Παιδιατρική | Τόμος 69 • Τεύχος 3 • Μάιος - Ιούνιος 2006

Exof Pediatr May-June 06

16-05-06

16:42

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TfiÌÔ˜ 69 ñ ∆‡¯Ô˜ 3 ñ ª¿ÈÔ˜-πÔ‡ÓÈÔ˜ 2006 ¶·È‰È·ÙÚÈ΋ ñ TfiÌÔ˜ 69 ñ ∆‡¯Ô˜ 3 ñ ª¿ÈÔ˜-πÔ‡ÓÈÔ˜ 2006 Volume 69 ñ Number 3 ñ May-June 2006 ISSN 0377-2551

www.paediatriki.edu.gr

Pediatr May-June 06

16-05-06

17:16

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¶·È‰È·ÙÚÈ΋ ∆fiÌÔ˜ 69 ñ ∆‡¯Ô˜ 3 ñ ª¿ÈÔ˜-IÔ‡ÓÈÔ˜ 2006 ¢ÈÌËÓÈ·›· ¤Î‰ÔÛË Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜ ¶Úfi‰ÚÔ˜ ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘ ∫. ™ÙÂÊ·Ó›‰Ë˜ M¤ÏË ¶. ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘ ∂. ∞ÓÙˆÓÔÔ‡ÏÔ˘ A. µ·˙·›Ô˘-°ÂÚ·ÛÈÌ›‰Ë °. µ·ÚÏ¿Ì˘ ª. ∫·Ó¿ÚÈÔ˘ ∂. ∫·ÙÛ·ÚÔ‡-¶ÂÎÙ·Û›‰Ë A. K·ÙÙ¿Ì˘ Ã. ∫ÒÛÙ·ÏÔ˜ ¡. ¶··‰fiÔ˘ÏÔ˜ ∞. ¶··‰ÔÔ‡ÏÔ˘ ∞. ™È·ÌÔÔ‡ÏÔ˘-ª·˘Ú›‰Ô˘ ª. ∆ÛÔÏÈ¿ ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ· ∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈÌ¤ÓˆÓ º. ª·˘ÚÔÂȉ‹ EÈ̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ™. ¡¿ÎÔ˘

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∂¡¢π∞º∂ƒ√À™∂™ ¶∂ƒπ¶∆ø™∂π™

165 Proteus syndrome: a syndrome named after a Greek god J.G. Hall

213 ¡fiÛÔ˜ Niemann-Pick ∆‡Ô˘ µ Ì Û¿ÓÈÔ ÁÔÓfiÙ˘Ô – ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ¡. ª·ÓˆÏ¿ÎË, †∞. ª‹ÙÛÈη, ¢. ™ÌÒÎÔ˘, ∫. ¶Ú›ÊÙ˘, ∞. ºÏÒÎÔ˘, ∂. ªÈ¯ÂϷοÎË

167 ¶fiÛÔ ÂÊ·ÚÌfi˙ÔÓÙ·È ÔÈ ¢ÈÂıÓ›˜ √‰ËÁ›Â˜ ÁÈ· ÙÔ ·È‰ÈÎfi ¿ÛıÌ·; ¡.°. ¶··‰fiÔ˘ÏÔ˜ ∞¡∞™∫O¶∏™∂π™

218 ÀÔÍ›· ÛÎÏËÚ˘ÓÙÈ΋ ·ÓÂÁÎÂÊ·Ï›Ùȉ· ÌÂÙ¿ ·fi ÂÓ‰ÔÌ‹ÙÚÈ· Ïԛ̈ÍË ·fi ÙÔÓ Èfi Ù˘ ÈÏ·Ú¿˜ ª. ¢·ÛÔÔ‡ÏÔ˘, ∞. ∫Ô‚¿Ó˘

169 ªË ÂÂÌ‚·ÙÈΤ˜ ̤ıÔ‰ÔÈ ÂϤÁ¯Ô˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿ Ã. ∫Ô˘ÙÛ·˘Ù›ÎË, ¶. ∆·Ú·ÓÙ˙‹-¶ÔÙ·ÌÈ¿ÓÔ˘, Ã. °ÎÚ¿Ù˙ÈÔ˘, ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜

223 Proteus syndrome: a case report H. Hatzissevastou-Loukidou, E. Michalopoulou-Manoloutsiou, X.M. Stefopoulou

178 ∂Ó‰ÔÎÚ¿ÓÈ· ÓÂÔÏ¿ÛÌ·Ù· Û ·È‰È¿ Ì Ó¢ÚÈӈ̿وÛË Ù‡Ô˘ 1 ¢.π. ∑·ÊÂÈÚ›Ô˘, ∞. µ·Ú‰·ÚÈÓfi˜, ¢. ∫ÔÏÈÔ‡Ûη˜

227 ™‡Ó‰ÚÔÌÔ Saethre-Chotzen Û ·ÛıÂÓ‹ Ì ÌÂÚÈ΋ ÌÔÓÔۈ̛· Ù˘ ¯ÚˆÌÔÛˆÌÈ΋˜ ÂÚÈÔ¯‹˜ 7pter→p15 µ. ∆Ô˘ÏÈ¿ÙÔ˘, ∞. ª·‡ÚÔ˘, ∞. ∫ÔÏȷϤÍË, ∂. ¶··‰ÔÔ‡ÏÔ˘, ¶. ¶·Ó·ÁȈÙÔÔ‡ÏÔ˘, Ã. ∆ÛÂÁ΋, ∂. ∫·Ó·‚¿Î˘, ™. ∫›ÙÛÈÔ˘-∆˙¤ÏË

K. °ÚÈ‚¤·˜

187 √˙҉˘ ÛÎÏ‹Ú˘ÓÛË: ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ÓfiÛÔ˜ ÌÂ Û˘Ó‹ıˆ˜ ‰˘ÛÌÂÓ‹ ÚfiÁÓˆÛË ∫. ¶ÔÏ˘Ì¤ÚË, ∫. ∆ÛÔ˘Ì¿Î·˜

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193 ™˘ÌÌfiÚʈÛË ∂ÏÏ‹ÓˆÓ È·ÙÚÒÓ ÛÙȘ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜ ÁÈ· ÙËÓ ÚfiÏË„Ë Î·È ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿ ¶. •Â··‰¿ÎË, ¡. ¢Ô˘Ï·‰›Ú˘, ª. ª·ÓÔ˘Û¿Î˘, º. ™·ÍÒÓË-¶··ÁˆÚÁ›Ô˘, ¡.°. ¶··‰fiÔ˘ÏÔ˜

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ISSN 0377-2551

199 ∂›‰· ÛÂÏËÓ›Ô˘ Î·È ¯·ÏÎÔ‡ ·›Ì·ÙÔ˜ ÌËÙ¤ÚˆÓ Î·È ÓÂÔÁÓÒÓ ÂÏÏËÓÈ΋˜ Î·È ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ∫. ™Ô‡ÏË, ™. °·‚Ú›ÏË, £. ∫·Ú·ÎˆÓÛÙ·ÓÙ¿Î˘, °. ∫·Ú›Î·˜, °. µÏ¿¯Ô˜, Ã. ∫ÒÛÙ·ÏÔ˜, π. ¶··ÛˆÙËÚ›Ô˘

¶ƒ∞∫∆π∫O £∂ª∞ 233 ¶Ï·ÁÈÔÎÂÊ·Ï›·: Û˘Û¯¤ÙÈÛË Ì ÙË ı¤ÛË ‡ÓÔ˘, ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ∞. ºÈÏÈ›‰Ë, π. °ÂÚÌ·Ó¿Î˘, ª. ∫·ÏÌ·ÓÙ‹ 242 ∫§π¡π∫√ ∫√Àπ∑ ¡. ª·ÚΤ·˜, ∫. µÔ‡‰Ú˘ 243 ∂¶π™∆√§∏ ¶ƒ√™ ∆∏ ™À¡∆∞•∏ 243 ¡∂∫ƒ√§√°π∞ 244 ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√

205 µÈÙ·Ì›ÓË D Û ÌËÙ¤Ú˜ Î·È ‚Ú¤ÊË Ì ·ÔÎÏÂÈÛÙÈÎfi ıËÏ·ÛÌfi ÛÙËÓ ∂ÏÏ¿‰· ∞. ÿÏÏ·, ∞. ¡ÙÔ˘ÚÓÙÔ‡ÊË, µ. ÃÔϤ‚·˜, π. µÏ¿¯Ô˘, ª. ªÈÙÛÒÚË, ∂. °·Ï·Ó¿Î˘, ™. ∞Ó‰ÚÔÓ›ÎÔ˘

∏ ·Ó¿Ù˘ÍË ÛÙÔ ÚÔÛ΋ÓÈÔ ¡. ¶··‰fiÔ˘ÏÔ˜

Pediatr May-June 06

16-05-06

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Paediatriki Volume 69 ñ Number 3 ñ May-June 2006 Bimonthly Publication of the Greek Paediatric Society President A. Constantopoulos Editorial Board Editor-in-Chief 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 Manuscript Editing Greek Editing F. Mavroidi English Editing S. Nakou

Contents EDITORIALS

CASE REPORTS

165 Proteus syndrome: a syndrome named after a Greek god J.G. Hall

213 Niemann-Pick disease type B with a rare genetic profile - A case report N. Manolaki, †A. Mitsika, D. Sbokou, K. Priftis, A. Flokou, E. Michelakaki

167 To what extent International Guidelines on childhood asthma are applied?

N.G. Papadopoulos REVIEW ARTICLES 169 Non-invasive methods of asthma assessment in children C. Koutsaftiki, P. Taprantzi-Potamianou, C. Gratziou, A. Constantopoulos 178 CNS neoplasms in children with type 1 neurofibromatosis D.π. Zafeiriou, ∞. Vardarinos, D. Koliouskas

K. Griveas

187 Tuberous sclerosis complex: a multisystem disease with adverse prognosis C. Polimeri, C. Tsoumakas

Publishing Coordinator

ORIGINAL ARTICLES

SCIENTIFIC PUBLICATIONS Ltd 3 Artemidos str.

GR - 151 25, Maroussi Tel.: +30 210 87 78 810 Fax: +30 210 87 78 822

193 Adherence of Greek physicians to the international guidelines for prevention and management of asthma in children P. Xepapadaki, ¡. Douladiris, ª. Manoussakis, F. Saxoni-Papageorgiou, N.G. Papadopoulos

Publisher

Owner Greek Paediatric Society© 92 Michalakopoulou str.

GR - 115 28, Athens Tel.: +30 210 7771 140 +30 210 7771 663 Fax: +30 210 7758 354 e-mail: hps@ath.forthnet.gr Annual Subscription All foreign countries: US $ 50

218 Subacute sclerosing panencephalitis after intrauterine infection with measles virus M. Dasopoulou, A. Kovanis

199 Maternal and neonatal selenium and copper blood concentrations in Greeks and Albanians K.H. Schulpis, S. Gavrili, T. Karakonstantakis, G. Karikas, G. Vlachos, C. Costalos, J. Papassotiriou 205 Vitamin D status in breast-fed babies and their mothers in Greece ∞. Challa, A. Ntourntoufi, V. Cholevas, I. Vlachou, M. Bitsori, E. Galanakis, S. Andronikou

223 Proteus syndrome: a case report H. Hatzissevastou-Loukidou, E. Michalopoulou-Manoloutsiou, X.M. Stefopoulou 227 Saethre-Chotzen syndrome in a patient with partial monosomy of chromosomic region 7pter→p15 V. Touliatou, A. Mavrou, A. Kolialexi, E. Papadopoulou, P. Panayiotopoulou, C. Tsengi, E. Kanavakis, S. Kitsiou-Tzeli PRACTICAL ISSUE 233 Plagiocephaly: relation to sleep position, differential diagnosis and management A. Filippidi, I. Germanakis, M. Kalmanti 242 CLINICAL QUIZ N. Markeas, K. Voudris 243 LETTER TO THE EDITOR 243 OBITUARY 161 NEWS FROM THE INTERNET Children’s growth in the spotlight N. Papadopoulos

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™À¡∆∞∫∆π∫∏ E¶π∆ƒ√¶∏

EDITORIAL BOARD

¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘

Editor-in-Chief

∫ˆÓÛÙ·ÓÙ›ÓÔ˜ ™ÙÂÊ·Ó›‰Ë˜, ∞ı‹Ó·

Constantinos Stefanidis, Athens

À‡ı˘ÓÔÈ ™‡ÓÙ·Í˘ ∂ÍÂȉÈ·ۈÓ

Section Editors

∂ϤÓË ∞ÓÙˆÓÔÔ‡ÏÔ˘, ∞ı‹Ó· ∞Ó·Ù˘ÍȷΤ˜ ‰È·Ù·Ú·¯¤˜ - ¶ÚÔ‚Ï‹Ì·Ù· Û˘ÌÂÚÈÊÔÚ¿˜

Eleni Antonopoulou, Athens Developmental and behavioral pediatrics

¶ÂÚÛÂÊfiÓË ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘, £ÂÛÛ·ÏÔÓ›ÎË KÏËÚÔÓÔÌÈο ÌÂÙ·‚ÔÏÈο ÓÔÛ‹Ì·Ù·

Persefoni Avgoustidou-Savopoulou, Thessaloniki Metabolic disorders

∞Ó‰ÚÈ·Ó‹ µ·˙·›Ô˘-°ÂÚ·ÛÈÌ›‰Ë, ∞ı‹Ó· ∂Ó‰ÔÎÚÈÓÔÏÔÁ›·

Andriani Vazaiou-Gerasimidi, Athens Endocrinology

°ÂÒÚÁÈÔ˜ µ·ÚÏ¿Ì˘, £ÂÛÛ·ÏÔÓ›ÎË ∫·Ú‰ÈÔÏÔÁ›·

George Varlamis, Thessaloniki Cardiology

ª·Ú›· ∫·Ó¿ÚÈÔ˘, ∞ı‹Ó· ∞ÓÔÛÔÏÔÁ›·

Maria Kanariou, Athens Immunology

∂˘ÛÙ·ı›· ∫·ÙÛ·ÚÔ‡-¶ÂÎÙ·Û›‰Ë, ∞ı‹Ó· ¡Â˘ÚÔÏÔÁ›·

Eustathia Katsarou-Pektasidou, Athens Neurology

∞ÓÙÒÓ˘ ∫·ÙÙ¿Ì˘, ∞ı‹Ó· ∞ÈÌ·ÙÔÏÔÁ›· - OÁÎÔÏÔÁ›·

Antonis Kattamis, Athens Haematology - √ncology

ÃÚ‹ÛÙÔ˜ ∫ÒÛÙ·ÏÔ˜, ∞ı‹Ó· ¡ÂÔÁÓÔÏÔÁ›·

Christos Kostalos, Athens Neonatology

∞ÏÂÍ¿Ó‰Ú· ¶··‰ÔÔ‡ÏÔ˘, ∞ı‹Ó· °·ÛÙÚÂÓÙÂÚÔÏÔÁ›· - ∏·ÙÔÏÔÁ›· - ¢È·ÙÚÔÊ‹

Alexandra Papadopoulou, Athens Gastroenterology - Hepatology - Nutrition

¡ÈÎfiÏ·Ô˜ ¶··‰fiÔ˘ÏÔ˜, ∞ı‹Ó· ∞ÏÏÂÚÁÈÔÏÔÁ›· - ¶Ó¢ÌÔÓÔÏÔÁ›·

Nicos Papadopoulos, Athens Allergology - Pneumonology

∞ÓÙÈÁfiÓË ™È·ÌÔÔ‡ÏÔ˘-ª·˘Ú›‰Ô˘, πˆ¿ÓÓÈÓ· ƒÂ˘Ì·ÙÔÏÔÁ›·

Antigoni Siamopoulou-Mavridou, Ioannina Rheumatology

ª·Ú›˙· ∆ÛÔÏÈ¿, ∞ı‹Ó· §ÔÈ̈ÍÈÔÏÔÁ›·

Marisa Tsolia, Athens Infectious Diseases

ª¤ÏË Ù˘ ¢ÈÂıÓÔ‡˜ ™˘ÓÙ·ÎÙÈ΋˜ ∂ÈÙÚÔ‹˜ ñ Members of the International Editorial Board Alexis Arzimanoglou, Paris, France

Christer Holmberg, Helsinki, Finland

Ellis D. Avner, Milwaukee, USA

Peter Hoyer, Essen, Germany

Swati Bhave, New Delhi, India

Jan Janda, Prague, Czech Republic

Alberto Bissot, Panama, Panama

Jan Kimpen, Ultrecht, Netherlands

David Branski, Jerusalem, Israel

John Manis, Boston, USA

Francesco Chiarelli, Chieti, Italy

Manuel Moya, Alicante, Spain

Chok-Wan Chan, Hong Kong, China

Hugh O'Brodovich, Toronto, Canada

Denis Daneman, Toronto, Canada

Ross Petty, Vancouver, Canada

Jochen Ehrich, Hannover, Germany

Willem Proesmans, Leuven, Belgium

Demetrius Ellis, Pittsburgh, USA

Jose Ramet, Antwerp, Belgium

Yoshikatsu Eto, Tokyo, Japan

Alan Sinaiko, Minneapolis, USA

Richard N. Fine, Stony Brook, USA

Nick J. Spencer, Coventry, UK

Margaret C. Fisher, Philadelphia, USA

Alfred Tenore, Udine, Italy

Raif Geha, Boston, USA

Alkis Togias, Bethesda, USA

Adenike Grange, Lagos, Nigeria

Eva Tsalikian, Iowa City, USA

Judith G. Hall, Vancouver, Canada

Catherine Weil-Olivier, Paris, France

Patricia Hamilton, London, UK

Johannes Zschocke, Heidelberg, Germany

Enver Hasanoglu, Ankara, Turkey

vii

Pediatr May-June 06

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

2. ∞Ó·ÛÎÔ‹ÛÂȘ.

3. µÚ·‚Â˘Ì¤Ó˜ ÂÚÁ·Û›Â˜.

4. ∂Ú¢ÓËÙÈΤ˜ ÌÂϤÙ˜.

5. ∫ÏÈÓÈο ∫Ô˘›˙.

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

7. ∂›Î·ÈÚ· ı¤Ì·Ù·.

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

9. ∂ӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ.

10. ™‡ÓÙÔÌ· Ó¤·.

11. µÚ·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ.

12. ∂ÈÛÙÔϤ˜ ÚÔ˜ ÙË Û‡ÓÙ·ÍË.

13. ¶ÂÚÈÏ‹„ÂȘ Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜.

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

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

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

xv

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

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

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

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

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

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

Pediatr May-June 06

16-05-06

17:17

™ÂÏ›‰·14

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

484-500.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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™À¡∆√ª√°ƒ∞ºπ∂™ o

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

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

™˘Ó‰˘·˙fiÌÂÓ· ÚÔı¤Ì·Ù· 12

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

ABBREVIATIONS

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

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

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

2. Review articles.

3. Award-winning articles.

4. Original articles.

5. Clinical Quiz.

6. Round tables.

7. Current issues.

8. Issues of healthcare management and education.

9. Case reports.

10. News.

11. Brief reports.

12. Letters to the editor.

13. Abstracts.

14. Future congresses and events.

15. Book reviews.

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

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

Scientific Publications Ltd.

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

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

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

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

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

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

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

Text length shall be: ñ review articles 2000-3000 words; ñ original articles 1500-2500 words and case reports 1000-1500 words; ñ brief reports 1000-1500 words; ñ letters 250-500 words.

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

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

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

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

Text Original articles include: introduction, methods, results and discussion. The introduction includes the latest research data on the subject and the main references and the objectives of the paper. The description of the methods should be precise and detailed so as to enable reproduction by other researchers. In addition, the statistical methods of analysis and evaluation of the results should be described. Results should be presented clearly, together with the appropriate statistical analysis. Discussion should cover the results ensuing from the research, their significance and possible associations with the observations of other researchers.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

484-500.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

7. Text (double space, separate page).

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

9. References (double space, separate page).

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

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

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

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∞ƒ£ƒ√ ™À¡∆∞•∏™

EDITORIAL

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Proteus syndrome: a syndrome named after a Greek god J.G. Hall The Greek sea god Proteus was capable of assuming many different forms. For this reason, in 1983 Professor Weidemann chose his name for this unusual syndrome that can take many forms and has a phenotype that changes dramatically over time. Proteus syndrome was first described

by Cohen and Hayden in 1979 pointing out the strikingly unusual, asymmetric overgrowth features. Many cases have been described since, although it is considered a rare disorder.

It is one of those syndromes with patchy involvement that almost surely represent genetic mosaicism, i.e., there is some normal tissue and some affected tissue. The affected tissue usually involves overgrowth. Proteus syndrome mainly involves bone (particularly long bones and digits), blood vessels, lipomas, and skin. It can occasionally involve malignant degeneration. Almost every tissue and part of the body may be affected.

Affected children are often normal or almost normal at birth and then have dramatic and sometimes grotesque overgrowth which interestingly, usually stops at puberty. The overgrowth apparently stops, for the most part, because the sex hormones bring about cessation of growth. In other words, the effected tissues usually “mature” and stop growing at puberty. Then the affected individual is left with asymmetry which may be very difficult to address cosmetically. As in the case reported on page 223, frequently surgery is required during the growth period, with amputation of affected tissue when it becomes so large that it is non-functional.

Fortunately, this appears to be a sporadic condition, rarely have familial recurrences even been suggested and these were atypical reports.

Mutation in one cell of the developing embryo must occur early in embryogenesis. The abnormal daughter cells then get distributed to many parts of the body, thereafter growing at an abnormally rapid rate. Hopefully, when molecular genetics discovers which gene or genes are involved, molecular treatment will be possible by blocking the pathway(s) in which the overgrowth occurs.

The differential diagnosis of Proteus syndrome includes other congenital hamartomatous

disorders such as Klippel-Trenaunay-Weber syndrome, Ollier disease, and Maffucci syndrome.

Interestingly, Cohen has suggested that the famous “Elephant Man” thought originally to represent Neurofibromatosis Type I probably had Proteus syndrome.

Biesecker and Cohen have excellent reviews of the syndrome which undoubtedly overlaps other hamartomatous conditions. Unfortunately, affected individuals often die early because of vascular compromise, thrombosis, and pulmonary embolism.

The hypertrophy of the skin of the soles of the feet seems to be a unique feature of Proteus syndrome. Perhaps suggesting the god Proteus needed to be “grounded” to be able to work his magic.

Molecular studies of various genes for overgrowth have been inconclusive thus far in studies on individuals with Proteus syndrome. However, as additional new techniques develop and more growth related pathways are elucidated we can hope that the mysteries of Proteus will be revealed.

Professor Emeritus of Pediatrics and Medical Genetics UBC & Children's and Women's Health Centre of BC Department of Pediatrics BC Children's Hospital AÏÏËÏÔÁÚ·Ê›·: Judith G. Hall Professor Emeritus of Pediatrics and Medical Genetics UBC & Children's and Women's Health Centre of BC Department of Pediatrics BC Children's Hospital Vancouver, Canada.

E-mail: jhall@cw.bc.ca

References 1. Biesecker LG, Happle R, Mulliken JB, Weksberg R, Graham JM Jr, Viljoen DL, et al. Proteus syndrome: diagnostic criteria, differential diagnosis, and patient evaluation. Am J Med Genet 1999;84:389-395.

2. Cohen MM Jr. Proteus syndrome: clinical evidence for somatic mosaicism and selective review. Am J Med Genet 1993;47:645-652.

3. Cohen MM Jr. Understanding Proteus syndrome unmasking the elephant man and stemming elephant fever. Neurofibromatosis 1988;1:260-280.

4. Cohen MM Jr, Hayden PW. A newly recognized hamartomatous syndrome. Birth Defects Orig Artic Ser 1979;15:291-296.

5. Gilbert-Barness E, Cohen MM Jr, Opitz JM. Multiple meningiomas craniofacial hyperostosis and retinal abnormalities in Proteus syndrome. Am J Med Genet 2000;93:234-240.

6. Slavotinek AM, Vacha SJ, Peters KF, Biesecker LG. Sudden death caused by pulmonary thromboembolism in Proteus syndrome. Clin Genet 2000;58:386-389.

7. Turner JT, Cohen MM Jr, Biesecker LG. Reassessment ¶·È‰È·ÙÚÈ΋ 2006;69:165-166

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J.G. Hall

of the Proteus syndrome literature: application of diagnostic criteria to published cases. Am J Med Genet 2004:130:111-112.

8. Wiedemann HR, Burgio GR, Aldenhoff P, Kunze J, Kaufmann HJ, Schirg E. The Proteus syndrome:

ª·Ú›· ¶··Ê›ÏË °È· ÏËÚÔÊÔڛ˜: www.euran.com ªaria Papafili For information: www.euran.com

¶·È‰È·ÙÚÈ΋ 2006;69:165-166

partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections. Europ J Pediatr 1983;140:5-12.

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EDITORIAL

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¶fiÛÔ ÂÊ·ÚÌfi˙ÔÓÙ·È ÔÈ ¢ÈÂıÓ›˜ √‰ËÁ›Â˜ ÁÈ· ÙÔ ·È‰ÈÎfi ¿ÛıÌ·; ¡.°. ¶··‰fiÔ˘ÏÔ˜ √È Ô‰ËÁ›Â˜ Î·È ÔÌÔʈӛ˜, ȉ›ˆ˜ ÂΛӘ Ô˘ ‚·Û›˙ÔÓÙ·È Û ‰Â‰Ô̤ӷ (evidence-based), ·ÔÙÂÏÔ‡Ó ÛÙȘ ̤Ú˜ Ì·˜ “µ›‚ÏÔ˘˜” ÚÔ˜ ¯Ú‹ÛË ÙfiÛÔ ÙˆÓ ÂÍÂȉÈÎÂ˘Ì¤ÓˆÓ fiÛÔ Î·È ÙˆÓ ÁÂÓÈÎÒÓ ÁÈ·ÙÚÒÓ. ∏ Ù¿ÛË ·˘Ù‹ Â›Ó·È ·fiÏ˘Ù· ‰ÈηÈÔÏÔÁË̤ÓË: ·fi ÙË Ì›· ÌÂÚÈ¿, Ô Ú˘ıÌfi˜ ·Ú·ÁˆÁ‹˜ Ó¤ˆÓ ‰Â‰ÔÌ¤ÓˆÓ Â›Ó·È Ù¤ÙÔÈÔ˜, Ô˘ Â›Ó·È ·‰‡Ó·ÙÔÓ ·˘Ù¿ Ó· ÂÓۈ̷وıÔ‡Ó Û ÙÂÎÌËÚȈ̤ÓË ÁÓÒÛË ÛÙË ÌÔÚÊ‹ ÙˆÓ ÎÏ·ÛÈÎÒÓ Û˘ÁÁÚ·ÌÌ¿ÙˆÓ. ∆·¯¤ˆ˜ ·Ó·Ù˘ÛÛfiÌÂÓ· ‰›·, fiˆ˜ ·˘Ùfi Ù˘ ∞ÏÏÂÚÁÈÔÏÔÁ›·˜, ÂÎ ÙˆÓ Ú·ÁÌ¿ÙˆÓ ıˆÚÔ‡Ó ··Ú¯·ÈˆÌ¤Ó·, ‰Â‰Ô̤ӷ ·Ï·ÈfiÙÂÚ· ÌÈ·˜ 5ÂÙ›·˜. √ ›‰ÈÔ˜ ·˘Ùfi˜ Ú˘ıÌfi˜, ·fi ÙËÓ ÔÙÈ΋ ÁˆÓ›· ÙÔ˘ ·È‰È¿ÙÚÔ˘, οÓÂÈ ‰‡ÛÎÔÏË ÙËÓ ÎÚÈÙÈ΋ ·Ó¿ÁÓˆÛË fi¯È ÌfiÓÔ ÂÚ¢ÓËÙÈÎÒÓ ÂÚÁ·ÛÈÒÓ ·ÏÏ¿ Î·È ·Ó·ÛÎÔ‹ÛˆÓ. ŒÙÛÈ, ÔÌ¿‰Â˜ ÂȉÈÎÒÓ ·Ó·Ï·Ì‚¿ÓÔ˘Ó Ó· “ÊÈÏÙÚ¿ÚÔ˘Ó” Ù· ÚˆÙÔÁÂÓ‹ ‰Â‰Ô̤ӷ, Û˘¯Ó¿ Ì ¿ÚÈÛÙÔ ÙÚfiÔ, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ·ÏÁÔÚ›ıÌÔ˘˜ Ô˘ ·ÍÈÔÏÔÁÔ‡Ó ÙËÓ ÔÈfiÙËÙ· οı ÌÂϤÙ˘, Ì ¯·Ú·ÎÙËÚÈÛÙÈÎfi ·Ú¿‰ÂÈÁÌ· ÙËÓ Cochrane Library (1). ŸÛÔÓ ·ÊÔÚ¿ ÛÙÔ ¿ÛıÌ·, ÔÈ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜ GINA (Global INitiative for Asthma), ·ÔÙÂÏÔ‡Ó ÂÈÙ˘¯Ë̤ÓÔ ·Ú¿‰ÂÈÁÌ· ‰È¿‰ÔÛ˘ Î·È ÈÛÔÚÚÔ›·˜ ÌÂٷ͇ ‰È·ÊfiÚˆÓ ÚÔÛÂÁÁ›ÛˆÓ, ·Ó Î·È Û·ÊÒ˜ ÔÈ Ô‰ËÁ›Â˜ ·˘Ù¤˜ ÏËÛÈ¿˙Ô˘Ó ÂÚÈÛÛfiÙÂÚÔ Û ÂΛӘ ÙˆÓ ∏.¶.∞. (2). √È Ô‰ËÁ›Â˜ ·˘Ù¤˜, Ô˘ ÌÔÚ›Ù ӷ ÙȘ ‚Ú›Ù ÛÙÔ ‰È·‰›ÎÙ˘Ô (2), ·Ó Î·È Â›Ó·È Ï‹ÚÂȘ Î·È Î·Ù·ÍȈ̤Ó˜, ¤¯Ô˘Ó ‰‡Ô ÛËÌ·ÓÙÈο ÚÔ‚Ï‹Ì·Ù·. ∆Ô ÚÒÙÔ, ¤¯ÂÈ Ó· οÓÂÈ Ì ÙÔÓ fiÁÎÔ ÙÔ˘˜. ∂›Ó·È ·Ï‹ıÂÈ· fiÙÈ ›Ûˆ˜ Ó· ··ÈÙÂ›Ù·È ÁÈ· ÙË ÌÂϤÙË ÙÔ˘˜ Ï›ÁÔ ·Ú·¿Óˆ ¯ÚfiÓÔ˜, ·fi ·˘ÙfiÓ Ô˘ ı· ÌÔÚÔ‡Û ӷ ·ÊÈÂÚÒÛÂÈ Ô Ì¤ÛÔ˜ ·È‰›·ÙÚÔ˜. ™·Ó ·ÓÙÂȯ›ÚËÌ· ‚¤‚·È·, ÌÔÚ› Ó· ı¤ÛÂÈ Î·Ó›˜ ÙÔÓ ·ÓÙ›ÛÙÔÈ¯Ô ¯ÚfiÓÔ Ô˘ ı· ÂÍÔÈÎÔÓÔÌ‹ÛÂÈ Ô ÁÈ·ÙÚfi˜, ¤¯ÔÓÙ·˜ ¿ÓÂÛË ÛÙÔÓ ¯ÂÈÚÈÛÌfi Ù˘ ÓfiÛÔ˘. ™ÙÔ Ù‡¯Ô˜ ·˘Ùfi Ù˘ “¶·È‰È·ÙÚÈ΋˜”, ·ÚÔ˘ÛÈ¿˙ÂÙ·È ÂÚ¢ÓËÙÈ΋ ÌÂϤÙË Ô˘ ÂÍÂÙ¿˙ÂÈ ÙË ¯Ú‹ÛË ÙˆÓ Ô‰ËÁÈÒÓ GINA ·fi ÙÔ˘˜ ŒÏÏËÓ˜ ·È‰È¿ÙÚÔ˘˜ (3): ‰ÂÓ ·ÔÙÂÏ› ȉȷ›ÙÂÚË ¤ÎÏËÍË fiÙÈ Î·È ÛÙË ¯ÒÚ· Ì·˜, Ë ¯Ú‹ÛË ÙˆÓ Ô‰ËÁÈÒÓ Â›Ó·È ÏËÌÌÂÏ‹˜, Ì ÌÂÁ¿ÏË ‰È·Î‡Ì·ÓÛË (4). ∂ӉȷʤÚÔÓ ¤¯ÂÈ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô‡ÙÂ Ë ¤ÏÏÂÈ„Ë ÁÓÒÛˆÓ, Ô‡ÙÂ Ë ¤ÏÏÂÈ„Ë Î·Ù·-

ÓfiËÛ˘, Ô‡ÙÂ Î·Ó Ë ‰È·ÊˆÓ›· ‰ÂÓ ÌÔÚ› Ó· ÂÍËÁ‹ÛÂÈ ÙË ÌÈÎÚ‹ ¯Ú‹ÛË ÙˆÓ Ô‰ËÁÈÒÓ. ∞Ó Î·È ÙÔ Û˘Ì¤Ú·ÛÌ· Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜ ·Ô‰›‰ÂÈ ÙÔ Úfi‚ÏËÌ· ÛÙËÓ ·Ó¿ÁÎË Û˘Ó¯È˙fiÌÂÓ˘ ÂÎ·›‰Â˘Û˘, ÁÂÁÔÓfi˜ ÙÔ ÔÔ›Ô Û·ÊÒ˜ ·ÏËı‡ÂÈ Û ÌÂÁ¿ÏÔ ‚·ıÌfi, ‰ÂÓ ÌÔÚ›, ÁÈ· ÏfiÁÔ˘˜ ÂÈÛÙËÌÔÓÈ΋˜ ‰ÂÔÓÙÔÏÔÁ›·˜ Ó· ı¤ÛÂÈ ¤Ó· Úfi‚ÏËÌ· ÈÔ ¿‰ËÏÔ. ∆Ô ÛËÌ·ÓÙÈÎfiÙÂÚÔ, Èı·ÓÒ˜, Úfi‚ÏËÌ· ÙˆÓ Ô‰ËÁÈÒÓ GINA, Â›Ó·È ÙÔ fiÙÈ Û¯Â‰È¿ÛÙËÎ·Ó Ì ԉËÁfi Î·È ¿ÍÔÓ· ÙÔ ¿ÛıÌ· ÙˆÓ ÂÓËϛΈÓ. ∏ ÌÂÙ·‚ÔÏ‹ ÙˆÓ ‰È·Ì¤ÙÚˆÓ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ, Ë ˆÚ›Ì·ÓÛË Ù˘ ·ÓÔÛȷ΋˜ ·¿ÓÙËÛ˘, ·ÏÏ¿ Î·È ÔÈ ·ÏÏ·Á¤˜ ÛÙ· ÂÚÈ‚·ÏÏÔÓÙÈο ÂÚÂı›ÛÌ·Ù· Ô˘ ÚÔ·ÙÔ˘Ó Ì ÙËÓ ÚfiÔ‰Ô Ù˘ ËÏÈΛ·˜, Â›Ó·È ÌÂÚÈο ·fi Ù· ·›ÙÈ· Ô˘ οÓÔ˘Ó ÙÔ ¿ÛıÌ· ͯˆÚÈÛÙfi fi¯È ÌfiÓÔ ÌÂٷ͇ ·È‰ÈÒÓ Î·È ÂÓËϛΈÓ, ·ÏÏ¿ ·ÎfiÌË Î·È ÌÂٷ͇ ·È‰ÈÒÓ ‰È·ÊÔÚÂÙÈÎÒÓ ËÏÈÎÈÒÓ. √È ÛËÌÂÚÈÓ¤˜ Ô‰ËÁ›Â˜, ‚·Û›˙Ô˘Ó ÙË ‰ÔÌË̤ÓË ÙÔ˘˜ ÚÔÛ¤ÁÁÈÛË ÛÙË ‚·Ú‡ÙËÙ· Ù˘ ÓfiÛÔ˘, ÂÈÎÂÓÙÚÒÓÔÓÙ·˜ ÛÙÔÓ ¯ÚfiÓÈÔ-ÂÈ̤ÓÔÓÙ· ¯·Ú·ÎÙ‹Ú· Ù˘. ™Ù· ·È‰È¿ fï˜, ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ·ÚÔ˘ÛÈ¿˙ÂÈ Û˘Ó‹ıˆ˜ ‰È·Ï›Ô˘Û· ÓfiÛÔ, Ì ÌÂÙ·ÏÔÈÌÒ‰Ë ¯·Ú·ÎÙ‹Ú· (5). ™ÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜, Ë ¯Ú‹ÛË ‹ ÌË Ì·ÎÚÔ¯ÚfiÓÈ·˜ ÚÔÏËÙÈ΋˜ ıÂÚ·›·˜, ·ÏÏ¿ Î·È Ë ÂÈÏÔÁ‹ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘, ‰ÂÓ Â›Ó·È ÍÂοı·ÚË, ·ÏÏ¿ ‰ÂÓ ¤¯ÂÈ ÌÂÏÂÙËı› Î·È ·ÚÎÂÙ¿, ·ÎÚÈ‚Ò˜ ÏfiÁˆ ÙÔ˘ ÁÂÁÔÓfiÙÔ˜ ÙÔ˘ fiÙÈ ÔÈ ÌÂϤÙ˜ Ù›ÓÔ˘Ó Ó· ÂÈϤÁÔ˘Ó ÙÔ˘˜ ·ÛıÂÓ›˜ ÙÔ˘˜ Û‡Ìʈӷ Ì ÙË ‰ÈÂıÓ‹ ηٿٷÍË, “Û˘ÌȤ˙ÔÓÙ¿˜” ÙÔ˘˜ ÛÙȘ ˘·ÚÎÙ¤˜ ηÙËÁÔڛ˜. ŒÙÛÈ, ‰ÂÓ ¤¯Ô˘Ó ··ÓÙËı› ÂÚˆÙ‹Ì·Ù· fiˆ˜, ·fi ÔÈ· ËÏÈΛ· Î·È Û ÔÈÔ Ê·ÈÓfiÙ˘Ô ÂӉ›ÎÓ˘Ù·È Ë ¤Ó·ÚÍË ¯ÚfiÓÈ·˜ ¯Ú‹Û˘ ÛÙÂÚÔÂȉÒÓ, ÔÈ· Ë ı¤ÛË ÙˆÓ ÛÙ·ıÂÚÒÓ Û˘Ó‰˘·ÛÌÒÓ, ‹ fiÛÔ ÓˆÚ›˜ ÍÂÎÈÓ¿ÂÈ Î·Ó›˜ Ù· ·ÓÙÈÏ¢ÎÔÙÚÈÂÓÈο Ê¿Ú̷η. √È ·Ú·¿Óˆ ÚÔ‚ÏËÌ·ÙÈÛÌÔ› Â›Ó·È ÎÔÈÓÔ› Û fiÛÔ˘˜ ·Û¯ÔÏÔ‡ÓÙ·È Û˘ÛÙËÌ·ÙÈο Ì ÙÔ ·È‰ÈÎfi ¿ÛıÌ·. ŒÙÛÈ, ˘¿Ú¯ÂÈ ÙÔÓ ÙÂÏÂ˘Ù·›Ô ηÈÚfi ÌÂÁ¿ÏË ÎÈÓËÙÈÎfiÙËÙ· Û¯ÂÙÈο Ì Èı·Ófi Â·Ó·Û¯Â‰È·ÛÌfi ÙˆÓ Ô‰ËÁÈÒÓ Û¯ÂÙÈο Ì ÙÔ ·È‰ÈÎfi ¿ÛıÌ·. ¶ÚfiÛÊ·Ù· Ì¿ÏÈÛÙ·, ¤ÁÈÓÂ Û˘Ó¿ÓÙËÛË ÂȉÈÎÒÓ ·fi fiÏË ÙËÓ ∂˘ÚÒË ÛÙ· Ï·›ÛÈ· ÙÔ˘ ∂˘Úˆ·˚ÎÔ‡ ™˘Ó‰ڛԢ ¶Ó¢ÌÔÓÔÏÔÁ›·˜, Ô˘ η٤ÏËÍ Û ÚÔÙ¿ÛÂȘ fiˆ˜ ÔÈ ·Ú·¿Óˆ. √È ÎÈÓ‹ÛÂȘ ·˘Ù¤˜ ·Ó·Ì¤ÓÂÙ·È Ó· ‚ÂÏÙÈÒÛÔ˘Ó

§¤ÎÙÔÚ·˜ ¶·È‰È·ÙÚÈ΋˜ ∞ÏÏÂÚÁÈÔÏÔÁ›·˜, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ∞ÏÏÂÚÁÈÔÏÔÁÈÎfi ÙÌ‹Ì· ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”

¶·È‰È·ÙÚÈ΋ 2006;69:167-168

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¡.°. ¶··‰fiÔ˘ÏÔ˜

ÛËÌ·ÓÙÈο ÙËÓ Â˘¯ÚËÛÙ›· ÙˆÓ Ô‰ËÁÈÒÓ fiÛÔÓ ·ÊÔÚ¿ ÛÙÔ˘˜ ·È‰È¿ÙÚÔ˘˜. ™Â οı ÂÚ›ÙˆÛË ‚¤‚·È·, ÔÈ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜ ·Ú·Ì¤ÓÔ˘Ó ÛËÌ·ÓÙÈÎfi ΛÌÂÓÔ ·Ó·ÊÔÚ¿˜, fi¯È ÂÚÈÔÚ›˙ÔÓÙ·˜, ·ÏÏ¿ ˘Ô‚ÔËıÒÓÙ·˜ ÙȘ È·ÙÚÈΤ˜ ÂÈÏÔÁ¤˜ ÙÔ˘ ·È‰È¿ÙÚÔ˘ Î·È ÂÏ›˙Ô˘Ì fiÙÈ ÙfiÛÔ ÙÔ ¿ÚıÚÔ ·˘Ùfi, fiÛÔ Î·È Ë ÂÚÁ·Û›· Ô˘ ı· ‰È·‚¿ÛÂÙ (3) ı· Û˘Ì‚¿ÏÔ˘Ó ÛÙË ‚ÂÏÙ›ˆÛË Ù˘ ÂÊ·ÚÌÔÁ‹˜ ÙÔ˘˜.

µÈ‚ÏÈÔÁÚ·Ê›· 1. The Cochrane Library http://www.cochrane.org/reviews/clibintro.htm

¶·È‰È·ÙÚÈ΋ 2006;69:167-168

2. Workshop Report, Global Strategy for Asthma Management and Prevention http://www.ginasthma.com/Guidelineitem.asp?l1=2&l2=1&intId=60 3. •Â··‰¿ÎË ¶, ¢Ô˘Ï·‰›Ú˘ ¡, ª·ÓÔ˘Û¿Î˘ ª, ™·ÍÒÓË-¶··ÁˆÚÁ›Ô˘ º, ¶··‰fiÔ˘ÏÔ˜ ¡°. ™˘ÌÌfiÚʈÛË ∂ÏÏ‹ÓˆÓ È·ÙÚÒÓ ÛÙȘ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜ ÁÈ· ÙËÓ ÚfiÏË„Ë Î·È ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿. ¶·È‰È·ÙÚÈ΋ 2006;69:193-198.

4. Cabana MD, Rand CS, Becher OJ, Rubin HR. Reasons for pediatrician nonadherence to asthma guidelines. Arch Pediatr Adolesc Med 2001;155: 1057-1062.

5. Papadopoulos NG. All that wheezes is not asthma (or is it?): Defining and treating virus-induced asthma.

AZ-Air 2004, http://www.az-air.com/default.asp?n= 2004-03-14

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ªË ÂÂÌ‚·ÙÈΤ˜ ̤ıÔ‰ÔÈ ÂϤÁ¯Ô˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿ Ã. ∫Ô˘ÙÛ·˘Ù›ÎË1, ¶. ∆·Ú·ÓÙ˙‹ - ¶ÔÙ·ÌÈ¿ÓÔ˘1, Ã. °ÎÚ¿Ù˙ÈÔ˘2, ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜1

¶ÂÚ›ÏË„Ë: ∏ ÔÏÔ¤Ó· ·˘Í·ÓfiÌÂÓË Û˘¯ÓfiÙËÙ· Î·È Â›ÙˆÛË ÙÔ˘ ·È‰ÈÎÔ‡ ¿ÛıÌ·ÙÔ˜ ·ÁÎÔÛÌ›ˆ˜, ÂÈ‚¿ÏÏÂÈ ÙËÓ ·Ó¿ÁÎË ÁÈ· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘. ™ˆÛÙfiÙÂÚË ÛÙÚ·ÙËÁÈ΋ ÚÔÛ¤ÁÁÈÛ˘ Ù˘ ÔÏ˘Ê·ÈÓÔÙ˘È΋˜ Î·È ÔÏ˘·Ú·ÁÔÓÙÈ΋˜ ·˘Ù‹˜ ÓfiÛÔ˘ ıˆÚÂ›Ù·È Ë ·ÈÙÈÔ·ıÔÁÂÓÂÙÈ΋, ‰ËÏ·‰‹, Ë ·Ó›¯Ó¢ÛË Î·È ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ÊÏÂÁÌÔÓ‹˜ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Û ۯ¤ÛË Ì ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ· Î·È ÙË ¯ÔÚËÁÔ‡ÌÂÓË ıÂÚ·›·. √ ¤ÏÂÁ¯Ô˜ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ÏÂÈÙÔ˘ÚÁÈÒÓ Ì ÙË ÛÈÚÔ̤ÙÚËÛË, ÙË ÚÔÔ̤ÙÚËÛË ‹ Ë ·ÎÚÈ‚‹˜ ηٷÁÚ·Ê‹ Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜ ¤¯Ô˘Ó Ê·Ó› ·ÓÂ·ÚΛ˜ ÙÚfiÔÈ ·Ó›¯Ó¢Û˘ Ù˘ ÊÏÂÁÌÔÓÒ‰Ô˘˜ ‰ÈÂÚÁ·Û›·˜. ∆Ô ÂÚ¢ÓËÙÈÎfi ÂӉȷʤÚÔÓ ¤¯ÂÈ ÛÙÚ·Ê› ÛÙËÓ Â͇ÚÂÛË ÌË ÂÂÌ‚·ÙÈÎÒÓ ÌÂıfi‰ˆÓ ·Ú·ÎÔÏÔ‡ıËÛ˘ Ù˘ ·ÛıÌ·ÙÈ΋˜ ÊÏÂÁÌÔÓ‹˜ Ì ˘„ËÏ‹ ÂȉÈÎfiÙËÙ· Î·È Â˘·ÈÛıËÛ›·, ·ÏÏ¿ Â›Û˘ ·ÛÊ¿ÏÂÈ· Î·È Â·Ó·ÏË„ÈÌfiÙËÙ·, ¤ÙÛÈ ÒÛÙ ӷ ÌÔÚÔ‡Ó Ó· ÂÊ·ÚÌÔÛÙÔ‡Ó ÛÙËÓ Î·ıË̤ڷ ÎÏÈÓÈ΋ Ú¿ÍË. ∞˘Ùfi Â›Ó·È Î·È ÙÔ ·ÓÙÈΛÌÂÓÔ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, fiÔ˘ ·Ó·ÛÎÔÔ‡ÓÙ·È Î·È Û¯ÔÏÈ¿˙ÔÓÙ·È Î¿ÔȘ ·fi ÙȘ ÛËÌ·ÓÙÈÎfiÙÂÚ˜ Î·È ·Ô‰ÂÎÙ¤˜ ÌË ÂÂÌ‚·ÙÈΤ˜ ÌÂıfi‰Ô˘˜ fiˆ˜ Â›Ó·È Ë Ì¤ıÔ‰Ô˜ ÙˆÓ ÚÔÎÏËÙÒÓ Ù˘¤ÏˆÓ, ÙÔ ÂÎÓÂfiÌÂÓÔ ¡√ Î·È ÙÔ Û˘Ì‡Îӈ̷ ÙÔ˘ ÂÎÓÂfiÌÂÓÔ˘ ·¤Ú· (EBC).

1 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡” 2 ∫¤ÓÙÚÔ ÕÛıÌ·ÙÔ˜, ªÔÓ¿‰· ¡ÔÛËÌ¿ÙˆÓ £ÒÚ·ÎÔ˜, ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ, ∂˘ÁÂÓ›‰ÂÈÔ £ÂÚ·Â˘Ù‹ÚÈÔ AÏÏËÏÔÁÚ·Ê›·: ÃÚ˘Û‹ ∫Ô˘ÙÛ·˘Ù›ÎË ™ÔÊ. µÂÓÈ˙¤ÏÔ˘ 77, ∆.∫. 152 32, ÷ϿӉÚÈ E-mail: kochrysa@yahoo.com ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 18-01-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 18-01-2006

§¤ÍÂȘ ÎÏÂȉȿ: ¶·È‰ÈÎfi ¿ÛıÌ·, ÊÏÂÁÌÔÓ‹ ·ÂÚ·ÁˆÁÒÓ, ÌË ÂÂÌ‚·ÙÈΤ˜ ̤ıÔ‰ÔÈ ·Ú·ÎÔÏÔ‡ıËÛ˘.

¡on-invasive methods of asthma assessment in children C. Koutsaftiki1, P. Taprantzi - Potamianou1, C. Gratziou2, A. Constantopoulos1

Abstract: There appears to be an increasing prevalence in childhood asthma all over the world, and hence the need for effective diagnosis and treatment is greater than ever. The pathogenetic approach to this multiphenotypic and multifactorial disease, in other words the assessment of the airway inflammation in relation to the clinical symptoms and pharmaceutical treatment, has proved to be the most appropriate. Spirometry, peak expiratory flow and recording of symptoms cannot reflect sufficiently and accurately the extent of the inflammatory process. For this reason the scientific world has directed its interest to the search for non-invasive methods of assessment of the airway inflammation (“inflammometry”) with high sensitivity and specificity, as well as safety and reproducibility, which could be applied in routine practice. This paper reviews some of the most acceptable non-invasive methods, such as the induced sputum, the exhaled nitric oxide (NO), and the exhaled breath condensate (EBC).

Key words: Childhood asthma, airway inflammation, non-invasive methods.

™˘ÓÙÔÌÔÁڷʛ˜ BAL µÀ∞ ECP FEV1 FVC eNO nNO PEFR EBC LTs IL-4

‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· ‚ÚÔÁ¯È΋ ˘ÂÚ··ÓÙËÙÈÎfiÙËÙ· ˈÛÈÓÔÊÈÏÈ΋ ηÙÈÔÓÈ΋ ÚˆÙ½ÓË ‚›·È· ÂÎÓÂfiÌÂÓÔ˜ fiÁÎÔ˜ ‚›·ÈË ÂÎÓÂfiÌÂÓË ¯ˆÚËÙÈÎfiÙËÙ· ÂÎÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ ÙÔ˘ ∞˙ÒÙÔ˘ ÚÈÓÈÎfi ÌÔÓÔÍ›‰ÈÔ ÙÔ˘ ∞˙ÒÙÔ˘ ̤ÁÈÛÙË ÂÎÓ¢ÛÙÈ΋ ÚÔ‹ Û˘Ì‡Îӈ̷ ÂÎÓÂfiÌÂÓÔ˘ ·¤Ú· Ï¢ÎÔÙÚȤÓÈ· ÈÓÙÂÚÏ¢ΛÓË-4

INF-Á Th EPO EPX/EDN

1 2nd Paediatric Clinic, University of Athens, “P. & A. Kyriakou” Children’s Hospital 2 Asthma Center, Thoracic Diseases Unit, University of Athens, “Eugenidio” Therapeuterio Correspondence: Chrysie Koutsaftiki Sof. Venizelou 77, 152 32, Chalandri, Greece E-mail: kochrysa@yahoo.com Date of submission: 18-01-2005 Date of approval: 18-01-2006

ÈÓÙÂÚÊÂÚfiÓË-Á ∆ ‚ÔËıËÙÈÎfi ÏÂÌÊÔ·ÙÙ·ÚÔ ËˆÛÈÓÔÊÈÏÈ΋ ÂÚÔÍÂȉ¿ÛË ËˆÛÈÓÔÊÈÏÈ΋ ÚˆÙ½ÓË Ã/Ó¢ÚÔÙÔÍ›ÓË ÚÔÂÚ¯fiÌÂÓË ·fi Ù· ˈÛÈÓfiÊÈÏ·

EÈÛ·ÁˆÁ‹ ∆Ô ·È‰ÈÎfi ¿ÛıÌ· Â›Ó·È ›Ûˆ˜ Ë ÈÔ Û˘¯Ó‹ ¯ÚfiÓÈ· ¿ıËÛË ÙˆÓ ·È‰ÈÒÓ Ô˘ fï˜, Û˘Ó¯›˙ÂÈ Ó· ˘ԉȷÁÈÁÓÒÛÎÂÙ·È Î·È Ó· ˘ÔıÂÚ·‡ÂÙ·È ·ÁÎoÛÌ›ˆ˜ ·Ú¿ ÙËÓ ÏËıÒÚ· ÙˆÓ ¶·È‰È·ÙÚÈ΋ 2006;69:169-177

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·170

170

Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

ÌÂÏÂÙÒÓ Î·È ÂÚÁ·ÛÈÒÓ Ô˘ ·Ó·ÎÔÈÓÒÓÔÓÙ·È ÂÓÙ·ÙÈο Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· (1). ∆Ô ÚÔÂÍ¿Ú¯ÔÓ ·ıÔÊ˘ÛÈÔÏÔÁÈÎfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÙÔ˘ ¿ÛıÌ·ÙÔ˜ Â›Ó·È Ë ÊÏÂÁÌÔÓ‹ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ, οÙÈ Ô˘ ÛËÌ·›ÓÂÈ fiÙÈ ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰ÈÂÚ¢ÓËıÔ‡Ó ÔÈ ˘ÔΛÌÂÓÔÈ Ì˯·ÓÈÛÌÔ› Ù˘ ÓfiÛÔ˘, Ó· ÂϤÁ¯ÂÙ·È Ë ÂͤÏÈÍ‹ Ù˘ ηıÒ˜ Î·È Ë ·ÓÙ·fiÎÚÈÛË ÛÙË ¯ÔÚËÁÔ‡ÌÂÓË ıÂÚ·›·, Ú¤ÂÈ Ó· ÌÔÚԇ̠ӷ ÂÎÙÈÌ¿Ì ÙË ÊÏÂÁÌÔÓ‹ ·˘Ù‹. ∆Ô Û‡Á¯ÚÔÓÔ ÂÚ¢ÓËÙÈÎfi ÂӉȷʤÚÔÓ ¤¯ÂÈ ÛÙÚ·Ê› ÂÓÙ·ÙÈο ÚÔ˜ ÙËÓ Â͇ÚÂÛË ÌË ÂÂÌ‚·ÙÈÎÒÓ ÌÂıfi‰ˆÓ Ô˘ Ó· ÌÔÚÔ‡Ó Ó· ·Ó·‰ÂÈÎÓ‡Ô˘Ó ÙÔ ÚÔÊ›Ï Ù˘ ·ÛıÌ·ÙÈ΋˜ ÊÏÂÁÌÔÓ‹˜, ¤ÙÛÈ ÒÛÙ ӷ ÌÔÚÔ‡Ó Ó· ÚԂϤÔ˘Ó ‹/Î·È Ó· ÈÛÙÔÔÈÔ‡Ó ÙÔÓ ·ÚÔ͢ÛÌfi ·ÏÏ¿ Î·È ÙËÓ ÂÚ›Ô‰Ô ÂϤÁ¯Ô˘ Ù˘ ÊÏÂÁÌÔÓ‹˜, ÂÈ ‰˘Ó·ÙfiÓ, ÚÈÓ ·fi ÙËÓ ÂΉ‹ÏˆÛË Û˘Ìو̿وÓ. ŒÙÛÈ ı· Â›Ó·È È‰È·›ÙÂÚ· ¯Ú‹ÛÈÌÔÈ ÛÙȘ ÂÚÈÙÒÛÂȘ ÂΛӘ, Ô˘ Ô‡ÙÂ Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· ·ÏÏ¿ Ô‡ÙÂ Î·È Ë ÛÈÚÔ̤ÙÚËÛË ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó. ºÈÏÔ‰ÔÍÔ‡Ó Ó· ¤¯Ô˘Ó ÙËÓ ·ÎÚ›‚ÂÈ· Ù˘ ‚ÈÔ„›·˜, ·ÏÏ¿ ·Ú¿ÏÏËÏ· Ó· Â›Ó·È ·ÛÊ·Ï›˜ Î·È ·ÓÂÎÙ¤˜ ̤ıÔ‰ÔÈ Ì Â·Ó·ÏË„ÈÌfiÙËÙ· Î·È ˘„ËÏ‹ ÂȉÈÎfiÙËÙ· Î·È Â˘·ÈÛıËÛ›·. ∂›Ó·È ÌÂÁ¿ÏÔ ÙÔ ÔÛÔÛÙfi ·ÛıÂÓÒÓ Ô˘ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ÂÎÙÈÌ‹ÛÔ˘Ó ÙÔÓ ‚ÚÔÁ¯fiÛ·ÛÌÔ ‹ Ù· ¿ÏÏ· Û˘ÌÙÒÌ·Ù· Ô˘ ÂӉ¯Ô̤ӈ˜ Ó· ÂΉËÏÒÓÔ˘Ó, ÂÓÒ ÔÈ ÛÈÚÔÌÂÙÚÈΤ˜ ·Ú¿ÌÂÙÚÔÈ Â›Ó·È ϤÔÓ ÁÓˆÛÙfi fiÙÈ ‰ÂÓ ÌÂÙ·‚¿ÏÏÔÓÙ·È ¿ÓÙ· ·Ó¿ÏÔÁ· Ì ÙȘ ÌÂÙ·‚ÔϤ˜ Ù˘ ·ÛıÌ·ÙÈ΋˜ ÊÏÂÁÌÔÓ‹˜ Î·È ‰ÂÓ ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ÙȘ Ú·ÁÌ·ÙÈΤ˜ ÌÂÙ·‚ÔϤ˜ ÛÙÔ Â›Â‰Ô ÙˆÓ ‚ÚfiÁ¯ˆÓ. √È ÌË ÂÂÌ‚·ÙÈΤ˜ ̤ıÔ‰ÔÈ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ Ô˘ Ù˘Á¯¿ÓÔ˘Ó Â˘Ú›·˜ ÌÂϤÙ˘ ·˘Ù‹ ÙË ÛÙÈÁÌ‹ - ÂÎÙfi˜ ·fi ·˘Ù¤˜ Ô˘ ÂϤÁ¯Ô˘Ó ÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· - Â›Ó·È ÂΛӘ Ô˘ ÚÔÛ‰ÈÔÚ›˙Ô˘Ó Î‡ÙÙ·Ú· Î·È ÌÂÛÔÏ·‚ËÙ¤˜ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÛÙ· Ù‡ÂÏ· Ô˘ ÚÔηÏÔ‡ÓÙ·È ÌÂÙ¿ ·fi ÂÈÛÓÔ‹ ‰È·Ï‡Ì·ÙÔ˜ ˘¤ÚÙÔÓÔ˘ ÔÚÔ‡, ÛÙ· ÂÎÓÂfiÌÂÓ· ·¤ÚÈ·, ÛÙÔÓ Û›ÂÏÔ, ÛÙÔ ·›Ì·, ÛÙ· Ô‡Ú· ‹ Û ¿ÏÏ· ‚ÈÔÏÔÁÈο ˘ÁÚ¿. ∏ ·ÚÔ‡Û· ÌÂϤÙË-·Ó·ÛÎfiËÛË ÙˆÓ ‰ËÌÔÛÈÂ˘Ì¤ÓˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ù˘ ÓÂfiÙÂÚ˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ Û¯ÂÙÈο Ì ·˘Ù¤˜ ÙȘ ÌÂıfi‰Ô˘˜, ı· ÂÈÎÂÓÙÚˆı› ÛÙËÓ ·ÚÔ˘Û›·ÛË Î·È ÙÔÓ Û¯ÔÏÈ·ÛÌfi Ù˘ ÌÂıfi‰Ô˘ ÙˆÓ ÚÔÎÏËÙÒÓ Ù˘¤ÏˆÓ (induced sputum), ÙÔ˘ eNO Î·È ÙÔ˘ EBC, ÂÓÒ ı· Á›ÓÂÈ Î·È ÌÈÎÚ‹ ·Ó·ÊÔÚ¿ ÛÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi ˈÛÈÓÔÊÈÏÈÎÒÓ ÚˆÙÂ˚ÓÒÓ ÛÙÔ ·›Ì· Î·È ÛÙ· Ô‡Ú·. ¶·È‰È·ÙÚÈ΋ 2006;69:169-177

¶ÚÔÎÏËÙ¿ Ù‡ÂÏ· (induced sputum) ∏ ¯Ú‹ÛË Î·È ·Ó¿Ï˘ÛË ÙˆÓ ÚÔÎÏËÙÒÓ Ù˘¤ÏˆÓ Â›Ó·È ¤Ó·˜ ÙÔ̤·˜ Ô˘ ¤¯ÂÈ ÌÂÏÂÙËı› ‰ÈÂÍÔ‰Èο ÛÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜ Î·È Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ·Ó¿Ï˘Û‹˜ ÙÔ˘˜ ıˆÚÔ‡ÓÙ·È ·fi ÔÏÏÔ‡˜ Û˘ÁÎÚ›ÛÈÌ· Ì ÂΛӷ ÙÔ˘ BAL Î·È Ù˘ ‚ÈÔ„›·˜ (2-4). ŒÙÛÈ, ÌÔÚÔ‡Ó Ó· χÛÔ˘Ó ‰È·ÊÔÚԉȷÁÓˆÛÙÈο ÚÔ‚Ï‹Ì·Ù· ÛÙȘ ηٷÛÙ¿ÛÂȘ ÛÙȘ Ôԛ˜ Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ·, Ë ÎÏÈÓÈ΋ ÂͤٷÛË Î·È ÙÔ ÈÛÙÔÚÈÎfi ‰›ÓÔ˘Ó ·Û·Ê›˜ ÏËÚÔÊÔڛ˜ (6,9). ŸÛÔÓ ·ÊÔÚ¿ fï˜, ÛÙÔÓ ·È‰ÈÎfi ÏËı˘ÛÌfi, Á›ÓÔÓÙ·È ·ÎfiÌË Û˘˙ËÙ‹ÛÂȘ ÁÈ· ÙÔ Î·Ù¿ fiÛÔÓ Â›Ó·È ÂÊÈÎÙfi Ó· ·Ú·¯ıÔ‡Ó Ù‡ÂÏ· ·fi Ù· ·È‰È¿ Î·È Î·Ù’ Â¤ÎÙ·ÛË, Ó· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ÁÈ· ·Ó¿Ï˘ÛË. ∏ ÚfiÎÏËÛË Î·È ÂÍ·ÁˆÁ‹ ÙˆÓ Ù˘¤ÏˆÓ Á›ÓÂÙ·È Û fiϘ ÙȘ ËÏÈÎȷΤ˜ ÔÌ¿‰Â˜ Ì ÂÈÛÓÔ‹ ‰È·Ï‡Ì·ÙÔ˜ ˘¤ÚÙÔÓÔ˘ ÔÚÔ‡, ¿ÏÏÔÙ ¿ÏÏ˘ Û˘ÁΤÓÙÚˆÛ˘, Èı·ÓfiÓ ÏfiÁˆ: 1) Ù˘ Ì›ˆÛ˘ ÙÔ˘ ÈÍÒ‰Ô˘˜ Ù˘ ÙÚ·¯ÂÈÔ‚ÚÔÁ¯È΋˜ ‚ϤÓÓ·˜, 2) ·‡ÍËÛ˘ Ù˘ ·Ú·ÁˆÁ‹˜ ‚ϤÓÓ·˜ ‹ ÙÔ˘ fiÁÎÔ˘ ÙˆÓ ÂÎÎÚ›ÛÂˆÓ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ ÁÂÓÈο, 3) ÎÈÓËÙÔÔ›ËÛ˘ Ù˘ οı·ÚÛ˘ ÙÔ˘ ÎÚÔÛÛˆÙÔ‡ ÂÈıËÏ›Ô˘ Î·È 4) Ù˘ ÚfiÎÏËÛ˘ ‚‹¯· - ·ÎfiÌË Î·È ‚ÚÔÁ¯fiÛ·ÛÌÔ˘ - ¤ÌÌÂÛ·, Ì ÙËÓ Â›‰Ú·ÛË ÙÔ˘ ˘¤ÚÙÔÓÔ˘ ÔÚÔ‡ ÛÙ· Ì·ÛÙÔ·ÙÙ·Ú·, Ù· ÔÔ›· ·ÔÎÔÎÎÈÒÓÔÓÙ·È Î·È ÂÚÂı›˙Ô˘Ó ÙȘ ÎÂÓÙÚÔÌfiϘ ›Ó˜ ÙˆÓ ÓÂ‡ÚˆÓ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ (5). §fiÁˆ ÙÔ˘ ÙÂÏÂ˘Ù·›Ô˘, Á›ÓÂÙ·È Ë ÛΤ„Ë fiÙÈ Ù·˘Ùfi¯ÚÔÓ· Ì ٷ Ù‡ÂÏ· ÌÔÚ› ηÓ›˜ Ó· ÌÂÏÂÙ‹ÛÂÈ Î·È ÙË µÀ∞, Ë ÔÔ›· ıˆÚÂ›Ù·È ÛËÌ·ÓÙÈÎfi˜ ‰Â›ÎÙ˘ ÁÈ· ÙËÓ Î·Ù·ÓfiËÛË Ù˘ ·Ó·ÛÙÚ„ÈÌfiÙËÙ·˜ Ù˘ ·fiÊڷ͢ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔ ¿ÛıÌ· (6,7). °È· ηχÙÂÚË Û˘ÓÂÚÁ·Û›· ÛÙ· ·È‰È¿, ·ÓÙ› Ù˘ Û˘Ó‰˘·Ṳ̂Ó˘ ‰È·‰Èηۛ·˜ (ÚfiÎÏËÛË Ù˘¤ÏˆÓ Î·È Û˘Á¯ÚfiÓˆ˜ ÚfiÎÏËÛË ‚ÚÔÁ¯È΋˜ ˘ÂÚ··ÓÙËÙÈÎfiÙËÙ·˜), ÚÔÙÈÌ¿Ù·È Ó· ¯ÔÚËÁÂ›Ù·È ÂÈÛÓÔ‹ Ì ‚2-‰ÈÂÁ¤ÚÙË Ï›ÁÔ ÚÈÓ ÙËÓ ÂÈÛÓÔ‹ ÙÔ˘ ˘¤ÚÙÔÓÔ˘ ‰È·Ï‡Ì·ÙÔ˜ ÚÔÏ·Ì‚¿ÓÔÓÙ·˜ ÙÔÓ ‚ÚÔÁ¯fiÛ·ÛÌÔ (6). ∏ ̤ıÔ‰Ô˜ ÂÊ·ÚÌfi˙ÂÙ·È Û ¿ÙÔÌ· ËÏÈΛ·˜ <14 ÂÙÒÓ, ÛÙ· Ï·›ÛÈ· ÂÚ¢ÓËÙÈÎÒÓ ÌÂÏÂÙÒÓ, ¿Óˆ ·fi 12 ¯ÚfiÓÈ· Î·È Ù· ÔÛÔÛÙ¿ ÂÈÙ˘¯›·˜ ‰ÈÂÍ·ÁˆÁ‹˜ Ù˘ ‰È·‰Èηۛ·˜ Ô˘ ¤¯Ô˘Ó ηٿ ηÈÚÔ‡˜ ·Ó·ÎÔÈÓˆı›, Î˘Ì·›ÓÔÓÙ·È ·fi 68100% (5). √È ÌÂϤÙ˜ Ì ¯·ÌËÏ¿ ÔÛÔÛÙ¿ ÂÈÙ˘¯›·˜ Î·È ··ÈÛÈfi‰ÔÍ· Û˘ÌÂÚ¿ÛÌ·Ù· ÁÈ· ÙË ÌÂıfi‰Ô, ›¯·Ó ¯ÚËÛÈÌÔÔÈ‹ÛÂÈ Û˘Ó‰˘·Ṳ̂ÓË ÚfiÎÏËÛË µÀ∞ Î·È Ù˘¤ÏˆÓ (8), ÂÓÒ ·ÓÙ›ıÂÙË ÛÙ¿ÛË Î·È

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·171

171

ªË ÂÂÌ‚·ÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿

ıÂÙÈο ·ÔÙÂϤÛÌ·Ù· ¤¯Ô˘Ó ÂÈÙ¢¯ı› Û ÂΛӘ ÛÙȘ Ôԛ˜ ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› “ÚÔıÂÚ·›·” Ì ‚2-‰ÈÂÁ¤ÚÙË (6). ªÂıÔ‰ÔÏÔÁÈÎfi Úfi‚ÏËÌ· ·ÔÙÂÏ› Î·È Ô ÔÚÈÛÌfi˜ ÙÔ˘ ÈηÓÔÔÈËÙÈÎÔ‡ ‰Â›ÁÌ·ÙÔ˜ Ù˘¤ÏˆÓ ˆ˜ ÚÔ˜ ÙËÓ ÔÈfiÙËÙ·, ÙËÓ ÔÛfiÙËÙ· Î·È ÙË Û‡ÛÙ·Û‹ ÙÔ˘˜. ∆· Ù‡ÂÏ· Â›Ó·È ¤Ó· Ì›ÁÌ· ·fi ÙÚ·¯ÂÈÔ‚ÚÔÁ¯ÈΤ˜ ÂÎÎÚ›ÛÂȘ, Û›ÂÏÔ Î·È ˘¤ÚÙÔÓÔ ÔÚfi. ∏ ‡·ÚÍË ÛȤÏÔ˘ ÛÙÔ ÚÔ˜ ·Ó¿Ï˘ÛË ‰Â›ÁÌ· ıˆÚÂ›Ù·È ·fi ÙÔ˘˜ ÂÚÈÛÛfiÙÂÚÔ˘˜ ˆ˜ “ÂÈÌfiÏ˘ÓÛË” ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜ ·˘ÙÔ‡ (6,8,10). ŒÙÛÈ, ›Ù ÌÂÏÂÙ¿Ù·È ‰Â›ÁÌ· Ù˘¤ÏˆÓ, Ô˘ ÌÈÎÚÔÛÎÔÈο ÂϤÁ¯ÂÙ·È ˆ˜ ·¯‡Ú¢ÛÙÔ Ì ‚ÏÂÓÓÔÎ˘Ù·ÚÈΤ˜ ÔÛfiÙËÙ˜, ›Ù ÔÏÈÎfi ‰Â›ÁÌ· ·fi Û›ÂÏÔ Ì Ù‡ÂÏ· Ì·˙› (10-12). ∆· ‰Â›ÁÌ·Ù· ÁÈ· Ó· ıˆÚÔ‡ÓÙ·È ÈηÓÔÔÈËÙÈο Ú¤ÂÈ Ó· ÌËÓ ÂÚȤ¯Ô˘Ó Ï·ÎÒ‰Ë ÂÈıËÏȷο ·ÙÙ·Ú· (squamous cells) Û ÔÛÔÛÙfi >80% (13) ‹ ηْ ¿ÏÏÔ˘˜ >50% (15,6,16), ‹ Ó· ηٷÌÂÙÚÒÓÙ·È Û ·˘Ù¿ ÙÔ˘Ï¿¯ÈÛÙÔÓ 500 ÌË Ï·ÎÒ‰Ë-ÂÈıËÏȷο ·ÙÙ·Ú· (non-squamous cells) (8). ™Â ÔÚÈṲ̂Ó˜ ÌÂϤÙ˜, ΢ڛˆ˜ Û ÂÓËÏ›ÎÔ˘˜, ÛËÌ·Û›· ‰›ÓÂÙ·È Î·È ÛÙÔÓ Ì·ÎÚÔÛÎÔÈÎfi fiÁÎÔ ÙˆÓ Ù˘¤ÏˆÓ, Ô ÔÔ›Ô˜, ÁÈ· Ó· ıˆÚËı› ηٿÏÏËÏÔ ÙÔ ‰Â›ÁÌ·, Ú¤ÂÈ Ó· Â›Ó·È Û˘ÁÎÂÎÚÈ̤ÓÔ˜. ∏ ̤ıÔ‰Ô˜ Ô˘ ÂÏ·¯ÈÛÙÔÔÈ› ÙËÓ ÂÈÌfiÏ˘ÓÛË ·fi ÙÔÓ Û›ÂÏÔ Ê·›ÓÂÙ·È Ó· ÂÍ·ÛÊ·Ï›˙ÂÈ ÈÔ ‚ÈÒÛÈÌ· ·ÙÙ·Ú·, ÂÚÈÛÛfiÙÂÚ· ˈÛÈÓfiÊÈÏ· Î·È ˘„ËÏfiÙÂÚË Û˘ÁΤÓÙÚˆÛË Ù˘ ECP Î·È Ù˘Á¯¿ÓÂÈ Â˘Ú‡ÙÂÚ˘ ÂÊ·ÚÌÔÁ‹˜ ·fi ÙË Ì¤ıÔ‰Ô Ô˘ ·Ó·Ï‡ÂÈ ÔÏÈÎfi ‰Â›ÁÌ· Ù˘¤ÏˆÓ Î·È ÛȤÏÔ˘ Ì·˙› (14). ∏ ÏÔÈ‹ ÂÂÍÂÚÁ·Û›· ÙˆÓ Ù˘¤ÏˆÓ (ÔÏÈÎfi ‰Â›ÁÌ· ‹ ‰Â›ÁÌ· ¯ˆÚ›˜ Û›ÂÏÔ) Á›ÓÂÙ·È Ì ÙÔÓ ›‰ÈÔ ÙÚfiÔ Û fiϘ ÙȘ ÌÂϤÙ˜ (Ì ÌÈÎÚ¤˜ ·ÔÎÏ›ÛÂȘ) Î·È ˆ˜ ÚˆÙfiÎÔÏÏ· ·Ó·ÊÔÚ¿˜ ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› Û¯¤‰ÔÓ ¿ÓÙÔÙ ٷ ›‰È· (5,10,11). ∞ÎfiÌË ÌÈ· ‰È·‰ÈηÛÙÈ΋ ·Ó·ÁηÈfiÙËÙ· ·ÔÙÂÏ› Ô ¤ÏÂÁ¯Ô˜ Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ ÂÍÂÙ·˙ÔÌ¤ÓˆÓ Ì ÛÈÚÔ̤ÙÚËÛË. ∞ӷʤÚıËΠÚÔËÁÔ˘Ì¤Óˆ˜ fiÙÈ ÙÔ ˘¤ÚÙÔÓÔ ‰È¿Ï˘Ì· ÌÔÚ› Ó· ÚÔηϤÛÂÈ ‚ÚÔÁ¯fiÛ·ÛÌÔ ¶›Ó·Î·˜ 1. ¢Â›ÎÙ˜ ÊÏÂÁÌÔÓ‹˜ Ô˘ ·ÓȯÓ‡ÔÓÙ·È ÛÙ· Ù‡ÂÏ· ñ ∫‡ÙÙ·Ú· (ˈÛÈÓfiÊÈÏ·, Ô˘‰ÂÙÂÚfiÊÈÏ·, ÌÂÙ·¯ÚˆÌ·ÙÈο, ÏÂÌÊÔ·ÙÙ·Ú·) ñ ∫˘ÙÙÔΛÓ˜ (ÈÓÙÂÚÏ¢ΛÓË-5, ÈÓÙÂÚÏ¢ΛÓË-8 Î.¿.) ñ °Ï˘ÎÔÚˆÙ½Ó˜ (mucin-like glucoprotein MLG Î.¿.) ñ §·ÎÙÔÊÂÚÚ›ÓË ñ πÓˆ‰ÔÁfiÓÔ ñ ∆Ú˘Ù¿ÛË ñ DNA ñ ¶·Ú¿ÁˆÓ ÂÓÂÚÁÔÔ›ËÛ˘ ÙˆÓ Ì·ÎÚÔÊ¿ÁˆÓ ÎÔÎÎÈÔ΢ÙÙ¿ÚˆÓ (GM-CSF)

ηٿ ÙËÓ ÂÈÛÓÔ‹ ÙÔ˘. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ ·ÎfiÌË ÎÈ ·Ó ÚÔËÁËı› Ù˘ ‰È·‰Èηۛ·˜ Ë ¯ÔÚ‹ÁËÛË ‚2-‰ÈÂÁ¤ÚÙË, ı· Ú¤ÂÈ ÚÈÓ ·˘Ù‹ ÍÂÎÈÓ‹ÛÂÈ, Ó· ¤¯ÂÈ Á›ÓÂÈ ÂÎÙ›ÌËÛË ÙÔ˘ FEV1 Î·È Ë ÂͤٷÛË Ó· ÂÈÙÚ·› ÌfiÓÔ fiÙ·Ó Ô FEV1 >80%, ÂÓÒ Ó· ÛÙ·Ì·Ù¿ fiÙ·Ó ˘¿Ú¯ÂÈ ÙÒÛË ÙÔ˘ FEV1 >20% (6,8,13). ∫·Ù·ÓÔԇ̠‰ËÏ·‰‹, fiÙÈ ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰ÈÂÍ·¯ı› Ë Ì¤ıÔ‰Ô˜ ··ÈÙÂ›Ù·È Î·È Ôχ˜ ¯ÚfiÓÔ˜ ·ÏÏ¿ Î·È Ôχ˜ ÎfiÔ˜ ·fi ÙË ÌÂÚÈ¿ ÙÔ˘ ÂÍÂÙ·˙fiÌÂÓÔ˘ Î·È ‰Â˘ÙÂÚ¢fiÓÙˆ˜, ·fi ÙË ÌÂÚÈ¿ ÙÔ˘ ÂÍÂÙ·ÛÙ‹. ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ‰˘Û¯ÂÚ·›ÓÂÈ ÙË Û˘ÓÂÚÁ·Û›· ÙˆÓ ·È‰ÈÒÓ Î·È Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÂÙ·È ÛÔ‚·Ú¿ ˘’ fi„ÈÓ ·Ó ı¤ÏÔ˘Ì ӷ ÙËÓ ÂÊ·ÚÌfiÛÔ˘Ì ˆ˜ ̤ıÔ‰Ô ÚÔ˘Ù›Ó·˜ ÛÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜. ™ÙȘ ÌÂϤÙ˜ ÙˆÓ ÂÓËϛΈÓ, ÂÎÙfi˜ ·fi ÙÔ˘˜ ΢ÙÙ·ÚÈÎÔ‡˜ ÏËı˘ÛÌÔ‡˜ ÙˆÓ Ù˘¤ÏˆÓ ¤¯Ô˘Ó ÌÂÏÂÙËı› Î·È ¿ÏÏÔÈ ‰Â›ÎÙ˜ Ô˘ Ê·›ÓÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1, fiˆ˜ Ë ·fiÙˆÛË (΢ÙÙ·ÚÈÎfi˜ ı¿Ó·ÙÔ˜) ÙˆÓ ËˆÛÈÓÔÊ›ÏˆÓ Î·È ÔÏÏÔ› ¿ÏÏÔÈ (2,15,20). ∆Ô ÓÂfiÙÂÚÔ ÂÚ¢ÓËÙÈÎfi ÂӉȷʤÚÔÓ fï˜, Î·È ÂȉÈο ÛÙ· ·È‰È¿, Ê·›ÓÂÙ·È Ó· ¤¯ÂÈ Î·Ù·Ï‹ÍÂÈ Î·È ‰›ÓÂÈ ÌÂÁ·Ï‡ÙÂÚË ¤ÌÊ·ÛË ÛÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi-ηٷÁÚ·Ê‹ ÙˆÓ Î˘ÙÙ¿ÚˆÓ Ô˘ ·Ó¢ڛÛÎÔÓÙ·È ÛÙ· Ù‡ÂÏ· Î·È ÂȉÈο ÛÙÔ ÔÛÔÛÙfi ÙˆÓ ËˆÛÈÓÔÊ›ÏˆÓ (5,6,8,15,17-19,21,22,29). √È ˘fiÏÔÈÔÈ Î˘ÙÙ·ÚÈÎÔ› ÏËı˘ÛÌÔ› - Ô˘‰ÂÙÂÚfiÊÈÏ·, Ì·ÛÙÔ·ÙÙ·Ú· Î.Ï. - ÌÂÏÂÙÒÓÙ·È ‰Â˘ÙÂÚ¢fiÓÙˆ˜, ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰È·ÈÛÙˆıÔ‡Ó ÎÈ ¿ÏÏÔÈ Ê·ÈÓfiÙ˘ÔÈ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ‹ Û˘ÌÌÂÙÔ¯‹ ¿ÏÏˆÓ ·Ú·ÁfiÓÙˆÓ, fiˆ˜ Â›Ó·È ÁÈ· ·Ú¿‰ÂÈÁÌ· Ë ¤ÎıÂÛË Û ·˘ÍË̤Ó˜ Û˘ÁÎÂÓÙÚÒÛÂȘ fi˙ÔÓÙÔ˜ (15). ∞fi ÙË ‚È‚ÏÈÔÁÚ·Ê›· Ê·›ÓÂÙ·È Ó· ˘¿Ú¯ÂÈ Û˘Ìʈӛ· ˆ˜ ÚÔ˜ Ù· Û˘ÌÂÚ¿ÛÌ·Ù· fiÙÈ ·˘ÍË̤ӷ ˈÛÈÓfiÊÈÏ· ÙˆÓ Ù˘¤ÏˆÓ Û˘Ó‰¤ÔÓÙ·È Ì ÙËÓ ·ÙÔ›·, ·˘ÍË̤ÓÔ ÔÛÔÛÙfi ·ÓÙÈÛÙÚÔÊ‹˜ ÌÂÙ¿ ·fi ‚ÚÔÁ¯Ô‰È·ÛÙÔÏ‹, ÌÂȈ̤ÓÔ ‰Â›ÎÙË FEV1 /FVC, ·˘ÍË̤ӷ ˈÛÈÓfiÊÈÏ· ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· ηıÒ˜ Î·È Ì ·‡ÍËÛË Ù˘ ÛÔ‚·ÚfiÙËÙ·˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ (17,18,21-23). ÕÏÏÔ˜ ‰Â›ÎÙ˘ Ô˘ Ê·›ÓÂÙ·È Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ·ÚÎÂÙ¿ Û˘¯Ó¿, Â›Ó·È Ë ECP ÙˆÓ Ù˘¤ÏˆÓ (Î·È ¿ÏÏˆÓ ËˆÛÈÓÔÊÈÏÈÎÒÓ ÚˆÙÂ˚ÓÒÓ), Ë ÔÔ›· ·fi ÔÚÈṲ̂ÓÔ˘˜ ıˆÚÂ›Ù·È ˆ˜ ÈÔ ·ÍÈfiÈÛÙÔ˜ ‰Â›ÎÙ˘ ÂÎÙ›ÌËÛ˘ Ù˘ ·ÛıÌ·ÙÈ΋˜ ÊÏÂÁÌÔÓ‹˜ ·fi ÙËÓ ECP ÙÔ˘ ÔÚÔ‡ (18,22,24), ηıÒ˜ ·ÓÙ·Ó·ÎÏ¿ ÈÔ ÈÛÙ¿ ÙË ÊÏÂÁÌÔÓÒ‰Ë ‰ÈÂÚÁ·Û›· Ô˘ ˘Ê›ÛÙ·ÓÙ·È ÙÔÈο ÔÈ ÈÛÙÔ› (25). ∂›Û˘, Ë ECP Ù˘¤ÏˆÓ ‚Ú›ÛÎÂÙ·È ÂÏ·Ùو̤ÓË ÌÂÙ¿ ·fi ÙË ¯ÔÚ‹ÁËÛË ıÂÚ·›·˜ Ì ÂÈÛÓÂfiÌÂÓ· ÛÙÂÚÔÂȉ‹ (25), ÂÓÒ Ë ECP ÔÚÔ‡ ÌÔÚ› Î·È Ó· ÌËÓ ÌÂÙ·‚¿ÏÏÂÙ·È (25,24). ¶·È‰È·ÙÚÈ΋ 2006;69:169-177

Pediatr May-June 06

16-05-06

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172

Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

°ÂÓÈο, fiÏÔÈ ÔÈ ‰Â›ÎÙ˜ Ô˘ ÌÂÏÂÙÒÓÙ·È ÛÙ· Ù‡ÂÏ·, Ê·›ÓÂÙ·È Ó· Û˘Û¯ÂÙ›˙ÔÓÙ·È ÙfiÛÔ Ì ÙÔ ·ÛıÌ·ÙÈÎfi ÚÔÊ›Ï ÙˆÓ ·ÛıÂÓÒÓ (Ì ‹ ¯ˆÚ›˜ ¯Ú‹ÛË ÚÔÊ˘Ï·ÎÙÈ΋˜ ·ÁˆÁ‹˜), fiÛÔ Î·È ÌÂٷ͇ ÙÔ˘˜, ·ÏÏ¿ Î·È Ì ¿ÏÏÔ˘˜ ‰Â›ÎÙ˜, fiˆ˜ ÁÈ· ·Ú¿‰ÂÈÁÌ· ÙÔ ÂÎÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘ (eNO) Ì fi¯È Û·Ê‹ Î·È ÛÙ·ıÂÚ‹ Û¯¤ÛË fï˜, Ì ٷ ˆ˜ Ù· ÙÒÚ· ‰Â‰Ô̤ӷ. ™˘ÌÂÚ·ÛÌ·ÙÈο, ı· ϤÁ·Ì fiÙÈ Ë Ì¤ıÔ‰Ô˜ ÙˆÓ ÚÔÎÏËÙÒÓ Ù˘¤ÏˆÓ ÛÙ· ·È‰È¿, fiˆ˜ Î·È ÛÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜, ÌÔÚ› Ó· ‚ÔËı‹ÛÂÈ ÙÔÓ ÎÏÈÓÈÎfi ÁÈ·ÙÚfi Î·È Ó· ÂȂ‚·ÈÒÛÂÈ ‹ Ó· ·ÔÚÚ›„ÂÈ ÙËÓ ‡·ÚÍË ·ÛıÌ·ÙÈÎÔ‡ Ù‡Ô˘ ηٿÛÙ·Û˘, ÛÙÔÓ ‚·ıÌfi Ô˘ ÂÈÙ˘Á¯¿ÓÂÙ·È Ì ÙË ‚ÈÔ„›· Î·È ÙÔ BAL, ›Ù ÚfiÎÂÈÙ·È ÁÈ· ÂÚ›Ô‰Ô ‡ÊÂÛ˘ ›Ù ·ÚÔ͢ÛÌÔ‡ Ù˘ ÓfiÛÔ˘, Ì ‹ ¯ˆÚ›˜ ÚÔÊ˘Ï·ÎÙÈ΋ Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ (2,26,27). √ ·Ú¯ÈÎfi˜ ÂÓıÔ˘ÛÈ·ÛÌfi˜ ÁÈ· ÙÔÓ ÌË ÂÂÌ‚·ÙÈÎfi Î·È ·Ú¿ÏÏËÏ· ‡ÎÔÏÔ, ·ÓÂÎÙfi Î·È ·ÛÊ·Ï‹ ¯·Ú·ÎÙ‹Ú· Ù˘ ÌÂıfi‰Ô˘ ηıÒ˜ Î·È ÁÈ· ÙËÓ Â·Ó·ÏË„ÈÌfiÙËÙ· ÛÙ· ·ÔÙÂϤÛÌ·Ù·, ·ÎfiÌË Î·È fiÙ·Ó ÂÊ·ÚÌfi˙ÂÙ·È Û ·È‰È¿, ÌÔÈ¿˙ÂÈ Ó· ¤¯ÂÈ ·ÓÙÈηٷÛÙ·ı› ·fi ÙËÓ ÈÔ Ú·ÏÈÛÙÈ΋ ıÂÒÚËÛË fiÙÈ Ë Ì¤ıÔ‰Ô˜ Â›Ó·È Û¯ÂÙÈο ÌË ÂÂÌ‚·ÙÈ΋ (ËÌÈÂÂÌ‚·ÙÈ΋) Ì ÔÛÔÛÙfi ·È‰ÈÒÓ Ô˘ ÌÔÚÔ‡Ó Ó· ÙË Ê¤ÚÔ˘Ó ÂȘ ¤Ú·˜ ÌÂٷ͇ 70-80% (18,21). ∞Ó·ÁÓˆÚ›˙ÂÙ·È fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚÔ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ÂÈÙ˘¯›· ‰ÈÂÍ·ÁˆÁ‹˜ Ù˘ ·˘Í¿ÓÂÈ: ·) fiÛÔ ·˘Í¿ÓÂÈ Î·È Ë ËÏÈΛ· ÙˆÓ ÂÍÂÙ·˙fiÌÂÓˆÓ ·È‰ÈÒÓ (21), ‚) fiÙ·Ó ¤¯ÂÈ ÚÔËÁËı› ÂÈÛÓÔ‹ Ì ‚2-‰ÈÂÁ¤ÚÙË (6), οÙÈ Ô˘ ÙÔ˘Ï¿¯ÈÛÙÔÓ ÛÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜ ‰ÂÓ ·ÏÏÔÈÒÓÂÈ Ù· ·ÔÙÂϤÛÌ·Ù· (28), Á) fiÛÔ ÈÔ Û‡ÓÙÔÌÔ˜ Â›Ó·È Ô ¯ÚfiÓÔ˜ ÂÈÛÓÔ‹˜ ÙÔ˘ ˘¤ÚÙÔÓÔ˘ ‰È·Ï‡Ì·ÙÔ˜, οÙÈ Ô˘ ÂÍ·ÚÙ¿Ù·È ·ÓÙÈÛÙÚfiʈ˜ ·Ó¿ÏÔÁ· ·fi ÙË Û˘ÁΤÓÙÚˆÛ‹ ÙÔ˘ (Û˘Ó‹ı˘ Û˘ÁΤÓÙÚˆÛË 4,5%) Î·È Ê˘ÛÈο, ‰) fiÛÔ ÈÔ ¤ÌÂÈÚÔ˜ Â›Ó·È Ô ÂÚ¢ÓËÙ‹˜ Ô˘ ‰ÈÂÍ¿ÁÂÈ ÙË Ì¤ıÔ‰Ô, ηıÒ˜ Î·È ÙÔ ÂÚÁ·ÛÙ‹ÚÈÔ Ô˘ ı· ÂÂÍÂÚÁ·ÛÙ› Ù· ‰Â›ÁÌ·Ù·. À¿Ú¯ÂÈ ·Ó¿ÁÎË Â·ÎÚÈ‚Ô‡˜ ÚÔÛ‰ÈÔÚÈÛÌÔ‡ ÙÔ˘ Ù¯ÓÈÎÔ‡ ÂÍÔÏÈÛÌÔ‡, ÙˆÓ ÚÔ¸Ôı¤ÛÂˆÓ Î·Ù·ÏÏËÏfiÙËÙ·˜ ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜ (ÔÛÔÙÈÎÒÓ Î·È ÔÈÔÙÈÎÒÓ), ÙˆÓ Û˘ÓıËÎÒÓ ÂÂÍÂÚÁ·Û›·˜ ÙÔ˘ (·Ó¿ÏÔÁ· Ì ÙÔ ·Ó ÚfiÎÂÈÙ·È Ó· ÌÂÏÂÙËıÔ‡Ó Î‡ÙÙ·Ú· ‹ ÌÂÛÔÏ·‚ËÙ¤˜ Ù˘ ÊÏÂÁÌÔÓ‹˜) Î·È ÙÈÌÒÓ ·Ó·ÊÔÚ¿˜ ÁÈ· ÙȘ ·Ú·Ì¤ÙÚÔ˘˜ Ô˘ ˘ÔÏÔÁ›˙Ô˘Ì οı ÊÔÚ¿. ∞˘ÙÔ› ÂÍ¿ÏÏÔ˘ Ú¤ÂÈ Ó· Â›Ó·È Î·È ÔÈ ÏfiÁÔÈ Ô˘ ÙfiÛ· ¯ÚfiÓÈ· ÌÂÙ¿ ·fi ÙËÓ ·Ú¯È΋ ÂÊ·ÚÌÔÁ‹ Ù˘, ·Ú·Ì¤ÓÂÈ ·ÎfiÌ· ÂÚ¢ÓËÙÈÎfi ÂÚÁ·ÏÂ›Ô ·Ú¿ ̤ıÔ‰Ô˜ ÚÔ˘Ù›Ó·˜ ÁÈ· ÙË ‰È¿ÁÓˆÛË Î·È ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ÔÚ›·˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿ (5,18). ¶·È‰È·ÙÚÈ΋ 2006;69:169-177

∂ÎÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘ (eNO) √È ÌÂÙÚ‹ÛÂȘ ÙÔ˘ eNO ¤¯Ô˘Ó ÚÔÙ·ı› ˆ˜ ÌÈ· ‡ÎÔÏË ÌË ÂÂÌ‚·ÙÈ΋ ̤ıÔ‰Ô˜ Ù˘ ÂÎÙ›ÌËÛ˘ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Û ·ÛıÌ·ÙÈÎÔ‡˜ ·ÛıÂÓ›˜, ηıÒ˜ Î·È ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ÓfiÛÔ˘ (monitoring) ·ÎfiÌ· Î·È ÌÂÙ¿ ·fi ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË (30-33). ÃÚËÛÈÌÔÔÈÂ›Ù·È Ôχ ÂÚÈÛÛfiÙÂÚÔ Î·ÈÚfi ·fi ÙË Ì¤ıÔ‰Ô ÙˆÓ ÚÔÎÏËÙÒÓ Ù˘¤ÏˆÓ Î·È Û¯ÂÙÈο Ì ·˘Ù‹Ó ˘¿Ú¯Ô˘Ó ÈÔ ÙÂÎÌËÚȈ̤Ó˜-‰ÔÎÈÌ·Ṳ̂Ó˜ ηÙ¢ı˘ÓÙ‹ÚȘ Ô‰ËÁ›Â˜ (guidelines) ˆ˜ ÚÔ˜ ÙÔÓ ÙÚfiÔ ˘ÔÏÔÁÈÛÌÔ‡, ÙÔÓ ÂÍÔÏÈÛÌfi Î·È ÙȘ ÙÈ̤˜ ·Ó·ÊÔÚ¿˜ (32,34,35). ∆Ô ¡√ Û˘ÓÙ›ıÂÙ·È ·fi ÙÔ ·ÌÈÓÔ͇ L-·ÚÁÈÓ›ÓË Ì ÙË ‚Ô‹ıÂÈ· ÙˆÓ 3 Ù‡ˆÓ ÙÔ˘ ÂÓ˙‡ÌÔ˘ ¡√ Û˘ÓıÂÙ¿ÛË. ªÔÚ› Ó· ÌÂÙÚËı› ÛÙÔÓ ÂÎÓÂfiÌÂÓÔ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ·¤Ú· ·ÏÏ¿ Î·È ·›ÚÓÔÓÙ·˜ ÙÔ ‰Â›ÁÌ· ·¢ı›·˜ ·fi ÙȘ ÚÈÓÈΤ˜ ÎÔÈÏfiÙËÙ˜. ™ÙËÓ ÚÒÙË ÂÚ›ÙˆÛË, ÌÈÏ¿Ì ÁÈ· ÙÔ ÂÎÓÂfiÌÂÓÔ ¡√ Ô˘ ‰ÈÂıÓÒ˜ Û˘Ì‚ÔÏ›˙ÂÙ·È ˆ˜ eNO, ÂÓÒ ÛÙË ‰Â‡ÙÂÚË, ÁÈ· ÙÔ ÚÈÓÈÎfi ¡√ Ô˘ Û˘Ì‚ÔÏ›˙ÂÙ·È ˆ˜ nNO. ¶·Ú¿ ÙËÓ Â˘Ú›· ÌÂϤÙË ÙÔ˘ e¡√ Î·È ÙËÓ È‰È·›ÙÂÚË Â˘ÎÔÏ›· Ô˘ ¤¯ÂÈ Ë Ï‹„Ë Î·È Ô ˘ÔÏÔÁÈÛÌfi˜ ÙÔ˘ Û fiϘ ÙȘ ËÏÈÎȷΤ˜ ÔÌ¿‰Â˜, ·ÎfiÌ· Î·È ÛÙ· ‚Ú¤ÊË (36), Ë ‚È‚ÏÈÔÁÚ·Ê›· Ê·›ÓÂÙ·È ‰È¯·Ṳ̂ÓË ˆ˜ ÚÔ˜ ÙËÓ ·Í›· ·˘ÙÔ‡ ÙÔ˘ ‰Â›ÎÙË. ∞˘Ùfi Ô˘ Â›Ó·È ÎÔÈÓÒ˜ ·Ô‰ÂÎÙfi Â›Ó·È fiÙÈ ÙÔ eNO ‚Ú›ÛÎÂÙ·È ·˘ÍË̤ÓÔ ÙfiÛÔ ÛÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜ fiÛÔ Î·È ÛÙ· ·È‰È¿ Ì ¿ÛıÌ· (31,32,37). ∂›Û˘, ·˘ÍË̤ӷ Â›‰¿ ÙÔ˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Ù· ¿ÙÔÌ· Ì ‚ÚÔÁ¯È΋ ˘ÂÚ·ÓÙȉڷÛÙÈÎfiÙËÙ·, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ ÈÛÙÔÚÈÎfi ·ÛıÌ·ÙÈÎÒÓ Û˘Ìو̿وÓ, Ù· ¿ÙÔÌ· Ô˘ ÂÌÊ·Ó›˙Ô˘Ó Û˘ÚÈÁÌfi Î·È fi¯È ·Ï¿ ÚÔ‚Ï‹Ì·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi (fiˆ˜ ÍËÚfi-Â›ÌÔÓÔ ‚‹¯· ÌfiÓÔ) Î·È ÛÙËÓ ·ÙÔ›· ÁÂÓÈÎfiÙÂÚ· (41,42). ∫·Ù¿ ¿ÏÏÔ˘˜ Ë ·ÙÔ›· ‰ÂÓ ÂËÚ¿˙ÂÈ ÙȘ ÙÈ̤˜ ÙÔ˘ (43). ∂›Ó·È Â›Û˘ ÁÓˆÛÙfi, fï˜, fiÙÈ Ù· Â›‰¿ ÙÔ˘ ·Ó¢ڛÛÎÔÓÙ·È ·˘ÍË̤ӷ ÌÂÙ¿ ·fi ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ‹ Û ¿ÏϘ ·ı‹ÛÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ (38). ∏ ıÂÙÈ΋ Î·È Ë ·ÚÓËÙÈ΋ ÚÔÁÓˆÛÙÈ΋ ·Í›· ÛÙo ¿ÛıÌ· Ì ¯Ú‹ÛË ÙÔ˘ e¡√, ¤¯ÂÈ ‚ÚÂı› Û ÌÈ· ÌÂϤÙË ˆ˜ 100% Î·È 80% ·ÓÙ›ÛÙÔȯ· (39), ÂÓÒ ÔÈ Jones et al ‚Ú‹Î·Ó fiÙÈ ÔÈ ÌÂÙÚ‹ÛÂȘ eNO ¤¯Ô˘Ó ıÂÙÈ΋ ÚÔÁÓˆÛÙÈ΋ ·Í›· ÌÂٷ͇ 80-90%, ÁÈ· ÙËÓ ÚfiÁÓˆÛË Î·È ÙË ‰È¿ÁÓˆÛË ·ÒÏÂÈ·˜ ÙÔ˘ ÂϤÁ¯Ô˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜. ∞ӷʤÚÔ˘Ó Â›Û˘, fiÙÈ Â›Ó·È ÙÔ ›‰ÈÔ ¯Ú‹ÛÈ̘ Ì ٷ ˈÛÈÓfiÊÈÏ· ÙˆÓ

Pediatr May-June 06

16-05-06

17:21

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173

ªË ÂÂÌ‚·ÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿

ÚÔÎÏËÙÒÓ Ù˘¤ÏˆÓ, ¤¯ÔÓÙ·˜ fï˜ ÙÔ ÏÂÔÓ¤ÎÙËÌ· Ù˘ ¢ÎÔÏ›·˜ ÛÙË ¯Ú‹ÛË (44). ∂ÈϤÔÓ, ÁÓˆÚ›˙Ô˘Ì fiÙÈ ·ÓÙ·ÔÎÚ›ÓÂÙ·È Ì Ì›ˆÛË ÙˆÓ ÂÈ¤‰ˆÓ ÙÔ˘ ηÙfiÈÓ ·ÁˆÁ‹˜ Ì ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ (30,39,40), ÂÓÒ Ê·›ÓÂÙ·È fiÙÈ ÌÂÈÒÓÂÙ·È Î·È ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ·ÓÙ·ÁˆÓÈÛÙÒÓ Ï¢ÎÔÙÚÈÂÓ›ˆÓ (40). ŸÙ·Ó ÙÔ e¡√ ÚfiÎÂÈÙ·È Ó· Û˘Û¯ÂÙÈÛÙ› Ì ¿ÏϘ ·Ú·Ì¤ÙÚÔ˘˜ ·Ó›¯Ó¢Û˘ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ, ηıÒ˜ Î·È Ì ÌÂÙÚ‹ÛÂȘ Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Î·È Â‰Ò Ê·›ÓÂÙ·È Ó· Â›Ó·È ·Û·Ê‹ Î·È ·ÓÂ·Ú΋. ŒÙÛÈ, Û ÔÚÈṲ̂Ó˜ ÌÂϤÙ˜, ÙÔ e¡√ ‰ÂÓ Û˘Û¯ÂÙ›˙ÂÙ·È Î·ıfiÏÔ˘ Ì ÙȘ ÙÈ̤˜ ÙÔ˘ FEV1 (33,39) ‹ Ù˘ PEFR, (33) ÂÓÒ Ù· Â›‰· ÙÔ˘ e¡√ Û¯ÂÙ›˙ÔÓÙ·È ıÂÙÈο Ì ٷ ˈÛÈÓfiÊÈÏ· ÙˆÓ Ù˘¤ÏˆÓ Î·È ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ (33,44), Ì ÙËÓ ECP ÙˆÓ Ù˘¤ÏˆÓ Î·È ÙÔ˘ ÔÚÔ‡ (33). ªÂÁ·Ï‡ÙÂÚË Û˘Ìʈӛ· ÌÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ ÂÚ¢ÓÒÓ Ê·›ÓÂÙ·È Ó· ˘¿Ú¯ÂÈ ÛÙÔÓ ÚfiÏÔ ÙÔ˘ e¡√ ÛÙË ‰È·ÊÔÚԉȿÁÓˆÛË ÙÔ˘ steroidnaive asthma (29,40), fiÔ˘ Ê·›ÓÂÙ·È fiÙÈ ıˆÚÂ›Ù·È ÂÍ›ÛÔ˘ ηÏfi˜ ‰Â›ÎÙ˘ Ì ÙÔ ÔÛÔÛÙfi ÙˆÓ ËˆÛÈÓÔÊ›ÏˆÓ ÛÙ· Ù‡ÂÏ· (29). ªÂ ÛÈÁÔ˘ÚÈ¿ ÌÔÚԇ̠ӷ ԇ̠ÌfiÓÔ fiÙÈ ÙÔ e¡√ Â›Ó·È ÌÈ· ·ÚÎÂÙ¿ ‡ÎÔÏË Ì¤ıÔ‰Ô˜ Ô˘ ‰›ÓÂÈ ··ÓÙ‹ÛÂȘ Û ‰È·ÊÔÚԉȷÁÓˆÛÙÈο ÚÔ‚Ï‹Ì·Ù·, ÌÔÚ› Ó· ÂÊ·ÚÌÔÛÙ› Ì ·ÛÊ¿ÏÂÈ· ·ÎfiÌ· Î·È Û Ôχ ÌÈÎÚ¤˜ ËÏÈ˘ ˆ˜ ̤ıÔ‰Ô˜ ÚÔ˘Ù›Ó·˜, ·ÏÏ¿ ÔÈ ÙÈ̤˜ ÙÔ˘ ÂËÚ¿˙ÔÓÙ·È ·fi ÔÏÏÔ‡˜ ·Ú¿ÁÔÓÙ˜ Î·È ı· Ú¤ÂÈ Ó· Û˘ÓÂÎÙÈÌ¿Ù·È Î·È Ì ¿ÏÏÔ˘˜ ÌÂÛÔÏ·‚ËÙ¤˜ ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÍ·¯ıÔ‡Ó ·ÛÊ·Ï‹ Û˘ÌÂÚ¿ÛÌ·Ù·, Èηӿ Ó· ηıÔÚ›ÛÔ˘Ó ÙË ÛÙ¿ÛË ÙÔ˘ ÎÏÈÓÈÎÔ‡ ÁÈ·ÙÚÔ‡ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÌÈÎÚÒÓ ·ÛıÂÓÒÓ ÙÔ˘.

™˘Ì‡Îӈ̷ ÂÎÓÂfiÌÂÓÔ˘ ·¤Ú· (Exhaled breath condensate) ªÈ· ̤ıÔ‰Ô˜ Ô˘ ‚·Û›˙ÂÙ·È Î·È ·˘Ù‹ ÛÙËÓ ·Ó¿Ï˘ÛË ÙÔ˘ ÂÎÓÂfiÌÂÓÔ˘ ·¤Ú· ·fi ÙÔ˘˜ Ó‡ÌÔÓ˜ Â›Ó·È Ë Û˘ÏÏÔÁ‹ Î·È ·Ó¿Ï˘ÛË ÙÔ˘ Û˘Ì˘ÎÓÒÌ·ÙÔ˜ ÙÔ˘ ÂÎÓÂfiÌÂÓÔ˘ ·¤Ú· Ô˘ ÛÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· ¤¯ÂÈ ÔÚÈÛÙ› ˆ˜ EBC Î·È ¤ÙÛÈ ı· ·Ó·Ê¤ÚÂÙ·È ÛÙË Û˘Ó¤¯ÂÈ· Î·È ÛÙË ÌÂϤÙË ·˘Ù‹. √ ÂÎÓÂfiÌÂÓÔ˜ ·¤Ú·˜ Ô˘ ı· Û˘Ì˘ÎÓˆı› ÚÔÎÂÈ̤ÓÔ˘ Ó· ·Ó·Ï˘ı›, ÚÔ¤Ú¯ÂÙ·È ·fi ÙÔ Î·ÙÒÙÂÚÔ ·Ó·Ó¢ÛÙÈÎfi Û‡ÛÙËÌ· Î·È ÂÚȤ¯ÂÈ ·ÂÚÔχ̷ٷ Î·È ˘ÁÚ¿ ÛÙ·ÁÔÓ›‰È· ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ̤۷ Û ¤Ó· ÂÚÈ‚¿ÏÏÔÓ ÎÔÚÂṲ̂ÓÔ ·fi ˘‰Ú·ÙÌÔ‡˜. √È ˘‰Ú·ÙÌÔ› ·˘ÙÔ› Â›Ó·È Ô˘

ı· Û˘Ì˘ÎÓˆıÔ‡Ó Ì ÙË ‚Ô‹ıÂÈ· ÂÓfi˜ Û˘ÛÙ‹Ì·ÙÔ˜ „‡Í˘ ‹ ηٿ„˘Í˘, ÂÓÒ Ù· ˘ÁÚ¿ ۈ̷ٛ‰È· ·ÂÏ¢ıÂÚÒÓÔÓÙ·È ·fi ÙÔ ˘ÁÚfi Ô˘ ˘¿Ú¯ÂÈ ÛÙÔ ‚ÏÂÓÓÔÎÚÔÛÛˆÙfi ÂÈı‹ÏÈÔ (lung epithelial lining fluid) Î·È ÂÚȤ¯Ô˘Ó ›¯ÓË ·fi ‰È·Ï˘Ì¤Ó˜ ÌË ÙËÙÈΤ˜ Ô˘Û›Â˜ Ù· ÔÔ›· Î·È ı· ·ÓȯÓ¢ÙÔ‡Ó ÛÙÔ EBC (45-47,54). ªÂÛÔÏ·‚ËÙ¤˜ Ù˘ ÊÏÂÁÌÔÓ‹˜ Ì ÌÔÚÈ·Îfi ‚¿ÚÔ˜ <65 kD ·Ó¢ڛÛÎÔÓÙ·È Ì¤Û· ÙÔ˘˜ ÂÎÓÂfiÌÂÓÔ˘˜ ˘‰Ú·ÙÌÔ‡˜ ÛÂ Ê˘ÛÈÔÏÔÁÈο ¿ÙÔÌ· ·ÏÏ¿ Î·È Û ·ÛıÌ·ÙÈÎÔ‡˜ ·ÛıÂÓ›˜ (48). √È ÊÏÂÁÌÔÓÒ‰ÂȘ ÓfiÛÔÈ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ ÂËÚ¿˙Ô˘Ó ÙÔÓ Û¯ËÌ·ÙÈÛÌfi ÙˆÓ ·ÂÚÔÏ˘Ì¤ÓˆÓ ÛˆÌ·Ùȉ›ˆÓ ÌÂ Û˘ÁÎÂÎÚÈ̤ÓÔ ÙÚfiÔ (49). §fiÁˆ ÙÔ˘ fiÙÈ ÚfiÎÂÈÙ·È ÁÈ· ÌÈ· Ó¤· ̤ıÔ‰Ô Û ÂÚ¢ÓËÙÈÎfi ÛÙ¿‰ÈÔ, Ô Ù¯ÓÈÎfi˜ ÂÍÔÏÈÛÌfi˜ ÁÈ· ÙË Û˘Ì‡ÎÓˆÛË ‰È·Ê¤ÚÂÈ ·fi ÂÚÁ·ÛÙ‹ÚÈÔ Û ÂÚÁ·ÛÙ‹ÚÈÔ. ∏ ·Ú¯‹ ÛÙËÓ ÔÔ›· ‚·Û›˙ÔÓÙ·È fï˜ fiÏÔÈ, Â›Ó·È ·˘Ù‹ Ù˘ „‡Í˘ ÙÔ˘ ÂÎÓÂfiÌÂÓÔ˘ ·¤Ú· (50-53). ™‡Ìʈӷ Ì ÙËÓ ÚfiÛÊ·ÙË Î·È ÏÂÙÔÌÂÚ‹ ·Ó·ÛÎfiËÛË ÙˆÓ Rosia Î·È Û˘Ó, Ù· ˘ÏÈο Ô˘ Â·ÏÏ›ÊÔ˘Ó ÙËÓ ÂÛˆÙÂÚÈ΋ ÂÈÊ¿ÓÂÈ· ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ Û˘Ì‡ÎÓˆÛ˘ ‹ ÙÔ˘ ‰Ô¯Â›Ô˘ Û˘ÏÏÔÁ‹˜, ÌÔÚ› Ó· ÂËÚ¿ÛÔ˘Ó ÙË Û˘ÁΤÓÙÚˆÛË ÙˆÓ ‰È·ÊfiÚˆÓ ÊÏÂÁÌÔÓˆ‰ÒÓ ·Ú·ÁfiÓÙˆÓ. ∞ÏÏ¿ ÂÎÙfi˜ ·fi ÙȘ ÂοÛÙÔÙ ·ÚÂÌ‚¿ÛÂȘ ÏfiÁˆ Ù˘ ηٷÛ΢·ÛÙÈ΋˜ ʇÛ˘ Ù˘ ÌÂıfi‰Ô˘, Ù· ·ÔÙÂϤÛÌ·Ù· Î·È Ë Â·Ó·ÏË„ÈÌfiÙËÙ· ÙˆÓ ÌÂÙÚ‹ÛÂˆÓ ÂÍ·ÚÙÒÓÙ·È ÛËÌ·ÓÙÈο: ·) ·fi ÙËÓ ¤ÓÙ·ÛË Ù˘ ÂÎÓ¢ÛÙÈ΋˜ ÚÔ‹˜, ‚) ÙËÓ ÂÚÈ‚·ÏÏÔÓÙÈ΋ ıÂÚÌÔÎÚ·Û›·, ÙË ıÂÚÌÔÎÚ·Û›· Ù˘ ·Ó·ÓÔ‹˜ Î·È ÙË ıÂÚÌÔÎÚ·Û›· Û˘ÓÙ‹ÚËÛ˘ ÙÔ˘ Û˘Ì˘ÎÓÒÌ·ÙÔ˜, Á) ·fi ÙËÓ ÂÚÈÂÎÙÈÎfiÙËÙ· ÙÔ˘ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ Û fi˙ÔÓ ÙËÓ ÒÚ· Ù˘ Û˘ÏÏÔÁ‹˜ ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜, ‰) ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ÎÈÚο‰ÈÔ˘ Ú˘ıÌÔ‡, Â) ÙËÓ ÂÈÌfiÏ˘ÓÛË ·fi ÙÔÓ ÚÈÓÈÎfi ·¤Ú· ‹ ÙÔÓ Û›ÂÏÔ Î·È ÔÏÏ¿ ¿ÏÏ· ·ÎfiÌË (54). ∏ ›‰È· ·Ó·ÛÎfiËÛË Î·Ù·Ï‹ÁÂÈ ÛÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ ÌÈ· ‰È·‰Èηۛ· Ô˘ ·ÎÔÏÔ˘ıÂ›Ù·È ÁÈ· ¤Ó·Ó ÌÂÛÔÏ·‚ËÙ‹, ÌÔÚ› Ó· ÌËÓ Â›Ó·È Î·Ù¿ÏÏËÏË Î·È ÁÈ· οÔÈÔÓ ¿ÏÏÔ, Î·È Ú¤ÂÈ ¿ÓÙ· ÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙȘ Û˘ÁÎÂÓÙÚÒÛÂȘ ÙÔ˘˜ Ó· Û˘Ó˘ÔÏÔÁ›˙ÔÓÙ·È Î·È Ó· ·ÍÈÔÏÔÁÔ‡ÓÙ·È ·Ó¿ÏÔÁ· (54). ∞Ó·Áη›Ô Â›Ó·È Â›Û˘, Ó· Û˘ÁÎÂÎÚÈÌÂÓÔÔÈËıÔ‡Ó ÔÈ ÙÈ̤˜ ·Ó·ÊÔÚ¿˜, ÚÔÎÂÈ̤ÓÔ˘ Ó· Á›ÓÂÈ ÂÊÈÎÙfi˜ Ô ˘ÔÏÔÁÈÛÌfi˜ Ù˘ ÂȉÈÎfiÙËÙ·˜ Î·È Ù˘ ¢·ÈÛıËÛ›·˜ Ù˘ ÌÂıfi‰Ô˘ ·ÏÏ¿ Î·È Ù˘ Â·Ó·ÏË„ÈÌfiÙËÙ·˜ ÙˆÓ ÌÂÙÚ‹ÛÂˆÓ (47,54,55,58). ªÈ· ÏËıÒÚ· ‰ÂÈÎÙÒÓ ¤¯Ô˘Ó ÌÂÙÚËı› ÛÙÔ EBC fiˆ˜ Ê·›ÓÂÙ·È Î·È ÛÙÔÓ ¶›Ó·Î· 2. ∫·Ù¿ ηÈÚÔ‡˜, ¤¯Ô˘Ó ·Ó·ÎÔÈÓˆı› Û˘Û¯ÂÙ›ÛÂȘ ÙˆÓ ¶·È‰È·ÙÚÈ΋ 2006;69:169-177

Pediatr May-June 06

16-05-06

17:21

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Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

·Ó¢ÚÈÛÎÔÌ¤ÓˆÓ Û˘ÁÎÂÓÙÚÒÛÂˆÓ ÙˆÓ ‰ÂÈÎÙÒÓ ·˘ÙÒÓ Ì ÙËÓ ·ÚÔ˘Û›· ¿ÛıÌ·ÙÔ˜ ·ÏÏ¿ Î·È ¿ÏÏˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ·ı‹ÛÂˆÓ Î·ıÒ˜ Î·È ÙÚÔÔÔ›ËÛ‹ ÙÔ˘˜ Ì ÙË ¯ÔÚ‹ÁËÛË ıÂÚ·›·˜. ∂Ó‰ÂÈÎÙÈο ·Ú·‰Â›ÁÌ·Ù· Â›Ó·È Ô ·˘ÍË̤ÓÔ˜ Û¯ËÌ·ÙÈÛÌfi˜ ÂÎÓÂfiÌÂÓˆÓ Î˘ÛÙÂ˚Ó‡Ï-Ï¢ÎÔÙÚÈÂÓ›ˆÓ (cysteinyl leukotriens) Î·È Ï¢ÎÔÙÚÈÂÓ›ˆÓ µ4 (LTB4) ÛÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ ·È‰ÈÒÓ Ì ‹ÈÔ Î·È Ì¤ÙÚÈÔ ÚÔ˜ ÛÔ‚·Úfi Â›ÌÔÓÔ ¿ÛıÌ·, ˘fi ·ÁˆÁ‹ Ì ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, Û ۯ¤ÛË Ì ˘ÁÈ›˜ Ì¿ÚÙ˘Ú˜, ÁÂÁÔÓfi˜ Ô˘ ÌÔÚ› Ó· ·ÓÙ·Ó·ÎÏ¿ ÙË Û˘ÌÌÂÙÔ¯‹ ÙˆÓ Ï¢ÎÔÙÚÈÂÓ›ˆÓ ·˘ÙÒÓ ÛÙË ¯ÚfiÓÈ· ÊÏÂÁÌÔÓ‹ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ (53). ∂›Û˘, ÌÔÚ› Ó· ÛËÌ·›ÓÂÈ fiÙÈ Ô ÏfiÁÔ˜ πL-4/IFN-Á Â›Ó·È ·˘ÍË̤ÓÔ˜, οÙÈ Ô˘ Û˘ÌʈÓ› Ì ÙËÓ Î˘ÚÈ·Ú¯›· ÙˆÓ Th2 ΢ÙÙ¿ÚˆÓ ÛÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ ÙˆÓ ·È‰ÈÒÓ Ì ¿ÛıÌ· (52). ™ËÌ·ÓÙÈο Â›Ó·È Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ô˘ Û˘Ó‰¤Ô˘Ó ÙÔ˘˜ ‰Â›ÎÙ˜ ÙÔ˘ EBC Ì ÙȘ ÌÂÙÚ‹ÛÂȘ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, fiˆ˜ ÛÙË ÌÂϤÙË ÙˆÓ Straub Î·È Û˘Ó, fiÔ˘ ‚Ú¤ıËΠfiÙÈ ÔÈ ÌÂÙÚ‹ÛÂȘ ÙˆÓ ÓÈÙÚˆ‰ÒÓ ÌÔÚÔ‡Ó Ó· ·ÔÙÂϤÛÔ˘Ó ÙÔÓ ·ÓÙÈÎÂÈÌÂÓÈÎfi ÙÚfiÔ ÂϤÁ¯Ô˘ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÛÙ· ·ÛıÌ·ÙÈο ·È‰È¿, ¤ÙÛÈ ÒÛÙ ӷ ·ÓȯÓ‡ÂÙ·È Û˘Ó¯È˙fiÌÂÓË ÊÏÂÁÌÔÓ‹ ·ÎfiÌ· Î·È ÛÙ· ·È‰È¿ ÂΛӷ ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ FEV1 ·ÏÏ¿ Â›ÌÔÓ· Û˘ÌÙÒÌ·Ù· (55). √È ÌÂϤÙ˜ ÛÙÔ EBC, fiÛÔÓ ·ÊÔÚ¿ ÙÔÓ ·È‰È·ÙÚÈÎfi ÏËı˘ÛÌfi ΢ڛˆ˜, Â›Ó·È Ï›Á˜, ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈ‹ÛÂÈ ÌÈÎÚ¿ Û ·ÚÈıÌfi ‰Â›ÁÌ·Ù· ·ÛıÂÓÒÓ Î·È Ê˘ÛÈÔÏÔÁÈÎÒÓ Ì·ÚÙ‡ÚˆÓ Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù¿ ÙÔ˘˜ ‰È·Ù˘ÒÓÔÓÙ·È ÂÚÈÛÛfiÙÂÚÔ ˆ˜ ·Ú·ÙËÚ‹ÛÂȘ ·Ú¿ ˆ˜ ÈÛ¯˘Ú¿ ·ԉ‰ÂÈÁ̤Ó˜ ÚÔÙ¿ÛÂȘ. ∏ ·ÈÛÈÔ‰ÔÍ›· ÙˆÓ ÂÚ¢ÓËÙÒÓ fiÛÔÓ ·ÊÔÚ¿ ÙË ¯Ú‹ÛË ÙÔ˘ EBC ËÁ¿˙ÂÈ ·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Â›Ó·È ÌÈ· ‡ÎÔÏË Ì¤ıÔ‰Ô˜, ȉȷÈÙ¤Úˆ˜ ÌË ÂÂÌ‚·ÙÈ΋, ÂÊ·ÚÌfiÛÈÌË Û οı ËÏÈΛ·, ·ÎfiÌË Î·È ¶›Ó·Î·˜ 2. ¢Â›ÎÙ˜ ÊÏÂÁÌÔÓ‹˜ Ô˘ ÌÂÙÚÒÓÙ·È ÛÙÔ EBC ñ ÀÂÚÔÍ›‰ÈÔ ÙÔ˘ ˘‰ÚÔÁfiÓÔ˘ (Hydrogen peroxide H2O2) ñ ∂ÈÎÔÛ·ÓÔÂȉ‹ (ÚÔÛÙ·ÁÏ·Ó‰›Ó˜, ıÚÔÌ‚ÔÍ¿Ó˜, ÈÛÔÚÔÛÙ¿Ó˜, Ï¢ÎÔÙÚȤÓ˜ Î.¿.) ñ ¢Ú·ÛÙÈο ›‰Ë ÙÔ˘ ·˙ÒÙÔ˘ (ÓÈÙÚÒ‰Ë/ÓÈÙÚÈο, S-ÓÈÙÚÔ˙ÔıÈfiÏË, 3-ÓÈÙÚÔÙ˘ÚÔÛ‡ÓË) ñ ¢Â›ÎÙ˜ ÔÍÂȉˆÙÈÎÔ‡ ÛÙÚ˜ (ÁÏÔ˘Ù·ıÈfiÓË, Ì·ÏÔÓ‰˘·Ï‰Â˛‰Ë, ·,‚-·ÎfiÚÂÛÙ˜ ·Ï‰Â˛‰Â˜) ñ πÓÙÂÚÊÂÚfiÓ˜ (ÈÓÙÂÚÊÂÚfiÓË Á) ñ ∫˘ÙÙÔΛÓ˜ ñ ∞ÓÔÛÔÛÊ·ÈÚ›ÓË E (IgE) ñ ∏ˆÛÈÓÔÊÈÏÈΤ˜ ÚˆÙ½Ó˜ (ˈÛÈÓÔÊÈÏÈ΋ ηÙÈÔÓÈ΋ ÚˆÙ½ÓË Î.¿.) ñ ¢Â›ÎÙ˘ Ô͇ÙËÙ·˜ ÛÙÔ EBC (pH) ¶·È‰È·ÙÚÈ΋ 2006;69:169-177

ÛÙ· ‚Ú¤ÊË, ›Ù ˘¿Ú¯ÂÈ ·˘ÙfiÌ·ÙË ·Ó·ÓÔ‹ ›Ù ·ÎfiÌ· Î·È Ì˯·ÓÈÎfi˜ ·ÂÚÈÛÌfi˜ (56,58).

∏ˆÛÈÓÔÊÈÏÈΤ˜ ÚˆÙ½Ó˜ ÛÙÔ ·›Ì· Î·È ÛÙ· Ô‡Ú· ∂›Ó·È ÁÓˆÛÙfi ·fi ηÈÚfi, fiÙÈ ·Ú¿ÁˆÁ· ÙˆÓ ËˆÛÈÓÔʛψÓ, ΢ڛˆ˜ ÚˆÙ½Ó˜ Ô˘ ·ÓÙ·Ó·ÎÏÔ‡Ó Î·Ù·ÛÙ¿ÛÂȘ fiˆ˜ Ë ‰È¤ÁÂÚÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ·˘ÙÒÓ, ·Ó¢ڛÛÎÔÓÙ·È Û ÔÏÏ¿ ‚ÈÔÏÔÁÈο ˘ÁÚ¿ Ì ȉȷ›ÙÂÚË ¤ÌÊ·ÛË ÛÙËÓ ·Ó¿Ï˘ÛË ‰ÂÈÁÌ¿ÙˆÓ ·›Ì·ÙÔ˜ Î·È ‰Â˘ÙÂÚ¢fiÓÙˆ˜, Ô‡ÚˆÓ (59). ªÂٷ͇ ÙˆÓ ÚÔ˚fiÓÙˆÓ ÙˆÓ ËˆÛÈÓÔʛψÓ, ÈÔ ÂȉÈΤ˜ ıˆÚÔ‡ÓÙ·È Ë ËˆÛÈÓÔÊÈÏÈ΋ ηÙÈÔÓÈ΋ ÚˆÙ½ÓË (ECP) Î·È Ë ËˆÛÈÓÔÊÈÏÈ΋ ÂÚÔÍÂȉ¿ÛË (EPO), (60,61) ÂÓÒ Ë ËˆÛÈÓÔÊÈÏÈ΋ ÚˆÙ½ÓË Ã/Ó¢ÚÔÙÔÍ›ÓË ÚÔÂÚ¯fiÌÂÓË ·fi Ù· ˈÛÈÓfiÊÈÏ· (EPX/EDN), ·Ú¿ÁÂÙ·È Û ÌÈÎÚ¿ ÔÛ¿ Î·È ·fi Ù· Ô˘‰ÂÙÂÚfiÊÈÏ·. √ ‰Â›ÎÙ˘ Ô˘ ˆ˜ ÙÒÚ· Â›Ó·È ÈÔ Â˘Ú¤ˆ˜ ¯ÚËÛÈÌÔÔÈÔ‡ÌÂÓÔ˜ ÛÙËÓ Î·ıËÌÂÚÈÓ‹ ÎÏÈÓÈ΋ ÚÔ˘Ù›Ó· Â›Ó·È Ë ECP, Ô˘ Û˘Ó‹ıˆ˜ ÌÂÙÚ¿Ù·È ÛÙÔÓ ÔÚfi ÙÔ˘ ·›Ì·ÙÔ˜ (62). ∆· Â›‰· Ù˘ ECP ÛÙÔÓ ÔÚfi ·ÓÙ·Ó·ÎÏÔ‡Ó ÙfiÛÔ Ù· ΢ÎÏÔÊÔÚÔ‡ÓÙ· Â›‰· fiÛÔ Î·È ÙËÓ ÂÎÎÚÈÙÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ ËˆÛÈÓÔÊ›ÏˆÓ ÛÙÔ ·›Ì· (63). ŸÌˆ˜ Î·È Û ·˘Ù¤˜ ÙȘ ÌÂÙÚ‹ÛÂȘ ÛËÌ·Û›· ¤¯ÂÈ Ë ÂοÛÙÔ٠ٯÓÈ΋ Ï‹„˘ Î·È ·ÍÈÔÏfiÁËÛ˘, ηıÒ˜ ¤¯ÂÈ ‰Âȯı› fiÙÈ ‰È·ÊÔÚÂÙÈΤ˜ ÙÈ̤˜ Ù˘ ECP ‚Ú›ÛÎÔÓÙ·È ·Ó¿ÏÔÁ· Ì ÙȘ Û˘Óı‹Î˜ Î·È Ù· ˘ÏÈο Ô˘ ı· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó (62-64). ∂›Û˘, Ú¤ÂÈ Ó· ÌËÓ Í¯ӿÌ fiÙÈ fiÏÔÈ ÔÈ ËˆÛÈÓÔÊÈÏÈÎÔ› ‰Â›ÎÙ˜ ˘fiÎÂÈÓÙ·È Û ÌÂÙ·‚ÔϤ˜ Û‡Ìʈӷ Ì ÙÔÓ ÎÈÚο‰ÈÔ Ú˘ıÌfi, Ì ٷ ÌÂÁ·Ï‡ÙÂÚ· Â›‰¿ ÙÔ˘˜ Ó· ·Ó¢ڛÛÎÔÓÙ·È ÙȘ Ó˘¯ÙÂÚÈÓ¤˜ ÒÚ˜ (65). ∫·Ù¿ Û˘Ó¤ÂÈ·, ÔÈ ·ÈÌÔÏË„›Â˜ ı· Ú¤ÂÈ Ó· Á›ÓÔÓÙ·È ¿ÓÙ· ÙËÓ ›‰È· ÒÚ· Ù˘ Ë̤ڷ˜. ∞fi Ù· ÚÔ·Ó·ÊÂÚı¤ÓÙ·, ηٷÓÔԇ̠fiÙÈ ·Ó ‰ÂÓ ÙËÚËıÔ‡Ó Î·È Û ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË ·˘ÛÙËÚ¿ ÚˆÙfiÎÔÏÏ· Ï‹„˘ Î·È ÂÂÍÂÚÁ·Û›·˜ ı· ¤¯Ô˘Ì ·ÔÙÂϤÛÌ·Ù· Ô˘ ı· ˘ÂÚÂÎÙÈÌÒÓÙ·È ‹ ı· ˘ÔÙÈÌÒÓÙ·È, ÁÂÁÔÓfi˜ ÛÙÔ ÔÔ›Ô Èı·ÓÒ˜ Ó· ÔÊ›ÏÂÙ·È Î·È Ë ·ÌÊÈÏÂÁfiÌÂÓË ÛÙ¿ÛË Ù˘ ÂÚ¢ÓËÙÈ΋˜ ÎÔÈÓfiÙËÙ·˜ ˆ˜ ÚÔ˜ ÙÔÓ ‰Â›ÎÙË ·˘Ùfi, Ì ¿ÏÏÔ˘˜ Ó· ÙÔÓ Î·Ù·‰Èο˙Ô˘Ó ˆ˜ ·ÓÂ·Ú΋ Î·È ¿ÏÏÔ˘˜ Ó· ˘ÔÁÚ·ÌÌ›˙Ô˘Ó ÔÏÔ¤Ó· Î·È ÂÚÈÛÛfiÙÂÚÔ ÙËÓ ·Í›· ÙÔ˘ (63,64). ∂Ó·ÏÏ·ÎÙÈÎfi˜ ‰Â›ÎÙ˘ ·ÔÙÂÏ› Ë ECP ÙˆÓ Ù˘¤ÏˆÓ, Ë Û˘ÁΤÓÙÚˆÛË Ù˘ ÔÔ›·˜ Ê·›ÓÂÙ·È Ó· ·ÓÙ·Ó·ÎÏ¿ ÈÔ ÈÛÙ¿ ÙËÓ ¤ÎÙ·ÛË Ù˘ ÙÔÈ΋˜

Pediatr May-June 06

16-05-06

17:21

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175

ªË ÂÂÌ‚·ÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿

ÊÏÂÁÌÔÓÒ‰Ô˘˜ ‰ÈÂÚÁ·Û›·˜ ÛÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜, ·ÏÏ¿ ÁÈ’ ·˘Ùfi ¤ÁÈÓ·Ó Û¯fiÏÈ· ÚˆÙ‡ÙÂÚ· ÛÙË ÌÂϤÙË ·˘Ù‹. ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ EPX/EDN, Ô ˘ÔÏÔÁÈÛÌfi˜ Ù˘ Á›ÓÂÙ·È ÛÙ· Ô‡Ú· (59,66,67) Î·È Ê·›ÓÂÙ·È Ó· Â›Ó·È ·˘ÍË̤ÓË ÛÙËÓ ·ÙÔÈ΋ ‰ÂÚÌ·Ù›Ùȉ· (68) Î·È ÛÙÔ ¿ÛıÌ·. ª¿ÏÈÛÙ·, ¤¯ÂÈ ‚ÚÂı› fiÙÈ Ù· Â›‰¿ Ù˘ ÌÂÈÒÓÔÓÙ·È Ì ÙËÓ ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë ıÂÚ·›· (66). √È Û˘ÁÎÂÓÙÚÒÛÂȘ Ù˘ ˘fiÎÂÈÓÙ·È Û ÌÂÙ·‚ÔϤ˜ Ì ÙÔÓ ÎÈÚο‰ÈÔ Ú˘ıÌfi, ÂÓÒ ÛËÌ·Û›· ¤¯ÂÈ Î·È Ô Î·ıÔÚÈÛÌfi˜ ÙÔ˘ ‚·ıÌÔ‡ ‰È¿Ï˘Û˘ ÙˆÓ Ô‡ÚˆÓ Û ÓÂÚfi (63). ∏ ·ÏfiÙËÙ· Î·È Â˘ÎÔÏ›· ÛÙË Û˘ÏÏÔÁ‹ ÙÔ˘ ·›Ì·ÙÔ˜ Î·È ÙˆÓ Ô‡ÚˆÓ Ù· ηıÈÛÙÔ‡Ó ¯Ú‹ÛÈÌÔ˘˜ ‰Â›ÎÙ˜ ÁÈ· ÙË ÚÔ˘Ù›Ó· Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜. ŸÌˆ˜, Ë ÂȉÈÎfiÙËÙ· Î·È Ë Â˘·ÈÛıËÛ›· Ù˘ ÌÂıfi‰Ô˘ ‰ÂÓ Â›Ó·È Û˘ÁÎÂÎÚÈ̤ÓË, ηıÒ˜ Ú¤ÂÈ ÚÒÙ· Ó· Ï˘ıÔ‡Ó Ù· Ù¯ÓÈο Î·È ÌÂıÔ‰ÔÏÔÁÈο ÚÔ·Ó·ÊÂÚı¤ÓÙ· ÚÔ‚Ï‹Ì·Ù·. ∂ÈÚÔÛı¤Ùˆ˜, Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ‰ÂÓ ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó Â·ÚÎÒ˜ ÙË ÊÏÂÁÌÔÓÒ‰Ë ‰ÈÂÚÁ·Û›· ÛÙÔÓ ·Ó·Ó¢ÛÙÈÎfi ‚ÏÂÓÓÔÁfiÓÔ (69).

™˘Ì¤Ú·ÛÌ· ∏ ·Ó¿ÁÎË Î·Ù·ÓfiËÛ˘ Î·È ·Ú·ÎÔÏÔ‡ıËÛ˘ Ù˘ ·ÛıÌ·ÙÈ΋˜ ÊÏÂÁÌÔÓ‹˜ Â›Ó·È ·‰È·ÌÊÈÛ‚‹ÙËÙÔ ÁÂÁÔÓfi˜. √È ÌÂÛÔÏ·‚ËÙ¤˜ Ô˘ ÂϤÁ¯ÔÓÙ·È ÛÙ· ÚÔÎÏËÙ¿ Ù‡ÂÏ·, ÛÙÔ ·›Ì·, ÛÙ· Ô‡Ú· Î·È ÛÙ· ÂÎÓÂfiÌÂÓ· ·¤ÚÈ· - ·¢ı›·˜ ‹ ÌÂÙ¿ ·fi Û˘Ì‡ÎÓˆÛ‹ ÙÔ˘˜ - Ê·›ÓÂÙ·È Ó· ˘fiÛ¯ÔÓÙ·È ÔÏÏ¿, ·ÏÏ¿ ¯ÚÂÈ¿˙ÔÓÙ·È ÂÚ·ÈÙ¤Úˆ ÌÂϤÙË Î·È ‰ÔÎÈÌ‹ Û ÌÂÁ¿Ï˜ ÔÌ¿‰Â˜ ·ÙfïÓ. ¶Ú¤ÂÈ Â›Û˘ Ó· ÂÏÂÁı› Ë ÌÂÙ·‚ÔÏ‹ ÙÔ˘˜ Î·È ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË Î·È ¿ÏÏˆÓ ıÂÚ·¢ÙÈÎÒÓ Û¯ËÌ¿ÙˆÓ, ¤Ú·Ó ÙˆÓ ÂÈÛÓÂfiÌÂÓˆÓ ÎÔÚÙÈÎÔÂȉÒÓ, fiˆ˜ Â›Ó·È Ù· ·ÓÙÈÏ¢ÎÔÙÚÈÂÓÈο Î·È ÔÈ ‚2-‰ÈÂÁ¤ÚÙ˜ Ì·ÎÚ¿˜ ‰Ú¿Û˘, ηıÒ˜ Ë ¯Ú‹ÛË ÙÔ˘˜ Á›ÓÂÙ·È ÔÏÔ¤Ó· ÈÔ Û˘¯Ó‹ Î·È ·Ó·Áη›·. £· ‹Ù·Ó ÛÊ¿ÏÌ· Ó· ÂÚÈ̤ӷÌ ·fi ¤Ó·Ó ‰Â›ÎÙË Ó· Ì·˜ χÛÂÈ fiÏ· Ù· ÎÏÈÓÈο ÚÔ‚Ï‹Ì·Ù·. ∂Ú¢ÓËÙÈÎfi˜ ÛÎÔfi˜ ı· Ú¤ÂÈ Ó· Â›Ó·È Ë ·ÔÛ·Ê‹ÓÈÛË Ù˘ Û¯¤Û˘ ÙˆÓ ÙÈÌÒÓ ÙˆÓ ÌÂÛÔÏ·‚ËÙÒÓ Ù˘ ÊÏÂÁÌÔÓ‹˜ Ì ÙȘ ÌÂÙ·‚ÔϤ˜ ÛÙÔ Â›Â‰Ô ÙˆÓ ‚ÚfiÁ¯ˆÓ, ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ·, ÙË ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË ·ÏÏ¿ Î·È Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù˘ ÌÂٷ͇ ÙÔ˘˜ Û¯¤Û˘, ÚÔÎÂÈ̤ÓÔ˘ Ó· ηıÔÚÈÛÙ› Ë Â˘·ÈÛıËÛ›· Î·È Ë ÂȉÈÎfiÙËÙ· ÙÔ˘ ηıÂÓfi˜, ÁÈ· Ó· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ˆ˜ ‰È·ÁÓˆÛÙÈÎÔ› ·ÏÏ¿ Î·È ˆ˜ ÚÔÁÓˆÛÙÈÎÔ› ‰Â›ÎÙ˜.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Newacheck PW, Halfon N. Prevalence, impact and trends in childhood disability due to asthma. Arch Pediatr Adolesc Med 2000;154:287-293.

2. Woolley KL, Gibson PG, Carty K., Wilson AJ, Twaddell SH, Woolley MJ. Eosinophil apoptosis and the resolution of airway inflammation in asthma.

Am J Respir Crit Care Med 1996;154:237-243.

3. Fahy JV, Liu J, Wong H, Boushey HA. Analysis of cellular and biochemical constituents of induced sputum after allergen challenge: a method for studying allergic airway inflammation. J Allergy Clin Immunol 1994;93:1031-1039.

4. Pin I, Freitag AP, O’Byrne PM, Girgis-Gabardo A, Watson RM, Dolovich J et al. Changes in the cellular profile of induced sputum after allergen-induced asthmatic responses Am Rev Respir Dis 1992;145: 1265-1269.

5. Gibson PG, Henry RL, Thomas P. Noninvasive assessment of airway inflammation in children: induced sputum, exhaled nitric oxide, and breath condensate. Eur Respir J 2000;16:1008-1015.

6. Jones P, Hankin R, Simpson J, Gibson P, Henry R. The tolerability, safety, and success of sputum induction and combined hypertonic saline challenge in children. Am J Respir Crit Care Med 2001;164: 1146-1149.

7. Anderson SD. Exercise-induced asthma and the use of hypertonic saline aerosol as a bronchial challenge.

Respirology 1996;1:175-181.

8. Wilson NM, Bridge P, Spanevello A, Silverman M. Induced sputum in children: feasibility, repeatability, and relation of findings to asthma severity. Thorax 2000;55:786-774.

9. Gibson PG, Woolley KL, Carty K, Murree-Allen K, Saltos N. Safety of combined hypertonic saline challenge to induce sputum and to measure airway responsiveness. Am J Respir Crit Care Med 1995;151: A401.

10. Pin I, Gibson PG, Kolendowicz R, Girgis-Gabardo A, Denburg JA, Hargreave FE et al. Use of induced sputum cell counts to investigate airway inflammation in asthma. Thorax 1992;47:25-29.

11. Gibson PG, Girgis-Gabardo A, Morris MM, Mattoli S, Kay JM, Dolovich J et al. Cellular characteristics of sputum from patients with asthma and chronic bronchitis. Thorax 1989;44:693-699.

12. Fahy JV, Liu J, Wong H, Boushey HA. Cellular and biochemical analysis of induced sputum from asthmatic and from healthy subjects. Am Rev Respir Dis 1993;147:1126-1131.

13. Veen JC, Smits HH, Ravensberg AJ, Hiemstra PS, Sterk PJ, Bel EH. Impaired perception of dyspnea in patients with severe asthma: Relation to sputum eosinophils. Am J Respir Crit Care Med 1998;158: 1134-1141.

14. Spanevello A, Beghe B, Bianchi A, Migliori GB, Ambrosetti M, Neri M et al. Comparison of two methods of processing induced sputum: selected versus entire sputum. Am J Respir Crit Care Med 1998;157:665-668. ¶·È‰È·ÙÚÈ΋ 2006;69:169-177

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·176

176

Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

15. Nightingale JA, Rogers DF, Barnes PJ. Effect of inhaled ozone on exhaled nitric oxide, pulmonary function, and induced sputum in normal and asthmatic subjects. Thorax 1999;54:1061-1069.

16. Timmins N, Simpson JL, Fakes K, Gibson PG. The effects of salivary contamination on sputum cell counts: establishing quality criteria. Respirology 2000;5:A35.

17. Jatakanon A, Lim S, Barnes P. Changes in sputum eosinophils predict loss of asthma control. Am J Respir Crit Care Med 2000;161:64-72.

18. Covar RA, Spahn JD, Martin RJ, Silkoff PE, Sundstrom DA, Murphy J, et al. Safety and application of induced sputum analysis in childhood asthma.

J Allergy Clin Immunol. 2004;114(3):575-582.

19. Anderson SD. Exercise-induced asthma in children: a marker of airway inflammation. Med J Aust 2002;117 (Suppl 16):S61-S63.

20. Fahy JV, Steiger DJ, Liu J, Basbaum CB, Finkbeiner WE, Boushey H. Markers of mucus secretion and DNA levels in induced sputum from asthmatic and from healthy subjects. Am Rev Respir Dis 1993;147:1132-1137.

21. Rytila P, Pelkonen AS, Metso T, Nikander K, Haahtela T, Turpeinen M. Induced sputum in children with newly diagnosed mild asthma: the effect of 6 months of treatement with budesonide or disodium cromoglycate. Allergy 2004;59(8):839-844.

22. Bacci E, Cianchetti S, Ruocco L, Bartoli ML, Carnevali S, Dente FL et al. Comparison between eosinophilic markers in induced sputum and blood in asthmatic patients. Clin Exp Allergy 1998;28: 1237-1243.

23. Gibson PG, Simpson JL, Hankin R, Powell H, Henry RL. Relationship between induced sputum eosinophils and the clinical pattern of childhood asthma. Thorax 2003;58:116-121.

24. Sorva R, Metso T, Turpeinen M, JuntunenBackman K, Bjorksten F, Haahtela T. Eosinophil cationic protein in induced sputum as a marker of inflammation in asthmatic children. Pediatr Allergy Immunol 1997;8:45-50.

25. Venge P. Monitoring the allergic inflammation.

Allergy 2004;59:26-32.

26. Grootendorst DC, Sont JK, Willems LN, KluinNelemans JC, Van Krieken JH, Veselic-Charvat M et al. Comparison of inflammatory cell counts in asthma: induced sputum versus bronchoalveolar lavage and bronchial biopsies. Clin Exp Allergy 1997;27:769-779.

27. Pizzichini E, Pizzichini MM, Kidney JC, Efthimiadis A, Hussack P, Popov T et al. Induced sputum, bronchoalveolar lavage and blood from mild asthmatics: inflammatory cells, lymphocyte subsets and soluble markers compared. Eur Respir J 1998; 11:828-834.

28. Cianchetti S, Bacci E, Ruocco L, Bartoli ML, Carnevali S, Dente FL et al. Salbutamol pretreatment does not change eosinophil percentage and eosinophilic cationic protein concentration in hypertonic saline-induced sputum in asthmatic subjects. Clin Exp Allergy 1999;29:712-718. ¶·È‰È·ÙÚÈ΋ 2006;69:169-177

29. Berlyne GS, Parameswaran K, Kamada D, Efthimiadis A, Hargreave FE. A comparison of exhaled nitric oxide and induced sputum as markers of airway inflammation. J Allergy Clin Immunol 2000;106:638-644.

30. Piacentini GL, Bodini A, Peroni DG, Miraglia Del Giudice M Jr, Costella S, Boner AL. Reduction in exhaled nitric oxide immediately after methacholine challenge in asthmatic children. Thorax 2002;57: 771-773.

31. Kharitonov SA, Yates D, Robbins RA, LoganSinclair R, Shinebourne EA, Barnes PJ. Increased nitric oxide in exhaled air of asthmatic patients.

Lancet 1994;343:133-135.

32. Kharitonov SA. Exhaled markers of inflammatory lung diseases: ready for routine monitoring? Swiss Med Wkly 2004;134:175-192.

33. Strunk RC, Szefler SJ, Phillips BR, Zeiger RS, Chinchilli VM, Larsen G et al; Childhood Asthma Research and Education Network of the National Heart, Lung, and Blood Institute. Relationship of exhaled nitric oxide to clinical and inflammatory markers of persistent asthma in children. J Allergy Clin Immunol 2003;112:883-892.

34. American Thoracic Society. Recommendation for standardized procedures for the online and offline measurement of exhaled lower respiratory nitric oxide and nasal nitric oxide in adults and in children - 1999. Am J Respir Crit Care Med 1999; 160:2104-2117.

35. Kharitonov S, Alving K, Barnes PJ. The European Respiratory Society Task Force. Exhaled and nasal nitric oxide measurements: recommendations. Eur Respir J 1997;10:1683-1693.

36. Artlich A, Busch T, Lewandowski K, Schaible T, Falke KJ, Gortner L. Exhaled nitric oxide in preterm infants. Respir Physiol 1998;114:195-200.

37. Artlich A, Hagenah JU, Jonas S, Ahrens P, Gortner L. Exhaled nitric oxide in childhood asthma. Eur J Pediatr 1996;155:698-701.

38. Kharitonov SA, Yates D, Barnes PJ. Increased nitric oxide in exhaled air of normal subjects with upper respiratory tract infections. Eur Respir J 1995;8: 295-297.

39. Narang I, Ersu R, Wilson NM, Bush A. Nitric oxide in chronic airway inflammation in children: diagnostic use and pathophysiological significance.

Thorax 2002;57:586-589.

40. Warke TJ, Fitch PS, Brown V, Taylor R, Lyons JD, Ennis M et al. Exhaled nitric oxide correlates with airway eosinophils in childhood asthma. Thorax 2002;57:383-387.

41. Leuppi JD, Downs SH, Downie SR, Marks GB, Salome CM. Exhaled nitric oxide levels in atopic children: relation to specific allergic sensitisation, AHR, and respiratory symptoms. Thorax 2002;57: 518-523.

42. Franklin PJ, Taplin R, Stick SM. A community study of exhaled nitric oxide in healthy children. Am J Respir Crit Care Med 1999;159:69-73.

43. Frank TL, Adisesh A, Pickering AC, Morrison JFJ, Wright T, Francis H et al. Relationship between

Pediatr May-June 06

16-05-06

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™ÂÏ›‰·177

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ªË ÂÂÌ‚·ÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿

44.

45.

46.

47.

48.

49.

50.

51.

52.

53.

54.

55.

56.

exhaled nitric oxide and childhood asthma. Am J Respir Crit Care Med 1998;158:1032-1036.

Jones SL, Kittelson J, Cowan JO, Flannery EM, Hancox RJ, McLachlan CR et al. The predictive value of exhaled nitric oxide measurements in assessing changes in asthma control. Am J Respir Crit Care Med 2001;164:738-743.

Dwyer TM. Expired breath condensate (EBC) and the ultimate disposition of airway surface liquid (ASL). Am J Respir Crit Care Med 2001;163:A406.

Effros RM, Hoagland KW, Bosbous M, Castillo D, Foss B, Dunning M et al. Dilution of respiratory fluids by exhaled water vapour. Am J Respir Crit Care Med 2002;165:A342.

Hunt J. Exhaled breath condensate: an evolving tool for non-invasive evaluation of lung disease. J Allergy Clin Immunol 2002;110:28-34.

Kharitonov SA, Barnes PJ. Exhaled markers of pulmonary disease [State of the Art Review]. Am J Respir Crit Care Med 2001;163:1692-1722.

Khorovskaya LA, Sazonetz OI, Emanuel VL. Noninvasive laboratory investigation of lung secretion in bronchial asthma. Eur Respir J 2002;20 (Suppl 38):S90.

Horvath I, Donnelly LE, Kiss A, Kharitonov SA, Lim S, Chung KF et al. Combined use of exhaled hydrogen peroxide and nitric oxide in monitoring asthma. Am J Respir Crit Care Med 1998;158:10421046.

Becher G, Winsel K, Beck E, Neubauer G, Stresemann E. Breath condensate as noninvasive assessment of inflammation mediators from the lower airways.

Pneumonologie 1997;51 (Suppl 2):456-459.

Shahid SK, Kharitonov SA, Wilson NM, Bush A, Barnes PJ. Increased interleukin-4 and decreased interferon-Á in exhaled breath condensate of children with asthma. Am J Respir Crit Care Med 2002;165: 1290-1293.

Csoma Z, Kharitonov SA, Balint B, Bush A, Wilson NM, Barnes PJ. Increased leukotrienes in exhaled breath condensate in childhood asthma. Am J Respir Crit Care Med 2002;166:1345-1349.

Rosias PP, Dompeling E, Hendriks HJ, Heijnens JW, Donckerwolcke RA, Jobsis Q. Exhaled breath condensate in children: Pearls and pitfalls. Pediatr Allergy Immunol 2004;15:4-19.

Straub DA, Ehmann R, Hall GL, Mœller A, Hamacher J, Frey U, et al. Correlation of nitrites in breath condensates and lung function in asthmatic children. Pediatr Allergy Immunol 2004;15:20-25.

Hitka P, Cerny M, Vizek M, Wilhelm J, Zoban P. Assessment of exhaled gases in ventilated preterm infants. Physiol Res 2004;53:561-564.

57. Baraldi E, Ghiro L, Piovan V, Carraro S, Zacchello F, Zanconato S. Safety and success of exhaled breath condensate collection in asthma. Arch Dis Child 2003;88:358-360.

58. Rosias PP, Dompeling E, Dentener MA, Pennings HJ, Hendriks HJ, Van Iersel MP, et al. Childhood asthma: exhaled markers of airway inflammation, asthma control score, and lung function tests.

Pediatr Pulmonol 2004;38:107-114.

59. Wennergren G. Inflammatory mediators in blood and urine. Pediatr Respir Rev 2000;1:259-265.

60. Byström J, Garcia RC, Hakansson L, Karawajczyk M, Moberg L, Soukka J et al. Eosinophil cationic protein is stored in, but not produced by, peripheral blood neutrophils. Clin Exp Allergy 2002;32:1082-1091.

61. Metso T, Venge P, Haahtela T, Peterson CG, Seveus L. Cell specific markers for eosinophils and neutrophils in sputum and bronchoalveolar lavage fluid of patients with respiratory conditions and healthy subjects. Thorax 2002;57:449-451.

62. Venge P, Byström J, Carlson M, Hakansson L, Karawacjzyk M, Peterson C et al. Eosinophil cationic protein (ECP): molecular and biological properties and the use of ECP as a marker of eosinophil activation in disease. Clin Exp Allergy 1999;29:11721186.

63. Venge P. Monitoring the allergic inflammation.

Allergy 2004;59:26-32.

64. Björk A, Venge P, Peterson CG. Measurements of ECP in serum and the impact of plasma coagulation.

Allergy 2000;55:442-448.

65. Wolthers OD, Heuck C. Circadian variations in serum eosinophil cationic protein, and serum and urine eosinophil protein X. Pediatr Allergy Immunol 2003;14:130-133.

66. Kristjansson S, Strannegard IL, Strannegard Q, Peterson C, Enander I, Wennergren G. Urinary eosinophil protein X in children with atopic asthma: a useful marker of antiinflammatory treatment. J Allergy Clin Immunol 1996;97:1179-1187.

67. Labbe A, Aublet-Cuvelier B, Jouaville L, Beaugeon G, Fiani L, Petit I et al. Prospective longitudinal study of urinary eosinophil protein X in children with asthma and chronic cough. Pediatr Pulmonol 2001;31:354-362.

68. Breuer K, Kapp A, Werfel T. Urine eosinophil protein X (EPX) is an in vitro parameter of inflammation in atopic dermatitis of the adult age. Allergy 2001;56: 780-784.

69. Wilson NM, James A, Uasuf C, Payne DN, Hablas H, Agrafioti C et al. Asthma severity and inflammation markers in children. Pediatr Allergy Immunol 2001; 12:125-132. o

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∂Ó‰ÔÎÚ¿ÓÈ· ÓÂÔÏ¿ÛÌ·Ù· Û ·È‰È¿ Ì Ó¢ÚÈӈ̿وÛË Ù‡Ô˘ 1 1 ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£, °¶¡£ πÔÎÚ¿ÙÂÈÔ 2 ¶·È‰ÔÔÁÎÔÏÔÁÈÎfi ∆Ì‹Ì·, °¶¡£ πÔÎÚ¿ÙÂÈÔ AÏÏËÏÔÁÚ·Ê›·: ¢ËÌ‹ÙÚÈÔ˜ π. ∑·ÊÂÈÚ›Ô˘ ∂›ÎÔ˘ÚÔ˜ ∫·ıËÁËÙ‹˜ ¶·È‰È·ÙÚÈ΋˜ ¡Â˘ÚÔÏÔÁ›·˜∞Ó·Ù˘ÍÈÔÏÔÁ›·˜ ∞¶£ ∂ÁÓ·Ù›·˜ 106, ∆.∫. 546 22 £ÂÛÛ·ÏÔÓ›ÎË E-mail: jeff@med.auth.gr ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 28-09-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 31-03-2006

¢.π. ∑·ÊÂÈÚ›Ô˘1, ∞. µ·Ú‰·ÚÈÓfi˜1, ¢. ∫ÔÏÈÔ‡Ûη˜2

¶ÂÚ›ÏË„Ë: √È ·ÛıÂÓ›˜ Ì Ó¢ÚÈӈ̿وÛË Ù‡Ô˘ 1 ·ÔÙÂÏÔ‡Ó Ì›· ÔÌ¿‰· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Î·ÏÔ‹ıˆÓ Î·È Î·ÎÔ‹ıˆÓ fiÁÎˆÓ Û fiÏÔ ÙÔ ÛÒÌ· Ì ÙÔ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· Ó· ·ÔÙÂÏ› Ì›· ÂÓÙfiÈÛË Ì ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· Î·È È‰È·›ÙÂÚË ÛËÌ·Û›· ÁÈ· ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ· Î·È ÙËÓ ÚfiÁÓˆÛË ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ. ∆· ·È‰È¿, ÂȉÈο ÛÙȘ ËÏÈ˘ οو ÙˆÓ ‰¤Î· ÂÙÒÓ, Â›Ó·È ÈÔ ÂÈÚÚÂ‹ ÛÙËÓ ·Ó¿Ù˘ÍË ÂÓ‰ÔÎÚ¿ÓÈˆÓ fiÁΈÓ, Ë ÏÂÈÔ„ËÊ›· ÙˆÓ ÔÔ›ˆÓ Â›Ó·È ÈÏÔ΢ÙÙ·ÚÈο ·ÛÙÚÔ΢ÙÙÒÌ·Ù· Ô˘ ÂÓÙÔ›˙ÔÓÙ·È Î·Ù¿ Ì‹ÎÔ˜ Ù˘ ÔÙÈ΋˜ Ô‰Ô‡, Ì ÙÔ˘˜ fiÁÎÔ˘˜ Ù˘ ÂÚÈÔ¯‹˜ ÙÔ˘ ÛÙÂϤ¯Ô˘˜ Ó· ·ÎÔÏÔ˘ıÔ‡Ó ÛÂ Û˘¯ÓfiÙËÙ·. ¶ÚfiÎÂÈÙ·È ÁÈ· ÓÂÔϷۛ˜ Ì ۯÂÙÈο ηÏÔ‹ıË ÔÚ›·, ÔÈ Ôԛ˜ ··ÈÙÔ‡Ó ÚÔÛÂÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË Î·È Û˘ÓÙËÚËÙÈ΋ ıÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË, ÂÓÒ Ë ÌÂϤÙË Ù˘ ·ıÔÊ˘ÛÈÔÏÔÁ›·˜ Î·È Ë Î·Ù·ÓfiËÛË Ù˘ ‚ÈÔÏÔÁÈ΋˜ Û˘ÌÂÚÈÊÔÚ¿˜ ÙÔ˘˜ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û Ӥ˜ ηÙ¢ı‡ÓÛÂȘ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÓÂÔÏ·ÛÈÒÓ ÁÂÓÈÎfiÙÂÚ·. §¤ÍÂȘ ÎÏÂȉȿ: ¡Â˘ÚÈӈ̿وÛË Ù‡Ô˘ π, NF1, ÔÙÈο ÁÏÔÈÒÌ·Ù·, ·Ì·ÚÙÒÌ·Ù·, ÁÏÔÈÒÌ·Ù· ÛÙÂϤ¯Ô˘˜, ÂÓ‰ÔÎÚ¿ÓÈ· ÓÂÔÏ¿ÛÌ·Ù·.

CNS neoplasms in children with type 1 neurofibromatosis 1 1st Department of Pediatrics, Aristotle University, “Ippokrateion” General University Hospital, Thessaloniki 2 Department of Paediatric Oncology, “Ippokrateion” General University Hospital, Thessaloniki

Correspondence: Dimitrios π. Zafeiriou Assistant Professor of Paediatric Neurology and Developmental Pediatrics Aristotle University of Thessaloniki 106, Egnatia Str., 546 22 Thessaloniki E-mail: jeff@med.auth.gr Date of submission: 28-09-2004 Date of approval: 31-03-2006

D.π. Zafeiriou1, ∞. Vardarinos1, D. Koliouskas2

Abstract: Patients with type 1 neurofibromatosis are a high-risk group for the development of benign and malignant tumours in various parts of the body. The central nervous system (CNS) is a frequent location for such tumours, with serious implications for the clinical presentation and prognosis of these patients. Children under the age of ten are more susceptible to the development of CNS neoplasms, the majority of which are pilocytic astrocytomas located along the optic pathway and in the brainstem.

Usually they have an indolent course and can be managed with a conservative therapeutic approach, but they need close monitoring. Understanding of the natural history and pathophysiology of these tumours could lead to new forms of therapy for the treatment of malignancies in general.

Key words: Type 1 neurofibromatosis, NF1, optical glioma, hamartoma, brainstem glioma, CNS neoplasm.

∂ÈÛ·ÁˆÁ‹ ∏ Ó¢ÚÈӈ̿وÛË Ù‡Ô˘ 1 (NF1) Â›Ó·È Ì›· ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ‰È·Ù·Ú·¯‹ ÌÂ Û˘¯ÓfiÙËÙ· ÂÚ›Ô˘ 1:3000, Ù˘ ÔÔ›·˜ ÙÔ Î‡ÚÈÔ ÌÔÚÊÔÏÔÁÈÎfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi Â›Ó·È Ë ·ÚÔ˘Û›· ηÊÂÁ·ÏÔÎÙfi¯ÚÔˆÓ ÎËÏ›‰ˆÓ ÛÙÔ ‰¤ÚÌ· ÙÔÓ ·ÛıÂÓÒÓ (1). ∏ ÓfiÛÔ˜ ÚÔηÏÂ›Ù·È ·fi ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ¯ÚˆÌfiۈ̷ 17q11.2, Û ¤Ó· ÔÁÎÔηٷÛÙ·ÏÙÈÎfi ÁÔÓ›‰ÈÔ ÙÔ ÔÔ›Ô Ú˘ıÌ›˙ÂÈ ÙËÓ ·‡ÍËÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ. ∫ÏËÚÔÓÔÌÂ›Ù·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú·, ·Ó Î·È ÛÙÔ 50% ÂÚ›Ô˘ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÚfiÎÂÈÙ·È ÁÈ· Ӥ˜ ÌÂÙ·ÏÏ¿ÍÂȘ. ∏ ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ Ù›ıÂÙ·È ÎÏÈ-

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ÓÈο Û‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· Ô˘ fiÚÈÛ ÙÔ ∞ÌÂÚÈηÓÈÎfi πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ (National Institute of Health Consensus Development Conference; NIHCDC), Ù· ÔÔ›· ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1. °È· Ó· ÙÂı› Ë ‰È¿ÁÓˆÛË ··ÈÙÔ‡ÓÙ·È ÙÔ˘Ï¿¯ÈÛÙÔÓ ‰‡Ô ·fi Ù· ¤ÍÈ ÎÚÈÙ‹ÚÈ·. ªÂ ‚¿ÛË Ù· ·Ú·¿Óˆ ÂÈÙ˘Á¯¿ÓÂÙ·È Ë ‰È¿ÁÓˆÛË ÛÙÔ 94% ÙˆÓ ·ÛıÂÓÒÓ Ì¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ ¤ÍÈ ÂÙÒÓ (2). √È ·ÏÏ·Á¤˜ ÛÙÔ ÁÔÓ›‰ÈÔ Ù˘ NF1 Î·È Ë Â›‰Ú·ÛË ·˘ÙÒÓ ÙˆÓ ·ÏÏ·ÁÒÓ ÛÙ· ·ÛÙÚÔ·ÙÙ·Ú· Î·È Ù· ·ÙÙ·Ú· ÙÔ˘ Schwann ÛÂ Û˘Ó‰˘·ÛÌfi Ì ¿ÏÏÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÔÁÎÔÁ¤ÓÂÛ˘, ¤¯Ô˘Ó ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÔÈ ·ÛıÂÓ›˜ Ó· ÂÌÊ·Ó›˙Ô˘Ó Ì›·

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¡Â˘ÚÈӈ̿وÛË - ÂÓ‰ÔÎÚ¿ÓÈ· ÓÂÔÏ¿ÛÌ·Ù·

¶›Ó·Î·˜ 1. ∫ÚÈÙ‹ÚÈ· ‰È¿ÁÓˆÛ˘ Ó¢ÚÈӈ̿وÛ˘ Ù‡Ô˘ 1 1. ⁄·ÚÍË ¤ÍÈ ‹ ÂÚÈÛÛÔÙ¤ÚˆÓ Î·ÊÂÁ·ÏÔÎÙfi¯ÚÔˆÓ ÎËÏ›‰ˆÓ, ‰È·Ì¤ÙÚÔ˘ ÙÔ˘Ï¿¯ÈÛÙÔÓ 5 mm ÚÔÂÊË‚Èο ‹ 15 mm ÌÂÙ¿ ÙËÓ ÂÊ˂›·, 2. ⁄·ÚÍË ÙÔ˘Ï¿¯ÈÛÙÔÓ ‰‡Ô Ó¢ÚÈÓˆÌ¿ÙˆÓ ÔÔÈÔ˘‰‹ÔÙ هÔ˘ ‹ ÂÓfi˜ ÏÂÁÌ·ÙfiÌÔÚÊÔ˘ Ó¢ÚÈÓÒÌ·ÙÔ˜, 3. º·Î›‰ˆÛË Ù˘ Ì·Û¯·ÏÈ·›·˜ ‹ Ù˘ ‚Ô˘‚ˆÓÈ΋˜ ¯ÒÚ·˜, 4. ∆Ô˘Ï¿¯ÈÛÙÔÓ ‰‡Ô Ô˙›‰È· ÙÔ˘ Lisch (·Ì·ÚÙÒÌ·Ù· Ù˘ ›Úȉ·˜), 5. ª›· ‰È·ÎÚÈÙ‹ ÔÛÙÈ΋ ·ÓˆÌ·Ï›· (·Ï·Û›· Ù¤Ú˘Á·˜ ÙÔ˘ ÛÊËÓÔÂȉԇ˜ ÔÛÙÔ‡ ‹ ÎÓËÌÈ·›· „¢‰¿ÚıÚˆÛË), 6. ŒÓ· ÁÏÔ›ˆÌ· Ù˘ ÔÙÈ΋˜ Ô‰Ô‡ Î·È 7. ŒÓ·˜ Û˘ÁÁÂÓ‹˜ 1Ô˘ ‚·ıÌÔ‡ Ì ‰È·ÁÓˆṲ̂ÓË NF1, Û‡Ìʈӷ Ì ٷ ·Ú·¿Óˆ.

ÚԉȿıÂÛË ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Î·ÏÔ‹ıˆÓ Î·È Î·ÎÔ‹ıˆÓ fiÁÎˆÓ (3), Ì ÙÔ˘˜ ÌÈÎÚÔ‡˜ ·ÛıÂÓ›˜ ËÏÈΛ·˜ οو ÙˆÓ ‰¤Î· ÂÙÒÓ Ó· ÎÈÓ‰˘ÓÂ‡Ô˘Ó ÂÚÈÛÛfiÙÂÚÔ. ™ÙËÓ ÂÚ›ÙˆÛË ÙˆÓ ÂÓ‰ÔÎÚ·Ó›ˆÓ ÓÂÔÏ·ÛÌ¿ÙˆÓ, ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÚfiÎÂÈÙ·È ÁÈ· ηÏÔ‹ıË ÈÏÔ΢ÙÙ·ÚÈο ·ÛÙÚÔ΢ÙÙÒÌ·Ù· Ô˘ ÂÓ‰¤¯ÂÙ·È Ó· ÂÓÙÔ›˙ÔÓÙ·È ÔÔ˘‰‹ÔÙ ÛÙÔÓ ÂÁΤʷÏÔ, ·Ó Î·È Ù· ÔÙÈο Ó‡ڷ, ÙÔ ÔÙÈÎfi ¯›·ÛÌ· Î·È ÙÔ ÌÂÛÔÏfi‚ÈÔ Â›Ó·È ÔÈ ı¤ÛÂȘ fiÔ˘ ··ÓÙÒÓÙ·È Û˘¯ÓfiÙÂÚ· (4). ∏ Û¯ÂÙÈο ·Û˘Ó‹ı˘ ÂÍ·ÏÏ·Á‹ ÙÔ˘˜ Û ηÎÔËı¤ÛÙÂÚ˜ ÌÔÚʤ˜ ÔÊ›ÏÂÙ·È Û ·˘ÍË̤ÓË ¤ÎÊÚ·ÛË ˘Ô‰Ô¯¤ˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ÙËÓ ·ÚÔ˘Û›· ÌÔÚ›ˆÓ Ô˘ ÚÔ¿ÁÔ˘Ó ÙËÓ Â¤ÎÙ·ÛË ÙÔ˘ fiÁÎÔ˘, ηıÒ˜ Î·È ÙËÓ ·ÂÓÂÚÁÔÔ›ËÛË Ú˘ıÌÈÛÙÒÓ Ù˘ ΢ÙÙ·ÚÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (3). ∆· ÁÏÔÈÒÌ·Ù· ÛÙËÓ NF1 ÂÌÊ·Ó›˙Ô˘Ó ‰‡Ô ȉȷ›ÙÂÚ· ¯·Ú·ÎÙËÚÈÛÙÈο, ÙÔÓ ¯·ÌËÏfi Ú˘ıÌfi ·‡ÍËÛ˘ Î·È ÙËÓ Èı·ÓfiÙËÙ· Ù˘ ·˘ÙfiÌ·Ù˘ ˘ÔÛÙÚÔÊ‹˜, Ô˘ Ù· ‰È·ÎÚ›ÓÔ˘Ó ·fi ÁÏÔÈÒÌ·Ù· Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔÓ ˘fiÏÔÈÔ ÏËı˘ÛÌfi, ·Ó Î·È ÂÓ›ÔÙ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÈÔ ÂÈıÂÙÈÎfi ¯·Ú·ÎÙ‹Ú· Ì ‰Ú·Ì·ÙÈΤ˜ ÎÏÈÓÈΤ˜ ÂÈÙÒÛÂȘ (5-7). √È ‰‡Ô ·˘Ù¤˜ ȉȷÈÙÂÚfiÙËÙ˜ ¤¯Ô˘Ó ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ Î·Ï‡ÙÂÚË ÚfiÁÓˆÛË ÙˆÓ ·ÛıÂÓÒÓ, ·ÏÏ¿ Î·È ÙËÓ ‡·ÚÍË ÂÓ‰ÔÈ·ÛÌÒÓ fiÛÔÓ ·ÊÔÚ¿ ÛÙË ıÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË.

°ÏÔÈÒÌ·Ù· Ù˘ ÔÙÈ΋˜ Ô‰Ô‡ ¶ÚfiÎÂÈÙ·È ÁÈ· ÙÔÓ Û˘¯ÓfiÙÂÚÔ Ù‡Ô ÂÓ‰ÔÎÚ¿ÓÈ·˜ ηÎÔ‹ıÂÈ·˜ ÛÙËÓ NF1. ™ÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÚfiÎÂÈÙ·È ÁÈ· ¯·ÌËÏ‹˜ ηÎÔ‹ıÂÈ·˜ ÈÏÔ΢ÙÙ·ÚÈο ·ÛÙÚÔ΢ÙÙÒÌ·Ù·, Û‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· Ù˘ ¶·ÁÎfiÛÌÈ·˜ √ÚÁ¿ÓˆÛ˘ ÀÁ›·˜ (8). ªÔÚÔ‡Ó Ó· ·Ó·Ù˘¯ıÔ‡Ó Û ÔÔÈÔ‰‹ÔÙ ÛËÌÂ›Ô Ù˘ ÔÙÈ΋˜ Ô‰Ô‡, ·Ó Î·È Ù· ÔÙÈο Ó‡ڷ Î·È ÙÔ ÔÙÈÎfi ¯›·ÛÌ· Â›Ó·È ÔÈ ı¤ÛÂȘ Ì ÙË Û˘¯ÓfiÙÂÚË ÂÓÙfiÈÛË. ∏ ·ÌÊÔÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË ÛÙ· ÔÙÈο Ó‡ڷ Â›Ó·È ¤Ó· ·ÔÎÏÂÈÛÙÈÎfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi

Ù˘ NF1 (9), ÂÓÒ Ë ‰È¿ÎÚÈÛË ÙˆÓ fiÁÎˆÓ ÙÔ˘ ¯È¿ÛÌ·ÙÔ˜ ·fi ·˘ÙÔ‡˜ ÙÔ˘ ˘Ôı·Ï¿ÌÔ˘ Î·È Ù˘ ÙÚ›Ù˘ ÎÔÈÏ›·˜, Û˘¯Ó¿ ·ÚÔ˘ÛÈ¿˙ÂÈ ‰˘ÛÎÔϛ˜ Î·È ‰È·ÌÔÚÊÒÓÂÙ·È Ë Ù¿ÛË Ó· ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È ˆ˜ Ì›· ÔÓÙfiÙËÙ· fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ÎÏÈÓÈ΋ ÔÚ›·, ÙË ıÂÚ·›· Î·È ÙËÓ ÚfiÁÓˆÛË. ¶·ıÔÏÔÁÔ·Ó·ÙÔÌÈο, Ù· ÔÙÈο ÁÏÔÈÒÌ·Ù· ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ÂÓ·ÏÏ·Á¤˜ ÌÈÎÚÔ΢ÛÙÈÎÒÓ ÂÚÈÔ¯ÒÓ Ô˘ ÂÚȤ¯Ô˘Ó ÎÔÎÎÈÒ‰Ë ËˆÛÈÓfiÊÈÏ· ۈ̿ÙÈ·, ÌÂ Û˘Ì·Á›˜ ÂÚÈÔ¯¤˜ Ô˘ ·ÔÙÂÏÔ‡ÓÙ·È ·fi ÂÈÌË΢Ṳ̂ӷ ÈÓÒ‰Ë Î‡ÙÙ·Ú·, Û˘¯Ó¿ ˘¿Ú¯ÂÈ ÛËÌ·ÓÙÈ΋ ·ÈÌ·ÙÈ΋ ¿Ú‰Â˘ÛË Î·È ÓÂÔ·ÁÁ›ˆÛË, ÂÓÒ Â›Ó·È Û¿ÓÈ· Ë ·ÚÔ˘Û›· ÛËÌ›ˆÓ ηÎÔ‹ıÂÈ·˜. ÷ڷÎÙËÚÈÛÙÈÎfi˜ Â›Ó·È Î·È Ô ÙÚfiÔ˜ ·Ó¿Ù˘Í˘ ÙˆÓ fiÁÎˆÓ ·˘ÙÒÓ ÛÙȘ ‰È¿ÊÔÚ˜ ı¤ÛÂȘ. ∆· ÁÏÔÈÒÌ·Ù· Ô˘ ÚÔÛ‚¿ÏÏÔ˘Ó Ù· ÔÙÈο Ó‡ڷ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Ì›· ·‡ÍËÛË Î·Ù¿ Ì‹ÎÔ˜ ÙÔ˘ ¿ÍÔÓ· ÙÔ˘ Ó‡ÚÔ˘, Ô˘ Ô‰ËÁ› Û ηٷÛÙÚÔÊ‹ Ù˘ ·Ú¯ÈÙÂÎÙÔÓÈ΋˜ ÙÔ˘ Ó‡ÚÔ˘, ‹ Û˘¯ÓfiÙÂÚ·, ÂÌÊ·Ó›˙Ô˘Ó Ì›· ΢ÎÏÔÙÂÚ‹ Â¤ÎÙ·ÛË Ì ÚÔÛ‚ÔÏ‹ ÙˆÓ ÏÂÙÔÌËÓÈÁÁÈÎÒÓ ÛÙÔȯ›ˆÓ Ô˘ ÂÚÈ‚¿ÏÏÔ˘Ó ÙÔ Ó‡ÚÔ, ¯ˆÚ›˜ fï˜ ȉȷ›ÙÂÚË Î·Ù·ÛÙÚÔÊ‹ ÙÔ˘ ȉ›Ô˘ ÙÔ˘ ÔÙÈÎÔ‡ Ó‡ÚÔ˘. ∞ÓÙÈı¤Ùˆ˜, Ù· ÓÂÔÏ¿ÛÌ·Ù· Ù˘ ÂÚÈÔ¯‹˜ ÙÔ˘ ¯È¿ÛÌ·ÙÔ˜ ‰Â›¯ÓÔ˘Ó Ó· ÂÚÈÔÚ›˙ÔÓÙ·È ÏÈÁfiÙÂÚÔ ·fi Ù· ÂÚÈ‚¿ÏÏÔÓÙ· ÌËÓÈÁÁÈο ÛÙÔȯ›· Î·È ÂÌÊ·Ó›˙Ô˘Ó Ì›· ÛÊ·ÈÚÈÎfiÙÂÚË Â¤ÎÙ·ÛË Î·È ÂÚÈÛÛfiÙÂÚÔ ÔÈΛϷ Û¯‹Ì·Ù· (10-12). ∂› ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ÂÓ‰ÔÎÚ¿ÓÈˆÓ fiÁÎˆÓ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜, ÌfiÓÔ ¤Ó· 2%-5% ·ÊÔÚ¿ ÛÙËÓ ÔÙÈ΋ Ô‰fi Ì ÙËÓ NF1, fï˜, Ó· ÂÌϤÎÂÙ·È ÛÙÔ 70% ·˘ÙÒÓ ÙˆÓ ÂÚÈÙÒÛÂˆÓ (8,13). ŒÚ¢Ó˜ Ô˘ ¤ÁÈÓ·Ó Û ΤÓÙÚ· ·Ó·ÊÔÚ¿˜ Ù˘ ÓfiÛÔ˘, ·Ó‚¿˙Ô˘Ó ÙÔ ÔÛÔÛÙfi ·Ó¿Ù˘Í˘ ÙˆÓ √ƒ∆ Û 15% (14). ™Â ÌÂÙ·ÁÂÓ¤ÛÙÂÚ˜ ¤Ú¢Ó˜ Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó ·fi ÙÔ˘˜ Listernick et al. (15,16) Î·È ÛÙȘ Ôԛ˜ ·ÍÈÔÏÔÁ‹ıËÎ·Ó ÙfiÛÔ Û˘Ìو̷ÙÈÎÔ› fiÛÔ Î·È ·Û˘Ìو̷ÙÈÎÔ› ·ÛıÂÓ›˜, ÂȂ‚·›ˆıËÎ·Ó Ù· ·Ú·¿Óˆ ÔÛÔÛÙ¿, ÂÓÒ Û ¿ÏϘ ÌÂϤÙ˜ ·Ó·Ê¤ÚÂÙ·È ÂÈÔÏ·ÛÌfi˜ ÙˆÓ √ƒ∆ Ù˘ Ù¿Í˘ ÙÔ˘ 15-21% (17-19,21,28). ∆· ÔÙÈο ÁÏÔÈÒÌ·¶·È‰È·ÙÚÈ΋ 2006;69:178-186

Pediatr May-June 06

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¢.π. ∑·ÊÂÈÚ›Ô˘ Î·È Û˘Ó.

Ù· ÛÙËÓ NF1 Â›Ó·È ¤Ó· Ê·ÈÓfiÌÂÓÔ Ô˘ ÂÌÊ·Ó›˙ÂÙ·È Î·Ù¿ ·ÚÈÔ ÏfiÁÔ ÛÙËÓ ÚÒÙË ‰ÂηÂÙ›· Ù˘ ˙ˆ‹˜. ŒÚ¢Ó˜ ı¤ÙÔ˘Ó ÙË Ì¤ÛË ËÏÈΛ· ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË √ƒ∆ Ó· Î˘Ì·›ÓÂÙ·È ·fi Ù· 4,2 ¤ˆ˜ Ù· 5,2 ¯ÚfiÓÈ·, ÂÓÒ ·Ó·Ê¤ÚÔÓÙ·È Î·È Ó·ÚfiÙÂÚ˜ ËÏÈ˘ Û ΤÓÙÚ· Ô˘ οÓÔ˘Ó Û˘ÛÙËÌ·ÙÈ΋ ¯Ú‹ÛË Ù˘ MRI ·fi ÙË ÛÙÈÁÌ‹ Ù˘ ‰È¿ÁÓˆÛ˘ Ù˘ ÓfiÛÔ˘. ∏ ·Ó¿Ù˘ÍË Û˘Ìو̷ÙÈÎÒÓ √ƒ∆ ÌÂÙ¿ ÙËÓ ËÏÈΛ· ÙˆÓ ¤ÍÈ ÂÙÒÓ Â›Ó·È ¤Ó· Ì¿ÏÏÔÓ Û¿ÓÈÔ Ê·ÈÓfiÌÂÓÔ, ·Ó Î·È Ì›· ÚfiÛÊ·ÙË ÌÂϤÙË Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠ΤÓÙÚÔ ·Ó·ÊÔÚ¿˜ ÁÈ· ÙË ÓfiÛÔ ÂÈÛËÌ·›ÓÂÈ fiÙÈ 17 ·fi ÙÔ˘˜ 54 ·ÛıÂÓ›˜ Ô˘ ÌÂÏÂÙ‹ıËηÓ, ·Ó¤Ù˘Í·Ó ÔÙÈÎfi ÁÏÔ›ˆÌ· ÌÂÙ¿ ÙËÓ ËÏÈΛ· ÙˆÓ 6 ÂÙÒÓ Î·È ·ÚÎÂÙÔ› ·fi ·˘ÙÔ‡˜ ·ÚÔ˘Û›·Û·Ó Âȉ›ӈÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Î·È ÙÔ˘ fiÁÎÔ˘ ηٿ ÙÔ ‰È¿ÛÙËÌ· Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ (20). ∞Í›˙ÂÈ Ó· ÛËÌÂȈı› Ë ˘ÂÚÔ¯‹ ÙˆÓ ıËϤˆÓ ˆ˜ ÚÔ˜ ÙÔ˘˜ ¿ÚÚÂÓ˜ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ·˘ÙÒÓ ÙˆÓ fiÁΈÓ, Ì ̛· Û˘¯ÓfiÙËÙ· 2:1 (15,16,18,22). ∞Ó Î·È ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ù· √ƒ∆ ·ÔÙÂÏÔ‡Ó Ù˘¯·›· Â˘Ú‹Ì·Ù·, ÂÓ›ÔÙ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÛËÌ·ÓÙÈ΋ ‰ÈfiÁΈÛË Ì ·ÔÙ¤ÏÂÛÌ· Ó· ÚÔηÏÔ‡Ó ÈÂÛÙÈο Ê·ÈÓfiÌÂÓ· ÛÙȘ ÁÂÈÙÔÓÈΤ˜ ÂÚÈÔ¯¤˜. √È ·ÓˆÌ·Ï›Â˜ ÛÙËÓ fiÚ·ÛË Î·È Ù· ÛËÌ›· Ù˘ ˘Ôı·Ï·ÌÈ΋˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ ·ÔÙÂÏÔ‡Ó ÙȘ Û˘¯ÓfiÙÂÚ˜ ÂΉËÏÒÛÂȘ. ∆· ÔÊı·ÏÌÔÏÔÁÈο ÚÔ‚Ï‹Ì·Ù· ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙË Ì›ˆÛË Ù˘ ÔÙÈ΋˜ Ô͇ÙËÙ·˜, ÙËÓ ¤ÎÙˆÛË ÙˆÓ ÔÙÈÎÒÓ ‰›ˆÓ, ÙË ÌÂȈ̤ÓË ÈηÓfiÙËÙ· ‰È¿ÎÚÈÛ˘ ¯ÚˆÌ¿ÙˆÓ, ÙËÓ ÚfiÙˆÛË ÙÔ˘ ‚ÔÏ‚Ô‡ Î·È ÙÔÓ ÛÙÚ·‚ÈÛÌfi, ÂÓÒ Î·Ù¿ ÙË ‚˘ıÔÛÎfiËÛË Â›Ó·È ‰È·ÎÚÈÙ¤˜ ÔÈ ‚Ï¿‚˜ ÛÙËÓ ÔÙÈ΋ ıËÏ‹ (·ÙÚÔÊ›·, Ô›‰ËÌ·, ·ԯڈ̷ÙÈÛÌfi˜) Î·È ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÔÈÛı›Ô˘ fiÏÔ˘ (10,20,22). ∆· ÛËÌ›· Ù˘ ˘Ôı·Ï·ÌÈ΋˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ ··ÓÙÒÓÙ·È ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û fiÁÎÔ˘˜ ÙÔ˘ ¯È¿ÛÌ·ÙÔ˜ Î·È Û˘¯Ó¿ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Î·È Â¤ÎÙ·ÛË ÙÔ˘ fiÁÎÔ˘ ÛÙȘ ‰Ô̤˜ Ù˘ ÂÚÈÔ¯‹˜ ÙÔ˘ ˘Ôı·Ï¿ÌÔ˘. ∂ΉËÏÒÓÔÓÙ·È Ì ÙË ÌÔÚÊ‹ Ù˘ ÚfiˆÚ˘ ÂÊ˂›·˜ ηıÒ˜ Î·È ¿ÏÏˆÓ ÂÓ‰ÔÎÚÈÓÔ·ıÂÈÒÓ. ™ÙȘ ˘¿Ú¯Ô˘Û˜ ÌÂϤÙ˜, Ë Û˘¯ÓfiÙËÙ· Ù˘ Û˘Û¯¤ÙÈÛ˘ Ù˘ ÚÒÈÌ˘ ‹‚˘ Ì ÁÏÔÈÒÌ·Ù· Ù˘ ÔÙÈ΋˜ Ô‰Ô‡ Û ۯ¤ÛË Ì ÙÔ Û‡ÓÔÏÔ ÙÔ˘ ·ÛıÂÓÒÓ Ì NF1 Î˘Ì·›ÓÂÙ·È ·fi 26-39%. ∏ ÂÈÙ·¯˘ÓfiÌÂÓË ·‡ÍËÛË, ·ÏÏ¿ Î·È ÂÈ‚Ú¿‰˘ÓÛË Û ‡„Ô˜, ·ÔÙÂÏÔ‡Ó Û˘Ó‹ıˆ˜ ÙÔ ÚÒÙÔ Û‡Ìو̷ (10,23,25). ∆· √ƒ∆ Î·È È‰È·›ÙÂÚ· ·˘Ù¿ Ù˘ ÂÚÈÔ¯‹˜ ÙÔ˘ ¯È¿ÛÌ·ÙÔ˜ ÌÔÚÔ‡Ó Ó· ÂΉËψıÔ‡Ó Î·È Ì ÙË ÌÔÚÊ‹ Û˘ÛÙËÌ·ÙÈ΋˜ ÓfiÛÔ˘, ·ÊÔ‡ ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÓÙfiÈÛË Î·È ÙÔ Ì¤ÁÂıfi˜ ÙÔ˘˜ ÌÔÚÔ‡Ó Ó· ÚÔηϤÛÔ˘Ó ·Ó·Ù˘Íȷ΋ ηı˘ÛÙ¤ÚËÛË, ¶·È‰È·ÙÚÈ΋ 2006;69:178-186

‰È·Ù·Ú·¯¤˜ Û˘ÌÂÚÈÊÔÚ¿˜ ‹ ÛÙ·ÛÈÌfiÙËÙ· ‚¿ÚÔ˘˜. µÂ‚·›ˆ˜ ‰ÂÓ Ï›ÂÈ Î·È Ë Ù˘È΋ Û˘Ìو̷ÙÔÏÔÁ›· Ù˘ ·˘ÍË̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ˘¤ÚÙ·Û˘ Ì ÎÂÊ·Ï·ÏÁ›Â˜, Ó·˘Ù›· Î·È Â̤ÙÔ˘˜. Ÿ¯È Û¿ÓÈ· ÌÔÚ› Ó· ÂΉËψıÔ‡Ó Ì ‰È¿Ù·ÛË ÙÔ˘ ÎÔÈÏÈ·ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ‹ Î·È ˘‰ÚÔÎÂÊ·Ï›·, Ë ÔÔ›· ÌÔÚ› Ó· ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ˘Ú·ÌȉÈ΋ Û˘Ó‰ÚÔÌ‹ (Û˘Ó‹ıˆ˜ ËÌÈ¿ÚÂÛË), ·Ù·Í›· Î·È ÎÒÌ· (26). ∆· √ƒ∆ Û˘ÌÂÚÈʤÚÔÓÙ·È Ì ηÏÔËı¤ÛÙÂÚÔ ÙÚfiÔ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ¡F1 Û ۯ¤ÛË Ì ÛÔÚ·‰ÈΤ˜ ÂÚÈÙÒÛÂȘ ÔÙÈÎÒÓ ÁÏÔÈˆÌ¿ÙˆÓ (13,15,16,18,26,27). ™ÙÔ 76% ÙˆÓ √ƒ∆ Ë ·Ó·Î¿Ï˘„Ë ÙÔ˘ fiÁÎÔ˘ ·ÔÙÂÏÔ‡ÛÂ Ù˘¯·›Ô ‡ÚËÌ·. ªfiÓÔ ÙÔ 9% ÙˆÓ ·ÛıÂÓÒÓ ÂÌÊ¿ÓÈÛ·Ó ·‡ÍËÛË ÙÔ˘ fiÁÎÔ˘ ‹ ¤ÎÙˆÛË Ù˘ fiÚ·Û˘ Î·È ÂÚfiÎÂÈÙÔ ÁÈ· ·ÛıÂÓ›˜ Ì ÂÓÙfiÈÛË ÙÔ˘ fiÁÎÔ˘ ÛÙÔ ¯›·ÛÌ·, ÂÓÒ Î·Ó¤Ó·˜ ·fi ÙÔ˘˜ fiÁÎÔ˘˜ Ô˘ ÂÓÙÔ›˙ÔÓÙ·Ó ÛÙ· ÔÙÈο Ó‡ڷ ‰ÂÓ ·ÚÔ˘Û›·Û ·ÍÈÔÛËÌ›ˆÙË ÂͤÏÈÍË (10,15). ∞ÎfiÌË, ¤Ó· ȉȷ›ÙÂÚÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÙˆÓ √ƒ∆, Â›Ó·È Ë ÂÓ›ÔÙ ·Ú·ÙËÚÔ‡ÌÂÓË ·˘ÙfiÌ·ÙË ˘ÔÛÙÚÔÊ‹ ÙÔ˘˜, ÛÙÔÈ¯Â›Ô Ô˘ Û˘ÓËÁÔÚ› ˘¤Ú Ù˘ Ì¿ÏÏÔÓ Î·ÏÔ‹ıÔ˘˜ ʇÛ˘ ÙÔ˘˜. ∏ ·˘ÙfiÌ·ÙË ˘ÔÛÙÚÔÊ‹ Èı·ÓÒ˜ ÔÊ›ÏÂÙ·È Û ӤÎÚˆÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ fiÁÎÔ˘, ‹ Û ·fiÙˆÛË ÙˆÓ Î·ÚÎÈÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì ڢıÌfi ÌÂÁ·Ï‡ÙÂÚÔ ·fi ·˘Ùfi Ù˘ ·Ú·ÁˆÁ‹˜ ÙÔ˘˜ ‹ Û ÂÓ‰ÔÎÚÈÓÈÎÔ‡˜ ‹ ·ÓÔÛÔÏÔÁÈÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜ (5-7). ∞fi Ù· ·Ú·¿Óˆ ÚÔ·ÙÂÈ fiÙÈ Ù· √ƒ∆ ÛÙËÓ NF1 ·ÔÙÂÏÔ‡Ó Ì›· Û‡ÓıÂÙË ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ· Ì ÔχÏÔÎË Ê˘ÛÈ΋ ÂͤÏÈÍË, Ë ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÔÔ›ˆÓ ··ÈÙ› ÚÔÛÂÎÙÈÎfi ۯ‰ȷÛÌfi.

£ÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Î·È ·ÓÙÈÌÂÙÒÈÛË √ ¤ÏÂÁ¯Ô˜ Ì Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈΤ˜ ÌÂıfi‰Ô˘˜ Ô‰ËÁ› Û ÚˆÈÌfiÙÂÚË ‰È¿ÁÓˆÛË, Î·È Ë ÛÙÂÓ‹ ·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ ·ÛıÂÓÒÓ ·Ú¤¯ÂÈ ÏËÚÔÊÔڛ˜ ÁÈ· ÙËÓ ¤ÁηÈÚË Î·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·Ú¤Ì‚·ÛË ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ Ô fiÁÎÔ˜ ηٷÛÙ› Û˘Ìو̷ÙÈÎfi˜. ∫˘Ú›·Ú¯Ô ÚfiÏÔ ÛÙËÓ Ó¢ÚÔ·ÂÈÎfiÓÈÛË ¤¯ÂÈ Ë Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘ (MRI), Ë ÔÔ›· ·Ú¤¯ÂÈ ˘„ËÏ‹ ‰È·ÎÚÈÙÈ΋ ‰˘Ó·ÙfiÙËÙ· ÁÈ· ÙȘ ‰Ô̤˜ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ÂÓÒ Ë ‰˘Ó·ÙfiÙËÙ· Ù˘ ¯Ú‹Û˘ ÂÓ‰ÔÊϤ‚ÈÔ˘ ÛÎÈ·ÛÙÈÎÔ‡ (Á·‰ÔÏ›ÓÈÔ) ·Ú¤¯ÂÈ Î·È ÙËÓ ‰˘Ó·ÙfiÙËÙ· Ù˘ ·ÛÊ·ÏÔ‡˜ ‰È·ÊÔÚԉȿÁÓˆÛ˘ ·fi ÙȘ ·Ì·Úو̷ÙÒ‰ÂȘ ‚Ï¿‚˜ Ù˘ ÓfiÛÔ˘, ÔÈ Ôԛ˜ ‰ÂÓ ÚÔÛÏ·Ì‚¿ÓÔ˘Ó ÛÎÈ·ÛÙÈÎfi (8,10).

Pediatr May-June 06

16-05-06

17:21

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¡Â˘ÚÈӈ̿وÛË - ÂÓ‰ÔÎÚ¿ÓÈ· ÓÂÔÏ¿ÛÌ·Ù·

∆· ÔÙÈο ÚÔÎÏËÙ¿ ‰˘Ó·ÌÈο (V∂P) ¤¯Ô˘Ó Â›Û˘ ¯ÚËÛÈÌÔÔÈËı› ÛÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ·È‰ÈÒÓ Ì NF1 Î·È ·Ó·ÊÔÚ¤˜ ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ‰›ÓÔ˘Ó 100% ¢·ÈÛıËÛ›· Î·È 60% ÂȉÈÎfiÙËÙ· ÛÙËÓ ·Ó›¯Ó¢ÛË ‚Ï·‚ÒÓ, ·ÎfiÌË Î·È Û ·Û˘Ìو̷ÙÈÎÔ‡˜ ·ÛıÂÓ›˜ ‹ Î·È Û ·ÛıÂÓ›˜ Ì ·ÚÓËÙÈ΋ ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË (17,28). ∂ÓÙÔ‡ÙÔȘ, ˘¿Ú¯Ô˘Ó Î·È ÂÚÈÙÒÛÂȘ fiÔ˘ ·ÓˆÌ·Ï›Â˜ ÛÙ· VEP ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Î·È Û ÂÚÈÙÒÛÂȘ fiÔ˘ ‰ÂÓ ˘¿Ú¯ÂÈ ÔÙÈÎfi ÁÏÔ›ˆÌ·, ·ÏÏ¿ ·ÏÒ˜ Ì›· ÂÎÙ·Û›· Ù˘ ÛÎÏËÚ¿˜ Ì‹ÓÈÁÁ·˜ Ô˘ ÂÚÈ‚¿ÏÏÂÈ ÙÔ Ó‡ÚÔ, ÂÓÒ ‰ÂÓ Ú¤ÂÈ Ó· ͯÓԇ̠fiÙÈ Î·È Ë Û˘ÓÂÚÁ·Û›· ÙÔ˘ ÌÈÎÚÔ‡ ·ÛıÂÓÔ‡˜ ÌÔÚ› Ó· ÌËÓ Â›Ó·È È‰È·›ÙÂÚ· ÈηÓÔÔÈËÙÈ΋ Û ËÏÈ˘ ÌÈÎÚfiÙÂÚ˜ ÙˆÓ 5 ÂÙÒÓ (17). √ Û˘Ó‰˘·ÛÌfi˜ ÙˆÓ VEP Ì ÙȘ ¿ÏϘ ‰È·ÁÓˆÛÙÈΤ˜ ÌÂıfi‰Ô˘˜ ÂϤÁ¯Ô˘ ‰›ÓÂÈ Ù· ϤÔÓ ·ÍÈfiÈÛÙ· ·ÔÙÂϤÛÌ·Ù· (10). ™Â ·Û˘Ìو̷ÙÈÎÔ‡˜ ·ÛıÂÓ›˜ Ì NF1, ·fi ÏÂ˘Ú¿˜ ÚÔÏËÙÈÎÒÓ ÂÍÂÙ¿ÛˆÓ, ȉȷ›ÙÂÚË ÛËÌ·Û›· ‰›‰ÂÙ·È ÛÙÔ˘˜ Â·Ó·Ï·Ì‚·ÓfiÌÂÓÔ˘˜ ÔÊı·ÏÌÔÏÔÁÈÎÔ‡˜ ÂϤÁ¯Ô˘˜, ÔÈ ÔÔ›ÔÈ Î·Ïfi Â›Ó·È Ó· ·Ú¯›˙Ô˘Ó ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘. ¶Ï‹Ú˘ ÔÊı·ÏÌÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÙÔ˘Ï¿¯ÈÛÙÔÓ Ì›· ÊÔÚ¿ ÙÔÓ ¯ÚfiÓÔ ÛÙȘ ËÏÈ˘ οو ÙˆÓ ¤ÍÈ ÂÙÒÓ, ÌÈ· Î·È Û ·˘Ù‹ ÙËÓ ËÏÈÎȷ΋ ÔÌ¿‰· ÂÌÊ·Ó›˙ÂÙ·È Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ √ƒ∆, ÂÓÒ Û ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ˘ ÌÔÚ› Ó· Á›ÓÂÙ·È Î·È Û ·Ú·ÈfiÙÂÚ· ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù· (10). √ Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÚÔ˘Ù›Ó·˜ (Ì›· ÊÔÚ¿ ÙÔÓ ¯ÚfiÓÔ) ‰ÂÓ Û˘ÓÈÛÙ¿Ù·È Û ·È‰È¿ ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 6 ÂÙÒÓ, ÂÎÙfi˜ Î·È ·Ó ÚÔ·„Ô˘Ó Â˘Ú‹Ì·Ù· ·fi ÙÔÓ ÔÊı·ÏÌÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô. √È ·ÛıÂÓ›˜ ·˘Ù‹˜ Ù˘ ηÙËÁÔÚ›·˜ Ú¤ÂÈ Ó· ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È ÛÙÂÓ¿ Î·È ÁÈ· ÙËÓ ÂΉ‹ÏˆÛË Û˘ÌÙˆÌ¿ÙˆÓ ÚÒÈÌ˘ ‹‚˘, fiˆ˜ Ë ·fiÙÔÌË ·‡ÍËÛË ·ÏÏ¿ Î·È ÂÈ‚Ú¿‰˘ÓÛË ÙÔ˘ ‡„Ô˘˜ ‹ Ë ÚfiˆÚË ÂÌÊ¿ÓÈÛË ÙˆÓ ‰Â˘ÙÂÚ¢fiÓÙˆÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ ÙÔ˘ ʇÏÔ˘. ∂ÈϤÔÓ, Û fiÛÔ˘˜ ·ÛıÂÓ›˜ ÙÂı› Ë ‰È¿ÁÓˆÛË ÂÓfi˜ ÁÏÔÈÒÌ·ÙÔ˜ ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ¯È¿ÛÌ·ÙÔ˜, ÂÈ‚¿ÏÏÂÙ·È Ó· Á›ÓÂÈ ¤ÏÂÁ¯Ô˜ ÁÈ· ÚÒÈÌË ‹‚Ë ‹ ¿ÏϘ ÂÓ‰ÔÎÚÈÓÔÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜. √È ‰ÔÎÈ̷ۛ˜ ̤ÙÚËÛ˘ Ù˘ ˆ¯ÚÈÓÔÔÈËÙÈ΋˜ ÔÚÌfiÓ˘ (LH) ÌÔÚÔ‡Ó Ó· ‰ÒÛÔ˘Ó ‚ÈÔ¯ËÌÈο ÛÙÔȯ›· ÚÒÈÌ˘ ÂÓÂÚÁÔÔ›ËÛ˘ ÙÔ˘ ¿ÍÔÓ· ˘Ôı·Ï¿ÌÔ˘ - ˘fiÊ˘Û˘ - ÁÔÓ¿‰ˆÓ, ·ÎfiÌ· Î·È ÚÈÓ ÙËÓ ÎÏÈÓÈ΋ ÂΉ‹ÏˆÛË Û˘Ìو̿وÓ. √È ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘ Ù˘ LHRH ‰ÂÓ ÎÚ›ÓÔÓÙ·È ··Ú·›ÙËÙ˜ (24). ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Ù˘ ÚÒÈÌ˘ ‹‚˘ Â›Ó·È ÛËÌ·ÓÙÈ΋ Î·È ÁÈ· ÙÔÓ ÏfiÁÔ fiÙÈ ·ÔÙÂÏ› ÌÈ· ÂÓ ‰˘Ó¿ÌÂÈ È¿ÛÈÌË Î·Ù¿ÛÙ·ÛË, ÌÈ· Î·È Ê·›ÓÂÙ·È fiÙÈ ÔÈ ÌÈÎÚÔ› ·ÛıÂÓ›˜

··ÓÙÔ‡Ó ıÂÙÈο ÛÙË ıÂÚ·›· Ì ·ÓÙ·ÁˆÓÈÛÙ¤˜ Ù˘ ÂÎÏ˘ÙÈ΋˜ ÔÚÌfiÓ˘ ÙˆÓ LH-FSH (LHRH) (23). ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ú¤ÂÈ Ó· Á›ÓÂÈ ·fi ÙÔÓ Û·Î¯·ÚÒ‰Ë ‰È·‚‹ÙË, ÙË ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ı˘ÚÂÔÂȉԇ˜ Î·È ÙÔ Û‡Ó‰ÚÔÌÔ ·Ú¿‰Ô͢ ¤ÎÎÚÈÛ˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘, Ô˘ ÌÔÚ› Ó· ÚÔηϤÛÔ˘Ó ·ÚfiÌÔÈ· ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ÂÈÎfiÓ·. ªÈ· ·Ú¯È΋ ÂÚ›Ô‰Ô˜ ÚÔÛÂÎÙÈ΋˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ Â›Ó·È ›Ûˆ˜ Ë Î·Ï‡ÙÂÚË ÛÙÚ·ÙËÁÈ΋ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì ÌÂÌÔӈ̤ӷ ÁÏÔÈÒÌ·Ù· ÙÔ˘ ÔÙÈÎÔ‡ Ó‡ÚÔ˘ ‹ ÙÔ˘ ¯È¿ÛÌ·ÙÔ˜ Î·È Ì ϛÁ· ‹ Î·È Î·ıfiÏÔ˘ Û˘ÌÙÒÌ·Ù·. ™‡Ìʈӷ Ì ÙËÓ ∂ÈÙÚÔ‹ ∂Ú¢ӷ˜ ÁÈ· Ù· ÁÏÔÈÒÌ·Ù· Ù˘ ÔÙÈ΋˜ Ô‰Ô‡ ÛÙË NF1 (NF1 Optic Pathway Glioma ∆ask Force) Û ·È‰È¿ Ì ‚Ï¿‚˜ ÛÙ·ıÂÚ¤˜ Î·È ¯ˆÚ›˜ Ù¿ÛË ÁÈ· Â¤ÎÙ·ÛË, ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì MRI ÛÙÔ˘˜ 3, 9, 15, 24 Î·È 36 Ì‹Ó˜ ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË Î·È Ï‹Ú˘ ÔÊı·ÏÌÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÛÙÔ˘˜ 3, 6, 9, 12, 18, 24 Î·È 36 Ì‹Ó˜ (8). ∆· ‰È·ı¤ÛÈÌ· ıÂÚ·¢ÙÈο ̤۷ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÎÙÔÌ‹, ÙËÓ ·ÎÙÈÓÔ‚fiÏËÛË ÙÔ˘ fiÁÎÔ˘ Î·È ÙË ¯ËÌÂÈÔıÂÚ·›·, ÌfiÓ· ÙÔ˘˜ ‹ ÛÂ Û˘Ó‰˘·ÛÌfi (8). ∂ÈıÂÙÈ΋ ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË ··ÈÙÂ›Ù·È fiÙ·Ó Ô fiÁÎÔ˜ ÂÌÊ·Ó›˙ÂÈ Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈ΋ ‹ ÎÏÈÓÈ΋ Âȉ›ӈÛË. ∏ ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÂÓÙÔÈÛÌ¤ÓˆÓ ÛÙ· ÔÙÈο Ó‡ڷ ÁÏÔÈˆÌ¿ÙˆÓ ¤¯ÂÈ ¤Ó‰ÂÈÍË ÌfiÓÔ ÛÙȘ ÂÚÈÙÒÛÂȘ Ô˘ ˘¿Ú¯ÂÈ Î›Ó‰˘ÓÔ˜ ÁÈ· Ô›ÛıÈ· Â¤ÎÙ·ÛË ÙÔ˘ ·Ó·Ù˘ÛÛfiÌÂÓÔ˘ ÓÂÔÏ¿ÛÌ·ÙÔ˜, ‹ ÁÈ· ·ÈÛıËÙÈÎÔ‡˜ ÏfiÁÔ˘˜ Û ÔÊı·ÏÌÔ‡˜ Ì ÌÂÁ¿ÏË ÚfiÙˆÛË (11). ™˘¯Ó¿ ··ÈÙÂ›Ù·È ÂÚÈÔÚÈṲ̂ÓË ·ÎÙÈÓÔ‚fiÏËÛË ‹ ¯ËÌÂÈÔıÂÚ·›· ÙÔ˘ fiÁÎÔ˘ ÚÈÓ ÙËÓ ÂÍ·›ÚÂÛË, Ì ÛÎÔfi ÙË Ì›ˆÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙÔ˘. §fiÁˆ Ù˘ ÁÂÈÙÓ›·Û˘ ÙˆÓ ÁÏÔÈˆÌ¿ÙˆÓ ÙÔ˘ ¯È¿ÛÌ·ÙÔ˜/˘Ôı·Ï¿ÌÔ˘ Ì ˙ˆÙÈΤ˜ ÂÚÈÔ¯¤˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, Ë ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘˜ Â›Ó·È ÔÏ˘ÏÔÎfiÙÂÚË Î·È ÈÔ ÚÔ‚ÏËÌ·ÙÈ΋. ∂Ӊ›ÎÓ˘Ù·È ÏÔÈfiÓ, Î·È Û ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË, Ë ÛÙÂÓ‹ ÙÔ˘˜ ·Ú·ÎÔÏÔ‡ıËÛË ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË, ·ÏÏ¿ Î·È Ë ·Ó¿ÏË„Ë ÂÈıÂÙÈÎfiÙÂÚ˘ ıÂÚ·¢ÙÈ΋˜ ‰Ú¿Û˘ Û ÂӉ›ÍÂȘ ÎÏÈÓÈ΋˜ ‹ Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈ΋˜ Âȉ›ӈÛ˘. ∏ ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙˆÓ fiÁΈÓ, Û·Ó›ˆ˜ Ô‰ËÁ› ÛÙËÓ Ï‹ÚË ·Ê·›ÚÂÛ‹ ÙÔ˘˜. ∂ÓÙÔ‡ÙÔȘ, Î·È Ë ÌË Ï‹Ú˘ ·Ê·›ÚÂÛË ÙÔ˘ fiÁÎÔ˘ ¤¯ÂÈ ÂÓ›ÔÙ ¤Ó‰ÂÈÍË, ÂȉÈο Û ÂÚÈÙÒÛÂȘ fiÔ˘ ··ÈÙÂ›Ù·È Ë ¿ÌÂÛË ¿ÚÛË ÙˆÓ Û˘Ìو̿وÓ, fiˆ˜ Ô ·ÔÊÚ·ÎÙÈÎfi˜ ˘‰ÚÔΤʷÏÔ˜, ‹ ÂΛ ¶·È‰È·ÙÚÈ΋ 2006;69:178-186

Pediatr May-June 06

16-05-06

17:21

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¢.π. ∑·ÊÂÈÚ›Ô˘ Î·È Û˘Ó.

fiÔ˘ ÂÎÙÈÌ¿Ù·È fiÙÈ ı· ÂÈ‚Ú·‰˘Óı› Ë ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘. ™Â οı ÂÚ›ÙˆÛË, fï˜, ¯ÂÈÚÔ˘ÚÁÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘ ··ÈÙÂ›Ù·È Û˘ÓÙËÚËÙÈ΋ ÚÔÛ¤ÁÁÈÛË Î·È Û˘ÓÂÎÙ›ÌËÛË ÙˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ Î·È ÂÓ‰ÔÎÚÈÓÔÏÔÁÈÎÒÓ ÂÈÙÒÛÂˆÓ Ù˘ Â¤Ì‚·Û˘ (30,31). ∏ ·ÎÙÈÓÔıÂÚ·›· Â›Ó·È Â›Û˘ ÚÔ‚ÏËÌ·ÙÈ΋ Û ÂÚÈÙÒÛÂȘ ÁÏÔÈˆÌ¿ÙˆÓ Ù˘ ÂÚÈÔ¯‹˜ ÙÔ˘ ¯È¿ÛÌ·ÙÔ˜ ÏfiÁˆ ÙˆÓ ÂÓ‰ÔÎÚÈÓÔÏÔÁÈÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ (·ÓÂ¿ÚÎÂÈ· Ù˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘, ˘Ôı˘ÚÂÔÂȉÈÛÌfi, ¿ÔÈÔ ‰È·‚‹ÙË, ÚÒÈÌË ‹‚Ë Î·È ÚˆÙÔ·ı‹ ·ÌËÓfiÚÚÔÈ· ‹ ·ÎfiÌË Î·È Ï‹ÚË ·ÓÂ¿ÚÎÂÈ· Ù˘ ˘fiÊ˘Û˘) Î·È Ó¢ÚÔ„˘¯ÔÏÔÁÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ, Ì ÙÔ˘˜ ÌÈÎÚfiÙÂÚÔ˘˜ Û ËÏÈΛ· ·ÛıÂÓ›˜ Ó· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙË ÌÂÁ·Ï‡ÙÂÚË Â˘·ÈÛıËÛ›· ∂›Û˘, Ë ÚÔηÏÔ‡ÌÂÓË ·ÁÁÂȷ΋ ‚Ï¿‚Ë ÛÙËÓ ÂÚÈÔ¯‹ ÌÔÚ› Û ‚¿ıÔ˜ ¯ÚfiÓÔ˘ Ó· Ô‰ËÁ‹ÛÂÈ Û ÈÛ¯·ÈÌÈο ÂÂÈÛfi‰È· Ô˘ ı· ÂȉÂÈÓÒÛÔ˘Ó ÙË Ó¢ÚÔÏÔÁÈ΋ ηٿÛÙ·ÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ ‹ ÂӉ¯Ô̤ӈ˜ Î·È Ó· ·Ô‚Ô‡Ó ÌÔÈÚ·›· (10,31,32). ∂ÈÚfiÛıÂÙ·, Ë ¯Ú‹ÛË Ù˘ ·ÎÙÈÓÔıÂÚ·›·˜ Û ·ÛıÂÓ›˜ Ì NF1, ÌÈ· ÔÌ¿‰· ÏËı˘ÛÌÔ‡ Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ÓÂÔÏ·ÛÈÒÓ, ÈÛÙ‡ÂÙ·È fiÙÈ ÌÔÚ› Ó· ÚÔηϤÛÂÈ ‰Â˘ÙÂÚÔ·ı‹ ÔÁÎÔÁ¤ÓÂÛË. ∂ÓÙÔ‡ÙÔȘ, ‰ÂÓ ˘¿Ú¯Ô˘Ó ̤¯ÚÈ Û‹ÌÂÚ· ·‰È¿ÛÂÈÛÙ· ÛÙÔȯ›· ÁÈ· ÙËÓ ÙÂÎÌËÚ›ˆÛË ·˘Ù‹˜ Ù˘ ¿Ԅ˘ (33). √È ·Ó·ÛÙÔϤ˜ Û ۯ¤ÛË Ì ÙË ¯Ú‹ÛË ÙˆÓ ¯ÂÈÚÔ˘ÚÁÈÎÒÓ ÌÂıfi‰ˆÓ Î·È Ù˘ ·ÎÙÈÓÔıÂÚ·¢ÙÈ΋˜, Ô‰‹ÁËÛ·Ó ÛÙËÓ ·Ó¿Ù˘ÍË ¯ËÌÂÈÔıÂÚ·¢ÙÈÎÒÓ Û¯ËÌ¿ÙˆÓ Ô˘ ·ÔÙÂÏÔ‡ÓÙ·È ·fi ¤Ó· ‹ ÂÚÈÛÛfiÙÂÚ· ÛÂ Û˘Ó‰˘·ÛÌfi ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Û˘Ìو̷ÙÈÎÒÓ Î·È ÂÂÎÙÂÈÓfiÌÂÓˆÓ ¯È·ÛÌ·ÙÈÎÒÓ ÁÏÔÈˆÌ¿ÙˆÓ Ê¿Ú̷η. √ Û˘Ó‰˘·ÛÌfi˜ Ù˘ ‚ÈÓÎÚÈÛÙ›Ó˘ Î·È Ù˘ ηڂÔÏ·Ù›Ó˘, Û 58 ·ÛıÂÓ›˜ Ì √ƒ∆, ÂÎ ÙˆÓ ÔÔ›ˆÓ ÔÈ 15 Ì NF1, ¤‰ˆÛ ÈηÓÔÔÈËÙÈο ·ÔÙÂϤÛÌ·Ù·, Ì ÙÔ 70% ÙˆÓ ·ÛıÂÓÒÓ Ó· ÌËÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Â¤ÎÙ·ÛË ÙÔ˘ fiÁÎÔ˘ ÁÈ· Ì›· ÙÚÈÂÙ›·. ∏ ·¿ÓÙËÛË ÛÙËÓ ıÂÚ·›· ‹Ù·Ó ·ÓÂÍ¿ÚÙËÙË ·fi ÙËÓ ‡·ÚÍË Ù˘ NF1, ÂÓÒ ÔÈ ÌÈÎÚfiÙÂÚ˘ ËÏÈΛ·˜ ·ÛıÂÓ›˜ ›¯·Ó ηχÙÂÚ· ·ÔÙÂϤÛÌ·Ù· (34). ∂›Û˘, Ë ÙÔÍÈÎfiÙËÙ· Ô˘ ·Ó·Ê¤ÚıËΠ‹Ù·Ó ÂÏ¿¯ÈÛÙË (35). ∏ ·ÛÊ¿ÏÂÈ· Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ηڂÔÏ·Ù›Ó˘ ÂȂ‚·ÈÒÓÂÙ·È Î·È ·fi ·ÎfiÌË Ì›· ¤Ú¢ӷ Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠ÌÈÎÙfi ‰Â›ÁÌ· ·È‰ÈÒÓ (ÌÂ Î·È ¯ˆÚ›˜ NF1) Ì √ƒ∆, fiÔ˘ ÙÔ ÈηÓÔÔÈËÙÈÎfi ıÂÚ·¢ÙÈÎfi ·ÔÙ¤ÏÂÛÌ· Û˘Ó‰˘¿ÛÙËΠ̠ÂÚÈÔÚÈṲ̂Ó˜ ÙÔÍÈΤ˜ ·ÓÙȉڿÛÂȘ ·fi ÙȘ Ôԛ˜ ÔÈ ÛÔ˘‰·ÈfiÙÂÚ˜ ‹Ù·Ó Ë Ô˘‰ÂÙÂÚÔÂÓ›· Î·È Î˘Ú›ˆ˜, Ë ıÚÔÌ‚ÔÂÓ›· (36). ¶·È‰È·ÙÚÈ΋ 2006;69:178-186

√ ·ÚÈÔ˜ ıÂÚ·¢ÙÈÎfi˜ ÛÙfi¯Ô˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÂÈıÂÙÈο √ƒ∆ Â›Ó·È Ó· ÂÈÙ‡¯Ô˘Ì ÙË ‰È·ÛÊ¿ÏÈÛË Ù˘ ηχÙÂÚ˘ ‰˘Ó·Ù‹˜ ÔÈfiÙËÙ·˜ ˙ˆ‹˜, Ì ‰È·Ù‹ÚËÛË Ê˘ÛÈÔÏÔÁÈ΋˜ fiÚ·Û˘ Î·È ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ ÂÓ‰ÔÎÚÈÓÒÓ ·‰¤ÓˆÓ Î·È ·Ú¿ÏÏËÏË ÂÏ·¯ÈÛÙÔÔ›ËÛË ÙˆÓ ·ÚÂÓÂÚÁÂÈÒÓ Ù˘ ¯ÔÚËÁÔ‡ÌÂÓ˘ ıÂÚ·›·˜ (37). ∏ ¯Ú‹ÛË Ù˘ ¯ËÌÂÈÔıÂÚ·›·˜ ¤¯ÂÈ ·Ô‰ÂȯÙ› fiÙÈ Î·ı˘ÛÙÂÚ› ‹ Î·È ·Ó·‚¿ÏÏÂÈ Â’ ·fiÚÈÛÙÔÓ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙˆÓ ÂÚÈÛÛfiÙÂÚÔ ÎÏ·ÛÈÎÒÓ ÌÂıfi‰ˆÓ (ÂÁ¯Â›ÚËÛË, ·ÎÙÈÓÔıÂÚ·›·) (25,38) ∏ ‰È·ÁÓˆÛÙÈ΋ ‚ÈÔ„›·, Û ÂÏ¿¯ÈÛÙ· ΤÓÙÚ· ·ÔÙÂÏ› ‰È·‰Èηۛ· ÚÔ˘Ù›Ó·˜, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ÙË ÌÂÁ¿ÏË ÏÂÈÔÓfiÙËÙ· ÙˆÓ OPT ÛÙËÓ ·È‰È΋ ËÏÈΛ· ·ÔÙÂÏÔ‡Ó ÈÏÔ΢ÙÙ·ÚÈο ·ÛÙÚÔ΢ÙÙÒÌ·Ù· (10). ∞Ó·ÎÂÊ·Ï·ÈÒÓÔÓÙ·˜, ÂÈÛËÌ·›ÓÂÙ·È Ë ·Ó·ÁηÈfiÙËÙ· ÙÔ˘ ÂÙ‹ÛÈÔ˘ ÔÊı·ÏÌÔÏÔÁÈÎÔ‡ Î·È Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘ fiÏˆÓ ÙˆÓ ·ÛıÂÓÒÓ Ì NF1 ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ ¤ÍÈ ÂÙÒÓ, ÂÓÒ Ì ÙË ‰È¿ÁÓˆÛË ÂÓfi˜ OPT ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È Û˘¯ÓfiÙÂÚ· Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì MRI, ÂÊfiÛÔÓ Ô fiÁÎÔ˜ ·Ú·Ì¤ÓÂÈ ÛÙ·ıÂÚfi˜ Î·È Ô ·ÛıÂÓ‹˜ ·Û˘Ìو̷ÙÈÎfi˜. ™Â ·ÓÙ›ıÂÙË ÂÚ›ÙˆÛË ··ÈÙÂ›Ù·È ‰È·ÊÔÚÂÙÈ΋ ÚÔÛ¤ÁÁÈÛË Ì ÙÔ˘˜ ÙÚfiÔ˘˜ Ô˘ ¤¯Ô˘Ó ‹‰Ë ÂÚÈÁÚ·Ê›.

°ÏÔÈÒÌ·Ù· ÙÔ˘ ÛÙÂϤ¯Ô˘˜ ∆· ÁÏÔÈÒÌ·Ù· Ù˘ ÂÚÈÔ¯‹˜ ÙÔ˘ ÛÙÂϤ¯Ô˘˜ Û ·È‰È¿ Ì NF1 ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÂÁ¿ÏÔ ÂӉȷʤÚÔÓ. ∏ Ê˘ÛÈ΋ ÙÔ˘˜ ÈÛÙÔÚ›· ηıÒ˜ Î·È Ë ÎÏÈÓÈ΋ Î·È Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈ΋ ÙÔ˘˜ Û˘ÌÂÚÈÊÔÚ¿ ‰ÂÓ ¤¯Ô˘Ó Á›ÓÂÈ Î·Ù·ÓÔËÙ¤˜ Û fiÏÔ ÙÔ Â‡ÚÔ˜ ÙÔ˘˜, Ì ·ÔÙ¤ÏÂÛÌ· Î·È Ë ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘˜ Ó· ·ÔÙÂÏ› Û˘¯Ó¿ ¤Ó· ÁÚ›ÊÔ. ∏ ·ÎÚÈ‚‹˜ ÙÔ˘˜ Û˘¯ÓfiÙËÙ· ‰ÂÓ Â›Ó·È ÁÓˆÛÙ‹, ·ÏÏ¿ Ë ËÏÈΛ· ÂÌÊ¿ÓÈÛ˘ Â›Ó·È Û˘Ó‹ıˆ˜ ÌÂÚÈο ¯ÚfiÓÈ· ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘. ™ÙÔ˘˜ 17 ·ÛıÂÓ›˜ Ô˘ ·ÍÈÔÏÔÁ‹ıËÎ·Ó ·fi ÙÔ˘˜ Molloy et al., Ë Ì¤ÛË ËÏÈΛ· Ù˘ ‰È¿ÁÓˆÛ˘ ÙÔ˘ fiÁÎÔ˘ ‹Ù·Ó 8,4 ¤ÙË.

15 ·ÛıÂÓ›˜ (88%) ÂÌÊ¿ÓÈ˙·Ó Û˘Ìو̷ÙÔÏÔÁ›· ηٿ ÙË ÛÙÈÁÌ‹ Ù˘ ‰È¿ÁÓˆÛ˘ ÙÔ˘ ÁÏÔÈÒÌ·ÙÔ˜ Ô˘ Û˘Ó›ÛÙ·ÙÔ Û ‰˘Û·ÚıÚ›·, Ó¢ÚÔ¿ıÂȘ ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ ÓÂ‡ÚˆÓ Î·È ‰È·Ù·Ú·¯¤˜ Ù˘ ·‰Ú‹˜ ÎÈÓËÙÈÎfiÙËÙ·˜, ÂÓ‰ÂÈÎÙÈο Ù˘ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ÛÙÂϤ¯Ô˘˜. √ fiÁÎÔ˜ ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ (82%) ÂÓÙÔÈ˙fiÙ·Ó ÛÙÔÓ ÚÔÌ‹ÎË Ì˘ÂÏfi (Û ·ÓÙ›ıÂÛË Ì ·ÛıÂÓ›˜ ¯ˆÚ›˜ NF1 fiÔ˘ Ë Û˘¯ÓfiÙÂÚË ÂÓÙfiÈÛË ‹Ù·Ó ÛÙË Á¤Ê˘Ú·). ŒÍÈ ·ÛıÂÓ›˜ ·ÚÔ˘Û›·Û·Ó Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈ΋ Âȉ›ӈÛË Î·È ÙÚÂȘ ·fi ·˘ÙÔ‡˜

Pediatr May-June 06

16-05-06

17:21

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¡Â˘ÚÈӈ̿وÛË - ÂÓ‰ÔÎÚ¿ÓÈ· ÓÂÔÏ¿ÛÌ·Ù·

Î·È ÎÏÈÓÈ΋ Âȉ›ӈÛË. ™Â ÂÙ¿ ·ÛıÂÓ›˜ ··ÈÙ‹ıËÎÂ Ë ÙÔÔı¤ÙËÛË ÎÔÈÏÈÔÂÚÈÙÔÓ·˚΋˜ ·ÚÔ¯¤Ù¢Û˘ ÁÈ· ÙËÓ ¿ÚÛË ÙˆÓ Û˘ÓÂÂÈÒÓ ÙÔ˘ ·ÔÊÚ·ÎÙÈÎÔ‡ ˘‰ÚÔΤʷÏÔ˘, ÂÓÒ ÌfiÓÔ Û ÙÚÂȘ ¯ÚÂÈ¿ÛÙËÎÂ Ë ÂÊ·ÚÌÔÁ‹ ÂÈϤÔÓ ¯ËÌÂÈÔıÂÚ·›·˜ ‹/Î·È ·ÎÙÈÓÔıÂÚ·›·˜ (39). ™Â ·ÓÙ›ÛÙÔȯ· ·ÔÙÂϤÛÌ·Ù· Ô‰‹ÁËÛ ÙÔ˘˜ Pollack et al. Ë ¤Ú¢ӷ Ô˘ Ú·ÁÌ·ÙÔÔ›ËÛ·Ó Û 21 ·ÛıÂÓ›˜ Ì NF1 Î·È ÁÏÔ›ˆÌ· ÙÔ˘ ÛÙÂϤ¯Ô˘˜. ¢Ò‰Âη ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ ÂÌÊ¿ÓÈ˙·Ó Û˘Ìو̷ÙÔÏÔÁ›·, fiˆ˜ Û˘ÌÙÒÌ·Ù· ·˘ÍË̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›ÂÛ˘ Û˘Ó¤ÂÈ· ÙÔ˘ ˘‰ÚÔÎÂÊ¿ÏÔ˘ Î·È Ó¢ÚÔ¿ıÂȘ ÙˆÓ ÂÁÎÂÊ·ÏÈÎÒÓ Û˘˙˘ÁÈÒÓ, ÂÓÙÔ‡ÙÔȘ ÌfiÓÔ Û 4 ·ÛıÂÓ›˜ ¯ÚÂÈ¿ÛÙËÎÂ Û˘ÁÎÂÎÚÈ̤ÓË ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË (ÂÎÙÔÌ‹, ·ÎÙÈÓÔıÂÚ·›·), ÂÓÒ Û ¿ÏÏÔ˘˜ 4 Ì fiÁÎÔ Ù˘ ÔÚÔÊ‹˜ Ù˘ 4˘ ÎÔÈÏ›·˜, ··ÈÙ‹ıËÎÂ Ë ÙÔÔı¤ÙËÛË ÎÔÈÏÔÂÚÈÙÔÓ·˚΋˜ ·ÚÔ¯¤Ù¢Û˘. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ (̤ÛÔ˜ fiÚÔ˜ 3,7 ¯ÚfiÓÈ·), 9 ·ÛıÂÓ›˜ ·ÚÔ˘Û›·Û·Ó Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈ΋ Âȉ›ӈÛË Î·È ÌfiÓÔ ÙÚÂȘ ÎÏÈÓÈ΋. √È ÂÚ¢ÓËÙ¤˜ ‰È·¯ÒÚÈÛ·Ó ÙÔ˘˜ ·ÛıÂÓ›˜ Û ÔÌ¿‰Â˜ ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÓÙfiÈÛË Î·È Ù· Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ fiÁÎÔ˘ Î·È Î·Ù¤ÏËÍ·Ó ÛÙÔ Û˘Ì¤Ú·ÛÌ· ˆ˜ ÔÈ ‚Ï¿‚˜ Ì ÙË ÌÂÁ·Ï‡ÙÂÚË Èı·ÓfiÙËÙ· Ó· ··ÈÙ‹ÛÔ˘Ó ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË Â›Ó·È ÔÈ Î·Ï¿ ·ÊÔÚÈ˙fiÌÂÓ˜ Ô˘ ÚÔÛÏ·Ì‚¿ÓÔ˘Ó ÛÎÈ·ÛÙÈÎfi, ÔÈ Ôԛ˜ ÛÙËÓ ÚÔÎÂÈ̤ÓË ÂÚ›ÙˆÛË ·ÔÙÂÏÔ‡Û·Ó ÙÔ 30% (¡=7). ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ ‹Ù·Ó ÂÓ ˙ˆ‹ ηٿ ÙËÓ ÂÔ¯‹ Ù˘ ‰ËÌÔÛ›Â˘Û˘ (40). ∂ÈϤÔÓ, Û ̛· ÙÚ›ÙË ·Ó·ÎÔ›ÓˆÛË ·fi ÙÔ˘˜ Billaniuk et al. ÂȂ‚·ÈÒÓÔÓÙ·È Ù· ·Ú·¿Óˆ, ηıÒ˜ Û ÌÂϤÙË 25 ·ÛıÂÓÒÓ Ë Û˘¯ÓfiÙÂÚË ÂÓÙfiÈÛË (68%) ‹Ù·Ó ÛÙÔÓ ÚÔÌ‹ÎË Ì˘ÂÏfi. ÃÂÈÚÔ˘ÚÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ fiÁÎÔ˘ ··ÈÙ‹ıËΠÌfiÓÔ Û 4 ·ÛıÂÓ›˜, ÂÓÒ Û ‰È¿¯˘ÙÔ˘˜ fiÁÎÔ˘˜ - ·Ó Î·È Û˘¯Ó¿ ··ÈÙ‹ıËÎÂ Ë ÙÔÔı¤ÙËÛË ÎÔÈÏÈÔÂÚÈÙÔÓ·˚ÎÔ‡ shunt (67%) - Ë ÂÈ‚›ˆÛË ‹Ù·Ó ÁÂÓÈο ηχÙÂÚË. ∆¤ÏÔ˜, ÛÙËÓ ·Ú·¿Óˆ ÌÂϤÙË ·Ó·Ê¤ÚÂÙ·È Û˘Ó‡·ÚÍË √ƒ∆ Î·È fiÁÎˆÓ ÙÔ˘ ÛÙÂϤ¯Ô˘˜ Û ÔÛÔÛÙfi 40% (41). ™˘ÌÂÚ·ÛÌ·ÙÈο, Ù· ÁÏÔÈÒÌ·Ù· ÙÔ˘ ÛÙÂϤ¯Ô˘˜ Ê·›ÓÂÙ·È Ó· Û˘ÌÂÚÈʤÚÔÓÙ·È ·ÚfiÌÔÈ· Ì ٷ √ƒ∆ ÛÙ· ·È‰È¿ Ì NF1. Œ¯Ô˘Ó ηٿ ÙÔ Ì¿ÏÏÔÓ ‹ ‹ÙÙÔÓ Â˘ÓÔÈ΋ ÔÚ›· Û ۇÁÎÚÈÛË Ì ٷ ÛÔÚ·‰Èο ÁÏÔÈÒÌ·Ù· Ù˘ ÂÚÈÔ¯‹˜ Û ·ÛıÂÓ›˜ ¯ˆÚ›˜ NF1 Î·È ÂÓ‰¤¯ÂÙ·È Ó· ÛÙ·ıÂÚÔÔÈËıÔ‡Ó ‹ Î·È Ó· Û˘ÚÚÈÎÓˆıÔ‡Ó ·ÎfiÌË Î·È ·ÊÔ‡ ηٷÛÙÔ‡Ó Û˘Ìو̷ÙÈο. ™˘¯Ó¿, Ô fiÁÎÔ˜ ‰ÂÓ ··ÈÙ› ·ÓÙÈÌÂÙÒÈÛË Î·È Ë ÙÔÔı¤ÙËÛË Ì›·˜ ÎÔÈÏÔÂÚÈÙÔÓ·˚΋˜ ·ÚÔ¯¤Ù¢Û˘ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ·ÔÊÚ·ÎÙÈÎÔ‡ ˘‰ÚÔ-

ÎÂÊ¿ÏÔ˘ ·ÚΛ. ∏ ÂÈÏÔÁ‹ Ù˘ ·ÎÙÈÓÔıÂÚ·›·˜ (ηٿ ·ÚÈÔ ÏfiÁÔ) ‹ Ù˘ ¯ÂÈÚÔ˘ÚÁÈ΋˜ ÂÎÙÔÌ‹˜ Ú¤ÂÈ Ó· Á›ÓÂÙ·È Ì ÊÂÈ‰Ò fiˆ˜ Î·È ÛÙ· √ƒ∆, ÂÓÒ, ·Ó Î·È ˘‹ÚÍ·Ó ÂÓı·ÚÚ˘ÓÙÈο ·ÔÙÂϤÛÌ·Ù· ·fi ÙËÓ ¯Ú‹ÛË Ù˘ ¯ËÌÂÈÔıÂÚ·›·˜, ·ÎfiÌË ‰ÂÓ ¤¯Ô˘Ó ηıÔÚÈÛÙ› Ì ·ÎÚ›‚ÂÈ· ÔÈ Î·Ù¿ÏÏËÏÔÈ ¯ËÌÂÈÔıÂÚ·¢ÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Î·È Û¯‹Ì·Ù· (39,40,42). ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ·Ó·ÁηÈfiÙËÙ· Ù˘ ‰È·ÁÓˆÛÙÈ΋˜ ‚ÈÔ„›·˜, ÈÛ¯‡ÂÈ fi,ÙÈ Î·È ÁÈ· Ù· OPT, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Ù· ÁÏÔÈÒÌ·Ù· ÙÔ˘ ÛÙÂϤ¯Ô˘˜ Û ·È‰È¿ Ì NF1 Ù›ÓÔ˘Ó Ó· Û˘ÌÂÚÈʤÚÔÓÙ·È Û·Ó OPT.

∞Ì·ÚÙÒÌ·Ù· ŒÓ· ·fi Ù· ۯ‰fiÓ ·ıÔÁÓˆÌÔÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο Ù˘ NF1 Â›Ó·È Ù· ·ÔηÏÔ‡ÌÂÓ· “·ÁÓÒÛÙÔ˘ Ù·˘ÙfiÙËÙ·˜ ʈÙÂÈÓ¿ ·ÓÙÈΛÌÂÓ·” (unidentified bright objects-UBOs), Ù· ÔÔ›· ··ÓÙÒÓÙ·È Û¯Â‰fiÓ ·ÔÎÏÂÈÛÙÈο ÛÙËÓ NF1. ∆· UBOs ̤¯ÚÈ ÚfiÛÊ·Ù·, ıˆÚÔ‡ÓÙ·Ó ·‰È¢ÎÚ›ÓÈÛÙ˘ ÈÛÙÔÏÔÁÈ΋˜ ÚÔ¤Ï¢Û˘ Î·È ‰È¿ÊÔÚ˜ ·fi„ÂȘ ›¯·Ó ηٷÙÂı› Û ۯ¤ÛË Ì ÙË Ê˘ÛÈ΋ ÙÔ˘˜ ÈÛÙÔÚ›·. ∂ÓÙÔ‡ÙÔȘ, ϤÔÓ ¤¯Ô˘Ó ·Ó·ÁÓˆÚÈÛÙ› ˆ˜ ηÏÔ‹ıÂȘ ·Ì·Úو̷ÙÒ‰ÂȘ ‚Ï¿‚˜ Ù˘ÈΤ˜ Ù˘ ÓfiÛÔ˘, Ô˘ ۯ‰fiÓ ¿ÓÙ· ˘ÔÛÙÚ¤ÊÔÓÙ·È Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘. ¶·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ˆ˜ ÂÛٛ˜ ·ıÔÏÔÁÈο ·˘ÍË̤ÓÔ˘ Ì·ÁÓËÙÈÎÔ‡ Û‹Ì·ÙÔ˜ ÛÙȘ ∆2 ·ÎÔÏÔ˘ı›Â˜ ÛÙËÓ MRI. ∞ÔÙÂÏÔ‡Ó ÙÔ Û˘¯ÓfiÙÂÚÔ Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎfi ‡ÚËÌ· ÛÙËÓ NF1 ÌÂ Û˘¯ÓfiÙËÙ· Ô˘ Î˘Ì·›ÓÂÙ·È ·fi 38% ¤ˆ˜ 93% ÛÙÔ˘˜ ·È‰È·ÙÚÈÎÔ‡˜ ·ÛıÂÓ›˜ (10,39,43). ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ·fi Ù· ÁÏÔÈÒÌ·Ù·, Î·È È‰›ˆ˜ ·˘Ù¿ ÙÔ˘ ÛÙÂϤ¯Ô˘˜ fiÔ˘ Á›ÓÂÙ·È ÂÓ›ÔÙÂ Î·È Ë ÌÂÁ·Ï‡ÙÂÚË Û‡Á¯˘ÛË (39), Â›Ó·È Û˘Ó‹ıˆ˜ ‡ÎÔÏË, ηıÒ˜ ÂÌÊ·Ó›˙ÔÓÙ·È ÌfiÓÔ ÛÙȘ ∆2 ·ÎÔÏÔ˘ı›Â˜, ‰ÂÓ ¤¯Ô˘Ó ¯ˆÚÔηٷÎÙËÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο, ‰ÂÓ ÚÔÛÏ·Ì‚¿ÓÔ˘Ó Á·‰ÔÏ›ÓÈÔ, ‰ÂÓ ÚÔηÏÔ‡Ó ÈÂÛÙÈο Ê·ÈÓfiÌÂÓ· Î·È ÂÛÙȷ΋ Û˘Ìو̷ÙÔÏÔÁ›·, Î·È ‰ÂÓ ¯ÚÂÈ¿˙ÔÓÙ·È Î·Ì›· ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË. UBOs ·Ó·Ù‡ÛÛÔÓÙ·È Î·È Û ¿ÏϘ ı¤ÛÂȘ, fiˆ˜ Â›Ó·È Ù· ËÌÈÛÊ·›ÚÈ· Ù˘ ·ÚÂÁÎÂÊ·Ï›‰·˜, Ô ÛÎÒÏËη˜, Ù· ‚·ÛÈο Á¿ÁÁÏÈ·, Ù· ËÌÈÛÊ·›ÚÈ· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È ÙÔ ÌÂÛÔÏfi‚ÈÔ (10). ™Â ÌÂϤÙË Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËΠ·fi ÙÔ˘˜ Iannaccone et al. Û 16 ·ÛıÂÓ›˜ Ì NF1 Î·È UBOs, ÌfiÓÔ ÔÈ 10 ·ÚÔ˘Û›·Û·Ó ·ıÔÏÔÁÈο VEP, ÂÓ‰ÂÈÎÙÈÎfi ÙÔ˘ fiÙÈ Ù· ·Ì·ÚÙÒÌ·Ù· ·˘Ù¿ ‰ÂÓ Ê·›ÓÂÙ·È Ó· ÂËÚ¿˙Ô˘Ó ·fiÏ˘Ù· ÙË Û˘ÁÎÂÎÚÈ̤ÓË ÂͤٷÛË (29). ¶·È‰È·ÙÚÈ΋ 2006;69:178-186

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¢.π. ∑·ÊÂÈÚ›Ô˘ Î·È Û˘Ó.

∏ ۯ‰fiÓ ·ÔÎÏÂÈÛÙÈ΋ Û¯¤ÛË ÙˆÓ UBOs Î·È Ù˘ NF1 Ô‰‹ÁËÛ ÂÚ¢ÓËÙ¤˜ Ó· ÚÔÙ›ÓÔ˘Ó ÙËÓ ÂÓۈ̿وۋ ÙÔ˘˜ ÛÙ· ÎÚÈÙ‹ÚÈ· ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘. µ¤‚·È·, Ë ÂÌÊ¿ÓÈÛ‹ ÙÔ˘˜ Û ηÙËÁÔڛ˜ ·ÛıÂÓÒÓ Ì ¿ÏϘ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ∫¡™, ηıÒ˜ Î·È Ë ˘ÔÛÙÚÔÊ‹ ÙÔ˘˜ Û ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ˘ ÚÔ¸Ôı¤ÙÂÈ fiÙÈ ÁÈ· Ó· ÛÙÔȯÂÈÔıÂÙËı› ‰È¿ÁÓˆÛË Ì ‚¿ÛË Ù· UBOs, Ù· ·Ì·ÚÙÒÌ·Ù· ı· Ú¤ÂÈ Ó· ÂÓÙÔ›˙ÔÓÙ·È ÌfiÓÔ ÛÙËÓ ·ÚÂÁÎÂÊ·Ï›‰·, ÙÔ Û٤ϯԘ Î·È Ù· ‚·ÛÈο Á¿ÁÁÏÈ· Î·È Ë ËÏÈΛ· ÙÔ˘ ·ÛıÂÓÔ‡˜ Ó· Â›Ó·È ÌÈÎÚfiÙÂÚË ÙˆÓ 6 ÂÙÒÓ (44). ∂ӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ Ë ÈÛ¯˘Ú‹ Û˘Û¯¤ÙÈÛË ÙˆÓ UBOs Ì ÙȘ Ì·ıËÛȷΤ˜ ‰È·Ù·Ú·¯¤˜ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ÔÈ ÌÈÎÚÔ› ·ÛıÂÓ›˜ Ì NF1 (45,46). ∂ÓÙÔ‡ÙÔȘ, Ù· UBOs ‰ÂÓ Â˘ı‡ÓÔÓÙ·È ·ÔÎÏÂÈÛÙÈο ÁÈ· ÙȘ Ì·ıËÛȷΤ˜ ‰È·Ù·Ú·¯¤˜, ηıÒ˜ ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ Û˘Û¯ÂÙ›˙Ô˘Ó ÙȘ ÌÂȈ̤Ó˜ ·Î·‰ËÌ·˚Τ˜ ÂȉfiÛÂȘ ·ÛıÂÓÒÓ Ì NF1 Ì ·Ú·Ì¤ÙÚÔ˘˜ fiˆ˜ Ô fiÁÎÔ˜ Ù˘ Ê·È¿˜ Ô˘Û›·˜ Î·È Ë ·Ó·ÏÔÁ›· fiÁÎÔ˘ Ê·È¿˜ ÚÔ˜ fiÁÎÔ Ï¢΋˜ Ô˘Û›·˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, ÙÔ Ì¤ÁÂıÔ˜ ÙÔ˘ ÌÂÛÔÏÔ‚›Ô˘, ηıÒ˜ Î·È ‰È·Ù·Ú·¯¤˜ Û ‰È·‰Èηۛ˜ fiˆ˜ Ô ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓÔ˜ ΢ÙÙ·ÚÈÎfi˜ ı¿Ó·ÙÔ˜ (·fiÙˆÛË) (47,48). ∏ ·fiÙˆÛË Â›Ó·È Ì›· Ê˘ÛÈÔÏÔÁÈ΋ ‰È·‰Èηۛ· Ô˘ Ï·Ì‚¿ÓÂÈ ¯ÒÚ· Û ‰È¿ÊÔÚ· Û˘ÛÙ‹Ì·Ù· Î·È Û Â›Â‰Ô ÂÁÎÂÊ¿ÏÔ˘ Ô‰ËÁ› Û ·Ó·Î·Ù·Û΢‹ ÙˆÓ Ó¢ÚÈÎÒÓ Û˘Ó¿„ˆÓ. ¢È·ÎÔ‹ ‹ ηı˘ÛÙ¤ÚËÛË Ù˘ ·Ú·¿Óˆ ‰È·‰Èηۛ·˜ ¤¯ÂÈ ˆ˜ Û˘Ó¤ÂÈ· ÙËÓ ·Ó¿Ù˘ÍË ˘ÂÚÏ·ÛÙÈÎÒÓ ÂÚÈÔ¯ÒÓ Ì ¿Ù˘· ÌÔÓ٤Ϸ Ó¢ÚÈÎÒÓ Û˘Ó¿„ˆÓ, Ù· ÔÔ›· Ì ÙË ÛÂÈÚ¿ ÙÔ˘˜ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ· Ì·ıËÛȷΤ˜ ‰È·Ù·Ú·¯¤˜ (48).

§ÔÈ¿ ÂÓ‰ÔÎÚ¿ÓÈ· ÓÂÔÏ¿ÛÌ·Ù· ∂ÎÙfi˜ ÙˆÓ ¿ÏψÓ, Ù· ·È‰È¿ Ì NF1 Ê·›ÓÂÙ·È Ó· ·ÓÙÈÌÂÙˆ›˙Ô˘Ó Î·È ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ·Ó¿Ù˘Í˘ Î·È ¿ÏÏˆÓ ÂÓ ‰˘Ó¿ÌÂÈ Î·ÎÔ‹ıˆÓ ÓÂÔÏ·ÛÈÒÓ. √È Ì¤¯ÚÈ ÙÒÚ· ¤Ú¢Ó˜ ‰Â›¯ÓÔ˘Ó fiÙÈ ˘¿Ú¯ÂÈ ÌÈ· ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ηÏÔ‹ıˆÓ Î·È Î·ÎÔ‹ıˆÓ fiÁÎˆÓ fiˆ˜ ·ÛÙÚÔ΢ÙÒÌ·Ù·, ÂÂÓ‰˘ÌÒÌ·Ù·, ÌËÓÈÓÁÁÈÒÌ·Ù·, Ó¢ÚÔÛ·ÚÎÒÌ·Ù· Î·È Ì˘ÂÏÔ‚Ï·ÛÙÒÌ·Ù·, ȉȷ›ÙÂÚ· ÛÙ· ·È‰È¿ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ÔÙÈο ÁÏÔÈÒÌ·Ù· (49). ∏ Û˘¯ÓfiÙËÙ· ÙˆÓ ÓÂÔÏ·ÛÈÒÓ ·˘ÙÒÓ Î˘Ì·›ÓÂÙ·È ·fi 1-3% (50), Ì ÙËÓ ·ÚÂÁÎÂÊ·Ï›‰· Î·È Ù· ÎÚ·Óȷο Ó‡ڷ Ó· ·ÔÙÂÏÔ‡Ó ‰‡Ô ·fi ÙȘ Û˘¯ÓfiÙÂÚ˜ ı¤ÛÂȘ ÂÌÊ¿ÓÈÛ˘. ∏ Ê˘ÛÈ΋ ÈÛÙÔÚ›· ·˘ÙÒÓ ÙˆÓ fiÁÎˆÓ ÌÔÈ¿˙ÂÈ Ì ·˘Ù‹ ÙˆÓ ÛÔÚ·‰ÈÎÒÓ fiÁÎˆÓ Ô˘ ··ÓÙÒÓÙ·È ÛÙÔÓ ÁÂÓÈÎfi Ï˶·È‰È·ÙÚÈ΋ 2006;69:178-186

ı˘ÛÌfi Î·È ‰ÂÓ ˘¿Ú¯ÂÈ Î¿ÔÈ· ÂȉÈÎfiÙÂÚË ·ÓÙÈÌÂÙÒÈÛË ·˘ÙÒÓ (10).

∂›ÏÔÁÔ˜ ∏ ÔÁÎÔÁ¤ÓÂÛË, Â›Ó·È ¤Ó· ȉȷ›ÙÂÚÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi Ù˘ NF1 Î·È ·ÔÙÂÏ› Î·È Ì›· ·fi ÙȘ ΢ÚÈfiÙÂÚ˜ ·Èٛ˜ ÁÈ· ÙË ÓÔÛËÚfiÙËÙ· Î·È ÙË ıÓËÙfiÙËÙ¿ Ù˘. ¶·Ú·ÙËÚÂ›Ù·È ˆ˜ Â› ÙÔ Ï›ÛÙÔÓ ÛÙȘ Ó·ڤ˜ ËÏÈ˘ οو ÙˆÓ 10 ÂÙÒÓ, ·ÏÏ¿ ‰ÂÓ ·‡ÂÈ Ó· Â›Ó·È Î·È ¤Ó· ÁÂÓÈÎfiÙÂÚÔ Úfi‚ÏËÌ· ÁÈ· fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Ì NF1, Ô˘ ÙÔ˘˜ Û˘Óԉ‡ÂÈ Û fiÏË ÙÔ˘˜ ÙË ˙ˆ‹ (51).

µÈ‚ÏÈÔÁÚ·Ê›· 1. Friedman JM. Epidemiology of neurofibromatosis type 1. Am J Med Genet 1999;89:1-6.

2. Friedman JM. Neurofibromatosis 1: clinical manifestations and diagnostic criteria. J Child Neurol 2002;17:548-554.

3. Arun D, Gutmann DH. Recent advances in neurofibromatosis type 1. Curr Opin Neurol 2004;17: 101-105.

4. Listernick R, Charrow J, Gutmann DH. Intracranial gliomas in neurofibromatosis type 1. Am J Med Genet 1999;89:38-44.

5. Perilongo G, Moras P, Carollo C, Battistella A, Clementi M, Laverda A, et al. Spontaneous partial regression of low-grade glioma in children with neurofibromatosis-1: a real possibility. J Child Neurol 1999;14:352-356.

6. Parsa CF, Hoyt CS, Lesser RL, Weinstein JM, Strother CM, Muci-Mendoza R, et al. Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging. Arch Opthalmol 2001;119:516-529.

7. Schmandt SM, Packer RJ, Vezina LG, Jane J. Spontaneous regression of low-grade astrocytomas in childhood. Pediatr Neurosurg 2000;32:132-136.

8. Listernick R, Louis DN, Packer RJ, Gutmann DH. Optic pathway gliomas in children with neurofibromatosis 1: consensus statement from the NF1 Optic Pathway Glioma Task Force. Ann Neurol 1997;41: 143-149.

9. Listernick R, Darling C, Greenwald M, Strauss L, Charrow J. Optic pathway tumors in children: the effect of neurofibromatosis type 1 on clinical manifestations and natural history. J Pediatr 1995;127: 718-722.

10. Rosser T, Packer RJ. Intracranial neoplasms in children with neurofibromatosis 1. J Child Neurol 2002;17:630-637.

11. Friedman JM, Gutmann DH, MacCollin M, Riccardi VM, editors. Neurofibromatosis: Phenotype, Natural history and Pathogenesis. 3rd ed. Baltimore: John Hopkins University Press; 1999.

Pediatr May-June 06

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™ÂÏ›‰·185

185

¡Â˘ÚÈӈ̿وÛË - ÂÓ‰ÔÎÚ¿ÓÈ· ÓÂÔÏ¿ÛÌ·Ù·

12. Dutton JJ. Gliomas of the anterior visual pathway.

Surv Opthalmol 1994;38:427-452.

13. Czyzyk E, Jozwiak S, Roszkowski M, Schwartz RA. Optic pathway gliomas in children with and without neurofibromatosis 1. J Child Neurol 2003;18: 471-478.

14. Lewis RA, Gerson LP, Axelson KA, Riccardi VM, Whitford RP. von Recklinhausen neurofibromatosis. II. Incidence of optic gliomata. Ophthalmology 1984;91:929-935.

15. Listernick R, Charrow J, Greenwald M, Mets M. Natural history of optic pathway tumors in children with neurofibromatosis type 1: a longitudinal study.

J Pediatr 1994;125:63-66.

16. Listernick R, Charrow J, Greenwald MJ, Esterly NB. Optic gliomas in children with neurofibromatosis type 1. J Pediatr 1989;114:788-792.

17. ¡Ôrth K, Cochineas C, Tang E, Fagan E. Optic gliomas in neurofibromatosis type 1: role of visual evoked potentials. Pediatr Neurol 1994;10:117-123.

18. Kuenzle C, Weissert M, Roulet E, Bode H, Schefer S, Huisman T, et al. Follow up of optic pathway gliomas in children with neurofibromatosis type 1.

Neuropediatrics 1994;25:295-300.

19. Curless RG, Siatkowski M, Glaser JS, Shatz NJ. MRI diagnosis of NF-1 in children without café-au-lait skin spots. Pediatr Neurol 1998;18:269-271.

20. Thiagalingam S, Flaherty M, Billson F, North K. Neurofibromatosis type 1 and optic pathway gliomas: follow up of 54 patients. Ophthalmology 2004;111:568-577.

21. Blazo MA, Lewis RA, Chintagumpala MM, Frazier M, McCluggage C, Plon SE. Outcomes of systematic screening for optic pathway tumors in children with neurofibromatosis type 1. Am J Med Genet 2004;127:224-229.

22. Schroder S, Baumann-Schroder U, Hazim W, Haase W, Mautner VF. Long term outcome of gliomas of the visual pathway in type 1 neurofibromatosis. Klin Monatsbl Augenheilkd 1999:215;349-354.

23. Virdis R, Sigorini M, Laiolo A, Lorenzetti E, Street ME, Villani AR, et al. Neurofibromatosis type 1 and precocious puberty. J Pediatr Endocrinol Metab 2000;13 (Suppl 11):841-844.

24. Habiby R, Silverman B, Listernick R, Charrow J. Precocious puberty in children with neurofibromatosis type 1. J Pediatr 1995;126:364-367.

25. Janss AJ, Grundy R, Cnaan A, Savino PJ, Packer RJ, Zackai EH, Goldwein JW, et al. Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with a 6-year follow-up.

Cancer 1995;75:1051-1059.

26. Wright JE, McNab AA, McDonald WI. Optic nerve glioma and the management of optic nerve tumors in the young. Br J Ophthalmol 1989;73:967-974.

27. Astrup J. Natural history and clinical management of optic pathway glioma. Br J Neurosurg 2003;17: 327-335.

28. Ng YT, North KN. Visual-evoked potentials in the assessment of optic gliomas. Pediatr Neurol 2001; 24:44-48.

29. Iannaccone A, McCluney RA, Brewer VR, Spiegel

30.

31.

32.

33.

34.

35.

36.

37.

38.

39.

40.

41.

42.

43.

44.

45.

PH, Taylor JS, Kerr NC, et al. Visual evoked potentials in children with neurofibromatosis type 1. Doc Ophthalmol 2002;105:63-81.

Pierce SM, Barnes PD, Loefler JS, et al. Definitive radiation therapy in the management of symptomatic patients with optic glioma. Survival and long-term effects. Cancer 1990;65:45-52.

Sutton LN, Molloy PT, Sernyak H, Goldwein J, Phillips PL, Rorke LB, et al. Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery. J Neurosurg 1995;83:583-589.

Garvey M, Packer RJ. An integrated approach to the treatment of chiasmatic-hypothalamic gliomas. J Neurooncol 1996;28:167-183.

Johnston M, editor. Neurobase. La Jolla, CA: Arbor Publishing; 2001.

Packer RJ, Ater J, Allen J, Phillips P, Geyer R, Nicholson HS, et al. Carboplatin and vincristine chemotherapy for children with newly diagnosed progressive low-grade gliomas. J Neurosurg 1997;86:747-754.

Listernick R, Charrow J, Tomita T, Goldman S. Carboplatin therapy for optic pathway tumors in children with neurofibromatosis type-1. J Neurooncol 1999;45:185-190.

Mahoney DH Jr, Cohen ME, Friedman HS, Kepner JL, Gemer L, Langston JW, et al. Carboplatin is effective therapy for young children with progressive optic pathway tumors: a Pediatric Oncology Group phase II study. Neuro-oncol 2000;2:213-220.

Allen JC. Initial management of children with hypothalamic and thalamic tumors and the modifying role of neurofibromatosis-1. Pediatr Neurosurg 2000;32:154-162.

Lacaze E, Kieffer V, Streri A, Lorenzi C, Gentaz E, Habrand JL, et al. Neuropsychological outcome in children with optic pathway tumors when first-line treatment is chemotherapy. Br J Cancer 2003;89: 2038-2044.

Molloy PT, Bilaniuk LT, Vaughan SN, Needle MN, Liu GT, Zackai EH, et al. Brainstem tumors in patients with neurofibromatosis type 1: A distinct clinical entity. Neurology 1995;45:1897-1902.

Pollack IF, Shultz B, Mulvihill JJ. The management of brainstem gliomas in patients with neurofibromatosis 1. Neurology 1996;46:1652-1660.

Bilaniuk LT, Molloy PT, Zimmerman RA, Phillips PC, Vaughan SN, Liu GT, et al. Neurofibromatosis type 1: brain stem tumors. Neuroradiology 1997;39:642-653.

Kedar A. Chemotherapy for pediatric brain tumors.

Semin Pediatr Neurol 1997;4:320-332.

Van Es S, North KN, McHugh K, De Silva M. MRI findings in children with neurofibromatosis type 1: a prospective study. Pediatr Radiol 1996;26:478-487.

DeBella K, Poskitt K, Szudek J, Friedman JM. Use of “unidentified bright objects” on MRI diagnosis of neurofibromatosis 1 in children. Neurology 2000; 54:1646-1650.

North K, Joy P, Yuille D, Cocks N, Mobbs E, Hutchins P, et al. Specific learning disability in children with ¶·È‰È·ÙÚÈ΋ 2006;69:178-186

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™ÂÏ›‰·186

186

¢.π. ∑·ÊÂÈÚ›Ô˘ Î·È Û˘Ó.

neurofibromatosis type 1: significance of MRI abnormalities. Neurology 1994;44:878-883.

46. Feldmann R, Denecke J, Grenzebach M, Schuierer G, Weglage J. Neurofibromatosis type 1: motor and cognitive function and T2-weighted MRI hyperintensities. Neurology 2003;61;1725-1728.

47. Moore BD 3rd, Slopis JM, Jackson EF, De Winter AE, Leeds NE. Brain volume in children with neurofibromatosis type 1. Neurology 2000;54:914-920.

48. Hyman SL, Gill DS, Shores EA, Steinberg A, Joy P, Gibikote SV, et al. Natural history of cognitive deficits and their relationship to MRI T2-hyperintensities in NF1. Neurology 2003;60:1139-1145.

¶·È‰È·ÙÚÈ΋ 2006;69:178-186

49. Friedman JM, Birch P. An association between optic glioma and other tumors of the central nervous system in neurofibromatosis type 1. Neuropediatrics 1997;28:131-132.

50. Friedman JM, Birch PH. Type 1 neurofibromatosis: a descriptive analysis of the disorder in 1,728 patients. Am J Med Genet. 1997 May 16;70:138-143.

51. Gutmann DH, Rasmussen SA, Wolkenstein P, MacCollin MM, Guha A, Inskip PD, et al. Gliomas presenting after age 10 in individuals with neurofibromatosis type 1 (NF1). Neurology 2002;59: 759-761.

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187

√˙҉˘ ÛÎÏ‹Ú˘ÓÛË: ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ÓfiÛÔ˜ ÌÂ Û˘Ó‹ıˆ˜ ‰˘ÛÌÂÓ‹ ÚfiÁÓˆÛË ∫. ¶ÔÏ˘Ì¤ÚË, ∫. ∆ÛÔ˘Ì¿Î·˜

¶ÂÚ›ÏË„Ë: ∏ √˙҉˘ ™ÎÏ‹Ú˘ÓÛË (√™) Â›Ó·È ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹ ÌÂÙ·‚È‚·˙fiÌÂÓË Ì ÙÔÓ ·˘ÙfiÛˆÌÔ ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú· Î·È Ô˘ ÚÔηÏ› ÙÔÓ Û¯ËÌ·ÙÈÛÌfi ηÏÔ‹ıˆÓ fiÁÎˆÓ ÛÙÔÓ ÂÁΤʷÏÔ, ÛÙ· Ì¿ÙÈ·, ÛÙËÓ Î·Ú‰È¿, ÛÙÔ˘˜ ÓÂÊÚÔ‡˜, ÛÙÔ ‰¤ÚÌ·, ÛÙÔ˘˜ Ó‡ÌÔÓ˜ Î·È Û ¿ÏÏ· fiÚÁ·Ó·. ∏ ÎÏËÚÔÓÔÌÈ΋ ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ ·Ó¤Ú¯ÂÙ·È ÛÙÔ 30-40% Î·È ÙÔ ˘fiÏÔÈÔ ÚÔ¤Ú¯ÂÙ·È ·fi Ӥ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ˘‡ı˘Ó· ÁÔÓ›‰È·. √È ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ Â›Ó·È ÔÏÏ·Ϥ˜ Î·È ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙÔ Î·Ú‰È·Îfi Ú·‚‰Ô̇ˆÌ·, ÙÔ˘˜ fi˙Ô˘˜ ÛÙÔÓ ÊÏÔÈfi ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, Ù· ˘ÔÂÂÓ‰˘Ì·ÙÈο Ô˙›‰È· Î·È ÙÔ ˘ÔÂÂÓ‰˘Ì·ÙÈÎfi ÁÈÁ·ÓÙÔ΢ÙÙ·ÚÈÎfi ·ÛÙÚÔ·Ùو̷ ÛÙÔÓ ÂÁΤʷÏÔ, ÙË ÏÂÌÊ·ÁÁÂÈÔÏÂÈÔÌ˘ÔÌ¿ÙˆÛË, ÙËÓ ÔÏ˘ÂÛÙȷ΋ ÌÈÎÚÔÔ˙Ò‰Ë Ó¢ÌÔ΢ÙÙ·ÚÈ΋ ˘ÂÚÏ·Û›· Î·È ÙÔ˘˜ “ηı·ÚÔ‡˜” ΢ÙÙ·ÚÈÎÔ‡˜ fiÁÎÔ˘˜ ÛÙÔ˘˜ Ó‡ÌÔÓ˜, ÙÔ Î·ÏfiËı˜ ·ÁÁÂÈÔÌ˘ÔÏ›ˆÌ·, ÙȘ ·ÛÙÂȘ, ÙÔ Î·ÎfiËı˜ ·ÁÁÂÈÔÌ˘ÔÏ›ˆÌ·, ÙÔ ÔÁÎÔ·Ùو̷ Î·È ÙÔ Î·ÚΛӈ̷ ÙˆÓ ÓÂÊÚÒÓ, ÙȘ ˘ÔÌÂÏ·ÓˆÙÈΤ˜ ÎËÏ›‰Â˜, ÙȘ ϿΘ ÎÔÏÏ·ÁfiÓÔ˘, Ù· ÂÚÈÔÓ‡¯È· Î·È ˘ÔÓ‡¯È· ÈÓÒÌ·Ù·, Ù· ·ÁÁÂÈÔ˚ÓÒÌ·Ù· Î·È ÙËÓ ÈÓÒ‰Ë Ͽη ÙÔ˘ ÌÂÙÒÔ˘ ÛÙÔ ‰¤ÚÌ· Î·È Ù· ·Ì·ÚÙÒÌ·Ù· ÛÙÔÓ ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹. ∂ÈϤÔÓ, Â›Ó·È ÂÌÊ·Ó›˜ ÔÈ ‰È·Ù·Ú·¯¤˜ Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜ Î·È ÔÈ „˘¯È·ÙÚÈΤ˜ ÂΉËÏÒÛÂȘ, fiˆ˜ Ë ÂÈıÂÙÈÎfiÙËÙ·, Ë ˘ÂÚÎÈÓËÙÈÎfiÙËÙ·, Ô ·˘ÙÈÛÌfi˜, Ë ¤ÏÏÂÈ„Ë Û˘ÁΤÓÙÚˆÛ˘, Ë Í·ÊÓÈ΋ ÔÚÁ‹, Ë ·ıËÙÈ΋-ÂÓÛÙÈÎÙ҉˘ Û˘ÌÂÚÈÊÔÚ¿ Î·È ÔÈ ÂÈÏËÙÈΤ˜ ÎÚ›ÛÂȘ. ∏ ‰È¿ÁÓˆÛË Ù˘ √™ ‚·Û›˙ÂÙ·È ÛÙÔÓ Û˘Ó‰˘·ÛÌfi ÔÚÈÛÌ¤ÓˆÓ ÌÂÈ˙fiÓˆÓ Î·È ÂÏ·ÛÛfiÓˆÓ ÎÚÈÙËÚ›ˆÓ. ∏ ·ÓÙÈÌÂÙÒÈÛË ·ÚÔ˘ÛÈ¿˙ÂÈ ÛËÌ·ÓÙÈΤ˜ ‰˘ÛÎÔϛ˜ Î·È ÂÍ·ÙÔÌÈ·ÂÙ·È ·Ó·ÏfiÁˆ˜ ÙˆÓ ÂÓÙÔ›ÛÂˆÓ Ù˘ ÓfiÛÔ˘, Ë ÚfiÁÓˆÛË Â›Ó·È ÂÈÊ˘Ï·ÎÙÈ΋, ÂÓÒ Ë ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋ ÌÔÚ› Ó· ‚ÔËı‹ÛÂÈ Ô˘ÛÈ·ÛÙÈο ÛÙËÓ ÚfiÏË„Ë Ù˘ ÓfiÛÔ˘.

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∆Ì‹Ì·ÙÔ˜ ¡ÔÛËÏ¢ÙÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ AÏÏËÏÔÁÚ·Ê›·: ∫. ∆ÛÔ˘Ì¿Î·˜ ¢. ªfiÛ¯· 8, ∆.∫. 151 24 ª·ÚÔ‡ÛÈ, ∞ı‹Ó· ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 11-05-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 31-01-2006

§¤ÍÂȘ ÎÏÂȉȿ: √˙҉˘ ÛÎÏ‹Ú˘ÓÛË, ÂÈÏË„›·, ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË, ·˘ÙÈÛÌfi˜, fi˙ÔÈ, ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋.

Tuberous sclerosis complex: a multisystem disease with adverse prognosis C. Polimeri, C. Tsoumakas

Abstract: Tuberous sclerosis complex (TSC) is a genetic disorder transmitted by autosomal dominant inheritance. It causes benign tumours to form in many organs, primarily in the brain, eyes, heart, kidney, skin and lungs. Approximately 30-40% of people with TSC inherit it from a parent who also has TSC. The remaining 60-70% of TSC patients have no family history of the disease and appear to represent new mutations. The multitude of clinical manifestations of TSC include: cardiac rhabdomyoma, cortical tubers, subependymal nodules and subependymal giant cell astrocytoma in the brain; lymphangioleiomyomatosis, multifocal micronodular pneumocyte hyperplasia and clear cell tumour in the lungs; benign angiomyolipoma, cysts, malignant angiomyolipoma, oncocytoma and renal cell carcinoma in the kidneys; hypomelanotic macules, shagreen patches, periungual or subungual fibroma, facial angiofibroma of the skin; retinal hamartoma in the eyes. The psychiatric and behavioural manifestations include aggression, sudden rage, hyperactivity, attention deficit, acting out, obsessive-compulsive behaviour, autism, epilepsy, mental retardation and others. The treatment of this disease presents many difficulties and is individualized according to localization of the manifestations of the disease. The prognosis is doubtful. Genetic counselling is very important in this devastating disease with high penetrance, particularly because of the broad spectrum and variability of manifestations.

Pediatric Clinic, Nursing Department, University of Athens Correspondence: C. Tsoumakas 8, D. Moscha Str., 151 24 Maroussi, Athens, Greece Date of submission: 11-05-2005 Date of approval: 31-01-2006

Key words: ∆uberous sclerosis complex, epilepsy, mental retardation, autism, tubers, genetic counselling

¶·È‰È·ÙÚÈ΋ 2006;69:187-192

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∫. ¶ÔÏ˘Ì¤ÚË, ∫. ∆ÛÔ˘Ì¿Î·˜

∂ÈÛ·ÁˆÁ‹ ∏ √˙҉˘ ™ÎÏ‹Ú˘ÓÛË (√™) Â›Ó·È ·˘ÙfiÛˆÌË ÂÈÎÚ·Ùo‡Û· ÁÂÓÂÙÈο ÌÂÙ·‚È‚·˙fiÌÂÓË ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ‰È·Ù·Ú·¯‹, Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÂÎÙÂٷ̤ӷ ·Ì·ÚÙÒÌ·Ù· Û ۯ‰fiÓ fiÏ· Ù· fiÚÁ·Ó· Î·È Î˘ÚÈfiÙÂÚ˜ ÂÓÙÔ›ÛÂȘ ÛÙÔÓ ÂÁΤʷÏÔ, ÛÙÔ ‰¤ÚÌ·, ÛÙÔ˘˜ ÓÂÊÚÔ‡˜, ÛÙÔ ‹·Ú, ÛÙ· Ì¿ÙÈ· Î·È ÛÙËÓ Î·Ú‰È¿ (1). °ÂÓÈο Â›Ó·È ÂÈÎÚ·Ù¤ÛÙÂÚÔ˜ Ô fiÚÔ˜ ™‡Ó‰ÚÔÌÔ √˙Ò‰Ô˘˜ ™ÎÏ‹Ú˘ÓÛ˘, ÁÈ·Ù› ÙÔÓ›˙ÂÈ ÙËÓ ÚÔÛ‚ÔÏ‹ ÔÏÏÒÓ ÔÚÁ¿ÓˆÓ, ÂÓÒ Ô fiÚÔ˜ “Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË ÙˆÓ ÂÁÎÂÊ·ÏÈÎÒÓ Û·ÛÌÒÓ” ÚÔÙ¿ıËΠ·fi ÙÔÓ DesireMagloire Bourneville ÙÔ 1880, fiÙ·Ó ÂÚȤÁÚ·„ ÙËÓ ˘ÂÚÙÚÔÊ›· ÙˆÓ ÂÏ›ÎˆÓ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ (2).

∂ȉËÌÈÔÏÔÁÈο ÛÙÔȯ›· √ ÂÈÔÏ·ÛÌfi˜ Ù˘ ÓfiÛÔ˘ Â›Ó·È ¿ÁÓˆÛÙÔ˜, ·ÏÏ¿ ÔÈ ÂÈÙÒÛÂȘ Ù˘ ÂÎÙÈÌÒÓÙ·È Û 1 ¿ÙÔÌÔ ·Ó¿ 6.000 ÁÂÓÓ‹ÛÂȘ, ÂÓÒ Ë Û˘¯ÓfiÙËÙ· ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi, Û 1 ÛÙ· 10.000 ¿ÙÔÌ·. ∏ ÂÚÌËÓ›· ÙˆÓ ·Ó·ÏÔÁÈÒÓ ·˘ÙÒÓ Â›Ó·È fiÙÈ ÓÔÛÔ‡Ó ÂÚ›Ô˘ 500.000 ¿ÙÔÌ· ÛÙȘ ∏.¶.∞. Î·È ÂÚÈÛÛfiÙÂÚÔ ·fi 1.000.000 ·ÁÎÔÛÌ›ˆ˜ (1,3). ∞Ó ÏËÊıÔ‡Ó ˘’ fi„ÈÓ ÔÈ ‰˘ÛÎÔϛ˜ ÛÙË ‰È¿ÁÓˆÛË Î·È ÔÈ ·Û˘Ìو̷ÙÈÎÔ› ·ÛıÂÓ›˜, Ë Û˘¯ÓfiÙËÙ· Ú¤ÂÈ Ó· Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË (4). √ ·ÚÈıÌfi˜ ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ¿Û¯Ô˘Ó ·fi ÙËÓ ÎÏËÚÔÓÔÌÈ΋ ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ Â›Ó·È ÙÔ 30-40%, ÂÓÒ ÙÔ ˘fiÏÔÈÔ 6070% ÚÔ¤Ú¯ÂÙ·È ·fi Ӥ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ˘‡ı˘Ó· ÁÔÓ›‰È·. ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ˘Ô‰ËÏÒÓÂÈ ˘„ËÏÔ‡˜ Ú˘ıÌÔ‡˜ ÌÂÙ·ÏÏ·ÎÙÈÎfiÙËÙ·˜ (4).

°ÂÓÂÙÈ΋ Ù˘ Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘ ∏ ·ÈÙ›· Ù˘ √™ Â›Ó·È ÔÈ ÌÂÙ·ÏÏ¿ÍÂȘ Û ‰‡Ô ‰È·ÊÔÚÂÙÈο ÁÔÓ›‰È·, ÙÔ TSC1 ÛÙÔ 9q34 ¯ÚˆÌfiۈ̷ Î·È ÙÔ TSC2 ÛÙÔ 16p13.3 ¯ÚˆÌfiۈ̷ (4). ∆Ô TSC1 ¤¯ÂÈ 23 ÂÍfiÓÈ·, Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ÌÈ· ÚˆÙ½ÓË 1.164 ·ÌÈÓÔͤˆÓ Ô˘ ÔÓÔÌ¿˙ÂÙ·È “·Ì·ÚÙ›ÓË”, ÂÓÒ ÙÔ TSC2 ¤¯ÂÈ 42 ÂÍfiÓÈ·, Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ÌÈ· ÚˆÙ½ÓË 1.807 ·ÌÈÓÔͤˆÓ Ô˘ ηÏÂ›Ù·È “Ô˙ˆ‰›ÓË” (5). ¢˘ÛÙ˘¯Ò˜, ̤¯ÚÈ Û‹ÌÂÚ·, Ô ÚÔÁÂÓÓËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· ÙË ÓfiÛÔ ·ÚÔ˘ÛÈ¿˙ÂÈ ÔÏϤ˜ ‰˘ÛÎÔϛ˜ (6,7). ¶ÚfiÛÊ·Ù· ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ ÔÈ ÚˆÙ½Ó˜ ·Ì·ÚÙ›ÓË Î·È Ô˙ˆ‰›ÓË, ¤¯Ô˘Ó ·Ó·ÛÙ·ÏÙÈ΋ ‰Ú¿ÛË ÛÙËÓ Ô‰fi mTOR (mammalian Target of Rapamycin), Ë ÔÔ›· ÂËÚ¿˙ÂÈ ÙËÓ ·‡ÍËÛË Î·È ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ Î˘ÙÙ¿ÚˆÓ Î·È fiÙÈ Ë ¯ÔÚ‹ÁËÛË ÙÔ˘ ¶·È‰È·ÙÚÈ΋ 2006;69:187-192

·ÓÔÛÔηٷÛÙ·ÏÙÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ Rapamycin Û ·ÛıÂÓ›˜ Ì √™ ·Ó·ÛÙ¤ÏÏÂÈ ÙËÓ ·‡ÍËÛË ·ÁÁÂÈÔÌ˘ÔÏÈˆÌ¿ÙˆÓ ÛÙÔ˘˜ ÓÂÊÚÔ‡˜ (6,7). ∆Ô 50% ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÏËÚÔÓÔÌÈ΋ ÌÔÚÊ‹ Ù˘ √™ ÂÎÙÈÌ¿Ù·È fiÙÈ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔ ÁÔÓ›‰ÈÔ TSC1 Î·È ÙÔ ˘fiÏÔÈÔ 50% Ì ÙÔ ÁÔÓ›‰ÈÔ TSC2 (4). ∏ ÁÂÓÂÙÈ΋ ı¤ÛË ÙÔ˘ TSC1 ÁÔÓȉ›Ô˘ ·Ó·ÁÓˆÚ›ÛÙËΠÙÔ 1987, ·ÏÏ¿ ¯ÚÂÈ¿ÛıËÎ·Ó Û¯Â‰fiÓ 10 ¯ÚfiÓÈ· Ó· ·Ó·ÁÓˆÚÈÛÙ› ÙÔ TSC1 ÁÔÓ›‰ÈÔ (8,9). ∆Ô ÁÔÓ›‰ÈÔ TSC2 ·ÔÌÔÓÒıËΠÙÔÓ ¢ÂΤ̂ÚÈÔ ÙÔ˘ 1993, ‚Ú›ÛÎÂÙ·È ÎÔÓÙ¿ ÛÙÔ ÁÔÓ›‰ÈÔ PKD1 Ô˘ Â›Ó·È ˘‡ı˘ÓÔ ÁÈ· ÙËÓ ÔÏ˘Î˘ÛÙÈ΋ ÓfiÛÔ ÙˆÓ ÓÂÊÚÒÓ Î·È Ë ÚˆÙ½ÓË Ô˙ˆ‰›ÓË, Ô˘ ·Ú¿ÁÂÙ·È ·fi ·˘Ùfi, ÂÓ‰¤¯ÂÙ·È Ó· Û˘ÌÌÂÙ¤¯ÂÈ ÛÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ Ú‡ıÌÈÛ˘ ÙÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡, Ù˘ ‰È·ÊÔÚÔÔ›ËÛ˘ ‹ ÙˆÓ ÂÎÎÚÈÙÈÎÒÓ Ì˯·ÓÈÛÌÒÓ ÙÔ˘ ΢ÙÙ¿ÚÔ˘, ‰ÈfiÙÈ ÂÓÙÔ›˙ÂÙ·È ÛÙÔ Û‡ÛÙËÌ· Golgi Î·È Û ÈÛÙÔ‡˜ Ì ÏÔ‡ÛÈ· ·ÈÌ¿ÙˆÛË (10-14).

∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ∏ √™ ·Ó·Ê¤ÚÂÙ·È ÁÈ· ÚÒÙË ÊÔÚ¿ ÙÔ 1880, ˆ˜ ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹ Ë ÔÔ›· ÂÌÊ·Ó›˙ÂÙ·È Û ÔÏÏ¿ fiÚÁ·Ó· ÙÔ˘ ·ÓıÚÒÈÓÔ˘ ÛÒÌ·ÙÔ˜. ∏ ÓfiÛÔ˜ Â›Ó·È ÁÓˆÛÙ‹ Î·È ˆ˜ ÓfiÛÔ˜ ÙÔ˘ Bourneville Î·È ÚÔηÏ› ΢ڛˆ˜ fiÁÎÔ˘˜ ÛÙÔ ‰¤ÚÌ·, ÛÙ· ÓÂÊÚ¿, ÛÙÔÓ ÂÁΤʷÏÔ, ÛÙËÓ Î·Ú‰È¿, ÛÙÔ˘˜ Ó‡ÌÔÓ˜ Î·È ÛÙ· ‰fiÓÙÈ·, ÂËÚ¿˙ÔÓÙ·˜ ·ÎÔÏÔ‡ıˆ˜ Î·È ¿ÏÏ· ÔÚÁ·ÓÈο Û˘ÛÙ‹Ì·Ù· (3,4,15). √È ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ Ù˘ ÓfiÛÔ˘ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ÓˆÚ›˜ ‹ Î·È ·ÚÁfiÙÂÚ· ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ˙ˆ‹˜.

1) ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·fi ÙËÓ Î·Ú‰È¿ ∏ ηډȿ ÌÔÚ› Ó· ÚÔÛ‚ÏËı› ·fi ¤Ó·Ó Ù‡Ô ·Ì·ÚÙÒÌ·ÙÔ˜, ÙÔ Ú·‚‰Ô̇ˆÌ·, Ô˘ ÂÌÊ·Ó›˙ÂÙ·È Î˘Ú›ˆ˜ ηٿ ÙË ‚ÚÂÊÈ΋ Î·È ·È‰È΋ ËÏÈΛ·, ÂÓÒ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ Û˘¯ÓfiÙÂÚÔ Â›Ó·È ÙÔ Ì‡ÍˆÌ·. ∏ ‰È¿ÁÓˆÛË Á›ÓÂÙ·È Ì ˘ÂÚ˯ÔÁÚ¿ÊËÌ· Î·È ÔÈ ÂÈÙÒÛÂȘ Î˘Ì·›ÓÔÓÙ·È ÛÙÔ 47-67%. ™ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ ÔÈ Î·Ú‰È·ÎÔ› fiÁÎÔÈ Â›Ó·È ÌÂÁ¿ÏÔÈ Î·Ù¿ ÙË Á¤ÓÓËÛË, Ì Èı·ÓfiÙËÙ· Û˘ÚÚ›ÎÓˆÛ˘ ηٿ ÙËÓ ·Ó¿Ù˘ÍË (15,16). ∆· ÛËÌ›· Î·È Û˘ÌÙÒÌ·Ù· ÂÍ·ÚÙÒÓÙ·È ·fi ÙËÓ ËÏÈΛ· ÙÔ˘ ·ÛıÂÓÔ‡˜, ÙÔÓ ·ÚÈıÌfi, ÙÔ Ì¤ÁÂıÔ˜, ÙËÓ ÂÓÙfiÈÛË (ÂÓ‰ÔÎÔÈÏÔÙÈÎfi ‹ ÂÓ‰ÔÌ˘ÔηډȷÎfi) Î·È ÙÔÓ ‚·ıÌfi ·Ó¿Ù˘Í˘ ÙÔ˘ fiÁÎÔ˘ (15,16).

2) ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·fi ÙÔÓ ÂÁΤʷÏÔ Œ¯Ô˘Ó ‰È·ÈÛÙˆı› ·ÚÎÂÙÔ› Ù‡ÔÈ ÂÁÎÂÊ·ÏÈÎÒÓ ‚Ï·‚ÒÓ Û ¿ÙÔÌ· Ì √™ (4).

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·189

189

√˙҉˘ ÛÎÏ‹Ú˘ÓÛË

·) ºÏÔÈÒ‰ÂȘ fi˙ÔÈ: Â›Ó·È ÌÈÎÚ¤˜ ÂÚÈÔ¯¤˜ ÛÙÔÓ ÊÏÔÈfi ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Ô˘ ‰ÂÓ ·Ó·Ù‡ÛÛÔÓÙ·È ÔÌ·Ï¿ Ì ·ÔÙ¤ÏÂÛÌ· Ó· ·Ú·ÎˆÏ‡ÂÙ·È Ë Ê˘ÛÈÔÏÔÁÈ΋ ÌÂÙ·ÊÔÚ¿ “ÛËÌ¿ÙˆÓ” ÛÙÔÓ ÂÁΤʷÏÔ ÚÔηÏÒÓÙ·˜ Û·ÛÌÔ‡˜. √ ·ÚÈıÌfi˜ Î·È Ë ÂÚÈÔ¯‹ ·Ó¿Ù˘Í‹˜ ÙÔ˘˜ ¢ı‡ÓÔÓÙ·È ÁÈ· ÙËÓ ÔÈÎÈÏÔÌÔÚÊ›· ÙˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ ÂΉËÏÒÛˆÓ. ∆· Û˘ÌÙÒÌ·Ù· ÙˆÓ ÊÏÔȈ‰ÒÓ fi˙ˆÓ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙÔ˘˜ Û·ÛÌÔ‡˜, ÙË ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË, ÙȘ Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜, ÙȘ ‰È·Ù·Ú·¯¤˜ Ù˘ ÚÔÛÔ¯‹˜, ÙËÓ ˘ÂÚÎÈÓËÙÈÎfiÙËÙ· Î·È ÙÔÓ ·˘ÙÈÛÌfi (4). ‚) ÀÔÂÂÓ‰˘Ì·ÙÈο Ô˙›‰È·: ÂÓÙÔ›˙ÔÓÙ·È ÛÙ· ÙÔȯÒÌ·Ù· ÙˆÓ Ï¿ÁÈˆÓ ÎÔÈÏÈÒÓ, Û˘ÛÛˆÚÂ‡Ô˘Ó ·Û‚¤ÛÙÈÔ Î·Ù¿ ÙËÓ ÂÚÈÁÂÓÓËÙÈ΋ ËÏÈΛ· Î·È ÌÔÚÔ‡Ó Ó· ‰È·ÁÓˆÛıÔ‡Ó Ì ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· (4). Á) ÀÔÂÂÓ‰˘Ì·ÙÈÎfi ÁÈÁ·ÓÙÔ΢ÙÙ·ÚÈÎfi ·ÛÙÚÔ·Ùو̷: ηÏÔ‹ı˘ fiÁÎÔ˜ Ô˘ ·Ó·Ù‡ÛÛÂÙ·È ÛÙÔ 15% ÂÚ›Ô˘ ÙˆÓ ·ÙfiÌˆÓ Ì √™. ¢ÂÓ ·Ú·ÙËÚÂ›Ù·È Û ÌÈÎÚ¿ ·È‰È¿. ™Â ÔÚÈṲ̂Ó˜ ÂÚÈÙÒÛÂȘ Ô fiÁÎÔ˜ ÌÔÚ› Ó· Â›Ó·È ÂÈıÂÙÈÎfi˜ ÚÔηÏÒÓÙ·˜ Ó¢ÚÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜. ™˘ÌÙÒÌ·Ù· fiˆ˜ ¤ÌÂÙÔÈ, Ó·˘Ù›·, ÔÓÔΤʷÏÔÈ, ηıÒ˜ Î·È ·ÏÏ·Á¤˜ ÛÙËÓ fiÚÂÍË, ÙË Û˘ÌÂÚÈÊÔÚ¿ Î·È ÙË ‰È¿ıÂÛË Ú¤ÂÈ Ó· ·ÍÈÔÏÔÁÔ‡ÓÙ·È, ÁÈ·Ù› ÌÔÚ› Ó· ˘Ô‰ËÏÒÓÔ˘Ó ·‡ÍËÛË ÙÔ˘ fiÁÎÔ˘ (3,4,6,7,15).

3) ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·fi ÙÔ˘˜ ÓÂÊÚÔ‡˜ ·) ∫·ÏfiËı˜ ·ÁÁÂÈÔÌ˘ÔÏ›ˆÌ·: ∏ ÈÔ Û˘¯Ó‹ ‚Ï¿‚Ë Ù˘ √™ ÙˆÓ ÌÂÁ·Ï‡ÙÂÚˆÓ ·È‰ÈÒÓ Î·È ÂÓËϛΈÓ. √ fiÁÎÔ˜ ÌÔÏÔÓfiÙÈ Â›Ó·È Î·ÏÔ‹ı˘, ÂÌÊ·Ó›˙ÂÈ ÙÔÈ΋ ‰È‹ıËÛË Ô˘ Â›Ó·È ‰˘Ó·Ùfi Ó· ÂÎÙ›ÓÂÙ·È ÛÙÔ ÂÚÈÓÂÊÚÈÎfi Ï›Ô˜, Û·ÓÈfiÙÂÚ· ÛÙ· ·ıÚÔÈÛÙÈο ÛˆÏËÓ¿ÚÈ·, ÛÙȘ ÓÂÊÚÈΤ˜ ÊϤ‚˜, ·ÎfiÌ· Î·È ÛÙËÓ Î¿Ùˆ ÎÔ›ÏË ÊϤ‚· Î·È ÛÙÔÓ ‰ÂÍÈfi ÎfiÏÔ (17-19). ™˘Ó‹ıˆ˜ ¤¯ÂÈ ÔÏÏ·Ï‹ Î·È ÂÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË Î·È ÔÈ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÚÔÛ‰ÈÔÚ›˙ÔÓÙ·È ·fi ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ÂΉËÏÒÛÂÈ ·ÈÌÔÚÚ·Á›· Î·È ·fi ÙÔ Ì¤ÁÂıÔ˜ Ù˘ Ì¿˙·˜, ÂÓÒ Ë ‰È¿ÁÓˆÛË Â›Ó·È ‰‡ÛÎÔÏË Î·È Á›ÓÂÙ·È Ì ˘ÂÚ˯ÔÁÚ¿ÊËÌ·, ·ÍÔÓÈ΋ ‹ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· (20-23). ‚) ¡ÂÊÚÈΤ˜ ·ÛÙÂȘ: ™˘Ó‹ıˆ˜ Â›Ó·È ÔÏÏ·Ϥ˜ Î·È ·ÌÊÔÙÂÚfiÏ¢Ú˜, ·ÔÙÂÏÔ‡Ó ÙË ‰Â‡ÙÂÚË ÈÔ Û˘¯Ó‹ ÂΉ‹ÏˆÛË Ù˘ ÓfiÛÔ˘ (6,18,21). ∆· ΢ÚÈfiÙÂÚ· ÂÓԯϋ̷ٷ Â›Ó·È ·ÚÔÛ‰ÈfiÚÈÛÙ· ÎÔÈÏȷο ¿ÏÁË, ÔÛÊ˘·ÏÁ›·, Ó·˘Ù›·, ¤ÌÂÙÔÈ, ˘ÚÂÙfi˜, Ì·ÎÚÔÛÎÔÈ΋ ·ÈÌ·ÙÔ˘Ú›· (6,18,21). ¶ÚÔԉ¢ÙÈ΋ ÓÂÊÚÈ΋ ·ÓÂ¿ÚÎÂÈ· Î·È ˘¤ÚÙ·ÛË ÌÔÚ› Ó· ÂÌÊ·ÓÈÛıÔ‡Ó ·ÚÁfiÙÂÚ· (7,15,21).

Á) ∫·ÎfiËı˜ ·ÁÁÂÈÔÌ˘ÔÏ›ˆÌ·: ÕÙÔÌ· Ì √™ Î·È ÓÂÊÚÈÎfi ·ÁÁÂÈÔÌ˘ÔÏ›ˆÌ· ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚÔ Î›Ó‰˘ÓÔ Ó· ·Ó·Ù‡ÍÔ˘Ó Î·ÎÔ‹ıÂȘ fiÁÎÔ˘˜ ÛÙÔ˘˜ ÓÂÊÚÔ‡˜. ∆· ηÎÔ‹ıË ·ÁÁÂÈÔÌ˘ÔÏÈÒÌ·Ù· Â›Ó·È ··Ú·›ÙËÙÔ Ó· ·Ê·ÈÚÔ‡ÓÙ·È ÙÔ Û˘ÓÙÔÌfiÙÂÚÔ ‰˘Ó·Ùfi (6,15,21). ‰) √ÁÎÔ·Ùو̷: ∞ÔÙÂÏ› ηÏÔ‹ıË fiÁÎÔ Ô˘ ·Ú·ÙËÚÂ›Ù·È Û ¿ÙÔÌ· Ì √™ (6,15,21). Â) ¡ÂÊÚÈÎfi ΢ÙÙ·ÚÈÎfi ηÚΛӈ̷: ∂›Ó·È Û¿ÓÈÔ Û ¿ÙÔÌ· Ì √™ (21).

4) ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·fi ÙÔ ‰¤ÚÌ· ·) ∞ÁÁÂÈÔ˚ÓÒÌ·Ù·: ∂ÌÊ·Ó›˙ÔÓÙ·È ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 4 ¤ˆ˜ 10 ÂÙÒÓ. ∞Ó¢ڛÛÎÔÓÙ·È ÛÙÔ 80-90% ÙˆÓ ·ÛıÂÓÒÓ Î·È ÂÓÙÔ›˙ÔÓÙ·È ÛÙÔ ÎÂÓÙÚÈÎfi ÙÌ‹Ì· ÙÔ˘ ÚÔÛÒÔ˘. ªÔÈ¿˙Ô˘Ó Ì ÂÚ˘ıËÌ·ÙÒ‰ÂȘ ÎËÏ›‰Â˜ Î·È ‚Ï·Ù›‰Â˜, ‰›ÓÔÓÙ·˜ ÔÏϤ˜ ÊÔÚ¤˜ ÙËÓ ÂÓÙ‡ˆÛË Ù˘ ·ÎÌ‹˜ ‹ ÌÂÁ¿ÏˆÓ Ô˙ȉ›ˆÓ. πÛÙÔÏÔÁÈο ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·˘ÍË̤ÓÔ ·ÚÈıÌfi ‰È·ÙÂÙ·ÁÌ¤ÓˆÓ ÙËÏ·ÁÁÂÈÂÎÙ·ÛÈÒÓ Î·È ÈÓÔ‚Ï·ÛÙÒÓ Ì ·Ó¿Ù˘ÍË ÙÔ˘ ·ÁÁÂÈ·ÎÔ‡ Î·È ÙÔ˘ ÈÓÒ‰Ô˘˜ ÛÙÔÈ¯Â›Ô˘ ̤۷ ÛÙËÓ ›‰È· ‚Ï¿‚Ë (6,24). ‚) ÀÔÌÂÏ·ÓˆÙÈΤ˜ ÎËÏ›‰Â˜: ∞ÓÔȯÙfi¯ÚˆÌ˜ ϿΘ ÛÙÔ ‰¤ÚÌ· ‰È·ÊfiÚÔ˘ ÌÂÁ¤ıÔ˘˜ Î·È Û¯‹Ì·ÙÔ˜. ∂ÌÊ·Ó›˙ÔÓÙ·È ·fi ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜, ·ÏÏ¿ ‰ÂÓ Â›Ó·È ¿ÓÙ· ÔÚ·Ù¤˜ ÛÙÔ ÎÔÈÓfi ʈ˜. √È ·ÛıÂÓ›˜ ÌÔÚ› Ó· ¤¯Ô˘Ó ·fi Ì›· ¤ˆ˜ Î·È ÂÚÈÛÛfiÙÂÚ˜ ·fi ‰Ò‰Âη ‚Ï¿‚˜ Ì ÔÈÎÈÏ›· ÌÂÁ¤ıÔ˘˜ (ÏÈÁfiÙÂÚÔ ·fi 1 cm ̤¯ÚÈ ÔÏÏ¿ ÂηÙÔÛÙ¿), Î·È ¤¯Ô˘Ó Û˘¯Ó¿ Û¯‹Ì· ʇÏÏÔ˘ (15,18). Á) πÓ҉˘ Ͽη ÌÂÙÒÔ˘: ∏ ÂÌÊ¿ÓÈÛ‹ Ù˘ Â›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈ΋ Î·È ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ ÛÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘. πÛÙÔÏÔÁÈο ·ÔÙÂÏÂ›Ù·È ·fi ·ÁÁÂÈÔ˚ÓÒ‰Ë ÈÛÙfi. ™Â ÔÚÈṲ̂Ó˜ ÂÚÈÙÒÛÂȘ ÔÈ ÈÓÒ‰ÂȘ ϿΘ ÌÔÚ› Ó· ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ Ì¤ÙˆÔ ‹ ÛÙÔ ÙÚȯˆÙfi Ù˘ ÎÂÊ·Ï‹˜ fiÔ˘ ÂÚÈ‚¿ÏÏÔÓÙ·È ·fi ÏÂÙ¤˜ ¿ÛÚ˜ “Ùԇʘ” Ì·ÏÏÈÒÓ (15,18). ‰) ¶Ï¿Î˜ ÎÔÏÏ·ÁfiÓÔ˘: ∂ËṲ́Ó˜ ÌÂÏ·Á¯ÚˆÌ·ÙÈΤ˜ ϿΘ ÛÙËÓ Ô›ÛıÈ· ÔÛÊ˘˚΋ ¯ÒÚ·, ÌÔÚ› Ó· ˘¿Ú¯Ô˘Ó ·fi ÙË Á¤ÓÓËÛË Î·È ·Ú·ÙËÚÔ‡ÓÙ·È ÛÙÔ 40% ÙˆÓ ·ÛıÂÓÒÓ (15,18). Â) ¶ÂÚÈÔÓ‡¯È· Î·È ˘ÔÓ‡¯È· ÈÓÒÌ·Ù·: ∂ÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 50% ÙˆÓ ·ÛıÂÓÒÓ (15,18).

5) ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·fi ÙÔ˘˜ Ó‡ÌÔÓ˜ √È Î˘ÚÈfiÙÂÚ˜ Ó¢ÌÔÓÈΤ˜ ‚Ï¿‚˜ Ô˘ ¤¯Ô˘Ó ·Ú·ÙËÚËı› ÛÙËÓ √™ Â›Ó·È Ë ÏÂÌÊ·ÁÁÂÈÔÏÂÈÔÌ˘ÔÌ¿ÙˆÛË, Ë ÔÏ˘ÂÛÙÈ΋ ÌÈÎÚÔÔ˙҉˘ Ó¢ÌÔ΢ÙÙ·ÚÈ΋ ˘ÂÚÏ·Û›· Î·È ÔÈ “ηı·ÚÔ›” ΢ÙÙ·ÚÈÎÔ› fiÁÎÔÈ ÌÂ Û˘¯ÓfiÙÂÚË ÙË ¶·È‰È·ÙÚÈ΋ 2006;69:187-192

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·190

190

∫. ¶ÔÏ˘Ì¤ÚË, ∫. ∆ÛÔ˘Ì¿Î·˜

ÏÂÌÊ·ÁÁÂÈÔÏÂÈÔÌ˘ÔÌ¿ÙˆÛË. ∆· Úfi‰ÚÔÌ· Û˘ÌÙÒÌ·Ù· Â›Ó·È Ë ‰‡ÛÓÔÈ· ÌÂÙ¿ ·fi ÂÏ·ÊÚ¿ ¿ÛÎËÛË, Ô ·˘ÙfiÌ·ÙÔ˜ Ó¢ÌÔıÒڷη˜ ‹ Ô ‚‹¯·˜, ÂÓÒ ÚÔԉ¢ÙÈο ÌÔÚ› Ó· ·Ó·Ù˘¯ı› ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ· (6,7,25). ∏ ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ Î·È Î˘Ú›ˆ˜ Ë ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó ÛÙË ‰È¿ÁÓˆÛË (15,18,25).

6) ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·fi Ù· Ì¿ÙÈ· ∏ ·ÚÈ· ÔÊı·ÏÌÔÏÔÁÈ΋ ÂΉ‹ÏˆÛË Ù˘ ÓfiÛÔ˘ Â›Ó·È Ù· ·Ì·ÚÙÒÌ·Ù· ÛÙÔÓ ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ˆ˜ ÛÙÚÔÁÁ˘Ï¤˜ ‹ ˆÔÂȉ›˜ ÂÚÈÔ¯¤˜ ÛÙËÓ ÂÚÈʤÚÂÈ· ÙÔ˘ ‚˘ıÔ‡. πÛÙÔÏÔÁÈο ÔÈ fiÁÎÔÈ ·˘ÙÔ› ·ÔÙÂÏÔ‡ÓÙ·È ·fi ˘·Ï›ÓË Ô˘Û›· Ì ÛËÌ›· ·Û‚ÂÛÙÔÔ›ËÛ˘. ∏ fiÚ·ÛË ÙˆÓ ·ÛıÂÓÒÓ Û˘Ó‹ıˆ˜ ‰ÂÓ ÂËÚ¿˙ÂÙ·È (6,15,18).

7) ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·fi ¿ÏÏ· fiÚÁ·Ó· ∫‡ÛÙÂȘ Î·È fiÁÎÔÈ ·ÚfiÌÔÈÔÈ Ì ·˘ÙÔ‡˜ Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È ÛÙÔ˘˜ ÓÂÊÚÔ‡˜ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ ‹·Ú, ÛÙÔ ¿ÁÎÚ·˜ Î·È Û ¿ÏÏ· fiÚÁ·Ó·. ™˘Ó‹ıˆ˜ Â›Ó·È ·Û˘Ìو̷ÙÈÎÔ› Î·È ‰È·ÈÛÙÒÓÔÓÙ·È Ì ÙËÓ ÂÓËÏÈΛˆÛË. ∫‡ÛÙÂȘ ·Ó·Ù‡ÛÛÔÓÙ·È Î·È ÛÙ· ÔÛÙ¿ (26). ∂ÓÙ˘ÒÌ·Ù· ÛÙ· ÓÂÔÁÈÏ¿ ·ÏÏ¿ Î·È ÛÙ· ÌfiÓÈÌ· ‰fiÓÙÈ· Â›Ó·È Û˘Ó‹ıË, ηıÒ˜ Ôχ Û˘¯Ó‹ Â›Ó·È Î·È Ë ÔÏ˘Ô‰›·ÛË ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘ (15,18).

8) æ˘¯È·ÙÚÈΤ˜ ÂΉËÏÒÛÂȘ Î·È ‰È·Ù·Ú·¯¤˜ Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜ ∏ Û˘ÌÂÚÈÊÔÚ¿ ÙÔ˘ ·È‰ÈÔ‡ Ô˘ ¿Û¯ÂÈ ·fi √™ ·ÔÙÂÏ› Û˘¯Ó¿ ÛËÌ·ÓÙÈÎfi Úfi‚ÏËÌ·. ∂ÈıÂÙÈÎfiÙËÙ·, ˘ÂÚÎÈÓËÙÈÎfiÙËÙ·, ¤ÏÏÂÈ„Ë Û˘ÁΤÓÙÚˆÛ˘, ÂÎÚ‹ÍÂȘ ı˘ÌÔ‡, ·ÚÔÚÌËÙÈ΋ Û˘ÌÂÚÈÊÔÚ¿, ·˘ÙÔÙÚ·˘Ì·ÙÈÛÌfi˜, ·ıËÙÈ΋ ÂÓÛÙÈÎÙ҉˘ Û˘ÌÂÚÈÊÔÚ¿, ÛÙÂÚÂÔÙ˘È΋ Û˘ÌÂÚÈÊÔÚ¿, ηı˘ÛÙ¤ÚËÛË ÛÙÔÓ ÏfiÁÔ, ·ÓËÛ˘¯›·, ηٿıÏÈ„Ë, ˘ÂÚ‚ÔÏÈΤ˜ ÊÔ‚›Â˜, ˘ÂÚ‚ÔÏÈ΋ Û˘ÛÙÔÏ‹, ·Ú·ÙËÚÔ‡ÓÙ·È Û ÔÏÏ¿ ·È‰È¿. ™Â ÔÚÈṲ̂Ó˜ ÂÚÈÙÒÛÂȘ ÌÔÚ› Ó· ‰È·ÁÓˆÛÙ› Û¯È˙ÔÊÚ¤ÓÂÈ·, ‰ÈÔÏÈ΋ ‰È·Ù·Ú·¯‹ (Ì·ÓÈÔηٿıÏÈ„Ë), ηٿıÏÈ„Ë, ‹ ¿ÏϘ „˘¯È·ÙÚÈΤ˜ ‰È·Ù·Ú·¯¤˜ (4,7,18). ·) ¶ÚÔ‚Ï‹Ì·Ù· ‡ÓÔ˘: ¢È·Ù·Ú·¯¤˜ fiˆ˜ ͇ÓËÌ· ÛÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜, ÚÔ‚Ï‹Ì·Ù· ‡ÓÔ˘ Û¯ÂÙÈ˙fiÌÂÓ· Ì Û·ÛÌÔ‡˜ Î·È ˘ÓËÏ›· ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ë̤ڷ˜ Ê·›ÓÂÙ·È Ó· Â›Ó·È ·fi Ù· ÈÔ ÎÔÈÓ¿ ÚÔ‚Ï‹Ì·Ù· Û˘ÌÂÚÈÊÔÚ¿˜ Û ·È‰È¿ Ì √™. ∆· ·È‰È¿ Ì Û·ÛÌÔ‡˜ Ê·›ÓÂÙ·È Ó· ¤¯Ô˘Ó ÈÔ ‰È·Û·Ṳ̂ÓÔ ‡ÓÔ ·fi Ù· ·È‰È¿ ¯ˆÚ›˜ Û·ÛÌÔ‡˜. ¶·ÚfiÏ· ¶·È‰È·ÙÚÈ΋ 2006;69:187-192

·˘Ù¿, ÔÈ ‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘ Ê·›ÓÂÙ·È Ó· ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙȘ ÂÈÏËÙÈΤ˜ ÎÚ›ÛÂȘ Ô˘ Û˘Ì‚·›ÓÔ˘Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜ (4,26,27). ‚) ∞˘ÙÈÛÌfi˜: ∂ÌÊ·Ó›˙ÂÙ·È ÚÒÈÌ· Ì ‰È·Ù·Ú·¯‹ ÛÙÔÓ ÏfiÁÔ, ÙËÓ ÂÈÎÔÈÓˆÓ›· Î·È ÙÔ ·È¯Ó›‰È (28-30). ∏ ÏÂÈÔ„ËÊ›· ÙˆÓ ·È‰ÈÒÓ Ì √™ Ô˘ ¤¯Ô˘Ó Î·È ·˘ÙÈÛÌfi ›¯·Ó ·ÚÔ˘ÛÈ¿ÛÂÈ ‚ÚÂÊÈÎÔ‡˜ Û·ÛÌÔ‡˜ Î·È ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË (31-33).

9) ∂ÈÏË„›· ™Â ÔÛÔÛÙfi 71% ÂÌÊ·Ó›˙ÂÙ·È ÚÈÓ ÙËÓ ËÏÈΛ· ÙˆÓ 2 ÂÙÒÓ Î·È Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ·ÔÙÂÏ› ÙÔ ÚÒÙÔ Î·È ÌÔÓ·‰ÈÎfi Û‡Ìو̷ Ù˘ ÓfiÛÔ˘ (34,35). √È ÂÈÏËÙÈΤ˜ ÎÚ›ÛÂȘ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ù˘ ·ÚÔ˘Û›·˜ ÙˆÓ fi˙ˆÓ ÛÙÔÓ ÂÁΤʷÏÔ Î·È Ì ‰Â‰Ô̤ÓË ÙËÓ ·ÚÔ˘Û›· ÙÔ˘˜ Û ÔÔÈÔ‰‹ÔÙ ÛËÌ›Ô, ÌÔÚ› Ó· ÚÔηϤÛÔ˘Ó fiÏÔ˘˜ ÙÔ˘˜ Ù‡Ô˘˜ ÙˆÓ ÎÚ›ÛÂˆÓ (34,35). ·) µÚÂÊÈÎÔ› Û·ÛÌÔ›: ™˘Ì‚·›ÓÔ˘Ó Û ÔÛÔÛÙfi 36-69% ·ÛıÂÓÒÓ Ì √™. ∂ÌÊ·Ó›˙ÔÓÙ·È ·fi ÙËÓ ÚÒÙË Â‚‰ÔÌ¿‰· Ù˘ ˙ˆ‹˜ Ô˘ ÔÈ Èı·ÓfiÙËÙ˜ ÁÈ· ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË ·˘Í¿ÓÔÓÙ·È. √È Û·ÛÌÔ› ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ ·Ú¯‹ ÛÙË ıÂÚ·›·, ÛÙË Û˘Ó¤¯ÂÈ· fï˜, ·Ó·Ù‡ÛÛÂÙ·È ·ÓıÂÎÙÈÎfiÙËÙ· (4,34,35). ‚) ∂ÛÙȷΤ˜ ÂÈÏËÙÈΤ˜ ÎÚ›ÛÂȘ: i) ∞Ϥ˜ ÂÛÙȷΤ˜ Ì ÎÈÓËÙÈΤ˜, ·ÈÛıËÙÈΤ˜, ‹ ·ÈÛıËÙËÚȷΤ˜. ii) ™‡ÓıÂÙ˜ ‹ Û‡ÌÏÔΘ ÂÛÙȷΤ˜, Ô˘ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ÙÒÛË ‹ ·ÒÏÂÈ· ÂÈ¤‰Ô˘ Û˘Ó›‰ËÛ˘. iii) ∂ÛÙȷΤ˜ ÎÚ›ÛÂȘ Ô˘ ÛÙË Û˘Ó¤¯ÂÈ· ÌÂÙ·›ÙÔ˘Ó Û ÁÂÓÈÎÂ˘Ì¤Ó˜ (4,34,35). Á) ∞ÛÙ·ÙÈΤ˜ Ì˘ÔÎÏÔÓÈΤ˜ ÎÚ›ÛÂȘ: ÷ڷÎÙËÚ›˙ÔÓÙ·È ·fi ·ÈÊÓ›‰È· ·ÒÏÂÈ· ÙÔ˘ Ì˘˚ÎÔ‡ ÙfiÓÔ˘ Î·È ÙÒÛË ÛÙÔ ¤‰·ÊÔ˜ Ì ÌÈÎÚ‹˜ ‰È¿ÚÎÂÈ·˜ ·ÒÏÂÈ· Û˘Ó›‰ËÛ˘ Î·È Ì˘ÔÎÏÔÓÈΤ˜ ‹ ÙÔÓÈΤ˜ ÎÚ›ÛÂȘ fiˆ˜ Î·È Û˘¯Ó¿ ÁÂÓÈÎÂ˘Ì¤ÓÔ˘˜ Û·ÛÌÔ‡˜ (4,34,35). ‰) ÕÙ˘˜ ·Ê·ÈÚ¤ÛÂȘ: ¶ÚÔÛÔÌÔÈ¿˙Ô˘Ó Ì ÙȘ Ù˘ÈΤ˜ ·Ê·ÈÚ¤ÛÂȘ, ‰È·Ê¤ÚÔ˘Ó fï˜, ÁÈ·Ù› ‰ÂÓ ÂÌÊ·Ó›˙ÔÓÙ·È Ô‡Ù ÛÙ·Ì·ÙÔ‡Ó ·ÈÊÓ›‰È· (4,34-36).

10) ¡ÔËÙÈ΋ ˘ÛÙ¤ÚËÛË ∏ Û˘¯ÓfiÙËÙ· Ù˘ ÓÔËÙÈ΋˜ ˘ÛÙ¤ÚËÛ˘ ÛÙËÓ √™ ·Ó·Ê¤ÚÂÙ·È Û ÔÛÔÛÙfi ¿Óˆ ·fi 50% ÙˆÓ ÌÂÏÂÙÒÓ. ∏ ·ıÔÁ¤ÓÂÈ¿ Ù˘ Â›Ó·È ¿ÁÓˆÛÙË, ÂÓÒ ˘¿Ú¯ÂÈ Û˘Û¯ÂÙÈÛÌfi˜ Ì ÙËÓ ËÏÈΛ· ¤Ó·Ú͢ ÙˆÓ ÂÈÏËÙÈÎÒÓ ÎÚ›ÛÂˆÓ Î·È ÙË ÛÔ‚·ÚfiÙËÙ· Ù˘ ÂÈÏË„›·˜. ∏ Â›ÙˆÛË ÙˆÓ Ì·ıËÛÈ·ÎÒÓ ‰˘ÛÎÔÏÈÒÓ ÔÈΛÏÂÈ ·fi 38-80% Î·È ÂÌÊ·Ó›˙ÂÙ·È Ó· Â›Ó·È Ì¤ÛÔ˘ ‹ ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ (4).

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¶›Ó·Î·˜ 1. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· Ù˘ √˙Ò‰Ô˘˜ ™ÎÏ‹Ú˘ÓÛ˘ Curatolo P, Verdecchia M, Bombardieri R. Tuberous sclerosis complex: a review of neurological aspects. Eur J Paediatr Neurol 2002;6:15-23. ªÂ›˙ÔÓ· ÎÚÈÙ‹ÚÈ· ∞ÁÁÂÈÔ˚ÓÒÌ·Ù· ÚÔÛÒÔ˘ ‹ ϿΘ ÛÙÔ Ì¤ÙˆÔ ªË ÙÚ·˘Ì·ÙÈο ˆÓ‡¯È· ‹ ÂÚȈӇ¯È· ÈÓÒÌ·Ù· ÀÔÌÂÏ·ÓˆÙÈΤ˜ ÎËÏ›‰Â˜ (ÙÚÂȘ ‹ ÂÚÈÛÛfiÙÂÚ˜) ¶Ï¿Î˜ ÎÔÏÏ·ÁfiÓÔ˘ ¶ÔÏÏ·Ïfi Ô˙҉˜ ·Ì¿Úو̷ ·ÌÊÈ‚ÏËÛÙÚÔÂȉԇ˜ ºÏÔÈÒ‰ÂȘ fi˙ÔÈ ÀÔÂÂÓ‰˘Ì·ÙÈÎÔ› fi˙ÔÈ ÀÔÂÂÓ‰˘Ì·ÙÈÎfi ÁÈÁ·ÓÙÔ΢ÙÙ·ÚÈÎfi ·ÛÙÚÔ·Ùو̷ ∫·Ú‰È·Îfi Ú·‚‰Ô̇ˆÌ·, ÌÔÓfi ‹ ÔÏÏ·Ïfi §ÂÌÊ·ÁÁÂÈÔÏÂÈÔÌ˘ÔÌ¿ÙˆÛË Î·È/‹ ÓÂÊÚÈÎfi ·ÁÁÂÈÔÌ˘ÔÏ›ˆÌ· ∂Ï¿ÛÛÔÓ· ÎÚÈÙ‹ÚÈ· ¶ÔÏÏ·Ï¿, Ù˘¯·›· ηٷÓÂÌË̤ӷ ÂÓÙ˘ÒÌ·Ù· ÛÙËÓ ·‰·Ì·ÓÙ›ÓË ÙˆÓ ‰ÔÓÙÈÒÓ ∞Ì·Úو̷ÙÒ‰ÂȘ Ôχԉ˜ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘ ∫‡ÛÙÂȘ ÛÙ· ÔÛÙ¿ ∂ÁÎÂÊ·ÏÈΤ˜ ·ÎÙÈÓˆÙ¤˜ ÌÂÙ·Ó·ÛÙ¢ÙÈΤ˜ ÁÚ·Ì̤˜ Ï¢ÎÔ‡ Ù‡Ô˘ (cerebral white matter radial migration lines) ÿӈ̷ Ô‡ÏˆÓ ªË ÓÂÊÚÈÎfi ·Ì¿Úو̷ Õ¯ÚˆÌË Ͽη ·ÌÊÈ‚ÏËÛÙÚÔÂȉԇ˜ ¢ÂÚÌ·ÙÈΤ˜ ‚Ï¿‚˜ Û·Ó “ÎÔÓÊÂÙ›” ¶ÔÏÏ·Ϥ˜ ÓÂÊÚÈΤ˜ ·ÛÙÂȘ

∂Ú¢ÓËÙ¤˜ ¤‰ÂÈÍ·Ó fiÙÈ fi˙ÔÈ ÛÙÔÓ ÌÂÙˆÈ·›Ô ÏÔ‚fi ÌÔÚ› Ó· ·ÔÙÂÏÔ‡Ó ‰Â›ÎÙË ÓÔËÙÈ΋˜ ·Ó¿Ù˘Í˘ (37). ŒÓ·˜ ¿ÏÏÔ˜ ηÏfi˜ ‰Â›ÎÙ˘ ÁÈ· ÙÔÓ ‚·ıÌfi Ù˘ ÓÔËÙÈ΋˜ ˘ÛÙ¤ÚËÛ˘ Ê·›ÓÂÙ·È Ó· Â›Ó·È Ô ·ÚÈıÌfi˜ ÙˆÓ ÎÚÔÙ·ÊÈÎÒÓ fi˙ˆÓ (38,39).

¢È¿ÁÓˆÛË - ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ∆· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· Ù˘ √™ ‰È·ÎÚ›ÓÔÓÙ·È Û Ì›˙ÔÓ· Î·È ÂÏ¿ÛÛÔÓ·. ¢‡Ô ·fi Ù· Ì›˙ÔÓ· ÎÚÈÙ‹ÚÈ· ‹ ¤Ó· Ì›˙ÔÓ Î·È ‰‡Ô ÂÏ¿ÛÛÔÓ· ı· Ô‰ËÁ‹ÛÔ˘Ó ÛÙË ‰È¿ÁÓˆÛË (1,4,40,41) (¶›Ó·Î·˜ 1).

£ÂÚ·¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË ∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ √™ ·ÚÔ˘ÛÈ¿˙ÂÈ ÛËÌ·ÓÙÈΤ˜ ‰˘ÛÎÔϛ˜ Î·È ÂÍ·ÙÔÌÈ·ÂÙ·È ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÓÙfiÈÛË. ∆Ô Î·Ú‰È·Îfi Ú·‚‰Ô̇ˆÌ· ·ÓÙÈÌÂÙˆ›˙ÂÙ·È ¯ÂÈÚÔ˘ÚÁÈο, ÂÓÒ Â›Ó·È ·Ó·Áη›· Î·È Ë Û˘Ìو̷ÙÈ΋ ·ÁˆÁ‹ (1,15,16). √È fiÁÎÔÈ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ÌÔÚ› Ó· ·˘ÍËıÔ‡Ó ·ÚÎÂÙ¿ Ì ·ÔÙ¤ÏÂÛÌ· Ó· ·Ú·ÎˆÏ‡Ô˘Ó ÙË ÚÔ‹ ÙÔ˘ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘ ˘ÁÚÔ‡ Î·È Ú¤ÂÈ Ó· ·Ê·È-

ÚÔ‡ÓÙ·È (3,4,6,7,15). ∆· ÓÂÊÚÈο ηÚÎÈÓÒÌ·Ù· Ú¤ÂÈ Ó· ·Ê·ÈÚÔ‡ÓÙ·È ÙÔ Û˘ÓÙÔÌfiÙÂÚÔ ‰˘Ó·Ùfi ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË, ÂÓÒ Ù· ·ÁÁÂÈÔÌ˘ÔÏÈÒÌ·Ù· Û˘Ó‹ıˆ˜ ‰ÂÓ ··ÈÙÔ‡Ó ıÂÚ·›· (6,7,15,18,21). ∏ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ‰ÂÚÌ·ÙÈÎÒÓ ·ÁÁÂÈÔ˚ÓˆÌ¿ÙˆÓ Â›Ó·È ·Ó·Áη›· ÂÍ·ÈÙ›·˜ ÙˆÓ ·Ú·ÌÔÚÊÒÛÂˆÓ Î·È Ù˘ ‰˘Û¯¤ÚÂÈ·˜ Ô˘ ÚÔηÏÔ‡Ó ÛÙËÓ fiÚ·ÛË. ∏ ·Ê·›ÚÂÛË ÙˆÓ ÂÚÈÔÓ‡¯ÈˆÓ Î·È ˘ÔÓ‡¯ÈˆÓ ÈÓˆÌ¿ÙˆÓ Á›ÓÂÙ·È ¯ÂÈÚÔ˘ÚÁÈο ‹ Ì Laser (6,7,15,18,24). ¶ÚfiÛÊ·Ù· ‰Â‰Ô̤ӷ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë Ù·ÌÔÍÈʤÓË Î·È Ë ÚÔÁÂÛÙÂÚfiÓË ¤¯Ô˘Ó ÂÓı·ÚÚ˘ÓÙÈο ·ÔÙÂϤÛÌ·Ù· ÛÙË ıÂÚ·›· ÙˆÓ ÎÏÈÓÈÎÒÓ ÂΉËÏÒÛÂˆÓ Ù˘ √™ ·fi ÙÔ˘˜ Ó‡ÌÔÓ˜ (15,18,25). ªÂ ‰Â‰Ô̤ÓÔ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ fiÏÔÈ ÔÈ Ù‡ÔÈ ÙˆÓ ÂÈÏËÙÈÎÒÓ ÎÚ›ÛÂˆÓ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ÛÙËÓ √™, fiÏ· Ù· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η ¤¯Ô˘Ó ı¤ÛË ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛ‹ Ù˘. ∏ ÂÈÏÔÁ‹ ı· Á›ÓÂÈ Ì ‚¿ÛË ÙÔÓ Ù‡Ô ÙˆÓ ÎÚ›ÛˆÓ, ÙfiÛÔ ·fi ÙËÓ ÎÏÈÓÈ΋ ÂÚÈÁÚ·Ê‹ fiÛÔ Î·È ·fi Ù· Â˘Ú‹Ì·Ù· ÙÔ˘ ∏.∂.° Î·È ÔÏϤ˜ ÊÔÚ¤˜ ‰ÂÓ ·ÚΛ Ë ÌÔÓÔıÂÚ·›· (4,34-36). ∏ √™ ·ÔÙÂÏ› ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹ ÌÂÙ·‚È‚·˙fiÌÂÓË Î·Ù¿ ÙÔÓ ·˘ÙfiÛˆÌÔ ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú·. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ ÔÈÎÔÁ¤ÓÂÈ· Ì ¤Ó·Ó ÁÔÓ¤· Ì √™ ¤¯ÂÈ 50% Èı·ÓfiÙËÙ· Ó· ·ÔÎÙ‹ÛÂÈ ·È‰› Ì ÙËÓ ›‰È· ÓfiÛÔ. ∏ ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋ Â›Ó·È ··Ú·›ÙËÙË ÁÈ· Ó· ·ÔÊ¢¯ı› Ë Á¤ÓÓËÛË ·ÙfiÌˆÓ Ì ÙË ÓfiÛÔ (42-44).

µÈ‚ÏÈÔÁÚ·Ê›· 1. 1. Jozwiak S, Schwartz RA, Janniger CK, BielickaCymerman J. Usefulness of diagnostic criteria of tuberous sclerosis complex in pediatric patients. J Child Neurol 2000;15: 652-659.

2. Bourneville DM. Sclerose tuberouse des circonvolutions celebrales: idiotie et epilepsie hemiplegique.

Arch Neurologie 1880;1:81-91.

3. Humphrey A, Williams J, Pinto E, Bolton PF. A prospective longitudinal study of early cognitive development in tuberous sclerosis – a clinic based study.

Eur Child Adolesc Psychiatry 2004; 13:159-165.

4. Curatolo P, Verdecchia M, Bombardieri R. Tuberous sclerosis complex: a review of neurological aspects. Eur J Paediatr Neurol 2002;6:15-23.

5. Melean G, Sestini R, Ammannati F, Papi L. Genetic insights into familial tumors of the nervous system.

Am J Med Genet C Semin Med Genet 2004;129:74-84.

6. °ÈÔ˘ÚÔ‡ÎÔ˜ ™. ¡Â˘ÚÔÏÔÁÈο ÓÔÛ‹Ì·Ù· Ì ÂΉËÏÒÛÂȘ ·fi ÙÔ ‰¤ÚÌ·. In: ∏̤Ú˜ ·È‰È·ÙÚÈ΋˜ ÂÓË̤ڈÛ˘. ∞ı‹Ó·: °ã ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ; 2005. p. 17-29.

7. °ÈÔ˘ÚÔ‡ÎÔ˜ ™. ¡Â˘ÚÔ‰ÂÚÌ·ÙÈο Û‡Ó‰ÚÔÌ·. In: ªÂٷ͈ÙÔ‡ ∞, °ÈÔ˘ÚÔ‡ÎÔ˜ ™. £¤Ì·Ù· ¶·È‰ÔÓ¢ÚÔÏÔÁ›·˜. ∞ı‹Ó·: ∂ΉfiÛÂȘ ∑‹Ù·; 2000. p. 53-75.

8. Narayanan V. Tuberous sclerosis complex: genetics ¶·È‰È·ÙÚÈ΋ 2006;69:187-192

Pediatr May-June 06

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∫. ¶ÔÏ˘Ì¤ÚË, ∫. ∆ÛÔ˘Ì¿Î·˜

to pathogenesis. Pediatr Neurol 2003;29:404-409.

9. Young J, Povey S. The genetic basis of tuberous sclerosis. Mol Med Today 1998;4:313-319.

10. Yeung RS. Multiple roles of the tuberous sclerosis complex genes. Genes Chromosomes Cancer 2003;38:368-375.

11. Cheadle JP, Reeve MP, Sampson JR, Kwiatkowski DJ. Molecular genetic advances in tuberous sclerosis. Hum Genet 2000;107:97-114.

12. Niida Y, Lawrence-Smith N, Banwell A, Hammer E, Lewis J, Beauchamp RL, et al. Analysis of both TSC1 and TSC2 for germline mutations in 126 unrelated patients with tuberous sclerosis. Hum Mutat 1999;14:412-422.

13. Rose VM, Au KS, Pollom G, Roach ES, Prashner HR, Northrup H. Germ-line mosaicism in tuberous sclerosis: how common? Am J Hum Genet 1999;64:986-992.

14. Verhoef S, Bakker L, Tempelaars AM, HesselingJanssen AL, Mazurczak T, Jozwiak S, et al. High rate of mosaicism in tuberous sclerosis complex. Am J Hum Genet 1999;64:1632-1637.

15. Sparagana SP, Roach ES. Tuberous sclerosis complex. Curr Opin Neurol 2000;13:115-119.

16. Fesslova V, Villa L, Rizzuti T, Mastrangelo M, Mosca F. Natural history and long-term outcome of cardiac rhabdomyomas detected prenatally. Prenat Diagn 2004;24:241-248.

17. Camunez F, Lafuente J, Robledo R, Echenagusia A, Perez M, Simo G, et al. CT demonstration of extension of renal angiomyolipoma into the inferior vena cava in a patient with tuberous sclerosis. Urol Radiol 1987;9:152-154.

18. Barron RP, Kainulainen VT, Forrest CR, Krafchik B, Mock D, Sandor GK. Tuberous sclerosis: clinicopathologic features and review of the literature. J Craniomaxillofac Surg 2002;30:361-366.

19. Bissler JJ, Kingswood JC. Renal angiomyolipomata.

Kidney Int 2004;66:924-934.

20. Kitano Y, Honna T, Nihei K, Miyauchi J, Matsuoka K, Kuroda T, et al. Renal angiomyolipoma in Japanese tuberous sclerosis patients. J Pediatr Surg 2004;39:1784-1786.

21. O’Callaghan FJ, Noakes MJ, Martyn CN, Osborne JP. An epidemiological study of renal pathology in tuberous sclerosis complex. BJU Int 2004;94:853-857.

22. Oesterling JE, Fishman EK, Goldman SM, Marshall FF. The management of renal angiomyolipoma. J Urol 1986;135:1121-1124.

23. Blute ML, Malek RS, Segusa JW. Angiomyolipoma: clinical metamorphosis and concepts for management. J Urol 1988, 139:20-24.

24. Kane Y. The “bumps” on my face. J Am Acad Dermatol 2004;51 (1 Suppl):S11-S12.

25. Vicente MP, Pons M, Medina M. Pulmonary involvement in tuberous sclerosis. Pediatr Pulmonol 2004;37:178-180.

26. O’Callaghan FJ, Clarke AA, Hancock E, Hunt A, Osborne JP. Use of melatonin to treat sleep disorders

¶·È‰È·ÙÚÈ΋ 2006;69:187-192

27.

28.

29.

30.

31.

32.

33.

34.

35.

36.

37.

38.

39.

40.

41.

42.

43.

44.

in tuberous sclerosis. Dev Med Child Neurol 1999;41:123-126.

Hunt A, Stores G. Sleep disorder and epilepsy in children with tuberous sclerosis: a questionnaire-based study. Dev Med Child Neurol 1994;36:108-115.

Bolton PF. Neuroepileptic correlates of autistic symptomatology in tuberous sclerosis. Mental Retard Dev Disabil Res Rev 2004;10:126-131.

Muhle R, Trentacoste SV, Rapin I. The genetics of autism. Pediatrics 2004;113:e472-486.

Kielinen M, Rantala H, Timonen E, Linna SL, Moilanen I. Associated medical disorders and disabilities in children with autistic disorder: a population-based study. Autism 2004;8:49-60.

Curatolo P, Cusmai R. Autism and infantile spasms in children with tuberous sclerosis. Dev Med Child Neurol 1987;29:551.

Curatolo P, De Luca D, Bottini N, Dowling MM, Lucarelli P. Autism in tuberous sclerosis complex. J Child Neurol 2001;16:679.

Curatolo P, Porfirio MC, Manzi B, Seri S. Autism in tuberous sclerosis. Eur J Paediatr Neurol 2004;8:327-332.

Anisya-Vasanth AV, Satishchandra P, Nagaraja D, Swamy HS, Jayakumar PN. Spectrum of epilepsy in tuberous sclerosis. Neurol India 2004;52:210-212.

Crino PB. Knockout of a tuberous sclerosis gene highlights role of glia in epileptogenesis. Epilepsy Curr 2003;3:139-141.

Tomson T. Drug selection for the newly diagnosed patient: when is a new generation antiepileptic drug indicated? J Neurol 2004;251:1043-1049.

Shepherd CW, Houser OW, Gomez MR. MR findings in tuberous sclerosis complex and correlation with seizure development and mental impairment.

AJNR Am J Neuroradiol 1995;16:149-155.

Goodman M, Lamm SH, Engel A, Shepherd CW, Houser OW, Gomez MR. Cortical tuber count: a biomarker indicating neurologic severity of tuberous sclerosis complex. J Child Neurol 1997;12:85-90.

Hosoya M, Naito H, Nihei K. Neurological prognosis correlated with variations over time in the number of subependymal nodules in tuberous sclerosis.

Brain Dev 1999;21:544-547.

Roach ES, Sparagana SP. Diagnosis of tuberous sclerosis complex. J Child Neurol 2004;19:643-649.

Roach ES, Gomez MR, Northrup H. Tuberous Sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 1998;13: 624-628.

Harper PS. Practical Genetic Counseling. 4th ed.

Oxford: Butterworth-Heinemann Ltd; 1994.

Elcioglu N, Karatekin G, Elcioglu M, Nuhoglu M, Cenani A. Tuberous sclerosis: clinical evaluation in a family and implications for genetic counseling.

Genet Couns 1998;9:131-138.

Mueller RF. Genetic counseling in Tuberous Sclerosis. Scan Facts, Fact-Sheet No 20 of the Tuberous Sclerosis Association, 1998.

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∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞

ORIGINAL ARTICLE

193

™˘ÌÌfiÚʈÛË ∂ÏÏ‹ÓˆÓ È·ÙÚÒÓ ÛÙȘ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜ ÁÈ· ÙËÓ ÚfiÏË„Ë Î·È ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿ ¶. •Â··‰¿ÎË, ¡. ¢Ô˘Ï·‰›Ú˘, ª. ª·ÓÔ˘Û¿Î˘, º. ™·ÍÒÓË - ¶··ÁˆÚÁ›Ô˘, ¡.°. ¶··‰fiÔ˘ÏÔ˜

¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: ∏ ·˘Í·ÓfiÌÂÓË Û˘¯ÓfiÙËÙ· ÙÔ˘ ¿ÛıÌ·ÙÔ˜ Ô‰‹ÁËÛ ÙËÓ ÂÈÛÙËÌÔÓÈ΋ ÎÔÈÓfiÙËÙ· ÛÙË ‰È·Ù‡ˆÛË ‰ÈÂıÓÒÓ ÔÌÔʈÓÈÒÓ, ÁÈ· ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ (Global Initiative for Asthma, GINA). ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ÁÓÒÛ˘, Ù˘ ηٷÓfiËÛ˘, Ù˘ ÁÓÒÌ˘ Î·È Ù˘ ¯Ú‹Û˘ ÙˆÓ ‰ÈÂıÓÒÓ Ô‰ËÁÈÒÓ ·fi ÙÔ˘˜ ŒÏÏËÓ˜ È·ÙÚÔ‡˜. ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ: ∞ӷχıËÎ·Ó ÛÙÔȯ›· ·fi ·ÓÒÓ˘Ì· ÂÚˆÙËÌ·ÙÔÏfiÁÈ· Ô˘ ‰È·ÓÂÌ‹ıËÎ·Ó Î·È Û˘ÌÏËÚÒıËÎ·Ó ·fi ·È‰È¿ÙÚÔ˘˜, Ó¢ÌÔÓÔÏfiÁÔ˘˜ Î·È ·ÏÏÂÚÁÈÔÏfiÁÔ˘˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÂÈÛÙËÌÔÓÈ΋˜ ËÌÂÚ›‰·˜. ∞ÔÙÂϤÛÌ·Ù·: ∏ Û˘ÌÌfiÚʈÛË ÙˆÓ ∂ÏÏ‹ÓˆÓ È·ÙÚÒÓ ÛÙȘ ‰ÈÂıÓ›˜ ÔÌÔʈӛ˜ Ê¿ÓËΠfiÙÈ Â›Ó·È Ùˆ¯‹, ·Ú¿ ÙÔ fiÙÈ Ë ÏÂÈÔ„ËÊ›· ÁÓˆÚ›˙ÂÈ Î·È Û˘ÌʈÓ› Ì ÙȘ Ô‰ËÁ›Â˜. ªfiÏȘ ÛÙÔ 25% ÙˆÓ ·È‰ÈÒÓ Ì Â›ÌÔÓÔ ¿ÛıÌ· ¯ÔÚËÁÔ‡ÓÙ·È ÚÔÊ˘Ï·ÎÙÈο ÂÈÛÓÂfiÌÂÓ· ÛÙÂÚÔÂȉ‹ ÁÈ· ‰È¿ÛÙËÌ· ÌÂÁ·Ï‡ÙÂÚÔ ÙˆÓ 3 ÌËÓÒÓ, ·Ó Î·È 96% ÙˆÓ ÂÚˆÙËı¤ÓÙˆÓ ÁÓˆÚ›˙Ô˘Ó ÙËÓ Ô‰ËÁ›· ·˘Ù‹. ªfiÓÔ ÙÔ 7% ÙˆÓ ·È‰ÈÒÓ Ì Â›ÌÔÓÔ ¿ÛıÌ· ¤Ï·‚·Ó Ô‰ËÁ›· ÁÈ· ¯Ú‹ÛË ÚÔÔ̤ÙÚÔ˘ ÛÙÔ Û›ÙÈ, ·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ 76% ·fi ·˘ÙÔ‡˜ ÁÓˆÚ›˙Ô˘Ó ÙË Û˘ÁÎÂÎÚÈ̤ÓË Ô‰ËÁ›·. ∆¤ÏÔ˜, ÔÈ ŒÏÏËÓ˜ È·ÙÚÔ› ÂÓËÌÂÚÒÓÔ˘Ó Û ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ÙˆÓ ÂÈÛΤ„ÂˆÓ (~67%) ÙÔ˘˜ ÁÔÓ›˜ ÙˆÓ ·È‰ÈÒÓ ÁÈ· ÙËÓ ·Ó¿ÁÎË ‰È·ÎÔ‹˜ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜. ∫·Ù·ÁÚ¿ÊËΠÛËÌ·ÓÙÈο Û˘¯ÓfiÙÂÚË ¯ÔÚ‹ÁËÛË ÚÔÔ̤ÙÚÔ˘ ÛÙÔ˘˜ ·ÛıÌ·ÙÈÎÔ‡˜ ·ÛıÂÓ›˜ ·fi ·ÏÏÂÚÁÈÔÏfiÁÔ˘˜ /Ó¢ÌÔÓÔÏfiÁÔ˘˜ Û ۯ¤ÛË Ì ÙÔ˘˜ ·È‰È¿ÙÚÔ˘˜. ™˘ÌÂÚ¿ÛÌ·Ù·: ∏ ÂÊ·ÚÌÔÁ‹ ÙˆÓ ‰ÈÂıÓÒÓ Ô‰ËÁÈÒÓ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ·fi ÙÔ˘˜ ŒÏÏËÓ˜ ·È‰È¿ÙÚÔ˘˜ Â›Ó·È ÏËÌÌÂÏ‹˜, ·Ú¿ ÙËÓ ÈηÓÔÔÈËÙÈ΋ ÁÓÒÛË ·˘ÙÒÓ. ∏ ¤ÏÏÂÈ„Ë ¿ÓÂÛ˘ ÛÙË ¯Ú‹ÛË ÙˆÓ Ô‰ËÁÈÒÓ, Ô˘ Û¯ÂÙ›˙ÂÙ·È ÛÙÂÓ¿ Ì ÙËÓ ·Ó¿ÏÔÁË Û˘Ó¯È˙fiÌÂÓË È·ÙÚÈ΋ ÂÎ·›‰Â˘ÛË, Ê·›ÓÂÙ·È Ó· ¢ı‡ÓÂÙ·È ÁÈ· ÙËÓ ÏËÌÌÂÏ‹ Û˘ÌÌfiÚʈÛË, ˘Ô‰ÂÈÎÓ‡ÔÓÙ·˜ ÙËÓ ·Ó¿ÁÎË ÁÈ· Û˘Ó¯‹ ÂÓË̤ڈÛË Î·È ÂÎ·›‰Â˘ÛË ÙˆÓ È·ÙÚÒÓ Î˘Ú›ˆ˜ Û Ú·ÎÙÈο ı¤Ì·Ù·.

∞ÏÏÂÚÁÈÔÏÔÁÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡” AÏÏËÏÔÁÚ·Ê›·: ¶·Ú·Û΢‹ •Â··‰¿ÎË ∞ÏÏÂÚÁÈÔÏÔÁÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·Ó/Ì›Ô˘ ∞ıËÓÒÓ £Ë‚ÒÓ Î·È §Â‚·‰Â›·˜ ∆.∫. 115 27, °Ô˘‰› E-mail: panvik@hol.gr ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 18-05-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 01-02-2006

§¤ÍÂȘ ÎÏÂȉȿ: ÕÛıÌ·, Û˘ÌÌfiÚʈÛË, ηÙ¢ı˘ÓÙ‹ÚȘ Ô‰ËÁ›Â˜.

Adherence of Greek physicians to the international guidelines for the prevention and management of asthma in children P. Xepapadaki, ¡. Douladiris, ª. Manoussakis, F. Saxoni - Papageorgiou, N.G. Papadopoulos

Abstract Introduction: Asthma is a severe chronic disease affecting a large proportion of the population.

International guidelines for the diagnosis and management of asthma have been published (Global Initiative for Asthma, GINA), with a view to improving asthma management. This study was conducted to assess the adherence of Greek doctors managing children with asthma, to the GINA guidelines.

Methods: Analyses were performed on 160 anonymous questionnaires delivered to doctors (paediatricians, allergists and lung specialists) during a medical conference.

Results: In general, reported adherence to the GINA guidelines was poor, although the majority of doctors seemed to agree with the recommendations. Specifically, only 25% of children with persistent asthma receive long-term inhaled steroid treatment, although 96% of the doctors were aware of the relevant recommendation. In addition, only 7% of children with persistent asthma had a peak flow meter for daily monitoring, although 76% of the doctors reported awareness of the recommendation.

Finally, the rate of adherence for counselling parents on smoking cessation was 67%. Peak-flow meter

Department of Allergology, 2nd Paediatric Clinic, University of Athens, “P. & A. Kyriakou” Children’s Hospital Correspondence: Paraskevi Xepapadaki Department of Allergology, 2nd Paediatric Clinic, University of Athens Thivon & Levadias 115 27, Goudi E-mail: panvik@hol.gr Date of submission: 18-05-2005 Date of approval: 01-02-2006

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prescription rates were significantly higher among allergists/lung specialists than paediatricians.

Conclusions: Although the doctors in this sample were aware of the GINA guidelines, their compliance with the recommendations was limited. It appears that lack of confidence in their ability to deliver the guideline components, a parameter closely related to appropriate training and knowledge, may have affected guideline use, indicating a need for additional, practically-oriented continued medical education for doctors managing children with asthma.

Key words: ∞sthma, adherence, guidelines.

∂ÈÛ·ÁˆÁ‹ ∆Ô ¿ÛıÌ· Â›Ó·È ¯ÚfiÓÈ· ÊÏÂÁÌÔÓ҉˘ ÓfiÛÔ˜ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Ô˘ ÂËÚ¿˙ÂÈ ¿Óˆ ·fi 150 ÂηÙÔÌ̇ÚÈ· ·ÛıÂÓ›˜ Û fiÏÔ ÙÔÓ ÎfiÛÌÔ (1). ¶·Ú¿ ÙȘ ÛËÌ·ÓÙÈΤ˜ ‰È·Î˘Ì¿ÓÛÂȘ ÛÙËÓ Â›ÙˆÛË Ù˘ ÓfiÛÔ˘ ÌÂٷ͇ ÏËı˘ÛÌÒÓ, ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ ·Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ ÂÌÊ·Ó›˙ÂÙ·È Û fiϘ ÙȘ ÂÚÈÔ¯¤˜ ÙÔ˘ Ï·Ó‹ÙË, Ì ÙȘ ¯ÒÚ˜ Ù˘ ¢˘ÙÈ΋˜ ∂˘ÚÒ˘ Ó· η٤¯Ô˘Ó Ì›· ·fi ÙȘ ˘„ËÏfiÙÂÚ˜ ı¤ÛÂȘ ÛÙË Û˘¯ÓfiÙËÙ· ηٷÁÚ·Ê‹˜ ·ÛıÌ·ÙÈÎÒÓ ·ÛıÂÓÒÓ (2). ™‡Ìʈӷ Ì ‰Â‰Ô̤ӷ ·fi ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜, ÛËÌÂÈÒÓÂÙ·È ·‡ÍËÛË Ù˘ Ù¿Í˘ ÙÔ˘ 2-4% ÂÙËÛ›ˆ˜ ÛÙË Û˘¯ÓfiÙËÙ· ÙÔ˘ ¿ÛıÌ·ÙÔ˜ Ù· ÙÂÏÂ˘Ù·›· 15 ¯ÚfiÓÈ· Û ‰È¿ÊÔÚ˜ ¯ÒÚ˜ ÙÔ˘ ÎfiÛÌÔ˘, ÙËÓ ∂˘ÚÒË, ·ÏÏ¿ Î·È ÙËÓ ∂ÏÏ¿‰· (3,4). °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, ¤ÁÈÓ ۷ʋ˜ ÛÙËÓ ÂÈÛÙËÌÔÓÈ΋ ÎÔÈÓfiÙËÙ· Ë ·Ó¿ÁÎË ‰È·ÌfiÚʈÛ˘ ÎÔÈÓÒÓ Î·Ù¢ı˘ÓÙ‹ÚÈˆÓ Ô‰ËÁÈÒÓ ÁÈ· ÙË ‰È¿ÁÓˆÛË Î·È ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜. ∆Ô 1995 ‰ËÌÔÛȇıËÎ·Ó ÔÈ ÚÒÙ˜ ‰ÈÂıÓ›˜ ÔÌÔʈӛ˜ Ù˘ GINA (Global Initiative for Asthma) Ô˘ ·Ó·ıˆڋıËÎ·Ó ÙÔ 1997, ÙÔ 2002 Î·È ÙÔ 2004. ™ÙȘ ÔÌÔʈӛ˜ ·˘Ù¤˜ ·Ó·ÁÓˆÚ›˙ÂÙ·È Ô ÛËÌ·ÓÙÈÎfi˜ ÚfiÏÔ˜ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÛÙÔ ¿ÛıÌ· ηıÒ˜ Î·È Ù˘ ¯ÔÚ‹ÁËÛ˘, ·Ó¿ÏÔÁ· Ì ÙË ‚·Ú‡ÙËÙ·, ηٿÏÏËÏ˘ ·ÓÙÈ·ÛıÌ·ÙÈ΋˜ ·ÁˆÁ‹˜. ∂ÈÛËÌ·›ÓÂÙ·È Â›Û˘, Ë ·Ó¿ÁÎË ‰È·ÚÎÔ‡˜ ÂÎ·›‰Â˘Û˘ ÙˆÓ ·ÛıÂÓÒÓ Î·È Ë ·Ó·ÁηÈfiÙËÙ· ¯ÚËÛÈÌÔÔ›ËÛ˘ ·ÓÙÈÎÂÈÌÂÓÈÎÒÓ ‰ÂÈÎÙÒÓ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, fiˆ˜ Ë Î·ıËÌÂÚÈÓ‹ ÚÔÔ̤ÙÚËÛË ÛÙÔ Û›ÙÈ. ¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÈ ·Ú·¿Óˆ ÔÌÔʈӛ˜ ¤¯Ô˘Ó ‰È·Ù˘ˆı› ¿Óˆ ·fi Ì›· 10ÂÙ›·, Ë Û˘ÌÌfiÚʈÛË ÙˆÓ È·ÙÚÒÓ Û ·˘Ù¤˜ Â›Ó·È Û˘¯Ó¿ Ùˆ¯‹ Î·È ·ÓÂ·Ú΋˜ (5-7), ÁÂÁÔÓfi˜ Ô˘ ÌÔÚ› Ó· ¤¯ÂÈ ÛËÌ·ÓÙÈΤ˜ ·ÚÓËÙÈΤ˜ ÂÈÙÒÛÂȘ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ¿ÛıÌ·. ÀÔı¤Û·Ì fiÙÈ Ë ÁÓÒÛË Î·È ÂÊ·ÚÌÔÁ‹ ÙˆÓ Ô‰ËÁÈÒÓ Ù˘ GINA Â›Ó·È ÏËÌÌÂÏ‹˜ Î·È ÂÔ̤ӈ˜ Âȉ¤¯ÂÙ·È ‚ÂÏÙ›ˆÛ˘. ™ÎÔfi˜ Ù˘ ·¶·È‰È·ÙÚÈ΋ 2006;69:193-198

ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ÁÓÒÛ˘, Ù˘ ηٷÓfiËÛ˘, Ù˘ ÁÓÒÌ˘ Î·È Ù˘ ¯Ú‹Û˘ ÛÙÔȯ›ˆÓ ÙˆÓ ‰ÈÂıÓÒÓ Ô‰ËÁÈÒÓ Ù˘ GINA ·fi ŒÏÏËÓ˜ È·ÙÚÔ‡˜ Ô˘ ıÂÚ·Â‡Ô˘Ó ·È‰È¿ Ì ¿ÛıÌ·.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ¶ÏËı˘ÛÌfi˜ ÂϤÁ¯Ô˘ ∏ ÌÂϤÙË ‰ÈÂÓÂÚÁ‹ıËΠÛÙ· Ï·›ÛÈ· Ù˘ 5˘ ÌÂÙÂÎ·È‰Â˘ÙÈ΋˜ ËÌÂÚ›‰·˜ “∆Ô ·ÏÏÂÚÁÈÎfi ·È‰› ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË”, Ô˘ ‰ÈÔÚÁ·ÓÒÓÂÙ·È ·fi ÙÔ ∞ÏÏÂÚÁÈÔÏÔÁÈÎfi ∆Ì‹Ì· Ù˘ µã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, fiÔ˘ ‰È·ÓÂÌ‹ıËÎÂ Î·È Û˘ÌÏËÚÒıËΠ·fi ÙÔ˘˜ Û˘ÌÌÂÙ¤¯ÔÓÙ˜ È·ÙÚÔ‡˜ ·ÓÒÓ˘ÌÔ ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ. ™˘ÓÔÏÈο Û˘ÌÌÂÙ›¯·Ó 160 ¿ÙÔÌ·, ÂȉÈ΢fiÌÂÓÔÈ Î·È ÂȉÈÎÔ› ·È‰›·ÙÚÔÈ, Ó¢ÌÔÓÔÏfiÁÔÈ Î·È ·ÏÏÂÚÁÈÔÏfiÁÔÈ. ÃÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÔÈ ·Ú·Î¿Ùˆ 3 ÂÚˆÙ‹ÛÂȘ: ·) Û ÙÈ ÔÛÔÛÙfi ·ÛıÂÓÒÓ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ì Â›ÌÔÓÔ ¿ÛıÌ· ¯ÔÚËÁ›Ù ÚÔÊ˘Ï·ÎÙÈο ÂÈÛÓÂfiÌÂÓ· ÛÙÂÚÔÂȉ‹ ÁÈ· ‰È¿ÛÙËÌ· ÌÂÁ·Ï‡ÙÂÚÔ ÙˆÓ 3 ÌËÓÒÓ; ‚) Û ÙÈ ÔÛÔÛÙfi ·ÛıÂÓÒÓ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ì Â›ÌÔÓÔ ¿ÛıÌ· ¯ÔÚËÁ›Ù ÚÔfiÌÂÙÚÔ Î·È Ô‰ËÁ›Â˜ ÁÈ· ηıËÌÂÚÈÓ‹ ¯Ú‹ÛË ÙÔ˘; Á) Û ÙÈ ÔÛÔÛÙfi ÂÈÛΤ„ÂˆÓ ÌÈÏ¿ÙÂ Û˘ÛÙËÌ·ÙÈο ÛÙÔ˘˜ ÁÔÓ›˜ ÁÈ· ÙËÓ ·Ó¿ÁÎË ‰È·ÎÔ‹˜ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜; ¶ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÍÈÔÏÔÁËıÔ‡Ó ÔÛÔÙÈΤ˜ ‰È·ÊÔÚ¤˜ ¯ÚËÛÈÌÔÔÈ‹ıËΠÎϛ̷η 6 ÛËÌ›ˆÓ, ÂÓÒ ·ÍÈÔÏfiÁËÛË Ì Îϛ̷η 4 ÛËÌ›ˆÓ ¤ÁÈÓ ÁÈ· ÙËÓ ·Ó·ÁÓÒÚÈÛË “ÊÚ·ÁÌÒÓ” Ô˘ ı· ÌÔÚÔ‡Û·Ó Ó· Û˘Ì‚¿ÏÔ˘Ó ÛÙË ÌË Ù‹ÚËÛË ÙˆÓ Ô‰ËÁÈÒÓ Î·È ·ÊÔÚÔ‡Û·Ó ÛÙË ÁÓÒÛË ÙˆÓ Ô‰ËÁÈÒÓ, ÛÙËÓ ¿ÓÂÛË ÂÊ·ÚÌÔÁ‹˜ ÙÔ˘˜, ÛÙËÓ ÂÓÙ‡ˆÛË fiÙÈ ÔÈ Ô‰ËÁ›Â˜ ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó ÙÔÓ ·ÛıÂÓ‹, ÛÙË Û˘Ìʈӛ· Ì ÙȘ Ô‰ËÁ›Â˜ Î·È Ù¤ÏÔ˜, ÛÙË ‰˘Ó·ÙfiÙËÙ· ÂÊ·ÚÌÔÁ‹˜ ÙÔ˘˜ ÛÙÔ È·ÙÚ›Ô. ∂›Û˘, ‰ÈÂÚ¢ӋıËÎÂ Ë ·Ó·ÁÓÒÚÈÛË ‹ ÌË Î·ıÂÌ›·˜ ·fi ÙȘ ·Ú·¿Óˆ Ô‰ËÁ›Â˜ ˆ˜ ̤ÚÔ˜ Ù˘ GINA. ∏ ·Ú·¿Óˆ ÌÂıÔ‰ÔÏÔÁ›· ›¯Â ·Ó·Ù˘¯ı› Î·È Ù˘ÔÔÈËı› Û ÚÔËÁÔ‡ÌÂÓË ÌÂϤÙË (8). ™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË £ÂˆÚ‹ıËΠfiÙÈ ˘‹Ú¯Â Û˘Ìʈӛ· Ì ÙȘ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜ fiÙ·Ó ÔÈ È·ÙÚÔ› ‰‹ÏˆÓ·Ó ÂÊ·ÚÌÔÁ‹ ÙÔ˘˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Û ÔÛÔÛÙfi ¿Óˆ ·fi 90%. ∂ÈϤÔÓ, fiÛÔÓ ·ÊÔÚ¿ ÛÙË ÁÓÒÛË, ÙËÓ ¿ÓÂÛË, ÙËÓ ÂÊ·ÚÌÔÁ‹ Î·È ÙËÓ ÂÓÙ‡ˆÛË fiÙÈ Ë Ô‰ËÁ›· ı· ‚ÔËı‹ÛÂÈ ÙÔÓ ·ÛıÂÓ‹, ıˆڋıËÎ·Ó ıÂÙÈΤ˜ ÔÈ ··ÓÙ‹ÛÂȘ Ô˘ ·Ó‹Î·Ó ÛÙËÓ 3Ë ‹ ÛÙËÓ 4Ë Î·ÙËÁÔÚ›·. ∆¤ÏÔ˜, Û˘Ìʈӛ· Ì ÙËÓ Ô‰ËÁ›· ηÈ

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·195

195

™˘ÌÌfiÚʈÛË ÛÙȘ ηÙ¢ı˘ÓÙ‹ÚȘ Ô‰ËÁ›Â˜

‰˘Ó·ÙfiÙËÙ· ÂÊ·ÚÌÔÁ‹˜ Ù˘ ÛÙÔ È·ÙÚÂ›Ô ˘‹Ú¯·Ó fiÙ·Ó ÔÈ ··ÓÙ‹ÛÂȘ ·Ó‹Î·Ó ÛÙËÓ 1Ë ‹ ÛÙË 2Ë Î·ÙËÁÔÚ›·. ™˘Û¯ÂÙ›ÛÂȘ ÌÂٷ͇ ·ÓÂÍ¿ÚÙËÙˆÓ ÌÂÙ·‚ÏËÙÒÓ ÂÎÙÈÌ‹ıËÎ·Ó ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ¯2.

ŸÛÔÓ ·ÊÔÚ¿ ÛÙË ¯ÔÚ‹ÁËÛË ÚÔÔ̤ÙÚÔ˘ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ·ÛıÂÓÒÓ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ì Â›ÌÔÓÔ ¿ÛıÌ·, Ù· ÔÛÔÛÙ¿ ÂÊ·ÚÌÔÁ‹˜ Ù˘ Ô‰ËÁ›·˜ ‹Ù·Ó ȉȷ›ÙÂÚ· ¯·ÌËÏ¿. ™‡Ìʈӷ Ì ÙȘ ··ÓÙ‹ÛÂȘ, ÌfiÓÔ ÙÔ 7% ÙˆÓ ·È‰ÈÒÓ Ì Â›ÌÔÓÔ ¿ÛıÌ· ¤Ï·‚·Ó Ô‰ËÁ›· ÁÈ· ¯Ú‹ÛË ÚÔÔ̤ÙÚÔ˘ ÛÙÔ Û›ÙÈ ·fi ÙÔ˘˜ ıÂÚ¿ÔÓÙ˜ È·ÙÚÔ‡˜, ·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ 76% ·˘ÙÒÓ ÁÓˆÚ›˙Ô˘Ó fiÙÈ Ë Ô‰ËÁ›· ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙȘ ‰ÈÂıÓ›˜ ÔÌÔʈӛ˜ Ù˘ GINA, 73,1% Û˘ÌʈÓÔ‡Ó Î·È 59% ÈÛÙÂ‡Ô˘Ó fiÙÈ ÌÔÚ› Ó· ‚ÔËı‹ÛÂÈ. ∏ ¯·ÌËÏ‹ ‰È›ۉ˘ÛË Ù˘ Ô‰ËÁ›·˜ ·˘Ù‹˜ Èı·ÓÒ˜ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÏÈÁfiÙÂÚÔÈ ·fi ÙÔ 50% ÙˆÓ È·ÙÚÒÓ Â›¯·Ó Â·Ú΋ ÁÓÒÛË Ù˘ ¯Ú‹Û˘ ÙÔ˘ ÚÔÔ̤ÙÚÔ˘ Î·È ÌfiÏȘ 37,5% ‰‹ÏˆÛ·Ó ¿ÓÂÛË ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ Ô‰ËÁ›·˜ (∂ÈÎfiÓ· 2). √È ÂÚÈÛÛfiÙÂÚÔÈ (83,3%) ÁÓÒÚÈ˙·Ó ÙËÓ Ô‰ËÁ›· ·ÔÊ˘Á‹˜ ¤ÎıÂÛ˘ ÛÙÔÓ Î·Ófi ÙÔ˘ ÙÛÈÁ¿ÚÔ˘ Î·È ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙȘ ‰ÈÂıÓ›˜ ÔÌÔʈӛ˜ (92%) Ì Â·ÎfiÏÔ˘ıË ¿ÓÂÛË ÛÙË ¯Ú‹ÛË Ù˘ (72,5%). ∆Ô 90% ÙˆÓ Û˘Ó·‰¤ÏÊˆÓ Û˘ÌʈÓÔ‡Ó Ì ÙËÓ Ô‰ËÁ›· ·˘Ù‹ Î·È ÙÔ 67% ÂÓËÌÂÚÒÓÔ˘Ó Û ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ÙˆÓ ÂÈÛΤ„ÂˆÓ ÙÔ˘˜ ÁÔÓ›˜ ÙˆÓ ·È‰ÈÒÓ ÁÈ· ÙËÓ ·Ó¿ÁÎË ‰È·ÎÔ‹˜ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜ (∂ÈÎfiÓ· 3). ∞ÍÈÔÏÔÁ‹Û·Ì ÛÙË Û˘Ó¤¯ÂÈ· ÙË Û˘ÌÌfiÚʈÛË ÙˆÓ ıÂÚ·fiÓÙˆÓ È·ÙÚÒÓ Ì ÙȘ ‰ÈÂıÓ›˜ ÔÌÔʈӛ˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ηٿ ÂȉÈÎfiÙËÙ·. ¢ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÛÙË Û˘¯ÓfiÙËÙ· ¯ÔÚ‹ÁËÛ˘ ÂÈÛÓÂfiÌÂÓˆÓ ÛÙÂÚÔÂȉÒÓ Î·È ÂÓË̤ڈÛ˘

∞ÔÙÂϤÛÌ·Ù· ∞fi ÙÔ˘˜ Û˘ÌÌÂÙ¤¯ÔÓÙ˜, ÙÔ 37% ‹Ù·Ó ¿Ó‰Ú˜ Î·È ÙÔ 63% Á˘Ó·›Î˜. ∏ ÏÂÈÔ„ËÊ›· ÙˆÓ È·ÙÚÒÓ ‹Ù·Ó ·È‰›·ÙÚÔÈ (86%), 9,8% ·ÏÏÂÚÁÈÔÏfiÁÔÈ Î·È ÔÈ ˘fiÏÔÈÔÈ (4,2%) Ó¢ÌÔÓÔÏfiÁÔÈ Î·È ÁÂÓÈÎÔ› È·ÙÚÔ›. ∆Ô 67% ÙˆÓ Û˘ÌÌÂÙ¯fiÓÙˆÓ ‹Ù·Ó ÂȉÈÎÂ˘Ì¤ÓÔÈ Î·È ÙÔ 26% ÂȉÈ΢fiÌÂÓÔÈ.

To 35% ÙˆÓ È·ÙÚÒÓ Î·Ù›¯·Ó ÙÔÓ Ù›ÙÏÔ ÂȉÈÎfiÙËÙ·˜ ¿Óˆ ·fi 15 ¯ÚfiÓÈ·, ÙÔ 32% 5-14 ¯ÚfiÓÈ· Î·È 33% <5 ¯ÚfiÓÈ·. ∆Ô 50% ÙˆÓ Û˘ÌÌÂÙ¯fiÓÙˆÓ ‹Ù·Ó ÂχıÂÚÔÈ Â·ÁÁÂÏ̷ٛ˜, 36% ÂÚÁ¿˙ÔÓÙ·Ó Û ÓÔÛÔÎÔÌ›·, 8% Û ∫¤ÓÙÚ· ÀÁ›·˜ Î·È 7% ÛÙÔ π∫∞. ∆Ô 96% ÙˆÓ ÂÚˆÙËı¤ÓÙˆÓ ÁÓÒÚÈ˙·Ó fiÙÈ Ë ¯ÔÚ‹ÁËÛË ÛÙÂÚÔÂȉÒÓ Â› Ì·ÎÚfiÓ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙȘ Ô‰ËÁ›Â˜ Ù˘ GINA ÁÈ· ÙÔ Â›ÌÔÓÔ ¿ÛıÌ·. ∂›Û˘, Ë Û˘Ìʈӛ· Ì ÙËÓ Ô‰ËÁ›· ‹Ù·Ó ÌÂÁ¿ÏË (94,6%), fiˆ˜ Î·È Ë ıÂÙÈ΋ ÂÓÙ‡ˆÛË Û¯ÂÙÈο Ì ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ Ô‰ËÁ›·˜ ·˘Ù‹˜ (83,1%) Î·È ÙËÓ ÂÊ·ÚÌÔÛÈÌfiÙËÙ¿ Ù˘ (87,5%). ªfiÓÔ 17% ‰‹ÏˆÛ·Ó ¤ÏÏÂÈ„Ë ÁÓÒÛ˘ Î·È 27% ¤ÏÏÂÈ„Ë ¿ÓÂÛ˘ ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ Ô‰ËÁ›·˜ ·˘Ù‹˜. ¶·ÚfiÏ· ·˘Ù¿, ÌfiÏȘ ÙÔ 25% ‰‹ÏˆÛ fiÙÈ ·ÎÔÏÔ˘ı› ÙËÓ Ô‰ËÁ›· ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ú·ÎÙÈ΋ (∂ÈÎfiÓ· 1).

¶ÔÛÔÛÙfi ÂÊ·ÚÌÔÁ‹˜ Ù˘ Ô‰ËÁ›·˜

25

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83

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83

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73

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100

¶ÔÛÔÛÙfi (%)

∂ÈÎfiÓ· 1. ™˘¯ÓfiÙËÙ· ¯ÔÚ‹ÁËÛ˘ Ì·ÎÚÔ¯ÚfiÓÈ· ÂÈÛÓÂfiÌÂÓˆÓ ÛÙÂÚÔÂȉÒÓ Û ·È‰È¿ Ì Â›ÌÔÓÔ ¿ÛıÌ· Î·È ·Ú¿ÁÔÓÙ˜ Ô˘ Û˘Ì‚¿ÏÏÔ˘Ó ÛÙË Û˘ÌÌfiÚʈÛË ÙˆÓ ÁÈ·ÙÚÒÓ ÛÙËÓ Ô‰ËÁ›· ·˘Ù‹. ¶·È‰È·ÙÚÈ΋ 2006;69:193-198

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·196

196

¶. •Â··‰¿ÎË Î·È Û˘Ó.

¶ÔÛÔÛÙfi ÂÊ·ÚÌÔÁ‹˜ Ù˘ Ô‰ËÁ›·˜

7

°ÓÒÛË Ù˘ Ô‰ËÁ›·˜

49

£ÂÙÈ΋ ÂÓÙ‡ˆÛË ÁÈ· ÙËÓ Ô‰ËÁ›·

59

ÕÓÂÛË ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ Ô‰ËÁ›·˜

37

™˘Ìʈӛ· Ì ÙËÓ Ô‰ËÁ›·

73 0

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20

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40

50

60

70

80

90

100

¶ÔÛÔÛÙfi (%)

∂ÈÎfiÓ· 2. ¶ÔÛÔÛÙ¿ ¯ÔÚ‹ÁËÛ˘ ÚÔÔ̤ÙÚÔ˘ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÛÙÔ Û›ÙÈ ·È‰ÈÒÓ Ì Â›ÌÔÓÔ ¿ÛıÌ· Î·È ·Ú¿ÁÔÓÙ˜ Ô˘ Û˘Ì‚¿ÏÏÔ˘Ó ÛÙË Û˘ÌÌfiÚʈÛË ÙˆÓ ÁÈ·ÙÚÒÓ ÛÙËÓ Ô‰ËÁ›· ·˘Ù‹.

ÙˆÓ ÁÔÓ¤ˆÓ ÁÈ· ÙËÓ ·Ó·ÁηÈfiÙËÙ· ‰È·ÎÔ‹˜ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜ ÌÂٷ͇ ·È‰È¿ÙÚˆÓ (28,1%, Î·È 69,5%) Î·È È·ÙÚÒÓ ¿ÏÏˆÓ ÂȉÈÎÔÙ‹ÙˆÓ (·ÏÏÂÚÁÈÔÏfiÁˆÓ/Ó¢ÌÔÓÔÏfiÁˆÓ - 26,7% Î·È 60%, p=0,94 Î·È p=0,38 ·ÓÙ›ÛÙÔȯ·). ∞ÓÙ›ıÂÙ·, ηٷÁÚ¿ÊËΠÛÙ·ÙÈÛÙÈο Û˘¯ÓfiÙÂÚË ¯ÔÚ‹ÁËÛË ÚÔÔ̤ÙÚÔ˘ ·fi ÙÔ˘˜ ·ÏÏÂÚÁÈÔÏfiÁÔ˘˜ / Ó¢ÌÔÓÔÏfiÁÔ˘˜ Û ۯ¤ÛË Ì ÙÔ˘˜ ·È‰È¿ÙÚÔ˘˜ (12,5 Î·È 5,7% ·ÓÙ›ÛÙÔȯ·, p=0,005). ¢ÂÓ ˘‹Ú¯·Ó ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ÂȉÈÎÂ˘Ì¤ÓˆÓ Î·È ÂȉÈ΢fiÌÂÓˆÓ È·ÙÚÒÓ fiÛÔÓ ·ÊÔÚ¿ ÛÙË ¯ÔÚ‹ÁËÛË ÛÙÂÚÔÂȉÒÓ Û Â›ÌÔÓÔ ¿ÛıÌ· (26,5 Î·È 27,3% ·ÓÙ›ÛÙÔȯ·, p=0,67), ÙË ¯ÔÚ‹ÁËÛË ÚÔÔ̤ÙÚÔ˘ (6,2 Î·È 5,9%, p=0,98) Î·È ÙË Û‡ÛÙ·ÛË ‰È·ÎÔ‹˜ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜ (70,5 Î·È 83,3% ·ÓÙ›ÛÙÔȯ·, p=0,13). ∆¤ÏÔ˜, ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ˆ˜ ÚÔ˜ ÙÔÓ ‚·ıÌfi Û˘ÌÌfiÚʈÛ˘ ÚÔ˜ ÙȘ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜ ·ÓÙÈÌÂÙÒÈÛ˘ ·ÛıÌ·ÙÈÎÒÓ ·ÛıÂÓÒÓ ÌÂٷ͇ ȉȈÙÒÓ Î·È ÓÔÛÔÎÔÌÂÈ·ÎÒÓ È·ÙÚÒÓ (¯ÔÚ‹ÁËÛË ÛÙÂÚÔÂȉÒÓ: p=0,70, Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË ÚÔÔ̤ÙÚÔ˘: p=0,67, Û‡ÛÙ·ÛË ‰È·ÎÔ‹˜ ηÓ›ÛÌ·ÙÔ˜: p=0,67 ÌÂٷ͇ ÙˆÓ ÔÌ¿‰ˆÓ).

™˘˙‹ÙËÛË ∏ ‰È·Ú΋˜ ·‡ÍËÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ÛÙËÓ ·È‰È΋ ËÏÈΛ· ÒıËÛ ÙËÓ È·ÙÚÈ΋ ÎÔÈÓfiÙËÙ· ÛÙË ‰È·ÌfiÚʈÛË ÂÓfi˜ ÎÔÈÓÔ‡ ¶·È‰È·ÙÚÈ΋ 2006;69:193-198

ÎÒ‰Èη ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÈÙ¢¯ı› ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ÚfiÏË„Ë Î·È ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜. ∏ ÂÊ·ÚÌÔÁ‹ ÛÙËÓ Î·ıËÌÂÚÈÓ‹ È·ÙÚÈ΋ Ú·ÎÙÈ΋ ÙˆÓ Ô‰ËÁÈÒÓ ·˘ÙÒÓ ÚÔ¸Ôı¤ÙÂÈ ÙË Û˘Ó¯‹ ÂÓË̤ڈÛË, ÂÎ·›‰Â˘ÛË Î·È Î·Ù·ÓfiËÛË ÂΠ̤ÚÔ˘˜ ÙˆÓ ıÂÚ·fiÓÙˆÓ È·ÙÚÒÓ ÙˆÓ ‰ÈÂıÓÒÓ ÔÌÔʈÓÈÒÓ (9). ∏ ·ÚÔ‡Û· ÌÂϤÙË Î·Ù¤‰ÂÈÍ ÙËÓ ÏËÌÌÂÏ‹ Û˘ÌÌfiÚʈÛË ÙˆÓ ∂ÏÏ‹ÓˆÓ È·ÙÚÒÓ ÛÙȘ ÔÌÔʈӛ˜ Ù˘ GINA, ÙfiÛÔ ÛÙÔÓ ÙÔ̤· Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ fiÛÔ Î·È Ù˘ ıÂÚ·›·˜ ÙˆÓ ·ÛıÌ·ÙÈÎÒÓ ·È‰ÈÒÓ, ÁÂÁÔÓfi˜ ÙÔ ÔÔ›Ô ¤¯ÂÈ ‰È·ÈÛÙˆı› Î·È Û ·ÓÙ›ÛÙÔȯ˜ ‰ÈÂıÓ›˜ ÌÂϤÙ˜, fiÔ˘ Ù· ÔÛÔÛÙ¿ ÂÊ·ÚÌÔÁ‹˜ ÙˆÓ ‰ÈÂıÓÒÓ Ô‰ËÁÈÒÓ Î˘Ì¿ÓıËÎ·Ó ·fi 10 ¤ˆ˜ 65% (10,11). ¶·Ú¿ ÙËÓ ÏËÌÌÂÏ‹ ÂÊ·ÚÌÔÁ‹, ÔÈ ŒÏÏËÓ˜ È·ÙÚÔ› ‰ËÏÒÓÔ˘Ó ÂÓËÌÂڈ̤ÓÔÈ ˆ˜ ÚÔ˜ ÙÔ ÂÚȯfiÌÂÓÔ ÙˆÓ Ô‰ËÁÈÒÓ Ù˘ GINA Û ÔÛÔÛÙfi >80%, ÔÛÔÛÙfi ·Ó¿ÏÔÁÔ ‹ ÌÂÁ·Ï‡ÙÂÚÔ ·fi ÚÔËÁÔ‡ÌÂÓ˜ ÌÂϤÙ˜ Ù˘ ∂˘ÚÒ˘ Î·È ÙˆÓ ∏.¶.∞. (12-15). ∂ÈÚfiÛıÂÙ·, Ë ÏÂÈÔ„ËÊ›· ÙˆÓ È·ÙÚÒÓ Â›Ó·È Û‡ÌʈÓË Ì ÙÔ ÂÚȯfiÌÂÓÔ ÙˆÓ Ô‰ËÁÈÒÓ Ù˘ GINA Î·È ¤¯ÂÈ ÂÈÛı› ÁÈ· ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ¿ ÙÔ˘˜. ∂ȉÈο fiÛÔÓ ·ÊÔÚ¿ ÛÙË ¯ÔÚ‹ÁËÛË ÂÈÛÓÂfiÌÂÓˆÓ ÎÔÚÙÈÎÔÂȉÒÓ Û ·È‰È¿ Ì Â›ÌÔÓÔ ¿ÛıÌ·, Ù· ¯·ÌËÏ¿ ÔÛÔÛÙ¿ ÂÊ·ÚÌÔÁ‹˜ Ù˘ Ô‰ËÁ›·˜ Ê·›ÓÂÙ·È Ó· ÔÊ›ÏÔÓÙ·È Û ÌÂȈ̤ÓË ¿ÓÂÛË ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ Ô‰ËÁ›·˜. ∆Ô Ê·ÈÓfiÌÂÓÔ ·˘Ùfi ·Ó·Ê¤ÚÂÙ·È Î·È Û ¯ÒÚ˜ Ù˘ ¢˘ÙÈ΋˜ ∂˘ÚÒ˘ (™Ô˘Ë‰›·, °·ÏÏ›·, √ÏÏ·Ó‰›·, ∏ӈ̤ÓÔ

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·197

197

™˘ÌÌfiÚʈÛË ÛÙȘ ηÙ¢ı˘ÓÙ‹ÚȘ Ô‰ËÁ›Â˜

¶ÔÛÔÛÙfi ÂÊ·ÚÌÔÁ‹˜ Ù˘ Ô‰ËÁ›·˜

67

°ÓÒÛË Ù˘ Ô‰ËÁ›·˜

83

£ÂÙÈ΋ ÂÓÙ‡ˆÛË ÁÈ· ÙËÓ Ô‰ËÁ›·

95

ÕÓÂÛË ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ Ô‰ËÁ›·˜

72

™˘Ìʈӛ· Ì ÙËÓ Ô‰ËÁ›·

90 0

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30

40

50

60

70

80

90

100

¶ÔÛÔÛÙfi (%)

∂ÈÎfiÓ· 3. ¶ÔÛÔÛÙ¿ Û‡ÛÙ·Û˘ ‰È·ÎÔ‹˜ ηÓ›ÛÌ·ÙÔ˜ ÛÙÔ˘˜ ÁÔÓ›˜ ·È‰ÈÒÓ Ì ¿ÛıÌ· Î·È ·Ú¿ÁÔÓÙ˜ Ô˘ Û˘Ì‚¿ÏÏÔ˘Ó ÛÙË Û˘ÌÌfiÚʈÛË ÙˆÓ ÁÈ·ÙÚÒÓ ÛÙËÓ Ô‰ËÁ›· ·˘Ù‹.

µ·Û›ÏÂÈÔ Î·È °ÂÚÌ·Ó›·) Î·È ÛÙȘ ∏.¶.∞. Ì ·Ó¿ÏÔÁ· ‹ ÌÈÎÚfiÙÂÚ· ÔÛÔÛÙ¿ (10,15,16). ∂ÈÚfiÛıÂÙ·, Û ÌÂϤÙË ·fi ÙȘ ∏.¶.∞., ηٷÁÚ¿ÊÂÙ·È fiÙÈ ÌfiÓÔ ÙÔ 40% ÙˆÓ ·È‰È¿ÙÚˆÓ ¯ÔÚËÁ› ÚÔfiÌÂÙÚÔ ÁÈ· ηıËÌÂÚÈÓ‹ ·Ú·ÎÔÏÔ‡ıËÛË ·È‰ÈÒÓ Ì ¿ÛıÌ·, ÔÛÔÛÙfi ¿ÓÙˆ˜ Ôχ ˘„ËÏfiÙÂÚÔ ·fi Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ (7%) (8,17). √È ·Ú·¿Óˆ ‰˘ÛÎÔϛ˜ Èı·ÓÒ˜ Û¯ÂÙ›˙ÔÓÙ·È Ì ·ÓÂ·Ú΋ Ú·ÎÙÈ΋ ÂÎ·›‰Â˘ÛË Ì ·ÔÙ¤ÏÂÛÌ· ÌÂȈ̤ÓË ¯Ú‹ÛË Ô‰ËÁÈÒÓ Ô˘ ··ÈÙÔ‡Ó ‰ÂÍÈÔÙ¯ÓÈÎÔ‡˜ ·Ú¿ ıˆÚËÙÈÎÔ‡˜ ¯ÂÈÚÈÛÌÔ‡˜. ∆Ô Û˘Ì¤Ú·ÛÌ· ·˘Ùfi, ÙÔ ÔÔ›Ô ‚¤‚·È· ı· Ú¤ÂÈ Ó· ÂȂ‚·Èˆı› Ì ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜, ˘Ô‰ËÏÒÓÂÈ ÙËÓ ·Ó¿ÁÎË ÔÚÁ¿ÓˆÛ˘ ÛÂÌÈÓ·Ú›ˆÓ Û˘Ó¯È˙fiÌÂÓ˘ È·ÙÚÈ΋˜ ÂÎ·›‰Â˘Û˘ Ì Ú·ÎÙÈÎfi ÂÚȯfiÌÂÓÔ. ™Â ·ÓÙ›ıÂÛË Ì ٷ ·Ú·¿Óˆ, ηٷÁÚ¿ÊËΠÛËÌ·ÓÙÈ΋ ¢·ÈÛıËÙÔÔ›ËÛË ÙˆÓ ∂ÏÏ‹ÓˆÓ È·ÙÚÒÓ fiÛÔÓ ·ÊÔÚ¿ ÛÙË Û˘¯ÓfiÙËÙ· ÂÓË̤ڈÛ˘ ÙˆÓ ÁÔÓ¤ˆÓ ÁÈ· ÙËÓ ·Ó·ÁηÈfiÙËÙ· ‰È·ÎÔ‹˜ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜ (ÔÛÔÛÙfi 67% ÙˆÓ ÂÈÛΤ„ˆÓ), ÔÛÔÛÙfi ˘„ËÏfiÙÂÚÔ ·fi ·˘Ùfi Ô˘ ¤¯ÂÈ ‰È·Ù˘ˆı› ·fi ÙÔ˘˜ Cabana Î·È Û˘Ó (<55%.) (8). ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi Â›Ó·È ÂӉȷʤÚÔÓ, ·Ó Ï¿‚ÂÈ Î·Ó›˜ ˘’ fi„ÈÓ ÙÔ˘ fiÙÈ Ë ∂ÏÏ¿‰· Â›Ó·È Ë ¯ÒÚ· Ù˘ ∂˘ÚÒ˘ Ì ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ηÓÈÛÙÒÓ (44% ÙˆÓ ÂÓËÏ›ÎˆÓ Î·È 10% ÙˆÓ ÂÊ‹‚ˆÓ Â›Ó·È Î·ÓÈÛÙ¤˜), ÔfiÙÂ Ë ·Ó·ÁηÈfiÙËÙ· ÁÈ· Ì›ˆÛË ÙÔ˘ ÔÛÔÛÙÔ‡ ÙˆÓ ·ÙfiÌˆÓ Ô˘ ηÓ›˙Ô˘Ó Ê·›ÓÂÙ·È ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ ÂÈÙ·ÎÙÈ΋ (18).

√È Doerschung Î·È Û˘Ó, ÙÔ 1999 Û ÌÂϤÙË ·ÍÈÔÏfiÁËÛ˘ Ù˘ ηٷÓfiËÛ˘ Î·È Ú·ÎÙÈ΋˜ ÙˆÓ È·ÙÚÒÓ ‰È·ÊfiÚˆÓ ÂȉÈÎÔًوÓ, ‰È·Ù‡ˆÛ·Ó fiÙÈ ÔÈ ÂÍÂȉÈÎÂ˘Ì¤ÓÔÈ È·ÙÚÔ› (Ó¢ÌÔÓÔÏfiÁÔÈ Î·È ·ÏÏÂÚÁÈÔÏfiÁÔÈ) ηٷÓÔÔ‡Ó Î·Ï‡ÙÂÚ· ÙȘ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜, ÂÓÒ ¿ÏϘ ÌÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó ÏÈÁfiÙÂÚ˜ ÂÈÛ·ÁˆÁ¤˜ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ÏfiÁˆ ¿ÛıÌ·ÙÔ˜ fiÙ·Ó ÔÈ ·ÛıÂÓ›˜ ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È ·fi ÂÍÂȉÈÎÂ˘Ì¤ÓÔ˘˜ È·ÙÚÔ‡˜ (6,19,20). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, ·ÚÔ˘ÛÈ¿ÛÙËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÌfiÓÔ ÛÙ· ÔÛÔÛÙ¿ ¯ÔÚ‹ÁËÛ˘ ÚÔÔ̤ÙÚÔ˘ ÁÈ· ηıËÌÂÚÈÓ‹ ·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ ·ÛıÌ·ÙÈÎÒÓ ·È‰ÈÒÓ, ÁÂÁÔÓfi˜ Ô˘ Èı·ÓÒ˜ ˘Ô‰ËÏÒÓÂÈ ÙÔÓ ÈηÓÔÔÈËÙÈÎfi ‚·ıÌfi ¢·ÈÛıËÙÔÔ›ËÛ˘ ÙˆÓ ∂ÏÏ‹ÓˆÓ ·È‰È¿ÙÚˆÓ, ÙÔ˘Ï¿¯ÈÛÙÔÓ Û οÔȘ ·fi ÙȘ ‰ÈÂıÓ›˜ Ô‰ËÁ›Â˜. ∂›Ó·È ‚¤‚·È· ۷ʤ˜ fiÙÈ ÙÔ ‰Â›ÁÌ· Ù˘ ÌÂϤÙ˘ ‰ÂÓ ÌÔÚ› Ó· ıˆÚËı› ·ÓÙÈÚÔÛˆ¢ÙÈÎfi fiÏˆÓ ÙˆÓ È·ÙÚÒÓ, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ·ÔÙÂÏ›ÙÔ ·fi È·ÙÚÔ‡˜ Ô˘ ›¯·Ó ÙÔ ÂӉȷʤÚÔÓ Ó· ·Ú·ÎÔÏÔ˘ı‹ÛÔ˘Ó ÌÈ· ÂÈÛÙËÌÔÓÈ΋ ÂΉ‹ÏˆÛË ÂȉÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜. ∏ ‰È·‰Èηۛ· ·˘Ù‹ ‰ÂÈÁÌ·ÙÔÏË„›·˜ Î·È Ô Û¯ÂÙÈο ÌÈÎÚfi˜ ·ÚÈıÌfi˜ ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜, ·ÔÙÂÏÔ‡Ó ÙÔ˘˜ ÂÚÈÔÚÈÛÌÔ‡˜ Ù˘ ÌÂϤÙ˘. ™Â οı ÂÚ›ÙˆÛË fï˜, Ë ÌÂϤÙË ·˘Ù‹ ·ÔÙÂÏ›, ÂÍ fiÛˆÓ ÁÓˆÚ›˙Ô˘ÌÂ, ÙËÓ ÚÒÙË Û˘ÛÙËÌ·ÙÈ΋ ÚÔÛ¿ıÂÈ· ηٷÁÚ·Ê‹˜ Ù˘ ÁÓÒÛ˘ Î·È Ù˘ Û˘ÌÌfiÚʈÛ˘ ∂ÏÏ‹ÓˆÓ È·ÙÚÒÓ ÛÙȘ ‰ÈÂıÓ›˜ ÔÌÔʈӛ˜ ÁÈ· ÙÔ ¿ÛıÌ·. ¶·È‰È·ÙÚÈ΋ 2006;69:193-198

Pediatr May-June 06

16-05-06

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198

¶. •Â··‰¿ÎË Î·È Û˘Ó.

™˘ÌÂÚ·ÛÌ·ÙÈο, Ù· ‰Â‰Ô̤ӷ Ù˘ ÌÂϤÙ˘ Ì·˜ ˘Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ ·Ú¿ ÙËÓ ÈηÓÔÔÈËÙÈ΋ ηٷÓfiËÛË Î·È ÁÓÒÛË ÙˆÓ ‰ÈÂıÓÒÓ ÔÌÔʈÓÈÒÓ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ·fi ÙÔ˘˜ ŒÏÏËÓ˜ ·È‰È¿ÙÚÔ˘˜, Ë ÂÊ·ÚÌÔÁ‹ ÙˆÓ ·Ú·¿Óˆ Ô‰ËÁÈÒÓ ·Ú·Ì¤ÓÂÈ ÏËÌÌÂÏ‹˜. ∏ Û˘Ó¯‹˜ ÂÓË̤ڈÛË Î·È ÂÎ·›‰Â˘ÛË, Î·È Ì¿ÏÈÛÙ· Ì Ú·ÎÙÈÎfi ÂÚȯfiÌÂÓÔ, ı· Ú¤ÂÈ Ó· ·ÔÙÂÏ› ÛÙfi¯Ô ÙfiÛÔ ÙˆÓ È·ÙÚÒÓ fiÛÔ Î·È ÙˆÓ ÂÍÂȉÈÎÂ˘Ì¤ÓˆÓ ÎÏÈÓÈÎÒÓ Î·È ÙÌËÌ¿ÙˆÓ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÈÙ¢¯ı› ÏËÚ¤ÛÙÂÚË ÁÓÒÛË Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ¯Ú‹ÛË ÙˆÓ ‰ÈÂıÓÒÓ Ô‰ËÁÈÒÓ Î·È Û˘ÓÂ·ÎfiÏÔ˘ıË ‚ÂÏÙ›ˆÛË Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜.

µÈ‚ÏÈÔÁÚ·Ê›· 1. WHO Media centre. Fact sheets. Bronchial Asthma.

[Webpage] Fact sheet NÆ206. Revised Jan 2000: http://www.who.int/mediacentre/factsheets/fs206/e n/index.html 2. Global Initiative for asthma (GINA). Global strategy for asthma management and prevention.

NHLBI/WHO workshop report. National Institutes of Health NH, Lung and Blood institute. In: 02-3659 PN, editor. February 2002.

3. Sears MR. Epidemiology of childhood asthma.

Lancet 1997;350:1015-1020.

4. Anthracopoulos M, Karatza A, Liolios E, Triga M, Triantou K, Priftis K. Prevalence of asthma among schoolchildren in Patras, Greece: three surveys over 20 years. Thorax 2001;56:569-571.

5. Goeman DP, Hogan CD, Aroni RA, Abramson MJ, Sawyer SM, Stewart K et al. Barriers to delivering asthma care: a qualitative study of general practitioners. Med J Aust 2005;183:457-460.

6. Legorreta AP, Christian-Herman J, O'Connor RD, Hasan MM, Evans R, Leung KM. Compliance with national asthma management guidelines and specialty care: a health maintenance organization experience. Arch Intern Med 1998;158:457-464.

7. Nurmi J, Rosenberg P, Castren M. Adherence to guidelines when positioning the defibrillation electrodes. Resuscitation 2004;61:143-147.

8. Cabana MD, Rand CS, Becher OJ, Rubin HR. Rea-

¶·È‰È·ÙÚÈ΋ 2006;69:193-198

9.

10.

11.

12.

13.

14.

15.

16.

17.

18.

19.

20.

sons for pediatrician nonadherence to asthma guidelines. Arch Pediatr Adolesc Med 2001;155:1057-1062.

Doerschug KC, Peterson MW, Dayton CS, Kline JN. Asthma guidelines: an assessment of physician understanding and practice. Am J Respir Crit Care Med 1999;159:1735-1741.

Rabe KF, Vermeire PA, Soriano JB, Maier WC. Clinical management of asthma in 1999: the Asthma Insights and Reality in Europe (AIRE) study. Eur Respir J 2000;16:802-807.

Portnoy JM. Barriers to asthma treatment adherence. Curr Allergy Asthma Rep 2005;5:430.

Lomas J. Words without action? The production, dissemination, and impact of consensus recommendations. Ann Rev Public Health 1991;12:41-65.

Crain EF, Weiss KB, Fagan MJ. Pediatric asthma care in US emergency departments. Current practice in the context of the National Institutes of Health guidelines. Arch Pediatr Adolesc Med 1995;149:893-901.

Finkelstein JA, Lozano P, Shulruff R, Inui TS, Soumerai SB, Ng M et al. Self-reported physician practices for children with asthma: are national guidelines followed? Pediatrics 2000;106:886-896.

Cabana MD, Ebel BE, Cooper-Patrick L, Powe NR, Rubin HR, Rand CS. Barriers pediatricians face when using asthma practice guidelines. Arch Pediatr Adolesc Med 2000;154:685-693.

Piecoro LT, Potoski M, Talbert JC, Doherty DE. Asthma prevalence, cost, and adherence with expert guidelines on the utilization of health care services and costs in a state Medicaid population. Health Serv Res 2001;36:357-371.

Garcia-Marcos L, Castro-Rodriguez JA, Montaner AE, Garde JG, Bernabe JJ, Belinchon JP. The use of spirometers and peak flow meters in the diagnosis and management of asthma among Spanish pediatricians. Results from the TRAP study. Pediatr Allergy Immunol 2004;15:365-371. °ÎÚ¿Ù˙ÈÔ˘ Ã. ∆Ô Î¿ÓÈÛÌ· ˆ˜ ÂÍ¿ÚÙËÛË, ÂÎÙ›ÌËÛË Î·È ·ÓÙÈÌÂÙÒÈÛË. 19th International Meeting on Clinical Cardiology 2004; Athens, Greece.

Hernandez DV, Schmaling KB. Understanding and resolving adherence problems. Clin Rev Allergy Immunol 2004;27:65-73.

Schatz M, Cook EF, Nakahiro R, Petitti D. Inhaled corticosteroids and allergy specialty care reduce emergency hospital use for asthma. J Allergy Clin Immunol 2003;111:503-508.

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∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞

ORIGINAL ARTICLE

199

∂›‰· ÛÂÏËÓ›Ô˘ Î·È ¯·ÏÎÔ‡ ·›Ì·ÙÔ˜ ÌËÙ¤ÚˆÓ Î·È ÓÂÔÁÓÒÓ ÂÏÏËÓÈ΋˜ Î·È ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ∫. ™Ô‡ÏË1, ™. °·‚Ú›ÏË2, £. ∫·Ú·ÎˆÓÛÙ·ÓÙ¿Î˘3, °. ∫·Ú›Î·˜4, °. µÏ¿¯Ô˜5, Ã. ∫ÒÛÙ·ÏÔ˜2, π. ¶··ÛˆÙËÚ›Ô˘3

¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: ™ÎÔfi˜ Ù˘ ÂÚÁ·Û›·˜ ‹Ù·Ó Ë ÂÎÙ›ÌËÛË ÙˆÓ ÂÈ¤‰ˆÓ ÛÂÏËÓ›Ô˘ (Se) Î·È ¯·ÏÎÔ‡ (Cu) ÛÙÔÓ ÔÚfi ÌËÙ¤ÚˆÓ Î·È ÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ ÓÂÔÁÓÒÓ ÂÏÏËÓÈ΋˜ Î·È ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ∆· Â›‰· ÙÔ˘ Se Î·È Cu ÌÂÙÚ‹ıËÎ·Ó ÛÙÔÓ ÔÚfi 1118 EÏÏËÓ›‰ˆÓ Î·È 820 AÏ‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ Î·È ÛÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ Ì ÙȘ ÌÂıfi‰Ô˘˜ ÊÔ‡ÚÓÔ˘ ÁÚ·Ê›ÙË Î·È ·ÙÔÌÈ΋˜ ·ÔÚÚfiÊËÛ˘, ·ÓÙ›ÛÙÔȯ·. ∂›Û˘, ·Ó·Ï‡ıËÎ·Ó Ù· Â› 30 Ë̤Ú˜ ηٷÁÚ·ÊfiÌÂÓ· ‰È·ÈÙÔÏfiÁÈ·, Ù· ÔÔ›· ¤‰ÂÈÍ·Ó ÌÂȈ̤ÓË ÚfiÛÏË„Ë ˙ˆÈ΋˜ ÚÔ¤Ï¢Û˘ ÚˆÙ½Ó˘, Se Î·È Cu ÛÙËÓ ÔÌ¿‰· ÙˆÓ ∞Ï‚·Ó›‰ˆÓ. ∞ÔÙÂϤÛÌ·Ù·: ∆· Â›‰· Se ÛÙÔÓ ÔÚfi ÙˆÓ ∂ÏÏËÓ›‰ˆÓ (68,3±8,5 Ìg/l) Î·È ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ (37,0±8,9 Ìg/l) ‹Ù·Ó ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· (p<0,001) Û˘ÁÎÚÈÙÈο Ì ÂΛӷ ÙˆÓ ∞Ï‚·Ó›‰ˆÓ (37,4±9,9 Ìg/l) Î·È ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ (34,3±9,1 Ìg/l). ∂›Û˘, Ù· Â›‰· Cu ÔÚÔ‡ ÛÙȘ ∂ÏÏËÓ›‰Â˜ (1687±353 Ìg/l) Î·È Ù· ÓÂÔÁÓ¿ ÙÔ˘˜ (449±87 Ìg/l) ‹Ù·Ó ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· (p<0,001) ·fi ÂΛӷ ÙˆÓ AÏ‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ (959±318 Ìg/l) Î·È ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ (229±67 Ìg/l). ∞ÍÈÔÛËÌ›ˆÙÔ Â›Ó·È fiÙÈ ÛÙÔ 34,5% ÙˆÓ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ÓÂÔÁÓÒÓ, Ô˘ ÁÂÓÓ‹ıËÎ·Ó ·fi ÌËÙ¤Ú˜ Ì Â›‰· Se<28 Ìg/l ÛÙÔÓ ÔÚfi ÙÔ˘˜, Ù· Â›‰· Se ‚Ú¤ıËÎ·Ó Ó· Â›Ó·È ˘„ËÏfiÙÂÚ· ·fi ÂΛӷ ÙˆÓ ÌËÙ¤ÚˆÓ ÙÔ˘˜. ∆Ô ·ÓÙ›ÛÙÔÈ¯Ô ‚Ú¤ıËΠÛÙÔ 3,1% ÙˆÓ ÓÂÔÁÓÒÓ EÏÏËÓ›‰ˆÓ ÌËÙ¤ÚˆÓ. ™˘ÌÂÚ¿ÛÌ·Ù·: ∆· ¯·ÌËÏ¿ Â›‰· Se Î·È Cu Ô˘ ÌÂÙÚ‹ıËÎ·Ó ÛÙÔÓ ÔÚfi ÙˆÓ AÏ‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ Î·È ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ù˘ ¯·ÌËÏ‹˜ ÚfiÛÏ˄˘ ˙ˆÈ΋˜ ÚÔ¤Ï¢Û˘ ÚˆÙ½Ó˘ ÏfiÁˆ ¯·ÌËÏÔ‡ ÎÔÈÓˆÓÈÎÔ‡ Î·È ÔÈÎÔÓÔÌÈÎÔ‡ ÂÈ¤‰Ô˘ ‹ ‰È·ÈÙËÙÈ΋˜ Û˘Ó‹ıÂÈ·˜.

1 πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡, ¡ÔÛ. ¶·›‰ˆÓ “∞Á. ™ÔÊ›·”, ∞ı‹Ó· 2 NÂÔÁÓÔÏÔÁÈÎfi, ¡ÔÛ. “∞ÏÂÍ¿Ó‰Ú·” ∞ı‹Ó· 3 ∫ÏÈÓÈ΋ µÈÔ¯ËÌ›·, ¡ÔÛ/ÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· 4 ªÔÓ¿‰· º·ÚÌ·ÎÔÎÈÓËÙÈ΋˜ Î·È ∂Ó‰ÔÊÏ‚›ˆÓ ¢È·Ï˘Ì¿ÙˆÓ, ¡ÔÛ. ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· 5 ¶·ÓÂÈÛÙËÌȷ΋ ª·È¢ÙÈ΋ ∫ÏÈÓÈ΋ ¡ÔÛÔÌ›Ԣ “∞ÏÂÍ¿Ó‰Ú·”, ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: ∫ÏÂÔ¿ÙÚ· ™Ô‡ÏË E-mail: inchildh@otenet.gr ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 17-11-2004 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 10-02-2006

§¤ÍÂȘ ÎÏÂȉȿ: ™ÂÏ‹ÓÈÔ, ¯·ÏÎfi˜, ã∂ÏÏËÓ˜, ∞Ï‚·ÓÔ›, ·ËÛË, ÓÂÔÁÓ¿.

Maternal and neonatal blood selenium and copper concentrations in Greeks and Albanians K.H. Schulpis1, S. Gavrili2, T. Karakonstantakis3, G. Karikas4, G. Vlachos5, C. Costalos2, J. Papassotiriou3

Abstract Background: The aim of this study was to evaluate the concentration of Selenium (Se) and Copper (Cu)

in the venous blood of native Greek and immigrant Albanian mothers and in the cord blood of their babies.

Methods: Venous blood was taken from 1,118 Greek and 820 Albanian mothers and cord blood was

collected at birth from their babies. The concentration of Se and Cu was determined in the serum using graphite furnace atomic absorption spectroscopy (GFAAS) and atomic absorption spectrometry, respectively. The 30-day nutrient intake was evaluated in both groups of mothers.

Results: The intake of animal protein, Se and Cu was poor in the Albanian compared to the Greek mothers (p<0.001). The serum levels of Se were higher in the Greek mothers (68.3±8.5 Ìg/L) and in their babies’ cord blood (37.02±8.9 Ìg/L) than in the Albanian mothers (37.4±9.9 Ìg/L) and their babies (34.3±9.1 Ìg/L) (p<0.001). The serum Cu levels were also higher in the Greek mothers (1687±353 Ìg/L) and in their babies’ cord blood (449±87 Ìg/L) than in the Albanian mothers (959±318 Ìg/L) and their babies (229±67Ìg/L) (p<0.001). In the cord blood of 34.5% of the Albanian babies the Se levels were higher compared to those of their mothers, who had extremely low (< 28 Ìg/L) serum levels of Se. This phenomenon was observed in 3.1% of the Greek mother-baby pairs.

Conclusions: The low serum levels of Se and Cu found in the Albanian mothers and their newborn babies could be related to their poor animal protein intake as a consequence of their low socio-economic status and/or their nutritional habits.

1 Institute of Child’s Health, “Agia Sofia” Children’s Hospital, Athens 2 Neonatology Department, “Alexandra” Hospital, Athens 3 Clinical Biochemistry, “Agia Sofia” Children’s Hospital, Athens 4 Pharmacokinetics and Intravenous Solutions Unit, “Agia Sofia” Children’s Hospital, Athens 5 University Obstetric Clinic, “Alexandra” Hospital, Athens Correspondence: K.H. Schulpis E-mail: inchildh@otenet.gr Date of submission: 17-11-2004 Date of approval: 10-02-2006

Key words: Selenium, copper, pregnancy, neonates, Greeks, Albanians. ¶·È‰È·ÙÚÈ΋ 2006;69:199-204

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200

∫. ™Ô‡ÏË Î·È Û˘Ó.

∂ÈÛ·ÁˆÁ‹ ∏ ÂÁ΢ÌÔÛ‡ÓË Â›Ó·È Ì›· ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô˜ ·˘ÍËÌ¤ÓˆÓ ·ÏÏ·ÁÒÓ ÛÙÔÓ ÌÂÙ·‚ÔÏÈÛÌfi, Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È ÛÙË Ê˘ÛÈÔÏÔÁ›· Ù˘ Á˘Ó·›Î·˜ Î·È ÙÔ˘ ·Ó·Ù˘ÛÛfiÌÂÓÔ˘ ÂÌ‚Ú‡Ô˘ (1,2). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ΢ÔÊÔÚ›·˜, Ë ÂÏ·Ùو̤ÓË ÚfiÛÏË„Ë ÙˆÓ ·Ó·Áη›ˆÓ ‰È·ÙÚÔÊÈÎÒÓ ÛÙÔȯ›ˆÓ ·fi ÙË ÌËÙ¤Ú·, ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÌÊ¿ÓÈÛË ·Ó·ÈÌ›·˜, ˘¤ÚÙ·Û˘ Î.¿. Ô˘ ı· ÌÔÚÔ‡Û·Ó Ó· ‰ËÌÈÔ˘ÚÁ‹ÛÔ˘Ó ÚÔ‚Ï‹Ì·Ù· ÛÙÔ ¤Ì‚Ú˘Ô, fiˆ˜ ‰È·Ì·Úٛ˜ ÂÚ› ÙË ‰È¿Ï·ÛË, ÚÔˆÚfiÙËÙ·, ÌÂȈ̤ÓË ÂÓ‰ÔÌ‹ÙÚÈÔ ·Ó¿Ù˘ÍË Î.¿. (2,3). ∆Ô ÛÂÏ‹ÓÈÔ (Se) Â›Ó·È ¤Ó· ÛËÌ·ÓÙÈÎfi ȯÓÔÛÙÔÈ¯Â›Ô ÁÈ· ÙËÓ ˘Á›· ÙÔ˘ ·ÓıÚÒÔ˘. ∏ ÛÔ˘‰·ÈfiÙËÙ¿ ÙÔ˘ ‹Ù·Ó ¿ÁÓˆÛÙË ¤ˆ˜ ÙÔ 1957, fiÙ·Ó ÔÈ Schawartz Î·È Folz ·¤‰ÂÈÍ·Ó fiÙÈ Ë ÌÂȈ̤ÓË ÚfiÛÏË„Ë Se ·fi ıËÏ·ÛÙÈο ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÌÊ¿ÓÈÛË ÓÂÎÚˆÙÈ΋˜ ÂÎʇÏÈÛ˘ ÙÔ˘ ‹·ÙÔ˜, ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜, Ù˘ ηډȿ˜ Î·È ÙˆÓ ÓÂÊÚÒÓ (4-7). ™ÙÔÓ ¿ÓıÚˆÔ ÙÔ Se Â›Ó·È ··Ú·›ÙËÙÔ ÛÙË ÏÂÈÙÔ˘ÚÁ›· ÔÏÏÒÓ ÂÓ˙‡ÌˆÓ Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Ù˘ ˘ÂÚÔÍÂȉ¿Û˘ Ù˘ ÁÏÔ˘Ù·ıÂÈfiÓ˘ Ô˘ ÚÔÛٷهÂÈ ·fi ÙË ‚Ï·ÙÈ΋ ‰Ú¿ÛË ÙÔ˘ ÔÍÂȉˆÙÈÎÔ‡ ÛÙÚ˜. ™ÙÔÓ ÓÂÔÁÓÈÎfi ÏËı˘ÛÌfi ¤¯ÂÈ ‚ÚÂı› fiÙÈ ÔÈ ÂχıÂÚ˜ Ú›˙˜ ÌÔÚÔ‡Ó Ó· ÚÔηϤÛÔ˘Ó Ó¢ÚÔÏÔÁÈ΋ ‚Ï¿‚Ë (4,5,7). ∆· Â›‰· Se ÛÙÔ ·›Ì· ÂÍ·ÚÙÒÓÙ·È ¿ÌÂÛ· ·fi ÙËÓ ÂÚÈÂÎÙÈÎfiÙËÙ¿ ÙÔ˘ ÛÙȘ ÚÔÛÏ·Ì‚·ÓfiÌÂÓ˜ ÙÚÔʤ˜. ∆· Â›‰· ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ ÂÍ·ÚÙÒÓÙ·È ·fi ÙËÓ ÂÚÈÂÎÙÈÎfiÙËÙ· Se ÙÔ˘ ‰¿ÊÔ˘˜ ·fi fiÔ˘ ÚÔ¤Ú¯ÔÓÙ·È (6-8). ∆ÂÏÂ˘Ù·›·, ÈÛÙ‡ÂÙ·È fiÙÈ Ô x·ÏÎfi˜ (Cu) Â›Ó·È ¤Ó· ·fi Ù· 15 ··Ú·›ÙËÙ· ȯÓÔÛÙÔȯ›· ÁÈ· ÙÔÓ ¿ÓıÚˆÔ Î·È ·ÓȯÓ‡ÂÙ·È Û ÔÏϤ˜ ÂÚÈÔ¯¤˜ ÙÔ˘ ∫¡™. ™ÙȘ Ó¢ÚÈΤ˜ ·ÔÏ‹ÍÂȘ, Ô Cu ¯ÚËÛÈ̇ÂÈ ÛÙËÓ fiψÛË Î·È ·ÔfiψÛË Ù˘ ÌÂÌ‚Ú¿Ó˘ Î·È ÌÔÚ› Ó· Êı¿ÛÂÈ Ù· 100-300 mM ÛÙȘ ÂÍˆÎ˘ÙÙ¿ÚȘ Û˘ÁÎÂÓÙÚÒÛÂȘ ÙÔ˘ (911). ∂›Û˘, Ô Cu ÂÌϤÎÂÙ·È Û Ó¢ÚÔ·ıÔÏÔÁÈΤ˜ ÓfiÛÔ˘˜, fiˆ˜ ÙË ÓfiÛÔ Menke, ÙË ÓfiÛÔ Wilson Î·È ÙËÓ ÂÌÊ¿ÓÈÛË ÂÈÏËÙÈÎÒÓ ÎÚ›ÛˆÓ. ∆Ô Ì¤Ù·ÏÏÔ ÂÌÂÚȤ¯ÂÙ·È Û ÔÏÏ¿ ÌÂÙ·ÏÏÔ¤Ó˙˘Ì· Î·È Â›Ó·È Ì¤ÚÔ˜ ÔÏÏÒÓ ·ÓÙÈÔÍÂȉˆÙÈÎÒÓ ÂÓ˙‡ÌˆÓ. ÷ڷÎÙËÚÈÛÙÈÎfi ·Ú¿‰ÂÈÁÌ· ·ÔÙÂÏ› Ë ˘ÂÚÔÍÂȉˆÙÈ΋ ‰˘ÛÌÔ˘Ù¿ÛË, ÁÓˆÛÙ‹ ÁÈ· ÙË ‰Ú¿ÛË Ù˘ ÛÙÔ Â›Â‰Ô ÌÂÙ·ÙÚÔ‹˜ Ù˘ ÓÔÚ·‰ÚÂÓ·Ï›Ó˘ Û ÓÙÔ·Ì›ÓË ÛÙÔÓ ÂÁΤʷÏÔ. £ÂˆÚÂ›Ù·È Â›Û˘ ‰Â›ÎÙ˘ ηϋ˜ Ï·ÎÔ˘ÓÙȷ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (11). ◊‰Ë ·fi ÙÔ 1991, ¿Ú¯ÈÛ ӷ ÂÈÛÚ¤ÂÈ ¤Ó· ÌÂÁ¿ÏÔ ÌÂÙ·Ó·ÛÙ¢ÙÈÎfi ·̷ ·fi ÙËÓ ÁÂÈÙÔÓÈ΋ ∞Ï‚·Ó›·. ∂Âȉ‹ οı ¯ÒÚ· ¤¯ÂÈ ÙÔ˘˜ ‰ÈÎÔ‡˜ Ù˘ ¶·È‰È·ÙÚÈ΋ 2006;69:199-204

‰È·ÙÚÔÊÈÎÔ‡˜ ηÓfiÓ˜ Î·È Û˘Ó‹ıÂȘ, ıˆڋ۷Ì ÂӉȷʤÚÔÓ Ó· ÌÂÏÂÙ‹ÛÔ˘Ì ٷ Â›‰· ÙÔ˘ Se Î·È ÙÔ˘ Cu ÛÙÔÓ ÔÚfi ÙˆÓ ∂ÏÏËÓ›‰ˆÓ ÌËÙ¤ÚˆÓ, ÙˆÓ ∞Ï‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ Î·È ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∏ ÌÂϤÙË ¤ÁÈÓ ‰ÂÎÙ‹ ·fi ÙÔ ∂ÈÛÙËÌÔÓÈÎfi ™˘Ì‚Ô‡ÏÈÔ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ “∞ÏÂÍ¿Ó‰Ú·”. ∫·Ù¿ ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· 1˘ π·ÓÔ˘·Ú›Ô˘ 2001 ¤ˆ˜ 31Ë ¢ÂÎÂÌ‚Ú›Ô˘ 2002, Û˘ÁÎÂÓÙÚÒıËÎ·Ó 2020 ‰Â›ÁÌ·Ù· ÔÚÒÓ ·fi ÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ ÓÂÔÁÓÒÓ Ô˘ ÏËÚÔ‡Û·Ó Ù· ·Ú·Î¿Ùˆ ÎÚÈÙ‹ÚÈ·: ·) ÌÔÓ‹Ú˘ ·ËÛË, ‚) ÔÌ·Ïfi˜ ÙÔÎÂÙfi˜, Á) ËÏÈΛ· ·ËÛ˘ 37˘-41˘ ‚‰ÔÌ¿‰·˜, ‰) ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ 2500 g - 4000 g, Â) ηϋ ˘Á›· ÌËÙ¤ÚˆÓ Î·ı’ fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘. ∞fi Ù· ÚÔ·Ó·ÊÂÚfiÌÂÓ· 2020 ‰Â›ÁÌ·Ù· ÔÌÊ·Ï›Ô˘ ÏÒÚÔ˘ ÌfiÓÔÓ Ù· 1938 ÏËÚÔ‡Û·Ó ÙȘ Û˘ÁÎÂÎÚÈ̤Ó˜ ÚÔ¸Ôı¤ÛÂȘ Î·È Ï‹Úˆ˜ ηٷÁÚ·Ì̤ӷ ÛÙÔȯ›·. ∆· ‰Â›ÁÌ·Ù· ·ÊÔÚÔ‡Û·Ó ∂ÏÏËÓ›‰Â˜ (¡=1118) Î·È ∞Ï‚·Ó›‰Â˜ (¡=820) ÌËÙ¤Ú˜ Ô˘ ˙Ô‡Û·Ó ÛÙË ¯ÒÚ· ηٿ Ù· ÙÂÏÂ˘Ù·›· 5,8±1,2 ¯ÚfiÓÈ·. ∆· Ì·È¢ÙÈο ÙÔ˘˜ ÈÛÙÔÚÈο Î·È ÔÈ Û˘Óı‹Î˜ ÙÔÎÂÙÔ‡ ·Ó·Ï‡ıËÎ·Ó ·fi π·ÙÚÔ‡˜ Ù˘ ∞ã ª·È¢ÙÈ΋˜ - °˘Ó·ÈÎÔÏÔÁÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ÂÓÒ Ù· ÈÛÙÔÚÈο ÙˆÓ ÓÂÔÁÓÒÓ ·fi È·ÙÚÔ‡˜ ÙÔ˘ ¡ÂÔÁÓÔÏÔÁÈÎÔ‡ ∆Ì‹Ì·ÙÔ˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ “∞ÏÂÍ¿Ó‰Ú·”. ∞ÌÊfiÙÂÚ˜ ÔÈ ÔÌ¿‰Â˜ ÙˆÓ ÌËÙ¤ÚˆÓ ÂÏ¿Ì‚·Ó·Ó Û΢¿ÛÌ·Ù· Ûȉ‹ÚÔ˘. ªËÙ¤Ú˜ Ô˘ οÓÈ˙·Ó ‹ ·Ó¤ÊÂÚ·Ó Î¿ÔÈÔ ÓfiÛËÌ· ÛÙÔ ÈÛÙÔÚÈÎfi ÙÔ˘˜ ·ÔÎÏ›ÛıËÎ·Ó ·fi ÙË ÌÂϤÙË. ∏ ËÌÂÚ‹ÛÈ· ÚfiÛÏË„Ë ÙÚÔÊ‹˜, ÙÔ Â›‰Ô˜ Ù˘ Î·È ÙÔ ‚¿ÚÔ˜ Ù˘ ηÙÂÁÚ¿ÊËÛ·Ó Â› 30 Ë̤Ú˜ Û ¤ÓÙ˘· ‰È·ÈÙÔÏÔÁ›ˆÓ Î·È ·Ó·Ï‡ıËÎ·Ó ‚¿ÛË ›Ó·Î· (12). ∂ΛӘ ÔÈ ÌËÙ¤Ú˜ ÛÙȘ Ôԛ˜ ‰ÂÓ ‹Ù·Ó ‰˘Ó·ÙfiÓ Ó· ÚÔÛ‰ÈÔÚ›ÛÔ˘Ì ÙÔ Ï‹Ú˜ ‰È·ÈÙÔÏÔÁÈÎfi ÙÔ˘˜ ÈÛÙÔÚÈÎfi ηٿ ÙÔÓ ÚÔËÁÔ‡ÌÂÓÔ Ì‹Ó· Ù˘ ·ËÛ˘, ‰ÂÓ ÂÚÈÂÏ‹ÊıËÛ·Ó Û ·˘Ù‹ ÙË ÌÂϤÙË. ∫·Ù¿ ÙËÓ ¤Ó·ÚÍË ÙÔ˘ ÙÔÎÂÙÔ‡ Ï·Ì‚¿ÓÔÓÙ·Ó 3,0 ml ·›Ì·ÙÔ˜ ·fi ÙË ÌËÙ¤Ú· Î·È ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙÔÓ ÙÔÎÂÙfi 3,0 ml ·›Ì·ÙÔ˜ ·fi ÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ. ∆· ‰Â›ÁÌ·Ù· ·›Ì·ÙÔ˜ Ê˘ÁÔÎÂÓÙÚÔ‡ÓÙ·Ó Î·È ÔÈ ÔÚÔ› Ê˘Ï¿ÛÔÓÙ·Ó ÛÙÔ˘˜ -20ÆC ̤¯ÚÈ Ó· ·Ó·Ï˘ıÔ‡Ó. √ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙÔ˘ ÂÈ¤‰Ô˘ ÙÔ˘ Se ¤ÁÈÓ Û ÊÔ‡ÚÓÔ ÁÚ·Ê›ÙË (13) Î·È ÙÔ˘ Cu Ì ·ÙÔÌÈ΋ ·ÔÚÚfiÊËÛË (14).

™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ∆· Â˘Ú‹Ì·Ù· ·ÍÈÔÏÔÁ‹ıËÎ·Ó Ì ÙË Ì¤ıÔ‰Ô ANOVA ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙÔ ÛÙ·ÙÈÛÙÈÎfi ÚfiÁÚ·ÌÌ· SPSS 8.0. ∆È̤˜ p<0,05 ıˆڋıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜.

∞ÔÙÂϤÛÌ·Ù· Ÿˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ ¶›Ó·Î· 1, Ë ËÏÈΛ· ÙˆÓ ÌËÙ¤ÚˆÓ, Ô ¯ÚfiÓÔ˜ ·ËÛ˘, ÙÔ ‚¿ÚÔ˜ ÙÔ˘ Ï·ÎÔ‡ÓÙ· Î·È Ù· Â›‰· Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ ÛÙȘ ÌËÙ¤Ú˜ Î·È ÛÙ· ÓÂÔÁÓ¿ ‰ÂÓ ‰È¤ÊÂÚ·Ó

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·201

201

∂›‰· ÛÂÏËÓ›Ô˘ Î·È ¯·ÏÎÔ‡ ·›Ì·ÙÔ˜ ÌËÙ¤ÚˆÓ Î·È ÓÂÔÁÓÒÓ

¶›Ó·Î·˜ 1. ∫ÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ∂ÏÏËÓ›‰ˆÓ Î·È ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ÌËÙ¤ÚˆÓ Î·È ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ ∂ÏÏËÓ›‰Â˜ (N=1118) ∏ÏÈΛ· (¯ÚfiÓÈ·) Hb g/dl (ÌËÙ¤ÚˆÓ) Hb g/dl (ÓÂÔÁÓÒÓ) ÃÚ. ·ËÛ˘ (‚‰.) µ¿ÚÔ˜ Ï·ÎÔ‡ÓÙ· (g) µ¿ÚÔ˜ Á¤ÓÓËÛ˘ (g) º‡ÏÔ ÓÂÔÁÓÒÓ (·ÁfiÚÈ· %) (ÎÔÚ›ÙÛÈ· %)

∞Ï‚·Ó›‰Â˜ (N=820)

27,5 ± 3,4 26,8 ± 2,5 (18 - 31) (14 - 30) 12,3 ± 1,6 11,9 ± 1,8 (10,5 - 14,0) (10,0 - 13,8) 20,2 ± 1,8 19,8 ± 1,7 (18,4 - 22,0) (18,1 - 21,5) 39,5 ± 1,3 38,7 ± 1,4 (37 - 42) (36 - 41) 488,4 ± 70,5 499,3 ± 71,5 (410 - 560) (429 - 560) 3378 ± 386a 2898 ± 396b (2610 -3850) (2550 - 3280) 52 48

54 46

( ) = ·ÎÚ·›Â˜ ÙÈ̤˜ √È ÙÈ̤˜ ÂÎÊÚ¿˙ÔÓÙ·È: ª¤ÛË ÙÈÌ‹ ± ÛÙ·ıÂÚ¿ ·fiÎÏÈÛË a/b = ƒ<0,01

ÛÙ·ÙÈÛÙÈο. ∞ÓÙÈı¤Ùˆ˜, Ô Ì¤ÛÔ˜ fiÚÔ˜ ‚¿ÚÔ˘˜ Á¤ÓÓËÛ˘ ÙˆÓ ÓÂÔÁÓÒÓ ÙˆÓ ∂ÏÏËÓ›‰ˆÓ ÌËÙ¤ÚˆÓ ‹Ù·Ó ˘„ËÏfiÙÂÚÔ ·fi ÂΛÓÔ ÙˆÓ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ÓÂÔÁÓÒÓ. Ÿˆ˜ ·ÚÔ˘ÛÈ¿˙ÂÙ·È ÛÙÔÓ ¶›Ó·Î· 2, Ë ÚfiÛÏË„Ë ıÂÚÌ›‰ˆÓ, ˘‰·Ù·ÓıڿΈÓ, ÔÏÈÎÒÓ ÏÈÒÓ, ÎÂÎÔÚÂÛÌ¤ÓˆÓ ÏÈÒÓ Î·È ¯ÔÏËÛÙÂÚfiÏ˘, ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ˜ ÛÙȘ ·Ï‚·Ó›‰Â˜, Û˘ÁÎÚÈÙÈο Ì ÙȘ ·ÓÙ›ÛÙÔȯ˜ ‰È·ÙÚÔÊÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜ ÙˆÓ ∂ÏÏËÓ›‰ˆÓ ÌËÙ¤ÚˆÓ. ŸÌˆ˜, Ë Î·Ù·Ó¿ÏˆÛË Ù˘ ˙ˆÈ΋˜ ÚÔ¤Ï¢Û˘ ÚˆÙ½Ó˘, Ô˘ Â›Ó·È ÏÔ‡ÛÈ· Û ÂÚÈÂÎÙÈÎfiÙËÙ· Se Î·È Cu, ÙˆÓ ÌÔÓÔ·ÎfiÚÂÛÙˆÓ Î·È ÙˆÓ ÔÏ˘·ÎfiÚÂÛÙˆÓ ÏÈ·ÚÒÓ ÔͤˆÓ, fiˆ˜ Â›Û˘, Ë Î·Ù·Ó¿ÏˆÛË ÙˆÓ ÌÂÏÂÙÔ‡ÌÂÓˆÓ ÌÂÙ¿ÏÏˆÓ ‹Ù·Ó Ôχ ÛËÌ·ÓÙÈο ÌÂȈ̤ÓË ÛÙȘ ÌÂÙ·Ó¿ÛÙÚȘ ÌËÙ¤Ú˜ Û˘ÁÎÚÈÙÈο Ì ÂΛӘ ÙˆÓ ∂ÏÏËÓ›‰ˆÓ. £· Ú¤ÂÈ Ó· ÛËÌÂÈÒÛÔ˘Ì fiÙÈ ÌÂȈ̤ÓË ÚfiÛÏË„Ë Î·È ÛÙ· ‰‡Ô ̤ٷÏÏ· ÂÓÙÔ›ÛıËΠÛÙÔ 34,5% ÙˆÓ ∞Ï‚·Ó›‰ˆÓ Î·È ÛÙÔ 3,2% ÙˆÓ ∂ÏÏËÓ›‰ˆÓ ÌËÙ¤ÚˆÓ. ŸÛÔÓ ·ÊÔÚ¿ ÙÔ Se (¶›Ó·Î·˜ 3), Ù· Â›‰¿ ÙÔ˘ ‚Ú¤ıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· ÛÙÔÓ ÔÚfi ÙˆÓ ∂ÏÏËÓ›‰ˆÓ Û˘ÁÎÚÈÓfiÌÂÓ· Ì ÂΛӷ ÙˆÓ ∞Ï‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ. ∂›Û˘, ‹Ù·Ó ˘„ËÏfiÙÂÚ· ÛÙȘ ÌËÙ¤Ú˜ Û˘ÁÎÚÈÓfiÌÂÓ· Ì ÂΛӷ ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜. ŸÌˆ˜, ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ Se ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ¯·ÌËÏfiÙÂÚ˜ ÛÙÔ 3,1% ÙˆÓ ∂ÏÏËÓ›‰ˆÓ Î·È ÛÙÔ 34,5% ÙˆÓ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ÌËÙ¤ÚˆÓ ·fi ÂΛӘ ÙˆÓ ÓÂÔÁÓÒÓ

¶›Ó·Î·˜ 2. ¢È·ÙÚÔÊÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ‰È·ÈÙÔÏÔÁ›ˆÓ (30 ËÌÂÚÒÓ) ∂ÏÏËÓ›‰ˆÓ Î·È ∞Ï‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ ¢È·ÙÚÔÊ‹

∂ÏÏËÓ›‰Â˜ ∞Ï‚·Ó›‰Â˜ (N=1118) (N=820)

£ÂÚÌ›‰Â˜ (Kcal) √ÏÈ΋ ÚˆÙ½ÓË (g) ∑ˆÈ΋ ÚˆÙ½ÓË (g) º˘ÙÈ΋ ÚˆÙ½ÓË (g) À‰·Ù¿ÓıڷΘ (g) √ÏÈÎfi Ï›Ô˜ (g) ∫ÔÚÂṲ̂ÓÔ (g) ªÔÓÔ·ÎfiÚÂÛÙÔ (g) ¶ÔÏ˘·ÎfiÚÂÛÙÔ (g) ¶ÔÏ˘/ÎÔÚÂṲ̂ÓÔ (ËÏ›ÎÔ) ÃÔÏËÛÙÂÚfiÏË (mg) Se (RNI=75 Ìg) Cu (RNI=1000 Ìg)

2350 ± 150a 2970 ± 140a 64 ± 14b 58 ± 15b 50 ± 12a 36 ± 10a 14 ± 9a 22 ± 10a a 280 ± 40 388 ± 44a 106 ± 10b 134 ± 12b 58 ± 8a 78 ± 10a b 34 ± 12 40 ± 12b b 13 ± 2 16 ± 3,0b 0,23 ± 0,10c 0,21 ±0,10c 380 ± 38b 425 ± 40b a 78 ± 12 48 ± 8a 1098 ± 118a 618 ± 122a

√È ÙÈ̤˜ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È: ª¤ÛË ÙÈÌ‹ ± ™Ù·ıÂÚ‹ ·fiÎÏÈÛË (RNI = Reference Nutrient Intake = ™˘ÓÈÛÙÒÌÂÓË ËÌÂÚ‹ÛÈ· ÚfiÛÏË„Ë) Garrow JS, James Wp. (2000) Human Nutrition and Dietetics. 10th Edition. pp. 427 448. New York Churchill Livingstone, a/a p<0,001, b/b p<0,01 c/c= ªË ™Ù·ÙÈÛÙÈο ™ËÌ·ÓÙÈÎfi

ÙÔ˘˜. ™ÙȘ ÚÔ·Ó·ÊÂÚfiÌÂÓ˜ ÌËÙ¤Ú˜ ÙÔ Ì¤Ù·ÏÏÔ ÌÂÙÚ‹ıËΠ۠ÛËÌ·ÓÙÈο ¯·ÌËÏ¿ Â›‰· (<28 Ìg/l) ÛÙÔÓ ÔÚfi ÙÔ˘˜. √È Û˘ÁÎÂÓÙÚÒÛÂȘ Cu ÛÙÔÓ ÔÚfi ÙˆÓ ∂ÏÏËÓ›‰ˆÓ Î·È ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ ‹Ù·Ó ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ˜ ·fi ÂΛӘ ÙˆÓ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ (¶›Ó·Î·˜ 3). √ Cu ÌÂÙÚ‹ıËΠÛÙ· ηÙÒÙÂÚ· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· ÛÙȘ ∞Ï‚·Ó›‰Â˜ ÌËÙ¤Ú˜ Î·È ‹Ù·Ó ·ÍÈÔÛËÌ›ˆÙ· ÌÂȈ̤ÓÔ˜ ÛÙ· ÓÂÔÁÓ¿ ÙÔ˘˜. ŸÛÔÓ ·ÊÔÚ¿ ÛÙ· Ëϛη Û˘ÁΤÓÙÚˆÛ˘ ÙˆÓ ÌÂÙ¿ÏÏˆÓ ÛÙȘ ÌËÙ¤Ú˜ Î·È ÛÙ· ÓÂÔÁÓ¿ ÙÔ˘˜ (ª/¡), Ù· Ëϛη Se ‹Ù·Ó ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· ÛÙȘ ∂ÏÏËÓ›‰Â˜ Û˘ÁÎÚÈÓfiÌÂÓ· ÌÂ ÙˆÓ ∞Ï‚·Ó›‰ˆÓ, ÂÓÒ ‰ÂÓ ‰È¤ÊÂÚ·Ó ÌÂٷ͇ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ Ù· Û¯ÂÙÈ˙fiÌÂÓ· Ì ٷ Cu Ëϛη (ª/¡).

™˘˙‹ÙËÛË ∏ ÛÔ˘‰·ÈfiÙËÙ· ÙÔ˘ ÚfiÏÔ˘ ÙˆÓ È¯ÓÔÛÙÔȯ›ˆÓ Â›Ó·È ϤÔÓ ÁÓˆÛÙ‹ ·fi ÔÏϤ˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜, fiˆ˜ Â›Û˘ Ë Û¯¤ÛË ·ÓÂ¿ÚÎÂÈ¿˜ ÙÔ˘˜ Î·È ÂÌÊ¿ÓÈÛ˘ ÎÏÈÓÈÎÒÓ Û˘ÌÙˆÌ¿ÙˆÓ È‰È·›ÙÂÚ· ηٿ ÙËÓ Î‡ËÛË (2,3). ∆Ô Se Â›Ó·È ÁÓˆÛÙfi fiÙÈ Â›Ó·È ··Ú·›ÙËÙÔ ÁÈ· ÙË Ì›ˆÛË Ù˘ ‰Ú¿Û˘ ÙˆÓ ˘‰ÚÔ͢ÏÈÎÒÓ ÚÈ˙ÒÓ Î·È ÈÛ¯˘ÚÔÔÈ› ÙÔ ·ÓÔÛÔÔÈËÙÈÎfi Û‡ÛÙËÌ· (8). ∆Ô Ì¤Ù·ÏÏÔ ·ÔÚÚÔÊ¿Ù·È Â‡ÎÔÏ· ·fi ÙÔÓ Á·ÛÙÚÂÓÙÂÚÈÎfi ۈϋӷ, Èı·ÓfiÓ Ì ÂÓÂÚÁËÙÈ΋ ¶·È‰È·ÙÚÈ΋ 2006;69:199-204

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·202

202

∫. ™Ô‡ÏË Î·È Û˘Ó.

¶›Ó·Î·˜ 3. ∂›‰· ÛÂÏËÓ›Ô˘ (Se) Î·È ¯·ÏÎÔ‡ (Cu) ÛÙÔÓ ÔÚfi ∂ÏÏËÓ›‰ˆÓ Î·È ∞Ï‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ Î·È ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ ∂ÏÏËÓ›‰Â˜ (N=1118) ªËÙ¤Ú˜ ¡ÂÔÁÓ¿ Se (Ìg/L) ¢È¿ÌÂÛË ÙÈÌ‹ ∞ÎÚ·›Â˜ ÙÈ̤˜ (MËÙ¤Ú·/NÂÔÁÓfi) Cu (Ìg/L) ¢È¿ÌÂÛË ÙÈÌ‹ ∞ÎÚ·›Â˜ ÙÈ̤˜ (MËÙ¤Ú·/NÂÔÁÓfi)

68,30 ± 8,50a 61,0 28-133 2,1±0,3a 1687±353a 1611,0 1210-2070 3,5±0,5c

37,02 ± 8,90a* 37,0 26-83 449±87a 443,0 4338-541

∞Ï‚·Ó›‰Â˜ (N=820) ªËÙ¤Ú˜ ¡ÂÔÁÓ¿ 37,40 ± 8,9b 38,0 23-92 1,1±1,03b 959±318b 956,0 624-1376 3,3:0,3,1c

34,33 ± 9,10b* 35,0 21-53 229±67b 221,0 169-443

√È ÙÈ̤˜ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È: ª¤ÛË ÙÈÌ‹ ± ™Ù·ıÂÚ‹ ∞fiÎÏÈÛË a/b = p<0,0001, c/d = p<0,03, e/f= ªË ™Ù·ÙÈÛÙÈο, ™ËÌ·ÓÙÈ΋ ªËÙ/¡ÂÔÁÓfi = ËÏ›ÎÔ Û˘ÁΤÓÙÚˆÛ˘ ªËÙ¤Ú·˜/¡ÂÔÁÓÔ‡ *™ËÌ›ˆÛË: ∆· Â›‰Â‰· Se ÌÂÙÚ‹ıËÎ·Ó ¯·ÌËÏfiÙÂÚ· ÛÙÔ 3,1% ÙˆÓ ∂ÏÏËÓ›‰ˆÓ ÌËÙ¤ÚˆÓ Î·È ÛÙÔ 34,5% ÙˆÓ ∞Ï‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ Û˘ÁÎÚÈÓfiÌÂÓ˜ Ì ÂΛӷ ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ Î·È ·ÊÔÚ¿ Û ÂΛӘ ÙȘ ÌËÙ¤Ú˜ ÙˆÓ ÔÔ›ˆÓ Ù· Â›‰· Se ‹Ù·Ó <28 Ìg/l.

ÌÂÙ·ÊÔÚ¿, ÂÓÒ ÂÏ¿¯ÈÛÙ· Â›Ó·È ÁÓˆÛÙ¿ ÁÈ· ÙË ÌÂÙ·ÊÔÚ¿ ÙÔ˘ ÛÙÔ ·›Ì· ·fi ÙÔ ÂÙÈÎfi Û‡ÛÙËÌ·. ∆Ô Se ηْ ÂÍÔ¯‹Ó ¢ڛÛÎÂÙ·È Û ˘„ËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ÛÙÔ˘˜ ÓÂÊÚÔ‡˜, ÙÔ ‹·Ú Î·È ÛÙÔ˘˜ fiÚ¯ÂȘ. ∏ ·¤ÎÎÚÈÛ‹ ÙÔ˘ Á›ÓÂÙ·È Î˘Ú›ˆ˜ ·fi ÙÔ˘˜ ÓÂÊÚÔ‡˜, Î·È Ì¤Ûˆ ÙˆÓ Ó¢ÌfiÓˆÓ ˆ˜ ‰ÈÌÂı˘ÏÛÂÏ‹ÓÈÔ (5). √È Û˘ÁÎÂÓÙÚÒÛÂȘ ÙˆÓ È¯ÓÔÛÙÔȯ›ˆÓ ÛÙÔÓ ÔÚfi ÌÂÈÒÓÔÓÙ·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘, ÂÓÒ ·Ú·Ì¤ÓÔ˘Ó ÛÙ·ıÂÚ¤˜ ÛÙ· ÂÚ˘ıÚÔ·ÙÙ·Ú·. ∆· Â›‰· Se ÛÙË ÌËÙ¤Ú· Â›Ó·È Û·ÊÒ˜ ˘„ËÏfiÙÂÚ· ÂÎÂ›ÓˆÓ Ô˘ ·ÓȯÓ‡ÔÓÙ·È ÛÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ (11,15,16). ™Â ·˘Ù‹ ÙË ÌÂϤÙË, ÙfiÛÔ ÔÈ ∂ÏÏËÓ›‰Â˜ fiÛÔ Î·È ÔÈ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ÌËÙ¤Ú˜ ‰È·ÙÚ¤ÊÔÓÙ·È ÛˆÛÙ¿ fiÛÔ ·ÊÔÚ¿ ÛÙËÓ ÚfiÛÏË„Ë ıÂÚÌ›‰ˆÓ, ˘‰·Ù·ÓıڿΈÓ, Ï›Ô˘˜ Î·È ÔÏÈ΋˜ ÚˆÙ½Ó˘. ŸÌˆ˜, ÔÈ ·Ó¿ÁΘ Û ÔÏÏ¿ ‰È·ÙÚÔÊÈο ÛÙÔȯ›· ·˘Í¿ÓÔ˘Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘. ∞ÓÂ¿ÚÎÂȘ Û ‰È·ÙÚÔÊÈο ÛÙÔȯ›· ÌÔÚÔ‡Ó Ó· ·ÚÔ˘ÛÈ·ÛıÔ‡Ó Â›Ù ·fi ÌÂȈ̤ÓË ÚfiÛÏË„Ë, ›Ù ·fi ·˘ÍË̤ÓË ·ÒÏÂÈ· ‹ ·fi η΋ ·ÔÚÚfiÊËÛË Î·È ¤¯Ô˘Ó ¿ÌÂÛË Â›ÙˆÛË ÙfiÛÔ ÛÙËÓ ÂÁ΢ÌÔÓÔ‡Û· fiÛÔ Î·È ÛÙÔ ¤Ì‚Ú˘Ô (17). √È Û˘ÁÎÂÓÙÚÒÛÂȘ Se ÛÙÔÓ ÔÚfi ÙˆÓ ÌÂÙ·Ó·ÛÙÚÈÒÓ ‹Ù·Ó Ôχ ÌÂȈ̤Ó˜. ∂ÊfiÛÔÓ ÔÈ ∂ÏÏËÓ›‰Â˜ Î·È ÔÈ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ÌËÙ¤Ú˜ ‰È·Ì¤ÓÔ˘Ó ÛÙËÓ ›‰È· ÂÚÈÔ¯‹ (ÛÙËÓ ∞ı‹Ó·) Â›Ó·È ÂÌÊ·Ó¤˜ fiÙÈ Î·Ù·Ó·Ï›ÛÎÔÓÙ·˜ ÙȘ ›‰È˜ ÔÛfiÙËÙ˜ ÙÚÔÊ‹˜ Ô˘ ·Ó·Ù‡ÛÛÔÓÙ·È ÛÙÔ ›‰ÈÔ ¤‰·ÊÔ˜ Ù˘ ∂ÏÏ¿‰Ô˜ Ô˘ Â›Ó·È ÏÔ‡ÛÈÔ Û Se, ı· ¤ÚÂ ӷ ›¯·Ó ÙÔ ›‰ÈÔ Â›Â‰Ô Û Se (6,18). √È ‰È·ÙÚÔÊÈΤ˜ fï˜ Û˘Ó‹ıÂȘ ÙˆÓ ÌËÙ¤ÚˆÓ, ·ÏÏ¿ Î·È ÙÔ ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎfi ÙÔ˘˜ Â›Â‰Ô ÂËÚ¿˙Ô˘Ó Ù· Â›‰· ÙÔ˘ ÚÔ·Ó·¶·È‰È·ÙÚÈ΋ 2006;69:199-204

ÊÂÚÔ̤ÓÔ˘ ÌÂÙ¿ÏÏÔ˘ ÛÙÔÓ ÔÚfi ÙÔ˘˜ (11,13). ∆· ‰ËÌËÙÚȷο, ÙÔ Á¿Ï· Î·È Ù· Ï·¯·ÓÈο ıˆÚÔ‡ÓÙ·È Ùˆ¯¤˜ ËÁ¤˜ Û Se (17). ∆· ‰ËÌËÙÚȷο ıˆÚÂ›Ù·È fiÙÈ Â›Ó·È Ë ϤÔÓ ‰ËÌÔÊÈÏ‹˜ ÙÚÔÊ‹ ·ÎfiÌË Î·È ÁÈ· ÙȘ Ùˆ¯fiÙÂÚ˜ ÔÈÎÔÁ¤ÓÂȘ. ∂›Û˘, ¤¯ÂÈ ‰ËÌÔÛÈ¢ı› fiÙÈ ÔÈ ÌËÙ¤Ú˜ Ì Â›‰· Hb<8,0 g/dl ¤¯Ô˘Ó Â›Û˘ ¯·ÌËÏ¿ Â›‰· Se ÛÙÔ ·›Ì· ÙÔ˘˜ (13,14). ŸÌˆ˜, Ù· Â›‰· Hb Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ÌËÙ¤ÚˆÓ Ù˘ ÌÂϤÙ˘ ‰ÂÓ ‰È¤ÊÂÚ·Ó ÛÙ·ÙÈÛÙÈο ÌÂٷ͇ ÙÔ˘˜, Èı·ÓfiÓ ÏfiÁˆ Ù˘ ¯ÔÚ‹ÁËÛ˘ Ûȉ‹ÚÔ˘ per os (14). ∏ ÚÔ·Ó·ÊÂÚfiÌÂÓË, ÏÔÈfiÓ, ˘fiıÂÛË ‰ÂÓ ÈÛ¯‡ÂÈ fiÛÔÓ ·ÊÔÚ¿ ÛÙ· ¯·ÌËÏ¿ Â›‰· Se ÙˆÓ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ÌËÙ¤ÚˆÓ. ¶ÚÔÊ·Ó¤˜ Â›Ó·È fiÙÈ Ë ·fi ̤ÚÔ˘˜ ÙˆÓ ÌÂÙ·Ó·ÛÙÚÈÒÓ ¯·ÌËÏ‹ ηٷӿψÛË ˙ˆÈ΋˜ ÚÔ¤Ï¢Û˘ ÚˆÙ½Ó˘ Î·È Èı·ÓfiÓ „·ÚÈÒÓ, Ô˘ Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ‰··ÓËÚ¿, ‹ Ô˘ ‰ÂÓ ÂÌÂÚȤ¯ÔÓÙ·È ÛÙȘ ‰È·ÙÚÔÊÈΤ˜ ÙÔ˘˜ Û˘Ó‹ıÂȘ, ı· ÌÔÚÔ‡Û·Ó Ó· ÂÍËÁ‹ÛÔ˘Ó Ù· ¯·ÌËÏ¿ Â›‰· ÙÔ˘ ÌÂÙ¿ÏÏÔ˘ ÛÙÔÓ ÔÚfi ÙÔ˘˜. ∞˘Ù‹ Ë ˘fiıÂÛË Ê·›ÓÂÙ·È fiÙÈ ÂȂ‚·ÈÒÓÂÙ·È ·fi ÙË ÌÂȈ̤ÓË ÚfiÛÏË„Ë ÙfiÛÔ Ù˘ ˙ˆÈ΋˜ ÚÔ¤Ï¢Û˘ ÚˆÙ½Ó˘ fiÛÔ Î·È ·˘ÙÔ‡ ÙÔ˘ Se, fiˆ˜ ˘ÔÏÔÁ›ÛıËΠ·fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ‰È·ÈÙÔÏÔÁ›ˆÓ ÙÔ˘˜ (¶›Ó·Î·˜ 2). ŸÛÔÓ ·ÊÔÚ¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÙÔ 30% ÙˆÓ AÏ‚·Ó›‰ˆÓ ÌËÙ¤ÚˆÓ Ì Ôχ ¯·ÌËÏ¿ Â›‰· Se ÛÙÔÓ ÔÚfi ÙÔ˘˜ (Se<28 Ìg/l), ›¯·Ó ηÙÒÙÂÚ· Â›‰· ÙÔ˘ ÌÂÙ¿ÏÏÔ˘ ·fi ÂΛӷ ÙˆÓ ÓÂÔÁÓÒÓ ÙÔ˘˜ ı· ÌÔÚÔ‡Û ӷ ÂÚÌËÓ¢ı› ˆ˜ ÂÍ‹˜: ˘Ôı¤ÙÔ˘Ì fiÙÈ Ë Ï·ÎÔ˘ÓÙȷ΋ ÌÂÌ‚Ú¿ÓË ‰Ú· ÂÓÂÚÁËÙÈο ÂȘ fiÊÂÏÔ˜ ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÒÛÙ ӷ ˘¿Ú¯ÂÈ Â·Ú΋˜ ÔÛfiÙËÙ· ÙÔ˘ ÌÂÙ¿ÏÏÔ˘ ÁÈ· ÙȘ ·Ó¿ÁΘ ÙÔ˘ ÂÌ‚Ú‡Ô˘. √È Û˘ÁÎÂÓÙÚÒÛÂȘ ÙÔ˘ Se ÛÙÔÓ ÔÚfi Ù˘ ÌËÙ¤Ú·˜ Â›Ó·È ÙÔ

Pediatr May-June 06

16-05-06

17:21

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∂›‰· ÛÂÏËÓ›Ô˘ Î·È ¯·ÏÎÔ‡ ·›Ì·ÙÔ˜ ÌËÙ¤ÚˆÓ Î·È ÓÂÔÁÓÒÓ

55% ÂÎÂ›ÓˆÓ Ô˘ ÌÂÙÚÒÓÙ·È ÛÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ (11), fiˆ˜ ˘ÔÏÔÁ›ÛıËÎ·Ó Î·È ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ∂ÏÏËÓ›‰Â˜ ÌËÙ¤Ú˜ Î·È ÛÙ· ÓÂÔÁÓ¿ ÙÔ˘˜. ∞ÓÙ›ıÂÙ·, Ù· Â›‰· Se ÔÏÏÒÓ ÌÂÙ·Ó·ÛÙÚÈÒÓ ‹Ù·Ó ÙfiÛÔ ¯·ÌËÏ¿, ÒÛÙ ÏËÛ›·˙·Ó ·˘Ù¿ ÙˆÓ ÂÏÏËÓÈ΋˜ ηٷÁˆÁ‹˜ ÓÂÔÁÓÒÓ (¶›Ó·Î·˜ 3). ¢ÂÓ ˘¿Ú¯Ô˘Ó ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ Ô˘ Ó· ÂÚÌËÓÂ‡Ô˘Ó ÙËÓ ÙfiÛÔ ¯·ÌËÏ‹˜ Û˘ÁΤÓÙÚˆÛË Se Ù˘ ÌËÙ¤Ú·˜ Î·È ÙˆÓ ÂÈ¤‰ˆÓ ÙÔ˘ ÛÙÔ Î˘ÔÊÔÚÔ‡ÌÂÓÔ ¤Ì‚Ú˘Ô. ™ÙËÓ ÂÚÁ·Û›· ·˘Ù‹ ·Ú·ÙËÚÔ‡ÓÙ·È ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÛÙË ‰È·Ï·ÎÔ˘ÓÙȷ΋ ÌÂÙ·ÊÔÚ¿ ÙÔ˘ Se Î·È Cu, ηıfiÛÔÓ ‰ÂÓ ·Ú·ÙËÚÔ‡ÓÙ·È ·˘ÍË̤ӷ Â›‰· Cu Û ÓÂÔÁÓ¿ ÌËÙ¤ÚˆÓ Ì Ôχ ¯·ÌËÏ¿ Â›‰· ÙÔ˘ ÌÂÙ¿ÏÏÔ˘. ∏ ‰È·ÊÔÚÂÙÈ΋ ·˘Ù‹ Û˘ÌÂÚÈÊÔÚ¿ ÙÔ˘ Se Î·È Cu, ı· ÌÔÚÔ‡Û ӷ ¤¯ÂÈ Û¯¤ÛË Ì ٷ ‰È·ÊÔÚÂÙÈο ÌÔÚȷο ‚¿ÚË ÙˆÓ ÌÂÏÂÙËı¤ÓÙˆÓ ÌÂÙ¿ÏÏˆÓ ‹ Î·È Ì ÙÔ Â›‰Ô˜ ÙˆÓ ÚˆÙÂ˚ÓÒÓ Ì ÙȘ Ôԛ˜ ηı¤Ó· ·fi Ù· ̤ٷÏÏ· Û˘Ó‰¤ÔÓÙ·È. √È ˘Ôı¤ÛÂȘ ·˘Ù¤˜, fï˜, ¯ÚÂÈ¿˙ÔÓÙ·È ÂÚÈÛÛfiÙÂÚË ¤Ú¢ӷ ÁÈ· Â·Ï‹ı¢ÛË. ∆Ô Ê˘ÛÈÔÏÔÁÈÎfi ÙÂÏÂÈfiÌËÓÔ ÓÂÔÁÓfi ÁÂÓÓÈ¤Ù·È Ì ·ÚÎÂÙ‹ ÔÛfiÙËÙ· Cu, ΢ڛˆ˜ ÛÙÔ ‹·Ú ÙÔ˘, ÒÛÙÂ Ë ·Ó‡ÚÂÛË ÌÂÈˆÌ¤ÓˆÓ ÂÈ¤‰ˆÓ Ó· ·ÔÙÂÏ› Û¿ÓÈÔ Â‡ÚËÌ·. ™ÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜ Ù· ÌÂȈ̤ӷ Â›‰· ÙÔ˘ ÌÂÙ¿ÏÏÔ˘ Û˘Ó˘¿Ú¯Ô˘Ó Ì ·Ó·ÈÌ›·, Ô˘‰ÂÙÂÚÔÂÓ›· Î·È ÌÂȈ̤ÓË ÌÂÙ¿ÏψÛË ÙˆÓ ÔÛÙÒÓ Â˘Ú‹Ì·Ù· Ô˘ ·ÊÔÚÔ‡Ó ÙfiÛÔ Ù· ÚfiˆÚ· fiÛÔ Î·È Ù· ÙÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿ (19-22). ∏ ·ÈÙ›· ÙˆÓ ÌÂÈˆÌ¤ÓˆÓ ÂÈ¤‰ˆÓ Cu ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ÌÂÏÂÙËı›. ªÂϤÙ˜ Û ÚfiˆÚ· ÓÂÔÁÓ¿ ·¤Ù˘¯·Ó Ó· ÚÔÍÂÓ‹ÛÔ˘Ó ¤ÏÏÂÈ„Ë Cu ·Ú¿ ÙË ÌÂȈ̤ÓË ¯ÔÚ‹ÁËÛË Cu Â› 10 ‚‰ÔÌ¿‰Â˜ (14,19,21). ∆· Â›‰· Cu Â›Ó·È ¯·ÌËÏ¿ ÛÙË Á¤ÓÓËÛË (35Ìg/lL) Î·È ·˘Í¿ÓÔ˘Ó Û‡ÓÙÔÌ· Êı¿ÓÔÓÙ·˜ ·˘Ù¿ ÙˆÓ ÂÓËÏ›ÎˆÓ (70-140 Ìg/L) ηٿ ÙÔÓ 3Ô Ì‹Ó· ˙ˆ‹˜. ∏ ÚfiÛÏË„Ë ÂÚ›Ô˘ 75 Ìg/24ˆÚÔ ÛÙÔȯÂÈ·ÎÔ‡ Cu ηχÙÔ˘Ó Ï‹Úˆ˜ ÙȘ ·Ó¿ÁΘ Ù˘ ΢ÔÊÔÚÔ‡Û·˜, ÂÓÒ ·fi ·˘Ù¿ ÙÔ ¤Ì‚Ú˘Ô ¯ÚÂÈ¿˙ÂÙ·È ÂÚ› Ù· 51 Ìg/24ˆÚÔ, ÛÙÔ Ù¤ÏÔ˜ Ù˘ ·ËÛ˘ (22,23). √È ·Ú·ÙËÚÔ‡ÌÂÓ˜, ÏÔÈfiÓ, ¯·ÌËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ Cu ÛÙȘ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ ÌËÙ¤Ú˜, ÔÈ Ôԛ˜ ÚÔÛÏ¿Ì‚·Ó·Ó Â›Û˘ ¯·ÌËϤ˜ ÔÛfiÙËÙ˜ ÙÔ˘ ÌÂÙ¿ÏÏÔ˘, fiˆ˜ ˘ÔÏÔÁ›ÛıËΠ·fi ÙËÓ ·Ó¿Ï˘ÛË ÙÔ˘ ‰È·ÈÙÔÏÔÁ›Ô˘ ÙÔ˘˜, Ê·›ÓÂÙ·È fiÙÈ Â˘ı‡ÓÂÙ·È ÁÈ· ÙȘ ¯·ÌËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ Cu Ô˘ ÌÂÙÚ‹ıËÎ·Ó ÛÙ· ÓÂÔÁÓ¿ ÙÔ˘˜ (19). ¢˘ÛÙ˘¯Ò˜, Û ·˘Ù¤˜ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÓÂÔÁÓÒÓ Ì ÌÂȈ̤ÓÔ Cu ÛÙÔÓ ÔÚfi ÙÔ˘˜, ‰ÂÓ Â›¯·Ì ÙËÓ Â˘Î·ÈÚ›· ÂÚ·ÈÙ¤Úˆ ¤Ú¢ӷ˜.

203

∆· ÌÂȈ̤ӷ Â›‰· ȯÓÔÛÙÔȯ›ˆÓ ¤¯ÂÈ ‚ÚÂı› fiÙÈ Û¯ÂÙ›˙ÔÓÙ·È Ì ÌÂȈ̤ÓÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ (24). Ÿˆ˜ ·ÚÔ˘ÛÈ¿˙ÂÙ·È Û ·˘Ù‹ ÙËÓ ÂÚÁ·Û›·, ¯·ÌËÏfiÙÂÚÔ ‹Ù·Ó ÙÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ Ô˘ ·Ú·ÙËÚ‹ıËΠ۠ÓÂÔÁÓ¿ ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜ Û˘ÁÎÚÈÓfiÌÂÓÔ Ì ÂΛÓÔ ÙˆÓ ∂ÏÏ‹ÓˆÓ. ∂›Û˘ ¤¯ÂÈ ·Ó·ÎÔÈÓˆı› fiÙÈ ÙÔ Se ¤¯ÂÈ ÛÙÂÓ‹ Û¯¤ÛË Ì ÙË ‰Ú·ÛÙÈÎfiÙËÙ· ÙÔ˘ ÂÓ˙‡ÌÔ˘ ˘ÂÚÔÍÂȉ¿ÛË Ù˘ ÁÏÔ˘Ù·ıÂÈfiÓ˘ (GPx) fiˆ˜ Â›Û˘, fiÙÈ Ù· ÌÂȈ̤ӷ Â›‰· Cu ÌÂÈÒÓÔ˘Ó ÙË ‰Ú·ÛÙÈÎfiÙËÙ· ÙÔ˘ ȉ›Ô˘ ÂÓ˙‡ÌÔ˘ (8,11). ∂›Ó·È ÂÌÊ·Ó¤˜, ÏÔÈfiÓ, fiÙÈ ÙfiÛÔ ÙÔ Se fiÛÔ Î·È Ô Cu ¤¯Ô˘Ó ÛÔ˘‰·›Ô ÚfiÏÔ ÙfiÛÔ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ ·ÏÏ¿ Î·È ÛÙ· ÓÂÔÁÓ¿ Û˘ÌÌÂÙ¤¯ÔÓÙ·˜ ÛÙÔ ·ÓÙÈÔÍÂȉˆÙÈÎfi ÙÔ˘˜ Û‡ÛÙËÌ· (20,23). ™˘Ì¤Ú·ÛÌ·: ¶ÚÔÁÚ¿ÌÌ·Ù· ‰È·ÈÙÔÏÔÁÈ΋˜ ηıÔ‰‹ÁËÛ˘ Î·È ·Ú·ÎÔÏÔ‡ıËÛ˘ ı· Ú¤ÂÈ Ó· ıÂÛÈÛÙÔ‡Ó ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ÙˆÓ ª·È¢ÙÈÎÒÓ ∫ÏÈÓÈÎÒÓ.

∂˘¯·ÚÈÛٛ˜ √È Û˘ÁÁÚ·Ê›˜ ¢¯·ÚÈÛÙÔ‡Ó ÙËÓ Î. ÕÓÓ· ™Ù·Ì¿ÙË ÁÈ· ÙËÓ ÂÈ̤ÏÂÈ· Ù˘ ÂÚÁ·Û›·˜ ·˘Ù‹˜.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Black RE. Micronutrients in pregnancy. Br J Nutr 2001;85 (Suppl 2):S193-S197.

2. King JC. Physiology of pregnancy and nutrient metabolism. Am J Clin Nutr 2000;71 (Suppl 5):S1218S1225.

3. Aggett PJ. Aspects of neonatal metabolism of trace metals. Acta Paediatr Suppl 1994;402:75-82.

4. Keshan Disease Research Group: Selenium in Heart of China. Lancet 1979;2:889-890.

5. Varo P, Alfthan G, Huttunen JK, Aro A. Nationwide selenium supplementation in Finland - effects on diet, blood and tissue levels and health. In: Burk RF, editor. Selenium in Biology and Human Health. New York: Springer Verlag. 1994; p. 207-208.

6. Bratakos MS, Ioannou PV. The regional distribution of selenium in Greek cereals. Sci Total Environ 1989;84:237-247.

7. Volpe JJ. Hypoxic-ischaemic encephalopathy: biochemical and physiological aspects. In: Volpe JJ, editor. Neurology of the Newborn. 3rd ed. Philadelphia: WB Saunders Company; 1995. p. 211-259.

8. Butler JA, Whanger PD, Tripp MJ. Blood selenium and glutathione peroxidase activity in pregnant women: comparative assays in primates and other animals. Am J Clin Nutr 1982;36:15-23.

9. Horning MS, Blakemore LJ, Trombley PQ. Endogenous mechanisms of neuroprotection: role of zinc, ¶·È‰È·ÙÚÈ΋ 2006;69:199-204

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∫. ™Ô‡ÏË Î·È Û˘Ó.

copper and carnosine. Brain Res 2000;852:56-61.

10. Dorea JG, Brito M, Araujo MO. Concentration of copper and zinc in liver of fetuses and infants. J Am Coll Nutr 1987;6:491-495.

11. Rossipal E, Krachler M, Li F, Micetic-Turk D.. Investigation of the transport of trace elements across barriers in humans: studies of placental and mammary transfer. Acta Paediatr 2000;89:1190-1195.

12. Paul AA, Southgate GA, Russel J. The Composition of food. London HMSO, 1980.

13. Sheehan TM, Halls DJ. Measurement of selenium in clinical specimens. Ann Clin Biochem 1999;36: 301-315.

14. Turner RE, Langkamp-Henken B, Littell RC, Lukowski MJ, Suarez MF. Comparing nutrient intake from food to the estimated average requirements shows middle- to upper-income pregnant women lack iron and possibly magnesium. J Am Diet Assoc 2003;103:461-466.

15. Gathwala G, Yadav OP, Singh I, Sangwan K. Maternal and Cord Plasma Selenium Levels in Full term Neonates. Indian J Pediatr 2000;67:729-731.

16. Gathwala G, Yadav OP. Selenium in the neonate. Indian Journal of Pediatrics 2002;69:443-446.

17. Thomas B. Manual of Dietetic Practice. Nutritional Sciences. Nutricia Healthcare 2001; p. 217-225.

¶·È‰È·ÙÚÈ΋ 2006;69:199-204

18. Bratakos MS, Kanaki HC, Vasiliou-Waite A, Ioannou PV. The nutritional selenium status of healthy Greeks. Sci Total Environ 1990;91:161-176.

19. Terres-Martos C, Navarro-Alarcon M, Martin-Lagos F, Lopez-G de la Serrana H, Lopez-Martinez MC. Determination of Cu levels in serum of healthy subjects by atomic absorption spectometry. Sci Total Environ 1997;198:97-103.

20. Cohen-Kerem R, Koren G. Antioxidants and fetal protection against ethanol teratogenicity. I. Review of the experimental data and implications to humans.

Neurotoxical Teratol 2003;25:1-9.

21. Vuori E, Makinen SM, Kara R, Kuitunen P. The effects of the dietary intakes of copper, iron, manganese, and zinc on the trace element content of human milk. Am J Clin Nutr 1980;13:227-231.

22. Widdowson EM. The demands of the fetal and maternal tissues for nutrients and the bearing of these on the needs of the mother to "eat for two". In: Dobbing J, editor. Maternal nutrition in pregnancy-eating for two. London: Academic Press; 1981. p. 1-19.

23. Chan S, Gerson B, Subramaniam S. The role of copper, molybdenum, selenium and zinc in nutrition and health. Clin Lab Med 1998;18:673-685.

24. Bhaskaram P. Micronutrient deficiencies in children the problem and extent. Indian J Pediatr 1995;62: 145-156.

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ORIGINAL ARTICLE

205

BÈÙ·Ì›ÓË D Û ÌËÙ¤Ú˜ Î·È ‚Ú¤ÊË Ì ·ÔÎÏÂÈÛÙÈÎfi ıËÏ·ÛÌfi ÛÙËÓ EÏÏ¿‰· Õ. X¿ÏÏ·1, A. NÙÔ˘ÚÓÙÔ‡ÊË2, B. XÔϤ‚·˜1, I. BÏ¿¯Ô˘3, M. MÈÙÛÒÚË4, E. °·Ï·Ó¿Î˘4, ™. AÓ‰ÚÔÓ›ÎÔ˘2

¶ÂÚ›ÏË„Ë EÈÛ·ÁˆÁ‹: T· ÓÂÔÁÓ¿ Î·È Ù· ‚Ú¤ÊË Ô˘ ıËÏ¿˙Ô˘Ó ·ÔÎÏÂÈÛÙÈο, ÂÍ·ÚÙÒÓÙ·È ·fi ÙË ÌËÙ¤Ú· ÁÈ· ÙË ‚ÈÙ·Ì›ÓË D. H ·ÚÔ‡Û· ÌÂϤÙË ÚÔÛ¿ıËÛ ӷ ‰ÈÂÚ¢ӋÛÂÈ ·Ó ÌËÙ¤Ú˜ Î·È ‚Ú¤ÊË ¤¯Ô˘Ó Â·ÚΛ˜ ÔÛfiÙËÙ˜ ·˘Ù‹˜ Ù˘ ‚ÈÙ·Ì›Ó˘.

YÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: TÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿ Ô˘ ÁÂÓÓ‹ıËÎ·Ó IÔ‡ÓÈÔ - NԤ̂ÚÈÔ (N=35) Î·È ÔÈ ÌËÙ¤Ú˜ ÙÔ˘˜ ·ÔÙ¤ÏÂÛ·Ó ÙË ıÂÚÈÓ‹ ÔÌ¿‰· Î·È ÙˆÓ ÌËÓÒÓ ¢ÂÎÂÌ‚Ú›Ô˘ - ª·˝Ô˘ (N=31) ÙË ¯ÂÈÌÂÚÈÓ‹. ¶ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó Ù· Â›‰· 25OHD, ·Ú·ıÔÚÌfiÓ˘ (iPTH), ·Û‚ÂÛÙ›Ô˘ (Ca), ʈÛÊfiÚÔ˘ (Pi), ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ (ALP) Î·È ÔÛÙÂÔηÏÛ›Ó˘ (OC) Û ÌËÙ¤Ú˜ Î·È ÓÂÔÁÓ¿ ÙËÓ ÚÒÙË Â‚‰ÔÌ¿‰· ÌÂÙ¿ ÙË Á¤ÓÓËÛË Î·È ÛÙ· ‚Ú¤ÊË Û ·ÔÎÏÂÈÛÙÈο ÌËÙÚÈÎfi ıËÏ·ÛÌfi ÙÔÓ 1Ô, 3Ô Î·È 6Ô Ì‹Ó· ˙ˆ‹˜.

AÔÙÂϤÛÌ·Ù·: TËÓ ÚÒÙË Â‚‰ÔÌ¿‰· ˙ˆ‹˜ Ù· Â›‰· Ù˘ 25OHD ‚Ú¤ıËÎ·Ó ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ· ÛÙËÓ ÔÌ¿‰· ÓÂÔÁÓÒÓ Ù˘ ¯ÂÈÌÂÚÈÓ‹˜ ÂÚÈfi‰Ô˘ ·fi Ù˘ ıÂÚÈÓ‹˜ (6,7±0,7 ¤Ó·ÓÙÈ 10,1±0,9 ng/ml, p<0,01). AÓÙ›ÛÙÔȯ· Ù· Â›‰· ·Ú·ıÔÚÌfiÓ˘ (iPTH) ‹Ù·Ó 64,9±13,4 Î·È 33,9±4,4 pg/ml, p<0,01. √È ÌËÙ¤Ú˜ ›¯·Ó 25OHD 10,8±1,0 ng/ml Î·È iPTH 15,2±3,5 pg/ml ÙÔÓ ¯ÂÈÌÒÓ· Î·È 12,9±1,3 pg/ml Î·È 24,8±4,8 pg/ml ÙÔ Î·ÏÔη›ÚÈ. M¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ ¤ÍÈ ÌËÓÒÓ, ÛÙ· ‚Ú¤ÊË Ô˘ ›¯·Ó ÁÂÓÓËı› ÙÔ ¯ÂÈÌÒÓ· ·Ú·ÙËÚ‹ıËΠÛÙ·ıÂÚ‹ ·‡ÍËÛË ÙÈÌÒÓ Ù˘ 25OHD (19,4±2,8 ng/ml, p<0,0001) ·ÏÏ¿ Î·È Ù˘ iPTH Ô˘ ÛÙÔÓ 6Ô Ì‹Ó· ÌÂÈÒıËΠ(26,8±3,5 pg/ml, p=0,10). ™ÙË ıÂÚÈÓ‹ ÔÌ¿‰· ÔÈ ÙÈ̤˜ Ù˘ 25OHD ·Ú¤ÌÂÈÓ·Ó ÛÙ· ›‰È· Â›‰·, ·ÏÏ¿ Ù˘ iPTH ·˘Í‹ıËÎ·Ó ÛËÌ·ÓÙÈο ̤¯ÚÈ ÙÔ 3Ô Ì‹Ó· (59,4±21,8 pg/ml, p<0,05). ™˘ÌÂÚ¿ÛÌ·Ù·: ™ÙËÓ ∂ÏÏ¿‰· Ù· ÓÂÔÁÓ¿ Ô˘ ÛÈÙ›˙ÔÓÙ·È ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi ıËÏ·ÛÌfi ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ 6 ÌËÓÒÓ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ Î·È ·ÓÂÍ¿ÚÙËÙ· ·fi ÂÔ¯‹, Ê·›ÓÂÙ·È Ó· ¯ÚÂÈ¿˙ÔÓÙ·È Û˘Ìϋڈ̷ ‚ÈÙ·Ì›Ó˘ D.

1 EÚÁ·ÛÙ‹ÚÈÔ ¶·È‰È·ÙÚÈ΋˜, TÔ̤·˜ YÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡, I·ÙÚÈ΋ ™¯ÔÏ‹, ¶·ÓÂÈÛÙ‹ÌÈÔ Iˆ·ÓÓ›ÓˆÓ 2 NÂÔÁÓÔÏÔÁÈ΋ KÏÈÓÈ΋, TÔ̤·˜ YÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡, I·ÙÚÈ΋ ™¯ÔÏ‹, ¶·ÓÂÈÛÙ‹ÌÈÔ Iˆ·ÓÓ›ÓˆÓ 3 BÈÔ¯ËÌÈÎfi EÚÁ·ÛÙ‹ÚÈÔ, ¶·ÓÂÈÛÙËÌÈ·Îfi °ÂÓÈÎfi NÔÛÔÎÔÌÂ›Ô Iˆ·ÓÓ›ÓˆÓ 4 ¶·È‰È·ÙÚÈ΋ KÏÈÓÈ΋, I·ÙÚÈ΋ ™¯ÔÏ‹, ¶·ÓÂÈÛÙ‹ÌÈÔ KÚ‹Ù˘ AÏÏËÏÔÁÚ·Ê›·: AÓ. K·ıËÁ‹ÙÚÈ· ÕÓÓ· X¿ÏÏ· TÔ̤·˜ YÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡, I·ÙÚÈ΋ ™¯ÔÏ‹ ¶·ÓÂÈÛÙËÌ›Ô˘ Iˆ·ÓÓ›ÓˆÓ T.K. 451 10, Iˆ¿ÓÓÈÓ· E-mail: achalla@cc.uoi.gr ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 30-09-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 28-03-2006

§¤ÍÂȘ ÎÏÂȉȿ: µÈÙ·Ì›ÓË D, ÌËÙÚÈÎfi˜ ıËÏ·ÛÌfi˜, ÙÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿.

Vitamin D status in breast-fed babies and their mothers in Greece ∞. Challa1, A. Ntourntoufi2, V. Cholevas1, I. Vlachou3, M. Bitsori4, E. Galanakis4, S. Andronikou2

Abstract Background: Neonates and infants who are exclusively breast fed are dependent on their mothers for vitamin D. The present study explored the adequacy of vitamin D levels in mothers and their breastfed offspring at different seasons.

Methods: Full term neonates born from June to November comprised the summer group (N=35) and those born from December to May the winter group (N=31). Serum levels of 25OHD, intact parathyroid hormone (iPTH), calcium (Ca), phosphate (Pi), alkaline phosphatase (ALP) and osteocalcin (OC) were determined in the first week after birth in both mothers and neonates and at the 1st, 3rd and 6th months of life in the infants.

Results: In the first week the serum 25OHD was significantly lower in the winter than in the summer group of infants (6.7±0.7 vs 10.1±0.9 ng/ml, p<0.01). The respective iPTH levels were 64.9±13.4 Î·È 33.9±4.4 pg/ml (p<0.01). The levels of the mothers were 25OHD 10.8±1.0 ng/ml and iPTH 15.2±3.5 pg/ml in winter and 12.9±1.3 pg/ml and 24.8±4.8 pg/ml respectively in summer. Up to the age of six months, a steady increase in the 25OHD levels of the winter-born infants was observed, reaching 19.4±2.8 ng/ml (p<0.0001). Similar changes were seen initially in iPTH but at 6 months, it decreased to 26.8±3.5 pg/ml (p=0.10). In the summer-born infants circulating 25OHD remained steady, while iPTH increased significantly up to the 3rd month (59.4±21.8 pg/ml, p<0.05).

1 Laboratory of Paediatrics, Children’s Health Division, Medical School, University of Ioannina, 2 Neonatology Clinic, Children’s Health Division, Medical School, University of Ioannina, 3 Biochemical Laboratory, University General Hospital Of Ioannina 4 Pediatric Clinic, Medical School, University of Crete Correspondence: Anna Chala, Associate Professor Department of Paediatrics, Medical School, University of Ioannina 451 10, Ioannina E-mail: achalla@cc.uoi.gr Date of submission: 30-09-2005 Date of approval: 28-03-2006

¶·È‰È·ÙÚÈ΋ 2006;69:205-212

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·206

206

Õ. X¿ÏÏ· Î·È Û˘Ó.

Conclusions: In Greece, infants exclusively breast fed during their first six months of life, appear to be

in need of vitamin D supplementation, independent of the season.

Key words: Vitamin D, breast feeding, full term neonates.

™˘ÓÙÔÌÔÁڷʛ˜: 25OHD: 25-˘‰ÚÔ͢‚ÈÙ·Ì›ÓË D 1,25(OH)2D3: 1·,25-‰È˘‰ÚÔ͢¯ÔÏËηÏÛÈÊÂÚfiÏË (1·,25-‰È˘‰ÚÔ͢‚ÈÙ·Ì›ÓË D3) Ca: AÛ‚¤ÛÙÈÔ Pi: AÓfiÚÁ·ÓÔ˜ ʈÛÊfiÚÔ˜ iPTH: OÏÈÎfi ÌfiÚÈÔ (intact) ·Ú·ıÔÚÌfiÓ˘ ALP: AÏηÏÈ΋ ʈÛÊ·Ù¿ÛË OC: OÛÙÂÔηÏÛ›ÓË

∂ÈÛ·ÁˆÁ‹ H ‚ÈÙ·Ì›ÓË D Î·È ÔÈ ÌÂÙ·‚Ôϛ٘ Ù˘ ÛÙÔÓ ·ÓıÚÒÈÓÔ ÔÚÁ·ÓÈÛÌfi ·›˙Ô˘Ó ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÔÌÔÈfiÛÙ·ÛË ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ Î·È ÊˆÛÊfiÚÔ˘ ηıÒ˜ Î·È ÙËÓ ÂÓ·fiıÂÛË ·˘ÙÒÓ ÙˆÓ ÈfiÓÙˆÓ ÛÙ· ÔÛÙ¿ (1). TÔ fiÓÔÌ¿ Ù˘ Â›Ó·È Û˘ÓÒÓ˘ÌÔ Ì ÙËÓ ·ÓÙÈÚÚ·¯ÈÙÈ΋ ‰Ú¿ÛË Ù˘. ¶·Ú’ fiÏ· ·˘Ù¿ ÙËÓ ÙÂÏÂ˘Ù·›· ‰ÂηÂÓÙ·ÂÙ›· Ë ‚ÈÙ·Ì›ÓË D ʤÚÂÙ·È Ó· ¤¯ÂÈ Î·È ¿ÏϘ ‰Ú¿ÛÂȘ, fiˆ˜ ÛÙÔ ·ÓÔÛÔÔÈËÙÈÎfi Û‡ÛÙËÌ· (2).

H ‚ÈÙ·Ì›ÓË D3 Û¯ËÌ·Ù›˙ÂÙ·È ÛÙÔ ‰¤ÚÌ· ·fi ÙËÓ ÂÓ‰ÔÁÂÓ‹ ÚÔ‚ÈÙ·Ì›ÓË 7-‰Â¸‰ÚÔ¯ÔÏËÛÙÂÚfiÏË Ì ÙË ‰Ú¿ÛË Ù˘ ˘ÂÚÈÒ‰Ô˘˜ ·ÎÙÈÓÔ‚ÔÏ›·˜ (UV) ÙÔ˘ ËÏÈ·ÎÔ‡ ʈÙfi˜ (3). ™ÙË Û˘Ó¤¯ÂÈ·, Ì ÙËÓ Î˘ÎÏÔÊÔÚ›·, ÌÂٷʤÚÂÙ·È ÛÙÔ ‹·Ú fiÔ˘ Î·È ˘‰ÚÔ͢ÏÈÒÓÂÙ·È ÛÙË ı¤ÛË 25 ÁÈ· ÙËÓ ·Ú·ÁˆÁ‹ Ù˘ 25OHD (4). OÈ Û˘ÁÎÂÓÙÚÒÛÂȘ ·˘ÙÔ‡ ÙÔ˘ ÌÂÙ·‚ÔÏ›ÙË ÛÙÔ ·›Ì· Â›Ó·È Û ¿ÌÂÛË ÂÍ¿ÚÙËÛË ·fi ÙËÓ ·Ú·ÁˆÁ‹ Ù˘ ‚ÈÙ·Ì›Ó˘ D, Ô˘ Ì ÙË ÛÂÈÚ¿ Ù˘ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ¤ÎıÂÛË ÛÙËÓ ËÏȷ΋ ·ÎÙÈÓÔ‚ÔÏ›·. °È’ ·˘ÙfiÓ ÙÔ ÏfiÁÔ ÂÌÊ·Ó›˙ÂÈ ÛËÌ·ÓÙÈ΋ Âԯȷ΋ ‰È·Î‡Ì·ÓÛË Ì ¯·ÌËÏfiÙÂÚ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ÙÔÓ ¯ÂÈÌÒÓ· Î·È ˘„ËÏfiÙÂÚ˜ ÙÔ Î·ÏÔη›ÚÈ. O ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù˘ 25OHD ÛÙÔÓ ÔÚfi ·ÔÙÂÏ› ‰Â›ÎÙË Â¿ÚÎÂÈ·˜ ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡ Û ‚ÈÙ·Ì›ÓË D (5), ·Ú¿ ÙÔ fiÙÈ Ô Î·Ù’ ÂÍÔ¯‹Ó ‰Ú·ÛÙÈÎfi˜ ÌÂÙ·‚ÔÏ›Ù˘ Â›Ó·È Ë 1,25(OH)2D3 Ô˘ Û˘ÓÙ›ıÂÙ·È ÛÙÔ˘˜ ÓÂÊÚÔ‡˜ (6). Yfi ȉ·ÓÈΤ˜ Û˘Óı‹Î˜ Ô ¿ÓıÚˆÔ˜ ÌÔÚ› Ó· ·ÔÎÙ‹ÛÂÈ ÙÔ 90%-100% Ù˘ ‚ÈÙ·Ì›Ó˘ D Ô˘ ¯ÚÂÈ¿˙ÂÙ·È ·fi ÙËÓ ¤ÎıÂÛ‹ ÙÔ˘ ÛÙËÓ ËÏȷ΋ ·ÎÙÈÓÔ‚ÔÏ›· (7). √ ÌËÙÚÈÎfi˜ ıËÏ·ÛÌfi˜ Â›Ó·È ˆÊ¤ÏÈÌÔ˜ ÁÈ· Ù· ÓÂÔÁÓ¿, ÙȘ ÌËÙ¤Ú˜ Î·È ÙȘ ÔÈÎÔÁ¤ÓÂȤ˜ ÙÔ˘˜. ∆Ô ÌËÙÚÈÎfi Á¿Ï· ÌÔÚ› Ó· ÈηÓÔÔÈ‹ÛÂÈ ¶·È‰È·ÙÚÈ΋ 2006;69:205-212

·fiÏ˘Ù· ÙȘ ıÚÂÙÈΤ˜ ·Ó¿ÁΘ ÙÔ˘ ·ÓıÚÒÈÓÔ˘ ÔÚÁ·ÓÈÛÌÔ‡ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ 6 ÌËÓÒÓ Ù˘ ˙ˆ‹˜, Ì ÂÍ·›ÚÂÛË ÙË ‚ÈÙ·Ì›ÓË D Î·È ÙË ‚ÈÙ·Ì›ÓË K (8,9). Œ¯ÂÈ ˘ÔÏÔÁÈÛÙ› fiÙÈ Ë ÂÚÈÂÎÙÈÎfiÙËÙ· ÙÔ˘ ·ÓıÚÒÈÓÔ˘ Á¿Ï·ÎÙÔ˜ Û ‚ÈÙ·Ì›ÓË D Î˘Ì·›ÓÂÙ·È ÌÂٷ͇ 5 ¤ˆ˜ 136 IU/L ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÔ¯‹ (10). MÂȈ̤ӷ Â›‰· ‚ÈÙ·Ì›Ó˘ D Û ¤ÁÎ˘Â˜ ÌËÙ¤Ú˜, Ô˘ fï˜ ‰ÂÓ Â›Ó·È ÙfiÛÔ ¯·ÌËÏ¿ ÒÛÙ ӷ ÙÔ˘˜ ÚÔηÏÔ‡Ó ÔÛÙÂÔÌ·Ï¿Î˘ÓÛË ‹ ÙÚÔÊÈÎfi Ú·¯ÈÙÈÛÌfi ÛÙ· ÓÂÔÁÓ¿, ¤¯Ô˘Ó Û˘Ó‰Âı› Ì ¿ÏϘ ‰È·Ù·Ú·¯¤˜ ÛÙË ÓÂÔÁÓÈ΋ Î·È ‚ÚÂÊÈ΋ ÂÚ›Ô‰Ô, fiˆ˜ ÙË ÓÂÔÁÓÈ΋ ˘·Û‚ÂÛÙÈ·ÈÌ›·, ÔÛÙÂÔÂÓ›·, ηı˘ÛÙ¤ÚËÛË ÛÙËÓ ·‡ÍËÛË ÙÔ˘ ÛÎÂÏÂÙÔ‡ ηٿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ˙ˆ‹˜ Î·È ˘ÔÏ·Û›· ÙÔ˘ ÛÌ¿ÏÙÔ˘ ÙˆÓ ÓÂÔÁÈÏÒÓ ‰ÔÓÙÈÒÓ (11). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÚÔËÁÔ‡ÌÂÓ˘ ‰ÂηÂÙ›·˜ Ù· Â˘Ú‹Ì·Ù· ÛËÌ·ÓÙÈÎÔ‡ ·ÚÈıÌÔ‡ ÌÂÏÂÙÒÓ Û ·ÓıÚÒÔ˘˜ Î·È ˙Ò· ˘ÔÛÙËÚ›˙Ô˘Ó ÙË ıˆڛ· fiÙÈ Ë ¯ÚfiÓÈ· ·ÓÂ¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ D Û˘Ì‚¿ÏÏÂÈ Û ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÂÌÊ¿ÓÈÛ˘ ÔÏÏÒÓ ¯ÚfiÓÈˆÓ ·ı‹ÛˆÓ, fiˆ˜ ‰È·‚‹ÙË Ù‡Ô˘ π, ηډȷÁÁÂÈ·ÎÒÓ ·ı‹ÛˆÓ, ÔÛÙÂÔfiÚˆÛ˘ ·ÏÏ¿ Î·È ÌÂÚÈÎÒÓ ÎÔÈÓÒÓ ÓÂÔÏ·ÛÈÒÓ (12).

TÔ ·Ú¿‰ÔÍÔ Â›Ó·È fiÙÈ ÁÈ· ÙËÓ Â·ÓÂÌÊ¿ÓÈÛË ·ÓÂ¿ÚÎÂÈ·˜ ‚ÈÙ·Ì›Ó˘ D ÛÙÔ ‚ÚÂÊÈÎfi ÏËı˘ÛÌfi ¢ı‡ÓÂÙ·È ÂÓ Ì¤ÚÂÈ Ë ÂÎÛÙÚ·Ù›· ÂÓı¿ÚÚ˘ÓÛ˘ Ù˘ ÌËÙ¤Ú·˜ ÁÈ· ·ÔÎÏÂÈÛÙÈο ÌËÙÚÈÎfi ıËÏ·ÛÌfi, ¯ˆÚ›˜ Ó· ¯ÔÚËÁÂ›Ù·È ·Ú¿ÏÏËÏ· ÛÙËÓ ›‰È· ‹ ÛÙÔ ‚Ú¤ÊÔ˜ Û˘Ìϋڈ̷ ‚ÈÙ·Ì›Ó˘ D. MÂϤÙ˜ ·fi ÙËÓ πÙ·Ï›· Î·È ÙË °·ÏÏ›· ·Ó·Ê¤ÚÔ˘Ó fiÙÈ Ë ˘ÔÎÏÈÓÈ΋ ·ÓÂ¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ D ‰ÂÓ Â›Ó·È ÙfiÛÔ Û¿ÓÈ· ÛÙË ÂÚÈÔ¯‹ Ù˘ MÂÛÔÁ›Ԣ, ›Ù ÛÙȘ ¤ÁÎ˘Â˜ ÌËÙ¤Ú˜ ›Ù ÛÙ· ÓÂÔÁÓ¿ ÙÔ˘˜ Î·È Î˘Ú›ˆ˜ ηٿ ÙÔ˘˜ ¯ÂÈÌÂÚÈÓÔ‡˜ Ì‹Ó˜ (13-15). ∏ ·ÓÂ¿ÚÎÂÈ· Û ‚ÈÙ·Ì›ÓË D Ù˘ ÌËÙ¤Ú·˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ Ô‰ËÁ› Û ·ÓÂ·Ú΋ ·ÓÂÊԉȷÛÌfi ÙÔ˘ ÂÌ‚Ú‡Ô˘ ̤ۈ ÙÔ˘ Ï·ÎÔ‡ÓÙ·, Ô˘ ·ÔÙÂÏ› Î·È ÙÔÓ ‚·ÛÈÎfi ÙÚÔÊÔ‰fiÙË ÙÔ˘ Û 25OHD (16). TÔ ·ÔÙ¤ÏÂÛÌ· Â›Ó·È ÂÏÏÂÈÌÌ·ÙÈΤ˜ ·Ôı‹Î˜ ÙÔ˘ ÓÂÔÁÓÔ‡ ·fi ÙË Á¤ÓÓËÛË. ∂ÈϤÔÓ Ì¤Û· Û ÔÎÙÒ Â‚‰ÔÌ¿‰Â˜ ·ÎfiÌË Î·È ÔÈ Â·ÚΛ˜ ·Ôı‹Î˜ ‚ÈÙ·Ì›Ó˘ D ÂÍ·ÓÙÏÔ‡ÓÙ·È (17) Î·È Ô ·ÔÎÏÂÈÛÙÈÎfi˜ ıËÏ·ÛÌfi˜ ÙˆÓ ‚ÚÂÊÒÓ ¯ˆÚ›˜ Û˘Ìϋڈ̷

Pediatr May-June 06

16-05-06

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BÈÙ·Ì›ÓË D Î·È ÌËÙÚÈÎfi˜ ıËÏ·ÛÌfi˜

‚ÈÙ·Ì›Ó˘ D ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ ÛÙËÓ ·Ó¿Ù˘ÍË ÙÚÔÊÈÎÔ‡ Ú·¯ÈÙÈÛÌÔ‡ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ ˙ˆ‹˜ (15). ∫·Ì›· Û¯ÂÙÈ΋ ÂÚÁ·Û›· ‰ÂÓ ˘¿Ú¯ÂÈ ÁÈ’ ·˘Ù‹ ÙËÓ ËÏÈÎȷ΋ ÔÌ¿‰· ÛÙËÓ ∂ÏÏ¿‰·, ÏfiÁˆ Ù˘ ıˆÚÔ‡ÌÂÓ˘ Â·ÚÎÔ‡˜ ËÏȷ΋˜ ¤ÎıÂÛ˘ fiÏˆÓ ÙˆÓ ·ÓıÚÒˆÓ ÛÙË ¯ÒÚ· Ì·˜, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Î·Ó¤Ó· ÙÚfiÊÈÌÔ ‰ÂÓ ÂÌÏÔ˘Ù›˙ÂÙ·È Ì ‚ÈÙ·Ì›ÓË D. √ ÛÙfi¯Ô˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ó· ‰ÈÂÚ¢ÓËı› Ë Â¿ÚÎÂÈ· ‹ ÌË Û ‚ÈÙ·Ì›ÓË D ÙˆÓ ÂÏÏËÓ›‰ˆÓ ÌËÙ¤ÚˆÓ Î·È ÙˆÓ ‚ÚÂÊÒÓ ÙÔ˘˜, ·fi ÙËÓ ÚÒÙË Â‚‰ÔÌ¿‰· ÌÂÙ¿ ÙË Á¤ÓÓËÛË Ì¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ ¤ÍÈ ÌËÓÒÓ ÂÓÒ ÙÚ¤ÊÔÓÙ·Ó ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi Á¿Ï· Î·È ¯ˆÚ›˜ ÙË Ï‹„Ë Û˘ÌÏËÚˆÌ¿ÙˆÓ ‚ÈÙ·Ì›Ó˘ D ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ ‹ ÙÔ˘ ıËÏ·ÛÌÔ‡.

YÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∆ÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿ (N=35) Ô˘ ÁÂÓÓ‹ıËÎ·Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ηÏÔηÈÚÈÔ‡ Î·È ÙÔ˘ ÊıÈÓÔÒÚÔ˘ (πÔ‡ÓÈÔ-¡Ô¤Ì‚ÚÈÔ) Î·È ÔÈ ÌËÙ¤Ú˜ ÙÔ˘˜ Û˘ÌÂÚÈÏ‹ÊıËÎ·Ó ÛÙË ıÂÚÈÓ‹ ÔÌ¿‰· (K) Î·È Ù· ˘fiÏÔÈ· (N=31) Ô˘ ÁÂÓÓ‹ıËÎ·Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ¯ÂÈÌÂÚÈÓÒÓ-·ÚÈÓÒÓ ÌËÓÒÓ (¢ÂΤ̂ÚÈÔ-M¿ÈÔ) ÔÚ›ÛÙËÎ·Ó Û·Ó ¯ÂÈÌÂÚÈÓ‹ ÔÌ¿‰· (X). ŸÏ· Ù· ÓÂÔÁÓ¿ ›¯·Ó Ê˘ÛÈÔÏÔÁÈÎfi ÁÈ· ÙËÓ ËÏÈΛ· ·ËÛ˘ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘, ¯ˆÚ›˜ ÈÛÙÔÚÈÎfi ÔÛÙÈ΋˜, Á·ÛÙÚÂÓÙÂÚÈ΋˜ ‹ ÓÂÊÚÈ΋˜ ‰È·Ù·Ú·¯‹˜ ‹ ÔÔÈ·Û‰‹ÔÙÂ Û˘ÁÁÂÓÔ‡˜ ·ÓˆÌ·Ï›·˜. ∆Ô ÙÂÛÙ Apgar (Apgar score) ‹Ù·Ó 7-10 ÛÙ· 5 ÏÂÙ¿. √È ÌËÙ¤Ú˜ ÙÔ˘˜ ‹Ù·Ó ÎÏÈÓÈο ˘ÁÈ›˜. ŸÏ· Ù· ‚Ú¤ÊË ÙÚ¤ÊÔÓÙ·Ó ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi ıËÏ·ÛÌfi ÁÈ· ÙÔ˘˜ ÚÒÙÔ˘˜ ¤ÍÈ Ì‹Ó˜ ˙ˆ‹˜, ‰È¿ÛÙËÌ· ηٿ ÙÔ ÔÔ›Ô Û˘ÌÌÂÙ›¯·Ó Î·È ÛÙË ÌÂϤÙË. H ËÏÈΛ· ·ËÛ˘ (̤ÛË ÙÈÌ‹ ±SD) ‹Ù·Ó ÌÂٷ͇ 37 Î·È 41 ‚‰ÔÌ¿‰ˆÓ, X: 39,1±0,7, K: 38,7±1,0 ‚‰.) Î·È ÙÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ ·fi 2570 ̤¯ÚÈ 3900 ÁÚ. (X: 3314±351, K: 3158±417 ÁÚ.). ∏ ‰ÂÈÁÌ·ÙÔÏË„›· ¤ÁÈÓ ηٿ ÙËÓ Â›Û΄‹ ÙÔ˘˜ ÛÙÔ Ù·ÎÙÈÎfi Â͈ÙÂÚÈÎfi È·ÙÚÂ›Ô ·Ú·ÎÔÏÔ‡ıËÛ˘ Ù˘ NÂÔÁÓÔÏÔÁÈ΋˜ KÏÈÓÈ΋˜ ÙÔ˘ ¶·Ó/ÎÔ‡ NÔÛÔÎÔÌ›Ԣ Iˆ·ÓÓ›ÓˆÓ Î·È ÙËÓ ¶·È‰È·ÙÚÈ΋ KÏÈÓÈ΋ ÙÔ˘ ¶·Ó/ÎÔ‡ NÔÛÔÎÔÌ›Ԣ KÚ‹Ù˘ Ì ÙË Û˘ÁηٿıÂÛË ÙˆÓ ÁÔÓ¤ˆÓ ÌÂÙ¿ ·fi ÂÓË̤ڈۋ ÙÔ˘˜. ¶ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó Ù· Â›‰· Ù˘ 25OHD, ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ (Ca), ÙÔ˘ ʈÛÊfiÚÔ˘ (Pi), Ù˘ ·Ú·ıÔÚÌfiÓ˘ (iPTH), Ù˘ ÔÛÙÂÔηÏÛ›Ó˘ (OC) Î·È ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ (ALP) ÛÙÔÓ ÔÚfi ÌËÙ¤ÚˆÓ Î·È ÓÂÔÁÓÒÓ Î·Ù¿ ÙËÓ ÚÒÙË Â‚‰ÔÌ¿‰· ÌÂÙ¿ ÙË Á¤ÓÓËÛË Î·È ÛÙË Û˘Ó¤¯ÂÈ· ÛÙ· ‚Ú¤ÊË Î·Ù¿ ÙÔÓ 1Ô, 3Ô Î·È 6Ô Ì‹Ó· ˙ˆ‹˜. ∂›Û˘, ·Ú¿ÏÏËÏ· ηٷÁÚ¿ÊÔÓÙ·Ó ÙÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ (µ°) Î·È ÛÒÌ·ÙÔ˜ (B™) ηıÒ˜ Î·È ÙÔ Ì‹ÎÔ˜ ÛÒÌ·ÙÔ˜ (ª™) ÙˆÓ ÓÂÔÁÓÒÓ Î·È ‚ÚÂÊÒÓ.

O ÔÚÈÛÌfi˜ ·ÓÂ¿ÚÎÂÈ·˜ ‚ÈÙ·Ì›Ó˘ D Û ÌËÙ¤Ú˜ Î·È ÓÂÔÁÓ¿ ‰fiıËΠfiÙ·Ó Ù· Â›‰· Ù˘ 25OHD ÛÙÔÓ ÔÚfi ‹Ù·Ó <10 ng/ml (18). ∆Ô ·Û‚¤ÛÙÈÔ ÔÚÔ‡ Î·È Ë ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË ÌÂÙÚ‹ıËÎ·Ó Û ‚ÈÔ¯ËÌÈÎfi ·Ó·Ï˘Ù‹ Vision Analyser (Abbott, ∏¶∞). √ ʈÛÊfiÚÔ˜ ÔÚÔ‡ ÚÔÛ‰ÈÔÚ›ÛÙËΠ̠ÙË ¯ÚˆÌ·ÙÔÌÂÙÚÈ΋ ̤ıÔ‰Ô ÙˆÓ Ã¿ÏÏ· Î·È Û˘Ó. (19).

∆· Â›‰· Ù˘ ÔÛÙÂÔηÏÛ›Ó˘ ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó Ì ڷ‰ÈÔ·ÓÔÛÔÏÔÁÈ΋ ̤ıÔ‰Ô ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ·ÓÙȉڷÛÙ‹ÚÈ· Ù˘ ÂÙ·ÈÚ›·˜ Incstar Co (Stillwater, Minn., ∏¶∞). O Û˘ÓÙÂÏÂÛÙ‹˜ ‰È·Î‡Ì·ÓÛ˘ ÛÙÔÓ ›‰ÈÔ ÚÔÛ‰ÈÔÚÈÛÌfi (intra-assay) ‹Ù·Ó 10% Î·È ÌÂٷ͇ ‰ÔÎÈÌ·ÛÈÒÓ (inter-assay) 9,8%. OÈ Û˘ÁÎÂÓÙÚÒÛÂȘ Ù˘ 25OHD ÌÂÙÚ‹ıËÎ·Ó ÔÛÔÙÈο Ì ڷ‰ÈÔ˚ÛÔÙÔÈ΋ ̤ıÔ‰Ô ·ÓÙ·ÁˆÓÈÛÙÈ΋˜ ‰¤ÛÌ¢Û˘ Ì ÚˆÙ½ÓË, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ·ÓıÚÒÈÓË ‰ÂÛÌ¢ÙÈ΋ ÚˆÙ½ÓË Ù˘ ‚ÈÙ·Ì›Ó˘ D. ¶ÚÔËÁ‹ıËΠ·ÔÚˆÙÂ˝ÓˆÛË ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ ÔÚÔ‡ Ì ·ÎÂÙÔÓÈÙÚ›ÏÈÔ Î·È ÌÂÚÈÎfi˜ ηı·ÚÈÛÌfi˜ Ù˘ ‚ÈÙ·Ì›Ó˘ D ·fi ¿ÏÏ· ÏÈ›‰È· Ì ¯ÚˆÌ·ÙÔÁÚ·Ê›· Û ÌÈÎÚÔÛًϘ Sep-Pak C-18. ™ÙË Û˘Ó¤¯ÂÈ· Ô ÌÂÙ·‚ÔÏ›Ù˘ 25OHD Âί˘Ï›ÛÙËΠ̠·ÎÂÙÔÓÈÙÚ›ÏÈÔ Î·È ÌÂÙ¿ ·fi ÂÍ¿ÙÌÈÛË ·Ó·ÎÙ‹ıËΠÁÈ· ÂÂÍÂÚÁ·Û›· Û ·Èı·ÓfiÏË. ™Ù· ‰Â›ÁÌ·Ù· ÔÚÔ‡ ÚÔÛÙ¤ıËΠ25OHD ȯÓËıÂÙË̤ÓË Ì ڷ‰ÈÂÓÂÚÁfi ÈÛfiÙÔÔ ÙÚÈÙ›Ô˘ (25-hydroxy[23,24(n)3 H]cholecalciferol) ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ·Ó¿ÎÙËÛ‹˜ Ù˘ ÌÂÙ¿ ·fi fiÏ· Ù· ÛÙ¿‰È· ÂÂÍÂÚÁ·Û›·˜. ∏ ÚÔÌ‹ıÂÈ· Ù˘ ÙÚÈÙȈ̤Ó˘ ÌÔÚÊ‹˜ Ù˘ 25OHD ¤ÁÈÓ ·fi ÙËÓ Amersham Int. (∞ÁÁÏ›·). H Û˘ÁΤÓÙÚˆÛË Ù˘ 25OHD ÛÙÔÓ ÔÚfi ˘ÔÏÔÁ›ÛÙËΠ̠ÙË ‚Ô‹ıÂÈ· ÚfiÙ˘˘ ηÌ‡Ï˘. TÔ ÚfiÙ˘Ô ÌË Ú·‰ÈÂÓÂÚÁfi ‰È¿Ï˘Ì· Ù˘ 25OHD ·Ú·¯ˆÚ‹ıËΠ¢ÁÂÓÈο ·fi ÙË Hoffman La Roche. ∏ ̤ıÔ‰Ô˜ ·ÚÔ˘ÛÈ¿˙ÂÈ Û˘ÓÙÂÏÂÛÙ‹ ‰È·Î‡Ì·ÓÛ˘ (CV) ÌÂٷ͇ ‰È·ÊÔÚÂÙÈÎÒÓ ÚÔÛ‰ÈÔÚÈÛÌÒÓ (interassay) 10,5% Î·È ÛÙÔÓ ›‰ÈÔ ÚÔÛ‰ÈÔÚÈÛÌfi (intra-assay) 7,8%. √ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙÔ˘ ÔÏÈÎÔ‡ ÌÔÚ›Ô˘ Ù˘ ·Ú·ıÔÚÌfiÓ˘ (iPTH) Ú·ÁÌ·ÙÔÔÈ‹ıËΠ̠·ÓÔÛÔÂÓ˙˘ÌÈ΋ ̤ıÔ‰Ô ¯ËÌÂÈÔʈٷ‡ÁÂÈ·˜ ÛÙÂÚ¿˜ Ê¿Û˘ ‰‡Ô-ÂÚÈÔ¯ÒÓ Û ·Ó·Ï˘Ù‹ Immulite Ì intra- Î·È interassay CVs 6,3% Î·È 8,6% ·ÓÙ›ÛÙÔȯ·.

™Ù·ÙÈÛÙÈ΋ ∆· ·ÔÙÂϤÛÌ·Ù· ·Ó·Ï‡ıËÎ·Ó Ì ÙËÓ Ì¤ıÔ‰Ô Ù˘ ÁÚ·ÌÌÈ΋˜ ·Ó¿Ï˘Û˘ ηٿ Ì›· ‰È‡ı˘ÓÛË (one way ANOVA) ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙÔ Fisher’s PLSD ÙÂÛÙ ÁÈ· ÙË Û‡ÁÎÚÈÛË ÙˆÓ Ì¤ÛˆÓ ÙÈÌÒÓ ÙˆÓ ÙÚÈÒÓ ¯ÚÔÓÈÎÒÓ ÌÂÙÚ‹ÛÂˆÓ Ì ÙÔ ÚÒÙÔ ‰Â›ÁÌ· Î·È ÁÈ· ÙË Û‡ÁÎÚÈÛË ÌÂٷ͇ ÙˆÓ ÔÌ¿‰ˆÓ. √È ·Ó·Ï‡ÛÂȘ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙÔ ÏÔÁÈÛÌÈÎfi StatView Ù˘ SAS Institute Inc.

∞ÔÙÂϤÛÌ·Ù· ∆· Â›‰· Ù˘ 25OHD ÛÙÔÓ ÔÚfi ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ ÁÂÓÓ‹ıËÎ·Ó ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ¯ÂÈÌÂÚÈÓ‹˜ ÂÚÈfi‰Ô˘ ηٿ ÙËÓ ÚÒÙË Â‚‰ÔÌ¿‰· ˙ˆ‹˜ ‚Ú¤ıËÎ·Ó ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ· ·fi ÂΛӷ ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ ÁÂÓÓ‹ıËÎ·Ó ÛÙË ‰È¿ÚÎÂÈ· ÙˆÓ Î·ÏÔηÈÚÈÓÒÓ ÌËÓÒÓ (6,7±0,7 ng/ml ¤Ó·ÓÙÈ 10,1±0,9 ng/ml, p<0,01) (¶›Ó·Î·˜ 1). EÍÂÙ¿˙ÔÓÙ·˜ ηٿ ÂÚ›ÙˆÛË ÙȘ ÙÈ̤˜ ·˘ÙÒÓ ÙˆÓ ÓÂÔÁÓÒÓ ‚Ú¤ıËΠfiÙÈ ÛÙÔ 91,3% ÙˆÓ ÁÂÓÓËı¤ÓÙˆÓ ÙÔÓ ¯ÂÈÌÒÓ· Î·È ÛÙÔ 56% ·˘ÙÒÓ Ù˘ ıÂÚÈÓ‹˜ ÔÌ¿‰·˜ Ù· Â›‰· 25OHD ÛÙÔÓ ÔÚfi ‹Ù·Ó <10 ng/ml. ∏ ·Ú·ıÔÚÌfiÓË ÔÚÔ‡ ‹Ù·Ó ηٿ ̤ÛÔÓ fiÚÔ ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚË ÛÙË ¶·È‰È·ÙÚÈ΋ 2006;69:205-212

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·208

208

Õ. X¿ÏÏ· Î·È Û˘Ó.

¯ÂÈÌÂÚÈÓ‹ ÔÌ¿‰· (64,9±13,4 pg/ml) Û ۯ¤ÛË Ì ÙËÓ ıÂÚÈÓ‹ (33,9±4,4 pg/ml, p<0,01) (¶›Ó·Î·˜ 1). K·Ù¿ ÂÚ›ÙˆÛË ¿ÏÈ, ‚Ú¤ıËΠfiÙÈ ÔÈ ÙÈ̤˜ Ù˘ iPTH ‹Ù·Ó ÂÏ·ÊÚÒ˜ ¿Óˆ ·fi 60 pg/ml, Û ÌÂÚÈο ·fi Ù· ÓÂÔÁÓ¿ ÙÔ˘ ηÏÔηÈÚÈÔ‡ Ô˘ ›¯·Ó ¯·ÌËÏ¿ Â›‰· 25OHD, ÂÓÒ Û ·˘Ù¿ ÙÔ˘ ¯ÂÈÌÒÓ· Ù· Â›‰· iPTH ¤Êı·Û·Ó Û Ôχ ˘„ËϤ˜ ÙÈ̤˜ (100-200 pg/ml). A˘Ùfi Û˘Ó¤‚·ÈÓ ·Ú¿ ÙÔ fiÙÈ ÔÈ ÙÈ̤˜ ·Û‚ÂÛÙ›Ô˘ ÔÚÔ‡ Û fiÏ· Ù· ÓÂÔÁÓ¿ ‹Ù·Ó ¿Óˆ ·fi 8,5 mg/dl Î·È Î·Ù¿ ̤ÛÔÓ fiÚÔ ‰ÂÓ ‰È¤ÊÂÚ ÌÂٷ͇ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ (9,73±0,20 ¤Ó·ÓÙÈ 9,91±0,15 mg/dl) (¶›Ó·Î·˜ 1). √È ÌËÙ¤Ú˜ Ô˘ ‚Ú¤ıËÎ·Ó Ì ¯·ÌËÏ¿ Â›‰· 25OHD (<10 ng/ml) ‹Ù·Ó ÏÈÁfiÙÂÚ˜ ·fi Ù· ÓÂÔÁÓ¿ Î·È ‹Ù·Ó ηٷÓÂÌË̤Ó˜ ÂÍ›ÛÔ˘ ÌÂٷ͇ ÙˆÓ ‰‡Ô ÂÔ¯ÒÓ (35% ÙÔ ¯ÂÈÌÒÓ· Î·È 31% ÙÔ Î·ÏÔη›ÚÈ) ÂÓÒ ‰ÂÓ ˘‹Ú¯·Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ Î·È ˆ˜ ÚÔ˜ ÙȘ ̤Û˜ ÙÈ̤˜ ÙˆÓ ÂÈ¤‰ˆÓ ÙÔ˘˜ (10,8±1,0 ¤Ó·ÓÙÈ 12,9±1,3 ng/ml, ·ÓÙ›ÛÙÔȯ·) (¶›Ó·Î·˜ 2). ∞ÎfiÌË ÌÈÎÚfiÙÂÚÔ˜ ·ÚÈıÌfi˜ ·fi ·˘Ù¤˜ ›¯Â ˘„ËÏ‹ (>60 pg/ml) iPTH (4% ÙÔÓ ¯ÂÈÌÒÓ· Î·È 12,5% ÙÔ Î·ÏÔη›ÚÈ). √È Ì¤Û˜ ÙÈ̤˜ ÙˆÓ ÂÈ¤‰ˆÓ Ù˘ ·Ú·ıÔÚÌfiÓ˘ ÙÔ˘˜ ‹Ù·Ó 15,2±3,5 Î·È 24,8±4,5 pg/ml ÁÈ· ÙÔÓ ¯ÂÈÌÒÓ· Î·È ÙÔ Î·ÏÔη›ÚÈ ·ÓÙ›ÛÙÔȯ·, ηıÒ˜ Î·È ÛÙȘ ¿ÏϘ ·Ú·Ì¤ÙÚÔ˘˜ Ô˘ ÌÂÏÂÙ‹ıËÎ·Ó ‰ÂÓ ‚Ú¤ıËÎ·Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ (¶›Ó·Î·˜ 2). √È ÙÈ̤˜ ʈÛÊfiÚÔ˘ ‚Ú¤ıËÎ·Ó Ó· Â›Ó·È ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ˜ ÛÙ· ÓÂÔÁÓ¿ Ô˘ ÁÂÓÓ‹ıË-

Î·Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ¯ÂÈÌÒÓ· (7,43±0,38 mg/dl) ¤Ó·ÓÙÈ ·˘ÙÒÓ ÙÔ˘ ηÏÔηÈÚÈÔ‡ (6,27±0,23 mg/dl, p<0,01). ¶·Ú¿ÏÏËÏ· ÔÈ Ì¤Û˜ ÙÈ̤˜ ÙˆÓ ‰ÂÈÎÙÒÓ ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙˆÓ ÔÛÙÒÓ ÛÙÔÓ ÔÚfi, Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ Î·È Ù˘ ÔÛÙÂÔηÏÛ›Ó˘, ‰ÂÓ ‰È¤ÊÂÚ·Ó ÌÂٷ͇ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ (¶›Ó·Î·˜ 1). Ÿˆ˜ Â›Û˘ ‰ÂÓ ‰È¤ÊÂÚ ÙÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ (3314±56 Î·È 3158±69 ÁÚ.) Î·È ÙÔ Ì‹ÎÔ˜ ÛÒÌ·Ùfi˜ ÙÔ˘˜ (51,5±0,4 Î·È 52,1±0,5 ÂηÙ.). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ 3 ÌËÓÒÓ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ·Ú·ÙËÚ‹ıËΠÌÈ· ·˘ÍËÙÈ΋ Ù¿ÛË ÛÙ· Â›‰· Ù˘ 25OHD ÛÙÔÓ ÔÚfi Ù˘ ÔÌ¿‰·˜ ÙˆÓ ¯ÂÈÌÂÚÈÓÒÓ ÓÂÔÁÓÒÓ (·fi 6,7±0,7 Û 9,4±1,9 Î·È 11,3±1,6 ng/ml, p=0,08) ÂÓÒ ·Ú¤ÌÂÈÓ·Ó ÛÙ·ıÂÚ¿ ÛÙË ıÂÚÈÓ‹ ÔÌ¿‰· (·fi 10,1±0,9 Û 9,0±1,7 Î·È 9,8±1,2 ng/ml) (¶›Ó·Î·˜ 1). ∆ÔÓ 6o Ì‹Ó· ˙ˆ‹˜ Ë 25OHD ›¯Â ·˘ÍËı› ηٿ ̤ÛÔÓ fiÚÔ ÛËÌ·ÓÙÈο Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ÓÂÔÁÓÒÓ (Û 19,4±2,8 ng/ml, p<0,0001 ÛÙË ¯ÂÈÌÂÚÈÓ‹ ÔÌ¿‰· Î·È Û 13,3±1,6 ng/ml ÛÙË ıÂÚÈÓ‹ ÔÌ¿‰·, p<0,05) Û ۇÁÎÚÈÛË Ì ٷ Â›‰· ηٿ ÙË Á¤ÓÓËÛË (¶›Ó·Î·˜ 1). ŸÌˆ˜ ÙÔ 30% ÙˆÓ ÓÂÔÁÓÒÓ ÁÂÓÓËÌ¤ÓˆÓ ÙÔ Î·ÏÔη›ÚÈ ‚Ú¤ıËÎ·Ó Ó· ¤¯Ô˘Ó ÙÈ̤˜ 25OHD <10 ng/ml, ÂÓÒ ÙÔ ÔÛÔÛÙfi ÂÎÂ›ÓˆÓ ·fi ÙËÓ ÔÌ¿‰· ÁÂÓÓË̤ÓË ÙÔÓ ¯ÂÈÌÒÓ· ‹Ù·Ó ηٿ ÙÈ ÌÈÎÚfiÙÂÚÔ (21%). ™ÙÔÓ 6Ô Ì‹Ó· ˙ˆ‹˜ Ô˘ ¤ÁÈÓÂ Ë ÙÂÏÂ˘Ù·›· ̤ÙÚËÛË, ۯ‰fiÓ fiÏ· Ù· ÓÂÔÁÓ¿ ›¯·Ó ÂÈÛ¤ÏıÂÈ ÛÙËÓ ·ÓÙ›ıÂÙË ÂÔ¯‹ ·fi ·˘Ù‹ Ô˘ ÁÂÓÓ‹ıËηÓ.

¶›Ó·Î·˜ 1. OÈ Ì¤Û˜ ÙÈ̤˜ (±SEM) ÛÙÔÓ ÔÚfi ·Û‚ÂÛÙ›Ô˘ (Ca), ʈÛÊfiÚÔ˘ (Pi), 25OHD, ·Ú·ıÔÚÌfiÓ˘ (iPTH), ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ (ALP), ÔÛÙÂÔηÏÛ›Ó˘ (OC) Î·È Ù· ‚¿ÚË Î·È Ì‹ÎË ÛÒÌ·ÙÔ˜ ÙÂÏÂÈfiÌËÓˆÓ ÓÂÔÁÓÒÓ Î·È ‚ÚÂÊÒÓ Ô˘ ı‹Ï·˙·Ó ·ÔÎÏÂÈÛÙÈο Î·È Â›¯·Ó ÁÂÓÓËı› ηٿ ÙË ‰È¿ÚÎÂÈ· ¯ÂÈÌÒÓ·-¿ÓÔÈ͢ (X) Î·È Î·ÏÔηÈÚÈÔ‡-ÊıÈÓÔÒÚÔ˘ (K) ÙÔ˘˜ ÚÒÙÔ˘˜ ¤ÍÈ Ì‹Ó˜ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ HÏÈΛ· Ca (mg/dl) Pi (mg/dl) 25OHD (ng/ml) iPTH (pg/ml) ALP (IU/L) OC (ng/ml) B¿ÚÔ˜ (ÁÚ) M‹ÎÔ˜ (cm)

1Ë Â‚‰ÔÌ¿‰· X K X K X K X K X K X K X K X K

9,73 ± 0,20 9,91 ± 0,15 7,43 ± 0,38a 6,27 ± 0,23c 6,7 ± 0,7a 10,1 ± 0,9c 64,9± 13,4a 33,9 ± 4,4c 164 ± 15 189 ± 14 32,0 ± 3,4 28,9 ± 3,5 3411 ± 96 3255 ± 95 51,5 ± 0,4 52,1 ± 0,5

1Ô˜ Ì‹Ó·˜

3Ô˜ Ì‹Ó·˜ *

10,15 ± 0,09 10,0 ± 0,18 6,43 ± 0,17** 6,28 ± 0,22 9,4± 1,9 9,0 ± 1,7 63,3 ± 7,7a 41,7 ± 5,9b 284 ± 18+ 266 ± 19** 21,8± 3,4* 20,6 ± 1,8* 4421 ± 169+ 4146 ± 140+ 55,6 ± 0,7+ 55,0 ± 0,4+

*p<0,05, **p<0,01, ***p<0,001, +p<0,0001 Û ۇÁÎÚÈÛË Ì ÙÔ 1Ô ‰Â›ÁÌ· a vs b p<0,05, a vs c p<0,01 Û‡ÁÎÚÈÛË ÌÂٷ͇ ÙˆÓ ÔÌ¿‰ˆÓ ¶·È‰È·ÙÚÈ΋ 2006;69:205-212

6Ô˜ Ì‹Ó·˜ ***

10,39 ± 0,11 10,42 ± 0,11* 5,61 ± 0,18+ 5,57 ± 0,15* 11,3 ± 1,6 9,8 ± 1,2 96,7 ± 21,4 59,4 ± 21,8* 287 ± 21+ 298 ± 21*** 15,6 ± 1,9***,a 20,9 ± 2,3b 6188 ± 179+ 5847 ± 196+ 61,7 ± 0,5+ 61,8 ± 0,8+

10,47 ± 0,11*** 10,46 ± 0,14* 5,62 ± 0,12+ 5,55 ± 0,11* 19,4± 2,8+ 13,3 ± 1,6 26,8± 3,5a 50,3 ± 7,9c 219 ± 17* 288 ± 35*** 21,5 ± 3,4* 26,5 ± 2,8 7875 ± 258+ 7427 ± 256+ 67,8 ± 0,6+ 67,8 ± 1,0+

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·209

209

BÈÙ·Ì›ÓË D Î·È ÌËÙÚÈÎfi˜ ıËÏ·ÛÌfi˜

¶›Ó·Î·˜ 2. OÈ Ì¤Û˜ ÙÈ̤˜ (±SEM) ÛÙÔÓ ÔÚfi ·Û‚ÂÛÙ›Ô˘ (Ca), ʈÛÊfiÚÔ˘ (Pi), 25OHD, ·Ú·ıÔÚÌfiÓ˘ (iPTH) Î·È ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ (ALP) ÙˆÓ ÌËÙ¤ÚˆÓ Ô˘ Á¤ÓÓËÛ·Ó ÙÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿ ηٿ ÙË ‰È¿ÚÎÂÈ· ¯ÂÈÌÒÓ·-¿ÓÔÈ͢ Î·È Î·ÏÔηÈÚÈÔ‡-ÊıÈÓÔÒÚÔ˘

Ca (mg/dl) Pi (mg/dl) 25OHD (ng/ml) iPTH (pg/ml) ALP (IU/L)

ÃÂÈÌÒÓ·˜ÕÓÔÈÍË

∫·ÏÔη›ÚȺıÈÓfiˆÚÔ

P

9,16 ± 0,11 3,82 ± 0,17 10,8 ± 1,0 15,2 ± 3,5 89 ± 6

8,85 ± 0,15 3,96 ± 0,15 12,9 ± 1,3 24,8 ± 4,5 100 ± 5

NS NS NS NS NS

√È Û˘ÁÎÂÓÙÚÒÛÂȘ Ù˘ iPTH ÛÙÔÓ ÔÚfi ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ 3 ÌËÓÒÓ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ·˘Í‹ıËÎ·Ó ÂÚ·ÈÙ¤Úˆ ÛÙ· ÓÂÔÁÓ¿ Ù˘ ¯ÂÈÌÂÚÈÓ‹˜ (·fi 64,9±1,4 Û 96,7±21,4 pg/ml) ·ÏÏ¿ Î·È Ù˘ ıÂÚÈÓ‹˜ ÔÌ¿‰·˜ (·fi 33,9±4,4 Û 59,4±21,8 pg/ml, p<0,05) (¶›Ó·Î·˜ 1). ™ÙÔÓ 6Ô Ì‹Ó· ˙ˆ‹˜ ·Ú¿ ÙÔ fiÙÈ Ë ¯ÂÈÌÂÚÈÓ‹ ÔÌ¿‰· ›¯Â ÍÂÎÈÓ‹ÛÂÈ Ì ÛËÌ·ÓÙÈο ÈÔ ˘„ËÏ¿ Â›‰· (64,9±1,4 ¤Ó·ÓÙÈ 33,9±4,4 pg/ml p<0,05) ‚Ú¤ıËΠ̠¯·ÌËÏfiÙÂÚ· Â›‰· iPTH (26,8±3,5 pg/ml, p<0,01) ·fi ÙË ıÂÚÈÓ‹ ÔÌ¿‰· (50,3±7,9 pg/ml). ∫·ı’ fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘ ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙÔÓ ÔÚfi Û fiÏ· Ù· ÓÂÔÁÓ¿ ‹Ù·Ó ÛÂ Ê˘ÛÈÔÏÔÁÈο Â›‰· (>8,5 mg/dl) Î·È Î·Ù¿ ̤ÛÔÓ fiÚÔ ‰ÂÓ ‰È¤ÊÂÚ·Ó ÛËÌ·ÓÙÈο ÌÂٷ͇ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ. ¶·Ú·ÙËÚ‹ıËÎ·Ó ·˘Í‹ÛÂȘ ̤۷ ÛÙ· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ÙÔÓ 6Ô Ì‹Ó· (¶›Ó·Î·˜ 1). √È Ì¤Û˜ ÙÈ̤˜ ÙÔ˘ ʈÛÊfiÚÔ˘ ÔÚÔ‡ ¿Ú¯ÈÛ·Ó Ó· ÌÂÈÒÓÔÓÙ·È ÌÂÙ¿ ÙËÓ ÚÒÙË Â‚‰ÔÌ¿‰· ˙ˆ‹˜ Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ÓÂÔÁÓÒÓ Î·È Î·ÙfiÈÓ ‹Ù·Ó Û˘ÁÎÚ›ÛÈ̘ ÌÂٷ͇ ÙÔ˘˜ (¶›Ó·Î·˜ 1). ∏ ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË ·˘Í‹ıËΠÛËÌ·ÓÙÈο Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ÓÂÔÁÓÒÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘, ·fi 164±15 Û 219±17 IU/L, p<0,05 ÛÙË ¯ÂÈÌÂÚÈÓ‹ ÔÌ¿‰· ÓÂÔÁÓÒÓ Î·È ·fi 189±14 Û 288±35 IU/L, p<0.001 ÛÙ· ÓÂÔÁÓ¿ ÁÂÓÓË̤ӷ ÙË ıÂÚÈÓ‹ ÂÚ›Ô‰Ô (¶›Ó·Î·˜ 1).

Y‹Ú¯Â ‰È·ÊÔÚÔÔ›ËÛË ÌÂٷ͇ ÙˆÓ ÔÌ¿‰ˆÓ ÛÙ· Â›‰· Ù˘ ÔÛÙÂÔηÏÛ›Ó˘. ™ÙË ¯ÂÈÌÂÚÈÓ‹ ÔÌ¿‰· ÌÈ· ÙÒÛË Ù˘ ÔÛÙÂÔηÏÛ›Ó˘ ÛÙÔÓ ÔÚfi ·Ú·ÙËÚ‹ıËΠ̤¯ÚÈ ÙÔÓ 6Ô Ì‹Ó· (·fi 32,0±3,4 Û 21,5±3,4 pg/ml, p<0,05), ÂÓÒ ÛÙÔÓ 3Ô Ì‹Ó· ‹Ù·Ó ·ÎfiÌ· ÌÂÁ·Ï‡ÙÂÚË (Û 15,6±1,9, p<0,001). H ̤ÛË ÙÈÌ‹ Ù˘ ‹Ù·Ó ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚË ·fi ·˘Ù‹ Ù˘ ıÂÚÈÓ‹˜ ÔÌ¿‰·˜ (20,9±2,3 pg/ml, p<0,05) (¶›Ó·Î·˜ 1). ™ÙË ıÂÚÈÓ‹ ÔÌ¿‰· ÙˆÓ ÓÂÔÁÓÒÓ Ù· Â›‰· Ù˘ ÔÛÙÂÔηÏÛ›Ó˘ ‰ÂÓ ÌÂÙ·‚Ï‹ıËÎ·Ó ÛËÌ·ÓÙÈο ηٿ

ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘ (¶›Ó·Î·˜ 1). ∫·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ·fi ÙÔÓ ÚÒÙÔ Ì‹Ó· Ù˘ ˙ˆ‹˜ Î·È ÌÂÙ¿, ÌÂÚÈο ÓÂÔÁÓ¿ ›¯·Ó Â›‰· ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡ >300 IU/L, Ô˘ Ù›ıÂÙ·È Û·Ó ·ÓÒÙÂÚÔ ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ (20). ∆· ·ÓÙ›ÛÙÔȯ· ÔÛÔÛÙ¿ ÁÈ· ÙË ¯ÂÈÌÂÚÈÓ‹ ÔÌ¿‰· ‹Ù·Ó 25,8%, 48,4% Î·È 14,3% ÛÙÔ˘˜ Ì‹Ó˜ 1, 3 Î·È 6 Î·È ÁÈ· ÙË ıÂÚÈÓ‹ ÔÌ¿‰· 18,8%, 27,3% Î·È 24%. ∆· ̤۷ ‚¿ÚË Î·È Ù· Ì‹ÎË ÛÒÌ·ÙÔ˜ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ ‰ÂÓ ‰È¤ÊÂÚ·Ó ÌÂٷ͇ ÙÔ˘˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘ (¶›Ó·Î·˜ 1).

™˘˙‹ÙËÛË √ ÚÔ‚ÏËÌ·ÙÈÛÌfi˜ ÁÈ· ÙÔ ·Ó ˘¿Ú¯ÂÈ ·Ó¿ÁÎË ‹ fi¯È Û˘ÌÏËڈ̷ÙÈ΋˜ ¯ÔÚ‹ÁËÛ˘ ‚ÈÙ·Ì›Ó˘ D ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ ÌËÓÒÓ ˙ˆ‹˜ ÛÙ· ÓÂÔÁÓ¿ Ô˘ ıËÏ¿˙Ô˘Ó ·ÔÎÏÂÈÛÙÈο, Û˘Ó¯›˙ÂÈ Ó· ˘¿Ú¯ÂÈ ·ÎfiÌ· Û‹ÌÂÚ·. ∞fi Ù· Â˘Ú‹Ì·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, Â›Ó·È ÂÌÊ·Ó¤˜ fiÙÈ ·Ú¿ ÙËÓ ËÏÈÔÊ¿ÓÂÈ· ÛÙËÓ ∂ÏÏ¿‰·, Ë ·ÓÂ¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ D ·Ú·ÙËÚÂ›Ù·È Û ¤Ó· ˘„ËÏfi ÔÛÔÛÙfi ÂÏÏËÓ›‰ˆÓ ÌËÙ¤ÚˆÓ Î·È ÙˆÓ ÙÂÏÂÈfiÌËÓˆÓ ÓÂÔÁÓÒÓ ‹ ‚ÚÂÊÒÓ Û ·ÔÎÏÂÈÛÙÈÎfi ıËÏ·ÛÌfi. ªÂÙ¿ ÙË Á¤ÓÓËÛË Î·È ÁÈ· ÙÔ˘˜ ÚÒÙÔ˘˜ ¤ÍÈ Ì‹Ó˜ ˙ˆ‹˜ Ô ·ÔÎÏÂÈÛÙÈÎfi˜ ıËÏ·ÛÌfi˜ ‰ÂÓ Ê·›ÓÂÙ·È Ó· ·Ú¤¯ÂÈ Â·Ú΋ ÔÛfiÙËÙ· ‚ÈÙ·Ì›Ó˘ D ·ÎfiÌ· Î·È ÛÙ· ÓÂÔÁÓ¿ Ô˘ ÁÂÓÓ‹ıËÎ·Ó ÙÔ Î·ÏÔη›ÚÈ ÒÛÙ ӷ ‰È·ÙËÚÔ‡Ó ÈηÓÔÔÈËÙÈο Â›‰· 25OHD Î·È ÙÔ˘˜ ¯ÂÈÌÂÚÈÓÔ‡˜ Ì‹Ó˜. A˘Ùfi ÙÔ Â‡ÚËÌ· ÂÓÈÛ¯‡ÂÙ·È Î·È ·fi Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ˘„ËÏÒÓ ÂÈ¤‰ˆÓ ·Ú·ıÔÚÌfiÓ˘ Ô˘ Ì·˙› Ì ٷ ¯·ÌËÏ¿ Â›‰· 25OHD Ô‰ËÁÔ‡Ó ÛÙË ıˆڛ· ÁÈ· ‚ÈÔ¯ËÌÈÎfi Ú·¯ÈÙÈÛÌfi Û ¤Ó· ˘„ËÏfi ÔÛÔÛÙfi ÙˆÓ ‚ÚÂÊÒÓ Ô˘ ÌÂÏÂÙ‹ıËÎ·Ó Ì¤¯ÚÈ ÙÔ˘˜ ÚÒÙÔ˘˜ 6 Ì‹Ó˜ ˙ˆ‹˜ Î·È ·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ÂÔ¯‹ Á¤ÓÓËÛ˘. ∞˘Ùfi Ì¿ÏÏÔÓ ÔÊ›ÏÂÙ·È ÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ Î·È Ù· ÓÂÔÁÓ¿ Ô˘ ÁÂÓÓ‹ıËÎ·Ó ÙËÓ ÂÚ›Ô‰Ô Î·ÏÔηÈÚÈÔ‡-ÊıÈÓÔÒÚÔ˘, ÌÂÙ¿ ·fi 3-4 Ì‹Ó˜ ›¯·Ó ÌÂÈ ÛÙË ¯ÂÈÌÂÚÈÓ‹ ÂÚ›Ô‰Ô. ¢È¿ÊÔÚÔÈ ·Ú¿ÁÔÓÙ˜ Èı·ÓfiÓ Ó· ¢ı‡ÓÔÓÙ·È ÁÈ’ ·˘Ù¿ Ù· Â˘Ú‹Ì·Ù·. ŒÓ·˜ ·fi ·˘ÙÔ‡˜ ÌÔÚ› Ó· Â›Ó·È ÙÔ fiÙÈ Ù· ÙÚfiÊÈÌ· ÛÙËÓ ∂ÏÏ¿‰· ‰ÂÓ ÂÌÏÔ˘Ù›˙ÔÓÙ·È Ì ‚ÈÙ·Ì›ÓË D ‹ ·Ó ÂÌÏÔ˘Ù›˙ÔÓÙ·È, Â›Ó·È Ì Ôχ ÌÈÎÚ¤˜ ÔÛfiÙËÙ˜, Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ ÙˆÓ Á·Ï¿ÙˆÓ Ì ÂÍ·›ÚÂÛË ·˘ÙÒÓ ÁÈ· ÙË ‚ÚÂÊÈ΋ ËÏÈΛ·. OÈ ·ÓÙËÏȷΤ˜ Îڤ̘ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û ¢Ú›· Îϛ̷η ·fi ÙȘ Á˘Ó·›Î˜, ηıÒ˜ Î·È Ô Û‡Á¯ÚÔÓÔ˜ ÙÚfiÔ˜ ˙ˆ‹˜ ÙÔ˘˜ ÛÙȘ ·ÛÙÈΤ˜ ÂÚÈÔ¯¤˜ - Ô˘ ÌÂÙ·ÊÚ¿˙ÂÙ·È Û ÌÂÁ·Ï‡ÙÂÚÔ ¶·È‰È·ÙÚÈ΋ 2006;69:205-212

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·210

210

Õ. X¿ÏÏ· Î·È Û˘Ó.

̤ÚÔ˜ ÙÔ˘ ¯ÚfiÓÔ˘ ÙÔ˘˜ Û ÂÛˆÙÂÚÈÎÔ‡˜ ¯ÒÚÔ˘˜ - ÌÔÚ› Ó· ·›˙Ô˘Ó ÛËÌ·ÓÙÈÎfi ÚfiÏÔ. √È Á˘Ó·›Î˜-ÌËÙ¤Ú˜ ·˘Ù‹˜ Ù˘ ÌÂϤÙ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ ÙÔ˘˜ ¤·ÈÚÓ·Ó ÛÙËÓ ÏÂÈÔÓfiÙËÙ¿ ÙÔ˘˜ ÌfiÓÔ Û˘Ìϋڈ̷ ·Û‚ÂÛÙ›Ô˘ (1 ÁÚ./Ë̤ڷ), ·ÏÏ¿ fi¯È ‚ÈÙ·Ì›ÓË D. ªÂϤÙ˜ ·fi ¿ÏϘ ÌÂÛÔÁÂȷΤ˜ ¯ÒÚ˜ fiˆ˜ ÙË °·ÏÏ›· Î·È ÙËÓ πÙ·Ï›·, ·Ó·Ê¤ÚÔ˘Ó ·ÚfiÌÔÈ· Ì ٷ ‰Èο Ì·˜ ·ÔÙÂϤÛÌ·Ù·. ™Â ÌÈ· ÔÌ¿‰· ÂÁ·ˆÓ Á˘Ó·ÈÎÒÓ ·fi ÙË ‚ÔÚÂÈÔ‰˘ÙÈ΋ °·ÏÏ›·, ÛÙËÓ ÔÔ›· ‰ÂÓ ¯ÔÚËÁ‹ıËÎÂ Û˘Ìϋڈ̷ ‚ÈÙ·Ì›Ó˘ D, Ù· Â›‰¿ ÙÔ˘˜ ηٿ ÙË Á¤ÓÓËÛË ‚Ú¤ıËÎ·Ó Ôχ ¯·ÌËÏ¿ (<4 ng/ml).

AÓÙ›ıÂÙ· Û ÂΛӘ Ô˘ ÙÔ˘˜ ›¯Â ¯ÔÚËÁËı› ‚ÈÙ·Ì›ÓË D ›Ù Û ‰fiÛÂȘ 1000 IU/Ë̤ڷ ÁÈ· ÙÔ ÙÂÏÂ˘Ù·›Ô ÙÚ›ÌËÓÔ Ù˘ ·ËÛ˘, ›Ù Û ̛· ÂÓÈ·›· ‰fiÛË 5 mg ÙÔÓ 7Ô Ì‹Ó·) ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο (>10 ng/ml) (14). ™Â ¿ÏÏË ÌÂϤÙË ·fi ÙË ‚fiÚÂÈ· °·ÏÏ›·, ·Ó·Ê¤ÚıËΠfiÙÈ ÙÔ 24% ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ ÁÂÓÓ‹ıËÎ·Ó ÙËÓ ÂÚ›Ô‰Ô ¯ÂÈÌÒÓ·¿ÓÔÈ͢ ·fi ÌËÙ¤Ú˜ Ô˘ ‰ÂÓ ¤Ï·‚·Ó ‚ÈÙ·Ì›ÓË D ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ ÙÔ˘˜, ›¯Â ·ÓÂ¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ D (25OHD <12 ng/ml Î·È iPTH >60 ng/L) (15). ™Â ÌÂϤÙË ·fi ÙËÓ IÙ·Ï›· ‚Ú¤ıËÎ·Ó ÂÚÈÙÒÛÂȘ Ú·¯ÈÙÈÛÌÔ‡ ·fi ·ÓÂ¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ D, ÛÙÔ ‰È¿ÛÙËÌ· ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ ˙ˆ‹˜, Û ·È‰È¿ Â˘Úˆ·˚΋˜ ηٷÁˆÁ‹˜. E›¯·Ó ÁÂÓÓËı› ÙÂÏÂÈfiÌËÓ· ·fi ÌËÙ¤Ú˜ Ô˘ ‰ÂÓ ¤·ÈÚÓ·Ó Û˘Ìϋڈ̷ ‚ÈÙ·Ì›Ó˘ D ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ¯ÂÈÌÒÓ·, ÂÓÒ ÙÚ¤ÊÔÓÙ·Ó ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi ıËÏ·ÛÌfi, ÁÈ· ÙÔ˘˜ ÚÒÙÔ˘˜ ¤ÍÈ Ì‹Ó˜ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ (13). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË Û¯Â‰fiÓ fiÏ· Ù· ÓÂÔÁÓ¿ Ô˘ ÁÂÓÓ‹ıËÎ·Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ¯ÂÈÌÂÚÈÓÒÓ ÌËÓÒÓ Î·È Û¯Â‰fiÓ Ù· ÌÈÛ¿ ·fi ·˘Ù¿ Ô˘ ÁÂÓÓ‹ıËÎ·Ó ÙÔ˘˜ ηÏÔηÈÚÈÓÔ‡˜ Ì‹Ó˜ ›¯·Ó Â›‰· 25OHD <10 ng/ml ÙËÓ 1Ë Â‚‰ÔÌ¿‰· ˙ˆ‹˜. ª¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 6 ÌËÓÒÓ Î·È ¯ˆÚ›˜ ÙË Ï‹„Ë Û˘ÌÏËÚÒÌ·ÙÔ˜ ‚ÈÙ·Ì›Ó˘ D, ÙÔ ÔÛÔÛÙfi ÌÂÈÒıËΠÛËÌ·ÓÙÈο. ¶·Ú’ fiÏ· ·˘Ù¿ Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ·Ú¤ÌÂÈÓ ¤Ó· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi (21% Î·È 30% ·ÓÙ›ÛÙÔȯ·) Ì ¯·ÌËÏ¿ Â›‰·, Ô˘ Ô‰ËÁ› Û ÚÔ‚ÏËÌ·ÙÈÛÌfi.

E›Û˘ Ì›· ÛÙȘ ÙÚÂȘ ÌËÙ¤Ú˜ ‚Ú¤ıËΠ̠¯·ÌËÏ¿ Â›‰· ‚ÈÙ·Ì›Ó˘ D (<10 ng/ml) Ï›ÁÔ ÌÂÙ¿ ÙÔÓ ÙÔÎÂÙfi ·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ÂÔ¯‹. ™Â Ì›· ÌÂϤÙË fiÔ˘ ÔÈ ÌËÙ¤Ú˜ Ï¿Ì‚·Ó·Ó ‚ÈÙ·Ì›ÓË D (400 IU/Ë̤ڷ) ÙfiÛÔ Î·Ù¿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË fiÛÔ Î·È Î·Ù¿ ÙË Á·ÏÔ˘¯›·, ‰ÂÓ ‚Ú¤ıËΠ‰È·ÊÔÚ¿ ÛÙ· Â›‰· Ù˘ 25OHD ‹ Ù˘ PTH ÔÚÔ‡ ÌÂٷ͇ ÙˆÓ ·ÔÎÏÂÈÛÙÈο ıËÏ·˙fiÓÙˆÓ ‚ÚÂÊÒÓ ÙÔ˘˜, ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ ¤ÍÈ ÌËÓÒÓ Ù˘ ˙ˆ‹˜ ÙÔ˘˜, ›Ù Û ·˘Ù¿ ¶·È‰È·ÙÚÈ΋ 2006;69:205-212

¯ÔÚËÁÔ‡ÓÙ·Ó ‚ÈÙ·Ì›ÓË D ›Ù fi¯È (21). ∂ÈϤÔÓ, fiÏ· Ù· ‚Ú¤ÊË Â›¯·Ó ÈηÓÔÔÈËÙÈο Â›‰· ‚ÈÙ·Ì›Ó˘ D ÛÙÔ ·›Ì·, ·Ó Î·È ÛÙÔÓ 6Ô Ì‹Ó· Ù˘ ˙ˆ‹˜ ÙÔ˘˜ ÂΛӷ Ô˘ ‰ÂÓ ¤·ÈÚÓ·Ó ‚ÈÙ·Ì›ÓË D ÂÌÊ¿ÓÈÛ·Ó ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ· Â›‰·. ¶ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔÊ¢¯ı› ÙÔ Úfi‚ÏËÌ· ·ÓÂ¿ÚÎÂÈ·˜ ‚ÈÙ·Ì›Ó˘ D ÛÙ· ÓÂÔÁÓ¿ Î·È Ù· ‚Ú¤ÊË ÁÈ· ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ ˙ˆ‹˜ ÙÔ˘˜, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ÂÔ¯‹, ÛÙȘ ¯ÒÚ˜ fiÔ˘ Ù· ÙÚfiÊÈÌ· ‰ÂÓ ÂÌÏÔ˘Ù›˙ÔÓÙ·È Ì ‚ÈÙ·Ì›ÓË D, ›Ù ÔÈ ÌËÙ¤Ú˜ Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÔ˘Ó Û˘Ìϋڈ̷ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ Î·È Ù˘ Á·ÏÔ˘¯›·˜ ‹/Î·È Ù· ‚Ú¤ÊË ı· Ú¤ÂÈ Ó· ÂÓÈÛ¯‡ÔÓÙ·È Ì ηٿÏÏËÏÔ Û˘Ìϋڈ̷ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÚÈfi‰Ô˘ ·ÔÎÏÂÈÛÙÈ΋˜ Û›ÙÈÛ˘ ÙÔ˘˜ Ì ÌËÙÚÈÎfi Á¿Ï·. ∞˘Ùfi Ê·›ÓÂÙ·È Ó· ıˆÚÂ›Ù·È ·Ó·Áη›Ô ·ÎfiÌË Î·È Û ¯ÒÚ˜ Ì ÌÂÁ¿ÏË ËÏÈÔÊ¿ÓÂÈ· fiˆ˜ Ë ∂ÏÏ¿‰·. §·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ·ÎfiÌË Î·È Ù· ‚Ú¤ÊË ÌËÙ¤ÚˆÓ Ì ÈηÓÔÔÈËÙÈο Â›‰· ‚ÈÙ·Ì›Ó˘ D ÂÌÊ·Ó›˙Ô˘Ó ·ÓÂ¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ D fiÙ·Ó ‰ÂÓ Ï·Ì‚¿ÓÔ˘Ó Û˘Ìϋڈ̷ ÌÂÙ¿ ·fi 8 ‚‰ÔÌ¿‰Â˜ Á·ÏÔ˘¯›·˜ (17), ÂÓÈÛ¯‡ÂÈ ÙËÓ ·Ú·¿Óˆ Û‡ÛÙ·ÛË. ∆Ô ÂÚÒÙËÌ· Ô˘ ÛÙË Û˘Ó¤¯ÂÈ· Ú¤ÂÈ Ó· ··ÓÙËı›, Â›Ó·È Â¿Ó Ë ¯ÔÚ‹ÁËÛË Û˘ÌÏËÚÒÌ·ÙÔ˜ ÛÙËÓ ÌËÙ¤Ú· ÌfiÓÔ, ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ ‹/Î·È Ù˘ Á·ÏÔ˘¯›·˜, Â›Ó·È ·ÚÎÂÙ‹ Ó· ‰È·ÙËÚ‹ÛÂÈ ÙËÓ 25OHD ÙÔ˘ ‚Ú¤ÊÔ˘˜ Û ÈηÓÔÔÈËÙÈο Â›‰·. ªÂÚÈÎÔ› ÂÚ¢ÓËÙ¤˜ (22,23) ‚Ú‹Î·Ó fiÙÈ ÁÈ· Ó· ˘¿Ú¯ÂÈ ÈηÓÔÔÈËÙÈ΋ Â›‰Ú·ÛË ÛÙȘ Û˘ÁÎÂÓÙÚÒÛÂȘ Ù˘ 25OHD ÛÙÔ ıËÏ¿˙ÔÓ ÓÂÔÁÓfi, Ë ÌËÙ¤Ú· ¯ÚÂÈ¿˙ÂÙ·È Ó· Ï·Ì‚¿ÓÂÈ Â͈ÁÂÓÒ˜ 2.000-4.000 IU ‚ÈÙ·Ì›Ó˘ D ÙËÓ Ë̤ڷ. H ¯ÔÚ‹ÁËÛË Û˘ÌÏËÚÒÌ·ÙÔ˜ ÛÙ· ›‰È· Ù· ‚Ú¤ÊË Ê·›ÓÂÙ·È Ó· Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ Û Ôχ ¯·ÌËÏfiÙÂÚ˜ ‰fiÛÂȘ (400 IU/Ë̤ڷ) (22). ∏ AÌÂÚÈηÓÈ΋ Aη‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ Û˘ÓÈÛÙ¿ ÛÙ· ‚Ú¤ÊË Ô˘ ˙Ô˘Ó ÛÙȘ Hӈ̤Ó˜ ¶ÔÏÈÙ›˜ ËÌÂÚ‹ÛÈ· ÚfiÛÏË„Ë ÙÔ˘Ï¿¯ÈÛÙÔÓ 200 IU ‚ÈÙ·Ì›Ó˘ D (24). ™ÙÔÓ ∫·Ó·‰¿, ÔÈ Û˘ÛÙ¿ÛÂȘ Ù˘ ÚÔËÁÔ‡ÌÂÓ˘ ‰ÂηÂÙ›·˜ ‹Ù·Ó ÁÈ· 400 IU/Ë̤ڷ ÛÙË ‰È¿ÚÎÂÈ· ÙˆÓ ¯ÂÈÌÂÚÈÓÒÓ ÌËÓÒÓ (25).

T· Â˘Ú‹Ì·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ·ÏÏ¿ Î·È ¿ÏÏˆÓ Ì ·ÚÂÌÊÂÚ‹ ·ÔÙÂϤÛÌ·Ù· ·ÔÎÙÔ‡Ó ÌÂÁ·Ï‡ÙÂÚË ‚·Ú‡ÙËÙ· ÌÂÙ¿ ·fi ÙȘ Û˘Ó¯Ҙ ·˘Í·ÓfiÌÂÓ˜ ÁÓÒÛÂȘ Ì·˜ ÁÈ· ÙȘ ‰Ú¿ÛÂȘ Ù˘ ‚ÈÙ·Ì›Ó˘ D. ŒÙÛÈ, ÂÎÙfi˜ ·fi ÙË ÁÓˆÛÙ‹ ‰Ú¿ÛË Ù˘ ÛÙÔÓ ÔÛÙÈÎfi ÌÂÙ·‚ÔÏÈÛÌfi Î·È ÙËÓ ·‡ÍËÛË, Ë ·ÓÂ¿ÚÎÂÈ¿ Ù˘ ÌÔÚ› Ó· ¤¯ÂÈ ‰˘ÛÌÂÓ›˜ ÂÈÙÒÛÂȘ Î·È Û ¿ÏÏ· Û˘ÛÙ‹Ì·Ù·, fiˆ˜ ÌÂÁ·Ï‡ÙÂÚË ÂÈÚÚ¤ÂÈ· Û ÏÔÈÌÒ‰ÂȘ

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ÓfiÛÔ˘˜ Î·È ÙË Ê˘Ì·Ù›ˆÛË (26,27), ÙËÓ ÙÂÚˉfiÓ· ÙˆÓ ‰ÔÓÙÈÒÓ (28) Î·È ·˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù· fiˆ˜ Ô ‰È·‚‹Ù˘ Ù‡Ô˘ I (29). À¿Ú¯Ô˘Ó Â›Û˘ ‰Â‰Ô̤ӷ fiÔ˘ Ë Û˘Ìو̷ÙÈ΋ ˘·Û‚ÂÛÙÈ·ÈÌ›· ˘Ô¯ˆÚ› ̤۷ ÛÙÔ ÚÒÙÔ 48ˆÚÔ ÌÂÙ¿ ÙË Á¤ÓÓËÛË Û ÓÂÔÁÓ¿ ÙˆÓ ÔÔ›ˆÓ ÔÈ ÌËÙ¤Ú˜ ¤·ÈÚÓ·Ó ‚ÈÙ·Ì›ÓË D ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÙÂÏÂ˘Ù·›Ô˘ ÙÚÈÌ‹ÓÔ˘ Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ (30). ª›· ¿ÏÏË ÌÂϤÙË ¤‰ÂÈÍ fiÙÈ Ë ÙÒÛË ÙˆÓ ÂÈ¤‰ˆÓ ·Û‚ÂÛÙ›Ô˘ ·›Ì·ÙÔ˜ Û 4 ËÌÂÚÒÓ ÓÂÔÁÓ¿ Ô˘ ›¯·Ó ÁÂÓÓËı› ·fi ÌËÙ¤Ú˜ Ô˘ ¤·ÈÚÓ·Ó ‚ÈÙ·Ì›ÓË D, ‹Ù·Ó ηٿ Ôχ ÌÈÎÚfiÙÂÚË ·fi ÂÎÂ›ÓˆÓ Ô˘ ÔÈ ÌËÙ¤Ú˜ ÙÔ˘˜ ‰ÂÓ Â›¯·Ó ¿ÚÂÈ (31). EÈϤÔÓ, Û fiÏ· Ù· ÓÂÔÁÓ¿ ÙÔ ·Û‚¤ÛÙÈÔ ·›Ì·ÙÔ˜ Ù˘ 4˘ Ë̤ڷ˜ ˙ˆ‹˜ ‚Ú¤ıËΠӷ ¤¯ÂÈ Û˘Û¯¤ÙÈÛË Ì ÙȘ Û˘ÁÎÂÓÙÚÒÛÂȘ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ, ÔÈ Ôԛ˜ Ì ÙË ÛÂÈÚ¿ ÙÔ˘˜ ›¯·Ó ¿ÌÂÛË Û¯¤ÛË Ì ٷ Â›‰· Ù˘ 25OHD Â›Û˘ ÛÙÔÓ ÔÌÊ¿ÏÈÔ ÏÒÚÔ.

Afi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‰ÂÓ Â›Ó·È Û·Ê‹˜ οÔÈ· Â›ÙˆÛË ÛÙÔÓ ÌÂÙ·‚ÔÏÈÛÌfi ÙˆÓ ÔÛÙÒÓ, ÂÎÙfi˜ ·fi ÙË ÛËÌ·ÓÙÈ΋ ÙÒÛË ÛÙ· Â›‰· ÔÛÙÂÔηÏÛ›Ó˘ Ô˘ ·Ú·ÙËÚ‹ıËΠÛÙË ¯ÂÈÌÂÚÈÓ‹ ÔÌ¿‰· ̤¯ÚÈ ÙÔÓ 6Ô Ì‹Ó· Ù˘ ˙ˆ‹˜. ∏ ıÂÚÈÓ‹ ÔÌ¿‰· ‰ÂÓ ÂÌÊ¿ÓÈÛ ÛËÌ·ÓÙÈΤ˜ ÌÂÙ·‚ÔϤ˜ ÛÙËÓ ÔÛÙÂÔηÏÛ›ÓË Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘. ™¯ÂÙÈο Ì ٷ Â›‰· Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘, Ì›· ¿ÓÔ‰Ô˜ ÙÔÓ ÚÒÙÔ Ì‹Ó· ÌÂÙ¿ ÙË Á¤ÓÓËÛË Ê¿ÓËÎÂ Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜, ÂÓÒ ÛÙË Û˘Ó¤¯ÂÈ· ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÌÂÁ¿Ï˜ ÌÂÙ·‚ÔϤ˜. À‹ÚÍÂ, fï˜, ¤Ó·˜ ÛËÌ·ÓÙÈÎfi˜ ·ÚÈıÌfi˜ ‚ÚÂÊÒÓ Ì ˘„ËÏ‹ ALP (>300 IU/L) ÌÂÙ¿ ·fi ÙÔÓ ÚÒÙÔ Ì‹Ó· Ù˘ ˙ˆ‹˜ Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜. OÈ Gessner Î·È Û˘Ó. (20) ·Ó·Ê¤ÚÔ˘Ó ·˘ÍË̤Ó˜ ÙÈ̤˜ ALP Û ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÓÂÔÁÓÒÓ (30%) Ì ¯·ÌËÏ¿ Â›‰· 25OHD (<15 ng/ml) Û ۇÁÎÚÈÛË Ì ÂÎÂ›ÓˆÓ (6%) Ô˘ ›¯·Ó ÈÔ „ËÏ¿ Â›‰· 25OHD (>25 ng/ml). Y„ËÏfiÙÂÚ˜ ÙÈ̤˜ ALP ÔÚÔ‡ ·Ó·Ê¤ÚÔÓÙ·È Î·È Û ‚Ú¤ÊË ËÏÈΛ·˜ ¤ÍÈ Â‚‰ÔÌ¿‰ˆÓ Ô˘ ›¯·Ó 25OHD <10 ng/ml ¤Ó·ÓÙÈ ‚ÚÂÊÒÓ Ì ˘„ËÏfiÙÂÚ· Â›‰· 25OHD (32). ªÈ· Ù¿ÛË ÁÈ· ˘„ËÏfiÙÂÚ˜ ÙÈ̤˜ ALP ÔÚÔ‡ Î·È ¯·ÌËÏfiÙÂÚ· Â›‰· ÔÛÙÂÔηÏÛ›Ó˘ ÛÙ· ‚Ú¤ÊË Ì 25OHD <10 ng/ml Î·È ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ, ·Ú·ÙËÚ‹ıËÎÂ Î·È ÛÙË ‰È΋ Ì·˜ ÌÂϤÙË, ¯ˆÚ›˜ fï˜ ÔÈ ‰È·ÊÔÚ¤˜ Ó· Â›Ó·È ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜. ¢È·ÊÔÚ¤˜ ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙÔ ‚¿ÚÔ˜ ‹ ÛÙÔ Ì‹ÎÔ˜ ÛÒÌ·ÙÔ˜ ÙˆÓ ‚ÚÂÊÒÓ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ÌÂٷ͇ ÙˆÓ ‰‡Ô ÂÔ¯ÒÓ ÁÈ· ÙÔ˘˜ 6 ÚÒÙÔ˘˜ Ì‹Ó˜ ˙ˆ‹˜, Y¿Ú¯Ô˘Ó ˆÛÙfiÛÔ ÌÂÚÈΤ˜ ÌÂϤÙ˜ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ‰È·ÊÔÚ¤˜ ÛÙ·

ۈ̷ÙÔÌÂÙÚÈο Î·È ¿ÏϘ Ô˘ Û˘ÌʈÓÔ‡Ó Ì ٷ ‰Èο Ì·˜ ·ÔÙÂϤÛÌ·Ù·. OÈ Brook Î·È Û˘Ó.

(33) ·Ó·Ê¤ÚÔ˘Ó ÌÂÁ·Ï‡ÙÂÚÔ ‚¿ÚÔ˜ Î·È Ì‹ÎÔ˜ ÛÒÌ·ÙÔ˜ ÛÙÔ ÚÒÙÔ ¤ÙÔ˜ ˙ˆ‹˜ Û ӋÈ· ÌËÙ¤ÚˆÓ Ô˘ ¤·ÈÚÓ·Ó ‚ÈÙ·Ì›ÓË D ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÙÂÏÂ˘Ù·›Ô˘ ÙÚÈÌ‹ÓÔ˘ ·ËÛ˘. AÓÙ›ıÂÙ· ÔÈ Greer Î·È Marshal (21) ‰ÂÓ ‚Ú‹Î·Ó ‰È·ÊÔÚ¤˜ ÛÙÔ ‚¿ÚÔ˜ Î·È ÙÔ Ì‹ÎÔ˜ ÛÒÌ·ÙÔ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ ¤ÍÈ ÌËÓÒÓ Ù˘ ˙ˆ‹˜, ÌÂٷ͇ ÙˆÓ ‚ÚÂÊÒÓ Ô˘ ¤·ÈÚÓ·Ó Î·È ·˘ÙÒÓ Ô˘ ‰ÂÓ ¤·ÈÚÓ·Ó ‚ÈÙ·Ì›ÓË D Î·È ÛÈÙ›˙ÔÓÙ·Ó ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi ıËÏ·ÛÌfi. ™Â ·˘Ù‹ ÙË ÌÂϤÙË fï˜, fiϘ ÔÈ ÌËÙ¤Ú˜ ›¯·Ó ¿ÚÂÈ ‚ÈÙ·Ì›ÓË D ηٿ ÙËÓ Î‡ËÛË.

T· Û˘ÌÂÚ¿ÛÌ·Ù· ·˘Ù‹˜ Ù˘ ÌÂϤÙ˘ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ÓÂÔÁÓÒÓ Ô˘ ÁÂÓÓÈÔ‡ÓÙ·È Ì ¯·ÌËÏ¿ Â›‰· ‚ÈÙ·Ì›Ó˘ D ÛÙËÓ EÏÏ¿‰· Â›Ó·È ÌÂÁ¿ÏË, ·ÎfiÌË Î·È Î·Ù¿ ÙÔ˘˜ ıÂÚÈÓÔ‡˜ Ì‹Ó˜ Î·È Ì¿ÏÏÔÓ Ù· ‚Ú¤ÊË Ô˘ ÛÈÙ›˙ÔÓÙ·È ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi ıËÏ·ÛÌfi ¯Ú‹˙Ô˘Ó ¯ÔÚ‹ÁËÛ˘ ‚ÈÙ·Ì›Ó˘ D ̤¯ÚÈ ÙÔÓ 6Ô Ì‹Ó· Ù˘ ˙ˆ‹˜. M›· ÂÓ·ÏÏ·ÎÙÈ΋ χÛË ı· ‹Ù·Ó Ë ¯ÔÚ‹ÁËÛË ‚ÈÙ·Ì›Ó˘ D ÛÙȘ ÌËÙ¤Ú˜ ηٿ ÙÔ ÙÂÏÂ˘Ù·›Ô ÙÚ›ÌËÓÔ Ù˘ ·ËÛ˘.

E˘¯·ÚÈÛٛ˜ E˘¯·ÚÈÛÙԇ̠ÙËÓ Î· AÊÚÔ‰›ÙË ¶··ÁÈ¿ÓÓË ÁÈ· ÙËÓ Ù¯ÓÈ΋ ˘ÔÛÙ‹ÚÈÍË Î·È ÙËÓ EÙ·ÈÚ›· Hoffman La Roche ÁÈ· ÙËÓ Â˘ÁÂÓ‹ ¯ÔÚ‹ÁËÛË Ù˘ ÌË ÙÚÈÙȈ̤Ó˘ 25OHD ÁÈ· ÙËÓ ·Ú·Û΢‹ ÙˆÓ ÚÔÙ‡ˆÓ ‰È·Ï˘Ì¿ÙˆÓ ÁÈ· ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ 25OHD ÛÙÔ ·›Ì·.

BÈ‚ÏÈÔÁÚ·Ê›· 1. DeLuca HF. The metabolism, physiology and function of vitamin D. In: Kumar R, editor. Vitamin D. Basic and Clinical Aspects. Boston: Martinus Nijhoff; 1984. p. 1-68.

2. Rigby WF. The immunobiology of vitamin D. Immunol Today 1988;9:54-58.

3. Esvelt RP, Schnoes HK, DeLuca HF. Vitamin D3 from rat skins irradiated in vitro with ultraviolet light. Arch Biochem Biophys 1978;188:282-286.

4. Ponchon G, DeLuca HF. The role of the liver in the metabolism of vitamin D. J Clin Invest 1969;48: 1273-1279.

5. Hollis BW. Assessment of vitamin D nutritional and hormonal status: what to measure and how to do it.

Calcif Tissue Int 1996;58:4-5.

6. Reeve L, Tanaka Y, DeLuca HF. Studies on the site of 1,25-dihydroxyvitamin D3 synthesis in vivo. J Biol Chem 1983;258:3615-3617.

7. Holick MF. Vitamin D: the underappreciated Dlightful hormone that is important for skeletal and ¶·È‰È·ÙÚÈ΋ 2006;69:205-212

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Õ. X¿ÏÏ· Î·È Û˘Ó.

8.

9.

10.

11.

12.

13.

14.

15.

16.

17.

18.

19.

20.

21.

¶·È‰È·ÙÚÈ΋ 2006;69:205-212

cellular health. Curr Opin Endocrinol Diabetes 2002;9:87-98.

Dewey KG. Nutrition, growth and complementary feeding of the breastfed infant. Pediatr Clin North Am 2001;48:87-104.

Greer FR. Do breastfed infants need supplemental vitamins? Pediatr Clin North Am 2001;48:415-423.

Institute of Medicine, Food and Nutrition Board, Standing Committee on the Scientific Evaluation of Dietary Reference Intakes. Vitamin D. In: Dietary reference intakes for calcium, phosphorus, magnesium, vitamin D and fluoride. Washington, DC: National Academy Press; 1997. p. 250-287.

Salle BL, Delvin EE, Lapillonne A, Bishop NJ, Glorieux FH. Perinatal metabolism of vitamin D. Am J Clin Nutr 2000;71 (5 Suppl):S1317-S1324.

Holick MF. Vitamin D: importance in the prevention of cancers, type 1 diabetes, heart disease and osteoporosis. Am J Clin Nutr 2004;79:362-371.

Baroncelli GI, Bertelloni S, Ceccarelli C, Amato V, Saggese G. Bone turnover in children with vitamin D deficiency rickets before and during treatment.

Acta Paediatr 2000;89:513-518.

Mallet E, Gugi B, Brunelle P, Henocq A, Basuyau JP, Lemeur H. Vitamin D supplementation in pregnancy: a controlled trial of two methods. Obstet Gynecol 1986;68:300-304.

Zeghoud F, Vervel C, Guillozo H, Walrant-Debray O, Boutignon H, Garabedian M. Subclinical vitamin D deficiency in neonates: definition and response to vitamin D supplements. Am J Clin Nutr 1997;65:771-778.

Gertner JM, Glassman MS, Coustan DR, Goodman DB. Fetomaternal vitamin D relationships at term. J Pediatr 1980;97:637-640.

Hoogenboezem T, Degenhart HJ, de Muinck KeizerSchrama SM, Bouillon R, Grose WF, Hackeng WH, et al. Vitamin D metabolism in breast-fed infants and their mothers. Pediatr Res 1989;25:623-628.

Heaney RP, Dowell MS, Hale CA, Bendich A. Calcium absorption varies within the reference range for serum 25-hydroxyvitamin D. J Am Coll Nutr 2003;22:142-146.

Challa A, Bevington A, Angier CM, Asbury AJ, Preston CJ, Russell RG. A technique for the measurement of orthophosphate in human erythrocytes, and some studies of its determinants. Clin Sci (Lond) 1985;69:429-434.

Gessner BD, Plotnik J, Muth PT. 25-hydroxyvitamin D levels among healthy children in Alaska. J Pediatr 2003;143:434-437.

Greer FR, Marshall S. Bone mineral content, serum vitamin D metabolite concentrations and ultraviolet

22.

23.

24.

25.

26.

27.

28.

29.

30.

31.

32.

33.

B light exposure in infants fed human milk with and without vitamin D2 supplements. J Pediatr 1989;114: 204-212.

Ala-Houhala M, Koskinen T, Terho A, Koivula T, Visakorpi J. Maternal compared with infant vitamin D supplementation. Arch Dis Child 1986;61: 1159-1163.

Hollis BW, Wagner CL. Vitamin D requirements during lactation: high-dose maternal supplementation as therapy to prevent hypovitaminosis D for both the mother and the nursing infant. Am J Clin Nutr 2004;80(6 Suppl):S1752-S1758.

Rothberg AD, Pettifor JM, Cohen DF, Sonnendecker EW, Ross FP. Maternal-infant vitamin D relationships during breast-feeding. J Pediatr 1982;101: 500-503.

Canadian Paediatric Society, Health Canada, Dietitians of Canada. Nutrition for healthy term infants: statement of the joint working group. [Internet] Webpage: http://www.caringforkids.cps.ca/babies/VitaminD.htm.

Muhe L, Lulseged S, Mason KE, Simoes EA. Casecontrol study of the role of nutritional rickets in the risk of developing pneumonia in Ethiopian children. Lancet 1997;349:1801-1804.

Wilkinson RJ, Llewelyn M, Toossi Z, Patel P, Pasvol G, Lalvani A, et al. Influence of vitamin D deficiency and vitamin D receptor polymorphisms on tuberculosis among Gujarati Asians in west London: a case-control study. Lancet 2000;355:618-621.

Nikiforuk G, Fraser D. The etiology of enamel hypoplasia: a unifying concept. J Pediatr 1981;98:888893.

Cantorna MT. Vitamin D and autoimmunity: is vitamin D status an environmental factor affecting autoimmune disease prevalence? Proc Soc Exp Biol Med 2000;223:230-233.

Brooke OG, Brown IR, Bone CD, Carter ND, Cleeve HJ, Maxwell JD, et al. Vitamin D supplements in pregnant Asian women: effects on calcium status and fetal growth. Br Med J 1980;280:751-754.

Delvin EE, Salle BL, Glorieux FH, Adeleine P, David LS. Vitamin D supplementation during pregnancy: effect on neonatal calcium homeostasis. J Pediatr 1986;109:328-334.

Dawodu A, Agarwal M, Hossain M, Kochiyil J, Zayed R. Hypovitaminosis D and vitamin D deficiency in exclusively breast-feeding infants and their mothers in summer: a justification for vitamin D supplementation of breast-feeding infants. J Pediatr 2003;142:169-173.

Brooke OG, Butters F, Wood C. Intrauterine vitamin D nutrition and postnatal growth in Asian infants. Br Med J (Clin Res Ed) 1981;283:1024.

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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

CASE REPORT

213

¡fiÛÔ˜ Niemann-Pick Ù‡Ô˘ µ Ì Û¿ÓÈÔ ÁÔÓfiÙ˘Ô - ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ¡. ª·ÓˆÏ¿ÎË1, †∞. ª‹ÙÛÈη1, ¢. ™ÌÒÎÔ˘1, ∫. ¶Ú›ÊÙ˘1, ∞. ºÏÒÎÔ˘2, ∂. ªÈ¯ÂϷοÎË2

¶ÂÚ›ÏË„Ë: ∏ ÓfiÛÔ˜ Niemann-Pick Â›Ó·È Û¿ÓÈ·, ÎÏËÚÔÓÔÌÔ‡ÌÂÓË Ì ·˘ÙÔÛˆÌÈÎfi ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·, ÓfiÛÔ˜ ·Ôı‹Î¢Û˘ ÏÈȉ›ˆÓ. √ Ù‡Ô˜ µ ÔÊ›ÏÂÙ·È Û ·ÓÂ¿ÚÎÂÈ· Ù˘ Ï˘ÛÔÛˆÌȷ΋˜ fiÍÈÓ˘ ÛÊÈÁÁÔÌ˘ÂÏÈÓ¿Û˘, Ô˘ Ô‰ËÁ› Û ÂÓ·fiıÂÛË ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘, ΢ڛˆ˜ ÛÙ· ·ÙÙ·Ú· ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ÌÔÓÔ΢ÙÙ¿ÚˆÓ-Ì·ÎÚÔÊ¿ÁˆÓ Ô˘ ·ÔÎÙÔ‡Ó ÙË ¯·Ú·ÎÙËÚÈÛÙÈ΋ ÌÔÚÊÔÏÔÁ›· “·ÊÚˆ‰ÒÓ” ΢ÙÙ¿ÚˆÓ. ™Â Û‡ÁÎÚÈÛË Ì ¿ÏÏÔ˘˜ Ù‡Ô˘˜ Ù˘ ÓfiÛÔ˘, Ë ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Â›Ó·È Û¿ÓÈ·. ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ·ÁÔÚÈÔ‡, ËÏÈΛ·˜ 2 ÂÙÒÓ, Ì ÓfiÛÔ Niemann-Pick Ù‡Ô˘ µ Î·È ‰È¿¯˘ÙË ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ó‡ÌÔÓ·, Ô˘ ·ÔÙ¤ÏÂÛÂ Ù˘¯·›Ô ·ÎÙÈÓÔÏÔÁÈÎfi ‡ÚËÌ·. √ ·ÛıÂÓ‹˜ ›¯Â Â›Û˘, ÎÂÚ·Ûfi¯ÚÔË ÎËÏ›‰· ÛÙÔÓ ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹ Ì ·Ú¯fiÌÂÓË ˘Ú·ÌȉÈ΋ ÚÔÛ‚ÔÏ‹. ∞ÊÚÒ‰Ë Î‡ÙÙ·Ú· ‚Ú¤ıËÎ·Ó ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Î·È ÛÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì·. ∞Ó¿Ï˘ÛË DNA ·ÔÎ¿Ï˘„ ÔÌÔ˙˘ÁˆÙ›· ˆ˜ ÚÔ˜ ÙË ÌÂÙ¿ÏÏ·ÍË W391G. ™ÙËÓ ÙÚ¤¯Ô˘Û· ‚È‚ÏÈÔÁÚ·Ê›·, ‰ÂÓ ˘¿Ú¯ÂÈ ¿ÏÏË ·Ó·ÊÔÚ¿ ÙÔ˘ ÁÔÓÔÙ‡Ô˘ ·˘ÙÔ‡, Ô ÔÔ›Ô˜ ‰ÂÓ ·ÔÎÏ›ÂÙ·È Ó· Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ÎÏÈÓÈ΋ ÚÔ‚ÔÏ‹ Ù˘ ÓfiÛÔ˘ ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È.

1 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ Î·È ∞ÏÏÂÚÁÈÔÏÔÁÈÎfi ∆Ì‹Ì· ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ ¶ÂÓÙ¤Ï˘ 2 ¢È‡ı˘ÓÛË ∂Ó˙˘ÌÔÏÔÁ›·˜ Î·È ∫˘ÙÙ·ÚÈ΋˜ §ÂÈÙÔ˘ÚÁ›·˜, πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ AÏÏËÏÔÁÚ·Ê›·: ¡›Ó· ª·ÓˆÏ¿ÎË µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ ¶ÂÓÙ¤Ï˘ ¶·Ï·È¿ ¶ÂÓÙ¤ÏË, ∆.∫. 152 36 E-mail: ninamanolaki@in.gr ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 26-01-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 30-09-2005

§¤ÍÂȘ ÎÏÂȉȿ: ¡fiÛÔ˜ Niemann-Pick µ, ·È‰È¿, Ó¢ÌÔÓÈ΋ ÓfiÛÔ˜, ÎÂÚ·Ûfi¯ÚÔ˘˜ ÎËÏ›‰·, ÁÔÓfiÙ˘Ô˜.

Niemann-Pick disease type B with a rare genetic profile A case report N. Manolaki1, †A. Mitsika1, D. Sbokou1, K. Priftis1, A. Flokou2, E. Michelakaki2

Abstract: Niemann-Pick disease is a rare, autosomal recessive, lipid storage disease. Type B is characterized by low lysosomal acid sphingomyelinase activity, resulting in the accumulation of sphingomyelin, mainly in the cells of the monocyte-macrophage system, which have the characteristics of “foam” cells. Neurologic manifestations of the disease are relatively rare. The case of a 2-year old boy with Niemann-Pick disease type B and diffuse pulmonary infiltrations is described. The patient also had a “cherry red” retinal spot and early signs of neurologic involvement. Foam cells were present in the bone marrow and in the bronchoalveolar lavage. DNA analysis revealed homozygosity for the W391G mutation, a genotype not previously reported. An association between this genotype and the clinical presentation cannot be excluded.

Key words: Niemann-Pick type B, children, pulmonary involvement, ocular manifestations, genotype.

∂ÈÛ·ÁˆÁ‹ ∏ ÓfiÛÔ˜ Niemann-Pick (OMIM: 607616) ·ÔÙÂÏ› ÂÙÂÚÔÁÂÓ¤˜ Û‡Ó‰ÚÔÌÔ Û‡ÌÊ˘Ù˘ ‰È·Ù·Ú·¯‹˜ ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙˆÓ ÏÈȉ›ˆÓ ÛÙ· Ï·›ÛÈ· ÙÔ˘ ÔÔ›Ô˘ ‰È·ÎÚ›ÓÔÓÙ·È, ·Ó¿ÏÔÁ· Ì ÙË Ê·ÈÓÔÙ˘È΋ ÚÔ‚ÔÏ‹ Î·È ÙË ÌÔÚȷ΋ ‚¿ÛË, 6 Ù‡ÔÈ (∞-F). ªÂ ÙËÓ ÂÍ·›ÚÂÛË ÙˆÓ Ù‡ˆÓ C Î·È D, Ô˘ ÔÊ›ÏÔÓÙ·È Û ‰È·Ù·Ú·¯‹ ÙÔ˘ ÂÓ‰Ô΢ÙÙ¿ÚÈÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ Ù˘ ¯ÔÏËÛÙÂÚfiÏ˘, ÔÈ ˘fiÏÔÈÔÈ ÔÊ›ÏÔÓÙ·È Û ·ÓÂ¿ÚÎÂÈ· ÙÔ˘ Ï˘ÛÔÛˆÌÈ·ÎÔ‡ ÂÓ˙‡ÌÔ˘ fiÍÈÓË ÛÊÈÁÁÔÌ˘ÂÏÈÓ¿ÛË

1 2nd Paediatric Clinic and Allergology Department, Penteli Children’s Hospital 2 Division of Enzymology and Cell Function, Institute of Child’s Health

Correspondence: Nina Manolaki 2nd Paediatric Clinic, Penteli Children’s Hospital Palaia Penteli, 152 36 E-mail: ninamanolaki@in.gr Date of submission: 26-01-2005 Date of approval: 30-09-2005

(ASM; EC 3.2.4.12) Î·È ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ÙËÓ ÂÓ·fiıÂÛË ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ (Î·È ¿ÏÏˆÓ ÏÈȉ›ˆÓ) ÛÙ· Ì·ÎÚÔÊ¿Á· (1). ™Â ·ÓÙ›ıÂÛË Ì ÙÔÓ Ù‡Ô ∞, Ô˘ ¤¯ÂÈ Ù˘È΋ ÎÏÈÓÈ΋ ÚÔ‚ÔÏ‹ ·fi ÙË ‚ÚÂÊÈ΋ ËÏÈΛ· (Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·, ÚÔÛ‚ÔÏ‹ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ÎÂÚ·Ûfi¯ÚÔË ÎËÏ›‰· ÛÙÔÓ ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹) Î·È Ù·¯¤ˆ˜ ÂȉÂÈÓÔ‡ÌÂÓË ÔÚ›· Ì ı·Ó·ÙËÊfiÚÔ Î·Ù¿ÏËÍË Ù· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜, Ô Ù‡Ô˜ µ ¤¯ÂÈ ÌÂÁ·Ï‡ÙÂÚË ÂÙÂÚÔÁ¤ÓÂÈ· Ì Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·, ˘ÂÚÏÈȉ·ÈÌ›·, ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ó‡ÌÔÓ· Î·È ÌÂÁ¿ÏÔ Â‡ÚÔ˜ ˆ˜ ÚÔ˜ ÙËÓ ËÏÈΛ· ÂΉ‹ÏˆÛ˘ ¶·È‰È·ÙÚÈ΋ 2006;69:213-217

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¡. ª·ÓˆÏ¿ÎË Î·È Û˘Ó.

Î·È ÙËÓ ÂÈ‚›ˆÛË. ∆˘Èο, Ô Ù‡Ô˜ µ ‰ÂÓ Û˘Óԉ‡ÂÙ·È ·fi ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ·Ó Î·È ¤¯Ô˘Ó ·Ó·ÊÂÚı› ·ıÔÏÔÁÈο Ó¢ÚÔÏÔÁÈο Â˘Ú‹Ì·Ù· Û ¿Û¯ÔÓÙ˜ ÂÓËÏ›ÎÔ˘˜, ÂÓÒ Ë ·ÚÔ˘Û›· ÎÂÚ·Ûfi¯ÚÔ˘ ÎËÏ›‰·˜ Â›Ó·È Û¿ÓÈ· (1,2). ∆Ô ˘‡ı˘ÓÔ ÁÈ· ÙËÓ fiÍÈÓË ÛÊÈÁÁÔÌ˘ÂÏÈÓ¿ÛË ÁÔÓ›‰ÈÔ ÂÓÙÔ›˙ÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 11 (11p15.1-11p15.4) Î·È Ì¤¯ÚÈ Û‹ÌÂÚ·, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ¿Óˆ ·fi 50 ÌÂÙ·ÏÏ¿ÍÂȘ Û ·ÛıÂÓ›˜ Ì هÔ ∞ Î·È µ (3). ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ·ÛıÂÓÔ‡˜, ËÏÈΛ·˜ 2 ¯ÚfiÓˆÓ, Ì ÓfiÛÔ Niemann-Pick Ù‡Ô˘ µ Ì Û¿ÓÈÔ ÁÔÓfiÙ˘Ô, ÎÂÚ·Ûfi¯ÚÔË ÎËÏ›‰· Î·È ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ó‡ÌÔÓ· Î·È ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜.

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ∞ÁfiÚÈ, ËÏÈΛ·˜ 2 ¯ÚfiÓˆÓ, ·Ú·¤ÌÊıËΠÁÈ· ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛË ‰È¿¯˘ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ‰ÈËı‹ÛˆÓ, Ô˘ ‰È·ÈÛÙÒıËÎ·Ó Û ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη ÚÈÓ ·fi ¤Ó· Ì‹Ó· ÂÚ›Ô˘. ∏ ·ÎÙÈÓÔÁÚ·Ê›· ¤ÁÈÓ ηٿ ÙË ÓÔÛËÏ›· ÙÔ˘ ·È‰ÈÔ‡ ÏfiÁˆ ÂÌ˘Ú¤ÙÔ˘ Ì ԢÏÔÛÙÔÌ·Ù›Ùȉ·, ÌÈÎÚ‹ ‰ÈfiÁΈÛË ÙÚ·¯ËÏÈÎÒÓ ÏÂÌÊ·‰¤ÓˆÓ Î·È Ë·ÙÔÛÏËÓÈ΋ ‰ÈfiÁΈÛË, Ô˘ ·Ô‰fiıËΠ۠ÏÔÈÌÒ‰Ë ÌÔÓÔ˘Ú‹ÓˆÛË. √ ·ÛıÂÓ‹˜ Â›Ó·È ÙÔ ÚÒÙÔ ·È‰› Ê·ÈÓÔÙ˘Èο ˘ÁÈÒÓ, ÌË Û˘ÁÁÂÓÒÓ ÌÂٷ͇ ÙÔ˘˜, ÁÔÓ¤ˆÓ. °ÂÓÓ‹ıËΠÌÂÙ¿ ·fi ÙÂÏÂÈfiÌËÓË Î‡ËÛË ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ÙÔÎÂÙfi Î·È ‚¿ÚÔ˜ 3300 g. ∏ ÂÚÈÁÂÓÓËÙÈ΋ ÂÚ›Ô‰Ô˜ ‹Ù·Ó ÔÌ·Ï‹ Î·È ÙÔ ·ÙÔÌÈÎfi Î·È ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ‹Ù·Ó ÂχıÂÚ·. ∏ ÌËÙ¤Ú· ‹Ù·Ó ¤ÁÎ˘Ô˜ ÛÙÔ ‰Â‡ÙÂÚÔ ·È‰› Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜, ÛÙÔÓ 2Ô Ì‹Ó· Ù˘ ·ËÛ˘. ∫·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹, Ô ·ÛıÂÓ‹˜ ‹Ù·Ó Û ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ۈ̷ÙÈ΋ ·Ó¿Ù˘ÍË Î·È „˘¯ÔÎÈÓËÙÈ΋ ÂͤÏÈÍË. ∞fi ÙËÓ Î·Ù¿ Û˘ÛÙ‹Ì·Ù· ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË, Ù· ÌfiÓ· ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ‹Ù·Ó „ËÏ·ÊËÙfi ‹·Ú (ÂÚ› Ù· 5 cm) Î·È ÛÏ‹Ó·˜ (ÂÚ› Ù· 2 cm), ·ÌÊfiÙÂÚ· ̷Ϸ΋˜ Û‡ÛÙ·Û˘. √ ·Ú¯ÈÎfi˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ (ÁÂÓÈ΋ ·›Ì·ÙÔ˜, ∆∫∂, CRP, Û˘Ó‹ı˘ ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ Î·È ‚ÈÔ¯ËÌÈΤ˜ ·Ú¿ÌÂÙÚÔÈ Ù˘ Ë·ÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜) ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. √ ÔÚÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· EBV, CMV, HBV, HIV ‹Ù·Ó ·ÚÓËÙÈÎfi˜, fiˆ˜ Î·È Ë ‰ÂÚÌÔ·ÓÙ›‰Ú·ÛË Mantoux. ∏ ·ÎÙÈÓÔÏÔÁÈ΋ ÂÈÎfiÓ· ÙÔ˘ ıÒڷη ·Ú¤ÌÂÓÂ Ë ›‰È· Ì ÙËÓ ·Ú¯È΋ Ì ‰È¿¯˘Ù˜ ÌÈÎÚÔÔ¶·È‰È·ÙÚÈ΋ 2006;69:213-217

˙Ò‰ÂȘ Ó¢ÌÔÓÈΤ˜ ‰ÈËı‹ÛÂȘ ·ÌÊÔÙÂÚfiÏ¢ڷ (∂ÈÎfiÓ· 1). ™Â ˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒڷη ˘„ËÏ‹˜ ¢ÎÚ›ÓÂÈ·˜ ‰È·ÈÛÙÒıËΠ‰È¿¯˘ÙË ıÔÏÂÚfiÙËÙ· ÙÔ˘ Ó¢ÌÔÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜ ·ÌÊÔÙÂÚfiÏ¢ڷ (ÂÈÎfiÓ· ıÔÏ‹˜ ˘¿ÏÔ˘ ground glass) Î·È Â›Ù·ÛË ÙÔ˘ ‰È¿ÌÂÛÔ˘ ÈÛÙÔ‡ Û fiÏ· Ù· Ó¢ÌÔÓÈο ‰›· (∂ÈÎfiÓ· 2). ∞ÎÔÏÔ‡ıËÛ ‚ÚÔÁ¯ÔÛÎfiËÛË ÛÙËÓ ÔÔ›· ‰È·ÈÛÙÒıËΠ¤ÓÙÔÓ· ÔȉËÌ·Ù҉˘ ‚ÏÂÓÓÔÁfiÓÔ˜ ÙÔ˘ ÙÚ·¯ÂÈÔ‚ÚÔÁ¯ÈÎÔ‡ ‰¤Ó‰ÚÔ˘ Ì ÏÂ˘Îˆ¿ ÂÎÎڛ̷ٷ. ∏ ηÏÏȤÚÁÂÈ· ÙÔ˘ ‚ÚÔÁ¯ÈÎÔ‡ ÂÎχ̷ÙÔ˜ ‹Ù·Ó ·ÚÓËÙÈ΋ ÁÈ· ·ıÔÁfiÓ·. ∏ ΢ÙÙ·ÚÔÏÔÁÈ΋ ÂͤٷÛË ¤‰ÂÈÍ ¿ÊıÔÓ· Ì·ÎÚÔÊ¿Á· (98% ÙˆÓ Î˘ÙÙ¿ÚˆÓ) Ì ÌÂÚÈÎÒ˜ ¤ÎÎÂÓÙÚÔ ˘Ú‹Ó· Î·È ÎÂÓÔÙÔÈ҉˜ ΢ÙÙ·ÚfiÏ·ÛÌ·, ¯·Ú·ÎÙËÚÈÛÙÈο “·ÊÚˆ‰ÒÓ” ΢ÙÙ¿ÚˆÓ. ™ÙÔ Ì˘ÂÏfiÁÚ·ÌÌ· ˘‹Ú¯Â Â›Û˘, ‰È‹ıËÛË Ì ÈÛÙÈÔ·ÙÙ·Ú·, ÌÂÚÈο ·fi Ù· ÔÔ›· ›¯·Ó ÎÂÓÔÙÔÈ҉˜ ΢ÙÙ·ÚfiÏ·ÛÌ· (“·ÊÚ҉˔ ·ÙÙ·Ú·). ∏ ÌÂϤÙË Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ ÙˆÓ Ï˘ÛÔÛˆÌÈ·ÎÒÓ ÂÓ˙‡ÌˆÓ Û ÂÚÈÊÂÚÈο ÏÂÌÊÔ·ÙÙ·Ú· ¤‰ÂÈÍ Ôχ ¯·ÌËÏ‹ ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ fiÍÈÓ˘ ÛÊÈÁÁÔÌ˘ÂÏÈÓ¿Û˘ ı¤ÙÔÓÙ·˜ ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ Niemann-Pick. ™˘ÁÎÂÎÚÈ̤ӷ, ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ >1,2 nmoles/mg ÚˆÙ½Ó˘/hr, Ô ·ÛıÂÓ‹˜ ›¯Â 0,15 Î·È ·fi ÙÔ˘˜ ÁÔÓ›˜, 0,9 Ô ·Ù¤Ú·˜ Î·È 1,0 Ë ÌËÙ¤Ú·. ∞fi ÙËÓ ·Ó¿Ï˘ÛË DNA, Ô˘ ¤ÁÈÓ ÛÙÔ Department of Human Genetics, Mount Sinai School of Medicine, New York, USA (Dr. Schuchman), ‰È·ÈÛÙÒıËΠÔÌÔ˙˘ÁˆÙ›· ˆ˜ ÚÔ˜ ÙË ÌÂÙ¿ÏÏ·ÍË W391G, ‡ÚËÌ· Ô˘ ÂȂ‚·›ˆÛ ÙË ‰È¿ÁÓˆÛË ÓfiÛÔ˘. ªÂ ‰Â‰Ô̤ÓË ÙË ‰È¿ÁÓˆÛË, ¤ÁÈÓ ÛÙÔ ·È‰› ÏÂÙÔÌÂÚ‹˜ Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË ÛÙËÓ ÔÔ›· ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∂ÓÙÔ‡ÙÔȘ, Ë ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË ·ÔÎ¿Ï˘„ ÙËÓ ·ÚÔ˘Û›· ÎÂÚ·Ûfi¯ÚÔ˘ ÎËÏ›‰·˜ ¿Ìʈ.

∂ÈÎfiÓ· 1. ¢È¿Û·ÚÙ˜, ‰ÈÎÙ˘ÔÔ˙Ò‰ÂȘ ‰ÈËı‹ÛÂȘ ¿Ìʈ.

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·215

215

¡fiÛÔ˜ Niemann-Pick Ù‡Ô˘ µ

∂ÈÎfiÓ· 2. ∂ÈÎfiÓ· “ground-glass” Î·È Â›Ù·ÛË ÙÔ˘ ‰È¿ÌÂÛÔ˘ ÈÛÙÔ‡.

∂Ù¿ Ì‹Ó˜ ·ÚÁfiÙÂÚ·, Ù· ·ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· ·Ú·Ì¤ÓÔ˘Ó Ù· ›‰È· Î·È Ô ·ÛıÂÓ‹˜ ÂÍ·ÎÔÏÔ˘ı› Ó· Â›Ó·È ·Û˘Ìو̷ÙÈÎfi˜, ·ÏÏ¿ ¤¯ÂÈ ÂÌÊ·Ó›ÛÂÈ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎfi Babinski ¿Ìʈ, ÛËÌÂ›Ô ÂÓ‰ÂÈÎÙÈÎfi ˘Ú·ÌȉÈ΋˜ ÚÔÛ‚ÔÏ‹˜ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ∏ ÌËÙ¤Ú· ˘Ô‚Ï‹ıËΠ۠ÚÔÁÂÓÓËÙÈÎfi ¤ÏÂÁ¯Ô Ì ÂÓ˙˘ÌÈÎfi ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ ÛÊÈÁÁÔÌ˘ÂÏÈÓ¿Û˘ Û ÙÚÔÊÔ‚Ï¿ÛÙË, ÛÙÔÓ ÔÔ›Ô ‰È·ÈÛÙÒıËÎ·Ó Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ÙÔ˘ ÂÓ˙‡ÌÔ˘ ÎÈ ¤ÙÛÈ, ·ÔÎÏ›ÛıËÎÂ Ë ÓfiÛÔ˜ ÛÙÔ Î‡ËÌ·.

™˘˙‹ÙËÛË √ Ù‡Ô˜ µ Ù˘ ÓfiÛÔ˘ Niemann-Pick ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÂÙÂÚÔÁ¤ÓÂÈ· ˆ˜ ÚÔ˜ ÙËÓ ÎÏÈÓÈ΋ ÚÔ‚ÔÏ‹, ÙËÓ ËÏÈΛ· ‰È¿ÁÓˆÛ˘, ÙË ‚·Ú‡ÙËÙ· Î·È ÙËÓ ÂÈ‚›ˆÛË Î·È, ÁÂÓÈο, ¤¯ÂÈ Î·Ï‡ÙÂÚË ÚfiÁÓˆÛË Û ۇÁÎÚÈÛË Ì ¿ÏÏÔ˘˜ Ù‡Ô˘˜ Ù˘ ÓfiÛÔ˘. ™ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ, Ë ‰È¿ÁÓˆÛË Ù›ıÂÙ·È ÛÙË ‚ÚÂÊÈ΋ ‹ ·È‰È΋ ËÏÈΛ· Ì ·ÊÔÚÌ‹ ÙËÓ Ù˘¯·›· ·Ó‡ÚÂÛË ‰ÈfiÁΈÛ˘ ÙÔ˘ ‹·ÙÔ˜, ÙÔ˘ ÛÏËÓfi˜ ‹ Î·È ÙˆÓ ‰‡Ô. ªÂ ÙËÓ ¿ÚÔ‰Ô Ù˘ ËÏÈΛ·˜, Ë Ë·ÙÔÛÏËÓÈ΋ ‰ÈfiÁΈÛË ÌÔÚ› Ó· Á›ÓÂÈ ÏÈÁfiÙÂÚÔ ÂÎÛÂÛËÌ·Ṳ̂ÓË, ÂÓÒ Û ÂÚÈÙÒÛÂȘ ÈÔ ‹È·˜ ÓfiÛÔ˘, Ë ‰ÈfiÁΈÛË ÙÔ˘ ÛÏËÓfi˜ ÌÔÚ› Ó· Â›Ó·È ÙfiÛÔ ÌÈÎÚ‹, ÒÛÙ ӷ Á›ÓÂÈ ·ÓÙÈÏËÙ‹ ÛÙËÓ ÂÓ‹ÏÈÎÔ ˙ˆ‹. ™Â ‚·ÚȤ˜ ÌÔÚʤ˜, ÌÔÚ›, Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘, Ó· ·Ó·Ù˘¯ı› ˘ÂÚÛÏËÓÈÛÌfi˜ Ì ·Ó΢ÙÙ·ÚÔÂÓ›·, ΛÚÚˆÛË ÙÔ˘ ‹·ÙÔ˜, ˘Ï·›· ˘¤ÚÙ·ÛË, Ë·ÙÈ΋ ·ÓÂ¿ÚÎÂÈ· (4,5). ªÔÚ› Â›Û˘, Ó· ˘¿Ú¯ÂÈ Î·ı˘ÛÙ¤ÚËÛË ÛÙË ÛˆÌ·ÙÈ΋ ·Ó¿Ù˘ÍË Î·È ÙËÓ ˆÚ›Ì·ÓÛË ÙÔ˘ ÛÎÂ-

ÏÂÙÔ‡ (6). ∂ȉÈ΋ ıÂÚ·›· ‰ÂÓ ˘¿Ú¯ÂÈ. ™Â ÌÂÌÔӈ̤Ó˜ ÂÚÈÙÒÛÂȘ ¤¯ÂÈ ‰ÔÎÈÌ·Ûı› Ë ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ, Û ÂͤÏÈÍË ‚Ú›ÛÎÂÙ·È Ë ·Ó¿Ù˘ÍË ıÂÚ·›·˜ ÂÓ˙˘ÌÈ΋˜ ˘ÔηٿÛÙ·Û˘, ÂÓÒ Á›ÓÔÓÙ·È ÚÔÛ¿ıÂȘ ÛÙÔÓ ÙÔ̤· Ù˘ ÁÔÓȉȷ΋˜ ıÂÚ·›·˜. ¶ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ó¢ÌÔÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜ ÌÔÚ› Ó· ˘¿Ú¯ÂÈ Û fiÏÔ˘˜ ÙÔ˘˜ Ù‡Ô˘˜ Ù˘ ÓfiÛÔ˘ Niemann-Pick, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤ÓÔ˘ Î·È ÙÔ˘ Ù‡Ô˘ µ ÛÙÔÓ ÔÔ›Ô Ì¿ÏÈÛÙ·, ıˆÚÂ›Ù·È Û˘¯Ó‹. ∫·Ù¿ ηÓfiÓ·, Â›Ó·È Û¿ÓÈ· ÛÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜ Î·È Û˘ÓËı¤ÛÙÂÚË Û ‚Ú¤ÊË Î·È ·È‰È¿. ∏ ·ÎÚÈ‚‹˜ Â›ÙˆÛË Ù˘ ÚÔÛ‚ÔÏ‹˜ ÙÔ˘ Ó‡ÌÔÓ· Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ηıÔÚÈÛı›, ηıfiÛÔÓ ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÔÓÙ·È Î˘Ú›ˆ˜ ÌÂÌÔӈ̤Ó˜ ÂÚÈÙÒÛÂȘ (4). ª¤¯ÚÈ Û‹ÌÂÚ·, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› 42 ÂÚÈÙÒÛÂȘ ÓfiÛÔ˘ Niemann-Pick Ì Ó¢ÌÔÓÈ΋ Û˘ÌÌÂÙÔ¯‹, ·fi ÙȘ Ôԛ˜ Ë ‰È¿ÁÓˆÛË ÙÔ˘ Ù‡Ô˘ µ ‹Ù·Ó ÙÂÎÌËÚȈ̤ÓË Û 17 Î·È Èı·Ó‹ Û 6. ∞fi ÙÔ˘˜ 23 ·˘ÙÔ‡˜ ·ÛıÂÓ›˜, ÔÈ 13 ›¯·Ó ËÏÈΛ· ÌÈÎÚfiÙÂÚË ÙˆÓ 18 ¯ÚfiÓˆÓ (4,5). ∏ ·ÎÙÈÓÔÏÔÁÈ΋ ÂÈÎfiÓ· ÙÔ˘ Ó‡ÌÔÓ· Û ÓfiÛÔ Niemann-Pick Û˘Ó›ÛÙ·Ù·È ÛÙËÓ ·ÚÔ˘Û›· ‰È¿¯˘ÙˆÓ ‰ÈÎÙ˘Ô˙ˆ‰ÒÓ ‰ÈËı‹ÛˆÓ, Ô˘ ÌÔÚ› Ó· ÚÔÛ‰›‰Ô˘Ó ÂÈÎfiÓ· ÌÂÏÈÎËÚ‹ıÚ·˜ (7,8). √È ‰ÈËı‹ÛÂȘ ÌÔÚ› ·Ú¯Èο, Ó· ·ÊÔÚÔ‡Ó ÌfiÓÔ ÛÙȘ ‚¿ÛÂȘ Î·È ·ÚÁfiÙÂÚ· Ó· ÂÂÎÙ·ıÔ‡Ó Û ÔÏfiÎÏËÚÔ ÙÔ Ó¢ÌÔÓÈÎfi ‰›Ô (4). ∏ ˘„ËÏ‹˜ ¢ÎÚ›ÓÂÈ·˜ ˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›· (HRCT) ıÒڷη ·ÔηχÙÂÈ ıÔÏÂÚfiÙËÙ˜ Ô˘ ÌÔÈ¿˙Ô˘Ó Ì “ıÔÏ‹ ‡·ÏÔ” (ground-glass) ÛÙȘ ·ÓÒÙÂÚ˜ Ó¢ÌÔÓÈΤ˜ ˙ÒÓ˜ Î·È ·¯‡ÓÛÂȘ ÙˆÓ ÌÂÛÔÏfi‚ÈˆÓ ‰È·ÊÚ·ÁÌ¿ÙˆÓ ÛÙȘ ηÙÒÙÂÚ˜ (7,9). OÈ ·ÎÙÈÓÔÏÔÁÈΤ˜ ·ÏÏÔÈÒÛÂȘ ÔÊ›ÏÔÓÙ·È ÛÙË Û˘ÛÛÒÚ¢ÛË “·ÊÚˆ‰ÒÓ” ΢ÙÙ¿ÚˆÓ ÛÙȘ ΢„ÂÏ›‰Â˜ Î·È Ù· ÌÂÛÔÏfi‚È· ‰È·ÊÚ¿ÁÌ·Ù·. ™ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ë ÎÂÁ¯ÚÔÂȉ‹˜ Ê˘Ì·Ù›ˆÛË, Ë Ó¢ÌÔÓÈ΋ ·ÈÌÔÛȉ‹ÚˆÛË, Ë Î˘„ÂÏȉÈ΋ ÚˆÙÂ˝ÓˆÛË, ηıÒ˜ Î·È ¿ÏϘ ÛÊÈÁÁÔÏÈȉÒÛÂȘ fiˆ˜ Ë ÓfiÛÔ˜ Gaucher Î·È ÔÈ Á·ÁÁÏÈÔÛȉÒÛÂȘ GM1 Î·È GM2 (10). √È ·ÏÏÔÈÒÛÂȘ ÙÔ˘ Ó¢ÌÔÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜ ÌÔÚ› Ó· Â›Ó·È ÂÌÊ·Ó›˜ ·fi Ôχ ÓˆÚ›˜ Î·È ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› Û ‚Ú¤ÊÔ˜ ËÏÈΛ·˜ 3 ÌËÓÒÓ. ¶ÔÏϤ˜ ÊÔÚ¤˜, fï˜, ·ÔÙÂÏÔ‡Ó Ù˘¯·›Ô ‡ÚËÌ·, ›Ûˆ˜ Î·È ¯ÚfiÓÈ· ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘, ÂÂȉ‹, ÙÔ˘Ï¿¯ÈÛÙÔÓ ÛÙËÓ ·È‰È΋ ËÏÈΛ·, ÌÔÚ› Ó· ÌËÓ ÚÔηÏÔ‡Ó Û˘ÌÙÒÌ·Ù· (4,10). ∂ÓÙÔ‡ÙÔȘ, Ë ‰È‹ıËÛË ÙÔ˘ Ó¢ÌÔÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜ ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ ÛÔ‚·Ú¿ ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· Ì ¯·ÌËϤ˜ ÙÈ̤˜ ƒ√2, ‰‡ÛÓÔÈ· Û ÎfiˆÛË, Ó¢ÌÔÓÈ΋ ηډ›· ¶·È‰È·ÙÚÈ΋ 2006;69:213-217

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·216

216

¡. ª·ÓˆÏ¿ÎË Î·È Û˘Ó.

Î·È ·ÂÈÏËÙÈο ÁÈ· ÙË ˙ˆ‹ ÂÂÈÛfi‰È· ‚ÚÔÁ¯ÔÓ¢ÌÔÓ›·˜ Û ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ˘ (11).

O ·ÛıÂÓ‹˜ Ì·˜, Û ËÏÈΛ· 2 ¯ÚfiÓˆÓ, ›¯Â Ù· Ù˘Èο Â˘Ú‹Ì·Ù· ÛÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· Î·È ÙËÓ HRCT ıÒڷη Ì ٷ ¯·Ú·ÎÙËÚÈÛÙÈο Ì·ÎÚÔÊ¿Á· -“·ÊÚ҉˔ ·ÙÙ·Ú·- ÛÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì·, ¯ˆÚ›˜ Ó· ·ÚÔ˘ÛÈ¿˙ÂÈ Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi. ™Â ·ÓÙ›ıÂÛË, fï˜, Ì ¿ÏϘ ÂÚÈÙÒÛÂȘ Ë ÂÈÎfiÓ· Ù˘ “ıÔÏ‹˜ ˘¿ÏÔ˘” ‰ÂÓ ÂÚÈÔÚÈ˙fiÙ·Ó ÛÙȘ ·ÓÒÙÂÚ˜ Ó¢ÌÔÓÈΤ˜ ˙ÒÓ˜, ·ÏÏ¿ ÂÂÎÙÂÈÓfiÙ·Ó Û fiÏÔ ÙÔÓ Ó‡ÌÔÓ·. ∏ ·ÚÔ˘Û›· ÙˆÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ “·ÊÚˆ‰ÒÓ” ΢ÙÙ¿ÚˆÓ ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Î·È ¿ÏÏÔ˘˜ ÈÛÙÔ‡˜ (‹·Ú, Ó‡ÌÔÓ·˜) ˘ÔÛÙËÚ›˙ÂÈ ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ Niemann-Pick, Ë ÔÔ›· ÂȂ‚·ÈÒÓÂÙ·È Ì ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ fiÍÈÓ˘ ÛÊÈÁÁÔÌ˘ÂÏÈÓ¿Û˘ Û ÂÚÈÊÂÚÈο Ï¢ÎÔ·ÙÙ·Ú· Î·È Î·ÏÏȤÚÁÂÈ· ÈÓÔ‚Ï·ÛÙÒÓ ‹ ÏÂÌÊÔ‚Ï·ÛÙÒÓ. ™ÙÔÓ Ù‡Ô ∞ Î·È µ Ë ‰Ú·ÛÙÈÎfiÙËÙ· Â›Ó·È Ôχ ÌÂȈ̤ÓË (1-10% Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜). ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Â›Ó·È ÂÊÈÎÙ‹ Ì ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ fiÍÈÓ˘ ÛÊÈÁÁÔÌ˘ÂÏÈÓ¿Û˘ Û ¯ÔÚȷ΋ Ï¿¯ÓË ‹ Û ηÏÏȤÚÁÂÈ· ·ÌÓÈÔ΢ÙÙ¿ÚˆÓ (12, 13) ηÈ, ›Ûˆ˜, Ì ÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË ÁÈ· ÙËÓ ·ÚÔ˘Û›· ÌÂÙ·ÏϿ͈Ó. ∆Ô ÁÔÓ›‰ÈÔ Ù˘ fiÍÈÓ˘ ÛÊÈÁÁÔÌ˘ÂÏÈÓ¿Û˘ ‰ڿ˙ÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 11p15.1-11p15.4 Î·È ·ÔÙÂÏÂ›Ù·È ·fi ¤ÍÈ ÂÍfiÓÈ· Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó 629 ·ÌÈÓÔͤ·. ∂›Ó·È ‹‰Ë ÁÓˆÛÙ¤˜ ÂÚÈÛÛfiÙÂÚ˜ ·fi 50 ÌÂÙ·ÏÏ¿ÍÂȘ Û ·ÛıÂÓ›˜ Ì ÙÔ˘˜ Ù‡Ô˘˜ ∞ Î·È µ Ù˘ ÓfiÛÔ˘. √ Ù‡Ô˜ ∞ Â›Ó·È Û˘¯ÓfiÙÂÚÔ˜ ÛÙÔ˘˜ Ashkenazi ∂‚Ú·›Ô˘˜, fiÔ˘ ÙÔ 92% ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ·ÔÙÂÏÔ‡Ó ÔÈ ÌÂÙ·ÏÏ¿ÍÂȘ R496L, L302P Î·È fsP330. ∞ÓÙ›ıÂÙ·, Û ·ÛıÂÓ›˜ ÌË Â‚Ú·˚΋˜ ηٷÁˆÁ‹˜ Ì ÙÔÓ ›‰ÈÔ Ù‡Ô Ù˘ ÓfiÛÔ˘, ¤¯Ô˘Ó ·ÓȯÓ¢ı› ÔÏϤ˜ ‰È·ÊÔÚÂÙÈΤ˜ “ȉȈÙÈΤ˜” ÌÂÙ·ÏÏ¿ÍÂȘ. ™ÙÔÓ Ù‡Ô µ Ë ÈÔ Û˘¯Ó‹ ÌÂÙ¿ÏÏ·ÍË Â›Ó·È Ë ¢R608, Ô˘ ¤¯ÂÈ ·ÓȯÓ¢ı› Û ÔÌÔ˙˘ÁˆÙ›· (12/15) Î·È ÂÙÂÚÔ˙˘ÁˆÙ›· (2/15) Û ·ÛıÂÓ›˜ ·fi ÙËÓ ∆˘ÓËÛ›·, ÙËÓ ∞ÏÁÂÚ›· Î·È ÙÔ ª·ÚfiÎÔ. ∂›Û˘, ÌË Ashkenazi ∂‚Ú·›ÔÈ Î·È °¿ÏÏÔÈ ·ÛıÂÓ›˜ ¤¯Ô˘Ó ‚ÚÂı› Ó· Â›Ó·È ÂÙÂÚÔ˙˘ÁÒÙ˜ ÁÈ· ÙË ÌÂÙ¿ÏÏ·ÍË ·˘Ù‹, ÂÓÒ Û ÂÙÂÚÔ˙˘ÁˆÙ›· ¤¯ÂÈ ‚ÚÂı› Î·È Ë ÌÂÙ¿ÏÏ·ÍË R496L (3). ∏ ÌÂÙ¿ÏÏ·ÍË ¢R608 Ê·›ÓÂÙ·È fiÙÈ ¤¯ÂÈ “Ó¢ÚÔÚÔÛٷ٢ÙÈÎfi” ÚfiÏÔ Î·È “·ÔÙÚ¤ÂÈ” ÙËÓ ÂÌÊ¿ÓÈÛË Ó¢ÚÔÏÔÁÈÎÒÓ Û˘Ìو̿وÓ, ÂÓÒ Ë ÔÌÔ˙˘ÁˆÙ›· ˆ˜ ÚÔ˜ ·˘Ù‹ Ê·›ÓÂÙ·È, ÁÂÓÈο, Ó· Û¯ÂÙ›˙ÂÙ·È Ì ÈÔ ‹È· ÓfiÛÔ Î·È Ê˘ÛÈÔÏÔÁÈ΋ ·Ó¿Ù˘ÍË (6,14,15). ªÂϤÙ˜ ¤ÎÊÚ·Û˘ ¤‰ÂÈÍ·Ó fiÙÈ ÌÂÙ·ÏÏ¿ÍÂȘ fiˆ˜ ¶·È‰È·ÙÚÈ΋ 2006;69:213-217

ÔÈ R496L, L302P, fsP330, M38111I Î·È ¿ÏϘ, Ô‰ËÁÔ‡Ó ÛÙË Û‡ÓıÂÛË Î·Ù·Ï˘ÙÈο ·‰Ú·ÓÔ‡˜ ÚˆÙ½Ó˘, ÂÚÌËÓ‡ÔÓÙ·˜ ¤ÙÛÈ, ÙË ‚·ÚÈ¿ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ Ù‡Ô˘ ∞ Ô˘ ¤¯Ô˘Ó ÔÈ ÔÌÔ˙˘ÁÒÙ˜ ·ÛıÂÓ›˜. ∞ÓÙ›ıÂÙ·, ÌÂÙ·ÏÏ¿ÍÂȘ Ô˘ ¤¯Ô˘Ó ‚ÚÂı› ›Ù Û ÔÌÔ˙˘ÁˆÙ›· ›Ù Û ÂÙÂÚÔ˙˘ÁˆÙ›· ÛÙÔÓ Ù‡Ô µ Û˘Ó‰¤ÔÓÙ·È Ì ÙË Û‡ÓıÂÛË ÚˆÙ½Ó˘ Ô˘ ¤¯ÂÈ ÛËÌ·ÓÙÈ΋ ‰Ú·ÛÙÈÎfiÙËÙ· (16). ∂ÓÙÔ‡ÙÔȘ, ÔÈ Û˘ÁÎÂÎÚÈ̤ÓÔÈ Û˘Ó‰˘·ÛÌÔ› ÌÂÙ·ÏÏ¿ÍÂˆÓ ‰ÂÓ Û˘ÓÂ¿ÁÔÓÙ·È Ù·˘ÙfiÛËÌË ÎÏÈÓÈ΋ ÔÚ›·, fiˆ˜ Ê·›ÓÂÙ·È ·ÎfiÌË Î·È Û ÂÚ›ÙˆÛË ‰È‰‡ÌˆÓ (3,14). ™ÙÔÓ ·ÛıÂÓ‹ Ì·˜, Ë ·Ó¿Ï˘ÛË ÙÔ˘ DNA ·ÔÎ¿Ï˘„ ÔÌÔ˙˘ÁˆÙ›· ˆ˜ ÚÔ˜ ÙË ÌÂÙ¿ÏÏ·ÍË W391G. √ ÁÔÓfiÙ˘Ô˜ ·˘Ùfi˜ Ú¤ÂÈ Ó· Â›Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈÔ˜. ™ÙË ‰È·ı¤ÛÈÌË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È ¤Ó·˜ ·ÛıÂÓ‹˜ Ì ÙËÓ ›‰È· ÌÂÙ¿ÏÏ·ÍË, ·ÏÏ¿ ÂÙÂÚÔ˙˘ÁÒÙ˘ (W391G/R496C). ™˘ÁÎÂÎÚÈ̤ӷ, ÚfiÎÂÈÙ·È ÁÈ· ÎÔÚ›ÙÛÈ 12 ÂÙÒÓ, ÙÔ ÔÔ›Ô Â›¯Â, Â›Û˘, ÎÂÚ·Ûfi¯ÚÔË ÎËÏ›‰· (17). ∫ÂÚ·Ûfi¯ÚÔ˘˜ ÎËÏ›‰· ·Ú·ÙËÚÂ›Ù·È Û¿ÓÈ· ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÙÔÓ Ù‡Ô µ Ù˘ ÓfiÛÔ˘ Î·È ÔÊ›ÏÂÙ·È ÛÙËÓ ÂÓ·fiıÂÛË ÏÈȉ›ˆÓ ÛÙÔ˘˜ Ó¢ÚÒÓ˜ Ô˘ ÂÚÈ‚¿ÏÏÔ˘Ó ÙÔ ‚ÔıÚ›Ô Ù˘ ˆ¯Ú‹˜ ÎËÏ›‰·˜ (18). ∏ ·ÚÔ˘Û›· Ù˘ ıˆÚÂ›Ù·È ¤Ó‰ÂÈÍË ÂÌÏÔ΋˜ ÙÔ˘ Ó¢ÚÈÎÔ‡ ÈÛÙÔ‡ ÛÙË ÓfiÛÔ, ¯ˆÚ›˜, fï˜, ·˘Ùfi Ó· Â›Ó·È ·fiÏ˘ÙÔ. ™Â Û¯ÂÙÈ΋ ·Ó·ÛÎfiËÛË, ·fi 45 ·ÛıÂÓ›˜, ËÏÈΛ·˜ 3-65 ¯ÚfiÓˆÓ, Ì هÔ µ, ÎÂÚ·Ûfi¯ÚÔË ÎËÏ›‰· ‹ ¿Ïˆ ›¯·Ó ÔÈ 15 (33%) ¯ˆÚ›˜, fï˜, Ó· Û˘Ó˘¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ Ó¢ÚÔÏÔÁÈ΋˜ ÂÎʇÏÈÛ˘ (17). ™ÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜, ·Ú¿ ÙËÓ ‡·ÚÍË ÎÂÚ·Ûfi¯ÚÔ˘ ÎËÏ›‰·˜, Ë Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó ·Ú¯Èο ·ÚÓËÙÈ΋ ÁÈ· ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∂ÓÙÔ‡ÙÔȘ, Ë ÌÂÙ¿ ·fi 6 Ì‹Ó˜ ÂÌÊ¿ÓÈÛË ÛËÌ›Ԣ Babinski ·ÌÊÔÙÂÚfiÏ¢ڷ, ·ÔÙÂÏ› ¤Ó‰ÂÈÍË Èı·Ó‹˜ Û˘ÌÌÂÙÔ¯‹˜ ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ÁÂÁÔÓfi˜ Ô˘ οÓÂÈ ÙËÓ ·ÒÙÂÚË ÚfiÁÓˆÛË ÈÔ ÂÈÊ˘Ï·ÎÙÈ΋. ¶Èı·ÓfiÓ, Ë ·ÚÔ˘Û›· Ù˘ ÎÂÚ·Ûfi¯ÚÔ˘ ÎËÏ›‰·˜ Ó· ·ÓÙÈÚÔÛˆ‡ÂÈ Î·È ÛÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜ ÙËÓ ÎÏ·ÛÈ΋ Û˘Û¯¤ÙÈÛ‹ Ù˘ Ì ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ∞Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÎÂÚ·Ûfi¯ÚÔ˘˜ ÎËÏ›‰· ‹ ¿Ïˆ˜ Ù˘ ˆ¯Ú‹˜ ÎËÏ›‰·˜ ·Ú·ÙËÚÂ›Ù·È Û˘ÓËı¤ÛÙÂÚ· Û ·ÛıÂÓ›˜ Ô˘ ‰ÂÓ Ê¤ÚÔ˘Ó ÙË ÌÂÙ¿ÏÏ·ÍË ¢R608 Î·È Û˘ÁÎÂÎÚÈ̤ӷ, Û ÔÛÔÛÙfi 48% ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ Û ۇÁÎÚÈÛË Ì 20% ÙˆÓ ÂÙÂÚÔ˙˘ÁˆÙÒÓ ÁÈ· ÙË ÌÂÙ¿ÏÏ·ÍË ¢R608 (17). ∏ ÎÏÈÓÈ΋ ÚÔ‚ÔÏ‹ Ù˘ ÓfiÛÔ˘ Niemann-Pick ÛÙÔÓ ·ÛıÂÓ‹ Ì·˜ Â›Ó·È Û˘Ì‚·Ù‹ Ì ÙÔÓ Ù‡Ô µ Ù˘ ÓfiÛÔ˘, Û˘Ó‰˘¿˙ÂÙ·È, fï˜, Ì ÂΉËÏÒÛÂȘ Ô˘ ‰ÂÓ Â›Ó·È Û˘¯Ó¤˜ ÛÙÔÓ Ù‡Ô ·˘Ùfi, fiˆ˜ Ë

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·ÚÔ˘Û›· ÎÂÚ·Ûfi¯ÚÔ˘ ÎËÏ›‰·˜ Î·È Ë ·Ú¯fiÌÂÓË ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ∂›Û˘, Ô ·ÛıÂÓ‹˜ ¤¯ÂÈ Û¿ÓÈÔ ÁÔÓfiÙ˘Ô Ì ÔÌÔ˙˘ÁˆÙ›· ˆ˜ ÚÔ˜ ÙË ÌÂÙ¿ÏÏ·ÍË W391G. ∞Ó Î·È Ù· ‰È·ı¤ÛÈÌ· ÛÙÔȯ›· ‰ÂÓ ÌÔÚÔ‡Ó Ó· ÙÂÎÌËÚÈÒÛÔ˘Ó Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙÔ˘ ÁÔÓÔÙ‡Ô˘ Î·È Ù˘ ÎÏÈÓÈ΋˜ ÚÔ‚ÔÏ‹˜, ‰ÂÓ ·ÔÎÏ›ÂÙ·È Ô Û˘ÁÎÂÎÚÈ̤ÓÔ˜ ÁÔÓfiÙ˘Ô˜ Ó· ÂÈ‚·Ú‡ÓÂÈ ÙËÓ ÎÏÈÓÈ΋ ÚÔ‚ÔÏ‹ ÙÔ˘ Ù‡Ô˘ µ Ù˘ ÓfiÛÔ˘.

10.

11.

12.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Elleder M. Niemann-Pick disease. Path Res Pract 1989;185:293-328.

2. Crocker AC, Farber S. Niemann-Pick disease: A review of eighteen patients. Medicine (Baltimore) 1958;37:1-95.

3. Kolodny EH. Niemann-Pick disease. Curr Opin in Hematol 2000;7:48-52.

4. Minai OA, Sullivan EJ, Stoller JK. Pulmonary involvement in Niemann-Pick disease: Case report and literature review. Respir Med 2000;94:1241-1251.

5. Gonzalez-Reimers E, Sanchez-Perez JM, Bonilla-Arjona A, Rodriguez-Gaspar M, Garrazo-Juan JL, Alvarez-Arguelles H, et al Pulmonary involvement in an adult male affected by type B Niemann-Pick disease. Br J Radiol 2003;76:838-840.

6. Wasserstein MP, Larkin AE, Glass RB, Schuchman EH, Desnick RJ, McGovern MM. Growth restriction in children with type B Niemann-Pick disease. J Pediatr 2003;142: 424-428.

7. Duchateau F, Dechambre S, Coche E. Imaging of pulmonary manifestations in subtype B of Niemann-Pick disease. Br J Radiol 2001;74:1059-1061.

8. Ferretti GR, Lantuejoul S, Brambilla E, Coulomb M. Pulmonary involvement in Niemann-Pick disease subtype B: CT findings. J. Comput Assist Tomogr 1996;20:990-992.

9. Lachman R, Crocker A, Schulman J, Strand R. Radi-

13.

14.

15.

16.

17.

18.

ological findings in Niemann-Pick disease. Radiology 1973;108:659-664.

Niggemann B, Rebien W, Rahn W, Wahn U. Asymptomatic pulmonary involvement in 2 children with Niemann-Pick disease type B. Respiration 1994;61:55-57.

Nicholson AG, Wells AU, Hooper J, Hansell DM, Kelleher A, Morgan C. Successful treatment of endogenous lipoid pneumonia due to Niemann-Pick type B disease with whole-lung lavage. Am J Respir Crit Care Med 2002;165:128-131.

Maziere JC, Maziere C, Hosli P. An ultramicrochemical assay for sphingomyelinase: rapid prenatal diagnosis of a fetus at risk for Niemann-Pick disease.

Monogr Hum Genet 1978;9:198-201.

Vanier MT, Boue J, Dumez Y. Niemann-Pick disease type B: first-trimester prenatal diagnosis on chorionic villi and biochemical study of a foetus at 12 weeks of development. Clin Genet 1985;28:348-354.

Schuchman EH, Miranda SR. Niemann-Pick disease. Mutation update, genotype / phenotype correlations and prospects for genetic testing. Genet Test 1997;13-19.

Levran O, Desnick RJ, Schuchman EH. NiemannPick type B disease. Identification of a single codon deletion in the acid sphingomyelinase gene and genotype/phenotype correlations in type A and B patients. J Clin Invest 1991;88:806-810.

Schuchman EH, Desnick RJ. Niemann-Pick disease types A and B: Acid sphingomyelinase deficiencies.

In: Scriver CR, Beaudet Al, Sly KW, Volgestein B, editors. The Metabolic and Molecular Bases of Inherited Disease.. McGraw-Hill Medical Publishing Division, 8th ed. 2001. pp 3589-3611.

Mc Govern MM, Wasserstrein MP, Aron A, Desnick RJ, Schuchman EH, Brodie SE. Ocular manifestations of Niemann-Pick disease type B. Ophthalmology 2004;111:1424-1427.

Lipson MH, O’Donnell J, Callahan JW, Wenger DA, Packman S. Ocular involvement in Niemann-Pick disease type B. J. Pediatr 1986;108:582-584.

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∂¡¢π∞º∂ƒOÀ™∞ ¶∂ƒπ¶∆ø™∏

CASE REPORT

ÀÔÍ›· ÛÎÏËÚ˘ÓÙÈ΋ ·ÓÂÁÎÂÊ·Ï›Ùȉ· ÌÂÙ¿ ·fi ÂÓ‰ÔÌ‹ÙÚÈ· Ïԛ̈ÍË ·fi ÙÔÓ Èfi Ù˘ ÈÏ·Ú¿˜ 1 ¡ÂÔÁÓÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô “∞ÏÂÍ¿Ó‰Ú·”, ∞ı‹Ó· 2 ¡Â˘ÚÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: ª·Ú›· ¢·ÛÔÔ‡ÏÔ˘ ¶··‰È·Ì·ÓÙÔÔ‡ÏÔ˘ 11 ∆.∫. 115 28, πÏ›ÛÈ· ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 11-01-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 08-07-2005

ª. ¢·ÛÔÔ‡ÏÔ˘1, ∞. ∫Ô‚¿Ó˘2

¶ÂÚ›ÏË„Ë: ∏ ˘ÔÍ›· ÛÎÏËÚ˘ÓÙÈ΋ ·ÓÂÁÎÂÊ·Ï›Ùȉ· (À™¶∂) ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÔÊ›ÏÂÙ·È Û ÚÔÛ‚ÔÏ‹ ·fi ÙÔ ¿ÁÚÈÔ Û٤ϯԘ ÙÔ˘ ÈÔ‡ Ù˘ ÈÏ·Ú¿˜, ·Ó Î·È ˘¿Ú¯Ô˘Ó ÔÚÈṲ̂Ó˜ ·Ó·ÊÔÚ¤˜ Ô˘ Û˘Û¯ÂÙ›˙Ô˘Ó ÙËÓ À™¶∂ Ì ÙÔÓ ÂÌ‚ÔÏÈ·ÛÌfi ¤Ó·ÓÙÈ ÙÔ˘ Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ÈÔ‡. ¶·ÚÔ˘ÛÈ¿˙ÂÙ·È Ë ÂÚ›ÙˆÛË ÂÓfi˜ ÂÌ‚Ú‡Ô˘, ÙÔ ÔÔ›Ô ÌÔχÓıËΠÛÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÓ‰ÔÌ‹ÙÚÈ·˜ ˙ˆ‹˜ ·fi ÙÔÓ Èfi Ù˘ ÈÏ·Ú¿˜ ηÈ, ÛÙË Û˘Ó¤¯ÂÈ·, ·Ó¤Ù˘Í À™¶∂ ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ ˙ˆ‹˜, ÌÂÙ¿ ·fi Û‡ÓÙÔÌË ÂÚ›Ô‰Ô ÂÒ·Û˘. ∏ ÓfiÛÔ˜ ÂÍÂÏ›¯ÙËΠٷ¯‡Ù·Ù·, ÌÂÙ¿ ·fi ‹È· ‡ÊÂÛË Ì ÙË ıÂÚ·›·. ∏ À™¶∂ Â›Ó·È Ì›· ÌÔÈÚ·›· ÂÎÊ˘ÏÈÛÙÈ΋ ÓfiÛÔ˜ ÙÔ˘ ∫¡™ Î·È ·Ó Î·È ¤¯ÂÈ ÂÚÈÔÚÈÛÙ› ·ÁÎÔÛÌ›ˆ˜ Û ÌÂÁ¿ÏÔ ‚·ıÌfi ÏfiÁˆ Ù˘ ÂÊ·ÚÌÔÁ‹˜ ÙÔ˘ ÂÌ‚ÔÏÈ·ÛÌÔ‡ Û fiÏÔÓ Û¯Â‰fiÓ ÙÔÓ ÎfiÛÌÔ, ˆÛÙfiÛÔ ·Ú·Ì¤ÓÂÈ Ì›· ÛÔ‚·Ú‹ Ïԛ̈ÍË ÛÙ· ·È‰È¿ Ô˘ ¤¯Ô˘Ó ÚÔÛ‚ÏËı› ·fi ÙÔÓ ·ÓıÚÒÂÈÔ Èfi Ù˘ ·ÓÔÛÔ·ÓÂ¿ÚÎÂÈ·˜ (HIV). ∏ ·Ô˘Û›· ÂÌÊ¿ÓÈÛ˘ ÓÂfiÙÂÚˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ À™¶∂ ·Ó¿ÌÂÛ· Û ˘ÁÈ‹ ·È‰È¿ ÛÙË ÛËÌÂÚÈÓ‹ ÂÔ¯‹ ‰ÂÓ ı· Ú¤ÂÈ Ó· ÌÂÈÒÛÂÈ ÙËÓ Â·ÁÚ‡ÓËÛË ÙˆÓ Ì·ÈÂ˘Ù‹ÚˆÓ Î·È ÙˆÓ ·È‰È¿ÙÚˆÓ, ÒÛÙ ӷ ·Ó·ÁÓˆÚ›˙Ô˘Ó ÙÔÓ Î›Ó‰˘ÓÔ Ù˘ ÚÔÛ‚ÔÏ‹˜ ·fi ÈÏ·Ú¿ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘. ∞Ó Î·È ˘¿Ú¯Ô˘Ó ÌÂÚÈΤ˜ ·Ó·ÊÔÚ¤˜ ÂÚÈÛÙ·ÙÈÎÒÓ À™¶∂ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÂÌ‚ÔÏÈ·ÛÌfi, ÓÂfiÙÂÚ˜ ÂÚÁ·Û›Â˜ ·ÌÊÈÛ‚ËÙÔ‡Ó ÙȘ Èı·Ó¤˜ Û¯¤ÛÂȘ ÌÂٷ͇ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ηٿ Ù˘ ÈÏ·Ú¿˜ Î·È Ù˘ À™¶∂. ∏ ·ÚÔ‡Û· ÌÂϤÙË ÂȉÈÒÎÂÈ Ó· ÙÔÓ›ÛÂÈ ÙË ÛËÌ·Û›· Î·È ÙËÓ ÂÈÙ˘¯›· Ù˘ Ì›ˆÛ˘ Ù˘ À™¶∂ Ì ÙË ‚Ô‹ıÂÈ· ÁÂÓÈÎÂ˘Ì¤ÓÔ˘ ÂÌ‚ÔÏÈ·ÛÌÔ‡ ηٿ Ù˘ ÈÏ·Ú¿˜ Ì ˘„ËÏ‹ Î¿Ï˘„Ë. §¤ÍÂȘ ÎÏÂȉȿ: ¡ÂÔÁÓfi, ÂÓ‰ÔÌ‹ÙÚÈ· Ïԛ̈ÍË, ÈÏ·Ú¿, ÂÚÈÁÂÓÓËÙÈ΋ Ïԛ̈ÍË, ˘ÔÍ›· ÛÎÏËÚ˘ÓÙÈ΋ ·ÓÂÁÎÂÊ·Ï›Ùȉ·.

Subacute sclerosing panencephalitis after intrauterine infection with measles virus 1 Department of Neonatology, “Alexandra” General Hospital, Athens 2 Department of Neurology, “Aghia Sophia” Children’s Hospital, Athens Correspondence: Maria Dasopoulou 11 Papadiamantopoulou Str. 115 28, Ilissia, Athens Date of submission: 11-01-2005 Date of approval: 08-07-2005

M. Dasopoulou1, A. Kovanis2

Abstract: Subacute sclerosing panencephalitis (SSPE), in the majority of cases, is caused by the wild measles virus, although some reports relate SSPE to measles vaccination. The case is presented of a male infant who was infected prenatally by the measles virus and developed SSPE within the first year of life, after a short incubation period. The disease progressed rapidly after a mild interruption following treatment. SSPE is a fatal degenerative disease which, although it had almost disappeared following near-universal measles vaccination, remains a serious infection among children affected by human immunodeficiency virus (HIV). The absence of new cases of SSPE among normal children nowadays should not lead to a decrease in alertness by obstetricians and paediatricians, who should recognize the risk of measles during pregnancy and the need for prevention and diagnosis of SSPE at an early stage.

Although earlier references link SSPE cases to measles vaccination, recent research casts doubt on such an association. This case report emphasises the importance of general measles vaccination with high coverage in reducing the problem of SSPE. Key words: Infant, intrauterine infection, measles, perinatally acquired infection, subacute sclerosing panencephalitis.

∂ÈÛ·ÁˆÁ‹ ∏ ˘ÔÍ›· ÛÎÏËÚ˘ÓÙÈ΋ ·ÓÂÁÎÂÊ·Ï›Ùȉ· Â›Ó·È Ì›· ¯ÚfiÓÈ· Ïԛ̈ÍË ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ ¶·È‰È·ÙÚÈ΋ 2006;69:218-222

Û˘ÛÙ‹Ì·ÙÔ˜ ·fi ÙÔÓ Èfi Ù˘ ÈÏ·Ú¿˜. ∏ ÓfiÛÔ˜ Û˘Ó‹ıˆ˜ ÚÔÛ‚¿ÏÏÂÈ ·È‰È¿ ËÏÈΛ·˜ 8-10 ÂÙÒÓ. ∏ ÎÏÈÓÈ΋ ÂÌÊ¿ÓÈÛË Ù˘ ÓfiÛÔ˘ ·ÔÙÂÏ› ·Ú¯Èο Ì›· ¿Ù˘Ë ‹È· ‰È·Ù·Ú·¯‹ Û˘ÌÂÚÈÊÔÚ¿˜, Ô˘

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ÀÔÍ›· ÛÎÏËÚ˘ÓÙÈ΋ ·ÓÂÁÎÂÊ·Ï›Ùȉ· ÌÂÙ¿ ·fi ÂÓ‰ÔÌ‹ÙÚÈ· Ïԛ̈ÍË

219

∂ÈÎfiÓ· 2. ∂ÈÎfiÓ· 1.

ÂÍÂÏ›ÛÛÂÙ·È ÚÔԉ¢ÙÈο - ̤۷ ·fi ‰È·ÊÔÚÂÙÈο ÛÙ¿‰È· ηٿ Jabour (1) - ÛÙÔ ÙÂÏÈÎfi ÛÙ¿‰ÈÔ Î·È ÛÙÔÓ ı¿Ó·ÙÔ. ∏ ·ıÔÁ¤ÓÂÈ· Ù˘ ÓfiÛÔ˘ Â›Ó·È Ì¤¯ÚÈ ÛÙÈÁÌ‹˜ ¿ÁÓˆÛÙË Î·È ·ÓÙÈÎÚÔ˘fiÌÂÓ˜ ·fi„ÂȘ ¤¯Ô˘Ó ‰È·Ù˘ˆı› ˆ˜ ÚÔ˜ ÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ·. ∏ ·ıÔÏÔÁÈ΋ ·Ú·ÁˆÁ‹ Ù˘ ÚˆÙ½Ó˘ ÙÔ˘ ÛÙÚÒÌ·ÙÔ˜ (ª) ‹ Ù˘ ÚˆÙ½Ó˘ ‰È¿¯˘Û˘ (F) ÙÔ˘ ÈÔ‡ ·fi Ù· ÚÔۂ‚ÏË̤ӷ ·fi ÙÔÓ Èfi ·ÙÙ·Ú·, ηıÒ˜ Î·È Ì›· ·Ú¿‰ÔÍË ·ÓÙÈÁÔÓÈ΋ ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ÈÔ‡ ·fi ·ÓÙÈÛÒÌ·Ù· ÙÔ˘ ÍÂÓÈÛÙ‹, Ì›· ‰È·Ù·Ú·¯‹ ÛÙË Û‡ÓıÂÛË Î˘ÙÔÎÈÓÒÓ ‹ Ì›· ÌÂÙ·‚ÔÏ‹ ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ·fiÙˆÛ˘ Î·È ·ÚÓËÙÈ΋˜ ÛËÌ·ÙÔ‰fiÙËÛ˘ ÙˆÓ ∆ ΢ÙÙ¿ÚˆÓ ·fi ÁÏ˘ÎÔÚˆÙ½Ó˜ ÙÔ˘ ÈÔ‡, ·ÔÙÂÏÔ‡Ó ·fi ÔÚÈṲ̂ÓÔ˘˜ ÂÚ¢ÓËÙ¤˜ ÙËÓ Èı·Ó‹ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ· Ù˘ ÓfiÛÔ˘.

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ÙÂÏÂÈfiÌËÓÔ˘ ¿ÚÚÂÓÔ˜ ÓÂÔÁÓÔ‡, Ô˘ ÁÂÓÓ‹ıËΠ̠ηÈÛ·ÚÈ΋ ÙÔÌ‹ ·fi ÌË Û˘ÁÁÂÓ›˜ ÁÔÓ›˜, ÌÂÙ¿ ·fi Â͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔÔ›ËÛË. ∂›ÎÔÛÈ Ù¤ÛÛÂÚȘ ÒÚ˜ ÚÔ Ù˘ Á¤ÓÓËÛ˘, Ë ÌËÙ¤Ú· ·ÚÔ˘Û›·Û ˘„ËÏfi ˘ÚÂÙfi Î·È ÙÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÂÍ¿ÓıËÌ· Ù˘ ÈÏ·Ú¿˜, ηıÈÛÙÒÓÙ·˜ Èı·Ó‹ ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘. ∏ ÌËÙ¤Ú· ‰ÂÓ Â›¯Â ıÂÙÈÎfi ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi ÁÈ· ÂÓÂÚÁËÙÈ΋ ‹ ·ıËÙÈ΋ ·ÓÔÛ›· ·fi ÙÔÓ Èfi Ù˘ ÈÏ·Ú¿˜, ·ÊÔ‡ ‰ÂÓ ·Ó·ÊÂÚfiÙ·Ó ÓfiÛËÛË ‹ ÂÌ‚ÔÏÈ·ÛÌfi˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜. ∂ÚÁ·ÛÙËÚȷ΋ ÂȂ‚·›ˆÛË Ù˘ ÓfiÛÔ˘ ‰ÂÓ ¤ÁÈÓ ÙË Û˘ÁÎÂÎÚÈ̤ÓË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹. øÛÙfiÛÔ, ˘„ËÏfi˜ Ù›ÙÏÔ˜ ·ÓÙÈÛˆÌ¿ÙˆÓ IgG ÛÙÔÓ ÔÚfi Ù˘ ÌËÙ¤Ú·˜ ‰È·ÈÛÙÒıËΠÌÂÙ¿ ·fi 14 Ì‹Ó˜. ∆Ô ÓÂÔÁÓfi ‹Ù·Ó ˘ÁȤ˜ ηٿ ÙËÓ ¤ÍÔ‰fi ÙÔ˘ ·fi ÙÔ ÓÔÛÔÎÔÌ›Ô. ∏ ˘fiÏÔÈË ÂÚÈÁÂÓÓËÙÈ΋ ÂÚ›Ô‰Ô˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ™Â ËÏÈΛ· 5 ÌËÓÒÓ, ÙÔ ·È‰› ÂÌÊ¿-

ÓÈÛ ÂÛÙÈ·ÎÔ‡˜ Û·ÛÌÔ‡˜ Ì ‰ÂÍÈ¿ ÂÓÙfiÈÛË.

OÈ ÂÚÁ·ÛÙËÚȷΤ˜ ÂÍÂÙ¿ÛÂȘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Ù˘ ÂͤٷÛ˘ ÙÔ˘ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘ ˘ÁÚÔ‡, ÙÔ˘ ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ·Ê‹Ì·ÙÔ˜ Î·È Ù˘ ·ÍÔÓÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ ÂÁÎÂÊ¿ÏÔ˘. ªÂÙ¿ ·fi Û‡ÓÙÔÌÔ ‰È¿ÛÙËÌ· ‡ÊÂÛ˘, ÙÔ ‚Ú¤ÊÔ˜ ·ÚÔ˘Û›·ÛÂ Î·È ‰Â‡ÙÂÚÔ ÂÂÈÛfi‰ÈÔ Ì Â·Ó·Ï·Ì‚·ÓfiÌÂÓÔ˘˜ Û·ÛÌÔ‡˜ Î·È ÌÂ Î¿Ì„Ë ÙÔ˘ ‰ÂÍÈÔ‡ Ô‰ÈÔ‡. ªÂÙ¿ ·fi ·˘Ùfi ÙÔ ÂÂÈÛfi‰ÈÔ, ÙÔ ‚Ú¤ÊÔ˜ ·ÚÔ˘ÛÈ·˙fiÙ·Ó Û˘¯Ó¿ Ó· ÎÚ·Ù¿ ÙÔ ‰ÂÍ› fi‰È ÛÂ Î¿Ì„Ë Î·È ÙÔ ‰ÂÍ› ¯¤ÚÈ Û ÛÊȯً ÁÚÔıÈ¿. ∏ Ó¢ÚÔ·Ó·Ù˘Íȷ΋ ÙÔ˘ ÂͤÏÈÍË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋ ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 10 ÌËÓÒÓ. ◊Ù·Ó ÈηÓfi˜ Ó· οıÂÙ·È ¯ˆÚ›˜ ˘ÔÛÙ‹ÚÈÍË Î·È Ó· ÛËÎÒÓÂÙ·È ÛÙËÓ fiÚıÈ· ı¤ÛË ÛÙËÚÈ˙fiÌÂÓÔ˜ Û οÔÈÔ ¤ÈÏÔ. ªÂÙ¿ ·fi ·˘Ù‹ ÙËÓ ËÏÈΛ· ¿Ú¯ÈÛ ̛· ·ÚÁ‹ ·ÏÈÓ‰ÚfiÌËÛË. ¢ÂÓ ÌÔÚÔ‡Û ϤÔÓ Ó· οıÂÙ·È Î·È Ó· ÛÙËÚ›˙ÂÈ ÙÔ ÎÂÊ¿ÏÈ ÛÙ·ıÂÚ¿. Œ¯·Û ÙÔ ÂӉȷʤÚÔÓ ÙÔ˘, ‹Ù·Ó ÏÈÁfiÙÂÚÔ ÈηÓfi˜ Ó· ÂÈÎÔÈÓˆÓ› Î·È ¤‰ÂȯÓ ÎÔ˘Ú·Ṳ̂ÓÔ˜ Î·È Â˘ÂÚ¤ıÈÛÙÔ˜. •·ÊÓÈο, Ë Î·Ù¿ÛÙ·Û‹ ÙÔ˘ ÂȉÂÈÓÒıËΠÂÚÈÛÛfiÙÂÚÔ. Œ¯·Û ÙËÓ Â·Ê‹ ÙÔ˘, ÎÔÈÌfiÙ·Ó Ôχ Î·È ÓÈÁfiÙ·Ó Û˘¯Ó¿ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Û›ÙÈÛ˘. ∂Í·ÈÙ›·˜ Ù˘ Âȉ›ӈۋ˜ ÙÔ˘, ·Ú·¤ÌÊıËΠÁÈ· ÂÚ·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô Û ËÏÈΛ· 14 ÌËÓÒÓ. ∫·Ù¿ ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË ‹Ù·Ó ÓˆıÚfi˜ Î·È Â˘ÂÚ¤ıÈÛÙÔ˜, ¯ˆÚ›˜ Â·Ê‹ Ì ÙÔ ÂÚÈ‚¿ÏÏÔÓ. ¢ÂÓ ÌÔÚÔ‡Û ӷ ÂÛÙÈ¿ÛÂÈ ÙÔ ‚ϤÌÌ· ÙÔ˘, ÂÓÒ ‰ÂÓ ˘‹Ú¯·Ó ÂÓÂÚÁËÙÈΤ˜ ÎÈÓ‹ÛÂȘ. ∂ÌÊ¿ÓÈ˙ ÎÔÚÌÈ΋ ˘ÔÙÔÓ›· Î·È ‰ÂÓ ÌÔÚÔ‡Û ӷ ηı›ÛÂÈ Î·È Ó· ÛÙËÚ›ÍÂÈ ÙÔ ÎÂÊ¿ÏÈ ÙÔ˘, ηıÒ˜ Î·È ‹È· ˘ÂÚÙÔÓ›· ÛÙ· ¿ÎÚ·, ÂÚÈÛÛfiÙÂÚÔ ¤ÓÙÔÓË ÛÙ· οو ¿ÎÚ· ¤Ó·ÓÙÈ ÙˆÓ ¿Óˆ ¿ÎÚˆÓ Î·È ÈÔ Ôχ ‰ÂÍÈ¿ ¤Ó·ÓÙÈ ·ÚÈÛÙÂÚ¿. ∆· ·Ú¯¤ÁÔÓ· ·ÓÙ·Ó·ÎÏ·ÛÙÈο, fiˆ˜ ÙÔ ·Û‡ÌÌÂÙÚÔ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎfi ÙÔ˘ ·˘¯¤Ó·, ÂÎχÔÓÙ·Ó ÂÚÈÔ‰Èο Î·È ·˘ÙfiÌ·Ù·. ∆· ÂÓ Ùˆ ‚¿ıÂÈ ÙÂÓfiÓÙÈ· ·ÓÙ·Ó·ÎÏ·ÛÙÈο ‹Ù·Ó ˙ˆËÚ¿, Ì ·˘ÙfiÌ·ÙÔ ÎÏfiÓÔ ÛÙËÓ Ï‹ÍË. ∆· ·ÓÙ·Ó·ÎÏ·ÛÙÈο ÙÔ˘ ·¯›ÏÏÂÈÔ˘ ‹Ù·Ó Û ¤ÎÙ·ÛË ¶·È‰È·ÙÚÈ΋ 2006;69:218-222

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220

ª. ¢·ÛÔÔ‡ÏÔ˘, A. ∫Ô‚¿Ó˘

·ÌÊÔÙÂÚfiÏ¢ڷ. OÈ ÂÚÈÔ‰ÈÎÔ› Û·ÛÌÔ› ÂÚÈÂÏ¿Ì‚·Ó·Ó Ì›· Ù·¯Â›· ¤Ó·ÚÍË, Ì ·fiÙÔÌË Î¿Ì„Ë ÙˆÓ Î¿Ùˆ ¿ÎÚˆÓ ·ÎÔÏÔ˘ıÔ‡ÌÂÓË ·fi Ì›· ‚Ú·‰Â›· ÙÔÓÈ΋ ¤ÎÙ·ÛË, ηıÒ˜ Î·È Â·Ó·Ï·Ì‚·ÓfiÌÂÓÔ ¯·ÛÌÔ˘ÚËÙfi Î·È Ï·Ù¿ÁÈÛÌ· Ù˘ ÁÏÒÛÛ·˜. ºÂ˘Á·Ï¤Â˜ Ì˘ÔÎÏÔӛ˜ ÙˆÓ ‰·¯Ù‡ÏˆÓ ÙˆÓ ¯ÂÚÈÒÓ Î·È ÙˆÓ Ô‰ÈÒÓ ‹Ù·Ó, Â›Û˘, Û˘¯Ó¤˜. ∆Ô ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ· (EEG) ‹Ù·Ó Ù˘ÈÎfi ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù˘ ˘ÔÍ›·˜ ÛÎÏËÚ˘ÓÙÈ΋˜ ·ÓÂÁÎÂÊ·Ï›Ùȉ·˜, Ì ٷ ¯·Ú·ÎÙËÚÈÛÙÈο ÂÚÈÔ‰Èο Û˘ÌϤÁÌ·Ù· ¢ڤˆÓ, ˘„ËÏÔ‡ ‰˘Ó·ÌÈÎÔ‡, ·ÚÁÒÓ ‰¤ÏÙ· Î˘Ì¿ÙˆÓ. ∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘ (brain CT scan), (A & B) Î·È Ë Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· (MRI) (C & D) ÂÁÎÂÊ¿ÏÔ˘ ·ÔÎ¿Ï˘„·Ó ÔÏÏ·Ϥ˜ ˘fi˘ÎÓ˜ ÂÛٛ˜, ȉȷ›ÙÂÚ· ÂÓÙÔÈ˙fiÌÂÓ˜ ÛÙȘ ‚ÚÂÁÌ·ÙÔ-ÈÓȷΤ˜ ÂÚÈÔ¯¤˜ (∂ÈÎfiÓ·). ∆Ô ËÏÂÎÙÚÔ·ÌÊÈ‚ÏËÛÙÚÔÂȉÔÁÚ¿ÊËÌ· (ERG) Î·È Ù· ÚÔÎÏËÙ¿ ‰˘Ó·ÌÈο ÔÙÈÎÔ‡ ÊÏÔÈÔ‡ (VER) ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. ∏ ‰È¿ÁÓˆÛË Ù˘ À™¶∂ ÂȂ‚·ÈÒıËΠ̠ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi ÙÔ˘ Ù›ÙÏÔ˘ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ Ù˘ ÈÏ·Ú¿˜ ÛÙÔÓ ÔÚfi Î·È ÙÔ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi ÙÔ˘ ·ÛıÂÓÔ‡˜. OÈ ÂȉÈΤ˜ ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ Ù˘ ÈÏ·Ú¿˜ IgG ·ÓÔÛÔÛÊ·ÈÚ›Ó˜ Î·È ·ÓÙÈÛÒÌ·Ù· Ô˘ ·Ó·Ï‡ıËÎ·Ó ÛÙÔ ∂ÚÁ·ÛÙ‹ÚÈÔ ÙÔ˘ Pasteur Institute Ì ÙË ¯Ú‹ÛË ELISA (by Virion Pharmaceutical Company) ‹Ù·Ó ·˘ÍË̤Ó˜ [IgG ÔÚÔ‡ ¤Ó·ÓÙÈ ÈÏ·Ú¿˜: 5,5 (>1 ELISA units), IgG ∂¡À ¤Ó·ÓÙÈ ÈÏ·Ú¿˜: 5 (>1 ELISA units), Ô˘ ˘ÂÚ¤‚·ÈÓ ÙȘ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ÁÈ· ÙËÓ ·ÓÙ›‰Ú·ÛË], ÂÓÒ ÛÙÔÓ ÔÚfi Ù˘ ÌËÙ¤Ú·˜ ÔÈ ÂȉÈΤ˜ ¤Ó·ÓÙÈ ÈÏ·Ú¿˜ IgG ·ÓÔÛÔÛÊ·ÈÚ›Ó˜ ‹Ù·Ó 5,2 (>1 ELISA units). ∞˘ÍË̤ӷ ÂȉÈο ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ ·ÓÙÈÛÒÌ·Ù· ·ÓȯÓ‡ıËÎ·Ó ÛÙÔ ‚Ú¤ÊÔ˜ ·fi ÙÔ ›‰ÈÔ ÂÚÁ·ÛÙ‹ÚÈÔ 15 Ë̤Ú˜ ·ÚÁfiÙÂÚ·, ·Ú¿ ÙËÓ ¤Ó·ÚÍË Ê·Ú̷΢ÙÈ΋˜ ·ÁˆÁ‹˜ [IgG ÔÚÔ‡: 5,4 (>1 ELISA units) Î·È IgG ∂¡À: 5,2 (>1 ELISA units)]. ∏ ÂÓ‰ÔÚ·¯È·›· Û‡ÓıÂÛË IgG ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ ·fi ÙÔ ‚Ú¤ÊÔ˜ ·ԉ›¯ÙËΠ·fi ‰‡Ô ‰È·ÊÔÚÂÙÈο ÂÚÁ·ÛÙ‹ÚÈ· Î·È ‚Ú¤ıËÎÂ Î·È ÛÙȘ ‰‡Ô ÌÂÙÚ‹ÛÂȘ ·˘ÍË̤ÓË. ∏ IgG ∂¡À/ÔÚÔ‡ ‹Ù·Ó ·˘ÍË̤ÓË Û‡Ìʈӷ Ì ÙÔ ÚÒÙÔ ÂÚÁ·ÛÙ‹ÚÈÔ: IgG ∂¡À: 0,241 g/l (º∆: 0,008-0,061 g/l), IgG ÔÚÔ‡: 24,36 g/l (º∆: 5,6417,65 g/l), ·Ï‚Ô˘Ì›ÓË ∂¡À: 0,154 g/l (º∆: 0,134-0,40 g/l), ·Ï‚Ô˘Ì›ÓË ÔÚÔ‡: 49,2 g/l (º∆: 35-52 g/l), ·Ó·ÏÔÁ›· IgG (IgG ratio) x 10-3: 9,9 (º∆: <3,8), ·Ó·ÏÔÁ›· ·Ï‚Ô˘Ì›Ó˘ (albumine ratio) x 10-3: 3,1 (º∆: <10), Û‡ÓıÂÛË IgG ∂¡À: 93,8 mg/24 h (º∆: 3,3 mg/24 h ηٿ Tourtellote) (5), ÙÔÈ΋ ·Ú·ÁˆÁ‹ IgG ∂¡À [IgG (loc)]: 187,7 mg/l (º∆: 0 mg/l ηٿ Reiber) ¶·È‰È·ÙÚÈ΋ 2006;69:218-222

∂ÈÎfiÓ· 3.

(6), IgG index: 3,2 (º∆: <0,65 ηٿ Tibbling/Link) (7). ∆Ô ‰Â‡ÙÂÚÔ ÂÚÁ·ÛÙ‹ÚÈÔ ¤‰ˆÛ ·ÚfiÌÔÈ· ·ÔÙÂϤÛÌ·Ù· Ì ÙÔ ÚÒÙÔ: IgG ∂¡À: 0,153 g/l, IgG ÔÚÔ‡ 17,92 g/l, ·Ï‚Ô˘Ì›ÓË ∂¡À: 0,0918 g/l, ·Ï‚Ô˘Ì›ÓË ÔÚÔ‡: 35,81 g/l, ·Ï‚Ô˘Ì›ÓË ∂¡À/·Ï‚Ô˘Ì›ÓË ÔÚÔ‡ x 10-3: 2,6 (º∆: <10), IgG ∂¡À/ÔÚÔ‡ ÷ ·Ï‚Ô˘Ì›ÓË ∂¡À/ÔÚÔ‡ (IgG index): 3,3 (º∆: <0,65). ™ÙÔ ∂¡À ·ÓȯÓ‡ıËηÓ, Â›Û˘, ÔÏÈÁÔÎψÓÈο ·ÓÙÈÛÒÌ·Ù·. OÈ IgM ·ÓÔÛÔÛÊ·ÈÚ›Ó˜ ‹Ù·Ó ·ÚÓËÙÈΤ˜, fiˆ˜ Â›Û˘ ·ÚÓËÙÈ΋ ‹Ù·Ó Î·È Ë Î·ÏÏȤÚÁÂÈ· ÙÔ˘ ÈÔ‡, ÙfiÛÔ ÛÙÔ ∂¡À fiÛÔ Î·È ÛÙÔÓ ÔÚfi. ∏ ‰È¿ÁÓˆÛË ¤ıÂÛ À™¶∂ ÛÙ·‰›Ô˘ 2 ÚÔ˜ 3 - Û‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· ηٿ Jabour - Î·È ·ÓÙÈÌÂÙˆ›ÛÙËΠ̠ʷÚ̷΢ÙÈ΋ ·ÁˆÁ‹. ∞ÊÔ‡ ÙÔÔıÂÙ‹ıËΠ¤Ó· Rickmann reservoir ÛÙÔ ÚfiÛıÈÔ Î¤Ú·˜ Ù˘ ‰ÂÍÈ¿˜ Ï¿ÁÈ·˜ ÎÔÈÏ›·˜, ÍÂΛÓËÛ ıÂÚ·›· Ì ÈÓÙÂÚÊÂÚfiÓË-· (10,12) Û ‰fiÛË ÂÊfi‰Ô˘ 100.000 IU/m2, Ì ÛÙ·‰È·Î‹ ·‡ÍËÛË ¤ˆ˜ 106 IU/m2 ηٿ ÙËÓ 5Ë Ë̤ڷ ıÂÚ·›·˜, ·ÎÔÏÔ˘ıÔ‡ÌÂÓË ·fi 6 Û˘Ó‰ڛ˜ ‰È¿ÚÎÂÈ·˜ 5 ËÌÂÚÒÓ Ë Î¿ı ̛·. O ·ÛıÂÓ‹˜ ¤Ï·‚Â Û˘ÓÔÏÈο 30 ‰fiÛÂȘ ̤۷ Û ‰È¿ÛÙËÌ· 6 ‚‰ÔÌ¿‰ˆÓ. ∂›Û˘, ¯ÔÚËÁ‹ıËΠÈÛÔÚÈÓÔÛ‡ÓË (8,9,12) Û ‰fiÛË 100 mg/kg, Ù¤ÛÛÂÚȘ ÊÔÚ¤˜ ËÌÂÚËÛ›ˆ˜ ·fi ÙÔ ÛÙfiÌ· Î·È Á-ÛÊ·ÈÚ›ÓË (11) 0,4 mg/kg ∂º ÁÈ· 5 Ë̤Ú˜, 6 ÊÔÚ¤˜ ÙÔÓ Ì‹Ó· ÁÈ· 3 Ì‹Ó˜. ™·Ó ·ÔÙ¤ÏÂÛÌ· Ù˘ ıÂÚ·›·˜, ˘‹ÚÍ ̛· ÂÚ›Ô‰Ô˜ ÛÙ·ıÂÚÔÔ›ËÛ˘ Ù˘ ÓfiÛÔ˘, ηٿ ÙËÓ ÔÔ›· ÙÔ ·È‰› ¤‰ÂȯÓ ÏÈÁfiÙÂÚÔ Â˘ÂÚ¤ıÈÛÙÔ Î·È ÈηÓfi Ó· ηٷ›ÓÂÈ ÙÔ Ê·ÁËÙfi ÙÔ˘ ¯ˆÚ›˜ ÚÔ‚Ï‹Ì·Ù·. ∆Ô ÂfiÌÂÓÔ ∏∂° ¤‰ÂÈÍ ¯·ÔÙÈÎfi ˘fi‚·ıÚÔ. ∆· ·ÔÙÂϤÛÌ·Ù· ÙÔ˘ ÈÔÏÔÁÈÎÔ‡ ÂϤÁ¯Ô˘ ‰ÂÓ ‚ÂÏÙÈÒıËÎ·Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ ÙÔ˘. ∆Ô ‚Ú¤ÊÔ˜ ·ÚÁfiÙÂÚ· ·Ó¤Ù˘Í Û·ÛÙÈ΋ ÙÂÙÚ·ÏËÁ›·. OÈ Û·ÛÌÔ› ÙÔ˘ ‹Ù·Ó ÏÈÁfiÙÂÚÔ ÛÔ‚·ÚÔ› ηıÒ˜ ÚÔ¯ˆÚÔ‡Û ÚÔ˜ ÙÔ ÙÂÏÈÎfi ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘. OÈ ÂÈÛ·ÁˆÁ¤˜ ÙÔ˘ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ‹Ù·Ó Û˘¯Ó¤˜, ÏfiÁˆ ·Ó·Ó¢ÛÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ Ô˘ ÔÊ›ÏÔÓÙ·Ó Û ÂÂÈÛfi‰È· ÂÈÛÚfiÊËÛ˘.

Pediatr May-June 06

16-05-06

17:21

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ÀÔÍ›· ÛÎÏËÚ˘ÓÙÈ΋ ·ÓÂÁÎÂÊ·Ï›Ùȉ· ÌÂÙ¿ ·fi ÂÓ‰ÔÌ‹ÙÚÈ· Ïԛ̈ÍË

∆ÂÏÈο, ÂÈÛ‹Ïı ÛÙÔ ÙÂÏÈÎfi Ê˘ÙÈÎfi ÛÙ¿‰ÈÔ Î·È ¤ı·Ó Û ËÏÈΛ· 3 ÂÙÒÓ ·fi Ó¢ÌÔÓ›· ÏfiÁˆ ÂÈÛÚfiÊËÛ˘.

™˘˙‹ÙËÛË ¶ÚfiÎÂÈÙ·È ÁÈ· ¤Ó· ÂÚÈÛÙ·ÙÈÎfi ÂÓ‰ÔÌ‹ÙÚÈ·˜ Ïԛ̈͢ ·fi ÙÔÓ Èfi Ù˘ ÈÏ·Ú¿˜, Ì ÂΉ‹ÏˆÛË À™¶∂ ÌÂÙ¿ ·fi ‚Ú·¯‡ ¯ÚfiÓÔ ÂÒ·Û˘. O ΛӉ˘ÓÔ˜ ÁÈ· ÙÔ ¤Ì‚Ú˘Ô ‹ ÙÔ ÓÂÔÁÓfi ÌÂÙ¿ ·fi ÂÚÈÁÂÓÓËÙÈ΋ Ïԛ̈ÍË ·fi ÈÏ·Ú¿ Â›Ó·È Û ÌÂÁ¿ÏË Îϛ̷η ¿ÁÓˆÛÙÔ˜ Î·È ·˘Ùfi ÙÔ ÂÚÈÛÙ·ÙÈÎfi ·ÔÙÂÏ› ÙÔ ÙÚ›ÙÔ Ô˘ ¤¯ÂÈ ·Ó·ÊÂÚı› ÛÙËÓ ·ÁÎfiÛÌÈ· ‚È‚ÏÈÔÁÚ·Ê›·. ŒÓ· ·ÎfiÌË ÂÚÈÛÙ·ÙÈÎfi ·Ó·ÎÔÈÓÒıËΠ·fi ÙÔ˘˜ Zwiauer Î·È Û˘Ó (13) ÙÔ 1995 Î·È ÚfiÛÊ·Ù· ·fi ÙÔ˘˜ Cruzado Î·È Û˘Ó (14) ÙÔ 2003. ª›· ·ÎfiÌË ÂÚ›ÙˆÛË À™¶∂ ÌÂÙ¿ ·fi ÓfiÛËÛË ·fi ÈÏ·Ú¿ ηٿ ÙËÓ 28Ë ÓÂÔÁÓÈ΋ Ë̤ڷ ˙ˆ‹˜, ¤¯ÂÈ Â›Û˘ ·Ó·ÎÔÈÓˆı› ·fi ÙÔ˘˜ Sawaishi Î·È Û˘Ó ÙÔ 1999. ∏ ÌfiÏ˘ÓÛË ·fi ÙÔÓ Èfi ÛÙË Û˘ÁÎÂÎÚÈ̤ÓË ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È, Û˘Ó¤‚Ë Èı·Ófiٷٷ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÊÔÚ›·˜ ÙÔ˘ ÈÔ‡, ÛÙËÓ ÂÚ›Ô‰Ô ÓfiÛËÛ˘ Ù˘ ÌËÙ¤Ú·˜ Ï›ÁÔ ÚÈÓ ÙÔÓ ÙÔÎÂÙfi. ∏ ÌÂÙ·ÊÔÚ¿ ÙÔ˘ ÈÔ‡ Î·È Ë ÚÔÛ‚ÔÏ‹ ÛÙË Û˘Ó¤¯ÂÈ· ÙÔ˘ ∫¡™ ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÔÊÂÈÏfiÙ·Ó Èı·ÓÒ˜ Û ¤ÏÏÂÈ„Ë ÌËÙÚÈÎÒÓ ·ÓÙÈۈ̿وÓ, ·ÓÒÚÈÌË ·¿ÓÙËÛË ÙÔ˘ ·ÓÔÛÔÔÈËÙÈÎÔ‡ Î·È ·Ô˘Û›· ¯ÔÚ‹ÁËÛ˘ ÂȉÈ΋˜ ¤Ó·ÓÙÈ ÈÏ·Ú¿˜ ·ÓÔÛÔÛÊ·ÈÚ›Ó˘. ŸÏ˜ ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ó·ÊÔÚ¤˜ ÂÚÈÁÚ¿ÊÔ˘Ó Ì›· Û‡ÓÙÔÌË ÂÚ›Ô‰Ô ÂÒ·Û˘ Ù˘ ÓfiÛÔ˘, Û ·ÓÙ›ıÂÛË Ì ÙËÓ ÂÚ›Ô‰Ô 5-16 ÂÙÒÓ Ô˘ ··ÈÙÂ›Ù·È ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂΉËψı› Ë ÓfiÛÔ˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ. ∂›Û˘, ÂÚÈÁÚ¿ÊÔ˘Ó Ì›· ÁÚ‹ÁÔÚË ÂͤÏÈÍË ÛÙ· ‰È¿ÊÔÚ· ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘. O ·ÛıÂÓ‹˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚ›ÙˆÛ˘, ÌÂÙ¿ ·fi ˘ÔÎÏÈÓÈ΋ Èı·ÓÒ˜ ÓfiÛËÛË ·fi ÈÏ·Ú¿ ÙȘ ÚÒÙ˜ Ë̤Ú˜ ˙ˆ‹˜ ‹ ÂÓ‰ÔÌ‹ÙÚÈ·, ·ÚÔ˘ÛÈ¿ÛÙËΠ5 Ì‹Ó˜ ·ÚÁfiÙÂÚ· Ì ÂÛÙÈ·ÎÔ‡˜ Î·È ÁÂÓÈÎÂ˘Ì¤ÓÔ˘˜ Ì˘ÔÎÏÔÓÈÎÔ‡˜ Û·ÛÌÔ‡˜ Û˘Ì‚·ÙÔ‡˜ Ì ÛÙ¿‰ÈÔ 2 À™¶∂ - Î·È Ì ̛· ÂÈıÂÙÈ΋ ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘ Û „˘¯ÔÎÈÓËÙÈ΋ ·ÏÈÓ‰ÚfiÌËÛË Î·È Û·ÛÙÈÎfiÙËÙ·, Ô˘ ·ÓÙÈÛÙÔÈ¯Ô‡Û·Ó Û ÛÙ¿‰ÈÔ 3 À™¶∂. ∏ ÂͤÏÈÍ‹ ÙÔ˘ Û ÛÙ¿‰ÈÔ 4 À™¶∂ ‹Ù·Ó ÁÚ‹ÁÔÚË, ·Ú¿ ÙËÓ ¤Ó·ÚÍË ıÂÚ·›·˜ Ì ÈÛÔÚÈÓÔÛ‡ÓË, ÂÓ‰ÔÎÔÈÏȷ΋ ÈÓÙÂÚÊÂÚfiÓË-· Î·È ·ÓÔÛÔÛÊ·ÈÚ›ÓË-Á. ªÂ ·˘Ù‹ ÙËÓ ¤ÓÓÔÈ· Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ·Ô‰Âȯı› Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ·ÁˆÁ‹˜. ∏ Û‡ÓÙÔÌË ÛÙ·ıÂÚÔÔ›ËÛË Ì ÙË ıÂÚ·›· ÌÔÚ› Ó· ·Ô‰Ôı› ›Ù ÛÙË ıÂÚ·›· ›Ù ÛÙËÓ ›‰È· ÙË Ê˘ÛÈ΋ ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘, Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi

221

ÂÚÈfi‰Ô˘˜ Ì·ÎÚ¿˜ ÛÙ·ıÂÚÔÔ›ËÛ˘ ·ÎfiÌË Î·È ÚÔÛˆÚÈÓ‹˜ ‚ÂÏÙ›ˆÛ˘, Ô˘ ·ÎÔÏÔ˘ı› Ù· ·Ú¯Èο ÛÙ¿‰È· ·Ó¿Ù˘Í‹˜ Ù˘. ∏ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙˆÓ ÂÚÈÔ‰ÈÎÒÓ Û·ÛÌÒÓ ·fiÙÔÌ˘ ¤Ó·Ú͢ Î·È ÛÙ·‰È·Î‹˜ ‚Ú·‰Â›·˜ χÛ˘ (Û·Ó Ó· ¯ÚÂÈ¿˙ÂÙ·È Ô Û·ÛÌfi˜ ÂÚÈÛÛfiÙÂÚÔ ¯ÚfiÓÔ Ó· ÂÍ·ÛıÂÓ‹ÛÂÈ ·Ú¿ Ó· ·Ó·‰˘ı›), ηıÒ˜ Î·È Ë ¯·Ú·ÎÙËÚÈÛÙÈ΋ ÂÈÎfiÓ· ÙÔ˘ ∏∂°, Ì ٷ ÂÚÈÔ‰Èο ‚Ú·‰¤· ·̷ٷ, Ô˘ fï˜ ‰È‹ÚÎÂÛ ÁÈ· Û‡ÓÙÔÌÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ηٿ ÙËÓ ÂͤÏÈÍË ·fi ÙÔ ÛÙ¿‰ÈÔ 2 ÛÙÔ ÛÙ¿‰ÈÔ 3, ¤ıÂÛ·Ó ÙË ‰È¿ÁÓˆÛË Ù˘ À™¶∂. ∆· ÂfiÌÂÓ· ∏∂° ‰ÂÓ ‹Ù·Ó ‰È·ÁÓˆÛÙÈο. ∏ ·Ó·ÊÔÚ¿ ÙˆÓ ÂÈÙÒÛÂˆÓ ÛÙÔ ¤Ì‚Ú˘Ô ÌÂÙ¿ ·fi ÚÔÛ‚ÔÏ‹ Ù˘ ÂÁ·Ԣ ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÙÂÏÂ˘Ù·›Ô˘ ÙÚÈÌ‹ÓÔ˘ ·ËÛ˘ Â›Ó·È Û¿ÓÈ· ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·. ¶ÚÈÓ ÁÂÓÈ΢ٛ Î·È Á›ÓÂÈ ˘Ô¯ÚˆÙÈÎfi˜ Ô ÚÔÏËÙÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜, Ë ÈÏ·Ú¿ ‹Ù·Ó Ì›· ÓfiÛÔ˜ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜. ∏ Û˘ÓÙÚÈÙÈ΋ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÓËÏ›ÎˆÓ ‹Ù·Ó ¿ÓÔÛÔÈ Î·È Ù· ÓÂÔÁÓ¿ ÚÔÛٷهÔÓÙ·Ó ·fi ÙËÓ ·ıËÙÈ΋ ·ÓÔÛ›·. O ·ÓÂ·Ú΋˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ Ù˘ ÎÔÈÓfiÙËÙ·˜ ÂÏ·¯ÈÛÙÔÔÈ›, ·ÏÏ¿ ‰ÂÓ ÂÍ·Ê·Ó›˙ÂÈ ÙËÓ Î˘ÎÏÔÊÔÚ›· ÙÔ˘ ÈÔ‡, ÂÓÒ ·˘Í¿ÓÂÈ ÙÔ Û‡ÓÔÏÔ ÙˆÓ Â›ÓÔÛˆÓ ·ÙfïÓ, Ù· ÔÔ›· Â›Ó·È Î˘Ú›ˆ˜ ·ÓÂÌ‚ÔÏ›·ÛÙ· ·È‰È¿ ËÏÈΛ·˜ 2-4 ÂÙÒÓ, Ô˘ ·ÔÙÂÏÔ‡Ó ËÁ‹ ÌfiÏ˘ÓÛ˘ ÁÈ· ·È‰È¿ ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 2 ÂÙÒÓ, ÛÙ· ÔÔ›· Ô Î›Ó‰˘ÓÔ˜ À™¶∂ ÌÂÙ¿ ·fi ÓfiÛËÛË ·fi ÙÔÓ Èfi Ù˘ ÈÏ·Ú¿˜ Â›Ó·È ÁÓˆÛÙfi fiÙÈ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚÔ˜ (16). ∞ÓÙ›ıÂÙ·, Ô ˘Ô¯ÚˆÙÈÎfi˜ ηıÔÏÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ ¤¯ÂÈ Û·Ó ·ÔÙ¤ÏÂÛÌ· Ó· ÌËÓ Î˘ÎÏÔÊÔÚ› Ô Èfi˜ ϤÔÓ ÛÙËÓ ÎÔÈÓfiÙËÙ· Î·È Ó· ÌËÓ ÚÔÛ‚¿ÏÏÔÓÙ·È È· ÔÈ ¤ÁÎ˘Â˜ Á˘Ó·›Î˜. OÈ ·Ó·ÊÔÚ¤˜ ÂÚÈÛÙ·ÙÈÎÒÓ Ì À™¶∂ ÌÂÙ¿ ·fi ÂÚÈÁÂÓÓËÙÈ΋ Ïԛ̈ÍË ·Ô‰ÂÈÎÓ‡ÂÈ ÙËÓ ·Ó¿ÁÎË ÚÔÛÙ·Û›·˜ ·fi ÙÔÓ Èfi ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘. O ˘Ô¯ÚˆÙÈÎfi˜ ηıÔÏÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ ·›˙ÂÈ, ÂÔ̤ӈ˜, ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÚÔ˜ ·˘Ù‹ ÙËÓ Î·Ù‡ı˘ÓÛË. ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ë ·Ú·ÙËÚÔ‡ÌÂÓË ¯·ÌËÏ‹ Â›ÙˆÛË Ù˘ ÈÏ·Ú¿˜ ÔÊ›ÏÂÙ·È ÛÙÔÓ ˘Ô¯ÚˆÙÈÎfi Î·È Û ¢Ú›· Îϛ̷η ÚÔÏËÙÈÎfi ÂÌ‚ÔÏÈ·ÛÌfi Î·È ÛÙËÓ ˘„ËÏ‹ ÚÔÛÙ·Û›· Ô˘ ÚÔÛʤÚÂÈ ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡. ∞Ó Î·È ˘¿Ú¯Ô˘Ó ÔÚÈṲ̂Ó˜ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ó·ÊÔÚ¤˜ Ô˘ Û˘Û¯ÂÙ›˙Ô˘Ó ÙÔ ÂÌ‚fiÏÈÔ Î·Ù¿ Ù˘ ÈÏ·Ú¿˜ Ì ÂÚÈÛÙ·ÙÈο À™¶∂, Û ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ, ˆÛÙfiÛÔ, Ô ˘‡ı˘ÓÔ˜ ÁÈ· ÙË ÓfiÛÔ Èfi˜ Ô˘ Ù·˘ÙÔÔÈ‹ıËΠ‹Ù·Ó Ô ¿ÁÚÈÔ˜ Èfi˜ Ù˘ ÈÏ·Ú¿˜, ÁÂÁÔÓfi˜ Ô˘ Û˘ÓËÁÔÚ› ÁÈ· Ïԛ̈ÍË ·fi ÙÔÓ ¿ÁÚÈÔ Èfi Ù˘ ÈÏ·Ú¿˜ Î·È fi¯È ·fi ÙÔ Û٤ϯԘ ÙÔ˘ ÈÔ‡ Ô˘ ÂÚȤ¯ÂÙ·È ÛÙÔ ÂÌ‚fiÏÈÔ, ÌÂÈÒÓÔÓÙ·˜ ¤ÙÛÈ ÙËÓ ·Í›· ÙˆÓ ·Ú·¿Óˆ ·Ó·ÊÔÚÒÓ (17-19). ¶·È‰È·ÙÚÈ΋ 2006;69:218-222

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ª. ¢·ÛÔÔ‡ÏÔ˘, A. ∫Ô‚¿Ó˘

∏ ÂÚÈÁÚ·Ê‹ ÙÔ˘ ·ÚfiÓÙÔ˜ ÂÚÈÛÙ·ÙÈÎÔ‡ ¤¯ÂÈ ˆ˜ ÛÎÔfi Ó· ·˘Í‹ÛÂÈ ÙËÓ ÂÁÚ‹ÁÔÚÛË ÙˆÓ ·È‰È¿ÙÚˆÓ Î·È ÙˆÓ Á˘Ó·ÈÎÔÏfiÁˆÓ ÁÈ· ÙËÓ ¤ÁηÈÚË ·Ó·ÁÓÒÚÈÛË ÈÏ·Ú¿˜ ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ Î·È ÙË ¯ÔÚ‹ÁËÛË ÚÔÛٷ٢ÙÈ΋˜ Á-ÛÊ·ÈÚ›Ó˘ ÛÙÔ ÓÂÔÁÓfi ηٿ ÙË Á¤ÓÓËÛË, ηıÒ˜ Î·È Ó· ÙÔÓ›ÛÂÈ ÙËÓ ·Ó¿ÁÎË Û˘Ó¯ԇ˜ ÚÔÛ¿ıÂÈ·˜ ÁÈ· ÁÂÓÈÎÂ˘Ì¤ÓÔ Î·ıÔÏÈÎfi ÂÌ‚ÔÏÈ·ÛÌfi Ù˘ ÎÔÈÓfiÙËÙ·˜ ¤Ó·ÓÙÈ Ù˘ ÈÏ·Ú¿˜.

™˘ÌÂÚ¿ÛÌ·Ù· ∏ À™¶∂ Â›Ó·È Ì›· ı·Ó·ÙËÊfiÚÔ˜ ÓfiÛÔ˜ ÙÔ˘ ∫¡™ Î·È Ë Â›ÙˆÛ‹ Ù˘ ÛÙË ÛËÌÂÚÈÓ‹ ÂÔ¯‹ ¤¯ÂÈ ÌÂȈı› ÛËÌ·ÓÙÈο ÏfiÁˆ ÙÔ˘ ˘Ô¯ÚˆÙÈÎÔ‡ Û ¢Ú›· Îϛ̷η ÂÌ‚ÔÏÈ·ÛÌÔ‡. π‰È·›ÙÂÚË ÊÚÔÓÙ›‰· ı· Ú¤ÂÈ Ó· ‰›‰ÂÙ·È ÛÙȘ ¯ÒÚ˜ Ô˘ ÙÔ ÔÛÔÛÙfi Î¿Ï˘„˘ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ‰ÂÓ Â›Ó·È ÈηÓÔÔÈËÙÈÎfi, ÒÛÙ fiϘ ÔÈ Á˘Ó·›Î˜ Û ËÏÈΛ· ΢ÔÊÔÚ›·˜ Ó· Ï·Ì‚¿ÓÔ˘Ó ˘Ô¯ÚˆÙÈο ÙËÓ Â·Ó·ÏËÙÈ΋ ‰fiÛË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Jabbour JT, Duenas DA, Modlin J. SSPE: clinical staging, course, and frequency. Arch Neurol 1975;32:493-494.

2. Cathomen T, Mrkic B, Spehner D, Drillien R, Naef R, Pavlovic J et al. A matrix-less measles virus is infectious and elicits extensive cell fusion: consequences for propagation in the brain. EMBO J 1998;17:38993908.

3. Watanabe M, Wang A, Sheng J, Gombart AF, Ayata M, Ueda S et al. Delayed activation of altered fusion glycoprotein in a chronic measles virus variant that causes subacute sclerosing panencephalitis. J Neurovirol 1995;1:177-188.

4. Schneider-Schaulies S, ter Meulen V. Pathogenic aspects of measles virus infections. Arch Virol Suppl 1999;15:139-158.

5. Tourtellotte WW, Ma BI. Multiple sclerosis: the blood-brain-barrier and the measurement of de novo central nervous system IgG synthesis. Neurology 1978;28:76-83.

6. Reiber H, Felgenhauer K. Protein transfer at the blood cerebrospinal fluid barrier and the quantitation of the humoral immune response within the

¶·È‰È·ÙÚÈ΋ 2006;69:218-222

central nervous system. Clin Chim Acta 1987;163:319-328.

7. Link H, Tibbling G. Principles of albumin and IgG analyses in neurological disorders. III. Evaluation of IgG synthesis within the central nervous system in multiple sclerosis. Scand J Clin Lab Invest 1977;37:397-401.

8. Fukuyama Y, Nihei K, Matsumoto S, Ebina T, Kamoshita S, Sato T et al. Clinical effects of MND-19 (Inosiplex) on subacute sclerosing panencephalitis: a multi-institutional collaborative study. The Inosiplex-SSPE Research Committee. Brain Dev (Tokyo) 1987;9:270-282.

9. Silverberg R, Brenner T, Abramsky O. Inosiplex in the treatment of subacute sclerosing panencephalitis.

Arch Neurol 1979;36:374-375.

10. Panitch HS, Gomez-Plascencia J, Norris FH, Cantell K, Smith RA. Subacute sclerosing panencephalitis: remission after treatment with intraventricular interferon. Neurology 1986;36:562-566.

11. Gurer YK, Kukner S, Sarica B. Intravenous gammaglobulin treatment in a patient with subacute sclerosing panencephalitis. Pediatr Neurol 1996;14:72-74.

12. Yalaz K, Anlar B, Oktem F, Aysun S, Ustacelebi S, Gurcay O et al. Intraventricular interferon and oral inosiplex in the treatment of subacute sclerosing panencephalitis. Neurology 1992;42:488-491.

13. Zwiauer K, Forstenpointner E, Popow-Kraupp T, Hauser E, Jellinger KA. Rapid progressive subacute sclerosing panencephalitis after perinatally acquired measles virus infection. Lancet 1995;345:1124.

14. Cruzado D, Masserey-Spicher V, Roux L, Delavelle J, Picard F, Haenggeli CA. Early onset and rapidly progressive subacute sclerosing panencephalitis after congenital measles infection. Eur J Pediatr 2002;161:438-441.

15. Sawaishi Y, Abe T, Yano T, Ishikawa K, Takada G. SSPE following neonatal measles infection. Pediatr Neurol 1999;20:63-65.

16. Sussman J, Compston DA. Subacute sclerosing panencephalitis in Wales. Q J Med 1994;87:23-34.

17. Duclos P, Ward BJ. Measles vaccines: a review of adverse events. Drug Saf 1998;19:435-454.

18. Kawashima H, Miyajima T, Mori T, Yuan L, Ogihara M, Kinoue K et al. A case of intractable epilepsy positive for the detection of measles virus genome in the cerebrospinal fluid and peripheral mononuclear cells using reverse transcriptase-polymerase chain reaction. Brain Dev 1996;18:220-223.

19. Kai C, Yamanouchi K, Sakata H, Miyashita N, Takahashi H, Kobune F. Nucleotide sequences of the M gene of prevailing wild measles viruses and a comparison with subacute sclerosing panencephalitis virus. Virus Genes 1996;12:85-87.

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Proteus syndrome: a case report H. Hatzissevastou - Loukidou, E. Michalopoulou - Manoloutsiou, X.M. Stefopoulou

Abstract: Proteus Syndrome (PS) is a rare congenital disorder characterized by progressive irregular overgrowth of multiple tissues. We report on a female patient with PS who was clinically evaluated at first at the age of 13 and later when she was 16. Manifestations included facial and cranial asymmetry with dolichocephaly, cervicothoracic scoliosis, high myopia, finger deformities with syndactyly and hemihyperplasia of the left shank. The progressive nature of the syndrome is indicated by comparing the clinical features in two different periods of time.

Key words: Hemihyperplasia, asymmetry, disproportionate overgrowth, mosaicism, Proteus syndrome.

Introduction Proteus syndrome (PS) is a rare disorder characterized by disproportionate overgrowth of multiple tissues. This was delineated by Cohen and Hayden in 1979 and was further defined by Wiedemman, who proposed the term Proteus syndrome in order to highlight the morphologic variability of the disease. Complications of PS include, among others, progressive skeletal deformities, invasive lipomas, benign and malignant tumors and deep venous thrombosis with pulmonary embolism (1). The disease process is not usually apparent at birth but develops rapidly in childhood. The clinical expression of the disorder is extremely variable. Molecular pathogenesis of the syndrome is still unknown.

Although specific diagnostic criteria have been published, the rate of misdiagnosis in PS is really high and the variable features of this disorder make differential diagnosis challenging for clinicians. We describe a female patient with PS, who was clinically evaluated first at the age of 13 and later when she was 16 years old.

Case report The patient was evaluated at first for many deformities at the age of 13. Her delivery had been normal without complications. At birth, she was not noted to have any deformities. Later in childhood, she developed progressive deformities of several fingers and asymmetry of the left foot. On examination at 13 years, the patient had a normal height (25th percentile). Referring to the facial phenotype, she had asymmetry of the face, crani-

um, eyes and ears. She also had a long neck and presented high myopia (Figure 1A). Moreover, the young girl had finger deformities with syndactyly of several fingers. The median and ring finger of the left hand had been amputated because of severe deformities. Enlargements from lipomatosis were noted on the fingers joints (Figure 1B). The toes of the right foot presented serious deformities, with over-riding of the third finger upon the second and syndacyly of the fourth and fifth toe (Figure 1C). Furthermore, skeletal abnormalities were already present. The patient presented cervicothoracic scoliosis, hemihyperplasia and asymmetry of the left shank (Figure 1D). Facing all these problems, the girl was trying to hide the finger deformities by wearing bracelets and rings. At the age of 16, the patient was clinically evaluated again. Her mother was pregnant again and came with her daughter to our clinic, for genetic counseling. On examination she was noted to have normal weight, height and development. Facial and cranial asymmetry has deteriorated and she also developed dolichocephaly (Figure 2A,B). Finger and skeletal deformities of the left shank had also gradually deteriorated (Figure 2C,D). The patient was also noted to have developed kyphosis (Figure 2D). Despite the findings of clinical evaluation, the most remarkable sign of this patient was her behavior; she was quiet, depressed and never smiled during her visit. The young patient was introvert and seemed to feel embarrassed about herself.

1st Department of Pediatrics, Cytogenetic Lab, Medical School of Aristotle University of Thessaloniki, Ippokration General Hospital, Thessaloniki-Greece AÏÏËÏÔÁÚ·Ê›·: H. Hatzissevastou-Loukidou 1st Pediatric Department, Lab of Cytogenetics Medical School, Aristotle University of Thessaloniki Ippokration General Hospital, 49, Konstantinoupoleos Str. 546 42, Thessaloniki, Greece E-mail: vnlouk@egnatia.gr Date of submission: 14-02-2006 Date of approval: 31-03-2006

Discussion The cause of PS is yet unknown. Molecular ¶·È‰È·ÙÚÈ΋ 2006;69:223-226

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D

∞

µ

C Figure 1. The patient at the age of 13. A) She has facial and cranial asymmetry; asymmetry of the eyes and ears, long neck and cervicothoracic scoliosis, high myopia. B) Finger deformities with syndacyly of several fingers. The median and ring finger of the left hand have been amputated because of severe deformities, enlargements on the fingers joints. C) Severe deformities of the toes of the right foot, syndactyly of the fourth and D). Facial asymmetry, neck deformity, scoliosis, hypertrophy and asymmetry of the left shank.

investigations have revealed a possible involvement of the PTEN and the glypican 3 (GPC3) gene, but with contradictory results. Recent molecular studies showed that mutation in coding region of PTEN and GPC3 do not appear to be a major cause of PS. It is possible that other genetic or epigenetic alterations, such as deletion, duplication, inversion, methylation, might affect the expression of these two genes (2).

Somatic mosaicism is almost surely responsible for Proteus. Furthermore, the affected infants usually appear normal at birth. They progressively develop the variable features of the ¶·È‰È·ÙÚÈ΋ 2006;69:223-226

disease during childhood and furthermore the overgrowth stops at puberty. Consequently the changes, which have occurred in a adolescent patient, are likely to be the most serious that will occur. We believe that this is the genetics of our girl. Because of the variability in clinical expression, specific diagnostic criteria and evaluation guidelines were developed to clarify the diagnostic confusion surrounding the syndrome (3,4).

Referring to the clinical manifestations of PS, progressive skeletal abnormalities are the most frequent findings and these were mentioned on our patient. One of the most characteristic

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C

D

Figure 2. The patient at the age of age 16. A) Severe facial and cranial asymmetry with dolichocephaly. B) Lateral aspect of our patient, open mouth at rest C) The finger deformities got worst. D) Cervicothoracic scoliosis and kyphosis are apparent; asymmetry of the left shank.

findings is the disorganization and distortion of skeletal features, a finding that contrasts strikingly with more common forms of osseous overgrowth in which the enlarged bones retain their normal proportional relationships. This finding, when accompanied by the typical rapid progression seen in PS, is a key diagnostic criterion (5), and the female patient met this criterion. Cutaneous and soft tissue abnormalities are less frequent. Visceral anomalies, as well as masses other than fatty, muscular and vascular malformations are less common (5-7). Moreover, several ocular manifestations in PS have been reported and high myopia that was mentioned on our pa-

tient is included in those. The most frequent ocular findings associated with the disease, are strabismus and epibulbar tumors (8).

In the absence of a specific diagnostic genetic test, the differential diagnosis of PS presents challenges to physicians. The disorders most commonly confused with PS are Klippel-Trenaunay syndrome, neurofibromatosis type 1 and hemihyperplasia multiple lipomatosis syndrome and it is important to mention that the differential diagnosis is still mainly based on clinical findings and less on imaging. Complications of PS include several neoplasms such as lipomas, meningiomas, ovarian cystadenomas, ¶·È‰È·ÙÚÈ΋ 2006;69:223-226

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monomorphic parotid adenomas and malignant neoplasms (5,7,9-10) Furthermore, deep venous thrombosis with pulmonary embolism (11), is a serious complication and is associated with premature death and high mortality among patients with PS. The treatment efforts are focused on patients who are at high risk for complications (3), and also intend to correct those deformities that cause difficulties in movement. In our case the median and ring finger had been amputated.

In addition to the numerous medical complications, PS is challenging from the psychosocial consequences as well. The majority of individuals have normal intelligence, they are aware of their situation and this leads to more serious psychological effects. Such as our patient who had a depressive disorder, was embarrassed about herself and was trying desperately to hide the severe deformities of her fingers. Parents and affected patients report feelings of isolation due to the extreme rarity of the disorder and social stigmatization attributed to the progressive disfiguring nature of the condition.

In conclusion supportive counseling of parents and affected patients is critical. The doctors should be able to provide the patients and parents with all the necessary information about medical and social coping with the disease. In our case the patient’s terrified mother came to us for genetic counseling. She was counseled that recurrences have not occurred in families and therefore, it would have been extremely unlikely to have another affected child. But she was very anxious and decided to end the pregnancy. Both the rarity and variability of the disorder make it difficult to find practical and effective medical advice for its management. Until a specific genetic test is available, diagnosis and management will depend entirely on the results of physical examination and imaging studies.

¶·È‰È·ÙÚÈ΋ 2006;69:223-226

References 1. Biesecker LG. The multifaceted challenges of Proteus syndrome. JAMA 2001;285:2240-2243.

2. Thiffault I, Schwartz CE, Der Kaloustian V, Foulkes WD. Mutation analysis of the tumor suppressor PTEN and the glypican 3 (GPC3) gene in patients diagnosed with Proteus syndrome. Am J Med Genet 2004;130:123-127.

3. Turner JT, Cohen MM Jr, Biesecker LG. Reassessment of the Proteus syndrome literature: application of diagnostic criteria to published cases. Am J Med Gen 2004;130:111-122.

4. Biesecker LG, Happle R, Mulliken JB, Weksberg R, Graham JM Jr. Proteus syndrome: diagnostic criteria, differential diagnosis, and patient evaluation.

Am J Med Genet 1999;84:389-395.

5. Jamis-Dow CA, Turner J∆, Biesecker LG, Choyke PL. Radiologic manifestations of Proteus syndrome.

Radiographics 2004; 24:1051-1068.

6. Nguyen D, Turner JT, Olsen C, Biesecker LG, Darling TN. Cutaneous manifestations of Proteus syndrome: correlations with general clinical severity.

Arch Dermatol 2004;140:947-953.

7. Andres BM, McCarthy EF Jr, Frassica FJ. A muscular lesion suggestive of focal myositis in a child with Proteus syndrome. Clin Orthop Related Res 2002; 404:326-329.

8. De Becker I, Gajda DJ, Gilbert-Barness E, Cohen MM Jr. Ocular manifestations in Proteus syndrome.

Am J Med Genet 2000;92:350-352.

9. Malamitsi-Puchner A, Kitsiou S, Bartsocas CS. Severe Proteus syndrome in an 18-month-old boy. Am J Med Genet 1987;27:119-125.

10. Demetriadis D, Hager J, Nikolaides N, MalamitsiPuchner A, Bartsocas CS. Proteus syndrome: musculoskeletal manifestations and management: a report of two cases. J Pediatr Orthop 1992;12(1):106-113.

11. Slavotinek AM, Vacha SJ, Peters KF, Biesecker LG. Sudden death caused by pulmonary thromboembolism in Proteus Syndrome. Clin Genet 2000;58: 386-389.

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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

CASE REPORT

227

™‡Ó‰ÚÔÌÔ Saethre-Chotzen Û ·ÛıÂÓ‹ Ì ÌÂÚÈ΋ ÌÔÓÔۈ̛· Ù˘ ¯ÚˆÌÔÛˆÌÈ΋˜ ÂÚÈÔ¯‹˜ 7pter→p15 µ. ∆Ô˘ÏÈ¿ÙÔ˘, ∞. ª·‡ÚÔ˘, ∞. ∫ÔÏȷϤÍË, ∂. ¶··‰ÔÔ‡ÏÔ˘, ¶. ¶·Ó·ÁȈÙÔÔ‡ÏÔ˘, Ã. ∆ÛÂÁ΋, ∂. ∫·Ó·‚¿Î˘, ™. ∫›ÙÛÈÔ˘ - ∆˙¤ÏË

¶ÂÚ›ÏË„Ë: ∆o Û‡Ó‰ÚÔÌÔ Saethre-Chotzen ·ÓÙÈÚÔÛˆ‡ÂÈ ¤Ó·Ó ·fi ÙÔ˘˜ ÈÔ Û˘¯ÓÔ‡˜ Ù‡Ô˘˜ ÎÏËÚÔÓÔÌÈ΋˜ ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛ˘ Ô˘ ÌÂÙ·‚È‚¿˙ÂÙ·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ¯·Ú·ÎÙ‹Ú· Î·È Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ Â›Ó·È ÛÔÚ·‰ÈΤ˜. ¶·ÚÔ˘ÛÈ¿˙ÂÈ È‰È·›ÙÂÚ˜ ‰˘ÛÎÔϛ˜ ÛÙËÓ ÎÏÈÓÈ΋ ‰È¿ÁÓˆÛË, ÏfiÁˆ Ù˘ ÌÂÁ¿Ï˘ ‰ÈÂÈÛ‰˘ÙÈÎfiÙËÙ·˜ Î·È Ê·ÈÓÔÙ˘È΋˜ ÂÙÂÚÔÁ¤ÓÂÈ·˜ Ô˘ ÙÔ ¯·Ú·ÎÙËÚ›˙ÂÈ. ¢ÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7 ¤¯Ô˘Ó ·Ó·ÊÂÚı› Û ÔÚÈṲ̂ÓÔ˘˜ ·ÛıÂÓ›˜ Ô˘ ÏËÚÔ‡Ó Ù· ÂȉÈο ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ·ÁÔÚÈÔ‡ ËÏÈΛ·˜ 4 ÂÙÒÓ Ô˘ ·Ú·¤ÌÊıËΠÁÈ· ÁÂÓÂÙÈ΋ ÂÎÙ›ÌËÛË ÏfiÁˆ ηı˘ÛÙ¤ÚËÛ˘ ÛÙËÓ „˘¯ÔÎÈÓËÙÈ΋ ÂͤÏÈÍË, ȉÈfiÌÔÚÊÔ˘ ÚÔÛˆ›Ԣ Î·È ÈÛÙÔÚÈÎÔ‡ ¯ÂÈÚÔ˘ÚÁËı›۷˜ ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛ˘. ™ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ÂÌÊ¿ÓÈ˙ ÚfiˆÚË Û‡ÁÎÏÂÈÛË ÙˆÓ Ú·ÊÒÓ ÙÔ˘ ÎÚ·Ó›Ô˘, ¯·Ú·ÎÙËÚÈÛÙÈο Ê·ÈÓÔÙ˘Èο ÁÓˆÚ›ÛÌ·Ù· ÙÔ˘ ÚÔÛÒÔ˘ Î·È ·ÓˆÌ·Ï›Â˜ ÙˆÓ ¿Óˆ Î·È Î¿Ùˆ ¿ÎÚˆÓ. ªÂ ‚¿ÛË Ù· ·ÓÙÈÎÂÈÌÂÓÈο Â˘Ú‹Ì·Ù· ÙÔ˘ ·ÛıÂÓ‹ Î·È ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘, ¯·Ú·ÎÙËÚ›ÛıËΠˆ˜ ÛÔÚ·‰È΋ ÂÚ›ÙˆÛË Û˘Ó‰ÚfiÌÔ˘ Saethre-Chotzen. ∏ ÌÂϤÙË ÙˆÓ ¯ÚˆÌÔÛˆÌ¿ÙˆÓ Î·È Ë ÌÔÚȷ΋ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ¤‰ÂÈÍ·Ó fiÙÈ Ô ·ÛıÂÓ‹˜ ›¯Â ÌÂÚÈ΋ ÌÔÓÔۈ̛· Ù˘ ¯ÚˆÌÔÛˆÌÈ΋˜ ÂÚÈÔ¯‹˜ 7pter→p15, ‡ÚËÌ· de novo. ™ÙËÓ ÂÚÈÔ¯‹ 7p21 ÂÓÙÔ›˙ÂÙ·È ÙÔ ÁÔÓ›‰ÈÔ TWIST, Ô˘ ¤¯ÂÈ ‰Ú¿ÛË ÌÂÙ·ÁÚ·ÊÈÎÔ‡ ·Ú¿ÁÔÓÙ·, Ô ÔÔ›Ô˜ Ú˘ıÌ›˙ÂÈ ÙËÓ ·‡ÍËÛË Î·È ·Ó¿Ù˘ÍË ÙˆÓ ÔÛÙÒÓ. ∏ Ï‹Ú˘ ·Ô˘Û›· ÙÔ˘ ÂÓfi˜ ÂÎ ÙˆÓ ‰‡Ô ·ÓÙÈÁÚ¿ÊˆÓ ÙÔ˘ ÁÔÓȉ›Ô˘ (·ÏÔ·ÓÂ¿ÚÎÂÈ·) Ô‰ËÁ› ÛÙËÓ ÂÌÊ¿ÓÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Saethre-Chotzen.

∂ÚÁ·ÛÙ‹ÚÈÔ π·ÙÚÈ΋˜ °ÂÓÂÙÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ÈڤÌÂÈÔ ∂Ú¢ÓËÙÈÎfi ∂ÚÁ·ÛÙ‹ÚÈÔ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: µ·ÛÈÏÈ΋ ∆Ô˘ÏÈ¿ÙÔ˘, ∂ÚÁ·ÛÙ‹ÚÈÔ π·ÙÚÈ΋˜ °ÂÓÂÙÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ÈڤÌÂÈÔ ∂Ú¢ÓËÙÈÎfi ∂ÚÁ·ÛÙ‹ÚÈÔ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∆.∫. 115 27, ∞ı‹Ó·. ∂-mail: antonat@otenet.gr ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 15-09-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 10-02-2006

§¤ÍÂȘ ÎÏÂȉȿ: ™‡Ó‰ÚÔÌÔ Saethre-Chotzen, ÌÔÓÔۈ̛· 7p, ÁÔÓ›‰ÈÔ TWIST, ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛË.

Saethre-Chotzen syndrome in a patient with partial monosomy of the chromosomic region 7pter→p15 V. Touliatou, A. Mavrou, A. Kolialexi, E. Papadopoulou, P. Panayiotopoulou, C. Tsengi, E. Kanavakis, S. Kitsiou - Tzeli

Abstract: Saethre-Chotzen syndrome represents one of the most common types of inherited craniosynostosis. It is an autosomal dominant disorder and most cases are sporadic. It is characterized by high penetrance and variable expression, leading to difficulties in clinical diagnosis. Some patients exhibiting most of the diagnostic criteria of Saethre-Chotzen syndrome have structural abnormalities of chromosome 7. The case is described of a 4 year-old boy with psychomotor retardation, notable dysmorphic features and a history of surgical repair of craniosynostosis. The craniofacial findings included premature fusion of the cranial sutures, characteristic facial appearance and limb abnormalities. Based on the clinical examination of the patient and his parents, he was characterized as a sporadic case of Saethre-Chotzen syndrome. Conventional and molecular cytogenetic analysis of peripheral blood revealed partial monosomy of the chromosomal region 7pter→p15 de novo. The TWIST gene, located on chromosome 7p21, is thought to be a negative transcriptional regulator involved in osteoblast differentiation and maturation, and it is also believed that haploinsufficiency of the gene can cause the disorder.

Medical Genetics Laboratory, University Of Athens, Choremio Research Laboratory, “Aghia Sophia” Children’s Hospital, Athens Correspondence: Vassiliki Touliatou, Medical Genetics Laboratory, University Of Athens, Choremio Research Laboratory, “Aghia Sophia” Children’s Hospital 115 27, Athens E-mail: antonat@otenet.gr Date of submission: 15-09-2005 Date of approval: 10-02-2006

Key words: Saethre-Chotzen syndrome, monosomy 7p, TWIST gene, craniosynostosis.

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µ. ∆Ô˘ÏÈ¿ÙÔ˘ Î·È Û˘Ó.

™˘ÓÙÔÌÔÁڷʛ˜ FISH: ºıÔÚ›˙ˆÓ in situ ˘‚ÚȉÈÛÌfi˜ (fluorescence in situ hybridization) ∂£: ∂ηÙÔÛÙÈ·›· ı¤ÛË FGFR: ÀÔ‰Ô¯¤·˜ ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ (Fibroblast Growth Factor Receptor) bp: ˙‡ÁË ‚¿ÛÂˆÓ (base pairs)

∂ÈÛ·ÁˆÁ‹ ∆Ô Û‡Ó‰ÚÔÌÔ Saethre-Chotzen (acrocephalosyndactyly type III, MIM 101400) ÂÚÈÁÚ¿ÊËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÛÙȘ ·Ú¯¤˜ Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ 1930 Î·È ·ÔÙÂÏ› ÌÈ· ·fi ÙȘ Û˘¯ÓfiÙÂÚ˜ ÌÔÚʤ˜ ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛ˘ ÌÂ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ 1/25000-1/50000 ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi (1). ∫‡ÚÈ· ÁÓˆÚ›ÛÌ·Ù· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Â›Ó·È Ë ÚfiˆÚË Û‡ÁÎÏÂÈÛË ÙˆÓ Ú·ÊÒÓ ÙÔ˘ ÎÚ·Ó›Ô˘ Ô˘ Ô‰ËÁ› Û ÛÙ·ıÂÚ¿ Ê·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ÚÔÛÒÔ˘ Î·È ÙÔ˘ ÎÚ·Ó›Ô˘, ηıÒ˜ Î·È ÔÈ ·ÓˆÌ·Ï›Â˜ ÙˆÓ ¿ÎÚˆÓ. ∫ÏËÚÔÓÔÌÂ›Ù·È Ì ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ¯·Ú·ÎÙ‹Ú·, fï˜ Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È Â›Ó·È ÛÔÚ·‰ÈΤ˜ (2,3). ™Â ÔÚÈṲ̂ÓÔ˘˜ ·ÛıÂÓ›˜ Ì ۇӉÚÔÌÔ Saethre-Chotzen ¤¯Ô˘Ó ·Ó‚ÚÂı› ¯ÚˆÌÔÛˆÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ Î·È ·ÊÔÚÔ‡Ó ‰ÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7, fiˆ˜ ÂÏÏ›ÌÌ·Ù· ‰È·ÊfiÚÔ˘ ÌÂÁ¤ıÔ˘˜, ÌÂÙ·ı¤ÛÂȘ, ·Ó·ÛÙÚÔʤ˜ Î·È ‰·ÎÙ˘ÏÈÔÂȉ¤˜ ¯ÚˆÌfiۈ̷ (4). ÷ڷÎÙËÚÈÛÙÈÎfi ÙˆÓ ·Ó·Î·Ù·Ù¿ÍÂˆÓ ·˘ÙÒÓ Â›Ó·È Ë ÛÙ·ıÂÚ‹ Û˘ÌÌÂÙÔ¯‹ Ù˘ ÂÚÈÔ¯‹˜ 7p21, fiÔ˘ ÂÓÙÔ›˙ÂÙ·È ÙÔ ˘‡ı˘ÓÔ ÁÔÓ›‰ÈÔ TWIST (ªπª 601622) (∂ÈÎfiÓ· 1). ∆Ô ÁÔÓ›‰ÈÔ ·˘Ùfi ·Ó·ÁÓˆÚ›ÛıËΠÙÔ 1997 Î·È Îˆ‰ÈÎÔÔÈ› ÙËÓ ÚˆÙ½ÓË Twist Ô˘ ¤¯ÂÈ ‰Ú¿ÛË ÌÂÙ·ÁÚ·ÊÈÎÔ‡

·Ú¿ÁÔÓÙ·, Ô ÔÔ›Ô˜ Ú˘ıÌ›˙ÂÈ ÙËÓ ·‡ÍËÛË Î·È ·Ó¿Ù˘ÍË ÙˆÓ ÔÛÙÒÓ (5). ª¤¯ÚÈ Û‹ÌÂÚ· ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ÙÔ˘Ï¿¯ÈÛÙÔÓ 80 ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ ·˘Ùfi, ΢ڛˆ˜ ÂÏÏ›ÌÌ·Ù·, ‹ ÚÔÛı‹Î˜ ÓÔ˘ÎÏÂÔÙȉ›ˆÓ, ·ÓÂÚÌËÓ‡ÛÈ̘, ·ÚÂÚÌËÓ‡ÛÈ̘ ηıÒ˜ Î·È ÛËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ Ô˘ ÂËÚ¿˙Ô˘Ó ÙË Û‡ÓıÂÛË ÂȉÈÎÒÓ ÂÚÈÔ¯ÒÓ Ù˘ ÔÏ˘ÂÙȉÈ΋˜ ·Ï˘Û›‰·˜ Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÌË ·Ú·ÁˆÁ‹ ÏÂÈÙÔ˘ÚÁÈ΋˜ ÚˆÙ½Ó˘ (6). ¢ÂÓ ¤¯ÂÈ ·Ú·ÙËÚËı› Û·Ê‹˜ Û˘Û¯¤ÙÈÛË Ê·ÈÓÔÙ‡Ô˘-ÁÔÓÔÙ‡Ô˘ Î·È Â›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈ΋ Ë ÌÂÁ¿ÏË Ê·ÈÓÔÙ˘È΋ ÂÙÂÚÔÁ¤ÓÂÈ· Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÔÈ ·ÛıÂÓ›˜ Ì ۇӉÚÔÌÔ Saethre-Chotzen. ¶·ÚfiÏÔ Ô˘ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ÂÌÊ·Ó›˙Ô˘Ó ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ TWIST, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ¿ÙÔÌ· Ì οÔÈ· Ê·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Ô˘ ʤÚÔ˘Ó ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ÁÔÓ›‰È· FGFR2 (ªπª 176943) Î·È FGFR3 (ªπª 134934), Ù· ÔÔ›· ·ÔÙÂÏÔ‡Ó Ì›· ÔÈÎÔÁ¤ÓÂÈ· ‰È·ÌÂÌ‚Ú·ÓÈÎÒÓ ÎÈÓ·ÛÒÓ Ù˘ Ù˘ÚÔÛ›Ó˘ Ì ڢıÌÈÛÙÈÎfi ÚfiÏÔ ÛÙËÓ ·‡ÍËÛË Î·È ·Ó¿Ù˘ÍË ÙˆÓ ÔÛÙÒÓ (7). ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ·ÁÔÚÈÔ‡ Ì ۇӉÚÔÌÔ Saethre-Chotzen Î·È ÌÂÚÈ΋ ÌÔÓÔۈ̛· Ù˘ ¯ÚˆÌÔÛˆÌÈ΋˜ ÂÚÈÔ¯‹˜ 7pter→p15, ·Ó·Ï‡ÔÓÙ·È Ù· ÎÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο, Ù· Â˘Ú‹Ì·Ù· Ù˘ ÎÏ·ÛÈ΋˜ Î·È ÌÔÚȷ΋˜ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋˜ ÌÂϤÙ˘ Î·È Á›ÓÂÙ·È ·Ó·ÛÎfiËÛË Ù˘ ‰ÈÂıÓÔ‡˜ ‚È‚ÏÈÔÁÚ·Ê›·˜.

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ √ ·ÛıÂÓ‹˜ Â›Ó·È ÙÔ ÚÒÙÔ ·È‰› Ê·ÈÓÔÙ˘Èο ˘ÁÈÒÓ ÁÔÓ¤ˆÓ (30 ÂÙÒÓ Ë ÌËÙ¤Ú· Î·È 40 ÂÙÒÓ Ô ·Ù¤Ú·˜), Ô˘ ÁÂÓÓ‹ıËΠÌÂ Ê˘ÛÈÔÏÔÁÈÎfi

∂ÈÎfiÓ· 1. π‰ÂfiÁÚ·ÌÌ· ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7 Ô˘ ˘Ô‰ÂÈÎÓ‡ÂÈ ÙËÓ ÂÚÈÔ¯‹ Ô˘ ÂÓÙÔ›˙ÂÙ·È ÙÔ ÁÔÓ›‰ÈÔ TWIST (ÎfiÎÎÈÓÔ ¯ÚÒÌ·). ¶·È‰È·ÙÚÈ΋ 2006;69:227-232

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229

™‡Ó‰ÚÔÌÔ Saethre-Chotzen Ì ¤ÏÏÂÈÌÌ· 7pter→p15

ÙÔÎÂÙfi ÌÂÙ¿ ·fi ÙÂÏÂÈfiÌËÓË ÔÌ·Ï‹ ·ËÛË Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÔÔ›·˜ Ë ÌËÙ¤Ú· ‰ÂÓ ÓfiÛËÛÂ, Ô‡Ù ÂÎÙ¤ıËΠ۠ÂÌ‚Ú˘ÔÙÔÍÈο Ê¿Ú̷η ‹ ÛËÌ·ÓÙÈ΋ ‰fiÛË ·ÎÙÈÓÔ‚ÔÏ›·˜. ™ÙË Á¤ÓÓËÛË ÙÔ ‚¿ÚÔ˜ ‹Ù·Ó 2900 ÁÚ (25Ë ∂£), ÙÔ Ì‹ÎÔ˜ ÛÒÌ·ÙÔ˜ 50 ÂÎ (50Ë ∂£), Ë ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜ 34,5 ÂÎ (25Ë-50Ë ∂£) Î·È ÙÔ ÂÚÈÁÂÓÓËÙÈÎfi ÈÛÙÔÚÈÎfi ÂχıÂÚÔ. ∞fi ÙÔ ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÔÓÙ·È ‰È¿ÓÔÈÍË ÙÔ˘ ÚÈÓÔ‰·ÎÚ˘˚ÎÔ‡ fiÚÔ˘ Û ËÏÈΛ· 4 ÌËÓÒÓ Î·È ÓÔÛËÏ›· Û ·È‰È·ÙÚÈ΋ ÎÏÈÓÈ΋ Û ËÏÈΛ· 2 ÂÙÒÓ ÏfiÁˆ „˘¯ÔÎÈÓËÙÈ΋˜ ηı˘ÛÙ¤ÚËÛ˘ Î·È ÌÈÎÚÔÎÂÊ·Ï›·˜. ∞fi ÙÔÓ ÎÏÈÓÈÎfi Î·È ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ‰È·ÈÛÙÒıËÎ·Ó ‚·ÚËÎÔ˝· ·ÁˆÁÈÌfiÙËÙ·˜ ¿Ìʈ, ˘ÂÚÌÂÙÚˆ›· Î·È ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛË. ∏ ·ÎÙÈÓÔÁÚ·Ê›· ÎÚ·Ó›Ô˘ ¤‰ÂÈÍ Ô͢ÎÂÊ·Ï›·, ÏfiÁˆ ÚfiˆÚ˘ Û‡ÁÎÏÂÈÛ˘ Ù˘ ÛÙÂÊ·ÓÈ·›·˜, Ù˘ ÌÂÙˆÈ·›·˜ Î·È Ù˘ Ï·Ì-

∂ÈÎfiÓ· 3. √ ·ÛıÂÓ‹˜ Û ËÏÈΛ· 4 ÂÙÒÓ.

‚‰ÔÂȉԇ˜ Ú·Ê‹˜ Î·È ‰·ÎÙ˘ÏÈÔÂȉ‹ ÂÓÙ˘ÒÌ·Ù·, ÂÓÒ Ë ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘ Î·È ÙÔ ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ· ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. ™˘ÛÙ‹ıËÎ·Ó ÏÔÁÔıÂÚ·›· Î·È ÂÚÁÔıÂÚ·›· Î·È Ô ·ÛıÂÓ‹˜ ˘Ô‚Ï‹ıËΠ۠ӢÚÔ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË Û ËÏÈΛ· 2,5 ÂÙÒÓ. ™ÙÔ È·ÙÚÂ›Ô ∫ÏÈÓÈ΋˜ °ÂÓÂÙÈ΋˜ Ô ·ÛıÂÓ‹˜ ·Ú·¤ÌÊıËΠ۠ËÏÈΛ· 4 ÂÙÒÓ ÏfiÁˆ „˘¯ÔÎÈÓËÙÈ΋˜ ηı˘ÛÙ¤ÚËÛ˘ Î·È È‰ÈfiÌÔÚÊÔ˘ ÚÔÛˆ›Ԣ ÛÙ· Ï·›ÛÈ· ÙÔ˘ ÈÛÙÔÚÈÎÔ‡ ¯ÂÈÚÔ˘ÚÁËı›۷˜ ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛ˘. ™ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ÂÌÊ¿ÓÈ˙ Ô͢ÎÂÊ·Ï›·, ÌÂÁ¿ÏÔ Ì¤ÙˆÔ, ˘ÔÏ·Û›· ̤ÛÔ˘ ÚÔÛÒÔ˘, ˘ÂÚÙÂÏÔÚÈÛÌfi, ÂÍfiÊı·ÏÌÔ Ì Â›Î·ÓıÔ, ‚ÏÂÊ·ÚfiÙˆÛË, ÌÈÎÚ‹ ̇ÙË Ì ¢Ú›· ‚¿ÛË, ÌÈÎÚÔ-ÔÈÛıÔÁÓ·ı›·, ÌÈÎÚÔÛÙÔÌ›·, ıÔψً ˘ÂÚÒ·, ÌÈÎÚ¿ ·˘ÙÈ¿ Ì ÚÔ¤¯ÔÓ ÛΤÏÔ˜ Ù˘ ¤ÏÈη˜ ÙÔ˘ ÙÂÚ˘Á›Ô˘, ¯·ÌËÏ‹ ÚfiÛÊ˘ÛË ÙÔ˘ ÙÚȯˆÙÔ‡ ÛÙÔÓ ·˘¯¤Ó·, ÌÂÚÈ΋ Û˘Ó‰·ÎÙ˘Ï›· 2Ô˘-3Ô˘ ‰·ÎÙ‡ÏÔ˘ ¯ÂÚÈÒÓ ¿Ìʈ, ÎÏÈÓÔ‰·ÎÙ˘Ï›· 5Ô˘ ‰·ÎÙ‡ÏÔ˘ ¯ÂÚÈÒÓ ¿Ìʈ ηıÒ˜ Î·È Â˘Ú›˜ ·ÓÙ›¯ÂÈÚ˜ (∂ÈÎfiÓ˜ 2, 3). ªÂ ‚¿ÛË Ù· Ê·ÈÓÔÙ˘Èο ÁÓˆÚ›ÛÌ·Ù· ¤ÁÈÓÂ Ë ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Saethre-Chotzen Î·È Û˘ÛÙ‹ıËÎ·Ó ˘ÂÚ˯ÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ù˘ ηډȿ˜ Î·È ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Ô˘ ‰ÂÓ ¤‰ÂÈÍ·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· Î·È ¤ÏÂÁ¯Ô˜ ÔÛÙÈ΋˜ ËÏÈΛ·˜ Ô˘ ‹Ù·Ó Û˘Ì‚·Ù‹ Ì ÙË ¯ÚÔÓÔÏÔÁÈ΋.

∫˘ÙÙ·ÚÔÁÂÓÂÙÈ΋ ÌÂϤÙË

∂ÈÎfiÓ· 2. √ ·ÛıÂÓ‹˜ Û ËÏÈΛ· 10 ËÌÂÚÒÓ.

∫Ï·ÛÈ΋ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË: ∞fi ÙË ÌÂϤÙË 50 ÌÂÙ·Ê¿ÛÂˆÓ Ì ÙËÓ Ù¯ÓÈ΋ GTG banding ÌÂÙ¿ ·fi ηÏÏȤÚÁÂÈ· ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ‰È·ÈÛÙÒıËΠ¤ÏÏÂÈÌÌ· ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ‚Ú·¯¤Ô˜ ÛΤÏÔ˘˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7 [del (7)(pter→p15)] (∂ÈÎfiÓ· 4). ¶·È‰È·ÙÚÈ΋ 2006;69:227-232

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µ. ∆Ô˘ÏÈ¿ÙÔ˘ Î·È Û˘Ó.

ªÔÚȷ΋ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË: ªÂϤÙË ÙˆÓ ¯ÚˆÌÔÛˆÌ¿ÙˆÓ Ì ÙËÓ Ù¯ÓÈ΋ ÙÔ˘ ÊıÔÚ›˙ÔÓÙÔ˜ in situ ˘‚ÚȉÈÛÌÔ‡ (FISH) Ì ÙË ¯Ú‹ÛË ·ÓȯÓÂ˘Ù‹ ‚·Ê‹˜ ÔÏfiÎÏËÚÔ˘ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7 (whole chromosome paint) ·¤ÎÏÂÈÛ ÌÂÙ¿ıÂÛË ÙÌ‹Ì·Ùfi˜ ÙÔ˘ Û ¿ÏÏÔ ·˘ÙÔۈ̷ÙÈÎfi ‹ Ê˘ÏÂÙÈÎfi ¯ÚˆÌfiۈ̷ Î·È ÂȂ‚·›ˆÛ ÙËÓ ¤ÏÏÂÈ„Ë ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ‚Ú·¯¤Ô˜ ÛΤÏÔ˘˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7 (∂ÈÎfiÓ· 5). √È Î·Ú˘fiÙ˘ÔÈ ÙˆÓ ÁÔÓ¤ˆÓ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎÔ› Î·È Û˘ÓÂÒ˜, Ë ¯ÚˆÌÔÛˆÌÈ΋ ·ÓˆÌ·Ï›· ÛÙÔÓ ·ÛıÂÓ‹ ‹Ù·Ó de novo. ™˘ÛÙ‹ıËΠÚÔÁÂÓÓËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÛÙËÓ ÂfiÌÂÓË Î‡ËÛË Ù˘ ÌËÙ¤Ú·˜, ηٿ ÙÔÓ ÔÔ›Ô ‰È·ÈÛÙÒıËΠfiÙÈ ÙÔ ¤Ì‚Ú˘Ô ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi ·ÁfiÚÈ Ì ηڢfiÙ˘Ô 46,ÃÀ Î·È Ë Î‡ËÛË Û˘Ó¯›˙ÂÙ·È Ì¤¯ÚÈ Û‹ÌÂÚ· ¯ˆÚ›˜ ÂÈÏÔΤ˜.

™˘˙‹ÙËÛË ∆Ô Û‡Ó‰ÚÔÌÔ Saethre-Chotzen (·ÎÚÔÎÂÊ·ÏÔÛ˘Ó‰·ÎÙ˘Ï›· Ù‡Ô˘ III), ·ÓÙÈÚÔÛˆ‡ÂÈ ¤Ó·Ó ·fi ÙÔ˘˜ Û˘¯ÓfiÙÂÚÔ˘˜ Ù‡Ô˘˜ ÎÏËÚÔÓÔÌÈ΋˜ ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛ˘ ÌÂ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ 1/25.000-1/50.000 ÁÂÓÓ‹ÛÂȘ (1). ∆· ·ÚÈ· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Saethre-Chotzen ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Ù· ÂÍ‹˜: ‚Ú·¯˘ÎÂÊ·Ï›·, ÚÔ‚ÔÏ‹ ÌÂÙÒÔ˘, ·Û˘ÌÌÂÙÚ›· ÚÔÛÒÔ˘ Ô˘ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ù˘ ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛ˘, ÛÙÚ·‚ÈÛÌfi, ˘ÂÚÙÂÏÔÚÈÛÌfi, ‚ÏÂÊ·ÚfiÙˆÛË, ‰˘ÛÏ·ÛÙÈο ÒÙ·, “ηÌÔ˘ÚˆÙ‹ ̇ÙË”, ηıÒ˜ Î·È Û˘Ó‰·ÎÙ˘Ï›· 2Ô˘-3Ô˘ ‰·ÎÙ‡ÏÔ˘ ¿ÎÚ·˜ ¯ÂÈÚfi˜ Î·È 4Ô˘-5Ô˘ ‰·ÎÙ‡ÏÔ˘

∂ÈÎfiÓ· 4. ∫·Ú˘fiÙ˘Ô˜ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ÙÔ˘ ·ÛıÂÓ‹ 46,ÃÀ, del (7)(pter→p15). ∆Ô ‚¤ÏÔ˜ ‰Â›¯ÓÂÈ ÙÔ ¯ÚˆÌfiۈ̷ 7 Ì ÙË ÌÂÚÈ΋ ÌÔÓÔۈ̛· Ù˘ ÂÚÈÔ¯‹˜ 7pter→p15. ¶·È‰È·ÙÚÈ΋ 2006;69:227-232

∂ÈÎfiÓ· 5. ªÂÙ¿Ê·ÛË ·fi ηÏÏȤÚÁÂÈ· ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ÙÔ˘ ·ÛıÂÓ‹ Ì FISH Î·È ·ÓȯÓÂ˘Ù‹ ‚·Ê‹˜ ÔÏfiÎÏËÚÔ˘ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7. Ÿˆ˜ Ê·›ÓÂÙ·È, ‰ÂÓ ˘¿Ú¯ÂÈ ÌÂÙ¿ıÂÛË ÙÌ‹Ì·Ùfi˜ ÙÔ˘ Û ¿ÏÏÔ ·˘ÙÔۈ̷ÙÈÎfi ‹ Ê˘ÏÂÙÈÎfi ¯ÚˆÌfiۈ̷, ·ÏÏ¿ ¤ÏÏÂÈÌÌ· ÙÔ˘ ‚Ú·¯¤Ô˜ ÛΤÏÔ˘˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7.

¿ÎÚÔ˘ Ô‰fi˜. ÕÏÏ· Û˘ÓÔ‰¿ Â˘Ú‹Ì·Ù· Â›Ó·È ‰˘Ó·ÙfiÓ Ó· Â›Ó·È ÙÔ ¯·ÌËÏfi ·Ó¿ÛÙËÌ·, ÔÈ Â˘Ú›˜ ·ÓÙ›¯ÂÈÚ˜, Ë ÌÔÓ‹Ú˘ ¯ÂÈÚÔÌ·ÓÙÈ΋ ÁÚ·ÌÌ‹ ¿Ìʈ ηıÒ˜ Î·È Ë Û˘ÓÔÛÙ¤ˆÛË ÎÂÚΛ‰·˜-ˆÏ¤Ó˘ (2,4). ™·ÓÈfiÙÂÚ· ÌÔÚ› Ó· ·Ú·ÙËÚËıÔ‡Ó Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ ·fi ÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi, ÙÔ Ô˘ÚÔÔÈËÙÈÎfi ‹ ÙÔ ÁÂÓÓËÙÈÎfi Û‡ÛÙËÌ· (2). ™ËÌ·ÓÙÈÎfi˜ ·ÚÈıÌfi˜ ·ÙfiÌˆÓ Ì ۇӉÚÔÌÔ Saethre-Chotzen ÂÌÊ·Ó›˙ÂÈ Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜, ‹ÈÔ˘ ‹ ̤ÙÚÈÔ˘ ‚·ıÌÔ‡ ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË (ÓÔËÙÈÎfi ËÏ›ÎÔ 50-90) Î·È Î·ı˘ÛÙ¤ÚËÛË ÔÌÈÏ›·˜. √È ‰È·Ù·Ú·¯¤˜ ·˘Ù¤˜ Èı·ÓfiÓ Ó· Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ Û˘Ó˘¿Ú¯Ô˘Û· ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛË, fï˜ ÌÂÙ¿ ÙËÓ Ù·˘ÙÔÔ›ËÛË ÙÔ˘ ˘‡ı˘ÓÔ˘ ÁÔÓȉ›Ô˘ TWIST ‰È·ÈÛÙÒıËΠfiÙÈ ·ÛıÂÓ›˜ Ì ÌÂÁ¿Ï· ÂÏÏ›ÌÌ·Ù· ÛÙÔ ÁÔÓ›‰ÈÔ ÂÌÊ·Ó›˙Ô˘Ó ÛÙËÓ ÏÂÈÔÓfiÙËÙ¿ ÙÔ˘˜ (90%) ηı˘ÛÙ¤ÚËÛË ÛÙËÓ „˘¯ÔÎÈÓËÙÈ΋ ·Ó¿Ù˘ÍË Û ۇÁÎÚÈÛË Ì ÂΛÓÔ˘˜ Ô˘ ʤÚÔ˘Ó ÛËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ (10%) (8). ∆Ô ÁÔÓ›‰ÈÔ TWIST ¤¯ÂÈ ‰‡Ô ÂÍÒÓÈ· (ÙÔ ÚÒÙÔ Ì‹ÎÔ˘˜ 772 bp ÂÚȤ¯ÂÈ fiÏË ÙËÓ Îˆ‰ÈÎÔÔÈÔ‡Û· ÂÚÈÔ¯‹) Î·È ¤Ó· ÂÛÒÓÈÔ (538 bp). ∏ ÚˆÙ½ÓË Ô˘ Έ‰ÈÎÔÔÈ› ·ÔÙÂÏÂ›Ù·È ·fi 202 ·ÌÈÓÔͤ· Î·È ¤¯ÂÈ ‰Ú¿ÛË ÌÂÙ·ÁÚ·ÊÈÎÔ‡ ·Ú¿ÁÔÓÙ·, Ô ÔÔ›Ô˜ Ú˘ıÌ›˙ÂÈ ÙË ‰È·ÊÔÚÔÔ›ËÛË Î·È ˆÚ›Ì·ÓÛË Î˘Ú›ˆ˜ ÙˆÓ ÔÛÙÂÔ‚Ï·ÛÙÒÓ Î·È ÙˆÓ Ì˘Ô‚Ï·ÛÙÒÓ, ÂÓÒ Û˘Á¯ÚfiÓˆ˜ ¤¯ÂÈ ·ÚÓËÙÈ΋ Ú˘ıÌÈÛÙÈ΋ ‰Ú¿ÛË Û ˘ԉԯ›˜ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ (Fibroblast Growth Factor Receptor-FGFRs) (9). ™ÙÔ 50-68% ÙˆÓ

Pediatr May-June 06

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™‡Ó‰ÚÔÌÔ Saethre-Chotzen Ì ¤ÏÏÂÈÌÌ· 7pter→p15

·ÛıÂÓÒÓ Ì ۇӉÚÔÌÔ Saethre-Chotzen ¤¯Ô˘Ó ‰È·ÈÛÙˆı› ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ TWIST Î·È ÔÈ Û˘¯ÓfiÙÂÚ˜ ·ÊÔÚÔ‡Ó ÌÂÁ¿Ï· ÂÏÏ›ÌÌ·Ù·, Ô˘ ¤¯Ô˘Ó ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ô˘Û›· ÏÂÈÙÔ˘ÚÁÈ΋˜ ÚˆÙ½Ó˘. ∞fi ÙË Ê‡ÛË ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ Ê·›ÓÂÙ·È fiÙÈ Ë ·ÏÔ·ÓÂ¿ÚÎÂÈ· (haploinsufficiency), ‰ËÏ·‰‹, Ë Ï‹Ú˘ ·Ô˘Û›· ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ÂÓfi˜ ÂÎ ÙˆÓ ‰‡Ô ·ÏÏËÏÔÌfiÚÊˆÓ ÁÔÓȉ›ˆÓ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ ÛÙËÓ ÂÌÊ¿ÓÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, ˘Ô‰ËÏÒÓÔÓÙ·˜ fiÙÈ Ë Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁ›· Ù˘ ÚˆÙ½Ó˘ Twist ÂÍ·ÚÙ¿Ù·È Ô˘ÛÈ·ÛÙÈο ·fi ÙËÓ ÔÛÔÙÈ΋ Ù˘ Â¿ÚÎÂÈ· (10). ª¤¯ÚÈ Û‹ÌÂÚ· ‰ÂÓ ˘¿Ú¯ÂÈ Û·Ê‹˜ Û˘Û¯¤ÙÈÛË Ê·ÈÓÔÙ‡Ô˘-ÁÔÓÔÙ‡Ô˘, ¤¯ÂÈ, fï˜, ·Ó·ÊÂÚı› Û˘Û¯¤ÙÈÛË ·Ó¿ÌÂÛ· ÛÙË ‚·Ú‡ÙËÙ· Ù˘ ·Ó·Ù˘Íȷ΋˜ ηı˘ÛÙ¤ÚËÛ˘ Î·È ÛÙÔ Ì¤ÁÂıÔ˜ ÙˆÓ ÂÏÏÂÈÌÌ¿ÙˆÓ ÙÔ˘ ÁÔÓȉ›Ô˘, ÁÂÁÔÓfi˜ ȉȷ›ÙÂÚ˘ ÚÔÁÓˆÛÙÈ΋˜ ·Í›·˜ ÛÙËÓ ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋ ÙˆÓ ·ÛıÂÓÒÓ (8). √È ÎÚ·ÓÈÔÛ˘ÓÔÛÙÂÒÛÂȘ (Û˘¯ÓfiÙËÙ· 1/2500 ÁÂÓÓ‹ÛÂȘ) ·ÓÙÈÚÔÛˆÂ‡Ô˘Ó Ì›· ÂÙÂÚÔÁÂÓ‹ ÔÌ¿‰· ·ÓˆÌ·ÏÈÒÓ Ô˘ ÔÊ›ÏÔÓÙ·È ÛÙËÓ ÚfiˆÚË Û‡ÁÎÏÂÈÛË ÙˆÓ Ú·ÊÒÓ ÙÔ˘ ÎÚ·Ó›Ô˘. ª¤¯ÚÈ Û‹ÌÂÚ· ¤¯Ô˘Ó ·Ó·ÁÓˆÚÈÛÙ› ÂÚÈÛÛfiÙÂÚÔÈ ·fi 100 ‰È·ÊÔÚÂÙÈÎÔ› Ù‡ÔÈ, ÔÈ 30 ÂÚ›Ô˘ ·fi ÙÔ˘˜ ÔÔ›Ô˘˜ Â›Ó·È ÁÂÓÂÙÈο ηıÔÚÈṲ̂ÓÔÈ (Û˘Ó‰ÚÔÌÈΤ˜ ÌÔÚʤ˜), fiˆ˜ Ù· Û‡Ó‰ÚÔÌ· Saethre-Chotzen, Pfeiffer, Crouzon, JacksonWeiss Î·È Apert. ∏ ÌÂÁ¿ÏË ÔÈÎÈÏÔÌÔÚÊ›· Î·È Ë ·ÏÏËÏÔÂÈÎ¿Ï˘„Ë ÙˆÓ Ê·ÈÓÔÙ˘ÈÎÒÓ ÁÓˆÚÈÛÌ¿ÙˆÓ ‰ËÌÈÔ˘ÚÁÔ‡Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÁÓˆÛÙÈΤ˜ ‰˘ÛÎÔϛ˜. √È ÂÚÈÛÛfiÙÂÚ˜ ·fi ÙȘ Û˘Ó‰ÚÔÌÈΤ˜ ÌÔÚʤ˜ ÎÏËÚÔÓÔÌÔ‡ÓÙ·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ¯·Ú·ÎÙ‹Ú· Î·È Û˘Ó‹ıˆ˜ ÚfiÎÂÈÙ·È ÁÈ· ÛÔÚ·‰ÈΤ˜ ÂÚÈÙÒÛÂȘ . ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ‰È·ÈÛÙÒıËÎÂ Ë ‚Ï¿‚Ë Û ÌÔÚÈ·Îfi Â›Â‰Ô ÁÈ· ÔÚÈṲ̂ӷ Û‡Ó‰ÚÔÌ· ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛ˘, Ô˘ ÚÔËÁÔ˘Ì¤Óˆ˜ ›¯·Ó Ù·ÍÈÓÔÌËı› ‚¿ÛÂÈ ÙˆÓ ÎÏÈÓÈÎÒÓ ÁÓˆÚÈÛÌ¿ÙˆÓ ÙÔ˘˜. ªÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ÁÔÓ›‰È· MSX2, FGFR1, FGFR2, FGFR3 Î·È TWIST ‚Ú¤ıËΠfiÙÈ Û¯ÂÙ›˙ÔÓÙ·È Ì ·ÓˆÌ·Ï›Â˜ ÛÙÔ Û¯ËÌ·ÙÈÛÌfi ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ Ú·ÊÒÓ Î·Ù¿ ÙËÓ ÂÌ‚Ú˘ÔÁ¤ÓÂÛË, ÚÔÛʤÚÔÓÙ·˜ Ӥ˜ ÏËÚÔÊÔڛ˜ ÛÙËÓ ·ıÔÁ¤ÓÂÈ· ·˘ÙÒÓ ÙˆÓ ‰È·Ù·Ú·¯ÒÓ (11). √ ·ÛıÂÓ‹˜ Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È ·ÚÔ˘Û›·˙ ٷ ÂÚÈÛÛfiÙÂÚ· ·fi Ù· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Saethre-Chotzen Î·È ÔÚÈṲ̂ӷ ·fi Ù· ÂÏ¿ÛÛÔÓ·. ∏ ÚfiˆÚË Û‡ÁÎÏÂÈÛË Ù˘ ÛÙÂÊ·ÓÈ·›·˜, Ù˘ ÌÂÙˆÈ·›·˜ Î·È Ù˘ Ï·Ì‚‰ÔÂȉԇ˜ Ú·Ê‹˜ Ô‰‹ÁËÛ fi¯È ÌfiÓÔ Û ‰˘ÛÌÔÚÊ›· ÙÔ˘ ÎÚ·Ó›Ô˘, ‰·ÎÙ˘ÏÈÔÂȉ‹ ÂÓÙ˘ÒÌ·Ù· ÛÙÔÓ ıfiÏÔ Î·È ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ÚÈÓÔ‰·ÎÚ˘˚ÎÔ‡ fiÚÔ˘,

·ÏÏ¿ Î·È Û ÂÈÚfiÛıÂÙ˜ ·ÓˆÌ·Ï›Â˜, fiˆ˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË, Ô›‰ËÌ· Ù˘ ÔÙÈ΋˜ ıËÏ‹˜ Î·È ‰È·Ù·Ú·¯¤˜ Ù˘ ·ÎÔ‹˜ Î·È Ù˘ ÔÌÈÏ›·˜. ∏ ¿ÌÂÛË ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÎÚ›ıËΠÂȂ‚ÏË̤ÓË ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔÊ¢¯ıÔ‡Ó ·ÒÙÂÚ˜ ÂÈÏÔΤ˜, fiˆ˜, Ë ·ÙÚÔÊ›· ÙÔ˘ ÔÙÈÎÔ‡ Ó‡ÚÔ˘ Î·È Ô ˘‰ÚÔΤʷÏÔ˜. ∏ ·Ó¿Ù˘ÍË ÙÔ˘ ÏfiÁÔ˘ ÛÙÔ ·È‰› ‹Ù·Ó ȉȷ›ÙÂÚ· ÂÚÈÔÚÈṲ̂ÓË, Èı·ÓfiÓ Î·È ÏfiÁˆ Ù˘ Û˘Ó˘¿Ú¯Ô˘Û·˜ ‚·ÚËÎÔ˝·˜ ·ÁˆÁÈÌfiÙËÙ·˜, ÂÓÒ Ë ·Ó·Ù˘Íȷ΋ ÂÎÙ›ÌËÛË Ô˘ ¤ÁÈÓ Û ËÏÈΛ· 3,5 ÂÙÒÓ ·ÔÎ¿Ï˘„ ÛÔ‚·Ú‹ ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË (ÓÔËÙÈÎfi ËÏ›ÎÔ 30). ™ÙÔÓ ·ÛıÂÓ‹ Ì·˜ ÂÓÙÔ›ÛıËΠ¤ÏÏÂÈÌÌ· ÛÙÔ ·ÎÚ·›Ô ÙÌ‹Ì· ÙÔ˘ ‚Ú·¯¤Ô˜ ÛΤÏÔ˘˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 7 [del(7)(pter→p15)], ‰ËÏ·‰‹ ÌÂÚÈ΋ ÌÔÓÔۈ̛· Ù˘ ÂÚÈÔ¯‹˜ 7pter→p15. ∏ ¯ÚˆÌÔÛˆÌÈ΋ ·˘Ù‹ ·ÓˆÌ·Ï›· ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÌfiÓÔ Û ÙÚ›· ‚Ú¤ÊË, Ô˘ ›¯·Ó ÎÔÈÓfi ÁÓÒÚÈÛÌ· ÙËÓ ÚÒÈÌË Û‡ÁÎÏÂÈÛË ÙˆÓ Ú·ÊÒÓ ÙÔ˘ ÎÚ·Ó›Ô˘ Î·È ÙÔ È‰ÈfiÌÔÚÊÔ ÚÔÛˆ›Ô, ¯ˆÚ›˜ fï˜ Ó· ÏËÚÔ‡Ó Ù· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Saethre-Chotzen (12). ªÂÁ·Ï‡ÙÂÚÔ ¤ÏÏÂÈÌÌ· 7pter→p21 ·Ó·Ê¤ÚÂÙ·È Û ÌÈ· ·ÎfiÌ· ÂÚ›ÙˆÛË ‚Ú¤ÊÔ˘˜ Ì ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛË, ÔÏÏ·Ϥ˜ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ Î·È „˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË (12). ÕÏϘ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙȘ ·Ú·¿Óˆ ÂÚÈÙÒÛÂȘ, ·ÏÏ¿ ‰ÂÓ ÂÌÊ¿ÓÈ˙Â Ô ·ÛıÂÓ‹˜ Ì·˜, ‹Ù·Ó Ë Û˘ÁÁÂÓ‹˜ ηډÈÔ¿ıÂÈ·, ÔÈ Û¯ÈÛٛ˜, Ë Ú·È‚Ô˚ÔÔ‰›· Î·È Ë ·ÙÚËÛ›· ÚˆÎÙÔ‡. ™ÙËÓ ÔÈÎÔÁ¤ÓÂÈ· Ô˘ ÂÚÈÁÚ¿ÊÔ˘ÌÂ Ë ¯ÚˆÌÔÛˆÌÈ΋ ·ÓˆÌ·Ï›· Ì ʷÈÓfiÙ˘Ô Û˘Ó‰ÚfiÌÔ˘ Saethre-Chotzen Â›Ó·È ÛÔÚ·‰È΋ Î·È Î·Ù¿ Û˘Ó¤ÂÈ· Ë Èı·ÓfiÙËÙ· Ó· ·ÔÎÙ‹ÛÂÈ Î·È ‰Â‡ÙÂÚÔ ¿Û¯ÔÓ ·È‰› Â›Ó·È Ôχ ÌÈÎÚ‹, ÂÎÙfi˜ ·Ó ÚfiÎÂÈÙ·È ÁÈ· ̈۷˚ÎÈÛÌfi ÙˆÓ ÁÔÓ¿‰ˆÓ Û ¤Ó·Ó ·fi ÙÔ˘˜ ‰‡Ô ÁÔÓ›˜ (Á·ÌÂÙÈÎfi˜ ̈۷˚ÎÈÛÌfi˜). ™˘ÛÙ‹ıËΠfï˜, Ó· Á›ÓÂÈ ÚÔÁÂÓÓËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Û οı ÂfiÌÂÓË Î‡ËÛË Ù˘ ÌËÙ¤Ú·˜ ÁÈ· ÙËÓ Èı·ÓfiÙËÙ· Â·Ó¿Ï˄˘ Ù˘ ›‰È·˜ ‹ ¿ÏÏ˘ ¯ÚˆÌÔÛˆÌÈ΋˜ ·ÓˆÌ·Ï›·˜, fiˆ˜ ÈÛ¯‡ÂÈ Û οı ·ÚfiÌÔÈ· ÂÚ›ÙˆÛË (1-2%). ∆· ÔÏÏ·Ï¿ Î·È Û‡ÓıÂÙ· ÚÔ‚Ï‹Ì·Ù· Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·fi ÓˆÚ›˜ ÔÈ ·ÛıÂÓ›˜ Ì ۇӉÚÔÌÔ Saethre-Chotzen, ··ÈÙÔ‡Ó ÙË Û˘ÓÂÚÁ·Û›· ÔÏÏÒÓ ·È‰È·ÙÚÈÎÒÓ ˘ÔÂȉÈÎÔÙ‹ÙˆÓ Î·È ÙË Û˘Ó·ÈÛıËÌ·ÙÈ΋ Î·È „˘¯ÔÏÔÁÈ΋ ˘ÔÛÙ‹ÚÈÍË ÔÏfiÎÏËÚ˘ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. √ ÚfiÏÔ˜ ÙÔ˘ ÁÂÓÂÙÈÛÙ‹ Â›Ó·È Ô˘ÛÈ·ÛÙÈ΋˜ ÛËÌ·Û›·˜ ÙfiÛÔ ÁÈ· ÙËÓ ¤ÁηÈÚË Î·È ·ÎÚÈ‚‹ ‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË, fiÛÔ Î·È ÁÈ· ÙÔÓ Î·ıÔÚÈÛÌfi Ù˘ ÚfiÁÓˆÛ˘ Î·È ÙËÓ Î·ıÔ‰‹ÁËÛË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. ¶·È‰È·ÙÚÈ΋ 2006;69:227-232

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µ. ∆Ô˘ÏÈ¿ÙÔ˘ Î·È Û˘Ó.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Paznekas WA, Cunningham ML, Howard TD, Korf BR, Lipson MH, Grix AW et al. Genetic heterogeneity of Saethre-Chotzen syndrome, due to TWIST and FGFR mutations. Am J Hum Genet 1998;62: 1370-1380.

2. Jones, KL. Saethre-Chotzen syndrome. In: WB Saunders, editor. Smith's Recognizable Patterns of Human Malformation. 5th ed. Philadelphia: WB Saunders Company; 1997. p 412-415.

3. McKusick VA. Mendelian inheritance in man. A catalogue of human genes and genetic disorders, OMIM #101400: Saethre-Chotzen syndrome. 12th ed. Baltimore: The Johns Hopkins University Press; 1998.

4. Gripp KW, Kasparcova V, McDonald-McGinn DM, Bhatt S, Bartlett SP, Storm AL, et al. A diagnostic approach to identifying submicroscopic 7p21 deletions in Saethre-Chotzen syndrome: fluorescence in situ hybridization and dosage-sensitive Southern blot analysis. Genet Med 2001;3:102-108.

5. de Heer IM, de Klein A, van den Ouweland AM, Vermeij-Keers C, Wouters CH, Vaandrager JM, et al. Clinical and genetic analysis of patients with Saethre-Chotzen syndrome. Plast Reconstr Surg 2005;115:1894-1902.

6. Firulli BA, Krawchuk D, Centonze VE, Vargesson N, Virshup DM, Conway SJ, et al. Altered Twist1 and Hand2 dimerization is associated with Saethre-

¶·È‰È·ÙÚÈ΋ 2006;69:227-232

7.

8.

9.

10.

11.

12.

Chotzen syndrome and limb abnormalities. Nature Genet 2005;37:373-381.

Chun K, Teebi AS, Jung JH, Kennedy S, Laframboise R, Meschino WS, et al. Genetic analysis of patients with the Saethre-Chotzen phenotype. Am J Med Genet 2002;110:136-143.

Cai J, Goodman BK, Patel AS, Mulliken JB, Van Maldergem L, Hoganson GE, et al. Increased risk for developmental delay in Saethre-Chotzen syndrome is associated with TWIST deletions: an improved strategy for TWIST mutation screening. Hum Genet 2003;114:68-76.

El Ghouzzi V, Legeai-Mallet L, Aresta S, Benoist C, Munnich A, Gunzburg J, et al. Saethre-Chotzen mutations cause TWIST protein degradation or impaired nuclear location. Hum Mol Genet 2000;9:813-819.

Bourgeois P, Bolcato-Bellemin AL, Danse JM, Bloch-Zupan A, Yoshiba K, Stoetzel C, et al. The variable expressivity and incomplete penetrance of the twist-null heterozygous mouse phenotype resemble those of human Saethre-Chotzen syndrome.

Hum Mol Genet 1998;7:945-957.

Wilkie AO. Craniosynostosis: genes and mechanisms. Hum Mol Genet 1997;6:1647-1656.

Schinzel A. Chromosome 7: Deletions. In: Walter de Gruyter, editor. Catalogue of unbalanced chromosome aberrations in man. 2nd ed. Berlin - New York; 2001. p. 295-297.

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¶Ï·ÁÈÔÎÂÊ·Ï›·: Û˘Û¯¤ÙÈÛË Ì ÙË ı¤ÛË ‡ÓÔ˘, ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ∞. ºÈÏÈ›‰Ë, π. °ÂÚÌ·Ó¿Î˘, ª. ∫·ÏÌ·ÓÙ‹

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·.°.¡.∏, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘

¶ÂÚ›ÏË„Ë: ∏ Ï·ÁÈÔÎÂÊ·Ï›· (plagiocephaly) Â›Ó·È ¤Ó·˜ ÂÏÏËÓÈÎfi˜ fiÚÔ˜ Ô˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÛÙË ‰ÈÂıÓ‹ È·ÙÚÈ΋ ÔÚÔÏÔÁ›· ÁÈ· Ó· ÂÚÈÁÚ¿„ÂÈ ÙËÓ ÂȉÈ΋ ÂΛÓË ·Ú·ÌfiÚʈÛË ÙÔ˘ ÎÚ·Ó›Ô˘ ηٿ ÙËÓ ÔÔ›· Ë ÂÓÙÔÈṲ̂ÓË ÂÈ¤‰ˆÛË ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÎÚ·Ó›Ô˘ Ô‰ËÁ› Û ·ÒÏÂÈ· Ù˘ Û˘ÌÌÂÙÚ›·˜ ÙÔ˘. ∏ Û˘¯ÓfiÙÂÚË ·ÈÙ›· Ù˘ Â›Ó·È Ë Â͈ÙÂÚÈ΋ Û˘Ì›ÂÛË ÙÔ˘ ÎÚ·Ó›Ô˘ ηٿ ÙËÓ ÙÔÔı¤ÙËÛË ÙˆÓ ‚ÚÂÊÒÓ Û˘Ó¯Ҙ ÛÙËÓ ›‰È· ı¤ÛË ‡ÓÔ˘ (Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘). ∏ ÂÊ·ÚÌÔÁ‹ ÙˆÓ Û˘ÛÙ¿ÛÂˆÓ ÁÈ· ‡ÙÈ· ı¤ÛË ‡ÓÔ˘ Ì ÛÙfi¯Ô ÙËÓ ÚfiÏË„Ë ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ·ÈÊÓȉ›Ô˘ ı·Ó¿ÙÔ˘ ÙˆÓ ‚ÚÂÊÒÓ ¤¯ÂÈ ¿ÌÂÛË Â›ÙˆÛË ÛÙË Û˘¯ÓfiÙËÙ· Î·È ÂÓÙfiÈÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘. ™ÙËÓ ·ÚÔ‡Û· ·Ó·ÛÎfiËÛË ·Ó·Ê¤ÚÂÙ·È Ë Ù·ÍÈÓfiÌËÛË Î·È ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ηıÒ˜ Î·È Ë ‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË Î·È ıÂÚ·¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ηٿÛÙ·Û˘ Ì ¤ÌÊ·ÛË ÛÙËÓ Â›ÎÙËÙË Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ Ù˘ ‚ÚÂÊÈ΋˜ ËÏÈΛ·˜. √ ·È‰›·ÙÚÔ˜ η٤¯ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ¤ÁηÈÚË ·Ó·ÁÓÒÚÈÛË Î·È ·ÓÙÈÌÂÙÒÈÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜, Ì ÙË ÛˆÛÙ‹ ηıÔ‰‹ÁËÛË ÙˆÓ ÁÔÓ¤ˆÓ ÛÙȘ ÂÚÈÙÒÛÂȘ Ù˘È΋˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘, ηıÒ˜ Î·È Ì ÙËÓ ¤ÁηÈÚË ·Ú·ÔÌ‹ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÂΛӈÓ, fiÔ˘ Ë Ï·ÁÈÔÎÂÊ·Ï›· Â›Ó·È Èı·ÓÒ˜ ¿ÏÏ˘ ·ÈÙÈÔÏÔÁ›·˜ Î·È ¯Ú‹˙ÂÈ ÂÍÂȉÈÎÂ˘Ì¤Ó˘ ·ÓÙÈÌÂÙÒÈÛ˘.

AÏÏËÏÔÁÚ·Ê›·: ª·Ú›· ∫·ÏÌ·ÓÙ‹, ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¶·.°.¡.∏. ∆.∫. 713 05 µÔ‡Ù˜, ∏Ú¿ÎÏÂÈÔ, ∫Ú‹ÙË E-mail: pedhem@med.uoc.gr ∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 28-03-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 22-07-2005

§¤ÍÂȘ ÎÏÂȉȿ: ¶Ï·ÁÈÔÎÂÊ·Ï›·, ı¤ÛË ‡ÓÔ˘, ÎÚ·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛË, ‚ÚÂÊÈ΋ ËÏÈΛ·.

Plagiocephaly: relation to sleep position, differential diagnosis and management A. Filippidi, I. Germanakis, M. Kalmanti

Abstract: Plagiocephaly is a Greek term which has been used to describe a specific cranial deformity, characterized by the presence of an asymmetric flattening. Most commonly this deformity is due to external cranial compression produced as the infant always lies on the same side of the head (positional plagiocephaly). The recommendations of sleep positioning to prevent Sudden Infant Death Syndrome appear to affect the frequency and location of positional plagiocephaly. In this paper the risk factors and classification of plagiocephaly, the diagnostic approach and the therapeutic recommendations are reviewed, with special emphasis on positional plagiocephaly. The pediatrician has a key role in recognizing plagiocephaly, counseling parents to prevent positional plagiocephaly and referring atypical or severe cases of plagiocephaly, where a more serious diagnosis cannot be ruled out.

Paediatric Clinic, University General Hospital of Heraklion, Heraklion, Crete Correspondence: Maria Kalmanti, Pediatric Clinic, University General Hospital of Heraklion, 713 05 Voutes, Heraklion, Crete E-mail: pedhem@med.uoc.gr Date of submission: 28-03-2005 Date of approval: 22-07-2005

Key words: Positional plagiocephaly, craniosynostosis, sleep position, infancy.

∂ÈÛ·ÁˆÁ‹ ∏ Ï·ÁÈÔÎÂÊ·Ï›· (plagiocephaly) ÔÚ›˙ÂÙ·È ˆ˜ ÂȉÈ΋ ·Û˘ÌÌÂÙÚ›· ÙÔ˘ Û¯‹Ì·ÙÔ˜ ÙÔ˘ ÎÚ·Ó›Ô˘, Ì ÂÓÙÔÈṲ̂ÓË ÂÈ¤‰ˆÛË, Ô˘ Ô‰ËÁ› Û ·Û˘ÌÌÂÙÚ›· ηٿ ÙÔ Ô‚ÂÏÈ·›Ô ‹ ÙÔ ÛÙÂÊ·ÓÈ·›Ô Â›‰Ô. ∏ Û˘¯ÓfiÙÂÚË ·ÈÙ›· Ù˘ Â›Ó·È Ë Â͈ÙÂÚÈ΋ ›ÂÛË ÙÔ˘ ÎÚ·Ó›Ô˘ ηٿ ÙË ÓÂÔÁÓÈ΋ Î·È ‚ÚÂÊÈ΋ ËÏÈΛ· (Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘), Û ÂÚÈÙÒÛÂȘ Ô˘ Û˘Ó¯Ҙ Ë ›‰È· ÏÂ˘Ú¿ Ù˘ ÎÂ-

Ê·Ï‹˜ ¤Ú¯ÂÙ·È Û Â·Ê‹ Ì ÙÔ ÎÚ‚¿ÙÈ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÌÊ¿ÓÈÛË ÂÓÙÔÈṲ̂Ó˘ ÂÈ¤‰ˆÛ˘ ÙÔ˘ ÎÚ·Ó›Ô˘ ÛÙËÓ ÂÈÊ¿ÓÂÈ· Â·Ê‹˜ (1). ¶·Ú¿ÏÏËÏ·, ÔÈ Û˘ÛÙ¿ÛÂȘ ÁÈ· ÙË ÛˆÛÙ‹ ÙÔÔı¤ÙËÛË ÙˆÓ ‚ÚÂÊÒÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ ÂËÚ¿˙Ô˘Ó ÙË Û˘¯ÓfiÙËÙ· Î·È ÙËÓ ÂÓÙfiÈÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ (2-4). ∏ ¤ÁηÈÚË ‰È¿ÎÚÈÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ ·fi ¿ÏÏ˘ ·ÈÙÈÔÏÔÁ›·˜ Ï·ÁÈÔÎÂÊ·Ï›· Â›Ó·È È‰È·›ÙÂÚ· ÛËÌ·ÓÙÈ΋. ¶·È‰È·ÙÚÈ΋ 2006;69:233-241

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·234

234

∞. ºÈÏÈ›‰Ë Î·È Û˘Ó.

∆·ÍÈÓfiÌËÛË Ï·ÁÈÔÎÂÊ·Ï›·˜ ªÂ ‚¿ÛË ÙÔ˘˜ ·ıÔÁÂÓÂÙÈÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜, Ë Ï·ÁÈÔÎÂÊ·Ï›· Ù·ÍÈÓÔÌÂ›Ù·È Û 2 ·ÚȘ ηÙËÁÔڛ˜: ·) ÙË Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›· (synostotic plagiocephaly), Ë ÔÔ›· ÚÔ·ÙÂÈ ·fi ·ÓÒÌ·ÏË ·Û‡ÌÌÂÙÚË Û‡ÁÎÏÂÈÛË ÙˆÓ ÎÚ·ÓÈ·ÎÒÓ Ú·ÊÒÓ Î·È ‚) ÙËÓ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ ‹ ÌË Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›· (deformational plagiocephaly or nonsynostotic plagiocephaly or positional plagiocephaly), Ë ÔÔ›· ÔÊ›ÏÂÙ·È Û Â͈ÙÂÚÈÎÔ‡˜ Ì˯·ÓÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂȉÚÔ‡Ó ¿Óˆ ÛÙÔ ÎÚ·Ó›Ô. ∏ ‰È¿ÎÚÈÛË ¤¯ÂÈ È‰È·›ÙÂÚË ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· ηıÒ˜ Ë Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›·, Û˘Ó‹ıˆ˜, ¯Ú‹˙ÂÈ ¯ÂÈÚÔ˘ÚÁÈ΋˜ ·ÔηٿÛÙ·Û˘ (5,6), ÂÓÒ Ë Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ Â›Ó·È Î·ÏÔ‹ı˘ ηٿÛÙ·ÛË, Ô˘ Û˘Ó‹ıˆ˜ ‚ÂÏÙÈÒÓÂÙ·È Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ Î·È Ì ԉËÁ›Â˜ ηٿÏÏËÏ˘ ÙÔÔı¤ÙËÛ˘ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ (7).

AÓ¿ÏÔÁ· Ì ÙË ÂÓÙfiÈÛË Ù˘ ·Û‡ÌÌÂÙÚ˘ ÂÈ¤‰ˆÛ˘ ÙÔ˘ ÎÚ·Ó›Ô˘ Ë Ï·ÁÈÔÎÂÊ·Ï›· ‰È·ÎÚ›ÓÂÙ·È ÛÂ: ·) ÚfiÛıÈ·-ÌÂÙˆÈ·›· Î·È ‚) Ô›ÛıÈ·-ÈÓȷ΋. ∏ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ Â›Ó·È Û˘¯ÓfiÙÂÚ· ÈÓȷ΋, ÂÓÒ Ë Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›· ÌÔÚ› Ó· Â›Ó·È Â›Ù ÌÂÙˆÈ·›· ›Ù ÈÓȷ΋ (Ô˘ ÔÊ›ÏÂÙ·È ÛÂ Û˘ÓÔÛÙ¤ˆÛË Ù˘ ÛÙÂÊ·ÓÈ·›·˜ Î·È Ï·Ì‚‰ÔÂȉԇ˜ Ú·Ê‹˜ ·ÓÙ›ÛÙÔȯ·) (7). ¶Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ ∏ ‰˘ÛÌÔÚÊ›· ÙÔ˘ ÎÚ·Ó›Ô˘ Á›ÓÂÙ·È ·ÓÙÈÏËÙ‹ ›Ù ηٿ ÙË Á¤ÓÓËÛË ‹ ·ÚÁfiÙÂÚ·, ηٿ ÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, ·Ó¿ÏÔÁ· Ì ÙÔ ·Ó Ë Â͈ÙÂÚÈ΋ Ì˯·ÓÈ΋ Û˘Ì›ÂÛË ÙÔ˘ ÎÚ·Ó›Ô˘ ¤Ï·‚ ¯ÒÚ· ÛÙËÓ ÂÓ‰ÔÌ‹ÙÚÈÔ ˙ˆ‹ ‹ ÌÂÙ¿ ÙÔÓ ÙÔÎÂÙfi. √È ÂÚÈÙÒÛÂȘ Ï·ÁÈÔÎÂÊ·Ï›·˜ Ô˘ Â›Ó·È ÂÌÊ·Ó›˜ ηٿ ÙË Á¤ÓÓËÛË, ÔÊ›ÏÔÓÙ·È Û ÂÓ‰ÔÌ‹ÙÚÈ· Û˘Ì›ÂÛË ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡ ÎÚ·Ó›Ô˘, ›Ù ·fi Ù· ÔÛÙ¿ Ù˘ ÌËÙÚÈ΋˜ ˘¤ÏÔ˘ ›Ù ·fi Ù· ¿ÎÚ· ÙÔ˘ ¿ÏÏÔ˘ ÂÌ‚Ú‡Ô˘ Û ÂÚÈÙÒÛÂȘ Ôχ‰˘Ì˘ ·ËÛ˘. ∏ ‰ÂÍÈ¿ ÈÓȷ΋ Ï·ÁÈÔÎÂÊ·Ï›· Â›Ó·È Û˘¯ÓfiÙÂÚË Ù˘ ·ÚÈÛÙÂÚ‹˜ ÈÓȷ΋˜ (Û ·Ó·ÏÔÁ›· ÙÔ˘Ï¿¯ÈÛÙÔÓ 3:2) ηıÒ˜ Ë Û˘ÓËı¤ÛÙÂÚË ÂÓ‰ÔÌ‹ÙÚÈ· ı¤ÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘, Û ÔÛÔÛÙfi ¤ˆ˜ 80%, Â›Ó·È Ë ÎÂÊ·ÏÈ΋ ÚÔ‚ÔÏ‹ Ì Â·Ê‹ Ù˘ ‰ÂÍÈ¿˜ ÈÓȷ΋˜ ÂÚÈÔ¯‹˜ ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡ ÎÚ·Ó›Ô˘ Ì ٷ ÔÛÙ¿ Ù˘ ÌËÙÚÈ΋˜ Ë‚È΋˜ Û‡ÌÊ˘Û˘ (7-10). ∏ Ï·ÁÈÔÎÂÊ·Ï›·, ÏfiÁˆ ÂÓ‰ÔÌ‹ÙÚÈ·˜ ı¤Û˘, ‚ÂÏÙÈÒÓÂÙ·È ·˘ÙfiÌ·Ù· ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ ÌËÓÒÓ Ù˘ ˙ˆ‹˜, ˘fi ÙËÓ ÚÔ¸fiıÂÛË fiÙÈ ÙÔ ‚Ú¤ÊÔ˜ ‰ÂÓ ÙÔÔıÂÙÂ›Ù·È Û ı¤ÛË ‡ÓÔ˘ Ì ¶·È‰È·ÙÚÈ΋ 2006;69:233-241

ÙËÓ ÂÈ‰ˆÌ¤ÓË ÂÚÈÔ¯‹ ÙÔ˘ ÎÚ·Ó›Ô˘ Û Â·Ê‹ Ì ÙÔ ÎÚ‚¿ÙÈ (2). ∞ÓÙÈı¤Ùˆ˜, ÛÙËÓ Â›ÎÙËÙË Ï·ÁÈÔÎÂÊ·Ï›· ÏfiÁˆ ı¤Û˘ ‡ÓÔ˘ Ë ‰˘ÛÌÔÚÊ›· ÙÔ˘ ÎÚ·Ó›Ô˘ Á›ÓÂÙ·È ·ÓÙÈÏËÙ‹ ÌÂٷ͇ ÙÔ˘ 2Ô˘ Î·È 3Ô˘ Ì‹Ó· Ù˘ ˙ˆ‹˜. ™˘ÁÎÂÎÚÈ̤ӷ ÙÔ Û¯‹Ì· ÙÔ˘ ÎÚ·Ó›Ô˘ ÂÓÒ ÂÚÈÁÚ¿ÊÂÙ·È Ó· Â›Ó·È Û˘ÌÌÂÙÚÈÎfi ηٿ ÙË Á¤ÓÓËÛË, Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ ·ÏÏ¿˙ÂÈ Î·È Á›ÓÂÙ·È ·ÓÙÈÏËÙ‹ Ë ·Û‡ÌÌÂÙÚË ÂÈ¤‰ˆÛË, Û˘¯ÓfiÙÂÚ· ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÈÓÈ·ÎÔ‡ ÔÛÙÔ‡ (11,12). ∏ ¯ÚÔÓÈ΋ ÂͤÏÈÍË Ù˘ ‰˘ÛÌÔÚÊ›·˜ Â›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈ΋: ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿ ÙȘ ÚÒÙ˜ ‚‰ÔÌ¿‰Â˜ Ù˘ ˙ˆ‹˜, ηٿ ÙȘ Ôԛ˜ ÙÔ ‚Ú¤ÊÔ˜ ÙÔÔıÂÙÂ›Ù·È ÛÙÔ ÎÚ‚¿ÙÈ Ì ÙËÓ ›‰È· ¿ÓÙÔÙ ÏÂ˘Ú¿ Ù˘ ÎÂÊ·Ï‹˜ Ó· ‚Ú›ÛÎÂÙ·È ¯·ÌËÏfiÙÂÚ· Î·È Ó· ‰¤¯ÂÙ·È ›ÂÛË, ÂÓÒ ‚ÂÏÙÈÒÓÂÙ·È ·ÚÁfiÙÂÚ· ·ÎÔÏÔ˘ıÒÓÙ·˜ ÙËÓ ÂͤÏÈÍË Ù˘ ·‰Ú‹˜ ÎÈÓËÙÈÎfiÙËÙ·˜ ηıÒ˜ ÙÔ ‚Ú¤ÊÔ˜ ÂÈÙ˘Á¯¿ÓÂÈ ÙÔÓ Ï‹ÚË ¤ÏÂÁ¯Ô Ù˘ ÛÙ‹ÚÈ͢ Ù˘ ÎÂÊ·Ï‹˜ Î·È Ù˘ ÂχıÂÚ˘ ΛÓËÛ˘ ÙÔ˘ ·˘¯¤Ó·.

∂ȉËÌÈÔÏÔÁ›· ∏ Ï·ÁÈÔÎÂÊ·Ï›· ‰ÂÓ Â›Ó·È Û¿ÓÈ· ηٿÛÙ·ÛË ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi. ∏ Û˘¯ÓfiÙËÙ¿ Ù˘ Î˘Ì·›ÓÂÙ·È ·fi 1 ÂÚ›ÙˆÛË ·Ó¿ 300 ÁÂÓÓ‹ÛÂȘ (13) Êı¿ÓÔÓÙ·˜ Û ÔÛÔÛÙfi 48% ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô (14). ¶·Ú¿ÏÏËÏ·, ¤ˆ˜ 10% ÙˆÓ ‚ÚÂÊÒÓ Ì Ï·ÁÈÔÎÂÊ·Ï›· ı· ¤¯Ô˘Ó Î·È ÌÂÏÏÔÓÙÈο οÔÈÔ˘ ‚·ıÌÔ‡ ‰˘ÛÌÔÚÊ›· ÙÔ˘ ÎÚ·Ó›Ô˘, ÂÓÒ 14% ÙˆÓ ÂÓËÏ›ÎˆÓ ¤¯Ô˘Ó Ì ÌÔÚÊÔÌÂÙÚÈο ÎÚÈÙ‹ÚÈ· Ï·ÁÈÔÎÂÊ·Ï›·, ·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ‰˘ÛÌÔÚÊ›· Û˘Ó‹ıˆ˜ ‰ÂÓ ¤¯ÂÈ Á›ÓÂÈ ˘ÔÎÂÈÌÂÓÈο ·ÓÙÈÏËÙ‹ (15). ∏ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ï·ÁÈÔÎÂÊ·Ï›·˜ Â›Ó·È Û˘Ó¤ÂÈ· Â͈ÙÂÚÈ΋˜ Ì˯·ÓÈ΋˜ Û˘Ì›ÂÛ˘ ÙÔ˘ ÎÚ·Ó›Ô˘. ™˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›· ·Ó·Ê¤ÚÂÙ·È Ó· ˘¿Ú¯ÂÈ Û ÔÛÔÛÙfi ÌÈÎÚfiÙÂÚÔ ·fi 1% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÈÓȷ΋˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ (7,16).

¶ÚԉȷıÂÛÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ º‡ÏÔ: ∏ Ï·ÁÈÔÎÂÊ·Ï›· Â›Ó·È Û˘¯ÓfiÙÂÚË ÛÙ· ·ÁfiÚÈ· (Û ۯ¤ÛË 1,5:1 ¤ˆ˜ 3:1 ÚÔ˜ Ù· ÎÔÚ›ÙÛÈ·). ∏ ‰È·ÊÔÚ¿ ·˘Ù‹ ¤¯ÂÈ ·Ô‰Ôı› ÛÙÔ ÌÂÁ·Ï‡ÙÂÚÔ Ì¤ÁÂıÔ˜ ÙÔ˘ ÎÚ·Ó›Ô˘ ÛÙ· ·ÁfiÚÈ· Î·È Û Èı·Ó‹ ÌÂȈ̤ÓË ÂÓ‰ÔÌ‹ÙÚÈ· ÎÈÓËÙÈÎfiÙËÙ· ÙˆÓ ·ÚÚ¤ÓˆÓ ÂÌ‚Ú‡ˆÓ (2,7,8,10,12,17). ¢›‰˘ÌË Î‡ËÛË: ™Â ‰›‰˘ÌË Î‡ËÛË Ë Èı·ÓfiÙËÙ· ÂÓfi˜ ·fi Ù· ‰‡Ô ¤Ì‚Ú˘· Ó· ·Ó·Ù‡ÍÂÈ

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Ï·ÁÈÔÎÂÊ·Ï›· Â›Ó·È ·˘ÍË̤ÓË Î·È Êı¿ÓÂÈ ÙÔ 56% (2,18). √ ΛӉ˘ÓÔ˜ Â›Ó·È ·˘ÍË̤ÓÔ˜ ÁÈ· ÙÔ ¤Ì‚Ú˘Ô Ô˘ ‚Ú›ÛÎÂÙ·È ¯·ÌËÏfiÙÂÚ·, ηıÒ˜ ‰¤¯ÂÙ·È ÙËÓ ›ÂÛË ·fi ÙÔ ‚¿ÚÔ˜ ÙÔ˘ ˘ÂÚΛÌÂÓÔ˘ ÂÌ‚Ú‡Ô˘, ÂÓÒ Ù·˘Ùfi¯ÚÔÓ· ‚Ú›ÛÎÂÙ·È Û ¿ÌÂÛË Â·Ê‹ Ì ٷ ÔÛÙ¿ Ù˘ ÌËÙÚÈ΋˜ ˘¤ÏÔ˘. ¶ÚÔˆÚfiÙËÙ·: ∆· ÚfiˆÚ· ÓÂÔÁÓ¿, ηıÒ˜ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ÌÂȈ̤ÓÔ Ì˘˚Îfi ÙfiÓÔ Î·È ÎÈÓËÙÈÎfiÙËÙ· Î·È Â‡Ï·ÛÙ· ÔÛÙ¿ ÙÔ˘ ÎÚ·Ó›Ô˘, Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ÂÈÚÚÂ‹ ÛÙËÓ ÂÌÊ¿ÓÈÛË Ï·ÁÈÔÎÂÊ·Ï›·˜ (2,12). ∫ÂÊ·Ï·È̿و̷: ∂¿Ó ÙÔ ‚Ú¤ÊÔ˜ Ì ÙËÓ ÂÓÙÔÈṲ̂ÓË Â›Â‰Ë ÂÚÈÔ¯‹ ÛÙÔ ÎÚ·Ó›Ô Î·Ù¿ ÙË Á¤ÓÓËÛË ·Ó·Ù‡ÍÂÈ Î·Ù¿ ÙÔÓ ÙÔÎÂÙfi ÎÂÊ·Ï·È̿و̷ ÛÙËÓ ÂÙÂÚfiÏ¢ÚË ¯ÒÚ· ÙÔ˘ ÎÚ·Ó›Ô˘, ÙfiÙ ˘¿Ú¯ÂÈ ÌÂÁ·Ï‡ÙÂÚË Èı·ÓfiÙËÙ· ÂÌÌÔÓ‹˜ Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ηıÒ˜ ˘¿Ú¯ÂÈ ·˘ÍË̤ÓË Èı·ÓfiÙËÙ· Ó· Û˘Ó¯›ÛÂÈ ÙÔ ‚Ú¤ÊÔ˜ Ó· ÎÔÈÌ¿Ù·È Ì Â·Ê‹ ÙÔ˘ ÂÈ‰ˆÌ¤ÓÔ˘ ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÎÚ·Ó›Ô˘ Ì ÙÔ ÎÚ‚¿ÙÈ (2).

£¤ÛË ‡ÓÔ˘ Î·È Ï·ÁÈÔÎÂÊ·Ï›· ∏ Â·Ó·Ï·Ì‚·ÓfiÌÂÓË ÙÔÔı¤ÙËÛË ÙÔ˘ ‚Ú¤ÊÔ˘˜ ÛÙË ı¤ÛË ‡ÓÔ˘ Ì Â·Ê‹ Ù˘ ›‰È·˜ ¿ÓÙ· ÏÂ˘Ú¿˜ Ù˘ ÎÂÊ·Ï‹˜ Ì ÙËÓ ÂÈÊ¿ÓÂÈ· ÙÔ˘ ÎÚ‚·ÙÈÔ‡, ̤ۈ Ù˘ ÛÙ·ıÂÚ‹˜ Î·È Ì·ÎÚÔ¯ÚfiÓÈ·˜ Ì˯·ÓÈ΋˜ Û˘Ì›ÂÛ˘ Ô˘ ·ÛΛٷÈ, Ô‰ËÁ› Û ÂÈ¤‰ˆÛË Ù˘ ÂÈÊ¿ÓÂÈ·˜ Â·Ê‹˜ ÙÔ˘ ÎÚ·Ó›Ô˘ Î·È ÛÙËÓ ÂÌÊ¿ÓÈÛË Ï·ÁÈÔÎÂÊ·Ï›·˜ ‹ ÛÙËÓ Âȉ›ӈÛË ÚÔ¸¿Ú¯Ô˘Û·˜ ÌË Û˘ÓÔÛÙˆÙÈ΋˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ (8). ∏ Û˘¯ÓfiÙËÙ· Î·È Ë ÂÓÙfiÈÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ Ô˘ ηٷÁÚ¿ÊÂÙ·È ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· Û¯ÂÙ›˙ÂÙ·È ¿ÌÂÛ· Ì ÙȘ Ô‰ËÁ›Â˜ ÁÈ· ÙË ÛˆÛÙ‹ ÙÔÔı¤ÙËÛË ÙˆÓ ‚ÚÂÊÒÓ. ∆Ô 1992 Ë ∞ÌÂÚÈηÓÈ΋ ¶·È‰È·ÙÚÈ΋ ∞η‰ËÌ›· ηıfiÚÈÛ ÙËÓ ‡ÙÈ· ı¤ÛË ‡ÓÔ˘ ˆ˜ ÙËÓ ÂӉ‰ÂÈÁ̤ÓË, Ì ÛÙfi¯Ô ÙË Ì›ˆÛË Ù˘ Â›ÙˆÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÙÔ˘ ·ÈÊÓ›‰ÈÔ˘ ‚ÚÂÊÈÎÔ‡ ı·Ó¿ÙÔ˘ (SIDS) (2,4,11). ∏ ‡ÙÈ· ı¤ÛË ‡ÓÔ˘ ·Ó·Ê¤ÚÂÙ·È fiÙÈ Â›Ó·È 6 ÊÔÚ¤˜ ÈÔ ·ÛÊ·Ï‹˜ ·fi ÙËÓ ÚËÓ‹ ı¤ÛË ‡ÓÔ˘ Î·È 2 ÊÔÚ¤˜ ÈÔ ·ÛÊ·Ï‹˜ ·fi ÙËÓ Ï¿ÁÈ· ı¤ÛË ‡ÓÔ˘ (8). H ˘ÈÔı¤ÙËÛË ÙˆÓ Ô‰ËÁÈÒÓ ·˘ÙÒÓ Ô‰‹ÁËÛÂ, ̤ۈ Ù˘ Ì›ˆÛ˘ Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ ÚËÓÔ‡˜ ı¤Û˘ ‡ÓÔ˘ ÙˆÓ ‚ÚÂÊÒÓ (·fi 70% Û 24% Û 4 ¤ÙË), ·Ú¿ÏÏËÏ· Ì ÙË Ì›ˆÛË Ù˘ Â›ÙˆÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ·ÈÊÓ›‰ÈÔ˘ ‚ÚÂÊÈÎÔ‡ ı·Ó¿ÙÔ˘, Û ÛËÌ·ÓÙÈ΋ (ÂÍ·Ï¿ÛÈ·) ·‡ÍËÛË Ù˘ Ô›ÛıÈ·˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ (8,11). ∆·˘Ùfi¯ÚÔÓ·, ·Ú·ÙËÚ‹ıËΠÛËÌ·ÓÙÈ΋ Ì›ˆÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ ÚfiÛıÈ·˜ Ï·ÁÈÔÎÂÊ·-

Ï›·˜ ı¤Û˘. ∏ ·‡ÍËÛË Ù˘ Ô›ÛıÈ·˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ ·Ô‰fiıËΠÛÙËÓ ‡ÙÈ· ı¤ÛË ‡ÓÔ˘ (19-21). µÚ¤ÊË Ô˘ ÙÔÔıÂÙÔ‡ÓÙ·È ÛÙËÓ ‡ÙÈ· ı¤ÛË Û˘ÁÎÚÈÙÈο Ì ÂÓ·ÏÏ·ÛÛfiÌÂÓË ı¤ÛË ‡ÓÔ˘, ¤¯Ô˘Ó ÂÙ·Ï¿ÛÈÔ Î›Ó‰˘ÓÔ (O.R.) ·Ó¿Ù˘Í˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ (95% CI: 2,7-18,3) (8). ™ÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ Â›ÎÙËÙ˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ ·fi Û˘Ì›ÂÛË ÙÔ˘ ÎÚ·Ó›Ô˘ ÌÂÙ¿ ÙÔÓ ÙÔÎÂÙfi, ÂÎÙfi˜ ·fi ÙËÓ ÙÔÔı¤ÙËÛË ÙÔ˘ ‚Ú¤ÊÔ˘˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ Û˘Ó¯Ҙ ÛÙËÓ ›‰È· ÏÂ˘Ú¿, ȉȷ›ÙÂÚÔ ÂӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ fiÙÈ Î·È Ù· ›‰È· Ù· ‚Ú¤ÊË ÌÔÚ› Ó· ÂÌÊ·Ó›˙Ô˘Ó ÚÔÙ›ÌËÛË ÛÙËÓ ÙÔÔı¤ÙËÛË Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘˜ ÛÙËÓ ›‰È· ¿ÓÙÔÙ ÏÂ˘Ú¿. ∏ ÚÔÙ›ÌËÛË ·˘Ù‹ ÂΠ̤ÚÔ˘˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È Â›Ù ÛÂ Û˘Ó‡·ÚÍË ¿ÏÏˆÓ ÔÚıÔ‰ÈÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ (.¯. Û˘ÁÁÂÓ¤˜ Ú·È‚fiÎÚ·ÓÔ ·fi ›ÓˆÌ· ‹ ‚Ú¿¯˘ÓÛË ÙÔ˘ ÛÙÂÚÓÔÎÏÂȉÔÌ·ÛÙÔÂȉԇ˜, ȉÈÔ·ı‹ ÓÂÔÁÓÈ΋ ÛÎÔÏ›ˆÛË (22), Û˘ÁÁÂÓ‹ ·ÓˆÌ·Ï›· ÙˆÓ ÛÔÓ‰‡ÏˆÓ Ù˘ ·˘¯ÂÓÈ΋˜ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘), ·ÏÏ¿ Î·È ÛÙË Û˘Ó‹ıÂÈ· ÙˆÓ ÁÔÓ¤ˆÓ Ó· ÚÔÛʤÚÔ˘Ó ÙÔ Á‡̷ ÛÙÔ ‚Ú¤ÊÔ˜ ¿ÓÙ· ·fi ÙËÓ ›‰È· ÏÂ˘Ú¿, ‹ ÛÙËÓ ÂÙÂÚfiÏ¢ÚË ·ÚÔ˘Û›· ·ÈÛıËÙËÚÈ·ÎÒÓ ÂÚÂıÈÛÌ¿ÙˆÓ (.¯. Ù˘ fiÚÙ·˜ ÙÔ˘ ‰ˆÌ·Ù›Ô˘, ‰È·Ì¤ÛÔ˘ Ù˘ ÔÔ›·˜ ‚ϤÂÈ ÙÔ˘˜ ÁÔÓ›˜ ÙÔ˘ Î.Ï.). ∞ӷʤÚÂÙ·È fiÙÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ 8% ÙˆÓ ‚ÚÂÊÒÓ ¤¯ÂÈ Î¿ÔÈ· ÚÔÙ›ÌËÛË ÙÔÔı¤ÙËÛ˘ Ù˘ ÎÂÊ·Ï‹˜, ÂÎ ÙˆÓ ÔÔ›ˆÓ 47% ¤¯ÂÈ Î¿ÔÈÔ˘ ‚·ıÌÔ‡ ÂÈ¤‰ˆÛË ÙÔ˘ ÈÓÈ·ÎÔ‡ ÎÚ·Ó›Ô˘ (12).

¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ∏ ·Û‡ÌÌÂÙÚË ÂÈ¤‰ˆÛË ÙÔ˘ ÎÚ·Ó›Ô˘, Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙËÓ Ï·ÁÈÔÎÂÊ·Ï›·, ‰ÂÓ Ú¤ÂÈ Ó· Û˘Á¯¤ÂÙ·È ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ Ê˘ÏÂÙÈΤ˜ ·Ú·ÏÏ·Á¤˜ ÙÔ˘ Û¯‹Ì·ÙÔ˜ ÙÔ˘ ÎÚ·Ó›Ô˘. ŒÙÛÈ, Ë ‰ÔÏȯÔÎÂÊ·Ï›· Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙËÓ ÈÓ‰ÔÂ˘Úˆ·˚΋ Ê˘Ï‹, ‹ Ë Û˘ÌÌÂÙÚÈ΋ ÂÈ¤‰ˆÛË Ù˘ ÈÓȷ΋˜ ÂÚÈÔ¯‹˜ Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙËÓ ÈÏÏ˘ÚÈ΋ Ê˘Ï‹, (23) ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ·Ô˘Û›· ÂÓÙÔÈṲ̂Ó˘ ·Û˘ÌÌÂÙÚ›·˜ ÙÔ˘ Û¯‹Ì·ÙÔ˜ ÙÔ˘ ÎÚ·Ó›Ô˘ Î·È Ú¤ÂÈ Ó· ‰È·ÎÚ›ÓÔÓÙ·È ·fi ÙËÓ Ï·ÁÈÔÎÂÊ·Ï›·. ∏ ‰È¿ÎÚÈÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ Û Ï·ÁÈÔÎÂÊ·Ï›· ·fi Â͈ÙÂÚÈ΋ Û˘Ì›ÂÛË Î·È ÛÂ Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›· ‹ Û‡Ó‰ÚÔÌ· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ·˘Ù‹Ó (24-26), Â›Ó·È È‰È·›ÙÂÚ· ÛËÌ·ÓÙÈ΋. ™ÙËÓ Ù˘È΋ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘, ÙÔ ÈÛÙÔÚÈÎfi Î·È Ë ÂÈÛÎfiËÛË ÙÔ˘ ÎÚ·Ó›Ô˘ ı¤ÙÔ˘Ó ÙË ‰È¿ÁÓˆÛË: ÙÔ ‚Ú¤ÊÔ˜, ÂÓÒ ÂÚÈÁÚ¿ÊÂÙ·È Ó· ¶·È‰È·ÙÚÈ΋ 2006;69:233-241

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∞. ºÈÏÈ›‰Ë Î·È Û˘Ó.

¤¯ÂÈ Ù˘Èο Û˘ÌÌÂÙÚÈÎfi ÎÂÊ¿ÏÈ Î·Ù¿ ÙË Á¤ÓÓËÛË, ·ÔÎÙ¿ ÈÓȷ΋ ÂÈ¤‰ˆÛË Î·È ·Ú·ÏÏËÏfiÁÚ·ÌÌÔ Û¯‹Ì· ÎÚ·Ó›Ô˘ ÌÂÚÈΤ˜ ‚‰ÔÌ¿‰Â˜ ‹ Ì‹Ó˜ ·ÚÁfiÙÂÚ·. ∞Ó Î·Ù¿ ÙË Á¤ÓÓËÛË ‰È·ÈÛÙˆı› ÈÓȷ΋ ÂÈ¤‰ˆÛË ÙÔ˘ ÎÚ·Ó›Ô˘, ÂÎÙfi˜ ·fi ÙËÓ Èı·ÓfiÙËÙ· ÂÓ‰ÔÌ‹ÙÚÈ·˜ Û˘Ì›ÂÛ˘, Ú¤ÂÈ Ó· ·ÔÎÏÂÈÛı› Ë Èı·ÓfiÙËÙ· Û˘ÓÔÛÙ¤ˆÛ˘ Ù˘ Ï·Ì‚‰ÔÂȉԇ˜ Ú·Ê‹˜ (27). ™ÙË ‰È¿ÎÚÈÛË Ù˘ Û˘ÓÂÔÛÙˆÙÈ΋˜ ·fi ÙË ÌË Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›· Ë ÂÓÙfiÈÛË Ù˘ ÂÈ¤‰ˆÛ˘ ÙÔ˘ ÎÚ·Ó›Ô˘ Â›Ó·È ÛËÌ·ÓÙÈ΋: ÂÓÒ Ë Ô›ÛıÈ· Ï·ÁÈÔÎÂÊ·Ï›· Û¿ÓÈ· Â›Ó·È Û˘ÓÔÛÙˆÙÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜, ÔÛÔÛÙfi ¤ˆ˜ 80% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÚfiÛıÈ·˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ ÂÌÊ·Ó›˙Ô˘Ó Û˘ÓÔÛÙ¤ˆÛË Ù˘ ÛÙÂÊ·ÓÈ·›·˜ Ú·Ê‹˜ (28). π‰È·›ÙÂÚ· ÛËÌ·ÓÙÈÎfi˜ Â›Ó·È Ô ·ÔÎÏÂÈÛÌfi˜ Ù˘ ·ÚÔ˘Û›·˜ Û˘ÓÔ‰ÒÓ ÔÚıÔ‰ÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ, Ô˘ Û˘¯Ó¿ Û˘Ó˘¿Ú¯Ô˘Ó Ì Ï·ÁÈÔÎÂÊ·Ï›·. ∏ Û˘Ó‡·ÚÍË Ï·ÁÈÔÎÂÊ·Ï›·˜ Î·È Ú·È‚fiÎÚ·ÓÔ˘ ·Ó·Ê¤ÚÂÙ·È fiÙÈ Î˘Ì·›ÓÂÙ·È ·fi 25% (29,30) ¤ˆ˜ Î·È 84% (14). ∏ Ï·ÁÈÔÎÂÊ·Ï›· ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ڷȂfiÎÚ·ÓÔ, ˆ˜ Û˘Ó¤ÂÈ· ‚Ú¿¯˘ÓÛ˘ ÙÔ˘ ÛÙÂÚÓÔÎÏÂȉÔÌ·ÛÙÔÂȉԇ˜ Ì˘fi˜, ÏfiÁˆ Ù˘ Û˘Ó¯ԇ˜ ÂÙÂÚfiÏ¢Ú˘ ÙÔÔı¤ÙËÛ˘ Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜ Î·È Ù˘ ÂÚÈÔÚÈṲ̂Ó˘ ΛÓËÛ˘ ÙÔ˘ ·˘¯¤Ó· (11), ·ÏÏ¿ Î·È ·ÓÙ›ÛÙÚÔÊ·, Ë ·ÚÔ˘Û›· Ú·È‚fiÎÚ·ÓÔ˘ Ô‰ËÁ› Û ÂÈÏÂÎÙÈ΋ ı¤ÛË ‡ÓÔ˘ Î·È Ï·ÁÈÔÎÂÊ·Ï›· (31). ™Â Û˘Ó‡·ÚÍË Ú·È‚fiÎÚ·ÓÔ˘ Ô ·ÔÎÏÂÈÛÌfi˜ ÛÔ‚·Ú‹˜ ·Ó·ÙÔÌÈ΋˜ ·ÓˆÌ·Ï›·˜ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Â›Ó·È ÛËÌ·ÓÙÈÎfi˜ (32,33). ™Â οı ÂÚ›ÙˆÛË Ï·ÁÈÔÎÂÊ·Ï›·˜ Ú¤ÂÈ Ù¤ÏÔ˜, Ó· Á›ÓÂÙ·È Û˘ÛÙËÌ·ÙÈ΋ ·Ó·˙‹ÙËÛË ÁÈ· Û˘Ó˘¿Ú¯Ô˘Û˜ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ Ì˘ÔÛÎÂÏÂÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, fiˆ˜ ‰˘ÛÏ·Û›· ÈÛ¯›ˆÓ, ˘ÔÏ·Û›· οو ÁÓ¿ıÔ˘, ·ÚÔ˘Û›· ·ÓˆÌ·ÏÈÒÓ ÙˆÓ ·˘¯ÂÓÈÎÒÓ ÛÔÓ‰‡ÏˆÓ ‹ Ù˘ ÈÓÈÔ-·˘¯ÂÓÈ΋˜ ÂÚÈÔ¯‹˜, ȉÈÔ·ıÔ‡˜ ÓËȷ΋˜ ÛÎÔÏ›ˆÛ˘ Î.Ï. (2,15,22,32,34,35).

∏ ÎÏÈÓÈ΋ ‰È¿ÁÓˆÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ÛÙËÚ›˙ÂÙ·È ÛÙËÓ ÂÈÛÎfiËÛË ÙÔ˘ ‚ÚÂÊÈÎÔ‡ ÎÚ·Ó›Ô˘ ·fi ÙËÓ ÎÔÚ˘Ê‹ Ù˘ ÎÂÊ·Ï‹˜ (birds eye view), ηٿ ÙËÓ ÔÔ›· ·ÍÈÔÏÔÁÂ›Ù·È Ë Û˘ÌÌÂÙÚ›· Ù˘ ı¤Û˘ ÙˆÓ ‰‡Ô ËÌÈÌÔÚ›ˆÓ ÙÔ˘ ÎÚ·Ó›Ô˘, ÙˆÓ ÙÂÚ˘Á›ˆÓ ÙˆÓ ·˘ÙÈÒÓ Î·È ÙˆÓ ˙˘ÁˆÌ·ÙÈÎÒÓ ÔÛÙÒÓ Û ۯ¤ÛË Ì ԂÂÏÈ·›· ÓÔËÙ‹ ÁÚ·ÌÌ‹, Ô˘ ÂÓÒÓÂÈ ÙÔ ÚÈ˙ÔÚ›ÓÈÔ Ì ÙËÓ ÈÓȷ΋ ÚÔ‚ÔÏ‹ ÙÔ˘ ÎÚ·Ó›Ô˘ (∂ÈÎfiÓ· 1). ™ÙËÓ Ù˘È΋ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ ·Ú·ÙËÚÂ›Ù·È ·Ú·ÏÏËÏfiÁÚ·ÌÌÔ Û¯‹Ì· ÎÂÊ·Ï‹˜, ηıÒ˜ Ë Û˘Ì›ÂÛË Î·È ÂÈ¤‰ˆÛË ÙÔ˘ (ÈÓÈ·ÎÔ‡ Û˘Ó‹ıˆ˜) ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÎÚ·Ó›Ô˘ ¤¯ÂÈ ˆ˜ Û˘Ó·ÎfiÏÔ˘ıÔ ·ÔÙ¤ÏÂÛÌ· ÙË (ÌÂÙˆÈ·›· Û˘Ó‹ıˆ˜) ÚÔ‚ÔÏ‹ ÙˆÓ ‰ÔÌÒÓ ÙÔ˘ Û‡ÛÙÔÈ¯Ô˘ ËÌÈÌÔÚ›Ô˘ ÙÔ˘ ÎÚ·Ó›Ô˘. ŒÙÛÈ, Û˘Ó‹ı˘ Â›Ó·È Ë ÂÙÂÚfiÏ¢ÚË ÂÈ¤‰ˆÛË Ù˘ ÈÓȷ΋˜ ÂÚÈÔ¯‹˜, Ë ÔÔ›· ÌÔÚ› Ó· Û˘Ó‰˘¿˙ÂÙ·È Ì ÚÔ‚ÔÏ‹ Ù˘ ÌÂÙˆÈ·›·˜ ¯ÒÚ·˜ Û‡ÛÙÔȯ·, ˘ÂÚÔ¯‹ ˙˘ÁˆÌ·ÙÈÎÔ‡ ÛÙËÓ ›‰È· ÏÂ˘Ú¿, ηıÒ˜ Î·È ÚfiÛıÈ· ·ÚÂÎÙfiÈÛË ÙÔ˘ Û‡ÛÙÔÈ¯Ô˘ ˆÙÈÎÔ‡ ÙÂÚ˘Á›Ô˘ (9,11), (∂ÈÎfiÓ· 2,3). ™ÙËÓ ·Û˘ÌÌÂÙÚ›· ÎÚ·Ó›Ô˘ ÏfiÁˆ Û˘ÓÔÛÙ¤ˆÛ˘ Ú·ÊÒÓ (΢ڛˆ˜ Ï·Ì‚‰ÔÂȉԇ˜ Ú·Ê‹˜), ÙÔ ÎÚ·Ó›Ô ¤¯ÂÈ ÙÚ·Â˙ÔÂȉ¤˜ Û¯‹Ì·, ÙÔ ÔÔ›Ô ÔÊ›ÏÂÙ·È ÛÙËÓ ÈÓȷ΋ ÂÈ¤‰ˆÛË Î·È ÙËÓ ÂÙÂÚfiÏ¢ÚË ÚÔ‚ÔÏ‹ Ù˘ ‚ÚÂÁÌ·ÙÈ΋˜ Î·È Ù˘ ÌÂÙˆÈ·›·˜ ¯ÒÚ·˜ (·ÓÙ›ıÂÙ· ·fi ÙËÓ ÔÌfiÏ¢ÚË ÌÂÙˆÈ·›· ÚÔ‚ÔÏ‹ Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘) (27). ∂›Û˘, ÙÔ Û‡ÛÙÔÈ¯Ô ˆÙÈÎfi ÙÂÚ‡ÁÈÔ Ì ÙËÓ ÈÓȷ΋ ÂÈ¤‰ˆÛË ÌÂÙ·ÙÔ›˙ÂÙ·È ÚÔ˜ Ù· ¤Ûˆ Î·È ›Ûˆ, ·ÓÙ›ıÂÙ· Ì ÙËÓ ÚfiÛıÈ· ÚÔ‚ÔÏ‹ ÙÔ˘ ÛÙËÓ Ï·ÁÈÔÎÂÊ·Ï›·

∫ÏÈÓÈ΋ ÂͤٷÛË H ·ÚÔ˘Û›· Ï·ÁÈÔÎÂÊ·Ï›·˜ ÌÔÚ› Ó· ‰È·ÁÓˆÛı› ÛÙËÓ ÂͤٷÛË ÙÔ˘ ÓÂÔÁÓÔ‡ ηٿ ÙË Á¤ÓÓËÛË ‹, ·ÚÁfiÙÂÚ·, ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ù·ÎÙÈ΋˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ·fi ÙÔÓ ·È‰›·ÙÚÔ ÛÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜. ™ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË, ÂÎÙfi˜ ·fi ÙËÓ ÂͤٷÛË ÙÔ˘ ÎÚ·Ó›Ô˘, ‰ÂÓ Ú¤ÂÈ Ó· ·Ú·Ï›ÂÙ·È Ë ÏÂÙÔÌÂÚ‹˜ ÂͤٷÛË fiÏÔ˘ ÙÔ˘ Ì˘ÔÛÎÂÏÂÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÁÈ· ·Ó·ÁÓÒÚÈÛË Û˘Ó˘·Ú¯fiÓÙˆÓ ÔÚıÔ‰ÈÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ. ¶·È‰È·ÙÚÈ΋ 2006;69:233-241

∂ÈÎfiÓ· 1. ∫ÏÈÓÈ΋ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ ·fi Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›·. ™ÙËÓ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Ûˆ˜ (·ÚÈÛÙÂÚ¿) ·Ú·ÙËÚÂ›Ù·È ÚfiÛıÈ· ·ÚÂÎÙfiÈÛË ÙÔ˘ ˆÙfi˜ Î·È ÚÔ‚ÔÏ‹ Ù˘ ÌÂÙˆÈ·›·˜ ¯ÒÚ·˜ Û‡ÛÙÔȯ· Ì ÙËÓ ÈÓȷ΋ ÂÈ¤‰ˆÛË. ™ÙË Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›· (‰ÂÍÈ¿) ˘¿Ú¯ÂÈ Ô›ÛıÈ· ·ÚÂÎÙfiÈÛË ÙÔ˘ ˆÙfi˜ Û‡ÛÙÔȯ· Ì ÙËÓ ÈÓȷ΋ ÂÈ¤‰ˆÛË, ÂÓÒ Ë ‰˘ÛÌÔÚÊ›· Ù˘ ÌÂÙˆÈ·›·˜ ¯ÒÚ·˜ ‰ÂÓ Â›Ó·È ÂÎÛÂÛËÌ·Ṳ̂ÓË.

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·237

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¶Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘

™ÙË Û˘Ó¤¯ÂÈ·, ÌÈ· ·‰Ú‹ ÂÈÛÎfiËÛË ÙÔ˘ ÚÔÛÒÔ˘ Î·È ÙÔ˘ ÙÚ·¯‹ÏÔ˘ ÌÔÚ› Ó· ÂÓÙÔ›ÛÂÈ ÂÚ·ÈÙ¤Úˆ ÔÚıÔ‰ÈΤ˜ ·ÓˆÌ·Ï›Â˜ Ô˘ Û˘¯Ó¿ Û˘Ó˘¿Ú¯Ô˘Ó. ∆Ô Â‡ÚÔ˜ ÛÙÚÔÊÈ΋˜ ΛÓËÛ˘ Ù˘ ÎÂÊ·Ï‹˜ Ô˘ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ÎÈÓËÙÈÎfiÙËÙ· ÙÔ˘ ·˘¯¤Ó· ·ÍÈÔÏÔÁÂ›Ù·È ÁÈ· ÙÔÓ ·ÔÎÏÂÈÛÌfi Ù˘ ·ÚÔ˘Û›·˜ Ú·È‚fiÎÚ·ÓÔ˘. ™Â Û˘Ó‡·ÚÍË Ú·È‚fiÎÚ·ÓÔ˘ Î·È Ï·ÁÈÔÎÂÊ·Ï›·˜, ÙÔ ‚Ú¤ÊÔ˜ ÂÌÊ·Ó›˙ÂÈ Û˘¯Ó¿ ÌÂȈ̤ÓË ÈηÓfiÙËÙ· ÛÙÚÔÊ‹˜ Ù˘ ÎÂÊ·Ï‹˜ Û ·ÓÙ›ıÂÙË Î·Ù‡ı˘ÓÛË ·fi ÙÔ ÂÈ‰ˆÌ¤ÓÔ ÈÓÈ·Îfi ÙÌ‹Ì·. ™˘ÁÎÂÎÚÈ̤ӷ, Ô ÂÍÂÙ·ÛÙ‹˜ οıÂÙ·È Û ÂÚÈÛÙÚÂÊfiÌÂÓË Î·Ú¤ÎÏ· Ì ÙÔ ‚Ú¤ÊÔ˜ ÛÙËÓ ·ÁηÏÈ¿ ÙÔ˘. ∆Ô ‚Ú¤ÊÔ˜ ‚Ú›ÛÎÂÙ·È Ì ÙÔ ÚfiÛˆÔ ÛÙÚ·Ì̤ÓÔ ÚÔ˜ ÙË ÌËÙ¤Ú· ÙÔ˘, Ë ÔÔ›· ÚÔÛ·ı› Ó· ‰È·ÙËÚ‹ÛÂÈ ÙÔ ÂӉȷʤÚÔÓ ÙÔ˘ ·È‰ÈÔ‡ ÁÈ· ÔÙÈ΋ Â·Ê‹ Ì·˙› ÙÔ˘, ÂÓÒ Ô ÂÍÂÙ·ÛÙ‹˜ ÂÚÈÛÙÚ¤ÊÂÙ·È ÛÙËÓ Î·Ú¤ÎÏ· ÙÔ˘ Î·È Î·Ù·ÁÚ¿ÊÂÈ ÙȘ ÎÈÓ‹ÛÂȘ Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜. ∆Ô ›‰ÈÔ ÌÔÚ› Ó· Á›ÓÂÈ Î·È ·Ó ÙÔ ‚Ú¤ÊÔ˜ Â›Ó·È ÛÙËÓ ·ÁηÏÈ¿ Ù˘ ÌËÙ¤Ú·˜ ÙÔ˘ Î·È Ô ÂÍÂÙ·ÛÙ‹˜ ÙÔ˘ ÂȉÂÈÎÓ‡ÂÈ ·ÓÙÈΛÌÂÓ· Ô˘ ÎÈÓÔ‡Ó ÙÔ ÂӉȷʤÚÔÓ ÙÔ˘ ‚Ú¤ÊÔ˘˜.

¶·Ú·ÎÏÈÓÈΤ˜ ÂÍÂÙ¿ÛÂȘ

∂ÈÎfiÓ· 2 Î·È 3. ∂ÈÎfiÓ· ‚Ú¤ÊÔ˘˜ Ì ‹È· Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘. ¶·Ú·ÙËÚÂ›Ù·È ·ÚÈÛÙÂÚ‹ ÈÓȷ΋ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘, ‹ÈÔ˘ ‚·ıÌÔ‡. ∂ÌÊ·Ó‹˜ ·ÚÂÎÙfiÈÛË ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ˆÙfi˜ ÚÔ˜ Ù· ÂÌÚfi˜, Û‡ÛÙÔȯ· Ì ÙËÓ ÈÓȷ΋ ÂÈ¤‰ˆÛË.

ı¤Û˘ (9), ÂÓÒ ÌÔÚ› Ó· Á›ÓÂÈ ·ÓÙÈÏËÙ‹, „ËÏ·ÊËÙ¿, ÂÊ›¢ÛË Ú·ÊÒÓ (∂ÈÎfiÓ· 1). ∏ ÂÈÛÎfiËÛË ÙÔ˘ ÙÚȯˆÙÔ‡ Ù˘ ÎÂÊ·Ï‹˜ ÚÔÛʤÚÂÈ ·ÍÈfiÏÔÁ˜ Û˘ÌÏËڈ̷ÙÈΤ˜ ÏËÚÔÊÔڛ˜, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Ë Û˘ÌÌÂÙÚÈ΋ ·ÒÏÂÈ· ÙˆÓ ÙÚȯÒÓ Ù˘ ÎÂÊ·Ï‹˜ ·ÔÙÂÏ› ¤Ó‰ÂÈÍË Î·Ï‹˜ ÛÙÚÔÊ‹˜ Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ÚÔ˜ fiϘ ÙȘ ηÙ¢ı‡ÓÛÂȘ, Û ·ÓÙ›ıÂÛË Ì ÙËÓ ÂÓÙÔÈṲ̂ÓË ·ÏˆÂΛ·, Û‡ÛÙÔȯ· Ì ÙËÓ ÂÚÈÔ¯‹ ÂÈ¤‰ˆÛ˘ ÙÔ˘ ÎÚ·Ó›Ô˘, Û ÂÚÈÙÒÛÂȘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ (8,11).

∆Ô Â›‰Ô˜ Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ηıÔÚ›˙ÂÙ·È, ·‰Ú¿, Ì ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË Î·È ÙÔ Ù˘ÈÎfi ÈÛÙÔÚÈÎfi ÙÔ˘ ¯ÚfiÓÔ˘ ÂÌÊ¿ÓÈÛ˘ Ù˘ ·Û˘ÌÌÂÙÚ›·˜. ™Â Ù˘ÈΤ˜ ÂÚÈÙÒÛÂȘ ‰ÂÓ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ÂÚ·ÈÙ¤Úˆ ÂϤÁ¯Ô˘ (11). ™Â ·ÚÔ˘Û›· ÛÔ‚·Ú‹˜ ·Û˘ÌÌÂÙÚ›·˜, ·ÌÊ›‚ÔÏˆÓ ÎÏÈÓÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ ‹ Âȉ›ӈÛ˘ Ù˘ ·Û˘ÌÌÂÙÚ›·˜, ·Ú¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙˆÓ Ô‰ËÁÈÒÓ ÁÈ· ÛˆÛÙ‹ ÙÔÔı¤ÙËÛË ÙÔ˘ ‚Ú¤ÊÔ˘˜, ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ÂÚ·ÈÙ¤Úˆ ÂϤÁ¯Ô˘. ∏ ÎÏ·ÛÈ΋ ̤ıÔ‰Ô˜ ‰È¿ÁÓˆÛ˘ Ù˘ Û˘ÓÔÛÙˆÙÈ΋˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ Â›Ó·È Ë ·ÎÙÈÓÔÁÚ·Ê›· ÙÔ˘ ÎÚ·Ó›Ô˘. ∏ ÂÍ¿ÏÂÈ„Ë Ù˘ ‰È·‡Á·Û˘ Ù˘ Ú·Ê‹˜ ıˆÚÂ›Ù·È ‰È·ÁÓˆÛÙÈ΋, ÂÓÒ ·ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· fiˆ˜ Ë ‡ÎÓˆÛË ÙÔ˘ ÔÛÙÔ‡ Âη٤ڈıÂÓ Ù˘ Ú·Ê‹˜ ‹ ÙÔ ÌÂȈ̤ÓÔ Â‡ÚÔ˜ Ù˘ Ú·Ê‹˜ Ô˘ ÂÚȤÁÚ·Ê·Ó ÙËÓ Î·Ù¿ÛÙ·ÛË Ù˘ ÏÂÈÙÔ˘ÚÁÈ΋˜ Û˘ÓÔÛÙ¤ˆÛ˘, ‰ÂÓ ıˆÚÔ‡ÓÙ·È ϤÔÓ ·ÍÈfiÈÛÙ· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· (11). ∂¿Ó Ô ·Ú¯ÈÎfi˜ ·ÎÙÈÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‰ÂÓ Â›Ó·È ‰È·ÁÓˆÛÙÈÎfi˜, ÙfiÙ ·ÎÔÏÔ˘ı› ¤ÏÂÁ¯Ô˜ Ì ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÙˆÓ ÔÛÙÒÓ ÙÔ˘ ÎÚ·Ó›Ô˘ Î·È Ï‹„Ë ÙÚÈۉȿÛÙ·ÙˆÓ ·ÂÈÎÔÓ›ÛÂˆÓ (36,37). ∏ ̤ıÔ‰Ô˜ Â›Ó·È Â˘·›ÛıËÙË ÛÙË ‰È¿ÁÓˆÛË Ù˘ Û˘ÓÔÛÙ¤ˆÛ˘ Î·È ÛÙËÓ ·ÂÈÎfiÓÈÛË ÙÔ˘ ÌÂȈ̤ÓÔ˘ fiÁÎÔ˘ Ù˘ Ô›ÛıÈ·˜ ÎÚ·Óȷ΋˜ ÎÔÈÏfiÙËÙ·˜, Û‡ÛÙÔȯ· Ì ÙË Û˘ÓÔÛÙˆ̤ÓË ¶·È‰È·ÙÚÈ΋ 2006;69:233-241

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∞. ºÈÏÈ›‰Ë Î·È Û˘Ó.

Ú·Ê‹ (7,11), ·ÏÏ¿ Ë ·˘ÍË̤ÓË ·ÎÙÈÓÔ‚ÔÏ›· ÂÚÈÔÚ›˙ÂÈ ÙË ¯Ú‹ÛË Ù˘ Û ÌÂÌÔӈ̤Ó˜ ÂÚÈÙÒÛÂȘ Ì ˘„ËÏ‹ ÎÏÈÓÈ΋ Èı·ÓfiÙËÙ· ·ÚÔ˘Û›·˜ Û˘ÓÔÛÙ¤ˆÛ˘. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÔÛÙÒÓ ÎÚ·Ó›Ô˘ Ì ÙË ¯Ú‹ÛË ˘„ËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ˯ԂÔϤˆÓ, Û˘ÁÎÚÈÙÈο Ì ÙËÓ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›·, ¤¯ÂÈ Â˘·ÈÛıËÛ›· 100% Î·È ÂȉÈÎfiÙËÙ· 89%, ¯ˆÚ›˜ Ó· ÂÎı¤ÙÂÈ ÙÔ ‚Ú¤ÊÔ˜ Û ·ÎÙÈÓÔ‚ÔÏ›· Î·È ÂӉ¯Ô̤ӈ˜ Û ΤÓÙÚ· Ì ÂÌÂÈÚ›· ÛÙË ¯Ú‹ÛË ÙÔ˘, ¤¯ÂÈ ı¤ÛË ÛÙÔÓ ·Ú¯ÈÎfi ·ÂÈÎÔÓÈÛÙÈÎfi ¤ÏÂÁ¯Ô (38,39).

∂ÈÏÔΤ˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ ∏ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ Â›Ó·È Î·ÏÔ‹ı˘ ηٿÛÙ·ÛË Î·È Ú¤ÂÈ Ó· ‰È·ÎÚ›ÓÂÙ·È ·fi ÙË Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›·, Ë ÔÔ›· Û˘¯Ó¿ Û˘Óԉ‡ÂÙ·È ·fi ÛÔ‚·Ú¤˜ ÂÈÏÔΤ˜, Â¿Ó ‰ÂÓ ·ÓÙÈÌÂÙˆÈÛı› ¤ÁηÈÚ·. √È Û¿ÓȘ ÂÈÏÔΤ˜ Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ Ù·ÍÈÓÔÌÔ‡ÓÙ·È ÛÂ: ·) ¢È·Ù·Ú·¯¤˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ‡ÙÈ· ı¤ÛË ‡ÓÔ˘ (Ë ÔÔ›· Ô‰ËÁ› Û Ï·ÁÈÔÎÂÊ·Ï›·). ∏ ‡ÙÈ· ı¤ÛË ‡ÓÔ˘ ¤¯ÂÈ Û˘Û¯ÂÙÈÛı› Ì ‹È· ηı˘ÛÙ¤ÚËÛË ÛÙËÓ ÂͤÏÈÍË Ù˘ ·‰Ú‹˜ ÎÈÓËÙÈÎfiÙËÙ·˜ ÙÔ˘ ÎÔÚÌÔ‡ (.¯. ÛÙËÓ ÈηÓfiÙËÙ· ÙˆÓ ‚ÚÂÊÒÓ Ó· Á˘ÚÓÔ‡Ó ·fi ÙËÓ ÚËÓ‹ ÛÙËÓ ‡ÙÈ· ı¤ÛË, ÏfiÁˆ ·ÓÂ·ÚÎÔ‡˜ ¿ÛÎËÛ˘ ÙˆÓ Ì˘ÒÓ Ù˘ ˆÌÈ΋˜ ˙ÒÓ˘), Ë ÔÔ›· fï˜, Â›Ó·È ·ÚÔ‰È΋ Î·È ¯ˆÚ›˜ Ì·ÎÚÔ¯ÚfiÓÈ· Â›ÙˆÛË (40). ‚) √Êı·ÏÌÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜: ™Ù· ·È‰È¿ Ì ÌÂÙˆÈ·›· Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ ¤¯ÂÈ ÂÚÈÁÚ·Ê› ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ·ÛÙÈÁÌ·ÙÈÛÌÔ‡, ÂÓÒ Ë Û˘Û¯¤ÙÈÛË ·˘Ù‹ ·ÌÊÈÛ‚ËÙÂ›Ù·È ÛÙËÓ Ù˘È΋ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ Ì ÂÓÙfiÈÛË ÛÙËÓ ÈÓȷ΋ ¯ÒÚ· (41,42). Á) √ÚıÔ‰ÔÓÙÈΤ˜ ‰È·Ù·Ú·¯¤˜: ∏ ÎÚ·Óȷ΋ ·Û˘ÌÌÂÙÚ›·, Â¿Ó Û˘Óԉ‡ÂÙ·È ·fi ·Û˘ÌÌÂÙÚ›· ÚÔÛÒÔ˘, ÌÔÚ› Ó· ‰ËÌÈÔ˘ÚÁ‹ÛÂÈ ÔÚıÔ‰ÔÓÙÈο ÚÔ‚Ï‹Ì·Ù· Î·È ·ÓˆÌ·Ï›· ÛÙËÓ ÎÚÔÙ·ÊÔÁÓ·ıÈ΋ ¿ÚıÚˆÛË (15). ‰) ¡Â˘ÚÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜: ¢ÂÓ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ¤ˆ˜ ÙÒÚ·, ÁÈ· ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ Ó¢ÚÔÏÔÁÈ΋˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ Û ·È‰È¿ Ì ÌË Û˘ÓÔÛÙˆÙÈ΋ Ï·ÁÈÔÎÂÊ·Ï›·, ·Ó Î·È ¤¯ÂÈ Á›ÓÂÈ ÏfiÁÔ˜ ÁÈ· Èı·Ó¿ Ì·ıËÛȷο ÚÔ‚Ï‹Ì·Ù· ‹ ‰˘ÛÎÔÏ›· Û˘ÁΤÓÙÚˆÛ˘. √È Miller Î·È Û˘Ó ·Ó·Ê¤ÚÔ˘Ó fiÙÈ ÙÔ 40% ÙˆÓ ·È‰ÈÒÓ Ì ÂÌ̤ÓÔ˘Û· Ï·ÁÈÔÎÂÊ·Ï›· ¯ÚÂÈ¿ÛÙËÎ·Ó ÂȉÈ΋ ‚Ô‹ıÂÈ· ÛÙÔ Û¯ÔÏÂ›Ô Î·È Û˘ÓÈÛÙÔ‡Ó Û ·È‰È¿ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (Ô˘ ÔÚ›˙Ô˘Ó ˆ˜ ¿ÚÚÂÓ· Ì ·ÓÒÌ·ÏÔ Û¯‹Ì· ÎÚ·Ó›Ô˘ ηٿ ÙË Á¤ÓÓËÛË) Ó· ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È ÛÙÂÓ¿ ÁÈ· Èı·Ó¤˜ ·Ó·Ù˘ÍÈ·¶·È‰È·ÙÚÈ΋ 2006;69:233-241

Τ˜ ‰È·Ù·Ú·¯¤˜ Î·È Ù˘¯fiÓ Ì·ıËÛȷΤ˜ ‰˘ÛÎÔϛ˜ ηٿ ÙË Û¯ÔÏÈ΋ ËÏÈΛ· (1). Â) ∂Ì̤ÓÔ˘Û· ‰˘ÛÌÔÚÊ›· ÎÚ·Ó›Ô˘: ∏ ÏÂÈÔ„ËÊ›· ÙˆÓ ·È‰ÈÒÓ ı· ¤¯ÂÈ ·Û‹Ì·ÓÙË ‰˘ÛÌÔÚÊ›· Ë ÔÔ›· ‰ÂÓ Â›Ó·È ÂÌÊ·Ó‹˜ ÏfiÁˆ Ù˘ Î¿Ï˘„‹˜ Ù˘ ·fi ÙÔ ÙÚ›¯ˆÌ· Ù˘ ÎÂÊ·Ï‹˜ Î·È ÌfiÓÔ 10% ı· ¤¯ÂÈ ÛÔ‚·ÚfiÙÂÚÔ˘ ‚·ıÌÔ‡ ·Û˘ÌÌÂÙÚ›· ÎÚ·Ó›Ô˘ (43).

∞Ú¯È΋ ·ÓÙÈÌÂÙÒÈÛË ∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÚfiÏË„Ë, ̤ۈ Ù˘ Û˘Ì‚Ô˘Ï¢ÙÈ΋˜ ·ÁˆÁ‹˜ ÛÙÔ˘˜ ÁÔÓ›˜, ÙËÓ ÂÓ·ÏÏ·Á‹ ı¤Ûˆ˜ ‡ÓÔ˘, ÙË Ê˘ÛÈÔıÂÚ·›·, Î·È Ù· Â͈ÙÂÚÈο ÔÚıÔ‰Èο ‚ÔËı‹Ì·Ù· (10). ¶ÚÔÏËÙÈο ̤ÙÚ·: ¶ÚÔÎÂÈ̤ÓÔ˘ Ó· ÚÔÏËÊı› Ù˘¯fiÓ ·Û˘ÌÌÂÙÚ›·, ÔÈ ÁÔÓ›˜ ı· Ú¤ÂÈ Ó· ÙÔÔıÂÙÔ‡Ó ÙÔ ‚Ú¤ÊÔ˜ Û ‡ÙÈ· ı¤ÛË ‡ÓÔ˘ ÌÂ Û˘¯Ó‹ ÂÓ·ÏÏ·Á‹ ı¤Ûˆ˜ (‰ÂÍÈ¿, ·ÚÈÛÙÂÚ¿). ∆Ô ‚Ú¤ÊÔ˜ ÌÔÚ› Ó· ÙÔÔıÂÙÂ›Ù·È Î·È ÁÈ· οÔÈÔ ‰È¿ÛÙËÌ· Û ÚËÓ‹ ı¤ÛË, ÂÊfiÛÔÓ Â›Ó·È Í‡ÓÈÔ Î·È ¿ÓÙ· ˘fi ÂÈÙ‹ÚËÛË. ∏ ÚÔÛÊÔÚ¿ ÙÚÔÊ‹˜ Î·È ·ÈÛıËÙËÚÈ·ÎÒÓ ÂÚÂıÈÛÌ¿ÙˆÓ Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÂÓ·ÏÏ¿Í Î·È ·fi ÙȘ ‰‡Ô Ï¢ڤ˜ Ù˘ ÎÏ›Ó˘. ∂›Û˘, Ú¤ÂÈ Ó· ·ÔʇÁÂÙ·È Ë ÙÔÔı¤ÙËÛË ÙˆÓ ‚ÚÂÊÒÓ ÁÈ· ·Ú·ÙÂٷ̤ÓÔ ‰È¿ÛÙËÌ· Û ηı›ÛÌ·Ù· Ô˘ Ù· ˘Ô¯ÚÂÒÓÔ˘Ó Ó· ÂÚÈÔÚ›˙ÔÓÙ·È ÛÂ Û˘ÁÎÂÎÚÈ̤ÓË ı¤ÛË (.¯. ·È‰Èο ηı›ÛÌ·Ù· ·˘ÙÔÎÈÓ‹ÙÔ˘). £ÂÚ·¢ÙÈΤ˜ Ô‰ËÁ›Â˜: √È ÁÔÓ›˜ ı· Ú¤ÂÈ Ó· ·ÔʇÁÔ˘Ó Ó· ÙÔÔıÂÙÔ‡Ó ÙÔ ‚Ú¤ÊÔ˜ Ì ÙËÓ ÂÈ‰ˆÌ¤ÓË ÂÚÈÔ¯‹ ÙÔ˘ ÎÚ·Ó›Ô˘ Û Â·Ê‹ Ì ÙÔ ÎÚ‚¿ÙÈ Î·È Ó· ÊÚÔÓÙ›˙Ô˘Ó Ó· ‰È·ÙËÚÔ‡Ó ÙËÓ ÂÈı˘ÌËÙ‹ ı¤ÛË Ì ̷ÍÈÏ¿ÚÈ· ‹ ‰Èψ̤ÓÔ ÛÂÓÙfiÓÈ, ÔfiÙ ӷ Â›Ó·È ϤÔÓ ‰‡ÛÎÔÏÔ ÙÔ ‚Ú¤ÊÔ˜ Ó· ·ÏÏ¿ÍÂÈ ÏÂ˘Ú¿ (11,28). ŸÛÔ ÓˆÚ›ÙÂÚ· Á›ÓÂÈ ¤Ó·ÚÍË Ù˘ ÂÊ·ÚÌÔÁ‹˜ ÙˆÓ ·ÏÒÓ ·˘ÙÒÓ Ì¤ÙÚˆÓ, ÙfiÛÔ Î·Ï‡ÙÂÚ· Â›Ó·È Ù· ıÂÚ·¢ÙÈο ·ÔÙÂϤÛÌ·Ù·. ™˘Ó‡·ÚÍË Ú·È‚fiÎÚ·ÓÔ˘: ∂¿Ó Û˘Ó˘¿Ú¯ÂÈ Ú·È‚fiÎÚ·ÓÔ, ÙÔ ÔÔ›Ô ÂÈÙ›ÓÂÈ ÙË ÛÙÚÔÊ‹ Ù˘ ÎÂÊ·Ï‹˜ ÚÔ˜ ÙËÓ ÂÈ‰ˆÌ¤ÓË ÂÚÈÔ¯‹ ÙÔ˘ ÎÚ·Ó›Ô˘ Î·È ÙË ‰˘ÛÌÔÚÊ›· ÙÔ˘ ÚÔÛÒÔ˘, ı· Ú¤ÂÈ Ó· ÂÊ·ÚÌfi˙ÔÓÙ·È Ì˘˚Τ˜ ·Û΋ÛÂȘ ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ·˘¯¤Ó· ·fi ÙÔ˘˜ ÁÔÓ›˜, ÔÈ Ôԛ˜ ÛÙÔ¯Â‡Ô˘Ó: ·) ÛÙË ‰È¿Ù·ÛË ÙÔ˘ ÛÙÂÚÓÔÎÏÂȉÔÌ·ÛÙÔÂȉԇ˜ Ì˘fi˜. ªÂ ÙÔ ¤Ó· ¯¤ÚÈ, Ô ÁÔÓÈfi˜ ÎÚ·Ù¿ ÛÙËÓ ·ÓÒÙÂÚË ıˆÚ·ÎÈ΋ ÂÚÈÔ¯‹ ÙÔ˘ ‚Ú¤ÊÔ˘˜ Î·È Ì ÙÔ ¿ÏÏÔ ÛÙÚ¤ÊÂÈ ··Ï¿ ÙÔ ÎÂÊ¿ÏÈ, ÎÚ·ÙÒÓÙ·˜ ÙÔ Û ·˘Ù‹ ÙË ı¤ÛË ÁÈ· 10 ‰Â˘ÙÂÚfiÏÂÙ·. ™ÙË Û˘Ó¤¯ÂÈ· Â·Ó·Ï·Ì‚¿ÓÂÈ

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239

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ÙÔ ›‰ÈÔ, ÛÙÚ¤ÊÔÓÙ·˜ ÙËÓ ÎÂÊ·Ï‹ ÚÔ˜ ÙËÓ ¿ÏÏË ÏÂ˘Ú¿, ‚) ÛÙË ‰È¿Ù·ÛË ÙÔ˘ ÙÚ·Â˙ÔÂȉԇ˜ Ì˘fi˜, Ì Ï¿ÁÈ· Î¿Ì„Ë Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ÁÈ· 10 ‰Â˘ÙÂÚfiÏÂÙ·. ∆Ô ›‰ÈÔ Á›ÓÂÙ·È Î·È ÚÔ˜ ÙÔÓ ·ÓÙ›ıÂÙÔ ÒÌÔ. √È ·Û΋ÛÂȘ ·˘Ù¤˜ Á›ÓÔÓÙ·È Û οı ·ÏÏ·Á‹ ¿Ó·˜ Î·È Ô ¯ÚfiÓÔ˜ Ô˘ ˘ÔÏÔÁ›˙ÂÙ·È fiÙÈ ¯ÚÂÈ¿˙ÂÙ·È Â›Ó·È ÂÚ›Ô˘ 2 ÏÂÙ¿, ηıÒ˜ Á›ÓÔÓÙ·È 3 Â·Ó·Ï‹„ÂȘ ÁÈ· οı ¿ÛÎËÛË. ∂ÈÚfiÛıÂÙ·, ÔÈ ÁÔÓ›˜ ÌÔÚÔ‡Ó Ó· ‰È‰·¯ıÔ‡Ó ÙË Ù¯ÓÈ΋ Ù˘ ÂÚÈÛÙÚÂÊfiÌÂÓ˘ ηڤÎÏ·˜, fiˆ˜ ·˘Ù‹ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ·fi ÙÔÓ ·È‰›·ÙÚÔ ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜. ªÂ ·˘Ùfi ÙÔÓ ÙÚfiÔ ÂÈÙ˘Á¯¿ÓÂÙ·È ÂÓ‰˘Ó¿ÌˆÛË Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜ ÙÔ˘ ÙÚ·¯‹ÏÔ˘ Î·È ÙÔ˘ ·˘¯¤Ó· ÙÔ˘ ‚Ú¤ÊÔ˘˜ (11).

∂Ӊ›ÍÂȘ ·Ú·ÔÌ‹˜ ∞Ó Ì ٷ ·Ú·¿Óˆ ̤ÙÚ· ‰ÂÓ ˘¿ÚÍÂÈ Ë ÂÈı˘ÌËÙ‹ ‚ÂÏÙ›ˆÛË, ̤۷ Û ¤Ó· ‰È¿ÛÙËÌ· 23 ÌËÓÒÓ ‹ Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· ‰ÂÓ Â›Ó·È Û˘Ì‚·Ù‹ ÌÂ Ù˘È΋ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘, ÙÔ ‚Ú¤ÊÔ˜ ı· Ú¤ÂÈ Ó· ·Ú·ÂÌÊı› ¤ÁηÈÚ· Û ÂÍÂȉÈÎÂ˘Ì¤ÓÔ ·È‰Ô-Ó¢ÚÔ¯ÂÈÚÔ˘ÚÁfi, ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂȂ‚·Èˆı› Ë ÔÚıfiÙËÙ· Ù˘ ‰È¿ÁÓˆÛ˘.

√ÚıÔ‰Èο ‚ÔËı‹Ì·Ù· ∏ ·Ó·‰È·ÌfiÚʈÛË ÙÔ˘ Û¯‹Ì·ÙÔ˜ ÙÔ˘ ÎÚ·Ó›Ô˘ ÁÈ· ·ÈÛıËÙÈÎÔ‡˜ ÏfiÁÔ˘˜ Ì ÙË ¯Ú‹ÛË ÔÚıÔ‰ÈÎÒÓ Û˘Û΢ÒÓ ¤¯ÂÈ ÙȘ Ú›˙˜ Ù˘ ÛÙËÓ ·Ú¯·ÈfiÙËÙ·. °È· ÙË Û‡Á¯ÚÔÓË ıÂÚ·¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û˘Û΢¤˜ Ì ÌÔÚÊ‹ ÎÚ¿ÓÔ˘˜, ÔÈ Ôԛ˜ ›ÙÂ Û˘ÌȤ˙Ô˘Ó ÙËÓ ÚÔÂͤ¯Ô˘Û· ÏÂ˘Ú¿ Ù˘ ÎÂÊ·Ï‹˜, ›Ù ÂÈÙÚ¤Ô˘Ó ÙËÓ ·Ó¿Ù˘ÍË Ù˘ ÂÈ‰ˆÌ¤Ó˘ ÂÚÈÔ¯‹˜ ÌfiÓÔ (8,10,28,44-46). ∏ Û˘Û΢‹ ÊÔÚÈ¤Ù·È ÁÈ· 15-22 ÒÚ˜ ËÌÂÚËÛ›ˆ˜, ÂÓÒ ÙÔ ÎÚ·Ó›Ô ı· Ú¤ÂÈ Ó· ÂÈÛÎÔÂ›Ù·È Î·ıËÌÂÚÈÓ¿ ÁÈ· Ù˘¯fiÓ ÂÚÂıÈÛÌÔ‡˜ ‹ Âί˘ÌÒÛÂȘ Î·È Ó· Á›ÓÂÙ·È ¤ÏÂÁ¯Ô˜ Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ·‡ÍËÛ˘ Ù˘ ÂÚÈ̤ÙÚÔ˘ Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜. ∏ ηχÙÂÚË ·ÓÙ·fiÎÚÈÛË ÛÙË ¯Ú‹ÛË ·˘ÙÒÓ ÙˆÓ Û˘Û΢ÒÓ Â›Ó·È Û ËÏÈΛ· 4-12 ÌËÓÒÓ. ∏ ¯Ú‹ÛË ·˘ÙÒÓ ÙˆÓ ‚ÔËıËÌ¿ÙˆÓ ÚÔÙ›ÓÂÙ·È Û ÛÔ‚·Ú‹ ÌÔÚÊ‹ ·Û˘ÌÌÂÙÚ›·˜ ‹ fiÙ·Ó ‰ÂÓ ˘¿Ú¯ÂÈ ‰ÈfiÚıˆÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ Ì ÙȘ Ô‰ËÁ›Â˜ ÛˆÛÙ‹˜ ÙÔÔı¤ÙËÛ˘ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ÛÙËÓ ÎÏ›ÓË. ∏ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ ‚ÔËıËÌ¿ÙˆÓ ·˘ÙÒÓ Â›Ó·È ·ÓÒÙÂÚË Ù˘ Û˘ÓÙËÚËÙÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘ (7),

·ÏÏ¿ ÙÔ Û˘ÓÔ‰fi ÎfiÛÙÔ˜ Û ۯ¤ÛË Ì ÙË ÁÂÓÈο ηϋ ÚfiÁÓˆÛË Ù˘ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ Û˘ÓËÁÔÚÔ‡Ó ÛÙË ¯ÚËÛÈÌÔÔ›ËÛ‹ ÙÔ˘˜ Û ÂÈÏÂÁ̤Ó˜ ÂÚÈÙÒÛÂȘ.

ÃÂÈÚÔ˘ÚÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË ŒÓ‰ÂÈÍË ¯ÂÈÚÔ˘ÚÁÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘ ˘¿Ú¯ÂÈ Û ÂÚÈÙÒÛÂȘ Û˘ÓÔÛÙˆÙÈ΋˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ Î·È Û¿ÓÈ· Û ÂÚÈÙÒÛÂȘ ÛÔ‚·Ú‹˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘ Ô˘ ‰ÂÓ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ Û˘ÓÙËÚËÙÈ΋ ·ÁˆÁ‹ (6,7,11). ™ÙÔ ·ÚÂÏıfiÓ Ë ¯ÚËÛÈÌÔÔ›ËÛË ÙÔ˘ fiÚÔ˘ «ÏÂÈÙÔ˘ÚÁÈ΋ Û˘ÓÔÛÙ¤ˆÛË», ÁÈ· Ó· ÂÚÈÁÚ¿„ÂÈ ÂӉȿÌÂÛ˜ ·ÎÙÈÓÔÏÔÁÈΤ˜ ÂÈÎfiÓ˜ ÌÂٷ͇ Ù˘ Û˘ÓÔÛÙ¤ˆÛ˘ Î·È Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ÌÔÚÊÔÏÔÁ›·˜ ÙˆÓ Ú·ÊÒÓ ÙÔ˘ ÎÚ·Ó›Ô˘, Ô‰‹ÁËÛ Û ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÂÚÈÙÒÛÂˆÓ Ô˘ ·ÚÁfiÙÂÚ· ·ԉ›¯ıËΠfiÙÈ ı· ›¯·Ó ÂÍ›ÛÔ˘ ηϿ ·ÔÙÂϤÛÌ·Ù· Î·È ÌÂ Û˘ÓÙËÚËÙÈ΋ ·ÁˆÁ‹ (7). ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë Ï·ÁÈÔÎÂÊ·Ï›· Â›Ó·È Û˘Ó‹ı˘ ηٿÛÙ·ÛË Ô˘ ÔÊ›ÏÂÙ·È Û˘Ó‹ıˆ˜ ÛÙËÓ ÙÔÔı¤ÙËÛË ÙÔ˘ ‚Ú¤ÊÔ˘˜ ÛÙËÓ ›‰È· ¿ÓÙ· ı¤ÛË ‡ÓÔ˘. ∏ Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘ (47) Ú¤ÂÈ Ó· ‰È·ÎÚ›ÓÂÙ·È ·fi ÙË ÛÔ‚·ÚfiÙÂÚË Î·Ù¿ÛÙ·ÛË Ù˘ Û˘ÓÔÛÙˆÙÈ΋˜ Ï·ÁÈÔÎÂÊ·Ï›·˜, ÂÓÒ Ë ÏÂÙÔÌÂÚ‹˜ ÂͤٷÛË ÙÔ˘ Ì˘ÔÛÎÂÏÂÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Â›Ó·È ÂȂ‚ÏË̤ÓË ÁÈ· ÙËÓ ·Ó·ÁÓÒÚÈÛË Û˘ÓÔ‰ÒÓ ÔÚıÔ‰ÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ. ™Â ÂÚÈÙÒÛÂȘ Ù˘È΋˜ Ï·ÁÈÔÎÂÊ·Ï›·˜ ı¤Û˘, Ì ‚¿ÛË ÙÔ ÈÛÙÔÚÈÎfi Î·È ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË, ‰ÂÓ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ÂÚ·ÈÙ¤Úˆ ·ÂÈÎÔÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘ Î·È Ë ·ÓÙÈÌÂÙÒÈÛË ‚·Û›˙ÂÙ·È ÛÙȘ ηٿÏÏËϘ Ô‰ËÁ›Â˜ ÙÔÔı¤ÙËÛ˘ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ηٿ ÙÔÓ ‡ÓÔ.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Miller RI, Clarren SK. Long-term developmental outcomes in patients with deformational plagiocephaly. Pediatrics [Internet]. 2000 Feb: Webpage: http://www.pediatrics.org/cgi/content/full/105/2/e26 2. Peitsch WK, Keefer CH, LaBrie RA, Mulliken JB. Incidence of cranial asymmetry in healthy newborns. Pediatrics [Internet]. 2002 Dec: Webpage: http://www.pediatrics.org/cgi/content/full/110/6/e72 3. Habal MB, Leimkuehler T, Chambers C, Scheuerle J, Guilford AM. Avoiding the sequela associated with deformational plagiocephaly. J Craniofac Surg 2003;14:430-437.

4. American Academy of Pediatrics. Changing concepts of sudden infant death syndrome: implications for infant sleeping environment and sleep position.

Task Force on Infant Sleep Position and Sudden ¶·È‰È·ÙÚÈ΋ 2006;69:233-241

Pediatr May-June 06

16-05-06

17:21

™ÂÏ›‰·240

240

∞. ºÈÏÈ›‰Ë Î·È Û˘Ó.

Infant Death Syndrome. Pediatrics 2000;105:650-656.

5. Flores-Sarnat L. New insights into craniosynostosis.

Semin Pediatr Neurol 2002;9:274-291.

6. Panchal J, Uttchin V. Management of craniosynostosis. Plast Reconstr Surg 2003;111:2032-2048.

7. Mulliken JB, Vander Woude DL, Hansen M, LaBrie RA, Scott RM. Analysis of posterior plagiocephaly: deformational versus synostotic. Plast Reconstr Surg 1999;103:371-380.

8. Hutchison BL, Thompson JM, Mitchell EA. Determinants of nonsynostotic plagiocephaly: a case-control study. Pediatrics [Internet] 2003, Oct: Webpage: http://www.pediatrics.org/cgi/content/full/112/4/e316 9. O'Broin ES, Allcutt D, Earley MJ. Posterior plagiocephaly: proactive conservative management. Br J Plast Surg 1999;52:18-23.

10. Bridges SJ, Chambers TL, Pople IK. Plagiocephaly and head binding. Arch Dis Child 2002;86:144-145.

11. Persing J, James H, Swanson J, Kattwinkel J. American Academy of Pediatrics Committee on Practice and Ambulatory Medicine, Section on Plastic Surgery and Section on Neurological Surgery.

Prevention and management of positional skull deformities in infants. Pediatrics 2003;112:199-202.

12. Boere-Boonekamp MM, van der Linden-Kuiper LT LT. Positional preference: prevalence in infants and follow-up after two years. Pediatrics 2001;107:339343.

13. Clarren SK, Smith DW, Hanson JW. Helmet treatment for plagiocephaly and congenital muscular torticollis. J Pediatr 1979;94:43-46.

14. Bruneteau RJ, Mulliken JB. Frontal plagiocephaly: synostotic, compensational, or deformational. Plast Reconstr Surg 1992;89:21-31.

15. Rekate HL. Occipital plagiocephaly: a critical review of the literature. J Neurosurg 1998;89:24-30.

16. Shuper A, Merlob P, Grunebaum M, Reisner SH. The incidence of isolated craniosynostosis in the newborn infant. Am J Dis Child 1985;139:85-86.

17. Jones BM, Hayward R, Evans R, Britto J. Occipital plagiocephaly: an epidemic of craniosynostosis?

BMJ 1997;315:693-694.

18. Littlefield TR, Kelly KM, Pomatto JK, Beals SP. Multiple-birth infants at higher risk for development of deformational plagiocephaly: II. is one twin at greater risk? Pediatrics 2002;109:19-25.

19. Kane AA, Mitchell LE, Craven KP, Marsh JL. Observations on a recent increase in plagiocephaly without synostosis. Pediatrics 1996;97:877-885.

20. Turk AE, McCarthy JG, Thorne CH, Wisoff JH. The “back to sleep campaign” and deformational plagiocephaly: is there cause for concern? J Craniofac Surg 1996;7:12-18.

21. Argenta LC, David LR, Wilson JA, Bell WO. An increase in infant cranial deformity with supine sleeping position. J Craniofac Surg 1996;7:5-11.

22. °Ú›‚·˜ £µ, Webb JK, Burwell RG. ¡Ëȷ΋ ȉÈÔ·ı‹˜ ÛÎÔÏ›ˆÛË (¡π™). ™ÙÔ: °Ú›‚·˜ £µ. ™‡Á¯ÚÔÓ˜ ∂ÍÂÏ›ÍÂȘ ÛÙËÓ ŒÚ¢ӷ Î·È £ÂÚ·›· Ù˘ ™ÎÔÏ›ˆÛ˘ π·ÙÚÈΤ˜ ∂ΉfiÛÂȘ ¶Ã ¶·Û¯·Ï›‰Ë˜; 2001. Û. 155-178.

23. Hanihara T. Comparison of craniofacial features of ¶·È‰È·ÙÚÈ΋ 2006;69:233-241

24.

25.

26.

27.

28.

29.

30.

31.

32.

33.

34.

35.

36.

37.

38.

39.

40.

major human groups. Am J Phys Anthropol 1996; 99:389-412. ¶ÂÙÚÔÔ‡ÏÔ˘ Ã, ∑ÒÛË ¶, ∆۷ΛÚ˘ ∞, °ÂˆÚÁÈ¿‰Ë˜ ∏, £ÂÔ‰ˆÚ›‰Ô˘ ¡, ∫·Ó¿Ú˘ § Î·È Û˘Ó. ™‡Ó‰ÚÔÌÔ Crouzon (ÎÚ·ÓÈÔÚÔÛˆÈ΋ ‰˘ÛfiÛÙˆÛË). π·ÙÚÈο XÚÔÓÈο 1997;20:410-411. ºÚ˘Û›Ú· ∂, •·È‰¿Ú· ∞, ºˆÙ›Ô˘ ∫, ¶··ı·Ó·Û›Ô˘∫ÏÒÓÙ˙· ¢, ∫·Ó·‚¿Î˘ ∂. ªÈ· ÔÈÎÔÁ¤ÓÂÈ· Ì ۇӉÚÔÌÔ Crouzon: °ÂÓÂÙÈ΋ ÌÂϤÙË. 35o ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ 1997;363∂∞. ¡ÙÈÔ‡‰Ë ƒºÌÂ Û˘Ó‡·ÚÍË ‰˘ÛÏ·ÛÈÒÓ ·fi ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi ۈϋӷ. ¶·È‰È·ÙÚÈ΋ BÔÚ›Ԣ ∂ÏÏ¿‰Ô˜ 1990;2:59-64.

Ehret FW, Whelan MF, Ellenbogen RG, Cunningham ML, Gruss JS. Differential diagnosis of the trapezoid-shaped head. Cleft Palate Craniofac J 2004;41:13-19.

Pollack IF, Losken HW, Fasick P. Diagnosis and management of posterior plagiocephaly. Pediatrics 1997;99:180-185.

O'Broin ES, Allcutt D, Earley MJ. Posterior plagiocephaly: proactive conservative management. Br J Plast Surg 1999;52:18-23.

Pople IK, Sanford RA, Muhlbauer MS. Clinical presentation and management of 100 infants with occipital plagiocephaly. Pediatr Neurosurg 1996;25:1-6.

Levine RM. Ocular plagiocephaly. Ophthalmology 2000;107:2123-2124. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ ∞, ƒÒÌ· ∂, °ÈÔ˘ÚÔ‡ÎÔ˜ ™, ª·ÙÛ·ÓÈÒÙ˘ ¡. ∞ÛÙÚÔ·و̷ Ù˘ ·˘¯ÂÓÈ΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ Ì ÌÔÓ·‰È΋ ÂΉ‹ÏˆÛË ÙÔ Ú·È‚fiÎÚ·ÓÔ. ¢ÂÏÙ›Ô ∞ã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ 1982;29:52-56. ¶ÂÙÚfiÔ˘ÏÔ˜ ∞, ∆ÛÔ˘Ì¿Î·˜ ∫, °È·ÓÓÔ‡ÏÈ·-∫·Ú·ÓÙ·Ó¿ ∞, ªÂÛÛ·ÚÈÙ¿Î˘ π. ∆Ô Ô͇ Ú·È‚fiÎÚ·ÓÔ ˆ˜ ·È‰È·ÙÚÈÎfi Úfi‚ÏËÌ·. ¢ÂÏÙ›Ô ∞ã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ 1996;43:25-27.

Demirbilek S, Atayurt HF. Congenital muscular torticollis and sternomastoid tumor: results of nonoperative treatment. J Pediatr Surg 1999;34:549-551.

Greenberg MF, Pollard ZF. Ocular plagiocephaly: ocular torticollis with skull and facial asymmetry.

Ophthalmology 2000;107:173-179.

Medina LS, Richardson RR, Crone K. Children with suspected craniosynostosis: a cost-effectiveness analysis of diagnostic strategies. Am J Roentgenol 2002; 179:215-221.

Captier G, Leboucq N, Bigorre M, Canovas F, Bonnel F, Bonnafe A et al. Plagiocephaly: morphometry of skull base asymmetry. Surg Radiol Anat 2003;25:226-233.

Sze RW, Parisi MT, Sidhu M, Paladin AM, Ngo AV, Seidel KD et al. Ultrasound screening of the lambdoid suture in the child with posterior plagiocephaly. Pediatr Radiol 2003;33:630-636.

Soboleski D, Mussari B, McCloskey D, Sauerbrei E, Espinosa F, Fletcher A. High-resolution sonography of the abnormal cranial suture. Pediatr Radiol 1998;28:79-82.

Jantz JW, Blosser CD, Fruechting LA. A motor milestone change noted with a change in sleep position.

Arch Pediatr Adolesc Med 1997;151:565-568.

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¶Ï·ÁÈÔÎÂÊ·Ï›· ı¤Û˘

41. Fredrick DR, Mulliken JB, Robb RM. Ocular manifestations of deformational frontal plagiocephaly. J Pediatr Ophthalmol Strabismus 1993;30:92-95.

42. Gupta PC, Foster J, Crowe S, Papay FA, Luciano M, Traboulsi EI. Ophthalmologic findings in patients with nonsyndromic plagiocephaly. J Craniofac Surg 2003;14:529-532.

43. Clarren SK. Plagiocephaly and torticollis: etiology, natural history, and helmet treatment. J Pediatr 1981;98:92-95.

44. Biggs WS. Diagnosis and management of positional head deformity. Am Fam Physician 2003;67:19531956.

45. Teichgraeber JF, Seymour-Dempsey K, Baumgartner JE, Xia JJ, Waller AL, Gateno J. Molding helmet therapy in the treatment of brachycephaly and plagiocephaly. J Craniofac Surg 2004;15:118-123.

46. de Chalain T. The Safe-T-Sleep device: safety and efficacy in maintaining infant sleeping position. N Z Med J 2003;116:U581.

47. Losee JE, Mason AC. Deformational plagiocephaly: diagnosis, prevention, and treatment. Clin Plast Surg 2005;32:53-64.

¶·È‰È·ÙÚÈ΋ 2006;69:233-241

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1 √ÚıÔ‰ÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. Î·È ∞. ∫˘ÚÈ·ÎÔ‡” 2 ¡Â˘ÚÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. Î·È ∞. ∫˘ÚÈ·ÎÔ‡” AÏÏËÏÔÁÚ·Ê›·: ¡ÈÎfiÏ·Ô˜ ª·ÚΤ·˜ ™ÈÎÂÏÈ·ÓÔ‡ 42, T.K. 122 43, ∞ÈÁ¿Ïˆ E-mail: markeasnik@teledomenet.gr

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∫§π¡π∫√ ∫√Àπ∑ ¡. ª·ÚΤ·˜1, ∫. µÔ‡‰Ú˘2

∞ÁfiÚÈ 2 ÂÙÒÓ ÚÔÛÎÔÌ›˙ÂÙ·È ·fi ÙÔ˘˜ ÁÔÓ›˜ ÙÔ˘ ÛÙ· Â›ÁÔÓÙ· È·ÙÚ›· ÙÔ˘ ÂÊËÌÂÚ‡ÔÓÙÔ˜ ÓÔÛÔÎÔÌ›Ԣ ÏfiÁˆ Ú·È‚fiÎÚ·ÓÔ˘. √ ÌÈÎÚfi˜ ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·Û ÙÔ Ú·È‚fiÎÚ·ÓÔ ·ÈÊÓ›‰È·, ÚÔ 2 ‚‰ÔÌ¿‰ˆÓ. ∂›¯Â ÚÔËÁËı› ÈÔÁÂÓ‹˜ Ïԛ̈ÍË ÙÔ˘ ·ÓˆÙ¤ÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ÚÈÓ 3 ‚‰ÔÌ¿‰Â˜, Ë ÔÔ›· ·ÓÙÈÌÂÙˆ›ÛÙËΠ̠·ÓÙÈ˘ÚÂÙÈ΋ ·ÁˆÁ‹. ªË¯·ÓÈÛÌfi˜ ÙÚ·˘Ì·ÙÈÛÌÔ‡ ‰ÂÓ ÂÚÈÁÚ¿ÊËÎÂ. ∞fi ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ÂÈÛËÌ¿ÓıËÎÂ Ë „ˆÚÈ·ÛÈ΋ ·ÚıÚ›Ùȉ· ·fi ÙËÓ ÔÔ›· ¿Û¯ÂÈ Ë ·‰ÂÏÊ‹ Ù˘ ÌËÙ¤Ú·˜. ∫·Ù¿ ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË, Ô ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·˙ ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË, ‹Ù·Ó ·‡ÚÂÙÔ˜ Î·È Ë fi„Ë Î·È ıÚ¤„Ë ÙÔ˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜. À‹Ú¯Â ÎÏ›ÛË Ù˘ ÎÂÊ·Ï‹˜ ÚÔ˜ Ù· ·ÚÈÛÙÂÚ¿, ÂÓÒ ÔÈ ÛÙÚÔÊÈΤ˜ ÎÈÓ‹ÛÂȘ ÙÔ˘ ·˘¯¤Ó· ‹Ù·Ó ÂÒ‰˘Ó˜ Î·È ÂÚÈÔÚÈṲ̂Ó˜. ∫·Ù¿ ÙËÓ „ËÏ¿ÊËÛË ÙÔ˘ ÙÚ·¯‹ÏÔ˘ Á›ÓÔÓÙ·Ó ·ÓÙÈÏËÙÔ› ÌÈÎÚÔ› ¢ΛÓËÙÔÈ ÏÂÌÊ·‰¤Ó˜ ·ÌÊÔÙÂÚfiÏ¢ڷ. ª·Û¯·ÏÈ·›ÔÈ Î·È ‚Ô˘‚ˆÓÈÎÔ› ÏÂÌÊ·‰¤Ó˜ ‰ÂÓ ‹Ù·Ó „ËÏ·ÊËÙÔ›. ∆Ô ·Ó·Ó¢ÛÙÈÎfi Î·È ÙÔ Î˘ÎÏÔÊÔÚÈÎfi Û‡ÛÙËÌ· ÂϤÁ¯ıËÎ·Ó Ê˘ÛÈÔÏÔÁÈο. ∆· ·Ú›ÛıÌÈ· Â›Û˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο, ÂÓÒ Ë ÎÔÈÏ›· ÂÎÙÈÌ‹ıËΠ̷Ϸ΋, ¢›ÂÛÙË, ·ÓÒ‰˘ÓË, Ì ˘Ô¯fiÓ‰ÚÈ· ÛÏ¿Á¯Ó· ·„ËÏ¿ÊËÙ·. ∞Ô˘Û›·˙·Ó ÂÛÙȷο Ó¢ÚÔÏÔÁÈο

·ıÔÏÔÁÈο ÛËÌ›·, ÂÓÒ Ë ÔÊı·ÏÌÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË ¤‰ÂÈÍ ÔÚıÔÊÔÚ›· Û fiϘ ÙȘ ‚ÏÂÌÌ·ÙÈΤ˜ ı¤ÛÂȘ, ‚˘ıfi Ê˘ÛÈÔÏÔÁÈÎfi Î·È ‰È·ıÏ·ÛÙÈ΋ ÈηÓfiÙËÙ· ¿ÚÈÛÙË. ∏ øƒ§ ÂͤٷÛË ·¤ÎÏÂÈÛ ÔÔÈ·‰‹ÔÙ ·ÚÂÎÙfiÈÛË ÙˆÓ ÙÔȯˆÌ¿ÙˆÓ ÙÔ˘ Ê¿Ú˘ÁÁÔ˜ ‹ ÂÚÈ·Ì˘Á‰·ÏÈÎfi ·fiÛÙËÌ·. ∆Ô Ù˘Ì·ÓfiÁÚ·ÌÌ· ·¤‚Ë Ê˘ÛÈÔÏÔÁÈÎfi. ∂ÚÁ·ÛÙËÚȷΤ˜ ÂÍÂÙ¿ÛÂȘ: §Â˘Î¿ ·ÈÌÔÛÊ·›ÚÈ· 17.900 Îί (49% ÔÏ˘ÌÔÚÊÔ‡ÚËÓ·, 40,4% ÏÂÌÊÔ·ÙÙ·Ú·), ·ÈÌÔÂÙ¿ÏÈ· 204.000 Îί, ∆∫∂ 75 mm ÙËÓ 1Ë ÒÚ· Î·È CRP 5. √È ·ÎÙÈÓÔÁڷʛ˜ ÙÔ˘ ·˘¯¤Ó· ¤‰ÂÈÍ·Ó ÌfiÓÔ ÙËÓ ·ÚÈÛÙÂÚ¿ Ï·ÁÈÔÎÂÊ·Ï›·, ÂÓÒ Ë ı¤ÛË, ÙÔ Û¯‹Ì· Î·È ÔÈ Û¯¤ÛÂȘ ÙˆÓ ·˘¯ÂÓÈÎÒÓ ÛÔÓ‰‡ÏˆÓ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. ¢ÂÓ ·Ú·ÙËÚ‹ıËΠԛ‰ËÌ· ÙˆÓ Ì·Ï·ÎÒÓ ÌÔÚ›ˆÓ ÛÙÔÓ ÔÈÛıÔÊ·Ú˘ÁÁÈÎfi ¯ÒÚÔ. ∏ MRI ÂÁÎÂÊ¿ÏÔ˘ ·¤ÎÏÂÈÛ ÔÔÈ·‰‹ÔÙ ¯ˆÚÔηٷÎÙËÙÈ΋ ÂÍÂÚÁ·Û›· ÙÔ˘ ÔÈÛı›Ô˘ ‚fiıÚÔ˘. ™ÙËÓ MRI ÙÔ˘ ·˘¯¤Ó· ·ÔηχÊıËÎÂ, Û ‰‡Ô Û˘Ó¯fiÌÂÓ˜ ÙÔ̤˜ ÛÙÔ ÛÙÂÊ·ÓÈ·›Ô Â›‰Ô, Ë ·ÚÔ˘Û›· ÂÏ·ÊÚ¿ ‰ÈÔÁΈ̤ÓÔ˘ ÔÈÛıÔÊ·Ú˘ÁÁÈÎÔ‡ ÏÂÌÊ·‰¤Ó· ÛÙË ‰ÂÍÈ¿ ÏÂ˘Ú¿, ÂÓÒ Û ÚÔÛıÈÔ›ÛıÈ· ÙÔÌ‹ (∂ÈÎfiÓ·) ·Ó·‰Â›¯ıËΠ¯·Ú·ÎÙËÚÈÛÙÈÎfi ‡ÚËÌ·, ÙÔ ÔÔ›Ô ıˆڋıËΠ·ıÔÁÓˆÌÔÓÈÎfi ÁÈ· ÙË ‰È¿ÁÓˆÛË Î·È ÙËÓ ÂÊ·ÚÌÔ˙fiÌÂÓË ıÂÚ·›·. ¶Èı·Ó¤˜ ‰È·ÁÓÒÛÂȘ: 1. ª˘ÔÁÂÓ¤˜ (Û˘ÁÁÂÓ¤˜) Ú·È‚fiÎÚ·ÓÔ 2. ∆Ú·˘Ì·ÙÈÎfi Ú·È‚fiÎÚ·ÓÔ 3. ∞ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ˘ÂÍ¿ÚıÚËÌ· ÊÏÂÁÌÔÓÒ‰Ô˘˜ ·ÈÙÈÔÏÔÁ›·˜ (™‡Ó‰ÚÔÌÔ Grisel) 4. √Êı·ÏÌÔÏÔÁÈÎfi Ú·È‚fiÎÚ·ÓÔ

∏ ·¿ÓÙËÛË ·ÎÔÏÔ˘ı› ÛÙË ÛÂÏ›‰· 246 ¶·È‰È·ÙÚÈ΋ 2006;69:242,246

Pediatr May-June 06

16-05-06

17:22

™ÂÏ›‰·243

∂¶π™∆√§H ¶ƒ√™ ∆∏ ™À¡∆∞•∏

LETTER TO THE EDITOR

243

∞fi ÙËÓ ∂Ù·ÈÚ›· æ˘¯ÔÎÔÈÓˆÓÈ΋˜ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ Î·È ÙÔ˘ ∂Ê‹‚Ô˘ (∂æÀ¶∂) £· ı¤Ï·Ì ӷ Û·˜ ÂÓËÌÂÚÒÛÔ˘Ì ÁÈ· ÙË ÏÂÈÙÔ˘ÚÁ›· Ù˘ ÙËÏÂʈÓÈ΋˜ ÁÚ·ÌÌ‹˜ 801 801 1177 °Ú·ÌÌ‹ - ™‡Ó‰ÂÛÌÔ˜ ÁÈ· ÙËÓ „˘¯È΋ ˘Á›· ÙÔ˘ ·È‰ÈÔ‡ Î·È ÙÔ˘ ÂÊ‹‚Ô˘. ∞˘Ù‹ Ë ÙËÏÂʈÓÈ΋ ÁÚ·ÌÌ‹ Â›Ó·È Ë ÚÒÙË ÛÙËÓ ∂ÏÏ¿‰· ÁÈ· ÚÔ‚Ï‹Ì·Ù· „˘¯È΋˜ ˘Á›·˜ ÁÈ· ÙÔ ·È‰› Î·È ÙÔÓ ¤ÊË‚Ô.

•ÂΛÓËÛ ÙË ÏÂÈÙÔ˘ÚÁ›· Ù˘ ˘fi ÙËÓ ÂÔÙ›· ÙÔ˘ Î. πˆ¿ÓÓË ∆ÛÈ¿ÓÙË, ∞Ó. ∫·ıËÁËÙ‹ ¶·È‰Ô„˘¯È·ÙÚÈ΋˜ Î·È ∂ÈÛÙËÌÔÓÈÎÔ‡ À‡ı˘ÓÔ˘ Ù˘ ∂Ù·ÈÚ›·˜ æ˘¯ÔÎÔÈÓˆÓÈ΋˜ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ Î·È ÙÔ˘ ∂Ê‹‚Ô˘ (∂æÀ¶∂), Ë ÔÔ›· Â›Ó·È Î·È Ô ÊÔÚ¤·˜ ÙÔ˘ ÚÔÁÚ¿ÌÌ·ÙÔ˜. ¶ÂÚÈÛÛfiÙÂÚ· ÛÙÔȯ›· ÁÈ· ÙËÓ ∂æÀ¶∂ ˘¿Ú¯Ô˘Ó ÛÙËÓ ÈÛÙÔÛÂÏ›‰·: http://www.epsype.gr ∏ ÙËÏÂʈÓÈ΋ ÁÚ·ÌÌ‹ 801 801 1177 ÛÙÂϯÒıËΠ·fi ‰ÈÂÈÛÙËÌÔÓÈ΋ ÔÌ¿‰· Ô˘ ·ÔÙÂÏÂ›Ù·È ·fi ·È‰Ô„˘¯È¿ÙÚÔ˘˜, ÎÏÈÓÈÎÔ‡˜ „˘¯ÔÏfiÁÔ˘˜ Î·È ÂÈÛΤÙ˜ ˘Á›·˜. ∞¢ı‡ÓÂÙ·È Û fiÔÈÔÓ ¤¯ÂÈ ·Ó¿ÁÎË Û˘Ì‚Ô˘Ï‹˜, ˘ÔÛÙ‹ÚÈ͢ ‹ ‚Ô‹ıÂÈ·˜ Û ı¤Ì·Ù· Ô˘ ·ÊÔÚÔ‡Ó ÙËÓ „˘¯È΋ ˘Á›· Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. ∞ÎfiÌË, Û Â·ÁÁÂÏ̷ٛ˜ ˘Á›·˜ Î·È ÂÎ·È‰Â˘ÙÈÎÔ‡˜. °ÓˆÚ›˙ÔÓÙ·˜ ÙË ÌÂÁ¿ÏË ·ÓÙ·fiÎÚÈÛË Ô˘ ¤¯ÂÈ ÙÔ ÂÚÈÔ‰ÈÎfi Û·˜ ÛÙÔ˘˜ Â·ÁÁÂÏ̷ٛ˜ ˘Á›·˜, Î·È ÂÎÙÈÌÒÓÙ·˜ ÙËÓ ÚÔÛ¿ıÂÈ· Ô˘ ¤¯ÂÈ ÍÂÎÈÓ‹ÛÂÈ ÒÛÙÂ Ë ÁÚ·ÌÌ‹ Ó· Á›ÓÂÈ ÁÓˆÛÙ‹ ÛÙÔ˘˜ ·Ú·¿Óˆ Â·ÁÁÂÏ̷ٛ˜, ÈÛÙ‡ԢÌ fiÙÈ ı· ‹Ù·Ó ÛËÌ·ÓÙÈ΋ Ë ÚÔ‚ÔÏ‹ Ù˘ ̤۷ ·fi ÙȘ ÛًϘ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ªÂ ÂÎÙ›ÌËÛË ƒ¤Ó· ¶ÔÁÈ·Ù˙fiÁÏÔ˘ ¶·È‰Ô„˘¯›·ÙÚÔ˜/ ™˘ÓÙÔÓ›ÛÙÚÈ· °Ú·ÌÌ‹˜

¡∂∫ƒ√§√°π∞

OBITUARY

∞ÁÁÂÏÈ΋˜ ª‹ÙÛÈη-∫·Ú·Ó¿ÛÈÔ˘ ¶·È‰È¿ÙÚÔ˘, ∞Ó·ÏËÚÒÙÚÈ·˜ ¢È¢ı‡ÓÙÚÈ·˜ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜, ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ ¶ÂÓÙ¤Ï˘ ªÂ ÙËÓ ·Ó·ÙÔÏ‹ ÙÔ˘ ηÈÓÔ‡ÚÁÈÔ˘ ¯ÚfiÓÔ˘, ¤Ê˘Á ͷÊÓÈο ·fi ÎÔÓÙ¿ Ì·˜ Ë ÂÎÏÂÎÙ‹ Û˘Ó¿‰ÂÏÊÔ˜ ∞ÁÁÂÏÈ΋ ª‹ÙÛÈη-∫·Ú·Ó¿ÛÈÔ˘. ∏ ›‰ËÛË ¤ÂÛÂ Û·Ó ÎÂÚ·˘Ófi˜ ÛÙÔ˘˜ Û˘Ó·‰¤ÏÊÔ˘˜, Ê›ÏÔ˘˜ Î·È Û˘ÓÂÚÁ¿Ù˜ Ù˘ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô Î·È Û fiÏË ÙËÓ ·È‰È·ÙÚÈ΋ ÎÔÈÓfiÙËÙ·. ∞fi ÙËÓ ·Ú¯‹ Ù˘ ηÚȤڷ˜ Ù˘, Ë ∞ÁÁÂÏÈ΋ ΤډÈÛ ÙËÓ ÂÎÙ›ÌËÛË, ÙËÓ ·Á¿Ë Î·È ÙÔÓ Û‚·ÛÌfi Ù˘ ·È‰È·ÙÚÈ΋˜ ÔÈÎÔÁ¤ÓÂÈ·˜ Î·È ‰ÂÓ ‰È¤„¢Û ÙȘ ÚÔÛ‰Ô˘ Ù˘. ªÂ ÙÔ ˘„ËÏfi ·›ÛıËÌ· ¢ı‡Ó˘, ÙË Û˘Ó¤ÂÈ·, ÙËÓ ÂÚÁ·ÙÈÎfiÙËÙ·, ÙËÓ Â˘Á¤ÓÂÈ· Î·È ÙÔ ‹ıÔ˜ Ô˘ ÙË ¯·Ú·ÎÙ‹ÚÈ˙·Ó, ·ÔÙÂÏÔ‡Û ʈÙÂÈÓfi ·Ú¿‰ÂÈÁÌ· ÁÈ· Ì›ÌËÛË Î·È ËÁ‹ ıÂÙÈ΋˜ ¤ÌÓ¢Û˘ ÁÈ· fiÏÔ˘˜ Ì·˜. ∆Ô ÎÂÓfi Ô˘ ·Ê‹ÓÂÈ Â›Ó·È ‰˘Û·Ó·Ï‹ÚˆÙÔ. £· ̤ÓÂÈ ÛÙË ÌÓ‹ÌË Î·È ÙËÓ Î·Ú‰È¿ Ì·˜ ·Í¤¯·ÛÙË Î·È ·Á·Ë̤ÓË. ∑ˆ‹ ∫·Ú·Î·ÙÛ¿ÓË ∞Ó·ÏËÚÒÙÚÈ· ¢È¢ı‡ÓÙÚÈ· ¶·È‰È·ÙÚÈ΋˜

¶·È‰È·ÙÚÈ΋ 2006;69:243

Pediatr May-June 06

244

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

16-05-06

17:22

™ÂÏ›‰·244

¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√

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

∏ ·Ó¿Ù˘ÍË ÛÙÔ ÚÔÛ΋ÓÈÔ ∏ ·Ó¿Ù˘ÍË ·ÔÙÂÏ› ÌÈ· ·fi ÙȘ ÛËÌ·ÓÙÈÎfiÙÂÚ˜ ·Ú·Ì¤ÙÚÔ˘˜ ·Ó·ÊÔÚ¿˜ ÁÈ· ÙËÓ ˘Á›· ÂÓfi˜ ·È‰ÈÔ‡. ∏ÏÂÎÙÚÔÓÈο ÂÚÁ·Ï›· Î·È ÏËÚÔÊÔڛ˜, ı· Ì·˜ ‰È¢ÎÔχÓÔ˘Ó ÛÙËÓ ÂÎÙ›ÌËÛ‹ Ù˘.

¶›Ó·Î˜ ·Ó¿Ù˘Í˘ ·fi ÙÔ CDC www.cdc.gov/nccdphp/dnpa/growthcharts/training.htm √ÏÔÎÏËڈ̤ÓË Î·È Ô˘ÛÈ·ÛÙÈ΋, Ë ÈÛÙÔÛÂÏ›‰· ÙÔ˘ CDC (∂ıÓÈÎfi ∫¤ÓÙÚÔ ÁÈ· ÙËÓ ¶ÚfiÏË„Ë ¡ÔÛËÌ¿ÙˆÓ Î·È ÙËÓ ¶ÚÔ·ÁˆÁ‹ Ù˘ ÀÁ›·˜) Û¯ÂÙÈο Ì ÙÔ˘˜ ›Ó·Î˜ ·Ó¿Ù˘Í˘ ÚÔÛʤÚÂÈ ÏËÚÔÊÔڛ˜, on-line ÂÎ·›‰Â˘ÛË Î·È ‚¤‚·È· Ù· ›‰È· Ù· ·Ú¯Â›· Ì ÙȘ ηÌ‡Ï˜ ·Ó¿Ù˘Í˘ ÙÔ˘ 2000. ÀÔ‰ÂÈÎÓ‡ÂÈ Â›Û˘, ÂÚÁ·Ï›· Î·È ÚÔÁÚ¿ÌÌ·Ù· ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ ‰Â›ÎÙË Ì¿˙·˜ ÛÒÌ·ÙÔ˜ (µªπ). ŒÓ·˜ ‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ Ô ÔÔ›Ô˜ ¤¯ÂÈ Ó· ÚÔÛʤÚÂÈ ÛËÌ·ÓÙÈο, Û οı ·È‰›·ÙÚÔ. ∞Ó Î·È Ù· ‰Â‰Ô̤ӷ ÙÔ˘ CDC ηχÙÔ˘Ó Û¯Â‰fiÓ Ï‹Úˆ˜ ÙȘ ·Ó¿ÁΘ, ÂÓ·ÏÏ·ÎÙÈΤ˜ ηÌ‡Ï˜ ·Ó¿Ù˘Í˘ ˘¿Ú¯Ô˘Ó ÛÙÔ http://www.halls.md/chart/child-growth/pediatric.htm fiÔ˘ ÔÈ ÏËÚÔÊÔڛ˜ Â›Ó·È Î·Ù·Ù·Á̤Ó˜ ·Ó·ÏfiÁˆ˜ Ê˘Ï‹˜.

¶ÚÔÛˆÈÎÔ› „ËÊÈ·ÎÔ› ‚ÔËıÔ› ™ÙȘ Ë̤Ú˜ Ì·˜, fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚÔÈ ·È‰›·ÙÚÔÈ ¯ÚËÛÈÌÔÔÈÔ‡Ó “ÚÔÛˆÈÎÔ‡˜ „ËÊÈ·ÎÔ‡˜ ‚ÔËıÔ‡˜” (personal digital assistant, PDA) ‹ ÈÔ ·Ï¿, ÌÈÎÚÔÛÎÔÈÎÔ‡˜ ˘ÔÏÔÁÈÛÙ¤˜ Ô˘ ¯ˆÚÔ‡Ó ÛÙË ÙÛ¤Ë Î·È ÂÎÙfi˜ ·fi ÏÂÈÙÔ˘ÚÁ›Â˜ ÙËÏÂʈÓÈÎÔ‡ ηٷÏfiÁÔ˘ Î·È ËÌÂÚÔÏÔÁ›Ô˘ ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ˆ˜ È·ÙÚÈο ÂÚÁ·Ï›· ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ú¿ÍË.

STAT Growth - www.statcoder.com/growthcharts.htm ∞Ó¿ÌÂÛ· ÛÙ· ÚÔÁÚ¿ÌÌ·Ù· Ù˘ STAT, ÂӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ ÙÔ Growth, Ì ÙÔ ÔÔ›Ô ÌÔÚ› ηÓ›˜ Ó· ·Ú·ÎÔÏÔ˘ı‹ÛÂÈ ÙËÓ ·Ó¿Ù˘ÍË Î·È ÁÚ‹ÁÔÚ· Ó· ‰ÂÈ Û ÔÈÔ ÂηÙÔÛÙËÌfiÚÈÔ ‚Ú›ÛÎÂÙ·È ¤Ó· ·È‰› fiÛÔÓ ·ÊÔÚ¿ ÙÔ ‚¿ÚÔ˜, ÙÔ ‡„Ô˜ ÙËÓ ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜, ÙÔÓ ‰Â›ÎÙË Ì¿˙·˜ ÛÒÌ·ÙÔ˜ Î·È ¿ÏÏ·. µ·Û›˙ÂÙ·È ÛÙȘ ηÌ‡Ï˜ ÙÔ˘ CDC ÙÔ˘ 2000 Î·È Â›Ó·È Î·Ù·Û΢·Ṳ̂ÓÔ ÁÈ· Ï·ÙÊfiÚÌ· Palm, ·ÏÏ¿ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› Î·È Û Pocket PC.

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Essentials - www.epocrates.com ∂ÓÙ˘ˆÛȷ΋ ÔÛfiÙËÙ· Î·È ÔÈfiÙËÙ· ÏËÚÔÊÔÚÈÒÓ Ô˘ ·ÊÔÚÔ‡Ó ÛÂ Û˘ÓÙ·ÁÔÁÚ·Ê›·, ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Î·È ÂÚÁ·ÛÙËÚȷΤ˜ ÂÍÂÙ¿ÛÂȘ, ÂÚȤ¯ÔÓÙ·È ÛÙÔ ÚfiÁÚ·ÌÌ· Essentials, Ù˘ ÂÙ·ÈÚ›·˜ Epocrates (www.epocrates.com). ª¤Û· ÛÙÔ ÚfiÁÚ·ÌÌ· ÌÔÚ› Ó· ‚Ú› ‚¤‚·È· ηÓ›˜ Î·È ˘ÔÏÔÁÈÛÙ‹ ‰Â›ÎÙË Ì¿˙·˜ ÛÒÌ·ÙÔ˜, ·Ó·Ï˘Ù‹ ·¯˘Û·ÚΛ·˜ Î.¿.

PediSuite - www.collectivemed.com/jump/pedis.shtml ¶ÈÔ ÎÔÓÙ¿ ÛÙÔ˘˜ ¶·È‰È¿ÙÚÔ˘˜, ÙÔ PediSuite Â›Ó·È ¤Ó· Ï‹Ú˜ ÚfiÁÚ·ÌÌ· ÁÈ· PDAs (PPC Î·È Palm), ÙÔ ÔÔ›Ô ·Ó¿ÌÂÛ· ÛÙȘ ÏÔ‡ÛȘ ‰˘Ó·ÙfiÙËÙ¤˜ ÙÔ˘ ÂÚȤ¯ÂÈ Î·È ˘ÔÏÔÁÈÛÙ‹ ¢ª™ Î·È Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘, ‚·ÛÈṲ̂Ó˜ ÛÙȘ CDC 2000. °È· ÂÚÈÛÛfiÙÂÚ· ÚÔÁÚ¿ÌÌ·Ù·, ·Í›˙ÂÈ Î·Ó›˜ Ó· Ú›ÍÂÈ ÌÈ· Ì·ÙÈ¿ ÛÙȘ ‚È‚ÏÈÔı‹Î˜ collectivemed Î·È medspda, (www.collectivemed.com Î·È www.medspda.com ·ÓÙ›ÛÙÔȯ·) fiÔ˘ ˘¿Ú¯ÂÈ ÔÈÎÈÏ›· È·ÙÚÈÎÒÓ ÚÔÁÚ·ÌÌ¿ÙˆÓ, ÔÏÏ¿ ÂÎ ÙˆÓ ÔÔ›ˆÓ Â›Ó·È ‰ˆÚ¿Ó.

ÀÔÏÔÁÈÛÙ‹˜ ÙÔ˘ ‰Â›ÎÙË SDS http://spitfire.emmes.com/study/ped/resources/htwtcalc.htm ™ÙËÓ ÈÛÙÔÛÂÏ›‰· Ù˘ µÔÚÂÈÔ·ÌÂÚÈηÓÈ΋˜ ªÂϤÙ˘ ¶·È‰È·ÙÚÈ΋˜ ¡ÂÊÚÈ΋˜ ªÂÙ·ÌfiÛ¯Â˘Û˘ (The North American Pediatric Renal Transplant Cooperative Study NAPRTCS), ˘¿Ú¯ÂÈ ¤Ó·˜ ˘ÔÏÔÁÈÛÙ‹˜ ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ ‰Â›ÎÙË SDS (standard deviation score) Î·È ÙˆÓ ÂηÙÔÛÙÈ·›ˆÓ ı¤ÛÂˆÓ ÙÔ˘ ‚¿ÚÔ˘˜ Î·È ÙÔ˘ ‡„Ô˘˜ ÙˆÓ ·È‰ÈÒÓ. ∂Ï›˙Ô˘Ì ӷ Û·˜ Ê·Ó› ¯Ú‹ÛÈÌÔ˜.

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CLINICAL QUIZ ∫ÏÈÓÈÎfi QUIZ ∞¶∞¡∆∏™∏

∞ÙÏ·ÓÙÔ·ÍÔÓÈÎfi ˘ÂÍ¿ÚıÚËÌ· ÊÏÂÁÌÔÓÒ‰Ô˘˜ ·ÈÙÈÔÏÔÁ›·˜ (™‡Ó‰ÚÔÌÔ Grisel).

™¯ÔÏÈ·ÛÌfi˜ ∏ MRI ÙÔ˘ ·˘¯¤Ó· ÛÙËÓ ÚÔÛıÈÔ›ÛıÈ· ÙÔÌ‹, fiˆ˜ Ê·›ÓÂÙ·È ÛÙËÓ ÂÈÎfiÓ·, ·Ó¤‰ÂÈÍÂ Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡ ÛÙËÓ ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ¿ÚıÚˆÛË. ∆Ô Â‡ÚËÌ· ·˘Ùfi ‹Ù·Ó ·ıÔÁÓˆÌÔÓÈÎfi ÁÈ· Û‡Ó‰ÚÔÌÔ Grisel Î·È ‰ÈηÈÔÏÔÁÔ‡Û ·fiÏ˘Ù· ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ·. √ ·ıÔÁÂÓÂÙÈÎfi˜ Ì˯·ÓÈÛÌfi˜ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Û¯ÂÙ›˙ÂÙ·È Ì ÚÔ¸¿Ú¯Ô˘Û· Ïԛ̈ÍË ÙÔ˘ ·ÓˆÙ¤ÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ (Ê·Ú˘ÁÁ›Ùȉ·, ÂÚÈ·Ì˘Á‰·ÏÈÎfi ·fiÛÙËÌ· ‹ ˘Ò‰Ë ˆÙ›Ùȉ·) ‹ ¤Ó· ¯ÂÈÚÔ˘ÚÁÈÎfi ÙÚ·˘Ì·ÙÈÛÌfi (·Ì˘Á‰·ÏÂÎÙÔÌ‹ ‹ ·Ê·›ÚÂÛË ·‰ÂÓÔÂȉÒÓ Â΂ϷÛÙ‹ÛˆÓ). √È Î·Ù·ÛÙ¿ÛÂȘ ·˘Ù¤˜ ÌÔÚÔ‡Ó Ó· Ô‰ËÁ‹ÛÔ˘Ó Û ··Û‚¤ÛÙˆÛË ÙÔ˘ ÚÔÛı›Ô˘ ÙfiÍÔ˘ ÙÔ˘ ¿ÙÏ·ÓÙ· ÏfiÁˆ ˘ÂÚ·ÈÌ›·˜ Î·È ¯·Ï·ÚfiÙËÙ· ÙÔ˘ ÂÁηÚÛ›Ô˘ Û˘Ó‰¤ÛÌÔ˘, Ô ÔÔ›Ô˜ Ê˘ÛÈÔÏÔÁÈο Û˘ÁÎÚ·Ù› ÙÔÓ Ô‰fiÓÙ· ÙÔ‡ ¿ÍÔÓ· ÛÙË ı¤ÛË ÙÔ˘ (1). ™‡Ìʈӷ Ì ÙËÓ Ù·ÍÈÓfiÌËÛË ÙÔ˘ Fielding (2), Ë ·ÙÏ·ÓÙÔ·ÍÔÓÈ΋ ¿ÚıÚˆÛË ÛÙÔ Û‡Ó‰ÚÔÌÔ Grisel ÌÔÚ› Ó· ˘ÔÛÙ› ˘ÂÍ¿ÚıÚËÌ· Û ‰È¿ÊÔÚÔ˘˜ Ù‡Ô˘˜. ™ÙÔÓ Ù‡Ô 1 ˘¿Ú¯ÂÈ ÛÙÚÔÊ‹ ÙÔ˘ ¿ÙÏ·ÓÙ· ¯ˆÚ›˜ ·ÚÂÎÙfiÈÛË, fiˆ˜ ·Ú·ÙËÚ‹ıËΠÛÙËÓ ÚÔÎÂÈ̤ÓË ÂÚ›ÙˆÛË. ™ÙÔÓ Ù‡Ô 2 ˘¿Ú¯ÂÈ ÛÙÚÔÊ‹ ÙÔ˘ ¿ÙÏ·ÓÙ· ÂÙÂÚfiÏ¢ڷ (‰ËÏ·‰‹ ÛÙË Ì›· ÌfiÓÔ Ï¿ÁÈ· ·ÚıÚÈ΋ ·fiÊ˘ÛË), Ì ÚfiÛıÈ· ·ÚÂÎÙfiÈÛË Ô˘ Î˘Ì·›ÓÂÙ·È ·fi 3 ̤¯ÚÈ 5 mm. ™ÙÔÓ Ù‡Ô 3 Ë ÛÙÚÔÊ‹ ÙÔ˘ ¿ÙÏ·ÓÙ· Û˘Ó˘¿Ú¯ÂÈ Ì ÚfiÛıÈ· ·ÚÂÎÙfiÈÛË Ô˘ ÍÂÂÚÓ¿ Ù·

5 mm. ™ÙÔÓ Ù‡Ô 4, Ì·˙› Ì ÙË ÛÙÚÔÊ‹ ÙÔ˘ ¿ÙÏ·ÓÙ·, Û˘Ó˘¿Ú¯ÂÈ Ô›ÛıÈ· ·ÚÂÎÙfiÈÛË. ∏ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Grisel Û¯ÂÙ›˙ÂÙ·È Ì ÙÔÓ Ù‡Ô ÙÔ˘ ·ÙÏ·ÓÙÔ·ÍÔÓÈÎÔ‡ ˘ÂÍ·ÚıÚ‹Ì·ÙÔ˜, ÙË ‰È¿ÚÎÂÈ¿ ÙÔ˘ Î·È ÙËÓ ¤ÁηÈÚË ‰È¿ÁÓˆÛË. ∏ ·ÓÙÈÌÈÎÚԂȷ΋ ·ÁˆÁ‹ ıˆÚÂ›Ù·È ··Ú·›ÙËÙË, ·Ó Î·È ÛÙË ‰È΋ Ì·˜ ÂÚ›ÙˆÛË, ÂÊfiÛÔÓ ¤ÏÂÈ·Ó ÔÈ ÎÏÈÓÈΤ˜ ÂӉ›ÍÂȘ Î·È ÔÈ ‰Â›ÎÙ˜ Ïԛ̈͢ ‰ÂÓ ‹Ù·Ó ¯·Ú·ÎÙËÚÈÛÙÈÎÔ› ÂÓÂÚÁÔ‡˜ ÓfiÛÔ˘, ‰ÂÓ ıˆڋıËΠԢÛÈ҉˘ (3). ∂¿Ó Ô ÂÁοÚÛÈÔ˜ Û‡Ó‰ÂÛÌÔ˜ Â›Ó·È ·Î¤Ú·ÈÔ˜, Ë ˆÁˆÓÔ˚Óȷ΋ ¤ÏÍË ÁÈ· Ï›Á˜ Ë̤Ú˜ Î·È Ë Û˘Ó·ÎfiÏÔ˘ıË ÂÊ·ÚÌÔÁ‹ ÛÎÏËÚÔ‡ ÂÚÈÏ·›ÌÈÔ˘ ÁÈ· 6-8 ‚‰ÔÌ¿‰Â˜, Â›Ó·È Û˘Ó‹ıˆ˜ ·ÚÎÂÙ¿ Î·È Ô‰ËÁÔ‡Ó Û ·Ó¿Ù·ÍË ÙÔ˘ ˘ÂÍ·ÚıÚ‹Ì·ÙÔ˜. ∂¿Ó Ô ÂÁοÚÛÈÔ˜ Û‡Ó‰ÂÛÌÔ˜ ‰ÂÓ Â›Ó·È ·Î¤Ú·ÈÔ˜, Ë ÛÎÂÏÂÙÈ΋ ¤ÏÍË ·Ô‰ÂÈÎÓ‡ÂÙ·È ··Ú·›ÙËÙË, ÂÓÒ Ë ÂÊ·ÚÌÔÁ‹ ÂÓfi˜ ÂȉÈÎÔ‡ ·˘¯ÂÓÈÎÔ‡ ÎˉÂÌfiÓ· Ù‡Ô˘ Minerva ÁÈ· 6-8 ‚‰ÔÌ¿‰Â˜, ıˆÚÂ›Ù·È ·Ó·Áη›· ÁÈ· ÙË Û˘ÁÎÚ¿ÙËÛË ÙÔ˘ ¿ÙÏ·ÓÙ· Î·È ÙËÓ ÂԇψÛË ÙÔ˘ Û˘Ó‰¤ÛÌÔ˘. ∂¿Ó ÙÔ ˘ÂÍ¿ÚıÚËÌ· ‰È·ÚΛ ÂÚÈÛÛfiÙÂÚÔ ·fi 3 Ì‹Ó˜, Û˘ÓÈÛÙ¿Ù·È Ë ·ÚıÚfi‰ÂÛË (ÛÔÓ‰˘ÏÔ‰ÂÛ›·) ÙˆÓ ‰‡Ô ÚÒÙˆÓ ·˘¯ÂÓÈÎÒÓ ÛÔÓ‰‡ÏˆÓ, ÁÈ· ÙËÓ ·ÔÊ˘Á‹ ÛÙÔ Ì¤ÏÏÔÓ ÔÔÈ·Û‰‹ÔÙ Ó¢ÚÔÏÔÁÈ΋˜ ÂÈÏÔ΋˜ (4,5). √ ·ÛıÂÓ‹˜ ·Ú¤ÌÂÈÓ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ÎÏÈÓ‹Ú˘ ÌÂ Û˘Ó¯‹ ˆÁˆÓÔ˚Óȷ΋ ¤ÏÍË, Ï·Ì‚¿ÓÔÓÙ·˜ ÌË-ÛÙÂÚÔÂȉ‹ ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë Ê¿Ú̷η ·fi ÙÔ ÛÙfiÌ·. ªÂÙ¿ 7‹ÌÂÚË ÓÔÛËÏ›·, Ô Ì˘˚Îfi˜ Û·ÛÌfi˜ ˘Ô¯ÒÚËÛÂ, ÙÔ Ú·È‚fiÎÚ·ÓÔ ÂÏ·ÙÙÒıËÎÂ, ÔÈ ÎÈÓ‹ÛÂȘ ÙÔ˘ ·˘¯¤Ó· ¤ÁÈÓ·Ó ÏÈÁfiÙÂÚÔ ÂÒ‰˘Ó˜ Î·È Ô ·ÛıÂÓ‹˜ ÊfiÚÂÛ ̷ϷÎfi ÂÚÈÏ·›ÌÈÔ ÂÍÂÚ¯fiÌÂÓÔ˜ ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ. ªÂÙ¿ 10 Ë̤Ú˜, Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ ·ÛıÂÓÔ‡˜ ›¯Â Ï‹Úˆ˜ ·ÔηٷÛÙ·ı›.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Welinder NR, Hoffmann P, Hakansson S. Pathogenesis of non-traumatic atlanto-axial subluxation (Grisel’s syndrome). Eur Arch Otorhinolaryngol 1997;254:251-254.

2. Wetzel FT, La Rocca H. Grisel’s syndrome. Clin Orthop Relat Res 1989;240:141-152.

3. Yu KK, White DR, Weissler MC, Pillsbury HC. Nontraumatic atlantoaxial subluxation (Grisel syndrome): a rare complication of otolaryngological procedures. Laryngoscope 2003;113(6):1047-1049.

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4. Park SW, Cho KH, Shin YS, Kim SH, Ahn YH, Cho KG, et al. Successful reduction for a pediatric chronic atlantoaxial rotatory fixation (Grisel syndrome) with long-term halter traction: case report. Spine 2005;30:E444-449.

5. Martinez-Lage JF, Martinez Perez M, Fernandez Cornejo V, Poza M. Atlanto-axial rotatory subluxation in children: early management. Acta Neurochir (Wien) 2001;143:1223-1228.

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9-11 πÔ˘Ó›Ô˘ 2006

44o ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ ¶ÏËÚÔÊÔڛ˜: C&C International SA Conventions & Congresses ¶·Ú·‰Â›ÛÔ˘ 16, 151 25 ∞ı‹Ó· ∆ËÏ.: 210 6889 100 Fax: 210 6844 777 E-mail: registrations@cnc.gr Website: www.pediatric2006.gr

ƒfi‰Ô˜, Rodos Palace

10-14 πÔ˘Ó›Ô˘ 2006

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

Vienna, Austria

30 πÔ˘Ó›Ô˘-3 πÔ˘Ï›Ô˘ 2006 45th Annual Meeting of the European Society for Paediatric Endocrinology ¶ÏËÚÔÊÔڛ˜: Congrex Sweden AB ∆ËÏ.: +46 8 459 66 00 Fax: +46 8 661 91 25 E-mail: espe2006@congrex.se Website: www.espe2006.org

Rotterdam, The Netherlands

8-11 πÔ˘Ï›Ô˘ 2006

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

Montreal, Canada

4-7 ™ÂÙÂÌ‚Ú›Ô˘ 2006

30th UMEMPS Congress Contact: Eng Rasha Hasan Tel.: 00963 9274 5746 Fax: 00963 1123 13326 E-mail: info@umemps30-sy.com Website: www.umemps30-sy.com

Damascus, Syria

18-21 ™ÂÙÂÌ‚Ú›Ô˘ 2006 38th Congress of the International Society of Paediatric Oncology Contact: Sandrine Haemmerli Tel.: 41 223 399 648 Fax: 41 223 399 601 E-mail: siop2006@mci-group.com

Geneva, Switzerland

21-23 ™ÂÙÂÌ‚Ú›Ô˘ 2006 12o E˘Úˆ·˚Îfi ™˘Ó¤‰ÚÈÔ Ù˘ ¢ÈÂıÓÔ‡˜ √ÚÁ¿ÓˆÛ˘ ÁÈ· ÙËÓ ˘Á›· ÙˆÓ ∂Ê‹‚ˆÓ Hotel Grecotel Athens Imperial ¶ÏËÚÔÊÔڛ˜: ∫·ı. °ÂÒÚÁÈÔ˜ ÃÚÔ‡ÛÔ˜ ∆ËÏ./Fax: 210 7794023 √ÚÁ¿ÓˆÛË: PRC Congress & Travel, ∆ËÏ.: 210 7711673 Fax: 210 7711289 E-mail: secretariat@prctravel.gr

∞ı‹Ó·

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22-24 ™ÂÙÂÌ‚Ú›Ô˘ 2006 ∂ÈÛÙËÌÔÓÈ΋ ™˘Ó¿ÓÙËÛË °ÓˆÚÈÌ›·˜ ™Î·Êȉȿ ∏Ï›·˜ Ù˘ ∂ÏÏËÓÈ΋˜ ∞η‰ËÌ›·˜ ¶·È‰È·ÙÚÈ΋˜ Ì ÙÔ˘˜ ŒÏÏËÓ˜ ¶·È‰›·ÙÚÔ˘˜ ªÂ ÙË Û˘ÓÂÚÁ·Û›· Ù˘ ∂ÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ ∂Ù·ÈÚ›·˜ •ÂÓÔ‰Ô¯Â›Ô Aldemar Olympian Village ¢ÈÔÚÁ¿ÓˆÛË Û˘Ó‰ڛԢ: Firstevent Prousis 3 st, 10440 Athens, Greece Tel.:+30 2108228950 Fax: +30 2108228901 E-mail: info@firstevent.gr Website: www.firstevent.gr 28 ™ÂÙÂÌ‚Ú›Ô˘ 1 √ÎÙˆ‚Ú›Ô˘ 2006

18Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ¶·È‰›, ∏Ú¿ÎÏÂÈÔ ÀÁ›· Î·È ¶ÔÏÈÙÈÛÌfi˜ ∫Ú‹ÙË ∆Ì‹Ì· π·ÙÚÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘ ¢ÈÔÚÁ¿ÓˆÛË: ∂ÏÏËÓÈ΋ ∂Ù·ÈÚ›· ∫ÔÈÓˆÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ Î·È ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜ ¶ÏËÚÔÊÔڛ˜: ∂ÚÁ·ÛÙ‹ÚÈÔ ÀÁÈÂÈÓ‹˜ Î·È ∂ȉËÌÈÔÏÔÁ›·˜ π·ÙÚÈ΋ ™¯ÔÏ‹, ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ ª. ∞Û›·˜ 75, 11527 ∞ı‹Ó· ∆ËÏ.: 210-7462201 Fax: 210-7462105 ¶ÚÔıÂÛÌ›· ˘Ô‚ÔÏ‹˜ ÂÚÈÏ‹„ÂˆÓ ÂÚÁ·ÛÈÒÓ: 15 πÔ˘Ó›Ô˘ 2006

5-8 √ÎÙˆ‚Ú›Ô˘ 2006

Advances in Paediatric Gastroenterology, Hepatology & Nutrition Turkish - Greek Joint Postgraduate Course endorsed by ESPGHAN ¶ÏËÚÔÊÔڛ˜: ∞ÏÂÍ¿Ó‰Ú· ¶··‰ÔÔ‡ÏÔ˘, ¶Úfi‰ÚÔ˜ ∂∂¶°∏¢ ∆ËÏ.: 210 77 26 355 E-mail: papadop5@otenet.gr ∂ÁÁڷʤ˜: Prof. Ender Pehlivanoglou, Marmara University, Istanbul Fax: 90 212 296 5481 E-mail: e.pehlivanoglu@tnn.net

Cappadocia, Turkey

7-10 √ÎÙˆ‚Ú›Ô˘ 2006

40th Meeting of the European Society of Paediatric Nephrology ¶ÏËÚÔÊÔڛ˜: E.DI.PO. s.r.l.Via della Libertà, 103 - 90143 Palermo ∆ËÏ.: ++39 091 340255 Fax: ++39 091 340440 E-mail: edi.po@tiscali.it Website: www.espn2006.org/

Palermo, Italy

29-31 √ÎÙˆ‚Ú›Ô˘ 2006

International Conference on Childhood Cancer Contact: Dr. Alireza Mosavi-Jarrahi ∆el.: 98 2 122 481 011 Fax: 98 2 122 481 010 E-mail: rmosavi@yahoo.com

Tehran, Iran