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I‰ÈÔÎÙ‹Ù˘ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·© Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 Aı‹Ó· 115 28 TËÏ.: 210 7771 140 210 7771 663 Fax: 210 7758 354 e-mail: hps@ath.forthnet.gr EÙ‹ÛÈ· ™˘Ó‰ÚÔÌ‹: 40 € EȉÈ΢fiÌÂÓÔÈ, ºÔÈÙËÙ¤˜: 20 € ∫ˆ‰ÈÎfi˜ ¢È‡ı˘ÓÛ˘ ∂ÔÙ›·˜ ªª∂: 3889
ISSN 0377-2551
80 ∂¶π™∆√§∏ ¶ƒ√™ ∆∏ ™À¡∆∞•∏
Paediatriki Volume 70 ñ Number 1 ñ January-February 2007
Bimonthly Publication of the Greek Paediatric Society President A. Constantopoulos Editorial Board Editor-in-Chief C. Stefanidis Members P. Augoustides-Savvopoulou H. Antonopoulou A. Vazeou-Gerasimidi G. Varlamis M. Kanariou ∂. Katsarou-Pectasides A. Kattamis S. Kitsiou-Tzeli Ch. Costalos N. Papadopoulos ∞. Papadopoulou A. Siamopoulou-Mavridou M. Tsolia Manuscript Editing Greek Editing F. Mavroidi English Editing S. Nakou Publisher K. Griveas Publishing Coordinator SCIENTIFIC PUBLICATIONS Ltd 1a Pierias St. GR - 144 51, Metamorfosi Tel.: +30 210 87 78 810 Fax: +30 210 87 78 822 Owner Greek Paediatric Society© 92 Michalakopoulou str. GR - 115 28, Athens Tel.: +30 210 7771 140 +30 210 7771 663 Fax: +30 210 7758 354 e-mail: hps@ath.forthnet.gr Annual Subscription All foreign countries: US $ 50
Contents REVIEW ARTICLES 1 Mitochondrial disorders in childhood A. Dinopoulos, A. Papadimitriou 18 High frequency oscillatory ventilation: How it works P. Karagianni, N. Nikolaides ORIGINAL ARTICLES 24 Prevalence of metabolic syndrome in children and adolescents of Crete and its relation to diet M. Linardakis, K. Sarri, G. Bertsias, A. Kafatos, Division of Preventive Medicine and Nutrition 37 The role of mycoplasmal/chlamydial infection in chronic cough in children I.M. Velissariou, N.G. Papadopoulos, M. Giannaki, P. Saxoni-Papageorgiou, D.A. Kafetzis 42 Efficacy of intermittent rectal diazepam prophylaxis for the prevention of febrile seizures E. Pavlidou, M. Tzitiridou, E. Kontopoulos, C. Panteliadis
CASE REPORTS 49 Hypochondroplasia with the mutation N540K of the fibroblast growth factor receptor 3 – FGFR3. A case report V. Aggelakou, G. Niotakis, S. Ilia, A. Bakosi, E. Kokori 54 Nutritional vitamin B12 deficiency in an infant. Dietary errors of a vegan V. Aggelakou, M. Orfanou, G. Niotakis, E. Kokori, I. Charoniti 60 Round atelectasis in adolescent T. Tavladaki, M. Malathraki, G. Drakonakis, I. Saridakis, I. Katsarakis, M. Kladitou, A. Augenaki, A. Nearchou PRACTICAL ISSUES 64 Severe protracted diarrhea of infancy – New aspects C. Costalos 70 Growth curves for Greek children A. Kafatos 75 CLINICAL QUIZ 76 NEWS FROM THE INTERNET Pediatric Blogs ¡. Papadopoulos 80 LETTER TO THE EDITOR
™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 ∞ÓÙˆÓÔÔ‡ÏÔ˘, ∞ı‹Ó· ∞Ó·Ù˘ÍȷΤ˜ ‰È·Ù·Ú·¯¤˜ - ¶ÚÔ‚Ï‹Ì·Ù· Û˘ÌÂÚÈÊÔÚ¿˜
Eleni Antonopoulou, Athens Developmental and Behavioral Pediatrics
¶ÂÚÛÂÊfiÓË ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡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
™ÔÊ›· ∫›ÙÛÈÔ˘-∆˙¤ÏË, ∞ı‹Ó· °ÂÓÂÙÈ΋
Sophia Kitsiou-Tzeli, Athens Genetics
ÃÚ‹ÛÙÔ˜ ∫ÒÛÙ·ÏÔ˜, ∞ı‹Ó· ¡ÂÔÁÓÔÏÔÁ›·
Christos Kostalos, Athens Neonatology
∞ÏÂÍ¿Ó‰Ú· ¶··‰ÔÔ‡ÏÔ˘, ∞ı‹Ó· °·ÛÙÚÂÓÙÂÚÔÏÔÁ›· - ∏·ÙÔÏÔÁ›· - ¢È·ÙÚÔÊ‹
Alexandra Papadopoulou, Athens Gastroenterology - Hepatology - Nutrition
¡ÈÎfiÏ·Ô˜ ¶··‰fiÔ˘ÏÔ˜, ∞ı‹Ó· ∞ÏÏÂÚÁÈÔÏÔÁ›· - ¶Ó¢ÌÔÓÔÏÔÁ›·
Nicos Papadopoulos, Athens Allergology - Pneumonology
∞ÓÙÈÁfiÓË ™È·ÌÔÔ‡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
Peter Hoyer, Essen, Germany
Ellis D. Avner, Milwaukee, USA
Jan Janda, Prague, Czech Republic
Swati Bhave, New Delhi, India
Jan Kimpen, Ultrecht, Netherlands
Alberto Bissot, Panama, Panama
Craig B. Langman, Chicago, USA
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
Max Zach Graz, Austria
Enver Hasanoglu, Ankara, Turkey
Johannes Zschocke, Heidelberg, Germany
Christer Holmberg, Helsinki, Finland
v
∂¶π™∆√§∏ ∞¶√ ∆∏ ™À¡∆∞•∏
EDITORIAL
∞Á·ËÙÔ› Û˘Ó¿‰ÂÏÊÔÈ, ™·˜ ¢¯fiÌ·ÛÙÂ Â˘Ù˘¯ÈṲ̂ÓÔ ÙÔÓ Î·ÈÓÔ‡ÚÁÈÔ ¯ÚfiÓÔ Î·È Û·˜ ¢¯·ÚÈÛÙԇ̠ÁÈ· ÙË Û˘Ó¯‹ Û·˜ ˘ÔÛÙ‹ÚÈÍË ÛÙȘ ÚÔÛ¿ıÂȘ ÁÈ· ·Ó·‚¿ıÌÈÛË Ù˘ “¶·È‰È·ÙÚÈ΋˜”. π‰È·›ÙÂÚ· ¢¯·ÚÈÛÙԇ̠ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜, ÙÔ˘˜ ÎÚÈÙ¤˜ ÙˆÓ ÂÚÁ·ÛÈÒÓ Î·È Ù· ̤ÏË Ù˘ ¢ÈÂıÓÔ‡˜ ™˘ÓÙ·ÎÙÈ΋˜ ∂ÈÙÚÔ‹˜. ∏ Û˘ÌÌÂÙÔ¯‹ ÙˆÓ ‰È·ÎÂÎÚÈÌ¤ÓˆÓ ·˘ÙÒÓ Û˘Ó·‰¤ÏÊˆÓ ÛÙËÓ ‰È·‰Èηۛ· ¤Î‰ÔÛ˘ ı· Û˘ÓÙÂϤÛÂÈ ÛÙÔ Ó· ·ÔÎÙ‹ÛÂÈ Ë “¶·È‰È·ÙÚÈ΋” ‰ÈÂıÓ¤˜ ·ÚÔ˜. ™·˜ ˘ÂÓı˘Ì›˙Ô˘Ì fiÙÈ Ë ˘Ô‚ÔÏ‹ ÙˆÓ ÂÚÁ·ÛÈÒÓ Û·˜ ÌÔÚ› Ó· Á›ÓÂÙ·È Î·È ÛÙ· ∞ÁÁÏÈο. ∂›Û˘ ÌËÓ Í¯ӿÙÂ, fiÙÈ ÌÔÚ›Ù ӷ ‚Ú›Ù ÙȘ ‰ËÌÔÛȇÛÂȘ Ù˘ “¶·È‰È·ÙÚÈ΋˜” ÛÙËÓ ÈÛÙÔÛÂÏ›‰· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ªÂ ıÂṲ́˜ ¢¯¤˜ °È· ÙË ™˘ÓÙ·ÎÙÈ΋ ∂ÈÙÚÔ‹
∫ˆÓÛÙ·ÓÙ›ÓÔ˜ ™ÙÂÊ·Ó›‰Ë˜ ¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘
Dear colleagues, We would like to wish you a Happy New Year. On this occasion, we would like to thank all of you for supporting the efforts for improvement of “Paediatriki”. Especially we would like to thank the authors, the reviewers and the members of the International Editorial Board. We believe that the participation of this distinguished group of colleagues will contribute to our effort for “Paediatriki” to achieve a world-class level. We would like to remind you that papers may be submitted in English. Finally do not forget that you can find past issues of “Paediatriki” at the web page of the journal. Warm greetings √n behalf of the Editorial Board Constantinos Stefanidis Editor-in-Chief
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√¢∏°π∂™ ¶ƒ√™ ∆√À™ ™À°°ƒ∞º∂π™ A. °ÂÓÈΤ˜ ¶ÏËÚÔÊÔڛ˜ ∆Ô ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ΋” Â›Ó·È Ë ÂÈÛÙËÌÔÓÈ΋ ¤Î‰ÔÛË Î·È È‰ÈÔÎÙËÛ›· Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜, Ô˘ ‰È·Ó¤ÌÂÙ·È ÛÙ· ̤ÏË Ù˘. Œ¯ÂÈ ˆ˜ ‚·ÛÈÎÔ‡˜ ÛÙfi¯Ô˘˜ ÙËÓ ·ÔÙ‡ˆÛË ÙÔ˘ ·È‰È·ÙÚÈÎÔ‡ ÂÈÛÙËÌÔÓÈÎÔ‡ ¤ÚÁÔ˘ Î·È ÙË Û˘Ó¯‹ ÂÓË̤ڈÛË ÙˆÓ ¶·È‰È¿ÙÚˆÓ. °È· ÙÔ ÛÎÔfi ·˘Ùfi ‰¤¯ÂÙ·È ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÔÈÎÈÏ›· ¿ÚıÚˆÓ Î·È Û˘ÁÎÂÎÚÈ̤ӷ: 1. ÕÚıÚ· Û‡ÓÙ·Í˘ (ÌÂÙ¿ ·fi ÚfiÛÎÏËÛË Ù˘ ™˘ÓÙ·ÎÙÈ΋˜ EÈÙÚÔ‹˜). 2. ∞Ó·ÛÎÔ‹ÛÂȘ. 3. µÚ·‚Â˘Ì¤Ó˜ ÂÚÁ·Û›Â˜. 4. ∂Ú¢ÓËÙÈΤ˜ ÌÂϤÙ˜. 5. ∫ÏÈÓÈο ∫Ô˘›˙. 6. ∂ÈÏÂÁ̤Ó˜ Û˘˙ËÙ‹ÛÂȘ ÛÙÚÔÁÁ˘ÏÒÓ ÙÚ·Â˙ÒÓ. 7. ∂›Î·ÈÚ· ı¤Ì·Ù·. 8. £¤Ì·Ù· Âη›‰Â˘Û˘ Î·È ÔÚÁ¿ÓˆÛ˘ ˘Á›·˜. 9. ∂ӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ. 10. ™‡ÓÙÔÌ· Ó¤·. 11. µÚ·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ. 12. ∂ÈÛÙÔϤ˜ ÚÔ˜ ÙË Û‡ÓÙ·ÍË. 13. ¶ÂÚÈÏ‹„ÂȘ Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜. 14. ∞Ó·ÎÔÈÓÒÛÂȘ ÚÔÛ¯ÒÓ Û˘Ó‰ڛˆÓ Î·È ÂÈÛÙËÌÔÓÈÎÒÓ ÂΉËÏÒÛÂˆÓ 15. ∞Ó·ÊÔÚ¤˜ Î·È ÎÚÈÙÈ΋ Ó¤ˆÓ ÂΉfiÛˆÓ, ÂÏÏËÓÈÎÒÓ Î·È Í¤ÓˆÓ, ·È‰È·ÙÚÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜. H ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ó· ‰ËÌÔÛȇÂÈ ¿ÚıÚ· Ì ȉȷ›ÙÂÚÔ ÂÈÛÙËÌÔÓÈÎfi ÂӉȷʤÚÔÓ, ηıÒ˜ Î·È ¿ÚıÚ· Ì ›ηÈÚ· ı¤Ì·Ù· ¯ˆÚ›˜ Ó· ÙËÚÂ›Ù·È Ë ÛÂÈÚ¿ ˘Ô‚ÔÏ‹˜. ∂›Û˘, ÌÔÚ› 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 ÂÈÛÙÔÏÒÓ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ‰ËÌÔÛÈÂ˘Ì¤Ó· ÂÈÛÙËÌÔÓÈο ¿ÚıÚ· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ™ÙȘ ÂÚÁ·Û›Â˜ Ì ›ηÈÚ· ı¤Ì·Ù· Ú¤ÂÈ Ó· ·Ó·ÁÚ¿ÊÂÙ·È ÛÙËÓ ÚÒÙË ÛÂÏ›‰· Ë ÚfiıÂÛË ÙˆÓ Û˘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. ∂ÈϤÔÓ, ÛÙËÓ ÂÚÁ·Û›· ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹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 ÂÎÚÔÛÒˆÓ 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 ÂÈÙÚÔ‹ ∏ıÈ΋˜ Î·È ¢ÂÔÓÙÔÏÔÁ›·˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ. OÈ ·fi„ÂȘ Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ Ô˘ ‰È·Ù˘ÒÓÔÓÙ·È ÛÙȘ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ‰ÂÓ ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ··Ú·›ÙËÙ· ·˘Ù¤˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. H ηٷ¯ÒÚÈÛË ‰È·ÊËÌ›ÛÂˆÓ ÛÙÔ ÂÚÈÔ‰ÈÎfi ‰ÂÓ ˘Ô‰ËÏÒÓÂÈ ÔˆÛ‰‹ÔÙ ¤ÁÎÚÈÛË ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘˜ ·fi ÙËÓ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·, ÙË ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‹ ·fi ÙÔÓ EΉfiÙË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ŸÏ˜ ÔÈ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ıˆÚÔ‡ÓÙ·È È‰ÈÔÎÙËÛ›· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ “¶·È‰È·ÙÚÈ΋” Î·È Ë ÔÏÈ΋ ‹ ÌÂÚÈ΋
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·Ó·‰ËÌÔÛ›Â˘Û‹ ÙÔ˘˜ ÂÈÙÚ¤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 Ôԛ˜ ÙÚÔÔÔÈ‹ıËÎ·Ó ÚfiÛÊ·Ù· Î·È ‰ËÌÔÛȇÔÓÙ·È ÛÙȘ ‰È¢ı‡ÓÛÂȘ: http://www.icmje.org Î·È http://www.icmje.org/icmje.pdf TÔ Û‡ÓÔÏÔ Ù˘ ÂÚÁ·Û›·˜ (Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ ÙˆÓ ÈÓ¿ÎˆÓ Î·È ÙˆÓ Ù›ÙÏˆÓ ÙˆÓ ÂÈÎfiÓˆÓ) Ú¤ÂÈ Ó· Â›Ó·È Ù˘ˆÌ¤ÓÔ ÛÙË ÌÈ· fi„Ë Ï¢ÎÔ‡ ¯·ÚÙÈÔ‡ ÌÂÁ¤ıÔ˘˜ A4 (21x29,7 cm), Ì ‰ÈÏfi ‰È¿ÛÙËÌ· ÌÂٷ͇ ÙˆÓ ÁÚ·ÌÌÒÓ Î·È ÂÚÈıÒÚÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 2,5 cm ÛÙȘ ‰‡Ô Ï¢ڤ˜. TÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ¤¯ÂÈ ÙËÓ ·ÎfiÏÔ˘ıË ‰È¿Ù·ÍË: 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 ÂȉÔÙ‹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˜ ÂÚÈÙÒÛÂȘ Û 1000-1500 ϤÍÂȘ. ñ ÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ Û 1000-1500 ϤÍÂȘ. ñ ÙȘ ÂÈÛÙÔϤ˜ Û 250-500 ϤÍÂȘ.
™ÂÏ›‰· Ù›ÙÏÔ˘ ¶Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ: ñ ÙÔÓ Ù›ÙÏÔ (<14 ϤÍÂȘ), ηıÒ˜ Î·È ÙÔÓ ‚Ú·¯‡ Ù›ÙÏÔ (<5 ϤÍÂȘ) ÙÔ˘ ¿ÚıÚÔ˘. ¢ÂÓ ÂÈÙÚ¤ÔÓÙ·È Û˘ÓÙÌ‹ÛÂȘ. ñ ÙÔ fiÓÔÌ· Î·È ÙÔ ÂÒÓ˘ÌÔ Î¿ıÂ Û˘ÁÁڷʤ· ÛÙËÓ ÔÓÔÌ·ÛÙÈ΋. ñ ÙÔ ÂÈÛÙËÌÔÓÈÎfi ΤÓÙÚÔ (›‰Ú˘Ì·, ÎÏÈÓÈ΋, ÂÚÁ·ÛÙ‹ÚÈÔ) ·fi fiÔ˘ ÚÔ¤Ú¯ÂÙ·È Ë ÂÚÁ·Û›·. ™Â ÂÚ›ÙˆÛË ¤ÏÏÂȄ˘ Û˘ÓÂÚÁ·Û›·˜ ÌÂ Û˘ÁÎÂÎÚÈ̤ӷ ΤÓÙÚ·, Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÂÙ·È Ë È‰ÈfiÙËÙ· ÙÔ˘/ÙˆÓ Û˘ÁÁڷʤ·/ˆÓ (.¯. ȉÈÒÙ˘ ·È‰›·ÙÚÔ˜) Î·È Ô ÙfiÔ˜ ‰È·ÌÔÓ‹˜ ÙÔ˘/ÙÔ˘˜. ñ ÙËÓ Ï‹ÚË ‰È‡ı˘ÓÛË, e-mail Î·È ÙÔ ÙËϤʈÓÔ ÙÔ˘ Û˘ÁÁڷʤ· Ì ÙÔÓ ÔÔ›Ô Á›ÓÂÙ·È Ë ·ÏÏËÏÔÁÚ·Ê›·.
¶ÂÚÈÏ‹„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˘ÌÂÚ¿ÛÌ·Ù· Ù˘ ÌÂϤÙ˘. ñ £· Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ 200 ϤÍÂȘ, fï˜ ‰ÂÓ ı· Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÂÈ ÙȘ 250 ϤÍÂȘ. ñ ∏ ÂÚ›ÏË„Ë ÙˆÓ ÂÚÁ·ÛÈÒÓ Ú¤ÂÈ Ó· Â›Ó·È ‰ÔÌË̤ÓË ÛÙȘ ÂÍ‹˜ ·Ú·ÁÚ¿ÊÔ˘˜: ÂÈÛ·ÁˆÁ‹, ̤ıÔ‰ÔÈ, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘ÌÂÚ¿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˘ÌÂÚ¿ÛÌ·Ù· (conclusions). H ÂÚ›ÏË„Ë ÛÙ· ·ÁÁÏÈο ‰ÂÓ Ú¤ÂÈ Ó· ‰È·Ê¤ÚÂÈ ·fi ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ÛÙ· ÂÏÏËÓÈο.
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K¿Ùˆ ·fi ÙËÓ ÂÏÏËÓÈ΋ Î·È ÙËÓ ·ÁÁÏÈ΋ ÂÚ›ÏË„Ë ÛËÌÂÈÒÓÔÓÙ·È ÙÚÂȘ ¤ˆ˜ ¤ÓÙ ϤÍÂȘ ÎÏÂȉȿ Ô˘ ı· ¯ÚËÛÈÌÔÔÈËıÔ‡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·Ô̤˜ Î·È ÙÔÓ ÛÎÔfi Ù˘ ÂÚÁ·Û›·˜. H ÂÚÈÁÚ·Ê‹ ÙˆÓ ÌÂıfi‰ˆÓ Ú¤ÂÈ Ó· Â›Ó·È ·ÎÚÈ‚‹˜ Î·È ·ÚÎÂÙ¿ ÏÂÙÔÌÂÚ‹˜, ÒÛÙ ӷ ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó··Ú·ÁˆÁ‹ ÙÔ˘˜ ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜. ∂›Û˘, Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ì ÏÂÙÔ̤ÚÂȘ 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˘ÌÂÚ¿ÛÌ·Ù· Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙËÓ ÂÚÁ·Û›·, ÙË ÛËÌ·Û›· Ô˘ ÌÔÚ› Ó· ¤¯Ô˘Ó Î·È ÙËÓ Èı·Ó‹ Û˘Û¯¤ÙÈÛ‹ ÙÔ˘˜ Ì ·Ú·ÙËÚ‹ÛÂȘ ¿ÏÏˆÓ ÂÚ¢ÓËÙÒÓ. OÈ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û‡ÓÙÔÌË ÂÈÛ·ÁˆÁ‹, ÂÚÈÁÚ·Ê‹ Ù˘ ÂÚ›ÙˆÛ˘ Î·È ‚Ú·¯Â›· Û˘˙‹ÙËÛË, Ì ¤ÌÊ·ÛË ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË. T· ˘fiÏÔÈ· ı¤Ì·Ù· ¤¯Ô˘Ó ÂχıÂÚË ‰ÔÌ‹ ηٿ ÙËÓ ÎÚ›ÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ÙÔ˘˜. ∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢) ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Ù¤ÏÔ˜ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Î·È ÚÈÓ ·fi ÙȘ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜.
TÈ̤˜ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿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 ÂÈ‚ÏËı› ‰ÈÂıÓÒ˜, ‰ËÌÔÛȇÔÓÙ·È Û οı Ù‡¯Ô˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ™‡ÓıÂÙÔÈ Î·È Ì·ÎÚÔÛÎÂÏ›˜ fiÚÔÈ Ô˘ ·ӷϷ̂¿ÓÔÓÙ·È Û˘¯Ó¿ ÛÙÔ Î›ÌÂÓÔ ÌÔÚÔ‡Ó Ó· ·ÓÙÈηı›ÛÙ·ÓÙ·È ·fi Û˘ÓÙÔÌÔÁڷʛ˜ Ô˘ ÂÂÍËÁÔ‡ÓÙ·È ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ Û ÂȉÈÎfi ηٿÏÔÁÔ, Ô ÔÔ›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·Ô̤˜ ™ÙÔ ÙÌ‹Ì· Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ ηٷ¯ˆÚÔ‡ÓÙ·È fiϘ ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ™ÙÔ Î›ÌÂÓÔ, ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ ·Ó·Ê¤ÚÔÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Û ·Ú¤ÓıÂÛË. OÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ ‰ÂÓ Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÔ˘Ó: ñ ÙȘ 70 ÛÙȘ ·Ó·ÛÎÔ‹ÛÂȘ. ñ ÙȘ 30 ÛÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜. ñ ÙȘ 12 ÛÙ· ›ηÈÚ· ı¤Ì·Ù· Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ. ñ ÙȘ 5 ÛÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ Î·È ÙȘ ÂÈÛÙÔϤ˜. H Û‡ÓÙ·ÍË ÙˆÓ ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ Á›ÓÂÙ·È Û‡Ìʈӷ Ì ÙȘ ÚfiÛÊ·Ù· ÙÚÔÔÔÈË̤Ó˜ ÚԉȷÁڷʤ˜ Ù˘ International Committee of Medical Journal 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·ÔÌÒÓ I. ¶∂ƒπO¢π∫∞ AÓ ÔÈ Û˘ÁÁÚ·Ê›˜ Â›Ó·È ¤ˆ˜ ¤ÍÈ, ·Ó·ÁÚ¿ÊÔÓÙ·È fiÏÔÈ, ÂÓÒ ·Ó Â›Ó·È ÂÙ¿ ‹ ÂÚÈÛÛfiÙÂÚÔÈ, ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ 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·: ›‰Ú·ÛË ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· 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. ™˘ÌÏËڈ̷ÙÈÎfi 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. ¶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. ¢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. ¢È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹: ¶··‰fiÔ˘ÏÔ˜ Ã. ∏ ıÂڷ›· ÙÔ˘ ÛÙÚ·‚ÈÛÌÔ‡ [‰È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹]. ∞ı‹Ó·: ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ; 1979. 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.
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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 πÛÙÔÛÂÏ›‰Â˜: Cancer-Pain.org [Webpage, Internet]. New York: Association of Cancer Online Resources, Inc.; 2002: http://www.cancerpain.org/
°. ÀÔ‚ÔÏ‹ Î·È ¢ËÌÔÛ›Â˘ÛË ÙˆÓ ∂ÚÁ·ÛÈÒÓ ŸÏ˜ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ˘Ô‚¿ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó Î·È Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ‰ÈÛΤٷ ‹ CD, ηıÒ˜ Î·È ·fi ÂÈÛÙÔÏ‹, Ë ÔÔ›· ı· Û˘Ó˘ÔÁÚ¿ÊÂÙ·È ·fi fiÏÔ˘˜ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜, ÛÙËÓ ÔÔ›· ı· ‰ËÏÒÓÂÙ·È fiÙÈ Ë ÂÚÁ·Û›· ‰ÂÓ ¤¯ÂÈ ÂÓ Ì¤ÚÂÈ ‹ ÂÍ ÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚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΋”. ∂ÈϤÔÓ, ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢. EÊfiÛÔÓ Ë ÂÚÁ·Û›· Á›ÓÂÈ ·Ô‰ÂÎÙ‹, ÙÔ ‰ÈÔÚıˆÌ¤ÓÔ Û‡Ìʈӷ Ì ÙȘ ˘Ô‰Â›ÍÂȘ ÙˆÓ ÎÚÈÙÒÓ Î·È ÂÎ Ó¤Ô˘ ‰·ÎÙ˘ÏÔÁÚ·ÊË̤ÓÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ˘Ô‚¿ÏÂÙ·È ÛÙË ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹, Û˘Óԉ¢fiÌÂÓÔ ·fi ‰ÈÛΤٷ ‹ CD Ô˘ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÚÁ·Û›· Û ÚfiÁÚ·ÌÌ· Word, ηıÒ˜ Î·È ÂÈÛÙÔÏ‹ fiÔ˘ Ó· ·Ó·Ê¤ÚÔÓÙ·È ·Ó·Ï˘ÙÈο ÔÈ ÙÚÔÔÔÈ‹ÛÂȘ ‹ ÔÈ ·ÓÙÈÚÚ‹ÛÂȘ ÛÙȘ ÚÔÙ¿ÛÂȘ ÙˆÓ ÎÚÈÙÒÓ. H ηı˘ÛÙ¤ÚËÛË Ù˘ ·ӢԂÔÏ‹˜ ÙÔ˘ ÙÚÔÔÔÈË̤ÓÔ˘ ÎÂÈ̤ÓÔ˘ ¤Ú·Ó ÙˆÓ 30 ËÌÂÚÒÓ Û˘Ó¿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 ÂÈÛÙÚ¤ÊÔÓÙ·È. MÔÚ› Ó· ÂÈÛÙÚ·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 ÙÚÈÏÔ‡Ó ÛÙË ‰È‡ı˘ÓÛË: ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹, EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›· Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92, 115 28 Aı‹Ó·
¶›Ó·Î˜ Î·È EÈÎfiÓ˜ AÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó (¤Ó· ÚˆÙfiÙ˘Ô Î·È ‰‡Ô ʈÙÔ·ÓÙ›ÁÚ·Ê·). ¶Ú¤ÂÈ Ó· ¤¯Ô˘Ó Ï¿ÙÔ˜ ›ÛÔ Ì ÙÔ Ï¿ÙÔ˜ ÙÔ˘ ÌÔÓfiÛÙËÏÔ˘ (7,5 cm) ‹ Ì ÙÔ Ï¿ÙÔ˜ fiÏ˘ Ù˘ 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 ÂÂÍ‹ÁËÛË ÙˆÓ Û˘ÓÙÌ‹ÛÂˆÓ ÛÙÔ Î¿Ùˆ ̤ÚÔ˜. ™ÙÔ˘˜ ›Ó·Î˜ Ú¤ÂÈ Ó· ·ÔʇÁÔÓÙ·È ÔÈ Î¿ıÂÙ˜ ÁÚ·Ì̤˜. ŸÏÔ ÙÔ ·ÂÈÎÔÓÈÛÙÈÎfi ˘ÏÈÎfi (Û¯‹Ì·Ù·, ‰È·ÁÚ¿ÌÌ·Ù·, ʈÙÔÁڷʛ˜, Î.Ï.) ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ÂÈÎfiÓ˜ Î·È ı· Ú¤ÂÈ Ó· Â›Ó·È ¿ÚÈÛÙ˘ ÔÈfiÙËÙ·˜. ™ÙÔ ›Ûˆ ̤ÚÔ˜ 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 ÔÔ›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.
¶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 ÂÒÓ˘ÌÔ Û˘ÁÁڷʤˆÓ (ÔÏÔÁڿʈ˜) Á. ÂÈÛÙËÌÔÓÈÎfi/¿ ΤÓÙÚÔ/· fiÔ˘ ¤ÁÈÓÂ Ë ÂÚÁ·Û›· ‰.fiÓÔÌ·, ‰È‡ı˘ÓÛË Î·È ÙËϤʈÓÔ Û˘ÁÁڷʤ· ÁÈ· ·ÏÏËÏÔÁÚ·Ê›·. 5. ¶ÂÚ›ÏË„Ë ÂÏÏËÓÈ΋-·ÁÁÏÈ΋, ‰ÔÌË̤ÓË ÛÙȘ ÂÍ‹˜ ·Ú·ÁÚ¿ÊÔ˘˜: ÂÈÛ·ÁˆÁ‹, ̤ıÔ‰ÔÈ, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘ÌÂÚ¿ÛÌ·Ù·, ηıÒ˜ Î·È Ï¤ÍÂȘ ÎÏÂȉȿ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·). 6. ∫·Ù¿ÏÔÁÔ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·). 7. ∫›ÌÂÓÔ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·). 8. ∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢). 9. µÈ‚ÏÈÔÁÚ·Ê›· (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·). 10. ¶›Ó·Î˜ (Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó. 11. ∂ÈÎfiÓ˜, Ì ‚¤ÏÔ˜ ÛÙÔ ›Ûˆ ̤ÚÔ˜ ÙÔ ÔÔ›Ô Ó· ‰Â›¯ÓÂÈ ÚÔ˜ Ù· ¿ӈ, ·ÚÈıÌË̤Ó˜, ÂȘ ÙÚÈÏÔ‡Ó. 12. ∆›ÙÏÔ˘˜ ÂÈÎfiÓˆÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó.
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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
ABBREVIATIONS
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 ÏÂÙfi ¯ÈÏÈÔÛÙfiÌÂÙÚÔ ÁÚ·ÌÌÔÌfiÚÈÔ ·ÚÈıÌfi˜ ÌË ÛËÌ·ÓÙÈÎfi ÔÛÌÒÏÈÔ Èı·ÓfiÙËÙ· ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË ÛÙ·ıÂÚfi ÛÊ¿ÏÌ· ‰Â˘ÙÂÚfiÏÂÙÔ ÌÔÓ¿‰·
™˘Ó‰˘·˙fiÌÂÓ· ÚÔı¤Ì·Ù·
Combining prefixes
tera- (1012) 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)
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
INSTRUCTIONS TO AUTHORS A. General Information The Greek Paediatric Society is the owner of “Paediatriki”, its official scientific journal, which is distributed to its members. Its objectives are the publication of paediatric scientific work and the continuing education of paediatricians. For this purpose, it publishes a variety of articles, and in particular: 1. Editorials (upon invitation by the Editorial Board). 2. Review articles. 3. Award-winning articles. 4. Original articles. 5. Clinical Quiz. 6. Round tables. 7. Current issues. 8. Issues of healthcare management and education. 9. Case reports. 10. News. 11. Brief reports. 12. Letters to the editor. 13. Abstracts. 14. Future congresses and events. 15. Book reviews. The Editorial Board reserves the right to publish articles of special scientific interest and articles on current issues without observing submission order. In addition it publishes upon decision original papers presented at the Annual Paediatric Conference, presentations of special interest - in whole or in part, and letters - in whole or in part - referring to scientific articles published in the journal. Regarding papers on current issues, the author’s request for immediate publication should be quoted on the first page. The Editorial Board reserves the right to accept such papers for immediate publication. All manuscripts should not have been published previously, in whole or in part, and not be under consideration by another publication. Manuscripts should acknowledge any funding, sponsorship or other financial support. All clinical research should have been conducted following informed consent of participants or of their legal representatives according to the Declarations of Helsinki and Tokyo. In addition, the US National Institute of Health guide for the care and use of laboratory animals (DHEW Publication, NIH, 80-23) should have been observed. Clinical trials should have been approved by the Ethics Committee of the Hospital. Authors’ opinions and conclusions expressed in the published papers do not necessarily reflect those of the journal. The Greek Paediatric Society, the Editorial Board and the Publisher of the journal do not necessarily approve the content of the advertisements appearing in the journal. The copyright of all published papers is held by “Paediatriki” and their reproduction in whole or in part is authorized only following written consent of the journal.
B. Manuscript Preparation “Paediatriki” suggests compliance with the “Uniform Requirements for Manuscripts Submitted to Biomedical Journals”, recently modified and published on the websites: http://www.icmje.org and http://www.icmje.org/icmje.pdf The entire paper (including figure legends and tables)
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should be typed on one side of blank paper format A4 (21x29.7 cm), double line spacing and minimum indent 2.5 cm on both sides. The paper should have the following structure: title page, short title, abstract in Greek and English, list of abbreviations, text, acknowledgements and quoting of grants, sponsorships or other financial support sources, references, tables, figures, figure legends. Each of these sections should be started on a new page. Pages should be numbered consecutively, beginning with the title page. Text length shall be: ñ review articles 2000-3000 words; ñ original articles 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 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. 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. 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. Book or monograph: Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck. 3rd ed. New York: Oxford University Press; 1990. Publication in a volume of proceedings: Bauer AW. The two definitions of bacterial resistance. In: Smith AJ, Rogers CA, eds. Proceedings of the Third 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.cancerpain.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.
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.
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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.
“√È ÂÚ›ÔÏÔÈ Ù˘ ÂÈÚ‹Ó˘” Diana Marcela Cortes, 10 ¯ÚÔÓÒÓ, ∫ÔÏÔÌ‚›· ¶·È‰› Ì ÌÂÚÈ΋ fiÚ·ÛË “∆· Ù˘ÊÏ¿ ·È‰È¿ ˙ˆÁÚ·Ê›˙Ô˘Ó”, ÕÓÓ· §·Ô˘Ù¿ÚË°ÎÚÈÙ˙¿Ï·, ∞ı‹Ó· 2006
“The patrol of the Peace”, Diana Marcela Cortes, 10 years old, Colombia Partially blind child “Blind children paint”, Anna Laoutari-Gritzala, Athens 2006
∞¡∞™∫√¶∏™∏
REVIEW ARTICLE
1
ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ∞. ¡ÙÈÓfiÔ˘ÏÔ˜1, ∞. ¶··‰ËÌËÙÚ›Ô˘2 ¶ÂÚ›ÏË„Ë: ∆· ÌÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· ·ÔÙÂÏÔ‡Ó ÌÈ· ÔÌ¿‰· ÛÔÚ·‰ÈÎÒÓ ‹ ÎÏËÚÔÓÔÌÔ‡ÌÂÓˆÓ ÓÔÛËÌ¿ÙˆÓ Ù· ÔÔ›· ÚÔηÏÔ‡ÓÙ·È ·fi ÌÂÙ·ÏÏ¿ÍÂȘ ÁÔÓȉ›ˆÓ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ ‹ ˘ÚËÓÈÎÔ‡ DNA Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ÚˆÙ½Ó˜ ··Ú·›ÙËÙ˜ ÁÈ· ÙË ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÌÈÙÔ¯ÔÓ‰Ú›Ô˘. ∏ ÈÔ Û˘¯Ó‹ ‰È·Ù·Ú·¯‹ ·ÊÔÚ¿ ÙËÓ ÔÍÂȉˆÙÈ΋ ʈÛÊÔÚ˘Ï›ˆÛË Î·È ÔÊ›ÏÂÙ·È Û ·Ó·Ú΋ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ Û˘ÌÏÂÁÌ¿ÙˆÓ (I-V) Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Ì ·ÔÙ¤ÏÂÛÌ· ÙË ÌÂȈ̤ÓË ÂÓ‰Ô΢ÙÙ·ÚÈ΋ ·Ú·ÁˆÁ‹ ÂÓ¤ÚÁÂÈ·˜. ∂ÈÚÔÛı¤Ùˆ˜ Ë ÚÔÛ‚ÔÏ‹ ¿ÏÏˆÓ ÏÂÈÙÔ˘ÚÁÈÒÓ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰Ú›Ô˘, fiˆ˜ Ù˘ ÚˆÙÂ˚ÓÈ΋˜ Û‡ÓıÂÛ˘, Ù˘ ‰È·ÌÂÌ‚Ú·ÓÈ΋˜ ÌÂÙ·ÊÔÚ¿˜, Ù˘ Û˘Ó·ÚÌÔÏfiÁËÛ˘ ÙˆÓ Û˘ÌÏÂÁÌ¿ÙˆÓ, Ù˘ Û˘ÓÙ‹ÚËÛ˘ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA ÌÔÚ› Ó· ÚÔηϤÛÔ˘Ó ÌÈÙÔ¯ÔÓ‰Úȷ΋ ÓfiÛÔ. ¶ÚfiÎÂÈÙ·È ÁÈ· ÔÏ˘Û˘ÛÙËÌ·ÙÈο ÓÔÛ‹Ì·Ù· Ì ÂΉËÏÒÛÂȘ ·fi ÙÔ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Î·È Ó¢ÚÔÌ˘˚Îfi Û‡ÛÙËÌ·, ÙÔ˘˜ ÔÊı·ÏÌÔ‡˜, ÙËÓ Î·Ú‰È¿, ÙÔ˘˜ ÓÂÊÚÔ‡˜, ÙÔÓ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ, ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi Î·È ÂÓ‰ÔÎÚÈÓÈÎfi Û‡ÛÙËÌ·. ∏ ‰È·ÁÓˆÛÙÈ΋ ÚÔۤϷÛË Â›Ó·È ‰‡ÛÎÔÏË ÏfiÁˆ Ù˘ ¿Ù˘Ë˜ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜, ·Ô˘Û›·˜ ·ıÔÁÓˆÌÔÓÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ Î·È ÂÓ‰ÔÁÂÓÒÓ ÂÚÈÔÚÈÛÌÒÓ ÛÙËÓ ·ÍÈÔÏfiÁËÛË ÙˆÓ ‚ÈÔ¯ËÌÈÎÒÓ Î·È ÌÔÚÈ·ÎÒÓ ÂÍÂÙ¿ÛˆÓ. °È· Ù· ·È‰È¿ ¤¯Ô˘Ó ÚÔÙ·ı› ÙÚÔÔÔÈË̤ӷ ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· Ù· ÔÔ›· ÂÓۈ̷ÙÒÓÔ˘Ó ÎÏÈÓÈο, ‚ÈÔ¯ËÌÈο, ÂÓ˙˘ÌÈο, ÈÛÙÔ·ıÔÏÔÁÈο Î·È ÌÔÚȷο ‰Â‰Ô̤ӷ. ∏ ÌÔÚȷ΋ ‰È¿ÁÓˆÛË Ú¤ÂÈ Ó· ·ÔÙÂÏ› ÙÔÓ ÙÂÏÈÎfi ÛÙfi¯Ô ÁÈ·Ù› ÌfiÓÔÓ ¤ÙÛÈ ÌÔÚ› Ó· Â›Ó·È ‰˘Ó·Ù‹ Ë ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï‹ Î·È Î·ıÔ‰‹ÁËÛË. ∞Ó Î·È ·ÈÙÈÔÏÔÁÈ΋ ıÂڷ›· ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ‰ÂÓ ˘¿Ú¯ÂÈ, ÔÏÏ¿ ‰Â˘ÙÂÚÔ·ı‹ ÚÔ‚Ï‹Ì·Ù· ¯Ú‹˙Ô˘Ó ·ÓÙÈÌÂÙÒÈÛ˘. ∏ ·Ó·ÛÎfiËÛË ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÈÔ ÚfiÛÊ·ÙË ÁÓÒÛË Û¯ÂÙÈο Ì ÙÔ ÁÂÓÂÙÈÎfi ˘fi‚·ıÚÔ, ·ıÔÁÂÓÂÙÈÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜, ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ, ‰È·ÁÓˆÛÙÈ΋ ÚÔۤϷÛË Î·È ıÂڷ¢ÙÈ΋ ÛÙÚ·ÙËÁÈ΋ ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ.
1 Cincinnati Children’s Hospital Medical Center, Dept of Pediatric Neurology 2 ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô §¿ÚÈÛ·˜, ∆Ì‹Ì· ¡Â˘ÚÔÏÔÁ›·˜ AÏÏËÏÔÁÚ·Ê›·: ∞ÚÁ‡Ú˘ ¡ÙÈÓfiÔ˘ÏÔ˜ argidino@yahoo.com
§¤ÍÂȘ ÎÏÂȉȿ: ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù·, ÁÂÓÂÙÈ΋, ÔÏ˘Û˘ÛÙËÌ·ÙÈο ÓÔÛ‹Ì·Ù·, ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË.
Mitochondrial disorders in childhood A. Dinopoulos1, A. Papadimitriou2 Abstract: Mitochondrial disorders are caused by mutations, either sporadic or inherited, in nuclear or mitochondrial DNA genes which encode proteins essential for the mitochondrial function. The most common level of dysfunction is primary or secondary compromise of respiratory chain activity, leading to impaired oxygen utilization and reduced intracellular energy production. Other mitochondrial functions can be impaired, such as intergenomic signalling, assembling, transport and mitochondrial motility. Mitochondrial disorders are usually multisystem disorders involving predominantly tissues or organs with high oxygen and energy demands, such as skeletal muscle, myocardium, brain, eyes, ears, kidneys, intestines and bone marrow. Several mitochondrial syndromes have been described, but many patients and particularly children, who meet the morphological, biochemical and/or molecular criteria for a mitochondrial disorder, do not fall into a syndrome phenotype. During childhood the clinical and genetic heterogeneity and the absence of “gold standards” makes the diagnostic evaluation challenging. Generally the diagnostic approach has to be individualized and requires an integral approach, incorporating clinical, biochemical, imaging, histological and genetic investigations. Consensus diagnostic criteria have been proposed for respiratory chain defects in infants and children and, despite some limitations, they appear to be sensitive in the paediatric population. The diagnostic efforts should aim at the genetic characterization, because under certain circumstances this may provide the framework for genetic counselling. Although there is no specific therapy and cure for the mitochondrial diseases, many secondary problems require specific treatment. This review summarizes present knowledge about the genetic background, pathogenetic mechanisms, clinical manifestations, diagnostic approach and therapeutic strategies of mitochondriopathies.
1 Cincinnati Children’s Hospital Medical Center, Dept of Pediatric Neurology 2 University Hospital of Larissa, Dept of Neurology Correspondence: Argirios Dinopoulos argidino@yahoo.com
Key words: Mitochondrial disorders, genetics, multisystem disorders, genetic counselling.
¶·È‰È·ÙÚÈ΋ 2007;70:1-17
2
∞. ¡ÙÈÓfiÔ˘ÏÔ˜, ∞. ¶··‰ËÌËÙÚ›Ô˘
Â›Ó·È ÚˆÙÂ˚ÓÈ΋ Î·È Û˘ÌÌÂÙ¤¯ÂÈ ÛÙËÓ ÔÍÂȉˆÙÈ΋ ʈÛÊÔÚ˘Ï›ˆÛË. ∆· ¤Ó˙˘Ì· Ù˘ ·Ó·Ó¢ÛÙÈ΋ ·Ï˘Û›‰·˜, ··Ú·›ÙËÙ· ÁÈ· ÙËÓ ÔÍÂȉˆÙÈ΋ ʈÛÊÔÚ˘Ï›ˆÛË, ‚Ú›ÛÎÔÓÙ·È ÛÙȘ ·ÎÚÔÏÔʛ˜ Ô˘ Û¯ËÌ·Ù›˙ÔÓÙ·È ·fi ·Ó·‰ÈÏÒÛÂȘ Ù˘ ÂÛˆÙÂÚÈ΋˜ ÌÂÌ‚Ú¿Ó˘. ¢˘ÛÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ÚÈÓ ·fi 44 ¯ÚfiÓÈ· Ì ÙËÓ ÎÏ·ÛÈ΋ ‰ËÌÔÛ›Â˘ÛË ÙˆÓ Luft et al. Î·È ÙËÓ ÂÚÈÁÚ·Ê‹ ÂÓfi˜ ·ÛıÂÓÔ‡˜ Ì ˘ÂÚÌÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ ÌÈÙÔ¯ÔÓ‰Úȷ΋˜ ·ÈÙÈÔÏÔÁ›·˜ (1). ∏ Û˘¯ÓfiÙËÙ· ‰È·Ù·Ú·¯ÒÓ ÛÙËÓ ·Ó·Ó¢ÛÙÈ΋ ·Ï˘Û›‰· ÛÙËÓ ·È‰È΋ ËÏÈΛ· ˘ÔÏÔÁ›˙ÂÙ·È ÌÂٷ͇ 1/5.000 Î·È 1/11.000 ÁÂÓÓ‹ÛÂȘ (2,3), ÂÓÒ
∂ÈÛ·ÁˆÁ‹ ∆· ÌÈÙÔ¯fiÓ‰ÚÈ· Â›Ó·È ÔÚÁ·Ó›‰È· ÙÔ˘ ΢ÙÙ·ÚÔÏ¿ÛÌ·ÙÔ˜ ˘Â‡ı˘Ó· ÁÈ· ÙËÓ ÂÓ‰Ô΢ÙÙ¿ÚÈ· ·Ú·ÁˆÁ‹ ÂÓ¤ÚÁÂÈ·˜. ™ÙÔ ÂÛˆÙÂÚÈÎfi ÙÔ˘ ÌÈÙÔ¯ÔÓ‰Ú›Ô˘ ‚Ú›ÛÎÂÙ·È Ë ıÂ̤ÏÈ· Ô˘Û›·, Ô˘ ÂÚȤ¯ÂÈ ¤Ó˙˘Ì· ÁÈ· ‰È¿ÊÔÚ˜ ÌÂÙ·‚ÔÏÈΤ˜ Ô‰Ô‡˜ Î·È ÚˆÙÂ˚ÓÔÛ‡ÓıÂÛË, (∂ÈÎfiÓ· 1) Î·È ÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA ÙÔ ÔÔ›Ô Îˆ‰ÈÎÔÔÈ› ÚˆÙ½Ó˜ ÁÈ· ÙË ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘. ∏ ÌÂÌ‚Ú¿ÓË Ô˘ ÂÚÈ‚¿ÏÂÈ Ù· ÌÈÙÔ¯fiÓ‰ÚÈ· Â›Ó·È ‰ÈÏ‹: Ë Â͈ÙÂÚÈ΋ ÌÂÌ‚Ú¿ÓË Â›Ó·È ÏÈȉÈ΋ Î·È Â›Ó·È ˘Â‡ı˘ÓË ÁÈ· ÙË ÌÂÙ·ÊÔÚ¿ ÙˆÓ Û˘ÛÙ·ÙÈÎÒÓ ·fi ÙÔ Î˘ÙÙ·ÚfiÏ·ÛÌ· ÛÙË ıÂ̤ÏÈ· Ô˘Û›·, ÂÓÒ Ë ÂÛˆÙÂÚÈ΋ ÌÂÌ‚Ú¿ÓË
∞Ï·Ó›ÓË
∂͈ÙÂÚÈ΋ ªÈÙÔ¯ÔÓ‰Úȷ΋ ªÂÌ‚Ú¿ÓË
°·Ï·ÎÙÈÎfi Tim
¶˘ÚÔÛÙ·Ê˘ÏÈÎfi ¶ÚˆÙ½Ó˜ ¶∞
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27,19 ATP ADP
M-CK
NADH ∞ÎÂÙ˘ÏÔCoA
πÛÔÎÈÙÚÈÎfi √Í·ÏÔ·ÎÂÙÈÎfi
NAD+
NAD
∫ÈÙÚÈÎfi
NADH
∂ÛˆÙÂÚÈ΋ ªÈÙÔ¯ÔÓ‰Úȷ΋ ªÂÌ‚Ú¿ÓË
NADH
NADH µ-ÔÍ›‰ˆÛË ÙˆÓ ÏÈ·ÚÒÓ ÔͤˆÓ
NADH
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2-√∏-ÎÂÙÔÁÏ˘Ù·ÚÈÎfi
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NADH ™Ô˘ÎÈÓ˘ÏÔÛ˘Ó¤Ó˙˘ÌÔ-∞
ºÔ˘Ì·ÚÈÎfi
™Ô˘ÎÈÓÈÎfi ATP
FADH2 FADH2 Û. ππ
ADP H+
Û. π
Û. V
™˘ÓQ Û. πππ
∫˘Ùfi¯Ú. c
Û. IV
√2 H2√
∞ÓÈfiÓÙ· ATP, ADP (ADP-ATP-ÙÚ·ÓÛÏÔοÛË)
∂͈ÙÂÚÈ΋ ªÈÙÔ¯ÔÓ‰Úȷ΋ ªÂÌ‚Ú¿ÓË
H+
∂ÈÎfiÓ· 1. ªÂÙ·‚ÔÏÈΤ˜ Ô‰Ô› ÛÙÔ ÌÈÙÔ¯fiÓ‰ÚÈÔ: ∞Ó·ÁˆÁÈο ÈÛÔ‰‡Ó·Ì· (NADH, FADH2) Û˘ÓÙ›ıÂÓÙ·È ·fi ÙÔ˘˜ ˘‰·Ù¿ÓıڷΘ ̤ۈ ÙÔ˘ ·ÎÏÔ˘ ÙÔ˘ Krebs Î·È ·fi Ù· ÏÈ·Ú¿ Ôͤ· ̤ۈ Ù˘ ‚-ÔÍ›‰ˆÛ˘ Î·È ÂÈÛ¤Ú¯ÔÓÙ·È Ì ÙË ÌÔÚÊ‹ ËÏÂÎÙÚÔÓ›ˆÓ Û ‰È·ÊÔÚÂÙÈο ÛËÌ›· Ù˘ ·Ï˘Û›‰·˜. ∏ÏÂÎÙÚfiÓÈ· ·fi NADH (ÁÏÔ˘Ù·ÌÈÎfi, ˘ÚÔÛÙ·Ê˘ÏÈÎfi, 3√∏-‚Ô˘Ù˘ÚÈÎfi Ô͇ Î·È ÏÈ·Ú¿ Ôͤ·) ÚÔÛ‰›‰ÔÓÙ·È ÛÙÔ Û‡ÌÏÂÁÌ· I, ËÏÂÎÙÚfiÓÈ· ·fi FADH2 (ÛÔ˘ÎÈÓÈÎfi) ÛÙÔ Û‡ÌÏÂÁÌ· II Î·È ËÏÂÎÙÚfiÓÈ· ·fi FADH2 (ÏÈ·Ú¿ Ôͤ·) ·Â˘ı›·˜ ÛÙÔ Û˘Ó¤Ó˙˘ÌÔ Q. ™ÙË Û˘Ó¤¯ÂÈ· Ì ÙË ‚Ô‹ıÂÈ· ÙÔ˘ Û˘ÓÂÓ˙‡ÌÔ˘ Q Ù· ËÏÂÎÙÚfiÓÈ· ÌÂٷʤÚÔÓÙ·È ·fi Ù· Û˘ÌϤÁÌ·Ù· π Î·È ππ ÛÙÔ Û‡ÌÏÂÁÌ· πππ Î·È ·ÎÔÏÔ‡ıˆ˜, ̤ۈ ÙÔ˘ ΢ÙÔ¯ÚÒÌ·ÙÔ˜ c, ÂÚÓÔ‡Ó ÛÙÔ Û‡ÌÏÂÁÌ· IV. ∏ ÂÓ¤ÚÁÂÈ· Ô˘ ·ÂÏ¢ıÂÚÒÓÂÙ·È ·fi ÙË ÚÔ‹ ÙˆÓ ËÏÂÎÙÚÔÓ›ˆÓ ηٿ Ì‹ÎÔ˜ ÙˆÓ Û˘ÌÏÂÁÌ¿ÙˆÓ π, ππ, πππ, IV ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙËÓ ¿ÓÙÏËÛË ÚˆÙÔÓ›ˆÓ ·fi ÙË ıÂ̤ÏÈ· Ô˘Û›· ÛÙÔÓ ‰È·ÌÂÌ‚Ú·ÓÈ·Îfi ¯ÒÚÔ Î·È ÙË ‰ËÌÈÔ˘ÚÁ›· ËÏÂÎÙÚÔ¯ËÌÈ΋˜ ‰È·ÊÔÚ¿˜ ‰˘Ó·ÌÈÎÔ‡ (¢„). ∏ ‰˘Ó·ÌÈ΋ ·˘Ù‹ ÂÓ¤ÚÁÂÈ·, ·ÔıËÎÂ˘Ì¤ÓË ˆ˜ ¢„, ¿ÁÂÈ ÙË Û‡ÓıÂÛË ATP ·fi ADP ̤ۈ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ V (ATP Û˘ÓıÂÙ¿Û˘), Ì ٷ˘Ùfi¯ÚÔÓË Â·Ó·ÊÔÚ¿ ÙˆÓ ÚˆÙÔÓ›ˆÓ ÛÙË ıÂ̤ÏÈ· Ô˘Û›· (6). Paediatriki 2007;70:1-17
3
ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜
Ë Û˘¯ÓfiÙËÙ· ÌÂÙ·ÏÏ¿ÍÂˆÓ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA ÛÙÔ˘˜ ÂÓ‹ÏÈΘ ÂÎÙÈÌ¿Ù·È 1:8000 (4). ªÈ· Û¯ÂÙÈο ÚfiÛÊ·ÙË ÌÂϤÙË ·fi ÙËÓ ∞˘ÛÙÚ·Ï›·, ‚·ÛÈṲ̂ÓË Û ÎÚÈÙ‹ÚÈ· ÁÈ· ÙË ‰È¿ÁÓˆÛË, ˘ÔÏÔÁ›˙ÂÈ ÙÔÓ ÂÏ¿¯ÈÛÙÔ ÂÈÔÏ·ÛÌfi ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ÛÙȘ 1/7634 ÁÂÓÓ‹ÛÂȘ (5). √È ·ÓˆÙ¤Úˆ Û˘¯ÓfiÙËÙ˜ ‰ÂÓ Ê·›ÓÂÙ·È fiÙÈ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ· ÁÈ·Ù›, ÏfiÁˆ Ù˘ ÂÙÂÚÔÁ¤ÓÂÈ·˜ Ù˘ ÓfiÛÔ˘, ÌfiÓÔÓ ¤Ó· ÔÛÔÛÙfi ·ÛıÂÓÒÓ ·Ú·¤ÌÔÓÙ·È Û ÂȉÈο ΤÓÙÚ· ÁÈ· ÙÂÎÌËÚ›ˆÛË Ù˘ ‰È¿ÁÓˆÛ˘.
¶·ıÔÁ¤ÓÂÛË ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ∆Ô ÌÈÙÔ¯fiÓ‰ÚÈÔ ÂÚȤ¯ÂÈ ¤Ó· ΢ÎÏÈÎfi DNA ÁÔÓȉ›ˆÌ· ÙÔ ÔÔ›Ô ·ÔÙÂÏ› ÙËÓ ‚¿ÛË ÙÔ˘ ‰ÈÎÔ‡ ÙÔ˘ ÁÂÓÂÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ∏ Έ‰ÈÎÔÔ›ËÛË ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÚˆÙÂ˚ÓÒÓ ‚Ú›ÛÎÂÙ·È ˘fi ÙËÓ ÂÈÚÚÔ‹ ‰‡Ô ÁÂÓÂÙÈÎÒÓ Û˘ÛÙËÌ¿ÙˆÓ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ Î·È ÙÔ˘ ˘ÚËÓÈÎÔ‡. ∆Ô ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA Έ‰ÈÎÔÔÈ› 13 ˘ÔÔÌ¿‰Â˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Î·È Ù· tRNAs Î·È rRNAs ··Ú·›ÙËÙ· ÁÈ· ÙË ÌÂÙ¿ÊÚ·Û‹ ÙÔ˘˜, ÂÓÒ Ô ÌÂÁ·Ï‡ÙÂÚÔ˜ ·ÚÈıÌfi˜ ˘ÔÔÌ¿‰ˆÓ Î·È ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÚˆÙÂ˚ÓÒÓ Îˆ‰ÈÎÔÔÈÂ›Ù·È ·fi ÙÔ ˘ÚËÓÈÎfi DNA. ∏ ‰ÈÏ‹ ·˘Ù‹ ÁÂÓÂÙÈ΋ ÂÈÚÚÔ‹ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Â›Ó·È ˘Â‡ı˘ÓË ÁÈ· ÙÔ fiÙÈ ÛÙ· ÌÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› fiÏÔÈ ÔÈ Ù‡ÔÈ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜. ∏ ÔÌ·Ï‹ ·Ú·ÁˆÁ‹ ÂÓ¤ÚÁÂÈ·˜ ÛÙÔ ÌÈÙÔ¯fiÓ‰ÚÈÔ ··ÈÙ› ÙÔÓ Û˘ÓÙÔÓÈÛÌfi ÔÏÏÒÓ ÏÂÈÙÔ˘ÚÁÈÒÓ. ªÈÙÔ¯ÔÓ‰ÚÈ·Îfi ÓfiÛËÌ· ÚÔ·ÙÂÈ fiÙ·Ó Ë ÎÏËÚÔÓÔÌÔ‡ÌÂÓË ‹ ÛÔÚ·‰È΋ ‚Ï¿‚Ë ÛÙÔ ˘ÚËÓÈÎfi ‹ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA ÂËÚ¿ÛÂÈ ¤Ó· ·fi Ù· ›‰· ÏÂÈÙÔ˘ÚÁ›·˜ fiˆ˜ ·Ó·ÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1 (7). ÷ڷÎÙËÚÈÛÙÈÎfi ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ Â›Ó·È Ë ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔÓ ÎÏÈÓÈÎfi Ê·ÈÓfiÙ˘Ô. °È· ÙËÓ ÂÍ‹ÁËÛË Ù˘ Ê·ÈÓÔÙ˘È΋˜ ÂÙÂÚÔÁ¤ÓÂÈ·˜ Â›Ó·È ÛËÌ·ÓÙÈÎfi Ó· ηٷÓÔËıÔ‡Ó ‰‡Ô ‚·ÛÈΤ˜ ·Ú¯¤˜: Ë ÂÙÂÚÔÏ·ÛÌ›· (heteroplasmy) Î·È Ô Ô˘‰fi˜ Ê·ÈÓÔÙ˘È΋˜ ¤ÎÊÚ·Û˘ (threshold effect). ∫¿ı ·ÙÙ·ÚÔ ÂÚȤ¯ÂÈ ÌÂÁ¿ÏÔ ·ÚÈıÌfi ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ (Î˘Ì·›ÓÂÙ·È ·fi ÌÂÚÈΤ˜ ÂηÙÔÓÙ¿‰Â˜ ̤¯ÚÈ ÌÂÚÈ-
Τ˜ ¯ÈÏÈ¿‰Â˜) Î·È Î¿ı ÌÈÙÔ¯fiÓ‰ÚÈÔ ÂÚȤ¯ÂÈ 2-10 ·ÓÙ›ÁÚ·Ê· ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA. ∫·Ù¿ ÙË ÌÈÙˆÙÈ΋ ‰È·›ÚÂÛË ÙÔ Î‡ÙÙ·ÚÔ ‰›ÓÂÈ ¤Ó·Ó Ù˘¯·›Ô ·ÚÈıÌfi ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ÛÙ· ı˘Á·ÙÚÈο ·ÙÙ·Ú·, Ê·ÈÓfiÌÂÓÔ Ô˘ ÔÓÔÌ¿˙Ô˘Ì ÌÈÙˆÙÈÎfi ‰È·¯ˆÚÈÛÌfi (mitotic segregation). ªÂÙ¿ÏÏ·ÍË ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA ÛÙÔ ÚÔÁÔÓÈÎfi ·ÙÙ·ÚÔ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË ‰ËÌÈÔ˘ÚÁ›· ‰‡Ô ÏËı˘ÛÌÒÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ÛÙ· ı˘Á·ÙÚÈο ·ÙÙ·Ú· (∂ÈÎfiÓ· 2), Ê·ÈÓfiÌÂÓÔ Ô˘ ÔÓÔÌ¿˙ÂÙ·È ÂÙÂÚÔÏ·ÛÌ›· (heteroplasmy). ∫ÏÈÓÈ΋ Û˘Ìو̷ÙÔÏÔÁ›· ÂÌÊ·Ó›˙ÂÙ·È fiÙ·Ó ÙÔ ÔÛÔÛÙfi ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ Ô˘ ʤÚÔ˘Ó ÙË ÌÂÙ¿ÏÏ·ÍË ÍÂÂÚ¿ÛÂÈ ¤Ó·Ó Û˘ÁÎÂÎÚÈ̤ÓÔ Ô˘‰fi ÛÙÔ Î‡ÙÙ·ÚÔ (Ô˘‰fi˜ Ê·ÈÓÔÙ˘È΋˜ ¤ÎÊÚ·Û˘/ threshold effect) Î·È Â›Ó·È ÈηÓfi Ó· ÂËÚ¿ÛÂÈ ÙËÓ ÂÓ‰Ô΢ÙÙ¿ÚÈ· ·Ú·ÁˆÁ‹ ÂÓ¤ÚÁÂÈ·˜. √ Ô˘‰fi˜ ÂÍ·ÚÙ¿Ù·È ·fi ÙȘ ÂÓÂÚÁÂȷΤ˜ ·Ó¿ÁΘ ÙÔ˘ ÈÛÙÔ‡ Î·È ·fi ÙÔ Â›‰Ô˜ Ù˘ ÌÂÙ¿ÏÏ·Í˘ (6,8).
∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ & ªÈÙÔ¯ÔÓ‰Úȷο Û‡Ó‰ÚÔÌ· ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› Û fiϘ ÙȘ ËÏÈÎȷΤ˜ ÔÌ¿‰Â˜. ∏ ÓfiÛÔ˜ ·ÎÔÏÔ˘ı› Û˘Ó‹ıˆ˜ ÌÈ· ‚Ú·‰Â›· ÂÍÂÏÈÛÛfiÌÂÓË ÔÚ›· Ì ÂÍ¿ÚÛÂȘ Ù˘ ÓfiÛÔ˘ ˘fi ÙË ÌÔÚÊ‹ ÌÂÙ·‚ÔÏÈÎÒÓ ÎÚ›ÛÂˆÓ Î¿Ùˆ ·fi ηٷÛÙ¿ÛÂȘ stress. ∏ ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘ ÌÔÚ› Ó· Â›Ó·È Ú·Á‰·›· ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·, ÂÓÒ ˘¿Ú¯ÂÈ ÌÈ· Ù¿ÛË ÛÙ·ıÂÚÔÔ›ËÛ˘ ÌÂÙ¿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜. °ÂÓÈÎfiÙÂÚ· Ë ÔÚ›· Ù˘ ÓfiÛÔ˘ ÂÍ·ÚÙ¿Ù·È Û ÌÂÁ¿ÏÔ ‚·ıÌfi ·fi ÙÔ Â›‰Ô˜ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ ÓÔÛ‹Ì·ÙÔ˜ Î·È ·fi ÙËÓ ·ÚÔ˘Û›· ‹ fi¯È ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋˜ Û˘ÌÌÂÙÔ¯‹˜. ŸÚÁ·Ó· Î·È ÈÛÙÔ› Ì ·˘ÍË̤Ó˜ ÂÓÂÚÁÂȷΤ˜ ·Ó¿ÁΘ fiˆ˜ ÂÁΤʷÏÔ˜, ̇˜, ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹˜, ÓÂÊÚÈο ÛˆÏËÓ¿ÚÈ· Â›Ó·È ÈÔ Â˘¿ÏˆÙ· ÛÙË ÌÈÙÔ¯ÔÓ‰Úȷ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Î·È ÚÔÛ‚¿ÏÏÔÓÙ·È ÈÔ Û˘¯Ó¿. ™ÙËÓ ·È‰È΋ ËÏÈΛ· ÔÈ ÌÈÙÔ¯ÔÓ‰ÚÈÔ¿ıÂȘ ÚÔ‚¿ÏÏÔ˘Ó ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛÂˆÓ ˆ˜ ¿Ù˘Ë ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ· Ì ‹ ¯ˆÚ›˜ ÙË Û˘ÌÌÂÙÔ¯‹ ¿ÏÏˆÓ Û˘ÛÙËÌ¿ÙˆÓ (¶›Ó·Î·˜ 2). ∆· ÁÓˆÛÙ¿ ÌÈÙÔ¯ÔÓ‰Úȷο Û‡Ó‰ÚÔÌ· Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È Î·ÙˆÙ¤Úˆ ··ÓÙÒÓÙ·È ÈÔ
¶›Ó·Î·˜ 1. ¶·ıÔÁÂÓÂÙÈÎÔ› Ì˯·ÓÈÛÌÔ› Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ÚfiÎÏËÛË ÚˆÙÔ·ıÒÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ªÂÙ·ÏÏ¿ÍÂȘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA
ªÂÙ·ÏÏ¿ÍÂȘ ˘ÚËÓÈÎÔ‡ DNA
i. ªÂÙ·ÏÏ¿ÍÂȘ Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó i. ¢È·Ù·Ú·¯¤˜ Û ‰ÔÌÈο ÁÔÓ›‰È· Ù˘ ÔÍÂȉˆÙÈ΋˜ ˘ÔÔÌ¿‰Â˜ ÙˆÓ Û˘ÌÏÂÁÌ¿ÙˆÓ ÊˆÛÊÔÚ˘Ï›ˆÛ˘ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ ii. ¢È·Ù·Ú·¯¤˜ Û ÁÔÓ›‰È· Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙË Û˘Ó·ÚÌÔÏfiÁËÛË ii. ªÂÙ·ÏÏ¿ÍÂȘ Ô˘ ÂËÚ¿˙Ô˘Ó ÙË (assembly) ÙˆÓ Û˘ÌÏÂÁÌ¿ÙˆÓ ÌÈÙÔ¯ÔÓ‰Úȷ΋ ÚˆÙÂ˚ÓÈ΋ Û‡ÓıÂÛË iii. ¢È·Ù·Ú·¯¤˜ ÛÙË Û˘ÓÙ‹ÚËÛË ÙÔ˘ mtDNA Î·È ÛÙË (tRNA, rRNA) ‰È·ÁÔÓȉȷ΋ Û‹Ì·ÓÛË (intergenomic signalling) ñ ™ÔÚ·‰Èο ÂÏÏ›ÌÌ·Ù· / ·Ó·‰ÈÏ·ÛÈ·ÛÌÔ› iv. ¢È·Ù·Ú·¯¤˜ Û ÁÔÓ›‰È· Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ÔÌÔÈfiÛÙ·ÛË Î·È ñ ™ËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÂÈÛ·ÁˆÁ‹ (import) Û˘ÛÙ·ÙÈÎÒÓ (ÚˆÙ½Ó˜, ADP) v. ¢È·Ù·Ú·¯¤˜ ÙÔ˘ ÏÈȉÈÎÔ‡ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ Ù˘ ÂÛˆÙÂÚÈ΋˜ ÌÂÌ‚Ú¿Ó˘ vi. ¢È·Ù·Ú·¯¤˜ ÛÙËÓ ÎÈÓËÙÈÎfiÙËÙ· ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ¶·È‰È·ÙÚÈ΋ 2007;70:1-17
4
∞. ¡ÙÈÓfiÔ˘ÏÔ˜, ∞. ¶··‰ËÌËÙÚ›Ô˘
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√˘‰fi˜ Ê·ÈÓÔÙ˘È΋˜ ¤ÎÊÚ·Û˘
¶ÚÔÁÔÓÈÎfi ·ÙÙ·ÚÔ
£˘Á·ÙÚÈο ·ÙÙ·Ú·
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∫ÏÈÓÈ΋ Û˘Ìو̷ÙÔÏÔÁ›·
∂ÈÎfiÓ· 2. ∆Ô Ê·ÈÓfiÌÂÓÔ Ù˘ ÂÙÂÚÔÏ·ÛÌ›·˜ Î·È Ë ·Ú¯‹ ÙÔ˘ Ô˘‰Ô‡ Ê·ÈÓÔÙ˘È΋˜ ¤ÎÊÚ·Û˘: √È Ì·‡ÚÔÈ Î‡ÎÏÔÈ ·ÓÙÈÚÔÛˆÂ‡Ô˘Ó ÌÈÙÔ¯fiÓ‰ÚÈ· Ô˘ ʤÚÔ˘Ó ÙË ÌÂÙ¿ÏÏ·ÍË ÛÙÔ DNA ÙÔ˘˜ (mutant) Î·È ÔÈ Ï¢ÎÔ› ·ÎÏÔÈ Ê˘ÛÈÔÏÔÁÈο ÌÈÙÔ¯fiÓ‰ÚÈ· (wild). ªÂ ÙÔÓ ÌÈÙˆÙÈÎfi ‰È·¯ˆÚÈÛÌfi ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ‰ËÌÈÔ˘ÚÁÂ›Ù·È ÌÈ· ̈۷˚΋ ηٿÛÙ·ÛË ÛÙÔ˘˜ ÈÛÙÔ‡˜, fiÔ˘ ÙÔ ÔÛÔÛÙfi ÙˆÓ ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ‰È·Ê¤ÚÂÈ. ∫ÏÈÓÈÎfi˜ Ê·ÈÓfiÙ˘Ô˜ ÂÌÊ·Ó›˙ÂÙ·È fiÙ·Ó ÙÔ ÔÛÔÛÙfi ÙˆÓ ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ Â›Ó·È ÈηÓfi Ó· ÂËÚ¿ÛÂÈ ÙËÓ ÂÓ‰Ô΢ÙÙ¿ÚÈ· ·Ú·ÁˆÁ‹ ÂÓ¤ÚÁÂÈ·˜.
Û˘¯Ó¿ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹, ÂÓÒ fiÙ·Ó ÂÌÊ·ÓÈÛÙÔ‡Ó ÛÙËÓ ·È‰È΋ ËÏÈΛ· ‰ÂÓ ÂÌÊ·Ó›˙ÔÓÙ·È Ì ÙËÓ ÎÏ·ÛÈ΋ ÙÔ˘˜ Û˘Ìو̷ÙÔÏÔÁ›· (8-14). ÕÏϘ Û˘Ó‰ÚÔÌÈΤ˜ ηٷÛÙ¿ÛÂȘ Ì ÌÈÙÔ¯ÔÓ‰Úȷ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 3 (15). ¶ÚÔԉ¢ÙÈ΋ Â͈ÙÂÚÈ΋ ÔÊı·ÏÌÔÏËÁ›· & ™‡Ó‰ÚÔÌÔ Kearns-Sayre (Progressive external ophthalmoplegia PEO & Kearns-Sayre syndrome). ¶ÚÔԉ¢ÙÈ΋ ÔÊı·ÏÌÔÏËÁ›· Â›Ó·È ¤Ó· ·fi Ù· ÈÔ Û˘¯Ó¿ Û˘ÌÙÒÌ·Ù· ÛÙ· ÌÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù·. ¶ÙÒÛË ‚ÏÂÊ¿ÚˆÓ Î·È
ÔÊı·ÏÌÔÏËÁ›· ¯ˆÚ›˜ ‰Èψ›· (∂ÈÎfiÓ· 3) Â›Ó·È Ù· ·Ú¯Èο Û˘ÌÙÒÌ·Ù· Ù· ÔÔ›· ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙËÓ fi„ÈÌË ·È‰È΋ ‹ ÂÊË‚È΋ ËÏÈΛ· Î·È ÌÔÚ› Ó· Û˘Ó‰˘¿˙ÔÓÙ·È ÌÂ Ì˘˚΋ ·‰˘Ó·Ì›·, ¯ÚfiÓÈ· ÎfiˆÛË Î·È ‰˘Û·ÓÂÍ›· ÛÙËÓ ¿ÛÎËÛË. ∆Ô Û‡Ó‰ÚÔÌÔ Kearns-Sayre Â›Ó·È ÔÏ˘Û˘ÛÙËÌ·ÙÈÎfi ÓfiÛËÌ·. √ ·ÎÚÈ‚‹˜ ÔÚÈÛÌfi˜ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Â›Ó·È Ë ÂÌÊ¿ÓÈÛË, ÚÈÓ ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 20 ÂÙÒÓ, ÔÊı·ÏÌÔÏËÁ›·˜ Î·È ÌÂÏ·Á¯ÚˆÛÙÈ΋˜ ·ÌÊÈ‚ÏËÛÙÚÔÂȉԿıÂÈ·˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ¤Ó· ·fi Ù· ÂfiÌÂÓ·: ·) ‰È·Ù·Ú·¯‹ Ù˘ ·ÁˆÁÈÌfiÙËÙ·˜ ÛÙÔ Î·Ú‰È·Îfi Ì˘ÔοډÈÔ (conduction block), ‚) ·ÚÂÁÎÂÊ·ÏȉÈ΋ ·Ù·Í›·, Á) ·˘ÍË̤ÓË ÚˆÙ½ÓË ÛÙÔ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi (∂¡À). ÕÏÏ· ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ¯·ÌËÏfi ·Ó¿ÛÙËÌ·, Ó¢ÚÔ·ÈÛıËÙËÚȷ΋ ·ÒÏÂÈ· Ù˘ ·ÎÔ‹˜, ‰È·Ù·Ú·¯‹ ÛÙË ‰È·ÓÔËÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·, ÛˆÏËÓ·Úȷ΋ ÓÂÊÚÔ¿ıÂÈ·. ∏ ÚfiÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Kearns-Sayre Â›Ó·È Î·Ù¿ Ôχ ‰˘ÛÌÂÓ¤ÛÙÂÚË Ù˘ PEO (14). √È ÂÚÈÛÛfiÙÂÚÔÈ ·ÛıÂÓ›˜ Ì ۇӉÚÔÌÔ KearnsSayre Î·È PEO ¤¯Ô˘Ó ¤Ó· ÌÔÓ‹Ú˜ ¤ÏÏÂÈÌÌ· ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA Î·È Ë ÂÌÊ¿ÓÈÛ‹ ÙÔ˘˜ Â›Ó·È ÛÔÚ·‰È΋. ™ËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA (A3243G) Â›Ó·È ˘Â‡ı˘Ó˜ ÁÈ· ÙÔ Û‡Ó‰ÚÔÌÔ PEO Ô˘ ÌÂÙ·‚È‚¿˙ÂÙ·È Ì ÙÔÓ ÌËÙÚÈÎfi Ù‡Ô ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜. ªÂÙ·ÏÏ¿ÍÂȘ ˘ÚËÓÈÎÒÓ ÁÔÓȉ›ˆÓ (ANT1, POLG1, Twinkle), Ô˘ Ô‰ËÁÔ‡Ó Û ÔÏÏ·Ï¿ ÂÏÏ›ÌÌ·Ù· ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA Â›Ó·È ˘Â‡ı˘Ó˜ ÁÈ· ÙÔ ÎÏËÚÔÓÔÌÔ‡ÌÂÓÔ PEO (16-19). ™‡Ó‰ÚÔÌÔ Pearson. ¶·ÚÔ˘ÛÈ¿˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÛȉËÚÔ‚Ï·ÛÙÈ΋
¶›Ó·Î·˜ 2. ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ·Ó¿ Û˘ÛÙ‹Ì·Ù· ∫¡™
æ˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË ‹ ˘ÔÛÙÚÔÊ‹, ‰È·ÓÔËÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ÎÂÓÙÚÈ΋ ˘ÔÙÔÓ›· (Û˘Ó‹ıˆ˜ Û˘Ó‰˘·ÛÌfi˜ ˘ÔÙÔÓ›·˜ ÙÔ˘ ÎÔÚÌÔ‡ Î·È ˘ÂÚÙÔÓ›·˜ ÙˆÓ ¿ÎÚˆÓ), ·˘ÙÈÛÌfi˜, ·ÚÂÁÎÂÊ·ÏȉÈ΋ ·Ù·Í›·, ‰˘ÛÙÔÓ›· Î·È ¿ÏϘ ‰˘ÛÎÈÓËۛ˜, ÂÈÏË„›· (Ì˘ÔÎÏÔӛ˜ ‹ ¿ÏÏ· ›‰Ë Û·ÛÌÒÓ), ÔÏ˘Ì˘ÔÎÏÔÓ›· (ÌË ÂÈÏËÙÈ΋˜ ʇÛˆ˜), ¿Ù˘· ÂÁÎÂÊ·ÏÈο ÂÂÈÛfi‰È· (stroke-like events), ˘Ú·ÌȉÈ΋ Û˘Ó‰ÚÔÌ‹ Ì ۷ÛÙÈÎfiÙËÙ·, Â͈˘Ú·ÌȉÈ΋ Û˘Ó‰ÚÔÌ‹, „‡¯ˆÛË, ËÌÈÎÚ·Ó›·, ÎÒÌ·
¡Â˘ÚÔÌ˘˚Îfi
ÀÔÙÔÓ›· (΢ڛˆ˜ ÛÙ· ‚Ú¤ÊË), Ì˘˚΋ ·ÙÚÔÊ›· (ÂÈÎfiÓ· ÓˆÙÈ·›·˜ Ì˘˚΋˜ ·ÙÚÔÊ›·˜), Ì˘Ô¿ıÂÈ· (Û ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ Î·È ÂÓ‹ÏÈΘ), ‡ÎÔÏË ÎfiˆÛË Î·È ‰˘Û·ÓÂÍ›· ÛÙËÓ ¿ÛÎËÛË, Ì˘˚Τ˜ Îڿ̘ Î·È Ì˘·ÏÁ›Â˜, Ú·‚‰ÔÌ˘fiÏ˘ÛË, ÂÚÈÊÂÚÈ΋ Ó¢ÚÔ¿ıÂÈ· (Û˘Ó‹ıˆ˜ ·ÍÔÓÈ΋ ÌÂ Û˘ÌÌÂÙÔ¯‹ ÙˆÓ ÎÈÓËÙÈÎÒÓ Î·È ·ÈÛıËÙÈÎÒÓ Ó‡ڈÓ)
√Êı·ÏÌÔ›
∫·Ù·ÚÚ¿¯Ù˘, ÌÂÏ·Á¯ÚˆÛÙÈ΋ ·ÌÊÈ‚ÏËÛÙÚÔÂȉԿıÂÈ·, ÔÙÈ΋ Ó¢ÚÔ¿ıÂÈ·, ·ÙÚÔÊ›· ÔÙÈÎÔ‡ Ó‡ÚÔ˘
∞ÎÔ‹
¡Â˘ÚÔ·ÈÛıËÙËÚȷ΋ ·ÒÏÂÈ· ·ÎÔ‹˜, ˘ÂÚ·ÎÔ˘Û›·
∫·Ú‰È¿
¢È·Ù·Ú·¯¤˜ ·ÁˆÁÈÌfiÙËÙ·˜, ˘ÂÚÙÚÔÊÈ΋ Î·È Û·ÓÈfiÙÂÚ· ‰È·Ù·ÙÈ΋ ηډÈÔÌ˘Ô¿ıÂÈ·
°·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ·
¶ÚÒÈÌË Ë·ÙÈ΋ ·Ó¿ÚÎÂÈ· (Û˘Ó‹ıˆ˜ Û ‚Ú¤ÊË), ¯ÚfiÓÈ· Ë·ÙÔ¿ıÂÈ·, ÔÍ›· ÎÂÚ·˘ÓÔ‚fiÏÔ˜ Ë·Ù›Ùȉ· Î·È Ó¤ÎÚˆÛË (ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ‚·ÏÚÔ˚ÎÔ‡ ÔͤԘ Û ۇӉÚÔÌÔ Alper), „¢‰Ô·ÔÊÚ·ÎÙÈÎfi Û‡Ó‰ÚÔÌÔ, ¯ÚfiÓÈÔ ‰È·ÚÚÔ˚Îfi Û‡Ó‰ÚÔÌÔ, ·Ó¿ÚÎÂÈ· Â͈ÎÚÈÓÔ‡˜ ÌÔ›Ú·˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜, ΢ÎÏÈÎÔ› ¤ÌÂÙÔÈ, ‰È·Ù·Ú·¯‹ ·Ó¿Ù˘Í˘ Î·È Î·¯ÂÍ›·
¡ÂÊÚÔ›
™ˆÏËÓ·Úȷ΋ ·Ó¿ÚÎÂÈ·, Û‡Ó‰ÚÔÌÔ DeToni-Debre-Fanconi, ‰È¿ÌÂÛË ÛˆÏËÓ·Úȷ΋ ÓÂÊÚ›Ùȉ·
∂Ó‰ÔÎÚÈÓÔÏÔÁÈÎfi Û‡ÛÙËÌ·
÷ÌËÏfi ·Ó¿ÛÙËÌ·, ÚÒÈÌË ÂÌÊ¿ÓÈÛË Û·Î¯·ÚÒ‰Ô˘˜ ‰È·‚‹ÙË Ù‡Ô˘ 2 (ÂÓËϛΈÓ), ˘Ô·Ú·ı˘ÚÂÔȉÈÛÌfi˜, ÔÛÙÂÔfiÚˆÛË, ˘ÔÊ˘Ûȷ΋ ·Ó¿ÚÎÂÈ·
ª˘ÂÏfi˜ ÙˆÓ ÔÛÙÒÓ ™È‰ËÚÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ Paediatriki 2007;70:1-17
5
ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜
∂ÈÎfiÓ· 3. ∞ÌÊÔÙÂÚfiÏ¢ÚË ‚ÏÂÊ·ÚfiÙˆÛË Û ·ÛıÂÓ‹ Ì ÂÏ·ÊÚ¿ ·‰˘Ó·Ì›· Î·È Û‡Ó‰ÚÔÌÔ PEO ·fi ¤ÏÏÂÈÌÌ· ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA.
·Ó·ÈÌ›·, ıÚÔÌ‚ÔÂÓ›· Î·È ·Ó¿ÚÎÂÈ· Ù˘ Â͈ÎÚÈÓÔ‡˜ ÌÔ›Ú·˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜. §ÈÁfiÙÂÚÔ Û˘¯Ó‹ Â›Ó·È Ë ÓÂÊÚÈ΋ ÛˆÏËÓ·Úȷ΋ ÔͤˆÛË (20), Ë·ÙÈ΋ ·Ó¿ÚÎÂÈ· Î·È ‰È·Ù·Ú·¯‹ Ù˘ ÂÓ‰ÔÎÚÈÓÔ‡˜ ÌÔ›Ú·˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜ Ì ÈÓÛÔ˘ÏÈÓÔ-ÂÍ·ÚÙÒÌÂÓÔ Û·Î¯·ÚÒ‰Ë ‰È·‚‹ÙË (21). √È ÂÚÈÛÛfiÙÂÚÔÈ ·ÛıÂÓ›˜ ˘Ô·ÙÔ˘Ó ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜, ·ÏÏ¿ ÌÂÚÈÎÔ› ·ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó ÂÈ˙‹ÛÂÈ ·Ó¤Ù˘Í·Ó ÛÙËÓ ÔÚ›· Û‡Ó‰ÚÔÌÔ Kearns-Sayre. ∂ÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Ì·ÎÚÔ΢ÙÙ·ÚÈ΋ ·Ó·ÈÌ›·, ·‡ÍËÛË Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ F, ÛȉËÚÔ‚Ï¿ÛÙ˜ ÛÙË Ì˘ÂÏÈ΋ ‚ÈÔ„›·, Á·Ï·ÎÙÈ΋ ÔͤˆÛË, ÌÂı˘ÏÔÁÏÔ˘Ù·ÎÔÓÈ΋ ÔÍÂÔ˘Ú›·, Î·È ·Ó‡ÚÂÛË ÂÚ˘ıÚÒÓ Ú·Îˆ‰ÒÓ ÈÓÒÓ ÛÙËÓ Ì˘˚΋ ‚ÈÔ„›· (22,23). ∆o Û‡Ó‰ÚÔÌÔ Pearson ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÌÂÁ¿Ï˘ Îϛ̷η˜ ·Ó·Û˘Ó‰˘·ÛÌÔ‡˜ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA (24). ™È‰ËÚÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›· ¤¯ÂÈ ÂÚÈÁÚ·Ê› ›Û˘ Ì ÙËÓ ÛËÌÂȷ΋ ÌÂÙ¿ÏÏ·ÍË A8344G ηıÒ˜ Î·È Ì ÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ ÌÂÙ·ÊÔÚ¤· Ûȉ‹ÚÔ˘ (25). ª˘ÔÎÏÔÓÈ΋ ÂÈÏË„›· Ì ÂÚ˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜ (Myoclonic Epilepsy with Ragged Red Fibbers). ∆Ô Û‡Ó‰ÚÔÌÔ MERRF ··ÓÙ¿Ù·È Û˘Ó‹ıˆ˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ ·ÏÏ¿ Ë Û˘Ìو̷ÙÔÏÔÁ›· ÌÔÚ› Ó· ·Ú¯›ÛÂÈ ÛÙËÓ ·È‰È΋ ËÏÈΛ·. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ·ÚÔ˘Û›· Ì˘ÔÎÏÔÓÈÎÒÓ Û·ÛÌÒÓ, ÂÈÏË„›·˜, ÂÏ·ÊÚ¿˜ Ì˘˚΋˜ ·‰˘Ó·Ì›·˜ Î·È ·ÚÂÁÎÂÊ·ÏȉÈ΋˜ ·Ù·Í›·˜. ÕÏÏ· ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ¿ÓÔÈ·, ÔÙÈ΋ Ó¢ÚÔ¿ıÂÈ·, Ó¢ÚÔ·ÈÛıËÙËÚȷ΋ ·ÒÏÂÈ· ·ÎÔ‹˜, ÂÚÈÊÂÚÈ΋ Ó¢ÚÔ¿ıÂÈ·, ÔÊı·ÏÌÔÏËÁ›·, ÔÏÏ·Ï¿ ÏÈÒÌ·Ù· Î.Ï. √ Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Û˘¯Ó¿ ·Ó·‰ÂÈÎÓ‡ÂÈ ÌË ÂȉÈο ÛËÌ›· fiˆ˜ ÂÁÎÂÊ·ÏÈ΋ Î·È ·ÚÂÁÎÂÊ·ÏȉÈ΋ ·ÙÚÔÊ›·. ∏ ÂÌÊ¿ÓÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÛÙËÓ ·È‰È΋ ËÏÈΛ· Û˘Ó‰˘¿˙ÂÙ·È Ì ‰˘ÛÌÂÓ¤ÛÙÂÚË ÚfiÁÓˆÛË (26). ™ÙË ‚ÈÔ„›· ÛÎÂÏÂÙÈÎÔ‡ Ì˘fi˜ Û˘¯Ó¿ ·Ó¢ڛÛÎÔÓÙ·È ÂÚ˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜ Î·È ÛÙÔÓ Ì˘˚Îfi ÈÛÙfi Û˘¯Ó‹ Â›Ó·È Ë ·ÓÂ-
¿ÚÎÂÈ· ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ IV. ™ÙÔ 80% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ·ÓȯÓ‡ÂÙ·È Ë ÛËÌÂȷ΋ ÌÂÙ¿ÏÏ·ÍË A8344G (27,28). ªÈÙÔ¯ÔÓ‰Úȷ΋ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·, Á·Ï·ÎÙÈ΋ ÔͤˆÛË Î·È ÂÁÎÂÊ·ÏÈο ÂÂÈÛfi‰È· (Mitochondrial encephalopathy, Lactic acidosis and Stroke). ∫ÚÈÙ‹ÚÈ· ÁÈ· ÙË ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ MELAS ÚÔÙ¿ıËÎ·Ó ·fi ÙÔÓ Hirano et al. ÌÂÙ¿ ·fi ·Ó·ÛÎfiËÛË 69 ÂÚÈÙÒÛÂˆÓ (29) Î·È ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ·) ¿Ù˘Ô ÂÁÎÂÊ·ÏÈÎfi ÂÂÈÛfi‰ÈÔ ÚÈÓ ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 40 ÂÙÒÓ, ‚) ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ¯·Ú·ÎÙËÚÈ˙fiÌÂÓË ·fi Û·ÛÌÔ‡˜ ‹/Î·È ¿ÓÔÈ·, Á) Á·Ï·ÎÙÈ΋ ÔͤˆÛË ‹/Î·È ÂÚ˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜ ÛÙË Ì˘˚΋ ‚ÈÔ„›·. ™Ù· ·È‰È¿ ·Ú¯Èο ÌÔÚ› Ó· ÚÔ‚¿ÏÂÈ ˆ˜ ¿Ù˘Ë ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ·, ‰È·Ù·Ú·¯‹ ÛÙËÓ ·Ó¿Ù˘ÍË Ì ‹ ¯ˆÚ›˜ Á·Ï·ÎÙÈ΋ ÔͤˆÛË, ÂÓÒ Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ÂÁÎÂÊ·ÏÈο ÂÂÈÛfi‰È· ÂÌÊ·Ó›˙ÔÓÙ·È ·ÚÁfiÙÂÚ·. ∆· ÂÁÎÂÊ·ÏÈο ÂÂÈÛfi‰È· ÚÔ‚¿ÏÔ˘Ó Ì ËÌÈ·ÓÔ„›·, ËÌÈÎÚ·Ó›· Î·È Û‡Á¯ÚÔÓË ·ÚÔ˘Û›· ÂÛÙÈ·ÎÒÓ Û·ÛÌÒÓ Î·È fiÙ·Ó ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙËÓ ·È‰È΋ ËÏÈΛ· ˘ÔÙÚÔÈ¿˙Ô˘Ó, Û˘Óԉ‡ÔÓÙ·È ·fi ·ÓıÂÎÙÈ΋ ÂÛÙȷ΋ ÂÈÏË„›· Î·È Ô‰ËÁÔ‡Ó Û ÂÁÎÂÊ·ÏÈ΋ ·ÙÚÔÊ›· Î·È ¿ÓÔÈ· (30). ∞ÂÈÎÔÓÈÛÙÈÎÒ˜ (∂ÈÎfiÓ· 4), Ë ÈÛ¯·ÈÌÈ΋ ÂÚÈÔ¯‹ ‰ÂÓ ¤¯ÂÈ Û˘ÁÎÂÎÚÈ̤ÓË ·ÁÁÂȷ΋ ·Ó·ÙÔÌÈ΋ ηٷÓÔÌ‹, ÂÓÙÔ›˙ÂÙ·È ÛÙË ‚ÚÂÁÌ·ÙÔ˚Óȷ΋ ÂÚÈÔ¯‹ Î·È Ë Ì·ÁÓËÙÈ΋ Ê·ÛÌ·ÙÔÛÎÔ›· ÚˆÙÔÓ›Ô˘ (1HMRS) ‰Â›¯ÓÂÈ ·È¯Ì‹ Á·Ï·ÎÙÈÎÔ‡ (31). ∞˘ÍË̤ÓÔ Á·Ï·ÎÙÈÎfi Â›Ó·È Â‡ÚËÌ· ÈÔ Û˘¯Ófi ÛÙÔ ∂¡À ·Ú¿ ÛÙÔÓ ÔÚfi (32). ∂ÈÚfiÛıÂÙ· ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÌÔÚ› Ó· Â›Ó·È Ë Ì˘Ô¿ıÂÈ·, Ë Î·Ú‰ÈÔÌ˘Ô¿ıÂÈ·, Ë ÔÙÈ΋ ·ÙÚÔÊ›·, Ë ÂÚÈÊÂÚÈ΋ Ó¢ÚÔ¿ıÂÈ· ·ÍÔÓÈÎÔ‡ Ù‡Ô˘, ÙÔ ¯·ÌËÏfi ·Ó¿ÛÙËÌ·, Ô Û·Î¯·Ú҉˘ ‰È·‚‹Ù˘ Î·È Ë ÔÏ˘ÂÓ‰ÔÎÚÈÓÔ¿ıÂÈ· (33). ∞Ó¿ÚÎÂÈ· ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ I Â›Ó·È Ë ÈÔ Û˘¯Ó‹ ·Ó¿ÚÎÂÈ· Î·È ÛÙÔ 80% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ·Ó¢ڛÛÎÂÙ·È Ë ÛËÌÂȷ΋ ÌÂÙ¿ÏÏ·ÍË A3243G (14). ¡Â˘ÚÔÁÂÓ‹˜ Ì˘˚΋ ·‰˘Ó·Ì›·, ·Ù·Í›·, ÌÂÏ·Á¯ÚˆÛÙÈ΋ ·ÌÊÈ‚ÏËÛÙÚÔÂȉԿıÂÈ· & ÌËÙÚÈÎÒ˜ ÎÏËÚÔÓÔÌÔ‡ÌÂÓÔ
∂ÈÎfiÓ· 4. ™‡Ó‰ÚÔÌÔ MELAS: ∂ÁοÚÛÈ· ÙÔÌ‹ Flair ·ÎÔÏÔ˘ı›· (10 ÂÙÒÓ). ¶·ıÔÏÔÁÈÎfi Û‹Ì· ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ‚ÚÂÁÌ·ÙÈÎÔ‡ÈÓÈ·ÎÔ‡ ÏÔ‚Ô‡ ηٿ ÙË ‰È¿ÚÎÂÈ· ÂÓfi˜ stroke-like ÂÂÈÛÔ‰›Ô˘. ™ÙËÓ 1HMRS (Ì·ÎÚ¿ ·ÎÔÏÔ˘ı›·) ÌÂÁ¿ÏË ‰›ÎÔÚÊË ·È¯Ì‹ Á·Ï·ÎÙÈÎÔ‡ (‚¤ÏÔ˜). ¶·È‰È·ÙÚÈ΋ 2007;70:1-17
6
∞. ¡ÙÈÓfiÔ˘ÏÔ˜, ∞. ¶··‰ËÌËÙÚ›Ô˘
Û‡Ó‰ÚÔÌÔ Leigh (Neurogenic muscle weakness, ∞taxia, and Retinitis Pigmentosa - NARP & Maternal Inherited Leigh syndrome - MILS). ∆Ô Û‡Ó‰ÚÔÌÔ ÂÌÊ·Ó›˙ÂÙ·È Ì ÔÔÈÔÓ‰‹ÔÙÂ Û˘Ó‰˘·ÛÌfi ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ÙÔ˘ ·ÎÚˆÓ˘Ì›Ô˘ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ηı˘ÛÙ¤ÚËÛË Ù˘ ·Ó¿Ù˘Í˘, ÂÈÏË„›·, ¿ÓÔÈ· Î·È ·ÈÛıËÙËÚȷ΋ ÂÚÈÊÂÚÈ΋ Ó¢ÚÔ¿ıÂÈ·. ¶·ÚÂÁÎÂÊ·ÏȉÈ΋ ·Ù·Í›· ÎÔÚÌÔ‡ Î·È ¿ÎÚˆÓ Â›Ó·È ÂÌÊ·Ó‹˜, ÂÓÒ Ë Ì˘˚΋ ·‰˘Ó·Ì›· ‰‡ÛÎÔÏ· ·ÓȯÓ‡ÂÙ·È ÏfiÁˆ Ù˘ ·Ù·Í›·˜. ∆· ÙÂÓfiÓÙÈ· ·ÓÙ·Ó·ÎÏ·ÛÙÈο ·Ô˘ÛÈ¿˙Ô˘Ó Î·È ÛÙÔ ËÏÂÎÙÚÔÌ˘ÔÁÚ¿ÊËÌ· ˘¿Ú¯Ô˘Ó Â˘Ú‹Ì·Ù· ·ÍÔÓÈ΋˜ ·ÈÛıËÙËÚȷ΋˜ ÂÚÈÊÂÚÈ΋˜ Ó¢ÚÔ¿ıÂÈ·˜. ∂Ú˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜ ·Ô˘ÛÈ¿˙Ô˘Ó Î·È Ë ·ÓÙ›‰Ú·ÛË ÔÍÂȉ¿Û˘ ÙÔ˘ ΢ÙÔ¯ÚÒÌ·ÙÔ˜ (COX) Â›Ó·È ıÂÙÈ΋ (14). √È ÈÔ ÔÏÏÔ› ·ÛıÂÓ›˜ ʤÚÔ˘Ó ÙË ÛËÌÂȷ΋ ÌÂÙ¿ÏÏ·ÍË T8993G Ô˘ Έ‰ÈÎÔÔÈ› ÙËÓ ˘ÔÔÌ¿‰· 6 ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ V (ATP Û˘ÓıÂÙ¿ÛË). ∆Ô Û‡Ó‰ÚÔÌÔ ·ÔÙÂÏ› ¤Ó· ¿ÚÈÛÙÔ ·Ú¿‰ÂÈÁÌ· Ê·ÈÓÔÙ˘È΋˜ ÂÙÂÚÔÁ¤ÓÂÈ·˜ ÏfiÁˆ ÂÙÂÚÔÏ·ÛÌ›·˜: ÔÛÔÛÙfi ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ÛÙÔÓ Ì˘˚Îfi ÈÛÙfi ÌÈÎÚfiÙÂÚÔ ·fi 70% Â›Ó·È ·Û˘Ìو̷ÙÈ΋, ÔÛÔÛÙfi 70-90% ÚÔηÏ› ÓfiÛÔ NARP Î·È ÔÛÔÛÙfi ÌÂÁ·Ï‡ÙÂÚÔ ·fi 90% ÚÔηÏ› ÛÔ‚·Úfi Ê·ÈÓfiÙ˘Ô Û˘Ó‰ÚfiÌÔ˘ Leigh (34,35). ™‡Ó‰ÚÔÌÔ Leigh. ∆Ô 1951 Ô Leigh ÂÚȤÁÚ·„ ¤Ó· ·È‰› Ô˘ ˘¤Î˘„ ·fi ˘ÔÍ›· ÛÔ‚·Ú‹ ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ· Ì ·ıÔÏÔÁÔ·Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ ΢ڛˆ˜ ÛÙ· ‚·ÛÈο Á¿ÁÁÏÈ·, ÛÙÔÓ ı¿Ï·ÌÔ, ÛÙÔ˘˜ ˘Ôı·Ï¿ÌÈÔ˘˜ ˘Ú‹Ó˜ Î·È ÛÙÔ˘˜ ˘Ú‹Ó˜ ÙÔ˘ ÂÁÎÂÊ·ÏÈÎÔ‡ ÛÙÂϤ¯Ô˘˜ (36). ∏ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘ ÂÌÊ·Ó›˙ÂÙ·È Û˘Ó‹ıˆ˜ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, ·ÏÏ¿ ÌÔÚʤ˜ Ù˘ ÓfiÛÔ˘ ÌÔÚ› Ó· ÚÔ‚¿ÏÔ˘Ó Î·Ù¿ ÙËÓ ÚÒÈÌË Î·È fi„ÈÌË Ó·ÓÈ΋ ËÏÈΛ·. ™˘Ó‰˘¿˙ÂÙ·È Ì ÌÂÁ¿ÏÔ Â‡ÚÔ˜ Ó¢ÚÔÏÔÁÈÎÒÓ ÂΉËÏÒÛÂˆÓ fiˆ˜ „˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ÔÙÈ΋ ·ÙÚÔÊ›·, ÔÊı·ÏÌÔÏËÁ›·, ‚ÏÂÊ·ÚfiÙˆÛË, Ó˘ÛÙ·ÁÌÔÂȉ›˜ ÎÈÓ‹ÛÂȘ, ·Ù·Í›·, ÙÚfiÌÔ, ‰˘ÛÙÔÓ›·, ˘Ú·ÌȉÈ΋ Û˘Ó‰ÚÔÌ‹, Ù·¯‡ÓÔÈ· Î·È ¿ÓÔÈ· ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜ (37). ™ÙË ‰È¿ÁÓˆÛË ‚ÔËı¿ Ë ·ÂÈÎfiÓÈÛË ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ΢ڛˆ˜ Ì MRI, Ë ÔÔ›· ¤¯ÂÈ ·ÓÙÈηٷÛÙ‹ÛÂÈ ÙËÓ ·ıÔÏÔÁÔ·Ó·ÙÔÌÈ΋ ‰È¿ÁÓˆÛË. ™ÙËÓ MRI ÂÁÎÂÊ¿ÏÔ˘ ¤¯Ô˘Ì ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ ÛÙ· ‚·ÛÈο Á¿ÁÁÏÈ· Î·È ÂÁÎÂÊ·ÏÈÎfi Û٤ϯԘ Î·È ÛÙËÓ 1HMRS ¤¯Ô˘Ì ·È¯Ì‹ Á·Ï·ÎÙÈÎÔ‡ (∂ÈÎfiÓ· 5), ΢ڛˆ˜ ÛÙÔ ·ıÔÏÔÁÈÎfi Û‹Ì· (38-43). ∆Ô Û‡Ó‰ÚÔÌÔ Leigh Â›Ó·È ‚ÈÔ¯ËÌÈο Î·È ÁÂÓÂÙÈο ÂÙÂÚÔÁÂÓ‹˜ ÓfiÛÔ˜ Î·È ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙ› ÏfiÁˆ ‰È·Ù·Ú·¯‹˜ ‰È·ÊfiÚˆÓ ÂÓ˙˘ÌÈÎÒÓ Û˘ÌÏÂÁÌ¿ÙˆÓ Ô˘ Έ‰ÈÎÔÔÈÔ‡ÓÙ·È Â›Ù ·fi ÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi ›Ù ·fi ÙÔ ˘ÚËÓÈÎfi DNA (¶›Ó·Î·˜ 4). ªÈÙÔ¯ÔÓ‰Úȷ΋ Ó¢ÚÔ-Á·ÛÙÚÂÓÙÂÚÈ΋ ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ· (Mitochondrial Neuro-Gastro-Intestinal Encephalomyopathy - MNGIE). ∆o Û‡Ó‰ÚÔÌÔ ¯·Ú·Paediatriki 2007;70:1-17
ÎÙËÚ›˙ÂÙ·È ·fi ‰˘ÛÎÈÓËÙÈÎfiÙËÙ· ÙÔ˘ Á·ÛÙÚÂÓÙÂÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Ì „¢‰Ô·ÔÊÚ¿ÍÂȘ, η¯ÂÍ›·, ÚÔԉ¢ÙÈ΋ Â͈ÙÂÚÈ΋ ÔÊı·ÏÌÔÏËÁ›·, ÔÏ˘Ó¢ÚÔ¿ıÂÈ· Î·È Ï¢ÎÔÂÁÎÂÊ·ÏÔ¿ıÂÈ· (44). ™ÙË ‚ÚÂÊÈ΋ ËÏÈΛ· Ë ÓfiÛÔ˜ ÚÔ‚¿ÏÂÈ ÌÂ Û˘ÌÙÒÌ·Ù· ·fi ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi (Á·ÛÙÚÔ¿ÚÂÛË, ‰˘ÛÊ·Á›·, „¢‰Ô·fiÊÚ·ÍË) (45). ∏ ÎÈÓËÙÈÎÔ-·ÈÛıËÙËÚȷ΋ ÂÚÈÊÂÚÈ΋ Ó¢ÚÔ¿ıÂÈ· Â›Ó·È ÂÏ·ÊÚ¿˜ ÌÔÚÊ‹˜ Î·È ·ÔÌ˘ÂÏÈÓˆÙÈÎÔ‡ ¯·Ú·ÎÙ‹Ú·. ªÂÙ·‚È‚¿˙ÂÙ·È Ì ÙÔÓ ˘ÔÏÂÈfiÌÂÓÔ ÛˆÌ·ÙÈÎfi Ù‡Ô ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜ Î·È ÔÊ›ÏÂÙ·È Û ÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ı˘ÌȉÈÓÔʈÛÊˆÚ˘Ï¿Û˘ (thymidine phosphorylase), ÂÓ˙‡ÌÔ˘ ··Ú·›ÙËÙÔ˘ ÁÈ· ÙÔÓ Û¯ËÌ·ÙÈÛÌfi ÓÔ˘ÎÏÂÔÙȉ›ˆÓ. ∏ ‰È·Ù¿Ú·ÍË Ù˘ ÈÛÔÚÚÔ›·˜ ÛÙË ‰ÂÍ·ÌÂÓ‹ ÓÔ˘ÎÏÂÔÙȉ›ˆÓ ÚÔηÏ› ÔÏÏ·Ï¿ ÂÏÏ›ÌÌ·Ù· ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA (46). ∫ÏËÚÔÓÔÌÔ‡ÌÂÓË ÔÙÈ΋ Ó¢ڛÙȉ· ÙÔ˘ Leber (Leber’s Hereditary Optic Neuropathy - LHON). ∏ ÔÙÈ΋ Ó¢ڛÙȉ· ÙÔ˘ Leber ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ˘ÔÍ›· ÂÌÊ¿ÓÈÛË ÛÔ‚·Ú‹˜ ·ÌÊÔÙÂÚfiÏ¢Ú˘ ·ÒÏÂÈ·˜ Ù˘ fiÚ·Û˘ Ô˘ ·Ú·ÙËÚÂ›Ù·È Î˘Ú›ˆ˜ ÛÙÔ˘˜ ¿ÚÚÂÓ˜ ËÏÈΛ·˜ 11-30 ÂÙÒÓ. √ ̤ÛÔ˜ ¯ÚfiÓÔ˜ ·Ó¿ÌÂÛ· ÛÙËÓ ÚÔÛ‚ÔÏ‹ ÙˆÓ ‰‡Ô ÔÊı·ÏÌÒÓ Â›Ó·È 8 ‚‰ÔÌ¿‰Â˜ Î·È Ë ÚÔԉ¢ÙÈ΋ Âȉ›ӈÛË Ù˘ fiÚ·Û˘ Á›ÓÂÙ·È Û 1-6 ‚‰ÔÌ¿‰Â˜ (47). √È ·ÛıÂÓ›˜ ·Ú·ÔÓÔ‡ÓÙ·È ÁÈ· “ı¿Ì‚ˆÌ·” Ù˘ fiÚ·Û˘ Ô˘ ÛÙËÓ ÂÚÈÌÂÙÚÈ΋ ̤ÙÚËÛË ÙˆÓ ÔÙÈÎÒÓ Â‰›ˆÓ ·ÓÙÈÛÙÔȯ› Û ‰È‡ڢÓÛË ÙÔ˘ Ù˘ÊÏÔ‡ ÛËÌ›Ԣ Î·È ·ÒÏÂÈ· Ù˘ ÎÂÓÙÚÈ΋˜ fiÚ·Û˘. ¶fiÓÔ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÔÊı·ÏÌÈÎÒÓ ÎÈÓ‹ÛÂˆÓ Û¿ÓÈ· ÂÌÊ·Ó›˙ÂÙ·È, ÁÂÁÔÓfi˜ Ô˘ ‚ÔËı¿ ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ·fi ÙËÓ ÂÒ‰˘ÓË ÔÙÈ΋ Ó¢ڛÙȉ· Ô˘ ·Ú·ÙËÚÂ›Ù·È Û ÊÏÂÁÌÔÓÒ‰ÂȘ ηٷÛÙ¿ÛÂȘ. ø¯ÚfiÙËÙ· ÙÔ˘ ÔÙÈÎÔ‡ ‰›ÛÎÔ˘ ÂÌÊ·Ó›˙ÂÙ·È ÌÂÙ¿ ÙËÓ ÂÁηٿÛÙ·ÛË Ù˘ ·ÒÏÂÈ·˜ fiÚ·Û˘. √È ÂÚÈÛÛfiÙÂÚÔÈ ·ÛıÂÓ›˜ ʤÚÔ˘Ó ÔÌÔÈÔÏ·ÛÌÈΤ˜ ÛËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA Ô˘ Έ‰ÈÎÔÔÈ› ‰ÔÌÈΤ˜ ˘ÔÔÌ¿‰Â˜ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ I
°·Ï·ÎÙÈÎfi: ‰ÈÎfiÚ˘ÊË ·È¯Ì‹
∂ÈÎfiÓ· 5. ™‡Ó‰ÚÔÌÔ Leigh: ∆2 ·ÎÔÏÔ˘ı›· ÂÁοÚÛÈ· ÙÔÌ‹. √ÌÔÁÂÓ¤˜ ˘„ËÏfi Û‹Ì· ÛÙÔ Ú·‚‰ˆÙfi ÛÒÌ· Ì ÛÙÈÎÙ¤˜ ÂÚÈÔ¯¤˜ ¯·ÌËÏÔ‡ Û‹Ì·ÙÔ˜ Èı·ÓfiÓ ÏfiÁˆ ·˘ÍËÌ¤ÓˆÓ ÂÚÈ·ÁÁÂÈ·ÎÒÓ ¯ÒÚˆÓ. ™ÙË Ì·ÁÓËÙÈ΋ Ê·ÛÌ·ÙÔÛÎÔ›· ·Ú·ÙËÚÂ›Ù·È (1HMRS ‚Ú·¯Â›· ·ÎÔÏÔ˘ı›·) ÌÂÁ¿ÏË ·È¯Ì‹ Á·Ï·ÎÙÈÎÔ‡ (‚¤ÏÔ˜) (6).
7
ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜
¶›Ó·Î·˜ 3. ™‡Ó‰ÚÔÌ· Ì ڈÙÔ·ı‹ ‹ ‰Â˘ÙÂÚÔ·ı‹ ÌÈÙÔ¯ÔÓ‰Úȷ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ¡fiÛËÌ· / ∫ÏËÚÔÓÔÌÈÎfiÙËÙ·
∫ÏÈÓÈÎÔÂÚÁ·ÛÙËÚÈ·Îfi˜ Ê·ÈÓfiÙ˘Ô˜
°ÂÓÂÙÈ΋ ‚Ï¿‚Ë / ªË¯·ÓÈÛÌfi˜
∞Ù·Í›· ÙÔ˘ Freidreich / À™
∞Ù·Í›·, ·Ô˘Û›· ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ, ÛËÌÂ›Ô Babinski, ηډÈÔÌ˘Ô¿ıÂÈ·, ‰È·‚‹Ù˘, ÔÙÈ΋ ·ÙÚÔÊ›· ∂ÈÏË„›·, „˘¯Ôۈ̷ÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ·ÓˆÌ·Ï›Â˜ ÙÚȯÒÓ ÎÂÊ·Ï‹˜, ÔÛÙÈ΋ ¢ıÚ·˘ÛÙfiÙËÙ·, ÂÁÎÂÊ·ÏÈ΋ ·ÙÚÔÊ›·, AÙ·Í›· ÛȉËÚÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›·
ªÂÙ·ÏÏ¿ÍÂȘ (GAA repeat expansion) ÛÙÔ ÁÔÓ›‰ÈÔ Ù˘ ÊÚ·Ù·Í›Ó˘ (FRDA1)/
™‡Ó‰ÚÔÌÔ Menkes / ºÀ
∞Ù·Í›· ÛȉËÚÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›· / ºÀ ™‡Ó‰ÚÔÌÔ ÎÒʈÛ˘-‰˘ÛÙÔÓ›·˜ (Mohr-Tranebjaerg) / ºÀ ∫ÏËÚÔÓÔÌÔ‡ÌÂÓË Û·ÛÙÈ΋ ·Ú·ÏËÁ›· / À™ ™‡Ó‰ÚÔÌÔ Barth / ºÀ
™‡Ó‰ÚÔÌÔ Alper / À™ - ™ÔÚ·‰È΋
Charcot-Marie-Tooth type 2A
∞Ó¿ÚÎÂÈ· Û˘ÓÂÓ˙‡ÌÔ˘ Q10
ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ ATP7A Ô˘ Έ‰ÈÎÔÔÈ› ÙÔÓ ATPase ÌÂÙ·ÊÔÚ¤· ¯·ÏÎÔ‡ ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ ABC7 Ô˘ Έ‰ÈÎÔÔÈ› ÙÔÓ ÌÂÙ·ÊÔÚ¤· Ûȉ‹ÚÔ˘ ÛÙÔ ÌÈÙÔ¯fiÓ‰ÚÈÔ ∫ÒʈÛË, ‰˘ÛÙÔÓ›·, ηٷÚÚ¿ÎÙ˘, ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ DDP1/ ‰˘ÛÊ·Á›· TIMM8A Ô˘ Έ‰ÈÎÔÔÈ› ÙÔÓ ÚˆÙÂ˚ÓÈÎfi ÌÂÙ·ÊÔÚ¤· ™·ÛÙÈ΋ ·Ú·ÏËÁ›· ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ SPG7 ÁÔÓ›‰ÈÔ Ô˘ Έ‰ÈÎÔÔÈ› Ì›· ÌÂÙ·ÏÏÔÚˆÙ¿ÛË Ã·ÌËÏfi ·Ó¿ÛÙËÌ·, ηډÈÔÌ˘Ô¿ıÂÈ·, ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ Ù˘ Ô˘‰ÂÙÂÚÔÂÓ›· Î·È Ù·Ê·˙›Ó˘ Ô˘ ÚÔηÏ› ‰È·Ù·Ú·¯¤˜ 3-ÌÂı˘ÏÁÏÔ˘Ù·ÎÔÓÈ΋ ÔÍÂÔ˘Ú›· ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ Ù˘ ηډÈÔÏÈ›Ó˘ ÛÙË ÌÈÙÔ¯ÔÓ‰Úȷ΋ ÌÂÌ‚Ú¿ÓË ∞Ù·Í›·, Ì˘ÔÎÏÔÓÈ΋ ÂÈÏË„›·, ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ POLG1 Ë·ÙÔ¿ıÂÈ· (ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙ› Â›Ó·È ˘Â‡ı˘Ó˜ ÁÈ· ÙȘ ÂÚÈÙÒÛÂȘ ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ‚·ÏÚÔ˚ÎÔ‡ ÔͤԘ). Û˘Ó‰ÚfiÌÔ˘ Alper Ì ˷ÙÔ¿ıÂÈ· ¶ÚÔԉ¢ÙÈ΋ ÂÁÎÂÊ·ÏÈ΋ ·ÙÚÔÊ›· Î·È Ó¢ÚÔÂÎʇÏÈÛË ∞ÍÔÓÈÎÔ‡ Ù‡Ô˘ ÔÏ˘Ó¢ÚÔ¿ıÂÈ· ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ MFN2 Ô˘ Έ‰ÈÎÔÔÈ› ÙË ÌÈÙÔÛ˘ÓÙËÍ›ÓË 2, ÚˆÙ½ÓË ··Ú·›ÙËÙË ÁÈ· ÙËÓ ÎÈÓËÙÈÎfiÙËÙ· ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ñ ª˘Ô¿ıÂÈ· (ÂÚ˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜), ÷ÌËÏ¿ ›‰· ÔÏÈÎÔ‡ Q10 ÛÙÔ˘˜ Ì˘ÔÛÊ·ÈÚÈÓÔ˘Ú›·, Ì˘˜, Ê˘ÛÈÔÏÔÁÈ΋ ‰Ú·ÛÙÈÎfiÙËÙ· ·ÚÂÁÎÂÊ·ÏȉÈ΋ ·Ù·Í›· Û˘ÌÏÂÁÌ¿ÙˆÓ I, II, III, IV ·ÏÏ¿ ñ ™Ô‚·Ú‹ ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ· Î·È ÂÏ·Ùو̤ÓË ‰Ú·ÛÙÈÎfiÙËÙ· ÓÂÊÚÈ΋ ·Ó¿ÚÎÂÈ· ÙˆÓ I-III Î·È II-III. ÕÁÓˆÛÙË ÁÂÓÂÙÈ΋ ‚Ï¿‚Ë
À™: ÀÔÏÂÈfiÌÂÓÔ ™ˆÌ·ÙÈÎfi, ∂™: ∂ÈÎÚ·ÙÔ‡Ó ™ˆÌ·ÙÈÎfi, ºÀ: º˘ÏÔÛ‡Ó‰ÂÙÔ ÀÔÏÂÈfiÌÂÓÔ
(ND1, ND4, ND6). ∞ÔηٿÛÙ·ÛË Ù˘ fiÚ·Û˘ Â›Ó·È Ôχ Û¿ÓÈ· fiÙ·Ó ÔÈ ·ÛıÂÓ›˜ ʤÚÔ˘Ó ÙË ÌÂÙ¿ÏÏ·ÍË G11778A, ·ÏÏ¿ Û¯ÂÙÈο Û˘¯Ó‹ Û ·ÛıÂÓ›˜ Ì ÙË ÌÂÙ¿ÏÏ·ÍË T14484C (48). ÕÏϘ Ó¢ÚÔÏÔÁÈΤ˜ ÂΉËÏÒÛÂȘ Â›Ó·È Û¿ÓȘ (49). ™‡Ó‰ÚÔÌÔ ÔÛÔÙÈ΋˜ Ì›ˆÛ˘ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA (Mitochondrial DNA depletion syndrome (MDS). ∞ÔÙÂÏÔ‡Ó ·Ûı¤ÓÂȘ Ù˘ ‚ÚÂÊÈ΋˜ ËÏÈΛ·˜ Ô˘ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÙÔ˘˜ Â›Ó·È ÔÛÔÙÈ΋ ‰È·Ù·Ú·¯‹ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA ÏfiÁÔ˘ ÂÏ¿ÙÙˆÛ˘ Ù˘ ‰ÂÍ·ÌÂÓ‹˜ ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ‰ÂÔ͢ÚÈ‚ÔÓÔ˘ÎÏÂÔÙȉ›ˆÓ (dNTPs) ··Ú·›ÙËÙˆÓ ÁÈ· ÙË Û‡ÓıÂÛË ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA. À¿Ú¯Ô˘Ó ‰‡Ô ¤Ó˙˘Ì· Ô˘ ÂϤÁ¯Ô˘Ó ·˘Ù‹ ÙË ‰ÂÍ·ÌÂÓ‹. ∏ ÎÈÓ¿ÛË Ù˘ ‰ÂÔ͢ÁÔ˘·ÓÔÛ›Ó˘ (dGK) ÌÂÙ·ÏÏ¿ÍÂȘ Ù˘ ÔÔ›·˜ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔÓ Ë·ÙÔÂÁÎÂÊ·ÏÈÎfi Ù‡Ô Î·È Ë ÎÈÓ¿ÛË Ù˘ ı˘Ìȉ›Ó˘ (TK2) ÌÂÙ·ÏÏ¿ÍÂȘ Ù˘ ÔÔ›·˜ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔÓ Ì˘Ô·ıËÙÈÎfi Ù‡Ô (50).
ªËÙÚÈÎÒ˜ ÎÏËÚÔÓÔÌÔ‡ÌÂÓË Ó¢ÚÔ·ÈÛıËÙËÚȷ΋ ·ÒÏÂÈ· ·ÎÔ‹˜ (Maternal Inherited Hearing Loss - MIHL). ∆· ÙÚȯˆÙ¿ ·ÙÙ·Ú· ÙÔ˘ Îԯϛ· Â›Ó·È ·ÚÎÂÙ¿ ¢·›ÛıËÙ· ÛÙË ‰È·Ù·Ú·¯‹ Ù˘ ÌÈÙÔ¯ÔÓ‰Úȷ΋˜ ·Ú·ÁˆÁ‹˜ ÂÓ¤ÚÁÂÈ·˜. ∏ ÛËÌÂȷ΋ ÌÂÙ·ÏÏ¿ÍË, Û˘Ó‹ıˆ˜ Û ÔÌÔÈÔÏ·ÛÌ›·, ÛÙË ı¤ÛË G1555A ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA Â›Ó·È Û˘¯Ófi ‡ÚËÌ· Ó¢ÚÔ·ÈÛıËÙËÚȷ΋˜ ·ÒÏÂÈ·˜ ·ÎÔ‹˜. ∂ÈÚÔÛı¤Ùˆ˜, ¿ÙÔÌ· Ô˘ ʤÚÔ˘Ó ÙË ÌÂÙ¿ÏÏ·ÍË C1494T Â›Ó·È Â˘·›ÛıËÙ· ÛÙËÓ ÚfiÎÏËÛË ·ÒÏÂÈ·˜ ·ÎÔ‹˜ ÌÂÙ¿ ·fi ¤ÎıÂÛË ÛÙȘ ·ÌÈÓÔÁÏ˘ÎÔÛ›‰Â˜ (51,52). ªÈÙÔ¯ÔÓ‰Úȷ΋ Ì˘ÔηډÈÔ¿ıÂÈ·. √È ÈÔ Û˘¯Ó¤˜ ηډȷΤ˜ ·ÓˆÌ·Ï›Â˜ Â›Ó·È ÔÈ ‰È·Ù·Ú·¯¤˜ ·ÁˆÁÈÌfiÙËÙ·˜ Ô˘ Â›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Kearns-Sayre Î·È Ë ˘ÂÚÙÚÔÊÈ΋ ηډÈÔÌ˘Ô¿ıÂÈ· (53). ∏ ‰È·Ù·ÙÈ΋ ηډÈÔÌ˘Ô¿ıÂÈ· Â›Ó·È ÏÈÁfiÙÂÚÔ Û˘¯Ó‹. °ÂÓÂÙÈΤ˜ ‚Ï¿‚˜ Ô˘ Û˘Ó‰˘¿˙ÔÓÙ·È Ì ÙËÓ ·ÚÔ˘Û›· ηډÈÔÌ˘Ô¿ıÂÈ·˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ¶·È‰È·ÙÚÈ΋ 2007;70:1-17
8
∞. ¡ÙÈÓfiÔ˘ÏÔ˜, ∞. ¶··‰ËÌËÙÚ›Ô˘
¶›Ó·Î·˜ 4. ∂Ó˙˘ÌÈΤ˜ ‰È·Ù·Ú·¯¤˜ Î·È ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ˘ÚËÓÈÎÔ‡ ‹ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA Ô˘ Û˘Ó‰˘¿˙ÔÓÙ·È Ì ۇӉÚÔÌÔ Leigh ªÂÙ·ÏÏ¿ÍÂȘ ˘ÚËÓÈÎÔ‡ DNA (˘ÔÏÂÈfiÌÂÓË ÎÏËÚÔÓÔÌÈÎfiÙËÙ·) ñ ªÂÙ·ÏÏ¿ÍÂȘ ÁÔÓȉ›ˆÓ Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙË Û˘Ó·ÚÌÔÏfiÁËÛË ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ IV (΢ÙÔ¯ÚˆÌÈ΋˜ ÔÍÂȉ¿Û˘ COX) Ô˘ ΢ڛˆ˜ ·ÊÔÚ¿ ÙÔ SURF1 ÁÔÓ›‰ÈÔ Î·È ÈÔ Û¿ÓÈ· ÙÔ SCO2 (ÂÁÎÂÊ·ÏÔ¿ıÂÈ·, ˘ÂÚÙÚÔÊÈ΋ ηډÈÔÌ˘Ô¿ıÂÈ·) Î·È SCO1 ÁÔÓ›‰È· (57,77) ñ ªÂÙ·ÏÏ¿ÍÂȘ ÁÔÓȉ›ˆÓ Ô˘ ·ÊÔÚÔ‡Ó ‰ÔÌÈΤ˜ ˘ÔÔÌ¿‰Â˜ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ I (NDUFS1, NDUFS8, NDUFS4, NDUFS7, NDUFS2, NDUFV2) (78) ñ ªÂÙ¿ÏÏ·ÍË SDHA ÁÔÓȉ›Ô˘ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ ππ (79) ñ ªÂÙ¿ÏÏ·ÍË BSC1 ÁÔÓȉ›Ô˘ Ô˘ Û˘ÌÌÂÙ¤¯ÂÈ ÛÙË Û˘Ó·ÚÌÔÏfiÁËÛË (chaperone) ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ πππ (80) ªÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA ñ ªÂÙ·ÏÏ¿ÍÂȘ ÁÔÓȉ›ˆÓ Ù˘ ATP Û˘ÓıÂÙ¿Û˘ (NARP/Leigh) A8344G (tRNALys) G8363A ñ ªÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ÁÔÓ›‰È· ND3 ‹ ND5 Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ‰ÔÌÈΤ˜ ÌÔÓ¿‰Â˜ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ π (81,82) ñ ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ COXIII ÁÔÓ›‰ÈÔ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ IV ¶ÔÛÔÙÈ΋ Ì›ˆÛË ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA ñ ¶ÔÏÏ·Ï‹ Û˘ÌÏÂÁÌ·ÙÈ΋ ·Ó¿ÚÎÂÈ· (83) ÕÏÏˆÓ ÂÓ˙˘ÌÈÎÒÓ ·Ó·ÚÎÂÈÒÓ ñ ∞Ó¿ÚÎÂÈ· ˘ÚÔÛÙ·Ê˘ÏÈ΋˜ ·Ê˘‰ÚÔÁÔÓ¿Û˘ (E1a, E3, E3BP, ÚÔÛÊ¿Ùˆ˜ E1b) ñ ∞Ó¿ÚÎÂÈ· ‚ÈÔÙÈÓȉ¿Û˘ (ÔÏÏ·Ï‹ ·Ó¿ÚÎÂÈ· ηڂÔÍ˘Ï·ÛÒÓ) (84) ñ ∞Ó¿ÚÎÂÈ· ˘ÚÔÛÙ·Ê˘ÏÈ΋˜ ηڂÔÍ˘Ï¿Û˘
i. ™ËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA (54) ii. ªÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ˘ÚËÓÈÎÔ‡ DNA Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ‰ÔÌÈΤ˜ ÌÔÓ¿‰Â˜ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ I (NDUFS2, NDUFV2) (55) iii. ∞Ó¿ÚÎÂÈ· ÙÔ˘ ΢ÙÔ¯ÚÒÌ·ÙÔ˜ b (56) iv. ªÂÙ¿ÏÏ·ÍË ÛÙÔ SCO2 ÁÔÓ›‰ÈÔ Ô˘ Έ‰ÈÎÔÔÈ› ÚˆÙ½ÓË ··Ú·›ÙËÙË ÁÈ· ÙË Û˘Ó·ÚÌÔÏfiÁËÛË ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ IV (57) v. ¶ÔÛÔÙÈ΋ Ì›ˆÛË ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA (58). ªÈÙÔ¯ÔÓ‰Úȷ΋ Ì˘Ô¿ıÂÈ· Î·È ‰˘Û·ÓÂÍ›· ÛÙËÓ ¿ÛÎËÛË (Isolated myopathy and exercise intolerance). ¶ÔÏϤ˜ ÊÔÚ¤˜ Ì˘˚΋ ·‰˘Ó·Ì›· ÌÔÚ› Ó· Â›Ó·È ÙÔ ÌfiÓÔ ÎÏÈÓÈÎfi ‡ÚËÌ·. ∏ ÔÚ›· Â›Ó·È ·ÚÁ‹ Î·È ÚÔԉ¢ÙÈ΋ Î·È Û˘Ó‹ıˆ˜ ·ÊÔÚ¿ ÙȘ ÂÁÁ‡˜ Ì˘˚Τ˜ ÔÌ¿‰Â˜. ∏ Û˘Ìو̷ÙÔÏÔÁ›· ÂÚÈÏ·Ì‚¿ÓÂÈ ÌÂȈ̤ÓË ·ÓÙÔ¯‹ ÛÙËÓ ¿ÛÎËÛË, ¯ÚfiÓÈ· ÎfiˆÛË, Ì˘˚ο ¿ÏÁË Î·È Û·Ó›ˆ˜ ÂÂÈÛfi‰È· Ú·‚‰ÔÌ˘fiÏ˘Û˘ (59,60). ∏ ηٿÛÙ·ÛË Â›Ó·È ÁÂÓÂÙÈÎÒ˜ ÂÙÂÚÔÁÂÓ‹˜ Î·È ¤¯ÂÈ Û˘Ó‰˘·ÛÙ› ÌÂ: ·) ÛËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ Ô˘ Έ‰ÈÎÔÔÈ› ÙÔ Î˘Ùfi¯ÚˆÌ· b, ‚) ¤ÏÏÂÈÌÌ· ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA Î·È Á) Ì ÂÚÈÙÒÛÂȘ ÔÛÔÙÈ΋˜ Ì›ˆÛ˘ (depletion) ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA (14).
¢È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË Î·È ÎÚÈÙ‹ÚÈ· ∏ ‰˘ÛÎÔÏ›· ÛÙË ‰È·ÁÓˆÛÙÈ΋ ÚÔۤϷÛË ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ÔÊ›ÏÂÙ·È ÛÙË ÁÂÓÂÙÈ΋ Î·È Ê·ÈÓÔÙ˘È΋ ÂÙÂÚÔÁ¤ÓÂÈ· Î·È ÛÙËÓ ·Ô˘Û›· ÂȉÈÎÒÓ ‰È·ÁÓˆÛÙÈÎÒÓ Ù¯ÓÈÎÒÓ. ∏ ÎÏËÚÔÓÔÌÈÎfiÙËPaediatriki 2007;70:1-17
Ù· ÔÈΛÏÂÈ Î·È ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi Â›Ó·È ·ÚÓËÙÈÎfi. ™Ù· ·È‰È¿ Ë ‰È¿ÁÓˆÛË Â›Ó·È ·ÎfiÌË ÈÔ ‰‡ÛÎÔÏË ÁÈ·Ù›: ·) Ù· ÎÏÈÓÈο ÌÈÙÔ¯ÔÓ‰Úȷο Û‡Ó‰ÚÔÌ· Â›Ó·È Û¿ÓÈ·, ‚) Ù· Â˘Ú‹Ì·Ù· ·fi ÙË Ì˘˚΋ ‚ÈÔ„›· ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙÔ˘˜ Â›Ó·È ¿Ù˘· Î·È Á) Ô ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‰ÂÓ Â›Ó·È ¿ÓÙ· ·ÔÎ·Ï˘ÙÈÎfi˜ (61). ∏ ÚÔÛ¤ÁÁÈÛË ·ÛıÂÓÔ‡˜ Ì ˘Ô„›· ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ ÓÔÛ‹Ì·ÙÔ˜ Ú¤ÂÈ Ó· ÂÍ·ÙÔÌÈ·ÂÙ·È Î·È ··ÈÙ› ÙËÓ Ù·˘Ùfi¯ÚÔÓË ·ÍÈÔÏfiÁËÛË ÎÏÈÓÈÎÒÓ, Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎÒÓ, ‚ÈÔ¯ËÌÈÎÒÓ, ÈÛÙÔ·ıÔÏÔÁÈÎÒÓ Î·È ÁÂÓÂÙÈÎÒÓ ÏËÚÔÊÔÚÈÒÓ. °È· ÙËÓ Â˘ÎÔÏfiÙÂÚË Î·ÙËÁÔÚÈÔÔ›ËÛË Î·È ‰È¿ÁÓˆÛË ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ¤¯Ô˘Ó ÚÔÙ·ı› Î·È ÙÚÔÔÔÈËı› ÁÈ· Ù· ·È‰È¿ ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· (¶›Ó·Î·˜ 5) (62). ∆· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ‚ÔËıÔ‡Ó ÒÛÙ ӷ Ù·ÍÈÓÔÌËı› Ô ·ÛıÂÓ‹˜ ·Ó¿ÏÔÁ· Ì ÙË ‰È·ÁÓˆÛÙÈ΋ ‚‚·ÈfiÙËÙ· (“ÔÚÈÛÙÈ΋”, “Èı·Ó‹”, “‰˘Ó·Ù‹”) Ó· ʤÚÂÈ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi ÓfiÛËÌ·. µÈÔ¯ËÌÈ΋ ÌÂϤÙË ∏ ·ÓˆÌ·Ï›· Ù˘ ÔÍÂȉˆÙÈ΋˜ ʈÛÊÔÚ˘Ï›ˆÛ˘ ÚÔηÏ› ¿ÌÂÛË Î·È ¤ÌÌÂÛË ‰È·Ù·Ú·¯‹ ‰È·ÊfiÚˆÓ ÌÂÙ·‚ÔÏÈÎÒÓ Ô‰ÒÓ fiˆ˜ Ù˘ ÁÏ˘ÎfiÏ˘Û˘, ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ ˘ÚÔÛÙ·Ê˘ÏÈÎÔ‡ ÔͤԘ, ÙÔ˘ ·ÎÏÔ˘ ÙÔ˘ Krebs Î·È ÙÔ˘ ηٷ‚ÔÏÈÛÌÔ‡ ÚˆÙÂ˚ÓÒÓ. ÕÌÂÛË Û˘Ó¤ÂÈ· ÙˆÓ ‰È·Ù·Ú·¯ÒÓ ·˘ÙÒÓ Â›Ó·È Ë ·‡ÍËÛË ÛÙÔÓ ÔÚfi Î·È ∂¡À ÙÔ˘ Á·Ï·ÎÙÈÎÔ‡ Î·È ˘ÚÔÛÙ·Ê˘ÏÈÎÔ‡ (·‡ÍËÛË ÙÔ˘ ÏfiÁÔ˘ Á·Ï·ÎÙÈÎfi/˘ÚÔÛÙ·Ê˘ÏÈÎfi ¿Óˆ ·fi 20) Î·È Ù˘ ·Ï·Ó›Ó˘. ™ÙÔÓ ÔÚfi ¤¯Ô˘Ì ›Û˘
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ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜
·‡ÍËÛË ÙˆÓ ÎÂÙÔÓÔÛˆÌ¿ÙˆÓ È‰È·›ÙÂÚ· ÌÂÙ¿ ÙË Ï‹„Ë ÙÚÔÊ‹˜ (·Ú¿‰ÔÍË ÎÂÙÔÓ·ÈÌ›·). ∏ ʈÛÊÔÎÈÓ¿ÛË Ù˘ ÎÚ·ٛÓ˘ (CPK) Â›Ó·È Û˘Ó‹ıˆ˜ Ê˘ÛÈÔÏÔÁÈ΋ ‹ ÂÏ·ÊÚ¿ ·˘ÍË̤ÓË Û ÌÂÚÈΤ˜ ÌÈÙÔ¯ÔÓ‰ÚȷΤ˜ Ì˘Ô¿ıÂȘ. √ÚÈṲ̂ӷ ÌÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· ÚÔÛ‚¿ÏÔ˘Ó ÙÔ ‹·Ú Ì ‰È·Ù·Ú·¯‹ ÙˆÓ Ë·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ Î·È ¿ÏÏ· ÙÔ˘˜ ÓÂÊÚÔ‡˜ Ì ÂÌÊ¿ÓÈÛË ÓÂÊÚÈ΋˜ ÛˆÏËÓ·Úȷ΋˜ ÔͤˆÛ˘ (De-Toni-Fabre-Fanconi). ∏ ·Ó·ÈÌ›· ÌÔÚ› Ó· Â›Ó·È Â›Ù ڈÙÔ·ı‹˜ (ÛȉËÚÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›·) ›Ù ‰Â˘ÙÂÚÔ·ı‹˜ ÏfiÁˆ ÓÂÊÚÔ¿ıÂÈ·˜. ¢˘ÛÙ˘¯Ò˜, ÙÔ ‚ÈÔ¯ËÌÈÎfi ÌÂÙ·‚ÔÏÈÎfi screening ‰ÂÓ Â›Ó·È ¿ÓÙ· ·ÔÎ·Ï˘ÙÈÎfi, Î·È Ù· ‰È·ÁÓˆÛÙÈο Ï¿ıË Â›Ó·È Û˘¯Ó¿ (13,63). √ ¶›Ó·Î·˜ 6 ÂÚÈÁÚ¿ÊÂÈ ÙÔÓ ‚ÈÔ¯ËÌÈÎfi ÌÂÙ·‚ÔÏÈÎfi ¤ÏÂÁ¯Ô Ô˘ Û˘ÓÈÛÙ¿Ù·È Â› ˘Ô„›·˜ ÓÔÛ‹Ì·ÙÔ˜ Ì ÌÈÙÔ¯ÔÓ‰Úȷ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·. πÛÙÔ·ıÔÏÔÁ›· Ì˘˚ÎÔ‡ ÈÛÙÔ‡ ∆Ô ÈÛÙÔÏÔÁÈÎfi Û‹Ì· ηٷÙÂı¤Ó ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈÔ·ıÂÈÒÓ Â›Ó·È ÔÈ ÂÚ˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜ (ragged red fibers) Ô˘ ·ÓȯÓ‡ÔÓÙ·È Ì ÙË ¯ÚÒÛË Gomoritrichrome (∂ÈÎfiÓ· 6·) Î·È ÙË ¯ÚÒÛË ÛÔ˘ÎÈÓÈ΋˜ ·Ê˘‰ÚÔÁÔÓ¿Û˘ (∂ÈÎfiÓ· 6‚). √È ÂÚ˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜ Û¯ËÌ·Ù›˙ÔÓÙ·È ·fi ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi Î·È ÙË Û˘ÁΤÓÙÚˆÛË ˘ÔÛ·ÚÎËÏÂÈÌÌ·ÙÈÎÒÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ Î·È ÙËÓ ÂÎʇÏÈÛË ÙˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ. √È ÈÛÙÔÏÔÁÈΤ˜ ·˘Ù¤˜ ·ÏÏÔÈÒÛÂȘ ·Ú·ÙËÚÔ‡ÓÙ·È Î˘Ú›ˆ˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ Î·È Û˘¯ÓfiÙÂÚ· Û ÌÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· ·fi ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA. ∂Ú˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜ Û·Ó›ˆ˜ ·ÓȯÓ‡ÔÓÙ·È ÛÙË ‚ÚÂÊÈ΋ Î·È ·È‰È΋ ËÏÈΛ·, ÂÓÒ ÈÔ Û˘¯Ófi ‡ÚËÌ· Â›Ó·È Ë ·ÚÔ˘Û›· ˘ÔÛ·ÚÎËÏÂÈÌÌ·ÙÈÎÒÓ Û˘ÛÛˆÚ‡ÛÂˆÓ (64). ∏ ·ÓÙ›‰Ú·ÛË Ù˘ ÔÍÂȉ¿Û˘ ÙÔ˘ ΢ÙÔ¯ÚÒÌ·ÙÔ˜ (COX) ÔÈΛÏÂÈ. ÕÏϘ ÌË ÂȉÈΤ˜ ÈÛÙÔ·ıÔÏÔÁÈΤ˜ ·ÏÏÔÈÒÛÂȘ Â›Ó·È Û˘¯Ó¤˜ ·ÏÏ¿ ÌË ‰È·ÁÓˆÛÙÈΤ˜ (65). ∏ ·Ó¿Ï˘ÛË Ì ËÏÂÎÙÚÔÓÈÎfi ÌÈÎÚÔÛÎfiÈÔ ÌÔÚ› Ó· ·ÓȯÓ‡ÛÂÈ ‰ÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ fiˆ˜ ·Ú·ÎÚ˘ÛÙ·ÏÏÈο ¤ÁÎÏÂÈÛÙ·, ˘ÂÚÌÂÁ¤ıË ÌÈÙÔ¯fiÓ‰ÚÈ·, ÌÈÙÔ¯fiÓ‰ÚÈ· Ì ·ÏÏÔÈÒÛÂȘ ÙˆÓ ·ÎÚÔÏÔÊÈÒÓ, ·˘ÍË̤ÓË ÔÈÎÈÏÔÌÔÚÊ›· ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ Î·È ˘ÔÛ·ÚÎÂÈÏËÌÌ·ÙÈΤ˜ Î·È ÂÓ‰ÔÌ˘Ô˚ÓȉȷΤ˜ Û˘ÛÛˆÚ‡ÛÂȘ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ. ∏ ·ÚÔ˘Û›· ·Ú·ÎÚ˘ÛÙ·ÏÏÈÎÒÓ ¤ÁÎÏÂÈ-
·
ÛÙˆÓ Î·È Û˘ÛÛˆÚ‡ÛÂȘ ·ıÔÏÔÁÈÎÒÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ Â›Ó·È ÂȉÈÎÔ› ‰ÔÌÈÎÔ› ‰Â›ÎÙ˜ ÌÈÙÔ¯ÔÓ‰ÚÈÔ¿ıÂÈ·˜ (∂ÈÎfiÓ· 7 ) ·ÏÏ¿ ‰ÂÓ ·ÓȯÓ‡ÔÓÙ·È ¿ÓÙ·. ∂Ó˙˘Ì·ÙÈ΋ ̤ÙÚËÛË Î·È ÔÍÂȉˆÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÛÙÔ˘˜ ÈÛÙÔ‡˜ ∏ ÂÓ˙˘ÌÈ΋ ·Ó¿Ï˘ÛË ÚÔÙÈÌ¿Ù·È Ó· Á›ÓÂÙ·È Û ÊÚ¤ÛÎÔ Ì˘˚Îfi ÈÛÙfi, ÂÂȉ‹ Ù· ÌÈÙÔ¯fiÓ‰ÚÈ· ·Ú·Ì¤ÓÔ˘Ó ·Î¤Ú·È· Î·È ¤ÙÛÈ ÌÔÚ› Ó· ÌÂÙÚËı› Ô ‚·ıÌfi˜ ÔÍ›‰ˆÛ˘ Î·È Ë ·Ú·ÁˆÁ‹ ATP. ∏ ̤ÙÚËÛË Ù˘ ÔÍÂȉˆÙÈ΋˜ ÈηÓfiÙËÙ·˜ Û ÊÚ¤Ûη Ì˘˚ο ÌÈÙÔ¯fiÓ‰ÚÈ· Á›ÓÂÙ·È Â›Ù Ì ÔÏ·ÚÔÁÚ·ÊÈ΋ ̤ıÔ‰Ô Â›Ù Ì ڷ‰ÈÔ¯ËÌÈ΋ ̤ıÔ‰Ô (6). ∆Ô Úfi‚ÏËÌ· Ì ÙË ‰ÈÂÚ‡ÓËÛË Û ÊÚ¤ÛÎÔ ÈÛÙfi Â›Ó·È fiÙÈ ÙÔ ‰Â›ÁÌ· Ú¤ÂÈ Ó· ÊÙ¿ÛÂÈ ÛÙÔ ÂÚÁ·ÛÙ‹ÚÈÔ Ì¤Û· Û 2-3 ÒÚ˜ ·fi ÙË ‚ÈÔ„›· Î·È Ë ‰ÔÎÈÌ·Û›· Á›ÓÂÙ·È ÌfiÓÔ Û ϛÁ· ÂÍÂȉÈÎÂ˘Ì¤Ó· ÂÚÁ·ÛÙ‹ÚÈ·. ∞Ó Ë ÂͤٷÛË Û ÊÚ¤ÛÎÔ ÈÛÙfi ‰ÂÓ Â›Ó·È ‰˘Ó·Ù‹, ÙÔ ‰Â›ÁÌ· Ú¤ÂÈ Ó· ηٷ„‡¯ÂÙ·È ·Ì¤Ûˆ˜ Û ˘ÁÚfi ¿˙ˆÙÔ, ¯ˆÚ›˜ ̤ÛÔ, Î·È Ó· Ê˘Ï¿ÛÛÂÙ·È ÛÙÔ˘˜ -80ÔC. ªÂÙ·ÊÔÚ¿ ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜ ÛÙÔ ÂÚÁ·ÛÙ‹ÚÈÔ Ú¤ÂÈ Ó· Á›ÓÂÙ·È Ì ÍËÚfi ¿ÁÔ. ™ÙËÓ ÂÚ›ÙˆÛË ·˘Ù‹ Ë Ì¤ÙÚËÛË Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÂÓ˙‡ÌˆÓ Á›ÓÂÙ·È Û ÔÌÔÁÂÓÔÔÈË̤ÓÔ ˘ÏÈÎfi ÙÔ˘ ÈÛÙÔ‡ Ì ÙË Ì¤ıÔ‰Ô Ù˘ Ê·ÛÌ·ÙÔʈÙÔÌÂÙÚ›·˜ (66). ∞ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ∂› ÎÏÈÓÈ΋˜ ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ·˜ Ô Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Â›Ó·È ··Ú·›ÙËÙÔ˜ ÁÈ·Ù› ÌÔÚ›
‚
∂ÈÎfiÓ· 6. ·) ÃÚÒÛË Gomori Trichrome: ÂÚ˘ıÚ¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜ (Ï¢Îfi ‚¤ÏÔ˜) Î·È ˘ÔÛ·ÚÎËÏÂÈÌÌ·ÙÈΤ˜ Û˘ÛÛˆÚ‡ÛÂȘ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ (Ì·‡Ú· ‚¤ÏË) ‚) ÃÚÒÛË SDH “΢·Ó¤˜ Ú·ÎÒ‰ÂȘ ›Ó˜” (ÂÚ˘ıÚfi ‚¤ÏÔ˜).
∂ÈÎfiÓ· 7. ¶·Ú·ÎÚ˘ÛÙ·ÏÏÈο ¤ÁÎÏÂÈÛÙ· ÛÙÔÓ ‰È·ÌÂÌ‚Ú·ÓÈÎfi ¯ÒÚÔ ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ˘fi ÙË ÌÔÚÊ‹ “¯ÒÚÔ˘ ÛÙ¿ıÌ¢Û˘ (parking lot)” ‚¤ÏÔ˜. ¶·È‰È·ÙÚÈ΋ 2007;70:1-17
10
∞. ¡ÙÈÓfiÔ˘ÏÔ˜, ∞. ¶··‰ËÌËÙÚ›Ô˘
¶›Ó·Î·˜ 5. ∆ÚÔÔÔÈË̤ӷ ÎÚÈÙ‹ÚÈ· ÂÓËÏ›ÎˆÓ ÁÈ· ÙË ‰È¿ÁÓˆÛË ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ªÂ›˙ÔÓ· ∫ÚÈÙ‹ÚÈ·
∂Ï¿ÛÛÔÓ· ∫ÚÈÙ‹ÚÈ·
∫ÏÈÓÈ΋ ÂΉ‹ÏˆÛË ¶Ï‹Ú˘ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÌÈÙÔ¯ÔÓ‰Úȷ΋˜ ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ·˜* ‹ ÎÏÈÓÈ΋ ÂÈÎfiÓ· Ô˘ ÏËÚÔ› ÙÚÂȘ ÚÔ¸Ôı¤ÛÂȘ: ñ ¶ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ Û˘Ìو̷ÙÔÏÔÁ›· Ì ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘Ï¿¯ÈÛÙÔÓ ÙÚÈÒÓ ÔÚÁ·ÓÈÎÒÓ Û˘ÛÙËÌ¿ÙˆÓ (∫¡™, Ì˘˚Îfi, ηډȷÁÁÂÈ·Îfi, ÓÂÊÚÈÎfi, Á·ÛÙÚÂÓÙÂÚÈÎfi, Ë·ÙÈÎfi, ÂÓ‰ÔÎÚÈÓÈÎfi, ·ÈÌ·ÙÔÏÔÁÈÎfi) ‹ ÔÚÁ¿ÓˆÓ (·ÎÔ‹, fiÚ·ÛË, ‰˘ÛÌÔÚÊ›·) ñ ¶ÚÔԉ¢ÙÈ΋ ÎÏÈÓÈ΋ ÔÚ›· Ì ÂÍ¿ÚÛÂȘ ‹ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi Ì Èı·Ó‹ ÌÈÙÔ¯ÔÓ‰ÚÈÔ¿ıÂÈ· ñ ∞ÔÎÏÂÈÛÌfi˜ ¿ÏÏˆÓ ÌÂÙ·‚ÔÏÈÎÒÓ ‹ Ó¢ÚÔÂÎÊ˘ÏÈÛÙÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ πÛÙÔ·ıÔÏÔÁ›· >2% ÂÚ˘ıÚÒÓ Ú·Îˆ‰ÒÓ ÈÓÒÓ Û ÛÎÂÏÂÙÈÎfi Ì˘˚Îfi ÈÛÙfi ∂Ó˙˘ÌÔÏÔÁ›·** ñ >2% COX-·ÚÓËÙÈΤ˜ ›Ó˜ (<50 ÂÙÒÓ) ñ >5% COX-·ÚÓËÙÈΤ˜ ›Ó˜ (>50 ÂÙÒÓ) ñ <20% ‰Ú·ÛÙÈÎfiÙËÙ· ÂÓ˙˘ÌÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Û ÈÛÙfi ñ <30% ‰Ú·ÛÙÈÎfiÙËÙ· ÂÓ˙˘ÌÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Û ΢ÙÙ·ÚÈ΋ ÁÚ·ÌÌ‹ ñ <30% ‰Ú·ÛÙÈÎfiÙËÙ· ÂÓ˙˘ÌÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Û ‰‡Ô ÈÛÙÔ‡˜ §ÂÈÙÔ˘ÚÁÈΤ˜ ‰ÔÎÈ̷ۛ˜ ªÂȈ̤ÓË Û˘ÓıÂÙÈ΋ ÈηÓfiÙËÙ· ATP Û ÈÓÔ‚Ï¿ÛÙ˜ (>3 ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ Ù˘ ̤Û˘ ÙÈÌ‹˜) ªÔÚȷο Â˘Ú‹Ì·Ù· ∆·˘ÙÔÔ›ËÛË ÌÂÙ¿ÏÏ·Í˘ Û ˘ÚËÓÈÎfi ‹ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA ·‰È·ÌÊÈÛ‚‹ÙËÙ˘ ·ıÔÁÂÓÂÙÈÎfiÙËÙ·˜
∫ÏÈÓÈ΋ ÂΉ‹ÏˆÛË ∫ÏÈÓÈ΋ Û˘Ìو̷ÙÔÏÔÁ›· Û˘Ì‚·Ù‹ Ì ÌÈÙÔ¯ÔÓ‰Úȷ΋ ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ·* πÛÙÔ·ıÔÏÔÁ›· ñ 1-2% ÂÚ˘ıÚÒÓ Ú·Îˆ‰ÒÓ ÈÓÒÓ (ËÏÈΛ· 30-50 ÂÙÒÓ) ñ √ÔÈ·‰‹ÔÙ ·ÚÔ˘Û›· ÂÚ˘ıÚÒÓ Ú·Îˆ‰ÒÓ ÈÓÒÓ (<30 ÂÙÒÓ) ñ >2% ˘ÔÛ·ÚÎÂÈÏËÌ·ÙÈΤ˜ ÌÈÙÔ¯ÔÓ‰ÚȷΤ˜ Û˘ÛÛˆÚ‡ÛÂȘ (<16 ÂÙÒÓ) ñ ∂ÎÙÂٷ̤Ó˜ ÌÈÙÔ¯ÔÓ‰ÚȷΤ˜ ·ÏÏÔÈÒÛÂȘ ÛÙÔ ËÏÂÎÙÚÔÓÈÎfi ÌÈÎÚÔÛÎfiÈÔ ∂Ó˙˘ÌÔÏÔÁ›· ñ 20-30% ‰Ú·ÛÙÈÎfiÙËÙ· ÂÓ˙˘ÌÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Û ÈÛÙfi ñ 30-40% ‰Ú·ÛÙÈÎfiÙËÙ· ÂÓ˙˘ÌÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Û ΢ÙÙ·ÚÈ΋ ÁÚ·ÌÌ‹ ñ 30-40% ‰Ú·ÛÙÈÎfiÙËÙ· ÂÓ˙˘ÌÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜ Û ‰‡Ô ÈÛÙÔ‡˜ §ÂÈÙÔ˘ÚÁÈΤ˜ ‰ÔÎÈ̷ۛ˜ ñ ªÂȈ̤ÓË Û˘ÓıÂÙÈ΋ ÈηÓfiÙËÙ· ATP Û ÈÓÔ‚Ï¿ÛÙ˜ (2-3 ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ ·fi ÙË Ì¤ÛË) ªÔÚȷο Â˘Ú‹Ì·Ù· ∆·˘ÙÔÔ›ËÛË ÌÂÙ¿ÏÏ·Í˘ Û ˘ÚËÓÈÎfi ‹ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA Èı·Ó‹˜ ·ıÔÁÂÓÂÙÈÎfiÙËÙ·˜ ªÂÙ·‚ÔÏÈΤ˜ ‰È·Ù·Ú·¯¤˜ ŒÓ·˜ ‹ ÂÚÈÛÛfiÙÂÚÔÈ ÌÂÙ·‚ÔÏÈÎÔ› ‰Â›ÎÙ˜ ‰È·Ù·Ú·¯‹˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ï˘Û›‰·˜
* ∫ÏÈÓÈ΋ ÚÔ‚ÔÏ‹ Ù˘È΋ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ (Leigh, Alper, Pearson’s, Kearns-Sayre, MELAS, MERRF, NARP, MNGIE, LHON) ** ∂Ó˙˘ÌÈ΋ ‰Ú·ÛÙÈÎfiÙËÙ· ·ÓÙÈÚÔۈ‡ÂÈ ÔÛÔÛÙÈ·›· ·Ó·ÏÔÁ›· Û ۯ¤ÛË Ì ÙË Ê˘ÛÈÔÏÔÁÈ΋ ̤ÛË ÙÈÌ‹
Ó· ‰ÒÛÂÈ ÏËÚÔÊÔڛ˜ ÔÈ Ôԛ˜ Ó· ηÙ¢ı‡ÓÔ˘Ó ÙË ‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË. √ Ì·ÁÓËÙÈÎfi˜ ·ÂÈÎÔÓÈÛÙÈÎfi˜ Û˘ÓÙÔÓÈÛÌfi˜ ÚÔÙÈÌ¿Ù·È, ÂÓÒ Ë ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘ ÌÔÚ› Ó· ·ÂÈÎÔÓ›ÛÂÈ Î·Ï‡ÙÂÚ· ÙȘ Èı·Ó¤˜ ·Û‚ÂÛÙÔÔÈ‹ÛÂȘ ÛÙ· ‚·ÛÈο Á¿ÁÁÏÈ· (67). π‰È·›ÙÂÚ· ÛÙȘ ÂÚÈÙÒÛÂȘ Û˘Ó‰ÚfiÌÔ˘ Leigh, Û˘ÌÌÂÙÚÈÎfi ·ıÔÏÔÁÈÎfi Û‹Ì· ·Ó¢ڛÛÎÂÙ·È ÛÙ· ‚·ÛÈο Á¿ÁÁÏÈ· (∂ÈÎfiÓ· 5) ·ÏÏ¿ Î·È ¿ÏÏÔ˘˜ ÂÁÎÂÊ·ÏÈÎÔ‡˜ ˘Ú‹Ó˜ (˘Ôı·Ï¿ÌÈÔ˘˜, ̤ϷÈÓ· Ô˘Û›·, Ê·È¿ Ô˘Û›· ÙÔ˘ ˘‰Ú·ÁˆÁÔ‡ ÙÔ˘ Sylvious Î·È ˘Ú‹Ó˜ ÙÔ˘ ÛÙÂϤ¯Ô˘˜). ÷ڷÎÙËÚÈÛÙÈ΋ ÂÈÎfiÓ· Ï¢ÎÔ‰˘ÛÙÚÔÊ›·˜ ¤¯Ô˘Ì ÛÙÔ Û‡Ó‰ÚÔÌÔ Kearns-Sayre (∂ÈÎfiÓ· 8). ¶ÚÔÛ‚ÔÏ‹ Ù˘ Ï¢΋˜ Ô˘Û›·˜ ¤¯Ô˘Ì ›Û˘ ÛÙÔ Û‡Ó‰ÚÔÌÔ MNGIE Î·È Û‡Ó‰ÚÔÌÔ Leigh (41,68). ∂ÁÎÂÊ·ÏÈ΋ Î·È ·ÚÂÁÎÂÊ·ÏȉÈ΋ ÊÏÔÈ҉˘ ·ÙÚÔÊ›· Â›Ó·È Û˘¯Ófi ‡ÚËÌ·, ηٿ ηÓfiÓ· ‚Ú›ÛÎÂÙ·È ÛÙÔ Û‡Ó‰ÚÔÌÔ Alper, ÂÓÒ Û·Ó›ˆ˜ ··ÓÙ¿Ù·È ÛÙÔ Û‡Ó‰ÚÔÌÔ Leigh (31,39,41,69). Paediatriki 2007;70:1-17
* ∆· ÎÚÈÙ‹ÚÈ· ÁÈ· ÂÓ‹ÏÈΘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û˘ÌÌÂÙÔ¯‹ ÙÔ Ó¢ÚÈÎÔ‡ ‹ Ì˘˚ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ™Ù· ·È‰È¿ Û˘ÌÙÒÌ·Ù· fiˆ˜ ÓÂÔÁÓÈÎfi˜ ı¿Ó·ÙÔ˜, ÓÂÔÁÓÈ΋ ˘ÔÙÔÓ›·, ÛÔ‚·Ú‹ ηı˘ÛÙ¤ÚËÛË ·Ó¿Ù˘Í˘, ÓÂÔÁÓÈ΋ ˘ÂÚÙÔÓ›· ıˆÚÔ‡ÓÙ·È ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÌÈÙÔ¯ÔÓ‰ÚÈÔ¿ıÂÈ·˜
∂ÈÎfiÓ· 8. ™‡Ó‰ÚÔÌÔ Kearns-Sayre: ∂ÁοÚÛÈ· ÙÔÌ‹ ∆2 ·ÎÔÏÔ˘ı›· (11 ÂÙÒÓ). ∂ÎÙÂٷ̤ÓË Û˘ÌÌÂÙÔ¯‹ Ù˘ ˘ÔÊÏÔÈÒ‰Ô˘˜ Ï¢΋˜ Ô˘Û›·˜ (ÎfiÎÎÈÓÔ ‚¤ÏÔ˜). ¶·ıÔÏÔÁÈÎfi ˘„ËÏfi ∆2 Û‹Ì· ·Ú·ÙËÚÂ›Ù·È ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ Ê·ÎÔÂȉԇ˜ ˘Ú‹Ó· (6).
11
ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜
¶›Ó·Î·˜ 6. ¶ÚÔÙÂÈÓfiÌÂÓÔ ÌÂÙ·‚ÔÏÈÎfi ÚÔÊ›Ï Â› ˘Ô„›· ÂÓÂÚÁÂȷ΋˜ ‰È·Ù·Ú·¯‹˜ ¢ÂÈÁÌ·ÙÔÏË„›· ÓËÛÙ›·˜ (8 ÒÚ˜ Û ·È‰È¿, 4-5 ÒÚ˜ Û ‚Ú¤ÊË)
¢ÂÈÁÌ·ÙÔÏË„›· ÌÂÙ¿ Ê·ÁËÙfi (1-2 ÒÚ˜ ÌÂÙ¿ ·fi ηÏfi Á‡̷)
√Úfi˜ °Ï˘Îfi˙Ë, ÓÂÊÚÈ΋/Ë·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·, Á·Ï·ÎÙÈÎfi, ˘ÚÔÛÙ·Ê˘ÏÈÎfi, ÎÂÙÔÓÔÛÒÌ·Ù·, ·ÌÈÓÔͤ·, Ô˘ÚÈÎfi Ô͇, ÔÏÈ΋ ηÚÓÈÙ›ÓË, ·ÎÂÙ˘ÏÔηÚÓÈÙ›Ó˜, ÂχıÂÚ· ÏÈ·Ú¿ Ôͤ·, ·Ì̈ӛ·, CPK, ‰Â›ÁÌ· ÚÔ˜ ·Ôı‹Î¢ÛË
√Úfi˜ °Ï˘Îfi˙Ë, Á·Ï·ÎÙÈÎfi, ˘ÚÔÛÙ·Ê˘ÏÈÎfi, ÎÂÙÔÓÔÛÒÌ·Ù·, ·ÌÈÓÔͤ·
√‡Ú· °ÂÓÈ΋ Ô‡ÚˆÓ, ÎÂÙÔÓÔÛÒÌ·Ù·, ÔÚÁ·ÓÈο Ôͤ· Î·È ÔÚÔÙÈÎfi, ·ÌÈÓÔͤ· Ô‡ÚˆÓ, ‰Â›ÁÌ· ÚÔ˜ ·Ôı‹Î¢ÛË
√‡Ú· °ÂÓÈ΋ Ô‡ÚˆÓ, ÎÂÙÔÓÔÛÒÌ·Ù·, ÔÚÁ·ÓÈο Ôͤ· Î·È ÔÚÔÙÈÎfi, ·ÌÈÓÔͤ· Ô‡ÚˆÓ
∂¡À °Ï˘Îfi˙Ë, ÚˆÙ½ÓË, Á·Ï·ÎÙÈÎfi, ˘ÚÔÛÙ·Ê˘ÏÈÎfi, ·ÌÈÓÔͤ·, ‰Â›ÁÌ· ÚÔ˜ ·Ôı‹Î¢ÛË ¶ÂÚÈÔÚÈÛÌÔ› ñ ∂Ó˙˘Ì·ÙÈο Î·È ÁÂÓÂÙÈο ·Ô‰Â‰ÂÈÁ̤Ó˜ ÌÈÙÔ¯ÔÓ‰ÚÈÔ¿ıÂȘ ÌÔÚ› Ó· ¤¯Ô˘Ó Ê˘ÛÈÔÏÔÁÈÎfi Á·Ï·ÎÙÈÎfi Ô͇ Î·È ¿ÏÏÔ˘˜ ‚ÈÔ¯ËÌÈÎÔ‡˜ ‰Â›ÎÙ˜ ÛÙÔÓ ÔÚfi. ñ ™Â ÂÚ›Ô‰Ô ‡ÊÂÛ˘ Î·È ·Ô˘Û›·˜ ÌÂÙ·‚ÔÏÈÎÔ‡ stress, Ô ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÌÔÚ› Ó· Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi˜. ñ ŒÓÙÔÓÔ ÎÏ¿Ì· (ȉȷ›ÙÂÚ· ÛÙ· ·È‰È¿), Ë ÂÊ·ÚÌÔÁ‹ ÂÚ›ÛÊÈ͢ (tourniquet) ÁÈ· ÙË ÊÏ‚ÔΤÓÙËÛË ÌÔÚ› Ê˘ÛÈÔÏÔÁÈο Ó· ÂËÚ¿ÛÔ˘Ó ÙË Û˘ÁΤÓÙÚˆÛË ÙÔ˘ Á·Ï·ÎÙÈÎÔ‡ ÛÙÔÓ ÔÚfi. ∏ ·Ï·Ó›ÓË ÔÚÔ‡ Î·È ÙÔ Á·Ï·ÎÙÈÎfi Ô͇ ÛÙÔ ∂¡À ÂËÚ¿˙ÔÓÙ·È Û ÌÈÎÚfiÙÂÚÔ ‚·ıÌfi. ñ ∂ÁÁ‡˜ ÛˆÏËÓ·Úȷ΋ ‚Ï¿‚Ë ÂÏ·ÙÙÒÓÂÈ ÙË Û˘ÁΤÓÙÚˆÛË Á·Ï·ÎÙÈÎÔ‡ ÛÙÔÓ ÔÚfi ·ÏÏ¿ ÙËÓ ·˘Í¿ÓÂÈ ÛÙ· Ô‡Ú·. ñ ∏ ·ÚÔ˘Û›· ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË ÂÌÔ‰›˙ÂÈ ÙËÓ Â›ÛÔ‰Ô ÙÔ˘ ˘ÚÔÛÙ·Ê˘ÏÈÎÔ‡ ÛÙÔÓ Î‡ÎÏÔ ÙÔ˘ ÎÈÙÚÈÎÔ‡ ÔͤԘ Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ Á·Ï·ÎÙÈÎÔ‡. ñ ∏ ̤ÙÚËÛË ÙÔ˘ Á·Ï·ÎÙÈÎÔ‡ ÛÙÔ ∂¡À Â›Ó·È ÈÔ Â˘·›ÛıËÙË Î·È ÂȉÈ΋ ̤ıÔ‰Ô˜ Î·È Û˘ÓÈÛٿٷÈ.
∏ 1HMRS ÌÔÚ› Ó· ‰ÒÛÂÈ ¯Ú‹ÛÈ̘ in vivo ÌÂÙ·‚ÔÏÈΤ˜ ÏËÚÔÊÔڛ˜ Î·È Ë ·ÚÔ˘Û›· ·È¯Ì‹˜ Á·Ï·ÎÙÈÎÔ‡ ÛÙËÓ 1HMRS ÌÔÚ› Ó· ÂÚÈÏ·Ì‚¿ÓÂÙ·È ·˘ÙfiÓÔÌ· ˆ˜ ¤Ï·ÛÛÔÓ ÎÚÈÙ‹ÚÈÔ ÛÙÔ Û¯‹Ì· ÎÚÈÙËÚ›ˆÓ (MAC) (70). ÷ÌËÏ‹ ÙÈÌ‹ ¡∞∞ Â›Ó·È Û˘¯Ófi ‡ÚËÌ· ·ÏÏ¿ ‰ÂÓ ·ÔÙÂÏ› ÂȉÈÎfi ÌÂÙ·‚ÔÏÈÎfi ‰Â›ÎÙË (41). °ÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ∏ ÁÂÓÂÙÈ΋ ÚÔۤϷÛË ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ Â›Ó·È ‰··ÓËÚ‹ ‰È·‰Èηۛ· Î·È ··ÈÙ› ÂÍÂȉÈÎÂ˘Ì¤ÓÔ ÂÚÁ·ÛÙ‹ÚÈÔ. ŸÙ·Ó ¤¯Ô˘ÌÂ Ù˘ÈÎfi ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi Ê·ÈÓfiÙ˘Ô, ÙfiÙÂ Ô ¤ÏÂÁ¯Ô˜ ÙˆÓ ÎÔÈÓÒÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÚÈÓ ÙËÓ ÂÊ·ÚÌÔÁ‹ Ì˘˚΋˜ ‚ÈÔ„›·˜. ∞ÚÓËÙÈÎfi ·ÔÙ¤ÏÂÛÌ· ÌÂÈÒÓÂÈ ÙËÓ Èı·ÓfiÙËÙ· ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ ÓÔÛ‹Ì·ÙÔ˜ ·ÏÏ¿ ‰ÂÓ ÙÔ ·ÔÎÏ›ÂÈ, ÁÈ·Ù› ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA ‰ÂÓ ÂÎÊÚ¿˙ÔÓÙ·È Û fiÏÔ˘˜ ÙÔ˘˜ ÈÛÙÔ‡˜. ∂ÏÏ›ÌÌ·Ù· ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA ÂÎÊÚ¿˙ÔÓÙ·È Î·Ï‡ÙÂÚ· ÛÙÔÓ Ì˘˚Îfi ÈÛÙfi ·Ú¿ ÛÙ· Ï¢ÎÔ·ÙÙ·Ú· (ÂÍ·›ÚÂÛË ·ÔÙÂÏ› ÙÔ Û‡Ó‰ÚÔÌÔ Pearson). ŸÙ·Ó ÎÏÈÓÈο, ‚ÈÔ¯ËÌÈο Î·È ÈÛÙÔ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· Â›Ó·È Û˘Ì‚·Ù¿ Ì ÌÈÙÔ¯ÔÓ‰ÚÈÔ¿ıÂÈ· Î·È ÔÈ ÎÔÈÓ¤˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÌÈÙÔ¯fiÓ‰ÚÈ· DNA ·Ô˘ÛÈ¿˙Ô˘Ó, ÙfiÙÂ Ô ÂÚ·ÈÙ¤Úˆ ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ú¤ÂÈ Ó· ÂÍ·ÙÔÌÈ·ÂÙ·È. ∏ ·ÔΈ‰ÈÎÔÔ›ËÛË ÙˆÓ ·ÏÏËÏÔ˘¯ÈÒÓ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA (sequencing) Û˘ÓÈÛÙ¿Ù·È Ì ÛÎÔfi ÙËÓ ·Ó‡ÚÂÛË ÌË ÎÔÈÓÒÓ ‹ ·ÎfiÌË Î·È Ó¤ˆÓ ÌÂ-
Ù·ÏϿ͈Ó. ∏ ·Ó‡ÚÂÛË ÌÈ·˜ Ó¤·˜ ÌÂÙ¿ÏÏ·Í˘ ‰ÂÓ ÛËÌ·›ÓÂÈ fiÙÈ ·˘Ù‹ Â›Ó·È ··Ú·›ÙËÙ· ·ıÔÏÔÁÈ΋ Î·È Ë ÂÚÌËÓ›· ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È ·fi ÂÍÂȉÈÎÂ˘Ì¤Ó· ΤÓÙÚ·. ™Ù· ·È‰È¿ ÈÛÙ‡ÂÙ·È fiÙÈ Ë ÏÂÈÔ„ËÊ›· ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ÚÔηÏÂ›Ù·È ·fi ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ˘ÚËÓÈÎfi DNA. ∏ ·fiÊ·ÛË ÁÈ· ÙÔ ÔÈ· ÁÂÓÂÙÈ΋ ηÙ‡ı˘ÓÛË ı· ·ÎÔÏÔ˘ı‹ÛÔ˘ÌÂ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ÚÔÛÂÎÙÈ΋˜ ·Ó¿Ï˘Û˘ ÙÔ˘ ÎÏÈÓÈÎÔ‡ Ê·ÈÓÔÙ‡Ô˘, ÈÛÙÔ·ıÔÏÔÁ›·˜, ÂÓ˙˘ÌÈ΋˜ ·Ó¿ÚÎÂÈ·˜ Î·È ÙÔ˘ Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘. °È· ·Ú¿‰ÂÈÁÌ·, Û ·ÛıÂÓ‹ Ì ۇӉÚÔÌÔ Leigh Î·È ·ÚÓËÙÈ΋ ·ÓÙ›‰Ú·ÛË COX ÛÙË Ì˘˚΋ ‚ÈÔ„›·, ··Ú·›ÙËÙË Â›Ó·È Ë ·Ó›¯Ó¢ÛË ÌÂÙ·ÏÏ¿ÍÂˆÓ ÛÙÔ ÁÔÓ›‰ÈÔ SURF1. ¶·ÚÔ˘Û›· ΢ÛÙÈ΋˜ Ï¢ÎÔÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ Û ·ÛıÂÓ‹ Ì ·Ó¿ÚÎÂÈ· ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ π Ì¿˜ ηÙ¢ı‡ÓÂÈ ÚÔ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ NDUFV1. ∞ÛıÂÓ‹˜ Ì ηډÈÔÌ˘Ô¿ıÂÈ· Î·È ·ÚÓËÙÈ΋ ·ÓÙ›‰Ú·ÛË COX Ì¿˜ ηÙ¢ı‡ÓÂÈ ÛÙÔ ÁÔÓ›‰ÈÔ SCO2 Î·È ·ÛıÂÓ‹˜ Ì ÂÁÎÂÊ·ÏÔ¿ıÂÈ·, ÓÂÊÚÈ΋ ÛˆÏËÓ·Úȷ΋ ‚Ï¿‚Ë Î·È ·ÚÓËÙÈ΋ ·ÓÙ›‰Ú·ÛË COX Ì¿˜ ηÙ¢ı‡ÓÂÈ ÛÙÔ ÁÔÓ›‰ÈÔ COX10. √ ηٿÏÔÁÔ˜ ÙˆÓ ˘Â‡ı˘ÓˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ÁÈ· ÚˆÙÔ·ı‹ ÌÈÙÔ¯ÔÓ‰Úȷ΋ ÓfiÛÔ Û˘Ó¯Ҙ ·˘Í¿ÓÂÙ·È Î·È ÙÔ ¢È·‰›ÎÙ˘Ô ·ÔÙÂÏ› ÙËÓ Î·Ï‡ÙÂÚË ËÁ‹ ÏËÚÔÊfiÚËÛ˘ (mitomap.org). √È ¶›Ó·Î˜ 7 Î·È 8 ÂÚÈÁÚ¿ÊÔ˘Ó ÁÓˆÛÙ¤˜ ·ıÔÏÔÁÈΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ˘ÚËÓÈÎfi ‹ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA ·ÓÙÈÛÙÔ›¯ˆ˜ (6,8,14,33,71). ¶·È‰È·ÙÚÈ΋ 2007;70:1-17
12
∞. ¡ÙÈÓfiÔ˘ÏÔ˜, ∞. ¶··‰ËÌËÙÚ›Ô˘
¶›Ó·Î·˜ 7. ªÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ˘ÚËÓÈÎÔ‡ DNA ˘Â‡ı˘Ó˜ ÁÈ· ÚˆÙÔ·ı‹ ÌÈÙÔ¯ÔÓ‰Úȷ΋ ÓfiÛÔ °ÔÓ›‰ÈÔ
ªÈÙÔ¯ÔÓ‰Úȷ΋ ‚Ï¿‚Ë
∫ÏËÚÔÓÔÌÈÎfiÙËÙ· / ∫ÏÈÓÈÎfi˜ Ê·ÈÓfiÙ˘Ô˜
ªÂÙ·ÏÏ¿ÍÂȘ Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ‰ÔÌÈΤ˜ ÌÔÓ¿‰Â˜ Û˘ÌÏÂÁÌ¿ÙˆÓ NDUFS1 ™‡ÌÏÂÁÌ· π À™ / ÂÁÎÂÊ·ÏÔ¿ıÂÈ· NDUFS2 » NDUFS4 » À™ / ¶ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ÓfiÛÔ˜ NDUFS7 » À™ / Leigh NDUFS8 » À™ / Leigh NDUFV1 » À™ / §Â˘ÎÔÂÁÎÂÊ·ÏÔ¿ıÂÈ· / Ì˘ÔÎÏÔÓ›· SDHA ™‡ÌÏÂÁÌ· ππ À™ / Leigh SDHB » ∂™ ‹ ™ÔÚ·‰È΋ / º·ÈÔ¯ÚˆÌÔ·Ùو̷, ÙÚ·¯ËÏÈÎfi ·Ú·Á·ÁÁÏ›ˆÌ· SDHD Î·È SDHC » ∫ÏËÚÔÓÔÌÈÎfi ·Ú·Á·ÁÁÏ›ˆÌ· UQCRB ™‡ÌÏÂÁÌ· πππ ÀÔηÏÈ·ÈÌ›·, Á·Ï·ÎÙÈ΋ ÔͤˆÛË ªÂÙ·ÏÏ¿ÍÂȘ Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ÚˆÙ½Ó˜ Û˘Ó·ÚÌÔÏfiÁËÛ˘ Û˘ÌÏÂÁÌ¿ÙˆÓ SURF1 ™‡ÌÏÂÁÌ· πV À™ / Leigh SCO1 » À™ / ∏·ÙÔ¿ıÂÈ· SCO2 » À™ / µÚÂÊÈ΋ ηډÈÔÌ˘Ô¿ıÂÈ· COX10 » À™ / §Â˘ÎÔÂÁÎÂÊ·ÏÔ¿ıÂÈ·, ÓÂÊÚÈ΋ ÛˆÏËÓ·Úȷ΋ ‚Ï¿‚Ë COX15 » À™ / ÀÂÚÙÚÔÊÈ΋ ηډÈÔÌ˘Ô¿ıÂÈ· LRPPRC » À™ / Leigh BCS1L ™‡ÌÏÂÁÌ· πII À™ / ∂ÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ·, Ë·ÙÈ΋ ·Ó¿ÚÎÂÈ·, ÓÂÊÚÈ΋ ÛˆÏËÓ·Úȷ΋ ‚Ï¿‚Ë ATP12 ™‡ÌÏÂÁÌ· V À™ / ∂ÁÎÂÊ·ÏÔ¿ıÂÈ· ªÂÙ·ÏÏ¿ÍÂȘ Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ÚˆÙ½Ó˜ ··Ú·›ÙËÙ˜ ÁÈ· ÙË ‰È·Ù‹ÚËÛË ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA Twinkle ¶ÔÏÏ·Ï¿ ÂÏÏ›ÌÌ·Ù· À™ ‹ ∂™ / ¶ÚÔԉ¢ÙÈ΋ ÔÊı·ÏÌÔÏËÁ›· (PEO) ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA POLG1 À™ / ™‡Ó‰ÚÔÌÔ Alpers Thymidine ¶ÔÏÏ·Ï¿ ÂÏÏ›ÌÌ·Ù· ‹ À™ / ªÈÙÔ¯ÔÓ‰Úȷ΋ Ó¢ÚÔ-Á·ÛÙÚÂÓÙÂÚÈ΋ phosphorylase ÔÛÔÙÈ΋ Ì›ˆÛË ÙÔ˘ ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ· (MNGIE) ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA TK2 ¶ÔÛÔÙÈ΋ Ì›ˆÛË ÙÔ˘ µÚÂÊÈ΋ Û˘ÁÁÂÓ‹˜ Ì˘Ô¿ıÂÈ· Ì ‹ ¯ˆÚ›˜ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA Û‡Ó‰ÚÔÌÔ De Toni-Fanconi DGUOK ¶ÔÛÔÙÈ΋ Ì›ˆÛË µÚÂÊÈ΋ Ë·ÙÔ¿ıÂÈ· Î·È ÂÁÎÂÊ·ÏÔÌ˘Ô¿ıÂÈ· ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA ªÂÙ·ÏÏ¿ÍÂȘ Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ÚˆÙ½Ó˜ ÌÂÙ·ÊÔÚ›˜ DDP1/TIMM8A ºÀ / ∫ÒʈÛË ‰˘ÛÙÔÓ›· (Û‡Ó‰ÚÔÌÔ Mohr-Tranebjaerg) ANT1 ¶ÔÏÏ·Ï¿ ÂÏÏ›ÌÌ·Ù· À™ ‹ ∂™ / ¶ÚÔԉ¢ÙÈ΋ ÔÊı·ÏÌÔÏËÁ›· (PEO) ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA ÕÏϘ ÌÂÙ·ÏÏ¿ÍÂȘ Tafazzin
ºÀ / ™‡Ó‰ÚÔÌÔ Barth
À™: ÀÔÏÂÈfiÌÂÓÔ ™ˆÌ·ÙÈÎfi, ∂™: ∂ÈÎÚ·ÙÔ‡Ó ™ˆÌ·ÙÈÎfi, ºÀ: º˘ÏÔÛ‡Ó‰ÂÙÔ ÀÔÏÂÈfiÌÂÓÔ
£Âڷ›· Î·È ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË £Âڷ›· ¢˘ÛÙ˘¯Ò˜ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·ÈÙÈÔÏÔÁÈ΋ ıÂڷ›· ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ‰ÂÓ ˘¿Ú¯ÂÈ Î·È Ë ıÂڷ›· ÂÚÈÔÚ›˙ÂÙ·È ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Û˘Ìو̿وÓ, ˘ÔÛÙ‹ÚÈÍË ÙˆÓ ÔÚÁ¿ÓˆÓ ‹ Û˘ÛÙËÌ¿ÙˆÓ Ô˘ Û˘ÌÌÂÙ¤¯Ô˘Ó Î·È ·ÔÊ˘Á‹ ÂÈÏÔÎÒÓ ·fi ·Ó·Ëڛ˜ Ù˘ ÓfiÛÔ˘ (72,73). ¢È·ÈÙËÙÈο ̤ÙÚ·. ™˘ÓÈÛÙ¿Ù·È Ë ·ÔÊ˘Á‹ ·Ú·ÙÂٷ̤Ó˘ ÓËÛÙ›·˜ Î·È Ë Î·Ï‹ ÂÓ˘‰¿ÙˆÛË, ȉȷ›ÙÂÚ· fiÙ·Ó ÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi ÓfiÛËÌ· Û˘Óԉ‡ÂÙ·È ·fi ˘ÔÁÏ˘Î·ÈÌ›·. ∏ ÎÂÙÔÁÂÓ‹˜ ‰›·ÈÙ· Û˘ÓÈÛÙ¿Ù·È Û ·Ó¿ÚÎÂÈ· Ù˘ ˘ÚÔÛÙ·Ê˘ÏÏÈ΋˜ ·Ê˘‰ÚÔÁÔÓ¿Û˘ Paediatriki 2007;70:1-17
ÂÓÒ ÌÔÚ› Ó· Â›Ó·È ‚ÔËıËÙÈ΋ Û ·Ó¿ÚÎÂÈ· ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ I Ì ·ÓıÂÎÙÈ΋ ÂÈÏË„›·. ™ËÌ·ÓÙÈ΋ Â›Ó·È Ë ‰È·Ù‹ÚËÛË ‰È·ÈÙËÙÈÎÔ‡ ÈÛÔ˙˘Á›Ô˘ Ì ÚfiÛÏË„Ë fiÏˆÓ ÙˆÓ ··Ú·›ÙËÙˆÓ ‚ÈÙ·ÌÈÓÒÓ Î·È È¯ÓÔÛÙÔȯ›ˆÓ ÂÂȉ‹ ÔÈ ·ÛıÂÓ›˜ Ì ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi ÓfiÛËÌ· Â›Ó·È È‰È·›ÙÂÚ· ÂÈÚÚ‹˜ Û ·Ó¿ÚÎÂȘ. º˘ÛÈÎÔıÂڷ›·. ∏ Ê˘ÛÈ΋ ¿ÛÎËÛË Ú¤ÂÈ Ó· ÂÓı·ÚÚ‡ÓÂÙ·È Î·È Î·Ïfi Â›Ó·È Ó· Á›ÓÂÙ·È ÚÔÁÚ·ÌÌ·ÙÈṲ̂ӷ ˘fi ÙËÓ Â›‚ÏÂ„Ë ÙÔ˘ Ê˘ÛÈÎÔıÂÚ·Â˘Ù‹ Î·È ·ÊÔ‡ ¤¯ÂÈ ·ÔÎÏÂÈÛÙ› ˘ÔÎÏÈÓÈ΋ ηډÈÔ¿ıÂÈ·. ∏ Ê˘ÛÈÎÔıÂڷ›· ‚ÔËı¿ÂÈ ÛÙËÓ ÚfiÏË„Ë ÙˆÓ Û˘Áο̄ˆÓ, ÛÎÔÏ›ˆÛ˘ Î·È ÛÙËÓ ÚfiÏË„Ë Ù˘ ÔÛÙÂÔfiÚˆÛ˘. ∏ ˘ÂÚ‚ÔÏÈ΋ ¿ÛÎËÛË Î·Ïfi Â›Ó·È Ó·
13
ªÈÙÔ¯ÔÓ‰Úȷο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜
¶›Ó·Î·˜ 8. º·ÈÓÔÙ˘È΋ ¤ÎÊÚ·ÛË ·ıÔÁfiÓˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ÙÔ˘ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÔ‡ DNA (Ì ¤ÓÙÔÓ· ÁÚ¿ÌÌ·Ù· ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ ÈÔ Û˘¯Ó¤˜ ÌÂÙ·ÏÏ¿ÍÂȘ) ∫ÏÈÓÈÎfi˜ º·ÈÓfiÙ˘Ô˜ °ÔÓ›‰ÈÔ
ªÂÙ·ÏÏ¿ÍÂȘ
MELAS
ñ {3243, 3252, 3260, 5814}A>G, {1642, 583}G>A, {3271, 3291}T>C ñ ŒÏÏÂÈÌÌ· (deletion) ñ 13513G>A, 13514A>G ñ 14787del4 ñ 9957T>C ñ 3308T>C
MELAS/Ó¤ÎÚˆÛË Ú·‚‰ˆÙÔ‡ ÛÒÌ·ÙÔ˜ MELAS/MERRF MERRF PEO
tRNA
ND5 (Û‡ÌÏÂÁÌ· π) Cyt b (Û‡ÌÏÂÁÌ· IIπ) COX III (Û‡ÌÏÂÁÌ· πV) ND5 (Û‡ÌÏÂÁÌ· π) tRNA
PEO/ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ÓfiÛÔ˜ PEO/‰È·‚‹Ù˘, ·Ù·Í›· ª˘Ô¿ıÂÈ· Cyt b (Û‡ÌÏÂÁÌ· III)
ÀÂÚÙÚÔÊÈ΋ ηډÈÔÌ˘Ô¿ıÂÈ· ¡Â˘ÚÔ·ÈÛıËÙËÚȷ΋ ·ÒÏÂÈ· ·ÎÔ‹˜ NARP/MILS
LHON /
tRNA Cyt b (Û‡ÌÏÂÁÌ· IIπ) tRNA rRNA tRNA ATPase 6 (Û‡ÌÏÂÁÌ· V) ND6 (Û‡ÌÏÂÁÌ· π) COX III (Û‡ÌÏÂÁÌ· πV) ND1 (Û‡ÌÏÂÁÌ· π) ND4 (Û‡ÌÏÂÁÌ· π) ND6 (Û‡ÌÏÂÁÌ· π)
LHON / ¢˘ÛÙÔÓ›· ∫™™ / Pearson
tRNA
ñ {8356,7512}T>C ñ 8344A>G, 8363G>A ñ ŒÏÏÂÈÌÌ· (deletion) ñ {3243, 5692, 3251}A>G, {4309, 5703, 12315, 4298, 8342}G>A ñ 3256C>T ñ {3264, 12311}T>C ñ {3288, 3302, 3243, 12320}A>G, 5521G>A, 15990C>T {618, 3250, 4409}T>C ñ {14846, 15059, 15084, 15150, 15168, 15242, 15615, 15723, 15762}G>A, 15197T>C ñ 15498del24 ñ {3243, 3254, 3260, 4269, 4295, 4300, 8296}A>G, {3303, 4320} C>T, 9997T>C, 8363 G>A ñ 15243G>A ñ {3243, 7445}A>G, {7510, 7511, 7512}T>C, 12258G>A ñ 7472-insC ñ delT961Cn, 1095T>C, 1555A>G ñ {1624, 1644}C>T, 8344A>G, 8363G>A, 5537-insT ñ 8993A>G, {8851, 9171}T>C ñ 14459 G>A ñ 9537 insC ñ {3460, 5244, 7444, 9438, 9804, 13708, 15257, 15812}G>A, 4917A>G , {3394, 4160, 4216, 9101}T>C ñ 11778G>A (Ï¢ÎÔÂÁÎÂÊ·ÏÔ¿ıÂÈ·), 10663T>C ñ 14482G>A, 14484T>C, 14495A>G ñ 11696, 14459 (‚Ï¿‚Ë ÛÙ· ‚·ÛÈο Á¿ÁÁÏÈ·), 14596 ñ ∂ÏÏ›ÌÌ·Ù· ñ ∞Ó·‰ÈÏ·ÛÈ·ÛÌÔ›
·ÔʇÁÂÙ·È ÁÈ·Ù› ·ÊÂÓfi˜ ˘¿Ú¯ÂÈ Î›Ó‰˘ÓÔ˜ Ú·‚‰ÔÌ˘fiÏ˘Û˘ Î·È Î›Ó‰˘ÓÔ˜ ηډȷ΋˜ ‚Ï¿‚˘, ·ÊÂÙ¤ÚÔ˘ ÌÔÚ› Ó· ·˘Í‹ÛÂÈ ÙÔ ÔÛÔÛÙfi ÙˆÓ ·ıÔÏÔÁÈÎÒÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ. º·Ú̷΢ÙÈ΋ Î·È ·Ó·ÏËڈ̷ÙÈ΋ ıÂڷ›·. ∏ ¯ÔÚ‹ÁËÛË Û˘ÓÂÓ˙‡ÌÔ˘ Q10 (4-10 mg/kg/Ë̤ڷ) ‹ ¿ÏÏÔ˘ ·Ó¿ÏÔÁÔ˘ (idebenone 90-200 mg/Ë̤ڷ) Û˘ÓÈÛٿٷÈ, ·Ó Î·È ‰ÂÓ ˘¿Ú¯Ô˘Ó Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ Ô˘ Ó· ·Ô‰ÂÈÎÓ‡Ô˘Ó ÙËÓ Â˘ÂÚÁÂÙÈ΋ ÙÔ˘˜ ‰Ú¿ÛË. ∏ ¯ÔÚ‹ÁËÛË ‚ÈÙ·ÌÈÓÒÓ Â›Ó·È ··Ú·›ÙËÙË fiˆ˜ ‚ÈÙ·Ì›ÓË C (100-1000 mg/Ë̤ڷ), ıÈ·Ì›ÓË (50-200 mg/Ë̤ڷ), ÚÈ‚ÔÊÏ·‚›ÓË (50-200 mg/Ë̤ڷ), ÂÓÒ ¿ÏϘ ‚Èٷ̛Ә (D, E) ¯ÔÚËÁÔ‡ÓÙ·È ·Ó¿ ÂÚ›ÙˆÛË. ∏ ¯ÔÚ‹ÁËÛË L-ηÚÓÈÙ›Ó˘ (25-50 mg/kg/Ë̤ڷ) Â›Ó·È ·ÌÊÈÏÂÁfiÌÂÓË ·ÏÏ¿ ÁÂÓÈÎÒ˜ Û˘ÓÈÛÙ¿Ù·È Û ÂÚÈÙÒÛÂȘ Ô˘ ¤¯Ô˘Ì ·Ô‰Â‰ÂÈÁ-
̤ÓË ·Ó¿ÚÎÂÈ·. ™Â ÂȉÈΤ˜ ÂÚÈÙÒÛÂȘ fiˆ˜ ÛÔ‚·ÚÔ‡˜ Û˘ÁÁÂÓÔ‡˜ Á·Ï·ÎÙÈ΋˜ ÔͤˆÛ˘ ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› ÙÔ ‰È¯ÏˆÚÔ·ÎÂÙÔÙÈÎfi Ô͇ (DCA) Î·È Û ÂÚÈÙÒÛÂȘ ÂÁÎÂÊ·ÏÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ Û ۇӉÚÔÌÔ MELAS Ë L-·ÚÁÈÓ›ÓË (74,75). ∆Ô ‚·ÏÚÔ˚Îfi Ô͇ ηÏfi Â›Ó·È Ó· ·ÔʇÁÂÙ·È ÁÈ·Ù› ȉȷ›ÙÂÚ· ÛÙÔ Û‡Ó‰ÚÔÌÔ Alper ÌÔÚ› Ó· ¿ÁÂÈ ÎÂÚ·˘ÓÔ‚fiÏÔ Ë·ÙÈ΋ Ó¤ÎÚˆÛË. ∞ÓÙÈÂÈÏËÙÈο fiˆ˜ ÙÔÈÚ·Ì¿ÙË Î·È ˙ÔÓÈÛ·Ì›‰Ë ÌÔÚ› Ó· ÂȉÂÈÓÒÛÔ˘Ó ÙË ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË Î·È Ú¤ÂÈ Ó· ¯ÔÚËÁÔ‡ÓÙ·È Ì ÚÔÛÔ¯‹ Î·È ÛÂ Û˘Ó‰˘·ÛÌfi Ì ‰ÈÙÙ·ÓıÚ·ÎÈο. ÕÏÏ· Ê¿Ú̷η ÙÔÍÈο ÁÈ· Ù· ÌÈÙÔ¯fiÓ‰ÚÈ· Â›Ó·È ·ÓÙÈ‚ÈÔÙÈο fiˆ˜ ÔÈ ·ÌÈÓÔÁÏ˘ÎÔÛ›‰Â˜ (ΛӉ˘ÓÔ˜ ·ÒÏÂÈ· ·ÎÔ‹˜) Î·È Ë ¯ÏˆÚ·ÌÊÂÓÈÎfiÏË, ÈÔÛÙ·ÙÈο (AZT) Î·È ·ÓÙÈ·Ú·ÛÈÙÈο fiˆ˜ Ë ÌÂÙÚÔÓȉ·˙fiÏË. ¶·È‰È·ÙÚÈ΋ 2007;70:1-17
14
∞. ¡ÙÈÓfiÔ˘ÏÔ˜, ∞. ¶··‰ËÌËÙÚ›Ô˘
ÀÔÛÙËÚÈÎÙÈ΋ ıÂڷ›·. i. ∞ÓÙÈÌÂÙÒÈÛË ÙˆÓ Û·ÛÌÒÓ Ì ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹. ¢ÂÓ ˘¿Ú¯ÂÈ Î¿ÔÈÔ ÂȉÈÎfi ·ÓÙÈÂÈÏËÙÈÎfi Ê¿ÚÌ·ÎÔ. ∞fi Ù· ÓÂfiÙÂÚ· ·ÓÙÈÂÈÏËÙÈο Ë Ï·ÌÔÙÚÈÁ›ÓË Î·È Ë Ï‚ÂÙÈÚ·ÎÂÙ¿ÌË ‰ÂÓ ÚÔηÏÔ‡Ó ÌÂÙ·‚ÔÏÈ΋ ‰È·Ù·Ú·¯‹ Î·È Â›Ó·È Î·Ï¿ ·ÓÂÎÙ¿. ii. ∞ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Î·È ¿ÏÏˆÓ ÂÓ‰ÔÎÚÈÓÔ·ıÂÈÒÓ. iii. ÀÔÛÙËÚÈÎÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Ì˘ÔηډÈÔ¿ıÂÈ·˜ (ACE ·Ó·ÛÙÔÏ›˜, ‰ÈÔ˘ÚËÙÈο Î.Ï.) Î·È ·ÚÚ˘ıÌÈÒÓ (Ê¿Ú̷η ‹ ‚ËÌ·ÙÔ‰fiÙ˘). iv. ŒÁηÈÚË ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ Û˘ÁÁÂÓÔ‡˜ ηٷÚÚ¿¯ÙË Î·È Ë ‚ÏÂÊ·ÚÔÏ·ÛÙÈ΋ Û˘ÓÈÛÙ¿Ù·È fiÙ·Ó ¤¯Ô˘Ì ÛÔ‚·Ú‹ ‚ÏÂÊ·ÚfiÙˆÛË. v. ∞ÓÙÈÌÂÙÒÈÛË Ù˘ ‚·ÚËÎÔ˝·˜ Ì ·ÎÔ˘ÛÙÈο ‹ ·Ó ¯ÚÂÈ·ÛÙ›, ÎÔ¯Ïȷο ÂÌÊ˘Ù‡̷ٷ. vi. ∞ÓÙÈÌÂÙÒÈÛË ÓÂÊÚÈ΋˜ ÓfiÛÔ˘ Î·È Ù˘ Û˘ÓÔ‰Ô‡ ·Ó·ÈÌ›·˜. ∞ÓÙÈÌÂÙÒÈÛË ÌÂÙ·‚ÔÏÈ΋˜ ÎÚ›Û˘. ∏ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÎÚ›ÛÂˆÓ Â›Ó·È Û˘Ìو̷ÙÈ΋ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ Î·Ï‹ ÂÓ˘‰¿ÙˆÛË, ‰ÈfiÚıˆÛË Ù˘ ˘ÔÁÏ˘Î·ÈÌ›·˜ Î·È ÛˆÛÙ‹ Ú‡ıÌÈÛË Ù˘ ÔÍÂÔ‚·ÛÈ΋˜ ÈÛÔÚÚÔ›·˜. ™ËÌ·ÓÙÈÎfi˜ Â›Ó·È Ô ¤ÏÂÁ¯Ô˜ Ù˘ Ë·ÙÈ΋˜ Î·È È‰È·›ÙÂÚ· Ù˘ ÓÂÊÚÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ fiÙ·Ó ¤¯Ô˘ÌÂ Û˘ÓÔ‰fi Ú·‚‰ÔÌ˘fiÏ˘ÛË. ∏ ¯ÔÚ‹ÁËÛË ‰ÈÙÙ·ÓıÚ·ÎÈÎÒÓ Û ÂÚÈÙÒÛÂȘ Á·Ï·ÎÙÈ΋˜ ÔͤˆÛ˘ ÂÈ‚¿ÏÏÂÙ·È. ∞Ó Ë Á·Ï·ÎÙÈ΋ ÔͤˆÛË Â›Ó·È ÛÔ‚·Ú‹, ÙfiÙ ÌÔÚԇ̠ӷ ¯ÔÚËÁ‹ÛÔ˘Ì ‰È¯ÏˆÚÔ·ÎÂÙÔÙÈÎfi Ô͇ DCA (25 mg/kg/day) (76). ∏ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Û·ÛÌÒÓ Ô˘ ÔÏϤ˜ ÊÔÚ¤˜ ÂÈϤÎÔ˘Ó ÌÈ· ÎÚ›ÛË Á›ÓÂÙ·È Ì ÂÓ‰ÔÊϤ‚È· ‹ ·fi ÙÔ ÔÚıfi ¯ÔÚ‹ÁËÛË ‚ÂÓ˙ԉȷ˙ÂÈÓÒÓ, ÂÓÒ ·fi ÚÔÛˆÈ΋ ÂÌÂÈÚ›·, Ë ¯ÔÚ‹ÁËÛË Ê·ÈÓÔ‚·Ú‚ÈÙ¿Ï˘ ÌÔÚ› Ó· Â›Ó·È ‚ÔËıËÙÈ΋. °ÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË ™ËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA. √ ΛӉ˘ÓÔ˜ Ù˘ ÌËÙ¤Ú·˜ Ì ÛËÌÂȷ΋ ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA Ó· ÌÂÙ·‚È‚¿ÛÂÈ ÙË ÓfiÛÔ ÛÙ· ·È‰È¿ ÂÍ·ÚÙ¿Ù·È ·fi ÙÔ Â›Â‰Ô Ù˘ ÂÙÂÚÔÏ·ÛÌ›·˜ Ù˘ ÌÂÙ¿ÏÏ·Í˘ ÛÙÔ˘˜ ÈÛÙÔ‡˜. √ ΛӉ˘ÓÔ˜ ·˘Í¿ÓÂÙ·È Ì ÙÔ ÔÛÔÛÙfi ÂÙÂÚÔÏ·ÛÌ›·˜ ÛÙÔ˘˜ ÈÛÙÔ‡˜ Î·È ‰ÂÓ ˘¿Ú¯ÂÈ ¤Ó· ÂÏ¿¯ÈÛÙÔ Â›Â‰Ô ÂÙÂÚÔÏ·ÛÌ›·˜ οو ÙÔ˘ ÔÔ›Ô˘ Ô Î›Ó‰˘ÓÔ˜ ÌÂÙ·‚›‚·Û˘ Ó· ··Ï›ÊÂÙ·È. ÕÚÚÂÓ˜ Ì ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA ÂÍ·ÈÚÂÙÈο Û¿ÓÈ· ÌÔÚ› Ó· ÌÂÙ·‚È‚¿ÛÔ˘Ó ÙË ÓfiÛÔ. ∞Ó·Û˘Ó‰˘·ÛÌÔ› ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA. √È ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ Ì ÌÔÓ‹Ú˜ ¤ÏÏÂÈÌÌ· ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA Â›Ó·È ÛÔÚ·‰ÈΤ˜ (4). √ ΛӉ˘ÓÔ˜ ÂÌÊ¿ÓÈÛ˘ Ù˘ ÓfiÛÔ˘ ÛÙÔ ·È‰› ‰ÂÓ ·˘Í¿ÓÂÙ·È Ì ÙËÓ ËÏÈΛ· Ù˘ ÌËÙ¤Ú·˜. ªËÙ¤Ú˜ Ì ÌÔÓ‹Ú˜ ¤ÏÏÂÈÌÌ· Î·È Û‡Ó‰ÚÔÌÔ Kearn-Sayre ‹ PEO ¤¯Ô˘Ó ΛӉ˘ÓÔ 4% Ó· ÌÂÙ·‚È‚¿ÛÔ˘Ó ÙË ÓfiÛÔ. Paediatriki 2007;70:1-17
ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ˘ÚËÓÈÎfi DNA. ∏ ÁÂÓÂÙÈ΋ ÙˆÓ ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ ·fi ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ˘ÚËÓÈÎfi DNA ·ÎÔÏÔ˘ı› ÙÔ˘˜ ÌÂÓ‰¤ÏÈÔ˘˜ ηÓfiÓ˜ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜ Î·È Û ·˘Ù¤˜ ÙȘ ÂÚÈÙÒÛÂȘ ÌfiÓÔÓ ·Ó Ù·˘ÙÔÔÈËı› Ë ÌÂÙ¿ÏÏ·ÍË Â›Ó·È ‰˘Ó·Ù‹ Ë ÚÔÁÂÓÂÙÈ΋ ‰È¿ÁÓˆÛË. ¶Ú¤ÂÈ Ó· ÙÔÓ›ÛÔ˘Ì fiÙÈ Ë ·Ó‡ÚÂÛË Ù˘ ˘Â‡ı˘Ó˘ ÌÂÙ¿ÏÏ·Í˘ ‰ÂÓ Â›Ó·È ¿ÓÙ· ÂÊÈÎÙ‹ Î·È ÔÏϤ˜ ÊÔÚ¤˜ ÌÔÚ› Ó· ÂÚ¿ÛÔ˘Ó Î·È ¯ÚfiÓÈ· ̤¯ÚÈ Ó· ÙË ‚ÚÔ‡ÌÂ. ™˘ÌÏÂÁÌ·ÙÈ΋ ·Ó¿ÚÎÂÈ· Ì ¿ÁÓˆÛÙË ÁÂÓÂÙÈ΋ ‚Ï¿‚Ë. ¶ÚÔÁÂÓÂÙÈ΋ ‰È¿ÁÓˆÛË Û ·Ô‰Â‰ÂÈÁ̤ÓË Û˘ÌÏÂÁÌ·ÙÈ΋ ·Ó¿ÚÎÂÈ· ·ÁÓÒÛÙÔ˘ ÁÂÓÂÙÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜ Â›Ó·È ‰˘Ó·Ù‹ Û ÂÍÂȉÈÎÂ˘Ì¤Ó· ΤÓÙÚ·, ·ÊÔ‡ ÚÒÙ· ·ÔÎÏÂÈÛÙÔ‡Ó ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÌÈÙÔ¯ÔÓ‰ÚÈ·Îfi DNA (71). ∏ ‰Ú·ÛÙÈÎfiÙËÙ· ÙˆÓ Û˘ÌÏÂÁÌ¿ÙˆÓ ÌÂÙÚ¿Ù·È Û ÙÚÔÊÔ‚Ï¿ÛÙË Î·È ·ÌÓȷο ·ÙÙ·Ú·. ∏ ̤ıÔ‰Ô˜ Â›Ó·È ÈÔ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÁÈ· ·Ó¿ÚÎÂÈ· Û˘ÌϤÁÌ·ÙÔ˜ π Î·È IV, ·ÏÏ¿ ÂÓ¤¯ÂÈ ÔÏϤ˜ Ù¯ÓÈΤ˜ ‰˘ÛÎÔϛ˜ Î·È Ú¤ÂÈ Ó· Á›ÓÂÙ·È Û ÂÍÂȉÈÎÂ˘Ì¤Ó· ÌÈÙÔ¯ÔÓ‰Úȷο ΤÓÙÚ· Ì ÂÌÂÈÚ›· ÛÙËÓ ÚÔÁÂÓÂÙÈ΋ ‰È¿ÁÓˆÛË.
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¶. ∫·Ú·ÁÈ¿ÓÓË, ¡. ¡ÈÎÔÏ·˝‰Ë˜ ¶ÂÚ›ÏË„Ë: O ˘„›Û˘¯ÓÔ˜ ·ÂÚÈÛÌfi˜ Ì ٷϷÓÙÒÛÂȘ (À∞∆) ·ÔÙÂÏ› ¤Ó·Ó ÂÓ·ÏÏ·ÎÙÈÎfi ÙÚfiÔ Ì˯·ÓÈÎÔ‡ ·ÂÚÈÛÌÔ‡, Ô ÔÔ›Ô˜ ¯ÚËÛÈÌÔÔÈ› ÌÈÎÚÔ‡˜ ·Ó·ÓÂfiÌÂÓÔ˘˜ fiÁÎÔ˘˜ ·¤Ú·, Û˘¯Ó¿ ÌÈÎÚfiÙÂÚÔ˘˜ ·fi ÙÔÓ ·Ó·ÙÔÌÈο ÓÂÎÚfi ¯ÒÚÔ Î·È ÂÍ·ÈÚÂÙÈο ÁÚ‹ÁÔÚÔ˘˜ ·Ó·Ó¢ÛÙÈÎÔ‡˜ Ú˘ıÌÔ‡˜, ¤Ú·Ó ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡, ÁÈ· ÙËÓ ÂÍ·ÛÊ¿ÏÈÛË Ù˘ Ô͢ÁfiÓˆÛ˘ Î·È ÙÔ˘ ·ÂÚÈÛÌÔ‡ ÙˆÓ ÓÂÔÁÓÒÓ. ∞ÚÎÂÙ¤˜ ÂÈÚ·Ì·ÙÈΤ˜ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ ÙÔ ÔÁÎfiÙÚ·˘Ì· Ì¿ÏÏÔÓ ·Ú¿ ÙÔ ‚·ÚfiÙÚ·˘Ì· Â›Ó·È Ë ·Ú¯È΋ ·ÈÙ›· Ù˘ ÔÍ›·˜ Ó¢ÌÔÓÈ΋˜ ‚Ï¿‚˘ Ô˘ ÚÔηÏÂ›Ù·È ·fi ÙÔ Û˘Ì‚·ÙÈÎfi ·ÂÚÈÛÌfi (™∞). π‰È·›ÙÂÚ· ÁÈ· ÙËÓ ÚfiÎÏËÛË ·˘Ù‹˜ Ù˘ ‚Ï¿‚˘ ·Ô‰Â›¯ıËΠfiÙÈ ÛËÌ·ÓÙÈÎfiÙÂÚÔ ÚfiÏÔ ‰È·‰Ú·Ì·Ù›˙Ô˘Ó ÔÈ ‰È·Î˘Ì¿ÓÛÂȘ ÙÔ˘ fiÁÎÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ ÂÍ·ÈÙ›·˜ Ù˘ ÚÔηıÔÚÈṲ̂Ó˘ ÂÚÈÔ‰ÈÎfiÙËÙ·˜ ÙÔ˘ ™∞, ·Ú¿ ÔÈ ÌÂÙ·‚ÔϤ˜ ÛÙȘ ȤÛÂȘ ÙˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ. ∆Ô ÁÂÁÔÓfi˜ fiÙÈ Î·Ù¿ ÙÔÓ À∞∆, Ô fiÁÎÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ ·Ú·Ì¤ÓÂÈ Û¯ÂÙÈο ÛÙ·ıÂÚfi˜ Ì ÌÈÎÚ¤˜ ÌfiÓÔ ‰È·Î˘Ì¿ÓÛÂȘ, ı· ÌÔÚÔ‡Û ıˆÚËÙÈο Ó· ‰ÈηÈÔÏÔÁ‹ÛÂÈ ÙË ¯Ú‹ÛË ÙÔ˘, ÒÛÙ ӷ ÚÔÛٷ٤„ÂÈ ÙÔÓ ·ÓÒÚÈÌÔ ÓÂÔÁÓÈÎfi Ó‡ÌÔÓ· ·fi ‚Ï¿‚Ë. Œ¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› ΢ڛˆ˜ ˆ˜ ıÂڷ›· ‰È¿ÛˆÛ˘ Û ÙÂÏÂÈfiÌËÓ· ‹ ÌÂÁ¿Ï· ÚfiˆÚ· Ì ÛÔ‚·Ú‹ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· Î·È ·Ú·Ì¤ÓÔ˘Û· Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Î·È ˆ˜ ·Ú¯È΋ ıÂڷ¢ÙÈ΋ ÂÈÏÔÁ‹ Û ÚfiˆÚ· Ì ÛÔ‚·Úfi Û‡Ó‰ÚÔÌÔ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜ Î·È ÛÙ· Û‡Ó‰ÚÔÌ· ‰È·Ê˘Á‹˜ ·¤Ú·. √È ·fi„ÂȘ Û¯ÂÙÈο Ì ÙȘ ÂӉ›ÍÂȘ ÂÊ·ÚÌÔÁ‹˜ ÙÔ˘ Â›Ó·È ·ÎfiÌË ·ÓÙÈÎÚÔ˘fiÌÂÓ˜. ™ÙËÓ ·ÚÔ‡Û· ·Ó·ÛÎfiËÛË ı· ÂÚÈÁÚ·ÊÔ‡Ó ÔÈ ·Ú¯¤˜ ÏÂÈÙÔ˘ÚÁ›·˜, Ë Ê˘ÛÈÔÏÔÁ›· Ù˘ Ô͢ÁfiÓˆÛ˘ Î·È ÙÔ˘ ·ÂÚÈÛÌÔ‡ Î·È ÔÈ Ì˯·ÓÈÛÌÔ› ·ÓÙ·ÏÏ·Á‹˜ ·ÂÚ›ˆÓ Û ·˘Ùfi ÙÔ ÌÔÓÙ¤ÏÔ Ì˯·ÓÈÎÔ‡ ·ÂÚÈÛÌÔ‡. §¤ÍÂȘ ÎÏÂȉȿ: À„›Û˘¯ÓÔ˜ ·ÂÚÈÛÌfi˜ Ì ٷϷÓÙÒÛÂȘ, Ô͢ÁfiÓˆÛË, ·ÂÚÈÛÌfi˜, ·ÓÙ·ÏÏ·Á‹ ·ÂÚ›ˆÓ.
High frequency oscillatory ventilation: How it works 2nd NICU and Neonatology Department of the Aristotle University of Thessaloniki, G.P.N. “Papageorgiou”, Thessaloniki Correspondence: Karagianni Paraskevi karagpar@med.auth.gr 2nd NICU and Neonatology Department of Aristotle University of Thessaloniki. G.P.N. “Papageorgiou” Ring road Nea Efkarpia, 546 03, Thessaloniki
P. Karagianni, N. Nikolaides Abstract: High frequency oscillatory ventilation (HFOV) is an alternative mode of mechanical ventilation that uses small tidal volumes, usually smaller than the anatomically dead space and extremely rapid respiratory rates, above the normal, in order to achieve the oxygenation and ventilation of neonates. Several experimental studies showed that volutrauma rather than barotrauma is the initial cause of acute pulmonary damage, caused by conventional ventilation (CV). It was proved that the fluctuations of the lung volume due to the time cycled of conventional ventilation and not the pressure changes in the airway tracts play important role for this damage. During the application of HFOV, the lung volume remains relatively constant with only small fluctuations. This could theoretically justify its use in order to protect the immature neonatal lung from damage. It has been used mainly as rescue therapy in full term or near term neonates with serious respiratory insufficiency and persistent pulmonary hypertension and also as an initial therapy in premature neonates with serious respiratory distress and air leak syndromes. Opinions regarding the indication of its application are still controversial. This review describes the operational principles of HFOV and the relevant physiology and mechanisms of gas exchange during this mode of mechanical ventilation. Key words: High frequency oscillatory ventilation, oxygenation, ventilation, mechanisms of gas exchange.
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Y„›Û˘¯ÓÔ˜ ·ÂÚÈÛÌfi˜ Ì ٷϷÓÙÒÛÂȘ (HFOV) ™∞ ™˘Ì‚·ÙÈÎfi˜ ·ÂÚÈÛÌfi˜ Vt ∞Ó·ÓÂfiÌÂÓÔ˜ fiÁÎÔ˜ ·¤Ú· ™∞¢ ™‡Ó‰ÚÔÌÔ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜ Pa-w ª¤ÛË ›ÂÛË ·ÂÚ·ÁˆÁÒÓ ¢ƒ ¢È·ÊÔÚ¿ ›ÂÛ˘ Ù·Ï¿ÓÙˆÛ˘ Amplitude ∂‡ÚÔ˜ Ù·Ï¿ÓÙˆÛ˘ f ™˘¯ÓfiÙËÙ· Paediatriki 2007;70:18-23
√ ˘„›Û˘¯ÓÔ˜ ·ÂÚÈÛÌfi˜ Ì ٷϷÓÙÒÛÂȘ (High frequency oscillatory ventilation, HFOV) ·ÔÙÂÏ› ¤Ó·Ó ÂÓ·ÏÏ·ÎÙÈÎfi ÙÚfiÔ Ì˯·ÓÈÎÔ‡ ·ÂÚÈÛÌÔ‡ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ·Ó¿ÚÎÂÈ·˜ ÛÙ· ÓÂÔÁÓ¿. ∆Ô Î‡ÚÈÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÙÔ˘ Â›Ó·È fiÙÈ ¯ÚËÛÈÌÔÔÈ› ÌÈÎÚÔ‡˜ ·Ó·ÓÂfiÌÂÓÔ˘˜ fiÁÎÔ˘˜ ·¤Ú· (Vt, tidal volume), Û˘¯Ó¿ ÌÈÎÚfiÙÂÚÔ˘˜ ·fi ÙÔÓ ·Ó·ÙÔÌÈο ÓÂÎÚfi ¯ÒÚÔ Î·È Ôχ ÁÚ‹ÁÔÚÔ˘˜ ·Ó·Ó¢ÛÙÈÎÔ‡˜
19
À„›Û˘¯ÓÔ˜ ·ÂÚÈÛÌfi˜ Ì ٷϷÓÙÒÛÂȘ
Ú˘ıÌÔ‡˜ (·fi 2,5 ¤ˆ˜ 30 Hz) ÁÈ· ÙËÓ ÂÍ·ÛÊ¿ÏÈÛË Ù˘ Ô͢ÁfiÓˆÛ˘ Î·È ÙÔ˘ ·ÂÚÈÛÌÔ‡ ÛÙ· ÓÂÔÁÓ¿ (1-3). ∆· ÚÒÙ· ÂÈÚ¿Ì·Ù· Û¯ÂÙÈο Ì ÙȘ ·Ú¯¤˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ˘„›Û˘¯ÓÔ˘ ·ÂÚÈÛÌÔ‡ Ì ٷϷÓÙÒÛÂȘ (À∞∆) ÍÂΛÓËÛ·Ó ÙÔ 1915 ·fi ÙÔÓ Henderson (4), ·ÏÏ¿ Ô ÚÒÙÔ˜ ·Ó·Ó¢ÛÙ‹Ú·˜ ˘„ËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ηٷÛ΢¿ÛÙËΠ·fi ÙÔÓ Emmerson ÙÔ 1959. √È Lunkenheimer Î·È Û˘Ó. ÙÔ 1972, ·¤‰ÂÈÍ·Ó Û ÂÈÚ·Ì·ÙÈ΋ ÌÂϤÙË fiÙÈ Ë Ù·Ï¿ÓÙˆÛË ÌÈÎÚÒÓ fiÁÎˆÓ ·¤Ú· ÛÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ ÙˆÓ ÂÈÚ·Ì·Ùfi˙ˆˆÓ ‰È·ÙËÚÔ‡Û ÙÔ ·ÚÙËÚÈ·Îfi ‰ÈÔÍ›‰ÈÔ ÙÔ˘ ¿Óıڷη ÛÂ Ê˘ÛÈÔÏÔÁÈο ›‰· (5). ∞ÚÁfiÙÂÚ· ·Ô‰Â›¯ıËΠfiÙÈ ·Ó ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ·Ó·ÓÂfiÌÂÓÔÈ fiÁÎÔÈ ÌÈÎÚfiÙÂÚÔÈ ·fi ÙÔÓ ·Ó·ÙÔÌÈο ÓÂÎÚfi ¯ÒÚÔ, ÌÔÚ› Ó· ‰È·ÙËÚËı› Ô Î˘„ÂÏȉÈÎfi˜ ·ÂÚÈÛÌfi˜ Û ÈηÓÔÔÈËÙÈο ›‰· (6,7). ∏ ÚÒÙË ÎÏÈÓÈ΋ ÂÊ·ÚÌÔÁ‹ ÙÔ˘ À∞∆ Û ÓÂÔÁÓ¿ Ú·ÁÌ·ÙÔÔÈ‹ıËΠ·fi ÙÔ˘˜ Marchak Î·È Û˘Ó. (8). √ À∞∆ ¯ÚËÛÈÌÔÔÈ‹ıËΠ·Ú¯Èο Ì ÙËÓ ÂÔ›ıËÛË fiÙÈ ÏfiÁˆ ÙˆÓ È‰È·›ÙÂÚˆÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ ÙÔ˘ ÌÔÚ› Ó· ‚ÂÏÙÈÒÛÂÈ ÙËÓ ·ÓÙ·ÏÏ·Á‹ ÙˆÓ ·ÂÚ›ˆÓ Û ÓÂÔÁÓ¿ Ì ÛÔ‚·Ú‹ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ·, ·ÏÏ¿ Î·È Ó· ÌÂÈÒÛÂÈ ÙȘ ÔÍ›˜ ‹ ¯ÚfiÓȘ ÂÈÏÔΤ˜ Ô˘ ÔÊ›ÏÔÓÙ·È ÛÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘ Ì˯·ÓÈÎÔ‡ ·ÂÚÈÛÌÔ‡. ∞ÚÎÂÙ¤˜ ÂÈÚ·Ì·ÙÈΤ˜ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ ÙÔ ÔÁÎfiÙÚ·˘Ì· Ì¿ÏÏÔÓ ·Ú¿ ÙÔ ‚·ÚfiÙÚ·˘Ì· Â›Ó·È Ë ·Ú¯È΋ ·ÈÙ›· Ù˘ ÔÍ›·˜ Ó¢ÌÔÓÈ΋˜ ‚Ï¿‚˘ Ô˘ ÚÔηÏÂ›Ù·È ·fi ÙÔÓ Û˘Ì‚·ÙÈÎfi ·ÂÚÈÛÌfi (™∞) (9-12). π‰È·›ÙÂÚ· ÁÈ· ÙËÓ ÚfiÎÏËÛË ·˘Ù‹˜ Ù˘ ‚Ï¿‚˘ ·Ô‰Â›¯ıËΠfiÙÈ ÛËÌ·ÓÙÈÎfiÙÂÚÔ ÚfiÏÔ ‰È·‰Ú·Ì·Ù›˙Ô˘Ó ÔÈ ‰È·Î˘Ì¿ÓÛÂȘ ÙÔ˘ fiÁÎÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ ÂÍ·ÈÙ›·˜ Ù˘ ÚÔηıÔÚÈṲ̂Ó˘ ÂÚÈÔ‰ÈÎfiÙËÙ·˜ (time cycled) ÙÔ˘ ™∞, ·Ú¿ ÔÈ ÌÂÙ·‚ÔϤ˜ ÛÙȘ ȤÛÂȘ ÙˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ. ∆Ô ÁÂÁÔÓfi˜ fiÙÈ Î·Ù¿ ÙÔÓ À∞∆, Ô fiÁÎÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ ·Ú·Ì¤ÓÂÈ Û¯ÂÙÈο ÛÙ·ıÂÚfi˜ Ì ÌÈÎÚ¤˜ ÌfiÓÔ ‰È·Î˘Ì¿ÓÛÂȘ, ı· ÌÔÚÔ‡Û ıˆÚËÙÈο Ó· ‰ÈηÈÔÏÔÁ‹ÛÂÈ ÙË ¯Ú‹ÛË ÙÔ˘, ÒÛÙ ӷ ÚÔÛٷ٤„ÂÈ ÙÔÓ ·ÓÒÚÈÌÔ ÓÂÔÁÓÈÎfi Ó‡ÌÔÓ· ·fi ‚Ï¿‚Ë (13). ∂ÈϤÔÓ ¤¯ÂÈ ÙÔ ÏÂÔÓ¤ÎÙËÌ·, ÂÊfiÛÔÓ ÂÊ·ÚÌÔÛÙ› Ë Î·Ù¿ÏÏËÏË ÛÙÚ·ÙËÁÈ΋, Ó· ÂÙ‡¯ÂÈ ÈÔ ÔÌÔÈÔÁÂÓ‹ ¤ÎÙ˘ÍË ÙÔ˘ Ó‡ÌÔÓ·, Ì›ˆÛË ÙˆÓ ÂÓ‰ÔÓ¢ÌÔÓÈÎÒÓ ‰È·Ê˘ÁÒÓ, Ù·¯‡ÙÂÚË ‚ÂÏÙ›ˆÛË ÛÙË Û¯¤ÛË ·ÂÚÈÛÌÔ‡-·ÈÌ¿ÙˆÛ˘ Î·È Ù˘ Ô͢ÁfiÓˆÛ˘. ªÂ ÙËÓ Ù·¯‡ÙÂÚË ‚ÂÏÙ›ˆÛË ÛÙËÓ Ô͢ÁfiÓˆÛË ÌÂÈÒÓÂÙ·È Ô ¯ÚfiÓÔ˜ ¯ÔÚ‹ÁËÛ˘ √2, ¿Ú· Î·È Ë ¤ÎıÂÛË ÙÔ˘ Ó‡ÌÔÓ· ÛÙȘ ÂχıÂÚ˜ Ú›˙˜ ÙÔ˘ Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ· ÚfiÎÏËÛË Ó¢ÌÔÓÈ΋˜ ‚Ï¿‚˘ (14).
∞Ú¯¤˜ ÏÂÈÙÔ˘ÚÁ›·˜ - ·Ú¿ÌÂÙÚÔÈ √ ˘„›Û˘¯ÓÔ˜ ·Ó·Ó¢ÛÙ‹Ú·˜ ÏÂÈÙÔ˘ÚÁ› Û·Ó ·ÓÙÏ›· Ì ÈÛÙfiÓÈ (¤Ì‚ÔÏÔ) ‹ Ù·Ï·ÓÙÔ‡ÌÂÓ· ‰È·ÊÚ¿ÁÌ·Ù·, Ù· ÔÔ›· ¿ÏÏÔÓÙ·È ÌÂ Û˘¯ÓfiÙËÙ· 4002400 ·Ó·ÓÔÒÓ ÛÙÔ ÏÂÙfi (15). ∫·Ù¿ ÙË ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ À∞∆ Ë ÂÈÛÓÔ‹ Î·È Ë ÂÎÓÔ‹ Â›Ó·È ÂÓÂÚÁËÙÈΤ˜.
√È Ù·Ï·Óو٤˜ ·Ú¿ÁÔ˘Ó Î·È ‰È·Ó¤ÌÔ˘Ó ÌÂÁ¿ÏÔ˘˜ fiÁÎÔ˘˜ ·¤Ú·. ™˘Ó¯‹˜ Î·È ÚÔηıÔÚÈṲ̂ÓË (Û l/min) ÚÔ‹ ·¤Ú· ‰ÈÔ¯ÂÙ‡ÂÙ·È Ì ٷ¯‡ Ú˘ıÌfi ·fi Ì›· ËÁ‹, Ë ÔÔ›· ·Ú¿ÁÂÈ ‹ ·Ú¤¯ÂÈ ÂÓ¤ÚÁÂÈ· ÛÙȘ Ù·Ï·ÓÙÒÛÂȘ. ŒÓ· ÂÏÂÁ¯fiÌÂÓÔ ÛËÌÂ›Ô ‰È·ÚÚÔ‹˜ ‹ ¯·ÌËÏ‹˜ ‰È¿‚·Û˘ Ê›ÏÙÚÔ ÂÈÙÚ¤ÂÈ ÛÙÔÓ ·¤Ú· Ó· ÂͤگÂÙ·È ·fi ÙÔ Û‡ÛÙËÌ· (16). ¶ÂÚÈÛÛfiÙÂÚÔ ·fi fiÏÔ˘˜ ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› Ô SensorMedics 3100A (SensorMedics, Corp Yorba Linda, CA) (17). √ Û˘ÁÎÂÎÚÈ̤ÓÔ˜ ·Ó·Ó¢ÛÙ‹Ú·˜ ʤÚÂÈ ÛÊÚ·ÁÈṲ̂ÓÔ ¤Ì‚ÔÏÔ Ì ڢıÌÈ˙fiÌÂÓË ÌÂٷΛÓËÛË fiÁÎÔ˘, ÚÔηÏÒÓÙ·˜ Ù·Ï·ÓÙÒÛÂȘ ÛÙȘ ·ÂÚÔÊfiÚÔ˘˜ Ô‰Ô‡˜. ∏ Û˘¯ÓfiÙËÙ· (frequency-f) Ú˘ıÌ›˙ÂÙ·È ÌÂٷ͇ 180-900 ·Ó·ÓÔ¤˜ ÙÔ ÏÂÙfi (315Hz), Ë Ì¤ÛË ›ÂÛË ·ÂÚ·ÁˆÁÒÓ (mean airway pressure - Pa-w) ·fi 3-45 cm H2O Î·È Ô ÂÈÛÓ¢ÛÙÈÎfi˜ ¯ÚfiÓÔ˜ (inspiratory time - π∆) Â›Ó·È ÌÂÙ·‚·ÏÏfiÌÂÓÔ˜ ̤¯ÚÈ ÙÔ 50% ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·Ó·Ó¢ÛÙÈÎÔ‡ ·ÎÏÔ˘. ∏ ‰È·ÊÔÚ¿ ›ÂÛ˘ Ù·Ï¿ÓÙˆÛ˘ (¢ƒ) ‹ ‡ÚÔ˜ Ù·Ï¿ÓÙˆÛ˘ (amplitude) ηıÔÚ›˙ÂÈ ÙÔÓ ·Ó·ÓÂfiÌÂÓÔ fiÁÎÔ Î·È Ú˘ıÌ›˙ÂÙ·È ¤ˆ˜ 90 cmH2O. ∏ ÚÔ‹ (flow) Ú˘ıÌ›˙ÂÙ·È ·fi 6-20 lt/min ÁÈ· Ù· ÌÈÎÚfiÙÂÚ· ÙˆÓ 1000 gr Î·È Ù· ÙÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿ ·ÓÙ›ÛÙÔȯ·. ∆Ô ÎÏ¿ÛÌ· ÂÈÛÓÂfiÌÂÓÔ˘ √2 (Fraction of inspired √xygen - Fi√2) Ú˘ıÌ›˙ÂÙ·È fiˆ˜ Î·È Û fiÏÔ˘˜ ÙÔ˘˜ ·Ó·Ó¢ÛÙ‹Ú˜. √ ·Ó·Ó¢ÛÙ‹Ú·˜ SensorMedics 3100A ¯ÚËÛÈÌÔÔÈ› ÙÔ ËÏÂÎÙÚÔÌ·ÁÓËÙÈο ηıÔ‰ËÁÔ‡ÌÂÓÔ ‰È¿ÊÚ·ÁÌ· ÁÈ· ÙËÓ ·Ú·ÁˆÁ‹ ›ÂÛ˘ ËÌÈÙÔÓÔÂȉԇ˜ ÌÔÚÊ‹˜ ̤۷ ÛÙÔ Î‡Îψ̷ ÙÔ˘ ·Ó·Ó¢ÛÙ‹Ú·, Ë ÔÔ›· ÂÊ·ÚÌfi˙ÂÙ·È ÛÙÔÓ ÂÓ‰ÔÙÚ·¯ÂÈ·Îfi ۈϋӷ. ∏ ΛÓËÛË Ù·Ï¿ÓÙˆÛ˘ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ÚÔηÏ› ÂÓÂÚÁËÙÈΤ˜ ÂÈÛÓ¢ÛÙÈΤ˜ Î·È ÂÎÓ¢ÛÙÈΤ˜ Ê¿ÛÂȘ, ÔÈ Ôԛ˜ ηÙ¢ı‡ÓÔ˘Ó ÙÔ Ì›ÁÌ· ÙˆÓ ·ÂÚ›ˆÓ ÌÂٷ͇ ÙÔ˘ ΢ÎÏÒÌ·ÙÔ˜ ÙÔ˘ ·Ó·Ó¢ÛÙ‹Ú· Î·È ÙˆÓ Ó¢ÌÔÓÈÎÒÓ Î˘„ÂÏ›‰ˆÓ ÙÔ˘ ·ÛıÂÓ‹. ∏ ‰È·ÊÔÚ¿ ›ÂÛ˘ ˘ÔÏÔÁ›˙ÂÙ·È ÛÙËÓ ˘Ô‰Ô¯‹ ÙÔ˘ ÂÓ‰ÔÙÚ·¯ÂÈ·ÎÔ‡ ۈϋӷ ˆ˜ ¢ƒ Î·È Î·ıÔÚ›˙ÂÈ ÙÔÓ ·Ó·ÓÂfiÌÂÓÔ fiÁÎÔ Ô˘ Êı¿ÓÂÈ ÛÙÔÓ ·ÛıÂÓ‹ Û οı ·Ó·ÓÔ‹. ∫¿ı ÌÂÙ·‚ÔÏ‹ ÛÙËÓ ¢ƒ ÂËÚ¿˙ÂÈ Î·È ÙÔÓ ·ÂÚÈÛÌfi ÙÔ˘ ÓÂÔÁÓÔ‡. ∏ ̤ÛË ›ÂÛË ÙˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ ‰È¤ÂÙ·È ·fi ÙÔÓ Ú˘ıÌfi ÚÔ‹˜ ·¤Ú· ̤۷ ÛÙÔ Î‡Îψ̷ Î·È ÙËÓ ·ÓÙ›ÛÙ·ÛË ÚÔ˜ ÙËÓ ÂÎÓ¢ÛÙÈ΋ ÏÂ˘Ú¿ ÙÔ˘ ΢ÎÏÒÌ·ÙÔ˜. ∆Ô Â‡ÚÔ˜ Ù·Ï¿ÓÙˆÛ˘ (amplitude) Ù˘ ‰È·ÊÔÚ¿˜ ›ÂÛ˘ (¢P), Ô˘ ·Ú¿ÁÂÙ·È ·fi ÙÔ ‰È¿ÊÚ·ÁÌ· Î·È Ë Ì¤ÛË ›ÂÛË ÙˆÓ ·ÂÚ·ÁˆÁÒÓ, ÌÔÚÔ‡Ó Ó· Ú˘ıÌÈÛÙÔ‡Ó ·ÓÂÍ¿ÚÙËÙ·. ÕÚ·, ÌÔÚԇ̠ӷ ¤¯Ô˘Ì ÙËÓ ›‰È· ›ÂÛË Ì ‰È·ÊÔÚÂÙÈÎfi ‡ÚÔ˜ Ù·Ï¿ÓÙˆÛ˘ (17).
√͢ÁfiÓˆÛË Î·È ·ÂÚÈÛÌfi˜ ∏ Ê˘ÛÈÔÏÔÁ›· Ù˘ ·ÓÙ·ÏÏ·Á‹˜ ·ÂÚ›ˆÓ Ì ÙÔÓ À∞∆ Û ۇÁÎÚÈÛË Ì ™∞ Â›Ó·È ÂÍ·ÈÚÂÙÈο ÈÔ Û‡ÓıÂÙË. ∏ ¶·È‰È·ÙÚÈ΋ 2007;70:18-23
20
¶. ∫·Ú·ÁÈ¿ÓÓË, ¡. ¡ÈÎÔÏ·˝‰Ë˜
·. √͢ÁfiÓˆÛË ∏ Ô͢ÁfiÓˆÛË ÛÙÔÓ À∞∆ ÂÍ·ÚÙ¿Ù·È ·fi ÙË Ì¤ÛË ›ÂÛË ·ÂÚ·ÁˆÁÒÓ (Pa-w). √È ÂÚÈÛÛfiÙÂÚ˜ ·Ó·Ó¢ÛÙÈΤ˜ ·ı‹ÛÂȘ ÙˆÓ ÓÂÔÁÓÒÓ Â›Ó·È Î˘Ú›ˆ˜ ÂÚÈÔÚÈÛÙÈÎÔ‡ Ù‡Ô˘. ∏ ‚·ÛÈ΋ ·Ú¯‹ Ù˘ ÂÈı˘ÌËÙ‹˜ Ô͢ÁfiÓˆÛ˘ ÛÙÔÓ À∞∆ Â›Ó·È Ë Â›Ù¢ÍË Î·È ‰È·Ù‹ÚËÛË Ù˘ ‚¤ÏÙÈÛÙ˘ ¤ÎÙ˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ (optimal lung inflation) Ì ÙËÓ ÂÈÛÙÚ¿Ù¢ÛË ÂÚÈÛÛfiÙÂÚˆÓ Î˘„ÂÏ›‰ˆÓ, ¯ˆÚ›˜ ˘ÂډȿٷÛË ÙˆÓ Ó¢ÌfiÓˆÓ (22). √È ˙ÒÓ˜ Ù˘ ¤ÎÙ˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙÔÓ À∞∆ ·ÂÈÎÔÓ›˙ÔÓÙ·È ÛÙËÓ ∂ÈÎfiÓ· 1. ™ÙË ˙ÒÓË ∞, Ô Ó‡ÌÔÓ·˜ ˘Ô-ÂÎÙ‡ÛÛÂÙ·È, ÂÓÒ Ë ·‡ÍËÛË ÙÔ˘ ‡ÚÔ˘˜ Ù·Ï·ÓÙÒÛÂˆÓ ÂÈʤÚÂÈ ÌfiÓÔ ÌÈÎÚ¤˜ ·ÏÏ·Á¤˜ ÛÙÔÓ fiÁÎÔ ÙÔ˘. ∫ÏÈÓÈο, ÂÎÊÚ¿˙ÂÙ·È Ì ˘„ËϤ˜ ··ÈÙ‹ÛÂȘ Û Ô͢ÁfiÓÔ Î·È ÂÚÈÔÚÈṲ̂Ó˜ ‰ÔÓ‹ÛÂȘ ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ ÙÔȯÒÌ·ÙÔ˜. ∏ ˙ÒÓË µ ·ÔÙÂÏ› ÙËÓ ÂÚÈÔ¯‹ Ù˘ ‚¤ÏÙÈÛÙ˘ ¤ÎÙ˘Í˘. ∂ÊfiÛÔÓ Ô Ó‡ÌÔÓ·˜ ¤¯ÂÈ ÂÎÙ˘¯ı› Ì ˘„ËÏ‹ Pa-w, Ë Ó¢ÌÔÓÈ΋ ·ÁÁÂȷ΋ ·ÓÙ›ÛÙ·ÛË ÌÂÈÒÓÂÙ·È Î·È ÙfiÙÂ, ÌÈÎÚfiÙÂÚ· ‡ÚË Ù·Ï¿ÓÙˆÛ˘ Ô‰ËÁÔ‡Ó Û ÌÂÁ·Ï‡ÙÂÚ˜ ·ÏÏ·Á¤˜ ÛÙÔÓ fiÁÎÔ. ∫ÏÈÓÈο, ÂÎÊÚ¿˙ÂÙ·È Ì ÙÒÛË ÙˆÓ ··ÈÙ‹ÛÂˆÓ Û Ô͢ÁfiÓÔ Î·È Î·Ï¤˜ ‰ÔÓ‹ÛÂȘ ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ ÙÔȯÒÌ·ÙÔ˜. ∏ ˙ÒÓË ° ·ÓÙ·Ó·ÎÏ¿ ÙËÓ ˘ÂډȿٷÛË ÙÔ˘ Ó‡ÌÔÓ·. ∫·È ÛÙË ˙ÒÓË ·˘Ù‹ ÌÔÚ› Ó· ··ÈÙËı› ÌÂÁ·Ï‡ÙÂÚÔ Â‡ÚÔ˜ Ù·Ï¿ÓÙˆÛ˘ ÁÈ· Ó· ÚÔηϤÛÂÈ ·ÏÏ·Á¤˜ ÛÙÔÓ fiÁÎÔ, ÂÓÒ Ô ˘ÂډȷÙÂٷ̤ÓÔ˜ Ó‡ÌÔÓ·˜ ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ ÙËÓ Î·Ú‰È·Î‹ ·ÚÔ¯‹, ÏfiÁˆ Ì›ˆÛ˘ Ù˘ ÊÏ‚È΋˜ ·ӷÊÔÚ¿˜. ∞˘Ùfi ·ÔÙÂÏ› Î·È ÙÔ ÈÔ ÂÈΛӉ˘ÓÔ ÛËÌÂ›Ô ÛÙË ¯Ú‹ÛË ÙÔ˘. ∂›Ó·È ‰‡ÛÎÔÏÔ Ó· ·Ó·ÁÓˆÚÈÛı› ÎÏÈÓÈο, ÁÈ·Ù› ÔÈ ··ÈÙ‹ÛÂȘ Û Ô͢ÁfiÓÔ ‰È·ÙËÚÔ‡ÓÙ·È ·Ú¯Èο ¯·ÌËϤ˜ Î·È Ë Ì›ˆÛË ÙˆÓ ‰ÔÓ‹ÛÂˆÓ ÌÔÚ› Ó· ÌËÓ ·ÍÈÔÏÔÁËı›. ∏ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ·Ú·Ì¤ÓÂÈ ÙÔ ·ÛʷϤÛÙÂÚÔ Ì¤ÛÔÓ ÂÎÙ›ÌËÛ˘ Ù˘ ¤ÎÙ˘Í˘. ∏ ˙ÒÓË ¢, Ù¤ÏÔ˜, ·ÓÙÈηÙÔÙÚ›˙ÂÈ ÙÔÓ ‚¤ÏÙÈÛÙÔ fiÁÎÔ ÁÈ· ÙËÓ ·Ô‰¤ÛÌ¢ÛË ·fi ÙÔÓ ·Ó·Ó¢ÛÙ‹Ú·. ™Ùfi¯Ô˜ Â›Ó·È Ë ÌÂٿوÛË ÙˆÓ Ó¢ÌfiÓˆÓ ·fi ÙÔ ÛËÌÂ›Ô µ ÛÙÔ ÛËÌÂ›Ô ¢, ·ÔʇÁÔÓÙ·˜ ÙË ˙ÒÓË °. ∂ÊfiÛÔÓ ÂÈÙ¢¯ı› Ë ‰È·Ù‹ÚËÛË Ù˘ ‚¤ÏÙÈÛÙ˘ ¤ÎÙ˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ Î·È ÙÔ˘ ·ÂÚÈÛÌÔ‡ Ì ÛÙ·‰È·Î¤˜ ÌÂÈÒÛÂȘ Ù˘ Pa-w ÌÔÚԇ̠ӷ ˘ÔÛÙËÚ›ÍÔ˘Ì ÙÔ ÓÂÔÁÓfi Ì ¯·ÌËÏ‹ Pa-w. ∂¿Ó fï˜ Ë Pa-w ÌÂȈı› ¿Ú· Ôχ, ˘¿Ú¯ÂÈ Î›Ó‰˘ÓÔ˜ Ó· ·˘ÍËıÔ‡Ó Î·È ¿ÏÈ ÔÈ ··ÈÙ‹ÛÂȘ Û Ô͢ÁfiÓÔ (∂ÈÎfiÓ· 1). ‚. ∞ÂÚÈÛÌfi˜ ∏ Ô˘ÛÈ·ÛÙÈ΋ ‰È·ÊÔÚ¿ ÛÙËÓ ·Ô‚ÔÏ‹ ‰ÈÔÍÂȉ›Ô˘ ÙÔ˘ ¿Óıڷη ÌÂٷ͇ ™∞ Î·È À∞∆, Â›Ó·È fiÙÈ Ô ™∞ ·Ô‰›‰ÂÈ ·Ó·Óˆ̤ÓÔ ·¤Ú· ÛÙȘ ΢„ÂÏ›‰Â˜ ÌfiÓÔ Ì ÙÔ Ê·ÈÓfiÌÂÓÔ Ù˘ ÔÁÎÒ‰Ô˘˜ ÚÔ‹˜ (bulk flow), ÂÓÒ Ô Paediatriki 2007;70:18-23
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·ÏÏËÏ›‰Ú·ÛË ÙˆÓ ·Ú·ÁfiÓÙˆÓ Ô˘ ÂËÚ¿˙Ô˘Ó ÙËÓ ·ÓÙ·ÏÏ·Á‹ ·ÂÚ›ˆÓ ÛÙÔÓ À∞∆ ¤¯ÂÈ ÌÂÏÂÙËı› ·fi ·ÚÎÂÙÔ‡˜ ÂÚ¢ÓËÙ¤˜ (18-21).
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∞ ¶π∂™∏ (cmH2O) ∂ÈÎfiÓ· 1. ∑ÒÓ˜ Ù˘ ¤ÎÙ˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙÔÓ À∞∆: ∑ÒÓË ∞: ˘Ô¤ÎÙ˘ÍË. ∑ÒÓË B: ‚¤ÏÙÈÛÙË ¤ÎÙ˘ÍË. ∑ÒÓË °: ˘ÂډȿٷÛË. ∑ÒÓË ¢: ‚¤ÏÙÈÛÙË ¤ÎÙ˘ÍË Î·Ù¿ ÙÔÓ ·ÔÁ·Ï·ÎÙÈÛÌfi. §ÀÃ: §ÂÈÙÔ˘ÚÁÈ΋ ˘ÔÏÂÈfiÌÂÓË ¯ˆÚËÙÈÎfiÙËÙ·, À∞∆: À„›Û˘¯ÓÔ˜ ·ÂÚÈÛÌfi˜ Ì ٷϷÓÙÒÛÂȘ.
À∞∆ ÚÔηÏ› ÙfiÛÔ ÔÁÎÒ‰Ë ÚÔ‹, fiÛÔ Î·È Ì›ÍË. ∞˘Ù‹ Ë ‰˘Ó·ÙfiÙËÙ· ·Ó¿ÌÈ͢ ÙÔ˘ ·¤Ú· ÌÂٷ͇ ÙˆÓ ·ÓÒÙÂÚˆÓ ·ÂÚ·ÁˆÁÒÓ Î·È ÙˆÓ Î˘„ÂÏ›‰ˆÓ οÓÂÈ ÙÔÓ À∞∆ ÂÚÈÛÛfiÙÂÚÔ ·ÔÙÂÏÂÛÌ·ÙÈÎfi. ™ÙÔÓ ™∞ o ·ÂÚÈÛÌfi˜, ‰ËÏ·‰‹ Ë ·ÔÌ¿ÎÚ˘ÓÛË ÙÔ˘ ‰ÈÔÍÂȉ›Ô˘ ÙÔ˘ ¿Óıڷη, Â›Ó·È ·Ó¿ÏÔÁÔ˜ Ù˘ Û˘¯ÓfiÙËÙ·˜ › ÙÔÓ ·Ó·ÓÂfiÌÂÓÔ fiÁÎÔ (VECO2 = f x Vt ). ∞ÓÙÈı¤Ùˆ˜ ÛÙÔÓ À∞∆ Ë ·ÔÌ¿ÎÚ˘ÓÛË ÙÔ˘ ‰ÈÔÍÂȉ›Ô˘ ÙÔ˘ ¿Óıڷη ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ·fi ÙËÓ Â͛ۈÛË VECO2 = fa x Vtb ‹ fa x amplitudeb), fiÔ˘ a Î˘Ì·›ÓÂÙ·È ·fi 0,75 ¤ˆ˜ 1,24 Î·È b ·fi 1,5 ¤ˆ˜ 2,2. ª›· ·fi ÙȘ ÂÈÙÒÛÂȘ ·˘Ù‹˜ Ù˘ Û¯¤Û˘ Â›Ó·È fiÙÈ ÛÙÔÓ À∞∆, ÌÈÎÚ¤˜ ·ÏÏ·Á¤˜ ÛÙÔÓ ·Ó·ÓÂfiÌÂÓÔ fiÁÎÔ ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚË Â›‰Ú·ÛË ÛÙÔÓ ·ÂÚÈÛÌfi ·’ fi,ÙÈ ÔÈ ÌÈÎÚ¤˜ ·ÏÏ·Á¤˜ ÛÙË Û˘¯ÓfiÙËÙ· (17,22). À„ËÏfiÙÂÚ· ‡ÚË Ù·Ï¿ÓÙˆÛ˘ ·˘Í¿ÓÔ˘Ó ÙÔÓ ·Ó·ÓÂfiÌÂÓÔ fiÁÎÔ Î·È, ˆ˜ ·ÎfiÏÔ˘ıÔ, ÙËÓ ·ÔÌ¿ÎÚ˘ÓÛË ÙÔ˘ CO2. ∞ÓÙÈı¤Ùˆ˜ Ë ·‡ÍËÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ Ô‰ËÁ› ÛÙÔ ·Ú¿‰ÔÍÔ Ê·ÈÓfiÌÂÓÔ Ù˘ Ì›ˆÛ˘ Ù˘ ·Ô‚ÔÏ‹˜ ÙÔ˘ CO2 (23) Î·È Û ÛËÌ·ÓÙÈ΋ Ì›ˆÛË ÙÔ˘ Vt. ∆Ô ·Ú·¿Óˆ ÈÛ¯‡ÂÈ ÛÙÔÓ SensorMedics, ·Ó Ë ¢ƒ ·Ú·Ì¤ÓÂÈ ÛÙ·ıÂÚ‹. À¿Ú¯Ô˘Ó HFOV Ô˘ ‰È·ÙËÚÔ‡Ó ÛÙ·ıÂÚfi Vt ·ÓÂÍ¿ÚÙËÙ· ·fi ÙË Û˘¯ÓfiÙËÙ· (Hummingbird). ∞˘Ù‹ Ë Û‡ÓıÂÙË Û¯¤ÛË ÌÂٷ͇ Û˘¯ÓfiÙËÙ·˜ Î·È CO2 ÂÍËÁ› ÁÈ·Ù› Ë Û˘¯ÓfiÙËÙ· ‰ÂÓ Â›Ó·È Ë ÚˆÙ·Ú¯È΋ ·Ú¿ÌÂÙÚÔ˜ fiÙ·Ó ı¤ÏÔ˘Ì ӷ ‚ÂÏÙÈÒÛÔ˘Ì ÙÔÓ ·ÂÚÈÛÌfi ÛÙÔÓ À∞∆ (24).
ªË¯·ÓÈÛÌÔ› ÌÂÙ·ÊÔÚ¿˜ Î·È ·ÓÙ·ÏÏ·Á‹˜ ·ÂÚ›ˆÓ √È Ì˯·ÓÈÛÌÔ› Ì ÙÔ˘˜ ÔÔ›Ô˘˜ Ô À∞∆ ‚ÂÏÙÈÒÓÂÈ ÙËÓ ·Ó¿ÌÈÍË ÙÔ˘ ·¤Ú· ÌÂٷ͇ ÙˆÓ ·ÓÒÙÂÚˆÓ ·ÂÚ·ÁˆÁÒÓ Î·È ÙˆÓ Î˘„ÂÏ›‰ˆÓ Â›Ó·È ÂÍ·ÈÚÂÙÈο Û‡ÓıÂÙÔÈ. §ÂÙÔÌÂÚ‹˜ ·Ó¿Ï˘ÛË ÙˆÓ Ì˯·ÓÈÛÌÒÓ ·˘ÙÒÓ ÂÚÈÁÚ¿ÊÂÙ·È ÛÙËÓ ÎÏ·ÛÈ΋ ÂÚÁ·Û›· ÙÔ˘ Chang (22) Î·È ÈÔ ÚfiÛÊ·Ù· Ù˘ Pillow (25).
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À„›Û˘¯ÓÔ˜ ·ÂÚÈÛÌfi˜ Ì ٷϷÓÙÒÛÂȘ
·. ªÂÙ·ÊÔÚ¿ (Convection) ªÂÙ¿ ·fi Ï›ÁÔ˘˜ ·Ó·Ó¢ÛÙÈÎÔ‡˜ ·ÎÏÔ˘˜ Ù·Ï¿ÓÙˆÛ˘, Ù· ÌfiÚÈ· ·¤Ú· Ô˘ ·Ú¯Èο ‹Ù·Ó ÛÙÔ Î¤ÓÙÚÔ ÙÔ˘ ·ÂÚ·ÁˆÁÔ‡ ÂÎÙÔ͇ÔÓÙ·È ÚÔ˜ Ù· ÚfiÛˆ, ÛÙÔ Ì¤ÛÔÓ ÙˆÓ ‰‡Ô ÂÚÈÊÂÚÈÎÒÓ ·ÂÚ·ÁˆÁÒÓ Î·È Ù· ÌfiÚÈ·, Ô˘ ‹Ù·Ó ·Ú¯Èο ÛÙËÓ ÂÚÈʤÚÂÈ· ÎÔÓÙ¿ ÛÙȘ Ï¢ڤ˜ ÙÔ˘ ·ÚÈÔ˘ ·ÂÚ·ÁˆÁÔ‡, ÌÂÙ·ÎÈÓÔ‡ÓÙ·È ÚÔ˜ Ù· ›Ûˆ ‹ ·Â˘ı›·˜ ÛÙËÓ ÙÚ·¯Â›·. Œ¯ÂÈ ˘ÔÏÔÁÈÛÙ› fiÙÈ Û οı ·ÎÏÔ Û˘Ì‚·›ÓÂÈ ·ÓÙ·ÏÏ·Á‹ ·ÂÚ›ˆÓ Ì ÌÂÙ·ÊÔÚ¿ ηٿ 5% (26). ‚. ∂·˘Í·ÓfiÌÂÓË ‰È¿¯˘ÛË (Augmented diffusion) ™ÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ ‹ ÛÙ· ÛËÌ›· ÙÔ˘ ‰È¯·ÛÌÔ‡, Û˘Ì‚·›ÓÂÈ ‰Â˘ÙÂÚÔ·ı‹˜ ·Ó¿ÌÈÍË ÙÔ˘ ·¤Ú·, Ë ÔÔ›· ·Ê’ ÂÓfi˜ ÌÂÓ Ô‰ËÁ› ۠ϢÚÈ΋ ·Ó¿ÌÈÍË ÙˆÓ ·ÂÚ›ˆÓ ·ÏÏ¿, ·Ê’ ÂÙ¤ÚÔ˘ ÂÌÔ‰›˙ÂÈ ÙËÓ Î·Ù¿ Ì‹ÎÔ˜ ÌÂÙ·ÊÔÚ¿ ÙÔ˘˜. ∏ ·ÏÌÈ΋ ΛÓËÛË ÙˆÓ ÙÔȯˆÌ¿ÙˆÓ ÙˆÓ ‚ÚfiÁ¯ˆÓ ÌÔÚ› Ó· ·Ó·ÛÙÚ¤„ÂÈ ÙË ÚÔ‹ ÙÔ˘ ·¤Ú·, ·˘Í¿ÓÔÓÙ·˜ ¤ÙÛÈ ÙË ‰È·ÊÔÚ¿ ÛÙË Û˘ÁΤÓÙÚˆÛË ÌÂٷ͇ ÙˆÓ ‰‡Ô ·ÂÚ›ˆÓ. ∞˘Ùfi ÚÔηÏ› ÌÂÁ·Ï‡ÙÂÚË Î·Ù¿ Ì‹ÎÔ˜ ÌÂٷΛÓËÛË ÙÔ˘ ·¤Ú·. Á. ƒÔ‹ ÌÂÁ¿ÏÔ˘ fiÁÎÔ˘ (Bulk flow) ∂›Ó·È ÁÂÓÈο ·Ô‰ÂÎÙfi fiÙÈ Ô À∞∆ ÏÂÈÙÔ˘ÚÁ› Ì ·Ó·ÓÂfiÌÂÓÔ˘˜ fiÁÎÔ˘˜ ·¤Ú· Ô˘ Â›Ó·È ÂÚ›Ô˘ ÈÛÔ‰‡Ó·ÌÔÈ Ì ÙÔÓ fiÁÎÔ ÙÔ˘ ·Ó·ÙÔÌÈο ÓÂÎÚÔ‡ ¯ÒÚÔ˘. øÛÙfiÛÔ ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ·, ÌÂÚÈΤ˜ ΢„ÂÏ›‰Â˜ ‚Ú›ÛÎÔÓÙ·È ÈÔ ÎÔÓÙ¿, ·’ fiÙÈ Î¿ÔȘ ¿ÏϘ, ÛÙ· ·ÓÒÙÂÚ· ÙÌ‹Ì·Ù· ÙÔ˘ ·ÂÚ·ÁˆÁÔ‡. ŒÙÛÈ, Ô ·ÂÚÈÛÌfi˜ Ì ·Ó·ÓÂfiÌÂÓÔ˘˜ fiÁÎÔ˘˜ ·¤Ú· Ô˘ ÚÔÛÂÁÁ›˙Ô˘Ó ÙÔÓ fiÁÎÔ ÙÔ˘ ÓÂÎÚÔ‡ ¯ÒÚÔ˘, ‰›ÓÂÈ ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ· ÊÚ¤ÛÎÔ ·¤Ú· Û οÔȘ “ÏËÛȤÛÙÂÚ˜” ΢„ÂÏ›‰Â˜. ‰. º·ÈÓfiÌÂÓÔ Pendelluft O ÁÂÚÌ·ÓÈÎfi˜ fiÚÔ˜ Pendelluft ‹ ηٿ ÚÔÛ¤ÁÁÈÛË “·ÈˆÚÔ‡ÌÂÓÔ˜ ·¤Ú·˜”, ‰ËÏÒÓÂÈ ¤Ó·Ó ÛËÌ·ÓÙÈÎfi Ì˯·ÓÈÛÌfi ·ÓÙ·ÏÏ·Á‹˜ ·ÂÚ›ˆÓ ÛÙÔÓ À∞∆. ¶ÚfiÎÂÈÙ·È ÁÈ· ÌÂٷΛÓËÛË ÙÔ˘ ·¤Ú· ÌÂٷ͇ “·Ú·Î›ÌÂÓˆÓ” ÌÔÓ¿‰ˆÓ ÙÔ˘ Ó‡ÌÔÓ· Î·È fi¯È ÌÂٷ͇ ÙˆÓ ·ÓÒÙÂÚˆÓ ·ÂÚ·ÁˆÁÒÓ Î·È ÙˆÓ Î˘„ÂÏ›‰ˆÓ. ∂Âȉ‹ Ë ÛÙ·ıÂÚ¿ ¯ÚfiÓÔ˘ (Tc, time constant) ÈÛÔ‡Ù·È Ì ÙÔ ÁÈÓfiÌÂÓÔ Ù˘ ·ÓÙ›ÛÙ·Û˘ (Resistance) › ÙËÓ Â˘ÂÓ‰ÔÙfiÙËÙ· (Compliance), (∆c=RxC) ˘¿Ú¯Ô˘Ó ÌÈÎÚ¤˜ ‰È·ÊÔÚ¤˜ ÛÙȘ ÙÈ̤˜ Û ‰È·ÊÔÚÂÙÈΤ˜ ΢„ÂÏ›‰Â˜. ∫·ıÒ˜ ÔÈ ·Ó·Ó¢ÛÙÈΤ˜ Û˘¯ÓfiÙËÙ˜ ·˘Í¿ÓÔ˘Ó Î·È ÔÈ ¯ÚfiÓÔÈ ÂÈÛÓÔ‹˜ Î·È ÂÎÓÔ‹˜ Á›ÓÔÓÙ·È ÌÈÎÚÔ› Û ۯ¤ÛË Ì ÙËÓ ∆c, ‰ÂÓ ˘¿Ú¯ÂÈ Ï¤ÔÓ Â·Ú΋˜ ¯ÚfiÓÔ˜ ÁÈ· ÙȘ ΢„ÂÏȉÈΤ˜ ȤÛÂȘ Ó· ÂÍÈÛÔÚÚÔËıÔ‡Ó Ì ÙȘ ȤÛÂȘ ÙˆÓ ·ÓÒÙÂÚˆÓ ·ÂÚ·ÁˆÁÒÓ. ∂¿Ó ÔÈ Û˘¯ÓfiÙËÙ˜ ·˘ÍËıÔ‡Ó ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ Î·È ·ÓÙ›ÛÙÔȯ· ÌÂȈıÔ‡Ó ÔÈ ¯ÚfiÓÔÈ ÂÎ-
ÓÔ‹˜ Î·È ÂÈÛÓÔ‹˜, ÙfiÙ ÌÔÚ› Ó· ˘¿ÚÍÔ˘Ó ÛÙÈÁ̤˜ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ·ÎÏÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÔÔ›ˆÓ ˘¿Ú¯ÂÈ ÌÈ· ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÛÙËÓ ›ÂÛË (gradient) ÌÂٷ͇ ÙˆÓ ·Ú·Î›ÌÂÓˆÓ Î˘„ÂÏ›‰ˆÓ. ™ÙȘ ¯ÚÔÓÈΤ˜ ·˘Ù¤˜ ÛÙÈÁ̤˜, Ô ·¤Ú·˜ ı· Ú¤ÂÈ Ì¿ÏÏÔÓ ·fi ÙË Ì›· ΢„ÂÏ›‰· ÛÙËÓ ¿ÏÏË, ·Ú¿ ·fi ÙÔÓ ·ÂÚ·ÁˆÁfi ÚÔ˜ ÙȘ ΢„ÂÏ›‰Â˜. ¶Ú·ÁÌ·ÙÈο, Ë Â·Ú΋˜ Û˘ÓÙfiÌ¢ÛË ÙˆÓ ¯ÚfiÓˆÓ ÂÈÛÓÔ‹˜ Î·È ÂÎÓÔ‹˜ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÔÈ ÁÂÈÙÔÓÈΤ˜ ΢„ÂÏ›‰Â˜ Ó· Ô‰ËÁÔ‡ÓÙ·È ÂÎÙfi˜ Ê¿Û˘, Û ۯ¤ÛË Ì ÙȘ ¿ÏϘ. ŸÙ·Ó ·˘Ùfi Û˘Ì‚·›ÓÂÈ, Ë ‰È·ÓÔÌ‹ Û¯ÂÙÈο ÌÈÎÚÒÓ fiÁÎˆÓ ÚÔ˜ ÙÔ˘˜ ·ÓÒÙÂÚÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ Ô‰ËÁ› Û “Ì›ÍË ·¤Ú·” ·Ó¿ÌÂÛ· ÛÙȘ ΢„ÂÏ›‰Â˜ ·˘Ù¤˜. ∞˘Ùfi˜ Â›Ó·È Ô Ì˯·ÓÈÛÌfi˜ ÙÔ˘ “·ÈˆÚÔ‡ÌÂÓÔ˘ ·¤Ú·” Ô˘ ÌÂÙ·ÎÈÓÂ›Ù·È ÌÂٷ͇ ÙˆÓ ·Ú·Î›ÌÂÓˆÓ Î˘„ÂÏ›‰ˆÓ Î·È ÌÔÚ› Ó· ·˘Í¿ÓÂÈ ÙËÓ ·Ó¿ÌÈÍ‹ ÙÔ˘ ÛÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ (25,27). Â. ∞Û‡ÌÌÂÙÚ˜ ηٷÙÔ̤˜ Ù·¯˘Ù‹ÙˆÓ (Asymmetric velocity profiles) ŸÙ·Ó ÛÙÔÓ Î˘ÎÏÔÊÔÚÔ‡ÓÙ· ·¤Ú· οÔÈÔ˘ ·ÁˆÁÔ‡ ÂÊ·ÚÌfi˙ÂÙ·È ·fiÙÔÌË ›ÂÛË, Ô ·¤Ú·˜ ‰ÂÓ ÂÈÙ·¯‡ÓÂÈ ÔÌÔÈfiÌÔÚÊ·. √ ·¤Ú·˜ Ô˘ ‚Ú›ÛÎÂÙ·È ÛÙÔ Î¤ÓÙÚÔ ÙÔ˘ ·ÁˆÁÔ‡ ÎÈÓÂ›Ù·È Ì ٷ¯‡ÙËÙ· ÌÂÁ·Ï‡ÙÂÚË ·fi ÂΛÓË ÙÔ˘ ·¤Ú· ÛÙËÓ ÂÚÈʤÚÂÈ· ÙÔ˘ ۈϋӷ. ∏ ‰È·ÊÔÚ¿ ÌÂٷ͇ ÙÔ˘ ·Ó‡ÛÌ·ÙÔ˜ Ù·¯‡ÙËÙ·˜ ÙÔ˘ ·¤Ú· ÛÙÔ Î¤ÓÙÚÔ ÙÔ˘ ·ÁˆÁÔ‡ Î·È Ù˘ Ù·¯‡ÙËÙ·˜ ÛÙËÓ ÂÚÈʤÚÂÈ· ‹ Ë ·Û˘ÌÌÂÙÚ›· ÛÙËÓ Î·Ù·ÓÔÌ‹ ÙˆÓ Ù·¯˘Ù‹ÙˆÓ, ÂÍ·ÚÙ¿Ù·È ·fi ÔÏÏ·ÏÔ‡˜ ·Ú¿ÁÔÓÙ˜, ÛÙÔ˘˜ ÔÔ›Ô˘˜ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ë ‰È¿ÌÂÙÚÔ˜ ÙÔ˘ ·ÁˆÁÔ‡ Î·È Ë ÂÊ·ÚÌÔ˙fiÌÂÓË ›ÂÛË. ŸÙ·Ó ÂÊ·ÚÌfi˙ÂÙ·È Í·ÊÓÈο ÛÙÔÓ ·¤Ú· ˘„ËÏ‹ ›ÂÛË ÛÙÔÓ ·ÓÒÙÂÚÔ ·ÂÚ·ÁˆÁfi, Ô ·¤Ú·˜ ÛÙÔ Î¤ÓÙÚÔ ÙÔ˘ Ù·Íȉ‡ÂÈ Ì ÌÂÁ·Ï‡ÙÂÚË Ù·¯‡ÙËÙ· Î·È ÂÔ̤ӈ˜ ‚·ı‡ÙÂÚ· ̤۷ ÛÙÔÓ Ó‡ÌÔÓ·, Û ۇÁÎÚÈÛË Ì ÙËÓ ÂÚ›ÙˆÛË Ô˘ Ë ÂÊ·ÚÌÔÁ‹ Ù˘ ›ÂÛ˘ Á›ÓÂÙ·È ·ÚÁ¿ (fiˆ˜ ÛÙÔÓ ™∞) (28). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÎÓÔ‹˜, Ô ·¤Ú·˜ ÂÈÙ·¯‡ÓÂÈ ÚÔ˜ ÙÔ Â͈ÙÂÚÈÎfi ÙˆÓ Î˘„ÂÏ›‰ˆÓ Î·È Ë ÔÚ›· ÙÔ˘ ·ÓÙÈÛÙÚ¤ÊÂÙ·È. ∂Âȉ‹, Ë ‰È¿ÌÂÙÚÔ˜ ÙÔ˘ ·ÂÚ·ÁˆÁÔ‡ Â›Ó·È ÌÈÎÚfiÙÂÚË ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÎÓÔ‹˜ ·fi ÂΛÓË Ù˘ ÂÈÛÓÔ‹˜, Ô ‚·ıÌfi˜ ·Û˘ÌÌÂÙÚ›·˜ ÙˆÓ Î·Ù·ÙÔÌÒÓ Ù·¯‡ÙËÙ·˜ Â›Ó·È ÌÈÎÚfiÙÂÚÔ˜ ÛÙËÓ ÂÎÓÔ‹. ŒÙÛÈ ÂÍËÁÂ›Ù·È ÁÈ·Ù› Ù· ‰È·Ó‡ÛÌ·Ù· Ù‹˜ ÚÔ˜ Ù· ÂÌÚfi˜ (ÂÈÛÓ¢ÛÙÈ΋˜) Î·È Ù˘ ·Ó¿ÛÙÚÔÊ˘ (ÂÎÓ¢ÛÙÈ΋˜) Ù·¯‡ÙËÙ·˜ ÂÓfi˜ Ï‹ÚÔ˘˜ ·ÎÏÔ˘ À∞∆ ‰ÂÓ ·ÏÏËÏÔ·Ó·ÈÚÔ‡ÓÙ·È, ÂÈÛÙÚ¤ÊÔÓÙ·˜ fiÏÔ ÙÔÓ ·¤Ú· ÛÙËÓ ·Ú¯È΋ ÙÔ˘ ı¤ÛË. ÛÙ. ¢È·ÛÔÚ¿ Ù‡Ô˘ Taylor (Taylor-type dispersion) ª›· ·fi ÙȘ Û˘Ó¤ÂȘ ÙˆÓ ·Û‡ÌÌÂÙÚˆÓ Î·Ù·ÓÔÌÒÓ Ù·¯‡ÙËÙ·˜ Â›Ó·È fiÙÈ o ·Ó·Óˆ̤ÓÔ˜ ·¤Ú·˜ ÛÙÔ ¶·È‰È·ÙÚÈ΋ 2007;70:18-23
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¶. ∫·Ú·ÁÈ¿ÓÓË, ¡. ¡ÈÎÔÏ·˝‰Ë˜
ΤÓÙÚÔ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ ÂÚÈ‚¿ÏÏÂÙ·È ·fi ·¤Ú· ÙÔ˘ ÚÔËÁÔ‡ÌÂÓÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ·ÎÏÔ˘, ÛÙËÓ ÂÚÈʤÚÂÈ·. À¿Ú¯ÂÈ ‰ËÏ·‰‹ ‰È·ÊÔÚ¿ Û˘ÁΤÓÙÚˆÛ˘, ·fi ÙÔ Î¤ÓÙÚÔ ÚÔ˜ ÙËÓ ÂÚÈʤÚÂÈ· ÙfiÛÔ ÁÈ· ÙÔ √2 fiÛÔ Î·È ÁÈ· ÙÔ CO2. ∂Âȉ‹ Ë ·Ó¿ÌÈÍË Û˘Ì‚·›ÓÂÈ Û οı ÂÚ›ÙˆÛË fiÔ˘ ˘¿Ú¯ÂÈ Ë ‰È·ÊÔÚ¿ ÛÙË Û˘ÁΤÓÙÚˆÛË √2 Î·È CO2, ·˘Ùfi Ô‰ËÁ› Û ·ÎÙÈÓˆÙ‹ ‰È¿¯˘ÛË ·fi ÙÔÓ ·ÂÚ·ÁˆÁfi ÚÔ˜ ÙÔ Î¤ÓÙÚÔ Î·È ·fi ÙÔ Î¤ÓÙÚÔ ÚÔ˜ ÙËÓ ÂÚÈʤÚÂÈ·. ∞˘Ù‹ Ë ‰È¿¯˘ÛË, Ô˘ ÚÔηÏÂ›Ù·È ·fi ÙÔÓ Û˘Ó‰˘·ÛÌfi ÙˆÓ ·ÍÔÓÈÎÒÓ ·Û‡ÌÌÂÙÚˆÓ ·Ó˘ÛÌ¿ÙˆÓ Ù·¯‡ÙËÙ·˜ Î·È Ù˘ ·ÎÙÈÓˆÙ‹˜ ÎÏ›Û˘ Û˘ÁΤÓÙÚˆÛ˘, ÔÓÔÌ¿˙ÂÙ·È ‰È·ÛÔÚ¿ Ù‡Ô˘ Taylor.
ıÒ˜ Î·È ·ÍÈÔÏfiÁËÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ·ıÔÊ˘ÛÈÔÏÔÁ›·˜ ÙÔ˘ οı ÓÂÔÁÓÔ‡ ı· ‚ÔËıÔ‡Û ÛÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘. √È ÂÍÂÏ›ÍÂȘ ÛÙÔÓ ÙÔ̤· ÙÔ˘ Ì˯·ÓÈÎÔ‡ ·ÂÚÈÛÌÔ‡ ·ÔÙÂÏÔ‡Ó ÚfiÎÏËÛË ÁÈ· ÙÔ˘˜ ÓÂÔÁÓÔÏfiÁÔ˘˜. ∏ ÛˆÛÙ‹ ÂÈÏÔÁ‹ ÙÔ˘ ÌÔÓÙ¤ÏÔ˘ ı· Ú¤ÂÈ Ó· Â›Ó·È ÂÍ·ÙÔÌÈÎÂ˘Ì¤ÓË ÁÈ· οı ÓÂÔÁÓfi. ∏ ·Ó¿Ù˘ÍË Ù˘ Ù¯ÓÔÏÔÁ›·˜ Û·ÊÒ˜ Â›Ó·È ·ÏÌ·Ù҉˘, ‰ÂÓ ı· Ú¤ÂÈ fï˜ Ó· ·Ú·‚ϤÂÙ·È Ë ÎÏÈÓÈ΋ ÂÎÙ›ÌËÛË Î·È Ë ÂÌÂÈÚ›· ÛÙË ¯Ú‹ÛË ÂÓfi˜ Ì˯·Ó‹Ì·ÙÔ˜, ÁÈ·Ù› ·ÏÔ‡Ûٷٷ ¤Ó· Û‡Á¯ÚÔÓÔ Ì˯¿ÓËÌ· Èı·ÓÒ˜ Ó· Â›Ó·È ÂÈΛӉ˘ÓÔ ÁÈ· ÙÔÓ ·ÛıÂÓ‹, Â¿Ó ‰ÂÓ ¤¯Ô˘Ì ηٷÓÔ‹ÛÂÈ Ï‹Úˆ˜ ÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘.
˙. ÕÏÏÔÈ Ì˯·ÓÈÛÌÔ› ·ÓÙ·ÏÏ·Á‹˜ ·ÂÚ›ˆÓ Ÿˆ˜ ÛÙÔÓ ™∞, ¤ÙÛÈ Î·È ÛÙÔÓ À∞∆, Ë ÌÔÚȷ΋ ‰È¿¯˘ÛË (molecular diffusion) Û˘ÌÌÂÙ¤¯ÂÈ ÛÙËÓ ·Ó¿ÌÈÍË ÙˆÓ ·ÂÚ›ˆÓ, ·ÎÚÈ‚Ò˜ ‰›Ï· ·fi ÙËÓ Î˘„ÂÏȉÈ΋ ÂÈÊ¿ÓÂÈ·. ∂›Û˘, ¤Ó· ÌÈÎÚfi ÙÌ‹Ì· ÙˆÓ ÏËÛȤÛÙÂÚˆÓ ÛÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ ΢„ÂÏ›‰ˆÓ ·ÂÚ›˙ÂÙ·È ·Â˘ı›·˜ (direct alveolar ventilation). ŒÙÛÈ, Ë ·ÓÙ·ÏÏ·Á‹ ·ÂÚ›ˆÓ Á›ÓÂÙ·È fiˆ˜ Î·È ÛÙÔÓ ™∞.
µÈ‚ÏÈÔÁÚ·Ê›·
™ÙÚ·ÙËÁÈΤ˜ ·ÂÚÈÛÌÔ‡ ∏ ÚÔÙÂÈÓfiÌÂÓË ÛÙÚ·ÙËÁÈ΋ ¯Ú‹Û˘ ÙÔ˘ À∞∆ ÛÙËÚ›˙ÂÙ·È ÛÙË ‰È·Ù‹ÚËÛË ÙÔ˘ “‚¤ÏÙÈÛÙÔ˘” fiÁÎÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ Û ÛÙÂÓ¿ Ï·›ÛÈ·, ·Ó¿ÌÂÛ· ÛÙËÓ ·ÔÊ˘Á‹ ·ÙÂÏÂÎÙ·Û›·˜ Î·È ˘ÂډȿٷÛ˘, ÚÔÎÂÈ̤ÓÔ˘ Ó· “·Ô‰ÂÛÌ¢Ù›” Û‡ÓÙÔÌ· ÙÔ ÓÂÔÁÓfi ·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi (9,29,30). ÃÚËÛÈÌÔÔÈÒÓÙ·˜ ÙËÓ Ù·ÎÙÈ΋ ÙÔ˘ “˘„ËÏÔ‡ fiÁÎÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ”, ÂÊ·ÚÌfi˙ÂÙ·È Û˘Ó¯‹˜ ‰È·Ù·ÙÈ΋ ›ÂÛË, ˘„ËÏfiÙÂÚË ·fi ÙËÓ ›ÂÛË ‰È¿ÓÔÈ͢ ÙˆÓ Î˘„ÂÏ›‰ˆÓ, Ì ·ÔÙ¤ÏÂÛÌ· ÙË ÛÙÚ·ÙÔÏfiÁËÛË ÙˆÓ ·ÙÂÏÂÎÙ·ÙÈÎÒÓ Î˘„ÂÏ›‰ˆÓ Î·È ÙËÓ Î·Ï‡ÙÂÚË ¤ÎÙ˘ÍË ÙˆÓ Ó¢ÌfiÓˆÓ (31). ∞˘Ù¤˜ ÔÈ Ô‰ËÁ›Â˜ ·Â˘ı‡ÓÔÓÙ·È ÛÙÔ ÚfiˆÚÔ ÓÂÔÁÓfi Ô˘ ¿Û¯ÂÈ Î˘Ú›ˆ˜ ·fi ÙËÓ ·ÙÂÏÂÎÙ·ÙÈ΋ ÓfiÛÔ ÙˆÓ Ó¢ÌfiÓˆÓ. √È Ô‰ËÁ›Â˜ fï˜, Ú¤ÂÈ Ó· ÂÍ·ÙÔÌÈ·ÔÓÙ·È Î·È Ó· ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È ·Ó¿ÏÔÁ· Ì ÙËÓ ÚˆÙÔ·ı‹ Ó¢ÌÔÓÈ΋ ÓfiÛÔ (21,32-34). ∂›Ó·È ÛËÌ·ÓÙÈÎfi Ô ÓÂÔÁÓÔÏfiÁÔ˜ Ó· ·ÍÈÔÏÔÁ› ÚÔÛÂÎÙÈο ÙËÓ Ó¢ÌÔÓÈ΋ ·ıÔÊ˘ÛÈÔÏÔÁ›· οı ·ÛıÂÓ‹ Î·È Ó· ηıÔÚ›˙ÂÈ ÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ Ô˘ ¢ı‡ÓÔÓÙ·È ÁÈ· ÙË ÌË Â·Ú΋ ·ÓÙ·ÏÏ·Á‹ ÙˆÓ ·ÂÚ›ˆÓ. ªfiÓÔ ÙfiÙ ÌÔÚ› Ó· ۯ‰ȷÛÙ› Ë Î·Ï‡ÙÂÚË ÛÙÚ·ÙËÁÈ΋ ·ÂÚÈÛÌÔ‡ Ô˘ ı· Â›Ó·È Ë Î·Ù·ÏÏËÏfiÙÂÚË ÁÈ· οı ÓÂÔÁÓfi. ∏ ÔÚ›· Ù˘ ÓfiÛÔ˘ Ú¤ÂÈ Ó· ·ӷÍÈÔÏÔÁÂ›Ù·È Û˘¯Ó¿ Î·È Ó· ÙÚÔÔÔÈÂ›Ù·È ·Ó·ÏfiÁˆ˜ Ë ÛÙÚ·ÙËÁÈ΋ ÙÔ˘ ·ÂÚÈÛÌÔ‡ (34). ™˘Ì¤Ú·ÛÌ· ∏ ηχÙÂÚË Î·Ù·ÓfiËÛË ÙÔ˘ ÙÚfiÔ˘ Î·È ÙˆÓ Ì˯·ÓÈÛÌÒÓ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ˘„›Û˘¯ÓÔ˘ ·ÂÚÈÛÌÔ‡ ηPaediatriki 2007;70:18-23
1. Boros SJ, Mammel MC, Coleman JM, Lewallen PK, Gordon MJ, Bing DR, et al. Neonatal high-frequency jet ventilation: four years’ experience. Pediatrics 1985;75:657-663. 2. Roithmair A, Hultzsch W, Lipowsky G, Reinhardt D. Airway pressure measurements during high-frequency positive pressure ventilation in extremely low birth weight neonates. Crit Care Med 1994;22 (9 Suppl):S71-S76. 3. Truog WE. High Frequency Oscillatory Ventilation. In: Spitzer AR, editor. Intensive Care of Fetus and Neonate. St Louis, MO: Mosby - Yearbook Publishing Company; 1996. p. 584-593. 4. Henderson Y, Chillingworth FP, Whitney JL. The respiratory dead space. Am J Physiol 1915;38:1-19. 5. Lunkenheimer PP, Rafflenbeul W, Keller H, Frank I, Dickhut HH, Fuhrmann C. Application of transtracheal pressure oscillations as a modification of “diffusing respiration”. Br J Anaesth 1972;44:627. 6. Drazen JM, Kamm RD, Slutsky AS. High frequency ventilation. Physiol Rev 1984;64:505-543. 7. Boynton BR, Vilanueva D, Hammond MD, Vreeland PN, Buckley B, Frantz ID 3rd. Effect of mean airway pressure on gas exchange during high-frequency oscillatory ventilation. J Appl Physiol 1991; 70:701-707. 8. Marchak BE, Thompson WK, Duffy P, Miyaki T, Bryan MH, Bryan AC, et al. Treatment of RDS by high-frequency oscillatory ventilation: a preliminary report. J Pediatr 1981;99:287-292. 9. Delemos RA, Coalson JJ, Gerstmann DR, Null DM Jr, Ackerman NB, Escobedo MB, et al. Ventilatory management of infant baboons with hyaline membrane disease: the use of high frequency ventilation. Pediatr Res 1987; 21:594-602. 10. Meredith KS, deLemos RA, Coalson JJ, King RJ, Gerstmann DR, Kumar R, et al. Role of lung injury in the pathogenesis of hyaline membrane disease in premature baboons. J Appl Physiol 1989;66:2150-2158. 11. Kinsella JP, Gerstmann DR, Clark RH, Null DM Jr, Morrow WR, Taylor AF, et al. High-frequency oscillatory ventilation versus intermittent mandatory ventilation: early hemodynamic effects in the premature baboon with hyaline membrane disease. Pediatr Res 1991;29:160-166. 12. Simma B, Luz G, Trawoger R, Hormann C, Klima G, Kreczy A, et al. Comparison of different modes of highfrequency ventilation in surfactant-deficient rabbits. Pediatr Pulmonol 1996;22:263-270.
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13. Clark RH, Gerstmann DR, Jobe AH, Moffitt ST, Slutsky AS, Yoder BA. Lung injury in neonates: causes, strategies for prevention, and long-term consequences. J Pediatr 2001;139:478-486. 14. Clark RH, Gerstmann DR. High-Frequency Oscillatory Ventilation. In: Sinha SK and Donn SM, editors. Manual of Neonatal Respiratory Care, 2nd ed. Mosby Inc; 2006. p. 237-246. 15. Smith RB. Ventilation at high respiratory frequencies. High frequency positive pressure ventilation, high frequency jet ventilation and high frequency oscillation. Anaesthesia 1982;37:1011-1018. 16. Butler WJ, Bohn DJ, Bryan AC, Froese AB. Ventilation by high-frequency oscillation in humans. Anesth Analg 1980;59:577-584. 17. Mammel MC, Boros SJ. High-frequency ventilation. In: Goldsmith JP, Karotkin EH, editors. Assisted Ventilation of the Neonate, 3rd ed. Philadelphia: W.B. Saunders Company; 1996. p. 199-214. 18. Venegas JG, Custer J, Kamm RD, et al: Relationship for gas transport during high-frequency ventilation in dogs. J Appl Phsyiol 1985;59:1539-1547. 19. Venegas JG, Gredberg JJ. Understanding the pressure cost of ventilation: why does high-frequency ventilation work? Crit Care Med 1994;22 (9 Suppl):S49-S57. 20. Venegas JG, Hales CA, Strieder DJ. A general dimensionless equation of gas transport by high-frequency ventilation. J Appl Physiol 1986;60:1025-1030. 21. Durand DJ, Asselin JM. Physiology of High-Frequency Ventilation. In: Polin RA, Fox WW, editors. Philadelphia: Fetal and Neonatal Physiology, 2nd ed. WB Saunders Company; 1998. p. 1212-1218. 22. Chang HK. Mechanisms of gas transport during ventilation by high-frequency oscillation. J Appl Physiol 1984;56:553-563. 23. Durand DJ, Asselin JM. SensorMedics 3100a High-Frequency Oscillatory Ventilator. In: Sinha SK and Donn SM,
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¶·È‰È·ÙÚÈ΋ 2007;70:18-23
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ORIGINAL ARTICLE
ªÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ù˘ ∫Ú‹Ù˘ Û ۯ¤ÛË Ì ÙË ‰È·ÙÚÔÊ‹ ÙÔ˘˜ ¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘, ∆Ì‹Ì· π·ÙÚÈ΋˜, ∆Ô̤·˜ ∫ÔÈÓˆÓÈ΋˜ π·ÙÚÈ΋˜, ∫ÏÈÓÈ΋ ¶ÚÔÏËÙÈ΋˜ π·ÙÚÈ΋˜ Î·È ¢È·ÙÚÔÊ‹˜, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘ AÏÏËÏÔÁÚ·Ê›·: ª·ÓfiÏ˘ §ÈÓ·Ú‰¿Î˘ linman@med.uoc.gr ¢È·ÛÙ·‡ÚˆÛË µÔ‡Ù˜ ™Ù·˘Ú¿ÎÈ· ∆.∫. 710 03, ∆.£.: 2208, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘
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Ã. ÷Ù˙‹˜, ™. ºÏÔ˘Ú‹, π. ªÔÛ¯·Ó‰Ú¤·, º. ªÂÚ‚·Ó¿ÎË, ª. ∫˘Úȷο΢, °. ∆ÛÈÌÈÓfi˜, C. Codrington, °. ª·Ì·Ï¿ÎȘ
ª. §ÈÓ·Ú‰¿Î˘, ∫. ™·ÚÚ‹, °. ªÂÚÙÛÈ¿˜, ∞. ∫·Ê¿ÙÔ˜, √Ì¿‰· ∫ÏÈÓÈ΋˜ ¶ÚÔÏËÙÈ΋˜ π·ÙÚÈ΋˜ Î·È ¢È·ÙÚÔÊ‹˜* ¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ Â›Ó·È Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù˘ Û˘¯ÓfiÙËÙ·˜ ÂÌÊ¿ÓÈÛ˘ ÙÔ˘ ªÂÙ·‚ÔÏÈÎÔ‡ ™˘Ó‰ÚfiÌÔ˘ (ªÂÙ™˘Ó) Û ·È‰È¿ & ÂÊ‹‚Ô˘˜ Ù˘ ∫Ú‹Ù˘ Û ۯ¤ÛË Ì ÙË ‰È·ÙÚÔÊ‹ ÙÔ˘˜. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ∂ÈϤ¯ıËÎ·Ó 1209 ·È‰È¿ Î·È ¤ÊË‚ÔÈ. ÀÔÏÔÁ›ÛÙËÎÂ Ë Û˘¯ÓfiÙËÙ· Û˘Ó‡·Ú͢ ÙÚÈÒÓ ‹ ÂÚÈÛÛÔÙ¤ÚˆÓ ·Ú·ÁfiÓÙˆÓ ÙÔ˘ ªÂÙ™˘Ó Î·È Û˘Û¯ÂÙ›ÛÙËΠ̠ÙÔÓ ¢Â›ÎÙË ÀÁÈÂÈÓ‹˜ ¢È·ÙÚÔÊ‹˜ (¢À¢), Ô ÔÔ›Ô˜ ·ÍÈÔÏÔÁ› Î·È Ù·ÍÈÓÔÌ› ÙË ‰È·ÙÚÔÊ‹ οı ·ÙfiÌÔ˘ Û Îϛ̷η ·fi 1-100. ¢È·ÙÚÔÊ‹ Ì ‚·ıÌÔÏÔÁ›· <51 ıˆÚÂ›Ù·È “·Ó·Ú΋˜”, ·fi 51-80 fiÙÈ “¯ÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË” Î·È ·fi 81-100 “ηϋ”. ∞ÔÙÂϤÛÌ·Ù·: √ ÂÈÔÏ·ÛÌfi˜ ÙÔ˘ ªÂÙ™˘Ó ‚Ú¤ıËΠ3,9% (4,2% ÛÙ· ·ÁfiÚÈ·, 3,6% ÛÙ· ÎÔÚ›ÙÛÈ·). ∏ ̤ÛË ‚·ıÌÔÏÔÁ›· ÙÔ˘ ¢À¢ ‹Ù·Ó 58,7 Ì 58,4 ÛÙ· ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ªÂÙ™˘Ó ¤Ó·ÓÙÈ 59,8 Û ÂΛӷ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó (p>0,05). ∆Ô 36% ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ì ªÂÙ™˘Ó, ›¯Â ‰È·ÙÚÔÊ‹ “·Ó·Ú΋” Î·È 5% “ηϋ”, ¤Ó·ÓÙÈ 28% Î·È 8% ·ÓÙ›ÛÙÔȯ·, ÂÎÂ›ÓˆÓ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó (p=0,026). ∂›Û˘, Ù· ·È‰È¿ - ¤ÊË‚ÔÈ Ì ªÂÙ™˘Ó, ¤Ó·ÓÙÈ ÂÎÂ›ÓˆÓ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó, ›¯·Ó ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ Ì ·Ó·Ú΋ Ï‹„Ë Ê˘ÏÈÎÔ‡ ÔͤԘ (55% ¤Ó·ÓÙÈ 53%, p=0,010) Î·È ‚ÈÙ·Ì›Ó˘ ∞ (45% ¤Ó·ÓÙÈ 43%, p=0,023). ™Ù· ·¯‡Û·Úη ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜, Ô Î›Ó‰˘ÓÔ˜ Ó· ·Ó·Ù‡ÍÔ˘Ó ªÂÙ™˘Ó ‚Ú¤ıËΠ85 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚÔ˜ Û ۯ¤ÛË Ì ٷ Ê˘ÛÈÔÏÔÁÈο (95% ¢∂: 33-219, p<0,001). ∆· ·È‰È¿ Î·È ¤ÊË‚ÔÈ Ì ªÂÙ™˘Ó ›¯·Ó ›Û˘ 30% ¯·ÌËÏfiÙÂÚË Èı·ÓfiÙËÙ· ÁÈ· ηϋ ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·ÓÙÔ¯‹ Û ۯ¤ÛË Ì ÂΛӷ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó (OR=0,70, 95% ¢∂: 0,49-0,99, p=0,042). ™˘ÌÂÚ¿ÛÌ·Ù·: ∞fi Ù· ·ÔÙÂϤÛÌ·Ù· ‰È·ÈÛÙÒÓÂÙ·È fiÙÈ Ë ÚˆÙÔÁÂÓ‹˜ ÚfiÏË„Ë Î·È ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ªÂÙ™˘Ó ÌÔÚ› Ó· ÂÈÙ¢¯ı› ÌfiÓÔ Ì ηٿÏÏËÏË ·ÁˆÁ‹ ˘Á›·˜ ·Â˘ı˘ÓfiÌÂÓË ÛÙ· ·È‰È¿ Î·È ÛÙȘ ÔÈÎÔÁ¤ÓÂȤ˜ ÙÔ˘˜, ¤ÙÛÈ ÒÛÙ ӷ ÙÔ˘˜ ‰Ôı› Ë ‰˘Ó·ÙfiÙËÙ· Ó· οÓÔ˘Ó Î·Ï‡ÙÂÚ˜ ÂÈÏÔÁ¤˜ ÛÙË ‰È·ÙÚÔÊ‹, ÛÙË ÛˆÌ·ÙÈ΋ ¿ÛÎËÛË Î·È ÁÂÓÈÎfiÙÂÚ· ÛÙÔÓ ÙÚfiÔ ˙ˆ‹˜ ÙÔ˘˜.
§¤ÍÂȘ ÎÏÂȉȿ: ªÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ, ‰È·ÙÚÔÊ‹, ‰Â›ÎÙ˘ ˘ÁÈÂÈÓ‹˜ ‰È·ÙÚÔÊ‹˜, ·¯˘Û·ÚΛ·, ÏÈÔÚˆÙ½Ó˜.
Prevalence of metabolic syndrome in children and adolescents of Crete and its relation to diet University of Crete, Faculty of Medicine, Department of Social Medicine, Division of Preventive Medicine and Nutrition, Heraklion, Crete Correspondence: Manolis Linardakis linman@med.uoc.gr Voutes-Stavrakia Junction, 71 003, Po. Box: 2208, Herakleion, Crete
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H. Hatzis, S. Flouri, I. Moschandrea, F. Bervanaki, M. Kiriakakis, G. Tsibinos, C. Codrington, G. Mamalakis
M. Linardakis, K. Sarri, G. Bertsias, A. Kafatos, Division of Preventive Medicine and Nutrition* Abstract Background: The aim of this study was to determine the prevalence of the metabolic syndrome (MetSyn) in children and adolescents of Crete, and to explore its association with dietary intake. Methods: The study included 1209 children and adolescents. The prevalence of MetSyn was examined and associated with the USDA Healthy Eating Index (HEI), which classifies the dietary intake of individuals, providing a score of 1-100. The scores indicate respectively: <51 a “poor” diet, 51-80 a diet that “needs improvement”, and >80 a “good” diet. Results: The rate of MetSyn in the children and adolescents was 3.9% (4.2% of boys, 3.6% of girls). The overall mean HEI score was 58.7 (58.4 in children and adolescents with MetSyn and 59.8 in risk-free individuals, p>0.05). Of the children and adolescents with MetSyn, 36% had a “poor” diet and only 5% had a “good” diet according to the HEI score, while the respective rates in risk-free individuals were 28% and 8% (p=0.026). Inadequate intakes of folate (55% vs 53%, p=0.010) and vitamin A (45% vs 43%, p=0.023) were also more common in children and adolescents with MetSyn. Compared to their non-obese counterparts, obese children and adolescents had an 85 times higher risk for MetSyn (95% CI: 33-219, p<0.001). The children and adolescents with MetSyn were 30% less likely to have good cardiorespiratory fitness (OR=0.70, 95% CI: 0.49-0.99, p=0.042) than those with no risk factors. Conclusions: Primary prevention of MetSyn could be achieved when appropriate health and nutrition intervention is initiated early in childhood, aimed at behaviour modification in children, parents and teachers, for better health choices in nutrition and physical activity.
Key words: Metabolic syndrome, nutrition, healthy eating index, obesity, serum lipids. Paediatriki 2007;70:24-36
25
ªÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜
™˘ÓÙÔÌÔÁڷʛ˜ ªÂÙ™˘Ó ¢ª™ HDL-C
ªÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ ¢Â›ÎÙ˘ Ì¿˙·˜ ÛÒÌ·ÙÔ˜ ÃÔÏËÛÙÂÚfiÏË ÙˆÓ ˘„ËÏ‹˜ ˘ÎÓfiÙËÙ·˜ ÏÈÔÚˆÙÂ˚ÓÒÓ LDL-C ÃÔÏËÛÙÂÚfiÏË ÙˆÓ ¯·ÌËÏ‹˜ ˘ÎÓfiÙËÙ·˜ ÏÈÔÚˆÙÂ˚ÓÒÓ ¢À¢ ¢Â›ÎÙ˘ ˘ÁÈÂÈÓ‹˜ ‰È·ÙÚÔÊ‹˜ USDA United States Department of Agriculture ∏™¶ ∏ÌÂÚ‹ÛÈ· Û˘ÓÈÛÙÒÌÂÓË ÔÛfiÙËÙ· RDA Recommended dietary allowances ª™ ™Ù·ÙÈÛÙÈÎÒ˜ ÌË ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ANCOVA ∞Ó¿Ï˘ÛË Û˘Ó‰È·Î‡Ì·ÓÛ˘ OR Odds ratio NHANES III 3rd National Health and Nutrition Examination Survey
∂ÈÛ·ÁˆÁ‹ ŒÓ· ·fi Ù· ÌÂÁ·Ï‡ÙÂÚ· Û‡Á¯ÚÔÓ· ÚÔ‚Ï‹Ì·Ù· Ù˘ ‰ËÌfiÛÈ·˜ ˘Á›·˜ ·ÔÙÂÏ› ÙÔ ÌÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ (ªÂÙ™˘Ó) ‹ Û‡Ó‰ÚÔÌÔ Ã, ·ÊÔ‡ ηıÔÚ›˙ÂÙ·È ·fi ÙË Û˘ÁΤÓÙÚˆÛË ÙÚÈÒÓ ‹ ÂÚÈÛÛfiÙÂÚˆÓ ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Î·Ú‰È·ÁÁÂÈ·ÎÒÓ Î·È ¿ÏÏˆÓ ¯ÚfiÓÈˆÓ ÓÔÛËÌ¿ÙˆÓ (1-3). ™‹ÌÂÚ·, ·Ú¿ ÙÔ˘˜ ‰È·ÊÔÚÂÙÈÎÔ‡˜ ÔÚÈÛÌÔ‡˜ Ô˘ ¤¯Ô˘Ó ‰Ôı›, ¤¯ÂÈ Á›ÓÂÈ ·Ô‰ÂÎÙfi fiÙÈ ªÂÙ™˘Ó ˘Ê›ÛÙ·Ù·È fiÙ·Ó Û˘Ó˘¿Ú¯Ô˘Ó ÙÚÂȘ ‹ ÂÚÈÛÛfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ (2-4), fiˆ˜ ·˘ÍË̤ÓË ÙÈÌ‹ ۷ί¿ÚÔ˘ ÔÚÔ‡ ·›Ì·ÙÔ˜ ˆ˜ ‰Â›ÎÙ˘ ˘ÂÚÁÏ˘Î·ÈÌ›·˜ ‹ ۷ί·ÚÒ‰Ô˘˜ ‰È·‚‹ÙË Ù‡Ô˘ 2, ¯·ÌËÏ‹ HDL ¯ÔÏËÛÙÂÚfiÏË, ˘ÂÚÙÚÈÁÏ˘ÎÂÚȉ·ÈÌ›·, ·˘ÍË̤ÓË Û˘ÛÙÔÏÈ΋ Î·È ‰È·ÛÙÔÏÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË ˆ˜ ‰Â›ÎÙ˜ ˘¤ÚÙ·Û˘ ηÈ, Ù¤ÏÔ˜, ·˘ÍË̤ÓË ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ ˆ˜ ‰Â›ÎÙ˘ ÎÂÓÙÚÈ΋˜ ·¯˘Û·ÚΛ·˜. ∏ Û˘Ó¤ÚÁÂÈ· ÙˆÓ ·Ú·ÁfiÓÙˆÓ ·˘ÙÒÓ Ô‰ËÁ› ÛÙËÓ ·Ó¿Ù˘ÍË ·ıËÚÔÛÎÏËÚˆÙÈÎÒÓ Ï·ÎÒÓ Î·È, ηٿ Û˘Ó¤ÂÈ·, ÛÙÂÊ·ÓÈ·›·˜ ÓfiÛÔ˘ ‹ ÁÂÓÈÎfiÙÂÚ· ηډȷÁÁÂÈ·ÎÒÓ ÓÔÛËÌ¿ÙˆÓ (1-7). ∂ÎÙÈÌ‹ıËΠ‰Â ·fi ÙË ÌÂϤÙË NHANES 1999-2000, fiÙÈ ÂÚ›Ô˘ 47 ÂηÙÔÌ̇ÚÈ· ∞ÌÂÚÈηÓÒÓ ¤¯Ô˘Ó ªÂÙ™˘Ó, ηıÒ˜ Î·È ÂÚÈÛÛfiÙÂÚÔÈ ·fi 2 ÂηÙÔÌ̇ÚÈ· ¤ÊË‚ÔÈ (8-10). ∏ ·ÚÔ˘Û›· ÈÓÒ‰Ô˘˜ Ͽη˜ ÂÍÂÏ›ÛÛÂÙ·È Û ·ıËÚÔÛÎÏ‹ÚˆÛË ·fi ÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ·ÎfiÌ· ËÏÈΛ· (11,12). ∞ÔÙÂÏ› Û˘Ó¤ÂÈ· Û˘Ó‰˘·ÛÌÔ‡ Ù˘ η΋˜ ‰È·ÙÚÔÊ‹˜, Ù˘ ·Ó·ÚÎÔ‡˜ ¿ÛÎËÛ˘ Î·È Ù˘ ·¯˘Û·ÚΛ·˜, Ô˘ fiˆ˜ ·Ó·Ê¤ÚÂÙ·È Û ÌÂϤÙË ÛÙȘ ∏¶∞ (13), ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 300.000 ı·Ó¿ÙÔ˘˜ ÂÙËÛ›ˆ˜ Î·È ÂÚ›Ô˘ 99 ‰ÈÛ. ‰ÔÏ¿ÚÈ· ÂÙ‹ÛÈÔ ÔÈÎÔÓÔÌÈÎfi ÎfiÛÙÔ˜. ∞Ó Î·È Ë ·ıÔÁ¤ÓÂÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ηٷÓÔËı›, Â›Ó·È ÁÓˆÛÙ‹ Ë Û¯¤ÛË ÙÔ˘ Ì ÁÂÓÂÙÈÎÔ‡˜ Î·È ÂÚÈ‚·ÏÏÔÓÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë Î·ıÈÛÙÈ΋ ˙ˆ‹ Î·È Ë ‰˘ÙÈÎÔ‡ Ù‡Ô˘ ‰È·ÙÚÔÊ‹ (14), ÛÙÔȯ›· Ô˘ ·ÔÙÂÏÔ‡Ó Â›Û˘ ‚·ÛÈÎfi ¿ÍÔÓ· Ù˘ Û˘ÓÔÏÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ‹˜ ÙÔ˘ (15).
™ÙË ¯ÒÚ· Ì·˜ ‰ÂÓ ¤¯ÂÈ ·ÎfiÌË ÌÂÏÂÙËı› Ë Û˘¯ÓfiÙËÙ· ÙÔ˘ ªÂÙ™˘Ó ÛÙÔÓ ·È‰ÈÎfi Î·È ÂÊË‚ÈÎfi ÏËı˘ÛÌfi, ηıÒ˜ Î·È Ë Û¯¤ÛË ÙÔ˘ Ì ÙË ‰È·ÙÚÔÊ‹. ∏ ·ÏÏ·Á‹ ÙˆÓ ‰È·ÈÙËÙÈÎÒÓ Û˘ÓËıÂÈÒÓ ÛÙȘ ÙÂÏÂ˘Ù·›Â˜ ‰ÂηÂٛ˜ Î·È ÁÂÓÈÎfiÙÂÚ· ÙÔ˘ ÙÚfiÔ˘ ˙ˆ‹˜ ÙˆÓ Û‡Á¯ÚÔÓˆÓ ∫ÚËÙÈÎÒÓ (16,17), ÙÔÓ›˙ÂÈ ÙÔ È‰È·›ÙÂÚÔ ÂӉȷʤÚÔÓ Ô˘ ¤¯ÂÈ Ë ÌÂϤÙË Ù˘ ·ÚÔ˘Û›·˜ ÙÔ˘ ªÂÙ™˘Ó Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ù˘ ∫Ú‹Ù˘ Î·È Ë Û¯¤ÛË ÙÔ˘ Ì ÙË ‰È·ÙÚÔÊ‹ ÙÔ˘˜. ™ÙËÓ ·ÚÔ‡Û· ¤Ú¢ӷ ÌÂÏÂÙ‹ıËÎÂ Ô ÂÈÔÏ·ÛÌfi˜ ÙÔ˘ ªÂÙ™˘Ó Û ‰Â›ÁÌ· ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ù˘ ∫Ú‹Ù˘ Î·È Ë Û¯¤ÛË ÙÔ˘ Ì ÙË ‰È·ÙÚÔÊ‹ ÙÔ˘˜, ·ÏÏ¿ Î·È Ì ¿ÏÏÔ˘˜ ·Ú¿ÁÔÓÙ˜ fiˆ˜ Â›Ó·È Ë ÛˆÌ·ÙÈ΋ ¿ÛÎËÛË, Ë Û˘ÓÔÏÈ΋ ÏÈȉ·ÈÌÈ΋ ÂÈÎfiÓ·, ÙÔ Î¿ÓÈÛÌ· (ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜), ηıÒ˜ Î·È Ë Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÛÙ· ·¯‡Û·Úη ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∏ ÌÂϤÙË Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ù˘ ∫Ú‹Ù˘ ηٿ Ù· ¤ÙË 2001-2003. ∞Ú¯Èο Û˘ÌÌÂÙ›¯·Ó 1381 ·È‰È¿-¤ÊË‚ÔÈ, ·fi ÙÔ˘˜ ÔÔ›Ô˘˜ 172 ·ÚÓ‹ıËÎ·Ó Ó· Û˘ÌÌÂÙ¿Û¯Ô˘Ó ÛÙȘ ۈ̷ÙÔÌÂÙÚ‹ÛÂȘ ‹ Î·È ÛÙËÓ ·ÈÌÔÏË„›·. ∆ÂÏÈο, 1209 ·È‰È¿-¤ÊË‚ÔÈ (545 ·ÁfiÚÈ· Î·È 677 ÎÔÚ›ÙÛÈ·), ËÏÈΛ·˜ 3 ¤ˆ˜ 17ó ÂÙÒÓ, ›¯·Ó Ï‹ÚË ÛÙÔȯ›· Î·È Û˘ÌÂÚÈÏ‹ÊıËÛ·Ó ÛÙȘ ·Ó·Ï‡ÛÂȘ. ∂›¯·Ó Û˘ÌÌÂÙ¿Û¯ÂÈ Û ÌÂϤÙ˜ Û˘Á¯ÚÔÓÈ΋˜ ‹ Î·È ‰È·¯ÚÔÓÈ΋˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ Ù˘ ∫ÏÈÓÈ΋˜ ¶ÚÔÏËÙÈ΋˜ π·ÙÚÈ΋˜ Î·È ¢È·ÙÚÔÊ‹˜ (¶›Ó·Î·˜ 1) Î·È ÚÔ¤Ú¯ÔÓÙ·È ·fi ¤ÓÙ ‰È·ÊÔÚÂÙÈΤ˜ ÂÚÈÔ¯¤˜ ÙˆÓ ¡ÔÌÒÓ Ã·Ó›ˆÓ Î·È ∏Ú·ÎÏ›Ԣ. ∆ÔÓ π·ÓÔ˘¿ÚÈÔ ÙÔ˘ 2001, Ë ∫ÏÈÓÈ΋ ÍÂΛÓËÛ ÛÙ· Û¯ÔÏ›· Ù˘ ËÌÈÔÚÂÈÓ‹˜ ÂÚÈÔ¯‹˜ ÙÔ˘ ¢‹ÌÔ˘ ƒÔ‡‚· ∏Ú·ÎÏ›Ԣ ¶ÚfiÁÚ·ÌÌ· ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜, ·fi ÙÔ ÔÔ›Ô ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ¤¯Ô˘Ó Û˘ÌÂÚÈÏËÊı› Û˘ÓÔÏÈο 188 Ì·ıËÙ¤˜ (18). ¢Ò‰Âη ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙÔ ÓËÈ·ÁˆÁ›Ô, 108 ·fi Ù· ‰ËÌÔÙÈο Î·È 68 ·fi ÙÔ Á˘ÌÓ¿ÛÈÔ. ∞fi ÙËÓ ÙÂÏÂ˘Ù·›· ·ÍÈÔÏfiÁËÛË (2001-2002) ÂÍ·ÂÙÔ‡˜ ÚÔÁÚ¿ÌÌ·ÙÔ˜ ÂÎ·È‰Â˘ÙÈ΋˜ ·Ú¤Ì‚·Û˘, Ô˘ ¿Ú¯ÈÛ ÛÙËÓ ÚÒÙË Ù¿ÍË ÙˆÓ ‰ËÌÔÙÈÎÒÓ Û¯ÔÏ›ˆÓ Ù˘ ∫Ú‹Ù˘ ÙÔ Û¯ÔÏÈÎfi ¤ÙÔ˜ 1992-1993 (19,20), ÂÈϤ¯ıËÎ·Ó 246 Ì·ıËÙ¤˜ ·fi ÙÔ ¡ÔÌfi ÷ӛˆÓ (ÔÌ¿‰· ÂϤÁ¯Ô˘). ∆ËÓ ¿ÓÔÈÍË ÙÔ˘ 2003, Ë ∫ÏÈÓÈ΋ ÛÂ Û˘ÓÂÚÁ·Û›· Ì ÙÔÓ ¢‹ÌÔ ÃÂÚÛÔÓ‹ÛÔ˘ ¿Ú¯ÈÛ ¶ÚfiÁÚ·ÌÌ· ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜ Î·È ∞Ó¿Ù˘Í˘ ÙˆÓ ·È‰ÈÒÓ Û ÂÙ¿ ÓËÈ·ÁˆÁ›·. ∞fi ·˘Ù¿, Ô ‚ÚÂÊÔÓËÈ·Îfi˜ ÛÙ·ıÌfi˜, ÙÔ 1Ô & 2Ô ¡ËÈ·ÁˆÁÂ›Ô Î·È ÙÔ ¡ËÈ·ÁˆÁÂ›Ô ÃÂÚÛÔÓ‹ÛÔ˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ §È̤ӷ ÃÂÚÛÔÓ‹ÛÔ˘, ÂÓÒ Â›Û˘ Û˘ÌÂÚÈÏ‹ÊıËÎ·Ó Ù· ÓËÈ·ÁˆÁ›· ∫Ô˘ÙÔ˘ÏÔ˘Ê·Ú›Ô˘, ∞Ó¿Ï˄˘ Î·È °ˆÓÈÒÓ ¶Â‰È¿‰Ô˜. ø˜ ÚÒÙË Ê¿ÛË ˘ÏÔÔ›ËÛ˘ ÙÔ˘ ÚÔÁÚ¿ÌÌ·ÙÔ˜ ηıÔÚ›ÛÙËÎÂ Ô ¤ÏÂÁ¯Ô˜ Î·È Ë ÂÎÙ›ÌËÛË ÙÔ˘ ÂȤ‰Ô˘ ˘Á›·˜ ÙˆÓ ·È‰ÈÒÓ, ·fi Ù· ÔÔ›· Û˘ÌÌÂÙ›¯·Ó 89 ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. ¶·ÚfiÌÔÈ·, ÙÔ 2002-2003, ¿Ú¯ÈÛ ¶ÚfiÁÚ·ÌÌ· ÕıÏËÛ˘ Î·È ¢È·ÙÚÔÊ‹˜ Û ۯÔÏ›· Ù˘ ÂÚÈÔ¯‹˜ ¢ÂÈÏÈÓÒÓ ÙÔ˘ ¢‹ÌÔ˘ ∏Ú·ÎÏ›Ԣ, fiÔ˘ Û˘ÌÌÂÙ›¯Â ÙÔ 30fi & 45Ô ¡ËÈ·ÁˆÁ›Ô, ÙÔ 33Ô & 45Ô ¢ËÌÔÙÈÎfi, ÙÔ ∞ıÏËÙÈÎfi 8Ô & 9Ô °˘ÌÓ¿ÛÈÔ Î·È ÙÔ 7Ô & 10Ô §‡ÎÂÈÔ. ∂›Û˘, Û˘ÌÌÂÙ›¯·Ó ÙÔ ¡ËÈ·ÁˆÁ›Ô, ¢ËÌÔÙÈÎfi Î·È °˘ÌÓ¿ÛÈÔ Î·È ÙÔ˘ ¢‹ÌÔ˘ ∆˘Ï›ÛÛÔ˘. ∏ ÚÒÙË Ê¿ÛË ÙÔ˘ ÚÔÁÚ¿ÌÌ·ÙÔ˜ ÔÏÔÎÏËÚÒıËΠÙÔ ‰È¿ÛÙËÌ· ·fi ÙÔÓ ¢ÂΤ̂ÚÈÔ 2002 ¤ˆ˜ ÙÔÓ ∞Ú›ÏÈÔ 2003. ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË Û˘ÌÌÂÙ›¯·Ó Û˘ÓÔÏÈο 686 ·È‰È¿. ¶·È‰È·ÙÚÈ΋ 2007;70:24-36
26
ª. §ÈÓ·Ú‰¿Î˘ Î·È Û˘Ó.
¶›Ó·Î·˜ 1. ¶ÂÚÈÁÚ·ÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ ÌÂϤÙ˘ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ √Ì¿‰Â˜ ÌÂϤÙ˘ ª·ıËÙ¤˜ ¢‹ÌÔ˘ ƒÔ‡‚· ª·ıËÙ¤˜ ÷ӛˆÓ ¡‹È· ¢‹ÌÔ˘ ÃÂÚÛÔÓ‹ÛÔ˘ ¡‹È· Î·È Ì·ıËÙ¤˜ ¢ÂÈÏÈÓÒÓ ∏Ú·ÎÏ›Ԣ & ¢‹ÌÔ˘ ∆˘Ï›ÛÛÔ˘ ™‡ÓÔÏÔ
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192 (94) 303 (68) 110 (63) 779 (67)
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89 103 46 304
99 143 43 382
4-16 14-17 3-7 3-17,5
1381
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3-17ó
* ¶·È‰È¿ Ô˘ Û˘ÌÌÂÙ›¯·Ó ÛÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ÌÂϤÙË Î·È ÔÛÔÛÙfi Û˘ÌÌÂÙÔ¯‹˜ ÙÔ˘˜ (ÛÙÔ Û‡ÓÔÏÔ ÙˆÓ Ì·ıËÙÒÓ ÛÙ· Û¯ÔÏ›· ÙÔ˘˜) ** ¶·È‰È¿ Ô˘ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË
ªÂÙÚ‹ÛÂȘ √È ÌÂÙÚ‹ÛÂȘ Î·È ÔÈ ÎÏÈÓÈΤ˜ ÂÍÂÙ¿ÛÂȘ ·ÊÔÚÔ‡Ó ÛÙËÓ Î·Ù·ÁÚ·Ê‹ Ù˘ Û˘Ó‹ıÂÈ·˜ ηӛÛÌ·ÙÔ˜ (ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜), ÛÙË Ì¤ÙÚËÛË ÙˆÓ ÛˆÌ·ÙÔÌÂÙÚÈÎÒÓ ÌÂÁÂıÒÓ, Ù˘ ·ÚÙËÚȷ΋˜ ›ÂÛ˘, ÙÔ˘ ۷ί¿ÚÔ˘ ÔÚÔ‡ ·›Ì·ÙÔ˜ Î·È ÙˆÓ ÏÈÔÚˆÙÂ˚ÓÒÓ, ÛÙËÓ ·ÍÈÔÏfiÁËÛË Ù˘ Ê˘ÛÈ΋˜ ηٿÛÙ·Û˘ Î·È ÛÙË Ï‹„Ë Î·È Î·Ù·ÁÚ·Ê‹ ‰È·ÈÙËÙÈÎÔ‡ ÈÛÙÔÚÈÎÔ‡. °È· ÙË Û˘ÌÌÂÙÔ¯‹, ÔÈ ÁÔÓ›˜ ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ ˘¤ÁÚ·Ê·Ó Û˘ÌʈÓËÙÈÎfi ·Ô‰Ô¯‹˜, ·ÊÔ‡ ›¯·Ó ÂÓËÌÂÚˆı› ÁÚ·ÙÒ˜ ÁÈ· ÙË ‰È·‰Èηۛ· Î·È ÙÔ Â›‰Ô˜ ÙˆÓ ÌÂÙÚ‹ÛˆÓ. ∏ ÎÏÈÓÈ΋ ÂͤٷÛË ¤ÁÈÓ ÛÙ· ÓËÈ·ÁˆÁ›· Î·È Ù· Û¯ÔÏ›·, ·ÊÔ‡ ÚÔËÁ‹ıËΠۯÂÙÈ΋ ¤ÁÎÚÈÛË ÙˆÓ ¶ÚÔÁÚ·ÌÌ¿ÙˆÓ ∞ÁˆÁ‹˜ Î·È ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜ ·fi ÙÔ ¶·È‰·ÁˆÁÈÎfi πÓÛÙÈÙÔ‡ÙÔ Î·È ÙÔ ÀÔ˘ÚÁÂ›Ô ¶·È‰Â›·˜. °È· fiϘ ÙȘ ÌÂϤÙ˜ ÚÔËÁ‹ıËÎÂ, ›Û˘, ¤ÁÎÚÈÛË ·fi ÙËÓ ÂÈÙÚÔ‹ ∏ıÈ΋˜ Î·È ¢ÂÔÓÙÔÏÔÁ›·˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘. ∂ÚˆÙËÌ·ÙÔÏfiÁÈÔ °È· ÙË Û˘Ó‹ıÂÈ· ÙÔ˘ ηӛÛÌ·ÙÔ˜ ‰fiıËΠÂÚˆÙËÌ·ÙÔÏfiÁÈÔ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜, ÙÔ ÔÔ›Ô Û˘ÌÏ‹ÚˆÓ·Ó ÌfiÓÔÈ ÙÔ˘˜, Û‡Ìʈӷ Ì ÙȘ ··Ú·›ÙËÙ˜ Ô‰ËÁ›Â˜. À‹Ú¯·Ó ¤ÓÙ ÂÚˆÙ‹ÛÂȘ, ·ÓÔÈÎÙÔ‡ Î·È ÎÏÂÈÛÙÔ‡ Ù‡Ô˘, ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·ÍÈÔÈÛÙ›·˜ ÙˆÓ ··ÓÙ‹ÛÂˆÓ (21). ø˜ ηÓÈÛÙ¤˜ ıˆڋıËÎ·Ó fiÛÔÈ Î¿ÓÈ˙·Ó ÂÚÈÛÛfiÙÂÚÔ ·fi ¤Ó· ÙÛÈÁ¿ÚÔ ÙËÓ Ë̤ڷ, ÁÈ· ‰È¿ÛÙËÌ· ÌÂÁ·Ï‡ÙÂÚÔ ÙˆÓ ÙÚÈÒÓ Û˘Ó¯fiÌÂÓˆÓ ÌËÓÒÓ. ™ˆÌ·ÙÔÌÂÙÚ‹ÛÂȘ ™Â 1209 ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ÌÂÙÚ‹ıËΠÙÔ ‚¿ÚÔ˜, ÙÔ ‡„Ô˜ (Û ‡ÙÈ· ı¤ÛË ÁÈ· ·È‰È¿ ËÏÈΛ·˜ <6 ÂÙÒÓ) Î·È Ë ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ Î·È ÏÂοÓ˘, ÂÓÒ ÂÎÙÈÌ‹ıËÎ·Ó ÔÈ ‰Â›ÎÙ˜ ÂÚÈ̤ÙÚÔ˘ ̤Û˘ ÚÔ˜ ÏÂοÓË, ÂÚÈ̤ÙÚÔ˘ ̤Û˘ ÚÔ˜ ‡„Ô˜ Î·È Ô ¢Â›ÎÙ˘ ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™) ˆ˜ ÎÈÏ¿ ÚÔ˜ ÙÔ ‡„Ô˜ Û ̤ÙÚ· ÛÙÔ ÙÂÙÚ¿ÁˆÓÔ (kg/m2). ¶Ï‹Ú˘ ÂÚÈÁÚ·Ê‹ ÙˆÓ ÌÂıfi‰ˆÓ ̤ÙÚËÛ˘ ·Ó·Ê¤ÚÂÙ·È Û ÚÔËÁÔ‡ÌÂÓ˜ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÌÂϤÙ˜ (19,22). °È· ÙÔÓ Î·ıÔÚÈÛÌfi ÙˆÓ ˘¤Ú‚·ÚˆÓ Î·È ·¯‡Û·ÚÎˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ, ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Ô ‰È·¯ˆÚÈÛÌfi˜ ÙÔ˘ ¢ª™, Ô˘ ·ÓÙÈÛÙÔȯ› Û fiÚÈ· ≤25 kg/m2, 25,1 kg/m2 ¤ˆ˜ 30 kg/m2 Î·È ≥30 kg/m2 (23). ª¤ÙÚËÛË ·ÚÙËÚȷ΋˜ ›ÂÛ˘ ·›Ì·ÙÔ˜ ∏ ·ÚÙËÚȷ΋ ›ÂÛË ÌÂÙÚ‹ıËΠ۠1209 ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜, Û ηıÈÛÙ‹ ı¤ÛË Î·È ÌÂÙ¿ ·fi ·Ó¿·˘ÛË 5-10 ÏÂÙÒÓ, Ì ˘‰Ú·ÚÁ˘ÚÈÎfi ÛÊ˘ÁÌÔÌ·ÓfiÌÂÙÚÔ Î·Ù¿ÏÏËÏÔ Û ̤ÁÂıÔ˜ ÂÚȯÂÈÚ›‰Ô˜, ηχÙÔÓÙ·˜ ÙÔ 50-75% Ù˘ ÂÚÈ̤ÙÚÔ˘ ÙÔ˘ ‰ÂÍÈÔ‡ ‚Ú·¯›ÔÓ·. O ÚÒÙÔ˜ Î·È ¤ÌÙÔ˜ ÙfiÓÔ˜ Korotkoff Paediatriki 2007;70:24-36
·ÔÙ¤ÏÂÛ·Ó, ·ÓÙÈÛÙÔ›¯ˆ˜, ÙË Û˘ÛÙÔÏÈ΋ Î·È ‰È·ÛÙÔÏÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË, Ô˘ ÌÂÙÚ‹ıËÎ·Ó ÙÚÂȘ ÊÔÚ¤˜ Ì ÌÂÛԉȿÛÙËÌ· 1-2 ÏÂÙÒÓ Î·È ÂÏ‹ÊıË Ô Ì¤ÛÔ˜ fiÚÔ˜ ÙˆÓ ÌÂÙÚ‹ÛÂˆÓ (ÚˆÙfiÎÔÏÏÔ MONICA) (18). §‹„Ë ·›Ì·ÙÔ˜ Î·È ‚ÈÔ¯ËÌÈΤ˜ ·Ó·Ï‡ÛÂȘ ∞ÈÌÔÏË„›· Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠1209 ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ÙȘ ÚÒÙ˜ ÚˆÈÓ¤˜ ÒÚ˜, Û ‡ÙÈ· ı¤ÛË, ÌÂÙ¿ ·fi 12ˆÚË ÓËÛÙ›·. ∂Ï‹ÊıËÛ·Ó ‰Â›ÁÌ·Ù· ÙˆÓ 10 ml Û ۈÏËÓ¿ÚÈ· ¯ˆÚ›˜ ·ÓÙÈËÎÙÈÎfi ÁÈ· ÙÔÓ ‰È·¯ˆÚÈÛÌfi ÙÔ˘ ÔÚÔ‡ Î·È ÙÔÔıÂÙ‹ıËÎ·Ó Û ÊÔÚËÙfi „˘ÁÂ›Ô Ì ·ÁÔ·ÛÙ˜, ÒÛÙ ӷ ‰È·ÙËÚËıÔ‡Ó Û ıÂÚÌÔÎÚ·Û›· 3-4ÔC. ªÂٷʤÚıËÎ·Ó ÂÓÙfi˜ 2-4 ˆÚÒÓ ÛÙ· ÂÚÁ·ÛÙ‹ÚÈ· Ù˘ ∫ÏÈÓÈ΋˜ Î·È ·Ì¤Ûˆ˜ Ê˘ÁÔÎÂÓÙÚ‹ıËÎ·Ó Û 4000 rpm ÁÈ· 15 ÏÂÙ¿, ÂÓÒ Û ϷÛÙÈο ÛˆÏËÓ¿ÚÈ· Eppendorf ‰È·¯ˆÚ›ÛÙËÎÂ Ô ÔÚfi˜. ¶ÂÚÈÛÛfiÙÂÚ˜ ÏÂÙÔ̤ÚÂȘ ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ ۷ί¿ÚÔ˘ Î·È ÙˆÓ ‚ÈÔ¯ËÌÈÎÒÓ ‰ÂÈÎÙÒÓ (ÔÏÈ΋ ¯ÔÏËÛÙÂÚfiÏË, ÙÚÈÁÏ˘ÎÂÚȉ›·, HDL-C, LDL-C) Î·È ÙˆÓ ·ıËڈ̷ÙÈÎÒÓ ‰ÂÈÎÙÒÓ (ÔÏÈ΋ ¯ÔÏËÛÙÂÚfiÏË ÚÔ˜ HDL-C Î·È LDL-C ÚÔ˜ HDL-C) ·Ó·Ê¤ÚÔÓÙ·È Û ¿ÏϘ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÌÂϤÙ˜ (18-22). ŒÏÂÁ¯Ô˜ Ê˘ÛÈ΋˜ ηٿÛÙ·Û˘ ∏ Ê˘ÛÈ΋ ηٿÛÙ·ÛË Î·È Ë Î·Ú‰ÈÔ·Ó·Ó¢ÛÙÈ΋ ·ÓÙÔ¯‹ ÂϤÁ¯ıËÎ·Ó Û 433 ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ÙÔ ·Ï›Ó‰ÚÔÌÔ ÙÚ¤ÍÈÌÔ ·ÓÙÔ¯‹˜ (20). ∆· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ Û¯ËÌ¿ÙÈ˙·Ó ÔÌ¿‰Â˜ ÙˆÓ 5 ·ÙfiÌˆÓ Î·È Î·ÏÔ‡ÓÙ·Ó Ó· ‰È·Ó‡ÛÔ˘Ó Ì›· ·fiÛÙ·ÛË 20 ̤ÙÚˆÓ ÌÂٷ͇ ‰‡Ô ÁÚ·ÌÌÒÓ, fiÛÔ ÙÔ ‰˘Ó·ÙfiÓ ÈÔ ÔÏϤ˜ ÊÔÚ¤˜. √ Ú˘ıÌfi˜ ÁÈ· ÙËÓ Ù·¯‡ÙËÙ· ‰ÈÓfiÙ·Ó ·fi ¤Ó· Ì·ÁÓËÙÔʈÓË̤ÓÔ Ë¯ËÙÈÎfi Û‹Ì·, ÙÔ˘ ÔÔ›Ô˘ Ô Ú˘ıÌfi˜ ÛÙ·‰È·Î¿ ·˘Í·ÓfiÙ·Ó. ∏ Û˘ÌÏ‹ÚˆÛË Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ·ÚÈıÌÔ‡ ‰È·‰ÚÔÌÒÓ ·ÓÙÈÛÙÔȯԇÛ Û ¤Ó· ÛÙ¿‰ÈÔ. ø˜ ÙÂÏÈ΋ ‚·ıÌÔÏÔÁ›· ηٷÁÚ¿ÊËÎ·Ó Ù· ÛÙ¿‰È· Ô˘ ηٿÊÂÚ·Ó Ó· ‰È·Ó‡ÛÔ˘Ó Ù· ·È‰È¿-¤ÊË‚ÔÈ Û˘Ó¯fiÌÂÓ· Î·È Ì¤Û· ÛÙÔÓ Ú˘ıÌfi Ô˘ ¤‚·Ï ÙÔ Ë¯ËÙÈÎfi Û‹Ì·. ¢È·ÈÙËÙÈÎfi ÈÛÙÔÚÈÎfi ∏ Ï‹„Ë ‰È·ÈÙËÙÈÎÔ‡ ÈÛÙÔÚÈÎÔ‡ ÙÂÏÂ˘Ù·›Ô˘ 24ÒÚÔ˘ ¤ÁÈÓ ÌÂ Û˘Ó¤ÓÙ¢ÍË ·fi ‰È·ÈÙÔÏfiÁÔ Û 1124 ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜. ∫·Ù¿ ÙË ‰È·‰Èηۛ· ÙˆÓ Û˘ÓÂÓه͈Ó, ÁÈ· ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ ÔÛfiÙËÙ·˜ ηٷӿψÛ˘ ÙÚÔÊ›ÌˆÓ (Û ÁÚ·ÌÌ¿ÚÈ·), ·ÚÔ˘ÛÈ¿˙ÔÓÙ·Ó ÌÔÓ٤Ϸ ÙÚÔʛ̈Ó, ÂÓÒ ÁÈ· Ù· ·È‰È¿ ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 8 ÂÙÒÓ, Ë Ï‹„Ë ÙÔ˘ ÈÛÙÔÚÈÎÔ‡ ¤ÁÈÓÂ Î·È Ì ÙË Û˘ÓÂÚÁ·Û›· ÙˆÓ ÁÔÓ¤ˆÓ. ∏ ·Ó¿Ï˘ÛË ÙˆÓ ‰È·ÈÙÔÏÔÁ›ˆÓ ¤ÁÈÓ ·fi ÙËÓ ∏ÏÂÎÙÚÔÓÈ΋ µ¿ÛË ∆ÚÔÊ›ÌˆÓ Ù˘ ∫ÏÈÓÈ΋˜ (24).
27
ªÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜
°È· ÙËÓ ·ÍÈÔÏfiÁËÛË Ù˘ ‰È·ÙÚÔÊ‹˜, ˘ÔÏÔÁ›ÛÙËÎ·Ó Ù· ÚÔÛÏ·Ì‚·ÓfiÌÂÓ· ıÚÂÙÈο Û˘ÛÙ·ÙÈο, ηıÒ˜ Î·È 16 ÔÌ¿‰Â˜ ηٷӿψÛ˘ ÙÚÔʛ̈Ó. ∂›Û˘, ÂÎÙÈÌ‹ıËÎ·Ó Ô ¢Â›ÎÙ˘ ÀÁÈÂÈÓ‹˜ ¢È·ÙÚÔÊ‹˜ (¢À¢) (25,26) Î·È Ù· ÔÛÔÛÙ¿ ·Ó·ÚÎÔ‡˜ ıÚ¤„˘ ·fi ÙȘ ËÌÂÚ‹ÛȘ Û˘ÓÈÛÙÒÌÂÓ˜ ÔÛfiÙËÙ˜ (∏™¶) ÚfiÛÏ˄˘ ıÚÂÙÈÎÒÓ Û˘ÛÙ·ÙÈÎÒÓ. √ ¢À¢ (¶›Ó·Î·˜ 2) ·ÔÙÂÏ› ̤ıÔ‰Ô ÔÈÔÙÈ΋˜ ·ÍÈÔÏfiÁËÛ˘ Ù˘ ËÌÂÚ‹ÛÈ·˜ ‰È·ÙÚÔÊ‹˜ ·ÙfiÌˆÓ ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 2 ÂÙÒÓ Î·È ÂÎÙÈÌ¿Ù·È ·fi ‰È·ÈÙÔÏfiÁÈÔ Î·Ù·ÁÚ·Ê‹˜ ÙÂÏÂ˘Ù·›Ô˘ 24ÒÚÔ˘ (25,26). Œ¯ÂÈ Û¯Â‰È·ÛÙ› ·fi ÙÔ USDA Ì ‚¿ÛË ÙȘ ‰È·ÈÙËÙÈΤ˜ Ô‰ËÁ›Â˜ Ô˘ ·Â˘ı‡ÓÔÓÙ·È ÚÔ˜ ÙÔ˘˜ ∞ÌÂÚÈηÓÔ‡˜ (27), ÂÊ·ÚÌfi˙ÂÙ·È fï˜ Î·È Û ÌÂÛÔÁÂÈ·ÎÔ‡˜ ÏËı˘ÛÌÔ‡˜ (28) Î·È ¤¯ÂÈ Û˘Û¯ÂÙÈÛÙ› Ì ÙÔ˘˜ ‚ÈÔÏÔÁÈÎÔ‡˜ ‰Â›ÎÙ˜ ÙˆÓ ÏÈÔÚˆÙÂ˚ÓÒÓ Î·È ÙËÓ ·¯˘Û·ÚΛ· (29,30). √ ¢À¢ ·ÔÙÂÏ› ‰È·‚¿ıÌÈÛË ‚·ıÌÔÏÔÁ›·˜ Û Îϛ̷η Ì ¯Â›ÚÈÛÙË ÙÈÌ‹ 0 Î·È ¿ÚÈÛÙË 100. ∆È̤˜ <51 ·ÍÈÔÏÔÁÔ‡Ó ÙË ‰È·ÙÚÔÊ‹ ˆ˜ “·Ó·Ú΋”, 51-80 ‰ËÏÒÓÔ˘Ó fiÙÈ “¯ÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË” Î·È ÙÈ̤˜ 81100 ¯·Ú·ÎÙËÚ›˙Ô˘Ó ÙË ‰È·ÙÚÔÊ‹ ˆ˜ “ηϋ”. ∏ ‚·ıÌÔÏÔÁ›· ÚÔ·ÙÂÈ ·fi ÙÔ ¿ıÚÔÈÛÌ· ‰¤Î· Û˘ÓÈÛÙˆÛÒÓ, ÔÈ Ôԛ˜ ¯ˆÚ›˙ÔÓÙ·È Û ÙÚÂȘ ·ÚȘ ÔÌ¿‰Â˜ Î·È ÂÓ¤¯Ô˘Ó ‰È·‚·ıÌÈṲ̂ÓË ‚·ıÌÔÏÔÁ›· Ì ¯Â›ÚÈÛÙË ÙÈÌ‹ 0 Î·È ¿ÚÈÛÙË 10. ∏ ÚÒÙË Î‡ÚÈ· ÔÌ¿‰· ·ÊÔÚ¿ ÛÙËÓ Î·Ù·Ó¿ÏˆÛË ÙÚÔÊ›ÌˆÓ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ ÙȘ Û˘ÓÈÛÙÒÛ˜: ‰ËÌËÙÚȷο („ˆÌ›, Ú‡˙È, ‰ËÌËÙÚȷο, ˙˘Ì·ÚÈο), Ï·¯·ÓÈο, ÊÚÔ‡Ù·, Á·Ï·ÎÙÔÎÔÌÈο (Á¿Ï·, ÁÈ·Ô‡ÚÙÈ, Ù˘Ú›) Î·È ÎÚ¤·˜ (ÎÚ¤·˜, Ô˘ÏÂÚÈο, ÍËÚ¿ Ê·ÛfiÏÈ·, „¿ÚÈ, ·˘Á¿, ÍËÚÔ› ηÚÔ›). ∏ ‰Â‡ÙÂÚË ·ÊÔÚ¿ ÛÙËÓ ÚfiÛÏË„Ë ıÚÂÙÈÎÒÓ Û˘ÛÙ·ÙÈÎÒÓ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ ÙȘ Û˘ÓÈÛÙÒÛ˜: ÔÏÈÎfi Î·È ÎÔÚÂṲ̂ÓÔ Ï›Ô˜ ˆ˜ ÔÛÔÛÙ¿ ÂÓ¤ÚÁÂÈ·˜, ¯ÔÏËÛÙÂÚfiÏË ÙÚÔÊ‹˜ Î·È Ó¿ÙÚÈÔ. ∆¤ÏÔ˜, Ë ÙÚ›ÙË ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ Î·Ù·Ó¿ÏˆÛË ÔÈÎÈÏ›·˜ ÙÚÔÊ›ÌˆÓ Î·È ˘ÔÏÔÁ›˙ÂÙ·È ·fi ‰È·ÊÔÚÂÙÈο ˆ˜ ÚÔ˜ ÙÔ Â›‰Ô˜ ÙÔ˘˜ ÙÚfiÊÈÌ·. ∏ ·fi‰ÔÛË ‚·ıÌÔÏÔÁ›·˜ ·fi 0 ¤ˆ˜ 10 ‰È·Ê¤ÚÂÈ Û οı ̛· Û˘ÓÈÛÙÒÛ· Î·È Â›Ó·È ·Ó¿ÏÔÁË ÙˆÓ ÌÂÚ›‰ˆÓ, Ô˘ ˘ÔÏÔÁ›˙ÔÓÙ·È Û ÁÚ·ÌÌ¿ÚÈ·, ÙˆÓ ÔÚ›ˆÓ ÚfiÛÏ˄˘ ıÚÂÙÈÎÒÓ Û˘ÛÙ·ÙÈÎÒÓ Î·È ÙÔ˘ ·ÚÈıÌÔ‡ ηٷӿψÛ˘ ‰È·ÊÔÚÂÙÈÎÒÓ ÙÚÔÊ›ÌˆÓ ÛÙȘ ÙÚÂȘ ·ÚȘ ÔÌ¿‰Â˜, ·ÓÙÈÛÙÔ›¯ˆ˜. ¶ÈÔ Û˘ÁÎÂÎÚÈ̤ӷ, ÛÙËÓ ÚÒÙË Î‡ÚÈ· ÔÌ¿‰·, Ë ¯Â›ÚÈÛÙË ÙÈÌ‹ 0 ·Ô‰›‰ÂÙ·È ÛÙË ÌˉÂÓÈ΋ ηٷӿψÛË ÌÂÚ›‰ˆÓ. ∆Ô ¿ÚÈÛÙ· 10, ·ÓÙÈÛÙÔȯ› Û ηٷӿψÛË 6-11 ÌÂÚ›‰ˆÓ ‰ËÌËÙÚÈ·ÎÒÓ, 3-5 Ï·¯·ÓÈÎÒÓ, 2-4 ÊÚÔ‡ÙˆÓ, 2-3 Á·Ï·ÎÙÔÎÔÌÈÎÒÓ Î·È 2-3 ÎÚ¤·ÙÔ˜. √È ÌÂÚ›‰Â˜ ·˘Ù¤˜ ηıÔÚ›˙ÔÓÙ·È Î·È Â›Ó·È ·Ó¿ÏÔÁ˜ ·fi ÙËÓ Î·Ù¿ ʇÏÔ Î·È ËÏÈΛ· ËÌÂÚ‹ÛÈ· Û˘ÓÈÛÙÒÌÂÓË ÂÓ¤ÚÁÂÈ· Î·È Î·Ù·Ó¿ÏˆÛË ÙÚÔÊ›ÌˆÓ (¶›Ó·Î·˜ 2) (25). ™ÙË ‰Â‡ÙÂÚË Î‡ÚÈ· ÔÌ¿‰·, ¯Â›ÚÈÛÙË ÙÈÌ‹ 0 ·Ô‰›‰ÂÙ·È ÛÙËÓ ÚfiÛÏË„Ë ÔÏÈÎÔ‡ Ï›Ô˘˜ ≥45% Ù˘ ÚÔÛÏ·Ì‚·ÓfiÌÂÓ˘ ÂÓ¤ÚÁÂÈ·˜, ÎÔÚÂṲ̂ÓÔ˘ Ï›Ô˘˜ ≥15%, ¯ÔÏËÛÙÂÚfiÏ˘ ÙÚÔÊ‹˜ ≥450 mg Î·È Ó·ÙÚ›Ô˘ ≥4800 mg. ÕÚÈÛÙ· 10 ·Ô‰›‰ÂÙ·È ÛÙËÓ ÚfiÛÏË„Ë ÔÏÈÎÔ‡ Ï›Ô˘˜ ≤30% Ù˘ ÂÓ¤ÚÁÂÈ·˜, ÎÔÚÂṲ̂ÓÔ˘ Ï›Ô˘˜ <10%, ¯ÔÏËÛÙÂÚfiÏ˘ ÙÚÔÊ‹˜ ≤300 mg Î·È Ó·ÙÚ›Ô˘ ≤2400 mg. ∆¤ÏÔ˜, ÛÙËÓ ÙÚ›ÙË Î‡ÚÈ· ÔÌ¿‰·, ¯Â›ÚÈÛÙË ÙÈÌ‹ 0 ·Ô‰›‰ÂÙ·È fiÙ·Ó Ë Î·Ù·Ó¿ÏˆÛË ‰ÂÓ ˘ÂÚ‚·›ÓÂÈ Ù· ÙÚ›· ‰È·ÊÔÚÂÙÈο ›‰Ë ÙÚÔʛ̈Ó, ÂÓÒ ¿ÚÈÛÙË 10 fiÙ·Ó Ë Î·Ù·Ó¿ÏˆÛË Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË ‹ ›ÛË ÙˆÓ 8 ‰È·ÊÔÚÂÙÈÎÒÓ ÙÚÔʛ̈Ó. ∫·È ÛÙȘ ‰¤Î· Û˘ÓÈÛÙÒÛ˜ ÙÔ˘ ¢À¢, Ë ‚·ıÌÔÏÔÁ›· ÁÈ· ÙȘ ÂӉȿÌÂÛ˜ ÙÈ̤˜ Ù˘ ¯Â›ÚÈÛÙ˘ Î·È Ù˘ ¿ÚÈÛÙ˘ ˘ÔÏÔÁ›˙ÂÙ·È ·Ó·ÏÔÁÈο. ∆¤ÏÔ˜, ·fi ÙȘ ∏™¶ ÚfiÛÏ˄˘ ÙˆÓ ıÚÂÙÈÎÒÓ Û˘ÛÙ·ÙÈÎÒÓ (31), ˘ÔÏÔÁ›ÛÙËηÓ, ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, Ù· fiÚÈ· ÙÔ˘ 67%, Ô˘ ηıÔÚ›˙Ô˘Ó ÙËÓ ·Ó·Ú΋ ıÚ¤„Ë. ŸÚÈ· ·Ú·ÁfiÓÙˆÓ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó fiÚÈ· ÙˆÓ ·-
Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó Ô˘ ηıÔÚ›˙ÔÓÙ·È ·fi ÙÔ Ê‡ÏÔ Î·È ÙËÓ ËÏÈΛ· (2,32). ∆· fiÚÈ· ÙˆÓ ÙÚÈÁÏ˘ÎÂÚȉ›ˆÓ Î·È Ù˘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘ ·ÊÔÚÔ‡Ó ÙËÓ 90‹ ÂηÙÔÛÙÈ·›· ı¤ÛË Î·È ÙË 10Ë ÁÈ· ÙËÓ HDL-C (32). ∂›Û˘, ÂÂȉ‹ Ë Û˘ÛÙÔÏÈ΋ Î·È ‰È·ÛÙÔÏÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË Û˘ÓÂÎÙÈÌÒÓÙ·È ˆ˜ ¤Ó·˜ ÎÔÈÓfi˜ ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘, ˆ˜ ·ıÔÏÔÁÈ΋ ıˆڋıËÎÂ Ë ˘¤Ú‚·ÛË ÙÔ˘ ÔÚ›Ô˘, ›Ù Ù˘ Û˘ÛÙÔÏÈ΋˜ ›Ù Ù˘ ‰È·ÛÙÔÏÈ΋˜. ∆· fiÚÈ· ·˘Ù¿ ·ÊÔÚÔ‡Û·Ó ÛÙËÓ ÂÈÏÔÁ‹ Ù˘ 90‹˜ ÂηÙÔÛÙÈ·›·˜ ı¤Û˘ Ô˘ ·ÓÙÈÛÙÔȯ› ÛÙËÓ 50‹ ÂηÙÔÛÙÈ·›· ı¤ÛË ÙÔ˘ ·Ó·ÛÙ‹Ì·ÙÔ˜ (2,33). ∆¤ÏÔ˜, ˆ˜ fiÚÈÔ ÁÈ· ÙÔ Û¿Î¯·ÚÔ ÔÚÔ‡ ·›Ì·ÙÔ˜ ηıÔÚ›ÛÙËÎ·Ó Ù· 100 mg/dl (2). ™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË °È· ÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ¯ÚËÛÈÌÔÔÈ‹ıËΠÙÔ ÚfiÁÚ·ÌÌ· SPSS 12.0. ÃÚËÛÈÌÔÔÈ‹ıËÎ·Ó Ì¤ıÔ‰ÔÈ ·Ó¿Ï˘Û˘ Û ÛÙڈ̷ÙÔÔÈË̤ӷ ‰Â‰Ô̤ӷ, ÂϤÁ¯ÔÓÙ·˜ Î·È ÁÈ· ÙËÓ Â›‰Ú·ÛË Û˘Á¯˘ÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (34), ηıÒ˜ ÙÔ ‰Â›ÁÌ· ÌÂϤÙ˘ ‰È·¯ˆÚ›ÛÙËΠ۠ÔÌ¿‰Â˜ ËÏÈÎÈÒÓ, fiˆ˜ 3-6, 6ó-10, 10ó-14 Î·È 14ó-17ó ÂÙÒÓ. ªÂ ÙÔÓ ¤ÏÂÁ¯Ô χ2 (Chi-square test) ÂϤÁ¯ıËÎÂ Ë ‡·ÚÍË ‰È·ÊÔÚ¿˜ ÛÙËÓ Î·Ù·ÓÔÌ‹ ÙÔ˘ ·ÚÈıÌÔ‡ Û˘ÁΤÓÙÚˆÛ˘ ·Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó ˆ˜ ÚÔ˜ ÙÔ Ê‡ÏÔ Î·È ÙȘ ∏™¶ ÚfiÛÏ˄˘ ıÚÂÙÈÎÒÓ Û˘ÛÙ·ÙÈÎÒÓ, ηıÒ˜ Î·È Ë ‡·ÚÍË ÁÚ·ÌÌÈ΋˜ Ù¿Û˘ (linear trend) ˆ˜ ÚÔ˜ ÙȘ ÔÌ¿‰Â˜ ËÏÈÎÈÒÓ Î·È ÙÔÓ ·ÚÈıÌfi Û˘ÁΤÓÙÚˆÛ˘ ·Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó. ªÂ ÙÔÓ ¤ÏÂÁ¯Ô Student t (equal variances not assumed) ÂϤÁ¯ıËÎÂ Ë ‰È·ÊÔÚÔÔ›ËÛË Ù˘ ‚·ıÌÔÏÔÁ›·˜ ÙˆÓ Û˘ÓÈÛÙˆÛÒÓ Î·È ÙÔ˘ ¢À¢ ÛÙ· ‰‡Ô ʇϷ ÌÂٷ͇ ÙˆÓ ËÏÈÎÈÒÓ 3-6 Î·È 14ó-17ó ÂÙÒÓ. ¶·ÚÔÌÔ›ˆ˜, Ì ÙÔÓ ¤ÏÂÁ¯Ô Mann-Whitney ¤ÁÈÓ ۇÁÎÚÈÛË Ù˘ ηٷӿψÛ˘ ÙÚÔÊ›ÌˆÓ ÌÂٷ͇ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó Î·È ÂÎÂ›ÓˆÓ Ì ªÂÙ™˘Ó. ªÂ ÙË Ì¤ıÔ‰Ô ·Ó¿Ï˘Û˘ Û˘Ó‰È·Î‡Ì·ÓÛ˘ (ANCOVA) ÂÎÙÈÌ‹ıËÎÂ Ë ‡·ÚÍË ÁÚ·ÌÌÈ΋˜ Ù¿Û˘ (linear trend) ÛÙËÓ ÚfiÛÏË„Ë ıÚÂÙÈÎÒÓ Û˘ÛÙ·ÙÈÎÒÓ Î·È ÛÙË ‚·ıÌÔÏÔÁ›· ÙÔ˘ ¢À¢ Î·È ÙˆÓ Û˘ÓÈÛÙˆÛÒÓ ÙÔ˘, ˆ˜ ÚÔ˜ ÙÔÓ ·ÚÈıÌfi Û˘ÁΤÓÙÚˆÛ˘ ·Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó. ø˜ Û˘ÌÌÂÙ·‚ÏËÙ¤˜ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÙÔ Ê‡ÏÔ, Ë ËÏÈΛ· Î·È Ô ¢ª™, ÂÓÒ Ë ÂÙÂÚÔÁ¤ÓÂÈ· ÂϤÁ¯ıËΠ̠ÙË Ì¤ıÔ‰Ô Levene (Levene’s test). ∆¤ÏÔ˜, ÁÈ· ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ªÂÙ™˘Ó, ÂÎÙÈÌ‹ıËÎ·Ó Ì ÙË Ì¤ıÔ‰Ô ·Ó¿Ï˘Û˘ ÏÔÁÈÛÙÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘ (Logistic linear regression), ÔÈ ÏfiÁÔÈ Û˘ÌÏËڈ̷ÙÈÎÒÓ Èı·ÓÔÙ‹ÙˆÓ ‹ Û¯ÂÙÈÎÔ› ΛӉ˘ÓÔÈ (Odds Ratio) Î·È Ù· 95% ¢È·ÛÙ‹Ì·Ù· ∂ÌÈÛÙÔÛ‡Ó˘. ∏ ÂÎÙ›ÌËÛË ÔÚ›ÛÙËΠˆ˜ ÚÔ˜ ÂΛӷ Ù· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ Ô˘ ‰ÂÓ ¤¯Ô˘Ó ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó, ÂÓÒ ˆ˜ Û˘ÌÌÂÙ·‚ÏËÙ¤˜ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÙÔ Ê‡ÏÔ Î·È Ë ËÏÈΛ·. ø˜ ÛÙ·ÙÈÛÙÈÎÒ˜ ÌË ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ (ª™) ÔÚ›ÛÙËÎ·Ó Ù· ›‰· Ì p>0,05.
∞ÔÙÂϤÛÌ·Ù· Ÿˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ ¶›Ó·Î· 3, ÛÙË Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ·Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó ‰ÂÓ ‚Ú¤ıËΠÛÙ· ·ÁfiÚÈ· ÛËÌ·ÓÙÈ΋ Ù¿ÛË ·‡ÍËÛ˘ ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ·. ªÂ ªÂÙ™˘Ó ‚Ú¤ıËΠÔÛÔÛÙfi 2,5% ÛÙȘ ËÏÈ˘ 3-6 ÂÙÒÓ Î·È 4,6% ÛÙȘ ËÏÈ˘ 14ó-17ó ÂÙÒÓ, ÂÓÒ ÛÙ· ÎÔÚ›ÙÛÈ· ‹Ù·Ó 2,9% Î·È 3%, ·ÓÙ›ÛÙÔȯ·. ™Ù· ÎÔÚ›ÙÛÈ·, ˆÛÙfiÛÔ, ‹Ù·Ó ÛËÌ·ÓÙÈ΋ Ë ‰È·ÊÔÚÔÔ›ËÛË ÛÙËÓ Î·Ù·ÓÔÌ‹ ÙÔ˘˜ ÛÙȘ ÔÌ¿‰Â˜ ËÏÈÎÈÒÓ ˆ˜ ÚÔ˜ ÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ·Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó (p<0,05). ¶·È‰È·ÙÚÈ΋ 2007;70:24-36
28
ª. §ÈÓ·Ú‰¿Î˘ Î·È Û˘Ó.
¶›Ó·Î·˜ 2. ∫ÚÈÙ‹ÚÈ· Î·È ‚·ıÌÔÏfiÁËÛË Û˘ÓÈÛÙˆÛÒÓ ÙÔ˘ ¢Â›ÎÙË ÀÁÈÂÈÓ‹˜ ¢È·ÙÚÔÊ‹˜ (25) ∫ÚÈÙ‹ÚÈ· ‚·ıÌÔÏfiÁËÛ˘ ∂Ï¿¯ÈÛÙË (Ìˉ¤Ó) ª¤ÁÈÛÙË (‰¤Î·) 1. √Ì¿‰· ‰ËÌËÙÚÈ·ÎÒÓ („ˆÌ›, Ú‡˙È, ‰ËÌËÙÚȷο, ˙˘Ì·ÚÈο) 2. §·¯·ÓÈο 3. ºÚÔ‡Ù· 4. √Ì¿‰· Á·Ï·ÎÙÔÎÔÌÈÎÒÓ (Á¿Ï·, ÁÈ·Ô‡ÚÙÈ, Ù˘Ú›) 5. √Ì¿‰· ÎÚ¤·ÙÔ˜ (ÎÚ¤·˜, Ô˘ÏÂÚÈο, ÍËÚ¿ Ê·ÛfiÏÈ·, „¿ÚÈ, ·˘Á¿, ÍËÚÔ› ηÚÔ›) 6. √ÏÈÎfi ϛԘ (% ÂÓ¤ÚÁÂÈ·˜) 7. ∫ÔÚÂṲ̂ÓÔ Ï›Ô˜ (% ÂÓ¤ÚÁÂÈ·˜) 8. ÃÔÏËÛÙÂÚfiÏË ÙÚÔÊ‹˜ 9. ¡¿ÙÚÈÔ 10. ¶ÔÈÎÈÏ›· ÙÚÔʛ̈Ó
0 ÌÂÚ›‰Â˜
6-11 ÌÂÚ›‰Â˜
0 ÌÂÚ›‰Â˜ 0 ÌÂÚ›‰Â˜ 0 ÌÂÚ›‰Â˜
3-5 ÌÂÚ›‰Â˜ 2-4 ÌÂÚ›‰Â˜ 2-3 ÌÂÚ›‰Â˜
0 ÌÂÚ›‰Â˜
2-3 ÌÂÚ›‰Â˜
≥45% Ù˘ ÂÓ¤ÚÁÂÈ·˜
≤30% Ù˘ ÂÓ¤ÚÁÂÈ·˜
≥15% Ù˘ ÂÓ¤ÚÁÂÈ·˜
<10% Ù˘ ÂÓ¤ÚÁÂÈ·˜
≥450 mg ≥4800 mg ≤3 ‰È·ÊÔÚÂÙÈο ›‰Ë ÙÚÔʛ̈Ó
≤300 mg ≤2400 mg ≥8 ‰È·ÊÔÚÂÙÈο ›‰Ë ÙÚÔʛ̈Ó
[
™˘ÓÈÛÙÒÌÂÓË ÂÓ¤ÚÁÂÈ· & Û˘ÓÈÛÙÒÌÂÓ˜ ÌÂÚ›‰Â˜ ÙÚÔÊ›ÌˆÓ ·Ó¿ Ë̤ڷ (USDA, 1996) ∏ÏÈΛ·
∂Ó¤ÚÁÂÈ· (kcal)
¢ËÌËÙÚȷο
§·¯·ÓÈο
ºÚÔ‡Ù·
°·Ï·ÎÙÔÎÔÌÈο
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∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
2-3 4-6 7-10 11-14 15-18 11-24
1300 1800 2000 2500 3000 2200
6 7 7,8 9,9 11 9
3 3,3 3,7 4,5 5 4
2 2,3 2,7 3,5 4 3
2 2 2 3 3 3
2 2,1 2,3 2,6 2,8 2,4
[ ÕıÚÔÈÛÌ· 10 Û˘ÓÈÛÙˆÛÒÓ Î·È Ù·ÍÈÓfiÌËÛË ¢Â›ÎÙ˘ ÀÁÈÂÈÓ‹˜ ¢È·ÙÚÔÊ‹˜
<51
51-80
>80
∞Ó·Ú΋˜
ÃÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË
∫·Ï‹
* √È Û˘ÓÈÛÙÒÛ˜ Î·È Ô ¢Â›ÎÙ˘ ÀÁÈÂÈÓ‹˜ ¢È·ÙÚÔÊ‹˜ ÂÎÙÈÌÒÓÙ·È ·fi ‰È·ÈÙÔÏfiÁÈÔ 24ÒÚÔ˘ ** ∏ ‚·ıÌÔÏfiÁËÛË ÌÂٷ͇ Ù˘ ÂÏ¿¯ÈÛÙ˘ Î·È Ì¤ÁÈÛÙ˘ ÙÈÌ‹˜ (0 ¤ˆ˜ 10) ÂÎÙÈÌ¿Ù·È ·Ó·ÏÔÁÈο
™˘ÓÔÏÈο, Ô ÂÈÔÏ·ÛÌfi˜ ÙÔ˘ ªÂÙ™˘Ó ‹Ù·Ó 3,9% ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ (4,2% ÛÙ· ·ÁfiÚÈ· Î·È 3,9% ÛÙ· ÎÔÚ›ÙÛÈ·, ª™). ™‡Ìʈӷ Ì ÙÔÓ ¶›Ó·Î· 4, ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ‚Ú¤ıËÎ·Ó ÛÙ· ‰‡Ô ʇϷ, ÌÂٷ͇ ÙˆÓ ËÏÈÎÈÒÓ 3-6 Î·È 14ó-17ó ÂÙÒÓ, ÛÙË ‚·ıÌÔÏÔÁ›· ÚfiÛÏ˄˘ ÎÔÚÂṲ̂ÓÔ˘ Ï›Ô˘˜ (·ÁfiÚÈ·: 2,0 ¤Ó·ÓÙÈ 3,9, p<0,001, ÎÔÚ›ÙÛÈ·: 2,2 ¤Ó·ÓÙÈ 3,9, p<0,001), Ó·ÙÚ›Ô˘ (·ÁfiÚÈ·: 9,9 ¤Ó·ÓÙÈ 7,8, p<0,001, ÎÔÚ›ÙÛÈ·: 9,8 ¤Ó·ÓÙÈ 9,2, p<0,001) Î·È ÛÙÔÓ ¢À¢ (·ÁfiÚÈ·: 62,8 ¤Ó·ÓÙÈ 57,8, p<0,01, ÎÔÚ›ÙÛÈ·: 61,5 ¤Ó·ÓÙÈ 57,5, p<0,05). ∂›Û˘, ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚË ‚·ıÌÔÏÔÁ›· ‚Ú¤ıËΠÛÙ· ·ÁfiÚÈ· ÌÂٷ͇ ÙˆÓ ËÏÈÎÈÒÓ 3-6 Î·È 14ó-17ó ÂÙÒÓ, ÛÙËÓ Î·Ù·Ó¿ÏˆÛË ÊÚÔ‡ÙˆÓ (5,8 ¤Ó·ÓÙÈ 4,2, p<0,05), Á·Ï·ÎÙÔÎÔÌÈÎÒÓ (9,9 ¤Ó·ÓÙÈ 8,6, p<0,001) Î·È ÔÈÎÈÏ›·˜ ÙÚÔÊ›ÌˆÓ (4,8 ¤Ó·ÓÙÈ 6,2, p<0,01). Paediatriki 2007;70:24-36
∏ Û˘ÓÔÏÈ΋ ̤ÛË ‚·ıÌÔÏÔÁ›· ÙÔ˘ ¢À¢ ‚Ú¤ıËΠ58,7, Ì ÙË ‰È·ÙÚÔÊ‹ Ù˘ ÏÂÈÔÓfiÙËÙ·˜ ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ó· Ù·ÍÈÓÔÌÂ›Ù·È ÛÙËÓ Î·ÙËÁÔÚ›· “¯ÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË”. ¢ÂÓ ‚Ú¤ıËΠÛËÌ·ÓÙÈ΋ Ù¿ÛË ÌÂÙ·‚ÔÏ‹˜ Ì ÙËÓ ·‡ÍËÛË ÙˆÓ ·Ú·ÁfiÓÙˆÓ ÙÔ˘ ªÂÙ™˘Ó ÛÙËÓ ÚfiÛÏË„Ë ıÚÂÙÈÎÒÓ Û˘ÛÙ·ÙÈÎÒÓ, ÛÙËÓ Î·Ù·Ó¿ÏˆÛË ÙÚÔʛ̈Ó, ÛÙÔÓ ¢À¢ Î·È ÛÙȘ Û˘ÓÈÛÙÒÛ˜ ÙÔ˘ (‰Â‰Ô̤ӷ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Û ›Ó·Î·). øÛÙfiÛÔ, Ù· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó, Û ۇÁÎÚÈÛË Ì ÂΛӷ Ì ªÂÙ™˘Ó, ›¯·Ó ÌÈÎÚfiÙÂÚË Ï‹„Ë ÎÔÚÂÛÌ¤ÓˆÓ ÏÈ·ÚÒÓ ÔͤˆÓ (31,9 g ¤Ó·ÓÙÈ 34,8 g, ª™) Î·È Î·Ù·Ó¿ÏˆÛË ÎÚ¤·ÙÔ˜ (115 g ¤Ó·ÓÙÈ 146 g, ª™), ÂÓÒ ‚Ú¤ıËÎ·Ó Ì ÌÂÁ·Ï‡ÙÂÚË Î·Ù·Ó¿ÏˆÛË ÊÚÔ‡ÙˆÓ Î·È Ï·¯·ÓÈÎÒÓ (299 g ¤Ó·ÓÙÈ 244 g, ª™).
29
ªÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜
¶›Ó·Î·˜ 3. ¶·È‰È¿ Î·È ¤ÊË‚ÔÈ Ì ‹ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ Û ۯ¤ÛË Ì ÙÔ Ê‡ÏÔ Î·È ÙËÓ ËÏÈΛ· ∏ÏÈΛ· (¤ÙË) 3-6
6ó-10
10ó-14
14ó-17ó
™‡ÓÔÏÔ
¡ (%) ∞ÁfiÚÈ·
™‡ÓÔÏÔ ¶·Ú¿ÁÔÓÙ˜ 0 1 2 ≥3
∫ÔÚ›ÙÛÈ·*
™‡ÓÔÏÔ ¶·Ú¿ÁÔÓÙ˜ 0 1 2 ≥3
81
113
152
196
542
43 (53,1) 29 (35,8) 7 (8,6) 2 (2,5) 68
42 (37,2) 48 (42,5) 15 (13,3) 8 (7,1) 120
63 (41,4) 65 (42,8) 20 (13,2) 4 (2,6) 215
80 (40,8) 81 (41,3) 26 (13,3) 9 (4,6) 264
228 (42,1) 223 (41,1) 68 (12,5) 23 (4,2) 667
41 (60,3) 19 (27,9) 6 (8,8) 2 (2,9)
49 (40,8) 48 (40,0) 19 (15,8) 4 (3,3)
63 (29,3) 116 (54,0) 26 (12,1) 10 (4,7)
128 (48,5) 106 (40,2) 22 (8,3) 8 (3,0)
281 (42,1) 289 (43,3) 73 (10,9) 24 (3,6)
* ŒÏÂÁ¯Ô˜ χ2 (p<0,05) ** ŒÏÂÁ¯Ô˜ χ2 ÁÈ· ÁÚ·ÌÌÈ΋ Ù¿ÛË (p value for linear trend): p<0,05
¶›Ó·Î·˜ 4. ª¤ÛË ‚·ıÌÔÏÔÁ›· Û˘ÓÈÛÙˆÛÒÓ Î·È ¢Â›ÎÙË ÀÁÈÂÈÓ‹˜ ¢È·ÙÚÔÊ‹˜ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ ˆ˜ ÚÔ˜ ÙÔ Ê‡ÏÔ Î·È ÙȘ ÔÌ¿‰Â˜ ËÏÈÎÈÒÓ ∏ÏÈΛ· (¤ÙË) 3-6 ™˘ÓÈÛÙÒÛ˜ & ‰Â›ÎÙ˘
º‡ÏÔ
6ó-10
10ó-14
14ó-17ó
™‡ÓÔÏÔ
ª¤ÛË ÙÈÌ‹ ± ∆˘È΋ ·fiÎÏÈÛË
1. √Ì¿‰· ‰ËÌËÙÚÈ·ÎÒÓ („ˆÌ›, Ú‡˙È, ‰ËÌËÙÚȷο, ˙˘Ì·ÚÈο)
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
6,4±3,6 6,0±3,5
7,3±3,1 6,6±3,2
6,1±3,1 5,4±3,1
5,7±2,8 5,7±3,3
6,2±3,1 5,8±3,3
2. §·¯·ÓÈο
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
3,4±3,7 3,1±3,6
2,5±3,4 2,9±3,6
2,3±3,1 2,4±3,2
3,0±3,1 2,8±3,3
2,8±3,3 2,7±3,3
3. ºÚÔ‡Ù·
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
5,8±4,7* 6,0±4,5
4,9±4,6 5,0±4,8
4,2±4,6 5,0±4,8
4,2±4,4 4,3±4,6
4,6±4,6 4,8±4,72
4. √Ì¿‰· Á·Ï·ÎÙÔÎÔÌÈÎÒÓ (Á¿Ï·, ÁÈ·Ô‡ÚÙÈ, Ù˘Ú›)
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
9,9±0,6*** 9,8±1,4
9,5±2,0 9,2±2,6
8,7±2,7 7,4±3,8
8,6±3,0 7,4±3,9
9,0±2,6 7,9±3,6
5. √Ì¿‰· ÎÚ¤·ÙÔ˜ (ÎÚ¤·˜, Ô˘ÏÂÚÈο, ÍËÚ¿ Ê·ÛfiÏÈ·, „¿ÚÈ, ·˘Á¿, ÍËÚÔ› ηÚÔ›)
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
8,7±3,2 8,1±3,8
7,8±3,8 8,4±3,4
8,4±3,4 7,7±3,9
8,2±3,5 7,3±4,1
8,2±3,5 7,7±3,9
6. √ÏÈÎfi ϛԘ (% ÂÓ¤ÚÁÂÈ·˜)
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
3,0±2,9 3,5±3,0
2,9±3,3 2,8±3,2
3,7±3,6 3,0±3,5
3,2±3,4 3,8±3,8
3,3±3,4 3,4±3,6
7. ∫ÔÚÂṲ̂ÓÔ Ï›Ô˜ (% ÂÓ¤ÚÁÂÈ·˜)
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
2,0±2,9*** 2,2±3,0***
3,1±3,6 2,2±3,3
3,6±3,7 3,8±3,9
3,9±3,9 3,9±4,0
3,4±3,7 3,4±3,8
8. ÃÔÏËÛÙÂÚfiÏË ÙÚÔÊ‹˜
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
8,9±2,8*** 8,3±3,3
8,0±3,6 9,1±2,5
7,8±3,8 8,6±3,0
6,9±4,2 8,6±3,2
7,6±3,9 8,6±3,0
9. ¡¿ÙÚÈÔ
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
9,9±0,2*** 9,8±0,6***
9,6±1,4 9,6±1,3
8,6±2,5 9,6±1,4
7,8±3,0 9,2±2,0
8,7±2,5 9,5±1,6
10. ¶ÔÈÎÈÏ›· ÙÚÔʛ̈Ó
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
4,8±3,1** 4,6±3,3
4,7±3,1 4,4±3,1
5,6±3,4 4,5±3,1
6,2±3,3 4,3±3,1
5,6±3,3 4,4±3,1
¢Â›ÎÙ˘ ÀÁÈÂÈÓ‹˜ ¢È·ÙÚÔÊ‹˜
∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
62,8±12,7** 61,5±13,5*
60,3±14,3 60,2±12,6
59,0±11,9 57,4±13,0
57,8±12,2 59,3±12,7 57,5±14,7 58,3±13,7
∆· ‰È·ÈÙÔÏfiÁÈ· ·fi Ù· ÔÔ›· ÚÔ¤Ú¯ÔÓÙ·È ÔÈ ·Ú·¿Óˆ ÂÎÙÈÌ‹ÛÂȘ ·Ó¤Ú¯ÔÓÙ·È Û 1124 Û ۇÓÔÏÔ 1209 ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ. ∏ ηٷÓÔÌ‹ ÙÔ˘˜ (¡) ÛÙȘ ·Ú·¿Óˆ ÔÌ¿‰Â˜ ËÏÈÎÈÒÓ Â›Ó·È ·ÓÙ›ÛÙÔȯ· ÛÙ· ·ÁfiÚÈ·: 66, 95,151 Î·È 191 Î·È ÛÙ· ÎÔÚ›ÙÛÈ·: 53, 103, 209 Î·È 256. # ŒÏÂÁ¯Ô˜ Student t test (equal variances not assumed) ÌÂٷ͇ ÙˆÓ ËÏÈÎÈÒÓ 3-6 Î·È 14ó-17ó: * p≤0,05, ** p≤0,01, *** p≤0,001
¶·È‰È·ÙÚÈ΋ 2007;70:24-36
30
ª. §ÈÓ·Ú‰¿Î˘ Î·È Û˘Ó.
ªÂ ÙËÓ ·‡ÍËÛË ÙÔ˘ ·ÚÈıÌÔ‡ ·Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó (¶›Ó·Î·˜ 5), ·˘Í¿ÓÔÓÙ·È ÛËÌ·ÓÙÈο Ù· ÔÛÔÛÙ¿ ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ Ì ·Ó·Ú΋ Ï‹„Ë ÚˆÙÂ˚ÓÒÓ (Ï‹„Ë <67% ÙÔ˘ RDA) ·fi 4% Û 11% (p=0,027), Ì·ÁÓËÛ›Ô˘ ·fi 45% Û 61% (p=0,018), Ê˘ÏÈÎÔ‡ ÔͤԘ ·fi 53% Û 55% (p=0,010) Î·È ‚ÈÙ·Ì›Ó˘ ∞ ·fi 43% Û 45% (p=0,023). ∞ÓÙ›ıÂÙ·, ‰ÂÓ ÌÂÙ·‚¿ÏÏÂÙ·È ÛËÌ·ÓÙÈο Ë ‚·ıÌÔÏÔÁ›· ÙÔ˘ ¢À¢ Î·È ÙˆÓ Û˘ÓÈÛÙˆÛÒÓ ÙÔ˘ Ì ÙËÓ ·‡ÍËÛË ÙÔ˘ ·ÚÈıÌÔ‡ ·Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó (‰Â‰Ô̤ӷ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Û ›Ó·Î·). ¶·È‰È¿ Î·È ¤ÊË‚ÔÈ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó, Û ۯ¤ÛË Ì ÂΛӷ Ì ªÂÙ™˘Ó, ‚Ú¤ıËÎ·Ó Ì ˘„ËÏfiÙÂÚË ‚·ıÌÔÏÔÁ›· ÛÙËÓ Î·Ù·Ó¿ÏˆÛË ‰ËÌËÙÚÈ·ÎÒÓ (6,1 ¤Ó·ÓÙÈ 5,6, ª™), ÊÚÔ‡ÙˆÓ (5,1 ¤Ó·ÓÙÈ 4,4, ª™) Î·È ÛÙÔÓ ¢À¢ (59,8 ¤Ó·ÓÙÈ 58,4, ª™). ∏ ηٷÓÔÌ‹ ÙˆÓ ·ÁÔÚÈÒÓ Î·È ÙˆÓ ÎÔÚÈÙÛÈÒÓ ˆ˜ ÚÔ˜ ÙȘ ηÙËÁÔڛ˜ ÙÔ˘ ¢À¢ (¶›Ó·Î·˜ 6) ‰ÂÓ ‰È·ÊÔÚÔÔÈ›ٷÈ, ›Û˘, ÛËÌ·ÓÙÈο. ¶ÔÛÔÛÙfi ÌfiÏȘ 6% ÙˆÓ ·ÁÔÚÈÒÓ Â›¯Â “ηϋ” ‰È·ÙÚÔÊ‹ Î·È 27% “·Ó·Ú΋”, ÂÓÒ ÛÙ· ÎÔÚ›ÙÛÈ· ÔÛÔÛÙfi 7% ›¯Â “ηϋ” Î·È 32% “·Ó·Ú΋” ‰È·ÙÚÔÊ‹. øÛÙfiÛÔ, ÙÔ ÔÛÔÛÙfi ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ Ì “ηϋ” ‰È·ÙÚÔÊ‹ ·fi 10% ÛÙȘ ËÏÈ˘ 3-6 ÂÙÒÓ, ÌÂÈÒÓÂÙ·È ÛËÌ·ÓÙÈο Û 7% ÛÙȘ ËÏÈ˘ ÙˆÓ 14ó-17ó ÂÙÒÓ, ÂÓÒ Ì “·Ó·Ú΋” ‰È·ÙÚÔÊ‹ ·˘Í¿ÓÂÙ·È ·fi 24% Û 32% (p=0,019). ∂ÈϤÔÓ, ÙÔ 36% ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ì ªÂÙ™˘Ó, ›¯Â “·Ó·Ú΋” ‰È·ÙÚÔÊ‹ Î·È ÙÔ 5% “ηϋ”, ¤Ó·ÓÙÈ 28% Î·È 8%, ·ÓÙ›ÛÙÔȯ·, ÂÎÂ›ÓˆÓ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó (p=0,026).
√ Û¯ÂÙÈÎfi˜ ΛӉ˘ÓÔ˜ (Odds Ratio) (¶›Ó·Î·˜ 7) ÁÈ· ªÂÙ™˘Ó ‚Ú¤ıËΠӷ ·˘Í¿ÓÂÙ·È Î·Ù¿ 3,16 ÊÔÚ¤˜ ÁÈ· Ù· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ Ì ·˘ÍË̤ӷ ›‰· LDL-C (95% ¢∂: 1,59-6,29, p=0,001), ηٿ 71,2 ÊÔÚ¤˜ ÁÈ· fiÛ· ¤¯Ô˘Ó ·˘ÍË̤ӷ ›‰· ·ıËڈ̷ÙÈÎÔ‡ ‰Â›ÎÙË ÔÏÈ΋˜ ÚÔ˜ ∏DL-C (95% ¢∂: 19,5260,1, p<0,001) Î·È Î·Ù¿ 84,8 ÊÔÚ¤˜ ÁÈ· fiÛ· Â›Ó·È ·¯‡Û·Úη (¢ª™ >30 kg/m2) (95% ¢∂: 32,7-219,4, p<0,001). ∂›Û˘, ·È‰È¿ Î·È ¤ÊË‚ÔÈ Ì ªÂÙ™˘Ó ‚Ú¤ıËÎ·Ó Ó· ¤¯Ô˘Ó ÛËÌ·ÓÙÈο ÌÂȈ̤Ó˜ Èı·ÓfiÙËÙ˜ (ηٿ 30%) ÁÈ· ηϋ ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·ÓÙÔ¯‹ (95% ¢∂: 0,49-0,99, p<0,042). ∆Ô 85% ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ Ì ªÂÙ™˘Ó (∂ÈÎfiÓ· 1) ˘ÂÚ‚·›ÓÂÈ Ù· fiÚÈ· Ù˘ 90‹˜ ÂηÙÔÛÙÈ·›·˜ ı¤Û˘ Ù˘ Û˘ÛÙÔÏÈ΋˜ ›ÂÛ˘ οÙÈ Ô˘ ·ÔÙÂÏ› ÙÔÓ ÈÔ Û˘¯Ófi ·Ú¿ÁÔÓÙ· ªÂÙ™˘Ó. ∞ÎÔÏÔ˘ıÔ‡Ó Ù· ·˘ÍË̤ӷ ÙÚÈÁÏ˘ÎÂÚ›‰È· Î·È Ë ¯·ÌËÏ‹ HDL-C. ™Â 243 ÂÊ‹‚Ô˘˜ ËÏÈΛ·˜ 15-17 ÂÙÒÓ, ÔÛÔÛÙfi 12% ‚Ú¤ıËΠӷ Â›Ó·È Î·ÓÈÛÙ¤˜ (‰Â‰Ô̤ӷ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Û ›Ó·Î·) (13% ·ÁfiÚÈ· Î·È 11% ÎÔÚ›ÙÛÈ·, χ2 ¤ÏÂÁ¯Ô˜, ª™). øÛÙfiÛÔ, ‰ÂÓ Û¯ÂÙ›˙ÂÙ·È ÛËÌ·ÓÙÈο ÙÔ Î¿ÓÈÛÌ· Ì ÙÔÓ ÂÈÔÏ·ÛÌfi ÙÔ˘ ªÂÙ™˘Ó. ∆¤ÏÔ˜, Ì ‚¿ÛË ÙÔÓ ¢ª™, ÙÔ 23,2% ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ ‚Ú¤ıËΠ̠·˘ÍË̤ÓÔ ‚¿ÚÔ˜ Î·È ÙÔ 9,9% Ì ·¯˘Û·ÚΛ· (‰Â‰Ô̤ӷ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Û ›Ó·Î·). ªÂ ·˘ÍË̤ÓÔ ‚¿ÚÔ˜ ‚Ú¤ıËΠÙÔ 25% ÙˆÓ ·ÁÔÚÈÒÓ Î·È ÙÔ 21% ÙˆÓ ÎÔÚÈÙÛÈÒÓ Î·È Ì ·¯˘Û·ÚΛ· ÔÛÔÛÙfi 12% Î·È 9%, ·ÓÙ›ÛÙÔȯ· (χ2 ¤ÏÂÁ¯Ô˜,
¶›Ó·Î·˜ 5. ¶ÔÛÔÛÙ¿ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ô˘ ÚÔÛÏ·Ì‚¿ÓÔ˘Ó ıÚÂÙÈο Û˘ÛÙ·ÙÈο ÏÈÁfiÙÂÚÔ ·fi 67% Ù˘ ËÌÂÚ‹ÛÈ·˜ Û˘ÓÈÛÙÒÌÂÓ˘ ÔÛfiÙËÙ·˜ (RDA) Û ۯ¤ÛË Ì ÙËÓ ·ÚÔ˘Û›· ·Ú·ÁfiÓÙˆÓ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ¶·Ú¿ÁÔÓÙ˜ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ 0
1
<67% RDA ª·ÎÚÔıÚÂÙÈο
ª¤Ù·ÏÏ· & ȯÓÔÛÙÔȯ›·
µÈٷ̛Ә
≥3
¡ (%) ∂Ó¤ÚÁÂÈ· ¶ÚˆÙ½Ó˜ ÿÓ˜ ∞Û‚¤ÛÙÈÔ ™›‰ËÚÔ˜ ª·ÁÓ‹ÛÈÔ ºÒÛÊÔÚÔ˜ º˘ÏÈÎfi Ô͇ µÈÙ·Ì›ÓË ∞ µÈÙ·Ì›ÓË ∂ µÈÙ·Ì›ÓË C µÈÙ·Ì›ÓË µ1 µÈÙ·Ì›ÓË µ2 µÈÙ·Ì›ÓË µ6 µÈÙ·Ì›ÓË µ12 ¡È·Û›ÓË
106 (22) 19 (4) 362 (77) 239 (51) 103 (22) 208 (45) 140 (31) 252 (53) 202 (43) 304 (64) 96 (20) 20 (4) 17 (4) 58 (12) 103 (22) 66 (14)
* ŒÏÂÁ¯Ô˜ χ2 ÁÈ· ÁÚ·ÌÌÈ΋ Ù¿ÛË (p value for linear trend) Paediatriki 2007;70:24-36
2
127 (26) 26 (5) 382 (80) 253 (53) 108 (23) 238 (51) 166 (36) 310 (65) 232 (48) 308 (64) 125 (26) 22 (5) 16 (3) 68 (14) 102 (21) 82 (17)
p* 35 (27) 9 (7) 109 (83) 67 (51) 30 (23) 66 (51) 45 (35) 86 (66) 74 (56) 87 (67) 38 (29) 9 (7) 5 (4) 22 (17) 33 (25) 28 (22)
14 (32) 5 (11) 34 (77) 25 (57) 12 (27) 27 (61) 19 (44) 24 (55) 20 (45) 27 (61) 7 (16) 0 4 (9) 9 (20) 13 (30) 7 (16)
ª™ 0,027 ª™ ª™ ª™ 0,018 ª™ 0,010 0,023 ª™ ª™ ª™ ª™ ª™ ª™ ª™
31
ªÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜
¶›Ó·Î·˜ 6. ∞ÚÈıÌfi˜ Î·È ÔÛÔÛÙ¿ ·È‰ÈÒÓ Ì ‚·ıÌÔÏÔÁ›· ¢À¢ “·Ó·Ú΋”, “¯ÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË” Î·È “ηϋ” Û ۯ¤ÛË Ì ÙÔ Ê‡ÏÔ, ÙËÓ ËÏÈΛ· Î·È ÙËÓ ·ÚÔ˘Û›· ·Ú·ÁfiÓÙˆÓ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ¢Â›ÎÙ˘ ÀÁÈÂÈÓ‹˜ ¢È·ÙÚÔÊ‹˜ ∞Ó·Ú΋˜ ÃÚÂÈ¿˙ÂÙ·È (<51) ‚ÂÏÙ›ˆÛË (51-80)
∫·Ï‹ (>80)
¡ (%)
p*
º‡ÏÔ ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ· ™‡ÓÔÏÔ
139 (27) 197 (32) 336 (30)
336 (67) 382 (61) 718 (64)
28 (6) 42 (7) 70 (6)
∏ÏÈΛ· 3-6 6ó-10 10ó-14 14ó-17ó
28 (24) 52 (26) 113 (31) 143 (32)
79 (66) 130 (66) 234 (65) 275 (62)
12 (10) 16 (8) 0,019** 13 (4) 29 (7)
¶·Ú¿ÁÔÓÙ˜ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ 0 131 (28) 303 (64) 1 144 (30) 311 (65) 2 45 (35) 78 (60) ≥3 16 (36) 26 (59)
38 (8) 23 (5) 0,026** 7 (5) 2 (5)
* **
ª™*
ŒÏÂÁ¯Ô˜ χ2 ŒÏÂÁ¯Ô˜ χ2 ÁÈ· ÁÚ·ÌÌÈ΋ Ù¿ÛË (p value for linear trend)
p=0,024). ∂›Û˘, Ì ·¯˘Û·ÚΛ· ‚Ú¤ıËΠÙÔ 51% ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ Ì ªÂÙ™˘Ó, ¤Ó·ÓÙÈ 8% ÂÎÂ›ÓˆÓ Ì 0-2 ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó (p<0,001).
∂ÈÔÏ·ÛÌfi˜ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ §›Á˜ ¤Ú¢Ó˜ ·ÁÎÔÛÌ›ˆ˜ ¤¯Ô˘Ó ÌÂÏÂÙ‹ÛÂÈ ÙÔ ªÂÙ™˘Ó ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ· (35). √ ÂÈÔÏ·ÛÌfi˜ ÙÔ˘, ˆÛÙfiÛÔ, ÂÎÙÈÌ¿Ù·È ÌÂٷ͇ 4% Î·È 5% (36). ∞fi ÙË ÌÂϤÙË NHANES 1999-2000 ÙˆÓ ∏¶∞, Û ¤ÊË‚Ô˘˜ ËÏÈΛ·˜ 12-19 ÂÙÒÓ (10), ‚Ú¤ıËΠfiÙÈ 6,4% ›¯Â ªÂÙ™˘Ó (9,1% Ù· ·ÁfiÚÈ· Î·È 3,7% Ù· ÎÔÚ›ÙÛÈ·) Î·È fiÙÈ ÂÚÈÛÛfiÙÂÚÔÈ ·fi ‰‡Ô ÂηÙÔÌ̇ÚÈ· ¤ÊË‚ÔÈ
39
™¿Î¯·ÚÔ ¶·Ú¿ÁÔÓÙ˜ ªÂÙ·‚ÔÏÈÎÔ‡ ™˘Ó‰ÚfiÌÔ˘
™˘˙‹ÙËÛË ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, ÔÛÔÛÙfi 3,9% ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ù˘ ∫Ú‹Ù˘ ‚Ú¤ıËΠ̠ÙÚÂȘ ‹ ÂÚÈÛÛfiÙÂÚÔ˘˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó (4,2% ÛÙ· ·ÁfiÚÈ·, 3,6% ÛÙ· ÎÔÚ›ÙÛÈ·). ∏ ‚·ıÌÔÏÔÁ›· ÙÔ˘ ¢À¢ ‰ÂÓ ‰È¤ÊÂÚ ÛËÌ·ÓÙÈο ·fi ÙË ‚·ıÌÔÏÔÁ›· ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ Ì ηıfiÏÔ˘ ‹ ÏÈÁfiÙÂÚÔ˘˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó. øÛÙfiÛÔ, Ë ·ÍÈÔÏfiÁËÛË Ù˘ ‰È·ÙÚÔÊ‹˜ ÙÔ˘˜ ¤‰ÂÈÍ fiÙÈ ¤¯Ô˘Ó Û ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi “·Ó·Ú΋” ‰È·ÙÚÔÊ‹ Î·È Û ÌÈÎÚfiÙÂÚÔ “ηϋ”. ∂›Û˘, Û ÔÛÔÛÙfi 64% fiÏˆÓ ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ù˘ ÌÂϤÙ˘, ·ÓÂÍ·Úًو˜ ‰ËÏ·‰‹ Ù˘ ·ÚÔ˘Û›·˜ ‹ ÌË ·Ú·ÁfiÓÙˆÓ ªÂÙ™˘Ó, ‚Ú¤ıËΠfiÙÈ Ë ‰È·ÙÚÔÊ‹ “¯ÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË”. ∞ÎfiÌË, Ù· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ Ì ÙÚÂȘ ‹ ÂÚÈÛÛfiÙÂÚÔ˘˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó ηٷӷÏÒÓÔ˘Ó ÌÈÎÚfiÙÂÚË ÔÛfiÙËÙ· ÊÚÔ‡ÙˆÓ Î·È Ï·¯·ÓÈÎÒÓ, ÂÓÒ Â›¯·Ó ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚ· ÔÛÔÛÙ¿ ·Ó·ÚÎÔ‡˜ Ï‹„˘ ÚˆÙÂ˚ÓÒÓ, Ê˘ÏÈÎÔ‡ ÔͤԘ Î·È ‚ÈÙ·Ì›Ó˘ ∞.
13 87
™˘ÛÙÔÏÈ΋ ∞¶
83 44 42
¢È·ÛÙÔÏÈ΋ ∞¶
52
∆ÚÈÁÏ˘ÎÂÚ›‰È·
83 57
HDL-C
75 ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·
61 63
¶ÂÚ. ª¤Û˘ 0
10
20
30
40
50
60
70
80
90
100
¶ÔÛÔÛÙfi ·È‰ÈÒÓ / ÂÊ‹‚ˆÓ (%)
∂ÈÎfiÓ· 1. ¶ÔÛÔÛÙ¿ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ì ÌÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ Ô˘ ˘ÂÚ‚·›ÓÔ˘Ó Ù· fiÚÈ· (cut offs) ÙˆÓ ·Ú·ÁfiÓÙˆÓ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘. ¶·È‰È·ÙÚÈ΋ 2007;70:24-36
32
ª. §ÈÓ·Ú‰¿Î˘ Î·È Û˘Ó.
¶›Ó·Î·˜ 7. ∂ÎÙ›ÌËÛË ‰ÂÈÎÙÒÓ Û¯ÂÙÈÎÔ‡ ÎÈÓ‰‡ÓÔ˘ (Odds Ratio) ‰È·ÊfiÚˆÓ ·Ú·ÁfiÓÙˆÓ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ÌÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ Û ۯ¤ÛË Ì ·È‰È¿ ¯ˆÚ›˜ ·Ú¿ÁÔÓÙ˜ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘
√ÏÈ΋ ¯ÔÏËÛÙÂÚfiÏË* LDL ¯ÔÏËÛÙÂÚfiÏË** ∞ıËڈ̷ÙÈÎfi˜ ‰Â›ÎÙ˘ ÔÏÈ΋˜ ¯ÔÏËÛÙÂÚfiÏ˘ ÚÔ˜ HDL-C*** ∞ıËڈ̷ÙÈÎfi˜ ‰Â›ÎÙ˘ LDL-C ÚÔ˜ HDL-C$ ¢Â›ÎÙ˘ ª¿˙·˜ ™ÒÌ·ÙÔ˜ (kg/m2) ¶·Ï›Ó‰ÚÔÌÔ ÙÚ¤ÍÈÌÔ ·ÓÙÔ¯‹˜ ∫¿ÓÈÛÌ·
¡
OR (95% ‰È¿ÛÙËÌ· ÂÌÈÛÙÔÛ‡Ó˘)
p-value#
556 556 556
1,58 (0,70-3,57) 3,16 (1,59-6,29) 71,2 (19,5-260,1)
ª™ 0,001 <0,001
556
47,4 (9,4-239,5)
<0,001
25-30$$ >30 181 ∫·ÓÈÛÙ¤˜
556 556 0,70 (0,49-0,99) 106
7,32 (3,07-14,47)<0,001 84,8 (32,7-219,4)<0,001 0,042 0,98 (0,11-8,97) ª™
∏ ·Ó¿Ï˘ÛË ·ÊÔÚ¿ ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ô˘ ‰ÂÓ ¤¯Ô˘Ó ·Ú¿ÁÔÓÙ˜ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ (Ìˉ¤Ó ·Ú¿ÁÔÓÙ˜) Û ۯ¤ÛË Ì ÂΛӷ Ì ÌÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ (ÙÚÂȘ ‹ ÂÚÈÛÛfiÙÂÚÔ˘˜ ·Ú¿ÁÔÓÙ˜). # ∞Ó¿Ï˘ÛË ÏÔÁÈÛÙÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘ (logistic linear regression analysis): ˆ˜ Û˘ÌÌÂÙ·‚ÏËÙ¤˜ ÂϤÁ¯Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÙÔ Ê‡ÏÔ Î·È Ë ËÏÈΛ· * √ÏÈ΋ ¯ÔÏËÛÙÂÚfiÏË >200 mg/dl, ** LDL ¯ÔÏËÛÙÂÚfiÏË >130 mg/dl, *** ∞ıËڈ̷ÙÈÎfi˜ ‰Â›ÎÙ˘ ÔÏÈ΋˜ ¯ÔÏËÛÙÂÚfiÏ˘ ÚÔ˜ HDL >5,00, $ ∞ıËڈ̷ÙÈÎfi˜ ‰Â›ÎÙ˘ LDL ¯ÔÏËÛÙÂÚfiÏ˘ ÚÔ˜ HDL >4,00, $$ ∆· fiÚÈ· ÙÔ˘ ¢ª™ ηıÔÚ›˙ÔÓÙ·È ·fi ÙÔ˘˜ Cole Î·È Û˘Ó, 2000 (23)
¤¯Ô˘Ó ªÂÙ™˘Ó. ™ÙË ÌÂϤÙË NHANES πππ 1988-1992, Ô ÂÈÔÏ·ÛÌfi˜ ‹Ù·Ó ÌÈÎÚfiÙÂÚÔ˜ Î·È ÚÔÛ¤ÁÁÈ˙ ÙÔ 4,2% (10), ÂÓÒ ·fi ÙËÓ Bogalusa Heart Study, Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ËÏÈΛ·˜ 8-17 ÂÙÒÓ, ηٿ ÙÔ ‰È¿ÛÙËÌ· 1978-1996, ‚Ú¤ıËΠ3,6% (37). ™Â ¿ÏÏË ÌÂϤÙË ÛÙȘ ∏¶∞, ÙÔ 1999, Û 439 ·¯‡Û·Úη ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ‚Ú¤ıËΠÂÈÔÏ·ÛÌfi˜ ªÂÙ™˘Ó 50% (38). ™ÙÔÓ ∫·Ó·‰¿, ÙËÓ ›‰È· ÂÚ›Ô‰Ô, Û ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›ÁÌ· 2244 ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ, ËÏÈΛ·˜ 9, 13 Î·È 16 ÂÙÒÓ, Ô ÂÈÔÏ·ÛÌfi˜ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ·ÓÙ›ÛÙ·Û˘ ÛÙËÓ ÈÓÛÔ˘Ï›ÓË ‚Ú¤ıËΠ11,5% (39). ¶·¯˘Û·ÚΛ· ∏ ·¯˘Û·ÚΛ· ·ÔÙÂÏ› ·ÚÈÔ ÁÓÒÚÈÛÌ· ÙÔ˘ ªÂÙ™˘Ó Î·È Û˘Óԉ‡ÂÙ·È Û˘¯Ó¿ ·fi ¿ÏÏÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘, fiˆ˜ Â›Ó·È Ù· ·˘ÍË̤ӷ ÏÈ›‰È· ÔÚÔ‡ ·›Ì·ÙÔ˜, ÙÔ Û¿Î¯·ÚÔ Î·È Ô Û·Î¯·Ú҉˘ ‰È·‚‹Ù˘ Ù‡Ô˘ 2 (40). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, ÔÛÔÛÙfi 33% fiÏˆÓ ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ ‚Ú¤ıËΠӷ Â›Ó·È ˘¤Ú‚·Ú· Î·È ·¯‡Û·Úη Û‡Ìʈӷ Ì ÙÔÓ ¢ª™, ÂÓÒ ÙÔ 51% ÂÎÂ›ÓˆÓ Ì ªÂÙ™˘Ó ‹Ù·Ó ·¯‡Û·Úη. ªÂ ÎÂÓÙÚÈ΋ ·¯˘Û·ÚΛ· ‚Ú¤ıËΠÙÔ 62% ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ì ªÂÙ™˘Ó. ∂ÈϤÔÓ, Ù· ·¯‡Û·Úη ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ ‚Ú¤ıËÎ·Ó Ó· ¤¯Ô˘Ó 85 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚÔ Î›Ó‰˘ÓÔ Ó· ·Ó·Ù‡ÍÔ˘Ó ªÂÙ™˘Ó, Û ۯ¤ÛË Ì ٷ Ê˘ÛÈÔÏÔÁÈο. ∞fi ÙË ÌÂϤÙË NHANES 1999-2000 (10), ÙÔ 32% ÙˆÓ ·¯‡Û·ÚÎˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ, Û‡Ìʈӷ Ì ÙÔÓ ¢ª™, ‚Ú¤ıËΠӷ ¤¯Ô˘Ó ªÂÙ™˘Ó (36). ™ÙË ‰ÂηÂÙ›· ÙÔ˘ 1990, Ô ÂÈÔÏ·ÛÌfi˜ ˘¤Ú‚·ÚˆÓ Î·È ·¯‡Û·ÚÎˆÓ ·È‰ÈÒÓ ËÏÈΛ·˜ 5-17 ÂÙÒÓ ÚÔÛÂÁÁ›˙ÂÈ ·ÁÎÔÛÌ›ˆ˜ ÙÔ 10%, Ì ÙËÓ ∞ÌÂÚÈ΋ Î·È ÙËÓ ∂˘ÚÒË Ó· ˘ÂÚ‚·›ÓÔ˘Ó ÙÔ 20% (34). ™ÙËÓ Paediatriki 2007;70:24-36
∂ÏÏ¿‰· ‹Ù·Ó 23% Î·È ÛÙȘ ∏¶∞ 26% (41), ÂÓÒ ÛÙËÓ πÙ·Ï›· ‚Ú¤ıËΠÙÔ ˘„ËÏfiÙÂÚÔ ÔÛÔÛÙfi (36%) (42). ªÂϤÙË ÏËı˘ÛÌȷ΋˜ Ù¿Û˘ Û ·ÁfiÚÈ· Ù˘ ∫Ú‹Ù˘ ËÏÈΛ·˜ 9-16 ÂÙÒÓ, ¤‰ÂÈÍ fiÙÈ Î·Ù¿ ÙÔ ‰È¿ÛÙËÌ· 1982-2002, ·˘Í‹ıËÎ·Ó Ù· ˘¤Ú‚·Ú· Î·È ·¯‡Û·Úη ·fi 20% Û 40% (43). ∏ ÚfiÏË„Ë Ù˘ ·¯˘Û·ÚΛ·˜ ı· Ú¤ÂÈ Ó· ·Ú¯›˙ÂÈ ·fi ÙË ‚ÚÂÊÈ΋ ËÏÈΛ· (40). √ ıËÏ·ÛÌfi˜, Ë ·Ú·‰ÔÛȷ΋ ÂÏÏËÓÈ΋ ‰È·ÙÚÔÊ‹ Î·È Ë ¿ÛÎËÛË Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈο ̤۷ ÚfiÏ˄˘ Î·È ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ·¯˘Û·ÚΛ·˜ Î·È ÙÔ˘ ªÂÙ™˘Ó. ªÂ ·˘ÙfiÓ ÙÔÓ ÙÚfiÔ ÌÔÚ› Ó· ÌÂȈı› Ô Î›Ó‰˘ÓÔ˜ ÁÈ· ηډȷο ÓÔÛ‹Ì·Ù· ηٿ 35-55% (40,42,44-46). §ÈÔÚˆÙ½Ó˜ Î·È Û¿Î¯·ÚÔ ÔÚÔ‡ ·›Ì·ÙÔ˜ ™Ù· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ Ì ªÂÙ™˘Ó, ÔÛÔÛÙfi 68% ‚Ú¤ıËΠ̠·˘ÍË̤ӷ ›‰· ÙÚÈÁÏ˘ÎÂÚȉ›ˆÓ, 66% Ì ¯·ÌËÏ‹ HDL-C, ÂÓÒ Â›¯·Ó ÂÚ›Ô˘ 3,2 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚÔ Î›Ó‰˘ÓÔ ÁÈ· ·ıÔÏÔÁÈο ›‰· LDL-C. ∆· ˘„ËÏ¿ ›‰· ÏÈÔÚˆÙÂ˚ÓÒÓ Î·È Û·Î¯¿ÚÔ˘, fiˆ˜ Î·È Ë ·¯˘Û·ÚΛ·, Û¯ÂÙ›˙ÔÓÙ·È ¿ÌÂÛ· Ì ÙË ‰È·ÙÚÔÊ‹ Î·È ÙË ÛˆÌ·ÙÈ΋ ¿ÛÎËÛË. ∏ ÔÏÈ΋ ¯ÔÏËÛÙÂÚfiÏË ·˘Í¿ÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· ¤ÌÊÚ·ÁÌ· ÙÔ˘ Ì˘Ôηډ›Ô˘ ηٿ 2,4 ÊÔÚ¤˜, ÂÓÒ Ë ÛˆÛÙ‹ ‰È·ÙÚÔÊ‹, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ¿ÏÏÔ˘˜ ·Ú¿ÁÔÓÙ˜, Û˘Ì‚¿ÏÏÂÈ ÛËÌ·ÓÙÈο ÛÙË Ì›ˆÛ‹ Ù˘ (47). ÕÙÔÌ· Ì ˘„ËÏ¿ ›‰· ÙÚÈÁÏ˘ÎÂÚȉ›ˆÓ Î·È ¯·ÌËÏ‹ HDL-C Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÔ˘Ó ÌÈÎÚ¤˜ ÔÛfiÙËÙ˜ ÎÔÚÂÛÌ¤ÓˆÓ ÏÈ·ÚÒÓ ÔͤˆÓ Î·È ¯ÔÏËÛÙÂÚfiÏ˘ ÙÚÔÊ‹˜, Ó· ÂÚÈÔÚ›˙Ô˘Ó ÙËÓ ÂÓ¤ÚÁÂÈ· Ô˘ Ï·Ì‚¿ÓÔ˘Ó ·Ó¿ÏÔÁ· Ì ÙÔ ‚¿ÚÔ˜ ÙÔ˘˜ Î·È Ó· ¤¯Ô˘Ó ηıËÌÂÚÈÓ‹ ۈ̷ÙÈ΋ ¿ÛÎËÛË Ì ̤ÙÚÈÔ ÚÔ˜ ¤ÓÙÔÓÔ Ú˘ıÌfi (47).
33
ªÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜
™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, ÙÔ ·˘ÍË̤ÓÔ Û¿Î¯·ÚÔ, ˆ˜ ¤Ó‰ÂÈÍË ‰È·Ù·Ú·¯‹˜ Ù˘ ۷ί·Ú·ÈÌÈ΋˜ η̇Ï˘, ·ÚÔ˘ÛÈ¿˙ÂÈ ÙË ¯·ÌËÏfiÙÂÚË Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ (26%) Û ۯ¤ÛË Ì ÙÔ˘˜ ˘fiÏÔÈÔ˘˜ Ù¤ÛÛÂÚȘ ·Ú¿ÁÔÓÙ˜. ∏ ηٿÏÏËÏË ‰È·ÙÚÔÊ‹ Î·È Ë ÛˆÌ·ÙÈ΋ ¿ÛÎËÛË Â›Ó·È Ù· ÌÔÓ·‰Èο ̤۷ ÁÈ· ÙËÓ ·‡ÍËÛË Ù˘ ¢·ÈÛıËÛ›·˜ ÛÙË ‰Ú¿ÛË Ù˘ ÈÓÛÔ˘Ï›Ó˘. ŒÙÛÈ ÂÏ·ÙÙÒÓÔÓÙ·È Ù· ›‰· ۷ί¿ÚÔ˘, ÂÓÒ Ì ÙËÓ ·ÓÙÈηٿÛÙ·ÛË ÙÔ˘ ÎÔÚÂṲ̂ÓÔ˘ Ï›Ô˘˜ Ì ÌÔÓÔ·ÎfiÚÂÛÙÔ ·fi ÂÏ·ÈfiÏ·‰Ô, ·˘Í¿ÓÂÙ·È Ë Â˘·ÈÛıËÛ›· ÙˆÓ ÈÛÙÒÓ ÛÙË ‰Ú¿ÛË Ù˘ ÈÓÛÔ˘Ï›Ó˘, ÌÂ Û˘Ó¤ÂÈ· ÙËÓ ÂÏ¿ÙÙˆÛË ÙÔ˘ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Ù‡Ô˘ 2 (3,15). ∂›Û˘, Ë Î·Ù·Ó¿ÏˆÛË ÙÚÔÊ›ÌˆÓ Ì ˘„ËÏfi ÁÏ˘Î·ÈÌÈÎfi ‰Â›ÎÙË Û¯ÂÙ›˙ÂÙ·È Ì ·‡ÍËÛË ÙÔ˘ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Ù‡Ô˘ 2 (48). ™ÙË ÌÂϤÙË Framingham, Û ÂÓ‹ÏÈΘ Ì ªÂÙ™˘Ó ‚Ú¤ıËΠ¯·ÌËÏfiÙÂÚË Î·Ù·Ó¿ÏˆÛË ‰ËÌËÙÚÈ·ÎÒÓ Ì ‰È·ÈÙËÙÈΤ˜ ›Ó˜, Û ۯ¤ÛË Ì ¿ÙÔÌ· ¯ˆÚ›˜ ªÂÙ™˘Ó. √ ÂÈÔÏ·ÛÌfi˜ ÙÔ˘ ªÂÙ™˘Ó Û¯ÂÙÈ˙fiÙ·Ó ıÂÙÈο Ì ÙËÓ ·‡ÍËÛË ÙÔ˘ ÁÏ˘Î·ÈÌÈÎÔ‡ ‰Â›ÎÙË ÙˆÓ ÙÚÔÊ›ÌˆÓ Ô˘ Î·Ù·Ó¿ÏˆÓ·Ó (49). ∞ÚÙËÚȷ΋ ›ÂÛË ·›Ì·ÙÔ˜ ∏ ˘¤ÚÙ·ÛË Â›¯Â ÙË ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ Ì ÔÛÔÛÙfi 96% ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ Ì ªÂÙ™˘Ó. √ ÂÈÔÏ·ÛÌfi˜ Ù˘ ıˆÚÂ›Ù·È ·ÚÎÂÙ¿ ˘„ËÏfi˜ ÛÙȘ ·ÓÂÙ˘Á̤Ó˜ ¯ÒÚ˜ Î·È ÚÔÛÂÁÁ›˙ÂÈ ÙÔ 30% ÙÔ˘ ÂÓ‹ÏÈÎÔ˘ ÏËı˘ÛÌÔ‡, ÂÓÒ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ˘¤ÚÙ·ÛË ‰ÈÏ·ÛÈ¿˙ÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· ηډÈÔ¿ıÂÈ· (15) ηٷ‰ÂÈÎÓ‡ÂÈ ÙËÓ ·Ó·ÁηÈfiÙËÙ· ÁÈ· ¤ÁηÈÚË ÌË Ê·Ú̷΢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË, Ì ‚ÂÏÙ›ˆÛË ÙÔ˘ ÙÚfiÔ˘ ˙ˆ‹˜, ‰È·ÙÚÔÊ‹˜ Î·È ¿ıÏËÛ˘ (50,51). ∏ Ì›ˆÛË ÙÔ˘ ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜, Ë ÂÚÈÔÚÈṲ̂ÓË Î·Ù·Ó¿ÏˆÛË ·Ï·ÙÈÔ‡, Ë ¯Ú‹ÛË ÌfiÓÔ ÂÏ·ÈÔÏ¿‰Ô˘, Ë ·˘ÍË̤ÓË Ï‹„Ë Î·Ï›Ô˘ ·fi Ï·¯·ÓÈο, ÊÚÔ‡Ù·, fiÛÚÈ· Î·È ·Ó¿Ï·ÙÔ˘˜ ÍËÚÔ‡˜ ηÚÔ‡˜, ηıÒ˜ Î·È Ë Î·ıËÌÂÚÈÓ‹ ̤ÙÚÈ· ÚÔ˜ ¤ÓÙÔÓË ÛˆÌ·ÙÈ΋ ¿ÛÎËÛË, ·ÔÙÂÏÔ‡Ó Î‡ÚȘ ÌÂıfi‰Ô˘˜ ÌË Ê·Ú̷΢ÙÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘ (50). º˘ÛÈ΋ ¿ÛÎËÛË ∏ ¤ÏÏÂÈ„Ë ÛˆÌ·ÙÈ΋˜ ¿ÛÎËÛ˘ Û¯ÂÙ›˙ÂÙ·È ÛÙÂÓ¿ Ì ÙÔ ªÂÙ™˘Ó, ηıÒ˜ ·˘Í¿ÓÂÙ·È Ô Î›Ó‰˘ÓÔ˜ ÁÈ· ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË, ·¯˘Û·ÚΛ·, ˘¤ÚÙ·ÛË Î·È ‰˘ÛÏÈȉ·ÈÌ›· (15). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, Ù· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ Ì ªÂÙ™˘Ó ‚Ú¤ıËÎ·Ó Ì ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ˜ Èı·ÓfiÙËÙ˜ (ηٿ 30%) Ó· ¤¯Ô˘Ó ηϋ ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·ÓÙÔ¯‹. √È Ribeiro Î·È Û˘Ó (52) ·Ó·Ê¤ÚÔ˘Ó (·fi ÌÂϤÙË ÛÙËÓ ¶ÔÚÙÔÁ·Ï›·) fiÙÈ ·ÁfiÚÈ· ËÏÈΛ·˜ 815 ÂÙÒÓ, Ì ¯·ÌËÏ¿ ›‰· ۈ̷ÙÈ΋˜ ¿ÛÎËÛ˘, ›¯·Ó ÂÚÈÛÛfiÙÂÚÔ˘˜ ·Ú¿ÁÔÓÙ˜ ªÂÙ™˘Ó. ∞ÎfiÌË,
·fi ÌÂϤÙË ÂÓËÏ›ÎˆÓ Ù˘ ∞ÙÙÈ΋˜, fiÛÔÈ ‰‹ÏˆÓ·Ó fiÙÈ ·ÛÎÔ‡ÓÙ·Ó Ì ̤ÙÚÈ· ¤ÓÙ·ÛË ‚Ú¤ıËÎ·Ó Ì 25% ¯·ÌËÏfiÙÂÚÔ Î›Ó‰˘ÓÔ ÁÈ· ·Ó¿Ù˘ÍË ªÂÙ™˘Ó (53). ∫¿ÓÈÛÌ· ∆Ô Î¿ÓÈÛÌ·, ·Ó Î·È ‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÂÙ·È Ï¤ÔÓ ÛÙÔ ªÂÙ™˘Ó (5), ·ÔÙÂÏ› ÚˆÙ‡ÔÓÙ· ·ıËÚÔÁfiÓÔ ·Ú¿ÁÔÓÙ· Î·È ‰Ú· ÛÂ Û˘Ó¤ÚÁÂÈ· Ì ÙÔ˘˜ ˘fiÏÔÈÔ˘˜. ¢ÂÓ ‚Ú¤ıËΠÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË Ó· Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ·ÚÔ˘Û›· ªÂÙ™˘Ó, ·Ú’ fiÏÔ Ô˘ ÔÛÔÛÙfi 12% ÙˆÓ ÂÊ‹‚ˆÓ ‰‹ÏˆÓ·Ó ηÓÈÛÙ¤˜. ∆Ô Î¿ÓÈÛÌ· Ô˘ ·Ú¯›˙ÂÈ Û˘Ó‹ıˆ˜ ·fi ÙËÓ ÂÊË‚È΋ ËÏÈΛ·, ·ÔÙÂÏ› ÙÔ ÛÔ‚·ÚfiÙÂÚÔ Úfi‚ÏËÌ· ‰ËÌfiÛÈ·˜ ˘Á›·˜ ·ÁÎÔÛÌ›ˆ˜ (13). ∞˘Í¿ÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· ηډÈÔ¿ıÂÈ· ηٿ 2,5 ÊÔÚ¤˜ (48), ÂÓÒ Â˘ı‡ÓÂÙ·È ÁÈ· ÙÔ 13% fiÏˆÓ ÙˆÓ ı·Ó¿ÙˆÓ Î·È ÁÈ· ÂÚ›Ô˘ 19-23 “¯·Ì¤Ó·” ¯ÚfiÓÈ· ˙ˆ‹˜ (54). ∏ ÛËÌ·Û›· Ù˘ ÂÎ·È‰Â˘ÙÈ΋˜ ·Ú¤Ì‚·Û˘ ÛÙË ‰È·ÎÔ‹ ‹ ÙËÓ ÂÏ¿ÙÙˆÛË ÙÔ˘ ηӛÛÌ·ÙÔ˜ ·Ó·‰Â›¯ÙËΠ·fi Û¯ÂÙÈο ÚÔÁÚ¿ÌÌ·Ù· Ô˘ ÂÊ·ÚÌfiÛÙËÎ·Ó Û ۯÔÏ›· Ù˘ ∫Ú‹Ù˘ (19,55). ¢È·ÙÚÔÊ‹ Î·È ÌÂÙ·‚ÔÏÈÎfi Û‡Ó‰ÚÔÌÔ ™‡Ìʈӷ Ì ÙÔÓ ¢À¢, Ë ‰È·ÙÚÔÊ‹ ÙˆÓ 2/3 ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ “¯ÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË”, ÙÔ 1/3 ¤¯ÂÈ “·Ó·Ú΋” ‰È·ÙÚÔÊ‹ Î·È ÂÏ¿¯ÈÛÙÔ ÔÛÔÛÙfi (6%) ¤¯ÂÈ “ηϋ”. √ ¢À¢, ˆ˜ ̤ıÔ‰Ô˜ ÔÈÔÙÈ΋˜ ·ÍÈÔÏfiÁËÛ˘ Ù˘ ËÌÂÚ‹ÛÈ·˜ ‰È·ÙÚÔÊ‹˜, ˘ÔÏÔÁ›˙ÂÙ·È ·fi ÙÔÓ Û˘Ó‰˘·ÛÌfi Ù˘ Ï‹„˘ ıÚÂÙÈÎÒÓ Û˘ÛÙ·ÙÈÎÒÓ Î·È Î·Ù·Ó¿ÏˆÛ˘ ÙÚÔʛ̈Ó. ∂Ê·ÚÌfi˙ÂÙ·È Û ¯ÒÚ˜ Î·È ÏËı˘ÛÌÔ‡˜ Ì ‰È·ÊÔÚÂÙÈΤ˜ ‰È·ÈÙËÙÈΤ˜ Û˘Ó‹ıÂȘ, fiˆ˜ Ë πÛ·Ó›· (28), ÂÚÈÔ¯‹ Ì ÙËÓ ÔÔ›· Ë ∂ÏÏ¿‰· ¤¯ÂÈ ÎÔÈÓ¿ ¯·Ú·ÎÙËÚÈÛÙÈο ÁÓˆÚ›ÛÌ·Ù·, ÛÙÔ Ï·›ÛÈÔ È‰›ˆ˜ Ù˘ ·Ó·ÊÂÚfiÌÂÓ˘ ÌÂÛÔÁÂȷ΋˜ ‰È·ÙÚÔÊ‹˜. øÛÙfiÛÔ, Ô ¢À¢ Ú¤ÂÈ Ó· ·Ó·ÓÂÒÓÂÙ·È, Ó· ÙÚÔÔÔÈÂ›Ù·È Î·È Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ ÂÎÙÈÌ‹ÛÂȘ Î·È ÁÈ· ÙËÓ Î·Ù·Ó¿ÏˆÛË ÌË ˘ÁÈÂÈÓÒÓ ÙÚÔÊ›ÌˆÓ (25,56). ™ÙȘ ∏¶∞, Ù· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ Î·Ù¿ Ù· ¤ÙË 1999-2000, ›¯·Ó ‚·ıÌÔÏÔÁ›· ¢À¢ 62,8, Ô˘ Â›Ó·È ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚË ·fi ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ (¤ÏÂÁ¯Ô˜ Student, 62,8 ¤Ó·ÓÙÈ 58,7, p<0,001), Ì ÔÛÔÛÙfi 16% Ó· ¤¯ÂÈ “·Ó·Ú΋” ‰È·ÙÚÔÊ‹ Î·È 10% “ηϋ” (25). ™ÙËÓ πÛ·Ó›· ÂÎÙÈÌ‹ıËΠfiÙÈ ·È‰È¿ ËÏÈΛ·˜ 6-7 ÂÙÒÓ Â›¯·Ó ‚·ıÌÔÏÔÁ›· 64,6, Ì 3,7% Ó· ¤¯ÂÈ “·Ó·Ú΋” ‰È·ÙÚÔÊ‹ Î·È 1,6% “ηϋ” (28). °È· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Î·È ÙË ıÂڷ›· ÙÔ˘ ªÂÙ™˘Ó, Ë ‰È·ÙÚÔÊ‹ Ú¤ÂÈ Ó· Â›Ó·È ¯·ÌËÏ‹ Û ÎÔÚÂṲ̂ӷ ÏÈ·Ú¿ Ôͤ· Î·È ÏÔ‡ÛÈ· Û ‰È·ÈÙËÙÈΤ˜ ›Ó˜ - ·fi ÙÚfiÊÈÌ· Ô˘ ¤¯Ô˘Ó ¯·ÌËÏfi ÁÏ˘Î·ÈÌÈÎfi ‰Â›ÎÙË - Î·È ÌÔÓÔ·ÎfiÚÂÛÙ· ÏÈ·Ú¿ Ôͤ· Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙÔ ÂÏ·ÈfiÏ·‰Ô (46). ∏ ÓËÛÙÂȷ΋ ‰›·ÈÙ· ¶·È‰È·ÙÚÈ΋ 2007;70:24-36
34
ª. §ÈÓ·Ú‰¿Î˘ Î·È Û˘Ó.
Ô˘ ηıÔÚ›˙ÂÙ·È ·fi ÙÔ˘˜ ηÓfiÓ˜ Ù˘ √Úıfi‰Ô͢ ∂ÏÏËÓÈ΋˜ ∂ÎÎÏËÛ›·˜ (40,57-59) ‚Ú¤ıËΠӷ ¤¯ÂÈ ÙËÓ ˘„ËÏfiÙÂÚË ÂÚÈÂÎÙÈÎfiÙËÙ· Û ۇÓıÂÙÔ˘˜ ˘‰·Ù¿ÓıڷΘ Ì ¯·ÌËÏfi ÁÏ˘Î·ÈÌÈÎfi ‰Â›ÎÙË, Ú¿ÁÌ· Ô˘ Û˘Ó¿‰ÂÈ Ì ÙȘ ‚·ÛÈΤ˜ Ô‰ËÁ›Â˜ Ô˘ ·ÊÔÚÔ‡Ó ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ªÂÙ™˘Ó (46,60). ∂ÈϤÔÓ, ·fi ÙÔ˘˜ Davy Î·È Melby (60) ÂȂ‚·ÈÒÓÂÙ·È fiÙÈ ‰È·ÙÚÔÊ‹ ˘„ËÏ‹ Û ۇÓıÂÙÔ˘˜ ˘‰·Ù¿ÓıڷΘ Î·È ¯·ÌËÏ‹ Û ÏÈ·Ú¿, ÌÂÈÒÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· ¯ÚfiÓȘ ·Ûı¤ÓÂȘ, ÂÓÒ Ë Ï‹„Ë ‰È·ÈÙËÙÈÎÒÓ ÈÓÒÓ, fiˆ˜ ηıÔÚ›˙ÂÙ·È ·fi ÙÔ American Dietetic Association, Â›Ó·È Â˘ÂÚÁÂÙÈ΋ ÁÈ· ÙËÓ ÚfiÏË„Ë Î·È ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ªÂÙ™˘Ó, ·ÊÔ‡ Ô‰ËÁ› ÛÙË Ì›ˆÛË ÙÔ˘ ÎÈÓ‰‡ÓÔ˘ ıÚÔÌ‚ÔÁ¤ÓÂÛ˘.
™˘ÌÂÚ¿ÛÌ·Ù· ∞Ó Î·È Ë Û˘¯ÓfiÙËÙ· ÙÔ˘ ªÂÙ™˘Ó ‚Ú¤ıËΠ¯·ÌËÏfiÙÂÚË Û ۯ¤ÛË Ì ¿ÏϘ ‰ÈÂıÓ›˜ ÌÂϤÙ˜, Ë ·ÓËÛ˘¯›· ÁÈ· ˘„ËÏ‹ ›وۋ ÙÔ˘ ÛÙÔÓ ÎÚËÙÈÎfi ·È‰ÈÎfi Î·È ÂÊË‚ÈÎfi ÏËı˘ÛÌfi ·Ú·Ì¤ÓÂÈ, ·ÊÔ‡ ‚Ú¤ıËÎ·Ó Û ˘„ËÏ‹ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ÔÚÈṲ̂Ó˜ Û˘ÓÈÛÙÒÛ˜ ÙÔ˘, fiˆ˜ Ë ·˘ÍË̤ÓË ·ÚÙËÚȷ΋ ›ÂÛË Î·È Ë ·¯˘Û·ÚΛ·. ∏ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·È‰ÈÒÓ Â›¯Â ‰È·ÙÚÔÊ‹ Ô˘ “¯ÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË”, ÂÓÒ ‹Ù·Ó Ôχ ¯·ÌËÏfi ÙÔ ÔÛÔÛÙfi ÙˆÓ ·È‰ÈÒÓ Î·È ÙˆÓ ÂÊ‹‚ˆÓ Ì “ηϋ” ÔÈÔÙÈÎÒ˜ ‰È·ÙÚÔÊ‹. ∆fiÛÔ Ë “·Ó·Ú΋˜” ‰È·ÙÚÔÊ‹ fiÛÔ Î·È ·˘Ù‹ Ô˘ “¯ÚÂÈ¿˙ÂÙ·È ‚ÂÏÙ›ˆÛË”, ÔÊ›ÏÔÓÙ·È Î˘Ú›ˆ˜ ÛÙË ¯·ÌËÏ‹ ηٷӿψÛË ÊÚÔ‡ÙˆÓ, Ï·¯·ÓÈÎÒÓ Î·È ÔÛÚ›ˆÓ, ÂÓÒ ˘„ËÏ‹ ‹Ù·Ó Ë Ï‹„Ë ÎÔÚÂṲ̂ÓÔ˘ Ï›Ô˘˜ Î·È ÎÚ¤·ÙÔ˜. ∏ ÚˆÙÔÁÂÓ‹˜ ÚfiÏË„Ë Â›Ó·È Ô ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚÔ˜ ÙÚfiÔ˜ ÂÏ¿ÙÙˆÛ˘ Ù˘ Û˘¯ÓfiÙËÙ·˜ ÂÌÊ¿ÓÈÛ˘ ÙÔ˘ ªÂÙ™˘Ó (36). ∫·Ù¿ÏÏËÏ· ÂÎ·È‰Â˘ÙÈο ÚÔÁÚ¿ÌÌ·Ù· Ô˘ ¢·ÈÛıËÙÔÔÈÔ‡Ó Ù· ·È‰È¿ Û ı¤Ì·Ù· ˘Á›·˜ Î·È ‰È·ÙÚÔÊ‹˜, ÌÔÚÔ‡Ó Ó· Û˘Ì‚¿ÏÏÔ˘Ó ÒÛÙ ӷ Á›ÓÔÓÙ·È Î·Ï‡ÙÂÚ˜ ÂÈÏÔÁ¤˜ fiÛÔÓ ·ÊÔÚ¿ ÛÙË ‰È·ÙÚÔÊ‹ Î·È ÛÙËÓ ·‡ÍËÛË Ù˘ ۈ̷ÙÈ΋˜ ¿ÛÎËÛ˘. ∂˘¯·ÚÈÛٛ˜ ∂˘¯·ÚÈÛÙԇ̠ıÂÚÌ¿ ÁÈ· ÙË ¯ÚËÌ·ÙÔ‰fiÙËÛË Ù˘ ÌÂϤÙ˘, ÙË °ÂÓÈ΋ °Ú·ÌÌ·Ù›· ∞ıÏËÙÈÛÌÔ‡, ÙÔ ÀÔ˘ÚÁÂ›Ô ¶·È‰Â›·˜ Î·È £ÚËÛÎÂ˘Ì¿ÙˆÓ, ÙËÓ ÂÙ·ÈÚ›· Kellogg’s, ηıÒ˜ Î·È ÙȘ ‰ËÌÔÙÈΤ˜ ·˘ÙÔ‰ÈÔÈ΋ÛÂȘ ÙˆÓ ¢‹ÌˆÓ ƒÔ‡‚· Î·È ÃÂÚÛÔÓ‹ÛÔ˘ ∏Ú·ÎÏ›Ԣ. ∂›Û˘, Û˘Ó¤‚·Ï·Ó ·ÔÙÂÏÂÛÌ·ÙÈο ÔÈ Û˘ÓÂÚÁ¿Ù˜: ª·Ú›· ªÈÙÛÒÚË, πˆ¿ÓÓ· ∞ÔÛÙÔÏ¿ÎË, ∞ÁÁÂÏÈ΋ ¶··‰¿ÎË Î·È ¢ËÌ‹ÙÚ˘ ∞ı·Ó·ÛfiÔ˘ÏÔ˜.
µÈ‚ÏÈÔÁÚ·Ê›· 1. Alberti KG, Zimmet PZ. Definition, diagnosis and classification of diabetes mellitus and its complications. Part 1: diagnosis and classification of diabetes mellitus provisional report of a WHO consultation. Diabet Med 1998;15:539-553. Paediatriki 2007;70:24-36
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57. Kafatos A, Verhagen H, Moschandreas J, Apostolaki I, Van Westerop JJ. Mediterranean diet of Crete: foods and nutrient content. J Am Diet Assoc 2000; 100:1487-1493. 58. Sarri ∫O, Tzanakis NE, Linardakis MK, Mamalakis GD, Kafatos AG. Effects of Greek Orthodox Christian Church fasting on serum lipids and obesity. BMC Public Health 2003;3:16. 59. Sarri ∫O, Linardakis MK, Bervanaki FN, Tzanakis NE, Kafatos AG. Greek Orthodox fasting rituals: a hidden characteristic of the Mediterranean diet of Crete. Br J Nutr 2004;92:277-284. 60. Davy BM, Melby CL. The effect of fiber-rich carbohydrates on features of Syndrome X. J Am Diet Assoc 2003;103:86-96.
∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞
ORIGINAL ARTICLE
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√ ÚfiÏÔ˜ ÙˆÓ ¿Ù˘ˆÓ ‚·ÎÙËÚ›ˆÓ ÛÙÔÓ ¯ÚfiÓÈÔ ‚‹¯· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ π.ª. µÂÏÈÛÛ·Ú›Ô˘, ¡.°. ¶··‰fiÔ˘ÏÔ˜, M. ∆ÛÔÏÈ¿, ª. °È·ÓÓ¿ÎË, º. ™·ÍÒÓË-¶··ÁˆÚÁ›Ô˘, ¢.∞. ∫·ÊÂÙ˙‹˜ ¶ÂÚ›ÏË„Ë: ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ Â›Ó·È Ë ‰ÈÂÚ‡ÓËÛË ÙÔ˘ ÚfiÏÔ˘ ÙÔ˘ Ì˘ÎÔÏ¿ÛÌ·ÙÔ˜ Î·È ÙÔ˘ ¯Ï·Ì˘‰›Ô˘ Ù˘ Ó¢ÌÔÓ›·˜ ÛÙÔÓ ·È‰ÈÎfi ÏËı˘ÛÌfi Ì ¯ÚfiÓÈÔ ‹/Î·È ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯· Î·È Ë ·ÍÈÔÏfiÁËÛË Ù˘ ÎÏÈÓÈ΋˜ ·¿ÓÙËÛ˘ ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ ÛÙËÓ ·Ó¿ÏÔÁË ıÂڷ›·. ∂Í‹ÓÙ· ÂÙ¿ ·È‰È¿, ·fi 2,5 ¤ˆ˜ 16 ÂÙÒÓ, Ì ¯ÚfiÓÈÔ ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯· Û˘ÌÂÚÈÂÏ‹ÊıËÛ·Ó ÛÙË ÌÂϤÙË. ∞ÓÙÈÛÒÌ·Ù· ÁÈ· Mycoplasma pneumoniae (M. pneumoniae) Î·È Chlamydia pneumoniae (C. pneumoniae) ·ÓȯÓ‡ıËÎ·Ó Û ‰Â›ÁÌ· ÔÚÔ‡ ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ Ì ‰‡Ô ¢ڤˆ˜ ‰È·‰Â‰Ô̤Ó˜ ÌÂıfi‰Ô˘˜, ÙËÓ ELISA (enzyme-linked immunosorbent assay) Î·È ÙÔÓ ¤ÌÌÂÛÔ ·ÓÔÛÔÊıÔÚÈÛÌfi (IFA). √È ·ÛıÂÓ›˜ Ì ıÂÙÈο ·ÓÙÈÛÒÌ·Ù· IgM ÁÈ· M. pneumoniae ‹ C. pneumoniae Î·È ·Ó·Ú΋ ·ÓÙ·fiÎÚÈÛË ÛÙËÓ ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹, ¤Ï·‚·Ó ÎÏ·ÚÈıÚÔÌ˘Î›ÓË ·fi ÙÔ ÛÙfiÌ·. ∂›ÎÔÛÈ ·ÛıÂÓ›˜ (29,9%) ¤·Û¯·Ó ·fi ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋ Ïԛ̈ÍË Î·È 4 (6,0%) ·fi ÔÍ›· ¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË, ÂÓÒ ¤Ó· ·È‰› ›¯Â Ù·˘Ùfi¯ÚÔÓ· Î·È ÙȘ ‰‡Ô ÏÔÈÌÒÍÂȘ. ∞fi ÙÔ˘˜ 23 ÔÚÔıÂÙÈÎÔ‡˜ ·ÛıÂÓ›˜, ÔÈ 9 (39,1%) ¤·Û¯·Ó ·fi ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋/¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË ÌfiÓÔ, ÂÓÒ ÔÈ ˘fiÏÔÈÔÈ 14 (60,8%) ·fi Û˘Ó‰˘·ÛÌfi ¿ÛıÌ·ÙÔ˜ ‹/Î·È ÚÈÓ›Ùȉ·˜/·Ú·ÚÚÈÓÔÎÔÏ›Ùȉ·˜. ∏ ¯ÔÚ‹ÁËÛË ÎÏ·ÚÈıÚÔÌ˘Î›Ó˘ Ê·›ÓÂÙ·È ˆ˜ ‹Ù·Ó ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙËÓ ÂÍ¿ÏÂÈ„Ë ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ÛÙ· 15 ·È‰È¿ (65,2%), ÂÓÒ 8 ·ÛıÂÓ›˜ (34,7%) ‚ÂÏÙÈÒıËÎ·Ó ¯ˆÚ›˜ Ï‹„Ë Ì·ÎÚÔÏȉÒÓ. ∏ ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋/¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË Â›Ó·È Û˘¯Ó‹ ÛÙ· ·È‰È¿ Ì ¯ÚfiÓÈÔ ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯· Î·È Ê·›ÓÂÙ·È fiÙÈ ·ÓÙ·ÔÎÚ›ÓÂÙ·È Î·Ï¿ ÛÙË ¯ÔÚ‹ÁËÛË ÎÏ·ÚÈıÚÔÌ˘Î›Ó˘.
2Ë ¶·ÓÂÈÛÙËÌȷ΋ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡”, £Ë‚ÒÓ Î·È §Â‚·‰Â›·˜, ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: πˆ¿ÓÓ· ª. µÂÏÈÛÛ·Ú›Ô˘, jane_vel@hotmail.com ∞ÌÊÈÙÚ›Ù˘ 3, ¶·Ï·Èfi º¿ÏËÚÔ, ∆.∫. 175 61, ∞ı‹Ó·
§¤ÍÂȘ ÎÏÂȉȿ: ÕÙ˘Ë Ïԛ̈ÍË, Ì˘ÎfiÏ·ÛÌ·, ·È‰È¿, ¯ÚfiÓÈÔ˜ ‚‹¯·˜.
The role of mycoplasmal/chlamydial infection in chronic cough in children I.M. Velissariou, N.G. Papadopoulos, M. Giannaki, P. Saxoni-Papageorgiou, D.A. Kafetzis Abstract Objectives: The aim of this study was to assess the role of recent mycoplasmal/chlamydial infection in children with a chronic cough, and to evaluate the clinical response to treatment with clarithromycin. Methods: Sixty-seven children, aged 2.5 to 16 years, with chronic or recurrent cough were enrolled in the study. A serum sample from each child was processed for Mycoplasma pneumoniae and Chlamydia pneumoniae antibody status, using two widely approved methods, the enzyme-linked immunosorbent assay (ELISA) and the immunofluorescence assay (IFA). Clarithromycin was prescribed for children with positive IgM M. pneumoniae and/or C. pneumoniae antibodies, according to their response to antiasthmatic treatment. Results: Twenty patients (29.9%) had evidence of recent mycoplasmal infection and 4 (6.0%) of recent chlamydial infection, with one child having both mycoplasmal and chlamydial infection. Of the 23 positive patients, 9 (39.1%) had recent mycoplasmal/chlamydial infection only, while in 14 (60.8%) the atypical bacterial infection was in addition to asthma or rhino-sinusitis. Clarithromycin treatment was effective in diminishing symptoms in 15 (65.2%) children, while 8 patients (34.7%) improved without it. Conclusions: Chronic or recurrent cough in children is commonly due to recent mycoplasmal/ chlamydial infection and tends to respond well to clarithromycin.
2nd Pediatric Clinic, University of Athens “P. & A. Kyriakou” Children’s Hospital, Athens Correspondence: Ioanna M. Velissariou, jane_vel@hotmail.com 3, Amphitritis St., 175 61, Athens, Greece
Key words: Atypical respiratory infection, mycoplasma, chronic cough.
∂ÈÛ·ÁˆÁ‹ ∆Ô Ì˘ÎfiÏ·ÛÌ· Î·È ÙÔ ¯Ï·Ì‡‰ÈÔ Ù˘ Ó¢ÌÔÓ›·˜ ·ÔÙÂÏÔ‡Ó ÁÓˆÛÙÔ‡˜ ·ÈÙÈÔÏÔÁÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ¿Ù˘Ë˜ Ó¢ÌÔÓ›·˜ Û ·È‰È¿ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜, ·ÏÏ¿ Î·È Û Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ
(1,2). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, ‰ÈÂÍ¿ÁÔÓÙ·È ÌÂϤÙ˜ Ô˘ ÛÙfi¯Ô ¤¯Ô˘Ó Ó· ·ÔÛ·ÊËÓ›ÛÔ˘Ó ÙËÓ Èı·Ó‹ Û¯¤ÛË ÌÂٷ͇ ‚ÚÔÁ¯È΋˜ ˘ÂÚ··ÓÙËÙÈÎfiÙËÙ·˜ Î·È ÈÔÁÂÓÒÓ ‹ Ì˘ÎÔÏ·ÛÌ·ÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ (1,3,4). ¶·È‰È·ÙÚÈ΋ 2007;70:37-41
38
π.ª. µÂÏÈÛÛ·Ú›Ô˘ Î·È Û˘Ó.
√ ¯ÚfiÓÈÔ˜ ‚‹¯·˜ ·ÔÙÂÏ› Û¯ÂÙÈο Û˘¯Ófi Û‡Ìو̷ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜, ÚÔηÏÒÓÙ·˜ ÛËÌ·ÓÙÈÎÔ‡ ‚·ıÌÔ‡ ÂÓfi¯ÏËÛË ÛÙÔÓ ¿Û¯ÔÓÙ·, Ì ·ÔÙ¤ÏÂÛÌ· ·˘ÍË̤ÓË ·ÓËÛ˘¯›· ÙˆÓ ÁÔÓ¤ˆÓ Î·È Û˘¯Ó¤˜ ·Ô˘Û›Â˜ ·fi ÙÔ Û¯ÔÏÂ›Ô (5). ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ ·ÈÙÈÔÏÔÁ›· ÙÔ˘, ϤÁÂÙ·È fiÙÈ ÔÔÈ·‰‹ÔÙ ¿ıËÛË ÙÔ˘ Ó‡ÌÔÓ· ÌÔÚ› Ó· ÚÔηϤÛÂÈ ¯ÚfiÓÈÔ ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯· ÛÙ· ·È‰È¿, ·Ó Î·È ÔÈ Û˘¯ÓfiÙÂÚ˜ ·Èٛ˜ Â›Ó·È ÙÔ ¿ÛıÌ·, Ë ÚÈÓ›Ùȉ·, Ë ·Ú·ÚÚÈÓÔÎÔÏ›Ùȉ·, ÔÈ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Î·È ÔÈ ·Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ (5,6). √ ÔÚÈÛÌfi˜ ÙÔ˘ ¯ÚfiÓÈÔ˘ ‚‹¯·, ›Û˘, ÔÈΛÏÂÈ, ·Ó Î·È ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ÂÚ¢ÓËÙ¤˜ ıˆÚÔ‡Ó ¯ÚfiÓÈÔ ÙÔÓ ‚‹¯· Ô˘ ‰È·ÚΛ ÂÚÈÛÛfiÙÂÚÔ ·fi 3 ‚‰ÔÌ¿‰Â˜ (5,6). ∆fiÛÔ ÔÈ Ì˘ÎÔÏ·ÛÌ·ÙÈΤ˜, fiÛÔ Î·È ÔÈ ¯Ï·Ì˘‰È·Î¤˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ÂΉËÏÒÓÔÓÙ·È Ôχ Û˘¯Ó¿ Ì ‚‹¯·. øÛÙfiÛÔ, Ë ÌÂÁ·Ï‡ÙÂÚË ‰˘ÛÎÔÏ›· ÛÙË Û˘Û¯¤ÙÈÛË Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜/¯Ï·Ì˘‰È·Î‹˜ Ïԛ̈͢ Î·È ¯ÚfiÓÈÔ˘ ‚‹¯· ÛÙ· ·È‰È¿ ¤ÁÎÂÈÙ·È ÛÙ· ÌË ÂȉÈο, Û˘¯Ó¿ Û˘ÌÙÒÌ·Ù· Î·È ÛÙËÓ ÂÚÈÏÔÎfiÙËÙ· Ù˘ ‚·ÎÙËÚÈÔÏÔÁÈ΋˜ ‰È¿ÁÓˆÛ˘ (7-10). √ ÛÎÔfi˜ Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜ ‹Ù·Ó Ó· ηıÔÚÈÛÙ› fiÛÔ Û˘¯Ó¿ Û¯ÂÙ›˙ÔÓÙ·È ÔÈ Ì˘ÎÔÏ·ÛÌ·ÙÈΤ˜/¯Ï·Ì˘‰È·Î¤˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Ì ¯ÚfiÓÈÔ ‚‹¯· ÛÙ· ·È‰È¿ Î·È Î·Ù¿ fiÛÔ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ ·ÈÙÈÔÏÔÁÈ΋ ıÂڷ›·.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∞ÛıÂÓ›˜ ∆Ô ¿ÚıÚÔ ·˘Ùfi ·ÔÙÂÏ› ̤ÚÔ˜ Ì›·˜ ÚÔÔÙÈ΋˜ ÌÂϤÙ˘ 67 ·È‰ÈÒÓ Ì ¯ÚfiÓÈÔ ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯·, Ù· ÔÔ›· ·ÚÔ˘ÛÈ¿ÛÙËÎ·Ó ÛÙ· ∂͈ÙÂÚÈο π·ÙÚ›· ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ “∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡” ÛÙÔ ‰È¿ÛÙËÌ· ÂÓfi˜ ¤ÙÔ˘˜. √È ·ÛıÂÓ›˜ ·˘ÙÔ› ·ÎÔÏÔ‡ıËÛ·Ó ¤Ó·Ó ‰È·ÁÓˆÛÙÈÎfi Î·È ıÂڷ¢ÙÈÎfi ·ÏÁfiÚÈıÌÔ, Ô ÔÔ›Ô˜ ÂÚÈÂÏ¿Ì‚·Ó ÂÓ‰Âϯ‹ ¤ÏÂÁ¯Ô Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (Ù·Ï·ÓÙÔÛÈÌÂÙÚ›· Î·È ÛÈÚÔ̤ÙÚËÛË ÚÈÓ Î·È ÌÂÙ¿ ·fi ‚ÚÔÁ¯Ô‰È·ÛÙÔÏ‹ Î·È ‚ÚÔÁ¯È΋ ÚfiÎÏËÛË ·‰ÂÓÔÛ›Ó˘, fiÔ˘ ¯ÚÂÈ¿ÛÙËÎÂ) Î·È ·ÚÔ˘ÛÈ¿˙ÂÙ·È ·ÏÏÔ‡. ø˜ ¯ÚfiÓÈÔ˜ ‚‹¯·˜ ÔÚ›ÛÙËÎÂ Ô ‚‹¯·˜ Ì ‰È¿ÚÎÂÈ· ¿Óˆ ÙˆÓ 3 Û˘Ó¯ÒÓ Â‚‰ÔÌ¿‰ˆÓ, ÂÓÒ ˆ˜ ˘ÔÙÚÔÈ¿˙ˆÓ, Ô ‚‹¯·˜ ‰È¿ÚÎÂÈ·˜ ÙÔ˘Ï¿¯ÈÛÙÔÓ 2 ‚‰ÔÌ¿‰ˆÓ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ 3 ÂÂÈÛfi‰È· ηı’ ¤ÙÔ˜. ∞ÛıÂÓ›˜ ¿Û¯ÔÓÙ˜ ·fi ÁÓˆÛÙ¤˜ ·ı‹ÛÂȘ Ô˘ ÚÔηÏÔ‡Ó ¯ÚfiÓÈÔ ‚‹¯·, fiˆ˜ Ë ÈÓÔ΢ÛÙÈ΋ ÓfiÛÔ˜, Ë ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋ ‰˘ÛÏ·Û›·, ‚ÚÔÁ¯ÈÂÎٷۛ˜, Î.Ï., ·ÔÎÏ›ÛÙËÎ·Ó ·fi ÙË ÌÂϤÙË, fiˆ˜ Î·È ·È‰È¿ ÌÂ Ù˘ÈÎfi ÈÛÙÔÚÈÎfi Î·È Û˘Ìو̷ÙÔÏÔÁ›· ¿ÛıÌ·ÙÔ˜ (Û˘ÚÈÁÌfi˜, ·Ú¿Ù·ÛË ÂÎÓÔ‹˜), ‹ Ì ·ÎÙÈÓÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ÏÔ‚Ò‰Ô˘˜ Ó¢ÌÔÓ›·˜. ∏ ¤Ú¢ӷ ÂÁÎÚ›ıËΠ·fi ÙËÓ ∂ÈÙÚÔ‹ ¢ÂÔÓÙÔÏÔÁ›·˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ, ÂÓÒ ¤ÁÁÚ·ÊË Û˘ÁηٿıÂÛË Û˘ÌÌÂÙÔ¯‹˜ ˙ËÙ‹ıËΠ·fi fiÏÔ˘˜ ÙÔ˘˜ ÎˉÂÌfiÓ˜ ÙˆÓ ·ÛıÂÓÒÓ. ∫·Ù¿ ÙËÓ Â›ÛÔ‰Ô ÛÙË ÌÂϤÙË, fiÏ· Ù· ·È‰È¿ Î·È ÔÈ ÁÔÓ›˜ ÙÔ˘˜ Û˘ÌÏ‹ÚˆÛ·Ó ¤Ó· ÏÂÙÔÌÂÚ¤˜ ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ fiÛÔÓ ·ÊÔÚ¿ ÙË ‰È¿ÚÎÂÈ· Î·È ÙËÓ ¤ÓÙ·ÛË ÙˆÓ Û˘Ìو̿وÓ, ÂÓÒ ·ÎÔÏÔ‡ıËÛÂ Ê˘ÛÈ΋ ÂͤٷÛË Î·È Ì¤ÙÚËÛË ÙˆÓ ·ÓıÚˆÔÌÂÙÚÈÎÒÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ. √ ¤ÏÂÁ¯Ô˜ Ù˘ ·ÙÔ›·˜ ÂÚÈÂÏ¿Ì‚·Ó Paediatriki 2007;70:37-41
‰ÂÚÌ·ÙÈΤ˜ ‰ÔÎÈ̷ۛ˜ Ó˘ÁÌÔ‡ Û 8 ÎÔÈÓ¿ ·ÏÏÂÚÁÈÔÁfiÓ· (ÂÏÈ¿, ·ÚÈÂÙ¿ÚÈ·, ·ÏÙÂÚÓ¿ÚÈ·, ÔÈÎȷ΋ ÛÎfiÓË, Ì›ÁÌ· ÁÚ·ÛȉÈÒÓ, Á¿Ù·, Û·ÏÔ˜ Î·È Ì›ÁÌ· ‰¤ÓÙÚˆÓ) (11), ÂÓÒ Ô ¤ÏÂÁ¯Ô˜ ·ÓÙÈÛˆÌ¿ÙˆÓ ÁÈ· M. pneumoniae Î·È C. pneumoniae ¤ÁÈÓ Û ÌÔÓfi ‰Â›ÁÌ· ÔÚÔ‡. ∞Ó›¯Ó¢ÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ÁÈ· M. pneumoniae Î·È C. pneumoniae ™ÙË ÌÂϤÙË ·˘Ù‹ ÂÊ·ÚÌfiÛÙËÎ·Ó ‰‡Ô ¢ڤˆ˜ ‰È·‰Â‰Ô̤Ó˜ ̤ıÔ‰ÔÈ ·Ó›¯Ó¢Û˘ ·ÓÙÈۈ̿وÓ, Ë ELISA Î·È Ô ¤ÌÌÂÛÔ˜ ·ÓÔÛÔÊıÔÚÈÛÌfi˜. ∞Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ¤Ó·˜ ÌÂÁ¿ÏÔ˜ ·ÚÈıÌfi˜ ·ÛıÂÓÒÓ Î·ÙÔÈÎÔ‡Û ÂÎÙfi˜ ∞ıËÓÒÓ, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ÌËÓ Â›Ó·È ¿ÓÙ· ÂÊÈÎÙ‹ Ë Ï‹„Ë ‰Â‡ÙÂÚÔ˘ ‰Â›ÁÌ·ÙÔ˜ ÔÚÔ‡, ÔfiÙÂ Ë ·Ó›¯Ó¢ÛË ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÂÚÈÔÚ›ÛÙËΠ۠¤Ó· ÌfiÓÔ ‰Â›ÁÌ·. ¢È¿ÁÓˆÛË ÔÍ›·˜ Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜ ‹ ¯Ï·Ì˘‰È·Î‹˜ Ïԛ̈͢ Ù¤ıËΠÛÙȘ ÂÚÈÙÒÛÂȘ fiÔ˘ Î·È ÔÈ ‰‡Ô ̤ıÔ‰ÔÈ ¤‰ÈÓ·Ó Û·ÊÒ˜ ıÂÙÈÎfi ·ÔÙ¤ÏÂÛÌ·. £ÂÙÈÎÔ› Ù›ÙÏÔÈ IgM ·ÓÙÈÛˆÌ¿ÙˆÓ ÁÈ· Ì˘ÎfiÏ·ÛÌ· ‹ ¯Ï·Ì‡‰ÈÔ Ù˘ Ó¢ÌÔÓ›·˜ ıˆڋıËÎ·Ó ÂÓ‰ÂÈÎÙÈÎÔ› ÚfiÛÊ·Ù˘ ¿Ù˘Ë˜ Ïԛ̈͢. ÃÚËÛÈÌÔÔÈ‹ıËÎÂ Ë ELISA Ù˘ NovaTec Immunodiagnostica, Dietzenbach, GmBH, Germany (·ÚÈıÌfi˜ ÚÔ˚fiÓÙÔ˜ CHLG0510) ÁÈ· ·ÓÙÈÛÒÌ·Ù· IgG Î·È IgM, ÙfiÛÔ ÙÔ˘ Ì˘ÎÔÏ¿ÛÌ·ÙÔ˜, fiÛÔ Î·È ÙÔ˘ ¯Ï·Ì˘‰›Ô˘ Ù˘ Ó¢ÌÔÓ›·˜, Ì ̤ÙÚÈ· ¢·ÈÛıËÛ›· (<70%) Î·È Î·Ï‹ ÂȉÈÎfiÙËÙ· (70-90%) Î·È ÁÈ· ÙÔ˘˜ ‰‡Ô ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜ (12). ∆· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂıfi‰Ô˘ ·˘Ù‹˜ ÂÎÊÚ¿˙ÔÓÙ·È Û ÌÔÓ¿‰Â˜ NovaTec (NTU), ˆ˜ ÂÍ‹˜: ·ÌÊ›‚ÔÏË ˙ÒÓË: 9-11 ¡∆U, ·ÚÓËÙÈÎfi ·ÔÙ¤ÏÂÛÌ·: <9 NTU, ıÂÙÈÎfi ·ÔÙ¤ÏÂÛÌ·: >11 NTU. ∞Ó¿ÏÔÁË ‰È·ÁÓˆÛÙÈ΋ ·ÎÚ›‚ÂÈ· ÂÈÙ˘Á¯¿ÓÂÙ·È Î·È Ì ÙÔÓ ·ÓÔÛÔÊıÔÚÈÛÌfi Ù˘ Biognost, Zagreb, Croatia (antibody assay E0001), ÁÈ· IgG Î·È IgM Î·È ÙˆÓ ‰‡Ô ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ (13). ∏ ̤ıÔ‰Ô˜ ·˘Ù‹ ÂÊ·ÚÌfiÛÙËΠÂÈÎÔ˘ÚÈο ÚÔ˜ ÙËÓ ELISA, ÁÈ· ÙËÓ ÂȂ‚·›ˆÛË ÙˆÓ ıÂÙÈÎÒÓ Î·È ·ÚÓËÙÈÎÒÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Î·È ÁÈ· ÙË ‰È·Ï‡ηÓÛË Ù˘ ·ÌÊ›‚ÔÏ˘ ˙ÒÓ˘. ∞Ó Î·È ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ÂÚ¢ÓËÙ¤˜ ‰ÂÓ ‚·Û›˙Ô˘Ó ÙË ‰È¿ÁÓˆÛË ÛÙÔÓ IFA, ˆÛÙfiÛÔ Ë Ì¤ıÔ‰Ô˜ ·˘Ù‹ ¤¯ÂÈ 70% ¢·ÈÛıËÛ›· Î·È 70-90% ÂȉÈÎfiÙËÙ· Î·È ÁÈ· ÙÔ˘˜ ‰‡Ô ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜ (12,13). ∂Í¿ÏÏÔ˘, ˘¿Ú¯Ô˘Ó ÂÚ¢ÓËÙ¤˜ Ô˘ ıˆÚÔ‡Ó ÙÔÓ ·ÓÔÛÔÊıÔÚÈÛÌfi ˆ˜ ÙË Ì¤ıÔ‰Ô ÂÎÏÔÁ‹˜ ÁÈ· ÙË ‰È¿ÁÓˆÛË ÙÔ˘ C. pneumoniae (14). £ÂÙÈÎfi˜ ıˆÚÂ›Ù·È Ô Ù›ÙÏÔ˜ >1/160 ÁÈ· ÔÔÈ·‰‹ÔÙ ٿÍË ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ. £Âڷ¢ÙÈÎfi ÚˆÙfiÎÔÏÏÔ ∏ ·Ú¯È΋ ıÂڷ¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË ÁÈ· fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜, ·ÓÂÍ¿ÚÙËÙ· ·fi Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ÂÍÂÙ¿ÛˆÓ, Û˘Ó›ÛÙ·Ù·È Û ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹ ÁÈ· 3 ‚‰ÔÌ¿‰Â˜ (Û˘Ó‰˘·ÛÌfi˜ ÂÈÛÓÂfiÌÂÓÔ˘ ‚2 ‰ÈÂÁ¤ÚÙË Ì·ÎÚ¿˜ ‰È¿ÚÎÂÈ·˜ ‰Ú¿Û˘ Î·È ÛÙÂÚÔÂȉԇ˜ Û ‰fiÛË ·Ó¿ÏÔÁË ÚÔ˜ ÙËÓ ËÏÈΛ·, ÁÈ· ̤ÙÚÈÔ, ÂÈ̤ÓÔÓ ¿ÛıÌ·). ∫·Ù¿ ÙËÓ Â·ÓÂͤٷÛË, ÔÈ ÂÚ¢ÓËÙ¤˜ ·ÍÈÔÏÔÁÔ‡Û·Ó ÙËÓ ÎÏÈÓÈ΋ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂڷ›·, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ٷ ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÙˆÓ M. pneumoniae Î·È C. pneumoniae. √È ·ÛıÂÓ›˜ ÂΛÓÔÈ Ô˘ ‰ÂÓ ·Ó¤ÊÂÚ·Ó ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Ì ÙËÓ ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹ Î·È ÙˆÓ ÔÔ›ˆÓ Ù· ·ÓÙÈÛÒÌ·Ù· ‹Ù·Ó ÂÓ‰ÂÈÎÙÈο ÔÍ›·˜ Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜/¯Ï·Ì˘‰È·Î‹˜ Ïԛ̈͢, ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó ÛÙË Û˘Ó¤¯ÂÈ· Ì ¯ÔÚ‹ÁËÛË ÎÏ·ÚÈıÚÔÌ˘Î›Ó˘ ·fi ÙÔ ÛÙfiÌ·, Û ‰fiÛË 15 mg/kg/Ë̤ڷ ÁÈ· 21 Ë̤Ú˜. ∂›Û˘, ÔÈ ·ÛıÂÓ›˜ Ô˘ ›¯·Ó ÛÙÔȯ›· ¿ÛıÌ·ÙÔ˜ ÛÙÔÓ ÏÂÈÙÔ˘ÚÁÈÎfi ¤ÏÂÁ¯Ô Ù˘ ·Ó·ÓÔ‹˜, ηıÒ˜ Î·È ·ÓÙÈÛÒÌ·Ù· ÂÓ‰ÂÈÎÙÈο ÔÍ›·˜ Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜/¯Ï·Ì˘‰È·Î‹˜ Ïԛ̈͢, ¤Ï·‚·Ó ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÎÏ·ÚÈıÚÔÌ˘Î›ÓË,
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ª˘ÎfiÏ·ÛÌ· Î·È ¯ÚfiÓÈÔ˜ ‚‹¯·˜
ÂÓÒ ÂΛÓÔÈ Ô˘ ›¯·Ó Â˘Ú‹Ì·Ù· ÚÈÓ›Ùȉ·˜/·Ú·ÚÚÈÓÔÎÔÏ›Ùȉ·˜ ÛÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ·Ú·ÚÚÈÓ›ˆÓ ÎfiÏˆÓ Î·È ÔÍ›· Ïԛ̈ÍË ·fi ¿Ù˘· ÌÈÎÚfi‚È·, ¤Ï·‚·Ó ÎÏ·ÚÈıÚÔÌ˘Î›ÓË Î·È ÙÔÈο ÛÙÂÚÔÂȉ‹. ∆ÚÂȘ ‚‰ÔÌ¿‰Â˜ ·ÚÁfiÙÂÚ·, ÔÈ ·ÛıÂÓ›˜ ·ÓÂÎÙÈÌ‹ıËÎ·Ó Î·È Ë ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘˜ ·ÎÔÏÔ‡ıˆ˜, ÂÍ·ÙÔÌÈ·ÙËÎÂ.
∞ÔÙÂϤÛÌ·Ù· ∂Í‹ÓÙ· ÂÙ¿ ·È‰È¿ Û˘ÌÌÂÙ›¯·Ó ÛÙË ÌÂϤÙË ·˘Ù‹, ·fi 2,5 ¤ˆ˜ 16 ÂÙÒÓ, Ì ̤ÛË ËÏÈΛ· Ù· 7 ¤ÙË. ∆ÚÈ¿ÓÙ· ‰‡Ô (47,8%) ‹Ù·Ó ·ÁfiÚÈ· Î·È 35 (52,2%) ÎÔÚ›ÙÛÈ·. ™·Ú¿ÓÙ· ‰‡Ô ·ÛıÂÓ›˜ (62,7%) ¤·Û¯·Ó ·fi ¯ÚfiÓÈÔ Î·È 25 (37,3%) ·fi ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯·. ™·Ú¿ÓÙ· ÙÚ›· ·È‰È¿ (64,2%) ηÙÔÈÎÔ‡Û·Ó ÛÙËÓ ∞ı‹Ó·, 23 (34,3%) Û ËÌÈ·ÛÙÈΤ˜ Î·È ·ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜, ÂÓÒ ¤Ó· (1,5%) ηÙÔÈÎÔ‡Û ÛÙËÓ ∫‡ÚÔ. ŸÏ· ‹Ù·Ó Ï‹Úˆ˜ ÂÌ‚ÔÏÈ·Ṳ̂ӷ ÁÈ· ÙËÓ ËÏÈΛ· ÙÔ˘˜ Î·È Û ηӤӷ ‰ÂÓ ·Ó‚ڤıËÎ·Ó ÛËÌ›· ˘ÔÛÈÙÈÛÌÔ‡. ∏ ‰È¿ÁÓˆÛË Ù˘ ÔÍ›·˜ Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜ ‹ ¯Ï·Ì˘‰È·Î‹˜ Ïԛ̈͢ ·Ô‰fiıËΠÌfiÓÔ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ıÂÙÈÎfi Ù›ÙÏÔ IgM ·ÓÙÈۈ̿وÓ, ÂÓÒ ÔÈ Ù›ÙÏÔÈ ÙˆÓ IgG ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ ‰ÂÓ ·Ó¤‰ÂÈÍ·Ó ÂÈÚfiÛıÂÙ˜ ÂÚÈÙÒÛÂȘ ¿Ù˘Ë˜ Ïԛ̈͢. ™ÙÔÓ ¶›Ó·Î· 1, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ù· Û˘ÁÎÂÓÙÚˆÙÈο ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ ÙˆÓ ·ÛıÂÓÒÓ Î·È ÁÈ· ÙÔ˘˜ ‰‡Ô ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜. ∞fi ÙÔ˘˜ 67 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘, ÔÈ 20 (29,9%) ¤·Û¯·Ó ·fi ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋ Ïԛ̈ÍË Î·È ÔÈ 4 (6,0%) ·fi ¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË, ÂÓÒ ¤Ó·˜ ·ÛıÂÓ‹˜ ›¯Â Ù·˘Ùfi¯ÚÔÓ·, ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋ Î·È ¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË. ∫·Ó¤Ó·˜ ‰ÂÓ ·ÚÔ˘Û›·˙ ÛÙÔȯ›· ÂÍ·Óı‹Ì·ÙÔ˜, ·ÚıÚ›Ùȉ·˜, ·ÈÌÔÏ˘ÙÈ΋˜ ·Ó·ÈÌ›·˜ ‹ ¿ÏÏˆÓ ÂΉËÏÒÛÂˆÓ Ïԛ̈͢ ·fi ¿Ù˘·. ∆· ·ÔÙÂϤÛÌ·Ù· Ô˘ ·Ú·Ù›ıÂÓÙ·È ÛÙË Û˘Ó¤¯ÂÈ·, ·Ó·Ê¤ÚÔÓÙ·È ÌfiÓÔ ÛÙÔ˘˜ 23 ÔÚÔıÂÙÈÎÔ‡˜ ·ÛıÂÓ›˜ ÁÈ· Ì˘ÎfiÏ·ÛÌ· ‹ ¯Ï·Ì‡‰È·. ∞fi ÙÔ˘˜ 23 ·˘ÙÔ‡˜ ·ÛıÂÓ›˜, ÔÈ 9 (39,1%) ¤·Û¯·Ó ·fi ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋/ ¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË ÌfiÓÔ. ™ÙÔ˘˜ ˘fiÏÔÈÔ˘˜ 14 (60,8%) ·Ó‚ڤıËÎ·Ó ÂÚÈÛÛfiÙÂÚ˜ ·fi Ì›· ·Èٛ˜ ÁÈ· ÙÔÓ ‚‹¯· ÙÔ˘˜. √È 6 ¤·Û¯·Ó Î·È ·fi ¿ÛıÌ· (Ì ‚¿ÛË Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ÏÂÈÙÔ˘ÚÁÈÎÒÓ ‰ÔÎÈÌ·ÛÈÒÓ Ù˘ ·Ó·ÓÔ‹˜ Î·È ÙËÓ ·ÓÙ·fiÎÚÈÛË ÛÙËÓ ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹), ÔÈ ¶›Ó·Î·˜ 1. ∞ÔÙÂϤÛÌ·Ù· ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ ÁÈ· M. pneumoniae / C. pneumoniae ∞ÓÔÛÔÛÊ·ÈÚ›Ó˜ M. pneumoniae IgG M. pneumoniae IgM C. pneumoniae IgG C. pneumoniae IgM
¶ÂÚÈÛÙ·ÙÈο (%) › 67 ·ÛıÂÓÒÓ 32 (47,8%) 20 (29,9%) 1 (1,5%) 4 (6,0%)
7 ›¯·Ó Î·È ÚÈÓ›Ùȉ·/ ·Ú·ÚÚÈÓÔÎÔÏ›Ùȉ· (Ì ‚¿ÛË ÙËÓ ‡·ÚÍË ÚÈÓÈ΋˜ Û˘ÌÊfiÚËÛ˘, ÔÓÔÎÂÊ¿ÏÔ˘, ¢·ÈÛıËÛ›·˜ ÛÙËÓ „ËÏ¿ÊËÛË ÙˆÓ ·Ú·ÚÚÈÓ›ˆÓ ÎfiÏˆÓ Î·È ˘ÁÚ·ÂÚÈÎÔ‡ ÂȤ‰Ô˘ ÛÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ·Ú·ÚÚÈÓ›ˆÓ), ÂÓÒ ¤Ó·˜ ÂÌÊ¿ÓÈ˙Â Î·È ˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Â΂ϷÛÙ‹ÛÂˆÓ (Ô˘ ‰È·ÈÛÙÒıËΠÌÂÙ¿ ·fi ·Ú·ÔÌ‹ ÙÔ˘ ·ÛıÂÓÔ‡˜ Û ˆÙÔÚÈÓÔÏ·Ú˘ÁÁÔÏfiÁÔ). ∏ ̤ÛË ËÏÈΛ· ÙˆÓ ·È‰ÈÒÓ ‹Ù·Ó 7,3 ¤ÙË (±3,1) Î·È Ë Ì¤ÛË ‰È¿ÚÎÂÈ· ÙÔ˘ ‚‹¯· ÚÈÓ ÙËÓ Â›ÛÔ‰fi ÙÔ˘˜ ÛÙË ÌÂϤÙË, 60,4 Ë̤Ú˜ (±22,5). ™¯Â‰fiÓ fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ (97%) ıˆÚÔ‡Û·Ó ÙÔÓ ‚‹¯· ˆ˜ ÙÔ ÈÔ ÂÓÔ¯ÏËÙÈÎfi Û‡Ìو̷ Ô˘ ÂÌÊ¿ÓÈ˙·Ó. ∞fi Ù· 23 ·È‰È¿, Ù· 10 (43,3%) ›¯·Ó ÙÔ˘Ï¿¯ÈÛÙÔÓ Ì›· ıÂÙÈ΋ ‰ÂÚÌ·ÙÈ΋ ‰ÔÎÈÌ·Û›· Ó˘ÁÌÔ‡, Î·È ˆ˜ ÂÎ ÙÔ‡ÙÔ˘ ıˆڋıËÎ·Ó ·ÙÔÈο. √È ÁÔÓ›˜ ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ ·Ó¤ÊÂÚ·Ó ·ÚfiÌÔÈ· ÂÂÈÛfi‰È· ‚‹¯· Î·È ÛÙÔ ·ÚÂÏıfiÓ, Ù· ÔÔ›· ›¯·Ó ·Ô‰Ôı› Û ‚ÚÔÁ¯›Ùȉ· ‹ Ó¢ÌÔÓ›·, ÂÓÒ 7 ·fi Ù· ·È‰È¿ ·˘Ù¿ ›¯·Ó ¯ÚÂÈ·ÛÙ› ÓÔÛËÏ›· Û ·Ó¿ÏÔÁ˜ ÂÚÈÙÒÛÂȘ. ∞˘Ù¿ Ù· ·È‰È¿, ηıÒ˜ Î·È ¤Ó· ·fi Ù· ˘fiÏÔÈ· (Û‡ÓÔÏÔ 11 ·ÛıÂÓ›˜ - 47,8%) ·Ó¤ÊÂÚ·Ó Âԯȷ΋ ‰È·Î‡Ì·ÓÛË ÛÙÔÓ ‚‹¯· ÙÔ˘˜, Ì ¤Í·ÚÛË ÙÔ˘˜ ¯ÂÈÌÂÚÈÓÔ‡˜ Ì‹Ó˜. Ÿˆ˜ ·Ó·Ê¤ÚıËΠÚÔËÁÔ˘Ì¤Óˆ˜, Î·È ÔÈ 23 ·ÛıÂÓ›˜ ¤Ï·‚·Ó ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹ ˆ˜ ÚÒÙÔ ‚‹Ì· ·ÓÙÈÌÂÙÒÈÛ˘. ∫·Ù¿ ÙÔÓ Â·Ó¤ÏÂÁ¯Ô, Ô ‚‹¯·˜ ›¯Â ˘Ô¯ˆÚ‹ÛÂÈ ÂÓÙÂÏÒ˜ ÛÙÔ˘˜ 8 ·ÛıÂÓ›˜ (34,7%), ÔfiÙ ‰ÂÓ ¯ÔÚËÁ‹ıËΠÎÏ·ÚÈıÚÔÌ˘Î›ÓË. ∆ÚÂȘ ·fi ·˘ÙÔ‡˜ ›¯·Ó ÛÙÔȯ›· ·fiÊڷ͢ ηٿ ÙȘ ‰ÔÎÈ̷ۛ˜ ÏÂÈÙÔ˘ÚÁÈÎÔ‡ ÂϤÁ¯Ô˘ Ù˘ ·Ó·ÓÔ‹˜, ÔfiÙÂ Û˘Ó¤¯ÈÛ·Ó ÙËÓ ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹, ÂÓÒ ÔÈ ˘fiÏÔÈÔÈ 5 ›¯·Ó Â˘Ú‹Ì·Ù· ·Ú·ÚÚÈÓÔÎÔÏ›Ùȉ·˜ ÛÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ·Ú·ÚÚÈÓ›ˆÓ ÎfiψÓ, ÔfiÙÂ Û˘Ó¤¯ÈÛ·Ó ÙËÓ ·ÁˆÁ‹ ÙÔ˘˜ Ì ÙÔÈ΋ ¯Ú‹ÛË ÚÈÓÈÎÔ‡ ÛÙÂÚÔÂȉԇ˜ Î·È ¿ÏÏ˘ ·ÓÙÈ‚›ˆÛ˘ ·fi ÙÔ ÛÙfiÌ·. √È ˘fiÏÔÈÔÈ 15 ·ÛıÂÓ›˜ (65,2%) ‰ÂÓ ‚ÂÏÙÈÒıËÎ·Ó Ì ÙËÓ ·ÓÙÈ·ÛıÌ·ÙÈ΋ ıÂڷ›·, ÔfiÙ ¤Ï·‚·Ó ÎÏ·ÚÈıÚÔÌ˘Î›ÓË Û ‰Â‡ÙÂÚÔ ¯ÚfiÓÔ. √ÎÙÒ ¤Ï·‚·Ó ÌfiÓÔ ÎÏ·ÚÈıÚÔÌ˘Î›ÓË, ÂÓÒ 5 ›¯·Ó Â˘Ú‹Ì·Ù· ·fiÊڷ͢ ηٿ ÙË Ù·Ï·ÓÙÔÛÈÌÂÙÚ›· Î·È ÙË ÛÈÚÔ̤ÙÚËÛË, ÔfiÙ ¤Ï·‚·Ó ÎÏ·ÚÈıÚÔÌ˘Î›ÓË Î·È ·ÓÙÈ·ÛıÌ·ÙÈο Î·È 2 ›¯·Ó ·ıÔÏÔÁÈ΋ ·ÎÙÈÓÔÁÚ·Ê›· ·Ú·ÚÚÈÓ›ˆÓ, ÔfiÙ ¤Ï·‚·Ó ÎÏ·ÚÈıÚÔÌ˘Î›ÓË Î·È ÙÔÈÎfi ÚÈÓÈÎfi ÛÙÂÚÔÂȉ¤˜. ∫·Ù¿ ÙËÓ Â·ÓÂͤٷÛË, Ô ‚‹¯·˜ ›¯Â ˘Ô¯ˆÚ‹ÛÂÈ Û fiÏ· Ù· ·È‰È¿ ÂÎÙfi˜ ·fi ¤Ó·. ∆Ô ·È‰› ·˘Ùfi ¤·ÈÚÓ ÌfiÓÔ ÎÏ·ÚÈıÚÔÌ˘Î›ÓË, ˆÛÙfiÛÔ Â›¯Â ¤Ó·Ó ˘ÔÏÂÈÌÌ·ÙÈÎfi ‚‹¯· ÛÙȘ 3 ‚‰ÔÌ¿‰Â˜ ıÂڷ›·˜. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, ·ÔÊ·Û›ÛÙËÎÂ Ë Û˘Ó¤¯ÈÛË Ù˘ ·ÁˆÁ‹˜ ÁÈ· ¿ÏϘ 3 ‚‰ÔÌ¿‰Â˜, ÔfiÙÂ Î·È Ô ‚‹¯·˜ ˘Ô¯ÒÚËÛÂ. √ ¶›Ó·Î·˜ 2 ÂÚÈÁÚ¿ÊÂÈ ÙË ıÂڷ¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Î·È ·¿ÓÙËÛË fiÏˆÓ ÙˆÓ ·ÛıÂÓÒÓ Ì ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋/ ¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË. ¶·È‰È·ÙÚÈ΋ 2007;70:37-41
40
π.ª. µÂÏÈÛÛ·Ú›Ô˘ Î·È Û˘Ó.
™˘˙‹ÙËÛË ∏ ÌÂϤÙË ·˘Ù‹ Âȯ›ÚËÛ ӷ ·ÍÈÔÏÔÁ‹ÛÂÈ ÙÔÓ ÚfiÏÔ ÙÔ˘ Ì˘ÎÔÏ¿ÛÌ·ÙÔ˜ Î·È ÙÔ˘ ¯Ï·Ì˘‰›Ô˘ Ù˘ Ó¢ÌÔÓ›·˜ ÛÙÔÓ ¯ÚfiÓÈÔ Î·È ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯· ÙÔ˘ ·È‰ÈÎÔ‡ ÏËı˘ÛÌÔ‡, ηıÒ˜ Î·È ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ıÂڷ›·˜ ÙÔ˘˜ Ì ̷ÎÚÔÏ›‰Â˜. ∏ Ì·ÎÚ¿ ‰È¿ÚÎÂÈ· ÙÔ˘ ‚‹¯· (̤ÛË ‰È¿ÚÎÂÈ· 2 Ì‹Ó˜), ·ÎfiÌË Î·È fiÙ·Ó Ë ¤ÓÙ·ÛË ‰ÂÓ ‹Ù·Ó ÌÂÁ¿ÏË, Ê¿ÓËΠӷ ÎÔ˘Ú¿˙ÂÈ ÙÔ˘˜ ·ÛıÂÓ›˜ Î·È ÙÔ˘˜ Û˘ÁÁÂÓ›˜ ÙÔ˘˜. ÕÏψÛÙÂ, ۯ‰fiÓ fiÏÔÈ ÔÈ ÁÔÓ›˜ (97%) ·Ó¤ÊÂÚ·Ó fiÙÈ Ô ‚‹¯·˜ ‹Ù·Ó ÙÔ ÈÔ ÂÓÔ¯ÏËÙÈÎfi Û‡Ìو̷ ÙˆÓ ·È‰ÈÒÓ ÙÔ˘˜. √ ÚfiÏÔ˜ Ù˘ ÔÍ›·˜ Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜/¯Ï·Ì˘‰È·Î‹˜ Ïԛ̈͢ Û ·È‰È¿ Ì ·ÚÔ͇ÓÛÂȘ ¿ÛıÌ·ÙÔ˜ ‰ÈÂÚ¢ӋıËΠÚfiÛÊ·Ù·. √Í›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋ Ïԛ̈ÍË ·Ó‚ڤıËΠÛÙÔ 20% ÙˆÓ ·ÚÔ͇ÓÛÂˆÓ ¿ÛıÌ·ÙÔ˜, ÂÓÒ ÔÍ›· ¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË ÛÙÔ 3,4% ÙˆÓ ÂÚÈÙÒÛˆÓ. ∂ÈϤÔÓ, ÙÔ ÔÛÔÛÙfi ÔÍ›·˜ Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜ Ïԛ̈͢ ÛÙÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¿ÛıÌ·ÙÔ˜ Êı¿ÓÂÈ ÙÔ 50% (15). ∏ ÌÂϤÙË ·˘Ù‹ ·Ó¤‰ÂÈÍ ·Ú·Ï‹ÛÈ· ÔÛÔÛÙ¿ ÔÍ›·˜ Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜/¯Ï·Ì˘‰È·Î‹˜ Ïԛ̈͢ (34,3%) Û ÂÚÈÙÒÛÂȘ ¯ÚfiÓÈÔ˘ ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙÔ˜ ‚‹¯·, ¯ˆÚ›˜ ÚÔËÁËı›۷ ‰È¿ÁÓˆÛË ¿ÛıÌ·ÙÔ˜. ¶Ú¤ÂÈ, ˆÛÙfiÛÔ Ó· ·Ó·ÊÂÚı› fiÙÈ 6 ·È‰È¿ (26%) Ì ıÂÙÈο IgM ·ÓÙÈÛÒÌ·Ù· ÁÈ· M. pneumoniae, ÂÌÊ¿ÓÈÛ·Ó ÛÙË Û˘Ó¤¯ÂÈ· ÛÙÔȯ›· ¿ÛıÌ·ÙÔ˜ ÛÙȘ ‰ÔÎÈ̷ۛ˜ ÏÂÈÙÔ˘ÚÁÈÎÔ‡ ÂϤÁ¯Ô˘ Ù˘ ·Ó·ÓÔ‹˜. ∂›Û˘, ·Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ¿ÏϘ ÌÂϤÙ˜ ·È‰ÈÒÓ Ì ¯ÚfiÓÈÔ ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯·, ·Ó¤‰ÂÈÍ·Ó ÌÈÎÚfi-
ÙÂÚÔ ÔÛÔÛÙfi ÏÔÈÌÒÍÂˆÓ ·fi ¿Ù˘· ·’ fi,ÙÈ Ë ‰È΋ Ì·˜ (6,16). ∞ÍÈÔÛËÌ›ˆÙÔ Â›Ó·È ÙÔ ÁÂÁÔÓfi˜ fiÙÈ 10 ·ÛıÂÓ›˜ (43,4%) Ì ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋ Ïԛ̈ÍË, ·Ó¤ÊÂÚ·Ó ·Ó¿ÏÔÁ· ÂÂÈÛfi‰È· ‚‹¯· ÛÙÔ ·ÚÂÏıfiÓ, ÂÓÒ ÔÈ 7 ·fi ·˘ÙÔ‡˜ (30,4%) ¯ÚÂÈ¿ÛÙËÎ·Ó ÓÔÛËÏ›·. ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ˘ÔÛÙËÚ›˙ÂÈ ÙËÓ ¿Ô„Ë fiÙÈ ÙÔ M. pneumoniae ÌÔÚ› Ó· ÏÂÈÙÔ˘ÚÁ› ˆ˜ ÂÎÏ˘ÙÈÎfi ·›ÙÈÔ ˘ÔÙÚÔÈ¿˙ÔÓÙÔ˜ Û˘ÚÈÁÌÔ‡ Î·È ‚‹¯· (15). √È ›‰ÈÔÈ 10 ·ÛıÂÓ›˜ (43,4%) ‹Ù·Ó Î·È ·ÙÔÈÎÔ›. ∞˘Ùfi ÙÔ ÔÛÔÛÙfi ·ÙÔ›·˜ Û ·È‰È¿ Ì ¿Ù˘Ë Ïԛ̈ÍË Â›Ó·È ·Ó¿ÏÔÁÔ ÙÔ˘ ÔÛÔÛÙÔ‡ Ô˘ ·Ó·Ê¤ÚÂÙ·È ·ÏÏÔ‡ (15). Ÿˆ˜ Ê·›ÓÂÙ·È Î·È ÛÙÔÓ ¶›Ó·Î· 1, 32 ·fi Ù· 67 ·È‰È¿ Ô˘ ÌÂÏÂÙ‹ıËÎ·Ó (47,8%) ›¯·Ó ıÂÙÈο ·ÓÙÈÛÒÌ·Ù· IgG ÁÈ· M. pneumoniae Î·È ¤Ó· ·È‰› (1,5%) ›¯Â ıÂÙÈο IgG ÁÈ· C. pneumoniae. ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ Ô˘ ›¯·Ó ıÂÙÈο IgM ·ÓÙÈÛÒÌ·Ù· ÁÈ· Ì˘ÎfiÏ·ÛÌ·, ›¯·Ó ›Û˘ ıÂÙÈο Î·È IgG ·ÓÙÈÛÒÌ·Ù· ÁÈ· ÙÔÓ ›‰ÈÔ ÌÈÎÚÔÔÚÁ·ÓÈÛÌfi. ∆Ô ˘„ËÏfi ÔÛÔÛÙfi ÙˆÓ ıÂÙÈÎÒÓ IgG ·ÓÙÈÛˆÌ¿ÙˆÓ ÙÔ˘ Ì˘ÎÔÏ¿ÛÌ·ÙÔ˜ (47,8%) ˘Ô‰ÂÈÎÓ‡ÂÈ fiÙÈ Ë Â·Ê‹ ÌÂ Ì˘ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌÔÓ›·˜ Â›Ó·È ·ÚÎÂÙ¿ Û˘¯Ó‹ ÛÙ· ·È‰È¿ Î·È Ú¤ÂÈ Ó· ·Ó·˙ËÙÂ›Ù·È Û fiϘ ÙȘ ÂÚÈÙÒÛÂȘ ·Ú·ÙÂٷ̤Ó˘ Û˘Ìو̷ÙÔÏÔÁ›·˜ ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi. ¶Ú¤ÂÈ, ›Û˘, Ó· ·Ó·ÊÂÚı› fiÙÈ ¤Ó· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ·È‰ÈÒÓ Ì ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋/ ¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË, ›¯Â Ù·˘Ùfi¯ÚÔÓ· Î·È ¿ÏϘ ·Èٛ˜ ÁÈ· ÙÔÓ ‚‹¯· ÙÔ˘, ΢ڛˆ˜ ¿ÛıÌ· ‹ ÚÈÓ›Ùȉ·/ ·Ú·ÚÚÈÓÔÎÔÏ›Ùȉ·. ∏ Û˘Ó‡·ÚÍË Ì˘ÎÔÏ·ÛÌ·ÙÈ΋˜ Ïԛ̈͢ Î·È ¿ÛıÌ·ÙÔ˜ ÛÙÔÓ ›‰ÈÔ ·ÛıÂÓ‹, ÌÔÚ› Ó·
¶›Ó·Î·˜ 2. ™¯ËÌ·ÙÈ΋ ·ÔÙ‡ˆÛË Ù˘ ıÂڷ¢ÙÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘ ÙˆÓ ÔͤˆÓ Ì˘ÎÔÏ·ÛÌ·ÙÈÎÒÓ/¯Ï·Ì˘‰È·ÎÒÓ ÏÔÈÌÒÍÂˆÓ 8 ·È‰È¿ (34,7%) ‚ÂÏÙ›ˆÛË
3 ·È‰È¿ (13,0%) ·ÓÙÈ·ÛıÌ·ÙÈο
5 ·È‰È¿ (21,7%) ÚÈÓÈÎfi ÛÙÂÚÔÂȉ¤˜ + ·ÓÙÈ‚›ˆÛË 23 ·È‰È¿ (100,0%) (M. pneumoniae IgM + ‹ C. pneumoniae IgM+) ˘fi ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹ 8 ·È‰È¿ (34,7%) ÎÏ·ÚÈıÚÔÌ˘Î›ÓË 15 ·È‰È¿ (65,2%) fi¯È ‚ÂÏÙ›ˆÛË 5 ·È‰È¿ (21,7%) ÎÏ·ÚÈıÚÔÌ˘Î›ÓË + ·ÓÙÈ·ÛıÌ·ÙÈο
2 ·È‰È¿ (8,6%) ÎÏ·ÚÈıÚÔÌ˘Î›ÓË + ÚÈÓÈÎfi ÛÙÂÚÔÂȉ¤˜ Paediatriki 2007;70:37-41
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ª˘ÎfiÏ·ÛÌ· Î·È ¯ÚfiÓÈÔ˜ ‚‹¯·˜
·Ô‰Ôı› ÛÙËÓ È‰ÈfiÙËÙ· ÙÔ˘ M. pneumoniae Ó· ÚÔηÏ› ‚ÚÔÁ¯È΋ ˘ÂÚ··ÓÙËÙÈÎfiÙËÙ· › Ì·ÎÚfi ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· (1,3,4). ∏ ¯ÔÚ‹ÁËÛË ÎÏ·ÚÈıÚÔÌ˘Î›Ó˘ ·fi ÙÔ ÛÙfiÌ· ÁÈ· 3 ‚‰ÔÌ¿‰Â˜ Ê·›ÓÂÙ·È Ó· Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙË Ì›ˆÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ÙˆÓ ·È‰ÈÒÓ Ì ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋/ ¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË, ·Ó Î·È 8 ·ÛıÂÓ›˜ (34,7%) ‚ÂÏÙÈÒıËÎ·Ó ¯ˆÚ›˜ ÂȉÈ΋ ıÂڷ›·. ÿÛˆ˜, ÏÔÈfiÓ, Ó· ‹Ù·Ó ÏÔÁÈÎfi Ó· ‰ÔÎÈÌ¿ÛÂÈ Î·Ó›˜ ÙË ¯ÔÚ‹ÁËÛË ÎÏ·ÚÈıÚÔÌ˘Î›Ó˘ Û ·È‰È¿ Ì ¯ÚfiÓÈÔ ‚‹¯· Ô˘ ‰ÂÓ ·ÓÙ·ÔÎÚ›ıËÎ·Ó ÛÙËÓ ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹. ∆ÔÓ›˙Ô˘ÌÂ, ˆÛÙfiÛÔ, fiÙÈ ··ÈÙÔ‡ÓÙ·È ÌÂÁ·Ï‡ÙÂÚ˜ ÌÂϤÙ˜ ÁÈ· Ó· ÙÂÎÌËÚÈÒÛÔ˘Ó ·˘Ù‹ ÙËÓ ˘fiıÂÛË, ηıÒ˜ Î·È Û‡ÁÎÚÈÛË ·ÛıÂÓÒÓ-Ì·ÚÙ‡ÚˆÓ. ∂›Û˘, ‰ÂÓ ÌÔÚ› Ó· ·ÁÓÔËı› ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô ¯ÚfiÓÈÔ˜ ‚‹¯·˜ Î·È ÔÈ ÏÔÈÌÒÍÂȘ ·fi ¿Ù˘· Â›Ó·È Û˘¯Ó¿ ·˘ÙÔ-È¿ÛÈ̘ ÛÙËÓ ·È‰È΋ ËÏÈΛ·, ÂÔ̤ӈ˜ Â›Ó·È Ôχ ‰‡ÛÎÔÏÔ Ó· ÂÎÙÈÌËı› Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ ıÂÚ·ÂÈÒÓ, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ÔÈ ·ÛıÂÓ›˜ ÌÔÚ› Ó· ‚ÂÏÙȈıÔ‡Ó Ì ÙÔ ¤Ú·ÛÌ· ÙÔ˘ ¯ÚfiÓÔ˘ Î·È ¯ˆÚ›˜ ·ÁˆÁ‹. ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ÔÍ›· Ì˘ÎÔÏ·ÛÌ·ÙÈ΋/¯Ï·Ì˘‰È·Î‹ Ïԛ̈ÍË Â›Ó·È Û¯ÂÙÈο Û˘¯Ó‹ ÛÙ· ·È‰È¿ Ì ¯ÚfiÓÈÔ ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ· ‚‹¯· Î·È Ù›ÓÂÈ Ó· ·ÓÙ·ÔÎÚ›ÓÂÙ·È Î·Ï¿ ÛÙË ıÂڷ›· Ì ÎÏ·ÚÈıÚÔÌ˘Î›ÓË. ∆Ô ˘„ËÏfi ÔÛÔÛÙfi ·ÛıÂÓÒÓ Ì ıÂÙÈο IgM ·ÓÙÈÛÒÌ·Ù· ÁÈ· Ì˘ÎfiÏ·ÛÌ·, ˘ÔÁÚ·ÌÌ›˙ÂÈ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÈ ÏÔÈÌÒÍÂȘ ·˘Ù¤˜ Ú¤ÂÈ Ó· ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È ÂÁη›Úˆ˜, ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔʇÁÂÙ·È Ë ‚ÚÔÁ¯È΋ ˘ÂÚ··ÓÙËÙÈÎfiÙËÙ· Ô˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ÂÈÌÔÓ‹ ÙÔ˘ ‚‹¯·.
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∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞
ORIGINAL ARTICLE
¶˘ÚÂÙÈÎÔ› Û·ÛÌÔ›: ∂›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ Ë ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË Ì ‰È·˙¿ÌË; 1 3Ë ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ πÔÎÚ¿ÙÂÈÔ˘ ¡ÔÛÔÎÔÌ›Ԣ, ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢ 2 1Ë ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ πÔÎÚ¿ÙÂÈÔ˘ ¡ÔÛÔÎÔÌ›Ԣ, ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢ AÏÏËÏÔÁÚ·Ê›·: ÃÚ‹ÛÙÔ˜ ¶. ¶·ÓÙÂÏÈ¿‰Ë˜, cpantel@hol.gr ∫·ıËÁËÙ‹˜ ¶·È‰È·ÙÚÈ΋˜ ∫. ¶·Ï·Ì¿ 4, ∆.∫. 551 33, £ÂÛÛ·ÏÔÓ›ÎË
∂. ¶·˘Ï›‰Ô˘1, ª. ∆˙ËÙËÚ›‰Ô˘1, ∂. ∫ÔÓÙfiÔ˘ÏÔ˜2, Ã. ¶·ÓÙÂÏÈ¿‰Ë˜1 ¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: ∂ÎÙÈÌ‹ıËÎÂ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ‰È·ÏÂ›Ô˘Û·˜ ÚÔʇϷ͢ Ì ‰È·˙¿ÌË ·fi ÙÔ ÔÚıfi ÛÙËÓ ÚfiÏË„Ë ÙˆÓ ˘ÚÂÙÈÎÒÓ Û·ÛÌÒÓ (¶™). ª¤ıÔ‰ÔÈ: ¶ÚfiÎÂÈÙ·È ÁÈ· ÚÔÔÙÈ΋ Ù˘¯·ÈÔÔÈË̤ÓË ÌÂϤÙË Û 139 ·È‰È¿ (77 ÎÔÚ›ÙÛÈ· Î·È 62 ·ÁfiÚÈ·), Ô˘ ›¯·Ó ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™. ∆· ·È‰È¿ ¯ˆÚ›ÛÙËÎ·Ó Û ‰‡Ô ÔÌ¿‰Â˜: ÙËÓ ÔÌ¿‰· (∞) Ô˘ ¤Ï·‚ ÚÔʇϷÍË (n=68) Î·È ÙËÓ ÔÌ¿‰· (µ) ¯ˆÚ›˜ ÚÔʇϷÍË (n=71). ∆· ·È‰È¿ Î·È ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ ·Ú¤ÌÂÈÓ·Ó Û ·Ú·ÎÔÏÔ‡ıËÛË ÁÈ· 3 ¯ÚfiÓÈ·. ∆· ÎÚÈÙ‹ÚÈ· ÂÈÛ·ÁˆÁ‹˜ ÛÙË ÌÂϤÙË ‹Ù·Ó: ÂχıÂÚÔ ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi ·‡ÚÂÙˆÓ Û·ÛÌÒÓ, Ê˘ÛÈÔÏÔÁÈ΋ „˘¯ÔÎÈÓËÙÈ΋ ·Ó¿Ù˘ÍË, Ó· ÌËÓ ¤¯ÂÈ ¯ÔÚËÁËı› ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹ Î·È ËÏÈΛ· ¤Ó·Ú͢ ÌÂٷ͇ 6 ÌËÓÒÓ Î·È 3 ÂÙÒÓ. ∏ οı ÔÌ¿‰· Ù·ÍÈÓÔÌ‹ıËΠ۠ÙÚÂȘ ˘ÔÔÌ¿‰Â˜, ·Ó¿ÏÔÁ· Ì ÙÔÓ ‚·ıÌfi ÎÈÓ‰‡ÓÔ˘ ˘ÔÙÚÔ‹˜. ø˜ ·Ú¿ÁÔÓÙ˜ ÂÈÎÈÓ‰˘ÓfiÙËÙ·˜ ıˆڋıËηÓ: Ë ËÏÈΛ· ¤Ó·Ú͢ ≤15 ÌËÓÒÓ, ÙÔ ÈÛÙÔÚÈÎfi ÂÈÏË„›·˜ ÛÂ Û˘ÁÁÂÓ‹ ·’ ‚·ıÌÔ‡, ÙÔ ÈÛÙÔÚÈÎfi ¶™ ÛÂ Û˘ÁÁÂÓ‹ ·’ ‚·ıÌÔ‡, ÙÔ ÂÈÏÂÁ̤ÓÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™ Î·È ÔÈ Û˘¯Ó¤˜ Â̇ÚÂÙ˜ ÏÔÈÌÒÍÂȘ. ∆ËÓ ÚÒÙË Ë̤ڷ ÙÔ˘ ˘ÚÂÙÔ‡ ¯ÔÚËÁ‹ıËΠ‰È·˙¿ÌË 0,33 mg/kg µ™/8ˆÚÔ Î·È ÙË ‰Â‡ÙÂÚË 0,33 mg/kg µ™/12ˆÚÔ, Î·È ÔÙ¤ ¿Óˆ ·fi 7,5 mg/‰fiÛË. ™Â fiÏ· Ù· ·È‰È¿ ¤ÁÈÓ ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ· ÌfiÏȘ ·˘Ú¤ÙËÛ·Ó, ÌÂÙ¿ ÙÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™. ∞ÔÙÂϤÛÌ·Ù·: ™ÙÔ Ù¤ÏÔ˜ ÙˆÓ 3 ÂÙÒÓ ·Ú·ÎÔÏÔ‡ıËÛ˘, Ë Û˘¯ÓfiÙËÙ· ˘ÔÙÚÔÒÓ ÛÙËÓ ÔÌ¿‰· Ô˘ ‰ÂÓ ¤Ï·‚ ‰È·˙¿ÌË ‹Ù·Ó 83% ÁÈ· Ù· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, 55% ÛÙ· ÂӉȷ̤ÛÔ˘ Î·È 46% ÛÙ· ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·È‰È¿. ™Ù· ·È‰È¿ Ì ÚÔʇϷÍË ÔÈ Û˘¯ÓfiÙËÙ˜ ˘ÔÙÚÔ‹˜ ÌÂÈÒıËÎ·Ó Û fiϘ ÙȘ ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘: 38%, 35% Î·È 33% ·ÓÙ›ÛÙÔȯ·. ™˘ÌÂÚ¿ÛÌ·Ù·: ∏ ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË Ì ‰È·˙¿ÌË ·fi ÙÔ ÔÚıfi ÌÂÈÒÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ˘ÔÙÚÔ‹˜ ȉȷ›ÙÂÚ· ÛÙ· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·È‰È¿, ·ÚΛ Ó· ¯ÔÚËÁÂ›Ù·È ¤ÁηÈÚ· Î·È Â·ÚÎÒ˜.
§¤ÍÂȘ ÎÏÂȉȿ: ¢È·˙¿ÌË, ÚÔʇϷÍË ˘ÚÂÙÈÎÒÓ Û·ÛÌÒÓ.
Efficacy of intermittent rectal diazepam prophylaxis for the prevention of febrile seizures 1 3rd Department of Pediatrics, “Ippokration” Hospital, Aristotle University of Thessaloniki 2 1st Department of Pediatrics, “Ippokration” Hospital, Aristotle University of Thessaloniki Correspondence: Christos P. Panteliadis, cpantel@hol.gr Professor of Pediatrics 4, K. Palama St., 551 33, Thessaloniki
E. Pavlidou1, M. Tzitiridou1, E. Kontopoulos2, C. Panteliadis1 Abstract Objectives: The aim of this study was to assess the efficacy of intermittent rectal diazepam prophylaxis for the prevention of febrile seizures (FS). Methods: This was a prospective randomized trial of 139 children (77 females, 62 males), who had experienced a first episode of FS. The children were allocated to two groups: group (A), which received intermittent rectal diazepam prophylaxis (n=68), and group (B) without prophylaxis (n=71). The children in both groups were monitored for 36 months. The inclusion criteria were: no personal history of afebrile seizures, normal neurodevelopment, no previous anticonvulsant therapy, and age between 6 months and 3 years. The children in each group were stratified into low, intermediate and high risk according to clinical factors: age at onset ≤15 months, epilepsy in first degree relatives, FS in first degree relatives, a first complex FS and increased febrile episodes. Diazepam was administered rectally at a dose of 0.33 mg/kg BW/8 hours on the first day of a febrile illness and 0.33 mg/kgBW/12 hours the second day, maximum 7.5 mg/dose. All children had an EEG recording after the first FS as soon as they were afebrile. Results: The 36-month recurrence rates for the no prophylaxis group were 83% in high-risk, 55% in intermediate and 46% in low-risk patients. For the prophylaxis group the recurrence rates were lower in all risk groups: 38%, 35% and 33% respectively. Conclusions: Intermittent rectal diazepam prophylaxis reduces the recurrence rate of FS, especially for high-risk children, provided that adequate doses are given early in the febrile illness.
Key words: Diazepam, febrile seizure prophylaxis.
Paediatriki 2007;70:42-48
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¶˘ÚÂÙÈÎÔ› Û·ÛÌÔ›
∂ÈÛ·ÁˆÁ‹ ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ‰ËÌÔÛȇıËÎ·Ó ÔÏϤ˜ ÂÚÁ·Û›Â˜ Ô˘ ·ÊÔÚÔ‡Û·Ó ÙË ıÂڷ›·, ÚÔʇϷÍË Î·È ÚfiÁÓˆÛË ÙˆÓ ˘ÚÂÙÈÎÒÓ Û·ÛÌÒÓ (¶™) (1,2). ∞fi ÙȘ ÌÂϤÙ˜ ·˘Ù¤˜ ÚԤ΢„·Ó ÔÏÏ¿ Ó¤· ÛÙÔȯ›· Û¯ÂÙÈο Ì ÙËÓ ÎÏËÚÔÓÔÌÈ΋ ÂÈ‚¿Ú˘ÓÛË ‹ ÚԉȿıÂÛË ÙˆÓ ¶™ (3,4). ∏ ¿Ô„Ë fiÙÈ ÔÈ ·ÏÔ› ¶™ Â›Ó·È Î·ÏÔ‹ıÂȘ Î·È Û¿ÓÈ· ··ÈÙÔ‡Ó ıÂڷ›· ÈÛ¯‡ÂÈ Î·È Û‹ÌÂÚ·. øÛÙfiÛÔ, Â›Ó·È Èı·Ófi Û ¤Ó· ÌÈÎÚfi ÔÛÔÛÙfi ·È‰ÈÒÓ Ó· ÚÔÎÏËıÔ‡Ó ÂÈÏËÙÈΤ˜ (΢ڛˆ˜ ÂÛÙȷΤ˜) ÎÚ›ÛÂȘ. ∏ ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË Ì ‰È·˙¿ÌË ·fi ÙÔ ÛÙfiÌ·, ˘ÔÁÏÒÛÛÈ· ‹ ÙÔ ÔÚıfi (˘ÔÎÏ˘ÛÌfi) ¤¯ÂÈ Á›ÓÂÈ ı¤Ì· ¤Ú¢ӷ˜ ·fi ÔÏÏÔ‡˜ Û˘ÁÁÚ·Ê›˜ Ù· ÙÂÏÂ˘Ù·›· 20 ¯ÚfiÓÈ·. √È ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ ÂÈÎÂÓÙÚÒıËÎ·Ó ÛÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ‰È·ÏÂ›Ô˘Û·˜ ÚÔʇϷ͢ Ì ‰È·˙¿ÌË Î·È ÙËÓ Â›‰Ú·Û‹ Ù˘ ÛÙËÓ ˘ÔÙÚÔ‹ ÙˆÓ ¶™, ÂÓÒ ˆ˜ ÌÂÈÔÓ¤ÎÙËÌ· ıˆÚÂ›Ù·È Ë ¯ÔÚ‹ÁËÛË ÂÓfi˜ Ê·ÚÌ¿ÎÔ˘ Û ÌÈÎÚ¿ ·È‰È¿ ÁÈ· ÌÈ· ηÏÔ‹ıË Î·Ù¿ÛÙ·ÛË (5). √È ÌÂϤÙ˜ ·˘Ù¤˜ ¤‰ÂÈÍ·Ó fiÙÈ Ë ‰È·˙¿ÌË Ú¤ÂÈ Ó· ¯ÔÚËÁÂ›Ù·È Û ÂÈÏÂÁ̤Ó˜ ÂÚÈÙÒÛÂȘ, fiˆ˜ Û ·È‰È¿ Ì ·Ú·ÙÂٷ̤ÓÔ˘˜ Û·ÛÌÔ‡˜, ÂÛÙȷΤ˜ ÎÚ›ÛÂȘ Î·È Û ·È‰È¿ Ì ·ÚÎÂÙÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ (6,7). ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ Ì·˜ Â›Ó·È Ó· ÂÎÙÈÌËı› Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ‰È·ÏÂ›Ô˘Û·˜ ÚÔʇϷ͢ Ì ‰È·˙¿ÌË, ÛÂ Û˘Û¯¤ÙÈÛË Ì ÙÔÓ Î›Ó‰˘ÓÔ ˘ÔÙÚÔ‹˜. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, ÌÂÏÂÙ‹Û·Ì ¤Ó· ÌÂÁ¿ÏÔ ‰Â›ÁÌ· ·È‰ÈÒÓ Î·È Ë ‰È¿ÚÎÂÈ· ·Ú·ÎÔÏÔ‡ıËÛ˘ ‹Ù·Ó ÙÔ˘Ï¿¯ÈÛÙÔÓ 3 ¯ÚfiÓÈ·. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∏ ¤Ú¢ӷ ¤ÁÈÓ Û ÔÌ¿‰· 145 ·È‰ÈÒÓ Ì ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™ Î·È Â›Ó·È ÚÔÔÙÈ΋, ÂÏÂÁ¯fiÌÂÓË Î·È Ù˘¯·ÈÔÔÈË̤ÓË. ∞fi ÙË ÌÂϤÙË ·ÔÎÏ›ÛÙËÎ·Ó 6 ·È‰È¿ ÏfiÁˆ ‰˘ÛÎÔÏÈÒÓ ·Ú·ÎÔÏÔ‡ıËÛ˘. ∆· ˘fiÏÔÈ· 139 ·È‰È¿ ·Ú¤ÌÂÈÓ·Ó Û ·Ú·ÎÔÏÔ‡ıËÛË ÁÈ· ‰È¿ÛÙËÌ· 3 ¯ÚfiÓˆÓ. ∆· ·È‰È¿ ¯ˆÚ›ÛÙËÎ·Ó Û ‰‡Ô ÔÌ¿‰Â˜: ÙËÓ ÔÌ¿‰· ∞ Ô˘ ¤Ï·‚ ÚÔʇϷÍË Ì ‰È·˙¿ÌË Î·È ÙËÓ ÔÌ¿‰· µ (Ì·ÚÙ‡ÚˆÓ) Ô˘ ‰ÂÓ ¤Ï·‚ ·ÁˆÁ‹. ∫¿ı ·È‰› Ù˘¯·›· ηٷ¯ˆÚ‹ıËΠÛÙËÓ ÔÌ¿‰· ∞ ‹ µ (ÌÔÓ¤˜ Î·È ˙˘Á¤˜ ̤Ú˜ ·ÓÙ›ÛÙÔȯ·) Î·È Ô ˘Â‡ı˘ÓÔ˜ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÁÈ·ÙÚfi˜ ‰ÂÓ ÁÓÒÚÈ˙ ÙËÓ Ù˘¯·ÈÔÔ›ËÛË. √È ‰‡Ô ÔÌ¿‰Â˜ ‹Ù·Ó Û˘ÁÎÚ›ÛÈ̘ fiÛÔÓ ·ÊÔÚ¿ Ù· ‰ËÌÔÁÚ·ÊÈο Î·È ÎÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘˜ (¶›Ó·Î·˜ 1). ∏ ÔÌ¿‰· ∞ (ÔÌ¿‰· ÚÔʇϷ͢) ·ÔÙÂÏÂ›Ù·È ·fi 68 ·È‰È¿ Î·È Ë ÔÌ¿‰· µ (Ì·ÚÙ‡ÚˆÓ, ¯ˆÚ›˜ ÚÔʇϷÍË) ·fi 71 ·È‰È¿. ∆· ÎÚÈÙ‹ÚÈ· ÂÈÛ·ÁˆÁ‹˜ ÛÙË ÌÂϤÙË Â›Ó·È: ÂχıÂÚÔ ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi ·‡ÚÂÙˆÓ Û·ÛÌÒÓ, Ê˘ÛÈÔÏÔÁÈ΋ „˘¯ÔÎÈÓËÙÈ΋ ·Ó¿Ù˘ÍË, Ó· ÌËÓ ¤¯ÂÈ ¯ÔÚËÁËı› ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹ Î·È Ë ËÏÈΛ· ¤Ó·Ú͢ Ó· Â›Ó·È ÌÂٷ͇ 6 ÌËÓÒÓ Î·È 3 ÂÙÒÓ. √È ¶™ ÔÚ›ÛÙËÎ·Ó ‚¿ÛÂÈ Ù˘ ÔÌÔʈӛ·˜ ÙÔ˘ National Institutes of Health. ™‡Ìʈӷ Ì ÙËÓ ÔÔ›·, ˆ˜ ¶™ ÔÚ›˙ÔÓÙ·È ÔÈ Û·ÛÌÔ› Ô˘ Û˘Ì‚·›ÓÔ˘Ó Û ‚Ú¤ÊË Î·È ·È‰È¿ ËÏÈΛ·˜ ·fi 3 ÌËÓÒÓ ¤ˆ˜ 5 ÂÙÒÓ, Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔÓ ˘ÚÂÙfi ·ÏÏ¿ ‰ÂÓ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ÂÓ‰ÔÎÚ¿ÓÈ·˜ Ïԛ̈͢ ‹ ¿ÏÏ˘ ÔÚÈṲ̂Ó˘ ·ÈÙ›·˜, Ô‡Ù ÈÛÙÔÚÈÎfi ÚÔËÁÔ‡ÌÂÓÔ˘ ÂÂÈÛÔ‰›Ô˘ ·‡ÚÂÙˆÓ Û·ÛÌÒÓ (8).
√È ÁÔÓ›˜ ÙˆÓ ·È‰ÈÒÓ ÂÓËÌÂÚÒıËÎ·Ó ÏÂÙÔÌÂÚÒ˜ Û¯ÂÙÈο Ì ÙË Ê‡ÛË Î·È ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ¶™ Î·È ¤‰ˆÛ·Ó ÙË Û˘ÁηٿıÂÛ‹ ÙÔ˘˜ ÁÈ· ÙË ÌÂϤÙË. ∂ÈϤÔÓ, ÔÈ ÁÔÓ›˜ Ù˘ ÔÌ¿‰·˜ ∞ ‹Ú·Ó ÚÔÊÔÚÈΤ˜ Î·È ÁÚ·Ù¤˜ Ô‰ËÁ›Â˜ Û¯ÂÙÈο Ì ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ÎχÛÌ·ÙÔ˜ ‰È·˙¿Ì˘ ·fi ÙÔ ÔÚıfi Î·È ÏËÚÔÊÔÚ‹ıËÎ·Ó ÁÈ· ÙȘ Èı·Ó¤˜ ·ÚÂÓ¤ÚÁÂȘ. ∏ ÚÔʇϷÍË ÂÊ·ÚÌfiÛÙËΠÙȘ ‰‡Ô ÚÒÙ˜ Ë̤Ú˜ ÙÔ˘ ˘ÚÂÙÔ‡ Î·È fiÙ·Ó Ë ıÂÚÌÔÎÚ·Û›· ÛÒÌ·ÙÔ˜ ·fi ÙÔ ÔÚıfi ‹Ù·Ó >38ÔC. ∞Ó¿ÏÔÁ· Ì ÙÔ ‚¿ÚÔ˜ ÛÒÌ·ÙÔ˜ ¯ÔÚËÁ‹ıËΠÙËÓ ÚÒÙË Ì¤Ú· ÙÔ˘ ˘ÚÂÙÔ‡ 0,33 mg/kg µ™/8ˆÚÔ Î·È ÙË ‰Â‡ÙÂÚË 0,33 mg/kg µ™/12ˆÚÔ Î·È fi¯È ¿Óˆ ·fi 7,5 mg/‰fiÛË. ¶ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔÊ¢¯ı› ·ÓÂÈı‡ÌËÙË Î·Ù·ÛÙÔÏ‹ ·fi ÙË Û˘ÛÛÒÚ¢ÛË Ù˘ ‰È·˙¿Ì˘, ‰fiıËÎ·Ó ÙÔ Ì¤ÁÈÛÙÔ 5 Û˘Ó¯›˜ ‰fiÛÂȘ Û οı Â̇ÚÂÙË Ïԛ̈ÍË, Û‡Ìʈӷ Ì ÙÔ ·Ú·¿Óˆ Û¯‹Ì·. ¶ÚÔÊ›Ï ÎÈÓ‰‡ÓÔ˘ ˘ÔÙÚÔ‹˜ ¤ÁÈÓ Û οı ·È‰› Ù˘ ÌÂϤÙ˘ ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜ Î·È ÔÈ ÁÔÓ›˜ ÂÓËÌÂÚÒıËÎ·Ó ÁÈ· ÙËÓ ÂӉ¯fiÌÂÓË ˘ÔÙÚÔ‹ ÙˆÓ ¶™ (¯·ÌËÏfi˜, ÂӉȿÌÂÛÔ˜ ‹ ˘„ËÏfi˜ ΛӉ˘ÓÔ˜). ∂Âȉ‹ Ù· ˘„ËÏÔ‡, ÂӉȷ̤ÛÔ˘ ‹ ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·È‰È¿ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ‰È·ÊÔÚÂÙÈο ÛÙË ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË Ì ‰È·˙¿ÌË, Ë Î¿ı ÔÌ¿‰· Ù·ÍÈÓÔÌ‹ıËΠ۠ÙÚÂȘ ˘ÔÔÌ¿‰Â˜, ·Ó¿ÏÔÁ· Ì ÙÔÓ ‚·ıÌfi ÎÈÓ‰‡ÓÔ˘ ˘ÔÙÚÔ‹˜. ∆Ô ÚÔÊ›Ï ÂÈÎÈÓ‰˘ÓfiÙËÙ·˜ ηıÔÚ›ÛÙËΠ‚¿ÛÂÈ ÙˆÓ ÎÏÈÓÈÎÒÓ ÎÚÈÙËÚ›ˆÓ ·fi ÙÔ ÈÛÙÔÚÈÎfi. √È ÚÔÁÓˆÛÙÈÎÔ› ‰Â›ÎÙ˜ ˘ÔÙÚÔ‹˜ ÙˆÓ ¶™, Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó Î·È ÛÙË ‰È΋ Ì·˜ ÌÂϤÙË, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 2. ∆· ·È‰È¿ Ô˘ ›¯·Ó ·fi Ìˉ¤Ó ¤ˆ˜ ¤Ó·Ó ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ηٷÓÂÌ‹ıËÎ·Ó ÛÙ· ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÂΛӷ Ì ‰‡Ô ·Ú¿ÁÔÓÙ˜ ÛÙ· ÂӉȷ̤ÛÔ˘ Î·È ÂΛӷ Ì ÙÚÂȘ ¤ˆ˜ ¤ÓÙ ÛÙ· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘. ∏ ÌÂϤÙË ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ¿Ú¯ÈÛ ÌÂÙ¿ ÙÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™ ·fi ÙÔ ÙÌ‹Ì· ¶·È‰È·ÙÚÈ΋˜ ¡Â˘ÚÔÏÔÁ›·˜ (ÎÏÈÓÈ΋ Î·È Â͈ÙÂÚÈο È·ÙÚ›·). ŸÏ· Ù· ·È‰È¿ ›¯·Ó ÂχıÂÚÔ ÚÔÁÂÓÓËÙÈÎfi Î·È ÂÚÈÁÂÓÓËÙÈÎfi ÈÛÙÔÚÈÎfi, Ê˘ÛÈÔÏÔÁÈ΋ „˘¯ÔÎÈÓËÙÈ΋ ·Ó¿Ù˘ÍË Î·È Î·Ó¤Ó· ‰ÂÓ Â›¯Â ÎÏÈÓÈΤ˜ ‹ ÂÚÁ·ÛÙËÚȷΤ˜ ÂӉ›ÍÂȘ ÂÎÊ˘ÏÈÛÙÈÎÔ‡ ÓÔÛ‹Ì·ÙÔ˜ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (∫¡™). ™Â fiÏ· ¤ÁÈÓ ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ· (∏∂°), ÌfiÏȘ ·˘Ú¤ÙËÛ·Ó, ÌÂÙ¿ ÙÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™ Î·È ·Ú·ÎÔÏÔ˘ı‹ıËÎ·Ó ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 3 ¯ÚfiÓÈ·. ∏ ÙÂÏÈ΋ ·ÓÂÎÙ›ÌËÛË ¤ÁÈÓ ÛÙËÓ ËÏÈΛ· ÙˆÓ 3-6 ÂÙÒÓ, ̤ÛË ËÏÈΛ· 4,8±1,2 ÂÙÒÓ. ∫¿ı 4 Ì‹Ó˜ ˘‹Ú¯Â ·ʋ Ì ÙÔ˘˜ ÁÔÓ›˜ ÙˆÓ ·È‰ÈÒÓ ÁÈ· ÙÔÓ ¤ÏÂÁ¯Ô ÂÊ·ÚÌÔÁ‹˜ ÙÔ˘ ÚˆÙÔÎfiÏÏÔ˘ Î·È ÙËÓ ·ÔÊ˘Á‹ Ï·ıÒÓ. ∂›Û˘, Û οı Â̇ÚÂÙÔ ÂÂÈÛfi‰ÈÔ ˘‹Ú¯Â ͯˆÚÈÛÙ‹ ÙËÏÂʈÓÈ΋ ÂÈÎÔÈÓˆÓ›· Ì ÙÔ˘˜ ÁÔÓ›˜ ÁÈ· Ó· Û˘ÁÎÂÓÙÚˆıÔ‡Ó fiϘ ÔÈ ··Ú·›ÙËÙ˜ ÏËÚÔÊÔڛ˜ Û¯ÂÙÈο Ì ÙÔÓ ˘ÚÂÙfi, ÙÔ˘˜ Û·ÛÌÔ‡˜ Î·È ÙȘ Èı·Ó¤˜ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ. ™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ∏ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ÌÂٷ͇ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ ¤ÁÈÓ ۇÌʈӷ Ì ÙÔ Pearson Chi-Square Test (™˘Ó¯‹˜ ¢ÈfiÚıˆÛË Î·Ù¿ Yates). ™Ù·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ıˆڋıËÎ·Ó ÔÈ ÙÈ̤˜ ÙÔ˘ P<0,05.
∞ÔÙÂϤÛÌ·Ù· ∏ ÔÌ¿‰· ÚÔʇϷ͢ (n=68) ·ÔÙÂÏ›ÙÔ ·fi 39 ÎÔÚ›ÙÛÈ· Î·È 29 ·ÁfiÚÈ· Î·È Ë ËÏÈΛ· ¤Ó·Ú͢ Î˘Ì·ÈÓfiÙ·Ó ·fi 6 ¤ˆ˜ 36 Ì‹Ó˜ (̤ÛË ËÏÈΛ·±SD, 15±7). ™˘ÓÔÏÈο 54/68 ÂÌÊ¿ÓÈÛ·Ó ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ·ÏÒÓ ¶™, ÂÓÒ 14/68 ›¯·Ó ·ÚÔ˘ÛÈ¿ÛÂÈ ÛÙÔ ÚÒÙÔ ¶·È‰È·ÙÚÈ΋ 2007;70:42-48
44
∂. ¶·˘Ï›‰Ô˘ Î·È Û˘Ó.
¶›Ó·Î·˜ 1. µ·ÛÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ÔÌ¿‰ˆÓ ∞ Î·È µ ¢ËÌÔÁÚ·ÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο £‹Ï· ∏ÏÈΛ· ¤Ó·Ú͢ a* √ÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ¶™* √ÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ÂÈÏË„›·˜* æ˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË ¶·È‰ÈÎfi˜ ÛÙ·ıÌfi˜* ¶Ï‹Ú˘ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ëb ÷ڷÎÙËÚÈÛÙÈο ÙÔ˘ ·Ú¯ÈÎÔ‡ ÂÂÈÛÔ‰›Ô˘ ∏ÏÈΛ· ¤Ó·Ú͢ a* >15 ÌËÓÒÓ ≤15 ÌËÓÒÓ ¶™∂c* ∂ÈÏÂÁ̤ÓÔÈ ¶™ (ÂÛÙÈ·ÎÔ›)* ∂ÈÏÂÁ̤ÓÔÈ ¶™ (˘ÔÙÚÔ¤˜ ÛÙÔ ›‰ÈÔ Â̇ÚÂÙÔ)* ∂ÈÏÂÁ̤ÓÔÈ ¶™ (≥15 ÏÂÙ¿ ‰È¿ÚÎÂÈ·)* £ÂÚÌÔÎÚ·Û›· ÔÚıÔ‡ (ÔC) µ·ıÌfi˜ ÎÈÓ‰‡ÓÔ˘ ˘ÔÙÚÔ‹˜ 0-1 (÷ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘) 2 (ªÂÛ·›Ô˘ ÎÈÓ‰‡ÓÔ˘) 3-5 (À„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘)
√Ì¿‰· A (n=68)
√Ì¿‰· B (n=71)
39 (57%) 15 (6-36) 21 (31%) 6 (9%) ∫·Ó¤Ó· 21 (31%) 56 (82%)
38 (54%) 15 (6-36) 21 (30%) 8 (11%) ∫·Ó¤Ó· 24 (34%) 59 (83%)
15 (6-36) 30 (44%) 38 (56%) 2 (3%) 3 (4%)
15 (6-36) 31 (44%) 40 (56%) 2 (3%) 4 (6%)
12 (18%) 1 (2%) 38,5 (37-40oC)
10 (14%) 1 (2%) 38,8 (37-41oC)
27 (40%) 20 (29%) 21 (31%)
28 (39%) 20 (28%) 23 (32%)
a
™Â Ì‹Ó˜, b ™‡Ìʈӷ Ì ÙÔ ∂ıÓÈÎfi ¶ÚfiÁÚ·ÌÌ· ∂Ì‚ÔÏÈ·ÛÌÔ‡, c ¶˘ÚÂÙÈÎfi status epilepticus (‰È¿ÚÎÂÈ· >30 ÏÂÙ¿), * ¶·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙËÓ ˘ÔÙÚÔ‹ ÙˆÓ ¶™, fiˆ˜ Û˘˙ËÙ‹ıËÎ·Ó ÛÙÔ “ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ”.
ÂÂÈÛfi‰ÈÔ ÂÈÏÂÁ̤ÓÔ˘˜ ¶™ Î·È ·fi ·˘Ù¿ 3 ÂÌÊ¿ÓÈÛ·Ó ÂÛÙȷ΋ ÛËÌÂÈÔÏÔÁ›·, ÂÓÒ 12 ›¯·Ó ˘ÔÙÚÔ‹ ÛÙÔ ›‰ÈÔ Â̇ÚÂÙÔ. ∏ ‰È¿ÚÎÂÈ· ÙˆÓ ÎÚ›ÛÂˆÓ ‹Ù·Ó Û 65 ·È‰È¿ ÌÈÎÚfiÙÂÚË ·fi 10 ÏÂÙ¿. ™Â 3 ÂÚÈÙÒÛÂȘ ÔÈ Û·ÛÌÔ› ›¯·Ó ‰È¿ÚÎÂÈ· >15 ÏÂÙ¿, ·fi ÙȘ Ôԛ˜ ‰‡Ô ÂΉËÏÒıËÎ·Ó Ì ÙË ÌÔÚÊ‹ ˘ÚÂÙÈÎÔ‡ status epilepticus (>30 ÏÂÙ¿). ™˘ÓÔÏÈο 30 ·È‰È¿ Ù˘ ÔÌ¿‰·˜ ∞ ›¯·Ó ËÏÈΛ· ¤Ó·Ú͢ ¿Óˆ ÙˆÓ 15 ÌËÓÒÓ, ÂÓÒ 38 ‹Ù·Ó ÌÈÎÚfiÙÂÚ· (¶›Ó·Î·˜ 1). ∏ ÔÌ¿‰· ÙˆÓ Ì·ÚÙ‡ÚˆÓ (n=71) ·ÔÙÂÏ›ÙÔ ·fi 38 ÎÔÚ›ÙÛÈ· Î·È 33 ·ÁfiÚÈ· Î·È Ë ËÏÈΛ· ¤Ó·Ú͢ Î˘Ì·ÈÓfiÙ·Ó ·fi 6 ¤ˆ˜ 36 Ì‹Ó˜ (̤ÛË ËÏÈΛ·±SD, 15±8). ¶ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ·ÏÒÓ ¶™ ÂÌÊ¿ÓÈÛ·Ó 59/71 ·È‰È¿ Î·È 12/71 ›¯·Ó ÂÈÏÂÁ̤ÓÔ. ™·ÛÌÔ› ‰È¿ÚÎÂÈ·˜ 1-5 ÏÂÙ¿ ηٷÁÚ¿ÊËÎ·Ó Û 68/71 ·ÛıÂÓ›˜, ‰‡Ô ›¯·Ó ˘ÚÂÙÈÎfi status epilepticus, ÂÓÒ 10/71 ›¯·Ó Î·È ‰Â‡ÙÂÚÔ ÂÂÈÛfi‰ÈÔ ÛÙÔ ›‰ÈÔ Â̇ÚÂÙÔ. ∂ÛÙÈ·ÎÔ› ¶™ ·Ú·ÙËÚ‹ıËÎ·Ó Û ٤ÛÛÂÚȘ ·ÛıÂÓ›˜. ∆ÚÈ¿ÓÙ· ¤Ó· ·È‰È¿ ‹Ù·Ó ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ· ·fi 15 ÌËÓÒÓ Î·È 40 ÌÈÎÚfiÙÂÚ· (¶›Ó·Î·˜ 1). ¶›Ó·Î·˜ 2. ¶ÚÔÁÓˆÛÙÈÎÔ› ‰Â›ÎÙ˜ ˘ÔÙÚÔ‹˜ ˘ÚÂÙÈÎÒÓ Û·ÛÌÒÓ ñ ∏ÏÈΛ· ¤Ó·Ú͢ ≤15 ÌËÓÒÓ ñ √ÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ¶™ ÛÂ Û˘ÁÁÂÓ‹ ·ã ‚·ıÌÔ‡ ñ √ÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ÂÈÏË„›·˜ ÛÂ Û˘ÁÁÂÓ‹ ·ã ‚·ıÌÔ‡ ñ ∂ÈÏÂÁ̤ÓÔ ÙÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™ ñ ™˘¯Ó¤˜ Â̇ÚÂÙ˜ ÏÔÈÌÒÍÂȘ (·È‰ÈÎfi˜ ÛÙ·ıÌfi˜) Paediatriki 2007;70:42-48
ªÂٷ͇ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ ‰ÂÓ ˘‹Ú¯·Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ fiÛÔÓ ·ÊÔÚ¿ ÙÔ Ê‡ÏÔ, ÙËÓ ËÏÈΛ· ¤Ó·Ú͢, ÙË ÌÔÚÊ‹ Î·È ÙË ‰È¿ÚÎÂÈ· Û·ÛÌÒÓ. £ÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ÂÈÏË„›·˜ ÛÂ Û˘ÁÁÂÓ‹ ·ã ‚·ıÌÔ‡ ˘‹Ú¯Â Û 6 ·ÛıÂÓ›˜ Ù˘ ÔÌ¿‰·˜ ∞ Î·È Û 8 Ù˘ ÔÌ¿‰·˜ µ, ÂÓÒ ÈÛÙÔÚÈÎfi ¶™ ÛÂ Û˘ÁÁÂÓ‹ ·ã ‚·ıÌÔ‡ ›¯·Ó 21/68 ·È‰È¿ Ù˘ ÔÌ¿‰·˜ ∞ Î·È 21/71 Ù˘ ÔÌ¿‰·˜ µ. ∏ Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË Î·È Ë „˘¯ÔÎÈÓËÙÈ΋ ·Ó¿Ù˘ÍË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜ Û fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ∆· ∏∂° fiÏˆÓ ÙˆÓ ·È‰ÈÒÓ (ÔÌ¿‰· ∞ Î·È µ) ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο, ÂÎÙfi˜ ·fi Ì›· ÂÚ›ÙˆÛË fiÔ˘ ηٷÁÚ¿ÊËÎ·Ó Û·Ê›˜ ·ÚÔ͢ÓÙÈΤ˜ ÂÎÊÔÚÙ›ÛÂȘ ·ÚÈÛÙÂÚ¿ Î·È Ù¿ÛË ÁÂÓ›Î¢Û˘. ∆Ô ·È‰› ·Ó‹Î ÛÙËÓ ÔÌ¿‰· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ¯ˆÚ›˜ ÚÔʇϷÍË Î·È ÂÌÊ¿ÓÈÛÂ Û˘ÓÔÏÈο 6 ˘ÔÙÚÔ¤˜. ŸÏ· Ù· ·È‰È¿ Ù˘ ÌÂϤÙ˘ ›¯·Ó ÙÔ˘Ï¿¯ÈÛÙÔÓ ¤ÍÈ Â̇ÚÂÙ· ÂÂÈÛfi‰È· ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ 3 ¯ÚfiÓˆÓ ·Ú·ÎÔÏÔ‡ıËÛ˘ Î·È Ô Û˘ÓÔÏÈÎfi˜ ·ÚÈıÌfi˜ ÙˆÓ Â̇ÚÂÙˆÓ Î·Ù·ÛÙ¿ÛÂˆÓ ‹Ù·Ó 443 ÁÈ· ÙËÓ ÔÌ¿‰· ∞ Î·È 485 ÁÈ· ÙË µ. ∆· ·›ÙÈ· ÙÔ˘ ˘ÚÂÙÔ‡ ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ ‹Ù·Ó ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡. ∏ ıÂÚÌÔÎÚ·Û›· ÛÙÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™ Î˘Ì·›ÓÔÓÙ·Ó ÛÙ· ·È‰È¿ ˘fi ÚÔʇϷÍË ÌÂٷ͇ 37,5ÔC Î·È 40ÔC (̤ÛË ÙÈÌ‹ 38,5ÔC) Î·È ÛÙËÓ ÔÌ¿‰· ¯ˆÚ›˜ ÚÔʇϷÍË ÌÂٷ͇ 37,6ÔC Î·È 41ÔC (̤ÛË ÙÈÌ‹ 38,8ÔC). øÛÙfiÛÔ, ÛÙȘ ˘ÔÙÚÔ¤˜ Ë ıÂÚÌÔÎÚ·Û›· ›¯Â ‰È·Î‡Ì·ÓÛË ·fi 38ÔC ¤ˆ˜ 39,5ÔC (̤ÛË ÙÈÌ‹ 39ÔC). ∏ ÏÂÈÔÓfiÙËÙ·
45
ÙˆÓ ˘ÔÙÚÔÒÓ ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ·ÚÔ˘ÛÈ¿ÛÙËΠÙȘ ÚÒÙ˜ 24 ÒÚ˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ÙÔ˘ ˘ÚÂÙÔ‡. ™˘ÓÔÏÈο ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘ ÛÙËÓ ÔÌ¿‰· ∞ Ô˘ ¤Ï·‚ ‰È·˙¿ÌË, ˘‹Ú¯·Ó 38 ˘ÔÙÚÔ¤˜ Î·È 103 ÛÙËÓ ÔÌ¿‰· µ ÙˆÓ Ì·ÚÙ‡ÚˆÓ. ∆Ô˘˜ ÚÒÙÔ˘˜ 6 Ì‹Ó˜ Ù˘ ÌÂϤÙ˘ ‰Âη¤ÓÙ ·ÛıÂÓ›˜ ·fi ÙËÓ ÔÌ¿‰· ∞ (22%) Î·È ÂÈÎÔÛÈÙ¤ÛÛÂÚȘ ·fi ÙËÓ ÔÌ¿‰· µ (34%) ›¯·Ó ÙÔ˘Ï¿¯ÈÛÙÔÓ Ì›· ˘ÔÙÚÔ‹. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ 20/68 ·È‰È¿ Ô˘ ‹Ú·Ó ÚÔʇϷÍË (29%) Î·È 33/71 Ô˘ ‰ÂÓ ‹Ú·Ó ÚÔʇϷÍË (46%) ˘ÔÙÚÔ›·Û·Ó. ∆Ú›· ¯ÚfiÓÈ· ÌÂÙ¿ ÙËÓ Ù˘¯·ÈÔÔ›ËÛË, Ë ·ıÚÔÈÛÙÈ΋ Û˘¯ÓfiÙËÙ· Ù˘ ÚÒÙ˘ ˘ÔÙÚÔ‹˜ ÙˆÓ ¶™ ‹Ù·Ó 60% ÛÙËÓ ÔÌ¿‰· ÙˆÓ Ì·ÚÙ‡ÚˆÓ Î·È 35% ÛÙËÓ ÔÌ¿‰· Ù˘ ‰È·˙¿Ì˘. ∞˘Ùfi ‰Â›¯ÓÂÈ fiÙÈ ÛËÌÂÈÒıËΠ̛ˆÛË 25% ÛÙËÓ ÔÌ¿‰· ∞ Ô˘ ¤Ï·‚ ÙËÓ ÚÔʇϷÍË (¶›Ó·Î·˜ 3). ™ÙËÓ ÔÌ¿‰· Ù˘ ‰È·˙¿Ì˘ 7/68 ·È‰È¿ ›¯·Ó Û˘ÓÔÏÈο 15 ˘ÔÙÚÔ¤˜, 15/68 Ì›· ˘ÔÙÚÔ‹, ÌfiÓÔ ‰‡Ô ·È‰È¿ Ù¤ÛÛÂÚȘ, ÂÓÒ 44/68 ‰ÂÓ ÂÌÊ¿ÓÈÛ·Ó ˘ÔÙÚÔ‹. ™ÙËÓ ÔÌ¿‰· Ô˘ ‰ÂÓ ¤Ï·‚ ÚÔʇϷÍË, 22/71 ·ÛıÂÓ›˜ ›¯·Ó Û˘ÓÔÏÈο 73 ˘ÔÙÚÔ¤˜, 20/71 Ì›· Î·È 1 ·È‰› ›¯Â 10. ∏ Û˘¯ÓfiÙËÙ· Ù˘ ˘ÔÙÚÔ‹˜ ÌÂȈÓfiÙ·Ó Ì ÙËÓ ·‡ÍËÛË Ù˘ ËÏÈΛ·˜ ÙˆÓ ·È‰ÈÒÓ, ÂȉÈÎfiÙÂÚ· ÌÂÙ¿ ÙÔÓ 2Ô ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜. ™ÙËÓ ÔÌ¿‰· µ, Ë Û˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ ÛÙÔ Ù¤ÏÔ˜ Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙˆÓ ÙÚÈÒÓ ¯ÚfiÓˆÓ ‹Ù·Ó 46% ÁÈ· Ù· ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·È‰È¿, 55% ÁÈ· Ù· ÂӉȷ̤ÛÔ˘ Î·È 83% ÁÈ· Ù· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (∂ÈÎfiÓ· 1). ∞ÓÙ›ıÂÙ·, ÛÙËÓ ÔÌ¿‰· ∞ Ë Û˘¯ÓfiÙËÙ· Ù˘ ˘ÔÙÚÔ‹˜ ÁÈ· ÙÔ ›‰ÈÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÌÂÈÒıËΠ۠33% ÛÙ· ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, 35% ÛÙ· ÂӉȷ̤ÛÔ˘ Î·È 38% ÛÙ· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·È‰È¿ (∂ÈÎfiÓ· 2). ªÂ ÙË ¯Ú‹ÛË Ù˘ ‰È·˙¿Ì˘ ·Ú·ÙËÚ‹ıËΠ̛ˆÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ ˘ÔÙÚÔ‹˜ ηٿ 45% ÛÙ· ·È‰È¿ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Î·È ÌfiÓÔ 13% Î·È 20% ·ÓÙ›ÛÙÔȯ·, ÛÙ· ¯·ÌËÏÔ‡ Î·È ÂӉȿÌÂÛÔ˘ ÎÈÓ‰‡ÓÔ˘. ∏ ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË Ì ‰È·˙¿ÌË Ì›ˆÛ ÙË Û˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ ÛËÌ·ÓÙÈο ÛÙ· ˘„ËÏÔ‡ ÎÈÓ-
™˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ (%)
¶˘ÚÂÙÈÎÔ› Û·ÛÌÔ›
™˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ Û ۯ¤ÛË Ì ÙÔÓ ‚·ıÌfi ÎÈÓ‰‡ÓÔ˘ ÛÙËÓ ÔÌ¿‰· µ 100 80 60 40 20 0 0
6
12
18
24
30
36
ª‹Ó˜ ÌÂÙ¿ ÙÔ 1Ô ÂÂÈÛfi‰ÈÔ ¶™ ÷ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ∂Ӊȷ̤ÛÔ˘ ÎÈÓ‰‡ÓÔ˘ À„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ∂ÈÎfiÓ· 1. ™˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ Û ۯ¤ÛË Ì ÙÔÓ ‚·ıÌfi ÎÈÓ‰‡ÓÔ˘ ÛÙËÓ ÔÌ¿‰· µ.
‰‡ÓÔ˘ ·È‰È¿ (p=0,005, ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿), ÂÓÒ ÛÙ· ¯·ÌËÏÔ‡ (p=0,412, ÛÙ·ÙÈÛÙÈο ÌË ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿) Î·È ÛÙ· ÌÂÛ·›Ô˘ ÎÈÓ‰‡ÓÔ˘ (p=0,341, ÛÙ·ÙÈÛÙÈο ÌË ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿) ›¯Â ¯·ÌËÏ‹ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· (¶›Ó·Î·˜ 3). √È ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙËÓ ÔÌ¿‰· ∞ (14%), fiˆ˜ ˘ÓËÏ›· Î·È Â˘ÂÚÂıÈÛÙfiÙËÙ·, ‹Ù·Ó ‹È˜ Î·È ·ÚÔ‰ÈΤ˜, ÂÓÒ Ì·ÎÚÔÚfiıÂÛ̘ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÚÈÒÓ ¯ÚfiÓˆÓ. ¶ÚÔ‚Ï‹Ì·Ù· ÂÏÏÈÔ‡˜ Û˘ÌÌfiÚʈÛ˘ ‹ ÂÊ·ÚÌÔÁ‹˜ Ù˘ ÌÂϤÙ˘ ‰ÂÓ ÛËÌÂÈÒıËηÓ, ‰ÈfiÙÈ ˘‹Ú¯Â Û˘¯Ó‹ ·ʋ Ì ÙÔ˘˜ ÁÔÓ›˜ ÁÈ· ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙˆÓ Ô‰ËÁÈÒÓ. ™Â 14 ÂÚÈÙÒÛÂȘ Ë ˘ÔÙÚÔ‹ ÙˆÓ ¶™ Û˘Ó¤‚Ë Ì ÙËÓ ¿ÓÔ‰Ô ÙÔ˘ ˘ÚÂÙÔ‡. √È 6 ·fi ·˘Ù¤˜ ·Ó‹Î·Ó Û ·È‰È¿ Ù˘ ÔÌ¿‰·˜ ∞, ÛÙ· ÔÔ›· ÔÈ ÁÔÓ›˜ ‰ÂÓ ÚfiÏ·‚·Ó Ó· ¯ÔÚËÁ‹ÛÔ˘Ó ¤ÁηÈÚ· ÙÔ Ê¿ÚÌ·ÎÔ. ∞fi Ù· ·È‰È¿ ·˘Ù¿ 1 ·Ó‹Î ÛÙËÓ ˘ÔÔÌ¿‰· ˘„ËÏÔ‡
¶›Ó·Î·˜ 3. ™‡ÁÎÚÈÛË ÙˆÓ Û˘¯ÓÔÙ‹ÙˆÓ ˘ÔÙÚÔ‹˜ ÛÙ· ·È‰È¿ ¯·ÌËÏÔ‡, ÌÂÛ·›Ô˘ Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ˘ÔÙÚÔ‹˜ ÙˆÓ ÔÌ¿‰ˆÓ ∞ Î·È µ (·Ú·ÎÔÏÔ‡ıËÛË 3 ¯ÚfiÓˆÓ) ÷ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘
ªÂÛ·›Ô˘ ÎÈÓ‰‡ÓÔ˘
À„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘
ÀÔÙÚÔ‹
√Ì¿‰· µ
√Ì¿‰· ∞
Exact Sig. (2-sided)
Ÿ¯È ¡·È ™‡ÓÔÏÔ Ÿ¯È ¡·È ™‡ÓÔÏÔ Ÿ¯È ¡·È ™‡ÓÔÏÔ
15 13 28 9 11 20 4 19 23
18 9 27 13 7 20 13 8 21
p=0,412 NS p=0,341 NS p=0,005 S
NS: ªË ™ËÌ·ÓÙÈÎfi, S: ™ËÌ·ÓÙÈÎfi ¶·È‰È·ÙÚÈ΋ 2007;70:42-48
46
™˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ (%)
∂. ¶·˘Ï›‰Ô˘ Î·È Û˘Ó.
™˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ Û ۯ¤ÛË Ì ÙÔÓ ‚·ıÌfi ÎÈÓ‰‡ÓÔ˘ ÛÙËÓ ÔÌ¿‰· ∞ 100 80 60 40 20 0 0
6 12 18 24 30 ª‹Ó˜ ÌÂÙ¿ ÙÔ 1Ô ÂÂÈÛfi‰ÈÔ ¶™
36
÷ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ∂Ӊȷ̤ÛÔ˘ ÎÈÓ‰‡ÓÔ˘ À„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘
∂ÈÎfiÓ· 2. ™˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ Û ۯ¤ÛË Ì ÙÔÓ ‚·ıÌfi ÎÈÓ‰‡ÓÔ˘ ÛÙËÓ ÔÌ¿‰· ∞.
ÎÈÓ‰‡ÓÔ˘, 3 ÛÙËÓ ÂӉȷ̤ÛÔ˘ Î·È 2 ÛÙË ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘. °È· ÙË ÛˆÛÙfiÙÂÚË ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Ù˘ ÌÂϤÙ˘, ÔÈ Û˘ÁÎÂÎÚÈ̤Ó˜ ˘ÔÙÚÔ¤˜ ‰ÂÓ Î·Ù·ÌÂÙÚ‹ıËηÓ. ªÂÙ¿ ÙËÓ ˘ÔÙÚÔ‹ ÔÈ ÁÔÓ›˜ ¤Ï·‚·Ó Ô‰ËÁ›Â˜ Ó· Û˘Ó¯›ÛÔ˘Ó Î·ÓÔÓÈο ÙÔ ÚˆÙfiÎÔÏÏÔ Ù˘ ÚÔʇϷ͢.
™˘˙‹ÙËÛË ∏ ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË Ì ‰È·˙¿ÌË ÂÊ·ÚÌfiÛÙËΠٷ ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ·fi ÔÏÏÔ‡˜ ÂÚ¢ÓËÙ¤˜ ÛÙËÓ ∂˘ÚÒË Î·È ÙËÓ π·ˆÓ›· (6,9). ∏ ‰È·˙¿ÌË ÌÔÚ› Ó· ¯ÔÚËÁËı› ·fi ÙÔ ÛÙfiÌ·, ˘ÔÁÏÒÛÛÈ· Î·È ·fi ÙÔ ÔÚıfi (10,11). ∏ ÚÈÓÈ΋ ¯ÔÚ‹ÁËÛË ‰È·˙¿Ì˘ ‰ÂÓ ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› ·ÎfiÌË ÁÈ· ÙË ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË ÙˆÓ ¶™ (12). ™ÙË ÌÂϤÙË Ì·˜ ÂÈϤͷÌ ÙË ‰È·˙¿ÌË ·fi ÙÔ ÔÚıfi, ÁÈ·Ù› Â›Ó·È Â‡ÎÔÏË Ë ¯Ú‹ÛË Ù˘, ÊÙ¿ÓÂÈ ÁÚ‹ÁÔÚ· Û ˘„ËÏ¿ ıÂڷ¢ÙÈο ›‰· ÛÙÔÓ ÔÚfi (<10 ÏÂÙ¿) Î·È Ë Ì¤ÁÈÛÙË Û˘ÁΤÓÙÚˆÛ‹ Ù˘ ÂÈÙ˘Á¯¿ÓÂÙ·È Û 30 ̤¯ÚÈ 60 ÏÂÙ¿ (10). √È Minagawa Î·È Û˘Ó. ÌÂϤÙËÛ·Ó ÙË Ê·ÚÌ·ÎÔÎÈÓËÙÈ΋ Ù˘ ‰È·˙¿Ì˘ ·fi ÙÔ ÔÚıfi Û ·ӷϷ̂·ÓfiÌÂÓ˜ ‰fiÛÂȘ. ™Â 6 ‚Ú¤ÊË Ì Â̇ÚÂÙË Ïԛ̈ÍË ¯ÔÚ‹ÁËÛ·Ó ‰È·˙¿ÌË Û ‰‡Ô ‰fiÛÂȘ ÙˆÓ 0,5 mg/kg ·fi ÙÔ ÔÚıfi, Ì ÌÂÛԉȿÛÙËÌ· 8 ˆÚÒÓ Î·È ¤‰ÂÈÍ·Ó fiÙÈ ÂÈÙ‡¯ıËÎ·Ó ıÂڷ¢ÙÈο ›‰· ÛÙÔ Ï¿ÛÌ· Û 30 ÏÂÙ¿ ·fi ÙË ¯ÔÚ‹ÁËÛË Î·È ‰È·ÙËÚ‹ıËÎ·Ó ÁÈ· 24 ÒÚ˜ (13). ∏ ËÏÈΛ· ¤Ó·Ú͢ Ù˘ ÌÂϤÙ˘ Ì·˜, Û‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ·, Î˘Ì·ÈÓfiÙ·Ó ·fi 6 Ì‹Ó˜ ¤ˆ˜ 3 ¯ÚfiÓÈ· ÎÈ ·˘Ùfi ÁÈ·Ù› ÙÔ 90% ÂÚ›Ô˘ ÙˆÓ ·È‰ÈÒÓ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ¶™ Â›Ó·È Î¿Ùˆ ÙˆÓ 3 ÂÙÒÓ. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ ·È‰È¿ Ô˘ ¤¯Ô˘Ó ÙÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ ¶™ Û ÌÈÎÚ‹ ËÏÈΛ·, ¤¯Ô˘Ó ÂÚÈÛÛfiÙÂÚÔ ¯ÚfiÓÔ ÁÈ· Ó· ÂÌÊ·Paediatriki 2007;70:42-48
Ó›ÛÔ˘Ó ˘ÔÙÚÔ‹ Û ۯ¤ÛË Ì ٷ ÌÂÁ·Ï‡ÙÂÚ˘ ËÏÈΛ·˜. √È Offringa Î·È Û˘Ó. (1994) Û ÌÈ· ÌÂÙ·-·Ó¿Ï˘ÛË 5 ÌÂÏÂÙÒÓ ·Û¯ÔÏ‹ıËÎ·Ó Ì ÙÔÓ Î›Ó‰˘ÓÔ ˘ÔÙÚÔ‹˜ Û ۯ¤ÛË Ì ÙËÓ ËÏÈΛ· ¤Ó·Ú͢ Î·È ‚Ú‹Î·Ó ˆ˜ ÔÈ ¶™ ÂÏ·ÙÙÒÓÔÓÙ·Ó Û˘Ó¯Ҙ Ì ÙËÓ ¿ÚÔ‰Ô Ù˘ ËÏÈΛ·˜ ·fi 12 ¤ˆ˜ 42 ÌËÓÒÓ (14). ∞fi ÙË ‚È‚ÏÈÔÁÚ·Ê›· ÁÓˆÚ›˙Ô˘ÌÂ, fiÙÈ Ô Î›Ó‰˘ÓÔ˜ ÂÌÊ¿ÓÈÛ˘ ˘ÔÙÚÔ‹˜ ÙˆÓ ¶™ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Î˘Ì·›ÓÂÙ·È ÌÂٷ͇ 29 Î·È 42,5%, ηٿ ̤ÛÔÓ fiÚÔ 35% (15). ∏ ‰È΋ Ì·˜ ÌÂϤÙË ¤‰ÂÈÍ ˆ˜ Ë Û˘ÓÔÏÈ΋ Û˘¯ÓfiÙËÙ· ˘ÔÙÚÔÒÓ ÛÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÚÈÒÓ ¯ÚfiÓˆÓ ‹Ù·Ó 60% ÛÙËÓ ÔÌ¿‰· Ô˘ ‰ÂÓ ¯ÔÚËÁ‹ıËΠ‰È·˙¿ÌË Î·È 35% ÛÙËÓ ÔÌ¿‰· Ô˘ ¤Ï·‚ ÙÔ Ê¿ÚÌ·ÎÔ. ∫·Ù¿ ÙË ÁÓÒÌË Ì·˜ ÔÈ ˘„ËÏÔ› ·ÚÈıÌÔ› ˘ÔÙÚÔ‹˜ Ô˘ ‚ڋηÌÂ Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ÔÊ›ÏÔÓÙ·È Û ‰‡Ô ÏfiÁÔ˘˜: ·) ÛÙË ÌÂÁ¿ÏË ‰È¿ÚÎÂÈ· ·Ú·ÎÔÏÔ‡ıËÛ˘ (3 ¯ÚfiÓÈ·) Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·‡ÍËÛË Ù˘ Èı·ÓfiÙËÙ·˜ ÂÌÊ¿ÓÈÛ˘ ˘ÔÙÚÔÒÓ Î·È ‚) ÛÙË ÌÈÎÚ‹ ËÏÈΛ· ¤Ó·Ú͢ ÙˆÓ ¶™ (<15 ÌËÓÒÓ) ÛÙÔ 60% ÂÚ›Ô˘ ÙˆÓ ·È‰ÈÒÓ Û οı ÔÌ¿‰·. ™ÙȘ ¿ÏϘ ÌÂϤÙ˜ Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ ÙÔ ÔÛÔÛÙfi ÙˆÓ ·È‰ÈÒÓ Ì ËÏÈΛ· οو ÙˆÓ 15 ÌËÓÒÓ ‰ÂÓ ‹Ù·Ó Û η̛· ÂÚ›ÙˆÛË ÙfiÛÔ ˘„ËÏfi (11,12,14). √È ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ‰È΋˜ Ì·˜, Û˘ÌʈÓÔ‡Ó fiÙÈ Ë ÌÈÎÚ‹ ËÏÈΛ· ¤Ó·Ú͢ Â›Ó·È Ô ÈÔ ÛËÌ·ÓÙÈÎfi˜ ÚÔÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË ˘ÔÙÚÔÒÓ (16-18). °È· Ó· ÂÎÙÈÌ‹ÛÔ˘Ì ÙÔÓ Î›Ó‰˘ÓÔ ˘ÔÙÚÔ‹˜ ηχÙÂÚ·, ˘ÈÔıÂÙ‹Û·Ì ¤Ó·Ó ÚÔÁÓˆÛÙÈÎfi ‰Â›ÎÙË ‚·ÛÈṲ̂ÓÔ Û ¤ÓÙ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘. °È· Ú·ÎÙÈÎÔ‡˜ ÏfiÁÔ˘˜ fiÏÔÈ ÔÈ ·Ú¿ÁÔÓÙ˜ ıˆڋıËÎ·Ó ˆ˜ ¤¯Ô˘Ó ÙËÓ ›‰È· ÚÔÁÓˆÛÙÈ΋ ·Í›·. ∞ÏÏ¿ Î·È ÁÂÓÂÙÈÎÔ› Î·È ÎÔÈÓˆÓÈÎÔ-ÔÈÎÔÓÔÌÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÌÔÚÔ‡Ó Â›Û˘ Ó· ÂËÚ¿ÛÔ˘Ó ÙÔÓ Ù‡Ô, ÙÔÓ ·ÚÈıÌfi, ÙËÓ ·ÏÏËÏ›‰Ú·ÛË Î·È ÙËÓ ÈÛ¯‡ ÙˆÓ ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘. ∏ ÌÂϤÙË Ì·˜ ‰Â›¯ÓÂÈ fiÙÈ Ë ÚÔʇϷÍË Ì ‰È·˙¿ÌË Â› ˘ÚÂÙÔ‡ ÚÔÛʤÚÂÈ ÈηÓÔÔÈËÙÈÎfi ¤ÏÂÁ¯Ô ÙˆÓ ˘ÔÙÚÔÒÓ ÛÙ· ·È‰È¿ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Î·È fi¯È ÛÙ· ·È‰È¿ Ì ¯·ÌËÏfi ‹ ÌÂÛ·›Ô ΛӉ˘ÓÔ ˘ÔÙÚÔ‹˜. ∆· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜ ÂȂ‚·ÈÒÓÔ˘Ó ÙË ÌÂϤÙË ÙÔ˘ Knudsen (1985b), Ô ÔÔ›Ô˜ Ì ÙÔ ›‰ÈÔ ÚÔÊ›Ï ÂÈÎÈÓ‰˘ÓfiÙËÙ·˜, ˘ÔÏfiÁÈÛ ÙË Û˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ ÙˆÓ ¶™ Û ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· 18 ÌËÓÒÓ. ÃÔÚ‹ÁËÛ ‰È·˙¿ÌË ·fi ÙÔ ÔÚıfi 0,5 mg/kg Û ‰‡Ô ‰fiÛÂȘ ÛÙË ‰È¿ÚÎÂÈ· ÂÌ˘Ú¤ÙÔ˘ ÓÔÛ‹Ì·ÙÔ˜, ̤¯ÚÈ Ô ˘ÚÂÙfi˜ Ó· ¤ÛÂÈ Î¿Ùˆ ·fi 38,5ÔC. ∆· Û˘ÌÂÚ¿ÛÌ·Ù· Ù˘ ÌÂϤÙ˘ ¤‰ÂÈÍ·Ó fiÙÈ Ë ‰È·ÏÂ›Ô˘Û· ¯ÔÚ‹ÁËÛË ‰È·˙¿Ì˘ Â›Ó·È ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙ· Ôχ ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·È‰È¿ (ηӤӷ˜ ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘), ¤¯ÂÈ Ì¤ÙÚÈ· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÛÙ· ÌÂÛ·›Ô˘ ÎÈÓ‰‡ÓÔ˘ Î·È Â›Ó·È ÂÍ·ÈÚÂÙÈο ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙ· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘. ™˘ÁÎÂÎÚÈ̤ӷ, ÛÙËÓ ÔÌ¿‰· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ‚ڋΠfiÙÈ Ë Û˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ ÌÂÈÒıËΠ·fi 75-100% Û 10-15% Ì ÙËÓ ÚÔʇϷÍË (17).
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¶˘ÚÂÙÈÎÔ› Û·ÛÌÔ›
∏ ‰È΋ Ì·˜ ÌÂϤÙË ¤¯ÂÈ ÌÂÁ·Ï‡ÙÂÚÔ ¯ÚfiÓÔ ·Ú·ÎÔÏÔ‡ıËÛ˘ (36 Ì‹Ó˜ ·ÓÙ› 18) Î·È ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ ·Ú·ÙËÚ‹ıËÎ·Ó Û ÌÈÎÚfiÙÂÚÔ ÔÛÔÛÙfi, ÏfiÁˆ Ù˘ ¯ÔÚ‹ÁËÛ˘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ÌfiÓÔ ÁÈ· ‰‡Ô Ë̤Ú˜ (3 ‰fiÛÂȘ ÙËÓ ÚÒÙË Ë̤ڷ Î·È 2 ÙË ‰Â‡ÙÂÚË) ÛÙË ‰È¿ÚÎÂÈ· Ù˘ Â̇ÚÂÙ˘ Ïԛ̈͢. ™ÙÔ ›‰ÈÔ Û˘Ì¤Ú·ÛÌ· Ô‰ËÁ‹ıËÎ·Ó Î·È ÔÈ Û˘ÁÁÚ·Ê›˜ Ù˘ ·Ú·¿Óˆ ÌÂϤÙ˘ (Knudsen, 1985b), ÔÈ ÔÔ›ÔÈ ¿ÏÏ·Í·Ó ÙÔ Û¯‹Ì· ÚÔʇϷ͢ Û ٤ÛÛÂÚȘ ‰fiÛÂȘ. ∞fi ÙËÓ ¿ÏÏË ÌÂÚÈ¿, ·ÓÙÈÎÚÔ˘fiÌÂÓ· ·ÔÙÂϤÛÌ·Ù· ÚÔ·ÙÔ˘Ó ·fi ÙȘ ÌÂϤÙ˜ ÙˆÓ Uhari Î·È Û˘Ó. (1995) Î·È ÙˆÓ Autret Î·È Û˘Ó. (1990), ÙˆÓ ÔÔ›ˆÓ ÔÈ ¤Ú¢Ó˜ ¤‰ÂÈÍ·Ó ˆ˜ ‰ÂÓ ÂÈÙ‡¯ıËΠÛËÌ·ÓÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÎÚ›ÛÂˆÓ (11,19). ŸÌˆ˜, ÛÙË ÌÂϤÙË ÙˆÓ Uhari Î·È Û˘Ó. (1995) Ê·›ÓÂÙ·È fiÙÈ Ë ¯·ÌËÏ‹ ‰fiÛË Ù˘ ‰È·˙¿Ì˘, ·Ú¯Èο ·fi ÙÔ ÔÚıfi Î·È ·ÎÔÏÔ‡ıˆ˜ ·fi ÙÔ ÛÙfiÌ· (0,2 mg/kg ÙÚÂȘ ÊÔÚ¤˜/Ë̤ڷ) ‹Ù·Ó ˘Â‡ı˘ÓË ÁÈ· ÙËÓ ¤ÏÏÂÈ„Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜. ∏ ÌÂϤÙË ÙˆÓ Autret Î·È Û˘Ó. (1990) ·ÚÔ˘Û›·˙ ÛÔ‚·Ú¿ ÚÔ‚Ï‹Ì·Ù· Û˘ÌÌfiÚʈÛ˘ ÛÙÔ Û¯‹Ì· ÚÔʇϷ͢, ·ÊÔ‡ ÌfiÓÔ ¤Ó· ÛÙ· 15 ·È‰È¿ Ì ˘ÔÙÚÔ‹ ¤Ï·‚·Ó ÙË ‰È·˙¿ÌË Û‡Ìʈӷ Ì ÙȘ Ô‰ËÁ›Â˜. ™Â ÌÈ· ÚfiÛÊ·ÙË ÌÂϤÙË ÙˆÓ Okumura Î·È Û˘Ó. (2004) ‰ÈÂÚ¢ӋıËÎÂ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ÚÔÊ˘Ï·ÎÙÈ΋˜ ·ÁˆÁ‹˜ Û ·È‰È¿ Ì ¶™, ÛÙ· ÔÔ›· ÙÔ ∏∂° η٤ÁÚ·„ ÂÈÏËÙÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·. ∆· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ ÙÔ˘˜ ¤‰ÂÈÍ·Ó ˆ˜ Ë ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË Ì ‰È·˙¿ÌË ‰ÂÓ Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙË Ì›ˆÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ ˘ÔÙÚÔÒÓ ÙˆÓ ¶™. øÛÙfiÛÔ, Ô ·ÚÈıÌfi˜ ÙˆÓ ·È‰ÈÒÓ Ô˘ ÌÂϤÙËÛ·Ó ‰ÂÓ ‹Ù·Ó ÈηÓÔÔÈËÙÈο ÌÂÁ¿ÏÔ˜ (10/43 ¤Ï·‚·Ó ‰È·ÏÂ›Ô˘Û· ÚÔʇϷÍË ‰È·˙¿Ì˘), Ô‡Ù ·Ó·Ê¤ÚÔ˘Ó ÙÔ Û¯‹Ì· ¯ÔÚ‹ÁËÛ˘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ (20). ™Â ¿ÏϘ ÌÂϤÙ˜ Ê¿ÓËΠfiÙÈ Ë ‰È·ÏÂ›Ô˘Û· ¯ÔÚ‹ÁËÛË ‰È·˙¿Ì˘ ·fi ÙÔ ÛÙfiÌ· ÌÂÈÒÓÂÈ ÛËÌ·ÓÙÈο ÙÔ ÔÛÔÛÙfi ˘ÔÙÚÔÒÓ (21,22). ™ÙȘ ÌÂϤÙ˜ ·˘Ù¤˜ ηٷÁÚ¿ÊËÎ·Ó ÔÈ ›‰È˜ ·ÚÂÓ¤ÚÁÂȘ, fiˆ˜ ÛÙÔ˘˜ ‰ÈÎÔ‡˜ Ì·˜ ·ÛıÂÓ›˜, ·ÏÏ¿ Û ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi. ∞˘Ùfi ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È ÛÙË ÌÂÁ·Ï‡ÙÂÚË ‰È¿ÚÎÂÈ· ¯ÔÚ‹ÁËÛ˘ ‰È·˙¿Ì˘. √È ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ ÛÙË ‰È΋ Ì·˜ ¤Ú¢ӷ ‹Ù·Ó ‹È˜, ·ÚÔ‰ÈΤ˜ Î·È Û η̛· ÂÚ›ÙˆÛË ‰ÂÓ ‹Ù·Ó ÛÔ‚·Ú¤˜. ÕÏÏÔÈ Û˘ÁÁÚ·Ê›˜ ÚÔ‚ÏËÌ·Ù›˙ÔÓÙ·È Ì ÙËÓ ÚÔÊ˘Ï·ÎÙÈ΋ ¯ÔÚ‹ÁËÛË ‰È·˙¿Ì˘, ÂÂȉ‹ ÊÔ‚Ô‡ÓÙ·È Ì‹ˆ˜ ÙÔ˘˜ ‰È·Ê‡ÁÂÈ Ë ‰È¿ÁÓˆÛË ÛÔ‚·Ú‹˜ ˘ÔΛÌÂÓ˘ ÓfiÛÔ˘ (.¯. ÌËÓÈÁÁ›Ùȉ·˜ ‹ ÂÁÎÂÊ·Ï›Ùȉ·˜) (5,23). ŸÛÔÓ ·ÊÔÚ¿ fï˜, ÛÙ· ·È‰È¿ ËÏÈΛ·˜ οو ÙˆÓ 18 ÌËÓÒÓ Ì ¶™, ÛÙË ‰È·ÁÓˆÛÙÈ΋ Ì·˜ ÛΤ„Ë Ì·›ÓÂÈ ·˘ÙÔÌ¿Ùˆ˜ Î·È Ë Èı·Ó‹ Ïԛ̈ÍË ÙÔ˘ ∫¡™. ™Â ‡ÔÙ˜ ÂÚÈÙÒÛÂȘ Á›ÓÂÙ·È Î·Ù¿ ηÓfiÓ· ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙËÛË (√¡¶). ™ÙË ÌÂϤÙË Ì·˜ ¤ÁÈÓ √¡¶ Û ‰¤Î· ·È‰È¿ ÁÈ· ‰È·ÁÓˆÛÙÈÎÔ‡˜ ÏfiÁÔ˘˜ Î·È ‹Ù·Ó fiϘ ·ÚÓËÙÈΤ˜.
∆· ·ÚÈ· ÂÚˆÙ‹Ì·Ù· Ô˘ ÚÔ‚ÏËÌ·Ù›˙Ô˘Ó ÙÔ˘˜ ÂÚÈÛÛfiÙÂÚÔ˘˜ ÂÚ¢ÓËÙ¤˜ Â›Ó·È Î·Ù¿ fiÛÔÓ ÔÈ ¶™ ÌÔÚÔ‡Ó Ó· ‚Ï¿„Ô˘Ó ÙÔÓ ÂÁΤʷÏÔ, Î·È È‰›ˆ˜ ÙÔÓ ·ÓÒÚÈÌÔ, ‹ ·ÎfiÌË Î·È Ó· ÚÔηϤÛÔ˘Ó ı¿Ó·ÙÔ ÙˆÓ Ó¢ÚÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È ˆ˜ ÂÎ ÙÔ‡ÙÔ˘ ÂÈ‚¿ÏÏÂÙ·È Ë ÚfiÏË„‹ ÙÔ˘˜ (24,25). ∞ÓÙ›ıÂÙ˜ ÚÔ˜ ·˘Ù‹Ó ÙËÓ ¿Ô„Ë Â›Ó·È ÔÈ ÌÂϤÙ˜ ÙˆÓ Verity Î·È Û˘Ó. (1991, 1998) (26,27). √È Morell Î·È Û˘Ó. Û ÂÈÚ·Ì·ÙÈÎfi ÌÔÓÙ¤ÏÔ ¤‰ÂÈÍ·Ó ˆ˜ ÔÈ Û·ÛÌÔ› (οو ·fi Û˘Óı‹Î˜ ˘ÚÂÙÔ‡) Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÚÔηϤÛÔ˘Ó ·˘ÍË̤ÓË Â˘·ÈÛıËÛ›· ÛÙË Á¤ÓÂÛË ÙˆÓ Û·ÛÌÒÓ ÂÓÂÚÁÔÔÈÒÓÙ·˜ Ì˯·ÓÈÛÌÔ‡˜ ÛÙÔÓ ÂÁΤʷÏÔ (28). ªÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó fiÙÈ ÔÈ ˘ÔÙÚÔÈ¿˙ÔÓÙ˜ Û·ÛÌÔ› Û¯ÂÙ›˙ÔÓÙ·È Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ Â·ÎfiÏÔ˘ı˘ ÂÈÏË„›·˜ (24,25). ∞ÓÙ›ıÂÙ· ÔÈ Berg Î·È Shinnar (1994a) ¤‰ÂÈÍ·Ó ˆ˜ Ô Î›Ó‰˘ÓÔ˜ ηٿÏË͢ Û ÂÈÏË„›· Û ·È‰È¿ ÌÂÙ¿ ·fi ¶™ Â›Ó·È ¯·ÌËÏfi˜ (29). ÕÏÏÔÈ ¿ÏÈ ·Ó·Ê¤ÚÔÓÙ·È Û ÌÂÁ·Ï‡ÙÂÚ· ÔÛÔÛÙ¿ ÂÌÊ¿ÓÈÛ˘ ÂÈÏË„›·˜ Û ·È‰È¿ Ì ˘„ËÏfi ΛӉ˘ÓÔ ˘ÔÙÚÔ‹˜ ÙˆÓ ¶™. ¶ÈÔ ·Ó·Ï˘ÙÈο ÙÔÓ›˙Ô˘Ó ˆ˜ Ù· ˘ÔÙÚÔÈ¿˙ÔÓÙ· ÂÂÈÛfi‰È· ¶™ ·ÔÙÂÏÔ‡Ó ÛËÌ·ÓÙÈÎfi ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙËÓ Â·ÎfiÏÔ˘ıË ÂÌÊ¿ÓÈÛË ÂÈÏË„›·˜. ∏ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ÂÈÏË„›·˜ ÛÙ· ·È‰È¿ Ì ¤Ó· ·Ïfi ÂÂÈÛfi‰ÈÔ ¶™ Î·È ¯ˆÚ›˜ Î·Ó¤Ó·Ó ¿ÏÏÔ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ˘ÔÏÔÁ›ÛÙËΠÂÚ›Ô˘ Û 1%, ÂÓÒ ÛÙ· ·È‰È¿ Ì ٤ÛÛÂÚ· ‹ ÂÚÈÛÛfiÙÂÚ· ÂÂÈÛfi‰È· ¶™ ÛÙÔ ÈÛÙÔÚÈÎfi ÙÔ˘˜ Ô Î›Ó‰˘ÓÔ˜ ·˘Ùfi˜ ·Ó¤Ú¯ÂÙ·È Û 4% (19,25,30,31). °ÂÓÈο, ÔÈ ÁÈ·ÙÚÔ› ı· Ú¤ÂÈ Ó· ηıËÛ˘¯¿˙Ô˘Ó ÙÔ˘˜ ÁÔÓ›˜ Î·È Ó· ÙÔ˘˜ ‰È·‚‚·ÈÒÓÔ˘Ó ÁÈ· ÙËÓ Î·Ï‹ ÔÚ›· ÙˆÓ ¶™. ™‡Ìʈӷ Ì ÌÈ· ÚfiÛÊ·ÙË ¤Ú¢ӷ, Ë ÛˆÛÙ‹ ÂÓË̤ڈÛË ÙˆÓ ÁÔÓÈÒÓ ÙÔ‡˜ ÂÌ„˘¯ÒÓÂÈ Î·È ÌÂÈÒÓÂÈ ÙÔ ¿Á¯Ô˜ ÙÔ˘˜ (32). ∫·Ù¿ ÙËÓ ¿Ô„‹ Ì·˜, Ë Î·Ù·ÏÏËÏfiÙÂÚË ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ¶™ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÚfiÏË„Ë ÙˆÓ ˘ÔÙÚÔÒÓ ÌfiÓÔ ÛÙÔ˘˜ ÏËı˘ÛÌÔ‡˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘. ∞˘Ùfi ÌÔÚ› Ó· Á›ÓÂÈ ·ÔÙÂÏÂÛÌ·ÙÈο Ì ÙË ‰È·ÏÂ›Ô˘Û· ¯ÔÚ‹ÁËÛË ‰È·˙¿Ì˘ ·fi ÙÔ ÔÚıfi, ·ÚΛ Ó· ‰›ÓÂÙ·È ¤ÁηÈÚ· Î·È ÛˆÛÙ¿ ÛÙËÓ ¤Ó·ÚÍË ÙÔ˘ ˘ÚÂÙÔ‡.
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CASE REPORT
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ÀÔ¯ÔÓ‰ÚÔÏ·Û›· Ì ÌÂÙ¿ÏÏ·ÍË ¡540∫ ÛÙÔÓ ˘Ô‰Ô¯¤· 3 ÙÔ˘ ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ - FGFR3. ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ µ. ∞ÁÁÂÏ¿ÎÔ˘, °. ¡ÈˆÙ¿Î˘, ™. ∏Ï›·, ™. ª¿ÎˆÛË, ∂. ∫fiÎÔÚË ¶ÂÚ›ÏË„Ë: ∏ ˘Ô¯ÔÓ‰ÚÔÏ·Û›· (ªπª 146000) Â›Ó·È ·˘ÙÔۈ̷ÙÈ΋ ÂÈÎÚ·ÙËÙÈ΋ ÛÎÂÏÂÙÈ΋ ‰˘ÛÏ·Û›· ÌÂ Û˘¯ÓfiÙËÙ· 1:30.000 ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi. ™˘¯Ó¿, ÔÏϤ˜ ÂÚÈÙÒÛÂȘ ‰È·Ê‡ÁÔ˘Ó Ù˘ ‰È¿ÁÓˆÛ˘ ‹ ıˆÚÔ‡ÓÙ·È Ï·Óı·Ṳ̂ӷ ˆ˜ ÂÚÈÙÒÛÂȘ ÔÈÎÔÁÂÓÔ‡˜ ‚Ú·¯˘ÛˆÌ›·˜, ÂÂȉ‹ ÔÈ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ Â›Ó·È ‹È˜. ∏ ‰È¿ÁÓˆÛË ÌÔÚ› Ó· Á›ÓÂÈ Î·ı˘ÛÙÂÚË̤ӷ (ÛÙ· 2-4 ¯ÚfiÓÈ·), fiÙ·Ó ‰È·ÈÛÙÒÓÂÙ·È fiÙÈ ÙÔ ·È‰› ÛÙ·ıÂÚ¿ ‚Ú›ÛÎÂÙ·È Î¿Ùˆ ·fi ÙËÓ 3Ë ∂.£., ÂÓÒ Ë ÚÔÛÂÎÙÈ΋ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË ·ÔηχÙÂÈ ÙË ÚÈ˙ÔÌÂÏÈ΋ ‚Ú¿¯˘ÓÛË ÙˆÓ ¿ÎÚˆÓ. ™Ù· ÎÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ‹È· ‚Ú·¯˘ÛˆÌ›·, ÎÔÓÙ¿ ¿ÎÚ·, ÏfiÚ‰ˆÛË Ù˘ ÔÛÊ˘˚΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘, ÎÔÓÙ¤˜ Î·È Ï·ÙÂȤ˜ ·Ï¿Ì˜ Î·È ¤ÏÌ·Ù·, ÚÔ¤¯ÔÓ Ì¤ÙˆÔ Î·È ·Ô‡Û· ‹ ‹È· Ì·ÎÚÔÎÂÊ·Ï›·. ¶ÚfiÛÊ·Ù· ‚Ú¤ıËΠfiÙÈ ÛÙÔ 70% ÂÚ›Ô˘ ÙˆÓ ·ÛıÂÓÒÓ Ì ˘Ô¯ÔÓ‰ÚÔÏ·Û›· ˘¿Ú¯ÂÈ ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ÁÔÓ›‰ÈÔ ÙÔ˘ ˘Ô‰Ô¯¤· 3 ÙÔ˘ ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ (FGFR3), Ô˘ ‚Ú›ÛÎÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 4p16.3. ¶ÂÚÈÁÚ¿ÊÔ˘Ì ÎÔÚ›ÙÛÈ ËÏÈΛ·˜ 7ó ÂÙÒÓ, Ì ‰˘Û·Ó¿ÏÔÁÔ ÎÔÓÙfi ·Ó¿ÛÙËÌ·, Î·È Ì ÎÏÈÓÈο Î·È ·ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· Û˘Ì‚·Ù¿ Ì ÙË ‰È¿ÁÓˆÛË Ù˘ ˘Ô¯ÔÓ‰ÚÔÏ·Û›·˜. ∏ ÌÔÚȷ΋ ÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË ¤‰ÂÈÍ fiÙÈ Ë ·ÛıÂÓ‹˜ Â›Ó·È ÂÙÂÚÔ˙˘ÁÒÙ˘ ÁÈ· ÙË ÌÂÙ¿ÏÏ·ÍË C1620A ÛÙÔ ¯ÚˆÌfiۈ̷ 4p16.3, Ô˘ Ô‰ËÁ› ÛÙËÓ ·ÓÙÈηٿÛÙ·ÛË ¡540∫ ÛÙÔÓ FGFR3 Î·È ·ÓÙÈÚÔۈ‡ÂÈ ÙËÓ ÈÔ Û˘¯Ó‹ ÌÂÙ¿ÏÏ·ÍË ÛÙËÓ ˘Ô¯ÔÓ‰ÚÔÏ·Û›·.
∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ µÂÓÈ˙¤ÏÂÈÔ˘ ¡ÔÛÔÎÔÌ›Ԣ, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘ AÏÏËÏÔÁÚ·Ê›·: µ·ÛÈÏÈ΋ ∞ÁÁÂÏ¿ÎÔ˘ vaagge3@yahoo.gr °Ô‡ÚÓ˜ ∆Â̤ÓÔ˘˜ ∆.∫. 71 500 ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘
§¤ÍÂȘ ÎÏÂȉȿ: ÀÔ¯ÔÓ‰ÚÔÏ·Û›·, ÛÎÂÏÂÙÈ΋ ‰˘ÛÏ·Û›·, FGFR3, ‰˘Û·Ó¿ÏÔÁÔ ÎÔÓÙfi ·Ó¿ÛÙËÌ·, ·˘ÍËÙÈ΋ ÔÚÌfiÓË.
Hypochondroplasia with the mutation N540K of the fibroblast growth factor receptor 3 - FGFR3. A case report V. Aggelakou, G. Niotakis, S. Ilia, S. Bakosi, E. Kokori
1st Pediatric Clinic, Venizeleio Hospital, Herakleion, Crete
Abstract: Hypochondroplasia (MIM 146000) is an autosomal dominant skeletal dysplasia affecting 1 in 30,000 individuals. It is not uncommon for many cases to be overlooked or misdiagnosed as being familial short stature, since the clinical manifestations may be mild, and the diagnosis is often delayed (2-4 years). Suspicions arise when the child is noted to “fall off the growth curve”, and a careful examination reveals the rhizomelic shortening. Clinical features of hypochondroplasia include mild short stature, micromelia, lumbar lordosis, short broad hands and feet, a large forehead and absent or mild macrocephaly. Recently, it has been reported that almost 70% of patients with hypochondroplasia carry a gene mutation encoding for the receptor 3 of the fibroblast growth factor (FGFR3) on chromosome 4q16.3. The case is described of a 7ó year-old girl who presented with disproportional short stature and clinical and radiological findings consistent with hypochondroplasia. Molecular genetic analysis confirmed that the patient was heterozygous for the C1620A mutation resulting in N540K substitution in FGFR3, the most common mutation in hypochondroplasia.
Correspondence: Vassiliki Aggelakou vaagge3@yahoo.gr Gournes Temenous 71 500, Herakleion, Crete
Key words: Hypochondroplasia, skeletal dysplasia, FGFR3, disproportional short stature, growth hormone.
∂ÈÛ·ÁˆÁ‹ ∏ ˘Ô¯ÔÓ‰ÚÔÏ·Û›· (ÀÃ) ·Ó‹ÎÂÈ ÛÙËÓ ÔÌ¿‰· Ù˘ ·¯ÔÓ‰ÚÔÏ·Û›·˜ Ì ‚¿ÛË ÌÈ· Ó¤· ·Ó·ıˆÚË̤ÓË ‰ÈÂıÓ‹ ÔÓÔÌ·ÙÔÏÔÁ›· Î·È Ù·ÍÈÓfiÌËÛË (1), Ì·˙› Ì ÙËÓ ·¯ÔÓ‰ÚÔÏ·Û›· Î·È ÙË ı·Ó·ÙÔÙÚÔÊÈ΋ ‰˘ÛÏ·Û›· Î·È ÌÂÙ·‚È‚¿˙ÂÙ·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ¯·Ú·ÎÙ‹Ú·. ∏ ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹ ·ÊÔÚ¿ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ ÙÔ˘ ˘Ô‰Ô¯¤· 3 ÙÔ˘ ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·
ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ (FGFR 3) (∂ÈÎfiÓ· 1), Ô˘ ‚Ú›ÛÎÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 4p16.3 (2). √È ÂÚÈÛÛfiÙÂÚ˜ ÌÂÙ·ÏÏ¿ÍÂȘ Â›Ó·È Ù˘¯·›Â˜ (de novo), ÂÓÒ ˘¿Ú¯ÂÈ ÌÂÁ¿ÏË Ê·ÈÓÔÙ˘È΋ ÔÈÎÈÏÔÌÔÚÊ›· Ù˘ Àà ÏfiÁˆ ÙÔ˘ ›‰Ô˘˜ ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ (2). ∏ Û˘¯ÓfiÙËÙ· ˘ÔÏÔÁ›˙ÂÙ·È Û 1:30.000 ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi. ∫ÏÈÓÈο ·Ú·ÙËÚÂ›Ù·È ‹È· ‚Ú·¯˘ÛˆÌ›· (‡„Ô˜ 145-165 cm ÛÙ· ·ÁfiÚÈ· Î·È 133-150 cm ÛÙ· ÎÔÚ›ÙÛÈ·), ÎÔÓÙ¿ ¿ÎÚ·, ÚÔ¤¯ÔÓ Ì¤ÙˆÔ, ¶·È‰È·ÙÚÈ΋ 2007;70:49-53
50
µ. ∞ÁÁÂÏ¿ÎÔ˘ Î·È Û˘Ó.
S
Ig I
A
Ig II
Ig III
TM
TK1
TK2
NH2
COOH
TD I
ACH
HCH
TD II
TD I
∂ÈÎfiÓ· 1. ™¯ËÌ·ÙÈ΋ ·Ú¿ÛÙ·ÛË Ù˘ ÚˆÙÂ˚ÓÈ΋˜ ‰ÔÌ‹˜ ÙÔ˘ FGFR-3 Î·È Ù˘ ı¤Û˘ ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ Ô˘ ÚÔηÏÔ‡Ó ÙÚÂȘ ÛÎÂÏÂÙÈΤ˜ ‰˘ÛϷۛ˜: ·¯ÔÓ‰ÚÔÏ·Û›· (ACH), ˘Ô¯ÔÓ‰ÚÔÏ·Û›· (HCH) Î·È ı·Ó·ÙÔÙÚÔÊÈ΋ ¯ÔÓ‰ÚÔ‰˘ÛÏ·Û›· Ù‡Ô˘ π Î·È ππ (TD I, TD II). TM: ‰È·ÌÂÌ‚Ú·ÓÈÎfi ÙÌ‹Ì·. ∆∫1, ∆∫2: ÙÌ‹Ì·Ù· ÎÈÓ¿ÛË Ù˘ÚÔÛ›Ó˘ 1 Î·È 2. Ig π, Ig II, Ig III: ÙÌ‹Ì·Ù· Ô˘ ÌÔÈ¿˙Ô˘Ó Ì ·ÓÔÛÔÛÊ·ÈÚ›ÓË.
·Ô‡Û· ‹ ‹È· Ì·ÎÚÔÎÂÊ·Ï›·, ÏfiÚ‰ˆÛË Ù˘ ÔÛÊ˘˚΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ Î·È ÎÔÓÙ¿ Î·È Ï·ÙÈ¿ ÌÂٷοÚÈ· Î·È Ê¿Ï·ÁÁ˜ (3). ∆· ·ÎÙÈÓÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÌÂȈ̤ÓË ‰È·˘¯ÂÓÈ΋ ·fiÛÙ·ÛË ÛÙÔ˘˜ ηÙÒÙÂÚÔ˘˜ ÔÛÊ˘˚ÎÔ‡˜ ÛÔÓ‰‡ÏÔ˘˜, ¢ڤ· Î·È ÎÔÓÙ¿ Ì·ÎÚ¿ ÔÛÙ¿, ÌÂٷοÚÈ· Î·È Ê¿Ï·ÁÁ˜, ηıÒ˜ Î·È Â˘Ú‡ Î·È ÎÔÓÙfi ·˘¯¤Ó· ÙÔ˘ ÌËÚÈ·›Ô˘, ÂÓÒ ÙÔ Ì‹ÎÔ˜ Ù˘ ÂÚfiÓ˘ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚÔ Ù˘ ÎÓ‹Ì˘ (4). ¶·ÚÔ˘ÛÈ¿˙Ô˘Ì ÂÚ›ÙˆÛË ˘Ô¯ÔÓ‰ÚÔÏ·Û›·˜ Ì ÌÂÙ¿ÏÏ·ÍË ¡540∫ ÛÙÔÓ FGFR 3, ÛÙËÓ ÔÔ›· Ë ‰È¿ÁÓˆÛË Ù¤ıËΠηı˘ÛÙÂÚË̤ӷ Û ËÏÈΛ· 7,5 ÂÙÒÓ, Ì ÛÎÔfi Ó· ¢·ÈÛıËÙÔÔÈ‹ÛÔ˘Ì ÙÔ˘˜ Û˘Ó·‰¤ÏÊÔ˘˜ ·È‰È¿ÙÚÔ˘˜, ÒÛÙ ӷ ·Ó·ÁÓˆÚ›˙Ô˘Ó ¤ÁηÈÚ· ÙË ÓfiÛÔ Î·È Ó· ÌÔÚÔ‡Ó Ó· ˙ËÙ‹ÛÔ˘Ó ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ÁÂÓÂÙÈ΋ ÙÂÎÌËÚ›ˆÛË. ∏ ÂÚ›ÙˆÛË Â›Ó·È Ë ÚÒÙË Ô˘ ‰ËÌÔÛȇÂÙ·È ÛÙËÓ ÚÔÛÈÙ‹ ÂÏÏËÓÈ΋ ‚È‚ÏÈÔÁÚ·Ê›·.
¶ÂÚÈÁÚ·Ê‹ ÂÚÈÛÙ·ÙÈÎÔ‡ ¶ÚfiÎÂÈÙ·È ÁÈ· ÎÔÚ›ÙÛÈ ËÏÈΛ·˜ 7ó ÂÙÒÓ Ô˘ Ó ÔÛËχıËΠÁÈ· Ïԛ̈ÍË ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡. ™ÙÔ ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi Ù˘ ·Ó·Ê¤ÚÂÙ·È fiÙÈ ÁÂÓÓ‹ıËΠÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ÙÔÎÂÙfi, ÌÂÙ¿ ·fi ÙÂÏÂÈfiÌËÓË Î‡ËÛË Ì ‚¿ÚÔ˜ 2.930 g (25Ë ∂.£.), Ì‹ÎÔ˜ 46 cm (3Ë ∂.£.) Î·È ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜ 34 cm (50‹ ∂.£.). ™Â ËÏÈΛ· 3 ÂÙÒÓ ÙÔ ‡„Ô˜ ‹Ù·Ó 81 cm (-4 SD ·fi 50‹ ∂.£.), ÙÔ ‚¿ÚÔ˜ 11,5 kg (-2 SD ·fi 50‹ ∂.£.) Î·È Ë ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜ 48 cm (-2 SD ·fi 50 ‹ ∂.£.), ÂÓÒ ‰ÂÓ ˘¿Ú¯Ô˘Ó ¿ÏϘ ηٷÁÂÁÚ·Ì̤Ó˜ ÌÂÙÚ‹ÛÂȘ. §fiÁˆ Ù˘ ÌÂÁ¿Ï˘ ·fiÎÏÈÛ˘ ÙÔ˘ ‡„Ô˘˜ ·fi ÙË Ì¤ÛË ÙÈÌ‹ ÁÈ· ÙËÓ ËÏÈΛ· Ù˘, ¤ÁÈÓ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ Ô˘ ·¤ÎÏÂÈÛ ٷ ¯ÚfiÓÈ· ÓÔÛ‹Ì·Ù· Î·È ÙËÓ ¤ÏÏÂÈ„Ë ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘, ÂÓÒ Èı·ÓÔÏÔÁ‹ıËΠοÔÈ· ÌÔÚÊ‹ ÛÎÂÏÂÙÈ΋˜ ‰˘ÛÏ·Û›·˜, ¯ˆÚ›˜ fï˜ Ó· Á›ÓÂÈ ÂÚ·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô˜. ∏ „˘¯ÔÎÈÓËÙÈ΋ ÂͤÏÈÍË Ù˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó ÔÌ·Ï‹, Ì ηϋ Û¯ÔÏÈ΋ ›‰ÔÛË. √È ÁÔÓ›˜ Î·È Ô ·‰ÂÏÊfi˜ Ù˘ ¤¯Ô˘Ó Ê˘ÛÈÔÏÔÁÈÎfi ‚¿ÚÔ˜, ‡„Ô˜ Î·È ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜, ¯ˆÚ›˜ ‰˘Û·Ó·ÏÔÁ›· ÎÔÚÌÔ‡-¿ÎÚˆÓ. Paediatriki 2007;70:49-53
∏ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË Î·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ Ù˘ ÛÙËÓ ÎÏÈÓÈ΋ ·ÔÎ¿Ï˘„ ˘ÔÏÂÈfiÌÂÓË ÛˆÌ·ÙÈ΋ ·Ó¿Ù˘ÍË Î·È ‰˘Û·Ó¿ÏÔÁÔ ÎÔÓÙfi ·Ó¿ÛÙËÌ· Ì ÎÔÓÙ¿ ¿Óˆ Î·È Î¿Ùˆ ¿ÎÚ· [‡„Ô˜: 102,5 cm (-6 SD ·fi 50‹ ∂.£.), ‚¿ÚÔ˜: 18 kg (-2,5 SD ·fi 50‹ ∂.£.), ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜: 50 cm (-2 SD ·fi 50‹ ∂.£.), ηıÈÛÙfi ‡„Ô˜: 59 cm, ¿ÓÔÈÁÌ· ¯ÂÚÈÒÓ: 96 cm, ·Ó·ÏÔÁ›· ¿Óˆ/οو ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÛÒÌ·ÙÔ˜: 1,35 (Ê.Ù. ÁÈ· ÙËÓ ËÏÈΛ·: 1,06), ‰È·ÊÔÚ¿ ·ÓÔ›ÁÌ·ÙÔ˜ ¯ÂÚÈÒÓ - ‡„Ô˜: 0,65 (Ê.Ù.: -2) Î·È ÏfiÁÔ˜ ηıÈÛÙÔ‡ ‡„Ô˘˜ / Û˘ÓÔÏÈÎfi ‡„Ô˜: 0,575 (+3 SD ·fi 50‹ ∂.£.)]. ∆· ‰¿ÎÙ˘Ï¿ Ù˘ ‹Ù·Ó ÎÔÓÙ¿ Î·È Ï·ÙÈ¿, ÂÓÒ ·Ú·ÙËÚ‹ıËΠ‹È· ÏfiÚ‰ˆÛË Ù˘ ÔÛÊ˘˚΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘. ¢ÂÓ ÂÌÊ¿ÓÈ˙ ‰˘ÛÌÔÚÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÛÙÔ ÚfiÛˆÔ Î·È ÛÙÔ ÎÚ·Ó›Ô (∂ÈÎfiÓ· 2). ∏ ÔÛÙÈ΋ ËÏÈΛ· ‹Ù·Ó Û˘Ì‚·Ù‹ Ì ÙË ¯ÚÔÓÔÏÔÁÈ΋. ∆· ·ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· ·ÊÔÚÔ‡Û·Ó ÎÔÓÙ¿ Î·È Ê·Ú‰È¿ ÌÂٷοÚÈ· Î·È Ê¿Ï·ÁÁ˜ ¿Óˆ ¿ÎÚˆÓ, Ì›ˆÛË Ù˘ ‰È·˘¯ÂÓÈ΋˜ ·fiÛÙ·Û˘ ·fi ÙÔÓ √1 ¤ˆ˜ ÙÔÓ √5 ÛfiÓ‰˘ÏÔ, ÎÔÓÙfi Î·È Â˘Ú‡ ·˘¯¤Ó· ÌËÚÈ·›Ô˘ ÔÛÙÔ‡, ‹È· ÂÏ·Ù˘Ṳ̂ӷ Î·È ÎÔÓÙ¿ Ï·ÁfiÓÈ· ÔÛÙ¿ Ì ÂȤ‰ˆÛË Ù˘ ÔÚÔÊ‹˜ Ù˘ ÎÔÙ‡Ï˘, ÂÚfiÓË ÌÂÁ·Ï‡ÙÂÚË Ù˘ ÎÓ‹Ì˘ Î·È Ù¤ÏÔ˜, ÎÔÓÙ¿ Î·È Ê·Ú‰È¿ Ì·ÎÚ¿ ÔÛÙ¿ ÙˆÓ Î¿Ùˆ ¿ÎÚˆÓ (∂ÈÎfiÓ· 3). √ ˘fiÏÔÈÔ˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜ fiˆ˜ Î·È Ô ¤ÏÂÁ¯Ô˜ ı˘ÚÂÔÂȉԇ˜ Î·È ·Ú·ı˘ÚÂÔÂȉÒÓ ·‰¤ÓˆÓ [·Ú·ıÔÚÌfiÓË: 31 pg/ml (Ê.Ù. 1-43), TSH: 1,90 IU/ml (Ê.Ù. 0,4-4), FT3: 5,4 pmol/L (Ê.Ù. 2,2-6,2), FT4 18,2 pmol/L (Ê.Ù. 10,224,4)], Ë ¤ÎÎÚÈÛË ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ Ì ‰ÔÎÈ̷ۛ˜ ‡ÓÔ˘ Î·È ¿ÛÎËÛ˘, ηıÒ˜ Î·È ÔÈ ÙÈ̤˜ ÙÔ˘ ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· IGF-1 Î·È Ù˘ ÚˆÙ½Ó˘ IGF-BP3. ªÂ ‚¿ÛË Ù· ·ÓˆÙ¤Úˆ ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Ô˘ Û˘ÓËÁÔÚÔ‡Û·Ó ˘¤Ú Ù˘ ÀÃ, ‰Â›ÁÌ· ·›Ì·ÙÔ˜ ÂÛÙ¿ÏË ÁÈ· ÌÔÚÈ·Îfi ÁÔÓȉȷÎfi ¤ÏÂÁ¯Ô ÛÙÔ ∂˘Úˆ·˚Îfi ¢›ÎÙ˘Ô ™ÎÂÏÂÙÈÎÒÓ ¢˘ÛÏ·ÛÈÒÓ (European Skeletal Dysplasia Network: www.esdn.org) Î·È ‰È·ÈÛÙÒıËΠfiÙÈ Ë ·ÛıÂÓ‹˜ Â›Ó·È ÂÙÂÚÔ˙˘ÁÒÙ˘ ÁÈ· ÙË
51
ÀÔ¯ÔÓ‰ÚÔÏ·Û›· ÌÂ ÌÂÙ¿ÏÏ·ÍË ¡540∫ ÛÙÔÓ FGFR3
∞
°
∂ÈÎfiÓ· 2. ∏ ·ÛıÂÓ‹˜ Û ËÏÈΛ· 10 ÂÙÒÓ.
ÌÂÙ¿ÏÏ·ÍË C1620A ÛÙÔ ÁÔÓ›‰ÈÔ ÙÔ˘ FGFR3. ∏ ÌÂÙ¿ÏÏ·ÍË ·˘Ù‹ Ô˘ Ô‰ËÁ› ÛÙËÓ ·ÓÙÈηٿÛÙ·ÛË ¡540∫ ÛÙÔÓ FGFR3 ·ÔÙÂÏ› ÙËÓ ÈÔ Û˘¯Ó‹ Î·È ÛÔ‚·Ú‹ ÌÂÙ¿ÏÏ·ÍË Û ·ÛıÂÓ›˜ Ì ˘Ô¯ÔÓ‰ÚÔÏ·Û›·. √ ÁÔÓȉȷÎfi˜ ¤ÏÂÁ¯Ô˜ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ‹Ù·Ó ·ÚÓËÙÈÎfi˜ ÁÈ· ÙË ÌÂÙ¿ÏÏ·ÍË.
™˘˙‹ÙËÛË ∏ ÀÃ Â›Ó·È Ë ËÈfiÙÂÚË ÌÔÚÊ‹ ÛÎÂÏÂÙÈ΋˜ ‰˘ÛÏ·Û›·˜ ÛÙËÓ ÔÌ¿‰· Ù˘ ·¯ÔÓ‰ÚÔÏ·Û›·˜ (5,6), Î·È ÔÊ›ÏÂÙ·È Û˘Ó‹ıˆ˜ Û ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔÓ FGFR3 (6,7). √ FGFR3 Â›Ó·È ÌÈ· ÁÏ˘ÎÔÚˆÙ½ÓË Ô˘ ·Ó‹ÎÂÈ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ ˘Ô‰Ô¯¤ˆÓ Ù˘ Ù˘ÚÔÛÈÓÈ΋˜ ÎÈÓ¿Û˘ (∆∫). ∏ ·¯ÔÓ‰ÚÔÏ·Û›· ÚÔηÏÂ›Ù·È ·fi ÛËÌÂȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ‰È·ÌÂÌ‚Ú·ÓÈÎfi ÙÌ‹Ì· ÙÔ˘ FGFR3, ÂÓÒ Ë ÀÃ, fiˆ˜ Î·È Ë ı·Ó·ÙÔÙÚÔÊÈ΋ ‰˘ÛÏ·Û›·, ·fi ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙË ‰ÔÌ‹ Ù˘ ∆∫1, TK2 ·ÏÏ¿ Î·È ¿ÏÏˆÓ ÙÌËÌ¿ÙˆÓ ÙÔ˘ FGFR3 (∂ÈÎfiÓ· 1) (7). ∏ ÌÂÁ¿ÏË ÎÏÈÓÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· ÙˆÓ ÚÔ·Ó·ÊÂÚı¤ÓÙˆÓ ÓÔÛËÌ¿ÙˆÓ ÔÊ›ÏÂÙ·È ÛÙË ‰È·ÊÔÚÂÙÈ΋˜ ‚·Ú‡ÙËÙ·˜ ‰È·Ù·Ú·¯‹ Ù˘ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ·˜ ÙÔ˘ FGFR3 Ô˘ ÚÔηÏÔ‡Ó ÔÈ ‰È¿ÊÔÚ˜ ÌÂÙ·ÏÏ¿ÍÂȘ (7). ™ÙÔ 70% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Àà ¤¯Ô˘Ó Ù·˘ÙÔÔÈËı› ÂÓÓ¤· ÌÂÙ·ÏÏ¿ÍÂȘ ÁÈ· ÙËÓ ∆∫1 ÛÙÔ ÁÔÓ›‰ÈÔ ÙÔ˘ FGFR3, ÔÈ Ôԛ˜ ÚÔηÏÔ‡Ó ÂÙ¿ ‰È·ÊÔÚÂÙÈΤ˜ ·ÓÙÈηٷÛÙ¿ÛÂȘ ·ÌÈÓÔͤˆÓ (2,8), Ì ÈÔ Û˘¯Ó‹ ÌÂÙ¿ÏÏ·ÍË ÙË ¡540∫ Ô˘ ·ÓÙÈÚÔۈ‡ÂÈ ÂÚ›Ô˘ ÙÔ 50% fiÏˆÓ ÙˆÓ ÌÂÙ·ÏÏ¿ÍÂ-
µ
¢
∂ÈÎfiÓ· 3. ∞ÎÙÈÓÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù·: ∞. ∫ÔÓÙ‹ Î·È Ï·ÙÈ¿ ÎÓ‹ÌË Î·È ÂÚfiÓË Ì·ÎÚ‡ÙÂÚË Ù˘ ÎÓ‹Ì˘. µ. ¢È·˘¯ÂÓÈ΋ ·fiÛÙ·ÛË ÛÙÔ˘˜ ηÙÒÙÂÚÔ˘˜ ÔÛÊ˘˚ÎÔ‡˜ ÛÔÓ‰‡ÏÔ˘˜ ÌÈÎÚfiÙÂÚË ·fi ÙÔ˘˜ ·ÓÒÙÂÚÔ˘˜. °. µÚ·¯¤· Î·È Ï·ÙÈ¿ ÌÂٷοÚÈ· Î·È Ê¿Ï·ÁÁ˜. ¢. ∫ÔÓÙfi˜ Î·È Ï·Ù‡˜ ·˘¯¤Ó·˜ ÌËÚÈ·›Ô˘, ÎÔÓÙ¿ Î·È Ï·ÙÈ¿ Ï·ÁfiÓÈ·.
ˆÓ ÙÔ˘ FGFR3 ÛÙËÓ YX (2). ™ÙÔ ˘fiÏÔÈÔ 30% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÀÃ, ‰ÂÓ ¤¯ÂÈ ‚ÚÂı› Ë ÁÔÓȉȷ΋ ‰È·Ù·Ú·¯‹. ™Â ÔÏϤ˜ ÌÂϤÙ˜ ¤¯ÂÈ Á›ÓÂÈ ÚÔÛ¿ıÂÈ· Ê·ÈÓÔÙ˘È΋˜-ÁÔÓÔÙ˘È΋˜ Û˘Û¯¤ÙÈÛ˘ (2,4,9,10) (¶›Ó·Î·˜ 1). √È ·ÛıÂÓ›˜ Ì ÙËÓ ¡540∫ ÌÂÙ¿ÏÏ·ÍË Ù›ÓÔ˘Ó Ó· ¤¯Ô˘Ó Ôχ ÎÔÓÙfi ·Ó¿ÛÙËÌ· Î·È ÌÂÁ·Ï‡ÙÂÚË ‰˘Û·Ó·ÏÔÁ›· ÎÔÚÌÔ‡-¿ÎÚˆÓ Û ۯ¤ÛË Ì ¿ÏϘ ÌÂÙ·ÏÏ¿ÍÂȘ, ÂÓÒ Ë Ì·ÎÚÔÎÂÊ·Ï›· ÌÔÈ¿˙ÂÈ Ó· Â›Ó·È ÛÔ‚·ÚfiÙÂÚË Û ·ÛıÂÓ›˜ Ì ÙËÓ ∫650¡ Î·È ∫650Q ÌÂÙ¿ÏÏ·ÍË (4,9,10). ∆ÂÏÂ˘Ù·›· ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ÙÚÂȘ ÂÚÈÙÒÛÂȘ ÔÈÎÔÁÂÓÂÈÒÓ ÙˆÓ ÔÔ›ˆÓ Ù· ̤ÏË Â›¯·Ó ‡„Ô˜ ÂÓÙfi˜ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ, ·ÏÏ¿ Ì ̤ÙÚÈ· ‰˘Û·Ó·ÏÔÁ›· ÎÔÚÌÔ‡ Î·È ¿ÎÚˆÓ, Ì·ÎÚÔÎÂÊ·Ï›·, ÚÔ¤¯ÔÓ Ì¤ÙˆÔ, ηı›˙ËÛË Ù˘ Ú›˙·˜ Ù˘ ̇Ù˘, ¢ڇ ıÒڷη Î·È ÏfiÚ‰ˆÛË Ù˘ ÔÛÊ˘˚΋˜ ÌÔ›Ú·˜ Ù˘ ™™, ·ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· Û˘Ì‚·Ù¿ Ì ÀÃ, ÂÓÒ Î·È ÛÙȘ ÙÚÂȘ ÔÈÎÔÁ¤ÓÂȘ ·ÓȯÓ‡ÙËÎÂ Ë ÌÂÙ¿ÏÏ·ÍË ¡540S (2,8). ∂›Û˘, ‹È· Ê·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο Àà ·ÚÔ˘ÛÈ¿˙Ô˘Ó ¿ÙÔÌ· Ì ÙË ÌÂÙ¿ÏÏ·ÍË I538V (11), ÛÙ· ÔÔ›· ÙÔ ‡„Ô˜ Î·È Ë ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜ Â›Ó·È ÂÓÙfi˜ ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ, ÂÓÒ Ë ·Ó·ÏÔÁ›· ηıÈÛÙfi ‡„Ô˜/Û˘ÓÔÏÈÎfi ‡„Ô˜ Â›Ó·È ·ıÔÏÔÁÈ΋. ∫·Ù¿ Û˘Ó¤ÂÈ·, ¿ÙÔÌ· Ì ÙȘ ÌÂÙ·ÏÏ¿ÍÂȘ ¡540S Î·È I538V ÌÔÚ› Ó· ‰È·Ê‡ÁÔ˘Ó Ù˘ ‰È¿ÁÓˆÛ˘. ∂ÎÙfi˜ ·fi ÙȘ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙËÓ ∆∫1, ÚfiÛÊ·Ù· ‰ËÌÔÛȇıËΠÂÚ›ÙˆÛË Àà ¶·È‰È·ÙÚÈ΋ 2007;70:49-53
52
µ. ∞ÁÁÂÏ¿ÎÔ˘ Î·È Û˘Ó.
¶›Ó·Î·˜ 1. ™˘Û¯¤ÙÈÛË ÙˆÓ ‰È·ÊfiÚˆÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ÛÙÔÓ FGFR-3 Ì ÙÔ ‡„Ô˜ Î·È ÙËÓ ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜ Û ·ÛıÂÓ›˜ Ì ˘Ô¯ÔÓ‰ÚÔÏ·Û›· N328I N540K N540T N540S I538V K650N, K650Q
ª¤ÛÔ ‡„Ô˜
¶ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜
µÈ‚ÏÈÔÁÚ·Ê›·
-2,5 SD -3,25 ±1 SD -2,5 SD -1,5 SD -1,8 SD 1,87 SD ± 0,99 SD
+2 SD +1,72 ± 1,1 SD +2,5 SD +0,5 ¤ˆ˜ 3 SD +0,81 SD +2,48 ± 1,65 SD
Winterpacht et al, 2000 Matsui et al, 1998 Deutz-Terlow et al, 1998 Mortier et al, 2000 Grigelioniene et al, 1997 Bellus et al, 2000
Ì ÙË ÛËÌÂȷ΋ ÌÂÙ¿ÏÏ·ÍË ¡328π ÛÙÔ ÂÍˆÎ˘ÙÙ¿ÚÈÔ ·ÓÔÛÔÛÊ·ÈÚÈÓÈÎfi ÌfiÚÈÔ Ig III ÙÔ˘ FGFR3. ∏ ÌÂÙ¿ÏÏ·ÍË ·˘Ù‹ ¤¯ÂÈ ˆ˜ Û˘Ó¤ÂÈ· ÙË ‰È·Ù·Ú·¯‹ Ù˘ ÁÏ˘ÎÔ˙˘Ï›ˆÛ˘ Î·È Ê·›ÓÂÙ·È Ó· ·›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙË Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ˘Ô‰Ô¯¤· (12). ∆· Ê·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó Ù· ÎÏ·ÛÈο Ù˘ ÀÃ. ¶ÔÏÏÔ› ·ÛıÂÓ›˜ Ì Àà ÂÌÊ·Ó›˙Ô˘Ó „˘¯ÔÎÔÈÓˆÓÈο ÚÔ‚Ï‹Ì·Ù· ÏfiÁˆ Ù˘ ÌÂȈ̤Ó˘ ·˘ÙÔÂÎÙ›ÌËÛ‹˜ ÙÔ˘˜, ·ÏÏ¿ Î·È ÙÔ˘ ÎÔÈÓˆÓÈÎÔ‡ ·ÔÎÏÂÈÛÌÔ‡ Ô˘ Û˘¯Ó¿ Û˘Ì‚·›ÓÂÈ Û ¿ÙÔÌ· Ì ۈ̷ÙÈο ÂÏÏ›ÌÌ·Ù·. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi ¤¯ÂÈ ÂȯÂÈÚËı› Ë ·‡ÍËÛË ÙÔ˘ ‡„Ô˘˜ ÙˆÓ ·ÛıÂÓÒÓ Ì ÙË ¯ÔÚ‹ÁËÛË ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ Î·È ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÈÌ‹Î˘ÓÛË ÙˆÓ ÔÛÙÒÓ ÌÂÙ¿ ÙËÓ Û‡ÁÎÏÂÈÛË ÙˆÓ ÂÈʇÛÂˆÓ (1315). √È ÌÂϤÙ˜ fï˜ ¯ÔÚ‹ÁËÛ˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ Â›Ó·È ÂÚÈÔÚÈṲ̂Ó˜ Û ÌÈÎÚfi ·ÚÈıÌfi ·ÛıÂÓÒÓ Î·È ÁÈ· Û‡ÓÙÔÌÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ·Ú·ÎÔÏÔ‡ıËÛ˘. √È ÂÚÈÛÛfiÙÂÚÔÈ Û˘ÁÁÚ·Ê›˜ Û˘ÌʈÓÔ‡Ó fiÙÈ Ù· ·ÔÙÂϤÛÌ·Ù· Â›Ó·È Î·Ï‡ÙÂÚ· fiÙ·Ó Ë ·˘ÍËÙÈ΋ ÔÚÌfiÓË ¯ÔÚËÁËı› ÛÙËÓ ‹‚Ë, ÂÂȉ‹ ÔÈ ·ÛıÂÓ›˜ Ì Àà ‰ÂÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙËÓ Ù˘È΋ Ù·¯Â›· ·Ó¿Ù˘ÍË Ù˘ ‹‚˘ (pubertal growth spurt) Ô˘ ·Ú·ÙËÚÂ›Ù·È ÛÙ· Ê˘ÛÈÔÏÔÁÈο ·È‰È¿, Û˘ÓÂÒ˜ Ë ·˘ÍËÙÈ΋ ÔÚÌfiÓË Û ‰fiÛË ˘„ËÏfiÙÂÚË ·fi ÙË Û˘Ó‹ıË Ô˘ ‰›‰ÂÙ·È Û ·ÛıÂÓ›˜ Ì ·Ó¿ÚÎÂÈ· Ù˘ ˘fiÊ˘Û˘, ÌÔÚ› Ó· ‰Ú¿ÛÂÈ ·Ó·ÏËÚÒÓÔÓÙ·˜ ·˘Ùfi ÙÔ ¤ÏÏÂÈÌÌ· (3,13). (3). ∞Ó Î·È ÁÂÓÈο ‰ÂÓ ¤¯Ô˘Ó ·Ú·ÙËÚËı› ·ÓÂÈı‡ÌËÙ˜ ‰Ú¿ÛÂȘ ÛÙ· ÔÛÙ¿, fiˆ˜ ÂÈÙ¿¯˘ÓÛË Ù˘ ÔÛÙÈ΋˜ ËÏÈΛ·˜ ‹ Âȉ›ӈÛË Ù˘ ‰˘Û·Ó·ÏÔÁ›·˜ ÎÔÚÌÔ‡-¿ÎÚˆÓ (3,13), ˆÛÙfiÛÔ, Â›Ó·È ··Ú·›ÙËÙ˜ ÂÚ·ÈÙ¤Úˆ ÌÂϤÙ˜. ŸÛÔÓ ·ÊÔÚ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÈÌ‹Î˘ÓÛË ÙˆÓ ÔÛÙÒÓ, Ô˘ ·ÔÙÂÏ› ÌÈ· ÂÂÌ‚·ÙÈ΋, ÂÈΛӉ˘ÓË, ÂÚ›ÏÔÎË Î·È Ì·ÎÚÔ¯ÚfiÓÈ· ‰È·‰Èηۛ·, Ë ÂÈÏÔÁ‹ Ù˘ ı· Ú¤ÂÈ Ó· ·ÔÊ·Û›˙ÂÙ·È Ì ÌÂÁ¿ÏË ÂÚ›Û΄Ë. ™Â ÚfiÛÊ·ÙË ÌÂϤÙË 20 ·ÛıÂÓÒÓ Ì ÀÃ Ô˘ ˘Ô‚Ï‹ıËÎ·Ó Û ÂÈÌ‹Î˘ÓÛË ÔÛÙÒÓ, ÌÂÙ¿ ÙËÓ ÂÈÌ‹Î˘ÓÛË ÙÔ˘ ÌËÚÈ·›Ô˘ Î·È Ù˘ ÎÓ‹Ì˘, ÛÙÔ Û‡ÓÔÏfi ÙÔ˘˜ ÍÂ¤Ú·Û·Ó Ù· 150 cm Î·È ·Ú¿ ÙÔ fiÙÈ ÂÌÊ·Ó›ÛıËÎ·Ó ‰È¿ÊÔÚ˜ ÂÈÏÔΤ˜ ÛÙÔ 46% ÙˆÓ ·ÛıÂÓÒÓ, ‰ÂÓ ·Ú¤ÌÂÈÓ·Ó ÌfiÓÈ̘ ‚Ï¿‚˜ (15). ∂ÈÛËÌ·›ÓÂÙ·È ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ fiÙÈ Ë ıÂÙÈ΋ ›‰Ú·ÛË Ù˘ ·‡ÍËÛ˘ ÙÔ˘ ‡„Ô˘˜ ÛÙËÓ ÔÈfiPaediatriki 2007;70:49-53
ÙËÙ· ˙ˆ‹˜ ÙˆÓ ·ÛıÂÓÒÓ, Ô˘ ·ÊÔÚÔ‡Û ÙËÓ ·ÓÂÍ·ÚÙËÛ›· ÙÔ˘˜, ÙËÓ ·˘ÙÔÂÎÙ›ÌËÛ‹ ÙÔ˘˜ Î·È ÙË ‚ÂÏÙ›ˆÛË ÙˆÓ ÎÔÈÓˆÓÈÎÒÓ ÙÔ˘˜ Û¯¤ÛˆÓ, ͤڷÛ ηٿ Ôχ ÙÔ ÎfiÛÙÔ˜ Û ¯Ú‹Ì·Ù· Î·È ¯ÚfiÓÔ Ô˘ ··ÈÙ‹ıËΠ(15). ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Ù˘ Àà Ì ÙË ¯Ú‹ÛË ˘ÂÚ‹¯ˆÓ ÌÔÚ› ‡ÎÔÏ· Ó· ‰È·Ê‡ÁÂÈ Û ÂÚÈÙÒÛÂȘ ·Ô˘Û›·˜ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ ÈÛÙÔÚÈÎÔ‡. √È ·Ó·ÊÔÚ¤˜ ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Ù˘ Àà ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· Â›Ó·È Ï›Á˜ (5,16,17). ™ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ Ë ˘ÂÚ˯ÔÁÚ·ÊÈ΋ ‰È¿ÁÓˆÛË ¤ÁÈÓ ÛÙÔ ‰Â‡ÙÂÚÔ ÙÚ›ÌËÓÔ Ù˘ ·ËÛ˘, ÌÂٷ͇ 23˘-26˘ ‚‰ÔÌ¿‰·˜ ·ËÛ˘, Ì ÛÙ·ıÂÚfi ‡ÚËÌ· ÙÔ ÌÈÎÚfi Ì‹ÎÔ˜ ÙÔ˘ ÌËÚÈ·›Ô˘ ÔÛÙÔ‡ Û ۯ¤ÛË Ì ÙËÓ ·ÌÊÈ‚ÚÂÁÌ·ÙÈ΋ ‰È¿ÌÂÙÚÔ, ÙËÓ ÂÚ›ÌÂÙÚÔ Ù˘ ÎÔÈÏ›·˜ Î·È ÙÔ Ì‹ÎÔ˜ ÙÔ˘ ¿ÎÚÔ˘ Ô‰fi˜, ÂÓÒ ·Ô˘Û›·˙·Ó ¿ÏÏ· ÛÔ‚·Ú¿ ·ıÔÏÔÁÈο ˘ÂÚ˯ÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· (5,16). ™ÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜ Ô ÚÔÁÂÓÓËÙÈÎfi˜ ÁÔÓȉȷÎfi˜ ¤ÏÂÁ¯Ô˜ ÌÔÚ› Ó· Û˘Ì‚¿ÏÂÈ ÛÙË ‰È¿ÁÓˆÛË. ŸÌˆ˜, ÏfiÁˆ ÙˆÓ Èı·ÓÒÓ ÎÈÓ‰‡ÓˆÓ Ù˘ ·ÌÓÈԷڷΤÓÙËÛ˘ Î·È ÂÂȉ‹ Ë Àà ‰ÂÓ Â›Ó·È ı·Ó·ÙËÊfiÚÔ˜ ·Ûı¤ÓÂÈ·, ÚÔÙ›ÓÂÙ·È Ë ·ÌÓÈԷڷΤÓÙËÛË Ó· Á›ÓÂÙ·È ÌfiÓÔ fiÙ·Ó Ë ÀÃ Â›Ó·È ‰È·ÁÓˆṲ̂ÓË ÛÙÔÓ ¤Ó· ‹ Î·È ÛÙÔ˘˜ ‰‡Ô ÁÔÓ›˜ (16). ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ÀÃ Â›Ó·È ÌÈ· ÓfiÛÔ˜ Ô˘ ÌÔÚ› Ó· ‰È·Ê‡ÁÂÈ Ù˘ ‰È¿ÁÓˆÛ˘, ·Ó ‰ÂÓ ˘¿Ú¯ÂÈ ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi. °È· ÙËÓ ¤ÁηÈÚË ‰È¿ÁÓˆÛË ··ÈÙÂ›Ù·È ÏÂÙÔÌÂÚ‹˜ ÎÏÈÓÈ΋ ÂͤٷÛË Î·È ÁÓÒÛË ÙˆÓ ÎÏÈÓÈÎÒÓ Î·È ·ÎÙÈÓÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ Ô˘ Û˘ÓÔ‰Â‡Ô˘Ó ÙË ÓÔÛÔÏÔÁÈ΋ ·˘Ù‹ ÔÓÙfiÙËÙ·, ÚÔ¸Ôı¤ÛÂȘ Ô˘ ··ÈÙÔ‡ÓÙ·È ÁÈ· ÙËÓ ·Ó·˙‹ÙËÛË Ù˘ Û˘ÁÎÂÎÚÈ̤Ó˘ ÁÔÓȉȷ΋˜ ‰È·Ù·Ú·¯‹˜. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Â›Ó·È ‰˘Ó·Ù‹ Ì ÙË ¯Ú‹ÛË ˘ÂÚ‹¯ˆÓ Î·È ÁÔÓȉȷÎfi ¤ÏÂÁ¯Ô. ™Â ÂÚÈÙÒÛÂȘ Ì ÛÔ‚·Ú‹ ‚Ú·¯˘ÛˆÌ›· Â›Ó·È ‰˘Ó·Ù‹ ÙfiÛÔ Ë ¯Ú‹ÛË ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ ÛÙËÓ ÂÊ˂›·, fiÛÔ Î·È Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÈÌ‹Î˘ÓÛË ÙˆÓ ÔÛÙÒÓ ÌÂÙ¿ ÙËÓ Û‡ÁÎÏÂÈÛË ÙˆÓ ÂÈʇÛˆÓ.
∂˘¯·ÚÈÛٛ˜ ∂˘¯·ÚÈÛÙԇ̠ÙËÓ ÔÌ¿‰· ÂȉÈÎÒÓ ÙÔ˘ ∂˘Úˆ·˚ÎÔ‡ ¢ÈÎÙ‡Ô˘ ™ÎÂÏÂÙÈÎÒÓ ¢˘ÛÏ·ÛÈÒÓ Ô˘ Ì·˜ ‚Ô‹ıËÛ ÛÙËÓ ÎÏÈÓÈ΋ Î·È ÁÂÓÂÙÈ΋ ‰È¿ÁÓˆÛË. ∂›Û˘, ¢¯·ÚÈÛÙԇ̠ÙÔ ∂Ó‰ÔÎÚÈÓÔÏÔÁÈÎfi ∆Ì‹Ì· Î·È ÙÔ ∞ÎÙÈÓÔÏÔÁÈÎfi ∂ÚÁ·ÛÙ‹ÚÈÔ ÙÔ˘ µÂÓÈ˙¤ÏÂÈÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ÁÈ· ÙË Û˘Ì‚ÔÏ‹ ÙÔ˘˜ ÛÙË ‰È¿ÁÓˆÛË.
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ÀÔ¯ÔÓ‰ÚÔÏ·Û›· ÌÂ ÌÂÙ¿ÏÏ·ÍË ¡540∫ ÛÙÔÓ FGFR3
µÈ‚ÏÈÔÁÚ·Ê›· 1. International Working Group on Constitutional Diseases of Bone. International nomenclature and classification of the osteochondrodysplasias (1997). Am J Med Genet 1998;79:376-382. 2. Thauvin-Robinet C, Faivre L, Lewin P, De Monleon JV, Francois C, Huet F, et al. Hypochondroplasia and stature within normal limits: another family with an Asn540Ser mutation in the fibroblast growth factor receptor 3 gene. Am J Med Genet A 2003;119:81-84. 3. Kanaka-Gantenbein C. Present status of the use of growth hormone in short children with bone diseases (diseases of the skeleton). J Pediatr Endocrinol Metab 2001;14:17-26. 4. Prinster C, Del Maschio M, Beluffi G, Maghnie M, Weber G, Del Maschio A, et al. Diagnosis of hypochondroplasia: the role of radiological interpretation. Italian Study Group of Hypochondroplasia. Pediatr Radiol 2001;31:203-208. 5. Kataoka S, Sawai H, Yamada H, Kanazawa N, Koyama K, Nishimura G, et al. Radiographic and genetic diagnosis of sporadic hypochondroplasia early in the neonatal period. Prenat Diagn 2004;24:45-49. 6. Hecht JT, Herrera CA, Greenhaw GA, Francomano CA, Bellus GA, Blanton SH. Confirmatory linkage of hypochondroplasia to chromosome arm 4p. Am J Med Genet 1995;57:505-506. 7. Bonaventure J, Rousseau F, Legeai-Mallet L, Le Merrer M, Munnich A, Maroteaux P. Common mutations in the fibroblast growth factor receptor 3 (FGFR 3) gene account for achondroplasia, hypochondroplasia and thanatophoric dwarfism. Am J Med Genet 1996;63:148-154. 8. Riepe FG, Krone N, Sippell WG. Disproportionate stature but normal height in hypochondroplasia. Eur J Pediatr 2005;164:397-399.
9. Prinster C, Carrera P, Del Maschio M, Weber G, Maghnie M, Vigone MC, et al. Comparison of clinical-radiological and molecular findings in hypochondroplasia. Am J Med Genet 1998;75:109-112. 10. Ramaswami U, Rumsby G, Hindmarsh PC, Brook CG. Genotype and phenotype in hypochondroplasia. J Pediatr 1998;133:99-102. 11. Grigelioniene G, Hagenas L, Eklof O, Neumeyer L, Haereid PE, Anvret M. A novel missense mutation Ile538Val in the fibroblast growth factor receptor 3 in hypochondroplasia. Mutations in brief no. 122. Hum Mutat 1998;11:333. 12. Winterpacht A, Hilbert K, Stelzer C, Schweikardt T, Decker H, Segerer H, et al. A novel mutation in FGFR 3 disrupts a putative N-glycosylation site and results in hypochondroplasia. Physiol Genomics 2000;2:9-12. 13. Tanaka N, Katsumata N, Horikawa R, Tanaka T. The comparison of the effects of short-term growth hormone treatment in patients with achondroplasia and with hypochondroplasia. Endocr J 2003;50:69-75. 14. Kanazawa H, Tanaka H, Inoue M, Yamanaka Y, Namba N, Seino Y. Efficacy of growth hormone therapy for patients with skeletal dysplasia. J Bone Miner Metab 2003;21: 307-310. 15. Aldegheri R, Dall’Oca C. Limb lengthening in short stature patients. J Pediatr Orthop B 2001;10:238-247. 16. Huggins MJ, Mernagh JR, Steele L, Smith JR, Nowaczyk MJ. Prenatal sonographic diagnosis of hypochondroplasia in a high-risk fetus. Am J Med Genet 1999;87:226-229. 17. Karadimas C, Sifakis S, Valsamopoulos P, Makatsoris C, Velissariou V, Petersen MB, et al. Prenatal diagnosis of hypochondroplasia: report of two cases. Am J Med Genet A 2006;140:998-1003.
¶·È‰È·ÙÚÈ΋ 2007;70:49-53
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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏
CASE REPORT
™ÈÙÈÔÁÂÓ‹˜ ·Ó¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ µ12 Û ‚Ú¤ÊÔ˜. ∆· ÛÈÙÈÛÙÈο Ï¿ıË ÙˆÓ ¯ÔÚÙÔÊ¿ÁˆÓ ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, µÂÓÈ˙¤ÏÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ, ∫Ú‹ÙË AÏÏËÏÔÁÚ·Ê›·: ∞ÁÁÂÏ¿ÎÔ˘ µ·ÛÈÏÈ΋ vaagge3@yahoo.gr °Ô‡ÚÓ˜ ∆Â̤ÓÔ˘˜ ∆.∫. 715 00, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘
µ. ∞ÁÁÂÏ¿ÎÔ˘, ª. OÚÊ·ÓÔ‡, °. ¡ÈˆÙ¿Î˘, ∂. ∫fiÎÔÚË, π. ÷ڈӛÙË ¶ÂÚ›ÏË„Ë: ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ·Ó¿ÚÎÂÈ·˜ ‚ÈÙ·Ì›Ó˘ µ12 Û ı‹Ï˘ ‚Ú¤ÊÔ˜ ËÏÈΛ·˜ 12 ÌËÓÒÓ, Ô˘ ÁÂÓÓ‹ıËÎÂ Î·È ı‹Ï·Û ·fi ÌËÙ¤Ú· ·ÔÎÏÂÈÛÙÈο ¯ÔÚÙÔÊ¿ÁÔ. ∆· Û˘ÌÙÒÌ·Ù· ηٿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘ ‹Ù·Ó ·ÓÔÚÂÍ›·, ·Ó·Ú΋˜ ÚfiÛÏË„Ë ‚¿ÚÔ˘˜, ¤ÌÂÙÔÈ, ·¿ıÂÈ· Î·È Ì˘˚΋ ˘ÔÙÔÓ›·. ∆Ô ‚Ú¤ÊÔ˜ ı‹Ï·˙ ·ÔÎÏÂÈÛÙÈο ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 8,5 ÌËÓÒÓ, fiÙ·Ó ¿Ú¯ÈÛ·Ó Ó· ÚÔÛÙ›ıÂÓÙ·È ÛÙË ‰È·ÙÚÔÊ‹ ÙÔ˘ ÌÈÎÚ¤˜ ÔÛfiÙËÙ˜ ·fi Á¿Ï· ÛfiÁÈ·˜, ÊÚÔ‡Ù· Î·È Ï·¯·ÓÈο. ∏ ·ÓÔÚÂÍ›· ‹Ù·Ó ÙÔ ÚÒÙÔ Û‡Ìو̷ Ô˘ ÂÌÊ·Ó›ÛÙËΠ۠ËÏÈΛ· 6 ÌËÓÒÓ. ™ÙËÓ ËÏÈΛ· ÙˆÓ 9 ÌËÓÒÓ ÚÔÛÙ¤ıËÎ·Ó ¤ÌÂÙÔÈ Î·È ·ÒÏÂÈ· ‚¿ÚÔ˘˜. ∏ „˘¯ÔÎÈÓËÙÈ΋ ÂͤÏÈÍË ÙÔ˘ ‚Ú¤ÊÔ˘˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋ ηٿ ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ Ù˘ ˙ˆ‹˜. ™ÙËÓ ËÏÈΛ· ÙˆÓ 10 ÌËÓÒÓ ÂÌÊ·Ó›ÛÙËÎ·Ó ÛËÌ›· „˘¯ÔÎÈÓËÙÈ΋˜ ηı˘ÛÙ¤ÚËÛ˘ Î·È Ë ÌËÙ¤Ú· ÂÚÈÁÚ¿ÊÂÈ ÙÔ ‚Ú¤ÊÔ˜ Û·Ó ÏÈÁfiÙÂÚÔ ˙ˆËÚfi Î·È Ì ÛÙ·‰È·Î¿ ·˘Í·ÓfiÌÂÓË ·¿ıÂÈ·. ∆· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ¤‰ÂÈÍ·Ó ÛÔ‚·Ú‹ ÌÂÁ·ÏÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›·. ∆· ›‰· ‚ÈÙ·Ì›Ó˘ µ12 ÛÙÔÓ ÔÚfi ÙÔ˘ ‚Ú¤ÊÔ˘˜ Î·È Ù˘ ÌËÙ¤Ú·˜ ‹Ù·Ó ¯·ÌËÏ¿. ∆Ô ‚Ú¤ÊÔ˜ ·ÓÙ·ÔÎÚ›ıËΠηϿ ÛÙË ¯ÔÚ‹ÁËÛË ‚ÈÙ·Ì›Ó˘ µ12 fiÛÔÓ ·ÊÔÚ¿ ÛÙ· ÁÂÓÈο Û˘ÌÙÒÌ·Ù·, ÙȘ ·ÈÌ·ÙÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÙËÓ ˘ÔÙÔÓ›· Î·È ÙËÓ „˘¯ÔÎÈÓËÙÈ΋ ÂͤÏÈÍË. §¤ÍÂȘ ÎÏÂȉȿ: ÃÔÚÙÔÊ·Á›·, ·ÔÎÏÂÈÛÙÈο ¯ÔÚÙÔÊ¿ÁÔÈ, ·Ó¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ µ12, ‰È·ÙÚÔÊ‹, ‚Ú¤ÊË.
1st Paediatric Clinic, Venizelion General Hospital of Heraklion, Crete Correspondence: V. Aggelakou vaagge3@yahoo.gr Gournes Temenous 715 00, Heraklion, Crete
Nutritional vitamin B12 deficiency in an infant. Dietary errors of a vegan V. Aggelakou, M. Orfanou, G. Niotakis, H. Kokori, I. Charoniti Abstract: The case is reported of vitamin B12 deficiency in a twelve month-old female infant born to and breastfed by a strictly vegan mother. The infant presented with anorexia, failure to thrive, vomiting, apathy and muscular hypotonia. She had been exclusively breast fed up to the age of 8.5 months, when small amounts of soya milk, vegetables and fruits were added to her diet. The first symptom was reduced appetite, which presented at the age of 6 months, followed by vomiting and weight loss at the age of 9 months. The psychomotor development was normal during the first months of life, but around 10 months of age signs of psychomotor delay were noted and the mother described the baby as being less active and increasingly apathetic. Laboratory tests revealed severe megaloblastic anaemia. Low levels of serum vitamin B12 were found in both the infant and her mother. The infant responded well to vitamin B12 administration, with improvement in the general symptoms and in the haematologic manifestations, muscular hypotonia and psychomotor development. Key words: Vegetarianism, vegan, vitamin B12 deficiency, infant, nutrition.
∂ÈÛ·ÁˆÁ‹ ∆· ·ÚÈ· ·›ÙÈ· ·Ó¿ÚÎÂÈ·˜ ‚ÈÙ·Ì›Ó˘ µ12 (∞µµ12) ÛÙ· ·È‰È¿ ‰È·ÈÚÔ‡ÓÙ·È Û ÙÚÂȘ ηÙËÁÔڛ˜: ÌÂȈ̤ÓË ÚfiÛÏË„Ë, ·ÓÒÌ·ÏË ·ÔÚÚfiÊËÛË Î·È ÂÓ‰ÔÁÂÓ›˜ ‰È·Ù·Ú·¯¤˜ Ù˘ ÌÂÙ·ÊÔÚ¿˜ Î·È ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ Ù˘ ‚ÈÙ·Ì›Ó˘ µ12 (µµ12 ) (¶›Ó·Î·˜ 1, ∂ÈÎfiÓ· 1) (1). ∞Ó Î·È Ë ∞µµ12 ÛÙ· ·È‰È¿ ÛÙȘ ·ÓÂÙ˘Á̤Ó˜ ¯ÒÚ˜ ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È Û ÂȉÈΤ˜ ‰›·ÈÙ˜ (.¯. ·ÛıÂÓ›˜ Ì ʷÈÓ˘ÏÎÂÙÔÓÔ˘Ú›· ‹ ÁÏ˘ÎÔÁÔÓ›·ÛË Ib) ‹ Û ÂËÚ·Ṳ̂ÓË ·ÔÚÚfiÊËÛË (.¯. ÎÔÈÏÈÔοÎË ‹ ¯ÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ ÛÙÔ ÛÙÔÌ¿¯È ‹ ÙÔ ¤ÓÙÂÚÔ), ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒPaediatriki 2007;70:54-59
ÛÂȘ ∞µµ12 Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ·ÊÔÚÔ‡Ó Û ıËÏ¿˙ÔÓÙ· ‚Ú¤ÊË ÌËÙ¤ÚˆÓ Ì ∞µµ12 (2). ∏ ∞µµ12 ÙˆÓ ıËÏ·˙Ô˘ÛÒÓ ÌËÙ¤ÚˆÓ Û˘Ó‹ıˆ˜ ÔÊ›ÏÂÙ·È Û ·˘ÛÙËÚ‹ ¯ÔÚÙÔÊ·Á›· (veganism), Ë ÔÔ›· ·ÔÎÏ›ÂÈ Ï‹Úˆ˜ Ù· ÙÚfiÊÈÌ· ˙ˆÈ΋˜ ÚÔ¤Ï¢Û˘ (ÎÚ¤·˜, „¿ÚÈ, ·˘Á¿, Á¿Ï· Î·È ¿ÏÏ· ˙ˆÈο ÚÔ˚fiÓÙ·) ·fi ÙË ‰È·ÙÚÔÊ‹ (2). ∏ ¯ÔÚÙÔÊ·Á›· (vegetarianism) Â›Ó·È ÛÈÙÈÛÙÈ΋ Ú·ÎÙÈ΋ Û ÔÏϤ˜ ÂÚÈÔ¯¤˜ ÙÔ˘ Ï·Ó‹ÙË, fiˆ˜ Ë πÓ‰›·, ÙÔ ªÂÍÈÎfi, Ë ∫ÂÓÙÚÈ΋ Î·È ¡fiÙÈ· ∞ÌÂÚÈ΋ Î·È ÔÚÈṲ̂Ó˜ ÂÚÈÔ¯¤˜ Ù˘ ∞ÊÚÈ΋˜ (3). √ ÊÈÏfiÛÔÊÔ˜ ¶˘ı·ÁfiÚ·˜ ‹Ù·Ó ·fi ÙÔ˘˜ ÚÒÙÔ˘˜ ˘ÔÛÙËÚÈÎÙ¤˜ Ù˘
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™ÈÙÈÔÁÂÓ‹˜ ·Ó¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ µ12 Û ‚Ú¤ÊÔ˜
¶›Ó·Î·˜ 1. ∞›ÙÈ· ·Ó¿ÚÎÂÈ·˜ ‚ÈÙ·Ì›Ó˘ µ12 ÛÙ· ·È‰È¿ ªÂȈ̤ÓË ÚfiÛÏË„Ë ñ ÃÔÚÙÔÊ·ÁÈ΋ ‰›·ÈÙ· ñ £ËÏ¿˙ÔÓÙ· ‚Ú¤ÊË ·fi ÌËÙ¤Ú˜ Ì ·Ó¿ÚÎÂÈ· µµ12 ÏfiÁˆ ηÎÔ‹ıÔ˘˜ ·Ó·ÈÌ›·˜, ¯ÔÚÙÔÊ·ÁÈ΋˜ ‰›·ÈÙ·˜, ÂÁ¯Â›ÚËÛ˘ ·Ú¿Î·Ì„˘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘, Ï‹„˘ ·Ó·ÛÙÔϤˆÓ Ù˘ ·ÓÙÏ›·˜ ÚˆÙÔÓ›ˆÓ ñ ÕÏϘ ‰›·ÈÙ˜, .¯. ÌË ÛˆÛÙ‹ ‰›·ÈÙ· Û ʷÈÓ˘ÏÎÂÙÔÓÔ˘Ú›· ∞ÓÒÌ·ÏË ·ÔÚÚfiÊËÛË ñ ∞Ô˘Û›·/‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÂÓ‰ÔÁÂÓ‹ ·Ú¿ÁÔÓÙ· Ô˘ ·Ú¿ÁÂÙ·È ·fi Ù· ÙÔȯˆÌ·ÙÈο ·ÙÙ·Ú· ÙÔ˘ ÛÙÔÌ¿¯Ô˘, ÏfiÁˆ: - ∞Ê·›ÚÂÛ˘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ - ∞˘ÙÔ¿ÓÔÛ˘ ηÎÔ‹ıÔ˘˜ ·Ó·ÈÌ›·˜ ñ ªÂȈ̤ÓË ÔÛfiÙËÙ· Á·ÛÙÚÈÎÔ‡ ÔͤԘ - ¶·Ú·ÙÂٷ̤ÓË ıÂڷ›· Ì ·Ó·ÛÙÔÏ›˜ Ù˘ ·ÓÙÏ›·˜ ÚˆÙÔÓ›ˆÓ ñ ¶·ÁÎÚ·ÙÈ΋ ·Ó¿ÚÎÂÈ· ñ ∞ÓÙ·ÁˆÓÈÛÌfi˜ ÁÈ· µµ12 ÛÙÔ ¤ÓÙÂÚÔ - ¶·Ú·ÛÈÙÈΤ˜ ÏÔÈÌÒÍÂȘ - ÀÂÚ·Ó¿Ù˘ÍË ‚·ÎÙËÚȉ›ˆÓ ñ ¢È·Ù·Ú·¯‹ Ù˘ ·ÔÚÚfiÊËÛ˘ ηٿ Ì‹ÎÔ˜ ÙÔ˘ ÂÈÏÂÔ‡ - ¡fiÛÔ˜ Crohn - ∫ÔÈÏÈÔοÎË - ÃÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ ÛÙÔÓ ÂÈÏÂfi - ∞ӈ̷ϛ· ÙÔ˘ ˘Ô‰Ô¯¤· ÛÙÔÓ ÂÈÏÂfi (ÓfiÛÔ˜ Imerslund-Grasbeck) ∂Ó‰ÔÁÂÓ›˜ ‰È·Ù·Ú·¯¤˜ Ù˘ ÌÂÙ·ÊÔÚ¿˜ Î·È ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ Ù˘ µµ12 ñ ¢È·Ù·Ú·¯‹ ÛÙË ÌÂÙ·ÊÔÚ¿ - ∞Ó¿ÚÎÂÈ· ÙÚ·ÓÛÎÔ‚·Ï·Ì›Ó˘ ππ - ∞Ó¿ÚÎÂÈ· ÙˆÓ R-binder ÚˆÙÂ˚ÓÒÓ ñ ¢È·Ù·Ú·¯‹ ÌÂÙ·‚ÔÏÈÛÌÔ‡ - ∞Ó¿ÚÎÂÈ· ·‰ÂÓÔÛ˘ÏÎÔ‚·Ï·Ì›Ó˘: ÓfiÛÔ˜ cblA Î·È cblµ - ∞Ó¿ÚÎÂÈ· ÌÂı˘ÏÎÔ‚·Ï·Ì›Ó˘: ÓfiÛÔ˜ cblE Î·È cblG - ™˘Ó‰˘·Ṳ̂ÓË ·Ó¿ÚÎÂÈ· ·‰ÂÓÔÛ˘ÏÎÔ‚·Ï·Ì›Ó˘ Î·È ÌÂı˘ÏÎÔ‚·Ï·Ì›Ó˘: cblC, cblD Î·È cblF
¯ÔÚÙÔÊ·Á›·˜ ÛÙË ¢‡ÛË (4). ™ÙȘ ‰ÂηÂٛ˜ ÙÔ˘ 1960 Î·È 1970 ¿Ú¯ÈÛ ӷ ·˘Í¿ÓÂÙ·È ÙÔ ÔÛÔÛÙfi ÙˆÓ ¯ÔÚÙÔÊ¿ÁˆÓ ÛÙËÓ ∂˘ÚÒË Î·È ÙȘ ∏¶∞ ÏfiÁˆ ıÚËÛ΢ÙÈÎÒÓ, ÊÈÏÔÛÔÊÈÎÒÓ Î·È ÔÈÎÔÏÔÁÈÎÒÓ ÂÔÈı‹ÛˆÓ, ·ÏÏ¿ Î·È ÁÈ· ÏfiÁÔ˘˜ ‚ÂÏÙ›ˆÛ˘ Ù˘ ˘Á›·˜ (4). ¶·Ú¿ÏÏËÏ·, Ê·›ÓÂÙ·È Ó· ·˘Í¿ÓÂÈ Î·È ÙÔ ÔÛÔÛÙfi ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ô˘ ·ÎÔÏÔ˘ıÔ‡Ó ¯ÔÚÙÔÊ·ÁÈ΋ ‰È·ÙÚÔÊ‹, Û˘Ó‹ıˆ˜ ÏfiÁˆ ¯ÔÚÙÔÊ·Á›·˜ ÙˆÓ ÁÔÓ¤ˆÓ (5). ∏ ÌË ÈÛÔÚÚÔË̤ÓË ¯ÔÚÙÔÊ·ÁÈ΋ ‰È·ÙÚÔÊ‹ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ÌÂȈ̤ÓË ÚfiÛÏË„Ë ÂÓ¤ÚÁÂÈ·˜, ÚˆÙÂ˚ÓÒÓ, ‚ÈÙ·ÌÈÓÒÓ (΢ڛˆ˜ µ12 Î·È D) Î·È È¯ÓÔÛÙÔȯ›ˆÓ fiˆ˜ Fe, Ca Î·È Zn (4). ∏ ∞µµ12 Â›Ó·È Û˘¯Ó‹ ÛÙÔ˘˜ ·˘ÛÙËÚ¿ ¯ÔÚÙÔÊ¿ÁÔ˘˜ (vegan) (3). ∏ ‰È¿ÁÓˆÛ‹ Ù˘ ÌÔÚ› Ó· ‰È·Ê‡ÁÂÈ ‹ Ó· ηı˘ÛÙÂÚ‹ÛÂÈ ÂÂȉ‹ Ù· Û˘ÌÙÒÌ·Ù¿ Ù˘ ‰ÂÓ Â›Ó·È ÂȉÈο. √È Û˘ÓËı¤ÛÙÂÚ˜ ÂΉËÏÒÛÂȘ Ù˘ ∞µµ12 ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Á·ÛÙÚÂÓÙÂÚÈΤ˜ ‰È·Ù·Ú·¯¤˜ Î·È Ó¢ÚÔÏÔÁÈο
™ÙfiÌ· ∂χıÂÚË µ12 ÌÔÚ› Ó· ·ÔÚÚÔÊËı› ˘ÔÁÏÒÛÛÈ·
◊·Ú ∏ µ12 ·ÔıË·ÂÙ·È Î·È ÂÎÎÚ›ÓÂÙ·È ÛÙÔ ¤ÓÙÂÚÔ Ì ÙË ¯ÔÏ‹ ·fi fiÔ˘ Î·È Â·Ó·ÚÚÔÊ¿Ù·È
∞›Ì· ∏ ÙÚ·ÓÛÎÔ‚·Ï·Ì›ÓË ππ ÌÂٷʤÚÂÈ ÙË µ12 ÛÙÔ˘˜ ÈÛÙÔ‡˜ fiÔ˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È Î·È ÛÙÔ ‹·Ú fiÔ˘ ·ÔıË·ÂÙ·È ÛÙËÓ ÙÚ·ÓÛÎÔ‚·Ï·Ì›ÓË πππ
™ÙÔÌ¿¯È ñ ŒÓ˙˘Ì· Î·È Ôͤ· ·ÔÛ˘Ó‰¤Ô˘Ó ÙË µ12 ·fi ÙȘ ÚˆÙ½Ó˜ ñ R-ÚˆÙ½Ó˜ ‰ÂÛÌÂ‡Ô˘Ó ÙË µ12 ñ ŒÎÎÚÈÛË ÙÔ˘ ÂÓ‰ÔÁÂÓ‹ ·Ú¿ÁÔÓÙ· (IF)
∞ÓÒÙÂÚÔ ÏÂÙfi ¤ÓÙÂÚÔ ñ R-ÚˆÙ½Ó˜ ·Ô‰ÂÛÌÂ‡Ô˘Ó ÙË µ12 ñ IF ‰ÂÛ̇ÂÈ ÙË µ12
∫·ÙÒÙÂÚÔ ÏÂÙfi ¤ÓÙÂÚÔ ∆Ô Û‡ÌÏÂÁÌ· IF- µ12 Û˘Ó‰¤ÂÙ·È ÛÙÔÓ IF- µ12 ˘Ô‰Ô¯¤· ÛÙ· ÂÓÙÂÚÈο ·ÙÙ·Ú·
∂ÓÙÂÚÈο ·ÙÙ·Ú· ∏ µ12 Û˘Ó‰¤ÂÙ·È ÛÙËÓ ÙÚ·ÓÛÎÔ‚·Ï·Ì›ÓË ππ
∂ÈÎfiÓ· 1. ∞ÔÚÚfiÊËÛË Î·È ÌÂÙ·‚ÔÏÈÛÌfi˜ Ù˘ ‚ÈÙ·Ì›Ó˘ µ12.
Û˘ÌÙÒÌ·Ù·, Ù· ÔÔ›· ÌÔÚ› Ó· Â›Ó·È ·ÚÔ‰Èο ‹ ÌfiÓÈÌ· (6-9).
¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ £‹Ï˘ ‚Ú¤ÊÔ˜, ËÏÈΛ·˜ 12 ÌËÓÒÓ, ÚÔÛÎÔÌ›ÛÙËΠÛÙËÓ ÎÏÈÓÈ΋ ÏfiÁˆ ·ÓÔÚÂÍ›·˜, ÓˆıÚfiÙËÙ·˜, Â̤وÓ, ˆ¯ÚÔΛÙÚÈÓ˘ ¯ÚÔÈ¿˜ ‰¤ÚÌ·ÙÔ˜ Î·È ·ÒÏÂÈ·˜ ‚¿ÚÔ˘˜. √È ÁÔÓ›˜ ÙÔ˘ Â›Ó·È ·˘ÛÙËÚÒ˜ ¯ÔÚÙÔÊ¿ÁÔÈ ·fi 20ÂÙ›·˜ ÏfiÁˆ ıÚËÛ΢ÙÈÎÒÓ ÂÔÈı‹ÛˆÓ. ∆Ô ‚Ú¤ÊÔ˜ Â›Ó·È ÙÔ ÚÒÙÔ ·È‰› Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. °ÂÓÓ‹ıËΠÌÂÙ¿ ·fi Ê˘ÛÈÔÏÔÁÈ΋ ÙÂÏÂÈfiÌËÓË Î‡ËÛË, ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ÙÔÎÂÙfi Î·È Ì ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ 3820 g. ∆Ô ‚Ú¤ÊÔ˜ ÙÚÂÊfiÙ·Ó ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi ıËÏ·ÛÌfi ¤ˆ˜ ÙËÓ ËÏÈΛ· ÙˆÓ 8,5 ÌËÓÒÓ, ÂÓÒ ÛÙË Û˘Ó¤¯ÂÈ· ÚÔÛÙ¤ıËÎ·Ó ÛÙË ‰È·ÙÚÔÊ‹ ÙÔ˘ Á¿Ï· ÛfiÁÈ·˜, Ê˘ÙÈΤ˜ ÙÚÔʤ˜ Î·È ÊÚÔ‡Ù·, ·fi Ù· ÔÔ›· ÂÏ¿Ì‚·Ó ÂÏ¿¯ÈÛÙ˜ ÔÛfiÙËÙ˜. ∆Ô ‚Ú¤ÊÔ˜ ›¯Â Ê˘ÛÈÔÏÔÁÈ΋ ۈ̷ÙÈ΋ ·Ó¿Ù˘ÍË Ì¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 6 ÌËÓÒÓ. ∞fi ·˘Ù‹ ÙËÓ ËÏÈΛ· ·ÚÔ˘Û›·Û ÌÂȈ̤ÓË fiÚÂÍË, ÂÓÒ ·fi ÙÔÓ ¤Ó·ÙÔ Ì‹Ó· ηıËÌÂÚÈÓÔ‡˜ Â̤ÙÔ˘˜ (12 ËÌÂÚËÛ›ˆ˜) Î·È ·ÒÏÂÈ· ‚¿ÚÔ˘˜. ™‡Ìʈӷ Ì ÙȘ ÏËÚÔÊÔڛ˜ ÙˆÓ ÁÔÓ¤ˆÓ, ηٿ ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ Ù˘ ˙ˆ‹˜ ÙÔ ‚Ú¤ÊÔ˜ ›¯Â ÔÌ·Ï‹ „˘¯ÔÎÈÓËÙÈ΋ ÂͤÏÈÍË. ™Â ËÏÈΛ· 10 ÌËÓÒÓ, ÙÔ ‚Ú¤ÊÔ˜ ÌÔÚÔ‡Û ӷ οıÂÙ·È ÛÙ·ıÂÚ¿ Î·È Ó· ÛÙ¤ÎÂÙ·È fiÚıÈÔ ÎÚ·ÙÒÓÙ·˜ ¤ÈÏ·, Ó· È¿ÓÂÈ ·ÓÙÈΛÌÂÓÔ Ì ‰Â›ÎÙË-·ÓÙ›¯ÂÈÚ·, Ó· Û˘Ó‰˘¿˙ÂÈ ‰‡Ô Û˘ÏÏ·‚¤˜, ÂÓÒ Ë ·ÓÙ›ÏË„Ë Î·È Ë ¶·È‰È·ÙÚÈ΋ 2007;70:54-59
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µ. ∞ÁÁÂÏ¿ÎÔ˘ Î·È Û˘Ó.
ÎÔÈÓˆÓÈÎfiÙËÙ· ‹Ù·Ó Û˘Ì‚·Ù¤˜ Ì ÙËÓ ËÏÈΛ· ÙÔ˘. ∞fi ÙËÓ ËÏÈΛ· ÙˆÓ 10 ÌËÓÒÓ, Ë Î·Ù¿ÛÙ·ÛË ÙÔ˘ ‚Ú¤ÊÔ˘˜ ·ÚÔ˘Û›·Û ÛÙ·‰È·Î‹ Âȉ›ӈÛË Ì ӈıÚfiÙËÙ·, ÌÂȈ̤ÓË ÎÈÓËÙÈÎfiÙËÙ·, ·‰˘Ó·Ì›· Ó· ÛÙ¤ÎÂÙ·È ÁÈ· ·ÚÎÂÙ‹ ÒÚ· fiÚıÈÔ, ÂÓÒ ·Ú¿ÏÏËÏ· ÙÔ ·È‰› ÛÙ·Ì¿ÙËÛ ӷ ¯·ÌÔÁÂÏ¿ Î·È ·ÚÔ˘Û›·Û ¤ÓÙÔÓË ˆ¯ÚÔΛÙÚÈÓË ¯ÚÔÈ¿ ‰¤ÚÌ·ÙÔ˜, ÛÙÔÌ·Ù›Ùȉ· Î·È ‰˘ÛÎÔÏ›· ÛÙË Û›ÙÈÛË. ∫·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ÙÔ ‚Ú¤ÊÔ˜ ‹Ù·Ó Û ÂËÚ·Ṳ̂ÓË ÁÂÓÈ΋ ηٿÛÙ·ÛË, ÓˆıÚfi Î·È Â›¯Â ÌÂȈ̤ÓË ıÚ¤„Ë. ∆Ô ‚¿ÚÔ˜ ‹Ù·Ó 7300 g (<3Ë ∂.£.), ÂÓÒ ÙÔ Ì‹ÎÔ˜ Î·È Ë ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜ ‹Ù·Ó ÛÙËÓ 50‹ ∂.£. Î·È ÌÂٷ͇ 25˘-50‹˜ ∂.£., ·ÓÙ›ÛÙÔȯ·. ∂›¯Â ˆ¯ÚÔΛÙÚÈÓË ¯ÚÔÈ¿ ‰¤ÚÌ·ÙÔ˜, Ë ·ÚÙËÚȷ΋ ›ÂÛË ‹Ù·Ó 95/51 mmHg Î·È Ë Î·Ú‰È·Î‹ Û˘¯ÓfiÙËÙ· 163 ÛʇÍÂȘ/min. ∞fi ÙËÓ ·‰Ú‹ Ó¢ÚÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË ·ÚÔ˘Û›·˙ ÁÂÓÈÎÂ˘Ì¤ÓË Ì˘˚΋ ˘ÔÙÔÓ›·, ÂÓÒ ·fi Ù· ˘fiÏÔÈ· Û˘ÛÙ‹Ì·Ù· ‰ÂÓ Â›¯Â ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∞fi ÙË ÁÂÓÈ΋ ÂͤٷÛË ·›Ì·ÙÔ˜ ‰È·ÈÛÙÒıËΠÛÔ‚·Ú‹ ·Ó·ÈÌ›· Ì Hb: 5,2 g/dl, Ht: 15,2%, ̤ÛÔ fiÁÎÔ ÂÚ˘ıÚÒÓ (MCV): 109 fl, ̤ÛË ÔÛfiÙËÙ· ·ÈÌÔÛÊ·ÈÚ›Ó˘ (MCH): 37,6 pg, ÏÂ˘Î¿ ·ÈÌÔÛÊ·›ÚÈ·: 6,72 K/Ìl, ·ÈÌÔÂÙ¿ÏÈ·: 207 K/Ìl Î·È ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú·: 4,3%. ∏ ÌÔÚÊÔÏÔÁ›· ÙˆÓ ÂÚ˘ıÚÒÓ ¤‰ÂÈÍ ·ÓÈÛÔ΢ÙÙ¿ÚˆÛË, ÔÈÎÈÏÔ΢ÙÙ¿ÚˆÛË, Ì·ÎÚÔ΢ÙÙ¿ÚˆÛË, ‚·ÛÂfiÊÈÏË ÛÙ›ÍË Î·È ‰·ÎÚ˘Ô·ÙÙ·Ú·, ÛÙÔȯ›· Ô˘ Û˘ÓËÁÔÚÔ‡Ó ˘¤Ú ÌÂÁ·ÏÔ‚Ï·ÛÙÈ΋˜ ·Ó·ÈÌ›·˜. √ ˘fiÏÔÈÔ˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ: ÁÏ˘Îfi˙Ë: 83 mg/dl, Ô˘Ú›·: 19 mg/dl, ÎÚ·ÙÈÓ›ÓË: 0,3 mg/dl, Na: 142 mEq/L, K: 4,4 mEq/L, Cl: 102 mEq/L, Ca: 8,9 mg/dl, Ph: 4,6 mg/dl, Mg: 2,4 mg/dl, ÔÏÈο Ï¢ÎÒÌ·Ù· ÔÚÔ‡: 5,6 g/dl, ·Ï‚Ô˘Ì›ÓË: 3,7 g/dl, oÏÈ΋ ¯ÔÏÂÚ˘ıÚ›ÓË: 2,7 mg/dl, ¿ÌÂÛË ¯ÔÏÂÚ˘ıÚ›ÓË: 0,7 mg/dl, SGOT: 44U/L, SGPT: 14U/L, Û›‰ËÚÔ˜ ÔÚÔ‡: 156 Ìg/dl, ÊÂÚÚÈÙ›ÓË ÔÚÔ‡: 116 ng /ml, ‰ÔÎÈÌ·Û›· ‰Ú¿ӈÛ˘ ·ÚÓËÙÈ΋, ¿ÌÂÛË Coombs ·ÚÓËÙÈ΋, ¿ÚÎÂÈ· ÙÔ˘ ÂÓ˙‡ÌÔ˘ G6PD, ›‰· ÙÔ˘ ‰È·Ï˘ÙÔ‡ ˘Ô‰Ô¯¤· Ù˘ ÙÚ·ÓÛÊÂÚ›Ó˘ (sTfR): 2,32 mg/l (Ê.Ù.: 0,83-1,76 mg/l), Ê˘ÏÏÈÎfi Ô͇: 17 ng /ml (Ê.Ù.: 5-17,5 ng/ml), ‚ÈÙ·Ì›ÓË µ12 ÔÚÔ‡: 102 pg/ml (Ê.Ù.: 120-970 pg/ml), LDH: 1460 πU/L, Ï‹ÚË ¤ÏÂÁ¯Ô ËÎÙÈÎfiÙËÙ·˜ Ê˘ÛÈÔÏÔÁÈÎfi. ŒÁÈÓ ‚·ÚÈÔ‡¯Ô Á‡̷, Ô˘ ¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈÎfi ÛÙfiÌ·¯Ô Î·È ‰ˆ‰Âη‰¿ÎÙ˘ÏÔ. ∏ ÌËÙ¤Ú· ÙÔ˘ ‚Ú¤ÊÔ˘˜ ›¯Â: ∏b: 12,6 g/dl, Ht: 37,9%, MCV: 87,9 fl, MCH: 29,3 pg, ÏÂ˘Î¿ ·ÈÌÔÛÊ·›ÚÈ·: 6,80 K/Ìl Î·È PLT: 287 K/Ìl, ‚ÈÙ·Ì›ÓË µ12: 112 pg/ml. ∆o ‚Ú¤ÊÔ˜ ÌÂÙ·ÁÁ›ÛÙËΠ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘ Ì 90 ml Û˘Ì˘ÎÓˆÌ¤ÓˆÓ ÂÚ˘ıÚÒÓ. §fiÁˆ Ù˘ ¿ÚÓËÛ˘ Ï‹„˘ ÙÚÔÊ‹˜ ÂÙ¤ıË ÚÈÓÔÁ·ÛÙÚÈÎfi˜ ηıÂÙ‹Ú·˜ Î·È ÛÈÙÈ˙fiÙ·Ó Ì ÌÂÚÈÎÒ˜ ˘‰ÚÔÏ˘Ì¤ÓÔ Á¿Ï·. ÃÔÚËÁ‹ıËΠÂÓ‰ÔÌ˘˚ÎÒ˜ ˘‰ÚÔ͢ÎÔ‚·Ï·Ì›ÓË Û ‰fiÛË 250 Ìg οı 2 Ë̤Ú˜ › 2 ‚‰ÔÌ¿‰Â˜ Î·È ÛÙË Û˘Ó¤¯ÂÈ· Paediatriki 2007;70:54-59
250 Ìg ·Ó¿ ‚‰ÔÌ¿‰·. ∂›Û˘, ¯ÔÚËÁ‹ıËΠۛ‰ËÚÔ˜ per os Û ‰fiÛË 3 mg/kg/24 h. ∞fi ÙȘ ÚÒÙ˜ Ë̤Ú˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ÙÔ ‚Ú¤ÊÔ˜ ·ÚÔ˘Û›·Û ‚ÂÏÙ›ˆÛË ÙÔ˘ Ì˘˚ÎÔ‡ ÙfiÓÔ˘ Î·È Ù˘ ÎÈÓËÙÈÎfiÙËÙ¿˜ ÙÔ˘. ÕÚ¯ÈÛ ӷ ¯·ÌÔÁÂÏ¿ Î·È Ó· ·›˙ÂÈ Ì ÙÔ˘˜ ÔÈΛԢ˜ ÙÔ˘. √ ÚÈÓÔÁ·ÛÙÚÈÎfi˜ ηıÂÙ‹Ú·˜ ·Ê·ÈÚ¤ıËÎÂ Î·È ÙÔ ·È‰› ¿Ú¯ÈÛ ӷ ÛÈÙ›˙ÂÙ·È ·fi ÙÔ ÛÙfiÌ·, Ì ÛÙ·‰È·Î‹ ‚ÂÏÙ›ˆÛË Ù˘ fiÚÂÍ‹˜ ÙÔ˘ Î·È Ù˘ ÚfiÛÏ˄˘ ‚¿ÚÔ˘˜. ¶¤ÓÙ ‚‰ÔÌ¿‰Â˜ ÂÚ›Ô˘ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜ Ì ˘‰ÚÔ͢ÎÔ‚·Ï·Ì›ÓË, Ë ÁÂÓÈ΋ ÂͤٷÛË ·›Ì·ÙÔ˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋ Î·È ÔÈ ÙÈ̤˜ Ù˘ µµ12 ÛÙÔÓ ÔÚfi ‹Ù·Ó >1000 pg/ml. ∆Ô ·È‰› ¿Ú¯ÈÛ ӷ ÛÈÙ›˙ÂÙ·È Ì ‰È·ÙÚÔÊ‹ Ô˘ ÂÚÈÂÏ¿Ì‚·Ó Á·Ï·ÎÙÔÎÔÌÈο ÚÔ˚fiÓÙ·, ·ÏÏ¿ fi¯È „¿ÚÈ Î·È ÎÚ¤·˜ (ÏfiÁˆ ¿ÚÓËÛ˘ ÙˆÓ ÁÔÓ¤ˆÓ). ∆Ô ‚¿ÚÔ˜ ·ӋÏı Û ›‰· ¿Óˆ Ù˘ 50‹˜ ∂.£. ÁÈ· ÙËÓ ËÏÈΛ· ÙÔ˘ ·È‰ÈÔ‡. ™Â ËÏÈΛ· 14 ÌËÓÒÓ ÂÚ·ÙÔ‡Û ηÓÔÓÈο Î·È ¿Ú¯ÈÛ ӷ ϤÂÈ 2-3 ϤÍÂȘ Ì ¤ÓÓÔÈ·. ∏ ·ÓÙ›ÏË„Ë Î·È Ë ÎÔÈÓˆÓÈÎfiÙËÙ¿ ÙÔ˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜. ∏ ·Ú·ÎÔÏÔ‡ıËÛË ÙÔ˘ ·È‰ÈÔ‡ Û˘Ó¯›ÛÙËΠ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 16 ÌËÓÒÓ, ÔfiÙÂ Ë ÔÈÎÔÁ¤ÓÂÈ· ÌÂÙ·ÎfiÌÈÛ ÛÙÔ Â͈ÙÂÚÈÎfi.
™˘˙‹ÙËÛË ∏ µµ12 ‰ÂÓ ·Ú¿ÁÂÙ·È ÛÙÔÓ ·ÓıÚÒÈÓÔ ÔÚÁ·ÓÈÛÌfi Î·È ËÁ¤˜ Ù˘ Â›Ó·È ·ÔÎÏÂÈÛÙÈο ÔÈ ˙ˆÈΤ˜ ÙÚÔʤ˜, fiˆ˜ ÙÔ ÎÚ¤·˜, ÙÔ „¿ÚÈ, Ù· ·˘Á¿ Î·È Ù· Á·Ï·ÎÙÔÎÔÌÈο ÚÔ˚fiÓÙ·. √È ËÌÂÚ‹ÛȘ ·Ó¿ÁΘ Û µµ12 ÛÙ· ‚Ú¤ÊË ˘ÔÏÔÁ›˙ÔÓÙ·È Û 0,4 Ìg, ÛÙ· ·È‰È¿ ËÏÈΛ·˜ 1-8 ÂÙÒÓ Û 1,1 Ìg, ÛÙ· ·È‰È¿ ËÏÈΛ·˜ 9-13 ÂÙÒÓ Û 1,8 Ìg, ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ Î·È ÂÓ‹ÏÈΘ Û 2,4 Ìg, ÂÓÒ ÛÙȘ ÂÁ·Ԣ˜ Î·È ıËÏ¿˙Ô˘Û˜ Á˘Ó·›Î˜ ÔÈ ·Ó¿ÁΘ ·˘Í¿ÓÔÓÙ·È Û 2,8 Ìg (3). ∆· Ë·ÙÈο ·Ôı¤Ì·Ù· ÙˆÓ ÂÓËÏ›ÎˆÓ Ô˘ ηٷӷÏÒÓÔ˘Ó ˙ˆÈΤ˜ ÙÚÔʤ˜ Â›Ó·È ÂÚ›Ô˘ 3-5 mg µ12 , ÂÓÒ Î·Ù¿ ÙË Á¤ÓÓËÛË ˘¿Ú¯Ô˘Ó ÌÈÎÚ¿ ·Ôı¤Ì·Ù·, ÂÚ›Ô˘ 25Ìg. ∆· ·Ôı¤Ì·Ù· ·˘Ù¿ Â›Ó·È ÌÈÎÚfiÙÂÚ· Â¿Ó Ë ÌËÙ¤Ú· ÙÔ˘ ÓÂÔÁÓÔ‡ ¤¯ÂÈ ∞µµ12 ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ·ËÛ˘ (7). ∂¿Ó ÏËÊıÔ‡Ó ˘’ fi„ÈÓ ÔÈ ËÌÂÚ‹ÛȘ ·Ó¿ÁΘ ÙˆÓ ÂÓËÏ›ÎˆÓ Û µµ12 Û ۯ¤ÛË Ì ٷ ·Ôı¤Ì·Ù¿ ÙÔ˘˜, ÌÔÚ› Ó· Á›ÓÂÈ Î·Ù·ÓÔËÙfi ÁÈ·Ù› ·˘ÛÙËÚÒ˜ ¯ÔÚÙÔÊ¿ÁÔÈ ÂÓ‹ÏÈΘ ηı˘ÛÙÂÚÔ‡Ó ÔÏÏ¿ ¯ÚfiÓÈ· Ó· ÂÌÊ·Ó›ÛÔ˘Ó Û˘ÌÙÒÌ·Ù· ∞µµ12 ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ¯ÔÚÙÔÊ·ÁÈ΋˜ ‰›·ÈÙ·˜. ∆· ÓÂÔÁÓ¿, ·ÓÙ›ıÂÙ·, ÏfiÁˆ ÙˆÓ ÌÈÎÚÒÓ ·ÔıÂÌ¿ÙˆÓ ÙÔ˘˜ Û µµ12 , ÂÌÊ·Ó›˙Ô˘Ó Û˘ÌÙÒÌ·Ù· ·Ó¿ÚÎÂÈ·˜ ̤۷ Û ϛÁÔ˘˜ Ì‹Ó˜, ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ Ë ÚfiÛÏË„Ë ‰ÂÓ Î·Ï‡ÙÂÈ ÙȘ ËÌÂÚ‹ÛȘ ·Ó¿ÁΘ Û µµ12 (7). ™˘Ó‹ıˆ˜, ∞µµ12 ÂÌÊ·Ó›˙Ô˘Ó Ù· ‚Ú¤ÊË Ô˘ ıËÏ¿˙Ô˘Ó ·ÔÎÏÂÈÛÙÈο ÁÈ· ÌÂÁ¿ÏÔ ‰È¿ÛÙËÌ· ·fi ·˘ÛÙËÚÒ˜ ¯ÔÚÙÔÊ¿ÁÔ˘˜ ÌËÙ¤Ú˜, ÛÙȘ Ôԛ˜ ‰ÂÓ ¯ÔÚËÁÂ›Ù·È Û˘ÌÏËڈ̷ÙÈο µµ12. ∆· ›‰· µµ12 ÛÙÔ ÌËÙÚÈÎfi Á¿Ï· ¤¯Ô˘Ó ÁÚ·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË Ì ·˘Ù¿ ÙÔ˘ Ï¿ÛÌ·ÙÔ˜ ηÈ
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™ÈÙÈÔÁÂÓ‹˜ ·Ó¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ µ12 Û ‚Ú¤ÊÔ˜
ÛÙȘ ·˘ÛÙËÚÒ˜ ¯ÔÚÙÔÊ¿ÁÔ˘˜ ÌËÙ¤Ú˜ ·˘Ù¿ ·Ó¢ڛÛÎÔÓÙ·È Ôχ ¯·ÌËÏ¿ (1,2). ∆· ·ÚÈ· ÎÏÈÓÈο Û˘ÌÙÒÌ·Ù· Ù˘ Aµµ12 ÂÍËÁÔ‡ÓÙ·È ÏfiÁˆ Ù˘ ÂÌÏÔ΋˜ Ù˘ µµ12 Û ÛËÌ·ÓÙÈΤ˜ ÌÂÙ·‚ÔÏÈΤ˜ ·ÓÙȉڿÛÂȘ (1) (∂ÈÎfiÓ· 1). ∏ µµ12 Â›Ó·È Û˘Ó¤Ó˙˘ÌÔ ·Ó·Áη›Ô ÁÈ· ÙË ÌÂı˘Ï›ˆÛË Ù˘ ÔÌÔ΢ÛÙ½Ó˘ Û ÌÂıÂÈÔÓ›ÓË, ÙË ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ÌÂı˘ÏÌ·ÏÔÓ˘Ï-Û˘ÓÂÓ˙‡ÌÔ˘ ∞ Û ÛÔ˘ÎÈÓ˘Ï-Û˘Ó¤Ó˙˘ÌÔ ∞ Î·È ÁÈ· ÙË Û‡ÓıÂÛË ÙÔ˘ ÙÂÙÚ·¸‰ÚÔÊ˘ÏÏÈÎÔ‡ ÔͤԘ ·fi ÙÔ ÌÂı˘ÏÙÂÙÚ·¸‰ÚÔÊ˘ÏÏÈÎfi Ô͇. ™Â ·ÛıÂÓ›˜ Ì Aµµ12 ·ıÚÔ›˙ÔÓÙ·È Úfi‰ÚÔ̘ Ô˘Û›Â˜, fiˆ˜ ÔÌÔ΢ÛÙ½ÓË Î·È ÌÂı˘ÏÌ·ÏÔÓÈÎfi Ô͇, ÔÈ Ôԛ˜ ÌÔÚ› Ó· ÚÔÛ‰ÈÔÚÈÛÙÔ‡Ó ÛÙÔÓ ÔÚfi Î·È ·ÔÙÂÏÔ‡Ó ÚÒÈÌÔ˘˜ Î·È ·ÍÈfiÈÛÙÔ˘˜ ‰Â›ÎÙ˜ Aµµ12 (1). ∏ ÌÂıÂÈÔÓ›ÓË Î·È ÙÔ ÙÂÙÚ·¸‰ÚÔÊ˘ÏÏÈÎfi Â›Ó·È ··Ú·›ÙËÙ˜ Ô˘Û›Â˜ ÁÈ· ÙË Û‡ÓıÂÛË DNA Î·È Ë ·Ó¿ÚÎÂÈ¿ ÙÔ˘˜ Ô‰ËÁ› Û ‰È·Ù·Ú·¯¤˜ Û fiÏ· Ù· Ù·¯¤ˆ˜ ‰È·ÈÚÔ‡ÌÂÓ· ·ÙÙ·Ú·, fiˆ˜ Â›Ó·È Ù· ·ÈÌÔÔÈËÙÈο Î·È Ù· ·ÙÙ·Ú· ÙÔ˘ Á·ÛÙÚÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘. ªÂÁ·ÏÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, ÁψÛÛ›Ùȉ· Î·È ÌÂÚÈ΋ ·ÙÚÔÊ›· ÙˆÓ Ï·¯ÓÒÓ Â›Ó·È Û˘¯Ó¤˜ ÂΉËÏÒÛÂȘ Ù˘ ∞µµ12 (9). ÕÏÏË ÌÂÙ·‚ÔÏÈ΋ ·ÓÙ›‰Ú·ÛË Ô˘ ÂÍ·ÚÙ¿Ù·È ·fi ÙË µµ12 Â›Ó·È Ë ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ÌÂı˘ÏÌ·ÏÔÓÈÎÔ‡-CoA Û ÛÔ˘ÎÈÓ˘ÏCoA. ∏ ·Ó·Ú΋˜ ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ÌÂı˘ÏÌ·ÏÔÓÈÎÔ‡-CoA Ô‰ËÁ› Û ·‡ÍËÛË Ù˘ Úfi‰ÚÔÌ˘ Ô˘Û›·˜ ÚÔÈÔÓ˘Ï-CoA. ∏ ¿ıÚÔÈÛË ÌÂı˘ÏÌ·ÏÔÓÈÎÔ‡-CoA Î·È ÚÔÈÔÓ˘Ï-CoA Ô‰ËÁ› Û ۇÓıÂÛË ·ÓÒÌ·ÏˆÓ ·Ï˘Û›‰ˆÓ ÏÈ·ÚÒÓ ÔͤˆÓ, ÔÈ Ôԛ˜ Ô‰ËÁÔ‡Ó Û ÂÓۈ̿وÛË ÌÂÁ¿ÏˆÓ ÔÛÔÙ‹ÙˆÓ ·Û˘Ó‹ıÈÛÙˆÓ ÏÈ·ÚÒÓ ÔͤˆÓ C15 Î·È C17 ÛÙÔ ÙÔ›¯ˆÌ· ÙˆÓ Ó‡ڈÓ. ∆Ô ·ÔÙ¤ÏÂÛÌ· Â›Ó·È ·ÔÌ˘ÂϛӈÛË, ·ÍÔÓÈ΋ ÂÎʇÏÈÛË Î·È ı¿Ó·ÙÔ˜ Ó¢ÚÒÓˆÓ (7,8). ∆· Ó¢ÚÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙÔ˘˜ ÂÓ‹ÏÈΘ ·ÊÔÚÔ‡Ó Î˘Ú›ˆ˜ ÛÙÔ ÂÚÈÊÂÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ·, ÂÓÒ ÛÙ· ‚Ú¤ÊË ÛÙÔ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· Î·È ·Ô‰›‰ÔÓÙ·È Î˘Ú›ˆ˜ ÛÙȘ ·ÓˆÙ¤Úˆ ÌÂÙ·‚ÔÏÈΤ˜ ‰È·Ù·Ú·¯¤˜ (1), ¯ˆÚ›˜ fï˜ Ó· ·ÔÎÏ›ÔÓÙ·È Î·È ¿ÏÏÔÈ Ì˯·ÓÈÛÌÔ› (7,9). ∏ ·‡ÍËÛË Ù˘ ÔÌÔ΢ÛÙ½Ó˘ ıˆÚÂ›Ù·È ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ·ÔÊÚ·ÎÙÈ΋ ·ÁÁÂÈÔ¿ıÂÈ·, Û˘ÁÁÂÓ‹ ηډÈÔ¿ıÂÈ·, ÓfiÛÔ Alzheimer Î·È „˘¯ÒÛÂȘ (2,3). ∏ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÛÙ· ‚Ú¤ÊË Ì ∞µµ12 Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ·ÎÔÏÔ˘ı› ¤Ó· ÛÙ·ıÂÚfi ÌÔÓÙ¤ÏÔ. ∏ ÂÌÊ¿ÓÈÛË Û˘ÌÙˆÌ¿ÙˆÓ ·Ú¯›˙ÂÈ Û˘Ó‹ıˆ˜ ÛÙËÓ ËÏÈΛ· ÙˆÓ 4-15 ÌËÓÒÓ, ÌÂÙ¿ ·fi Ì›· ÂÚ›Ô‰Ô Ê˘ÛÈÔÏÔÁÈ΋˜ ·Ó¿Ù˘Í˘. ∆· ÚÒÙ· Û˘ÌÙÒÌ·Ù· Â›Ó·È ·ÓÔÚÂÍ›·, ¢ÂÚÂıÈÛÙfiÙËÙ·, ·¿ıÂÈ·, ¤ÌÂÙÔÈ, ·‰‡Ó·ÌÔ ÎÏ¿Ì· Î·È Î·ı˘ÛÙ¤ÚËÛË ‹ ·ÏÈÓ‰ÚfiÌËÛË ÛÙËÓ „˘¯ÔÎÈÓËÙÈ΋ ·Ó¿Ù˘ÍË. ∏ ۈ̷ÙÈ΋ ·Ó¿Ù˘ÍË ·Ú¯›˙ÂÈ Ó· ˘ÔÏ›ÂÙ·È, ÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ÂÚÈ̤ÙÚÔ˘ ÎÂÊ·Ï‹˜, Û˘¯Ó¿ ˘¿Ú¯ÂÈ ˆ¯ÚfiÙËÙ· ‰¤ÚÌ·ÙÔ˜ Î·È ÁψÛÛ›Ùȉ·, ÂÓÒ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó Ó¢ÚÔÏÔÁÈο Û˘ÌÙÒÌ·Ù·, fiˆ˜ ˘ÔÙÔÓ›·, ÌÂȈ̤ӷ ÙÂÓfiÓÙÈ· ·ÓÙ·Ó·ÎÏ·ÛÙÈο
Î·È ¯ÔÚÈÔ·ıÂÙˆÛÈΤ˜ ÎÈÓ‹ÛÂȘ ηÈ, Û ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ, ÎÒÌ· Ë ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· (9). √È ÏÔȤ˜ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ Ù˘ ∞µµ12 ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 2 (1). ∏ ÁÂÓÈ΋ ÂͤٷÛË ·›Ì·ÙÔ˜ ÛÙ· ·È‰È¿ Û˘Ó‹ıˆ˜ ·ÔηχÙÂÈ ·‡ÍËÛË ÙÔ˘ MCV Î·È MCH, ÂÓÒ Ù· ›‰· Ù˘ Hb ÔÈΛÏÏÔ˘Ó. ÕÏÏÔ Û˘¯Ófi ‡ÚËÌ· ÛÙË ÁÂÓÈ΋ ·›Ì·ÙÔ˜ Â›Ó·È Ë ˘ÂÚηٿÙÌËÛË ÙÔ˘ ˘Ú‹Ó· ÙˆÓ Ô˘‰ÂÙÂÚÔʛψÓ. √È ÌËÙ¤Ú˜ ÙˆÓ ·Û¯fiÓÙˆÓ ‚ÚÂÊÒÓ Û˘Ó‹ıˆ˜ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Ì·ÎÚÔ΢ÙÙ·ÚÈ΋ ·Ó·ÈÌ›· Î·È Ù· ›‰· Ù˘ µµ12 Â›Ó·È ÛÙ· ηÙÒÙÂÚ· Ê˘ÛÈÔÏÔÁÈο ›‰·. °ÂÓÈο, ÔÈ ·˘ÛÙËÚÒ˜ ¯ÔÚÙÔÊ¿ÁÔÈ ÂÓ‹ÏÈΘ Û¿ÓÈ· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÂÁ·ÏÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›·. ∆Ô ·Ú·¿Óˆ Û˘Ì‚·›ÓÂÈ ÏfiÁˆ ÙˆÓ ˘„ËÏÒÓ ·ÔıÂÌ¿ÙˆÓ ÙÔ˘˜ ÛÂ Ê˘ÏÏÈÎfi Ô͇, Ô˘ ÔÊ›ÏÂÙ·È ÛÙËÓ Â·Ú΋ ÚfiÛÏË„Ë Ê˘ÙÈÎÒÓ ÙÚÔÊÒÓ, ·ÏÏ¿ Î·È ÛÙËÓ ¿ıÚÔÈÛË Ê˘ÏÏÈÎÔ‡ ÔͤԘ ÛÙÔ˘˜ ÈÛÙÔ‡˜ ÏfiÁˆ Ù˘ ÌË ÌÂÙ·ÙÚÔ‹˜ ÙÔ˘ Û ÙÂÙÚ·¸‰ÚÔÊ˘ÏÏÈÎfi Ô͇ (∂ÈÎfiÓ· 2). ∫·Ù’ ·˘ÙfiÓ ÙÔÓ ÙÚfiÔ Û˘ÁηχÙÂÙ·È Ë Ì·ÎÚÔ΢ÙÙ¿ÚˆÛË Ô˘ ·Ú·ÙËÚÂ›Ù·È ÛÙËÓ ∞µµ12. ∏ ÛȉËÚÔÂÓ›·, Ô˘ Â›Ó·È Û˘¯Ó‹ Û fiϘ ÙȘ ÌÔÚʤ˜ ¯ÔÚÙÔÊ·ÁÈ΋˜ ‰›·ÈÙ·˜, Â›Ó·È ¤Ó·˜ ¿ÏÏÔ˜ ÏfiÁÔ˜ Ô˘ Û˘¯Ó¿ Û˘ÁηχÙÂÈ ÙË Ì·ÎÚÔ΢ÙÙ¿ÚˆÛË, Ô‰ËÁÒÓÙ·˜ ÌÂÚÈΤ˜ ÊÔÚ¤˜ Ì¿ÏÈÛÙ· Û ÌÈÎÚÔ΢ÙÙ¿ÚˆÛË (10,11). ™ÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜, Ë Ì¤ÙÚËÛË ÙÔ˘ ªª∞ ı¤ÙÂÈ ÙË ‰È¿ÁÓˆÛË Ù˘ ∞µµ12. ™˘¯Ó¿ ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Ù˘ ∞µµ12 ÛÙ· ·È‰È¿ Â›Ó·È Ë ·‡ÍËÛË Ù˘ ¤ÌÌÂÛ˘ ¯ÔÏÂÚ˘ıÚ›Ó˘ Î·È Ù˘ Á·Ï·ÎÙÈ΋˜ ‰Â¸‰ÚÔÁÂÓ¿Û˘ (LDH) ÛÙÔÓ ÔÚfi, Ô˘ ·ÓÙ·Ó·ÎÏÔ‡Ó ÙË ÌË ·Ô‰ÔÙÈ΋ ÂÚ˘ıÚÔÔ›ËÛË Î·È Î·Ù·ÛÙÚÔÊ‹ ÙˆÓ Úfi‰ÚÔÌˆÓ ÌÔÚÊÒÓ ÙˆÓ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ. ™ÙËÓ ·ÂÈÎfiÓÈÛË ÙÔ˘ ∫¡™ Ì ̷ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· (MRI), ·Ó¢ڛÛÎÂÙ·È Û˘¯Ó¿ ÂÁÎÂÊ·ÏÈ΋ ·ÙÚÔÊ›· Î·È Î·ı˘ÛÙ¤ÚËÛË ÛÙË Ì˘ÂϛӈÛË (1,7,9). ∆Ô ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ· (HE°) ÌÔÚ› Ó· ‰Â›ÍÂÈ ‰È¿¯˘ÙË ÂÈ‚Ú¿‰˘ÓÛË ‹ ·Ô˘Û›· ÙÔ˘ ‚·ÛÈÎÔ‡ Ú˘ıÌÔ‡ (1,7,9). ∏ ‰È¿ÁÓˆÛË Ù˘ ∞µµ12 ÌÔÚ› Ó· ηı˘ÛÙÂÚ‹ÛÂÈ ‹ Ó· ‰È·Ê‡ÁÂÈ ÏfiÁˆ ¤ÏÏÂȄ˘ ÂȉÈÎÒÓ Û˘Ìو̿وÓ. ∏ ̤ÙÚËÛË Ù˘ µµ12 ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi ÙˆÓ ÂȤ‰ˆÓ ÙÔ˘ ªª∞ Î·È Ù˘ ÔÏÈ΋˜ ÔÌÔ΢ÛÙ½Ó˘ ÛÙÔÓ ÔÚfi ıˆÚÂ›Ù·È fiÙÈ ·ÔÙÂÏÔ‡Ó ··Ú·›ÙËÙ˜ ÂÍÂÙ¿ÛÂȘ Ô˘ ı¤ÙÔ˘Ó ÙË ‰È¿ÁÓˆÛË fiÙ·Ó ˘¿Ú¯Ô˘Ó Û˘ÌÙÒÌ·Ù· Ù· ÔÔ›· ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 2, ·ÏÏ¿ Î·È Î·Ù·ÛÙ¿ÛÂȘ Ô˘ Û˘Óԉ‡ÔÓÙ·È ·fi ∞µµ12 , fiˆ˜ Ê·›ÓÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1 (1,12). ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÌÂٷ͇ Ù˘ ÛÈÙÈÔÁÂÓÔ‡˜ ¤ÏÏÂȄ˘ µµ12 Î·È ÙˆÓ ÂÓ‰ÔÁÂÓÒÓ ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ Ù˘ µµ12 Â›Ó·È ÛËÌ·ÓÙÈ΋, ȉȷ›ÙÂÚ· ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·. ∆Ô ÈÛÙÔÚÈÎfi ·ÚÎÔ‡˜ ÚfiÛÏ˄˘ µµ12 ÛÂ Û˘Ó‰˘·ÛÌfi ÌÂ Ê˘ÛÈÔÏÔÁÈο ‹ Î·È ·˘ÍË̤ӷ ›‰· µµ12 Î·È ªª∞ ÛÙÔÓ ÔÚfi ÙÔ˘ ‚Ú¤ÊÔ˘˜ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ÎÏÈÓÈΤ˜ ¶·È‰È·ÙÚÈ΋ 2007;70:54-59
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µ. ∞ÁÁÂÏ¿ÎÔ˘ Î·È Û˘Ó.
¶›Ó·Î·˜ 2. ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·Ó¿ÚÎÂÈ·˜ ‚ÈÙ·Ì›Ó˘ µ12 ÛÙ· ·È‰È¿ °ÂÓÈο Û˘ÌÙÒÌ·Ù· ∞‰˘Ó·Ì›· ∂‡ÎÔÏË ÎfiˆÛË ∞ÓÔÚÂÍ›· ∞Ó·Ú΋˜ ·‡ÍËÛË ∂˘ÂÚÂıÈÛÙfiÙËÙ· ¡Â˘ÚÔÏÔÁÈο/„˘¯È·ÙÚÈο Û˘ÌÙÒÌ·Ù· ∞Ó·Ù˘Íȷ΋ ηı˘ÛÙ¤ÚËÛË ‹ ·ÏÈÓ‰ÚfiÌËÛË ¶·Ú·ÈÛıËۛ˜ ÀÔÙÔÓ›· ÃÔÚÈÔ·ıÂÙˆÛÈΤ˜ ÎÈÓ‹ÛÂȘ ™·ÛÌÔ› ∞Ù·Í›· ∫ÒÌ· ∞ÒÏÂÈ· ÌÓ‹Ì˘ ∞ÏÏ·Á‹ Ù˘ ÚÔÛˆÈÎfiÙËÙ·˜ ªÂ›ˆÛË ÙˆÓ Û¯ÔÏÈÎÒÓ ÂȉfiÛÂˆÓ ∫·Ù¿ıÏÈ„Ë ∞ÈÌ·ÙÔÏÔÁÈΤ˜ ÂΉËÏÒÛÂȘ ª·ÎÚÔ΢ÙÙ¿ÚˆÛË ∞Ó·ÈÌ›· ÀÂÚηٿÙÌËÛË ÙÔ˘ ˘Ú‹Ó· ÙˆÓ Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ §Â˘ÎÔÂÓ›· £ÚÔÌ‚ÔÂÓ›· ¶·Ó΢ÙÙ·ÚÔÂÓ›· ÕÏϘ ÂΉËÏÒÛÂȘ °ÏˆÛÛ›Ùȉ· À¤Ú¯ÚˆÛË ‰¤ÚÌ·ÙÔ˜ ŒÌÂÙÔÈ›/‰È¿ÚÚÔÈ· ÿÎÙÂÚÔ˜ ∫·Ú‰È·Î‹ ·Ó¿ÚÎÂÈ·
ÂΉËÏÒÛÂȘ Û˘Ì‚·Ù¤˜ Ì ∞µµ12 , Û˘ÓËÁÔÚÔ‡Ó ˘¤Ú ÂÓ‰ÔÁÂÓÔ‡˜ ‰È·Ù·Ú·¯‹˜ ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ Ù˘ µµ12 (1).
∏ ÎÏÈÓÈ΋ ·ÓÙ·fiÎÚÈÛË Û¯Â‰fiÓ fiÏˆÓ ÙˆÓ ‚ÚÂÊÒÓ Ì ÛÈÙÈÔÁÂÓ‹ ¤ÏÏÂÈ„Ë µµ12 ÛÙË ¯ÔÚ‹ÁËÛË µµ12 Â›Ó·È ı·̷ÙÈ΋ Û fi,ÙÈ ·ÊÔÚ¿ ÛÙ· ÁÂÓÈο Û˘ÌÙÒÌ·Ù·, ÛÙȘ Á·ÛÙÚÂÓÙÂÚÈΤ˜ Î·È ·ÈÌ·ÙÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÛÙË ÛˆÌ·ÙÈ΋ ·Ó¿Ù˘ÍË, ·ÏÏ¿ Î·È Û ÔÚÈṲ̂ӷ Ó¢ÚÔÏÔÁÈο Û˘ÌÙÒÌ·Ù·, fiˆ˜ Ë ˘ÔÙÔÓ›· (1,6-9). ∆· Â˘Ú‹Ì·Ù· ÛÙËÓ MRI Î·È ÙÔ ∏∂° Û˘Ó‹ıˆ˜ ·Ôηı›ÛÙ·ÓÙ·È (1,7,9), ¯ˆÚ›˜ ˆÛÙfiÛÔ Ó· ÌÔÚ› Ó· ·ÔÎÏÂÈÛÙ› Ë ·Ú·ÌÔÓ‹ Ó¢ÚÔ·Ó·Ù˘ÍÈ·ÎÒÓ ·ıÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ. ∆¤ÙÔÈ· Â˘Ú‹Ì·Ù· Â›Ó·È Ë ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË, ÔÈ ‰È·Ù·Ú·¯¤˜ ‚¿‰ÈÛ˘ ‹ Ë ÂÈÏË„›· (10). √ ΛӉ˘ÓÔ˜ ·Ú·ÌÔÓ‹˜ ÌfiÓÈÌˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ ÂΉËÏÒÛÂˆÓ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÙË ÛÔ‚·ÚfiÙËÙ· ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Î·Ù¿ ÙË ‰È¿ÁÓˆÛË, ·ÏÏ¿ Î·È Ì ÙËÓ Î·ı˘ÛÙ¤ÚËÛË Ù˘ ‰È¿ÁÓˆÛ˘ Î·È Ù˘ ¤Ó·Ú͢ ıÂڷ›·˜ (1,7,10). ªÂϤÙË Û ÂÊ‹‚Ô˘˜ Ô˘ ÛÙË ‚ÚÂÊÈ΋ Î·È ÚÒÙË ·È‰È΋ ËÏÈΛ· ÛÈÙ›ÛÙËÎ·Ó Ì ·ÌÈÁÒ˜ ¯ÔÚÙÔÊ·ÁÈ΋ ‰›·ÈÙ· Î·È ÛÙË Û˘Ó¤¯ÂÈ· ¿Ú¯ÈÛ·Ó Ó· ηٷӷÏÒÓÔ˘Ó ˙ˆ˚Τ˜ ÙÚÔʤ˜, ·Ú¤¯ÂÈ Ôχ ÛËÌ·ÓÙÈΤ˜ ÏËÚÔÊÔڛ˜ (13). ™ÙË ÌÂϤÙË ·˘Ù‹ Ê·›ÓÂÙ·È fiÙÈ ÔÈ ·ÓÂÈı‡ÌËÙ˜ ÂȉڿÛÂȘ Ù˘ ∞µµ12 ÛÙÔ˘˜ ¯ÔÚÙÔÊ¿ÁÔ˘˜ ÌÔÚ› Ó· ÌËÓ ÂÚÈÔÚ›˙ÔÓÙ·È ÌfiÓÔ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, ·ÏÏ¿ Ó· ÂÂÎÙ›ÓÔÓÙ·È Î·È ÛÙËÓ ÂÊË‚È΋ ËÏÈΛ·. ∏ ·ÏÏ·Á‹ Ù˘ ‰È·ÙÚÔÊ‹˜ ·fi ·ÌÈÁÒ˜ ¯ÔÚÙÔÊ·ÁÈ΋ Û ‰›·ÈÙ· Ô˘ ÂÚȤ¯ÂÈ ·˘Á¿, Á·Ï·ÎÙÔÎÔÌÈο ‹ Î·È ÎÚ¤·˜ ‰ÂÓ Â›Ó·È ÈηӋ ÁÈ· ÙË ‰È·Ù‹ÚËÛË Ù˘ ¿ÚÎÂÈ·˜ µµ12 Â¿Ó Î·Ù·Ó·ÏÒÓÔÓÙ·È Ì¤ÙÚȘ ÔÛfiÙËÙ˜ ˙ˆ˚ÎÒÓ ÙÚÔÊÒÓ Î·È ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ÁÓˆÛÙÈΤ˜ Î·È „˘¯ÔÎÈÓËÙÈΤ˜ ‰È·Ù·Ú·¯¤˜. ™˘ÌÂÚ·ÛÌ·ÙÈο, ˘¿Ú¯Ô˘Ó ÔÏϤ˜ ÂӉ›ÍÂȘ fiÙÈ Ë ∞µµ12 Â›Ó·È ·ÈÙ›· ÛËÌ·ÓÙÈ΋˜ ÓÔÛËÚfiÙËÙ·˜ ÛÙËÓ ÔÌ¿‰· ÙˆÓ ·˘ÛÙËÚÒ˜ ¯ÔÚÙÔÊ¿ÁˆÓ ·ÙfiÌˆÓ Î·È ·ÊÔÚ¿ Û fiϘ ÙȘ ËÏÈ˘, Ì ÌÂÁ¿ÏË Èı·ÓfiÙËÙ·
ªÂı˘ÏÙÂÙÚ·¸‰ÚÔÊ˘ÏÏÈÎfi
∆ÂÙÚ·¸‰ÚÔÊ˘ÏÏÈÎfi
ªÂı˘ÏÙÚ·ÓÛÊÂÚ¿ÛË √ÌÔ΢ÛÙ½ÓË
ªÂıÂÈÔÓ›ÓË ªÂı˘ÏÎÔ‚·Ï·Ì›ÓË
µÈÙ·Ì›ÓË µ12 (ÎÔ‚·Ï·Ì›ÓË)
5ã-∞‰ÂÓÔÛ˘ÏÎÔ‚·Ï·Ì›ÓË L-ªÂı˘ÏÌ·ÏÔÓ˘Ï-CoA
™Ô˘ÎÈÓ˘Ï-CoA ªÂı˘ÏÌ·ÏÔÓ˘Ï-CoA ÌÔ˘Ù¿ÛË
∂ÈÎfiÓ· 2. ∏ ‚ÈÙ·Ì›ÓË µ12 Â›Ó·È Û˘Ó¤Ó˙˘ÌÔ ÁÈ· ÙË ÌÂı˘Ï›ˆÛË Ù˘ ÔÌÔ΢ÛÙ½Ó˘ Û ÌÂıÂÈÔÓ›ÓË, ÙË ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ÌÂı˘ÏÌ·ÏÔÓ˘ÏÛ˘ÓÂÓ˙‡ÌÔ˘ ∞ Û ÛÔ˘ÎÈÓ˘Ï-Û˘Ó¤Ó˙˘ÌÔ ∞ Î·È ÙË ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ÌÂı˘ÏÙÂÙÚ·¸‰ÚÔÊ˘ÏÏÈÎÔ‡ ÔͤԘ Û ÙÂÙÚ·¸‰ÚÔÊ˘ÏÏÈÎfi Ô͇. Paediatriki 2007;70:54-59
59
™ÈÙÈÔÁÂÓ‹˜ ·Ó¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ µ12 Û ‚Ú¤ÊÔ˜
Ó· Ô‰ËÁ‹ÛÂÈ Û ÌfiÓÈ̘ ·ıÔÏÔÁÈΤ˜ Ó¢ÚÔÏÔÁÈΤ˜ Î·È ·Ó·Ù˘ÍȷΤ˜ ÂΉËÏÒÛÂȘ. √È Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ µµ12 Î·È Ë ·Ô˘Û›· ·Ó·ÈÌ›·˜ ‰ÂÓ ·ÔÎÏÂ›Ô˘Ó ÙËÓ ·Ó¿ÚÎÂÈ· Ù˘ µµ12. √È ·˘ÛÙËÚÒ˜ ¯ÔÚÙÔÊ¿ÁÔÈ ÌËÙ¤Ú˜ Ú¤ÂÈ Ó· Â›Ó·È ÂÓËÌÂڈ̤Ó˜ ÁÈ· ÙË ÛËÌ·Û›· Ù˘ ·ÚÎÔ‡˜ ÚfiÛÏ˄˘ µµ12 ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ Î·È ÙÔ˘ ıËÏ·ÛÌÔ‡. ™Â ‚Ú¤ÊË ·˘ÛÙËÚÒ˜ ¯ÔÚÙÔÊ¿ÁˆÓ ÌËÙ¤ÚˆÓ Ô˘ ÙÚ¤ÊÔÓÙ·È ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi Á¿Ï·, Ú¤ÂÈ Ó· ¯ÔÚËÁÂ›Ù·È Û˘Ìϋڈ̷ µµ12. ŸÙ·Ó ˘¿Ú¯ÂÈ ˘Ô„›· ∞µµ12 Û ‚Ú¤ÊÔ˜, Ë Ï‹„Ë ÏÂÙÔÌÂÚÔ‡˜ ‰È·ÙÚÔÊÈÎÔ‡ ÈÛÙÔÚÈÎÔ‡ ÂΠ̤ÚÔ˘˜ Ù˘ ÌËÙ¤Ú·˜ Î·È ÙÔ˘ ‚Ú¤ÊÔ˘˜, ·ÏÏ¿ Î·È Ô ¤ÏÂÁ¯Ô˜ ÁÈ· ηÎÔ‹ıË ·Ó·ÈÌ›· Ù˘ ÌËÙ¤Ú·˜ ıˆÚÔ‡ÓÙ·È ··Ú·›ÙËÙ·. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È ıÂڷ›· Ù˘ ∞µµ12 ÛÙ· ‚Ú¤ÊË Â›Ó·È ··Ú·›ÙËÙË ÁÈ· ÙËÓ ·ÔÙÚÔ‹ ÌfiÓÈÌˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ.
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µÈ‚ÏÈÔÁÚ·Ê›· 1. Rasmussen SA, Fernhoff PM, Scanlon KS. Vitamin B12 deficiency in children and adolescents. J Pediatr 2001;138:10-17. 2. Asok A. Vegetarianism and vitamin B12 (cobalamin) deficiency. Am J Clin Nutr 2003;78:3-6. 3. Stabler SP, Allen RH. Vitamin B12 deficiency as a worldwide problem. Annu Rev Nutr 2004;24:299-326. 4. Sanders TA. Vegetarian diets and children. Pediatr Clin North Am 1995;42:955-965. 5. Hebbelinck M, Clarys P, De Malsche A. Growth, develop-
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ment, and physical fitness of Flemish vegetarian children, adolescents, and young adults. Am J Clin Nutr 1999;70 (3 Suppl):S579-S585. Centers for Disease Control and Prevention (CDC). Neurologic impairment in children associated with maternal dietary deficiency of cobalamin - Georgia, 2001. MMWR 2003;52:61-64. von Schenck U, Bender-Gotze C, Koletzko B. Persistence of neurological damage induced by dietary vitamin B12 deficiency in infancy. Arch Dis Child 1997;77:137-139. Graham SM, Arvela OM, Wise GA. Long-term neurologic consequences of nutritional vitamin B12 deficiency in infants. J Pediatr 1992;121:710-714. Codazzi D, Sala F, Parini R, Langer M. Coma and respiratory failure in a child with severe vitamin B(12) deficiency. Pediatr Crit Care Med 2005;6:483-485. Weiss R, Fogelman Y, Bennett M. Severe vitamin B12 deficiency in an infant associated with a maternal deficiency and a strict vegetarian diet. J Pediatr Hematol Oncol 2004; 26:270-271. Obeid R, Geisel J, Schorr H, Hubner U, Herrmann W. The impact of vegetarianism on some haematological parameters. Eur J Haematol 2002;69:275-279. Roschitz B, Plecko B, Huemer M, Biebl A, Foerster H, Sperl W. Nutritional infantile vitamin B12 deficiency: pathobiochemical considerations in seven patients. Arch Dis Child Fetal Neonatal Ed 2005;90:F281-F282. van Dusseldorp M, Schneede J, Refsum H, Ueland PM, Thomas CM, de Boer E et al. Risk of persistent cobalamin deficiency in adolescents fed a macrobiotic diet in early life. Am J Clin Nutr 1999;69:664-671.
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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏
CASE REPORT
¢ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· Û ¤ÊË‚Ô 1 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ™ËÙ›·˜, ∫Ú‹ÙË 2 ¶Ó¢ÌÔÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ™ËÙ›·˜, ∫Ú‹ÙË 3 ∞ÎÙÈÓÔÏÔÁÈÎfi ∆Ì‹Ì·, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ™ËÙ›·˜, ∫Ú‹ÙË AÏÏËÏÔÁÚ·Ê›·: £ÂÔÓ‡ÌÊË ∆·‚Ï·‰¿ÎË efi.tavladaki@mail.gr ∂ıÓÈ΋˜ ∞ÓÙ›ÛÙ·Û˘ 9 ∆.∫. 724 00, ¡Â¿ÔÏË §·ÛÈı›Ô˘ ∫Ú‹Ù˘
£. ∆·‚Ï·‰¿ÎË1, ª. ª·Ï·ıÚ¿ÎË1, °. ¢Ú·ÎˆÓ¿Î˘2, π. ™·Úȉ¿Î˘3, π. ∫·ÙÛ·Ú¿Î˘1, ª. ∫Ï·‰›ÙÔ˘1, ∞. ∞˘ÁÂÓ¿ÎË1, ∞. ¡Â¿Ú¯Ô˘1 ¶ÂÚ›ÏË„Ë: ∏ ‰ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· Â›Ó·È ¤Ó· ȉȷ›ÙÂÚÔ Â›‰Ô˜ ÂÚÈÊÂÚÈ΋˜ Ó¢ÌÔÓÈ΋˜ Û‡ÌÙ˘Í˘, Ë ÔÔ›· ÌÈÌÂ›Ù·È ÓÂfiÏ·ÛÌ· ÛÙËÓ ÂÚÈʤÚÂÈ· ÙÔ˘ Ó‡ÌÔÓ· ‹ ÌÂÛÔıËÏ›ˆÌ·. ∞Ó·Ù‡ÛÛÂÙ·È ˆ˜ ·ÔÙ¤ÏÂÛÌ· ·ıÔÏÔÁÈ΋˜ ηٿÛÙ·Û˘ ÙÔ˘ ˘Â˙ˆÎfiÙ·. ™˘Ó·ÓÙ¿Ù·È Ù˘¯·›· Î·È Û¿ÓÈ· Û ·Û˘Ìو̷ÙÈÎfi ÂÓ‹ÏÈη Î·È ·ÎfiÌ· ÈÔ Û¿ÓÈ· Û ·È‰È¿. ¶·ÚfiÌÔȘ ÂÚÈÙÒÛÂȘ ‰ÂÓ ¤¯Ô˘Ó ·Ó·ÊÂÚı› ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·. ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ÂÊ‹‚Ô˘, ËÏÈΛ·˜ ‰ÂηÙÚÈÒÓ ÂÙÒÓ, Ì ‰ÂηÙÈ΋ ˘ÚÂÙÈ΋ ΛÓËÛË, Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹, ‰ÈÛÎÔÂȉ‹ ·ÙÂÏÂÎÙ·Û›· Î·È ÔÚȷο ‰ÈÔÁΈ̤ÓÔ ÂÚÈ‚ÚÔÁ¯ÈÎfi ÏÂÌÊ·‰¤Ó· ÛÙËÓ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜. √ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· Ê˘Ì·Ù›ˆÛË ‹Ù·Ó ·ÚÓËÙÈÎfi˜ ÁÈ· ÙÔÓ ›‰ÈÔ Î·È ÙÔ ÂÚÈ‚¿ÏÏÔÓ ÙÔ˘, ÂÓÒ Ô Ù›ÙÏÔ˜ ·ÓÙÈÛˆÌ¿ÙˆÓ ÁÈ· Ì˘ÎfiÏ·ÛÌ· (‰‡Ô ‰Â›ÁÌ·Ù·), ‹Ù·Ó Û˘Ì‚·Ùfi˜ Ì Ïԛ̈ÍË ·fi Ì˘ÎfiÏ·ÛÌ·. ∏ ‰ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· ÙÔ˘ ·ÛıÂÓÔ‡˜ ·Ô‰fiıËΠ۠ϢÚÈÙÈ΋ Û˘ÏÏÔÁ‹ Î·È ·˘Ù‹ ÛÂ Ì˘ÎÔÏ·ÛÌ·ÙÈ΋ Ó¢ÌÔÓ›·. §¤ÍÂȘ ÎÏÂȉȿ: ¢ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›·, Ì˘ÎÔÏ·ÛÌ·ÙÈ΋ Ó¢ÌÔÓ›·, ¤ÊË‚Ô˜.
Round atelectasis in adolescent 1 Paediatric Clinic, General Hospital of Sitia, Crete 2 Pneumonology Clinic, General Hospital of Sitia, Crete 3 Department of Radiology, General Hospital of Sitia, Crete Correspondence: Theonymfi Tavladaki efi.tavladaki@mail.gr 9, Ethnikis Antistasis St. 724 00 Neapoli, Lasithi, Crete
T. Tavladaki1, M. Malathraki1, G. Drakonakis2, I. Saridakis3, I. Katsarakis1, M. Kladitou1, A. Augenaki1, A. Nearchou1 Abstract: Round atelectasis is a special type of peripheral lung collapse that can mimic a peripheral lung tumour or a mesothelioma. It develops as a result of pleural disease. It is a rare incidental finding in asymptomatic adults and even rarer in children, for whom there are no published reports. The case of a thirteen year-old adolescent male is described, who presented with a low grade fever and the findings of pleural effusion, round atelectasis and a slightly enlarged peribronchial lymph node on thoracic computed tomography. The laboratory investigation for tuberculosis was negative for the patient and his environment; however antibody titers for mycoplasma (two samples) were positive. The round atelectasis was therefore attributed to pleural effusion due to mycoplasma pneumonia. Key words: Round atelectasis, mycoplasma pneumonia, adolescent.
∂ÈÛ·ÁˆÁ‹ ∏ ‰ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· (folded lung ‹ atelectatic pseudotumor ‹ Blesousky’s syndrome) Â›Ó·È ÌÈ· ·Û˘Ó‹ıÈÛÙË ÌÔÚÊ‹ ÂÚÈÊÂÚÈ΋˜ ·ÙÂÏÂÎÙ·Û›·˜. ∞Ó·ÊÔÚÈο Ì ÙËÓ ·ıÔÁ¤ÓÂÛ‹ Ù˘ ¤¯Ô˘Ó ·Ó·Ù˘¯ı› ‰‡Ô ‚·ÛÈΤ˜ ıˆڛ˜ (2,3). ™‡Ìʈӷ Ì ÙËÓ ÚÒÙË, Ë Â·ÓÂÈÏËÌ̤ÓË ¤ÎıÂÛË Û ·Ì›·ÓÙÔ ÚÔηÏ› ÌÈ· ÚÔԉ¢ÙÈ΋ ÈÓÒ‰Ë ·ÓÙ›‰Ú·ÛË ÙÔ˘ ˘Â˙ˆÎfiÙ·. ªÂ ÙËÓ ˆÚ›Ì·ÓÛË Ù˘ ·ÓÙ›‰Ú·Û˘ ·˘Ù‹˜, Ô ÈÓ҉˘ ÈÛÙfi˜ Û˘ÚÚÈÎÓÒÓÂÙ·È Î·È ˆı› ÙÌ‹Ì·Ù· ÙÔ˘ ˘ÔΛÌÂÓÔ˘ Ó‡ÌÔÓ· Û ۇÌÙ˘ÍË (2,3). ∏ ‰Â‡ÙÂÚË ıˆڛ· ÂÎÙÈÌ¿ fiÙÈ Ë ‰ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· ÌÔÚ› Ó· ·Ó·Ù˘¯ı› ÌÂÙ¿ ·fi ›ÂÛË Û ÌÈ· ÂÚÈÊÂÚÈ΋ ı¤ÛË ÙÔ˘ Ó‡ÌÔÓ· ÏfiÁˆ οÔÈ·˜ Ï¢ÚÈÙÈ΋˜ Û˘ÏÏÔÁ‹˜, Ì ·ÔÙ¤ÏÂÛÌ· ÙÔ Ó¢ÌÔÓÈÎfi ·Ú¤Á¯˘Ì· Ó· Û˘ÌÙ‡ÛÛÂÙ·È Î·È Ó· ÂÁÎψ‚›˙ÂÙ·È Ì¤Û· ÛÙË Û˘ÏÏÔÁ‹. ªÔÚ› Ó· ·Ú·Ì¤ÓÂÈ ÂÁÎψ‚ÈṲ̂ÓÔ, ÏfiPaediatriki 2007;70:60-63
Áˆ Ù˘ ·Ó¿Ù˘Í˘ Û˘ÌʇÛˆÓ, ÌÂÙ¿ ÙËÓ ˘Ô¯ÒÚËÛ‹ Ù˘ (1-3,7,8). ™˘Ó‹ıˆ˜ ÂÓÙÔ›˙ÂÙ·È ÛÙ· Ô›ÛıÈ· ÙÌ‹Ì·Ù· ÙˆÓ Î¿Ùˆ ÏÔ‚ÒÓ, ·ÏÏ¿ ÌÔÚ› Ó· ÂÓÙÔÈÛÙ› Î·È Û ¿ÏϘ ı¤ÛÂȘ ÙˆÓ Ó¢ÌfiÓˆÓ. ™˘¯ÓfiÙÂÚË Â›Ó·È Ë ÂÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË, ·Ó Î·È ¤¯Ô˘Ó ·Ó·ÊÂÚı› ·ÌÊÔÙÂÚfiÏ¢Ú˜ ÂÓÙÔ›ÛÂȘ (1,2,7). ™ÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ Ê·›ÓÂÙ·È ÛÙÚÔÁÁ˘Ï‹ ‹ ÛÊËÓÔÂȉ‹˜ ÛΛ·ÛË, ‰È·Ì¤ÙÚÔ˘ 47 cm, Ô˘ Û¯ËÌ·Ù›˙ÂÈ ÔÍ›˜ ÁˆÓ›Â˜ Ì ÙÔÓ ˘Â˙ˆÎfiÙ·. ™˘¯Ó¿ Û˘Ó˘¿Ú¯Ô˘Ó ˘Â˙ˆÎÔÙÈ΋ ¿¯˘ÓÛË Î·È Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹ (3,6). ™ÙËÓ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ‰È·ÎÚ›ÓÂÙ·È: ·) ÛÙÚÔÁÁ˘Ï‹ ‹ ÛÊËÓÔÂȉ‹˜ ‹ ÂÏÏÂÈ„ÔÂȉ‹˜ Ì¿˙· Ô˘ Û˘Ó¤¯ÂÙ·È Ì ÌÈ· ‰È¿¯˘ÙË ˘Â˙ˆÎÔÙÈ΋ ¿¯˘ÓÛË Ì ‹ ¯ˆÚ›˜ Û˘ÓÔ‰¤˜ ·ۂÂÛÙÒÛÂȘ, ‚) ‚ÚÔÁ¯Ô·ÁÁÂȷ΋ ‰ÔÌ‹ Ô˘ ÂÈÛ¤Ú¯ÂÙ·È ÛÙ· fiÚÈ· Ù˘ Ì¿˙·˜, ·Ó·Ê¤ÚÂÙ·È ˆ˜
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¢ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· Û ¤ÊË‚Ô
“ÛËÌÂ›Ô Ô˘Ú¿˜ ÎÔÌ‹ÙË” Î·È ·ÔÙÂÏ› ¯·Ú·ÎÙËÚÈÛÙÈÎfi ‡ÚËÌ·, Á) ·ÂÚÔ‚ÚÔÁ¯fiÁÚ·ÌÌ· ‹ ·ÎÙÈÓԉȷʷÓ›˜ ÎÂÓÙÚÈΤ˜ ÂÚÈÔ¯¤˜ Ô˘ Èı·ÓfiÓ ÂÚȤ¯ÔÓÙ·È Ì¤Û· ÛÙË Ì¿˙·, ÏfiÁˆ ˘ÔÏÂÈÌÌ·ÙÈÎÔ‡ ·ÂÚÈÛÌÔ‡ ÙÔ˘ ·ÙÂÏÂÎÙ·ÙÈÎÔ‡ Ó¢ÌÔÓÈÎÔ‡ ÈÛÙÔ‡ Î·È ‰) ·Ó·ÏËڈ̷ÙÈÎfi˜ ˘ÂÚ·ÂÚÈÛÌfi˜ ÙÔ˘ ·Ú·Î›ÌÂÓÔ˘ Ó¢ÌÔÓÈÎÔ‡ ÈÛÙÔ‡ (4-6). À¿Ú¯ÂÈ ÏËıÒÚ· ·ÎÙÈÓÔÏÔÁÈÎÒÓ ·Ú·ÏÏ·ÁÒÓ fiÛÔÓ ·ÊÔÚ¿ ÛÙÔ Ì¤ÁÂıÔ˜, ÙÔ Û¯‹Ì·, ÙË ı¤ÛË Î·È ÙË ÌÔÚÊÔÏÔÁ›·. ™˘Ó‹ıˆ˜ ·Ú·Ì¤ÓÂÈ ÛÙ¿ÛÈÌË, ·ÏÏ¿ ÌÔÚ› Ó· ˘ÔÛÙÚ·Ê› ‹ Î·È Ó· ÂÍ·ÏÂÈÊı› Ï‹Úˆ˜. ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÂÈ ÙÔ ÌÂÛÔıËÏ›ˆÌ· Î·È ÙÔ ‚ÚÔÁ¯ÔÁÂÓ¤˜ ηÚΛӈ̷. ∏ ‰È¿ÁÓˆÛË ı· ÛÙËÚȯı› ÛÙÔ˘˜ ȉȷ›ÙÂÚÔ˘˜ ·ÎÙÈÓÔÏÔÁÈÎÔ‡˜ ¯·Ú·ÎÙ‹Ú˜ Ù˘ ‰ÈÛÎÔÂȉԇ˜ ·ÙÂÏÂÎÙ·Û›·˜, ÛÙË Û˘ÓÔÏÈ΋ ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ÂÈÎfiÓ· Î·È ÛÙËÓ Â·ÓÂÎÙ›ÌËÛË. ™Â ·ÌÊ›‚ÔϘ ÂÚÈÙÒÛÂȘ ÌÔÚ› Ó· ··ÈÙËı› Î·È ‚ÈÔ„›·.
¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ÂÊ‹‚Ô˘ ËÏÈΛ·˜ 13 ÂÙÒÓ, Ô ÔÔ›Ô˜ - 30 Ë̤Ú˜ ÚÈÓ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘ ·ÚÔ˘Û›·Û ‰‡Ô ÂÂÈÛfi‰È· ÔͤԘ ¿ÏÁÔ˘˜ ÛÙÔ ÚfiÛıÈÔ Î·È Ô›ÛıÈÔ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ Î·È ¤Ó· ˘ÚÂÙÈÎfi ·̷ ¤ˆ˜ 38ÔC. ∆· Û˘ÌÙÒÌ·Ù· ·Ô‰fiıËÎ·Ó Û ÈÔÁÂÓ‹ Ïԛ̈ÍË. ∂›ÎÔÛÈ Ë̤Ú˜ ·ÚÁfiÙÂÚ· ·ÚÔ˘Û›·Û ‰ÂηÙÈ΋ ˘ÚÂÙÈ΋ ΛÓËÛË Î·Ù¿ ÙȘ ·ÔÁÂ˘Ì·ÙÈÓ¤˜ Î·È ‚Ú·‰ÈÓ¤˜ ÒÚ˜. ∞fi ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË ÙˆÓ Û˘ÛÙËÌ¿ÙˆÓ ·ÚÔ˘Û›·Û Ì›ˆÛË ·Ó·Ó¢ÛÙÈÎÔ‡ „Èı˘Ú›ÛÌ·ÙÔ˜ ÛÙÔ ·ÚÈÛÙÂÚfi ̤ÛÔ Ó¢ÌÔÓÈÎfi ‰›Ô. ∞fi ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È ‚ÚÔÁ¯ÈÎfi ¿ÛıÌ· ·fi ÙË ÌËÙÚÈ΋ ÁÈ·ÁÈ¿, ÂÓÒ ·fi ÙÔ ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È ÚÔˆÚfiÙËÙ· (34 ‚‰ÔÌ¿‰ˆÓ ·ËÛË, 1900 g ‚¿ÚÔ˜ Á¤ÓÓËÛ˘) Î·È ÁÈ· ÙÔÓ ÏfiÁÔ ·˘Ùfi ÓÔÛËχÙËΠ۠ÙÌ‹Ì· ÚÔÒÚˆÓ. ™Â ‚ÚÂÊÈ΋ ËÏÈΛ· ÓÔÛËχÙËΠÁÈ· ‚ÚÔÁ¯ÈÔÏ›Ùȉ· Î·È ÛÙË Û˘Ó¤¯ÂÈ· ›¯Â ‹È˜ ÎÚ›ÛÂȘ ‚ÚÔÁ¯ÈÎÔ‡ ¿ÛıÌ·ÙÔ˜. ∆· Â˘Ú‹Ì·Ù· ·fi ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ηٿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ‹Ù·Ó Ù· ·ÎfiÏÔ˘ı·: ™ÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ·Ú·ÙËÚ‹ıËΠϢÚÈÙÈ΋ Û˘ÏÏÔÁ‹ ·ÚÈÛÙÂÚÔ‡ οو ÏÔ‚Ô‡ (∂ÈÎfiÓ· 1). °ÂÓÈ΋ ·›Ì·ÙÔ˜: §Â˘Î¿: 10.000 Îί (¶: 27%, §ÂÌÊ: 62%, ª: 7%), Ht: 40%, Hb: 13,2 g%, Plt: 366.000. µÈÔ¯ËÌÈο: O˘Ú›·: 32 mg%, ∫Ú·ÙÈÓ›ÓË: 1 mg%, SGOT: 29, SGPT: 14, ALP: 454, LDH: 375, ™¿Î¯·ÚÔ: 97 mg%. ∏ ∆∫∂ ‹Ù·Ó 55 mm/1 h. CRP: 45 mg/l. WIDAL: (-), WRIGHT: (-). Mantoux: 15 mm (ÂÌ‚ÔÏÈ·ÛÌfi˜ Ì µCG ÚÔ 18Ì‹ÓÔ˘). ∆· Â˘Ú‹Ì·Ù· ·fi ÙË ÁÂÓÈ΋ Ô‡ÚˆÓ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο Î·È Ë Î·ÏÏȤÚÁÂÈ· Ô‡ÚˆÓ ÁÈ· µ. Koch (3 ‰Â›ÁÌ·Ù·) ‹Ù·Ó ·ÚÓËÙÈ΋. °·ÛÙÚÈÎfi ˘ÁÚfi (¿ÌÂÛÔ Î·È Î·ÏÏÈÂÚÁÂÈ·, 3 ‰Â›ÁÌ·Ù·): ·ÚÓËÙÈ΋. ∆Ô ˘ÁÚfi ·Ú·Î¤ÓÙËÛ˘ ıÒÚ·ÎÔ˜ ‹Ù·Ó ÔÚÔ·ÈÌ·-
∂ÈÎfiÓ· 1. ∞ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ηٿ ÙËÓ ÂÈÛ·ÁˆÁ‹. ¶Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹ ·ÚÈÛÙÂÚ¿.
ÙËÚfi, Ì ÔÚÚÒ‰Ë ¯ÚÔÈ¿ Î·È ‰È·˘Á‹ fi„Ë Ì ›˙ËÌ·. ∞fi ÙË ÁÂÓÈ΋ ÂͤٷÛË: ∫‡ÙÙ·Ú·: WBC: 7400/Îί (¶: 50%, §: 47%, ª: 3%), RBC: 15000, ™¿Î¯·ÚÔ: 115 mg/dl, §Â‡ÎˆÌ·: 4,9 g/dl, LDH: 1605 IU/L, ∞Ì˘Ï¿ÛË: 29 u/l. ∏ ηÏÏȤÚÁÂÈ· ÁÈ· ÎÔÈÓ¿ ÌÈÎÚfi‚È· ‹Ù·Ó ÛÙ›ڷ. ∞fi ÙËÓ Î˘ÙÙ·ÚÔÏÔÁÈ΋ ÂͤٷÛË ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÓÂÔÏ·ÛÌ·ÙÈο ·ÙÙ·Ú·, Ô‡ÙÂ Ê˘Ì¿ÙÈ·, ÂÓÒ ÛËÌÂÈÒÓÂÙ·È Ë ·ÚÔ˘Û›· ˈÛÈÓÔÊ›ÏˆÓ ÛÙÔ Â›¯ÚÈÛÌ·. ∆Ô U/S ηډȿ˜ ¤‰ÂÈÍ ÂχıÂÚÔ ÂÚÈοډÈÔ. ∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ¤‰ÂÈÍ ˘fi˘ÎÓË ˘Â˙ˆÎÔÙÈ΋ Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡ ηٿ ÙË Ú·¯È·›· ·Ú·ÛÔÓ‰˘ÏÈ΋ Î·È ¤Íˆ Ï¿ÁÈ· ÂÈÊ¿ÓÂÈ· ÙÔ˘ ηÙÒÙÂÚÔ˘ ·ÚÈÛÙÂÚÔ‡ ËÌÈıˆÚ·Î›Ô˘ ÌÂ Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ηÙÒÙÂÚÔ˘ ÙÌ‹Ì·ÙÔ˜ Ù˘ Û‡ÛÙÔÈ¯Ô˘ Ì›˙ÔÓÔ˜ ÌÂÛÔÏÔ‚›Ô˘ Û¯ÈÛÌ‹˜. ™ÙÔ ¤Íˆ ‚·ÛÈÎfi ÙÌ‹Ì· ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ οو Ó¢ÌÔÓÈÎÔ‡ ÏÔ‚Ô‡ ·Ó·‰ÂÈÎÓ‡ÂÙ·È ÛÊËÓÔÂȉԇ˜ Û¯‹Ì·ÙÔ˜ ÌÈÎÚ‹ ·ÚÂÁ¯˘Ì·ÙÈ΋ ‚Ï¿‚Ë, Ë ÔÔ›· ÏËÚÔ› ÔÏÏ¿ ÂÎ ÙˆÓ ·ÂÈÎÔÓÈÛÙÈÎÒÓ ÎÚÈÙËÚ›ˆÓ ÌÈÎÚ‹˜ ‰ÈÛÎÔÂȉԇ˜ ·ÙÂÏÂÎÙ·Û›·˜, ÂÓÙfi˜ ÙˆÓ ÔÚ›ˆÓ Ù˘ ÔÔ›·˜ ·Ó·‰ÂÈÎÓ‡ÔÓÙ·È ÌfiÏȘ ˘ÔÛËÌ·ÈÓfiÌÂÓ˜ ÛˆÏËÓÔÂȉ›˜ ‰Ô̤˜ Ì ·¤Ú· Ô˘ ·ÓÙÈÚÔÛˆÂ‡Ô˘Ó ÔÚȷο ‰È·ÙÂٷ̤ÓÔ˘˜ ‚ÚfiÁ¯Ô˘˜ (∂ÈÎfiÓ˜ 2 Î·È 3). ∂Î ÙÔ˘ ÏÔÈÔ‡ ÂϤÁ¯Ô˘ ·Ó·Ê¤ÚÂÙ·È Ë ·ÚÔ˘Û›· ÌÈÎÚÔ‡ ˘ÔÛÙÚfiÁÁ˘ÏÔ˘ ÌÔÚÊÒÌ·ÙÔ˜ ‰È·Ì¤ÙÚÔ˘ 1,2 cm ÛÙÔ ·ÚÈÛÙÂÚfi ¯Â›ÏÔ˜ ÙÔ˘ Ô›ÛıÈÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘ ‰›Ï· ·fi ÙÔÓ ÔÈÛÔÊ¿ÁÔ. ∂›Û˘, ‰›‰ÂÙ·È Ë ÂÓÙ‡ˆÛË ÔÚȷ΋˜ ·ÔÛÙÚÔÁÁ‡ÏˆÛ˘ Ù˘ ·ÚÈÛÙÂÚ‹˜ Ó¢ÌÔÓÈ΋˜ ‡Ï˘, ÛÙÔ ‡„Ô˜ Ù˘ ·ÚÈÛÙÂÚ‹˜ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜. ∆· ÙÂÏÂ˘Ù·›· Â˘Ú‹Ì·Ù· ¤ıÂÛ·Ó ÙËÓ ˘fiÓÔÈ· ÌÈÎÚÔ‡ ÌÂÛÔıˆÚ·ÎÈÎÔ‡ Î·È Èı·ÓÒ˜ ÔÚȷο ‰ÈÔÁΈ̤ÓÔ˘ ·ÚÈÛÙÂÚÔ‡ ÂÚÈ‚ÚÔÁ¯ÈÎÔ‡ ÏÂÌÊ·‰¤Ó·. √˘‰¤Ó ·ıÔÏÔÁÈÎfi ·Ó¢ڤıË ·fi ÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ˘fiÏÔÈˆÓ Ó¢ÌÔÓÈÎÒÓ Â‰›ˆÓ. ¶·È‰È·ÙÚÈ΋ 2007;70:60-63
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£. ∆·‚Ï·‰¿ÎË Î·È Û˘Ó.
∂ÈÎfiÓ· 2. ∞ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ηٿ ÙËÓ ÂÈÛ·ÁˆÁ‹. ™ÊËÓÔÂȉԇ˜ Û¯‹Ì·ÙÔ˜ ‰ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›·. ™˘Ó¯È˙fiÌÂÓË Ì ÙÔÓ ˘Â˙ˆÎfiÙ·, Û˘Ó˘¿Ú¯ÂÈ ˘Â˙ˆÎÔÙÈ΋ ¿¯˘ÓÛË Î·È Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹.
√ ·ÛıÂÓ‹˜ ·ÓÙÈÌÂÙˆ›ÛÙËΠ̠ÎÔÚÙÈ˙fiÓË Î·È ·ÓÙÈÊ˘Ì·ÙÈ΋ ·ÁˆÁ‹ ̤¯ÚÈ Ó· ÔÏÔÎÏËÚˆı› Ô ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· ÙÔÓ ›‰ÈÔ Î·È ÙÔ ÂÚÈ‚¿ÏÏÔÓ ÙÔ˘ Î·È ÛÙË Û˘Ó¤¯ÂÈ· ¤Ï·‚ ̷ÎÚÔÏ›‰Ë (15 mg/kg/24h) Û ‰‡Ô ‰fiÛÂȘ ÁÈ· 15 Ë̤Ú˜. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘ ·Ú¤ÌÂÈÓ Û ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË, Û˘Ó¤¯È˙ fï˜ ‰ÂηÙÈ΋ ˘ÚÂÙÈ΋ ΛÓËÛË ÁÈ· ¤ÓÙ Ë̤Ú˜ ηٿ ÙȘ ·ÔÁÂ˘Ì·ÙÈÓ¤˜ ÒÚ˜. ªÂÙ¿ ÙËÓ ¤Ó·ÚÍË ıÂڷ›·˜, ˘‹ÚÍ ·ÎÚÔ·ÛÙÈÎÒ˜ ÚÔԉ¢ÙÈ΋ ‚ÂÏÙ›ˆÛË ÙˆÓ Â˘ÚËÌ¿ÙˆÓ ·fi ÙÔÓ ıÒڷη, ÂÓÒ ·ÔηٿÛÙ·ÛË ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ „Èı˘Ú›ÛÌ·ÙÔ˜ ˘‹ÚÍ ÌÂÙ¿ ÙË ‰¤Î·ÙË Ë̤ڷ Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘. √ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ ÙÔ˘ ÂÊ‹‚Ô˘ ÁÈ· Ê˘Ì·Ù›ˆÛË ‹Ù·Ó ·ÚÓËÙÈÎfi˜, ÂÓÒ ÙÔ ÚÒÙÔ ‰Â›ÁÌ· ÈÔÏÔÁÈÎÔ‡ ÂϤÁ¯Ô˘ Ê·›ÓÂÙ·È ÛÙÔÓ ¶›Ó·Î· 1. √ ¤ÊË‚Ô˜ Û˘Ó¤¯ÈÛ ӷ ·Ú·ÎÔÏÔ˘ıÂ›Ù·È ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· Ù˘ ÎÏÈÓÈ΋˜. ŒÏ·‚ ·ÓÙÈÊ˘Ì·ÙÈ΋ ·ÁˆÁ‹ ÁÈ· Û˘ÓÔÏÈÎfi ‰È¿ÛÙËÌ· 20 ËÌÂÚÒÓ. ∏ ÂÍ·Ê¿ÓÈÛË Ù˘ ·ÙÂÏÂÎÙ·Û›·˜ ‰È·ÈÛÙÒıËΠ·ÎÙÈÓÔÏÔÁÈο ‰‡Ô Ì‹Ó˜ ÌÂÙ¿ ÙË ‰È¿ÁÓˆÛ‹ Ù˘ Î·È ¤ÎÙÔÙÂ Ô ¤ÊË‚Ô˜ ·Ú·Ì¤ÓÂÈ Û ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË. ∏ Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹ ·Ô-
∂ÈÎfiÓ· 3. ∞ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ηٿ ÙËÓ ÂÈÛ·ÁˆÁ‹. ™Ù· fiÚÈ· Ù˘ ·ÏÏÔ›ˆÛ˘ ·Ó·‰ÂÈÎÓ‡ÔÓÙ·È ÔÚȷο ‰È·ÙÂٷ̤ÓÔÈ ‚ÚfiÁ¯ÔÈ. ¢È·Ê·›ÓÂÙ·È Ë ¯·Ú·ÎÙËÚÈÛÙÈ΋ “Ô˘Ú¿ ÎÔÌ‹ÙË”.
‰fiıËΠ۠ӢÌÔÓ›· ·fi Ì˘ÎfiÏ·ÛÌ· Î·È Ë ‰ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· ÛÙËÓ ‡·ÚÍË ÂÍȉÚÒÌ·ÙÔ˜.
™˘˙‹ÙËÛË ∏ ‰ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· Â›Ó·È Ì›· ·Û˘Ó‹ıÈÛÙË ÌÔÚÊ‹ ÂÚÈÊÂÚÈ΋˜ ·ÙÂÏÂÎÙ·Û›·˜. ∞Ó·Ù‡ÛÛÂÙ·È ˆ˜ ·ÔÙ¤ÏÂÛÌ· ·ıÔÏÔÁÈ΋˜ ηٿÛÙ·Û˘ ÙÔ˘ ˘Â˙ˆÎfiÙ·. √È ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ ¤¯Ô˘Ó ·Ô‰Ôı› Û ÂÈÛÓÔ‹ ·ÌÈ¿ÓÙÔ˘, ·ÏÏ¿ ¤¯Ô˘Ó ·Ó·ÊÂÚı› Î·È ÔÏϤ˜ ¿ÏϘ ·Èٛ˜, fiˆ˜ Ó¢ÌÔÓÈ΋ Û˘ÏÏÔÁ‹ (ÂÍ›‰ÚˆÌ· ‹ ‰È›‰ÚˆÌ·) Î·È ÙÚ·‡Ì·. ∏ ‰ÈÛÎÔÂȉ‹˜ ·ÙÂÏÂÎÙ·Û›· ÌÔÚ› Ó· ‰È·ÁÓˆÛÙ› ·fi ÏËıÒÚ· ·ÎÙÈÓÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ. ™ÙËÓ ÂÚ›ÙˆÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÙ·È, ˘¿Ú¯Ô˘Ó ÔÏÏ¿ ·fi Ù· ÎÚÈÙ‹ÚÈ· Ù˘ ‰ÈÛÎÔÂȉԇ˜ ·ÙÂÏÂÎÙ·Û›·˜, fiÛÔÓ ·ÊÔÚ¿: ·) ÛÙËÓ ÂÓÙfiÈÛË: Â›Ó·È Û˘Ó‹ıˆ˜ ÂÙÂÚfiÏ¢ÚË Î·È Î·Ù·Ï·Ì‚¿ÓÂÈ Ù· Ô›ÛıÈ· ÙÌ‹Ì·Ù· ÙˆÓ Î¿Ùˆ ÏÔ‚ÒÓ, ‚) ÛÙËÓ ·ÈÙÈÔÏÔÁ›·: Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹, fiˆ˜ Ê·›ÓÂÙ·È ÛÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ·ÚÈÛÙÂÚ¿ Î·È Á) ÛÙÔ Û¯‹Ì·: ÛÊËÓÔÂȉ‹˜ Ì¿˙·, fiˆ˜ Ê·›ÓÂÙ·È ÛÙËÓ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· Ô˘ Û˘Ó¤¯ÂÙ·È Ì ÌÈ· ‰È¿¯˘ÙË ˘Â˙ˆÎÔÙÈ΋ ¿¯˘ÓÛË.
¶›Ó·Î·˜ 1. ¶ÚÒÙÔ ‰Â›ÁÌ· ÈÔÏÔÁÈÎÔ‡ ÂϤÁ¯Ô˘ Echo (1Ô ‰Â›ÁÌ·) Coxsakie Mycoplasm (1Ô ‰Â›ÁÌ·) Chlamydia Mycoplasm (2Ô ‰Â›ÁÌ·-2 ‚‰ÔÌ¿‰Â˜ ·ÚÁfiÙÂÚ·) Echo (2Ô ‰Â›ÁÌ·) Paediatriki 2007;70:60-63
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∂›Û˘, ÛÙËÓ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ·ÂÈÎÔÓ›˙ÂÙ·È Ë ‚ÚÔÁ¯Ô·ÁÁÂȷ΋ ‰ÔÌ‹ Ô˘ ÂÈÛ¤Ú¯ÂÙ·È ÛÙ· fiÚÈ· Ù˘ Ì¿˙·˜ Î·È ·Ó·Ê¤ÚÂÙ·È ˆ˜ “ÛËÌÂ›Ô Ô˘Ú¿˜ ÎÔÌ‹ÙË”, ÙÔ ÔÔ›Ô ·ÔÙÂÏ› ¯·Ú·ÎÙËÚÈÛÙÈÎfi ‡ÚËÌ· Ù˘ ‰ÈÛÎÔÂȉԇ˜ ·ÙÂÏÂÎÙ·Û›·˜. ∆· Ì˘ÎÔÏ¿ÛÌ·Ù· Â›Ó·È ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ›, ÔÚÈṲ̂ӷ ›‰Ë ÂÎ ÙˆÓ ÔÔ›ˆÓ Â›Ó·È ·ıÔÁfiÓ· ÁÈ· ÙÔÓ ¿ÓıÚˆÔ. ∫ÏÈÓÈο Û˘Ó‹ıˆ˜ ÚÔ‚¿ÏÏÔ˘Ó ˆ˜ Ïԛ̈ÍË ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ‹ ˆ˜ Ó¢ÌÔÓ›·. ∏ Ó¢ÌÔÓ›· ÂÌÊ·Ó›˙ÂÙ·È Î˘Ú›ˆ˜ Û ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜. ∏ Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹ Î·È Ë ·ıÔÏÔÁÈ΋ ηٿÛÙ·ÛË ÙÔ˘ ˘Â˙ˆÎfiÙ· ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ó¿Ù˘ÍË ‰ÈÛÎÔÂȉԇ˜ ·ÙÂÏÂÎÙ·Û›·˜, fiˆ˜ Ê·›ÓÂÙ·È ÛÙËÓ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜.
µÈ‚ÏÈÔÁÚ·Ê›· 1. Szydlowski GW, Cohn HE, Steiner RM, Edie RN. Rounded atelectasis: a pulmonary pseudotumor. Ann Thorac Surg 1992;53:817-821. 2. Hanke R, Kretzschmar R. Round atelectasis. Semin Roentgenol 1980;15:174-182. 3. Fraser R, Muller N, Colman N, Pare P. Fraser and Pare’s diagnosis of diseases of the chest. 4th edition. Philadelphia, PA: WB Saunders Company; 1999. p. 513-522. 4. Doyle TC, Lawler GA. CT features of rounded atelectasis of the lung. Am J Roentgenol 1984;143:225-228. 5. McHugh K, Blaquiere RM. CT features of rounded atelectasis. Am J Roentgenol 1989;153:257-260. 6. Roach HD, Davies GJ, Attanoos R, Crane M, Adams H, Phillips S. Asbestos: when the dust settles an imaging review of asbestos-related disease. Radiographics 2002;22: 167-184. 7. Medford ARL, Darby M, Maskell NA. Rounded atelectasis mimicking a lung cancer. J R Col Physicians Edinb 2005;35: 123-125. 8. Woodring Jh. Pleural effusion is a cause of round atelectasis of the lung. J Ky Med Assoc 2000;98:527-532.
¶·È‰È·ÙÚÈ΋ 2007;70:60-63
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¶ÂÚ›ÏË„Ë: ø˜ ÛÔ‚·Ú‹ ·Ú·ÙÂÈÓfiÌÂÓË ‰È¿ÚÚÔÈ· ıˆÚԇ̠οı ‰È·ÚÚÔ˚Îfi ÂÂÈÛfi‰ÈÔ ÙÔ ÔÔ›Ô ‰È·ÚΛ ÂÚÈÛÛfiÙÂÚÔ ·fi 15 Ë̤Ú˜ Î·È Â›Ó·È ·ÚÎÔ‡ÓÙˆ˜ ÛÔ‚·Úfi ÒÛÙ ӷ ··ÈÙ› ıÂڷ›· Ì ·ÚÂÓÙÂÚÈ΋ ‰È·ÙÚÔÊ‹. ¶ÚfiÎÂÈÙ·È ÁÈ· ‚·ÚÈ¿ ¿ıËÛË Ù˘ ÔÔ›·˜ Ë ıÓËÙfiÙËÙ· Î˘Ì·›ÓÂÙ·È ÌÂٷ͇ 5-47%. §ÔÈÌÒ‰ÂȘ ·Ú¿ÁÔÓÙ˜ Î·È ÙÚÔÊÈΤ˜ ·ÏÏÂÚÁ›Â˜ ·ÔÙÂÏÔ‡Ó ÙȘ ÈÔ Û˘¯Ó¤˜ ·Èٛ˜ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ¶ÚÔÁÓˆÛÙÈο ÛËÌ›· Ô˘ ÚԉȷÁÚ¿ÊÔ˘Ó Î·Î‹ ¤Î‚·ÛË, ·ÔÙÂÏÔ‡Ó Ù· ·ÎfiÏÔ˘ı·: ¯·ÌËÏfi ‚¿ÚÔ˜ Á¤ÓÓËÛ˘, ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi, ÚÒÈÌË ¤Ó·ÚÍË, ÎÔÈÓˆÓÈ΋ Ù¿ÍË, Î·È ÌË Ï‹„Ë ÌËÙÚÈÎÔ‡ Á¿Ï·ÎÙÔ˜. ∏ ÂÈÛ·ÁˆÁ‹ Ó¤ˆÓ ‰È·ÁÓˆÛÙÈÎÒÓ ÌÂıfi‰ˆÓ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ¤ÙÚ„ ÙË ‰È¿ÁÓˆÛË Ó¤ˆÓ ·ı‹ÛÂˆÓ ÔÈ Ôԛ˜ ÚÔηÏÔ‡Ó ·Ú·ÙÂÈÓfiÌÂÓË ‰È¿ÚÚÔÈ·, fiˆ˜ Â›Ó·È Ë Û˘ÁÁÂÓ‹˜ „¢‰Ô·fiÊÚ·ÍË, Ë ÓfiÛÔ˜ ÙˆÓ ÌÈÎÚÔÏ·¯ÓÈ·ÎÒÓ ÂÁÎÏ›ÛÙˆÓ, Ë ÂÓÙÂÚÔ¿ıÂÈ· Ì ı˘Û¿ÓÔ˘˜, Ë ·˘ÙÔ¿ÓÔÛË ÂÓÙÂÚÔ¿ıÂÈ·, ÙÔ Û‡Ó‰ÚÔÌÔ ·ÓÔÛÔÏÔÁÈ΋˜ ‰˘ÛÚ˘ıÌ›·˜, ÔÏ˘ÂÓ‰ÔÎÚÈÓÔ¿ıÂÈ·˜, ÂÓÙÂÚÔ¿ıÂÈ·˜ Î·È Ê˘ÏÔÛ‡Ó‰ÂÙ˘ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜ (IPEX), Ë ÂÈ̤ÓÔ˘Û· ÂÏÎ҉˘ ÎÔÏ›Ùȉ·, ÔÈ ‰È·Ù·Ú·¯¤˜ ÁÏ˘ÎÔ˙˘Ï›ˆÛ˘, ηıÒ˜ Î·È ÂÓÙÂÚÔ¿ıÂȘ ·fi Ó¤· ÏÔÈÌÒ‰Ë ·›ÙÈ·. √È Ó¤Â˜ ·˘Ù¤˜ ·ı‹ÛÂȘ ·ÔÙÂÏÔ‡Ó ÙÔ ·ÓÙÈΛÌÂÓÔ Ù˘ ·ÚÔ‡Û·˜ ·Ó·ÛÎfiËÛ˘. ∞Ó Î·È ÁÈ· ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ·fi ·˘Ù¤˜, ÂÎÙfi˜ ·fi ÙȘ ÏÔÈÌÒ‰ÂȘ, ‰ÂÓ ˘¿Ú¯ÂÈ ÂȉÈ΋ ıÂڷ›·, Ë ÚÒÈÌË ‰È¿ÁÓˆÛË Ô‰ËÁ› Û ηχÙÂÚË ÂÎÙ›ÌËÛË Ù˘ ÚfiÁÓˆÛ˘ Î·È ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ÁÈ· ÙËÓ ·ÚÔ¯‹ ÁÂÓÂÙÈÎÒÓ Û˘Ì‚Ô˘ÏÒÓ ÛÙËÓ ÔÈÎÔÁ¤ÓÂÈ·. Àfi ‰ÔÎÈÌ‹ ‚Ú›ÛÎÔÓÙ·È Û‹ÌÂÚ· Ó¤· ıÂڷ¢ÙÈο ̤۷ ˘fi ÌÔÚÊ‹ ·˘ÍËÙÈÎÒÓ, ‚ÈÔÏÔÁÈÎÒÓ Î·È ·ÓÙÈÊÏÂÁÌÔÓˆ‰ÒÓ ·Ú·ÁfiÓÙˆÓ Î·ıÒ˜ Î·È ıÚÂÙÈÎÒÓ Ô˘ÛÈÒÓ.
§¤ÍÂȘ ÎÏÂȉȿ: ÃÚfiÓÈ· ‰È¿ÚÚÔÈ·, ÂÓÙÂÚÔ¿ıÂÈ·, ÓÂÔÁÓfi.
Severe protracted diarrhÔea of infancy - New aspects Department of Neonatal Medicine, Alexandra Regional General Hospital, Athens, Greece Correspondence: C. Costalos ccostalos@yahoo.gr 130, Artemidos St., 175 62 P. Faliro, Athens, Greece
C. Costalos Abstract: Severe protracted diarrhoea of infancy is defined as diarrhoea lasting for more than 15 days, severe enough to necessitate parenteral nutrition. It is a serious disease with a mortality rate ranging from 5 to 47%. Infectious agents and trophic allergies account for most of the cases. Poor prognostic factors include low birth weight, positive family history, early onset, atopy, low social class and absence of breast feeding. The availability of novel diagnostic tools in recent years has expanded understanding of the aetiology and pathophysiology of protracted diarrhoea, resulting in the identification of a number of new diarrhoeal disease entities, which are discussed in this review. These include congenital pseudoobstruction, familial microvillus inclusion disease, tufting enteropathy, autoimmune enteropathy, immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome (IPEX) and persistent ulcerative colitis, as well as disorders of glucoprotein glycosylation. New infective enteropathogens have also been identified. Although definitive treatment for severe protracted diarrhoea is available only in the case of infectious agents, early identification of these diseases permits improved counseling of parents about the long-term prognosis and therapeutic options, and allows better assessment of the potential risk for subsequent affected siblings. New forms of management are currently under way, with the use of enteral growth factors, biologic and anti-inflammatory agents and nutrients.
Key words: Enteropathies, chronic intractable diarrhoea, newborn infant.
∂ÈÛ·ÁˆÁ‹ ø˜ ·Ú·ÙÂÈÓfiÌÂÓË ÛÔ‚·Ú‹ ‰È¿ÚÚÔÈ· (¶™¢) ÔÚ›˙ÂÙ·È Ë ‰È¿ÚÚÔÈ· Ë ÔÔ›· ·Ú·Ù›ÓÂÙ·È ¤Ú·Ó ÙˆÓ 15 ËÌÂÚÒÓ Î·È Â›Ó·È ·ÚÎÔ‡ÓÙˆ˜ ÛÔ‚·Ú‹ ÒÛÙ ӷ ··ÈÙ› ÂȉÈ΋ ‰È·ÈÙËÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Î·È Û˘¯Ó¿ ·ÚÂÓÙÂÚÈ΋ ‰È·ÙÚÔÊ‹ (1). ∆Ô ı¤Ì· ¤¯ÂÈ ··Û¯ÔÏ‹ÛÂÈ ÙÔ˘˜ ÂȉÈÎÔ‡˜ ·fi ·ÏÈ¿. √È ÛËÌ·ÓÙÈΤ˜ ÚfiÔ‰ÔÈ Ô˘ ¤ÁÈÓ·Ó Ù· ÙÂÏÂ˘Ù·›· Paediatriki 2007;70:64-69
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Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ˆ˜ ÚÔ˜ ÙËÓ ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ Î·È ÙÔÓ Î›Ó‰˘ÓÔ ÂÌÊ¿ÓÈÛ‹˜ Ù˘ Û ÌÂÙ·ÁÂÓ¤ÛÙÂÚË Î‡ËÛË. ∞Ó Î·È Ë Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ¶™¢ ‰ÂÓ ÍÂÂÚÓ¿ÂÈ ÙË 1 ÂÚ›ÙˆÛË/100.000 ÁÂÓÓ‹ÛÂȘ (2), Ë ıÓËÙfiÙËÙ¿ Ù˘ Â›Ó·È Ôχ ˘„ËÏ‹ (5-47%) (1) Î·È Ë ÔÈÎÔÓÔÌÈ΋ ÂÈ‚¿Ú˘ÓÛË ÙÂÚ¿ÛÙÈ·. §fiÁˆ ÙÔ˘ ÌÂÁ¿ÏÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ·ı‹ÛÂˆÓ ÔÈ Ôԛ˜ ÚÔηÏÔ‡Ó ¶™¢, Ë ·Ó·ÛÎfiËÛË ı· ·Ó·ÊÂÚı› ·Ó·Ï˘ÙÈÎfiÙÂÚ· ÌfiÓÔ ÛÙȘ ÚfiÛÊ·Ù· ÂÚÈÁڷʛۘ ÎÏÈÓÈΤ˜ ÔÓÙfiÙËÙ˜ ηıÒ˜ Î·È Û ӤԢ˜ ÏÔÈÌÔÁfiÓÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÔÈ ÔÔ›ÔÈ ÚÔηÏÔ‡Ó ¶™¢. ∆· ΢ÚÈfiÙÂÚ· ·›ÙÈ· ¶™¢ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1 (3). ø˜ ÂÈ‚·Ú˘ÓÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÁÈ· ¶™¢ ·Ó·Ê¤ÚÔÓÙ·È ÔÈ ÂÍ‹˜: ¯·ÌËÏfi ‚¿ÚÔ˜ Á¤ÓÓËÛ˘, ÌË Ï‹„Ë ÌËÙÚÈÎÔ‡ Á¿Ï·ÎÙÔ˜, ·ÙÔÈ΋ ÚԉȿıÂÛË, ÚÒÈÌË ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘ (ÓˆÚ›ÙÂÚ· ·fi ÙÔÓ 1Ô Ì‹Ó· ˙ˆ‹˜), ¯·ÌËÏ‹ ÎÔÈÓˆÓÈ΋ Ù¿ÍË, Î·È ÚÔÛ‚ÔÏ‹ Î·È ¿ÏÏÔ˘ ̤ÏÔ˘˜ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ·fi ¶™¢ (3). ∞. ™˘ÁÁÂÓ‹˜ „¢‰Ô·fiÊÚ·ÍË ∂ÌÊ·Ó›˙ÂÙ·È Î·Ù¿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô Ì ÛËÌ›· ÂÓÙÂÚÈ΋˜ ·fiÊڷ͢: ¤ÌÂÙÔ, ‰È¿Ù·ÛË ÎÔÈÏÈ¿˜, ·Ô˘Û›· ÎÂÓÒÛˆÓ, ¯ˆÚ›˜ fï˜ ·Ó·ÙÔÌÈÎfi ÎÒÏ˘Ì· (4). ™˘Ó‹ıˆ˜ ÂÌÊ·Ó›˙ÂÙ·È ÛÔÚ·‰Èο, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› fï˜ ÂÚÈÙÒÛÂȘ ÔÈ Ôԛ˜ ÎÏËÚÔÓÔÌÔ‡ÓÙ·È Ì ÂÈÎÚ·ÙÔ‡ÓÙ· ‹ ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. √Ê›ÏÂÙ·È Û˘Ó‹ıˆ˜ Û ·ÓÒÌ·ÏË ·Ó¿Ù˘ÍË ÙÔ˘ Ï›Ԣ Ì˘˚ÎÔ‡ ¯ÈÙÒÓ· ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ÙÔȯÒÌ·ÙÔ˜ ‹ Û ·ÙÂÏ‹ ·Ó¿Ù˘ÍË ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡ ϤÁÌ·ÙÔ˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘ (4). ∂› ÚÔÛ‚ÔÏ‹˜ ÙÔ˘ Ì˘˚ÎÔ‡ ¯ÈÙÒÓ· ·Ú·ÙËÚÂ›Ù·È ·ÓÙÈηٿÛÙ·ÛË ÙÔ˘ ÂÈÌ‹ÎÔ˘˜ Ì˘˚ÎÔ‡ ¯ÈÙÒÓ· Ì ÈÓÒ‰Ë ÈÛÙfi. ™Â Ó¢ÚÔÏÔÁÈ΋ ‚Ï¿‚Ë ·Ú·ÙËÚÂ›Ù·È Ì›ˆÛË ÙˆÓ Á·ÁÁÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ. ™Â ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ ·Ó·Ê¤ÚÔÓÙ·È ·ÓˆÌ·Ï›Â˜ ÛÙ· ·ÙÙ·Ú· ÙÔ˘ Cajal, ÂÓÒ Û ¿ÏϘ ÂÚÈÙÒÛÂȘ, ·ÓˆÌ·Ï›Â˜ ÛÙ· ÌÈÙÔ¯fiÓ‰ÚÈ· ÙˆÓ ÂÓÙÂÚÈÎÒÓ Î˘ÙÙ¿ÚˆÓ (5). ™ÙÔ 50% ÙˆÓ ÚÔۂ‚ÏËÌ¤ÓˆÓ ·È‰ÈÒÓ Ù· Û˘ÌÙÒÌ·Ù· ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ˘˜ ÙÚÂȘ ÚÒÙÔ˘˜ Ì‹Ó˜ ˙ˆ‹˜. ∆· 2/3 ÙˆÓ ÂÚÈÙÒÛÂˆÓ ·ÊÔÚÔ‡Ó Û ÚfiˆÚ· ‚Ú¤ÊË. ™ÙÔ 40% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û˘Ó˘¿Ú¯ÂÈ ·ÓÒÌ·ÏË ÂÚÈÛÙÚÔÊ‹ ÙÔ˘ ÂÓÙ¤ÚÔ˘. ∏ ‰˘ÛÎÔÈÏÈfiÙËÙ· ·ÔÙÂÏ› ÙËÓ ÈÔ Û˘¯Ó‹ ÂΉ‹ÏˆÛË, ÂÓÒ Ë ‰È¿ÚÚÔÈ· ÂÌÊ·Ó›˙ÂÙ·È ÛÙÔ 30-40% ÙˆÓ ÂÚÈÙÒÛˆÓ. ™˘ÌÌÂÙÔ¯‹ ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ·Ú·ÙËÚÂ›Ù·È Û˘¯Ó¿ Î·È ÂΉËÏÒÓÂÙ·È ÌÂ Û˘¯Ó¤˜ Ô˘ÚÔÏÔÈÌÒÍÂȘ. ∏ ‰È¿ÁÓˆÛË Ù›ıÂÙ·È Ì ̷ÓÔÌÂÙÚÈÎfi ¤ÏÂÁ¯Ô Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜ ÙÔ˘ ÂÙÈÎÔ‡, ÂÓÒ ÙÂÏÂ˘Ù·›· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Î·È ÛÈÓıËÚÔÁÚ·ÊÈΤ˜ ̤ıÔ‰ÔÈ Ì ڷ‰ÈÔ˚ÛfiÙÔ·. (6). ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ı· Á›ÓÂÈ ·fi ÙË ÓfiÛÔ ÙÔ˘ Hirschprung. ™Â ·ÓÙ›ıÂÛË fï˜, Ì ÙÔ Û˘ÁÁÂÓ¤˜ ÌÂÁ¿ÎÔÏÔ, Ë ÎÈÓËÙÈÎfiÙËÙ· ÙÔ˘ ÔÚıÔ‡ Î·È ÙÔ˘ ÚˆÎÙÔ‡ Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋. ∏ ÙÂÏÈ΋ ‰È¿ÁÓˆÛË Á›ÓÂÙ·È
¶›Ó·Î·˜ 1. ™˘¯ÓfiÙÂÚ· ·›ÙÈ· ÛÔ‚·Ú‹˜ ·Ú·ÙÂÈÓfiÌÂÓ˘ ‰È¿ÚÚÔÈ·˜ Û 38 ‚Ú¤ÊË (3) ∂ÓÙÂÚÈ΋ Ïԛ̈ÍË ∆ÚÔÊÈ΋ ·ÏÏÂÚÁ›· ∞˘ÙÔ¿ÓÔÛË ÂÓÙÂÚÔ¿ıÂÈ· ¡fiÛÔ˜ ÌÈÎÚÔÏ·¯ÓÈ·ÎÒÓ ÂÁÎÏ›ÛÙˆÓ ∫ÔÈÏÈÔοÎË HˆÛÈÓÔÊÈÏÈ΋ ÂÓÙÂÚÔ¿ıÂÈ· §ÂÌÊ·ÁÁÈÂÎÙ·Û›· 梉ԷfiÊÚ·ÍË ¡Â˘ÚÔ‰˘ÛÏ·Û›· ÕÁÓˆÛÙ· ·›ÙÈ·
18 8 2 2 1 1 1 1 1 3
Ì ÈÛÙÔÏÔÁÈ΋ ÂͤٷÛË ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘ ÌÂÙ¿ ·fi ‚ÈÔ„›·. ∏ ıÂڷ›· Û˘Ó›ÛÙ·Ù·È Û ¯ÔÚ‹ÁËÛË ÚÔÎÈÓËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ .¯. ·ÓÙ·ÁˆÓÈÛÙÒÓ Ù˘ 5-˘‰ÚÔ͢ÙÚ˘Ù·Ì›Ó˘ (ÛÈÛ·Ú›‰Ë), ‹ Ì ٷ ÓÂfiÙÂÚ· Ê¿Ú̷η alosetron (·ÓÙ·ÁˆÓÈÛÙ‹˜ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ Ù˘ 5-∏∆3) ‹ tegaserod (Û˘Ó·ÁˆÓÈÛÙ‹˜ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ Ù˘ 5-∏∆4). ™Â ÛÔ‚·Ú¤˜ ÂÚÈÙÒÛÂȘ Á›ÓÂÙ·È ÎÔÏÂÎÙÔÌ‹ ‹ ÂÈÏÂÔÛÙÔÌ›· (7,8). µ. ¡fiÛÔ˜ ÌÈÎÚÔÏ·¯ÓÈ·ÎÒÓ ÂÁÎÏ›ÛÙˆÓ ¶ÚfiÎÂÈÙ·È ÁÈ· ÛÔ‚·Ú‹ ‰È·ÚÚÔ˚΋ Û˘Ó‰ÚÔÌ‹ Ì ˘‰·Ú›˜ ÎÂÓÒÛÂȘ Î·È ÙÂÚ¿ÛÙȘ ·ÒÏÂȘ ËÏÂÎÙÚÔÏ˘ÙÒÓ ÛÙ· ÎfiÚ·Ó·, Ë ÔÔ›· ÂÌÊ·Ó›˙ÂÙ·È ·fi ÙȘ ÚÒÙ˜ Ë̤Ú˜ ˙ˆ‹˜ Î·È ·ÔÙÂÏ› ÙË Û˘¯ÓfiÙÂÚË ·ÈÙ›· ¶™¢ Ù˘ ÓÂÔÁÓÈ΋˜ ÂÚÈfi‰Ô˘ ÌÂÙ¿ ÙË ÏÔÈÌÒ‰Ë ·ÈÙÈÔÏÔÁ›· (9,10). ∂›Ó·È ·ÁÓÒÛÙÔ˘ ·ÈÙÈÔÏÔÁ›·˜ Î·È Èı·ÓfiÓ ÎÏËÚÔÓÔÌÂ›Ù·È Ì ۈ̷ÙÈο ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. ∏ ‰È¿ÁÓˆÛË Ù›ıÂÙ·È Ì ‚ÈÔ„›· ÙÔ˘ ÂÓÙ¤ÚÔ˘, Ë ÔÔ›· ‰Â›¯ÓÂÈ ·ÙÚÔÊ›· ÙˆÓ Ï·¯ÓÒÓ. ™ÙÔ ËÏÂÎÙÚÔÓÈÎfi ÌÈÎÚÔÛÎfiÈÔ, Ù· ·ÙÙ·Ú· ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘ Û˘ÁÎÂÓÙÚÒÓÔÓÙ·È Î·Ù¿ ÛˆÚÔ‡˜ Î·È ‰Â›¯ÓÔ˘Ó ÎÂÓÔÙfiÈ· ÙÔ˘ ΢ÙÙ·ÚÔÏ¿ÛÌ·ÙÔ˜. ∏ „˘ÎÙÚÔÂȉ‹˜ ·Ú˘Ê‹ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¤¯ÂÈ ·Û·Ê‹ fiÚÈ· (11). ∞ÓÔÛԯڈ̷ÙÈÛÌfi˜ ÁÈ· “‚ÈÏÏ›ÓË”, ¤Ó· ÌÈÎÚÔÓËÌ·Ù҉˜ Û˘ÛÙ·ÙÈÎfi ÙˆÓ ÌÈÎÚÔÏ·¯ÓÒÓ ·ÔηχÙÂÈ ÙËÓ ·ÚÔ˘Û›· ΢ÙÙ·ÚÈÎÒÓ ÂÁÎÏ›ÛÙˆÓ ·ÔÙÂÏÔ‡ÌÂÓˆÓ ·fi ·ÓÒ̷Ϙ ÌÈÎÚÔÏ¿¯Ó˜ ÔÈ Ôԛ˜ ʤÚÔ˘Ó „˘ÎÙÚÔÂȉ‹ ·Ú˘Ê‹, ÌÂÌ‚Ú¿ÓË, ÌÈÎÚÔÓËÌ¿ÙÈ·, ÓËÌ·ÙÈ҉˜ ÂÚ›‚ÏËÌ· (12). ∆· ¤ÁÎÏÂÈÛÙ· ·˘Ù¿ ·Ó¢ڛÛÎÔÓÙ·È Î·È Û ¿ÏÏ· ÂÈıËÏȷο ·ÙÙ·Ú· ÙfiÛÔ ÙÔ˘ ÂÙÈÎÔ‡ (ÛÙÔÌ¿¯È, ¯ÔÏˉfi¯Ô˜ ·ÛÙË, ÎfiÏÔÓ), fiÛÔ Î·È ÛÙ· ÓÂÊÚÈο ÛˆÏËÓ¿ÚÈ· (11). ∂›Û˘ ·ÓȯÓ‡ÔÓÙ·È Ê˘Û·ÏÈ‰Ò‰Ë ÛˆÌ¿ÙÈ· Ù· ÔÔ›· ÂÚȤ¯Ô˘Ó ÎÔÎÎÈ҉˜ ˘ÏÈÎfi ¿ÁÓˆÛÙ˘ ÚÔ¤Ï¢Û˘. ∏ ·ÈÙÈÔÏÔÁ›· Ù˘ ÓfiÛÔ˘ Â›Ó·È ¿ÁÓˆÛÙË. ¶Èı·ÓÔÏÔÁÂ›Ù·È ‰È·Ù·Ú·¯‹ ÛÙË ÌÂÙ·ÊÔÚ¿ ÚˆÙÂ˚ÓÒÓ ··Ú·›ÙËÙˆÓ ÁÈ· ÙË ‰ÔÌ‹ ÙÔ˘ ΢ÙÙ·ÚÔÏ¿ÛÌ·ÙÔ˜ ÙˆÓ ÂÓÙÂÚÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ªÂ ‚¿ÛË ÙË ıˆڛ· ·˘Ù‹, ÔÈ Ê˘Û·Ï›‰Â˜ ÔÈ Ôԛ˜ ·Ú·ÙËÚÔ‡ÓÙ·È ÛÙÔ ËÏÂÎÙÚÔÓÈÎfi ÌÈÎÚÔÛÎfiÈÔ ÚÔ¤Ú¯ÔÓÙ·È ·fi Ù· ۈ̿ÙÈ· ÙÔ˘ ¶·È‰È·ÙÚÈ΋ 2007;70:64-69
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Ã. ∫ÒÛÙ·ÏÔ˜
Golgi ·’ fiÔ˘ ÌÂÙ·Ó·ÛÙÂ‡Ô˘Ó ÚÔ˜ ÙËÓ ÂÈÊ¿ÓÂÈ· ÙÔ˘ ΢ÙÙ¿ÚÔ˘ ÌÂٷʤÚÔÓÙ·˜ Û˘ÛÙ·ÙÈο ··Ú·›ÙËÙ· ÁÈ· ÙË ‰fiÌËÛË Ù˘ „˘ÎÙÚÔÂȉԇ˜ ·Ú˘Ê‹˜. ™ÙËÓ ¿ıËÛË ·˘Ù‹ Ë ÔÚ›· ÙˆÓ Ê˘Û·Ï›‰ˆÓ ·Ó·ÎfiÙÂÙ·È Î·È ÔÈ Ê˘Û·Ï›‰Â˜ ηٷÎÚ·ÙÔ‡ÓÙ·È ÛÙÔ Î˘ÙÙ·ÚfiÏ·ÛÌ· ÙÔ˘ ΢ÙÙ¿ÚÔ˘ fiÔ˘ Î·È ‰ËÌÈÔ˘ÚÁÔ‡Ó ·ıÔÏÔÁÈο ÌÈÎÚÔÏ·¯ÓÒ‰Ë ¤ÁÎÏÂÈÛÙ· (9,13). ŒÓ· ¿ÏÏÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi Ù˘ ¿ıËÛ˘ Â›Ó·È Ë ·ÚÔ˘Û›· ·ÓÙÈÛˆÌ¿ÙˆÓ Î·Ù¿ ÙÔ˘ ηÚÎÈÓÔÂÌ‚Ú˘˚ÎÔ‡ (CEA) Î·È ÙÔ˘ Ï¢¯·ÈÌÈÎÔ‡ (CD10) ·ÓÙÈÁfiÓÔ˘ (12). ¢ÂÓ ˘¿Ú¯ÂÈ ÂȉÈ΋ ıÂڷ›· ÁÈ· ÙË ÓfiÛÔ. ÃÚËÛÈÌÔÔÈÔ‡ÓÙ·È ‰È¿ÊÔÚ· ·ÓÙÈÂÎÎÚÈÙÈο Ê¿Ú̷η .¯. ·ÓıÚ·ÎÈÎfi Ï›ıÈÔ (14) Î·È ÛˆÌ·ÙÔÛÙ·Ù›ÓË (15). ∏ Û›ÙÈÛË Á›ÓÂÙ·È ·ÚÂÓÙÂÚÈο ̤¯ÚȘ fiÙÔ˘ ‚ÚÂı› ÙÔ Î·Ù¿ÏÏËÏÔ ÌfiÛ¯Â˘Ì· ÂÓÙ¤ÚÔ˘ ÁÈ· ÌÂÙ·ÌfiÛ¯Â˘ÛË. °. ∂ÓÙÂÚÔ¿ıÂÈ· Ì ı˘Û¿ÓÔ˘˜ ∂ÌÊ·Ó›˙ÂÙ·È Î·Ù¿ ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ ˙ˆ‹˜ Ì ¯ÚfiÓÈ· ˘‰·Ú‹ ‰È¿ÚÚÔÈ· Î·È Î·ı˘ÛÙ¤ÚËÛË ÛÙË ÛˆÌ·ÙÈ΋ ·Ó¿Ù˘ÍË. √ÚÈṲ̂ӷ ‚Ú¤ÊË ÂÌÊ·Ó›˙Ô˘Ó Î·È ‰˘ÛÌÔÚÊÈÎfi ÚfiÛˆÔ. ¶·Ú·ÙËÚÂ›Ù·È Û˘¯ÓfiÙÂÚ· Û ¿ÙÔÌ· Ì·ÏÙ¤˙È΢ ηٷÁˆÁ‹˜ (16). º·›ÓÂÙ·È fiÙÈ ÔÊ›ÏÂÙ·È Û ·ÓˆÌ·Ï›Â˜ ÛÙËÓ ¤ÎÊÚ·ÛË Î˘ÙÙ·ÚÈÎÒÓ ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘. ∞ӷʤÚÔÓÙ·È ‰È·Ù·Ú·¯¤˜ ÛÙËÓ Î·Ù·ÓÔÌ‹ Ù˘ ¿ÏÊ·2/‚‹Ù·1 ÈÓÙÂÁÎÚ›Ó˘ Î·È ‰ÔÌÈΤ˜ ÌÂÙ·‚ÔϤ˜ ÛÙ· ‰ÂÛÌÔۈ̿ÙÈ· ÙÔ˘ ÂÓÙ¤ÚÔ˘ (17,18). ™Â ÔÓÙ›ÎÈ· ¿Û¯ÔÓÙ· ·fi ÙË ÓfiÛÔ ‚Ú¤ıËÎ·Ó Â›Û˘ ÌÂȈ̤ӷ ›‰· ÙÔ˘ ˘Ô‰Ô¯¤· Ù‡Ô˘ 2 ÙÔ˘ ÌÂÙ·ÙÚÂÙÈÎÔ‡ ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ‚. √È ·ÓˆÌ·Ï›Â˜ ·˘Ù¤˜ Ô‰ËÁÔ‡Ó Û ‰È·Ù·Ú·¯¤˜ ˆ˜ ÚÔ˜ ÙË Û‡ÛÙ·ÛË Ù˘ ‚·ÛÈ΋˜ ÌÂÌ‚Ú¿Ó˘ Î·È Î˘Ú›ˆ˜ Ù˘ Ï·ÌÈÓ›Ó˘ Î·È Ù˘ ÚˆÙÂÔÁÏ˘Î¿Ó˘ ÙˆÓ ÂÈıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ÂÓÙ¤ÚÔ˘, ÌÂ Û˘Ó¤ÂÈ· Ó· ˘Ê›ÛÙ·ÓÙ·È ·fiÙˆÛË (19). ∏ ‚ÈÔ„›· ÙÔ˘ ÂÓÙ¤ÚÔ˘ ‰Â›¯ÓÂÈ ÔÏÈ΋ ‹ ÌÂÚÈ΋ ·ÙÚÔÊ›· ÙˆÓ Ï·¯ÓÒÓ, ˘ÂÚÏ·Û›· ÎÚ˘ÙÒÓ Î·È ·‡ÍËÛË ÙˆÓ ÊÏÂÁÌÔÓˆ‰ÒÓ Î˘ÙÙ¿ÚˆÓ ÛÙÔ ¯fiÚÈÔ. ÷ڷÎÙËÚÈÛÙÈÎfi ‡ÚËÌ· ·ÔÙÂÏ› Ë ·Û‡ÓÙ·ÎÙË Û˘ÛÛÒÚ¢ÛË ÂÈıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì ‰ËÌÈÔ˘ÚÁ›· “ı˘Û¿ÓˆÓ” ÏfiÁˆ ÙÔ˘ fiÙÈ Ë Î˘ÙÙ·ÚÈ΋ ÙÔ˘˜ ÌÂÌ‚Ú¿ÓË Â›Ó·È ÛÙÚÔÁÁ˘ÏÂ̤ÓË ÛÙËÓ ÎÔÚ˘Ê‹, ÒÛÙ ÙÔ ÂÈıËÏÈ·Îfi ·ÙÙ·ÚÔ Ó· ÚÔÛÔÌÔÈ¿˙ÂÈ Ì ÛÙ·ÁfiÓ· ‰·ÎÚ‡Ô˘ (16). ¢ÂÓ ˘¿Ú¯ÂÈ ÂȉÈ΋ ıÂڷ›· Î·È Ë ÌfiÓË ‰È¤ÍÔ‰Ô˜ ÛÙËÓ ÂÚ›ÙˆÛË ·˘Ù‹ Â›Ó·È Ë ÌÂÙ·ÌfiÛ¯Â˘ÛË ÂÓÙ¤ÚÔ˘. ¢. ∞˘ÙÔ¿ÓÔÛË ÂÓÙÂÚÔ¿ıÂÈ· √Ê›ÏÂÙ·È ÛÙËÓ ‡·ÚÍË ·ÓÙÈÛˆÌ¿ÙˆÓ Î·Ù¿ ·ÓÙÈÁfiÓˆÓ ÙˆÓ ÂÓÙÂÚÔ΢ÙÙ¿ÚˆÓ. ∆Ô ·˘ÙÔ·ÓÙÈÁfiÓÔ ÙÔ ÔÔ›Ô ÚÔηÏ› ÙËÓ ·ÓÔÛÔÏÔÁÈ΋ ·¿ÓÙËÛË ÔÓÔÌ¿˙ÂÙ·È MCC2 Î·È Î·ıÔÚ›˙ÂÙ·È ·fi ÁÔÓ›‰ÈÔ ÛÙÔ ¯ÚˆÌfiۈ̷ 19p13. ∏ ÓfiÛÔ˜ Û˘¯Ó¿ Û˘Óԉ‡ÂÙ·È ·fi Â͈-ÂÓÙÂÚÈΤ˜ ÂΉËÏÒÛÂȘ ·˘ÙÔ·ÓÔÛ›·˜ (9). ∏ ‚ÈÔ„›· ÂÓÙ¤ÚÔ˘ ‰Â›¯ÓÂÈ ·ÙÚÔÊ›· ÙˆÓ Ï·¯ÓÒÓ Î·È ‰È‹ıËPaediatriki 2007;70:64-69
ÛË ÙÔ˘ ¯ÔÚ›Ô˘ Ì ∆ ÏÂÌÊÔ·ÙÙ·Ú·, ηıÒ˜ Î·È ˘ÂÚÏ·Û›· ÙˆÓ ÎÚ˘ÙÒÓ. ∂›Û˘ ·Ú·ÙËÚÂ›Ù·È ‰ÂηϿÛÈ· ·‡ÍËÛË CD-3 ıÂÙÈÎÒÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ ÛÙÔ ÂÈı‹ÏÈÔ Î·È ÛÙÔ ¯fiÚÈÔ ÙÔ˘ ÏÂÙÔ‡ Î·È ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘ (21,22). ∏ ıÂڷ›· Û˘Ó›ÛÙ·Ù·È ÛÙË ¯ÔÚ‹ÁËÛË ·ÓÔÛÔηٷÛÙ·ÏÙÈÎÒÓ Ê·ÚÌ¿ÎˆÓ .¯. ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ, ΢ÎÏÔÛÔÚ›Ó˘, ΢ÎÏÔʈÛÊ·Ì›‰Ë˜, tacrolimus, Î·È ÙÔ˘ Ó¤Ô˘ ·Ú¿ÁÔÓÙ· infliximab ÙÔ ÔÔ›Ô ·Ó·ÛÙ¤ÏÏÂÈ ÙË ‰Ú¿ÛË ÙÔ˘ ∆¡F (23). ∂. ™‡Ó‰ÚÔÌÔ ·ÓÔÛÔÏÔÁÈ΋˜ ‰˘ÛÚ˘ıÌ›·˜, ÔÏ˘ÂÓ‰ÔÎÚÈÓÔ¿ıÂÈ·˜, ÂÓÙÂÚÔ¿ıÂÈ·˜ Î·È Ê˘ÏÔÛ‡Ó‰ÂÙ˘ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜ (IPEX) ∏ ÔÓÔÌ·Û›· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÚÔ¤Ú¯ÂÙ·È ·fi ÙÔ ·ÎÚˆÓ‡ÌÈÔ ÙˆÓ ‰È·Ù·Ú·¯ÒÓ ÙȘ Ôԛ˜ ÚÔηÏ› (Immune dysregulation, Polyendocrino-pathy, Enteropathy, X-linked syndrome) ‰ËÏ·‰‹ ·ÓÔÛÔÏÔÁÈ΋ ‰˘ÛÚ˘ıÌ›·, ÔÏ˘ÂÓ‰ÔÎÚÈÓÔ¿ıÂÈ·, ÂÓÙÂÚÔ¿ıÂÈ· Î·È Ê˘ÏÔÛ‡Ó‰ÂÙË ÎÏËÚÔÓÔÌÈÎfiÙËÙ· (24,25). ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ‰È‹ıËÛË ÙÔ˘ ¯ÔÚ›Ô˘ Ì ÂÓÂÚÁÔÔÈË̤ӷ ∆ ÏÂÌÊÔ·ÙÙ·Ú· ηıÒ˜ Î·È ·fi ·ÙÚÔÊ›· ÙˆÓ Ï·¯ÓÒÓ. ¶ÚÔηÏÂ›Ù·È ·fi ÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ÁÔÓȉ›Ô˘ FOXP3 (ÛηÚÊ›ÓË) Ë ÔÔ›· Û¯ÂÙ›˙ÂÙ·È Ì ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ CD4+ T ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ (26). ∂ÎÙfi˜ ·fi ‰È·ÚÚÔ˚Îfi Û‡Ó‰ÚÔÌÔ, Ù· ¿Û¯ÔÓÙ· ¿ÙÔÌ· ÂÌÊ·Ó›˙Ô˘Ó Û·Î¯·ÚÒ‰Ë ‰È·‚‹ÙË, ı˘ÚÂÔÂȉԿıÂÈ·, ȯı‡ˆÛË, ·ÈÌÔÏ˘ÙÈ΋ ·Ó·ÈÌ›·. ∏ ÓfiÛÔ˜ ·ÓÙÈÌÂÙˆ›˙ÂÙ·È Ì ΢ÙÙ·ÚÔÛÙ·ÙÈο. ™Ù. ∂È̤ÓÔ˘Û· ÂÏÎ҉˘ ÎÔÏ›Ùȉ· ¶ÚfiÎÂÈÙ·È ÁÈ· Ôχ Û¿ÓÈ· ÓÔÛÔÏÔÁÈ΋ ÔÓÙfiÙËÙ· Ë ÔÔ›· Èı·ÓfiÓ ÎÏËÚÔÓÔÌÂ›Ù·È Ì ÙÔÓ ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. ∆· ÎÚÈÙ‹ÚÈ· ‰È¿ÁÓˆÛ˘ ›ӷÈ: Û˘ÁÁÂÓÈ΋ Û¯¤ÛË ÙˆÓ ÁÔÓ¤ˆÓ, Î·È ÂΉËÏÒÛÂȘ ·fi ÙÔ ÛÙfiÌ· Î·È ÙËÓ ÂÚÈÚˆÎÙÈ΋ ÂÚÈÔ¯‹ (ÂÍÂÏÎÒÛÂȘ, ÊÏÂÁÌÔÓ‹, Û˘Ú›ÁÁÈ·) (27,28). ∂ÎÙfi˜ ·fi ÙË ‰È¿ÚÚÔÈ·, Ë ¿ıËÛË Û˘Óԉ‡ÂÙ·È ·fi ‚ÏÂÓÓÔ·ÈÌÔÚÚ·ÁÈΤ˜ ÎÂÓÒÛÂȘ. ∫·Ù¿ ÙËÓ ÂÓ‰ÔÛÎfiËÛË ‹ ÙË ‚ÈÔ„›· ·Ú·ÙËÚÂ›Ù·È ÂÈÎfiÓ· ÎÔÏ›Ùȉ·˜ Ì ‰È·ÙÔȯˆÌ·ÙÈο ¤ÏÎË Û ۯ‹Ì· ÊÏ·ÛÎÈÔ‡ Ì ÂÈÎÚÂÌ¿ÌÂÓ· ¯Â›ÏË, ηıÒ˜ Î·È ·ÔÛÙËÌ¿ÙÈ· ÎÚ˘ÙÒÓ. ∏ ÈÛÙÔÏÔÁÈ΋ ÂÈÎfiÓ· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ‰È‹ıËÛË ÙÔ˘ ¯ÔÚ›Ô˘ ·fi ÊÏÂÁÌÔÓÒ‰Ë Î‡ÙÙ·Ú·. ™Â ÛÔ‚·Ú¤˜ ÌÔÚʤ˜ ˘¿Ú¯ÂÈ ÂÎÙÂٷ̤ÓË ·Ó¿Ù˘ÍË ÎÔÎÎÈÒ‰Ô˘˜ ÈÛÙÔ‡ Î·È Û¯ËÌ·ÙÈÛÌfi˜ „¢‰ÔÔÏ˘fi‰ˆÓ. ∂ÎÙfi˜ ·fi ÙÔ ÎfiÏÔÓ ·Ú·ÙËÚÂ›Ù·È Û˘ÌÌÂÙÔ¯‹ Î·È ÙÔ˘ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘ Ì Ì›ˆÛË Ù˘ Û¯¤Û˘ Ï·¯ÓÒÓ/ÎÚ˘ÙÒÓ. ∂ÎÙfi˜ ·fi ÙËÓ ÂÓ‰ÔÛÎfiËÛË Î·È ÙË ‚ÈÔ„›·, Ë ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ Î·È ÁÂÓÈο ÙˆÓ ÊÏÂÁÌÔÓˆ‰ÒÓ ·ı‹ÛÂˆÓ ÙÔ˘ ÂÓÙ¤ÚÔ˘ ·fi ÙȘ ÂÎÎÚÈÙÈÎÔ‡ Ù‡Ô˘ ÂÓÙÂÚÔ¿ıÂȘ ÌÔÚ› Ó· Á›ÓÂÈ Ì ÂͤٷÛË ÛÙ· ÎfiÚ·Ó· ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË Ù˘ Ô˘Û›·˜
67
µÚÂÊÈ΋ ·Ú·ÙÂÈÓfiÌÂÓË ‰È¿ÚÚÔÈ·
ηÏÚÔÙÂÎÙ›Ó˘. ¶ÚfiÎÂÈÙ·È ÁÈ· ÚˆÙ½ÓË Ô˘ ‰ÂÛ̇ÂÈ ÙÔ ·Û‚¤ÛÙÈÔ Î·È ÙÔÓ „¢‰¿ÚÁ˘ÚÔ ÛÙ· Ô˘‰ÂÙÂÚfiÊÈÏ· ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ÙÔȯÒÌ·ÙÔ˜. ∏ ÚˆÙ½ÓË ·˘Ù‹ ·Ú·Ì¤ÓÂÈ ¿ıÈÎÙË ÛÙ· ÎfiÚ·Ó· ̤¯ÚÈ Î·È 7 Ë̤Ú˜ ÌÂÙ¿ ÙËÓ ·Ô‚ÔÏ‹ Ù˘ (29). ∏ ·ÚÔ˘Û›· Ù˘ ˘Ô‰ËÏÒÓÂÈ ÊÏÂÁÌÔÓ‹ ÙÔ˘ ÂÓÙ¤ÚÔ˘. ÕÏÏÔÈ ‰Â›ÎÙ˜ ÊÏÂÁÌÔÓ‹˜ Â›Ó·È Ë ·ÚÔ˘Û›· ÙÔ˘ ·Ú¿ÁÔÓÙ· ÊÏÂÁÌÔÓ‹˜ TNF, Ë ÂÎÙ›ÌËÛË ÙÔ˘ ·Ô‚·ÏÏfiÌÂÓÔ˘ ÛÙ· ÎfiÚ·Ó· ÌÔÓÔÍÂȉ›Ô˘ ÙÔ˘ ·˙ÒÙÔ˘ (30) Î·È Ì¤ÙÚËÛË ÙÔ˘ ÂÓ˙‡ÌÔ˘ ÂÏ·ÛÙ¿ÛË ÛÙ· ÎfiÚ·Ó· (31). ∏ ıÂڷ›· Û˘Ó›ÛÙ·Ù·È Û ˘ÊÔÏÈ΋ ÎÔÏÂÎÙÔÌ‹ Î·È Û ¯ÔÚ‹ÁËÛË Î˘ÙÙ·ÚÔÛÙ·ÙÈÎÒÓ. ¶·Ú·ÙËÚÂ›Ù·È fï˜ Û˘¯Ó¿, ·Ó¿Ù˘ÍË ÏÂÌÊˆÌ¿ÙˆÓ (32). ¶ÚfiÛÊ·Ù· ‰ÔÎÈÌ¿ÛÙËÎ·Ó Ì ÂÈÙ˘¯›·, Û ÂÓ‹ÏÈΘ fï˜, ÔÚÈṲ̂ӷ Ó¤· Ê¿Ú̷η .¯. Ô ·ÓÙ·ÁˆÓÈÛÙ‹˜ ÙÔ˘ ∆¡F infliximab Î·È ÔÈ ·ÓÙ·ÁˆÓÈÛÙ¤˜ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ Ù˘ IL-2 daclizumab, basiliximab, visilizumab (33). ∑. ¢È·Ù·Ú·¯¤˜ ÁÏ˘ÎÔ˙˘Ï›ˆÛ˘ ÙˆÓ ¡-ÁÏ˘ÎÔÚˆÙÂ˚ÓÒÓ ∆· ‚Ú¤ÊË Ì ÙËÓ ¿ıËÛË ·˘Ù‹ ÂÌÊ·Ó›˙Ô˘Ó ÂÎÙfi˜ ·fi ›ÌÔÓË ‰È¿ÚÚÔÈ· Î·È ‰˘ÛÌÔÚÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο (ÌÈÎÚÔÁÓ·ı›·, ·ÓÒÌ·ÏË Î·Ù·ÓÔÌ‹ ÙÔ˘ ˘Ô‰fiÚÈÔ˘ Ï›Ô˘˜, ÌÈÎÚÔÛÙÔÌ›·), Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·, Ë·ÙÔ¿ıÂÈ·, ıÚÔÌ‚ÔÂÓ›·, ˘ÂÚÛÏËÓÈÛÌfi, ηٷÚÚ¿ÎÙË, ÌÂÏ·Á¯ÚˆÌ·ÙÈ΋ ·ÌÊÈ‚ÏËÛÙÚÔÂȉԿıÂÈ·, ηıÒ˜ Î·È ¯·ÌËÏ¿ ›‰· ÁÏ˘ÎÔÚˆÙÂ˚ÓÒÓ .¯. ·ÙÔÛÊ·ÈÚ›Ó˘, ÙÚ·ÓÛÊÂÚ›Ó˘, ı˘ÚÂÔÙÚfiÔ˘ ÔÚÌfiÓ˘. ∏ ‰È¿ÁÓˆÛË Ù›ıÂÙ·È Ì ËÏÂÎÙÚÔÊfiÚËÛË Ù˘ ÙÚ·ÓÛÊÂÚÚ›Ó˘. ¢ÂÓ ˘¿Ú¯ÂÈ ÂȉÈ΋ ıÂڷ›· ÁÈ· ÙË Û˘ÁÎÂÎÚÈ̤ÓË ¿ıËÛË. ™Â ÔÚÈṲ̂Ó˜ ÌÔÚʤ˜ ¤¯ÂÈ ‰ÔÎÈÌ·ÛÙ› Ë ¯ÔÚ‹ÁËÛË Ì·ÓÓfi˙˘ ·fi ÙÔ ÛÙfiÌ· (34). ∏. §ÔÈÌÒ‰Ë ·›ÙÈ· ∂ÎÙfi˜ ·fi Ù· ÁÓˆÛÙ¿ ÏÔÈÌÒ‰Ë ·›ÙÈ· Ù· ÔÔ›· ÚÔηÏÔ‡Ó ·Ú·ÙÂÈÓfiÌÂÓË ‰È¿ÚÚÔÈ·, Ë ÚfiÔ‰Ô˜ Ë ÔÔ›· ¤¯ÂÈ Û˘ÓÙÂÏÂÛÙ› Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ÛÙË ÌÈÎÚÔ‚ÈÔÏÔÁ›· Î·È Ë ¯ÚËÛÈÌÔÔ›ËÛË Ù˘ ·Ï˘ÛȉˆÙ‹˜ ·ÓÙ›‰Ú·Û˘ Ù˘ ÔÏ˘ÌÂÚ¿Û˘ (PCR) Î·È ÙÔ˘ ËÏÂÎÙÚÔÓÈÎÔ‡ ÌÈÎÚÔÛÎÔ›Ô˘, η٤ÛÙËÛ ‰˘Ó·Ù‹ ÙËÓ ·Ó›¯Ó¢ÛË Ó¤ˆÓ ÏÔÈÌÔÁfiÓˆÓ ·Ú·ÁfiÓÙˆÓ ÔÈ ÔÔ›ÔÈ ÚÔηÏÔ‡Ó ÂÓÙÂÚÈΤ˜ ÏÔÈÌÒÍÂȘ ÂȉÈο Û ·ÓÔÛÔηٷÛÙ·Ï̤ӷ ¿ÙÔÌ· ηıÒ˜ Î·È Û ÚfiˆÚ· ‹ ‰˘ÛÙÚÔÊÈο ‚Ú¤ÊË. ªÈ· Ó¤· ÔÌ¿‰· ÎÔÏÔ‚·ÎÙËÚȉ›ˆÓ (enteroaggregative E. coli) ¤¯ÂÈ ·Ó·‰Âȯı› ˆ˜ ‚·ÛÈÎfi ·›ÙÈÔ ¶™¢, fiˆ˜ Î·È ÈÔ› (ÌÂÁ·ÏÔ΢ÙÙ·ÚÔ˚fi˜, Î·È Ô Èfi˜ ∏πV) (35,36). √ Ì˯·ÓÈÛÌfi˜ Ì ÙÔÓ ÔÔ›Ô Ô HIV ÚÔηÏ› ÂÓÙÂÚÔ¿ıÂÈ· Â›Ó·È Ì¤Ûˆ ‰Â˘ÙÂÚÔÁÂÓÒÓ ÏÔÈÌÒÍÂˆÓ Ì ‚·ÎÙËÚ›‰È·, ÚˆÙfi˙ˆ·, Î·È ·Ú¿ÛÈÙ· ÏfiÁˆ Ù˘ ·ÓÔÛÔÏÔÁÈ΋˜ ·Ó¿ÚÎÂÈ·˜. ∏ ¤ÎÏ˘ÛË ÊÏÂÁÌÔÓˆ‰ÒÓ ÌÂÛÔÏ·‚ËÙÒÓ Î·È Ë Î·¯ÂÍ›· Ë ÔÔ›· Û˘Ó‹ıˆ˜ Û˘Óԉ‡ÂÙ·È ·fi ¤ÏÏÂÈ„Ë ··Ú·›ÙËÙˆÓ
ȯÓÔÛÙÔȯ›ˆÓ .¯. ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘, Û˘Ì‚¿ÏÏÔ˘Ó Â›Û˘ ÛËÌ·ÓÙÈο ÛÙË ‰È·ÈÒÓÈÛË Ù˘ ÂÓÙÂÚÈ΋˜ ‚Ï¿‚˘ (37). ∂ÎÙfi˜ ·fi ÙÔ˘˜ ·Ú·¿Óˆ ÏÔÈÌÔÁfiÓÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÙˆÓ ÔÔ›ˆÓ Ô ÚfiÏÔ˜ ÛÙËÓ ÚfiÎÏËÛË ¶™¢ Â›Ó·È ·Ô‰Â‰ÂÈÁ̤ÓÔ˜, Ë ÂÊ·ÚÌÔÁ‹ Û‡Á¯ÚÔÓˆÓ ÌÂıfi‰ˆÓ ·ÔÎ¿Ï˘„ ÙËÓ ·ÚÔ˘Û›· Î·È Ï‹ıÔ˘˜ Ó¤ˆÓ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ ÛÙÔÓ ÂÙÈÎfi ۈϋӷ fiˆ˜ ÈÒÓ (astrovirus, calicivirus, torovirus, coronavirus) (38), ηıÒ˜ Î·È ·Ú·Û›ÙˆÓ (ÎÚ˘ÙÔÛÔÚ›‰È·, ΢ÎÏfiÛÔÚ·, ÈÛfiÛÔÚ·) (39). O ÚfiÏÔ˜ fiÏˆÓ ·˘ÙÒÓ ÙˆÓ ·Ú·ÁfiÓÙˆÓ ÛÙËÓ ÚfiÎÏËÛË ¶™¢ ̤ÓÂÈ Ó· ‰È¢ÎÚÈÓÈÛı›. ∏ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÂÓÙÂÚÈÎÒÓ ÏÔÈÌÒÍÂˆÓ Á›ÓÂÙ·È Ì ÙË ¯Ú‹ÛË ·ÓÙÈ‚ÈÔÙÈÎÒÓ, ·ÓÙÈ-ÈÈÎÒÓ Î·È ·ÓÙÈ·Ú·ÛÈÙÈÎÒÓ Ê·Ú̿ΈÓ. ªÂÁ¿ÏÔ ÂӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ Î·È Ë ÚÔÛ¿ıÂÈ· ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· ÂÌ‚ÔÏ›ˆÓ ηٿ ÔÏÏÒÓ ÂÓÙÂÚÈÎÒÓ ·ıÔÁfiÓˆÓ (40,41). ∂ȉÈ΋ ıÂڷ›· ÁÈ· ÙȘ ·Ú·¿Óˆ ·ı‹ÛÂȘ, ÏËÓ ·˘ÙÒÓ ÔÈ Ôԛ˜ ÔÊ›ÏÔÓÙ·È Û ÏÔÈÌÒ‰Ë ·›ÙÈ·, ‰ÂÓ ˘¿Ú¯ÂÈ. µÚ›ÛÎÔÓÙ·È, fï˜, Û ÂͤÏÈÍË ‰È¿ÊÔÚ˜ Ӥ˜ ÌÔÚʤ˜ ıÂڷ›·˜ .¯. ·˘ÍËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ -ÂȉÂÚÌÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁˆÓ (4244), ÂÚ˘ıÚÔÔÈËÙ›ÓË (45), ÈÓÛÔ˘Ï›ÓË (46)- ÔÈ Ôԛ˜ ÚÔ¿ÁÔ˘Ó ÙËÓ ·Ó¿Ï·ÛË ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ÂÈıËÏ›Ô˘ Î·È Èı·ÓÒ˜ ÛÙÔ Ì¤ÏÏÔÓ ı· Û˘ÓÙÂϤÛÔ˘Ó ÛÙËÓ Ï¤ÔÓ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ¶™¢. ªÂÁ¿ÏÔ ÂӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Î·È ‚ÈÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜ (ÚÔ‚ÈÔÙÈο, Ú‚ÈÔÙÈο) ÔÈ ÔÔ›ÔÈ Â˘Ô‰ÒÓÔ˘Ó ÙËÓ ·Ó¿Ù˘ÍË Ê˘ÛÈÔÏÔÁÈ΋˜ ¯ÏˆÚ›‰·˜ ÛÙÔ ¤ÓÙÂÚÔ ·ÏÏ¿ ¤¯Ô˘Ó Î·È ·ÓÙÈÊÏÂÁÌÔÓÒ‰ÂȘ ȉÈfiÙËÙ˜ (47-49). ∂›Û˘ ÂÚÂ˘Ó¿Ù·È Î·È Ô ÚfiÏÔ˜ ıÚÂÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÔÈ ÔÔ›ÔÈ Â›Ó·È ··Ú·›ÙËÙÔÈ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘. ∂‰Ò ˘¿ÁÔÓÙ·È Ù· ÓÔ˘ÎÏÂÔÙ›‰È·, Ù· ÔÏ˘·ÎfiÚÂÛÙ· ÏÈ·Ú¿ Ôͤ·, Ë ÁÏÔ˘Ù·Ì›ÓË, Ë ·ÚÁÈÓ›ÓË Î.¿. (50-52). ¢È¿ÊÔÚ· ȯÓÔÛÙÔȯ›· ¤¯Ô˘Ó ›Û˘ ÎÚÈı› ··Ú·›ÙËÙ· ÁÈ· ÙË ÛˆÛÙ‹ ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘, fiˆ˜ Ô „¢‰¿ÚÁ˘ÚÔ˜. ∏ ¤ÏÏÂÈ„Ë Ûȉ‹ÚÔ˘ ¤¯ÂÈ ‚ÚÂı› fiÙÈ ·˘Í¿ÓÂÈ ÙË ‰È·ÂÚ·ÙfiÙËÙ· ÙÔ˘ ÂÓÙ¤ÚÔ˘ Û ·ıÔÁfiÓÔ˘˜ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜ Î·È Ô‰ËÁ› Û ¶™¢ (41). ™Â οı ÂÚ›ÙˆÛË, Ô ÌËÙÚÈÎfi˜ ıËÏ·ÛÌfi˜ ·ÔÙÂÏ› ÛËÌ·ÓÙÈÎfi ̤ÛÔ ÚfiÏ˄˘ Î·È ıÂڷ›·˜ Ù˘ ¶™¢ ÏfiÁˆ ÙˆÓ ·ÓÙÈÌÈÎÚÔ‚È·ÎÒÓ Î·È ·ÓÙÈÊÏÂÁÌÔÓˆ‰ÒÓ È‰ÈÔÙ‹ÙˆÓ ÙÔ˘ Á˘Ó·ÈΛԢ Á¿Ï·ÎÙÔ˜ ηıÒ˜ Î·È ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÙÔ˘˜ ÔÔ›Ô˘˜ ÂÚȤ¯ÂÈ (53).
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poietin present in human milk? Studies of erythropoietin receptors on enterocytes of human and rat neonates. Pediatr Res 1999;46:263-268. Shulman RJ. Effect of enteral administration of insulin on intestinal development and feeding tolerance in preterm infants: a pilot study. Arch Dis Child Fetal Neonatal Ed 2002;86:F131-F133. Millar M, Wilks M, Costeloe K. Probiotics for preterm infants? Arch Dis Child Fetal Neonatal Ed 2003;88:F354-F358. Agostoni C, Axelsson I, Goulet O, Koletzko B, Michaelsen KF, Puntis JW et al. Prebiotic oligosaccharides in dietetic products for infants: a commentary by the ESPGAN Committee on Nutrition. J Pediatr Gastroenterol Nutr 2004;39: 465-473. Isolauri E, Ribeiro H da C, Gibson G, Saavedra J, Saliminen S, Vanderhoof J et al. Functional foods and probiotics: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 2002;35 (2 Suppl):S106-109. Yau KI, Huang CB, Chen W, Chen SJ, Chou YH, Huang FX et al. Effect of nucleotides on diarrhea and immune responses in healthy term infants in Taiwan. J Pediatr Gastroenterol Nutr 2003;36:37-43. Kleinman RE, Berseth C, Castillo-Duran C, Cleghorn G, Devane S, Garcia RJ et al. Perinatal nutrition and gastrointestinal disorders: Working Group Report of the Second World Congress of Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 2004;39 (2 Suppl):S703-S710. Burrin DG, Stoll B. Key nutrients and growth factors for the neonatal gastrointestinal tract. Clin Perinatol 2002;29:65-96. Kunz C, Rodriguez-Palmero M, Koletzko B, Jensen R. Nutritional and biochemical properties of human milk, part I: General aspects, proteins, and carbohydrates. Clin Perinatol 1999;26:307-333.
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70
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PRACTICAL ISSUE
∫·Ì‡Ï˜ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘ ÙˆÓ ·È‰ÈÒÓ Ù˘ ¯ÒÚ·˜ Ì·˜ ¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘, ∆Ì‹Ì· π·ÙÚÈ΋˜, ∆Ô̤·˜ ∫ÔÈÓˆÓÈ΋˜ π·ÙÚÈ΋˜, ∫ÏÈÓÈ΋ ¶ÚÔÏËÙÈ΋˜ π·ÙÚÈ΋˜ Î·È ¢È·ÙÚÔÊ‹˜ AÏÏËÏÔÁÚ·Ê›·: ∞ÓÙÒÓ˘ ∫·Ê¿ÙÔ˜ kafatos@med.uoc.gr ¢È·ÛÙ·‡ÚˆÛË µÔ‡Ù˜ ™Ù·˘Ú¿ÎÈ·, ∆.∫. 71 003, ∆.£.: 2208, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘
∞. ∫·Ê¿ÙÔ˜ ¶ÂÚ›ÏË„Ë: ∞·Ú·›ÙËÙÔ ÂÚÁ·ÏÂ›Ô Î¿ı ·È‰›·ÙÚÔ˘ Î·È ÁÔÓÈÔ‡ Â›Ó·È ÔÈ Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘ ÙÔ˘ ·È‰ÈÔ‡ ·fi ÙË Á¤ÓÓËÛË ¤ˆ˜ Î·È ÙËÓ ÔÏÔÎÏ‹ÚˆÛ‹ Ù˘. ™ÙÔ ‚È‚ÏÈ¿ÚÈÔ ˘Á›·˜ ÙÔ˘ ·È‰ÈÔ‡ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÁÈ· ÔÏÏ¿ ¯ÚfiÓÈ· ·fi ÙÔ ÀÔ˘ÚÁÂ›Ô ÀÁ›·˜, ÔÈ Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘ ÙÔ˘ πÓÛÙÈÙÔ‡ÙÔ˘ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ (πÀ¶) ÁÈ· ·È‰È¿ 6-18 ÂÙÒÓ Î·È ÂΛӘ ÙˆÓ ∏¶∞ ÁÈ· ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜. ∆Ô 2003 Ӥ˜ η̇Ϙ ·Ó¿Ù˘Í˘ ÂÌÊ·Ó›ÛıËÎ·Ó ÛÙ· ‚È‚ÏÈ¿ÚÈ· ˘Á›·˜ Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙËÓ ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ∞Á›· ™ÔÊ›· (∞’ ¶∫¶∞). ™¯ÂÙÈ΋ ‰ËÌÔÛ›Â˘ÛË ·Ó¤ÊÂÚ fiÙÈ ÙÔ ‰Â›ÁÌ· ÚÔÂÚ¯fiÙ·Ó ·fi ÙËÓ ÂÚÈÔ¯‹ ∞ıËÓÒÓ ¯ˆÚ›˜ Ó· ‰›‰ÔÓÙ·È ÏËÚÔÊÔڛ˜ ·Ó ÚfiÎÂÈÙ·È ÁÈ· ηÓÔÓÈο, ÌÈÎÚ¿ ‹ ÌÂÁ¿Ï· ÁÈ· ÙËÓ ËÏÈΛ· Ù˘ ΢‹Ûˆ˜ ‚Ú¤ÊË, ıËÏ¿˙ÔÓÙ· ‹ ÌË Î·ıÒ˜ Î·È ÁÈ· ÙȘ ÌÂıfi‰Ô˘˜ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËηÓ. √ ¶·ÁÎfiÛÌÈÔ˜ √ÚÁ·ÓÈÛÌfi˜ ÀÁ›·˜ (WHO) ¯ÚËÛÈÌÔÔ›ËÛ ÙȘ η̇Ϙ ·Ó¿Ù˘Í˘ ÙˆÓ ∏¶∞ (CDC 2000) Ô˘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‰Â‰Ô̤ӷ ·fi Ù¤ÛÛÂÚȘ ÂıÓÈΤ˜ ‰ÂÈÁÌ·ÙÔÏË„›Â˜ ·È‰ÈÒÓ ÌÂٷ͇ 1963 Î·È 1990 Î·È ÙȘ ÚÔÛ¿ÚÌÔÛ ÁÈ· ¯Ú‹ÛË ·fi ¯ÒÚ˜ ÔÈ Ôԛ˜ ‰ÂÓ ‰È·ı¤ÙÔ˘Ó Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘. ¢ÂÓ ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Ë ÂıÓÈ΋ ÌÂϤÙË ÙÔ˘ 1994 ÁÈ· ·È‰È¿ ¿Óˆ ÙˆÓ 6 ÂÙÒÓ ÏfiÁˆ ¤Í·ÚÛ˘ Ù˘ ·¯˘Û·ÚΛ·˜. ∏ Û‡ÁÎÚÈÛË ÙˆÓ Î·Ì˘ÏÒÓ ÙÔ˘ CDC-WHO Î·È Ù˘ ∞’ ¶∫¶∞ ·ÔηχÙÂÈ fiÙÈ ÔÈ ÂÏÏËÓÈΤ˜ η̇Ϙ ·Ó¿Ù˘Í˘ ‚Ú›ÛÎÔÓÙ·È Ôχ ˘„ËÏfiÙÂÚ· ÁÈ· fiϘ ÙȘ ÌÂÙÚ‹ÛÂȘ. ø˜ ·ÔÙ¤ÏÂÛÌ·, ÔÏÏ¿ ·È‰È¿ ‚Ú›ÛÎÔÓÙ·È Ó· ¤¯Ô˘Ó ·Ó·Ú΋ ·Ó¿Ù˘ÍË Û‡Ìʈӷ Ì ÙȘ η̇Ϙ Ù˘ ∞’ ¶∫¶∞, ÂÓÒ Â›Ó·È ÛÙ· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· Ì ‚¿ÛË ÂΛӘ ÙÔ˘ CDC. ∞ÓÙ›ıÂÙ·, Á›ÓÂÙ·È ˘ÔÂÎÙ›ÌËÛË ÙˆÓ ˘¤Ú‚·ÚˆÓ Î·È ·¯‡Û·ÚÎˆÓ ·È‰ÈÒÓ. √È Î·Ì‡Ï˜ Ù˘ ∞’ ¶∫¶∞ Ô˘ ‚Ú›ÛÎÔÓÙ·È Û‹ÌÂÚ· ÛÙ· ‚È‚ÏÈ¿ÚÈ· ˘Á›·˜ ı· Ú¤ÂÈ ¿ÌÂÛ· Ó· ·ÓÙÈηٷÛÙ·ıÔ‡Ó ·fi ÙȘ η̇Ϙ ÙÔ˘ πÀ¶. °È· ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ ˘¿Ú¯Ô˘Ó Û‹ÌÂÚ· ÌÂÙÚ‹ÛÂȘ ·’ fiÏË ÙË ¯ÒÚ· Ô˘ ÂÈÙÚ¤Ô˘Ó ÙË ‰ËÌÈÔ˘ÚÁ›· ηχÙÂÚˆÓ Î·Ì˘ÏÒÓ ·Ó¿Ù˘Í˘. §¤ÍÂȘ ÎÏÂȉȿ: ∫·Ì‡Ï˜ ·Ó¿Ù˘Í˘, ·È‰È¿, ·¯˘Û·ÚΛ·, ˘ÔıÚ„›·, ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ÂıÓÈÎfi ‰Â›ÁÌ·.
Growth curves for Greek children University of Crete, Medical School, Department of Social Medicine, Clinic of Preventive Medicine and Nutrition Correspondence: Antonis Kafatos kafatos@med.uoc.gr Voutes-Stavrakia Junction, 71 003, Po. Box: 2208, Herakleion, Crete
A. Kafatos Abstract: Physical growth curves enable paediatricians and parents to monitor the pattern of child growth from birth onwards. Growth curves should be derived from large, nationally representative samples. In 1981, the Institute of Child Health (ICH) in Athens conducted a national study and created growth curves for children aged 6-17 years in Greece. Up until the end of 2003 the Greek children’s health booklet contained US Centers for Disease Control (CDC) curves for preschool children and the ICH growth curves for children over the age of 6 years. In 2003, the Greek Ministry of Health replaced these with curves constructed by the A’ Paediatric Clinic of the University of Athens (APCUA), which are based on children living in Athens, but for which very little information about sampling procedures is available. For preschool children it is stated that children were measured in private clinics and health centres, without details of birth weight, the presence of illness or the number of measurements taken on each child. Comparing the APCUA growth curves with CDC-WHO curves for international use and with the ICH curves, the APCUA curves overestimate the proportion of children with stunted growth at every age while underestimating the proportions of overweight and obese children. From the above observations, it is apparent that the growth curves presently in use by the Ministry of Health should be replaced by the original ICH curves for children over the age of 6 years. The ICH growth curves are the most appropriate, since the children in the representative national sample were measured in 1981, before the present epidemic of obesity started. For preschool children, other data are available for more appropriate growth curves. Key words: Growth curves, children, overweight, obese, underweight, national representative sample.
∂ÈÛ·ÁˆÁ‹ ∞·Ú·›ÙËÙÔ ÂÚÁ·ÏÂ›Ô ÁÈ· οı ·È‰›·ÙÚÔ Î·È ÁÔÓÈfi ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ÔÚ›Paediatriki 2007;70:70-74
·˜ Ù˘ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘ ÙÔ˘ ·È‰ÈÔ‡ ·fi ÙË Á¤ÓÓËÛË ¤ˆ˜ Î·È ÙËÓ ÔÏÔÎÏ‹ÚˆÛ‹ Ù˘ Â›Ó·È ÔÈ Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘.
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¢ÂÓ ˘¿Ú¯ÂÈ ·ÌÊÈ‚ÔÏ›· fiÙÈ ÔÈ Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘ Ú¤ÂÈ Ó· Â›Ó·È Î·Ù¿ÏÏËÏ· ۯ‰ȷṲ̂Ó˜ Î·È Ó· ‚·Û›˙ÔÓÙ·È Û ÌÂÙÚ‹ÛÂȘ ·ÓÙÈÚÔÛˆÂ˘ÙÈÎÔ‡ ÂıÓÈÎÔ‡ ‰Â›ÁÌ·ÙÔ˜ ˘ÁÈÒÓ ·È‰ÈÒÓ. √È ∏¶∞ ‹Ù·Ó Ë ÚÒÙË ¯ÒÚ· Ô˘ ‰ËÌÈÔ‡ÚÁËÛ η̇Ϙ ·Ó¿Ù˘Í˘ Ì ÂıÓÈΤ˜ ‰ÂÈÁÌ·ÙÔÏË„›Â˜ (¶›Ó·Î·˜ 1) Î·È ·ÎÔÏÔ‡ıËÛ·Ó ÔÈ µÚÂÙ·ÓÔ› (¶›Ó·Î·˜ 2). √ ¶·ÁÎfiÛÌÈÔ˜ √ÚÁ·ÓÈÛÌfi˜ ÀÁ›·˜ (WHO) ¯ÚËÛÈÌÔÔ›ËÛ ÙȘ ·ÌÂÚÈηÓÈΤ˜ η̇Ϙ ·Ó¿Ù˘Í˘ Ô˘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‰Â‰Ô̤ӷ ·fi Ù¤ÛÛÂÚȘ ÂıÓÈΤ˜ ‰ÂÈÁÌ·ÙÔÏË„›Â˜ ·È‰ÈÒÓ ÛÙȘ ∏¶∞ (fiϘ ÔÈ ÂıÓÈÎfiÙËÙ˜) ÌÂٷ͇ 1963 Î·È 1990 Î·È ¤ÓÙÂ Û˘ÌÏËڈ̷ÙÈΤ˜ ÌÂϤÙ˜ Î·È ÙȘ ÚÔÛ¿ÚÌÔÛ ÁÈ· ¯Ú‹ÛË ·fi ¯ÒÚ˜ ÔÈ Ôԛ˜ ‰ÂÓ ‰È·ı¤ÙÔ˘Ó ‰ÈΤ˜ ÙÔ˘˜ η̇Ϙ ·Ó¿Ù˘Í˘ (CDC 2000) (¢ÂÓ ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Ë ÂıÓÈ΋ ÌÂϤÙË ÙˆÓ ∏¶∞ ÙÔ˘ 1994 ÁÈ· ·È‰È¿ ¿Óˆ ÙˆÓ 6 ÂÙÒÓ ÏfiÁˆ ÂȉËÌÈ΋˜ ¤Í·ÚÛ˘ Ù˘ ·¯˘Û·ÚΛ·˜). ∏ ¯ÒÚ· Ì·˜ ¯ÚËÛÈÌÔÔ›ËÛ ̤¯ÚÈ ÙÔ 2003 ÙȘ η̇Ϙ ·Ó¿Ù˘Í˘ ÙˆÓ ∏¶∞ (CDC) ÁÈ· ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ Î·È ÁÈ· ÙȘ ËÏÈ˘ 6-18 ÂÙÒÓ ÙȘ η̇Ϙ ·Ó¿Ù˘Í˘ ÙÔ˘ πÓÛÙÈÙÔ‡ÙÔ˘ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡. ∏ ϤÔÓ ÛÔ‚·Ú‹ ÚÔÛ¿ıÂÈ· ‰ËÌÈÔ˘ÚÁ›·˜ Î·Ì˘ÏÒÓ ·Ó¿Ù˘Í˘ ÁÈ· ·È‰È¿ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ ‹Ù·Ó ÂΛÓË ÙÔ˘ πÓÛÙÈÙÔ‡ÙÔ˘ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ ÙÔ 1981 (7). √È Î·Ì‡Ï˜ ÙÔ˘ πÀ¶ ¤ÁÈÓ·Ó Û ڿÁÌ·ÙÈ ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›ÁÌ· 27.000 ·È‰ÈÒÓ ËÏÈΛ·˜ 6-18 ÂÙÒÓ ·’ fiÏË ÙË ¯ÒÚ· ÙÔ 1981. ∞˘Ù‹ ‹Ù·Ó Ë Ï¤ÔÓ Î·Ù¿ÏÏËÏË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô˜ Ó· Á›ÓÔ˘Ó Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘ ÁÈ·Ù› ‰ÂÓ Â›¯Â ·Ú¯›ÛÂÈ ·ÎfiÌ· Ë ÂȉËÌ›· Ù˘ ·¯˘Û·ÚΛ·˜. ∞Í›˙ÂÈ Û˘Á¯·ÚËÙ‹ÚÈ· ÙÔ πÀ¶ Ì ÙÔÓ ÙfiÙ Úfi‰ÚÔ ÙÔ˘ ™‡ÚÔ ¢ÔÍÈ¿‰Ë ÁÈ· ÙËÓ ÙÂÚ¿ÛÙÈ·˜ ÛËÌ·Û›·˜ ÚÔÛÊÔÚ¿ ÙÔ˘, fiˆ˜ ·Ô‰ÂÈÎÓ‡ÂÙ·È Û‹ÌÂÚ·. ™Â ÚfiÛÊ·ÙË ‰ËÌÔÛ›Â˘ÛË (9) Ê·›ÓÂÙ·È Ë ÌÂÁ¿ÏË ·‡ÍËÛË ÙÔ˘ ‚¿ÚÔ˘˜ ÙˆÓ ·È‰ÈÒÓ ·fi ÙÔ 1982 ̤¯ÚÈ ÙÔ 2002 Û ۇÁÎÚÈÛË Ì ÙËÓ Ôχ ÌÈÎÚ‹ ·‡ÍËÛË ÙÔ˘ ·Ó·ÛÙ‹Ì·ÙÔ˜ ηٿ ÙËÓ ›‰È· ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô (¶›Ó·Î·˜ 3). √ ¶›Ó·Î·˜ 4 Û˘ÓÔ„›˙ÂÈ ÙȘ ÌÂϤÙ˜ Ô˘ ¤ÁÈÓ·Ó ÛÙË ¯ÒÚ· Ì·˜ ÁÈ· ‰ËÌÈÔ˘ÚÁ›· Î·Ì˘ÏÒÓ ·Ó¿Ù˘Í˘ ÙˆÓ ·È‰ÈÒÓ (7,11,12,14).
¶›Ó·Î·˜ 2. µÚÂÙ·ÓÈΤ˜ η̇Ϙ ·Ó¿Ù˘Í˘ (5,6) ñ ∫·Ì‡Ï˜ Tanner-Whitehouse 1966 ñ ∞ËÚ¯·ÈˆÌ¤Ó˜ ÏfiÁˆ Ù˘ ·‡ÍËÛ˘ ‡„Ô˘˜ ÙȘ ÙÂÏÂ˘Ù·›Â˜ ‰ÂηÂٛ˜ ñ ∫·Ì‡Ï˜ Buckler-Tanner ÚÔÛ·ÚÌfiÛÙËÎ·Ó ÙÔ 1999 Î·È ¤Ï·‚·Ó ˘’ fi„ÈÓ ÙËÓ ·‡ÍËÛË ‚¿ÚÔ˘˜ - ‡„Ô˘˜ ñ ∫·Ì‡Ï˜ UK 90 - ÚԤ΢„·Ó ·fi ÙȘ ÚÔËÁËı›Û˜ ÌÂϤÙ˜. Œ¯Ô˘Ó ÌÂÁ¿ÏË ·ÍÈÔÈÛÙ›· Û˘ÁÎÚÈÓfiÌÂÓ˜ Ì ÙȘ ·Ï·ÈfiÙÂÚ˜ (ËÏÈ˘ 2-20 ¤ÙË). ™ÙËÓ ÂÊ˂›· ‰È·ÛÙ·˘ÚÒÓÔÓÙ·È Û˘Ó‹ıˆ˜ ÔÈ ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ ÏfiÁˆ ÌÂÁ¿ÏˆÓ ‰È·ÊÔÚÒÓ ÛÙËÓ ËÏÈΛ· ¤Ó·Ú͢ Ù˘ ÂÚÈfi‰Ô˘ Ù·¯Â›·˜ ·Ó¿Ù˘Í˘ (growth spurt). ÃÚËÛÈÌÔÔÈ‹ıËÎ·Ó ‰Â‰Ô̤ӷ ̤¯ÚÈ ÙÔ 1990 ÏfiÁˆ Ù˘ ÂȉËÌ›·˜ ·¯˘Û·ÚΛ·˜ ÙËÓ ÙÂÏÂ˘Ù·›· ‰ÂηÂÙ›· ñ √È ‚ÚÂÙ·ÓÈΤ˜ η̇Ϙ ·Ó·ÊÔÚ¿˜ [UK 90] ÁÈ· ¢ª™ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ‰ÈÂıÓÒ˜ ÁÈ· ÂÎÙ›ÌËÛË ÙˆÓ ˘¤Ú‚·ÚˆÓ Î·È ·¯‡Û·ÚÎˆÓ ·È‰ÈÒÓ (IOTF)
∆Ô 2003 ÂÌÊ·Ó›ÛÙËÎ·Ó Ó¤Â˜ η̇Ϙ ·Ó¿Ù˘Í˘ ÛÙ· ‚È‚ÏÈ¿ÚÈ· ˘Á›·˜ ÙÔ˘ ÀÔ˘ÚÁ›Ԣ ÀÁ›·˜ Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙËÓ ∞ã ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ∞Á›· ™ÔÊ›·. ∞ÚÁfiÙÂÚ· ÙÔ 2004, Û¯ÂÙÈ΋ ‰ËÌÔÛ›Â˘ÛË ·Ó¤ÊÂÚ fiÙÈ ÙÔ ‰Â›ÁÌ· ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ÙËÓ ÂÚÈÔ¯‹ ∞ıËÓÒÓ, ·ÏÏ¿ ¯ˆÚ›˜ Ó· ‰›‰ÔÓÙ·È ÏËÚÔÊÔڛ˜ ·Ó ÚfiÎÂÈÙ·È ÁÈ· ·È‰È¿ ÌÈÎÚ¿ ‹ ÌÂÁ¿Ï· ÁÈ· ÙËÓ ËÏÈΛ· ΢‹Ûˆ˜, ıËÏ¿˙ÔÓÙ· ‹ ÌË Î·ıÒ˜ Î·È ÁÈ· ÙȘ ÌÂıfi‰Ô˘˜ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó (1-3). ™ÙË ÌÂϤÙË ·˘Ù‹ Ù· 10.000 ÂÚ›Ô˘ ·È‰È¿ Î·È ¤ÊË‚ÔÈ Ô˘ ÌÂÙÚ‹ıËηÓ, Èı·ÓfiÓ Ó· ÌËÓ ·ÓÙÈÚÔÛˆÂ‡Ô˘Ó Î·Ó Ù· ·È‰È¿ Ù˘ ÂÚÈÔ¯‹˜ ∞ıËÓÒÓ ÁÈ·Ù› ‰ÂÓ ÂÚÈÁÚ¿ÊÂÙ·È Ë ÌÂıÔ‰ÔÏÔÁ›· ÂÈÏÔÁ‹˜ ‰Â›ÁÌ·ÙÔ˜ ÛÙȘ ‰‡Ô ‰ËÌÔÛȇÛÂȘ Ù˘ ›‰È·˜ ∫ÏÈÓÈ΋˜ (13). √‡Ù ÂÚÈÁÚ¿ÊÂÙ·È ÙÔ ‰Â›ÁÌ· ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ô˘ Ï‹ÊıËΠ·fi ȉȈÙÈΤ˜ ÎÏÈÓÈΤ˜, ȉȈÙÈο È·ÙÚ›· Î·È Î¤ÓÙÚ· ˘Á›·˜. ¶Ò˜ ÂÂϤÁËÛ·Ó; ∞ÔÎÏ›ÛÙËÎ·Ó Ù· ¿ÚÚˆÛÙ· ·È‰È¿; ¢ÂÓ ·Ó·Ê¤ÚÔÓÙ·È Ù· ÎÚÈÙ‹ÚÈ· ÂÈÏÔÁ‹˜ ÙˆÓ ·È‰ÈÒÓ, Ô‡Ù ·Ó·Ê¤ÚÂÙ·È Ô˘ ‚Ú›ÛÎÔÓÙ·È Ù· È·ÙÚ›· Î·È Î¤ÓÙÚ· ˘Á›·˜ Î·È fiÛ· ·fi ·˘Ù¿ Û˘ÌÌÂÙ›¯·Ó ÛÙË ÌÂϤÙË. ¢ÂÓ ·Ó·Ê¤ÚÂÙ·È Â›Û˘, ·Ó οı ·È‰› ÌÂÙÚ‹ıËΠ̛· ÊÔÚ¿ ‹ ·Ó οı ÊÔÚ¿ Ô˘ ÂÈÛÎÂÙfiÙ·Ó ÙÔ È·ÙÚÂ›Ô ‹ ΤÓÙÚÔ ˘Á›·˜ ηٷÁÚ·ÊfiÙ·Ó ˆ˜ ηÈÓÔ‡ÚÁÈÔ ·È‰›. ∞Ó ‰ÂÓ Û˘Ó¤‚·ÈÓ ·˘Ùfi, fiÛ· ·È‰È¿ ÂÍÂÙ¿ÛıËÎ·Ó Â·ÓÂÈÏËÌ̤ӷ - ‰È·¯ÚÔÓÈο;
¶›Ó·Î·˜ 1. ªÂϤÙ˜ ÙˆÓ ∏¶∞ ÁÈ· η̇Ϙ ·Ó¿Ù˘Í˘ (4) ŒÙÔ˜ 1963-65 1966-70 1971-74 1976-80 1988-94 1960-94
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NHES II ∂ıÓÈ΋ 6-11 ÂÙÒÓ NHES III -//12-17 ÂÙÒÓ NHANES I -//1-18 ÂÙÒÓ NHANES II -//6 ÌËÓÒÓ-18 ÂÙÒÓ NHANES III -//2 ÌËÓÒÓ-18 ÂÙÒÓ ¶¤ÓÙÂ Û˘ÌÏËڈ̷ÙÈΤ˜ ÌÂϤÙ˜ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ‚ÚÂÊÒÓ
¶·È‰È¿ 3632 3223 3659 3921 3698 >23000 ¶·È‰È·ÙÚÈ΋ 2007;70:70-74
72
∞. ∫·Ê¿ÙÔ˜
¶›Ó·Î·˜ 3. ™ˆÌ·ÙÔÌÂÙÚ‹ÛÂȘ Î·È ÔÛÔÛÙ¿ ˘¤Ú‚·ÚˆÓ Î·È ·¯‡Û·ÚÎˆÓ ·È‰ÈÒÓ Ù˘ ∫Ú‹Ù˘, ËÏÈΛ·˜ 9 Ì 17 ÂÙÒÓ ÙÔ 1982 Î·È 2002 (9) 1982 (¡=528)
2002 (¡=620)
∞ÏÏ·Á‹
ª¤ÛË ∆ÈÌ‹ µ¿ÚÔ˜ (kg) ⁄„Ô˜ (cm) ¢ª™ (kg/m2) º˘ÛÈÔÏÔÁÈο À¤Ú‚·Ú· ¶·¯‡Û·Úη À¤Ú‚·Ú· & ¶·¯‡Û·Úη
45,8 150,8 19,4 % 79 16,5 4,2 20,6
50,2 152,5 21,0
% 9,6 1,1 8,2
60 27 12,7 39,7
™ÙÔ ›‰ÈÔ ¿ÚıÚÔ ÂÈÛËÌ·›ÓÂÙ·È Ë ÌÂÁ¿ÏË ·‡ÍËÛË ÙÔ˘ ‚¿ÚÔ˘˜ ·ÁÔÚÈÒÓ Î·È ÎÔÚÈÙÛÈÒÓ Ù· ÙÂÏÂ˘Ù·›· 20 ¯ÚfiÓÈ·. Ÿˆ˜ Ê·›ÓÂÙ·È ÛÙȘ ∂ÈÎfiÓ˜ 1 & 2 ÔÈ Î·Ì‡Ï˜ ·˘Ù¤˜ ÙȘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ˘ÔÙÈÌÔ‡Ó Ù· ̤ÁÈÛÙ· ÙÔÓ ·ÚÈıÌfi ÙˆÓ ·È‰ÈÒÓ Ì ·˘ÍË̤ÓÔ ‚¿ÚÔ˜ Î·È ·¯˘Û·ÚΛ· Î·È ˘ÂÚÂÎÙÈÌÔ‡Ó ÙÔÓ ·ÚÈıÌfi ÙˆÓ ·È‰ÈÒÓ Ì ·Ó·Ú΋ ·Ó¿Ù˘ÍË. √ ‰Â›ÎÙ˘ Ì¿˙·˜ ÛÒÌ·ÙÔ˜ (¢ª™) ÙfiÛÔ ÛÙ· ·ÁfiÚÈ· fiÛÔ Î·È ÛÙ· ÎÔÚ›ÙÛÈ· Ù˘ 3˘ ∂.£. Ù˘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ·ÓÙÈÛÙÔȯ› ÛÙË 10Ë ÙÔ˘ CDC ÂÓÒ Ë 97Ë ∂.£. Â›Ó·È Ôχ ÈÔ ¿Óˆ ·fi ÙËÓ 97Ë ÙˆÓ Î·Ì˘ÏÒÓ ÙÔ˘ CDC ÛÙȘ ∏¶∞. ∆Ô ›‰ÈÔ ·Ú·ÙËÚÂ›Ù·È Î·È ÛÙËÓ 50‹ ∂.£.. °È’ ·˘ÙfiÓ ·ÎÚÈ‚Ò˜ ÙÔÓ ÏfiÁÔ ˘ÔÙÈÌ¿Ù·È ÙÔ ÔÛÔÛÙfi ·¯˘Û·ÚΛ·˜ Ì ‚¿ÛË ÙȘ η̇Ϙ Ù˘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜, ÂÓÒ ˘ÂÚÂÎÙÈÌ¿Ù·È ÙÔ ÔÛÔÛÙfi ÙˆÓ ·È‰ÈÒÓ Ì ·Ó·Ú΋ ·Ó¿Ù˘ÍË Î·È ˘ÔıÚ„›·. ™ÙË ‰ËÌÔÛ›Â˘ÛË Ù˘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔÓ›˙ÂÙ·È fiÙÈ ·ıÔÏÔÁÈο ·˘ÍË̤ÓÔ ¢ª™ Î·È ·¯˘Û·ÚΛ· ¤¯Ô˘Ó fiÛ· ·ÁfiÚÈ· Â›Ó·È ¿Óˆ ·fi ÙËÓ
50‹ ∂.£. ÙˆÓ Î·Ì˘ÏÒÓ ÙÔ˘˜ (∂ÈÎfiÓ· 3) ‹ ¿Óˆ ·fi ÙËÓ 75Ë ∂.£. ÁÈ· Ù· ÎÔÚ›ÙÛÈ·, Û‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· ÙÔ˘ IOTF (International Obesity Task Force) (8). √È Î·Ì‡Ï˜ fï˜, Ù˘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ‰ËÌÈÔ˘ÚÁÔ‡Ó Û‡Á¯˘ÛË ÛÙÔ˘˜ ·È‰›·ÙÚÔ˘˜ Î·È ÁÔÓ›˜ ÁÈ·Ù› Ê˘ÛÈÔÏÔÁÈο ıˆÚÔ‡ÓÙ·È Ù· ·ÁfiÚÈ· Ô˘ Â›Ó·È Î¿Ùˆ Ù˘ 50‹˜ ∂.£. Î·È Î¿Ùˆ Ù˘ 75˘ ÁÈ· Ù· ÎÔÚ›ÙÛÈ·. ∞ÓÙ›ıÂÙ· ÔÈ Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘ ÙÔ˘ πÀ¶ Ô˘ ¤ÁÈÓ·Ó Û ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›ÁÌ· ÂÏÏËÓÈÎÔ‡ ÏËı˘ÛÌÔ‡ ËÏÈΛ·˜ 6-18 ÂÙÒÓ Î·È Û ηٿÏÏËÏË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ (1981), ÚÈÓ ‰ËÏ·‰‹ ÙËÓ ¤Ó·ÚÍË Ù˘ ÂȉËÌ›·˜ Ù˘ ·¯˘Û·ÚΛ·˜, ·ÓÙÈÛÙÔÈ¯Ô‡Ó Ï‹Úˆ˜ ÛÙȘ ·ÓÙ›ÛÙÔȯ˜ ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ ÙˆÓ Î·Ì˘ÏÒÓ ÙÔ˘ CDC (ÔÈ ÙÈ̤˜ ÙˆÓ ÂηÙÔÛÙÈ·›ˆÓ ı¤ÛÂˆÓ ÙÔ˘ ‚¿ÚÔ˘˜ Î·È ÙÔ˘ ·Ó·ÛÙ‹Ì·ÙÔ˜ ÙˆÓ ·È‰ÈÒÓ ÛÙȘ ‰È¿ÊÔÚ˜ ËÏÈ˘ ‰ÂÓ ‰È¤ÊÂÚ·Ó ÛËÌ·ÓÙÈο - ANOVA, p>0,05). ŸÛÔÓ ·ÊÔÚ¿ ÙȘ η̇Ϙ ·Ó¿Ù˘Í˘ ηٿ ÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, Ë ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¯ÚËÛÈÌÔÔ›ËÛ 139 ·ÁfiÚÈ· Î·È ·ÚfiÌÔÈÔ ·ÚÈıÌfi ÎÔÚÈÙÛÈÒÓ. ∞Ó ÌÂÙÚ‹ıËÎ·Ó ÌfiÓÔ Ì›· ÊÔÚ¿ (‰Â›ÁÌ· ÙÔÌ‹˜), Â›Ó·È ÂÍ·ÈÚÂÙÈο ÌÈÎÚfi ‰Â›ÁÌ· ÁÈ· Ó· ·ÚÔ˘ÛÈ·Ûı› ˆ˜ ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ÁÈ· ÂıÓÈΤ˜ η̇Ϙ. ∏ ÂÚÈÁÂÓÓËÙÈ΋ ÌÂϤÙË ∫Ú‹Ù˘ Ì 1.200 ÂÚ›Ô˘ ˘ÁÈ‹ ·È‰È¿ ‰È·¯ÚÔÓÈ΋˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÁÈ· 6 ¯ÚfiÓÈ· (11), Â›Ó·È Ôχ ÈÔ ÏÔÁÈÎfi ‰Â›ÁÌ·, ÂÓÙÔ‡ÙÔȘ ‰ÂÓ ÈÛ¯˘ÚÈ˙fiÌ·ÛÙ ˆ˜ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÂıÓÈΤ˜ η̇Ϙ ·Ó¿Ù˘Í˘.
™˘ÌÂÚ¿ÛÌ·Ù· ∆Ô ÂÍ·ÈÚÂÙÈο ÛÔ‚·Úfi Úfi‚ÏËÌ· Ù˘ ·¯˘Û·ÚΛ·˜ Ô˘ ¤¯ÂÈ ¿ÚÂÈ ÂȉËÌÈΤ˜ ‰È·ÛÙ¿ÛÂȘ ˘ÔÙÈÌ¿Ù·È ·fi ÙȘ ˘¿Ú¯Ô˘Û˜ Û‹ÌÂÚ· η̇Ϙ ·Ó¿Ù˘Í˘ ÛÙ· ‚È‚ÏÈ¿ÚÈ· ˘Á›·˜ ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘
¶›Ó·Î·˜ 4. ªÂϤÙ˜ ÛÙËÓ ∂ÏÏ¿‰· ÁÈ· η̇Ϙ ·Ó¿Ù˘Í˘ πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡
∂ıÓÈο ÚfiÙ˘· ·Ó¿Ù˘Í˘ ¶·È‰È·ÙÚÈ΋ 1986;49:1-15 ∂ıÓÈ΋ ‰ÂÈÁÌ·ÙÔÏË„›· Û 230 Û¯ÔÏ›· - ÕÓÔÈÍË 1981 ∏ÏÈΛ· 6-18 ÂÙÒÓ, 27.000 ·È‰È¿
¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋
∆Ô Ê˘ÛÈÔÏÔÁÈÎfi ·È‰›. ¶ÚfiÙ˘· ÛÙÔȯ›· ·Ó¿Ù˘Í˘ £ÂÛÛ·ÏÔÓ›ÎË, 1990 1148 ¡ÂÔÁÓ¿ 0-30 ÌËÓÒÓ, ∞ı‹Ó·, 1984-1985 3419 ·È‰È¿ 3-6 ÂÙÒÓ, µÚÂÊÔÓËÈ·ÎÔ›-¶·È‰ÈÎÔ› ÛÙ·ıÌÔ› 7926 ·È‰È¿ 6-17 ÂÙÒÓ, ‰Â›ÁÌ· 38 Û¯ÔÏ›ˆÓ ∫·Ì‡Ï˜ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘ ·È‰ÈÒÓ ‚ÚÂÊÈ΋˜ Î·È ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ô˘ ÚԤ΢„·Ó ·fi ‰È·¯ÚÔÓÈ΋ ·Ú·ÎÔÏÔ‡ıËÛ‹ ÙÔ˘˜ ¶·È‰È·ÙÚÈ΋ 2000;63:391-407 1142 ·È‰È¿ 0-6 ÂÙÒÓ, ∫Ú‹ÙË, 1988-1995, ‰È¿ÚÎÂÈ· 7 ¯ÚfiÓÈ· ÀÁ›·, ‰È·ÙÚÔÊ‹ & ·Ó¿Ù˘ÍË ·È‰ÈÒÓ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ ÷ÓÈ¿, 2004 6663 ·È‰È¿ ËÏÈΛ·˜ 5 ¤ˆ˜ 17,5 ÂÙÒÓ, 1987-2004
¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘ ∫ÏÈÓÈ΋ ¶ÚÔÏËÙÈ΋˜ π·ÙÚÈ΋˜ & ¢È·ÙÚÔÊ‹˜
Paediatriki 2007;70:70-74
73
∫·Ì‡Ï˜ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘ ÛÙËÓ ∂ÏÏ¿‰·
™¯ËÌ·ÙÈ΋ Û‡ÁÎÚÈÛË. ª™ Î·Ì˘ÏÒÓ ·Ó¿Ù˘Í˘ CDC Î·È ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ∞ıËÓÒÓ
™¯ËÌ·ÙÈ΋ Û‡ÁÎÚÈÛË. ª™ Î·Ì˘ÏÒÓ ·Ó¿Ù˘Í˘ CDC Î·È ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ∞ıËÓÒÓ
97%
97%
50%
50%
3% 3%
∂ÈÎfiÓ· 1. ∫·Ì‡Ï˜ ¢ª™ ÎÔÚÈÙÛÈÒÓ ·fi CDC Î·È ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ıËÓÒÓ.
∂ÈÎfiÓ· 2. ∫·Ì‡Ï˜ ¢ª™ ·ÁÔÚÈÒÓ ·fi CDC Î·È ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ıËÓÒÓ.
ÀÔ˘ÚÁ›Ԣ ÀÁ›·˜. √È Î·Ì‡Ï˜ ·˘Ù¤˜ ‰ÂÓ ¤ÁÈÓ·Ó Û ηٿÏÏËÏÔ ÏËı˘ÛÌÈ·Îfi ‰Â›ÁÌ· Ô‡Ù ÙËÓ Î·Ù¿ÏÏËÏË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô. ∆Ô πÀ¶ ¤Î·Ó ÂÍ·ÈÚÂÙÈο ÛÔ˘‰·›Ô ¤ÚÁÔ ÙÔ 1981 Ì ÙË ‰ËÌÈÔ˘ÚÁ›· Î·Ì˘ÏÒÓ ·Ó¿Ù˘Í˘ ·fi ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›ÁÌ· 27.000 ·È‰ÈÒÓ ËÏÈΛ·˜ 6-18 ¯ÚfiÓˆÓ ·’ fiÏË ÙË ¯ÒÚ·. √È Î·Ì‡Ï˜ ·˘Ù¤˜ ‹Ù·Ó ÛÙ· ‚È‚ÏÈ¿ÚÈ· ˘Á›·˜ ÙÔ˘ ·È‰ÈÔ‡ ̤¯ÚÈ ÙÔ Ù¤ÏÔ˜ ÙÔ˘ 2003. √È Î·Ì‡Ï˜ ·˘Ù¤˜ ı· Ú¤ÂÈ Ó· ·ӤÏıÔ˘Ó ¿ÌÂÛ·, ·ÊÔ‡ ÂÈÛ·¯ıÔ‡Ó Ù· ‰Â‰Ô̤ӷ ÙˆÓ 27.000 ·È‰ÈÒÓ ÛÙÔ˘˜ ˘ÔÏÔÁÈÛÙ¤˜ Î·È Ì ۇÁ¯ÚÔÓ˜ ÌÂıfi‰Ô˘˜ ‚ÈÔÛÙ·ÙÈÛÙÈ΋˜ Á›ÓÔ˘Ó Í·Ó¿ ÔÈ Î·Ì‡Ï˜ ·Ó¿Ù˘Í˘. ¶ÚÈÓ ·fi 25 ¯ÚfiÓÈ· ‰ÂÓ Ú·ÁÌ·ÙÔÔÈ›ÙÔ Ë Ì¤ÙÚËÛË Ù˘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘. ™‹ÌÂÚ· fï˜, ıˆÚÂ›Ù·È ÂÍ·ÈÚÂÙÈο ÛÔ˘‰·›· ̤ÙÚËÛË ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ÎÂÓÙÚÈ΋˜ ·¯˘Û·ÚΛ·˜. ∏ ¤Î‰ÔÛË ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘ “ÀÁ›·, ‰È·ÙÚÔÊ‹ Î·È ·Ó¿Ù˘ÍË ·È‰ÈÒÓ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜” ÙÔ 2004 (14), ÂÚÈÏ·Ì‚¿ÓÂÈ Î·Ì‡Ï˜ ÂÚÈ̤ÙÚÔ˘ ̤Û˘, fiˆ˜ Î·È ÔÈ ‰ËÌÔÛȇÛÂȘ ÙˆÓ ™¿‚‚· Î·È Û˘Ó. (13), Û ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›ÁÌ· ·È‰ÈÒÓ Ù˘ ∫‡ÚÔ˘. ¶ÏËı˘ÛÌȷΤ˜ ÔÌ¿‰Â˜ ·È‰ÈÒÓ ·ÎfiÌ· ·fi ÙËÓ ÚÔÛ¯ÔÏÈ΋ ËÏÈΛ·, ‚Ú¤ıËÎ·Ó Ó· ¤¯Ô˘Ó ‰ÈÏ¿ÛÈ· ›‰· ÈÓÛÔ˘Ï›Ó˘ Û ÂΛӷ Ô˘ ›¯·Ó ÂÚ›ÌÂÙÚÔ Ì¤Û˘ ¿Óˆ ·fi ÙËÓ 85Ë ∂.£. Û ۇÁÎÚÈÛË Ì’ ·˘Ù¿ Ô˘ ‹Ù·Ó οو ·fi ÙËÓ 15Ë ∂.£. (10). °È’ ·˘Ùfi ÌÂ
Ó¤· ÂıÓÈ΋ ‰ÂÈÁÌ·ÙÔÏË„›· ·È‰ÈÒÓ fiÏ˘ Ù˘ ¯ÒÚ·˜ Ô˘ ı· ÛÙԯ‡ÂÈ ÛÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÂȤ‰Ô˘ ˘Á›·˜, ıÚ¤„˘ Î·È ·Ó¿Ù˘Í˘, Ë ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ Ú¤ÂÈ ™¯ËÌ·ÙÈ΋ Û‡ÁÎÚÈÛË. ª™ Î·Ì˘ÏÒÓ ·Ó¿Ù˘Í˘ ∞’ ¶∫¶∞ Î·È ÔÚ›ˆÓ ˘¤Ú‚·ÚˆÓ Î·È ·¯‡Û·ÚÎˆÓ ·È‰ÈÒÓ ‚¿ÛË ÙÔ˘ IOTF
¢ª™ - ∞ÁfiÚÈ·
¢ª™ 30 kg/m
2
¢ª™ 25 kg/m 2
∂ÈÎfiÓ· 3. ™¯ËÌ·ÙÈ΋ Û‡ÁÎÚÈÛË Î·Ì˘ÏÒÓ ¢ª™ ·ÁÔÚÈÒÓ ·fi CDC Î·È ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞ıËÓÒÓ. ¶·È‰È·ÙÚÈ΋ 2007;70:70-74
74
∞. ∫·Ê¿ÙÔ˜
Ó· ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙȘ ÌÂÙÚ‹ÛÂȘ. √È Î·Ì‡Ï˜ ÂÚÈ̤ÙÚÔ˘ ̤Û˘ Ú¤ÂÈ Ó· ÌÔ˘Ó ÛÙ· ‚È‚ÏÈ¿ÚÈ· ˘Á›·˜ ÙÔ˘ ·È‰ÈÔ‡ ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ‚·ıÌÔ‡ ÎÂÓÙÚÈ΋˜ ·¯˘Û·ÚΛ·˜.
µÈ‚ÏÈÔÁÚ·Ê›· 1. ÃÈÒÙ˘ ¢, ∆Û›ÊÙ˘ °, ÷Ù˙ËÛ˘ÌÂÒÓ ª, ª·ÓÈ¿ÙË-ÃÚËÛÙ›‰Ë ª, ∫Ú›ÎÔ˜ •, ¢¿ÎÔ˘-µÔ˘ÙÂÙ¿ÎË ∞. ∞Ó¿ÛÙËÌ· Î·È ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜ ÂÏÏËÓÔ·›‰ˆÓ ËÏÈΛ·˜ 0-18 ÂÙÒÓ (20002001): Û‡ÁÎÚÈÛË Ì ‰Â‰Ô̤ӷ ÌÂϤÙ˘ 1978-1979. ¢ÂÏÙ›Ô ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋˜ 2003;50:136-156. 2. ÃÈÒÙ˘ ¢, ∫Ú›ÎÔ˜ •, ∆Û›ÊÙ˘ °, ÷Ù˙ËÛ˘ÌÂÒÓ ª, ª·ÓÈ¿ÙË-ÃÚËÛÙ›‰Ë ª, ¢¿ÎÔ˘-µÔ˘ÙÂÙ¿ÎË ∞. ¢Â›ÎÙ˘ Ì¿˙·˜ ÛÒÌ·ÙÔ˜ (BMI) Î·È ÔÛÔÛÙfi ·¯˘Û·ÚΛ·˜ Û ¿ÙÔÌ· Ù˘ ¢ڇÙÂÚ˘ ÂÚÈÔ¯‹˜ ∞ıËÓÒÓ, ËÏÈΛ·˜ 0-18 ÂÙÒÓ. ¢ÂÏÙ›Ô Õ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ 2004;51:139-154. 3. ÃÚÔ‡ÛÔ˜ °¶. ¶·È‰È΋ ·¯˘Û·ÚΛ· ÛÙËÓ ∂ÏÏ¿‰·. ¶·È‰È·ÙÚÈ΋ £Âڷ¢ÙÈ΋ ∂ÓË̤ڈÛË 2003; 225-234. 4. National Center for Health Statistics. [Webpage, Internet]. Growth Charts for the United States: Methods and Development, CDC 2000:http://www.cdc.gov/growthcharts 5. Cole TJ, Freeman JV, Preece MA. Body mass index reference curves for the UK, 1990. Arch Dis Child 1995;73(1):25-29. 6. Cameron N. British growth charts for height and weight with recommendations concerning their use in axiological assessment. Ann Hum Biol 2002; 29(1):1-10.
“√ÓÂÈÚ‡ÔÌ·È ÙËÓ ∂ÈÚ‹ÓË”, Lina Maria Ocampo, 12 ¯ÚÔÓÒÓ, ∫ÔÏÔÌ‚›· ¶·È‰› Ì ÌÂÚÈ΋ fiÚ·ÛË “∆· Ù˘ÊÏ¿ ·È‰È¿ ˙ˆÁÚ·Ê›˙Ô˘Ó”, ÕÓÓ· §·Ô˘Ù¿ÚË°ÎÚÈÙ˙¿Ï·, ∞ı‹Ó· 2006
“Dreaming with the Peace”, Lina Maria Ocampo, 12 years old, Colombia Partially blind child “Blind children paint”, Anna Laoutari-Gritzala, Athens 2006
Paediatriki 2007;70:70-74
7. ª·Ù˙·ÁÚÈÒÙË-ªÂ˚ÌÂÚ›‰Ë ª, ¶·ÓÙ·˙›‰Ë˜ ¡, ¢ÔÍÈ¿‰Ë˜ ™, ƒ·Ê·‹Ï ª. ∂ıÓÈο ÚfiÙ˘· ·Ó¿Ù˘Í˘: µ¿ÚÔ˜ Î·È ‡„Ô˜ ·È‰ÈÎÔ‡ ÏËı˘ÛÌÔ‡ ÛÙËÓ ∂ÏÏ¿‰·. ¶·È‰È·ÙÚÈ΋ 1986; 49:1-15. 8. Lobstein T, Baur L, Uauy R; IASO International Obesity TaskForce. Obesity in children and young people: a crisis in public health. Report to the WHO. Obes Rev 2004; 5(1 Suppl):S4-S104. 9. Magkos F, Manios Y, Christakis G, Kafatos AG. Secular trends in cardiovascular risk factors among school-aged boys from Crete, Greece, 1982-2002. Eur J Clin Nutr 2005; 59:1-7. 10. ªÔÛ¯·Ó‰Ú¤· I, ÷Ù˙‹˜ X, ª·ÌÌ¿˜ I, ªÂÚÙÛÈ¿˜ °, §ÈÓ·Ú‰¿Î˘ ª, ∫˘Úȷο΢ ª, et al. ¢Â›ÎÙ˜ ·¯˘Û·ÚΛ·˜ Î·È ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ Û ·È‰È¿ ·ÁÚÔÙÈ΋˜ ÔÚÂÈÓ‹˜ ÂÚÈÔ¯‹˜ Ù˘ ∫Ú‹Ù˘. ¶·È‰È·ÙÚÈ΋ 2003;66:264-277. 11. §ÈÓ·Ú‰¿Î˘ ª, ªÔÛ¯·Ó‰Ú¤· I, ∫·Ê¿ÙÔ˜ ∞. ∫·Ì‡Ï˜ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘ ·È‰ÈÒÓ ‚ÚÂÊÈ΋˜ Î·È ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ù˘ ∫Ú‹Ù˘ Ô˘ ÚԤ΢„·Ó ·fi ‰È·¯ÚÔÓÈ΋ ·Ú·ÎÔÏÔ‡ıËÛ‹ ÙÔ˘˜. ¶·È‰È·ÙÚÈ΋ 2000;63:391-407. 12. ∞˚‚¿˙˘ µ. ∆Ô Ê˘ÛÈÔÏÔÁÈÎfi ·È‰› - ¶ÚfiÙ˘· ÛÙÔȯ›· ·Ó¿Ù˘Í˘. £ÂÛÛ·ÏÔÓ›ÎË 1990. 13. Savva SC, Kourides Y, Tornaritis M, Epiphaniou-Savva M, Tafouna P, Kafatos A. Reference growth curves for Cypriot children 6 to 17 years of age. Obes Res 2001;9:754-762. 14. ∫·Ê¿ÙÔ˜ ∞. ÀÁ›·, ¢È·ÙÚÔÊ‹ Î·È ·Ó¿Ù˘ÍË ·È‰ÈÒÓ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜. ¡Ô̷گȷ΋ ∞˘ÙÔ‰ÈÔ›ÎËÛË Ã·Ó›ˆÓ ¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘, ÂΉfiÙ˜. ÷ÓÈ¿: 2004.
∫§π¡π∫√ ∫√Àπ∑
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¡ÂÊÚÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ “¶. Î·È ∞. ∫˘ÚÈ·ÎÔ‡”
K. ™ÙÂÊ·Ó›‰Ë˜, µ. ∞ÛÎËÙ‹
∞ÁfiÚÈ ËÏÈΛ·˜ 8 ÂÙÒÓ ·ÚÔ˘Û›·Û ̷ÎÚÔÛÎÔÈ΋ ·ÈÌ·ÙÔ˘Ú›·, ‰È·ÚΛ·˜ ‰‡Ô ËÌÂÚÒÓ Î·È ÛÙË Û˘Ó¤¯ÂÈ· ÂÈ̤ÓÔ˘Û· ÌÈÎÚÔÛÎÔÈ΋ ·ÈÌ·ÙÔ˘Ú›·. ∆Ô ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi ‹Ù·Ó ÂχıÂÚÔ. ¢ÂÓ ·Ó·Ê¤ÚıËΠÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ÓÂÊÚÔ¿ıÂÈ·˜ ‹ ¿ÏÏÔ˘ ¯ÚfiÓÈÔ˘ ÓÔÛ‹Ì·ÙÔ˜. ∞fi ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠ‡„Ô˜ Î·È ‚¿ÚÔ˜ ÛÙËÓ 50‹ ÂηÙÔÛÙÈ·›· ı¤ÛË, Ê˘ÛÈÔÏÔÁÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË Î·È Î·Ù¿ Û˘ÛÙ‹Ì·Ù· ÂͤٷÛË ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·, ÂÎÙfi˜ ·fi ÙËÓ ‡·ÚÍË ÎÈÚÛÔ΋Ï˘ ·ÚÈÛÙÂÚ¿. ∏ ÁÂÓÈ΋ ·›Ì·ÙÔ˜ Î·È Ô ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ (Ô˘Ú›·, ÎÚ·ÙÈÓ›ÓË, Ô˘ÚÈÎfi Ô͇, ÁÏ˘Îfi˙Ë, ·Û‚¤ÛÙÈÔ, οÏÈÔ, Ó¿ÙÚÈÔ, pH Î·È ‰ÈÙÙ·ÓıÚ·ÎÈο) ‰ÂÓ ¤‰ÂÈÍ·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. √ ¤ÏÂÁ¯Ô˜ ËÎÙÈÎfiÙËÙ·˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. ∆· Â˘Ú‹Ì·Ù· ·fi ÙË ÁÂÓÈ΋ Ô‡ÚˆÓ ‹Ù·Ó: ÂȉÈÎfi ‚¿ÚÔ˜ 1020, χΈ̷ ·ÚÓËÙÈÎfi, ·ÈÌÔ-
ÛÊ·ÈÚ›ÓË +++ Î·È ÂÚ˘ıÚÔ·ÙÙ·Ú· 15-20/ÔÙÈÎfi ‰›Ô. ¢ÂÓ ‰È·ÈÛÙÒıËÎ·Ó ‰‡ÛÌÔÚÊ· ÂÚ˘ıÚÔ·ÙÙ·Ú· ÛÙËÓ ÂͤٷÛË ÙÔ˘ È˙‹Ì·ÙÔ˜ ÙˆÓ Ô‡ÚˆÓ Ì ÌÈÎÚÔÛÎfiÈÔ ·ÓÙÈı¤ÙˆÓ Ê¿ÛˆÓ. √ ÏfiÁÔ˜ ·Û‚ÂÛÙ›Ô˘ Ô‡ÚˆÓ/ÎÚ·ÙÈÓ›ÓË Ô‡ÚˆÓ, Ô˘ÚÈÎfi Ô͇ Ô‡ÚˆÓ/ÎÚ·ÙÈÓ›ÓË Ô‡ÚˆÓ Î·È ÔÍ·ÏÈÎfi Ô͇ Ô‡ÚˆÓ/ÎÚ·ÙÈÓ›ÓË Ô‡ÚˆÓ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· Ô˘ÚÔÔÈËÙÈÎÔ‡ ¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈÎfi ̤ÁÂıÔ˜ Î·È Ë¯ÔÁ¤ÓÂÈ· ÓÂÊÚÒÓ ¯ˆÚ›˜ ‰È¿Ù·ÛË ÙÔ˘ ˘ÂÏÔÎ·Ï˘ÎÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ‹ Î·È ÙˆÓ Ô˘ÚËÙ‹ÚˆÓ. ™Â ËÏÈΛ· 9 ÂÙÒÓ ÙÔ ·È‰› ·ÚÔ˘Û›·Û 2Ë ÊÔÚ¿ Ì·ÎÚÔÛÎÔÈ΋ ·ÈÌ·ÙÔ˘Ú›·. ™ÙËÓ Î˘ÛÙÂÔÛÎfiËÛË ‰È·ÈÛÙÒıËΠ·ÈÌ·ÙÔ˘Ú›· ÌfiÓÔ ·fi ÙÔÓ ·ÚÈÛÙÂÚfi Ô˘ÚËÙ‹Ú·. ™ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÎÔÈÏ›·˜ ‰È·ÈÛÙÒıËΠÛËÌ·ÓÙÈ΋ ‰È¿Ù·ÛË Ù˘ ·ÚÈÛÙÂÚ¿˜ ÓÂÊÚÈ΋˜ ÊϤ‚·˜ (∂ÈÎfiÓ· 1).
AÏÏËÏÔÁÚ·Ê›·: K. ™ÙÂÊ·Ó›‰Ë˜ stefanid@hol.gr ¡ÂÊÚÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ “¶. Î·È ∞. ∫˘ÚÈ·ÎÔ‡”
¶Èı·Ó¤˜ ‰È·ÁÓÒÛÂȘ: 1. IgA ÓÂÊÚÔ¿ıÂÈ· 2. ¡ÂfiÏ·ÛÌ· ÓÂÊÚÔ‡ 3. ¡ÂÊÚÔÏÈı›·ÛË 4. ™‡Ó‰ÚÔÌÔ Î·Ú˘ÔıÚ·‡ÛÙË
∂ÈÎfiÓ· 1. ∂ÁοÚÛÈ· ÙÔÌ‹ ˘ÂÚ˯ÔÁÚ·Ê‹Ì·ÙÔ˜ ÎÔÈÏ›·˜ (1: ¿Óˆ ÌÂÛÂÓÙ¤ÚÈÔ˜ ·ÚÙËÚ›·, 2: ·ÔÚÙ‹, 3: ÓÂÊÚÈ΋ ÊϤ‚·).
∏ ·¿ÓÙËÛË ·ÎÔÏÔ˘ı› ÛÙË ÛÂÏ›‰· 78 ¶·È‰È·ÙÚÈ΋ 2006;69:75,78-79
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¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√
µ’ ¶·È‰È·ÙÚÈ΋ ¶·ÓÂÈÛÙËÌȷ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”, ∞ı‹Ó·
¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜, ¶·È‰›·ÙÚÔ˜, ∞ÏÏÂÚÁÈÔÏfiÁÔ˜
AÏÏËÏÔÁÚ·Ê›·: ¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜ E-mail: ngp@allergy.gr
T· ÙÂÏÂ˘Ù·›· ‰‡Ô ¯ÚfiÓÈ· ¤Ó· Ó¤Ô Ê·ÈÓfiÌÂÓÔ ÂÌÊ·Ó›ÛÙËÎÂ Î·È ‹Ú ÛËÌ·ÓÙÈΤ˜ ‰È·ÛÙ¿ÛÂȘ ÛÙÔ ‰È·‰›ÎÙ˘Ô: Ù· blogs (Û‡ÓÙÌËÛË ÙÔ˘ web-logs) ·ÔÙÂÏÔ‡Ó ‰È·‰ÈÎÙ˘·Î¿ ËÌÂÚÔÏfiÁÈ·-ÛËÌÂȈ̷ٿÚÈ·, Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ÙËÓ Î·Ù·ÁÚ·Ê‹ Î·È ÙËÓ Ù·ÍÈÓfiÌËÛË ÙˆÓ ÛΤ„ÂˆÓ ÙÔ˘ Û˘ÓÙ¿ÎÙË, Ì ‚¿ÛË ÙËÓ ËÌÂÚÔÌËÓ›· Î·È ÙËÓ ÒÚ· Ô˘ ηٷ¯ˆÚÔ‡ÓÙ·È. ™ËÌ·ÓÙÈ΋ ÒıËÛË ÛÙËÓ ÂͿψÛË ÙÔ˘˜ ·ÔÙ¤ÏÂÛ·Ó Ù· ÁÂÁÔÓfiÙ· Ù˘ 11˘ ™ÂÙÂÌ‚Ú›Ô˘ 2001, fiÙ·Ó ÔÏÏÔ› ·ÏÔ› ¯Ú‹ÛÙ˜ ¤ÓÈˆÛ·Ó ÙËÓ ·Ó¿ÁÎË Ó· ÌÔ˘Ó ÛÙÔ ‰È·‰›ÎÙ˘Ô ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÓËÌÂÚˆıÔ‡Ó, Ó· ÌÔÈÚ·ÛÙÔ‡Ó ÙȘ ÛΤ„ÂȘ Î·È Ù· Û˘Ó·ÈÛı‹Ì·Ù¿ ÙÔ˘˜, ηıÒ˜ Î·È Ó· ÚÔ‚Ô‡Ó ÛÙÔÓ Û¯ÔÏÈ·ÛÌfi ÙˆÓ ÁÂÁÔÓfiÙˆÓ. ™‹ÌÂÚ· Ù· weblogs ˘¿Ú¯Ô˘Ó Û ‰È¿ÊÔÚ˜ ·Ú·ÏÏ·Á¤˜. °È· ¤Ó·Ó ·Ïfi ¯Ú‹ÛÙË, ÙÔ weblog ÌÔÚ› Ó· Â›Ó·È ¤Ó·˜ ÙÚfiÔ˜ ÁÈ· Ó· ÂÎÊÚ¿ÛÂÈ ÙȘ ÚÔÛˆÈΤ˜ ÙÔ˘ ·fi„ÂȘ Û¯ÂÙÈο Ì ÙËÓ ÂÈηÈÚfiÙËÙ·, Ì ı¤Ì·Ù· ·ÁÁÂÏÌ·ÙÈο ‹ ÚÔÛˆÈο, ÂÓÒ ÁÈ· ÌÈ· ÂÙ·ÈÚ›· ÌÔÚ› Ó· Â›Ó·È ¤Ó· ̤ÛÔ Û˘ÏÏÔÁÈ΋˜ ηٷÁÚ·Ê‹˜ ÛΤ„ˆÓ, ˘Ô‰Â›ÍÂˆÓ Î·È ÂÈÛËÌ¿ÓÛˆÓ, ÂÍ·Ï›ÙÔÓÙ·˜ ¤ÙÛÈ ÙËÓ ·Ó¿ÁÎË ·ÔÛÙÔÏ‹˜ e-mail. ∞Ó ı¤ÏÂÙ ÌÔÚ›Ù ÎÈ ÂÛ›˜, ¯ˆÚ›˜ ȉȷ›ÙÂÚ˜ Ù¯ÓÈΤ˜ ÁÓÒÛÂȘ Ó· ÍÂÎÈÓ‹ÛÂÙ ӷ ÁÚ¿ÊÂÙ ¤Ó· blog. ∂ÈÛÎÂÊı›Ù ÙÔ www.blogger.com, fiÔ˘ ˘¿Ú¯Ô˘Ó ÙfiÛÔ Ù· ̤۷ fiÛÔ Î·È ÔÈ ··Ú·›ÙËÙ˜ ÏËÚÔÊÔڛ˜ Û¯ÂÙÈο Ì ÙË ÊÈÏÔÛÔÊ›· ÙˆÓ blogs. ∞Ó ¿ÏÈ, ı¤ÏÂÙ ӷ ‰È·‚¿ÛÂÙ ‹ Ó· ÌÔÈÚ·ÛÙ›Ù ÛΤ„ÂȘ ¿ÏÏˆÓ Û˘Ó·‰¤ÏÊˆÓ ‹ ·ÛıÂÓÒÓ, ˘¿Ú¯ÂÈ ÙÂÚ¿ÛÙÈ· ÔÈÎÈÏ›·, ÌÔÚ›Ù fï˜ Ó· ÍÂÎÈÓ‹ÛÂÙ ·fi Ù· ·Ú·Î¿Ùˆ:
Pediatric Blogs
Medicine World - http://medicineworld.org/news/pediatric-news.html ™ÙËÓ ÈÛÙÔÛÂÏ›‰· Medicine World Ô ÂÈÛΤÙ˘ ¤¯ÂÈ ÚfiÛ‚·ÛË Û ÏËıÒÚ· ¿ÚıÚˆÓ ÛÙÔÓ ÙÔ̤· ÙˆÓ Pediatric News, Ù· ÔÔ›· ηٷٿÛÛÔÓÙ·È Ì ¯ÚÔÓÔÏÔÁÈ΋ ÛÂÈÚ¿. ∂ÈÚfiÛıÂÙ· ÙÔ site ÚÔÛʤÚÂÈ ÛÙÔÓ ÂÈÛΤÙË ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ·Ó·ÁÓÒÛÂÈ ÔÏϤ˜ ÂÚÈÙÒÛÂȘ Û¯ÂÙÈΤ˜ Ì ÎÔÈÓ¿ ÚÔ‚Ï‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ Ù· ÔÔ›· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ¤Ó·Ó ȉȷ›ÙÂÚ· ÁÏ·Ê˘Úfi ÂÚÈÁÚ·ÊÈÎfi ÙÚfiÔ. ÕÚıÚ· fiˆ˜ “¡¤· ∂Ï›‰· ÁÈ· Ù· ·È‰È¿ Ô˘ ¿Û¯Ô˘Ó ·fi §Â˘¯·ÈÌ›·”, fiÔ˘ ·Ó·ÎÔÈÓÒÓÂÙ·È Ë ÂÊ·ÚÌÔÁ‹ Ó¤ˆÓ ‚ÂÏÙÈˆÌ¤ÓˆÓ Ù¯ÓÈÎÒÓ ÁÈ· ÌÂÙ·ÌÔۯ‡ÛÂȘ ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Û ·È‰È¿, ‹ ¿ÚıÚ· Ô˘ ·ÊÔÚÔ‡Ó ÂÚÈÛÛfiÙÂÚÔ ˙ËÙ‹Ì·Ù· „˘¯ÔÏÔÁÈ΋˜ ʇÛˆ˜ fiˆ˜ “∞ÓÙÈÌÂÙˆ›˙ÔÓÙ·˜ Ù· ·È‰È¿ Ì ÂÓÔ‡ÚËÛË” Â›Ó·È ÌÂÚÈο ÌfiÓÔ ·fi ·˘Ù¿ Ô˘ ÌÔÚ› ηÓ›˜ Ó· ÂÓÙÔ›ÛÂÈ ÛÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ blog. ∆Ô site ‰›ÓÂÈ ÙËÓ Â˘Î·ÈÚ›· Û οı ·È‰›·ÙÚÔ Ó· ÂÁÁÚ·Ê› ˆ˜ ̤ÏÔ˜ Î·È Ó· ‰ËÌÔÛȇÛÂÈ ÙÔ ¿ÚıÚÔ ÙÔ˘ ·ÏÏ¿ Ù·˘Ùfi¯ÚÔÓ· ·Ó·Ê¤ÚÂÈ fiÙÈ Ù· ¿ÚıÚ· Ú¤ÂÈ Ó· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ˆ˜ ËÁ‹ ÚÔÛˆÈ΋˜ ÏËÚÔÊfiÚËÛ˘ Î·È fi¯È ˆ˜ ‚¿ÛË È·ÙÚÈÎÒÓ ÂÊ·ÚÌÔÁÒÓ.
Paediatriki 2007;70:76-77
NEWS FROM THE INTERNET
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WebMD - http://blogs.webmd.com/healthy-children/ ∆ËÓ ÂͿψÛË ÙˆÓ blogs ¤¯Ô˘Ó ·Ó·Î·Ï‡„ÂÈ Î·È ¯ÚËÛÈÌÔÔÈÔ‡Ó Î·È ÌÂÁ¿Ï·, ÔÚÁ·ÓˆÌ¤Ó· sites, fiˆ˜ .¯. ÙÔ webMD, ÛÙÔ ÔÔ›Ô ÔÈ ÛΤ„ÂȘ /ÛËÌÂÈÒÛÂȘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÂȉÈÎÔ‡˜. ∞Ó Î·È Ë ÚÔÛ¤ÁÁÈÛË ·˘Ù‹ ÌÂÈÒÓÂÈ ÙÔÓ ·˘ıÔÚÌËÙÈÛÌfi ÙˆÓ blogs, ˘¿Ú¯ÂÈ ÌÂÁ·Ï‡ÙÂÚË ÛÈÁÔ˘ÚÈ¿ Û¯ÂÙÈο Ì ÙË ÌË-·˘ı·ÈÚÂÛ›· Ù˘ ÏËÚÔÊÔÚ›·˜
Your Child’s Health - http://yourchildshealth.blogspot.com/2006_12_01_archive.html ∫·È ¤Ó· ·Ú¿‰ÂÈÁÌ· blog ·fi ·È‰›·ÙÚÔ: ηÏÔÛÙË̤ÓË, ¢¯¿ÚÈÛÙË Î·È ‰ËÌÈÔ˘ÚÁÈ΋, Ë ÚÔÛˆÈ΋ ÈÛÙÔÛÂÏ›‰· Ù˘ Dr Gwenn ÔÓÔÌ¿˙ÂÙ·È “Your Child’s Health” Î·È ·ÔÙÂÏ› ¤Ó· blog ÁÈ· ÙȘ Û‡Á¯ÚÔÓ˜ ÔÈÎÔÁ¤ÓÂȘ Ì ÂӉȷʤÚÔÓÙ· Ó¤·, Û¯ÔÏÈ·ÛÌÔ‡˜ Î·È ÛΤ„ÂȘ. ∏ ·È‰›·ÙÚÔ˜ ÂÎı¤ÙÂÈ Ù˘ ȉ¤Â˜ Ù˘ Î·È ÌÔÈÚ¿˙ÂÙ·È ÙȘ ÂÌÂÈڛ˜ Ù˘ Ì ÙÔ˘˜ ÁÔÓ›˜ Â͢ËÚÂÙÒÓÙ·˜ Ù·˘Ùfi¯ÚÔÓ· ¤Ó·Ó ‰È·‰Ú·ÛÙÈÎfi ÛÎÔfi. ∆›ÙÏÔÈ ¿ÚıÚˆÓ fiˆ˜ “¢È·ÙÚÔÊÈΤ˜ ·ÏÏÂÚÁ›Â˜”, “£¤Ì·Ù· Û¯ÂÙÈο Ì ٷ Ó‹È·”, “¢È·ÛΤ‰·ÛË Î·È ·ÛÊ¿ÏÂÈ· ÛÙȘ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÂÎÙfi˜ ÛÈÙÈÔ‡” ‹ “∏ ˘Á›· ÙˆÓ ÂÊ‹‚ˆÓ”, οÓÔ˘Ó ÙËÓ ¤Ú¢ӷ ÈÔ Â‡ÎÔÏË Î·È Ú·ÎÙÈ΋. ∆Ô site ¤¯ÂÈ ·ÎfiÌË Û ÛÙ‹ÏË ·Ú¯ÂÈÔıÂÙË̤ӷ Ù· ÚÔËÁÔ‡ÌÂÓ· blogs ·ÏÏ¿ Î·È Ï›ÛÙ· Ì links ¿ÏÏˆÓ ¯Ú‹ÛÈÌˆÓ ÈÛÙÔÛÂÏ›‰ˆÓ (∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È¿ÙÚˆÓ, ™‡Á¯ÚÔÓË ¶·È‰È·ÙÚÈ΋ Î.¿). ∏ Û˘ÁÎÂÎÚÈ̤ÓË ÈÛÙÔÛÂÏ›‰· ÚÔÛʤÚÂÈ ÌÈ· ¢¯¿ÚÈÛÙË ·Ó¿ÁÓˆÛË ‰È·ÓıÈṲ̂ÓË Ì ÂÈÎfiÓ˜ Î·È ÏÔ‡ÛÈ· ÁÚ·ÊÈο, ÂÓÒ ·Ú¿ÏÏËÏ· ˘¿Ú¯ÂÈ ‰˘Ó·ÙfiÙËÙ· ÂÈÎÔÈÓˆÓ›·˜, ·Ú¿ıÂÛ˘ Û¯ÔÏ›ˆÓ ‹ Î·È ÂÓÂÚÁÔ‡ Û˘ÌÌÂÙÔ¯‹˜ ÛÙËÓ ·ÚıÚÔÁÚ·Ê›·.
∞˜ ÛËÌÂȈı› fiÙÈ Ë ·ÍÈÔÈÛÙ›· ÙˆÓ ÏËÚÔÊÔÚÈÒÓ ÛÙ· blogs ‰ÂÓ ÂϤÁ¯ÂÙ·È ·fi Î·Ó¤Ó·Ó ÎÈ ¤ÙÛÈ, ÂÓÒ ÌÔÚ› Ó· ·ÔÙÂϤÛÔ˘Ó Â˘¯¿ÚÈÛÙÔ ·Ó¿ÁÓˆÛÌ·, ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È Û·Ê¤˜ fiÙÈ ‰ÂÓ ¤¯Ô˘Ó Û η̛· ÂÚ›ÙˆÛË ÚfiÏÔ È·ÙÚÈ΋˜ ÁÓÒÌ˘ ‹ Û˘Ì‚Ô˘Ï‹˜!
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CLINICAL QUIZ
∫ÏÈÓÈÎfi QUIZ ∞¶∞¡∆∏™∏
∏ ‰È¿ÁÓˆÛË ‹Ù·Ó Û‡Ó‰ÚÔÌÔ ÙÔ˘ ηڢÔıÚ·‡ÛÙË (nutcracker), Ë ÔÔ›· ‚·Û›ÛıËΠÛÙ· Ù˘Èο Â˘Ú‹Ì·Ù· Ù˘ ˘ÔÙÚÔÈ¿˙Ô˘Û·˜ Ì·ÎÚÔÛÎÔÈ΋˜ ·ÈÌ·ÙÔ˘Ú›·˜ ÌfiÓÔ ·fi ÙÔÓ ·ÚÈÛÙÂÚfi Ô˘ÚËÙ‹Ú·, ÌÈÎÚÔÛÎÔÈ΋˜ ·ÈÌ·ÙÔ˘Ú›·˜ Ì ÈÛÔÌÔÚÊÈο ÂÚ˘ıÚÔ·ÙÙ·Ú· ÛÙËÓ ÂͤٷÛË ÙÔ˘ È˙‹Ì·ÙÔ˜ Ô‡ÚˆÓ Ì ÌÈÎÚÔÛÎfiÈÔ ·ÓÙÈı¤ÙˆÓ Ê¿ÛˆÓ, ÛÙËÓ ÎÈÚÛÔ΋ÏË Î·È ÂȂ‚·ÈÒıËΠ̠ÙÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ‡ÚËÌ· ‰È¿Ù·ÛË Ù˘ ·ÚÈÛÙÂÚ‹˜ ÓÂÊÚÈ΋˜ ÊϤ‚·˜ ÛÙËÓ ÂÁοÚÛÈ· ÙÔÌ‹ ÙÔ˘ ˘ÂÚ˯ÔÁÚ·Ê‹Ì·ÙÔ˜ ÎÔÈÏ›·˜ (∂ÈÎfiÓ· 2). ∆Ô Û‡Ó‰ÚÔÌÔ ÙÔ˘ ηڢÔıÚ·‡ÛÙË Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Û˘Ì›ÂÛ˘ Ù˘ ·ÚÈÛÙÂÚ‹˜ ÓÂÊÚÈ΋˜ ÊϤ‚·˜ ·fi ÙËÓ ·ÔÚÙ‹ Î·È ÙËÓ ¿Óˆ ÌÂÛÂÓÙ¤ÚÈ· ·ÚÙËÚ›·, Ô˘ ÏÂÈÙÔ˘ÚÁÔ‡Ó Û·Ó Ù· ‰‡Ô ÛΤÏË ÂÓfi˜ ηڢÔıÚ·‡ÛÙË (1). ∆Ô Û‡Ó‰ÚÔÌÔ ·˘Ùfi ÔÊ›ÏÂÙ·È Û ·ÓÒÌ·ÏË ‰È·ÎÏ¿‰ˆÛË Ù˘ ¿Óˆ ÌÂÛÂÓÙ¤ÚÈ·˜ ·ÚÙËÚ›·˜ ·fi ÙËÓ ·ÔÚÙ‹ ‹ ÛÙËÓ ÂÏ¿ÙÙˆÛË Ù˘ ÁˆÓ›·˜ Ô˘ Û¯ËÌ·Ù›˙ÂÙ·È ·fi ÙȘ ‰‡Ô ·˘Ù¤˜ ·ÚÙËڛ˜ (1,2) (∂ÈÎfiÓ· 3). ∏ ÁˆÓ›· ·˘Ù‹ ÂÏ·ÙÙÒÓÂÙ·È ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ ÛÙËÓ fiÚıÈ· ı¤ÛË Î·È ¤ÙÛÈ ÌÔÚ› Ó· ÂÍËÁËı› Î·È Ë ÂÌÊ¿ÓÈÛË ÔÚıÔÛÙ·ÙÈ΋˜ ÏÂ˘ÎˆÌ·ÙÔ˘Ú›·˜ Û ·ÛıÂÓ›˜ Ì ÙÔ Û‡Ó‰ÚÔÌÔ ·˘Ùfi (3) ‹ Ë ÂÌÊ¿ÓÈÛË ÙˆÓ ÂÂÈÛÔ‰›ˆÓ Ì·ÎÚÔÛÎÔÈ΋˜ ·ÈÌ·ÙÔ˘Ú›·˜ ÌÂÙ¿ ·fi ¿ÛÎËÛË. ∏ ·‡ÍËÛË Ù˘ ›ÂÛ˘ ÛÙËÓ ·ÚÈÛÙÂÚ‹ ÓÂÊÚÈ΋ ÊϤ‚· ‰ËÌÈÔ˘ÚÁ› ·Ú¿Ï¢ÚË ÊÏ‚È΋ ΢ÎÏÔÊÔÚ›· Î·È ÚÔηÏ› ·ÏÈÓ‰ÚfiÌËÛË ·›Ì·ÙÔ˜ ÛÙËÓ ¤Ûˆ ÛÂÚÌ·ÙÈ΋ ÊϤ‚· Î·È ÂÌÊ¿ÓÈÛË ÎÈÚÛÔ΋Ï˘, ηıÒ˜ Î·È ÌÈÎÚÔÚ‹ÍÂȘ ÙˆÓ ÊÏ‚ÒÓ Ì ÏÂÙfi ÙÔ›¯ˆÌ· ÙÔ˘ ÕÓˆ ÌÂÛÂÓÙ¤ÚÈ· ·ÚÙËÚ›· ŒÛˆ ÛÂÚÌ·ÙÈ΋ ÊϤ‚·
¡ÂÊÚÈ΋ ÊϤ‚· ∞ÔÚÙ‹
∞ÚÈÛÙÂÚfi˜ ÓÂÊÚfi˜
∂ÈÎfiÓ· 2. ªË¯·ÓÈÛÌfi˜ ÚfiÎÏËÛ˘ ‚Ï¿‚˘ ÛÙÔ Û‡Ó‰ÚÔÌÔ ÙÔ˘ ηڢÔıÚ·‡ÛÙË. Paediatriki 2007;70:75,78-79
ÕÓˆ ÌÂÛÂÓÙ¤ÚÈ· ·ÚÙËÚ›·
∞ÚÈÛÙÂÚfi˜ ÓÂÊÚfi˜
¡ÂÊÚÈ΋ ÊϤ‚· ∞ÔÚÙ‹
ŒÛˆ ÛÂÚÌ·ÙÈ΋ ÊϤ‚·
∂ÈÎfiÓ· 3. ∏ ÔÚ›· Ù˘ ÓÂÊÚÈ΋˜ ÊϤ‚·˜ ÌÂٷ͇ ¿Óˆ ÌÂÛÂÓÙÂÚ›Ô˘ ·ÚÙËÚ›·˜ Î·È ·ÔÚÙ‹˜.
·Ô¯ÂÙ¢ÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ŒÙÛÈ ÂÍËÁÂ›Ù·È Î·È Ë ÂÌÊ¿ÓÈÛË ·ÈÌ·ÙÔ˘Ú›·˜ Ì ÂÚ˘ıÚÔ·ÙÙ·Ú· Ì ÌÔÚÊÔÏÔÁ›· ·Ó¿ÏÔÁË ÂÎÂ›ÓˆÓ ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ (ÈÛÔÌÔÚÊÈο) (4). ∆· Ù˘Èο Â˘Ú‹Ì·Ù· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Â›Ó·È Ë ·Û˘Ìو̷ÙÈ΋ ÌÈÎÚÔÛÎÔÈ΋, ÌË ÛÂÈÚ·Ì·ÙÈ΋ ·ÈÌ·ÙÔ˘Ú›· Ì ‹ ¯ˆÚ›˜ ÔÚıÔÛÙ·ÙÈ΋ ÏÂ˘ÎˆÌ·ÙÔ˘Ú›· Î·È Ë ·ÈÊÓ›‰È· ÂÌÊ¿ÓÈÛË ÂÂÈÛÔ‰›ˆÓ Ì·ÎÚÔÛÎÔÈ΋˜ ·ÈÌ·ÙÔ˘Ú›·˜ Û˘Ó‹ıˆ˜ ÌÈÎÚ‹˜ ‰È¿ÚÎÂÈ·˜, Ù· ÔÔ›· ‰ÂÓ Û¯ÂÙ›˙ÔÓÙ·È Ì ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡, Û ·ÓÙ›ıÂÛË Ì ÙËÓ IgA ÓÂÊÚÔ¿ıÂÈ·. ∞ÎfiÌË ÌÂÚÈÎÔ› ·ÛıÂÓ›˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÎÔÈÏȷο ¿ÏÁË ‹ ¿ÏÁË ÛÙËÓ ÔÛÊ˘˚΋ ¯ÒÚ·, fï˜ Û˘Ó‹ıˆ˜ ‰ÂÓ ‰È·ÈÛÙÒÓÔÓÙ·È ÛÔ‚·Ú¤˜ ‰È·Ù·Ú·¯¤˜. ∆Ô Û‡Ó‰ÚÔÌÔ ‰ÂÓ ·ÔÙÂÏ› ÌÈ· ÎÏËÚÔÓÔÌÈ΋ ÓfiÛÔ Î·È Ë ÂÌÊ¿ÓÈÛ‹ ÙÔ˘ Û ̤ÏË Ù˘ ›‰È·˜ ÔÈÎÔÁ¤ÓÂÈ·˜ Â›Ó·È Û¿ÓÈ·. ¶ÚfiÛÊ·Ù· ¤¯ÂÈ ÂÚÈÁÚ·Ê› Û ‰‡Ô ·‰¤ÏÊÈ· (5). ∆Ô Û‡Ó‰ÚÔÌÔ ÙÔ˘ ηڢÔıÚ·‡ÛÙË Ú¤ÂÈ Ó· ‰È·ÎÚ›ÓÂÙ·È ·fi ÙÔ Ê·ÈÓfiÌÂÓÔ ÙÔ˘ ηڢÔıÚ·‡ÛÙË, ‰ËÏ·‰‹ ÙËÓ ·Û˘Ìو̷ÙÈ΋ ‰È¿Ù·ÛË Ù˘ ÓÂÊÚÈ΋˜ ÊϤ‚·˜, Ô˘ Û˘Ó‹ıˆ˜ Â›Ó·È Ù˘¯·›Ô ‡ÚËÌ· (6). ∏ ÚÒÙË ÂÚÈÁÚ·Ê‹ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ¤ÁÈÓ ÙÔ 1972 ·fi ÙÔÓ De Schepper Û ‰‡Ô ·ÛıÂÓ›˜ Ì ÂÈ̤ÓÔ˘Û· “ȉÈÔ·ı‹” ·ÈÌ·ÙÔ˘Ú›· Î·È ‰È¿Ù·ÛË Ù˘ ·ÚÈÛÙÂÚ‹˜ ÓÂÊÚÈ΋˜ ÊϤ‚·˜ (7). ∆· ÂfiÌÂÓ· ¯ÚfiÓÈ· ÂÚÈÁÚ¿ÊËÎ·Ó ÔÏÏÔ› ·ÛıÂÓ›˜ Ì ÙÔ Û‡Ó‰ÚÔÌÔ ·˘Ùfi, ÔÈ ÔÔ›ÔÈ Û˘Ó‹ıˆ˜ ›¯·Ó ηÏÔ‹ıË ÔÚ›· Î·È Ê¿ÓËΠfiÙÈ ÙÔ Û‡Ó‰ÚÔÌÔ ·˘Ùfi ‹Ù·Ó Ôχ ÈÔ Û˘¯Ófi ·’ fi,ÙÈ ·Ú¯Èο ıˆÚÔ‡ÓÙ·Ó. ŒÙÛÈ Û ÌÈ· ÂȉËÌÈÔÏÔÁÈ΋
CLINICAL QUIZ ÌÂϤÙË ·fi ÙËÓ ∫ÔÚ¤· Û 1044 ·È‰È¿ Ì ·ÈÌ·ÙÔ˘Ú›· ‹/Î·È ÏÂ˘ÎˆÌ·ÙÔ˘Ú›·, ÙÔ Û‡Ó‰ÚÔÌÔ ·˘Ùfi ‰È·ÁÓÒÛÙËΠ۠65 ·È‰È¿, ·ÓÙÈÚÔۈ‡ÔÓÙ·˜ ÙÔ Û˘¯ÓfiÙÂÚÔ ·ıÔÏÔÁÈÎfi ‡ÚËÌ· ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ Ì ‹ ¯ˆÚ›˜ Doppler (8). ™Â ¿ÏÏË ÌÂϤÙË Û 52 ·È‰È¿ Ì ÌÈÎÚÔÛÎÔÈ΋ ·ÈÌ·ÙÔ˘Ú›· ‰È·ÈÛÙÒıËÎ·Ó ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ Ì Doppler Â˘Ú‹Ì·Ù· Ô˘ ÏËÚÔ‡Û·Ó Ù· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÛÙÔ 33% ÙˆÓ ·ÛıÂÓÒÓ (9). øÛÙfiÛÔ, ‰ÂÓ ˘¿Ú¯ÂÈ ÔÌÔʈӛ· ÁÈ· ÙÔ Ò˜ Ú¤ÂÈ Ó· Á›ÓÂÙ·È Ì ·ÍÈÔÈÛÙ›· Ë ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ∏ ÓÂÊÚÈ΋ ÊÏ‚ÔÁÚ·Ê›·, Ì ٷ˘Ùfi¯ÚÔÓË Ì¤ÙÚËÛË ÙˆÓ È¤ÛÂˆÓ ÛÙËÓ Î¿Ùˆ ÎÔ›ÏË Î·È ÙË ÓÂÊÚÈ΋ ÊϤ‚· ıˆÚÂ›Ù·È Ë ÈÔ ·ÍÈfiÈÛÙË Ì¤ıÔ‰Ô˜. ŸÌˆ˜ Ë Ù¯ÓÈ΋ ·˘Ù‹ Â›Ó·È ·ÚÂÌ‚·ÙÈ΋ Î·È Ë ÂÊ·ÚÌÔÁ‹ Ù˘ ÛÙ· ·È‰È¿ ‰˘Û¯ÂÚ‹˜. ∏ ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, ·ÎfiÌ· Î·È ÛÙ· ·Ú¯Èο ÛÙ¿‰È·, ÌÔÚ› Ó· Á›ÓÂÈ Î·È Ì ·Ïfi ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ‹ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· Doppler, Ì ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ‹ Ì ̷ÁÓËÙÈ΋ ·ÁÁÂÈÔÁÚ·Ê›· (10). ∆· Â˘Ú‹Ì·Ù· ÛÙËÓ ÂͤٷÛË ÙÔ˘ È˙‹Ì·ÙÔ˜ Ô‡ÚˆÓ Ì ÌÈÎÚÔÛÎfiÈÔ ·ÓÙÈı¤ÙˆÓ Ê¿ÛÂˆÓ ‰ÂÓ Â›Ó·È ¿ÓÙÔÙ ·ÍÈfiÈÛÙ· ÁÈ· ÙË ‰È¿ÎÚÈÛË Ù˘ ÛÂÈÚ·Ì·ÙÈ΋˜, ·fi ÙË ÌË ÛÂÈÚ·Ì·ÙÈ΋ ·ÈÌ·ÙÔ˘Ú›·. ∂›Ó·È ÂӉȷʤÚÔÓ fiÙÈ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ·ÛıÂÓ›˜ Ì ÌÈÎÚÔÛÎÔÈ΋ ·ÈÌ·ÙÔ˘Ú›· Î·È Ù˘Èο ˘ÂÚ˯ÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, Ô˘ ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ‹˜ ÙÔ˘˜ ‰È·ÁÓÒÛıËΠIgA ÓÂÊÚÔ¿ıÂÈ· (11). ∞Û˘Ìʈӛ· ˘¿Ú¯ÂÈ Î·È ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ∂Ӊ›ÍÂȘ ÁÈ· ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË Â›Ó·È Ë ÛÔ‚·Ú‹ ·ÈÌ·ÙÔ˘Ú›· Ô˘ ÚÔηÏ› ·Ó·ÈÌ›·, ÛÔ‚·Ú¿ ÎÔÈÏȷο ¿ÏÁË ‹ ¿ÏÁË ÛÙËÓ ÔÛÊ˘˚΋ ¯ÒÚ· (10,12). ŸÌˆ˜ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ·ÛıÂÓ›˜ ÌÔÚÔ‡Ó Ó· ·ÓÙÈÌÂÙˆÈÛıÔ‡Ó Ì ̷ÎÚÔ¯ÚfiÓÈ· ·Ú·ÎÔÏÔ‡ıËÛË, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Ë ÚfiÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Û˘Ó‹ıˆ˜ Â›Ó·È Î·Ï‹. ™Â ÚfiÛÊ·ÙË ‰ËÌÔÛ›Â˘ÛË ·Ó·Ê¤ÚÂÙ·È fiÙÈ ÌfiÓÔ 15 ·fi Ù· 20 ·È‰È¿ Ì ۇӉÚÔÌÔ ÙÔ˘ ηڢÔıÚ·‡ÛÙË Û˘Ó¤¯È˙·Ó Ó· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ÈÌ·ÙÔ˘Ú›· ÌÂÙ¿ ·fi 0,5-3,5 ¯ÚfiÓÈ· (10). ŒÙÛÈ Û‹ÌÂÚ· Â›Ó·È ÁÂÓÈο ·Ô‰ÂÎÙfi fiÙÈ Ë ¤ÁηÈÚË
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‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Â›Ó·È Ôχ ÛËÌ·ÓÙÈ΋ ÁÈ· Ó· ·ÔʇÁÔÓÙ·È ÔÈ ÂÚÈÙÙ¤˜ ÂÍÂÙ¿ÛÂȘ.
µÈ‚ÏÈoÁÚ·Ê›· 1. Chang CT, Hung CC, Hg KK, Yen TH. Nutcracker syndrome and left unilateral haematuria. Nephrol Dial Transplant 2005;20:460-461. 2. Ahmed K, Sampath R, Khan MS. Current trends in the diagnosis and management of renal nutcracker syndrome: a review. Eur J Vasc Endovasc Surg 2006;31:410-416. 3. Ekim M, Ozcakar ZB, Fitoz S, Soygur T, Yuksel S, Acar B, Yalcinkaya F, Arikan N. The “nutcracker phenomenon” with orthostatic proteinuria: case reports. Clin Nephrol 2006;65:280-283. 4. Wolfish NM, McLaine PN, Martin D. Renal vein entrapment syndrome: frequency and diagnosis. A lesson in conservatism. Clin Nephrol 1986;26:96-100. 5. Matsukura H, Arai M, Miyawaki T. Nutcracker phenomenon in two siblings of a Japanese family. Pediatr Nephrol 2005;20:237-238. 6. Liebl R. Nutcracker phenomenon or nutcracker syndrome? Nephrol Dial Transplant 2005;20:2009. 7. de Schepper A. “Nutcracker” phenomenon of the renal vein and venous pathology of the left kidney. J Belge Radiol 1972; 55:507-511. 8. Park YH, Choi JY, Chung HS, Koo JW, Kim SY, Namgoong MK, et al. Hematuria and proteinuria in a mass school urine screening test. Pediatr Nephrol 2005;20:1126-1130. 9. Park SJ, Lim JW, Cho BS, Yoon TY, Oh JH. Nutcracker syndrome in children with orthostatic proteinuria: diagnosis on the basis of Doppler sonography. J Ultrasound Med 2002; 21:39-45. 10. Shin JI, Park JM, Lee SM, Shin YH, Kim JH, Lee JS, et al. Factors affecting spontaneous resolution of hematuria in childhood nutcracker syndrome. Pediatr Nephrol 2005;20: 609-613. 11. Ozono Y, Harada T, Namie S, Ichinose H, Shimamine R, Nishimawa Y, et al. The “nutcracker” phenomenon in combination with IgA nephropathy. J Int Med Res 1995;23: 126-131. 12. Chen W, Chu J, Yang JY, Li HP, Zhuang WQ, Huang YH, et al. Endovascular stent placement for the treatment of nutcracker phenomenon in three pediatric patients. J Vasc Interv Radiol 2005;16:1529-1533.
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LETTER TO THE EDITOR
∞Á·ËÙÔ› ·ÚÈÔÈ, ªÂ ¯·Ú¿ Û·˜ ·Ó·ÎÔÈÓÒÓÔ˘Ì fiÙÈ ÙÔ Ó¤Ô ÙÌ‹Ì· Ù˘ µã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ªÔÓ¿‰· ∂ÊË‚È΋˜ ÀÁ›·˜ (ª.∂.À.) “∞§§∞∑ø”, ¿Ú¯ÈÛ ӷ ÏÂÈÙÔ˘ÚÁ› ·fi 15-10-2006, ÛÙÔ ¶·Ú¿ÚÙËÌ· ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ “¶·Ó. & ∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡”, ªÂÛÔÁ›ˆÓ 24 ÛÙÔÓ 6Ô fiÚÔÊÔ (¶Ù¤Ú˘Á· ∂ϤÓ˘ ¢·Ï·ÎÔ‡Ú·). ∆Ô ∆Ì‹Ì· ÂÈÛΤÙÔÓÙ·È ¤ÊË‚ÔÈ ËÏÈΛ·˜ 11-19 ÂÙÒÓ Î·È Ë ÔÈÎÔÁ¤ÓÂÈ¿ ÙÔ˘˜, ÁÈ· Û˘˙‹ÙËÛË Î·È Â›Ï˘ÛË ıÂÌ¿ÙˆÓ Ô˘ ÙÔ˘˜ ··Û¯ÔÏÔ‡Ó: ñ ∂Ó‰ÔÎÚÈÓÔÏÔÁÈο ñ µ¿ÚÔ˘˜ ÛÒÌ·ÙÔ˜ ñ ¢È·ÙÚÔÊ‹˜ ñ ÕÛÎËÛ˘ ñ ¢ÂÚÌ·ÙÔÏÔÁÈο ñ °˘Ó·ÈÎÔÏÔÁÈο ñ æ˘¯ÔÏÔÁÈο ñ £¤Ì·Ù· Û¯ÔÏÈ΋˜ ›‰ÔÛ˘ ñ ¢˘ÛÏÂÈÙÔ˘ÚÁ›· ÔÈÎÔÁ¤ÓÂÈ·˜, Û˘Ì‚Ô˘Ï¢ÙÈ΋ Î.Ï. √È ˘ËÚÂۛ˜ ·Ú¤¯ÔÓÙ·È ·fi ÂȉÈÎÔ‡˜ Û ı¤Ì·Ù· ÂÊË‚È΋˜ ˘Á›·˜, ¯ˆÚ›˜ ÔÈÎÔÓÔÌÈ΋ ÂÈ‚¿Ú˘ÓÛË, Î·È Û ÒÚ˜ Ô˘ Â͢ËÚÂÙÔ‡Ó ¿ÓÙ· ÙÔ ÚfiÁÚ·ÌÌ· ÙˆÓ ÂӉȷÊÂÚÔÌ¤ÓˆÓ (·ÔÁ‡̷ٷ). Ÿˆ˜ fiÏÔÈ ÁÓˆÚ›˙Ô˘ÌÂ, Ë ÂÊ˂›· Â›Ó·È ÌÈ· “ȉȷ›ÙÂÚË” ËÏÈΛ·. ∞ÔÙÂÏ› ÌÈ· ÌÂÙ·‚·ÙÈ΋ ÂÚ›Ô‰Ô, ÌÂٷ͇ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ Î·È Ù˘ ÂÓËÏÈΛˆÛ˘. √ ¤ÊË‚Ô˜ ‰ÂÓ Â›Ó·È Ô‡Ù ·È‰›, Ô‡Ù ÂÓ‹ÏÈη˜ Î·È ·ÚÔ˘ÛÈ¿˙ÂÈ È‰È·ÈÙÂÚfiÙËÙ˜ Î·È Û˘ÁÎÂÎÚÈ̤Ó˜ ·Ó¿ÁΘ. ∏ ËÏÈΛ· ·˘Ù‹ Â›Ó·È ÁÂÌ¿ÙË ÚˆÙfiÁÓˆÚ˜ Î·È Û˘Ó·Ú·ÛÙÈΤ˜ ÂÌÂÈڛ˜, ·ÏÏ¿ Î·È ‰˘ÛÎÔϛ˜, ¿Á¯Ô˜ Î·È ÔÚÈṲ̂Ó˜ ÊÔÚ¤˜ ΛӉ˘ÓÔ. §fiÁˆ Ù˘ ۈ̷ÙÈ΋˜ Î·È „˘¯ÔÏÔÁÈ΋˜ ·ÛÙ¿ıÂÈ·˜ Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙÔÓ ¤ÊË‚Ô, ÌÔÚ› Ó· ·Ó·‰˘ıÔ‡Ó ‰È¿ÊÔÚ· ÚÔ‚Ï‹Ì·Ù· ‹ Ó· ÂȉÂÈÓˆıÔ‡Ó Ù· ‹‰Ë ˘¿Ú¯ÔÓÙ·. ∂ÈϤÔÓ, Ë ÔÈÎÔÁ¤ÓÂÈ· ÌÔÈ¿˙ÂÈ Û˘¯Ó¿ ·Ì‹¯·ÓË, ÁÂÌ¿ÙË ‰¤Ô˜ ÌÚÔÛÙ¿ Û ·˘Ùfi ÙÔÓ Î·Ù·ÈÁÈÛÌfi ۈ̷ÙÈÎÒÓ Î·È „˘¯ÔÏÔÁÈÎÒÓ ÌÂÙ·‚ÔÏÒÓ. √ ¤ÊË‚Ô˜ Î·È Ë ÔÈÎÔÁ¤ÓÂÈ¿ ÙÔ˘ ¤¯Ô˘Ó ·Ó¿ÁÎË ·fi “Ê¿ÚÔ˘˜” ÛÙȘ Û‡Á¯ÚÔÓ˜ ÎÔÈӈӛ˜. ™ÙËÓ ∂ÏÏ¿‰· ‰ÂÓ ˘¿Ú¯Ô˘Ó ·ÚÎÂÙ¤˜ ‰Ô̤˜ ÁÈ· ÙËÓ ÂÊË‚È΋ ËÏÈΛ·, Ì ·ÔÙ¤ÏÂÛÌ· ÔÈ ¤ÊË‚ÔÈ Ó· ·Ú·ÌÂÏÔ‡ÓÙ·È Î·È Ó· ÂÎÙ›ıÂÓÙ·È Û ÎÈÓ‰‡ÓÔ˘˜. ∏ ·Ó¿ÁÎË ‰ËÌÈÔ˘ÚÁ›·˜ ÌÈ·˜ Ó¤·˜ ‰ÔÌ‹˜ ÁÈ· ÙËÓ ÂÊË‚È΋ ËÏÈΛ· ˘·ÁÔÚ‡ÂÙ·È ·fi ÙË ÁÂÓÈÎfiÙÂÚË ¤ÏÏÂÈ„Ë ‰ÔÌÒÓ ÁÈ· ÙË ÊÚÔÓÙ›‰· ÙˆÓ ∂ÏÏ‹ÓˆÓ ÂÊ‹‚ˆÓ, ηıÒ˜ Î·È ·fi ÙËÓ ·Ó·Ú΋ ηٷÁÚ·Ê‹ Î·È ÌÂϤÙË ÙˆÓ ÚÔ‚ÏËÌ¿ÙˆÓ Ù˘ ËÏÈΛ·˜ ·˘Ù‹˜. °È· ÂÚÈÛÛfiÙÂÚ˜ ÏËÚÔÊÔڛ˜ Î·È Ú·ÓÙ‚ԇ ÔÈ ÂӉȷÊÂÚfiÌÂÓÔÈ ÌÔÚÔ‡Ó Ó· ·Â˘ı‡ÓÔÓÙ·È ÛÙË ªÔÓ¿‰· ∂ÊË‚È΋˜ ÀÁ›·˜ (ª.∂.À.) ηıËÌÂÚÈÓ¿ 10:00-14:00, ÛÙÔ ÙËϤʈÓÔ 210-7710824.
ªÂ ÙÈÌ‹, √ ¢È¢ı˘ÓÙ‹˜ ∞Ó‰Ú¤·˜ ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ ∫·ıËÁËÙ‹˜ - ¢È¢ı˘ÓÙ‹˜ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ
Paediatriki 2007;70:80
∏ ∂ÈÛÙËÌÔÓÈ΋ À‡ı˘ÓË ÕÚÙÂÌȘ ∆Û›ÙÛÈη ¶·È‰›·ÙÚÔ˜ - ∂ÊË‚È΋ π·ÙÚÈ΋ ¢È‰¿ÎÙˆÚ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ
¶ƒ√™∂Ã∏ ™À¡∂¢ƒπ∞
xxiii
10-11 ºÂ‚ÚÔ˘·Ú›Ô˘ 2007
™ÂÌÈÓ¿ÚÈÔ ¢È·Ù·Ú·¯‹ ∂ÏÏÂÈÌÌ·ÙÈ΋˜ ¶ÚÔÛÔ¯‹˜ – ÀÂÚÎÈÓËÙÈÎfiÙËÙ· MÔ˘ÛÂ›Ô ∞ıÏËÙÈÛÌÔ‡ £ÂÛÛ·ÏÔӛ΢ ¶ÏËÚÔÊÔڛ˜: Diaskepsis - ª·Ù›Ó· ∆Û¿ÌË ∆ËÏ./Fax: 2310 230 459 ∂-mail: mtsami@otenet.gr Website: www.diaskepsis.gr
£ÂÛÛ·ÏÔÓ›ÎË
17 ºÂ‚ÚÔ˘·Ú›Ô˘ 2007
3Ë ∂ÈÛÙËÌÔÓÈ΋ ∏ÌÂÚ›‰· °¡¶∞ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡” “¶·È‰ÈÎfi ∞Ù‡¯ËÌ·” ¢ÈÔÚÁ¿ÓˆÛË: ∂ÈÛÙËÌÔÓÈÎfi ™˘Ì‚Ô‡ÏÈÔ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ AÌÊÈı¤·ÙÚÔ “∫ˆÓÛÙ·ÓÙ›ÓÔ˜ ¶··‰¿ÙÔ˜” NÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡” £Ë‚ÒÓ & §Â‚·‰Â›·˜, ∞ÌÂÏfiÎËÔÈ ∆ËÏ.: 210 7726000
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28 ºÂ‚ÚÔ˘·Ú›Ô˘ 2 ª·ÚÙ›Ô˘ 2007
Current Concepts in Paediatric Orthopaedics Royal Institute of British Architects Contact: Deborah Eastwood Tel.: 020 8909 5758 Fax: 020 8954 4566 E-mail: Gloria.Richter@rnoh.nhs.uk Website: http://www.bscos.com
London, United Kingdom
8-11 ª·ÚÙ›Ô˘ 2007
Pediatric Anesthesiology 2007 Contact: Society for Pediatric Anesthesia Tel.: 804-282-9780 Fax: 804-282-0090 E-mail: spa@societyhq.com
Phoenix, AZ, United States
31 ª·ÚÙ›Ô˘ 2007
5Ë ∂ÈÛÙËÌÔÓÈ΋ ∏ÌÂÚ›‰· ∂˘ÚˆÎÏÈÓÈ΋˜ ¶·›‰ˆÓ ∞›ıÔ˘Û· Olympia •ÂÓÔ‰Ô¯Â›Ô Royal Olympic ¶ÏËÚÔÊÔڛ˜: FIRSTEVENT ∂¶∂ ∆ËÏ.: 210 8228950 Fax: 210 8228901 E-mail: info@firstevent.gr
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16-18 ∞ÚÈÏ›Ô˘ 2007
The 7th Annual International Neonatology Symposium Contact: Saleh Al-Alaiyan, MD Tel.: 00-96-614-427-762 Fax: 00-96-614-427-784 E-mail: alaiyan@kfshrc.edu.sa
Riyadh, Saudi Arabia
25-28 ∞ÚÈÏ›Ô˘ 2007
18th Annual Congress of the European Society for Paediatric Urology (ESPU) HALL Ambassadeur, Oud-Sint Jan, Zonnekemeers Website: www.espu2007.com/
Brugge, Belgium
xxiv
2-4 ª·˝Ô˘ 2007
25th European Congress for Pediatric Infectious Diseases ~es Centro de Congressos e Exposiço Contact: Kenes International / ESPID 2007 17, Rue du Cendrier, PO Box 1726 CH-1211 Geneva 1, Switzerland Tel: +41 22 908 0488 Fax: +41 22 732 2850 E-mail: espid@kenes.com Website: www.kenes.com/espid/
Porto, Portugal
2-5 ª·˝Ô˘ 2007
15th TRANSMED: 15th European / Mediterranean Congress about Mother & Child Health Contact: Kamel BARGAOUI Tel.: 33-607-686-118 Fax: 33-143-839-985 E-mail: kamel@medicom-international.com
Cairo, Egypt
9-12 ª·˝Ô˘ 2007
40th Annual meeting of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) Catalunya Congress Palace Contact: ESPGHAN 2007 C/o Colloquium 12, rue de la Croix Faubin 75557 Paris Cedex 11 - France Tel.: 33 (0) 1 44 64 15 15 Fax: 33 (0) 1 44 64 15 16 E-mail: espghan2007@colloquium.fr
Barcelona, Spain
25-27 ª·˝Ô˘ 2007
45Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ ™˘Ó‰ÚÈ·Îfi ∫¤ÓÙÚÔ Porto Carras “Olympic” ¶ÏËÚÔÊÔڛ˜: AC&C International S.A. ∆ËÏ.: 210 6889 100 Fax: 210 6844 777 ∂-mail: pediatric2007@acnc.gr Website: www.pediatric2007.gr
™ÈıˆÓ›·, ÷ÏÎȉÈ΋˜
3-7 πÔ˘Ó›Ô˘ 2007
44th Annual Meeting of the European Society of Pediatric Radiology Contact: Viajes Iberia Congresos Fontanella 21-23, 4o1a 08010 Barcelona, Spain Tel.: +34 93 510 1005 Fax: +34 93 510 1009 E-mail: espr2007.meeting@viajesiberia.com Website: www.espr2007.info/
Barcelona, Spain
25-30 ∞˘ÁÔ‡ÛÙÔ˘ 2007
25th International Congress of Pediatrics ª¤Á·ÚÔ ªÔ˘ÛÈ΋˜ ∞ıËÓÒÓ ∞C&C International, PCO Tel: 210 68 89 100 Fax: 210 68 44 777 E-mail: icp2007@acnc.gr Website: www.icp2007.gr
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