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TfiÌÔ˜ 69 ñ ∆‡¯Ô˜ 6 ñ No¤Ì‚ÚÈÔ˜-¢ÂΤ̂ÚÈÔ˜ 2006TfiÌÔ˜ 69 ñ ∆‡¯Ô˜ 6 ñ No¤Ì‚ÚÈÔ˜-¢ÂΤ̂ÚÈÔ˜ 2006
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www.paediatriki.edu.grwww.paediatriki.edu.grISSN 0377-2551ISSN 0377-2551
Volume 69 ñ Number 6 ñ ¡ovember-December 2006Volume 69 ñ Number 6 ñ ¡ovember-December 2006
Exof Pediatr Nov-Dec 06 06-12-06 17:42 ™ÂÏ›‰·1
¢ÈÌËÓÈ·›· ¤Î‰ÔÛË Ù˘
EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜
¶Úfi‰ÚÔ˜
∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜
™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹
¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘
∫. ™ÙÂÊ·Ó›‰Ë˜
M¤ÏË
¶. ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘
∂. ∞ÓÙˆÓÔÔ‡ÏÔ˘
A. µ·˙·›Ô˘-°ÂÚ·ÛÈÌ›‰Ë
°. µ·ÚÏ¿Ì˘
ª. ∫·Ó¿ÚÈÔ˘
∂. ∫·ÙÛ·ÚÔ‡-¶ÂÎÙ·Û›‰Ë
A. K·ÙÙ¿Ì˘
Ã. ∫ÒÛÙ·ÏÔ˜
¡. ¶··‰fiÔ˘ÏÔ˜
∞. ¶··‰ÔÔ‡ÏÔ˘
∞. ™È·ÌÔÔ‡ÏÔ˘-ª·˘Ú›‰Ô˘
ª. ∆ÛÔÏÈ¿
ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ·
∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈ̤ӈÓ
º. ª·˘ÚÔÂȉ‹
EÈ̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈ̤ӈÓ
™. ¡¿ÎÔ˘
EΉfiÙ˘
K. °ÚÈ‚¤·˜
™˘ÓÙÔÓÈÛÙ‹˜ ∂ΉfiÛˆ˜
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¶ÂÚȯfiÌÂÓ·
∞¡∞™∫O¶∏™∂π™
409 O ÚfiÏÔ˜ Ù˘ ·fiÙˆÛ˘ ÛÙË ‰È·‚ËÙÈ΋
ÂÌ‚Ú˘Ô¿ıÂÈ·
µ. ¢ÚfiÛÔ˘-∞Á·Î›‰Ô˘, ∂. ™ˆÙËÚ›‰Ô˘
417 O Û˘Ó‰˘·ÛÌfi˜ ÂÈÛÓÂfiÌÂÓˆÓ
ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Î·È ‚2-·ÁˆÓÈÛÙÒÓ
Ì·ÎÚ¿˜ ‰Ú¿Û˘ ÛÙË ıÂڷ›· ÙÔ˘
·È‰ÈÎÔ‡ ¿ÛıÌ·ÙÔ˜
ª.µ. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜, ∫.¡. ¶Ú›ÊÙ˘
425 ∞ÓÙÈÊÏÂÁÌÔÓ҉˘ ·ÁˆÁ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙÔ˜
Û˘ÚÈÁÌÔ‡ ÛÙ· ¤ÓÙ ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜
ª.µ. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜, º. ∫˘Ú‚·Û›Ï˘, π.¡. ∆۷ӿη˜
∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™
432 H Û˘Ì‚ÔÏ‹ Ù˘ ÚÒÈÌ˘ ‰È·Ù·Ú·¯‹˜
ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ηÈ
·Ú·ÌÔÓ‹ ˘„ËÏ‹˜ ·ÚÙËÚȷ΋˜ ›ÂÛ˘
ηٿ ÙË Ó‡ÎÙ· ÛÙËÓ ·ıÔÁ¤ÓÂÈ·
Ù˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜ Û ÂÊ‹‚Ô˘˜
Ì ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Ù‡Ô˘ 1
∫. K·Ú·‚·Ó¿ÎË, °. ∫·˙È¿Ó˘, Ã. ∫·Ú·ÁÈ¿ÓÓË, ∞. ™Î·Ú‰Ô‡ÙÛÔ˘, ∫. ∫·ÎϤ·˜, ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜
440 ™˘Û¯¤ÙÈÛË Î·Ù·Ó¿ÏˆÛ˘ ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜
Î·È ÎÏÈÓÈ΋˜ ηٿÛÙ·Û˘
·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË
∆. ªÔ‡‰ÈÔ˘, ∞. °·ÏÏ‹-∆ÛÈÓÔÔ‡ÏÔ˘, ∂. µ·Ì‚·ÎÔ‡‰Ë˜, ™. ¡Ô‡ÛÈ·-∞Ú‚·ÓÈÙ¿ÎË
∂¡¢π∞º∂ƒ√À™∂™ ¶∂ƒπ¶∆ø™∂π™
447 °ÂÓÂÙÈÎfi˜ ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚÈ·Îfi˜
¤ÏÂÁ¯Ô˜ Û ÔÈÎÔÁ¤ÓÂÈ· Ì ÎÏÈÓÈ΋
ÂÙÂÚÔÁ¤ÓÂÈ· Û˘Ó‰ÚfiÌÔ˘ Noonan
™. ∫›ÙÛÈÔ˘-∆˙¤ÏË, Õ. ¶··‰ÔÔ‡ÏÔ˘, ∞. •·˚‰¿Ú·, °. ¶··‰fiÔ˘ÏÔ˜, ∞.-ª. ª·ÁÈ¿ÎÔ˘, ∞. ∫ÔÏȷϤÍË, ∞. ª·‡ÚÔ˘, ∂. ∫·Ó·‚¿Î˘, ¶. ¡ÈÎÔÏ·˝‰Ô˘
452 ∞ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÛÙÂÔfiÚˆÛ˘ ηÈ
ÙˆÓ Î·Ù·ÁÌ¿ÙˆÓ Ì ‰ÈÓ·ÙÚÈÔ‡¯Ô
·ÌȉÚÔÓ¿ÙË Û ‚Ú¤ÊÔ˜ Ì ·ÙÂÏ‹
ÔÛÙÂÔÁ¤ÓÂÛË Ù‡Ô˘ IV
ª. ¶··Ê˘Ï·ÎÙÔ‡, ¶. §··ÙÛ¿Ó˘, ¶. ¶Ô˘ÏfiÔ˘ÏÔ˜, M.B. Petersen, ∫. ªÈ¯·Ï·Î¿ÎÔ˘, ∫. ¶ÚÔ‡ÓÙ˙Ô˘-∫·ÛÛÈÔ‡
458 ¶·Ú·ÛÔÓ‰˘ÏÈÎfi Â͈ÔÛÙÈÎfi Û¿ÚΈ̷
ÙÔ˘ Ewing: ¶ÂÚÈÁÚ·Ê‹ 2 ÂÚÈÙÒÛˆÓ
º. ∞ı·Ó·ÛÈ¿‰Ô˘, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜, ª. ∫Ô‡ÚÙË,£. ¶··ÁˆÚÁ›Ô˘, ¶. Ã˘Ù›ÚÔÁÏÔ˘, µ. ∫ÔÓÙfiÔ˘ÏÔ˜, ∞. ∫·ÏÔÁÂÚ¿, ∞. ¢Ú‚ÂϤÁη˜
463 °ÂÓÓ‹ÛÂȘ ·È‰ÈÒÓ ÌÂ
·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ ÌÂÙ¿ ·fi
˘Ô‚ÔËıÔ‡ÌÂÓË ÁÔÓÈÌÔÔ›ËÛË
π. ∆Û¿ÙÚ·, ∫. ∫·Ú·ÛÌ¿Ó˘, º. µ·ÚÂÏ¿˜, Ã. ∆Û¿ÓÙ·ÏË, ∞. ∫Ô‡ÛË, ª. ∞ı·Ó·Û›Ô˘
¶ƒ∞∫∆π∫O £∂ª∞
468 √‰ËÁ›Â˜ ÁÈ· ¿ÛÎËÛË Û ·È‰È¿ ηÈ
ÂÊ‹‚Ô˘˜ ÌÂ Û˘ÁÁÂÓ›˜ ‹ ›ÎÙËÙ˜
·ı‹ÛÂȘ ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡
¢. °ÂˆÚÁ·ÎfiÔ˘ÏÔ˜, °. ¶··‰fiÔ˘ÏÔ˜
478 µπµ§π√¶∞ƒ√À™π∞™∏
«∫ÏÈÓÈ΋ ¶·È‰È·ÙÚÈ΋»
479 ∫§π¡π∫√ ∫√Àπ∑
∞. ¶··‰ÔÔ‡ÏÔ˘, °. ƒÔ˘Ì¿ÓË, §. ™Ù·ÌÔÁÈ¿ÓÓÔ˘, ∂. ∫·ÙÛ·ÚÔ‡-¶ÂÎÙ·Û›‰Ë
480 ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√
™‡Á¯ÚÔÓ· ËÏÂÎÙÚÔÓÈο ÓËÈ·ÁˆÁ›·
¡. ¶··‰fiÔ˘ÏÔ˜
483 ∂Àƒ∂∆∏ƒπ√ ∫ƒπ∆ø¡ ∆√À ∆√ª√À 69
484 ∂Àƒ∂∆∏ƒπ√ ™À°°ƒ∞º∂ø¡ ∆√À ∆√ª√À 69
486 ∂Àƒ∂∆∏ƒπ√ §∂•∂ø¡ ∫§∂π¢πø¡ ∆√À∆√ª√À 69
∫ˆ‰ÈÎfi ̃¢È‡ı˘ÓÛË ̃∂ÔÙ›· ̃ªª∂: 3889
ISSN 0377-2551
¶·È‰È·ÙÚÈ΋∆fiÌÔ˜ 69 ñ ∆‡¯Ô˜ 6 ñ ¡Ô¤Ì‚ÚÈÔ˜-¢ÂΤ̂ÚÈÔ˜ 2006
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
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
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Contents
REVIEW ARTICLES
409 The role of apoptosis in diabetic
embryopathy
V. Drossou-Agakidou, E. Sotiridou
417 Combination therapy with inhaled
corticosteroids and long-acting
b2-agonists in childhood asthma
M. B. Anthracopoulos, K. N. Priftis
425 Anti-inflammatory therapy of recurrent
wheezing during the first five years of life
M. B. Anthracopoulos, F. Kyrvassilis, J. N. Tsanakas
ORIGINAL ARTICLES
432 The contribution of nocturnal blood
pressure change and autonomic
dysfunction in the pathogenesis of
microalbuminuria in adolescents
with type 1 diabetes mellitus
K. Karavanaki, G. Kazianis, C. Karagianni, A. Skardoutsou, C. Kakleas, A. Constantopoulos
440 Relation of resting energy expenditure
with the clinical condition in cystic
fibrosis patients
T. Moudiou, A. Galli-Tsinopoulou, E. Vamvacoudis, S. Noussia-Arvanitakis
CASE REPORTS
447 Molecular, clinical and laboratory
investigation in a Noonan syndrome
family presenting with clinical variability
S. Kitsiou-Tzeli, A. Papadopoulou, A. Xaidara, G. Papadopoulos, A.-M.- Magiakou, A. Kolialexi,A. Mavrou, E. Kanavakis, P. Nikolaidou
452 Symptomatic pamidronate treatment
of an infant with osteogenesis
imperfecta type IV
M. Papaphylactou, P. Lapatsanis, P. Poulopoulos, M. B. Petersen, K. Michalakakou, K. Prountzou-Kassiou
458 Extraskeletal Ewing’s sarkoma:
report of 2 cases
F. Athanassiadou, A. Tragiannidis, M. Kourti,T. Papageorgiou, P. Chytiroglou, V. Kontopoulos, A. Kalogera, A. Drevelegas
463 Birth of children with
hemoglobinopathies following use of
IVF
I. Tsatra, K. Karasmanis, F. Varelas, C. Tsantali, A. Koussi, M. Athanassiou
PRACTICAL ISSUE
468 Exercise recommendations for children
and adolescents with congenital and
acquired cardiovascular disorders
D. Georgakopoulos, G. Papadopoulos
478 BOOK PRESENTATION
479 CLINICAL QUIZ
A. Papadopoulou, G. Roumani, L. Stamogiannou, E. Katsarou-Pectasidi
480 NEWS FROM THE INTERNET
Modern e-kindergardens
N. Papadopoulos
483 REVIEWERS OF MANUSCRIPTS INDEX,VOLUME 69
484 AUTHORS INDEX, VOLUME 69
486 SUBJECT INDEX, VOLUME 69
PaediatrikiVolume 69 ñ Number 6 ñ November-December 2006
xiii√¢∏°π∂™ ¶ƒ√™ ∆√À™ ™À°°ƒ∞º∂π™
A. °ÂÓÈΤ˜ ¶ÏËÚÔÊÔڛ˜∆Ô ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ΋” Â›Ó·È Ë ÂÈÛÙËÌÔÓÈ΋ ¤Î-
‰ÔÛË Î·È È‰ÈÔÎÙËÛ›· Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›-·˜, Ô˘ ‰È·Ó¤ÌÂÙ·È ÛÙ· ̤ÏË Ù˘. Œ¯ÂÈ ˆ˜ ‚·ÛÈÎÔ‡˜ ÛÙfi-¯Ô˘˜ ÙËÓ ·ÔÙ‡ˆÛË ÙÔ˘ ·È‰È·ÙÚÈÎÔ‡ ÂÈÛÙËÌÔÓÈÎÔ‡¤ÚÁÔ˘ Î·È ÙË Û˘Ó¯‹ ÂÓË̤ڈÛË ÙˆÓ ¶·È‰È¿ÙÚˆÓ. °È· ÙÔÛÎÔfi ·˘Ùfi ‰¤¯ÂÙ·È ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÔÈÎÈÏ›· ¿ÚıÚˆÓ Î·ÈÛ˘ÁÎÂÎÚÈ̤ӷ:
1. ÕÚıÚ· Û‡ÓÙ·Í˘ (ÌÂÙ¿ ·fi ÚfiÛÎÏËÛË Ù˘ ™˘ÓÙ·-ÎÙÈ΋˜ EÈÙÚÔ‹˜).
2. ∞Ó·ÛÎÔ‹ÛÂȘ.3. µÚ·‚Â˘Ì¤Ó˜ ÂÚÁ·Û›Â˜.4. ∂Ú¢ÓËÙÈΤ˜ ÌÂϤÙ˜.5. ∫ÏÈÓÈο ∫Ô˘›˙.6. ∂ÈÏÂÁ̤Ó˜ Û˘˙ËÙ‹ÛÂȘ ÛÙÚÔÁÁ˘ÏÒÓ ÙÚ·Â˙ÒÓ.7. ∂›Î·ÈÚ· ı¤Ì·Ù·.8. £¤Ì·Ù· Âη›‰Â˘Û˘ Î·È ÔÚÁ¿ÓˆÛ˘ ˘Á›·˜.9. ∂ӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ.
10. ™‡ÓÙÔÌ· Ó¤·.11. µÚ·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ.12. ∂ÈÛÙÔϤ˜ ÚÔ˜ ÙË Û‡ÓÙ·ÍË.13. ¶ÂÚÈÏ‹„ÂȘ Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜.14. ∞Ó·ÎÔÈÓÒÛÂȘ ÚÔÛ¯ÒÓ Û˘Ó‰ڛˆÓ Î·È ÂÈÛÙËÌÔÓÈ-
ÎÒÓ ÂΉËÏÒÛˆÓ15. ∞Ó·ÊÔÚ¤˜ Î·È ÎÚÈÙÈ΋ Ó¤ˆÓ ÂΉfiÛˆÓ, ÂÏÏËÓÈÎÒÓ
Î·È Í¤ÓˆÓ, ·È‰È·ÙÚÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜.
H ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ó· ‰Ë-ÌÔÛȇÂÈ ¿ÚıÚ· Ì ȉȷ›ÙÂÚÔ ÂÈÛÙËÌÔÓÈÎfi ÂӉȷʤÚÔÓ,ηıÒ˜ Î·È ¿ÚıÚ· Ì ›ηÈÚ· ı¤Ì·Ù· ¯ˆÚ›˜ Ó· ÙËÚÂ›Ù·È ËÛÂÈÚ¿ ˘Ô‚ÔÏ‹˜. ∂›Û˘, ÌÔÚ› Ó· ·ÔÊ·Û›˙ÂÈ ÁÈ· ÙË ‰Ë-ÌÔÛ›Â˘ÛË ÂÚÁ·ÛÈÒÓ Ô˘ ¤¯Ô˘Ó ·ÚÔ˘ÛÈ·ÛÙ› ÛÙÔ ÂÙ‹ÛÈÔ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ÙËÓ ÔÏÈ΋ ‹ ÌÂÚÈ΋ ‰ËÌÔÛ›Â˘ÛË·ÚÔ˘ÛÈ¿ÛÂˆÓ È‰È·›ÙÂÚÔ˘ ÂӉȷʤÚÔÓÙÔ˜ Î·È ÙËÓ ÔÏÈ΋ ‹ÌÂÚÈ΋ ‰ËÌÔÛ›Â˘ÛË ÂÈÛÙÔÏÒÓ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ‰ËÌÔ-ÛÈÂ˘Ì¤Ó· ÂÈÛÙËÌÔÓÈο ¿ÚıÚ· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡.
™ÙȘ ÂÚÁ·Û›Â˜ Ì ›ηÈÚ· ı¤Ì·Ù· Ú¤ÂÈ Ó· ·Ó·ÁÚ¿-ÊÂÙ·È ÛÙËÓ ÚÒÙË ÛÂÏ›‰· Ë ÚfiıÂÛË ÙˆÓ Û˘ÁÁڷʤˆÓÁÈ· ÙËÓ ·Ó·ÁηÈfiÙËÙ· Ù˘ Ù·¯Â›·˜ ‰ËÌÔÛ›Â˘Û˘. ∏ ∂È-ÙÚÔ‹ ™‡ÓÙ·Í˘ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ù˘ ·Ô‰Ô¯‹˜ Ù˘ٷ¯Â›·˜ ‰ËÌÔÛ›Â˘Û˘.
ŸÏ˜ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ÌËÓ ¤¯Ô˘Ó ÂÓ Ì¤ÚÂÈ ‹ ÂÍÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚ÏËı› ÁÈ· ÎÚ›ÛË Û ¿ÏÏÔÂÚÈÔ‰ÈÎfi. ∂ÈϤÔÓ, ÛÙËÓ ÂÚÁ·Û›· ı· Ú¤ÂÈ Ó· ·Ó·Ê¤-ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘Ô-ÛÙ‹ÚÈ͢. ŸÏ˜ ÔÈ ÎÏÈÓÈΤ˜ ¤Ú¢Ó˜ Ú¤ÂÈ Ó· ¤¯Ô˘Ó Á›ÓÂÈÌÂÙ¿ ·fi ÏËÚÔÊÔÚË̤ÓË Û˘Ó·›ÓÂÛË ÙˆÓ ÌÂÙ¯fiÓÙˆÓ ‹ÙˆÓ ÓÔÌ›ÌˆÓ ÂÎÚÔÛÒˆÓ ÙÔ˘˜, Û‡Ìʈӷ Ì ÙȘ ‰È·ÎËÚ‡-ÍÂȘ ÙÔ˘ EÏÛ›ÓÎÈ Î·È ÙÔ˘ TfiÎÈÔ. E›Û˘, Ú¤ÂÈ Ó· ¤¯Ô˘ÓÙËÚËı› ÔÈ Ô‰ËÁ›Â˜ ÁÈ· ÙË ÊÚÔÓÙ›‰· ÂÈÚ·Ì·Ùfi˙ˆˆÓ ÙÔ˘∂ıÓÈÎÔ‡ πÓÛÙÈÙÔ‡ÙÔ˘ ÀÁ›·˜ ÙˆÓ ∏¶∞ (DHEWPublication, NIH, 80-23). OÈ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Ú¤ÂÈ Ó·¤¯Ô˘Ó ÂÁÎÚÈı› ·fi ÙËÓ ÂÈÙÚÔ‹ ∏ıÈ΋˜ Î·È ¢ÂÔÓÙÔÏÔ-Á›·˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ.
OÈ ·fi„ÂȘ Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓÔ˘ ‰È·Ù˘ÒÓÔÓÙ·È ÛÙȘ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ‰ÂÓ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ··Ú·›ÙËÙ· ·˘Ù¤˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. Hηٷ¯ÒÚÈÛË ‰È·ÊËÌ›ÛÂˆÓ ÛÙÔ ÂÚÈÔ‰ÈÎfi ‰ÂÓ ˘Ô‰ËÏÒÓÂÈÔˆÛ‰‹ÔÙ ¤ÁÎÚÈÛË ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘˜ ·fi ÙËÓEÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·, ÙË ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹‹ ·fi ÙÔÓ EΉfiÙË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡.
ŸÏ˜ ÔÈ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ıˆÚÔ‡ÓÙ·È È‰ÈÔÎÙË-Û›· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ “¶·È‰È·ÙÚÈ΋” Î·È Ë ÔÏÈ΋ ‹ ÌÂÚÈ΋
·Ó·‰ËÌÔÛ›Â˘Û‹ ÙÔ˘˜ ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ ÌÂÙ¿ ·fi ¤ÁÁÚ·ÊË
Û˘ÁηٿıÂÛË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡.
µ. ™‡ÓÙ·ÍË ÙˆÓ ∂ÚÁ·ÛÈÒӟϘ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ·ÎÔÏÔ˘ıÔ‡Ó “∫ÔÈÓ¤˜ ¶ÚÔ-
‰È·Áڷʤ˜ ÁÈ· ÃÂÈÚfiÁÚ·Ê· Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È Û µÈÔ˚·-
ÙÚÈο ¶ÂÚÈÔ‰Èο” (Uniform Requirements for Manuscripts
Submitted to Biomedical Journals), ÔÈ Ôԛ˜ ÙÚÔÔÔÈ‹-
ıËÎ·Ó ÚfiÛÊ·Ù· Î·È ‰ËÌÔÛȇÔÓÙ·È ÛÙȘ ‰È¢ı‡ÓÛÂȘ:
http://www.icmje.org Î·È http://www.icmje.org/icmje.pdf
TÔ Û‡ÓÔÏÔ Ù˘ ÂÚÁ·Û›·˜ (Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ ÙˆÓ
ÈÓ¿ÎˆÓ Î·È ÙˆÓ Ù›ÙÏˆÓ ÙˆÓ ÂÈÎfiÓˆÓ) Ú¤ÂÈ Ó· Â›Ó·È Ù˘-
ˆÌ¤ÓÔ ÛÙË ÌÈ· fi„Ë Ï¢ÎÔ‡ ¯·ÚÙÈÔ‡ ÌÂÁ¤ıÔ˘˜ A4
(21x29,7 cm), Ì ‰ÈÏfi ‰È¿ÛÙËÌ· ÌÂٷ͇ ÙˆÓ ÁÚ·ÌÌÒÓ Î·È
ÂÚÈıÒÚÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 2,5 cm ÛÙȘ ‰‡Ô Ï¢ڤ˜.
TÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ¤¯ÂÈ ÙËÓ ·ÎfiÏÔ˘ıË ‰È¿Ù·ÍË: ÛÂ-
Ï›‰· Ù›ÙÏÔ˘, ‚Ú·¯‡˜ Ù›ÙÏÔ˜, ÂÚ›ÏË„Ë ÛÙ· ÂÏÏËÓÈο Î·È Ù·
·ÁÁÏÈο, ηٿÏÔÁÔ˜ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ, ΛÌÂÓÔ, ¢¯·ÚÈ-
Ûٛ˜ ηıÒ˜ Î·È ·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿Ï-
Ϙ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢, ‚È‚ÏÈÔÁÚ·Ê›·, ›Ó·Î˜, ÂÈÎfiÓ˜,
Ù›ÙÏÔÈ ÂÈÎfiÓˆÓ. K¿ı ¤Ó· ·fi Ù· ·Ú·¿Óˆ ÛÙÔȯ›· Ú¤-
ÂÈ Ó· ·Ú¯›˙ÂÈ Û ¯ˆÚÈÛÙfi ʇÏÏÔ Î·È ÔÈ ÛÂÏ›‰Â˜ Ó· ·ÚÈı-
ÌÔ‡ÓÙ·È ‰È·‰Ô¯Èο ·Ú¯›˙ÔÓÙ·˜ Ì ÙË ÛÂÏ›‰· Ù›ÙÏÔ˘.
H ¤ÎÙ·ÛË ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ÁÈ·:
ñ ÙȘ ·Ó·ÛÎÔ‹ÛÂȘ Û 2000-3000 ϤÍÂȘ.
ñ ÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ 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 ÔÓÔ-
Ì·ÛÙÈ΋.
ñ ÙÔ ÂÈÛÙËÌÔÓÈÎfi ΤÓÙÚÔ (›‰Ú˘Ì·, ÎÏÈÓÈ΋, ÂÚÁ·ÛÙ‹-
ÚÈÔ) ·fi fiÔ˘ ÚÔ¤Ú¯ÂÙ·È Ë ÂÚÁ·Û›·. ™Â ÂÚ›ÙˆÛË
¤ÏÏÂȄ˘ Û˘ÓÂÚÁ·Û›·˜ ÌÂ Û˘ÁÎÂÎÚÈ̤ӷ ΤÓÙÚ·, Ú¤-
ÂÈ Ó· ·Ó·Ê¤ÚÂÙ·È Ë È‰ÈfiÙËÙ· ÙÔ˘/ÙˆÓ Û˘ÁÁڷʤ·/ˆÓ
(.¯. ȉÈÒÙ˘ ·È‰›·ÙÚÔ˜) Î·È Ô ÙfiÔ˜ ‰È·ÌÔÓ‹˜
ÙÔ˘/ÙÔ˘˜.
ñ ÙËÓ Ï‹ÚË ‰È‡ı˘ÓÛË, e-mail Î·È ÙÔ ÙËϤʈÓÔ ÙÔ˘
Û˘ÁÁڷʤ· Ì ÙÔÓ ÔÔ›Ô Á›ÓÂÙ·È Ë ·ÏÏËÏÔÁÚ·Ê›·.
¶ÂÚÈÏ‹„ÂȘH ÂÚ›ÏË„Ë Ú¤ÂÈ Ó· ·Ó·ÎÂÊ·Ï·ÈÒÓÂÈ ÙÔ˘˜ ÛÙfi¯Ô˘˜
Ù˘ ÂÚÁ·Û›·˜, ÙË ÌÂıÔ‰ÔÏÔÁ›·, Ù· ΢ÚÈfiÙÂÚ· ·ÔÙÂϤÛÌ·-
Ù· Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ù˘ ÌÂϤÙ˘.
ñ £· Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ 200 ϤÍÂȘ,
fï˜ ‰ÂÓ ı· Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÂÈ ÙȘ 250 ϤÍÂȘ.
ñ ∏ ÂÚ›ÏË„Ë ÙˆÓ ÂÚÁ·ÛÈÒÓ Ú¤ÂÈ Ó· Â›Ó·È ‰ÔÌË̤ÓË
ÛÙȘ ÂÍ‹˜ ·Ú·ÁÚ¿ÊÔ˘˜: ÂÈÛ·ÁˆÁ‹, ̤ıÔ‰ÔÈ, ·ÔÙÂϤ-
ÛÌ·Ù· Î·È Û˘ÌÂÚ¿ÛÌ·Ù·.
™ÙËÓ ÂÚ›ÏË„Ë ÛÙ· ·ÁÁÏÈο Ú¤ÂÈ Ó· ·Ó·ÁÚ¿ÊÔ-
ÓÙ·È ÛÙËÓ ·Ú¯‹ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Ô Ù›ÙÏÔ˜ Ù˘ ÂÚÁ·Û›·˜ Î·È Ù·
ÔÓfiÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ ÛÙ· ·ÁÁÏÈο. TÔ ÂÚȯfiÌÂÓÔ
ÙÔ˘ ·ÁÁÏÈÎÔ‡ ÎÂÈ̤ÓÔ˘ Ú¤ÂÈ Ó· Â›Ó·È ‰ÔÌË̤ÓÔ ÛÂ: ÂÈ-
Û·ÁˆÁ‹ (background), ̤ıÔ‰ÔÈ (methods), ·ÔÙÂϤÛÌ·-
Ù· (results) Î·È Û˘ÌÂÚ¿ÛÌ·Ù· (conclusions). H ÂÚ›ÏË-
„Ë ÛÙ· ·ÁÁÏÈο ‰ÂÓ Ú¤ÂÈ Ó· ‰È·Ê¤ÚÂÈ ·fi ÙËÓ ·ÓÙ›-
ÛÙÔÈ¯Ë ÛÙ· ÂÏÏËÓÈο.
K¿Ùˆ ·fi ÙËÓ ÂÏÏËÓÈ΋ Î·È ÙËÓ ·ÁÁÏÈ΋ ÂÚ›ÏË„Ë ÛË-ÌÂÈÒÓÔÓÙ·È ÙÚÂȘ ¤ˆ˜ ¤ÓÙ ϤÍÂȘ ÎÏÂȉȿ Ô˘ ı· ¯ÚËÛÈ-ÌÔÔÈËıÔ‡Ó ÁÈ· ÙÔ ıÂÌ·ÙÈÎfi ¢ÚÂÙ‹ÚÈÔ.
K›ÌÂÓÔOÈ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ÂÈÛ·ÁˆÁ‹,
ÌÂıfi‰Ô˘˜, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘˙‹ÙËÛË. H ÂÈÛ·ÁˆÁ‹ Â-ÚÈÏ·Ì‚¿ÓÂÈ Ù· ÙÂÏÂ˘Ù·›· ‰Â‰Ô̤ӷ Ù˘ ¤Ú¢ӷ˜ ÛÙÔ Û˘-ÁÎÂÎÚÈ̤ÓÔ ı¤Ì· Ì ÙȘ ΢ÚÈfiÙÂÚ˜ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·-Ú·Ô̤˜ Î·È ÙÔÓ ÛÎÔfi Ù˘ ÂÚÁ·Û›·˜. H ÂÚÈÁÚ·Ê‹ ÙˆÓÌÂıfi‰ˆÓ Ú¤ÂÈ Ó· Â›Ó·È ·ÎÚÈ‚‹˜ Î·È ·ÚÎÂÙ¿ ÏÂÙÔÌÂ-Ú‹˜, ÒÛÙ ӷ ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó··Ú·ÁˆÁ‹ ÙÔ˘˜ ·fi ¿Ï-ÏÔ˘˜ ÂÚ¢ÓËÙ¤˜. ∂›Û˘, Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ì ÏÂ-ÙÔ̤ÚÂȘ ÔÈ ÛÙ·ÙÈÛÙÈΤ˜ ̤ıÔ‰ÔÈ ·Ó¿Ï˘Û˘ Î·È ·ÍÈÔÏfi-ÁËÛ˘ ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ. T· ·ÔÙÂϤÛÌ·Ù· Ú¤ÂÈÓ· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ۷ʋÓÂÈ· Î·È Ó· Û˘Óԉ‡ÔÓÙ·È·fi ÙËÓ ··Ú·›ÙËÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË. H Û˘˙‹ÙËÛËÚ¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ô˘ ÚÔ·-ÙÔ˘Ó ·fi ÙËÓ ÂÚÁ·Û›·, ÙË ÛËÌ·Û›· Ô˘ ÌÔÚ› Ó· ¤¯Ô˘ÓÎ·È ÙËÓ Èı·Ó‹ Û˘Û¯¤ÙÈÛ‹ ÙÔ˘˜ Ì ·Ú·ÙËÚ‹ÛÂȘ ¿ÏψÓÂÚ¢ÓËÙÒÓ.
OÈ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û‡-ÓÙÔÌË ÂÈÛ·ÁˆÁ‹, ÂÚÈÁÚ·Ê‹ Ù˘ ÂÚ›ÙˆÛ˘ Î·È ‚Ú·¯Â›·Û˘˙‹ÙËÛË, Ì ¤ÌÊ·ÛË ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË.
T· ˘fiÏÔÈ· ı¤Ì·Ù· ¤¯Ô˘Ó ÂχıÂÚË ‰ÔÌ‹ ηٿ ÙËÓÎÚ›ÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ÙÔ˘˜.
∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹-ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢) ı· Ú¤ÂÈ Ó··Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Ù¤ÏÔ˜ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Î·È ÚÈÓ ·fi ÙȘ ‚È-‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜.
TÈ̤˜ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛˆÓOÈ ÙÈ̤˜ ÙˆÓ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ Ú¤ÂÈ Ó· ÂÎ-
ÊÚ¿˙ÔÓÙ·È ÛÙÔ ¢ÈÂıÓ¤˜ ™‡ÛÙËÌ· MÔÓ¿‰ˆÓ (SI Units) ηÈÛÙÔ MÂÙÚÈÎfi (Conventional-™˘Ì‚·ÙÈÎfi) ™‡ÛÙËÌ· ̤۷ Û·ڤÓıÂÛË. ¶›Ó·Î˜ ÌÂÙ·ÙÚÔ‹˜ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙȘ‰È¢ı‡ÓÛÂȘ:http://www.icmje.org Î·È http://www.icmje.org/icmje.pdf
™˘ÓÙÔÌÔÁڷʛ˜OÈ Û˘ÓÙÔÌÔÁڷʛ˜ Ô˘ ¤¯Ô˘Ó ÂÈ‚ÏËı› ‰ÈÂıÓÒ˜,
‰ËÌÔÛȇÔÓÙ·È Û οı Ù‡¯Ô˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ™‡ÓıÂÙÔÈÎ·È Ì·ÎÚÔÛÎÂÏ›˜ fiÚÔÈ Ô˘ ·ӷϷ̂¿ÓÔÓÙ·È Û˘¯Ó¿ ÛÙÔ Î›ÌÂÓÔ ÌÔÚÔ‡Ó Ó· ·ÓÙÈηı›ÛÙ·ÓÙ·È ·fi Û˘ÓÙÔÌÔ-Áڷʛ˜ Ô˘ ÂÂÍËÁÔ‡ÓÙ·È ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ Û ÂȉÈ-Îfi ηٿÏÔÁÔ, Ô ÔÔ›Ô˜ ˘Ô‚¿ÏÏÂÙ·È Ì·˙› Ì ÙËÓ ÂÚÁ·Û›·. OÈ Û˘ÓÙÔÌÔÁڷʛ˜ ·Ó·Ê¤ÚÔÓÙ·È Û ·Ú¤ÓıÂÛË ÌfiÓÔÛÙȘ ÂÚÈÏ‹„ÂȘ.
BÈ‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜™ÙÔ ÙÌ‹Ì· Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ ηٷ¯ˆÚÔ‡ÓÙ·È fiϘ
ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ·Ó·Ê¤-ÚÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ™ÙÔ Î›ÌÂÓÔ, ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·-Ú·Ô̤˜ ·Ó·Ê¤ÚÔÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Û ·-Ú¤ÓıÂÛË. OÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ ‰ÂÓ Ú¤ÂÈ Ó·˘ÂÚ‚·›ÓÔ˘Ó:
ñ ÙȘ 70 ÛÙȘ ·Ó·ÛÎÔ‹ÛÂȘ. ñ ÙȘ 30 ÛÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜. ñ ÙȘ 12 ÛÙ· ›ηÈÚ· ı¤Ì·Ù· Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜
ÂÚÈÙÒÛÂȘ. ñ ÙȘ 5 ÛÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ Î·È ÙȘ ÂÈÛÙÔϤ˜.
H Û‡ÓÙ·ÍË ÙˆÓ ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ Á›ÓÂÙ·ÈÛ‡Ìʈӷ Ì ÙȘ ÚfiÛÊ·Ù· ÙÚÔÔÔÈË̤Ó˜ ÚԉȷÁÚ·-ʤ˜ Ù˘ International Committee of Medical Journal
Editors/ Uniform Requirements for Manuscripts Sub-mittedto Biomedical Journals, (http://www.icmje.org Î·È http://www.icmje.org/icmje.pdf). OÈ Û˘ÓÙÌ‹ÛÂȘ ÙˆÓ Ù›ÙÏˆÓ ÙˆÓÂÚÈÔ‰ÈÎÒÓ Á›ÓÔÓÙ·È Ì ‚¿ÛË ÙÔ Cumulated IndexMedicus [List of Journals Indexed in Index Medicus(http://www.nlm.nih.gov/bsd/uniform_requirements.html)].
¶·Ú·‰Â›ÁÌ·Ù· ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ
I. ¶∂ƒπO¢π∫∞
AÓ ÔÈ Û˘ÁÁÚ·Ê›˜ Â›Ó·È ¤ˆ˜ ¤ÍÈ, ·Ó·ÁÚ¿ÊÔÓÙ·È fiÏÔÈ,ÂÓÒ ·Ó Â›Ó·È ÂÙ¿ ‹ ÂÚÈÛÛfiÙÂÚÔÈ, ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ ÚÒ-ÙÔÈ ¤ÍÈ Î·È ÚÔÛÙ›ıÂÙ·È “et al” ‹ “Î·È Û˘Ó”.
T·ÎÙÈ΋ ¤Î‰ÔÛË ÂÚÈÔ‰ÈÎÔ‡:
∞ÓÙˆÓ›Ô˘ ¢, ª¿Ú· Ã, °È·ÓÓ¿ÙÔ˘ Ã. ∂Ì‚fiÏÈ·: ›‰Ú·-ÛË ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ÙˆÓ ÏÔÈ̈‰ÒÓ ÓÔÛËÌ¿ÙˆÓ Î·È ÙËӷȉȷÙÚÈ΋ Ú¿ÍË. ¶·È‰È·ÙÚÈ΋ 1999;59:272-279.
Proesmans W. Bartter syndrome and its neonatalvariant. Eur J Pediatr 1997;156:669-679.
™˘ÌÏËڈ̷ÙÈÎfi Ù‡¯Ô˜ ÂÚÈÔ‰ÈÎÔ‡:
Flyvbjerg A. Role of growth hormone, insulin-likegrowth factors (IGFs) and IGF-binding proteins in the renalcomplications of diabetes. Kidney Int 1997;52 (60Suppl):S12-S19.
Èڛ˜ Û˘ÁÁڷʤ·:
National Institutes of Health Consensus DevelopmentConference. Neurofibromatosis conference statement. ArchNeurol 1988;45:575-578.
¶ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù‡Ô˘ ¿ÚıÚÔ˘:
Schreiner GF, Lange L. Ethanol modulation ofmacrophage influx in glomerulonephritis [Abstract]. J AmSoc Nephrol 1991;2:562.
Should antileukotriene therapies be used instead of in-haled corticosteroids in asthma? [Editorial]. Am J RespirCrit Care Med 1998;158:1697-1701.
Laux-End R, Inaebnit D, Gerber HA, Bianchetti MG.Vasculitis associated with levamisole and circulating au-toantibodies [Letter]. Arch Dis Child 1996;75:355-356.
II. µπµ§π∞
∫ÂÊ¿Ï·ÈÔ Û ‚È‚Ï›Ô:
Clark AG, Barratt TM. Steroid-responsive nephroticsyndrome. In: Barratt TM, Arner ED, Harmon WE, editors.Pediatric Nephrology. 4th ed. Baltimore: Lippincott Wil-liam Wilkins; 1999. p. 742.
™‡ÁÁÚ·ÌÌ· ‹ ÌÔÓÔÁÚ·Ê›·:
Gorlin RJ, Cohen MM, Levin LS. Syndromes of the headand neck. 3rd ed. New York: Oxford University Press; 1990.
¢ËÌÔÛ›Â˘ÛË Û ÙfiÌÔ Ú·ÎÙÈÎÒÓ:
Bauer AW. The two definitions of bacterial resistance.In: Smith AJ, Rogers CA, eds. Proceedings of the Third In-ternational Congress of Chemotherapy; 1962 May 29-31;New York: International Society of Chemotherapy; 1963. p.484-500.
¢È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹:
¶··‰fiÔ˘ÏÔ˜ Ã. ∏ ıÂڷ›· ÙÔ˘ ÛÙÚ·‚ÈÛÌÔ‡ [‰È‰·-ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹]. ∞ı‹Ó·: ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ; 1979.
xiv
xv
Kaplan SJ. Post hospital home health care: the elderly’saccess and utilization [dissertation]. St. Louis (Mo): Wash-ington Univ.; 1995.
πππ. CD-ROMAnderson SC, Poulsen KB. Anderson’s electronic atlas
of hematology [CD-ROM]. Philadelphia: LippincottWilliams & Wilkins; 2002.
IV. ™∆O ¢π∞¢π∫∆ÀOÕÚıÚÔ Û ÂÚÈÔ‰ÈÎfi:
Abood S. Quality improvement initiative in nursinghomes: the ANA acts in an advisory role. Am J Nurs[Internet]. 2002 Jun: Webpage:http://www.nursingworld.org/AJN/2002/june/Wawatch.htm
ªÔÓÔÁÚ·Ê›·:Foley KM, Gelband H, editors. Improving palliative
care for cancer [Monograph, Internet]. Washington:National Academy Press; 2001. Webpage: http://www.nap.edu/books/0309074029/html
πÛÙÔÛÂÏ›‰Â˜:Cancer-Pain.org [Webpage, Internet]. New York:
Association of Cancer Online Resources, Inc.; 2002:http://www.cancer-pain.org/
¶›Ó·Î˜ Î·È EÈÎfiÓ˜AÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó (¤Ó· ÚˆÙfiÙ˘Ô Î·È ‰‡Ô
ʈÙÔ·ÓÙ›ÁÚ·Ê·). ¶Ú¤ÂÈ Ó· ¤¯Ô˘Ó Ï¿ÙÔ˜ ›ÛÔ Ì ÙÔ Ï¿-ÙÔ˜ ÙÔ˘ ÌÔÓfiÛÙËÏÔ˘ (7,5 cm) ‹ Ì ÙÔ Ï¿ÙÔ˜ fiÏ˘ Ù˘ ÛÂ-Ï›‰·˜ (15,5 cm). TÔ Ì¤ÁÈÛÙÔ Ì‹ÎÔ˜ ÙÔ˘˜, Ì·˙› Ì ÙÔ˘˜ Ù›Ù-ÏÔ˘˜, ‰ÂÓ Ú¤ÂÈ Ó· Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ·fi 22 cm.
OÈ ›Ó·Î˜ ·ÚÈıÌÔ‡ÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Ì ÙËÛÂÈÚ¿ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ¶ÂÚÈÏ·Ì‚¿ÓÔ˘Ó‚Ú·¯‡ Ù›ÙÏÔ Î·È ÂÂÍ‹ÁËÛË ÙˆÓ Û˘ÓÙÌ‹ÛÂˆÓ ÛÙÔ Î¿Ùˆ ̤-ÚÔ˜. ™ÙÔ˘˜ ›Ó·Î˜ Ú¤ÂÈ Ó· ·ÔʇÁÔÓÙ·È ÔÈ Î¿ıÂÙ˜ÁÚ·Ì̤˜.
ŸÏÔ ÙÔ ·ÂÈÎÔÓÈÛÙÈÎfi ˘ÏÈÎfi (Û¯‹Ì·Ù·, ‰È·ÁÚ¿ÌÌ·Ù·,ʈÙÔÁڷʛ˜, Î.Ï.) ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ÂÈÎfiÓ˜ Î·È ı·Ú¤ÂÈ Ó· Â›Ó·È ¿ÚÈÛÙ˘ ÔÈfiÙËÙ·˜. ™ÙÔ ›Ûˆ ̤ÚÔ˜ ÙˆÓÂÈÎfiÓˆÓ Ú¤ÂÈ Ó· ÛËÌÂÈÒÓÂÙ·È Ì ÌÔχ‚È Ô ·ÚÈıÌfi˜ Ù˘ÂÈÎfiÓ·˜ Î·È ÙÔ fiÓÔÌ· ÙÔ˘ ÚÒÙÔ˘ Û˘ÁÁڷʤ·, ηıÒ˜ ηȤӷ ‚¤ÏÔ˜ ÙÔ ÔÔ›Ô Ó· ‰Â›¯ÓÂÈ ÙÔ ¿Óˆ ̤ÚÔ˜ Ù˘ ÂÈÎfiÓ·˜.™ÙȘ ʈÙÔÁڷʛ˜ ·ÛıÂÓÒÓ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·ÁÓˆÚ›˙ÂÙ·ÈË Ù·˘ÙfiÙËÙ¿ ÙÔ˘˜. OÈ ·ÛıÂÓ›˜ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·ÈÔÓÔÌ·ÛÙÈο.
°. ÀÔ‚ÔÏ‹ Î·È ¢ËÌÔÛ›Â˘ÛË ÙˆÓ ∂ÚÁ·ÛÈÒӟϘ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· ˘Ô‚¿ÏÏÔÓÙ·È ÂȘ ÙÚÈ-
ÏÔ‡Ó Î·È Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ‰ÈÛΤٷ ‹ CD, ηıÒ˜ ηȷfi ÂÈÛÙÔÏ‹, Ë ÔÔ›· ı· Û˘Ó˘ÔÁÚ¿ÊÂÙ·È ·fi fiÏÔ˘˜ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜, ÛÙËÓ ÔÔ›· ı· ‰ËÏÒÓÂÙ·È fiÙÈ Ë ÂÚÁ·Û›·‰ÂÓ ¤¯ÂÈ ÂÓ Ì¤ÚÂÈ ‹ ÂÍ ÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚ÏË-ı› ÁÈ· ÎÚ›ÛË Û ¿ÏÏÔ ÂÚÈÔ‰ÈÎfi Î·È fiÙÈ ÔÈ Û˘ÁÁÚ·Ê›˜ÂÁÎÚ›ÓÔ˘Ó ÙË ‰ËÌÔÛ›Â˘Û‹ Ù˘ ÛÙÔ ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ-΋”. ∂ÈϤÔÓ, ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹-ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢.
EÊfiÛÔÓ Ë ÂÚÁ·Û›· Á›ÓÂÈ ·Ô‰ÂÎÙ‹, ÙÔ ‰ÈÔÚıˆÌ¤ÓÔÛ‡Ìʈӷ Ì ÙȘ ˘Ô‰Â›ÍÂȘ ÙˆÓ ÎÚÈÙÒÓ Î·È ÂÎ Ó¤Ô˘ ‰·ÎÙ˘-ÏÔÁÚ·ÊË̤ÓÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ˘Ô‚¿ÏÂÙ·È ÛÙË ™˘ÓÙ·-ÎÙÈ΋ EÈÙÚÔ‹, Û˘Óԉ¢fiÌÂÓÔ ·fi ‰ÈÛΤٷ ‹ CD Ô˘ Ó·ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÚÁ·Û›· Û ÚfiÁÚ·ÌÌ· Word, ηıÒ˜Î·È ÂÈÛÙÔÏ‹ fiÔ˘ Ó· ·Ó·Ê¤ÚÔÓÙ·È ·Ó·Ï˘ÙÈο ÔÈ ÙÚÔÔ-ÔÈ‹ÛÂȘ ‹ ÔÈ ·ÓÙÈÚÚ‹ÛÂȘ ÛÙȘ ÚÔÙ¿ÛÂȘ ÙˆÓ ÎÚÈÙÒÓ.
H ηı˘ÛÙ¤ÚËÛË Ù˘ ·ӢԂÔÏ‹˜ ÙÔ˘ ÙÚÔÔÔÈ-Ë̤ÓÔ˘ ÎÂÈ̤ÓÔ˘ ¤Ú·Ó ÙˆÓ 30 ËÌÂÚÒÓ Û˘Ó¿ÁÂÙ·ÈÓ¤· ˘Ô‚ÔÏ‹.
T· ¤ÍÔ‰· ÙˆÓ ÊÈÏÌ Î·È ÙˆÓ ·Ó·Ù‡ˆÓ ‚·Ú‡ÓÔ˘Ó ÙÔ˘˜Û˘ÁÁÚ·Ê›˜ Î·È Ú¤ÂÈ Ó· ÏËÚÒÓÔÓÙ·È Ì ÙËÓ ·ÔÛÙÔÏ‹ÙÔ˘ ÚÒÙÔ˘ ‰ÔÎÈÌ›Ô˘ ηÙ¢ı›·Ó ÛÙÔÓ Ù˘ÔÁÚ¿ÊÔ.
T· ΛÌÂÓ· ÙˆÓ ÂÚÁ·ÛÈÒÓ Ô˘ ‰ÂÓ Á›ÓÔÓÙ·È ·Ô‰ÂÎÙ¤˜ÁÈ· ‰ËÌÔÛ›Â˘ÛË, ‰ÂÓ ÂÈÛÙÚ¤ÊÔÓÙ·È. MÔÚ› Ó· ÂÈÛÙÚ·-ÊÔ‡Ó, ÂÊfiÛÔÓ ˙ËÙËı› ÂÓÙfi˜ ÂÍ·Ì‹ÓÔ˘, Ù· Û¯‹Ì·Ù· Î·È ÔÈʈÙÔÁڷʛ˜ Ô˘ Ù· Û˘Óԉ‡ԢÓ.
OÈ ÂÚÁ·Û›Â˜ Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È ÚÔ˜ ÎÚ›ÛË ÁÈ· ‰ËÌÔ-Û›Â˘ÛË ÛÙÔ ÂÚÈÔ‰ÈÎfi “¶·È‰È·ÙÚÈ΋” ·ÔÛÙ¤ÏÏÔÓÙ·È ÂȘÙÚÈÏÔ‡Ó ÛÙË ‰È‡ı˘ÓÛË:
™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹, EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92, 115 28 Aı‹Ó·
¶ÚÈÓ ·ÔÛÙ›ÏÂÙ ÙËÓ ÂÚÁ·Û›· Û·˜, ‚‚·Èˆı›Ù fiÙÈÂÚÈÏ·Ì‚¿ÓÂÈ:
1. 3 ·ÓÙ›ÁÚ·Ê· ÙÔ˘ ÎÂÈ̤ÓÔ˘ Ù˘ ÂÚÁ·Û›·˜, Ù˘ˆÌ¤Ó·Û‡Ìʈӷ Ì ÙȘ Ô‰ËÁ›Â˜.
2. ¢ÈÛΤٷ ‹ CD Ì fiÏÔ ÙÔ ˘ÏÈÎfi Ù˘ ÂÚÁ·Û›·˜ (ΛÌÂÓÔ, ›Ó·Î˜, ÂÈÎfiÓ˜).
3. ∂ÈÛÙÔÏ‹ ˘Ô‚ÔÏ‹˜ Î·È ‰‹ÏˆÛË ÌË ÚÔÁÂÓ¤ÛÙÂÚ˘‰ËÌÔÛ›Â˘Û˘ Ù˘ ÂÚÁ·Û›·˜.
4. ™ÂÏ›‰· Ù›ÙÏÔ˘ (¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂÚÈÏ·Ì‚¿ÓÂÈ: ·.Ù›ÙÏÔ Î·È ‚Ú·¯‡ Ù›ÙÏÔ ÂÚÁ·Û›·˜ ‚.fiÓÔÌ· Î·È ÂÒÓ˘ÌÔ Û˘ÁÁڷʤˆÓ (ÔÏÔÁڿʈ˜) Á. ÂÈÛÙËÌÔÓÈÎfi/¿ ΤÓÙÚÔ/· fiÔ˘ ¤ÁÈÓÂ Ë ÂÚÁ·Û›· ‰.fiÓÔÌ·, ‰È‡ı˘ÓÛË Î·È ÙËϤʈÓÔ Û˘ÁÁڷʤ· ÁÈ·
·ÏÏËÏÔÁÚ·Ê›·. 5. ¶ÂÚ›ÏË„Ë ÂÏÏËÓÈ΋-·ÁÁÏÈ΋, ‰ÔÌË̤ÓË ÛÙȘ ÂÍ‹˜
·Ú·ÁÚ¿ÊÔ˘˜: ÂÈÛ·ÁˆÁ‹, ̤ıÔ‰ÔÈ, ·ÔÙÂϤÛÌ·Ù·Î·È Û˘ÌÂÚ¿ÛÌ·Ù·, ηıÒ˜ Î·È Ï¤ÍÂȘ ÎÏÂȉȿ (‰ÈÏfi‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).
6. ∫·Ù¿ÏÔÁÔ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈ-ÛÙ‹ ÛÂÏ›‰·).
7. ∫›ÌÂÓÔ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).8. ∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹-
ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢).9. µÈ‚ÏÈÔÁÚ·Ê›· (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·).
10. ¶›Ó·Î˜ (Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈ-ÏÔ‡Ó.
11. ∂ÈÎfiÓ˜, Ì ‚¤ÏÔ˜ ÛÙÔ ›Ûˆ ̤ÚÔ˜ ÙÔ ÔÔ›Ô Ó· ‰Â›-¯ÓÂÈ ÚÔ˜ Ù· ¿ӈ, ·ÚÈıÌË̤Ó˜, ÂȘ ÙÚÈÏÔ‡Ó.
12. ∆›ÙÏÔ˘˜ ÂÈÎfiÓˆÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - Ô Î·ı¤Ó·˜ Û¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó.
xvii ™À¡∆√ª√°ƒ∞ºπ∂™ ABBREVIATIONS
Ao
angstrom angstromcal ıÂÚÌ›‰· caloriecm ÂηÙÔÛÙfi centimetercm2 ÙÂÙÚ·ÁˆÓÈÎfi ÂηÙÔÛÙfi square centimetercm3 ΢‚ÈÎfi ÂηÙÔÛÙfi cubic centimeteroC ‚·ıÌfi˜ ∫ÂÏÛ›Ô˘ degree Celsiusg ÁÚ·ÌÌ¿ÚÈÔ gramh ÒÚ· hourIU ‰ÈÂıÓ‹˜ ÌÔÓ¿‰· international unitkg ¯ÈÏÈfiÁÚ·ÌÌÔ kilograml Ï›ÙÚÔ literm ̤ÙÚÔ metermg ¯ÈÏÈfiÁÚ·ÌÌÔ milligrammin ÏÂÙfi minutemm ¯ÈÏÈÔÛÙfiÌÂÙÚÔ millimetermol ÁÚ·ÌÌÔÌfiÚÈÔ molen ·ÚÈıÌfi˜ numberNS ‹ ª™ ÌË ÛËÌ·ÓÙÈÎfi not significantosm ÔÛÌÒÏÈÔ osmole
p Èı·ÓfiÙËÙ· probabilitySD ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË standard deviationSE ÛÙ·ıÂÚfi ÛÊ¿ÏÌ· standard errorsec ‰Â˘ÙÂÚfiÏÂÙÔ secondU ÌÔÓ¿‰· unit
™˘Ó‰˘·˙fiÌÂÓ· ÚÔı¤Ì·Ù· Combining prefixes
tera- (1012) Tgiga- (109) Gmega- (106) Mkilo- (103) khector- (102) hdeca- (101) dadeci- (10-1) dcenti- (10-2) cmilli- (10-3) mmicro- (10-6) Ìnano- (10-9) npico- (10-12) pfemto- (10-15) fatto- (10-18) a
xixINSTRUCTIONS TO AUTHORS
A. General InformationThe Greek Paediatric Society is the owner of “Paedia-
triki”, its official scientific journal, which is distributed to
its members. Its objectives are the publication of paediatric
scientific work and the continuing education of paediatri-
cians. For this purpose, it publishes a variety of articles, and
in particular:
1. Editorials (upon invitation by the Editorial Board).
2. Review articles.
3. Award-winning articles.
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6. Round tables.
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8. Issues of healthcare management and education.
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The Editorial Board reserves the right to publish articles
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The entire paper (including figure legends and tables)
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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., privatepaediatrician) and home address;
ñ the complete address, e-mail and telephone number ofthe author to whom correspondence should be addressed.
Abstracts
The abstract should summarize the objectives, method-ology, main results and conclusions of the study.
ñ It should contain at least 200 words, and not exceed 250words.
ñ It should consist of the following paragraphs: back-ground, methods, results and conclusions.
The English abstract should cite at the beginning the ti-tle of the paper and the authors’ names in English. The con-tent of the text should consist of the following paragraphs:background, methods, results and conclusions. The abstractin English should not differ in content from the correspond-ing Greek abstract.
Beneath the Greek and English abstracts, three to fivekey words in the respective language should be supplied, tobe used in the thematic index.
Text
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Case reports comprise a short introduction, case de-scription and brief discussion, with emphasis on differentialdiagnosis.
The structure of all other articles is free, according to thejudgment of the authors.
Thanks or acknowledgements (reference to grants,sponsorships or other sources of financial support) shouldbe quoted at the end of the text, before references.
xx
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The reference section contains all references numberedin the order in which they appear in the text. In the text, ref-erences are to be indicated by Arabic numerals in parenthe-ses. References should be no more than:
ñ 70 in review articles; ñ 30 in original articles; ñ 12 in current issues and case reports; ñ 5 in brief reports and letters.
In listing references follow the recently modified stan-dards of the International Committee of Medical Journal Edi-tors/Uniform Requirements for Manuscripts Submitted toBiomedical Journals, (http://www.icmje.org and http://www.icmje.org/icmje.pdf). Abbreviated names of journalsshould conform to the Cumulated Index Medicus [List ofJournals Indexed in Index Medicus (http://www.nlm.nih.gov/bsd/uniform_requirements.html)].
Examples of reference style
I. JOURNALS
All authors are cited if they are six or less; if they are 7or more, the first six are cited, followed by “et al”.
Regular edition:
Proesmans W. Bartter syndrome and its neonatal vari-ant. Eur J Pediatr 1997;156:669-679.
Supplement issue:
Flyvbjerg A. Role of growth hormone, insulin-likegrowth factors (IGFs) and IGF-binding proteins in the renalcomplications of diabetes. Kidney Int 1997;52 (60Suppl):S12-S19.
No author:
National Institutes of Health Consensus DevelopmentConference. Neurofibromatosis conference statement. ArchNeurol 1988;45:575-578.
Article type specification:
Schreiner GF, Lange L. Ethanol modulation ofmacrophage influx in glomerulonephritis [Abstract]. J AmSoc Nephrol 1991;2:562.
Should antileukotriene therapies be used instead of in-haled corticosteroids in asthma? [Editorial]. Am J RespirCrit Care Med 1998;158:1697-1701.
Laux-End R, Inaebnit D, Gerber HA, Bianchetti MG.Vasculitis associated with levamisole and circulating au-toantibodies [Letter]. Arch Dis Child 1996;75:355-356.
II. BOOKS
Chapter in book:
Clark AG, Barratt TM. Steroid-responsive nephrotic
syndrome. In: Barratt TM, Arner ED, Harmon WE, editors.
Pediatric Nephrology. 4th ed. Baltimore: Lippincott Wil-
liam Wilkins; 1999. p. 742.
Book or monograph:
Gorlin RJ, Cohen MM, Levin LS. Syndromes of the
head and neck. 3rd ed. New York: Oxford University Press;
1990.
Publication in a volume of proceedings:
Bauer AW. The two definitions of bacterial resistance.
In: Smith AJ, Rogers CA, eds. Proceedings of the Third In-
ternational Congress of Chemotherapy; 1962 May 29-31;
New York: International Society of Chemotherapy; 1963. p.
484-500.
Doctoral dissertation:
Kaplan SJ. Post hospital home health care: the elderly’s
access and utilization [dissertation]. St. Louis (Mo): Wash-
ington Univ.; 1995.
πππ. CD-ROM
Anderson SC, Poulsen KB. Anderson’s electronic atlas
of hematology [CD-ROM]. Philadelphia: Lippincott Wil-
liams & Wilkins; 2002.
IV. ON THE INTERNET
Article in journal
Abood S. Quality improvement initiative in nursing
homes: the ANA acts in an advisory role. Am J Nurs [Inter-
net]. 2002 Jun: Webpage: http://www.nursingworld.org/
AJN/2002/june/Wawatch.htm
Monograph
Foley KM, Gelband H, editors. Improving palliative
care for cancer [Monograph, Internet]. Washington: Na-
tional Academy Press; 2001. Webpage:
http://www.nap.edu/books/0309074029/html
Websites
Cancer-Pain.org [Webpage, Internet]. New York: Asso-
ciation of Cancer Online Resources, Inc.; 2002:
http://www.cancer-pain.org/
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xxi
409∞¡∞™∫√¶∏™∏ REVIEW ARTICLE
√ ÚfiÏÔ˜ Ù˘ ·fiÙˆÛ˘ ÛÙË ‰È·‚ËÙÈ΋
ÂÌ‚Ú˘Ô¿ıÂÈ·
µ. ¢ÚfiÛÔ˘ - ∞Á·Î›‰Ô˘1, ∂. ™ˆÙËÚ›‰Ô˘2
¶ÂÚ›ÏË„Ë: ¶·Ú¿ ÙȘ ÛËÌ·ÓÙÈΤ˜ ÂÍÂÏ›ÍÂȘ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË, ÔÈ Û˘Á-ÁÂÓ›˜ ‰È·Ì·Úٛ˜ ·Ú·Ì¤ÓÔ˘Ó ÌÈ· ÛËÌ·ÓÙÈ΋ ·ÈÙ›· ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÛÈÌfiÙËÙ·˜ ÙˆÓ ÓÂÔ-ÁÓÒÓ ‰È·‚ËÙÈÎÒÓ ÌËÙ¤ÚˆÓ. Œ¯ÂÈ ÂÓÔ¯ÔÔÈËı› ¤Ó· Ï‹ıÔ˜ ÌÂÙ·‚ÔÏÈÎÒÓ Î·È ¿ÏÏˆÓ Ì˯·ÓÈÛÌÒÓÔ˘ Û˘ÌÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙËÓ ˘ÂÚ- ‹ ˘Ô-ÁÏ˘Î·ÈÌ›· Ù˘ ÌËÙ¤Ú·˜, ÙËÓ ˘ÂÚÎÂÙÔÓ·ÈÌ›·, ÙËÓ ·ÓÂ-¿ÚÎÂÈ· ıÚÂÙÈÎÒÓ Ô˘ÛÈÒÓ, fiˆ˜ ÙÔ˘ ·Ú·¯È‰ÔÓÈÎÔ‡ ÔͤԘ Î·È Ù˘ Ì˘Ô˚ÓÔÛÈÙfiÏ˘, ÙËÓ ·˘ÍË̤-ÓË ·Ú·ÁˆÁ‹ ÂÏ¢ı¤ÚˆÓ ÚÈ˙ÒÓ Ô͢ÁfiÓÔ˘ Î.¿. ªÈ· ηÈÓÔ‡ÚÁÈ· ˘fiıÂÛË Ô˘ ·Ó·‰‡ÂÙ·È Û¯ÂÙÈοÌ ÙËÓ ·ıÔÁ¤ÓÂÈ· ÙˆÓ Û˘ÁÁÂÓÒÓ ‰È·Ì·ÚÙÈÒÓ Û ‰È·‚ËÙÈΤ˜ ΢‹ÛÂȘ Â›Ó·È Ë Â·ÁˆÁ‹ Ù˘ ‰È·‰È-ηۛ·˜ Ù˘ ·fiÙˆÛ˘ ÏfiÁˆ Ù˘ ˘ÂÚÁÏ˘Î·ÈÌ›·˜. ™ÙÔ ÚÔÂÌÊ˘Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ ·Ó¿Ù˘Í˘ ÙÔ˘ÂÌ‚Ú‡Ô˘, Ë ˘ÂÚÁÏ˘Î·ÈÌ›· Ô‰ËÁ› Û ·‡ÍËÛË Ù˘ ¤ÎÊÚ·Û˘ ÙÔ˘ ÁÔÓȉ›Ô˘ p53, ÙÔ ÔÔ›Ô Â¿ÁÂÈÙËÓ ·˘ÍË̤ÓË ¤ÎÊÚ·ÛË Ù˘ ÚÔ·ÔÙˆÙÈ΋˜ ÚˆÙ½Ó˘ µ∞Ã. ™ÙÔ ÌÂÙÂÌÊ˘Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ ·Ó¿-Ù˘Í˘ ÙÔ˘ ÂÌ‚Ú‡Ô˘, Ë ˘ÂÚÁÏ˘Î·ÈÌ›· ÂÏ·ÙÙÒÓÂÈ ÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Pax3, ÙÔ ÔÔ›ÔÔ‰ËÁ› ÛÙËÓ ·˘ÍË̤ÓË ¤ÎÊÚ·ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Cdc46 ÛÙÔ ÚÒÈÌÔ ÛÙ¿‰ÈÔ Î·È ÛÙË ÌÂȈ̤ÓË ¤Î-ÊÚ·ÛË ÙÔ˘ Dep1 ÛÙÔ fi„ÈÌÔ ÛÙ¿‰ÈÔ ·Ó¿Ù˘Í˘ ÙÔ˘ ÂÌ‚Ú‡Ô˘, ÌÂ Û˘Ó¤ÂÈ· ÙËÓ Â·ÁˆÁ‹ Ù˘ ‰È·-‰Èηۛ·˜ Ù˘ ·fiÙˆÛ˘.
§¤ÍÂȘ ÎÏÂȉȿ: ¢È·‚ËÙÈ΋ ÂÌ‚Ú˘Ô¿ıÂÈ·, Û˘ÁÁÂÓ›˜ ‰È·Ì·Úٛ˜, ˘ÂÚÁÏ˘Î·ÈÌ›·, ·fiÙˆÛË.
The role of apoptosis in diabetic embryopathy
V. Drossou - Agakidou1, E. Sotiridou2
Abstract: Despite significant improvements in the management of diabetes mellitus, congenitalmalformations remain a major cause of morbidity and mortality among the infants of diabetic mothers.A variety of metabolic disturbances, including maternal hyper- and hypoglycaemia andhyperketonaemia, nutritional deficiencies such as arachidonic acid and myo-inositol deficiencies, andincreased oxygen free radical production, have been implicated. One emerging hypothesis for theaetiology of diabetes-associated malformations is that of hyperglycaemia induced apoptosis. During thepre-implantation stage of development, maternal hyperglycaemia triggers the expression of theproapoptotic protein BAX, which leads to activation of caspases, DNA fragmentation and morphologicalchanges consistent with apoptosis. During post-implantation embryogenesis maternal hyperglycaemiaresults in decreased Pax3 expression, leading to increased Cdc46 expression (early event) and decreasedDep1 expression (late event), with resultant increased apoptosis in the developing embryos.
Key words: Diabetic embryopathy, congenital malformations, hyperglycemia, apoptosis.
¶·È‰È·ÙÚÈ΋ 2006;69:409-416
1. ∞’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈÎ‹Î·È ∂ÓÙ·ÙÈ΋ ¡ÔÛËÏ›·¡ÂÔÁÓÒÓ ∞.¶.£.,“πÔÎÚ¿ÙÂÈÔ” °.¶.¡.£ÂÛÛ·ÏÔӛ΢
2. ªÂÙ·Ù˘¯È·Î‹ ÊÔÈÙ‹ÙÚÈ·(¡.¶.ª.™.) Ù˘ π·ÙÚÈ΋˜™¯ÔÏ‹˜ ∞.¶.£.,“πÔÎÚ¿ÙÂÈÔ” °.¶.¡.£ÂÛÛ·ÏÔӛ΢
AÏÏËÏÔÁÚ·Ê›·:
µ. ¢ÚfiÛÔ˘-∞Á·Î›‰Ô˘°ÏËÓÔ‡ 28, ∆∫ 543 52,£ÂÛÛ·ÏÔÓ›ÎË∂-mail: [email protected]
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 31-05-2005Date of approval: 07-04-2006
1. 1st Department ofNeonatology & NeonatalIntensive Care Unit,Aristotle University ofThessaloniki, IppokratioHospital, Thessaloniki
2. Postgraduate student inthe Faculty of Medicine,Aristotle University ofThessaloniki, IppokratioHospital, Thessaloniki
Correspondence:
V. Drossou-Agakidou28 Glinou St, 543 52 Thessaloniki, Greece∂-mail: [email protected]
Date of submission: 31-05-2005 Date of approval: 07-04-2006
™˘ÓÙÔÌÔÁڷʛ˜
BAX: BCL2-associated X protein
BCL2: B-cell leukemia/lymphoma 2
Cdc46: Cell division cycle 46
Dep1: Diabetic embryopathy 1
GLUT 1: Glucose transporter type 1
Hif-1a: Hypoxia-inducible factor 1 alpha
Pax3: Paired box gene 3
TNF: Tumor necrosis factor
Waf-1: Waf-1 (a cyclin-dependent kinase
inhibitor)
∂ÈÛ·ÁˆÁ‹
¶·Ú¿ ÙȘ ÛËÌ·ÓÙÈΤ˜ ÂÍÂÏ›ÍÂȘ ÛÙËÓ ·ÓÙÈÌÂ-
ÙÒÈÛË ÙÔ˘ ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË, ÔÈ Û˘ÁÁÂÓ›˜‰È·Ì·Úٛ˜ ·Ú·Ì¤ÓÔ˘Ó ÌÈ· ÛËÌ·ÓÙÈ΋ ·ÈÙ›·
ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÛÈÌfiÙËÙ·˜ ÙˆÓ ÓÂÔÁÓÒÓ
‰È·‚ËÙÈÎÒÓ ÌËÙ¤ÚˆÓ. ∏ Û˘¯ÓfiÙËÙ· ÌÂÈ˙fiÓˆÓ
‰È·Ì·ÚÙÈÒÓ Â›Ó·È 6-9%, ‰ËÏ·‰‹ 3-4 ÊÔÚ¤˜
˘„ËÏfiÙÂÚË ·’ fi,ÙÈ ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi, ηÈ¢ı‡ÓÔÓÙ·È ÁÈ· ÙÔ 33-66% ÙˆÓ ÂÚÈÁÂÓÓËÙÈÎÒÓ
410 µ. ¢ÚfiÛÔ˘-∞Á·Î›‰Ô˘, ∂. ™ˆÙËÚ›‰Ô˘
ı·Ó¿ÙˆÓ ÙˆÓ ÓÂÔÁÓÒÓ ‰È·‚ËÙÈÎÒÓ ÌËÙ¤ÚˆÓ
(1). √ ·ÚÈÔ˜ ÏfiÁÔ˜ ÁÈ· ÙËÓ ·‰˘Ó·Ì›· ÈηÓÔ-
ÔÈËÙÈ΋˜ Ì›ˆÛ˘ Ù˘ Û˘¯ÓfiÙËÙ·˜ ÙˆÓ Û˘ÁÁÂ-
ÓÒÓ ‰˘ÛÏ·ÛÈÒÓ Â›Ó·È Ë Ôχ ÚÒÈÌË ·Ó¿Ù˘-
Í‹ ÙÔ˘˜, ÙËÓ 1Ë-6Ë Â‚‰ÔÌ¿‰· Ù˘ ·ËÛ˘. ∆Ô
Ê¿ÛÌ· ÙˆÓ ‰È·Ì·ÚÙÈÒÓ ÛÙË ‰È·‚ËÙÈ΋ ÂÌ‚Ú˘Ô-
¿ıÂÈ· Â›Ó·È Â˘Ú‡ Î·È ·ÊÔÚ¿ ΢ڛˆ˜ ÙÔ Î·Ú-
‰È·ÁÁÂÈ·Îfi, ÙÔ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· ηÈ
ÙÔ Ì˘ÔÛÎÂÏÂÙÈÎfi Û‡ÛÙËÌ·. ∏ ‰È·Ì·ÚÙ›· Ë
ÔÔ›· ıˆÚÂ›Ù·È fiÙÈ Â›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈ΋ Ù˘
‰È·‚ËÙÈ΋˜ ÂÌ‚Ú˘Ô¿ıÂÈ·˜ Â›Ó·È ÙÔ Û‡Ó‰ÚÔÌÔ
Ô˘Ú·›·˜ ·ÏÈÓ‰ÚfiÌËÛ˘ ‹ ·ÁÂÓÂÛ›·˜ ÙÔ˘ ÈÂÚÔ‡
ÔÛÙÔ‡, 200-400 ÊÔÚ¤˜ ÈÔ Û˘¯Ófi ÛÙ· ÓÂÔÁÓ¿
‰È·‚ËÙÈÎÒÓ ÌËÙ¤ÚˆÓ Û˘ÁÎÚÈÙÈο Ì ÙÔÓ ÁÂÓÈÎfi
ÏËı˘ÛÌfi (1). ∏ ·ıÔÁ¤ÓÂÈ· Ù˘ ‰È·‚ËÙÈ΋˜
ÂÌ‚Ú˘Ô¿ıÂÈ·˜ ·ÔÙ¤ÏÂÛ ·ÓÙÈΛÌÂÓÔ Ï‹-
ıÔ˘˜ ÂÈÚ·Ì·ÙÈÎÒÓ Î·È ÎÏÈÓÈÎÒÓ ÌÂÏÂÙÒÓ Î·Ù¿
Ù· ÙÂÏÂ˘Ù·›· 30 ¯ÚfiÓÈ·. Œ¯ÂÈ ÂÓÔ¯ÔÔÈËı›
Ï‹ıÔ˜ ÌÂÙ·‚ÔÏÈÎÒÓ Î·È ¿ÏÏˆÓ Ì˯·ÓÈÛÌÒÓ,
ÔÈ ÔÔ›ÔÈ Û˘ÌÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙËÓ ˘ÂÚ- ‹ ˘Ô-
ÁÏ˘Î·ÈÌ›· Ù˘ ÌËÙ¤Ú·˜, ÙËÓ ˘ÂÚÎÂÙÔÓ·ÈÌ›·,
ÙËÓ ·Ó¿ÚÎÂÈ· ıÚÂÙÈÎÒÓ Ô˘ÛÈÒÓ, fiˆ˜ ÙÔ˘
·Ú·¯È‰ÔÓÈÎÔ‡ ÔͤԘ Î·È Ù˘ Ì˘Ô˚ÓÔÛÈÙfiÏ˘,
ÙËÓ ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ÂÏ¢ı¤ÚˆÓ ÚÈ˙ÒÓ
Ô͢ÁfiÓÔ˘ Î.¿. (2). ¶·ÚfiÏ· ·˘Ù¿, Ô ·ÎÚÈ‚‹˜ ÌË-
¯·ÓÈÛÌfi˜ ÙˆÓ Û˘ÁÁÂÓÒÓ ‰˘ÛÏ·ÛÈÒÓ ÛÙË ‰È·-
‚ËÙÈ΋ ·ËÛË ‰ÂÓ Â›Ó·È ·ÎfiÌ· ÁÓˆÛÙfi˜.
ªÈ· ηÈÓÔ‡ÚÁÈ· ˘fiıÂÛË Ô˘ ·Ó·‰‡ÂÙ·È
Û¯ÂÙÈο Ì ÙËÓ ·ıÔÁ¤ÓÂÈ· ÙˆÓ Û˘ÁÁÂÓÒÓ ‰È·-
Ì·ÚÙÈÒÓ Û ‰È·‚ËÙÈΤ˜ ΢‹ÛÂȘ Â›Ó·È Ë Â·Áˆ-
Á‹ Ù˘ ‰È·‰Èηۛ·˜ Ù˘ ·fiÙˆÛ˘ ÏfiÁˆ Ù˘
˘ÂÚÁÏ˘Î·ÈÌ›·˜ (3,4). ∏ ·fiÙˆÛË Â›Ó·È ÌÈ·
ÚԉȷÁÂÁÚ·Ì̤ÓË Î·È ÁÂÓÂÙÈο ÂÏÂÁ¯fiÌÂÓË
·¿ÓÙËÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ Û ÂȉÈο ÂÚÈ‚·ÏÏÔ-
ÓÙÈο Î·È ·Ó·Ù˘Íȷο ÂÚÂı›ÛÌ·Ù·, Ë ÔÔ›·
¿ÁÂÙ·È ·fi ÔÏÏÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ ›ӷÈ
Ù· ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ‹, Ë ˘ÂÚÈ҉˘ ·ÎÙÈÓÔ‚Ô-
Ï›·, Ë ·Ô˘Û›· ÔÚÈÛÌ¤ÓˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·Áfi-
ÓÙˆÓ, Ë ¤ÎÊÚ·ÛË ÙˆÓ ÁÔÓȉ›ˆÓ c-myc Î·È p53, Ë
ÈÔÁÂÓ‹˜ ÌfiÏ˘ÓÛË Î.¿. ∏ ·fiÙˆÛË Â›Ó·È ÌÈ·
Û·ÊÒ˜ ÚÔÁÚ·ÌÌ·ÙÈ˙fiÌÂÓË ‰È·‰Èηۛ·, Ë
ÔÔ›· ·ÔÛÎÔ› ÛÙÔÓ ı¿Ó·ÙÔ Î·È ÛÙËÓ ·Ô-
Ì¿ÎÚ˘ÓÛË Î˘ÙÙ¿ÚˆÓ, Ì ÙËÓ ÂÏ¿¯ÈÛÙË ‰˘Ó·Ù‹
‚Ï¿‚Ë ÛÙÔ˘˜ ÂÚÈ‚¿ÏÏÔÓÙ˜ ÈÛÙÔ‡˜, ÁÂÁÔÓfi˜
Ô˘ ˘ÔÁÚ·ÌÌ›˙ÂÈ ÙË ÛËÌ·Û›· Ù˘ ÛÙË Ê˘ÛÈÔ-
ÏÔÁÈ΋ ·Ó¿Ù˘ÍË ÙˆÓ ÈÛÙÒÓ. ™ÙËÓ ·fiÙˆÛË,
ÙÔ Î‡ÙÙ·ÚÔ Û˘ÌÌÂÙ¤¯ÂÈ ÂÓÂÚÁ¿ ÛÙÔ Û˘ÁÎÂÎÚÈ-
̤ÓÔ ÂÓ‰ÔÁÂÓ¤˜ ÚfiÁÚ·ÌÌ· ·˘ÙÔηٷÛÙÚÔÊ‹˜
ÙÔ˘, ·Ú¿ÁÔÓÙ·˜ Ù· ··Ú·›ÙËÙ· Ì·ÎÚÔÌfiÚÈ·
Î·È ÙËÓ ··ÈÙÔ‡ÌÂÓË ÂÓ¤ÚÁÂÈ·. °È· ÙÔÓ ÏfiÁÔ
·˘Ùfi, Ë ·fiÙˆÛË ÔÓÔÌ¿˙ÂÙ·È Î·È “ÂÓÂÚÁfi˜
΢ÙÙ·ÚÈÎfi˜ ı¿Ó·ÙÔ˜” (5).
À¿Ú¯Ô˘Ó ÔÏÏ¿ ÂÈÚ·Ì·ÙÈο ‰Â‰Ô̤ӷ
Ô˘ ˘Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Ë ·fiÙˆÛË ·›˙ÂÈ ÛË-
Ì·ÓÙÈÎfi ÚfiÏÔ ÛÙË ‰È·‚ËÙÈ΋ ÂÌ‚Ú˘Ô¿ıÂÈ·,
ÙfiÛÔ Î·Ù¿ ÙÔ ÚÔÂÌÊ˘Ù¢ÙÈÎfi fiÛÔ Î·È Î·Ù¿ ÙÔ
ÌÂÙÂÌÊ˘Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ Ù˘ ÂÌ‚Ú˘˚΋˜ ·Ó¿-
Ù˘Í˘. √È Ì˯·ÓÈÛÌÔ› Ì ÙÔ˘˜ ÔÔ›Ô˘˜ Ë
˘ÂÚÁÏ˘Î·ÈÌ›· Ô‰ËÁ› Û ·‡ÍËÛË Ù˘ ·fiÙˆ-
Û˘ ‰È·Ê¤ÚÔ˘Ó ÌÂٷ͇ ÚÔÂÌÊ˘Ù¢ÙÈÎÔ‡ ηÈ
ÌÂÙÂÌÊ˘Ù¢ÙÈÎÔ‡ ÛÙ·‰›Ô˘, ·ÏÏ¿ ‰ÂÓ ¤¯Ô˘Ó ‰È-
¢ÎÚÈÓÈÛÙ› Ï‹Úˆ˜.
·) ¶ÚÔÂÌÊ˘Ù¢ÙÈ΋ ‰È·‚ËÙÈ΋
ÂÌ‚Ú˘Ô¿ıÂÈ·
∆Ô ÚÔÂÌÊ˘Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ ÂÎÙ›ÓÂÙ·È ·fi
ÙË Û‡ÏÏË„Ë, ÙË ‰ËÌÈÔ˘ÚÁ›· ‚Ï·ÛÙÔÌÂÚȉ›ˆÓ
Î·È ‚Ï·ÛÙÔ·ÛÙ˘ ̤¯ÚÈ ÙËÓ ÂÌʇÙ¢ÛË. ªÂ-
ϤÙ˜ Û ÂÈÚ·Ì·ÙÈο ÌÔÓ٤Ϸ ˙ÒˆÓ ÛÙÔ ÚÔ-
ÂÌÊ˘Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ Ù˘ ·Ó¿Ù˘Í˘ ¤¯Ô˘Ó ‰Â›-
ÍÂÈ fiÙÈ Ë ÌËÙÚÈ΋ ˘ÂÚÁÏ˘Î·ÈÌ›· Ô‰ËÁ› ÛÙËÓ
ηÙÈÔ‡Û· Ú‡ıÌÈÛË ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ÌÂÙ·ÊÔÚ¤ˆÓ
ÁÏ˘Îfi˙˘ GLUT1-2 Î·È -3 ÛÙÔ ÛÙ¿‰ÈÔ ·Ó¿Ù˘-
͢ Ù˘ ‚Ï·ÛÙÔ·ÛÙ˘ (6). ™Â in vitro ÌÂϤÙ˜,
ηÏÏȤÚÁÂÈ· ÂÌ‚Ú‡ˆÓ ÛÙÔ ÛÙ¿‰ÈÔ ÙˆÓ ‰‡Ô-΢Ù-
Ù¿ÚˆÓ Û ˘„ËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ÁÏ˘Îfi˙˘ (30
‹ 52 mª) ÁÈ· 72 ÒÚ˜, ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ
ÂÏ¿ÙÙˆÛË Ù˘ ¤ÎÊÚ·Û˘ ÙÔ˘ mRNA Î·È Ù˘
Û‡ÓıÂÛ˘ ÙˆÓ ÚˆÙÂ˚ÓÒÓ. ÕÏϘ ÌÂϤÙ˜ ¤‰ÂÈ-
Í·Ó fiÙÈ Ë Ì›ˆÛË ÙˆÓ ÌÂÙ·ÊÔÚ¤ˆÓ Ô‰‹ÁËÛ ÛÂ
ÌÈ· ÛËÌ·ÓÙÈ΋ ÙÒÛË ÙˆÓ ÂÏ¢ı¤ÚˆÓ ÂȤ-
‰ˆÓ ÁÏ˘Îfi˙˘ Û ‚Ï·ÛÙÔ·ÛÙÂȘ ÔÓÙÈÎÒÓ, ÔÈ
ÔÔ›ÔÈ Â›¯·Ó ·Ó·Ù‡ÍÂÈ ˘ÂÚÁÏ˘Î·ÈÌ›· ›Ù ÌÂ
¤Á¯˘ÛË ÛÙÚÂÙÔ˙ÔÙÔΛÓ˘ ›Ù Ì ηÏÏȤÚÁÂÈ·
ÙˆÓ ÂÌ‚Ú‡ˆÓ ÛÙÔ ÛÙ¿‰ÈÔ ‰‡Ô-΢ÙÙ¿ÚˆÓ ÛÂ
˘„ËÏ¿ ›‰· ÁÏ˘Îfi˙˘ (6,7). ∫·È ¿ÏϘ ÌÂ-
ϤÙ˜ ¤¯Ô˘Ó Û˘Ó‰¤ÛÂÈ ÙË Ì›ˆÛË Ù˘ ÌÂÙ·ÊÔ-
Ú¿˜ Ù˘ ÁÏ˘Îfi˙˘ Ì ÚfiˆÚÔ ÚÔÁÚ·ÌÌ·ÙÈ-
Ṳ̂ÓÔ Î˘ÙÙ·ÚÈÎfi ı¿Ó·ÙÔ (8-10).
∞fi Ù· Â˘Ú‹Ì·Ù· ·˘Ù¿ Û˘ÌÂÚ·›ÓÂÙ·È fiÙÈ Ë
˘ÂÚÁÏ˘Î·ÈÌ›·, ÌÂÈÒÓÔÓÙ·˜ ÙÔ˘˜ ÌÂÙ·ÊÔÚ›˜
ÁÏ˘Îfi˙˘ ÛÙË ‚Ï·ÛÙÔ·ÛÙË, ÏÂÈÙÔ˘ÚÁ› Û·Ó
¤Ó· Û‹Ì· ı·Ó¿ÙÔ˘, ÙÔ ÔÔ›Ô ‰ÈÂÁ›ÚÂÈ ÙËÓ ·Ô-
ÙˆÙÈ΋ Ô‰fi Ô˘ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ÚˆÙ½ÓË
BAX (6,11). ¶Ú¿ÁÌ·ÙÈ, ‚Ï·ÛÙÔ·ÛÙÂȘ Ô˘
‚Ú¤ıËÎ·Ó ÛÂ Û˘Óı‹Î˜ ·˘ÍËÌ¤ÓˆÓ ÂȤ‰ˆÓ
ÁÏ˘Îfi˙˘, in vivo Î·È in vitro, ·ÚÔ˘Û›·Û·Ó ·‡-
ÍËÛË ÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Bax Û ۇ-
ÁÎÚÈÛË Ì ÔÌ¿‰· ÂϤÁ¯Ô˘ (3). ∏ BAX ›ӷÈ
ÚÔ·ÔÙˆÙÈÎfi ̤ÏÔ˜ Ù˘ BCL2 ÔÈÎÔÁ¤ÓÂÈ·˜
ÙˆÓ ÚˆÙÂ˚ÓÒÓ. ∂¿ÁÂÈ ÙËÓ ·fiÙˆÛË Î·È ÛÂ
‰È¿Ï˘Ì· Û¯ËÌ·Ù›˙ÂÈ ¤Ó· ÂÙÂÚÔ‰ÈÌÂÚ¤˜ Û‡ÌÏÔ-
ÎÔ Ì ÙËÓ BCL2 ·Ó·ÛÙ¤ÏÏÔÓÙ·˜ Ì ÙÔÓ ÙÚfiÔ
·˘Ùfi ÙËÓ ·ÓÙÈ·ÔÙˆÙÈ΋ Ù˘ ‰Ú¿ÛË (12-14).
∏ BAX Û˘Ó‰¤ÂÈ ÙË Ú‡ıÌÈÛË Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜
ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ Î·È Ù˘ ·fiÙˆÛ˘ Ì ÙËÓ
¶·È‰È·ÙÚÈ΋ 2006;69:409-416
411AfiÙˆÛË ÛÙË ‰È·‚ËÙÈ΋ ÂÌ‚Ú˘Ô¿ıÂÈ·
ÂÈÙ‹ÚËÛË Ù˘ ·ÎÂÚ·ÈfiÙËÙ·˜ ÙÔ˘ DNA ·fi ÙÔ
p53 ‹/Î·È ·fi ¿ÏÏÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ì ‰Ú¿ÛË
·ÚfiÌÔÈ· ÙÔ˘ p53. ∏ ‰È·‰Èηۛ· Ù˘ ·fiÙˆ-
Û˘ ··ÈÙ› ÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Bax.
∞˘Ùfi ÚÔ·ÙÂÈ ·fi ÌÂϤÙ˜ Û ¤Ì‚Ú˘· ÔÓÙÈ-
ÎÒÓ Ô˘ ‰ÂÓ Â›¯·Ó ÙÔ ÁÔÓ›‰ÈÔ Bax (Bax -/-), ÛÙ·
ÔÔ›· Ë ¤ÎıÂÛË Û ˘„ËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ÁÏ˘-
Îfi˙˘ ‰ÂÓ Â›¯Â η̛· ‰˘ÛÌÂÓ‹ ›‰Ú·ÛË (3).
™Â ÌÔÓÙ¤ÏÔ ÚÔÂÌÊ˘Ù¢ÙÈ΋˜ ÂÌ‚Ú˘Ô¿ıÂÈ-
·˜ ÔÓÙÈÎÒÓ, ¤¯ÂÈ ‚ÚÂı› Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙˆÓ
·ÔÙÂÏÂÛÌ¿ÙˆÓ Ù˘ ˘ÂÚÁÏ˘Î·ÈÌ›·˜ Î·È Ù˘ ¤Î-
ÊÚ·Û˘ ÙÔ˘ ÁÔÓȉ›Ô˘ p53 (15). ∆· ¤Ì‚Ú˘· Ù·
ÔÔ›· ÛÙÂÚÔ‡ÓÙ·Ó Ù˘ ¤ÎÊÚ·Û˘ ÙÔ˘ p53 ‹Ù·Ó
·ÓıÂÎÙÈο ÛÙËÓ Â›‰Ú·ÛË Ù˘ ˘ÂÚÁÏ˘Î·ÈÌ›·˜
in vivo Î·È in vitro. ∆Ô ›‰ÈÔ ·Ú·ÙËÚ‹ıËΠηÈ
ÛÙÔ˘˜ Bax -/- ÔÓÙÈÎÔ‡˜. ∆Ô ÁÔÓ›‰ÈÔ p53 Û˘ÌÌÂÙ¤-
¯ÂÈ Û ‰È¿ÊÔÚ· ΢ÙÙ·ÚÈο ÁÂÁÔÓfiÙ·, ÛÙ· ÔÔ›·
ÂÚÈÏ·Ì‚¿ÓÂÙ·È Ë ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ Ì˯·ÓÈ-
ÛÌÔ‡ ÂȉÈfiÚıˆÛ˘ ÙÔ˘ DNA Î·È Ë ·fiÙˆÛË
(16). ∏ Ê˘ÛÈÔÏÔÁÈ΋ ÔÁÎÔηٷÛÙ·ÏÙÈ΋ ‰Ú¿ÛË
ÙÔ˘ p53 ·ÛÎÂ›Ù·È Ì ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ Waf-
1 ÁÔÓȉ›Ô˘ (17), ÙÔ ÔÔ›Ô Ì ÙË ÛÂÈÚ¿ ÙÔ˘ ·Ó·-
ÛÙ¤ÏÏÂÈ ÙË ‰Ú¿ÛË ÂȉÈÎÒÓ ÎÈÓ·ÛÒÓ (΢ÎϛӘ 6
Î·È 8) ÛÙȘ G1 Î·È G2 Ê¿ÛÂȘ ÙÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ·-
ÎÏÔ˘, ÁÈ· Ó· Á›ÓÂÈ Ë ÂȉÈfiÚıˆÛË Ù˘ ‚Ï¿‚˘ ÙÔ˘
DNA (18). ∞Ó ·˘Ù‹ Â›Ó·È ·ÓÂÈÙ˘¯‹˜, ÙfiÙ ÙÔ
p53 ÍÂÎÈÓ¿ ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓÔ Î˘ÙÙ·ÚÈÎfi ı¿Ó·-
ÙÔ ÂÓÂÚÁÔÔÈÒÓÙ·˜ ÙÔÓ ˘Ô‰Ô¯¤· Fas Ô˘ ·Ô-
ÙÂÏ› ̤ÏÔ˜ Ù˘ ˘ÂÚÔÈÎÔÁ¤ÓÂÈ·˜ ÙˆÓ ˘Ô‰Ô¯¤-
ˆÓ ÙÔ˘ ·Ú¿ÁÔÓÙ· Ó¤ÎÚˆÛ˘ ÙˆÓ fiÁÎˆÓ (TNF
receptor superfamily, member 6) Î·È ÙÔ ÁÔÓ›‰ÈÔ
Bax, Ô‰ËÁÒÓÙ·˜ Ì ÙÔÓ ÙÚfiÔ ·˘Ùfi ÛÙËÓ ÂÓÂÚ-
ÁÔÔ›ËÛË Ù˘ ηۿÛ˘-3 (19). ŒÙÛÈ, ÙÔ ÁÔÓ›‰ÈÔ
p53 ·ÔÙÚ¤ÂÈ ÙËÓ ÂÚ·ÈÙ¤Úˆ ÂÈ‚›ˆÛË Î·È ·Ó·-
·Ú·ÁˆÁ‹ ÂÓfi˜ ΢ÙÙ¿ÚÔ˘ Ì ÌfiÓÈÌË ‚Ï¿‚Ë ÛÙÔ
ÁÔÓȉ›ˆÌ¿ ÙÔ˘. ∆Ô ÁÔÓ›‰ÈÔ p53 ¤¯ÂÈ ¿ÌÂÛË Â›-
‰Ú·ÛË ÛÙËÓ ·fiÙˆÛË, ÂÏ·ÙÙÒÓÔÓÙ·˜ ÙËÓ ¤Î-
ÊÚ·ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Bcl-2 Î·È ·˘Í¿ÓÔÓÙ·˜ ÙËÓ
¤ÎÊÚ·ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ Bax. ∂Âȉ‹ ÙÔ p53 ›ӷÈ
¤Ó·˜ ÌÂÙ·ÁÚ·ÊÈÎfi˜ Ú˘ıÌÈÛÙ‹˜ ÙÔ˘ Bax, Â›Ó·È È-
ı·Ófi fiÙÈ Ë ÌËÙÚÈ΋ ˘ÂÚÁÏ˘Î·ÈÌ›·, Ì ÙË Ì›ˆ-
ÛË ÙˆÓ ÂÓ‰Ô΢ÙÙ¿ÚÈˆÓ ÂȤ‰ˆÓ ÁÏ˘Îfi˙˘ ÛÙÔ
¤Ì‚Ú˘Ô, ÚÔηÏ› ¤ÎÊÚ·ÛË ÙÔ˘ Hif-1·, Ô˘
Ô‰ËÁ› Û ·˘ÍË̤ÓË ¤ÎÊÚ·ÛË ÙÔ˘ p53, ÙÔ ÔÔ›Ô
ÛÙË Û˘Ó¤¯ÂÈ· ¿ÁÂÈ ÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ Bax ηÈ,
ÙÂÏÈο, ÙË ‰È·‰Èηۛ· Ù˘ ·fiÙˆÛ˘ ̤۷ ÛÙË
‚Ï·ÛÙÔ·ÛÙË (11,20). ∏ ·ÔÙˆÙÈ΋ ‰Ú¿ÛË Ù˘
ÚˆÙ½Ó˘ BAX ÔÊ›ÏÂÙ·È Î˘Ú›ˆ˜ ÛÙËÓ ÂÓÂÚÁÔ-
Ô›ËÛË ÙˆÓ Î·Û·ÛÒÓ (11).
√È Î·Û¿Û˜ ·ÔÙÂÏÔ‡Ó ÌÈ· ÔÌ¿‰· ÚˆÙÂÔ-
Ï˘ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ Ô˘ ·›˙Ô˘Ó ÛËÌ·ÓÙÈÎfi ÚfiÏÔ
ÛÙËÓ ·ÔÙˆÙÈ΋ ‰ÈÂÚÁ·Û›· (21). √È Î·Û¿Û˜
‚Ú›ÛÎÔÓÙ·È Û ·‰Ú·Ó‹ ηٿÛÙ·ÛË, ̤¯ÚȘ fiÙÔ˘
οÔÈÔ Â·ÁˆÁÈÎfi Û‹Ì· ÂÓÂÚÁÔÔÈ‹ÛÂÈ Ì›· ·fi
·˘Ù¤˜. ∆fiÙ ·Ú¯›˙ÂÈ Ë ‰È·‰Ô¯È΋ ÂÓÂÚÁÔÔ›ËÛË
Î·È ¿ÏÏˆÓ Î·Û·ÛÒÓ, Ô˘ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô ΢-
Ú›ˆ˜ ‰‡Ô ÌÂÁ¿Ï˜ ηÙËÁÔڛ˜ ÚˆÙÂ˚ÓÒÓ: ·)
ÚˆÙ½Ó˜ Ô˘ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙË ‰ÔÌ‹ Î·È ÙËÓ
ÔÚÁ¿ÓˆÛË ÙÔ˘ ΢ÙÙ¿ÚÔ˘ (22) Î·È ‚) Î·Ù·Ï˘ÙÈ-
Τ˜ ÚˆÙ½Ó˜ Ô˘ ÂÌϤÎÔÓÙ·È ÛÙÔ˘˜ Ì˯·ÓÈ-
ÛÌÔ‡˜ ÔÌÔÈfiÛÙ·Û˘ ÙÔ˘ ΢ÙÙ¿ÚÔ˘, ÌÂٷ͇ ÙˆÓ
ÔÔ›ˆÓ Û˘ÌÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ì˯·ÓÈÛÌÔ› ÂÈ-
‰ÈfiÚıˆÛ˘ ÙÔ˘ DNA (23), ÂϤÁ¯Ô˘ ÙÔ˘ ΢ÙÙ·ÚÈ-
ÎÔ‡ ·ÎÏÔ˘ (24) Î·È ¿ÏÏÔÈ ÛËÌ·ÓÙÈÎÔ› ‚ÈÔ¯ËÌÈ-
ÎÔ› Ì˯·ÓÈÛÌÔ› (25). √È Î·Û¿Û˜ ÂÓÙÔ›˙ÔÓÙ·È
ÛÙÔ Î˘ÙÙ·ÚfiÏ·ÛÌ· Î·È ÛÙ· ÌÈÙÔ¯fiÓ‰ÚÈ· Ì ÙË
ÌÔÚÊ‹ ÂÓfi˜ ·ÓÂÓÂÚÁÔ‡ ÚÔÂÓ˙‡ÌÔ˘ (ÚÔ-η-
Û¿Û˜) Î·È ÂÓÂÚÁÔÔÈÔ‡ÓÙ·È Ì¤Ûˆ Ù˘ ÚˆÙÂ-
fiÏ˘Û‹˜ ÙÔ˘˜, Èı·ÓÒ˜ ·fi ¿ÏÏ· ̤ÏË Ù˘ “ÔÈ-
ÎÔÁ¤ÓÂÈ·˜” (26). ∫‡ÚÈÔ Ì¤ÏÔ˜ ÙˆÓ Î·Û·ÛÒÓ Â›-
Ó·È Ë Î·Û¿ÛË-3, Ë ÔÔ›· ·›˙ÂÈ ÛËÌ·ÓÙÈÎfi Úfi-
ÏÔ ÛÙÔÓ ·ÔÙˆÙÈÎfi ΢ÙÙ·ÚÈÎfi ı¿Ó·ÙÔ Î·Ù¿ ÙË
‰È¿ÚÎÂÈ· Ù˘ ·Ó¿Ù˘Í˘ (27-29).
™ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ Î·Û·ÛÒÓ ·›Ú-
ÓÔ˘Ó Ì¤ÚÔ˜ ÔÈ ˘Ô‰Ô¯Â›˜ Fas Î·È TNF (30). ∆Ô
Û‹Ì· ÁÈ· ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ Î·Û·ÛÒÓ
ÚÔ¤Ú¯ÂÙ·È ·fi ÙË Û‡Ó‰ÂÛË ÛÙÔ˘˜ ·Ú·¿Óˆ
˘Ô‰Ô¯Â›˜ Ù˘ ÚÔ-ηۿÛ˘-8, -10 ‹ -2, ÌÂ
·ÔÙ¤ÏÂÛÌ· ÙË ‰ËÌÈÔ˘ÚÁ›· ÂÓfi˜ Û˘ÌϤÁÌ·-
ÙÔ˜, ÙÔ ÔÔ›Ô Ô‰ËÁ› ÛÙËÓ ·˘ÙÔÂÓÂÚÁÔÔ›ËÛË
ÙˆÓ ·Ú·¿Óˆ ÌÔÚ›ˆÓ (31,32). ∂ȉÈÎfiÙÂÚ·, Ë
ηۿÛË-8 ÌÔÚ› Ó· ÂÓÂÚÁÔÔÈ‹ÛÂÈ ·Â˘ı›-
·˜ ¿ÏÏ· ‰Ú·ÛÙÈο ÌfiÚÈ·, fiˆ˜ Â›Ó·È Ë Î·Û¿-
ÛË-3. ∏ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ Î·Û·ÛÒÓ Â›Ó·È ‰˘-
Ó·ÙfiÓ Ó· ÂÌÔ‰ÈÛÙ› ·fi ÙË BCL-2 (33), ÂÓÒ
οو ·fi ÔÚÈṲ̂Ó˜ Û˘Óı‹Î˜ Â›Ó·È ‰˘Ó·ÙfiÓ Ë
ηۿÛË-3 Ó· ÂȉڿÛÂÈ Î·Ù·Ï˘ÙÈο ÛÙËÓ
ÚˆÙ½ÓË BCL-2, ÚÔηÏÒÓÙ·˜ ÙËÓ ÂÚ·ÈÙ¤Úˆ
ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ Î·Û·ÛÒÓ Î·È Î·Ù’ ¤ÎÙ·-
ÛË ÙËÓ ÂÓÂÚÁÔÔ›ËÛË Ù˘ ·fiÙˆÛ˘ (34).
∏ ‰È·‰Èηۛ· Ù˘ ·fiÙˆÛ˘ ·Ó·ÛÙ¤ÏÏÂ-
Ù·È ÌÂÚÈÎÒ˜ ›Ù Ì ÙÔÓ ·Ó·ÛÙÔϤ· Ù˘ η-
Û¿Û˘ zVAD-FMK (carbobenzoloxy-Val-Ala-
Asp-fluoromethylketone), ›Ù Ì ÙÔÓ ·Ó·ÛÙÔϤ·
Ù˘ Û˘ÓıÂÙ¿Û˘ Ù˘ ÎÂÚ·Ì›‰Ë˜ fumonisin B1.
À¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ fiÙÈ Ë Î·Û¿ÛË 1 Î·È Ë ÎÂ-
Ú·Ì›‰Ë ÂÌϤÎÔÓÙ·È ÛÙË ‰È·‰Èηۛ· Ù˘ ˘ÂÚ-
ÁÏ˘Î·ÈÌ›·˜ Î·È Ù˘ ·fiÙˆÛ˘ (3). ∆· ÁÂÁÔÓfiÙ·
Ô˘ Ô‰ËÁÔ‡Ó ·fi ÙËÓ ˘ÂÚÁÏ˘Î·ÈÌ›· ÛÙËÓ ·fi-
ÙˆÛË ÛÙÔ ÚÔÂÌÊ˘Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ Ù˘ ·Ó¿Ù˘-
͢ (19) Û˘ÓÔ„›˙ÔÓÙ·È ÛÙËÓ ∂ÈÎfiÓ· 1.
∏ ÂÓÂÚÁÔÔ›ËÛË Ù˘ ·fiÙˆÛ˘ ÛÙÔ ÛÙ¿-
‰ÈÔ ·˘Ùfi Ù˘ ·Ó¿Ù˘Í˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ
›Ù ÛÂ Û˘ÁÁÂÓ›˜ ‰È·Ì·Úٛ˜ ÙÔ˘ ÂÌ‚Ú‡Ô˘, ›-
ÙÂ, Â¿Ó Ë ·ÒÏÂÈ· ÂÌ‚Ú˘˚ÎÒÓ Î˘ÙÙ¿ÚˆÓ Â›Ó·È
ÛËÌ·ÓÙÈ΋, Û ·Ô‚ÔÏ‹. ™¯ÂÙÈΤ˜ ÌÂϤÙ˜
¶·È‰È·ÙÚÈ΋ 2006;69:409-416
412 µ. ¢ÚfiÛÔ˘-∞Á·Î›‰Ô˘, ∂. ™ˆÙËÚ›‰Ô˘
¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ ¤Ó·˜ ÎÚÈÙÈÎfi˜ ·ÚÈıÌfi˜ ÂÌ‚Ú˘˚-ÎÒÓ Î˘ÙÙ¿ÚˆÓ ÚÔ··ÈÙÂ›Ù·È ÁÈ· ÙË Ê˘ÛÈÔÏÔÁÈ-΋ ·Ó¿Ù˘ÍË ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÌÂÙ¿ ÙËÓ ÂÌʇÙ¢-Û‹ ÙÔ˘ (35,36). ∏ ·ÒÏÂÈ· ÂÚÈÛÛfiÙÂÚˆÓ ·fi60% ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ Î˘ÙÙ¿ÚˆÓ Ô‰ËÁ› ÛÙÔÓ ÂÌ-‚Ú˘˚Îfi ı¿Ó·ÙÔ Î·È ÙËÓ ·ÔÚÚfiÊËÛË ÙÔ˘ ÂÌ-‚Ú‡Ô˘ (36). ∂ÓÙÔ‡ÙÔȘ, Ë ·ÒÏÂÈ· ÏÈÁfiÙÂÚˆÓÂÌ‚Ú˘˚ÎÒÓ Î˘ÙÙ¿ÚˆÓ (.¯. 40-45%) ÌÔÚ› Ó·Ô‰ËÁ‹ÛÂÈ Â›Ù Û ·ÔÚÚfiÊËÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ ›-Ù ÛÙË ‰ËÌÈÔ˘ÚÁ›· Û˘ÁÁÂÓÒÓ ‰È·Ì·ÚÙÈÒÓ.
∏ ·ÁˆÁ‹ Ù˘ ·fiÙˆÛ˘ ÛÙÔ ÚÔÂÌÊ˘-Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ ÌÂÙ¿ ·fi ¤ÎıÂÛË Û ÙÔÍÈÎÔ‡˜·Ú¿ÁÔÓÙ˜ ÁÈ· ÌÈÎÚfi ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· Ô‰Ë-Á› Û ‰˘ÛϷۛ˜ ÙÔ˘ ÓˆÙÈ·›Ô˘ ۈϋӷ, ÙˆÓ¿ÎÚˆÓ Î·È ÙˆÓ ÎÔÈÏÈ·ÎÒÓ ÙÔȯˆÌ¿ÙˆÓ, ·Úfi-ÌÔȘ Ì ÙȘ ‰È·Ì·Úٛ˜ ÙˆÓ ‚ÚÂÊÒÓ ÙˆÓ ‰È·-‚ËÙÈÎÒÓ Á˘Ó·ÈÎÒÓ (37-39). º·›ÓÂÙ·È, ÏÔÈfiÓ,fiÙÈ Ô ·˘ÍË̤ÓÔ˜ ‹ ÂÈÙ·¯˘ÓfiÌÂÓÔ˜ ÚÔÁÚ·Ì-Ì·ÙÈṲ̂ÓÔ˜ ΢ÙÙ·ÚÈÎfi˜ ı¿Ó·ÙÔ˜ ηٿ ÙË ‰È¿Ú-ÎÂÈ· ÙÔ˘ ÚÔÂÌÊ˘Ù¢ÙÈÎÔ‡ ÛÙ·‰›Ô˘ Ù˘ ·Ó¿-Ù˘Í˘ ¢ı‡ÓÂÙ·È ÂÓ Ì¤ÚÂÈ ÁÈ· ÙȘ Û˘ÁÁÂÓ›˜‰È·Ì·Úٛ˜ ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÛÙÔÓ ·ÚÚ‡ıÌÈÛÙÔ Û·Î-¯·ÚÒ‰Ë ‰È·‚‹ÙË Ù˘ ÌËÙ¤Ú·˜.
‚) ªÂÙÂÌÊ˘Ù¢ÙÈ΋ ‰È·‚ËÙÈ΋
ÂÌ‚Ú˘Ô¿ıÂÈ·
∏ ›‰Ú·ÛË Ù˘ ˘ÂÚÁÏ˘Î·ÈÌ›·˜ ÛÙËÓ ÂÌ-‚Ú˘ÔÁ¤ÓÂÛË ¤¯ÂÈ ÌÂÏÂÙËı› ÂÎÙÂÓÒ˜ Î·È Î·Ù¿
ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÌÂÙÂÌÊ˘Ù¢ÙÈÎÔ‡ ÛÙ·‰›Ô˘
·Ó¿Ù˘Í˘. ∏ ÂÌʇÙ¢ÛË Ù˘ ‚Ï·ÛÙÔ·ÛÙ˘
ÛÙÔÓ ¿ÓıÚˆÔ Ï·Ì‚¿ÓÂÈ ¯ÒÚ· ÙË 2Ë Â‚‰ÔÌ¿‰·
Ù˘ ·ËÛ˘ (40). ∏ ‰È·‰Èηۛ· Ù˘ ·fiÙˆÛ˘
¤¯ÂÈ Û˘Ó‰Âı› ¿ÌÂÛ· Ì ÙËÓ ˘ÂÚÁÏ˘Î·ÈÌ›· ηÈ
ÙËÓ ·Ó¿Ù˘ÍË Û˘ÁÁÂÓÒÓ ‰È·Ì·ÚÙÈÒÓ Î·Ù¿ ÙË
‰È¿ÚÎÂÈ· ÙÔ˘ ÌÂÙÂÌÊ˘Ù¢ÙÈÎÔ‡ ÛÙ·‰›Ô˘ ·Ó¿-
Ù˘Í˘. ∞˘Ùfi ·Ô‰›‰ÂÙ·È Û ÌÂȈ̤ÓË ¤ÎÊÚ·-
ÛË ÂÓfi˜ ÛËÌ·ÓÙÈÎÔ‡ ·Ó·Ù˘ÍÈ·ÎÔ‡ ÁÔÓȉ›Ô˘
ÂϤÁ¯Ô˘, ÙÔ˘ Pax3. ∆Ô Pax3 Â›Ó·È ¤Ó·˜ ÌÂÙ·-
ÁÚ·ÊÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ··Ú·›ÙËÙÔ˜ ÁÈ· ÙË Ê˘-
ÛÈÔÏÔÁÈ΋ ·Ó¿Ù˘ÍË ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡ Ó¢ÚÈÎÔ‡
ۈϋӷ (41,42) Î·È Ë ÂÏ·Ùو̤ÓË ¤ÎÊÚ·Û‹ ÙÔ˘
ÛÙ· ÂÈÚ·Ì·Ùfi˙ˆ· ¿ÁÂÈ ÙË ‰È·‰Èηۛ· Ù˘
·fiÙˆÛ˘ ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÓˆÙÈ·›Ô˘ ۈϋ-
Ó· ÙÔ˘ ÂÌ‚Ú‡Ô˘ (4,43,44).
√È ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ Pax3 ¢ı‡ÓÔÓÙ·È ÁÈ·
ÙÔÓ Splotch Ê·ÈÓfiÙ˘Ô ÛÙÔ˘˜ ÔÓÙÈÎÔ‡˜ Î·È ÔÈ
ÔÌÔ˙˘ÁÒÙ˜ ÁÈ· ÙË ÌÂÙ¿ÏÏ·ÍË ·˘Ù‹ ·Ó·Ù‡Û-
ÛÔ˘Ó ‚Ï¿‚˜ ÙÔ˘ ÓˆÙÈ·›Ô˘ ۈϋӷ Î·È ¿ÏϘ
Û˘ÁÁÂÓ›˜ ‰È·Ì·Úٛ˜ Ì 100% ‰ÈÂÈÛ‰˘ÙÈÎfiÙËÙ·
(45-47). ∏ ÔÌ¿‰· ÙÔ˘ Loeken ÚÒÙË Î·Ù¤‰ÂÈÍÂ
fiÙÈ Ë ¤ÎÊÚ·ÛË ÙÔ˘ Pax3 ‹Ù·Ó ÂÚ›Ô˘ ÙÚÂȘ
ÊÔÚ¤˜ ¯·ÌËÏfiÙÂÚË ÛÙ· ¤Ì‚Ú˘· ‰È·‚ËÙÈÎÒÓ Ô-
ÓÙÈÎÒÓ Û˘ÁÎÚÈÙÈο Ì ٷ ¤Ì‚Ú˘· ÌË ‰È·‚ËÙÈÎÒÓ
ÔÓÙÈÎÒÓ (4). ∏ ˘fiıÂÛË fiÙÈ Ë ‰È·‚ËÙÈ΋ ÂÌ-
‚Ú˘Ô¿ıÂÈ· Û˘Ó‰¤ÂÙ·È Ì ÙËÓ ÂÏ·Ùو̤ÓË ¤Î-
ÊÚ·ÛË ÙÔ˘ Pax3, Û˘ÌʈÓ› Ì ¿ÏÏ· ·Ú·‰Â›Á-
Ì·Ù· ‰˘ÛÌÔÚÊÈÒÓ, ÔÈ Ôԛ˜ ·Ó·Ù‡ÛÛÔÓÙ·È
ÛÙÔ˘˜ ·ÔÁfiÓÔ˘˜ ÙˆÓ ‰È·‚ËÙÈÎÒÓ ÔÓÙÈÎÒÓ.
∞˘Ù¤˜ ÔÈ Û˘ÁÁÂÓ›˜ ‰È·Ì·Úٛ˜, Î·È Û˘ÁÎÂÎÚÈ-
̤ӷ Ë ÌÈÎÚÔÁÓ·ı›·, ÔÈ Ì˘ÔÛÎÂÏÂÙÈΤ˜ Î·È Î·Ú-
‰È·Î¤˜ ‰˘ÛϷۛ˜, ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È Â›Û˘
·fi ‰È·Ù·Ú·¯‹ Ù˘ ·Ó¿Ù˘Í˘ ÈÛÙÒÓ Ô˘ Û¯Â-
Ù›˙ÔÓÙ·È Ì ÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ Pax3 (48).
∆Ô Pax3 ‰Ú· ˆ˜ ÌÂÙ·ÁÚ·ÊÈÎfi˜ ·Ú¿ÁÔÓÙ·˜
Ô˘ Ú˘ıÌ›˙ÂÈ ÙËÓ ¤ÎÊÚ·ÛË ‰‡Ô ÁÔÓȉ›ˆÓ, ÙÔ˘
Cdc46 (37) Î·È ÙÔ˘ Dep1 (38). ∆Ô Cdc46 ÂÌϤ-
ÎÂÙ·È ÛÙË Û‡ÓıÂÛË DNA Î·È ÙË Ú‡ıÌÈÛË ÙÔ˘
΢ÙÙ·ÚÈÎÔ‡ ·ÎÏÔ˘ (49,50) Î·È ÂÎÊÚ¿˙ÂÙ·È È‰È-
·›ÙÂÚ· ÛÙÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ· (51). ¶Èı·ÓÔÏÔ-
Á›ٷÈ, ÏÔÈfiÓ, fiÙÈ Ë ·˘ÍË̤ÓË ¤ÎÊÚ·ÛË ÙÔ˘
Cdc46 ‰È·‰Ú·Ì·Ù›˙ÂÈ Î¿ÔÈÔ ÚfiÏÔ ÛÙË ‰È·‰Èη-
Û›· Ù˘ ·fiÙˆÛ˘ Ô˘ Ô‰ËÁ› ÛÙȘ ‰È·Ù·Ú·-
¯¤˜ ·Ó¿Ù˘Í˘ ÙÔ˘ ÂÌ‚Ú‡Ô˘. ∆Ô Dep1 Û˘Ó‰¤Â-
Ù·È, ›Û˘, Ì ÙËÓ ÂÌÊ¿ÓÈÛË Û˘ÁÁÂÓÒÓ ‰È·Ì·Ú-
ÙÈÒÓ (52) Û ÌË Ú˘ıÌÈ˙fiÌÂÓÔ ‰È·‚‹ÙË ÛÙËÓ
·ËÛË Î·È ıˆÚÂ›Ù·È fiÙÈ Îˆ‰ÈÎÔÔÈ› ¤Ó· ÌÈ-
ÎÚfi, ÌË ÌÂÙ·ÊÚ¿ÛÈÌÔ, Ú˘ıÌÈÛÙÈÎfi ÌfiÚÈÔ RNA,
Ë ·Ú·ÁˆÁ‹ ÙÔ˘ ÔÔ›Ô˘ ÌÂÈÒÓÂÙ·È ÛÙ· ¤Ì‚Ú˘·
Ì ‰È·Ù·Ú·¯¤˜ ÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ Pax3. H ›-
‰Ú·ÛË ÙÔ˘ Pax3 ÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ Dep1 ηı˘-
ÛÙÂÚ› Û ۇÁÎÚÈÛË Ì ÂΛÓË ÙÔ˘ Cdc46. °È·
¶·È‰È·ÙÚÈ΋ 2006;69:409-416
∂ÈÎfiÓ· 1. ∂ÓÂÚÁÔÔ›ËÛË Ù˘ ·fiÙˆÛ˘ ÛÙËÓ ÚÔÂÌÊ˘-Ù¢ÙÈ΋ ‰È·‚ËÙÈ΋ ÂÌ‚Ú˘Ô¿ıÂÈ·.
ÀÂÚÁÏ˘Î·ÈÌ›·
∫·ÙÈÔ‡Û· Ú‡ıÌÈÛË ÙˆÓ ÌÂÙ·ÊÔÚ¤ˆÓ ÁÏ˘Îfi˙˘
™ËÌ·ÓÙÈ΋ ÙÒÛË ÙˆÓ ÂχıÂÚˆÓ ÂȤ‰ˆÓ ÁÏ˘Îfi˙˘ ÛÙË ‚Ï·ÛÙÔ·ÛÙË
™‹Ì· ΢ÙÙ·ÚÈÎÔ‡ ı·Ó¿ÙÔ˘
∞‡ÍËÛË ÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ p53
∞‡ÍËÛË ÛÙËÓ ¤ÎÊÚ·ÛË Ù˘ ÚÔ·ÔÙˆÙÈ΋˜ ÚˆÙ½Ó˘ µ∞Ã
∂ÓÂÚÁÔÔ›ËÛË ÙˆÓ Î·Û·ÛÒÓ
∞fiÙˆÛË
413AfiÙˆÛË ÛÙË ‰È·‚ËÙÈ΋ ÂÌ‚Ú˘Ô¿ıÂÈ·
ÙÔÓ ÏfiÁÔ ·˘Ùfi, Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ÙÔ Cdc46
Ú˘ıÌ›˙ÂÙ·È ¿ÌÂÛ· ·fi ÙÔ Pax3, ÛÂ ·ÓÙ›ıÂÛË ÌÂ
ÙÔ Dep1 Ô˘ Ú˘ıÌ›˙ÂÙ·È ¤ÌÌÂÛ·. ∆· ÁÂÁÔÓfiÙ·
Ô˘ Ô‰ËÁÔ‡Ó ·fi ÙËÓ ˘ÂÚÁÏ˘Î·ÈÌ›· ÛÙËÓ
·fiÙˆÛË ÛÙÔ ÌÂÙÂÌÊ˘Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ ·Ó¿-
Ù˘Í˘ (19) Û˘ÓÔ„›˙ÔÓÙ·È ÛÙËÓ ∂ÈÎfiÓ· 2.
∞ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ ÂÚ¤ıÈÛÌ· Ô˘ ÂÓÂÚÁÔ-
ÔÈ› ÙÔÓ ·ÔÙˆÙÈÎfi Ì˯·ÓÈÛÌfi, Ë ÙÂÏÈ΋ Ê¿-
ÛË ÙÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ı·Ó¿ÙÔ˘ ̤ۈ Ù˘ ‰È·‰Èη-
Û›·˜ ·˘Ù‹˜ ·ÎÔÏÔ˘ı› ÎÔÈÓ‹ ÂÓ˙˘Ì·ÙÈ΋ Ô‰fi.
∞Ú¯Èο, ÎÔÈÓfi Ê·ÈÓfiÌÂÓÔ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘
·fiÙˆÛ˘ ·ÔÙÂÏ› Ë ÏÂÈÙÔ˘ÚÁÈ΋ ηٷÛÙÚÔ-
Ê‹ ÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ Ì ÙË ‰ËÌÈÔ˘ÚÁ›· ÏÂÈÙÔ˘Ú-
ÁÈÎÒÓ ÔÒÓ (53). ∞˘Ù¤˜ Â›Ó·È ÂÚÈÔ¯¤˜ ÌÂٷ͇
Ù˘ ÂÛˆÙÂÚÈ΋˜ Î·È Â͈ÙÂÚÈ΋˜ ÌÂÌ‚Ú¿Ó˘ ÙÔ˘
ÌÈÙÔ¯ÔÓ‰Ú›Ô˘ Ô˘ ·ÔÙÂÏÔ‡ÓÙ·È ·fi ÔÏ˘Úˆ-
ÙÂ˚ÓÈο ÌÔÚȷο Û˘ÌϤÁÌ·Ù·, Ù· ÔÔ›· ¤¯Ô˘Ó
ÙËÓ È‰ÈfiÙËÙ· Ó· ·˘Í¿ÓÔ˘Ó ÙË ÌÈÙÔ¯ÔÓ‰Úȷ΋
‰È·ÂÚ·ÙfiÙËÙ· ÁÈ· ‰È¿ÊÔÚ˜ “··ÁÔÚÂ˘Ì¤Ó˜”
Ô˘Û›Â˜. ∂ÈϤÔÓ, ÔÈ Î·Û¿Û˜ ·ÔÙÂÏÔ‡Ó Ì¤-
ÚÔ˜ Ù˘ ÎÔÈÓ‹˜ ÙÂÏÈ΋˜ ÂÓ˙˘Ì·ÙÈ΋˜ Ô‰Ô‡ Î·È Â›-
Ó·È ÙÂÏ›ˆ˜ ··Ú·›ÙËÙ˜ ÁÈ· ÙËÓ ·fiÙˆÛË
(54). ∏ ÚˆÙÂÔÏ˘ÙÈ΋ ‰Ú¿ÛË ÙˆÓ Î·Û·ÛÒÓ
¤¯ÂÈ ˆ˜ ÛÙfi¯Ô ÌÂÁ¿ÏÔ ·ÚÈıÌfi ÚˆÙÂ˚ÓÒÓ Ô˘
ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ÔÌÔÈfiÛÙ·ÛË, ÙËÓ ÂÈÛ΢‹ ηÈ
ÙË ‰È·Ù‹ÚËÛË Ù˘ ΢ÙÙ·ÚÈ΋˜ ‰ÔÌ‹˜ (55).
ÕÏÏÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ıˆÚÔ‡ÓÙ·È fiÙÈ
ÂÌϤÎÔÓÙ·È ÛÙËÓ ·Ó¿Ù˘ÍË Ù˘ ‰È·‚ËÙÈ΋˜
ÂÌ‚Ú˘Ô¿ıÂÈ·˜ Â›Ó·È ÔÈ ·ÁÎÚ·ÙÈÎÔ› ÌÂÙ·ÁÚ·-
ÊÈÎÔ› ·Ú¿ÁÔÓÙ˜ Isl-1 Î·È HLXB9, Ô˘ ›ӷÈ
··Ú·›ÙËÙÔÈ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜.
ªÂϤÙ˜ Û ÂÈÚ·Ì·ÙÈο ÌÔÓ٤Ϸ ÔÓÙÈÎÒÓ
¤‰ÂÈÍ·Ó fiÙÈ ÌÂÙ·ÏÏ¿ÍÂȘ ÙˆÓ ·ÁÎÚ·ÙÈÎÒÓ ÌÂ-
Ù·ÁÚ·ÊÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÚÔηÏÔ‡Ó ‰˘ÛÏ·-
ۛ˜ ÙÔ˘ Ô˘Ú·›Ô˘ ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÛÔÓ‰˘ÏÈÎÔ‡
ۈϋӷ, ·ÚfiÌÔȘ Ì ÂΛӘ Ô˘ ·Ú·ÙËÚÔ‡-
ÓÙ·È ÛÙË ‰È·‚ËÙÈ΋ ÂÌ‚Ú˘Ô¿ıÂÈ· (56,57). ™Ù·
¿Û¯ÔÓÙ· ÙÌ‹Ì·Ù· ‚Ú¤ıËΠ·ıÔÏÔÁÈ΋ ¤Î-
ÊÚ·ÛË ÙÔ˘ Pax-1 Î·È ·˘ÍË̤ÓË ·Ó·ÏÔÁ›· ΢Ù-
Ù·ÚÈ΋˜ ·fiÙˆÛ˘, Ô˘ ·ÊÔÚÔ‡Û ΢ڛˆ˜ Ù·
ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· (56). ∆· Â˘Ú‹Ì·Ù· ·˘-
Ù¿ ˘Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Ë ÌÂÙ·‚ÔÏÈ΋ ‰È·Ù·Ú·¯‹
Ù˘ ‰È·‚ËÙÈ΋˜ ·ËÛ˘ ÂËÚ¿˙ÂÈ ÙË ÏÂÈÙÔ˘Ú-
Á›· ÙˆÓ ·ÁÎÚ·ÙÈÎÒÓ ÌÂÙ·ÁÚ·ÊÈÎÒÓ ·Ú·Áfi-
ÓÙˆÓ, ÔÈ ÔÔ›ÔÈ Ì ÙË Ì ÙË ÛÂÈÚ¿ ÙÔ˘˜ Ô‰ËÁÔ‡Ó
ÛÙËÓ ÂΉ‹ÏˆÛË ÙÔ˘ ¯·Ú·ÎÙËÚÈÛÙÈÎÔ‡ Ê·ÈÓÔÙ‡-
Ô˘ Ù˘ ‰È·‚ËÙÈ΋˜ ÂÌ‚Ú˘Ô¿ıÂÈ·˜.
ÕÏÏÔÈ Ì˯·ÓÈÛÌÔ› Ô˘ ¤¯Ô˘Ó ÂÓÔ¯ÔÔÈËı›
ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Û˘ÁÁÂÓÒÓ ‰È·Ì·ÚÙÈÒÓ ÛÙÔ ÌÂ-
ÙÂÌÊ˘Ù¢ÙÈÎfi ÛÙ¿‰ÈÔ Â›Ó·È Ë ·Ó¿ÚÎÂÈ· ÙˆÓ
ÌÂÌ‚Ú·ÓÈÎÒÓ ÏÈȉ›ˆÓ (.¯. Ì˘Ô˚ÓÔÛÈÙfiÏË, ·Ú·-
¯È‰ÔÓÈÎfi Ô͇) (58,59), ÔÈ ·ÏÏ·Á¤˜ ÛÙË Û‡ÓıÂÛË
Î·È ÙÔÓ ÌÂÙ·‚ÔÏÈÛÌfi ÙˆÓ ÚÔÛÙ·ÁÏ·Ó‰ÈÓÒÓ
(60,61) Î·È Ë ‰È·Ù·Ú·¯‹ ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡ ÌÂÙ·‚Ô-ÏÈÛÌÔ‡ ÏfiÁˆ ÙˆÓ ˘„ËÏÒÓ ÂȤ‰ˆÓ ÁÏ˘Îfi˙˘(55,64). ¶Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ÔÈ ÙÚÂȘ ·˘ÙÔ› Ì˯·-ÓÈÛÌÔ› Û˘ÁÎÏ›ÓÔ˘Ó Û ¤Ó·Ó ÎÔÈÓfi ÌÂÙ·‚ÔÏÈÎfi‰ÚfiÌÔ, Ô˘ Ô‰ËÁ› ÙÂÏÈο ÛÙËÓ ·‡ÍËÛË ÙˆÓÂÏ¢ı¤ÚˆÓ ÚÈ˙ÒÓ ÙÔ˘ Ô͢ÁfiÓÔ˘, ÙËÓ ˘ÂÚÔÍ›-‰ˆÛË ÙˆÓ ÏÈȉ›ˆÓ Î·È ÙË ‚Ï¿‚Ë ÙˆÓ ÌÈÙÔ¯ÔÓ-‰Ú›ˆÓ (66,67). ∂ÎÙÈÌ¿Ù·È fiÙÈ Ë ‚Ï¿‚Ë ÙˆÓ ÌÈÙÔ-¯ÔÓ‰Ú›ˆÓ, Ì·˙› Ì ÙȘ ÂχıÂÚ˜ Ú›˙˜ Ô͢ÁfiÓÔ˘,ı· ÌÔÚÔ‡Û ӷ ÂÓÂÚÁÔÔÈ‹ÛÂÈ ÙË ‰È·‰Èηۛ·Ù˘ ·fiÙˆÛ˘, Ì ·ÔÙ¤ÏÂÛÌ· ‰È·Ù·Ú·¯¤˜ Ù˘·Ó¿Ù˘Í˘ ÙÔ˘ ÂÌ‚Ú‡Ô˘ (65-67). ∂ÓÙÔ‡ÙÔȘ, ·˘-Ù‹ Ë ˘fiıÂÛË Â›Ó·È ÌfiÓÔ ıˆÚËÙÈ΋. √È YangÎ·È Û˘Ó. ÂÎÙÈÌÔ‡Ó fiÙÈ ÔÈ ·ÏÏ·Á¤˜ ÛÙÔ Â›Â‰ÔÙˆÓ ÌÈÙÔ¯ÔÓ‰Ú›ˆÓ ÌÔÚÔ‡Ó Ó· ·ÓÙÈÛÙÚ·ÊÔ‡Ó ÌÂÙËÓ ·ÓÙÈÔÍÂȉˆÙÈ΋ ·ÁˆÁ‹ (68).
¢˘ÛÏÂÈÙÔ˘ÚÁ›· ÙˆÓ Ì˯·ÓÈÛÌÒÓ ·fiÙˆ-Û˘ ÛÙË ‰È·‚ËÙÈ΋ ·ËÛË ÌÔÚ› ·ÎfiÌË Ó·ÚÔ·„ÂÈ ÏfiÁˆ ÙˆÓ ·ÓÔÛÈ·ÎÒÓ ‰È·Ù·Ú·¯ÒÓ,Ô˘ ÔÏϤ˜ ÊÔÚ¤˜ Û˘ÓÔ‰Â‡Ô˘Ó ÙË ‰È·‚ËÙÈ΋·ËÛË, fiˆ˜ Â›Ó·È Ë ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ‰ÈÎÙ‡-Ô˘ ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ. ¶ÂÈÚ·Ì·ÙÈΤ˜ ÌÂϤÙ˜Û ‰È·‚ËÙÈο ÔÓÙ›ÎÈ· ¤‰ÂÈÍ·Ó fiÙÈ Ë ¯ÔÚ‹ÁËÛË·ÓÔÛÔ-‰ÈÂÁÂÚÙÒÓ ÛÙË ÌËÙ¤Ú· ÂËÚ¤·Û ÙËÏÂÈÙÔ˘ÚÁ›· ÔÏÏÒÓ ·ÔÙˆÙÈÎÒÓ Î·È ·ÓÙÈ-·ÔÙˆÙÈÎÒÓ ÁÔÓȉ›ˆÓ ηıÒ˜ Î·È ÁÔÓȉ›ˆÓÔ˘ Ú˘ıÌ›˙Ô˘Ó ÙÔÓ Î˘ÙÙ·ÚÈÎfi ÔÏÏ·Ï·ÛÈ·-ÛÌfi, ÂÏ·ÙÙÒÓÔÓÙ·˜ ÙË Û˘¯ÓfiÙËÙ· Û˘ÁÁÂÓÒÓ‰˘ÛÏ·ÛÈÒÓ (69).
™˘ÓÔÙÈο, Ù· ˘¿Ú¯ÔÓÙ· ‰Â‰Ô̤ӷ ‰Â›-¯ÓÔ˘Ó fiÙÈ, ·Ó Î·È ÔÈ Ì˯·ÓÈÛÌÔ› ‰ËÌÈÔ˘ÚÁ›·˜Û˘ÁÁÂÓÒÓ ‰˘ÛÏ·ÛÈÒÓ ÛÙË ‰È·‚ËÙÈ΋ ·ËÛˉȷʤÚÔ˘Ó ÌÂٷ͇ ÚÔÂÌÊ˘Ù¢ÙÈÎÔ‡ Î·È ÌÂ-ÙÂÌÊ˘Ù¢ÙÈÎÔ‡ ÛÙ·‰›Ô˘ ·Ó¿Ù˘Í˘ ÙÔ˘ ÂÌ‚Ú‡-Ô˘, Ë ‚·ÛÈ΋ ·ıÔÁ¤ÓÂÈ· Â›Ó·È ›‰È·: Ë ˘ÂÚÁÏ˘-ηÈÌ›· Ù˘ ÌËÙ¤Ú·˜ ‰ÈÂÁ›ÚÂÈ ÙÔÓ ÚÔÁÚ·ÌÌ·ÙÈ-Ṳ̂ÓÔ Î˘ÙÙ·ÚÈÎfi ı¿Ó·ÙÔ ÛÙÔ ·Ó·Ù˘ÛÛfiÌÂÓÔ¤Ì‚Ú˘Ô, Ì ·ÔÙ¤ÏÂÛÌ· ‰˘ÛϷۛ˜ ‹ ·Ô‚ÔÏ‹ÙÔ˘ ÂÌ‚Ú‡Ô˘ (19).
¶·È‰È·ÙÚÈ΋ 2006;69:409-416
∂ÈÎfiÓ· 2. ∂ÓÂÚÁÔÔ›ËÛË Ù˘ ·fiÙˆÛ˘ ÛÙË ÌÂÙÂÌÊ˘-Ù¢ÙÈ΋ ‰È·‚ËÙÈ΋ ÂÌ‚Ú˘Ô¿ıÂÈ·.
ÀÂÚÁÏ˘Î·ÈÌ›·
ªÂ›ˆÛË ÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ Pax3
∞‡ÍËÛË ÛÙËÓ ¤ÎÊÚ·ÛË ªÂ›ˆÛË ÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘ Cdc46 ÙÔ˘ Dep1
(ÚÒÈÌÔ ÛÙ¿‰ÈÔ) (fi„ÈÌÔ ÛÙ¿‰ÈÔ )
∞fiÙˆÛË
414 µ. ¢ÚfiÛÔ˘-∞Á·Î›‰Ô˘, ∂. ™ˆÙËÚ›‰Ô˘
µÈ‚ÏÈÔÁÚ·Ê›·
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417∞¡∞™∫√¶∏™∏ REVIEW ARTICLE
√ Û˘Ó‰˘·ÛÌfi˜ ÂÈÛÓÂfiÌÂÓˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ
Î·È ‚2-·ÁˆÓÈÛÙÒÓ Ì·ÎÚ¿˜ ‰Ú¿Û˘ ÛÙË ıÂڷ›·
ÙÔ˘ ·È‰ÈÎÔ‡ ¿ÛıÌ·ÙÔ˜
ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜1, ∫. ¡. ¶Ú›ÊÙ˘2
¶ÂÚ›ÏË„Ë: ™‡Ìʈӷ Ì ÙȘ ÔÌÔʈӛ˜ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜, Ô Û˘Ó‰˘·ÛÌfi˜ ÂÈ-ÛÓÂfiÌÂÓˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ (∂ÈÛ∫™) Î·È ‚2-·ÁˆÓÈÛÙÒÓ Ì·ÎÚ¿˜ ‰Ú¿Û˘ ·ÔÙÂÏ› ÙË ıÂڷ›·ÂÎÏÔÁ‹˜ ÛÙÔ Ì¤ÙÚÈÔ Î·È ÛÔ‚·Úfi (›ÌÔÓÔ) ¿ÛıÌ·, ÂÓÒ Ë ÚÔÛı‹ÎË ÙˆÓ ‚2-·ÁˆÓÈÛÙÒÓ Ì·ÎÚ¿˜ ‰Ú¿-Û˘ Û˘ÓÈÛÙ¿Ù·È ÛÙÔ ‹ÈÔ Â›ÌÔÓÔ ¿ÛıÌ· Ô˘ ‰ÂÓ ÂϤÁ¯ÂÙ·È Ì ¯·ÌËϤ˜ ‰fiÛÂȘ ∂ÈÛ∫™, ÙfiÛÔ Û ÂÓ‹-ÏÈΘ fiÛÔ Î·È Û ·È‰È¿, ȉ›ˆ˜ Û ÂΛӷ ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 5 ÂÙÒÓ. ∏ Û˘Ó‰˘·˙fiÌÂÓË ıÂڷ›· ‚·Û›˙ÂÙ·È ÛÙË Û˘ÌÏËڈ̷ÙÈ΋ Ê·Ú̷΢ÙÈ΋ ‰Ú¿ÛË ÙˆÓ ∂ÈÛ∫™(·ÓÙÈÊÏÂÁÌÔÓ҉˘) Î·È ÙˆÓ ‚2-·ÁˆÓÈÛÙÒÓ (‚ÚÔÁ¯Ô‰È·ÛÙÔÏ‹, ‚ÚÔÁ¯ÔÚÔÛÙ·Û›·), ·ÏÏ¿ Î·È ÛÙË Û˘-Ó¤ÚÁÂÈ· Ù˘ ‰Ú¿Û˘ ÙˆÓ ‰‡Ô ‰È·ÊÔÚÂÙÈÎÒÓ Î·ÙËÁÔÚÈÒÓ ·ÓÙÈ·ÛıÌ·ÙÈÎÒÓ Ê·ÚÌ¿ÎˆÓ Û ΢ÙÙ·ÚÈÎfiÂ›Â‰Ô (¢fi‰ˆÛË Ù˘ ¤ÎÊÚ·Û˘ ÙˆÓ ‚2-˘Ô‰Ô¯¤ˆÓ ÛÙËÓ ÂÈÊ¿ÓÂÈ· ÙˆÓ Î˘ÙÙ¿ÚˆÓ ·fi Ù· ∂ÈÛ∫™Î·È ÂÓ›Û¯˘ÛË ÙˆÓ ÂÓ‰Ô΢ÙÙ¿ÚÈˆÓ Ì˯·ÓÈÛÌÒÓ ‰Ú¿Û˘ ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ì¤Ûˆ ‰È¤ÁÂÚÛ˘ ÙˆÓ·‰ÚÂÓÂÚÁÈÎÒÓ ˘Ô‰Ô¯¤ˆÓ ·fi ÙÔ˘˜ ‚2-·ÁˆÓÈÛÙ¤˜). ∂ÎÙÂٷ̤ÓË ÎÏÈÓÈ΋ ¤Ú¢ӷ Û ÂÓ‹ÏÈΘ·ÛıÌ·ÙÈÎÔ‡˜ ¤¯ÂÈ ‰Â›ÍÂÈ, ¤Ú·Ó οı ·ÌÊÈ‚ÔÏ›·˜, ÙËÓ ˘ÂÚÔ¯‹ ÙÔ˘ Û˘Ó‰˘·ÛÌÔ‡ ∂ÈÛ∫™ Î·È ‚2-·Áˆ-ÓÈÛÙÒÓ Û˘ÁÎÚÈÙÈο Ì ÌÔÓÔıÂڷ›· Ì ÙËÓ ›‰È· ‹ Î·È ‰ÈÏ¿ÛÈ· ‰fiÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉԇ˜. ™ÙËÓ·ÚÔ‡Û· ·Ó·ÛÎfiËÛË Á›ÓÂÙ·È ·Ó·ÊÔÚ¿ ÛÙȘ ÂÚÈÔÚÈṲ̂Ó˜ ·ÚÈıÌËÙÈο ·È‰È·ÙÚÈΤ˜ ÌÂϤÙ˜ Ô˘‰ÈÂÚ¢ÓÔ‡Ó ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Î·È ÙËÓ ·ÛÊ¿ÏÂÈ· Ù˘ Û˘Ó‰˘·˙fiÌÂÓ˘ ıÂڷ›·˜ Û ۇÁÎÚÈ-ÛË Ì ÙË ¯ÔÚ‹ÁËÛË ›‰È·˜ ‹ ‰ÈÏ¿ÛÈ·˜ ‰fiÛ˘ ∂ÈÛ∫™. ™ÙËÓ ÏÂÈÔÓfiÙËÙ¿ ÙÔ˘˜ - Î·È ÔÈ ÌÂϤÙ˜ ·˘Ù¤˜- ˘ÔÛÙËÚ›˙Ô˘Ó ÙËÓ ˘ÂÚÔ¯‹ ÙÔ˘ Û˘Ó‰˘·ÛÌÔ‡ ∂ÈÛ∫™ Î·È ‚2-·ÁˆÓÈÛÙ‹ Ì·ÎÚ¿˜ ‰Ú¿Û˘. ∂›Û˘, Û˘-˙ËÙÔ‡ÓÙ·È Ù· Ôχ ÂӉȷʤÚÔÓÙ· Î·È ÂÏȉÔÊfiÚ· ·ÔÙÂϤÛÌ·Ù· ‰‡Ô ÚfiÛÊ·ÙˆÓ ÌÂÁ¿ÏˆÓ ÎÏÈÓÈ-ÎÒÓ ÌÂÏÂÙÒÓ Û ÂÓ‹ÏÈΘ ·ÛıÂÓ›˜ Ô˘ ‰ÈÂÚ¢ÓÔ‡Ó ÙË ‰˘Ó·ÙfiÙËÙ· “ȉ·ÓÈÎÔ‡” ÂϤÁ¯Ô˘ ÙÔ˘ ¿ÛıÌ·-ÙÔ˜ ÌÂ Û˘Á¯ÔÚ‹ÁËÛË (Ì ÂÓÈ·›· Û˘Û΢‹) ÙˆÓ Û˘Ó‰˘·ÛÌÒÓ Û·ÏÌÂÙÂÚfiÏ˘/ÊÏÔ˘ÙÈη˙fiÓ˘ Î·È ‚Ô˘-‰ÂÛÔÓ›‰Ë˜/ÊÔÚÌÔÙÂÚfiÏ˘.
§¤ÍÂȘ ÎÏÂȉȿ: ÕÛıÌ·, ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, ‚2-·ÁˆÓÈÛÙ¤˜ Ì·ÎÚ¿˜ ‰Ú¿Û˘, Û˘Ó‰˘·ÛÌfi˜(Ô›).
Combination therapy with inhaled corticosteroids and long-acting
‚2-agonists in childhood asthma
M. B. Anthracopoulos1, K. N. Priftis2
Abstract: According to current guidelines for the management of asthma, the combination of inhaledcorticosteroids (iCS) and long acting ‚2-agonists (LABA) is recommended in moderate and severe (per-sistent) asthma, and LABA constitute add-on treatment in mild persistent asthma that cannot be con-trolled by low-dose iCS, especially in adults and children over 5 years of age. Such combination treat-ment is based on sound scientific evidence of the complementary pharmacological effects of iCS (anti-inflammatory) and LABA (bronchodilation, bronchoprotection), as well as their synergistic effect at thecellular level (iCS induction of the expression of ‚2-agonists on the cellular membrane and amplificationof intracellular mechanisms of action of corticosteroids by agonist stimulation of ‚2-adrenoreceptors).Extensive clinical research in adults has proved beyond doubt that combination therapy with iCS andLABA is more effective than iCS monotherapy with an equal or double dose of corticosteroids. Thisreview summarizes the findings of the relatively small number of paediatric studies investigating theeffectiveness and safety of combination therapy as compared to administration of the same or a dou-ble dose of iCS therapy. Most of these studies, like the adult data, support the superiority of iCS/LABAcombination over iCS monotherapy. The intriguing and hopeful results of two recent large clinicaltrials that investigated the feasibility of improved, or even total, asthma control in adult patients
1 ¶·È‰ÔÓ¢ÌÔÓÔÏÔÁÈ΋ªÔÓ¿‰·, ¶·È‰È·ÙÚÈ΋∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘¶·ÙÚÒÓ
2 ∞ÏÏÂÚÁÈÔÏÔÁÈÎfi ∆Ì‹Ì·,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ¶ÂÓÙ¤Ï˘, ∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
ªÈ¯¿Ï˘ ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ¶·ÙÚÒÓ ∆.∫. 265 04, ƒ›Ô, ¶¿ÙÚ· ∂-mail: [email protected]
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 04-04-2006 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 19-09-2006
1 Unit of PaediatricPulmonology, PaediatricClinic of the University ofPatras
2 Department of Allergology,Penteli Children’s Hospital,Athens
Correspondence:
Michael Anthracopoulos Paediatric Clinic of theUniversity of Patras 265 04, Rio, Patra ∂-mail: [email protected]
Date of submission: 04-04-2006Date of approval: 19-09-2006
¶·È‰È·ÙÚÈ΋ 2006;69:417-424
418 ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜, ∫. ¡. ¶Ú›ÊÙ˘
¶·È‰È·ÙÚÈ΋ 2006;69:417-424
using novel management protocols (i.e., salmeterol/fluticasone and the budesonide/formoterolcombinations) are also discussed.
Key words: Asthma, inhaled corticosteroids, long-acting ‚2-agonists, combination(s).
™˘ÓÙÔÌÔÁڷʛ˜
∂ÈÛ∫™ ∂ÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹
cPLA2 ∫˘ÙÙ·ÚÔÏ·ÛÌ·ÙÈ΋ ʈÛÊÔÏÈ¿ÛË ∞2
FEV1 µ›·È· ÂÎÓÂfiÌÂÓÔ˜ fiÁÎÔ˜ ÛÙÔ ÚÒÙÔ
‰Â˘ÙÂÚfiÏÂÙÔ
GRE ∞ÏÏËÏÔ˘¯›Â˜ ÁÔÓȉ›ˆÓ ·ÓÙ·fiÎÚÈÛ˘
ÛÙ· ÁÏ˘ÎÔÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹
IL-5 πÓÙÂÚÏ¢ΛÓË-5
iCS ∂ÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹
LABA ‚2-·ÁˆÓÈÛÙ‹˜(-¤˜) Ì·ÎÚ¿˜ ‰Ú¿Û˘
MAPK ¶ÚˆÙÂ˚ÓÈ΋(-¤˜) ÎÈÓ¿ÛË(-˜) ÂÓÂÚÁÔÔÈ-
Ô‡ÌÂÓË(-˜) Ì ÌÈÙÔÁfiÓÔ
PAF ¶·Ú¿ÁÔÓÙ·˜ ÂÓÂÚÁÔÔ›ËÛ˘ ·ÈÌÔÂÙ·Ï›ˆÓ
PD20 ¢fiÛË ÚfiÎÏËÛ˘ ÙÒÛ˘ ÙÔ˘ FEV1 ηٿ
20%
PEF ª¤ÁÈÛÙË ÂÎÓ¢ÛÙÈ΋ ÚÔ‹
PKA ¶ÚˆÙÂ˚ÓÈ΋ ÎÈÓ¿ÛË A
∂ÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ (∂ÈÛ∫™)
Î·È ‚2-·ÁˆÓÈÛÙ¤˜ Ì·ÎÚ¿˜ ‰Ú¿Û˘
∏ Û˘ÓÂȉËÙÔÔ›ËÛË fiÙÈ ÙÔ ¿ÛıÌ· ·ÔÙÂÏ›
¯ÚfiÓÈ· ÊÏÂÁÌÔÓÒ‰Ë ÓfiÛÔ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Ô‰‹-
ÁËÛ ÛÙËÓ Â˘Ú‡Ù·ÙË ¯Ú‹ÛË ÙˆÓ ÂÈÛÓÂfiÌÂÓˆÓ
ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ (∂ÈÛ∫™) ˆ˜ ÙˆÓ Ï¤ÔÓ
·ÔÙÂÏÂÛÌ·ÙÈÎÒÓ Ê·ÚÌ¿ÎˆÓ ÁÈ· ÙÔÓ ¤ÏÂÁ¯Ô
Ù˘ ÓfiÛÔ˘. ¶Ú¿ÁÌ·ÙÈ, ÛÙÔ Ù¤ÏÔ˜ Ù˘ ‰ÂηÂÙ›·˜
ÙÔ˘ ’80 Î·È ÛÙȘ ·Ú¯¤˜ Ù˘ ÂfiÌÂÓ˘, ÔÈ ÓÂÔÛ˘-
ÛÙ·ı›Û˜ ÙfiÙ ÔÌÔʈӛ˜ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈ-
ÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ Û˘ÓÈÛÙÔ‡Û·Ó ÙËÓ ÚÔԉ¢ÙÈ-
΋ ·‡ÍËÛË Ù˘ ËÌÂÚ‹ÛÈ·˜ ‰fiÛ˘ ÙˆÓ ∂ÈÛ∫™,
ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÈÙ¢¯ı› Ô ¤ÏÂÁ¯Ô˜ ÙˆÓ Û˘-
ÌÙˆÌ¿ÙˆÓ Î·È Ë ‚¤ÏÙÈÛÙË Ó¢ÌÔÓÈ΋ ÏÂÈ-
ÙÔ˘ÚÁ›· ÙÔ˘ ·ÛıÌ·ÙÈÎÔ‡ ·ÛıÂÓÔ‡˜ (2). ™˘Á¯Úfi-
Óˆ˜, Ê·ÚÌ·ÎÔÏÔÁÈΤ˜ ÌÂϤÙ˜ η٤ÛÙËÛ·Ó Ê·-
ÓÂÚfi fiÙÈ Ù· ∂ÈÛ∫™ ÂÈÙ˘Á¯¿ÓÔ˘Ó ÙÔ Ì¤ÁÈÛÙÔ Ù˘
‰Ú¿Û˘ ÙÔ˘˜ Û ۯÂÙÈο ¯·ÌËϤ˜ ‰fiÛÂȘ, Ô˘
¿ÓÙˆ˜ ‰ÂÓ ˘ÂÚ‚·›ÓÔ˘Ó Ù· 1000 Ìg ÂÚ›Ô˘
ËÌÂÚËÛ›ˆ˜ (1).
√È ÂÈÛÓÂfiÌÂÓÔÈ ‚2-·ÁˆÓÈÛÙ¤˜ ·ÔÙÂÏÔ‡Ó
Ù· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚ· ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο
Ê¿Ú̷η ÛÙË ıÂڷ›· ÙÔ˘ ¿ÛıÌ·ÙÔ˜ (2) ηÈ
‰È·ÎÚ›ÓÔÓÙ·È ÛÙÔ˘˜ ‚Ú·¯Â›·˜ (̤ÁÈÛÙË ‰È¿Ú-
ÎÂÈ· 4-6 ÒÚ˜) Î·È ÛÙÔ˘˜ Ì·ÎÚ¿˜ ‰Ú¿Û˘ (̤ÁÈ-
ÛÙË ‰È¿ÚÎÂÈ· ÂÚ›Ô˘ 12 ÒÚ˜). √È ÂÎÏÂÎÙÈÎÔ›
‚2-·ÁˆÓÈÛÙ¤˜ ‚Ú·¯Â›·˜ ‰Ú¿Û˘ (Û·Ï‚Ô˘Ù·Ìfi-
ÏË, ÙÂÚ‚Ô˘Ù·Ï›ÓË) ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙÔ
¿ÛıÌ· ˆ˜ ·Ó·ÎÔ˘ÊÈÛÙÈ΋ “ηْ ›ÎÏËÛÈÓ”
·ÁˆÁ‹, ‰ËÏ·‰‹ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Û˘-
ÌÙˆÌ¿ÙˆÓ Ù˘ ÓfiÛÔ˘. ∂›Û˘, ÚÔÙ›ÓÔÓÙ·È
ˆ˜ ‚ÚÔÁ¯ÔÚÔÛÙ·Û›· Ï›Á· ÏÂÙ¿ ÚÈÓ ·fi ÙËÓ
¿ÛÎËÛË Û ·ÛıÂÓ›˜ Ì “¿ÛıÌ· ÌÂÙ¿ ·fi ¿ÛÎË-
ÛË” (2). ∞ÓÙÈı¤Ùˆ˜, ÔÈ ‚2-·ÁˆÓÈÛÙ¤˜ Ì·ÎÚ¿˜
‰Ú¿Û˘ (Û·ÏÌÂÙÂÚfiÏË: ‚Ú·‰Â›·˜ ¤Ó·Ú͢ ‰Ú¿-
Û˘, ÊÔÚÌÔÙÂÚfiÏË: Ù·¯Â›·˜ ¤Ó·Ú͢ ‰Ú¿Û˘)
ÂÈÛ‹¯ıËÛ·Ó ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË ·fi Ù· ̤۷
Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ ’90 Î·È ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È,
Û‡Ìʈӷ Ì ÙȘ ϤÔÓ ÚfiÛÊ·Ù˜ ÔÌÔʈӛ˜,
ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ˆ˜ Ú˘ıÌÈ-
ÛÙÈ΋ “Ì·ÎÚÔ¯ÚfiÓÈ·” ·ÁˆÁ‹ ÛÂ Û˘Ó‰˘·ÛÌfi ÌÂ
Ù· ∂ÈÛ∫™ (3-5). ™‡Ìʈӷ Ì ÙËÓ ÙÚ¤¯Ô˘Û·
Ú·ÎÙÈ΋ ·ÔÙÂÏÔ‡Ó ÙË ıÂڷ›· ÂÎÏÔÁ‹˜ ÛÙÔ
̤ÙÚÈÔ Î·È ÛÔ‚·Úfi (›ÌÔÓÔ) ¿ÛıÌ· ·È‰ÈÒÓ
ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 5 ÂÙÒÓ Î·È Û˘ÓÈÛÙ¿-
Ù·È Ë ÚÔÛı‹ÎË ÙÔ˘˜ Û ¯·ÌËÏ‹ ‰fiÛË ∂ÈÛ∫™,
ˆ˜ ÚÒÙË ıÂڷ¢ÙÈ΋ ÂÈÏÔÁ‹, ÛÙÔ ‹ÈÔ Â›-
ÌÔÓÔ ¿ÛıÌ· Ô˘ ‰ÂÓ ÂϤÁ¯ÂÙ·È Ì ÙȘ ¯·ÌËϤ˜
·˘Ù¤˜ ‰fiÛÂȘ (2,6-8). ™Â ·È‰È¿ ËÏÈΛ·˜ ÌÈÎÚfi-
ÙÂÚ˘ ÙˆÓ 5 ÂÙÒÓ, Ô Û˘Ó‰˘·ÛÌfi˜ ‚2-·ÁˆÓÈÛÙ‹
Ì·ÎÚ¿˜ ‰Ú¿Û˘ Î·È ¯·ÌËÏ‹˜ ‰fiÛ˘ ∂ÈÛ∫™ ·Ô-
ÙÂÏ› ÙË ıÂڷ›· ÂÎÏÔÁ‹˜ ÛÙÔ Ì¤ÙÚÈÔ (¯ÚfiÓÈÔ)
¿ÛıÌ· Û‡Ìʈӷ Ì ÙËÓ ∞ÌÂÚÈηÓÈ΋ √ÌÔʈ-
Ó›· (6) Î·È ÂÓ·ÏÏ·ÎÙÈ΋ ıÂڷ¢ÙÈ΋ ÂÈÏÔÁ‹
Û‡Ìʈӷ Ì ÙË ¢ÈÂıÓ‹ (ıÂڷ›· ÂÎÏÔÁ‹˜: ̤-
ÙÚÈ· ‰fiÛË ∂ÈÛ∫™) (2) Î·È ÙË µÚÂÙ·ÓÈ΋ √ÌÔ-
ʈӛ· (ıÂڷ›· ÂÎÏÔÁ‹˜: Û˘Ó‰˘·ÛÌfi˜ ·ÓÙÈ-
Ï¢ÎÔÙÚÈÂÓÈÎÔ‡ Î·È ¯·ÌËÏ‹˜ ‰fiÛ˘ ∂ÈÛ∫™) (7).
™ÙË Û˘Ó¤¯ÂÈ· ·ÚÔ˘ÛÈ¿˙ÂÙ·È ÂÚÈÏËÙÈο Ë
Ê·Ú̷΢ÙÈ΋ ‚¿ÛË Ù˘ ·ıÚÔÈÛÙÈ΋˜ Î·È Ë ÌÔ-
Úȷ΋ ‚¿ÛË Ù˘ Û˘ÓÂÚÁÈ΋˜ ‰Ú¿Û˘ ∂ÈÛ∫™ ηÈ
‚2-·ÁˆÓÈÛÙÒÓ Ì·ÎÚ¿˜ ‰Ú¿Û˘ Î·È ·Ú·Ù›ıÂÓÙ·È
Ù· ΢ÚÈfiÙÂÚ· ÎÏÈÓÈο ‰Â‰Ô̤ӷ ÁÈ· ÙËÓ ·ÔÙÂ-
ÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ Û˘Á¯ÔÚ‹ÁËÛ‹˜ ÙÔ˘˜ ÛÂ
·ÛıÌ·ÙÈο ·È‰È¿.
∞ıÚÔÈÛÙÈ΋ Î·È Û˘ÓÂÚÁÈ΋ ‰Ú¿ÛË ∂ÈÛ∫™
Î·È ‚2-·ÁˆÓÈÛÙÒÓ
∏ Û˘Ì‚·ÙÈ΋ ÁÓÒÛË ÁÈ· ÙÔÓ ·ÓÙÈ·ÛıÌ·ÙÈÎfi
ÚfiÏÔ ÙˆÓ ∂ÈÛ∫™ Î·È ÙˆÓ ‚2-·ÁˆÓÈÛÙÒÓ Ì·ÎÚ¿˜
‰Ú¿Û˘ ·Ó·ÁÓˆÚ›˙ÂÈ ÛÙȘ ‰‡Ô ·˘Ù¤˜ ηÙËÁÔڛ˜
Ô˘ÛÈÒÓ ·Ôχو˜ ‰È·ÎÚÈÙ¤˜ Ê·Ú̷΢ÙÈΤ˜
‰Ú·ÛÙËÚÈfiÙËÙ˜: ·) ∆· ∂ÈÛ∫™ ÂȉÚÔ‡Ó Û ̛·
ÔÈÎÈÏ›· ΢ÙÙ·ÚÔÎÈÓÒÓ Î·È ÌÂÛÔÏ·‚ËÙÒÓ, ÌÂ
419∂ÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Î·È ‚2-·ÁˆÓÈÛÙ¤˜ ÛÙÔ ·È‰ÈÎfi ¿ÛıÌ·
·ÔÙ¤ÏÂÛÌ· ÙËÓ Î·Ù·ÛÙÔÏ‹ Ù˘ ¯ÚfiÓÈ·˜ ÊÏÂÁ-
ÌÔÓ‹˜ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Î·È ÙËÓ ÂÏ¿ÙÙˆÛË Ù˘
‚ÚÔÁ¯È΋˜ ˘ÂÚ··ÓÙËÙÈÎfiÙËÙ·˜. ‚) √È ‚2-
·ÁˆÓÈÛÙ¤˜ Ì·ÎÚ¿˜ ‰Ú¿Û˘ ‰ÈÂÁ›ÚÔ˘Ó ÙÔ˘˜ ‚2-
˘Ô‰Ô¯Â›˜ ÙˆÓ Ï›ˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ ÛÙÔ ÙÔ›¯ˆÌ·
ÙˆÓ ·ÂÚ·ÁˆÁÒÓ, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ¿ÛÎËÛË
‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈ΋˜ ‰Ú¿Û˘ › 12ˆÚÔ ÂÚ›-
Ô˘ (∂ÈÎfiÓ· 1·) (9).
∞˘Í·ÓfiÌÂÓÔ˜ ·ÚÈıÌfi˜ ÌÂÏÂÙÒÓ Û ›‰Ô
‚·ÛÈ΋˜ ¤Ú¢ӷ˜ ¤¯ÂÈ ‰Â›ÍÂÈ fiÙÈ ÔÈ ‚2-·ÁˆÓÈ-
ÛÙ¤˜, ¤Ú·Ó Ù˘ ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈ΋˜, ·ÛÎÔ‡Ó
Î·È ‚ÚÔÁ¯ÔÚÔÛٷ٢ÙÈ΋ ‰Ú¿ÛË. ∂ȉÈÎfiÙÂÚ·,
¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ: ·) ÚÔηÏÔ‡Ó ·Ó·ÛÙÔÏ‹
Ù˘ ·ÔÎÔÎΛˆÛ˘ ÙˆÓ Ì·ÛÙÔ΢ÙÙ¿ÚˆÓ Ô˘
·ÔÙÂÏ› ‚·ÛÈÎfi Ì˯·ÓÈÛÌfi Ù˘ ¿ÌÂÛ˘ ·ÓÙ›-
‰Ú·Û˘ ÛÙÔ ¿ÛıÌ·, ‚) ÂÌÔ‰›˙Ô˘Ó ÙËÓ ÂÍ›‰Úˆ-
ÛË ÙÔ˘ Ï¿ÛÌ·ÙÔ˜ Ô˘ Ô‰ËÁ› Û ԛ‰ËÌ· ÙˆÓ
·ÂÚ·ÁˆÁÒÓ Î·È Á) Â›Ó·È Èı·Ófi Ó· ÂÏ·ÙÙÒÓÔ˘Ó
ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÈÛıËÙÈÎÒÓ Ó¢ÚÈÎÒÓ
ÈÓÒÓ ÛÙÔ˘˜ ‚ÚfiÁ¯Ô˘˜ (∂ÈÎfiÓ· 1‚) (10).
∂ÈϤÔÓ, Ù· ∂ÈÛ∫™ ¢ԉÒÓÔ˘Ó ÙËÓ ¤ÎÊÚ·-
ÛË ÙˆÓ ‚2-˘Ô‰Ô¯¤ˆÓ ÛÙËÓ ÂÈÊ¿ÓÂÈ· ÙˆÓ Î˘Ù-
Ù¿ÚˆÓ Ì¤Ûˆ ·‡ÍËÛ˘ Ù˘ ÌÂÙ·ÁÚ·Ê‹˜ ÙˆÓ
˘Â‡ı˘ÓˆÓ ÁÔÓȉ›ˆÓ. ∏ ‰Ú¿ÛË ÙÔ˘˜ ·˘Ù‹ Ì¿Ï-
ÏÔÓ ÛÙÂÚÂ›Ù·È ÎÏÈÓÈ΋˜ ÛËÌ·Û›·˜ ˆ˜ ÚÔ˜ ÙÔ
‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈÎfi ·ÔÙ¤ÏÂÛÌ· ÙˆÓ ‚2-·ÁˆÓÈ-
ÛÙÒÓ, ÂÓ fi„ÂÈ ÙÔ˘ ÙÂÚ¿ÛÙÈÔ˘ ·Ôı¤Ì·ÙÔ˜ ‚2-
˘Ô‰Ô¯¤ˆÓ ÛÙËÓ Î˘ÙÙ·ÚÈ΋ ÌÂÌ‚Ú¿ÓË ÙˆÓ Ï›-
ˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ Î·È ÂÂȉ‹ Ë ÂΉ‹ÏˆÛË ·ÓÔ¯‹˜
ÙˆÓ ‚2-·ÁˆÓÈÛÙÒÓ ·ÓÙÈÌÂÙˆ›˙ÂÙ·È Â‡ÎÔÏ·
ÛÙËÓ ÎÏÈÓÈ΋ Ú·ÎÙÈ΋ Ì ·‡ÍËÛË Ù˘ ‰fiÛ˘
ÙÔ˘ ‚2-·ÁˆÓÈÛÙ‹. øÛÙfiÛÔ, ÌÔÚ› Ó· ·Ô‚›
ÎÏÈÓÈο ÎÚ›ÛÈÌË Û ·ÙÙ·Ú· Ô˘ ‰È·ı¤ÙÔ˘Ó Â-
ÚÈÔÚÈṲ̂ÓÔ ·ÚÈıÌfi ‚2-˘Ô‰Ô¯¤ˆÓ - fiˆ˜ ›ӷÈ
Ù· Ì·ÛÙÔ·ÙÙ·Ú· - Î·È ÂÌϤÎÔÓÙ·È ¿ÌÂÛ·
ÛÙÔÓ Ì˯·ÓÈÛÌfi ‰È·Ù‹ÚËÛ˘ Ù˘ ‚ÚÔÁ¯ÔÚÔ-
Ûٷ٢ÙÈ΋˜ ÙÔ˘˜ ‰Ú¿Û˘ (10).
√È ‚2-·ÁˆÓÈÛÙ¤˜ Ê·›ÓÂÙ·È fiÙÈ ÂÓÈÛ¯‡Ô˘Ó
ÙÔ˘˜ ÂÓ‰Ô΢ÙÙ¿ÚÈÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ ‰Ú¿Û˘
ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ì ÙËÓ ÚÔ·ÁˆÁ‹ Ù˘
ÂÁηٿÛÙ·Û˘ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ ÙˆÓ ÎÔÚÙÈÎÔ-
ÛÙÂÚÔÂȉÒÓ ÛÙÔÓ ˘Ú‹Ó· ÙÔ˘ ΢ÙÙ¿ÚÔ˘ ÛÙȘ
ı¤ÛÂȘ GRE (·ÏÏËÏÔ˘¯›Â˜ ÁÔÓȉ›ˆÓ ·ÓÙ·fi-
ÎÚÈÛ˘ ÛÙ· ÁÏ˘ÎÔÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹) (10).
™ÙËÓ ∂ÈÎfiÓ· 2 Ê·›ÓÂÙ·È Û¯ËÌ·ÙÈο Ë ·ÏÏËÏÂ-
›‰Ú·ÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ì ÙÔ˘˜ ‚2-
·ÁˆÓÈÛÙ¤˜ Û ΢ÙÙ·ÚÈÎfi ›‰Ô. ÕÏÏË ‰Ú¿ÛË
ÙˆÓ ‚2-·ÁˆÓÈÛÙÒÓ, Èı·ÓfiÓ ÂÈÎÔ˘ÚÈ΋ ÙˆÓ
·ÓÙÈÊÏÂÁÌÔÓˆ‰ÒÓ È‰ÈÔÙ‹ÙˆÓ ÙˆÓ ∂ÈÛ∫™, ›ӷÈ
Ë ÌÂÙÂÁηٿÛÙ·ÛË (translocation) Ù˘ ΢ÙÙ·-
ÚÔÏ·ÛÌ·ÙÈ΋˜ ʈÛÊÔÏÈ¿Û˘ ∞2 (cPLA2)
ÛÙËÓ ˘ÚËÓÈ΋ ÌÂÌ‚Ú¿ÓË. ∏ ÂÓÂÚÁÔÔ›ËÛË
Ù˘ cPLA2 Ô‰ËÁ› ÛÙËÓ ·˘ÍË̤ÓË ÚÔÛÎfiÏÏË-
ÛË ÙˆÓ ËˆÛÈÓÔʛψÓ, ÙfiÛÔ Ì¤Ûˆ ÙÔ˘ PAF (·-
Ú¿ÁÔÓÙ·˜ ÂÓÂÚÁÔÔ›ËÛ˘ ·ÈÌÔÂÙ·Ï›ˆÓ) fiÛÔ
Î·È Ì¤Ûˆ ‰È¤ÁÂÚÛ˘ Ù˘ ·Ú·ÁˆÁ‹˜ IL-5
(ÈÓÙÂÚÏ¢ΛÓË-5) (11).
¶·È‰È·ÙÚÈ΋ 2006;69:417-424
∞ÓÙÈÁfiÓÔ
‚2-·ÁˆÓÈÛÙ¤˜πÔ›?∞‰ÂÓÔÛ›ÓËÕÛÎËÛËÀÁÚ·Û›·
∞ÔÎÔÎΛˆÛË Ì·ÛÔ΢ÙÙ¿ÚˆÓ
µÚÔÁ¯ÔÛ‡Û·ÛË∂Í·ÁÁ›ˆÛË Ï¿ÛÌ·ÙÔ˜
∂ÓÂÚÁÔÔ›ËÛË ·ÈÛıËÙ‹ÚÈˆÓ Ó‡ڈÓ
¢Ú¿ÛË: - µÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈ΋ - µÚÔÁ¯ÔÚÔÛٷ٢ÙÈ΋ - (∞ÓÙÈÊÏÂÁÌÔÓ҉˘;)
∞ÓÙÈÁfiÓÔ
πfi˜?
ÀÂÚ··ÓÙËÙÈÎfiÙËÙ· ·ÂÚ·ÁˆÁÒÓ
ª·ÎÚÔÊ¿Á· ∏ˆÛÈÓfiÊÈÏ·
∆-ÏÂÌÊÔ·ÙÙ·Ú·
∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹
∂ÈÎfiÓ· 1. ∞ıÚÔÈÛÙÈ΋ ‰Ú¿ÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Î·È ‚2-·ÁˆÓÈÛÙÒÓ ÛÙÔ ¿ÛıÌ·: ·) ∏ ·Ó·ÛÙ·ÏÙÈ΋ ‰Ú¿ÛË ÙˆÓ ÎÔÚÙÈ-ÎÔÛÙÂÚÔÂȉÒÓ ÛÙ· ·ÙÙ·Ú· Ô˘ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙËÓ ·ÛıÌ·-ÙÈ΋ ÊÏÂÁÌÔÓ‹. ‚) µÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈ΋ Î·È ‚ÚÔÁ¯ÔÚÔÛÙ·-Ù¢ÙÈ΋ ‰Ú¿ÛË ÙˆÓ ‚2-·ÁˆÓÈÛÙÒÓ [·fi Barnes PJ (10), ÙÚÔ-ÔÔÈË̤ÓÔ].
·
‚
¶˘Ú‹Ó·˜
GREGREGRE
mRNA
∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ¤˜‚2-˘Ô‰Ô¯¤·˜
ÀÔ‰Ô¯¤·˜ ∫™
‚2-·ÁˆÓÈÛÙ‹˜
+PKAPKA
cyclic AMPcyclic AMP
MAPK
°ÔÓ›‰ÈÔ ·¿ÓÙËÛ˘ ∫™
ÚˆÙ½ÓË
∂ÈÎfiÓ· 2. ™˘ÓÂÚÁÈ΋ ‰Ú¿ÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Î·È ‚2-·Áˆ-ÓÈÛÙÒÓ Û ΢ÙÙ·ÚÈÎfi ›‰Ô. ∏ ‰È¤ÁÂÚÛË ÙÔ˘ ‚2-˘Ô‰Ô¯¤··fi ÙÔÓ ‚2-·ÁˆÓÈÛÙ‹ ÚÔηÏ› ·‡ÍËÛË Ù˘ Û˘ÁΤÓÙÚˆÛ˘ÙÔ˘ ÂÓ‰Ô΢ÙÙ¿ÚÈÔ˘ cAMP, Ô˘ Ì ÙË ÛÂÈÚ¿ ÙÔ˘ ›Ù ¿ÌÂÛ·,ÂÓÂÚÁÔÔÈÒÓÙ·˜ ÙËÓ PKA, ›Ù ¤ÌÌÂÛ·, ̤ۈ ÂÓÂÚÁÔÔ›Ë-Û˘ Ù˘ MAPK, ÚÔ¿ÁÂÈ ÙË ÌÂÙÂÁηٿÛÙ·ÛË ÙˆÓ ˘Ô‰Ô¯¤-ˆÓ ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙȘ GRE ÂÚÈÔ¯¤˜ ÙÔ˘ ÁÔÓȉÈÒ-Ì·ÙÔ˜, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÂȉÈÎÒÓ ÁÔÓȉ›ˆÓÎ·È ÙËÓ ·Ú·ÁˆÁ‹ ÚˆÙÂ˚ÓÒÓ Ì ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë ‰Ú¿ÛË.∫™: ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ¤˜, GRE: glucocorticosteroid responseelement, PKA: protein kinase A, MAPK: mitogen-activatedprotein kinases [·fi Barnes PJ (10), ÙÚÔÔÔÈË̤ÓÔ].
420 ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜, ∫. ¡. ¶Ú›ÊÙ˘
∫ÏÈÓÈο ‰Â‰Ô̤ӷ ÁÈ· ÙÔÓ Û˘Ó‰˘·ÛÌfi
∂ÈÛ∫™ Î·È ‚2-·ÁˆÓÈÛÙÒÓ ÛÙ· ·È‰È¿
∏ ÌÔÓÔıÂڷ›· ÙÔ˘ ¿ÛıÌ·ÙÔ˜ Ì ‚2-·Áˆ-
ÓÈÛÙ‹ Ì·ÎÚ¿˜ ‰Ú¿Û˘ ‰ÂÓ ÂӉ›ÎÓ˘Ù·È, ÏfiÁˆ
ÙÔ˘ Êfi‚Ô˘ ·Ó·˙ˆ‡ÚˆÛ˘ Ù˘ ÊÏÂÁÌÔÓ‹˜ ÙˆÓ
·ÂÚ·ÁˆÁÒÓ, ·Ú¿ ÙË Û‡Á¯ÚÔÓË ÎÏÈÓÈ΋ ‚ÂÏÙ›ˆ-
ÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ (12,13). ¶Ú¿ÁÌ·ÙÈ, ÔÈ
Verberne Î·È Û˘Ó ¤‰ÂÈÍ·Ó fiÙÈ Ë ÌÔÓÔıÂڷ›·
·ÛıÌ·ÙÈÎÒÓ ·È‰ÈÒÓ Ì ۷ÏÌÂÙÂÚfiÏË ÁÈ· ¤Ó·
¤ÙÔ˜ Ô‰‹ÁËÛ ÌÂÓ Û ‚ÂÏÙ›ˆÛË ÙˆÓ Û˘Ìو̿-
ÙˆÓ, fï˜ Ë ÎÏÈÓÈ΋ ‚ÂÏÙ›ˆÛË ˘ÔÏÂÈfiÙ·Ó ÛÂ
Û‡ÁÎÚÈÛË Ì ÙË ¯ÔÚ‹ÁËÛË ÌÔÓÔıÂڷ›·˜ ÌÂ
ÌÂÎÏÔÌÂı·˙fiÓË (200 Ìg x 2). ∞ÎfiÌË ÈÔ ·ÓË-
Û˘¯ËÙÈÎfi ‹Ù·Ó ÙÔ Â‡ÚËÌ· Ù˘ ÌÂϤÙ˘ fiÙÈ Ë
‚ÚÔÁ¯ÔÛÙ¤ÓˆÛË Î·È Ë ‚ÚÔÁ¯È΋ ˘ÂÚ··ÓÙËÙÈ-
ÎfiÙËÙ· ÂȉÂÈÓÒıËÎ·Ó Û ۯ¤ÛË Ì ÙË ‚·ÛÈ΋
ÙÈÌ‹ ÛÙËÓ ÔÌ¿‰· Ù˘ Û·ÏÌÂÙÂÚfiÏ˘, ÂÓÒ ·ÓÙ›-
ıÂÙ· ·ÚÔ˘Û›·Û·Ó ‚ÂÏÙ›ˆÛË ÛÙËÓ ÔÌ¿‰· Ù˘
ÌÂÎÏÔÌÂı·˙fiÓ˘ (∂ÈÎfiÓ· 3) (14). ∆· Â˘Ú‹Ì·-
Ù· ·˘Ù¿, ηıÒ˜ Î·È ¿ÏÏ· (15), ÂÓÈÛ¯‡Ô˘Ó ÙËÓ
¿Ô„Ë fiÙÈ Ë ¯ÚfiÓÈ· ¯ÔÚ‹ÁËÛË ÙˆÓ ‚2-·ÁˆÓÈ-
ÛÙÒÓ Ì·ÎÚ¿˜ ‰Ú¿Û˘ Ô‰ËÁ› ÛÙËÓ ÂÌÊ¿ÓÈÛË
ÙÔ˘ Ê·ÈÓÔ̤ÓÔ˘ Ù˘ ·ÓÔ¯‹˜ ÛÙËÓ ÂΉ‹ÏˆÛË
Ù˘ ‚ÚÔÁ¯ÔÚÔÛٷ٢ÙÈ΋˜ ‰Ú¿Û˘ ÙÔ˘˜.
∂ÓÙÔ‡ÙÔȘ, ·ÔÙÂϤÛÌ·Ù· ¿ÏÏˆÓ ÌÂÏÂÙÒÓ Û˘-
ÓËÁÔÚÔ‡Ó ÁÈ· ÙÔ ·ÓÙ›ıÂÙÔ (16).
ªÈ· ÛÂÈÚ¿ ·fi ηϿ ۯ‰ȷṲ̂Ó˜ ÎÏÈÓÈΤ˜
ÌÂϤÙ˜ Û ÂÓ‹ÏÈΘ ·ÛıÌ·ÙÈÎÔ‡˜ ·ÛıÂÓ›˜
¤¯ÂÈ ‰Â›ÍÂÈ fiÙÈ Ë ÚÔÛı‹ÎË ‚2-·ÁˆÓÈÛÙ‹ Ì·-
ÎÚ¿˜ ‰Ú¿Û˘ Û ¯·ÌËÏ‹ ‹ ̤ÙÚÈ· ‰fiÛË ∂ÈÛ∫™
‚ÂÏÙÈÒÓÂÈ ÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ·ÛıÌ·ÙÈÎÒÓ Û˘-
ÌÙˆÌ¿ÙˆÓ Î·È Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜
Î·È ÂÏ·ÙÙÒÓÂÈ ÙÔ˘˜ ·ÚÔ͢ÛÌÔ‡˜ Ù˘ ÓfiÛÔ˘
(3-5,17,18), ÂÓÒ Ô Û˘Ó‰˘·ÛÌfi˜ ÙˆÓ ‰‡Ô ηÙË-
ÁÔÚÈÒÓ Ê·ÚÌ¿ÎˆÓ Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚÔ˜
Ù˘ ÌÔÓÔıÂڷ›·˜ Ì ‰ÈÏ¿ÛÈ· ‰fiÛË ∂ÈÛ∫™ÛÙÔÓ Ì·ÎÚÔ¯ÚfiÓÈÔ ¤ÏÂÁ¯Ô Ù˘ ÓfiÛÔ˘ (3,4).º·›ÓÂÙ·È fiÙÈ Ë ‚ÂÏÙ›ˆÛË ÙˆÓ Û˘Ìو̿وÓÔ˘ ÚÔ·ÙÂÈ ·fi ÙÔÓ Û˘Ó‰˘·ÛÌfi ‚2-·ÁˆÓÈ-ÛÙ‹ Ì·ÎÚ¿˜ ‰Ú¿Û˘ Ì ¯·ÌËÏ‹ ‰fiÛË ∂ÈÛ∫™ ‰ÂÓ“Û˘ÁηχÙÂÈ” ÙËÓ ‡·ÚÍË Â›ÌÔÓ˘ ‹ ÂȉÂÈ-ÓÔ‡ÌÂÓ˘ ·ÛıÌ·ÙÈ΋˜ ÊÏÂÁÌÔÓ‹˜ (19). ∂Ô̤-Óˆ˜, Ô ¤ÏÂÁ¯Ô˜ Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ ÌÂ Û˘Ó‰˘·-Ṳ̂ÓË ıÂڷ›· ‰ÂÓ ·ÔÚÔÛ·Ó·ÙÔÏ›˙ÂÈ ÙÔÓÎÏÈÓÈÎfi ÁÈ·ÙÚfi, ÒÛÙ ӷ ˘ÂÚÙÈÌ‹ÛÂÈ ÙÔÓ ¤ÏÂÁ-¯Ô Ù˘ ÓfiÛÔ˘ Î·È Ó· ˘ÔÙÈÌ‹ÛÂÈ ÙȘ Ú·ÁÌ·ÙÈ-Τ˜ ·Ó¿ÁΘ ÙÔ˘ ·ÛıÌ·ÙÈÎÔ‡ ·ÛıÂÓÔ‡˜ Û ·ÓÙÈ-ÊÏÂÁÌÔÓÒ‰Ë ·ÁˆÁ‹ (20).
√ ·ÚÈıÌfi˜ ÙˆÓ Ù˘¯·ÈÔÔÈËÌ¤ÓˆÓ ‰ÈÏÒÓ-Ù˘ÊÏÒÓ ÎÏÈÓÈÎÒÓ ÌÂÏÂÙÒÓ Ô˘ ‰ÈÂÚ¢ÓÔ‡Ó ÙËÓ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ Û˘Ó‰˘·˙fiÌÂÓ˘ ıÂ-ڷ›·˜ ∂ÈÛ∫™ Î·È ‚2-·ÁˆÓÈÛÙÒÓ Ì·ÎÚ¿˜ ‰Ú¿-Û˘ ÛÙ· ·È‰È¿ Â›Ó·È Ì¿ÏÏÔÓ ÂÚÈÔÚÈṲ̂ÓÔ˜ ηȷÊÔÚ¿ Û ËÏÈ˘ ¿Óˆ ÙˆÓ 4-6 ÂÙÒÓ (21-28).™¯Â‰fiÓ fiϘ ‰Â›¯ÓÔ˘Ó ‰È·ÊfiÚÔ˘ ‚·ıÌÔ‡ ˘Â-ÚÔ¯‹ Ù˘ Û˘Ó‰˘·˙fiÌÂÓ˘ ıÂڷ›·˜ ˆ˜ ÚÔ˜ÙÔ˘˜ ‰Â›ÎÙ˜ ÂϤÁ¯Ô˘ Ù˘ ¯ÚfiÓÈ·˜ ÓfiÛÔ˘ [‚·ı-ÌÔÏÔÁ›· Û˘Ìو̿وÓ, Ë̤Ú˜ ÂχıÂÚ˜ Û˘-Ìو̿وÓ, ¯Ú‹ÛË ·Ó·ÎÔ˘ÊÈÛÙÈ΋˜ ıÂڷ›-·˜, FEV1 (‚›·È· ÂÎÓÂfiÌÂÓÔ˜ fiÁÎÔ˜ ÛÙÔ ÚÒÙԉ¢ÙÂÚfiÏÂÙÔ), ÚˆÈÓ‹ Î·È ‚Ú·‰ÈÓ‹ PEF (̤ÁÈ-ÛÙË ÂÎÓ¢ÛÙÈ΋ ÚÔ‹), ÌÂÙ·‚ÏËÙfiÙËÙ· PEF,˘ÂÚ··ÓÙËÙÈÎfiÙËÙ·] Û ۯ¤ÛË Ì ÙË ÌÔÓÔıÂ-ڷ›· Ì ∂ÈÛ∫™ (∂ÈÎfiÓ˜ 4 Î·È 5) (21-27).øÛÙfiÛÔ, Ë ÌÂϤÙË ÙˆÓ Verberne Î·È Û˘Ó (28)‰ÂÓ ·Ó·‰ÂÈÎÓ‡ÂÈ ˘ÂÚÔ¯‹ Ù˘ Û˘Ó‰˘·˙fiÌÂÓ˘ıÂڷ›·˜ ¤Ó·ÓÙÈ Ù˘ ÌÔÓÔıÂڷ›·˜ Ì ‰‡Ô
¶·È‰È·ÙÚÈ΋ 2006;69:417-424
20
15
10
5
0
-5
-10
-15
ªÂÙ
·‚
ÔÏ
‹ F
EV
1(%
)
ªÂÎÏÔÌÂı·˙fiÓË™·ÏÌÂÙÂÚfiÏË
6 12 18 24 30 36 42 48 54¢È¿ÚÎÂÈ· ıÂڷ›·˜
(‚‰ÔÌ¿‰Â˜) ∂·Ó¤ÏÂÁ¯Ô˜
∂ÈÎfiÓ· 3. ªÂÙ·‚ÔÏ‹ ÙÔ˘ FEV1 ·ÛıÌ·ÙÈÎÒÓ ·È‰ÈÒÓ Î·Ù¿ÙË ‰È¿ÚÎÂÈ· ÌÔÓÔıÂڷ›·˜ Ì ۷ÏÌÂÙÂÚfiÏË ¤Ó·ÓÙÈ ÌÂ-ÎÏÔÌÂı·˙fiÓ˘ (200 Ìg x 2) › ¤Ó· ¤ÙÔ˜. ∏ ÌÔÓÔıÂڷ›·Ì ۷ÏÌÂÙÂÚfiÏË ÚÔηÏ› Âȉ›ӈÛË Ù˘ ‚ÚÔÁ¯ÔÛÙ¤Óˆ-Û˘ (·fi ÙË ‰ÈÏ‹ Ù˘ÊÏ‹ Ù˘¯·ÈÔÔÈË̤ÓË ÌÂϤÙË Û 67·È‰È¿ ËÏÈΛ·˜ 6-16 ÂÙÒÓ ÙˆÓ Verberne AA Î·È Û˘Ó (14),ÙÚÔÔÔÈË̤ÓÔ).
60
50
40
30
20
10
0
(p<0,001)
(p=0,043)(p=0,008)
™·ÏÌÂÙÂÚfiÏË (50 Ìg x 2)∂ÈÎÔÓÈÎfi Ê¿ÚÌ·ÎÔ
∆ÈÌ‹·Ó·ÊÔÚ¿˜
1-4 ‚‰ÔÌ¿‰Â˜
5-8‚‰ÔÌ¿‰Â˜
9-12‚‰ÔÌ¿‰Â˜
¢È¿
ÌÂÛ
Ë Ù
ÈÌ‹
ËÌ
ÂÚÒ
Ó ¯ˆ
Ú›˜
Û˘
Ì
ÙÒÌ
·Ù·
(%
)
∂ÈÎfiÓ· 4. ¶ÔÛÔÛÙfi ËÌÂÚÒÓ ¯ˆÚ›˜ Û˘ÌÙÒÌ·Ù· (‰È¿ÌÂÛËÙÈÌ‹) Û ·È‰È¿ Ì ̤ÙÚÈÔ ‹ ÛÔ‚·Úfi ¿ÛıÌ· ˘fi Ì·ÎÚÔ¯Úfi-ÓÈ· ·ÁˆÁ‹ Ì ÙÔ˘Ï¿¯ÈÛÙÔÓ 400 Ìg ÌÂÎÏÔÌÂı·˙fiÓ˘ ËÌÂ-ÚËÛ›ˆ˜ ‹ ÈÛÔ‰‡Ó·Ì˘ ‰fiÛ˘ ¿ÏÏÔ˘ ∂ÈÛ∫™, Ô˘ ¤Ï·‚·ÓÛ·ÏÌÂÙÂÚfiÏË ¤Ó·ÓÙÈ ÂÈÎÔÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ › 12 ‚‰ÔÌ¿-‰Â˜ (·fi ÙË ‰ÈÏ‹-Ù˘ÊÏ‹ Ù˘¯·ÈÔÔÈË̤ÓË ÌÂϤÙË Û 210·È‰È¿ ËÏÈΛ·˜ 4-16 ÂÙÒÓ ÙˆÓ Russell G Î·È Û˘Ó (22), ÙÚÔ-ÔÔÈË̤ÓÔ).
‰È·ÊÔÚÂÙÈΤ˜ ‰fiÛÂȘ ∂ÈÛ∫™. ¶ÚfiÎÂÈÙ·È ÁÈ· ‰È-
Ï‹-Ù˘ÊÏ‹ ÌÂϤÙË, ÂÏÂÁ¯fiÌÂÓË Ì ÂÈÎÔÓÈÎfi
Ê¿ÚÌ·ÎÔ, Ô˘ ÂÚȤϷ‚ 177 ·È‰È¿ ËÏÈΛ·˜ 6-
16 ÂÙÒÓ, Ù· ÔÔ›· ¯ˆÚ›ÛÙËÎ·Ó Û ÙÚÂȘ ÔÌ¿‰Â˜
(ÔÌ¿‰· ∞: ÌÂÎÏÔÌÂı·˙fiÓË 200 Ìg x 2 Î·È Û·Ï-
ÌÂÙÂÚfiÏË 50 Ìg x 2, ÔÌ¿‰· µ: ÌÂÎÏÔÌÂı·˙fiÓË
400 Ìg x 2, ÔÌ¿‰· °: ÌÂÎÏÔÌÂı·˙fiÓË 200 Ìg x
2) Î·È ·Ú·ÎÔÏÔ˘ı‹ıËÎ·Ó ÁÈ· 12 Ì‹Ó˜. ¢ÂÓ
‚Ú¤ıËÎ·Ó ‰È·ÊÔÚ¤˜ ÛÙÔÓ FEV1, ÙËÓ PD20 (‰fiÛË
ÚfiÎÏËÛ˘ ÙÒÛ˘ ÙÔ˘ FEV1 ηٿ 20%) ÌÂÙ¿
ÚfiÎÏËÛË Ì ÌÂÙ·¯ÔÏ›ÓË, ÛÙË ‚·ıÌÔÏÔÁ›· ÙˆÓ
Û˘ÌÙˆÌ¿ÙˆÓ Î·È ÛÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ·ÚÔ͢-
ÛÌÒÓ ÌÂٷ͇ ÙˆÓ ÙÚÈÒÓ ÔÌ¿‰ˆÓ. À‹ÚÍ·Ó Ìfi-
ÓÔ Î¿ÔȘ ÌÈÎÚ¤˜ ‰È·ÊÔÚ¤˜ ÛÙËÓ ÚˆÈÓ‹ ηÈ
‚Ú·‰ÈÓ‹ PEF ˘¤Ú Ù˘ ÔÌ¿‰·˜ Ô˘ Ï¿Ì‚·ÓÂ
Û·ÏÌÂÙÂÚfiÏË Î·Ù¿ ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ ıÂÚ·-
›·˜. ™ËÌÂÈÒÓÂÙ·È fiÙÈ ÛÙËÓ ÔÌ¿‰· Ô˘ ¤Ï·‚Â
˘„ËÏ‹ ‰fiÛË ÌÂÎÏÔÌÂı·˙fiÓ˘ ·Ú·ÙËÚ‹ıË-
Î·Ó ¯·ÌËÏfiÙÂÚÔÈ Ú˘ıÌÔ› ·‡ÍËÛ˘.
∞fi ÙÔ 1998 Ô Û˘Ó‰˘·ÛÌfi˜ Û·ÏÌÂÙÂÚfi-
Ï˘/ÊÏÔ˘ÙÈη˙fiÓ˘ Î·È ·fi ÙÔ 2000 Ô Û˘Ó‰˘·-
ÛÌfi˜ ‚Ô˘‰ÂÛÔÓ›‰Ë˜/ÊÔÚÌÔÙÂÚfiÏ˘ ÌÔÚÔ‡Ó
Ó· Û˘Á¯ÔÚËÁËıÔ‡Ó, ηı¤Ó·˜ Ì ÂÓÈ·›· Û˘-
Û΢‹. Œ¯ÂÈ ‰Âȯı›, Ì¿ÏÈÛÙ·, fiÙÈ Ë Û˘Á¯ÔÚ‹ÁË-
ÛË ·˘Ù‹ ÛÙ· ·È‰È¿, fiˆ˜ Î·È ÛÙÔ˘˜ ÂÓ‹ÏÈΘ,
Â›Ó·È ÙÔ˘Ï¿¯ÈÛÙÔÓ ÂÍ›ÛÔ˘ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ηÈ
·ÛÊ·Ï‹˜ Ì ÙË ‰È·‰Ô¯È΋ ¯ÔÚ‹ÁËÛË ÙˆÓ Ê·Ú-
Ì¿ÎˆÓ ÙÔ˘ οıÂ Û˘Ó‰˘·ÛÌÔ‡ ̠ͯˆÚÈÛÙ¤˜
Û˘Û΢¤˜ (29-31). √ÚÈṲ̂ÓÔÈ Ì¿ÏÈÛÙ· ˘ÔÛÙË-
Ú›˙Ô˘Ó, Û ıˆÚËÙÈ΋ ΢ڛˆ˜ ‚¿ÛË, fiÙÈ ˘¿Ú-
¯ÂÈ Û˘Ó¤ÚÁÂÈ· ÙˆÓ ‰‡Ô ηÙËÁÔÚÈÒÓ Ê·Ú̿ΈÓ
fiÙ·Ó ·˘Ù¿ Û˘Á¯ÔÚËÁÔ‡ÓÙ·È Û ̛· ÂÈÛÓÔ‹
(32). ™ËÌÂÈÒÓÂÙ·È fiÙÈ ·fi ÙȘ ÚÔ·Ó·ÊÂÚı›-
Û˜ ÌÂϤÙ˜ Ù˘ Û˘Ó‰˘·˙fiÌÂÓ˘ ıÂڷ›·˜ ÛÂ
·È‰È·ÙÚÈÎÔ‡˜ ·ÛıÂÓ›˜, ÌfiÓÔ Û ÂΛÓË ÙˆÓ
Tal Î·È Û˘Ó (26) ¯ÚËÛÈÌÔÔÈ‹ıËΠÂÓÈ·›· Û˘-
Û΢‹.
∆¤ÏÔ˜, ·Ó Î·È Ù· ‰Â‰Ô̤ӷ ÁÈ· ÙËÓ Â›‰Ú·-
ÛË Ù˘ Ù·˘Ùfi¯ÚÔÓ˘ ¯ÔÚ‹ÁËÛ˘ ∂ÈÛ∫™ Î·È ‚2-
·ÁˆÓÈÛÙ‹ Ì·ÎÚ¿˜ ‰Ú¿Û˘ Ì ÂÓÈ·›· Û˘Û΢‹
ÛÙË “Û˘ÌÌfiÚʈÛË” ÙˆÓ ·ÛıÌ·ÙÈÎÒÓ ·ÛıÂÓÒÓ
Â›Ó·È ÂÏ¿¯ÈÛÙ·, Ê·›ÓÂÙ·È fiÙÈ ·˘Ù‹ ‚ÂÏÙÈÒÓÂÈ ÙË
Û˘ÌÌfiÚʈÛË (33).
∏ ›Ù¢ÍË È‰·ÓÈÎÔ‡ ÂϤÁ¯Ô˘ ÙÔ˘
¿ÛıÌ·ÙÔ˜
√ Ï‹Ú˘ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜, fiˆ˜ ·˘-
Ùfi˜ ÔÚ›˙ÂÙ·È ÛÙȘ ÙÚ¤¯Ô˘Û˜ ÔÌÔʈӛ˜ (2,6,7)
‰ÂÓ Â›Ó·È ÂÊÈÎÙfi˜ Û ·ÚÎÂÙÔ‡˜ ·ÛıÂÓ›˜. ™Â Úfi-
ÛÊ·ÙË ÌÂÁ¿ÏË 12ÌËÓË ÌÂϤÙË ÙˆÓ Bateman ηÈ
Û˘Ó (34) Û ÂÓ‹ÏÈΘ ·ÛıÌ·ÙÈÎÔ‡˜ (ÂÚÈÛÛfiÙÂ-
ÚÔÈ ·fi 3000 ·ÛıÂÓ›˜) ÂȯÂÈÚ‹ıËΠӷ ÂÈÙ¢-
¯ı› Ô ‚¤ÏÙÈÛÙÔ˜ - Î·È ÂÊfiÛÔÓ ‹Ù·Ó ‰˘Ó·Ùfi - Ô
Ï‹Ú˘ ¤ÏÂÁ¯Ô˜ Ù˘ ÓfiÛÔ˘ Ì ÚÔԉ¢ÙÈ΋ ·‡-
ÍËÛË Ù˘ ‰fiÛ˘ ÙÔ˘ ÂÈÛÓÂfiÌÂÓÔ˘ Ê·ÚÌ¿ÎÔ˘
Ú‡ıÌÈÛ˘. ∏ ÌÂϤÙË ·˘Ù‹ ¤‰ÂÈÍ fiÙÈ Ë Û˘Á¯Ô-
Ú‹ÁËÛË Û·ÏÌÂÙÂÚfiÏ˘/ÊÏÔ˘ÙÈη˙fiÓ˘ Ì ÙËÓ
›‰È· Û˘Û΢‹ ÂÈÙ˘Á¯¿ÓÂÈ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂ-
ÚÔ ¤ÏÂÁ¯Ô Ù˘ ÓfiÛÔ˘ Û ۇÁÎÚÈÛË Ì ÙË ¯ÔÚ‹-
ÁËÛË ÌfiÓ˘ Ù˘ ÊÏÔ˘ÙÈη˙fiÓ˘, ¯ÚËÛÈÌÔÔÈÒ-
ÓÙ·˜ Ì¿ÏÈÛÙ· ÌÈÎÚfiÙÂÚË ËÌÂÚ‹ÛÈ· ‰fiÛË ∂ÈÛ∫™.
¶·Ú’ fiÏ· ·˘Ù¿, ÔÚÈṲ̂ÓÔÈ ·ÛıÂÓ›˜ ¯ÚÂÈ¿ÛÙËÎÂ
Ó· Ï¿‚Ô˘Ó ˘„ËϤ˜ ‰fiÛÂȘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉԇ˜
ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÏÂÁ¯ı› Ë ÓfiÛÔ˜ ÙÔ˘˜ (34).
¶·ÚfiÌÔÈ· ÚÔÛ¤ÁÁÈÛË ‰ÂÓ ¤¯ÂÈ ÌÂÏÂÙËı› ÚÔ˜
ÙÔ ·ÚfiÓ ÛÙ· ·È‰È¿.
™Â ¿ÏÏË ÌÂÁ¿ÏË 12ÌËÓË ÌÂϤÙË, ÙˆÓ O’Byrne
Î·È Û˘Ó (ÂÚ›Ô˘ 2700 ·ÛıÂÓ›˜) (35), ¤ÁÈÓÂ
ÚÔÛ¿ıÂÈ· Ó· ·ÍÈÔÔÈËı› Ë ‰˘Ó·ÙfiÙËÙ· Ù˘
ÊÔÚÌÔÙÂÚfiÏ˘ Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ˆ˜ ·Ó·ÎÔ˘-
ÊÈÛÙÈÎfi ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈÎfi Ê¿ÚÌ·ÎÔ ÏfiÁˆ Ù˘
Ù·¯Â›·˜ ¤Ó·Ú͢ ‰Ú¿Û˘ Ù˘ (36,37). ™ÙË ÌÂϤ-
ÙË ·˘Ù‹ Û˘ÁÎÚ›ıËÎÂ Ë Û˘Á¯ÔÚ‹ÁËÛË ÙÔ˘ Û˘Ó-
‰˘·ÛÌÔ‡ ‚Ô˘‰ÂÛÔÓ›‰Ë˜/ÊÔÚÌÔÙÂÚfiÏ˘ (80/4,5
Ìg x 2) ÛÙËÓ ›‰È· Û˘Û΢‹ ˆ˜ ıÂڷ›· Û˘ÓÙ‹-
ÚËÛ˘, ·ÏÏ¿ Î·È ˆ˜ ·Ó·ÎÔ˘ÊÈÛÙÈ΋ ıÂڷ›· (Ë
›‰È· ‰fiÛË ˆ˜ ηْ ›ÎÏËÛÈÓ ÂÈÛÓÔ¤˜) Ì ‰‡Ô
Û¯‹Ì·Ù· “ÎÏ·ÛÈ΋˜” ÚÔÊ˘Ï·ÎÙÈ΋˜ ¯ÔÚ‹ÁË-
Û˘ (80/4,5 Ìg x 2 Î·È 320/4,5 Ìg x 2) Î·È ¯Ú‹ÛË
‚2-·ÁˆÓÈÛÙ‹ ‚Ú·¯Â›·˜ ‰Ú¿Û˘ ÂÊfiÛÔÓ ¯ÚÂÈ·˙fi-
Ù·Ó ÁÈ· ·Ó·ÎÔ‡ÊÈÛË. √ ¤ÏÂÁ¯Ô˜ Ù˘ ÓfiÛÔ˘ ˆ˜
ÚÔ˜ ÙÔ˘˜ ·ÛıÌ·ÙÈÎÔ‡˜ ·ÚÔ͢ÛÌÔ‡˜, Ù· Û˘-
ÌÙÒÌ·Ù· Î·È ÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· ‹Ù·Ó
ÏËÚ¤ÛÙÂÚÔ˜ ÛÙËÓ ÔÌ¿‰· Ô˘ ¤Ï·‚ ÙÔÓ Û˘Ó-
‰˘·ÛÌfi ˆ˜ ÚÔÊ˘Ï·ÎÙÈ΋, ·ÏÏ¿ Î·È ˆ˜ ·Ó·ÎÔ˘-
ÊÈÛÙÈ΋ ıÂڷ›· Û ۇÁÎÚÈÛË Ì ÙȘ ¿ÏϘ ‰‡Ô
421∂ÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Î·È ‚2-·ÁˆÓÈÛÙ¤˜ ÛÙÔ ·È‰ÈÎfi ¿ÛıÌ·
¶·È‰È·ÙÚÈ΋ 2006;69:417-424
FE
V1(
%)
0 4 8 12
£Âڷ›· (‚‰ÔÌ¿‰Â˜)
p<0,05
µÔ˘‰ÂÛÔÓ›‰Ë 160 Ìg x 2 ∫∞π ÊÔÚÌÔÙÂÚfiÏË 9 Ìg x 2µÔ˘‰ÂÛÔÓ›‰Ë 200 Ìg x 2120
115
110
105
100
∂ÈÎfiÓ· 5. √ FEV1 ‹Ù·Ó ˘„ËÏfiÙÂÚÔ˜ ÛÙËÓ ÔÌ¿‰· ÙˆÓ ·È-‰ÈÒÓ Ô˘ ¤Ï·‚Â Û˘Ó‰˘·˙fiÌÂÓË ıÂڷ›· Ì ‚Ô˘‰ÂÛÔÓ›‰Ë(160 Ìg x 2) Î·È ÊÔÚÌÔÙÂÚfiÏË (9 Ìg x 2) ¤Ó·ÓÙÈ Ù˘ ÔÌ¿‰·˜Ô˘ ¤Ï·‚ ÌÔÓÔıÂڷ›· Ì ›‰È· ‰fiÛË ‚Ô˘‰ÂÛÔÓ›‰Ë˜ (·fiÙË ‰ÈÏ‹-Ù˘ÊÏ‹ Ù˘¯·ÈÔÔÈË̤ÓË ÌÂϤÙË 12 ‚‰ÔÌ¿‰ˆÓ ÛÂ286 ·ÛıÌ·ÙÈο ·È‰È¿ ËÏÈΛ·˜ 4-17 ÂÙÒÓ ÙˆÓ Tal A Î·È Û˘Ó(26), ÙÚÔÔÔÈË̤ÓÔ).
422 ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜, ∫. ¡. ¶Ú›ÊÙ˘
ÔÌ¿‰Â˜. ¢ÂÓ ·Ú·ÙËÚ‹ıËΠηٿ¯ÚËÛË Ê·ÚÌ¿-
ÎÔ˘ (35).
∆· ıÂÙÈο ·ÔÙÂϤÛÌ·Ù· Ù˘ “ÌË Û˘Ì‚·ÙÈ-
΋˜” Û˘Á¯ÔÚ‹ÁËÛ˘ ‚Ô˘‰ÂÛÔÓ›‰Ë˜/ÊÔÚÌÔÙÂ-
ÚfiÏ˘ Ì ÎÔÈÓ‹ Û˘Û΢‹, ÙfiÛÔ ÁÈ· ÙË Ì·ÎÚÔ-
¯ÚfiÓÈ· ÚÔʇϷÍË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ fiÛÔ Î·È ÁÈ·
ÙËÓ Î·Ù’ ›ÎÏËÛÈÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Û˘ÌÙˆ-
Ì¿ÙˆÓ Ù˘ ÓfiÛÔ˘, Á›ÓÔÓÙ·È Î·Ù·ÓÔËÙ¿ Â¿Ó Ï¿-
‚ÂÈ Î·Ó›˜ ˘’ fi„ÈÓ Ù· ·ÎfiÏÔ˘ı·:
·) √È ·ÚÔ͢ÛÌÔ› Ù˘ ÓfiÛÔ˘ ÂΉËÏÒÓÔÓÙ·È
Û˘Ó‹ıˆ˜ ÂÓÙfi˜ ‰È·ÛÙ‹Ì·ÙÔ˜ ÌÂÚÈÎÒÓ ÌfiÓÔ ËÌÂ-
ÚÒÓ (38), ‰›ÓÔÓÙ·˜ ¤ÙÛÈ ÛÙÔÓ ·ÛıÂÓ‹ ÙËÓ Â˘Î·È-
Ú›· Ó· ÍÂÎÈÓ‹ÛÂÈ ¤ÁηÈÚ· ÙËÓ “ÂÓÈÛ¯˘Ì¤ÓË” ·Ó·-
ÎÔ˘ÊÈÛÙÈ΋ ıÂڷ›· Ì ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ¤˜.
‚) ∆fiÛÔ Ë ‚Ô˘‰ÂÛÔÓ›‰Ë (39) fiÛÔ Î·È Ë
ÊÏÔ˘ÙÈη˙fiÓË (40) ÂΉËÏÒÓÔ˘Ó ÙËÓ ·ÓÙÈÊÏÂÁ-
ÌÔÓÒ‰Ë ‰Ú¿ÛË ÙÔ˘˜ Ôχ ÓˆÚ›ÙÂÚ· ·fi fi,ÙÈ
ıˆÚ›ÙÔ ·Ï·ÈfiÙÂÚ·.
∞Ó Î·È Ë ÌÂϤÙË ÙˆÓ O’Byrne Î·È Û˘Ó (35)
ÂÚÈÂÏ¿Ì‚·ÓÂ Î·È 341 ·È‰È¿ ËÏÈΛ·˜ 4-11 ÂÙÒÓ
(12% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ·ÛıÂÓÒÓ), Ù· ·ÔÙÂϤ-
ÛÌ·Ù· ÁÈ’ ·˘Ù‹ ÙËÓ ËÏÈÎȷ΋ ÔÌ¿‰· ‰ÂÓ ¤¯Ô˘Ó
Á›ÓÂÈ ÚÔ˜ ÙÔ ·ÚfiÓ ÁÓˆÛÙ¿.
∆· Ôχ ÂӉȷʤÚÔÓÙ· Â˘Ú‹Ì·Ù· ÙˆÓ ‰‡Ô
·Ú·¿Óˆ ÌÂÏÂÙÒÓ Ú¤ÂÈ Ó· ÂȂ‚·ÈˆıÔ‡Ó
Û ÌÂÁ·Ï‡ÙÂÚÔ˘˜ ÏËı˘ÛÌÔ‡˜, ÂÓÒ ‰ÂÓ ¤¯ÂÈ
ÚÔ˜ ÙÔ ·ÚfiÓ ·Ô‰Âȯı› Ë ÈÛ¯‡˜ ÙÔ˘˜ Î·È ÛÙ·
·È‰È¿.
™˘ÌÂÚ¿ÛÌ·Ù·
Èڛ˜ ·ÌÊÈ‚ÔÏ›·, Ù· ̤¯ÚÈ Û‹ÌÂÚ· ÂÈÛÙË-
ÌÔÓÈο ‰Â‰Ô̤ӷ ·Ó·‰ÂÈÎÓ‡Ô˘Ó ÙÔÓ ·ıÚÔÈÛÙÈÎfi
ÚfiÏÔ ÙÔ˘ Û˘Ó‰˘·ÛÌÔ‡ ∂ÈÛ∫™ Î·È ‚2-·ÁˆÓÈÛÙÒÓ
Ì·ÎÚ¿˜ ‰Ú¿Û˘ ÛÙÔÓ Ì·ÎÚÔ¯ÚfiÓÈÔ ¤ÏÂÁ¯Ô ÙÔ˘
¿ÛıÌ·ÙÔ˜, ÂÓÒ ÔÈ ÚfiÔ‰ÔÈ ÛÙË ‚·ÛÈ΋ ¤Ú¢ӷ
ÔÏÔ¤Ó· Î·È ÂÚÈÛÛfiÙÂÚÔ ÊˆÙ›˙Ô˘Ó ÙË Û˘ÓÂÚÁÈ-
΋ ‰Ú¿ÛË ÙˆÓ ‰‡Ô ·˘ÙÒÓ Î·ÙËÁÔÚÈÒÓ ·ÓÙÈ·-
ÛıÌ·ÙÈÎÒÓ Ê·ÚÌ¿ÎˆÓ Û ΢ÙÙ·ÚÈÎfi ›‰Ô
(41). øÛÙfiÛÔ, Ë Ì·ÎÚÔ¯ÚfiÓÈ· ÌÔÓÔıÂڷ›· ÌÂ
‚2-·ÁˆÓÈÛÙ‹ Ì·ÎÚ¿˜ ‰Ú¿Û˘ ÌÔÚ› Ó· Ô‰ËÁ‹-
ÛÂÈ Û Âȉ›ӈÛË ÙÔ˘ ¿ÛıÌ·ÙÔ˜ ηÈ, Û‡Ìʈӷ
Ì ÙȘ ÚfiÛÊ·Ù˜ ÔÌÔʈӛ˜, ‰ÂÓ ¤¯ÂÈ ı¤ÛË
ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÓfiÛÔ˘ (14,42).
™ÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË, Ô Û˘Ó‰˘·ÛÌfi˜ ∂ÈÛ∫™
Î·È ‚2-·ÁˆÓÈÛÙ‹ Ì·ÎÚ¿˜ ‰Ú¿Û˘ ¯ÔÚËÁ›ٷÈ
ÛÙÔ Ì¤ÙÚÈÔ Î·È ÛÔ‚·Úfi (›ÌÔÓÔ) ¿ÛıÌ· ηÈ
·ÔÙÂÏ› ÙË ıÂڷ›· ÂÎÏÔÁ‹˜ ÛÙÔ ‹ÈÔ Â›ÌÔ-
ÓÔ ·È‰ÈÎfi ¿ÛıÌ· Ô˘ ‰ÂÓ ÂϤÁ¯ÂÙ·È Ì ¯·ÌË-
Ϥ˜ ‰fiÛÂȘ ∂ÈÛ∫™, ÚˆÙ›ÛÙˆ˜ ·È‰ÈÒÓ ËÏÈΛ·˜
ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 5 ÂÙÒÓ (2,6-8,43). ∏ Û˘Á¯Ô-
Ú‹ÁËÛË ÙÔ˘ Û˘Ó‰˘·ÛÌÔ‡ ÙˆÓ ‰‡Ô ·˘ÙÒÓ Î·ÙË-
ÁÔÚÈÒÓ Ê·ÚÌ¿ÎˆÓ Ì ÙËÓ ›‰È· Û˘Û΢‹ ›ӷÈÙÔ˘Ï¿¯ÈÛÙÔÓ ÂÍ›ÛÔ˘ ·ÔÙÂÏÂÛÌ·ÙÈ΋ Î·È ·ÛÊ·-Ï‹˜ Ì ÙË ‰È·‰Ô¯È΋ ¯ÔÚ‹ÁËÛ‹ ÙÔ˘˜ Ì ‰‡Ô‰È·ÊÔÚÂÙÈΤ˜ Û˘Û΢¤˜.
√ ·ÚÈıÌfi˜ ÙˆÓ ÎÏÈÓÈÎÒÓ ÌÂÏÂÙÒÓ ÛÙ· ·È-‰È¿ Â›Ó·È ÂÚÈÔÚÈṲ̂ÓÔ˜ Û ۯ¤ÛË Ì ÂΛÓÔÓÙˆÓ ÂÓËϛΈÓ. øÛÙfiÛÔ, ÛÙËÓ ÏÂÈÔÓfiÙËÙ¿ÙÔ˘˜ Î·È ÔÈ ·È‰È·ÙÚÈΤ˜ ÌÂϤÙ˜ ˘ÔÛÙËÚ›-˙Ô˘Ó ÙË ÌÂÁ·Ï‡ÙÂÚË ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙÔ˘Û˘Ó‰˘·ÛÌÔ‡ ∂ÈÛ∫™ Î·È ‚2-·ÁˆÓÈÛÙ‹ Ì·ÎÚ¿˜‰Ú¿Û˘ Û˘ÁÎÚÈÙÈο Ì ÙË ÌÔÓÔıÂڷ›· ÌÂ∂ÈÛ∫™, ÂÓÒ ÔÈ ‰ÈÂıÓ›˜ ÔÌÔʈӛ˜ ÙÔÓ Û˘ÓÈ-ÛÙÔ‡Ó. ∞·ÈÙÂ›Ù·È ÂÚ·ÈÙ¤Úˆ ¤Ú¢ӷ ÁÈ· ÙÔÓ·ÎÚÈ‚‹ ÚfiÏÔ ÙˆÓ Û˘Ó‰˘·ÛÌÒÓ Û ËÏÈ˘ ÌÈ-ÎÚfiÙÂÚ˜ ÙˆÓ 12 ÂÙÒÓ.
µÈ‚ÏÈÔÁÚ·Ê›·
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¶·È‰È·ÙÚÈ΋ 2006;69:417-424
424 ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜, ∫. ¡. ¶Ú›ÊÙ˘
randomized controlled trial. Am J Respir Crit CareMed 2001;163:32-36.
40. Ketchell RI, Jensen MW, Lumley P, Wright AM, Al-lenby MI, O’Connor BJ. Rapid effect of inhaled flu-ticasone propionate on airway responsiveness toadenosine 5’-monophosphate in mild asthma. J Al-lergy Clin Immunol 2002;110:603-606.
41. Barnes PJ. Decision making in asthma therapy: whatis important in clinical practice? Respir Med 2004;98(Suppl B):S1-S3.
42. Ni Chroinin M, Greenstone IR, Danish A, Mag-
dolinos H, Masse V, Zhang X et al. Long-acting be-ta2-agonists versus placebo in addition to inhaledcorticosteroids in children and adults with chronicasthma. Cochrane Database Syst Rev 2005 Oct19;CD005535.
43. Greenstone IR, Ni Chroinin MN, Masse V, Danish A,Magdalinos H, Zhang X et al. Combination of in-haled long-acting beta2-agonists and inhaled steroidsversus higher dose of inhaled steroids in children andadults with persistent asthma. Cochrane DatabaseSyst Rev 2005; CD005533.
¶·È‰È·ÙÚÈ΋ 2006;69:417-424
425∞¡∞™∫√¶∏™∏ REVIEW ARTICLE
∞ÓÙÈÊÏÂÁÌÔÓ҉˘ ·ÁˆÁ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙÔ˜
Û˘ÚÈÁÌÔ‡ ÛÙ· ¤ÓÙ ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜
ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜1, º. ∫˘Ú‚·Û›Ï˘2, π. N. ∆۷ӿη˜2
¶ÂÚ›ÏË„Ë: ∆· ‚Ú¤ÊË Î·È Ù· ·È‰È¿ Ù˘ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ì ˘ÔÙÚÔÈ¿˙ÔÓÙ· Û˘ÚÈÁÌfi ·ÔÙÂ-ÏÔ‡Ó Î·ıËÌÂÚÈÓfi ÎÏÈÓÈÎfi Úfi‚ÏËÌ·. ™ÙËÓ ÏÂÈÔÓfiÙËÙ¿ ÙÔ˘˜ Ù· ·È‰È¿ ·˘Ù¿ ·‡Ô˘Ó Ó· ÂΉËÏÒ-ÓÔ˘Ó Û˘ÚÈÁÌfi Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ· Î·È ıˆÚÂ›Ù·È fiÙÈ Û˘ÓÈÛÙÔ‡Ó ‰È·ÊÔÚÂÙÈÎfi ÎÏÈÓÈÎfi Û‡Ó‰ÚÔÌÔ·fi ÙÔ ¿ÛıÌ·. ™ÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹, Ë·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ÔÔ›·˜ ÔÈΛÏÏÂÈ. ∆· ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ (∫™) Û˘ÓÈÛÙÔ‡ÓÙËÓ Ï¤ÔÓ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·ÁˆÁ‹ Î·È Ê·›ÓÂÙ·È fiÙÈ ‚ÔËıÔ‡Ó È‰È·›ÙÂÚ· Ù· ‚Ú¤ÊË Î·È Ù· Ó‹È· ̉ȷÏ›ÔÓÙ· Û˘ÚÈÁÌfi Î·È ·ÙÔÈÎfi ˘fiÛÙڈ̷ (ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ¿ÛıÌ·ÙÔ˜, ÈÛÙÔÚÈÎfi ‰È·ÁÓˆ-Ṳ̂ÓÔ˘ ÂÎ˙¤Ì·ÙÔ˜ ‹/Î·È ·ÏÏÂÚÁÈ΋˜ ÚÈÓ›Ùȉ·˜, ˘„ËÏ‹ ÔÏÈ΋ IgE, ˈÛÈÓÔÊÈÏ›·) ηıÒ˜ Î·È ÂΛӷԢ ÂΉËÏÒÓÔ˘Ó Â›ÌÔÓ· Û˘ÌÙÒÌ·Ù·, ÂÓÒ Ë Ì·ÎÚÔ¯ÚfiÓÈ· ¯ÔÚ‹ÁËÛ‹ ÙÔ˘˜ Â›Ó·È ·ÛÊ·Ï‹˜ Û ¯·-ÌËϤ˜ ‰fiÛÂȘ. ¶ÚfiÛÊ·Ù˜ ηϿ ۯ‰ȷṲ̂Ó˜ ÌÂϤÙ˜ Û ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ ¤¯Ô˘Ó ‰Â›-ÍÂÈ fiÙÈ Ë ÌÔÓÙÂÏÔ˘Î¿ÛÙË Â›Ó·È ·ÛÊ·Ï‹˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ Û ̷ÎÚÔ¯ÚfiÓÈ· Û˘Á¯ÔÚ‹ÁËÛË Ì ٷÂÈÛÓÂfiÌÂÓ· ∫™ (Û˘Ó‰˘·Ṳ̂ÓË ıÂڷ›·), ÂÓÒ ÌÔÚ› Ó· ·Ô‚› ·ÔÙÂÏÂÛÌ·ÙÈ΋ Î·È ˆ˜ ÌÔÓÔıÂ-ڷ›· ÛÙÔÓ ‰È·Ï›ÔÓÙ· ÈÔÁÂÓ‹ Û˘ÚÈÁÌfi Ù˘ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜. √È Í·Óı›Ó˜ ·ÔÙÂÏÔ‡Ó ÂÓ·Ï-Ï·ÎÙÈ΋, ÚfiÛıÂÙË ÛÙ· ÂÈÛÓÂfiÌÂÓ· ∫™ ıÂڷ›·, fï˜, ··ÈÙÂ›Ù·È ·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ ÂȤ-‰ˆÓ ıÂÔÊ˘ÏÏ›Ó˘ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ. ªÂÁ¿ÏË ÚÔÔÙÈ΋ ÌÂϤÙË Î·ıÒ˜ Î·È ÌÂÙ·-·Ó¿Ï˘ÛËÎÏÈÓÈÎÒÓ ÌÂÏÂÙÒÓ Ô˘ Û˘Ó¤ÎÚÈÓ·Ó ÙȘ ¯ÚˆÌfiÓ˜ Ì ٷ ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ·ÌÊÈÛ‚‹-ÙËÛ·Ó ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ¿ ÙÔ˘˜ ÛÙÔÓ Â·Ú΋ ¤ÏÂÁ¯Ô ÙÔ˘ ¿ÛıÌ·ÙÔ˜. øÛÙfiÛÔ, ·˘Ù¤˜ ‰È·ÙË-ÚÔ‡Ó ÙÔÓ ÚfiÏÔ ÙÔ˘˜, ·Ó Î·È ·Ô‰˘Ó·ÌˆÌ¤ÓÔ, ˆ˜ ÂÓ·ÏÏ·ÎÙÈ΋ ÌÔÓÔıÂڷ›· ÛÙÔ ‹ÈÔ Â›ÌÔÓÔ¿ÛıÌ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜.
§¤ÍÂȘ ÎÏÂȉȿ: ∞ÓÙÈÏ¢ÎÔÙÚÈÂÓÈο, ¿ÛıÌ·, ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, Ó‹È·, Í·Óı›Ó˜, Û˘ÚÈÁÌfi˜,¯ÚˆÌfiÓ˜.
Anti-inflammatory therapy of recurrent wheezing during the first
five years of life
M. B. Anthracopoulos1, F. Kyrvassilis2, J. N. Tsanakas2
Abstract: Recurrent wheezing is a common clinical problem in infants and toddlers. Although most ofthese children stop wheezing later in childhood, and their symptoms most probably constitute a clinicalsyndrome different from asthma, they seem to respond -albeit variably- to anti-inflammatory treatmentfor asthma. Inhaled corticosteroids (CS) have proved to be helpful, particularly in those infants andtoddlers with atopic background (i.e. positive family history of asthma, history of physician-diagnosedeczema and/or allergic rhinitis, increased total IgE levels and blood eosinophils) and in those withpersistent symptoms. Long-term inhalation of CS at low doses has proved to be safe in young children.Recent well-designed clinical studies have shown that montelukast constitutes a safe and effective add-on therapy in infants and toddlers, and may improve symptoms when used as monotherapy. Xanthenesconstitute an alternative additive to inhaled CS, combination therapy, but careful monitoring of serumlevels is needed. A large prospective study and a meta-analysis, which compared inhaled CS withcromones, questioned the efficacy of cromones in the control of persistent asthma; however, these drugsmaintain their role (albeit weakened) as an alternative monotherapy in mild persistent asthma.
Key words: Anti-leukotriene agents, asthma, cromones, infants, inhaled corticosteroids, wheezing,xanthenes.
1 ¶·È‰ÔÓ¢ÌÔÓÔÏÔÁÈ΋ªÔÓ¿‰·, ¶·È‰È·ÙÚÈ΋∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘¶·ÙÚÒÓ
2 ¶·È‰ÔÓ¢ÌÔÓÔÏÔÁÈ΋ªÔÓ¿‰·, °/¶¢,πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌ›Ô,∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ£ÂÛÛ·ÏÔӛ΢.
AÏÏËÏÔÁÚ·Ê›·:
ªÈ¯¿Ï˘ ∞ÓıÚ·ÎfiÔ˘ÏÔ˜¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ¶·ÙÚÒÓ∆.∫. 26504, ƒ›Ô∂-mail: [email protected]
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 28-11-2005∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 19-09-2006
1 Unit of PaediatricPneumonology, PaediatricClinic, University of Patras
2 Unit of PaediatricPneumonology, 3rdPaediatric Clinic, IppokratioHospital, AristotleUniversity of Thessaloniki
Correspondence:
Michael AnthracopoulosPaediatric Clinic, University of Patras26504, Rio∂-mail: [email protected]
Date of submission: 28-11-2005Date of approval: 19-09-2006
¶·È‰È·ÙÚÈ΋ 2006;69:425-431
426 ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ Î·È Û˘Ó.
™˘ÓÙÔÌÔÁڷʛ˜:
∫™: ∫ÔÚÙÈÎÔÛÙÂÚÔÂȉ‹¡√: ªÔÓÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘∆∂¶: ∆Ì‹Ì· ∂ÂÈÁfiÓÙˆÓ ¶ÂÚÈÛÙ·ÙÈÎÒÓ
∂ÈÛ·ÁˆÁ‹
√ ˘ÔÙÚÔÈ¿˙ˆÓ Û˘ÚÈÁÌfi˜, ȉ›ˆ˜ ÌÂÙ¿ ·fiÈÔÁÂÓ‹ Ïԛ̈ÍË ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡, ·ÔÙÂÏÂ›Û˘¯Ófi Û‡Ìو̷ ηٿ ÙË ‚ÚÂÊÈ΋ Î·È ÙËÓ ÚÔ-Û¯ÔÏÈ΋ ËÏÈΛ· (1,2). ∆· ÂÚÈÛÛfiÙÂÚ· ·fi ·˘-Ù¿ Ù· ‚Ú¤ÊË Î·È ÌÈÎÚ¿ ·È‰È¿ ·‡Ô˘Ó Ó· ÂΉË-ÏÒÓÔ˘Ó Û˘ÚÈÁÌfi Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ·, fï˜,Û ÌÂÚÈο Ù· Û˘ÌÙÒÌ·Ù· ·Ú·Ì¤ÓÔ˘Ó Î·È ÙÂ-ÏÈο ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ˆ˜ ·ÛıÌ·ÙÈο. ∞fi ÙËÓ¿ÏÏË ÌÂÚÈ¿, ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ ÙÔ ¿ÛıÌ· Û˘-Ó‹ıˆ˜ ÂΉËÏÒÓÂÙ·È ÓˆÚ›˜ ηٿ ÙËÓ ·È‰È΋ËÏÈΛ· (3), ÂÓÒ Ë ÚÒÈÌË ·Ú¤Ì‚·ÛË Ì ·ÓÙÈ-ÊÏÂÁÌÔÓÒ‰Ë ·ÁˆÁ‹ Â›Ó·È Èı·Ófi -¿ÓÙˆ˜ οı¿ÏÏÔ ·Ú¿ ÂȂ‚·ÈˆÌ¤ÓÔ- fiÙÈ ÂËÚ¿˙ÂÈ ÙËÊ˘ÛÈ΋ ÔÚ›· Ù˘ ÓfiÛÔ˘ Î·È ÚÔÏ·Ì‚¿ÓÂÈ ÙËÌË ·Ó·ÛÙÚ¤„ÈÌË ‚ÚÔÁ¯ÔÛÙ¤ÓˆÛË (4).
¢˘ÛÙ˘¯Ò˜, ·Ú¿ ÙȘ ‰È·ÊÔÚ¤˜ ÛÙËÓ Úfi-ÁÓˆÛË, ˘¿Ú¯ÂÈ ÛËÌ·ÓÙÈ΋ ·ÏÏËÏÔÂÈÎ¿Ï˘„ËÛÙËÓ ÎÏÈÓÈ΋ ÚÔ‚ÔÏ‹ ÙˆÓ ‰‡Ô ·Ú·¿Óˆ“Û˘Ó‰ÚfiÌˆÓ Û˘ÚÈÁÌÔ‡” ÙˆÓ ÌÈÎÚÒÓ ·È‰ÈÒÓÌ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·‚‚·ÈfiÙËÙ· Î·È ÙË Û‡Á¯˘-ÛË ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘˜ (5-7). ¶Ú¿ÁÌ·ÙÈ,ηٿ ÙËÓ ÚÔÛ¯ÔÏÈ΋ ËÏÈΛ·, ÙȘ ÂÚÈÛÛfiÙÂÚ˜ÊÔÚ¤˜ Ë ·ÛıÌ·ÙÈ΋ Û˘Ìو̷ÙÔÏÔÁ›· ÂΉË-ÏÒÓÂÙ·È Ì ‰È·ÎÚÈÙ¿ ÌÂٷ͇ ÙÔ˘˜ ÂÂÈÛfi‰È·(‹È·, ̤ÙÚÈ· ‹ ÛÔ‚·Ú¿) Î·È ·Û˘Ìو̷ÙÈοÌÂÛԉȷÛÙ‹Ì·Ù· ÌÂ Û˘ÓËı¤ÛÙÂÚÔ ÂÎÏ˘ÙÈÎfi·Ú¿ÁÔÓÙ· ÙȘ ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·-Ó¢ÛÙÈÎÔ‡. ∏ ·ÚÔ‡Û· ·Ó·ÛÎfiËÛË Ú·ÁÌ·-Ù‡ÂÙ·È ÂÓ Û˘ÓÙÔÌ›· ÙË ¯Ú‹ÛË ÙˆÓ ‰È·ÊfiÚˆÓ·ÓÙÈÊÏÂÁÌÔÓˆ‰ÒÓ Ê·ÚÌ¿ÎˆÓ ÛÙÔÓ ˘ÔÙÚÔ-È¿˙ÔÓÙ· Û˘ÚÈÁÌfi ηٿ Ù· ¤ÓÙ ÚÒÙ· ¯ÚfiÓÈ·Ù˘ ˙ˆ‹˜.
ª·ÎÚÔ¯ÚfiÓÈ· ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹ ÛÂ
ËÏÈΛ· <5 ÂÙÒÓ
∏ ÚfiÛÊ·ÙË ÂıÓÈ΋ ÔÌÔʈӛ· ÙˆÓ ∏¶∞ÁÈ· ÙË ‰È¿ÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ¿ÛıÌ·-ÙÔ˜ (8), ‚·ÛÈ˙fiÌÂÓË ÛÙËÓ ÙÚ¤¯Ô˘Û· ÎÏÈÓÈ΋ÂÌÂÈÚ›· Î·È ÛÙ· Â˘Ú‹Ì·Ù· Ù˘ ÚÔÔÙÈ΋˜ÌÂϤÙ˘ Ù˘ Tucson (9), ¤¯ÂÈ ı¤ÛÂÈ Ù· ·ÎfiÏÔ˘-ı· ÎÚÈÙ‹ÚÈ· ÚÔÎÂÈ̤ÓÔ˘ Ó· ¯ÔÚËÁËı› Ì·ÎÚÔ-¯ÚfiÓÈ· ·ÓÙÈ·ÛıÌ·ÙÈ΋ ·ÁˆÁ‹ Û ·È‰È¿ ÚÔ-Û¯ÔÏÈ΋˜ ËÏÈΛ·˜:
1. µÚ¤ÊË Î·È ÌÈÎÚ¿ ·È‰È¿ Ô˘ ¯ÚÂÈ¿˙ÔÓÙ·È
Û˘Ìو̷ÙÈ΋ ·ÁˆÁ‹ Ì ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο
Û˘¯ÓfiÙÂÚ· ·fi ‰‡Ô ÊÔÚ¤˜ ÙËÓ Â‚‰ÔÌ¿‰·.
2. µÚ¤ÊË Î·È ÌÈÎÚ¿ ·È‰È¿ Ô˘ ÂΉËÏÒÓÔ˘Ó
ÂÂÈÛfi‰È· ÛÔ‚·ÚÒÓ ·ÚÔ͢ÛÌÒÓ (Ô˘ ··È-
ÙÔ‡Ó ÂÈÛÓÂfiÌÂÓÔ ‚2-‰ÈÂÁ¤ÚÙË Û˘¯ÓfiÙÂÚ· ·fi
οı 4 ÒÚ˜ ÁÈ· ¤Ó· 24ˆÚÔ) Û˘¯ÓfiÙÂÚ· ·fi ο-
ı 6 ‚‰ÔÌ¿‰Â˜.
3. µÚ¤ÊË Î·È ÌÈÎÚ¿ ·È‰È¿ Ô˘ ¤¯Ô˘Ó ÂΉË-
ÏÒÛÂÈ ÂÚÈÛÛfiÙÂÚ· ·fi ÙÚ›· ÂÂÈÛfi‰È· Û˘ÚÈÁ-
ÌÔ‡ ‰È¿ÚÎÂÈ·˜ >24ÒÚÔ˘ Ì ‰È·Ù·Ú·¯‹ ÙÔ˘
‡ÓÔ˘ ηٿ ÙÔ ÙÂÏÂ˘Ù·›Ô ¤ÙÔ˜ Î·È ¤¯Ô˘Ó ·˘ÍË-
̤ÓÔ Î›Ó‰˘ÓÔ Â›ÌÔÓÔ˘ ¿ÛıÌ·ÙÔ˜. ∞˘ÍË̤ÓÔ˘
ÎÈÓ‰‡ÓÔ˘ ıˆÚÔ‡ÓÙ·È Ù· ‚Ú¤ÊË Î·È ÌÈÎÚ¿ ·È-
‰È¿ ÌÂ: (·) ÈÛÙÔÚÈÎfi ¿ÛıÌ·ÙÔ˜ ÙÔ˘Ï¿¯ÈÛÙÔÓ
ÂÓfi˜ ·fi ÙÔ˘˜ ÁÔÓ›˜, (‚) È·ÙÚÈ΋ ‰È¿ÁÓˆÛË
·ÙÔÈ΋˜ ‰ÂÚÌ·Ù›Ùȉ·˜ ‹ (Á) ‰‡Ô ·fi Ù· ·Îfi-
ÏÔ˘ı·: (i) ‰È·ÁÓˆṲ̂ÓË ·ÏÏÂÚÁÈ΋ ÚÈÓ›Ùȉ·, (ii)
ˈÛÈÓfiÊÈÏ· ≥5% ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì·, (iii) Û˘-
ÚÈÁÌfi ¯ˆÚ›˜ ÈÔÁÂÓ‹ Ïԛ̈ÍË.
∏ ÎÏÈÓÈ΋ ·¿ÓÙËÛË ÛÙË ¯ÔÚ‹ÁËÛË ÂÈÛÓÂ-
fiÌÂÓˆÓ ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈÎÒÓ Î·È ·ÓÙÈÊÏÂÁÌÔ-
Óˆ‰ÒÓ Ê·ÚÌ¿ÎˆÓ Û˘¯Ó¿ ·Ô‚·›ÓÂÈ ÙÔ Ï¤ÔÓ
·ÔÙÂÏÂÛÌ·ÙÈÎfi ÎÚÈÙ‹ÚÈÔ ÁÈ· ÙË Ï‹„Ë ÙˆÓ ıÂ-
ڷ¢ÙÈÎÒÓ ·ÔÊ¿ÛÂˆÓ (8,10).
∂ÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ (∫™)
∏ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜
Ú˘ıÌÈÛÙÈ΋˜ ·ÁˆÁ‹˜ Ì ÂÈÛÓÂfiÌÂÓ· ∫™ ÛÂ
‚Ú¤ÊË Î·È ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ ¤¯ÂÈ
‰Âȯı› Û ÛÂÈÚ¿ ÌÂÏÂÙÒÓ Ô˘ ¤¯Ô˘Ó ·ÍÈÔÏÔÁË-
ı› Û ‰‡Ô Û˘ÛÙËÌ·ÙÈΤ˜ ·Ó·ÛÎÔ‹ÛÂȘ
(11,12). ™ÙËÓ ÚÒÙË ÂÚÈÂÏ‹ÊıËÛ·Ó 24 Ù˘¯·È-
ÔÔÈË̤Ó˜ ‰ÈϤ˜ Ù˘ÊϤ˜ ÌÂϤÙ˜ Ì ÔÌ¿‰·
ÂÈÎÔÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ (Û˘ÓÔÏÈο 1087 ·ÛıÂ-
Ó›˜), ·fi ÙȘ Ôԛ˜ ÔÈ 10 ·ÊÔÚÔ‡Û·Ó Û ·È-
‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ (Û˘ÓÔÏÈο 377 ·ÛıÂ-
Ó›˜) (11). ∏ ·Ó·ÛÎfiËÛË ·˘Ù‹ ¤‰ÂÈÍ ¯·ÌËÏfi-
ÙÂÚË ‚·ıÌÔÏÔÁ›· (score) Û˘Ìو̿وÓ, ¯Ú‹ÛË
‚2-·ÁˆÓÈÛÙÒÓ Î·È per os ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ,
Î·È ˘„ËÏfiÙÂÚ˜ ÙÈ̤˜ ̤ÁÈÛÙ˘ ÂÎÓ¢ÛÙÈ΋˜
ÚÔ‹˜ (PEF) ÛÙ· ·È‰È¿ Ô˘ Ï¿Ì‚·Ó·Ó ÂÈÛÓÂ-
fiÌÂÓ· ∫™, ·Ó Î·È Ù· ÛÙÔȯ›· ÁÈ· Ù· ÌÈÎÚfiÙÂÚ·
·È‰È¿ ‹Ù·Ó ÂÏÏÈ‹ Û fi,ÙÈ ·ÊÔÚ¿ ÙȘ ÂÈÛ·Áˆ-
Á¤˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô. ∏ ‰Â‡ÙÂÚË Û˘ÛÙËÌ·ÙÈ΋
·Ó·ÛÎfiËÛË (12) ÂÚȤϷ‚ ٤ÛÛÂÚȘ ÂÈϤ-
ÔÓ ÌÂϤÙ˜ Ì 861 ·ÛıÂÓ›˜ ËÏÈΛ·˜ 3 ÌËÓÒÓ
¤ˆ˜ 8 ÂÙÒÓ (13-16), ÔÈ Ôԛ˜ η٤ÏËÁ·Ó ÛÂ
·Ó¿ÏÔÁ· Û˘ÌÂÚ¿ÛÌ·Ù·. ªÂÙ¿ ·fi Û˘ÛÙËÌ·ÙÈ-
΋ ¤Ú¢ӷ Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ ηٿ ÙËÓ ·ÚÔ‡-
Û· ·Ó·ÛÎfiËÛË ‚Ú¤ıËÎ·Ó 10 ÂÈϤÔÓ Ù˘¯·È-
ÔÔÈË̤Ó˜ ‰ÈϤ˜ Ù˘ÊϤ˜ ÌÂϤÙ˜ Ì ÔÌ¿‰·
¶·È‰È·ÙÚÈ΋ 2006;69:425-431
427∞ÓÙÈÌÂÙÒÈÛË Û˘ÚÈÁÌÔ‡ ‚ÚÂÊÒÓ Î·È ÓË›ˆÓ
ÂÈÎÔÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ Ô˘ ÂÍÂÙ¿˙Ô˘Ó ÙËÓ ·Ô-
ÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ ÂÈÛÓÂfiÌÂÓˆÓ ∫™ ÛÂ
‚Ú¤ÊË Î·È Ó‹È· Ì ›ÌÔÓÔ ‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ·
Û˘ÚÈÁÌfi (ÂÚ›Ô˘ 2000 ·ÛıÂÓ›˜) (17-26). ªÂ-
Á¿ÏË ÌÂϤÙË 528 ·È‰ÈÒÓ ËÏÈΛ·˜ 4-11 ÂÙÒÓ
Ô˘ Û˘ÁÎÚ›ÓÂÈ ‰‡Ô ‰fiÛÂȘ ÊÏÔ˘ÙÈη˙fiÓ˘ (200
¤Ó·ÓÙÈ 400 Ìg ËÌÂÚËÛ›ˆ˜), ‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÂÈ
ÔÌ¿‰· ÂÈÎÔÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ Î·È ‰ÂÓ ·Ú¤¯ÂÈ
‰Â‰Ô̤ӷ ÁÈ· ÙÔÓ ·ÚÈıÌfi ÙˆÓ ·È‰ÈÒÓ ÚÔÛ¯Ô-
ÏÈ΋˜ ËÏÈΛ·˜ Ô˘ ÂÚÈÂÏ‹ÊıËÛ·Ó ‹ ÁÈ· Ù·
·ÔÙÂϤÛÌ·Ù· Ù˘ ·ÁˆÁ‹˜ ÛÙËÓ ËÏÈÎȷ΋ ·˘Ù‹
ÔÌ¿‰· (27).
√È ÂÚÈÛÛfiÙÂÚ˜ ·fi ÙȘ ÌÂϤÙ˜ Ì·ÎÚÔ-
¯ÚfiÓÈ·˜ ¯ÔÚ‹ÁËÛ˘ ÂÈÛÓÂfiÌÂÓˆÓ ∫™ ‰Â›¯ÓÔ˘Ó
‚ÂÏÙ›ˆÛË ‰È·ÊfiÚˆÓ ÎÏÈÓÈÎÒÓ ‰ÂÈÎÙÒÓ Î·ıÒ˜
Î·È ÔÚÈÛÌ¤ÓˆÓ ‰ÂÈÎÙÒÓ (·Ó Î·È fi¯È fiψÓ) Ó¢-
ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ ·ÛıÂÓÒÓ (13-25).
øÛÙfiÛÔ, ÌÈÎÚ‹ ÌÂϤÙË 31 ·È‰ÈÒÓ Î¿Ùˆ ÙˆÓ 2
ÂÙÒÓ ‰ÂÓ ¤‰ÂÈÍ ‚ÂÏÙ›ˆÛË ÙˆÓ ÎÏÈÓÈÎÒÓ ‰ÂÈ-
ÎÙÒÓ Û ·ÛıÂÓ›˜ Ô˘ ¤Ï·‚·Ó 200 Ìg ÂÈÛÓÂfi-
ÌÂÓ˘ ÌÂÎÏÔÌÂı·˙fiÓ˘ Ì·˙› ÌÂ Û·Ï‚Ô˘Ù·Ìfi-
ÏË ‰‡Ô ÊÔÚ¤˜ ÙËÓ Ë̤ڷ › 8 ‚‰ÔÌ¿‰Â˜ ÛÂ
Û‡ÁÎÚÈÛË Ì ÙËÓ ÔÌ¿‰· ÂϤÁ¯Ô˘ (¯ÔÚ‹ÁËÛË Ìfi-
ÓÔ Û·Ï‚Ô˘Ù·ÌfiÏ˘) (23), ÂÓÒ ÌÂϤÙË 65 ‚ÚÂ-
ÊÒÓ (̤ÛË ËÏÈΛ· 11,3 Ì‹Ó˜) ‰ÂÓ ¤‰ÂÈÍ ‚ÂÏ-
Ù›ˆÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÂΛӈÓ
Ô˘ ¤Ï·‚·Ó 200 Ìg ÊÏÔ˘ÙÈη˙fiÓ˘ ËÌÂÚËÛ›ˆ˜
› 3 Ì‹Ó˜, ¤Ó·ÓÙÈ Ù˘ ÔÌ¿‰·˜ Ô˘ ¤Ï·‚ ÂÈ-
ÎÔÓÈÎfi Ê¿ÚÌ·ÎÔ (24).
™Â ÚfiÛÊ·ÙË ÔÏ˘ÎÂÓÙÚÈ΋ ÌÂϤÙË ·fi ÙȘ
∏¶∞ (n=285) Ô˘ ‰ËÌÔÛȇıËΠÛÙÔ New
England Journal of Medicine (25), ‰Â›¯ıËΠfiÙÈ
·È‰È¿ 2-3 ÂÙÒÓ Ô˘ ·Ó‹Î·Ó Û ÔÌ¿‰· ˘„ËÏÔ‡
ÎÈÓ‰‡ÓÔ˘ ÁÈ· ¿ÛıÌ· Î·È ¤Ï·‚·Ó 20 Ìg ÊÏÔ˘ÙÈ-
η˙fiÓ˘ ËÌÂÚËÛ›ˆ˜ › 2 ¤ÙË, ÂΉ‹ÏˆÛ·Ó ÏÈÁfi-
ÙÂÚ· ·ÛıÌ·ÙÈο Û˘ÌÙÒÌ·Ù· Î·È ·ÚÔ͢ÛÌÔ‡˜
Ù˘ ÓfiÛÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ·ÁˆÁ‹˜ ·fi
ÂΛӷ Ô˘ ¤Ï·‚·Ó ÂÈÎÔÓÈÎfi Ê¿ÚÌ·ÎÔ. ∂›Û˘,
Ë ÔÌ¿‰· Ù˘ ÊÏÔ˘ÙÈη˙fiÓ˘ ·ÚÔ˘Û›·Û ¯·ÌË-
ÏfiÙÂÚ˜ ·ÓÙÈÛÙ¿ÛÂȘ ·ÂÚ·ÁˆÁÒÓ (̤ÙÚËÛË ÌÂ
Ù·Ï·ÓÙˆÛÈÌÂÙÚ›·) ·fi ÙËÓ ÔÌ¿‰· ÙÔ˘ ÂÈÎÔÓÈ-
ÎÔ‡ Ê·ÚÌ¿ÎÔ˘. øÛÙfiÛÔ, ·Ú¿ ÙÔÓ ·ÔÙÂÏÂ-
ÛÌ·ÙÈÎfi ¤ÏÂÁ¯Ô ÙˆÓ ·ÛıÌ·ÙÈÎÒÓ ÂΉËÏÒÛˆÓ,
Ë ÊÏÔ˘ÙÈη˙fiÓË ‰ÂÓ ÌÂÙ¤‚·Ï ÙË Ê˘ÛÈ΋ Ô-
Ú›· Ù˘ ÓfiÛÔ˘, fiˆ˜ ·Ô‰Â›¯ıËΠ·fi ÙËÓ
·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ ·È‰ÈÒÓ ¯ˆÚ›˜ Ê·ÚÌ·-
΢ÙÈ΋ ·ÁˆÁ‹ ÁÈ· ¤Ó· ÂÈϤÔÓ ¤ÙÔ˜. ™Â ‰Â‡-
ÙÂÚË ‰ÈÏ‹ Ù˘ÊÏ‹ Ù˘¯·ÈÔÔÈË̤ÓË ÌÂϤÙË ÌÂ
ÔÌ¿‰· ÂÈÎÔÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ Ô˘ ‰È‹ÚÎÂÛ 3
¤ÙË Î·È ‰ËÌÔÛȇıËΠÛÙÔ ›‰ÈÔ Ù‡¯Ô˜ ÙÔ˘ Â-
ÚÈÔ‰ÈÎÔ‡, ÔÈ Bisgaard Î·È Û˘Ó (26) ÌÂϤÙËÛ·Ó
ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ηْ ›ÎÏËÛÈÓ
¯ÔÚ‹ÁËÛ˘ 400 Ìg ‚Ô˘‰ÂÛÔÓ›‰Ë˜ (¤Ó·ÚÍË ·Áˆ-
Á‹˜: ÙÚ›· 24ˆÚ· ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ÙˆÓ ÂÂÈÛÔ-
‰›ˆÓ, ‰È¿ÚÎÂÈ· ·ÁˆÁ‹˜: ‰‡Ô ‚‰ÔÌ¿‰Â˜ ·Ó¿
ÂÂÈÛfi‰ÈÔ) Û ‚Ú¤ÊË Ì ·ÛıÌ·ÙÈ΋ Û˘Ìو̷-
ÙÔÏÔÁ›· ÌÂÙ¿ ÙÔÓ ÚÒÙÔ Ì‹Ó· ˙ˆ‹˜. ¢Â›¯ıËÎÂ
fiÙÈ Ë ¯ÔÚ‹ÁËÛË Ù˘ ‚Ô˘‰ÂÛÔÓ›‰Ë˜ ‰ÂÓ Â›¯Â η-
Ì›· ›‰Ú·ÛË ‚Ú·¯˘ÚfiıÂÛÌ· ÛÙ· ·ÛıÌ·ÙÈο
Û˘ÌÙÒÌ·Ù· ÙˆÓ ·È‰ÈÒÓ Î·È ‰ÂÓ Â¤‰Ú·ÛÂ
ÛÙËÓ ÂͤÏÈÍË Ù˘ ‰È·ÏÂ›Ô˘Û·˜ Û˘Ìو̷ÙÔ-
ÏÔÁ›·˜ Û ›ÌÔÓÔ ¿ÛıÌ·. √È ‰‡Ô ·˘Ù¤˜ ÌÂϤ-
Ù˜ (25,26), ÂÎÙfi˜ ·fi ÙȘ ÏËÚÔÊÔڛ˜ Ô˘
·Ú¤¯Ô˘Ó Û fi,ÙÈ ·ÊÔÚ¿ ÙÔÓ ¤ÏÂÁ¯Ô (‹ ÌË) ÙˆÓ
·ÛıÌ·ÙÈÎÒÓ Û˘ÌÙˆÌ¿ÙˆÓ ·fi Ù· ‰È¿ÊÔÚ· ıÂ-
ڷ¢ÙÈο Û¯‹Ì·Ù· ÂÈÛÓÂfiÌÂÓˆÓ ∫™, ÂÓÈÛ¯‡-
Ô˘Ó ÙËÓ ¿Ô„Ë fiÙÈ Ù· Ê¿Ú̷η ·˘Ù¿, ·ÎfiÌË
Î·È Û ÚÒÈÌË ¯ÔÚ‹ÁËÛË, ‰ÂÓ ÌÂÙ·‚¿ÏÏÔ˘Ó ÙË
Ê˘ÛÈ΋ ÔÚ›· Ù˘ ÓfiÛÔ˘. ™Â ·Ó¿ÏÔÁ· Û˘ÌÂ-
Ú¿ÛÌ·Ù· ›¯Â ηٷϋÍÂÈ Î·È Ë ÌÂϤÙË CAMP Ë
ÔÔ›· ÂÚȤϷ‚ ·È‰È¿ ÌÂÁ·Ï‡ÙÂÚ˘ ËÏÈΛ·˜
(28).
¶·ÚfiÙÈ ‰ÂÓ ˘¿Ú¯Ô˘Ó ÌÂÁ¿Ï˜ Ì·ÎÚÔ¯Úfi-
ÓȘ ÌÂϤÙ˜ ‹ Û˘ÛÙËÌ·ÙÈΤ˜ ·Ó·ÛÎÔ‹ÛÂȘ
Ô˘ Ó· ‰ÈÂÚ¢ÓÔ‡Ó ÙË Û˘ÛÙËÌ·ÙÈ΋ ‰Ú¿ÛË ÙˆÓ
ÂÈÛÓÂfiÌÂÓˆÓ ∫™ ÂȉÈο Û ·È‰È¿ ÚÔÛ¯ÔÏÈ-
΋˜ ËÏÈΛ·˜, ÔÚÈṲ̂Ó˜ ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó fiÙÈ,
ÙÔ˘Ï¿¯ÈÛÙÔÓ ÛÙȘ ˘„ËÏfiÙÂÚ˜ ‰fiÛÂȘ, Ú¿ÁÌ·-
ÙÈ ˘Ê›ÛÙ·Ù·È Û˘ÛÙËÌ·ÙÈ΋ ‰Ú¿ÛË (19,29,30).
∂Í¿ÏÏÔ˘, Ë ‡·ÚÍË Û˘ÛÙËÌ·ÙÈ΋˜ ‰Ú¿Û˘ ÙˆÓ
ÂÈÛÓÂfiÌÂÓˆÓ ∫™ ¤¯ÂÈ ‰Âȯı› Û ÌÂϤÙ˜ ηÈ
ÌÂÙ·-·Ó·Ï‡ÛÂȘ ÌÂÁ·Ï‡ÙÂÚˆÓ ·È‰ÈÒÓ (31,32).
¶Ú¤ÂÈ ˆÛÙfiÛÔ Ó· ÙÔÓÈÛı› fiÙÈ Ë ·ÓÙÈÊÏÂÁÌÔ-
Ó҉˘ ‰Ú¿ÛË ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ fiÛÔÓ
·ÊÔÚ¿ ÛÙÔ ·Ó·Ó¢ÛÙÈÎfi Û‡ÛÙËÌ· Â›Ó·È ·Ó·Ì-
ÊÈÛ‚‹ÙËÙË Î·È fiÙÈ, fiˆ˜ Û˘Ì‚·›ÓÂÈ Ì ٷ ÌÂ-
Á·Ï‡ÙÂÚ· ·È‰È¿, ¤ÙÛÈ Î·È ÛÙ· Ó‹È·, Ù· ÂÈ-
ÛÓÂfiÌÂÓ· ∫™ ·ÔÙÂÏÔ‡Ó ÙË ıÂڷ›· ÂÎÏÔ-
Á‹˜ Û fiÏ· Ù· ÛÙ¿‰È· ÙÔ˘ ›ÌÔÓÔ˘ ¿ÛıÌ·ÙÔ˜,
ÌfiÓ· ‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ¿ÏÏ· Ê¿Ú̷η (‚2-
·ÁˆÓÈÛÙ¤˜ Ì·ÎÚ¿˜ ‰Ú¿Û˘, ·ÓÙÈÏ¢ÎÔÙÚÈÂÓÈο,
ıÂÔÊ˘ÏÏ›ÓË) (8,10,33). ∂Ô̤ӈ˜, Ë “ÎÔÚÙÈÎÔ-
ÊÔ‚›·” ‰ÂÓ ‰ÈηÈÔÏÔÁÂ›Ù·È ÂÊfiÛÔÓ Ô ÁÈ·ÙÚfi˜
‚Ú›ÛÎÂÙ·È ¿ÓÙ· Û ÂÁÚ‹ÁÔÚÛË ÚÔÎÂÈ̤ÓÔ˘
Ó· ÂÏ·ÙÙÒÛÂÈ ÙË ‰fiÛË ÛÙËÓ ÂÏ¿¯ÈÛÙË ··ÈÙÔ‡-
ÌÂÓË ÁÈ· ÙÔÓ ¤ÏÂÁ¯Ô ÙÔ˘ ¿ÛıÌ·ÙÔ˜ (34-36). ∆Ô-
Ó›˙ÂÙ·È fiÙÈ Ë ¯ÔÚ‹ÁËÛË Ì·ÎÚÔ¯ÚfiÓÈ·˜ Ú˘ıÌÈ-
ÛÙÈ΋˜ ·ÁˆÁ‹˜ Ì ÂÈÛÓÂfiÌÂÓ· ∫™ ηٿ ÙËÓ
ÚÔÛ¯ÔÏÈ΋ ËÏÈΛ· Û ‰fiÛË ˘„ËÏfiÙÂÚË Ù˘
ÔÚÈ˙fiÌÂÓ˘ ·fi ÙȘ ÔÌÔʈӛ˜ ˆ˜ “¯·ÌËÏ‹˜”
(¶›Ó·Î·˜ 1) Ú¤ÂÈ Ó· Û˘Ó·ÔÊ·Û›˙ÂÙ·È ¿-
ÓÙÔÙ Ì ÙÔÓ ÂȉÈÎfi ÁÈ·ÙÚfi (8,10,33).
∆· ‰È·Ï›ÔÓÙ· ÂÂÈÛfi‰È· Û˘ÚÈÁÌÔ‡ ÌÂÙ¿
·fi ÈÔÁÂÓ‹ Ïԛ̈ÍË Û ·ÛıÂÓ›˜ Ô˘ ‰ÂÓ
¤¯Ô˘Ó ηӤӷ Û‡Ìو̷ ηٿ Ù· ÌÂÛԉȷÛÙ‹-
Ì·Ù·, ·ÔÙÂÏÔ‡Ó È‰È·›ÙÂÚÔ Úfi‚ÏËÌ·, ÛÙËÓ
¶·È‰È·ÙÚÈ΋ 2006;69:425-431
428 ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ Î·È Û˘Ó.
ÚÔÛ¯ÔÏÈ΋ ËÏÈΛ·. ∆· ÂÂÈÛfi‰È· ·˘Ù¿ Ê·›ÓÂ-Ù·È fiÙÈ ··ÓÙÔ‡Ó, ÙÔ˘Ï¿¯ÈÛÙÔÓ ÂÓ Ì¤ÚÂÈ, Û ‰È·-ÏÂ›Ô˘Û· ·ÁˆÁ‹ Ì ˘„ËϤ˜ ‰fiÛÂȘ ÂÈÛÓÂfiÌÂ-ÓˆÓ ∫™. øÛÙfiÛÔ, Ë ¯ÚfiÓÈ· ‹ ‰È·ÏÂ›Ô˘Û· ·ÓÙÈ-ÊÏÂÁÌÔÓ҉˘ ·ÁˆÁ‹ Ì ¯·ÌËÏ‹ ‹ ̤ÙÚÈ· ‰fiÛË,‰ÂÓ Ê·›ÓÂÙ·È Ó· ÚÔÛٷهÂÈ ·ÔÙÂÏÂÛÌ·ÙÈο·fi ÙȘ ˘ÔÙÚÔ¤˜ (26,37).
∞ÓÙÈÏ¢ÎÔÙÚÈÂÓÈο
∆· ·ÓÙÈÏ¢ÎÔÙÚÈÂÓÈο ¤¯Ô˘Ó ·ÓÙÈÊÏÂÁÌÔÓÒ-‰Ë ηıÒ˜ Î·È ÌÈÎÚ‹ ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈ΋ ‰Ú¿ÛËÛÙÔ ¿ÛıÌ·. ∆· Ê¿Ú̷η ·˘Ù¿ ‚ÂÏÙÈÒÓÔ˘Ó Ù·Û˘ÌÙÒÌ·Ù·, ÂÈÙ˘Á¯¿ÓÔ˘Ó Ì¤ÙÚÈ· ·‡ÍËÛË Ù˘Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È ÂÏ·ÙÙÒÓÔ˘Ó ÙÔ˘˜·ÚÔ͢ÛÌÔ‡˜ Ù˘ ÓfiÛÔ˘. ∏ ‰Ú¿ÛË ÙÔ˘˜ ÛÙÔ›ÌÔÓÔ ¿ÛıÌ· ˘ÔÏ›ÂÙ·È ÂΛӢ ÙˆÓ ÂÈ-ÛÓÂfiÌÂÓˆÓ ∫™ (38). ™Â ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ËÏÈΛ·˜ Û˘ÓÈÛÙÒÓÙ·È Î˘Ú›ˆ˜ ˆ˜ ÚfiÛıÂÙË ıÂÚ·-›· (add-on therapy) ÛÙ· ÂÈÛÓÂfiÌÂÓ· ∫™ ÛÙÔ̤ÙÚÈÔ (›ÌÔÓÔ), ·ÏÏ¿ Î·È ˆ˜ ÂÓ·ÏÏ·ÎÙÈ΋ ÌÔ-ÓÔıÂڷ›· ÛÙÔ ‹ÈÔ (›ÌÔÓÔ ‹ ˘ÔÙÚÔÈ¿-˙ÔÓ) ¿ÛıÌ· (8,10,33,36). ªÔÚ› ÂÔ̤ӈ˜, Ó·Û˘ÓÂÈÛʤÚÔ˘Ó ÛÙË ‰È·Ù‹ÚËÛË Ù˘ ‰fiÛ˘ ÙˆÓ ÂÈ-ÛÓÂfiÌÂÓˆÓ ∫™ Û ¯·ÌËÏ¿ ›‰·.
™ËÌÂÈÒÓÂÙ·È fiÙÈ ÔÈ Û˘ÛÙ¿ÛÂȘ ÙˆÓ Ï¤ÔӉȷ‰Â‰ÔÌ¤ÓˆÓ ÔÌÔʈÓÈÒÓ ÁÈ· ÙË ıÂڷ›·ÂÎÏÔÁ‹˜ ÙÔ˘ ›ÌÔÓÔ˘ ¿ÛıÌ·ÙÔ˜ Ô˘ ‰ÂÓ ÂϤÁ-¯ÂÙ·È Ì ¯·ÌËÏ‹ ‰fiÛË ÂÈÛÓÂfiÌÂÓˆÓ ∫™ ·È-‰ÈÒÓ ËÏÈΛ·˜ <5 ÂÙÒÓ ‰ÂÓ Û˘Ì›ÙÔ˘Ó (8,10,33).ŒÙÛÈ: (·) Û‡Ìʈӷ Ì ÙË ¢ÈÂıÓ‹ √ÌÔʈӛ·(GINA) ıÂڷ›· ÂÎÏÔÁ‹˜ Â›Ó·È Ë ·‡ÍËÛË ÙÔ˘ÂÈÛÓÂfiÌÂÓÔ˘ ∫™ ÛÙÔ Â›Â‰Ô Ù˘ ̤ÙÚÈ·˜ ‰fi-Û˘ (¶›Ó·Î·˜ 1) (10), (‚) Û‡Ìʈӷ Ì ÙËÓ ∞ÌÂ-ÚÈηÓÈ΋ √ÌÔʈӛ· (NAEPP/EPR) Ë ıÂڷ›·ÂÎÏÔÁ‹˜ Û˘Ó›ÛÙ·Ù·È ÛÙË ¯ÔÚ‹ÁËÛË Û˘Ó‰˘·ÛÌÔ‡
¯·ÌËÏ‹˜ ‰fiÛ˘ ÂÈÛÓÂfiÌÂÓÔ˘ ∫™ Î·È ‚2-·ÁˆÓÈ-ÛÙ‹ Ì·ÎÚ¿˜ ‰Ú¿Û˘ (8), ÂÓÒ (Á) Ë µÚÂÙ·ÓÈ΋√ÌÔʈӛ· (BTS/SIGN) Û˘ÓÈÛÙ¿ ÙÔÓ Û˘Ó‰˘·ÛÌfi¯·ÌËÏ‹˜ ‰fiÛ˘ ÂÈÛÓÂfiÌÂÓÔ˘ ∫™ Î·È ·ÓÙÈÏ¢-ÎÔÙÚÈÂÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ (33).
™Â ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ¤¯ÂÈ ‰ÈÂÚ¢ÓËı› ÔÚfiÏÔ˜ ÙˆÓ ·ÓÙÈÏ¢ÎÔÙÚÈÂÓÈÎÒÓ Î·È ÂȉÈÎfiÙÂÚ·Ù˘ ÌÔÓÙÂÏÔ˘Î¿ÛÙ˘, ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙԢ›ÌÔÓÔ˘ Î·È ÙÔ˘ ˘ÔÙÚÔÈ¿˙ÔÓÙÔ˜ Û˘ÚÈÁÌÔ‡ÛÙ· Ó‹È·. Œ¯ÂÈ ‰Âȯı› fiÙÈ, fiˆ˜ Û˘Ì‚·›ÓÂÈ ÌÂÙ· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜, ¤ÙÛÈÎ·È ÛÙ· Ó‹È· Ì ›ÌÔÓ· ·ÛıÌ·ÙÈο Û˘ÌÙÒ-Ì·Ù· Ë ¯ÔÚ‹ÁËÛË ÌÔÓÙÂÏÔ˘Î¿ÛÙ˘ Û˘Óԉ‡Â-Ù·È ·fi ‚ÂÏÙ›ˆÛË ÌÈ·˜ ÛÂÈÚ¿˜ ÎÏÈÓÈÎÒÓ ·Ú·-̤ÙÚˆÓ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ (ËÌÂÚ‹ÛÈ· Î·È Ó˘ÎÙÂÚÈÓ¿Û˘ÌÙÒÌ·Ù·, ·ÚÈıÌfi˜ ·Û˘Ìو̷ÙÈÎÒÓ ËÌÂ-ÚÒÓ, ¯ÔÚ‹ÁËÛË ·Ó·ÎÔ˘ÊÈÛÙÈ΋˜ ıÂڷ›·˜ Ì‚2-·ÁˆÓÈÛÙ¤˜ ‹ per os ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹). ∏‚ÂÏÙ›ˆÛË ·˘Ù‹ Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ·ÓÂÍ¿ÚÙËÙË·fi ÙË Û˘Ó‡·ÚÍË ·ÙÔ›·˜ ‹ ÙË Û˘Á¯ÔÚ‹ÁËÛËÂÈÛÓÂfiÌÂÓÔ˘ ∫™ (39,40). ∂ÈϤÔÓ, ¤¯ÂÈ ‰ÂÈ-¯ı› Î·È ÛÙËÓ ËÏÈÎȷ΋ ·˘Ù‹ ÔÌ¿‰· ‚ÂÏÙ›ˆÛËÙfiÛÔ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, fiÛÔ Î·È‰ÂÈÎÙÒÓ ÊÏÂÁÌÔÓ‹˜ (ÂÌÓÂfiÌÂÓÔ˘ NO, ˈÛÈ-ÓÔÊ›ÏˆÓ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜) ÌÂÙ¿ ·fi ¯ÔÚ‹-ÁËÛË ÌÔÓÙÂÏÔ˘Î¿ÛÙ˘ (41,42).
∏ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ÌÔÓÙÂÏÔ˘Î¿ÛÙ˘ˆ˜ ÌÔÓÔıÂڷ›·˜ Û ·È‰È¿ 2-5 ÂÙÒÓ Ì ‰È·Ï›-ÔÓÙ· Û˘ÌÙÒÌ·Ù· ¿ÛıÌ·ÙÔ˜ ÂϤÁ¯ıËΠ۠ÌÂ-Á¿ÏË ‰ÈÏ‹-Ù˘ÊÏ‹ Ù˘¯·ÈÔÔÈË̤ÓË ÌÂϤÙË. µÚ¤-ıËΠfiÙÈ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ‰ˆ‰ÂοÌËÓ˘ ·ÁˆÁ‹˜Ë ÌÔÓÙÂÏÔ˘Î¿ÛÙË ÂÏ¿ÙÙˆÛ ÙÔ˘˜ ·ÚÔ͢ÛÌÔ‡˜Ù˘ ÓfiÛÔ˘ ηٿ 32%, ηı˘ÛÙ¤ÚËÛ ÙËÓ ÂΉ‹Ïˆ-ÛË ÙÔ˘ ÚÒÙÔ˘ ·ÚÔ͢ÛÌÔ‡ Î·È Ì›ˆÛ ٷ ¯Ô-ÚËÁÔ‡ÌÂÓ· ÂÈÛÓÂfiÌÂÓ· ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο ηÈ∫™ Û ۯ¤ÛË Ì ÙÔ ÂÈÎÔÓÈÎfi Ê¿ÚÌ·ÎÔ (43).
∆¤ÏÔ˜, Û Úfi‰ÚÔÌË Ù˘¯·ÈÔÔÈË̤ÓË ‰ÈÏ‹-
¶·È‰È·ÙÚÈ΋ 2006;69:425-431
¶›Ó·Î·˜ 1. ¢È·‚¿ıÌÈÛË ‰ÔÛÔÏÔÁ›·˜ ÂÈÛÓÂfiÌÂÓˆÓ ∫™ Û ·È‰È¿ <12 ÂÙÒÓ (10)*
º·Ú̷΢ÙÈ΋ Ô˘Û›· (Ìg)** ÷ÌËÏ‹ ‰fiÛË ª¤ÙÚÈ· ‰fiÛË À„ËÏ‹ ‰fiÛË
µÔ˘‰ÂÛÔÓ›‰Ë (DPI) 100-200 200-600 >600µÔ˘‰ÂÛÔÓ›‰Ë (neb. suspension) 0,25-0,5 mg 0,5-1,0 mg >1,0 mgªÔÌÂÙ·˙fiÓË (DPI)*** - - -ªÂÎÏÔÌÂı·˙fiÓË (pMDI-CFC) 100-250 250-500 >500ªÂÎÏÔÌÂı·˙fiÓË (pMDI-HFA) 50-200 200-400 >400ºÏÔ˘ÙÈη˙fiÓË (pMDI-CFC, DPI) 100-200 200-400 >400
* √È ·Ó·ÁÚ·ÊfiÌÂÓ˜ ÛÙÔÓ ¶›Ó·Î· ‰fiÛÂȘ ·ÓÙÈÚÔÛˆÂ‡Ô˘Ó ÙË Ê·Ú̷΢ÙÈ΋ Ô˘Û›· Ô˘ ·ÂÏ¢ıÂÚÒÓÂÙ·È ·fiÙË ‚·Ï‚›‰· Ù˘ ‰ÔÛÈÌÂÙÚÈ΋˜ Û˘Û΢‹˜ (pMDI) ‹ Ô˘ ‚Ú›ÛÎÂÙ·È ‰È·ı¤ÛÈÌË ÌÂÙ¿ ÙËÓ ÂÓÂÚÁÔÔ›ËÛË Ù˘ Û˘Û΢‹˜ ÍËÚ¿˜ ÛÎfiÓ˘ (DPI). √È ‰fiÛÂȘ ·˘Ù¤˜ ‰ÂÓ Â›Ó·È ÔÈ ‰fiÛÂȘ Ô˘ Êı¿ÓÔ˘Ó ÛÙÔÓ ·ÛıÂÓ‹. °›ÓÂÙ·È ·Ó·ÊÔÚ¿(·ÏÊ·‚ËÙÈο) ÌfiÓÔ ÛÙ· ÂÈÛÓÂfiÌÂÓ· ∫™ Ô˘ ΢ÎÏÔÊÔÚÔ‡Ó ÛÙËÓ ∂ÏÏ¿‰·.
** √È ·Ó·ÁÚ·ÊfiÌÂÓ˜ ÛÙÔÓ ¶›Ó·Î· ‰fiÛÂȘ Â›Ó·È Û Ìg ÏËÓ ÂÎÂ›ÓˆÓ ÙÔ˘ ÂӷȈڋ̷ÙÔ˜ ‚Ô˘‰ÂÛÔÓ›‰Ë˜ Ô˘ ·Ó·-ÁÚ¿ÊÔÓÙ·È Û mg.
*** ¢ÂÓ ·Ó·Ê¤ÚÂÙ·È Û˘ÓÈÛÙÒÌÂÓË ‰fiÛË Û ·È‰È¿ <12 ÂÙÒÓ (10).
429∞ÓÙÈÌÂÙÒÈÛË Û˘ÚÈÁÌÔ‡ ‚ÚÂÊÒÓ Î·È ÓË›ˆÓ
Ù˘ÊÏ‹ ÌÂϤÙË ‰ÈÂÚ¢ӋıËÎÂ Ë ·ÔÙÂÏÂÛÌ·ÙÈ-
ÎfiÙËÙ· Ù˘ ¯ÔÚ‹ÁËÛ˘ ÌÔÓÙÂÏÔ˘Î¿ÛÙ˘ ›
Ù¤ÛÛÂÚȘ ‚‰ÔÌ¿‰Â˜ ÛÙËÓ ÚfiÏË„Ë ÙˆÓ ÌÂÙ·-
‚ÚÔÁ¯ÈÔÏÈÙȉÈÎÒÓ Û˘ÌÙˆÌ¿ÙˆÓ (44). µÚ¤ıËÎÂ
fiÙÈ ‚Ú¤ÊË 3-36 ÌËÓÒÓ Ô˘ ¤Ï·‚·Ó ÙËÓ ·ÁˆÁ‹
ÂÓÙfi˜ ‚‰ÔÌ¿‰Ô˜ (‰È¿ÌÂÛË ÙÈÌ‹: 3 Ë̤Ú˜) ·fi
ÙËÓ ¤Ó·ÚÍË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ‚ÚÔÁ¯ÈÔÏ›Ùȉ·˜
Î·È ·Ú·ÎÔÏÔ˘ı‹ıËÎ·Ó Â› ¤Ó· Ì‹Ó·, ·ÚÔ˘-
Û›·Û·Ó ÂÚÈÛÛfiÙÂÚ˜ ·Û˘Ìو̷ÙÈΤ˜ Ë̤Ú˜
¤Ó·ÓÙÈ Ù˘ ÔÌ¿‰·˜ ÙÔ˘ ÂÈÎÔÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘,
ÂÓÒ Ë ÛËÌ·ÓÙÈÎfiÙÂÚË ‰Ú¿ÛË Ù˘ ÌÔÓÙÂÏÔ˘Î¿-
ÛÙ˘ ÂΉËÏÒıËΠÛÙËÓ ÚfiÏË„Ë ÙÔ˘ ÚˆÈÓÔ‡
‚‹¯·. ∆· ηْ ·Ú¯‹Ó ÂÓı·ÚÚ˘ÓÙÈο ·˘Ù¿ ¢ڋ-
Ì·Ù· ¯ÚÂÈ¿˙ÂÙ·È Ó· ÂȂ‚·ÈˆıÔ‡Ó Ì ÚfiÛıÂ-
Ù˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜.
∆· ̤¯ÚÈ Û‹ÌÂÚ· ‰Â‰Ô̤ӷ ‰Â›¯ÓÔ˘Ó fiÙÈ ÔÈ
·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ Ù˘ ÌÔÓÙÂÏÔ˘Î¿ÛÙ˘
‰ÂÓ ‰È·Ê¤ÚÔ˘Ó ·fi ÂΛӘ ÙÔ˘ ÂÈÎÔÓÈÎÔ‡ Ê·Ú-
Ì¿ÎÔ˘ ÛÙ· ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜, fiˆ˜
ÂÍ¿ÏÏÔ˘ Û˘Ì‚·›ÓÂÈ Î·È Ì ٷ ÌÂÁ·Ï‡ÙÂÚ· ·È-
‰È¿ (39,43).
ÃÚˆÌfiÓ˜ Î·È Í·Óı›Ó˜ (ıÂÔÊ˘ÏϛӘ)
√ ÚfiÏÔ˜ ÙˆÓ ¯ÚˆÌÔÓÒÓ (¯ÚˆÌÔÁÏ˘ÎÈÎfi Ó¿-
ÙÚÈÔ, Ó‰ԯڈ̛ÏË) ÛÙË ıÂڷ›· ÙÔ˘ ·È‰ÈÎÔ‡
¿ÛıÌ·ÙÔ˜ ¤¯ÂÈ ÛËÌ·ÓÙÈο ˘Ô‚·ıÌÈÛı› Ù· ÙÂ-
ÏÂ˘Ù·›· ¯ÚfiÓÈ·. ∞Ó Î·È Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜
¤¯ÂÈ ‰Âȯı› fiÙÈ Ù· Ê¿Ú̷η ·˘Ù¿ Â›Ó·È ·ÔÙÂ-
ÏÂÛÌ·ÙÈÎfiÙÂÚ· ÙÔ˘ ÂÈÎÔÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ ÛÙÔÓ
¤ÏÂÁ¯Ô ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Î·È ·ÔÙÚ¤Ô˘Ó
ÙÔ˘˜ ·ÛıÌ·ÙÈÎÔ‡˜ ·ÚÔ͢ÛÌÔ‡˜, ÙȘ ÂÈÛΤ-
„ÂȘ Û ∆∂¶ Î·È ÙȘ ÂÈÛ·ÁˆÁ¤˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô,
ÚfiÛÊ·Ù· ‰Â‰Ô̤ӷ ‰ÂÓ ÂȂ‚·ÈÒÓÔ˘Ó ÙË
¯ÚËÛÈÌfiÙËÙ¿ ÙÔ˘˜. ∏ ÌÂÁ·Ï‡ÙÂÚË Ù˘¯·ÈÔÔÈË-
̤ÓË ÌÂϤÙË Ì ÔÌ¿‰· ÂϤÁ¯Ô˘ ·È‰ÈÒÓ ÚÔ-
Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ Ì ̤ÙÚÈÔ ¿ÛıÌ· (218 ·ÛıÂ-
Ó›˜) ‰ÂÓ ·Ó¤‰ÂÈÍ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ¯ÚˆÌÔÁÏ˘-
ÎÈÎÔ‡ Ó·ÙÚ›Ô˘ Î·È ÂÈÎÔÓÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘ (45),
ÂÓÒ Û ÌÂÙ·-·Ó¿Ï˘ÛË 24 Ù˘¯·ÈÔÔÈËÌ¤ÓˆÓ ‰È-
ÏÒÓ Ù˘ÊÏÒÓ ÌÂÏÂÙÒÓ Ì ÔÌ¿‰· ÂÈÎÔÓÈÎÔ‡
Ê·ÚÌ¿ÎÔ˘ (ÂÚÈÛÛfiÙÂÚ· ·fi 1.000 ·È‰È¿ Û˘-
ÓÔÏÈο, ·fi Ù· ÔÔ›· Ù· ÌÈÛ¿ ÂÚ›Ô˘ ‹Ù·Ó Ó‹-
È·) ·ÌÊÈÛ‚ËÙÂ›Ù·È Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙÔ˘
¯ÚˆÌÔÁÏ˘ÎÈÎÔ‡ ˆ˜ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ·ÓÙÈ·ÛıÌ·ÙÈ-
΋˜ ·ÁˆÁ‹˜ (46). ¶ÚfiÛÊ·ÙË ÌÂÁ¿ÏË Ì·ÎÚÔ¯Úfi-
ÓÈ· Ù˘¯·ÈÔÔÈË̤ÓË ÌÂϤÙË ·ÛıÌ·ÙÈÎÒÓ ·È-
‰ÈÒÓ 5-12 ÂÙÒÓ ·fi ÙȘ ∏¶∞ (ÌÂϤÙË CAMP)
Û˘Ó¤ÎÚÈÓ ÙË Ì·ÎÚÔ¯ÚfiÓÈ· ¯ÔÚ‹ÁËÛË ‚Ô˘‰ÂÛÔ-
Ó›‰Ë˜ Î·È Ó‰ԯڈ̛Ï˘ Ì ÂΛÓË ÙÔ˘ ÂÈÎÔÓÈ-
ÎÔ‡ Ê·ÚÌ¿ÎÔ˘. µÚ¤ıËΠfiÙÈ Ë Ó‰ԯڈ̛ÏË
ÂÏ·ÙÙÒÓÂÈ ÌÂÓ ÙËÓ ·Ó¿ÁÎË Â›ÁÔ˘Û·˜ ıÂڷ›-
·˜ ÁÈ· ¿ÛıÌ· Î·È ÙË ¯Ú‹ÛË per os ÎÔÚÙÈÎÔÛÙÂÚÔ-
Âȉԇ˜, ·ÏÏ¿ ‰ÂÓ ÂËÚ¿˙ÂÈ Î·Ó¤Ó·Ó ¿ÏÏÔ ‰Â›-
ÎÙË ‚·Ú‡ÙËÙ·˜ (Û˘ÌÙÒÌ·Ù·, Ó¢ÌÔÓÈ΋ ÏÂÈ-
ÙÔ˘ÚÁ›·, ‚ÚÔÁ¯È΋ ˘ÂÚ··ÓÙËÙÈÎfiÙËÙ·) (28).
√È ¯ÚˆÌfiÓ˜ Â›Ó·È ÂÍ·ÈÚÂÙÈο ·ÛÊ·Ï‹ Ê¿Ú-
̷η Î·È ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ·ÔÙÂÏÔ‡Ó ÂÓ·ÏÏ·-
ÎÙÈ΋ Ì·ÎÚÔ¯ÚfiÓÈ· ·ÁˆÁ‹ ÛÙÔ ‹ÈÔ Â›ÌÔÓÔ
·È‰ÈÎfi ¿ÛıÌ·. ŸÌˆ˜, Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ
ÌÂÏÂÙÒÓ Ô˘ ÚԷӷʤÚıËηÓ, ÛÂ Û˘Ó‰˘·ÛÌfi
Ì ÙËÓ ·Ô‰Â‰ÂÈÁ̤ÓË ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ηÈ
·ÛÊ¿ÏÂÈ· ¿ÏÏˆÓ Ê·ÚÌ¿ÎˆÓ (¯·ÌËÏ‹ ‰fiÛË ÂÈ-
ÛÓÂfiÌÂÓˆÓ ∫™, ·ÓÙÈÏ¢ÎÔÙÚÈÂÓÈο, Û˘Ó‰˘·-
ÛÌÔ›), Ô‰‹ÁËÛ·Ó ÛÙËÓ ÂÁηٿÏÂÈ„Ë ·fi ÙȘ ‰È¿-
ÊÔÚ˜ ÔÌÔʈӛ˜ ÁÈ· ÙÔ ¿ÛıÌ·, Ù˘ ·Ï·ÈfiÙÂ-
Ú˘ Û‡ÛÙ·Û˘ ÁÈ· ηْ ·Ú¯‹Ó ‰ÔÎÈÌ·Û›· ¯ÚˆÌfi-
Ó˘ ÛÙÔ ·È‰ÈÎfi ‹ÈÔ Â›ÌÔÓÔ ¿ÛıÌ· (8,10,33).
√È Í·Óı›Ó˜ (ıÂÔÊ˘ÏϛӘ) Â›Ó·È ‚ÚÔÁ¯Ô-
‰È·ÛÙ·ÏÙÈΤ˜ Ô˘Û›Â˜ Ì ·ÓÔÛÔÙÚÔÔÔÈËÙÈ΋
‰Ú¿ÛË (47), Î·È Ù· Û΢¿ÛÌ·Ù· ‚Ú·‰Â›·˜ ·Ô-
‰¤ÛÌ¢Û˘ ·ÔÙÂÏÔ‡Ó ÂÓ·ÏÏ·ÎÙÈ΋ ÚfiÛıÂÙË
ÛÙ· ÂÈÛÓÂfiÌÂÓ· ∫™ ıÂڷ›· ÁÈ· ÙÔ Ì¤ÙÚÈÔ
(›ÌÔÓÔ) ¿ÛıÌ· ÙfiÛÔ ÙˆÓ ÌÂÁ·Ï‡ÙÂÚˆÓ, fiÛÔ
Î·È ÙˆÓ ·È‰ÈÒÓ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ (8,10). ∞Ó
Î·È Ë ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ¯ÔÚ‹ÁËÛË Î·È ÙÔ ¯·ÌË-
Ïfi ÎfiÛÙÔ˜ ÙÔ˘˜ ·ÔÙÂÏÔ‡Ó ÛËÌ·ÓÙÈο ÚÔÙÂ-
Ú‹Ì·Ù·, ÔÈ Û˘¯Ó¤˜ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ ηÈ
Ù· ÚÔ‚Ï‹Ì·Ù· ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙˆÓ ıÂÚ·-
¢ÙÈÎÒÓ ÂȤ‰ˆÓ ÛÙÔÓ ÔÚfi ÙˆÓ ÌÈÎÚÒÓ ·ÛıÂ-
ÓÒÓ Î·ıÈÛÙÔ‡Ó Û‹ÌÂÚ· ÙË ¯Ú‹ÛË ÙˆÓ ıÂÔÊ˘ÏÏÈ-
ÓÔ‡¯ˆÓ Û΢·ÛÌ¿ÙˆÓ ÂÈÏÔÁ‹ ¯·ÌËÏ‹˜ ÚÔÙÂ-
Ú·ÈfiÙËÙ·˜ ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ‰˘ÙÈΤ˜ ¯ÒÚ˜.
¶ÚfiÛÊ·Ù˜ ·Ó·ÊÔÚ¤˜, Ô˘ ·Ô‰ÂÈÎÓ‡Ô˘Ó
ÙË Û˘Ó¤ÚÁÂÈ· ‰Ú¿Û˘ ÌÂٷ͇ ÂÈÛÓÂÔ̤ӈÓ
ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Î·È ¯·ÌËÏ‹˜ ‰fiÛ˘ ıÂÔ-
Ê˘ÏÏ›Ó˘ ÛÙËÓ ¤ÎÊÚ·ÛË ÙˆÓ ÁÔÓȉ›ˆÓ Ô˘ Ú˘ı-
Ì›˙Ô˘Ó ÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ ÊÏÂÁÌÔÓ‹˜ ÛÙÔ
¿ÛıÌ·, ›Ûˆ˜ ʤÚÔ˘Ó ÙËÓ Î·ÙËÁÔÚ›· ·˘Ù‹ Ê·Ú-
Ì¿ÎˆÓ Î·È ¿ÏÈ ÛÙÔ ÚÔÛ΋ÓÈÔ ÙÔ˘ ÂӉȷʤÚÔ-
ÓÙÔ˜ (48).
™˘Ì¤Ú·ÛÌ·
∆· ÂÈÛÓÂfiÌÂÓ· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Ì ÙËÓ
ÈÛ¯˘Ú‹ ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë ‰Ú¿ÛË ÙÔ˘˜ Î·È ÙËÓ ¤Ï-
ÏÂÈ„Ë ·ÓÂÈı‡ÌËÙˆÓ ÂÓÂÚÁÂÈÒÓ Û ¯·ÌËϤ˜ ‰fi-
ÛÂȘ, ÌfiÓ· ‹ Û˘ÓÂÈÎÔ˘ÚÔ‡ÌÂÓ· ·fi Ù· ·ÓÙÈÏ¢-
ÎÔÙÚÈÂÓÈο, Ô˘ ¤¯Ô˘Ó ÙÔ ÏÂÔÓ¤ÎÙËÌ· Ù˘ ¢-
ÎÔÏ›·˜ Ù‹˜ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ¯ÔÚ‹ÁËÛ‹˜ ÙÔ˘˜,
·ÔÙÂÏÔ‡Ó ÙȘ Û‡Á¯ÚÔÓ˜ ÂÈÏÔÁ¤˜ ÁÈ· ÙËÓ
·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë ·ÁˆÁ‹ ÙÔ˘ ˘ÔÙÚÔÈ¿˙ÔÓÙÔ˜
Û˘ÚÈÁÌÔ‡ ÛÙ· ¤ÓÙ ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜. √
ÚfiÏÔ˜ ÙˆÓ ¯ÚˆÌÔÓÒÓ ¤¯ÂÈ ˘Ô‚·ıÌÈÛı› Ù·
¶·È‰È·ÙÚÈ΋ 2006;69:425-431
430 ª. B. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ Î·È Û˘Ó.
ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, ·Ó Î·È Ù· Ê¿Ú̷η ·˘Ù¿ ·-ڷ̤ÓÔ˘Ó ÂÓ·ÏÏ·ÎÙÈ΋ ÂÈÏÔÁ‹ Ì·ÎÚÔ¯ÚfiÓÈ·˜ÌÔÓÔıÂڷ›·˜ ÛÙÔ ‹ÈÔ Â›ÌÔÓÔ ¿ÛıÌ·.
µÈ‚ÏÈÔÁÚ·Ê›·
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¶·È‰È·ÙÚÈ΋ 2006;69:425-431
432 ∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞ ORIGINAL ARTICLE
µ’ ÈڤÌÂÈÔ Œ·ıÏÔ ÛÙÔ44Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi™˘Ó¤‰ÚÈÔ, ƒfi‰Ô˜, 9-11πÔ˘Ó›Ô˘ 2006
1 ¢È·‚ËÙÔÏÔÁÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ“¶. & ∞. ∫˘ÚÈ·ÎÔ‡”,∞ı‹Ó·
2 ∫·Ú‰ÈÔÏÔÁÈ΋ ∫ÏÈÓÈ΋, 7Ô ¡ÔÛÔÎÔÌÂ›Ô π.∫.∞.,∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
∫˘Úȷ΋ K·Ú·‚·Ó¿ÎË∂ÏÏËÓÈÎÔ‡ ™ÙÚ·ÙÔ‡ 22∞,∆.∫. 152 37, ºÈÏÔı¤Ë, ∞ı‹Ó·
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 23-10-2006∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 24-10-2006
2nd Choremeio Prize at the44th Panhellenic PaediatricCongress, Rhodes, 9-11 June, 2006
1 Diabetic Clinic, 2nd PaediatricDepartment, University of Athens, “P. & A. Kyriakou”Children’s Hospital, Athens
2 Cardiology Clinic, 7th IKAHospital, Athens
¶·È‰È·ÙÚÈ΋ 2006;69:432-439
H Û˘Ì‚ÔÏ‹ Ù˘ ÚÒÈÌ˘ ‰È·Ù·Ú·¯‹˜
ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ηÈ
·Ú·ÌÔÓ‹ ˘„ËÏ‹˜ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ ηٿ
ÙË Ó‡ÎÙ· ÛÙËÓ ·ıÔÁ¤ÓÂÈ·
Ù˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜
Û ÂÊ‹‚Ô˘˜ Ì ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Ù‡Ô˘ 1
∫. K·Ú·‚·Ó¿ÎË1, °. ∫·˙È¿Ó˘2, Ã. ∫·Ú·ÁÈ¿ÓÓË1, ∞. ™Î·Ú‰Ô‡ÙÛÔ˘1, ∫. ∫·ÎϤ·˜1, ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜1
¶ÂÚ›ÏË„Ë
∂ÈÛ·ÁˆÁ‹: ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ÂÎÙ›ÌËÛË Ù˘ Û˘Ì‚ÔÏ‹˜ ÙˆÓ ÌÂÙ·‚ÔÏÒÓ Ù˘ ·ÚÙË-Úȷ΋˜ ›ÂÛ˘ (∞¶) ηٿ ÙË Ó‡ÎÙ· Î·È Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (∞¡™)ÛÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜ Û ÂÊ‹‚Ô˘˜ Ì ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Ù‡Ô˘ 1 (™¢1).
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ªÂÏÂÙ‹ıËÎ·Ó 22 ¤ÊË‚ÔÈ ËÏÈΛ·˜ 16,7±4,1 ÂÙÒÓ Ì ™¢ Ù‡Ô˘ 1. ŒÁÈÓ 24ˆÚË Î·-Ù·ÁÚ·Ê‹ Ù˘ ∞¶, ÂÓÒ Ë ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ∞¡™ ÌÂÏÂÙ‹ıËΠ̠24ˆÚË Î·Ù·ÁÚ·Ê‹ ÙÔ˘ ηډȷÎÔ‡ Ú˘ı-ÌÔ‡ Î·È ·Ó·Ï‡ıËΠ̠ÙË Ì¤ıÔ‰Ô power spectra. ∏ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›· ÂÎÙÈÌ‹ıËΠ̠12ˆÚÂ˜Û˘ÏÏÔÁ¤˜ Ô‡ÚˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ë̤ڷ˜/Ó‡ÎÙ·˜. √È ¤ÊË‚ÔÈ ¯ˆÚ›ÛÙËÎ·Ó Û ‰‡Ô ÔÌ¿‰Â˜, ·Ó¿ÏÔÁ·Ì ÙË ‰È¿ÌÂÛË ÙÈÌ‹ ÏÂ˘ÎˆÌ·Ù›Ó˘ Ô‡ÚˆÓ 12ÒÚÔ˘ (1Ë ÔÌ¿‰·: ≥7,5 Ìg/min, 2Ë ÔÌ¿‰·: <7,5 Ìg/min).
∞ÔÙÂϤÛÌ·Ù·: ¢È·ÈÛÙÒıËΠfiÙÈ Ë 1Ë ÔÌ¿‰· ›¯Â ˘„ËÏfiÙÂÚ˜ ÙÈ̤˜ ̤Û˘ Û˘ÛÙÔÏÈ΋˜ (™¶)(109,3 vs 106,5 mmHg, p=0,028) Î·È Ì¤Û˘ ‰È·ÛÙÔÏÈ΋˜ ›ÂÛ˘ (¢¶) ηٿ ÙË Ó‡ÎÙ· (77,8 vs 73,6mmHg, p=0,035), ·ÓÙ›ÛÙÔȯ·, ηıÒ˜ ›Û˘ Î·È ˘„ËÏfiÙÂÚ˜ ÙÈ̤˜ Ù˘ ·Ú·Ì¤ÙÚÔ˘ pNN50 ÙÔ˘Î·Ú‰È·ÎÔ‡ Ú˘ıÌÔ‡ (32,9% vs 19,9%, p<0,009). ™ÙË Û˘Ó¤¯ÂÈ·, ÔÈ ·ÛıÂÓ›˜ ¯ˆÚ›ÛÙËÎ·Ó ·Ó¿ÏÔÁ· ÌÂÙËÓ ·Ô˘Û›· ÙÒÛ˘ (non-dippers) ‹ ÙËÓ ÙÒÛË (dippers) Ù˘ ¢¶ ηٿ ÙË Ó‡ÎÙ·. ∏ oÌ¿‰· ÙˆÓnon-dippers ·ÚÔ˘Û›·˙ ηٿ ÙË Ó‡ÎÙ· ˘„ËÏfiÙÂÚË ÙÈÌ‹ ̤ÛÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ (RR) (916 vs764 ms, p=0,001) Î·È ˘„ËÏfiÙÂÚË ·Ú¿ÌÂÙÚÔ SDNN-i (110,6 vs 88,1 ms, p=0,018) Û˘ÁÎÚÈÙÈο ÌÂÂΛÓË ÙˆÓ dippers, ÂÓÒ ‰ÂÓ ˘‹Ú¯Â ‰È·ÊÔÚ¿ ÛÙËÓ ·¤ÎÎÚÈÛË ÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡Ú· (∞§√). ∆ÔËÏ›ÎÔ Ù˘ ¢¶ Ó‡ÎÙ·˜/Ë̤ڷ˜ Û˘Û¯ÂÙÈ˙fiÙ·Ó ıÂÙÈο Ì ÙÔÓ Î·Ú‰È·Îfi Ú˘ıÌfi RR (r=0,49, p=0,001).
™˘ÌÂÚ¿ÛÌ·Ù·: ¢È·ÈÛÙÒÓÂÙ·È fiÙÈ Û ÂÊ‹‚Ô˘˜ ™¢1 ÔÈ ÚÒÈ̘ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ∞¡™ Î·È Ù˘ A¶ÚÔËÁÔ‡ÓÙ·È Ù˘ ÂΉ‹ÏˆÛ˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜ Î·È Â›Ó·È ‰˘Ó·Ùfi Ó· Û˘Ì‚¿ÏÏÔ˘Ó ÛÙËÓ ·-ıÔÁ¤ÓÂÈ· ·Ú¯fiÌÂÓ˘ ÓÂÊÚÔ¿ıÂÈ·˜.
§¤ÍÂȘ ÎÏÂȉȿ: ™·Î¯·Ú҉˘ ‰È·‚‹Ù˘ Ù‡Ô˘ 1, ·ÚÙËÚÙȷ΋ ˘¤ÚÙ·ÛË, ‰È·Ù·Ú·¯‹ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡Û˘ÛÙ‹Ì·ÙÔ˜.
The contribution of nocturnal blood pressure change and
autonomic dysfunction in the pathogenesis of microalbuminuria in
adolescents with type 1 diabetes mellitus
K. Karavanaki1, G. Kazianis2, C. Karagianni1, A. Skardoutsou1, C. Kakleas1, A. Constantopoulos1
Abstract
Background: The aim of the present study was to evaluate the contribution of nocturnal bloodpressure (DBP) changes and autonomic function in the pathogenesis of microalbuminuria inadolescents with type 1 diabetes mellitus (DM1).
Methods: Twenty-two normotensive, normoalbuminuric adolescents with DM1 were studied, aged16.7±4.1 years. Twenty-four-hour ambulatory BP was recorded. Twenty-four-hour heart rate spectralanalysis. Microalbuminuria was evaluated in 24-hour urine collections. The adolescents were divided into
H ÂÚÁ·Û›· ·˘Ù‹ ‚Ú·‚‡ıËΠ̠ÙÔ Bã ÈڤÌÂÈÔ Œ·ıÏÔ ÛÙÔ 44Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ƒfi‰Ô˜, 9-11 πÔ˘Ó›Ô˘ 2006
433¢È·Ù·Ú·¯¤˜ ÙÔ˘ ∞¡™ Î·È Ù˘ ™¶ ÛÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜ Û ™¢1
Correspondence:
Kyriaki Karavanaki 22A, Ellinikou Stratou St. 152 37, Filothei, Athens
Date of submission: 23-10-2006 Date of approval: 24-10-2006
¶·È‰È·ÙÚÈ΋ 2006;69:432-439
monitoring, as an index of autonomic neuropathy, was also performed and was analyzed with power
Results: In the first group, higher values of mean systolic blood pressure (SBP) (109.3 vs 106.5 mmHg,p=0.028) and mean DBP during night (77.8 vs 73.6 mmHg, p=0.035) and faster heart rate (pNN50:32.9% vs 19.9%, p<0.009) were observed, in comparison with the second group. Furthermore thepatients were divided into another two groups according to the absence of decrease (non-dippers) or thedecrease (dippers) of DBP during night. In the non-dippers’ group, mean heart rate RR during night(916.0 vs 764.4 ms, p=0.001) and parameter SDNN-i were faster (110.6 vs 88.1 ms, p=0.018) incomparison with the dippers’ group, while no difference was observed in terms of nocturnal UAE. DBPratio was positively related to HR (r=0.49, p=0.001).
Conclusions: In conclusion, early impairments of ANS and DBP seem to precede the development ofmicroalbuminuria and they possibly contribute to its pathogenesis.
Key words: Type 1 diabetes mellitus, arterial hypertension, autonomic dysfunction.
™˘ÓÙÔÌÔÁڷʛ˜
™¢1 ™·Î¯·Ú҉˘ ‰È·‚‹Ù˘ Ù‡Ô˘ 1 ∞¡™ ∞˘ÙfiÓÔÌÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ· ¢∞¡ ¢È·‚ËÙÈ΋ ·˘ÙfiÓÔÌË Ó¢ÚÔ¿ıÂÈ· ¢¡ ¢È·‚ËÙÈ΋ ÓÂÊÚÔ¿ıÂÈ· ∞¶ ∞ÚÙËÚȷ΋ ›ÂÛË ™¶ ™˘ÛÙÔÏÈ΋ ›ÂÛË ∞§√ ∞¤ÎÎÚÈÛË ÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡Ú· HRV ªÂÙ·‚ÏËÙfiÙËÙ· ηډȷÎÔ‡ Ú˘ıÌÔ‡
(heart rate variability) PSA ∞Ó¿Ï˘ÛË Î·Ù¿ power spectra
(power spectral analysis) HF ¶ÂÚÈÔ¯¤˜ ̆ „ËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ Ù˘ HRV
(high frequency) LF ¶ÂÚÈÔ¯¤˜ ̄ ·ÌËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ Ù˘ HRV
(low frequency) pNN50 ¶ÔÛÔÛÙfi ÙˆÓ ‰È·ÊÔÚÒÓ ÌÂٷ͇ ÙˆÓ
Û˘Ó¯fiÌÂÓˆÓ R-R ‰È·ÛÙËÌ¿ÙˆÓ >50 msηٿ ÙË Ó‡ÎÙ·
SDNN-i ª¤ÛË ÙÈÌ‹ ÙˆÓ ÛÙ·ıÂÚÒÓ ·ÔÎϛۈÓfiÏˆÓ ÙˆÓ ÊÈÏÙÚ·ÚÈÛÌ¤ÓˆÓ ‰È·ÛÙËÌ¿ÙˆÓRR ÁÈ· οı ‰È¿ÛÙËÌ· 5 mm ηٿ ÙˉȿÚÎÂÈ· ÙÔ˘ 24ÒÚÔ˘
EÈÛ·ÁˆÁ‹
∏ ·ıÔÁ¤ÓÂÈ· Ù˘ ‰È·‚ËÙÈ΋˜ ÓÂÊÚÔ¿ıÂÈ·˜(¢¡) Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ÂÌÊ¿ÓÈÛË ˘¤ÚÙ·Û˘.∫·Ù¿ ÙÔ ˘ÔÎÏÈÓÈÎfi ÛÙ¿‰ÈÔ Ù˘ ¢¡ Û ·ÛıÂÓ›˜Ì ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Ù‡Ô˘ 1 (™¢1), Û˘Ó‹ıˆ˜Ë ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›· ÚÔËÁÂ›Ù·È Ù˘ ˘¤Ú-Ù·Û˘ (1) ‹ Û·ÓÈfiÙÂÚ· ·Ó·Ù‡ÛÛÂÙ·È ·Ú¿Ï-ÏËÏ· (2,3). ∞ÓÙ›ıÂÙ·, Û ·ÛıÂÓ›˜ Ì ۷ί·Ú҉ˉȷ‚‹ÙË Ù‡Ô˘ 2 (™¢2), fiÙ·Ó ‰È·ÁÈÁÓÒÛÎÂÙ·È ËÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·, Ë ˘¤ÚÙ·ÛË Â›Ó·È ‹‰ËÂÁηÙÂÛÙË̤ÓË (4).
¶ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ Ì 24ˆÚË Î·Ù·ÁÚ·Ê‹Ù˘ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ (∞¶) Û ÂÓ‹ÏÈΘ Ì™¢1 (5,6), ·Ó·Ê¤ÚÔ˘Ó ˘„ËÏfiÙÂÚ˜ ÙÈ̤˜ ∞¶ η-Ù¿ ÙË Ó‡ÎÙ· Û ·ÛıÂÓ›˜ Ì ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘-
Ú›·, Û ۇÁÎÚÈÛË Ì ÂΛÓÔ˘˜ ¯ˆÚ›˜ ÌÈÎÚÔÏ¢-Έ̷ÙÈÓÔ˘Ú›·. ∂›Û˘, ÔÈ Lurbe Î·È Û˘Ó (7) ·Ó·-ʤÚÔ˘Ó fiÙÈ Ë ·‡ÍËÛË Ù˘ ™¶ Û ‰È·‚ËÙÈÎÔ‡˜ÂÊ‹‚Ô˘˜ ηٿ ÙË Ó‡ÎÙ· ÚÔËÁÂ›Ù·È Ù˘ ÂÌÊ¿ÓÈ-Û˘ Ù˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜ Î·È fiÙÈ Â›Ó·ÈÈı·Ófi Ó· Û˘Ì‚¿ÏÏÂÈ ÛÙËÓ ·ıÔÁ¤ÓÂÈ¿ Ù˘.
√È ‰È·Î˘Ì¿ÓÛÂȘ Ù˘ ∞¶ ηٿ ÙË ‰È¿ÚÎÂÈ·ÙÔ˘ 24ÒÚÔ˘ ¤¯Ô˘Ó Û˘Û¯ÂÙÈÛÙ› Ì ‰È·Ù·Ú·¯¤˜Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ Ó¢ÚÈÎÔ‡ Û˘-ÛÙ‹Ì·ÙÔ˜ (∞¡™) (8).
™Â ÂÓ‹ÏÈΘ ‰È·‚ËÙÈÎÔ‡˜ ·ÛıÂÓ›˜ Ì ·Ú¯fi-ÌÂÓÔ ÛÙ¿‰ÈÔ ¢¡, Ë ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›· ¤¯ÂÈÛ˘Û¯ÂÙÈÛÙ› Ì ÙËÓ ·ÚÔ˘Û›· ‰È·Ù·Ú·¯ÒÓ ÙÔ˘∞¡™ (9). ™Â ‰È·‚ËÙÈο ·È‰È¿, Ë ·ÚÔ˘Û›· ·˘-ÍËÌ¤ÓˆÓ ÙÈÌÒÓ ∞§√ Û˘Û¯ÂÙÈ˙fiÙ·Ó Ì ÙËÓ ·-ÚÔ˘Û›· ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ Î·È˘„ËÏÒÓ ÙÈÌÒÓ ∞¶ (10,11).
∆›ıÂÙ·È, Û˘ÓÂÒ˜, ÙÔ ÂÚÒÙËÌ· Â¿Ó Ë ‰È·‚Ë-ÙÈ΋ ·˘ÙfiÓÔÌË Ó¢ÚÔ¿ıÂÈ· (¢∞¡) Û˘Ì‚¿ÏÏÂÈÛÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ÓÂÊÚÔ¿ıÂÈ·˜, ̤ۈ ‰È·-Ù·Ú·¯ÒÓ ÛÙË ‰È·Î‡Ì·ÓÛË Ù˘ ∞¶ ηٿ ÙË ‰È¿Ú-ÎÂÈ· ÙÔ˘ 24ÒÚÔ˘.
ª¤¯ÚÈ ÚfiÛÊ·Ù·, ÔÈ ÌÂϤÙ˜ Ô˘ ÂÚ¢ÓÔ‡ÓÙË Û˘Û¯¤ÙÈÛË ·ıÔÏÔÁÈÎÒÓ ‰ÂÈÎÙÒÓ ¢∞¡ Ì ÙËÌÂÙ·‚ÔÏ‹ Ù˘ ∞¶ ηٿ ÙË Ó‡ÎÙ· Û ·ÛıÂÓ›˜Ì ‹ ¯ˆÚ›˜ ÚˆÙÂ˚ÓÔ˘Ú›·, ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÂÓ‹-ÏÈΘ (12,13). °È’ ·˘ÙÔ‡˜ ÙÔ˘˜ ÏfiÁÔ˘˜ ۯ‰ȿ-ÛÙËÎÂ Ë ·ÚÔ‡Û· ÌÂϤÙË, Ì ÛÎÔfi Ó· ÌÂÏÂÙË-ı› Û ÂÊ‹‚Ô˘˜ Ì ™¢1 Ë Û˘Û¯¤ÙÈÛË Ù˘ ÌÂÙ·-‚ÔÏ‹˜ Ù˘ ∞¢¶ ηٿ ÙË Ó‡ÎÙ· Ì ÙË ÏÂÈÙÔ˘Ú-Á›· ÙÔ˘ ∞¡™ Î·È ÙËÓ ·¤ÎÎÚÈÛË ÌÈÎÚÔÏÂ˘Îˆ-Ì·Ù›Ó˘ ηٿ ÙË Ó‡ÎÙ·.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ
∏ ·ÚÔ‡Û· ÌÂϤÙË Â›Ó·È Úfi‰ÚÔÌË ÚÔÔÙÈ΋˜ Ì·-ÎÚÔ¯ÚfiÓÈ·˜ ÌÂϤÙ˘ Ù˘ ¢∞¡ Î·È ¢¡, Ô˘ ‰ÈÂÓÂÚÁ›ٷÈ
434 ∫. K·Ú·‚·Ó¿ÎË Î·È Û˘Ó.
Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ‰È¿ÚÎÂÈ· ÓfiÛÔ˘ >5 ¯ÚfiÓÈ·, Ù·
ÔÔ›· ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È ÛÙÔ ¢È·‚ËÙÔÏÔÁÈÎfi ∂͈ÙÂÚÈ-
Îfi È·ÙÚÂ›Ô Ù˘ ÎÏÈÓÈ΋˜ Ì·˜. ∞˘Ù‹ Ë ÌÂϤÙË ÂÚÈÏ·Ì‚¿-
ÓÂÈ Ù· ÚÒÙ· ·ÔÙÂϤÛÌ·Ù· Ô˘ ÂÏ‹ÊıËÛ·Ó ÌÂÙ¿ ·fi
·Ú·ÎÔÏÔ‡ıËÛË 22 ·ÛıÂÓÒÓ.
ªÂıÔ‰ÔÏÔÁ›·
∞. MÂϤÙË Ù˘ 24ˆÚ˘ ‰È·Î‡Ì·ÓÛ˘ Ù˘ ∞¶: ∆ÔÔıÂ-
Ù‹ıËΠHolter ·ÚÙËÚȷ΋˜ ›ÂÛ˘ ÁÈ· 24 ÒÚ˜, Ô˘ η-
Ù¤ÁÚ·Ê ÙÈ̤˜ ·Ó¿ 30 min ÙËÓ Ë̤ڷ Î·È ·Ó¿ 1 ÒÚ· ÙË
Ó‡ÎÙ·. ÃÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Û˘Û΢‹ Holter Medilog
ABP. ªÂÏÂÙ‹ıËÎ·Ó ÔÈ ÂÍ‹˜ ·Ú¿ÌÂÙÚÔÈ Ù˘ ∞¶: ·)
Max Î·È Ì¤ÛË Û˘ÛÙÔÏÈ΋ ›ÂÛË 24ÒÚÔ˘ Î·È SD, ‚) ªax
Î·È Ì¤ÛË ‰È·ÛÙÔÏÈ΋ ›ÂÛË 24ˆÚÔ˘ Î·È SD, Á) ̤ÛË Û˘-
ÛÙÔÏÈ΋ ›ÂÛË Ë̤ڷ˜ (12ÒÚÔ˘) Î·È SD, ‰) ̤ÛË ‰È·-
ÛÙÔÏÈ΋ ›ÂÛË Ë̤ڷ˜ (12ÒÚÔ˘) Î·È SD, Â) ̤ÛË Û˘-
ÛÙÔÏÈ΋ ›ÂÛË Ó‡ÎÙ·˜ (12ÒÚÔ˘) Î·È SD, ÛÙ) ̤ÛË ‰È·-
ÛÙÔÏÈ΋ ›ÂÛË Ó‡ÎÙ·˜ (12ÒÚÔ˘) Î·È SD, ˙) ËÏ›ÎÔ Û˘-
ÛÙÔÏÈ΋˜ ›ÂÛ˘ Ó‡ÎÙ·˜/Ë̤ڷ˜ Î·È Ë) ËÏ›ÎÔ ‰È·ÛÙÔ-
ÏÈ΋˜ ›ÂÛ˘ Ó‡ÎÙ·˜/Ë̤ڷ˜. º˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ÁÈ·
ÙËÓ ·ÚÙËÚȷ΋ ›ÂÛË ÂÏ‹ÊıËÛ·Ó ·fi ÚÔËÁÔ‡ÌÂÓ˜
ÌÂϤÙ˜ (14).
µ. ªÂϤÙË Ù˘ 24ˆÚ˘ ÌÂÙ·‚ÏËÙfiÙËÙ·˜ ÙÔ˘ ηډȷÎÔ‡
Ú˘ıÌÔ‡ (Heart rate variability) (ˆ˜ ‰Â›ÎÙ˘ ·˘ÙfiÓÔÌ˘
Ó¢ÚÔ¿ıÂÈ·˜) Î·È ·Ó¿Ï˘Û‹ ÙÔ˘ Ì ÙË Ì¤ıÔ‰Ô power
spectra. ∆ÔÔıÂÙ‹ıËΠHolter ηډȷÎÔ‡ Ú˘ıÌÔ‡ ηٿ
ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ 24ÒÚÔ˘. ÃÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Û˘Û΢‹
Holter Ù‡Ô˘ Medilog 4,500 P (Oxford). √È Ù·Èӛ˜ ·Ó·-
χıËÎ·Ó Ì ÙÔ ÚfiÁÚ·ÌÌ· Medilog Excel, version 5.0
(Oxford). º˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ÁÈ· ÙȘ ·Ú·Ì¤ÙÚÔ˘˜ ÙÔ˘
ηډȷÎÔ‡ Ú˘ıÌÔ‡ Ì ÙË Ì¤ıÔ‰Ô power spectra ÂÏ‹-
ÊıËÛ·Ó ·fi ÚÔËÁÔ‡ÌÂÓ˜ ÌÂϤÙ˜ (8).
∏ ÌÂϤÙË Ù˘ ÌÂÙ·‚ÏËÙfiÙËÙ·˜ ÙÔ˘ ηډȷÎÔ‡ Ú˘ı-
ÌÔ‡ Ì ÙË Ì¤ıÔ‰Ô PSA ÂÚÈÂÏ¿Ì‚·ÓÂ: i) ÙËÓ ·Ó¿Ï˘ÛË
ÛÙÔÓ ¯ÚfiÓÔ (time domain analysis) Î·È ii) ÙËÓ ·Ó¿Ï˘ÛË
ηٿ Û˘¯ÓfiÙËÙ˜ (frequency domain analysis) (15). ∏
·Ó¿Ï˘ÛË ÛÙÔÓ ¯ÚfiÓÔ ·Ó·Ê¤ÚÂÙ·È ÛÙË ÌÂϤÙË Ù˘ ÛÙ·-
ıÂÚ‹˜ ·fiÎÏÈÛ˘ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ ÛÙË ÌÔÓ¿‰·
ÙÔ˘ ¯ÚfiÓÔ˘ (R-R intervals/time) (16). √È ·Ú¿ÌÂÙÚÔÈ
Ô˘ ÌÂÏÂÙÒÓÙ·È Â›Ó·È ÔÈ ·ÎfiÏÔ˘ı˜: ·) ª¤ÛÔ˜ RR (̤-
ÛË ÙÈÌ‹ fiÏˆÓ ÙˆÓ ÊÈÏÙÚ·ÚÈÛÌ¤ÓˆÓ ‰È·ÛÙËÌ¿ÙˆÓ RR),
‚) SDNN [ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË (SD) ÙˆÓ ‰È·ÛÙËÌ¿ÙˆÓ
R-R intervals ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ 24ÒÚÔ˘], Á) SDNNi
(̤ÛË ÙÈÌ‹ ÙˆÓ ÛÙ·ıÂÚÒÓ ·ÔÎÏ›ÛÂˆÓ fiÏˆÓ ÙˆÓ ÊÈÏ-
ÙÚ·ÚÈÛÌ¤ÓˆÓ ‰È·ÛÙËÌ¿ÙˆÓ RR ÁÈ· οı ‰È¿ÛÙËÌ· 5 ÏÂ-
ÙÒÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ 24ÒÚÔ˘), ‰) SDAN-i (ÛÙ·-
ıÂÚ‹ ·fiÎÏÈÛË ÙˆÓ SDNNi), Â) pNN50 (ÔÛÔÛÙfi ÙˆÓ
‰È·ÊÔÚÒÓ ÌÂٷ͇ ÙˆÓ Û˘Ó¯fiÌÂÓˆÓ R-R ‰È·ÛÙËÌ¿ÙˆÓ
>50 ms ηٿ ÙË Ó‡ÎÙ·) Î·È ÛÙ) r-MSSD (ÙÂÙÚ·ÁˆÓÈ΋
Ú›˙· ÙÔ˘ ̤ÛÔ˘ fiÚÔ˘ ÙÔ˘ ·ıÚÔ›ÛÌ·ÙÔ˜ ÙˆÓ ÙÂÙÚ·ÁÒÓˆÓ
Ù˘ pNN50). ∆o SDAN-i ·ÔÙÂÏ› ÙËÓ Î·Ï‡ÙÂÚË ·Ú¿-
ÌÂÙÚÔ Ì¤ÙÚËÛ˘ Ù˘ ÎÈÚη‰›Ô˘ ÌÂÙ·‚ÔÏ‹˜ ÙÔ˘ ηډȷ-
ÎÔ‡ Ú˘ıÌÔ‡ (16), ÂÓÒ ÔÈ ·Ú¿ÌÂÙÚÔÈ pNN50 ηÈ
rMSSD ıˆÚÔ‡ÓÙ·È ¿ÚÈÛÙÔÈ ‰Â›ÎÙ˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘
·Ú·Û˘Ì·ıËÙÈÎÔ‡ (16).
°È· ÙËÓ ·Ó¿Ï˘ÛË Ù˘ HRV ηٿ Û˘¯ÓfiÙËÙ˜ ¯ÚËÛÈ-
ÌÔÔÈÂ›Ù·È Ë Ù·¯Â›· ·Ó¿Ï˘ÛË Î·Ù¿ Fourier (17). ™‡Ì-
ʈӷ Ì ·˘Ù‹, ÔÈ ‰È·Î˘Ì¿ÓÛÂȘ ÛÙÔ Â‡ÚÔ˜ ÙˆÓ ‰È·ÛÙË-
Ì¿ÙˆÓ R-R ÌÂÙ·ÙÚ¤ÔÓÙ·È ÛÂ Î˘Ì·ÙÔÌÔÚʤ˜ Û˘¯ÓÔÙ‹-
ÙˆÓ Î·È ÂÎÊÚ¿˙Ô˘Ó ÙË ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ Û˘Ì·ıËÙÈÎÔ‡
Î·È ÙÔ˘ ·Ú·Û˘Ì·ıËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (17). ªÂÏÂ-
ÙÒÓÙ·È ‰‡Ô ÔÌ¿‰Â˜ Û˘¯ÓÔًوÓ: ·) Ë ÔÌ¿‰· ˘„ËÏÒÓ
Û˘¯ÓÔÙ‹ÙˆÓ (HF: 0,15-0,50 Hz), Ô˘ ·ÔÙÂÏÔ‡Ó ‰Â›-
ÎÙ˜ Ù˘ ·Ú·Û˘Ì·ıËÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, ÂÍ·ÚÙÒÓÙ·È
·fi ÙËÓ ·Ó·ÓÔ‹ Î·È Â›Ó·È ˘„ËÏfiÙÂÚ˜ ÛÙËÓ ‡ÙÈ· ı¤-
ÛË (17) Î·È ‚) Ë ÔÌ¿‰· ¯·ÌËÏÒÓ Û˘¯ÓÔÙ‹ÙˆÓ (LF: 0,05-
0,15 Hz), Ë ÔÔ›· ÛÙËÓ ‡ÙÈ· ı¤ÛË ÂÍ·ÚÙ¿Ù·È Î˘Ú›ˆ˜
·fi ÙË ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ·Ú·Û˘Ì·ıËÙÈÎÔ‡, ÂÓÒ ÛÙËÓ
fiÚıÈ· ı¤ÛË ÂËÚ¿˙ÂÙ·È ·fi ·ÏÏËÏ›‰Ú·ÛË Û˘Ì·-
ıËÙÈÎÔ‡ Î·È ·Ú·Û˘Ì·ıËÙÈÎÔ‡ (17). ∂ÈϤÔÓ, ˘Ô-
ÏÔÁ›ÛÙËΠÙÔ ËÏ›ÎÔ HF/LF Ô˘ ·ÔÙÂÏ›, ›Û˘,
·ÍÈfiÈÛÙÔ ‰Â›ÎÙË ÈÛÔÚÚÔ›·˜ ÌÂٷ͇ Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜
Û˘Ì·ıËÙÈÎÔ‡-·Ú·Û˘Ì·ıËÙÈÎÔ‡ (17). ø˜ ÂÚ›Ô‰Ô˜
‡ÓÔ˘ ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ÔÚ›ÛÙËΠÙÔ ‰È¿ÛÙËÌ· 10
ÌÌ-8 Ì, ÂÓÒ Ë Î·Ù·ÁÚ·Ê‹ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ ηÈ
Ù˘ ∞¶ ¤ÁÈÓ Ì Holter.
°. ∫·Ú‰ÈÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË: ·) ÀÂÚ˯ÔÁÚ·ÊÈÎfi˜
¤ÏÂÁ¯Ô˜ ηډȿ˜ (2D-Echo Doppler). ÃÚËÛÈÌÔÔÈ‹ıË-
ÎÂ Ë Û˘Û΢‹ ATL 5000 HDI. ªÂÏÂÙ‹ıËÎ·Ó ÔÈ ÂÍ‹˜
·Ú¿ÌÂÙÚÔÈ: i) ‰È·ÛÙ¿ÛÂȘ Ô›ÛıÈÔ˘ ÙÔȯÒÌ·ÙÔ˜ ·ÚÈ-
ÛÙÂÚ‹˜ ÎÔÈÏ›·˜, ii) ÌÂÛÔÎÔÈÏÈ·Îfi ‰È¿ÊÚ·ÁÌ· ·ÚÈÛÙÂÚ‹˜
ÎÔÈÏ›·˜, iii) ·ÚÈÛÙÂÚfi˜ ÎfiÏÔ˜, iv) ÙÂÏԉȷÛÙÔÏÈ΋ ‰È¿-
ÌÂÙÚÔ˜ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜, v) ÙÂÏÔÛ˘ÛÙÔÏÈ΋ ‰È¿ÌÂÙÚÔ˜
·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ Î·È vi) Ì¿˙· ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜. ‚)
∏ÏÂÎÙÚÔηډÈÔÁÚ¿ÊËÌ·: XÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Û˘-
Û΢‹ Nihon Kohden Cardiofax V.
¢. ™˘ÏÏÔÁ‹ Ô‡ÚˆÓ 24ÒÚÔ˘, Ô˘ ¯ˆÚ›ÛÙËΠÛÂ: ·)
12ˆÚË Û˘ÏÏÔÁ‹ Ë̤ڷ˜ Î·È ‚) 12ˆÚË Û˘ÏÏÔÁ‹ Ó‡ÎÙ·˜.
™Â οı 12ˆÚË Û˘ÏÏÔÁ‹, ηıÒ˜ Î·È ÛÙÔ Û‡ÓÔÏÔ ÙˆÓ
Ô‡ÚˆÓ 24ÒÚÔ˘, ÌÂÙÚ‹ıËÎ·Ó o fiÁÎÔ˜ ÙˆÓ Ô‡ÚˆÓ (˘Ô-
ÏÔÁÈÛÌfi˜ Ú˘ıÌÔ‡ ‰ÈÔ‡ÚËÛ˘) Î·È Ë ÌÈÎÚÔÏÂ˘ÎˆÌ·Ù›ÓË
Ô‡ÚˆÓ.
∂. √ ÁÏ˘Î·ÈÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÂÎÙÈÌ‹ıËΠ̠ÙË Ì¤ÙÚËÛË
Ù˘ ÁÏ˘ÎÔ˙˘ÏȈ̤Ó˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ HbA1c ÙËÓ Ë̤ڷ
Ù˘ ÌÂϤÙ˘, ηıÒ˜ Î·È ÙË Ì¤ÛË ÙÈÌ‹ Ù˘ ∏b∞1c ηٿ
ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ 12 ÌËÓÒÓ.
™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË
∏ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ¤ÁÈÓ Ì ÙÔ ÚfiÁÚ·ÌÌ·
SPSS-PC for Windows. ŒÁÈÓ ۇÁÎÚÈÛË ÙˆÓ ·Ú·Ì¤ÙÚˆÓ
ÌÂٷ͇ ‰‡Ô ÔÌ¿‰ˆÓ ‰È·‚ËÙÈÎÒÓ ·ÛıÂÓÒÓ Ì ÙÔ
unpaired t-test. √È Û˘Û¯ÂÙ›ÛÂȘ ÙˆÓ ·Ú·Ì¤ÙÚˆÓ ÚˆÙÂ-
˚ÓÔ˘Ú›·˜, ∞¶ Î·È HRV Ì ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓfiÛÔ˘ Î·È ÙËÓ
∏bA1c ¤ÁÈÓ·Ó Ì ÙË ¯Ú‹ÛË ÙˆÓ Û˘Û¯ÂÙ›ÛÂˆÓ Î·Ù¿
Pearson. ∏ ‰È·‰Ô¯È΋ ÔÏÏ·Ï‹ Û˘Û¯¤ÙÈÛË (stepwise
multiple regression) ¯ÚËÛÈÌÔÔÈ‹ıËΠÁÈ· ÙÔÓ ÚÔÛ‰ÈÔ-
ÚÈÛÌfi ÙˆÓ ÛËÌ·ÓÙÈÎfiÙÂÚˆÓ ·Ú·Ì¤ÙÚˆÓ Ô˘ Û˘Û¯ÂÙ›-
˙ÔÓÙ·È Ì ̛· ÌÂÙ·‚ÏËÙ‹ (.¯. ∞¶ ηٿ ÙË Ó‡ÎÙ·). ∏
‰È¿ÌÂÛË ÙÈÌ‹ ·¤ÎÎÚÈÛ˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡Ú·
12ÒÚÔ˘ (Ó‡ÎÙ·˜) Û ‰‡Ô Û˘ÏÏÔÁ¤˜ Ô‡ÚˆÓ ÙÔ˘˜ ÙÂÏ¢-
Ù·›Ô˘˜ 6 Ì‹Ó˜ ‹Ù·Ó 7,5 Ìg/min. ªÂ ‚¿ÛË ·˘Ù‹ ÙËÓ ÙÈ-
Ì‹, ¯ˆÚ›ÛÙËÎ·Ó ÔÈ ‰È·‚ËÙÈÎÔ› ¤ÊË‚ÔÈ Û 2 ÔÌ¿‰Â˜: π) ÌÂ
∞§√ ≥7,5 Ìg/min Î·È ππ) Ì ∞§√ <7,5 Ìg/min.
AÔÙÂϤÛÌ·Ù·
∆· ‰ËÌÔÁÚ·ÊÈο ÛÙÔȯ›· ÙˆÓ ÂÊ‹‚ˆÓ ÌÂ
™¢1 Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó ˆ˜ ÂÍ‹˜: ̤ÛË
ËÏÈΛ· 16,7±4,1 ¤ÙË (‡ÚÔ˜: 11,0-22,0 ¤ÙË), ̤ÛË
ËÏÈΛ· ‰È¿ÁÓˆÛ˘ 9,3±4,2 ¤ÙË, ̤ÛË ‰È¿ÚÎÂÈ·
ÓfiÛÔ˘ 8,5±3,3 ¤ÙË, ̤ÛË ‰fiÛË Ù˘ ÈÓÛÔ˘Ï›Ó˘
¶·È‰È·ÙÚÈ΋ 2006;69:432-439
435¢È·Ù·Ú·¯¤˜ ÙÔ˘ ∞¡™ Î·È Ù˘ ™¶ ÛÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜ Û ™¢1
0,94±0,3 πU/kg/Ë̤ڷ. ∏ ̤ÛË ÙÈÌ‹ Ù˘ HbA1c
ηٿ ÙËÓ Ë̤ڷ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó 7,7±1,2% ηÈ
Ë Ì¤ÛË ÙÈÌ‹ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ
12 ÌËÓÒÓ ‹Ù·Ó 8,2±1,3%. ∏ ̤ÛË ÙÈÌ‹ Ù˘ ∞¶
24ÒÚÔ˘ ‹Ù·Ó 110,3/72,5 mmHg, ÂÓÒ Ë Ì¤ÛË ÙÈ-
Ì‹ ∞¶ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ë̤ڷ˜ ‹Ù·Ó
117,8/72,5 mmHg Î·È Ù˘ Ó‡ÎÙ·˜ 106,4/58,7
mmHg, ·ÓÙ›ÛÙÔȯ·.
√È ‰È·‚ËÙÈÎÔ› ¤ÊË‚ÔÈ ¯ˆÚ›ÛÙËÎ·Ó Û ‰‡Ô
ÔÌ¿‰Â˜, ·Ó¿ÏÔÁ· Ì ÙË ‰È¿ÌÂÛË ÙÈÌ‹ ÌÈÎÚÔÏ¢-
Έ̷ٛÓ˘ Ô‡ÚˆÓ 12ÒÚÔ˘ (ÔÌ¿‰· π: ≥7,5
Ìg/min, ÔÌ¿‰· ππ: <7,5 Ìg/min). ™˘ÁÎÚ›ÓÔÓÙ·˜
ÙËÓ ÔÌ¿‰· π Ì ÙËÓ ÔÌ¿‰· Iπ (¶›Ó·Î·˜ 1), ‰È·-
ÈÛÙÒıËÎ·Ó ˘„ËÏfiÙÂÚ˜ ÙÈ̤˜ ̤Û˘ ™¶
(109,3 vs. 106,5 mmHg, p=0,028) Î·È Ì¤Û˘ ¢¶
ηٿ ÙË Ó‡ÎÙ· (77,8 vs. 73,6 mmHg, p=0,035),
·ÓÙ›ÛÙÔȯ·. ∂›Û˘, ‰È·ÈÛÙÒıËΠٷ¯‡ÙÂÚÔ˜
ηډȷÎfi˜ Ú˘ıÌfi˜ (pNN50) ÛÙËÓ ÔÌ¿‰· π Û˘-
ÁÎÚÈÙÈο Ì ÙËÓ ÔÌ¿‰· πI (32,9% vs. 19,9%,
p<0,009). √È ‰‡Ô ÔÌ¿‰Â˜ ‰ÂÓ ‰È¤ÊÂÚ·Ó ÛÙË
‰È¿ÚÎÂÈ· Ù˘ ÓfiÛÔ˘ ‹ ÛÙȘ ÙÈ̤˜ Ù˘ ∏bA1c.
™ÙË Û˘Ó¤¯ÂÈ·, ÔÈ ‰È·‚ËÙÈÎÔ› ¤ÊË‚ÔÈ ¯ˆÚ›-
ÛÙËÎ·Ó Û ‰‡Ô ˘ÔÔÌ¿‰Â˜, ·Ó¿ÏÔÁ· Ì ÙËÓ
·Ô˘Û›· ÙÒÛ˘ (non-dippers) ‹ ÙËÓ ÙÒÛË
(dippers) Ù˘ ¢¶ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡ÎÙ·˜
(¶›Ó·Î·˜ 2). ∏ ÌÂÙ·‚ÔÏ‹ Ù˘ ¢¶ ›ÂÛ˘ ÙË Ó‡-
ÎÙ· Û˘ÁÎÚÈÙÈο Ì ÙËÓ Ë̤ڷ ÂÎÙÈÌ‹ıËΠÌÂ
ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ ËÏ›ÎÔ˘ ¢¶ Ó‡ÎÙ·˜/Ë̤-
Ú·˜. ∏ 1Ë ÔÌ¿‰· (non-dippers) ·ÚÔ˘Û›·˙ η-
Ù¿ ÙË Ó‡ÎÙ· ˘„ËÏfiÙÂÚË ÙÈÌ‹ ̤ÛÔ˘ ηډȷÎÔ‡
Ú˘ıÌÔ‡ (RR) (916,0 vs. 764,4 ms, p=0,001) ηÈ
˘„ËÏfiÙÂÚË ·Ú¿ÌÂÙÚÔ SDNN-i (110,6 vs. 88,1
ms, p=0,018) Û˘ÁÎÚÈÙÈο Ì ÙËÓ ÔÌ¿‰· µ, ÂÓÒ
‰ÂÓ ˘‹Ú¯Â ‰È·ÊÔÚ¿ ÛÙËÓ ·¤ÎÎÚÈÛË ÏÂ˘Îˆ-
Ì·Ù›Ó˘ ÛÙ· Ô‡Ú· (∞§√).
∆Ô ËÏ›ÎÔ Ù˘ ¢¶ Ó‡ÎÙ·˜/Ë̤ڷ˜ Û˘Û¯ÂÙÈ-
˙fiÙ·Ó ıÂÙÈο Ì ÙÔÓ Î·Ú‰È·Îfi Ú˘ıÌfi RR (r=0,49,
p=0,001) Î·È Ì ÙË Ó˘ÎÙÂÚÈÓ‹ ·¤ÎÎÚÈÛË ∞§√
(r=0,35, p=0,032). ∫·Ù¿ ÙË ‰ÔÎÈÌ·Û›· ÔÏÏ·-
Ï‹˜ ‰È·‰Ô¯È΋˜ Û˘Û¯¤ÙÈÛ˘, Ë ÛËÌ·ÓÙÈÎfiÙË ·-
Ú¿ÌÂÙÚÔ˜ Ô˘ ÂËÚ¿˙ÂÈ ÙË ÌÂÙ·‚ÔÏ‹ Ù˘ ‰È·-
ÛÙÔÏÈ΋˜ ›ÂÛ˘ ηٿ ÙË Ó‡ÎÙ· ‹Ù·Ó Ë rMSSD
ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ (b=0,44, p=0,001).
™˘˙‹ÙËÛË
™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ‰È·ÈÛÙÒıËΠfiÙÈ
¤ÊË‚ÔÈ Ì ™¢1 Ì ·¤ÎÎÚÈÛË ÏÂ˘ÎˆÌ·Ù›Ó˘
ÛÙ· Ô‡Ú· ÛÙ· ·ÓÒÙÂÚ· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· ›¯·Ó
˘„ËÏ‹ ¢¶ ηٿ ÙË Ó‡ÎÙ·, ·Ú¿ÏÏËÏ· Ì ‰È·-
Ù·Ú·¯¤˜ ÙÔ˘ ∞¡™. ∞Ó¿ÏÔÁ˜ ÌÂϤÙ˜ Û ‰È·‚Ë-
ÙÈο ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ‰ÂÓ ˘¿Ú¯Ô˘Ó ·Ó·ÊÔ-
ÚÈο Ì ÙËÓ 24ˆÚË ‰È·Î‡Ì·ÓÛË Ù˘ ∞¶ Î·È ÙË
¶·È‰È·ÙÚÈ΋ 2006;69:432-439
¶›Ó·Î·˜ 1. ™‡ÁÎÚÈÛË ÔÌ¿‰ˆÓ ·Ó¿ÏÔÁ· Ì ÙËÓ ·¤ÎÎÚÈÛË ÌÈÎÚÔÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ó˘ÎÙÂÚÈÓ¿ Ô‡Ú·
ªÈÎÚÔÏÂ˘ÎˆÌ·Ù›ÓË ªÈÎÚÔÏÂ˘ÎˆÌ·Ù›ÓË pÔ‡ÚˆÓ Ó‡ÎÙ·˜ (12ÒÚÔ˘) Ô‡ÚˆÓ Ó‡ÎÙ·˜ (12ÒÚÔ˘)
(≥7,5 Ìg/min) (<7,5 Ìg/min)n=11 n=11
ª¤Û˱SD ª¤Û˱SD
HÏÈΛ· (¤ÙË) 16,1±3,4 19,6±4,1 0,003¢È¿ÚÎÂÈ· (¤ÙË) 8,3±3,5 8,6±3,0 ª™µªπ (kg/cm2) 22,6±2,8 21,7±2,4 M™∏bA1c (%) 8,0±1,4 8,2±1,2 M™¢fiÛË ÈÓÛÔ˘Ï›Ó˘ (πU/kg/Ë̤ڷ) 0,95±0,28 0,93±0,25 ª™ª¤ÛË 24ˆÚË ™¶ (mmHg) 112,0±11,0 108,0±6,9 ª™ª¤ÛË 24ˆÚË ¢¶ (mmHg) 65,6±9,7 62,4±5,4 ª™™¶ (Ó‡ÎÙ·˜) mmHg 109,3±11,5 106,5±8,2 0,028¢¶ (Ó‡ÎÙ·˜) mmHg 77,8±6,7 73,6±6,5 0,035§fiÁÔ˜ ¢¶ Ó‡ÎÙ·˜/Ë̤ڷ˜ 0,83±15,0 0,80±12,0 ª™ª¤ÛË Ó˘ÎÙÂÚÈÓ‹ ∞§√ (Ìg/min) 14,9±4,5 3,2±1,9 0,001ªÂÙ·‚ÏËÙfiÙËÙ· ηډȷÎÔ‡ Ú˘ıÌÔ‡pNN50 (%) 32,9±14,9 19,9±17,5 0,009LF/HF 1,5±0,68 2,0±1,13 0,048
™¶: ™˘ÛÙÔÏÈ΋ ›ÂÛË ¢¶: ¢È·ÛÙÔÏÈ΋ ›ÂÛË µªπ: ¢Â›ÎÙ˘ Ì¿˙·˜ ÛÒÌ·ÙÔ˜ ∞§√: ∞¤ÎÎÚÈÛË ÏÂ˘ÎˆÌ·Ù›Ó˘ Ô‡ÚˆÓ pNN50: ¶ÔÛÔÛÙfi ÙˆÓ ‰È·ÊÔÚÒÓ ÌÂٷ͇ ÙˆÓ Û˘Ó¯fiÌÂÓˆÓ R-R ‰È·ÛÙËÌ¿ÙˆÓ >50 ms ηٿ ÙË Ó‡ÎÙ· ∏F/LF: ¶ËÏ›ÎÔ ¯·ÌËÏÒÓ/˘„ËÏÒÓ Û˘¯ÓÔÙ‹ÙˆÓ Ù˘ HRV ηٿ ÙË Ó‡ÎÙ· M™: MË ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈÎfi
436 ∫. K·Ú·‚·Ó¿ÎË Î·È Û˘Ó.
Û˘Û¯¤ÙÈÛ‹ Ù˘ Ì ÙÔ ∞¡™, ÂÓÒ ÔÈ ÌÂϤÙ˜ ÙÔ˘
∞¡™ Ì ÙË Ì¤ıÔ‰Ô PSA Â›Ó·È Ôχ ÂÚÈÔÚÈṲ̂-
Ó˜ ÛÙȘ ËÏÈ˘ ·˘Ù¤˜ (18-20). ∂›Û˘, ‰ÂÓ ¤¯ÂÈ
Á›ÓÂÈ ÌÂϤÙË Û ÂÊ‹‚Ô˘˜ Ì ™¢ Û¯ÂÙÈο Ì ÙËÓ
Èı·Ó‹ ·ÏÏËÏ›‰Ú·ÛË ÙÔ˘ ∞¡™ Î·È Ù˘
24ˆÚ˘ ‰È·Î‡Ì·ÓÛ˘ Ù˘ ∞¶ ÛÙËÓ ÂÌÊ¿ÓÈÛË
Ù˘ ∞§√, ÂÓÒ ·ÓÙ›ÛÙÔȯ˜ ÌÂϤÙ˜ Û ÂÓ‹ÏÈΘ
Ì ™¢1 Â›Ó·È ÂÚÈÔÚÈṲ̂Ó˜ (10,13,21).
™˘Ìو̷ÙÔÏÔÁ›· ÎÏÈÓÈÎÔ‡ ∞¡™ ‰ÂÓ ÂÌÊ·-
Ó›˙ÂÙ·È Û˘Ó‹ıˆ˜ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ÌÂ
™¢1, fï˜, ÂËÚ·Ṳ̂ÓÔÈ ‰Â›ÎÙ˜ ¢∞¡ ¤¯Ô˘Ó
·Ó·ÊÂÚı› ÓˆÚ›˜ ÛÙËÓ ÔÚ›· Ù˘ ÓfiÛÔ˘
(10,11). ∏ ÌÂϤÙË Ù˘ ÌÂÙ·‚ÏËÙfiÙËÙ·˜ ÙÔ˘
ηډȷÎÔ‡ Ú˘ıÌÔ‡ (heart rate variability -
HRV) Ì ÙËÓ ·Ó¿Ï˘ÛË Î·Ù¿ power spectra
(PSA) Â›Ó·È Ì›· ÚfiÛÊ·ÙË Ì¤ıÔ‰Ô˜ ÌÂϤÙ˘
Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘ ∞¡™. ™Â ·È‰È¿ Î·È ÂÊ‹-
‚Ô˘˜ Ì ™¢1 ¤¯ÂÈ ·Ó·ÊÂÚı› Ë ‡·ÚÍË ·ıÔÏÔ-
ÁÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ ∏RV (18-20), ÔÈ Ôԛ˜ Û˘-
Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓfiÛÔ˘ (34) ηÈ
Ì ÙȘ ÙÈ̤˜ Ù˘ HbA1c (18). ¢È·Ù·Ú·¯¤˜ ÙˆÓ
·Ú·Ì¤ÙÚˆÓ ˘„ËÏ‹˜ (HF) Î·È ¯·ÌËÏ‹˜ Û˘¯Ófi-
ÙËÙ·˜ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ (LF) ¤¯Ô˘Ó Û˘-
Û¯ÂÙÈÛÙ› Ì ÙȘ ÌÂÙ·‚ÔϤ˜ Ù˘ ·ÚÙËÚȷ΋˜
›ÂÛ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ 24ÒÚÔ˘ (22), η-
ıÒ˜ ›Û˘ Î·È Ì ٷ ›‰· Ù˘ ÏÂ˘ÎˆÌ·Ù›-
Ó˘ Ô‡ÚˆÓ 24ÒÚÔ˘ Û ‰È·‚ËÙÈÎÔ‡˜ ·ÛıÂÓ›˜ ÌÂ
·Ú¯fiÌÂÓË (22) ‹ ÂÁηÙÂÛÙË̤ÓË ‰È·‚ËÙÈ΋ ÓÂ-
ÊÚÔ¿ıÂÈ· (23). ∞Ó¿ÏÔÁ· Â˘Ú‹Ì·Ù· ˘‹Ú¯·Ó
Î·È ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. ™Â Ê˘ÛÈÔÏÔÁÈÎÔ‡˜
ÂÊ‹‚Ô˘˜ ¤¯ÂÈ ‰È·ÈÛÙˆı› ·ÏÏËÏ›‰Ú·ÛË ÙÔ˘
∞¡™ Î·È Ù˘ ∞¶ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ 24ÒÚÔ˘
(8) Î·È ÂȉÈÎfiÙÂÚ· ·Ú·ÙËÚÂ›Ù·È ÙÒÛË Ù˘ ∞¶
ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡ÎÙ·˜ (24). √È Spallone
Î·È Û˘Ó (25) ·Ó·Ê¤ÚÔ˘Ó Û ÂÓ‹ÏÈΘ ‰È·‚ËÙÈ-
ÎÔ‡˜ ·ÛıÂÓ›˜ Ì ¢∞¡ ÙËÓ ·Ô˘Û›· Ó˘ÎÙÂÚÈÓ‹˜
ÙÒÛ˘ Ù˘ ∞¶ Î·È ÙË Û˘Û¯¤ÙÈÛ‹ Ù˘ Ì ÙËÓ
‡·ÚÍË ·ıÔÏÔÁÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ ˘„ËÏ‹˜
(HF) Î·È ¯·ÌËÏ‹˜ (LF) Û˘¯ÓfiÙËÙ·˜ Ù˘ HRV.
™˘ÓÂÒ˜, Ë ·Ô˘Û›· Ù˘ ÙÒÛ˘ Ù˘ ∞¶ ηٿ
ÙË Ó‡ÎÙ· Û ·ÛıÂÓ›˜ Ì ¢∞¡, Û˘Û¯ÂÙ›˙ÂÙ·È ÌÂ
΢ÚÈ·Ú¯›· ÙÔ˘ Û˘Ì·ıËÙÈÎÔ‡, ÏfiÁˆ ·ÒÏÂÈ·˜
ÙÔ˘ Ó˘ÎÙÂÚÈÓÔ‡ ÙfiÓÔ˘ ÙÔ˘ ·Ú·Û˘Ì·ıËÙÈÎÔ‡
(26). ∆· Â˘Ú‹Ì·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ Û˘Ì-
ʈÓÔ‡Ó Ì ·˘Ù¿ ÙˆÓ ·ÓˆÙ¤Úˆ, ·ÊÔ‡ Ë ÔÌ¿‰·
Ì ˘„ËÏ‹ ¢¶ ηٿ ÙË Ó‡ÎÙ· ·ÚÔ˘Û›·˙ ‰È·-
Ù·Ú·¯¤˜ Ù˘ ÌÂÙ·‚ÏËÙfiÙËÙ·˜ ÙÔ˘ ηډȷÎÔ‡
Ú˘ıÌÔ‡ (˘„ËÏfiÙÂÚË Û˘¯ÓfiÙËÙ· RR). ∂›Û˘,
ÙÔ ËÏ›ÎÔ ¢¶ Ó‡ÎÙ·˜/Ë̤ڷ˜ Û˘Û¯ÂÙÈ˙fiÙ·Ó ıÂ-
ÙÈο Ì ÙË Û˘¯ÓfiÙËÙ· ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡
RR, ‰ËÏ·‰‹ ‰È·ÈÛÙÒıËΠ¿ÌÂÛË Û˘Û¯¤ÙÈÛË
Ù˘ ·Ô˘Û›·˜ Ó˘ÎÙÂÚÈÓ‹˜ ÙÒÛ˘ Ù˘ ¢¶ ÌÂ
ÙËÓ ·ÚÔ˘Û›· ‰È·Ù·Ú·¯‹˜ ÙÔ˘ ∞¡™.
¶ÚÔËÁÔ‡ÌÂÓ˜ ÌÂϤÙ˜ (13,25) Û ÂÓ‹ÏÈΘ
Ì ™¢1 Èı·ÓÔÏÔÁÔ‡Ó fiÙÈ Ë ·Ô˘Û›· ÙÒÛ˘
Ù˘ ∞¶ ÙË Ó‡ÎÙ· ÏfiÁˆ ¢∞¡, Û˘Ì‚¿ÏÏÂÈ ÛÙËÓ
Âȉ›ӈÛË Ù˘ ‰È·‚ËÙÈ΋˜ ÚˆÙÂ˚ÓÔ˘Ú›·˜.
∞Ó¿ÏÔÁ· Â˘Ú‹Ì·Ù· ·Ó·Ê¤ÚÔÓÙ·È Û ‰È·‚ËÙÈ-
ÎÔ‡˜ ÂÊ‹‚Ô˘˜ Ì ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·, ÔÈ
ÔÔ›ÔÈ ·ÚÔ˘Û›·˙·Ó ·‡ÍËÛË Ù˘ ™¶ ηٿ ÙÔÓ
‡ÓÔ (7). √È Poulsen Î·È Û˘Ó (22) ·Ú·Ù‹ÚËÛ·Ó
fiÙÈ ÔÈ ‰È·‚ËÙÈÎÔ› Ô˘ ·ÚÔ˘Û›·˙·Ó ·¤ÎÎÚÈÛË
ÌÈÎÚÔÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡Ú· ÛÙ· ·ÓÒÙÂÚ· Ê˘-
ÛÈÔÏÔÁÈο ›‰· ›¯·Ó ˘„ËÏfiÙÂÚ˜ ÙÈ̤˜ ∞¶
Î·È Î·Ú‰È·ÎÔ‡ Ú˘ıÌÔ‡ ηٿ ÙË Ó‡ÎÙ·, ·˘ÍË̤-
ÓÔ ËÏ›ÎÔ ‰È·ÛÙÔÏÈ΋˜ ›ÂÛ˘ Ó‡ÎÙ·˜/Ë̤ڷ˜,
ηıÒ˜ Î·È ·ıÔÏÔÁÈÎÔ‡˜ ‰Â›ÎÙ˜ ÏÂÈÙÔ˘ÚÁ›·˜
ÙÔ˘ ∞¡™. ™˘ÓÂÒ˜, ·Ú·Ù‹ÚËÛ·Ó ÌÂÙ·‚ÔϤ˜
ÛÙËÓ ∞¶ Î·È ÙÔ ∞¡™ ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘
ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜ (22). ∞Ó¿ÏÔÁ· ¢ڋ-
Ì·Ù· ˘‹Ú¯·Ó Î·È ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. ¢È·-
ÈÛÙÒıËΠfiÙÈ Ë ˘ÔÔÌ¿‰· ÙˆÓ ‰È·‚ËÙÈÎÒÓ
¶·È‰È·ÙÚÈ΋ 2006;69:432-439
¶›Ó·Î·˜ 2. ™‡ÁÎÚÈÛË ÔÌ¿‰ˆÓ ·Ó¿ÏÔÁ· Ì ÙË ÌÂÙ·‚ÔÏ‹ ‰È·ÛÙÔÏÈ΋˜ ›ÂÛ˘ ηٿ ÙË Ó‡ÎÙ·
¶ËÏ›ÎÔ ¢¶ (¡/∏) >0,9 ¶ËÏ›ÎÔ ¢¶ (¡/∏) ≤0,9 p(non-dippers) (dippers)
n=6 n=8
∏ÏÈΛ· (¤ÙË) 18,9±4,7 16,7±3,3 ª™¢È¿ÚÎÂÈ· ÓfiÛÔ˘ (¤ÙË) 8,0±3,5 8,8±3,1 ª™∏bA1c (%) 8,8±1,3 7,6±1,2 0,004BMI (kg/m2) 22,4±3,0 22,2±2,5 ª™ª¤ÛË ™¶ (Ó‡ÎÙ·) mmHg 111,6±10,4 102,4±7,7 0,002ª¤ÛË ¢¶ (Ó‡ÎÙ·) mmHg 61,8±8,7 56,3±6,6 0,026∞§√ Ó‡ÎÙ·˜ (Ìg/min) 11,3±7,2 9,3±5,8 ª™
ª™: ™Ù·ÙÈÛÙÈο ÌË ÛËÌ·ÓÙÈÎfi¶ËÏ›ÎÔ ¢¶ (¡/∏): ¶ËÏ›ÎÔ ‰È·ÛÙÔÏÈ΋˜ ›ÂÛ˘ Ó‡ÎÙ·˜/Ë̤ڷ˜ ∞§√: ∞¤ÎÎÚÈÛË ÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡Ú· ™¶: ™˘ÛÙÔÏÈ΋ ›ÂÛË ¢¶: ¢È·ÛÙÔÏÈ΋ ›ÂÛË
437¢È·Ù·Ú·¯¤˜ ÙÔ˘ ∞¡™ Î·È Ù˘ ™¶ ÛÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜ Û ™¢1
ÂÊ‹‚ˆÓ Ì ÔÚȷο ·˘ÍË̤ÓË ·¤ÎÎÚÈÛË ÌÈ-
ÎÚÔÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡Ú·, ›¯Â ˘„ËϤ˜ ÙÈ̤˜
̤Û˘ ™¶ Î·È ¢¶ ηٿ ÙË Ó‡ÎÙ· Î·È Ù·¯‡ÙÂÚÔ
ηډȷÎfi Ú˘ıÌfi. ∂›Û˘, ÛÙËÓ ˘ÔÔÌ¿‰· ÙˆÓ
ÂÊ‹‚ˆÓ Ì ·˘ÍË̤ÓË ¢¶ ÙË Ó‡ÎÙ·, ‰È·ÈÛÙÒ-
ıËÎ·Ó ·ıÔÏÔÁÈΤ˜ ÙÈ̤˜ ÙˆÓ ·Ú·Ì¤ÙÚˆÓ Ù˘
ÌÂÙ·‚ÏËÙfiÙËÙ·˜ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡, ¯ˆÚ›˜
ÙËÓ ‡·ÚÍË ÌÈÎÚÔÏÂ˘ÎˆÌ·ÙÈÓÔ˘Ú›·˜.
ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ Â›‰Ú·ÛË ÙÔ˘ ∞¡™ ÛÙË
ÓÂÊÚÈ΋ ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜ (renal blood flow -
RBF), ÌÂϤÙ˜ Û ‰È·‚ËÙÈο ÔÓÙ›ÎÈ· ·Ó·Ê¤-
ÚÔ˘Ó fiÙÈ ÌÂÙ¿ ·fi Ó¢ÚÈ΋ ‰È¤ÁÂÚÛË, ·˘Í¿ÓÔ-
ÓÙ·È ÔÈ ÂÚÈÊÂÚÈΤ˜ ·ÁÁÂȷΤ˜ ·ÓÙÈÛÙ¿ÛÂȘ, ÌÂ
·ÔÙ¤ÏÂÛÌ· ÙË Ì›ˆÛË Ù˘ ÓÂÊÚÈ΋˜ ÚÔ‹˜ ·›-
Ì·ÙÔ˜ (RBF) Î·È ÙËÓ ·‡ÍËÛË Ù˘ ·ӷÚÚfiÊË-
Û˘ ÙÔ˘ ÓÂÚÔ‡ ·fi ÙÔ ÂÁÁ‡˜ ÂÛÂÈڷ̤ÓÔ Ûˆ-
ÏËÓ¿ÚÈÔ (27). ∞ÓÙ›ıÂÙ·, ·Ó·Ê¤ÚÂÙ·È ·˘ÍË̤ÓË
‰ÈÔ‡ÚËÛË Û ‰È·‚ËÙÈÎÔ‡˜ ·ÛıÂÓ›˜ Ì ·˘ÙfiÓÔ-
ÌË Ó¢ÚÔ¿ıÂÈ· (28). ™˘ÓÂÒ˜, Â›Ó·È Èı·Ófi
fiÙÈ Ë Ì›ˆÛË Ù˘ RBF ηٿ ÙËÓ Ë̤ڷ Î·È Ë ·‡-
ÍËÛ‹ Ù˘ ηٿ ÙË Ó‡ÎÙ· ·ÔÙÂÏÔ‡Ó ÙÔÓ Û˘Ó‰Â-
ÙÈÎfi ÎÚ›ÎÔ ÌÂٷ͇ Ù˘ ¢∞¡ Î·È Ù˘ ·ıÔÁ¤ÓÂÈ-
·˜ ‹ Âȉ›ӈÛ˘ Ù˘ ¢¡ (25).
¶Èı·ÓÔ› ·ıÔÁÂÓÂÙÈÎÔ› Ì˯·ÓÈÛÌÔ› ÁÈ· ÙȘ
‰È·Ù·Ú·¯¤˜ ·˘Ù¤˜ Û ·ÛıÂÓ›˜ Ì ¢∞¡ ÂÚÈ-
Ï·Ì‚¿ÓÔ˘Ó ÙËÓ ÂӉ¯fiÌÂÓË ÏÂÈÙÔ˘ÚÁÈ΋ ‰È·Ù·-
Ú·¯‹ ÙˆÓ Ï›ˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ ÙˆÓ ÚÔÛ·ÁˆÁÒÓ
·ÚÙËÚȉ›ˆÓ ÙÔ˘ ÓÂÊÚÈÎÔ‡ ÛÂÈÚ¿Ì·ÙÔ˜, ÌÂ
·ÔÙ¤ÏÂÛÌ· Ó· ÂÌÔ‰›˙ÂÙ·È Ô ÚÔÛٷ٢ÙÈÎfi˜
Ì˯·ÓÈÛÌfi˜ ÙÔ˘ ÓÂÊÚÔ‡ ÛÙËÓ ·ÚÔ˘Û›· ˘„Ë-
Ï‹˜ ∞¶ (29). ∂ÈϤÔÓ, Ë ·ÚÔ˘Û›· ¢∞¡ ·fi
ÌfiÓË Ù˘ Û˘Ó¿ÁÂÙ·È ÌÈÎÚfiÙÂÚ˜ Ó˘¯ıËÌÂÚfiÓ
ÌÂÙ·‚ÔϤ˜ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ Î·È Ù˘ ∞¶,
Ô˘ Ô‰ËÁ› Û ۯÂÙÈ΋ ˘¤ÚÙ·ÛË Î·Ù¿ ÙË ‰È¿Ú-
ÎÂÈ· Ù˘ Ó‡ÎÙ·˜. ™˘ÓÂÒ˜, ˆ˜ ·ıÔÁÂÓÂÙÈÎÔ›
Ì˯·ÓÈÛÌÔ› Ù˘ Û˘Ì‚ÔÏ‹˜ Ù˘ ¢∞¡ ÛÙËÓ ÂÈ-
‰Â›ÓˆÛË Ù˘ ‰È·‚ËÙÈ΋˜ ÓÂÊÚÔ¿ıÂÈ·˜ Èı·-
ÓÔÏÔÁÔ‡ÓÙ·È ÔÈ ·ÎfiÏÔ˘ıÔÈ: ∏ ¢∞¡ Û˘Ì‚¿ÏÏÂÈ:
¶·È‰È·ÙÚÈ΋ 2006;69:432-439
¶›Ó·Î·˜ 3. ∞Ó¿Ï˘ÛË ÌÂÙ·‚ÏËÙfiÙËÙ·˜ ηډȷÎÔ‡ Ú˘ıÌÔ‡ ηٿ ÙË Ó‡ÎÙ·
Non-Dippers Dippers pª¤ÛË ÙÈÌ‹±SD ª¤ÛË ÙÈÌ‹ ±SD
∞. ∞Ó¿Ï˘ÛË ÛÙÔÓ ¯ÚfiÓÔ (∆ime domain analysis)ª¤ÛË ÙÈÌ‹ RR (ms) 916,0±129,8 764,4±103,9 0,001SDNN (ms) 183,1±56,0 175,5±94,7 ª™SDNN-i (ms) 110,6±27,4 88,1±35,9 0,018SDAN-i (ms) 137,3±54,2 125,5±62,2 ª™r-MSSD (ms) 117,7±42,1 83,7±43,0 0,008pNN50 (%) 36,3±15,7 28,2±20,2 ª™µ. ∞Ó¿Ï˘ÛË Î·Ù¿ Û˘¯ÓfiÙËÙ˜ (Frequency domain analysis)LF (ms.ms) 1973±1111,3 1599,8±960,4 ª™HF (ms.ms) 1466,4±931,2 1059,5±898,4 ª™LF/HF 1,5±0,6 1,9±1,15 ª™
M¤ÛË ÙÈÌ‹ RR: ª¤ÛË ÙÈÌ‹ fiÏˆÓ ÙˆÓ R-R ‰È·ÛÙËÌ¿ÙˆÓ SDNN: ™Ù·ıÂÚ¿ ·fiÎÏÈÛË (SD) ÙˆÓ R-R ‰È·ÛÙËÌ¿ÙˆÓ Î·Ù¿ ÙË Ó‡ÎÙ· SDNN-i: ª¤ÛË ÙÈÌ‹ ÙˆÓ ÛÙ·ıÂÚÒÓ ·ÔÎÏ›ÛÂˆÓ fiÏˆÓ ÙˆÓ ÊÈÏÙÚ·ÚÈÛÌ¤ÓˆÓ R-R ‰È·ÛÙËÌ¿ÙˆÓ ÁÈ· fiÏ· Ù· ÙÌ‹Ì·Ù·ÙˆÓ 5 ÏÂÙÒÓ Î·Ù¿ ÙË Ó‡ÎÙ· SDAN-i: ™Ù·ıÂÚ¿ ·fiÎÏÈÛË Ù˘ SDNN-i pNN50: ¶ÔÛÔÛÙfi ÙˆÓ ‰È·ÊÔÚÒÓ ÌÂٷ͇ ÙˆÓ Û˘Ó¯fiÌÂÓˆÓ R-R ‰È·ÛÙËÌ¿ÙˆÓ >50 ms ηٿ ÙË Ó‡ÎÙ· r-MSSD: TÂÙÚ·ÁˆÓÈ΋ Ú›˙· ÙÔ˘ ̤ÛÔ˘ fiÚÔ˘ ÙÔ˘ ·ıÚÔ›ÛÌ·ÙÔ˜ ÙˆÓ ÙÂÙÚ·ÁÒÓˆÓ Ù˘ pNN50 ∏F, LF: ¶ÂÚÈÔ¯‹ ˘„ËÏÒÓ Î·È ¯·ÌËÏÒÓ Û˘¯ÓÔÙ‹ÙˆÓ Ù˘ HRV ηٿ ÙË Ó‡ÎÙ· LF/HF: ¶ËÏ›ÎÔ ¯·ÌËÏÒÓ/˘„ËÏÒÓ Û˘¯ÓÔÙ‹ÙˆÓ Ù˘ HRV ηٿ ÙË Ó‡ÎÙ· ª™: ™Ù·ÙÈÛÙÈο ÌË ÛËÌ·ÓÙÈÎfi
N˘
ÎÙ
ÂÚÈÓ
‹ ·
¤Î
ÎÚ
ÈÛË
ÌÈÎ
ÚÔ
Ï¢
ÎÔ
Ì·
Ù›Ó
˘
Ô‡
Úˆ
Ó (
mcg
/min
)
20,0
15,0
10,0
5,0
0,0
11
23
Dippers Non-dippers
¶ËÏ›ÎÔ ‰È·ÛÙÔÏÈ΋˜ ›ÂÛ˘ ηٿ ÙË Ó‡ÎÙ·
∂ÈÎfiÓ· 1. ∞¤ÎÎÚÈÛË ÌÈÎÚÔÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡Ú· ·Ó¿ÏÔ-Á· Ì ÙËÓ ÙÒÛË Ù˘ ‰È·ÛÙÔÏÈ΋˜ ›ÂÛ˘ ηٿ ÙË Ó‡ÎÙ·. ∏·¤ÎÎÚÈÛË ÌÈÎÚÔÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡Ú· ‹Ù·Ó ·˘ÍË̤ÓËÛÙËÓ ÔÌ¿‰· ÙˆÓ “non-dippers” Û˘ÁÎÚÈÙÈο Ì ÙËÓ ÔÌ¿‰·ÙˆÓ “dippers”.
438 ∫. K·Ú·‚·Ó¿ÎË Î·È Û˘Ó.
·) ÛÙË Ì›ˆÛË Ù˘ ÙÒÛ˘ Ù˘ ∞¶ ηٿ ÙË Ó‡-ÎÙ·, ‚) ÛÙËÓ ·‡ÍËÛË Ù˘ RBF Î·È Á) ÛÙË Ì›ˆÛËÙ˘ ·ӷÚÚfiÊËÛ˘ ÓÂÚÔ‡ ·fi ÙÔ ÂÁÁ‡˜ ÂÛÂÈ-ڷ̤ÓÔ ÛˆÏËÓ¿ÚÈÔ (25). √È ·Ú·¿Óˆ ·ÈÌÔ‰˘-Ó·ÌÈΤ˜ ÌÂÙ·‚ÔϤ˜ ÛÙÔÓ ÓÂÊÚfi Â›Ó·È Èı·Ófi fiÙÈÛ˘Ì‚¿ÏÏÔ˘Ó ÛÙËÓ ¤Ó·ÚÍË ‹ ÙËÓ Âȉ›ӈÛËÙ˘ ‰È·‚ËÙÈ΋˜ ÚˆÙÂ˚ÓÔ˘Ú›·˜.
™˘ÌÂÚ·ÛÌ·ÙÈο, ‰È·ÈÛÙÒıËΠfiÙÈ ‰È·‚Ë-ÙÈÎÔ› ¤ÊË‚ÔÈ ¯ˆÚ›˜ ˘¤ÚÙ·ÛË Î·È ÌÈÎÚÔÏÂ˘Îˆ-Ì·ÙÈÓÔ˘Ú›· ÌÔÚ› Ó· ÂÌÊ·Ó›˙Ô˘Ó ÔÚȷο ·˘-ÍË̤ÓË ·¤ÎÎÚÈÛË ÌÈÎÚÔÏÂ˘ÎˆÌ·Ù›Ó˘ ÛÙ· Ô‡-Ú·, ·Ú¿ÏÏËÏ· Ì ÙËÓ ·Ô˘Û›· ÙÒÛ˘ Ù˘ ∞¶Î·Ù¿ ÙË Ó‡ÎÙ· ηıÒ˜ Î·È Ì ‰È·Ù·Ú·¯¤˜ ÙÔ˘∞¡™. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Ù˘ ·Ô˘Û›·˜ ÙÒ-Û˘ ∞¶ ηٿ ÙË Ó‡ÎÙ· ıˆÚÂ›Ù·È ÚÔÁÓˆÛÙÈ-Îfi˜ ‰Â›ÎÙ˘ ÌÂÏÏÔÓÙÈ΋˜ ÂÌÊ¿ÓÈÛ˘ ÌÈÎÚÔÏ¢-Έ̷ÙÈÓÔ˘Ú›·˜ Î·È ¢¡ (30). ™˘ÓÂÒ˜, Ë ¤ÁηÈ-ÚË ·Ó›¯Ó¢ÛË ‰È·‚ËÙÈÎÒÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓÌ ˘„ËÏ‹ ∞¶ ÙË Ó‡ÎÙ· ı· ÌÔÚÔ‡Û ӷ Û˘Ì-‚¿ÏÂÈ ÛÙËÓ ÚfiÏË„Ë Ù˘ ÂÌÊ¿ÓÈÛ˘ ÌÈÎÚÔÏ¢-Έ̷ÙÈÓÔ˘Ú›·˜, Ì ÙË ¯Ú‹ÛË ÂӉ¯Ô̤ӈ˜Ê·Ú̷΢ÙÈ΋˜ ·ÁˆÁ‹˜.
µÈ‚ÏÈÔÁÚ·Ê›·
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¶·È‰È·ÙÚÈ΋ 2006;69:432-439
440 ∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞ ORIGINAL ARTICLE
¶·È‰È·ÙÚÈ΋ 2006;69:440-446
™˘Û¯¤ÙÈÛË Î·Ù·Ó¿ÏˆÛ˘ ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜ ηÈ
ÎÏÈÓÈ΋˜ ηٿÛÙ·Û˘ ·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË
∆. ªÔ‡‰ÈÔ˘1, ∞. °·ÏÏ‹ - ∆ÛÈÓÔÔ‡Ïo˘1, ∂. µ·Ì‚·ÎÔ‡‰Ë˜2, ™. ¡Ô‡ÛÈ· - ∞Ú‚·ÓÈÙ¿ÎË1
¶ÂÚ›ÏË„Ë
∂ÈÛ·ÁˆÁ‹: ∏ ηٷӿψÛË ÂÓ¤ÚÁÂÈ·˜ ÛÙËÓ ËÚÂÌ›· ηχÙÂÈ ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ Ì¤ÚÔ˜ ÙÔ˘ ÌÂÙ·‚ÔÏÈ-ÛÌÔ‡ ÛÙÔÓ ¿ÓıÚˆÔ. À¿Ú¯Ô˘Ó ÔÏÏÔ› ·Ú¿ÁÔÓÙ˜ ·fi ÙÔ˘˜ ÔÔ›Ô˘˜ ÂËÚ¿˙ÂÙ·È Ë Î·Ù·Ó¿ÏˆÛËÂÓ¤ÚÁÂÈ·˜. ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë Û˘Û¯¤ÙÈÛË Ù˘ ηٷӿψÛ˘ ÂÓ¤ÚÁÂÈ·˜ Û ËÚÂ-Ì›· (KEH) Ì ÙËÓ ÎÏÈÓÈ΋ ηٿÛÙ·ÛË ·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË (∫π) ¯ˆÚ›˜ ÚfiÛÊ·ÙË Ïԛ̈ÍË.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ∏ ∫∂∏ ÌÂÙÚ‹ıËΠ̠ÙË Ì¤ıÔ‰Ô Ù˘ ¤ÌÌÂÛ˘ ıÂÚÌȉÔÌÂÙÚ›·˜ Û ̛· ÔÌ¿‰· 38·ÛıÂÓÒÓ Ì ∫π Î·È ÂÍÂÙ¿ÛÙËÎÂ Ë Û¯¤ÛË Ù˘ Ì ÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· Î·È ÙËÓ ÎÏÈÓÈ΋ ηٿ-ÛÙ·ÛË ÙˆÓ ·ÛıÂÓÒÓ. °È· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÌÂÙÚ‹ıËÎ·Ó Ì ÛÈÚÔÌÂÙÚ›·Ë FEV1, Ë FVC Î·È Ë PEFR, ÂÓÒ Ë ÎÏÈÓÈ΋ ηٿÛÙ·ÛË ÚÔÛ‰ÈÔÚ›ÛÙËΠ·fi ÙÔÓ ‰Â›ÎÙË Schwachman.
∞ÔÙÂϤÛÌ·Ù·: ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π, Û ۇÁÎÚÈÛË Ì ˘ÁÈ‹ ¿ÙÔÌ·, ‚Ú¤ıËΠ·˘ÍË̤ÓË ∫∂∏, ÂÎÊÚ·-Ṳ̂ÓË ˆ˜ ÔÛÔÛÙfi Ù˘ ÚÔ‚ÏÂfiÌÂÓ˘ ∫∂∏ (∫∂∏%), (110,96±2,35%, p=0,001). ∏ ·‡ÍËÛË ·˘Ù‹·Ú·ÙËÚ‹ıËÎÂ Î·È ÛÙ· ‰‡Ô ʇϷ. ∂›Û˘, ˘¿Ú¯ÂÈ ÈÛ¯˘Ú‹ Û˘Û¯¤ÙÈÛË Ù˘ ∫∂∏% Ì ÙÔÓ ‰Â›ÎÙËSchwachman ÙˆÓ ·ÛıÂÓÒÓ (r=-0,654, p=0,0001) Î·È ÙËÓ Î·Ù¿ÛÙ·ÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ÙÔ˘˜ (FEV1: r=-0,475, p=0,003 Î·È FVC: r=-0,57, p=0,0001).
™˘ÌÂÚ¿ÛÌ·Ù·: ™˘ÌÂÚ·›ÓÂÙ·È fiÙÈ ÙÔ ÔÛÔÛÙfi Ù˘ ÚÔ‚ÏÂfiÌÂÓ˘ ∫∂∏ Â›Ó·È ÈηÓfi Ó· ÛÙ·ı› ˆ˜‰Â›ÎÙ˘ ‚·Ú‡ÙËÙ·˜ Ù˘ ÓfiÛÔ˘. ∂ÈϤÔÓ, Û˘ÓÈÛÙ¿Ù·È Ë Ì¤ÙÚËÛË Ù˘ ∫∂∏% ÛÙÔÓ ÂÙ‹ÛÈÔ ¤ÏÂÁ¯ÔÙˆÓ ·ÛıÂÓÒÓ Ì ∫π.
§¤ÍÂȘ ÎÏÂȉȿ: ∫˘ÛÙÈ΋ ›ÓˆÛË, ηٷӿψÛË ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜.
Relation of resting energy expenditure with the clinical condition in
cystic fibrosis patients
T. Moudiou1, A. Galli - Tsinopoulou1, E. Vamvakoudis2, S. Nousia - Arvanitakis1
Abstract:
Background: Resting energy expenditure (REE) is the main component of human metabolism.There are many factors affecting energy expenditure. The purpose of this study was to investigate therelationship of REE with the clinical condition of cystic fibrosis (CF) patients without active infection.The issue whether REE can serve alone as an indicator of the disease severity was addressed.
Methods: REE was measured with indirect calorimetry in a group of 38 CF patients and its correlationwith pulmonary function assessed with FEV1 and FVC, and clinical status assessed with Schwachmanscore was studied.
Results: An increased REE expressed as a percentage of predicted REE (REE%) was found in CFpatients, compared to healthy (110.96±2.35%, p=0.001). REE was not affected by gender differencesand exhibited a strong correlation with Schwachman score (r=-0.654, p=0.0001) and pulmonaryfunction (FEV1: r=-0.475, p=0.003 and FVC: r=-0.57, p=0.0001) of the patients.
Conclusions: This data analysis revealed that the percentage of predicted REE can serve alone as anindicator of disease severity. The use of REE% on yearly clinical follow up of the CF patient isrecommended.
Key words: Cystic fibrosis, resting energy expenditure.
1 ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞.¶.£., ¡ÔÛÔÎÔÌ›Զ··ÁˆÚÁ›Ô˘,£ÂÛÛ·ÏÔÓ›ÎË
2 ∂ÚÁ·ÛÙ‹ÚÈÔ ∂ÚÁÔÌÂÙÚ›·˜,∆∂º∞∞, ∞.¶.£.£ÂÛÛ·ÏÔÓ›ÎË
AÏÏËÏÔÁÚ·Ê›·:
∆·ÙÈ·Ó‹ ªÔ‡‰ÈÔ˘ ∞Á›·˜ ™ÔÊ›·˜ 9, ∆.∫. 570 10, ¶Â‡Î·£ÂÛÛ·ÏÔӛ΢ E-mail: [email protected]
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 10-02-2006∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 05-09-2006
1 4th Department ofPediatrics, AristotleUniversity of Thessaloniki,Papageorgiou Hospital,Thessaloniki
2 Ergophysiology andErgometry Laboratory,Faculty of PhysicalEducation and SportScience, Aristotle Universityof Thessaloniki
Correspondence:
Tatiani Moudiou 9, Agias Sofias Str. 57010 Pefka, Thessaloniki E-mail: [email protected]
Date of submission: 10-02-2006Date of approval: 05-09-2006
441∫·Ù·Ó¿ÏˆÛË ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜ ÛÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË
¶·È‰È·ÙÚÈ΋ 2006;69:440-446
™˘ÓÙÔÌÔÁڷʛ˜
∫∂∏ ∫·Ù·Ó¿ÏˆÛË ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜ ∫π ∫˘ÛÙÈ΋ ›ÓˆÛË ¢ª™ ¢Â›ÎÙ˘ Ì¿˙·˜ ÛÒÌ·ÙÔ˜
∂ÈÛ·ÁˆÁ‹
∏ ˘ÔıÚ„›· ·ÔÙÂÏ› ÌÈ· Û˘¯Ó‹ ÎÏÈÓÈ΋ÂΉ‹ÏˆÛË Ù˘ ∫π, Ë ÔÔ›· ·Ô‰fiıËΠÛÙÔ ·-ÚÂÏıfiÓ Î˘Ú›ˆ˜ ÛÙËÓ ·Ó·Ú΋ ÚfiÛÏË„Ë ÙÚÔ-Ê‹˜ Î·È ÛÙËÓ ÏËÌÌÂÏ‹ ·ÔÚÚfiÊËÛË (1). ∏Û‡Á¯ÚÔÓË, fï˜, ıÂڷ›· Ì ˘ÔηٿÛٷٷÌÈÎÚÔÛÊ·ÈÚÈÎÒÓ ·ÁÎÚ·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ, ËÔÔ›· Ô‰ËÁ› ÛÙËÓ Î·Ï‡ÙÂÚË ·ÔÚÚfiÊËÛË, ÛÂÛ˘Ó‰˘·ÛÌfi Ì ‰›·ÈÙ˜ ˘„ËÏ‹˜ ÂÓ¤ÚÁÂÈ·˜, ı·¤Ú ӷ ›¯Â ·ÓÙÈÌÂÙˆ›ÛÂÈ ÙÔ Úfi‚ÏËÌ· Ù˘˘ÔıÚ„›·˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π. ¶·Ú’ fiÏ··˘Ù¿, ÛÙÔ 20% ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ, ÙÔ ÛˆÌ·ÙÈ-Îfi ‚¿ÚÔ˜ ÂÍ·ÎÔÏÔ˘ı› Ó· ‚Ú›ÛÎÂÙ·È Î¿Ùˆ ·fiÙËÓ 5Ë ÂηÙÔÛÙÈ·›· ı¤ÛË (2). ∂›Ó·È ÁÓˆÛÙfi fiÙÈ ËηٷӿψÛË ÂÓ¤ÚÁÂÈ·˜ ÂËÚ¿˙ÂÙ·È ·fi ‰È¿-ÊÔÚÔ˘˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ ÙÔ ‚¿ÚÔ˜ ÛÒÌ·ÙÔ˜Î·È È‰È·›ÙÂÚ· ÙÔ ÔÛÔÛÙfi Ì˘˚΋˜ Ì¿˙·˜ ÛÒÌ·-ÙÔ˜, fiÔ˘ ΢ڛˆ˜ ηٷӷÏÒÓÂÙ·È ÙÔ Ô͢ÁfiÓÔ.∂›Û˘, Ë ¯ÚfiÓÈ· Ó¢ÌÔÓÔ¿ıÂÈ· ·ÔÙÂÏ›ÛËÌ·ÓÙÈÎfi ·Ú¿ÁÔÓÙ·, Ô ÔÔ›Ô˜ Ô‰ËÁ› Û¢ÂÚÌÂÙ·‚ÔÏÈÛÌfi. ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π ηÈÂÈ‚·Ú˘Ì¤ÓË Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›·, ÙÔ ÛˆÌ·-ÙÈÎfi ‚¿ÚÔ˜ Î·È Ë Ì˘˚΋ Ì¿˙· Â›Ó·È Û˘Ó‹ıˆ˜ÂÏ·Ùو̤ӷ. ∏ Û˘ÓÔÏÈ΋ ηٷӿψÛË ÂÓ¤ÚÁÂÈ-·˜ Û ·ÛıÂÓ›˜ Ì ∫π Î·È Ì¤ÙÚÈ· ÂÈ‚¿Ú˘ÓÛË Ù˘Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ¤¯ÂÈ ·Ó·ÊÂÚı› fiÙÈ Â›-Ó·È Û˘ÁÎÚ›ÛÈÌË Ì ÂΛÓË ÛÙÔÓ ˘ÁÈ‹ ÏËı˘ÛÌfi,·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ∫∂∏ ‚Ú¤ıËΠ·˘ÍË̤ÓË(3-5).
∏ ∫∂∏ ·ÔÙÂÏ› Ù· ‰‡Ô ÙÚ›Ù· Ù˘ ËÌÂÚ‹-ÛÈ·˜ ηٷӿψÛ˘ ÂÓ¤ÚÁÂÈ·˜. ∆Ô ˘fiÏÔÈÔ ¤Ó·ÙÚ›ÙÔ Â›Ó·È ÙÔ ¿ıÚÔÈÛÌ· Ù˘ ηٷӿψÛ˘ÂÓ¤ÚÁÂÈ·˜, ÙÔ ÔÔ›Ô Û¯ÂÙ›˙ÂÙ·È Ì ÙË Ê˘ÛÈ΋‰Ú·ÛÙËÚÈfiÙËÙ·, ÙË ıÂÚÌÔÁ¤ÓÂÛË Î·È ÙË ÛˆÌ·-ÙÈ΋ ·‡ÍËÛË ÛÙ· ·È‰È¿ (6). À¿Ú¯ÂÈ, ˆÛÙfiÛÔ,‰È·ÊˆÓ›· ÛÙË Û¯¤ÛË Ù˘ ∫∂∏ Ì ÙËÓ ¤Í·ÚÛËÙ˘ Ó¢ÌÔÓÈ΋˜ ÓfiÛÔ˘ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π.∫¿ÔȘ ÌÂϤÙ˜ (5,7,8) ‰Â›¯ÓÔ˘Ó ·‡ÍËÛË Ù˘∫∂∏ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ¤Í·ÚÛ˘ Ù˘ Ó¢-ÌÔÓÈ΋˜ ÓfiÛÔ˘, ÂÓÒ ¿ÏϘ fi¯È (9). ∂›Û˘,˘¿Ú¯Ô˘Ó ÌÂϤÙ˜ ÛÙȘ Ôԛ˜ ÂÍÂÙ¿˙ÂÙ·È Ë∫∂∏ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π, ÔÈ ÔÔ›ÔÈ ‰ÂÓ ¤¯Ô˘ÓÚfiÛÊ·ÙË Ïԛ̈ÍË (5,10,11). ™‡Ìʈӷ Ì ÙȘÌÂϤÙ˜ ·˘Ù¤˜, Ë ∫∂∏ Â›Ó·È ·˘ÍË̤ÓË ÛÙÔ˘˜·ÛıÂÓ›˜ Ì ∫π Û ۇÁÎÚÈÛË Ì ·ÓÙ›ÛÙÔÈ¯Ë ÔÌ¿-‰· ˘ÁÈÒÓ ·ÙfiÌˆÓ ‹ Ì ÙÈ̤˜ Ô˘ ÚÔ¤Ú¯ÔÓÙ·È·fi ÂÍÈÛÒÛÂȘ, ÔÈ Ôԛ˜ ÂÚÈÁÚ¿ÊÔ˘Ó ·ÓÙ›-
ÛÙÔÈ¯Ô ˘ÁÈ‹ ÏËı˘ÛÌfi. ¢ÂÓ ¤¯ÂÈ, fï˜, ‰È¢-
ÎÚÈÓÈÛÙ› Â¿Ó Ë ·‡ÍËÛË Ù˘ ∫∂∏, Ë ÔÔ›· ·Ó·-
ʤÚÂÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·, ÔÊ›ÏÂÙ·È ÛÙËÓ
¤Í·ÚÛË Ù˘ Ïԛ̈͢, ÛÙË ¯ÚfiÓÈ· ÊÏÂÁÌÔÓ‹,
ÛÙË ÁÔÓȉȷ΋ ‚Ï¿‚Ë ‹ ÛÙËÓ ÂÈ‚¿Ú˘ÓÛË Ù˘
Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (12,13).
™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ó·
·ÔÛ·ÊËÓ›ÛÂÈ ÙË Û¯¤ÛË Ù˘ ∫∂∏ Ì ÙËÓ Ó¢-
ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· Û ·ÛıÂÓ›˜ Ì ∫π, ÔÈ ÔÔ›ÔÈ
‹Ù·Ó ÎÏÈÓÈο ÛÙ·ıÂÚÔ› Î·È ‰ÂÓ Â›¯·Ó ¤Í·ÚÛË
Ù˘ Ó¢ÌÔÓÈ΋˜ ÓfiÛÔ˘. ∂›Û˘, Ë ÌÂϤÙË ·˘-
Ù‹ ·ÔÛÎÔ› ÛÙÔ Ó· ‰ÈÂÚ¢ӋÛÂÈ Î·Ù¿ fiÛÔÓ
Ë ∫∂∏ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› ˆ˜ ‰Â›ÎÙ˘
‚·Ú‡ÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Ù˘ ∫π.
ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ
ªÂÏÂÙ‹ıËÎ·Ó 38 ·ÛıÂÓ›˜ Ì ∫π, 19 ·ÁfiÚÈ· Î·È 19
ÎÔÚ›ÙÛÈ·, ËÏÈΛ·˜ 9-35 ÂÙÒÓ, Ì ̤ÛÔ fiÚÔ ËÏÈΛ·˜ Ù·
19,72±1,0 ¤ÙË. √È ·ÛıÂÓ›˜ ·˘ÙÔ› ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·Ó
Ù·ÎÙÈο ·fi ÙÔ Â͈ÙÂÚÈÎfi È·ÙÚÂ›Ô ∫π. √ ¤ÏÂÁ¯Ô˜ ¤ÁÈÓ ÛÂ
ÂÚ›Ô‰Ô Î·Ù¿ ÙËÓ ÔÔ›· ÔÈ ·ÛıÂÓ›˜ ‹Ù·Ó Û ηϋ ÁÂÓÈ-
΋ ηٿÛÙ·ÛË, ¯ˆÚ›˜ Á·ÛÙÚÔÛÙÔÌ›· ‹ ·ÚÂÓÙÂÚÈ΋ ‰È·-
ÙÚÔÊ‹ Î·È Î·Ó›˜ ‰ÂÓ Â›¯Â ¤Í·ÚÛË Ù˘ Ó¢ÌÔÓÈ΋˜ Ófi-
ÛÔ˘. ∏ ·ÍÈÔÏfiÁËÛË Ù˘ ÎÏÈÓÈ΋˜ ÙÔ˘˜ ηٿÛÙ·Û˘, fiˆ˜
ÂÎÊÚ¿˙ÂÙ·È ·fi ÙË ‚·ıÌÔÏfiÁËÛË Î·Ù¿ Shwachman,
‹Ù·Ó ·fi 74 ¤ˆ˜ 96 (̤ÛÔ˜ fiÚÔ˜: 87,0±1,18). ∞fi ÙÔ Û‡-
ÓÔÏÔ ÙˆÓ 38 ·ÛıÂÓÒÓ Ì ∫π, ÔÈ 6 ›¯·Ó ·ÁÎÚ·ÙÈ΋
¿ÚÎÂÈ· (¶∂). ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ Ì ·ÁÎÚ·ÙÈ΋ ·ÓÂ-
¿ÚÎÂÈ· (¶∞) ‚Ú›ÛÎÔÓÙ·Ó Û ıÂڷ›· ˘ÔηٿÛÙ·Û˘
Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì·. ∞fi ÙÔ˘˜ 38 ·ÛıÂÓ›˜, ÔÈ 32 ›-
¯·Ó ·ÔÈÎÈÛÙ› Ì „¢‰ÔÌÔÓ¿‰· Î·È ·ÎÔÏÔ˘ıÔ‡Û·Ó ÙÔ
ÚˆÙfiÎÔÏÏÔ ıÂڷ›·˜ Ì ÂÈÛÓÂfiÌÂÓ· ·ÓÙÈ„Â˘‰ÔÌÔ-
Ó·‰Èο ·ÓÙÈ‚ÈÔÙÈο. ∏ ¤ÏÏÂÈ„Ë ¤Í·ÚÛ˘ Ù˘ ÓfiÛÔ˘ ÂÈ-
‚‚·ÈÒıËΠ̠ÙË Ê˘ÛÈ΋ ÂͤٷÛË ÙˆÓ ·ÛıÂÓÒÓ Î·È ÙÔÓ
ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô Ì ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη, CRP ηÈ
ÙÔÓ ·ÚÈıÌfi ÙˆÓ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ.
∫·Ù·Ó¿ÏˆÛË ÂÓ¤ÚÁÂÈ·˜ ÛÙËÓ ËÚÂÌ›· (∫∂∏)
∏ ∫∂∏ ÌÂÙÚ‹ıËΠ̠¤ÌÌÂÛË ıÂÚÌȉÔÌÂÙÚ›·
(JAEGER EOS-Sprint Oxygen Analyzer, Würzburg,
Germany) ηٿ ÙȘ ÚˆÈÓ¤˜ ÒÚ˜, ÌÂÙ¿ ·fi ÔÏÔÓ‡ÎÙÈ·
·Ó¿·˘ÛË Ì ‡ÓÔ. ∏ ‰ÔÎÈÌ·Û›· ¤ÁÈÓ Û ¤Ó· ‰ˆÌ¿ÙÈÔ
‹Û˘¯Ô Î·È ıÂÚÌÈο Ô˘‰¤ÙÂÚÔ. √ ·ÛıÂÓ‹˜ ‚ÚÈÛÎfiÙ·Ó ÛÂ
‡ÙÈ· ı¤ÛË, ‹Ù·Ó ‹ÚÂÌÔ˜, ¯ˆÚ›˜ Ó· ÎÔÈÌ¿Ù·È ÂÚ›Ô˘
› 15 ÏÂÙ¿, ÚÈÓ ·Ú¯›ÛÂÈ Ë ‰ÔÎÈÌ·Û›·, ÚÔÎÂÈ̤ÓÔ˘
Ó· ÂÍÔÈÎÂȈı› Ì ÙÔÓ ¯ÒÚo Î·È Ó· Á›ÓÂÈ ÂÂÍ‹ÁËÛË Ù˘
‰È·‰Èηۛ·˜. ™ÙË Û˘Ó¤¯ÂÈ·, ÂȉÈÎfi ÚÈÓÔ›ÂÛÙÚÔ Î·È ÂȉÈ-
Îfi ÂÈÛÙfiÌÈÔ ÚÔÛ·ÚÌfi˙ÔÓÙ·Ó ÛÙÔÓ ·ÛıÂÓ‹. √ ÂÎÓÂ-
fiÌÂÓÔ˜ ·¤Ú·˜ ‰ÈÔ¯ÂÙ¢fiÙ·Ó ·fi Ì›· ‚·Ï‚›‰· ˘„ËÏ‹˜
Ù·¯‡ÙËÙ·˜ (Hans Rudolf) ‰È·Ì¤ÛÔ˘ ÂÓfi˜ Ï·ÛÙÈÎÔ‡ Ûˆ-
Ï‹Ó· ¯·ÌËÏ‹˜ ·ÓÙ›ÛÙ·Û˘ Û ¤Ó· Û¿ÎÔ ·Ó¿ÌÂÈ͢ ·Â-
Ú›ˆÓ 7 Ï›ÙÚˆÓ. ÃÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Ì¤ıÔ‰Ô˜ ·Ó¿Ï˘Û˘
·ÂÚ›ˆÓ Û οı ·Ó·ÓÔ‹ Î·È ˘‹Ú¯Â Ô Ì¤ÛÔ˜ fiÚÔ˜ ÙˆÓ
ÙÈÌÒÓ Î¿ı 35 sec. √È ·ÙÌÔÛÊ·ÈÚÈΤ˜ Û˘Óı‹Î˜ ıÂÚÌÔ-
ÎÚ·Û›·˜ Î·È ›ÂÛ˘ ηٷÁÚ¿ÊÔÓÙ·Ó. √È ÂÛˆÙÂÚÈÎÔ› ·È-
ÛıËÙ‹Ú˜ ÙÔ˘ Ì˯·Ó‹Ì·ÙÔ˜ ÂϤÁ¯ÔÓÙ·Ó ¿ÓÙ· ÚÈÓ
·fi ÙË ‰ÔÎÈÌ·Û›· ÁÈ· ÙËÓ ÂÏ¿ÙÙˆÛË Ù˘ Èı·ÓfiÙËÙ·˜
442 ∆. ªÔ‡‰ÈÔ˘ Î·È Û˘Ó.
Ï¿ıÔ˘˜. √È ·ÛıÂÓ›˜ ÚÈÓ ·fi ÙË ‰ÔÎÈÌ·Û›· ‹Ù·Ó ÓË-
ÛÙÈÎÔ›, ·ÏÏ¿ ›¯·Ó Ï¿‚ÂÈ fiÏ· Ù· Ê¿Ú̷ο ÙÔ˘˜, ÂÎÙfi˜
·fi Ù· ‚2 ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο, ÁÈ· Ù· ÔÔ›· ‰fiıËÎÂ
Ô‰ËÁ›· Ó· ‰È·ÎÔÔ‡Ó ÙÔ˘Ï¿¯ÈÛÙÔÓ ÁÈ· 12 ÒÚ˜. √ ÂÎ-
ÓÂfiÌÂÓÔ˜ fiÁÎÔ˜ ·¤Ú· ÌÂÏÂÙ‹ıËΠ› 25 ÏÂÙ¿. ∏
ÛÙ·ıÂÚ‹ ηٿÛÙ·ÛË ·ÂÚÈÛÌÔ‡ ıˆÚÔ‡ÓÙ·Ó fiÙÈ ÂÈÙ‡-
¯ıËΠfiÙ·Ó Ô Ì¤ÛÔ˜ fiÚÔ˜ ÌÂÙ·‚ÔÏÒÓ Ù˘ VO2 Î·È VCO2
‹Ù·Ó ÏÈÁfiÙÂÚÔ ·fi 10% Î·È Ô Ì¤ÛÔ˜ fiÚÔ˜ ÌÂÙ·‚ÔÏÒÓ
ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ËÏ›ÎÔ˘ ‹Ù·Ó ÌÈÎÚfiÙÂÚÔ˜ ·fi 5%
(14-16). ∞˘Ùfi ÂÈÙ‡¯ıËΠÛÙ· ÚÒÙ· 5 ÏÂÙ¿ Ù˘ ̤-
ÙÚËÛ˘ Û fiϘ ÙȘ ÂÚÈÙÒÛÂȘ. ∞Ó ‰ÂÓ ‹Ù·Ó ÂÊÈÎÙ‹ Ë
ηٿÛÙ·ÛË ÈÛÔÚÚÔ›·˜, Ë ‰ÔÎÈÌ·Û›· ·ӷϷ̂·Ófi-
Ù·Ó ¿ÏÏË Ë̤ڷ. ™Â fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ‰ÔÎÈÌ·Û›·˜
˘‹Ú¯Â ›‚Ï„Ë. √È ÌÂÙÚ‹ÛÂȘ ·Ì¤Ûˆ˜ ÌÂÙ¿ ·fi
ÂÂÈÛfi‰ÈÔ ‚‹¯· ‹ ÎÈÓ‹ÛÂˆÓ ÙÔ˘ ÛÒÌ·ÙÔ˜, ÂÍ·ÈÚ¤ıËηÓ.
∂›Û˘, Ù· ‰Â‰Ô̤ӷ ÙˆÓ ÚÒÙˆÓ 5 ÏÂÙÒÓ ‰ÂÓ Ï·Ì-
‚¿ÓÔÓÙ·Ó ˘’ fi„ÈÓ. ∞fi ÙȘ ÌÂÙÚ‹ÛÂȘ ÙˆÓ ˘fiÏÔȈÓ
20 ÏÂÙÒÓ ˘ÔÏÔÁ›ÛÙËÎÂ Ô Ì¤ÛÔ˜ fiÚÔ˜ ÁÈ· ÙÔ Î·Ù·Ó·-
ÏÔ‡ÌÂÓÔ √2 (VO2) Î·È ÙÔ ÂÎÓÂfiÌÂÓÔ ‰ÈÔÍ›‰ÈÔ ÙÔ˘ ¿Ó-
ıڷη (VCO2). °È· ÙÔÓ ˘ÔÏÔÁÈÛÌfi Ù˘ ∫∂∏ ¯ÚËÛÈÌÔ-
ÔÈ‹ıËÎÂ Ë Â͛ۈÛË ÙÔ˘ Weir (17,18) ∫∂∏ =
[3,941*VO2+1,11*VCO2]*1,44.
™ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘, ÙÚÂȘ ·ÛıÂÓ›˜ ¯ÚÂÈ¿-
ÛÙËΠӷ ·ӷϿ‚Ô˘Ó ÙË ‰ÔÎÈÌ·Û›·. ∏ ∫∂∏ ÂÎÊÚ¿-
ÛÙËΠ۠ıÂÚÌ›‰Â˜ ·Ó¿ Ë̤ڷ Î·È ¤ÁÈÓ ۇÁÎÚÈÛË ÌÂ
ÚÔ‚ÏÂfiÌÂÓ˜ ÙÈ̤˜ ÁÈ· ÙÔ Ê‡ÏÔ, ÙËÓ ËÏÈΛ·, ÙÔ ‚¿-
ÚÔ˜ Î·È ÙÔ ‡„Ô˜, ÔÈ Ôԛ˜ ˘ÔÏÔÁ›ÛÙËÎ·Ó ·fi ÙȘ ÂÍÈ-
ÛÒÛÂȘ ÙÔ˘ Schofield-HW (19-21). √È ÂÍÈÛÒÛÂȘ ·˘Ù¤˜
¤ÁÈÓ·Ó ·fi ÙÔÓ Schofield ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙˆÓ ÂÓÂÚ-
ÁÂÈ·ÎÒÓ ·Ó·ÁÎÒÓ Û ˘ÁÈ‹ ¿ÙÔÌ·, ÛÂ Ê˘ÛÈÔÏÔÁÈÎfi ÂÚÈ-
‚¿ÏÏÔÓ Î·È Ì ηϋ Ê˘ÛÈ΋ ηٿÛÙ·ÛË Î·È ÂÚÈÏ·Ì‚¿-
ÓÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1.
∏ ÚÔ‚ÏÂfiÌÂÓË Â› ÙÔȘ ÂηÙfi ∫∂∏ ÔÚ›˙ÂÙ·È ·fi
ÙË Û¯¤ÛË: ∫∂∏% = [ªÂÙÚÔ‡ÌÂÓË ∫∂∏/¶ÚÔ‚ÏÂfiÌÂÓË
∫∂∏] x 100.
∞fi ÙËÓ ·Ú·¿Óˆ Û¯¤ÛË ‰È·ÈÛÙÒÓÂÙ·È fiÙÈ fiÙ·Ó
Ë ÙÈÌ‹ Ù˘ ∫∂∏, Ë ÔÔ›· ÌÂÙÚ¿Ù·È ÛÙÔ ÂÚÁ·ÛÙ‹ÚÈÔ
ÏËÛÈ¿˙ÂÈ ·˘Ù‹ Ù˘ ÚÔ‚ÏÂfiÌÂÓ˘ ·fi ÙȘ ÂÍÈÛÒÛÂȘ
Schofield-HW, ÙfiÙÂ Ë ÚÔ‚ÏÂfiÌÂÓË Â› ÙÔȘ ÂηÙfi
∫∂∏ (∫∂∏%) ÏËÛÈ¿˙ÂÈ ÙÔ 100%.
∞ÓıÚˆÔÌÂÙÚÈο ‰Â‰Ô̤ӷ
∏ ̤ÙÚËÛË ÙÔ˘ ‡„Ô˘˜ (±0,1 cm) ¤ÁÈÓ Ì ÙË ¯Ú‹ÛË
·Ó·ÛÙËÌfiÌÂÙÚÔ˘ SECA, ÂÓÒ Ë Ì¤ÙÚËÛË ÙÔ˘ ‚¿ÚÔ˘˜
(±0,1 kg) Ì ÙË ¯Ú‹ÛË ËÏÂÎÙÚÔÓÈ΋˜ ˙˘Á·ÚÈ¿˜ ‰¿ÊÔ˘˜
(SECA model 713 max 130 kg, min 2 kg) Ì ÙÔ˘˜ ·ÛıÂ-
Ó›˜ Ôχ ÂÏ·ÊÚ¿ ÓÙ˘Ì¤ÓÔ˘˜. ŸÏ˜ ÔÈ ÌÂÙÚ‹ÛÂȘ ¤ÁÈÓ·Ó
·ÎÔÏÔ˘ıÒÓÙ·˜ ÙȘ ÎÏ·ÛÈΤ˜ Ù¯ÓÈΤ˜ Î·È Â·Ó·Ï‹ÊıË-
Î·Ó ‰Â‡ÙÂÚË ÊÔÚ¿ ÁÈ· ÂȂ‚·›ˆÛË (22,23).
√ ‰Â›ÎÙ˘ Ì¿˙·˜ ÛÒÌ·ÙÔ˜ (¢ª™) ˘ÔÏÔÁ›˙ÂÙ·È ˆ˜
ÙÔ ËÏ›ÎÔ ÙÔ˘ ‚¿ÚÔ˘˜ ÛÒÌ·ÙÔ˜ (kg) ÚÔ˜ ÙÔ ‡„Ô˜ (m)
ÛÙÔ ÙÂÙÚ¿ÁˆÓÔ.
∂ÎÙ›ÌËÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È Ù˘
ÎÏÈÓÈ΋˜ ηٿÛÙ·Û˘
√È ‰ÔÎÈ̷ۛ˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ¤ÁÈ-
Ó·Ó Û ·ÓÔÈÎÙfi ·Îψ̷ ÛÈÚÔ̤ÙÚËÛ˘ (JAEGER
EOS-Sprint Oxygen Analyzer, Würzburg, Germany).
¶ÚÈÓ ·fi ÙË Ì¤ÙÚËÛË ÁÈÓfiÙ·Ó Â›‰ÂÈÍË Ù˘ ‰È·‰Èηۛ·˜
̤ÙÚËÛ˘ Î·È ÛÙË Û˘Ó¤¯ÂÈ· ‰ÔÎÈÌ·ÛÙÈΤ˜ ÚÔÛ¿ıÂȘ
·fi ÙÔ˘˜ ·ÛıÂÓ›˜, ̤¯ÚÈ ÙËÓ ÈηÓÔÔÈËÙÈ΋ ÂÍÔÈΛˆ-
Û‹ ÙÔ˘˜ Ì ÙË ‰È·‰Èηۛ·. ªÂ ÙÔÓ ÙÚfiÔ ·˘Ùfi ÌÂÙÚ‹-
ıËÎÂ Ô ‚›·È· ÂÎÓÂfiÌÂÓÔ˜ fiÁÎÔ˜ ·¤Ú· Û ¤Ó· ‰Â˘ÙÂÚfi-
ÏÂÙÔ (FEV1), Ë ‰˘Ó·ÌÈ΋ ˙ˆÙÈ΋ ¯ˆÚËÙÈÎfiÙËÙ· (FVC)
Î·È Ë Ì¤ÁÈÛÙË ÂÎÓ¢ÛÙÈ΋ ÚÔ‹ (PEFR) Î·È ÂÎÊÚ¿ÛÙË-
Î·Ó ˆ˜ ÔÛÔÛÙfi ÙÔ˘ ÚÔ‚ÏÂfiÌÂÓÔ˘ ÁÈ· ÙÔ ·ÓÙ›ÛÙÔȯÔ
‡„Ô˜ Î·È Ê‡ÏÔ.
∏ ÎÏÈÓÈ΋ ηٿÛÙ·ÛË ÚÔÛ‰ÈÔÚ›ÛÙËΠ̠¤ÏÂÁ¯Ô
ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ Î·È Ù˘ C-·ÓÙÈ-
‰ÚÒÛ·˜ ÚˆÙ½Ó˘, Ù· ÔÔ›· ÌÂÙÚ‹ıËÎ·Ó Ì ÙȘ ÎÏ·ÛÈ-
Τ˜ Ù¯ÓÈΤ˜, Ì›· Ë̤ڷ ÚÈÓ ÙË Ì¤ÙÚËÛË Ù˘ ∫∂∏.
∆·˘Ùfi¯ÚÔÓ· ¤ÁÈÓÂ Î·È Ë ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη, Ë ÔÔ›·
·ÍÈÔÏÔÁ‹ıËΠηٿ Brasfield (24).
√È Î·ÏÏȤÚÁÂȘ Ù˘¤ÏˆÓ ÂÏ‹ÊıËÛ·Ó ÙËÓ Ë̤ڷ
Ù˘ ‰ÔÎÈÌ·Û›·˜. √ ÎÔÚÂÛÌfi˜ ÙÔ˘ Ô͢ÁfiÓÔ˘ (SatO2) ÌÂ-
ÙÚ‹ıËΠ̠·ÏÌÈÎfi Ô͇ÌÂÙÚÔ (BCI International
Waukes, HA Wisconcin Model 71000A1 Cat No 3301)
ÛÙËÓ ·Ú¯‹ Ù˘ ‰ÔÎÈÌ·Û›·˜. √ ‰Â›ÎÙ˘ ÎÏÈÓÈ΋˜ ηٿ-
ÛÙ·Û˘ ηıÔÚ›ÛÙËΠۇÌʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· ÙˆÓ
Shwachman-Kulczycki (25), ·fi ÙÔÓ ›‰ÈÔ ÂÍÂÙ·ÛÙ‹ ÁÈ·
fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜.
™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË
∏ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ¤ÁÈÓ Ì ÙË
¯Ú‹ÛË ÙÔ˘ ÂÍÂȉÈÎÂ˘Ì¤ÓÔ˘ ÛÙ·ÙÈÛÙÈÎÔ‡ ·Î¤ÙÔ˘ SPSS
v.11.0. ∆· ·ÔÙÂϤÛÌ·Ù· ÂÎÊÚ¿ÛÙËÎ·Ó ˆ˜ ̤ÛË ÙÈÌ‹
± Ù˘ÈÎfi ÛÊ¿ÏÌ·. ∆· ‰Â‰Ô̤ӷ Ù˘ ∫∂∏ ·fi ÙȘ ÌÂ-
ÙÚ‹ÛÂȘ Û˘ÁÎÚ›ıËÎ·Ó Ì ·ÓÙ›ÛÙÔȯ˜ ÙÈ̤˜ ÚÔ‚ÏÂfi-
ÌÂÓ˘ ∫∂∏ ·fi ÙȘ ÂÍÈÛÒÛÂȘ ÙÔ˘ Schofield-HW, ÛÙÔ
Ï·›ÛÈÔ ÙÔ˘ ‰›Ï¢ÚÔ˘ ÂϤÁ¯Ô˘ Ù˘ ηٷÓÔÌ‹˜
Student’s t test (two-tailed paired Student’s t-test). ∏
ÚÔ‚ÏÂfiÌÂÓË Â› ÙÔȘ ÂηÙfi ∫∂∏ (KEH%) Û˘ÁÎÚ›-
ıËΠ̠ÙËÓ ÙÈÌ‹ ÙÔ˘ 100%, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ Î·È ¿-
ÏÈ ÙÔ t student’s test (‰›Ï¢ÚÔ˜ ¤ÏÂÁ¯Ô˜ Ù˘ ηٷÓÔ-
Ì‹˜). √È Û˘Û¯ÂÙ›ÛÂȘ ÌÂٷ͇ ÙˆÓ ∫∂∏, ∫∂∏%
Î·È FEV1%, FVC%, PEFR%, SatO2% Î·È ÎÏÈÓÈÎÔ‡ ‰Â›-
ÎÙË Shwachman ÌÂÏÂÙ‹ıËÎ·Ó ÛÙÔ Ï·›ÛÈÔ Ù˘ ıˆ-
Ú›·˜ Ù˘ ·ÏÈÓ‰ÚfiÌËÛ˘ (Linear regression analysis).
√ Û¯ÂÙÈÎfi˜ Û˘ÓÙÂÏÂÛÙ‹˜ Û˘Û¯¤ÙÈÛ˘ Pearson ˘ÔÏÔ-
Á›ÛÙËΠÁÈ· ÙËÓ ÂÚ·ÈÙ¤Úˆ ·Ó¿Ï˘ÛË Ù˘ Û˘Û¯¤ÙÈÛ˘
ÌÂٷ͇ Ù˘ ηٷӿψÛ˘ ÂÓ¤ÚÁÂÈ·˜ Î·È ÙˆÓ ·Ú·¿-
Óˆ ÎÏÈÓÈÎÒÓ ‰Â‰Ô̤ӈÓ. ∆Ô Â›Â‰Ô ·ÍÈÔÈÛÙ›·˜
ÛÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ÔÚ›ÛÙËΠ̠ÙÈ̤˜ p <0,05
ÙÔ˘Ï¿¯ÈÛÙÔÓ.
¶·È‰È·ÙÚÈ΋ 2006;69:440-446
¶›Ó·Î·˜ 1. ∂ÍÈÛÒÛÂȘ Schofield-HW ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi Ù˘ ∫∂∏ (Kcal/Ë̤ڷ) ·fi ÙÔ ‡„Ô˜ (height-H) Î·È ÙÔ ‚¿ÚÔ˜(weight-W) Û ¿ÚÚÂÓ˜ Î·È ı‹ÏÂȘ
∏ÏÈΛ· (¤ÙË) ÕÚÚÂÓ˜ £‹ÏÂȘ
<3 0,167W + 1517,4H - 617,6 16,252W + 1023,2H - 413,53-10 19,59W + 130,3H + 414,9 16,969W + 161,8H + 371,210-18 16,25W + 137,2H + 515,5 8,365W + 465,0H + 200,0>18 15,057W + 10,04H + 705,8 13,623W + 283,0H + 98,2
443∫·Ù·Ó¿ÏˆÛË ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜ ÛÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË
∞ÔÙÂϤÛÌ·Ù·
∆· ·ÓıÚˆÔÌÂÙÚÈο ‰Â‰Ô̤ӷ Î·È Ù· ‰Â‰Ô-
̤ӷ ÁÈ· ÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·ÛıÂ-
ÓÒÓ Ì ∫π ηٷÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 2. H
KEH ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π ‚Ú¤ıËΠÌÂÁ·Ï‡ÙÂ-
ÚË ·fi ÙȘ ÚÔ‚ÏÂfiÌÂÓ˜ ÙÈ̤˜ Ì ‚¿ÛË ÙȘ
ÂÍÈÛÒÛÂȘ Schofield-HW, 1626±49 Û˘ÁÎÚÈÓfiÌÂ-
ÓË ÌÂ 1472±72 (t=4,54, p<0,0001). ∏ ∫∂∏%
·ÓÙÈÛÙÔȯ› ÛÙÔ 110,96±2,35% (p<0,001) Ù˘
ÚÔ‚ÏÂfiÌÂÓ˘ Î·È ¿ÏÈ Ì ‚¿ÛË ÙȘ ÂÍÈÛÒÛÂȘ
Schofield-HW.
√ ̤ÛÔ˜ fiÚÔ˜ ÁÈ· ÙËÓ ∫∂∏ ÛÙ· ·ÁfiÚÈ· Î·È ÛÙ·
ÎÔÚ›ÙÛÈ· ‚Ú¤ıËΠ1778,7±72,9 Î·È 1473,19±43,41,
·ÓÙ›ÛÙÔȯ·. ∞˘Ùfi ·ÓÙÈÛÙÔȯ› Û ∫∂∏%
109,36±3,08% (p<0,007) Î·È 112,56±3,6 %
(p<0,003), ·ÓÙ›ÛÙÔȯ· (¶›Ó·Î·˜ 3). ∞Ó Î·È Ô Ì¤-
ÛÔ˜ fiÚÔ˜ Ù˘ ∫∂∏% ‹Ù·Ó ·˘ÍË̤ÓÔ˜ Û ۯ¤ÛË
Ì ÙȘ ÚÔ‚ÏÂfiÌÂÓ˜ ÙÈ̤˜ Î·È ÛÙ· ‰‡Ô ʇϷ,
ˆÛÙfiÛÔ ·˘Ùfi˜ ÛÙ· ÎÔÚ›ÙÛÈ· ‹Ù·Ó ·ÎfiÌË ÈÔ ·˘-
ÍË̤ÓÔ˜ Û ۯ¤ÛË Ì ٷ ·ÁfiÚÈ·.
∏ ÁÚ·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË ÙˆÓ ‰Â‰Ô̤ӈÓ
¤‰ÂÈÍ fiÙÈ ˘¿Ú¯ÂÈ ·ÚÓËÙÈ΋ Û˘Û¯¤ÙÈÛË Ù˘
∫∂∏% Ì ÙÔÓ ÎÏÈÓÈÎfi ‰Â›ÎÙË Shwachman,
FEV1%, FVC% Î·È PEFR%. ¢ÂÓ ‚Ú¤ıËÎÂ Û˘-
Û¯¤ÙÈÛË ÌÂٷ͇ ÙÔ˘ ÎÔÚÂÛÌÔ‡ Ô͢ÁfiÓÔ˘
(SatO2) Î·È Ù˘ ∫∂∏%. ™˘ÁÎÂÎÚÈ̤ӷ, ‚Ú¤ıËÎÂ
ÛËÌ·ÓÙÈ΋ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙÔ˘ ÎÏÈÓÈÎÔ‡ ‰Â›-
ÎÙË Shwachman Î·È ∫∂∏%, ¯ˆÚ›˜ ÛÙ·ÙÈÛÙÈο
ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ·Ó¿ÌÂÛ· ÛÙ· ‰‡Ô ʇϷ. ∏
ÁÚ·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË ·Ó¿ÌÂÛ· ÛÙÔÓ ‰Â›ÎÙË
Shwachman Î·È ÛÙËÓ ∫∂∏% ÂÎÊÚ¿˙ÂÙ·È Ì ÙÔÓ
Û˘ÓÙÂÏÂÛÙ‹ Pearson Î·È Ê·›ÓÂÙ·È ÛÙËÓ ∂ÈÎfiÓ· 1(r=-0,654, p<0,0001 fiÏÔÈ ÔÈ ·ÛıÂÓ›˜, r=-0,681Î·È r=-0,662 ·ÁfiÚÈ· Î·È ÎÔÚ›ÙÛÈ·, ·ÓÙ›ÛÙÔȯ·).∂›Ó·È Ê·ÓÂÚfi fiÙÈ Î·ıÒ˜ Ô ÎÏÈÓÈÎfi˜ ‰Â›ÎÙ˘ÂÏ·ÙÙÒÓÂÙ·È, Ë ∫∂∏% ·˘Í¿ÓÂÙ·È. ∏ ÁÚ·ÌÌÈÎ‹Û˘Û¯¤ÙÈÛË ÌÂٷ͇ FEV1% Î·È ∫∂∏% Ê·›ÓÂÙ·ÈÛÙËÓ ∂ÈÎfiÓ· 2 (r=-0,475, p<0,003 fiÏÔÈ ÔÈ ·ÛıÂ-Ó›˜, r=-0,484 Î·È r=-0,510 ·ÁfiÚÈ· Î·È ÎÔÚ›ÙÛÈ·,·ÓÙ›ÛÙÔȯ·). ∏ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙˆÓ FVC%Î·È ∫∂∏% Ê·›ÓÂÙ·È ÛÙËÓ ∂ÈÎfiÓ· 3 (r=-0,570,p<0,0001 fiÏÔÈ ÔÈ ·ÛıÂÓ›˜, r=-0,570 Î·È r=-0,610·ÁfiÚÈ· Î·È ÎÔÚ›ÙÛÈ·, ·ÓÙ›ÛÙÔȯ·). ∂›Ó·È ۷ʤ˜fiÙÈ ˘¿Ú¯ÂÈ ÛËÌ·ÓÙÈ΋ ·ÚÓËÙÈ΋ ÁÚ·ÌÌÈ΋ Û˘-Û¯¤ÙÈÛË ·Ó¿ÌÂÛ· ÛÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›·Î·È ÛÙËÓ ∫∂∏%. ∆¤ÏÔ˜, Ë Û˘Û¯¤ÙÈÛË ÌÂٷ͇PEFR% Î·È ∫∂∏% ·ÚÔ˘ÛÈ¿˙ÂÙ·È ÛÙËÓ ∂ÈÎfiÓ·4 (r=-0,39, p<0,016 fiÏÔÈ ÔÈ ·ÛıÂÓ›˜, r=-0,440Î·È r=-0,310 ·ÁfiÚÈ· Î·È ÎÔÚ›ÙÛÈ·, ·ÓÙ›ÛÙÔȯ·).
™˘˙‹ÙËÛË
™Â fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂ-ϤÙ˘ Ì ∫π, Ô ¢ª™ ‹Ù·Ó >18,5. ∞˘Ùfi ‰Â›¯ÓÂÈηϋ ηٿÛÙ·ÛË ıÚ¤„˘, ·Ú¿ ÙËÓ ·ÁÎÚ·ÙÈ-΋ ·Ó¿ÚÎÂÈ·, Ë ÔÔ›· ¯·Ú·ÎÙ‹ÚÈ˙ ÙËÓ ÏÂÈ-Ô„ËÊ›· ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÌÂϤÙ˘. ∏ ηٿÏÏË-ÏË ·ÓÙÈÌÂÙÒÈÛË ÛÂ Û˘Ó‰˘·ÛÌfi Ì ‰È·ÈÙËÙÈ΋˘ÔÛÙ‹ÚÈÍË ÂÍËÁÔ‡Ó ÙÔÓ Ê˘ÛÈÔÏÔÁÈÎfi ¢ª™.
∞fi ÙȘ ̤Û˜ ÙÈ̤˜ ÙˆÓ FEV1% Î·È FVC%Û˘ÌÂÚ·›ÓÂÙ·È fiÙÈ Ë Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›·ÙˆÓ ·ÛıÂÓÒÓ ‹Ù·Ó ̤ÙÚÈ· ÂËÚ·Ṳ̂ÓË. ∏ ̤-ÛË ÙÈÌ‹ ÙÔ˘ FEV1% Î·È Ù˘ FVC% ÛÙ· ÎÔÚ›ÙÛÈ·‚Ú¤ıËΠÂÏ·ÊÚ¿ ˘„ËÏfiÙÂÚË ·fi fi,ÙÈ ÛÙ· ·Áfi-ÚÈ·. ∏ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰Ô̤ӈӤ‰ÂÈÍ fiÙÈ Ë ‰È·ÊÔÚ¿ ·˘Ù‹ ‰ÂÓ ‹Ù·Ó ÛÙ·ÙÈÛÙÈ-ο ÛËÌ·ÓÙÈ΋. ªÂ ÎÚÈÙ‹ÚÈÔ ÙË Ì¤ÛË ÙÈÌ‹ ÙÔ˘‰Â›ÎÙË Shwachman, ÔÈ ·ÛıÂÓ›˜ ηٿ ̤ÛÔÓ fiÚÔ‚Ú›ÛÎÔÓÙ·Ó Û ηϋ ÎÏÈÓÈ΋ ηٿÛÙ·ÛË (25).
™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π Î·È ¯·Ú·ÎÙËÚÈÛÙÈο̤ÙÚÈ·˜ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È Î·Ï‹˜ÎÏÈÓÈ΋˜ ηٿÛÙ·Û˘ ¯ˆÚ›˜ ÚfiÛÊ·ÙË Ïԛ̈ÍË,
¶·È‰È·ÙÚÈ΋ 2006;69:440-446
¶›Ó·Î·˜ 3. ∏ ÌÂÙÚÔ‡ÌÂÓË Î·Ù·Ó¿ÏˆÛË ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜ (∫∂∏), Ë ÚÔ‚ÏÂfiÌÂÓË ∫∂∏ ·fi ÙȘ ÂÍÈÛÒÛÂȘ Schofield-HWÎ·È ∫∂∏% ˆ˜ ÔÛÔÛÙfi Ù˘ ÚÔ‚ÏÂfiÌÂÓ˘ ·fi ÙȘ ÂÍÈÛÒÛÂȘ Schofield-HW. ∆È̤˜ p Î·È t ·fi ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ,Ì ÙË ¯Ú‹ÛË ÙÔ˘ ‰›Ï¢ÚÔ˘ ÂϤÁ¯Ô˘ Ù˘ ηٷÓÔÌ‹˜ Student t test.
ªÂÙÚÔ‡ÌÂÓË ∫∂∏ Schofield-HW ªÂÙÚÔ‡ÌÂÓË ∫∂∏ ∫∂∏% ∫∂∏% vs 100%(Kcal/Ë̤ڷ) Û ۯ¤ÛË Ì Schofield
t p t p
™˘ÓÔÏÈο 1625,95±48,8 1472,36±39,56 4,54 0,0001 110,96±2,35 4,658 0,0001ÕÚÚÂÓ˜ 1778,7±72,9 1626,94±51,78 3,008 0,008 109,36±3,08 3,037 0,007£‹ÏÂȘ 1473,19±43,41 1317,79±33,15 3,35 0,004 112,56±3,6 3,489 0,003
∞ÔÙÂϤÛÌ·Ù· ÂÎÊÚ·Ṳ̂ӷ ˆ˜ ̤ÛË ÙÈÌ‹ ± Ù˘ÈÎfi ÛÊ¿ÏÌ·
¶›Ó·Î·˜ 2. ∞ÓıÚˆÔÌÂÙÚÈο ÛÙÔȯ›· Î·È ‰Â‰Ô̤ӷ ·fiÙËÓ ·Ó·Ó¢ÛÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· ·ÛıÂÓÒÓ Ì ∫π
™˘ÓÔÏÈο ÕÚÚÂÓ˜ £‹ÏÂȘ
∏ÏÈΛ· 19,72±1,0 19,25±1,37 20,21±1,46µ¿ÚÔ˜ 54,2±2,2 57,1±3,35 51,87±2,77¢ª™ 20,5±0,63 20,4±0,9 20,4±0,6FEV1% 76,39±4,49 73,31±6,51 79,47±6,26FVC% 79,96±4,0 77,58±6,0 88,34±5,46
444 ∆. ªÔ‡‰ÈÔ˘ Î·È Û˘Ó.
Ë KEH ‚Ú¤ıËΠ·˘ÍË̤ÓË. ∞˘Ùfi Û˘ÌʈÓ› ÌÂÙË ‚È‚ÏÈÔÁÚ·Ê›· (10,11). ™Ù· ·ÁfiÚÈ·, Ë ∫∂∏‹Ù·Ó ˘„ËÏfiÙÂÚË ·fi fi,ÙÈ ÛÙ· ÎÔÚ›ÙÛÈ·, fiˆ˜·Ó·ÌÂÓfiÙ·Ó (6,26). ∏ ‰È·ÊÔÚ¿ ·Ô‰fiıËΠÛÙÔÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ¿ÏÈ˘ Ì¿˙·˜ ÛÙ· ·ÁfiÚÈ·Û ۯ¤ÛË Ì ٷ ÎÔÚ›ÙÛÈ· (6,27-29). ™ÙÔ˘˜ ›‰ÈÔ˘˜·ÛıÂÓ›˜, Ë ÚÔ‚ÏÂfiÌÂÓË ∫∂∏% ‹Ù·Ó ›Û˘·˘ÍË̤ÓË Î·È ÛÙ· ‰‡Ô ʇϷ, ·ÏÏ¿ Û ·ÓÙ›ıÂÛËÌ ÙË ÌÂÙÚÔ‡ÌÂÓË ÙÈÌ‹ Ù˘ ∫∂∏, Ë ∫∂∏% ÛÙ·ÎÔÚ›ÙÛÈ· ‚Ú¤ıËΠ˘„ËÏfiÙÂÚË ·fi fi,ÙÈ ÛÙ· ·Áfi-ÚÈ· (112% Î·È 109%, ·ÓÙ›ÛÙÔȯ·). ∏ ∫∂∏% ÔÚ›-ÛÙËΠˆ˜ ÙÔ Â› ÙÔȘ ÂηÙfi ÔÛÔÛÙfi Ù˘ ÚÔ-‚ÏÂfiÌÂÓ˘ ∫∂∏, Û˘ÁÎÚÈÓfiÌÂÓË Ì ·ÓÙ›ÛÙÔÈ-¯Â˜ ÙÈ̤˜ ˘ÁÈÒÓ ·ÙfiÌˆÓ Ì ٷ ›‰È· ۈ̷ÙÔÌÂ-ÙÚÈο ¯·Ú·ÎÙËÚÈÛÙÈο (26,30). ∏ ÌÂÙÚÔ‡ÌÂÓË∫∂∏ ÂÍ·ÚÙ¿Ù·È ¤ÓÙÔÓ· ·fi Ù· ۈ̷ÙÔÌÂÙÚÈο¯·Ú·ÎÙËÚÈÛÙÈο, ÂÓÒ ·ÓÙ›ıÂÙ· Ë ∫∂∏% ·ÓÙÈ-ÚÔۈ‡ÂÈ ÙËÓ ·fiÎÏÈÛË Ù˘ ηٷӿψÛ˘ÂÓ¤ÚÁÂÈ·˜ ÙÔ˘ ·ÛıÂÓ‹ Û ۯ¤ÛË Ì ·˘Ù‹ Ô˘ÚԂϤÂÙ·È ÁÈ· ¤Ó· ·ÓÙ›ÛÙÔÈ¯Ô ˘ÁȤ˜ ¿ÙÔÌÔ.√È Û˘ÁÎÚ›ÛÂȘ ‚·Û›ÛÙËÎ·Ó ÛÙȘ ÂÍÈÛÒÛÂȘ ÙÔ˘Schofield-HW (30). ∆Ô Î·Ù·ÏÏËÏfiÙÂÚÔ Ì¤ÁÂıÔ˜ÁÈ· ÙË Û˘Û¯¤ÙÈÛË Ù˘ ηٷӿψÛ˘ ÂÓ¤ÚÁÂÈ·˜ÛÙËÓ ËÚÂÌ›· Ì ÙËÓ ÎÏÈÓÈ΋ ηٿÛÙ·ÛË ·ÛıÂ-
ÓÒÓ Ì ∫π Â›Ó·È Ë ∫∂∏% (30), ÂÓÒ Ë ÌÂÙÚÔ‡ÌÂ-
ÓË ∫∂∏ ‰›ÓÂÈ ÏËÚÔÊÔڛ˜ ÁÈ· ÙȘ ÂÓÂÚÁÂȷΤ˜
ÙÔ˘˜ ·Ó¿ÁΘ. ¶ÂÚ·ÈÙ¤Úˆ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË
ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ÁÈ· ÙËÓ ∫∂∏% ¤‰ÂÈÍ fiÙÈ Ë ‰È·-
ÊÔÚ¿ Ù˘ KEH% ·Ó¿ÌÂÛ· ÛÙ· ·ÁfiÚÈ· Î·È ÛÙ·
ÎÔÚ›ÙÛÈ·, Ë ÔÔ›· ·Ó·Ê¤ÚıËΠ·Ú·¿Óˆ, ‰ÂÓ
Â›Ó·È ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋. ∞˘Ùfi Ê·›ÓÂÙ·È Ó·
¤Ú¯ÂÙ·È Û ·ÓÙ›ıÂÛË Ì ٷ Â˘Ú‹Ì·Ù· ÙˆÓ Allen
Î·È Û˘Ó (26), ÔÈ ÔÔ›ÔÈ ‚Ú‹Î·Ó ‰È·ÊÔÚ¿ ·Ó¿ÌÂ-
Û· ÛÙ· ‰‡Ô ʇϷ, ·ÎÔÏÔ˘ıÒÓÙ·˜ fï˜, ‰È·ÊÔ-
ÚÂÙÈ΋ ÌÂıÔ‰ÔÏÔÁ›·.
∏ Û˘Û¯¤ÙÈÛË Ù˘ ∫∂∏% Ì ÙËÓ Ó¢ÌÔÓÈ΋
ÏÂÈÙÔ˘ÚÁ›· ‰Â›¯ÓÂÈ ÌÈ· ÛËÌ·ÓÙÈ΋ ·ÚÓËÙÈ΋
ÁÚ·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ∫∂∏% Î·È FEV1%,
FVC%. ∞ÓÙ›ıÂÙ·, Ë ÌÂÙÚÔ‡ÌÂÓË ∫∂∏ ¤¯ÂÈ ÛÙ·ÙÈ-
ÛÙÈο ÛËÌ·ÓÙÈ΋ Û˘Û¯¤ÙÈÛË Ì FEV1% ηÈ
FVC% ÌfiÓÔ ÛÙ· ·ÁfiÚÈ·. øÛÙfiÛÔ, fiˆ˜ ¤¯ÂÈ ·Ó·-
ÊÂÚı›, Ë ¯Ú‹ÛË Ù˘ ∫∂∏ ·ÓÙ› Ù˘ ∫∂∏% ÌÔ-
Ú› Ó· Ô‰ËÁ‹ÛÂÈ Û ϷÓı·Ṳ̂ӷ Û˘ÌÂÚ¿ÛÌ·Ù·.
πÛ¯˘Ú‹ Û˘Û¯¤ÙÈÛË ‚Ú¤ıËΠÌÂٷ͇ Ù˘
∫∂∏% Î·È ÙÔ˘ ÎÏÈÓÈÎÔ‡ ‰Â›ÎÙË Shwachman.
¶·È‰È·ÙÚÈ΋ 2006;69:440-446
100
95
90
85
80
75
70
65
6070 80 90 100 110 120 130 140
y=-0,3288x+123,48r=-0,654 p<0,0001
Shw
ach
man
KEH%
∂ÈÎfiÓ· 1. °Ú·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË ÎÏÈÓÈÎÔ‡ ‰Â›ÎÙË ShwachmanfiÏˆÓ ÙˆÓ ·ÛıÂÓÒÓ Ì ÙËÓ ∫∂∏%. ŸÛÔ ÂÏ·ÙÙÒÓÂÙ·È Ô ‰Â›-ÎÙ˘ Shwachman, ÙfiÛÔ ·˘Í¿ÓÂÙ·È Ë ∫∂∏%.
160
140
120
100
80
60
40
20
070 80 90 100 110 120 130 140
y=-0,9061x+176,95r=-0,475 p<0,003
FE
V1%
∫∂∏%
∂ÈÎfiÓ· 2. °Ú·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË FEV1% fiÏˆÓ ÙˆÓ ·ÛıÂÓÒÓÌ ÙËÓ ∫∂∏%. ŸÛÔ ÂÏ·ÙÙÒÓÂÙ·È Ô FEV1%, ÙfiÛÔ ·˘Í¿ÓÂ-Ù·È Ë ∫∂∏%.
160
140
120
100
80
60
40
20
070 80 90 100 110 120 130 140
y=-0,9782x+188,51r=-0,57 p<0,0001
FV
C%
KEH%
∂ÈÎfiÓ· 3. °Ú·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË FVC% fiÏˆÓ ÙˆÓ ·ÛıÂÓÒÓÌ ÙËÓ ∫∂∏%. ŸÛÔ ÂÏ·ÙÙÒÓÂÙ·È Ë FVC%, ÙfiÛÔ ·˘Í¿ÓÂÙ·ÈË ∫∂∏%.
160
140
120
100
80
60
40
20
070 80 90 100 110 120 130 140
y=-0,6376x+160,69r=0,39
PE
FR
%
∫∂∏%
∂ÈÎfiÓ· 4. °Ú·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË PEFR% fiÏˆÓ ÙˆÓ ·ÛıÂÓÒÓÌ ÙËÓ ∫∂∏%. ŸÛÔ ÂÏ·ÙÙÒÓÂÙ·È o PEFR%, ÙfiÛÔ ·˘Í¿ÓÂ-Ù·È Ë ∫∂∏%.
445∫·Ù·Ó¿ÏˆÛË ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜ ÛÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË
∫·ıÒ˜ Ô ‰Â›ÎÙ˘ Shwachman ÂÏ·ÙÙÒÓÂÙ·È, ‰Ë-Ï·‰‹ ÂÈ‚·Ú‡ÓÂÙ·È Ë ÎÏÈÓÈ΋ ηٿÛÙ·ÛË ÙÔ˘·ÛıÂÓ‹, Ë ∫∂∏% ·˘Í¿ÓÂÙ·È (·ÚÓËÙÈ΋ ÁÚ·ÌÌÈ-΋ Û˘Û¯¤ÙÈÛË). √ ‰Â›ÎÙ˘ Shwachman ¤¯ÂȯÚËÛÈÌÔÔÈËı› ˆ˜ ¤Ó‰ÂÈÍË Ù˘ ‚·Ú‡ÙËÙ·˜ Ù˘ÓfiÛÔ˘ Ù˘ ∫π (30). øÛÙfiÛÔ, Ô ‰Â›ÎÙ˘Shwachman Â›Ó·È ¤Ó·˜ ˘ÔÎÂÈÌÂÓÈÎfi˜ ‰Â›ÎÙË˜Î·È Ú¤ÂÈ ¿ÓÙ· Ó· ˘ÔÏÔÁ›˙ÂÙ·È ·fi ÙÔÓ ›‰ÈÔÂÍÂÙ·ÛÙ‹, οÙÈ Ô˘ fï˜ ‰ÂÓ Â›Ó·È ¿ÓÙ· ÂÊÈ-ÎÙfi ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË. ∫·ıÒ˜ ˘¿Ú¯ÂÈ ÛËÌ·-ÓÙÈ΋ ÁÚ·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ Ù˘ ∫∂∏%Î·È ÙÔ˘ ‰Â›ÎÙË Shwachman, fiˆ˜ Î·È Ù˘ Ó¢-ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, ÚÔÙ›ÓÂÙ·È Ë ∫∂∏% ˆ˜¤Ó·˜ ·ÓÙÈÎÂÈÌÂÓÈÎfi˜ ‰Â›ÎÙ˘ ÂÎÙ›ÌËÛ˘ ‚·Ú‡-ÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Ù˘ ∫π. ∂ÈϤÔÓ, Ë ∫∂∏%Ì¿˜ ‰›ÓÂÈ ÏËÚÔÊÔڛ˜ Û¯ÂÙÈο Ì ÙȘ ÂÓÂÚÁÂÈ-·Î¤˜ ·Ó¿ÁΘ ÙÔ˘ ·ÛıÂÓ‹ Î·È ÌÔÚ› Ó· ›ӷȯڋÛÈÌË ÛÙËÓ ¤ÁηÈÚË ÚÔÛ·ÚÌÔÁ‹ Ù˘ ‰›·ÈÙ¿˜ÙÔ˘. ™˘ÌÂÚ·›ÓÂÙ·È fiÙÈ Ë ¯Ú‹ÛË Ù˘ ∫∂∏% ›-Ó·È ·Ó·Áη›· ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË ˆ˜ ‰Â›ÎÙË˜ÙˆÓ ÂÓÂÚÁÂÈ·ÎÒÓ ·Ó·ÁÎÒÓ Î·È Ù˘ ‚·Ú‡ÙËÙ·˜Ù˘ ÓfiÛÔ˘ ·ÛıÂÓÒÓ Ì ∫π ηٿ ÙËÓ ÂÙ‹ÛÈ· ÂÎÙ›-ÌËÛË Ù˘ ηٿÛÙ·Û‹˜ ÙÔ˘.
µÈ‚ÏÈÔÁÚ·Ê›·
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¶·È‰È·ÙÚÈ΋ 2006;69:440-446
447∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏ CASE REPORT
¶·È‰È·ÙÚÈ΋ 2006;69:447-451
°ÂÓÂÙÈÎfi˜ Î·È ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜
Û ÔÈÎÔÁ¤ÓÂÈ· Ì ÎÏÈÓÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· Û˘Ó‰ÚfiÌÔ˘
Noonan
™. ∫›ÙÛÈÔ˘ - ∆˙¤ÏË, Õ. ¶··‰ÔÔ‡ÏÔ˘, ∞. •·˚‰¿Ú·, °. ¶··‰fiÔ˘ÏÔ˜, ∞.-ª. ª·ÁÈ¿ÎÔ˘, ∞. ∫ÔÏȷϤÍË,
∞. ª·‡ÚÔ˘, ∂. ∫·Ó·‚¿Î˘, ¶. ¡ÈÎÔÏ·˝‰Ô˘
¶ÂÚ›ÏË„Ë: ∆Ô Û‡Ó‰ÚÔÌÔ Noonan (NS) Â›Ó·È Û˘¯Ófi ÁÂÓÂÙÈÎfi ÓfiÛËÌ·. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ‚Ú·¯˘-ۈ̛·, ÎÚ·ÓÈÔÚÔÛˆÈΤ˜ ·ÓˆÌ·Ï›Â˜, Û˘ÁÁÂÓ‹ ηډÈÔ¿ıÂÈ·, ÎÚ˘„ÔÚ¯›·, ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ıÒڷη,ÙÔ˘ ÛÎÂÏÂÙÔ‡ Î·È ¿ÏÏˆÓ ÔÚÁ¿ÓˆÓ, ·ÈÌÔÚÚ·ÁÈ΋ ‰È¿ıÂÛË Î·È ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË. ∫ÏËÚÔÓÔÌÂ›Ù·È Ì·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ¯·Ú·ÎÙ‹Ú· Î·È ÂÌÊ·Ó›˙ÂÈ ÔÈΛÏË ÎÏÈÓÈ΋ ¤ÎÊÚ·ÛË. ªÂÙ·ÏÏ¿ÍÂȘ ÛÙԢ‡ı˘ÓÔ ÁÔÓ›‰ÈÔ (ƒ∆ƒ¡ 11) ·Ú·ÙËÚÔ‡ÓÙ·È ÛÙÔ 33-60% ÙˆÓ ·ÛıÂÓÒÓ Ì ʷÈÓfiÙ˘Ô NS. ¶ÂÚÈÁÚ¿-ÊÂÙ·È ÔÈÎÔÁ¤ÓÂÈ· Ì ۇӉÚÔÌÔ Noonan Ì ÎÏÈÓÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· Û 3 ÁÂÓȤ˜ ·fi ÙË ÌËÙ¤Ú·. ∏ ÂÚ›-ÙˆÛË ‰Â›ÎÙ˘, ÎÔÚ›ÙÛÈ 4 ÂÙÒÓ Î·È ÚÔ˚fiÓ ‰›‰˘Ì˘ Â͈ۈ̷ÙÈ΋˜ ·ËÛ˘, ¤¯ÂÈ ‚Ú·¯˘ÛˆÌ›· ηȉÈÔÚıˆı›۷ ÛÙ¤ÓˆÛË Ó¢ÌÔÓÈ΋˜. ∏ ÌËÙ¤Ú· Â›Ó·È Â›Û˘ ‚Ú·¯‡ÛˆÌË Î·È ¤¯ÂÈ ÈÛÙÔÚÈÎfi ·ÈÌÔÚÚ·-ÁÈ΋˜ ‰È¿ıÂÛ˘ Î·È Î·ı˘ÛÙ¤ÚËÛ˘ ÂÌÌËÓ·Ú¯‹˜. √ ÌËÙÚÈÎfi˜ ı›Ԙ ¤¯ÂÈ Î·ÓÔÓÈÎfi ·Ó¿ÛÙËÌ· Î·È “Û˘-ÛÙÔÏÈÎfi ʇÛËÌ·”. ∏ ‰›‰˘ÌË ·‰ÂÏÊ‹ Ù˘ ·ÛıÂÓÔ‡˜ η٤ÏËÍ Û ËÏÈΛ· 9 ÌËÓÒÓ ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ-΋ ‰ÈfiÚıˆÛË ÛÙ¤ÓˆÛ˘ Ó¢ÌÔÓÈ΋˜. ∏ ÁÈ·ÁÈ¿ ·fi ÙË ÌËÙ¤Ú· η٤ÏËÍ Û ËÏÈΛ· 30 ÂÙÒÓ ·fi ηÚ-‰È·Î‹ ÚÔÛ‚ÔÏ‹. ¶·Ú¿ ÙË ‰È·ÊÔÚÂÙÈ΋ ÚÔÛ‚ÔÏ‹ ÔÚÁ¿ÓˆÓ Î·È Û˘ÛÙËÌ¿ÙˆÓ fiÏ· Ù· ·ÓˆÙ¤Úˆ ̤ÏËÙ˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ›¯·Ó ÎÔÈÓ¿ ÌÔÚÊÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο (ÙÚÈÁˆÓÈÎfi ÚfiÛˆÔ, ˘ÂÚÙÂÏÔÚÈÛÌfi,·ÓÙÈÌÔÁÁÔÏÔÂȉ›˜ ‚ÏÂÊ·ÚÈΤ˜ Û¯ÈṲ̂˜, ÚÔ¤¯ÔÓÙ˜ ÔÊı·ÏÌÔ‡˜, ›ηÓıÔ, ·ÔÂÏ·Ù˘Ṳ̂ÓË Ì‡-ÙË, ¯·ÌËÏ¿ ÚÔ¤¯ÔÓÙ· ÒÙ·, ‚Ú·¯˘Ï·ÈÌ›·, ·˘¯ÂÓÈÎfi ÙÂÚ‡ÁÈÔ, ÛηÊÔÂȉ¤˜ ÛÙ¤ÚÓÔ Î·È ÛÎÔÏ›ˆÛË). √ÌÔÚÈ·Îfi˜ ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÂȂ‚·›ˆÛ ÙËÓ ÎÏÈÓÈ΋ ‰È¿ÁÓˆÛË Î·È ·ÔÎ¿Ï˘„ fiÙÈ ÚfiÎÂÈÙ·È ÁÈ·ÌËÙÚÈ΋ ÌÂÙ·‚›‚·ÛË Ù˘ ÂÙÂÚfi˙˘Á˘ ·ÚÂÚÌËÓ‡ÛÈÌ˘ ÌÂÙ¿ÏÏ·Í˘ A188G ÛÙÔ ÂÍfiÓÈÔ 3 ÙÔ˘ ÁÔÓÈ-‰›Ô˘ PTPN 11. ∏ ›‰È· ÌÂÙ¿ÏÏ·ÍË ·Ó‚ڤıËÎÂ Î·È ÛÙÔÓ ıÂ›Ô ·fi ÙË ÌËÙ¤Ú·. ∆· ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚÈ·-ο Î·È Ù· Â˘Ú‹Ì·Ù· Ù˘ ÌÔÚȷ΋˜ ·Ó¿Ï˘Û˘ Û˘Û¯ÂÙ›˙ÔÓÙ·È Ì ٷ ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ Î·È ‰›‰Â-Ù·È ¤ÌÊ·ÛË ÛÙËÓ ÂÓ‰ÔÔÈÎÔÁÂÓÂȷ΋ ÔÈÎÈÏÔÌÔÚÊ›·, Ë ÔÔ›· ηıÈÛÙ¿ ·Ó·Áη›· ÙË ‰ÈÂÓ¤ÚÁÂÈ· ÌÔÚÈ·-ÎÔ‡ ÁÂÓÂÙÈÎÔ‡ ÂϤÁ¯Ô˘ ÛÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜ ÁÈ· ÙËÓ ÏËÚ¤ÛÙÂÚË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË.
§¤ÍÂȘ ÎÏÂȉȿ: ™‡Ó‰ÚÔÌÔ Noonan, ÁÔÓ›‰ÈÔ PTPN 11, ÎÏÈÓÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ·.
Molecular, clinical and laboratory investigation in a Noonan syndrome
family presenting with clinical variability
S. Kitsiou - Tzeli, A. Papadopoulou, A. Xaidara, G. Papadopoulos, A.-M. Magiakou, A. Kolialexi,
A. Mavrou, E. Kanavakis, P. Nikolaidou
Abstract: ¡ÔÔnan syndrome (NS) is a relatively common autosomal dominant genetic disorder withgreat clinical variability. It is characterized by craniofacial anomalies, short stature, congenital heartdefects, cryptorchidism, skeletal and other organ malformations, bleeding diathesis and mentalretardation. Mutations of the PTNP 11 gene have been identified in 33-60% of cases. A family is reportedwith NS transmitted through 3 maternal generations. The five affected members presented with differentorgan involvement. The index case, a 4 year-old girl with growth retardation, is the product of a twin IVFpregnancy. Her phenotypic characteristics, common to all the affected members, include: coarsetriangular-shaped face, hypertelorism, down-slanting prominent eyes with epicanthus, low-setposteriorly angulated ears with thickened helices, bulbous nasal tip, big mouth with arched upper lip,short webbed neck, pectus excavatum and scoliosis. She was operated on for pulmonary stenosis, as washer twin sister who died following surgery. Their mother had a delayed menarche and a history ofbleeding diathesis. A maternal uncle has a heart murmur. The grandmother died at the age of 30 yearsfrom a heart attack. The molecular investigation revealed a maternally transmitted heterozygous missense mutation A 118 G in exon 3, which was also observed in the maternal uncle. The intrafamiliar variability
∂ÚÁ·ÛÙ‹ÚÈÔ π·ÙÚÈ΋˜°ÂÓÂÙÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘∞ıËÓÒÓ, ∞’ ¶·È‰È·ÙÚÈ΋∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘∞ıËÓÒÓ, °’ ¶·È‰È·ÙÚÈ΋∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘∞ıËÓÒÓ
AÏÏËÏÔÁÚ·Ê›·:
™ÔÊ›· ∫›ÙÛÈÔ˘-∆˙¤ÏË∂ÚÁ·ÛÙ‹ÚÈÔ π·ÙÚÈ΋˜°ÂÓÂÙÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ“∏ ∞Á›· ™ÔÊ›·”∆∫ 115 27, ∞ı‹Ó·E-mail: [email protected]
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 24-11-05∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 21-07-06
Department of MedicalGenetics, First Department ofPediatrics, Third Departmentof Pediatrics, AthensUniversity, Athens Greece
Correspondence:
Sofia Kitsiou-Tzeli, Department of MedicalGenetics, “Aghia Sophia”Children’s Hospital 115 27 Athens, GreeceE-mail: [email protected]
Date of submission: 24-11-05 Date of approval: 21-07-06
448 ™. ∫›ÙÛÈÔ˘-∆˙¤ÏË Î·È Û˘Ó.
¶·È‰È·ÙÚÈ΋ 2006;69:447-451
of NS makes it essential for first degree relatives, even with an atypical phenotype, to be screened forPTNP 11 gene mutations in order to receive appropriate genetic counseling.
Key words: Noonan syndrome, PTNP 11 gene, clinical variability.
™˘ÓÙÔÌÔÁڷʛ˜
NS ¡ÔÔnan Syndrome
PTPN 11 Protein-Tyrosine Phosphatase,
Nonreceptor-type, 11-OMIM#163950
∂£ ∂ηÙÔÛÙÈ·›· ı¤ÛË
DGGE Denaturing Gradient Gel Electrophoresis
∂ÈÛ·ÁˆÁ‹
∏ ÌÔÚȷ΋ ‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘
Noonan (1) Â›Ó·È È‰È·›ÙÂÚ· ÛËÌ·ÓÙÈ΋ ·ÊÔ‡ ¯·-
Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÔÈΛÏË ÎÏÈÓÈ΋ ÂÎÊÚ·ÛÙÈÎfi-
ÙËÙ· (2), ÔÈ ¿Û¯ÔÓÙ˜ ‰‡ÛÎÔÏ· ·Ó·ÁÓˆÚ›˙ÔÓÙ·È
ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, ÂÓÒ Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘
ÚÔÛÒÔ˘ Â›Ó·È ÂÌÊ·Ó¤ÛÙÂÚ· ÛÙËÓ ·È‰È΋ ËÏÈ-
Λ· Î·È Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ ÌÔÚÔ‡Ó Ó·
Á›ÓÔ˘Ó ËÈfiÙÂÚ· (“ÌÂÙ·‚·ÏÏfiÌÂÓÔ˜ Ê·ÈÓfiÙ˘-
Ô˜”). ∂› ÌË Ù˘ÈÎÔ‡ Ê·ÈÓÔÙ‡Ô˘ ÙÔ ÏÂÙÔÌÂ-
Ú¤˜ ÈÛÙÔÚÈÎfi ηÙ¢ı‡ÓÂÈ ÙË ‰È¿ÁÓˆÛË. π‰È·›ÙÂÚË
¤ÌÊ·ÛË ‰›ÓÂÙ·È ÛÙËÓ ·Ó·˙‹ÙËÛË Û˘ÁÁÂÓÔ‡˜
ηډÈÔ¿ıÂÈ·˜ ‹/Î·È ¯·ÌËÏÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜ ÛÂ
¿ÏÏ· ̤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ÙÔ˘ ÚÔۂ‚ÏË̤-
ÓÔ˘ ·È‰ÈÔ‡. ¶·ÚÔ˘ÛÈ¿˙ÂÙ·È Ë ‰È·ÁÓˆÛÙÈ΋
ÚÔۤϷÛË ·ÛıÂÓÔ‡˜ Ì ʷÈÓfiÙ˘Ô Û˘Ó‰Úfi-
ÌÔ˘ Noonan (¡S) Ô˘ ÂÈÛ‹¯ıË ÛÙÔ ÓÔÛÔÎÔÌ›Ô
ÁÈ· ÙË ‰ÈÂÚ‡ÓËÛË Î·ı˘ÛÙ¤ÚËÛ˘ ۈ̷ÙÈ΋˜
·Ó¿Ù˘Í˘. √ ÌÔÚÈ·Îfi˜ ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ù˘
ÔÈÎÔÁ¤ÓÂÈ·˜ ·ÔÎ¿Ï˘„ ÙË ÌÂÙ¿ÏÏ·ÍË ∞188G
ÛÙÔ ÂÍfiÓÈÔ 3 ÙÔ˘ ÁÔÓȉ›Ô˘ PTPN 11 ÛÙËÓ ·ÛıÂ-
Ó‹, ÙË ÌËÙ¤Ú· Ù˘ Î·È ÙÔÓ ÌËÙÚÈÎfi ı›Ô.
¶ÂÚÈÁÚ·Ê‹ ÂÚÈÙÒÛˆÓ
∏ ·ÛıÂÓ‹˜ - ‰Â›ÎÙ˘ (πππ:1) Â›Ó·È ÙÔ ÌÔÓ·‰È-
Îfi ·È‰› Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ (∂ÈÎfiÓ· 1). °ÂÓÓ‹ıËÎÂ
Ì ηÈÛ·ÚÈ΋ ÙÔÌ‹ ÏfiÁˆ ‰›‰˘Ì˘ ·ËÛ˘ Ë
ÔÔ›· ‰È‹ÚÎÂÛ 34 ‚‰ÔÌ¿‰Â˜. ∫·Ù¿ ÙË Á¤ÓÓËÛË
ÙÔ ‚¿ÚÔ˜ Ù˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó 2030 g (25Ë ∂£), ÙÔ
Ì‹ÎÔ˜ ÛÒÌ·ÙÔ˜ 44 cm (50Ë ∂£) Î·È Ë ÂÚ›ÌÂ-
ÙÚÔ˜ ÎÂÊ·Ï‹˜ 31,5 cm (50‹ ∂£). §fiÁˆ Ù˘ ÚÔ-
ˆÚfiÙËÙ·˜ Î·È Èı·Ó‹˜ ÂÌ‚Ú˘ÔÂÌ‚Ú˘˚΋˜ ÌÂÙ¿Á-
ÁÈÛ˘ ÓÔÛËχıËΠ۠ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ ¡Ô-
ÛËÏ›·˜ ÁÈ· 25 Ë̤Ú˜. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔ-
ÛËÏ›·˜ ‰È·ÈÛÙÒıËÎÂ Û˘ÛÙÔÏÈÎfi ʇÛËÌ· 2/6
Ì ¯·Ú·ÎÙ‹Ú˜ ÂÚÈÊÂÚÈ΋˜ ÛÙ¤ÓˆÛ˘ Ó¢ÌÔ-
ÓÈ΋˜. ™Â ËÏÈΛ· 13 ÌËÓÒÓ ˘Â‚Ï‹ıË Û ηډÈÔ-ÏÔÁÈÎfi ¤ÏÂÁ¯Ô ηıÒ˜ Î·È Î·Ú‰È·Îfi ηıÂÙËÚÈ·-ÛÌfi Î·È ‚·Ï‚ȉÔÏ·ÛÙÈ΋ Ì ̷ÏfiÓÈ, ÏfiÁˆÛÙ¤ÓˆÛ˘ Ó¢ÌÔÓÈ΋˜ ‚·Ï‚›‰·˜ ÔÊÂÈÏfiÌÂÓ˘Û ‰˘ÛÏ·Û›· ·˘Ù‹˜. ∫·Ù¿ ÙÔÓ ÌÂÙÂÁ¯ÂÈÚËÙÈÎfi¤ÏÂÁ¯Ô Ì Triplex ηډȿ˜, ‰È·ÈÛÙÒıËΠ‰˘-ÛÏ·ÛÙÈ΋ Ó¢ÌÔÓÈ΋ ‚·Ï‚›‰· Ì Èı·ÓÒ˜˘ÂÚ‚·Ï‚ȉÈÎfi ÛÙÔÈ¯Â›Ô Ì¤ÙÚÈ·˜ ÛÙ¤ÓˆÛ˘.
™ÙËÓ ËÏÈΛ· ÙˆÓ 4 ÂÙÒÓ ·Ú·¤ÌÊıËΠÛÙÔπ·ÙÚÂ›Ô ∫ÏÈÓÈ΋˜ °ÂÓÂÙÈ΋˜ ÁÈ· ¤ÏÂÁ¯Ô ·Ó·Ú-ÎÔ‡˜ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘, ÂÓÒ Ë „˘¯ÔÎÈÓËÙÈ-΋ Ù˘ ÂͤÏÈÍË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∫·Ù¿ ÙËÓ ÎÏÈ-ÓÈ΋ ÁÂÓÂÙÈ΋ ÂÎÙ›ÌËÛË ÚԤ΢„·Ó ÔÈ ·ÎfiÏÔ˘-ı˜ ۈ̷ÙÔÌÂÙÚÈΤ˜ ·Ú¿ÌÂÙÚÔÈ: ª™: 95 cm(<3Ë ∂£), µ™: 13 kg (3Ë-5Ë ∂£), Î·È ¶∫: 50 cm(50‹ ∂£). ∏ ·ÛıÂÓ‹˜ ÂÌÊ¿ÓÈ˙ ÙÚÈÁˆÓÈÎfi Úfi-ÛˆÔ, ˘ÂÚÙÂÏÔÚÈÛÌfi, ›ηÓıÔ, Ô›‰ËÌ· ‚ÏÂ-Ê¿ÚˆÓ, ·ÓÙÈÌÔÁÁÔÏÔÂȉ›˜ ‚ÏÂÊ·ÚÈΤ˜ Û¯ÈṲ̂˜,ÂÏ·ÊÚ¿ ‚ÏÂÊ·ÚfiÙˆÛË Ì ÚÔ¤¯ÔÓÙ˜ ÔÊı·Ï-ÌÔ‡˜, ̇ÙË ‚ÔÏ‚Ò‰Ë, ÚÔ¤¯ÔÓÙ· ÒÙ· Ì ¯·ÌË-Ï‹ ÚfiÛÊ˘ÛË, ÏÔÍ‹ ÊÔÚ¿ Î·È ·¯È¿ ¤ÏÈη, ÌÂÁ¿-ÏÔ ÛÙfiÌ· Ì ¯Â›ÏÔ˜ ÙÔ͈Ùfi, ˘„ËÏ‹ ˘ÂÚÒ·, ·˘-¯ÂÓÈÎfi ÙÂÚ‡ÁÈÔ Î·È ‚Ú·¯‡ Ï·ÈÌfi. ™ÙÔÓ ıÒڷη·Ú·ÙËÚ‹ıËΠÛηÊÔÂȉ¤˜ ÛÙ¤ÚÓÔ, ˘ÔÏ·ÛÙÈ-Τ˜ ıËϤ˜ Ì ÌÂÁ¿ÏË ÌÂٷ͇ ÙÔ˘˜ ·fiÛÙ·ÛË Î·ÈÛÎÔÏ›ˆÛË.
∏ ÌËÙ¤Ú· Ù˘ ·ÛıÂÓÔ‡˜ (ππ:2) ËÏÈΛ·˜ 39ÂÙÒÓ, ¤¯ÂÈ ‡„Ô˜ 142 cm Î·È ÂÌÊ·Ó›˙ÂÈ Ù· ÂÍ‹˜Ê·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο: ÙÚÈÁˆÓÈÎfi Úfi-ÛˆÔ, ˘ÂÚÙÂÏÔÚÈÛÌfi, ›ηÓıÔ, ÛËÌ·ÓÙÈÎfi
I
II
III
1
1 2 3
43
21
2
∂ÈÎfiÓ· 1. ∆Ô ÁÂÓ·ÏÔÁÈÎfi ‰¤Ó‰ÚÔ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜.
Ô›‰ËÌ· ‚ÏÂÊ¿ÚˆÓ, ‚ÏÂÊ·ÚfiÙˆÛË, ·ÓÙÈÌÔÁ-ÁÔÏÔÂȉ›˜ ‚ÏÂÊ·ÚÈΤ˜ Û¯ÈṲ̂˜, ÛËÌ·ÓÙÈ΋ÚÔ¤ÙÂÈ· ÔÊı·ÏÌÈÎÒÓ ‚ÔÏ‚ÒÓ, ˘ÂÚÌÂÙÚˆ-›·, ‚ÔÏ‚Ò‰Ë Ì‡ÙË, ¯·ÌËÏ‹ ÚfiÛÊ˘ÛË ÒÙˆÓÌ ÏÔÍ‹ ÊÔÚ¿, ·ÓÒÌ·ÏË ÂϛΈÛË Î·È ·¯È¿¤ÏÈη, ÌÂÁ¿ÏÔ ÛÙfiÌ· Ì ÙÔ͈ٿ ¯Â›ÏË Î·È˘„ËÏ‹ ˘ÂÚÒ·, ‚Ú·¯‡ Ï·ÈÌfi Ì ·˘¯ÂÓÈÎfi ÙÂ-Ú‡ÁÈÔ Î·È Î˘ÊÔÛÎÔÏ›ˆÛË. √ ηډÈÔÏÔÁÈÎfi˜¤ÏÂÁ¯Ô˜ ÛÙÔÓ ÔÔ›Ô ˘Â‚Ï‹ıË Ë ÌËÙ¤Ú· ‰ÂÓ¤‰ÂÈÍ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ™ÙÔ ·ÙÔÌÈÎfiÙ˘ ·Ó·ÌÓËÛÙÈÎfi ·Ó·Ê¤ÚÔÓÙ·È ÓÔÛËÏ›˜ Ïfi-Áˆ ÂÎÛÂÛËÌ·Ṳ̂Ó˘ ·ÈÌÔÚÚ·Á›·˜ ηٿ ÙËӉȿÚÎÂÈ· ·ÏÒÓ ÁÓ·ıÔ¯ÂÈÚÔ˘ÚÁÈÎÒÓ ÂÂÌ‚¿-ÛÂˆÓ ÏfiÁˆ ·ÓÒÌ·Ï˘ ¤ÎÊ˘Û˘ Ô‰fiÓÙˆÓ, ÂÌ-ÌËÓ·Ú¯‹ Û ËÏÈΛ· 16 ¯ÚfiÓˆÓ Î·È ˘ÔÁÔÓÈÌfi-ÙËÙ·. ø˜ ÂÎ ÙÔ‡ÙÔ˘ Î·Ù¤Ê˘Á ÛÙËÓ Â͈ۈ̷-ÙÈ΋ ÁÔÓÈÌÔÔ›ËÛË, ÚÔ˚fiÓ Ù˘ ÔÔ›·˜ ‹Ù·Ó ˉ›‰˘ÌË Î‡ËÛË.
∏ ‰›‰˘ÌË ·‰ÂÏÊ‹ (πππ:2) Ù˘ ·ÛıÂÓÔ‡˜-‰Â›-ÎÙË, Ë ÔÔ›· η٤ÏËÍ Û ËÏÈΛ· 9 ÌËÓÒÓ ÌÂÙ¿·fi ‰ÈÔÚıˆÙÈ΋ ¤̂·ÛË ÛÙ¤ÓˆÛ˘ Ù˘ Ó¢-ÌÔÓÈ΋˜, ›¯Â ·ÚfiÌÔÈÔ Ê·ÈÓfiÙ˘Ô.
∏ ÁÈ·ÁÈ¿ ·fi ÙË ÌËÙ¤Ú· (π:4), Ë ÔÔ›· ›¯ÂÙ· ›‰È· ÌÔÚÊÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο Ì ÙËÓÎfiÚË (ππ:2) Î·È ÙȘ ‰›‰˘Ì˜ ÂÁÁÔÓ¤˜ (πππ:1 ηÈπππ:2), η٤ÏËÍ Û ËÏÈΛ· 30 ÂÙÒÓ ·fi “·Ó·ÎÔ-‹ ηډȿ˜”.
∆· ›‰È· ›Û˘ ÌÔÚÊÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈοÂÌÊ·Ó›˙ÂÈ Î·È Ô ı›Ԙ ·fi ÙË ÌËÙ¤Ú· (ππ:3), Ù˘·ÛıÂÓÔ‡˜-‰Â›ÎÙË, Ô ÔÔ›Ô˜ ¤¯ÂÈ ‡„Ô˜ 170 cmÎ·È ·ÚÔ˘ÛÈ¿˙ÂÈ “ʇÛËÌ· ÚÔηډ›Ô˘”, ¯ˆÚ›˜Ó· ¤¯ÂÈ ˘Ô‚ÏËı› Û ÂȉÈÎfi ¤ÏÂÁ¯Ô.
∆· Ê·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο Ù˘ ÔÈÎÔÁ¤-ÓÂÈ·˜ Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È Ê·›ÓÔÓÙ·È ÛÙÔÓ ¶›Ó·Î·1 Î·È ÛÙȘ ∂ÈÎfiÓ˜ 2 Î·È 3.
∫ÏÈÓÈÎÔÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜
∏ ·ÛıÂÓ‹˜ ˘Â‚Ï‹ıË Û ηډÈÔÏÔÁÈÎfi ¤ÏÂÁ-¯Ô Î·È ‰È·ÈÛÙÒıËΠ‚·Ï‚ȉÔÏ·ÛÙÈ΋ Ù˘Ó¢ÌÔÓÈ΋˜ Ì ËÈfiÙ·ÙË ÛÙ¤ÓˆÛË. ∆Ô ˘ÂÚÔ-¯ËÁÚ¿ÊËÌ· ¿Óˆ - οو ÎÔÈÏ›·˜ Î·È ÁÂÓÓËÙÈÎÒÓÔÚÁ¿ÓˆÓ, Ô ÔÊı·ÏÌÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜, Ô ¤ÏÂÁ¯Ô˜·ÈÌÔÚÚ·ÁÈ΋˜ ‰È¿ıÂÛ˘ (¯ÚfiÓÔ˜ ÚÔıÚÔÌ‚›Ó˘,¯ÚfiÓÔ˜ ıÚÔÌ‚›Ó˘, ∞ƒ∆∆, ÈÓˆ‰ÔÁfiÓÔ, ·Ú¿ÁÔ-ÓÙ˜ ‹Í˘), Î·È Ô ÂÓ‰ÔÎÚÈÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜(GH, IGF1, FSH, LH, PRL, T3, T4, TSH, ÔÈÛÙÚ·-‰ÈfiÏË, ÙÂÛÙÔÛÙÂÚfiÓË, ÚÔÁÂÛÙÂÚfiÓË, DHEA-S,17-OH ÚÔÁÂÛÙÂÚfiÓË, ‰ÔÎÈÌ·Û›· ‰È¤ÁÂÚÛ˘ ÌÂÁÏ˘Î·ÁfiÓË) ›¯·Ó Ê˘ÛÈÔÏÔÁÈο Â˘Ú‹Ì·Ù·.
°ÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜
√ ηڢfiÙ˘Ô˜ Ù˘ ·ÛıÂÓÔ‡˜ ¤‰ÂÈÍÂ Ê˘ÛÈÔ-ÏÔÁÈ΋ ¯ÚˆÌÔÛˆÌÈ΋ Û‡ÛÙ·ÛË ı‹ÏÂÔ˜ (46,ÃÃ).
∏ ·Ó¿Ï˘ÛË ÙÔ˘ ÁÔÓȉ›Ô˘ PTPN 11 ¤ÁÈÓ ÛÙËÓ·ÛıÂÓ‹ (πππ:1), ÛÙÔ˘˜ ÁÔÓ›˜ (ππ:1 Î·È II:2), ηÈÛÙÔÓ ÌËÙÚÈÎfi ıÂ›Ô (II:3), ÌÂÙ¿ ·fi ·ÔÌfiÓˆÛËDNA ·fi ÂÚÈÊÂÚÈÎfi ·›Ì·. ∂Ê·ÚÌfiÛÙËΠ˷ÓÙ›‰Ú·ÛË PCR Ì ηٿÏÏËÏÔ˘˜ ÂÎÎÈÓËÙ¤˜ Ô˘·ÓÙÈÛÙÔÈ¯Ô‡Ó ÛÙ· 15 ÂÍfiÓÈ· ÙÔ˘ ÁÔÓȉ›Ô˘ (3). ∏·ÎÔÏÔ˘ı›· ÙˆÓ ÓÔ˘ÎÏÂÔÙȉ›ˆÓ ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛËÈı·ÓÒÓ ÌÂÙ·ÏÏ¿ÍÂˆÓ ·Ó·Ï‡ıËΠ۠·˘ÙfiÌ·ÙÔ·Ó·Ï˘Ù‹ (Visgen Sequencer). ∞Ó·˙ËÙ‹ıËÎ·Ó ÌÂ-Ù·ÏÏ¿ÍÂȘ ‹ ÔÏ˘ÌÔÚÊÈÛÌÔ› ÛÙ· ÂÍfiÓÈ· Î·È ÛÙȘÂÚÈÔ¯¤˜ Û‡Ó‰ÂÛ‹˜ ÙÔ˘˜ Ì ٷ ·ÓÙ›ÛÙÔȯ· ÂÛfiÓÈ·(̤ıÔ‰Ô˜ Denaturing Gradient Gel Electro-phoresis, DGGE). ™Â ‰Â›ÁÌ·Ù· ÛÙ· ÔÔ›· ·Ú·-ÙËÚ‹ıËΠÚfiÙ˘Ô ËÏÂÎÙÚÔÊfiÚËÛ˘ ‰È·ÊÔÚÂ-ÙÈÎfi ·fi ·˘Ùfi ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ Ì·ÚÙ‡ÚˆÓ(controls) -ÔfiÙ ˘Ô‰ËÏÒÓÂÙ·È Èı·Ó‹ ·ÚÔ˘-Û›· ÌÂÙ¿ÏÏ·Í˘ ‹ ÔÏ˘ÌÔÚÊÈÛÌÔ‡- ·˘ÙÔ› ÚÔÛ-‰ÈÔÚ›ÛÙËÎ·Ó ÂÚ·ÈÙ¤Úˆ Ì ·ÏÏËÏÔ˘¯ÔÔ›ËÛË
449√ÈÎÔÁ¤ÓÂÈ· Ì ÂÙÂÚÔÁ¤ÓÂÈ· Û˘Ó‰ÚfiÌÔ˘ Noonan
¶·È‰È·ÙÚÈ΋ 2006;69:447-451
¶›Ó·Î·˜ 1. º·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ Ì ÎÏËÚÔÓÔÌÈÎfi NS
ÕÙÔÌÔ- ¢›‰˘ÌË ªËÙ¤Ú· ªËÙÚÈÎfi˜ ªËÙÚÈ΋ ‰Â›ÎÙ˘ ·‰ÂÏÊ‹ ı›Ԙ ÁÈ·ÁÈ¿
ª‹ÎÔ˜ ÛÒÌ·ÙÔ˜ 95 ÂÎ. 142 ÂÎ. 170 ÂÎ. (<3Ë ∂£) (<3Ë ∂£) (~10Ë ∂£)
∫Ú·ÓÈÔÚÔÛˆÈο Â˘Ú‹Ì·Ù·ÙÚÈÁˆÓÈÎfi ÚfiÛˆÔ + + + + +˘ÂÚÙÂÏÔÚÈÛÌfi˜ / ›ηÓıÔ˜ / + + + + +·ÓÙÈÌÔÁÁÔÏÔÂȉ›˜ ‚ÏÂÊ. Û¯ÈṲ̂˜ÚÔ¤¯ÔÓÙ˜ ÔÊı·ÏÌÔ› / ‚ÏÂÊ·ÚfiÙˆÛË + + + + +¯·ÌËÏ‹ ÚfiÛÊ˘ÛË / + + + + +·ÓÒÌ·ÏË ÂϛΈÛË ÒÙˆÓ / ÙÔ͈Ùfi ¿Óˆ ¯Â›ÏÔ˜ / ıÔψً ˘ÂÚÒ·
™ÎÂÏÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜·˘¯ÂÓÈÎfi ÙÂÚ‡ÁÈÔ / ‚Ú·¯‡˜ ·˘¯¤Ó·˜ + + + + +
™˘ÁÁÂÓ‹˜ ηډÈÔ¿ıÂÈ· ™Ù¤ÓˆÛË ™Ù¤ÓˆÛË - “º‡ÛËÌ·” ∫·Ú‰È·Î‹ Ó¢ÌÔÓÈ΋˜ Ó¢ÌÔÓÈ΋˜ ÚÔÛ‚ÔÏ‹
∞ÈÌÔÚÚ·ÁÈ΋ ‰È¿ıÂÛË +¡ÔËÙÈ΋ ˘ÛÙ¤ÚËÛË - - - - -
450 ™. ∫›ÙÛÈÔ˘-∆˙¤ÏË Î·È Û˘Ó.
ÙÔ˘ DNA Ù˘ ÂÚÈÔ¯‹˜ (sequencing). ™ÙÔÓ ·-Ù¤Ú· Ù˘ ·ÛıÂÓÔ‡˜ (ππ:1) ‰ÂÓ ·Ó‚ڤıËΠÌÂÙ¿Ï-Ï·ÍË, ÂÓÒ ÛÙ· ˘fiÏÔÈ· 3 ̤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜Ô˘ ÂϤÁ¯ıËÎ·Ó (ππ:2, ππ:3, πππ:1) ‰È·ÈÛÙÒıËΠËÂÙÂÚfi˙˘ÁË ·ÚÂÚÌËÓ‡ÛÈÌË (missense) ÌÂÙ¿Ï-Ï·ÍË A188G ÛÙÔ ÂÍfiÓÈÔ 3. ∂ÈÚÔÛı¤Ùˆ˜, ÛÙËÓ·ÛıÂÓ‹-‰Â›ÎÙË ‚Ú¤ıËΠÔÏ˘ÌÔÚÊÈÛÌfi˜21C→T ÛÙÔ ¿ÎÚÔ 3 ÙÔ˘ ÂÛoÓ›Ô˘ 7, Ô ÔÔ›Ô˜
·Ô˘ÛÈ¿˙ÂÈ ·fi ÙË ÌËÙ¤Ú· Î·È ÙÔÓ ·Ù¤Ú· (denovo) (∂ÈÎfiÓ· 4).
™˘˙‹ÙËÛË
∆Ô NS ÂÚÈÁÚ¿ÊËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÙÔ1963 ·fi ÙËÓ ·È‰ÔηډÈÔÏfiÁÔ JacquelineNoonan ÛÙÔ ¿ÚıÚÔ “Hypertelorism with Turnerphenotype. A new syndrome with associatedcongenital heart disease”. (4) ™‹ÌÂÚ· ÙÔ NS ›-Ó·È ÁÓˆÛÙfi ˆ˜ ÁÔÓȉȷÎfi ÓfiÛËÌ· Ì ÂÈÎÚ·ÙË-ÙÈÎfi ¯·Ú·ÎÙ‹Ú·, ÈÛfiÙÈÌË ÚÔÛ‚ÔÏ‹ ÙˆÓ ‰‡ÔÊ‡ÏˆÓ Î·È Ê˘ÛÈÔÏÔÁÈÎfi ηڢfiÙ˘Ô (5,6). ∏Û˘¯ÓfiÙËÙ· ÙÔ˘ NS Î˘Ì·›ÓÂÙ·È ·fi 1:100 ¤ˆ˜1:1000, ÂÓÒ ·ÔÙÂÏ› ÙË ‰Â‡ÙÂÚË Î·Ù¿ ÛÂÈÚ¿ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹ Ô˘ Û˘Ó‰˘¿˙ÂÙ·È ÌÂ Û˘Á-ÁÂÓ‹ ηډÈÔ¿ıÂÈ· ÌÂÙ¿ ÙÔ Û‡Ó‰ÚÔÌÔ Down(7). ∏ Û˘ÁÁÂÓ‹˜ ηډÈÔ¿ıÂÈ· ÂÌÊ·Ó›˙ÂÙ·È ÛÙÔÛ‡Ó‰ÚÔÌÔ ÌÂ Û˘¯ÓfiÙËÙ· 86%, ÌÂ Û˘¯ÓfiÙÂÚËÙËÓ ÛÙ¤ÓˆÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ‚·Ï‚›‰·˜, ÙËÓ˘ÂÚÙÚÔÊÈ΋ Ì˘ÔηډÈÔ¿ıÂÈ· Î·È ÙÔ ÌÂÛÔ-ÎÔÏÈÎfi ¤ÏÏÂÈÌÌ· (7,8). ∏ ÔÈΛÏË ÂÎÊÚ·ÛÙÈ-ÎfiÙËÙ· Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙÔ Û‡Ó‰ÚÔÌÔ, Û˘¯Ó¿‰ÂÓ ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó·ÁÓÒÚÈÛË ÂÓËÏ›ÎˆÓ Ì‹ȷ ÎÏÈÓÈ΋ ÂÈÎfiÓ·, ‰ÈfiÙÈ Â›Ó·È ÁÓˆÛÙfi fiÙÈ ÔÈÎÚ·ÓÈÔÚÔÛˆÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÌÂÙ·‚¿ÏÏÔÓÙ·ÈÌ ÙËÓ ËÏÈΛ· Î·È Â›Ó·È ÏÈÁfiÙÂÚÔ ÂÌÊ·Ó›˜ÛÙÔ˘˜ ÂÓ‹ÏÈΘ (2). ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ‰˘Û¯ÂÚ·›-
ÓÂÈ ÙË ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï¢ÙÈ΋, ‰Â‰Ô̤ÓÔ˘ fiÙÈ,
fiÙ·Ó ÛÙÔÓ ¤Ó· ÂÎ ÙˆÓ ‰‡Ô ÁÔÓ¤ˆÓ Ë ÎÏÈÓÈ΋ ÂÈ-
ÎfiÓ· Â›Ó·È Ù˘È΋, Ô ÁÂÓÂÙÈÎfi˜ ΛӉ˘ÓÔ˜ ·-
Ó¿Ï˄˘ (50%) ÌÔÚ› Ó· ‰Ôı› ÛÙËÓ ÔÈÎÔÁ¤-
ÓÂÈ·, ÂÓÒ fiÙ·Ó ÔÈ ÁÔÓ›˜ ÂÌÊ·Ó›˙Ô˘Ó Ï›Á· ‹/ηÈ
¿Ù˘· Â˘Ú‹Ì·Ù· ÙÔ˘ NS, Ô Î›Ó‰˘ÓÔ˜ ·ӿÏË-
„˘ Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÚÔ‚ÏÂÊı›. ™ÙȘ ÂÚÈ-
ÙÒÛÂȘ ·˘Ù¤˜ Â›Ó·È ··Ú·›ÙËÙÔ˜ Ô ÌÔÚÈ·Îfi˜
ÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· ÙËÓ ÂȂ‚·›ˆÛË Ù˘
ÎÏÈÓÈ΋˜ ‰È¿ÁÓˆÛ˘ (3).
∆Ô ˘Â‡ı˘ÓÔ ÁÈ· ÙÔ NS ÁÔÓ›‰ÈÔ PTPN 11
(Protein-Tyrosine Phosphatase, Nonreceptor-
type 11) ¯·ÚÙÔÁÚ·Ê‹ıËΠÙÔ 1994 ·fi ÙÔ˘˜
Jamieson et al (9) ÛÙÔ Ì·ÎÚfi ÛΤÏÔ˜ ÙÔ˘ ¯Úˆ-
ÌÔÛÒÌ·ÙÔ˜ 12(q24.1). ∆Ô ÁÔÓ›‰ÈÔ ¤¯ÂÈ Ì‹ÎÔ˜ 4
cM Î·È ·ÔÙÂÏÂ›Ù·È ·fi 15 ÂÍfiÓÈ· Ô˘ Έ‰ÈÎÔ-
ÔÈÔ‡Ó ÙËÓ ÚˆÙ½ÓË SHP-2. ∏ ÚˆÙ½ÓË ·˘Ù‹
Â›Ó·È ÛËÌ·ÓÙÈ΋ ÁÈ· ÙÔÓ ÛˆÛÙfi Û¯ËÌ·ÙÈÛÌfi ÙˆÓ
ÈÛÙÒÓ Î·Ù¿ ÙËÓ ·Ó¿Ù˘ÍË ·ÏÏ¿ Î·È ÁÈ· ÙË ‰È·›-
ÚÂÛË, ÂÍÂȉ›Î¢ÛË Î·È Î›ÓËÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ
¶·È‰È·ÙÚÈ΋ 2006;69:447-451
∂ÈÎfiÓ· 2. √È ‰›‰˘Ì˜ ·‰ÂÏʤ˜ (III:1 Î·È III:2) Û ËÏÈ-Λ· 6 ÌËÓÒÓ.
∂ÈÎfiÓ· 3. ∏ ·ÛıÂÓ‹˜-‰Â›ÎÙ˘ (III:1) Û ËÏÈΛ· 4 ÂÙÒÓÌ ÙË ÌËÙ¤Ú· Ù˘ (ππ:2).
∂ÈÎfiÓ· 4. °ÔÓÔÙ˘È΋ ·Ó¿Ï˘ÛË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜:a=·ÛıÂÓ‹˜ - ‰Â›ÎÙ˘ (πππ:1), b=ÌËÙ¤Ú· (ππ:2), c=·Ù¤-Ú·˜ (ππ:1).
∂ÍfiÌÈÔ 8
ba c
∂ÍfiÌÈÔ 3
ba c
{ {
451√ÈÎÔÁ¤ÓÂÈ· Ì ÂÙÂÚÔÁ¤ÓÂÈ· Û˘Ó‰ÚfiÌÔ˘ Noonan
Î·È ÂÎÊÚ¿˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙËÓ Î·Ú‰È¿, ÙÔÓ ÂÁΤ-
Ê·ÏÔ Î·È ÙÔ˘˜ ÛÎÂÏÂÙÈÎÔ‡˜ Ì˘˜ (10). √È ÂÚÈÛ-
ÛfiÙÂÚ˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ ƒ∆ƒ¡ 11
ÚÔηÏÔ‡Ó ÌÂÙ·‚ÔÏ‹ ÛÙËÓ ·ÏÏËÏÔ˘¯›· ÙˆÓ
·ÌÈÓÔͤˆÓ Ù˘ ÚˆÙ½Ó˘ SHP2, Ì ·ÎfiÏÔ˘-
ıÔ ÙË Û˘Ó¯‹ ÂÓÂÚÁÔÔ›ËÛË Ù˘ ÚˆÙ½Ó˘. ∏
‰È·ÚÎÒ˜ ÂÓÂÚÁÔÔÈË̤ÓË ÚˆÙ½ÓË ·ÔÚ˘ıÌ›-
˙ÂÈ Ù· Û˘ÛÙ‹Ì·Ù· ÂϤÁ¯Ô˘ ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ÏÂÈ-
ÙÔ˘ÚÁÈÒÓ ·Ó¿Ù˘Í˘ Î·È ‰È·›ÚÂÛ˘ Ì ·ÔÙ¤-
ÏÂÛÌ· ÙËÓ ÂÌÊ¿ÓÈÛË ÙˆÓ ÛËÌ›ˆÓ Î·È ¯·Ú·ÎÙË-
ÚÈÛÙÈÎÒÓ ÙÔ˘ NS (10). ªÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ƒ∆ƒ¡
11 ¤¯Ô˘Ó ·ÓȯÓ¢ı› Û ÔÛÔÛÙfi 33-60% ÙˆÓ
·ÛıÂÓÒÓ Ì NS, ¤¯Ô˘Ó ‰Â ÂÚÈÁÚ·Ê› ÂÚÈÛÛfi-
ÙÂÚ˜ ·fi 30 ‰È·ÊÔÚÂÙÈΤ˜, ·ÚÂÚÌËÓ‡ÛÈ̘
΢ڛˆ˜, ÌÂÙ·ÏÏ¿ÍÂȘ (11).
™ÙËÓ ÔÈÎÔÁ¤ÓÂÈ· Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È, ÂÌÊ·Ó›-
˙ÂÙ·È ÛÙ¤ÓˆÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ÛÙȘ ‰‡Ô ‰›‰˘-
̘ ·‰ÂÏʤ˜ (πππ:1, πππ:2), ÂÓÒ Â›Û˘ ηډÈÔ¿-
ıÂÈ· Ô˘ ‰ÂÓ ¤¯ÂÈ Ù·˘ÙÔÔÈËı› ·Ó·Ê¤ÚÂÙ·È ÛÂ
‰‡Ô ·ÎfiÌ· ̤ÏË (π:4 Î·È ππ:3). ∏ ÌÂÙ¿ÏÏ·ÍË Ô˘
‰È·ÈÛÙÒıËΠÛÙËÓ ·ÛıÂÓ‹-‰Â›ÎÙË Ì ÙË ÛÙ¤-
ÓˆÛË Ó¢ÌÔÓÈ΋˜ (πππ:1) ·ÊÔÚ¿ ÙÔ ÂÍfiÓÈÔ 3,
ÂÓÒ Û ÚÔËÁÔ‡ÌÂÓ˜ ÌÂϤÙ˜ Ë ÛÙ¤ÓˆÛË Ù˘
Ó¢ÌÔÓÈ΋˜ ‚·Ï‚›‰·˜ Û˘Û¯ÂÙ›˙ÂÙ·È Û˘¯ÓfiÙÂÚ·
Ì ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÂÍfiÓÈÔ 8 (3,8,11,12). ªÈ·
·ÎfiÌ· ÂӉȷʤÚÔ˘Û· ·Ú·Ù‹ÚËÛË Â›Ó·È fiÙÈ ÂÓÒ
fiÏ· Ù· ̤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ Ì ÙËÓ ÎÔÈÓ‹ ÌÂ-
Ù¿ÏÏ·ÍË (·ÛıÂÓ‹˜-‰Â›ÎÙ˘, ÌËÙ¤Ú·, ÌËÙÚÈÎfi˜
ı›Ԙ) ¤¯Ô˘Ó ÎÔÈÓ¿ ÌÔÚÊÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈ-
ο, ÂÌÊ·Ó›˙Ô˘Ó ÂÓÙÔ‡ÙÔȘ ÎÏÈÓÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ·.
™˘ÁÎÂÎÚÈ̤ӷ Ë ÌËÙ¤Ú· (ππ:2) ‰ÂÓ ¤¯ÂÈ Û˘ÁÁÂÓ‹
ηډÈÔ¿ıÂÈ· fiˆ˜ Ù· ‰›‰˘Ì· ·È‰È¿ Ù˘, ÂÓÒ
ÛÙÔ ·ÙÔÌÈÎfi Ù˘ ·Ó·ÌÓËÛÙÈÎfi ·Ó·Ê¤ÚÂÙ·È ÌfiÓÔ
·ÈÌÔÚÚ·ÁÈ΋ ‰È¿ıÂÛË. √ ‰Â ı›Ԙ ·fi ÙË ÌËÙ¤-
Ú· (ππ:3) ¤¯ÂÈ “Û˘ÛÙÔÏÈÎfi ʇÛËÌ·” Î·È Û ·ÓÙ›-
ıÂÛË Ì ٷ ˘fiÏÔÈ· ¿Û¯ÔÓÙ· ̤ÏË ÂÌÊ·Ó›˙ÂÈ
ηÓÔÓÈÎfi ·Ó¿ÛÙËÌ·. ∏ ÂÓ‰ÔÔÈÎÔÁÂÓÂȷ΋ ÔÈ-
ÎÈÏÔÌÔÚÊ›· ¤¯ÂÈ ·Ú·ÙËÚËı› Î·È Û ¿ÏÏÔ˘˜
·ÛıÂÓ›˜ Ì ÙË ÌÂÙ¿ÏÏ·ÍË A188G Î·È ·Ô‰›‰Â-
Ù·È Û ÌÂȈ̤ÓË ‰ÈÂÈÛ‰˘ÙÈÎfiÙËÙ· ÙÔ˘ ÁÔÓȉ›Ô˘
‹/Î·È Û Èı·Ó‹ Ú˘ıÌÈÛÙÈ΋ ‰Ú¿ÛË ¿ÏψÓ
(¿ÁÓˆÛÙˆÓ) ÁÔÓȉ›ˆÓ Î·È ÂÚÈ‚·ÏÏÔÓÙÈÎÒÓ ·-
Ú·ÁfiÓÙˆÓ (3,8,11,12).
ŸÛÔÓ ·ÊÔÚ¿ ÙÔÓ Èı·Ófi ·ıÔÁÂÓÂÙÈÎfi ÚfiÏÔ
ÙÔ˘ ÔÏ˘ÌÔÚÊÈÛÌÔ‡ -21C→T ÛÙÔ ÂÛfiÓÈÔ 7 ÙÔ˘
ÁÔÓȉ›Ô˘ PTPN 11 Ô˘ ‰È·ÈÛÙÒıËΠÛÙËÓ
·ÛıÂÓ‹-‰Â›ÎÙË, ı· Ú¤ÂÈ Ó· ÂÎÙÈÌËı› ÌfiÓÔ
·ÊÔ‡ ‰ÈÂÚ¢ÓËı› Û ÌÂÁ·Ï‡ÙÂÚÔ ·ÚÈıÌfi ·ÛıÂ-
ÓÒÓ Ì NS Î·È ÌÂÙ¿ ·fi Û˘Û¯¤ÙÈÛË Ì ÙÔÓ ˘ÁÈ‹
ÏËı˘ÛÌfi. ™‡ÓÙÔÌ· ı· ÍÂÎÈÓ‹ÛÂÈ Ô Î·Ù·Ú¯‹Ó
¤ÏÂÁ¯Ô˜ ÁÈ· ÙËÓ ·ÚÔ˘Û›· ‹ ÌË ÙÔ˘ ÔÏ˘ÌÔÚÊÈ-
ÛÌÔ‡ ·˘ÙÔ‡ Û ‰Â›ÁÌ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 100 Ê˘ÛÈÔ-
ÏÔÁÈÎÒÓ ·ÙfiÌˆÓ ÚÔÎÂÈ̤ÓÔ˘ Ó· ÚÔÛ‰ÈÔÚÈÛı›
ÙÔ ÔÛÔÛÙfi Ù˘ ·ÚÔ˘Û›·˜ ·˘ÙÔ‡ ÙÔ˘ ÔÏ˘ÌÔÚ-ÊÈÛÌÔ‡ ÛÙÔÓ Ê˘ÛÈÔÏÔÁÈÎfi ÏËı˘ÛÌfi.
∏ Ê·ÈÓÔÙ˘È΋ ÔÈÎÈÏÔÌÔÚÊ›· ÙÔ˘ NS ÂÈ-‚¿ÏÏÂÈ ÙÔÓ ÌÔÚÈ·Îfi ÁÂÓÂÙÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ PTPN11 Û fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ¿Ù˘Ô Ê·ÈÓfiÙ˘ÔηıÒ˜ Î·È ÛÙÔ˘˜ Û˘ÁÁÂÓ›˜ 1Ô˘ ‚·ıÌÔ‡ ÁÈ· ÙËÓÂȂ‚·›ˆÛË Ù˘ ÎÏÈÓÈ΋˜ ‰È¿ÁÓˆÛ˘ Î·È ÙË Ûˆ-ÛÙ‹ Î·È ¤ÁηÈÚË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË.
µÈ‚ÏÈÔÁÚ·Ê›·
1. McKusick VA. Mendelian Inheritance in Man. A Cat-alog of Human Genes and Genetic Disorders. 12thed. Baltimore and London: The Johns HopkinsUniversity Press; 1998, OMIM # 176876. Webpage:http://www.nlm.nih.gov/omim/
2. Allanson JE. Noonan syndrome. In: Cassidy SB, Al-lanson JE, editors. Management of Genetic Syn-dromes. 2nd ed. Wiley Liss, Inc; 2005. p. 385.
3. Targaglia M, Kalidas K, Shaw A, Song X, Musat LD,van der Burgt I et al. PTPN11 mutations in Noonansyndrome: molecular spectrum, genotype-phenotypecorrelation and phenotypic heterogeneity. Am J HumGenet 2002;70:1555-1563.
4. Noonan JA. Hypertelorism with Turner phenotype.A new syndrome with associated congenital heart dis-ease. Am J Dis Child 1968;116;373-380.
5. Noonan JA. Noonan syndrome revisited. J Pediatr1999;135:667-668.
6. Jongmans M, Otten B, Noordam K, van der Burgt I.Genetics and variation in phenotype in Noonan syn-drome. Horm Res 2004;62 (Suppl 3):S56-S59.
7. Marino B, Digilio MC, Toscano A, Giannotti A, Dal-lapiccola B. Congenital heart diseases in children withNoonan syndrome: An expanded cardiac spectrumwith high prevalence of atrioventricular canal. J Pedi-atr 1999;135:703-706.
8. Sarkozy A, Conti E, Seripa D, Digilio MC, Grifone N,Tandoi C et al. Correlation between PTPN11 genemutations and congenital heart defects in Noonanand LEOPARD syndromes. J Med Genet2003;40:704-708.
9. Jamieson CR, van der Burgt I, Brady AF, van Reen M,Elsawi MM, Hol F et al. Mapping a gene for Noonansyndrome to the long arm of chromosome 12. NatGenet 1994;8:357-360.
10. Tamir I, Dal Porto JM, Cambier JC. Cytoplasmic pro-tein tyrosine phosphatase SHP-1 and SHP-2: regula-tors of B cell signal transduction. Curr Opin Im-munol 2000;12:307-315.
11. Yoshida R, Hasegawa T, Hasegawa Y, Nagai T, Ki-noshita E, Tanaka Y et al. Protein-tyrosine phos-phatase, nonreceptor type II mutation analysis andclinical assessment in 45 patients with Noonan syn-drome. J Clin Endocrinol Metab 2004;89:3359-3364.
12. Bertola DR, Pereira CA, de Oliveira PSL, Kim CA,Krieger JE. Clinical variability in a Noonan syndromefamily with a new PTPN11 gene mutation. Am J Med
Genet 2004;130A:378-383.
¶·È‰È·ÙÚÈ΋ 2006;69:447-451
452 ∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏ CASE REPORT
¶·È‰È·ÙÚÈ΋ 2006;69:452-457
∞ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÛÙÂÔfiÚˆÛ˘ ηÈ
ÙˆÓ Î·Ù·ÁÌ¿ÙˆÓ Ì ‰ÈÓ·ÙÚÈÔ‡¯Ô ·ÌȉÚÔÓ¿ÙË
Û ‚Ú¤ÊÔ˜ Ì ·ÙÂÏ‹ ÔÛÙÂÔÁ¤ÓÂÛË Ù‡Ô˘ IV
ª. ¶··Ê˘Ï·ÎÙÔ‡1, ¶. §··ÙÛ¿Ó˘2, ¶. ¶Ô˘ÏfiÔ˘ÏÔ˜1, M. B. Petersen3, ∫. ªÈ¯·Ï·Î¿ÎÔ˘4,
∫. ¶ÚÔ‡ÓÙ˙Ô˘ - ∫·ÛÛÈÔ‡1
¶ÂÚ›ÏË„Ë: ∏ ·ÙÂÏ‹˜ ÔÛÙÂÔÁ¤ÓÂÛË (∞√) ·ÔÙÂÏ› ÂÙÂÚÔÁÂÓ‹ ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹ Ì ·˘ÍË̤ÓË¢ıÚ·˘ÛÙfiÙËÙ· ÙˆÓ ÔÛÙÒÓ, ÔÛÙÂÔfiÚˆÛË Î·È ÂΉËÏÒÛÂȘ ·fi ¿ÏÏÔ˘˜ Û˘Ó‰ÂÙÈÎÔ‡˜ ÈÛÙÔ‡˜. ¶ÂÚÈ-ÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ‚Ú¤ÊÔ˘˜ ËÏÈΛ·˜ ÔÎÙÒ ÌËÓÒÓ, ÙÔ ÔÔ›Ô ·ÚÔ˘Û›·Û ÙÚ›· ηٿÁÌ·Ù· ÙˆÓÔÛÙÒÓ, ÔÛÙÂÔfiÚˆÛË Î·È Î·ı›˙ËÛË ÙˆÓ ÛÔÓ‰˘ÏÈÎÒÓ ÛˆÌ¿ÙˆÓ Ù˘ ÔÛÊ˘˚΋˜ ÌÔ›Ú·˜. ∞ÔÊ·Û›-ÛÙËÎÂ Ë ÂÓ‰ÔÊϤ‚È· ıÂڷ›· Ì ‰ÈÓ·ÙÚÈÔ‡¯Ô ·ÌȉÚÔÓ¿ÙË Î·È Ì ‰ÔÛÔÏÔÁÈÎfi Û¯‹Ì· 0,5-1,5mg/kg ËÌÂÚËÛ›ˆ˜ ÁÈ· ‰‡Ô Ë̤Ú˜, οı 6-12 ‚‰ÔÌ¿‰Â˜. ™ÙËÓ ÚÒÙË Û˘Ó‰ڛ·, ÙÔ ‚Ú¤ÊÔ˜ ·ÚÔ˘-Û›·Û ˘ÚÂÙfi 39,5oC Î·È Ô˘‰ÂÙÂÚÔÂÓ›·, ÁÈ· ‚Ú·¯‡ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·. ∂›Û˘, ·Ú·ÙËÚ‹ıËÎÂÂÏ¿ÙÙˆÛË Ù˘ Û˘ÁΤÓÙÚˆÛ˘ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ (Ca++) ÛÙÔÓ ÔÚfi Î·È ·‡ÍËÛË Ù˘ ·Ú·ıÔÚÌfiÓ˘(PTH). ∆· ÎÏÈÓÈο Û˘ÌÙÒÌ·Ù· Ù˘ Ïԛ̈͢ Ô˘ ÌÔÈ¿˙Ô˘Ó Ì ÈÔÁÂÓ‹ Û˘Ó‰ÚÔÌ‹ Î·È ÔÈ ‚ÈÔ¯ËÌÈ-Τ˜ ‰È·Ù·Ú·¯¤˜ ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙÔ˘˜ ÂfiÌÂÓÔ˘˜ ·ÎÏÔ˘˜ ıÂڷ›·˜, ÂÎÙfi˜ ·fi ÙËÓ fiÁ‰ÔˤÁ¯˘ÛË, ηٿ ÙËÓ ÔÔ›· ‚Ú¤ıËΠÌÈÎÚ‹ ·‡ÍËÛË Ù˘ PTH. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜, Ô ·ÚÈı-Ìfi˜ ÙˆÓ Î·Ù·ÁÌ¿ÙˆÓ ÌÂÈÒıËÎÂ. ∏ Ï¿ÁÈ· ·ÎÙÈÓÔÁÚ·Ê›· (·/·) ÔÛÊ˘˚΋˜ ÌÔ›Ú·˜ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹-Ï˘ (√ª™™) ¤‰ÂÈÍ ÙËÓ Â·ÓfiÚıˆÛË ÙÔ˘ ‡„Ô˘˜ ÙˆÓ ÛÔÓ‰˘ÏÈÎÒÓ ÛˆÌ¿ÙˆÓ Î·È Ù˘ ÔÛÙÂÔfiÚˆ-Û˘. ∏ ÓfiÛÔ˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜ Ù·ÍÈÓÔÌ‹ıËΠÛÙÔÓ Ù‡Ô IV Ì ¯·ÌËÏfi ·Ó¿ÛÙËÌ·. ∆Ô Ì‹ÎÔ˜ ÛÒÌ·ÙÔ˜(ª™) ‚Ú¤ıËΠ67 cm, ÛÙË 10Ë ÂηÙÔÛÙÈ·›· ı¤ÛË (∂£) Ù˘ η̇Ï˘ ·Ó¿Ù˘Í˘ ÛÙËÓ ËÏÈΛ· ÙˆÓ 8ÌËÓÒÓ Î·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜, Û ËÏÈΛ· 26 ÌËÓÒÓ, ÌÈÎÚfiÙÂÚÔ ·fi ÙËÓ ÙÚ›ÙË ∂£ (ª™:81 cm). ∂Ô̤ӈ˜, Ë ‰ÈÓ·ÙÚÈÔ‡¯Ô˜ ·ÌȉÚÔÓ¿ÙË ‰ÂÓ Â›¯Â ¢ÓÔ˚΋ ›‰Ú·ÛË ÛÙÔ ·Ó¿ÛÙËÌ· ÙÔ˘·ÛıÂÓ‹. ™˘ÛÙ‹ıËÎÂ Ë ÙÔÔı¤ÙËÛË ÂÓ‰ÔÌ˘ÂÏÈÎÔ‡ ‹ÏÔ˘ ÛÙ· ÌËÚÈ·›· ÔÛÙ¿ ÁÈ· ÙË ‚ÂÏÙ›ˆÛË Ù˘ ‚¿-‰ÈÛ˘ Î·È Ù˘ ÈÛÔÚÚÔ›·˜. ∏ ·/· ·ÚÈÛÙÂÚÔ‡ ÌËÚÈ·›Ô˘ ÔÛÙÔ‡ ¤‰ÂÈÍ ÙËÓ ÂÌÊ¿ÓÈÛË Ï¢ÎÒÓ ÂÚÈÔ¯ÒÓÛÙËÓ Â›Ê˘ÛË, ÌÂÙ¿ ·fi ÙË ıÂڷ›· 18 ÌËÓÒÓ. ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ‰ÈÓ·ÙÚÈÔ‡¯Ô˜ ·ÌȉÚÔÓ¿ÙˉÂÓ ¤¯ÂÈ Â›‰Ú·ÛË ÛÙË Ê˘ÛÈ΋ ÔÚ›· Ù˘ ∞√, ·ÏÏ¿ Â›Ó·È Ë ÌÔÓ·‰È΋ Ê·Ú̷΢ÙÈ΋ ıÂڷ›· ÚÔ-ÎÂÈ̤ÓÔ˘ Ó· ÂÈÙ¢¯ı› Ë Î·Ï‡ÙÂÚË Û˘Ìو̷ÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÓfiÛÔ˘.
§¤ÍÂȘ ÎÏÂȉȿ: ∞ÙÂÏ‹˜ ÔÛÙÂÔÁ¤ÓÂÛË, ηٿÁÌ·Ù·, ·ÌȉÚÔÓ¿ÙË.
Symptomatic pamidronate treatment of an infant with osteogenesis
imperfecta type IV
M. Papaphylactou1, P. Lapatsanis2, P. Poulopoulos1, M. B. Petersen3, K. Michalakakou4,
K. Prountzou - Kassiou1
Abstract: Osteogenesis imperfecta (√π) is a heterogenous genetic disorder with increased bonefragility, osteoporosis and other connective tissue manifestations. The case is described of an 8month-old infant who had three fractures, osteoporosis and collapsed vertebral bodies of the lumbarspine. It was decided to administer treatment with intravenous pamidronate in doses of 0.5-1.5mg/kg/day for two days, every 6-12 weeks. On the first infusion the infant developed a fever of 39.5oCand neutropenia for a short period of time. Reduction in the serum concentration of calcium and anincrease of parathyroid hormone were observed. The clinical symptoms of a flu-like syndrome andthe biochemical disturbances were observed only on the first infusion, and on the eighth infusion asmall increase in serum parathyroid hormone was again found. Following treatment the number offractures diminished and X-ray of the spine revealed restoration of vertebral height and improvementof osteoporosis. The infant’s disease is classified as OI type IV with short stature. His length was 67cm (10th centile) at the age of 8 months and 81 cm (<3rd centile) at the age of 26 months, showingthat the pamidronate had no beneficial effect on the length of the child. It was suggested to place nails
1 ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó·
2 √ÌfiÙÈÌÔ˜ ∫·ıËÁËÙ‹˜¶·È‰È·ÙÚÈ΋˜¶·ÓÂÈÛÙËÌ›Ô˘ πˆ·ÓÓ›ÓˆÓ
3 ¢È‡ı˘ÓÛË °ÂÓÂÙÈ΋˜,πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ÙÔ˘¶·È‰ÈÔ‡, ¡ÔÛÔÎÔÌ›Զ·›‰ˆÓ “∞Á›· ™ÔÊ›·”,∞ı‹Ó·
4 µÈÔ¯ËÌÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
¶. ¢. §··ÙÛ¿Ó˘ ∞ÛÎÏËÈÔ‡ 44 ∆.∫. 114 71, ∞ı‹Ó·
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 23-02-2006∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 18-07-2006
1 1st Paediatric Clinic, “Aghia Sophia” Children’sHospital, Athens
2 Emeritus Professor ofPaediatrics, University ofIoannina
3 Department of Genetics,Institute of Child Health,”Aghia Sophia” Children’sHospital, Athens
4 Biochemical Department,“Aghia Sophia” Children’sHospital, Athens
Correspondence:
P. D. Lapatsanis 44, Asklepiou Str. 114 71, Athens
Date of submission: 23-02-2006Date of approval: 18-07-2006
453∞ÙÂÏ‹˜ ÔÛÙÂÔÁ¤ÓÂÛË Î·È ·ÌȉÚÔÓ¿ÙË
¶·È‰È·ÙÚÈ΋ 2006;69:452-457
in the femurs to improve ambulatory status. X-ray of the left femur showed the appearance of a whitearea in the epiphysis after 18 months of treatment. It was concluded that pamidronate did not affectthe natural course of the disease, but it is the only medical treatment available to provide the bestsymptomatic improvement of OI.
Key words: Osteogenesis imperfecta, fractures, pamidronate.
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Ì·ÎÚÒÓ ÔÛÙÒÓ Î·È ¤ÏÂÁ¯Ô˜ ÙˆÓ ÔÛÙÈÎÒÓ ·Ï-
ÁÒÓ. ∏ È·ÙÚÈ΋ ·‰˘Ó·Ù› Ó· ÚÔÛʤÚÂÈ ›·ÛË
Ù˘ ÓfiÛÔ˘.
¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÔÚ›· Î·È Ë ·ÓÙÈÌÂÙÒÈÛË
‚Ú¤ÊÔ˘˜, Ô˘ ˘Ô‚Ï‹ıËΠ۠ıÂڷ›· Ì ‰È-
Ó·ÙÚÈÔ‡¯Ô ·ÌȉÚÔÓ¿ÙË.
¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘
¶ÚfiÎÂÈÙ·È ÁÈ· ·ÁfiÚÈ ËÏÈΛ·˜ 8 ÌËÓÒÓ Ô˘ÂÈÛ‹¯ıË ÛÙËÓ ÎÏÈÓÈ΋ Ì ηٿÁÌ·Ù· ·fi ÙȘÚÒÙ˜ ‚‰ÔÌ¿‰Â˜ Ù˘ ˙ˆ‹˜ ÙÔ˘. ∆Ô ÚÒÙÔοٷÁÌ· ·ÚÔ˘ÛÈ¿ÛÙËΠÛÙËÓ ËÏÈΛ· ÙˆÓ ÙÂÛ-Û¿ÚˆÓ Â‚‰ÔÌ¿‰ˆÓ, ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Û›ÙÈ-Û˘, ÛÙÔ ·ÚÈÛÙÂÚfi ‚Ú·¯ÈfiÓÈÔ ÔÛÙfi, Ì ԛ‰ËÌ·Î·È ÂÚÈÔÚÈÛÌfi Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜. ∆Ô ‰Â‡ÙÂÚÔοٷÁÌ· ·ÚÔ˘ÛÈ¿ÛÙËÎÂ, Û ËÏÈΛ· 3 ÌËÓÒÓ,ÛÙÔ ‰ÂÍÈfi ÌËÚÈ·›Ô ÔÛÙfi ηٿ ÙËÓ ·ÏÏ·Á‹ Ù˘¿Ó·˜ (∂ÈÎfiÓ· 1·, ‚).
™Â ËÏÈΛ· 5 ÌËÓÒÓ ÂÈÛ‹¯ıË Û ¶·ÓÂÈÛÙË-Ìȷ΋ ∫ÏÈÓÈ΋ Ù˘ £ÂÛÛ·ÏÔӛ΢ ÁÈ· ‰ÈÂÚ‡ÓË-ÛË Î·È ÌÂÙ¿ ·fi ÙÚÂȘ Ë̤Ú˜ ‰È·ÁÓÒÛÙËΠ∞√.
™Â ËÏÈΛ· 7 ÌËÓÒÓ ·ÚÔ˘Û›·ÛÂ Ó¤Ô Î¿Ù·ÁÌ·ÛÙÔ ·ÚÈÛÙÂÚfi ÌËÚÈ·›Ô ÔÛÙfi. ™ÙÔÓ fiÁ‰ÔÔ Ì‹Ó··ÚÔ˘Û›·Û ¤Î‰ËÏË ˘ÔÎÈÓËÙÈÎfiÙËÙ· ÙÔ˘ ‰Â-ÍÈÔ‡ ÌËÚÈ·›Ô˘ ¿ÎÚÔ˘. ∞ÎÙÈÓÔÏÔÁÈο ‰ÂÓ ·Ú·-ÙËÚ‹ıËΠοٷÁÌ·, ·ÏÏ¿ Ú·È‚fi Û¯‹Ì· ÌËÚÈ·›-ˆÓ ÔÛÙÒÓ.
∫·Ù¿ ÙË Ê˘ÛÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËηÓÏ¢ÎfiÊ·ÈË ·fi¯ÚˆÛË ÛÎÏËÚÔ‡ ¯ÈÙÒÓ· ÔÊı·Ï-ÌÒÓ, ÌÂÁ¿ÏË ÚfiÛıÈ· ËÁ‹, ¤Ó· ‰fiÓÙÈ ÛÙËÓ Î¿-Ùˆ ÁÓ¿ıÔ Î·È ÌÔÁÁÔÏÔÂȉ‹˜ ÎËÏ›‰· ÛÙËÓ ÔÛÊ˘-Ô˚ÂÚ¿ ¯ÒÚ·. ∞fi Ù· ¿ÏÏ· Û˘ÛÙ‹Ì·Ù· ‰ÂÓ ·-Ú·ÙËÚ‹ıËÎ·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∆· ۈ̷-ÙÔÌÂÙÚÈο ÛÙÔȯ›· ‹Ù·Ó Ù· ·ÎfiÏÔ˘ı·: ‚¿ÚÔ˜ÛÒÌ·ÙÔ˜ (µ™): 7560 g (3Ë-10Ë ∂£), ª™: 67,5cm (10Ë ∂£) Î·È ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜ (¶∫): 46cm (95Ë ∂£).
√ ·ÈÌ·ÙÔÏÔÁÈÎfi˜ Î·È ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ‰ÂÈÎÙÒÓ ÛÙÔ ·›Ì·.™ÙËÓ Ï¿ÁÈ· ·/· √ª™™ ÚÈÓ ÙË ıÂڷ›·, ·-Ú·ÙËÚ‹ıËΠ¤Î‰ËÏË ÔÛÙÂÔfiÚˆÛË, ÎԛϷÓÛËÙˆÓ ÛÔÓ‰˘ÏÈÎÒÓ ÛˆÌ¿ÙˆÓ Î·È ÌÂÁ¿Ï· ÌÂÛÔ-ÛÔÓ‰‡ÏÈ· ‰È·ÛÙ‹Ì·Ù· (∂ÈÎfiÓ· 2·).
∞ÔÊ·Û›ÛÙËΠ¤Ó·ÚÍË ıÂڷ›·˜ Ì ÂÓ‰Ô-ÊϤ‚È· ¯ÔÚ‹ÁËÛË ‰ÈÓ·ÙÚÈÔ‡¯Ô˘ ·ÌȉÚÔÓ¿-Ù˘ Û ‰fiÛË 0,5 mg/kg ËÌÂÚËÛ›ˆ˜ ÁÈ· ‰‡ÔË̤Ú˜. √È Û˘Ó‰ڛ˜ ·ӷϷ̂¿ÓÔÓÙ·Ó Î¿-ı 6-12 ‚‰ÔÌ¿‰Â˜ Î·È Ë ‰fiÛË ÙÔ˘ Ê·ÚÌ¿ÎÔ˘Î˘Ì¿ÓıËΠ·fi 0,5 ¤ˆ˜ 1,5 mg/kg ËÌÂÚËÛ›ˆ˜(∂ÈÎfiÓ· 3).
454 ª. ¶··Ê˘Ï·ÎÙÔ‡ Î·È Û˘Ó.
¶·È‰È·ÙÚÈ΋ 2006;69:452-457
Ÿˆ˜ Ê·›ÓÂÙ·È Î·È ·fi ÙË Û¯ËÌ·ÙÈ΋ ·-
Ú¿ÛÙ·ÛË, ÛÙËÓ ÚÒÙË Û˘Ó‰ڛ· ·ÚÔ˘Û›·ÛÂ
Ì›ˆÛË ÙˆÓ ÙÈÌÒÓ ÙÔ˘ Ca++ ÛÙÔÓ ÔÚfi Î·È ·‡ÍË-
ÛË Ù˘ PTH, Ô˘ ·Ô‰fiıËΠÛÙËÓ ÎÈÓËÙÔÔ›Ë-
ÛË ·Û‚ÂÛÙ›Ô˘ ·fi ÙÔ˘˜ Ì·Ï·ÎÔ‡˜ ÈÛÙÔ‡˜ ÛÙ·
∂ÈÎfiÓ· 1. ·) ∫¿Ù·ÁÌ· ·ÚÈÛÙÂÚÔ‡ ‚Ú·¯ÈfiÓÈÔ˘ ÔÛÙÔ‡. ‚) √ÛÙÂÔfiÚˆÛË Ì οٷÁÌ· ‰ÂÍÈÔ‡ ÌËÚÈ·›Ô˘ ÔÛÙÔ‡.
·
‚
∂ÈÎfiÓ· 2. ·) ∏ Ï¿ÁÈ· ·/· √ª™™, ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂ-ڷ›·˜, ‰Â›¯ÓÂÈ ¤Î‰ËÏË ÔÛÙÂÔfiÚˆÛË Ì ηı›˙ËÛË ÙˆÓÛÔÓ‰˘ÏÈÎÒÓ ÛˆÌ¿ÙˆÓ. ‚) ªÂÙ¿ ·fi 12 Ì‹Ó˜ ıÂڷ›·˜Ì ‰ÈÓ·ÙÚÈÔ‡¯Ô ·ÌȉÚÔÓ¿ÙË, Ê·›ÓÂÙ·È ·‡ÍËÛË ÙÔ˘ ÌÂÁ¤-ıÔ˘˜ ÙˆÓ ÛˆÌ¿ÙˆÓ ÙˆÓ ÛÔÓ‰‡ÏˆÓ Î·È Ì›ˆÛË ÙˆÓ Â˘ÚË-Ì¿ÙˆÓ Ù˘ ÔÛÙÂÔfiÚˆÛ˘.
·
‚
455∞ÙÂÏ‹˜ ÔÛÙÂÔÁ¤ÓÂÛË Î·È ·ÌȉÚÔÓ¿ÙË
ÔÛÙ¿. ÕÚ¯ÈÛÂ Û˘ÛÙËÌ·ÙÈ΋ ¯ÔÚ‹ÁËÛË ‚ÈÙ·Ì›-
Ó˘ D (10 Ìg ËÌÂÚËÛ›ˆ˜), ·Ú¿ Ù· Ê˘ÛÈÔÏÔÁÈ-
ο ›‰· Ù˘ 25-˘‰ÚÔ͢-‚ÈÙ·Ì›Ó˘ D (25-
√∏-D) Û ‰‡Ô ÌÂÙÚ‹ÛÂȘ (23 Î·È 25 ng/ml).
º˘ÛÈÔÏÔÁÈ΋ Û˘ÁΤÓÙÚˆÛË Ca++ ÔÚÔ‡, ·ÏÏ¿ ÌÈ-
ÎÚ‹ ·‡ÍËÛË ÙÔ˘ ÂȤ‰Ô˘ Ù˘ PTH, ‚Ú¤ıËÎÂ
Î·È ÛÙËÓ fiÁ‰ÔË Û˘Ó‰ڛ·, Û ËÏÈΛ· 26 ÌËÓÒÓ
(™Â٤̂ÚÈÔ˜ 2005). ∆Ë ‰Â‡ÙÂÚË Ë̤ڷ ÌÂÙ¿
ÙËÓ ÚÒÙË ¤Á¯˘ÛË, Ô ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·Û ˘-
ÚÂÙfi 39,5ÔC, Ô˘ ˘Ô¯ÒÚËÛ Û 48 ÒÚ˜. ∆· ·È-
Ì·ÙÔÏÔÁÈο ·ÔÙÂϤÛÌ·Ù· ÌÂÙ¿ ÙËÓ ÚÒÙË Û˘-
Ó‰ڛ· ‹Ù·Ó Ù· ·ÎfiÏÔ˘ı·: ÏÂ˘Î¿ ·ÈÌÔÛÊ·›ÚÈ·
(WBC): 3300/mm3 [ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· (¶):
4%, ÏÂÌÊÔ·ÙÙ·Ú· (§): 84%, ÌÔÓÔ‡ÚËÓ·
(ª): 7%], ·fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ ¶: 132/mm3. ∂·-
Ó¿ÏË„Ë Ù˘ ÁÂÓÈ΋˜ ·›Ì·ÙÔ˜ Û ‰¤Î· Ë̤Ú˜
¤‰ÂÈÍÂ: WBC: 8500/mm3 (¶: 15%, §: 74%, ª:
7%), ·fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ ¶: 1275/mm3.
∏ ·Ó¿Ï˘ÛË ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ Î·È Ë cDNA
·Ó¿Ï˘ÛË ÙˆÓ COL1A1 (51 ÂÍÒÓÈ·) Î·È COL1A2
(52 ÂÍÒÓÈ·) ÁÔÓȉ›ˆÓ, ¤‰ÂÈÍ ÌÂÙ¿ÏÏ·ÍË ÛÙÔ
ÂÍÒÓÈÔ 37 ÙÔ˘ ÁÔÓȉ›Ô˘ COL1A1. ∏ ÌÂÙ¿ÏÏ·ÍË
·˘Ù‹ ÚÔ·ÙÂÈ Ì ·ÓÙÈηٿÛÙ·ÛË Ù˘ ÁÏ˘Î›-
Ó˘ ·fi ÙË ÛÂÚ›ÓË ÛÙË ı¤ÛË p.643 ÙÔ˘ ÁÔÓȉ›Ô˘
COL1A1 (p.Gly643Ser). ∆Ô ·ÔÙ¤ÏÂÛÌ· ·˘Ùfi
ÂȂ‚·›ˆÛ ٷ ÎÏÈÓÈο Î·È ‚ÈÔ¯ËÌÈο Â˘Ú‹Ì·-
Ù· Ù˘ ÓfiÛÔ˘ Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È. ∆· ÎÏÈÓÈο,
‚ÈÔ¯ËÌÈο Î·È ÌÔÚȷο Â˘Ú‹Ì·Ù· Â›Ó·È Û˘Ì‚·-
Ù¿ Ì ÙÔÓ Ù‡Ô IV Ù˘ ∞√.
∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜, ÙÔ ‚Ú¤ÊÔ˜‰ÂÓ ·ÚÔ˘Û›·˙ ¿ÏÁË Î·È Ô ‡ÓÔ˜ ÙÔ˘ ‹Ù·Ó ÈÔ‹ÚÂÌÔ˜ Û ۯ¤ÛË Ì ÙÔ ‰È¿ÛÙËÌ· ÚÈÓ ÙË ıÂÚ·-›·, Û‡Ìʈӷ Ì ÙËÓ ¿Ô„Ë ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘.
ªÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ·ÚÔ˘Û›·-Û ‰‡Ô ηٿÁÌ·Ù· - ÛÙËÓ ËÏÈΛ· ÙˆÓ 16 Î·È 19ÌËÓÒÓ - ηٿ ÙË ‚¿‰ÈÛË, ÛÙÔ ¿Óˆ ÙÚÈÙËÌfiÚÈÔÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ÌËÚÈ·›Ô˘ ÔÛÙÔ‡, fiÔ˘ Î·È ÙÔ Î¿-Ù·ÁÌ· ÛÙËÓ ËÏÈΛ· ÙˆÓ 7 ÌËÓÒÓ. ¢Ò‰Âη Ì‹Ó˜ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, Ë Ï¿ÁÈ· ·/·√ª™™ ¤‰ÂÈÍ fiÙÈ ·ÔηٷÛÙ¿ıËΠÙÔ ‡„Ô˜ÙˆÓ ÛÔÓ‰˘ÏÈÎÒÓ ÛˆÌ¿ÙˆÓ, Ì ۷ʋ Ì›ˆÛËÙˆÓ ÔÛÙÂÔÚˆÙÈÎÒÓ ·ÏÏÔÈÒÛÂˆÓ (∂ÈÎfiÓ· 2‚).™ÙËÓ ËÏÈΛ· ÙˆÓ 26 ÌËÓÒÓ Û˘ÛÙ‹ıËÎÂ Ë ÙÔÔ-ı¤ÙËÛË ÂÓ‰ÔÌ˘ÂÏÈÎÔ‡ ‹ÏÔ˘ ÛÙ· ÌËÚÈ·›· ÔÛÙ¿,ÏfiÁˆ ·Ú·ÌfiÚʈۋ˜ ÙÔ˘˜ (∂ÈÎfiÓ· 4). ™ÙËÓ·/· ·Ú·ÙËÚ‹ıËÎ·Ó Ï¢Τ˜ Ù·Èӛ˜ ÛÙËÓ Â›-Ê˘ÛË ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ÌËÚÈ·›Ô˘ ÔÛÙÔ‡.
™˘˙‹ÙËÛË
∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ∞√ Ì ÂÓ‰ÔÊϤ‚È· ¯Ô-Ú‹ÁËÛË ‰ÈÓ·ÙÚÈÔ‡¯Ô˘ ·ÌȉÚÔÓ¿Ù˘ Û˘ÓÈÛÙ¿-Ù·È ·fi ÙË ‚È‚ÏÈÔÁÚ·Ê›· (2-4). ŒÏÏËÓ˜ Û˘Á-ÁÚ·Ê›˜ (4) ·Ó·ÎÔ›ÓˆÛ·Ó ÂÚ›ÙˆÛË ÓÂÔÁÓԇ̠∞√, ÛÙÔ ÔÔ›Ô ¯ÔÚËÁ‹ıËΠ‰ÈÓ·ÙÚÈÔ‡¯Ô˜·ÌȉÚÔÓ¿ÙË, ·ÏÏ¿ ‰ÂÓ ÙË ‰ËÌÔÛ›Â˘Û·Ó Û ȷ-ÙÚÈÎfi ÂÚÈÔ‰ÈÎfi Î·È ‰ÂÓ ˘¿Ú¯Ô˘Ó ÛÙÔȯ›· ÁÈ·ÙËÓ ÔÚ›· Ù˘ ÂÚ›ÙˆÛ˘.
¶·È‰È·ÙÚÈ΋ 2006;69:452-457
HÏÈΛ· Û ̋Ó˜ 8 10 12 14 17 20 23 26
81
79
77
75
73
71
69
67
11
10
9
8
100
70
10
PT
H p
g/m
lC
a Ô
ÚÔ
‡ m
g/d
lª
‹Î
Ô˜
ÛÒ
Ì·
ÙÔ˜
cm
10Ë ∂£10Ë ∂£
3Ë ∂£ <3Ë ∂£
<3Ë ∂£
<3趣
<3Ë ∂£<3Ë ∂£
2004 2005
¢ÈÓ·ÙÚÈÔ‡¯Ô˜ ª¿ÈÔ˜ πÔ‡ÓÈÔ˜ ∞‡ÁÔ˘ÛÙÔ˜ √ÎÙÒ‚ÚÈÔ˜ ¶·ÌȉÚÔÓ¿ÙË 0,5 x 2 ËÌ. 0,75 x 2 ËÌ. 1,0 x 2 ËÌ. 1,25 x 2 ËÌ.
π·ÓÔ˘¿ÚÈÔ˜ ∞Ú›ÏÈÔ˜ πÔ‡ÏÈÔ˜ ™Â٤̂ÚÈÔ˜ 1,5 x 2 ËÌ. 1,0 x 3 ËÌ. 1,0 x 3 ËÌ. 1,15 x 2 ËÌ.
∂ÈÎfiÓ· 3. ∞ÂÈÎÔÓ›˙ÂÙ·È ÙÔ ‰ÔÛÔÏÔÁÈÎfi Û¯‹Ì· Ù˘ ÂÓ‰ÔÊϤ‚È·˜ ¯ÔÚ‹ÁËÛ˘ ‰ÈÓ·ÙÚÈÔ‡¯Ô˘ ·ÌȉÚÔÓ¿Ù˘, ÙÔ ª™, Ë Û˘ÁΤ-ÓÙÚˆÛË Ca++ Î·È PTH ÛÙÔÓ ÔÚfi Î·È Ë ËÏÈΛ· ÙÔ˘ ·ÛıÂÓ‹, ÛÙÔ˘˜ ·ÓÙ›ÛÙÔÈ¯Ô˘˜ ·ÎÏÔ˘˜ ıÂڷ›·˜.
456 ª. ¶··Ê˘Ï·ÎÙÔ‡ Î·È Û˘Ó.
™ÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È, ·Ú·-
ÙËÚ‹ıËΠ̛ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Î·Ù·ÁÌ¿-
ÙˆÓ Î·È ·‡ÍËÛË ÙÔ˘ ‡„Ô˘˜ ÙˆÓ ÛÔÓ‰˘ÏÈÎÒÓ
ÛˆÌ¿ÙˆÓ ÌÂÙ¿ ·fi 12 Ì‹Ó˜ ÂÓ‰ÔÊϤ‚È·˜ ıÂ-
ڷ›·˜, Ô˘ Û˘ÌʈÓÔ‡Ó Ì ÙË ‚È‚ÏÈÔÁÚ·Ê›·
(2,3,5-7). √ÚÈṲ̂ÓÔÈ ÂÚ¢ÓËÙ¤˜ (5) ÚÔÙ›ÓÔ˘Ó
ıÂڷ›· Û ·ÛıÂÓ›˜ Ì ∞√ fiÙ·Ó ·ÚÔ˘ÛÈ¿-
ÛÔ˘Ó ÂÚÈÛÛfiÙÂÚ· ·fi ‰‡Ô ηٿÁÌ·Ù· ÂÙËÛ›ˆ˜
Î·È Î·ı›˙ËÛË ÙˆÓ ÛÔÓ‰‡ÏˆÓ. ™ÙËÓ ·ÚÔ‡Û·
ÂÚ›ÙˆÛË, ÙÔ ‚Ú¤ÊÔ˜ ·ÚÔ˘Û›·Û ÙÚ›· ηٿÁ-
Ì·Ù· ÙÔ˘˜ ÚÒÙÔ˘˜ ÔÎÙÒ Ì‹Ó˜ Ù˘ ˙ˆ‹˜ ÙÔ˘
Î·È Î·ı›˙ËÛË ÙˆÓ ÛÔÓ‰˘ÏÈÎÒÓ ÛˆÌ¿ÙˆÓ.
∞ӷʤÚÂÙ·È (3,5) Ì›ˆÛË Ù˘ Û˘ÁΤÓÙÚˆÛ˘
ÙÔ˘ Ca++ ÛÙÔÓ ÔÚfi Î·È ·‡ÍËÛË Ù˘ PTH Û ·ÛıÂ-
Ó›˜ Ì ·Ó¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ D. ™ÙËÓ ÂÚ›Ùˆ-
ÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È, ·Ú¿ ÙȘ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ-
̤˜ ÙÔ˘ Ca++ Î·È Ù˘ 25-√∏-D ÛÙÔ ·›Ì·, ·Ú·-
ÙËÚ‹ıËΠÛÙËÓ ÚÒÙË ¤Á¯˘ÛË ÙÒÛË ÙÔ˘ ÂÈ-
¤‰Ô˘ ÙÔ˘ Ca++ ÛÙÔÓ ÔÚfi, ·fi 10,5 mg/dl Û 8,2
mg/dl, ηıÒ˜ Î·È ÌÂÁ¿ÏË ·‡ÍËÛË Ù˘ PTH, ·fi
20 pg/ml Û 105 pg/ml. ŒÙÛÈ, Û˘ÛÙ‹ıËÎÂ Û˘ÛÙË-
Ì·ÙÈ΋ ¯ÔÚ‹ÁËÛË ‚ÈÙ·Ì›Ó˘ D (10 Ìg ËÌÂÚË-
Û›ˆ˜) ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜. ¶·Úfi-
ÌÔÈ· ‚ÈÔ¯ËÌÈ΋ ‰È·Ù·Ú·¯‹ ‰ÂÓ ÛËÌÂÈÒıËΠÛÙȘ
ÂfiÌÂÓ˜ ¤ÍÈ ÂÁ¯‡ÛÂȘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘. øÛÙfiÛÔ,
ÛÙËÓ fiÁ‰ÔË Û˘Ó‰ڛ· ·Ú·ÙËÚ‹ıËΠÌÈÎÚ‹ ·‡-
ÍËÛË ÙÔ˘ ÂȤ‰Ô˘ Ù˘ PTH ÛÙÔÓ ÔÚfi (80
pg/ml), ÂÓÒ Ë ÙÈÌ‹ ÙÔ˘ Ca++ ÛÙÔ ·›Ì· ‚Ú¤ıËÎÂ
ÛÙ· ·ÓÒÙÂÚ· Ê˘ÛÈÔÏÔÁÈο ›‰· (11 mg/dl).
™ÙËÓ ÚÒÙË ¤Á¯˘ÛË ‰ÈʈÛÊÔÓÈÎÒÓ, ÂÚÈ-
ÁÚ¿ÊÂÙ·È Û‡Ó‰ÚÔÌÔ ÈÒÛˆ˜, Ì ˘ÚÂÙfi, Ï¢ÎÔ-
ÂÓ›· Î·È Ô˘‰ÂÙÂÚÔÂÓ›· (3,5). ™ÙÔ ‚Ú¤ÊÔ˜ Ù˘
·ÚÔ‡Û·˜ ÂÚ›ÙˆÛ˘ ·Ú·ÙËÚ‹ıËÎÂ, ÛÙËÓ
ÚÒÙË Û˘Ó‰ڛ·, ˘ÚÂÙfi˜ 39,5ÔC Î·È Ô˘‰ÂÙÂ-
ÚÔÂÓ›· (·fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ ¶: 132/mm3). ∆·
ÎÏÈÓÈο Û˘ÌÙÒÌ·Ù· ˘Ô¯ÒÚËÛ·Ó Û ‰‡Ô Ë̤-
Ú˜ Î·È Ù· ·ÈÌ·ÙÔÏÔÁÈο Â˘Ú‹Ì·Ù· Û ¯ÚÔÓÈÎfi
‰È¿ÛÙËÌ· 10 ËÌÂÚÒÓ. √ ·ÛıÂÓ‹˜ ‰ÂÓ ÂÌÊ¿ÓÈÛÂ
·ÚfiÌÔÈ· ÎÏÈÓÈο Î·È ·ÈÌ·ÙÔÏÔÁÈο Â˘Ú‹Ì·Ù·
ÛÙȘ ÂfiÌÂÓ˜ ÂÁ¯‡ÛÂȘ.
™‡Ìʈӷ Ì ٷ ÎÏÈÓÈο, ‚ÈÔ¯ËÌÈο Î·È ÌÔ-
Úȷο Â˘Ú‹Ì·Ù·, Ô ·ÛıÂÓ‹˜ ·Ó‹ÎÂÈ ÛÙÔÓ Ù‡Ô
IV Ù˘ ∞√, Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ¯·ÌËÏfi
‡„Ô˜, ÛÎÔÏ›ˆÛË, Ï¢ÎfiÊ·ÈÔ˘˜ ÛÎÏËÚÔ‡˜ ηÈ
‰˘ÛÁÂÓÂÛ›· ÙˆÓ Ô‰fiÓÙˆÓ (5). √ÚÈṲ̂ÓÔÈ ŒÏÏË-
Ó˜ ÂÈÛÙ‹ÌÔÓ˜ (6) ·Ó·Ê¤ÚÔ˘Ó ·‡ÍËÛË Ù˘
∂£ ÙÔ˘ ‡„Ô˘˜ Î·È ‚ÂÏÙ›ˆÛË ÙÔ˘ ÛÎÂÏÂÙÔ‡ ·È-
‰ÈÒÓ ËÏÈΛ·˜ 3-7 ÂÙÒÓ Ô˘ ·ÓÙÈÌÂÙˆ›ÛÙËηÓ
Ì ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ‰ÈʈÛÊÔÓÈο. ∆Ô ª™
‚Ú¤ıËÎÂ, ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È,
ÛÙË 10Ë ∂£ ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 10 ÌËÓÒÓ Î·È
ÌÂÙ¿ ÌÂÈÒıËÎÂ, οو ·fi ÙËÓ ÙÚ›ÙË ∂£, ̤¯ÚÈ
ÙËÓ ËÏÈΛ· ÙˆÓ 26 ÌËÓÒÓ. º·›ÓÂÙ·È fiÙÈ Ë ¯ÔÚ‹-
ÁËÛË Ù˘ ‰ÈÓ·ÙÚÈÔ‡¯Ô˘ ·ÌȉÚÔÓ¿Ù˘ ‰ÂÓ ·Ó¤-
ÛÙÂÈÏ ÙË Ê˘ÛÈ΋ ÔÚ›· Ù˘ ÓfiÛÔ˘ Û¯ÂÙÈο ÌÂ
ÙÔ ª™ ÙÔ˘ ·È‰ÈÔ‡, ·Ó Î·È Ô ·ÚÈıÌfi˜ ÙˆÓ Î·-
Ù·ÁÌ¿ÙˆÓ ÌÂÈÒıËÎÂ Î·È ÙÔ ‡„Ô˜ ÙˆÓ ÛÔÓ‰˘-
ÏÈÎÒÓ ÛˆÌ¿ÙˆÓ ·˘Í‹ıËÎÂ. ∏ ıÂڷ›· Ù˘ Ófi-
ÛÔ˘ Â›Ó·È Û˘Ìو̷ÙÈ΋, Ì Ì›ˆÛË Ù˘ ÔÛÙÂ-
ÔfiÚˆÛ˘ Î·È Ù˘ Û˘¯ÓfiÙËÙ·˜ ÙˆÓ Î·Ù·ÁÌ¿-
ÙˆÓ, ·Ó·ÎÔ‡ÊÈÛË ·fi ÙÔ ¯ÚfiÓÈÔ ¿ÏÁÔ˜ Î·È ÙËÓ
ÎfiˆÛË, ‚ÂÏÙ›ˆÛË Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜ Î·È Úfi-
ÏË„Ë ÙˆÓ ·Ú·ÌÔÚÊÒÛÂˆÓ ÙˆÓ Ì·ÎÚÒÓ ÔÛÙÒÓ
Î·È ÛÔÓ‰‡ÏˆÓ (2,3,5).
∞ӷʤÚÂÙ·È ·‡ÍËÛË ÙÔ˘ µ™ ηٿ ÙË ‰È¿Ú-
ÎÂÈ· Ù˘ ıÂڷ›·˜ (5). ™ÙËÓ ÂÚ›ÙˆÛË ÙÔ˘
‚Ú¤ÊÔ˘˜ ‰ÂÓ ·˘Í‹ıËΠ˘ÂÚ‚ÔÏÈο ÙÔ µ™. ™Â
ËÏÈΛ· 8 ÌËÓÒÓ ÙÔ µ™ ‚Ú¤ıËΠ7560 g (3Ë-10Ë
∂£) Î·È Û ËÏÈΛ· 26 ÌËÓÒÓ ÙÔ µ™ ‹Ù·Ó 10.500 g
(<3Ë ∂£).
√ÚıԷȉÈÎÔ› ·Ó·Ê¤ÚÔ˘Ó Ï¢Τ˜ ÂÚÈÔ¯¤˜
ÛÙȘ ·ÎÙÈÓÔÁڷʛ˜ ÙˆÓ ÂÈʇÛÂˆÓ ÙˆÓ Ì·-
ÎÚÒÓ ÔÛÙÒÓ ÌÂÙ¿ ·fi ıÂڷ›· Ì ‰ÈʈÛÊÔÓÈ-
ο (7). ¶·ÚfiÌÔȘ ÏÂ˘Îˆ¤˜ ˙ÒÓ˜ ·Ú·ÙËÚ‹-
ıËÎ·Ó Î·È ÛÙËÓ ·/· ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ÌËÚÈ·›Ô˘
ÔÛÙÔ‡ ÙÔ˘ Ó˛Ԣ Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È (∂ÈÎfiÓ·
4), fiˆ˜ ›Û˘ ¤¯Ô˘Ó ·Ú·ÙËÚËı› Û ·È‰È¿
Ì ڷ¯›Ùȉ· ·ÓıÂÎÙÈ΋ ÛÙË ‚ÈÙ·Ì›ÓË D. ∞˘Ù‹ Ë
ÂÈÎfiÓ· ıˆÚÂ›Ù·È fiÙÈ ‰ËÏÒÓÂÈ ‚ÂÏÙ›ˆÛË Ù˘
¶·È‰È·ÙÚÈ΋ 2006;69:452-457
∂ÈÎfiÓ· 4. ∏ ·/· ·ÚÈÛÙÂÚÔ‡ ÌËÚÈ·›Ô˘ ÔÛÙÔ‡, ÌÂÙ¿ ·fi 16Ì‹Ó˜ ıÂڷ›·˜, ‰Â›¯ÓÂÈ Î‡ÚÙˆÛË ÙÔ˘ ÔÛÙÔ‡ Î·È Ï¢Τ˜˙ÒÓ˜ ÛÙËÓ Â›Ê˘ÛË.
457∞ÙÂÏ‹˜ ÔÛÙÂÔÁ¤ÓÂÛË Î·È ·ÌȉÚÔÓ¿ÙË
¶·È‰È·ÙÚÈ΋ 2006;69:452-457
·Û‚ÂÛÙÔÔ›ËÛ˘ ÙÔ˘ ÔÛÙÔ‡. ™˘ÛÙ‹ıËΠËÂÊ·ÚÌÔÁ‹ ÂÓ‰ÔÌ˘ÂÏÈÎÔ‡ ‹ÏÔ˘ ÛÙ· ÌËÚÈ·›·ÔÛÙ¿. ∂Ú¢ÓËÙ¤˜ Û˘ÓÈÛÙÔ‡Ó ÙËÓ ÙÔÔı¤ÙËÛËÂÓ‰ÔÌ˘ÂÏÈÎÔ‡ ‹ÏÔ˘ ÛÙÔ ÌËÚÈ·›Ô ÔÛÙfi Î·È ÙËÓÎÓ‹ÌË, ÁÈ· ÙË ‚ÂÏÙ›ˆÛË Ù˘ ‚¿‰ÈÛ˘ Î·È Ù˘ÈÛÔÚÚÔ›·˜ (5,6).
∏ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ¯ÔÚ‹ÁËÛË ‰ÈʈÛÊÔÓÈ-ÎÒÓ Û ·È‰È¿ ËÏÈΛ·˜ 6-15 ÂÙÒÓ ·Ó·Ê¤ÚÂÙ·È·fi ÔÚıԷȉÈÎÔ‡˜ ·fi ÙËÓ ∫ÔÚ¤·, Ì ÈηÓÔ-ÔÈËÙÈο ·ÔÙÂϤÛÌ·Ù· ˆ˜ ÚÔ˜ ÙËÓ ÔÛÙÂÔ-fiÚˆÛË Î·È Ù· ηٿÁÌ·Ù· (7). ™ÙËÓ ∂ÏÏ¿‰·,¯ÔÚËÁ‹ıËÎ·Ó ‰ÈʈÛÊÔÓÈο 30 mg ‚‰ÔÌ·‰È·›-ˆ˜ Û ‰‡Ô ÎÔÚ›ÙÛÈ· Ì ∞√ ÁÈ· 15 Ì‹Ó˜ Î·È ·-Ú·ÙËÚ‹ıËΠ·‡ÍËÛË Ù˘ ÔÛÙÈ΋˜ Ì¿˙·˜, ¯ˆÚ›˜Ó¤Ô οٷÁÌ· Û ‰È¿ÛÙËÌ· 4 ÂÙÒÓ.
™‡Ìʈӷ Ì ÂÚ¢ÓËÙ¤˜ (3,5,8), ˘¿Ú¯Ô˘ÓοÔÈ· ·Ó·¿ÓÙËÙ· ÂÚˆÙ‹Ì·Ù· Û¯ÂÙÈο Ì Ù˯ڋÛË ÙˆÓ ‰ÈʈÛÊÔÓÈÎÒÓ, Ù· ÔÔ›· ΢ڛˆ˜·ÊÔÚÔ‡Ó ÛÙ· ·ÎfiÏÔ˘ı·: ·) ∫·ıËÏÒÓÔÓÙ·È ÛÙ·ÔÛÙ¿ ÁÈ· ‰ÂηÂٛ˜ Î·È ‰ÂÓ Â›Ó·È ÁÓˆÛÙ‹ Ë Â›-ÙˆÛ‹ ÙÔ˘˜ ·fi ÙÔÓ ÌËÙÚÈÎfi ÛÎÂÏÂÙfi ÛÙÔ ¤Ì-‚Ú˘Ô. ‚) ∏ ¯Ú‹ÛË ÙÔ˘˜ ÌÔÚ› Ó· Â›Ó·È ‰˘ÛÌÂ-Ó‹˜ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜, ‰ÈfiÙÈ ÚÔηÏÔ‡Ó Ì›ˆÛËÙÔ˘ ·Ó·Û¯ËÌ·ÙÈÛÌÔ‡ ÙÔ˘ ÔÛÙÔ‡ (remodeling).Á) ¢ÂÓ Â›Ó·È ÁÓˆÛÙfi˜ Ô ¯ÚfiÓÔ˜ Ô˘ ı· ‰È·ÚΤ-ÛÂÈ Ë ‚ÂÏÙ›ˆÛË, ·ÏÏ¿ Î·È ÔÈ ÂÈÙÒÛÂȘ. ‰) ™Ù··È‰È¿ Ì ÙËÓ ‹È· ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ ‰ÂÓ Û˘ÓÈ-ÛÙ¿Ù·È ıÂڷ›·.
¶ÚÔ˜ ÙÔ ·ÚfiÓ, Ë ·ÔÙÂÏÂÛÌ·ÙÈ΋ Û˘ÌÙˆ-Ì·ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÛÙȘ ̤ÙÚȘ Î·È ÛÔ‚·Ú¤˜ÌÔÚʤ˜ Ù˘ ∞√ Â›Ó·È Ë ¯ÔÚ‹ÁËÛË ÙˆÓ ‰Èʈ-ÛÊÔÓÈÎÒÓ. ªÂ Ù· ‰ÈʈÛÊÔÓÈο ‰ÂÓ ·Ú·ÙËÚ‹-ıËΠ¢ÓÔ˚΋ ›‰Ú·ÛË ÛÙÔ ª™ ηÈ, ÂÔ̤ӈ˜,ÛÙÔ ÁÂÓÂÙÈÎfi ˘fiÛÙڈ̷ Ù˘ ÓfiÛÔ˘, ÂÓÒ ·Ú·-̤ÓÂÈ ¿ÁÓˆÛÙË Ë Ì·ÎÚÔ¯ÚfiÓÈ· ¤Î‚·ÛË Ù˘ ¯Ô-Ú‹ÁËÛ‹˜ ÙÔ˘˜.
∂˘¯·ÚÈÛٛ˜
∂˘¯·ÚÈÛÙԇ̠ıÂÚÌ¿ ÙÔÓ ∫·ıËÁËÙ‹ Î. N. BishopÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ Sheffield ÙÔ˘ ∏ӈ̤ÓÔ˘ µ·ÛÈÏ›-Ô˘, Ô˘ ÚÔ¤ÙÚ„ ÙÔ˘˜ ÁÔÓ›˜ ÙÔ˘ Ó˛Ԣ Ó· ·ÓÙÈÌÂ-Ùˆ›ÛÔ˘Ó ÙË ÓfiÛÔ ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘˜ ÛÙËÓ ∂ÏÏ¿‰·.
µÈ‚ÏÈÔÁÚ·Ê›·
1. Glorieux FH. A disease of the osteoblast. Lancet2001;358 (Suppl): S45.
2. Landsmeer-Beker EA, Massa GG, Maaswinkel-MooyPD, van de Kamp JJ, Papapoulos SE. Treatment of os-teogenesis imperfecta with the bisphosphonate ol-padronate (dimethylaminohydroxypropylidene bis-phosphonate). Eur J Pediatr 1997;156:792-794.
3. Plotkin H, Rauch F, Bishop NJ, Montpetit K, Ruck-Gibis J, Travers R et al. Pamidronate treatment of se-vere osteogenesis imperfecta in children under 3 yearsof age. J Clin Endocrinol Metab 2000;85:1846-1850.
4. ∫ÒÛÙ·ÏÔ˜ Ã, √ÈÎÔÓÔÌ›‰Ô˘ √. ÃÔÚ‹ÁËÛË ‰ÈÓ·ÙÚÈ-Ô‡¯Ô˘ ·ÌȉÚÔÓ¿Ù˘ Û ÓÂÔÁÓfi. 38Ô ¶·ÓÂÏÏ‹ÓÈÔ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ; ∫ˆ˜: 16-18 πÔ˘Ó›Ô˘ 2000.
5. Rauch F, Glorieux FH. Osteogenesis imperfecta.Lancet 2004;363:1377-1385.
6. µÔÛοÎË ∂, ∞ı·Ó·ÛÔÔ‡ÏÔ˘ ∂, °ÂˆÚÁ·ÎÔÔ‡ÏÔ˘ ∞.£Âڷ¢ÙÈο ·ÔÙÂϤÛÌ·Ù· Ù˘ ·ÏÏÂÓ‰ÚÔÓ¿Ù˘ Û·ȉȿ Ì ·ÙÂÏ‹ ÔÛÙÂÔÁ¤ÓÂÛË. 43Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È-‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ; ∫ˆ˜: 17-19 πÔ˘Ó›Ô˘ 2005.
7. Cho TJ, Choi IH, Chung CY, Yoo WJ, Park MS, ParkYK. Efficacy of oral alendronate in children with os-teogenesis imperfecta. J Pediatr Orthop 2005;25: 607-612.
8. Marini JC. Do bisphosphonates make children’s bonesbetter or brittle? N Engl J Med 2003;349:423-426.
458 ∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏ CASE REPORT
¶·È‰È·ÙÚÈ΋ 2006;69:458-462
¶·Ú·ÛÔÓ‰˘ÏÈÎfi Â͈ÔÛÙÈÎfi Û¿ÚΈ̷
ÙÔ˘ Ewing: ÂÚÈÁÚ·Ê‹ ‰‡Ô ÂÚÈÙÒÛˆÓ
º. ∞ı·Ó·ÛÈ¿‰Ô˘1, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜1, ª. ∫Ô‡ÚÙË1, £. ¶··ÁˆÚÁ›Ô˘1, ¶. Ã˘Ù›ÚÔÁÏÔ˘2, µ. ∫ÔÓÙfiÔ˘ÏÔ˜3,
∞. ∫·ÏÔÁÂÚ¿4, ∞. ¢Ú‚ÂϤÁη˜4
¶ÂÚ›ÏË„Ë: ∆Ô Â͈ÔÛÙÈÎfi Û¿ÚΈ̷ ÙÔ˘ Ewing (Extraskeletal Ewing’s Sarcoma, EES) Â›Ó·È Û¿ÓÈÔ,˘„ËÏ‹˜ ηÎÔ‹ıÂÈ·˜ ÓÂfiÏ·ÛÌ· ÙˆÓ ÔÛÙÒÓ Ì ÈÛÙÔÏÔÁÈÎÔ‡˜ ¯·Ú·ÎÙ‹Ú˜ ·Ó¿ÏÔÁÔ˘˜ Ì ÂΛÓÔ˘˜ÙÔ˘ Û·ÚÎÒÌ·ÙÔ˜ ÙÔ˘ Ewing (Ewing’s Sarcoma, EWS). ∆Ô EES ··ÓÙ¿Ù·È Î˘Ú›ˆ˜ Û ÂÊ‹‚Ô˘˜ Î·È ÓÂ-·ÚÔ‡˜ ÂÓ‹ÏÈΘ Î·È ÂÍÔÚÌ¿Ù·È Û˘¯ÓfiÙÂÚ· ·fi ÙËÓ ·Ú·ÛÔÓ‰˘ÏÈ΋ ÂÚÈÔ¯‹ Î·È Ù· οو ¿ÎÚ·.¶ÂÚÈÁÚ¿ÊÔÓÙ·È 2 ÂÚÈÙÒÛÂȘ EES Ì ÂÓÙfiÈÛË ÙÔ˘ fiÁÎÔ˘ ·Ú·ÛÔÓ‰˘ÏÈο, Û ·È‰È¿ ËÏÈΛ·˜ 14Î·È 10 ÂÙÒÓ, ·ÓÙ›ÛÙÔȯ·. √È 2 ·ÛıÂÓ›˜ ·Ó¤ÊÂÚ·Ó Ú·¯È·ÏÁ›· Î·È Ï¢ÚÔ‰˘Ó›· Î·È Ë ‰È¿ÁÓˆÛË ‚·Û›-ÛÙËΠÛÙ· Â˘Ú‹Ì·Ù· ÙÔ˘ ·ÂÈÎÔÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘ Î·È Ù˘ ÈÛÙÔÏÔÁÈ΋˜/·ÓÔÛÔ˚ÛÙÔ¯ËÌÈ΋˜ ·Ó¿Ï˘-Û˘ Ô˘ ·ÔÎ¿Ï˘„ ÙËÓ ‡·ÚÍË Î·ÎÔ‹ıÔ˘˜ ÓÂÔÏ¿ÛÌ·ÙÔ˜ ·fi ÌÈÎÚ¿ ÛÙÚÔÁÁ˘Ï¿, ÌÏ ·ÙÙ·Ú·,Ì ¯·Ú·ÎÙ‹Ú˜ Û˘Ì‚·ÙÔ‡˜ Ì ۿÚΈ̷ Ewing/PNET. ∏ ÌÔÚȷ΋ ‰ÈÂÚ‡ÓËÛË Ô˘ ·ÎÔÏÔ‡ıËÛ ÌÂRT-PCR ·ÔÎ¿Ï˘„ ÙËÓ ·ÚÔ˘Û›· ˘‚ÚȉÈÎÒÓ ÌÂÙ·ÁÚ·ÊËÌ¿ÙˆÓ EWS-FLI-1 [t(11;22) (q24;q12)]. ∏ÌÂÙ¿ıÂÛË ·˘Ù‹ ÙÂÎÌËÚ›ˆÛ ÙË ‰È¿ÁÓˆÛË ÙÔ˘ EES Î·È ÛÙÔ˘˜ 2 ·ÛıÂÓ›˜, ÔÈ ÔÔ›ÔÈ ˘Ô‚Ï‹ıËÎ·Ó Û¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙÔ˘ fiÁÎÔ˘ Î·È ÛÙË Û˘Ó¤¯ÂÈ· Û ıÂڷ›· (¯ËÌÂÈÔ-, ·ÎÙÈÓÔıÂڷ›·) Ì ÂÈ-‰ÈÎfi ÚˆÙfiÎÔÏÏÔ ÁÈ· Û¿ÚΈ̷ ÙÔ˘ Ewing (IESS NCI) Û˘ÓÔÏÈ΋˜ ‰È¿ÚÎÂÈ·˜ 51 ‚‰ÔÌ¿‰ˆÓ ¯ˆÚ›˜˘ÔÙÚÔ‹ Ù˘ ÓfiÛÔ˘ ‹ ¿ÏϘ ÂÈÏÔΤ˜.
§¤ÍÂȘ ÎÏÂȉȿ: ∂͈ÔÛÙÈÎfi Û¿ÚΈ̷ ÙÔ˘ Ewing, ·Ú·ÛÔÓ‰˘ÏÈ΋ ÂÚÈÔ¯‹, Ú·¯È·ÏÁ›·.
Extraskeletal Ewing’s sarcoma: report of 2 cases
F. Athanassiadou1, A. Tragiannidis1, M. Kourti1, T. Papageorgiou1, P. Chytiroglou2, V. Kontopoulos3,
A. Kalogera4, A. Drevelegas4
Abstract: Extraskeletal Ewing’s sarcoma (EES) is a rare soft tissue malignant neoplasm histologicallysimilar to skeletal Ewing’s sarcoma (EWS). EES occurs mainly in adolescents and young adults andcommonly affects the paravertebral regions and the lower limbs. Two cases of EES in 14 and 10 year-oldchildren are reported, involving the paravertebral spaces. The diagnosis of EES was based on the clinicalsymptoms (back and chest pain) and radiological findings and on the histologic/immunohistochemistryfeatures of a malignant tumour with small, blue, round cells indicative of Ewing’s/PNET. The diagnosisof EES was confirmed by RT-PCR, which showed the presence of the EWS/FLI-1 fusion transcript[t(11;22) (q24;q12) chromosomal translocation]. Both patients were treated by surgical resection of themass and subsequent chemo- and radiotherapy (IESS NCI protocol) for a total of 51 weeks, withoutrelapse or other complications.
Key words: Extraskeletal Ewing’s sarcoma, paravertebral region, back pain.
1 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞.¶.£, ¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞
2 ¶·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎfi∂ÚÁ·ÛÙ‹ÚÈÔ ∞.¶.£.,¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞
3 ¡Â˘ÚÔ¯ÂÈÚÔ˘ÚÁÈ΋ ∫ÏÈÓÈ΋∞.¶.£., ¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞
4 ∞ÎÙÈÓÔÏÔÁÈÎfi ∂ÚÁ·ÛÙ‹ÚÈÔ∞.¶.£., ¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞
AÏÏËÏÔÁÚ·Ê›·:
º·Ó‹ ∞ı·Ó·ÛÈ¿‰Ô˘-¶ÈÂÚÔÔ‡ÏÔ˘,ªËÙÚÔfiψ˜ 55, ∆.∫. 54623, £ÂÛÛ·ÏÔÓ›ÎËE-mail: [email protected]@auth.gr
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 31-05-2005∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 05-09-2006
1 2nd Paediatric Clinic,Aristotle University ofThessaloniki, UniversityHospital AHEPA
2 Pathology Laboratory,Aristotle University ofThessaloniki, UniversityHospital AHEPA
3 Neurosurgery Clinic,Aristotle University ofThessaloniki, UniversityHospital AHEPA
4 Radiology Laboratory,Aristotle University ofThessaloniki, UniversityHospital AHEPA
Correspondence:
Fani Athanassiadou-Piperopoulou55, Mitropoleos St. 54623, ThessalonikiE-mail: [email protected]@auth.gr
Date of submission: 31-05-2005Date of approval: 05-09-2006
∂ÈÛ·ÁˆÁ‹
∆Ô Û¿ÚΈ̷ ÙÔ˘ ∂wing (EWS) Â›Ó·È Û¿ÓÈÔ,˘„ËÏ‹˜ ηÎÔ‹ıÂÈ·˜ ÓÂfiÏ·ÛÌ· ÙˆÓ ÔÛÙÒÓ.¶·ÚfiÙÈ ÙÔ EWS Â›Ó·È ¤Ó·˜ ·‰È·ÊÔÚÔÔ›ËÙÔ˜fiÁÎÔ˜ ÙˆÓ ÔÛÙÒÓ, Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÂÍÔÚÌ¿Ù·È·fi Ù· ̷Ϸο ÌfiÚÈ· (Â͈ÔÛÙÈÎfi Û¿ÚΈ̷ ÙÔ˘Ewing) (1-3). ∂ÈϤÔÓ, ÌÈ· ϤÔÓ ‰È·ÊÔÚÔ-ÔÈË̤ÓË ÌÔÚÊ‹ ÙÔ˘ fiÁÎÔ˘, ·ÔÙÂÏ› ÙÔ Ó¢-
ÚÔÂÈıËÏ›ˆÌ· ‹ ÂÚÈÊÂÚÈÎfi˜ ÚˆÙÔ·ı‹˜
Ó¢ÚÔ-ÂÈıËÏÈ·Îfi˜ fiÁÎÔ˜ (ƒ¡∂∆), Ô˘ ÌÔÚ›
Ó· ÂÍÔÚÌ¿Ù·È Â›Ù ·fi Ù· ÔÛÙ¿ ›Ù ·fi Ù· Ì·-
Ϸο ÌfiÚÈ·. ŸÏ˜ ·˘Ù¤˜ ÔÈ ÌÔÚʤ˜ ÂÌÊ·Ó›˙Ô˘Ó
·ÚfiÌÔÈ· ÈÛÙÔ¯ËÌÈο Î·È ‚ÈÔÏÔÁÈο ¯·Ú·ÎÙË-
ÚÈÛÙÈο Î·È ÂÔ̤ӈ˜ ηٷٿÛÛÔÓÙ·È Û ÌÈ·
ÂÓÈ·›· ÔÌ¿‰· fiÁΈÓ, ÙËÓ ÔÈÎÔÁ¤ÓÂÈ· ÙˆÓ Û·Ú-
ÎˆÌ¿ÙˆÓ ÙÔ˘ Ewing (Ewing’s Sarcoma family
tumors, ESFT). ™¯ÂÙÈο Ì ÙËÓ ÚԤϢÛË ÙÔ˘
459¶·Ú·ÛÔÓ‰˘ÏÈÎfi Â͈ÔÛÙÈÎfi Û¿ÚΈ̷ ÙÔ˘ Ewing
fiÁÎÔ˘ ÂÈÎÚ·Ù› Ë ¿Ô„Ë fiÙÈ ÙÔ ‚·ÛÈÎfi ·ÙÙ·-
ÚÔ Â›Ó·È ¤Ó· ·Ú¯¤ÁÔÓÔ ÔÏ˘‰‡Ó·ÌÔ Ó¢ÚÈÎfi
·ÙÙ·ÚÔ, Ô˘ ÚÔ¤Ú¯ÂÙ·È ·fi ÙÔ ·Ú·Û˘Ì·-
ıËÙÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ·, ÂÚÌËÓ‡ÔÓÙ·˜ ¤ÙÛÈ
ÙËÓ ÔÈÎÈÏ›· ÂÓÙfiÈÛ˘ ÙˆÓ fiÁÎˆÓ Ù˘ ηÙËÁÔ-
Ú›·˜ ·˘Ù‹˜. ™˘¯ÓfiÙÂÚ˜ ı¤ÛÂȘ ÂÓÙfiÈÛ˘ ÙÔ˘
EES Â›Ó·È Ô ıÒڷη˜, Ù· οو ¿ÎÚ· Î·È Ë ·-
Ú·ÛÔÓ‰˘ÏÈ΋ ÂÚÈÔ¯‹ Î·È ··ÓÙ¿Ù·È Û˘¯Ófi-
ÙÂÚ· Û Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ (<30 ÂÙÒÓ) (1,4).
¶ÂÚ›ÙˆÛË ÚÒÙË
∞ÁfiÚÈ ËÏÈΛ·˜ 14 ÂÙÒÓ ‰È·ÎÔÌ›ÛÙËΠÛÙËÓ
ÎÏÈÓÈ΋ Ì·˜ ·fi √ÚıԷȉÈ΋ ∫ÏÈÓÈ΋ ·ÎÈÓËÙÔ-
ÔÈË̤ÓÔ Û Á‡„ÈÓÔ Ó¿ÚıËη, ÏfiÁˆ ÔÛÙÂÔÏ˘ÙÈ-
΋˜ ÂÍÂÚÁ·Û›·˜ ÙÔ˘ £-9 ÛÔÓ‰‡ÏÔ˘ Ô˘ ·Ô-
ηχÊıËΠ̠·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· (∂ÈÎfiÓ· 1).
∞fi ÙÔ ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È Ú·¯È·ÏÁ›· ·fi ‰È-
Ì‹ÓÔ˘ Ì ›ٷÛË ÙÔ˘ ¿ÏÁÔ˘˜ ηٿ ÙËÓ Î¿Ì„Ë-
¤ÎÙ·ÛË ÙÔ˘ ÎÔÚÌÔ‡. ∏ Ó¢ÚÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË
Î·È Ë ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË Î·Ù¿ Û˘ÛÙ‹Ì·Ù·
‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. √ Ú·‰ÈÔ˚ÛÔÙÔÈÎfi˜ ¤ÏÂÁ¯Ô˜
Ì ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÙˆÓ ÔÛÙÒÓ ·ÔÎ¿Ï˘„Â
ÙËÓ ‡·ÚÍË ÂÚÈÔ¯‹˜ ·˘ÍË̤Ó˘ ÛÈÓıËÚÔÁÚ·-
ÊÈ΋˜ ·ÓÙ›ıÂÛ˘ ÛÙÔ ‡„Ô˜ ÙÔ˘ £-9, ‡ÚËÌ· Ô˘
·Ô‰fiıËΠ۠οٷÁÌ· ÙÔ˘ ÛÔÓ‰‡ÏÔ˘, ÂÓÒ
ÛÙ· ˘fiÏÔÈ· ÔÛÙ¿ Ë Î·ı‹ÏˆÛË ÙÔ˘ Ú·‰ÈÔÊ·Ú-
Ì¿ÎÔ˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. √ ¤ÏÂÁ¯Ô˜ ÔÏÔÎÏË-
ÚÒıËΠ̠CT Î·È MRI Ù˘ ıˆÚ·ÎÈ΋˜ ÌÔ›Ú·˜
Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ Ô˘ ·ÔÎ¿Ï˘„·Ó ÂÈ-
ÎfiÓ· ·Ô‰ÈÔÚÁ¿ÓˆÛ˘ Ì ‰È¿Û·ÛË ÙÔ˘ ÊÏÔÈÔ‡
Î·È Î·ı›˙ËÛË ÙÔ˘ ÛÒÌ·ÙÔ˜ ÙÔ˘ £-9 Î·È ÙËÓ
‡·ÚÍË ·Ú·ÛÔÓ‰˘ÏÈ΋˜ Â͈ÔÛÙÈ΋˜ ¯ˆÚÔ-
ηٷÎÙËÙÈ΋˜ ÂÍÂÚÁ·Û›·˜ ‰ÂÍÈ¿, Ì ¤ÎÙ·ÛË
ÛÙÔÓ Û‡ÛÙÔÈ¯Ô ÂÈÛÎÏËÚ›‰ÈÔ ¯ÒÚÔ Ô˘ Ȥ˙ÂÈ,
·ÏÏ¿ ‰ÂÓ ‰ÈËı› ÙÔ ÛÒÌ· ÙÔ˘ ÛÔÓ‰‡ÏÔ˘ (∂ÈÎfi-
Ó· 2). √ ·ÛıÂÓ‹˜ ˘Ô‚Ï‹ıËΠ۠ηÙ¢ı˘ÓfiÌÂ-
ÓË ‚ÈÔ„›· Ì ‚ÂÏfiÓË ·fi ÙËÓ ·Ú·ÛÔÓ‰˘ÏÈ΋
Ì¿˙· Ô˘ ·ÔÎ¿Ï˘„ ÙËÓ ‡·ÚÍË Î·ÎÔ‹ıÔ˘˜
ÓÂÔÏ¿ÛÌ·ÙÔ˜ ·fi ÌÈÎÚ¿ ÛÙÚÔÁÁ˘Ï¿, ÌÏÂ
·ÙÙ·Ú·, ¯·Ú·ÎÙ‹Ú˜ Û˘Ì‚·ÙÔ› Ì ۿÚΈ̷Ewing/PNET. ∞ÎÔÏÔ‡ıËÛÂ Û˘ÌÏËڈ̷ÙÈÎfifiÚÈÛÌ· ÌÔÚȷ΋˜ ‰ÈÂÚ‡ÓËÛ˘ fiÔ˘ Ì RT-PCR ¤ÁÈÓ ¤ÏÂÁ¯Ô˜ ÁÈ· ÙËÓ ‡·ÚÍË ˘‚ÚȉÈÎÒÓÌÂÙ·ÁÚ·ÊÒÓ EWS-FLI-1 [t(11;22) (q24;q12)].∆Ô ˘ÏÈÎfi ‹Ù·Ó ıÂÙÈÎfi ÁÈ· ÙË ÌÂÙ¿ıÂÛË ÙÂÎÌË-ÚÈÒÓÔÓÙ·˜ ÙË ‰È¿ÁÓˆÛË ÙÔ˘ Â͈ÔÛÙÈÎÔ‡ Û·Ú-ÎÒÌ·ÙÔ˜ ÙÔ˘ Ewing. ∂ÈϤÔÓ, Ô ÚÔ‚ÏÂfiÌÂ-ÓÔ˜ ‰È·ÁÓˆÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ô˘ ‰ÈÂÓÂÚÁ‹ıËÎÂÁÈ· ÙËÓ ‡·ÚÍË ÌÂÙ·ÛÙ¿ÛÂˆÓ ·¤‚Ë ·ÚÓËÙÈÎfi˜.√ ·ÛıÂÓ‹˜ ˘Ô‚Ï‹ıËΠ۠¯ËÌÂÈÔıÂڷ›· ÌÂÂȉÈÎfi ÚˆÙfiÎÔÏÏÔ ÁÈ· Û¿ÚΈ̷ ÙÔ˘ Ewing(IESS NCI; Intergroup Ewing’s Sarcoma Study,National Cancer Institute protocol) Ô˘ ÂÚÈ-Ï·Ì‚¿ÓÂÈ ÈʈÛÊ·Ì›‰Ë, ÂÙÔÔÛ›‰Ë, ‚ÈÓÎÚÈÛÙ›ÓË,‰ÔÍÔÚÔ˘‚ÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë Î·È ·‰ÚÈ·Ì˘-ΛÓË (5). ¢‡Ô Ì‹Ó˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ¯ËÌÂÈ-ÔıÂڷ›·˜ Ô ·ÛıÂÓ‹˜ ˘Ô‚Ï‹ıËΠ۠ۈ̷ÙÂ-ÎÙÔÌ‹ £-9, ·Ê·›ÚÂÛË Ù˘ ˘ÔÏÂÈÌÌ·ÙÈ΋˜ÂÍÂÚÁ·Û›·˜ Î·È ÛÔÓ‰˘ÏÔ‰ÂÛ›· £8-£10 Ì ÙÔ-Ôı¤ÙËÛË Îψ‚Ô‡ ÙÈÙ·Ó›Ô˘ Î·È ‚ȉÒÓ ÛÙ· ÛÒ-Ì·Ù· £8-£10 Ì ·ÚÂÌ‚ÔÏ‹ Ú¿‚‰Ô˘ Ì ‰ÂÍÈ¿ıˆÚ·ÎÔÙÔÌ‹ (∂ÈÎfiÓ· 3). ∏ ÌÂÙÂÁ¯ÂÈÚËÙÈ΋ ÙÔ˘ÔÚ›· ‹Ù·Ó ÔÌ·Ï‹. √ ·ÛıÂÓ‹˜ Û˘Ó¤¯ÈÛ ÙÔ¯ËÌÂÈÔıÂڷ¢ÙÈÎfi ÚˆÙfiÎÔÏÏÔ Î·È ‰‡Ô Ì‹-Ó˜ ·ÚÁfiÙÂÚ· ˘Ô‚Ï‹ıËΠ۠·ÎÙÈÓÔıÂڷ›·ÁÈ· ‰È¿ÛÙËÌ· ÂÓfi˜ Ì‹Ó· ÂÚ›Ô˘, fiÔ˘ ¤Ï·‚ÂÛÙËÓ ÂÚÈÔ¯‹ £8-£10 ‰fiÛË 42Gy Û 23 Û˘ÓÂ-‰Ú›Â˜. ŒÎÙÔÙÂ, Ô ·ÛıÂÓ‹˜ ‚Ú›ÛÎÂÙ·È Û ¿ÚÈÛÙËÎÏÈÓÈ΋ ηٿÛÙ·ÛË Î·È ÔÏÔÎÏ‹ÚˆÛ ÙÔ ¯ËÌÂÈ-ÔıÂڷ¢ÙÈÎfi Û¯‹Ì· Û˘ÓÔÏÈ΋˜ ‰È¿ÚÎÂÈ·˜ 51‚‰ÔÌ¿‰ˆÓ ¯ˆÚ›˜ ˘ÔÙÚÔ‹ Ù˘ ÓfiÛÔ˘ ‹ ¿Ï-Ϙ ÂÈÏÔΤ˜.
¶ÂÚ›ÙˆÛË ‰Â‡ÙÂÚË
∫ÔÚ›ÙÛÈ ËÏÈΛ·˜ 10 ÂÙÒÓ ÂÈÛ‹¯ıË ÛÙËÓ ÎÏÈÓÈ-΋ Ì·˜ ÏfiÁˆ Ï¢ÚÔ‰˘Ó›·˜ Î·È ÔÛÊ˘·ÏÁ›·˜ ·fi20Ë̤ÚÔ˘ ΢ڛˆ˜ ηٿ ÙË Ó˘¯ÙÂÚÈÓ‹ ηٿÎÏÈÛË.
¶·È‰È·ÙÚÈ΋ 2006;69:458-462
∂ÈÎfiÓ· 1. ∞ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜. √ÛÙÂÔÏ˘ÙÈ΋ ÂÍÂÚÁ·Û›·ÛÔÓ‰‡ÏÔ˘ £-9.
∂ÈÎfiÓ· 2. ª·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ıˆÚ·ÎÈ΋˜ ÌÔ›Ú·˜ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘. ⁄·ÚÍË ·Ú·ÛÔÓ‰˘ÏÈ΋˜ Â͈-ÛÎÏËÚ›‰È·˜ ¯ˆÚÔηٷÎÙËÙÈ΋˜ ÂÍÂÚÁ·Û›·˜ Ì ¤ÎÙ·ÛËÛÙÔÓ ÂÈÛÎÏËÚ›‰ÈÔ ¯ÒÚÔ Î·È ›ÂÛË ÛÙÔ ÓˆÙÈ·›Ô Ì˘ÂÏfi.
460 º. ∞ı·Ó·ÛÈ¿‰Ô˘ Î·È Û˘Ó.
∏ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ·ÔÎ¿Ï˘„ ÙËÓ ·-
ÚÔ˘Û›· ·ÎÙÈÓÔÛÎÈÂÚ‹˜ ÂÚÈÔ¯‹˜ ÛÙÔ Ì¤ÛÔ Î·È
οو Ó¢ÌÔÓÈÎfi ‰›Ô ‰ÂÍÈ¿, ÌÂ Û˘ÓÔ‰fi ‰È¿-
‚ÚˆÛË Ù˘ 7˘ ÏÂ˘Ú¿˜ Î·È ‰È¿‚ÚˆÛË-ÛÊËÓÔ-
Âȉ‹ ·Ú·ÌfiÚʈÛË ÙÔ˘ ÛÒÌ·ÙÔ˜ ÙÔ˘ £-7 ÛÔÓ-
‰‡ÏÔ˘. ¢ÈÂÓÂÚÁ‹ıËΠϋÚ˘ ·ÈÌ·ÙÔÏÔÁÈÎfi˜-
‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ô˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜,
ÂÓÒ ÔÏÔÎÏËÚÒıËÎÂ Ô ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜
Ì ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÔÛÙÒÓ, fiÔ˘ ηٷÁÚ¿-
ÊËΠ·˘ÍË̤ÓË ÌÂÙ·‚ÔÏÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·
ÛÙÔ ÛÒÌ· ÙÔ˘ £-7 ÛÔÓ‰‡ÏÔ˘ Î·È ÛÙÔ ÙfiÍÔ Ù˘
7˘ ÏÂ˘Ú¿˜ ‰ÂÍÈ¿. √ ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜
Ì CT ıÒÚ·ÎÔ˜-ÎÔÈÏ›·˜ Î·È MRI ıÒÚ·ÎÔ˜ ·Ô-
Î¿Ï˘„ ÙËÓ ·ÚÔ˘Û›· ¢ÌÂÁ¤ıÔ˘˜ ÚˆÙÔ·-
ıÔ‡˜ ‰ÈËıËÙÈ΋˜ ÂÍÂÚÁ·Û›·˜ Ì·Ï·ÎÒÓ ÌÔÚ›ˆÓ
‰ÂÍÈÔ‡ ËÌÈıˆÚ·Î›Ô˘ Ì ‰È‹ıËÛË Ù˘ 6˘ ηÈ
7˘ ÏÂ˘Ú¿˜ Î·È Â¤ÎÙ·ÛË Ù˘ Ì¿˙·˜ ÂÓ‰Ô-
Ó¢ÌÔÓÈο, ‰È‹ıËÛË ÙÔ˘ £-6 ÛÔÓ‰‡ÏÔ˘ ηÈ
¤ÎÙ·ÛË ÂÓ‰ÔηӷÏÈο ÛÙÔÓ Ô›ÛıÈÔ ÂÈ-
ÛÎÏËÚ›‰ÈÔ ¯ÒÚÔ (£5-£8) (∂ÈÎfiÓ· 4). ∏ ·ÛıÂ-
Ó‹˜ ˘Ô‚Ï‹ıËΠ۠ηÙ¢ı˘ÓfiÌÂÓË ‚ÈÔ„›· ÌÂ
‚ÂÏfiÓË ·fi ÙËÓ ·Ú·ÛÔÓ‰˘ÏÈ΋ Ì¿˙· Ô˘
·ÔÎ¿Ï˘„ ÙËÓ ‡·ÚÍË Î·ÎÔ‹ıÔ˘˜ ÓÂÔÏ¿-
ÛÌ·ÙÔ˜ ·fi ÌÈÎÚ¿ ÛÙÚÔÁÁ˘Ï¿, ÌÏ ·ÙÙ·Ú· ÌÂ
¯·Ú·ÎÙ‹Ú˜ Û˘Ì‚·ÙÔ‡˜ Ì ۿÚΈ̷ ÙÔ˘
Ewing/PNET. ∞fi ÙË Ó¢ÚÔÏÔÁÈ΋ ·ÓÙÈÎÂÈÌÂÓÈ-
΋ ÂͤٷÛË Ë ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·˙ ÂÚÈÔÚÈÛÌfi
Ù˘ ·˘ıfiÚÌËÙ˘ ÎÈÓËÙÈÎfiÙËÙ·˜ ÙÔ˘ ‰ÂÍÈÔ‡ ο-
Ùˆ ¿ÎÚÔ˘ (ۯ‰fiÓ Ï‹Ú˘ ·‰˘Ó·Ì›· Ú·¯È·›·˜
ο̄˘ Î·È ·Ó¤ÁÂÚÛ˘ οو ¿ÎÚÔ˘). ªÂÙ¿ ·fi
Ó¢ÚÔ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÎÙ›ÌËÛË ·ÔÊ·Û›ÛÙËΠË
¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙÔ˘ fiÁÎÔ˘ Ì ÛÙfi¯Ô ÙËÓ
·ÔÛ˘Ì›ÂÛË ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡. ªÂÙ¿ ·fi
‰ÂÍÈ¿ ıˆÚ·ÎÔÙÔÌ‹ Ô fiÁÎÔ˜ ÎÚ›ıËΠ·ÓÂÁ¯Â›ÚË-
ÙÔ˜ Î·È ÁÈ· ÙÔÓ ÏfiÁÔ ·˘Ùfi ¤ÁÈÓ ÌfiÓÔ Ï‹„Ë ˘ÏÈ-ÎÔ‡ ÁÈ· ‚ÈÔ„›· Î·È Û˘ÚÚ·Ê‹ Ù˘ ÙÔÌ‹˜. ∆Ô fi-ÚÈÛÌ· Ù˘ ‚ÈÔ„›·˜ ÂȂ‚·›ˆÛ ÙË ‰È¿ÁÓˆÛËÙÔ˘ Â͈ÔÛÙÈÎÔ‡ Û·ÚÎÒÌ·ÙÔ˜ ÙÔ˘ Ewing, Ë ‰ÂÌÔÚȷ΋ ·Ó¿Ï˘ÛË ·ÔÎ¿Ï˘„ ÙË ÌÂÙ¿ıÂÛËEWS-FLI-1 [t(11;22) (q24;q12)]. ∏ ·ÛıÂÓ‹˜ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ¿Ú¯ÈÛ ¯ËÌÂÈÔıÂڷ›· Ì ÂÈ-‰ÈÎfi ÚˆÙfiÎÔÏÏÔ ÁÈ· Û¿ÚΈ̷ ÙÔ˘ Ewing(IESS NCI) (5). ∏ ηٿÛÙ·ÛË Ù˘ ·ÛıÂÓÔ‡˜ ¤Ó·Ì‹Ó· ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ¯ËÌÂÈÔıÂڷ›·˜¤‰ÂÈÍ ۯÂÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜ÙÔ˘ οو ¿ÎÚÔ˘, Ë MRI ÙÔ˘ ıÒÚ·ÎÔ˜ ÂÏ¿ÙÙˆ-ÛË Ù˘ Ì¿˙·˜ ÙÔ˘ fiÁÎÔ˘ Î·È ˘Ô¯ÒÚËÛË Ù˘›ÂÛ˘ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡ ÂÓ‰ÔηӷÏÈο. ∏·ÛıÂÓ‹˜ ˘Ô‚Ï‹ıËΠ3 Ì‹Ó˜ ·ÚÁfiÙÂÚ· Û·ÎÙÈÓÔıÂڷ›· ÌÂ Û˘ÓÔÏÈ΋ ‰fiÛË 40Gy Û‡Ì-ʈӷ Ì ÙÔ ÚˆÙfiÎÔÏÏÔ Î·È ÛÙË Û˘Ó¤¯ÂÈ· ÛÂÂÈÙ˘¯‹ ÔÏÈ΋ ·Ê·›ÚÂÛË ÙÔ˘ fiÁÎÔ˘. ŒÎÙÔÙÂ, Ë·ÛıÂÓ‹˜ ÔÏÔÎÏ‹ÚˆÛ ÙÔ ¯ËÌÂÈÔıÂڷ¢ÙÈÎfiÛ¯‹Ì· Û˘ÓÔÏÈ΋˜ ‰È¿ÚÎÂÈ·˜ 51 ‚‰ÔÌ¿‰ˆÓ¯ˆÚ›˜ ˘ÔÙÚÔ‹ Ù˘ ÓfiÛÔ˘ ‹ ¿ÏϘ ÂÈÏÔΤ˜Î·È ‚Ú›ÛÎÂÙ·È Û ¿ÚÈÛÙË ÎÏÈÓÈ΋ ηٿÛÙ·ÛË.
™˘˙‹ÙËÛË
∆Ô Û¿ÚΈ̷ ÙÔ˘ Ewing ÂÚÈÁÚ¿ÊËΠÚÒ-ÙË ÊÔÚ¿ ·fi ÙÔÓ James Ewing ÙÔ 1921 ˆ˜ ÔÛÙÂ-ÔÏ˘ÙÈ΋ ÂÛÙ›· Ù˘ ÎÂÚΛ‰·˜ Û ÎÔÚ›ÙÛÈ ËÏÈΛ·˜14 ÂÙÒÓ (6). ∏ Â͈ÔÛÙÈ΋ ÌÔÚÊ‹ ÙÔ˘ Û·ÚÎÒÌ·-ÙÔ˜ ÙÔ˘ Ewing ÂÚÈÁÚ¿ÊËΠÚÒÙË ÊÔÚ¿ ÙÔ1969 ·fi ÙÔÓ Tefft Î·È Û˘Ó (7). ∆Ô EES ·ÔÙÂ-Ï› ÂÍ·ÈÚÂÙÈο Û¿ÓÈÔ Î·È ˘„ËÏ‹˜ ηÎÔ‹ıÂÈ·˜fiÁÎÔ Ô˘ ·ÓÙÈÚÔۈ‡ÂÈ ÂÚ›Ô˘ ÙÔ 5% ÙˆÓ
¶·È‰È·ÙÚÈ΋ 2006;69:458-462
∂ÈÎfiÓ· 3. ∞ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ÌÂÙÂÁ¯ÂÈÚËÙÈο. ∞ÂÈ-ÎfiÓÈÛË Îψ‚Ô‡ ÙÈÙ·Ó›Ô˘ ÛÙ· ÛÒÌ·Ù· £8-£10 Ì ·ÚÂÌ-‚ÔÏ‹ Ú¿‚‰Ô˘.
∂ÈÎfiÓ· 4. ª·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ıˆÚ·ÎÈ΋˜ ÌÔ›Ú·˜ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘. ∂˘ÌÂÁ¤ı˘ ‰ÈËıËÙÈ΋ ÂÍÂÚÁ·Û›· Ì·-Ï·ÎÒÓ ÌÔÚ›ˆÓ ‰ÂÍÈÔ‡ ËÌÈıˆÚ·Î›Ô˘ Ì ¤ÎÙ·ÛË ÂÓ‰Ôıˆ-Ú·ÎÈο, ÂÓ‰ÔÛÔÓ‰˘ÏÈο Î·È ÂÓ‰ÔηӷÏÈο ÛÙÔ ‡„Ô˜ ÙˆÓ£-6 Î·È £-7.
461¶·Ú·ÛÔÓ‰˘ÏÈÎfi Â͈ÔÛÙÈÎfi Û¿ÚΈ̷ ÙÔ˘ Ewing
Û·ÚÎˆÌ¿ÙˆÓ ÙˆÓ Ì·Ï·ÎÒÓ ÌÔÚ›ˆÓ Ù˘ ·È‰È-
΋˜ ËÏÈΛ·˜ (8). ™‡Ìʈӷ Ì ÌÈ· ÚfiÛÊ·ÙË ÌÂ-
Ù·-·Ó¿Ï˘ÛË Ù˘ ‰ÈÂıÓÔ‡˜ ‚È‚ÏÈÔÁÚ·Ê›·˜ ¤¯Ô˘Ó
ÂÚÈÁÚ·Ê› 32 ÂÚÈÙÒÛÂȘ Û·ÚΈ̿وÓ
PNET/EES Ì ÂÓÙfiÈÛË ·Ú·ÛÔÓ‰˘ÏÈο (1).
∞fi ÙÔ˘˜ 32 ·ÛıÂÓ›˜ ÔÈ 15 (46,8%) ‹Ù·Ó ËÏÈ-
Λ·˜ 10-20 ÂÙÒÓ, ÂÓÒ Î·Ù·ÁÚ¿ÊËÎÂ Û˘¯ÓfiÙÂÚ·
Û ¿ÚÚÂÓ˜ (22/32, 68,75%). ∏ ÔÛÊ˘·ÏÁ›·, Ë
Ú·¯È·ÏÁ›· Î·È Ë Ï¢ÚÔ‰˘Ó›·, fiˆ˜ ·ÎÚÈ‚Ò˜
·Ó¤ÊÂÚ·Ó Î·È ÔÈ 2 ·ÛıÂÓ›˜ Ì·˜, ·ÔÙÂÏÔ‡Ó Ù·
Û˘¯ÓfiÙÂÚ· Û˘ÌÙÒÌ·Ù·, ÂÓÒ Ë Ì¤ÛË ‰È¿ÚÎÂÈ·
ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Â›Ó·È 5,7 Ì‹Ó˜ (‡ÚÔ˜: 4
Ë̤Ú˜-18 Ì‹Ó˜) (1,9,10).
∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÙˆÓ fiÁÎˆÓ ·fi ÌÈ-
ÎÚ¿, ÛÙÚÔÁÁ˘Ï¿, ÌÏ ·ÙÙ·Ú· Â›Ó·È ‰‡ÛÎÔÏË
Î·È ··ÈÙÂ›Ù·È Ë Û˘Ì‚ÔÏ‹ Ù˘ ÈÛÙÔÏÔÁÈ΋˜/·ÓÔ-
ÛÔ˚ÛÙÔ¯ËÌÈ΋˜ ·Ó¿Ï˘Û˘, Ù˘ ÌÔÚȷ΋˜ ‚ÈÔÏÔ-
Á›·˜ Î·È ÙÔ˘ ËÏÂÎÙÚÔÓÈÎÔ‡ ÌÈÎÚÔÛÎÔ›Ô˘ ÁÈ·
ÙËÓ Â›Ù¢͋ Ù˘ (11,12). ∏ ÂÍ·ÈÚÂÙÈ΋ ÚfiÔ‰Ô˜
Ù˘ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋˜ Î·È Ù˘ ÌÔÚȷ΋˜ ‚ÈÔÏÔ-
Á›·˜ η٤ÛÙËÛ ‰˘Ó·Ù‹ ÙËÓ ·Ó›¯Ó¢ÛË ¯ÚˆÌÔ-
ÛˆÌÈÎÒÓ Î·È ÌÔÚÈ·ÎÒÓ ·Ó·‰È·Ù¿ÍÂˆÓ Ô˘ ÂÈ-
ÙÚ¤Ô˘Ó ÙÔÓ Î·ıÔÚÈÛÌfi ·ÎÚÈ‚Ô‡˜ ‰È¿ÁÓˆÛ˘
Î·È ÚfiÁÓˆÛ˘, fiˆ˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ù˘ ÌÂϤ-
Ù˘ Ì·˜, fiÔ˘ Ë RT-PCR ·ÔÎ¿Ï˘„ ÙËÓ ·-
ÚÔ˘Û›· ˘‚ÚȉÈÎÒÓ ÌÂÙ·ÁÚ·ÊÒÓ EWS-FLI-1 ÙÂÎ-
ÌËÚÈÒÓÔÓÙ·˜ ÙË ‰È¿ÁÓˆÛË ÙÔ˘ EES. ™ËÌ·ÓÙÈ΋
ÌÂÙ¿ ·fi ÎÏÈÓÈÎÔ·ıÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Â›Ó·È Ë
‰È·›ÛÙˆÛË fiÙÈ Ë ÂÌÊ¿ÓÈÛË Ù˘ ÌÂÙ¿ıÂÛ˘
EWS-FLπ Ù‡Ô˘ 1 Û¯ÂÙ›˙ÂÙ·È Ì ηχÙÂÚË Ô-
Ú›· Ù˘ ÓfiÛÔ˘ ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ ÛÙ¿‰ÈÔ Ù˘
ÓfiÛÔ˘ ηٿ ÙË ‰È¿ÁÓˆÛË (13,14). ∞Ó Î·È Ì¤¯ÚÈ
Û‹ÌÂÚ· ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ÂÚÈÛÛfiÙÂÚ· ·fi 15
˘‚ÚȉÈο ÌÂÙ·ÁÚ·Ê‹Ì·Ù· ÌÂÙ·ı¤ÛÂˆÓ ÛÙ·
EWS, ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ Ù· ¯ÈÌ·ÈÚÈο ÁÔÓ›‰È·
Ô˘ ÚÔ·ÙÔ˘Ó Â›Ó·È ˘Â‡ı˘Ó· ÁÈ· ÙËÓ ·Ú·-
ÁˆÁ‹ ·ÚfiÌÔÈˆÓ ÚˆÙÂ˚ÓÒÓ, Ì ·ÔÙ¤ÏÂÛÌ· Ô
ÚfiÏÔ˜ ÙˆÓ ¯ÚˆÌÔÛˆÌÈÎÒÓ ÌÂÙ·ı¤ÛÂˆÓ Ó· ›ӷÈ
·ÓÂÍ¿ÚÙËÙÔ˜ Ù˘ ÔÁÎÔÁÔÓÈ΋˜ Û˘ÌÂÚÈÊÔÚ¿˜
ÙˆÓ fiÁÎˆÓ Ù˘ ÔÌ¿‰·˜ ·˘Ù‹˜. ∂ÓÙÔ‡ÙÔȘ, Û‡Ì-
ʈӷ Ì ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ
ÔÈ ‰È·ÊÔÚÂÙÈΤ˜ ·ÓÙÈÌÂÙ·ı¤ÛÂȘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ
ÙËÓ ÎÏÈÓÈ΋ ÔÚ›· Î·È ÂͤÏÈÍË ÙˆÓ fiÁÎˆÓ ·˘-
ÙÒÓ (15). ™ÙÔÓ ¶›Ó·Î· 1 ·Ó·Ê¤ÚÔÓÙ·È ÔÈ Û˘¯Ófi-
ÙÂÚ˜ ¯ÚˆÌÔÛˆÌÈΤ˜ ÌÂÙ·ı¤ÛÂȘ Ù˘ ÔÈÎÔÁ¤ÓÂÈ-
·˜ ÙˆÓ Û·ÚÎˆÌ¿ÙˆÓ ÙÔ˘ Ewing (Ewing Sarcoma
family tumors, ESFT). H ¯Ú‹ÛË Û‹ÌÂÚ· Ó¤ˆÓ
ÂÍÂȉÈÎÂ˘Ì¤ÓˆÓ ÌÂıfi‰ˆÓ fiˆ˜ Â›Ó·È Ë RT-PCR
Î·È Ë Ì¤ıÔ‰Ô˜ FISH ÙÂÎÌËÚÈÒÓÔ˘Ó ÙË ‰È¿ÁÓˆÛË
Û ڷÁÌ·ÙÈο ‰‡ÛÎÔϘ ÂÚÈÙÒÛÂȘ, fiÔ˘
ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ı¤ÙÔ˘Ó ¤ÓÙÔÓË
ÙËÓ ˘Ô„›·, ·ÏÏ¿ ‰ÂÓ Â·ÚÎÔ‡Ó ÁÈ· Ó· ‚‚·ÈÒ-
ÛÔ˘Ó Ì ·ÎÚ›‚ÂÈ· ÙË ‰È¿ÁÓˆÛË Û·ÚÎÒÌ·ÙÔ˜ ÙÔ˘
Ewing. ∂ÈϤÔÓ, Ë RT-PCR Â›Ó·È ¯Ú‹ÛÈÌË Ì¤-
ıÔ‰Ô˜ ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘
‹ ÚÒÈÌ˘ ˘ÔÙÚÔ‹˜ ÛÙÔ ¿ÌÂÛÔ Ì¤ÏÏÔÓ (12).
√ Û˘Ó‰˘·ÛÌfi˜ ¯ËÌÂÈÔ-, ·ÎÙÈÓÔıÂڷ›·˜ ηÈ
¯ÂÈÚÔ˘ÚÁÈ΋˜ ·Ê·›ÚÂÛ˘ ÙÔ˘ fiÁÎÔ˘ ÚÔÛʤÚÂÈ
ÙËÓ Î·Ï‡ÙÂÚË ÂχıÂÚË ÓfiÛÔ˘ ÂÈ‚›ˆÛË (event
free survival, EFS) Î·È Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË
(overall survival, OS) ÙˆÓ ·ÛıÂÓÒÓ. ™‡Ìʈӷ ÌÂ
ÓÂfiÙÂÚ· ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ, ηٷÁÚ¿ÊÔ-
ÓÙ·È Û‹ÌÂÚ· ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘
fiÙ·Ó Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÚÔËÁÂ›Ù·È Ù˘
¯ËÌÂÈÔıÂڷ›·˜, fiˆ˜ ÛÙÔÓ ÚÒÙÔ ·ÛıÂÓ‹ Ù˘
ÌÂϤÙ˘ Ì·˜. ∞fi ÔÏ˘ÎÂÓÙÚÈΤ˜ ÌÂϤÙ˜ ÚÔ-
·ÙÂÈ fiÙÈ Ë 5ÂÙ‹˜ EFS ·Ó¤Ú¯ÂÙ·È ÛÙÔ 61% ÁÈ·
·ÛıÂÓ›˜ Ì EES Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÌÂ Û˘Ó-
‰˘·ÛÌfi ¯ËÌÂÈÔ-, ·ÎÙÈÓÔıÂڷ›·˜ Î·È ¯ÂÈÚÔ˘Ú-
ÁÈ΋˜ ·Ê·›ÚÂÛ˘, fï˜, Ë ÂÓÙfiÈÛË Ù˘ Ì¿˙·˜
·Ú·ÛÔÓ‰˘ÏÈο Û¯ÂÙ›˙ÂÙ·È Ì ÌÈÎÚfiÙÂÚË ÂÈ-
‚›ˆÛË (37,5%) (5,12,16). ∆¤ÏÔ˜, ÚÔ·ÙÂÈ fiÙÈ Ë
ÌÈÎÚ‹ Ì¿˙· ÙÔ˘ fiÁÎÔ˘, Ë Â˘Ú›· ¯ÂÈÚÔ˘ÚÁÈ΋
·Ê·›ÚÂÛ‹ ÙÔ˘ Î·È Ë ·Ô˘Û›· ÌÂÙ·ÛÙ¿ÛÂˆÓ Î·Ù¿
ÙË ‰È¿ÁÓˆÛË ·ÔÙÂÏÔ‡Ó ÛËÌ·ÓÙÈÎÔ‡˜ ¢ÓÔ˚ÎÔ‡˜
ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ (4,16).
™˘ÌÂÚ·ÛÌ·ÙÈο, ÚÔ·ÙÂÈ fiÙÈ ÙÔ EES, ·-
ÚfiÙÈ Û¿ÓÈÔ, ı· Ú¤ÂÈ Ó· Û˘ÌÂÚÈÏ·Ì‚¿ÓÂÙ·È
¶·È‰È·ÙÚÈ΋ 2006;69:458-462
¶›Ó·Î·˜ 1. ™˘¯ÓfiÙÂÚ˜ ¯ÚˆÌÔÛˆÌÈΤ˜ ÌÂÙ·ı¤ÛÂȘ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ÙˆÓ Û·ÚÎˆÌ¿ÙˆÓ ÙÔ˘ Ewing (Ewing’s Sarcoma familytumors, ESFT).
ªÂÙ¿ıÂÛË ÃÚˆÌÔÛˆÌÈ΋ ·ÓˆÌ·Ï›· ™˘¯ÓfiÙËÙ·
EWS-FLI-1 t(11;22)(q12;q24) 90-95%
∆‡Ô˘ 1 (65%)∆‡Ô˘ 2 (20%)
16 ¿ÏÏÔÈ Ù‡ÔÈ ÌÂÙ·ÁÚ·ÊÒÓ (15%)EWS-ERG t(21;22)(q22;q12) 5-10%∆‡Ô˘ 1 (60%)
4 ¿ÏÏÔÈ Ù‡ÔÈ ÌÂÙ·ÁÚ·ÊÒÓ (40%)EWS-ETV1 t(7;22)(p22;q12) <1%EWS-EIAF t(17;22)(q12;q12) <1%EWS-FEV t(2;22)(q33;q12) <1%
462 º. ∞ı·Ó·ÛÈ¿‰Ô˘ Î·È Û˘Ó.
¶·È‰È·ÙÚÈ΋ 2006;69:458-462
ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÙˆÓ fiÁÎˆÓ ÙˆÓ Ì·Ï·-ÎÒÓ ÌÔÚ›ˆÓ Ù˘ ·Ú·ÛÔÓ‰˘ÏÈ΋˜ ÂÚÈÔ¯‹˜,ÒÛÙ ӷ ηٷÛÙ› ‰˘Ó·Ù‹ Ë ¤ÁηÈÚË ‰È¿ÁÓˆÛËÎ·È ¤Ó·ÚÍË Ù˘ ıÂڷ¢ÙÈ΋˜ ·ÁˆÁ‹˜. ∂ÈϤ-ÔÓ, Ô ÁÂÓÈÎfi˜ ·È‰›·ÙÚÔ˜ ı· Ú¤ÂÈ Ó· Â›Ó·È È‰È-·›ÙÂÚ· ¢·ÈÛıËÙÔÔÈË̤ÓÔ˜ fiÙ·Ó ·ÓÙÈÌÂÙˆ›˙ÂÈÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ Ì ›ÌÔÓÔ fiÓÔÛÙ· ÔÛÙ¿ ‹ ÛÙ· ̷Ϸο ÌfiÚÈ·, ÒÛÙ ¤ÁηÈÚ· ӷηٷʇÁÂÈ Û ·ÂÈÎÔÓÈÛÙÈÎfi ¤ÏÂÁ¯Ô.
µÈ‚ÏÈÔÁÚ·Ê›·
1. Harimaya K, Oda Y, Matsuda S, Tanaka K, ChumanH, Iwamoto Y. Primitive neuroectodermal tumorand extraskeletal Ewing sarcoma arising primarilyaround the spinal column: report of four cases and areview of the literature. Spine 2003; 28:408-412.
2. Eroglu A, Kurkcuoglu IC, Karaoglanoglu N, Alper F,Gundogdu C. Extraskeletal Ewing sarcoma of the di-aphragm presenting with hemothorax. Ann ThoracSurg 2004;78:715-717.
3. Shin JH, Lee HK, Rhim SC, Cho KJ, Choi CG, SuhDC. Spinal epidural extraskeletal Ewing sarcoma:MR findings in two cases. AJNR Am J Neuroradiol2001;22:795-798.
4. Kutluk MT, Yalcin B, Akyuz C, Varan A, Ruacan S,Buyukpamukcu M. Treatment results and prognos-tic factors in Ewing sarcoma. Pediatr Hematol On-col 2004; 21:597-610.
5. Grier H, Krailo M, Link M et al. Improved outcomein non-metastatic Ewing’s sarcoma (EWS) andPNET of bone with the addition of ifosfamide (I)and etoposide (E) to vincristine (V), Adriamycin(Ad), cyclophosphamide (C), and Actinomycin (A):a Children’s Cancer Group (CCG) and PediatricOncology Group (POG) report. Proc Am Soc ClinOncol 1994;13:421.
6. Ewing J. Diffuse endothelioma of bone. Proc NYPathol Soc 1921;21;17:1-13.
7. Tefft M, Vawter GM, Mitus A. Paravertebral “roundcell” tumors in children. Radiology 1969;92:1501-1509.
8. Raney RB, Asmar L, Newton WA Jr, Bagwell C,Breneman JC, Crist W, et al. Ewing’s sarcoma of softtissues in childhood: a report from the IntergroupRhabdomyosarcoma Study, 1972 to 1991. J ClinOncol 1997;15:574-582.
9. Medrano Martin MC, Alvarez Guisasola FJ, AlvarezGago T, Valbuena Crespo C, Blanco Quiros A, Bo-billo del Amo H. Extraskeletal Ewing sarcoma. Re-port of a new case and revision of the subject. AnEsp Pediatr 1992;36:487-490.
10. O'Keeffe F, Lorigan JG, Wallace S. Radiological fea-tures of extraskeletal Ewing sarcoma. Br J Radiol1990;63:456-460.
11. Guiter GE, Gamboni MM, Zakowski MF. The cytol-ogy of extraskeletal Ewing sarcoma. Cancer1999;87:141-148.
12. Ahmad R, Mayol BR, Davis M, Rougraff BT. Ex-traskeletal Ewing’s sarcoma. Cancer 1999; 85:725-731.
13. De Alava E, Kawai A, Healey JH, Fligman I, MeyersPA, Huvos AG, et al. EWS-FLI-1 fusion transcriptstructure is an independent determinant of progno-sis in Ewing’s sarcoma. J Clin Oncol 1998;16:1248-1255.
14. De Alava E, Panizo A, Antonescu CR, Huvos AG,Pardo-Mindan FJ, Barr FG, et al. Association of EWS-FLI-1 type 1 fusion with lower proliferative rate inEwing’s sarcoma. Am J Pathol 2000;156:849-855.
15. Delattre O, Zucman J, Melot T, Garau XS, ZuckerJM, Lenoir GM, et al. The Ewing family of tumors -a subgroup of small-round-cell tumors defined byspecific chimeric transcripts. N Engl J Med 1994;331:294-299.
16. Koscielniak E, Morgan M, Treuner J. Soft tissue sar-coma in children: prognosis and management. Pae-diatr Drugs 2002; 4:21-28.
463∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏ CASE REPORT
°ÂÓÓ‹ÛÂȘ ·È‰ÈÒÓ Ì ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ
ÌÂÙ¿ ·fi ˘Ô‚ÔËıÔ‡ÌÂÓË ÁÔÓÈÌÔÔ›ËÛË
π. ∆Û¿ÙÚ·1, ∫. ∫·Ú·ÛÌ¿Ó˘1, º. µ·ÚÂÏ¿˜2, X. ∆Û¿ÓÙ·ÏË1, A. ∫Ô‡ÛË1, M. ∞ı·Ó·Û›Ô˘1
¶ÂÚ›ÏË„Ë: ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ì ÙË ÌÂÁ¿ÏË ÚfiÔ‰Ô ÙˆÓ Ù¯ÓÈÎÒÓ ˘Ô‚ÔËıÔ‡ÌÂÓ˘ ·Ó··Ú·Áˆ-Á‹˜, fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚ· ˙¢Á¿ÚÈ· Ì ÚÔ‚Ï‹Ì·Ù· ÁÔÓÈÌfiÙËÙ·˜ ηٷʇÁÔ˘Ó Û ·˘Ù¤˜. ¶·Ú¿ÏÏËÏ·,Ù· ÚÔÁÚ¿ÌÌ·Ù· ÚfiÏ˄˘ Ù˘ ‚-ªÂÛÔÁÂȷ΋˜ ∞Ó·ÈÌ›·˜ (‚-ª∞) ¤¯Ô˘Ó ÂÙ‡¯ÂÈ ÙÔÓ ÛÙfi¯Ô ÙÔ˘˜, ÌÂÈ-ÒÓÔÓÙ·˜ ‰Ú·ÛÙÈο ÙÔÓ ·ÚÈıÌfi ÁÂÓÓ‹ÛÂˆÓ Ó¤ˆÓ ·Û¯fiÓÙˆÓ. øÛÙfiÛÔ, ·Ú·ÙËÚÔ‡ÓÙ·È ÁÂÓÓ‹ÛÂȘ ·Û¯fi-ÓÙˆÓ ·fi ‚-ª∞ Î·È ¿ÏϘ ÛÔ‚·Ú¤˜ ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ ÌÂÙ¿ ·fi ¯Ú‹ÛË ÌÂıfi‰ˆÓ Ù¯ÓËÙ‹˜ ÁÔÓÈÌÔ-Ô›ËÛ˘. ¶ÂÚÈÁÚ¿ÊÔÓÙ·È ÙÚÂȘ ÂÚÈÙÒÛÂȘ ÁÂÓÓ‹ÛÂˆÓ ·ÛıÂÓÒÓ Ì ÛÔ‚·Ú¤˜ ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ Ô˘ÚÔ‹Ïı·Ó ·fi Â͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔÔ›ËÛË. √ ÚÒÙÔ˜ ·ÛıÂÓ‹˜ ÂÌÊ·Ó›˙ÂÈ ÔÌfi˙˘ÁÔ ‚-ª∞ ÌÂÙ¿ ·fi¯Ú‹ÛË ÁÂÓÂÙÈÎÔ‡ ˘ÏÈÎÔ‡ ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘, Ô ‰Â‡ÙÂÚÔ˜ ‰ÈÏ‹ ÂÙÂÚÔ˙˘ÁˆÙ›· ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋˜ ÓfiÛÔ˘Î·È ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂÈ·˜ D (D-Punjab) ÌÂÙ¿ ·fi ¯Ú‹ÛË ˆ·Ú›Ô˘ ‰fiÙÚÈ·˜ Î·È Ô ÙÚ›ÙÔ˜ ‰ÈÏ‹ ÂÙÂÚÔ˙˘-ÁˆÙ›· ‚-ª∞ Î·È ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂÈ·˜ Lepore ÌÂÙ¿ ·fi ¯Ú‹ÛË Û¤ÚÌ·ÙÔ˜ ‰fiÙË. √È ¿Û¯ÔÓÙ˜ ·Ú·-ÎÔÏÔ˘ıÔ‡ÓÙ·È Û˘ÛÙËÌ·ÙÈο Î·È ˘Ô‚¿ÏÏÔÓÙ·È Û ıÂڷ›· ÛÙË ªÔÓ¿‰· ªÂÛÔÁÂȷ΋˜ ∞Ó·ÈÌ›·˜. ™ÙËÓ∂ÏÏ¿‰· Û‹ÌÂÚ·, ·Ú¿ ÙËÓ Â˘Ú›· ̄ Ú‹ÛË ÙˆÓ Ù¯ÓÈÎÒÓ ̆ Ô‚ÔËıÔ‡ÌÂÓ˘ ·Ó··Ú·ÁˆÁ‹˜, ‰ÂÓ ̆ ¿Ú¯Ô˘ÓÛ·ÊÒ˜ ıÂÛÌÔıÂÙË̤ÓÔÈ Î·ÓfiÓ˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ Î¤ÓÙÚˆÓ Â͈ۈ̷ÙÈ΋˜ ÁÔÓÈÌÔÔ›ËÛ˘ Î·È ¯Ú‹Û˘ÁÂÓÂÙÈÎÔ‡ ˘ÏÈÎÔ‡. ∞ÔÙ¤ÏÂÛÌ· Â›Ó·È Ë ÂÌÊ¿ÓÈÛË ÛÔ‚·ÚÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ ÛÙË ‰ËÌfiÛÈ· ˘Á›·, fiˆ˜ ·˘-Ù¿ Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔ ·ÚfiÓ ¿ÚıÚÔ, ÁÈ· ÙËÓ ·ÔÊ˘Á‹ ÙˆÓ ÔÔ›ˆÓ Â›Ó·È ÂÈÙ·ÎÙÈ΋ ·Ó¿ÁÎË Ó· Á›-ÓÔ˘Ó ÔÈ ·Ó¿ÏÔÁ˜ ÓÔÌÔıÂÙÈΤ˜ Ú˘ıÌ›ÛÂȘ Ô˘ ı· ηıÔÚ›ÛÔ˘Ó Ì ۷ʋÓÂÈ· ÙÔÓ ÙÚfiÔ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓΤÓÙÚˆÓ Â͈ۈ̷ÙÈ΋˜ ÁÔÓÈÌÔÔ›ËÛ˘ Î·È ı· ÔÚ›ÛÔ˘Ó ÙÔ˘˜ ηٿÏÏËÏÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ ÂϤÁ¯Ô˘.
§¤ÍÂȘ ÎÏÂȉȿ: ∂͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔÔ›ËÛË, ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ.
Births of children with hemoglobinopathies following use of IVF
I. Tsatra1, K. Karasmanis1, F. Varelas2, C. Tsantali1, A. Koussi1, M. Athanassiou1
Abstract: Recent advances in techniques of artificial reproduction have lead to their use byconstantly increasing numbers of couples with fertility problems. Although ‚-thalassaemia screeningprogrammes have successfully reduced births of affected individuals a number of births have beenreported of children with ‚-thalassaemia and other major haemoglobinopathies following in vitrofertilization (IVF). The cases are described of three children with ‚-thalassaemia and other majorhaemoglobinopathies conceived by IVF. The first child was born with homozygous ‚-thalassaemiaafter the use of gametes from both parents, the second was a carrier of both sickle cell anaemia andhaemoglobinopathy D (D-Punjab) trait after the use of donor ova, and the third was a carrier of both‚-thalassaemia and haemoglobinopathy Lepore trait after the use of donor sperm. All the patients arebeing systematically monitored and treated in the Thalassaemia Unit. In Greece at present, despite theincreasing use of artificial reproduction techniques, no rules have yet been defined regulatinginfertility centres and the use of human gametes. The appropriate legal framework needs to be set upfor licensing, providing working guidelines and monitoring infertility centres where IVF is provided.
Key words: In vitro fertilization (IVF), haemoglobinopathies.
1 ªÔÓ¿‰· ªÂÛÔÁÂȷ΋˜∞Ó·ÈÌ›·˜, ∞’ ¶·È‰È·ÙÚÈ΋∫ÏÈÓÈ΋ ∞.¶.£., πÔÎÚ¿ÙÂÈÔ°ÂÓÈÎfi ¡ÔÛÔÎÔÌ›Ô,£ÂÛÛ·ÏÔÓ›ÎË
2 ¢’ °˘Ó·ÈÎÔÏÔÁÈ΋-ª·È¢ÙÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£.,πÔÎÚ¿ÙÂÈÔ °ÂÓÈÎfi¡ÔÛÔÎÔÌ›Ô, £ÂÛÛ·ÏÔÓ›ÎË
AÏÏËÏÔÁÚ·Ê›·:
ªÈÚ¿ÓÙ· ∞ı·Ó·Û›Ô˘-ªÂÙ·Í¿ªÔÓ¿‰· ªÂÛÔÁÂȷ΋˜∞Ó·ÈÌ›·˜, ∞’ ¶·È‰È·ÙÚÈ΋∫ÏÈÓÈ΋ ∞.¶.£.πÔÎÚ¿ÙÂÈÔ °ÂÓÈÎfi¡ÔÛÔÎÔÌÂ›Ô £ÂÛ/ӛ΢∂-mail: [email protected]
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 04-03-2005 ∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 22-07-2005
1 Beta-Thalassaemia Unit, 1stPaediatric Clinic, AristotleUniversity of Thessaloniki,Ippokration GeneralHospital of Thessaloniki
2 4th Clinic of Gynecology-Obstetrics, AristotleUniversity of Thessaloniki,Ippokration GeneralHospital of Thessaloniki
Correspondence:
Miranda Athanassiou-MetaxaBeta-Thalassaemia Unit, 1stPaediatric Clinic, AristotleUniversity of Thessaloniki, Ippokration General Hospitalof Thessaloniki∂-mail: [email protected]
Date of submission: 04-03-2005 Date of approval: 22-07-2005
¶·È‰È·ÙÚÈ΋ 2006;69:463-467
™˘ÓÙÔÌÔÁڷʛ˜
ª∞ ªÂÛÔÁÂȷ΋ ∞Ó·ÈÌ›·
IVF/ET Â͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔÔ›ËÛË Î·È
ÂÌ‚Ú˘ÔÌÂÙ·ÊÔÚ¿
GIFT ÂÓ‰ÔÛ·ÏÈÁÁÈ΋ ÌÂÙ·ÊÔÚ¿ Á·ÌÂÙÒÓ
ZIFT ÂÓ‰ÔÛ·ÏÈÁÁÈ΋ ÌÂÙ·ÊÔÚ¿ ˙˘ÁˆÙÒÓ
ICSI ÂÓ‰Ô΢ÙÙ·ÚÔÏ·ÛÌÈ΋/ÂÓ‰Ôˆ·ÚÈ΋
¤Á¯˘ÛË ÛÂÚÌ·ÙÔ˙ˆ·Ú›Ô˘
CVS ‚ÈÔ„›· ¯ÔÚÈ·ÎÒÓ Ï·¯ÓÒÓ
464 π. ∆Û¿ÙÚ· Î·È Û˘Ó.
∂ÈÛ·ÁˆÁ‹
∏ ªÂÛÔÁÂȷ΋ ∞Ó·ÈÌ›· (ª∞) Â›Ó·È ÌÈ· ÂÙÂ-
ÚÔÁÂÓ‹˜ ÔÌ¿‰· ÁÂÓÂÙÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ ÛÙ·
ÔÔ›· Ë ·Ú·ÁˆÁ‹ Ê˘ÛÈÔÏÔÁÈ΋˜ ·ÈÌÔÛÊ·ÈÚ›-
Ó˘ Â›Ó·È ÂÓ Ì¤ÚÂÈ ‹ ÙÂÏ›ˆ˜ ·Ó·Ú΋˜ ÏfiÁˆ
Ù˘ ÂÏ·Ùو̷ÙÈ΋˜ Û‡ÓıÂÛ˘ Ì›·˜ ‹ ÂÚÈÛÛÔ-
Ù¤ÚˆÓ ·Ï‡ÛˆÓ ÛÊ·ÈÚ›Ó˘. ∞Ó¿ÏÔÁ· Ì ÙȘ ·Ï‡-
ÛÔ˘˜ (· ‹ ‚) Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ÂÏ·Ùو̷ÙÈ΋
Û‡ÓıÂÛË ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ‰È¿ÊÔÚ· ı·Ï·ÛÛ·È-
ÌÈο Û‡Ó‰ÚÔÌ·.
√È ÊÔÚ›˜ Ù˘ ‚-ª∞ ÛÙËÓ ∂ÏÏ¿‰· ·Ó¤Ú¯Ô-
ÓÙ·È ÛÙÔ 7,5% › ÙÔ˘ ÁÂÓÈÎÔ‡ ÏËı˘ÛÌÔ‡ (1).
∞˘Ùfi ¤¯ÂÈ ˆ˜ Û˘Ó¤ÂÈ· ÙË ‰È·ÈÒÓÈÛË Ù˘ ÓfiÛÔ˘,
ȉȷ›ÙÂÚ· ·Ó ‰ÂÓ ÏËÊıÔ‡Ó Ù· ηٿÏÏËÏ· ̤ÙÚ·
ÚfiÏ˄˘.
∞fi ÙÔ 1978, Ì ڈÙÔ‚Ô˘Ï›· Ù˘ ¶·ÁÎfi-
ÛÌÈ·˜ √ÚÁ¿ÓˆÛ˘ ÀÁ›·˜ (¶.√.À.), ÂÊ·ÚÌfi˙Â-
Ù·È Ô ÚÔÁÂÓÓËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ (1). ŒÙÛÈ, ÂÓÒ ·-
Ï·ÈfiÙÂÚ· ÔÈ ÁÂÓÓ‹ÛÂȘ ·Û¯fiÓÙˆÓ ·fi ‚-ª∞
·Ó¤Ú¯ÔÓÙ·Ó Û 200 ÂÙËÛ›ˆ˜, Ù· ÙÂÏÂ˘Ù·›· ¯Úfi-
ÓÈ· Ô ·ÚÈıÌfi˜ ·˘Ùfi˜ ÌÂÈÒıËΠ۠ÏÈÁfiÙÂÚ˜
·fi 10. ™‡Ìʈӷ Ì ٷ ·ÓˆÙ¤Úˆ, ÙÔ ÚfiÁÚ·Ì-
Ì· ÚfiÏ˄˘ ÂÓ ÔÏÏÔ›˜ ¤¯ÂÈ ÂÙ‡¯ÂÈ. øÛÙfiÛÔ,
ÙÂÏÂ˘Ù·›· Ì ÙȘ Ӥ˜ Ù¯ÓÈΤ˜ ˘Ô‚ÔËıÔ‡ÌÂ-
Ó˘ ÁÔÓÈÌÔÔ›ËÛ˘, ¤¯Ô˘Ó ·Ú·ÙËÚËı› ÁÂÓÓ‹-
ÛÂȘ ·È‰ÈÒÓ Ì ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ.
™ÙË ªÔÓ¿‰· ªÂÛÔÁÂȷ΋˜ ∞Ó·ÈÌ›·˜ ÙÔ˘
πÔÎÚ¿ÙÂÈÔ˘ ¡ÔÛÔÎÔÌ›Ԣ £ÂÛÛ·ÏÔӛ΢
‰È·ÁÓÒÛÙËÎ·Ó ÙÚ›· ·È‰È¿ Ì ÛÔ‚·Ú¤˜ ·ÈÌÔ-
ÛÊ·ÈÚÈÓÔ¿ıÂȘ Ô˘ ¤¯Ô˘Ó ÁÂÓÓËı› ÌÂÙ¿ ·fi
Ù¯ÓËÙ‹ ÁÔÓÈÌÔÔ›ËÛË Ì ÁÂÓÂÙÈÎfi ˘ÏÈÎfi ÙˆÓ
ÁÔÓ¤ˆÓ ‹ Ï‹„Ë ·˘ÙÔ‡ (Û¤ÚÌ·/ˆ¿ÚÈÔ) ·fi
ÙÚ¿Â˙˜.
¶ÂÚÈÁÚ·Ê‹ ÂÚÈÛÙ·ÙÈÎÒÓ
1Ô ÂÚÈÛÙ·ÙÈÎfi
∫ÔÚ›ÙÛÈ 2 ÂÙÒÓ, ·ÔÙ¤ÏÂÛÌ· ‰›‰˘Ì˘ ·Ë-
Û˘ ÌÂÙ¿ ·fi Â͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔÔ›ËÛË. ∏
ÌËÙ¤Ú· ÁÓˆÛÙ‹ ÊÔÚ¤·˜ ‚-ª∞ (ηٷÁˆÁ‹ ·fi
ÂÚÈÔ¯‹ Ù˘ ÷ÏÎȉÈ΋˜), ÂÓÒ Ô ·Ù¤Ú·˜ ·Ó·-
ʤÚÂÈ fiÙÈ ‰ÂÓ Â›Ó·È ÊÔÚ¤·˜ ‚-ª∞.
√È ÁÔÓ›˜ ÌÂÙ¿ ·fi ÌÂÚÈο ¯ÚfiÓÈ· ¯ˆÚ›˜ Ê˘-
ÛÈÔÏÔÁÈ΋ ÙÂÎÓÔÔ›ËÛË ÛÙÚ¿ÊËÎ·Ó ÛÙËÓ ÙÂ-
¯ÓËÙ‹. ⁄ÛÙÂÚ· ·fi 3 ¯ÚfiÓÈ· ÚÔÛ·ıÂÈÒÓ ¤ÁÈ-
Ó ÂÈÙ˘¯Ë̤ÓË ·fiÂÈÚ· ˘Ô‚ÔËıÔ‡ÌÂÓ˘
ÁÔÓÈÌÔÔ›ËÛ˘. ŒÁÈÓ ÚfiÎÏËÛË ˆÔı˘Ï·ÎÈÔÚ-
ÚËÍ›·˜, ·ÎÔÏÔ‡ıËÛÂ ˆÔÏË„›·, in vitro ÁÔÓÈÌÔ-
Ô›ËÛË Ì ÁÂÓÂÙÈÎfi ˘ÏÈÎfi ÙÔ˘ Û˘˙‡ÁÔ˘ Î·È ÂÌ-
‚Ú˘ÔÌÂÙ·ÊÔÚ¿. ¶·Ú¿ÏÏËÏ·, ·Ó·Ê¤ÚÂÙ·È fiÙÈ
¤ÁÈÓ ϋÚ˘ ÚÔÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ -Î·È ÙÔ˘
·Ù¤Ú·- Ô ÔÔ›Ô˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜.
∆Ô ·È‰› ÁÂÓÓ‹ıËΠÙËÓ 34Ë Â‚‰ÔÌ¿‰· ·Ë-
Û˘ Ì ηÈÛ·ÚÈ΋ ÙÔÌ‹ Î·È ÓÔÛËχıËΠÁÈ· 10
Ë̤Ú˜ Û ÙÌ‹Ì· ÚÔÒÚˆÓ ÏfiÁˆ ÚÔˆÚfiÙËÙ·˜
Î·È ›ÎÙÂÚÔ˘.
™Â ËÏÈΛ· 5 ÌËÓÒÓ ·ÚÔ˘Û›·Û ˘ÓËÏ›·, ‡-
ÎÔÏË ÎfiˆÛË Î·Ù¿ ÙË Û›ÙÈÛË Î·È ˆ¯ÚfiÙËÙ·. √
·Ú¯ÈÎfi˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜, ¤‰ÂÈÍ ÛÔ‚·-
Ú‹ ·Ó·ÈÌ›· (Ht~18%) Î·È ÙÔ ‚Ú¤ÊÔ˜ ·-
Ú·¤ÌÊıËΠÛÙÔ ∞ÈÌ·ÙÔÏÔÁÈÎfi ∂͈ÙÂÚÈÎfi
π·ÙÚ›Ô. ™ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ÙÔ ·È‰› ÂÌÊ¿ÓÈ-
˙ ˆ¯ÚfiÙËÙ·, ‹·Ú „ËÏ·ÊËÙfi 2-3 cm, ÛÏ‹Ó·
„ËÏ·ÊËÙfi 2-3 cm. √ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜
ÙˆÓ ÁÔÓ¤ˆÓ Î·È ÙÔ˘ ‚Ú¤ÊÔ˘˜ (¶›Ó·Î·˜ 1) ¤‰ÂÈ-
Í fiÙÈ ÙÔ ‚Ú¤ÊÔ˜ ‹Ù·Ó ÔÌÔ˙˘ÁÒÙ˘ ÁÈ· ‚-ª∞.
∆Ô ‚Ú¤ÊÔ˜ Ù¤ıËΠ۠ٷÎÙÈÎfi ÚfiÁÚ·ÌÌ·
ÌÂÙ·ÁÁ›ÛˆÓ.
¶·È‰È·ÙÚÈ΋ 2006;69:463-467
¶›Ó·Î·˜ 1. ∂ÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ÔÈÎÔÁ¤ÓÂÈ·˜ 1Ô˘ ÂÚÈÛÙ·ÙÈÎÔ‡
¶¿Û¯Ô˘Û· ¶·Ù¤Ú·˜ ªËÙ¤Ú·
Hb (g/dl) 9,6 12,9 11,2
Ht (%) 29,5 41,8 33,3
MCV (fl) 79,3 63 68,4
MCH (pg) 25,8 19,5 21,1
ªÔÚÊÔÏÔÁ›· ÂÚ˘ıÚÒÓ ∞ÓÈÛÔ΢ÙÙ¿ÚˆÛË ++ ∞ÓÈÛÔ΢ÙÙ¿ÚˆÛË + ∞ÓÈÛÔ΢ÙÙ¿ÚˆÛË +
ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ªÈÎÚÔ΢ÙÙ¿ÚˆÛË ++ ¶ÔÈÎÈÏÔ΢ÙÙ¿ÚˆÛË+ ¶ÔÈÎÈÏÔ΢ÙÙ¿ÚˆÛË+ ¶ÔÈÎÈÏÔ΢ÙÙ¿ÚˆÛË ++ Àԯڈ̛· + Àԯڈ̛· +
Àԯڈ̛· + µ. ÛÙ›ÍË + µ. ÛÙ›ÍË +∂Ú˘ıÚÔ‚Ï¿ÛÙ˜ Û¿ÓÈÔÈ
¢ÈÏfi˜ ÏËı˘ÛÌfi˜
ºÂÚÚÈÙ›ÓË (ng/ml) 459 148 30
∞ÈÌÔÂÙ¿ÏÈ· (x 103/Ìl) 229.000 177.000 295.000
∏b A2 2,6% 6,4% 5,8%
Hb F 40% 0,5% 1%
ªÂÙ·ÏÏ·Á¤˜ ÁÔÓȉ›Ô˘ ‚ ·Ï‡ÛÔ˘ IVS1:110 / CD39 N / CD39 IVS1:110 / N
465∞ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ ÌÂÙ¿ ·fi ˘Ô‚ÔËıÔ‡ÌÂÓË ÁÔÓÈÌÔÔ›ËÛË
2Ô ÂÚÈÛÙ·ÙÈÎfi
∞ÁfiÚÈ ËÏÈΛ·˜ 5 ÂÙÒÓ, ·ÔÙ¤ÏÂÛÌ· ‰›‰˘Ì˘
·ËÛ˘ ÌÂÙ¿ ·fi Â͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔÔ›ËÛË
ÌÂ ¯Ú‹ÛË ‰fiÙÚÈ·˜ ˆ·Ú›Ô˘. ∏ ÌËÙ¤Ú· ˘Ô‚Ï‹-
ıËΠ۠ıÂڷ›· Ì ÁÔÓ·‰ÔÙÚÔ›Ó˜ Ì ·ÓÂÈ-
Ù˘¯‹ ˆÚ›Ì·ÓÛË ˆÔı˘Ï·Î›ˆÓ, ÔfiÙ ¯ÚËÛÈÌÔ-
ÔÈ‹ıËΠˆ¿ÚÈÔ ‰fiÙÚÈ·˜ Ô˘ ÁÔÓÈÌÔÔÈ‹ıËÎÂ
in vitro Ì ÁÂÓÂÙÈÎfi ˘ÏÈÎfi ÙÔ˘ ·Ù¤Ú·.
∏ ‰È·‰Èηۛ· ¤ÁÈÓ Û ΤÓÙÚÔ Ù¯ÓËÙ‹˜ ÁÔ-
ÓÈÌÔÔ›ËÛ˘ ÙÔ˘ Â͈ÙÂÚÈÎÔ‡ (§ÔÓ‰›ÓÔ). √ ·-
Ù¤Ú·˜ Â›Ó·È ÁÓˆÛÙfi˜ ÊÔÚ¤·˜ ‰Ú·ÓÔ΢ÙÙ·ÚÈ-
΋˜ ÓfiÛÔ˘, ÂÓÒ Ë ‰fiÙÚÈ· ˆ·Ú›Ô˘ ·Ó·Ê¤ÚÂÙÔ ˆ˜
Ê˘ÛÈÔÏÔÁÈ΋.
∆Ô ·È‰› Û ËÏÈΛ· 6 ÌËÓÒÓ ÂÌÊ¿ÓÈÛ ÂÒ-
‰˘ÓË ‰ÈfiÁΈÛË ‰·ÎÙ‡ÏÔ˘ ‰ÂÍÈ¿˜ ¿ÎÚ·˜ ¯ÂÈÚfi˜.
™Â ËÏÈΛ· 18 ÌËÓÒÓ ÂÌÊ¿ÓÈÛ ÂÒ‰˘ÓË ‰Èfi-
ÁΈÛË ‰ÂÍÈÔ‡ ·ÁÎÒÓ· Î·È ‰·ÎÙ‡ÏÔ˘ ‰ÂÍÈ¿˜
¿ÎÚ·˜ ¯ÂÈÚfi˜ Ì Â̇ÚÂÙÔ Î·È Ï›ÁÔ ·ÚÁfiÙÂÚ·
‰ÈfiÁΈÛË ‰·ÎÙ‡ÏÔ˘ ·ÚÈÛÙÂÚ‹˜ ¿ÎÚ·˜ ¯ÂÈÚfi˜.
∞Ú¯Èο, ÏfiÁˆ Ù˘ ÂÚÈÁÚ·Ê‹˜ ÙˆÓ ÂÂÈÛÔ‰›ˆÓ
ˆ˜ ·ÏÒÓ ‰ÈÔÁÎÒÛÂˆÓ Î·È Ì ‚¿ÛË ÙÔ ÔÈÎÔÁÂ-
ÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ·ÏÏÂÚÁ›·˜, ıˆڋıËÎ·Ó ˆ˜
·Ï‹ ÔÚÔÓÔÛ›· Î·È ¯ÔÚËÁ‹ıËÎ·Ó ÎÔÚÙÈÎÔÛÙÂ-
ÚÔÂȉ‹ Ì ηϋ ·ÓÙ·fiÎÚÈÛË.
∏ ÂÌÊ¿ÓÈÛË ›ÎÙÂÚÔ˘ Î·È ·Ó·ÈÌ›·˜ ηٿ ÙË
‰È¿ÚÎÂÈ· Ì›·˜ ÂÎ ÙˆÓ ÎÚ›ÛˆÓ, ÛÂ Û˘Ó‰˘·ÛÌfi
Ì ÙÔ ÈÛÙÔÚÈÎfi ÊÔÚ›·˜ ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋˜
·Ó·ÈÌ›·˜ ÙÔ˘ ·Ù¤Ú·, ¤ıÂÛÂ ÛÙË ıÂÚ¿ÔÓÙ· È·-
ÙÚfi ÙËÓ ˘fiÓÔÈ· ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂÈ·˜, ÔfiÙÂ
ÙÔ ·È‰› ·Ú·¤ÌÊıËΠÛÙÔ ·ÈÌ·ÙÔÏÔÁÈÎfi
Â͈ÙÂÚÈÎfi È·ÙÚ›Ô. ∫ÏÈÓÈο ·ÚÔ˘Û›·˙ ‹·Ú
ÌfiÏȘ „ËÏ·ÊËÙfi Î·È ÛÏ‹Ó· ·„ËÏ¿ÊËÙÔ.
√ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ·È‰ÈÔ‡, ÙÔ˘
·Ù¤Ú· Î·È ÙÔ˘ ‰›‰˘ÌÔ˘ ·‰ÂÏÊÔ‡ (¶›Ó·Î·˜ 2)
¤‰ÂÈÍ fiÙÈ ÙÔ ·È‰› Â›Ó·È ‰ÈÏfi˜ ÂÙÂÚÔ˙˘ÁÒÙ˘
‰Ú·ÓÔ΢ÙÙ·ÚÈ΋˜ ÓfiÛÔ˘ Î·È ·ÈÌÔÛÊ·ÈÚÈÓÔ-
¿ıÂÈ·˜ D (D-Punjab). √È ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ
Ù˘ ‰ÈÏ‹˜ ·˘Ù‹˜ ÂÙÂÚÔ˙˘ÁˆÙ›·˜ Â›Ó·È fiÌÔȘ
Ì ·˘Ù¤˜ Ù˘ ‰Ú·ÓÔ΢ÙÙ·ÚÈ΋˜ ÓfiÛÔ˘.
∆Ô ·È‰› ·Ú·ÎÔÏÔ˘ıÂ›Ù·È Û˘ÛÙËÌ·ÙÈο
ÛÙÔ ∞ÈÌ·ÙÔÏÔÁÈÎfi ∂͈ÙÂÚÈÎfi π·ÙÚ›Ô, ·ÚÔ˘-
ÛÈ¿˙ÂÈ ÂÚÈÛÙ·Ûȷο ÂÒ‰˘Ó˜ ıÚÔÌ‚ˆÙÈΤ˜
ÎÚ›ÛÂȘ ÔÈ Ôԛ˜ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È ÛÙÔ Û›ÙÈ ‹
ÙÔ ÓÔÛÔÎÔÌ›Ô, ÂÓÒ ‰È·ÙËÚ› Hb~7,8-8 gr/dl.
3Ô ÂÚÈÛÙ·ÙÈÎfi
∞ÁfiÚÈ ËÏÈΛ·˜ 2,5 ÂÙÒÓ, ·ÔÙ¤ÏÂÛÌ· ·ËÛ˘
ÌÂÙ¿ ·fi Â͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔÔ›ËÛË Ì ¯Ú‹ÛË
‰fiÙË Û¤ÚÌ·ÙÔ˜. √ ·Ù¤Ú·˜ ÂÌÊ¿ÓÈ˙ ÂӉȿÌÂ-
ÛË ‚-ª∞ Ù˘ ÔÔ›·˜ Ë ‰È¿ÁÓˆÛË ¤ÁÈÓ fiÙ·Ó ̆ Ë-
ÚÂÙÔ‡Û ÙË ÛÙÚ·ÙȈÙÈ΋ ÙÔ˘ ıËÙ›·. ¢ÂÓ ¯ÚÂÈ¿-
ÛÙËΠÔÙ¤ ÌÂÙ¿ÁÁÈÛË, ‰È·ÙËÚ› Hb~9-10 gr/dl,
‰ÂÓ ¤¯ÂÈ Á›ÓÂÈ ÛÏËÓÂÎÙÔÌ‹, ÂÓÒ ¿Û¯ÂÈ ·fi ÈÓ-
ÛÔ˘ÏÈÓÔÂÍ·ÚÙÒÌÂÓÔ ‰È·‚‹ÙË ·fi 20ÂÙ›·˜. ∏ ÌË-
Ù¤Ú· Â›Ó·È ÁÓˆÛÙ‹ ÊÔÚ¤·˜ ‚-ª∞ Î·È ·ÚÔ˘Û›·˙Â
˘ÔÁÔÓÈÌfiÙËÙ· ÏfiÁˆ Û·ÏÈÁÁÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜.
∞ÔÊ·Û›ÛÙËΠӷ ¯ÚËÛÈÌÔÔÈËı› ÁÂÓÂÙÈÎfi ˘ÏÈ-
Îfi ‰fiÙË ÁÈ· Ó· ·ÔʇÁÔ˘Ó Èı·Ó‹ ÂÁ΢ÌÔÛ‡ÓË
·È‰ÈÔ‡ Ì ‚-ª∞. ŒÁÈÓ Â͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔ-
Ô›ËÛË Ì ۤÚÌ· ‰fiÙË Ì ÂÈÙ˘¯‹ ηٿÏËÍË.
∆Ô ·È‰› ÁÂÓÓ‹ıËΠ̠ηÈÛ·ÚÈ΋ ÙÔÌ‹ ÙËÓ
38Ë Â‚‰ÔÌ¿‰· (ÏfiÁˆ ÔÏ˘ÙÈÌfiÙËÙ·˜ Ù˘ ·Ë-
Û˘). ™Â ËÏÈΛ· 2 ¯ÚfiÓˆÓ ‰È·ÈÛÙÒıËΠ·Ó·È-
Ì›· Î·È Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›· Ì ÈÎÙÂÚÈ΋
¯ÚÔÈ¿ ‰¤ÚÌ·ÙÔ˜, ÔfiÙ ·Ú·¤ÌÊıËΠÛÙÔ ÓÔ-
ÛÔÎÔÌÂ›Ô ÁÈ· ¤ÏÂÁ¯Ô.
∞fi ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËÎÂ
ˆ¯ÚfiÙËÙ·, ÈÎÙÂÚÈ΋ ¯ÚÔÈ¿ ‰¤ÚÌ·ÙÔ˜, Û˘ÛÙÔÏÈÎfi
ʇÛËÌ· 2/6, ‹·Ú „ËÏ·ÊËÙfi 3 cm, ÛÏ‹Ó·˜
„ËÏ·ÊËÙfi˜ 6,5 cm.
¶·È‰È·ÙÚÈ΋ 2006;69:463-467
¶›Ó·Î·˜ 2. ∂ÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ÔÈÎÔÁ¤ÓÂÈ·˜ 2Ô˘ ÂÚÈÛÙ·ÙÈÎÔ‡
¶¿Û¯ˆÓ ¶·Ù¤Ú·˜ ∞‰ÂÏÊfi˜
Hb (g/dl) 8,5 14,4 12,8Ht (%) 25,7 41,8 35,8MCV (fl) 84,3 89,3 72MCH (pg) 27,9 30,8 24,3ªÔÚÊÔÏÔÁ›· ÂÚ˘ıÚÒÓ ∞ÓÈÛÔ΢ÙÙ¿ÚˆÛË ++ ¶ÔÈÎÈÏÔ΢ÙÙ¿ÚˆÛË ±ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ¶ÔÈÎÈÏÔ΢ÙÙ¿ÚˆÛË ++ Àԯڈ̛· +
Àԯڈ̛· ++∂Ú˘ıÚÔ‚Ï¿ÛÙ˜ ~10%
¢Ú·ÓÔ·ÙÙ·Ú· +ºÂÚÚÈÙ›ÓË (ng/ml) 164,1 95,9 6,2∏b A 1,4% 55,3% 58,3%Hb F 21,4% 0,7% 1,7%HbD 42,6% 31,7%HbA2 3,8% 1,8%HbS 35,4% 33%∆ÂÛÙ ‰Ú¿ӈÛ˘ (+) (+) (-)
466 π. ∆Û¿ÙÚ· Î·È Û˘Ó.
√ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ (¶›Ó·Î·˜ 3) ¤‰ÂÈ-Í ‰ÈÏ‹ ÂÙÂÚÔ˙˘ÁˆÙ›· ‚-ª∞ Î·È ·ÈÌÔÛÊ·ÈÚÈ-ÓÔ¿ıÂÈ·˜ Lepore, ηٿÛÙ·ÛË Ô˘ ÂÎÊÚ¿˙ÂÙ·ÈÎÏÈÓÈο fiÌÔÈ· Ì ÔÌfi˙˘ÁÔ ‚-ª∞.
∆Ô ‚Ú¤ÊÔ˜ ‚Ú›ÛÎÂÙ·È Û ÚfiÁÚ·ÌÌ· Ù·ÎÙÈ-ÎÒÓ ÌÂÙ·ÁÁ›ÛˆÓ.
™˘˙‹ÙËÛË
∏ ·ÏÌ·Ù҉˘ ÚfiÔ‰Ô˜ Ù˘ ‚ÈÔÙ¯ÓÔÏÔÁ›·˜ÛÙÔÓ ÙÔ̤· Ù˘ Ù¯ÓËÙ‹˜ ÁÔÓÈÌÔÔ›ËÛ˘ ‰›ÓÂÈÙÂÚ¿ÛÙȘ ‰˘Ó·ÙfiÙËÙ˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘˘ÔÁÔÓÈÌfiÙËÙ·˜, ·ÏÏ¿ Ù·˘Ùfi¯ÚÔÓ· ı¤ÙÂÈ Î·ÈÔÏÏÔ‡˜ ÚÔ‚ÏËÌ·ÙÈÛÌÔ‡˜.
∏ ˘Ô‚ÔËıÔ‡ÌÂÓË ·Ó··Ú·ÁˆÁ‹, ÙfiÛÔ ÌÂÙË ÌÔÚÊ‹ Ù˘ ÛÂÚÌ·Ù¤Á¯˘Û˘, fiÛÔ Î·È Ì ÙËÌÔÚÊ‹ Ù˘ Â͈ۈ̷ÙÈ΋˜ ÁÔÓÈÌÔÔ›ËÛ˘,‚ÔËı¿ÂÈ Ù· ¿ÙÂÎÓ· ˙¢Á¿ÚÈ· ÛÙËÓ ·fiÎÙËÛ˷ȉÈÒÓ. √È ‰È¿ÊÔÚ˜ Ù¯ÓÈΤ˜ Â͈ۈ̷ÙÈ΋˜ÁÔÓÈÌÔÔ›ËÛ˘ ›ӷÈ: Ë Â͈ۈ̷ÙÈ΋ ÁÔÓÈÌÔ-Ô›ËÛË Î·È ÂÌ‚Ú˘ÔÌÂÙ·ÊÔÚ¿ (IVF/ET), Ë ÂÓ‰Ô-Û·ÏÈÁÁÈ΋ ÌÂÙ·ÊÔÚ¿ Á·ÌÂÙÒÓ (GIFT), Ë ÂÓ‰Ô-Û·ÏÈÁÁÈ΋ ÌÂÙ·ÊÔÚ¿ ˙˘ÁˆÙÒÓ (ZIFT) Î·È ËÂÓ‰Ô΢ÙÙ·ÚÔÏ·ÛÌÈ΋/ ÂÓ‰Ôˆ·ÚÈ΋ ¤Á¯˘ÛËÛÂÚÌ·ÙÔ˙ˆ·Ú›Ô˘ ‹ ·ÏÏÈÒ˜ “ÌÈÎÚÔÁÔÓÈÌÔÔ›-ËÛË” (ICSI) (2,3,4). ÃÚËÛÈÌÔÔÈÔ‡ÓÙ·È Î˘Ú›ˆ˜Ë IVF/ET Î·È Ë ICSI. ∏ ÙÂÏÂ˘Ù·›· ‰›ÓÂÈ ÙË ‰˘-Ó·ÙfiÙËÙ· ÁÔÓÈÌÔÔ›ËÛ˘ Î·È ·fiÎÙËÛ˘ ·È-‰ÈÒÓ ·fi ˙¢Á¿ÚÈ· ÙˆÓ ÔÔ›ˆÓ ÔÈ ¿Ó‰Ú˜ ¤¯Ô˘ÓÛÔ‚·Úfi Úfi‚ÏËÌ· ÛÙÔÓ ·ÚÈıÌfi, ÙËÓ ÎÈÓËÙÈÎfi-ÙËÙ· ‹ ÙË ÌÔÚÊÔÏÔÁ›· ÙˆÓ ÛÂÚÌ·ÙÔ˙ˆ·Ú›ˆÓÂÍ·Ï›ÊÔÓÙ·˜ ¤ÙÛÈ ÙËÓ ·Ó¿ÁÎË ¯Ú‹Û˘ ‰ˆÚË-ÙÒÓ Û¤ÚÌ·ÙÔ˜.
™ËÌ·ÓÙÈ΋ ÚfiÔ‰Ô˜ ¤¯ÂÈ Á›ÓÂÈ Î·È ÛÙÔÓ ÙÔ-̤· Ù˘ ÚÔÁÂÓÂÙÈ΋˜ ‰È¿ÁÓˆÛ˘. √ ηıÈÂÚˆ-̤ÓÔ˜ ÚÔÁÂÓÂÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ·ÔÙÂÏÂ›Ù·È ·fi
ÙËÓ ·ÌÓÈÔΤÓÙËÛË, ÙË ‚ÈÔ„›· ¯ÔÚÈ·ÎÒÓ Ï·¯ÓÒÓ(CVS), ÙËÓ ·Ó¿Ï˘ÛË DNA ÙÚÔÊÔ‚Ï¿ÛÙ˘. ™Â·Ú¯Èο ÛÙ¿‰È· ‚Ú›ÛÎÂÙ·È ÌÈ· Ó¤·, ÔÏÏ¿ ˘Ô-Û¯fiÌÂÓË Ì¤ıÔ‰Ô˜ Ë ÚÔÂÌÊ˘Ù¢ÙÈ΋ ÁÂÓÂÙÈ΋‰È¿ÁÓˆÛË (PGD). ∏ PGD ‰È·Ê¤ÚÂÈ ·fi ÙȘ Û˘-Ó‹ıÂȘ ÌÂıfi‰Ô˘˜, ηıÒ˜ ÚÔÛʤÚÂÈ ÛÙ· ˙¢Á¿-ÚÈ· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ·Ô-ʇÁÔ˘Ó ÌÈ· ·ËÛË ¿Û¯ÔÓÙÔ˜ ÂÌ‚Ú‡Ô˘, ηıÒ˜ÂÈÙÚ¤ÂÈ ÙËÓ ÂÈÏÔÁ‹ ÌË ·Û¯fiÓÙˆÓ ÂÌ‚Ú‡ˆÓÁÈ· ÂÌ‚Ú˘ÔÌÂÙ·ÊÔÚ¿. ∏ ̤ıÔ‰Ô˜ ·˘Ù‹ ÁÈ· Ù˯ÒÚ· Ì·˜ ¤¯ÂÈ ÙÂÚ¿ÛÙÈ· ÛËÌ·Û›·, ηıÒ˜ ÌÔ-Ú› Ó· ‰È·ÁÓÒÛÂÈ Ù· ¿Û¯ÔÓÙ· ·fi ÌÂÛÔÁÂȷοۇӉÚÔÌ· ¤Ì‚Ú˘· Î·È Ó· Ù· ·ÔÎÏ›ÛÂÈ ·fiÙËÓ ÂÌʇÙ¢ÛË (5,6).
øÛÙfiÛÔ, Ë Ù¯ÓËÙ‹ ÁÔÓÈÌÔÔ›ËÛË, ˆ˜ Ú·-Á‰·›· ·Ó·Ù˘ÛÛfiÌÂÓÔ˜ Ó¤Ô˜ ÙÔ̤·˜, ‰ÂÓ ¤¯ÂÈ·ÎfiÌ· Ï·ÈÛȈı› ·fi ÙÔ ··Ú·›ÙËÙÔ ÓÔÌÈÎfiÏ·›ÛÈÔ. ∫¤ÓÙÚ· ÁÔÓÈÌÔÔ›ËÛ˘ ȉڇÔÓÙ·È Î·ÈÙÔ ÛËÌ·ÓÙÈÎfiÙÂÚÔ, ÏÂÈÙÔ˘ÚÁÔ‡Ó ¯ˆÚ›˜ ıÂÛÌÔ-ıÂÙË̤ÓÔ˘˜ Î·È ÔÌÔÈÔÁÂÓ›˜ ηÓfiÓ˜ ÏÂÈÙÔ˘Ú-Á›·˜, Ô˘ Ó· ¤¯Ô˘Ó ÚÔ¤ÏıÂÈ ·fi ÙËÓ ·Ó·ÁÓˆ-ÚÈṲ̂ÓË ¤ÁÎÚÈÙË È·ÙÚÈ΋ ÎÔÈÓfiÙËÙ·. ™ÙËÓ∂ÏÏ¿‰· Û‹ÌÂÚ· ÏÂÈÙÔ˘ÚÁÔ‡Ó ÂÚ› Ù· 54 Τ-ÓÙÚ·, ηٷÓÂÌË̤ӷ Û ‰È¿ÊÔÚ˜ fiÏÂȘ, ËÏÂÈÔ„ËÊ›· ÙˆÓ ÔÔ›ˆÓ ·ÔÙÂÏÔ‡Ó È‰ÈˆÙÈΤ˜ÚˆÙÔ‚Ô˘Ï›Â˜.
÷ڷÎÙËÚÈÛÙÈο, ÛÙÔ ÚÒÙÔ ÂÚÈÛÙ·ÙÈÎfiÔ˘ ·Ó·Ê¤ÚıËÎÂ, ¤ÁÈÓ ÚÔÛ¿ıÂÈ· ·ʋ˜ ÌÂÙÔ Î¤ÓÙÚÔ fiÔ˘ ¤ÁÈÓÂ Ë Ù¯ÓËÙ‹ ÁÔÓÈÌÔÔ›ËÛË.∞ÔÙ¤ÏÂÛÌ· ‰ÂÓ ˘‹ÚÍÂ, ηıÒ˜ ÙÔ Î¤ÓÙÚÔ¤ÎÏÂÈÛ ÌÂÙ¿ ·fi Ï›ÁÔ˘˜ Ì‹Ó˜ ÏÂÈÙÔ˘ÚÁ›·˜,¯ˆÚ›˜ Ó· ‰È·ÙËÚËı› οÔÈÔ ·Ú¯Â›Ô. ∏ ‰È‡-ı˘ÓÛË ÙÔ˘ ΤÓÙÚÔ˘ ·Ó·ÊÂÚfiÙ·Ó fiÙÈ ·ÔÙÂÏ›ÙÔ·fi ·ÏÏÔ‰·Ô‡˜.
™·Ó ·ÔÙ¤ÏÂÛÌ·, ÛÙÔ ıÔÏfi ÙÔ›Ô Ô˘ ¤¯ÂȉËÌÈÔ˘ÚÁËı›, ÔÏϤ˜ ÊÔÚ¤˜ ÂÌÊ·Ó›˙ÔÓÙ·ÈÂÛÊ·Ï̤Ó˜ Ú·ÎÙÈΤ˜, Ô˘ Ô‰ËÁÔ‡Ó Û ·Ù˘¯‹
¶·È‰È·ÙÚÈ΋ 2006;69:463-467
¶›Ó·Î·˜ 3. ∂ÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ÔÈÎÔÁ¤ÓÂÈ·˜ 3Ô˘ ÂÚÈÛÙ·ÙÈÎÔ‡
¶¿Û¯ˆÓ ªËÙ¤Ú·
Hb (g/dl) 7,2 12,2Ht (%) 23 38,2MCV (fl) 58,4 70,3MCH (pg) 18,3 22,4ªÔÚÊÔÏÔÁ›· ∞ÓÈÛÔ΢ÙÙ¿ÚˆÛË +++ Àԯڈ̛· + ÂÚ˘ıÚÒÓ ¶ÔÈÎÈÏÔ΢ÙÙ¿ÚˆÛË +++ ∞ÓÈÛÔ΢ÙÙ¿ÚˆÛË +ÂÚÈÊÂÚÈÎÔ‡ ™ÙÔ¯Ô΢ÙÙ¿ÚˆÛË + ¶ÔÈÎÈÏÔ΢ÙÙ¿ÚˆÛË +·›Ì·ÙÔ˜ Àԯڈ̛· +++
µ·ÛÂfiÊÈÏÔ˜ ÛÙ›ÍË +¢∂∫ (%) 2,5%HbA 14%∏b A2 2% 5,1%∏b Lepore 12% (-)Hb F 70,6% <2%
467∞ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ ÌÂÙ¿ ·fi ˘Ô‚ÔËıÔ‡ÌÂÓË ÁÔÓÈÌÔÔ›ËÛË
·ÔÙÂϤÛÌ·Ù· fiˆ˜ ·˘Ù¿ ÙˆÓ ·Ó·ÊÂÚfiÌÂӈӷȉÈÒÓ. ∂›Ó·È ÂÈÙ·ÎÙÈ΋ ·Ó¿ÁÎË ÁÈ· ·˘ÛÙË-ÚfiÙÂÚÔ ¤ÏÂÁ¯Ô Û fiϘ ÙȘ ‰È·‰Èηۛ˜ ÙˆÓ Î¤-ÓÙÚˆÓ, ÂȉÈο ÛÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ‰ˆÚËÙÒÓ ÁÂÓÂ-ÙÈÎÔ‡ ˘ÏÈÎÔ‡. ÕÏψÛÙÂ Î·È Ù· ÙÚ›· ÂÚÈÛÙ·ÙÈÎ¿Ô˘ ÂÚÈÁÚ¿ÊËηÓ, ı· ›¯·Ó ¿ÏÏË Î·Ù¿ÏËÍË ·Ó›¯Â Á›ÓÂÈ ÏËÚ¤ÛÙÂÚÔ˜ ¤ÏÂÁ¯Ô˜ ÛÙÔ ÁÂÓÂÙÈÎfi˘ÏÈÎfi Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ. ∂ȉÈο ÛÙÔ ÙÚ›ÙÔÂÚÈÛÙ·ÙÈÎfi ÔÈ ÁÔÓ›˜ ¤ÏÂÍ·Ó Û˘ÓÂȉËÙ¿ Ù˯ڋÛË Í¤ÓÔ˘ ÁÂÓÂÙÈÎÔ‡ ˘ÏÈÎÔ‡, ÁÈ· Ó· ·Ôʇ-ÁÔ˘Ó Èı·Ó‹ Á¤ÓÓËÛË ·È‰ÈÔ‡ Ì ÛÔ‚·Ú‹ ·ÈÌÔ-ÛÊ·ÈÚÈÓÔ¿ıÂÈ·.
™Â ÔÏϤ˜ ¯ÒÚ˜ ÙÔ˘ Â͈ÙÂÚÈÎÔ‡ Á›ÓÔÓÙ·ÈÚÔÛ¿ıÂȘ Ó· ıÂÛÌÔıÂÙËıÔ‡Ó Û˘ÁÎÂÎÚÈ̤-ÓÔÈ Î·ÓfiÓ˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÁÈ· Ù· ΤÓÙÚ· Ù¯ÓË-Ù‹˜ ÁÔÓÈÌÔÔ›ËÛ˘, ÒÛÙ ӷ ·ÔʇÁÔÓÙ·È Ï¿-ıË fiˆ˜ Ù· ·Ú·¿Óˆ. ™ÙȘ ∏¶∞, ÚˆÙÔfiÚÔ¯ÒÚ· ÛÙËÓ ÂÊ·ÚÌÔÁ‹ ÙˆÓ Ó¤ˆÓ Ù¯ÓÈÎÒÓ, Á›ÓÂ-Ù·È ÂÂÍÂÚÁ·Û›· Î·È ¤Î‰ÔÛË Î·ÓfiÓˆÓ ÁÈ· ÙËÓÔÚı‹ Î·È ÂÓÈ·›· ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ Î¤ÓÙÚˆÓ Ù¯ÓË-Ù‹˜ ÁÔÓÈÌÔÔ›ËÛ˘ ·fi ÙËÓ American Societyfor Reproductive Medicine (7). ™ÙËÓ ∂˘Úˆ·˚-΋ ŒÓˆÛË ‰ÂÓ ˘¿Ú¯ÂÈ ÂÓÈ·›· ÓÔÌÔıÂÛ›· Î·È ÔÈÚ·ÎÙÈΤ˜ ÔÈΛÏÔ˘Ó ·fi ¯ÒÚ· Û ¯ÒÚ·. øÛÙfi-ÛÔ ¤¯Ô˘Ó Á›ÓÂÈ ÚÔÛ¿ıÂȘ ·Ó¿ÏÔÁ˜ Ì ÙȘ∏¶∞, Ì ÙËÓ ˘ÔÛÙ‹ÚÈÍË Î·È ÙËÓ ¯ÚËÌ·ÙÔ‰fi-ÙËÛË Ù˘ ∂˘Úˆ·˚΋˜ ∂ÈÙÚÔ‹˜, fiˆ˜ ÙÔÛ‡ÌʈÓÔ ÙÔ˘ Corsendonk (8).
™ÙËÓ ∂ÏÏ¿‰· ˆÛÙfiÛÔ ¤¯Ô˘Ì ÌÂÁ¿ÏÔ ÎÂÓfiÓÔÌÔıÂÛ›·˜. ∆Ô 1992 „ËÊ›ÛÙËΠÓfiÌÔ˜ (v.2071/92 ¿ÚıÚÔ 59 [º∂∫ 123/15-7-92]) Ô˘ ÚÔ-‚ϤÂÈ ÙË Û‡ÛÙ·ÛË Î·È ÏÂÈÙÔ˘ÚÁ›· ÌÔÓ¿‰ˆÓ ÙÂ-¯ÓËÙ‹˜ ÁÔÓÈÌÔÔ›ËÛ˘, ηıÒ˜ ›Û˘ Î·È ÙËӤΉÔÛË ¶ÚÔ‰ÚÈÎÔ‡ ¢È·Ù¿ÁÌ·ÙÔ˜ Ì ÙÔ ÔÔ›ÔηıÔÚ›˙ÔÓÙ·Ó “ÔÈ fiÚÔÈ Î·È ÔÈ ÚÔ¸Ôı¤ÛÂȘ Û˘-ÛÙ¿Ûˆ˜ Î·È ÏÂÈÙÔ˘ÚÁ›·˜ ÌÔÓ¿‰ˆÓ Ù¯ÓËÙ‹˜ ÁÔ-ÓÈÌÔÔ›ËÛ˘ ÙÔ˘ ·ÓıÚÒÔ˘ Î·È Î¿ı ÏÂÙÔ̤-ÚÂÈ· Û¯ÂÙÈο Ì ÙËÓ ËıÈ΋, ‰ÂÔÓÙÔÏÔÁÈ΋, ÓÔÌÈ-΋ Î·È ÔÈÎÔÓÔÌÈ΋ Ú‡ıÌÈÛË ÙÔ˘ fiÏÔ˘ ı¤Ì·ÙÔ˜”,ÙÔ ÔÔ›Ô ‰ÂÓ ¤¯ÂÈ ÂΉÔı› ·ÎfiÌË. ∆Ô 2002 ÂΉfi-ıËΠÓÔÌÔÛ¯¤‰ÈÔ (v. 3089/2002 [º∂∫ ∞ 327/23-12-2002] ÁÈ· ÙËÓ È·ÙÚÈο ˘Ô‚ÔËıÔ‡ÌÂÓË ·Ó·-·Ú·ÁˆÁ‹), Ô˘ Û˘ÌÏËÚÒÓÂÈ ÛÙÔȯ›· ÙÔ˘·ÛÙÈÎÔ‡ ÎÒ‰Èη, ¯ˆÚ›˜ fï˜ Ó· ·Ó·Ê¤ÚÂÙ·È ÛٷΤÓÙÚ· Ù¯ÓËÙ‹˜ ÁÔÓÈÌÔÔ›ËÛ˘. ∞fi ÏËÚÔÊÔ-ڛ˜ ÙÔ˘ ¤ÓÙ˘Ô˘ Î·È ËÏÂÎÙÚÔÓÈÎÔ‡ Ù‡Ô˘ Ê·›-ÓÂÙ·È fiÙÈ Â›ÎÂÈÙ·È „‹ÊÈÛË Û¯Â‰›Ô˘ ÓfiÌÔ˘ Ô˘ı· Ú˘ıÌ›˙ÂÈ fiϘ ÙȘ ÏÂÈÙÔ˘ÚÁ›Â˜ Î·È Ù· ÎÂÓ¿ ÙˆÓÚÔËÁËı¤ÓÙˆÓ ÓfiÌˆÓ Î·È ˘Ô˘ÚÁÈÎÒÓ ·ÔÊ¿-
ÛˆÓ. ™˘ÌÂÚ·ÛÌ·ÙÈο Û ÌÈ· ¯ÒÚ· Û·Ó ÙËÓ∂ÏÏ¿‰·, fiÔ˘ ÙÔ 7,5% ÙÔ˘ ÏËı˘ÛÌÔ‡ Ù˘ ›ӷÈÊÔÚ›˜ ‚-ª∞ Î·È fiÔ˘ ÌÂÙ¿ ·fi ÌÂÁ¿ÏË ÚÔ-Û¿ıÂÈ· Ù˘ ¶·ÁÎfiÛÌÈ·˜ √ÚÁ¿ÓˆÛ˘ ÀÁ›·˜ ÌÂÚÔÁÚ¿ÌÌ·Ù· ÚfiÏ˄˘ Ù˘ ÓfiÛÔ˘ ÂÈÙ‡¯ıË-Î·Ó ÙfiÛ· ÔÏÏ¿, Â›Ó·È ËıÈο Î·È ÂÈÛÙËÌÔÓÈο··Ú¿‰ÂÎÙÔ Ó· Á›ÓÔÓÙ·È ·ÚfiÌÔÈ· ÛÊ¿ÏÌ·Ù·.
∆Ô ÎÂÓfi Ô˘ ˘Ê›ÛÙ·Ù·È ·fi ÙÔÓ ÌË Î·ıÔÚÈ-ÛÌfi ̤¯ÚÈ Û‹ÌÂÚ· ·ÎÚÈ‚ÒÓ fiÚˆÓ ÏÂÈÙÔ˘Ú-Á›·˜ ÙˆÓ ÌÔÓ¿‰ˆÓ Ù¯ÓËÙ‹˜ ÁÔÓÈÌÔÔ›ËÛ˘ÚÔηÏ› ÎÈÓ‰‡ÓÔ˘˜ ÁÈ· ÙË ‰ËÌfiÛÈ· ˘Á›·. ∏¤ÏÏÂÈ„Ë Ô˘ÛÈ·ÛÙÈ΋˜ ÔÚÁ¿ÓˆÛ˘ ÙˆÓ ÂÌÏÂÎfi-ÌÂÓˆÓ ÊÔÚ¤ˆÓ Î·È Î·Ù¿ Û˘Ó¤ÂÈ· Ë ·‰˘Ó·Ì›·¿ÛÎËÛ˘ ·ÔÙÂÏÂÛÌ·ÙÈÎÔ‡ ÂϤÁ¯Ô˘, ·Ó·Ì¤ÓÂ-Ù·È Ó· ·˘Í‹ÛÂÈ ÙË Á¤ÓÓËÛË ·È‰ÈÒÓ Ì ·ÈÌÔ-ÛÊ·ÈÚÈÓÔ¿ıÂȘ. ¶Ú¤ÂÈ ÂÔ̤ӈ˜ Û‡ÓÙÔÌ·,Ó· Á›ÓÔ˘Ó ÔÈ ··Ú·›ÙËÙ˜ ÓÔÌÔıÂÙÈΤ˜ Ú˘ıÌ›-ÛÂȘ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÂÈÛÙËÌÔÓÈÎÒÓ,‰ÂÔÓÙÔÏÔÁÈÎÒÓ, ËıÈÎÒÓ, ÎÔÈÓˆÓÈÎÒÓ Î·È ÔÈÎÔ-ÓÔÌÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ ÙÔ˘ fiÏÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜.
µÈ‚ÏÈÔÁÚ·Ê›·
1. Kattamis C, Mallias A, Metaxotou-Mavromati A,Matsaniotis N. Screening for beta-thalassaemias.Lancet. 1981;2(8252):930.
2. Wen SW, Walker M., Léveillé MC, Leader A. Intracy-toplasmic sperm injection: promises and challenges.CMAJ 2004;171(8):845-846.
3. Toner J. Progress we can be proud of: U.S. trends inassisted reproduction over the first 20 years. FertilSteril 2002;78:943-950.
4. Van Voorhis BJ, Sparks AE, Allen BD, Stovall DW,Syrop CH, Chapler FK. Cost-effectiveness of infertil-ity treatments: a cohort study. Fertil Steril 1997;67:830-836.
5. Traeger-Synodinos J, Vrettou C, Palmer G, Tzetis M,Mastrominas M, Davies S et al. An evaluation of PGDin clinical genetic services through 3 years applicationfor prevention of beta-thalassaemia major and sicklecell thalassaemia. Mol Hum Reprod. 2003;9:301-307.
6. Kanavakis E, Traeger-Synodinos J. Preimplantationgenetic diagnosis in clinical practice. J Med Genet.2002;39:6-11.
7. The American Society for Reproductive Medicine.2002 guidelines for gamete and embryo donation: apractice committee report: guidelines and minimumstandards. Fertil Steril 2002;77 (Suppl 5):1.
8. Barratt C, Englert Y, Gottlieb C, Jouannet P. Gametedonation guidelines. The Corsendonk consensusdocument for the European Union. Hum Reprod.1998;13:500-501.
¶·È‰È·ÙÚÈ΋ 2006;69:463-467
468 ¶ƒ∞∫∆π∫√ £∂ª∞ PRACTICAL ISSUE
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
√‰ËÁ›Â˜ ÁÈ· ¿ÛÎËÛË Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ÌÂ
Û˘ÁÁÂÓ›˜ ‹ ›ÎÙËÙ˜ ·ı‹ÛÂȘ ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡
¢. °ÂˆÚÁ·ÎfiÔ˘ÏÔ˜, °. ¶··‰fiÔ˘ÏÔ˜
¶ÂÚ›ÏË„Ë: ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ Î·È ¤ÊË‚ÔÈ Û˘ÌÌÂÙ¤¯Ô˘Ó Û ÔÚÁ·Óˆ-̤Ó˜ ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜. ∆· ÔʤÏË ·fi ÙËÓ ÂÓ·Û¯fiÏËÛË ·˘Ù‹ Â›Ó·È ÔÏÏ¿ Î·È ÚÔÊ·Ó‹,ÙfiÛÔ Û ۈ̷ÙÈÎfi fiÛÔ Î·È Û „˘¯ÈÎfi ›‰Ô. ¶·Ú¿ÏÏËÏ· ¤¯ÂÈ ·˘ÍËı› Î·È Ô ·ÚÈıÌfi˜ ÙˆÓ ·È‰ÈÒÓÌ ηډÈÔ¿ıÂÈ· Ù· ÔÔ›· ÂÈ‚ÈÒÓÔ˘Ó Î·È Ù· ÔÔ›· Êı¿ÓÔ˘Ó ÛÙËÓ ÂÊË‚È΋ ‹ ÚÔÂÊË‚È΋ ËÏÈΛ· ÛÂηϋ ηٿÛÙ·ÛË Î·È Ù· ÔÔ›·, fiˆ˜ Â›Ó·È Â˘ÓfiËÙÔ, ÂÈı˘ÌÔ‡Ó Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó Î·È ·˘Ù¿ Ì·˙› ÌÂÙÔ˘˜ Û˘ÓÔÌËÏ›ÎÔ˘˜ ÙÔ˘˜ Û ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜. ¢˘ÛÙ˘¯Ò˜ fï˜, ÔÈ ÁÔÓ›˜ ·ÏÏ¿ Î·È ÌÂÁ¿ÏÔ̤ÚÔ˜ Ù˘ È·ÙÚÈ΋˜ ÎÔÈÓfiÙËÙ·˜ Â›Ó·È ‰ÈÛÙ·ÎÙÈÎÔ› ‹ ·ÎfiÌ· Î·È ÙÂÏ›ˆ˜ ·ÚÓËÙÈÎÔ› ÛÙÔ Ó· ÂÈÙÚ¤„Ô˘ÓÙËÓ ÂÓ·Û¯fiÏËÛË Ì ÙÔÓ ·ıÏËÙÈÛÌfi ÛÙ· ·È‰È¿ ·˘Ù¿, ÊÔ‚Ô‡ÌÂÓÔÈ ÙȘ Èı·Ó¤˜ ‰˘Û¿ÚÂÛÙ˜ Û˘Ó¤ÂÈ-˜. Ÿˆ˜ Ê·›ÓÂÙ·È, fï˜, ·fi ÙË Û¯ÂÙÈ΋ ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›·, Ë ¿ıÏËÛË ˘fi ÔÚÈṲ̂Ó˜ ÚÔ¸Ô-ı¤ÛÂȘ Â›Ó·È ·ÛÊ·Ï‹˜ ÁÈ· ÙË Û˘ÓÙÚÈÙÈ΋ ÏÂÈÔ„ËÊ›· ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ, ·ÎfiÌ· Î·È Ì ÙË ÌÔÚÊ‹Ù˘ ·ÓÙ·ÁˆÓÈÛÙÈ΋˜ Û˘ÌÌÂÙÔ¯‹˜ Î·È ÙÔ˘ ÚˆÙ·ıÏËÙÈÛÌÔ‡. ŸÌˆ˜, ·ÎfiÌ· Î·È fiÙ·Ó ‰ÂÓ ÂÈÙÚ¤ÂÙ·ÈË Û˘ÌÌÂÙÔ¯‹ Û ·ÓÙ·ÁˆÓÈÛÙÈο ·ıÏ‹Ì·Ù·, Â›Ó·È ÂÊÈÎÙ‹ Î·È ÂÈÙÚÂÙ‹ Ë ÂÓ·Û¯fiÏËÛË Ì οÔȘ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ Û ¯·Ï·Ú‹, „˘¯·ÁˆÁÈ΋ ÌÔÚÊ‹. ∆Ô ¿ÚıÚÔ ·˘Ùfi ÂȯÂÈÚ› Ó· Û˘Ì˘ÎÓÒ-ÛÂÈ Î·È Ó· ·ÚÔ˘ÛÈ¿ÛÂÈ Û ڷÎÙÈ΋ ÌÔÚÊ‹ Ù· Û¯ÂÙÈο Û˘ÌÂÚ¿ÛÌ·Ù· Î·È Ô‰ËÁ›Â˜ ‰ÈÂıÓÒÓ ÂÈÙÚÔ-ÒÓ Ô˘ ·ÊÔÚÔ‡Ó ÛÙËÓ ¿ıÏËÛË ·È‰ÈÒÓ Ì ÙȘ Û˘¯ÓfiÙÂÚ˜ Û˘ÁÁÂÓ›˜ ‹ ›ÎÙËÙ˜ ·ı‹ÛÂȘ ÙÔ˘ ΢-ÎÏÔÊÔÚÈÎÔ‡.
§¤ÍÂȘ ÎÏÂȉȿ: ÕÛÎËÛË, ηډÈÔ¿ıÂȘ.
Exercise recommendations for children and adolescents with
congenital and acquired cardiovascular disorders
D. Georgakopoulos, G. Papadopoulos
Abstract: During the last decade the numbers of children and adolescents participating in organizedathletic activities have increased. The benefits from this participation are multiple and obvious. Over thesame time, the number of children with cardiovascular abnormalities who survive and reachadolescence in relatively good condition is also increasing. These children, as it is to be expected, arekeen to participate in sports activities along with their healthy peers. Unfortunately, most of the parentstaking care of these children and the physicians responsible for their monitoring are reluctant to allowthem to exercise, let alone compete with other children. This reluctance is not supported by the relevantbibliography, which demonstrates that for the vast majority of these children some form of exercise isallowable, following careful, thorough and regular evaluation of their condition. Some of these childrenand adolescents can even participate in competitive sports, but even when this is not possible, certainforms of recreational sports activity may be well tolerated, without risk for the children. This reviewsummarizes and presents in a practical way the present state of knowledge and the recommendations ofinternational committees regarding the participation in sports of children and adolescents withcardiovascular abnormalities.
Key words: Exercise, cardiovascular disease.
∫·Ú‰ÈÔÏÔÁÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ∞ıËÓÒÓ, “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”
AÏÏËÏÔÁÚ·Ê›·:
¢ËÌ‹ÙÚ˘ °ÂˆÚÁ·ÎfiÔ˘ÏÔ˜∏Úȉ·ÓÔ‡ 16, ∆.∫. 115 28, ∞ı‹Ó· E-mail: [email protected]
∏ÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 20-03-2006∏ÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 10-10-2006
Cardiology Department, “P. & A. Kyriakou” Children’sHospital, Athens, Greece
Correspondence:
Dimitris Georgakopoulos16, Iridanou St., 115 28 Athens, Greece E-mail:[email protected]
Date of submission: 20-03-2006Date of approval: 10-10-2006
∂ÈÛ·ÁˆÁ‹
∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, ÏfiÁˆ ÙˆÓ ÛËÌ·ÓÙÈÎÒÓ
ÂÍÂÏ›ÍÂˆÓ ÛÙÔÓ ÙÔ̤· Ù˘ ¯ÂÈÚÔ˘ÚÁÈ΋˜ ηÈ
ÂÂÌ‚·ÙÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ Û˘ÁÁÂÓÒÓ
ηډÈÔ·ıÂÈÒÓ, ¤Ó·˜ ‰È·ÚÎÒ˜ ·˘Í·ÓfiÌÂÓÔ˜
·ÚÈıÌfi˜ ·ÛıÂÓÒÓ Êı¿ÓÂÈ ÛÙËÓ ÂÊ˂›· Î·È ÛÙËÓ
ÂÓ‹ÏÈÎË ˙ˆ‹ Û ÈηÓÔÔÈËÙÈ΋ ¤ˆ˜ Ôχ ηϋ
469ÕÛÎËÛË Î·È ·ı‹ÛÂȘ ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡
Ê˘ÛÈ΋ ηٿÛÙ·ÛË Î·È Ì fiϘ ÙȘ ÚÔ¸Ôı¤-
ÛÂȘ ÁÈ· Ó· ÌÔÚ› Ó· ˙‹ÛÂÈ ÌÈ· fiÛÔ ÙÔ ‰˘Ó·ÙfiÓ
ÈÔ Ê˘ÛÈÔÏÔÁÈ΋ ˙ˆ‹. Ÿˆ˜ Â›Ó·È ·Ó·ÌÂÓfiÌÂ-
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‰Ú·ÛÙËÚÈfiÙËÙ˜, ÁÂÁÔÓfi˜ Ô˘ ı· ÙÔ˘˜ ÚÔÛʤ-
ÚÂÈ ÛËÌ·ÓÙÈο ÔʤÏË ÙfiÛÔ ÛÙË ÛˆÌ·ÙÈ΋ fiÛÔ
Î·È ÛÙËÓ „˘¯È΋ ÙÔ˘˜ ˘Á›·. ∏ ÁÂÓÈÎfiÙÂÚË fï˜
·ÓÙ›ÏË„Ë, ÙfiÛÔ ÙˆÓ ÁÔÓ¤ˆÓ ÙˆÓ ·ÛıÂÓÒÓ ·˘-
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fiÙÈ ÏfiÁˆ Ù˘ ˘¿Ú¯Ô˘Û·˜ ηډÈÔ¿ıÂÈ·˜, ‰ÈÔÚ-
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Ù·È Ó· ·ıÏÔ‡ÓÙ·È Ì ÙÔÓ Êfi‚Ô ÙÔ˘ ·ÈÊÓ›‰ÈÔ˘ ı·-
Ó¿ÙÔ˘ ‹ Ù˘ Âȉ›ӈÛ˘ Ù˘ ηٿÛÙ·Û‹˜ ÙÔ˘˜.
∏ ·ÓÙ›ÏË„Ë ·˘Ù‹ ÂÓÈÛ¯‡ÂÙ·È Î·È ·fi ÙË ÌÂÁ¿ÏË
‰ËÌÔÛÈfiÙËÙ· Ô˘ Ï·Ì‚¿ÓÔ˘Ó Ù· Û¿ÓÈ· ÂÚÈ-
ÛÙ·ÙÈο ·ÈÊÓ›‰ÈˆÓ ı·Ó¿ÙˆÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó
ηٿ ÙËÓ ¿ÛÎËÛË Ó·ÚÒÓ ·ıÏËÙÒÓ (1). Ÿˆ˜
fï˜ Ê·›ÓÂÙ·È ·fi ÙË Û¯ÂÙÈ΋ ‚È‚ÏÈÔÁÚ·Ê›·, Ë
Û˘ÓÙÚÈÙÈ΋ ÏÂÈÔ„ËÊ›· ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ
ÌÔÚÔ‡Ó Ó· Ï·Ì‚¿ÓÔ˘Ó Ì¤ÚÔ˜ Û οÔÈ· ·ıÏË-
ÙÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·, ¯ˆÚ›˜ Êfi‚Ô ÁÈ· ÙËÓ ˘Á›·
ÙÔ˘˜, Ì ÙËÓ ÚÔ¸fiıÂÛË, ‚‚·›ˆ˜, fiÙÈ ı·
ÚÔËÁÂ›Ù·È Î¿ı ÊÔÚ¿ Ô ÂӉ‰ÂÈÁ̤ÓÔ˜ ¤ÏÂÁ¯Ô˜
Î·È ı· Û˘ÓÈÛÙ¿Ù·È Ë Î·Ù¿ÏÏËÏË ÁÈ· οı ·ÛıÂ-
Ó‹ ‰Ú·ÛÙËÚÈfiÙËÙ· (2). ™ÎÔfi˜ ÏÔÈfiÓ ÙÔ˘ ·-
ÚfiÓÙÔ˜ ¿ÚıÚÔ˘ Â›Ó·È Ó· Û˘ÁÎÂÓÙÚÒÛÂÈ Î·È Ó·
·ÚÔ˘ÛÈ¿ÛÂÈ ÂÚÈÏËÙÈο Î·È Ì ۷ʋÓÂÈ· ÙȘ
˘¿Ú¯Ô˘Û˜ Ô‰ËÁ›Â˜ ÁÈ· ¿ÛÎËÛË Ô˘ ·ÊÔÚÔ‡Ó
ÙȘ Û˘¯ÓfiÙÂÚ˜ ·ı‹ÛÂȘ ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡
Û˘ÛÙ‹Ì·ÙÔ˜, Û˘ÁÁÂÓ›˜ ‹ ÌË, ÔÈ Ôԛ˜ ··ÓÙÒ-
ÓÙ·È Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜.
∂›‰Ë ¿ÛÎËÛ˘ - ∆·ÍÈÓfiÌËÛË ·ıÏËÌ¿ÙˆÓ
∏ ¿ÛÎËÛË ‰È·ÎÚ›ÓÂÙ·È Û ‰‡Ô ‚·ÛÈÎÔ‡˜ Ù‡-
Ô˘˜, ÙË ‰˘Ó·ÌÈ΋ Î·È ÙË ÛÙ·ÙÈ΋ (3,4). ™ÙË
‰˘Ó·ÌÈ΋ ¿ÛÎËÛË Á›ÓÔÓÙ·È Ú˘ıÌÈΤ˜ Û˘Û¿-
ÛÂȘ ÙˆÓ ÂÌÏÂÎfiÌÂÓˆÓ Ì˘˚ÎÒÓ ÔÌ¿‰ˆÓ ÌÂ
ÌÂÙ·‚ÔϤ˜ ÛÙÔ Ì‹ÎÔ˜ Ù˘ Ì˘˚΋˜ ›Ó·˜ Î·È ÌÂ
·Ó¿Ù˘ÍË ÌÈÎÚ‹˜ ÂÓ‰ÔÌ˘˚΋˜ Ù¿Û˘. ∞ÓÙ›ıÂ-
Ù·, ÛÙË ÛÙ·ÙÈ΋ ¿ÛÎËÛË ·Ú·ÙËÚÂ›Ù·È ÌÂÁ¿ÏË
·‡ÍËÛË Ù˘ ÂÓ‰ÔÌ˘˚΋˜ Ù¿Û˘ Ì Ôχ ÌÈÎÚ‹
ÌÂÙ·‚ÔÏ‹ ÛÙÔ Ì‹ÎÔ˜ Ù˘ Ì˘˚΋˜ ›Ó·˜. Ÿˆ˜
Â›Ó·È ·Ó·ÌÂÓfiÌÂÓÔ, ÔÈ ÂÈÙÒÛÂȘ ÛÙÔ Î·Ú-
‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· Â›Ó·È ‰È·ÊÔÚÂÙÈΤ˜ ÛÂ
οıÂ Ù‡Ô ¿ÛÎËÛ˘. ∏ ‰˘Ó·ÌÈ΋ ¿ÛÎËÛË
ÚÔηÏ› ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË Ù˘ ηډȷ΋˜
Û˘¯ÓfiÙËÙ·˜, ÙÔ˘ fiÁÎÔ˘ ·ÏÌÔ‡, Ù˘ ηډȷ-
΋˜ ·ÚÔ¯‹˜ Î·È Ù˘ Û˘ÛÙÔÏÈ΋˜ ·ÚÙËÚȷ΋˜
›ÂÛ˘ Î·È ÙÒÛË ÙˆÓ ÔÏÈÎÒÓ ÂÚÈÊÂÚÈÎÒÓ
·ÓÙÈÛÙ¿ÛÂˆÓ Î·È Ù˘ ‰È·ÛÙÔÏÈ΋˜ ·ÚÙËÚȷ΋˜
›ÂÛ˘. ∞ÔÙ¤ÏÂÛÌ· fiÏˆÓ ·˘ÙÒÓ ÙˆÓ ÌÂÙ·-
‚ÔÏÒÓ Â›Ó·È Ë ÚfiÎÏËÛË ÛÙËÓ Î·Ú‰È¿ ˘ÂÚ-
ÊfiÚÙˆÛ˘ fiÁÎÔ˘. ∏ ÛÙ·ÙÈ΋ ¿ÛÎËÛË ÚÔη-
Ï› ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÙfiÛÔ Ù˘ Û˘ÛÙÔÏÈ΋˜
fiÛÔ Î·È Ù˘ ‰È·ÛÙÔÏÈ΋˜ ·ÚÙËÚȷ΋˜ ›ÂÛ˘,
Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ˘ÂÚÊfiÚÙˆÛË ›ÂÛ˘ ÙÔ˘
΢ÎÏÔÊÔÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ¡· ÛËÌÂȈı› fiÙÈ
ηٿ ÙË ‰˘Ó·ÌÈ΋ ¿ÛÎËÛË ÌÂÁ¿ÏˆÓ Ì˘˚ÎÒÓ
ÔÌ¿‰ˆÓ Û ˘„ËÏ¿ ›‰· ·ÓÙ›ÛÙ·Û˘ (.¯.
·ÁˆÓÈÛÙÈ΋ Ô‰ËÏ·Û›·), ÔÈ ÂÈÙÒÛÂȘ ÛÙÔ
΢ÎÏÔÊÔÚÈÎfi Â›Ó·È ·ÚfiÌÔȘ Ì ·˘Ù¤˜ Ô˘
·Ú·ÙËÚÔ‡ÓÙ·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ¿ÛÎË-
Û˘ ÛÙ·ÙÈÎÔ‡ Ù‡Ô˘ (5).
Ÿˆ˜ Â›Ó·È Î·Ù·ÓÔËÙfi, ÔÏÏ¿ ·ıÏ‹Ì·Ù·
ÂÚȤ¯Ô˘Ó Û˘ÓÈÛÙÒÛ˜ Î·È ÙˆÓ ‰‡Ô Ù‡ˆÓ
¿ÛÎËÛ˘, Û ‰È·ÊÔÚÂÙÈ΋ ‚‚·›ˆ˜ ·Ó·ÏÔÁ›·
ÙÔ Î·ı¤Ó· (6). °È· ÏfiÁÔ˘˜ Ù·ÍÈÓfiÌËÛ˘ ‰È·-
ÎÚ›ÓÔ˘Ì ÙÚ›· ›‰· ¤ÓÙ·Û˘ (¯·ÌËÏfi, ̤-
ÙÚÈÔ Î·È ˘„ËÏfi) ÁÈ· οı ¤Ó·Ó ·fi ÙÔ˘˜ Ù‡-
Ô˘˜ ¿ÛÎËÛ˘ (‰˘Ó·ÌÈ΋ ‹ ÛÙ·ÙÈ΋) Î·È ¤ÙÛÈ
οı ¿ıÏËÌ· ÙÔÔıÂÙÂ›Ù·È ÛÙËÓ ·Ó¿ÏÔÁË ı¤-
ÛË ÛÙÔÓ Û¯ÂÙÈÎfi ›Ó·Î· (¶›Ó·Î·˜ 1). ¶·Ú·-
‰Â›ÁÌ·ÙÔ˜ ¯¿ÚÈÓ, ÙÔ bowling Ù·ÍÈÓÔÌÂ›Ù·È ÛÙËÓ
ηÙËÁÔÚ›· π∞, Ô˘ ÛËÌ·›ÓÂÈ fiÙÈ Î·Ù¿ ÙËÓ
ÂÎÙ¤ÏÂÛ‹ ÙÔ˘ ··ÈÙÂ›Ù·È ¿ÛÎËÛË ¯·ÌËÏ‹˜
¤ÓÙ·Û˘ ÙfiÛÔ Û ‰˘Ó·ÌÈÎfi fiÛÔ Î·È Û ÛÙ·ÙÈÎfi
ÛÙÔȯ›Ô. ∞ÓÙ›ıÂÙ·, Ë ÎˆËÏ·Û›· ÙÔÔıÂÙ›-
Ù·È ÛÙËÓ Î·ÙËÁÔÚ›· πππ°, Ô˘ Û˘Ó¿ÁÂÙ·È
˘„ËÏ‹˜ ¤ÓÙ·Û˘ ‰˘Ó·ÌÈ΋ Î·È ÛÙ·ÙÈ΋ ÚÔ-
Û¿ıÂÈ·. ∞Ó Î·È ‰ÂÓ ·Ó·Ê¤ÚÂÙ·È ÛÙÔÓ Û¯ÂÙÈÎfi
›Ó·Î·, ı· ÌÔÚÔ‡Û ӷ ÚÔÛÙÂı› Î·È ÙÔ
̷ϤÙÔ, ÌÈ· ¿ÛÎËÛË ‹È·˜ ÌÔÚÊ‹˜, ȉȷ›ÙÂÚ·
ÚÔÛÊÈÏ‹˜ ÛÙ· ÎÔÚ›ÙÛÈ·. ∂›Û˘ ÌÈ· ¿ÏÏË Î·-
ÙËÁÔÚ›· Ô˘ ÂӉȷʤÚÂÈ ÙÔ˘˜ ·ÛıÂÓ›˜ ÌÂ
Û˘ÁÁÂÓ›˜ ηډÈÔ¿ıÂȘ Â›Ó·È Ù· ·ıÏ‹Ì·Ù·
ÂΛӷ Ù· ÔÔ›· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó-
‰˘ÓÔ ÛˆÌ·ÙÈ΋˜ ÚfiÛÎÚÔ˘Û˘, Ë ÔÔ›· ‚Â-
‚·›ˆ˜ ÂÁ΢ÌÔÓ› ÛÔ‚·ÚÔ‡˜ ÎÈÓ‰‡ÓÔ˘˜ ÁÈ· ÔÚÈ-
Ṳ̂ÓÔ˘˜ ·fi ·˘ÙÔ‡˜ (¶›Ó·Î·˜ 2).
™˘ÁÁÂÓ›˜ ηډÈÔ¿ıÂȘ (7)
ªÂÛÔÎÔÏÈ΋ ÂÈÎÔÈÓˆÓ›·: ŸÙ·Ó Ë ÂÈÎÔÈÓˆ-
Ó›· Â›Ó·È ÌÈÎÚÔ‡ ÌÂÁ¤ıÔ˘˜ (·Ô˘Û›· ‰È¿Ù·Û˘
Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜), Ë Û˘ÌÌÂÙÔ¯‹ Â›Ó·È ÂχıÂ-
ÚË Û fiÏ· Ù· ·ıÏ‹Ì·Ù·. ŸÙ·Ó Ë ÂÈÎÔÈÓˆÓ›· ›-
Ó·È ·ÈÌÔ‰˘Ó·ÌÈο ÛËÌ·ÓÙÈ΋, Ì ‰È¿Ù·ÛË Ù˘
‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È Ê˘ÛÈÔÏÔÁÈ΋, fï˜, ›ÂÛË
ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›·, ÙfiÙ ÂÈÙÚ¤ÂÙ·È Ë
Û˘ÌÌÂÙÔ¯‹ Û fiÏ· Ù· ·ıÏ‹Ì·Ù·. ŸÙ·Ó ˘¿Ú-
¯ÂÈ Ì¤ÙÚÈ· Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË, ÂÈÙÚ¤ÂÙ·È
Ë Û˘ÌÌÂÙÔ¯‹ Û ·ıÏ‹Ì·Ù· ηÙËÁÔÚ›·˜ π∞.
ŸÙ·Ó Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Â›Ó·È ÛÔ‚·Ú‹,
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
470 ¢. °ÂˆÚÁ·ÎfiÔ˘ÏÔ˜, °. ¶··‰fiÔ˘ÏÔ˜
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
Ì ÂΉ‹ÏˆÛË Î˘·ÓÒÛˆ˜, ÙfiÙ ··ÁÔÚ‡ÂÙ·ÈË Û˘ÌÌÂÙÔ¯‹ Û ·ÓÙ·ÁˆÓÈÛÙÈο ·ıÏ‹Ì·Ù·.
ªÂÛÔÎÔÈÏȷ΋ ÂÈÎÔÈÓˆÓ›·: ÕÙÔÌ· Ì ÌÈÎÚ¤˜‹ ̤ÙÚȘ ÂÈÎÔÈӈӛ˜ (Qp/Qs = 1,5-1,9) ÌÔ-ÚÔ‡Ó Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó ÂχıÂÚ· Û fiÏ· Ù··ıÏ‹Ì·Ù·. ŸÙ·Ó Ë ÂÈÎÔÈÓˆÓ›· Â›Ó·È ÌÂÁ¿ÏË(Qp/Qs >2) ÙfiÙ ÂÈÙÚ¤ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ Û·ıÏ‹Ì·Ù· ηÙËÁÔÚ›·˜ π∞. ∫·È ÛÙȘ ‰‡Ô ·˘Ù¤˜·ı‹ÛÂȘ, 6 Ì‹Ó˜ ÌÂÙ¿ ÙË ‰ÈfiÚıˆÛË Â›Ù ·˘Ù‹Á›ÓÂÈ ÂÂÌ‚·ÙÈο, ›Ù ¯ÂÈÚÔ˘ÚÁÈο, ÂÈÙÚ¤Â-
Ù·È ÂχıÂÚ· Ë ¿ıÏËÛË ÂÊfiÛÔÓ ÔÈ ·ÛıÂÓ›˜ ›-Ó·È ·Û˘Ìو̷ÙÈÎÔ› Î·È ‰ÂÓ ÂÌÊ·Ó›˙Ô˘Ó ·Ú-Ú˘ıÌ›·, Ì˘Ôηډȷ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Î·È ÂӉ›-ÍÂȘ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘.
∞ÓÔÈÎÙfi˜ ‚ÔÙ¿ÏÂÈÔ˜ fiÚÔ˜: ∂› ÌÈÎÚÔ‡ fiÚÔ˘Ë ¿ıÏËÛË Â›Ó·È ÂχıÂÚË. ŸÙ·Ó Ô fiÚÔ˜ ›ӷȷÈÌÔ‰˘Ó·ÌÈο ÛËÌ·ÓÙÈÎfi˜ (‰È¿Ù·ÛË Ù˘ ·ÚÈ-ÛÙÂÚ¿˜ ÎÔÈÏ›·˜), ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ Ë Î·ÙËÁÔÚ›·π∞. ∆ÚÂȘ Ì‹Ó˜ ÌÂÙ¿ ÙËÓ ÂÂÌ‚·ÙÈ΋ Û‡ÁÎÏÂÈ-ÛË Î·È ¤ÍÈ Ì‹Ó˜ ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋, ÂÈÙÚ¤-ÔÓÙ·È fiÏ· Ù· ·ıÏ‹Ì·Ù· ÂÊfiÛÔÓ ÈÛ¯‡Ô˘Ó ÔÈÚÔ¸Ôı¤ÛÂȘ Ô˘ ·Ó·Ê¤ÚıËÎ·Ó Î·È ÁÈ· ÙȘ¿ÏϘ ‰‡Ô ÂÈÎÔÈӈӛ˜.
™Ù¤ÓˆÛË Ó¢ÌÔÓÈ΋˜ ‚·Ï‚›‰·˜: ŸÙ·Ó Ë ÎÏ›-ÛË ›ÂÛ˘ (Doppler ̤ÙÚËÛË) Â›Ó·È <40 mmHgÎ·È Ë ÏÂÈÙÔ˘ÚÁ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Â›Ó·È Ê˘-ÛÈÔÏÔÁÈ΋, ÂÈÙÚ¤ÔÓÙ·È fiÏ· Ù· ·ıÏ‹Ì·Ù·.ŸÙ·Ó Ë ÎÏ›ÛË ›ÂÛ˘ ˘Âڂ› Ù· 40 mmHg,ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ Ë Î·ÙËÁÔÚ›· π∞ Î·È πµ, Î·È Ô·ÛıÂÓ‹˜ Û˘Ó‹ıˆ˜ ·Ú·¤ÌÂÙ·È ÁÈ· ‚·Ï‚ȉÔ-Ï·ÛÙÈ΋. ŒÓ· Ì‹Ó· ÌÂÙ¿ ÙË ‚·Ï‚ȉÔÏ·ÛÙÈ΋Ì ̷ÏfiÓÈ Î·È ÙÚÂȘ Ì‹Ó˜ ÌÂÙ¿ ÙÔ ¯ÂÈÚÔ˘Ú-ÁÂ›Ô Ë ¿ıÏËÛË Â›Ó·È ÂχıÂÚË, ÂÊfiÛÔÓ ‰ÂÓ˘¿Ú¯Ô˘Ó Û˘ÌÙÒÌ·Ù· Î·È Ë ÏÂÈÙÔ˘ÚÁ›· Ù˘ÎÔÈÏ›·˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋.
™Ù¤ÓˆÛË ·ÔÚÙÈ΋˜ ‚·Ï‚›‰·˜: ∞ÛıÂÓ›˜ Ì‹ȷ ÛÙ¤ÓˆÛË (Doppler ÎÏ›ÛË ›ÂÛ˘ <20mmHg) Û˘ÌÌÂÙ¤¯Ô˘Ó Û fiÏ· Ù· ·ıÏ‹Ì·Ù·, ·Ó‰ÂÓ ·Ó·Ê¤ÚÔ˘Ó Û˘ÌÙÒÌ·Ù· Û¯ÂÙÈ˙fiÌÂÓ· ÌÂÙËÓ ¿ıÏËÛË Î·È Ù· ∏∫° ËÚÂÌ›·˜ Î·È ÎfiˆÛ˘
¶›Ó·Î·˜ 1. ∆·ÍÈÓfiÌËÛË ·ıÏËÌ¿ÙˆÓ
∞ µ °
π - ÌfiÔ˘ÏÈÓÁÎ - ÂÈÙÚ·¤˙È· ·ÓÙÈÛÊ·›ÚÈÛË - Ô‰fiÛÊ·ÈÚÔ- ÁÎÔÏÊ - Ù¤ÓȘ (‰ÈÏfi) - Ù¤ÓȘ
- ÛÎÔÔ‚ÔÏ‹ - ‚fiÏÂ˚ - ÛÎÔ˘fi˜- ‰ÚfiÌÔÈ ÌÂÁ¿ÏˆÓ ·ÔÛÙ¿ÛˆÓ
ππ - ÙÔÍÔ‚ÔÏ›· - ÍÈÊ·ÛΛ· - Ì¿ÛÎÂÙ- È·Û›·* - ÛÙ›‚Ô˜ (¿ÏÌ·Ù·) - ¯fiÎÂ˚ › ¿ÁÔ˘
- ηٷ‰‡ÛÂȘ* - ÙÚ¤ÍÈÌÔ ÛÚÈÓÙ - ÎÔχ̂ËÛË- ·ÁÒÓ˜ ·˘ÙÔÎÈÓ‹ÙˆÓ Î·È - ηÏÏÈÙ¯ÓÈÎfi ·ÙÈÓ¿˙ - ¯¿ÓÙÌÔÏ
ÌÔÙÔÛÈÎÏÂÙÒÓ* - Û˘Á¯ÚÔÓÈṲ̂ÓË ÎÔχ̂ËÛË*- Û¤ÚÊÈÓÁÎ
πππ - ÛÙ›‚Ô˜ (Ú›„ÂȘ) - ¿ÏË - ˘ÁÌ·¯›·- Á˘ÌÓ·ÛÙÈ΋* - ÛÎÈ ‚Ô˘ÓÔ‡* - ηÓfi ηÁÈ¿Î
- ηڿÙ - Ô‰ËÏ·Û›·*- ÈÛÙÈÔÏÔ˝· - ‰¤Î·ıÏÔ
- ·Ó·ÚÚ›¯ËÛË* - ΈËÏ·Û›·- ı·Ï¿ÛÛÈÔ ÛÎÈ* - ·ÁÔ‰ÚƠ̂˜- ¿ÚÛË ‚·ÚÒÓ*- ÈÛÙÈÔÛ·Ó›‰·*
π = ¯·ÌËÏ‹ ÛÙ·ÙÈ΋, ππ = ̤ÙÚÈ· ÛÙ·ÙÈ΋, πππ = ˘„ËÏ‹ ÛÙ·ÙÈ΋, ∞ = ¯·ÌËÏ‹ ‰˘Ó·ÌÈ΋, µ = ̤ÙÚÈ· ‰˘Ó·ÌÈ΋, ° = ˘„ËÏ‹ ‰˘Ó·ÌÈ΋, * = ·˘ÍË̤ÓÔ˜ ΛӉ˘ÓÔ˜ Û˘ÁÎÔ‹˜
¶›Ó·Î·˜ 2. ∞ıÏ‹Ì·Ù· Ì ΛӉ˘ÓÔ ÛˆÌ·ÙÈ΋˜ ÚfiÛÎÚÔ˘Û˘
1. ¶Ô‰fiÛÊ·ÈÚÔ2. π·Û›·3. ∫·Ù·‰‡ÛÂȘ4. ∞ÁÒÓ˜ ·˘ÙÔÎÈÓ‹ÙˆÓ5. ∞ÁÒÓ˜ ÌÔÙÔÛÈÎÏÂÙÒÓ6. ∫·ÏÏÈÙ¯ÓÈÎfi ·ÙÈÓ¿˙7. ™˘Á¯ÚÔÓÈṲ̂ÓË ÎÔχ̂ËÛË8. ™¤ÚÊÈÓÁÎ9. ª¿ÛÎÂÙ10. ÃfiÎÂ˚ › ¿ÁÔ˘11. °˘ÌÓ·ÛÙÈ΋12. ∫·Ú¿ÙÂ13. ∞Ó·ÚÚ›¯ËÛË14. £·Ï¿ÛÛÈÔ ÛÎÈ15. ÕÚÛË ‚·ÚÒÓ16. πÛÙÈÔÛ·Ó›‰·17. ™ÎÈ ‚Ô˘ÓÔ‡18. ¶¿ÏË19. ¶˘ÁÌ·¯›·20. ¶Ô‰ËÏ·Û›·
Â›Ó·È Ê˘ÛÈÔÏÔÁÈο. ŸÙ·Ó Ë ÛÙ¤ÓˆÛË Â›Ó·È Ì¤-
ÙÚÈ· (ÎÏ›ÛË ›ÂÛ˘ 21-49 mmHg) ÂÈÙÚ¤ÂÙ·È
Ë Û˘ÌÌÂÙÔ¯‹ Û ·ıÏ‹Ì·Ù· ÙˆÓ Î·ÙËÁÔÚÈÒÓ π∞,
πµ Î·È ππ∞ ÂÊfiÛÔÓ ‰ÂÓ ˘¿Ú¯Ô˘Ó Û˘ÌÙÒÌ·Ù·,
‰ÂÓ ˘¿Ú¯ÂÈ strain ÛÙÔ ∏∫° ËÚÂÌ›·˜, ÛÙÔ Ë¯Ô-
ηډÈÔÁÚ¿ÊËÌ· ˘¿Ú¯ÂÈ ÌfiÓÔ ‹È· ˘ÂÚÙÚÔ-
Ê›· Ù˘ ·ÚÈÛÙÂÚ¿˜ ÎÔÈÏ›·˜ Î·È Ë ‰ÔÎÈÌ·Û›· ÎÔ-
ÒÛˆ˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋. ∞ÛıÂÓ›˜ Ì ÛÔ‚·-
Ú‹ ÛÙ¤ÓˆÛË (ÎÏ›ÛË ›ÂÛ˘ >50 mmHg) ‰ÂÓ
ÂÈÙÚ¤ÂÙ·È Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó Û ·ÓÙ·ÁˆÓÈÛÙÈ-
ο ·ıÏ‹Ì·Ù·. ∆ÚÂȘ Ì‹Ó˜ ÌÂÙ¿ ÙË ‚·Ï‚ȉÔ-
Ï·ÛÙÈ΋ ‹ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ‰È¿ÓÔÈÍË ÂÈÙÚ¤Â-
Ù·È Ë ÂÓ·Û¯fiÏËÛË ÌÂ ÙÔÓ ·ıÏËÙÈÛÌfi, ·Ó¿ÏÔÁ·
Ì ÙÔÓ ‚·ıÌfi Ù˘ ˘ÔÏÂÈfiÌÂÓ˘ ÛÙ¤ÓˆÛ˘ ‹
·Ó¿ÚÎÂÈ·˜.
∞Ó¿ÚÎÂÈ· ·ÔÚÙ‹˜ (8): ™˘Ó‹ıˆ˜ ÛÙ· ·È‰È¿
Â›Ó·È ·fiÙÔÎÔ˜ ‰›Ù˘¯Ë˜ ·ÔÚÙÈ΋˜ ‚·Ï‚›‰·˜ ‹
‚·Ï‚ȉÔÏ·ÛÙÈ΋˜ Ì ̷ÏfiÓÈ. ÷ڷÎÙËÚ›˙Â-
Ù·È ‹È· fiÙ·Ó ‰ÂÓ ˘¿Ú¯Ô˘Ó ÂÚÈÊÂÚÈο ÛË-
Ì›· ·ÔÚÙÈ΋˜ ·Ó¿ÚÎÂÈ·˜ Î·È ÙÔ Ì¤ÁÂıÔ˜ Ù˘
·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi, ̤ÙÚÈ·
fiÙ·Ó ˘¿Ú¯Ô˘Ó ÂÚÈÊÂÚÈο ÛËÌ›· Î·È Ë ·ÚÈ-
ÛÙÂÚ‹ ÎÔÈÏ›· ·ÚÔ˘ÛÈ¿˙ÂÈ ‹È· ‰È¿Ù·ÛË ÌÂ Ê˘-
ÛÈÔÏÔÁÈ΋, fï˜, Û˘ÛÙÔÏÈ΋ ÏÂÈÙÔ˘ÚÁ›· Î·È ÛÔ-
‚·Ú‹ fiÙ·Ó ˘¿Ú¯ÂÈ ÌÂÁ¿ÏË ‰È¿Ù·ÛË Ù˘ ·ÚÈ-
ÛÙÂÚ‹˜ ÎÔÈÏ›·˜ ‹/Î·È ˘ÔÛ˘ÛÙ·ÏÙÈÎfiÙËÙ· ·˘-
Ù‹˜. ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ Ì ·ÔÚÙÈ΋ ·Ó¿ÚÎÂÈ·
¯ÚÂÈ¿˙ÔÓÙ·È ÂÎÙ›ÌËÛË ÙÔ˘Ï¿¯ÈÛÙÔÓ Ì›· ÊÔÚ¿
ÙÔÓ ¯ÚfiÓÔ Ì ˯ÔηډÈÔÁÚ¿ÊËÌ·, ‰ÔÎÈÌ·Û›·
ÎÔÒÛˆ˜ Î·È ÂӉ¯Ô̤ӈ˜ Î·È Ì·ÁÓËÙÈ΋ ÙÔ-
ÌÔÁÚ·Ê›·, Ë ÔÔ›· ÂÈÙÚ¤ÂÈ Î·Ï‡ÙÂÚË ÂÎÙ›ÌË-
ÛË ÙÔ˘ ‚·ıÌÔ‡ Ù˘ ·Ó¿ÚÎÂÈ·˜ Î·È Ù˘ Èı·-
Ó‹˜ ‰È¿Ù·Û˘ Ù˘ ·ÔÚÙÈ΋˜ Ú›˙·˜ Ô˘ Û˘¯Ó¿
Û˘Ó˘¿Ú¯ÂÈ. ŸÙ·Ó Ë ·Ó¿ÚÎÂÈ· Â›Ó·È ‹È·
ÂÈÙÚ¤ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ Û fiϘ ÙȘ ηÙËÁÔ-
ڛ˜, ÂÓÒ fiÙ·Ó Â›Ó·È Ì¤ÙÚÈ· ÛÙȘ ηÙËÁÔڛ˜ π
Î·È ππ, Ì ÙËÓ ÚÔ¸fiıÂÛË, ‚‚·›ˆ˜, fiÙÈ ÔÈ
·ÛıÂÓ›˜ Â›Ó·È ·Û˘Ìو̷ÙÈÎÔ›. ∞Ó ˘¿Ú¯ÂÈ
ÎÔÈÏȷ΋ ·ÚÚ˘ıÌ›· ÛÙËÓ ËÚÂÌ›· ‹ ÛÙËÓ ¿ÛÎË-
ÛË, ÙfiÙ ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ Ë Î·ÙËÁÔÚ›· π∞. ∂›
ÛÔ‚·Ú‹˜ ·Ó¿ÚÎÂÈ·˜ ··ÁÔÚ‡ÂÙ·È Ë Û˘ÌÌÂ-
ÙÔ¯‹ Û fiÏ· Ù· ·ÓÙ·ÁˆÓÈÛÙÈο ·ıÏ‹Ì·Ù·. ŸÙ·Ó
Ë ·Ó¿ÚÎÂÈ· ÔÊ›ÏÂÙ·È Û ‰›Ù˘¯Ë ·ÔÚÙÈ΋
‚·Ï‚›‰·, Ô˘ Â›Ó·È Î·È ÙÔ Ï¤ÔÓ Û‡ÓËı˜, ı·
Ú¤ÂÈ Ó· ÏËÊı› ˘’ fi„ÈÓ Î·È ÙÔ Ì¤ÁÂıÔ˜ Ù˘
·ÔÚÙÈ΋˜ Ú›˙·˜. ŸÙ·Ó Ë ·ÔÚÙÈ΋ Ú›˙· Â›Ó·È <40
mm ÂÈÙÚ¤ÔÓÙ·È fiÏ· Ù· ·ıÏ‹Ì·Ù·, ÂÓÒ fiÙ·Ó
Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË ·fi 45 mm ÂÈÙÚ¤ÂÙ·È Ë
Û˘ÌÌÂÙÔ¯‹ ÌfiÓÔ Û ηÙËÁÔÚ›· π∞.
πÛıÌÈ΋ ÛÙ¤ÓˆÛË ·ÔÚÙ‹˜: ∞ÛıÂÓ›˜ Ì ‹È·
ÛÙ¤ÓˆÛË (ÎÏ›ÛË ›ÂÛ˘ < 20 mmHg), Ê˘ÛÈÔÏÔ-
ÁÈ΋ ‰ÔÎÈÌ·Û›· ÎÔÒÛˆ˜ Î·È Ì¤ÁÈÛÙË Û˘ÛÙÔ-
ÏÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË ÛÙËÓ ÎfiˆÛË <230
mmHg, ÌÔÚÔ‡Ó Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó Û fiÏ· Ù·
·ıÏ‹Ì·Ù·. ™Â ·ÓÙ›ıÂÙË ÂÚ›ÙˆÛË, Î·È Ì¤¯ÚÈ
Ó· ·ÓÙÈÌÂÙˆÈÛı› Ë ÓfiÛÔ˜, Û˘ÌÌÂÙ¤¯Ô˘Ó ÌfiÓÔ
ÛÙËÓ Î·ÙËÁÔÚ›· π∞. ŒÍÈ Ì‹Ó˜ ÌÂÙ¿ ÙË ‰ÈfiÚıˆ-
ÛË (¯ÂÈÚÔ˘ÚÁÈ΋ ‹ ·ÁÁÂÈÔÏ·ÛÙÈ΋) ÌÔÚÔ‡Ó
Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙ· ÂÚÈÛÛfiÙÂÚ· ·ıÏ‹Ì·Ù·
(ÂÍ·ÈÚÔ‡ÓÙ·È ·˘Ù¿ Ù˘ ηÙËÁÔÚ›·˜ πππ Î·È Ù·
·ıÏ‹Ì·Ù· Ì ΛӉ˘ÓÔ ÛˆÌ·ÙÈ΋˜ ÚfiÛÎÚÔ˘-
Û˘), ÂÊfiÛÔÓ Ë ÎÏ›ÛË ›ÂÛ˘ Â›Ó·È <20 mmHg
Î·È Ë Û˘ÛÙÔÏÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË Â›Ó·È Ê˘ÛÈÔ-
ÏÔÁÈ΋ ÙfiÛÔ ÛÙËÓ ËÚÂÌ›· fiÛÔ Î·È ÛÙËÓ ÎfiˆÛË.
ªÂÙ¿ ÙË Û˘ÌÏ‹ÚˆÛË ÂÓfi˜ ¤ÙÔ˘˜ ÂÈÙÚ¤Ô-
ÓÙ·È fiϘ ÔÈ Î·ÙËÁÔڛ˜ ·ıÏËÌ¿ÙˆÓ Ì ÌfiÓË
ÂÍ·›ÚÂÛË ÙËÓ ¿ÚÛË ‚·ÚÒÓ. ŸÙ·Ó ˘¿Ú¯ÂÈ ·Ó‡-
Ú˘ÛÌ· ‹ ÛËÌ·ÓÙÈ΋ ‰È¿Ù·ÛË Ù˘ ·ÔÚÙ‹˜, ÂÈ-
ÙÚ¤ÂÙ·È ÌfiÓÔ Ë Î·ÙËÁÔÚ›· π∞.
∆ÂÙÚ·ÏÔÁ›· Fallot: ∆Ë ÛËÌÂÚÈÓ‹ ÂÔ¯‹ Û¯Â-
‰fiÓ fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ Ì ÙÂÙÚ·ÏÔÁ›· ¤¯Ô˘Ó
˘Ô‚ÏËı› Û ¯ÂÈÚÔ˘ÚÁÈ΋ ‰ÈfiÚıˆÛË ÓˆÚ›˜
ηٿ ÙËÓ ÚÒÙË ·È‰È΋ ËÏÈΛ· Î·È ÛÙËÓ
ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ù· ·ÔÙÂϤ-
ÛÌ·Ù· Â›Ó·È ÈηÓÔÔÈËÙÈο. ŒÙÛÈ, ÌÔÚÔ‡Ó Ó·
Û˘ÌÌÂÙ¤¯Ô˘Ó Û fiϘ ÙȘ ‰Ú·ÛÙËÚÈfiÙËÙ˜
ÂÊfiÛÔÓ ÏËÚÔ‡ÓÙ·È ÔÈ ·Ú·Î¿Ùˆ ÚÔ¸Ôı¤-
ÛÂȘ: ·) Ê˘ÛÈÔÏÔÁÈÎfi ̤ÁÂıÔ˜ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜
ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ‹ ۯ‰fiÓ Ê˘ÛÈÔÏÔÁÈ΋ ›ÂÛË,
‚) ·Ô˘Û›· ÛËÌ·ÓÙÈ΋˜ ·Ú·Ì¤ÓÔ˘Û·˜ ÌÂÛÔ-
ÎÔÈÏȷ΋˜ ÂÈÎÔÈÓˆÓ›·˜ Î·È Á) ·Ô˘Û›· ‰È·Ù·-
Ú·¯ÒÓ ÙÔ˘ Ú˘ıÌÔ‡ ÛÙË ‰ÔÎÈÌ·Û›· ÎÔÒÛˆ˜
Î·È ÙËÓ 24ˆÚË Î·Ù·ÁÚ·Ê‹ ÙÔ˘ Ú˘ıÌÔ‡. ∞ÓÙ›-
ıÂÙ·, fiÙ·Ó ˘¿Ú¯ÂÈ ÛËÌ·ÓÙÈ΋ ·Ó¿ÚÎÂÈ·
Ó¢ÌÔÓÈ΋˜ ‚·Ï‚›‰·˜, ›ÂÛË ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜
>50% Ù˘ Û˘ÛÙËÌ·ÙÈ΋˜ ‹ ‰È·Ù·Ú·¯¤˜ ÙÔ˘
Ú˘ıÌÔ‡, ÙfiÙ ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ Ë Î·ÙËÁÔÚ›·
π∞, ÂÂȉ‹ Ô Î›Ó‰˘ÓÔ˜ ·ÈÊÓ›‰ÈÔ˘ ı·Ó¿ÙÔ˘ ›-
Ó·È ·˘ÍË̤ÓÔ˜ (9).
ªÂÙ¿ıÂÛË ÙˆÓ ÌÂÁ¿ÏˆÓ ·ÁÁ›ˆÓ: ™ÙÔ˘˜ ·ÛıÂ-
Ó›˜ Ô˘ ¤¯Ô˘Ó ˘Ô‚ÏËı› Û ¤̂·ÛË ·ÚÙË-
Úȷ΋˜ ·ÓÙÈÌÂÙ¿ıÂÛ˘ (arterial switch
operation) ÂÈÙÚ¤ÔÓÙ·È fiÏ· Ù· ·ıÏ‹Ì·Ù· fiÙ·Ó
ÙÔ Ì¤ÁÂıÔ˜ Î·È Ë Û˘ÛÙ·ÏÙÈÎfiÙËÙ· Ù˘ ·ÚÈÛÙÂ-
Ú‹˜ ÎÔÈÏ›·˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈο, ‰ÂÓ ˘¿Ú¯Ô˘Ó
˘ÔÏÂÈfiÌÂÓ˜ ·ÓˆÌ·Ï›Â˜, Ë ‰ÔÎÈÌ·Û›· ÎÔÒ-
Ûˆ˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋ Î·È ‰ÂÓ ‰È·ÈÛÙÒÓÔ-
ÓÙ·È ÛËÌ·ÓÙÈΤ˜ ·ÚÚ˘ı̛˜ ÛÙÔ Holter Ú˘ıÌÔ‡.
∂Âȉ‹ ˘¿Ú¯ÂÈ ÙÔ ıˆÚËÙÈÎfi ÂӉ¯fiÌÂÓÔ ÂÈ-
‚¿Ú˘ÓÛ˘ Ù˘ ÓÂÔ·ÔÚÙÈ΋˜ Ú›˙·˜, Û˘ÓÈÛٿٷÈ
·ÔÊ˘Á‹ ÙˆÓ ·ıÏËÌ¿ÙˆÓ Î·ÙËÁÔÚ›·˜ πππ. ŸÛÔÈ
·ÛıÂÓ›˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ‹È˜ ·ÈÌÔ‰˘Ó·ÌÈΤ˜
‰È·Ù·Ú·¯¤˜ ‹ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘ ·ÚÈÛÙÂÚ‹˜
ÎÔÈÏ›·˜, ÌÔÚÔ‡Ó Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙȘ ηÙË-
ÁÔڛ˜ π Î·È ππ∞ ÂÊfiÛÔÓ Ë ‰ÔÎÈÌ·Û›· ÎÔÒÛˆ˜
Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋.
471ÕÛÎËÛË Î·È ·ı‹ÛÂȘ ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
472 ¢. °ÂˆÚÁ·ÎfiÔ˘ÏÔ˜, °. ¶··‰fiÔ˘ÏÔ˜
™Â ·ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó ˘ÔÛÙ› ‰ÈfiÚıˆÛË
Ì ÙȘ ·Ï·ÈfiÙÂÚ˜ Ù¯ÓÈΤ˜ (Mustard ‹
Senning) Ù· ÚÔ‚Ï‹Ì·Ù· Â›Ó·È ÂÚÈÛÛfiÙÂÚ·,
ηıÒ˜ ÚfiÏÔ Û˘ÛÙËÌ·ÙÈ΋˜ ÎÔÈÏ›·˜ ‰È·‰Ú·Ì·-
Ù›˙ÂÈ Ë ‰ÂÍÈ¿ ÎÔÈÏ›·. ™ÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜
ÂÈÙÚ¤ÂÙ·È Û˘ÌÌÂÙÔ¯‹ Û ·ıÏ‹Ì·Ù· Ù‡Ô˘
π∞ Î·È ππ∞, ·Ó ˘¿Ú¯Ô˘Ó ÔÈ ÂÍ‹˜ ÚÔ¸Ôı¤-
ÛÂȘ: Ê˘ÛÈÔÏÔÁÈÎfi ̤ÁÂıÔ˜ ÎÔÈÏÔًوÓ, ·Ô˘-
Û›· ·ÚÚ˘ıÌÈÒÓ, ·Ô˘Û›· ÈÛÙÔÚÈÎÔ‡ Û˘ÁÎÔÙÈ-
ÎÒÓ ÂÂÈÛÔ‰›ˆÓ Î·È Ê˘ÛÈÔÏÔÁÈ΋ ‰ÔÎÈÌ·Û›·
ÎÔÒÛˆ˜.
™‡ÌÏÔΘ ΢·ÓˆÙÈΤ˜ ηډÈÔ¿ıÂȘ (ÌÂÙ¿
·fi ·ÚËÁÔÚËÙÈ΋ ¤̂·ÛË): ¶ÚfiÎÂÈÙ·È ÁÈ·
ÌÈ· ÌÂÁ¿ÏË Î·È ·ÓÔÌÔÈÔÁÂÓ‹ ÔÌ¿‰· ·ı‹ÛÂ-
ˆÓ, ÙˆÓ ÔÔ›ˆÓ ÎÔÈÓfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi Â›Ó·È Ô
¯·ÌËÏfi˜ ÎÔÚÂÛÌfi˜ Û Ô͢ÁfiÓÔ ÙÔ˘ ·ÚÙËÚÈ·-
ÎÔ‡ ·›Ì·ÙÔ˜. √È ÂÚÈÛÛfiÙÂÚÔÈ ·fi ÙÔ˘˜ ·ÛıÂ-
Ó›˜ ·˘ÙÔ‡˜ ÌÔÚÔ‡Ó Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙËÓ
ηÙËÁÔÚ›· π∞ Î·È ÔÚÈṲ̂ÓÔÈ ·fi ·˘ÙÔ‡˜ ηÈ
ÛÙËÓ Î·ÙËÁÔÚ›· πµ, ·Ó ¤¯Ô˘Ó Ê˘ÛÈÔÏÔÁÈÎfi ÎÔ-
ÚÂÛÌfi Î·È ÈηÓÔÔÈËÙÈ΋ ÎÔÈÏȷ΋ ÏÂÈÙÔ˘ÚÁ›·
¯ˆÚ›˜ ·ÚÚ˘ı̛˜.
™˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ ÛÙÂÊ·ÓÈ·›ˆÓ ·ÚÙËÚÈÒÓ:
¶Ú¤ÂÈ Ó· ÙȘ ˘Ô„È¿˙ÂÙ·È Î·Ó›˜ Û Ó·ÚÔ‡˜
·ıÏËÙ¤˜ Ì ÚÔοډÈÔ ¿ÏÁÔ˜ ‹ Û˘ÁÎÔÙÈÎfi
ÂÂÈÛfi‰ÈÔ Î·Ù¿ ÙËÓ ¿ÛÎËÛË. ŒÍÈ Ì‹Ó˜ ÌÂÙ¿ ÙË
¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË ÂÈÙÚ¤ÂÙ·È Ë
¿ıÏËÛË, ·Ó Ë Ì¤ÁÈÛÙË ‰ÔÎÈÌ·Û›· ÎÔÒÛˆ˜ ›-
Ó·È Ê˘ÛÈÔÏÔÁÈ΋ Î·È ‰ÂÓ ¤¯ÂÈ ÚÔ¸¿ÚÍÂÈ ¤Ì-
ÊÚ·ÁÌ· Ì˘Ôηډ›Ô˘.
™‡Ó‰ÚÔÌÔ Marfan: √È ·ÛıÂÓ›˜ ·˘ÙÔ› ÎÈÓ‰˘-
ÓÂ‡Ô˘Ó ·fi ‰È·¯ˆÚÈÛÙÈÎfi ·Ó‡ڢÛÌ· Ù˘ ·ÔÚ-
Ù‹˜ (10) Î·È ··ÁÔÚ‡ÂÙ·È Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÂ
·ıÏ‹Ì·Ù· Ì ΛӉ˘ÓÔ ÛˆÌ·ÙÈ΋˜ ÚfiÛÎÚÔ˘Û˘
(¶›Ó·Î·˜ 2). ∞Ó ‰ÂÓ ˘¿Ú¯ÂÈ ‰È¿Ù·ÛË ·ÔÚÙ‹˜
Î·È ·Ó¿ÚÎÂÈ· ÌÈÙÚÔÂȉԇ˜, ÌÔÚÔ‡Ó Ó· Û˘Ì-
ÌÂÙ¤¯Ô˘Ó Û ·ıÏ‹Ì·Ù· ηÙËÁÔÚ›·˜ π∞, ÙÔÍÔ‚Ô-
Ï›·, Ù¤ÓȘ ‰ÈÏfi, ÛÙ·ÙÈÎfi Ô‰‹Ï·ÙÔ Î·È ·ÙÈÓ¿˙
› ¿ÁÔ˘ (11). √ ˯ÔηډÈÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜
ÂÈ‚¿ÏÏÂÙ·È ·Ó¿ ÂÍ¿ÌËÓÔ.
¶ÚfiÙˆÛË ÌÈÙÚÔÂȉԇ˜ (12): ∏ ÚfiÙˆÛË
Ù˘ ÌÈÙÚÔÂȉԇ˜ Â›Ó·È È‰È·›ÙÂÚ· Û˘¯Ó‹ ÛÙÔÓ ÁÂ-
ÓÈÎfi ÏËı˘ÛÌfi, Ì ÙË Û˘¯ÓfiÙËÙ¿ Ù˘ Ó· ΢-
Ì·›ÓÂÙ·È ·fi 2-5% (13). ™˘Ó‹ıˆ˜ ÚfiÎÂÈÙ·È
ÁÈ· ηÏÔ‹ıË Î·Ù¿ÛÙ·ÛË, ·Ó Î·È Û ÔÚÈṲ̂Ó˜
ÂÚÈÙÒÛÂȘ Û˘Óԉ‡ÂÙ·È ·fi ·›ÛıËÌ· ÚÔ-
ηډ›ˆÓ ·ÏÌÒÓ, ˙¿ÏË Î·È ˘ÂÚÎÔÈÏȷΤ˜ ‹
ÎÔÈÏȷΤ˜ ·ÚÚ˘ı̛˜ (14). ªÔÚ› Ó· ·ÔÙÂ-
ϤÛÂÈ ÙÔ ·›ÙÈÔ ÛËÌ·ÓÙÈ΋˜ ÌÈÙÚÔÂȉÈ΋˜ ·ÓÂ-
¿ÚÎÂÈ·˜, ÂÌ‚ÔÏÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ Î·È ·ÈÊÓ›‰È-
Ô˘ ı·Ó¿ÙÔ˘ (15). ¶ÈÛÙ‡ÂÙ·È fiÙÈ ÔÈ ÛÔ‚·Ú¤˜
ÂÈÏÔΤ˜ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘
ÛÙËÓ ÔÔ›· ÔÈ ÌÔÚÊÔÏÔÁÈΤ˜ ·ÏÏÔÈÒÛÂȘ ÙˆÓ
Áψ¯›ÓˆÓ Â›Ó·È ¤Î‰ËϘ (13). ™Â ÁÂÓÈΤ˜ ÁÚ·Ì-
̤˜, Ë ÚfiÁÓˆÛË ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì Úfi-
ÙˆÛË Â›Ó·È Â˘ÓÔ˚΋ Î·È Ù· ÂÂÈÛfi‰È· ·ÈÊÓ›-
‰ÈˆÓ ı·Ó¿ÙˆÓ Ô˘ ¤¯Ô˘Ó ·Ó·ÊÂÚı› Â›Ó·È Ô-
χ Û¿ÓÈ· (16). ŒÙÛÈ, ·ÛıÂÓ›˜ Ì ÚfiÙˆÛË
ÌÔÚÔ‡Ó Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó Û fiÏ· Ù· ·ıÏ‹Ì·Ù·,
Ì Èı·Ó‹ ÂÍ·›ÚÂÛË ·˘Ù¿ Ù˘ ηÙËÁÔÚ›·˜ πππ,
·Ó ‰ÂÓ ˘¿Ú¯Ô˘Ó Ù· ÂÍ‹˜:
·) ÈÛÙÔÚÈÎfi Û˘ÁÎÔÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ·Ú-
Ú˘ıÌÈÔÏÔÁÈ΋˜ ÚÔ¤Ï¢Û˘,
‚) ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ·ÈÊÓ›‰ÈÔ˘ ı·Ó¿-
ÙÔ˘ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÚfiÙˆÛË ÌÈÙÚÔÂȉԇ˜,
Á) ·ӷϷ̂·ÓfiÌÂÓ· ÂÂÈÛfi‰È· ˘ÂÚÎÔÈ-
Ïȷ΋˜ Ù·¯˘Î·Ú‰›·˜ ‹ Û‡ÌÏÔΘ ÌÔÚʤ˜ ÎÔÈ-
ÏÈ·ÎÒÓ ·ÚÚ˘ıÌÈÒÓ, ÂȉÈο ·Ó ÂȉÂÈÓÒÓÔÓÙ·È
ÌÂ ÙËÓ ¿ÛÎËÛË,
‰) ÛËÌ·ÓÙÈ΋ ·Ó¿ÚÎÂÈ· ÌÈÙÚÔÂȉԇ˜ ηÈ
Â) ÚÔËÁËı¤Ó ÂÌ‚ÔÏÈÎfi ÂÂÈÛfi‰ÈÔ. ŸÙ·Ó
˘¿Ú¯ÂÈ Î¿ÔÈÔ ·fi Ù· ·Ú·¿Óˆ Â˘Ú‹Ì·Ù·
ÂÈÙÚ¤ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ ÛÙËÓ Î·ÙËÁÔÚ›· π∞.
ª˘ÔηډÈÔ¿ıÂȘ – ª˘Ôηډ›Ùȉ· –
¶ÂÚÈηډ›Ùȉ· (12)
ÀÂÚÙÚÔÊÈ΋ Ì˘ÔηډÈÔ¿ıÂÈ·: Ÿˆ˜ ›ӷÈ
ÁÓˆÛÙfi Ë ˘ÂÚÙÚÔÊÈ΋ Ì˘ÔηډÈÔ¿ıÂÈ·
·ÔÙÂÏ› ÙËÓ Î‡ÚÈ· ·ÈÙ›· ·ÈÊÓ›‰ÈÔ˘ ı·Ó¿ÙÔ˘
ηٿ ÙËÓ ¿ÛÎËÛË Û Ó·ڿ ¿ÙÔÌ· (17). °È· ÙÔÓ
ÏfiÁÔ ·˘Ùfi, fiÙ·Ó Ë ‰È¿ÁÓˆÛË Ù˘ Â›Ó·È Û›ÁÔ˘-
ÚË, ··ÁÔÚ‡ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ ÙˆÓ ·Û¯fi-
ÓÙˆÓ Û ÔÔÈÔ‰‹ÔÙ ·ÓÙ·ÁˆÓÈÛÙÈÎfi ¿ıÏËÌ·.
™Â ÔÚÈṲ̂ӷ ¿ÙÔÌ· ÛÙ· ÔÔ›· Ë ‰È¿ÁÓˆÛË ¤¯ÂÈ
ÙÂı› Ì ÁÂÓÂÙÈÎfi ¤ÏÂÁ¯Ô (18) ¯ˆÚ›˜ Ó· ¤¯ÂÈ
·ÎfiÌ· ÂΉËψı› Ê·ÈÓÔÙ˘Èο Ë ÓfiÛÔ˜, ‰ÂÓ
˘¿Ú¯Ô˘Ó › ÙÔ˘ ·ÚfiÓÙÔ˜ ÛÙÔȯ›· Ô˘ Ó·
·ÔÎÏÂ›Ô˘Ó ÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘˜ Û ·ıÏËÙÈΤ˜
‰Ú·ÛÙËÚÈfiÙËÙ˜, ÂÎÙfi˜ ·Ó ˘¿Ú¯Ô˘Ó Û˘ÌÙÒ-
Ì·Ù· ‹ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ·ÈÊÓ›‰ÈÔ˘ ı·-
Ó¿ÙÔ˘. ∂›Û˘, Û‡Ìʈӷ Ì ÙȘ ÙÂÏÂ˘Ù·›Â˜
Ô‰ËÁ›Â˜ Ù˘ ∞merican ∏eart ∞ssociation (11),
¿ÙÔÌ· ¿Û¯ÔÓÙ· ·fi ÙË ÓfiÛÔ ÌÔÚÔ‡Ó Ó·
Û˘ÌÌÂÙ¤¯Ô˘Ó Û ÌË ·ÓÙ·ÁˆÓÈÛÙÈΤ˜ ‰Ú·ÛÙË-
ÚÈfiÙËÙ˜, fiˆ˜ Ô‰ËÏ·Û›·, ÎÔχ̂ËÛË, Ù¤ÓȘ
‰ÈÏfi, ÌfiÔ˘ÏÈÓÁÎ, ÁÎÔÏÊ Î·È ·ÙÈÓ¿˙ ›
¿ÁÔ˘.
ª˘Ôηډ›Ùȉ·: ∏ Ì˘Ôηډ›Ùȉ·, Û˘Ó‹ıˆ˜
ÈÔÁÂÓÔ‡˜ ·ÈÙÈÔÏÔÁ›·˜, ıˆÚÂ›Ù·È Û¿ÓÈÔ ·›ÙÈÔ
·ÈÊÓ›‰ÈÔ˘ ı·Ó¿ÙÔ˘ ηٿ ÙËÓ ¿ÛÎËÛË (1). ¶·-
ÚfiÏ· ·˘Ù¿ Â›Ó·È ÛËÌ·ÓÙÈ΋ ·fi ÎÏÈÓÈ΋˜
·fi„ˆ˜, ‰ÈfiÙÈ Â›Ó·È ‰˘Ó·Ùfi Ó· ‰È·‰Ú¿ÌÂÈ ˘Ô-
ÎÏÈÓÈο, ¯ˆÚ›˜ ıÔÚ˘‚Ò‰Ë Û˘Ìو̷ÙÔÏÔÁ›· ηÈ
Ó· ·Ú·Ì›ÓÂÈ ·‰È¿ÁÓˆÛÙË. ∞ÛıÂÓ›˜ Ô˘ È-
ÛÙ‡ÂÙ·È fiÙÈ ¿Û¯Ô˘Ó ·fi Ì˘Ôηډ›Ùȉ· Ú¤ÂÈ
Ó· ·¤¯Ô˘Ó ·fi οı ·ıÏËÙÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
473ÕÛÎËÛË Î·È ·ı‹ÛÂȘ ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡
ÁÈ· 6 Ì‹Ó˜. ªÂÙ¿ ÙËÓ ¿ÚÔ‰Ô Ù˘ ÂÚÈfi‰Ô˘
·˘Ù‹˜, ÌÔÚÔ‡Ó Ó· ÂÈÛÙÚ¤„Ô˘Ó ÛÙÔÓ ·ıÏËÙÈ-
ÛÌfi ÂÊfiÛÔÓ ÔÈ ‰È·ÛÙ¿ÛÂȘ Î·È Ë Û˘ÛÙ·ÏÙÈÎfi-
ÙËÙ· Ù˘ ·ÚÈÛÙÂÚ¿˜ ÎÔÈÏ›·˜ ¤¯Ô˘Ó ·ӤÏıÂÈ
ÛÂ Ê˘ÛÈÔÏÔÁÈο ›‰· Î·È ÛÙÔ Holter Ú˘ı-
ÌÔ‡ ‰ÂÓ ˘¿Ú¯Ô˘Ó ÂÂÈÛfi‰È· ÂÈ̤ÓÔ˘Û·˜
˘ÂÚÎÔÈÏȷ΋˜ Ù·¯˘Î·Ú‰›·˜ ‹ Û‡ÌÏÔΘ
ÌÔÚʤ˜ ÎÔÈÏÈ·ÎÒÓ ·ÚÚ˘ıÌÈÒÓ.
¶ÂÚÈηډ›Ùȉ·: ∫·Ù¿ ÙËÓ ÔÍ›· Ê¿ÛË Ù˘ Ófi-
ÛÔ˘ ··ÁÔÚ‡ÂÙ·È Î¿ı ·ıÏËÙÈ΋ ‰Ú·ÛÙËÚÈfi-
ÙËÙ·. ∏ ¿ıÏËÛË ÂÈÙÚ¤ÂÙ·È ¿ÏÈ, fiÙ·Ó ÙÔ
˘ÁÚfi ¤¯ÂÈ ·ÔÚÚÔÊËı› Ï‹Úˆ˜, ÔÈ ÂÚÁ·ÛÙË-
ÚÈ·ÎÔ› ‰Â›ÎÙ˜ Ïԛ̈͢ ¤¯Ô˘Ó ˘Ô¯ˆÚ‹ÛÂÈ
Î·È ÙÔ ∏olter Ú˘ıÌÔ‡ Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi. ∞Ó
Û˘Ó˘¿Ú¯ÂÈ ÚÔÛ‚ÔÏ‹ ÙÔ˘ Ì˘Ôηډ›Ô˘, ÙfiÙÂ
·ÎÔÏÔ˘ıÔ‡ÓÙ·È ÔÈ Ô‰ËÁ›Â˜ Ô˘ ·ÊÔÚÔ‡Ó ÙË
Ì˘Ôηډ›Ùȉ·.
¡fiÛÔ˜ Kawasaki
∞ÔÙÂÏ› ÙÔ Û˘¯ÓfiÙÂÚÔ ·›ÙÈÔ Â›ÎÙËÙ˘
ÛÙÂÊ·ÓÈ·›·˜ ÓfiÛÔ˘ ÛÙËÓ ·È‰È΋ ËÏÈΛ· (19).
¶ÚfiÎÂÈÙ·È ÁÈ· ÌÈ· ‰È¿¯˘ÙË ÊÏÂÁÌÔÓÒ‰Ë ÌÈ-
ÎÚÔ·ÁÁÂÈ›Ùȉ· Ô˘ ÌÔÚ› Ó· ÚÔÛ‚¿ÏÂÈ ÙȘ
ÛÙÂÊ·ÓÈ·›Â˜ ·ÚÙËڛ˜ Î·È Ó· ÚÔηϤÛÂÈ
·Ó¢ڇÛÌ·Ù· Î·È ÛÙÂÓÒÛÂȘ ÔÈ Ôԛ˜ ‰˘Ó·Ùfi
Ó· ÂΉËψıÔ‡Ó ÎÏÈÓÈο, ÔÏÏ¿ ¯ÚfiÓÈ· ÌÂÙ¿
ÙËÓ ·Ú¯È΋ ÚÔÛ‚ÔÏ‹ (20,21). ∆· ·Ó¢ڇÛÌ·-
Ù· Û˘Ó‹ıˆ˜ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È Â·ÚÎÒ˜ Ì ÙÔ
˯ÔηډÈÔÁÚ¿ÊËÌ·, ·ÏÏ¿ ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË
Ù˘ Ì˘Ôηډȷ΋˜ ÈÛ¯·ÈÌ›·˜ ‰ÂÓ ·ÚΛ Ë ·Ï‹
‰ÔÎÈÌ·Û›· ÎÔÒÛˆ˜ Î·È Û˘Ó‹ıˆ˜ ··ÈÙ›ٷÈ
Î·È ÎfiˆÛË Ì ı¿ÏÏÈÔ. ∞ÛıÂÓ›˜ ¯ˆÚ›˜ ÚÔ-
Û‚ÔÏ‹ ÙˆÓ ÛÙÂÊ·ÓÈ·›ˆÓ ÌÔÚÔ‡Ó Ó· ·ıÏÔ‡-
ÓÙ·È ÂχıÂÚ· ÌÂÙ¿ ·fi 6-8 ‚‰ÔÌ¿‰Â˜. ∞ÛıÂ-
Ó›˜ Ô˘ ›¯·Ó ·Ó¢ڇÛÌ·Ù· Ô˘ ÂÍ·Ê·Ó›ÛıË-
Î·Ó Ú¤ÂÈ Ó· ÂϤÁ¯ÔÓÙ·È ÚÒÙ· Ì ‰ÔÎÈÌ·-
Û›· ÎÔÒÛˆ˜ Î·È ÛÈÓıËÚÔÁÚ¿ÊËÌ· ·ÈÌ¿Ùˆ-
Û˘ ÙÔ˘ Ì˘Ôηډ›Ô˘. ∞ÛıÂÓ›˜ Ì ÌÈÎÚ¿ ·Ó¢-
Ú‡ÛÌ·Ù· Û˘ÌÌÂÙ¤¯Ô˘Ó ̤¯ÚÈ ÙËÓ Î·ÙËÁÔÚ›·
ππµ, ·ÊÔ‡ ÚÔËÁÔ˘Ì¤Óˆ˜ ÂÏÂÁ¯ıÔ‡Ó ÁÈ· ÈÛ¯·È-
Ì›·, ÂÓÒ ·ÛıÂÓ›˜ Ì ÌÂÁ¿Ï· ·Ó¢ڇÛÌ·Ù· ‹
ÛÙÂÓÒÛÂȘ ηٷٿÛÛÔÓÙ·È ÛÙËÓ Î·ÙËÁÔÚ›· π∞.
∞ÚÚ˘ı̛˜ (22)
ŒÎÙ·ÎÙ˜ ÎÔÈÏȷΤ˜ Û˘ÛÙÔϤ˜ (∂∫™): √ ¤ÏÂÁ-
¯Ô˜ ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ ÂÚÈÏ·Ì‚¿ÓÂÈ, ÂÎÙfi˜
·fi ÙÔ ∏∫° 12 ··ÁˆÁÒÓ, ‰ÔÎÈÌ·Û›· ÎÔÒÛÂ-
ˆ˜ Î·È Ë¯ÔηډÈÔÁÚ¿ÊËÌ· ÁÈ· ÙÔÓ ·ÔÎÏÂÈÛÌfi
‰ÔÌÈ΋˜ ηډÈÔ¿ıÂÈ·˜. ∞Ó ÛÙË ‰ÔÎÈÌ·Û›· ÎÔ-
ÒÛˆ˜ ·˘Í¿ÓÂÙ·È Ô ·ÚÈıÌfi˜ ÙˆÓ ÂÎÙ¿ÎÙˆÓ ‹
ηı›ÛÙ·ÓÙ·È Û‡ÌÏÔΘ, ÙfiÙ ÂÈ‚¿ÏÏÂÙ·È Â-
Ú·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô˜, ÂӉ¯Ô̤ӈ˜ Î·È Ì ηډȷ-
Îfi ηıÂÙËÚÈ·ÛÌfi, ·ÎfiÌ· Î·È ·Ó ‰ÂÓ ˘¿Ú¯ÂÈ
ÂÌÊ·Ó‹˜ ‰ÔÌÈ΋ ηډÈÔ¿ıÂÈ· ÛÙÔ Ë¯ÔηډÈÔ-
ÁÚ¿ÊËÌ·. ŸÙ·Ó ÔÈ ∂∫™ ÂÍ·Ê·Ó›˙ÔÓÙ·È ‹ ÌÂÈÒ-
ÓÔÓÙ·È Ì ÙËÓ ÎfiˆÛË Î·È ‰ÂÓ ˘¿Ú¯ÂÈ ˘ÔΛ-
ÌÂÓË Î·Ú‰ÈÔ¿ıÂÈ·, Ë Û˘ÌÌÂÙÔ¯‹ Â›Ó·È ÂχıÂ-
ÚË. ŸÙ·Ó ˘¿Ú¯ÂÈ ‰ÔÌÈ΋ ηډÈÔ¿ıÂÈ· ‹ Ô
·ÛıÂÓ‹˜ ¯Ú‹˙ÂÈ ÁÈ· ÔÔÈÔÓ‰‹ÔÙ ÏfiÁÔ
·ÓÙÈ·ÚÚ˘ıÌÈ΋˜ ·ÁˆÁ‹˜, ÙfiÙ ÂÈÙÚ¤ÔÓÙ·È
ÌfiÓÔ Ù· ·ıÏ‹Ì·Ù· ηÙËÁÔÚ›·˜ π∞.
ÀÂÚÎÔÈÏȷ΋ Ù·¯˘Î·Ú‰›·: ∞ÔÙÂÏ› Ì›· ·fi
ÙȘ ϤÔÓ Û˘¯Ó¤˜ ·ÚÚ˘ı̛˜ ÛÙ· ·È‰È¿. ™Â ÁÂ-
ÓÈΤ˜ ÁÚ·Ì̤˜, Ë Û˘ÌÌÂÙÔ¯‹ ÛÙËÓ ¿ıÏËÛË Â›-
Ó·È ÂχıÂÚË ÂÊfiÛÔÓ ÔÈ ·ÛıÂÓ›˜ Â›Ó·È ·Û˘-
Ìو̷ÙÈÎÔ› Î·È Ë ·ÓÙÈ·ÚÚ˘ıÌÈ΋ ·ÁˆÁ‹ Úfi-
Ï˄˘ ÙˆÓ ˘ÔÙÚÔÒÓ Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋,
fiˆ˜ ÂϤÁ¯ÂÙ·È Ì ÙÔ ∏∫° 24ÒÚÔ˘ Î·È ÙË ‰Ô-
ÎÈÌ·Û›· ÎÔÒÛˆ˜. ∞Ó ˘¿Ú¯Ô˘Ó ÛÔ‚·Ú¿ Û˘-
ÌÙÒÌ·Ù· (Û˘ÁÎÔÙÈο ‹ ÚÔÛ˘ÁÎÔÙÈο
ÂÂÈÛfi‰È·, ¤ÓÙÔÓÔ ·›ÛıËÌ· ÚÔηډ›ˆÓ ·Ï-
ÌÒÓ), ÙfiÙÂ Ë ¿ıÏËÛË ÂÈÙÚ¤ÂÙ·È ÂχıÂÚ·
ÌÂÙ¿ ·fi 6 Ì‹Ó˜ ·Ô˘Û›·˜ ˘ÔÙÚÔÒÓ. ª¤-
¯ÚÈ ÙfiÙ ÌÔÚÔ‡Ó Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙËÓ Î·ÙË-
ÁÔÚ›· π∞. ªÂÙ¿ ·fi Î·Ù¿Ï˘ÛË Ì ηıÂÙ‹Ú·
ÂÈÙÚ¤ÂÙ·È ÂχıÂÚ· Ë ¿ıÏËÛË ÌÂÙ¿ ¿ÚÔ‰Ô
2 ÌËÓÒÓ Î·È ÂÊfiÛÔÓ ÛÙÔ ÌÂÛԉȿÛÙËÌ· ‰ÂÓ
˘‹ÚÍ·Ó ˘ÔÙÚÔ¤˜.
™‡Ó‰ÚÔÌÔ WPW: √ ·ÈÊÓ›‰ÈÔ˜ ı¿Ó·ÙÔ˜ ›ӷÈ
ÁÂÓÈο Û¿ÓÈÔ Ê·ÈÓfiÌÂÓÔ Û ·ıÏËÙ¤˜ Ì ۇÓ-
‰ÚÔÌÔ ÚԉȤÁÂÚÛ˘ Î·È Ê·›ÓÂÙ·È Ó· ÂÚÈÔÚ›-
˙ÂÙ·È Û ·ÛıÂÓ›˜ Ì ‚Ú·¯Â›· ·ÓÂÚ¤ıÈÛÙË Â-
Ú›Ô‰Ô ÙÔ˘ ·Ú·ÏËڈ̷ÙÈÎÔ‡ ‰ÂÌ·Ù›Ô˘
(23,24). √ ÚÔ·ıÏËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÂÚÈÏ·Ì‚¿-
ÓÂÈ ÔˆÛ‰‹ÔÙ ∏∫° 12 ··ÁˆÁÒÓ, 24ˆÚË
ηٷÁÚ·Ê‹ ÙÔ˘ Ú˘ıÌÔ‡, Ë ÔÔ›· ηÏfi Â›Ó·È Ó·
ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ÙËÓ ·ıÏËÙÈ΋ ‰Ú·ÛÙËÚÈfiÙË-
Ù· ÙÔ˘ ·ÛıÂÓÔ‡˜, ˯ÔηډÈÔÁÚ¿ÊËÌ· Î·È ‰Ô-
ÎÈÌ·Û›· ÎÔÒÛˆ˜. ∂ÎÙfi˜ ·fi ÙȘ ÚÔ·Ó·-
ÊÂÚı›Û˜ ÂÍÂÙ¿ÛÂȘ, ÔÏÏÔ› ı· Û˘ÓÈÛÙÔ‡Û·Ó
Î·È ËÏÂÎÙÚÔÊ˘ÛÈÔÏÔÁÈ΋ ÌÂϤÙË Û ¿ÙÔÌ·
ËÏÈΛ·˜ οو ÙˆÓ 20 ÂÙÒÓ. ªÂÙ¿ ·fi ÂÈÙ˘¯‹
Î·Ù¿Ï˘ÛË Ì ηıÂÙ‹Ú· ÙÔ˘ ·Ú·ÏËڈ̷ÙÈ-
ÎÔ‡ ‰ÂÌ·Ù›Ô˘, ÂÈÙÚ¤ÂÙ·È Ë ¿ıÏËÛË ÂχıÂ-
Ú· ÌÂÙ¿ ¿ÚÔ‰Ô 2 ÌËÓÒÓ, ·Ó ‰ÂÓ ˘¿Ú¯ÂÈ ÂÈ-
ÎfiÓ· ÚԉȤÁÂÚÛ˘ ÛÙÔ ∏∫°.
™‡Ó‰ÚÔÌÔ Ì·ÎÚÔ‡ QT: ∞ÔÙÂÏ› Û¿ÓÈÔ ·›ÙÈÔ
·ÈÊÓ›‰ÈÔ˘ ı·Ó¿ÙÔ˘. £ÂˆÚÂ›Ù·È ·Ú·ÙÂٷ̤ÓÔ
fiÙ·Ó ÙÔ ‰ÈÔÚıˆÌ¤ÓÔ QT Â›Ó·È ÌÂÁ·Ï‡ÙÂÚÔ ÙˆÓ
460 msec (25). ªÂ ÙËÓ ÂÎÙÂٷ̤ÓË ÂÊ·ÚÌÔÁ‹
ÙÔ˘ ÚÔ·ıÏËÙÈÎÔ‡ ÂϤÁ¯Ô˘ ı· ˘¿ÚÍÔ˘Ó ·ÚÎÂ-
Ù¿ ¿ÙÔÌ· Ù· ÔÔ›· ı· ÂÌÊ·Ó›˙Ô˘Ó ÔÚȷ΋ ·‡ÍË-
ÛË ÙÔ˘ QTc, Ì ·ÔÙ¤ÏÂÛÌ· Ó· Á›ÓÂÙ·È ˘ÂÚ-
‰È¿ÁÓˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ™ÙÔ˘˜ ·ıÏËÙ¤˜ ·˘-
ÙÔ‡˜, fiÙ·Ó Â›Ó·È ·Û˘Ìو̷ÙÈÎÔ›, ¤¯Ô˘Ó Âχ-
ıÂÚÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi, Ê˘ÛÈÔÏÔÁÈÎfi ηٿ
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
474 ¢. °ÂˆÚÁ·ÎfiÔ˘ÏÔ˜, °. ¶··‰fiÔ˘ÏÔ˜
Ù· ¿ÏÏ· ∏∫° Î·È Ê˘ÛÈÔÏÔÁÈ΋ ‰ÔÎÈÌ·Û›· ÎÔÒ-
Ûˆ˜, ‰ÂÓ ÂÈ‚¿ÏÏÔÓÙ·È ÂÚÈÔÚÈÛÌÔ›. ™Â ¿ÙÔÌ·
Ì ÂȂ‚·ÈˆÌ¤ÓË ÙË ‰È¿ÁÓˆÛË ÙÔ˘ Ì·ÎÚÔ‡ QT
··ÁÔÚ‡ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ Û ·ÓÙ·ÁˆÓÈÛÙÈο
·ıÏ‹Ì·Ù·, ÌÔÚÔ‡Ó fï˜ Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÂ
„˘¯·ÁˆÁÈΤ˜ ·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ (Ù¤ÓȘ
‰ÈÏfi, ÛÙ·ÙÈÎfi Ô‰‹Ï·ÙÔ, ÌfiÔ˘ÏÈÓÁÎ, ÁÎÔÏÊ
Î·È ·ÙÈÓ¿˙ › ¿ÁÔ˘) (11). ∂Í·›ÚÂÛË ·ÔÙÂ-
ÏÔ‡Ó ÔÈ ÂÚÈÙÒÛÂȘ ÙÔ˘ Ì·ÎÚÔ‡ QT Ù‡Ô˘ 1
ÛÙÔ ÔÔ›Ô ··ÁÔÚ‡ÂÙ·È Ë ÎÔχ̂ËÛË ·ÎfiÌ·
Î·È ÁÈ· „˘¯·ÁˆÁÈÎÔ‡˜ ÏfiÁÔ˘˜. ¶¿ÓÙˆ˜, ÂÂȉ‹
ÙÔ Û‡Ó‰ÚÔÌÔ ·ÚÔ˘ÛÈ¿˙ÂÈ ·ÚÎÂÙ¤˜ ·Ú·ÏÏ·Á¤˜,
ηÏfi Â›Ó·È Â› ˘Ô„›·˜ ¤ÛÙˆ ˘¿ÚÍÂÒ˜ ÙÔ˘ Ó·
Á›ÓÂÙ·È ÂÎÙ›ÌËÛË Î·È Î·ıÔ‰‹ÁËÛË ·fi ÂȉÈÎÔ‡˜.
∫ÔÏÔÎÔÈÏÈ·Îfi˜ ·ÔÎÏÂÈÛÌfi˜ 1Ô˘ ‚·ıÌÔ‡: ∞Ó
ÙÔ QRS Â›Ó·È ·ıÔÏÔÁÈÎfi ‹ ÙÔ PR >300 msec,
Û˘ÓÈÛÙ¿Ù·È Holter Ú˘ıÌÔ‡, ‰ÔÎÈÌ·Û›· ÎÔÒ-
Ûˆ˜ Î·È ÂӉ¯Ô̤ӈ˜ Î·È ËÏÂÎÙÚÔÊ˘ÛÈÔÏÔÁÈ-
΋ ÌÂϤÙË. ŸÙ·Ó Ô ·ÔÎÏÂÈÛÌfi˜ ‰ÂÓ ÂȉÂÈ-
ÓÒÓÂÙ·È Ì ÙËÓ ¿ÛÎËÛË, ÙfiÙÂ Ë Û˘ÌÌÂÙÔ¯‹ ›-
Ó·È ÂχıÂÚË Û fiÏ· Ù· ·ıÏ‹Ì·Ù·.
∫ÔÏÔÎÔÈÏÈ·Îfi˜ ·ÔÎÏÂÈÛÌfi˜ 2Ô˘ ‚·ıÌÔ‡ (Ù‡-
Ô˘ 1): ∞·ÓÙ¿Ù·È Û˘¯Ó¿ Û ·ıÏËÙ¤˜ ˘„ËÏÒÓ
ÂȉfiÛÂˆÓ (26). √ Û˘ÓÈÛÙÒÌÂÓÔ˜ ¤ÏÂÁ¯Ô˜ Â-
ÚÈÏ·Ì‚¿ÓÂÈ ÂÎÙfi˜ ·fi ÙÔ ∏∫° Î·È ÙÔ Ë¯ÔηÚ-
‰ÈÔÁÚ¿ÊËÌ·, ‰ÔÎÈÌ·Û›· ÎÔÒÛˆ˜ Î·È Holter
Ú˘ıÌÔ‡. ∞Ó Û˘Ó˘¿Ú¯ÂÈ Î·È ·ÔÎÏÂÈÛÌfi˜ ÛΤ-
ÏÔ˘˜, ÙfiÙÂ Û˘ÓÈÛÙ¿Ù·È Î·È ËÏÂÎÙÚÔÊ˘ÛÈÔÏÔÁÈ-
΋ ÌÂϤÙË. ŸÙ·Ó Ô ·ÔÎÏÂÈÛÌfi˜ ‚ÂÏÙÈÒÓÂÙ·È
‹ ¤ÛÙˆ ‰ÂÓ ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿ ÙËÓ ÎfiˆÛË ‹
ÙËÓ Â·Ó·ÊÔÚ¿, ÂÈÙÚ¤ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ ÛÂ
fiÏ· Ù· ·ıÏ‹Ì·Ù·. ∞ÓÙ›ıÂÙ·, fiÙ·Ó Ô ·ÔÎÏÂÈ-
ÛÌfi˜ ÚˆÙÔÂÌÊ·Ó›˙ÂÙ·È ‹ ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿
ÙËÓ ÎfiˆÛË ‹ ÙËÓ Â·Ó·ÊÔÚ¿, ÙfiÙÂ Û˘ÓÈÛÙ¿-
Ù·È ËÏÂÎÙÚÔÊ˘ÛÈÔÏÔÁÈ΋ ÌÂϤÙË.
¶Ï‹Ú˘ ÎÔÏÔÎÔÈÏ·Îfi˜ ·ÔÎÏÂÈÛÌfi˜: √ ¤ÏÂÁ-
¯Ô˜ Á›ÓÂÙ·È Ì ∏∫°, ˯ÔηډÈÔÁÚ¿ÊËÌ·, ‰Ô-
ÎÈÌ·Û›· ÎÔÒÛˆ˜ Î·È Holter Ú˘ıÌÔ‡. ŸÙ·Ó
‰ÂÓ ˘¿Ú¯ÂÈ ÈÛÙÔÚÈÎfi Û˘ÁÎÔÙÈÎÒÓ ÂÂÈÛÔ-
‰›ˆÓ, Ù· QRS Â›Ó·È ÛÙÂÓ¿, Ë Î·Ú‰È·Î‹ Û˘¯Ófi-
ÙËÙ· Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË ÙˆÓ 40-50/min Î·È ·˘-
Í¿ÓÂÙ·È Ì ÙËÓ ÎfiˆÛË, Ë Î·Ú‰È¿ Â›Ó·È ·Ó·ÙÔ-
ÌÈο Ê˘ÛÈÔÏÔÁÈ΋ Ì ηϋ Û˘ÛÙÔÏÈ΋ ÏÂÈ-
ÙÔ˘ÚÁ›· Î·È ‰ÂÓ ˘¿Ú¯Ô˘Ó ¤ÎÙ·ÎÙ˜ ÎÔÈÏȷΤ˜
Û˘ÛÙÔϤ˜ ‹ ÎÔÈÏȷ΋ Ù·¯˘Î·Ú‰›· ηٿ ÙËÓ Îfi-
ˆÛË, ÙfiÙ ÂÈÙÚ¤ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ Û fiÏ·
Ù· ·ıÏ‹Ì·Ù·. ™Â ·ÓÙ›ıÂÙË ÂÚ›ÙˆÛË Ú¤ÂÈ
Ó· ÙÔÔıÂÙÂ›Ù·È ‚ËÌ·ÙÔ‰fiÙ˘ ÚÈÓ ÂÈÙڷ›
Ë ÂÓ·Û¯fiÏËÛË Ì ·ÓÙ·ÁˆÓÈÛÙÈο ·ıÏ‹Ì·Ù·,
ÔfiÙ ··ÁÔÚ‡ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ Û ·ıÏ‹Ì·-
Ù· Ì ΛӉ˘ÓÔ ÛˆÌ·ÙÈ΋˜ ÚfiÛÎÚÔ˘Û˘ (¶›-
ӷη˜ 2). ªÂÙ¿ ÙËÓ ÙÔÔı¤ÙËÛË ÙÔ˘ ‚ËÌ·ÙÔ-
‰fiÙË ÚÔËÁÂ›Ù·È ¿ÓÙ· ‰ÔÎÈÌ·Û›· ÎÔÒÛˆ˜
ÒÛÙ ӷ ‰È·ÈÛÙˆı› ηٿ fiÛÔÓ Ë Î·Ú‰È·Î‹
Û˘¯ÓfiÙËÙ· ÌÔÚ› Ó· ·˘ÍËı› ÛÙÔ ÂÈı˘ÌËÙfi
ÁÈ· ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ¿ıÏËÌ· ›‰Ô. ∆· ›‰È·
ÈÛ¯‡Ô˘Ó Î·È ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÎÔÏÔÎÔÈ-
ÏÈ·Îfi ·ÔÎÏÂÈÛÌfi 2Ô˘ ‚·ıÌÔ‡ Ù‡Ô˘ 2.
À¤ÚÙ·ÛË
∞Ó Î·È ‰ÂÓ ¤¯Ô˘Ó ·Ó·ÊÂÚı› ÂÂÈÛfi‰È· ·ÈÊ-
Ó›‰ÈÔ˘ ı·Ó¿ÙÔ˘ Û ˘ÂÚÙ·ÛÈο ¿ÙÔÌ·, ˘¿Ú¯ÂÈ
ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi Ë ÚÔηٿÏË„Ë fiÙÈ Â›-
Ó·È ÂÎÙÂıÂÈ̤ӷ Û ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ‰˘Û¿ÚÂ-
ÛÙˆÓ Û˘Ì‚·Ì¿ÙˆÓ, ÂÓÒ Â›Ó·È ·Ô‰Â‰ÂÈÁ̤ÓÔ
fiÙÈ ˆÊÂÏÔ‡ÓÙ·È ·fi ÙËÓ Ù·ÎÙÈ΋ ¿ıÏËÛË (27).
™Ù· ·È‰È¿ Ë ‰Â˘ÙÂÚÔ·ı‹˜ ˘¤ÚÙ·ÛË Â›Ó·È
ÈÔ Û˘¯Ó‹ ·’ fi,ÙÈ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ Î·È Â›Ó·È Â˘-
ÓfiËÙÔ fiÙÈ ÛÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜ Ú¤ÂÈ Ó·
·ÓÙÈÌÂÙˆ›˙ÂÙ·È ÙÔ ÚˆÙÔ·ı¤˜ ·›ÙÈÔ ÚÔÙÔ‡
ÂÈÙڷ› Ë ÂχıÂÚË Û˘ÌÌÂÙÔ¯‹ ÛÙËÓ ¿ıÏË-
ÛË. ™ÙËÓ ÚˆÙÔ·ı‹ ˘¤ÚÙ·ÛË ‰ÂÓ Ù›ıÂÓÙ·È
ÂÚÈÔÚÈÛÌÔ› ·Ó Â›Ó·È ‹ÈÔ˘ ‹ ̤ÙÚÈÔ˘ ‚·ıÌÔ‡
Î·È ‰ÂÓ ˘¿Ú¯ÂÈ ‚Ï¿‚Ë ÙˆÓ ÔÚÁ¿ÓˆÓ-ÛÙfi¯ˆÓ
(¶›Ó·Î˜ 3,4,5,6). ™Â ÂÚÈÙÒÛÂȘ ÛÔ‚·Ú‹˜
˘¤ÚÙ·Û˘ ··ÁÔÚ‡ÂÙ·È Ë Û˘ÌÌÂÙÔ¯‹ ÛÂ
·ıÏ‹Ì·Ù· Ù˘ ηÙËÁÔÚ›·˜ πππ.
∂›Ó·È ÁÓˆÛÙfi, fiÙÈ ·ÊÂÓfi˜ ÌÂÓ Ë ·ÚÙËÚȷ΋
˘¤ÚÙ·ÛË Û˘Ó‰˘¿˙ÂÙ·È Û˘¯Ó¿ Ì ·‡ÍËÛË ÙÔ˘
ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜ Î·È ·ÊÂÙ¤ÚÔ˘ fiÙÈ Ù· ÙÂÏ¢-
Ù·›· ¯ÚfiÓÈ· fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ ÛÙÔÓ
·Ó·Ù˘Á̤ÓÔ ÎfiÛÌÔ Â›Ó·È ·¯‡Û·Úη. °È· Ù·
·È‰È¿ ·˘Ù¿ Â›Ó·È ÂÍ·ÈÚÂÙÈο ÛËÌ·ÓÙÈÎfi Ó·
Û˘ÌÌÂÙ¤¯Ô˘Ó Û ÔÚÁ·ÓˆÌ¤Ó˜ Î·È Ù·ÎÙÈΤ˜
·ıÏËÙÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜, ÒÛÙ ӷ ÂÈÙ‡-
¯Ô˘Ó Ì›ˆÛË ÙÔ˘ ۈ̷ÙÈÎÔ‡ ÙÔ˘˜ ‚¿ÚÔ˘˜. ∆·
˘¤Ú‚·Ú· ·È‰È¿ Î·È ¤ÊË‚ÔÈ Ú¤ÂÈ Ó· Û˘Ì-
ÌÂÙ¤¯Ô˘Ó Û ·ıÏ‹Ì·Ù· Ù· ÔÔ›· ÏËÚÔ‡Ó ÙȘ
·Ú·Î¿Ùˆ ÚÔ¸Ôı¤ÛÂȘ ÒÛÙ ӷ ÂÈÙ˘Á¯¿-
ÓÂÙ·È ·ÚÓËÙÈÎfi ÂÓÂÚÁÂÈ·Îfi ÈÛÔ˙‡ÁÈÔ (28):
·) Ë ‰Ú·ÛÙËÚÈfiÙËÙ· Ú¤ÂÈ Ó· ÚÔηÏ›
ΛÓËÛË ÌÂÁ¿ÏˆÓ Ì˘˚ÎÒÓ ÔÌ¿‰ˆÓ (.¯. ‚¿‰È-
ÛÌ·, ÙÚ¤ÍÈÌÔ, Ô‰ËÏ·Û›·, ÎÔχ̂ËÛË, ¯ÔÚfi˜),
‚) Ë È‰·ÓÈ΋ ‰È¿ÚÎÂÈ· Ù˘ ¿ÛÎËÛ˘ Ú¤ÂÈ
Ó· Â›Ó·È 30-45 ÏÂÙ¿ ηıËÌÂÚÈÓ¿, ÒÛÙ ӷ
ÂÈÙ˘Á¯¿ÓÂÙ·È ·ÒÏÂÈ· 300 ÂÚ›Ô˘ ıÂÚÌ›-
‰ˆÓ. ∞˘Ùfi Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÈÙ¢¯ı› ·fi
ÙËÓ ·Ú¯‹ ÛÙ· ˘¤Ú‚·Ú· ·È‰È¿ Î·È ÁÈ’ ·˘Ùfi
Û˘ÓÈÛÙ¿Ù·È Ó· ·ıÏÔ‡ÓÙ·È ·Ú¯Èο 10 ÏÂÙ¿
ËÌÂÚËÛ›ˆ˜, Ù· ÔÔ›· ÛÙË Û˘Ó¤¯ÂÈ·, ÚÔԉ¢-
ÙÈο, ·˘Í¿ÓÔÓÙ·È,
Á) Ó· ‰›‰ÂÙ·È ÛËÌ·Û›· ÛÙË ‰È¿ÚÎÂÈ· Î·È ÛÙË
Û˘¯ÓfiÙËÙ· Ù˘ ¿ÛÎËÛ˘ Î·È fi¯È ÛÙËÓ ¤ÓÙ·ÛË
Ì ÙËÓ ÔÔ›· ·˘Ù‹ ÂÎÙÂÏÂ›Ù·È (.¯. Â›Ó·È ÚÔ-
ÙÈÌfiÙÂÚÔ ¤Ó· ¯·Ï·Úfi ÙÚ¤ÍÈÌÔ ÌÈÛ‹˜ ÒÚ·˜ ·fi
¤Ó· ¤ÓÙÔÓÔ 15 ÏÂÙÒÓ).
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
475ÕÛÎËÛË Î·È ·ı‹ÛÂȘ ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
™˘ÌÂÚ¿ÛÌ·Ù·
Ÿˆ˜ Á›ÓÂÙ·È ·ÓÙÈÏËÙfi ·fi Ù· ÚÔ·Ó·ÊÂÚ-ı¤ÓÙ· ÛÙÔȯ›·, ۯ‰fiÓ fiÏÔÈ ÔÈ Ó·ÚÔ› ·ÛıÂÓ›˜ÌÂ Û˘ÁÁÂÓ‹ ‹ ›ÎÙËÙË Î·Ú‰ÈÔ¿ıÂÈ· ÌÔÚÔ‡ÓÓ· Û˘ÌÌÂÙ¤¯Ô˘Ó Û οÔÈ· ·ıÏËÙÈ΋ ‰Ú·ÛÙË-ÚÈfiÙËÙ·, ¤ÛÙˆ Î·È Ôχ ‹È·. ¡· ÛËÌÂȈı› fiÙÈÔÈ ÚÔ·Ó·ÊÂÚı›Û˜ Ô‰ËÁ›Â˜ ·ÊÔÚÔ‡Ó ÙË Û˘Ì-ÌÂÙÔ¯‹ ÙÔ˘˜ Û ·ÓÙ·ÁˆÓÈÛÙÈο ·ıÏ‹Ì·Ù· Î·È fiÙÈ
·ÎfiÌ· Î·È fiÙ·Ó Ë ÛÔ‚·ÚfiÙËÙ· Ù˘ ηٿÛÙ·Û˘ÙÔ˘˜ ‰ÂÓ ÂÈÙÚ¤ÂÈ ÙÔÓ ·ÓÙ·ÁˆÓÈÛÙÈÎfi ·ıÏËÙÈ-ÛÌfi, ÌÔÚÔ‡Ó Î·È Ú¤ÂÈ Ó· ‰È·ÙËÚÔ‡Ó Î¿ÔÈԢ›‰Ô˘˜ Ê˘ÛÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· „˘¯·ÁˆÁÈÎÔ‡ ¯·-Ú·ÎÙ‹Ú·, ÂȉÈο ˘fi ÙËÓ ÌÔÚÊ‹ οÔÈÔ˘ ÔÌ·‰È-ÎÔ‡ ·ıÏ‹Ì·ÙÔ˜, Ì ÙËÓ ÚÔ¸fiıÂÛË ‚¤‚·È·, fiÙÈÙfiÛÔ Ô ·ÛıÂÓ‹˜ fiÛÔ Î·È Ô Î·ıËÁËÙ‹˜ ۈ̷ÙÈ΋˜·ÁˆÁ‹˜ ¤¯Ô˘Ó ›ÁÓˆÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ηÈÙˆÓ ÂÚÈÔÚÈÛÌÒÓ Ô˘ ÂÈ‚¿ÏÏÔÓÙ·È. Ÿˆ˜ ›ӷÈ
¶›Ó·Î·˜ 3. ∆·ÍÈÓfiÌËÛË ˘¤ÚÙ·Û˘ ÛÙ· ·ÁfiÚÈ· ·Ó¿ÏÔÁ· Ì ÙÔ ‡„Ô˜ Î·È ÙËÓ ËÏÈΛ· (·fi 1-10 ÂÙÒÓ)
HÏÈΛ· ∞¶ ™˘ÛÙÔÏÈ΋ ∞¶ (mmHg) ¢È·ÛÙÔÏÈ΋ ∞¶ (mmHg)(¤ÙË) ∂ηÙÔÛÙÈ·›· ∂ηÙÔÛÙÈ·›· ı¤ÛË ‡„Ô˘˜ ∂ηÙÔÛÙÈ·›· ı¤ÛË ‡„Ô˘˜
ı¤ÛË
5Ë 10Ë 25Ë 50‹ 75Ë 90‹ 95Ë 5Ë 10Ë 25Ë 50‹ 75Ë 90‹ 95Ë
1 90‹ 94 95 97 99 100 102 103 49 50 51 52 53 53 5495Ë 98 99 101 103 104 106 106 54 54 55 56 57 58 58
2 90‹ 97 99 100 102 104 105 106 54 55 56 57 58 58 5995Ë 101 102 104 106 108 109 110 59 59 60 61 62 63 63
3 90‹ 100 101 103 105 107 108 109 59 59 60 61 62 63 6395Ë 104 105 107 109 110 112 113 63 63 64 65 66 67 67
4 90‹ 102 103 105 107 109 110 111 62 63 64 65 66 66 6795Ë 106 107 109 111 112 114 115 66 67 68 69 70 71 71
5 90‹ 104 105 106 108 110 111 112 65 66 67 68 69 69 7095Ë 108 109 110 112 114 115 116 69 70 71 72 73 74 74
6 90‹ 105 106 108 110 111 113 113 68 68 69 70 71 72 7295Ë 109 110 112 114 115 117 117 72 72 73 74 75 76 76
7 90‹ 106 107 109 111 113 114 115 70 70 71 72 73 74 7495Ë 110 111 113 115 117 118 119 74 74 75 76 77 78 78
8 90‹ 107 109 110 112 114 115 116 71 72 72 73 74 75 7695Ë 111 112 114 116 118 119 120 75 76 77 78 79 79 80
9 90‹ 109 110 112 114 115 117 118 72 73 74 75 76 76 7795Ë 113 114 116 118 119 121 121 76 77 78 79 80 81 81
10 90‹ 111 112 114 115 117 119 119 73 73 74 75 76 77 7895Ë 115 116 117 119 121 122 123 77 78 79 80 81 81 82
11 90‹ 113 114 115 117 119 120 121 74 74 75 76 77 78 7895Ë 117 118 119 121 123 124 125 78 78 79 80 81 82 82
12 90‹ 115 116 118 120 121 123 123 74 75 75 76 77 78 7995Ë 119 120 122 123 125 127 127 78 79 80 81 82 82 83
13 90‹ 117 118 120 122 124 125 126 75 75 76 77 78 79 7995Ë 121 122 124 126 128 129 130 79 79 80 81 82 83 83
14 90‹ 120 121 123 125 126 128 128 75 76 17 78 79 79 8095Ë 124 125 127 128 130 132 132 80 80 81 82 83 84 84
15 90‹ 122 124 125 127 129 130 131 76 77 78 79 80 80 8195Ë 126 127 129 131 133 134 135 81 81 82 83 84 85 85
16 90‹ 125 126 128 130 131 133 134 78 78 79 80 81 82 8295Ë 129 130 132 134 135 137 137 82 83 83 84 85 86 87
17 90‹ 127 128 130 132 134 135 136 80 80 81 82 83 84 8495Ë 131 132 134 136 138 139 140 84 85 86 87 87 88 89
476 ¢. °ÂˆÚÁ·ÎfiÔ˘ÏÔ˜, °. ¶··‰fiÔ˘ÏÔ˜
¶·È‰È·ÙÚÈ΋ 2006;69:468-477
¶›Ó·Î·˜ 5. ∆·ÍÈÓfiÌËÛË ˘¤ÚÙ·Û˘ ÛÙ· ÎÔÚ›ÙÛÈ· ·Ó¿ÏÔÁ· Ì ÙÔ ‡„Ô˜ Î·È ÙËÓ ËÏÈΛ· (1-10 ÂÙÒÓ)
HÏÈΛ· ∞¶ ™˘ÛÙÔÏÈ΋ ∞¶ (mmHg) ¢È·ÛÙÔÏÈ΋ ∞¶ (mmHg)(¤ÙË) ∂ηÙÔÛÙÈ·›· ∂ηÙÔÛÙÈ·›· ı¤ÛË ‡„Ô˘˜ ∂ηÙÔÛÙÈ·›· ı¤ÛË ‡„Ô˘˜
ı¤ÛË
5Ë 10Ë 25Ë 50‹ 75Ë 90‹ 95Ë 5Ë 10Ë 25Ë 50‹ 75Ë 90‹ 95Ë
1 90‹ 97 97 98 100 101 102 103 52 53 53 54 55 55 5695Ë 100 101 102 104 105 106 107 56 57 57 58 59 59 60
2 90‹ 98 99 100 101 103 104 105 57 58 58 59 60 61 6195Ë 102 103 104 105 107 108 109 61 62 62 63 64 65 65
3 90‹ 100 100 102 103 104 106 106 61 62 62 63 64 64 6595Ë 104 104 105 107 108 109 110 65 66 66 67 68 68 69
4 90‹ 101 102 103 104 106 107 108 64 64 65 66 67 67 6895Ë 105 106 107 108 110 111 112 68 68 69 70 71 71 72
5 90‹ 103 103 105 106 107 109 109 66 67 67 68 69 69 7095Ë 107 107 108 110 111 112 113 70 71 71 72 73 73 74
6 90‹ 104 105 106 108 109 110 111 68 68 69 70 70 71 7295Ë 108 109 110 111 113 114 115 72 72 73 74 74 75 76
7 90‹ 106 107 108 109 111 112 113 69 70 70 71 72 72 7395Ë 110 111 112 113 115 116 116 73 74 74 75 76 76 77
8 90‹ 108 109 110 111 113 114 114 71 71 71 72 73 74 7495Ë 112 112 114 115 116 118 118 75 75 75 76 77 78 78
9 90‹ 110 110 112 113 114 j-.e 116 72 72 72 73 74 75 7595Ë 114 114 115 117 118 119 120 76 76 76 11 78 79 79
10 90‹ 112 112 114 115 116 118 118 73 73 73 74 75 76 7695Ë 116 116 117 119 120 121 122 77 77 77 78 79 80 80
11 90‹ 114 114 116 117 118 119 120 74 74 74 75 76 77 7795Ë 118 118 119 121 122 123 124 78 78 78 79 80 81 81
12 90‹ 116 116 117 119 120 121 122 75 75 75 76 77 78 7895Ë 119 120 121 123 124 125 126 79 79 79 80 81 82 82
13 90‹ 117 118 119 121 122 123 124 76 76 76 77 78 79 7995Ë 121 122 123 124 126 127 128 80 80 80 81 82 S3 83
14 90‹ 119 120 121 122 124 125 125 77 77 77 78 79 80 8095Ë 123 123 125 126 127 129 129 81 81 81 82 83 84 84
15 90‹ 120 121 122 123 125 126 127 78 78 78 79 80 81 8195Ë 124 125 126 127 129 130 131 82 82 82 83 84 85 85
16 90‹ 121 122 123 124 126 127 128 78 78 79 80 81 81 8295Ë 125 126 127 128 130 131 132 82 82 83 84 85 85 86
17 90‹ 122 122 123 125 126 127 128 78 79 79 80 81 81 8295Ë 125 126 127 129 130 131 132 82 83 83 84 85 85 86
‡ÎÔÏ· ·ÓÙÈÏËÙfi, Ù· ÔʤÏË ·fi ÙËÓ „˘¯·ÁˆÁÈ-
΋ ·˘Ù‹ ÂÓ·Û¯fiÏËÛË Â›Ó·È ÔÏÏ·Ï¿, ÙfiÛÔ ÛÂ
Â›Â‰Ô Ê˘ÛÈ΋˜ fiÛÔ Î·È „˘¯È΋˜ ˘Á›·˜.
µÈ‚ÏÈÔÁÚ·Ê›·
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Med 2003;349:1064-1075.
2. Corrado D, Pelliccia A, Bjornstad HH, Vanhees L,
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478 µπµ§πO¶∞ƒOÀ™π∞™∏ BOOK REVIEW
¶·È‰È·ÙÚÈ΋ 2006;69:478
“∫ÏÈÓÈ΋ ¶·È‰È·ÙÚÈ΋”
Àfi ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ
∂ΉfiÛÂȘ ∑∏∆∞
∞ı‹Ó·, 2006
∆ÔÓ ™Â٤̂ÚÈÔ 2006 ΢ÎÏÔÊfiÚËÛ ·fi ÙȘ È·ÙÚÈΤ˜ ÂΉfiÛÂȘ “∑∏∆∞”ÙÔ Ó¤Ô ‚È‚Ï›Ô ¶·È‰È·ÙÚÈ΋˜ “∫ÏÈÓÈ΋ ¶·È‰È·ÙÚÈ΋”. ∂›Ó·È ¤ÚÁÔ ‰›ÙÔÌÔ, Û¯Â-‰fiÓ 1.600 ÛÂÏ›‰ˆÓ Î·È ÚÔ¤Ú¯ÂÙ·È ·fi ÙË µã ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ÙÔ˘ ¶·ÓÂ-ÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ.
∏ “∫ÏÈÓÈ΋ ¶·È‰È·ÙÚÈ΋” ·ÔÙÂÏ› ÙËÓ ÔÏÔÎÏ‹ÚˆÛË ÙÔ˘ ÛÙfi¯Ô˘ Ô˘¤ıÂÛ ÚÈÓ ·fi Ù¤ÛÛÂÚ· ÂÚ›Ô˘ ¯ÚfiÓÈ· Ô Î·ıËÁËÙ‹˜ Î. ∞. ∫ˆÓÛÙ·ÓÙfi-Ô˘ÏÔ˜, Ó· ÚÔÛʤÚÂÈ ‰ËÏ·‰‹, ¤Ó· Û‡Á¯ÚÔÓÔ ‚È‚Ï›Ô ¶·È‰È·ÙÚÈ΋˜ ÛÙÔ˘˜ŒÏÏËÓ˜ ÊÔÈÙËÙ¤˜ Ù˘ π·ÙÚÈ΋˜, ηıÒ˜ Î·È ÛÙÔ˘˜ ·È‰È¿ÙÚÔ˘˜. °È· ÙË Û˘Á-ÁÚ·Ê‹ ÙˆÓ ‰È·ÊfiÚˆÓ ÎÂÊ·Ï·›ˆÓ Ô Î. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ ¤ÏÂÍ 100 ÂÚ›-Ô˘ ̤ÏË ÙÔ˘ ¢.∂.¶. ·fi fiϘ ÙȘ π·ÙÚÈΤ˜ ™¯ÔϤ˜ Ù˘ ¯ÒÚ·˜ ηıÒ˜ Î·È ÛÙÂ-Ϥ¯Ë ÙÔ˘ ∂.™.À.
∏ ηٷÓÔÌ‹ ·˘Ù‹ Ù˘ ÚÔÛ¿ıÂÈ·˜ ‚Ô‹ıËÛ ÛÙË Û¯ÂÙÈο Û‡ÓÙÔÌË ÔÏÔ-ÎÏ‹ÚˆÛË ÙÔ˘ ¤ÚÁÔ˘ Î·È Î·Ù¿ Û˘Ó¤ÂÈ· ÛÙËÓ ÈÔ Û‡Á¯ÚÔÓË ‡ÏË ÙÔ˘. ∞ÎfiÌË, ËÛ˘ÁÁÚ·Ê‹ ·fi ÂȉÈÎÔ‡˜ ·ÓıÚÒÔ˘˜, ηٷÍȈ̤ÓÔ˘˜ Û ‰È¿ÊÔÚ˜ ˘ÔÂȉÈÎfi-ÙËÙ˜, Ì ·ÚÔ˜ ÛÙÔÓ ¯ÒÚÔ Î·È Ì ‚·ıÈ¿ ÁÓÒÛË ÙˆÓ ‰È·ÊfiÚˆÓ ıÂÌ¿ÙˆÓ,
ÚÔÛ‰›‰ÂÈ ÛÙÔ ‚È‚Ï›Ô ÌÂÁ·Ï‡ÙÂÚÔ ÂȉÈÎfi ‚¿ÚÔ˜. µ¤‚·È·, Ë ÔÏ˘‰È¿Û·ÛË ·˘Ù‹ ¤¯ÂÈ ÌÔÈÚ·›·, ·ÚÓËÙÈΤ˜ ÂÈÙÒÛÂȘ, fiˆ˜ Ë Û¯ÂÙÈ΋ ·ÓÈÛÔ̤ÚÂÈ· ÛÙËÓ
·Ó¿Ù˘ÍË ÙÔ˘ οı ÎÂÊ·Ï·›Ô˘, ηıÒ˜ Î·È ÛÙÔ ‰È·ÊÔÚÂÙÈÎfi ‡ÊÔ˜. ∆Ô ÙÂÏÂ˘Ù·›Ô, ηٿ ÙËÓ ÚÔÛˆÈ΋ ÌÔ˘ÁÓÒÌË, ·ÔÙÂÏ› ·ÓÙÈÛÙÚfiʈ˜ Î·È ÏÂÔÓ¤ÎÙËÌ·, ÁÈ·Ù› ·ÓÙ·Ó·ÎÏ¿ ÙËÓ ÚÔÛˆÈÎfiÙËÙ· ÙÔ˘ οıÂ Û˘ÁÁڷʤ·Î·È ÚÔÛ‰›‰ÂÈ Û οı ÎÂÊ¿Ï·ÈÔ È‰È·›ÙÂÚË ÁÔËÙ›·.
¶ÈÛÙ‡ˆ fiÙÈ Ë “∫ÏÈÓÈ΋ ¶·È‰È·ÙÚÈ΋” ηχÙÂÈ ÛËÌ·ÓÙÈÎfi ÎÂÓfi ÛÙÔÓ ·È‰È·ÙÚÈÎfi ¯ÒÚÔ, ÁÈ·Ù› ‰ÂÓ ˘¿Ú-¯ÂÈ ·ÓÙ›ÛÙÔȯfi ÙÔ˘ ÛÙËÓ ∂ÏÏ¿‰·, ÁÈ·Ù› Â›Ó·È Û‡Á¯ÚÔÓÔ Î·È, ÂÂȉ‹ Â›Ó·È ÁÚ·Ì̤ÓÔ ÛÙË ÌËÙÚÈ΋ ÁÏÒÛÛ·,ÚÔÛʤÚÂÙ·È ÁÈ· ÈÔ Â‡ÎÔÏË ·Ó¿ÁÓˆÛË Î·È ÌÂϤÙË. ™˘ÓÈÛÙԇ̠·ÓÂÈʇϷÎÙ· ÙÔ ‚È‚Ï›Ô ·˘Ùfi, Ô˘ ‰ÂÓ ı·Ú¤ÂÈ Ó· Ï›„ÂÈ ·fi ÙË ‚È‚ÏÈÔı‹ÎË ÙˆÓ ·È‰È¿ÙÚˆÓ. ÷ÈÚÂÙ›˙Ô˘Ì ÙËÓ ÚÔÛ¿ıÂÈ·, ¢¯fiÌ·ÛÙ ηϋ ÂÈ-Ù˘¯›· Î·È ÂÏ›˙Ô˘Ì Û ۇÓÙÔÌË Â·Ó¤Î‰ÔÛË.
¡ÈÎfiÏ·Ô˜ ª˘ÚÈÔÎÂÊ·ÏÈÙ¿Î˘,¢È¢ı˘ÓÙ‹˜ ∞ã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜, °.¡.¶. ¶ÂÓÙ¤Ï˘
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, °.¡.¶. ¶ÂÓÙ¤Ï˘
AÏÏËÏÔÁÚ·Ê›·:
¡ÈÎfiÏ·Ô˜ ª˘ÚÈÔÎÂÊ·ÏÈÙ¿Î˘
479∫§π¡π∫√ ∫√Àπ∑
¶·È‰È·ÙÚÈ΋ 2006;69:479,482
∞. ¶··‰ÔÔ‡ÏÔ˘1, °. ƒÔ˘Ì¿ÓË1, §. ™Ù·ÌÔÁÈ¿ÓÓÔ˘1, ∂. ∫·ÙÛ·ÚÔ‡ - ¶ÂÎÙ·Û›‰Ë2
∫oÚ›ÙÛÈ 8,5 ÂÙÒÓ ÂÈÛ‹¯ıË Ì ·‰˘Ó·Ì›· ‚¿‰ÈÛË˜Î·È ÔÚıÔ΢ÛÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ ·fi 12ÒÚÔ˘, ηıÒ˜Î·È Ì˘·ÏÁ›· Î·È ¿ÏÁÔ˜ ÛÙËÓ ÔÛʇ ·fi 48ÒÚÔ˘.¶ÚÔ ÂÓıË̤ÚÔ˘ ·ÚÔ˘Û›·Û Â̇ÚÂÙÔ Û˘Óԉ¢-fiÌÂÓÔ ·fi ‚‹¯· Î·È ÎÂÊ·Ï·ÏÁ›·, Ô˘ ‰È‹ÚÎÂÛ·Ó36 ÒÚ˜. ∞fi ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË Î·Ù¿ ÙËÓÂÈÛ·ÁˆÁ‹, ‰È·ÈÛÙÒıËÎÂ Ì˘˚΋ ·‰˘Ó·Ì›· ÛÙ· ο-Ùˆ ¿ÎÚ· Î·È ‰˘ÛÎÔÏ›· ÔÚıÔÛÙ¿ÙËÛ˘. ∆· ÙÂÓfiÓÙÈ··ÓÙ·Ó·ÎÏ·ÛÙÈο ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. ∏ ηٿ Û˘-ÛÙ‹Ì·Ù· ÂͤٷÛË ‹Ù·Ó ÂÓÙfi˜ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ.
∞fi ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ÚԤ΢„·Ó Ù··Ú·Î¿Ùˆ: ÁÂÓÈ΋ ·›Ì·ÙÔ˜ Î·È ·‰Úfi˜ ‚ÈÔ¯ËÌÈÎfi˜¤ÏÂÁ¯Ô˜: Î.Ê., ÁÂÓÈ΋ ∂¡À (ÙËÓ 5Ë Ë̤ڷ ·fi ÙËÓ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘): ·ÙÙ·Ú· 240 (Ù‡Ô˜ ÏÂÌÊÔ-΢ÙÙ·ÚÈÎfi˜), χΈ̷ 30 mg/dl, ۿί·ÚÔ 49mg/dl, ¿ÌÂÛÔ (-). ∆· ·ÓÙÈÛÒÌ·Ù· ÛÙÔ ∂¡À ÁÈ··‰ÂÓÔ˚Ô‡˜, ÂÚËÙo˚o‡˜, ÂÓÙÂÚo˚o‡˜, ÁÚ›Ë, ·Ú·-ÁÚ›Ë, CMV Î·È EBV ‹Ù·Ó fiÏ· ·ÚÓËÙÈο. ∏ PCRÛÙÔ ∂¡À ÁÈ· µorellia burgdorferi Î·È HSV1 Î·È HSV2
‹Ù·Ó ›Û˘ ·ÚÓËÙÈ΋. ∞fi ÙÔÓ ÔÚÔÏÔÁÈÎfi ¤ÏÂÁ¯ÔÚԤ΢„·Ó Ù· ·Ú·Î¿Ùˆ: C3: 194 mg/dl (º∆ 52-120), C4: 36 mg/dl, ASTO (-), ANA 1:160, antiDNA: (-), ÂÓÒ ÔÈ IgG, IgA Î·È IgM ‹Ù·Ó ÂÓÙfi˜ Ê˘-
ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ Î·È Ù· ·ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ ENA,RNP, Sm, SSA Î·È SSB fiÏ· ·ÚÓËÙÈο. ∆· ·ÓÙÈÛÒ-Ì·Ù· ÛÙÔÓ ÔÚfi ÁÈ· ¯Ï·Ì‡‰È·, HSV, CMV, EBV,Toxoplasma, Bartonella, Influenza A, B, ·‰ÂÓÔ˚Ô‡˜,ÂÓÙÂÚÔ˚Ô‡˜ ‹Ù·Ó fiÏ· ·ÚÓËÙÈο, ÂÓÒ, fiÛÔÓ ·ÊÔÚ¿Ù· ·ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ ÙÔ˘ Ì˘ÎÔÏ¿ÛÌ·ÙÔ˜ Ù˘Ó¢ÌÔÓ›·˜, ‚Ú¤ıËÎ·Ó ¤ÓÙÔÓ· ıÂÙÈο Ù· IgM ηȷÛıÂÓÒ˜ ıÂÙÈο Ù· IgG. √ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ∂¡À ÁÈ·ÔÏÈÁÔÎψÓÈο ·ÓÙÈÛÒÌ·Ù· ‹Ù·Ó ·ÚÓËÙÈÎfi˜. ∏ Ù·-¯‡ÙËÙ· ·ÁˆÁ‹˜ ÓÂ‡ÚˆÓ ‹Ù·Ó ÂÓÙfi˜ Ê˘ÛÈÔÏÔÁÈÎÒÓÔÚ›ˆÓ. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘ ‹Ù·ÓÊ˘ÛÈÔÏÔÁÈ΋, ÂÓÒ Ù˘ ıˆÚ·ÎÈ΋˜ Î·È Ù˘ ÔÛÊ˘˚-΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ ¤‰ÂÈÍ ÂÏ·-ÊÚ¿ ‰ÈfiÁΈÛË ÙÔ˘ Ì˘ÂÏÈÎÔ‡ ۈϋӷ ÛÙËÓ ·ÓÒÙÂ-ÚË ıˆÚ·ÎÈ΋ Î·È ÛÙËÓ Î·ÙÒÙÂÚË ·˘¯ÂÓÈ΋ ÌÔ›Ú·Ù‹˜ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ ηıÒ˜ Î·È ·˘ÍË̤ÓÔ Û‹-Ì· ÛÙËÓ ∆2 ·ÎÔÏÔ˘ı›· (∂ÈÎfiÓ· 1).
¶Èı·Ó¤˜ ‰È·ÁÓÒÛÂȘ:1. Guillain Barré2. ™ÎÏ‹Ú˘ÓÛË Î·Ù¿ Ͽη˜3. ∂ÁοÚÛÈ· Ì˘ÂÏ›Ùȉ·4. ™˘ÛÙËÌ·ÙÈÎfi˜ ÂÚ˘ıËÌ·Ù҉˘ χÎÔ˜
1 ∞’ ¶·È‰È·ÙÚÈ΋ ¶·È‰È·ÙÚÈ΋∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌ›Զ·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”
2 ¡Â˘ÚÔÏÔÁÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”
AÏÏËÏÔÁÚ·Ê›·:
∞ÏÂÍ¿Ó‰Ú· ¶··‰ÔÔ‡ÏÔ˘E-mail: [email protected]
∏ ·¿ÓÙËÛË ·ÎÔÏÔ˘ı› ÛÙË ÛÂÏ›‰· 482
480 ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√
¶·È‰È·ÙÚÈ΋ 2006;69:480-481
µ’ ¶·È‰È·ÙÚÈ΋¶·ÓÂÈÛÙËÌȷ΋ ∫ÏÈÓÈ΋,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”, ∞ı‹Ó·
AÏÏËÏÔÁÚ·Ê›·:
¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜E-mail: [email protected]
¡›ÎÔ˜ ¶··‰fiÔ˘ÏÔ˜, ¶·È‰›·ÙÚÔ˜, ∞ÏÏÂÚÁÈÔÏfiÁÔ˜
™‡Á¯ÚÔÓ· ËÏÂÎÙÚÔÓÈο ÓËÈ·ÁˆÁ›·
∞˘Ùfi ÙÔ Ì‹Ó·, Ë ÛÙ‹ÏË ·ÚÔ˘ÛÈ¿˙ÂÈ ÂȉÈÎfi ·ÊȤڈ̷ ÛÙÔ˘˜ ‰ÈÎÙ˘·ÎÔ‡˜ ÙfiÔ˘˜ Ì ÂÎ·È‰Â˘-ÙÈÎfi Î·È ÂÓËÌÂÚˆÙÈÎfi ÂÚȯfiÌÂÓÔ Û¯ÂÙÈο Ì ÙËÓ ÔÚı‹ ¯Ú‹ÛË ÙÔ˘ ¢È·‰ÈÎÙ‡Ô˘ ·fi Ù· ·È‰È¿ ηÈÙÔ˘˜ ÁÔÓ›˜. ∫·Ï‹ ÏÔ‹ÁËÛË!
¶·›˙ˆ Î·È Ì·ı·›Óˆ ·fi ÙÔ ÿÓÙÂÚÓÂÙ! – http://pbskids.org/
√ ‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ pbskids.org ·Ó‹ÎÂÈ ÛÙÔÓ ·ÌÂÚÈηÓÈ-Îfi Ú·‰ÈÔÙËÏÂÔÙÈÎfi ÊÔÚ¤· ‰ËÌÔÛ›Ô˘ Û˘ÌʤÚÔÓÙÔ˜,Public Broadcasting Service (PBS) Î·È ¤¯ÂÈ ˆ˜ ›ÎÂÓÙÚÔÙËÓ ËÏÂÎÙÚÔÓÈ΋ ‰È··È‰·ÁÒÁËÛË ÙˆÓ ·È‰ÈÒÓ, ÙˆÓ ÁÔÓ¤-ˆÓ Î·È ÙˆÓ ‰·ÛÎ¿ÏˆÓ ÁÈ· ÙËÓ ÚÔÛÙ·Û›· Î·È ÙË ıˆÚ¿ÎÈÛ‹ÙÔ˘˜ ·¤Ó·ÓÙÈ ÛÙ· Ó¤· ª¤Û· ∂ÈÎÔÈÓˆÓ›·˜, Î·È ÂȉÈÎfiÙÂ-Ú· ÛÙÔ ¢È·‰›ÎÙ˘Ô. ∞ÒÙÂÚÔ˜ ÛÙfi¯Ô˜ Â›Ó·È Ë Î·Ù·Ú¯¿˜ ıÂ-ÙÈ΋ ÚÔÛ¤ÁÁÈÛË ·¤Ó·ÓÙÈ ÛÙÔ ÿÓÙÂÚÓÂÙ Î·È Ë ÂÍÔÈΛˆÛ‹ÙÔ˘˜ Ì ÙÔ Ó¤Ô ·˘Ùfi ̤ÛÔ ÁÈ· „˘¯·ÁˆÁÈÎÔ‡˜ Î·È ÂηÈ-‰Â˘ÙÈÎÔ‡˜ ÛÎÔÔ‡˜, ‰ËÌÈÔ˘ÚÁÒÓÙ·˜ ¤Ó· ÂÓ ‰˘Ó¿ÌÂÈ “ËÏÂ-ÎÙÚÔÓÈÎfi ÓËÈ·ÁˆÁ›Ԕ.
∏ ÎÂÓÙÚÈ΋ ÛÂÏ›‰· (Homepage) Â›Ó·È ÂÓÙ˘ˆÛȷ΋ Ì ÔÏÏ¿ ¯ÚÒÌ·Ù·, ‹¯Ô˘˜ Î·È ÔÏϤ˜ ‰È·-‰Ú·ÛÙÈΤ˜ ÏÂÈÙÔ˘ÚÁ›Â˜ ·Ú·¤ÌÔÓÙ·˜ Û ·È¯Ó›‰È· ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜. √È ‚·ÛÈΤ˜ ηÙËÁÔ-ڛ˜, Â›Ó·È ÙÚÂȘ, PBS °È· ¢·ÛοÏÔ˘˜, PBS °È· ¶·È‰È¿ Î·È PBS °È· °ÔÓ›˜, Ë Î·ıÂÌ›· Ì ·ÓÙ›ÛÙÔȯÔÂÚȯfiÌÂÓÔ Î·È ‰È·ÊÔÚÂÙÈÎfi ۯ‰ȷÛÌfi. √È 3 ·˘Ù¤˜ ηÙËÁÔڛ˜ ÏÂÈÙÔ˘ÚÁÔ‡Ó Ù·˘Ùfi¯ÚÔÓ· Î·È ˆ˜‰ÈÎÙ˘·Î¤˜ ‡Ï˜ ÁÈ· ÙfiÔ˘˜ Ì ·ÚÂÌÊÂÚ¤˜ ÂÚȯfiÌÂÓÔ. π‰È·›ÙÂÚÔ ÂӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ ËηÙËÁÔÚ›· PBS °È· ¶·È‰È¿, Ë ÔÔ›· ·Â˘ı‡ÓÂÙ·È Û ÌÈÎÚ¤˜ ËÏÈ˘ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ ·È¯Ó›‰È·,‰È·ÁˆÓÈÛÌÔ‡˜, ˙ˆÁÚ·ÊÈ΋ ÎÈÓÔ˘Ì¤ÓˆÓ Û¯Â‰›ˆÓ ·ÏÏ¿ Î·È ·Ú·Ì‡ıÈ·, Ù· ÔÔ›· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Û‰ȷ‰Ú·ÛÙÈ΋ ÌÔÚÊ‹. ∆· ·È‰È¿-¯Ú‹ÛÙ˜ ¤¯Ô˘Ó ›Û˘ ÙË ‰˘Ó·ÙfiÙËÙ·, Ó· ·ÎÔ‡ÛÔ˘Ó ÌÔ˘ÛÈο ÎÔÌ-Ì¿ÙÈ· Î·È ÛÙË Û˘Ó¤¯ÂÈ· Ó· Û˘Óı¤ÛÔ˘Ó Ù· ›‰È· ÙË ÌÔ˘ÛÈ΋ Ô˘ ¿ÎÔ˘Û·Ó, ‹ ÔÔÈ·‰‹ÔÙ ¿ÏÏË ÌÔ˘-ÛÈ΋ Û‡ÓıÂÛË ÂÈı˘ÌÔ‡Ó. ∏ ηÙËÁÔÚ›· PBS °È· °ÔÓ›˜ ÂÚÈÏ·Ì‚¿ÓÂÈ ÏËÚÔÊÔڛ˜ Î·È Û˘Ì‚Ô˘Ï¤˜Û¯ÂÙÈο Ì ÙËÓ „˘¯Ôۈ̷ÙÈ΋ ·Ó¿Ù˘ÍË Î·È Û˘ÌÂÚÈÊÔÚ¿ ÙˆÓ ·È‰ÈÒÓ Î·ıÒ˜ Î·È Ô‰ËÁ›Â˜ ÁÈ· ÌÈ··ÔÙÂÏÂÛÌ·ÙÈ΋ ÂÈÎÔÈÓˆÓȷ΋ ÚÔÛ¤ÁÁÈÛË, Ï·Ì‚¿ÓÔÓÙ·˜ ˘fi„ÈÓ ÙÔÓ Â˘·›ÛıËÙÔ „˘¯ÈÎfi ÙÔ˘˜ÎfiÛÌÔ. ∆¤ÏÔ˜, ÛÙËÓ Î·ÙËÁÔÚ›· PBS °È· ¢·ÛοÏÔ˘˜ Â›Ó·È ‰È·ı¤ÛÈÌÔ˜ Ô‰ËÁfi˜ Ì ‚·ÛÈΤ˜ Ô‰ËÁ›Â˜ ηÈÚ·ÎÙÈΤ˜ ηϋ˜ ‰È‰·Ûηϛ·˜ ÛÙÔ Û¯ÔÏ›Ô. √ ‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ pbskids.org ¤¯ÂÈ ‰ËÌÈÔ˘ÚÁ‹ÛÂÈ Â›-Û˘ Î·È Ì›· ı˘Á·ÙÚÈ΋ ÈÛÙÔÛÂÏ›‰· http://pbskids.org/dontbuyit, Ë ÔÔ›· ·Â˘ı‡ÓÂÙ·È ÛÙÔ˘˜ ÁÔÓ›˜,‰·ÛοÏÔ˘˜ Î·È ·È‰È¿ - ηٷӷψ٤˜ Ì ÛÙfi¯Ô ÙËÓ ÂÓÂÚÁËÙÈ΋ ÚÔÛÙ·Û›· ÙÔ˘˜ ·fi ÙËÓ ÏËıÒ-Ú· ÚÔ˚fiÓÙˆÓ ÛÙÔ ¢È·‰›ÎÙ˘Ô.
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¶·È‰È·ÙÚÈ΋ 2006;69:480-481
∂ÈÎÔÈÓˆÓ›· …Î·È Â˘·ÈÛıËÛ›·! - http://www.media-awareness.ca/
∏ ÈÛÙÔÛÂÏ›‰· http://www.media-awareness.ca ·ÔÙÂÏ› ÙÔÓ›ÛËÌÔ ‰ÈÎÙ˘·Îfi ÙfiÔ ÙÔ˘ Media Awareness Network, ÂÓfi˜ ÌË-ÎÂÚ‰ÔÛÎÔÈÎÔ‡ ÔÚÁ·ÓÈÛÌÔ‡ Ô˘ ‰Ú‡ÂÈ ÛÙÔÓ ∫·Ó·‰¿ Î·È ¤¯ÂȈ˜ ·ÓÙÈΛÌÂÓÔ ÙËÓ Â˘·ÈÛıËÙÔÔ›ËÛË ÙˆÓ ÁÔÓ¤ˆÓ Î·È ÙˆÓ ‰·-ÛÎ¿ÏˆÓ ÛÙË ¯Ú‹ÛË ÙˆÓ Ó¤ˆÓ Ù¯ÓÔÏÔÁÈÒÓ. ™Ùfi¯Ô˜ Â›Ó·È Ë Î·-Ù¿ÚÙÈÛË Î·È Ë ÂÓË̤ڈÛË Û¯ÂÙÈο Ì ÙȘ ‚·ÛÈΤ˜ ÏÂÈÙÔ˘ÚÁ›Â˜ ÙˆÓÓ¤ˆÓ ª¤ÛˆÓ ∂ÈÎÔÈÓˆÓ›·˜ ÁÈ· ÙËÓ ·fiÎÙËÛË Ù˘ ··ÈÙÔ‡ÌÂÓ˘ÎÚÈÙÈ΋˜ ÛΤ„˘ Î·È Ù˘ ηٿ Û˘Ó¤ÂÈ· ÔÚı‹˜ ηٷӷψÙÈ΋˜Û˘ÌÂÚÈÊÔÚ¿˜. ∞˘Ùfi ÙÔ ÂÙ˘¯·›ÓÂÈ Ì¤Û· ·fi ÂȉÈο ۯ‰ȷṲ̂-Ó· ÚÔÁÚ¿ÌÌ·Ù·, ·fi Ù· ÔÔ›· ͯˆÚ›˙ÂÈ ÙÔ ÚfiÁÚ·ÌÌ· WebAwareness Canada, Ô˘ ÍÂΛÓËÛ ÙÔ 1999 Î·È Û˘Ó¯›˙ÂÙ·È Ì¤¯ÚÈ
Û‹ÌÂÚ·. √ ‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡ ·ÔÙÂÏ› ¤Ó· ̤ÛÔ ÚÔ‚ÔÏ‹˜ Ù˘ Û˘ÓÔÏÈ΋˜ ‰Ú¿Û˘ ηÈÙˆÓ ÂÈ̤ÚÔ˘˜ ÚˆÙÔ‚Ô˘ÏÈÒÓ ÙÔ˘. §ÂÈÙÔ˘ÚÁ› ›Û˘, Î·È ˆ˜ ËÁ‹ ÏËÚÔÊÔÚÈÒÓ Ì¤Û· ·fi blogs,forums ¿Óˆ Û ı¤Ì·Ù· η›ÚÈ·˜ ÛËÌ·Û›·˜ ÁÈ· ÁÔÓ›˜ Î·È ‰·ÛοÏÔ˘˜, fiˆ˜ Ë ·Ó··Ú¿ÛÙ·ÛË Ù˘ ‚›·˜,Ù· ÛÙÂÚÂfiÙ˘· ̤۷ ·fi Ù· ªª∂ Î.¿. ∂ӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ Ô ËÏÂÎÙÚÔÓÈÎfi˜ ηٿÏÔÁÔ˜ Ì ÔÙÈ-ÎÔ·Ô˘ÛÙÈÎfi ˘ÏÈÎfi ÁÈ· ÚÔÛˆÈ΋ ·ÏÏ¿ Î·È Â·ÁÁÂÏÌ·ÙÈ΋ ¯Ú‹ÛË, Ì ԉËÁ›Â˜ Î·È ÚÔÙ¿ÛÂȘ ÛˆÛÙ‹˜‰È··È‰·ÁÒÁËÛ˘ ÛÙ· ¡¤· ª¤Û·. ∏ ÈÛÙÔÛÂÏ›‰· Â›Ó·È ‰›ÁψÛÛË, ÛÙ· ∞ÁÁÏÈο Î·È Ù· °·ÏÏÈο, ÂÓÒÂÎÙfi˜ ·fi ÙË ÁÂÓÈ΋ ‰ÔÌ‹, ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È Í¯ˆÚÈÛÙ¤˜ ÂÓfiÙËÙ˜ ÚÔ˜ ÁÔÓ›˜ Î·È ‰·ÛοÏÔ˘˜.
Childnet International - http://www.childnet-int.org
™Ùfi¯Ô˜ ÙÔ˘ Childnet International Â›Ó·È Ë ‰ËÌÈÔ˘ÚÁ›· ÂÓfi˜·ÛʷϤÛÙÂÚÔ˘ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ ÁÈ· Ù· ·È‰È¿ ÛÙÔ ÿÓÙÂÚÓÂÙ. ¶Úfi-ÎÂÈÙ·È ÁÈ· ¤Ó·Ó ÔÚÁ·ÓÈÛÌfi ÌË-ÎÂÚ‰ÔÛÎÔÈÎÔ‡ ¯·Ú·ÎÙ‹Ú· ÔÔÔ›Ô˜, ̤۷ ·fi ¤Ó· ‰ÈÂıÓ¤˜ ‰›ÎÙ˘Ô Û˘ÓÂÚÁ·ÛÈÒÓ, ÂȯÂÈÚ›ÌÈ· ÈÛÔÚÚÔË̤ÓË ÚÔÛ¤ÁÁÈÛË ÛÙÔÓ ¯ÒÚÔ ÙÔ˘ ÿÓÙÂÚÓÂÙ Ì 3 ‚·-ÛÈÎÔ‡˜ ¿ÍÔÓ˜ ÂÓË̤ڈÛ˘: ÚfiÛ‚·ÛË Î·È ÚÔÒıËÛË ÔÈÔÙÈ-ÎÔ‡ ÂÚȯÔ̤ÓÔ˘, ∂˘·ÈÛıËÙÔÔ›ËÛË Î·È ¶ÚÔÛÙ·Û›·. ™ÙÔ Ï·›-ÛÈÔ ·˘Ùfi, Ë ÈÛÙÔÛÂÏ›‰· childnet-int.org ÏÂÈÙÔ˘ÚÁ› ˆ˜ ÂÓËÌÂÚˆ-ÙÈÎfi˜ ÎfiÌ‚Ô˜ ÙˆÓ ‰Ú·ÛÙËÚÈÔÙ‹ÙˆÓ ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡ Ì ‚·ÛÈ-ÎÔ‡˜ ·Ô‰¤ÎÙ˜ ÁÔÓ›˜ Î·È ·È‰È¿. ∞Ó¿ÌÂÛ· ÛÙ· ¤ÚÁ·-‰Ú¿ÛÂȘÌ ÛÙfi¯Ô ÙË ‰È··È‰·ÁÒÁËÛË Î·È ÙËÓ „˘¯·ÁˆÁ›· Â›Ó·È Î·È ÙÔKidsmart Ô˘ ·ÔÙÂÏ› ¤Ó· ·˘ÙfiÓÔÌÔ ÛÙËÓ Ô˘Û›· ‰ÈÎÙ˘·Îfi Ùfi-Ô Ì ÂÍ·ÙÔÌÈÎÂ˘Ì¤ÓÔ ÂÚȯfiÌÂÓÔ ÁÈ· ·È‰È¿ (·È¯Ó›‰È·,chats, quiz, test ÁÓÒÛˆÓ), ÁÈ· ‰·ÛοÏÔ˘˜ ÁÈ· ÙËÓ ÂÈÌfiÚʈÛË
Û¯ÂÙÈο Ì ÙËÓ ·ÛÊ·Ï‹ ¯Ú‹ÛË ÙÔ˘ ÿÓÙÂÚÓÂÙ ÛÙÔ Û¯ÔÏÂ›Ô Î·È ÁÈ· ÁÔÓ›˜/·ÁÁÂÏ̷ٛ˜ Ì ڷÎÙÈ-Τ˜ Ô‰ËÁ›Â˜ Î·È ÂÓËÌÂÚˆÙÈÎfi ˘ÏÈÎfi. ÕÏÏ· ÚÔÁÚ¿ÌÌ·Ù· ÙÔ˘ Childnet International Â›Ó·È ÙÔ Parent’ssupport, Blog safety, Know it All, Young people music & the Internet, Childnet Academy. √ ۯ‰ȷÛÌfi˜Ù˘ ÈÛÙÔÛÂÏ›‰·˜ Â›Ó·È ·ÚÎÂÙ¿ ÌÔÓÙ¤ÚÓÔ˜ Ì ÏÔ‡ÛÈÔ ÂÓËÌÂÚˆÙÈÎfi ˘ÏÈÎfi ÛÙËÓ ÚÒÙË ÛÂÏ›‰· Î·È ÌÈ·“¤Í˘ÓË” ÂÓ·ÏÏ·Á‹ ʈÙÔÁÚ·ÊÈÒÓ Ì¤Û· ·fi ÙÔ ÎÂÓÙÚÈÎfi flash.
482 CLINICAL QUIZ
¶·È‰È·ÙÚÈ΋ 2006;69:479,482
∫ÏÈÓÈÎfi QUIZ
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∂ÁοÚÛÈ· Ì˘ÂÏ›Ùȉ·
∏ ÂÁοÚÛÈ· Ì˘ÂÏ›Ùȉ· ÂÌÊ·Ó›˙ÂÙ·È Î˘Ú›ˆ˜Û ·È‰È¿ ÚoÂÊË‚È΋˜ ËÏÈΛ·˜, Î·È Û·ÓÈfiÙ·-Ù· Û Ӌȷ. ™ÙÔ 70% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ËÓfiÛo˜ ÂΉËÏÒÓÂÙ·È ÌÂÙ¿ ·fi Ïo›ÌˆÍË, 7-14̤Ú˜ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛo˘. OÈÏoÈÌoÁfiÓoÈ ·Ú¿ÁoÓÙ˜ o˘ ¤¯o˘Ó ÂÓo¯ooÈË-ı› ̤¯ÚÈ Û‹ÌÂÚ· Â›Ó·È oÈ Èo› Ù˘ ÈÏ·Ú¿˜, Ù˘·ÓÂÌ¢ÏoÁÈ¿˜, Ù˘ ·ÚˆÙ›Ùȉ·˜, Ù˘ Áڛ˘,EBV, CMV, HSV1,2, HIV, Coxsackie, Ë·Ù›Ùȉ·˜∞ Î·È µ, ηıÒ˜ Î·È Ù· ÌÈÎÚfi‚È· ·ÈÌoÏ˘ÙÈÎfi˜ÛÙÚÂÙfiÎoÎÎo˜ · Î·È ‚, Campylobacter,Salmonella, Chlamydia, Mycoplasma, Legionella,Leptospirosis, Borrelia burgdorferi Î·È Rickettsia.™ÙËÓ ÂÚÈÁÚ·Ê›۷ ÂÚ›ÙˆÛË ÚÔËÁ‹ıËÎÂÏԛ̈ÍË ·fi Ì˘ÎfiÏ·ÛÌ·. ™Â fi,ÙÈ ·ÊÔÚ¿ ÛÙËÓ·ıoÁ¤ÓÂÈ· Ù˘ ÓfiÛÔ˘, ÈÛÙ‡ÂÙ·È fiÙÈ ÔÊ›ÏÂÙ·ÈÛ ·˘Ùo¿ÓoÛË ‰ÈÂÚÁ·Û›· Ë ÔÔ›· ˘ÚÔ‰ÔÙ›ٷȷfi ÏoÈÌoÁfiÓo ÌÈÎÚooÚÁ·ÓÈÛÌfi ̤ۈ Ùo˘Ê·ÈÓo̤Óo˘ Ù˘ “ÌoÚȷ΋˜ ·oÌ›ÌËÛ˘”(molecular mimicry). ∫·Ù¿ Ùo Ê·ÈÓfiÌÂÓo ·˘Ùfi,ÚˆÙÂ˚ÓÈο ÂÙ›‰È· Ùo˘ ·ıoÁfiÓo˘ ÌÈ-ÎÚooÚÁ·ÓÈÛÌo‡, Ù· ÔÔ›· ¤¯Ô˘Ó ·ÚfiÌÔÈ· ‰ÔÌ‹Ì ٷ ÂÙ›‰È· Ù˘ ‚·ÛÈ΋˜ ÚˆÙ½Ó˘ Ù˘ Ì˘Â-Ï›Ó˘, Ù˘ ÚˆÙÂoÏÈȉÈ΋˜ ÚˆÙ½Ó˘ Î·È ÙË˜Ì˘ÂÏÈÓooÏÈÁo‰ÂÓ‰ÚÈÙÈ΋˜ ÁÏ˘ÎoÚˆÙ½Ó˘ Ùo˘·ÛıÂÓÔ‡˜, ÂÓÂÚÁÔÔÈÔ‡Ó Ù· ∆-ÏÂÌÊo·ÙÙ·Ú·ÛÙo ∫¡™ ÂÓ¿ÓÙÈ· ÛÙȘ Û˘ÁÎÂÎÚÈ̤Ó˜ ÚˆÙ½Ó˜ÙÔ˘ ·ÛıÂÓÔ‡˜. ¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ú¤ÂÈ Ó·Á›ÓÂÙ·È ·fi ÙÔ ÚÒÙÔ ÂÂÈÛfi‰ÈÔ Ù˘ ÛÎÏ‹Ú˘Ó-Û˘ ηٿ Ͽη˜, ÙË ÓfiÛÔ Guillain Barré, ÙË Û˘-Ì›ÂÛË ·fi fiÁÎÔ Î·È ¿ÏÏ· Û˘ÛÙËÌ·ÙÈο ÓÔÛ‹-Ì·Ù· °È· ÙË ıÂڷ›· Ù˘ ÓfiÛÔ˘ ¯ÚËÛÈÌooÈo‡-ÓÙ·È Ù· ÎoÚÙÈÎoÛÙÂÚoÂȉ‹ Û ˘„ËϤ˜ ‰fiÛÂȘ ¯Ô-ÚËÁÔ‡ÌÂÓ· ÂÓ‰oÊÏ‚›ˆ˜, ‰ÈfiÙÈ ÈÛÙ‡ÂÙ·È fiÙÈÌÂÈÒÓo˘Ó ÙË ‰È¿ÚÎÂÈ· ÙˆÓ Ó¢ÚoÏoÁÈÎÒÓ Û˘-Ìو̿وÓ. ™˘Ó‹ıˆ˜ ¯oÚËÁÂ›Ù·È ÌÂı˘ÏÚ‰-ÓÈ˙oÏfiÓË Û ‰fiÛË 20-30 mg/kgµ™/ËÌÂÚËÛ›ˆ˜ÁÈ· 3-5 Ë̤Ú˜, Ì ‹ ¯ˆÚ›˜ ÙË Û˘Ó¤¯ÈÛË Ù˘¯oÚ‹ÁËÛ˘ Ú‰ÓÈ˙oÏfiÓ˘ ·fi Ùo ÛÙfiÌ·, ÛÂÛÙ·‰È·Î‹ Ì›ˆÛË Ù˘ ‰fiÛ˘ Û ‰È¿ÛÙËÌ· 4-6‚‰oÌ¿‰ˆÓ, ·Ó¿ÏoÁ· Ì ÙËÓ oÚ›· ˘o¯ÒÚË-
Û˘ ÙˆÓ ÎÏÈÓÈÎÒÓ Û˘Ìو̿وÓ. ∏ ·Ú·¿Óˆ·ÁˆÁ‹ - ÙÚÂȘ ÒÛÂȘ ÌÂı˘ÏÚ‰ÓÈ˙oÏfiÓ˘ -ÂÊ·ÚÌfiÛıËΠÛÙËÓ Û˘ÁÎÂÎÚÈ̤ÓË ÂÚ›ÙˆÛËÌÂ Û˘Ó¤¯ÈÛË Ù˘ ¯ÔÚ‹ÁËÛ˘ Ú‰ÓÈ˙ÔÏfiÓ˘ ÛÂÛÙ·‰È·Î¿ ÌÂÈÔ‡ÌÂÓ˜ ‰fiÛÂȘ ·fi ÙÔ ÛÙfiÌ·. ∏ÎÏÈÓÈ΋ ·ÓÙ·fiÎÚÈÛË Ù˘ ·ÛıÂÓÔ‡˜ ÛÙË ıÂÚ·-›· ‹Ù·Ó ¿ÌÂÛË, Ì ϋÚË ·ÔηٿÛÙ·ÛË ‰‡ÔÌ‹Ó˜ ·fi ÙËÓ ÂÈÛ·ÁˆÁ‹. ∏ ÚfiÁÓˆÛË Ù˘ÂÁοÚÛÈ·˜ Ì˘ÂÏ›Ùȉ·˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ ¤¯ÂÈ ˆ˜ÂÍ‹˜: ÔÛÔÛÙfi 30% ÙˆÓ ·ÛıÂÓÒÓ ·Ôηı›ÛÙ·-ÓÙ·È Ï‹Úˆ˜, 30% ·ÚÔ˘ÛÈ¿˙Ô˘Ó ‚ÂÏÙ›ˆÛË Î·È30% Û˘Ó¯›˙Ô˘Ó Ó· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÛÔ‚·Ú¿ Ó¢-ÚÔÏÔÁÈο ÂÏÏ›ÌÌ·Ù·. ™Ù· ·È‰È¿ Ë ÚfiÁÓˆÛËÊ·›ÓÂÙ·È Ó· Â›Ó·È Î·Ï‡ÙÂÚË, Ì ÔÛÔÛÙfi 60% Ó··Ôηı›ÛÙ·Ù·È Ï‹Úˆ˜ Û ‰È¿ÛÙËÌ· ÌËÓÒÓ ·fiÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘. ∂È‚·Ú˘ÓÙÈÎÔ› ·Ú¿ÁÔ-ÓÙ˜ Â›Ó·È Ë ÔÍ›· ÂÁηٿÛÙ·ÛË Ï‹ÚÔ˘˜ ·Ú¿-Ï˘Û˘, Ë ·Ú¿Ù·ÛË ÙˆÓ ÛÔ‚·ÚÒÓ Ó¢ÚÔÏÔÁÈÎÒÓ¢ÚËÌ¿ÙˆÓ ¤Ú·Ó Ù˘ ‚‰ÔÌ¿‰·˜ Î·È Ë Î·ı˘-ÛÙ¤ÚËÛË ¤Ó·Ú͢ ‚¿‰ÈÛ˘ ¤Ú·Ó ÙÔ˘ ÌËÓfi˜.
µÈ‚ÏÈoÁÚ·Ê›·
1. Dunne K, Hopkins IJ, Shield LK. Acute transversemyelopathy in childhood. Dev Med Child Neurol1986;28:198-204.
2. Pittock SJ, Lucchinetti CF. Inflammatory transversemyelitis: evolving concepts. Curr Opin Neurol 2006;19:362-368.
3. Andronikou S, Albuquerque-Jonathan G, WilmshurstJ, Hewlett R. MRI findings in acute idiopathic trans-verse myelopathy in children. Pediatr Radiol 2003;33:624-629.
4. Smith R, Eviatar L. Neurologic manifestations of My-coplasma pneumoniae infections: diverse spectrum ofdiseases. A report of six cases and review of the litera-ture. Clin Pediatr (Phila) 2000;39:195-201.
5. Defresne P, Hollenberg H, Husson B, Tabarki B, Lan-drieu P, Huault G, et al. Acute transverse myelitis inchildren: clinical course and prognostic factors. J ChildNeurol 2003;18:401-406.
6. Miyazawa R, Ikeuchi Y, Tomomasa T, Ushiku H,Ogawa T, Morikawa A. Determinants of prognosis ofacute transverse myelitis in children. Pediatr Int 2003;45:512-516.
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¶·È‰È·ÙÚÈ΋ 2006;69:483
∂˘¯·ÚÈÛٛ˜ ÚÔ˜ ÙÔ˘˜ ∫ÚÈÙ¤˜ Ù˘ «¶·È‰È·ÙÚÈ΋˜»
∏ ™˘ÓÙ·ÎÙÈ΋ ∂ÈÙÚÔ‹ ¢¯·ÚÈÛÙ› ıÂÚÌ¿ ÙÔ˘˜ ÎÚÈÙ¤˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ «¶·È‰È·ÙÚÈ΋» Ô˘ÚÔÛ¤ÊÂÚ·Ó ÙËÓ Â›Ú· Î·È ÙÔÓ ¯ÚfiÓÔ ÙÔ˘˜ ÁÈ· Ó· ÂÍ·ÛÊ·Ï›Ûo˘Ó ÙÂÎÌËÚȈ̤Ó˜ Î·È ·ÓÙÈÎÂÈÌÂÓÈ-Τ˜ ·ÍÈÔÏÔÁ‹ÛÂȘ ÁÈ· ÙȘ ÂÈÛÙËÌÔÓÈΤ˜ ÂÚÁ·Û›Â˜ Ô˘ ‰ËÌÔÛȇıËÎ·Ó Î·Ù¿ ÙÔ ¤ÙÔ˜ 2006.
√ ¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘∫ˆÓÛÙ·ÓÙ›ÓÔ˜ ™ÙÂÊ·Ó›‰Ë˜
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ππ·ÙÚÈ΋ ‚·ÛÈṲ̂ÓË ÛÙȘ ÂӉ›ÍÂȘ 247π‰È¿˙ÔÓ ÚÔÛˆÂ›Ô 52πÏ·Ú¿ 218πfi˜ Epstein-Barr 302
∫∫·ı˘ÛÙ¤ÚËÛË ÛˆÌ·ÙÈ΋˜ ·Ó¿Ù˘Í˘ 385∫·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· 18∫·Ú‰ÈÔ¿ıÂȘ 468∫·Ù¿ÁÌ·Ù· 452∫·Ù·Ó¿ÏˆÛË ÂÓ¤ÚÁÂÈ·˜ ËÚÂÌ›·˜ 440∫·Ù¢ı˘ÓÙ‹ÚȘ Ô‰ËÁ›Â˜ 193∫·˘Î¿ÛÈ· Ê˘Ï‹ 143∫ÂÚ·Ûfi¯ÚÔ˘˜ ÎËÏ›‰· 213∫ÏÈÓÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· 447∫ÔÎ·Ù˘ 83∫Ú·ÓÈÔÛ˘ÓÔÛÙ¤ˆÛË 227, 233∫ÚÈÙÈ΋ ·ÍÈÔÏfiÁËÛË 247∫‡ËÛË 104, 199∫˘ÛÙÈ΋ ›ÓˆÛË 440∫˘ÙÙ·ÚÔΛÓ˜ 395
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√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· 361
√Í›· ·ÁÎÚ·ٛÙȉ· 310
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√Êı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË 65
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302, 313, 317
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¶·È‰Èο ·Ù˘¯‹Ì·Ù· 278
¶·È‰Èο ·Ù˘¯‹Ì·Ù· ÛÙÔ Û›ÙÈ 268
¶·È‰È΋ ËÏÈΛ· 118, 150
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¶·Ó‰ËÌ›· 1
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¶Ï·ÁÈÔÎÂÊ·Ï›· 233
¶Ó¢ÌÔÓÈ΋ ÓfiÛÔ˜ 213
¶ÔÏÈÔÌ˘ÂÏ›ÙȘ 90¶ÔÓÙÈÎfi˜ 255¶ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË 138¶ÚÔηÏÛÈÙÔÓ›ÓË 395¶ÚfiÏË„Ë 268, 278
ƒƒ·¯È·ÏÁ›· 458ƒfi‰ÈÓË ·ÓÙ·‡ÁÂÈ· 65
™™·Î¯·Ú҉˘ ‰È·‚‹Ù˘ Ù‡Ô˘ 1 432™ÂÏ‹ÓÈÔ 199™ËÙÈ΋ ·ÚıÚ›Ùȉ· 390™‹„Ë 376™Ù‹ÚÈÍË 369™ÙÚÂÙfiÎÔÎÎÔ˜ 97™˘ÁÁÂÓ›˜ ‰È·Ì·Úٛ˜ 409™˘ÁÁÂÓ›˜ ηډÈÔ¿ıÂȘ 110™˘ÁÁÂÓ‹˜ ˘ÂÚÏ·Û›· 286ÂÈÓÂÊÚȉ›ˆÓ™˘ÌÌfiÚʈÛË 193™‡Ó‰ÚÔÌÔ Meckel 134™‡Ó‰ÚÔÌÔ Noonan 447™‡Ó‰ÚÔÌÔ Rubinstein-Taybi 385™‡Ó‰ÚÔÌÔ Saethre-Chotzen 227™‡Ó‰ÚÔÌÔ SLO 134™˘Ó‰˘·Ṳ̂ÓÔ ÂÌ‚fiÏÈÔ ÙÂÙ¿ÓÔ˘- 83‰ÈÊıÂÚ›Ùȉ·˜-·Î˘ÙÙ·ÚÈÎÔ‡ ÎÔηÙË Ù‡Ô˘ ÂÓËÏ›ÎÔ˘™˘Ó‰˘·ÛÌfi˜(Ô› ) 417
™˘ÚÈÁÌfi˜ 425™˘ÛÙËÌ·ÙÈ΋ ·Ó·ÛÎfiËÛË 247™˘¯ÓfiÙËÙ· ÂÙÂÚÔ˙˘ÁˆÙÒÓ 286
∆∆ÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿ 205∆Ú·¯ËÏÈ΋ ¯ÒÚ· 313∆ÚfiÊÈÌ· 127∆ÚÔÊÈÌÔÁÂÓ›˜ ÓfiÛÔÈ 37∆˘ÚÔÛÈÓ·ÈÌ›· Ù‡Ô˘ π 45
ÀÀÂÚ¿ÚÈıÌÔ ¯ÚˆÌÔÛˆÌÈÎfi ıÚ·‡ÛÌ· 294ÀÂÚÁÏ˘Î·ÈÌ›· 409ÀÔÍ›· ÛÎÏËÚ˘ÓÙÈ΋ ·ÓÂÁÎÂÊ·Ï›Ùȉ· 218ÀÔÏ·Û›· ÛÎÒÏËη 134ÀÛÙ¤ÚËÛË ·‡ÍËÛ˘ 350
ººÏÂÁÌÔÓ‹ ·ÂÚ·ÁˆÁÒÓ 169ºÚÔÓÙ›‰· ÙÂÏÈÎÔ‡ ÛÙ·‰›Ô˘ 118
Ã÷ÏÎfi˜ 199ÃÚˆÌfiÓ˜ 425ÃÚˆÌfiۈ̷ 15 294ÃÚˆÌÔÛÒÌ·Ù· 327
øøÙÔÙÔÍÈÎfiÙËÙ· 336
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ª·Ú›· ¶··Ê›ÏË
°È· ÏËÚÔÊÔڛ˜:www.euran.com
ªaria Papafili
For information:www.euran.com
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10-11 ¡ÔÂÌ‚Ú›Ô˘ 2006 5Ô ™ÂÌÈÓ¿ÚÈÔ Ì ı¤Ì·: æ˘¯ÔÎÔÈÓˆÓÈ΋ ∞ı‹Ó·
™Ù‹ÚÈÍË ÙÔ˘ ¶·È‰ÈÔ‡ Ì ∫·ÚΛÓÔ Î·È
Ù˘ √ÈÎÔÁ¤ÓÂÈ¿˜ ÙÔ˘
•ÂÓÔ‰Ô¯Â›Ô Holiday Inn¢ÈÔÚÁ¿ÓˆÛË: √ÁÎÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡”∆ËÏ.: 210 7254360-2Fax: 210 7254363∂-mail: [email protected]: www.epsiloncongress.gr
15-17 ¡ÔÂÌ‚Ú›Ô˘ 2006 Advanced Minimally Invasive Operating Norderstedt,
Techniques in Paediatric Surgery Germany
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15 - 18 ¡ÔÂÌ‚Ú›Ô˘ 2006 Infant, Child and Adolescent Medicine St. Petersburg,
Contact: Karla Krause FL, United States
Tel: 800-274-2237, ext. 6522Fax: 913-906-6092E-mail: [email protected]
25-26 ¡ÔÂÌ‚Ú›Ô˘ 2006 6Ë ¢ÈËÌÂÚ›‰· ∫ÔÈÓˆÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ §¿ÚÈÛ·
∞ÌÊÈı¤·ÙÚÔ ¶·ÓÂÈÛÙËÌÈ·ÎÔ‡ ¡ÔÛÔÎÔÌ›Ԣ¢ÈÔÚÁ¿ÓˆÛË: ∂ÏÏËÓÈ‹ ∂Ù·ÈÚ›· ∫ÔÈÓˆÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ Î·È ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜ - ¶·Ú¿ÚÙËÌ· £ÂÛÛ·Ï›·˜ Î·È ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ £ÂÛÛ·Ï›·˜∆ËÏ.: 2410 682705, 6937 676725
29 ¡ÔÂÌ‚Ú›Ô˘ - 3rd International POSNA/AAOS Orlando,
3 ¢ÂÎÂÌ‚Ú›Ô˘ 2006 Pediatric Orthopaedic Symposium United States
Contact: Susan McSorleyTel.: 847-823-7186Fax: 847-823-8125E-mail: [email protected]
20 - 21 π·ÓÔ˘·Ú›Ô˘ 2007 17o ™˘Ó¤‰ÚÈÔ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ¶¿ÙÚ·
™˘Ó‰ÚÈ·Îfi & ¶ÔÏÈÙÈÛÙÈÎfi ∫¤ÓÙÚÔ ¶·ÓÂÈÛÙËÌ›Ô˘ ¶·ÙÚÒÓ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·Ó/Ì›Ô˘ ¶·ÙÚÒÓ, ∫·ı. ™Ù. ª·ÓÙ·Áfi˜™˘Ó‰ÚÈ·Îfi & ¶ÔÏÈÙÈÛÙÈÎfi ∫¤ÓÙÚÔ ¶·ÓÂÈÛÙËÌ›Ô˘ ¶·ÙÚÒÓ∆ËÏ.: 2610-993999Fax: 2610-994999∂-mail: [email protected]: www.confer.upatras.gr
25-27 π·ÓÔ˘·Ú›Ô˘ 2007 25th Annual Conference on Sleep Disorders Rancho Mirage,
in Infancy & Childhood CA, United States
Contact: Alice ClarkTel.: 760-773-4500Fax: 760-773-4551E-mail: [email protected]
xxvi
7-10 ºÂ‚ÚÔ˘·Ú›Ô˘ 2007 16th Annual Pediatric Infectious Diseases Calgary, AB,
Contact: Janis Pearson / Joan Sweeney Canada
Tel.: 220-7032 / 220-8458E-mail: [email protected] / [email protected]
17-24 ºÂ‚ÚÔ˘·Ú›Ô˘ 2007 8th JOPEOI (European / Indian Ocean Meeting Dubai, United
about Mother & Child Health Arab Emirates
Contact: Kamel BargaouiTel.:33-607-686-118Fax: 33-143-839-985E-mail: [email protected]
8-11 ª·ÚÙ›Ô˘ 2007 Pediatric Anesthesiology 2007 Phoenix, AZ,
Contact: Society for Pediatric Anesthesia United States
Tel.:804-282-9780Fax: 804-282-0090E-mail: [email protected]
16-18 ∞ÚÈÏ›Ô˘ 2007 The 7th Annual International Riyadh,
Neonatology Symposium Saudi Arabia
Contact: Saleh Al-Alaiyan, MDTel.:00-96-614-427-762Fax: 00-96-614-427-784E-mail: [email protected]
2-5 ª·˝Ô˘ 2007 15th TRANSMED: 15th European/Mediterranean Cairo, Egypt
Congress about Mother & Child Health
Contact: Kamel BARGAOUITel.:33-607-686-118Fax: 33-143-839-985E-mail: [email protected]
2-5 ª·˝Ô˘ 2007 33Ô ∂Ù‹ÛÈÔ ¶·ÓÂÏÏ‹ÓÈÔ π·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ ∞ı‹Ó·
•ÂÓÔ‰Ô¯Â›Ô Hilton¢ÈÔÁ¿ÓˆÛË: π·ÙÚÈ΋ ∂Ù·ÈÚ›· ∞ıËÓÒÓª·È¿Ó‰ÚÔ˘ 23, 11528, ∞ı‹Ó·∆ËÏ.: 210 7211845Fax: 210 7215082E-mail: [email protected]
7 - 10 πÔ˘Ó›Ô˘ 2007 5Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ¶·È‰Ô„˘¯È·ÙÚÈ΋˜ ∞ı‹Ó·
∂Ù·ÈÚ›·˜ ∂ÏÏ¿‰Ô˜
ª¤Á·ÚÔ ªÔ˘ÛÈ΋˜ ∞ıËÓÒÓ ¢ÈÔÚÁ¿ÓˆÛË: ¶·È‰Ô„˘¯È·ÙÚÈ΋ ∂Ù·ÈÚ›· ∂ÏÏ¿‰Ô˜¶ÏËÚÔÊÔڛ˜: AC&C International∆ËÏ: 210 6889100 Fax: 210 6844777E-mail: [email protected]
14-17 πÔ˘Ó›Ô˘ 2007 The 21st Congress of International Association Hong Kong,
of Paediatric Dentistry Hong Kong
Contact: Agnes Chung / Daniel ChokTel.:85-228-718-896 / 28-718-815Fax: 85-228-718-898E-mail: [email protected]