚Îfi ‰Èη›ˆÌ· ÁÈ· ¿ÚÓËÛË (3). ŒÓ· ·ÓÙ›ıÂÙÔ

Âȯ›ÚËÌ· ˘ÔÛÙËÚ›˙ÂÈ fiÙÈ ·Ú·‰ÔÛȷο ÔÈ

ÁÔÓ›˜ ¤¯Ô˘Ó ÙËÓ ·ÚÌÔ‰ÈfiÙËÙ· Ó· ÂÈϤÁÔ˘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· ÂÈϤÁÔ˘Ó ÌÂٷ͇ ‰È·ÊfiÚˆÓ ÚÔÔÙÈÎÒÓ

ÁÈ· ηٷÛÙ¿ÛÂȘ ÏÈÁfiÙÂÚÔ ·ÂÈÏËÙÈΤ˜ ÁÈ· ÙË

˙ˆ‹ ÙÔ˘. ªÂÚÈΤ˜ ÊÔÚ¤˜, Ì¿ÏÈÛÙ·, οÓÔ˘Ó Ù¤-

ÙÔȘ ÂÈÏÔÁ¤˜, ÙȘ Ôԛ˜ ÌÂÚÈÎÔ› ·ÁÁÂÏÌ·-

ٛ˜ ˘Á›·˜ ı· ıˆÚÔ‡Û·Ó ·Ú¿ÏÔÁ˜.

∞ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· ÊÔÚ¤ˆÓ

∏ ÚfiÔ‰Ô˜ Ù˘ È·ÙÚÈ΋˜ Ù¯ÓÔÏÔÁ›·˜ ÂÈ-

ÙÚ¤ÂÈ ÙËÓ ·Ó·ÁÓÒÚÈÛË ·ÙfiÌˆÓ Ô˘ Â›Ó·È ÊÔ-

Ú›˜ ÌÂÙ·ÏÏ¿ÍÂˆÓ Û ÁÔÓ›‰È· ‰È·ÊfiÚˆÓ ÓÔÛË-

Ì¿ÙˆÓ, fiˆ˜ Ù˘ ÓfiÛÔ˘ Tay-Sachs, Ù˘ Ì˘˚΋˜

‰˘ÛÙÚÔÊ›·˜, Ù˘ ‰Ú·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 ÙÚfiÔ

Î·È - Ì ÌÂÚÈΤ˜ ÂÍ·ÈÚ¤ÛÂȘ - ÚÔÛ‚¿ÏÏÔÓÙ·È

ÌfiÓÔ ·ÁfiÚÈ·. ∆Ô ÁÔÓ›‰ÈÔ Duchenne-Becker ›-

Ó·È ÁÓˆÛÙfi fiÙÈ Â›Ó·È ¤Ó· ·fi Ù· ÌÂÁ·Ï‡ÙÂÚ· ÁÔ-

Ó›‰È· ÙÔ˘ ·ÓıÚÒÔ˘ Î·È ÂÓÙÔ›˙ÂÙ·È ÛÙÔ ¯Úˆ-

Ìfiۈ̷ à (Ãp21) (12,22,23). ∞˘Ùfi ÙÔ ÁÔÓ›‰ÈÔ

Έ‰ÈÎÔÔÈ› ÙËÓ ÚˆÙ½ÓË ‰˘ÛÙÚÔÊ›ÓË, Ë

ÔÔ›· ¤¯ÂÈ ¤Ó·Ó ÚfiÏÔ-ÎÏÂȉ› ÛÙËÓ ·ÎÂÚ·ÈfiÙËÙ·

Ù˘ ‰ÔÌ‹˜ ÙˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ. ™ÙËÓ ÏÂÈÔÓfiÙËÙ·

ÙˆÓ ·ÛıÂÓÒÓ ¤¯ÂÈ ·Ú·ÙËÚËı› ÌÂÁ¿ÏË ÔÈÎÈ-

Ï›· ÌÂÙ·ÏϿ͈Ó, Ì ϋÚË ‹ ÌÂÚÈ΋ ·ÒÏÂÈ·

ÙÔ˘ ÁÔÓȉ›Ô˘. ŒÓ·˜ ·Ú·ÏÏ·Á̤ÓÔ˜ Ù‡Ô˜ ›ӷÈ

Ë Ì˘˚΋ ‰˘ÛÙÚÔÊ›· Becker (BMD), Ë ÔÔ›· ›-

Ó·È ÚÔԉ¢ÙÈ΋ ÓfiÛÔ˜ Ì ÌÂÁ·Ï‡ÙÂÚË ÂÈ‚›ˆ-

ÛË. ¢ÂÓ ˘¿Ú¯ÂÈ ÚÈ˙È΋ ıÂڷ›· ÁÈ· ÙËÓ DMD,

Ë ÔÔ›· ¤¯ÂÈ ÚÔԉ¢ÙÈ΋ Âȉ›ӈÛË, ÂÓÒ Ô

ηÓfiÓ·˜ 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˜ ÌÂ

DNA ·Ó¿Ï˘ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ÓfiÛÔ˘, fiÔ˘

¯ÚËÛÈÌÔÔÈÂ›Ù·È ÍËÚ¿ ÛÙ·ÁfiÓ· ·›Ì·ÙÔ˜ ÛÂ

ÚÔÛÚÔÊËÙÈÎfi ¯·ÚÙ›. ™ÙÔ ÂıÂÏÔÓÙÈÎfi ÚfiÁÚ·Ì-

Ì· 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

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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·ıÔÚÈ-

ÛÌfi 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 ›‰· CK >500 U/L

ÂÌÊ·Ó›˙Ô˘Ó ÊıÔÚÈÛÌfi. ∞ÎÔÏÔ˘ı› ÂÎÙ›ÌËÛË

Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ CK ÛÙÔÓ ÔÚfi Ì ·ÓÔÛÔ-

ÂÓ˙˘ÌÈ΋ ̤ıÔ‰Ô. ™Ù· ·ıÔÏÔÁÈο ‰Â›ÁÌ·Ù·

·ÎÔÏÔ˘ı› ÌÔÚȷ΋ ‰ÈÂÚ‡ÓËÛË ÁÈ· ÙËÓ ·Ó·˙‹-

ÙËÛË ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ (ÂÏÏ›„ˆÓ) ÙÔ˘ ÁÔÓÈ-

‰›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 ÌÂ

DMD, ÂÂȉ‹ οÔȘ 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 Â-

ÚÈÙÒÛÂȘ ·ÛıÂÓÒÓ ¯ˆÚ›˜ ·Ó·ÁÓˆÚ›ÛÈ̘ ÌÂ-

Ù·ÏÏ¿ÍÂȘ, ·ÎÔÏÔ˘ı› ‚ÈÔ„›· Ì˘ÒÓ ÁÈ· ÙËÓ

ÂÎÙ›ÌËÛË Ù˘ ηٿÛÙ·Û˘ Ù˘ ‰˘ÛÙÚÔÊ›Ó˘. ∏

‚ÈÔ„›· Û˘Ì‚¿ÏÏÂÈ ÛÙËÓ Ù·˘ÙÔÔ›ËÛË Ù˘ Ófi-

ÛÔ˘ ηÈ, ÂÈϤÔÓ, ηıÔÚ›˙ÂÈ ÙËÓ ÈÔ ‚·ÚÈ¿

ÌÔÚÊ‹ DMD ·fi ÙËÓ BMD, fiÙ·Ó Ë DNA ÌÂ-

Ù¿ÏÏ·ÍË ‰ÂÓ Â›Ó·È ÏËÚÔÊÔÚȷ΋.

∆Ô fiÊÂÏÔ˜ ÙÔ˘ ·ÛıÂÓÔ‡˜, Ù˘ ÔÈÎÔÁ¤ÓÂÈ¿˜

ÙÔ˘ Î·È Ù˘ ÎÔÈÓˆÓ›·˜ ·Ú·Ì¤ÓÂÈ ¤Ó· ·ÌÊÈÏÂ-

ÁfiÌÂÓÔ ı¤Ì·, Ï·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ ÙËÓ ·Ô˘-

Û›· - ̤¯ÚÈ Û‹ÌÂÚ· - Ì›·˜ ·ÔÙÂÏÂÛÌ·ÙÈ΋˜ ıÂ-

ڷ›·˜. Œ¯Ô˘Ó ÚÔ·„ÂÈ ÚÔ‚Ï‹Ì·Ù· Ô˘

·Ó·Ê¤ÚÔÓÙ·È ÛÙËÓ „˘¯ÔÏÔÁÈ΋ Î·È ÂÓ‰ÔÔÈÎÔ-

ÁÂÓÂȷ΋ ÂÈ‚¿Ú˘ÓÛË Û ÂÚÈÙÒÛÂȘ ÚÔÛ˘-

Ìو̷ÙÈÎÒÓ ‰È·ÁÓÒÛÂˆÓ (ÔÛÔÛÙfi ÂÚ›Ô˘

40% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û˘Ì‚·›ÓÂÈ de novo, ‰Ë-

Ï·‰‹ Û ÔÈÎÔÁ¤ÓÂȘ ¯ˆÚ›˜ ÚÔËÁÔ‡ÌÂÓ· ÚÔ-

ۂ‚ÏË̤ӷ ¿ÙÔÌ·). ∞fi ÙËÓ ¿ÏÏË ÏÂ˘Ú¿,

fï˜, Ë ‰È·ıÂÛÈÌfiÙËÙ· Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆ-

Û˘ ÌÔÚ› Ó· ÂÈÙÚ¤„ÂÈ ÛÙȘ ÔÈÎÔÁ¤ÓÂȘ Ó·

οÓÔ˘Ó Ú·ÎÙÈΤ˜ ÂÈÏÔÁ¤˜ Û¯ÂÙÈο Ì ÙË ˙ˆ‹

ÂÓfi˜ ·È‰ÈÔ‡ Ì ÂȉÈΤ˜ ·Ó¿ÁΘ Î·È Ó· ·Ô-

ʇÁÔ˘Ó Ì›· ·Ú·ÙÂٷ̤ÓË “‰È·ÁÓˆÛÙÈ΋

Ô‰‡ÛÛÂÈ·”. ªÔÚ›, ·ÎfiÌË, ÁÈ· ÌÂÚÈΤ˜ ÔÈÎÔ-

Á¤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 ÂÌÂÈÚ›· ÙÔ˘˜,

ÚÔÙ›ÓÔ˘Ó Ó· Â›Ó·È ÚÔ·ÈÚÂÙÈ΋ Ë ¯Ú‹ÛË Ù˘

‰È·ÁÓˆÛÙÈ΋˜ ÁÂÓÂÙÈ΋˜ ·Ó¿Ï˘Û˘. ¶·Ú¿ÏÏË-

Ï·, ¯ÚÂÈ¿˙ÂÙ·È Ó· ˘¿Ú¯ÂÈ ¤Ó· ·˘ÛÙËÚfi ÚˆÙfi-

ÎÔÏÏÔ ÁÈ· ÙËÓ ·ÚÔ¯‹ ˘ÔÛÙ‹ÚÈ͢ ÛÙÔ˘˜ ÁÔ-

Ó›˜ Î·È ÛÙÔ ¿Û¯ÔÓ ·È‰›, ÙÔ ÔÔ›Ô ÂÈÙÚ¤ÂÈ

ÙË ‰È·ÛÊ¿ÏÈÛË ÂÈÎÔÈÓˆÓ›·˜ Î·È Û˘ÓÂÚÁ·Û›·˜

ÌÂٷ͇ ÚˆÙÔ‚¿ıÌÈ·˜, ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ ηÈ

ÙÚÈÙÔ‚¿ıÌÈ·˜ ˘ËÚÂÛ›·˜. ∂ȉÈÎfiÙÂÚ·, ›ӷÈ

··Ú·›ÙËÙË Ë ÔÏÔÎÏËڈ̤ÓË ˘ËÚÂÛ›· Û˘Ì-

‚Ô˘Ï¢ÙÈ΋˜, Ë ·È‰È·ÙÚÈ΋ ˘ËÚÂÛ›· ÛÙ‹ÚÈ-

͢ 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 ÁÂÓÈ-

Îfi˜ ÏËı˘ÛÌÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ‰ÂÓ ¤¯Ô˘Ó ÂÊ·ÚÌÔ-

ÛÙ› 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 ÂÈÏÔ-

Á¤˜ ·Ó··Ú·ÁˆÁ‹˜ Û ÂΛÓÔ˘˜ Ô˘ ı· ‰È·È-

ÛÙÒÓÔÓÙ·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›˜, ÌÔÚ› Ó· ›-

Ó·È „˘¯ÔÏÔÁÈο ÈÔ Â˘¿ÏˆÙÔÈ Û ۇÁÎÚÈÛË ÌÂ

ÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜. ¶·Ú·Ì¤ÓÂÈ ‰Â ·ÌÊ›‚ÔÏÔ Â¿Ó Ô

¤ÏÂÁ¯Ô˜ Û Ó·ÚfiÙÂÚ˜ ËÏÈ˘ ı· ÂÈʤÚÂÈ ÛÙÔ

̤ÏÏÔÓ ·ÏÏ·Á¤˜ ÛÙË Û˘ÌÂÚÈÊÔÚ¿ Ù˘ ·Ó··-

Ú·ÁˆÁ‹˜ ÙÔ˘˜ (3).

∏ ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ ‰ÂÓ

˘ÔÛÙËÚ›˙ÂÈ ÙËÓ Â˘Ú›· ¯Ú‹ÛË ÙˆÓ ·ÓȯÓ¢ÙÈ-

ÎÒÓ 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 ÔÔ›ÔÈ ¤¯Ô˘Ó Ï‹Úˆ˜

ÏËÚÔÊÔÚËı› ÁÈ· Ù· ÔʤÏË Î·È ÙÔ˘˜ ÎÈÓ‰‡-

ÓÔ˘˜ Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙÔÓ ¤ÏÂÁ¯Ô.

¶ÚÔÏËÙÈÎfi˜ ¤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· ÔÔ›· ÂΉË-

ÏÒÓÔÓÙ·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‡˜ ÓÂ-

ÊÚÔ‡˜, ÙË ÓfiÛÔ Huntington Î·È ÌÂÚÈο ›‰Ë

ηÚΛÓÔ˘ (2). ∂ÈϤÔÓ, Û‹ÌÂÚ· Â›Ó·È ÂÊÈÎÙfi

Ó· ÂÓÙÔÈÛÙÔ‡Ó ÁÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜, ÔÈ ÔÔ›-

ÔÈ ·˘Í¿ÓÔ˘Ó ÙËÓ Èı·ÓfiÙËÙ· ÂΉ‹ÏˆÛ˘ Û˘-

¯ÓÒÓ ÓÔÛËÌ¿ÙˆÓ, fiˆ˜ Ë ÓfiÛÔ˜ ÙˆÓ ÛÙÂÊ·ÓÈ-

·›ˆÓ ·ÚÙËÚÈÒÓ, Ô ‰È·‚‹Ù˘, Ù· ÂÁÎÂÊ·ÏÈο

ÂÂÈÛfi‰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), ÔÚÈṲ̂-

ÓÔÈ Ù‡ÔÈ Î·ÚΛÓÔ˘ ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘ (6) ηÈ

¶·È‰È·ÙÚÈ΋ 2005;68:386-393

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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 ·˘ÙÔۈ̷-

ÙÈÎfi ÂÈÎÚ·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 ̛· ÂÈ‚›ˆÛË Ê˘ÛÈÔ-

ÏÔÁÈ΋˜ ‰È¿ÚÎÂÈ·˜. ª¤¯ÚÈ Û‹ÌÂÚ· ‰ÂÓ ˘¿Ú¯ÂÈ

·ÔÙÂÏÂÛÌ·ÙÈ΋ ıÂڷ›·, ·ÏÏ¿ Ô‡ÙÂ Î·È ÚÔ-

ÏËÙÈ΋. ∏ ·fiÊ·ÛË ÁÈ· ÙÔÓ ÚÔÎÏÈÓÈÎfi ¤ÏÂÁ-

¯Ô Â›Ó·È Î·ı·Ú¿ ÚÔÛˆÈ΋ Î·È Ù· ·ÔÙÂϤ-

ÛÌ·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‹˜ ÂÌÂÈÚ›·

·fi ÙË ª. µÚÂÙ·Ó›· ‰Â›¯ÓÂÈ fiÙÈ ÌfiÓÔ 20% Â-

Ú›Ô˘ ÙˆÓ ·ÙfiÌˆÓ Û ΛӉ˘ÓÔ ÁÈ· ÓfiÛÔ

Huntington ˙ËÙÔ‡Ó Ó· ÂÏÂÁ¯ıÔ‡Ó (23).

°È· οÔȘ ·fi ·˘Ù¤˜ ÙȘ ηٷÛÙ¿ÛÂȘ, Ë

ÁÓÒÛË ÙÔ˘ ÎÈÓ‰‡ÓÔ˘ ÌÔÚ› Ó· ‚ÔËı‹ÛÂÈ Ù·

¿ÙÔÌ· Ó· ÌÂÈÒÛÔ˘Ó ÙË ÓÔÛËÚfiÙËÙ· ‹ ÙÔÓ Î›Ó-

‰˘ÓÔ Ù˘ ıÓËÛÈÌfiÙËÙ·˜. ∂ÈϤÔÓ, ̤ÏË Ù˘

ÔÈÎÔÁ¤ÓÂÈ·˜ Û ΛӉ˘ÓÔ ÌÔÚ› Ó· ˆÊÂÏËıÔ‡Ó

„˘¯ÔÏÔÁÈο ›Ù ̷ı·›ÓÔÓÙ·˜ fiÙÈ ‰ÂÓ Â›Ó·È ÊÔ-

Ú›˜ Ù˘ ÌÂÙ¿ÏÏ·Í˘, ›Ù - ÂÂȉ‹ ı· ·‡ÛÂÈ Ë

·ÌÊÈ‚ÔÏ›· fiÙÈ ¤¯Ô˘Ó Èı·ÓÒ˜ ÙË ÓfiÛÔ - ·Ó ‰È·-

ÈÛÙˆı› fiÙÈ Â›Ó·È ÌfiÓÔ ÊÔÚ›˜. °È· ÔÏϤ˜ η-

Ù·ÛÙ¿ÛÂȘ, fï˜, ‰ÂÓ ¤¯ÂÈ ·Ô‰Âȯı› Ë Ì›ˆÛË

Ù˘ ÓÔÛËÚfiÙËÙ·˜ ‹ Ù˘ ıÓËÛÈÌfiÙËÙ·˜ ˆ˜ ·Ô-

Ù¤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 ·Ô-

ÙÂÏÂÛÌ·ÙÈΤ˜ Û ·˘ÙfiÓ ÙÔÓ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘

ÏËı˘ÛÌfi (3). ∂ÈϤÔÓ, Ë ÁÓˆÛÙÔÔ›ËÛË ÙÔ˘

·˘ÍË̤ÓÔ˘ ÎÈÓ‰‡ÓÔ˘ ÌÔÚ› Ó· ÂÓÂÚÁÔÔÈ›

Ì›· ·ÓÙ›ıÂÙË „˘¯ÔÏÔÁÈ΋ ·¿ÓÙËÛË Î·È ÂÓ‰Â-

¯Ô̤ӈ˜ ÎÔÈÓˆÓÈΤ˜ ‰È·ÎÚ›ÛÂȘ. °È· ÙÔÓ ÏfiÁÔ

·˘Ùfi, Ô ÁÚ‹ÁÔÚÔ˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÌÂÙ·ÏϿ͈Ó

ÙˆÓ ÁÔÓȉ›ˆÓ BRCA 1/BRCA 2 (Ù· ÔÔ›· ÚÔ-

‰È·ı¤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˘Ô‰È·ÛÈÎÔ‡

ÎÏËÚÔÓÔÌÈÎÔ‡ ηÚΛÓÔ˘ ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘)

ÛÙËÓ ÎÏÈÓÈ΋ È·ÙÚÈ΋ ÙˆÓ ÂÓËÏ›ÎˆÓ ‹Ù·Ó ·Ô-

ı·ÚÚ˘ÓÙÈÎfi˜ (6). ∏ ÔÏ˘ÏÔÎfiÙËÙ· ÙˆÓ ÁÂÓÂ-

ÙÈÎÒÓ tests Î·È Ë ·‚‚·ÈfiÙËÙ· ÁÈ· ÙÔ˘˜ ÎÈÓ‰‡-

ÓÔ˘˜ Î·È Ù· ÔʤÏË ·fi Ù· ·ÔÙÂϤÛÌ·Ù·, ÂÈ-

‚‚·ÈÒÓÔ˘Ó ÙËÓ ·Ó¿ÁÎË ‰ÈÂÍÔ‰È΋˜ ÁÂÓÂÙÈ΋˜

ηıÔ‰‹ÁËÛ˘ ÁÈ· ÙÔÓ ÚÔÏËÙÈÎfi ÁÂÓÂÙÈÎfi

¤ÏÂÁ¯Ô, fiÛÔÓ ·ÊÔÚ¿ ÛÙ· ÓÔÛ‹Ì·Ù· Ô˘ ÂÌÊ·-

Ó›˙ÔÓÙ·È Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ·. ¶ÔÏÏÔ› ÂÓ‹ÏÈ-

Θ ÂÈϤÁÔ˘Ó Ó· ÌËÓ ÂÏÂÁ¯ıÔ‡Ó, ÁÂÁÔÓfi˜ Ô˘

‰Â›¯ÓÂÈ fiÙÈ ‰ÂÓ ÌÔÚ› 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 ÂÈÙÚÔ¤˜ ·fi ÙËÓ ∞ÌÂÚÈ-

ηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ ¤¯Ô˘Ó ¿ÚÂÈ

ı¤ÛË ÁÈ· ÙÔÓ ÁÂÓÂÙÈÎfi ¤ÏÂÁ¯Ô ÛÙ· ·È‰È¿ ηÈ

ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ÁÈ· Ù· ÓÔÛ‹Ì·Ù· Ô˘ ÂΉËÏÒÓÔ-

ÓÙ·È ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (9). ∏ ÂÈÙÚÔ‹ Ù˘ °Â-

ÓÂÙÈ΋˜ Û˘ÛÙ‹ÓÂÈ fiÙÈ ¿ÙÔÌ· ËÏÈΛ·˜ ÌÈÎÚfiÙÂ-

Ú˘ 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· ¿Ï-

ÏÔ Ì¤ÏÔ˜ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ˆÊÂÏËı›, ÂÓÒ ‰ÂÓ

ÚԂϤÂÙ·È ‚Ï¿‚Ë ÁÈ· ÙÔ ¿ÙÔÌÔ Ô˘ ÂϤÁ¯Â-

Ù·È. ∏ ÂÈÙÚÔ‹ ıˆÚ› ‚·ÛÈÎfi ̤ÚÔ˜ Ù˘ ‰È·-

‰Èηۛ·˜ ÙË ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋ ÚÈÓ Î·È

ÌÂÙ¿ ÙËÓ ÂͤٷÛË (3).

∏ ÂÈÙÚÔ‹ 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˘

ÌÔÚ› Ó· ‰ÒÛÂÈ ·ÚÔÛ‰fiÎËÙ˜ ÏËÚÔÊÔڛ˜

Û¯ÂÙÈο Ì ÙËÓ ·ÙÚfiÙËÙ·. ∏ ∞ÌÂÚÈηÓÈ΋

∂Ù·ÈÚ›· ∞ÓıÚÒÈÓ˘ °ÂÓÂÙÈ΋˜ ¤¯ÂÈ ÙËÓ ¿Ô-

„Ë fiÙÈ Ì¤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ‰ÂÓ ÌÔÚÔ‡Ó Ó·

ÏËÚÔÊÔÚÔ‡ÓÙ·È ÁÈ· ÙËÓ ·ÙÚfiÙËÙ·, ·Ú¿ Ìfi-

ÓÔ Â¿Ó Ô Î·ıÔÚÈÛÌfi˜ Ù˘ ·ÙÚfiÙËÙ·˜ ‹Ù·Ó Ô

·ÔÎÏÂÈÛÙÈÎfi˜ ÛÎÔfi˜ ÙÔ˘ ÂϤÁ¯Ô˘ (5).

∂Í¿ÏÏÔ˘, ÛÙË ‰È·‰Èηۛ· ÙÔ˘ ÁÂÓÂÙÈÎÔ‡

ÂϤÁ¯Ô˘ ÁÈ· Ì›· ÓfiÛÔ, ÌÔÚ› Ó· ·ÔÎ·Ï˘Êı›

ÏËÚÔÊÔÚ›· Û¯ÂÙÈο Ì ̛· ¿ÏÏË ÓfiÛÔ. ∏ ‰È¿-

ÁÓˆÛË Ù˘ ‰Â‡ÙÂÚ˘ ÓfiÛÔ˘ ÌÔÚ› Ó· ›ӷÈ

·Úfi‚ÏÂÙË 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· ÂÚÈ-

Ï·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˜, fiˆ˜

ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ‰È·ÁÓˆÛÙÈΤ˜ ‹ ıÂڷ¢ÙÈΤ˜

·ÚÂÌ‚¿ÛÂȘ ÁÈ· Ù· ·È‰È¿, ··ÈÙ› Û˘Áηٿ-

ıÂÛË ·fi ÙÔ˘˜ ÏËÚÔÊÔÚË̤ÓÔ˘˜ ÁÔÓ›˜ ηÈ

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 ™ÂÏ›‰·391

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392 Ã. ÷Ù˙ËÛ‚·ÛÙÔ‡ - §Ô˘Î›‰Ô˘

·È‰È¿ Î·È ÂÊ‹‚Ô˘˜, ηıÒ˜ ¯ÚÂÈ¿˙ÂÙ·È ÂÈϤ-

ÔÓ ¤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· ·ÍÈÔ-

ÏÔÁËıÔ‡Ó ÚÔÛÂÎÙÈο Ì ÂÏÂÁ¯fiÌÂÓ˜ ÎÏÈÓÈΤ˜

‰ÔÎÈ̤˜. ∆· ·ÓȯÓ¢ÙÈο ÚÔÁÚ¿ÌÌ·Ù· ÂϤÁ¯Ô˘

ÊÔÚ¤ˆÓ ÌÔÚ› Ó· Â›Ó·È Î·Ù¿ÏÏËÏ· ÁÈ· ¤ÁÎ˘Â˜

ÂÊ‹‚Ô˘˜ ‹ ÁÈ· ÂΛӘ Ô˘ ÛΤÊÙÔÓÙ·È ÙËÓ ÙÂ-

ÎÓÔÔ›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 ÙÂ-

ÏÈ΋ ·fiÊ·ÛË. £ÂˆÚÂ›Ù·È fiÙÈ Ô ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ-

¯Ô˜ ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ ÁÈ· ÙËÓ Úfi-

ÏË„Ë ÙˆÓ ÓÔÛËÌ¿ÙˆÓ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ·ÚÁfi-

ÙÂÚ· Â›Ó·È ·Î·Ù¿ÏÏËÏÔ˜, ·ÊÔ‡ Ë ÁÂÓÂÙÈ΋ ÏË-

ÚÔÊÔÚ›· 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 ÚÔ-

ÁÚ¿ÌÌ·Ù· ÌÔÚ› 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 ÌÂ

ÙȘ ÂÚÈÔÚÈṲ̂Ó˜ ÁÂÓÂÙÈΤ˜ ÁÓÒÛÂȘ Î·È ıÂÚ·-

¢ÙÈΤ˜ ‰˘Ó·ÙfiÙËÙ˜ ÁÈ· ¤Ó·Ó ÌÂÁ¿ÏÔ ·ÚÈıÌfi

ÁÂÓÂÙÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ. ∂›Û˘, ˘¿Ú¯ÂÈ Èı·-

ÓfiÙËÙ· ÂÈ‚¿Ú˘ÓÛ˘, Ë ÔÔ›· ÌÔÚ› Ó· ÚÔ-

ÎÏËı› ·fi ÙËÓ ÂÍÂȉÈÎÂ˘Ì¤ÓË ÁÂÓÂÙÈ΋ ÏËÚÔ-

ÊÔÚ›·, ÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Ù˘ Èı·ÓfiÙËÙ·˜

„˘¯ÔÏÔÁÈ΋˜ ‚Ï¿‚˘ Î·È ÛÙÈÁÌ·ÙÈÛÌÔ‡. OÈ ·È-

‰›·ÙÚÔÈ ÌÔÚ› Ó· ‚ÔËıÔ‡Ó ÛÙÔÓ ¯ÂÈÚÈÛÌfi ÔÏ-

ÏÒÓ ıÂÌ¿ÙˆÓ Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙÔÓ ÁÂÓÂÙÈ-

Îfi ¤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˘

·ÓıÚÒÔ˘ 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 ÂÈÌfiÚʈÛ˘ ÁÈ· ÙÔ˘˜ ·ÁÁÂÏ̷ٛ˜ ÁÂ-

ÓÂÙÈÛÙ¤˜ (11).

µÈ‚ÏÈÔÁÚ·Ê›·

1. Guttmacher AE, Collins FS. Genomic medicine - a

primer. N Engl J Med 2002;347:1512-1520.

2. Clayton EW. Ethical, legal, and social implications of

genomic medicine. N Engl J Med 2003;349:562-569.

3. American Academy of Pediatrics, Committee on

Bioethics. Ethical issues with genetic testing in pedi-

atrics. Pediatrics 2001;107:1451-1455.

4. Major DA, Sane DC, Herrington DM. Cardiovascu-

lar implications of the factor V Leiden mutation.

Am Heart J 2000;140:189-195.

5. Khoury MJ, McCabe LL, McCabe ER. Population

screening in the age of genomic medicine. N Engl J

Med 2003;348:50-58.

6. Armstrong K, Eisen A, Weber B. Assessing the risk

of breast cancer. N Engl J Med 2000;342:564-571.

7. Kuusisto J, Koivisto K, Kervinen K, Mykkanen L,

Helkala EL, Vanhanen M et al. Association of

apolipoprotein E phenotypes with late onset

Alzheimer’s disease: population based study. BMJ

1994;309:636-638.

8. Campion D, Dumanchin C, Hannequin D, Dubois

B, Belliard S, Puel M et al. Early-onset autosomal

dominant Alzheimer disease: prevalence, genetic

heterogeneity, and mutation spectrum. Am J Hum

Genet 1999;65:664-670.

9. American Academy of Pediatrics, Committee on Ge-

netics. Molecular genetic testing in pediatric practice:

a subject review. Pediatrics 2000;106:1494-1497.

10. Noguchi P. Risks and benefits of gene therapy. N

Engl J Med 2003;348:193-194.

11. Chesney RW, Friedman A, Kanto WP Jr, Stanton BF,

Stull TL. Pediatric practice and education in the ge-

nomic/postgenomic era. J Pediatr 2002;141:453-458.

12. American Academy of Pediatrics, Committee on

Genetics. Newborn screening fact sheets. Pediatrics

1996;98:473-501.

13. Scotet V, de Braekeleer M, Roussey M, Rault G, Par-

ent P, Dagorne M et al. Neonatal screening for cys-

tic fibrosis in Brittany, France: assessment of 10

years’ experience and impact on prenatal diagnosis.

Lancet 2000;356:789-794.

14. Farrell PM, Kosorok MR, Rock MJ, Laxova A, Zeng

L, Lai HC et al. Early diagnosis of cystic fibrosis

through neonatal screening prevents severe malnu-

trition and improves long-term growth. Wisconsin

Cystic Fibrosis Neonatal Screening Study Group.

Pediatrics 2001;107:1-13.

15. Merelle ME, Huisman J, Alderden-van der Vecht A,

Taat F, Bezemer D, Griffioen RW et al. Early versus

late diagnosis: psychological impact on parents of

children with cystic fibrosis. Pediatrics 2003;111:

346-350.

16. Mischler EH, Wilfond BS, Fost N, Laxova A, Reiser

C, Sauer CM et al. Cystic fibrosis newborn screening:

impact on reproductive behavior and implications

for genetic counseling. Pediatrics 1998;102:44-52.

17. Parad RB. Buccal cell DNA mutation analysis for di-

agnosis of cystic fibrosis in newborns and infants in-

accessible to sweat chloride measurement. Pediatrics

1998;101:851-855.

18. American Academy of Pediatrics, Committee on

Bioethics. Informed consent, parental permission,

and assent in pediatric practice. Pediatrics 1995;95:

314-317.

19. Mehl AL, Thomson V. The Colorado newborn hear-

ing screening project, 1992-1999: on the threshold of

effective population-based universal newborn hear-

ing screening [abstract]. Pediatrics 2002;109:134.

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393¶ÚfiÏË„Ë Î·È ËıÈ΋

20. Cohn ES, Kelley PM. Clinical phenotype and muta-tions in connexin 26 (DFNB1/GJB2), the most com-mon cause of childhood hearing loss. Am J MedGenet 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 musculardystrophy: a psychosocial study. Arch Dis Child Fe-tal Neonatal Ed 2002;86:F91-F95.

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

¶·È‰È·ÙÚÈ΋ 2005;68:386-393

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394 SPECIAL ARTICLE

1st Paediatric Clinic, AristotleUniversity of Thessaloniki, “Ippokration” GeneralHospital, Thessaloniki

Correspondence:

H. Hatzissevastou-Loukidou Laboratory of Cytogenetics, 1st Paediatric Clinic, AristotleUniversity 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

Genetic preventive testing and ethical issues

H. Hatzissevastou - Loukidou

Abstract

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

Key words

DNA tests, ethical issues, cystic fibrosis, immunoreactive trypsinogen, Duchenne musculardystrophy.

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395∂¶π™∆O§∏ ¶ƒO™ ∆∏ ™À¡∆∞•∏ LETTER TO THE EDITOR

¶·È‰È·ÙÚÈ΋ 2005;68:395

∏ ∂ÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ ∂Ù·ÈÚ›· ›¯Â ÙË ¯·Ú¿ Ó· ·ÔÎÙ‹ÛÂÈ ¤Ó· ·ÍÈfiÏÔÁÔ ÓÙÔÎÈÌ·ÓÙ¤Ú ÙÔ˘ ΢ڛ-Ô˘ ¡¤ÛÙÔÚ· ª¿ÙÛ·, Ô˘ Á˘Ú›ÛÙËΠÙË ‰ÂηÂÙ›· ÙÔ˘ ’70, Ì ı¤Ì·: “∞˘Ùfi ÙÔ ·È‰› ÁÂÓÓ‹ıËΠ·‡ÚÈÔ”.

∆Ô ÓÙÔÎÈÌ·ÓÙ¤Ú ·˘Ùfi ÚÔ‚Ï‹ıËΠÛÙËÓ ÙÂÏÂÙ‹ Ï‹Í˘ ÙÔ˘ 43Ô˘ ¶·ÓÂÏÏËÓ›Ô˘ ¶·È‰È·ÙÚÈÎÔ‡ ™˘ÓÂ-‰Ú›Ô˘ ÛÙËÓ ∫ˆ, ÙÔ ÚÔÏfiÁÈÛÂ Ô ›‰ÈÔ˜ Ô Î‡ÚÈÔ˜ ª¿ÙÛ·˜ Î·È ·¤Û·Û ٷ ηχÙÂÚ· Û¯fiÏÈ·.

O ·ÚÈÔ˜ ª¿ÙÛ·˜ Ì¿˜ ¤ÛÙÂÈÏ ÙËÓ ·ÎfiÏÔ˘ıË ÂÈÛÙÔÏ‹.

∫ˆÓÛÙ·ÓÙ›ÓÔ˜ ™ÙÂÊ·Ó›‰Ë˜ ¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘

∆Ô ·È‰› ·’ ÙÔÓ ·˘ÚÈ·Ófi ÎfiÛÌÔ

¡¤ÛÙÔÚ·˜ ª¿ÙÛ·˜

ŒÓ· Û˘Ó¤‰ÚÈÔ ¿ÏÏˆÓ ‰È·ÛÙ¿ÛˆÓ. ªÂ ÙÔ ÈÔ ÂӉȷʤÚÔÓ ı¤Ì· Ô˘ ÌÔÚ› Ó· ·ÓÙÈÌÂÙˆ›˙ÂÈ Ë ÂÔ-¯‹ Ì·˜. ∆Ô ·È‰›. ∆Ô ·È‰› ÛÙËÓ ÒÚ· Ù˘ ‰ÔÎÈÌ·Û›·˜, Ù˘ ·ÚÚÒÛÙÈ·˜, ·ÏÏ¿ Î·È ÛÙËÓ ÒÚ· Ô˘ ÂÌÓ¤ÂÈ ÌÂÙËÓ ·ÚÔ˘Û›· ÙÔ˘ ÙÔÓ ÂÒÓ˘ÌÔ ‹ ·ÓÒÓ˘ÌÔ ‰ËÌÈÔ˘ÚÁfi ÁÈ· Ó· ÌÂÙÔ˘ÛÈÒÛÂÈ ÙË Û˘ÁΛÓËÛ‹ ÙÔ˘ Û ٤¯ÓË.

∏ ·ÎÚÈÙÈ΋ ∫ˆ˜ ¤˙ËÛ ÁÈ· ÙÚÂȘ Ë̤Ú˜ ÙÔÓ ‰ËÌÈÔ˘ÚÁÈÎfi ˘ÚÂÙfi ·˘ÙÔ‡ ÙÔ˘ ™˘ÌÔÛ›Ô˘, Ô˘ ·Ó·ÊÂ-ÚfiÙ·Ó Û fiÏ· Ù· ÚÔ‚Ï‹Ì·Ù· ÙÔ˘ ·È‰ÈÔ‡, fiˆ˜ ·˘Ù¿ ‰È·ÌÔÚÊÒıËÎ·Ó Î·È ‰È·ÌÔÚÊÒÓÔÓÙ·È ÛÙÔ˘˜ ηÈ-ÚÔ‡˜ Ì·˜.

∫ÔÚ˘Ê·›ÔÈ ·È‰›·ÙÚÔÈ, ·ÏÏ¿ Î·È ÓÂfiÙÂÚÔÈ ÂÈÛÙ‹ÌÔÓ˜ Ô˘ ·Û¯ÔÏÔ‡ÓÙ·È Û ‚¿ıÔ˜ Ì ÙÔ ı¤Ì·, η-Ù¤ıÂÛ·Ó ÙȘ ·fi„ÂȘ ÙÔ˘˜, ¤ÙÛÈ ÒÛÙ ӷ ‰ËÌÈÔ˘ÚÁËı› ¤Ó· Îϛ̷ Â˘Ú‡Ù·ÙÔ˘ ÂӉȷʤÚÔÓÙÔ˜ Á‡Úˆ ·fiÙÔ ·È‰› Û‹ÌÂÚ·.

∆ËÓ ÔÚÁ¿ÓˆÛË Â›¯Â Ë ¶·È‰È·ÙÚÈ΋ ∂Ù·ÈÚ›· Î·È ‹Ù·Ó ·ÈÛ›ˆ˜ ÙÔ 43Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ. ™Â ·˘Ù¿ Ù· ¯ÚfiÓÈ·, Ô˘ ÚÔÛÂÁÁ›˙Ô˘Ó ÙÔÓ ÌÈÛfi ·ÈÒÓ·, Ë ¶·È‰È·ÙÚÈ΋ ÚÔ¯ÒÚËÛ Ì ¿ÏÌ·Ù· Û ÙÔ-

Ì›˜ Î·È ¯ÒÚÔ˘˜ Ô˘ ‰ÂÓ ı· ÙÔÏÌÔ‡Û·Ó Ó· ˘Ô„È·ÛÙÔ‡Ó Ô‡Ù ÔÈ ÚˆÙÔfiÚÔÈ È‰Ú˘Ù¤˜ Ù˘, fiÙ·Ó ·Ô-Ê¿ÛÈÛ·Ó ·fi ÎÔÈÓÔ‡ Ó· Û˘ÛÙ‹ÛÔ˘Ó ÙËÓ ∂Ù·ÈÚ›·. ™‹ÌÂÚ· Â›Ó·È ¤Ó·˜ ı·ÏÂÚfi˜ ÔÚÁ·ÓÈÛÌfi˜ ¤Ú¢ӷ˜ ηÈÌÂϤÙ˘ Ù˘ ·È‰È·ÙÚÈ΋˜, Ì ¤Ó·Ó ‰ËÌÈÔ˘ÚÁÈÎfi Úfi‰ÚÔ, ÙÔÓ Î. ∞Ó‰Ú¤· ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ, ÛÙÔÓ ÔÔ›ÔÂÓ ÔÏÏÔ›˜, ·ÏÏ¿ Î·È ÛÙÔ˘˜ ·˘ÛÙËÚ¿ ÂÈÏÂÁ̤ÓÔ˘˜ Û˘ÓÂÚÁ¿Ù˜ ÙÔ˘, ÔÊ›ÏÂÙ·È Ë ÂÈÙ˘¯›· ÙÔ˘ Û˘Ó‰ڛ-Ô˘ Ì ÙË Û˘ÌÌÂÙÔ¯‹ ¯ÈÏ›ˆÓ ÔÎÙ·ÎÔÛ›ˆÓ Û˘Ó¤‰ÚˆÓ!

°È· ÂÌ¿˜, ÙÔ˘˜ ¿Û¯ÂÙÔ˘˜ ı· ÙÔÏÌÔ‡Û· Ó· ˆ, Ì ÙËÓ ÈÂÚfiÙËÙ· ÙÔ˘ ı¤Ì·ÙÔ˜, ·˘Ù‹ Ë ·ıÚfi· ÚÔÛ¤-Ï¢ÛË Î·È Û˘ÌÌÂÙÔ¯‹ ‹Ù·Ó Ú·ÁÌ·ÙÈο ¤Ó· ÁÂÁÔÓfi˜. ∫˘Ú›ˆ˜ ÔÈ ÂÈÛÙ‹ÌÔÓ˜ Ô˘ Û˘ÌÌÂÙ›¯·Ó (›¯· Ù˯·Ú¿ Ó· Û˘˙ËÙ‹Ûˆ Ì ÌÂÚÈÎÔ‡˜ ·fi ·˘ÙÔ‡˜) ‹Ù·Ó Ó¤ÔÈ ¿ÓıÚˆÔÈ Ì ÈÂÚfi ¿ıÔ˜ ÁÈ· ÙËÓ ÂÈÛÙ‹ÌË ÙÔ˘˜,ÌÈ· ÂÈÛÙ‹ÌË Ô˘ ‰ÂÓ ÛÙ·Ì·Ù¿ Ô˘ıÂÓ¿ Î·È ‰ÂÓ ı· ÛÙ·Ì·Ù‹ÛÂÈ Ô˘ıÂÓ¿.

™ÙÔÈ¯Â›Ô ·ÈÛÈfi‰ÔÍÔ ÁÈ· ηÈÚÔ‡˜ Ô˘, fiˆ˜ ÚÔ·Ó¤ÊÂÚ·, ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ¿ÚÓËÛË Î·È ·ÌÊÈÛ‚‹-ÙËÛË. µÏ¤ÔÓÙ¿˜ ÙÔ˘˜ Ó· ·Ú·ÎÔÏÔ˘ıÔ‡Ó Ì ÙfiÛÔ ÂӉȷʤÚÔÓ ÙÔ ™˘Ó¤‰ÚÈÔ Î·È Ó· οÓÔ˘Ó Î·›ÚȘ ·Ó·-ÎÔÈÓÒÛÂȘ, ı˘ÌfiÌÔ˘Ó Î·È Í·Ó·ı˘ÌfiÌÔ˘Ó ÙÔÓ ÏfiÁÔ ÙÔ˘ ÌÂÁ¿ÏÔ˘ ·ÓıÚˆÈÛÙ‹ ÁÈ·ÙÚÔ‡ ∞Ï‚¤ÚÙÔ˘ ™‚¿È-ÙÛÂÚ: “O ÔÏÈÙÈÛÌfi˜ ÌÈ·˜ ¯ÒÚ·˜ ÂÎÊÚ¿˙ÂÙ·È ·fi ÙËÓ ·Á¿Ë Î·È ÙËÓ ÚÔÛÙ·Û›· Ô˘ ‰›ÓÂÈ ÛÙÔ ·È‰›”.

∂›Ó·È ÁÓˆÛÙfi fiÙÈ Ô ™‚¿ÈÙÛÂÚ ÂÁη٤ÏÂÈ„Â Ì›· Ï·ÌÚ‹ ηÚȤڷ ÛÙËÓ ∂˘ÚÒË, Ô˘ ‹Ù·Ó ÂÍ›ÛÔ˘ ÛÔ˘-‰·›Ô˜ ÂÈÛÙ‹ÌÔÓ·˜ fiÛÔ Î·È ÌÔ˘ÛÈÎfi˜, ÁÈ· Ó· ·ÊÔÛȈı› ÛÙ· ÏÂÚ¿ ·È‰È¿ Ù˘ ∞ÊÚÈ΋˜. ∞‰È·ÊÔÚÒÓÙ·˜ÁÈ· fiÏ·, ÌÂÙÔ˘Û›ˆÛ ÙË ‰ˆÚ¿ Ô˘ ÙÔ˘ ¤‰ˆÛÂ Ô £Âfi˜ Û ÚÔÛÊÔÚ¿ ·Á¿Ë˜ ÁÈ· ÙÔ ·ÓÒÓ˘ÌÔ Ì·‡ÚÔ ·È-‰›, Ô˘ ˙Ô‡Û οو ·fi ÚˆÙfiÁÔÓ˜ Û˘Óı‹Î˜, Ì ·Ê¿ÓÙ·ÛÙ˜ ÛÙÂÚ‹ÛÂȘ, ·ÎfiÌË Î·È Ì ¤ÏÏÂÈ„Ë ÓÂÚÔ‡.

¢˘ÛÙ˘¯Ò˜, ÔÈ Û˘Óı‹Î˜, ı· ÚÔÛ¤ıÂÙ· ÂÁÒ, ‰ÂÓ ¿ÏÏ·Í·Ó ·fi ÙfiÙÂ Î·È Ù· ÛÙÂÚË̤ӷ ·˘Ù¿ ·È‰È¿·ÓÙÈÌÂÙˆ›˙Ô˘Ó ÙȘ ›‰È˜ ‰ÔÎÈ̷ۛ˜ ÂÈ‚›ˆÛ˘, Ì ̛· ÚfiÛıÂÙË Ô˘ Â›Ó·È Î·È ¯ÂÈÚfiÙÂÚË: ÙËÓ ·ÁˆÓ›·Ù˘ ·È‰ÂÚ·ÛÙ›·˜ ·fi ηÏÔ˙ˆÈṲ̂ÓÔ˘˜ ‚¿Ú‚·ÚÔ˘˜ ∂˘Úˆ·›Ô˘˜ Ô˘ ‰ÂÓ Û¤‚ÔÓÙ·È Ô‡Ù ÙËÓ ÙÚ·Áˆ‰›··˘ÙÒÓ ÙˆÓ ‰˘ÛÙ˘¯ÈÛÌ¤ÓˆÓ fiÓÙˆÓ.

∫·Ù¿ ÙÔ‡ÙÔ, ·˘Ù¿ Ù· Û˘Ó¤‰ÚÈ·, ˆ˜ ¿ÌÂÛ· ‹ ¤ÌÌÂÛ·, ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ ÈÔ Â˘·›ÛıËÙÔ, fiˆ˜ ÙÔ ¯·Ú·-ÎÙ‹ÚÈÛ·, ı¤Ì· ÙˆÓ Î·ÈÚÒÓ Ì·˜, ÙÔ ·È‰›, ÚÔÛʤÚÔÓÙ·˜ ¤Ó· ̤ÁÈÛÙÔ ËıÈÎfi Î·È ÎÔÈÓˆÓÈÎfi fiÊÂÏÔ˜ ÁÈ·ÙÔ˘˜ ÌË ÛÎÂÙfiÌÂÓÔ˘˜ ‹ ÙÔ˘˜ ·‰È·ÊÔÚÔ‡ÓÙ˜.

∆Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘ÌfiÛÈÔ Ù˘ ∫ˆ, ÔÚÁ·ÓˆÌ¤ÓÔ Ì ÙÔÓ ÈÔ ¿ÚÙÈÔ ÙÚfiÔ ·fi ÙÔÓ ¶Úfi‰ÚÔ Î·È ÙÔ ¢È-ÔÈÎËÙÈÎfi ™˘Ì‚Ô‡ÏÈÔ Ù˘ ¶·È‰È·ÙÚÈ΋˜ ∂Ù·ÈÚ›·˜, ‹Ù·Ó Ì›· ·ÊÔÚÌ‹ ÁÈ· ‚·ı‡ Î·È Ô˘ÛÈ·ÛÙÈÎfi ÚÔ‚ÏËÌ·-ÙÈÛÌfi Á‡Úˆ ·fi Ù· ı¤Ì·Ù· (Î·È Â›Ó·È ÙfiÛ· ÔÏÏ¿) Ô˘ ··Û¯ÔÏÔ‡Ó ÙÔ ¶·È‰› Î·È Â˘Ú‡ÙÂÚ· ÙËÓ ÂÓ ‰È·Ú-Λ ÂÍÂÏ›ÍÂÈ Î·È ¤Ú¢ӷ ¶·È‰È·ÙÚÈ΋ ∂ÈÛÙ‹ÌË.

∆Ô ·È‰› Ô˘ ¤Ú¯ÂÙ·È ·’ ÙÔÓ ∞˘ÚÈ·Ófi ∫fiÛÌÔ ¤¯ÂÈ Î¿ı ÓfiÌÈÌÔ ‰Èη›ˆÌ· Û ·˘Ù‹ ÙËÓ ·Í›ˆÛË.

Pediatr Sept-Oct 05 17-10-05 16:52 ™ÂÏ›‰·395

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396 ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√

µ‘ ¶·È‰È·ÙÚÈ΋¶·ÓÂÈÛÙËÌȷ΋ ∫ÏÈÓÈ΋,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”, ∞ı‹Ó·

AÏÏËÏÔÁÚ·Ê›·:

¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜E-mail: [email protected]

¶·È‰È·ÙÚÈ΋ 2005;68:396-397

¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜, ¶·È‰›·ÙÚÔ˜ - ∞ÏÏÂÚÁÈÔÏfiÁÔ˜

ª·ı·›ÓÔÓÙ·˜ ÙÔÓ ÎfiÛÌÔ ÙˆÓ ¡¤ˆÓ ∆¯ÓÔÏÔÁÈÒÓ - http://www.uptoten.com

ª›· ÔÌ¿‰· ·fi ÂÓıÔ˘ÛÈÒ‰ÂȘ ·ÁÁÂÏ̷ٛ˜Ì ¤‰Ú· ÙÚ›· ÛËÌ›· Ù˘ Á˘ (∏¶∞, °·ÏÏ›· ηȪ·˘Ú›ÎÈÔ) ÎÚ‡‚ÂÙ·È ›Ûˆ ·fi ÙÔÓ ‰ÈÎÙ˘·Îfi ÙfiÔUp∆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· °Ô-Ó›˜” Î·È “°È· ¶·È‰È¿”, Ì ‰È·ÊÔÚÂÙÈÎfi ÂÚȯfi-ÌÂÓÔ ·Ó¿ÏÔÁ· Ì ÙÔ ÎÔÈÓfi ÛÙÔ ÔÔ›Ô ·Â˘ı‡ÓÂ-Ù·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·ÏÂÎÙÈÎfiÙÚfiÔ Î·È Ú·ÎÙÈο ˙ËÙ‹Ì·Ù· ÂÁÁÚ·Ê‹˜. ∏ ηÙË-ÁÔÚ›· “°È· ¶·È‰È¿” ·ÔÙÂÏ› Î·È ÙÔ ÎÂÓÙÚÈÎfiÛÙÔÈ¯Â›Ô ÙÔ˘ Up∆oTen. ¢‡Ô ‚·ÛÈÎÔ› ¯·Ú·ÎÙ‹Ú˜,Ô Boowa - Ô ÔÔ›Ô˜ ·ÓÙÈÚÔۈ‡ÂÈ ÙÔÓ ÂÓ‹ÏÈÎÔÌ ˘Â‡ı˘ÓË Î·È ÒÚÈÌË Û˘ÌÂÚÈÊÔÚ¿ - Î·È Ë ÌÈ-ÎÚ‹ Kwala - Ë ÔÔ›· Â›Ó·È ÈÔ ˙ˆËÚ‹ Î·È ÂÈÚÚÂ-‹˜ ÛÙȘ ÛηӉ·ÏȤ˜ - ÚˆÙ·ÁˆÓÈÛÙÔ‡Ó Û fiϘÙȘ ˘ÔηÙËÁÔڛ˜, ÔÈ Ôԛ˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‰È·-‰ÈÎÙ˘·Î¿ ·È¯Ó›‰È·, ËÏÂÎÙÚÔÓÈÎfi ηٿÛÙËÌ· Ì·ȯӛ‰È·, ÚÔ‡¯·, ‚È‚Ï›·, ·Ê›Û˜ Î.¿. O ÙÔ̤·˜

ÙˆÓ ·È¯ÓȉÈÒÓ Â›Ó·È È‰È·›ÙÂÚ· ·ÓÂÙ˘Á̤ÓÔ˜ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ ÂÚÈÛÛfiÙÂÚ· ·fi 700 ÂÎ·È‰Â˘ÙÈ-ο ·È¯Ó›‰È· Ì ‰ˆÚÂ¿Ó ÚfiÛ‚·ÛË, ηıÒ˜ Î·È ÚÔÙ¿ÛÂȘ ÁÈ· ‰Ú·ÛÙËÚÈfiÙËÙ˜, Ù· ÔÔ›· ·Â˘ı‡-ÓÔÓÙ·È Û ‰È¿ÊÔÚÔ˘˜ ËÏÈÎÈ·ÎÔ‡˜ Û˘Ó‰˘·ÛÌÔ‡˜, ·Ó¿ÏÔÁ· Ì ÙȘ ··ÈÙ‹ÛÂȘ ÙÔ˘ οı ·È¯ÓȉÈÔ‡.

∏ ÏÔ‹ÁËÛË ÛÙÔÓ ÈÛÙfiÙÔÔ “°È· ¶·È‰È¿” Â›Ó·È ·Ï‹ Î·È ‰È·ÛΉ·ÛÙÈ΋, Ì ̛· ‡¯ÚËÛÙË ªË-¯·Ó‹ ∞Ó·˙‹ÙËÛ˘ ÛÙȘ ÂÈ̤ÚÔ˘˜ ÂÓfiÙËÙ˜.

O ‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ Up∆oTen ¤¯ÂÈ ÌÂÁ¿ÏË ÂÈÛ΄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˜ ÙfiÔ˜ Shockwave.com ·ÔÙÂÏ› ¤Ó·Ó ·fi ÙÔ˘˜ ÚˆÙÔfiÚÔ˘˜ÛÙÔÓ ÙÔ̤· ÙˆÓ ËÏÂÎÙÚÔÓÈÎÒÓ ·È¯ÓȉÈÒÓ (multimedia games) ̤ۈ ÙÔ˘ ‰È·-

‰ÈÎÙ‡Ô˘. ∞Ó‹ÎÂÈ ÛÙÔÓ ·ÌÂÚÈηÓÈÎfi fiÌÈÏÔ AtomShockwave Corp, Ì ̤ÛË ÌËÓÈ·›· ÂÈÛ΄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 ÂÚÈ-Ï·Ì‚¿ÓÂÈ Ì›· ¢Ú›· Áο̷ ·È¯ÓȉÈÒÓ, Ù· ÔÔ›· ·Ó·ÓÂÒÓÔÓÙ·È Û ‚‰ÔÌ·‰È·›· ‚¿ÛË. ∆· ·È¯Ó›‰È·

shockwave.com

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397NEWS FROM THE INTERNET

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·Ùfi-ÙËÙ· ÏÔ‹ÁËÛ˘ ÛÂ Û˘Óԉ¢ÙÈÎfi ÔÙÈÎÔ·ÎÔ˘ÛÙÈÎfi ˘ÏÈÎfi, fiˆ˜ 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˘ ÌÔÚ› Ó· ÚÔÌËı¢Ù› Ù· ÂÚÈÛÛfiÙÂÚ· ·fi ·˘Ù¿ ·fi ÙÔ ÂȉÈÎfi ηٿÛÙËÌ· ·ÁÔÚÒÓ, ÛÂÏÔÁÈΤ˜ 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˜ ÙfiÔ˜ 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 ·Ï¤˜ ÂÊ·ÚÌÔÁ¤˜ - ̤¯ÚÈ ÙȘ ÙÂÏÂ˘Ù·›-˜ 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,fiˆ˜ 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· ÔÔ›· ÌÔÚÔ‡Ó Ó· ·Ô-ıË΢ÙÔ‡Ó Î·È Ó· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó Î·È ÂÎÙfi˜ ‰È·‰È-ÎÙ‡Ô˘. ¶ÚÈÓ ÙËÓ ÂÎΛÓËÛË ÂÓfi˜ Ó¤Ô˘ ·È¯ÓȉÈÔ‡, Ô¯Ú‹ÛÙ˘ ‰È·‚¿˙ÂÈ ÙȘ Û‡ÓÙÔ̘ Ô‰ËÁ›Â˜ ¯Ú‹Û˘ Î·È ÂÓÛ˘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·˜ Ùˉ˘Ó·ÙfiÙËÙ· ÛÙÔÓ ¯Ú‹ÛÙË Ó· ‰ÔÎÈÌ¿ÛÂÈ fiÛÔ ÙÔ ‰˘Ó·ÙfiÓÂÚÈÛÛfiÙÂÚ·.

¶·È‰È·ÙÚÈ΋ 2005;68:396-397

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¢È·Ù·Ú·¯¤˜ Û¯ÔÏÈ΋˜ Ì¿ıËÛ˘ Î·È „˘¯Ô·ıÔÏÔÁ›·

Àfi ¢.∫. ∞Ó·ÁÓˆÛÙfiÔ˘ÏÔ Î·È ∞.£. ™›ÓË

∂ΉfiÛÂȘ µ∏∆∞

∞ı‹Ó·, 2004

∆Ô ‚È‚Ï›Ô ÙˆÓ ¢.∫. ∞Ó·ÁÓˆÛÙfiÔ˘ÏÔ˘ Î·È ∞.£. ™›ÓË Ú·ÁÌ·Ù‡Â-Ù·È ÙÔ ı¤Ì· ÙˆÓ ‰È·Ù·Ú·¯ÒÓ Ù˘ Ì¿ıËÛ˘, ÁÓˆÛÙÒÓ ÛÙÔ Â˘Ú‡ ÎÔÈÓfiˆ˜ ‰˘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·ÙÚԷȉ·ÁˆÁÈο ∫¤-ÓÙÚ· Î·È Ù· ∫¤ÓÙÚ· ¢È¿ÁÓˆÛ˘, ∞ÍÈÔÏfiÁËÛ˘ Î·È ÀÔÛÙ‹ÚÈ͢(∫.¢.∞.À.) Ì ·›ÙËÌ· Ó· ¿ÚÂÈ ÙÔ ·È‰› ÙÔ˘˜ ‚‚·›ˆÛË fiÙÈ ¤¯ÂÈ Úfi-‚ÏËÌ· ‰˘ÛÏÂÍ›·˜, ÔfiÙ - ‚¿ÛÂÈ ÙÔ˘ ÓfiÌÔ˘ - ¤¯ÂÈ ÙÔ ‰Èη›ˆÌ· Ó· ˙Ë-Ù‹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·ÓÙÈ-Τ˜ ‰˘ÛÎÔϛ˜ ÛÙËÓ ÂÎÌ¿ıËÛË Ù˘ ·Ó¿ÁÓˆÛ˘, Ù˘ ÁÚ·Ê‹˜, Ù˘ ÔÚıÔ-ÁÚ·Ê›·˜ Î·È ÔÚÈṲ̂Ó˜ ÊÔÚ¤˜ Î·È Ù˘ ·ÚÈıÌËÙÈ΋˜. OÈ ‰˘ÛÎÔϛ˜ ·˘Ù¤˜‰ÂÓ ÔÊ›ÏÔÓÙ·È Û - ·Ó Î·È ÌÔÚ› 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˘-ηÈÚÈÒÓ 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΋ ·ÓÙÈÌÂÙÒÈÛË, ÌÔÚÔ‡Ó Ó·

ÔÏÔÎÏËÚÒÛÔ˘Ó Ì ÂÈÙ˘¯›· 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΋ ÂÌÂÈÚ›· Û¯ÂÙÈο

Ì ÙÔ ı¤Ì· ÙˆÓ ‰È·Ù·Ú·¯ÒÓ Ù˘ Ì¿ıËÛ˘. O ·ÚÈÔ˜ ∞Ó·ÁÓˆÛÙfiÔ˘ÏÔ˜ Â›Ó·È ·È‰Ô„˘¯›·ÙÚÔ˜, ˘Â‡ı˘ÓÔ˜Ù˘ ÀËÚÂÛ›·˜ 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˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ. ∏ ‰È‰·ÎÙÔÚÈ΋ ÙÔ˘ ‰È·ÙÚÈ‚‹ ·ÊÔ-ÚÔ‡Û ÛÙȘ ‰È·Ù·Ú·¯¤˜ Ù˘ Ì¿ıËÛ˘. ∏ ΢ڛ· ™›ÓË Â›Ó·È „˘¯Ô·È‰·ÁˆÁfi˜ Î·È ÂÚÁ¿˙ÂÙ·È ÛÙËÓ ›‰È· ˘ËÚÂ-Û›· „˘¯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΋ ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘˜. OÈ ‰‡Ô Û˘ÁÁÚ·Ê›˜ Û˘-ÓÂÚÁ¿˙ÔÓÙ·È ÁÈ· ÙË ‰È¿ÁÓˆÛË Î·È ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·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·ÛÎfiËÛË Û¯ÂÙÈ-ο Ì ÙȘ ‰È·Ù·Ú·¯¤˜ Ù˘ Ì¿ıËÛ˘ Î·È ÂÍÂÙ¿˙ÔÓÙ·È - ÌÂٷ͇ ¿ÏÏˆÓ - ı¤Ì·Ù· ÔÚÔÏÔÁ›·˜, ÂȉËÌÈÔÏÔÁ›·˜,‰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‹˜, fiÔ˘ 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˘ÓÓÔ-ÛËÚfiÙËÙ·˜. ™ÙÔ Ù¤Ù·ÚÙÔ Î·È ÙÂÏÂ˘Ù·›Ô ̤ÚÔ˜ ÙÔ˘ ‚È‚Ï›Ô˘ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ù¤ÛÛÂÚȘ ÂÚÈÙÒÛÂȘ ·È‰ÈÒÓÌ ‰È·Ù·Ú·¯¤˜ Ù˘ Û¯ÔÏÈ΋˜ Ì¿ıËÛ˘, ÔÈ Ôԛ˜ ‰Â›¯ÓÔ˘Ó ·Ó¿ÁÏ˘Ê· ÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ·, fiˆ˜ ÙË ‚ÈÒÓÔ˘ÓÙ· ›‰È· Ù· ·È‰È¿.

∆Ô ‚È‚Ï›Ô ·˘Ùfi οÓÂÈ Ì›· Û˘ÓÔÙÈ΋, ·ÏÏ¿ Î·È ÛÊ·ÈÚÈ΋ ·ÚÔ˘Û›·ÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ÙˆÓ ‰È·Ù·Ú·¯ÒÓÙ˘ Ì¿ıËÛ˘, Û˘Ó‰˘¿˙ÔÓÙ·˜ ÌÈ· ηϋ ·Ó·ÛÎfiËÛË Ù˘ ‰ÈÂıÓÔ‡˜ Î·È ÂÏÏËÓÈ΋˜ Û¯ÂÙÈ΋˜ ‚È‚ÏÈÔÁÚ·Ê›·˜ ÌÂÙË Ì·ÎÚfi¯ÚÔÓË ÎÏÈÓÈ΋ ÂÌÂÈÚ›· ÙˆÓ ‰‡Ô Û˘ÁÁڷʤˆÓ ÛÙË ‰È¿ÁÓˆÛË Î·È ÛÙËÓ „˘¯Ô·È‰·ÁˆÁÈ΋ ·ÓÙÈÌÂ-ÙÒÈÛË ÙˆÓ Ì·ıËÛÈ·ÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ. ∆Ô Û‡ÁÁÚ·ÌÌ· ·˘Ùfi ı· ‰ÒÛÂÈ ÛÙÔÓ ·È‰›·ÙÚÔ Ì›· ÔÏÔÎÏËڈ̤ÓËÂÈÎfiÓ· ÙˆÓ Û‡Á¯ÚÔÓˆÓ ÁÓÒÛÂˆÓ Û¯ÂÙÈο Ì ÙË ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Ì·ıËÛÈ·ÎÒÓ ‰È·Ù·Ú·¯ÒÓ.

∂˘ÊÚÔÛ‡ÓË ªfiÙÙË ∫·ıËÁ‹ÙÚÈ·, ∆Ì‹Ì· ºÈÏÔÛÔÊ›·˜, ¶·È‰·ÁˆÁÈ΋˜ Î·È æ˘¯ÔÏÔÁ›·˜ ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ

398 µπµ§πO¶∞ƒOÀ™π∞™∏ BOOK REVIEW

∆Ì‹Ì· ºÈÏÔÛÔÊ›·˜,¶·È‰·ÁˆÁÈ΋˜ ηÈæ˘¯ÔÏÔÁ›·˜ ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ

AÏÏËÏÔÁÚ·Ê›·:

∂˘ÊÚÔÛ‡ÓË ªfiÙÙË E-mail: [email protected]

¶·È‰È·ÙÚÈ΋ 2005;68:398

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∫·Ú‰ÈÔ·Ó·Ó¢ÛÙÈ΋˜ ∞Ó·˙ˆÔÁfiÓËÛ˘ (∂∂∫∞∞¡)

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∞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: [email protected] Website: www.congressworld.gr

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18-19 ª·ÚÙ›Ô˘ 2006 4Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ÀÔÂȉÈÎÔÙ‹ÙˆÓ ∞ı‹Ó·

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: [email protected] Website: www.ped-subspec2006.gr

7-9 ∞ÚÈÏ›Ô˘ 2006 1Ô ¶·ÓÂÏÏ‹ÓÈÔ ¡ÂÔÁÓÔÏÔÁÈÎfi ™˘Ó¤‰ÚÈÔ ∞ı‹Ó·

•ÂÓÔ‰Ô¯Â›Ô Ledra Marriott ¶ÏËÚÔÊÔڛ˜: Triaena Tours ∆ËÏ.: (+30) 210 7499 300 Fax: (+30) 210 7705 752 E-mail: [email protected]

26-29 ∞ÚÈÏ›Ô˘ 2006 XVIIth Annual Congress of the European Society ∞ı‹Ó·

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: [email protected] Website: www.espu2006.com

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2-5 ª·˝Ô˘ 2006 11o ¢ÈÂıÓ¤˜ ™˘Ó¤‰ÚÈÔ ∂Ù·ÈÚ›·˜ æ˘¯ÔÏÔÁÈ΋˜ ∞ı‹Ó·

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 319Fax: (+30) 210 6842 079 E-mail: [email protected] Website: www.epsep.org.gr

3-5 ª·˝Ô˘ 2006 24th Annual Meeting of the European Society Basel,

for Paediatric Infectious Diseases - ESPID Switzerland

¶ÏËÚÔÊÔڛ˜: Kenes International / ESPID 2006 ∆ËÏ.: +41 22 908 0488 Fax: +41 22 732 2850 E-mail: [email protected] Website: www.kenes.com/espid/index2006.asp

10-14 πÔ˘Ó›Ô˘ 2006 XXV Congress of the European Academy of Vienna,

Allergology and Clinical Immunology Austria

¶ÏËÚÔÊÔڛ˜: Congrex Sweden ∆ËÏ.: +46 8 459 66 00 Fax: +46 8 661 91 25 E-mail: [email protected] Website: www.congrex.com/eaaci2006

30 πÔ˘Ó›Ô˘- 45th Annual Meeting of the European Society Rotterdam,

3 πÔ˘Ï›Ô˘ 2006 for Paediatric Endocrinology The Netherlands

¶ÏËÚÔÊÔڛ˜: Congrex Sweden AB ∆ËÏ.: +46 8 459 66 00 Fax: +46 8 661 91 25 E-mail: [email protected] Website: www.espe2006.org

8-11 πÔ˘Ï›Ô˘ 2006 7th International Congress on Pediatric Montreal,

Pulmonology Canada

¶ÏËÚÔÊÔڛ˜: Annie Bidart, MD ∆ËÏ.: +33 0 497 038 597 Fax: +33 0 497 038 598 E-mail: [email protected] Website: www.cipp-meeting.com

Pediatr Sept-Oct 05 17-10-05 16:52 ™ÂÏ›‰·402