ISSN 0377-2551
¶AI¢IATPIKH
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TOMO™ 64
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TEYXO™ 5
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ETO™ 2001
™Â٤̂ÚÈÔ˜ - √ÎÙÒ‚ÚÈÔ˜ 2001 . ∆fiÌÔ˜ 64 . ∆‡¯Ô˜ 5
ANA™KO¶H™H ™¯ÈÛٛ˜ ÙÔ˘ ÛÙÔÌ·ÙÔÁÓ·ıÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜
REVIEW ARTICLE 441
ª. ¡. ™˘ÚÔÔ‡ÏÔ˘
M. N. Spyropoulos
∂¶π∫∞πƒ∞ £∂ª∞∆∞ ∏ ˘¤ÚÙ·ÛË ÛÙËÓ ·È‰È΋ ËÏÈΛ·
CURRENT ISSUES 448
∞. ∑ÂÚ‚Ô˘‰¿ÎË, ∫. ∆ÛÔ˘Ì¿Î·˜
™‡Ó‰ÚÔÌÔ ˘ÂÚ¢·ÈÛıËÛ›·˜ ÛÙ· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η
460
ORIGINAL ARTICLES 465
°. ∆ÛfiÏ·˜, ¢. ÃÔϤ‚·˜, ™. ∆ÛÔ˘Î·Ï¿, Ã. ™Ù·˘ÚÈÓ¿‰Ë˜, ¶. ∫Ô˘ÎÔ˘ÙÛ¿Î˘, ¶. ™˘Ú›‰Ë˜, ¢. °Ô˘ÚÁÈÒÙ˘, ∫. ™ÈÓ·ÓÈÒÙ˘ 472
481
Immunologic parameters in the evaluation of the eventual outcome of childhood pneumonia M. Eboriadou, M. Hatzistilianou, K. Haidopoulou, C. Aggouridaki, V. Tsavdaridou, G. Varlamis, S. Karamperis
488
∫.∂. ™Ô‡ÏË, °.∞. ∫·Ú›Î·˜, π. ∆˙·ÌÔ˘Ú¿ÓË, ™. ƒÂÁÎÔ‡Ù·˜, ™. ∆۷ΛÚ˘
∂ȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË ÙˆÓ Î·ÎÒÛÂˆÓ ÛÙËÓ ÂÊ˂›·
Management of children with acute renal failure in a pediatric intensive care unit N. Zavras, A. Kapogiannis, P. Kalambalikis, G. Briasoulis, E. Georgaki, A. Hatzis
ª. ∂ÌÔÚÈ¿‰Ô˘, ª. ÷Ù˙ËÛÙ˘ÏÈ·ÓÔ‡, ∫. ÷˚‰ÔÔ‡ÏÔ˘, Ã. ∞ÁÁÔ˘Úȉ¿ÎË, µ. ∆Û·‚‰·Ú›‰Ô˘, °. µ·ÚÏ¿Ì˘, ™. ∫·Ú·Ì¤Ú˘
∏ ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ÂÈÏËÙÈÎÒÓ ·È‰ÈÒÓ Û ÌÔÓÔıÂڷ›· Ì ‚·ÏÚÔ˚Îfi Ô͇
Seasonal variation of urinary calcium excretion G. Tsolas, D. Cholevas, S. Tsoukala, C. Stavrinadis, P. Koukoutsakis, P. Spyridis, D. Gouriotis, C. Sinaniotis
¡. ∑¿‚Ú·˜, ∞. ∫·fiÁÈ·ÓÓ˘, ¶. ∫·Ï·Ì·Ï›Î˘, °. ªÚÈ·ÛÔ‡Ï˘, ∂. °ÂˆÚÁ¿ÎË, ∞. ÷Ù˙‹˜
∞ÓÔÛÔÏÔÁÈÎÔ› ·Ú¿ÌÂÙÚÔÈ ÛÙËÓ ÂÎÙ›ÌËÛË Ù˘ ÂͤÏÈ͢ Ù˘ ·È‰È΋˜ Ó¢ÌÔÓ›·˜
Anticonvulsant hypersensitivity syndrome I. Charoniti, D. Lambraki, V. Angelakou
∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™
∏ ÂÌÂÈÚ›· ·fi ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ÓÂÊÚÈ΋˜ ·Ó¿ÚÎÂÈ·˜ Û ·È‰È¿ ÛÙË ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £Âڷ›·˜
Hypertension in childhood A. Zervoudaki, K. Tsoumakas
π. X·ÚˆÓ›ÙË, ¢. §·ÌÚ¿ÎË, µ. ∞ÁÁÂÏ¿ÎÔ˘
∂Ô¯È΋ ÌÂÙ·‚ÔÏ‹ Ù˘ ·¤ÎÎÚÈÛ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú·
Clefts of the lip and palate
Serum biotinidase activity in epileptic children on valproic acid monotherapy K.H. Schulpis, G.A. Karikas, J. Tjamouranis, S. Regoutas, S. Tsakiris
493
Epidemiological study of injuries in adolescence
Õ. ¶·ÓÙ·˙‹, ¡. ÷Ù˙ËΈÓÛÙ·ÓÙ›ÓÔ˘, £. ∆Ú·ÁÈ·ÓÓ›‰Ë˜, °. ∫·Ú·‚¿Ó·, £. ∞ÏÌ·Ó›‰Ë˜, ™. ∞ÁÁÂÏfiÔ˘ÏÔ˜
A. Pantazi, N. Hatzikonstantinou, T. Tragianidis, G. Karavana, T. Almanidis, S. Agelopoulos
∏ Û˘Ó¤¯ÂÈ· ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÛÙËÓ ÚÒÙË ÂÛˆÙÂÚÈ΋ ÛÂÏ›‰·
Continuation of table of contents inside title page
September - October 2001 . Volume 64 . No 5
™Â٤̂ÚÈÔ˜ - √ÎÙÒ‚ÚÈÔ˜ 2001 . ∆fiÌÔ˜ 64 . ∆‡¯Ô˜ 5
™˘Ó¤¯ÂÈ· ÂÚȯÔ̤ӈÓ
Continuation of table of contents
CASE REPORTS
∂¡¢π∞º∂ƒOÀ™∂™ ¶∂ƒπ¶∆ø™∂π™ ™‡Ó‰ÚÔÌÔ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜ Û ·È‰È¿ Ì ¤Áη˘Ì·. ¶ÂÚÈÁÚ·Ê‹ ‰‡Ô ÂÚÈÙÒÛˆÓ
499
M. Narlioglou, P. Kalabalikis, G. Briassoulis, A. Hatzis
ª. ¡·ÚÏ›ÔÁÏÔ˘, ¶. ∫·Ï·Ì·Ï›Î˘, °. ªÚÈ·ÛÔ‡Ï˘, ∞. ÷Ù˙‹˜
™˘ÁÁÂÓ‹˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË Ì ·˘ÍË̤ӷ ›‰· ÂÚ˘ıÚÔÔÈËÙ›Ó˘ ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘
504
508
514
™ÔÁÁÒ‰ÂȘ ÂÁÎÂÊ·ÏÔ¿ıÂȘ. ¡ÂfiÙÂÚ˜ ·fi„ÂȘ
PROGRESS IN MEDICAL SCIENCE 518
527
Factors affecting neurogenesis during the neonatal period. A review K.H. Schulpis, A. Doulgeraki, S. Tsakiris
∫.∂. ™Ô‡ÏË, Õ. ¢Ô˘ÏÁÂÚ¿ÎË, ™. ∆۷ΛÚ˘
™‡Á¯ÚÔÓ˜ ·ÈÙÈÔÏÔÁÈΤ˜ Û˘Û¯ÂÙ›ÛÂȘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜
Spongiform encephalopathies. Recent advances K. Karavanaki - Karanasiou
∫. ∫·Ú·‚·Ó¿ÎË - ∫·Ú·Ó¿ÛÈÔ˘
¶·Ú¿ÁÔÓÙ˜ Ó¢ÚÔÁ¤ÓÂÛ˘ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô. ¡ÂfiÙÂÚ˜ ·fi„ÂȘ
Crossed renal ectopia with fusion and hydronephrosis in a girl with congenital adrenal hyperplasia D. Karakaidos, P. Zosi, M. Karafezi, A. Moutafi, G. Kaniaris, C. Karis
¢. ∫·Ú·Î·˚‰fi˜, ¶. ∑ÒÛË, ª. ∫·Ú·Ê¤˙Ë, ∞. ªÔ˘Ù¿ÊË, °. ∫·ÓÈ¿Ú˘, Ã. ∫·Ú‹˜
∂•∂§π•∂π™ ™∆∏¡ π∞∆ƒπ∫∏ ∂¶π™∆∏ª∏
Congenital muscular dystrophy with laminin-a2 deficiency C. Panteliadis, N. Flaris, E. Karatza-Xifilidou, J. Xinias
Ã. ¶. ¶·ÓÙÂÏÈ¿‰Ë˜, ¡. ºÏ¿Ú˘, ∂. ∫·Ú·Ù˙¿-•ÈÊÈÏ›‰Ô˘, π. •˘ÓÈ¿˜
¢È·ÛÙ·˘ÚÔ‡ÌÂÓË ÓÂÊÚÈ΋ ÂÎÙÔ›· Ì ˘‰ÚÔÓ¤ÊÚˆÛË Û ÎÔÚ›ÙÛÈ ÌÂ Û˘ÁÁÂÓ‹ ˘ÂÚÏ·Û›· ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ
Congenital erythrocytosis with increased erythropoietin levels. Case report M. Athanassiou-Metaxa, M. Oikonomou, C. Tsantali, A. Kousi, N. Gombakis
ª. ∞ı·Ó·Û›Ô˘-ªÂÙ·Í¿, ª. OÈÎÔÓfiÌÔ˘, Ã. ∆Û¿ÓÙ·ÏË, ∞. ∫Ô‡ÛË, ¡. °ÔÌ¿Î˘
™˘ÁÁÂÓ‹˜ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· ·fi ¤ÏÏÂÈ„Ë Ï·ÌÈÓ›Ó˘ ·2
∆oxic shock syndrome in children with burns. Report of 2 cases
535
Recent etiological correlations of childhood leukemia
∞. ¶Ô˘ÚÙÛ›‰Ë˜
A. G. Pourtsidis
™˘Ó¯›˙ÔÓÙ·È
Continued
September - October 2001 . Volume 64 . No 5
™Â٤̂ÚÈÔ˜ - √ÎÙÒ‚ÚÈÔ˜ 2001 . ∆fiÌÔ˜ 64 . ∆‡¯Ô˜ 5
™˘Ó¤¯ÂÈ· ÂÚȯÔ̤ӈÓ
Continuation of table of contents
¶∂ƒπ§∏æ∂π™ ∞ƒ£ƒø¡ ∞¶O ∆∏ µπµ§πO°ƒ∞ºπ∞ ∞˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ·ÛıÌ·ÙÈÎÔ‡ Ù‡Ô˘ Û˘ÌÙˆÌ¿ÙˆÓ Û ÎÔÚ›ÙÛÈ· Ô˘ Á›ÓÔÓÙ·È ˘¤Ú‚·Ú· ‹ ·¯‡Û·Úη ηٿ ÙË Û¯ÔÏÈ΋ ÂÚ›Ô‰Ô
LITERATURE ABSTRACTS 459
Castro-Rodriguez JA, Holberg CJ, Morgan WJ, Wright AL, Martinez FD ∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: £. ∆ÛÈÏÈÁÈ¿ÓÓ˘
ÀÂÚÔ˘ÚÈÎÔ˙Ô˘Ú›· ÛÙ· ·È‰È¿. ∫ÏÈÓÈ΋ ÚÔ‚ÔÏ‹ Î·È Ê˘ÛÈ΋ ÈÛÙÔÚ›·
Castro-Rodriguez JA, Holberg CJ, Morgan WJ, Wright AL, Martinez FD Greek translation: T. Tsiligiannis 471
La ªanna A, Polito C, MarÙe A, Iovene A, Di Toro R ∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: ∫. ∆ÛÔ˘Ì¿Î·˜
À¿Ú¯ÂÈ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙˆÓ ·ÓÙÈ·ÛıÌ·ÙÈÎÒÓ Ê·ÚÌ¿ÎˆÓ Î·È Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ Ì ÙÔ˘˜ ı·Ó¿ÙÔ˘˜ ·fi ¿ÛıÌ·;
480
Are asthma medications and management related to deaths from asthma? Abramson MJ, Bailey MJ, Couper FJ, Driver JS, Drummer OH, Forbes AB et al Greek translation: T. Tsiligiannis
492
Krawiec ME, Westcott JY, Chu HW, Balzar S, Trudeau JB, Schwartz LB et al ∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: £. ∆ÛÈÏÈÁÈ¿ÓÓ˘
™È‰ËÚÔÂÓ›· Î·È ÁÓˆÛÙÈ΋ ·ÓÙ›ÏË„Ë Û ·È‰È¿ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ Î·È ÂÊ‹‚Ô˘˜ ÛÙȘ ∏¶∞
Hyperuricosuria in children. Clinical presentation and natural history La ªanna A, Polito C, MarÙe A, Iovene A, Di Toro R Greek translation: ∫. ∆soumakas
Abramson MJ, Bailey MJ, Couper FJ, Driver JS, Drummer OH, Forbes AB et al ∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: £. ∆ÛÈÏÈÁÈ¿ÓÓ˘
∏ ›ÌÔÓË Û˘ÚÚ›ÙÔ˘Û· ·Ó·ÓÔ‹ Û Ôχ ÌÈÎÚ¿ ·È‰È¿ Û¯ÂÙ›˙ÂÙ·È Ì ÊÏÂÁÌÔÓ‹ ÙˆÓ Î·ÙÒÙÂÚˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ
Increased incidence of asthma like symptoms in girls who become overweight or obese during the school years
Persistent wheezing in very young children is associated with lower respiratory inflammation Krawiec ME, Westcott JY, Chu HW, Balzar S, Trudeau JB, Schwartz LB et al Greek translation: T. Tsiligiannis
507
Halterman JS, Kaczorowski JM, Allgne CA, Auinger P, Szilagyl PG ∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: ∞. Ã. ª¿Î˘
Iron deficiency and cognitive achievement among school-aged children and adolescents in the United States Halterman JS, Kaczorowski JM, Allgne CA, Auinger P, Szilagyl PG Greek translation: A. C. Makis
¶ÚÔÛ¯‹ ™˘Ó¤‰ÚÈ·
xiii
Scheduled Medical Meetings
™˘ÓÙÔÌÔÁڷʛ˜
xvii
Abbreviations
September - October 2001 . Volume 64 . No 5
™Â٤̂ÚÈÔ˜ - √ÎÙÒ‚ÚÈÔ˜ 2001 . ∆fiÌÔ˜ 64
.
∆‡¯Ô˜ 5
¢ÈÌËÓÈ·›· ¤Î‰ÔÛË E›ÛËÌÔ ¶ÂÚÈÔ‰ÈÎfi Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜
Bimonthly Publication The Official Journal of the Hellenic Paediatric Society
EΉfiÙ˘ K. °ÚÈ‚¤·˜
Publisher K. Griveas
I‰ÈÔÎÙ‹Ù˘© EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›· Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 Aı‹Ó·È 115 28
Owner© Hellenic Paediatric Society Michalakopoulou 92 Athens 115 28
TËÏ:. (01) 7771 140 / 7771 663, Fax: 7758 354
Tel. (01) 7771 140 / 7771 663, Fax: 7758 354
EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ¶Úfi‰ÚÔ˜ : ∫. ª·Ï·Î¿ ¢È¢ı˘ÓÙ‹˜ : ∑. ¶··‰ÔÔ‡ÏÔ˘-∫Ô˘ÏÔ˘Ì‹ M¤ÏË : ∂. ∞ÁÁÂÏ¿ÎË-°ÂˆÚÁ¿ÎË : º. Aı·Ó·ÛÈ¿‰Ô˘-¶ÈÂÚÔÔ‡ÏÔ˘ : ª. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ : °. µ·ÚÏ¿Ì˘ : ¶. ∫·ÊÚ›ÙÛ· : ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ : £. ∆ÛÈÏÈÁÈ¿ÓÓ˘ : ∫. ∆ÛÔ˘Ì¿Î·˜ : ™. ºˆÙfiÔ˘ÏÔ˜ : A. ÷Ù˙‹˜
Scientific President Editor Members
ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ· ∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈÌ¤ÓˆÓ I. K·Ú·‚Ú¿ÓÔ˘
Manuscript Editing
∂È̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ∑. ¶··‰ÔÔ‡ÏÔ˘-∫Ô˘ÏÔ˘Ì‹
English Editing Z. Papadopoulou-Couloumbis
∞ÏÏËÏÔÁÚ·Ê›· ∂ÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ ∂Ù·ÈÚ›· ªÈ¯·Ï·ÎÔÔ‡ÏÔ˘ 92 ∞ı‹Ó·È 115 28 ∆ËÏ: (01) 7771 140, Fax: 7758 354
Correspondence Hellenic Paediatric Society Michalakopoulou 92 Athens 115 28 Tel: (01) 7771 140, Fax: 7758 354
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September-October 2001 . Volume 64 .No 5
πSSN 0377-2551 i
O‰ËÁ›Â˜ ÚÔ˜ ÙÔ˘˜ ™˘ÁÁÚ·Ê›˜ A. °ÂÓÈΤ˜ ¶ÏËÚÔÊÔڛ˜ ∆Ô ÂÚÈÔ‰ÈÎfi “¶∞π¢π∞∆ƒπ∫◊” Â›Ó·È Ë Â›ÛËÌË ÂÈÛÙËÌÔÓÈ΋ ¤Î‰ÔÛË Î·È È‰ÈÔÎÙËÛ›· Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜. Œ¯ÂÈ ˆ˜ ‚·ÛÈÎÔ‡˜ ÛÙfi¯Ô˘˜ ÙËÓ ·ÔÙ‡ˆÛË ÙÔ˘ ·È‰È·ÙÚÈÎÔ‡ ¤ÚÁÔ˘ ÛÙËÓ ∂ÏÏ¿‰· Î·È ÙË Û˘Ó¯‹ È·ÙÚÈ΋ ÂÓË̤ڈÛË Î·È ÂÈÌfiÚʈÛË ÙˆÓ EÏÏ‹ÓˆÓ ¶·È‰È¿ÙÚˆÓ. °È· ÙÔ ÛÎÔfi ·˘Ùfi ‰¤¯ÂÙ·È ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÔÈÎÈÏ›· ¿ÚıÚˆÓ Î·È Û˘ÁÎÂÎÚÈ̤ӷ: ¿ÚıÚ· Û‡ÓÙ·Í˘ (ÌÂÙ¿ ·fi ÚfiÛÎÏËÛË Ù˘ EÈÛÙËÌÔÓÈ΋˜ ™˘ÓÙ·ÎÙÈ΋˜ EÈÙÚÔ‹˜), ·Ó·ÛÎÔ‹ÛÂȘ, ›ηÈÚ· ı¤Ì·Ù·, ÂÍÂÏ›ÍÂȘ ÛÙËÓ π·ÙÚÈ΋ ∂ÈÛÙ‹ÌË, ÚˆÙfiÙ˘Â˜ ÂÚ¢ÓËÙÈΤ˜ ÌÂϤÙ˜ Ì ·È‰È·ÙÚÈÎfi ‹ È·ÙÚÔÎÔÈÓˆÓÈÎfi ÂÚȯfiÌÂÓÔ, ÁÂÓÈο ı¤Ì·Ù· Ô˘ ·ÊÔÚÔ‡Ó ÙÔÓ ÚÔÁÚ·ÌÌ·ÙÈÛÌfi Î·È ÙËÓ ÔÚÁ¿ÓˆÛË Ù˘ È·ÙÚÈ΋˜ Âη›‰Â˘Û˘ Î·È ˘Á›·˜, ‚Ú·‚Â˘Ì¤Ó˜ ÂÚÁ·Û›Â˜, ÂÈÏÂÁ̤Ó˜ Û˘˙ËÙ‹ÛÂȘ ÛÙÚÔÁÁ˘ÏÒÓ ÙÚ·Â˙ÒÓ, ÂӉȷʤÚÔ˘Û˜ ÎÏÈÓÈΤ˜ Î·È ·ÂÈÎÔÓÈÛÙÈΤ˜ ÂÚÈÙÒÛÂȘ, ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ, ‚È‚ÏÈÔÎÚÈۛ˜, ÂÈÛÙÔϤ˜ ÚÔ˜ ÙË Û‡ÓÙ·ÍË, ÂÚÈÏ‹„ÂȘ Ù˘ ÂÏÏËÓÈ΋˜ Î·È Í¤Ó˘ ‚È‚ÏÈÔÁÚ·Ê›·˜, ·Ó·ÎÔÈÓÒÛÂȘ ÚÔÛ¯ÒÓ Û˘ÌÔÛ›ˆÓ Î·È Û˘Ó‰ڛˆÓ Û ı¤Ì·Ù· Û¯ÂÙÈο Ì ÙËÓ ¶·È‰È·ÙÚÈ΋ Î·È ·Ó·ÊÔÚ¤˜ Ó¤ˆÓ ÂΉfiÛˆÓ, ÂÏÏËÓÈÎÒÓ Î·È Í¤ÓˆÓ, ·È‰È·ÙÚÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜. H EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ó· ‰ËÌÔÛȇÂÈ ¿ÚıÚ· Ì ȉȷ›ÙÂÚÔ ÂÈÛÙËÌÔÓÈÎfi ÂӉȷʤÚÔÓ ¯ˆÚ›˜ Ó· ÙËÚÂ›Ù·È Ë ÛÂÈÚ¿ ˘Ô‚ÔÏ‹˜ Î·È Ó· ·ÔÊ·Û›˙ÂÈ ÁÈ· ÙË ‰ËÌÔÛ›Â˘ÛË ÂÚÁ·ÛÈÒÓ Ô˘ ¤¯Ô˘Ó ‚Ú·‚¢ı› ÛÙ· Ï·›ÛÈ· ÙÔ˘ ÂÙ‹ÛÈÔ˘ ¶·È‰È·ÙÚÈÎÔ‡ ™˘Ó‰ڛԢ, ÙËÓ ÔÏÈ΋ ‹ ÌÂÚÈ΋ ‰ËÌÔÛ›Â˘ÛË ÛÙÚÔÁÁ˘ÏÒÓ ÙÚ·Â˙ÒÓ È‰È·›ÙÂÚÔ˘ ÂӉȷʤÚÔÓÙÔ˜, Î·È ÙËÓ ÔÏÈ΋ ‹ ÌÂÚÈ΋ ‰ËÌÔÛ›Â˘ÛË ÂÈÛÙÔÏÒÓ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ‰ËÌÔÛÈÂ˘Ì¤Ó· ÂÈÛÙËÌÔÓÈο ¿ÚıÚ· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. µ. ÀÔ‚ÔÏ‹ Î·È ¢ËÌÔÛ›Â˘ÛË ÙˆÓ ∂ÚÁ·ÛÈÒÓ OÈ ÂÚÁ·Û›Â˜ Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È ÚÔ˜ ÎÚ›ÛË ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÛÙÔ ÂÚÈÔ‰ÈÎfi “¶∞π¢π∞∆ƒπ∫◊” ·ÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó ÛÙË ‰È‡ı˘ÓÛË: EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›· Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 115 28 Aı‹Ó· ŸÏ˜ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ÂÈÛÙÔÏ‹, Ë ÔÔ›· ı· Û˘Ó˘ÔÁÚ¿ÊÂÙ·È ·fi fiÏÔ˘˜ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ ÛÙËÓ ÔÔ›· ı· ‰ËÏÒÓÂÙ·È fiÙÈ Ë ÂÚÁ·Û›· ‰ÂÓ ¤¯ÂÈ ÂÓ Ì¤ÚÂÈ ‹ ÂÍ ÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚ÏËı› ÁÈ· ÎÚ›ÛË Û ¿ÏÏÔ ÂÚÈÔ‰ÈÎfi Î·È fiÙÈ ÔÈ Û˘ÁÁÚ·Ê›˜ ÂÁÎÚ›ÓÔ˘Ó ÙË ‰ËÌÔÛ›Â˘Û‹ Ù˘ ÛÙÔ ÂÚÈÔ‰ÈÎfi “¶∞π¢π∞∆ƒπ∫◊”. ∂ÈϤÔÓ, ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹ÛÂȘ - ¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢. ŸÏ˜ ÔÈ ÎÏÈÓÈΤ˜ ¤Ú¢Ó˜ Ú¤ÂÈ Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ÁÚ·Ù‹ ‰‹ÏˆÛË ÙˆÓ Û˘ÁÁڷʤˆÓ fiÙÈ ¤ÁÈÓ·Ó ÌÂÙ¿ ·fi ÏËÚÔÊÔÚË̤ÓË Û˘Ó·›ÓÂÛË ÙˆÓ ÌÂÙ¯fiÓÙˆÓ ‹ ÙˆÓ ÓÔÌ›ÌˆÓ ÂÎÚÔÛÒˆÓ ÙÔ˘˜ Û‡Ìʈӷ Ì ÙȘ ‰È·ÎËÚ‡ÍÂȘ ÙÔ˘ EÏÛ›ÓÎÈ Î·È ÙÔ˘ TfiÎÈÔ. ŸÏ˜ ÔÈ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ıˆÚÔ‡ÓÙ·È È‰ÈÔÎÙËÛ›· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ “¶∞π¢π∞∆ƒπ∫◊” Î·È Ë ÔÏÈ΋ ‹ ÌÂ-
ÚÈ΋ ·Ó·‰ËÌÔÛ›Â˘Û‹ ÙÔ˘˜ ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ ÌÂÙ¿ ·fi ¤ÁÁÚ·ÊË Û˘ÁηٿıÂÛË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ∏ ¶∞π¢π∞∆ƒπ∫∏ ÚÔÙ›ÓÂÈ Ó· ·ÎÔÏÔ˘ıÔ‡ÓÙ·È ÔÈ “∫ÔÈÓ¤˜ ¶ÚԉȷÁڷʤ˜ ÁÈ· ÃÂÈÚfiÁÚ·Ê· Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È Û µÔ˚·ÙÚÈο ¶ÂÚÈÔ‰Èο” (Uniform Requirements for Manuscripts Submitted to Biomedical Journals) Ô˘ ‰ËÌÔÛȇÙËÎ·Ó ÛÙÔ JAMA 1997;277:927934, http://jama.ama-assn.org/info/auinst_req.html. OÈ ·fi„ÂȘ Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ Ô˘ ‰È·Ù˘ÒÓÔÓÙ·È ÛÙȘ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ‰ÂÓ ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ··Ú·›ÙËÙ· ·˘Ù¤˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. H ηٷ¯ÒÚËÛË ‰È·ÊËÌ›ÛÂˆÓ ÛÙÔ ÂÚÈÔ‰ÈÎfi ‰ÂÓ ˘Ô‰ËÏÒÓÂÈ ÔˆÛ‰‹ÔÙ ¤ÁÎÚÈÛË ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘˜ ·fi ÙËÓ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·, ÙËÓ EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‹ ·fi ÙÔÓ EΉfiÙË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. EÊfiÛÔÓ Ë ÂÚÁ·Û›· Á›ÓÂÈ ·Ô‰ÂÎÙ‹, ÙÔ ‰ÈÔÚıˆÌ¤ÓÔ Û‡Ìʈӷ Ì ÙȘ ˘Ô‰Â›ÍÂȘ ÙˆÓ ÎÚÈÙÒÓ Î·È ÂÎ Ó¤Ô˘ ‰·ÎÙ˘ÏÔÁÚ·ÊË̤ÓÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ˘Ô‚¿ÏÂÙ·È ÛÙËÓ EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ Û˘Óԉ¢fiÌÂÓÔ ·fi ‰ÈÛΤٷ Ô˘ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÚÁ·Û›· Û ÚfiÁÚ·ÌÌ· Word.doc ‹ MacWrite Î·È ÙÔ˘˜ ›Ó·Î˜, ·Ó Â›Ó·È ‰˘Ó·ÙfiÓ, Û ÚfiÁÚ·ÌÌ· Excel ηıÒ˜ Î·È ÂÈÛÙÔÏ‹ fiÔ˘ Ó· ·Ó·Ê¤ÚÔÓÙ·È ·Ó·Ï˘ÙÈο ÔÈ ÙÚÔÔÔÈ‹ÛÂȘ ‹ ÔÈ ·ÓÙÈÚÚ‹ÛÂȘ ÛÙȘ ÚÔÙ¿ÛÂȘ ÙˆÓ ÎÚÈÙÒÓ. H ηı˘ÛÙ¤ÚËÛË Ù˘ ·ӢԂÔÏ‹˜ ÙÔ˘ ÙÚÔÔÔÈË̤ÓÔ˘ ÎÂÈ̤ÓÔ˘ ¤Ú·Ó ÙˆÓ 40 ËÌÂÚÒÓ Û˘Ó¿ÁÂÙ·È Ó¤· ˘Ô‚ÔÏ‹. °È· Ó· ÂÈÙ¢¯ı› Ë ¤ÁηÈÚË ‰ËÌÔÛ›Â˘ÛË Ù˘ ÂÚÁ·Û›·˜ Â›Ó·È ··Ú·›ÙËÙÔ Ó· ÂÈÛÙÚ¤ÊÂÙ·È ÙÔ ‰ÈÔÚıˆÌ¤ÓÔ Ù˘ÔÁÚ·ÊÈÎfi ‰ÔΛÌÈÔ ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ ̤۷ Û ÂÙ¿ (7) Ë̤Ú˜. T· ¤ÍÔ‰· ÙˆÓ ÊÈÏ̘ 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›, Ù· Û¯‹Ì·Ù· Î·È ÔÈ ÊˆÙÔÁڷʛ˜ Ô˘ Ù· Û˘Óԉ‡ԢÓ. °. ™‡ÓÙ·ÍË ÙˆÓ ∂ÚÁ·ÛÈÒÓ 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 ‰·ÎÙ˘ÏÔÁÚ·ÊË̤ÓÔ ÛÙË ÌÈ· fi„Ë Ï¢ÎÔ‡ ¯·ÚÙÈÔ‡ ÌÂÁ¤ıÔ˘˜ A4 (22x28 cm) Ì ‰ÈÏfi ‰È¿ÛÙËÌ· ÌÂٷ͇ ÙˆÓ ÁÚ·ÌÌÒÓ Î·È Ê·Ú‰È¿ ÂÚÈıÒÚÈ· (2,5 cm) ÛÙȘ ‰‡Ô Ï¢ڤ˜. TÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ¤¯ÂÈ ÙËÓ ·ÎfiÏÔ˘ıË ‰È¿Ù·ÍË: ÛÂÏ›‰· Ù›ÙÏÔ˘, ‚Ú·¯‡˜ Ù›ÙÏÔ˜, ÂÚ›ÏË„Ë ÛÙ· ÂÏÏËÓÈο Î·È Ù· ·ÁÁÏÈο, ΛÌÂÓÔ, ¢¯·ÚÈÛٛ˜ ηıÒ˜ Î·È ·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢, ‚È‚ÏÈÔÁÚ·Ê›·, ›Ó·Î˜, ÂÈÎfiÓ˜, Ù›ÙÏÔÈ ÂÈÎfiÓˆÓ, ηٿÏÔÁÔ˜ Û˘ÓÙÌ‹ÛˆÓ. K¿ı ¤Ó· ·fi Ù· ·Ú·¿Óˆ ÛÙÔȯ›· Ú¤ÂÈ Ó· ·Ú¯›˙ÂÈ Û ¯ˆÚÈÛÙfi ʇÏÏÔ Î·È ÔÈ ÛÂÏ›‰Â˜ Ó· ·ÚÈıÌÔ‡ÓÙ·È ‰È·‰Ô¯Èο ·Ú¯›˙ÔÓÙ·˜ Ì ÙË ÛÂÏ›‰· Ù›ÙÏÔ˘.
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H ¤ÎÙ·ÛË ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ÁÈ· ÙȘ ·Ó·ÛÎÔ‹ÛÂȘ Û 15-25, ÁÈ· ÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ Û 12-15 ÛÂÏ›‰Â˜, ÁÈ· Ù· ›ηÈÚ· ı¤Ì·Ù· Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ Û 3-5 ‰·ÎÙ˘ÏÔÁÚ·ÊË̤Ó˜ ÛÂÏ›‰Â˜, ÂÓÒ ÁÈ· ÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ Û 1000 ϤÍÂȘ Î·È ÁÈ· ÙȘ ÂÈÛÙÔϤ˜ Û 250-300 ϤÍÂȘ. ™ÂÏ›‰· Ù›ÙÏÔ˘ ¶Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ: - ÙÔÓ Ù›ÙÏÔ (<14 ϤÍÂȘ) ηıÒ˜ Î·È ÙÔÓ ‚Ú·¯‡ Ù›ÙÏÔ (<5 ϤÍÂȘ) ÙÔ˘ ¿ÚıÚÔ˘. ¢ÂÓ ÂÈÙÚ¤ÔÓÙ·È Û˘ÓÙÌ‹ÛÂȘ, - ÙÔ fiÓÔÌ· Î·È ÙÔ ÂÒÓ˘ÌÔ Î¿ıÂ Û˘ÁÁڷʤ· ÛÙËÓ ÔÓÔÌ·ÛÙÈ΋, - 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˘ÁÁڷʤˆÓ (.¯. ȉÈÒÙ˘ ·È‰›·ÙÚÔ˜) Î·È Ô ÙfiÔ˜ ‰È·ÌÔÓ‹˜ ÙÔ˘/ÙˆÓ, - ÙËÓ Ï‹ÚË ‰È‡ı˘ÓÛË Î·È ÙÔ ÙËϤʈÓÔ ÙÔ˘ Û˘ÁÁڷʤ· Ì ÙÔÓ ÔÔ›Ô Á›ÓÂÙ·È Ë ·ÏÏËÏÔÁÚ·Ê›·. ¶ÂÚÈÏ‹„ÂȘ H ÂÚ›ÏË„Ë Ú¤ÂÈ Ó· ·Ó·ÎÂÊ·Ï·ÈÒÓÂÈ ÙÔ˘˜ ÛÙfi¯Ô˘˜ Ù˘ ÂÚÁ·Û›·˜, ÙË ÌÂıÔ‰ÔÏÔÁ›·, Ù· ΢ÚÈfiÙÂÚ· ·ÔÙÂϤÛÌ·Ù· Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ù˘ ÌÂϤÙ˘. ¢ÂÓ Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÂÈ ÙȘ 250 ϤÍÂȘ. ™ÙËÓ ÂÚ›ÏË„Ë ÛÙ· ·ÁÁÏÈο Ú¤ÂÈ Ó· ·Ó·ÁÚ¿ÊÔÓÙ·È ÛÙËÓ ·Ú¯‹ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Ô Ù›ÙÏÔ˜ Ù˘ ÂÚÁ·Û›·˜ Î·È Ù· ÔÓfiÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ ÛÙ· ·ÁÁÏÈο. TÔ ÂÚȯfiÌÂÓÔ ÙÔ˘ ·ÁÁÏÈÎÔ‡ ÎÂÈ̤ÓÔ˘ ‰ÂÓ Ú¤ÂÈ Ó· ‰È·Ê¤ÚÂÈ ·fi ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô ÂÏÏËÓÈÎfi. T· ¿ÚıÚ· Û‡ÓÙ·Í˘ Î·È ÔÈ ·ÂÈÎÔÓÈÛÙÈΤ˜ ÂÚÈÙÒÛÂȘ ¤¯Ô˘Ó ÌfiÓÔ ·ÁÁÏÈ΋ ÂÚ›ÏË„Ë. K¿Ùˆ ·fi ÙËÓ ÂÏÏËÓÈ΋ Î·È ÙËÓ ·ÁÁÏÈ΋ ÂÚ›ÏË„Ë ÛËÌÂÈÒÓÔÓÙ·È ÙÚÂȘ ¤ˆ˜ ¤ÓÙ ϤÍÂȘ ÎÏÂȉȿ Ô˘ ı· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ÁÈ· ÙÔ ıÂÌ·ÙÈÎfi ¢ÚÂÙ‹ÚÈÔ. K›ÌÂÓÔ OÈ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ÂÈÛ·ÁˆÁ‹, ÌÂıfi‰Ô˘˜, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘˙‹ÙËÛË. H ∂ÈÛ·ÁˆÁ‹ ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· ÙÂÏÂ˘Ù·›· ‰Â‰Ô̤ӷ Ù˘ ¤Ú¢ӷ˜ ÛÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ı¤Ì· Ì ÙȘ ΢ÚÈfiÙÂÚ˜ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ Î·È ÙÔ ÛÎÔfi Ù˘ ÂÚÁ·Û›·˜. H ÂÚÈÁÚ·Ê‹ ÙˆÓ ªÂıfi‰ˆÓ Ú¤ÂÈ Ó· Â›Ó·È ·ÎÚÈ‚‹˜ Î·È ·ÚÎÂÙ¿ ÏÂÙÔÌÂÚ‹˜ ÒÛÙ ӷ ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó··Ú·ÁˆÁ‹ ÙÔ˘˜ ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜. T· ∞ÔÙÂϤÛÌ·Ù· Ú¤ÂÈ Ó· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ۷ʋÓÂÈ· Î·È Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ÙËÓ ··Ú·›ÙËÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË. H ™˘˙‹ÙËÛË Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙËÓ ÂÚÁ·Û›·, ÙË ÛËÌ·Û›· Ô˘ ÌÔÚ› Ó· ¤¯Ô˘Ó Î·È ÙËÓ Èı·Ó‹ Û˘Û¯¤ÙÈÛ‹ ÙÔ˘˜ Ì ·Ú·ÙËÚ‹ÛÂȘ ¿ÏÏˆÓ ÂÚ¢ÓËÙÒÓ. OÈ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û‡ÓÙÔÌË ÂÈÛ·ÁˆÁ‹, ÂÚÈÁÚ·Ê‹ Ù˘ ÂÚ›ÙˆÛ˘ Î·È ‚Ú·¯Â›· Û˘˙‹ÙËÛË. OÈ ·ÂÈÎÔÓÈÛÙÈΤ˜ ÂÚÈÙÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û‡ÓÙÔÌË ÂÚÈÁÚ·Ê‹ Ù˘ ÂÚ›ÙˆÛ˘ ηÈ
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‚Ú·¯Â›· Û˘˙‹ÙËÛË Ì ¤ÌÊ·ÛË ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË. T· ˘fiÏÔÈ· ı¤Ì·Ù· ¤¯Ô˘Ó ÂχıÂÚË ‰ÔÌ‹ ηٿ ÙËÓ ÎÚ›ÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ÙÔ˘˜. ∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ - ¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢) ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Ù¤ÏÔ˜ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Î·È ÚÈÓ ·fi ÙȘ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜. TÈ̤˜ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ OÈ ÙÈ̤˜ ÙˆÓ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ Ú¤ÂÈ Ó· ÂÎÊÚ¿˙ÔÓÙ·È ÛÙÔ ¢ÈÂıÓ¤˜ ™‡ÛÙËÌ· MÔÓ¿‰ˆÓ (SI Units) Î·È ÛÙÔ MÂÙÚÈÎfi (Conventional-™˘Ì‚·ÙÈÎfi) ™‡ÛÙËÌ· ̤۷ Û ·Ú¤ÓıÂÛË. ¶›Ó·Î˜ ÌÂÙ·ÙÚÔ‹˜ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙÔ JAMA 1986;255:2329-2339. ™˘ÓÙÔÌÔÁڷʛ˜ OÈ Û˘ÓÙÔÌÔÁڷʛ˜ Ô˘ ¤¯Ô˘Ó ÂÈ‚ÏËı› ‰ÈÂıÓÒ˜ ‰ËÌÔÛȇÔÓÙ·È Û οı Ù‡¯Ô˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ™‡ÓıÂÙÔÈ Î·È Ì·ÎÚÔÛÎÂÏ›˜ fiÚÔÈ Ô˘ ·ӷϷ̂¿ÓÔÓÙ·È Û˘¯Ó¿ ÛÙÔ Î›ÌÂÓÔ ÌÔÚÔ‡Ó Ó· ·ÓÙÈηı›ÛÙ·ÓÙ·È ·fi Û˘ÓÙÔÌÔÁڷʛ˜ Ô˘ ÂÂÍËÁÔ‡ÓÙ·È ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ Û ÂȉÈÎfi ηٿÏÔÁÔ Ô ÔÔ›Ô˜ ˘Ô‚¿ÏÏÂÙ·È Ì·˙› Ì ÙËÓ ÂÚÁ·Û›·. OÈ Û˘ÓÙÔÌÔÁڷʛ˜ ·Ó·Ê¤ÚÔÓÙ·È Û ·Ú¤ÓıÂÛË ÌfiÓÔ ÛÙȘ ÂÚÈÏ‹„ÂȘ. BÈ‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ ™ÙÔ ÙÌ‹Ì· Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ ηٷ¯ˆÚÔ‡ÓÙ·È fiϘ ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ™ÙÔ Î›ÌÂÓÔ ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ ·Ó·Ê¤ÚÔÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Û ·Ú¤ÓıÂÛË. OÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·Ô̤˜ ‰ÂÓ Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÔ˘Ó ÙȘ 70 ÛÙȘ ·Ó·ÛÎÔ‹ÛÂȘ, ÙȘ 30 ÛÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜, ÙȘ 12 ÛÙ· ›ηÈÚ· ı¤Ì·Ù· Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ, ÙȘ 5 ÛÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ. H Û‡ÓÙ·ÍË ÙˆÓ ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ Á›ÓÂÙ·È Û‡Ìʈӷ Ì ÙȘ ÚԉȷÁڷʤ˜ Ù˘ International Committee of Medical Journal Editors / Uniform Requirements for Manuscripts Submitted to Biomedical Journals (JAMA 1997;277:927-934), http://jama.amaassn.org/info/auinst_req.html. OÈ Û˘ÓÙÌ‹ÛÂȘ ÙˆÓ Ù›ÙÏˆÓ ÙˆÓ ÂÚÈÔ‰ÈÎÒÓ Á›ÓÔÓÙ·È Ì ‚¿ÛË ÙÔ Cumulated Index Medicus (List of Journals Indexed in Index Medicus, http://www.nlm.nih.gov). ¶·Ú·‰Â›ÁÌ·Ù· ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ I. ¶ÂÚÈÔ‰Èο AÓ ÔÈ Û˘ÁÁÚ·Ê›˜ Â›Ó·È ¤ˆ˜ ¤ÍÈ ·Ó·ÁÚ¿ÊÔÓÙ·È fiÏÔÈ, ·Ó Â›Ó·È ÂÙ¿ ‹ ÂÚÈÛÛfiÙÂÚÔÈ ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ ÚÒÙÔÈ ¤ÍÈ Î·È ÚÔÛÙ›ıÂÙ·È “et al” ‹ “Î·È Û˘Ó”. T·ÎÙÈ΋ ¤Î‰ÔÛË ÂÚÈÔ‰ÈÎÔ‡: ∞ÏÂ͛Ԣ ¢, ª¿Ú· Ã, °È·ÓÓ·ÎÔÔ‡ÏÔ˘ Ã. ∂Ì‚fiÏÈ·, ·ÚfiÓ Î·È Ì¤ÏÏÔÓ: ›‰Ú·ÛË ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ÙˆÓ ÏÔÈ̈‰ÒÓ ÓÔÛËÌ¿ÙˆÓ Î·È ÙËÓ ·È‰È·ÙÚÈ΋ Ú¿ÍË. ¶·È‰È·ÙÚÈ΋ 1996;59:272-279.
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™˘ÌÏËڈ̷ÙÈÎfi Ù‡¯Ô˜ ÂÚÈÔ‰ÈÎÔ‡: Flyvbjerg A. Role of growth hormone, insulin-like growth factors (IGFs) and IGF-binding proteins in the renal complications of diabetes. Kidney Int 1997;52 (60 Suppl):S12-S19.
¶›Ó·Î˜ Î·È EÈÎfiÓ˜ AÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó (¤Ó· ÚˆÙfiÙ˘Ô+‰‡Ô ʈÙÔ·ÓÙ›ÁÚ·Ê·). ¶Ú¤ÂÈ Ó· ¤¯Ô˘Ó Ï¿ÙÔ˜ ›ÛÔ Ì ÙÔ Ï¿ÙÔ˜ ÙÔ˘ ÌÔÓfiÛÙËÏÔ˘ (8 cm) ‹ Ì ÙÔ Ï¿ÙÔ˜ fiÏ˘ Ù˘ ÛÂÏ›‰·˜ (16,8 cm). TÔ Ì¤ÁÈÛÙÔ Ì‹ÎÔ˜ ÙÔ˘˜, Ì·˙› Ì ÙÔ˘˜ Ù›ÙÏÔ˘˜, ‰ÂÓ Ú¤ÂÈ Ó· Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ·fi 22 cm. OÈ ›Ó·Î˜ ·ÚÈıÌÔ‡ÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ¶ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‚Ú·¯‡ Ù›ÙÏÔ Î·È ÂÂÍ‹ÁËÛË ÙˆÓ Û˘ÓÙÌ‹ÛÂˆÓ ÛÙÔ Î¿Ùˆ ̤ÚÔ˜. ™ÙÔ˘˜ ›Ó·Î˜ Ú¤ÂÈ Ó· ·ÔʇÁÔÓÙ·È ÔÈ Î¿ıÂÙ˜ ÁÚ·Ì̤˜. ŸÏÔ ÙÔ ·ÂÈÎÔÓÈÛÙÈÎfi ˘ÏÈÎfi (Û¯‹Ì·Ù·, ‰È·ÁÚ¿ÌÌ·Ù·, ʈÙÔÁڷʛ˜, ÎÏ.) ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ÂÈÎfiÓ˜. £· Ú¤ÂÈ Ó· Â›Ó·È ¿ÚÈÛÙ˘ ÔÈfiÙËÙ·˜ Ì ÌÔÚÊ‹ ʈÙÔÁÚ·ÊÈÒÓ ‹ Î·È ÚˆÙÔÙ‡ˆÓ. ™ÙÔ ›Ûˆ ̤ÚÔ˜ ÙˆÓ ÂÈÎfiÓˆÓ Ú¤ÂÈ Ó· ÛËÌÂÈÒÓÂÙ·È Ì ÌÔχ‚È Ô ·ÚÈıÌfi˜ Ù˘ ÂÈÎfiÓ·˜ Î·È ÙÔ fiÓÔÌ· ÙÔ˘ ÚÒÙÔ˘ Û˘ÁÁڷʤ·, ηıÒ˜ Î·È ¤Ó· ‚¤ÏÔ˜ ÙÔ ÔÔ›Ô Ó· ‰Â›¯ÓÂÈ ÙÔ ¿Óˆ ̤ÚÔ˜ Ù˘ ÂÈÎfiÓ·˜. ™ÙȘ ʈÙÔÁڷʛ˜ ·ÛıÂÓÒÓ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·ÁÓˆÚ›˙ÂÙ·È Ë Ù·˘ÙfiÙËÙ¿ ÙÔ˘˜. OÈ ·ÛıÂÓ›˜ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È ÔÓÔÌ·ÛÙÈο.
Èڛ˜ Û˘ÁÁڷʤ·: National Institutes of Health Consensus Development Conference. Neurofibromatosis conference statement. Arch Neurol 1988;45:575-578. ¶ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù‡Ô˘ ¿ÚıÚÔ˘: ∫Ô˘Ú›‰Ë˜ Ã, ∫Ô˘Ú›‰Ë˜ °. ¶·¯˘Û·ÚΛ· Î·È ˘ÂÚÏÈȉ·ÈÌ›· Û ·È‰È¿ ‚ÔÚÂÈÔ‰˘ÙÈ΋˜ ∫‡ÚÔ˘ [¶ÂÚ›ÏË„Ë]. 36Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ; 1998 5-7 πÔ˘Ó›Ô˘; ¶¿ÊÔ˜, ∫‡ÚÔ˜;1998. ÛÂÏ. 172. Schreiner GF, Lange L. Ethanol modulation of macrophage influx in glomerulonephritis [abstract]. J Am Soc Nephrol 1991;2:562. Should antileukotriene therapies be used instead of inhaled corticosteroids in asthma? [Editorial]. Am J Respir Crit Care Med 1998;158:1697-1701. Laux-End R, Inaebnit D, Gerber HA, Bianchetti MG. Vasculitis associated with levamisole and circulating autoantibodies [letter]. Arch Dis Child 1996;75:355-356. II. µÈ‚Ï›· ∫ÂÊ¿Ï·ÈÔ Û ‚È‚Ï›Ô: Clark AG, Barratt TM. Steroid-responsive nephrotic syndrome. In: Barratt TM, Arner ED, Harmon WE, editors. Pediatric Nephrology. 4th ed. Baltimore: Lippincott William Wilkins; 1999. p. 742. ™‡ÁÁÚ·ÌÌ· ‹ ÌÔÓÔÁÚ·Ê›·: Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck. 3rd ed. New York: Oxford University Press; 1990. ¢ËÌÔÛ›Â˘ÛË Û ÙfiÌÔ Ú·ÎÙÈÎÒÓ: Bauer AW. The two definitions of bacterial resistance. In: Smith AJ, Rogers CA, eds. Proceedings of the Third International Congress of Chemotherapy; 1962 May 29-31; New York: International Society of Chemotherapy; 1963. p. 484-500.
¶ÚÈÓ ·ÔÛÙ›ÏÂÙ ÙËÓ ÂÚÁ·Û›· Û·˜ ‚‚·Èˆı›Ù fiÙÈ ÂÚÈÏ·Ì‚¿ÓÂÈ: - ÂÈÛÙÔÏ‹ ˘Ô‚ÔÏ‹˜ - ‰‹ÏˆÛË ÌË ÚÔÁÂÓ¤ÛÙÂÚ˘ ‰ËÌÔÛ›Â˘Û˘ Ù˘ ÂÚÁ·Û›·˜ - 3 ·ÓÙ›ÁÚ·Ê· ÙÔ˘ ÎÂÈ̤ÓÔ˘ Ù˘ ÂÚÁ·Û›·˜ - ÛÂÏ›‰· Ù›ÙÏÔ˘ (¯ˆÚÈÛÙ‹ ÛÂÏ›‰·): ÂÚÈÏ·Ì‚¿ÓÂÈ - Ù›ÙÏÔ Î·È ‚Ú·¯‡ Ù›ÙÏÔ ÂÚÁ·Û›·˜ - fiÓÔÌ· Î·È ÂÒÓ˘ÌÔ Û˘ÁÁڷʤˆÓ (ÔÏÔÁڿʈ˜), ÂÈÛÙËÌÔÓÈÎfi/¿ ΤÓÙÚÔ/· fiÔ˘ ¤ÁÈÓÂ Ë ÂÚÁ·Û›· - fiÓÔÌ·, ‰È‡ı˘ÓÛË Î·È ÙËϤʈÓÔ Û˘ÁÁڷʤ· ÁÈ· ·ÏÏËÏÔÁÚ·Ê›· - ÂÚ›ÏË„Ë ÂÏÏËÓÈ΋-·ÁÁÏÈ΋, ϤÍÂȘ ÎÏÂȉȿ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) - ηٿÏÔÁÔ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) - ΛÌÂÓÔ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) - ¢¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢) - ‚È‚ÏÈÔÁÚ·Ê›· (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) - ›Ó·Î˜ (Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó - ÂÈÎfiÓ˜, Ì ‚¤ÏÔ˜ ÛÙÔ ›Ûˆ ̤ÚÔ˜ Ó· ‰Â›¯ÓÂÈ ÚÔ˜ Ù· ¿ӈ, ·ÚÈıÌË̤Ó˜, ÂȘ ÙÚÈÏÔ‡Ó - Ù›ÙÏÔÈ ÂÈÎfiÓˆÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó.
¢È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹: ¡ÙÔ˘ÓÙÔ˘Ï¿Î˘ π. ∏ ¯ÂÈÚÔ˘ÚÁÈ΋ ıÂڷ›· ÙÔ˘ Û˘ÁÁÂÓÔ‡˜ ÂÍ·ÚıÚ‹Ì·ÙÔ˜ ÙÔ˘ ÈÛ¯›Ô˘ Û ·È‰È¿ οو ÙÔ˘ ÂÓfi˜ ¤ÙÔ˘˜ [‰È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹]. ∞ı‹Ó·: ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ; 1979. Kaplan SJ. Post hospital home health care: the
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Paediatriki 2001;64:441-477
REVIEW ARTICLE
™¯ÈÛٛ˜ ÙÔ˘ ÛÙÔÌ·ÙÔÁÓ·ıÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ ª. ¡. ™˘ÚÔÔ‡ÏÔ˘
Clefts of the lip and palate M. N. Spyropoulos
¶ÂÚ›ÏË„Ë: OÈ Û¯ÈÛٛ˜ ÙÔ˘ ¯Â›ÏÔ˘˜ ‹/Î·È Ù˘ ˘ÂÚÒ·˜ ·ÔÙÂÏÔ‡Ó ÙËÓ ÈÔ Û˘¯Ó‹ ÂΉ‹ÏˆÛË Û˘ÁÁÂÓÒÓ ·ÓˆÌ·ÏÈÒÓ Î·È ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÙˆÓ Û˘ÁÁÂÓÒÓ ‰˘ÛÌÔÚÊÈÒÓ Ô˘ ·ÊÔÚÔ‡Ó ÛÙÔ ÎÚ·ÓÈÔÚÔÛˆÈÎfi Û‡ÌÏÂÁÌ·. ∏ ·ıÔÁ¤ÓÂÈ·, Ë Ù·ÍÈÓfiÌËÛË Î·È ÔÈ ‰È·‰Èηۛ˜ Ô˘ Û˘Ì‚¿ÏÏÔ˘Ó ÛÙËÓ Î¿ı ÂÚ›ÙˆÛË Î·È ÛÙÔ ÌÂÁ·Ï‡ÙÂÚÔ Î·Ù¿ ÙÔ ‰˘Ó·ÙfiÓ ÂÚÈÔÚÈÛÌfi ÙˆÓ ÚÔ‚ÏËÌ¿ÙˆÓ Ô˘ Û˘Ó˘¿Ú¯Ô˘Ó, ·ÊÔÚÔ‡Ó Û ÔÏϤ˜ ÂȉÈÎfiÙËÙ˜ ÙˆÓ ÂÈÛÙËÌÒÓ ˘Á›·˜ ÔÈ Ôԛ˜ ı· Ú¤ÂÈ Ó· Û˘ÓÂÚÁ·ÛÙÔ‡Ó ·fi ÙË ÛÙÈÁÌ‹ Ô˘ ı· ÁÂÓÓËı› ¤Ó· ·È‰› Ì οÔÈ· ÌÔÚÊ‹ Û¯ÈÛÙ›·˜. ™ÙËÓ ·Ó·ÛÎfiËÛË ·˘Ù‹ ·Ú·Ù›ıÂÓÙ·È Û˘ÓÔÙÈο ÔÈ Û‡Á¯ÚÔÓ˜ ·fi„ÂȘ Ô˘ ·ÊÔÚÔ‡Ó ÛÙ· ÈÔ ¿Óˆ Î·È ‰›ÓÂÙ·È ¤ÌÊ·ÛË ÛÙ· ¯ÚÔÓԉȷÁÚ¿ÌÌ·Ù· ·˘Ù‹˜ Ù˘ Û˘ÓÂÚÁ·ÙÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘ fiˆ˜ ÈÛ¯‡Ô˘Ó ÛÙ· ·ÓÙ›ÛÙÔȯ· ÔÚÁ·ÓˆÌ¤Ó· ÂȉÈο ΤÓÙÚ· Î·È ¤¯Ô˘Ó ȉȷÈÙ¤Úˆ˜ ÈηÓÔÔÈËÙÈο ·ÔÙÂϤÛÌ·Ù·.
Abstract: Clefts of the lip and/or palate comprise the most frequent expressions of congenital anomalies and the highest percentage of malformations related to the craniofacial complex. The etiology and pathology of these abnormalities, their classification, their implications and the procedures that minimize the coexisting problems require the close cooperation of many medical and dental specialties starting from the very first day that a child is born with a congenital defect. This review article gives a concise overview of the current concepts regarding the aformentioned problems, and emphasizes the significance of the time-tables that must be followed by the various specialties in the highly organized medical centres in order to obtain the best possible results.
§¤ÍÂȘ ÎÏÂȉȿ: ¯ÂÈÏÂÈÔÛ¯ÈÛٛ˜, ˘ÂÚˆÈÔÛ¯ÈÛٛ˜, ¯ÂÈÏÂÔÁÓ·ıÔ¸ÂÚˆÈÔÛ¯ÈÛٛ˜.
Key words: cleft lip, cleft palate, cleft lip and palate.
∂ÈÛ·ÁˆÁ‹ OÈ Û¯ÈÛٛ˜ ÙÔ˘ ¯Â›ÏÔ˘˜ ‹/Î·È Ù˘ ˘ÂÚÒ·˜, ÁÈ· ÙȘ Ôԛ˜ ·Ï·ÈfiÙÂÚ· ¯ÚËÛÈÌÔÔÈÔ‡Û·Ì ÙÔ˘˜ ÂÓÙÂÏÒ˜ ·‰fiÎÈÌÔ˘˜ fiÚÔ˘˜ “Ï·ÁÒ¯ÂÈÏÔ˜” Î·È “Ï˘ÎfiÛÙÔÌ·”, ·ÔÙÂÏÔ‡Ó ÙËÓ ÈÔ Û˘¯Ó‹ ÂΉ‹ÏˆÛË Û˘ÁÁÂÓÒÓ ·ÓˆÌ·ÏÈÒÓ (ÔÛÔÛÙfi 8,6% fiÏˆÓ ÙˆÓ ‰È·Ì·ÚÙÈÒÓ ÛÙË ‰È¿Ï·ÛË) Î·È ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi (65%) ÙˆÓ Û˘ÁÁÂÓÒÓ ‰˘ÛÌÔÚÊÈÒÓ Ô˘ ·ÊÔÚÔ‡Ó ÛÙÔ ÎÚ·ÓÈÔÚÔÛˆÈÎfi Û‡ÌÏÂÁÌ·. ªÔÚ› Ó· Û˘Ó˘¿Ú¯Ô˘Ó Ì ¿ÏϘ ·ÓˆÌ·Ï›Â˜ Û ‰È¿ÊÔÚ· Û‡Ó‰ÚÔÌ· ‹ Ó· Â›Ó·È ÌÂÌÔӈ̤Ó˜ ηٷÛÙ¿ÛÂȘ ÛÂ
¿ÙÔÌ· Ô˘ ‰ÂÓ ÂÌÊ·Ó›˙Ô˘Ó Î·Ó¤Ó· ¿ÏÏÔ Úfi‚ÏËÌ·. ∏ Û˘¯ÓfiÙËÙ· ÙˆÓ ÌË Û˘Ó‰ÚÔÌÈÎÒÓ Û¯ÈÛÙÈÒÓ ÛÙË Ï¢΋ Ê˘Ï‹, Î˘Ì·›ÓÂÙ·È ·fi 0,8-2 ·Ó¿ 1000 ÁÂÓÓ‹ÛÂȘ. ™ÙË ¯ÒÚ· Ì·˜, ·Ó Î·È ÔÈ ‰˘ÛÎÔϛ˜ ηٷÁÚ·Ê‹˜ Â›Ó·È Ôχ ÌÂÁ¿Ï˜, Ë Û˘¯ÓfiÙËÙ· ‰ÂÓ ˘ÂÚ‚·›ÓÂÈ ÙÔ 1 ·Ó¿ 700-800 ÁÂÓÓ‹ÛÂȘ (13). ∏ Û˘¯ÓfiÙËÙ· ·˘Ù‹ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË ÛÙËÓ Î›ÙÚÈÓË Ê˘Ï‹ Î·È ÌÈÎÚfiÙÂÚË ÛÙË Ì·‡ÚË. ™ÎÔfi˜ Ù˘ ·Ó·ÛÎfiËÛ˘ Ô˘ ·ÎÔÏÔ˘ı› Â›Ó·È Ë ·Ó·ÊÔÚ¿ ÛÙȘ Û‡Á¯ÚÔÓ˜ ·fi„ÂȘ, Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ·, ÙËÓ Ù·ÍÈÓfiÌËÛË Î·È
∫·ıËÁ‹ÙÚÈ·-¢È¢ı‡ÓÙÚÈ· ∂ÚÁ·ÛÙ‹ÚÈÔ OÚıÔ‰ÔÓÙÈ΋˜ O‰ÔÓÙÈ·ÙÚÈÎfi ∆Ì‹Ì· ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ
Professor and Head Department of Orthodontics University of Athens Dental School, Athens
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¶·È‰È·ÙÚÈ΋ 2001;64:441-447
Paediatriki 2001;64:441-477
ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·˘ÙÒÓ ÙˆÓ Î·Ù·ÛÙ¿ÛˆÓ. ∏ ÂÓË̤ڈÛË ÙˆÓ ·È‰È¿ÙÚˆÓ ÁÈ· Ù· ı¤Ì·Ù· ·˘Ù¿ ıˆÚÂ›Ù·È È‰È·ÈÙ¤Úˆ˜ ÛËÌ·ÓÙÈ΋. ∞ÈÙÈÔ·ıÔÁ¤ÓÂÈ· ∏ ÌÔÚÊ‹ Î·È Ë ¤ÎÙ·ÛË ÙˆÓ Û¯ÈÛÙÈÒÓ ÂÍ·ÚÙ¿Ù·È ·fi ÙÔ ÔȘ ·ÔʇÛÂȘ, ·fi ·˘Ù¤˜ Ô˘ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙË ‰È¿Ï·ÛË ÙÔ˘ ·ÓıÚÒÈÓÔ˘ ÚÔÛÒÔ˘ ηٿ ÙËÓ ÂÓ‰ÔÌ‹ÙÚÈ· ˙ˆ‹, ‰ÂÓ ‹Úı·Ó Û ·ʋ ÛÙËÓ Î·Ù¿ÏÏËÏË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ ‹/Î·È ‰ÂÓ Û˘ÓÂÓÒıËηÓ, ÛÙË Û˘Ó¤¯ÂÈ·, Ì ‰È›ۉ˘ÛË ÌÂÛÂÁ¯‡Ì·ÙÔ˜. ÃÂÈÏÂÔÛ¯ÈÛÙ›· ÚÔ·ÙÂÈ ·fi ÙËÓ ·ÔÙ˘¯›· Û˘Ó¤ÓˆÛ˘ Ù˘ ¿Óˆ ÁÓ·ıÈ·›·˜ ·ÔʇÛˆ˜ Ì ÙË ÌÂÛË ÚÈÓÈ΋, ηٿ ÙËÓ 6Ë-7Ë ÂÌ‚Ú˘˚΋ ‚‰ÔÌ¿‰·. ÀÂÚˆÈÔÛ¯ÈÛÙ›· ÚÔ·ÙÂÈ ·fi ÙËÓ ·ÔÙ˘¯›· Û˘Ó¤ÓˆÛ˘ ÙˆÓ ‰‡Ô ˘ÂÚˆ›ˆÓ ÂÙ¿ÏˆÓ ÌÂٷ͇ ÙÔ˘˜ Î·È Ì ٷ ÛÙÔȯ›· Ù˘ ̤Û˘ ÁÚ·ÌÌ‹˜, ÁÈ· ÙÔ Û¯ËÌ·ÙÈÛÌfi Ù˘ ‰Â˘ÙÂÚÔÁÂÓÔ‡˜ ˘ÂÚÒ·˜, ηٿ ÙËÓ 9Ë-10Ë ÂÌ‚Ú˘˚΋ ‚‰ÔÌ¿‰·. ™˘Ó‰˘·ÛÌfi˜ ÙˆÓ ‰‡Ô ·˘ÙÒÓ Î·Ù·ÛÙ¿ÛÂˆÓ ÂΉËÏÒÓÂÙ·È Ì ¯ÂÈÏÂÔÁÓ·ıÔ¸ÂÚˆÈÔÛ¯ÈÛÙ›·, fiÙ·Ó ÙÔ ¯¿ÛÌ· ·Ú¯›˙ÂÈ ·fi ÙÔ ¯Â›ÏÔ˜, ‰È·Û¯›˙ÂÈ ÙËÓ ¿Óˆ ÁÓ¿ıÔ Î·È Î·Ù·Ï‹ÁÂÈ ÛÙË Ì·Ï·Î‹ ˘ÂÚÒ·. ŸÏ˜ ÔÈ ÈÔ ¿Óˆ ηٷÛÙ¿ÛÂȘ, Ì ‰È¿ÊÔÚÔ˘˜ Û˘Ó‰˘·ÛÌÔ‡˜ Ï‹ÚÔ˘˜, ·ÙÂÏÔ‡˜ ‹ Î·È ˘ÔÙ˘Ò‰Ô˘˜ ÌÔÚÊ‹˜ (ÌÈÎÚÔÌÔÚÊ‹˜), ÌÔÚ› Ó· Â›Ó·È ÂÙÂÚfiÏ¢Ú˜ ‹ ·ÌʛϢژ (∂ÈÎfiÓ· 1). ∏ ·ÈÙÈÔÏÔÁ›· ÙˆÓ Û¯ÈÛÙÈÒÓ ‰ÂÓ Â›Ó·È ·ÎfiÌË Ï‹Úˆ˜ ÂÍ·ÎÚÈ‚ˆÌ¤ÓË. ¢ÂÓ Ê·›ÓÂÙ·È Ó· Â›Ó·È ›‰È· ÁÈ· ÙȘ ‰È¿ÊÔÚ˜ ÂÓÙÔ›ÛÂȘ Î·È Û˘Ó‹ıˆ˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ÔÏ˘·Ú·ÁÔÓÙÈ΋. ªÂ ÙËÓ ÚfiÔ‰Ô, fï˜, ÙˆÓ ÂÚ¢ÓÒÓ Ô˘ ·ÊÔÚÔ‡Ó ÛÙÔ ÁÔÓȉ›ˆÌ· ÙÔ˘ ·ÓıÚÒÔ˘, ›Ûˆ˜ Ó· ÌËÓ ·¤¯ÂÈ Ôχ Ë ‰˘Ó·ÙfiÙËÙ· ÚÔÛ‰ÈÔÚÈÛÌÔ‡ Ôχ Û˘ÁÎÂÎÚÈÌ¤ÓˆÓ ÁÂÓÂÙÈÎÒÓ ÛÙÔȯ›ˆÓ Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ·ÈÙÈÔÏÔÁ›· ÙˆÓ Û¯ÈÛÙÈÒÓ. ª¤¯ÚÈ Û‹ÌÂÚ·, ¿ÓÙˆ˜, ÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ Â¯Ô˘Ó ÂÓÔ¯ÔÔÈËı› Â›Ó·È ÁÂÓÂÙÈÎÔ› Î·È ÂÚÈ‚·ÏÏÔÓÙÈÎÔ›. ∞˘ÙÔ› ÔÈ ÙÂÏÂ˘Ù·›ÔÈ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ÙÂÚ·ÙÔÁfiÓÔÈ Î·È Û˘ÌÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‰È¿ÊÔÚ˜ ÂÓ‰ÔÌ‹ÙÚȘ ηٷÛÙ¿ÛÂȘ, „˘¯ÔÏÔÁÈΤ˜ ηٷÛÙ¿ÛÂȘ Ù˘ ÌËÙ¤Ú·˜, ÌÔχÓÛÂȘ Ù˘ ÌËÙ¤Ú·˜ ·fi ÈÔ‡˜ ‹ Ï‹„Ë ‰È·ÊfiÚˆÓ Ê·ÚÌ¿ÎˆÓ ·fi ·˘Ù‹Ó, Ì˯·ÓÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, ·ÎÙÈÓÔ‚ÔÏ›· Î.¿. (12). OÈ ·Ú¿ÁÔÓÙ˜ ·˘ÙÔ› ‰ÚÔ˘Ó Û ÎÚ›ÛÈ̘ ÂÚÈfi‰Ô˘˜ Ù˘ ÂÓ‰ÔÌ‹ÙÚÈ·˜ ˙ˆ‹˜, Ì ÙÂÏÈÎfi ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÎÙÚÔ‹ ·fi ÙÔ Ê˘ÛÈÔÏÔÁÈÎfi ÔÚÈÛÌ¤ÓˆÓ ‚·ÛÈÎÒÓ ÌÔÚÊÔÁÂÓÂÙÈÎÒÓ Ì˯·ÓÈÛÌÒÓ ÙÔ˘ ÎÚ·ÓÈÔÚÔÛˆÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜, fiˆ˜ ›ӷÈ: ·) Ë Î˘ÙÙ·ÚÈ΋ ‰È·ÊÔÚÔÔ›ËÛË, ‚) Ë Î˘ÙÙ·ÚÈ΋ ÌÂÙ·Ó¿ÛÙ¢ÛË, Á) Ô ¤ÏÂÁ¯Ô˜ ÙÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡, ‰) ÔÈ ·ÏÏËÏÂȉڿÛÂȘ ÌÂٷ͇ ÁÂÈÙÔÓÈÎÒÓ ÈÛÙÒÓ (Î·È È‰È·›ÙÂÚ· ÌÂٷ͇ ÂÈıËÏ›Ô˘ Î·È ÌÂÛÂÁ¯‡Ì·ÙÔ˜), Â) Ë Î˘ÙÙ·ÚÈ΋ ÚÔÛÎfiÏÏËÛË, ÛÙ) Ô ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓÔ˜ ΢ÙÙ·ÚÈÎfi˜ ı¿Ó·ÙÔ˜/·fiÙˆÛË Î·È ˙) Ë
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∂ÈÎfiÓ· 1. ™¯ËÌ·ÙÈΤ˜ ·Ú·ÛÙ¿ÛÂȘ ‰È·ÊfiÚˆÓ ÌÔÚÊÒÓ Û¯ÈÛÙÈÒÓ ÙÔ˘ ¯Â›ÏÔ˘˜ Î·È Ù˘ ˘ÂÚÒ·˜, fiˆ˜ Ê·›ÓÔÓÙ·È ·fi ÙÔ ÂÛˆÙÂÚÈÎfi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ (·,‚,Á,‰): ·) ∂ÙÂÚfiÏ¢ÚË ¯ÂÈÏÂÔÁÓ·ıÔÛ¯ÈÛÙ›·, ‚) ∂ÙÂÚfiÏ¢ÚË ¯ÂÈÏÂÔÁÓ·ıÔ¸ÂÚˆÈÔÛ¯ÈÛÙ›·, Á) ∞ÌʛϢÚË ¯ÂÈÏÂÔÁÓ·ıÔ¸ÂÚˆÈÔÛ¯ÈÛÙ›·, ‰) ªÂÌÔӈ̤ÓË ˘ÂÚˆÈÔÛ¯ÈÛÙ›·. ™¯ËÌ·ÙÈΤ˜ ·Ú·ÛÙ¿ÛÂȘ ‰È·ÊfiÚˆÓ Û¯ÈÛÙÈÒÓ ÙÔ˘ ¯Â›ÏÔ˘˜ (Â,ÛÙ): Â) ∂ÙÂÚfiÏ¢ÚË ·ÚÈÛÙÂÚ‹ ¯ÂÈÏÂÔÛ¯ÈÛÙ›·, ÛÙ) ∞ÌʛϢÚË ¯ÂÈÏÂÔÛ¯ÈÛÙ›·. ™¯ËÌ·ÙÈΤ˜ ·Ú·ÛÙ¿ÛÂȘ ÌÈÎÚÔÌÔÚÊÒÓ (˙,Ë): ˙) ∂ÓÙÔÌ‹ ÙÔ˘ ¯Â›ÏÔ˘˜, Ë) ¢Èۯȉ‹˜ ÛÙ·Ê˘Ï‹.
›‰Ú·ÛË ÔÚÌÔÓÒÓ Î·È ‰È·ÊfiÚˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. OÈ Ì˯·ÓÈÛÌÔ› ·˘ÙÔ› Â›Ó·È Û˘ÓÙÔÓÈṲ̂ÓÔÈ ÌÂٷ͇ ÙÔ˘˜ Î·È ·ÎÔÏÔ˘ıÔ‡Ó ¤Ó·Ó ·˘ÛÙËÚfi ÚÔÁÚ·ÌÌ·ÙÈÛÌfi, ÙÔ˘ ÔÔ›Ô˘ Ë ‰È·Ù·Ú·¯‹ Û ̛· ÎÚ›ÛÈÌË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹, ÌÂÙ¿ ·fi ÚÔÛ‚ÔÏ‹ οÔÈ·˜ ¢¿ÏˆÙ˘ ÂÚÈÔ¯‹˜ ÙÔ˘ ÎÚ·ÓÈÔÚÔÛˆÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜, Â›Ó·È ‰˘Ó·Ùfi Ó· Ô‰ËÁ‹ÛÂÈ ÛÙËÓ ÂÌÊ¿ÓÈÛË
¶·È‰È·ÙÚÈ΋ 2001;64:441-447
‰˘ÛÌÔÚÊÔÁÂÓÂÙÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ, fiˆ˜ Â›Ó·È ÔÈ Û¯ÈÛٛ˜. ™Â ÌÔÚÈ·Îfi ›‰Ô, ÔÈ ÌÔÚÊÔÁÂÓÂÙÈÎÔ› Ì˯·ÓÈÛÌÔ› Ù˘ ·Ó¿Ù˘Í˘ ÙÔ˘ ÎÚ·ÓÈÔÚÔÛˆÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ ‰È·ÌÂÛÔÏ·‚Ô‡ÓÙ·È, ηٿ ·ÚÈÔ ÏfiÁÔ, ·fi ÂÓÒÛÂȘ Ù˘ ÂÍˆÎ˘ÙÙ¿ÚÈ·˜ ıÂÌÂÏ›Ô˘ Ô˘Û›·˜, fiˆ˜ Â›Ó·È ÙÔ ˘·ÏÔ˘ÚÔÓÈÎfi Ô͇, ÙÔ ÎÔÏÏ·ÁfiÓÔ Ù‡Ô˘ πÃ, Ë ÙÂÓ·ÛΛÓË Î.¿. (5) Î·È ·fi ‰È·Ï˘Ù¤˜ Ì·ÎÚÔ- ‹ ÌÈÎÚÔÌÔÚȷΤ˜ ÂÓÒÛÂȘ, ÔÈ Ôԛ˜ Û ÁÂÓÈΤ˜ ÁÚ·Ì̤˜ ·Ó‹ÎÔ˘Ó Û ‰‡Ô ÔÌ¿‰Â˜ (9): ·) ÛÙËÓ ˘ÂÚÔÈÎÔÁ¤ÓÂÈ· ÛÙÂÚÔÂȉÒÓ ÂÓÒÛÂˆÓ - ı˘ÚÂÔÂȉÈÎÒÓ ·Ú·ÁÒÁˆÓ-ÚÂÙÈÓÔ˚ÎÔ‡ ÔͤԘ Î·È ‚) ÛÙËÓ Î·ÙËÁÔÚ›· ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (Growth Factors). ∆· ‰Â‰Ô̤ӷ ·fi ÙȘ Û¯ÂÙÈΤ˜ ¤Ú¢Ó˜ ˘Ô‰ËÏÒÓÔ˘Ó ÙÔ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ˆ˜ ÌÂÛÔÏ·‚ËÙÒÓ ÁÈ· ÙË ÌÔÚÊÔÁ¤ÓÂÛË Î·È ‰˘ÛÌÔÚÊÔÁ¤ÓÂÛË ÙÔ˘ ÎÚ·ÓÈÔÚÔÛˆÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ Î·È ÂȉÈÎfiÙÂÚ· ÛÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ ·Ó¿Ù˘Í˘ ÙˆÓ Û¯ÈÛÙÈÒÓ, ηıÒ˜ ¤¯ÂÈ ·ÓȯÓ¢ı› ÛÙ· ·ÙÙ·Ú· ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛÂˆÓ Ë ‡·ÚÍË ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Î·È ÙÔ˘ ·ÓÙ›ÛÙÔÈ¯Ô˘ mRNA Ô˘ ÙÔ˘˜ Έ‰ÈÎÔÔÈ›. ¶·Ú·‰Â›ÁÌ·Ù· Ù¤ÙÔÈˆÓ ·Ú·ÁfiÓÙˆÓ Â›Ó·È: ·) Ô ÂȉÂÚÌÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ (EGF), Û ÚÒÈÌ· ÛÙ¿‰È· Û¯ËÌ·ÙÈÛÌÔ‡ Ù˘ ˘ÂÚÒ·˜ (1) Î·È ‚) Ô ÌÂÙ·ÌÔÚʈÙÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ¿ÏÊ· (TGF-a), ηı’ fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ‰È·‰Èηۛ·˜ Û˘Ó¤ÓˆÛ˘ ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛˆÓ, fiˆ˜ ›Û˘ Î·È ÔÈ ·Ú¿ÁÔÓÙ˜ TGF-‚1, TGF-‚2 Î·È TGF-‚3 (1). O ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (PDGF), Ô fiÔÈÔ˜ ·ÔÙÂÏ› ÛËÌ·ÓÙÈÎfi ·Ú¿ÁÔÓÙ· Ù˘ ÂԇψÛ˘ ÙˆÓ ÈÛÙÒÓ, ÌÔÚ› ‰˘ÓËÙÈο Ó· Û˘ÌÌÂÙ¤¯ÂÈ ÛÙÔ Û¯ËÌ·ÙÈÛÌfi Ù˘ ÂÈıËÏȷ΋˜ “Ú·Ê‹˜” ÌÂٷ͇ ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛˆÓ, ÛÙËÓ ·Ô‰ÈÔÚÁ¿ÓˆÛ‹ Ù˘, ·ÏÏ¿ Î·È ÛÙËÓ ·Ô‰ÈÔÚÁ¿ÓˆÛË Ù˘ ÌÂÛÂÁ¯˘Ì·ÙÈ΋˜ Û˘Ì˘ÎÓÒÛˆ˜ Ô˘ ÙËÓ ·ÎÔÏÔ˘ı› (5). ∆· Â˘Ú‹Ì·Ù· ÂÈÚ·Ì·ÙÈÎÒÓ ÂÚÁ·ÛÈÒÓ ¤¯Ô˘Ó ‰ÒÛÂÈ, ηٿ ÙËÓ ÙÂÏÂ˘Ù·›· ‰ÂηÂÙ›·, ‰Â‰Ô̤ӷ Ô˘ ‰È¢ڇÓÔ˘Ó ÂÓÙ˘ˆÛȷο ÙȘ ÁÓÒÛÂȘ Ì·˜ Û¯ÂÙÈο Ì ÙËÓ ÂÍÂȉÈÎÂ˘Ì¤ÓË, ÂÈÏÂÎÙÈ΋ Î·È ¯ÚÔÓÔÛ˘Ó·ÚÙÒÌÂÓË ‰Ú¿ÛË ÙˆÓ ‰È·ÊfiÚˆÓ ·˘ÙÒÓ ·Ú·ÁfiÓÙˆÓ. ∂Âȉ‹ Ù· ÛËÌ·ÓÙÈÎfiÙÂÚ· ·fi Ù· Â˘Ú‹Ì·Ù· ·˘Ù¿ ı· ·ÔÙÂϤÛÔ˘Ó ÙË ‚¿ÛË ÙˆÓ ÂÚ¢ÓËÙÈÎÒÓ ·Ó·˙ËÙ‹ÛÂˆÓ ÁÈ· Ù· ÚÔÛ¯‹ ¯ÚfiÓÈ·, Â›Ó·È ÛÎfiÈÌÔ Ó· Û˘ÓÔ„ÈÛÙÔ‡Ó ÛÙ· ÂÍ‹˜: ∞) Œ¯ÂÈ ·Ú·ÙËÚËı› fiÙÈ Ë Û‡ÓıÂÛË (·fi Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛˆÓ) ˘·ÏÔ˘ÚÔÓÈÎÔ‡ ÔͤԘ, Ì›·˜ Ô˘Û›·˜ Ì ÂÓÂÚÁfi ÚfiÏÔ Î·Ù¿ ÙË Ê¿ÛË Ù˘ ·Ó‡„ˆÛ˘ ÙˆÓ Ï·Á›ˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛÂˆÓ ÁÈ· ÙÔ Û¯ËÌ·ÙÈÛÌfi Ù˘ ‰Â˘ÙÂÚÔÁÂÓÔ‡˜ ˘ÂÚÒ·˜, ÚÔ¿ÁÂÙ·È ·fi ÙÔ˘˜ ·˘ÍËÙÈÎÔ‡˜
Paediatriki 2001;64:441-477
·Ú¿ÁÔÓÙ˜ EGF (4) Î·È TGF-‚ (16). µ) ∆· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· Ù˘ ˘ÂÚÒ·˜ ÂÚȤ¯Ô˘Ó ÌÂÁ¿ÏÔ ·ÚÈıÌfi ˘Ô‰Ô¯¤ˆÓ ÁÈ· ÙÔÓ EGF (15), ÂÓÒ Ù· ÂÈıËÏȷο ·ÙÙ·Ú· Ù˘ ·Ú˘Ê‹˜ ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛÂˆÓ ÂÌÊ·Ó›˙Ô˘Ó EGF- ˘Ô‰Ô¯Â›˜ ÌfiÓÔ ÌÂÙ¿ ÙËÓ ÔÚÈ˙ÔÓÙ›ˆÛ‹ ÙÔ˘˜ (5). O EGF ¤¯ÂÈ ·Ú·ÙËÚËı› fiÙÈ Â¿ÁÂÈ ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ù˘ ˘ÂÚÒ·˜ (7), ÂÓÒ Î¿Ùˆ ·fi ÙËÓ Â›‰Ú·ÛË ÙÔ˘ ÁÂÈÙÔÓÈÎÔ‡ ÌÂÛÂÁ¯‡Ì·ÙÔ˜, ·Ó·ÛÙ¤ÏÏÂÈ ÙËÓ ·fiÙˆÛË ÙˆÓ ÂÈıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ Ù˘ “Ú·Ê‹˜” Ô˘ ‰ËÌÈÔ˘ÚÁÂ›Ù·È Î·ıÒ˜ ÔÈ ÂÈıËÏȷΤ˜ ÂÈÊ¿ÓÂȘ ÙˆÓ Ï·Á›ˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛÂˆÓ ¤Ú¯ÔÓÙ·È Û ·ʋ (24). °) O ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓÔ˜ ΢ÙÙ·ÚÈÎfi˜ ı¿Ó·ÙÔ˜/·fiÙˆÛË ÙˆÓ ÂÈıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ Ù˘ “Ú·Ê‹˜” ÌÂٷ͇ ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛˆÓ, ηٿ ÙÔ Û¯ËÌ·ÙÈÛÌfi Ù˘ ‰Â˘ÙÂÚÔÁÂÓÔ‡˜ ˘ÂÚÒ·˜, Â›Ó·È Èı·Ófi Ó· ˘fiÎÂÈÙ·È ÛÙË ‰È·ÌÂÛÔÏ¿‚ËÛË ÙÔ˘ ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· TGF-‚ (5,6,23), ηıÒ˜ Ô ·Ú¿ÁÔÓÙ·˜ ·˘Ùfi˜, ÛÙȘ ‰È¿ÊÔÚ˜ ÈÛÔÌÔÚʤ˜ ÙÔ˘, ·ÓȯÓ‡ÂÙ·È ÛÙËÓ ·Ó·Ù˘ÛÛfiÌÂÓË ‰Â˘ÙÂÚÔÁÂÓ‹ ˘ÂÚÒ· (6) Î·È ¤¯ÂÈ ‚ÚÂı› fiÙÈ Â›Ó·È ÈηÓfi˜ Ó· ÂÓÂÚÁÔÔÈ‹ÛÂÈ ÙÔ ı¿Ó·ÙÔ ÂÈıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ (17). ¢) ∫·Ù¿ ÙÔ Û¯ËÌ·ÙÈÛÌfi Ù˘ ˘ÂÚÒ·˜ ÙˆÓ ÔÓÙÈÎÒÓ, ·ÓȯÓ‡ÂÙ·È ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÙˆÓ ÂȤ‰ˆÓ mRNA Ô˘ Έ‰ÈÎÔÔÈ› ÙÔ˘˜ ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ TGF-‚1, TGF-‚2 Î·È TGF-‚3 Î·È Ì›ˆÛË ÙˆÓ ·ÓÙ›ÛÙÔȯˆÓ ÂȤ‰ˆÓ ÁÈ· ÙÔÓ TGF-‚2. ∏ ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÂȉÒÓ (Ù· ÔÔ›·, fiÙ·Ó ¯ÔÚËÁÔ‡ÓÙ·È Û ÎÚ›ÛÈ̘ ÂÚÈfi‰Ô˘˜ Ù˘ ·Ó¿Ù˘Í˘, ·ÔÙÂÏÔ‡Ó ÈÛ¯˘ÚÔ‡˜ ·Ú¿ÁÔÓÙ˜ ‰˘ÛÌÔÚÊÔÁÂÓ¤Ûˆ˜ Î·È ÂȉÈÎfiÙÂÚ· Û¯ÈÛÙÈÒÓ) ‰ÂÓ ÂËÚ¿˙ÂÈ ÙȘ ¯ÚÔÓÈΤ˜ ÌÂÙ·‚ÔϤ˜ ÙˆÓ ÂȤ‰ˆÓ ÙˆÓ TGF‚1 Î·È TGF-‚3, ·ÏÏ¿ ηı˘ÛÙÂÚ› ηٿ Ì›· Ë̤ڷ ÙË Ì›ˆÛË ÙˆÓ ÂȤ‰ˆÓ ÙÔ˘ TGF-‚2. ∫·ıÒ˜ Ô TGF-‚2 ·Ó·¯·ÈÙ›˙ÂÈ ÙÔÓ Î˘ÙÙ·ÚÈÎfi ÔÏÏ·Ï·ÛÈ·ÛÌfi, Ë ‰È·Ù‹ÚËÛ‹ ÙÔ˘ Û ˘„ËÏ¿ ›‰· Û ÎÚ›ÛÈÌË ÂÚ›Ô‰Ô ·˘Í‹Ûˆ˜ ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛˆÓ, ›Ûˆ˜ Ó· ·ÔÙÂÏ› Î·È ÙÔ ÎÏÂȉ› Ù˘ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ·˜ ÙˆÓ Û¯ÈÛÙÈÒÓ ·fi Ï‹„Ë ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ (8). ∂) O ·Ú¿ÁÔÓÙ·˜ TGF-‚3 Ê·›ÓÂÙ·È Ó· ‰È·‰Ú·Ì·Ù›˙ÂÈ ¤Ó·Ó ȉȷ›ÙÂÚÔ ÚfiÏÔ ÛÙÔ Û¯ËÌ·ÙÈÛÌfi Ù˘ ˘ÂÚÒ·˜. O Brunet (3) Î·È ÔÈ Taya Î·È Û˘Ó (23) ÙÂÎÌËÚ›ˆÛ·Ó fiÙÈ, Û ÔÓÙ›ÎÈ·, ÂÊfiÛÔÓ ·Ó·ÛÙ·Ï› Ë ‰Ú·ÛÙËÚÈfiÙËÙ· ÙÔ˘ ·Ú¿ÁÔÓÙ· ·˘ÙÔ‡, ‰ÂÓ ÔÏÔÎÏËÚÒÓÂÙ·È Ë Û˘Ó¤ÓˆÛË ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛÂˆÓ Î·È ÚÔηÏÂ›Ù·È ˘ÂÚˆÈÔÛ¯ÈÛÙ›·. ∞ÓÙ›ÛÙÔȯ·, Û ÂÈÚ¿Ì·Ù· Ô˘ ¤ÁÈÓ·Ó Û ÎÔÙfiÔ˘Ï· (22), ‚Ú¤ıËΠfiÙÈ Ô ·Ú¿ÁÔÓÙ·˜ TGF-‚3 ÚÔ¿ÁÂÈ ÙË “ÌÂÙ·ÌfiÚʈÛË” ÙˆÓ ÂÈıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ Ù˘ ·Ú˘Ê‹˜ ÙˆÓ ˘ÂÚˆ›ˆÓ ·ÔʇÛÂˆÓ Û ÌÂÛÂÁ¯˘Ì·ÙÈο.
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¶·È‰È·ÙÚÈ΋ 2001;64:441-447
Paediatriki 2001;64:441-477
∆· Â˘Ú‹Ì·Ù· ·˘Ù¿, ·Ó Î·È ‰ÂÓ ··ÓÙÔ‡Ó Û fiÏ· Ù· ÂÚˆÙ‹Ì·Ù· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ· ÙˆÓ Û¯ÈÛÙÈÒÓ, ¤¯Ô˘Ó ·Ú¯›ÛÂÈ Ó· ·ÔηχÙÔ˘Ó ÙË ÌÔÚȷ΋ ‚ÈÔÏÔÁÈ΋ ‚¿ÛË Ù˘ ÂÈÚ·Ì·ÙÈ΋˜ ÚfiÎÏËÛ‹˜ ÙÔ˘˜. ∂Ô̤ӈ˜, ›Ûˆ˜ Î·È Ó· ÌËÓ Â›Ó·È ·Ú·ÎÈÓ‰˘ÓÂ˘Ì¤ÓÔ Ó· ˘Ôı¤ÛÔ˘Ì fiÙÈ Ì ÙËÓ ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛË - Û ˘ÂÚÌÈÎÚÔÛÎÔÈÎfi Â›Â‰Ô - Î·È ÙËÓ Ù·˘ÙÔÔ›ËÛË ÙˆÓ ˘Â‡ı˘ÓˆÓ ·ÓÙ›ÛÙÔȯˆÓ ÁÔÓȉ›ˆÓ, Ë ÂÓ‰ÔÌ‹ÙÚÈ· ¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È Ë ·ÓÙ›ÛÙÔÈ¯Ë ·ÓÙÈÌÂÙÒÈÛË (.¯. Ì ̛· ÂÓ‰ÔÊϤ‚È· ¤ÓÂÛË ÙÔ˘ ÂÏÏ›ÔÓÙÔ˜ ·Ú¿ÁÔÓÙo˜ ÛÙË ÌËÙ¤Ú·, ÙËÓ Î·Ù¿ÏÏËÏË Ë̤ڷ Î·È ÒÚ·) ı· Û˘Ì‚¿ÏÏÔ˘Ó Ô˘ÛÈ·ÛÙÈο ÛÙËÓ Â›Ï˘ÛË ÙÔ˘ ÛËÌ·ÓÙÈÎÔ‡ ·˘ÙÔ‡ ÚÔ‚Ï‹Ì·ÙÔ˜. ∆·ÍÈÓfiÌËÛË Œ¯Ô˘Ó ÚÔÙ·ı›, ηٿ ηÈÚÔ‡˜, ÔÏϤ˜ Ù·ÍÈÓÔÌ‹ÛÂȘ ÙˆÓ ‰È·ÊfiÚˆÓ ÎÏÈÓÈÎÒÓ ÂΉËÏÒÛÂˆÓ ÙˆÓ Û¯ÈÛÙÈÒÓ, ÔÈ Ôԛ˜ ¤¯Ô˘Ó ÚÔ·„ÂÈ ·fi ÙÚÂȘ ‚·ÛÈΤ˜ ÚԉȷÁڷʤ˜: ·) ÙËÓ ÂÌ‚Ú˘˚΋ ÚԤϢÛË ÙˆÓ Û¯ÈÛÙÈÒÓ, Ë ÔÔ›· Ô‰ËÁ› ÛÙËÓ Î·ÙËÁÔÚÈÔÔ›ËÛ‹ ÙÔ˘˜ Û ÙÚÂȘ ÔÌ¿‰Â˜ (10), fiˆ˜ Ê·›ÓÂÙ·È ÛÙÔ ™¯‹Ì· 1 (10), ‚) ÙË ÏÂÙÔÌÂÚÂȷ΋ ÔÙÈ΋ ηٷÁÚ·Ê‹ Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜ Î·È Á) ÙËÓ ÚÔÛ¿ıÂÈ· Û‡˙¢Í˘ Ù˘ ÎÏ·ÛÈ΋˜ Î·È ÂÌ‚Ú˘˚ÎÒ˜ ηıÔÚÈṲ̂Ó˘ Ù·ÍÈÓfiÌËÛ˘ Ì ̛· ‡¯ÚËÛÙË ÔÙÈ΋ ÁÚ·ÊÈ΋ ·Ú¿ÛÙ·ÛË Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜, Ô˘ Ô‰‹ÁËÛ ÛÙÔ “ÁÚ·Ì̈Ùfi ÏÔÁfiÙ˘Ô Ù‡Ô˘ À” (Kernahan 1971), ÙÔ˘ ÔÔ›-
Ô˘ Ë ÙÂÏÂ˘Ù·›· Û˘ÌÏËڈ̤ÓË ¤Î‰ÔÛË ¯ÚËÛÈÌÔÔÈÂ›Ù·È ·fi ÙËÓ OÌÔÛÔÓ‰›· ÙˆÓ ∫Ú·ÓÈÔÚÔÛˆÈÎÒÓ OÌ¿‰ˆÓ ÙÔ˘ Illinoi˜ ÙˆÓ ∏¶∞ (™¯‹Ì· 2). ™Â ·˘ÙfiÓ ÙÔÓ ÏÔÁfiÙ˘Ô ÛËÌÂÈÒÓÔÓÙ·È Ì ̷‡ÚÈÛÌ· ÙˆÓ ·ÓÙ›ÛÙÔȯˆÓ ÙÌËÌ¿ÙˆÓ, Ï‹Úˆ˜ ‹ Ì ÛÙÈÎÙ¤˜ ÁÚ·Ì̤˜ (·Ó ÚfiÎÂÈÙ·È ÁÈ· ·ÙÂÏ‹ ‹ ˘Ô‚ÏÂÓÓÔÁfiÓÈ· Û¯ÈÛÙ›·), Ù· ÎÏÈÓÈο Â˘Ú‹Ì·Ù·. ¶ÚÔ·ÙÂÈ, ¤ÙÛÈ, Ì›· ÁÚ‹ÁÔÚË Î·È Ï‹Ú˘ ÂÈÎfiÓ· Ù˘ ÂÎÙ¿Ûˆ˜ ÙÔ˘ Û˘ÁÎÂÎÚÈ̤ÓÔ˘, ÁÈ· οı ·ÛıÂÓ‹, ÚÔ‚Ï‹Ì·ÙÔ˜. ∂ÈÙÒÛÂȘ OÈ ÂÈÙÒÛÂȘ ·fi ÙȘ ‰È¿ÊÔÚ˜ Û¯ÈÛٛ˜ ÂÍ·ÚÙÒÓÙ·È ·fi ÙËÓ ÂÓÙfiÈÛË Î·È ÙË ‚·Ú‡ÙËÙ· Ù˘ ηٿÛÙ·Û˘. ™˘Ó‹ıˆ˜ ·ÊÔÚÔ‡Ó ÛÙË ‰˘Ó·ÙfiÙËÙ· ÔÌ·Ï‹˜ ‰È·ÙÚÔÊ‹˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜, ÛÙËÓ ·ÎÔ‹, ÛÙËÓ ÔÌÈÏ›·, ÛÙËÓ ·ÈÛıËÙÈ΋ ÙÔ˘ ÚÔÛÒÔ˘ Î·È ÛÙË ‰È·ÌfiÚʈÛË Ù˘ Ô‰ÔÓÙÈ΋˜ Û‡ÁÎÏÂÈÛ˘, ‰ÈfiÙÈ ÂÎÙfi˜ ÙˆÓ ÛÎÂÏÂÙÈÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ, ÌÔÚ› Ó· Û˘Ó˘¿Ú¯Ô˘Ó ‰È·Ù·Ú·¯¤˜ ÛÙÔÓ ·ÚÈıÌfi, ÙÔ Ì¤ÁÂıÔ˜ Î·È ÙÔ Û¯‹Ì· ÙˆÓ ‰ÔÓÙÈÒÓ (∂ÈÎfiÓ· 2). À¿Ú¯Ô˘Ó ¿ÓÙˆ˜ Î·È ÂÈÙÒÛÂȘ Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙËÓ ·ÔÛ·ÛÌ·ÙÈÎfiÙËÙ· Î·È ÙË ¯ˆÚ›˜ ÚÔÁÚ·ÌÌ·ÙÈÛÌfi ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÂÈ̤ÚÔ˘˜ ÚÔ‚ÏËÌ¿ÙˆÓ ÙÔ˘ οı ·ÛıÂÓÔ‡˜, Ë ÔÔ›· ÌÔÚ› Ó· ¤¯ÂÈ ‰˘ÛÌÂÓ‹ ›‰Ú·ÛË ÛÙËÓ ·‡ÍËÛË Î·È ÛÙË ‰È·ÌfiÚʈÛË Ù˘ Û˘ÁÎϛۈ˜, ÚÔÛı¤ÙÔÓÙ·˜ ¤ÙÛÈ ·ÚÓËÙÈΤ˜ È·ÙÚÔÁÂÓ›˜ ·Ú·Ì¤ÙÚÔ˘˜ ÛÙÔ ·Ú¯ÈÎfi Úfi‚ÏËÌ·.
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™¯‹Ì· 1. ∆·ÍÈÓfiÌËÛË ÙˆÓ Û¯ÈÛÙÈÒÓ Ì ‚¿ÛË ÙËÓ ÂÌ‚Ú˘˚΋ ÙÔ˘˜ ÚԤϢÛË.
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∞ ª˘ÎÙ‹Ú·˜ Œ‰·ÊÔ˜ ÚÈÓfi˜ ÛÏÔ˜ º·ÙÓ›Ô
ÀÂÚÒÈ· ÌÔ›Ú· Ê·ÙÓȷ΋˜ ·ÔʇÛˆ˜
∆ÔÌÈÎfi ÙÚ‹Ì· ™ÎÏËÚ‹ ˘ÂÚÒ·
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™Ù·Ê˘Ï‹ ™¯‹Ì· 2. ∏ ÈÔ ÚfiÛÊ·ÙË ÙÚÔÔÔ›ËÛË ÙÔ˘ ÁÚ·Ì̈ÙÔ‡ ÏÔÁfiÙ˘Ô˘ Ù‡Ô˘ "À" ÙÔ˘ Kernahan, fiˆ˜ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ·fi ÙËÓ Illinlois Assoc. of Craniofacial Teams ÙˆÓ ∏¶∞.
∞ÓÙÈÌÂÙÒÈÛË O ÎÏÈÓÈÎfi˜ ÁÈ·ÙÚfi˜ Ú¤ÂÈ Ó· ÁÓˆÚ›˙ÂÈ fiÙÈ Ë ÔÏ˘ÏÔÎfiÙËÙ· Î·È ÔÈ È‰È·ÈÙÂÚfiÙËÙ˜ ÙˆÓ ÚÔ‚ÏËÌ¿ÙˆÓ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ÙÔ ·È‰› Ô˘ ¿Û¯ÂÈ ·fi οÔÈ·˜ ÌÔÚÊ‹˜ Û¯ÈÛÙ›· Â›Ó·È ÔÏϤ˜ Î·È ··ÈÙÔ‡Ó Û˘ÓÂÚÁ·Û›· ·fi ÔÌ¿‰· ÂÈÛÙËÌfiÓˆÓ ‰È·ÊfiÚˆÓ ÂȉÈÎÔًوÓ, ÂÍÂȉÈÎÂ˘Ì¤ÓˆÓ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·˘ÙÒÓ ÙˆÓ ÚÔ‚ÏËÌ¿ÙˆÓ. ∏ ÔÌ¿‰· ·˘Ù‹ ÛÙÂÁ¿˙ÂÙ·È, ηٿ ηÓfiÓ·, Û` ¤Ó· ÂȉÈÎfi ΤÓÙÚÔ Ô˘ ˘¿ÁÂÙ·È Û οÔÈÔ ¡ÔÛÔÎÔÌÂ›Ô ‹ ¶·ÓÂÈÛÙ‹ÌÈÔ Î·È ·Ú·ÎÔÏÔ˘ı› ÙÔ ·È‰› ·fi ÙË Á¤ÓÓËÛË Ì¤¯ÚÈ ÙËÓ ÂÓËÏÈΛˆÛ‹ ÙÔ˘. ∞ÔÙÂÏÂ›Ù·È ‰Â, ηٿ ‰ÈÂıÓ‹ Ú·ÎÙÈ΋, ÂÎÙfi˜ ·fi ÙÔÓ ·È‰›·ÙÚÔ Ô˘ Ú¤ÂÈ Ó· ·Ú·ÎÔÏÔ˘ı› οı ·ÚÙÈÌÂϤ˜ Î·È ÌË ·È‰›, ·fi ÔÚıÔ‰ÔÓÙÈÎfi, Ï·ÛÙÈÎfi ¯ÂÈÚÔ˘ÚÁfi, ˆÙÔÚÈÓÔÏ·Ú˘ÁÁÔÏfiÁÔ, ÏÔÁÔ·ıÔÏfiÁÔ, ÁÓ·ıÔ¯ÂÈÚÔ˘ÚÁfi, ÚÔÛıÂÙÔÏfiÁÔ, ·È‰Ô‰ÔÓÙ›·ÙÚÔ, ÎÔÈÓˆÓÈÎfi ÏÂÈÙÔ˘ÚÁfi Î·È ÁÂÓÂÙÈÛÙ‹. OÈ ÂȉÈÎÔ› ÚÔÁÚ·ÌÌ·Ù›˙Ô˘Ó ·fi ÎÔÈÓÔ‡ ÙÔ ¯ÚÔÓԉȿÁÚ·ÌÌ· ÙˆÓ ‰È·ÊfiÚˆÓ ÂÂÌ‚¿ÛÂˆÓ Ô˘ ··ÈÙÔ‡ÓÙ·È, ¤ÙÛÈ ÒÛÙÂ Ë ÂÈ̤ÚÔ˘˜ ·ÁˆÁ‹ Ô˘ ı· ÂÊ·ÚÌfiÛÂÈ Ô Î¿ı ÂȉÈÎfi˜ Ó· Á›ÓÂÙ·È ÛÙËÓ Î·Ù¿ÏÏËÏË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ Î·È Ó· Û˘Ì‚¿ÏÏÂÈ ÛÙË ‰È¢ÎfiÏ˘ÓÛË Î·È ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ·ÁˆÁ‹˜ Ô˘ ı· ÂÊ·ÚÌfiÛÔ˘Ó Ù· ¿ÏÏ· ̤ÏË Ù˘ ÔÌ¿‰·˜ (2,20). ŒÓ· ˘fi‰ÂÈÁÌ· ‚·ÛÈÎÔ‡ ¯ÚÔÓԉȷÁÚ¿ÌÌ·ÙÔ˜
∂ÈÎfiÓ· 2. ∞ÎÙÈÓÔÁÚ¿ÊËÌ· status (ÕÓˆ) Î·È ÔÚıÔ·ÓÙÔÌÔÁÚ¿ÊËÌ· (∫¿Ùˆ) ÙˆÓ ÁÓ¿ıˆÓ ·È‰ÈÔ‡ Ì ÂÙÂÚfiÏ¢ÚË ‰ÂÍÈ¿ ¯ÂÈÏÂÔÁÓ·ıÔ¸ÂÚˆÈÔÛ¯ÈÛÙ›·. ¢È·ÎÚ›ÓÂÙ·È Û·ÊÒ˜ ÙÔ ÔÛÙÈÎfi ¤ÏÏÂÈÌÌ· Ô˘ ˘¿Ú¯ÂÈ ÛÙËÓ ¿Óˆ ÁÓ¿ıÔ Î·È Ë Û˘ÁÁÂÓ‹˜ ¤ÏÏÂÈ„Ë ÙÔ˘ ¿Óˆ ‰ÂÍÈÔ‡ Ï·Á›Ô˘ ÙÔ̤·.
ÙˆÓ Î˘ÚÈÔÙ¤ÚˆÓ ÂÂÌ‚¿ÛÂˆÓ Ô˘ ··ÈÙÔ‡ÓÙ·È ÁÈ· ÙË Û˘ÓÔÏÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÂÓfi˜ ·È‰ÈÔ‡ Ô˘ ÂÌÊ·Ó›˙ÂÈ Û¯ÈÛÙ›·, ·Ú·Ù›ıÂÙ·È ÛÙÔÓ ¶›Ó·Î· 1. ∞Ó Î·È ÙÔ ¯ÚÔÓԉȿÁÚ·ÌÌ· ·˘Ùfi Â›Ó·È ·Ô‰ÂÎÙfi Î·È ÂÊ·ÚÌfi˙ÂÙ·È ÛÙ· ÂÚÈÛÛfiÙÂÚ· ÂȉÈο ΤÓÙÚ·, ÌÔÚ› Ó· ¤¯ÂÈ ‰È¿ÊÔÚ˜ ·Ú·ÏÏ·Á¤˜. ∂›Ó·È ¢ÓfiËÙÔ fiÙÈ ÔÈ È‰È·ÈÙÂÚfiÙËÙ˜ Ù˘ ·Ó¿Ù˘Í˘ Î·È ÙˆÓ Û˘ÓıËÎÒÓ ‰È·‚›ˆÛ˘ ÙÔ˘ οı ·È‰ÈÔ‡ ÌÔÚ› Ó· ÙÚÔÔÔÈ‹ÛÔ˘Ó ÙÔ ÔÔÈÔ‰‹ÔÙ ‚·ÛÈÎfi ¯ÚÔÓԉȿÁÚ·ÌÌ·, ¿ÓÙ· ÌÂÙ¿ ·fi Û˘ÓÂÓÓfiËÛË Î·È ÎÔÈÓ‹ ·Ô‰Ô¯‹ ÙˆÓ ÌÂÏÒÓ Ù˘ ÔÌ¿‰·˜ ÙˆÓ ÂȉÈÎÒÓ Ô˘ ·Ú·ÎÔÏÔ˘ı› ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ·È‰›. ∏ ÂÓÂÚÁ‹ Û˘Ì‚ÔÏ‹ ÙÔ˘ οı ̤ÏÔ˘˜ Ù˘ ÔÌ¿‰·˜ ηχÙÂÈ ÔÚÈṲ̂ÓË ¯ÚÔÓÈ΋ ‰È¿ÚÎÂÈ·, Ë ÔÔ›· ÂÓ·ÚÌÔÓ›˙ÂÙ·È Ì ÔÚÈṲ̂Ó˜ Ê¿ÛÂȘ Ù˘ ËÏÈΛ·˜ ÙÔ˘ ·È‰ÈÔ‡, fiˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ ¶›Ó·Î· 2. ∏ Û˘ÓÂÚÁ·ÙÈ΋ ·˘Ù‹ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÂÈ̤ÚÔ˘˜ ÚÔ‚ÏËÌ¿ÙˆÓ ¤¯ÂÈ È‰È·ÈÙ¤Úˆ˜ ÈηÓÔÔÈËÙÈο ·ÔÙÂϤÛÌ·Ù· Û fi,ÙÈ ·ÊÔÚ¿ ÙËÓ ·ÈÛıËÙÈ΋ Î·È ÙË ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ (∂ÈÎfiÓ· 3), ·ÏÏ¿ Î·È Û fi,ÙÈ ·ÊÔÚ¿ ÙËÓ ·Ô‰Ô¯‹ Î·È ¤ÓÙ·Í‹ ÙÔ˘˜ ÛÙËÓ ÎÔÈÓˆÓ›· ÙˆÓ Û˘ÓÔÌËÏ›ÎˆÓ ÙÔ˘˜. ∏ ‰˘Ó·ÙfiÙËÙ· ·˘Ù‹ ¤¯ÂÈ, ›Û˘, Ôχ ÛËÌ·ÓÙÈΤ˜ „˘¯ÔÏÔÁÈΤ˜
445
¶·È‰È·ÙÚÈ΋ 2001;64:441-447
Paediatriki 2001;64:441-477
¶›Ó·Î·˜ 1. Àfi‰ÂÈÁÌ· ¯ÚÔÓԉȷÁÚ¿ÌÌ·ÙÔ˜ ÙˆÓ ¯ÂÈÚÔ˘ÚÁÈÎÒÓ ÂÂÌ‚¿ÛÂˆÓ Î·È ÙˆÓ ÔÚıÔ‰ÔÓÙÈÎÒÓ ˘ËÚÂÛÈÒÓ, fiˆ˜ ÂÊ·ÚÌfi˙ÂÙ·È Û ÔÏÏ¿ ·fi Ù· ÔÚÁ·ÓˆÌ¤Ó· ΤÓÙÚ· ∞ÓÙÈÌÂÙÒÈÛ˘ ™¯ÈÛÙÈÒÓ. ∏ÏÈΛ· 0-3 ÌËÓÒÓ Œˆ˜ 2 ÂÙÒÓ 2-4 ÂÙÒÓ 6 ÂÙÒÓ 7-9 ÂÙÒÓ 9-11 ÂÙÒÓ 10-12 ÂÙÒÓ 12-14 ÂÙÒÓ ªÂÙ¿ Ù· 16 ¯ÚfiÓÈ·
ÃÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ ñ ™˘Ó¤ÓˆÛË ¯Â›ÏÔ˘˜ ñ ™˘ÚÚ·Ê‹ ¯Â›ÏÔ˘˜ ñ ¢ÈfiÚıˆÛË ˘ÂÚÒ·˜ ñ ¢ÈÔÚıˆÙÈΤ˜ ÂÂÌ‚¿ÛÂȘ
OÚıÔ‰ÔÓÙÈ΋ ÊÚÔÓÙ›‰· ñ ÀÂÚÒÈ· Ͽη ñ ¶·Ú·ÎÔÏÔ‡ıËÛË Î·È ·ÏÏ·Á‹ Ͽη˜ ñ ŒÓ·ÚÍË ÔÚıÔ‰ÔÓÙÈ΋˜ ıÂڷ›·˜ ñ ™˘ÁÎÚ¿ÙËÛË Î·È ·Ú·ÎÔÏÔ‡ıËÛË ñ ¶ÚÔÂÙÔÈÌ·Û›· ·fi ÙÔÓ ÔÚıÔ‰ÔÓÙÈÎfi ÁÈ· Ó· ÙÔÔıÂÙËı› ÙÔ ÔÛÙÈÎfi ÌfiÛ¯Â˘Ì·
ñ OÛÙÈÎfi ÌfiÛ¯Â˘Ì· ñ ™˘ÌÏ‹ÚˆÛË ÔÚıÔ‰ÔÓÙÈ΋˜ ıÂڷ›·˜ ñ ∞Ó ¯ÚÂÈ¿˙ÂÙ·È, Á›ÓÂÙ·È ÔÚıÔ‰ÔÓÙÈ΋ ÚÔÂÙÔÈÌ·Û›·, Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛËÎ·È ÚÔÛıÂÙÈ΋ ·ÔηٿÛÙ·ÛË
ñ ∞ÍÈÔÏfiÁËÛË ÁÈ· ·Ó¿ÁÎË Ù˘¯fiÓ ‰ÈÔÚıˆÙÈÎÒÓ ‹ Î·È ÁÓ·ıÔ¯ÂÈÚÔ˘ÚÁÈÎÒÓ ÂÂÌ‚¿ÛˆÓ
¶›Ó·Î·˜ 2. ™˘ÓÔÙÈ΋ Û¯ËÌ·ÙÈ΋ ·ÂÈÎfiÓÈÛË ÙˆÓ ˘ËÚÂÛÈÒÓ Ô˘ ÚÔÛʤÚÔ˘Ó ÔÈ ÂÈ̤ÚÔ˘˜ ÂȉÈÎÔ› Ù˘ ÔÌ¿‰·˜, ·fi ÙË Á¤ÓÓËÛË Ì¤¯ÚÈ ÙËÓ ÂÓËÏÈΛˆÛË ÂÓfi˜ ·ÙfiÌÔ˘ Ô˘ ÂÌÊ·Ó›˙ÂÈ Û¯ÈÛÙ›·. O ÚfiÏÔ˜ ÙÔ˘ ·È‰È¿ÙÚÔ˘ Â›Ó·È ·˘ÙÔÓÔ‹ÙÔ˜. ∂ȉÈÎÔ› ÂÈÛÙ‹ÌÔÓ˜ Ù˘ ÔÌ¿‰·˜ OÚıÔ‰ÔÓÙÈÎfi˜ ¶Ï·ÛÙÈÎfi˜ ¯ÂÈÚÔ˘ÚÁfi˜ øÙÔÚÈÓÔÏ·Ú˘ÁÁÔÏfiÁÔ˜ ∫ÔÈÓˆÓÈ΋ ÏÂÈÙÔ˘ÚÁfi˜ °ÂÓÂÙÈÛÙ‹˜ §ÔÁÔ·ıÔÏfiÁÔ˜ °Ó·ıÔ¯ÂÈÚÔ˘ÚÁfi˜ ¶ÚÔÛıÂÙÔÏfiÁÔ˜ ¶·È‰Ô‰ÔÓÙ›·ÙÚÔ˜
0-1
1-2
2-4
∏ÏÈΛ· (Û ¯ÚfiÓÈ·) 4-6 6-8 8-12
12-14
14-16
18+
∂ÈÎfiÓ· 3. ∂Ó‰ÔÛÙÔÌ·ÙÈΤ˜ ʈÙÔÁڷʛ˜ ·È‰ÈÔ‡ Ì ·ÌʛϢÚË ¯ÂÈÏÂÔÁÓ·ıÔ¸ÂÚˆ˚ÔÛ¯ÈÛÙ›· ÚÈÓ Î·È ÌÂÙ¿ ÙË Û˘ÓÂÚÁ·ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË ·fi ·È‰Ô‰ÔÓÙ›·ÙÚÔ, ÁÓ·ıÔ¯ÂÈÚÔ˘ÚÁfi (ÁÈ· ÙÔÔı¤ÙËÛË ÔÛÙÈÎÒÓ ÌÔÛ¯Â˘Ì¿ÙˆÓ), ÔÚıÔ‰ÔÓÙÈÎfi Î·È ÚÔÛıÂÙÔÏfiÁÔ.
446
¶·È‰È·ÙÚÈ΋ 2001;64:441-447
ÚÔÂÎÙ¿ÛÂȘ, ÙfiÛÔ ÛÙ· ›‰È· Ù· ·È‰È¿, fiÛÔ Î·È ÛÙÔ˘˜ ÁÔÓ›˜ Î·È ÛÙÔ Û˘ÓÔÏÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÙÔ˘˜ ÂÚÈ‚¿ÏÏÔÓ. ∞ÓÙÈı¤Ùˆ˜, fiÙ·Ó ÙÔ ·È‰› Ô˘ ¿Û¯ÂÈ ·fi Û¯ÈÛÙ›· ·ÓÙÈÌÂÙˆ›˙ÂÙ·È ·ÔÛ·ÛÌ·ÙÈο Î·È ÌÂÌÔӈ̤ӷ ·fi οÔÈÔÓ ‹ οÔÈÔ˘˜ ·fi ÙÔ˘˜ ÂȉÈÎÔ‡˜ Ô˘ ··ÈÙ› Ë ÂÚ›ÙˆÛ‹ ÙÔ˘, ¯ˆÚ›˜ ÙÔ Û˘ÓÙÔÓÈÛÌfi Î·È ÙÔ ÌÂÏÂÙË̤ÓÔ ÚÔÁÚ·ÌÌ·ÙÈÛÌfi Ô˘ ÈÛ¯‡ÂÈ ÛÙ· ·ÓÙ›ÛÙÔȯ· ΤÓÙÚ·, Ù· ·ÔÙÂϤÛÌ·Ù· ·¤¯Ô˘Ó Ôχ ·fi ÙËÓ ÈηÓÔÔ›ËÛË ÙˆÓ ··Ú·›ÙËÙˆÓ ·ÈÛıËÙÈÎÒÓ Î·È ÏÂÈÙÔ˘ÚÁÈÎÒÓ ÎÚÈÙËÚ›ˆÓ (18).
Paediatriki 2001;64:441-477
14.
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16.
µÈ‚ÏÈÔÁÚ·Ê›· 1. Abbot BD, Probst MR, Perdew GH, Buckalew AR. Ah receptor, ARNT, glucocorticoid receptor, EGF receptor, EGF, TGF-a, TGF-‚1, TGF-‚2 and TGF-‚3 expression in human embryonic palate, and effects of 2,3,7,8tetrachlorodibenzo-p-dioxin (TCDD). Teratology 1998;58:30-34. 2. µÈ¤ÚÔ˘ ∞ª, ¶··ÁÈ·ÓÓÔ‡ÏË §, ™˘ÚÔÔ‡ÏÔ˘ ª¡. ∏ ·ÓÙÈÌÂÙÒÈÛË Ô˘ ·È‰ÈÔ‡ Ì ۯÈÛÙ›· ·fi ÙË Á¤ÓÓËÛË Ì¤¯ÚÈ Î·È ÙË Û˘ÌÏ‹ÚˆÛË ÙÔ˘ ÓÂÔÁÈÏÔ‡ ÊÚ·ÁÌÔ‡. O‰ÔÓÙÔÛÙÔÌ·ÙÔÏÔÁÈ΋ ¶ÚfiÔ‰Ô˜ 1989;43:193-202. 3. Brunet CI, Sharpe PM, Ferguson MW. Inhibition of TGF-‚3 (but not TGF-‚1 or TGF-‚2) activity prevents normal mouse embryonic palate fusion. Int J Dev Biol 1995;39:345-355. 4. Dixon MJ, Ferguson MWJ. The effect of EGF, TGF-a and ‚ and PDGF on murine palatal shelves in organ culture. Arch Oral Biol 1992;37:395-410. 5. Ferguson MWJ. Palate development. Development 1998;103(Suppl):41-60. 6. Heine VI, Munoz EF, Flanders KC, Ellingsworth LR, Lan HYU, Thompson NL. The role of TGF-‚ in the development of the mouse embryo. J Cell Biol 1987;105:2861-2867. 7. Izadnegadhar MF, Rathanaswami P, Shah RM. Effects of EGF and TGF-‚1 on c-myc gene expression and DNA synthesis in enbryonic hamster palate mesenchymal cells. Anat Rec 1999;254: 453-464. 8. Jaskoll T, Choy HA, Chen H, Melnick M. Developmental expression and CORT- regulation of TGF-‚ and EGF receptor mRNA during mouse palatal morphogenesis: correlation between CORT-induced cleft palate and TGF‚2 mƒ¡∞ expression. Teratology 1996;54:34-44. 9. Johnston MC, Bronsky PT. Prenatal craniofacial development: new insights on normal and abnormal mechanisms. Crit Rev Oral Biol Med 1995;64:368-422. 10. Kernahan D, Stark R. A new classification for cleft lip and cleft palate. Plast Reconstr Surg 1958;22:435-442. 11. Kernahan D. The stripped Y -a symbolc classification for cleft lip and cleft palate. Plast Reconstr Surg 1971;35:469-471. 12. ¡·Û›Î· ª™. ™‡Á¯ÚÔÓ˜ ·fi„ÂȘ ÁÈ· ÙËÓ ·ÈÙÈÔÏÔÁ›· ÙˆÓ Û¯ÈÛÙÈÒÓ. O‰ÔÓÙÔÛÙÔÌ·ÙÔÏÔÁÈ΋ ¶ÚfiÔ‰Ô˜ 1987;41:169-173. 13. ¡·Û›Î· ª™. ∂ȉËÌÔÏÔÁÈ΋ ªÂϤÙË ÙˆÓ ™¯ÈÛÙÈÒÓ ÙÔ˘ Û-
17.
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24.
ÏÔ˘˜ ± ÀÂÚÒ·˜ ÛÙÔÓ ¶ÏËı˘ÛÌfi Ù˘ ∂ÏÏ¿‰·˜, ÛÙË ‰ÂηÂÙ›· ÙÔ˘ 1980-1990 [¢È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹]. O‰ÔÓÙÈ·ÙÚÈÎfi ∆Ì‹Ì· ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ; 1997. Ponitz PV, Spyropoulos MN. The dental occlusion in treated and non-treated cleft lip and palate. Eur J Orthod 1979;1:181-187. Pratt RM, Kim CS, Groove RL. Role of glucocorticoids and EGF in normal and abnormal palatal development. Curr Top Dev Biol 1984;19:81-101. Sharpe PM, Foreman D, Carrete MJ, Schor SL, Ferguson MWJ. The effects of TGF-‚1 on protein production by mouse enbryonic palate mesenchymal cells in the presence or absence of serum. Arch Oral Biol 1992;37:39-48. Sporn MB, Roberts AB, Wakefield LM, De Crombrughe B. Some recent advances in the chemistry and biology of TGF. J Cell Biol 1985;105:1039-1045. Spyropoulos MN, Linder-Aronson S. Evaluation of facial features in two groups of cleft lip and palate patients receiving centralized and non-centralized treatment regimes. Eur J Orthod 1997;19:411-422. ™˘ÚÔÔ‡ÏÔ˘ ª¡. ¶·Ú·ÙËÚ‹ÛÂȘ ·fi ÙËÓ ¿Ô„Ë Ù˘ OÚıÔ‰ÔÓÙÈ΋˜ ÁÈ· ÙË ÛËÌ·Û›· ÙÔ˘ ¯ÚfiÓÔ˘ ¯ÂÈÚÔ˘ÚÁÈ΋˜ ÂÂÌ‚¿Ûˆ˜ ÛÙȘ Û¯ÈÛٛ˜. ∂ÏÏ ™ÙÔÌ/ÁÈο ÃÚÔÓ 1977;21:31-35. ™˘ÚÔÔ‡ÏÔ˘ ª¡, §¿ÛÔ˜ Ã, •ˆÊ‡ÏÏ˘ ∞, ∫ˆÓÛÙÈ¿ÓÙÔ˘ ∫. ¢È·Ù·Ú·¯¤˜ ÔÌÈÏ›·˜ Û ·È‰È¿ Ì ۯÈÛٛ˜. O‰ÔÓÙÔÛÙÔÌ·ÙÔÏÔÁÈ΋ ¶ÚfiÔ‰Ô˜ 1995;49:170-179. ™˘ÚÔÔ‡ÏÔ˘ ª¡. µ·ÛÈΤ˜ ∞Ú¯¤˜ OÚıÔ‰ÔÓÙÈ΋˜. ¶ÚÔ‚Ï‹Ì·Ù· ÛÙËÓ ¶ÂÚ›Ô‰Ô ÙˆÓ ªÈÎÙÒÓ ºÚ·ÁÌÒÓ. ∂ΉfiÛÂȘ µ∏∆∞: ∞ı‹Ó·; 2000. Sun D, Vandeburgg CR, Odierna GS, Hay ED. TGF-‚3 promotes transformation of chicken palate medial edge epithelium to mesenchyme in vitro. Development 1998;125:95-110. Taya Y, O’ Kane S, Ferguson MWJ. Pathogenesis of cleft palate in TGF-‚3 knockout mice. Development 1999;126:3869-3879. Tyler MS, Pratt RM. Effect of EGF on secondary palatal epithelium in vitro: Tissue isolation and recombination studies. J Embryol Exp Morphol 1980;58:93-106.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 12-01-2001 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 26-06-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∫·ıËÁ‹ÙÚÈ· ª.¡. ™˘ÚÔÔ‡ÏÔ˘ ∂ÚÁ·ÛÙ‹ÚÈÔ OÚıÔ‰ÔÓÙÈ΋˜, O‰ÔÓÙÈ·ÙÚÈ΋ ™¯ÔÏ‹ £Ë‚ÒÓ 2, 115 27, °Ô˘‰›, ∞ı‹Ó·
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Paediatriki 2001;64:448-459
CURRENT ISSUES
∏ ˘¤ÚÙ·ÛË ÛÙËÓ ·È‰È΋ ËÏÈΛ· ∞. ∑ÂÚ‚Ô˘‰¿ÎË, ∫. ∆ÛÔ˘Ì¿Î·˜
Hypertension in childhood A. Zervoudaki, K. Tsoumakas
¶ÂÚ›ÏË„Ë: ∏ ˘¤ÚÙ·ÛË ·Ú¯›˙ÂÈ ·fi ÙËÓ ·È‰È΋ ËÏÈΛ·, ¯ˆÚ›˜ ·Ú¤Ì‚·ÛË Ù›ÓÂÈ Ó· Û˘Ó¯›˙ÂÙ·È ÛÙËÓ ÂÓ‹ÏÈÎÔ ˙ˆ‹ Î·È ·ÔÙÂÏ› ÙÔÓ ÛËÌ·ÓÙÈÎfiÙÂÚÔ ÚԉȷıÂÛÈÎfi ·Ú¿ÁÔÓÙ· Ù˘ ηډȷÁÁÂȷ΋˜ ÓfiÛÔ˘. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È Ë ıÂڷ¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Û˘Ì‚¿ÏÏÔ˘Ó ÛËÌ·ÓÙÈο ÛÙËÓ ÂÍ·ÛÊ¿ÏÈÛË Î·Ï‡ÙÂÚ˘ ÔÈfiÙËÙ·˜ ˙ˆ‹˜. ∆Ô ÚÒÙÔ ‚‹Ì· ÛÙË ‰È¿ÁÓˆÛË Â›Ó·È Ë Û˘ÛÙËÌ·ÙÈ΋ ̤ÙÚËÛË Ù˘ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ Û οı ·È‰›. ∏ Û˘Ó¯‹˜ ÊÔÚËÙ‹ ηٷÁÚ·Ê‹ Ù˘ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È Ï¤ÔÓ Î·È ÛÙ· ·È‰È¿ Î·È Û˘Ì‚¿ÏÏÂÈ ÛËÌ·ÓÙÈο ÛÙË ‰È¿ÁÓˆÛË, ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Î·È ÛÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ıÂڷ›·˜. ™ÙÔ ¿ÚıÚÔ ·˘Ùfi Á›ÓÂÙ·È ·Ó·ÛÎfiËÛË Ù˘ ‰ÈÂıÓÔ‡˜ ‚È‚ÏÈÔÁÚ·Ê›·˜, fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ·ÈÙÈÔÏÔÁ›·, ÙË ‰È¿ÁÓˆÛË, ÙË ıÂڷ›· Î·È Ù· ̤ÙÚ· ÚfiÏ˄˘.
Abstract: Hypertension starts in childhood and without medical intervention it tends to track into adulthood and constitutes the most significant factor predisposing to cardiovascular disease. Prompt diagnosis and treatment are instrumental in securing a better quality of life. The first diagnostic step is the systematic measurement of the blood pressure in all children on a yearly basis. Continuous ambulatory blood pressure monitoring has become very useful for the diagnosis, differential diagnosis, treatment and follow up of hypertension in children. This article reviews the literature regarding etiology, diagnosis, treatment and measures of prevention of hypertension.
§¤ÍÂȘ ÎÏÂȉȿ: ˘¤ÚÙ·ÛË, ·ÚÙËÚȷ΋ ›ÂÛË, ·È‰È΋ ËÏÈΛ·.
Key words: hypertension, blood pressure, children.
∂ÈÛ·ÁˆÁ‹ ∏ ˘¤ÚÙ·ÛË ÌÔÚ› Ó· Û˘Ó·ÓÙ¿Ù·È Û ÔÛÔÛÙfi 1% ÙˆÓ ·È‰ÈÒÓ Ô˘ ÂÍÂÙ¿˙ÂÈ ¤Ó·˜ ·È‰›·ÙÚÔ˜. Èڛ˜ ·Ú¤Ì‚·ÛË, Ë ˘¤ÚÙ·ÛË Ù›ÓÂÈ Ó· Û˘Ó¯›˙ÂÙ·È ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (1-5). ¶·È‰È¿ Ì ÂÈ̤ÓÔ˘Û· ·ÚÙËÚȷ΋ ›ÂÛË (∞¶) ÌÂÁ·Ï‡ÙÂÚË Ù˘ 90˘ ∂£, ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÂÈÙÒÛÂȘ ÛÙ· fiÚÁ·Ó· ÛÙfi¯Ô˘˜, fiˆ˜ ·‡ÍËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ Ù˘ ηډȿ˜, ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ·ÏÏ·Á¤˜ ÛÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ·, ËÏÂÎÙÚÔηډÈÔÁÚ·ÊÈΤ˜ ·ÏÏÔÈÒÛÂȘ, ηıÒ˜ Î·È ·ıÔÏÔÁÔ·Ó·ÙÔÌÈΤ˜ ·ÏÏÔÈÒÛÂȘ ÛÙ· ÛÙÂÊ·ÓÈ·›·, Ù· ÓÂÊÚÈο Î·È Ù· ·ÁÁ›· ÙÔ˘ ·ÌÊÈ‚ÏËÛÙÚÔÂȉԇ˜ (6,7). ∏ ÔÚ›· Ù˘ ηډȷÁÁÂȷ΋˜ ÓfiÛÔ˘ Â›Ó·È ÛȈËÚ‹ Î·È ·‰˘ÛÒËÙË, ÌÂ Û˘Ó¤ÂÈ· ·Ú¯fiÌÂÓ˜ ·ÚÙË-
ÚÈÔÛÎÏËÚ˘ÓÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ, ÔÈ Ôԛ˜ ·Ú·ÙËÚÔ‡ÓÙ·È Û˘¯Ó¿ Û ÂÊ‹‚Ô˘˜, ·ÏÏ¿ Î·È Û ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜ (8-10). ∆˘¯·›· ·Ó‡ÚÂÛË ·˘ÍË̤Ó˘ ∞¶ ÌÔÚ› Ó· Â›Ó·È ·fiÚÚÔÈ· ÚˆÙÔ·ıÔ‡˜ ˘¤ÚÙ·Û˘ ‹ Ó· ·ÔÙÂÏ› ÙËÓ ÚÒÙË ÂΉ‹ÏˆÛË ˘ÔΛÌÂÓ˘ ÓfiÛÔ˘, fiˆ˜ Ë ·ÚÂÁ¯˘Ì·ÙÈ΋ ‹ Ë ·ÁÁÂȷ΋ ÓÂÊÚÈ΋ ‚Ï¿‚Ë, Ë Î·Ú‰ÈÔ¿ıÂÈ· Î·È Ë ÂÓ‰ÔÎÚÈÓÔ¿ıÂÈ·.
¶·È‰È·ÙÚÈÎfi ∆Ì‹Ì· ¶ÂÚÈÊÂÚÂÈ·Îfi °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ∫∞∆ ∞ÙÙÈ΋˜
448
OÚÈÛÌfi˜ Î·È ÁÂÓÈÎfiÙËÙ˜ º˘ÛÈÔÏÔÁÈ΋ ∞¶ Û ‚Ú¤ÊË, ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ÔÚ›˙ÂÙ·È Ë Û˘ÛÙÔÏÈ΋ ‹ Ë ‰È·ÛÙÔÏÈ΋ ›ÂÛË Ô˘ ‚Ú›ÛÎÂÙ·È Î¿Ùˆ ·fi ÙËÓ 90Ë ∂£ ÁÈ· ÙËÓ ËÏÈΛ·, ÙÔ ‡„Ô˜ Î·È ÙÔ Ê‡ÏÔ. À„ËÏ‹ “Ê˘ÛÈÔÏÔÁÈ΋” ∞¶ Pediatric Division Peripheral General Hospital KAT of Attiki, Athens
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
¯·Ú·ÎÙËÚ›˙ÂÙ·È Ë Û˘ÛÙÔÏÈ΋ ‹ Ë ‰È·ÛÙÔÏÈ΋ ›ÂÛË ÌÂٷ͇ Ù˘ 90˘-95˘ ∂£. À¤ÚÙ·ÛË ÔÚ›˙ÂÙ·È Ë Û˘ÛÙÔÏÈ΋ ‹ Ë ‰È·ÛÙÔÏÈ΋ ∞¶, fiÙ·Ó ‚Ú›ÛÎÂÙ·È ÌÂٷ͇ 95˘-99˘ ∂£ Î·È ¤¯ÂÈ ÂȂ‚·Èˆı› Û 3 ‹ ÂÚÈÛÛfiÙÂÚ˜ ÌÂÙÚ‹ÛÂȘ Ô˘ ¤ÁÈÓ·Ó Û ‰È·ÊÔÚÂÙÈο ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù·. ∆¤ÏÔ˜, ÛÔ‚·Ú‹ ˘¤ÚÙ·ÛË ‰È·ÈÛÙÒÓÂÙ·È, fiÙ·Ó Ë Û˘ÛÙÔÏÈ΋ ‹ Î·È Ë ‰È·ÛÙÔÏÈ΋ ∞¶ Â›Ó·È ›ÛË ‹ ÌÂÁ·Ï‡ÙÂÚË ·fi ÙËÓ 99Ë ∂£ (11-13). O ÔÚÈÛÌfi˜ Ù˘ ˘¤ÚÙ·Û˘ ‚·Û›˙ÂÙ·È Û ÂȉËÌÈÔÏÔÁÈο Î·È ÛÙ·ÙÈÛÙÈο ‰Â‰Ô̤ӷ ·fi ÂÚÈÛÙ·ÛȷΤ˜ ÌÂÙÚ‹ÛÂȘ Î·È ‰ÂÓ Ï·Ì‚¿ÓÔÓÙ·È ˘’ fi„ÈÓ ÔÈ Ù˘¯fiÓ ÂÈÙÒÛÂȘ Ù˘ ˘¤ÚÙ·Û˘ Û fiÚÁ·Ó· ÛÙfi¯Ô˘˜, fiˆ˜ Á›ÓÂÙ·È ÛÙÔ˘˜ ÂÓËÏ›ÎÔ˘˜ (7). °È· ÙÔÓ ·ÎÚÈ‚‹, ÏÔÈfiÓ, ηıÔÚÈÛÌfi ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ Ù˘ ∞¶ ÛÙ· ·È‰È¿ ¯ÚÂÈ¿˙ÔÓÙ·È ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜, ÒÛÙ ӷ Á›ÓÂÈ ÁÓˆÛÙfi Û ÔÈ· ›‰· ∞¶ ˘¿Ú¯Ô˘Ó ÂÈÙÒÛÂȘ ÛÙ· fiÚÁ·Ó· ÛÙfi¯Ô˘˜. O ‚·ıÌfi˜ Ù˘ ‚ÈÔÏÔÁÈ΋˜ ˆÚÈÌfiÙËÙ·˜ ÂÓfi˜ ·È‰ÈÔ‡ Â›Ó·È ÛËÌ·ÓÙÈÎfi˜, ‰ÈfiÙÈ Ë ∞¶ ·˘Í¿ÓÂÙ·È fiÛÔ ÌÂÁ·ÏÒÓÂÈ ÙÔ ·È‰› (14). ∏ ¯ÚÔÓÔÏÔÁÈ΋ ËÏÈΛ· ‰ÂÓ Â›Ó·È Î·ıÔÚÈÛÙÈ΋. ∆Ô ‡„Ô˜ ÙÔ˘ ·È‰ÈÔ‡, fï˜, Ú¤ÂÈ ¿ÓÙ· Ó· Ï·Ì‚¿ÓÂÙ·È ˘’ fi„ÈÓ, ‰ÈfiÙÈ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÙË ‚ÈÔÏÔÁÈ΋ ˆÚÈÌfiÙËÙ· ·ÏÏ¿ Î·È Ì ÙËÓ ∞¶, ·ÓÂÍ¿ÚÙËÙ· Ù˘ ¯ÚÔÓÔÏÔÁÈ΋˜ ËÏÈΛ·˜ (12). ŒÙÛÈ, ¤Ó· ·È‰› Ì ∞¶ ÌÂٷ͇ 90˘ Î·È 95˘ ∂£ ÙÔ˘ ÔÔ›Ô˘ ÙÔ ‡„Ô˜ Â›Ó·È ¿Óˆ ·fi ÙËÓ 90Ë ∂£, ÌÔÚ› Ó· ÌËÓ ¤¯ÂÈ ˘¤ÚÙ·ÛË, ·Ó Ë ∞¶ ·Ó·¯ı› ÛÙÔ ‡„Ô˜ ÙÔ˘. ∂Ô̤ӈ˜, Ï·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ ÙÔ ‡„Ô˜, ˘„ËÏ¿ ·È‰È¿ ‰ÂÓ ı· ¯·Ú·ÎÙËÚÈÛÙÔ‡Ó ˆ˜ ˘ÂÚÙ·ÛÈο - Ì fiÛ· ·˘Ùfi Û˘Ó¿ÁÂÙ·È - Î·È Ù·˘Ùfi¯ÚÔÓ· ‰ÂÓ ı· ‰È·Ê‡ÁÔ˘Ó Ù˘ ‰È¿ÁÓˆÛ˘ ˘ÂÚÙ·ÛÈο ·È‰È¿ ¯·ÌËÏÔ‡ ‡„Ô˘˜ (3,14). OÈ ÙÈ̤˜ Ù˘ ∞¶ ÁÈ· ÙËÓ 90Ë Î·È 95Ë ∂£ Û ·È‰È¿ Ù˘ ›‰È·˜ ËÏÈΛ·˜ Î·È ÙÔ˘ ›‰ÈÔ˘ ʇÏÔ˘, ·ÏÏ¿ ‰È·ÊÔÚÂÙÈÎÔ‡ ‡„Ô˘˜, ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ Ô˘ Î˘Ì·›ÓÔÓÙ·È ÛÙ· ·ÁfiÚÈ· ·fi 8-9 mmHg ÁÈ· ÙË Û˘ÛÙÔÏÈ΋ Î·È 4-5 mmHg ÁÈ· ÙË ‰È·ÛÙÔÏÈ΋ Î·È ·fi 6-7 mmHg Î·È 3-4 mmHg ÛÙ· ÎÔÚ›ÙÛÈ·, ÁÈ· ÙË Û˘ÛÙÔÏÈ΋ Î·È ÙË ‰È·ÛÙÔÏÈ΋ ·ÓÙ›ÛÙÔȯ·. ∆Ô ‚¿ÚÔ˜ ÙÔ˘ ·È‰ÈÔ‡, ·Ú’ fiÏÔ Ô˘ Û˘Û¯ÂÙ›˙ÂÙ·È ÛËÌ·ÓÙÈο Ì ÙËÓ ˘¤ÚÙ·ÛË, ‰ÂÓ ·ÔÙÂÏ› ηٿÏÏËÏÔ ‰Â›ÎÙË, ‰ÈfiÙÈ ·ÊÂÓfi˜ ‰ÂÓ ·ÓÙ·Ó·ÎÏ¿ ÙË ‚ÈÔÏÔÁÈ΋ ˆÚ›Ì·ÓÛË ÙÔ˘ ·È‰ÈÔ‡ Î·È ·ÊÂÙ¤ÚÔ˘ Ë ∞¶ ÂËÚ¿˙ÂÙ·È ·fi ÙÔÓ ˘¿Ú¯ÔÓÙ· ‚·ıÌfi ·¯˘Û·ÚΛ·˜. ∆¤ÏÔ˜, fiÛÔÓ ·ÊÔÚ¿ ÛÙÔ ·ÓÒÙ·ÙÔ fiÚÈÔ Ù˘ ‰È·ÛÙÔÏÈ΋˜ ›ÂÛ˘ Û ˘„ËÏÔ‡˜ 17¯ÚÔÓÔ˘˜ ÂÊ‹‚Ô˘˜, ı· Ú¤ÂÈ Ó· ÛËÌÂȈı› fiÙÈ Ë ‰ÈÂıÓÒ˜ ·Ô‰ÂÎÙ‹ ÙÈÌ‹ ÙˆÓ 90 mmHg ı· Ú¤ÂÈ Ó· ÈÛ¯‡ÂÈ Î·È Û ·˘ÙÔ‡˜. TÈ̤˜ Û˘ÛÙÔÏÈ΋˜ Î·È ‰È·ÛÙÔÏÈ΋˜ ∞¶ ÁÈ· ·ÁfiÚÈ· Î·È ÎÔÚ›ÙÛÈ· ÌÂÙ¿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜, ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ·, ÙÔ Ê‡ÏÔ Î·È ÙÔ ‡„Ô˜, Ê·›ÓÔÓÙ·È ÛÙÔ˘˜ ¶›Ó·Î˜ 1 Î·È 2.
Paediatriki 2001;64:448-459
¶·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙËÓ ∞¶ º˘ÛÈÔÏÔÁÈΤ˜ ‰È·Î˘Ì¿ÓÛÂȘ Ù˘ ∞¶ ·Ú·ÙËÚÔ‡ÓÙ·È Û οı ¿ÙÔÌÔ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ËÌÂÚÔÓ˘ÎÙ›Ô˘ (3,4,15). ¢‡Ô ÔÌ¿‰Â˜ ·Ú·ÁfiÓÙˆÓ ÌÔÚÔ‡Ó Ó· ÂËÚ¿ÛÔ˘Ó ÙËÓ ∞¶, ÔÈ ÂÚÈÛÙ·ÛÈ·ÎÔ› Î·È ÔÈ ÂÁÁÂÓ›˜. ∞) ™ÙÔ˘˜ ÂÚÈÛÙ·ÛÈ·ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÂÚÈÏ·Ì‚¿ÓÂÙ·È Ë ÁÂÓÈ΋ ηٿÛÙ·ÛË Ù˘ ˘Á›·˜ ÙÔ˘ ·È‰ÈÔ‡, Ë ıÂÚÌÔÎÚ·Û›· ÙÔ˘ ÂÚÈ‚¿ÏÏÔÓÙÔ˜, Ë ı¤ÛË ÙÔ˘ ÛÒÌ·ÙÔ˜, ÙÔ ¿ÎÚÔ ÛÙÔ ÔÔ›Ô Á›ÓÂÙ·È Ë Ì¤ÙÚËÛË, ÙÔ Â›‰Ô˜ Ù˘ Û˘Û΢‹˜ ̤ÙÚËÛ˘ (˘‰Ú·ÚÁ˘ÚÈÎfi Ì·ÓfiÌÂÙÚÔ, Doppler Î.¿.), ÙÔ ¿Á¯Ô˜ Î·È Ë ‰Ú·ÛÙËÚÈfiÙËÙ·. OÈ ·Ú¿ÁÔÓÙ˜ ·˘ÙÔ› Â›Ó·È Ôχ ÛËÌ·ÓÙÈÎÔ› ÛÙËÓ ÂÚÈÛÙ·Ûȷ΋ ̤ÙÚËÛË Ù˘ ∞¶, ÂÓÒ ‰ÂÓ ·›˙Ô˘Ó ÙfiÛÔ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ fiÙ·Ó Á›ÓÂÙ·È 24ˆÚË Î·Ù·Ì¤ÙÚËÛË. ™ÙÔ ÛËÌÂ›Ô ·˘Ùfi Ú¤ÂÈ Ó· ÙÔÓÈÛÙ› Î·È Ë “˘¤ÚÙ·ÛË Ù˘ Ï¢΋˜ ԉȿ˜”, ‰ËÏ·‰‹ Ë Â˘·ÈÛıËÛ›· ÙˆÓ ·È‰ÈÒÓ Î·È È‰È·›ÙÂÚ· ÙˆÓ ÂÊ‹‚ˆÓ, Ô˘ Ù›ÓÔ˘Ó Ó· ·Ó‚¿˙Ô˘Ó ÙËÓ ›ÂÛ‹ ÙÔ˘˜ ÏfiÁˆ ¿Á¯Ô˘˜, fiÙ·Ó ÂÍÂÙ¿˙ÔÓÙ·È ·fi ÙÔÓ ·È‰›·ÙÚÔ. ¶ÔÛÔÛÙfi 44% ÙˆÓ ·È‰ÈÒÓ Ì ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ˘¤ÚÙ·Û˘ Î·È 18% ÙˆÓ ·È‰ÈÒÓ Ì ·ÚÓËÙÈÎfi, ÂÌÊ·Ó›˙ÂÈ “˘¤ÚÙ·ÛË Ù˘ Ï¢΋˜ ԉȿ˜” (4,7,11). µ) ™ÙÔ˘˜ ÂÁÁÂÓ›˜ ·Ú¿ÁÔÓÙ˜ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÙÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘, Ë ËÏÈΛ· ΢‹Ûˆ˜, Ë ·ÚÙËÚȷ΋ ›ÂÛË Ù˘ ÌËÙ¤Ú·˜ (Ô˘ Â›Ó·È È‰È·›ÙÂÚ· ÛËÌ·ÓÙÈο ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·), ÙÔ ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜, Ë ÛˆÌ·ÙÈ΋ Ì¿˙·, ÙÔ Ê‡ÏÔ, ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ˘¤ÚÙ·Û˘, ÙÔ ·Ó ·ıÏÂ›Ù·È ‹ fi¯È ÙÔ ·È‰›, Ë ‚ÈÔÏÔÁÈ΋ ˆÚ›Ì·ÓÛË Û ۯ¤ÛË Ì ÙËÓ ËÏÈΛ· Î·È Ë ÂÚÈÂÎÙÈÎfiÙËÙ· Ù˘ ‰È·ÙÚÔÊ‹˜ Û ӿÙÚÈÔ, οÏÈÔ Î·È ·Û‚¤ÛÙÈÔ (4,6,11,16-18). ª¤ÙÚËÛË Ù˘ ∞¶ ∏ ̤ÙÚËÛË Ì ˘‰Ú·ÚÁ˘ÚÈÎfi Ì·ÓfiÌÂÙÚÔ Â›Ó·È Ë Ì¤ıÔ‰Ô˜ ÂÈÏÔÁ‹˜ ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜, ÂÓÒ Û ÓÂÔÁÓ¿ Î·È ‚Ú¤ÊË ÙÔ Doppler Â›Ó·È Î·Ù·ÏÏËÏfiÙÂÚÔ (3,13). OÈ ÂÚÈÛÛfiÙÂÚÔÈ Û˘ÁÁÚ·Ê›˜ ÚÔÙ›ÓÔ˘Ó Ó· Á›ÓÂÙ·È Ë Ì¤ÙÚËÛË ÌÂÙ¿ ·fi 3-5 min ·Ó¿·˘Û˘. OÈ Û˘Óı‹Î˜ ̤ÙÚËÛ˘ Ú¤ÂÈ Ó· Â›Ó·È ÛÙ·ıÂÚ¤˜. ™˘ÓÈÛÙ¿Ù·È ÙÔ ·È‰› Ó· Â›Ó·È Î·ıÈÛÙfi Î·È Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÙÔ ‰ÂÍ› ¿Óˆ ¿ÎÚÔ. ∏ ̤ÙÚËÛË Ú¤ÂÈ Ó· ·ӷϷ̂¿ÓÂÙ·È fiÛ˜ ÊÔÚ¤˜ ¯ÚÂÈ¿˙ÂÙ·È, ̤¯ÚÈ Ó· ËÚÂÌ‹ÛÂÈ ÙÔ ·È‰›. ÕÏÏÔÈ Û˘ÁÁÚ·Ê›˜ Û˘ÓÈÛÙÔ‡Ó Ó· ÂȉÈÒÎÂÙ·È ÚÒÙÈÛÙ· Ë ËÚÂÌ›· ÙÔ˘ ·È‰ÈÔ‡ Î·È ·Ó Ô ÁÈ·ÙÚfi˜ ÎÚ›ÓÂÈ fiÙÈ Û ‡ÙÈ· ı¤ÛË ·˘Ùfi ÂÈÙ˘Á¯¿ÓÂÙ·È Î·Ï‡ÙÂÚ·, ÙfiÙ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› Ë ı¤ÛË ·˘Ù‹, ·ÚΛ Ó· Á›ÓÔÓÙ·È Î·Ù¿ ÙÔÓ ›‰ÈÔ ÙÚfiÔ Î·È ÔÈ ÂfiÌÂÓ˜ ÌÂÙÚ‹ÛÂȘ, ÁÈ· Û˘ÁÎÚÈÙÈÎÔ‡˜ ÏfiÁÔ˘˜. ∂ÈϤÁÂÙ·È Ë ÌÂÁ·Ï‡ÙÂÚË Û ̋ÎÔ˜ ÂÚȯÂÈÚ›‰· Ô˘ ÂÚÈ‚¿ÏÏÂÈ
449
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
Paediatriki 2001;64:448-459
¶›Ó·Î·˜ 1. ∂›Â‰· ∞¶ ÁÈ· ÙËÓ 90Ë Î·È 95Ë ∂£ ÛÙ· ·ÁfiÚÈ·, ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· Î·È ÙËÓ ∂£ ÙÔ˘ ‡„Ô˘˜ ÙÔ˘˜ ™˘ÛÙÔÏÈ΋ ∞¶ (mmHg) ∞Ó¿ÏÔÁ· Ì ÙËÓ ∂£ ÙÔ˘ ‡„Ô˘˜ ∏ÏÈΛ· (¤ÙË) ∂£ 1 90Ë 95Ë 2 90Ë 95Ë 3 90Ë 95Ë 4 90Ë 95Ë 5 90Ë 95Ë 6 90Ë 95Ë 7 90Ë 95Ë 8 90Ë 95Ë 9 90Ë 95Ë 10 90Ë 95Ë 11 90Ë 95Ë 12 90Ë 95Ë 13 90Ë 95Ë 14 90Ë 95Ë 15 90Ë 95Ë 16 90Ë 95Ë 17 90Ë 95Ë
5% 94 98 98 102 101 105 103 107 104 108 105 109 106 110 108 112 109 113 111 115 113 117 115 119 118 121 120 124 123 127 126 130 128 132
10% 95 99 99 103 102 106 104 108 105 109 106 110 107 111 109 113 110 114 112 116 114 118 116 120 119 122 121 125 124 128 127 131 129 133
25% 97 101 101 105 103 107 105 109 107 111 108 112 109 113 110 114 112 116 113 117 115 119 118 122 120 124 123 127 126 130 129 133 131 135
50% 75% 90% 99 101 102 103 105 106 103 104 106 107 108 110 105 107 109 109 111 112 107 109 110 111 113 114 109 111 112 113 114 116 110 112 113 114 116 117 111 113 114 115 117 118 112 114 116 116 118 119 114 116 117 118 119 121 115 117 119 119 121 123 117 119 121 121 123 125 120 121 123 124 125 127 122 124 125 126 128 129 125 127 128 129 131 132 128 130 131 132 133 135 131 132 134 134 136 138 133 135 136 137 139 140
¢È·ÛÙÔÏÈ΋ ∞¶ (mmHg) AÓ¿ÏÔÁ· Ì ÙËÓ ∂£ ÙÔ˘ ‡„Ô˘˜ 95% 103 107 107 110 109 113 111 115 113 117 114 118 115 119 116 120 118 122 119 123 121 125 124 128 126 130 129 133 132 136 134 138 137 141
5% 49 54 54 58 59 63 63 67 66 71 70 74 72 77 74 79 76 80 77 81 77 82 78 83 78 83 79 83 80 84 81 86 83 88
10% 25% 50% 75% 90% 95% 49 50 51 52 53 54 54 55 56 57 58 58 54 55 56 57 58 58 59 60 61 62 63 63 59 60 61 62 63 63 63 64 65 66 67 68 63 64 65 66 67 67 68 68 69 70 71 72 67 68 69 69 70 71 71 72 73 74 75 76 70 71 72 73 74 74 75 75 76 77 78 79 73 73 74 75 76 77 77 78 79 80 81 81 75 75 76 77 78 79 79 80 81 82 83 83 76 77 78 79 80 80 81 81 82 83 84 85 77 78 79 80 81 81 82 83 83 84 85 86 78 79 80 81 81 82 82 83 84 85 86 87 78 79 80 81 82 83 83 84 85 86 87 87 79 80 81 81 82 83 83 84 85 86 87 88 79 80 86 87 87 88 84 85 82 83 83 15 80 81 82 83 84 84 85 86 86 87 88 89 82 82 83 84 85 86 86 87 86 89 90 90 84 85 86 87 87 88 88 89 90 91 92 93
Pediatrics 1996;98(4):649
ÙÔ ‚Ú·¯›ÔÓ· ¯ˆÚ›˜ Ó· Ȥ˙ÂÈ ÙË Ì·Û¯¿ÏË ‹ ÙÔÓ ·ÁΈÓÈ·›Ô ‚fiıÚÔ Î·È Ù˘ ÔÔ›·˜ Ë Î‡ÛÙË Î·Ï‡ÙÂÈ Û ϿÙÔ˜ ÙÔ˘Ï¿¯ÈÛÙÔÓ Ù· 2/3 ÙÔ˘ ‚Ú·¯›ÔÓ· Î·È ÙÔÓ ÂÚÈ‚¿ÏÏÂÈ Ï‹Úˆ˜. ¶Ú¤ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ ÙÔ Ì¤ÁÂıÔ˜ Ù˘ ·ÛÙ˘ Ù˘ ÂÚȯÂÈÚ›‰·˜ Â›Ó·È ÙÔ ÛËÌ·ÓÙÈÎfi Î·È fi¯È ÙÔ Ì¤ÁÂıÔ˜ ÙÔ˘ ˘Ê¿ÛÌ·ÙÔ˜ Ô˘ ÙËÓ Î·Ï‡ÙÂÈ. ∆Ô ‚ÔıÚ›Ô Ù˘ η̋˜ ÙÔ˘ ·ÁÎÒÓ· Ú¤ÂÈ Ó· Â›Ó·È ÛÙÔ ›‰ÈÔ ‡„Ô˜ Ì ÙËÓ Î·Ú‰È¿. ¶Ôχ ÌÂÁ¿ÏË ÂÚȯÂÈÚ›‰· ‰›ÓÂÈ „¢‰Ò˜ ¯·ÌËϤ˜ ÙÈ̤˜, ÂÓÒ Ë ÌÈÎÚfiÙÂÚË ‰›ÓÂÈ „¢‰Ò˜ ˘„ËϤ˜ ÙÈ̤˜. ∞Ó ‰ÂÓ ˘¿Ú¯ÂÈ ÙÔ Î·Ù¿ÏÏËÏÔ Ì¤ÁÂıÔ˜, Â›Ó·È ÚÔ-
450
ÙÈÌfiÙÂÚÔ Ó· ¯ÚËÛÈÌÔÔÈËı› ÌÂÁ·Ï‡ÙÂÚË ·Ú¿ ÌÈÎÚfiÙÂÚË ÂÚȯÂÈÚ›‰· (5,12). O ·È‰›·ÙÚÔ˜ Ú¤ÂÈ Ó· ¤¯ÂÈ ÛÙÔ È·ÙÚÂ›Ô ÙÔ˘ ÙÚÂȘ ·È‰È·ÙÚÈΤ˜ ÂÚȯÂÈÚ›‰Â˜ ‰È·ÊfiÚˆÓ ÌÂÁÂıÒÓ, Ì›· ÌÂÁ¤ıÔ˘˜ ÂÓËÏ›ÎÔ˘ Î·È Ù¤ÏÔ˜ Ì›· ˘ÂÚÌÂÁ¤ıË, ÁÈ· ÌÂÙÚ‹ÛÂȘ ÛÙ· οو ¿ÎÚ· Î·È Û ·¯‡Û·Úη ·È‰È¿ (3). ∏ ÂÚȯÂÈÚ›‰· Ú¤ÂÈ Ó· ÊÔ˘ÛÎÒÓÂÈ ÂÚ›Ô˘ 20-30 mmHg ¿Óˆ ·fi ÙÔ ÛËÌÂ›Ô ÛÙÔ ÔÔ›Ô ÛÙ·Ì·Ù¿ÂÈ Ó· „ËÏ·Ê¿Ù·È Ô ÛÊ˘ÁÌfi˜ ·fi ÙËÓ ÎÂÚÎȉÈ΋ ·ÚÙËÚ›·. O ÚÒÙÔ˜ ‹¯Ô˜ Korotkoff ηıÔÚ›˙ÂÈ ÙË Û˘ÛÙÔÏÈ΋ ›ÂÛË, ÂÓÒ Ô ¤ÌÙÔ˜ ‹¯Ô˜ Korotkoff (Ô
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
Paediatriki 2001;64:448-459
¶›Ó·Î·˜ 2. ∂›Â‰· ∞¶ ÁÈ· ÙËÓ 90Ë Î·È 95Ë ∂£ ÛÙ· ÎÔÚ›ÙÛÈ·, ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· Î·È ÙËÓ ∂£ ÙÔ˘ ‡„Ô˘˜ ÙÔ˘˜ ™˘ÛÙÔÏÈ΋ ∞¶ (mmHg) ∞Ó¿ÏÔÁ· Ì ÙËÓ ∂£ ÙÔ˘ ‡„Ô˘˜ ∏ÏÈΛ· (¤ÙË) ∂£ 1 90Ë 95Ë 2 90Ë 95Ë 3 90Ë 95Ë 4 90Ë 95Ë 5 90Ë 95Ë 6 90Ë 95Ë 7 90Ë 95Ë 8 90Ë 95Ë 9 90Ë 95Ë 10 90Ë 95Ë 11 90Ë 95Ë 12 90Ë 95Ë 13 90Ë 95Ë 14 90Ë 95Ë 15 90Ë 95Ë 16 90Ë 95Ë 17 90Ë 95Ë
5% 98 101 99 103 100 104 101 105 103 107 104 108 106 110 108 112 110 114 112 116 114 118 116 120 118 122 120 124 121 125 122 126 123 127
10% 98 102 99 103 101 104 102 106 103 107 105 109 107 111 109 113 111 115 113 117 115 119 117 121 119 123 121 125 122 126 123 127 123 127
25% 99 103 101 104 102 106 103 107 105 108 106 110 108 112 110 114 112 116 114 118 116 120 118 122 120 124 122 126 123 127 124 128 124 128
50% 101 104 102 106 103 107 104 108 106 110 107 111 109 113 111 115 113 117 115 119 117 121 119 123 121 125 123 127 124 128 125 129 126 130
75% 102 106 103 107 104 108 106 109 107 111 109 113 110 114 112 116 114 118 116 120 119 122 121 125 123 126 124 128 126 130 127 130 127 131
90% 103 107 104 108 105 109 107 111 108 112 110 114 112 115 114 117 116 119 118 122 120 124 122 126 124 128 125 129 127 131 128 132 128 132
¢È·ÛÙÔÏÈ΋ ∞¶ (mmHg) AÓ¿ÏÔÁ· Ì ÙËÓ ∂£ ÙÔ˘ ‡„Ô˘˜ 95% 104 108 105 109 106 110 108 111 109 113 111 114 112 116 114 118 116 120 118 122 120 124 123 126 124 128 126 130 128 131 129 132 129 133
5% 52 56 57 61 61 65 64 68 66 71 69 73 71 75 72 76 74 78 75 79 76 81 78 82 79 83 80 84 80 85 81 85 81 85
10% 25% 50% 75% 90% 95% 52 53 53 54 55 55 56 57 58 58 59 60 57 58 58 59 60 60 61 62 62 63 64 64 61 61 62 63 64 66 65 66 66 67 68 68 64 65 65 66 67 67 68 69 69 70 71 71 67 67 68 69 69 70 71 71 72 73 74 74 69 69 70 71 72 72 73 74 74 75 76 76 71 71 72 73 74 74 75 75 76 77 78 78 72 73 74 74 75 76 77 77 78 79 79 80 74 74 75 76 77 77 78 79 79 80 81 81 75 76 77 77 78 78 79 80 81 81 82 83 77 77 78 79 79 80 81 81 82 83 83 84 78 78 79 80 81 81 82 82 83 84 85 85 79 79 80 81 82 82 83 84 84 85 86 86 80 80 81 82 83 83 84 85 85 86 87 87 81 81 82 83 83 84 85 85 86 87 88 88 81 82 82 83 84 84 85 86 87 87 88 88 81 82 83 83 84 85 86 86 87 88 88 89
Pediatrics 1996;98(4):649
ÙÂÏÂ˘Ù·›Ô˜ Ô˘ ·ÎÔ‡ÁÂÙ·È) ηıÔÚ›˙ÂÈ ÙË ‰È·ÛÙÔÏÈ΋ ›ÂÛË, Û‡Ìʈӷ Ì ÙË Û‡ÛÙ·ÛË Ù˘ ∞ÌÂÚÈηÓÈ΋˜ ∫·Ú‰ÈÔÏÔÁÈ΋˜ ∂Ù·ÈÚ›·˜. ™Â ÌÂÚÈο ·È‰È¿, ‹¯ÔÈ Korotkoff ÌÔÚ› Ó· ·ÎÔ‡ÁÔÓÙ·È Ê˘ÛÈÔÏÔÁÈο Î·È Ì¤¯ÚÈ ÙÔ 0 mmHg. ∞˘Ùfi ‰ÂÓ ·ÔÙÂÏ› Úfi‚ÏËÌ·, ÂÊfiÛÔÓ ·ÔÎÏ›ÂÙ·È Ë ‰È·ÛÙÔÏÈ΋ ˘¤ÚÙ·ÛË. ∆ËÓ ÚÒÙË ÊÔÚ¿ Ô˘ ı· ÂϤÁ¯ÂÙ·È Ë ∞¶ ÂÓfi˜ ·È‰ÈÔ‡, Ë Ì¤ÙÚËÛË ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È Î·È ÛÙ· Ù¤ÛÛÂÚ· ¿ÎÚ· ÁÈ· Ó· ‰È·ÈÛÙˆı› Ù˘¯fiÓ ˘¿Ú¯Ô˘Û· ÈÛıÌÈ΋ ÛÙ¤ÓˆÛË ·ÔÚÙ‹˜. ™Â οı ÂfiÌÂÓË ÎÏÈÓÈ΋ ÂͤٷÛË, Ë A¶ Ú¤ÂÈ Ó· ÌÂÙÚ¿Ù·È ÙÔ˘Ï¿¯È-
ÛÙÔÓ ‰‡Ô ÊÔÚ¤˜. O ̤ÛÔ˜ fiÚÔ˜ ÙˆÓ Û˘ÛÙÔÏÈÎÒÓ Î·È ÙˆÓ ‰È·ÛÙÔÏÈÎÒÓ ÙÈÌÒÓ Ô˘ ı· ‚Ú›ÛÎÔÓÙ·È, ı· ·ÔÙÂÏ› ÙËÓ ÙÂÏÈ΋ ›ÂÛË (11,15). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ë Û˘Ó¯‹˜ ÊÔÚËÙ‹ ηٷÁÚ·Ê‹ Ù˘ ∞¶ ÂÊ·ÚÌfi˙ÂÙ·È fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚÔ ÛÙ· ·È‰È¿ Î·È ˘¿Ú¯Ô˘Ó ϤÔÓ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ·Ó·ÊÔÚ¿˜. ∂›Ó·È ȉȷ›ÙÂÚ· ¯Ú‹ÛÈÌË ÛÙËÓ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Ù˘ ˘¤ÚÙ·Û˘, ηıÒ˜ Î·È ÛÙËÓ ·ÔÊ˘Á‹ ˘ÂډȿÁÓˆÛ˘, ÂȉÈο ‰Â ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ù˘ “˘¤ÚÙ·Û˘ Ù˘ Ï¢΋˜ ԉȿ˜”. µÔËı¿ÂÈ ÛÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ıÂڷ›·˜ ηÈ
451
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
ȉȷ›ÙÂÚ· Û ·È‰È¿ Ì ¯ÚfiÓÈ· ÓÂÊÚÈο ÚÔ‚Ï‹Ì·Ù· ‹ ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Î·È ¤¯ÂÈ Î·Ï‡ÙÂÚË ÚÔÁÓˆÛÙÈ΋ ·Í›·, fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ Úfi‚ÏÂ„Ë ‚Ï¿‚˘ ÛÙ· fiÚÁ·Ó· ÛÙfi¯Ô˘˜ (4,6,7,15,19,20). Ÿˆ˜ ‹‰Ë ·Ó·Ê¤ÚıËÎÂ, Ë Ì¤ÙÚËÛË Ù˘ ∞¶ ÛÙ· ÓÂÔÁÓ¿ Â›Ó·È ÚÔÙÈÌfiÙÂÚÔ Ó· Á›ÓÂÙ·È Ì Doppler. ∏ Ê˘ÛÈÔÏÔÁÈ΋ ∞¶ ÂÓfi˜ ÙÂÏÂÈfiÌËÓÔ˘ ÓÂÔÁÓÔ‡ Â›Ó·È Û˘Ó‹ıˆ˜ 60-70 Ë Û˘ÛÙÔÏÈ΋ Î·È 40-50 Ë ‰È·ÛÙÔÏÈ΋ (4,12). ∂Ó‰ÂÈÎÙÈο ·Ó·Ê¤ÚÂÙ·È fiÙÈ Û˘ÛÙÔÏÈ΋ ∞¶ ÛÙ·ıÂÚ¿ ¿Óˆ ÙÔ˘ 90/60 ÛÙ· ÙÂÏÂÈfiÌËÓ· Î·È 80/50 ÛÙ· ÚfiˆÚ· ıˆÚÂ›Ù·È ·ıÔÏÔÁÈ΋ (21). ∆ÔÓ›˙ÂÙ·È ‰Â fiÙÈ Ë Û˘ÛÙÔÏÈ΋ ›ÂÛË Â›Ó·È ·˘Ù‹ Ô˘ ηıÔÚ›˙ÂÈ ÙËÓ ‡·ÚÍË ˘¤ÚÙ·Û˘ Û ·È‰È¿ ÌÈÎÚfiÙÂÚ· ÙÔ˘ ¤ÙÔ˘˜ (18). ∂ȉËÌÈÔÏÔÁ›· ∏ Û˘¯ÓfiÙËÙ· Ù˘ ˘¤ÚÙ·Û˘ ÛÙ· ·È‰È¿ ˘ÔÏÔÁ›˙ÂÙ·È Û 1% Î·È Ë Û˘¯ÓfiÙËÙ· Ù˘ ÂÈ̤ÓÔ˘Û·˜ ‰Â˘ÙÂÚÔ·ıÔ‡˜ ˘¤ÚÙ·Û˘ Û 0,1% (3,4,11,15). ¶·Ú’ fiÏÔ Ô˘ Ë ˘¤ÚÙ·ÛË Â›Ó·È ·Û˘Ó‹ı˘ ÛÙ· ÓÂÔÁÓ¿, ÔÚÈṲ̂ӷ ‰È·ÙÚ¤¯Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ, fiˆ˜ .¯. Ù· ÚfiˆÚ·. ÀÔÏÔÁ›˙ÂÙ·È fiÙÈ 0,2% ÙˆÓ ÙÂÏÂÈfiÌËÓˆÓ Î·È 0,79-2,5% ÙˆÓ ÚÔÒÚˆÓ ÌÔÚ› Ó· ÂÌÊ·Ó›ÛÔ˘Ó ˘¤ÚÙ·ÛË (21). ™ËÌ·ÓÙÈÎfi ‡ÚËÌ· ÚfiÛÊ·ÙˆÓ ÌÂÏÂÙÒÓ ·ÔÙÂÏ› ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ˘¤ÚÙ·ÛË Ù›ÓÂÈ Ó· ‰È·ÙËÚÂ›Ù·È ÂÊ’ fiÚÔ˘ ˙ˆ‹˜. ∆Ô ›‰ÈÔ ÈÛ¯‡ÂÈ Î·È ÁÈ· Ù· ›‰· ¯ÔÏËÛÙÂÚfiÏ˘ Î·È ÏÈÔÚˆÙÂ˚ÓÒÓ ¯·ÌËÏ‹˜ ˘ÎÓfiÙËÙ·˜ (1). ∆· ‰Â‰Ô̤ӷ Ù˘ ‰È·¯ÚÔÓÈ΋˜ ‰È·Ù‹ÚËÛ˘ Û˘Û¯ÂÙ›˙Ô˘Ó Ù· ·È‰Èο Î·È ÂÊË‚Èο ›‰· ∞¶ Ì ÙËÓ ˘¤ÚÙ·ÛË ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹, ·ÏÏ¿ ‰˘ÛÙ˘¯Ò˜ ·Ú¿ ÙÔ Û˘Û¯ÂÙÈÛÌfi, ‰ÂÓ ÌÔÚ› Ó· ÚÔ‚ÏÂÊı› ÔÈ· ·fi Ù· ·È‰È¿ Ì ˘„ËÏ‹ ∞¶ ı· Á›ÓÔ˘Ó ˘ÂÚÙ·ÛÈÎÔ› ÂÓ‹ÏÈΘ (4). ™Â ·ÓÙȉȷÛÙÔÏ‹ Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ, ÛÙËÓ ÂÚ›ÙˆÛË ÙˆÓ ·È‰ÈÒÓ ‰ÂÓ Ê·›ÓÂÙ·È Ó· ÂËÚ¿˙Ô˘Ó ÙËÓ ∞¶ ‰È·ÊÔÚ¤˜ ÛÙÔ ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎfi Â›Â‰Ô ‹ Ì¿ÏÏÔÓ ÔÈ ÂȉڿÛÂȘ ÙÔ˘˜ ‰ÂÓ ¤¯Ô˘Ó Á›ÓÂÈ ·ÎfiÌË ·ÓÙÈÏËÙ¤˜. ¶ÂÈÚ·Ì·ÙÈο ‰Â‰Ô̤ӷ Î·È ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó fiÙÈ ÙÔ stress Î·È Ë ÚfiÛÏË„Ë ˘„ËÏÒÓ ÔÛÔÙ‹ÙˆÓ ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ Ì ÙȘ ÙÚÔʤ˜ ÂËÚ¿˙Ô˘Ó ÙËÓ ∞¶ ÛÙË ÌÂÙ¤ÂÈÙ· ˙ˆ‹, ÙfiÛÔ Û ÂÈÚ·Ì·Ùfi˙ˆ· fiÛÔ Î·È ÛÙÔÓ ·ÓıÚÒÈÓÔ ÏËı˘ÛÌfi (22). ∞ӷʤÚÂÙ·È fiÙÈ ÛÙ· ·È‰È¿ ÙÔ˘ ‰˘ÙÈÎÔ‡ ÎfiÛÌÔ˘, Ë ÚfiÛÏË„Ë ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ Ì ÙȘ ÙÚÔʤ˜ Â›Ó·È ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚË ·fi ÙȘ ‰È·ÙÚÔÊÈΤ˜ ÙÔ˘˜ ·Ó¿ÁΘ. ™Ù· ·È‰È¿, ÏÔÈfiÓ, ·˘Ù¿ Î·È ÂÊfiÛÔÓ ¤¯Ô˘Ó ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ˘¤ÚÙ·Û˘, Ë ·˘ÍË̤ÓË ÚfiÛÏË„Ë ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ ·˘Í¿ÓÂÈ ÙËÓ ∞¶ (23). À¿Ú¯Ô˘Ó ÛËÌ·ÓÙÈΤ˜ ÂӉ›ÍÂȘ fiÙÈ Ë ÚˆÙÔ·ı‹˜ ˘¤ÚÙ·ÛË ·Ú¯›˙ÂÈ ·fi ÙËÓ ·È‰È΋ ËÏÈΛ·, ÙÔ ›‰ÈÔ Î·È Ù· ÚÒÙ· ÛËÌ›· Ù˘ ηډȷÁÁÂȷ΋˜ ÓfiÛÔ˘ ÙˆÓ ÂÓËÏ›ÎˆÓ Î·È Ì¿ÏÈÛÙ· ·ÚÎÂÙ¤˜ ‰ÂηÂٛ˜ ÚÈÓ ·fi ÙËÓ ÂÌÊ¿ÓÈÛË ÙˆÓ Û˘Ìو̿وÓ.
452
Paediatriki 2001;64:448-459
πÛ¯˘Úfiٷ٘ ÂӉ›ÍÂȘ ·fi ÓÂÎÚÔÙÔÌÈΤ˜ ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë ÛÙÂÊ·ÓÈ·›· ÓfiÛÔ˜ ·Ú¯›˙ÂÈ Û Ôχ ÌÈÎÚ‹ ËÏÈΛ· Î·È Û˘Ó‰¤ÂÙ·È ÛÙÂÓ¿ Ì ÙËÓ ·˘ÍË̤ÓË ·ÚÙËÚȷ΋ ›ÂÛË, Û˘ÛÙÔÏÈ΋ ‹ ‰È·ÛÙÔÏÈ΋ Î·È ÙËÓ ·¯˘Û·ÚΛ·, Â›Ó·È ‰Â ·ÓÙÈÛÙÚfiʈ˜ ·Ó¿ÏÔÁË ÙˆÓ ÂȤ‰ˆÓ ÙˆÓ ÏÈÔÚˆÙÂ˚ÓÒÓ ˘„ËÏ‹˜ ˘ÎÓfiÙËÙ·˜ (9). ªÂϤÙ˜ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ, ‹‰Ë ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 15 ÂÙÒÓ, ÂÚ›Ô˘ ÙÔ 50% ÙˆÓ ·È‰ÈÒÓ ¤¯ÂÈ ·ÚÙËÚÈÔÛÎÏËÚ˘ÓÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ ÛÙȘ ÛÙÂÊ·ÓÈ·›Â˜ ·ÚÙËڛ˜ Î·È ÙÔ 100% ÛÙËÓ ·ÔÚÙ‹ (9). ∂›Ó·È ‰Â ÁÓˆÛÙfi fiÙÈ Ë ˘¤ÚÙ·ÛË Ì·˙› Ì ¿ÏÏÔ˘˜ ÂÈ‚·Ú˘ÓÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ·¯˘Û·ÚΛ·, Ô Û·Î¯·Ú҉˘ ‰È·‚‹Ù˘, ÙÔ Î¿ÓÈÛÌ· Î·È ÔÈ ÏÈÔÚˆÙ½Ó˜ ¯·ÌËÏ‹˜ ˘ÎÓfiÙËÙ·˜, Û˘Ó‰¤ÔÓÙ·È ¿ÌÂÛ·, Û·Ó ÂÈ‚·Ú˘ÓÙÈÎÔ› ·Ú¿ÁÔÓÙ˜, Ì ÙË ÛÙÂÊ·ÓÈ·›· ÓfiÛÔ, ÂÓÒ ÔÈ ˘„ËÏ‹˜ ˘ÎÓfiÙËÙ·˜ ÏÈÔÚˆÙ½Ó˜ ÚÔÛٷهԢÓ. ™Â ·Ó·‰ÚÔÌÈΤ˜ ÌÂϤÙ˜ ¤¯ÂÈ Î·Ù·‰Âȯı› fiÙÈ Ë ·¯˘Û·ÚΛ·, Ë ‰˘ÛÏÈȉ·ÈÌ›· Î·È Ë ˘¤ÚÙ·ÛË ˘‹Ú¯·Ó ‹‰Ë ·fi ÙËÓ ·È‰È΋ ËÏÈΛ· Î·È ·˘Ùfi Â›Ó·È Ôχ ÛËÌ·ÓÙÈÎfi ÁÈ· ÙË Ï‹„Ë ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ. ∞ÈÙÈÔÏÔÁ›· ∏ ˘¤ÚÙ·ÛË ‰È·¯ˆÚ›˙ÂÙ·È Û ‰Â˘ÙÂÚÔ·ı‹ Î·È ÚˆÙÔ·ı‹. ™ÙË ‰Â˘ÙÂÚÔ·ı‹ ˘¤ÚÙ·ÛË, Ë ·ÈÙÈÔÏÔÁ›· ÌÔÚ› Ó· ηıÔÚÈÛÙ›. ¶ÂÚ›Ô˘ ÙÔ 85-90% ÙˆÓ ·È‰ÈÒÓ Î¿Ùˆ ÙˆÓ 10 ÂÙÒÓ ¤¯ÂÈ ‰Â˘ÙÂÚÔ·ı‹ ˘¤ÚÙ·ÛË. °ÂÓÈο, fiÛÔ ÈÔ ÌÈÎÚ‹˜ ËÏÈΛ·˜ Â›Ó·È ÙÔ ·È‰› Î·È fiÛÔ ÈÔ ˘„ËÏ‹ Ë ∞¶, ÙfiÛÔ Èı·ÓfiÙÂÚË Á›ÓÂÙ·È Ë ‰È¿ÁÓˆÛË ‰Â˘ÙÂÚÔ·ıÔ‡˜ ˘¤ÚÙ·Û˘, fiˆ˜ .¯. Ë ÔÊÂÈÏfiÌÂÓË Û ·ÚÂÁ¯˘Ì·ÙÈ΋ ‹ ·ÁÁÂȷ΋ ÓÂÊÚÈ΋ ‚Ï¿‚Ë, Ë ÔÔ›· ÊÙ¿ÓÂÈ ÙÔ 75-80% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (12,24). ∏ ‰Â˘ÙÂÚÔ·ı‹˜ ˘¤ÚÙ·ÛË ÌÔÚ› Ó· Â›Ó·È ·ÚÔ‰È΋ ‹ ¯ÚfiÓÈ·. ∏ ·ÚÔ‰È΋ ‹ ‰È·ÏÂ›Ô˘Û· ÌÔÚ› Ó· Â›Ó·È ÓÂÊÚÈ΋˜, ÂÚÈÊÂÚÈ΋˜ ‹ ÎÂÓÙÚÈ΋˜ Ó¢ÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜ ‹ Ó· ÔÊ›ÏÂÙ·È Û ʿÚ̷η ‹ ‰ËÏËÙËÚ›·ÛË, ·ÏÏ¿ Î·È Û ¿ÏϘ ÓÔÛÔÏÔÁÈΤ˜ ÔÓÙfiÙËÙ˜. £· Ú¤ÂÈ ÛÙÔ ÛËÌÂ›Ô ·˘Ùfi Ó· ÙÔÓÈÛÙ› Ë ¯Ú‹ÛË Ó·ÚΈÙÈÎÒÓ Î·È ·ÓÙÈÛ˘ÏÏËÙÈÎÒÓ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜. ™Ù· ·›ÙÈ· Ù˘ ¯ÚfiÓÈ·˜ ‰Â˘ÙÂÚÔ·ıÔ‡˜ ˘¤ÚÙ·Û˘ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÓÂÊÚÈΤ˜, ·ÁÁÂȷΤ˜, ÂÓ‰ÔÎÚÈÓÈΤ˜, ηıÒ˜ Î·È ÓfiÛÔÈ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ™ÙËÓ ÚˆÙÔ·ı‹ ˘¤ÚÙ·ÛË ‰ÂÓ ·Ó¢ڛÛÎÂÙ·È Û˘ÁÎÂÎÚÈ̤ÓË ˘ÔΛÌÂÓË ÓfiÛÔ˜. ∫ÏËÚÔÓÔÌÈÎÔ› Î·È ÂÚÈ‚·ÏÏÔÓÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙÔÓ Î·ıÔÚÈÛÌfi ÙˆÓ ÂȤ‰ˆÓ Ù˘ ∞¶ (25). OÈ ÁÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙËÓ ÚfiÎÏËÛË Ù˘ ˘¤ÚÙ·Û˘ ‰ÂÓ ¤¯Ô˘Ó ·ÎfiÌË Î·ıÔÚÈÛÙ›, ·ÏÏ¿ Ë ÂÈÎÚ·ÙÔ‡Û· ¿Ô„Ë Â›Ó·È fiÙÈ Ë ÎÏËÚÔÓÔÌÈÎfiÙËÙ· Â›Ó·È ÔÏ˘ÁÔÓÈ΋. ŒÙÛÈ, Ù· ·È‰È¿ ÙˆÓ ÔÔ›ˆÓ Ô ¤Ó·˜ ‹ Î·È ÔÈ ‰‡Ô ÁÔÓ›˜ ›ӷÈ
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
˘ÂÚÙ·ÛÈÎÔ›, ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚÔ Î›Ó‰˘ÓÔ Ó· ·Ó·Ù‡ÍÔ˘Ó ˘¤ÚÙ·ÛË Û ۯ¤ÛË Ì ·È‰È¿ ÌË ˘ÂÚÙ·ÛÈÎÒÓ ÁÔÓ¤ˆÓ (5). ¶·È‰È¿ Ì ∞¶ Û ˘„ËϤ˜ ∂£ ÚÔ¤Ú¯ÔÓÙ·È Û˘Ó‹ıˆ˜ ·fi ÔÈÎÔÁ¤ÓÂȘ Ì ÈÛÙÔÚÈÎfi ˘¤ÚÙ·Û˘ (3). ∞‰¤ÏÊÈ· ˘ÂÚÙ·ÛÈÎÒÓ ·È‰ÈÒÓ ¤¯Ô˘Ó ˘„ËÏfiÙÂÚË ∞¶ ·fi Ù· ·‰¤ÏÊÈ· ÌË ˘ÂÚÙ·ÛÈÎÒÓ ·È‰ÈÒÓ (18). ∂›Û˘, ˘¿Ú¯ÂÈ ÌÂÁ·Ï‡ÙÂÚË Û˘Û¯¤ÙÈÛË ∞¶ ÌÂٷ͇ ÌËÙ¤ÚˆÓ Î·È ·È‰ÈÒÓ ·’ fiÙÈ ÌÂٷ͇ ·Ù¤ÚˆÓ Î·È ·È‰ÈÒÓ, ÁÂÁÔÓfi˜ Ô˘ ˘·ÈÓ›ÛÛÂÙ·È Î·È ÚÔÁÂÓÓËÙÈ΋ ›‰Ú·ÛË. ¶·Ú’ fiÏ· ·˘Ù¿ Ù· ‰Â‰Ô̤ӷ, Â›Ó·È ·‰‡Ó·ÙÔÓ Ó· ÚÔ‚ÏÂÊı› ÔÈ· ·fi Ù· ÎÏËÚÔÓÔÌÈο ‚‚·Ú˘Ì¤Ó· ·È‰È¿ ı· ·ÚÔ˘ÛÈ¿ÛÔ˘Ó ˘¤ÚÙ·ÛË ÛÙÔ Ì¤ÏÏÔÓ. ¶ÂÚÈ‚·ÏÏÔÓÙÈÎÔ› ·Ú¿ÁÔÓÙ˜, fiˆ˜ ÙÔ stress Î·È Ë ÂÚÈÂÎÙÈÎfiÙËÙ· Ù˘ ÙÚÔÊ‹˜ Û ·Ï¿ÙÈ ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ÚfiÎÏËÛË Ù˘ ˘¤ÚÙ·Û˘. ∂›Û˘, ·È‰È¿ ˘ÂÚÙ·ÛÈÎÒÓ ÁÔÓ¤ˆÓ ·ÓÙȉÚÔ‡Ó Ì ÌÂÁ·Ï‡ÙÂÚË ·‡ÍËÛË Ù˘ ∞¶ ÌÂÙ¿ ·fi stress ‹ ÊfiÚÙÈÛË Ì ¯ÏˆÚÈÔ‡¯Ô Ó¿ÙÚÈÔ. ∆Ô ›‰ÈÔ Û˘Ì‚·›ÓÂÈ Î·È ÛÙ· ·¯‡Û·Úη ·È‰È¿ (16). °ÂÓÈο, ·¯‡Û·Úη ¿ÙÔÌ· ¤¯Ô˘Ó ˘¤ÚÙ·ÛË Ôχ ÈÔ Û˘¯Ó¿ ·fi Ù· ÏÂÙfiۈ̷. ∆Ô ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜, ηıÒ˜ Î·È Ë ÚfiÛÏË„Ë ‚¿ÚÔ˘˜ Â›Ó·È ÛËÌ·ÓÙÈÎÔ› ηıÔÚÈÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ù˘ ∞¶. ¶ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó fiÙÈ ˘¤ÚÙ·ÛË ÛÙË ÌËÙ¤Ú· ÌÔÚ› Ó· ÚÔηϤÛÂÈ ÂÓ‰ÔÌ‹ÙÚÈ· ηı˘ÛÙ¤ÚËÛË Ù˘ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘ Î·È Èı·ÓÒ˜ ÌÂÙ¤ÂÈÙ· ·Ó·Ù˘Íȷ΋ ηı˘ÛÙ¤ÚËÛË ‹ Ó· Â›Ó·È ÚÔÔÌfi˜ ˘¤ÚÙ·Û˘ ÛÙÔ˘˜ ·ÔÁfiÓÔ˘˜. ∞ÎfiÌË, ÙÔ ¯·ÌËÏfi ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ Û˘Ó‰¤ÂÙ·È ÛËÌ·ÓÙÈο Ì ÙÔÓ Î›Ó‰˘ÓÔ ÂÌÊ¿ÓÈÛ˘ ˘¤ÚÙ·Û˘ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (6,16). ∏ ˘ÂÚÈÓÛÔ˘ÏÈÓ·ÈÌ›· Î·È Ù· ˘„ËÏ¿ ›‰· ÁÏ˘Îfi˙˘ ÛÙÔ ·›Ì· Ê·›ÓÂÙ·È, Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜, Ó· Û˘Ó‰¤ÔÓÙ·È Ì ÙËÓ ˘¤ÚÙ·ÛË, ̤ۈ ÔÏÏ·ÏÒÓ Ì˯·ÓÈÛÌÒÓ (26). ∆· ›‰· ÚÂÓ›Ó˘ ÛÙÔ ·›Ì· ÌÔÚ› Ó· Â›Ó·È ˘„ËÏ¿, Ê˘ÛÈÔÏÔÁÈο ‹ ¯·ÌËÏ¿. ¶·Ú’ fiÏÔ Ô˘ Ë ÌÂϤÙË ÙˆÓ ÂȤ‰ˆÓ ÚÂÓ›Ó˘ ¤¯ÂÈ ·Ô‰Âȯı› ¯Ú‹ÛÈÌË ÛÙËÓ Î·Ù·ÓfiËÛË Ù˘ ·ıÔÊ˘ÛÈÔÏÔÁ›·˜ Î·È ÛÙË ıÂڷ›· Ù˘ ÓfiÛÔ˘ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, ‰˘ÛÙ˘¯Ò˜ ‰ÂÓ ¤¯Ô˘Ó ·ÎfiÌË ÔÏÔÎÏËÚˆı› ÔÈ ÌÂϤÙ˜ ÛÙ· ·È‰È¿. ™ÙÔ Ì¤ÏÏÔÓ, Ë ¯ÚËÛÈÌÔÔ›ËÛË ÙˆÓ ·Ó·ÛÙÔϤˆÓ ÙÔ˘ ÌÂÙ·ÙÚÂÙÈÎÔ‡ ÂÓ˙‡ÌÔ˘ ÚԂϤÂÙ·È Ó· ¤¯ÂÈ ÛËÌ·ÓÙÈ΋ ı¤ÛË ·ÎfiÌË Î·È ÛÙËÓ ÚfiÏË„Ë ÔÚÈÛÌ¤ÓˆÓ ÌÔÚÊÒÓ ÚˆÙÔ·ıÔ‡˜ ˘¤ÚÙ·Û˘. ∞Í›˙ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ ÛÙ· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ Ë Û˘¯ÓfiÙÂÚË ÌÔÚÊ‹ ˘¤ÚÙ·Û˘ Â›Ó·È Ë ÚˆÙÔ·ı‹˜ Î·È ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ ÊÙ¿ÓÂÈ ÙÔ 80% (5), ÂÓÒ ÛÙ· ÌÈÎÚfiÙÂÚ· ·È‰È¿ Î·È Ù· ‚Ú¤ÊË Û˘¯ÓfiÙÂÚË Â›Ó·È Ë ‰Â˘ÙÂÚÔ·ı‹˜ (ÓÂÊÚÈ΋ ·ÚÂÁ¯˘Ì·ÙÈ΋ ÓfiÛÔ˜ Î·È ÛÙ¤ÓˆÛË ·ÔÚÙ‹˜). ¢ÂÓ Ú¤ÂÈ fï˜ Ó· ‰È·Ê‡ÁÂÈ Ù˘ ÛΤ„˘ fiÙÈ ÌÈÎÚ‹ ·‡ÍËÛË Ù˘ ∞¶ Û ÌÈÎÚ¿ ·È‰È¿ ÌÔÚ› Û¿ÓÈ· Ó· ÔÊ›ÏÂÙ·È Û ÚÒÈÌË ÂÌÊ¿ÓÈÛË ÚˆÙÔ·ıÔ‡˜ ˘¤ÚÙ·Û˘. ∂›Û˘, ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ ı· Ú¤ÂÈ Ó· ÂϤÁ¯ÂÙ·È Ë
Paediatriki 2001;64:448-459
Èı·ÓfiÙËÙ· Ï‹„˘ ·ÏÎÔfiÏ˘, ·ÌÊÂÙ·Ì›Ó˘, ÎÔη˝Ó˘, ·ÓÙÈÛ˘ÏÏËÙÈÎÒÓ Î·È ÙÔ Î¿ÓÈÛÌ· (11). ∆· Û˘¯ÓfiÙÂÚ· ·›ÙÈ· Ù˘ ˘¤ÚÙ·Û˘, ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· ÙÔ˘ ·È‰ÈÔ‡, Ê·›ÓÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 3. ∫ÏÈÓÈ΋ ÂÈÎfiÓ· ™Ù· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜, Ë ÚˆÙÔ·ı‹˜ ˘¤ÚÙ·ÛË ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÂÓ·ÏÏ·Á¤˜ Ù˘ ∞¶, ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ˘¤ÚÙ·Û˘ Î·È Û˘¯Ó¿ ·¯˘Û·ÚΛ·. ∆· ÂÚÈÛÛfiÙÂÚ· ˘ÂÚÙ·ÛÈο ·È‰È¿ Â›Ó·È ·Û˘Ìو̷ÙÈο fiÙ·Ó ·Ó·Î·Ï‡ÙÂÙ·È Ë ˘¤ÚÙ·ÛË, Ë ÔÔ›· Â›Ó·È Û˘Ó‹ıˆ˜ Ù˘¯·›Ô ‡ÚËÌ· ηٿ ÙË ‰È¿ÚÎÂÈ· ÎÏÈÓÈ΋˜ ÂͤٷÛ˘ ÚÔ˘Ù›Ó·˜, Û˘¯Ó¿ ÁÈ· ÙË ¯ÔÚ‹ÁËÛË ‚‚·›ˆÛ˘ Û˘ÌÌÂÙÔ¯‹˜ ÛÙÔ Ì¿ıËÌ· Ù˘ Á˘ÌÓ·ÛÙÈ΋˜ (27). ∞Ó, fï˜, Ë ˘¤ÚÙ·ÛË ÚÔ¸¿Ú¯ÂÈ ·fi Ì·ÎÚfi ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·, ÙfiÙ ÙÔ ·È‰› ÌÔÚ› Ó· ·Ú·ÔÓÂ›Ù·È ÁÈ· ÔÓÔÎÂÊ¿ÏÔ˘˜ ‹ ˙¿ÏË, ÂȉÈο ¤ÓÙÔÓ˜ ÈÓȷΤ˜ ÎÂÊ·Ï·ÏÁ›Â˜ Ô˘ ·Ú¯›˙Ô˘Ó ÌÂÙ¿ ÙËÓ ·Ê‡ÓÈÛË (11). µÚ¤ÊË Î·È ÌÈÎÚ¿ ·È‰È¿ Ô˘ ‰˘ÛÎÔχÔÓÙ·È Ó· ÂÎÊÚ·ÛÙÔ‡Ó, ÌÔÚ› Ó· ÂÌÊ·Ó›˙Ô˘Ó ÌÈ· ·ÓÂÍ‹ÁËÙË Â˘ÂÚÂıÈÛÙfiÙËÙ·, Ë ÔÔ›· ˘Ô¯ˆÚ› Ì ÙË ıÂڷ›· Ù˘ ˘¤ÚÙ·Û˘. ÕÏÏ· Û˘ÌÙÒÌ·Ù· ÛÙËÓ ›‰È· ËÏÈÎȷ΋ ÔÌ¿‰· Â›Ó·È Ë Î·ı˘ÛÙ¤ÚËÛË Ù˘ ۈ̷ÙÈ΋˜ ·Ó¿Ù˘Í˘, ÔÈ ‰˘ÛÎÔϛ˜ Û›ÙÈÛ˘ Î·È ÔÈ ¤ÌÂÙÔÈ, Ë ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, Ë Î˘¿ÓˆÛË, Ë Î·Ú‰È·Î‹ ·Ó¿ÚÎÂÈ·, ÔÈ Û·ÛÌÔ›, Ë ÔÏ˘‰È„›· Î·È Ë ÔÏ˘Ô˘Ú›·, fiˆ˜ Î·È ÔÈ ‰È·Ù·Ú·¯¤˜ ÛÙË Û˘ÌÂÚÈÊÔÚ¿. ∂¿Ó Ë ∞¶ Â›Ó·È Ôχ ˘„ËÏ‹, ÌÔÚ› Ó· ·ÚÔ˘ÛÈ·ÛÙÔ‡Ó Û˘ÌÙÒÌ·Ù· ÂÁÎÂÊ·ÏÈ΋˜ ˘¤ÚÙ·Û˘, ‰ËÏ·‰‹ ¤ÌÂÙÔÈ, ÎÂÊ·Ï·ÏÁ›·, ˘ÚÂÙfi˜, ·Ù·Í›·, ÎÒÌ·, ÔÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ Ô˘ ÊÙ¿ÓÔ˘Ó Ì¤¯ÚÈ Ù‡ÊψÛË ‹ Î·È Û·ÛÌÔ›, ÂÓÒ ÛÙ· ÎÏÈÓÈο Â˘Ú‹Ì·Ù· ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÙÔ Ô›‰ËÌ· ÙˆÓ ÔÙÈÎÒÓ ıËÏÒÓ Î·È Ë ·Ú¿Ï˘ÛË ÂÁÎÂÊ·ÏÈÎÒÓ Û˘˙˘ÁÈÒÓ, fiˆ˜ Ë ¿ÚÂÛË ÚÔÛˆÈÎÔ‡. ŸÙ·Ó Ë ˘¤ÚÙ·ÛË Â›Ó·È ‰Â˘ÙÂÚÔ·ı‹˜, ÌÔÚ› Ó· Û˘Ó˘¿Ú¯Ô˘Ó Ù· Û˘ÌÙÒÌ·Ù· Î·È ÛËÌ›· Ù˘ ˘ÔΛÌÂÓ˘ ÓfiÛÔ˘. ™ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ı· Ú¤ÂÈ Ó· ÂÎÙÈÌËıÔ‡Ó: ·) §ÂÙÔÌÂÚÒ˜ ÙÔ Î˘ÎÏÔÊÔÚÈÎfi Û‡ÛÙËÌ·, ÔÈ ÛʇÍÂȘ Û fiÏ· Ù· ¿ÎÚ· (·‰˘Ó·Ì›· „ËÏ¿ÊËÛ˘ ÌËÚÈ·›ˆÓ), Ë Î·Ú‰È·Î‹ Û˘¯ÓfiÙËÙ·. ‚) ∏ ∞¶ ÛÙÔ ‰ÂÍÈfi ¿Óˆ Î·È Î¿Ùˆ ¿ÎÚÔ. Á) ∏ ηٷÓÔÌ‹ ۈ̷ÙÈÎÔ‡ Ï›Ô˘˜ (·¯˘Û·ÚΛ· Ù‡Ô˘ ‚Ô˘‚¿ÏÔ˘). ‰) ƒ·Á¿‰Â˜ ‰¤ÚÌ·ÙÔ˜, ηÊÂÁ·Ï·ÎÙfi¯ÚÔ˜ ÎËÏ›‰Â˜, Ó¢ÚÈÓÒÌ·Ù·. Â) §ÂÙÔÌÂÚÒ˜ ÙÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ·. ÛÙ) O ı˘ÚÂÔÂȉ‹˜ ·‰¤Ó·˜. ˙) ∏ ÎÔÈÏȷ΋ ¯ÒÚ· ÁÈ· Ì¿˙˜. ¶Ú¤ÂÈ, ›Û˘, Ó· Á›ÓÂÈ ·ÎÚfi·ÛË ÁÈ· ·ÔÎÏÂÈÛÌfi ‡·Ú͢ Ê˘ÛËÌ¿ÙˆÓ ÛÙË ÓÂÊÚÈ΋ ¯ÒÚ·.
453
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
¶›Ó·Î·˜ 3. ∞ÈÙÈÔÏÔÁ›· Ù˘ ˘¤ÚÙ·Û˘ ¡ÂÔÁÓ¿ Î·È µÚ¤ÊË ñ ™˘ÓËı¤ÛÙÂÚ· ·›ÙÈ· - £ÚfiÌ‚ˆÛË ÓÂÊÚÈ΋˜ ·ÚÙËÚ›·˜ ‹ ÊϤ‚·˜ ÌÂÙ¿ ηıÂÙËÚÈ·ÛÌfi ÔÌÊ·ÏÈ΋˜ ·ÚÙËÚ›·˜ - πÛıÌÈ΋ ÛÙ¤ÓˆÛË ·ÔÚÙ‹˜ - ™˘ÁÁÂÓ›˜ ÓÂÊÚÈΤ˜ ·ÓˆÌ·Ï›Â˜ - ™Ù¤ÓˆÛË ÓÂÊÚÈ΋˜ ·ÚÙËÚ›·˜ ñ §ÈÁfiÙÂÚÔ Û˘¯Ó¿ ·›ÙÈ· - µÚÔÁ¯ÔÓ¢ÌÔÓÈ΋ ‰˘ÛÏ·Û›· - µÔÙ¿ÏÂÈÔ˜ fiÚÔ˜ - ∂Ó‰ÔÎÔÈÏȷ΋ ÂÁÎÂÊ·ÏÈ΋ ·ÈÌÔÚÚ·Á›· - ŸÁÎÔÈ, π·ÙÚÔÁÂÓ‹˜ ¶·È‰È¿ 1-10 ÂÙÒÓ ñ ™˘ÓËı¤ÛÙÂÚ· ·›ÙÈ· - ¡ÂÊÚÈ΋ ÓfiÛÔ˜ - πÛıÌÈ΋ ÛÙ¤ÓˆÛË ·ÔÚÙ‹˜ ñ §ÈÁfiÙÂÚÔ Û˘¯Ó¿ ·›ÙÈ· - ™Ù¤ÓˆÛË ÓÂÊÚÈ΋˜ ·ÚÙËÚ›·˜ - ÀÂÚ·Û‚ÂÛÙÈ·ÈÌ›· - ¡Â˘ÚÔ˚ӈ̿وÛË - ¡Â˘ÚÔÁÂÓ›˜ fiÁÎÔÈ - º·ÈÔ¯ÚˆÌÔ·Ùو̷ - ÀÂÚ¤ÎÎÚÈÛË ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ - ÀÂÚı˘ÚÂÔÂȉÈÛÌfi˜ - ¶·ÚÔ‰È΋ ˘¤ÚÙ·ÛË ÌÂÙ¿ ·fi ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË Ô˘ÚÔÔÈËÙÈÎÔ‡ - À¤ÚÙ·ÛË ÏfiÁˆ ÎÏÈÓÔÛÙ·ÙÈÛÌÔ‡ - À¤ÚÙ·ÛË Ô˘ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ¿ÓÔÈ· ‡ÓÔ˘ - ¶ÚˆÙÔ·ı‹˜ ˘¤ÚÙ·ÛË (Û¿ÓÈ·) ¶·È‰È¿ >11 ÂÙÒÓ Î·È ¤ÊË‚ÔÈ ñ ™˘ÓËı¤ÛÙÂÚ· ·›ÙÈ· - ¡ÂÊÚÈ΋ ÓfiÛÔ˜ - ¶ÚˆÙÔ·ı‹˜ ˘¤ÚÙ·ÛË ñ §ÈÁfiÙÂÚÔ Û˘¯Ó¿ ·›ÙÈ· - ŸÌÔÈ· Ì ٷ ·È‰È¿ 1-10 ÂÙÒÓ (ÏËÓ Ù˘ ÚˆÙÔ·ıÔ‡˜) The New England J of Medicine 1996;335:1968
Ë) ⁄·ÚÍË Ôȉ‹Ì·ÙÔ˜. ı) ⁄„Ô˜ Î·È ‚¿ÚÔ˜ ÙÔ˘ ·È‰ÈÔ‡ (ηı˘ÛÙ¤ÚËÛË ÛˆÌ·ÙÈ΋˜ ·Ó¿Ù˘Í˘). È) ŒÍˆ ÁÂÓÓËÙÈο fiÚÁ·Ó· Î·È ÛÙ¿‰ÈÔ Tanner. È·) O ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹˜. ™ÙË ‚˘ıÔÛÎfiËÛË ÌÔÚ› Ó· ‚ÚÂıÔ‡Ó ·ÏÏÔÈÒÛÂȘ ÙˆÓ ·ÁÁ›ˆÓ ÙÔ˘ ·ÌÊÈ‚ÏËÛÙÚÔÂȉԇ˜. ™Â Ì›· ÌÂϤÙË ·È‰ÈÒÓ Ì ∞¶ >90˘ ∂£ › ÙÚ›ÌËÓÔ, ‚Ú¤ıËÎ·Ó ·ÏÏÔÈÒÛÂȘ ÙˆÓ ·ÁÁ›ˆÓ ÙÔ˘ ·ÌÊÈ‚ÏËÛÙÚÔÂȉԇ˜ Û ÛËÌ·ÓÙÈο ÔÛÔÛÙ¿ Î·È ÔÈ Û˘ÁÁÚ·Ê›˜ ÙÔÓ›˙Ô˘Ó ÙË ÛÔ˘‰·ÈfiÙËÙ· Ù˘ ‚˘ıÔÛÎfiËÛ˘ ÛÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ˘¤ÚÙ·Û˘ (6,11). ŸÙ·Ó Ë ·‡ÍËÛË Ù˘ ∞¶ Â›Ó·È ÔÍ›· Î·È Î˘Ì·›ÓÂÙ·È Û ˘„ËÏ¿ ›‰·, ÚfiÎÂÈÙ·È ÂÚ› ˘ÂÚÙ·ÛÈ΋˜ ÎÚ›Û˘, Ë ÔÔ›· ·ÂÈÏ› ÙË ˙ˆ‹ ÙÔ˘ ·È‰ÈÔ‡. ∫·Ù›ÁÔ˘Û· ıˆÚÂ›Ù·È Ë ÎÚ›ÛË, ηٿ ÙËÓ ÔÔ›· Ë ∞¶ Â›Ó·È 1,3-1,5 ÊÔÚ¤˜ ˘„ËÏfiÙÂÚË ·fi ÙËÓ 95Ë ∂£ Î·È ˘¿Ú¯Ô˘Ó ÛËÌ›· ‚Ï¿‚˘ ÙˆÓ ÔÚÁ¿ÓˆÓ
454
Paediatriki 2001;64:448-459
ÛÙfi¯ˆÓ, fiˆ˜ ÂÁÎÂÊ·ÏÈÎfi ¤ÌÊÚ·ÎÙÔ, ηډȷ΋ ·Ó¿ÚÎÂÈ·, Ó¢ÌÔÓÈÎfi Ô›‰ËÌ·, ˘ÂÚÙ·ÛÈ΋ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ‹ ÂÁÎÂÊ·ÏÈ΋ ·ÈÌÔÚÚ·Á›·. OÈ ·ÛıÂÓ›˜ ¤¯Ô˘Ó ÎÏÈÓÈο Â˘Ú‹Ì·Ù· ÛÙ· ÔÔ›· ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÎÂÊ·Ï·ÏÁ›·, ÚÈÓÈ΋ ·ÈÌÔÚÚ·Á›·, ıfiψÛË Ù˘ fiÚ·Û˘ Î·È ¤ÌÂÙÔÈ. ™Â ›ÁÔ˘Û· ÎÚ›ÛË, Ë ÔÔ›· Â›Ó·È Î·È Û˘¯ÓfiÙÂÚË, ‰ÂÓ ˘¿Ú¯ÂÈ Û˘ÓÔ‰fi˜ ‚Ï¿‚Ë ÙÂÏÈÎÒÓ ÔÚÁ¿ÓˆÓ ÛÙfi¯ˆÓ Î·È Ù· Û˘ÌÙÒÌ·Ù· Â›Ó·È ÏÈÁfiÙÂÚÔ ‰Ú·Ì·ÙÈο (28,29). ¢È¿ÁÓˆÛË AÓ Ë ∞¶ ‰È·ÈÛÙˆı› ·ÓÂÈÏËÌ̤ӷ (ÙÔ˘Ï¿¯ÈÛÙÔÓ Û 3 ÌÂÙÚ‹ÛÂȘ Ô˘ ¤ÁÈÓ·Ó Û ‰È·ÊÔÚÂÙÈο ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù·) fiÙÈ Â›Ó·È ¿Óˆ Ù˘ 95˘ ∂£, ÁÈ· ÙËÓ ËÏÈΛ· ÙÔ ‡„Ô˜ Î·È ÙÔ Ê‡ÏÔ, ÙfiÙ ÙÔ ·È‰› ıˆÚÂ›Ù·È fiÙÈ ¤¯ÂÈ ˘¤ÚÙ·ÛË Î·È ·ÎÔÏÔ˘ı› ¤ÏÂÁ¯Ô˜ ηıÔÚÈÛÌÔ‡ Ù˘ ·ÈÙÈÔÏÔÁ›·˜. ∏ ¤ÌÊ·ÛË ÛÙȘ ÔÏϷϤ˜ ÌÂÙÚ‹ÛÂȘ ‰ÈηÈÔÏÔÁÂ›Ù·È ·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ù· ›‰· Ù˘ ∞¶ Ù›ÓÔ˘Ó Ó· ‚Ú›ÛÎÔÓÙ·È ¯·ÌËÏfiÙÂÚ· ÌÂÙ¿ ·fi ·ÓÂÈÏËÌ̤ÓÔ˘˜ ηıÔÚÈÛÌÔ‡˜ (3,5,12,13). ∞Ó Ë ∞¶ Û ̛· ̤ÙÚËÛË ‚ÚÂı› ˘„ËÏ‹, ·ÏÏ¿ Û ÂfiÌÂÓ˜ ÌÂÙÚ‹ÛÂȘ ‰ÂÓ ˘ÂÚ‚·›ÓÂÈ ÙËÓ 95Ë ∂£, ¯ÚÂÈ¿˙ÂÙ·È ¿ÏÈ Ù·ÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË ‰ÈfiÙÈ ÌÂÚÈο ·fi ·˘Ù¿ Ù· ·È‰È¿ ÙÂÏÈο ·Ó·Ù‡ÛÛÔ˘Ó ˘¤ÚÙ·ÛË. ¶·Ú’ fiÏÔ Ô˘ Â›Ó·È Û¿ÓÈÔ Ó· ‚ÚÂı› ÚˆÙÔ·ı‹˜ ˘¤ÚÙ·ÛË Û ·È‰È¿ ÌÈÎÚfiÙÂÚ· ÙˆÓ 10 ÂÙÒÓ, Ù· ˘ÂÚÙ·ÛÈο, ˘¤Ú‚·Ú· ·È‰È¿ ÌÂÁ·Ï‡ÙÂÚ· ÙˆÓ 5 ÂÙÒÓ Ì ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi, Â›Ó·È Èı·Ófi Ó· ¿Û¯Ô˘Ó ·fi ÚˆÙÔ·ı‹ ˘¤ÚÙ·ÛË. §ÂÙÔÌÂÚ¤˜ ÈÛÙÔÚÈÎfi Î·È ÎÏÈÓÈ΋ ÂͤٷÛË ı· ‚ÔËı‹ÛÔ˘Ó ÛËÌ·ÓÙÈο, ÂȉÈο ÛÙËÓ ·Ó·˙‹ÙËÛË Ù˘ ·ÈÙÈÔÏÔÁ›·˜ (ÚˆÙÔ·ı‹˜ ‹ ‰Â˘ÙÂÚÔ·ı‹˜) Î·È Î·Ù’ ¤ÎÙ·ÛË Ù˘ ÂÓ‰ÂÈÎÓ˘fiÌÂÓ˘ ÂÚÁ·ÛÙËÚȷ΋˜ ‰ÈÂÚ‡ÓËÛ˘. ∂Ó‰ÂÈÎÙÈο ·Ó·Ê¤ÚÂÙ·È fiÙÈ Î·ı˘ÛÙ¤ÚËÛË ÛˆÌ·ÙÈ΋˜ ·Ó¿Ù˘Í˘ Î·È Û˘¯Ó¿ Â̇ÚÂÙ· ÂÂÈÛfi‰È· ÌÔÚ› Ó· ˘ÔÛËÌ·›ÓÔ˘Ó ˘¤ÚÙ·ÛË ÓÂÊÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜. ∂›Û˘, ÈÛÙÔÚÈÎfi ÚÔˆÚfiÙËÙ·˜ Î·È ÔÌÊ·ÏÈÎÔ‡ ηıÂÙ‹Ú· ÌÔÚ› Ó· ˘ÔÎÚ‡ÙÂÈ ÓÂÊÚÈο-·ÁÁÂȷο ÚÔ‚Ï‹Ì·Ù·. ∞˘Ùfi ·ÔÎÙ¿ ÌÂÁ·Ï‡ÙÂÚË ÛÔ˘‰·ÈfiÙËÙ· Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ô˘ Ë ÂÈ‚›ˆÛË ÌÈÎÚÒÓ ÚÔÒÚˆÓ Î·È ÚÔ‚ÏËÌ·ÙÈÎÒÓ ÓÂÔÁÓÒÓ ¤¯ÂÈ ‚ÂÏÙȈı› ÛËÌ·ÓÙÈο Î·È ˆ˜ ÂÎ ÙÔ‡ÙÔ˘ ÂÚÈÛÛfiÙÂÚ· ÚfiˆÚ· Î·È ÓÂÔÁÓ¿ Û ۯ¤ÛË Ì ÙÔ ·ÚÂÏıfiÓ ÌÔÚ› Ó· Â›Ó·È ˘ÂÚÙ·ÛÈο. ∞ÎfiÌË, ·‰˘Ó·Ì›· „ËÏ¿ÊËÛ˘ ÌËÚÈ·›ˆÓ Ô‰ËÁ› Û ‰È¿ÁÓˆÛË ÛÙ¤ÓˆÛ˘ ÈÛıÌÔ‡ ·ÔÚÙ‹˜. O ·ÏÁfiÚÈıÌÔ˜ ÛÙÔ ™¯‹Ì· 1 ‰›ÓÂÈ Ì›· Û˘ÓÔÙÈ΋ ÂÈÎfiÓ· Ù˘ ‰ÈÂÚ‡ÓËÛ˘ Î·È Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ˘¤ÚÙ·Û˘. ¶·Ú·ÎÏÈÓÈÎfi˜ ¤ÏÂÁ¯Ô˜ ∞Ó ÙÔ ÈÛÙÔÚÈÎfi Î·È Ë ÎÏÈÓÈ΋ ÂͤٷÛË Î·ıÈÛÙÔ‡Ó ÚÔÊ·Ó¤˜ fiÙÈ Ë ˘¤ÚÙ·ÛË Â›Ó·È ‰Â˘ÙÂÚÔ·ı‹˜, Ô
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
Paediatriki 2001;64:448-459
ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ Ú¤ÂÈ Ó· ηÙ¢ı‡ÓÂÙ·È ÛÙË ‰ÈÂÚ‡ÓËÛË ÙÔ˘ ·ÈÙ›Ô˘. ∞Ó Èı·ÓÔÏÔÁÂ›Ù·È ÚˆÙÔ·ı‹˜ ˘¤ÚÙ·ÛË, ÙfiÙ ÛÙȘ ÚÒÙ˜ ÁÂÓÈΤ˜ ÂÍÂÙ¿ÛÂȘ Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÌfiÓÔ Ï›Á˜ ‚·ÛÈΤ˜, fiˆ˜ ÁÂÓÈ΋ ·›Ì·ÙÔ˜, ∆∫∂, ÁÂÓÈ΋ Ô‡ÚˆÓ Î·È Î·ÏÏȤÚÁÂÈ· Ô‡ÚˆÓ, Ô˘Ú›· ÎÚ·ÙÈÓ›ÓË, ۿί·ÚÔ, ËÏÂÎÙÚÔχÙ˜, ·Û‚¤ÛÙÈÔ, Ô˘ÚÈÎfi Ô͇, ÏÈȉfiÁÚ·ÌÌ·, ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ Î·È ˘ÂÚ˯ÔηډÈÔÁÚ·ÊÈ΋ ÌÂϤÙË (3,13). ∂¿Ó Ë ˘¤ÚÙ·ÛË Â›Ó·È ÛÔ‚·Ú‹ (∞¶≥99˘ ∂£) ‹ Â¿Ó Ë ∞¶ ·Ú·Ì¤ÓÂÈ ÌÂٷ͇ 95˘-99˘ ∂£ ·Ú¿ ÙË ÌË Ê·Ú̷΢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË ¤Ú·Ó ÙˆÓ 6 ÌËÓÒÓ, ÙfiÙ ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÏÂÙÔÌÂÚ¤ÛÙÂÚÔ˜ ¤ÏÂÁ¯Ô˜ Ô˘ ı· ÂÚÈÏ·Ì‚¿ÓÂÈ, ÂÎÙfi˜ ÙˆÓ fiÛˆÓ ‹‰Ë ·Ó·Ê¤ÚıËηÓ, Ù· ÂÍ‹˜: ¯ÔÏËÛÙÂÚfiÏË, ËÏÂÎÙÚÔÊfiÚËÛË ÏÈÔÚˆÙÂ˚ÓÒÓ, ÙÚÈÁÏ˘ÎÂÚ›‰È· Î·È Ô˘ÚÈÎfi Ô͇, ÙÔ ÔÔ›Ô Û˘¯Ó¿ ‚Ú›ÛÎÂÙ·È ·˘ÍË̤ÓÔ Û ӤԢ˜ ·ÓıÚÒÔ˘˜ Ì ˘¤ÚÙ·ÛË. ∂›Û˘, ·/· ıÒÚ·ÎÔ˜, ∏∫° Î·È ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜, Ù· ÔÔ›· ÂÈÚÔÛı¤Ùˆ˜ ı· ‚ÔËı‹ÛÔ˘Ó ÛÙËÓ ÂÎÙ›ÌËÛË ÙˆÓ Ù˘¯fiÓ ÂÈÙÒÛÂˆÓ Ù˘ ˘¤ÚÙ·Û˘ ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ·. ¢ÂÓ
˘¿Ú¯ÂÈ ÔÌÔʈӛ· fiÛÔÓ ·ÊÔÚ¿ ÛÙÔÓ ÂÚ·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô Ù˘ ÓÂÊÚÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, ηıÒ˜ Î·È ÛÙÔÓ ÂÓ‰ÂÈÎÓ˘fiÌÂÓÔ ÂÓ‰ÔÎÚÈÓÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô (5,11). ∞fi ÌÂÚÈÎÔ‡˜ Û˘ÁÁÚ·Ê›˜ ÚÔÙ›ÓÂÙ·È Ë ·Ú·ÔÌ‹ ÙÔ˘ ·È‰ÈÔ‡ Û ÂȉÈÎÔ‡˜, ÂÓÒ ¿ÏÏÔÈ Û˘ÓÈÛÙÔ‡Ó ÓÂÊÚÔÁÚ¿ÊËÌ·, ÓÂÊÚÈ΋ ·ÁÁÂÈÔÁÚ·Ê›· Î·È ÚÔÛ‰ÈÔÚÈÛÌfi ÙÔ˘ ‰Ú·ÛÙÈÎÔ‡ ÎÏ¿ÛÌ·ÙÔ˜ ÚÂÓ›Ó˘ ·Ó Èı·ÓÔÏÔÁÂ›Ù·È ÓÂÊÚ·ÁÁÂȷ΋ ÓfiÛÔ˜, ηıÒ˜ Î·È ¤ÏÂÁ¯Ô ηÙ¯ÔÏ·ÌÈÓÒÓ Î·È ÛÙÂÚÔÂȉÒÓ ·›Ì·ÙÔ˜ Î·È Ô‡ÚˆÓ. ∆¤ÏÔ˜, ‰ÂÓ ı· Ú¤ÂÈ Ó· ·Ú·Ï›ÂÙ·È ¤Ó· ÙÂÛÙ Î˘‹Ûˆ˜ Û ¤Ê˂˜ Ì ˘¤ÚÙ·ÛË. £Âڷ›· ™ÎÔfi˜ Ù˘ ıÂڷ›·˜ Â›Ó·È Ë ÂÏ¿ÙÙˆÛË Ù˘ ∞¶ οو ·fi ÙËÓ 95Ë ∂£. ¢ÂÓ ˘¿Ú¯Ô˘Ó ÚÔ˜ ÙÔ ·ÚfiÓ ·ÓÙÈÎÂÈÌÂÓÈο ‰Â‰Ô̤ӷ ÛÙ· ÔÔ›· ÌÔÚ› Ó· ‚·ÛÈÛÙ› ÙÔ Â›‰Ô˜ Ù˘ Ê·Ú̷΢ÙÈ΋˜ ·ÁˆÁ‹˜ Ô˘ ı· ¯ÔÚËÁËı› Û ·È‰È¿ Ì ˘¤ÚÙ·ÛË, Ô‡ÙÂ Â›Ó·È ÁÓˆÛÙfi ·Ó Ù· ÔʤÏË Â›Ó·È ÌÂÁ·Ï‡ÙÂÚ· ÙˆÓ ·ÚÂÓÂÚÁÂÈÒÓ ÛÙȘ ËÏÈ˘ ·˘Ù¤˜. ŒÙÛÈ, Ë ÌË Ê·Ú̷΢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË, ÌÂ
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455
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
ÙÔ˘˜ ÂÏ¿¯ÈÛÙÔ˘˜ ÎÈÓ‰‡ÓÔ˘˜ Ô˘ ÂÌÂÚÈÎÏ›ÂÈ, Ê·›ÓÂÙ·È Ó· Â›Ó·È Ë ÚÒÙË ÂÈÏÔÁ‹ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ڈÙÔ·ı‹ ˘¤ÚÙ·ÛË Î·È Û˘Ó‹ıˆ˜ ·ÓÙ·ÔÎÚ›ÓÂÙ·È Û ·˘Ù‹ ÙÔ 85% ÙˆÓ ·È‰ÈÒÓ (12,13). ∞Ó ‰ÂÓ ˘¿ÚÍÂÈ ·ÓÙ·fiÎÚÈÛË ‹ ·Ó ˘¿Ú¯ÂÈ ÛÔ‚·Ú‹ ˘¤ÚÙ·ÛË, ··ÈÙÂ›Ù·È Ê·Ú̷΢ÙÈ΋ ·Ú¤Ì‚·ÛË (3). ∞Ó Ë ·‡ÍËÛË Ù˘ ∞¶ Â›Ó·È Ôχ ÌÂÁ¿ÏË (‚·ÚÈ¿ ˘¤ÚÙ·ÛË), Ë ·ÓÙÈÌÂÙÒÈÛË Ú¤ÂÈ Ó· Â›Ó·È ¿ÌÂÛË. ∏ ÌË Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ ÂÚÈÏ·Ì‚¿ÓÂÈ: ·) O‰ËÁ›Â˜ ‰È·ÙÚÔÊ‹˜ ÁÈ· ·ÒÏÂÈ· ‚¿ÚÔ˘˜, ‰ÈfiÙÈ Ôχ Û˘¯Ó¿ Ù· ·È‰È¿ ·˘Ù¿ Â›Ó·È ·¯‡Û·Úη. ∏ ‰È·ÙÚÔÊ‹ Ú¤ÂÈ Ó· Â›Ó·È ¯·ÌËÏ‹ Û ÏÈ·Ú¿ (ȉȷ›ÙÂÚ· Û ÎÂÎÔÚÂṲ̂ӷ ÏÈ·Ú¿ Ôͤ·) Î·È ˘„ËÏ‹˜ ÂÚÈÂÎÙÈÎfiÙËÙ·˜ Û ›Ó˜, Ì ÊÚ¤Ûη ÊÚÔ‡Ù· Î·È Ï·¯·ÓÈο. ∏ ÂÈÌfiÚʈÛË Î·È Ë Û˘ÌÌÂÙÔ¯‹ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ÛÙÔ˘˜ ηÓfiÓ˜ ÛˆÛÙ‹˜ ‰È·ÙÚÔÊ‹˜ Â›Ó·È ÛËÌ·ÓÙÈ΋. ¶·Ú·ÔÌ‹ Û ‰È·ÈÙÔÏfiÁÔ Á›ÓÂÙ·È ·Ó ·˘Ùfi ÎÚÈı› ··Ú·›ÙËÙÔ. Œ¯ÂÈ ‰È·ÈÛÙˆı› fiÙÈ Î·È Ì¤ÙÚÈ· ÂÏ¿ÙÙˆÛË ÙÔ˘ ‚¿ÚÔ˘˜ Û˘Óԉ‡ÂÙ·È ·fi ÂÏ¿ÙÙˆÛË Ù˘ ∞¶. ‚) ∂Ï¿ÙÙˆÛË ÙÔ˘ ÚÔÛÏ·Ì‚·ÓfiÌÂÓÔ˘ Ì ÙȘ ÙÚÔʤ˜ ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ Î·È ·‡ÍËÛË ÙÔ˘ ÚÔÛÏ·Ì‚·ÓfiÌÂÓÔ˘ Î·Ï›Ô˘. Á) ¶ÚfiÛÏË„Ë Â·ÚÎÔ‡˜ ÔÛfiÙËÙ·˜ ·Û‚ÂÛÙ›Ô˘. ‰) ™˘ÛÙËÌ·ÙÈ΋ ۈ̷ÙÈ΋ ¿ÛÎËÛË. ∏ ¿ÛÎËÛË ˆÊÂÏ› ÔÏÏ·Ï¿ ÙÔ ·È‰› Î·È ‰ÂÓ ˘¿Ú¯ÂÈ ·ÓÙ¤Ó‰ÂÈÍË ÛÙË Û˘ÌÌÂÙÔ¯‹ ÙÔ˘, ÂÎÙfi˜ Â¿Ó ÙÔ ·È‰› ¿Û¯ÂÈ ·fi ηډȷ΋ ÓfiÛÔ ÁÈ· ÙËÓ ÔÔ›· ·ÓÙÂӉ›ÎÓ˘Ù·È Ë Á˘ÌÓ·ÛÙÈ΋ ‹ ¤¯ÂÈ ˘ÂÚÙ·ÛÈ΋ ÎÚ›ÛË (30,31). ∞Ó Û˘Ó˘¿Ú¯ÂÈ ‚Ï¿‚Ë ÙÂÏÈÎÒÓ ÔÚÁ¿ÓˆÓ, Û˘ÓÈÛÙ¿Ù·È Û˘ÓÂÎÙ›ÌËÛË Ì ÙÔÓ Î·Ú‰ÈÔÏfiÁÔ (32). ªÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ Ô Û˘Ó‰˘·ÛÌfi˜ ‰›·ÈÙ·˜ Î·È ¿ÛÎËÛ˘ Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚÔ˜ ÛÙËÓ ÂÏ¿ÙÙˆÛË Ù˘ ∞¶. Â) ¢È·ÎÔ‹ ηӛÛÌ·ÙÔ˜ Î·È Î·Ù¿¯ÚËÛ˘ ·ÏÎÔÔÏÔ‡¯ˆÓ ÔÙÒÓ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜. ÛÙ) ¶Ú¤ÂÈ Ó· ÙÔÓ›˙ÂÙ·È ÛÙÔ˘˜ ·ÛıÂÓ›˜ fiÙÈ ··ÁÔÚ‡ÂÙ·È Ë Ï‹„Ë Ê·Ú̷΢ÙÈÎÒÓ Ô˘ÛÈÒÓ, ÂÎÙfi˜ ÂÎÂ›ÓˆÓ Ô˘ Û˘ÛÙ‹ÓÔÓÙ·È ·fi ÁÈ·ÙÚfi. ∞ÎfiÌË Î·È Ù· ·ÔÛ˘ÌÊÔÚËÙÈο ÙÔ˘ ÚÈÓÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘ ÌÔÚ› Ó· ·˘Í‹ÛÔ˘Ó ÙËÓ ∞¶. ˙) ÃÚËÛÈÌÔÔ›ËÛË „˘¯ÔÏÔÁÈÎÒÓ ÌÂıfi‰ˆÓ ÙÚÔÔÔ›ËÛ˘ Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜. ∏ Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ Û˘ÛÙ‹ÓÂÙ·È: 1) ÛÂ Û˘Ìو̷ÙÈÎÔ‡˜ ·ÛıÂÓ›˜, 2) Û ·˘ÙÔ‡˜ Ô˘ ¤¯Ô˘Ó ÛÔ‚·Ú‹ ˘¤ÚÙ·ÛË, ‰ËÏ·‰‹ Ë ‰È·ÛÙÔÏÈ΋ ›ÂÛË Â›Ó·È 12 mmHg ˘„ËÏfiÙÂÚË ‹ Ë Û˘ÛÙÔÏÈ΋ Â›Ó·È 25 mmHg ˘„ËÏfiÙÂÚË Ù˘ 99˘ ∂£, 3) Û ·È‰È¿ Ô˘ ¤¯Ô˘Ó ¤Ó‰ÂÈÍË ‚Ï¿‚˘ ÙÂÏÈÎÒÓ ÔÚÁ¿ÓˆÓ Î·È 4) Û ·ÛıÂÓ›˜ Ì ÈÓÛÔ˘ÏÈÓÔÂÍ·ÚÙÒÌÂÓÔ Û·Î¯·ÚÒ‰Ë ‰È·‚‹ÙË. º·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ Û˘ÛÙ‹ÓÂÙ·È, ›Û˘, Û ˘ÂÚÙ·ÛÈο ·È‰È¿ Ô˘ ‰ÂÓ ·ÓÙ·ÔÎÚ›ıËÎ·Ó ÛÙË ÌË Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ Î·È ÛÙ· ÔÔ›·
456
Paediatriki 2001;64:448-459
Û˘Ó˘¿Ú¯Ô˘Ó Î·È ¿ÏÏÔÈ ÂÈ‚·Ú˘ÓÙÈÎÔ› ·Ú¿ÁÔÓÙ˜, fiˆ˜ ÙÔ ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ÁÈ· ˘¤ÚÙ·ÛË ‹ ¿ÏÏ· ÚÔ‚Ï‹Ì·Ù· ·fi ÙÔ Î˘ÎÏÔÊÔÚÈÎfi. ∞Ó ˘¿Ú¯ÂÈ ·ÌÊÈ‚ÔÏ›· ÁÈ· ÙËÓ ·Ó·ÁηÈfiÙËÙ· Ù˘ Ê·Ú̷΢ÙÈ΋˜ ·ÁˆÁ‹˜, ÙÔ ·È‰› Â›Ó·È ·Û˘Ìو̷ÙÈÎfi, ¯ˆÚ›˜ ÚˆÙÂ˚ÓÔ˘Ú›·, ηډÈÔÌÂÁ·Ï›· ‹ ˘ÂÚ˯ÔÁÚ·ÊÈ΋ ¤Ó‰ÂÈÍË ˘ÂÚÙÚÔÊ›·˜ Ù˘ ·ÚÈÛÙÂÚ¿˜ ÎÔÈÏ›·˜, ÙfiÙ ›Ûˆ˜ Â›Ó·È ÚÔÙÈÌfiÙÂÚÔ Ó· ηı˘ÛÙÂÚ‹ÛÂÈ Ë ¤Ó·ÚÍË Ù˘ Ê·Ú̷΢ÙÈ΋˜ ·ÁˆÁ‹˜. ™˘Ó‹ıˆ˜ Á›ÓÂÙ·È ¤Ó·ÚÍË ıÂڷ›·˜ Ì ¤Ó· ÌfiÓÔ ·ÓÙÈ˘ÂÚÙ·ÛÈÎfi (‚-·Ó·ÛÙÔϤ· ‹ ·ÓÙ·ÁˆÓÈÛÙ‹ ·Û‚ÂÛÙ›Ô˘ ‹ ‰ÈÔ˘ÚËÙÈÎfi) Û ¯·ÌËÏ‹ ‰fiÛË. ∂› ÌË ÂϤÁ¯Ô˘ Ù˘ ˘¤ÚÙ·Û˘, ˘Ôηı›ÛÙ·Ù·È ·˘Ùfi ·fi ¿ÏÏÔ ·ÓÙȸÂÚÙ·ÛÈÎfi Ù˘ ›‰È·˜ ηÙËÁÔÚ›·˜ ‹ ÚÔÛÙ›ıÂÙ·È Î·È ‰Â‡ÙÂÚÔ Ê¿ÚÌ·ÎÔ (3,12,28). OÈ Î·ÙËÁÔڛ˜ ÙˆÓ Ê·ÚÌ¿ÎˆÓ ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 4. ™ÙËÓ ÂÚ›ÙˆÛË ˘ÂÚÙ·ÛÈ΋˜ ÎÚ›Û˘, ÙÔ ·È‰› Ú¤ÂÈ Ó· ÂÈÛ·¯ı› ÛÙÔ ÓÔÛÔÎÔÌ›Ô. ∏ ıÂڷ›· Â›Ó·È ÚÔÙÈÌfiÙÂÚÔ Ó· Á›ÓÂÈ ÂÓ‰ÔÊÏ‚›ˆ˜, Û˘Ó‹ıˆ˜ ÌÂ Û˘Ó¯‹ ¤Á¯˘ÛË, ‰ÈfiÙÈ ¤ÙÛÈ ÂϤÁ¯ÂÙ·È Î·Ï‡ÙÂÚ· Ô Ú˘ıÌfi˜ ¯ÔÚ‹ÁËÛ˘ Ê·Ú̿ΈÓ. ∞fiÙÔÌË ÂÏ¿ÙÙˆÛË Ù˘ ∞¶ Ú¤ÂÈ Ó· ·ÔʇÁÂÙ·È ‰ÈfiÙÈ ÂÌÂÚÈÎÏ›ÂÈ ÎÈÓ‰‡ÓÔ˘˜, ÁÈ’ ·˘Ùfi Î·È ¯ÚÂÈ¿˙ÂÙ·È Û˘Ó¯‹˜ ηٷÁÚ·Ê‹ Ù˘ ›ÂÛ˘. ∆Ô ÓÈÙÚÔÚˆÛÛÈÎfi Ó¿ÙÚÈÔ (0,5-8 Ìg/kg/min) Î·È Ë Ï·‚ÂÙ·ÏfiÏË (0,4-1 mg/kg/‰fiÛË), Ô˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ˘ÂÚÙ·ÛÈ΋ ÎÚ›ÛË ÏfiÁˆ Ê·ÈÔ¯ÚˆÌÔ΢ÙÙÒÌ·ÙÔ˜, Â›Ó·È ‰‡Ô ·fi Ù· ÂÓ‰ÂÈÎÓ˘fiÌÂÓ· Ê¿Ú̷η (12,28,33). ∏ ˘‰Ú·Ï·˙›ÓË Ô˘ ˘‹ÚÍ ÙÔ Ê¿ÚÌ·ÎÔ ÂÎÏÔÁ‹˜ ÁÈ· ÔÏÏ¿ ¯ÚfiÓÈ·, Ù›ÓÂÈ Ï¤ÔÓ Ó· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÏÈÁfiÙÂÚÔ, ‰ÈfiÙÈ Û ۇÁÎÚÈÛË Ì ٷ ÓÂfiÙÂÚ· Ê¿Ú̷η Â›Ó·È ÏÈÁfiÙÂÚÔ ·ÔÙÂÏÂÛÌ·ÙÈ΋ Î·È ¤¯ÂÈ ÂÚÈÛÛfiÙÂÚ˜ ·ÚÂÓ¤ÚÁÂȘ (28,29). ¶ÚfiÁÓˆÛË ¢ÂÓ ˘¿Ú¯Ô˘Ó ÂÚÁ·Û›Â˜ Ô˘ Ó· ¤¯Ô˘Ó ÔÏÔÎÏËÚÒÛÂÈ ÙË ÌÂϤÙË ÙˆÓ ÂÈÙÒÛÂˆÓ Ù˘ ÚˆÙÔ·ıÔ‡˜ ˘¤ÚÙ·Û˘ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ. ∫·Ì›· ÔÌ¿‰· ·È‰ÈÒÓ ‰ÂÓ ¤¯ÂÈ ·Ú·ÎÔÏÔ˘ıËı› ·ÎfiÌË ÁÈ· ÙfiÛÔ Ì·ÎÚfi ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·, fiÛÔ ··ÈÙÂ›Ù·È Ì¤¯ÚÈ Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ÔÈ ÂÈÏÔΤ˜ Ù˘ ˘¤ÚÙ·Û˘. ∞fi ÌÂϤÙ˜ Û ÂÓËÏ›ÎÔ˘˜ Â›Ó·È ÁÓˆÛÙfi fiÙÈ Ë ·ÓÙÈÌÂÙÒÈÛË Ù˘ ˘¤ÚÙ·Û˘ ÂÏ·ÙÙÒÓÂÈ ÙË Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ·ÔÊÚ·ÎÙÈ΋˜ ·ÚÙËÚÈÔÛÎÏËÚ˘ÓÙÈ΋˜ ·ÁÁÂȷ΋˜ ÓfiÛÔ˘, ÓÂÊÚÈ΋˜ ·Ó¿ÚÎÂÈ·˜ Î·È ÂÁÎÂÊ·ÏÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ, ÂÓÒ ‰ÂÓ Ê·›ÓÂÙ·È Ó· ÂËÚ¿˙ÂÈ ÛËÌ·ÓÙÈο ÙË Û˘¯ÓfiÙËÙ· ÂÌÊÚ¿ÁÌ·ÙÔ˜ ÙÔ˘ Ì˘Ôηډ›Ô˘. ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi Èı·ÓÒ˜ ÔÊ›ÏÂÙ·È ÛÙÔ fiÙÈ Ë ÛÙÂÊ·ÓÈ·›· ÓfiÛÔ˜ ¤¯ÂÈ ‹‰Ë ÂÁηٷÛÙ·ı› Ôχ ÚÈÓ ·Ó·ÁÓˆÚÈÛÙ› Ë ˘¤ÚÙ·ÛË Î·È ·Ú¯›ÛÂÈ Ë ıÂڷ¢ÙÈ΋ Ù˘ ·ÓÙÈÌÂÙÒÈÛË. ∞˘Ùfi οÓÂÈ ÂÈÙ·ÎÙÈ΋ ÙËÓ ·Ó¿ÁÎË Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘. Œ¯ÂÈ, ›Û˘, ηٷ‰Âȯı› ·fi ÌÂϤÙ˜ fiÙÈ ·È‰È¿ Î·È Î˘Ú›ˆ˜ ¤ÊË‚ÔÈ Ì ˘„ËÏ‹ ∞¶ ¤¯Ô˘Ó
¶·È‰È·ÙÚÈ΋ 2001;64:448-459
ÌÂÁ·Ï‡ÙÂÚË Èı·ÓfiÙËÙ· Ó· ÂÌÊ·Ó›ÛÔ˘Ó ·ÚÁfiÙÂÚ· ˘¤ÚÙ·ÛË. ™ÙË ‰Â˘ÙÂÚÔ·ı‹ ˘¤ÚÙ·ÛË, Ë ÚfiÁÓˆÛË Â›Ó·È ÛÙÂÓ¿ Û˘Ó‰Â‰Â̤ÓË Ì ÙËÓ ˘ÔΛÌÂÓË ÓfiÛÔ Î·È ÂÍ·ÚÙ¿Ù·È ·fi ·˘Ù‹Ó. ¶ÚfiÏË„Ë ∏ ÚˆÙÔ·ı‹˜ ˘¤ÚÙ·ÛË Û˘Û¯ÂÙ›˙ÂÙ·È ıÂÙÈο Ì ÙËÓ ·ÚÙËÚÈÔÛÎÏ‹Ú˘ÓÛË Î·È Ì·˙› Ì ÙË ‰˘ÛÏÈȉ·ÈÌ›· Î·È ÙÔ Î¿ÓÈÛÌ·, ·ÔÙÂÏÔ‡Ó Ù· ÙÚ›· ÛËÌ·ÓÙÈÎfiÙÂÚ· ÁÂÓÂÛÈÔ˘ÚÁ¿ ·›ÙÈ· Ù˘ ÛÙÂÊ·ÓÈ·›·˜ ÓfiÛÔ˘, ÙˆÓ ÂÁÎÂÊ·ÏÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ Î·È Ù˘ ÓÂÊÚÈ΋˜ ·Ó¿ÚÎÂÈ·˜. ∞ÎfiÌË Î·È ÌÈÎÚÔ‡ ‚·ıÌÔ‡ ˘¤ÚÙ·ÛË Ê·›ÓÂÙ·È fiÙÈ ÌÔÚ› Ó· ¤¯ÂÈ ÂÈÙÒÛÂȘ ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· (18). ∏ ·ÚÔ˘Û›· ·¯˘Û·ÚΛ·˜ ‰Ú· Û·Ó ¤Ó·˜ ·ÎfiÌË ÂÈ‚·Ú˘ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜. OÈ ÂÈ‚·Ú˘ÓÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ‰Â, Ù›ÓÔ˘Ó Ó· ÂÓÈÛ¯‡Ô˘Ó Ô ¤Ó·˜ ÙÔÓ ¿ÏÏÔ Î·È Ó· ÂȉÚÔ‡Ó ·ıÚÔÈÛÙÈο. °È· ÙÔ ÏfiÁÔ ·˘Ùfi, Ù· ÛËÌ·ÓÙÈÎfiÙÂÚ· ÚÔÏËÙÈο ̤ÙÚ· Ú¤ÂÈ Ó· ¤¯Ô˘Ó ·ÚÈÔ ÛÙfi¯Ô ¤ÓÙ ‚·ÛÈο ‰›·: ·) ÙËÓ ·¯˘Û·ÚΛ·, ‚) ÙËÓ ˘¤ÚÙ·ÛË, Á) ÙËÓ Î·Ï‹ ηډȷÁÁÂȷ΋ ηٿÛÙ·ÛË, ‰) ÙËÓ ˘ÂÚ¯ÔÏËÛÙÂÚÔÏ·ÈÌ›· Î·È Â) ÙÔ Î¿ÓÈÛÌ· (34). OÈ Î·Ú‰È·ÁÁÂȷΤ˜ ÓfiÛÔÈ, ·Ú¿ ÙËÓ ÂÏ¿ÙÙˆÛË Ù˘ Û˘¯ÓfiÙËÙ¿˜ ÙÔ˘˜, ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ·Ú·Ì¤ÓÔ˘Ó ÔÈ ÛËÌ·ÓÙÈÎfiÙÂÚ˜ ·Èٛ˜ ı·Ó¿ÙÔ˘ ÛÙÔ ‰˘ÙÈÎfi ÎfiÛÌÔ. ¶·Ú¿ÁÔÓÙ˜ Ô˘ ηıÔÚ›˙Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ˘¤ÚÙ·Û˘ ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ›ӷÈ: ·) ˘„ËÏ‹ Ê˘ÛÈÔÏÔÁÈ΋ ∞¶ (>90˘ ∂£) Û ·ÓÂÈÏËÌ̤Ó˜ ÌÂÙÚ‹ÛÂȘ, ‚) ∞¶ ÛÙ·ıÂÚ¿ ÛÙ· ·ÓÒÙÂÚ· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· (>70˘ ∂£) ‹ ∞¶ Ô˘ ηٿ Ê¿ÛÂȘ ÍÂÂÚÓ¿ ÙËÓ 95Ë ∂£, Á) ·¯˘Û·ÚΛ·, ÂȉÈÎfiÙÂÚ· ·¯‡Û·Úη ·È‰È¿, ·¯˘Û¿ÚÎˆÓ ÁÔÓ¤ˆÓ). ∂›Ó·È ÁÓˆÛÙfi fiÙÈ Ë ·¯˘Û·ÚΛ· (΢ڛˆ˜ ·˘Ù‹ Ì ÎÂÓÙÚÈ΋ ηٷÓÔÌ‹) Û˘Ó‰¤ÂÙ·È Ì ˘¤ÚÙ·ÛË, ‰˘ÛÏÈȉ·ÈÌ›·, ·˘ÍË̤ÓÔ Û¿Î¯·ÚÔ ·›Ì·ÙÔ˜, ·˘ÍË̤ӷ ›‰· ÈÓÛÔ˘Ï›Ó˘, ÂÓÒ fiÙ·Ó ·Ú¯›˙ÂÈ ÎÔÓÙ¿ ÛÙËÓ ÂÊ˂›·, Ù›ÓÂÈ Ó· Û˘Ó¯›˙ÂÙ·È ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ Î·È Û˘Óԉ‡ÂÙ·È ·fi ·˘ÍË̤ÓË Èı·ÓfiÙËÙ· ¿ÏÏˆÓ ÓfiÛˆÓ ÛÙ· ¿ÙÔÌ· ·˘Ù¿ (35,36), ‰) ˘ÂÚÏÈȉ·ÈÌ›· ‹ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ˘ÂÚÏÈȉ·ÈÌ›·˜, ÂȉÈο Â¿Ó Û˘Ó˘¿Ú¯ÂÈ Î·È ÛÙÂÊ·ÓÈ·›· ÓfiÛÔ˜ ‹ ÂÁÎÂÊ·ÏÈÎfi, Â) ۷ί·Ú҉˘ ‰È·‚‹Ù˘, ÛÙ) ˘¤ÚÙ·ÛË Ô˘ ··ÈÙ› Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹, Û ‰‡Ô ‹ ÂÚÈÛÛfiÙÂÚ· ̤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜, ˙) ·˘ÍË̤ÓË ÚfiÛÏË„Ë ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘, Ë) ¤ÏÏÂÈ„Ë ÛˆÌ·ÙÈ΋˜ ¿ÛÎËÛ˘ Î·È ı) ηٿ¯ÚËÛË ·ÏÎÔfiÏ˘ Î·È Î¿ÓÈÛÌ· (¤ÊË‚ÔÈ). ¶ÔÏÏÔ› ·fi ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘, fiˆ˜ ÚÔ·ÙÂÈ ·fi ÌÂϤÙ˜, Ù›ÓÔ˘Ó Ó· Û˘Ó˘¿Ú¯Ô˘Ó ·˘Í¿ÓÔÓÙ·˜ Ô ¤Ó·˜ ÙÔÓ ¿ÏÏÔ, Ì ·ÔÙ¤ÏÂÛÌ· Ó· Û˘Ó¯›˙ÔÓÙ·È ÂÊ’ fiÚÔ˘ ˙ˆ‹˜ (8).
Paediatriki 2001;64:448-459
¶›Ó·Î·˜ 4. ∫·ÙËÁÔڛ˜ Ê·ÚÌ¿ÎˆÓ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ˘¤ÚÙ·Û˘ ¢ÈÔ˘ÚËÙÈο ñ ¶·Ú·ÙÂٷ̤Ó˘ ‰Ú¿Û˘ - ÊÔ˘ÚÔÛÂÌ›‰Ë=1, [max=12] [2-6 ¢∏¢] ñ º¿Ú̷η Ô˘ ‰È·ÙËÚÔ‡Ó ÙÔ Î¿ÏÈÔ: - ™ÈÚÔÓÔÏ·ÎÙfiÓË=1, [max=3] [1-2 ¢∏¢] ñ £ÂÈ·˙ȉÈο ‰ÈÔ˘ÚËÙÈο - À‰ÚԯψÚÔıÂÈ·˙›‰Ë=1, [max=2-3] [2 ¢∏¢] ∞‰ÚÂÓÂÚÁÈÎÔ› ·Ó·ÛÙÔÏ›˜ ñ ‚-·‰ÚÂÓÂÚÁÈÎÔ› ·ÓÙ·ÁˆÓÈÛÙ¤˜ - ∞ÙÂÓÔÏfiÏË=1, [max=8] [1-2 ¢∏¢] - ¶ÚÔ·ÓÔÏfiÏË=1, [max=8] [2-3¢∏¢] ∫ÂÓÙÚÈÎÔ› ·‰ÚÂÓÂÚÁÈÎÔ› ·Ó·ÛÙÔÏ›˜ ñ ·-·‰ÚÂÓÂÚÁÈÎÔ› ·ÓÙ·ÁˆÓÈÛÙ¤˜ - ¶Ú·˙ÔÛ›ÓË=0,05-0,1, [max=0,5] [3-4 ¢∏¢] AÁÁÂÈԉȷÛÙ·ÏÙÈο ñ ¶·Ú¿ÁÔÓÙ˜ Ô˘ ¯·Ï·ÚÒÓÔ˘Ó ÙȘ Ï›˜ Ì˘˚Τ˜ ›Ó˜ - ¡ÈÙÚÔÚˆÛÛÈÎfi ¡¿ÙÚÈÔ=0,5 Ìg/kg/‰fiÛË [max=8] ñ ∞ÓÙ·ÁˆÓÈÛÙ¤˜ ·Û‚ÂÛÙ›Ô˘ - ¡ÂʉțÓË=1, [max=2], [4-6 ¢∏¢] ñ ∞Ó·ÛÙÔÏ›˜ ÙÔ˘ ÌÂÙ·ÙÚÂÙÈÎÔ‡ ÂÓ˙‡ÌÔ˘ Ù˘ ·ÁÁÂÈÔÙÂÓÛ›Ó˘ - ∂Ó·Ï·Ú›ÏË=0,15, [max=?] [1-2 ¢∏¢] - ∫·ÙÔÚ›ÏË=1,5, [max=6] [3 ¢∏¢] OÈ ·Ó·ÊÂÚfiÌÂÓ˜ ‰fiÛÂȘ Â›Ó·È Û mg/kg/Ë̤ڷ, ÂÎÙfi˜ ·Ó ˘¿Ú¯ÂÈ ‰È·ÊÔÚÂÙÈ΋ ¤Ó‰ÂÈÍË. [Max: ̤ÁÈÛÙË ‰fiÛË] [¢∏¢: ‰È·ÈÚÂ̤Ó˜ ËÌÂÚ‹ÛȘ ‰fiÛÂȘ]
O ÚfiÏÔ˜ ÙÔ˘ ·È‰È¿ÙÚÔ˘ ™ÙÔ˘˜ ·È‰È¿ÙÚÔ˘˜ ·ʛÂÙ·È Ë ÚfiÏË„Ë Ù˘ ˘¤ÚÙ·Û˘ Ì ÙȘ ηٷÛÙÚÂÙÈΤ˜ Ù˘ Û˘Ó¤ÂȘ. ∞˘Ùfi Û˘Û¯ÂÙ›˙ÂÙ·È ¿ÌÂÛ· Ì ÙÔÓ ¤ÏÂÁ¯Ô Ù˘ ∞¶ ·Ó¿ Ù·ÎÙ¿ ‰È·ÛÙ‹Ì·Ù·, ÙË ‰È¿ÁÓˆÛË Ù˘ ˘¤ÚÙ·Û˘ Î·È ÙËÓ ¤ÁηÈÚË ·ÓÙÈÌÂÙÒÈÛ‹ Ù˘. ∏ ∞ÌÂÚÈηÓÈ΋ ¶·È‰È·ÙÚÈ΋ ∂Ù·ÈÚ›· Û˘ÓÈÛÙ¿ ÂÙ‹ÛÈÔ ¤ÏÂÁ¯Ô Ù˘ ∞¶. ∂¿Ó ˘¿Ú¯Ô˘Ó ¿ÏÏÔÈ ÂÈ‚·Ú˘ÓÙÈÎÔ› ·Ú¿ÁÔÓÙ˜, Ë Ì¤ÙÚËÛË Ú¤ÂÈ Ó· Á›ÓÂÙ·È Û˘¯ÓfiÙÂÚ·. ∏ ‰È·ÙÚÔÊ‹ ·ÔÙÂÏ› ÙÔ ÛËÌ·ÓÙÈÎfiÙÂÚÔ ÂÚÈ‚·ÏÏÔÓÙÈÎfi ·Ú¿ÁÔÓÙ· Ô˘ Úԉȷı¤ÙÂÈ Û ·¯˘Û·ÚΛ·, ˘ÂÚÏÈȉ·ÈÌ›· Î·È ˘¤ÚÙ·ÛË (38). OÈ ·È‰›·ÙÚÔÈ ÌÔÚÔ‡Ó Ó· Û˘ÌÌÂÙ¤¯Ô˘Ó Û ÂÈÌÔÚʈÙÈο ÚÔÁÚ¿ÌÌ·Ù·, ·ÎfiÌË Î·È ÛÙËÓ ÚˆÙÔ‚¿ıÌÈ· Âη›‰Â˘ÛË, fiÛÔÓ ·ÊÔÚ¿ ÙË ÛˆÛÙ‹ ‰È·ÙÚÔÊ‹ Î·È ÙȘ ηıËÌÂÚÈÓ¤˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ Ô˘ ÚÔ¿ÁÔ˘Ó ÙËÓ Î·Ï‹ Ê˘ÛÈ΋ ηٿÛÙ·ÛË. ªÔÚÔ‡Ó, ›Û˘, Ó· ·›ÍÔ˘Ó ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÂÈÌfiÚʈÛË fiÏˆÓ ÙˆÓ ÌÂÏÒÓ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ÛÙÔ˘˜ ÙÔÌ›˜ ·˘ÙÔ‡˜, ÂÏ·ÙÙÒÓÔÓÙ·˜ ¤ÙÛÈ ÌÂÏÏÔÓÙÈÎÔ‡˜ ÎÈÓ‰‡ÓÔ˘˜ (39). ªÂϤÙ˜ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ fiÙ·Ó fiÏ· Ù· ̤ÏË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ Û˘ÌÌÂÙ¤¯Ô˘Ó, Ù· ·ÔÙÂϤÛÌ·Ù· fiÛÔÓ ·ÊÔÚ¿ ÙË Û˘ÌÌfiÚʈÛË Â›Ó·È Î·Ï‡ÙÂÚ·. ™ÙËÓ ∞ÌÂÚÈ΋, ÙÔ 80% ÙˆÓ ·È‰ÈÒÓ Î·Ù·Ó·ÏÒÓÂÈ ÙÚÔʤ˜ Ì ÂÚÈÂÎÙÈÎfiÙËÙ· Û ÎÂÎÔÚÂṲ̂ӷ ÏÈ·Ú¿ Ôͤ· ÌÂÁ·Ï‡ÙÂÚË Ù˘ Û˘ÓÈÛÙÒÌÂÓ˘ Î·È ÙÔ 60-70% ÌÂ
457
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ÂÚÈÛÛfiÙÂÚË Ù˘ ÂÈÙÚÂfiÌÂÓ˘ ¯ÔÏËÛÙÂÚ›ÓË. ™ÙÔ ‰˘ÙÈÎfi ÎfiÛÌÔ, Ù· ÙÂÏÂ˘Ù·›· 15-20 ¯ÚfiÓÈ·, ¤¯ÂÈ ·Ú·ÙËÚËı› ·‡ÍËÛË ÙÔ˘ ̤ÛÔ˘ fiÚÔ˘ ‚¿ÚÔ˘˜ ηٿ 10 ÎÈÏ¿ ÂÚ›Ô˘, ÂÓÒ ·Ó·Ê¤ÚÂÙ·È fiÙÈ ÙÔ 25% ÙˆÓ ·È‰ÈÒÓ ÛÙËÓ ∞ÌÂÚÈ΋ Â›Ó·È ·¯‡Û·Úη, ηıÒ˜ Î·È 1 ÛÙ· 5 ÂÏÏËÓfiÔ˘Ï· 2-12 ÂÙÒÓ. ∂›Û˘, ¤¯ÂÈ ·Ú·ÙËÚËı› ÂÏ¿ÙÙˆÛË Ù˘ ¿ıÏËÛ˘ ÙˆÓ ·È‰ÈÒÓ, Ë ÔÔ›· Èı·ÓÒ˜ ÂÓ¤¯ÂÙ·È ÛÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ ηډȷÁÁÂȷ΋˜ ÓfiÛÔ˘. O ·È‰›·ÙÚÔ˜ ÌÔÚ› Ó· ·Ú¤Ì‚ÂÈ Û˘ÛÙ‹ÓÔÓÙ·˜ ÏÈÁfiÙÂÚÔ ¯ÚfiÓÔ ÛÙËÓ ÙËÏÂfiÚ·ÛË ‹ ÙÔÓ ËÏÂÎÙÚÔÓÈÎfi ˘ÔÏÔÁÈÛÙ‹ Î·È ÂÚÈÛÛfiÙÂÚË ¿ıÏËÛË, Û˘Ì‚¿ÏÏÔÓÙ·˜ ¤ÙÛÈ ÛÙËÓ Î·Ù·ÔϤÌËÛË Î·ÎÒÓ Û˘ÓËıÂÈÒÓ. ∏ ·Ú·ÎÔÏÔ‡ıËÛË ÙÔ˘ ·È‰ÈÔ‡ ·fi ÙË Á¤ÓÓËÛ‹ ÙÔ˘ ̤¯ÚÈ ÙËÓ ÂÓËÏÈΛˆÛË ·fi ÙÔÓ ·È‰›·ÙÚÔ, ‰›ÓÂÈ Û’ ·˘ÙfiÓ ÙË ‰˘Ó·ÙfiÙËÙ· ÛËÌ·ÓÙÈ΋˜ ·Ú¤Ì‚·Û˘. Œ¯ÂÈ ˘ÔÏÔÁÈÛÙ› fiÙÈ ÙÔ 73% ÙˆÓ ∞ÌÂÚÈηÓÒÓ ·È‰È¿ÙÚˆÓ ·›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙË ‰È·ÌfiÚʈÛË Ù˘ ‰È·ÙÚÔÊ‹˜ ÙÔ˘ ·È‰ÈÔ‡ Î·È ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ fiÏË ÙËÓ ÔÈÎÔÁ¤ÓÂÈ·. µÈ‚ÏÈÔÁÚ·Ê›· 1. CHD Coronary Heart Disease. Overview and summary. Pediatrics 1992;89:S525-S536. 2. Berenson GS, Pickoff AS. Preventive cardiology and its potential influence on the early natural history of adult heart diseases: The Bogalusa Heart Study and the Heart Smart Program. Am J Med Sci 1995;301(Suppl 1):S134-S138. 3. National High Blood Pressure Education Program Working Group on Hypertension Control in children and adolescents. Update on the 1987 task force report on high blood pressure in children and adolescents: A Working Group Report from the National High Blood Pressure Education Program. Pediatrics 1996;98:649-658. 4. Portman RJ, Yetman RJ. Temporal blood pressure patterns in children. Ann NY Acad Sci 1996;783:227-241. 5. Mahan JD, Turman MA, Mentser MI. Evaluation of hematuria, proteinuria and hypertension in adolescents. Pediatr Clin N Am 1997;44:1573-1589. 6. Schieken RM. New perspectives in childhood blood pressure. Curr Opin Cardiol 1995;10:87-91. 7. Sorof MJ, Portman RJ. Ambulatory blood pressure monitoring in the pediatric patient. J Pediatr 2000;136:578-586. 8. Lenfant C, Savage PJ. The early natural history of atherosclerosis and hypertension in the young: National Institutes of Health perspectives. Am J Med Sciences 1995;310(Suppl 1):S3-S7. 9. Oalmann MC, Strong JP, Tracy RE, Malcom GT. Atherosclerosis in youth: are hypertension and the other coronary heart disease risk factors at work? Pediatr Nephrol 1997;11:99-107. 10. Berenson GS, Wattigney WA, Bao W, Srinivasan SR, Radhakrishnamurthy B. Rationale to study the early natural history of heart disease: The Bogalusa Heart Study. Am J Med Sci 1995;(Suppl 310):S22-S28. 11. Sinaiko AR. Hypertension in children. NEJM
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1996;335:1968-1973. 12. Sadowski RH, Falkner B. Hypertension in pediatric patients. Am J Kidney Dis 1996;27:305-315. 13. Hohn AR. Diagnosis and management of hypertension in childhood. Pediatric Annals 1997;26:105-110. 14. Rosner B, Prineas RJ, Loggie JMH, Daniels SR. Blood pressure normograms for children and adolescents, by height, sex, and age, in the United States. J Pediatr 1993;123:871-886. 15. Steiss JO, Rascher W. Automated blood pressure in children. Zeitschrift fur Kardiologie 1996;85(Suppl 3):81-84. 16. Hollyday MA. Is blood pressure in later life affected by events in infancy? Pediatr Nephrol 1995;9:663-666. 17. Gidding SS. A perspective on obesity. Am J Med Sci 1995;301(Suppl 1):S68-S71. 18. Bartosh SM, Aronson AJ. Childhood hypertension: An update on etiology, diagnosis and treatment. Pediatr Clin N Am 1999;46:235-253. 19. Alpert BS, Daniels SR. Editorials: Twenty-four-hour ambulatory blood pressure monitoring: Now that technology has come of age - we need to catch up. J Pediatr 1997;130:167-169. 20. Soergel M, Kirschstein M, Busch C, Danne T, Gellermann J, Holl R et al. Oscillometric twenty-four hour ambulatory blood pressure values in healthy children and adolescents: A multicenter trial including 1141 subjects. J Pediatr 1997;130:178-184. 21. Goble MM. Hypertension in infancy. Pediatr Clin N Am 1993;40:105-122. 22. Myers MM. Enduring effects of infant feeding experiences on adult pressure. Psychosom Med 1996;58:612-621. 23. Falkner B, Michel S. Blood pressure response to sodium in children and adolescents. Am J Clin Nutr 1997;65(Suppl 2):618S-621S. 24. Brouhard BH. Hypertension in children and adolescents. Clevelant Clin J Med 1995;62:21-28. 25. Schieken RM. Genetic factors that predespose the child to develop hypertension. Pediatr Clin North Am 1993;40:1-11. 26. Anderson J, Rocchini AP. Hypertension in individuals with insulin-dependent diabetes melitus. Pediatr Clin North Am 1993;40:93-104. 27. Daniels SR. The diagnosis of hypertension in children: An update. Pediatr Rev 1997;18:131-135. 28. ¢. ¶··ı·Ó·Û›Ô˘-∫ÏÒÓÙ˙·. À¤ÚÙ·ÛË: £Âڷ¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË. ¶·È‰È·ÙÚÈ΋ £Âڷ¢ÙÈ΋ ∂ÓË̤ڈÛË. 1997. ÛÂÏ. 45-50. 29. Fivush B, Neu A, Furth S. Acute hypertensive crises in children: emergencies and urgencies. Curr Opin Pediatr 1997;9:233-226. 30. Harsha DW. The benefits of physical activity in childhood. Am J Med Sci 1995;301(Suppl 1):S109-S113. 31. Alpert BS. Exercise in hypertensive children and adolescents: Any harm done? Pediatr Cardiol 1999;20:66-69. 32. Kaminer SJ, Hixon RL, Strong WB. Evaluation and recommendations for participation in athletics for children with heart disease. Curr Opin Pediatr 1995;7:595-600. 33. Flint PM, Middleton DB. Hypertension in children. NEJM 1997;336:1675.
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39. Hunter S MacD, Bao W, Berenson GS. Understanding the development of behavior risk factors for cardiovasculrar disease in the youth: The Bogalusa Heart Study. Am J Med Sci 1995;301(Suppl 1):S114-118.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 05-12-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 20-03-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∞Ó·ÛÙ·Û›· ∑ÂÚ‚Ô˘‰¿ÎË ºÚ·ÁÎÔÎÎÏËÛÈ¿˜ 55, 151 25, ¶Ôχ‰ÚÔÛÔ ∞Ì·ÚÔ˘Û›Ô˘
¶ÂÚ›ÏË„Ë ¿ÚıÚÔ˘ ·fi ÙË µÈ‚ÏÈÔÁÚ·Ê›· ∞˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ·ÛıÌ·ÙÈÎÔ‡ Ù‡Ô˘ Û˘ÌÙˆÌ¿ÙˆÓ Û ÎÔÚ›ÙÛÈ· Ô˘ Á›ÓÔÓÙ·È ˘¤Ú‚·Ú· ‹ ·¯‡Û·Úη ηٿ ÙË Û¯ÔÏÈ΋ ÂÚ›Ô‰Ô1 ™ÎÔfi˜: ∏ Èı·ÓfiÙËÙ· ‡·Ú͢ ·ÈÙÈÔÏÔÁÈ΋˜ Û˘Û¯¤ÙÈÛ˘ ÌÂٷ͇ ·¯˘Û·ÚΛ·˜ Î·È ¿ÛıÌ·ÙÔ˜ ¤¯ÂÈ ˘Ô‰Ëψı› ·fi ÙËÓ ÚfiÛÊ·ÙË ·‡ÍËÛË Ù˘ Û˘¯ÓfiÙËÙ¿˜ ÙÔ˘˜. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ™Â ‰Â›ÁÌ· ÁÂÓÈÎÔ‡ ÏËı˘ÛÌÔ‡, Ë Û˘¯ÓfiÙËÙ· Î·È Ô ÂÈÔÏ·ÛÌfi˜ ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ÙÔ˘ ¿ÛıÌ·ÙÔ˜, ÔÈ ‰ÂÚÌ·ÙÈΤ˜ ‰ÔÎÈ̷ۛ˜ Î·È Ô ‰Â›ÎÙ˘ ۈ̷ÙÈ΋˜ Ì¿˙·˜ (Body Mass Index - BMI), ÙÂÎÌËÚÈÒıËÎ·Ó Û ̤ÛË ËÏÈΛ· Ù· 6,3 ¤ÙË (n=688) Î·È Ù· 10,9 ¤ÙË (n=600). OÈ ‰ÔÎÈ̷ۛ˜ ‚ÚÔÁ¯Ô‰È·ÛÙÔÏ‹˜ Î·È Ë ËÌÂÚ‹ÛÈ· ‰È·Î‡Ì·ÓÛË Ù˘ ̤ÁÈÛÙ˘ ÂÎÓ¢ÛÙÈ΋˜ ÚÔ‹˜ ÌÂÙÚ‹ıËÎ·Ó ÛÙ· 11 ¯ÚfiÓÈ·. ∞ÔÙÂϤÛÌ·Ù·: ¢ÂÓ ˘‹Ú¯Â Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙÔ˘ ‰Â›ÎÙË ÛˆÌ·ÙÈ΋˜ Ì¿˙·˜ ÛÙËÓ ËÏÈΛ· ÙˆÓ 6 ÂÙÒÓ Î·È Ù˘ Û˘¯ÓfiÙËÙ·˜ ÂΉ‹ÏˆÛ˘ Û˘ÚÚ›ÙÔ˘Û·˜ ·Ó·ÓÔ‹˜ (wheezing) Û ÔÔÈ·‰‹ÔÙ ËÏÈΛ·. ∆· ÎÔÚ›ÙÛÈ·, ·ÏÏ¿ fi¯È Ù· ·ÁfiÚÈ·, Ô˘ ‹Ù·Ó ˘¤Ú‚·Ú· ‹ ·¯‡Û·Úη ÛÙËÓ ËÏÈΛ· ÙˆÓ 11 ¯ÚfiÓˆÓ Â›¯·Ó ÌÂÁ·Ï‡ÙÂÚË Èı·ÓfiÙËÙ· Ó· ÂÌÊ·Ó›ÛÔ˘Ó Û˘ÚÚ›ÙÔ˘Û· ·Ó·ÓÔ‹ ÛÙȘ ËÏÈ˘ ÙˆÓ 11 Î·È 13 ÂÙÒÓ, ·ÏÏ¿ fi¯È ÛÙȘ ËÏÈ˘ ÙˆÓ 6 ‹ 8 ÂÙÒÓ. ∞˘Ù‹ Ë Û¯¤ÛË ‹Ù·Ó ÂÓÙÔÓfiÙÂÚË ÛÙ· ÎÔÚ›ÙÛÈ· Ô˘ ›¯·Ó ¤Ó·ÚÍË ÂÓ‹‚ˆÛ˘ ÚÈÓ ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 11 ÂÙÒÓ. ∆· ÎÔÚ›ÙÛÈ· Ô˘ ¤ÁÈÓ·Ó ˘¤Ú‚·Ú· ‹ ·¯‡Û·Úη ÌÂٷ͇ ÙˆÓ 6 Î·È 11 ÂÙÒÓ, ›¯·Ó ÂÙ·Ï¿ÛÈ· Èı·ÓfiÙËÙ· Ó· ÂΉËÏÒÛÔ˘Ó Ó¤· Û˘ÌÙÒÌ·Ù· ¿ÛıÌ·ÙÔ˜ ÛÙËÓ ËÏÈΛ· ÙˆÓ 11 ‹ 13 ÂÙÒÓ
(p=0,0002), ÂÓÒ ÛÙËÓ ËÏÈΛ· ÙˆÓ 11 ÂÙÒÓ, Ë ‰È·Î‡Ì·ÓÛË Ù˘ ̤ÁÈÛÙ˘ ÂÎÓ¢ÛÙÈ΋˜ ÚÔ‹˜ Î·È Ë ·ÓÙ·fiÎÚÈÛË ÛÙ· ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο ›¯·Ó Ôχ ÌÂÁ·Ï‡ÙÂÚË Èı·ÓfiÙËÙ· Ó· Â›Ó·È ·˘ÍË̤Ó˜. ™˘ÌÂÚ¿ÛÌ·Ù·: ∆· ÎÔÚ›ÙÛÈ· Ô˘ Á›ÓÔÓÙ·È ˘¤Ú‚·Ú· ‹ ·¯‡Û·Úη Û ËÏÈΛ· 6-11 ÂÙÒÓ ÂÌÊ·Ó›˙Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÂΉ‹ÏˆÛ˘ Ó¤ˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ¿ÛıÌ·ÙÔ˜ Î·È ·˘ÍË̤ÓË ‚ÚÔÁ¯È΋ ··ÓÙËÙÈÎfiÙËÙ· ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÚÒÈÌ˘ ÂÊË‚È΋˜ ËÏÈΛ·˜.
1 Castro-Rodriguez JA, Holberg CJ, Morgan WJ, Wright AL, Martinez FD Increased incidence of asthma like symptoms in girls who become overweight or obese during the school years Am J Respir Crit Care Med 2001;163:1344-1349
£ÂÔÊ¿Ó˘ ∆ÛÈÏÈÁÈ¿ÓÓ˘ ¢È¢ı˘ÓÙ‹˜ ¶·È‰È·ÙÚÈÎÔ‡ ∆Ì‹Ì·ÙÔ˜ ¡·˘ÙÈÎÔ‡ ¡ÔÛÔÎÔÌ›Ԣ ∞ıËÓÒÓ
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¶·È‰È·ÙÚÈ΋ 2001;64:460-464
∂¶π∫∞πƒA £∂ª∞TA
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CURRENT ISSUES
™‡Ó‰ÚÔÌÔ ˘ÂÚ¢·ÈÛıËÛ›·˜ ÛÙ· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η π. X·ÚˆÓ›ÙË, ¢. §·ÌÚ¿ÎË, µ. ∞ÁÁÂÏ¿ÎÔ˘
Anticonvulsant hypersensitivity syndrome I. Charoniti, D. Lampraki, V. Aggelakou
¶ÂÚ›ÏË„Ë: ∆Ô Û‡Ó‰ÚÔÌÔ ˘ÂÚ¢·ÈÛıËÛ›·˜ ÛÙ· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η (™À∞) ·ÔÙÂÏ› ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋, ‰˘ÓËÙÈο ı·Ó·ÙËÊfiÚ· ·ÓÙ›‰Ú·ÛË, Ô˘ ÎÏ·ÛÈο ·Ô‰›‰ÂÙ·È ÛÙ· ·ÚˆÌ·ÙÈο ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η (Ê·ÈÓ˘ÙÔ˝ÓË, Ê·ÈÓÔ‚·Ú‚ÈÙ¿ÏË Î·È Î·Ú‚·Ì·˙›ÓË) Î·È Û·ÓÈfiÙÂÚ· ÛÙË Ï·ÌÔÙÚÈÁ›ÓË. ∆Ô ‚·ÏÚÔ˚Îfi Ó¿ÙÚÈÔ Î·È Ë ·ÈıÔÛÔ˘ÍÈÌ›‰Ë ¤¯Ô˘Ó ›Û˘ ÂÓÔ¯ÔÔÈËı›, ·Ó Î·È Ë Û·ÓÈfiÙËÙ· ÙˆÓ ·Ó·ÊÂÚfiÌÂÓˆÓ ÂÚÈÙÒÛÂˆÓ ‰›ÓÂÈ ·ÎfiÌË ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ˆ˜ ÂÓ·ÏÏ·ÎÙÈ΋ ıÂڷ›· Û ÂÚÈÙÒÛÂȘ Û˘Ó‰ÚfiÌÔ˘ ˘ÂÚ¢·ÈÛıËÛ›·˜ ÛÙ· ·ÚˆÌ·ÙÈο ·ÓÙÈÂÈÏËÙÈο. ∏ ¯·Ú·ÎÙËÚÈÛÙÈ΋ ÎÏÈÓÈ΋ ÙÚÈ¿‰· ÙÔ˘ ™À∞ Â›Ó·È ˘ÚÂÙfi˜, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· Î·È ÂÍ¿ÓıËÌ·. ¶·Ú’ fiÏ· ·˘Ù¿, ÌÔÚ› Ó· ÌÈÌÂ›Ù·È ÏÔÈÌÒ‰ÂȘ Î·È ·˘ÙÔ¿ÓÔÛ˜ ÓfiÛÔ˘˜ ‹ ·ÎfiÌË Î·È Î·ÎÔ‹ıÂȘ. ∏ ¤ÁηÈÚË ·Ó·ÁÓÒÚÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, ÙÔ ÔÔ›Ô ÂÌÊ·Ó›˙ÂÙ·È Ì ÔÈΛϘ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ, Â›Ó·È ÙÔ ÎÏÂȉ› ÁÈ· ÙËÓ ¿ÌÂÛË ‰È·ÎÔ‹ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ Î·È ÙË ÛˆÛÙ‹ ·Ú·ÎÔÏÔ‡ıËÛË Î·È ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘. ¶·Ú¿ÏÏËÏ·, Ë ÛˆÛÙ‹ ‰È¿ÁÓˆÛË Â›Ó·È ÛËÌ·ÓÙÈ΋, ÁÈ·Ù› ¤ÙÛÈ ı· ·ÔÊ¢¯ı› Ë ‰˘ÓËÙÈο ı·Ó·ÙËÊfiÚ· ·ӤÎıÂÛË ÛÙÔ ˘Â‡ı˘ÓÔ Ê¿ÚÌ·ÎÔ Î·È ı· ηıÔÚÈÛÙ› Ë ÂÚ·ÈÙ¤Úˆ ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹.
Abstract: Anticonvulsant hypersensitivity syndrome (AHS) is a multisystem, potentially fatal, drug reaction, classically attributed to the arene oxide-producing anticonvulsants (phenobarbital, carbamazepine, phenytoin), and less frequently to lamotrigine. Sodium valporate and ethosuximide have been also implicated but the reported cases are still rare, so they can still be used as an alternative treatment in cases of AHS due to arene oxide-producing anticonvulsants. The characteristic triad of symptoms of AHS is fever, rash and lymphadenopathy. The syndrome however may mimic infectious and autoimmune diseases or even malignancies. Early recognition of the syndrome, which has a variable presentation, is of great importance for the prompt discontinuation of the anticonvulsant drug as well as for close followup and therapeutic intervention. Furthermore, prompt diagnosis is crucial for the prevention of a potentially fatal outcome by re-exposure to the offending drug as well as for planning the type of antiepileptic therapy to be administered.
§¤ÍÂȘ ÎÏÂȉȿ: ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η, Û‡Ó‰ÚÔÌÔ ˘ÂÚ¢·ÈÛıËÛ›·˜.
Key words: syndrome.
∂ÈÛ·ÁˆÁ‹ ∆Ô Û‡Ó‰ÚÔÌÔ ˘ÂÚ¢·ÈÛıËÛ›·˜ ÛÙ· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η (™À∞) Â›Ó·È ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ·ÓÙ›‰Ú·ÛË È‰ÈÔÛ˘ÁÎÚ·Û›·˜, ‰˘ÓËÙÈο ı·Ó·ÙËÊfiÚ· (1). ¶ÂÚÈÁÚ¿ÊËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÙÔ 1934 ·fi ÙÔ˘˜ Silber Î·È Ebstein Î·È ÙÔ ÂÓÔ¯ÔÔÈÔ‡ÌÂÓÔ ÙfiÙÂ
·ÓÙÈÂÈÏËÙÈÎfi Ê¿ÚÌ·ÎÔ ‹Ù·Ó Ë Ê·ÈÓ˘ÏÂı˘Ï˘‰·ÙÔ˝ÓË, Ô˘ ÚfiÛÊ·Ù· ›¯Â ¯ÚËÛÈÌÔÔÈËı› ÛÙË ıÂڷ›· Ù˘ ¯ÔÚ›·˜ Sydenham. ŒÎÙÔÙÂ, ·˘Í¿ÓÔ˘Ó ÔÈ ·Ó·ÊÔÚ¤˜, ·Ú¯Èο ÁÈ· ÙȘ ˘‰·ÓÙÔ˝Ó˜ Î·È ·ÚÁfiÙÂÚ· ÁÈ· Ù· ˘fiÏÔÈ· ·ÓÙÈÂÈÏËÙÈο Ô˘ ʤÚÔ˘Ó ·ÚˆÌ·ÙÈÎfi ‰·ÎÙ‡ÏÈÔ ÛÙÔ ÌfiÚÈfi ÙÔ˘˜, fiˆ˜ Ë
∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, µÂÓÈ˙¤ÏÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ ∫Ú‹Ù˘
∞’ Pediatric Clinic of Venizelio Hospital Heraklion, Crete
460
anticonvulsant
hypersensitivity
¶·È‰È·ÙÚÈ΋ 2001;64:460-464
Ê·ÈÓÔ‚·Ú‚ÈÙ¿ÏË, Ë Î·Ú‚·Ì·˙›ÓË Î·È Ë Ï·ÌÔÙÚÈÁ›ÓË (2,3). ∆ÂÏÂ˘Ù·›·, ¤¯Ô˘Ó ÂÓÔ¯ÔÔÈËı› Î·È ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η Ì Ôχ ‰È·ÊÔÚÂÙÈ΋ ¯ËÌÈ΋ ‰ÔÌ‹, fiˆ˜ Ë ·ÈıÔ˘ÛÔÍÈÌ›‰Ë Î·È ÙÔ ‚·ÏÚÔ˚Îfi Ó¿ÙÚÈÔ, ·Ó Î·È ÔÈ ÂÚÈÙÒÛÂȘ Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È Â›Ó·È ·ÎfiÌË Û·ÓÈfiٷ٘ (¤¯ÂÈ ·Ó·ÊÂÚı› Ì›· ÂÚ›ÙˆÛË ™À∞ ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ‚·ÏÚÔ˚ÎÔ‡ Ó·ÙÚ›Ô˘ Î·È Ì›· ÌÂÙ¿ ·fi Ù·˘Ùfi¯ÚÔÓË ¯ÔÚ‹ÁËÛË ‚·ÏÚÔ˚ÎÔ‡ Ó·ÙÚ›Ô˘ Î·È ·ÈıÔ˘ÛÔÍÈÌ›‰Ë˜) (4). ªfiÏȘ ÙÔ 1988 ·Ó·ÁÓˆÚ›ÛÙËΠÙo ™À∞ ˆ˜ ͯˆÚÈÛÙ‹ ÔÓÙfiÙËÙ·. ∞ÎfiÌË Î·È Û‹ÌÂÚ·, fï˜, ÂÍ·ÎÔÏÔ˘ı› Ó· ÚÔηÏ› Û‡Á¯˘ÛË Î·È ‰È·ÁÓˆÛÙÈÎÔ‡˜ ÚÔ‚ÏËÌ·ÙÈÛÌÔ‡˜. ∏ ·ÎÚÈ‚‹˜ ‰È¿ÁÓˆÛË Â›Ó·È ‰‡ÛÎÔÏË ÏfiÁˆ ÙˆÓ ÔÈÎ›ÏˆÓ ÎÏÈÓÈÎÒÓ ÂΉËÏÒÛÂˆÓ Î·È ‚ÈÔ¯ËÌÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Ô˘ ÌÈÌÔ‡ÓÙ·È ÔÈÎÈÏ›· ÏÔÈ̈‰ÒÓ, ÓÂÔÏ·ÛÌ·ÙÈÎÒÓ Î·È ·˘ÙÔ¿ÓÔÛˆÓ ‰È·Ù·Ú·¯ÒÓ (5). ∏ ¤ÁηÈÚË ·Ó·ÁÓÒÚÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Â›Ó·È ˙ˆÙÈ΋˜ ÛËÌ·Û›·˜, ÁÈ·Ù› Ë ÌË ‰È·ÎÔ‹ ÙÔ˘ ‡ÔÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ı¤ÙÂÈ Û ΛӉ˘ÓÔ ÙË ˙ˆ‹ ÙÔ˘ ·ÛıÂÓÔ‡˜. ªÂ ·ÊÔÚÌ‹ ÙËÓ ÚfiÛÊ·ÙË ÓÔÛËÏ›· ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ‰‡Ô ·ÛıÂÓÒÓ Ì ™À∞, ·Ó·ÛÎÔԇ̠ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· Î·È ÂÚÈÁÚ¿ÊÔ˘Ì ÙȘ ÂÚÈÙÒÛÂȘ. ¶ÂÚÈÁÚ·Ê‹ ÂÚÈÙÒÛÂˆÓ ∏ ÚÒÙË ÂÚ›ÙˆÛË ·ÊÔÚ¿ ÎÔÚ›ÙÛÈ 7,5 ÂÙÒÓ Ô˘ ÚÔÛ‹Ïı Ì ˘ÚÂÙfi ˆ˜ 39,5ÔC ·fi 2Ë̤ÚÔ˘, ‰ÈfiÁΈÛË ÙÚ·¯ËÏÈÎÒÓ ÏÂÌÊ·‰¤ÓˆÓ, ÂͤڢıÚ· ·Ú›ÛıÌÈ·, ÎËÏȉԂϷÙȉ҉˜ ÂÍ¿ÓıËÌ· ΢ڛˆ˜ ÛÙËÓ ÔÈÛıÔˆÙÈ·›· ÂÚÈÔ¯‹ Î·È Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›· (‹·Ú 6 cm „ËÏ·ÊËÙfi, ÛÏ‹Ó·˜ 4 cm „ËÏ·ÊËÙfi˜). ™ÙÔ ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi ·Ó·ÊÂÚfiÙ·Ó fiÙÈ Ë ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·Û ÂÂÈÛfi‰È· ·Ê·ÈÚ¤ÛˆÓ, ÁÈ· Ù· ÔÔ›· ›¯Â ÚÔ ÌËÓfi˜ ·Ú¯›ÛÂÈ ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹ Ì ‚·ÏÚÔ˚Îfi Ó¿ÙÚÈÔ. ∞fi ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ÛÙËÓ ÂÈÛ·ÁˆÁ‹ Ù˘ ›¯Â: °ÂÓÈ΋ ·›Ì·ÙÔ˜: §Â˘Î¿ ·ÈÌÔÛÊ·›ÚÈ· 8120/mmÑ (ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· 72%, ÏÂÌÊÔ·ÙÙ·Ú· 10%, ÌÔÓÔ·ÙÙ·Ú· 10%, Ú·‚‰Ô‡ÚËÓ· 8%), Hgb 12 g/dl, Hct 36,6%, Aª¶ 224.000/mmÑ. ∆∫∂ 15 mm/h, SGOT 113,2 U/L, SGPT 112,4 U/L, ÁGT 39 U/L, ÔÏÈ΋ ¯ÔÏÂÚ˘ıÚ›ÓË 0,8 mg/dl, ÔÏÈο Ï¢ÎÒÌ·Ù· 6,7 g/dl, ·Ï‚Ô˘Ì›ÓË 4,1 g/dl. ∆Ș ÂfiÌÂÓ˜ Ë̤Ú˜, Ë Î·Ù¿ÛÙ·ÛË Ù˘ ·ÛıÂÓÔ‡˜ ÂȉÂÈÓÒıËÎÂ. ¶·ÚÔ˘Û›·˙ ˘ÚÂÙÈο ·̷ٷ ˆ˜ 40ÔC ·Ó¿ ÙÚ›ˆÚÔ, Ô˘ Û˘Óԉ‡ÔÓÙ·Ó ·fi Ú›ÁÔ˜. ∆Ô ÂÍ¿ÓıËÌ· ÂÍ·ÏÒıËΠÛÙÔÓ ÎÔÚÌfi Î·È Ù· ¿ÎÚ·, ¤ÁÈÓÂ Û˘ÚÚ¤ÔÓ Î·È ÎÓËÛÌ҉˜, Ì ¤ÓÙÔÓË ÂÚ˘ıÚfiÙËÙ· Î·È Î·Ù¿ ÙfiÔ˘˜ ·ÈÌÔÚÚ·ÁÈο ÛÙÔȯ›·. ∏ ·ӿÏË„Ë ÙÔ˘ ÂÚÁ·ÛÙËÚÈ·ÎÔ‡ ÂϤÁ¯Ô˘ ¤‰ÂȯÓ Âȉ›ӈÛË: °ÂÓÈ΋ ·›Ì·ÙÔ˜: §Â˘Î¿ ·ÈÌÔÛÊ·›ÚÈ· 20.100/mmÑ (ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· 10%,
Paediatriki 2001;64:460-464
ÏÂÌÊÔ·ÙÙ·Ú· 60%, ÌÔÓÔ·ÙÙ·Ú· 15%, Ú·‚‰Ô‡ÚËÓ· 11%), Hgb 12,3 g/dl, Hct 36,2%, AªT 188.000/mmÑ. H·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·: SGOT 867 U/L, SGPT 1111 U/L, ÔÏ. ¯ÔÏÂÚ˘ıÚ›ÓË 2,23 mg/dl, ¿ÌÂÛË ¯ÔÏÂÚ˘ıÚ›ÓË 1,59 mg/dl, ÔÏÈο Ï¢ÎÒÌ·Ù· 5,8 g/dl, ·Ï‚Ô˘Ì›ÓË 3,2 g/dl, PT 17 sec, INR 1,59, aPTT 37,55 sec, D-Dimers 1031,76 Ìg/l (ÊÙ: 125-375 Ìg/l). M ÙËÓ ˘Ô„›· ™À∞, ‰ÈÂÎfiË Ë ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹ ÙËÓ 3Ë Ë̤ڷ ÓÔÛËÏ›·˜. ∏ ·ÛıÂÓ‹˜, ÌÂÙ¿ ÙË ‰È·ÎÔ‹ ÙÔ˘ ‡ÔÙÔ˘ Ê·ÚÌ¿ÎÔ˘, ·ÚÔ˘Û›·Û ÛÙ·‰È·Î‹ ‡ÊÂÛË ÙÔ˘ ˘ÚÂÙÔ‡ Î·È ÙÔ˘ ÂÍ·Óı‹Ì·ÙÔ˜, ÙÔ ÔÔ›Ô ÛÙËÓ ·Ô‰ÚÔÌ‹ ÙÔ˘ ¤ÁÈÓ ·ÔÊÔÏȉˆÙÈÎfi. O ˘ÚÂÙfi˜ ˘Ô¯ÒÚËÛ ϋڈ˜ Û ̛· ‚‰ÔÌ¿‰· Î·È ÙÔ ÂÍ¿ÓıËÌ· Û 9 Ë̤Ú˜. O ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜, ÛÙÔ Ù¤ÏÔ˜ Ù˘ ‰Â‡ÙÂÚ˘ ‚‰ÔÌ¿‰·˜ Ù˘ ÓfiÛÔ˘ Ù˘, ‹Ù·Ó ÛËÌ·ÓÙÈο ‚ÂÏÙȈ̤ÓÔ˜: °ÂÓÈ΋ ·›Ì·ÙÔ˜: §Â˘Î¿ ·ÈÌÔÛÊ·›ÚÈ· 8.000/mmÑ (ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· 30%, ÏÂÌÊÔ·ÙÙ·Ú· 42%, ÌÔÓÔ·ÙÙ·Ú· 2%, ˈÛÈÓfiÊÈÏ· 26%), ∏gb 10,3 g/dl, Hct 30,2%, AªT 247.000/mmÑ. H·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·: SGOT 88 U/L, SGPT 193 U/L. O ¤ÏÂÁ¯Ô˜ ÁÈ· ÈÔÁÂÓ›˜ Î·È ÌÈÎÚԂȷΤ˜ ÏÔÈÌÒÍÂȘ (HAV, HBV, HCV, EBV, CMV, toxoplasma, ·‰ÂÓÔ˚Ô‡˜, echo-ÈÔ‡˜, parvo-ÈÔ‡˜, ƒÈΤÙÛȘ, Borrelia Burgdorferi, Ì˘ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌÔÓ›·˜) ·¤‚Ë ·ÚÓËÙÈÎfi˜. O ¤ÏÂÁ¯Ô˜ ÁÈ· ÎÔÈÓ¿ ·ıÔÁfiÓ· Ì ÔÏϷϤ˜ ηÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜, ›Û˘ ‹Ù·Ó ·ÚÓËÙÈÎfi˜. ∏ ‰Â‡ÙÂÚË ÂÚ›ÙˆÛË ·ÊÔÚ¿ ÎÔÚ›ÙÛÈ 11 ÂÙÒÓ Ô˘ ÂÈÛ‹¯ıË ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÁÈ·Ù› ·fi 24ÒÚÔ˘ ·ÚÔ˘Û›·Û ˘ÚÂÙfi ˆ˜ 38ÔC Î·È ÁÂÓÈÎÂ˘Ì¤ÓÔ ÎËÏȉ҉˜ ÂÍ¿ÓıËÌ· Ì ¤ÓÙÔÓË ÂÚ˘ıÚfiÙËÙ·. ™ÙÔ ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi Ù˘ ·ÛıÂÓÔ‡˜ ·Ó·Ê¤ÚÔÓÙ·Ó ÂÂÈÛfi‰È· ÁÂÓÈÎÂ˘Ì¤ÓˆÓ ÙÔÓÈÎÔÎÏÔÓÈÎÒÓ Û·ÛÌÒÓ, ÁÈ· Ù· ÔÔ›· ¿Ú¯ÈÛ ӷ Ï·Ì‚¿ÓÂÈ ıÂڷ›· Ì ηڂ·Ì·˙›ÓË ·fi 11Ë̤ÚÔ˘. ∏ Ê˘ÛÈ΋ ÂͤٷÛË ·ÔÎ¿Ï˘„ ‰ÈÔÁΈ̤ÓÔ˘˜ ˘ÔÁÓ¿ıÈÔ˘˜ Î·È ÙÚ·¯ËÏÈÎÔ‡˜ ÏÂÌÊ·‰¤Ó˜ Ô˘ ‹Ù·Ó ¢·›ÛıËÙÔÈ ÛÙËÓ „ËÏ¿ÊËÛË. O ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÛÙËÓ ÂÈÛ·ÁˆÁ‹ Ù˘ ‹Ù·Ó ¯ˆÚ›˜ ȉȷ›ÙÂÚË ·ıÔÏÔÁ›· Î·È ·Ú¤ÌÂÈÓ ¤ÙÛÈ ¤ˆ˜ ÙËÓ ¤ÍÔ‰fi Ù˘: §Â˘Î¿ ·ÈÌÔÛÊ·›ÚÈ· 4700/mmÑ (ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· 64,7%, ÏÂÌÊÔ·ÙÙ·Ú· 22%, ÌÔÓÔ·ÙÙ·Ú· 7%), Hgb 13,4 g/dl, Hct 40%, AªT 239.000/mmÑ, ∆∫∂ 20 mm/h. H·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·: SGOT 19 U/L, SGPT 15 U/L, ÔÏ. ¯ÔÏÂÚ˘ıÚ›ÓË 0,39 mg/dl, ÔÏÈο Ï¢ÎÒÌ·Ù· 7,3 g/dl, ·Ï‚Ô˘Ì›ÓË 4,7 g/dl, PT 12 sec, INR 1,09, aPTT 35,6 sec. ªÂ ÙËÓ ˘Ô„›· ™À∞, ¤ÁÈÓ ¿ÌÂÛ· ‰È·ÎÔ‹ Ù˘ ·ÓÙÈÂÈÏËÙÈ΋˜ ·ÁˆÁ‹˜, ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfiÓ˘ Î·È ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ¯ÔÚ‹ÁËÛË ˘‰ÚÔ͢˙›Ó˘. O ˘ÚÂÙfi˜, ÙÔ ÂÍ¿ÓıËÌ· Î·È Ë ÏÂÌÊ·‰ÂÓÈ΋ ‰ÈfiÁΈÛË ¿Ú¯ÈÛ·Ó Ó· ˘Ô¯ˆÚÔ‡Ó ·fi ÙÔ 3Ô 24ˆÚÔ ÓÔÛËÏ›·˜. ∏ ·ÛıÂÓ‹˜, ÙËÓ 4Ë
461
¶·È‰È·ÙÚÈ΋ 2001;64:460-464
Ë̤ڷ ÓÔÛËÏ›·˜, ÂÌÊ¿ÓÈÛ ÂÂÈÛfi‰ÈÔ ÁÂÓÈÎÂ˘Ì¤ÓˆÓ ÙÔÓÈÎÔÎÏÔÓÈÎÒÓ Û·ÛÌÒÓ ‰È¿ÚÎÂÈ·˜ Ï›ÁˆÓ ‰Â˘ÙÂÚÔϤوÓ. °È· ·˘ÙfiÓ ÙÔ ÏfiÁÔ, ÎÚ›ıËΠ··Ú·›ÙËÙË Ë ¤Ó·ÚÍË Ó¤·˜ ·ÓÙÈÂÈÏËÙÈ΋˜ ·ÁˆÁ‹˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔÛËÏ›·˜ Ù˘ Î·È ÚÈÓ ÙËÓ Ï‹ÚË ·Ó¿ÚÚˆÛ‹ Ù˘. ª›· ‚‰ÔÌ¿‰· ÌÂÙ¿, ‹Ù·Ó Û ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË, Ì ٷ Û˘ÌÙÒÌ·Ù· Û ϋÚË ·Ô‰ÚÔÌ‹. O ¤ÏÂÁ¯Ô˜ ÁÈ· ÈÔÁÂÓ›˜ Î·È ÌÈÎÚԂȷΤ˜ ÏÔÈÌÒÍÂȘ ·¤‚Ë ·ÚÓËÙÈÎfi˜. ∫ÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ∆Ô ™À∞ ÂΉËÏÒÓÂÙ·È ÙÔ˘˜ ‰‡Ô ÚÒÙÔ˘˜ Ì‹Ó˜ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÓÙÈÂÈÏËÙÈ΋˜ ·ÁˆÁ‹˜ Î·È Û˘Ó‹ıˆ˜ ÙË 2Ë ¤ˆ˜ 4Ë Â‚‰ÔÌ¿‰·. ™˘Ì‚·›ÓÂÈ ÓˆÚ›ÙÂÚ· Û ‹‰Ë ¢·ÈÛıËÙÔÔÈË̤ӷ ¿ÙÔÌ·. ™Â ÂÚ›ÙˆÛË Â·Ó·¯ÔÚ‹ÁËÛ˘ ÙÔ˘ ‡ÔÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ÂÌÊ·Ó›˙ÂÙ·È ÙȘ ÚÒÙ˜ 24 ÒÚ˜ ·fi ÙË ¯ÔÚ‹ÁËÛ‹ ÙÔ˘. ∂ÌÊ·Ó›˙ÂÙ·È Û˘Ó‹ıˆ˜ ÙËÓ ÚÒÙË ‰ÂηÂÙ›· Ù˘ ˙ˆ‹˜, Ì ›وÛË ÂÚ›Ô˘ 1 ÂÚ›ÙˆÛË ÛÙȘ 3000 (6). ∆· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1. O Û˘Ó‰˘·ÛÌfi˜ ˘ÚÂÙÔ‡, ÂÍ·Óı‹Ì·ÙÔ˜ Î·È ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·˜ Û ·ÛıÂÓ‹ Ô˘ Ï·Ì‚¿ÓÂÈ ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹ Ì ·ÚˆÌ·ÙÈο ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η, Ú¤ÂÈ Ó· ÂÁ›ÚÂÈ ˘Ô„›Â˜ ÁÈ· ÙÔ ™À∞. ™ÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË, fï˜, Ë ÂÌÊ¿ÓÈÛË ÙÔ˘ ™À∞ Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ÔχÏÔÎË. ∆· ÎÏÈÓÈο ÛËÌ›· Î·È Û˘ÌÙÒÌ·Ù· ÌÔÚ› Ó· ‰È·Ê¤ÚÔ˘Ó ÛËÌ·ÓÙÈο ˆ˜ ÚÔ˜ ÙÔ ¯ÚfiÓÔ ¤Ó·ÚÍ‹˜ ÙÔ˘˜ Î·È ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ÌÂÌÔӈ̤ӷ ‹ ηٿ Û˘ÚÚÔ‹. ŒÙÛÈ, ‰˘Û¯ÂÚ·›ÓÂÙ·È Ë ¤ÁηÈÚË ‰È¿ÁÓˆÛ‹ ÙÔ˘. ∂È‚¿ÏÏÂÙ·È ·˘ÍË̤ÓÔ˜ ‰Â›ÎÙ˘ ˘Ô„›·˜, ÚÔÎÂÈ̤ÓÔ˘ ÙÔ ™À∞ Ó· ·Ó·ÁÓˆÚÈÛÙ›, ¿Ú· Î·È Ó· ·ÓÙÈÌÂÙˆÈÛÙ› ¤ÁηÈÚ·. O ˘ÚÂÙfi˜ Û˘Ó·ÓÙ¿Ù·È ÛÙÔ Û‡ÓÔÏÔ Û¯Â‰fiÓ ÙˆÓ ÂÚÈÙÒÛˆÓ. ™˘Ó‹ıˆ˜ ÚÔËÁÂ›Ù·È ‹ ÂÌÊ·Ó›˙ÂÙ·È Ù·˘Ùfi¯ÚÔÓ· Ì ٷ ˘fiÏÔÈ· Û˘ÌÙÒÌ·Ù·. ∂›Ó·È ˘„ËÏfi˜, Î˘Ì·›ÓÂÙ·È ·fi 38ÔC ¤ˆ˜ 40ÔC Î·È ‰ËÌÈÔ˘ÚÁ› ˘Ô„›Â˜ ÁÈ· ˘ÔΛÌÂÓË Ïԛ̈ÍË. ªÔÚ› Ó· ÂÈ̤ÓÂÈ ÁÈ· ‚‰ÔÌ¿‰Â˜, ¤ˆ˜ fiÙÔ˘ Ë ¯ÔÚ‹ÁËÛË ÙÔ˘ ˘Â‡ı˘ÓÔ˘ Ê·ÚÌ¿ÎÔ˘ ‰È·ÎÔ›, ÔfiÙ ÙÂÏÈο Î·È ˘Ô¯ˆÚ› ¿ÌÂÛ· ‹, ÛÙȘ ÈÔ ÛÔ‚·Ú¤˜ ÂÚÈÙÒÛÂȘ, ÛÙ·‰È·Î¿. ŸÛÔÓ ·ÊÔÚ¿ ÙÔ ÂÍ¿ÓıËÌ·, ÚfiÎÂÈÙ·È Û˘Ó‹ıˆ˜ ÁÈ· ÂÚ˘ıËÌ·ÙÒ‰ÂȘ ÎËÏ›‰Â˜ Ô˘ ÛÙËÓ ÔÚ›· ·˘Í¿ÓÔÓÙ·È, Û˘ÚÚ¤Ô˘Ó, Ï·Ì‚¿ÓÔ˘Ó ÛÎÔ‡ÚÔ ÎfiÎÎÈÓÔ ¯ÚÒÌ· Î·È ÌÂÙ·ÙÚ¤ÔÓÙ·È Û ‚Ï·Ù›‰Â˜. ∞Ú¯›˙ÂÈ ·fi ÙÔÓ ÎÔÚÌfi, ÙÔ ÚfiÛˆÔ, Ù· ¿Óˆ ¿ÎÚ· Î·È ÂÍ·ÏÒÓÂÙ·È ÛÙ· οو ¿ÎÚ·. ∆Ô Ô›‰ËÌ· ÙÔ˘ ÚÔÛÒÔ˘ ÌÔÚ› Ó· Â›Ó·È ÙfiÛÔ ÛÔ‚·Úfi Î·È ÂÎÙÂٷ̤ÓÔ, ÒÛÙ ӷ ·Ú·ÌÔÚÊÒÓÔÓÙ·È Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ·ÛıÂÓÔ‡˜. ™ÙËÓ ÔȉË-
462
Paediatriki 2001;64:460-464
¶›Ó·Î·˜ 1. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· Û˘Ó‰ÚfiÌÔ˘ ˘ÂÚ¢·ÈÛıËÛ›·˜ ÛÙ· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η (1) ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ¶˘ÚÂÙfi˜ ∂Í¿ÓıËÌ· §ÂÌÊ·‰ÂÓÔ¿ıÂÈ· ¶ÔÏ˘ÔÚÁ·ÓÈ΋ Û˘ÌÌÂÙÔ¯‹ ∏·Ù›Ùȉ· ∞ÈÌ·ÙÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜ ¶ÂÚÈÔÊı·ÏÌÈÎfi ‹ Ô›‰ËÌ· ÚÔÛÒÔ˘ ª˘·ÏÁ›Â˜ - ∞ÚıÚ·ÏÁ›Â˜ º·Ú˘ÁÁ›Ùȉ·
∂›ÙˆÛË (%) 90-100 90 70 60 50-60 50 25 21 10
1
Arch Intern Med 1995;155:2285-2289
Ì·ÙÒ‰Ë ÂÚÈÔ¯‹ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó Ê˘Û·Ï›‰Â˜, ÂÚÈÊÂÚÈο ÙˆÓ ÔÔ›ˆÓ ÙÔ ‰¤ÚÌ· ·ÚÔ˘ÛÈ¿˙ÂÈ ·ÔϤÈÛË Î·È ÔÈ Ôԛ˜ ·ÚÁfiÙÂÚ· ı· ÂÍÂÏȯıÔ‡Ó Û ÊχÎÙ·ÈÓ˜. ªÂ ÙËÓ ˘Ô¯ÒÚËÛË ÙÔ˘ ÂÍ·Óı‹Ì·ÙÔ˜ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙ› ¤ÓÙÔÓË ·ÔÊÔÏ›‰ˆÛË. ™·ÓÈfiÙÂÚ·, ÔÈ ‰ÂÚÌ·ÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ ÌÔÚ› Ó· Á›ÓÔ˘Ó ÙfiÛÔ ÛÔ‚·Ú¤˜, ÒÛÙ ӷ ¿ÚÔ˘Ó ÙË ÌÔÚÊ‹ ÙÔ˘ ÔχÌÔÚÊÔ˘ ÂÚ˘ı‹Ì·ÙÔ˜ ‹ Ù˘ ÂȉÂÚÌÔÓÂÎÚfiÏ˘Û˘. ™Ô‚·Ú¤˜ ‰ÂÚÌ·ÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ Û˘Ì‚·›ÓÔ˘Ó Û˘¯ÓfiÙÂÚ· Û ·ÛıÂÓ›˜ ÛÙÔ˘˜ ÔÔ›Ô˘˜ ÙÔ ˘Â‡ı˘ÓÔ ·ÓÙÈÂÈÏËÙÈÎfi Ê¿ÚÌ·ÎÔ Â·Ó·¯ÔÚËÁ‹ıËÎÂ, ÂÓÒ ÛÙÔ ·ÚÂÏıfiÓ Â›¯Â ÚÔÍÂÓ‹ÛÂÈ ·ÚÂÓ¤ÚÁÂȘ Î·È Û ÂΛÓÔ˘˜ Ô˘ Ë ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹ Û˘Ó¯›˙ÂÙ·È ÌÂÙ¿ ÙËÓ ÂÌÊ¿ÓÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ˘ÂÚ¢·ÈÛıËÛ›·˜. H ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· ÌÔÚ› Ó· ·ÊÔÚ¿ ÌÂÌÔӈ̤ÓÔ˘˜ ÏÂÌÊ·‰¤Ó˜ ‹ Ó· Â›Ó·È ÁÂÓÈÎÂ˘Ì¤ÓË. OÈ ÏÂÌÊ·‰¤Ó˜ Â›Ó·È ‰ÈÔÁΈ̤ÓÔÈ Î·È Â˘·›ÛıËÙÔÈ. ™ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ, ˘¿Ú¯ÂÈ Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ‹·ÙÔ˜ Ì ÙË ÌÔÚÊ‹ Ë·Ù›Ùȉ·˜. ∏ Ë·ÙÈ΋ ÚÔÛ‚ÔÏ‹ ıˆÚÂ›Ù·È Ë ÛÔ‚·ÚfiÙÂÚË ÂÈÏÔ΋ ÁÈ·Ù› Â›Ó·È ‰˘ÓËÙÈο ı·Ó·ÙËÊfiÚ·. ∏ ıÓËÛÈÌfiÙËÙ· ÙÔ˘ ™À∞ ÌÂ Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ‹·ÙÔ˜ ·Ó¤Ú¯ÂÙ·È ÛÙÔ 18-40%. ∏ Ë·Ù›Ùȉ· Â›Ó·È Û˘Ó‹ıˆ˜ ·ÓÈÎÙÂÚÈ΋. ∏ ·ÚÔ˘Û›· ÈÎÙ¤ÚÔ˘ ‰ËÏÒÓÂÈ ¯ÂÈÚfiÙÂÚË ÚfiÁÓˆÛË. ™ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ·Ó¢ڛÛÎÂÙ·È Ë·ÙÔÌÂÁ·Ï›· Ì ‹ ¯ˆÚ›˜ ÛÏËÓÔÌÂÁ·Ï›·. OÈ ÙÚ·ÓÛ·ÌÈÓ¿Û˜ Â›Ó·È ÛËÌ·ÓÙÈο ·˘ÍË̤Ó˜ Î·È ÂӉ¯Ô̤ӈ˜ Ó· Û˘Ó¯›˙Ô˘Ó Ó· ·˘Í¿ÓÔÓÙ·È Î·È ÌÂÙ¿ ÙË ‰È·ÎÔ‹ ÙÔ˘ ‡ÔÙÔ˘ ·ÓÙÈÂÈÏËÙÈÎÔ‡. ∂ÈϤÔÓ, ÂËÚ¿˙ÔÓÙ·È ÔÈ ·Ú¿ÁÔÓÙ˜ ‹Í˘, ηıÒ˜ Î·È Ë Û˘ÓıÂÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ‹·ÙÔ˜. O ‚·ıÌfi˜ ÚÔÛ‚ÔÏ‹˜ ÙÔ˘ ‹·ÙÔ˜ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· Ô˘ ÌÂÛÔÏ·‚› ̤¯ÚÈ ÙË ‰È·ÎÔ‹ ÙÔ˘ ˘Â‡ı˘ÓÔ˘ Ê·ÚÌ¿ÎÔ˘. ∏ Ï‹Ú˘ ·ÔηٿÛÙ·ÛË ÌÔÚ› Ó· ··ÈÙ‹ÛÂÈ Ì‹Ó˜. OÈ ·ÈÌ·ÙÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜ Û˘Ó›ÛÙ·ÓÙ·È Û Ï¢ÎÔ΢ÙÙ¿ÚˆÛË, ˈÛÈÓÔÊÈÏ›· Î·È Û¿ÓÈ· ۠ϢÎÔÂÓ›·. ∏ ˈÛÈÓÔÊÈÏ›· ÂÌÊ·Ó›˙ÂÙ·È Û¯ÂÙÈο
¶·È‰È·ÙÚÈ΋ 2001;64:460-464
·ÚÁ¿ ÛÙËÓ ÔÚ›· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, Û˘Ó‹ıˆ˜ 1-2 ‚‰ÔÌ¿‰Â˜ ·fi ÙËÓ ¤Ó·ÚÍ‹ ÙÔ˘. Œ¯ÂÈ, ›Û˘, ÂÚÈÁÚ·Ê› ·ÈÌÔÏ˘ÙÈ΋ ·Ó·ÈÌ›· Ì ·ÚÓËÙÈ΋ Coombs. OÈ ·ÓÔÛÔÛÊ·ÈÚ›Ó˜ Â›Ó·È Û˘Ó‹ıˆ˜ Ê˘ÛÈÔÏÔÁÈΤ˜, fiˆ˜ ›Û˘ Ë ∆∫∂ Î·È ÙÔ Û˘Ìϋڈ̷. ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÙÔ˘ ™À∞ Ê·›ÓÂÙ·È ÛÙÔÓ ¶›Ó·Î· 2. ¶·ıÔÁ¤ÓÂÈ· O ·ÎÚÈ‚‹˜ Ì˯·ÓÈÛÌfi˜ ÚfiÎÏËÛ˘ ÙÔ˘ ™À∞ ‰ÂÓ ¤¯ÂÈ ‰È¢ÎÚÈÓÈÛÙ›. °ÂÁÔÓfi˜ Â›Ó·È fiÙÈ ÙÔ ™À∞ ‰ÂÓ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔ Ê‡ÏÔ, ÙË ‰fiÛË Î·È Ù· ›‰· ÙÔ˘ ·ÓÙÈÂÈÏËÙÈÎÔ‡ Ê·ÚÌ¿ÎÔ˘. ¢È¿ÊÔÚÔÈ Ì˯·ÓÈÛÌÔ›, ·ÓÔÛÔÏÔÁÈÎÔ› Î·È ÌË, ¤¯Ô˘Ó ÚÔÙ·ı›. ªÂÚÈÎÔ› ˘ÔÛÙËÚ›˙Ô˘Ó ˆ˜ ÚfiÎÂÈÙ·È ÁÈ· Ì›· ·ÓÔÛÔÏÔÁÈ΋ ·ÓÙ›‰Ú·ÛË ÌÔۯ‡̷ÙÔ˜ ÂÓ·ÓÙ›ÔÓ ÍÂÓÈÛÙ‹, ·ÊÔ‡ Ë ·Ú·¿Óˆ ·ÓÙ›‰Ú·ÛË Î·È ÙÔ ™À∞ ¤¯Ô˘Ó ÎÔÈÓ¿ ¯·Ú·ÎÙËÚÈÛÙÈο (1). ¶ÚÔÙ›ÓÂÙ·È ·ÎfiÌË fiÙÈ Ù· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η ÙÚÔÔÔÈÔ‡Ó Ù· ÏÂÌÊÔ·ÙÙ·Ú·, ÒÛÙ ӷ ‰È·Ù·Ú¿ÛÛÂÙ·È Ô Ì˯·ÓÈÛÌfi˜ ·Ó·ÁÓÒÚÈÛ‹˜ ÙÔ˘˜ ˆ˜ “›‰È·” ·ÙÙ·Ú· ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡. ÕÏÏÔÈ ÚÔÙ›ÓÔ˘Ó fiÙÈ ÙÔ ™À∞ ÔÊ›ÏÂÙ·È Û ΢ÎÏÔÊÔÚÔ‡ÓÙ· ·ÓÙÈÛÒÌ·Ù·. ª›· ¿ÏÏË ıˆڛ· ÂÓÔ¯ÔÔÈ› ÙÔ˘˜ ÙÔÍÈÎÔ‡˜ ÌÂÙ·‚Ôϛ٘ ·ÓÙÈÂÈÏËÙÈÎÒÓ Ê·Ú̿ΈÓ, Ì ·ÚˆÌ·ÙÈÎfi ‰·ÎÙ‡ÏÈÔ, Ô˘ ·Ú¿ÁÔÓÙ·È ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙÔ˘˜ ·fi ÙÔ Î˘Ùfi¯ÚˆÌ· P-450 ÙÔ˘ ‹·ÙÔ˜. ™‡Ìʈӷ Ì ÙËÓ ·Ú·¿Óˆ ıˆڛ·, ˘¿Ú¯Ô˘Ó ¿ÙÔÌ· Ô˘ ʤÚÔ˘Ó ÌÂÙ¿ÏÏ·ÍË ‹ ÛÙÂÚÔ‡ÓÙ·È ÙÔ˘ ÂÓ˙‡ÌÔ˘ ÂÔÍÈ΋ ˘‰ÚÔÍ˘Ï¿ÛË, Ô˘ Â›Ó·È ˘Â‡ı˘ÓÔ ÁÈ· ÙËÓ ÂÍÔ˘‰ÂÙ¤ÚˆÛË ÙˆÓ ÙÔÍÈÎÒÓ ÌÂÙ·‚ÔÏÈÙÒÓ ÙˆÓ ·ÓˆÙ¤Úˆ ·Ó·ÊÂÚı¤ÓÙˆÓ ·ÓÙÈÂÈÏËÙÈÎÒÓ Ê·Ú̿ΈÓ. ∫·Ù¿ Û˘Ó¤ÂÈ·, fiÙ·Ó Ë ·‰Ú·ÓÔÔ›ËÛË ÙˆÓ ÙÔÍÈÎÒÓ ÌÂÙ·‚ÔÏÈÙÒÓ Â›Ó·È ÂÏ·Ùو̷ÙÈ΋, Ë ¿ıÚÔÈÛ‹ ÙÔ˘˜ ÌÔÚ› Ó· ÚÔηϤÛÂÈ Î˘ÙÙ·ÚÈÎfi ı¿Ó·ÙÔ ‹ Ó· Ô‰ËÁ‹ÛÂÈ ÛÙÔ Û¯ËÌ·ÙÈÛÌfi ÓÂÔ·ÓÙÈÁfiÓˆÓ, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ˘ÚÔ¶›Ó·Î·˜ 2. ¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÙÔ˘ ™À∞ §ÔÈÌ҉˘ ÌÔÓÔ˘Ú‹ÓˆÛË ÕÙ˘Ë ÈÏ·Ú¿ §Ô›ÌˆÍË ·fi CMV §Ô›ÌˆÍË ·fi HIV πÔÁÂÓ›˜ Ë·Ù›Ùȉ˜ ™ËÙÈÎfi shock ∆ÔÍÈÎfi Û‡Ó‰ÚÔÌÔ Î·Ù·ÏËÍ›·˜ ™‡ÊÈÏË ™‡Ó‰ÚÔÌÔ Kawasaki ™˘ÛÙËÌ·ÙÈÎfi˜ ÂÚ˘ıËÌ·Ù҉˘ χÎÔ˜ ÀÂÚˈÛÈÓÔÊÈÏÈÎfi Û‡Ó‰ÚÔÌÔ ¶ÔÚÊ˘Ú›· ∆ÔÍÈ΋ ÂȉÂÚÌÈ΋ ÓÂÎÚfiÏ˘ÛË (LYELL Syndrome) ¶ÔχÌÔÚÊÔ ÂÚ‡ıËÌ· §¤Ìʈ̷
Paediatriki 2001;64:460-464
‰ÔÙËı› ·ÓÔÛÔÏÔÁÈ΋ ·ÓÙ›‰Ú·ÛË (7). ¢ÔÎÈ̷ۛ˜ in vitro ¤‰ÂÈÍ·Ó ˆ˜ ˘¿Ú¯ÂÈ ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ·ÓÙ›‰Ú·ÛË ÌÂٷ͇ ÙˆÓ ·ÓÙÈÂÈÏËÙÈÎÒÓ Ô˘ ÌÂÙ·‚ÔÏ›˙ÔÓÙ·È Ì¤Ûˆ ÙÔ˘ ΢ÙÔ¯ÚÒÌ·ÙÔ˜ P-450 ÙÔ˘ ‹·ÙÔ˜ Û ÔÛÔÛÙfi ÂÚ›Ô˘ 75%. ∏ ·ÈıÔ˘ÛÔÍÈÌ›‰Ë Î·È ÙÔ ‚·ÏÚÔ˚Îfi Ó¿ÙÚÈÔ ÌÂÙ·‚ÔÏ›˙ÔÓÙ·È Ì¤Ûˆ ‰È·ÊÔÚÂÙÈÎÒÓ Ô‰ÒÓ. ŒÙÛÈ, ıˆÚÂ›Ù·È fiÙÈ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ¯·ÌËÏfi ΛӉ˘ÓÔ ÂÌÊ¿ÓÈÛ˘ ·ÓÙȉڿÛÂˆÓ ·fi ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ˘ÂÚ¢·ÈÛıËÛ›·. ¶ÚfiÛÊ·Ù˜ ‰ËÌÔÛȇÛÂȘ ÂÓÔ¯ÔÔÈÔ‡Ó ÙË Û˘ÌÌÂÙÔ¯‹ ÙˆÓ ÈÒÓ ÛÙËÓ ·ıÔÁ¤ÓÂÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. πÔ› Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È Â›Ó·È ÔÈ: HHV6, EBV, HIV, HAV, HBV, HCV, CMV, HSV, Parvovirus B19 Î.¿. ∂›Û˘, ÂÓÔ¯ÔÔÈÂ›Ù·È ÙÔ Ì˘ÎfiÏ·ÛÌ· Ù˘ Ó¢ÌÔÓ›·˜. ¶ÈÛÙ‡ÂÙ·È ‰ËÏ. fiÙÈ ÔÈ ÈÔ› ›Ûˆ˜ ‰ÚÔ˘Ó ˆ˜ Û˘Ì·Ú¿ÁÔÓÙ˜ ÛÙËÓ ÙÚÔÔÔ›ËÛË ÙˆÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ ÙÔ˘ ÍÂÓÈÛÙ‹, ˘ÚÔ‰ÔÙÒÓÙ·˜ ¤ÙÛÈ ÛÙËÓ ÂΉ‹ÏˆÛË ™À∞. ÕÏÏÔÈ ˘ÔÛÙËÚ›˙Ô˘Ó ˆ˜ Ù· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η, ›Ûˆ˜ ̤ۈ ‰È¤ÁÂÚÛ˘ ÙˆÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ, ·ÓÂÓÂÚÁÔÔÈÔ‡Ó ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ. Œ¯ÂÈ, ›Û˘, ÂÎÊÚ·ÛÙ› Ë ¿Ô„Ë ˆ˜ ÔÈ ÈÔ›, fiˆ˜ Ô HHV6, ηıÈÛÙÔ‡Ó Ù· ÂÓ˙˘ÌÈο Û˘ÛÙ‹Ì·Ù· ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙˆÓ ·ÓÙÈÂÈÏËÙÈÎÒÓ Ê·ÚÌ¿ÎˆÓ ÏÈÁfiÙÂÚÔ ‰Ú·ÛÙÈο (4,8,9). ¶¿ÓÙˆ˜, ÂÂȉ‹ Ô ·ÎÚÈ‚‹˜ ÚfiÏÔ˜ ÙˆÓ ÈÒÓ ÛÙËÓ ·ıÔÁ¤ÓÂÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ‰ÂÓ Â›Ó·È ·ÎfiÌË ÍÂοı·ÚÔ˜, ÈÛÙ‡ÂÙ·È ˆ˜ ÔÈ ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ Ú¤ÂÈ Ó· ıÂڷ‡ÔÓÙ·È Ì ·ÓÙÈ-ÈÈο Ê¿Ú̷η, fiÔ˘ ˘¿Ú¯Ô˘Ó ÔÈ Û¯ÂÙÈΤ˜ ÂӉ›ÍÂȘ. £Âڷ›· Î·È ÚfiÁÓˆÛË ¶ÚÒÙÔ Ì¤ÏËÌ· ÙÔ˘ ÎÏÈÓÈÎÔ‡ ÁÈ·ÙÚÔ‡, fiÙ·Ó Ù›ıÂÙ·È Ë ˘Ô„›· ™À∞, ·ÔÙÂÏ› Ë ¿ÌÂÛË Î·È ¤ÁηÈÚË ‰È·ÎÔ‹ ÙÔ˘ ‡ÔÙÔ˘ Ê·ÚÌ¿ÎÔ˘ (1). §fiÁˆ ÙÔ˘ ÎÈÓ‰‡ÓÔ˘ Û·ÛÌÒÓ ı· Ú¤ÂÈ Ó· ‰Ôı› ÂÓ·ÏÏ·ÎÙÈ΋ χÛË ıÂڷ¢ÙÈ΋˜ ·ÁˆÁ‹˜, ÙfiÛÔ ÛÙËÓ ÔÍ›· Ê¿ÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ fiÛÔ Î·È Ì·ÎÚÔÚfiıÂÛÌ·, Ï·Ì‚¿ÓÔÓÙ·˜ ˘` fi„ÈÓ ÙÔ ˘„ËÏfi ÔÛÔÛÙfi (70%) ‰È·ÛÙ·˘ÚÔ‡ÌÂÓ˘ ·ÓÙ›‰Ú·Û˘ ÌÂٷ͇ ÙˆÓ ·ÚˆÌ·ÙÈÎÒÓ ·ÓÙÈÂÈÏËÙÈÎÒÓ (2). ∫·Ù¿ ÙËÓ ÔÍ›· Ê¿ÛË ÙÔ˘ ™À∞, ¯ÔÚ‹ÁËÛË Ó¤·˜ ·ÓÙÈÂÈÏËÙÈ΋˜ ·ÁˆÁ‹˜ Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÌfiÓÔ ÁÈ· ÙËÓ ¿ÌÂÛË ·ÓÙÈÌÂÙÒÈÛË Û·ÛÌÒÓ. ™ÙȘ ÂÚÈÙÒÛÂȘ Ô˘ ‰ÂÓ ˘¿Ú¯ÂÈ Ë·ÙÈ΋ Û˘ÌÌÂÙÔ¯‹, ÙÔ ‚·ÏÚÔ˚Îfi Ó¿ÙÚÈÔ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› ˆ˜ ÂÓ·ÏÏ·ÎÙÈ΋ ıÂڷ›· ·ÊÔ‡ ÌÂÙ·‚ÔÏ›˙ÂÙ·È Ì ÙÚfiÔ ‰È·ÊÔÚÂÙÈÎfi ·fi ÂΛÓÔ ÙˆÓ ·ÚˆÌ·ÙÈÎÒÓ ·ÓÙÈÂÈÏËÙÈÎÒÓ. ™˘ÓÈÛÙ¿Ù·È ¿ÓÙˆ˜ ÚÔÛÔ¯‹ ÛÙË ¯ÚËÛÈÌÔÔ›ËÛ‹ ÙÔ˘, ÁÈ·Ù› ·Ó·ÛÙ¤ÏÏÂÈ ÙÔ ¤Ó˙˘ÌÔ ÂÔÍÈ΋ ˘‰ÚÔÏ¿ÛË ÂÈ‚Ú·‰‡ÓÔÓÙ·˜ ÙËÓ ·Ô‚ÔÏ‹ ÙˆÓ ·ÚˆÌ·ÙÈÎÒÓ ·ÓÙÈÂÈÏËÙÈÎÒÓ ·fi ÙÔÓ ÔÚÁ·ÓÈÛÌfi (5). ÕÏÏË ·ÛÊ·Ï‹˜ ÂÓ·ÏÏ·ÎÙÈ΋ ·ÁˆÁ‹ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÔÍ›·˜ Ê¿Û˘ ÙÔ˘ ™À∞ Â›Ó·È Ë Áη̷ÂÓÙ›ÓË.
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¶·È‰È·ÙÚÈ΋ 2001;64:460-464
∂›Ó·È ÚÔÙÈÌfiÙÂÚÔ Ó· ·Ó·‚¿ÏÏÂÙ·È Ë ¤Ó·ÚÍË Ù˘ Ó¤·˜ ·ÓÙÈÂÈÏËÙÈ΋˜ ·ÁˆÁ‹˜ ̤¯ÚÈ Ó· ·Ó·ÚÚÒÛÂÈ Ï‹Úˆ˜ Ô ·ÛıÂÓ‹˜. ¡ÂfiÙÂÚ· ·ÓÙÈÂÈÏËÙÈο, fiˆ˜ Ë Áη̷ÂÓÙ›ÓË, Ë ÙÔÈÚÈÌ¿ÙË Î·È Ë ÙÈ·Áη̛ÓË ıˆÚÔ‡ÓÙ·È ·ÛÊ·Ï›˜, ·ÒÙÂÚ˜ ÂÓ·ÏÏ·ÎÙÈΤ˜ ıÂڷ¢ÙÈΤ˜ ÚÔÔÙÈΤ˜. ∏ Ï·ÌÔÙÚÈÁ›ÓË, ·Ú’ fiÏÔ Ô˘ ¤¯ÂÈ Û˘Û¯ÂÙÈÛÙ› Ì ÙÔ ™À∞, ÌÔÚ›, ›Û˘, Ó· ¯ÚËÛÈÌÔÔÈËı›, ÂÂȉ‹ Ô ÌÂÙ·‚ÔÏÈÎfi˜ Ù˘ ‰ÚfiÌÔ˜ Â›Ó·È ‰È·ÊÔÚÂÙÈÎfi˜ ·fi ÂΛÓÔÓ ÙˆÓ ·ÚˆÌ·ÙÈÎÒÓ ·ÓÙÈÂÈÏËÙÈÎÒÓ (1,10,11). ∞ÛıÂÓ›˜ Ì ™À∞ Ú¤ÂÈ Ó· ÓÔÛËχÔÓÙ·È, Ì ÛÙÂÓ‹ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ Ë·ÙÈ΋˜ Î·È ÓÂÊÚÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, ηıÒ˜ ›Û˘ ÙˆÓ ·ÈÌ·ÙÔÏÔÁÈÎÒÓ ÙÔ˘˜ ‰È·Ù·Ú·¯ÒÓ. ™ËÌ·ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ù˘ ˘ÔÛÙËÚÈÎÙÈ΋˜ ÙÔ˘˜ ıÂڷ›·˜ Â›Ó·È Ë ‰È·Ù‹ÚËÛË Ê˘ÛÈÔÏÔÁÈÎÔ‡ ÈÛÔ˙˘Á›Ô˘ ‡‰·ÙÔ˜ Î·È ËÏÂÎÙÚÔÏ˘ÙÒÓ. O ÚfiÏÔ˜ Ù˘ Û˘ÛÙËÌ·ÙÈ΋˜ ¯ÔÚ‹ÁËÛ˘ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ·Ú·Ì¤ÓÂÈ ·ÌÊÈÏÂÁfiÌÂÓÔ˜ (5). ∆· ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ Ê·›ÓÂÙ·È Ó· ÂÈÙ·¯‡ÓÔ˘Ó ÙËÓ ÂԇψÛË ÙˆÓ ‰ÂÚÌ·ÙÈÎÒÓ ‚Ï·‚ÒÓ, ·ÏÏ¿ Ó· ÌËÓ ÂËÚ¿˙Ô˘Ó ÙËÓ ÂͤÏÈÍË ÙˆÓ ‰È·Ù·Ú·¯ÒÓ ÛÙ· ˘fiÏÔÈ· Û˘ÛÙ‹Ì·Ù·. ¶ÔÏÏÔ› Â›Ó·È ÂΛÓÔÈ Ô˘ ÚÔÙÈÌÔ‡Ó ÙËÓ ÙÔÈ΋ ÂÊ·ÚÌÔÁ‹ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ˘ÁÚ¤˜ ÎÔÌÚ¤Û˜ Î·È ¯ÔÚ‹ÁËÛË ·ÓÙÈÈÛÙ·ÌÈÓÈÎÒÓ. °È· ÙËÓ ÂȂ‚·›ˆÛË Ù˘ ÎÏÈÓÈ΋˜ ‰È¿ÁÓˆÛ˘ ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› Ë ‰ÔÎÈÌ·Û›· ÂϤÁ¯Ô˘ Ù˘ ÏÂÌÊÔ΢ÙÙ·ÚÔÙÔÍÈÎfiÙËÙ·˜ ÙÔ˘ ÂÓÔ¯ÔÔÈÔ‡ÌÂÓÔ˘ Ê·ÚÌ¿ÎÔ˘, Ë ÔÔ›· Ú·ÁÌ·ÙÔÔÈÂ›Ù·È ÌfiÓÔ Û ÂÍÂȉÈÎÂ˘Ì¤Ó· ΤÓÙÚ·. ŒÙÛÈ, ‰ËÌÈÔ˘ÚÁ‹ıËÎÂ Ë ·Ó¿ÁÎË Ì›·˜ ·Ï‹˜, ‰È·ÁÓˆÛÙÈο, ÌÂıfi‰Ô˘. ¶ÚfiÛÊ·Ù· ¤¯Ô˘Ó ·Ú¯›ÛÂÈ Ó· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È patch tests Ì ÙÔ ÂÓÔ¯ÔÔÈÔ‡ÌÂÓÔ Ê¿ÚÌ·ÎÔ, Ì ÂÓı·ÚÚ˘ÓÙÈο ·ÔÙÂϤÛÌ·Ù· (4,12). ∆Ô ıÂÙÈÎfi patch test ÂȂ‚·ÈÒÓÂÈ ÙËÓ ÎÏÈÓÈ΋ ‰È¿ÁÓˆÛË Î·È ÙÚÔÔÔÈ› ÙË ÌÂÏÏÔÓÙÈ΋ ıÂڷ¢ÙÈ΋ ·ÁˆÁ‹ ÙÔ˘ ·ÛıÂÓÔ‡˜. O‰ËÁ›Â˜ ‰›ÓÔÓÙ·È Î·È ÛÙÔ˘˜ Û˘ÁÁÂÓ›˜ ÙÔ˘ ·ÛıÂÓÔ‡˜ Ô˘ ¿Û¯Ô˘Ó ·fi ÂÈÏË„›· Î·È Ï·Ì‚¿ÓÔ˘Ó ·ÓÙÈÂÈÏËÙÈ΋ ·ÁˆÁ‹, ÁÈ· ÙËÓ Èı·ÓfiÙËÙ· Ó· ÂÌÊ·Ó›ÛÔ˘Ó Î·È ÔÈ ›‰ÈÔÈ ™À∞, ·ÊÔ‡ Ê·›ÓÂÙ·È Ó· ÂÌϤÎÔÓÙ·È ÛÙËÓ ·ıÔÁ¤ÓÂÈ¿ ÙÔ˘ Î·È ÎÏËÚÔÓÔÌÈÎÔ› ·Ú¿ÁÔÓÙ˜.
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µÈ‚ÏÈÔÁÚ·Ê›· 1. Carmala C, Vittorio MD, Jennie J, Muglia MD. Anticonvulsant hypersensitivity syndrome. Arch Intern Med 1995;155:2285-2289. 2. Schienger RG, Shear NH. Antiepileptic drug hypersensitivity syndrome. Epilepsia 1998;39(Suppl 7):S3-S6. 3. Jones D, Chhiap V, Resor S, Appel G, Grossman ME. Phenytoin-like hypersensitivity associated with lamotrigine. J Am Acad Dermatol 1997;36:1016-1018. 4. Conilleau V, Dompmartin A, Verneuil L, Michel M, Leroy D. Hypersensitivity syndrome due to 2 anticonvulsant drugs. Contact Dermatitis 1999;41:141-144. 5. Griebel ML. Acute management of hypersensitivity reactions and seisures. Epilepsia 1998;39(Suppl 7):S17-S21. 6. Gilman JT, Duchowny M. Allergic hypersensitivity to antiepileptic drugs: Past, present and future. Epilepsia 1998;39(Suppl 7):S1-S2. 7. Shear NH, Spielberg SP. Anticonvulsant hypersensitivity syndrome: in vitro assessment of risk. J Clin Invest 1988;82:1826-1832. 8. Descamps V, Bouscarat F, Laglene S, Aslangul E, Veber B, Descamps D et al. Human herpesvirus 6 infection associated with anticonvulsant hypersensitivity syndrome and reactive haemophagocytic syndrome. Br J Dermatol 1997;137:605-608. 9. Suzuki Y, Inagi R, Aono T, Yamanishi K, Shiohara T. Human herpesvirus 6 infection as a risk factor for the development of severe drug induced hypersensitivity syndrome. Arch Dermatol 1998; 134:1108-1112. 10. Alldredge BK, Knusten AP, Ferriero D. Antiepileptic drug hypersensitivity syndrome: in vitro and clinical observations. Pediatr Neurol 1994;10:169-171. 11. Handfield-Jones SE, Jerkins RE. The anticonvulsant hypersensitivity syndrome. Br J Dermatol 1993;129:175-177. 12. Mosquera MR, Iglesias A, Saez A, Vidal C. Patch test diagnosis of carbamazepine sensitivity? Contact Dermatitis 1991;25:137-138.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 18-12-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 20-06-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: πˆ¿ÓÓ· ÷ڈӛÙË ∫ÔÓ‰˘Ï¿ÎË 85, 713 05, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘ e-mail: charon@otenet.gr
¶·È‰È·ÙÚÈ΋ 2001;64:465-471
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Paediatriki 2001;64:465-471
ORIGINAL ARTICLE
∂Ô¯È΋ ÌÂÙ·‚ÔÏ‹ Ù˘ ·¤ÎÎÚÈÛ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· °. ∆ÛfiÏ·˜, ¢. ÃÔϤ‚·˜, ™. ∆ÛÔ˘Î·Ï¿, Ã. ™Ù·˘ÚÈÓ¿‰Ë˜, ¶. ∫Ô˘ÎÔ˘ÙÛ¿Î˘, ¶. ™˘Ú›‰Ë˜, ¢. °Ô˘ÚÁÈÒÙ˘, ∫. ™ÈÓ·ÓÈÒÙ˘
Seasonal variation of urinary calcium excretion G. Tsolas, D. Cholevas, S. Tsoukala, C. Stavrinadis, P. Koukoutsakis, P. Spyridis, D. Gouriotis, C. Sinaniotis
¶ÂÚ›ÏË„Ë: ∏ ÁÓÒÛË ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÙÈÌÒÓ ·¤ÎÎÚÈÛ˘ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· ¤¯ÂÈ ÛËÌ·Û›· ÁÈ· ÙË ‰È¿ÁÓˆÛË ·ıÔÏÔÁÈÎÒÓ Î·Ù·ÛÙ¿ÛÂˆÓ Ô˘ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ·˘ÍË̤ÓË ·Ô‚ÔÏ‹ ÙÔ˘. ™Â ÚÔËÁÔ‡ÌÂÓË ÌÂϤÙË Ì·˜ ›¯·Ì ‰È·ÈÛÙÒÛÂÈ fiÙÈ Ë Û¯¤ÛË ÙÔ˘ ËÏ›ÎÔ˘ UCa/UCr ÛÂ Ù˘¯·›Ô ‰Â›ÁÌ· Ô‡ÚˆÓ ÛÂ Ê˘ÛÈÔÏÔÁÈο ·È‰È¿, Ë ÔÔ›· ·ÔÙÂÏ› ‰Â›ÎÙË Ù˘ ·¤ÎÎÚÈÛ˘ ·Û‚ÂÛÙ›Ô˘, Â›Ó·È 0,16±0,10. ªÂ ÛÎÔfi Ó· ‰È·ÈÛÙˆı› Â¿Ó Ë Û¯¤ÛË ·˘Ù‹ ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜, fiˆ˜ ¤¯ÂÈ ·Ú·ÙËÚËı› Û ÂÓ‹ÏÈΘ, ÌÂÏÂÙ‹Û·Ì 150 ˘ÁÈ‹ ·È‰È¿ ËÏÈΛ·˜ 6-12 ÂÙÒÓ Û ‰‡Ô ¯ÚÔÓÈΤ˜ ÂÚÈfi‰Ô˘˜. ∏ ÚÒÙË Ì¤ÙÚËÛË Ù˘ Û¯¤Û˘ UCa/UCr ¤ÁÈÓ ÙÔÓ OÎÙÒ‚ÚÈÔ, ÂÔ¯‹ Ô˘ ·ÓÙÈÛÙÔȯԇÛ Û ÚÔËÁËı›۷ ÛËÌ·ÓÙÈ΋ ¤ÎıÂÛË Û ËÏÈÔÊ¿ÓÂÈ· ηٿ ÙÔ˘˜ ıÂÚÈÓÔ‡˜ Ì‹Ó˜. ∂·Ó·Ï‹ÊıËΠÙÔÓ ∞Ú›ÏÈÔ ÙÔ˘ ÂfiÌÂÓÔ˘ ¤ÙÔ˘˜ Ô˘ ·ÓÙÈÛÙÔȯԇÛ Û ÂÚ›Ô‰Ô ¯·ÌËÏ‹˜ ËÏÈÔÊ¿ÓÂÈ·˜ ÙÔ˘ ¯ÂÈÌÒÓ· Ô˘ ›¯Â ÚÔËÁËı›. ∞fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ ‰È·ÈÛÙÒıËΠfiÙÈ ÔÈ ÙÈ̤˜ UCa/UCr ηٿ ÙËÓ ÚÒÙË Ì¤ÙÚËÛË (OÎÙÒ‚ÚÈÔ˜) ‹Ù·Ó ˘„ËÏfiÙÂÚ˜ Ù˘ ‰Â‡ÙÂÚ˘ ̤ÙÚËÛ˘ (∞Ú›ÏÈÔ˜), 0,17±0,11 Î·È 0,14±0,12 ·ÓÙ›ÛÙÔȯ· (p=0,022). OÈ ÙÈ̤˜ ·˘Ù¤˜ ‹Ù·Ó ·ÓÙ›ÛÙÔȯ˜ ·˘ÙÒÓ Ô˘ ›¯·Ó ‰È·ÈÛÙˆı› ÛÂ Ê˘ÛÈÔÏÔÁÈο ·È‰È¿ Ù˘ ÚÔËÁÔ‡ÌÂÓ˘ ÌÂϤÙ˘. ∫·Ù¿ ÙËÓ ÚÒÙË ÂÚ›Ô‰Ô, ‰È·ÈÛÙÒıËΠ·˘ÍË̤ÓË ·Ô‚ÔÏ‹ ·Û‚ÂÛÙ›Ô˘ (UCa/UCr >0,35) Û 11 ·È‰È¿ Î·È ·Ú¤ÌÂÈÓ ·˘ÍË̤ÓË Î·Ù¿ ÙË ‰Â‡ÙÂÚË ÂÚ›Ô‰Ô Û 3 ·fi ·˘Ù¿. ∆¤ÏÔ˜, ‰ÂÓ ‰È·ÈÛÙÒıËΠ‰È·ÊÔÚ¿ ÛÙËÓ ·Ô‚ÔÏ‹ ·Û‚ÂÛÙ›Ô˘ ÌÂٷ͇ ·ÁÔÚÈÒÓ Î·È ÎÔÚÈÙÛÈÒÓ. ™˘ÌÂÚ·›ÓÂÙ·È fiÙÈ Ë ·¤ÎÎÚÈÛË ·Û‚ÂÛÙ›Ô˘ ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ Î·È ÂÔ̤ӈ˜ Ù˘¯fiÓ ˘„ËϤ˜ ÙÈ̤˜ ·¤ÎÎÚÈÛ˘ ı· Ú¤ÂÈ Ó· ÂÎÙÈÌÒÓÙ·È ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÔ¯‹.
Abstract: Hypercalciuria is of continuing interest since it is one of the risk factors for stone disease in children. The measurement of the urinary calcium/creatinine concentration ratio (UCa/UCr) expressed in mg/dl per mg/dl, is an accepted screening test for elevated calcium excretion. Therefore, the knowledge of reference values for UCa/UCr ratio in children is very important. In a previous study we had found that UCa/UCr ratio for normal children is 0.16±0.10. In the present study we evaluated the possibility of seasonal variation in urinary calcium excretion in 150 healthy children, 6-12 year-old (82 boys, 68 girls), during two different seasons. Calcium excretion was studied in October, following exposure to summer sunshine, and after six months, in April, following a decreased exposure to sunlight during the winter months. It was found that there is a distinct seasonal variation in urinary calcium excretion (0.17±0.11 in October vs 0.14±0.12 in April, p=0.022). Eleven children were found to have an increased ratio of UCa/UCr (>0.35) in the first study period, but only three of them continued to have increased UCa/UCr ratios after the winter season. No difference was found between boys and girls. The findings of this study suggest that urinary calcium excretion is influenced by the season of the year, probably because of differences in sunshine exposure. This parameter should be taken into account when evaluating children for hypercalciuria.
µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶. & ∞. ∫˘ÚÈ·ÎÔ‡”
2nd Dept. of Paediatrics of University of Athens “ƒ. & ∞. Kyriakou” Children's Hospital, Athens
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¶·È‰È·ÙÚÈ΋ 2001;64:465-471
Paediatriki 2001;64:465-471
§¤ÍÂȘ ÎÏÂȉȿ: ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›·, ·¤ÎÎÚÈÛË ·Û‚ÂÛÙ›Ô˘, ËÏ›ÎÔ ·Û‚ÂÛÙ›Ô˘ ÚÔ˜ ÎÚ·ÙÈÓ›ÓË Ô‡ÚˆÓ, ÂÔ¯È΋ ηٷÓÔÌ‹, ËÏÈÔÊ¿ÓÂÈ·.
Key words: hypercalciuria, calcium excretion, urinary calcium-creatinine ratio, seasonal variation, sunshine exposure.
∂ÈÛ·ÁˆÁ‹ ∏ ȉÈÔ·ı‹˜ ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›· ·ÔÙÂÏ› ÙË Û˘¯ÓfiÙÂÚË ·ÈÙ›· Ô˘ÚÔÏÈı›·Û˘ ÛÙËÓ ·È‰È΋ ËÏÈΛ· Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÌÂÁ¿ÏË ·Ô‚ÔÏ‹ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú·, ÂÓÒ Ù· ›‰· ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÙÔ˘ ÔÚÔ‡ Â›Ó·È Ê˘ÛÈÔÏÔÁÈο (1,2) (¶›Ó·Î·˜ 1). ∞˘ÍË̤ÓË ·Ô‚ÔÏ‹ ·Û‚ÂÛÙ›Ô˘ ¤¯ÂÈ ‰È·ÈÛÙˆı› ÛÙÔ 50-80% ÂÚ›Ô˘ ÙˆÓ ·È‰ÈÒÓ Ì ÏÈı›·ÛË, ÂÓÒ Û¯ÂÙ›˙ÂÙ·È Î·È Ì ÙËÓ È‰ÈÔ·ıÔ‡˜ ·ÈÙÈÔÏÔÁ›·˜ ·ÈÌ·ÙÔ˘Ú›· ÛÙ· ·È‰È¿. ™˘ÓÂÒ˜, Ë ÁÓÒÛË ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÙÈÌÒÓ ÙÔ˘ ·Ô‚·ÏÏfiÌÂÓÔ˘ Ì ٷ Ô‡Ú· ·Û‚ÂÛÙ›Ô˘ ¤¯ÂÈ ÌÂÁ¿ÏË ÛËÌ·Û›· ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË. ¶·Ú¿ Ù·‡Ù·, Ù· ÛÙÔȯ›· Ù· ÔÔ›· ˘¿Ú¯Ô˘Ó Û¯ÂÙÈο Ì ÙËÓ ·¤ÎÎÚÈÛ‹ ÙÔ˘ ÛÙ· Ô‡Ú· Â›Ó·È ÂÚÈÔÚÈṲ̂ӷ, Ù· ‰Â ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ‰È·ÊfiÚˆÓ ÂÚ¢ÓÒÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ÙÔ˘˜. ªÂ ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ ¤¯ÂÈ ‰È·ÈÛÙˆı› fiÙÈ ÔÈ ÙÈ̤˜ ÙÔ˘ ·Ô‚·ÏÏfiÌÂÓÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ·fi ¯ÒÚ· Û ¯ÒÚ·, ÂÓÒ ·ÍÈfiÏÔÁ˜ ‰È·ÊÔÚ¤˜ ¤¯Ô˘Ó ‰È·ÈÛÙˆı› Î·È ÛÙȘ ›‰È˜ ÙȘ ¯ÒÚ˜ ÛÙȘ Ôԛ˜ ¤ÁÈÓ·Ó Û¯ÂÙÈΤ˜ ÌÂϤÙ˜. Œ¯ÂÈ ˘ÔÛÙËÚȯı› fiÙÈ ÔÈ ‰È·ÊÔÚ¤˜ ·˘Ù¤˜ ÔÊ›ÏÔÓÙ·È Û ‰È¿ÊÔÚÔ˘˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ ηٿ ÙËÓ ÔÔ›· ¤ÁÈÓÂ Ë ÌÂϤÙË ÏfiÁˆ Ù˘ ‰È·ÊÔÚÂÙÈ΋˜ ËÏÈÔÊ¿ÓÂÈ·˜, Ë ‰È·ÙÚÔÊ‹, Ô ÎÈÚο‰ÈÔ˜ Ú˘ıÌfi˜ ·Ô‚ÔÏ‹˜ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· Î.Ï. (3-17). ™Â ÚÔËÁÔ‡ÌÂÓË ÌÂϤÙË Ì·˜ (18) ›¯·Ì ‰È·ÈÛÙÒÛÂÈ ÛÂ Ê˘ÛÈÔÏÔÁÈο ·È‰È¿ fiÙÈ Ë Û¯¤ÛË ·Û‚ÂÛÙ›Ô˘ ÚÔ˜ ÎÚ·ÙÈÓ›ÓË ÛÂ Ù˘¯·›Ô ‰Â›ÁÌ· Ô‡ÚˆÓ, Ë ÔÔ›· ıˆÚÂ›Ù·È fiÙÈ ·ÔÙÂÏ› ‰Â›ÎÙË Ù˘ ·¤ÎÎÚÈÛ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú·, ‹Ù·Ó 0,16±0,10. ™ÙË ÌÂϤÙË, fï˜, ÂΛÓË ‰ÂÓ Â›¯·Ó Û˘ÓÂÎÙÈÌËı› ¿ÏϘ ·Ú¿ÌÂÙÚÔÈ, fiˆ˜ Ë ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ ηٿ ÙËÓ ÔÔ›· ‰ÈÂÍ‹¯ıË Ë ÌÂϤÙË Î·È Ë ‰È·ÙÚÔÊ‹ ÙˆÓ ·È-
‰ÈÒÓ Ù· ÔÔ›· Û˘ÌÌÂÙ›¯·Ó Û ·˘Ù‹. ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ó· ‰È·ÈÛÙˆı› Â¿Ó Ë ·¤ÎÎÚÈÛË ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· Ê˘ÛÈÔÏÔÁÈÎÒÓ ·È‰ÈÒÓ ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ Î·È ÂÊ’ fiÛÔÓ ‰È·ÈÛÙˆı› fiÙÈ ˘¿Ú¯ÂÈ fiÓÙˆ˜ ‰È·ÊÔÚ¿ ÛÙË Û˘ÁΤÓÙÚˆÛË ÙÔ˘ ·ÂÎÎÚÈÓfiÌÂÓÔ˘ ÛÙ· Ô‡Ú· ·Û‚ÂÛÙ›Ô˘ ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜, Ó· ‰Ôı› ÈηÓÔÔÈËÙÈ΋, ηٿ ÙÔ ‰˘Ó·ÙfiÓ, ÂÚÌËÓ›· ÛÙÔ Ê·ÈÓfiÌÂÓÔ ·˘Ùfi Î·È Ó· Û˘˙ËÙËı› Ë Ú·ÎÙÈ΋ ÛËÌ·Û›· ÙÔ˘.
¶›Ó·Î·˜ 1. ÀÂÚ·Û‚ÂÛÙÈÔ˘Ú›· ñ π‰ÈÔ·ı‹˜ ·) ∞˘ÍË̤ÓË ·ÔÚÚfiÊËÛË ·Û‚ÂÛÙ›Ô˘ ·fi ÙÔ ¤ÓÙÂÚÔ ‚) ∞˘ÍË̤ÓË ·Ô‚ÔÏ‹ ·Û‚ÂÛÙ›Ô˘ ·fi ÓÂÊÚfi ñ ¢Â˘ÙÂÚÔ·ı‹˜ - ™ˆÏËÓ·Úȷ΋ ÔͤˆÛË Ù‡Ô˘ π (ÎÏ·ÛÈ΋) - ™‡Ó‰ÚÔÌÔ Bartter - ¡Â·ÓÈ΋ ÚÂ˘Ì·ÙÔÂȉ‹˜ ·ÚıÚ›Ùȉ· - ∞ÎÈÓËÛ›· - ¶ÚˆÙÔ·ı‹˜ ˘ÂÚ·Ú·ı˘ÚÂÔÂȉÈÛÌfi˜ - ÀÂÚı˘ÚÂÔÂȉÈÛÌfi˜ - ™·ÚÎÔ›‰ˆÛË Î.Ï.
466
ÀÏÈÎfi Î·È ÌÂıÔ‰ÔÏÔÁ›· ™ÙË ÌÂϤÙË Û˘ÌÌÂÙ›¯·Ó 150 ˘ÁÈ‹ ·È‰È¿ ËÏÈΛ·˜ 6-12 ÂÙÒÓ, Ù· ÔÔ›· ÊÔÈÙÔ‡Û·Ó Û ‰ËÌÔÙÈο Û¯ÔÏ›· ÙˆÓ ÂÚÈÔ¯ÒÓ ¶·¿ÁÔ˘ Î·È µ‡ÚˆÓ· ÙÔ˘ ÓÔÌÔ‡ ∞ÙÙÈ΋˜. ∆· 82 ‹Ù·Ó ·ÁfiÚÈ· Î·È Ù· 68 ÎÔÚ›ÙÛÈ·. ∏ ÌÂϤÙË ‰ÈÂÍ‹¯ıË Û ‰‡Ô ¯ÚÔÓÈΤ˜ ÂÚÈfi‰Ô˘˜. ∏ ÚÒÙË Ì¤ÙÚËÛË Ù˘ ·¤ÎÎÚÈÛ˘ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· ¤ÁÈÓ ÙÔÓ OÎÙÒ‚ÚÈÔ, ÂÔ¯‹ Ë ÔÔ›· ·ÓÙÈÛÙÔȯԇÛ Û ÛËÌ·ÓÙÈ΋ ÚÔËÁËı›۷ ¤ÎıÂÛË Û ËÏÈÔÊ¿ÓÂÈ· ηٿ ÙÔ˘˜ ıÂÚÈÓÔ‡˜ Ì‹Ó˜ Ô˘ ÚÔËÁ‹ıËÎ·Ó Ù˘ ÌÂϤÙ˘ Î·È Â·Ó·Ï‹ÊıËÎÂ, ÛÙ· 140 ·fi Ù· ›‰È· ·ÎÚÈ‚Ò˜ ·È‰È¿, ÙÔÓ ∞Ú›ÏÈÔ ÙÔ˘ ÂfiÌÂÓÔ˘ ¤ÙÔ˘˜ Ô˘ ·ÓÙÈÛÙÔȯԇÛ Û ÚÔËÁËı›۷ ÂÚ›Ô‰Ô ¯·ÌËÏ‹˜ ËÏÈÔÊ¿ÓÂÈ·˜ ÏfiÁˆ ÙÔ˘ ¯ÂÈÌÒÓ· Ô˘ ›¯Â ÚÔËÁËı›. ¢¤Î· ·fi Ù· 150 ·È‰È¿ ‰ÂÓ ÚÔÛ‹Ïı·Ó ηٿ ÙË ‰Â‡ÙÂÚË Ì¤ÙÚËÛË ÙÔ˘ ∞ÚÈÏ›Ô˘. ™ÙÔȯ›·, fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ËÏÈÔÊ¿ÓÂÈ· Î·È Û˘ÁÎÂÎÚÈ̤ӷ ÙȘ ÌËÓÈ·›Â˜ ÙÈ̤˜ ËÏÈÔÊ¿ÓÂÈ·˜ Û ÒÚ˜, ÂÏ‹ÊıËÛ·Ó ·fi ÙËÓ ∂ıÓÈ΋ ªÂÙˆÚÔÏÔÁÈ΋ ÀËÚÂÛ›· Î·È ÚÔ¤Ú¯ÔÓÙ·È ·fi ÌÂÙÚ‹ÛÂȘ ÔÈ Ôԛ˜ ¤ÁÈÓ·Ó ÛÙÔ ªÂÙˆÚÔÏÔÁÈÎfi ™Ù·ıÌfi Ù˘ ¡¤·˜ ºÈÏ·‰¤ÏÊÂÈ·˜. ™Â fiÏ· Ù· ·È‰È¿, ÙfiÛÔ ÙÔÓ OÎÙÒ‚ÚÈÔ fiÛÔ Î·È ÙÔÓ ∞Ú›ÏÈÔ, ÂÏ‹ÊıËÛ·Ó Ù· ‰Â‡ÙÂÚ· ÚˆÈÓ¿ Ô‡Ú·. ∏ ÂÈÏÔÁ‹ ÙÔ˘ Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ‰Â›ÁÌ·ÙÔ˜ ¤ÁÈÓ ÁÈ· Ú·ÎÙÈÎÔ‡˜ ÏfiÁÔ˘˜, ÂÓÒ Î·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· Ë Ï‹„Ë ÙˆÓ ‰Â‡ÙÂÚˆÓ ÚˆÈÓÒÓ Ô‡ÚˆÓ ıˆÚÂ›Ù·È ·Ô‰ÂÎÙ‹ Î·È ·ÍÈfiÈÛÙË. ¶Ú¤ÂÈ Ó· ÛËÌÂȈı› fiÙÈ fiÏ· Ù· ·È‰È¿ Ô˘ Û˘ÌÌÂÙ›¯·Ó ÛÙË ÌÂϤÙË ‰ÂÓ Â›¯·Ó ¿ÚÂÈ ÚfiÁÂ˘Ì· Ì Á¿Ï· ‹ Á·Ï·ÎÙÔÎÔÌÈο ÚÔ˚fiÓÙ· ηٿ ÙȘ ‰‡Ô Ë̤Ú˜ - ÙÔÓ OÎÙÒ‚ÚÈÔ Î·È ÙÔÓ ∞Ú›ÏÈÔ, ·ÓÙ›ÛÙÔȯ· - ‰ÈÂÍ·ÁˆÁ‹˜ Ù˘ Û˘ÏÏÔÁ‹˜ ÙˆÓ Ô‡ÚˆÓ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔÎÏÂÈÛÙ› Ë ·ÔÚÚfiÊËÛË ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ Ù˘ ÙÚÔÊ‹˜ ·fi ÙÔ ¤ÓÙÂÚÔ. ∏ ̤ÙÚËÛË ÙˆÓ Û˘ÁÎÂÓÙÚÒÛÂˆÓ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ Î·È Ù˘ ÎÚ·ÙÈÓ›Ó˘ ÛÙ· Ô‡Ú· ¤ÁÈÓ ÛÙÔ ∂Ú¢ÓËÙÈÎfi ∂ÚÁ·ÛÙ‹ÚÈÔ Ù˘ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ Ì ÙË Ì¤ıÔ‰Ô Ù˘ ·ÙÔÌÈ΋˜ ·ÔÚÚfiÊËÛ˘. ∂›Û˘, ˘Ô‚Ï‹ıËΠÏÂÙÔÌÂÚ¤˜ ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ, ÚÈÓ ·fi ÙËÓ Î¿ı ‰ÂÈÁÌ·ÙÔÏË„›·, ÛÙÔ˘˜ ÁÔÓ›˜ fiÏˆÓ ÙˆÓ ·È‰ÈÒÓ Ô˘ Û˘ÌÌÂÙ›¯·Ó ÛÙË ÌÂϤÙË, fiÛÔÓ ·ÊÔÚ¿ ÛÙË ‰È·ÙÚÔÊ‹, Ì ¤ÌÊ·ÛË ÛÙË Ï‹„Ë ÙÚÔÊÒÓ ÏÔ‡ÛÈˆÓ Û ·Û‚¤ÛÙÈÔ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰È·ÈÛÙˆıÔ‡Ó
¶·È‰È·ÙÚÈ΋ 2001;64:465-471
Paediatriki 2001;64:465-471
Ù˘¯fiÓ ‰È·ÊÔÚ¤˜ ÛÙËÓ ÔÛfiÙËÙ· ÙÔ˘ ËÌÂÚËÛ›ˆ˜ ÚÔÛÏ·Ì‚·ÓfiÌÂÓÔ˘ ·Û‚ÂÛÙ›Ô˘, ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜. ∆Ô ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ ÂÚÈÂÏ¿Ì‚·Ó ηٷÁÚ·Ê‹ Ù˘ ËÌÂÚËÛ›ˆ˜ ÚÔÛÏ·Ì‚·ÓfiÌÂÓ˘ ÔÛfiÙËÙ·˜ Á¿Ï·ÎÙÔ˜, Á·Ï·ÎÙÔÎÔÌÈÎÒÓ ÚÔ˚fiÓÙˆÓ, ·ÏÏ¿ Î·È ¿ÏÏˆÓ ÏÔ˘Û›ˆÓ Û ·Û‚¤ÛÙÈÔ ÙÚÔÊÒÓ (·˘Á¿, Ï·¯·ÓÈο, ÂÏȤ˜, ÍËÚÔ› ηÚÔ›, ۇη, ÏÔÈ¿ ÊÚÔ‡Ù·) Î·È ˘ÔÏÔÁÈ˙fiÙ·Ó Ì ÙË ÌÂÁ·Ï‡ÙÂÚË ‰˘Ó·Ù‹ ÚÔÛ¤ÁÁÈÛË Ë Ì¤ÛË ËÌÂÚ‹ÛÈ· ÚfiÛÏË„Ë ·Û‚ÂÛÙ›Ô˘, ÙfiÛÔ ÁÈ· ÙÔ “ıÂÚÈÓfi” ÂÍ¿ÌËÓÔ ª·˝Ô˘ - OÎÙˆ‚Ú›Ô˘, ÙÔ ÔÔ›Ô ÚÔËÁ‹ıËÎ Ù˘ ÚÒÙ˘ ÌÂÙÚ‹Ûˆ˜, fiÛÔ Î·È ÁÈ· ÙÔ “¯ÂÈÌÂÚÈÓfi” ÂÍ¿ÌËÓÔ ¡ÔÂÌ‚Ú›Ô˘ - ∞ÚÈÏ›Ô˘ Ô˘ ÚÔËÁ‹ıËÎ Ù˘ ‰Â‡ÙÂÚ˘ ÌÂÙÚ‹Ûˆ˜. ∏ ÛÙ·ÙÈÛÙÈ΋ ÂÂÍÂÚÁ·Û›· ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ¤ÁÈÓ Ì ÙË ‰ÔÎÈÌ·Û›· t-test ·Ó¿ ˙‡ÁË.
∞ÔÙÂϤÛÌ·Ù· ∏ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Ù˘ ÌÂϤÙ˘ ¤‰ÂÈÍ fiÙÈ: ·) OÈ ÙÈ̤˜ ·Û‚ÂÛÙ›Ô˘ ÚÔ˜ ÎÚ·ÙÈÓ›ÓË Ô‡ÚˆÓ (UCa/UCr) ηٿ ÙËÓ ÚÒÙË Ì¤ÙÚËÛË ÙÔ˘ OÎÙˆ‚Ú›Ô˘ ‹Ù·Ó ˘„ËÏfiÙÂÚ˜ (̤ÛË ÙÈÌ‹: 0,17±0,11) ·fi ÙȘ ÙÈ̤˜ Ù˘ ‰Â‡ÙÂÚ˘ ̤ÙÚËÛ˘ ÙÔ˘ ∞ÚÈÏ›Ô˘ (̤ÛË ÙÈÌ‹: 0,14±0,12), p=0,022 Î·È ‹Ù·Ó ·ÓÙ›ÛÙÔȯ˜ ÂΛӈÓ, ÔÈ Ôԛ˜ ›¯·Ó ‰È·ÈÛÙˆı› ÛÂ Ê˘ÛÈÔÏÔÁÈο ·È‰È¿ Ù˘ ÚÔËÁÔ‡ÌÂÓ˘ ÌÂϤÙ˘ Ì·˜ (18). ∞fi Ù· ÛÙÔȯ›· Ù˘ ∂ıÓÈ΋˜ ªÂÙˆÚÔÏÔÁÈ΋˜ ÀËÚÂÛ›·˜ ‰È·ÈÛÙÒıËΠfiÙÈ Ë Ì¤ÛË ËÏÈÔÊ¿ÓÂÈ· ηٿ ÙÔ ÂÍ¿ÌËÓÔ ª·˝Ô˘ - OÎÙˆ‚Ú›Ô˘, ÙÔ ÔÔ›Ô ÚÔËÁ‹ıËÎ Ù˘ ÚÒÙ˘ ÌÂÙÚ‹Ûˆ˜ ‹Ù·Ó
1645 ÒÚ˜, ۯ‰fiÓ ‰ÈÏ¿ÛÈ· ÙˆÓ 877 ˆÚÒÓ Ù˘ ·ÓÙ›ÛÙÔȯ˘ ÂÚÈfi‰Ô˘ ¡ÔÂÌ‚Ú›Ô˘ - ∞ÚÈÏ›Ô˘ Ô˘ ÚÔËÁ‹ıËÎ Ù˘ ‰Â‡ÙÂÚ˘ ̤ÙÚËÛ˘. O˘ÛÈ·ÛÙÈΤ˜ ‰È·ÊÔÚ¤˜, fiÛÔÓ ·ÊÔÚ¿ ÛÙË ‰È·ÙÚÔÊ‹ Û ۯ¤ÛË Ì ÙËÓ ÔÛfiÙËÙ· ÙÔ˘ ËÌÂÚËÛ›ˆ˜ ÚÔÛÏ·Ì‚·ÓfiÌÂÓÔ˘ ·Û‚ÂÛÙ›Ô˘ ÌÂٷ͇ ÙˆÓ ‰‡Ô ÂÚÈfi‰ˆÓ, ‰ÂÓ ‰È·ÈÛÙÒıËηÓ. ™ÙÔ ™¯‹Ì· 1 ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÔÈ ÙÈ̤˜ ·¤ÎÎÚÈÛ˘ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· ÛÙȘ ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜ ÌÂÙÚ‹ÛÂȘ. ∂›Ó·È ÚÔÊ·Ó¤˜ fiÙÈ ÂÓÒ Î·Ù¿ ÙÔÓ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ Û˘ÁΤÓÙÚˆÛ˘ ÙÔ˘ ·ÂÎÎÚÈÓfiÌÂÓÔ˘ ÛÙ· Ô‡Ú· ·Û‚ÂÛÙ›Ô˘ ÙÔÓ OÎÙÒ‚ÚÈÔ, Ù· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ ¤¯Ô˘Ó ÙÈÌ‹ UCa/UCr Á‡Úˆ ÛÙÔ 0,15 ‹ Î·È ÌÂÁ·Ï‡ÙÂÚË, ÛÙËÓ Â·ÓÂÎÙ›ÌËÛË ÙÔ˘ ∞ÚÈÏ›Ô˘ Ë Û˘ÓÙÚÈÙÈ΋ ÏÂÈÔ„ËÊ›· ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ Â›¯·Ó ÙÈÌ‹ UCa/UCr <0,15. ‚) ™ÙÔÓ ¶›Ó·Î· 2 ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ ÙÈ̤˜, ÔÈ Ôԛ˜ ·ÓÙÈÛÙÔÈ¯Ô‡Ó ÛÙȘ ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ ÙÔ˘ ËÏ›ÎÔ˘ UCa/UCr, ÙfiÛÔ Î·Ù¿ ÙË Ì¤ÙÚËÛË ÙÔ˘ OÎÙˆ‚Ú›Ô˘, fiÛÔ Î·È Î·Ù¿ ÙË Ì¤ÙÚËÛË ÙÔ˘ ∞ÚÈÏ›Ô˘. ∞fi ÙË ÌÂϤÙË ÙÔ˘ ›Ó·Î· ‰È·ÈÛÙÒÓÂÙ·È fiÙÈ ÔÈ ÙÈ̤˜ UCa/UCr Â›Ó·È ·ÚÎÂÙ¿ ˘„ËÏfiÙÂÚ˜ ÙÔ˘ ËÏ›ÎÔ˘ 0,25, Ô˘ ¤¯ÂÈ ıˆÚËı› fiÙÈ ·ÔÙÂÏ› ÙË Ê˘ÛÈÔÏÔÁÈ΋ ÙÈÌ‹. ∂Ô̤ӈ˜, ÙÈ̤˜ ·ÚÎÂÙ¿ ˘„ËÏfiÙÂÚ˜ ÙÔ˘ 0,25 ı· ÌÔÚÔ‡Û·Ó Ó· ıˆÚËıÔ‡Ó ˆ˜ Ê˘ÛÈÔÏÔÁÈΤ˜ ÁÈ· Ù· ÂÏÏËÓÈο ‰Â‰Ô̤ӷ. Á) ¢È·ÊÔÚ¿ ÙÈÌÒÓ ÌÂٷ͇ ÙˆÓ ‰‡Ô Ê‡ÏˆÓ ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó Î·Ù¿ ÙȘ ‰‡Ô ÌÂÙÚ‹ÛÂȘ. ‰) ∏ Û‡ÁÎÚÈÛË ÙˆÓ ÙÈÌÒÓ UCa/UCr ÌÂٷ͇
35
™¯ÂÙÈ΋ Û˘¯ÓfiÙËÙ· (%)
30
O∫∆øµƒπO™ A¶ƒπ§πO™
25 20 15 10 5 0 -0,05 0
0,05
0,1
0,15
0,2
0,25
0,3
0,35
0,4
0,45
0,5
0,55
0,6
0,65 0,7
Ca/Cr{(mg/dl)/(mg/dl)} ™¯‹Ì· 1. ∞¤ÎÎÚÈÛË ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· Ê˘ÛÈÔÏÔÁÈÎÒÓ ·È‰ÈÒÓ Û ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜ ÂÔ¯¤˜ ÙÔ˘ ¤ÙÔ˘˜
467
¶·È‰È·ÙÚÈ΋ 2001;64:465-471
Paediatriki 2001;64:465-471
¶›Ó·Î·˜ 2. ∂ηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ Û¯¤Û˘ Ca/Cr Ô‡ÚˆÓ [(mg/dl) / (mg/dl)] Ê˘ÛÈÔÏÔÁÈÎÒÓ ·È‰ÈÒÓ Û ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜ ÂÔ¯¤˜ ÙÔ˘ ¤ÙÔ˘˜ UCa/UCr [(mg/dl) / (mg/dl)] ∂.£.
5 25 50 75 95
OÎÙÒ‚ÚÈÔ˜ (ÌÂÁ¿ÏË ÚÔËÁËı›۷ ËÏÈÔÊ¿ÓÂÈ·) 0,03 0,10 0,15 0,23 0,37
∞Ú›ÏÈÔ˜ (ÌÂȈ̤ÓË ÚÔËÁËı›۷ ËÏÈÔÊ¿ÓÂÈ·) 0,02 0,05 0,11 0,18 0,39
OÎÙˆ‚Ú›Ô˘ Î·È ∞ÚÈÏ›Ô˘, ͯˆÚÈÛÙ¿ ÁÈ· οı ¤ÙÔ˜ ËÏÈΛ·˜, ¤‰ÂÈÍ fiÙÈ ˘¿Ú¯ÂÈ ‰È·ÊÔÚ¿ ÌfiÓÔ ÛÙ· ·È‰È¿ ËÏÈΛ·˜ 7, 8 Î·È 9 ÂÙÒÓ, ÂÓÒ ÛÙȘ ˘fiÏÔȘ ËÏÈ˘ (6, 10 Î·È 11 ÂÙÒÓ) ‰ÂÓ ‰È·ÈÛÙÒıËΠÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ (¶›Ó·Î·˜ 3). Â) ¶·ıÔÏÔÁÈ΋ ÙÈÌ‹ ÙÔ˘ ·Ô‚·ÏÏfiÌÂÓÔ˘ ·Û‚ÂÛÙ›Ô˘ (UCa/UCr >0,35) ‰È·ÈÛÙÒıËΠ۠11 ·È‰È¿ ηٿ ÙËÓ ÚÒÙË Ì¤ÙÚËÛË ÙÔ˘ OÎÙˆ‚Ú›Ô˘. ∫·Ù¿ ÙÔÓ Â·Ó¤ÏÂÁ¯Ô ÙÔÓ ∞Ú›ÏÈÔ, ÛÙÔÓ ÔÔ›Ô Û˘ÌÌÂÙ›¯·Ó Ù· 10 ÂÍ ·˘ÙÒÓ, ‰È·ÈÛÙÒıËΠfiÙÈ ÛÙ· 7 ·È‰È¿ ÔÈ ÙÈ̤˜ ›¯·Ó ˘Ô¯ˆÚ‹ÛÂÈ ÛÂ Ê˘ÛÈÔÏÔÁÈο ›‰·, ÂÓÒ 3 ÌfiÓÔ ·È‰È¿ ÂÍ·ÎÔÏÔ˘ıÔ‡Û·Ó Ó· ¤¯Ô˘Ó ÙÈÌ‹ ÌÂÁ·Ï‡ÙÂÚË ÙÔ˘ 0,35. ∆· ·È‰È¿ ·˘Ù¿ ‹Ù·Ó ·Û˘Ìو̷ÙÈο, Ô ‰Â ÏÂÙÔÌÂÚ‹˜ ·Ú·ÎÏÈÓÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÛÙÔÓ ÔÔ›Ô ˘Ô‚Ï‹ıËÎ·Ó ·¤‚Ë ·ÚÓËÙÈÎfi˜ ÁÈ· ·ÈÌ·ÙÔ˘Ú›·, Ô˘ÚÔÏÈı›·ÛË ‹ ¿ÏÏÔ ·ıÔÏÔÁÈÎfi ‡ÚËÌ· (¶›Ó·Î·˜ 4). ™˘˙‹ÙËÛË ∆Ô ‚·ÛÈÎfi Û˘Ì¤Ú·ÛÌ· Ô˘ ÚÔ·ÙÂÈ ·fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Ù˘ ÌÂϤÙ˘ Ì·˜, Ì ڷÎÙÈ΋ ·Í›·, Â›Ó·È Ë ‰È·ÊÔÚ¿ Ô˘ ‰È·ÈÛÙÒıËΠÛÙËÓ ·Ô‚ÔÏ‹ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ Î·È Ë ÔÔ›· Ê·›ÓÂÙ·È fiÙÈ ÔÊ›ÏÂÙ·È Î˘Ú›ˆ˜ ÛÙË ‰È·ÊÔÚÂÙÈ΋ ËÏÈÔÊ¿ÓÂÈ·. ∂Ó‰¤¯ÂÙ·È Ì¿ÏÈÛÙ· Ó· Â›Ó·È ÙfiÛÔ ÌÂÁ¿ÏË Ë Âԯȷ΋ ·˘Ù‹ ‰È·Î‡Ì·ÓÛË Ù˘ ·¤ÎÎÚÈÛ˘ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘, ÒÛÙ ÌÂÚÈο ·È‰È¿ Ó· ·ÚÔ˘ÛÈ¿˙Ô˘Ó Âԯȷ΋ ·ÚÔ‰È΋ ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›·, fiˆ˜ ‰È·ÈÛÙÒıËÎÂ Î·È ÛÙË ‰È΋ Ì·˜ ÌÂϤÙË, ÛÙËÓ ÔÔ›· ·fi Ù· 11 ·È‰È¿ Ì ÙÈÌ‹ UCa/UCr >0,35 ÛÙË Ì¤ÙÚËÛË ÙÔ˘ OÎÙˆ‚Ú›Ô˘, ÛÙ· 8 Ë ÙÈÌ‹ ÙÔ˘ ËÏ›ÎÔ˘ ·ӋÏı ÛÂ Ê˘ÛÈÔÏÔÁÈο ›‰· ηٿ ÙË Ó¤· ̤ÙÚËÛË Ô˘ ¤ÁÈÓ ÌÂÙ¿ 6 Ì‹Ó˜. ∆· Â˘Ú‹Ì·Ù· ·˘Ù¿ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë ÙÈÌ‹ ÙÔ˘ ·Ô‚·ÏÏfiÌÂÓÔ˘ ÛÙ· Ô‡Ú· ·Û‚ÂÛÙ›Ô˘ ı· Ú¤ÂÈ Ó· ÂÎÙÈÌ¿Ù·È, ÂÎÙfi˜ ÙˆÓ ¿ÏψÓ, Î·È Ì ‚¿ÛË ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜, ηٿ ÙËÓ ÔÔ›· ¤Ï·‚ ¯ÒÚ· Ë Ì¤ÙÚËÛË. ∏ ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›· ·ÔÙÂÏ› ÙË Û˘¯ÓfiÙÂÚË ·È-
468
Ù›· Ô˘ÚÔÏÈı›·Û˘ ÛÙËÓ ·È‰È΋ ËÏÈΛ· (¶›Ó·Î·˜ 1). ™˘¯ÓfiÙÂÚË ÌÔÚÊ‹ ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›·˜ Â›Ó·È Ë È‰ÈÔ·ı‹˜, Ë ÔÔ›· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÌÂÁ¿ÏË ·Ô‚ÔÏ‹ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú·, ÂÓÒ ÙÔ ·Û‚¤ÛÙÈÔ ÙÔ˘ ÔÚÔ‡ Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi. Œ¯ÂÈ ·Ó·ÊÂÚı› fiÙÈ ··ÓÙ¿Ù·È ÛÙÔ 50-80% ÂÚ›Ô˘ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÏÈı›·Û˘ ηٿ ÙËÓ ·È‰È΋ ËÏÈΛ·, ÂÓÒ Û¯ÂÙ›˙ÂÙ·È Î·È Ì ÙËÓ È‰ÈÔ·ıÔ‡˜ ÂΉ‹ÏˆÛ˘ ·ÈÌ·ÙÔ˘Ú›· ÛÙ· ·È‰È¿ (1,2). O ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙÔ˘ Ca ÛÙ· Ô‡Ú· 24ÒÚÔ˘ Î·È Ù˘ Û¯¤Û˘ Ca ÚÔ˜ ÎÚ·ÙÈÓ›ÓË (UCa/UCr) ÛÂ Ù˘¯·›· ‰Â›ÁÌ·Ù· Ô‡ÚˆÓ, ·ÔÙÂÏ› ·ÍÈfiÈÛÙÔ ‰Â›ÎÙË Ù˘ ·Ô‚ÔÏ‹˜ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú·, Ë ‰Â ÙÈÌ‹ ÙÔ˘ ‰Â›ÎÙË Â›Ó·È ·ÓÂÍ¿ÚÙËÙË ·fi ÙËÓ ËÏÈΛ·. º˘ÛÈÔÏÔÁÈΤ˜ ıˆÚÔ‡ÓÙ·È ÔÈ ÙÈ̤˜, fiÙ·Ó ÙÔ Ca Ô‡ÚˆÓ 24ÒÚÔ˘ Â›Ó·È ÌÈÎÚfiÙÂÚÔ ·fi 4 mg/kg Î·È Ë ÙÈÌ‹ UCa/UCr ÌÈÎÚfiÙÂÚË ·fi 0,25 mg/mg ÛÂ Ù˘¯·›Ô ‰Â›ÁÌ· Ô‡ÚˆÓ ‹ Û ԇڷ 24ÒÚÔ˘ (1,19). ∏ ÁÓÒÛË ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÙÈÌÒÓ ÙÔ˘ ·Ô‚·ÏÏfiÌÂÓÔ˘ ÛÙ· Ô‡Ú· ·Û‚ÂÛÙ›Ô˘, ¤¯ÂÈ ÌÂÁ¿ÏË ÛËÌ·Û›· ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË. O ηıÔÚÈÛÌfi˜, fï˜, Ê˘ÛÈÔÏÔÁÈÎÒÓ ÙÈÌÒÓ Â›Ó·È ÂÍ·ÈÚÂÙÈο ‰‡ÛÎÔÏÔ˜, ÂÍ·ÈÙ›·˜ ΢ڛˆ˜ ÙˆÓ ‰È·ÊÔÚÒÓ Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È ·fi ¯ÒÚ· Û ¯ÒÚ·, ·ÏÏ¿ Î·È ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜. ∆· ÛÙÔȯ›·, ÂÍ¿ÏÏÔ˘, ·fi ÙË ‚È‚ÏÈÔÁÚ·Ê›· Â›Ó·È ÂÚÈÔÚÈṲ̂ӷ. À¿Ú¯Ô˘Ó, ΢ڛˆ˜, ÌÂϤÙ˜ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ÂÓ‹ÏÈΘ, ÂÓÒ fiÛ˜ ·ÊÔÚÔ‡Ó Û ·È‰È¿ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ÔÙÂϤÛÌ·Ù· Ì ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ÙÔ˘˜. ∂ÈϤÔÓ, ÔÈ ÌÂϤÙ˜ ·˘Ù¤˜ ›¯·Ó Î·È Ù· ÁÓˆÛÙ¿ ÌÂÈÔÓÂÎÙ‹Ì·Ù· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔ ÌÈÎÚfi ·ÚÈıÌfi ÙˆÓ ·È‰ÈÒÓ Ô˘ ÌÂÏÂÙ‹ıËηÓ, ÌÂ Û˘Ó¤ÂÈ· ÙËÓ ·‰˘Ó·Ì›· ÌÂϤÙ˘ ÌÂÁ¿ÏˆÓ ÔÌ¿‰ˆÓ ·È‰ÈÒÓ Û οı ËÏÈÎȷ΋ ÔÌ¿‰·, ·ÏÏ¿ Î·È Ì ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ‰ÈÂÍ¿ÁÔÓÙ·Ó Û ·È‰È¿ ÓÔÛËÏ¢fiÌÂÓ· ÁÈ· ¿ÏÏÔ˘˜ ÌÂÓ ÏfiÁÔ˘˜, ÙˆÓ ÔÔ›ˆÓ fï˜ ÙfiÛÔ ÙÔ ‰È·ÈÙÔÏfiÁÈÔ fiÛÔ Î·È Ë Ê˘ÛÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· fiÓÙ·˜ ‰È·ÊÔÚÂÙÈ΋ ·' fiÙÈ ˘fi Ê˘ÛÈÔÏÔÁÈΤ˜ Û˘Óı‹Î˜, ı· ÌÔÚÔ‡Û ›Ûˆ˜ Ó· ÂËÚ¿˙ÂÈ Î·È ÙËÓ ·¤ÎÎÚÈÛË ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ (2-5). ¶·Ú¿ Ù· ˘¿Ú¯ÔÓÙ· ÌÂÈÔÓÂÎÙ‹Ì·Ù·, fï˜, ·fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ÛÙÔȯ›ˆÓ ÙˆÓ ‰È·ÊfiÚˆÓ ÌÂÏÂÙÒÓ ÚÔ·ÙÔ˘Ó ÂӉȷʤÚÔÓÙ· Î·È ¯Ú‹ÛÈÌ· ÛÙÔȯ›·. ŒÙÛÈ, ¤¯Ô˘Ó ·Ú·ÙËÚËı› ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÛÙȘ ÙÈ̤˜ ÙÔ˘ ·ÂÎÎÚÈÓfiÌÂÓÔ˘ ÛÙ· Ô‡Ú· ·Û‚ÂÛÙ›Ô˘, ÙfiÛÔ ·fi ¯ÒÚ· Û ¯ÒÚ·, fiÛÔ Î·È Ì¤Û· ÛÙËÓ ›‰È· ¯ÒÚ·, ·fi ÂÚÈÔ¯‹ Û ÂÚÈÔ¯‹ (6). ∂›Û˘ ‰È·ÊÔÚ¤˜ (·ÚÎÂÙ¤˜ ÊÔÚ¤˜ ·ÓÙÈÊ·ÙÈΤ˜) ¤¯Ô˘Ó ·Ú·ÙËÚËı› Î·È ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ Ô˘ ·Ô‰›‰ÔÓÙ·È ÛÙË ‰È·ÊÔÚÂÙÈ΋ ËÏÈÔÊ¿ÓÂÈ·, ÛÙË ‰È·ÙÚÔÊ‹, ÛÙÔÓ ÎÈÚο‰ÈÔ Ú˘ıÌfi Ù˘ ·Ô‚ÔÏ‹˜ ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ Î.Ï. ŒÙÛÈ, ÂÓÒ ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó ÌÂÁ·Ï‡ÙÂÚË ·¤ÎÎÚÈÛË ·Û‚ÂÛÙ›Ô˘ ηٿ ÙÔ˘˜ ıÂÚÈÓÔ‡˜ Ì‹Ó˜ (7-11), ÙÔ ·ÎÚÈ‚Ò˜ ·ÓÙ›ıÂÙÔ ¤¯ÂÈ
¶·È‰È·ÙÚÈ΋ 2001;64:465-471
Paediatriki 2001;64:465-471
¶›Ó·Î·˜ 3. ∞¤ÎÎÚÈÛË ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· Ê˘ÛÈÔÏÔÁÈÎÒÓ ·È‰ÈÒÓ Û ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜ ÂÔ¯¤˜ ÙÔ˘ ¤ÙÔ˘˜ ηٿ ËÏÈΛ·
∏ÏÈΛ· (¤ÙË)
n
6 7 8 9 10 11
16 49 15 27 25 18
™À¡O§O
150
UCa/UCr Ô‡ÚˆÓ [(mg/dl) / (mg/dl)] (ª¤ÛË ÙÈÌ‹ ± SD) OÎÙÒ‚ÚÈÔ˜ n ∞Ú›ÏÈÔ˜ (ÌÂÁ¿ÏË ÚÔËÁËı›۷ (ÌÂȈ̤ÓË ÚÔËÁËı›۷ ËÏÈÔÊ¿ÓÂÈ·) ËÏÈÔÊ¿ÓÂÈ·) 0,15 ± 0,08 12 0,15 ± 0,1 0,16 ± 0,11 46 0,11 ± 0,1 0,21 ± 0,1 13 0,075 ± 0,05 0,21 ± 0,13 27 0,16 ± 0,13 0,18 ± 0,11 24 0,2 ± 0,17 0,14 ± 0,09 18 0,12 ± 0,1 0,17 ± 0,11
140
0.14 ± 0.12
P
ª™ 0,017 0,002 0,05 ª™ ª™ 0,022
ª™: ÌË ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿
¶›Ó·Î·˜ 4. ¶·È‰È¿ Ì ÙÈÌ‹ Ca/Cr Ô‡ÚˆÓ [(mg/dl) / (mg/dl)] >0,35 ÌÂÙ¿ ÙË ıÂÚÈÓ‹ ÂÚ›Ô‰Ô Ù˘ ÌÂÁ¿Ï˘ ËÏÈÔÊ¿ÓÂÈ·˜ Î·È ·ÔÙÂϤÛÌ·Ù· ·ÓÂϤÁ¯Ô˘ ÌÂÙ¿ ÙË ¯ÂÈÌÂÚÈÓ‹ ÂÚ›Ô‰Ô Ù˘ ÌÂȈ̤Ó˘ ¤ÎıÂÛ˘ ÛÙÔÓ ‹ÏÈÔ º‡ÏÔ ∞ ∞ ∞ ∞ ∫ ∞ ∞ ∫ ∞ ∞ ∫
UCa/UCr [(mg/dl) / (mg/dl)] OÎÙÒ‚ÚÈÔ˜ ∞Ú›ÏÈÔ˜ 0,35 0,26 0,55 0,44 0,48 0,32 0,35 0,42 0,41 0,57 0,37 0,075 0,38 0,18 0,41 0,30 0,44 0,30 0,57 0,07 0,36 -
∞: ∞ÁfiÚÈ, ∫: ∫ÔÚ›ÙÛÈ
‰È·ÈÛÙˆı› Û ÌÂϤÙË, Ë ÔÔ›· ÂÚÈÂÏ¿Ì‚·Ó ÌÈÎÚfi ·ÚÈıÌfi ·È‰ÈÒÓ ÛÙËÓ πÓ‰›· (12). ™Â ÌÂϤÙË Ô˘ ‰ÈÂÍ‹¯ıË ÛÙË ¡fiÙÈ· πÙ·Ï›·, Û ۇÓÔÏÔ 220 ˘ÁÈÒÓ ·È‰ÈÒÓ ËÏÈΛ·˜ 3-16 ÂÙÒÓ, Ë Ì¤ÛË ÙÈÌ‹ ÙÔ˘ ËÏ›ÎÔ˘ UCa/UCr ‹Ù·Ó 0,076±0,060 mg/mg (13). ™˘ÁÎÚ›ÓÔÓÙ·˜ ÙȘ ÙÈ̤˜ ÙÔ˘ ·ÂÎÎÚÈÓfiÌÂÓÔ˘ ÛÙ· Ô‡Ú· ·Û‚ÂÛÙ›Ô˘ ÛÙȘ ·ÓÙ›ÛÙÔȯ˜, Ì ÙË ‰È΋ Ì·˜ ÌÂϤÙË, ÔÌ¿‰Â˜ ËÏÈÎÈÒÓ, Ë Ì¤ÛË ÙÈÌ‹ UCa/UCr ÛÙ· ·È‰È¿ 6-8 ÂÙÒÓ Â›Ó·È 0,072±0,062, ÂÓÒ ÛÙ· ·È‰È¿ ËÏÈΛ·˜ 9-11 ÂÙÒÓ Ë Ì¤ÛË ÙÈÌ‹ UCa/UCr Â›Ó·È 0,063±0,042. ™ËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÌÂٷ͇ ÙˆÓ ‰‡Ô Ê‡ÏˆÓ ˆ˜ ÚÔ˜ ÙËÓ ÔÛfiÙËÙ· ÙÔ˘ ·ÂÎÎÚÈÓfiÌÂÓÔ˘ ÛÙ· Ô‡Ú· ·Û‚ÂÛÙ›Ô˘ ‰ÂÓ ‰È·ÈÛÙÒıËÎÂ, fiˆ˜ ¿ÏψÛÙÂ Î·È ÛÙË ‰È΋ Ì·˜ ÌÂϤÙË, fï˜ ÔÈ ÙÈ̤˜ UCa/UCr ÔÈ Ôԛ˜ ηٷÁÚ¿ÊËÎ·Ó ÛÙË ÌÂϤÙË ·˘Ù‹, ÙfiÛÔ ÛÙÔ Û‡ÓÔÏÔ ÙˆÓ ·È‰ÈÒÓ, fiÛÔ Î·È ·Ó¿ ËÏÈÎȷ΋ ÔÌ¿‰·, Â›Ó·È Û·ÊÒ˜ ÌÈÎÚfiÙÂÚ˜ ·fi ÙȘ ·ÓÙ›ÛÙÔȯ˜ Ù˘ ‰È΋˜ Ì·˜ ÌÂϤÙ˘, ‡ÚËÌ· ÙÔ ÔÔ›Ô Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÚÌËÓ¢ı›, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Ë ¡fiÙÈ· πÙ·Ï›· ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂÁ¿Ï˜ ÔÌÔÈfiÙËÙ˜ Ì ÙË ‰È΋ Ì·˜ ¯ÒÚ·, ÙfiÛÔ ˆ˜ ÚÔ˜
ÙȘ ‰È·ÙÚÔÊÈΤ˜ Û˘Ó‹ıÂȘ, fiÛÔ Î·È ·fi ÏÂ˘Ú¿˜ ÎÏÈÌ·ÙÔÏÔÁÈÎÒÓ Û˘ÓıËÎÒÓ. ™Â ÌÂϤÙË ÙÔ˘ ∆ÛÂÎÂÓÙ˙‹ Î·È Û˘ÓÂÚÁ·ÙÒÓ ÙÔ˘, Û 282 ˘ÁÈ‹ ·È‰È¿ ËÏÈΛ·˜ 3 ¤ˆ˜ 14 ÂÙÒÓ, ‰È·ÈÛÙÒıËΠfiÙÈ ÛÙÔ 96,2% ÙˆÓ ·ÁÔÚÈÒÓ Î·È Û fiÏ· Ù· ÎÔÚ›ÙÛÈ·, Ë Ì¤ÁÈÛÙË ÙÈÌ‹ ÙÔ˘ ËÏ›ÎÔ˘ UCa/UCr ‹Ù·Ó ÌÈÎÚfiÙÂÚË ÙÔ˘ 0,12 (14). ∂Í¿ÏÏÔ˘, Ë Û‡ÁÎÚÈÛË ÙˆÓ ÂηÙÔÛÙÈ·›ˆÓ ı¤ÛÂˆÓ ÙÔ˘ ËÏ›ÎÔ˘ UCa/UCr Ù˘ ‰È΋˜ Ì·˜ ÌÂϤÙ˘ (¶›Ó·Î·˜ 2) Ì ·˘Ù¤˜ ¿ÏÏˆÓ ÌÂÏÂÙÒÓ, ηٷ‰ÂÈÎÓ‡ÂÈ ÔÌÔÈfiÙËÙ˜ Ì ÔÚÈṲ̂Ó˜ Î·È ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ·fi ¿ÏϘ. ŒÙÛÈ, Û ÌÂϤÙË ÙˆÓ Esbjorner E Î·È Jones IL ·fi ÙË ™Ô˘Ë‰›· (15) ‚Ú¤ıËÎ·Ó ÙÈ̤˜ ÌÈÎÚfiÙÂÚ˜ ·fi ÙȘ ‰ÈΤ˜ Ì·˜ ÁÈ· ÙȘ ·ÓÙ›ÛÙÔȯ˜ ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ. ∂Ó‰ÂÈÎÙÈο, Û ·È‰È¿ ËÏÈΛ·˜ 2-6 ÂÙÒÓ, ̤ÛË ÙÈÌ‹ UCa/UCr 0,20±0,17, 50‹ ÂηÙÔÛÙÈ·›· ı¤ÛË (∂£) 0,16, 97Ë ∂£ 0,63 Î·È Û ·È‰È¿ 7-10 ÂÙÒÓ, ËÏÈÎȷ΋ ÔÌ¿‰· Ë ÔÔ›· ÚÔÛÂÁÁ›˙ÂÈ ÂÚÈÛÛfiÙÂÚÔ ÙÔ ‰ÈÎfi Ì·˜ ‰Â›ÁÌ·, ̤ÛË ÙÈÌ‹ 0,15±0,12, 50‹ ∂£ 0,112 Î·È 97Ë ∂£ 0,42. ∆È̤˜ ËÏ›ÎÔ˘ UCa/UCr ·ÚfiÌÔȘ Ì ·˘Ù¤˜ Ù˘ ‰È΋˜ Ì·˜ ÌÂϤÙ˘ ‰›‰ÂÈ, ·fi ÙËÓ ¿ÏÏË ÏÂ˘Ú¿, ÌÂϤÙË ÙÔ˘ Reusz GS Î·È Û˘ÓÂÚÁ·ÙÒÓ ÙÔ˘ ·fi ÙËÓ O˘ÁÁ·Ú›· (16). Ÿˆ˜ ÚԷӷʤÚıËÎÂ, fï˜, ÙÔ ‚·ÛÈÎfi ‡ÚËÌ· Ù˘ ‰È΋˜ Ì·˜ ÌÂϤÙ˘ Â›Ó·È Ë ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚË ·¤ÎÎÚÈÛË ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· ηٿ ÙË Ì¤ÙÚËÛË ÙÔ˘ OÎÙˆ‚Ú›Ô˘ Û ۯ¤ÛË Ì ·˘Ù‹ ÙÔ˘ ∞ÚÈÏ›Ô˘. ∆Ô Â‡ÚËÌ· ·˘Ùfi Û˘ÌʈÓ› Ì ٷ Â˘Ú‹Ì·Ù· ÙˆÓ ÂÚÈÛÛfiÙÂÚˆÓ ÌÂÏÂÙÒÓ Ô˘ ¤ÁÈÓ·Ó Û ÂÓ‹ÏÈΘ. ∂ӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ, ›Û˘, ÙÔ ÁÂÁÔÓfi˜ Ù˘ Û˘Û¯¤ÙÈÛ˘ Ô˘ ‚Ú¤ıËΠÌÂٷ͇ ÙÔ˘ ËÏ›ÎÔ˘ UCa/UCr Î·È Ù˘ ËÏÈÔÊ¿ÓÂÈ·˜, ÂÓÒ ·Ó¿ÏÔÁË Û˘Û¯¤ÙÈÛË ‰ÂÓ Î·Ù¤ÛÙË ‰˘Ó·Ùfi Ó· ÛÙÔȯÂÈÔıÂÙËı› Ì ÙËÓ ÂÚÈÂÎÙÈÎfiÙËÙ· Ù˘ ÙÚÔÊ‹˜ Û ·Û‚¤ÛÙÈÔ ÛÙȘ ‰‡Ô ¯ÚÔÓÈΤ˜ ÂÚÈfi‰Ô˘˜. ∆Ô Â‡ÚËÌ¿ Ì·˜ ·˘Ùfi, ›Û˘ Û˘ÌʈÓ› Ì ٷ ˘ÔÛÙËÚÈ˙fiÌÂÓ· ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·, ηı’ fiÛÔÓ Ë ·˘ÍË̤ÓË ·¤ÎÎÚÈÛË ·Û‚ÂÛÙ›Ô˘ ηٿ Î·È ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙÔ˘˜
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ıÂÚÈÓÔ‡˜ Ì‹Ó˜ ·Ô‰›‰ÂÙ·È ·fi ÙÔ˘˜ ÂÚÈÛÛfiÙÂÚÔ˘˜ ÂÚ¢ÓËÙ¤˜ ÛÙ· ·˘ÍË̤ӷ ›‰· Ù˘ 25-O∏ ‚ÈÙ·Ì›Ó˘ D, Ù· ÔÔ›· ‚Ú›ÛÎÔÓÙ·È Û ¢ı›· ·Ó·ÏÔÁ›· Ì ÙËÓ ·˘ÍË̤ÓË ‰È¿ÚÎÂÈ· Ù˘ ËÏÈÔÊ¿ÓÂÈ·˜ ηٿ ÙË ıÂÚÈÓ‹ ÂÚ›Ô‰Ô (17). ™ÙÔ ÛËÌÂ›Ô ·˘Ùfi Ú¤ÂÈ Ó· Û¯ÔÏÈ·ÛÙ› ÙÔ ÁÂÁÔÓfi˜ fiÙÈ, ·Ú¿ ÙË ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÌÂٷ͇ Ù˘ ̤Û˘ ÙÈÌ‹˜ Ù˘ Û¯¤Û˘ UCa/UCr ÙÔÓ OÎÙÒ‚ÚÈÔ Î·È Ù˘ ·ÓÙ›ÛÙÔȯ˘ ÙÈÌ‹˜ ÙÔ˘ ∞ÚÈÏ›Ô˘, Ë Ú·ÁÌ·ÙÈ΋ ‰È·ÊÔÚ¿ ÙˆÓ ÙÈÌÒÓ (0,17±0,11 ¤Ó·ÓÙÈ 0,14±0,12), ÔÈ Ôԛ˜ ›ӷÈ, Ô‡Ùˆ˜ ‹ ¿Ïψ˜, ·ÌÊfiÙÂÚ˜ Ê˘ÛÈÔÏÔÁÈΤ˜, ÛÙÂÚÂ›Ù·È ‚ÈÔÏÔÁÈ΋˜ ÛËÌ·Û›·˜ Î·È Î·Ù' ¤ÎÙ·ÛË Ú·ÎÙÈÎÔ‡ ÎÏÈÓÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜. øÛÙfiÛÔ, ÙÔ Â‡ÚËÌ· ÂΛÓÔ Ù˘ ÌÂϤÙ˘ Ì·˜ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ÂӉȷʤÚÔÓ, ·ÎÚÈ‚Ò˜ ÂÂȉ‹ ·ÚÔ˘ÛÈ¿˙ÂÈ ÎÏÈÓÈ΋ ÛËÌ·Û›·, Â›Ó·È ÂΛÓÔ ÙÔ ÔÔ›Ô Û˘ÓÔÙÈο ·ÂÈÎÔÓ›˙ÂÙ·È ÛÙÔÓ ¶›Ó·Î· 4, Û‡Ìʈӷ Ì ÙÔ ÔÔ›Ô ÌfiÓÔ 3 ·fi Ù· 11 ·È‰È¿ Ì “˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›·” (UCa/UCr >0,35) ηٿ ÙË Ì¤ÙÚËÛË ÙÔ˘ OÎÙˆ‚Ú›Ô˘, ÂÍ·ÎÔÏÔ˘ıÔ‡Û·Ó Ó· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ıÔÏÔÁÈ΋ ÙÈÌ‹ ÙÔ˘ ËÏ›ÎÔ˘ Î·È Î·Ù¿ ÙË ‰Â‡ÙÂÚË Ì¤ÙÚËÛË ÙÔ˘ ∞ÚÈÏ›Ô˘. £ÂˆÚ‹Û·Ì ÙÔ 0,35 ˆ˜ fiÚÈÔ ‰È¿ÎÚÈÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›· ·fi ÂΛӷ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ·¤ÎÎÚÈÛË ·Û‚ÂÛÙ›Ô˘, Ï·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ ÙȘ ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ Ù˘ ‰È΋˜ Ì·˜ ÌÂϤÙ˘, ıˆÚÒÓÙ·˜ ˆ˜ ·ıÔÏÔÁÈΤ˜ ÙȘ ÙÈ̤˜ Ô˘ ‚Ú›ÛÎÔÓÙ·È ¿Óˆ ·fi ÙËÓ 90‹ ÂηÙÔÛÙÈ·›· ı¤ÛË. ∞Í›˙ÂÈ ÛÙÔ ÛËÌÂ›Ô ·˘Ùfi Ó· ·Ó·ÊÂÚı› fiÙÈ fiÏ· ·˘Ù¿ Ù· ·È‰È¿ (Î·È Ù· 11) ‹Ù·Ó ·Û˘Ìو̷ÙÈο, Ô ‰Â ÂÓ‰Âϯ‹˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ (ÌÂÙ·‚ÔÏÈÎfi˜ Î·È ·ÂÈÎÔÓÈÛÙÈÎfi˜) ¤ÏÂÁ¯Ô˜ ÛÙÔÓ ÔÔ›Ô ˘Ô‚Ï‹ıËηÓ, ·¤‚Ë ·ÚÓËÙÈÎfi˜ ÁÈ· ·ÈÌ·ÙÔ˘Ú›·, ÏÈı›·ÛË ‹ ¿ÏÏÔ ·ıÔÏÔÁÈÎfi ‡ÚËÌ·. ∂Ô̤ӈ˜, Ë ·Ó‡ÚÂÛË ·ıÔÏÔÁÈÎÔ‡ ËÏ›ÎÔ˘ UCa/UCr Î·È ÁÂÓÈο Ë ‰È·›ÛÙˆÛË ·˘ÍË̤Ó˘ ·Ô‚ÔÏ‹˜ ·Û‚ÂÛÙ›Ô˘ ÛÙ· Ô‡Ú· ı· Ú¤ÂÈ Ó· Û˘ÓÂÎÙÈÌ¿Ù·È Ì ·Ú·Ì¤ÙÚÔ˘˜ fiˆ˜ Ë ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ Î·È Ë ËÏÈÔÊ¿ÓÂÈ·. ¶Ú¤ÂÈ ‰Â, Ó· ·ӷϷ̂¿ÓÂÙ·È Î·È Ó· ÂȂ‚·ÈÒÓÂÙ·È ·ÚÎÂÙ¤˜ ÊÔÚ¤˜ ÚÈÓ ıˆÚËı› fiÙÈ ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ¿ÙÔÌÔ ¿Û¯ÂÈ ·fi ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›·, Ì ÙË Û˘Ó·ÎfiÏÔ˘ıË Ù·Ï·ÈˆÚ›· ÂÓfi˜ ÂÎÙÂÓÔ‡˜ ·Ú·ÎÏÈÓÈÎÔ‡ ÂϤÁ¯Ô˘ ηÈ, ‚‚·›ˆ˜, ÚÈÓ ÏËÊıÔ‡Ó Ì¤ÙÚ· ·ÓÙÈÌÂÙÒÈÛ˘ ÙÔ˘ ˘ÔÙÈı¤ÌÂÓÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜. ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ·¤ÎÎÚÈÛË ÙÔ˘ ·Û‚ÂÛÙ›Ô˘ ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜, Ô˘ ÔÊ›ÏÂÙ·È Èı·Ófiٷٷ, ÛÙË ‰È·ÊÔÚÂÙÈ΋ ËÏÈÔÊ¿ÓÂÈ· Î·È ÙË Û˘Ó·ÎfiÏÔ˘ıË ‰È·ÊÔÚ¿ ÛÙËÓ ÂÓ‰ÔÁÂÓ‹ Û‡ÓıÂÛË Ù˘ ‚ÈÙ·Ì›Ó˘ D, Ì fiÛ· ·˘Ùfi Û˘Ó¿ÁÂÙ·È, ‰Â‰Ô̤ÓÔ˘ Ì¿ÏÈÛÙ· fiÙÈ ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ·Ó¿ÌÂÛ· ÛÙÔ ¯ÂÈÌÒÓ· Î·È ÙÔ Î·ÏÔη›ÚÈ, ˆ˜ ÚÔ˜ ÙËÓ ÂÚÈÂÎÙÈÎfiÙËÙ· Ù˘ ÙÚÔÊ‹˜ Û ·Û‚¤ÛÙÈÔ, ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó ÛÙË ÌÂϤÙË Ì·˜. ™˘ÓÂÒ˜, Ë ÁÓÒÛË ÙˆÓ ‰È·Î˘Ì¿ÓÛÂˆÓ ÙˆÓ ÙÈÌÒÓ ÙÔ˘ ·ÂÎÎÚÈÓfiÌÂÓÔ˘ ÛÙ·
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19. §··ÙÛ¿Ó˘ ¶¢. ÀÂÚ·Û‚ÂÛÙÈÔ˘Ú›· ÛÙ· ·È‰È¿. ¢ÂÏÙ ∞’ ¶·È‰È·ÙÚ ∫ÏÈÓ ¶·Ó ∞ıËÓÒÓ 1995;42:117-120.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 17-11-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 08-03-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: °ÂÒÚÁÈÔ˜ ∆ÛfiÏ·˜ ¶¿ÚÓËıÔ˜ 32, 190 13, ™·ÚˆÓ›‰· ∞ÙÙÈ΋˜
¶ÂÚ›ÏË„Ë ¿ÚıÚÔ˘ ·fi ÙË µÈ‚ÏÈÔÁÚ·Ê›· ÀÂÚÔ˘ÚÈÎÔ˙Ô˘Ú›· ÛÙ· ·È‰È¿. ∫ÏÈÓÈ΋ ÚÔ‚ÔÏ‹ Î·È Ê˘ÛÈ΋ ÈÛÙÔÚ›·1 ∂ÈÛ·ÁˆÁ‹: ∏ ȉÈÔ·ı‹˜ ˘ÂÚÔ˘ÚÈÎÔ˙Ô˘Ú›· ¤¯ÂÈ ·Ó·ÊÂÚı› ÛÙÔ ·ÚÂÏıfiÓ Û ÂÚÈÔÚÈṲ̂ÓÔ ·ÚÈıÌfi ·È‰ÈÒÓ Ì ·ÈÌ·ÙÔ˘Ú›·. ™ÎÔfi˜: ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ó· ÂÚÈÁÚ·Ê› Ë ÎÏÈÓÈ΋ ÚÔ‚ÔÏ‹ Î·È Ë Ê˘ÛÈ΋ ÈÛÙÔÚ›· Ù˘ ˘ÂÚÔ˘ÚÈÎÔ˙Ô˘Ú›·˜, fi¯È ÌfiÓÔ Û ·È‰È¿ Ì ·ÈÌ·ÙÔ˘Ú›· ·ÏÏ¿ Î·È Û ÂΛӷ Ì ‰˘ÛÔ˘Ú›· ηÈ/‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ· ¿ÏÁË ÛÙËÓ ÎÔÈÏÈ¿ ‹ ÙÔ˘˜ ÓÂÊÚÔ‡˜ Ì ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi Ô˘ÚÔÏÈı›·Û˘. ÀÏÈÎfi Î·È Ì¤ıÔ‰Ô˜: ŒÁÈÓ ·Ó·‰ÚÔÌÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ÛÙÔȯ›ˆÓ ·fi 102 ÂÚÈÙÒÛÂȘ ·È‰ÈÒÓ Ì ˘ÂÚÔ˘ÚÈÎÔ˙Ô˘Ú›· Î·È Ù˘ ¤Î‚·Û˘ Û 26 ·fi ·˘Ù¿ Ô˘ ·Ú·ÎÔÏÔ˘ı‹ıËÎ·Ó ÁÈ· ‰È¿ÛÙËÌ· ÂÓfi˜ ‹ ÂÚÈÛÛÔÙ¤ÚÔ˘ ¯ÚfiÓÔ˘ (̤ÛÔ˜ fiÚÔ˜ 3,1 ¯ÚfiÓÈ·) ¯ˆÚ›˜ ÂȉÈ΋ ıÂڷ›·. ∞ÔÙÂϤÛÌ·Ù·: ∆· 61 ·È‰È¿ ›¯·Ó ˘ÂÚÔ˘ÚÈÎÔ˙Ô˘Ú›· Î·È Ù· 41 ˘ÂÚÔ˘ÚÈÎÔ˙Ô˘Ú›· Î·È ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›·. ¶ÂÓ‹ÓÙ· ‰‡Ô ·ÛıÂÓ›˜ ‰ÂÓ ·Ó¤ÊÂÚ·Ó ·Ó¿ÌÂÛ· ÛÙ· Û˘ÌÙÒÌ·Ù· ·ÈÌ·ÙÔ˘Ú›· Î·È Û ÂÚÈÛÛfiÙÂÚÔ˘˜ ·fi ÙÔ 1/3 ÙˆÓ ·ÛıÂÓÒÓ Ë ·Ó¿Ï˘ÛË Ô‡ÚˆÓ ÛÙËÓ ÚÒÙË ÂͤٷÛË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ™ÙÔÓ ˘ÂÚ˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô, ÛÙÔ 50% ÙˆÓ ·ÛıÂÓÒÓ ‰È·ÈÛÙÒıËÎ·Ó Ï›ıÔÈ ‰È·Ì¤ÙÚÔ˘ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 3 mm Î·È ÛÙÔ 12% ‰È·Ì¤ÙÚÔ˘ 4-18 mm. ™Â 30 ·fi ÙÔ˘˜ 39 ·ÛıÂÓ›˜ Ô˘ ‰ÂÓ ·ÚÔ˘Û›·˙·Ó ·ÈÌ·ÙÔ˘Ú›· ÛÙËÓ ÚÒÙË ÂͤٷÛË (77%), ‰È·ÈÛÙÒıËÎ·Ó ÛÙÔÓ ˘ÂÚ˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô ÌÈÎÚÔ› ‹ ÌÂÁ¿ÏÔÈ Ï›ıÔÈ. ∏ ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ ÛÙÔ˘˜ ÔÔ›Ô˘˜ ‰È·ÈÛÙÒıËÎÂ Ë ·ÚÔ˘Û›· Ï›ıˆÓ ÌÈÎÚÔ‡ ÌÂÁ¤ıÔ˘˜ ‹Ù·Ó ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚË ·fi ÂΛÓË ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ‰ÂÓ ‰È·ÈÛÙÒıËÎÂ Ë ·ÚÔ˘Û›· Ï›-
ıˆÓ. ™ÙË ‰È¿ÚÎÂÈ· Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘, 4 ·fi ÙÔ˘˜ 26 ·ÛıÂÓ›˜ ‰ÂÓ ·ÚÔ˘Û›·Û·Ó ÔÙ¤ ·ÈÌ·ÙÔ˘Ú›· Î·È 8 ‰ÂÓ ·ÚÔ˘Û›·Û·Ó ·ÈÌ·ÙÔ˘Ú›· ÛÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ‰È¿ÛÙËÌ· Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘. ¢‡Ô ·ÛıÂÓ›˜ Ô˘ ·ÚÔ˘Û›·Û·Ó Ï›ıÔ˘˜ ·fi ÙËÓ ·Ú¯‹ Î·È ÙÚÂȘ ÛÙÔ˘˜ ÔÔ›Ô˘˜ Û¯ËÌ·Ù›ÛÙËÎ·Ó Ï›ıÔÈ ‰È·Ì¤ÙÚÔ˘ 4-12 mm, ÙÔ˘˜ ·¤‚·Ï·Ó 1-3,5 ¯ÚfiÓÈ· ·ÚÁfiÙÂÚ· ÛÙ· Ô‡Ú·. ™˘ÌÂÚ¿ÛÌ·Ù·: ∏ ·Ô˘Û›· ·ÈÌ·ÙÔ˘Ú›·˜ ‰ÂÓ ·ÔÙÂÏ› ‰Â›ÎÙË ·Ô˘Û›·˜ Ô˘ÚÔÏÈı›·Û˘, ÁÈ’ ·˘Ùfi Ë ÂÍ·Ê¿ÓÈÛË ÌfiÓÔ Ù˘ ·ÈÌ·ÙÔ˘Ú›·˜ ·ÔÙÂÏ› Ï¿ıÔ˜ ÎÚÈÙ‹ÚÈÔ ÁÈ· ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÂÊ·ÚÌÔ˙fiÌÂÓ˘ ıÂڷ›·˜. ∏ ˘fiÓÔÈ· ˘ÂÚÔ˘ÚÈÎÔ˙Ô˘Ú›·˜ Î·È ˘ÂÚ·Û‚ÂÛÙÈÔ˘Ú›·˜ Ú¤ÂÈ Ó· Ù›ıÂÙ·È Û ·È‰È¿ Ì ‰˘ÛÔ˘Ú›· ηÈ/‹ ˘ÔÙÚÔÈ¿˙ÔÓÙ· ¿ÏÁË ÛÙËÓ ÎÔÈÏÈ¿ ‹ ÙÔ˘˜ ÓÂÊÚÔ‡˜ Ì ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi Ô˘ÚÔÏÈı›·Û˘, ·ÎfiÌË Î·È ¯ˆÚ›˜ ÙËÓ ·ÚÔ˘Û›· ·ÈÌ·ÙÔ˘Ú›·˜.
1 La ªanna A, Polito C, MarÙe A, Iovene A, Di Toro R Hyperuricosuria in children. Clinical presentation and natural history Pediatrics 2001;107:86-90
∆ÛÔ˘Ì¿Î·˜ ∫ˆÓ/ÓÔ˜ ¶·È‰›·ÙÚÔ˜ - ¢È¢ı˘ÓÙ‹˜ ∂™À ¡ÔÛÔÎÔÌÂ›Ô ∫∞∆ - ∞ÙÙÈ΋˜
471
¶·È‰È·ÙÚÈ΋ 2001;64:472-480
∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞
Paediatriki 2001;64:472-480
ORIGINAL ARTICLE
∏ ÂÌÂÈÚ›· ·fi ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ÓÂÊÚÈ΋˜ ·Ó¿ÚÎÂÈ·˜ Û ·È‰È¿ ÛÙË ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £Âڷ›·˜ ¡. ∑¿‚Ú·˜1, ∞. ∫·fiÁÈ·ÓÓ˘2, ¶. ∫·Ï·Ì·Ï›Î˘1, °. ªÚÈ·ÛÔ‡Ï˘1, ∂. °ÂˆÚÁ¿ÎË2, ∞. ÷Ù˙‹˜1
Management of children with acute renal failure in a pediatric intensive care unit N. Zavras1, A. Kapogiannis2, P. Kalambalikis1, G. Briasoulis1, E. Georgaki2, A. Hatzis1
¶ÂÚ›ÏË„Ë: ™ÎÔfi˜ Ù˘ ÂÚÁ·Û›·˜ ·˘Ù‹˜ ‹Ù·Ó Ë Î·Ù·ÁÚ·Ê‹ Ù˘ ÂÌÂÈÚ›·˜ ·fi ÙË Û˘ÁÎÚÈÙÈ΋ ÌÂϤÙË ÙˆÓ ÌÂıfi‰ˆÓ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ÔÍ›· ÓÂÊÚÈ΋ ·Ó¿ÚÎÂÈ· (O¡∞) ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘˜ ÛÙË ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £Âڷ›·˜ (ª∂£). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÌÈ·˜ ÙÂÙÚ·ÂÙ›·˜, 25/1359 ·ÛıÂÓ›˜ (1,84%), ̤Û˘ ËÏÈΛ·˜ 5,56±1,13 ¤ÙË, ÓÔÛËχıËÎ·Ó ÛÙË ª∂£ Ì O¡∞ Ô˘ ÔÊÂÈÏfiÙ·Ó Â›Ù ÛÙÔ Û‡Ó‰ÚÔÌÔ ÔÏ˘ÔÚÁ·ÓÈ΋˜ ·Ó¿ÚÎÂÈ·˜ [17/25, (68%)] ‹ Û ¿ÏÏ· ·›ÙÈ· [8/25 (32%)]. °È· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÔÈ ·ÎfiÏÔ˘ı˜ ̤ıÔ‰ÔÈ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘: Û 7 ·fi ÙÔ˘˜ 25 ·ÛıÂÓ›˜ (28%) ÂÊ·ÚÌfiÛıËΠÔÍ›· ÂÚÈÙÔÓ·˚΋ οı·ÚÛË (¶∫), Û 16/25 (64%) Û˘Ó¯‹˜ ·ÈÌԉȋıËÛË (™∞¢) Î·È Û 2/25 (8%) ·ÈÌÔοı·ÚÛË (∞∫). ∞fi Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜ ÚÔ·ÙÂÈ fiÙÈ Î·È Ì ÙȘ ÙÚÂȘ ÌÂıfi‰Ô˘˜ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó ·ÔÙÂÏÂÛÌ·ÙÈο ÔÈ ‰È·Ù·Ú·¯¤˜ Ù˘ ÓÂÊÚÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È ÔÈ ‰È·Ù·Ú·¯¤˜ Ù˘ ÔÍÂÔ‚·ÛÈ΋˜ ÈÛÔÚÚÔ›·˜ ÙˆÓ ·ÛıÂÓÒÓ Ì·˜. ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ Â›Ù¢ÍË ·ÚÓËÙÈÎÔ‡ ÈÛÔ˙˘Á›Ô˘ ˘ÁÚÒÓ, ·fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Ì·˜ Ê·›ÓÂÙ·È fiÙÈ Ë ™∞¢ ˘ÂÚÙÂÚ› Ù˘ ¶∫ (p<0,005). ∆Ô ÔÛÔÛÙfi ÂÈ‚›ˆÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì O¡∞ ÛÙ· Ï·›ÛÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Ù˘ ÔÏ˘ÔÚÁ·ÓÈ΋˜ ·Ó¿ÚÎÂÈ·˜ ‹Ù·Ó ¯·ÌËÏfi (20%), ÂÓÒ fiÏ· Ù· ·È‰È¿ Ì O¡∞ ¿ÏÏ˘ ·ÈÙÈÔÏÔÁ›·˜ ¤˙ËÛ·Ó. ™˘ÌÂÚ·ÛÌ·ÙÈο, Î·È ÔÈ ÙÚÂȘ ̤ıÔ‰ÔÈ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ˘ÔηıÈÛÙÔ‡Ó ÈηÓÔÔÈËÙÈο ÙË ÓÂÊÚÈ΋ ÏÂÈÙÔ˘ÚÁ›·. OÈ ‰È·ÊÔÚ¤˜ Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙËÓ ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ Ê·›ÓÂÙ·È Ó· ÔÊ›ÏÔÓÙ·È ÛÙË ‚·Ú‡ÙËÙ· Ù˘ ˘ÔΛÌÂÓ˘ ÓfiÛÔ˘ Î·È fi¯È ÛÙË ¯ÚËÛÈÌÔÔÈÔ‡ÌÂÓË Ù¯ÓÈ΋.
Abstract: ∆he aim of this study was to report our experience based on the comparative study of the various modalities of renal replacement therapy (RRT) used for the management of patients with acute renal failure (ARF), during their hospitalization in our Pediatric Intensive Care Unit (PICU). During a 4-year period from October 1996 to November 2000, 25/1359 patients (1,84%), with a mean age 6,24±1,2 yrs, were admitted to the PICU with ARF, that occurred either as part of multiple organ system failure (MOSF) [17/25, (68%)] or due to other causes (8/25, 32%). Of these patients 7/25 (28%) were treated with acute peritoneal dialysis (PD), 16/25 (64%) with continuous hemofiltration (CHF) and 2/25 (8%) with hemodialysis (HD). Our results showed that acute renal failure and acid-base disturbances were successfully corrected with RRT. Nevertheless, negative fluid balance was better achieved by CHF, compared to PD (p<0,005). Survival of children with ARF and MOSF was low (20%), while all children with ARF, due to other causes, survived. We conclude, that children with ARF could be treated effectively with RRT. The outcome of these patients is mainly dependent on the severity of the underlying disease and not to the mode of RRT used.
1 MÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £Âڷ›·˜, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· 2 ¡ÂÊÚÔÏÔÁÈÎfi ∆Ì‹Ì·, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”
1 Intensive Care Unit “Agia Sophia” Children’s Hospital, Athens 2 Nephrology Department, “Agia Sophia” Children’s Hospital
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Paediatriki 2001;64:472-480
§¤ÍÂȘ ÎÏÂȉȿ: ÔÍ›· ÓÂÊÚÈ΋ ·Ó¿ÚÎÂÈ·, Â͈ÓÂÊÚÈ΋ οı·ÚÛË, ÂÚÈÙÔÓ·˚΋ οı·ÚÛË, ·ÈÌԉȋıËÛË, ·ÈÌÔοı·ÚÛË.
Key words: acute renal failure, renal replacement therapy, peritoneal dialysis, hemofiltration, hemodialysis.
∂ÈÛ·ÁˆÁ‹ H ÔÍ›· ÓÂÊÚÈ΋ ·Ó¿ÚÎÂÈ· (O¡∞) ÛÙË ‚ÚÂÊÈ΋ Î·È ·È‰È΋ ËÏÈΛ· ÔÊ›ÏÂÙ·È Û ‰È¿ÊÔÚ· ·›ÙÈ· (1,2) Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È Û˘Ó‹ıˆ˜ ·fi ÌÂȈ̤ÓË ·Ú·ÁˆÁ‹ Ô‡ÚˆÓ (<1 ml/kg/h) Î·È ·Ú¿ÏÏËÏË ·‡ÍËÛË Ù˘ Ô˘Ú›·˜ Î·È Ù˘ ÎÚ·ÙÈÓ›Ó˘, ηıÒ˜ Î·È ¿ÏÏˆÓ ÌÂÙ·‚ÔÏÈÎÒÓ ÚÔ˚fiÓÙˆÓ ÛÙÔ ·›Ì· (3,4). ∂ÓÙÔ‡ÙÔȘ, ÔÈ ÂÚÈÙÒÛÂȘ O¡∞ ÛÙȘ Ôԛ˜ Ë ·Ú·ÁˆÁ‹ Ô‡ÚˆÓ ·Ú·Ì¤ÓÂÈ Ê˘ÛÈÔÏÔÁÈ΋ ‹ Î·È ·˘ÍË̤ÓË (ÔÏ˘Ô˘ÚÈÎÔ‡ Ù‡Ô˘ O¡∞) Ì ·Ú¿ÏÏËÏË ‰È·Ù·Ú·¯‹ ÛÙËÓ ·¤ÎÎÚÈÛË ÙˆÓ ÚÔ˚fiÓÙˆÓ ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙÔ˘ ·˙ÒÙÔ˘, ‰ÂÓ Â›Ó·È Û¿ÓȘ ÛÙËÓ ·È‰È·ÙÚÈ΋ Ú¿ÍË. OÈ ·ÛıÂÓ›˜ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó O¡∞ ÏfiÁˆ ÂÓ‰ÔÁÂÓÔ‡˜ ·ÚÂÁ¯˘Ì·ÙÈ΋˜ ‚Ï¿‚˘ ¤¯Ô˘Ó ηٿ ηÓfiÓ· ηϋ ¤Î‚·ÛË, Ì ÔÛÔÛÙfi ıÓËÛÈÌfiÙËÙ·˜ οو ·fi 10% (5,6). ∞ÓÙ›ıÂÙ·, fiÙ·Ó Ë O¡∞ ÔÊ›ÏÂÙ·È Û ‰È·Ù·Ú·¯¤˜ Ù˘ ·ÈÌ¿ÙˆÛ˘ Î·È Û˘Ó˘¿Ú¯ÂÈ ÔÏ˘ÔÚÁ·ÓÈ΋ ·Ó¿ÚÎÂÈ·, ÙÔ ÔÛÔÛÙfi ıÓËÛÈÌfiÙËÙ·˜ Î˘Ì·›ÓÂÙ·È ·fi 60-100% (7). ∞ÛıÂÓ›˜ Ì O¡∞ Ô˘ ÔÊ›ÏÂÙ·È Û ÔÏ˘ÔÚÁ·ÓÈ΋ ·Ó¿ÚÎÂÈ·, ¤¯Ô˘Ó ·˘ÍË̤Ó˜ ·Ó¿ÁΘ Û ˘ÁÚ¿, ÔÈ Ôԛ˜ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙËÓ Î¿Ï˘„Ë ÙˆÓ ıÂÚÌȉÈÎÒÓ ·Ó·ÁÎÒÓ Î·È ÙËÓ ·˘ÍË̤ÓË Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹. ¶·Ú¿ÏÏËÏ·, ÂÍ·ÈÙ›·˜ Ù˘ ÌÂȈ̤Ó˘ ·ÂÎÎÚÈÙÈ΋˜ ÈηÓfiÙËÙ·˜ ÙˆÓ ÓÂÊÚÒÓ, ÌÂÈÒÓÂÙ·È Ë ·Ô‚ÔÏ‹ ÙÔÍÈÎÒÓ ÌÂÙ·‚ÔÏÈÙÒÓ. °È· ÙÔ˘˜ ÏfiÁÔ˘˜ ·˘ÙÔ‡˜, Ì ÙËÓ ¤ÁηÈÚË ¤Ó·ÚÍË Ù˘ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘ Â›Ó·È ÂÊÈÎÙ‹ Ë ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·ÔÌ¿ÎÚ˘ÓÛË ˘ÁÚÒÓ Î·È Ë ÈηÓÔÔÈËÙÈ΋ ıÂÚÌȉÈ΋ Î¿Ï˘„Ë (8). OÈ Â͈ÓÂÊÚÈΤ˜ ̤ıÔ‰ÔÈ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Â›Ó·È Ë ÂÚÈÙÔÓ·˚΋ οı·ÚÛË (¶∫), Ë Û˘Ó¯‹˜ ·ÈÌԉȋıËÛË (™∞¢) Î·È Ë ·ÈÌÔοı·ÚÛË (∞∫). ∏ ·ÈÌÔοı·ÚÛË (∞∫) ¯ÚËÛÈÌÔÔÈ‹ıËΠ·Ú¯Èο ˆ˜ ·ÚÈÔ Ì¤ÛÔ ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘ Û ·È‰È¿ Ì O¡∞ (5). ∏ ·ÈÌÔ‰˘Ó·ÌÈ΋ ·ÛÙ¿ıÂÈ·, fï˜, Ô˘ ·Ú·ÙËÚÂ›Ù·È Û ·È‰È¿ Ô˘ ÓÔÛËχÔÓÙ·È Û ª∂£ ¤¯ÂÈ ÂÚÈÔÚ›ÛÂÈ ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ (9). ∏ ÂÚÈÙÔÓ·˚΋ οı·ÚÛË (¶∫) ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı›, ›Û˘, ηٿ ÙÔ ·ÚÂÏıfiÓ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· Ì ÈηÓÔÔÈËÙÈο ·ÔÙÂϤÛÌ·Ù· (10,11). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Û ÔÏϤ˜ ÌÂϤÙ˜, ÙÔ ÂӉȷʤÚÔÓ ¤¯ÂÈ ÂÈÎÂÓÙÚˆı› ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ O¡∞ Û ÓÂÔÁÓ¿, ‚Ú¤ÊË Î·È ·È‰È¿ Ì ÙË Û˘Ó¯‹ ·ÈÌԉȋıËÛË (™∞¢) (12-15). ∏ ̤ıÔ‰Ô˜ ·˘Ù‹ Á›ÓÂÙ·È Î·Ï‡ÙÂÚ· ·ÓÂÎÙ‹ ÛÙÔ˘˜ ·ÈÌÔ‰˘Ó·ÌÈο ·ÛÙ·ı›˜ ·ÚÚÒÛÙÔ˘˜, ÂÓÒ ·Ú¿ÏÏËÏ· ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ·Ê·›ÚÂÛ˘ ÛËÌ·ÓÙÈÎÔ‡ fiÁÎÔ˘ ˘ÁÚÒÓ (16). ™ÙË ÌÂϤÙË ·˘Ù‹ ÂÚÈÁÚ¿ÊÔ˘Ì ÙËÓ ÂÌÂÈÚ›·
Ì·˜ ·fi ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ O¡∞ Ì ÙȘ ·Ú·¿Óˆ ÌÂıfi‰Ô˘˜ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∫·Ù¿ ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· OÎÙÒ‚ÚÈÔ˜ 1996 - ¡Ô¤Ì‚ÚÈÔ˜ 2000, ÓÔÛËχıËÎ·Ó 1359 ·ÛıÂÓ›˜ ÛÙË ª∂£ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”. TÚÈ¿ÓÙ· ·fi ·˘ÙÔ‡˜ (2,2%), ·ÚÔ˘Û›·Û·Ó O¡∞, Ë ÔÔ›· ·ÓÙÈÌÂÙˆ›ÛÙËΠ̛̠· ·fi ÙȘ ÌÂıfi‰Ô˘˜ Â͈ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘: ÔÍ›· ÂÚÈÙÔÓ·˚΋ οı·ÚÛË (¶∫) Û 8 ·È‰È¿, Û˘Ó¯‹˜ ·ÈÌԉȋıËÛË (™∞¢) Û 20 Î·È ‰È·ÏÂ›Ô˘Û· ·ÈÌÔοı·ÚÛË (∞∫) Û 2 ·È‰È¿. ∞ÛıÂÓ›˜ Ô˘ ¤˙ËÛ·Ó ÏÈÁfiÙÂÚÔ ·fi 24 ÒÚ˜ ‰ÂÓ Û˘ÌÂÚÈÂÏ‹ÊıËÛ·Ó ÛÙË ÌÂϤÙË Ì·˜. ∏ ÂÈÏÔÁ‹ Ù˘ ÌÂıfi‰Ô˘ ¤ÁÈÓ Û ۯ¤ÛË Ì ÙËÓ ËÏÈΛ· ÙÔ˘ ·ÛıÂÓÔ‡˜ Î·È ÙË ‚·Ú‡ÙËÙ· Ù˘ ÁÂÓÈ΋˜ ÙÔ˘ ηٿÛÙ·Û˘. ∏ ̤ÛË ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ˘Ô‚Ï‹ıËÎ·Ó Û ¶∫ ‹Ù·Ó 1,8 ¤ÙË (‡ÚÔ˜: 48 Ë̤Ú˜ ¤ˆ˜ 7 ¤ÙË) Î·È Û ™∞¢ 7,8 ¤ÙË (‡ÚÔ˜: 1,1 ¤ˆ˜ 17 ¤ÙË). ∆· 2 ·È‰È¿ Ô˘ ˘Ô‚Ï‹ıËÎ·Ó Û ∞∫ ›¯·Ó ÙËÓ ›‰È· ËÏÈΛ· (11 ¤ÙË) (¶›Ó·Î·˜ 1). ∞fi Ù· 30 ·È‰È¿, ·ÔÎÏ›ÛÙËÎ·Ó 5 ·fi ÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË. ¶ÈÔ Û˘ÁÎÂÎÚÈ̤ӷ, ¤Ó·˜ ·ÛıÂÓ‹˜ ‹Ù·Ó Û ¯ÚfiÓÈ· ¶∫ Î·È ÓÔÛËχÙËΠÛÙË ª∂£ ÁÈ· <48 ÒÚ˜ ÏfiÁˆ ·ÙÂÏÔ‡˜ ÂÈÏÂÔ‡, ÂÓÒ 4 ·È‰È¿ Ì ™∞¢ ¤˙ËÛ·Ó <24 ÒÚ˜. ™Ù· ˘fiÏÔÈ· 25 ·È‰È¿, Ù· ·›ÙÈ· Ô˘ ÚÔοÏÂÛ·Ó ÙËÓ O¡∞ Ù·ÍÈÓÔÌ‹ıËÎ·Ó Û ‰‡Ô oÌ¿‰Â˜. ∏ ÚÒÙË ÔÌ¿‰· ÂÚÈÂÏ¿Ì‚·Ó 17 ·È‰È¿ (68%), ÛÙ· ÔÔ›· ÙÔ ·›ÙÈÔ Ù˘ O¡∞ ‹Ù·Ó ÙÔ Û‡Ó‰ÚÔÌÔ ÔÏ˘ÔÚÁ·ÓÈ΋˜ ·Ó¿ÚÎÂÈ·˜ Î·È Ë ‰Â‡ÙÂÚË ÔÌ¿‰· ÂÚÈÂÏ¿Ì‚·Ó 8 ·È‰È¿ (32%), ÛÙ· ÔÔ›· ¿ÏÏ· ·›ÙÈ· ‹Ù·Ó ˘Â‡ı˘Ó· ÁÈ· ÙËÓ O¡∞ (¶›Ó·Î·˜ 2). OÚÈÛÌÔ› ø˜ O¡∞ ÔÚ›ÛÙËÎÂ Ë ÂÌÊ¿ÓÈÛË ÔÏÈÁÔ˘Ú›·˜ (·Ú·ÁˆÁ‹ Ô‡ÚˆÓ <1 ml/kg/h) ÁÈ· ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÌÂÁ·Ï‡ÙÂÚÔ ÙˆÓ 4 ˆÚÒÓ, ·Ú¿ ÙËÓ Â·Ú΋ ÂÓ˘‰¿ÙˆÛË Î·È ÙË ¯ÔÚ‹ÁËÛË ÓÙÔ·Ì›Ó˘ ‹/Î·È ÊÔ˘ÚÔÛÂÌ›‰Ë˜ Î·È Ë ·fiÙÔÌË ·‡ÍËÛË ÙˆÓ ÙÈÌÒÓ Ù˘ Ô˘Ú›·˜ Î·È ÎÚ·ÙÈÓ›Ó˘ ·›Ì·ÙÔ˜ (17). ø˜ ÛËÙÈ΋ ηٷÏËÍ›· ÔÚ›ÛÙËÎÂ Ë ·ÚÔ˘Û›· Û‹„˘ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÒÛË Ù˘ ·ÚÙËÚȷ΋˜ ›ÂÛ˘, ·Ú¿ ÙËÓ Â·Ú΋ ¯ÔÚ‹ÁËÛË ˘ÁÚÒÓ Î·È Û˘Ó‡·ÚÍË ‰È·Ù·Ú·¯ÒÓ ·ÈÌ¿ÙˆÛ˘, ÔÈ Ôԛ˜ ÌÔÚ› Ó· ÂΉËÏÒÓÔÓÙ·Ó Ì Á·Ï·ÎÙÈ΋ ÔͤˆÛË, ÔÏÈÁÔ˘Ú›· Î·È ‰È·Ù·Ú·¯‹ ÙÔ˘ ÂȤ‰Ô˘ Û˘Ó›‰ËÛ˘ (18). ø˜ Û‡Ó‰ÚÔÌÔ ÔÏ˘ÔÚÁ·ÓÈ΋˜ ·Ó¿ÚÎÂÈ·˜ (™¶A) ÔÚ›ÛÙËÎÂ Ë ·Ó¿ÚÎÂÈ· 2 ÙÔ˘Ï¿¯ÈÛÙÔÓ Û˘ÛÙËÌ¿ÙˆÓ (19). ∏ ‚·Ú‡ÙËÙ· Ù˘ ÓfiÛÔ˘ Û οı ·È‰› ÂÎÙÈÌ‹ıËΠ̠ÙËÓ
¶›Ó·Î·˜ 1. ∫ÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ·ÛıÂÓÒÓ Ô˘ ˘Ô‚Ï‹ıËÎ·Ó Û Â͈ÓÂÊÚÈ΋ οı·ÚÛË (n: 25) ∂›‰Ô˜
AÚÈıÌfi˜ ·ÛıÂÓÒÓ
∏ÏÈΛ· Û ¤ÙË (̤Û˱sem)
PRISM
™¶∞ (·Ú. ·ÛıÂÓÒÓ)
¶∫ ™∞¢ ∞∫
7 (28%) 16 (64 %) 2 (8%)
1,70±0,82 6,79±1,57 11
23,5±1,5 26,2±2,4 11,7±2,5
3 16 -
473
¶·È‰È·ÙÚÈ΋ 2001;64:472-480
Paediatriki 2001;64:472-480
¶›Ó·Î·˜ 2. ∞›ÙÈ· O¡∞ ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ÓÔÛËχıËÎ·Ó ÛÙË ª∂£ ∞›ÙÈÔ ∞) ¶ÔÏ˘ÔÚÁ·ÓÈ΋ ·Ó¿ÚÎÂÈ· - ÛËÙÈ΋ ηٷÏËÍ›· - Ë·ÙÔÓÂÊÚÈÎfi Û‡Ó‰ÚÔÌÔ - Ú·‚‰ÔÌ˘fiÏ˘ÛË B) ÕÏÏ· ·›ÙÈ· - ÂÓ‰ÔÁÂÓ‹˜ ÓÂÊÚÈ΋ ‚Ï¿‚Ë (˘ÔÏ·ÛÙÈÎÔ›-‰˘ÛÏ·ÛÙÈÎÔ› ÓÂÊÚÔ›, Ô˘Ú·ÈÌÈÎfi-·ÈÌÔÏ˘ÙÈÎfi Û‡Ó‰ÚÔÌÔ, Û˘ÁÁÂÓ‹˜ ÛÙ¤ÓˆÛË ÓÂÊÚÈÎÒÓ ·ÚÙ.Û‡Ó‰ÚÔÌÔ Williams, ÂÛÙȷ΋ ÛÂÈÚ·Ì·ÙÔÛÎÏ‹Ú˘ÓÛË) - ÙÔÍÈ΋ ›‰Ú·ÛË Ê·ÚÌ¿ÎˆÓ ™‡ÓÔÏÔ
∞ÛıÂÓ›˜
%
15 1 1
60 4 4
5
20
3
12
25
100
Îϛ̷η ¶·È‰È·ÙÚÈÎÔ‡ ∫ÈÓ‰‡ÓÔ˘ £ÓËÛÈÌfiÙËÙ·˜ (Pediatric Risk of Mortality - PRISM score) (20). ∏ ¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜ ÂÎÙÈÌ‹ıËΠ̠ÎÚÈÙ‹ÚÈÔ ÙËÓ ·ÔηٿÛÙ·ÛË ÙÔ˘ ÈÛÔ˙˘Á›Ô˘ ˘ÁÚÒÓ, ÙË Ì›ˆÛË ÙˆÓ ÂȤ‰ˆÓ Ô˘Ú›·˜ Î·È ÎÚ·ÙÈÓ›Ó˘ ÛÙÔ ·›Ì·, ηıÒ˜ Î·È ÙË ‰ÈfiÚıˆÛË ÙˆÓ ÌÂÙ·‚ÔÏÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ. ¢Â›ÁÌ·Ù· ·›Ì·ÙÔ˜ Ï·Ì‚¿ÓÔÓÙ·Ó ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘ Î·È ÛÙË Û˘Ó¤¯ÂÈ· οı 12 ÒÚ˜. ∞Ú¯¤˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È ÂÚÈÁÚ·Ê‹ ÙˆÓ Ù¯ÓÈÎÒÓ ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘ ŸÏ˜ ÔÈ Ù¯ÓÈΤ˜ ÛÙËÚ›˙ÔÓÙ·È ÛÙËÓ Î¿ı·ÚÛË ÙÔ˘ ‡‰·ÙÔ˜ ̤ۈ ËÌȉȷÂÚ·Ù‹˜ ÌÂÌ‚Ú¿Ó˘ Î·È ÂÓfi˜ Â͈ۈ̷ÙÈÎÔ‡ ΢ÎÏÒÌ·ÙÔ˜. ∏ ‰È·‰Èηۛ· ·˘Ù‹ ηÏÂ›Ù·È ˘ÂډȋıËÛË (ultrafiltration) Î·È Â›Ó·È ›‰È· Ì ÙË ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÓÂÊÚÈÎÔ‡ ÛÂÈÚ¿Ì·ÙÔ˜. ™ÙËÓ ¶∫, ÙÔ ›‰ÈÔ ÙÔ ÂÚÈÙfiÓ·ÈÔ ·ÔÙÂÏ› ÙË Ê˘ÛÈ΋ ÌÂÌ‚Ú¿ÓË ·ÓÙ·ÏÏ·Á‹˜ ÓÂÚÔ‡, ÂÓÒ ÛÙËÓ ÂÚ›ÙˆÛË Ù˘ ™∞¢ Î·È Ù˘ ∞∫ ¯ÚËÛÈÌÔÔÈÂ›Ù·È Ù¯ÓËÙ‹ Â͈ۈ̷ÙÈ΋ ÌÂÌ‚Ú¿ÓË. °È· ÙË ‰ËÌÈÔ˘ÚÁ›· ÙÔ˘ ˘ÂÚ‰ÈËı‹Ì·ÙÔ˜ ··ÈÙÂ›Ù·È Ë ·Ó¿Ù˘ÍË Ô‰ËÁÔ‡ ›ÂÛ˘ ‰È‹ıËÛ˘ (‰È·ÌÂÌ‚Ú·ÓÈ΋ ›ÂÛË). ™ÙË ™∞º∞, Ë Ô‰ËÁfi˜ ›ÂÛË ÂÈÙ˘Á¯¿ÓÂÙ·È ·fi ÙËÓ Î·Ú‰È·Î‹ ·ÓÙÏ›·. ™ÙË ™ºº∞ Î·È ÛÙËÓ ∞∫, Ë Ô‰ËÁfi˜ ›ÂÛË ÂÍ·ÛÊ·Ï›˙ÂÙ·È Ì ÙË ‚Ô‹ıÂÈ· ·ÓÙÏ›·˜. ∆¤ÏÔ˜, ÛÙËÓ ¶∫, Ë ·Ê·›ÚÂÛË ˘ÁÚÒÓ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ˆÛ̈ÙÈÎfiÙËÙ· ÙÔ˘ ‰È·Ï‡Ì·ÙÔ˜ οı·ÚÛ˘. ™Ù· ÂÚÈÙÔÓ·˚ο ‰È·Ï‡Ì·Ù·, Ë ˆÛ̈ÙÈο ‰Ú·ÛÙÈ΋ Ô˘Û›· Â›Ó·È Ë ÁÏ˘Îfi˙Ë. ∞‡ÍËÛË Ù˘ Û˘ÁΤÓÙÚˆÛ˘ ۷ί¿ÚÔ˘ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ê·›ÚÂÛË ÌÂÁ·Ï‡ÙÂÚÔ˘ fiÁÎÔ˘ ˘ÁÚÒÓ. ∏ οı·ÚÛË ÙˆÓ ‰È·Ï˘Ì¤ÓˆÓ Ô˘ÛÈÒÓ ·fi ÙȘ ËÌȉȷÂÚ·Ù¤˜ ÌÂÌ‚Ú¿Ó˜ ÙÔ˘ Ê›ÏÙÚÔ˘ Á›ÓÂÙ·È Ì ÙË ‰È·‰Èηۛ· Ù˘ ‰È¿¯˘Û˘ (diffusion) Î·È Û ÌÈÎÚfiÙÂÚÔ ‚·ıÌfi Ì ÌÂÙ·ÊÔÚ¿ (convection) ÙˆÓ Ô˘ÛÈÒÓ, ηıÒ˜ Û˘Ì·Ú·Û‡ÚÔÓÙ·È ·fi ÙÔ ÓÂÚfi Ì ÙË ‰È·‰Èηۛ· Ù˘ ˘ÂډȋıËÛ˘. O Ú˘ıÌfi˜ ·Ô‚ÔÏ‹˜ ÙˆÓ Ô˘ÛÈÒÓ ÂÍ·ÚÙ¿Ù·È ·fi ÙË ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜, ÙË ‰È¿ÚÎÂÈ· Ù˘ οı·ÚÛ˘, ÙÔ Ì¤ÁÂıÔ˜ ÙˆÓ fiÚˆÓ Ù˘ ÌÂÌ‚Ú¿Ó˘ ηÈ, Ù¤ÏÔ˜, ·fi ÙË ‰È·ÊÔÚ¿ Û˘ÁΤÓÙÚˆÛ˘ ÙˆÓ ‰È·Ï˘Ì¤ÓˆÓ Ô˘ÛÈÒÓ (21). ™ÙËÓ ÂÚ›ÙˆÛË Ù˘ ¶∫, Ë ·ÔÌ¿ÎÚ˘ÓÛË ÙˆÓ ‰È·Ï˘Ì¤ÓˆÓ Ô˘ÛÈÒÓ Ì¤Ûˆ ÙÔ˘ ÂÚÈÙÔÓ·›Ô˘ ÂÍ·ÚÙ¿Ù·È ·fi ÙÔÓ fiÁÎÔ ÙÔ˘ ÂÚÈÙÔÓ·˚ÎÔ‡ ‰È·Ï‡Ì·ÙÔ˜ Î·È ÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ·ÏÏ·ÁÒÓ. ·) ¶ÂÚÈÙÔÓ·˚΋ οı·ÚÛË: ∏ ¶∫ ÁÈÓfiÙ·Ó ÌÂÙ¿ ·fi ÙÔ-
474
Ôı¤ÙËÛË ÂÓfi˜ ηıÂÙ‹Ú· Tenckhoff Ì ‰ÈÏfi ‰·ÎÙ‡ÏÈÔ (cuff), ›Ù ÛÙÔ ¯ÂÈÚÔ˘ÚÁÂ›Ô Â›Ù ÛÙË ª∂£ (∂ÈÎfiÓ· 1). ∏ ¤Ó·ÚÍË Ù˘ ¶∫ ÁÈÓfiÙ·Ó Ì ÌÈÎÚÔ‡˜ fiÁÎÔ˘˜ ÙÔ ÚÒÙÔ 24ˆÚÔ (10-15 ml/kg/·Ó¿ ·ÎÏÔ) Ô˘ ÚÔԉ¢ÙÈο ·‡Í·Ó·Ó ̤¯ÚÈ 40 ml/kg/·Ó¿ ·ÎÏÔ, ·Ó ÁÈÓfiÙ·Ó Î·Ï¿ ·ÓÂÎÙfi. To ÂÚÈÙÔÓ·˚Îfi ‰È¿Ï˘Ì· ·ÔÙÂÏÔ‡ÓÙ·Ó ·fi ÁÏ˘Îfi˙Ë: 1,5%, 2,5% ‹ 4,25% Î·È ËÏÂÎÙÚÔχÙ˜ (¡a+: 136 mEq/l, Ca++: 3,5 mEq/l, Mg++: 0,76 mEq/l Î·È Á·Ï·ÎÙÈÎfi Ô͇). ∫·ÏÏȤÚÁÂȘ ·fi ÙÔ ˘ÂډȋıËÌ· Ï·Ì‚¿ÓÔÓÙ·Ó Î·ıËÌÂÚÈÓ¿. ‚) ™˘Ó¯‹˜ ·ÈÌԉȋıËÛË: ∏ ™∞¢ ÁÈÓfiÙ·Ó Â›Ù ˆ˜ Û˘Ó¯‹˜ ·ÚÙËÚÈÔÊÏ‚È΋ ·ÈÌԉȋıËÛË (™∞º∞) (∂ÈÎfiÓ· 2), ›Ù ˆ˜ Û˘Ó¯‹˜ ÊÏ‚Ô-ÊÏ‚È΋ ·ÈÌԉȋıËÛË (™ºº∞) Ì ÙË ‚Ô‹ıÂÈ· ÂÚÈÛÙÚÔÊÈ΋˜ ·ÓÙÏ›·˜ (CHERRY CHF-VER 10,ERGO) (∂ÈÎfiÓ· 3). ∏ ÂÊ·ÚÌÔÁ‹ ÁÈÓfiÙ·Ó ÌÂÙ¿ ·fi ηıÂÙËÚÈ·ÛÌfi ÙˆÓ ÌËÚÈ·›ˆÓ ·ÁÁ›ˆÓ Ì ÙË Ì¤ıÔ‰Ô Seldinger. H ™∞º∞ ÂÊ·ÚÌÔ˙fiÙ·Ó Û ·ÛıÂÓ›˜ Ì ¿ÚÎÂÈ· Ù˘ ηډȷ΋˜ ·ÓÙÏ›·˜, ÂÓÒ ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ·ÈÌÔ‰˘Ó·ÌÈ΋ ·ÛÙ¿ıÂÈ· ÂÊ·ÚÌÔ˙fiÙ·Ó Ë ™ºº∞. ∆Ô Û‡ÛÙËÌ· Ù˘ Â͈ۈ̷ÙÈ΋˜ ΢ÎÏÔÊÔÚ›·˜ Î·È ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ ·ÔÙÂÏÔ‡ÓÙ·Ó ·fi ÙÔ Ê›ÏÙÚÔ ÔÏ˘ÛÔ˘ÏÊfiÓ˘ (Diafilter, D20-NR, D30-NR) Ì ÂÈÊ¿ÓÂÈ· 0,3-0,66 m2. ∏ ÂÈÏÔÁ‹ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ›¯Â Û¯¤ÛË Ì ÙËÓ ËÏÈΛ· ÙÔ˘ ·ÛıÂÓÔ‡˜. ∆Ô Ì‹ÎÔ˜ Ù˘ ·ÚÙËÚȷ΋˜ Î·È ÊÏ‚È΋˜ ÁÚ·ÌÌ‹˜ ‹Ù·Ó 30 cm Î·È 40 cm ·ÓÙ›ÛÙÔȯ·. °È· ÙËÓ ·ÔÊ˘Á‹ ‰ËÌÈÔ˘ÚÁ›·˜ ıÚfiÌ‚ˆÓ ÛÙÔ Â͈ۈ̷ÙÈÎfi ·Îψ̷, ÁÈÓfiÙ·Ó ¯ÔÚ‹ÁËÛË Ë·Ú›Ó˘ ÛÂ Û˘Ó¯‹ ÛÙ¿Á‰ËÓ ¤Á¯˘ÛË ·fi ÙËÓ ÚÔ¤ÎÙ·ÛË Ù˘ ·ÚÙËÚȷ΋˜ ÁÚ·ÌÌ‹˜ ÙÔ˘ ΢ÎÏÒÌ·ÙÔ˜ Î·È ÛÙȘ ‰‡Ô ÌÂıfi‰Ô˘˜ ·ÈÌԉȋıËÛ˘, Ì ڢıÌfi 5-10 IU/kg/h. ™Ùfi¯Ô˜ ‹Ù·Ó Ë ‰È·Ù‹ÚËÛË ÙÔ˘ ¯ÚfiÓÔ˘ ÌÂÚÈ΋˜ ıÚÔÌ‚ÔÏ·ÛÙ›Ó˘ (∞PTT) ÛÙÔ Â͈ۈ̷ÙÈÎfi ·Îψ̷ ÛÙ· 90 sec. ™ÙȘ ÂÚÈÙÒÛÂȘ Ô˘ Ë ·Ô‚ÔÏ‹ ÙˆÓ Ô˘Ú·ÈÌÈÎÒÓ ÙÔÍÈÓÒÓ ‰ÂÓ ‹Ù·Ó ÈηÓÔÔÈËÙÈ΋ ÌÂÙ¿ ·fi ·Ú¤Ï¢ÛË 24 ˆÚÒÓ Û˘Ó¯ԇ˜ ·ÈÌԉȋıËÛ˘, Ë ™∞¢ (·ÚÙËÚÈÔÊÏ‚È΋ ‹ ÊÏ‚ÔÊÏ‚È΋) ÌÂÙ·ÙÚÂfiÙ·Ó ÛÂ Û˘Ó¯‹ ·ÈÌԉȷ‰È‹ıËÛË (™∞¢¢). ªÂ ÙË Ì¤ıÔ‰Ô ·˘Ù‹, ‰È¿Ï˘Ì· Ì ۇÛÙ·ÛË ¡a+: 140 mEq/l, HCO3-:40 mEq/l ,Cl-:100 mEq/l (‰È¿Ï˘Ì· D6 Bieffe Medital, BELCO) ‰ÈÔ¯ÂÙ¢fiÙ·Ó ÛÙÔ ÌË ·ÈÌ·ÙÈÎfi ¯ÒÚÔ ÙÔ˘ Ê›ÏÙÚÔ˘, Û ·ÓÙ›ıÂÙË ÚÔ‹ ·fi ·˘Ù‹ ÙÔ˘ ·›Ì·ÙÔ˜. ∏ ™∞¢¢ ·ÔÙÂÏ› ·Ú·ÏÏ·Á‹ Ù˘ ™∞¢ Î·È Û˘Ó‰˘¿˙ÂÈ ÙË ‰È¿¯˘ÛË Î·È ÙË ÌÂÙ·ÊÔÚ¿, Ì ÛÎÔfi ÙËÓ ·‡ÍËÛË Ù˘ οı·ÚÛ˘ ÙˆÓ Ô˘Ú·ÈÌÈÎÒÓ ÙÔÍÈÓÒÓ (22). H ·ÔηٿÛÙ·ÛË ÙˆÓ ·ˆÏÂÈÒÓ ÁÈÓfiÙ·Ó Ì ‰È¿Ï˘Ì· ˘ÔηٿÛÙ·Û˘ ·ÈÌÔ‰ÈËı‹Ì·ÙÔ˜: Na+: 135 mmol/l, K+: 2
∂͈Ù. ÙÌ‹Ì· ÙÔ˘ ηıÂÙ‹Ú·
∂ȉÂÚÌ›‰· ÀÔ‰fiÚÈÔ˜ ‰·ÎÙ‡ÏÈÔ˜ ∫ÔÈÏÈ·Îfi ÙÔ›¯ˆÌ· ∆ÔȯˆÌ·ÙÈÎfi ÂÚ.
∂Ó Ùˆ ‚¿ıÂÈ ‰·ÎÙ‡ÏÈÔ˜
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∂ÓÙÂÚÈΤ˜ ¤ÏÈΘ ∂ÈÎfiÓ· 1. ™¯ËÌ·ÙÈ΋ ·Ú¿ÛÙ·ÛË Ù˘ ÙÔÔı¤ÙËÛ˘ ÂÓ‰ÔÂÚÈÙÔÓ·˚ÎÔ‡ ηıÂÙ‹Ú· Tenckhoff.
¶·È‰È·ÙÚÈ΋ 2001;64:472-480
Paediatriki 2001;64:472-480
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∂ÈÎfiÓ· 3. ™¯ËÌ·ÙÈ΋ ·Ú¿ÛÙ·ÛË Ù˘ Û˘Ó¯ԇ˜ ÊÏ‚Ô-ÊÏ‚È΋˜ ·ÈÌԉȋıËÛ˘.
mmol/l, Ca++: 1,87 mmol/l, Mg++: 0,75 mmol/l, Cl-: 108,5 mmol/l, Á·Ï·ÎÙÈÎfi Ô͇: 33,75 mmol/l, osmolality: 290 mosm/l (Hemofiltration solution 2, Laboratorium Dr. G. Bichel AG 3800 Interlaken Switzerland) Û ÔÛfiÙËÙ· ·Ó¿ÏÔÁË Ì ÙȘ ˘‰ÚÈΤ˜ ·Ó¿ÁΘ ÙÔ˘ ·ÛıÂÓÔ‡˜. °) ∞ÈÌÔοı·ÚÛË: °È· ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ ∞∫ ÁÈÓfiÙ·Ó Î·ıÂÙËÚÈ·ÛÌfi˜ ÙˆÓ ÌÂÁ¿ÏˆÓ ·ÁÁ›ˆÓ Ì ÙËÓ ›‰È· ̤ıÔ‰Ô ÁÈ· ÙËÓ ÂÊ·ÚÌÔÁ‹ ™∞º∞ ‹ ™ºº∞. °È· ÙËÓ ·ÈÌÔοı·ÚÛË ¯ÚËÛÈÌÔÔÈ›ÙÔ ÙÔ Ì˯¿ÓËÌ· AK-10 Ì ‰ÈÙÙ·ÓıÚ·ÎÈο (GABRO CO) Î·È ÙÔ Ì¤ÁÂıÔ˜ ÙÔ˘ Ê›ÏÙÚÔ˘ (GFE-12,GABRO) ‹Ù·Ó 0,81,3 m2, ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· ÙÔ˘ ·ÛıÂÓÔ‡˜ Î·È ÙËÓ ÂÈÊ¿ÓÂÈ· ÙÔ˘ ÛÒÌ·Ùfi˜ ÙÔ˘. ™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ∞fi ÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ÂÍ·ÈÚ¤Û·Ì ÙÔ˘˜ ·ÛıÂÓ›˜ Ô˘ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Ì ∞∫, ÏfiÁˆ ÙÔ˘ ÌÈÎÚÔ‡ ‰Â›ÁÌ·ÙÔ˜ (2 ·ÛıÂÓ›˜). T· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ˆ˜ ̤ÛË ÙÈÌ‹±sem (ÛÙ·ıÂÚfi ÛÊ¿ÏÌ·). OÈ ÚÔÁÓˆÛÙÈÎÔ› ‰Â›ÎÙ˜ ‚·Ú‡ÙËÙ·˜ Û˘ÁÎÚ›ıËÎ·Ó Û ۯ¤ÛË Ì ÙËÓ ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ, ηıÒ˜ Î·È Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ ÌÂıfi‰ˆÓ fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ·ÔηٿÛÙ·ÛË ÙˆÓ ‰È·Ù·Ú·¯ÒÓ Ù˘ ÔÍÂÔ‚·ÛÈ΋˜ ÈÛÔÚÚÔ›·˜ Î·È ÙˆÓ ÙÈÌÒÓ Ô˘Ú›·˜/ÎÚ·ÙÈÓ›Ó˘, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙËÓ ÔÏ˘·Ú·ÁÔÓÙÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ÔÏÏ·ÏÒÓ ÌÂÙÚ‹ÛˆÓ. ∆¤ÏÔ˜, Ë Û‡ÁÎÚÈÛË ¶∫/™∞¢ ˆ˜ ÚÔ˜ ÙËÓ Â›Ù¢ÍË ·ÚÓËÙÈÎÔ‡ ÈÛÔ˙˘Á›Ô˘ ˘ÁÚÒÓ ¤ÁÈÓ Ì ÙË ‰ÔÎÈÌ·Û›· t ‰‡Ô ηÙ¢ı‡ÓÛÂˆÓ (two tailed-t-test). ∆È̤˜ Èı·ÓfiÙËÙ·˜ <0,05 ıˆڋıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜. OÈ ·Ó·Ï‡ÛÂȘ Ú·ÁÌ·ÙÔÔÈ‹ıËÎ·Ó Ì ÙË ‚Ô‹ıÂÈ· ÙÔ˘ ™Ù·ÙÈÛÙÈÎÔ‡ ¶ÚÔÁÚ¿ÌÌ·ÙÔ˜ ÁÈ· ÙȘ ∫ÔÈÓˆÓÈΤ˜ ∂ÈÛً̘ (SPSS) ÁÈ· Windows, ¤Î‰ÔÛË 8.
∞ÔÙÂϤÛÌ·Ù· ∞fi ÙÔ˘˜ 25 ·ÛıÂÓ›˜ Ì O¡∞, ‚Ú¤ıËΠfiÙÈ Â›¯·Ó ·ÓÔ˘Ú›· ÔÈ 23 (92%), Ô˘Ú·ÈÌ›· ÔÈ 24 (96%), ·‡ÍËÛË ÎÚ·ÙÈÓ›Ó˘ ÔÈ 25 (100%), Ó¢ÌÔÓÈÎfi Ô›‰ËÌ· ÔÈ 12 (48%) Î·È ˘ÂÚηÏÈ·ÈÌ›· ÔÈ 3 (12%). ∏ ¶∫ ÂÊ·ÚÌfiÛÙËΠ۠7 ·ÛıÂÓ›˜ (28%), Ë ™∞¢ Û 16 (64%), ÂÓÒ Ë ∞∫ Û 2 (8%). ™Â 3 ·ÛıÂÓ›˜ Ô˘ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Ì ™∞¢, ÂÊ·ÚÌfiÛÙËΠÂÈϤÔÓ ·ÈÌԉȷ‰È‹ıËÛË ÁÈ· ÌÂÁ·Ï‡ÙÂÚË ·Ô‚ÔÏ‹ Ô˘Ú·ÈÌÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ∏ ̤ÛË ‰È¿ÚÎÂÈ· ÂÊ·ÚÌÔÁ‹˜ Ù˘ ¶∫ ‹Ù·Ó 24,4±1 Ë̤Ú˜, Ù˘ ™∞¢ 7,31±2,8 Ë̤Ú˜, ÂÓÒ Ù˘ ∞∫ 2,2±0,54 Ë̤Ú˜. ™ÙÔÓ ¶›Ó·Î· 3 Ê·›ÓÂÙ·È Ë Ì¤ÛË ‰È¿ÚÎÂÈ· ÁÈ· οı ̤ıÔ‰Ô ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Î·È Ë ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ ˆ˜ ÚÔ˜ ÙËÓ ·ÔηٿÛÙ·ÛË Ù˘ ÓÂÊÚÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È ÙË ıÓËÛÈÌfiÙËÙ·. ∏ Ì›ˆÛË ÙˆÓ ÙÈÌÒÓ Ô˘Ú›·˜ Î·È ÎÚ·ÙÈÓ›Ó˘ Ì ÙË Û˘ÌÏ‹ÚˆÛË 48 ˆÚÒÓ ·fi ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ οı ÌÂıfi‰Ô˘ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋, fiˆ˜ Ê·›ÓÂÙ·È ·fi Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜. ∆Ô ›‰ÈÔ ÈÛ¯‡ÂÈ Î·È ÁÈ· ÙËÓ ·ÔηٿÛÙ·ÛË Ù˘ ÔÍÂÔ‚·ÛÈ΋˜ ÈÛÔÚÚÔ›·˜ (¶›Ó·Î·˜ 4). ™˘ÁÎÚ›ÓÔÓÙ·˜, fï˜, ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ ‰‡Ô ÌÂıfi‰ˆÓ (™∞¢/¶∫) ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜. ™ÙȘ ∂ÈÎfiÓ˜ 4 Î·È 5 Ê·›ÓÂÙ·È Ë ‰È·Î‡Ì·ÓÛË ÙˆÓ ÙÈÌÒÓ Ô˘Ú›·˜ Î·È ÎÚ·ÙÈÓ›Ó˘ ÔÚÔ‡ Ì ÙȘ 2 ÌÂıfi‰Ô˘˜ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘. ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ·Ô‚ÔÏ‹ Ù˘ Ô˘Ú›·˜ Î·È Ù˘ ÎÚ·ÙÈÓ›Ó˘, Ê¿ÓËΠfiÙÈ ÂÈÙ˘Á¯¿ÓÂÙ·È ÌÂÁ·Ï‡ÙÂÚË ·ÔÌ¿ÎÚ˘ÓÛË ÙˆÓ Ô˘Ú·ÈÌÈÎÒÓ ÙÔÍÈÓÒÓ Ì ™∞¢¢, Û˘ÁÎÚÈÙÈο Ì ÙË ™∞¢ Î·È Ë ‰È·ÊÔÚ¿ ·˘Ù‹ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ (p<0,05). ™˘ÁÎÂÎÚÈ̤ӷ, ÔÈ ÙÈ̤˜ Ô˘Ú›·˜ Î·È ÎÚ·ÙÈÓ›Ó˘ Û ·È‰È¿ Ù· ÔÔ›· ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó ·ÔÎÏÂÈÛÙÈο Ì ™∞¢, ‹Ù·Ó ·ÓÙ›ÛÙÔȯ· 135±2 mg/dl Î·È 1,8±0,58 mg/dl (ÚÒÙÔ 24ˆÚÔ) Î·È ÌÂÈÒıËÎ·Ó Û 104,8±18 mg/dl Î·È 1,5±0,3 mg/dl (‰Â‡ÙÂÚÔ 24ˆÚÔ). OÈ ·ÓÙ›ÛÙÔȯ˜ ÙÈ̤˜ ÙˆÓ ·È‰ÈÒÓ Ô˘ ˘Ô‚Ï‹ıËÎ·Ó Î·È Û ™∞¢¢ ÛÙÔ ‰Â‡ÙÂÚÔ 24ˆÚÔ ‹Ù·Ó ÁÈ· ÙËÓ Ô˘Ú›· 140±5,69 mg/dl (ÚÒÙÔ 24ˆÚÔ) Î·È 86,3±6 mg/dl (‰Â‡ÙÂÚÔ 24ˆÚÔ) Î·È ÁÈ· ÙËÓ ÎÚ·ÙÈÓ›ÓË 2,7±0,5 mg/dl (ÚÒÙÔ 24ˆÚÔ) Î·È 1,6±0,2 mg/dl (‰Â‡ÙÂÚÔ 24ˆÚÔ). §·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ¶∫ ÂÈÙ˘Á¯¿ÓÂÈ ÙÔ Ì¤ÁÈÛÙÔ Ù˘ ·fi‰ÔÛ˘ ÛÙ·‰È·Î¿, Û˘ÁÎÚ›Ó·Ì ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ¿ Ù˘ Û ۯ¤ÛË Ì ÙËÓ ™∞¢/™∞¢¢ ·Ó·ÊÔÚÈο Ì ÙËÓ ·Ô‚ÔÏ‹ ˘ÁÚÒÓ Ì ÙË Û˘ÌÏ‹ÚˆÛË 48 ˆÚÒÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘. ∞ÚÓËÙÈÎfi ÈÛÔ˙‡ÁÈÔ ÂÈÙ‡¯ıËΠ۠22 ·ÛıÂÓ›˜ (96%), ÂÓÒ ıÂÙÈÎfi ÈÛÔ˙‡ÁÈÔ ·Ú·ÙËÚ‹ıËΠ۠1 ·ÛıÂÓ‹ (4%) Ì ¶∫, ÏfiÁˆ ·Ó¿Ù˘Í˘ ÂÚÈÙÔÓ›Ùȉ·˜. ∞fi ÙË Û‡ÁÎÚÈÛË ÙˆÓ ‰‡Ô Ù¯ÓÈÎÒÓ ÚÔ·ÙÂÈ
475
¶·È‰È·ÙÚÈ΋ 2001;64:472-480
Paediatriki 2001;64:472-480
¶›Ó·Î·˜ 3. ¢È¿ÚÎÂÈ· Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘ Î·È ¤Î‚·ÛË ·ÛıÂÓÒÓ
¶∫ ™∞¢ / ™∞¢¢ ∞∫
∞ÚÈıÌfi˜ ·ÛıÂÓÒÓ
¢È¿ÚÎÂÈ· (̤Û˱sem)
ÂÈ‚›ˆÛË
ŒÎ‚·ÛË Ã¡∞
ı¿Ó·ÙÔ˜
7 15 2
24,40±1,0 Ë̤Ú˜ 7,31±2,8 Ë̤Ú˜ 2,20±0,54 Ë̤Ú˜
3 3 2
2 1
4 (57,1%) 13 (81,2%) -
á∞: ÃÚfiÓÈ· ¡ÂÊÚÈ΋ ∞Ó¿ÚÎÂÈ·
fiÙÈ Ë ™∞¢ ˘ÂÚÙÂÚ› Ù˘ ¶∫ ˆ˜ ÚÔ˜ ÙËÓ ·Ê·›ÚÂÛË ˘ÁÚÒÓ (p<0,005) (∂ÈÎfiÓ· 6). ∂ÈÏÔΤ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ™∞¢ ·ÚÔ˘ÛÈ¿ÛÙËÎ·Ó Û 3 ·È‰È¿ (18,7%). ∏ ÚÒÙË ÂÚ›ÙˆÛË ·ÊÔÚÔ‡Û ıÚfiÌ‚ˆÛË Ù˘ ÌËÚÈ·›·˜ ·ÚÙËÚ›·˜, Ë ÔÔ›· ·ÓÙÈÌÂÙˆ›ÛÙËΠ̠ıÚÔÌ‚ÔÏ˘ÙÈ΋ ·ÁˆÁ‹ Î·È ÙÚÔÔÔ›ËÛË Ù˘ ™∞º∞ Û ™ºº∞, ÂÓÒ ÔÈ ¿ÏϘ ‰‡Ô ·ÊÔÚÔ‡Û·Ó ·ÔÈÎÈÛÌfi ÙˆÓ ·ÁÁ›ˆÓ Ì ·ıÔÁfiÓÔ˘˜ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜ Ô˘ Ô‰‹ÁËÛ Û ·ÓÙÈηٿÛÙ·ÛË Ù˘ ™∞¢ Ì ¶∫. ∞fi Ù· ·È‰È¿ Ô˘ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Ì ¶∫, 3 (33,3%) ·ÚÔ˘Û›·Û·Ó ÂÈÏÔΤ˜. OÈ 2 ÂÚÈÙÒÛÂȘ ·ÊÔÚÔ‡Û·Ó ÂÚÈÙÔÓ›Ùȉ· Î·È Ë 1 ˘‰ÚÔıÒڷη. OÈ ÂÚÈÙÒÛÂȘ Ì ÂÚÈÙÔÓ›Ùȉ· ·ÓÙÈÌÂÙˆ›ÛÙËηÓ, Ë Ì›· ÌÂ Û˘ÛÙËÌ·ÙÈ΋ Î·È ÂÓ‰ÔÂÚÈÙÔÓ·˚΋ ¯ÔÚ‹ÁËÛË ·ÓÙÈ‚ÈÔÙÈÎÒÓ Î·È Ë ¿ÏÏË Ì ·Ê·›ÚÂÛË ÙÔ˘ ÂÚÈÙÔÓ·˚ÎÔ‡ ηıÂÙ‹Ú· Î·È ÂÊ·ÚÌÔÁ‹ ™∞¢, ÂÓÒ Ô ˘‰ÚÔıÒڷη˜ ·ÓÙÈÌÂÙˆ›ÛÙËΠ̠ÚÔÛˆÚÈÓ‹ ‰È·ÎÔ‹ Ù˘ ¶∫ Î·È Û˘Ó¤¯ÈÛË Ù˘ ÌÂıfi‰Ô˘ Ì ÌÈÎÚfiÙÂÚÔ˘˜ fiÁÎÔ˘˜. ™Ù· 2 ·È‰È¿ Û ∞∫ ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÂÈÏÔΤ˜. Afi ÙÔ Û‡ÓÔÏÔ ÙˆÓ 25 ·È‰ÈÒÓ Û ıÂڷ›· ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘, Ù· 16 (64%) ·Â‚›ˆÛ·Ó. H ·ÚÈ· ·ÈÙ›· ı·Ó¿ÙÔ˘ ‹Ù·Ó Ë ÔÏ˘ÔÚÁ·ÓÈ΋ ·Ó¿ÚÎÂÈ· ÏfiÁˆ ÛËÙÈ΋˜ ηٷÏËÍ›·˜. ∂›Û˘, ·Ú·ÙËÚ‹ıËΠÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÌÂٷ͇ ÙˆÓ ·È‰ÈÒÓ Ô˘ ÂÈ‚›ˆÛ·Ó Î·È ·˘ÙÒÓ Ô˘ ¤ı·Ó·Ó fiÛÔÓ ·ÊÔÚ¿ ÛÙÔ PRISM score (12±2 Û ۇÁÎÚÈÛË Ì 24±4,2, p<0,001) Î·È ÙÔÓ ·ÚÈıÌfi ÙˆÓ ·Ó·ÚÎÔ‡ÓÙˆÓ ÔÚÁ·ÓÈÎÒÓ Û˘ÛÙËÌ¿ÙˆÓ (·Ó·ÚÎÔ‡ÓÙ· Û˘ÛÙ‹Ì·Ù· 2 Û ۇÁÎÚÈÛË Ì 4, p<0,001). ™˘˙‹ÙËÛË ∆· ·›ÙÈ· O¡∞ Û ·È‰È¿ Ô˘ ÓÔÛËχÔÓÙ·È ÛÙË ª∂£ Â›Ó·È ÔÈΛϷ. ∏ ÛËÙÈ΋ ηٷÏËÍ›· ·ÔÙÂÏ› ÙÔ Û˘¯ÓfiÙÂÚÔ ·fi ·˘Ù¿, ÂÓÒ ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÊÔÚ¤˜ Û˘Ó˘¿Ú¯ÂÈ Ì ÔÏ˘ÔÚÁ·ÓÈ΋ ·Ó¿ÚÎÂÈ·. ™ÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜ Î·È Â› ·ÔÙ˘¯›·˜ ÙˆÓ Û˘ÓÙËÚËÙÈÎÒÓ Ì¤ÛˆÓ, ÂÈ‚¿ÏÏÂÙ·È Ë ¤ÁηÈÚË ¤Ó·ÚÍË Â͈ÓÂÊÚÈÎÒÓ ÌÂıfi‰ˆÓ ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘. ™ÙËÓ ÂÈÏÔÁ‹ Ù˘ ÌÂıfi‰Ô˘ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ‰È·‰Ú·Ì·Ù›˙ÂÈ Ë ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ: Û ‚Ú¤ÊË Î·È Ôχ ÌÈÎÚ¿ ·È‰È¿, fiÔ˘ Ë ·ÁÁÂȷ΋ ÚÔۤϷÛË Â›Ó·È ÔÏϤ˜ ÊÔÚ¤˜ ·‰‡Ó·ÙË, ·ÏÏ¿ Î·È Ô Î›Ó‰˘ÓÔ˜
476
¶›Ó·Î·˜ 4. ∞fi‰ÔÛË ÌÂıfi‰ˆÓ ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘ (̤ÛË ± sem) ™∞¢
¶∫
131,56±7,8 83,25±6,9 (p<0,001)
124,57±15,02 97,10±12,63 (p<0,001)
2,16±0,15 1,40±0,17 (p<0,001)
2,97±0,92 2,39±0,73 (p<0,001)
¢ÈÙÙ·ÓıÚ·ÎÈο (mEq/l) 0 ÒÚ˜ 14,65±0,3 12 ÒÚ˜ 21,20±0,4 (p<0,005)
16,40±1,5 21,89±0,32 (p<0,005)
O˘Ú›· (mg/dl) 0 ÒÚ˜ 48 ÒÚ˜ ∫Ú·ÙÈÓ›ÓË (mg/dl) 0 ÒÚ˜ 48 ÒÚ˜
ph 0 ÒÚ˜ 12 ÒÚ˜
7,21±0,8 7,38±0,14 (p<0,005)
7,27±0,2 7,40±0,1 (p<0,05)
0 ÒÚ˜ 6 ÒÚ˜
7,20±0,8 4,40±0,45 (p<0,05)
6,40±1,67 4,70±0,45 (p<0,05)
K
ÂÈÏÔÎÒÓ ·fi ÙÔÓ ÙÚ·˘Ì·ÙÈÛÌfi ÙˆÓ ·ÁÁ›ˆÓ ηٿ ÙÔÓ Î·ıÂÙËÚÈ·ÛÌfi Â›Ó·È ÌÂÁ·Ï‡ÙÂÚÔ˜ ÂÊ·ÚÌfi˙ÂÙ·È Î˘Ú›ˆ˜ Ë ¶∫, ÂÓÒ Û ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ ÂÊ·ÚÌfi˙ÔÓÙ·È ÔÈ ‰‡Ô ¿ÏϘ ̤ıÔ‰ÔÈ (23-25). ∏ ¶∫, ›Û˘, ·ÔÙÂÏ› ÙË Ì¤ıÔ‰Ô ÂÎÏÔÁ‹˜ Û ·È‰È¿ Ì ·˘ÍË̤ÓË Èı·ÓfiÙËÙ· Ó· ·Ó·Ù‡ÍÔ˘Ó ÙÂÏÈÎfi ÛÙ¿‰ÈÔ ÓÂÊÚÈ΋˜ ·Ó¿ÚÎÂÈ·˜ ÏfiÁˆ ÂÓ‰ÔÁÂÓÔ‡˜ ÓÂÊÚÈ΋˜ ‚Ï¿‚˘ (23). ™ÙË ÌÂϤÙË Ì·˜, Ù· ·È‰È¿ Ì ÂÓ‰ÔÁÂÓ‹ ÓÂÊÚÈ΋ ‚Ï¿‚Ë ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Ì ¶∫. Ÿˆ˜ Â›Ó·È ÁÓˆÛÙfi, Ë O¡∞ ¤¯ÂÈ ˆ˜ Û˘Ó¤ÂÈ· ÙËÓ ·‡ÍËÛË Ù˘ Ô˘Ú›·˜ Î·È Ù˘ ÎÚ·ÙÈÓ›Ó˘. ™·Ú¿ÓÙ· ÔÎÙÒ ÒÚ˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘ (¶∫ ‹ ™∞¢) ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ·Ú·ÙËÚ‹ıËΠÙÒÛË ÙˆÓ ÂȤ‰ˆÓ Ô˘Ú›·˜ Î·È ÎÚ·ÙÈÓ›Ó˘ Û ·Ô‰ÂÎÙ¿ ›‰· (26). ∞Ó Î·È Ë ÚÒÙË ÂÓÙ‡ˆÛË ‹Ù·Ó fiÙÈ Ë ™∞¢ ˘ÂÚÙÂÚÔ‡Û Ù˘ ¶∫ fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ·ÔÌ¿ÎÚ˘ÓÛË Ô˘Ú·ÈÌÈÎÒÓ ÙÔÍÈÓÒÓ, ÂÓÙÔ‡ÙÔȘ, ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ÙˆÓ ‰‡Ô
¶·È‰È·ÙÚÈ΋ 2001;64:472-480
Paediatriki 2001;64:472-480
3
™∞¢ ¶∫ 100
50
0
24
48
flÚ˜
∫Ú·ÙÈÓ›ÓË (mg/dl)
O˘Ú›· (mg/dl)
150
2,5
™∞¢ ¶∫
2
1,5
1
0
24
48
flÚ˜
∂ÈÎfiÓ· 4. ¢È·Î‡Ì·ÓÛË ÙˆÓ ÙÈÌÒÓ Ù˘ Ô˘Ú›·˜ Ì ÙËÓ ÂÊ·ÚÌÔÁ‹ ÂÚÈÙÔÓ·˚΋˜ οı·ÚÛ˘ Î·È Ù˘ Û˘Ó¯ԇ˜ ·ÈÌԉȋıËÛ˘.
∂ÈÎfiÓ· 5. ¢È·Î‡Ì·ÓÛË ÙˆÓ ÙÈÌÒÓ Ù˘ ÎÚ·ÙÈÓ›Ó˘ Ì ÙËÓ ÂÊ·ÚÌÔÁ‹ ÂÚÈÙÔÓ·˚΋˜ οı·ÚÛ˘ Î·È Ù˘ Û˘Ó¯ԇ˜ ·ÈÌԉȋıËÛ˘.
ÌÂıfi‰ˆÓ. ™Ù· ›‰È· Û˘ÌÂÚ¿ÛÌ·Ù· ηٷϋÁÔ˘Ó Î·È ¿ÏϘ ÌÂϤÙ˜ (17). ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ·Ô‚ÔÏ‹ Ù˘ ÎÚ·ÙÈÓ›Ó˘, ·Ó Î·È Ë ™∞¢ Ê·ÈÓfiÙ·Ó Ï¤ÔÓ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·fi ÙËÓ ¶∫ Î·È Û ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜. ∏ ™∞¢¢ ÂÚÈÁÚ¿ÊËΠÁÈ· ÚÒÙË ÊÔÚ¿ ·fi ÙÔÓ Geronymous ÙÔ 1984 (27) Î·È Û˘Ó‰˘¿˙ÂÈ Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· Ù˘ Û˘Ó¯ԇ˜ ·ÈÌԉȋıËÛ˘ Ì ÙË Û˘Ó¯‹ ·Ô‚ÔÏ‹ ‰È·Ï˘ÙÒÓ Ô˘ÛÈÒÓ Î·È ˘ÁÚÒÓ Ì ÂÈϤÔÓ ÙËÓ ·‡ÍËÛË Ù˘ οı·ÚÛ˘ ÙˆÓ Ô˘ÛÈÒÓ ·˘ÙÒÓ. ∞˘Ùfi ·ÔÙÂÏ› ¤Ó· ÂÈÚfiÛıÂÙÔ ÏÂÔÓ¤ÎÙËÌ· Û ·ÈÌÔ‰˘Ó·ÌÈο ·ÛÙ·ı›˜ ·ÚÚÒÛÙÔ˘˜ Ô˘ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ·Ó¯ıÔ‡Ó ÙÔ Û˘Ó‰˘·ÛÌfi ™∞¢ Î·È ∞∫ ÁÈ· ·Ô‚ÔÏ‹ ˘ÁÚÒÓ Î·È Ô˘Ú·ÈÌÈÎÒÓ ÙÔÍÈÓÒÓ, ·ÓÙ›ÛÙÔȯ·. ∆· Â˘Ú‹Ì·Ù· Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜ Â›Ó·È ·ÚfiÌÔÈ· Ì ·˘Ù¿ ¿ÏÏˆÓ ‰ËÌÔÛȇÛÂˆÓ (22,28), ¯ÚÂÈ¿˙ÂÙ·È fï˜ ÌÂÁ·Ï‡ÙÂÚÔ˜ ·ÚÈıÌfi˜ ·ÛıÂÓÒÓ ÁÈ· Ó· ÂÍ·¯ıÔ‡Ó ·ÛÊ·Ï‹ Û˘ÌÂÚ¿ÛÌ·Ù·. ∏ Ú‡ıÌÈÛË ÙˆÓ ˘ÁÚÒÓ ·ÔÙÂÏ› ÛËÌ·ÓÙÈÎfi ·Ú¿ÁÔÓÙ· ıÂڷ¢ÙÈ΋˜ ·Ú¤Ì‚·Û˘ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì O¡∞. ∏ ·Ê˘‰¿ÙˆÛË ÌÂÈÒÓÂÈ ÙËÓ Î·Ú‰È·Î‹ ·ÚÔ¯‹, ÂÓÒ Ë ˘ÂÚÊfiÚÙˆÛË Ì ˘ÁÚ¿ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·‡ÍËÛË ÙÔ˘ ηډȷÎÔ‡ ¤ÚÁÔ˘ Û ̛· ‹‰Ë ÂËÚ·Ṳ̂ÓË Î·Ú‰È·Î‹ ÏÂÈÙÔ˘ÚÁ›·. ∏ ηٿÛÙ·ÛË ·˘Ù‹ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û Ó¢ÌÔÓÈÎfi Ô›‰ËÌ· Ô˘ ·ÔÙÂÏ› Ì›· ·fi ÙȘ ÈÔ ÛËÌ·ÓÙÈΤ˜ ÂÈÏÔΤ˜ Ù˘ O¡∞. ∂ȉÈο, ÁÈ· Ù· ÌÈÎÚ¿ ‚Ú¤ÊË Î·È ·È‰È¿ Ì ÔÏ˘ÔÚÁ·ÓÈ΋ ·Ó¿ÚÎÂÈ· Ô˘ ¤¯Ô˘Ó ·˘ÍË̤Ó˜ ·Ó¿ÁΘ Û ۛÙÈÛË, ÛÂ Û˘¯Ó¤˜ ÌÂÙ·ÁÁ›ÛÂȘ Î·È ¯ÔÚËÁ‹ÛÂȘ Ê·Ú̿ΈÓ, Ô Î›Ó‰˘ÓÔ˜ Ó· ˘ÂÚÊÔÚÙˆıÔ‡Ó Ì ˘ÁÚ¿ Â›Ó·È ·˘ÍË̤ÓÔ˜ (29). ∞ÓÙ›ıÂÙ·, Ë ·fiÙÔÌË ·Ô‚ÔÏ‹ ˘ÁÚÒÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÙÒÛË Ù˘ ·ÚÙËÚȷ΋˜ ›ÂÛ˘. ∏
˘fiÙ·ÛË ·ÔÙÂÏ› Û˘¯Ó‹ ÂÈÏÔ΋, ΢ڛˆ˜ Ù˘ ∞∫ Î·È ·Ô‰›‰ÂÙ·È Û ÔÏÏ¿ ·›ÙÈ·, ÛÙ· ÔÔ›· Û˘ÌÂÚÈÏ·Ì‚¿ÓÂÙ·È Ë ÚÔ¸¿Ú¯Ô˘Û· ·ÈÌÔ‰˘Ó·ÌÈ΋ ·ÛÙ¿ıÂÈ·, Ë Ù·¯Â›· ·Ô‚ÔÏ‹ ˘ÁÚÒÓ, Ë ˘ÔˆÛ̈ÙÈÎfiÙËÙ· ÙÔ˘ ·›Ì·ÙÔ˜ Î·È Ë ·ÂÏ¢ı¤ÚˆÛË ·ÁÁÂÈԉȷÛÙ·ÏÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ fiˆ˜ ÙÔ ÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘, ÔÈ ÚÔÛÙ·ÁÏ·Ó‰›Ó˜ Î·È ÔÈ ÈÓÙÂÚÏ¢ΛÓ˜ (30,31). ∞ÓÙ›ıÂÙ·, Ë ™∞¢ ·ÔÙÂÏ› ÙË Ì¤ıÔ‰Ô ÂÎÏÔÁ‹˜, ȉȷ›ÙÂÚ· ÁÈ· ·ÈÌÔ‰˘Ó·ÌÈο ·ÛÙ·ı›˜ ·ÛıÂÓ›˜. A˘Ùfi ÔÊ›ÏÂÙ·È ÛÙË ‚·ıÌÈ·›· ·Ô‚ÔÏ‹ ‰È·Ï˘Ì¤ÓˆÓ Ô˘ÛÈÒÓ Î·È ÓÂÚÔ‡, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ÌË ‰È·Ù·Ú¿ÛÛÂÙ·È Ë ˆÛ̈ÙÈ΋ ÈÛÔÚÚÔ›· Î·È Ó· ‰È·ÙËÚÂ›Ù·È Â·Ú΋˜ Ô ÂÓ‰Ô·ÁÁÂÈ·Îfi˜ ¯ÒÚÔ˜ (32). ∂ÈÚfiÛıÂÙ·, ÌÂÚÈÎÔ› ÂÚ¢ÓËÙ¤˜ ·Ô‰›‰Ô˘Ó ÙËÓ ·ÈÌÔ‰˘Ó·ÌÈ΋ ‚ÂÏÙ›ˆÛË ÛÙËÓ ·ÔÌ¿ÎÚ˘ÓÛË Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ·ÈÌԉȋıËÛ˘ ÌÂÛ·›Ô˘ ÌÔÚÈ·ÎÔ‡ ‚¿ÚÔ˘˜ Ô˘ÛÈÒÓ, fiˆ˜ ‚Ú·‰˘ÎÈÓ›Ó˘, Ï¢ÎÔÙÚÈÂÓÒÓ Î·È ıÚÔÌ‚ÔÍ¿Ó˘, Ô˘ Ë ·ÚÔ˘Û›· ÙÔ˘˜ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ¤Ó·ÚÍË Ù˘ ÛËÙÈ΋˜ ηٷÏËÍ›·˜ (33). ™ÙË ÌÂϤÙË Ì·˜, Ë ·Ô‚ÔÏ‹ ˘ÁÚÒÓ Î·È Ì ÙȘ ÙÚÂȘ ÌÂıfi‰Ô˘˜ ÓÂÊÚÈ΋˜ ˘ÔηٿÛÙ·Û˘ ‹Ù·Ó ÈηÓÔÔÈËÙÈ΋, ¯ˆÚ›˜ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÂÈÙÚ¤ÔÓÙ·˜ ÙË Û˘Ó¤¯ÈÛË Ù˘ ·ÁˆÁ‹˜ Ì οı ̤ıÔ‰Ô Ô˘ ÂÊ·ÚÌfiÛÙËÎÂ. OÈ ·ÛıÂÓ›˜ ÛÙË ª∂£ Û˘¯Ó¿ ¤¯Ô˘Ó ‰È·Ù·Ú·¯¤˜ ‹Í˘ ˆ˜ ·ÔÙ¤ÏÂÛÌ· Ù˘ ÔÏ˘ÔÚÁ·ÓÈ΋˜ ·Ó¿ÚÎÂÈ·˜ Ô˘ Û˘Ó˘¿Ú¯ÂÈ Ì O¡∞. O ηıÂÙËÚÈ·ÛÌfi˜ ÙˆÓ ·ÁÁ›ˆÓ ÛÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ·ÈÌÔÚÚ·Á›Â˜ Î·È ‰ËÌÈÔ˘ÚÁ›· ·ÈÌ·ÙˆÌ¿ÙˆÓ ÛÙ· ÛËÌ›· ηıÂÙËÚÈ·ÛÌÔ‡ (34). ∂›Û˘, Ë ·ÓÙÈËÎÙÈ΋ ·ÁˆÁ‹ Ì ¯ÔÚ‹ÁËÛË Ë·Ú›Ó˘ Ô˘ Á›ÓÂÙ·È ÁÈ· ÙËÓ ·ÔÙÚÔ‹ ‰ËÌÈÔ˘ÚÁ›·˜ ıÚfiÌ‚ˆÓ ÛÙÔ Â͈ۈ̷ÙÈÎfi ·Îψ̷ (Ê›ÏÙÚÔ, ·ÚÙËÚȷΤ˜-ÊÏ‚ÈΤ˜ ÁÚ·Ì̤˜), ÌÔÚ› Ó·
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∂ÈÎfiÓ· 6. £ËÎfiÁÚ·ÌÌ· (box-plot) ÛÙÔ ÔÔ›Ô Ê·›ÓÂÙ·È ÙÔ Â‡ÚÔ˜, Ë Ì¤ÛË ÙÈÌ‹ Î·È Ë ÛÙ·ÙÈÛÙÈ΋ ‰È·ÊÔÚ¿ ÙÔ˘ ÈÛÔ˙˘Á›Ô˘ ÁÈ· οı ̤ıÔ‰Ô.
ÚÔηϤÛÂÈ ·ÈÌÔÚÚ·ÁÈ΋ ‰È¿ıÂÛË. ¶·Ú’ fiÏ· ·˘Ù¿, Ô Î·ıÂÙËÚÈ·ÛÌfi˜ ÙˆÓ ÌÂÁ¿ÏˆÓ ·ÁÁ›ˆÓ ÂÓ¤¯ÂÈ ¿ÓÙ· ÙÔÓ Î›Ó‰˘ÓÔ ‰ËÌÈÔ˘ÚÁ›·˜ ıÚfiÌ‚ˆÓ (35). ™ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›·, ÙÔ ÔÛÔÛÙfi ÙˆÓ ÂÈÏÔÎÒÓ ·fi ÙËÓ ÙÔÔı¤ÙËÛË ÎÂÓÙÚÈÎÒÓ ·ÁÁ›ˆÓ Î˘Ì·›ÓÂÙ·È ÌÂٷ͇ 3-4% (35). ™ÙË ÌÂϤÙË Ì·˜ ›¯·Ì ̛· ÂÚ›ÙˆÛË ÂÈÏÔ΋˜ ·fi ıÚfiÌ‚ˆÛË ÌËÚÈ·›·˜ ·ÚÙËÚ›·˜ Û ·È‰› Ì ™∞¢. ™ËÌ·ÓÙÈ΋, ›Û˘, ÂÈÏÔ΋ ÙˆÓ ÌÂıfi‰ˆÓ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘ Â›Ó·È Ë ÂÌÊ¿ÓÈÛË ÏÔÈÌÒÍÂˆÓ Ô˘ ¤¯Ô˘Ó Û¯¤ÛË Â›Ù Ì ÙÔÓ ·ÔÈÎÈÛÌfi ÙˆÓ ÂÓ‰Ô·ÁÁÂÈ·ÎÒÓ Î·ıÂÙ‹ÚˆÓ Ù˘ ™∞¢ ‹ ÙÔ˘ ÂÓ‰ÔÂÚÈÙÔÓ·˚ÎÔ‡ ηıÂÙ‹Ú· Ù˘ ¶∫. ∆Ô ÔÛÔÛÙfi ÙˆÓ ÏÔÈÌÒÍÂˆÓ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË ¯Ú‹ÛË ÂÓ‰Ô·ÁÁÂÈ·ÎÒÓ Î·ıÂÙ‹ÚˆÓ Û ª∂£ ·Ó¤Ú¯ÂÙ·È ¤ˆ˜ 40% (36,37) Î·È ¤¯ÂÈ Û¯¤ÛË Ì ÙË ‰È¿ÚÎÂÈ· ·Ú·ÌÔÓ‹˜ ÙˆÓ Î·ıÂÙ‹ÚˆÓ, ÙËÓ Ù‹ÚËÛË Ì¤ÙÚˆÓ ·ÓÙÈÛË„›·˜ ÁÈ· ÙËÓ ÙÔÔı¤ÙËÛË ·˘ÙÒÓ, ÙË ¯Ú‹ÛË ÙÔ˘˜ ÁÈ· ¿ÏÏÔ˘˜ ÏfiÁÔ˘˜ (Ï‹„Ë ‰ÂÈÁÌ¿ÙˆÓ ·›Ì·ÙÔ˜, ¯ÔÚ‹ÁËÛË Ê·Ú̿ΈÓ) Î·È ÙËÓ ·ÓÔÛÔÏÔÁÈ΋ ηٿÛÙ·ÛË ÙÔ˘ ·ÛıÂÓÔ‡˜. ™ÙË ÌÂϤÙË Ì·˜, ‰‡Ô ·ÛıÂÓ›˜ (13,3%) ·ÚÔ˘Û›·Û·Ó ‚·ÎÙËÚÈ·ÈÌ›· Ô˘ Û¯ÂÙÈ˙fiÙ·Ó Ì ÂÓ‰Ô·ÁÁÂÈ·Îfi ηıÂÙ‹Ú·. ŸÛÔÓ ·ÊÔÚ¿ ÛÙËÓ ¶∫, Ë ÂÚÈÙÔÓ›Ùȉ· ·ÔÙÂÏ› ÙË ÛËÌ·ÓÙÈÎfiÙÂÚË ÂÈÏÔ΋ Ù˘ Î·È ÔÊ›ÏÂÙ·È Û˘Ó‹ıˆ˜, ›Ù Û ·ÈÌ·ÙÔÁÂÓ‹ ‰È·ÛÔÚ¿ (38) ›Ù Û ÂÈÌfiÏ˘ÓÛË ·fi ‚·ÎÙËÚȉȷÎÔ‡˜ ÔÚÁ·ÓÈÛÌÔ‡˜ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ (39). ∆Ô ÔÛÔÛÙfi ÂÌÊ¿ÓÈÛ˘ ÂÚÈÙÔÓ›Ùȉ·˜ Î˘Ì·›ÓÂÙ·È ·fi 9-40% (40,41) Î·È Ê·›ÓÂÙ·È Ó· Û¯ÂÙ›˙ÂÙ·È Î˘Ú›ˆ˜ Ì ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÚÈÙÔÓ·˚΋˜ οı·ÚÛ˘ (42,43). ™ÙË ÌÂϤÙË Ì·˜, ‰‡Ô ·ÛıÂÓ›˜ (28%) Ì ̤ÛË ‰È¿ÚÎÂÈ· ¶∫ 22±6 Ë̤Ú˜ ·ÚÔ˘Û›·Û·Ó ÂÚÈÙÔÓ›Ùȉ·. ∏ ·ÓÙÈÌÂÙÒÈÛ‹ Ù˘ Á›ÓÂÙ·È Ì ÂÓ‰ÔÂÚÈÙÔÓ·˚΋ ¯ÔÚ‹ÁËÛË ·ÓÙÈ-
478
‚ÈÔÙÈÎÒÓ ‚¿ÛÂÈ ÚˆÙÔÎfiÏÏÔ˘ ·ÁˆÁ‹˜. ∞Ó¿ÏÔÁ· Ì ÙËÓ ·ÚÔ˘Û›· ÙÔ˘ Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ÌÈÎÚÔ‚›Ô˘ Î·È ÙËÓ ·ÓÙ·fiÎÚÈÛË ‹ ·ÓÙÔ¯‹ ·˘ÙÔ‡ ÛÙË ıÂڷ›·, ·ÔÊ·Û›˙ÂÙ·È Ë Û˘Ó¤¯ÈÛË ‹ Ë ‰È·ÎÔ‹ Ù˘ ¶∫ Î·È Ë ·ÏÏ·Á‹ Ù˘ Û ¿ÏÏË ÌÔÚÊ‹ οı·ÚÛ˘. O ˘‰ÚÔıÒڷη˜ ·ÔÙÂÏ› Ì›· Û¿ÓÈ· ÂÈÏÔ΋ Ù˘ ¶∫ ÛÙ· ·È‰È¿ (43,44). ∞Ó Î·È Ë ·ÈÙ›· ‰ÂÓ Â›Ó·È ÁÓˆÛÙ‹, Èı·ÓÔÏÔÁÂ›Ù·È Ë ‰È·Ê˘Á‹ ÙÔ˘ ‰È·Ï‡Ì·ÙÔ˜ ·fi ÙËÓ ÂÚÈÙÔÓ·˚΋ ÎÔÈÏfiÙËÙ· ÚÔ˜ ÙË ıˆÚ·ÎÈ΋ ÏfiÁˆ ‰È·ÊÔÚ¿˜ ›ÂÛ˘, ̤ۈ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ Î·È ÙˆÓ ÏÂÌÊ·ÁÁ›ˆÓ. ∞ÓÙÈÌÂÙˆ›˙ÂÙ·È Â›Ù ÌÂ Û˘Ó¤¯ÈÛË Ù˘ ¶∫ Ì ÌÈÎÚfiÙÂÚÔ˘˜ fiÁÎÔ˘˜ ‰È·Ï‡Ì·ÙÔ˜, ›Ù - ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ ·ÓÂÌÊ·ÓÈÛÙ› Ô ˘‰ÚÔıÒڷη˜ - Ì ‰È·ÎÔ‹ Ù˘ ÌÂıfi‰Ô˘ Î·È ÂÊ·ÚÌÔÁ‹ ¿ÏÏ˘ ÌÔÚÊ‹˜ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘. Ÿˆ˜ Ê·›ÓÂÙ·È ·fi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ Ì·˜, Ë ¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Ì O¡∞ Î·È ÔÏ˘ÔÚÁ·ÓÈ΋ ·Ó¿ÚÎÂÈ· Û˘Óԉ‡ÙËΠ·fi ÌÂÁ¿ÏË ıÓËÛÈÌfiÙËÙ· (68%). ¶·ÚfiÌÔÈ· ·ÔÙÂϤÛÌ·Ù· ¤¯Ô˘Ó ·Ó·ÎÔÈÓˆı› Î·È Û ¿ÏϘ ÌÂϤÙ˜ (27,4547) Î·È fiˆ˜ Ê·›ÓÂÙ·È ·fi ÙÔ PRISM score, Ë ‰˘ÛÌÂÓ‹˜ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘ ‰ÂÓ ÔÊ›ÏÂÙ·È ÛÙË ıÂڷ¢ÙÈ΋ ·Ú¤Ì‚·ÛË Ù˘ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘, ·ÏÏ¿ ÛÙËÓ ˘ÔΛÌÂÓË ÓfiÛÔ (48,49). ∞ÓÙ›ıÂÙ·, Ë ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂÓÒÓ Ì O¡∞ Ô˘ ÔÊÂÈÏfiÙ·Ó Û ¿ÏÏ· ·›ÙÈ· (·ÚÂÁ¯˘Ì·ÙÈ΋ ‚Ï¿‚Ë, ÙÔÍÈ΋ ›‰Ú·ÛË Ê·Ú̿ΈÓ) Î·È ‰ÂÓ Û˘Óԉ¢fiÙ·Ó ·fi ÔÏ˘ÔÚÁ·ÓÈ΋ ·Ó¿ÚÎÂÈ·, ‹Ù·Ó 100%, ÔÛÔÛÙfi Ô˘ ¤¯ÂÈ ·Ú·ÙËÚËı› Î·È Û ¿ÏϘ ÌÂϤÙ˜ (5,6). ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ÂÚÈÙÔÓ·˚΋ οı·ÚÛË, Ë ·ÈÌԉȋıËÛË Î·È Ë ·ÈÌÔοı·ÚÛË ·ÔÙÂÏÔ‡Ó ·ÔÙÂÏÂÛÌ·ÙÈΤ˜ ÌÂıfi‰Ô˘˜ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ O¡∞ ÛÙË ª∂£. ∂Î ÙˆÓ ÙÚÈÒÓ, Ë ÂÈÏÔÁ‹ Ù˘ ϤÔÓ Î·Ù¿ÏÏËÏ˘ ÌÂıfi‰Ô˘ Â͈ÓÂÊÚÈ΋˜ οı·ÚÛ˘ ÂÍ·ÚÙ¿Ù·È ·fi ÙË ‚·Ú‡ÙËÙ· Ù˘ ηٿÛÙ·Û˘ ÙÔ˘ ·ÛıÂÓÔ‡˜, ÙËÓ ËÏÈΛ· Î·È ÙËÓ ·Ó¿ÁÎË Ù·¯Â›·˜ ·ÔÌ¿ÎÚ˘ÓÛ˘ ÓÂÚÔ‡. ∫·È ÔÈ ÙÚÂȘ ̤ıÔ‰ÔÈ ·ÔÙÂÏÂÛÌ·ÙÈο ˘ÔηıÈÛÙÔ‡Ó ÙË ÓÂÊÚÈ΋ ·Ó¿ÚÎÂÈ·, ÂÓÒ ÙÔ ÔÛÔÛÙfi ÙˆÓ ÂÈÏÔÎÒÓ Â›Ó·È ÌÈÎÚfi. ŸÌˆ˜, Ë ÙÂÏÈ΋ ¤Î‚·ÛË Î·ıÔÚ›˙ÂÙ·È ·fi ÙËÓ ÂͤÏÈÍË Ù˘ ·Ó¿ÚÎÂÈ·˜ ÙˆÓ ¿ÏÏˆÓ ÔÚÁ¿ÓˆÓ Î·È fi¯È ÌfiÓÔ ·fi ÙË ÓÂÊÚÈ΋ ·Ó¿ÚÎÂÈ·. µÈ‚ÏÈÔÁÚ·Ê›· 1. Gianantonio CA, Vitacco M, Mendilarzu F, Rutty A. Acute renal failure in infancy and childhood. J Pediatr 1962;62:660-662. 2. Gaudio KM, Siegel NJ. Pathogenesis and treatment of acute renal failure. Pediatr Clin N Am 1987;34:771-778. 3. Donkerwolcke RA, Chantler C, Brojer MJC. Pediatric dialysis. In: Replacement of renal function by dialysis. Drukker W, Parsons FM, Maher JF, eds. Boston: Martinus Nijhoff Publisher; 1983. p. 514-535.
¶·È‰È·ÙÚÈ΋ 2001;64:472-480
4. Knochel J. Biochemical, electrolyte and acid-base disturbances in acute renal failure. In: Acute Renal Failure. Brenner BM, Lazarus JM, eds. Philadelphia: WB Saunders, Co; 1983. p. 568-585. 5. Hodson E, Kjellstrand C, Mauer S. Acute renal failure in infants and children: outcome of 53 patients requiring hemodialysis treatment. J Pediatr 1978;93:756-761. 6. Smoyer WE, McAdams C, Kaplan BS, Sherbotie JR. Determinants of survival in pediatric continuous hemofiltration. J Am Soc Nephrol 1995;6:1401-1409. 7. Wilkinson JD, Pollack MM, Ruttiman UE. Outcome of pediatric patients with multiple organ system failure. Crit Care Med 1986;14:271-274. 8. Maxwell L, Colombani P, Fivush B. Renal, endocrine and metabolic failure. In: Rogers M, ed. Textbook of pediatric intensive care. Baltimore: William and Wilkins; 1992. p. 1182-1234. 9. Davenport A, Will E, Davidson A. Improved cardiovascular stability during continuous modes of renal replacements therapy in critically ill patients with hepatic and renal failure. Crit Care Med 1993;21:328-338. 10. Reznik V, Griswold W, Peterson B, Rodarte A, Ferris M, Mendoza S. Peritoneal dialysis for acute renal failure in children. Pediatr Nephrol 1991;5:715-717. 11. Giuffre R, Tam K, Williams W, Freedom R. Acute renal failure complicating pediatric cardiac surgery: a comparison of survivors and nonsurvivors following acute peritoneal dialysis. Pediatr Cardiol 1992;13:208-213. 12. Ronco C, Brendolan A, Brangantini L, Chiarmonte S, Feriani M, Dell’Aquila R et al. Treatment of acute renal failure in newborns by continuous arteriovenous hemofiltration. Kidney Int 1986;29:908-915. 13. Zobel G, Ring E, Kuttning M, Grubbauer H. Five years experience with continuous extracorporeal renal support in pediatric intensive care. Intensive Care Med 1991;17:315319. 14. Bunchman T, Maxvold N, Kershaw D, Sedman A, Custer J. Continuous venovevous hemofiltration in infants and children. Am J Kidney Dis 1995;25:17-21. 15. Ellis EN, Pearson D, Belsha CW, Berry PL. Use of pumpassisted hemofiltration in children with acute renal failure. Pediatr Nephrol 1997;11:196-200. 16. Latta K, Krull F, Wilken M, Burdelski M, Rodeck B, Offner G. Continuous arteriovenous haemofiltration in critically ill children. Pediatr Nephrol 1994;8:334-337. 17. Lowrie L. Renal replacement therapies in multiorgan dysfunction syndrome. Pediatr Nephrol 2000;14:6-12. 18. ªembers of the American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference Committee. American College of Chest Physicians/ Society of Critical Care Medicine Consensus Conference: Definitions for sepsis and organ failure and guidelines for the use of innovative therapies in sepsis. Crit Care Med 1992;20:864-874. 19. Wilkinson JD, Pollack MM, Rutimann UE, Glass NL, Yeh TS. Outcome of pediatric patients with multiple organ system failure. Crit Care Med 1986;14:271-274. 20. Pollack MM, Rutimann UE, Getson P. Pediatric risk of
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mortality (PRISM) score. Crit Care Med 1988;16:1110-1116. 21. µellomo R, Ronco C. Principles of solute clearance during continuous renal replacement therapies. In: Textbook of Critical Care Nephrology. Ronco C, Bellomo R, eds. Klewer Academic Publishers; 1998. p. 1213-1224. 22. Ronco C, Bragantini L, Chiaramonte S, Feriani M, La Greca G. Arteriovenous hemdiafiltration (AVHDF) combined with continuous arteriovenous hemodiafiltration (CAVH). Trans Am Soc Artif Intern Organs XXXI 1995:349-352. 23. Bradley AW, Bunchman TE. Dialysis therapy for children with acute renal failure: survey results. Pediatr Nephrol 2000;15:11-13. 24. Parekh RS, Bunchman TE. Dialysis Support in the Pediatric Intensive Care Unit. Adv Ren Replace Ther 1996;3:326-336. 25. Ehrich JHH. Acute renal failure in infants and children. Int J Artif Organs 1996;19:121-123. 26. Evans ED, Greenbaum L, Ettenger RB. Principles of renal replacement therapy in children. Pediatr Clin N Am 1995;42:1579-1602. 27. Geronymous R, Schneider N. Continuous arteriovenous hemodialysis: A new modality for treatment of acute renal failure. Trans Am Soc Artif Intern Organs 1984;30:687-689. 28. µellomo R, Farmer M, Parkin G, Wright C, Boyce N. Severe acute renal failure: a comparison of acute continuous hemodiafiltration and conventional dialytic therapy. Nephron 1995;71:59-64. 29. Zobel G, Kuttnig M, Grubbauer H. Continuous arteriovenous hemofiltration versus continuous venovenous hemofiltration in critically ill pediatric patients. In: Sieberth H, ªann, Stummvol H, eds. Continuous hemofiltration. Basel: Karger; 1991. p. 257-260. 30. Noris M, Benigni M, Boccardo P, Aiello S, Gaspari F, Todeschini M et al. Enhanced nitric oxide synthesis in uremia: implications for platelet dysfunction and dialysis hypotension. Kidney Int 1993;44:445-450. 31. Klouche K, Beraud J. Risks and hazards of intermittent renal replacement therapy. In: Textbook of Critical Care Nephrology. Ronco C, Bellomo R, eds. Netherlands: Klewer Academic Publishers; 1998. p. 1441-1456. 32. Rogiers P, Zhang H, Vincent JL. Hemofiltration in sepsis and septic shock. In: Vincent JL, ed. Year Book of Intensive Care and Emergency Medicine. Springer; 1997. p. 133-139. 33. Bellomo R. Continuous hemofiltration as blood purification in sepsis. New Horizon 1995;3:732-737. 34. Wedel SK. Continuous Renal Replacement Therapy. In: Update in Intensive Care and Emergency Medicine 1991. Vincent JL, ed. Basel: Springer-Verlag; 1991. p. 444-453. 35. Ronco C, Bellomo R. Complications of continuous renal replacement therapies. In: Textbook of Critical Care Nephrology. Ronco C, Bellomo R, eds. Netherlands: Klewer Academic Publishers; 1998. p. 1351-1355. 36. Richet H, Hubert B, Nitemberg G. Prospective multicenter study of vascular-catheter-related complications and risk factors for positive central-catheter cultures in intensive care unit patients. J Clin Microbiol 1990;143:52-56. 37. Wirmer AF. Infection control and prevention strategies in the ICU. Intensive Care Med 1994;20:S7-S11. 38. Williams PS, Hendy MS, Ackrill P. Routine daily
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Olden RW, Haeck J, Struijk DG et al. Predicting mortality in intensive care patients with acute renal failure treated with dialysis. J Am Soc Nephrol 1997;8:111-117. 48. Mitchell JP, Schuller D, Calandrino S, Schuster DP. Improved outcome based on fluid management in critically ill patients requiring pulmonary artery catheterization. Am Rev Resp Dis 1992;145:990-998. 49. Bagshava O, Anaes F, Huthinson A. Continuous arteriovenous haemofiltration and respiratory function in multiple organ system failure. Intensive Care Med 1992;18:334-338.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 05-02-2001 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 27-03-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ¡ÈÎfiÏ·Ô˜ ∑¿‚Ú·˜ µÂÓÙÔ‡ÚË 33, 155 61, ÃÔÏ·ÚÁfi˜ e-mail: nikzav@otenet.gr
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1 Abramson MJ, Bailey MJ, Couper FJ, Driver JS, Drummer OH, Forbes AB et al Are asthma medications and management related to deaths from asthma? Am J Respir Crit Care Med 2001;163:12-18
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ORIGINAL ARTICLE
∞ÓÔÛÔÏÔÁÈÎÔ› ·Ú¿ÌÂÙÚÔÈ ÛÙËÓ ÂÎÙ›ÌËÛË Ù˘ ÂͤÏÈ͢ Ù˘ ·È‰È΋˜ Ó¢ÌÔÓ›·˜ ª. ∂ÌÔÚÈ¿‰Ô˘1, ª. ÷Ù˙ËÛÙ˘ÏÈ·ÓÔ‡2, ∫. ÷˚‰ÔÔ‡ÏÔ˘1, Ã. ∞ÁÁÔ˘Úȉ¿ÎË3, µ. ∆Û·‚‰·Ú›‰Ô˘3, °. µ·ÚÏ¿Ì˘1, ™. ∫·Ú·Ì¤Ú˘1
Immunologic parameters in the evaluation of the eventual outcome of childhood pneumonia M. Eboriadou1, M. Hatzistilianou2, K. Haidopoulou1, C. Aggouridaki3, V. Tsavdaridou3, G. Varlamis1, S. Karamperis1
¶ÂÚ›ÏË„Ë: ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ ‹Ù·Ó Ó· ÂÎÙÈÌ‹ÛÔ˘Ì ÙË ‰È·ÁÓˆÛÙÈ΋ ·Í›· ·ÓÔÛÔÏÔÁÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ ÛÙËÓ ÂͤÏÈÍË Ù˘ Ó¢ÌÔÓ›·˜ ÛÙ· ·È‰È¿. ∏ ÌÂϤÙË ÂÚÈÂÏ¿Ì‚·Ó ‰‡Ô ÔÌ¿‰Â˜ ·È‰ÈÒÓ, ∞ Î·È µ. ∆ËÓ ÔÌ¿‰· ∞ ·ÔÙÂÏÔ‡Û·Ó 39 ·È‰È¿ ËÏÈΛ·˜ 5±1,2 ÂÙÒÓ, Ì ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ‰È¿ÁÓˆÛË Ù˘ Ó¢ÌÔÓ›·˜, ÂÓÒ ÙËÓ ÔÌ¿‰· µ, 30 ˘ÁÈ‹ ·È‰È¿ Ù˘ ›‰È·˜ ËÏÈΛ·˜ Î·È Ê‡ÏÔ˘. ™Ù· ·È‰È¿ Î·È ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ ¤ÁÈÓ ÛÂÈÚÈ·Îfi˜ ÚÔÛ‰ÈÔÚÈÛÌfi˜: ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ Î·È ÙˆÓ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· Î·È ÙˆÓ ÂȤ‰ˆÓ Ù˘ C-·ÓÙȉÚÒÛ·˜ ÚˆÙ½Ó˘ (CRP), ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ IgG, IgM Î·È IgA, ÙˆÓ Î˘ÙÔÎÈÓÒÓ 2 (IL-2) Î·È 6 (IL-6) ηıÒ˜ Î·È ÙˆÓ ‰È·Ï˘ÙÒÓ ˘Ô‰Ô¯¤ˆÓ Ù˘ ÈÓÙÂÚÏ¢ΛÓ˘ 2 (sIL-2R). ™‡Ìʈӷ Ì ٷ ·ÔÙÂϤÛÌ·Ù¿ Ì·˜, Ë CRP Î·È Ë IL-6 Â›Ó·È ÔÈ ·ÓÔÛÔÏÔÁÈÎÔ› ‰Â›ÎÙ˜ Ô˘ ÌÂÙ·‚¿ÏÏÔÓÙ·È ÓˆÚ›˜ ‹‰Ë ·fi ÙËÓ 3Ë Ë̤ڷ ıÂڷ›·˜, ÂÓÒ Ë IL-2 Î·È ÔÈ sIL-2R ÌÂÙ·‚¿ÏÏÔÓÙ·È ÌÂÙ¿ ÙËÓ 5Ë Ë̤ڷ ıÂڷ›·˜. ∏ ·Ú·ÎÔÏÔ‡ıËÛË, ÏÔÈfiÓ, Ù˘ CRP Î·È Ù˘ IL-6 ‚ÔËı¿ ÛÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ÂͤÏÈ͢ Ù˘ ·È‰È΋˜ Ó¢ÌÔÓ›·˜.
Abstract: The aim of this study was to evaluate the diagnostic value of the immunologic parameters in the outcome of lung infection (pneumonia). A total of 39 hospitalized patients (mean age±SD: 5±1.2 years) with pneumonia (group A) and 30 healthy children (group B) were included in the study. Serial determinations of the white blood cell count, absolute number of total neutrophils, C-reactive protein, immunoglobulins IgG, IgM and IgA, interleukin-2 (IL-2), interleukin-6 (IL-6) and soluble interleukin-2 receptor (sIL-2R) were evaluated in all patients. Our results revealed that CRP and IL-6 were the two immunologic markers that changed early (on the 3rd day of treatment), while the levels of IL-2 and sIL-2R changed later (5th day of treatment). In conclusion, repeated examinations of CRP and IL-6 are important in evaluating the course and outcome of pneumonia in children.
§¤ÍÂȘ ÎÏÂȉȿ: Ó¢ÌÔÓ›·, ÈÓÙÂÚÏ¢ΛÓË 2, ÈÓÙÂÚÏ¢ΛÓË 6, ‰È·Ï˘ÙÔ› ˘Ô‰Ô¯Â›˜ Ù˘ ÈÓÙÂÚÏ¢ΛÓ˘ 2, C-·ÓÙȉÚÒÛ· ÚˆÙ½ÓË.
Key words: pneumonia, interleukin-2, interleukin6, soluble interleukin-2 receptor, C-reactive protein.
∂ÈÛ·ÁˆÁ‹ ∏ Ó¢ÌÔÓ›· ·Ú·Ì¤ÓÂÈ Ì›· Û˘¯Ó‹ ÓfiÛÔ˜ Î·È Ë Û˘¯ÓfiÙÂÚË ·ÈÙ›· ÂÓ‰ÔÓÔÛÔÎÔÌÂȷ΋˜ ÓÔÛËÏ›·˜ ÌÂٷ͇ ÙˆÓ ÓÔÛÔÎÔÌÂÈ·ÎÒÓ ÏÔÈÌÒÍÂˆÓ (1,2). ∏ ¿ÚÎÂÈ· ÙÔ˘ ·ÓÔÛÈ·ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Â›Ó·È Î·ıÔÚÈÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÛÙËÓ ¤Î‚·ÛË Ù˘ ÊÏÂÁÌÔÓ‹˜
Û ·ÛıÂÓ›˜ Ì Ó¢ÌÔÓ›· (3,4). ™Â Ê˘ÛÈÔÏÔÁÈΤ˜ Û˘Óı‹Î˜, Ù· ΢„ÂÏȉÈο Ì·ÎÚÔÊ¿Á· Â›Ó·È Ù· ΢ڛˆ˜ ·ÙÙ·Ú· Ô˘ ÚÔÛÙ·ÙÂ‡Ô˘Ó ÙÔ˘˜ Ó‡ÌÔÓ˜ ·fi ÙËÓ Â›ÛÔ‰Ô ÙˆÓ ÌÈÎÚÔ‚›ˆÓ (3-5). ∆· ·ÙÙ·Ú· ·˘Ù¿, ÛÂ Û˘ÓÂÚÁ·Û›· Ì ٷ ¿ÏÏ· ·ÙÙ·Ú· ÙÔ˘ ·ÓÔÛÔÔÈËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Û˘Ì‚¿ÏÏÔ˘Ó, Ì ÙËÓ
1 ¢’ ¶·ÓÂÈÛÙËÌȷ΋ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£. 2 µ’ ¶·ÓÂÈÛÙËÌȷ΋ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£. 3 OÚÔÏÔÁÈÎfi ∆Ì‹Ì· ªÈÎÚÔ‚ÈÔÏÔÁÈÎÔ‡ ∂ÚÁ·ÛÙËÚ›Ô˘ ¡ÔÛÔÎÔÌÂ›Ô ∞.Ã.∂.¶.∞., ∞.¶.£.
1 4th Pediatric Clinic of Aristotelion University, Thessaloniki 2 2nd Pediatric Clinic of Aristotelion University, Thessaloniki 3 Serology Division of Microbiology Laboratory AHEPA Hospital, Aristotelion University, Thessaloniki
481
¶·È‰È·ÙÚÈ΋ 2001;64:481-487
Paediatriki 2001;64:481-487
ÚÈÙÈÎfi˜ fiÓÔ˜, ÂÓÙÔÈṲ̂ÓË Ì›ˆÛË Ì¤¯ÚÈ ÂÍ·Ê¿ÓÈÛË ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ „Èı˘Ú›ÛÌ·ÙÔ˜ (΢ڛˆ˜ Û ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿), ‚ÚÔÁ¯È΋ ·Ó·ÓÔ‹ ‹ ÛˆÏËÓ҉˜ ʇÛËÌ· Î·È ÏÂÙÔ› ˘ÁÚÔ› ÚfiÁ¯ÔÈ ÂÓÙÔÈṲ̂ÓÔÈ. ‚) ∂ÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·, fiˆ˜: ·˘ÍË̤ÓÔ˜ ·ÚÈıÌfi˜ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ (>15.000 ·ÙÙ·Ú·/mm3) Ì ÔÏ˘ÌÔÚÊÔ˘Ú‹ÓˆÛË Î·È Èı·ÓfiÓ ÛÙÚÔÊ‹ ÙÔ˘ Ï¢ÎÔ΢ÙÙ·ÚÈÎÔ‡ Ù‡Ô˘ ÚÔ˜ Ù· ·ÚÈÛÙÂÚ¿ (Ú·‚‰Ô‡ÚËÓ·), ÌÂÁ¿ÏË ·‡ÍËÛË ∆∫∂ Î·È CRP Î·È ıÂÙÈΤ˜ ηÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜ ÁÈ· ÌÈÎÚԂȷ΋ Ïԛ̈ÍË Î·È Î·ÌÈ¿ ÊÔÚ¿ ˘ÔÍ·ÈÌ›· Ì ÎÔÚÂÛÌfi Ô͢·ÈÌÔÛÊ·ÈÚ›Ó˘ (SaO2) οو ÙˆÓ 93-95%. Á) ∞ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη, fiˆ˜ ÂÓÙÔÈṲ̂ÓË ‡ÎÓˆÛË (Û˘Ó‹ıˆ˜ ÏԂ҉˘) Ì ‹ ¯ˆÚ›˜ ·ÚÔ˘Û›· Ï¢ÚÈÙÈ΋˜ Û˘ÏÏÔÁ‹˜. ∏ ‰È¿ÚÎÂÈ· Ù˘ ÓfiÛÔ˘ ÂÎÙÈÌ‹ıËΠ·fi ÙËÓ Ë̤ڷ ¤Ó·Ú͢ ÙÔ˘ ˘ÚÂÙÔ‡. ¶ÂÚÈÙÒÛÂȘ Ì ·Î·ıfiÚÈÛÙË Ë̤ڷ ¤Ó·Ú͢ ÙÔ˘ ˘ÚÂÙÔ‡ ‹ Ì ·ÌÊ›‚ÔÏÔ ÈÛÙÔÚÈÎfi ‹ Â˘Ú‹Ì·Ù· ·fi ÙË Ê˘ÛÈ΋ ÂͤٷÛË, ·ÔÎÏ›ÛÙËÎ·Ó ·fi ÙË ÌÂϤÙË. ∆· ·ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÙˆÓ ·ÛıÂÓÒÓ ÌÂÏÂÙ‹ıËÎ·Ó ·fi ·ÎÙÈÓÔÏfiÁÔ˘˜ Ô˘ ‰ÂÓ ÁÓÒÚÈ˙·Ó Ù· ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·. ∆· ·È‰È¿ Ù˘ ÌÂϤÙ˘ ¯ˆÚ›ÛÙËÎ·Ó Û ‰‡Ô ÔÌ¿‰Â˜ (∞ Î·È µ). ∏ ÔÌ¿‰· ∞ ÂÚÈÂÏ¿Ì‚·Ó 39 ·È‰È¿ Ì Ó¢ÌÔÓ›· ËÏÈΛ·˜ 5±1,2 ÂÙÒÓ Ì ÙËÓ ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ‰È¿ÁÓˆÛË Ù˘ Ó¢ÌÔÓ›·˜, ÂÓÒ Ë ÔÌ¿‰· µ ÂÚÈÂÏ¿Ì‚·Ó 30 ˘ÁÈ‹ ·È‰È¿. ™Ù· ·È‰È¿ Î·È ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì·: Ô ·ÚÈıÌfi˜ ÙˆÓ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ Ì ·Ó·Ï˘Ù‹ (coulter), Ô ·fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ Î·È Ë ∆∫∂ ·fi ¿ÌÂÛÔ ·Ú·Û··ÛÌ· Î·È Ù· ›‰· Ù˘ C-·ÓÙȉÚÒÛ·˜ ÚˆÙ½Ó˘ (CRP) Î·È ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ IgG, IgM Î·È IgA Ì ÓÂÊÂÏÔÌÂÙÚ›·. ∂›Û˘, ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó ÔÈ ÈÓÙÂÚÏ¢ΛÓ˜ 2 (IL-2) Î·È 6 (IL-6) Î·È ÔÈ ‰È·Ï˘ÙÔ› ˘Ô‰Ô¯Â›˜ Ù˘ ÈÓÙÂÚÏ¢ΛÓ˘ 2 (sIL-2R) Ì ÙË Ì¤ıÔ‰Ô ELISA. ÃÚËÛÈÌÔÔÈ‹ıËΠkit ÂÌÔÚ›Ô˘ (R & D System) ·ÎÔÏÔ˘ıÒÓÙ·˜ ÙȘ Ô‰ËÁ›Â˜ Ù˘ ηٷÛ΢¿ÛÙÚÈ·˜ ÂÙ·ÈÚ›·˜. OÈ ·Ú·¿Óˆ ·Ú¿ÌÂÙÚÔÈ, ÂÎÙfi˜ ·fi ÙËÓ ∆∫∂, ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó ÛÙËÓ ÔÌ¿‰· ∞ ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ÙËÓ 3Ë, 5Ë Î·È 10Ë Ë̤ڷ Ù˘ ıÂڷ›·˜, ÂÓÒ ÛÙËÓ ÔÌ¿‰· µ (ÂϤÁ¯Ô˘) Ì›· ÊÔÚ¿. ∏ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ¤ÁÈÓ Ì ÂÎÙ›ÌËÛË ÙˆÓ Ì¤ÛˆÓ ÙÈÌÒÓ ±SD Î·È ¯ÚËÛÈÌÔÔÈ‹ıËΠÙÔ test ÛËÌ·ÓÙÈÎfiÙËÙ·˜ ÌÂ-
¤ÎÎÚÈÛË ÙˆÓ Î˘ÙÔÎÈÓÒÓ, ÛÙËÓ ÂͤÏÈÍË Ù˘ ÊÏÂÁÌÔÓÒ‰Ô˘˜ ·ÓÙ›‰Ú·Û˘ (3-7). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Û ÌÂϤÙ˜ Ô˘ ¤¯Ô˘Ó ‰ËÌÔÛÈ¢ı›, ‚Ú¤ıËΠ̛· Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙˆÓ ÂȤ‰ˆÓ ÙˆÓ ‰È·ÊfiÚˆÓ ÚÔÊÏÂÁÌÔÓˆ‰ÒÓ Î·È ÊÏÂÁÌÔÓˆ‰ÒÓ Î˘ÙÔÎÈÓÒÓ Î·È Ù˘ ‚·Ú‡ÙËÙ·˜ Ù˘ Ó¢ÌÔÓ›·˜ (8-10). ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜ ‹Ù·Ó: ·) Ë ‰ÈÂÚ‡ÓËÛË ÙˆÓ ÂȤ‰ˆÓ ·ÓÔÛÔÏÔÁÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ÔÍ›·˜ Ê¿Û˘ Ù˘ Ó¢ÌÔÓ›·˜ ÛÙ· ·È‰È¿, ‚) Ë Û˘Û¯¤ÙÈÛË ÙˆÓ Â˘ÚËÌ¿ÙˆÓ Ì ÙËÓ ÂͤÏÈÍË Ù˘ ÓfiÛÔ˘ Î·È Á) Ë ÂÎÙ›ÌËÛË Ù˘ ‰È·ÁÓˆÛÙÈ΋˜ ·Í›·˜ ÙˆÓ ·ÓÔÛÔÏÔÁÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ ÛÙËÓ ÂͤÏÈÍË Ù˘ Ó¢ÌÔÓ›·˜. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ
∞ÚÈıÌfi˜ Ï¢ÎÒÓ/ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ/mm3
∆Ô ˘ÏÈÎfi Ù˘ ÌÂϤÙ˘ ·ÔÙ¤ÏÂÛ·Ó 39 ·È‰È¿ (19 ·ÁfiÚÈ· Î·È 20 ÎÔÚ›ÙÛÈ·) ËÏÈΛ·˜ 14 ÌËÓÒÓ ¤ˆ˜ 13 ¯ÚfiÓˆÓ Ì ̤ÛË ËÏÈΛ· ±SD: 5±1,2 ¯ÚfiÓÈ·, Ô˘ ÓÔÛËχıËÎ·Ó ÛÙËÓ ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ÙÔ˘ ∞.¶.£.(¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞, £ÂÛÛ·ÏÔÓ›ÎË) ÁÈ· Èı·Ó‹ ÌÈÎÚԂȷ΋ Ó¢ÌÔÓ›· ÙÔ ‰È¿ÛÙËÌ· 19971998. ™Ù· ·È‰È¿, ÂÎÙfi˜ ·fi Ù· ÓÂÔÁÓ¿ Ô˘ ¤¯Ô˘Ó ÔÍ›· Ó¢ÌÔÓ›·, ‰ÂÓ ˘¿Ú¯Ô˘Ó ·ÍÈfiÈÛÙ· ÎÏÈÓÈο Û˘ÌÙÒÌ·Ù· ‹ ÛËÌ›· Ô˘ Ó· ‰È·ÊÔÚÔÔÈÔ‡Ó ÙË ÌÈÎÚԂȷ΋ Ó¢ÌÔÓ›· ·fi ¿ÏÏ· ÏÔÈÌÒ‰Ë ·›ÙÈ·. ∆· ÂÈÎÚ·Ù¤ÛÙÂÚ·, fï˜, ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· Û‡Ìʈӷ Ì ÙË ‚È‚ÏÈÔÁÚ·Ê›· (11-12), ÛÙ· ÔÔ›· Î·È ÛÙËÚȯı‹Î·ÌÂ, ‹Ù·Ó: ·) ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ηٿ ÙËÓ ÂÈÛ·ÁˆÁ‹, fiˆ˜ ˘„ËÏfi˜ ˘ÚÂÙfi˜ Ì ·ÈÊÓ›‰È· ¤Ó·ÚÍË, ‚‹¯·˜, ·›ÛıËÌ· ÎfiˆÛ˘, ·ÚÓËÙÈο ÛËÌ›· ÈÔÁÂÓÔ‡˜ Ïԛ̈͢ fiˆ˜ ÚÈÓÈ΋ Û˘ÌÊfiÚËÛË, ÚÈÓfiÚÚÔÈ·, ÂÈÂÊ˘Î›Ùȉ· Î·È Ù¤ÏÔ˜ ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· (·Ó·¤Ù·ÛË ÚÈÓÈÎÒÓ ÙÂÚ˘Á›ˆÓ, ÂÈÛÔÏΤ˜ ÌÂÛÔχÚÈˆÓ ‰È·ÛÙËÌ¿ÙˆÓ, ˘ÂÚÎÏ›‰È˜ Î·È ÂÈÁ·ÛÙÚ›Ô˘, Ù·¯‡ÓÔÈ· ≥50-60 ·Ó·ÓÔ¤˜/min ÁÈ· ·È‰È¿ οو ÙˆÓ 12 ÌËÓÒÓ Î·È ≥40 ·Ó·ÓÔ¤˜/min ÁÈ· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿, Ù·¯˘Î·Ú‰›· Î·È ÁÔÁÁ˘ÛÌfi˜) ȉȷ›ÙÂÚ· Û ‚Ú¤ÊË Î·È ÌÈÎÚ¿ ·È‰È¿, ÂÓÒ Û ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ ÂÈÚfiÛıÂÙ·, ·ÓËÛ˘¯›· Î·È fiÓÔ˜ ÛÙÔ ÛÙ‹ıÔ˜. ∂›Û˘, Ï¢-
45000 40000
A
35000
A
30000 25000 A
20000 15000
A B
10000 5000 0 ¶ÚÈÓ ÙË ıÂڷ›·
3Ë Ë̤ڷ
5Ë Ë̤ڷ
10Ë Ë̤ڷ
∏̤Ú˜ ıÂڷ›·˜ ∂ÈÎfiÓ· 1. ∞ÚÈıÌfi˜ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ Î·È ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ ÚÈÓ Î·È ÙËÓ 3Ë, 5Ë Î·È 10Ë Ë̤ڷ ıÂڷ›·˜ ÛÙȘ ÔÌ¿‰Â˜ ∞ Î·È µ.
482
¶·È‰È·ÙÚÈ΋ 2001;64:481-487
Paediatriki 2001;64:481-487
30
IgG (gr/L)
25 20
A
µ
A
A
A
15 10 5 0 ¶ÚÈÓ ÙË ıÂڷ›·
3Ë Ë̤ڷ
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6 5 A IgM (gr/L)
4
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3 A µ
2 1 0 ¶ÚÈÓ ÙË ıÂڷ›·
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IgA (gr/L)
3
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1,5 1 0,5 0 ¶ÚÈÓ ÙË ıÂڷ›·
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∂ÈÎfiÓ· 2. ∂›Â‰· ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ IgG (2A), IgM (2B) Î·È IgA (2°) ÚÈÓ Î·È ÙËÓ 3Ë, 5Ë Î·È 10Ë Ë̤ڷ ıÂڷ›·˜ ÛÙȘ ÔÌ¿‰Â˜ ∞ Î·È µ.
483
¶·È‰È·ÙÚÈ΋ 2001;64:481-487
Paediatriki 2001;64:481-487
25
CRP (mg/dl)
20 A 15
10
A
5
A µ
A
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3Ë Ë̤ڷ
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∏̤Ú˜ ıÂڷ›·˜ ∂ÈÎfiÓ· 3. ª¤ÛË ÙÈÌ‹ ± SD ÙˆÓ ÂȤ‰ˆÓ Ù˘ CRP ÚÈÓ Î·È ÙËÓ 3Ë, 5Ë Î·È 10Ë Ë̤ڷ ıÂڷ›·˜ ÛÙȘ ÔÌ¿‰Â˜ ∞ Î·È µ.
ٷ͇ ÙˆÓ ‰‡Ô ÔÌ¿‰ˆÓ. OÈ ·Ú¿ÌÂÙÚÔÈ Û˘Û¯ÂÙ›ÛÙËÎ·Ó ÌÂٷ͇ ÙÔ˘˜ Ì ÙË ‚Ô‹ıÂÈ· ÙÔ˘ Û˘ÓÙÂÏÂÛÙ‹ Û˘Û¯¤ÙÈÛ˘ ηٿ Pearson (r).
∞ÔÙÂϤÛÌ·Ù· ¢È·ÁÓÒÛÙËΠÏԂ҉˘ ‹ ÙÌËÌ·ÙÈ΋ Ó¢ÌÔÓ›· Û 31/39 ÂÚÈÙÒÛÂȘ Î·È Î·Ù¿ ÙfiÔ˘˜ ‰ÈËı‹ÛÂȘ ‹ ·ÙÂÏÂÎÙ·Û›· Û 8/39 ·È‰È¿. ™ÙËÓ ∂ÈÎfiÓ· 1 Ê·›ÓÂÙ·È ·Ó·Ï˘ÙÈο Ô ·ÚÈıÌfi˜ ÙˆÓ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ Î·È ÙˆÓ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ ÛÙ· ·È‰È¿ Ù˘ ÔÌ¿‰·˜ ∞ Î·È µ. ∏ Ì›ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ ‹Ù·Ó 12,6%, 5,6% Î·È 39,5% ÙËÓ 3Ë, 5Ë Î·È 10Ë Ë̤ڷ ıÂڷ›·˜ ·ÓÙ›ÛÙÔȯ· Î·È ÙˆÓ ÔÏ˘ÌÔÚÊÔ˘Ú‹ÓˆÓ ‹Ù·Ó 29,7%, 3,3% Î·È 50,4% ·ÓÙ›ÛÙÔȯ·. ™ÙËÓ ∂ÈÎfiÓ· 2 Ê·›ÓÔÓÙ·È ·Ó·Ï˘ÙÈο ÔÈ ÙÈ̤˜ ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ IgG (∂ÈÎfiÓ· 2·), IgM (∂ÈÎfiÓ· 2‚) Î·È IgA (∂ÈÎfiÓ· 2Á). ∏ ·‡ÍËÛË ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ IgG, IgM Î·È IgA ÙËÓ 3Ë Ë̤ڷ ‹Ù·Ó 16,7%, 36%, 25,8%, ÙËÓ 5Ë Ë̤ڷ 18,9%, 31,3% Î·È 26,3% Î·È ÙËÓ 10Ë Ë̤ڷ 19,6%, 58,1% Î·È 43,1% ·ÓÙ›ÛÙÔȯ·. ™ÙËÓ ∂ÈÎfiÓ· 3 Ê·›ÓÂÙ·È Ë Ì¤ÛË ÙÈÌ‹ Î·È Ë ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË ÙˆÓ ÂȤ‰ˆÓ Ù˘ CRP. ∏ ÙÒÛË ÙˆÓ ÂȤ‰ˆÓ Ù˘ CRP ÛÙËÓ ÔÌ¿‰· ∞ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ (p<0,001) ÌÂٷ͇ ·˘ÙÒÓ ÚÈÓ Î·È ÙËÓ 3Ë Ë̤ڷ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜. ™ÙËÓ ∂ÈÎfiÓ· 4 Ê·›ÓÔÓÙ·È ·Ó·Ï˘ÙÈο ÔÈ ÙÈ̤˜ ÙˆÓ IL-2 (∂ÈÎfiÓ· 4·), IL-6 (∂ÈÎfiÓ· 4‚) Î·È sIL-2R (∂ÈÎfiÓ· 4Á). ™ÙÔÓ ¶›Ó·Î· 1 Ê·›ÓÔÓÙ·È ÔÈ Î·Ù¿ Pearson Û˘Û¯ÂÙ›ÛÂȘ (r) ÙˆÓ ·Ú·¿Óˆ ·Ú·Ì¤ÙÚˆÓ Î·È Ù˘ ∆∫∂. ™˘˙‹ÙËÛË ∆· ›‰· ÙˆÓ Î˘ÙÔÎÈÓÒÓ ÙÔ˘ ÔÚÔ‡ Û ۯ¤ÛË Ì ‰È¿ÊÔÚ˜ ÊÏÂÁÌÔÓÒ‰ÂȘ ηٷÛÙ¿ÛÂȘ, ¤¯Ô˘Ó
484
ÌÂÏÂÙËı› Û ‰È¿ÊÔÚ˜ ÏÔÈÌÒÍÂȘ fiˆ˜ ÛËÙÈÎfi shock (6,13), ÛËÙÈÎfi Û‡Ó‰ÚÔÌÔ (7), Ó¢ÌÔӛ˜ (8-10,14-18) Î.¿. ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ¤ÁÈÓ ÚÔÛ¿ıÂÈ· Ó· ÌÂÏÂÙËıÔ‡Ó Ù· ›‰· ΢ÎÏÔÊÔÚÔ‡ÓÙˆÓ Î˘ÙÔÎÈÓÒÓ Û ·È‰È¿ Ì ÂÓ‰ÂÈÎÙÈο ÛÙÔȯ›· ÌÈÎÚԂȷ΋˜ Ó¢ÌÔÓ›·˜ ÛÙËÓ ÔÍ›· Ê¿ÛË Ù˘ ÓfiÛÔ˘, ÒÛÙ ӷ ÂÎÙÈÌËıÔ‡Ó ÂÚ·ÈÙ¤Úˆ Û·Ó ÚÔÁÓˆÛÙÈÎÔ› ‰Â›ÎÙ˜ ÛÙËÓ ÂÎÙ›ÌËÛË Î·È ÙËÓ ÂͤÏÈÍË Ù˘ Ïԛ̈͢. OÈ ÚˆÙ½Ó˜ ÔÍ›·˜ Ê¿Û˘ Î·È Ì¿ÏÈÛÙ· Ë CRP, ÚˆÙ½ÓË ÔÍ›·˜ Ê¿Û˘ Ô˘ ·Ú¿ÁÂÙ·È, fiˆ˜ Â›Ó·È ÁÓˆÛÙfi, ÛÙÔ ‹·Ú ÛÙËÓ ¤Ó·ÚÍË Ù˘ ÊÏÂÁÌÔÓÒ‰Ô˘˜ ·ÓÙ›‰Ú·Û˘, Â›Ó·È ‰Â›ÎÙ˘ ÊÏÂÁÌÔÓ‹˜, ‡ÎÔÏ· ÚÔÛ‰ÈÔÚ›˙ÂÙ·È, ·ÏÏ¿ ·Ó¢ڛÛÎÂÙ·È Î·È Û ¿ÏϘ ·ıÔÏÔÁÈΤ˜ ηٷÛÙ¿ÛÂȘ (19). ÃÚËÛÈÌÔÔÈÂ›Ù·È Ôχ ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË, fi¯È ÌfiÓÔ ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË, ·ÏÏ¿ Î·È ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ÔÚ›·˜ Ù˘ ÂͤÏÈ͢ Ù˘ ÊÏÂÁÌÔÓ‹˜. O ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù˘ Ú¤ÂÈ, ÂÔ̤ӈ˜, Ó· Á›ÓÂÙ·È Û ٷÎÙÈο ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù·, ÁÈ·Ù› ¤¯ÂÈ ‰È·ÁÓˆÛÙÈ΋ Î·È ÚÔÁÓˆÛÙÈ΋ ·Í›·. ™ÙËÓ ÚÔÛ¿ıÂÈ¿ Ì·˜ Ó· ‰ÈÂÚ¢ӋÛÔ˘Ì ·ÓÔÛÔÏÔÁÈΤ˜ ‰È·ÁÓˆÛÙÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜ ÛÙËÓ Ó¢ÌÔÓ›·, ÂÈϤͷÌ ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ÚÔÊÏÂÁÌÔÓÒ‰Ô˘˜ IL-6 Î·È Ù˘ ÌÂÙ·ÊÏÂÁÌÔÓÒ‰Ô˘˜ IL-2 ηıÒ˜ Î·È ÙˆÓ sIL-2R. ™ÙË ÌÂϤÙË Ì·˜, Ë CRP ‚Ú¤ıËΠfiÙÈ ‹Ù·Ó Ë ÚÒÙË ·Ú¿ÌÂÙÚÔ˜ Ô˘ ÂÏ·ÙÙÒıËΠÛËÌ·ÓÙÈο (p<0,001) Ôχ ÓˆÚ›˜ ‹‰Ë ·fi ÙËÓ 3Ë Ë̤ڷ ıÂڷ›·˜ Ù˘ Ó¢ÌÔÓ›·˜ ÛÙËÓ ÔÌ¿‰· ÙˆÓ ·ÚÚÒÛÙˆÓ (∞), ÂÓÒ Ë IL-6 ÛËÌ›ˆÛ ÛËÌ·ÓÙÈ΋, ›Û˘, ÙÒÛË ·fi ÙËÓ 5Ë Ë̤ڷ ıÂڷ›·˜. ∏ ÁÚ·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË Ô˘ ‚Ú¤ıËΠÁÈ· Ù· ÚÔ Ù˘ ıÂڷ›·˜ ›‰· ÌÂٷ͇ Ù˘ CRP Î·È Ù˘ πL-6 (r=0,81) ˘Ô‰ËÏÒÓÂÈ Î·È ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ÙËÓ ¿ÌÂÛË
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100 90 A
80
A
IL-2 (pg/ml)
70
A
60 A
µ
50 40 30 20 10 0
¶ÚÈÓ ÙË ıÂڷ›·
3Ë Ë̤ڷ
5Ë Ë̤ڷ
10Ë Ë̤ڷ
200 180
πÓÙÂÚÏ¢ΛÓË-6 (pg/ml)
160 A 140
A
120 A 100 80
A
60 40 µ 20 0
¶ÚÈÓ ÙË ıÂڷ›·
3Ë Ë̤ڷ
5Ë Ë̤ڷ
10Ë Ë̤ڷ
3000
2500 A A sIL-2R (IU/ml)
2000
A A
1500 µ 1000
500
0 ¶ÚÈÓ ÙË ıÂڷ›·
3Ë Ë̤ڷ
5Ë Ë̤ڷ
10Ë Ë̤ڷ
∏̤Ú˜ ıÂڷ›·˜ ∂ÈÎfiÓ· 4. ∂›Â‰· Ù˘ IL-2 (4∞), Ù˘ IL-6 (4µ) Î·È ÙˆÓ sIL-2R (4°) ÚÈÓ Î·È ÙËÓ 3Ë, 5Ë Î·È 10Ë Ë̤ڷ ıÂڷ›·˜ ÛÙȘ ÔÌ¿‰Â˜ ∞ Î·È µ.
485
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¶›Ó·Î·˜ 1. ™˘ÓÙÂÏÂÛÙ¤˜ ÁÚ·ÌÌÈ΋˜ Û˘Û¯¤ÙÈÛ˘ (r) ηٿ Pearson ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜ ÁÈ· fiϘ ÙȘ ˘fi ÌÂϤÙË ·Ú·Ì¤ÙÚÔ˘˜
§Â˘Î¿ ∞ÈÌÔÛÊ·›ÚÈ· ¶ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· IL-6 IL-2 sIL-2R CRP TKE
§Â˘Î¿ ∞ÈÌÔÛÊ·›ÚÈ· -
¶ÔÏ˘ÌÔÚÊÔ‡ÚËÓ· 0,99
IL-6
IL-2
sIL-2R
CRP
TKE
0,81
0,57
0,74
0,60
0,62
0,99
-
0,80
0,50
0,75
0,60
0,64
0,81 0,57 0,74 0,60 0,62
0,80 0,52 0,75 0,60 0,64
0,45 0,66 0,81 0,39
0,45 0,57 0,41 0,40
0,66 0,57 0,51 0,58
0,81 0,41 0,51 0,69
0,39 0,40 0,58 0,69 -
Û˘Û¯¤ÙÈÛË Ù˘ CRP Î·È Ù˘ IL-6 ÛÙËÓ ÂͤÏÈÍË Ù˘ Ó¢ÌÔÓ›·˜ (19). ∂Ê' fiÛÔÓ Ë Ì¤ÙÚËÛË Ù˘ CRP ÛÙÔÓ ÔÚfi Â›Ó·È ·Ï‹ Î·È Â‡ÎÔÏË, Ë ÚˆÙ½ÓË ·˘Ù‹ ÌÔÚ› Ó· Â›Ó·È ¤Ó·˜ ¯Ú‹ÛÈÌÔ˜ ·Ú·ÎÏÈÓÈÎfi˜ ÚÔÁÓˆÛÙÈÎfi˜ ‰Â›ÎÙ˘ ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ÂͤÏÈ͢ Ù˘ Ó¢ÌÔÓ›·˜ ÙȘ ÚÒÙ˜ Ë̤Ú˜ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜ Î·È Ë IL-6 Ó· ÂÎÙÈÌ¿Ù·È ÛÙËÓ ÔÚ›·, ̤¯ÚÈ Î·È ÙËÓ 5Ë Ë̤ڷ, Û ‚·Ú‡ÙÂÚ˜ ÌÔÚʤ˜ Ïԛ̈͢. ∏ IL-6 Èı·Ófiٷٷ Â›Ó·È Ë Î˘ÙÔΛÓË Ô˘ Û˘Ó‰¤ÂÙ·È Ì ÙËÓ ÚfiÁÓˆÛË, Ú¿ÁÌ· Ô˘ ¤¯ÂÈ ÌÂÏÂÙËı› Û ·ÛıÂÓ›˜ Ì ۇӉÚÔÌÔ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜ Ù‡Ô˘ ÂÓ‹ÏÈη Û ‰Â›ÁÌ·Ù· ÔÚÔ‡ Î·È ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· (20,21). O ¯ÚfiÓÔ˜ Ô˘ ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó Ù· ›‰· ÙˆÓ ·ÓÔÛÔÏÔÁÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ ÚÈÓ Î·È ÌÂÙ¿ ÙË ıÂڷ›·, ÂÈϤ¯ıËΠ̠‚¿ÛË ÙË ÁÓÒÛË fiÙÈ ÔÈ ÚˆÙ½Ó˜ ÔÍ›·˜ Ê¿Û˘ Î·È ÔÈ ÊÏÂÁÌÔÓÒ‰ÂȘ ΢ÙÔΛÓ˜, fiˆ˜ Ë IL-6 Î·È Ë IL-2, ·˘Í¿ÓÔÓÙ·È ÛËÌ·ÓÙÈο ÛÙË ÊÏÂÁÌÔÓ‹, ÌÂÈÒÓÔÓÙ·È ÁÚ‹ÁÔÚ· Ù· ›‰¿ ÙÔ˘˜ (6,12,22) Î·È ÌÔÚ› Ó· ÚÔÛ‰ÈÔÚÈÛÙÔ‡Ó ÛÙÔÓ ÔÚfi 36-48 ÒÚ˜ ÌÂÙ¿ ÙË ÊÏÂÁÌÔÓ‹ (6,22). ™ÙË ÌÂϤÙË Ì·˜ ¤ÁÈÓ ÛÂÈÚȷ΋ ̤ÙÚËÛË ÙˆÓ ·Ú·ÁfiÓÙˆÓ, ÁÈ· Ó· ÂÎÙÈÌËı› Ô ¯ÚfiÓÔ˜ ‰È¿ÚÎÂÈ·˜ Ù˘ ÔÍ›·˜ Ïԛ̈͢ Î·È Ù˘ ·ÓÙ·fiÎÚÈÛ˘ ÛÙËÓ ÂÌÂÈÚÈ΋ ·ÓÙÈÌÈÎÚԂȷ΋ ıÂڷ›· (20,21). ™˘ÌÂÚ¿ÛÌ·Ù· ™‡Ìʈӷ Ì ٷ ·ÔÙÂϤÛÌ·Ù¿ Ì·˜, Ë CRP Î·È Ë IL-6 Â›Ó·È ÔÈ ·ÓÔÛÔÏÔÁÈÎÔ› ‰Â›ÎÙ˜ Ô˘ ÌÂÙ·‚¿ÏÏÔÓÙ·È Ôχ ÓˆÚ›˜, ‹‰Ë ·fi ÙËÓ 3Ë Î·È 5Ë Ë̤ڷ ıÂڷ›·˜ ·ÓÙ›ÛÙÔȯ·, ÂÓÒ Ë IL-2 Î·È Ô sIL-2R ÌÂÙ¿ ÙËÓ 5Ë Ë̤ڷ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜ ÛÙËÓ Ó¢ÌÔÓ›·. OÈ Ì¤ıÔ‰ÔÈ ÚÔÛ‰ÈÔÚÈÛÌÔ‡ ÙˆÓ Î˘ÙÔÎÈÓÒÓ Û‹ÌÂÚ· Â›Ó·È ·ÎfiÌË ¯ÚÔÓÔ‚fiÚ˜ Î·È ‰··ÓËÚ¤˜, Ú¿ÁÌ· Ô˘ ÂÚÈÔÚ›˙ÂÈ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘˜ Û·Ó Ì¤ıÔ‰Ô ÚÔ˘Ù›Ó·˜ ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË. ¶ÈÛÙ‡ԢÌ fiÙÈ Ë ÈÛ¯˘Ú‹ ÁÚ·ÌÌÈ΋ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ Ù˘ ∆∫∂ Î·È Ù˘ CRP, Ù˘ CRP Î·È Ù˘ IL-6 ˘Ô‰ËÏÒÓÂÈ fiÙÈ Ë ∆∫∂ Î·È Ë CRP ÌÔÚÔ‡Ó Ó· ·Ú·Ì›ÓÔ˘Ó Û·Ó ÂÍÂÙ¿ÛÂȘ ÚÔ˘Ù›Ó·˜ ÛÙË ‰È¿ÁÓˆÛË
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HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 09-01-2001 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 10-06-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ª·Ú›· ∂ÌÔÚÈ¿‰Ô˘ ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌÂ›Ô ∞.Ã.∂.¶.∞. ™Ù›ÏˆÓÔ˜ ∫˘ÚȷΛ‰Ë 1, 546 36, £ÂÛÛ·ÏÔÓ›ÎË
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ORIGINAL PAPER
∏ ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ÂÈÏËÙÈÎÒÓ ·È‰ÈÒÓ Û ÌÔÓÔıÂڷ›· Ì ‚·ÏÚÔ˚Îfi Ô͇ ∫.∂. ™Ô‡ÏË1, °.∞. ∫·Ú›Î·˜2, π. ∆˙·ÌÔ˘Ú¿ÓË1, ™. ƒÂÁÎÔ‡Ù·˜2, ™. ∆۷ΛÚ˘3
Serum biotinidase activity in epileptic children on valproic acid monotherapy K.H. Schulpis1, G.A. Karikas2, J. Tjamouranis1, S. Regoutas2, S. Tsakiris3
¶ÂÚ›ÏË„Ë: ∆Ô µ·ÏÚÔ˚Îfi Ô͇ (µ∞) Â›Ó·È ¤Ó· ·ÔÙÂÏÂÛÌ·ÙÈÎfi ·ÓÙÈÂÈÏËÙÈÎfi Ê¿ÚÌ·ÎÔ, ÙÔ˘ ÔÔ›Ô˘ Ë ¯Ú‹ÛË ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂٷ͇ ¿ÏÏˆÓ ·ÚÂÓÂÚÁÂÈÒÓ, ÂÍ¿ÓıËÌ·, ·ÒÏÂÈ· Ì·ÏÏÈÒÓ (·ÏˆÂΛ·) Î.Ï. ∏ ÎÏÈÓÈ΋ ÂÈÎfiÓ· Ù˘ Ï‹ÚÔ˘˜, fiˆ˜ Î·È Ù˘ ÌÂÚÈ΋˜, ·Ó¿ÚÎÂÈ·˜ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Â›Ó·È ·ÚfiÌÔÈ· Ì ÂΛÓË ÙˆÓ ·ÛıÂÓÒÓ Ô˘ Ï·Ì‚¿ÓÔ˘Ó µ∞. ™ÎÔfi˜, ÏÔÈfiÓ, Ù˘ ÂÚÁ·Û›·˜ ‹Ù·Ó Ó· ÚÔÛ‰ÈÔÚÈÛÙ› Ë ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘, Û ۯ¤ÛË Ù˘ Ì ÙËÓ Ë·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÛÙÔÓ ÔÚfi ÂÈÏËÙÈÎÒÓ ·È‰ÈÒÓ Û ÌÔÓÔıÂڷ›· Ì µ∞. ∂‚‰ÔÌ‹ÓÙ· ¤ÓÙ (¡=75) ·ÛıÂÓ›˜ Ì ̤ÛË ËÏÈΛ· 8,6±1,9 ¯ÚfiÓÈ·, ‰È·ÈÚ¤ıËÎ·Ó Û ÈÛ¿ÚÈı̘ ÔÌ¿‰Â˜. ™ÙËÓ ÔÌ¿‰· ∞ (¡=25) ¯ÔÚËÁ‹ıËΠ28,7±8,5 mg/Kg/24h µ∞, ÛÙËÓ ÔÌ¿‰· µ (¡=25) 41,6±4,9 mg/Kg/24h Î·È ÛÙËÓ ÔÌ¿‰· ° 54,5±5,8 mg/Kg/24h. T· ›‰· ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ÛÙÔÓ ÔÚfi ÙÔ˘˜ ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó Û 40,9±13,2, 86,25±11,5 Î·È 137±14 5 Ìg/ml ·ÓÙ›ÛÙÔȯ·. ∏ ÔÌ¿‰· ÙˆÓ Ì·ÚÙ‡ÚˆÓ ·ÔÙÂÏÔ‡ÓÙ·Ó ·fi 50 (¡=50) ˘ÁÈ‹ ·È‰È¿ ·ÚfiÌÔÈ·˜ ËÏÈΛ·˜. ∆fiÛÔ ÛÙÔ˘˜ ·ÛıÂÓ›˜, fiÛÔ Î·È ÛÙÔ˘˜ Ì¿ÚÙ˘Ú˜ ¤ÁÈÓ ˷ÙÈÎfi˜ ¤ÏÂÁ¯Ô˜, ÌÂÙÚ‹ıËÎÂ Ë ·Ì̈ӛ· ÛÙÔÓ ÔÚfi ÙÔ˘˜, fiˆ˜ Î·È Ë ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ Ì ÊıÔÚÈÔÌÂÙÚÈ΋ ̤ıÔ‰Ô, ÌÂÙ¿ 45 Ë̤Ú˜ ıÂڷ›·˜. ∆· Ë·ÙÈο ¤Ó˙˘Ì· Î·È Ë ·Ì̈ӛ· ‹Ù·Ó ·˘ÍË̤ӷ ÛÙÔ ·›Ì· ÙˆÓ ·ÛıÂÓÒÓ ÙˆÓ ÔÌ¿‰ˆÓ µ Î·È °. ∞ÓÙ›ıÂÙ·, Ë ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ‚Ú¤ıËΠÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈο ÌÂȈ̤ÓË (p<0,001) ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÔÌ¿‰·˜ µ Î·È Ù˘ ÔÌ¿‰·˜ ° (1,22±1,11, 0,97±0,07 mmol/min/L ·ÓÙ›ÛÙÔȯ·), Û˘ÁÎÚÈÓfiÌÂÓË
Abstract: Valproic acid (VPA) is an effective antiepileptic drug which is associated with dose related adverse reactions such as skin rash, hair loss (alopecia) etc. Profound as well as partial biotinidase deficiency is known to cause dermatological manifestations such as those mentioned above. Therefore, it was of interest to evaluate the serum biotinidase activity in relation to liver enzymes in patients on VPA monotherapy. Seventy five (N=75) children with recurrent seizure activity, mean age 8,6±1,9 yrs, were divided into three groups. Group A (N=25) was treated with VPA 28,7±8,5 mg/kg/24h, Group B (N=25) with 41,6±4,9 mg/Kg/24h and Group C with 54,5±5,8 mg/kg/24h. The “trough” VPA serum levels were 40,9±13,2, 86,25±11,5 and 137±14,5 Ìg/ml respectively. Fifty (N=50) healthy children were used as controls. Patients and controls underwent various laboratory examinations that included liver function tests, complete blood counts, NH3 levels etc. Biotinidase serum levels were evaluated fluorometrically. Liver enzymes as well as NH3 levels were found to be elevated in groups B and C. On the contrary, biotinidase activity was significantly statistically lower in groups B and C (1,22±1,11, 0,97±0,07 mmol/min/l resp.), as compared to controls (5,20±0,90 mmol/min/l) (p<0,001). Strong inverse correlations were observed between the levels of liver enzymes and the activity of biotinidase. Additionally, no direct
1 πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” 2 ªÔÓ¿‰· ∂Ó‰ÔÊϤ‚ÈˆÓ ¢È·Ï˘Ì¿ÙˆÓ Î·È º·ÚÌ·ÎÔÎÈÓËÙÈ΋˜ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” 3 ∂ÚÁ·ÛÙ‹ÚÈÔ ¶ÂÈÚ·Ì·ÙÈ΋˜ º˘ÛÈÔÏÔÁ›·˜ π·ÙÚÈ΋ ™¯ÔÏ‹ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ
1 Institute of Child Health “Saint Sophia” Children’s Hospital, Athens 2 Division of Intravenous Solutions and Pharmacokinetics “Saint Sophia” Children’s Hospital, Athens 3 Laboratory of Experimental Physiology University of Athens Medical School
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Paediatriki 2001;64:488-492
Ì ÂΛÓË ÙˆÓ Ì·ÚÙ‡ÚˆÓ (5,2±0,9 mmol/min/L). πÛ¯˘Ú¤˜ ·ÚÓËÙÈΤ˜ Û˘Û¯ÂÙ›ÛÂȘ ·Ú·ÙËÚ‹ıËÎ·Ó ÌÂٷ͇ Ù˘ ÛÙ¿ıÌ˘ ÙˆÓ Ë·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ Î·È Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘. ∆· ·ÔÙÂϤÛÌ·Ù· Ù˘ in vitro ÌÂϤÙ˘ Ì·˜ ¤‰ÂÈÍ·Ó fiÙÈ ÙÔ µ∞ ‰ÂÓ ÂËÚ¿˙ÂÈ ¿ÌÂÛ· ÙË ‚ÈÔÙÈÓȉ¿ÛË ·ÎfiÌË Î·È fiÙ·Ó Âˆ¿ÛÙËΠÙÔ ¤Ó˙˘ÌÔ ÛÙÔÓ ÔÚfi Ì ˘„ËÏ‹ Û˘ÁΤÓÙÚˆÛË (1,2 mM) ÙÔ˘ Ê·ÚÌ¿ÎÔ˘. ™˘ÌÂÚ·ÛÌ·ÙÈο, ÙÔ µ∞ ÂȉÚÒÓÙ·˜ ÛÙËÓ Ë·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË Ì›ˆÛË Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ ÙÔ˘ ÂÓ˙‡ÌÔ˘. °È· ÙËÓ ÂÏ·¯ÈÛÙÔÔ›ËÛË ÌÂÚÈÎÒÓ ·ÚÂÓÂÚÁÂÈÒÓ ÙÔ˘ µ∞, ı· ÌÔÚÔ‡Û ӷ ÚÔÙ·ı› Ë ¯ÔÚ‹ÁËÛË ‚ÈÔÙ›Ó˘.
inhibitory effect of VPA on biotinidase activity was demonstrated even when the enzyme was incubated in vitro with high (1,2 mM) concentrations of the drug. Our results suggest that VPA, by impairing liver function, may result in a decrease of the biotinidase activity in the serum. It is suggested that biotin supplementation could restore some of the aforementioned side effects of the drug.
§¤ÍÂȘ ÎÏÂȉȿ: ‚ÈÔÙÈÓȉ¿ÛË, ‚·ÏÚÔ˚Îfi Ô͇.
Key words: biotinidase, valproic acid.
∂ÈÛ·ÁˆÁ‹ ∆Ô µ·ÏÚÔ˚Îfi Ô͇ (µ∞) (Depakine) ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙË ıÂڷ¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÂÈÏË„›·˜ › ·ÚÎÂÙ¿ ¯ÚfiÓÈ·. OÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ó·ÊÔÚ¤˜ Ô˘ ·ÊÔÚÔ‡Ó ÛÙÔ µ∞, ÙÔ ¯·Ú·ÎÙËÚ›˙Ô˘Ó ·ÛʷϤ˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfi ÙfiÛÔ ÛÙ· ·È‰È¿ fiÛÔ Î·È ÙÔ˘˜ ÂÓ‹ÏÈΘ, Û˘ÁÎÚÈÓfiÌÂÓÔ Ì ¿ÏÏ· ·ÓÙÈÂÈÏËÙÈο Ê¿Ú̷η (1-3). OÈ ÂÚÈÛÛfiÙÂÚÔ Û˘¯Ó¿ ·Ó·ÊÂÚfiÌÂÓ˜ ·ÚÂÓ¤ÚÁÂȘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ·ÊÔÚÔ‡Ó ÛÙËÓ ·‡ÍËÛË ÙÔ˘ ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜, ÙÔÓ ÙÚfiÌÔ, ÙËÓ ÂÌÊ¿ÓÈÛË ÂÍ·Óı‹Ì·ÙÔ˜ Î·È ÙËÓ ·Ú·›ˆÛË ‹ ·fiÙˆÛË ÙˆÓ ÙÚȯÒÓ Ù˘ ÎÂÊ·Ï‹˜ (·ÏˆÂΛ·). ∏ ·ÓˆÙ¤Úˆ Û˘Ìو̷ÙÔÏÔÁ›· ›Ù ‚ÂÏÙÈÒÓÂÙ·È, ›Ù ÂÍ·Ê·Ó›˙ÂÙ·È Ì ÙË Ì›ˆÛË Ù˘ ‰fiÛ˘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ‹ ÙË ‰È·ÎÔ‹ ÙÔ˘ (2,3). ∆· ÂÚÈÁÚ·ÊfiÌÂÓ· ÙÔÍÈο Î·È ÔÏϤ˜ ÊÔÚ¤˜ ı·Ó·ÙËÊfiÚ· Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ‹·Ú ı· Ú¤ÂÈ Ó· ıˆÚËıÔ‡Ó ÂÚÈÛÛfiÙÂÚÔ È‰ÈÔÛ˘ÁÎÚ·Ûȷο, ·Ú¿ Û¯ÂÙÈ˙fiÌÂÓ· Ì ÙË ‰fiÛË ÙÔ˘ µ∞ (1,3). ∏ ‚ÈÔÙÈÓȉ¿ÛË Â›Ó·È ¤Ó· ¤Ó˙˘ÌÔ Ô˘ ·Ó·Î˘ÎÏÒÓÂÈ ÙË ‚ÈÔÙ›ÓË, ·ÏÏ¿ Î·È ¿ÏÏ· ÌÈÎÚ¿ ÂÙ›‰È· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙȘ ‚ÈÔÙÈÓÔ-ÂÍ·ÚÙÒÌÂÓ˜ ηڂÔÍ˘Ï¿Û˜. ∆Ô ¤Ó˙˘ÌÔ ÂÈÙÚ¤ÂÈ ÛÙËÓ ÂχıÂÚË ‚ÈÔÙ›ÓË Ó· ·ӷ΢ÎÏÒÓÂÙ·È ·ÏÏ¿ Î·È Ó· ÙËÓ ·ÔÛ¿ fiÙ·Ó Â›Ó·È Û˘Ó‰Â‰Â̤ÓË Ì ÙȘ ÚˆÙ½Ó˜ Ù˘ ÙÚÔÊ‹˜. ∏ ‚ÈÔÎ˘Ù›ÓË, ¤Ó· Â-Ï˘ÛÈÓÔ-·ÌÈÓÔ-·Ì›‰ÈÔ Ù˘ ‚ÈÔÙ›Ó˘, ÚÔ¤Ú¯ÂÙ·È Èı·ÓfiÓ ·fi ÙËÓ ÚˆÙÂfiÏ˘ÛË ÙˆÓ ÂÍ·ÚÙˆÌ¤ÓˆÓ ·fi ÙË ‚ÈÔÙ›ÓË Î·Ú‚ÔÍ˘Ï·ÛÒÓ (4). ∏ Û˘Ìو̷ÙÔÏÔÁ›·, ÙfiÛÔ ÛÙËÓ ÔÏÈ΋ fiÛÔ Î·È ÛÙË ÌÂÚÈ΋ ·Ó¿ÚÎÂÈ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘, Û˘Óԉ‡ÂÙ·È ·fi ÎÏÈÓÈο ‰ÂÚÌ·ÙÔÏÔÁÈο Â˘Ú‹Ì·Ù·, ·ÚfiÌÔÈ· Ì ÂΛӷ Ù˘ ¤ÏÏÂȄ˘ Ù˘ ‚ÈÔÙ›Ó˘, Èı·ÓfiÓ ·fi ‰È·Ù·Ú·Á̤ÓË ÂÓÙÂÚÈ΋ ·ÔÚÚfiÊËÛË, ΢ÙÙ·ÚÈ΋ ¯Ú‹ÛË, ÓÂÊÚÈ΋ ·Ô‚ÔÏ‹ Ù˘ ‚ÈÔÙ›Ó˘, ηıÒ˜ ›Û˘ ·fi Ó¢ÚÔÏÔÁÈΤ˜ ÂΉËÏÒÛÂȘ, fiˆ˜ ÔÈ Û·ÛÌÔ›, ÛÙÔ Ì¤ÙÚÔ Ô˘ Â›Ó·È ÂÏ¿-
¯ÈÛÙË Ë ÌÂÙ·‚ÔÏÈο ÂÎÌÂÙ·ÏχÛÈÌË ‚ÈÔÙ›ÓË (5). ŒÙÛÈ, Ë ·Ú·Ù‹ÚËÛË fiÙÈ Ë ÂÓ‰ÔÁÂÓ‹˜ ÌÂÙ·‚ÔÏÈ΋ ·Ó¿ÚÎÂÈ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ·ÚÔ˘ÛÈ¿˙ÂÈ Û˘Ìو̷ÙÔÏÔÁ›· ÛÙÔ ‰¤ÚÌ· Î·È ÙÔ ÙÚȯˆÙfi Ù˘ ÎÂÊ·Ï‹˜ ·ÚfiÌÔÈ· Ì ÂΛÓË Ô˘ ÂÓÙÔ›˙ÂÙ·È Û ·ÛıÂÓ›˜ Û ıÂڷ›· Ì µ∞, Ì·˜ Ô‰‹ÁËÛ ÛÙË ÌÂϤÙË Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ Û ۯ¤ÛË Ì ÙȘ ÙÈ̤˜ ÙˆÓ Ë·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ ÛÙÔÓ ÔÚfi ÂÈÏËÙÈÎÒÓ ·È‰ÈÒÓ Û ıÂڷ›· Ì µ∞. ¶·Ú¿ÏÏËÏ·, ÌÂÏÂÙ‹ıËÎÂ Ë ¿ÌÂÛË Â›‰Ú·ÛË ÙÔ˘ µ∞ ÛÙË ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ Û in vitro ›ڷ̷. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∂‚‰ÔÌ‹ÓÙ· ¤ÓÙ (¡=75) ·ÛıÂÓ›˜, Ì ̤ÛË ËÏÈΛ· Ù· 8,6±1,9 ¯ÚfiÓÈ· Û ÌÔÓÔıÂڷ›· Ì µ∞, ‰È·¯ˆÚ›ÛÙËÎ·Ó Û 3 ÈÛ¿ÚÈı̘ ÔÌ¿‰Â˜. ¶ÂÓ‹ÓÙ· (¡=50) ˘ÁÈ‹, ·ÚfiÌÔÈ·˜ ËÏÈΛ·˜, ·È‰È¿ ·ÔÙ¤ÏÂÛ·Ó ÙËÓ ÔÌ¿‰· ÂϤÁ¯Ô˘ (Ì¿ÚÙ˘Ú˜). ∏ ÔÌ¿‰· ∞ ÙˆÓ ·ÛıÂÓÒÓ (¡=25) ÂÏ¿Ì‚·Ó 28,7±8,5 mg/Kg/24h BA, Ë ÔÌ¿‰· µ (¡=25) ÂÏ¿Ì‚·Ó 41,6±4,9mg/Kg/24h µ∞ Î·È Ë ÔÌ¿‰· ° (¡=25) 64,5±5,8 mg/Kg/24h. ŸÏ· Ù· ˘fi ÌÔÓÔıÂڷ›· ·È‰È¿ ˘¤ÊÂÚ·Ó ·fi Û˘¯Ó¿ ˘ÔÙÚÔÈ¿˙Ô˘Û˜ ÂÈÏËÙÈΤ˜ ÎÚ›ÛÂȘ (2-3/‚‰ÔÌ¿‰·). ™Â fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ ÂϤÁ¯ıËÎ·Ó Ù· Ë·ÙÈο ¤Ó˙˘Ì· (SGOT, SGPT, ÁGt, ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË), Ù· ›‰· ¡∏3 Î·È Ù· ÔÏÈο Ï¢ÎÒÌ·Ù·, ÚÈÓ ÙË ÌÔÓÔıÂڷ›· ·ÏÏ¿ Î·È ÌÂÙ¿ ·fi 45 Ë̤Ú˜ ıÂڷ›·˜ Ì µ∞. ∂›Û˘, ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó Ù· ›‰· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ÌÂÙ¿ ÙÔ ›‰ÈÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ıÂڷ›·˜, Ì ·ÓÔÛÔÊıÔÚÈÛÌÔÌÂÙÚÈ΋ ̤ıÔ‰Ô (6). ∏ ÌÂϤÙË Ù˘ in vitro ›‰Ú·Û˘ ÙÔ˘ µ∞ ÛÙËÓ ÂÓ˙˘ÌÈ΋ ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ¤ÁÈÓ ÌÂÙ¿ ·fi ÂÒ·ÛË ÔÚÔ‡ ÁÓˆÛÙ‹˜ ‰Ú·ÛÙÈÎfiÙËÙ·˜ ÙÔ˘ ÂÓ˙‡ÌÔ˘ Ì ‰È¿ÊÔÚ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘. ¶ÚfiÙ˘Ë Ô˘Û›· µ∞ ÚÔÛʤÚıËΠ¢ÁÂÓÈο ·fi ÙËÓ ÂÙ·ÈÚ›· Sanofi Ltd. T· ›‰· ÙÔ˘ µ∞ ÛÙÔÓ ÔÚfi ÚÔÛ‰ÈÔÚ›ÛÙËÎ·Ó Ì ÙÔ Û‡ÛÙËÌ· (∆DX) Ù˘ ABBOTTR (7). °È· ÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÔÈ ‰ÔÎÈ̷ۛ˜ Student's Î·È Spearman ·fi ÙÔ ÛÙ·ÙÈÛÙÈÎfi ·Î¤ÙÔ SPSS 7,5 Ì ∏/À.
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¶·È‰È·ÙÚÈ΋ 2001;64:488-492
Paediatriki 2001;64:488-492
∞ÔÙÂϤÛÌ·Ù· ∆· ÈÔ Û˘¯Ó¿ ÎÏÈÓÈο Â˘Ú‹Ì·Ù· ÌÂÙ¿ ÙË ıÂڷ¢ÙÈ΋ ·ÁˆÁ‹ Ì µ∞ Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó ÂÛÙÈ¿ÛÙËÎ·Ó Û ÁÂÓÈÎÂ˘Ì¤ÓÔ ÂÍ¿ÓıËÌ· (6/25 ÛÙËÓ ÔÌ¿‰· µ Î·È 7/25 ÛÙËÓ ÔÌ¿‰· °) Î·È Û ·ÒÏÂÈ· ÙˆÓ ÙÚȯÒÓ Ù˘ ÎÂÊ·Ï‹˜ (3/25 Î·È 6/25 ÛÙȘ ÔÌ¿‰Â˜ µ Î·È °). ∏ Û˘Ìو̷ÙÔÏÔÁ›· ·˘Ù‹ ·Ô˘Û›·˙ ÛÙËÓ ÔÌ¿‰· ∞ (¶›Ó·Î·˜ 1) ∆· Ë·ÙÈο ¤Ó˙˘Ì· Î·È Ù· ›‰· Ù˘ ¡∏3 ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈο ·˘ÍË̤ӷ ÛÙȘ ÔÌ¿‰Â˜ µ Î·È ° (¶›Ó·Î·˜ 2). ∆· ›‰· ÙÔ˘ µ∞ ÛÙÔÓ ÔÚfi, Û‡Ìʈӷ Ì ÙË Ê·ÚÌ·ÎÔÎÈÓËÙÈ΋ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘, ¯·Ú·ÎÙËÚ›ÛÙËÎ·Ó ˆ˜ “˘ÔıÂڷ¢ÙÈο” ÛÙËÓ ÔÌ¿‰· ∞ (40,9±13,2 Ìg/ml), ˆ˜ “ıÂڷ¢ÙÈο” (86,25±11,5 Ìg/ml) ÛÙËÓ ÔÌ¿‰· µ Î·È ˆ˜ “ÙÔÍÈο” (137±14,5 Ìg/ml) ÛÙËÓ ÔÌ¿‰· °. Ÿˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ ¶›Ó·Î· 3, Ë ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈο ÌÂȈ̤ÓË ÛÙȘ ÔÌ¿‰Â˜ µ Î·È °, Û ۇÁÎÚÈÛË Ì ÂΛÓË ÙˆÓ Ì·ÚÙ‡ÚˆÓ Î·È Ù˘ ÔÌ¿‰·˜ ∞. ∞ÚÓËÙÈΤ˜ ›Û˘, ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ Û˘Û¯ÂÙ›ÛÂȘ ·Ú·ÙËÚ‹ıËÎ·Ó ÌÂٷ͇ Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘, ÙˆÓ Ë·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ Î·È ÙˆÓ ÂȤ‰ˆÓ ÙÔ˘ µ∞ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ (¶›Ó·Î·˜ 4). ∫·Ì›· ·fi ÙȘ ¯ÚËÛÈÌÔÔÈÔ‡ÌÂÓ˜ Û˘ÁÎÂÓÙÚÒ-
¶›Ó·Î·˜ 1. ∫ÏÈÓÈο Â˘Ú‹Ì·Ù· ·ÛıÂÓÒÓ ÌÂÙ¿ ·fi ıÂڷ¢ÙÈ΋ ·ÁˆÁ‹ Ì µ∞ Û ۯ¤ÛË Ì ÙË ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ÙÔ˘˜ ∫ÏÈÓÈο OÌ¿‰· ∞ OÌ¿‰· µ Â˘Ú‹Ì·Ù· (¡=25) (¡=25) °ÂÓ. ÂÍ¿ÓıËÌ· 6/25 ∞ÒÏÂÈ· ÙÚȯÒÓ 3/25 µÈÔÙÈÓȉ¿ÛË (mmol/min/l) 3,95±1,08a 1,1±1,0b
OÌ¿‰· ° ª¿ÚÙ˘Ú˜ (¡=25) (¡=50) 7/25 6/25 0,9±0,9c 5,2±0,9d
OÈ ÙÈ̤˜ ÂÎÊÚ¿˙ÔÓÙ·È: ª¤ÛË ÙÈÌ‹ ± ™Ù·ıÂÚ‹ ·fiÎÏÈÛË a/b, a/c, a/d, b/d, c/d = p<0,001 b/c = NS (ÌË ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ÙÈÌ‹)
ÛÂȘ ÙÔ˘ µ∞ ‰ÂÓ ¤‰ÂÈÍ ¿ÌÂÛÔ ·ÔÙ¤ÏÂÛÌ· ÛÙË ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘, fiÙ·Ó Âˆ¿ÛÙËΠ̠ÙÔ µ∞ (ÂÈÚ·Ì·ÙÈ΋ ÌÂϤÙË in vitro) (¶›Ó·Î·˜ 5). ™˘˙‹ÙËÛË ™‡Ìʈӷ Ì ÙÔ ÁÂÓÈÎÒ˜ ·Ô‰ÂÎÙfi ıÂڷ¢ÙÈÎfi Û¯‹Ì·, ÙÔ µ∞ ¯ÔÚËÁÂ›Ù·È ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ Û ·Ú¯È΋ ‰fiÛË 15 mg/Kg/24h Ì ÛÙ·‰È·Î‹ ·‡ÍËÛË ·Ó¿ 1 ‚‰ÔÌ¿‰· ηٿ 5-10 mg, ¤ˆ˜ fiÙÔ˘ ÛÙ·Ì·Ù‹ÛÔ˘Ó ÔÈ Û·ÛÌÔ› ‹ ÂÌÊ·ÓÈÛÙÔ‡Ó ÔÈ ·ÚÂÓ¤ÚÁÂȘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘. ∏ ÌÂÁ·Ï‡ÙÂÚË Û˘ÓÈÛÙÒÌÂÓË ‰fiÛË ÌÔÚ› Ó· ·Ó¤ÏıÂÈ Û 60 mg/Kg/24h (3), fiˆ˜ ÛÙËÓ ÂÚ›ÙˆÛË ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÔÌ¿‰·˜ °. ™‡Ìʈӷ Ì ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ, ÌÂٷ͇ ÙˆÓ ·ÚÂÓÂÚÁÂÈÒÓ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘, fiÙ·Ó ÙÔ‡ÙÔ ¯ÔÚËÁÂ›Ù·È Û ıÂڷ¢ÙÈΤ˜ ‰fiÛÂȘ, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› Ë ·ÏˆÂΛ· (13/134), ÙÔ ‰ÂÚÌ·ÙÈÎfi ÂÍ¿ÓıËÌ· (15/134) Î·È Ô ÎÓËÛÌfi˜ (10/71) (3). ŸÙ·Ó Ë ¯ÔÚËÁÔ‡ÌÂÓË ‰fiÛË ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ·˘Í¿ÓÂÙ·È, ·˘Í¿ÓÂÈ Î·È Ë Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ÙˆÓ ÚÔ·Ó·ÊÂÚÔÌ¤ÓˆÓ Û˘Ìو̿وÓ, fiˆ˜ Ë ·ÏˆÂΛ· (23/131), ÙÔ ÂÍ¿ÓıËÌ· (30/131) Î·È Ô ÎÓËÛÌfi˜ (21/131). ∆· ·Ú·¿Óˆ Â˘Ú‹Ì·Ù· Û˘ÌʈÓÔ‡Ó Ì ·˘Ù¿ Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘. OÈ ÌÈÎÚ¤˜ ·˘Í‹ÛÂȘ ÙˆÓ ÙÚ·ÓÛ·ÌÈÓ·ÛÒÓ Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘ Ì·˜, Û˘Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË ¯ÔÚËÁÔ‡ÌÂÓË ‰fiÛË ÙÔ˘ Ê·ÚÌ¿ÎÔ˘. ∆Ô ›‰ÈÔ Û˘Ì‚·›ÓÂÈ Î·È Ì ÙËÓ ·‡ÍËÛË Ù˘ ·Ì̈ӛ·˜ (3). ∏ Ì›ˆÛË Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ÙÔ˘˜ ‰ÂÓ Ê·›ÓÂÙ·È Ó· Û˘Û¯ÂÙ›˙ÂÙ·È ¿ÌÂÛ· Ì ÙË ÛÔ‚·ÚfiÙËÙ· Ù˘ ÂÌÊ¿ÓÈÛ˘ ÙˆÓ ‰ÂÚÌ·ÙÈÎÒÓ ÙÔ˘˜ Û˘ÌÙˆÌ¿ÙˆÓ (¶›Ó·Î·˜ 1). ™‡Ìʈӷ ¿ÏÈ Ì ÙË ‚È‚ÏÈÔÁÚ·Ê›·, ÔÈ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ·fi ÙÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ· ÂÌÊ·Ó›˙ÔÓÙ·È Û ÂΛÓÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ϋÚË ·Ó¿ÚÎÂÈ· ‚ÈÔÙÈÓȉ¿Û˘. ∏ ·Ô˘Û›· ·˘ÙÔ‡ ÙÔ˘ Ù‡Ô˘ Û˘Ìو̷ÙÔÏÔÁ›·˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜, Èı·ÓfiÓ Ó· ÔÊ›ÏÂÙ·È Û ÌÂÚÈ΋ ·Ó¿ÚÎÂÈ· ÙÔ˘ ÂÓ˙‡ÌÔ˘, Ô˘ ¢ı‡ÓÂÙ·È ÁÈ· ÙȘ ‰ÂÚÌ·ÙÈΤ˜ ÎÏÈÓÈΤ˜
¶›Ó·Î·˜ 2. ∏·ÙÈÎfi˜ ¤ÏÂÁ¯Ô˜, ¡∏3 Ê·ÚÌ·ÎÔÎÈÓËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ µ∞ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Î·È ÙˆÓ Ì·ÚÙ‡ÚˆÓ
SGOT, U/L SGPT, U/L ∞ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË U/L ÁGt U/L NH3 Á% OÏ. Ï¢ÎÒÌ·Ù· (g/l) µ∞ Ìg/ml
OÌ¿‰· ∞ (N=25) 24,6±8,0a 20,4±8,1a 63,5±21a 10,5±4a 34,0±7,8a 6,7±0,3a 40,9±15,2a
a/b = b/c= p<0,01, a/c = p<0,001 a/a = a/d = d/d = NS (ÌË ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ÙÈÌ‹)
490
OÌ¿‰· B (N=25) 39,8±11,4b 39,6±15,7b 86,9±27b 17,3±7,5b 76,8±8,5b 5,93±0,6d 86,2±13,5b
OÌ¿‰· ° (N=25) 44,6±12c 43,9±16c 90,9±28c 19,6±8,5c 98±9,5c 5,8±0,5d 137,9±14,5c
ª¿ÚÙ˘Ú˜ (N=50) 23,0±9,0a 21,0±8,9a 60±15a 12,0±4,0a 28±9,0a 6,6±0,4a -
¶·È‰È·ÙÚÈ΋ 2001;64:488-492
Paediatriki 2001;64:488-492
¶›Ó·Î·˜ 3. ¢Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÔÚÔ‡ ÛÙȘ ÔÌ¿‰Â˜ ÙˆÓ ·ÛıÂÓÒÓ Î·È ÙˆÓ Ì·ÚÙ‡ÚˆÓ OÌ¿‰Â˜ A (¡=25) µ (¡=25) °(¡= 25) ª¿ÚÙ˘Ú˜ (N=50)
¢Ú·ÛÙÈÎfiÙËÙ· ‚ÈÔÙÈÓȉ¿Û˘ (mmol/min/L) 3,95±1,08a 1,22±1,11b 0,97±0,07c 5,20±0,90d
OÈ ÙÈ̤˜ ÂÎÊÚ¿˙ÔÓÙ·È: ª¤ÛË ÙÈÌ‹ ± ™Ù·ıÂÚ‹ ·fiÎÏÈÛË a/b p<0,01, a/d = b/c = ¡S (ÌË ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ÙÈÌ‹) a/c = b/d p<0,001, c/d p<0,0001
¶›Ó·Î·˜ 4. ™˘Û¯ÂÙ›ÛÂȘ ÌÂٷ͇ ‰Ú·ÛÙÈÎfiÙËÙ·˜ ‚ÈÔÙÈÓȉ¿Û˘, Ë·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ, ÔÏÈÎÒÓ ÏÂ˘ÎˆÌ¿ÙˆÓ Î·È ÙˆÓ ÂȤ‰ˆÓ ÙÔ˘ µ∞ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ OÌ¿‰Â˜ SGOT
SGPT
∞ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË
ÁGt
µA
OÏ. ¶ÚˆÙ½ÓË
A B C ∞ B C ∞ µ C A B C A B C A B C
¢Ú·ÛÙÈÎfiÙËÙ· ‚ÈÔÙÈÓȉ¿Û˘ -0,59* -0,62** -0,61*** -0,59* -0,59* -0,64** -0,48 -0,50 -0,52 -0,60* -0,64** -0,69*** -0,62** -0,61** -0,66*** 0,48 0,34 0,31
* p<0,05 ** p<0,01 *** p<0,001
¶›Ó·Î·˜ 5. ∏ ›‰Ú·ÛË ÙÔ˘ µ∞ ÛÙËÓ ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ (in vitro) µ∞ Û˘ÁÎÂÓÙÚÒÛÂȘ (mM) 0,3 (60 mg/100 ml) 0,6 (120 mg/100 ml) 0,9 (180 mg/100 ml) 1,2 (240 mg/100 ml)
¢Ú·ÛÙÈÎfiÙËÙ· (mmol/min/l) 4,3 4,3 5,8 4,8 4,6
¢Ú·ÛÙÈÎfiÙËÙ· (%) ‚ÈÔÙÈÓȉ¿Û˘ 100 100 134 111 106
OÈ ÙÈ̤˜ ÂÎÊÚ¿˙ÔÓÙ·È: ª¤ÛË ÙÈÌ‹ ± ™Ù·ıÂÚ‹ ·fiÎÏÈÛË
ÂΉËÏÒÛÂȘ (8). ∏ ÌÂÁ·Ï‡ÙÂÚË ‰Ú·ÛÙÈÎfiÙËÙ· ÙÔ˘ ÂÓ˙‡ÌÔ˘ ¤¯ÂÈ ·Ú·ÙËÚËı› ÛÙÔ ‹·Ú Î·È ÙÔÓ ÔÚfi (9) Î·È ÛËÌ·ÓÙÈο ÌÈÎÚfiÙÂÚË ÛÙÔÓ ÂÁΤʷÏÔ (10). O Pispa (11) ·Ú·Ù‹ÚËÛ ÌÂȈ̤ÓË ‰Ú·ÛÙÈÎfiÙËÙ·
Û ÔÛÔÛÙfi (-30%) Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ÌÂÚÈÎÒ˜ Ë·ÙÂÎÙÔÌËı¤ÓÙˆÓ ÔÓÙÈÎÒÓ, ·Ô‰ÂÈÎÓ‡ÔÓÙ·˜ fiÙÈ ÙÔ ‹·Ú Â›Ó·È Ô ÙfiÔ˜ ·Ú·ÁˆÁ‹˜ ÙÔ˘ ÂÓ˙‡ÌÔ˘. ™Â Ì›· ÚfiÛÊ·ÙË ÌÂϤÙË (12), Ù· ›‰· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ‚Ú¤ıËÎ·Ó ÌÂȈ̤ӷ ÛÙÔ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi Û ÂÈÏËÙÈÎÔ‡˜ ·ÛıÂÓ›˜ Û˘ÁÎÚÈÓfiÌÂÓ· Ì ÂΛӷ ÙˆÓ Ì·ÚÙ‡ÚˆÓ, Ë Û˘ÁΤÓÙÚˆÛ‹ Ù˘, fï˜, ÛÙÔÓ ÔÚfi, ÌfiÏȘ ‰È¤ÊÂÚ ÛÙ·ÙÈÛÙÈο Û ۯ¤ÛË Ì ÂΛÓË ÙˆÓ ˘ÁÈÒÓ. ™Â ÚÔËÁÔ‡ÌÂÓË ÂÚÁ·Û›· Ì·˜ (13), ÙÔ Ê¿ÚÌ·ÎÔ Ô˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙË ıÂڷ›· Ù˘ ΢ÛÙÈ΋˜ ·ÎÌ‹˜, Ë ÈÛÔÙÚÂÙÈÓÔ˝ÓË, ‚Ú¤ıËΠfiÙÈ ÚÔηÏ› ÌÂÚÈ΋ ·Ó·ÛÙÔÏ‹ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘, Ô˘ Èı·ÓfiÓ ÔÊ›ÏÂÙ·È ÛÙÔ˘˜ ÈÛÔÌÂÚ›˜ ÌÂÙ·‚Ôϛ٘ Ù˘ ÈÛÔÙÚÂÙÈÓÔ˝Ó˘ Ô˘ ‰ÚÔ˘Ó Â›Ù ¿ÌÂÛ· ›Ù ¤ÌÌÂÛ· ÛÙÔ ‹·Ú, ÌÂ Û˘Ó¤ÂÈ· ÙË Ì›ˆÛË ÙÂÏÈο Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ ÙÔ˘ ÂÓ˙‡ÌÔ˘. ÕÏÏ· ÚfiÛÊ·Ù· ÛÙÔȯ›· ·¤‰ÂÈÍ·Ó fiÙÈ Ë ‰Ú·ÛÙÈÎfiÙËÙ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ·ÛıÂÓÒÓ Ì ¯ÚfiÓÈ· Ë·ÙÈ΋ ÓfiÛÔ Â›Ó·È ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚË Û˘ÁÎÚÈÓfiÌÂÓË Ì ÂΛÓË ÙˆÓ ˘ÁÈÒÓ (14). £· ÌÔÚÔ‡ÛÂ, ÏÔÈfiÓ, Ó· ˘ÔÙÂı› fiÙÈ ÙÔ µ∞, ÂËÚ¿˙ÔÓÙ·˜ ÙËÓ Ë·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· (3), Ô‰ËÁ› ÛÙË Ì›ˆÛË Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ ÙÔ˘ ÂÓ˙‡ÌÔ˘ ÛÙÔÓ ÔÚfi (14). ∆Ô‡ÙÔ ÂÓÈÛ¯‡ÂÙ·È ·fi ÙÔ fiÙÈ Ë in vitro ÌÂϤÙË Ì·˜ ·¤‰ÂÈÍ fiÙÈ Ë ÂÒ·ÛË ÙÔ˘ µ∞ Ì ÔÚfi ÁÓˆÛÙ‹˜ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘, ‰ÂÓ ÌÂÙ¤‚·Ï ÙËÓ ‰Ú·ÛÙÈÎfiÙËÙ· ÙÔ˘ ÂÓ˙‡ÌÔ˘, ÂÓÈÛ¯‡ÔÓÙ·˜ ÂÚ·ÈÙ¤Úˆ ÙËÓ ¿Ô„Ë fiÙÈ Â›Ó·È ¤ÌÌÂÛË Èı·Ó¿ Ë ·ÈÙ›· Ì›ˆÛ˘ Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Ì·˜. ™˘ÌÂÚ¿ÛÌ·Ù· H ·Ú·ÙËÚÔ‡ÌÂÓË Ì›ˆÛË Ù˘ ‰Ú·ÛÙÈÎfiÙËÙ·˜ Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ Â›Ó·È ‰ÔÛÔ-ÂÍ·ÚÙÒÌÂÓË Î·È Èı·Ófiٷٷ ÔÊ›ÏÂÙ·È ÛÙËÓ Ë·ÙÈ΋ ÙÔÍÈ΋ ‰Ú¿ÛË ÙÔ˘ µ∞ ‹ ·ÎfiÌË Î·È Û οÔÈÔ ¿ÏÏÔ ¤ÌÌÂÛÔ Ì˯·ÓÈÛÌfi Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ٷ ¯·ÌËÏ¿ ›‰· Ù˘ ‚ÈÔÙ›Ó˘ ÛÙÔÓ ÔÚfi, ÙÔ Ê˘ÛÈÎfi ˘fiÛÙڈ̷ ÙÔ˘ ÂÓ˙‡ÌÔ˘. OÈ ‰ÂÚÌ·ÙÈΤ˜ ÂΉËÏÒÛÂȘ (·ÏˆÂΛ·˜, ÂÍ¿ÓıËÌ·) Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙÔ˘˜ ·ÛıÂÓ›˜ ÙˆÓ ÔÌ¿‰ˆÓ µ Î·È °, ı· ÌÔÚÔ‡Û·Ó Ó· ·Ô‰ÔıÔ‡Ó Û ·Ú¯Èο Û˘ÌÙÒÌ·Ù· ÌÂÚÈ΋˜ ·Ó¿ÚÎÂÈ·˜ ‚ÈÔÙÈÓȉ¿Û˘. ¶·ÚfiÌÔÈ· Â˘Ú‹Ì·Ù· ·Ó·Ê¤ÚÔÓÙ·È Û ·ÛıÂÓ›˜ Ì ÂÁÁÂÓ‹ ÌÂÚÈ΋ ·Ó¿ÚÎÂÈ· ÙÔ˘ ÂÓ˙‡ÌÔ˘. ™ÙÔ Ì¤ÙÚÔ Ô˘ Ë ÌÂÚÈ΋ ·Ó¿ÚÎÂÈ· Ù˘ ‚ÈÔÙÈÓȉ¿Û˘ ·ÓÙÈÌÂÙˆ›˙ÂÙ·È ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË Ì ÙË ¯ÔÚ‹ÁËÛË ‚ÈÔÙ›Ó˘, ı· ÌÔÚÔ‡ÛÂ Ë ¯ÔÚ‹ÁËÛË Ù˘ ÙÂÏÂ˘Ù·›·˜ Û ·ÛıÂÓ›˜ Ô˘ Ï·Ì‚¿ÓÔ˘Ó µ∞ Ó· Û˘Ì‚¿ÏÂÈ ÛÙËÓ ÂÏ·¯ÈÛÙÔÔ›ËÛË ÙˆÓ ÚÔÂÚ¯fiÌÂÓˆÓ ·fi ÙÔ µ∞ ‰ÂÚÌ·ÙÈÎÒÓ ÂΉËÏÒÛˆÓ.
491
¶·È‰È·ÙÚÈ΋ 2001;64:488-492
Paediatriki 2001;64:488-492
∂˘¯·ÚÈÛٛ˜ OÈ Û˘ÁÁÚ·Ê›˜ ıˆÚÔ‡Ó ˘Ô¯Ú¤ˆÛ‹ ÙÔ˘˜ Ó· ¢¯·ÚÈÛÙ‹ÛÔ˘Ó ÙȘ Î.Î. ∫. ∆·ÛÛÔÔ‡ÏÔ˘ Î·È ÕÓÓ· ™Ù·Ì¿ÙË ÁÈ· ÙËÓ ÂÈÌÂÏË̤ÓË ‰·ÎÙ˘ÏÔÁÚ¿ÊËÛË ·˘Ù‹˜ Ù˘ ÂÚÁ·Û›·˜.
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HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 17-01-2001 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 24-07-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: KÏÂÔ¿ÙÚ· ∂. ™Ô‡ÏË πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, 115 27, ∞ı‹Ó·
¶ÂÚ›ÏË„Ë ¿ÚıÚÔ˘ ·fi ÙË µÈ‚ÏÈÔÁÚ·Ê›· ∏ ›ÌÔÓË Û˘ÚÚ›ÙÔ˘Û· ·Ó·ÓÔ‹ Û Ôχ ÌÈÎÚ¿ ·È‰È¿ Û¯ÂÙ›˙ÂÙ·È Ì ÊÏÂÁÌÔÓ‹ ÙˆÓ Î·ÙÒÙÂÚˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ1 ™ÎÔfi˜: ¶·Ú¿ ÙȘ ÚÔfi‰Ô˘˜ ÛÙËÓ Î·Ù·ÓfiËÛË Ù˘ ·ıÔÊ˘ÛÈÔÏÔÁ›·˜ ÙÔ˘ ¿ÛıÌ·ÙÔ˜, Ë ÓÔÛËÚfiÙËÙ· Î·È Ë ıÓËÙfiÙËÙ· ÛÙ· ·È‰È¿ Û˘Ó¯›˙ÂÈ Ó· ·˘Í¿ÓÂÈ. §›Á· Â›Ó·È ÁÓˆÛÙ¿ Û¯ÂÙÈο Ì ÙËÓ ¤Ó·ÚÍË Î·È ÙË ¯ÚÔÓÈfiÙËÙ· Ù˘ ÊÏÂÁÌÔÓ‹˜ Ô˘ ¤¯ÂÈ Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙÔ ¿ÛıÌ· ÛÙÔ Ó·Úfi ·˘Ùfi ÏËı˘ÛÌfi. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ªÂÏÂÙ‹Û·Ì 20 ·È‰È¿ ÌÂ Û˘ÚÈÁÌfi (wheezing) ̤Û˘ ËÏÈΛ·˜ 14,9 ÌËÓÒÓ Ì 2 ÙÔ˘Ï¿¯ÈÛÙÔÓ ÂÂÈÛfi‰È· Û˘ÚÈÁÌÔ‡ ‹ ·Ú·ÙÂٷ̤ÓÔ˘ Û˘ÚÈÁÌÔ‡ ¿Óˆ ÙˆÓ 2 ÌËÓÒÓ, Û ‰È¿ÛÙËÌ· 6 ÌËÓÒÓ, Ì ‚ÚÔÁ¯ÔÛÎfiËÛË Î·È ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· (BAL). ™˘ÁÎÚ›ÛÂȘ ¤ÁÈÓ·Ó Ì 6 Ê˘ÛÈÔÏÔÁÈο ·È‰È¿ (̤Û˘ ËÏÈΛ·˜ 23,3 ÌËÓÒÓ) Ô˘ ‹Ú·Ó ÁÂÓÈ΋ Ó¿ÚΈÛË ÁÈ· ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓË ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË. ¶ÚÔÛ‰ÈÔÚ›ÛÙËÎÂ Ô ·ÚÈıÌfi˜ Î·È Ô Ù‡Ô˜ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎÔ‡ ÂÎÎڛ̷ÙÔ˜. ∆· ÂÈÎÔÛ·ÓÔÂȉ‹, Ù· Ï¢ÎÔÙÚȤÓÈ· LTB4, LTE4, Ë ÚÔÛÙ·ÁÏ·Ó‰›ÓËPGE2 Î·È ÙÔ 15-˘‰ÚÔ͢-ÂÈÎÔÛ·ÓÔÙÂÙÚ·ÓÔ˚Îfi Ô͇ Î·È ÔÈ ‰È·‚È‚·ÛÙ¤˜ ÙˆÓ Ì·ÛÙÔ΢ÙÙ¿ÚˆÓ ‚-ÙÚ˘Ù¿ÛË Î·È PGD2 ÂÎÙÈÌ‹ıËÎ·Ó Ì ·ÓÔÛÔÂÓ˙˘Ì·ÙÈ΋ ̤ıÔ‰Ô. ∞ÔÙÂϤÛÌ·Ù·: ∆· ·È‰È¿ ÌÂ Û˘ÚÈÁÌfi ›¯·Ó ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÙÔ˘ ÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ Î˘Ù¿ÚˆÓ ÙÔ˘ BAL/ml ηıÒ˜ Î·È ÙˆÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ, ÙÔ˘ ÏfiÁÔ˘ Ì·ÎÚÔÊ¿ÁˆÓ/ÌÔÓÔ΢ÙÙ¿ÚˆÓ, ÙˆÓ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ, ÙˆÓ ÂÈıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ
492
Î·È ÙˆÓ ËˆÛÈÓÔʛψÓ, Û ۇÁÎÚÈÛË ÌÂ Ê˘ÛÈÔÏÔÁÈο ·È‰È¿. ∆· ›‰· ÙˆÓ PGE4 (p=0,0005), 15-HETE (p=0,002), LTE4 (p=0,04) Î·È LTB4 (p=0,05) ‹Ù·Ó ›Û˘ ·˘ÍË̤ӷ ÛÙ· ·È‰È¿ ÌÂ Û˘ÚÈÁÌfi Û ۇÁÎÚÈÛË Ì ٷ Ê˘ÛÈÔÏÔÁÈο, ÂÓÒ Ë PGD4 Î·È Ë ‚-ÙÚ˘Ù¿ÛË ‰ÂÓ ‹Ù·Ó. ™˘ÌÂÚ¿ÛÌ·Ù·: ∞˘Ù‹ Ë ÌÂϤÙË ÂȂ‚·ÈÒÓÂÈ ÙÔ fiÙÈ Ë ÊÏÂÁÌÔÓ‹ Â›Ó·È ·ÚÔ‡Û· ÛÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ ÙˆÓ Ôχ ÌÈÎÚÒÓ ·È‰ÈÒÓ ÌÂ Û˘ÚÈÁÌfi Î·È ÌÔÚ› Ó· ‰È·Ê¤ÚÂÈ ·fi ÙÔÓ Ù‡Ô Ô˘ ·ÓÙÈÌÂÙˆ›˙Ô˘Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ Ì ¿ÛıÌ·.
1 Krawiec ME, Westcott JY, Chu HW, Balzar S, Trudeau JB, Schwartz LB et al Persistent wheezing in very young children is associated with lower respiratory inflammation Am J Respir Crit Care Med 2001;163:1338-1343
£ÂÔÊ¿Ó˘ ∆ÛÈÏÈÁÈ¿ÓÓ˘ ¢È¢ı˘ÓÙ‹˜ ¶·È‰È·ÙÚÈÎÔ‡ ∆Ì‹Ì·ÙÔ˜ ¡·˘ÙÈÎÔ‡ ¡ÔÛÔÎÔÌ›Ԣ ∞ıËÓÒÓ
¶·È‰È·ÙÚÈ΋ 2001;64:493-498
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Paediatriki 2001;64:493-498
ORIGINAL PAPER
∂ȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË ÙˆÓ Î·ÎÒÛÂˆÓ ÛÙËÓ ÂÊ˂›· Õ. ¶·ÓÙ·˙‹, ¡. ÷Ù˙ËΈÓÛÙ·ÓÙ›ÓÔ˘, £. ∆Ú·ÁÈ·ÓÓ›‰Ë˜, °. ∫·Ú·‚¿Ó·, £. ∞ÏÌ·Ó›‰Ë˜, ™. ∞ÁÁÂÏfiÔ˘ÏÔ˜
Epidemiological study of injuries in adolescence A. Pantazi, N. Hatzikonstantinou, T. Tragianidis, G. Karavana, T. Almanidis, S. Agelopoulos
¶ÂÚ›ÏË„Ë: ™Ùfi¯Ô˜ Ù˘ ¤Ú¢ӷ˜ ·˘Ù‹˜ Â›Ó·È Ë Û˘ÏÏÔÁ‹ Î·È Ë ÌÂϤÙË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ô˘ ·ÊÔÚÔ‡Ó ÙȘ ηÎÒÛÂȘ ÙˆÓ ÂÊ‹‚ˆÓ ÛÙËÓ ÂÚÈÔ¯‹ Ù˘ ƒÔ‰fi˘, ÒÛÙ ӷ ηıÔÚÈÛÙÔ‡Ó ÔÈ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ Î·È Ó· ¯·Ú·¯ÙÔ‡Ó ÛÙÚ·ÙËÁÈΤ˜ ÚfiÏ˄˘. ∏ ÌÂϤÙË Ì·˜ ·ÊÔÚ¿ 3334 ÂÊ‹‚Ô˘˜ ËÏÈΛ·˜ 13-19 ¯ÚfiÓˆÓ Ô˘ ÚÔÛ‹Ïı·Ó ÛÙÔ ∆Ì‹Ì· ∂ÂÈÁfiÓÙˆÓ ¶ÂÚÈÛÙ·ÙÈÎÒÓ Ù· 3 ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ÁÈ· ·Ù˘¯‹Ì·Ù· Î·È ‰ËÏËÙËÚÈ¿ÛÂȘ. ∆· ÛÙÔȯ›· Ô˘ ÂϤÁÍ·Ì ·ÊÔÚÔ‡Û·Ó ÛÙÔ Ê‡ÏÔ, ÙËÓ ËÏÈΛ·, ÙËÓ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· ÛÙËÓ ÔÔ›· ·Ó‹Î·Ó, ÙËÓ ·ÈÙ›· Ù˘ οΈÛ˘, ÙËÓ ÎÏÈÓÈ΋ ʇÛË Î·È ÙË ‚·Ú‡ÙËÙ¿ Ù˘. ™ÙÔ Û‡ÓÔÏÔ ÙˆÓ ÂÊ‹‚ˆÓ, ÙÔ 67,8% ‹Ù·Ó ¿ÚÚÂÓ˜ Î·È ÙÔ 32,2% ı‹ÏÂȘ. OÈ Î·ÎÒÛÂȘ ÔÊ›ÏÔÓÙ·Ó ÛÙ· ÛÔÚ ‹ ÙÔ ·È¯Ó›‰È Û ÔÛÔÛÙfi 40,8%, Û ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù· Û ÔÛÔÛÙfi 38% Î·È Û ·Ù˘¯‹Ì·Ù· ηٿ ÙËÓ ÂÚÁ·Û›· Û ÔÛÔÛÙfi 11,2%. ™˘ÓËı¤ÛÙÂÚ˜ ‹Ù·Ó ÔÈ Î·ÎÒÛÂȘ ÙˆÓ ¿ÎÚˆÓ (ÛÔÚ 38,1%, ÙÚÔ¯·›· 42%, ÂÚÁ·ÙÈο ·Ù˘¯‹Ì·Ù· 56,4%). OÈ ÎÚ·ÓÈÔÂÁÎÂÊ·ÏÈΤ˜ ηÎÒÛÂȘ ÛËÌÂÈÒıËÎ·Ó Î˘Ú›ˆ˜ ÛÙ· ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù· (77,8%). OÈ ÃÚÈÛÙÈ·ÓÔ› ˘¤ÛÙËÛ·Ó Î·ÎÒÛÂȘ ΢ڛˆ˜ ÛÙ· ÛÔÚ (46,3%), ÂÓÒ ÔÈ ªÔ˘ÛÔ˘ÏÌ¿ÓÔÈ Î·È ÔÈ ∞ı›ÁÁ·ÓÔÈ ÛÙËÓ ÂÚÁ·Û›· (20,8%, 23,1%). OÈ ‰ËÏËÙËÚÈ¿ÛÂȘ ·Ó‹Ïı·Ó Û ÔÛÔÛÙfi 6,9% Î·È ÔÊ›ÏÔÓÙ·Ó Î˘Ú›ˆ˜ ÛÙË Ï‹„Ë ·ÏÎÔfiÏ. OÈ ·fiÂÈÚ˜ ·˘ÙÔÎÙÔÓ›·˜ ·ÓÙÈÚÔÛÒ¢·Ó ÙÔ 2,8%. ∏ ‚·Ú‡ÙËÙ· ÙˆÓ Î·ÎÒÛÂˆÓ ÂϤÁ¯ıËΠ̠‚¿ÛË ÙȘ ÂÈÛ·ÁˆÁ¤˜, Ô˘ ·Ó‹Ïı·Ó ÛÙÔ 14%. ∆Ô 52% ÙˆÓ ÂÈÛ·ÁˆÁÒÓ ·ÊÔÚÔ‡Û ٷ ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù·. ∆Ô 0,4% ÙˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ Î·Ù¤ÏËÍ Û ı¿Ó·ÙÔ, Ô˘ ÚÔÎÏ‹ıËΠ΢ڛˆ˜ ·fi ÙÚÔ¯·›· Î·È ÂÚÁ·ÙÈο ·Ù˘¯‹Ì·Ù· (75%, 16,7%). ŒÓ· ·fi Ù· ÌÂÁ·Ï‡ÙÂÚ· ÚÔ‚Ï‹Ì·Ù· Ù˘ ‰ËÌfiÛÈ·˜ ˘Á›·˜ ÛÙË Û‡Á¯ÚÔÓË ÎÔÈÓˆÓ›· Â›Ó·È ÔÈ Î·ÎÒÛÂȘ ÛÙËÓ ÂÊ˂›·. O ηıÔÚÈÛÌfi˜ ÙÔ˘ ÚÔÊ›Ï ÙˆÓ Î·ÎÒÛÂˆÓ ı· Ú¤ÂÈ
Abstract: The aim of the study was to collect data concerning accidents in adolescents who live in the area of Rodopi, in order to determine the risk factors and recommend strategies for their prevention. The population of the study consisted of 3334 adolescents, 13-19 years of age, who presented during the last three years to the Emergency Room because of accidents and poisoning. The demographic data, the clinical nature, severity and cause of the injury were reviewed. The male to female ratio was about 2:1. Injuries due to sports accounted for 40,8% of the cases, traffic 38%, and labor accidents 11,2%. More common were injuries to the extremities (sports 38,1%, traffic accidents 42%, and occupational 56,4). Craniocerebral injuries were observed mostly in traffic accidents (77,8%). Sport accidents were observed most commonly in the Christian population while occupational accidents involved mainly Muslims (20,8%) and Gypsies (23,1%). Poisoning (6,9%) was usually due to alcohol abuse. Suicide attempts comprised 2,8% of the cases. The severity of injuries was evaluated on the basis of the number of admissions which comprised 14% of the cases. 52% of admissions concerned traffic injuries. 0,4% of injuries were fatal and were due primarily to traffic and occupational accidents (75%, 16,7% respectively). Injuries during adolescence constitute one of the greatest public health problems in today’s society. The magnitude of the problem requires a multidisciplinary approach and is therefore important to give priority to the investigation of the profile of injuries in order to plan strategies for their prevention.
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ™ÈÛÌ·ÓfiÁÏÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô ∫ÔÌÔÙËÓ‹˜
Pediatric Clinic Sismanoglio Hospital of Komotini, Komotini
493
¶·È‰È·ÙÚÈ΋ 2001;64:493-498
Paediatriki 2001;64:493-498
Ó· ·ÔÙÂϤÛÂÈ ÔÏÈÙÈ΋ ÚÔÙÂÚ·ÈfiÙËÙ·, ÒÛÙ ӷ ۯ‰ȷÛÙÔ‡Ó ÔÈ ÛÙfi¯ÔÈ Î·È ÔÈ ÛÙÚ·ÙËÁÈΤ˜ ·Ú¤Ì‚·Û˘.
§¤ÍÂȘ ÎÏÂȉȿ: ηÎÒÛÂȘ, ÂÊ˂›·, ÏËı˘ÛÌȷΤ˜ ÔÌ¿‰Â˜
Key words: injuries, adolescence, population groups.
∂ÈÛ·ÁˆÁ‹ ¢ÂÓ ˘¿Ú¯ÂÈ Û·Ê‹˜ ÔÚÈÛÌfi˜ ÁÈ· ÙËÓ ÂÊ˂›·, Ô ÔÔ›Ô˜ Ó· ·ÓÙ·ÔÎÚ›ÓÂÙ·È ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ·. ™‡Ìʈӷ Ì ÙÔÓ Piaget, Ë ÂÊ˂›· ·ÓÙÈÚÔۈ‡ÂÈ ÙËÓ ÂÚ›Ô‰Ô Ù˘ Ï‹ÚÔ˘˜ ÂÁηٿÛÙ·Û˘ ÙˆÓ Â›ÛËÌˆÓ ÏÂÈÙÔ˘ÚÁÈÒÓ ÙÔ˘ ·ÓıÚÒÔ˘. ™’ ·˘Ùfi ÙÔ ‰È¿ÛÙËÌ·, Ë ‰È·‰Èηۛ· Ù˘ ˆÚ›Ì·ÓÛ˘ ÙˆÓ ÛˆÌ·ÙÈÎÒÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ Î·È Ù˘ ·fiÎÙËÛ˘ ÙˆÓ ‰ÂÍÈÔÙ‹ÙˆÓ ÙÔ˘ ÂÓ‹ÏÈη ÔÏÔÎÏËÚÒÓÂÙ·È, ̤۷ Û ¤Ó· Ï·›ÛÈÔ Û˘Ó·ÈÛıËÌ·ÙÈÎÒÓ ÂÓ·ÏÏ·ÁÒÓ Î·È ‰È·Ù·Ú·¯ÒÓ Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜, Ô˘ Û ÔÔÈ·‰‹ÔÙ ¿ÏÏË ËÏÈΛ· ı· ÌÔÚÔ‡Û·Ó Ó· ¯·Ú·ÎÙËÚÈÛÙÔ‡Ó ·ıÔÏÔÁÈΤ˜. OÈ ¤ÊË‚ÔÈ ·Ó·Ù‡ÛÛÔ˘Ó Û˘¯Ó¿ ¤Ó· ·›ÛıËÌ· ·ı·Ó·Û›·˜, Ô˘ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ ˘ÂÚÂÎÙ›ÌËÛË ÙˆÓ ÈηÓÔÙ‹ÙˆÓ ÙÔ˘˜, ¢ı‡ÓÂÙ·È ÁÈ· ¤Ó· ÛËÌ·ÓÙÈÎfi ·ÚÈıÌfi ηÎÒÛÂˆÓ Ô˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÔ˘Ó ·ÎfiÌË Î·È ÛÙÔ ı¿Ó·ÙÔ (1). ™ÙȘ ∏¶∞, ÙÔ 1987, ÔÈ ı¿Ó·ÙÔÈ Û ӤԢ˜ 15-19 ÂÙÒÓ ‹Ù·Ó 84,6/100.000 ηÙÔ›ÎÔ˘˜, Ì ÚÔÂÍ¿Ú¯Ô˘Û· ·ÈÙ›· Ù· ·Ù˘¯‹Ì·Ù· (2). ∞Ó¿ÏÔÁ· ÔÛÔÛÙ¿, Û ‰ÈÂıÓ¤˜ ›‰Ô, ·Ó·Ê¤ÚÔÓÙ·È Î·È ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜, ÔÈ ÔÔ›ÔÈ Î·Ù·ÓfiËÛ·Ó ÙÔ Ì¤ÁÂıÔ˜ ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ Î·È Î·Ù¤ÁÚ·„·Ó ÙËÓ ÂȉËÌÈÔÏÔÁ›· ÙˆÓ Î·ÎÒÛÂˆÓ ÛÙËÓ ÂÊ˂›·, ÛÙ¤ÏÓÔÓÙ·˜ ÙÔ ·ÓËÛ˘¯ËÙÈÎfi Ì‹Ó˘Ì· fiÙÈ Ô ·ÚÈıÌfi˜ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ ·˘Í¿ÓÂÈ ‰Ú·Ì·ÙÈο οı ¯ÚfiÓÔ, Ì ·Ó˘ÔÏfiÁÈÛÙ˜ ÎÔÈÓˆÓÈΤ˜ Î·È ÔÈÎÔÓÔÌÈΤ˜ Û˘Ó¤ÂȘ (3-9). ∏ ∂ÏÏ¿‰·, Ì›· ¯ÒÚ· Ì ¤ÓÙÔÓÔ ‰ËÌÔÁÚ·ÊÈÎfi Úfi‚ÏËÌ·, η٤¯ÂÈ Ù· ıÏÈ‚ÂÚ¿ ÚˆÙ›· ÛÙËÓ ∂˘Úˆ·˚΋ ŒÓˆÛË Û ı·Ó¿ÙÔ˘˜ ·fi ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù· (10), ÁÂÁÔÓfi˜ Ô˘ ηıÈÛÙ¿ ÂÈÙ·ÎÙÈ΋ ÙËÓ ·Ó¿ÁÎË ÁÈ· ÔÏ˘Â›Â‰Ë ÚÔÛ¤ÁÁÈÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜, ۯ‰ȷÛÌfi ÛÙÚ·ÙËÁÈÎÒÓ ÚfiÏ˄˘ Î·È ¿ÌÂÛË ÂÊ·ÚÌÔÁ‹ ÙÔ˘˜. ªÂ ÙËÓ ¤ÚÂ˘Ó¿ Ì·˜ ·˘Ù‹, ÚÔÛ·ı‹Û·Ì ӷ Û˘ÏϤÍÔ˘ÌÂ Î·È Ó· ÌÂÏÂÙ‹ÛÔ˘Ì ٷ ‰Â‰Ô̤ӷ Ô˘ ·ÊÔÚÔ‡Ó ÙȘ ηÎÒÛÂȘ ηٿ ÙËÓ ÂÊ˂›· ÛÙËÓ ÂÚÈÔ¯‹ Ì·˜, ı¤ÏÔÓÙ·˜ ¤ÙÛÈ Ó· ηıÔÚ›ÛÔ˘Ì ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ Î·È Ó· Û˘Ì‚¿ÏÔ˘Ì ÛÙË Ï‹„Ë Ì¤ÙÚˆÓ ÁÈ· ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÂÓfi˜ ·Ú·ÁÓˆÚÈṲ̂ÓÔ˘ - ÛÙË ¯ÒÚ· Ì·˜ - ÚÔ‚Ï‹Ì·ÙÔ˜.
01/01/1994 ¤ˆ˜ 31/12/1996. °È· οı ¤ÊË‚Ô Î·Ù·ÁÚ¿„·Ì ٷ ‰ËÌÔÁÚ·ÊÈο ÛÙÔȯ›·, ÙËÓ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· (ÃÚÈÛÙÈ·ÓÔ›, ªÔ˘ÛÔ˘ÏÌ¿ÓÔÈ, ∞ı›ÁÁ·ÓÔÈ, ¶·ÏÈÓÓÔÛÙÔ‡ÓÙ˜), ÙËÓ ·ÈÙ›· Ù˘ οΈÛ˘, ÙËÓ ÎÏÈÓÈ΋ ʇÛË Î·È ÙË ‚·Ú‡ÙËÙ¿ Ù˘. ∫·Ù¿ ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ, ÔÈ ·Èٛ˜ ÙˆÓ Î·ÎÒÛÂˆÓ ÔÌ·‰ÔÔÈ‹ıËÎ·Ó Û ¤ÓÙ ÌÂÁ¿Ï˜ ηÙËÁÔڛ˜: ·) ηÎÒÛÂȘ ÛÙ· ÛÔÚ (¿ıÏËÛË, ·È¯Ó›‰È), ‚) ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù·, Á) ÂÚÁ·ÙÈο ·Ù˘¯‹Ì·Ù·, ‰) ·˘ÙÔÎÙÔӛ˜ Î·È Â) ηÎÒÛÂȘ ·fi ¿ÛÎËÛË ‚›·˜. OÈ Î·ÎÒÛÂȘ, ·Ó¿ÏÔÁ· Ì ÙËÓ ÎÏÈÓÈ΋ ÙÔ˘˜ ʇÛË, ÔÌ·‰ÔÔÈ‹ıËÎ·Ó Û‡Ìʈӷ Ì ÙÔ SCIPP (¶ÔÏÈÙÂÈ·Îfi ÚfiÁÚ·ÌÌ· ÁÈ· ÙËÓ ÚfiÏË„Ë ÙˆÓ Î·ÎÒÛÂˆÓ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ÙˆÓ ∏¶∞) (¶›Ó·Î·˜ 1). ∏ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ ¤ÁÈÓ Ì ÙÔ ÛÙ·ÙÈÛÙÈÎfi ÚfiÁÚ·ÌÌ· SPSS.
∞ÔÙÂϤÛÌ·Ù· ™Â ‰È¿ÛÙËÌ· ÙÚÈÒÓ ÂÙÒÓ, ÚÔÛ‹Ïı·Ó ÛÙÔ ∆Ì‹Ì· ∂ÂÈÁfiÓÙˆÓ ¶ÂÚÈÛÙ·ÙÈÎÒÓ 6635 ·ÛıÂÓ›˜ ËÏÈΛ·˜ 0-19 ÂÙÒÓ, ÁÈ· ·Ù˘¯‹Ì·Ù· ‹ ‰ËÏËÙËÚÈ¿ÛÂȘ. OÈ ÌÈÛÔ› ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ ·˘ÙÔ‡˜ (3334) ‹Ù·Ó ¤ÊË‚ÔÈ ËÏÈΛ·˜ 13-19 ÂÙÒÓ. ∏ ηٷÓÔÌ‹ ÙˆÓ Î·ÎÒÛÂˆÓ ÙˆÓ ÂÊ‹‚ˆÓ ÂÙËÛ›ˆ˜, ÛÙ· ÙÚ›· ¯ÚfiÓÈ· Ù˘ ÌÂϤÙ˘, Ê·›ÓÂÙ·È ÛÙÔÓ ¶›Ó·Î· 2. ™‡Ìʈӷ Ì ٷ ‰ËÌÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ ÙˆÓ ÌÂÏÂÙËı¤ÓÙˆÓ ÂÊ‹‚ˆÓ, Ë ·Ó·ÏÔÁ›· ÙˆÓ ·ÚÚ¤ÓˆÓ Ô˘ ˘¤ÛÙËÛ·Ó Î¿ÎˆÛË Â›Ó·È ˘ÂÚ‰ÈÏ¿ÛÈ· (67,8%) ·fi ·˘Ù‹ ÙˆÓ ıËϤˆÓ (32,2%). ÀÂÚ‰ÈÏ¿ÛÈ·, ›Û˘, ‹Ù·Ó Ë ·Ó·ÏÔÁ›· ÙˆÓ ÂÊ‹‚ˆÓ ÙˆÓ ·ÛÙÈÎÒÓ ÂÚÈÔ¯ÒÓ (66,7%) Û ۯ¤ÛË Ì ·˘ÙÔ‡˜ ÙˆÓ ·ÁÚÔÙÈÎÒÓ ÂÚÈÔ¯ÒÓ (33,3%). ŒÓ· ·ÎfiÌË ÛÙÔÈ¯Â›Ô Ô˘ ÚԤ΢„ ‹Ù·Ó fiÙÈ ÔÈ ÃÚÈÛÙÈ·ÓÔ› ˘¤ÛÙËÛ·Ó ÌÂÁ·Ï‡ÙÂÚÔ ·ÚÈıÌfi ·Ù˘¯ËÌ¿ÙˆÓ (57,6%) ·fi ÙÔ˘˜ ªÔ˘ÛÔ˘ÏÌ¿ÓÔ˘˜ (26,2%), Ï·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ Ù· ¶›Ó·Î·˜ 1. ∆·ÍÈÓfiÌËÛË Ù˘ ÎÏÈÓÈ΋˜ ʇÛ˘ ÙˆÓ Î·ÎÒÛÂˆÓ Û‡Ìʈӷ Ì ÙÔ ÔÏÈÙÂÈ·Îfi ÚfiÁÚ·ÌÌ· ÁÈ· ÙËÓ ÚfiÏË„Ë ÙˆÓ Î·ÎÒÛÂˆÓ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ÙˆÓ ∏¶∞ (SCIPP)
ÀÏÈÎfi Î·È Ì¤ıÔ‰Ô˜
∆·ÍÈÓfiÌËÛË Î·ÎÒÛÂˆÓ Î·Ù¿ ÙÔ SCIPP* ñ ∫·Ù¿ÁÌ·Ù· ñ ∂Í·ÚıÚ‹Ì·Ù· ñ ∞ÓÔÈÎÙ¿ Î·È Û˘ÓıÏÈÙÈο ÙÚ·‡Ì·Ù· ñ ∫ÂÊ·Ï‹˜ ñ ÕÎÚˆÓ ñ ÕÏÏ· ñ ªÒψ˜-∂ΉÔÚ¤˜ ñ ∫Ú·ÓÈÔÂÁÎÂÊ·ÏÈΤ˜ ηÎÒÛÂȘ ñ ∫·ÎÒÛÂȘ ÔÚÁ¿ÓˆÓ ñ ÕÏϘ
ÀÏÈÎfi Ù˘ ÌÂϤÙ˘ ·ÔÙ¤ÏÂÛ·Ó 3334 ¤ÊË‚ÔÈ, ËÏÈΛ·˜ 1319 ÂÙÒÓ, Ô˘ ˘¤ÛÙËÛ·Ó Î¿ÎˆÛË ÛÙÔ ‰È¿ÛÙËÌ· ·fi
*Statewide Childhood Injury Preventive Program
494
¶·È‰È·ÙÚÈ΋ 2001;64:493-498
Paediatriki 2001;64:493-498
¶›Ó·Î·˜ 2. ∫·Ù·ÓÔÌ‹ ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ˘¤ÛÙËÛ·Ó Î¿ÎˆÛË Î·Ù¿ ʇÏÔ, ηÙÔÈΛ· Î·È ÏËı˘ÛÌȷ΋ ÔÌ¿‰· º‡ÏÔ
ÕÚÚÂÓ˜
£‹ÏÂȘ
% ∆fiÔ˜ ηÙÔÈΛ·˜ % ¶ÏËı˘ÛÌȷ΋ ÔÌ¿‰· %
67,8 ∞ÛÙÔ› 66,7 ÃÚÈÛÙÈ·ÓÔ› 57,6 ∞ı›ÁÁ·ÓÔÈ 9,5
32,2 ∞ÁÚfiÙ˜ 33,3 ªÔ˘ÛÔ˘ÏÌ¿ÓÔÈ 26,2 ¶·ÏÈÓÓÔÛÙÔ‡ÓÙ˜ 6,7
%
19 18 17 16 15 14 13
1600 1400 1200
ÂÙÒÓ ÂÙÒÓ ÂÙÒÓ ÂÙÒÓ ÂÙÒÓ ÂÙÒÓ ÂÙÒÓ
1000 800 600 400
ÔÛÔÛÙ¿ ÙˆÓ ÔÌ¿‰ˆÓ ·˘ÙÒÓ ÛÙÔ ÁÂÓÈÎfi ÏËı˘ÛÌfi. ™¯ÂÙÈο Ì ÙȘ ·Èٛ˜ ÚfiÎÏËÛ˘ Ù˘ οΈÛ˘ Î·È ÙË Û˘¯ÓfiÙËÙ¿ ÙÔ˘˜ Û οı ËÏÈÎȷ΋ ÔÌ¿‰·, ÚԤ΢„ fiÙÈ ÙÔ 41,7% ÙˆÓ ÙÚ·˘Ì·ÙÈÛÌÒÓ Û˘Ó¤‚·ÈÓ ηٿ ÙËÓ ¿ıÏËÛË Î·È ÙÔ ·È¯Ó›‰È, ÙÔ 36,6% ÔÊÂÈÏfiÙ·Ó Û ÙÚÔ¯·›· Û˘Ì‚¿ÓÙ· Î·È ÙÔ 11,2% ÙˆÓ Î·ÎÒÛÂˆÓ ·ÊÔÚÔ‡Û ٷ ÂÚÁ·ÙÈο ·Ù˘¯‹Ì·Ù· (∂ÈÎfiÓ· 1). ∂›Û˘, Ô Ú˘ıÌfi˜ ÙˆÓ Î·ÎÒÛÂˆÓ ·˘Í·ÓfiÙ·Ó ·Ú¿ÏÏËÏ· Ì ÙËÓ ËÏÈΛ·, ÒÛÙ ÙÔ 35% ·˘ÙÒÓ Ó· ·Ú·ÙËÚÂ›Ù·È ÛÙȘ ËÏÈ˘ ÙˆÓ 17 Î·È 18 ÂÙÒÓ (∂ÈÎfiÓ· 1). ™ÙÔ˘˜ Ó¤Ô˘˜ ¿Óˆ ÙˆÓ 16 ÂÙÒÓ, Ë ·‡ÍËÛË ·ÊÔÚÔ‡Û ÛÙ· ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù·, ÂÓÒ ÛÙȘ ÌÈÎÚfiÙÂÚ˜ ËÏÈ˘ ·ÊÔÚÔ‡Û ÛÙ· ·Ù˘¯‹Ì·Ù· ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÛÔÚ (¿ıÏËÛË, ·È¯Ó›‰È). ™Ù·‰È·Î‹ ·‡ÍËÛË ‰È·ÈÛÙÒıËÎÂ Î·È ÛÙ· ·Ù˘¯‹Ì·Ù· ηٿ ÙËÓ ÂÚÁ·Û›·, ·ÏÏ¿ Î·È ÛÙȘ ‰ËÏËÙËÚÈ¿ÛÂȘ Ì ·ÏÎÔfiÏ. O ÙÚfiÔ˜ ˙ˆ‹˜ Ê·›ÓÂÙ·È fiÙÈ Úԉȷı¤ÙÂÈ Û ‰È·ÊÔÚÂÙÈο ›‰Ë ·Ù˘¯ËÌ¿ÙˆÓ, fiˆ˜ ÚÔ·ÙÂÈ ·fi ÙË Û˘Û¯¤ÙÈÛË Ù˘ ·ÈÙ›·˜ Ù˘ οΈÛ˘ Î·È Ù˘ ÏËı˘ÛÌȷ΋˜ ÔÌ¿‰·˜ ÛÙËÓ ÔÔ›· ·Ó‹ÎÂ Ô ¤ÊË‚Ô˜ (∂ÈÎfiÓ· 2). ™ÙËÓ ÔÌ¿‰· ÙˆÓ ÃÚÈÛÙÈ·ÓÒÓ, Û˘ÓËı¤ÛÙÂÚ˜ ‹Ù·Ó ÔÈ Î·ÎÒÛÂȘ Ô˘ Û˘Ó¤‚·ÈÓ·Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·È¯ÓȉÈÔ‡ Î·È Ù˘ ¿ıÏËÛ˘ (46,2%), ηıÒ˜ Î·È Ù· ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù· (37,6%). ŒÓ· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ∞ı›ÁÁ·ÓˆÓ (23,1%) Î·È ªÔ˘ÛÔ˘ÏÌ¿ÓˆÓ (20,8%) ˘¤ÛÙË Î¿ÎˆÛË Û ÂÚÁ·ÙÈÎfi ·Ù‡¯ËÌ·, ÂÓÒ ÛÙËÓ ÔÌ¿‰· ÙˆÓ ·ÏÈÓÓÔÛÙÔ‡ÓÙˆÓ ·Ú·ÙËÚ‹ıËΠÙÔ ˘„ËÏfiÙÂÚÔ ÔÛÔÛÙfi ÙÚÔ¯·›ˆÓ ·Ù˘¯ËÌ¿ÙˆÓ (42%) ÌÂٷ͇ fiÏˆÓ ÙˆÓ ÏËı˘ÛÌÈ·ÎÒÓ ÔÌ¿‰ˆÓ. ∏ ÎÏÈÓÈ΋ ʇÛË ÙˆÓ Î·ÎÒÛÂˆÓ Ê·›ÓÂÙ·È Ó· Û˘Ó‰¤ÂÙ·È ¿ÌÂÛ· Ì ÙËÓ ·ÈÙ›· ÚfiÎÏËÛ˘ Ù˘ οΈÛ˘ (∂ÈÎfiÓ· 3). ™ÙË ÌÂϤÙË Ì·˜, ÔÈ Î·ÎÒÛÂȘ ÙˆÓ ¿ÎÚˆÓ ‹Ù·Ó Û˘¯ÓfiÙÂÚ˜ ÛÙ· ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù· (30,4%), ÂÓÒ ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ·ÓÙÈÚÔÛÒ¢·Ó Î·È Ù· ÂÁη‡Ì·Ù· ÙˆÓ ¿ÎÚˆÓ (11,6%). ™’ ·˘Ù‹ ÙËÓ Î·ÙËÁÔÚ›· ·Ù˘¯ËÌ¿ÙˆÓ ÛËÌÂÈÒıËÎ·Ó ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ÎÚ·ÓÈÔÂÁÎÂÊ·ÏÈΤ˜ ηÎÒÛÂȘ (∫∂∫) (6,8%). OÈ Î·ÎÒÛÂȘ ÙˆÓ ¿ÎÚˆÓ ÂÈÎÚ·ÙÔ‡Û·Ó Î·È ÛÙÔ˘˜ ÙÚ·˘Ì·ÙÈÛÌÔ‡˜ ηٿ ÙËÓ ¿ıÏËÛË (48,1%), ·ÏÏ¿ Î·È ÛÙ· ÂÚÁ·ÙÈο ·Ù˘¯‹Ì·Ù· (56,4%). ™ÙËÓ Î·ÙË-
200 0 ∆ÚÔ¯·›·
™ÔÚ
∂ÚÁ·Û›· ∞˘ÙÔÎÙÔӛ˜ ∞ÏÎÔfiÏ
∂ÈÎfiÓ· 1. ∞ÂÈÎfiÓÈÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ ÙˆÓ Î·ÎÒÛÂˆÓ Î·Ù¿ ËÏÈÎȷ΋ ÔÌ¿‰·.
900 800 700 600 500 400
∆ÚÔ¯·›· ™fiÚ ∂ÚÁ·Û›· ∞˘ÙÔÎÙÔӛ˜ ∞ÏÎÔfiÏ
300 200 100 0 ÃÚÈÛÙÈ·ÓÔ› ªÔ˘ÛÔ˘ÏÌ¿ÓÔÈ ∞ı›ÁÁ·ÓÔÈ ¶·ÏÈÓÔÛÙÔ‡ÓÙ˜ ∂ÈÎfiÓ· 2. ™¯ËÌ·ÙÈ΋ ·ÂÈÎfiÓÈÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ ·ÈÙ›·˜ Ù˘ οΈÛ˘ ηٿ ÏËı˘ÛÌȷ΋ ÔÌ¿‰·.
ÁÔÚ›·, fï˜, ÙˆÓ ÂÚÁ·ÙÈÎÒÓ ·Ù˘¯ËÌ¿ÙˆÓ, Û˘¯ÓfiÙÂÚ· ‹Ù·Ó Ù· ηٿÁÌ·Ù· (11,2%). OÈ ·fiÂÈÚ˜ ·˘ÙÔÎÙÔÓ›·˜ ·Ó‹Ïı·Ó ÛÙÔ 2,9% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ Î·ÎÒÛˆÓ. ŒÓ· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ·˘ÙÒÓ ÔÊÂÈÏfiÙ·Ó Û ϋ„Ë Ê·Ú̷΢ÙÈÎÒÓ Ô˘ÛÈÒÓ ‹ Ê˘ÙÔÊ·Ú̿ΈÓ. ™ÙȘ ‰ËÏËÙËÚÈ¿ÛÂȘ, Û˘¯ÓfiÙÂÚÔ Â›‰Ô˜ ‹Ù·Ó Ë Ï‹„Ë ·ÏÎÔfiÏ Î·È ·Ú·ÙËÚ‹ıËΠ·ÔÎÏÂÈÛÙÈο ÛÙËÓ ÔÌ¿‰· ÙˆÓ ÃÚÈÛÙÈ·ÓÒÓ. ŒÓ· ÌÈÎÚfi ÔÛÔÛÙfi ·Ù˘¯ËÌ¿ÙˆÓ (2,1%) ÔÊÂÈÏfiÙ·Ó ÛÙË ¯Ú‹ÛË ‚›·˜ ÌÂٷ͇ ÙˆÓ ÂÊ‹‚ˆÓ, ȉȷ›ÙÂÚ· ÛÙȘ ËÏÈ˘ 15-18 ÂÙÒÓ. °È· ÙÔÓ Î·ıÔÚÈÛÌfi Ù˘ ‚·Ú‡ÙËÙ·˜ ÙˆÓ Î·ÎÒÛˆÓ, ıˆڋıËΠ·Ó·Áη›Ô ÁÈ· ÂÚÈÁÚ·ÊÈÎÔ‡˜
495
¶·È‰È·ÙÚÈ΋ 2001;64:493-498
Paediatriki 2001;64:493-498
800
∫·Ì›· ªÈÎÚÔÂÂÌ‚¿ÛÂȘ ∂ÈÛ·ÁˆÁ‹ £¿Ó·ÙÔ˜
80%
700 68%
70%
600
73%
60% 500
60%
400
47%
50% 300
43% ∆ÚÔ¯·›·
200 100
40%
™ÔÚ 0 ∂ Í¿ ∆Ú ∫ Úı ¿Ù ·‡ Œ ∫∂ Á ·Á ËÌ Ì· ∫ Ì· η˘ · Ì·
30%
24%
23%
20% ¶Ô Ï˘ ÙÚ ·˘ Ì.
∂ÈÎfiÓ· 3. ∞ÂÈÎfiÓÈÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ ÎÏÈÓÈ΋˜ ʇÛ˘ ÙˆÓ Î·ÎÒÛÂˆÓ ·Ó¿ÏÔÁ· Ì ÙËÓ ·ÈÙ›· Ô˘ ÙȘ ÚÔηÏÔ‡Ó.
ÛÎÔÔ‡˜ Ó· ÔÌ·‰ÔÔÈËıÔ‡Ó ÔÈ Î·ÎÒÛÂȘ Û 4 ›‰·, ·Ó¿ÏÔÁ· Ì ÙË ıÂڷ¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË. ∏ ηٿٷÍË ÙˆÓ Î·ÎÒÛÂˆÓ Ì ‚¿ÛË ÙÔ ÌÔÓÙ¤ÏÔ ·˘Ùfi ¤‰ÂÈÍ fiÙÈ ·fi ÙÔ˘˜ 3334 ·ÛıÂÓ›˜, ÔÈ 1663 (ÔÛÔÛÙfi 51%) ‰ÂÓ ¤¯ÚË˙·Ó ȉȷ›ÙÂÚ˘ ·ÓÙÈÌÂÙÒÈÛ˘, ÔÈ 1123 ˘Â‚Ï‹ıËÛ·Ó Û ÌÈÎÚÔÂÂÌ‚¿ÛÂȘ, ÔÈ 461 ÂÈÛ‹¯ıËÛ·Ó Î·È ÔÈ 12 η٤ÏËÍ·Ó. ™˘Û¯ÂÙ›˙ÔÓÙ·˜ ÙË ‚·Ú‡ÙËÙ· Ù˘ οΈÛ˘ Ì ÙËÓ ·ÈÙ›· ÚfiÎÏËÛ˘ (∂ÈÎfiÓ· 4) ÚÔ·ÙÂÈ fiÙÈ ÛÙ· ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù· ÂÈÛ‹¯ıË ÙÔ 20% (239 ·ÛıÂÓ›˜), ÂÓÒ ÔÈ ÓÂÎÚÔ› ·Ó‹Ïı·Ó ÛÙÔ˘˜ ÂÓÓ¤·, ÛÙÔ˘˜ ÙÚ·˘Ì·ÙÈÛÌÔ‡˜ ηٿ ÙËÓ ¿ıÏËÛË ÔÈ ÂÈÛ·ÁˆÁ¤˜ ÌfiÏȘ ÏËÛ›·Û·Ó ÙÔ 5,1% (70 ·ÛıÂÓ›˜) Î·È ÛÙ· ÂÚÁ·ÙÈο ·Ù˘¯‹Ì·Ù· ÔÈ ÂÈÛ·ÁˆÁ¤˜ ‹Ù·Ó 34 (9,1%) Î·È Î·Ù·ÁÚ¿ÊËÎ·Ó ‰‡Ô ı¿Ó·ÙÔÈ. ¢‡Ô ·ÎfiÌË ı¿Ó·ÙÔÈ ÔÊ›ÏÔÓÙ·Ó Û ·˘ÙÔÎÙÔÓ›·. ™˘˙‹ÙËÛË ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÊ˂›·˜, Ù· ·È‰È¿ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·Ú¿ÙÔÏÌË Î·È ÚÈ„ÔΛӉ˘ÓË Û˘ÌÂÚÈÊÔÚ¿, Ë ÔÔ›· ·Ô‰›‰ÂÙ·È Û˘Ó‹ıˆ˜ ÛÙËÓ Ù¿ÛË ÁÈ· ·ÓÂÍ·ÚÙËÛ›· Î·È ÛÙËÓ ·Ó¿ÁÎË ÁÈ· ·fiÎÙËÛË Ó¤ˆÓ ÂÌÂÈÚÈÒÓ, Ô‰ËÁ› fï˜ Û˘¯Ó¿ Û ·Ù˘¯‹Ì·Ù· (11). OÈ ¤ÊË‚ÔÈ Â›Ó·È Ë ÌfiÓË ËÏÈÎȷ΋ ÔÌ¿‰· Ô˘ ·Ó Î·È Â›Ó·È ··ÏÏ·Á̤ÓË ·fi ¯ÚfiÓÈ· ÓÔÛ‹Ì·Ù·, ·ÚÔ˘ÛÈ¿˙ÂÈ ·˘ÍË̤ÓÔ˘˜ Ú˘ıÌÔ‡˜ ı·Ó¿ÙÔ˘, ÔÈ ÔÔ›ÔÈ Û·Ó Î‡ÚÈ· ·ÈÙ›· ¤¯Ô˘Ó ÙȘ ηÎÒÛÂȘ (6,12). ∆Ô Ê·ÈÓfiÌÂÓÔ ·˘Ùfi, Ô˘ Ù›ÓÂÈ Ó· ¿ÚÂÈ ÙË ÌÔÚÊ‹ ÂȉËÌ›·˜, ·ÔÙÂÏ› ·ÓÙÈΛÌÂÓÔ ÌÂϤÙ˘ ÛÙȘ ÚÔËÁ̤Ó˜ ¯ÒÚ˜, fiÔ˘ ÔÈ ÚÔÙ·ı›Û˜ ÛÙÚ·ÙËÁÈΤ˜ ÚfiÏ˄˘ ¤¯Ô˘Ó ‹‰Ë ıÂÙÈο ·ÔÙÂϤÛÌ·Ù·
496
14% 30%
∂ÚÁ·Û›·
19% 13%
10%
8%
8%
10%
8% 1%
10% 7%
4%
0% ∆ÚÔ¯·›·
™ÔÚ
∂ÚÁ·Û›· ∞˘ÙÔÎÙÔӛ˜ ∞ÏÎÔfiÏ
∂ÈÎfiÓ· 4. ∏ ‚·Ú‡ÙËÙ· Ù˘ ηٿÛÙ·Û˘ ÙÔ˘ ·ÛıÂÓÔ‡˜ ·ÍÈÔÏÔÁ‹ıËΠ̠‚¿ÛË ÙÔÓ ÙÚfiÔ Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘. ™ÙËÓ ÂÈÎfiÓ· Ê·›ÓÂÙ·È Ë ‚·Ú‡ÙËÙ· Û ۯ¤ÛË Ì ÙËÓ ·ÈÙ›· ÚfiÎÏËÛ˘ Ù˘ οΈÛ˘.
(9,13). ∞ÓÙ›ıÂÙ·, ÛÙË ¯ÒÚ· Ì·˜, ÙÔ Ì¤ÁÂıÔ˜ ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ‰ÂÓ ¤¯ÂÈ ·ÎfiÌË ÂÎÙÈÌËı› ÛˆÛÙ¿, Ì ·ÔÙ¤ÏÂÛÌ· Ó· Ô‰ËÁÔ‡ÓÙ·È ÔÈ ˘ÁÈ›˜ ¤ÊË‚ÔÈ Û˘¯Ó¿ Û ·Ó·Ëڛ˜ ‹ ·ÎfiÌË Î·È ÛÙÔ ı¿Ó·ÙÔ (14). OÈ ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜ Ô˘ ·ÊÔÚÔ‡Ó Î·ÎÒÛÂȘ ÂÈÎÂÓÙÚÒÓÔ˘Ó ÙÔ ÂӉȷʤÚÔÓ ÙÔ˘˜ ÛÙȘ ÌÈÎÚfiÙÂÚ˜ ËÏÈ˘, ·Ú·‚ϤÔÓÙ·˜ ¤ÙÛÈ ÙÔ ÛËÌ·ÓÙÈÎfi ÎÔÈÓˆÓÈÎfi Î·È ÔÈÎÔÓÔÌÈÎfi Úfi‚ÏËÌ· ÙˆÓ Î·ÎÒÛÂˆÓ ÛÙËÓ ÂÊ˂›· (9,14). ∞fi ÙË ÌÂϤÙË Ì·˜ ÚÔ·ÙÂÈ fiÙÈ ÙÔ 50% ÙˆÓ Î·ÎÒÛÂˆÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó Ì¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 19 ÂÙÒÓ, ·ÊÔÚ¿ ÂÊ‹‚Ô˘˜. ™ÙÔ Û˘Ì¤Ú·ÛÌ· ·˘Ùfi Ô‰ËÁÔ‡ÓÙ·È Î·È ¿ÏÏÔÈ ÂÚ¢ÓËÙ¤˜ (9,13,14). ª›· ¿ÏÏË ÎÔÈÓ‹ ‰È·›ÛÙˆÛË Â›Ó·È fiÙÈ Ô Ú˘ıÌfi˜ ηÎÒÛÂˆÓ ÛÙÔ˘˜ ¿ÚÚÂÓ˜ ÂÊ‹‚Ô˘˜ Â›Ó·È ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚÔ˜, Û˘ÁÎÚÈÓfiÌÂÓÔ˜ Ì ·˘ÙfiÓ ÙˆÓ ıËϤˆÓ (15,16), ÁÂÁÔÓfi˜ Ô˘ Ê·›ÓÂÙ·È Î·È ÛÙË ‰È΋ Ì·˜ ¤Ú¢ӷ, fiÔ˘ Ë ·Ó·ÏÔÁ›· ·ÚÚ¤ÓˆÓ ÚÔ˜ ı‹ÏÂȘ Â›Ó·È 2 ÚÔ˜ 1. ª›· ÂÚÌËÓ›· Ô˘ ÚÔÙ›ÓÂÙ·È ÁÈ· ÙÔ Ê·ÈÓfiÌÂÓÔ ·˘Ùfi Â›Ó·È fiÙÈ Ù· ·ÁfiÚÈ· ˘ÈÔıÂÙÔ‡Ó ÈÔ ÂÈıÂÙÈÎÔ‡˜ Î·È ÚÈ„ÔΛӉ˘ÓÔ˘˜ ÙÚfiÔ˘˜ Û˘ÌÂÚÈÊÔÚ¿˜ ηٿ ÙË ‰È·‰Èηۛ· Ù˘ ˆÚ›Ì·ÓÛ˘, ÚÔÛ·ıÒÓÙ·˜ Ó· ·ÓÙ·ÔÎÚÈıÔ‡Ó Î·Ï‡ÙÂÚ· ÛÙÔÓ ·Ú¯ËÁÈÎfi ÚfiÏÔ Ô˘ ÙÔ˘˜ ÂÈ‚¿ÏÏÂÈ Ë ÎÔÈÓˆÓ›·. ∂›Û˘, Û‡Ìʈӷ Ì ÙËÓ ¤ÚÂ˘Ó¿ Ì·˜ ·ÏÏ¿ Î·È Ì ٷ Û˘ÌÂÚ¿ÛÌ·Ù· ¿ÏÏˆÓ ÂÚ¢ÓËÙÒÓ, ÔÈ Î·ÎÒÛÂȘ Û˘Ì‚·›ÓÔ˘Ó Û˘Ó‹ıˆ˜ Û ÂÊ‹‚Ô˘˜ Ô˘ ˙Ô˘Ó ÛÙËÓ fiÏË. ∏ ‰È·›ÛÙˆÛË ·˘Ù‹ ÂÎÊÚ¿˙ÂÈ ÙËÓ
¶·È‰È·ÙÚÈ΋ 2001;64:493-498
Ú·ÁÌ·ÙÈÎfiÙËÙ·, ·ÊÔ‡ ÔÈ Ó¤ÔÈ Â›Ó·È ·Ó·ÁηṲ̂ÓÔÈ Ó· ÎÈÓÔ‡ÓÙ·È Î·È Ó· ‰Ú·ÛÙËÚÈÔÔÈÔ‡ÓÙ·È Ì¤Û· Û ¤Ó· ÔÏ˘Û‡¯Ó·ÛÙÔ Î·È ÂÈΛӉ˘ÓÔ ÂÚÈ‚¿ÏÏÔÓ, ÂÎı¤ÙÔÓÙ·˜ ¤ÙÛÈ ÙÔÓ Â·˘Ùfi ÙÔ˘˜ Û˘Ó¯Ҙ Û ΛӉ˘ÓÔ. ŒÓ· ÛËÌ·ÓÙÈÎfi ÎÂÊ¿Ï·ÈÔ ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ÙˆÓ Î·ÎÒÛÂˆÓ ·ÔÙÂÏ› Ô Î·ıÔÚÈÛÌfi˜ ÙÔ˘ ·ÈÙ›Ô˘ Ô˘ ÙȘ ÚÔηÏ›. OÈ Î·ÎÒÛÂȘ Û˘Ì‚·›ÓÔ˘Ó fiÙ·Ó Ù· ÛÙÔȯ›· ÍÂÓÈÛÙ‹˜, ·Ú¿ÁÔÓÙ·˜ Î·È ÂÚÈ‚¿ÏÏÔÓ Û˘Ì›ÙÔ˘Ó Û ̛· Û˘ÁÎÂÎÚÈ̤ÓË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹. °È· ¤Ó· ·ÚÎÂÙ¿ ÌÂÁ¿ÏÔ ·ÚÈıÌfi ηÎÒÛˆÓ, Ù· ÛÙÔȯ›· ·˘Ù¿ ÌÔÚÔ‡Ó Ó· ÚÔ‚ÏÂÊıÔ‡Ó Î·È Ó· ·ÔÙÚ·Ô‡Ó. ŒÙÛÈ, Ë ÌÂϤÙË Ù˘ ·ÈÙ›·˜ Ù˘ οΈÛ˘ Â›Ó·È ÎÚÈÙÈ΋˜ ÛËÌ·Û›·˜ ÁÈ· ÙÔÓ ÂÚÈÔÚÈÛÌfi ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Î·ÎÒÛˆÓ. ∞ӷχÔÓÙ·˜ Ù· ‰Â‰Ô̤ӷ ÁÈ· ÙȘ ·Èٛ˜ οΈÛ˘ ÛÙËÓ ÂÚÈÔ¯‹ Ì·˜, ·Ú·ÙËÚԇ̠fiÙÈ ÙËÓ ÚÒÙË ı¤ÛË Î·Ù¤¯Ô˘Ó Ù· ·Ù˘¯‹Ì·Ù· Ô˘ Û˘Ì‚·›ÓÔ˘Ó ÛÙËÓ ¿ıÏËÛË Î·È ÙÔ ·È¯Ó›‰È (ÛÔÚ). ™ÙÔ ·ÔÙ¤ÏÂÛÌ· ·˘Ùfi Ô‰ËÁÔ‡ÓÙ·È Î·È ¿ÏÏÔÈ ÂÚ¢ÓËÙ¤˜, ÔÈ ÔÔ›ÔÈ Î·Ù·Ù¿ÛÛÔ˘Ó Ù· ÛÔÚ Û·Ó ÙËÓ ÚÒÙË ‹ ÙË ‰Â‡ÙÂÚË ·ÈÙ›· ÚfiÎÏËÛ˘ ηÎÒÛÂˆÓ ÛÙËÓ ÂÊ˂›· (9,15,17). Ÿˆ˜ ÚÔ·ÙÂÈ ·fi ÙËÓ ¤ÚÂ˘Ó¿ Ì·˜, ÛÙËÓ ËÏÈΛ· ÙˆÓ 13-16 ÂÙÒÓ, Ù· ·Ù˘¯‹Ì·Ù· ÛÙ· ÛÔÚ ·ÔÙÂÏÔ‡Ó ÛÙ·ıÂÚ¿ ÙË ‚·ÛÈÎfiÙÂÚË ·ÈÙ›· οΈÛ˘, ÂÓÒ ÌÂÙ¿ ÙËÓ ËÏÈΛ· ·˘Ù‹ ÔÈ Ú˘ıÌÔ› ÌÂÈÒÓÔÓÙ·È. ™˘Ó‹ıˆ˜ ÔÈ ÙÚ·˘Ì·ÙÈÛÌÔ› Â›Ó·È Ì¤ÙÚÈ·˜ ‚·Ú‡ÙËÙ·˜, ·ÊÔÚÔ‡Ó Ù· ¿ÎÚ· Î·È ÛÙÔ ÌÂÁ·Ï‡ÙÂÚfi ÙÔ˘˜ ÔÛÔÛÙfi ‰ÂÓ ¯Ú‹˙Ô˘Ó ÂȉÈ΋˜ ÓÔÛËÏ›·˜ (17). øÛÙfiÛÔ, Â›Ó·È ÛËÌ·ÓÙÈΤ˜ ÔÈ ÎÔÈÓˆÓÈΤ˜ Î·È ÔÈÎÔÓÔÌÈΤ˜ ÂÈÙÒÛÂȘ ·fi Ù· ·Ù˘¯‹Ì·Ù· ·˘Ù¿, ·ÊÔ‡ ÌÂÙ·ÊÚ¿˙ÔÓÙ·È Û ·ÒÏÂÈ· Û¯ÔÏÈÎÔ‡ ¯ÚfiÓÔ˘, ·Ô¯‹ ·fi ÙËÓ ÂÚÁ·Û›· ÙˆÓ ÁÔÓ¤ˆÓ Î·È ÎfiÛÙÔ˜ ÁÈ· ÙËÓ ·ÔηٿÛÙ·ÛË ÙÔ˘ ·ÛıÂÓÔ‡˜. ∆· ÙÚÔ¯·›· Û˘Ì‚¿ÓÙ· ·ÔÙÂÏÔ‡Ó ÙË ‰Â‡ÙÂÚË ÛËÌ·ÓÙÈ΋ ·ÈÙ›· ηÎÒÛÂˆÓ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ Ù˘ ÂÚÈÔ¯‹˜ Ì·˜. ¶·Ú·ÙËÚԇ̛̠· ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÙˆÓ Î·ÎÒÛÂˆÓ ·˘ÙÒÓ ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 16 ÂÙÒÓ Î·È ¿Óˆ, Ë ÔÔ›· ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È ÌÂٷ͇ ¿ÏÏˆÓ Î·È ÛÙËÓ Â˘Ú›· ¯Ú‹ÛË Ì˯·ÓÔΛÓËÙˆÓ ‰È·ÎψÓ, fiˆ˜ ˘ÔÛÙËÚ›˙Ô˘Ó Î·È ¿ÏÏÔÈ ÂÚ¢ÓËÙ¤˜ (18). OÈ Ì˯·Ó¤˜ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚÔ ·fi ÙÔ˘˜ Ó¤Ô˘˜ ·˘Ù‹˜ Ù˘ ËÏÈΛ·˜, ÏÂÈÙÔ˘ÚÁÒÓÙ·˜ ·ÊÂÓfi˜ ˆ˜ ̤ÛÔ ÚÔ‚ÔÏ‹˜ Î·È ·Ó·ÁÓÒÚÈÛ˘ Î·È ·ÊÂÙ¤ÚÔ˘ ˆ˜ ‡¯ÚËÛÙÔ Ì¤ÛÔ ÌÂÙ·ÊÔÚ¿˜. ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ ·Ô˘Û›· ΢ÎÏÔÊÔÚȷ΋˜ ·ÁˆÁ‹˜ Î·È ÙËÓ Î·Î‹ ηٿÛÙ·ÛË ÙÔ˘ Ô‰ÈÎÔ‡ ‰ÈÎÙ‡Ô˘, Û˘Ì‚¿ÏÏÂÈ ÛÙË Ú·Á‰·›· ·‡ÍËÛË ÙˆÓ ÙÚÔ¯·›ˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô˘ Û˘¯Ó¿ ·Ô‚·›ÓÔ˘Ó ÌÔÈÚ·›·, fiˆ˜ ¤¯ÂÈ ‰È·ÈÛÙˆı› Î·È Û ¿ÏϘ ¯ÒÚ˜ (4,5,8). ∏ ηÙËÁÔÚ›· ·˘Ù‹ ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô‰ËÁ› Û ÈÔ ÛÔ‚·Ú¤˜ ηÎÒÛÂȘ (19,20). ÷ڷÎÙËÚÈÛÙÈο ·Ó·Ê¤ÚÔ˘Ì fiÙÈ ÙÔ 6,8% ÙˆÓ ÙÚÔ¯·›ˆÓ ·Ù˘¯ËÌ¿ÙˆÓ Ô‰ËÁ› Û ‚·ÚȤ˜ ∫∂∫,
Paediatriki 2001;64:493-498
ÂÓÒ ·ÔÙÂÏ› ÙËÓ ÚÒÙË ·ÈÙ›· ı·Ó¿ÙÔ˘ ·fi ·Ù˘¯‹Ì·Ù· (8,13). ª›· ÔÌ¿‰· ·Ù˘¯ËÌ¿ÙˆÓ, ÛÙËÓ ÔÔ›· ‰ÂÓ ¤¯ÂÈ ‰Ôı› Ë ‰¤Ô˘Û· ÚÔÛÔ¯‹, Â›Ó·È ·˘Ù¿ Ô˘ Û˘Ì‚·›ÓÔ˘Ó Î·Ù¿ ÙËÓ ÂÚÁ·Û›·. OÈ ¤ÊË‚ÔÈ, Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È fiÏÔ Î·È ÂÚÈÛÛfiÙÂÚÔ Û·Ó ÊıËÓfi ÂÚÁ·ÙÈÎfi ‰˘Ó·ÌÈÎfi, ‰È·ı¤ÙÔ˘Ó ÙË ÛˆÌ·ÙÈ΋ ‰È¿Ï·ÛË ÙˆÓ ÂÓËϛΈÓ, ¯ˆÚ›˜ fï˜ Ó· ¤¯Ô˘Ó ÙËÓ ·Ó¿ÏÔÁË ÂÌÂÈÚ›· Î·È ˆÚÈÌfiÙËÙ·, Ì ·ÔÙ¤ÏÂÛÌ· Ó· Â›Ó·È ÂÈÚÚ›˜ Û ·Ù˘¯‹Ì·Ù· Ô˘ Û˘Ì‚·›ÓÔ˘Ó ÛÙÔÓ ÂÚÁ·ÛÈ·Îfi ¯ÒÚÔ. ™ÙËÓ ÂÚÈÔ¯‹ Ì·˜, Ù· ÂÚÁ·ÙÈο ·Ù˘¯‹Ì·Ù· ·ÔÙÂÏÔ‡Ó ÙËÓ ÙÚ›ÙË Î·Ù¿ ÛÂÈÚ¿ ·ÈÙ›· ηÎÒÛÂˆÓ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ Î·È Â›Ó·È ÈÔ Û˘¯Ó¿ ÌÂٷ͇ ÙˆÓ ¯·ÌËÏfiÙÂÚˆÓ ÔÈÎÔÓÔÌÈο Ù¿ÍÂˆÓ (∞ı›ÁÁ·ÓÔÈ 23,1%, ªÔ˘ÛÔ˘ÏÌ¿ÓÔÈ 20,8%), fiÔ˘ ÙÔ ÔÛÔÛÙfi ÙˆÓ ÂÚÁ·˙fiÌÂÓˆÓ ÂÊ‹‚ˆÓ Â›Ó·È ˘„ËÏfiÙÂÚÔ. ™Â ·˘Ù‹ ÙËÓ Î·ÙËÁÔÚ›·, ›Û˘, Û˘ÓËı¤ÛÙÂÚÔÈ Â›Ó·È ÔÈ ÙÚ·˘Ì·ÙÈÛÌÔ› ÙˆÓ ¿ÎÚˆÓ Î·È È‰È·›ÙÂÚ· Ù· ηٿÁÌ·Ù· (21). ∏ ¤Ú¢ӷ ÁÈ· ÙȘ ηÎÒÛÂȘ ÛÙËÓ ÂÊ˂›· ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ÙȘ ‰ËÏËÙËÚÈ¿ÛÂȘ ·fi Ï‹„Ë ‰È·ÊfiÚˆÓ Ô˘ÛÈÒÓ. ∞Ó Î·È Û ÔÏϤ˜ ÌÂϤÙ˜ ÙÔÓ›˙ÂÙ·È Ë ·˘Í·ÓfiÌÂÓË ¯Ú‹ÛË Ó·ÚΈÙÈÎÒÓ Ô˘ÛÈÒÓ ÌÂٷ͇ ÙˆÓ ÂÊ‹‚ˆÓ, ·fi ÙË ‰È΋ Ì·˜ ¤Ú¢ӷ ‰ÂÓ ÚԤ΢„·Ó Ù¤ÙÔÈ· ÛÙÔȯ›·. ™˘ÓËı¤ÛÙÂÚË Â›Ó·È Ë Î·Ù·Ó¿ÏˆÛË ·ÏÎÔfiÏ Ô˘ ¢ı‡ÓÂÙ·È ÁÈ· ÙÔ 4,3% ÙˆÓ ÂÊ‹‚ˆÓ Ô˘ ÚÔÛ¤Ú¯ÔÓÙ·È ÛÙȘ ∞’ µÔ‹ıÂȘ Î·È Â˘Ú›ÛÎÔÓÙ·È Û ηٿÛÙ·ÛË Ì¤ı˘. OÈ ¤ÊË‚ÔÈ ·˘ÙÔ› ·Ó‹ÎÔ˘Ó ·ÔÎÏÂÈÛÙÈο ÛÙËÓ ÔÌ¿‰· ÙˆÓ ÃÚÈÛÙÈ·ÓÒÓ. ∆Ô Ê·ÈÓfiÌÂÓÔ ·˘Ùfi ·Ú·ÙËÚÂ›Ù·È Û ÔÏϤ˜ ÚÔËÁ̤Ó˜ ÎÔÈӈӛ˜ Î·È Èı·ÓfiÓ Ó· ÔÊ›ÏÂÙ·È ÛÙËÓ ÂÈı˘Ì›· ÙˆÓ ÂÊ‹‚ˆÓ Ó· ·Ô‰Ú¿ÛÔ˘Ó ·fi ‰˘Û¿ÚÂÛÙ˜ ηٷÛÙ¿ÛÂȘ ‹ Ó· Á›ÓÔ˘Ó ·Ô‰ÂÎÙÔ› ·fi ÙÔ˘˜ Ê›ÏÔ˘˜ ÙÔ˘˜ (22). ™ÙȘ ηÎÒÛÂȘ ·Ó‹ÎÔ˘Ó Î·È ÔÈ ·fiÂÈÚ˜ ·˘ÙÔÎÙÔÓ›·˜ Ô˘ ·ÔÙÂÏÔ‡Ó, Û‡Ìʈӷ Ì ¿ÏϘ ÌÂϤÙ˜, ÙËÓ ÙÚ›ÙË ·ÈÙ›· ı·Ó¿ÙÔ˘ ÛÙȘ ËÏÈ˘ 15-19 ¯ÚfiÓˆÓ (22). ™ÙËÓ ¤ÚÂ˘Ó¿ Ì·˜ ηٷÁÚ¿„·Ì 95 ÂÊ‹‚Ô˘˜ (57 ÎÔÚ›ÙÛÈ· Î·È 38 ·ÁfiÚÈ·) Ô˘ ÚÔÛ¿ıËÛ·Ó Ó· ı¤ÛÔ˘Ó Ù¤ÏÔ˜ ÛÙË ˙ˆ‹ ÙÔ˘˜. ™˘ÓËı¤ÛÙÂÚË Ì¤ıÔ‰Ô˜ ‹Ù·Ó Ë Ï‹„Ë Ê·Ú̿ΈÓ. ∞fi ·˘ÙÔ‡˜ η٤ÏËÍ·Ó ‰‡Ô ·ÁfiÚÈ· Ô˘ ¯ÚËÛÈÌÔÔ›ËÛ·Ó ÈÔ ‚›·È˜ ÌÂıfi‰Ô˘˜ (··Á¯ÔÓÈÛÌfi˜, ˘ÚÔ‚ÔÏÈÛÌfi˜). Ÿˆ˜ ÚÔ·ÙÂÈ ·fi ÙËÓ ¤ÚÂ˘Ó¿ Ì·˜, Ù· ÎÔÚ›ÙÛÈ· ÚÔËÁÔ‡ÓÙ·È ·ÚÈıÌËÙÈο ÛÙȘ ·fiÂÈÚ˜, ·ÏÏ¿ Ù· ·ÁfiÚÈ· Â›Ó·È ÂΛӷ Ô˘ ÂÈÙ˘Á¯¿ÓÔ˘Ó (22). ¢˘ÛÙ˘¯Ò˜ ÛÙËÓ ÂÊ˂›· Â›Ó·È ‰˘Ó·ÙfiÓ Ë Û˘Ó·ÈÛıËÌ·ÙÈ΋ ·ÛÙ¿ıÂÈ· Î·È Ë „˘¯È΋ ÊfiÚÙÈÛË Ó· Ô‰ËÁ‹ÛÔ˘Ó Û ηٿÛÙ·ÛË ·˘ÙÔÎÙÔÓÈÎÔ‡ ȉ·ÛÌÔ‡ (23). ™ÙËÓ ∂ÏÏ¿‰·, Ù· ÔÛÔÛÙ¿ ·˘ÙÔÎÙÔÓÈÒÓ Â›Ó·È È‰È·›ÙÂÚ· ¯·ÌËÏ¿, Û˘ÁÎÚÈÓfiÌÂÓ· Ì ·˘Ù¿ ¿ÏÏˆÓ ¯ˆÚÒÓ Î·È ·ÊÔÚÔ‡Ó Î˘Ú›ˆ˜ ÂÊ‹‚Ô˘˜ ·ÁÚÔÙÈÎÒÓ ÂÚÈÔ¯ÒÓ (23). ™ÙË ‰È΋ Ì·˜ ÌÂϤÙË, ÔÈ ·fiÂÈÚ˜
497
¶·È‰È·ÙÚÈ΋ 2001;64:493-498
·˘ÙÔÎÙÔÓ›·˜ ‹Ù·Ó Û˘¯ÓfiÙÂÚ˜ ÌÂٷ͇ ÙˆÓ ıËϤˆÓ ªÔ˘ÛÔ˘ÏÌ¿ÓˆÓ (24 ÎÔÚ›ÙÛÈ·) Èı·ÓfiÓ ÏfiÁˆ ÙÔ˘ ηٷÈÂÛÙÈÎÔ‡ Î·È ÂÚÈÔÚÈÛÙÈÎÔ‡ ÙÚfiÔ˘ ˙ˆ‹˜ Ô˘ ÂÈ‚¿ÏÏÂÈ ·˘Ù‹ Ë ÌÂÈÔÓfiÙËÙ· ÛÙȘ Á˘Ó·›Î˜. ™˘ÌÂÚ¿ÛÌ·Ù· ∏ ÌÂϤÙË ÙˆÓ Î·ÎÒÛÂˆÓ ÛÙËÓ ÂÊË‚È΋ ËÏÈΛ· Û˘ÓÈÛÙ¿ ¤Ó· ¢ڇ ‰›Ô ¤Ú¢ӷ˜ Û ‰ÈÂıÓ¤˜ ›‰Ô. ¢˘ÛÙ˘¯Ò˜, ·Ó Î·È ÙÔ Úfi‚ÏËÌ· ÙˆÓ Î·ÎÒÛÂˆÓ ÛÙËÓ ¯ÒÚ· Ì·˜ ¤¯ÂÈ ‹‰Ë Ï¿‚ÂÈ ·ÓËÛ˘¯ËÙÈΤ˜ ‰È·ÛÙ¿ÛÂȘ, ‰ÂÓ ¤¯Ô˘Ó ·ÎfiÌ· ηٷÚÙÈÛÙ› ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Ô˘ ı· ÚÔÙ›ÓÔ˘Ó Ì›· ÛÊ·ÈÚÈ΋ ÚÔÛ¤ÁÁÈÛË Î·È ı· ‰ÒÛÔ˘Ó ÙË ‰˘Ó·ÙfiÙËÙ· ÛÙËÓ ÔÏÈÙ›· Ó· ۯ‰ȿÛÂÈ ¤Ó· ÔÏÔÎÏËڈ̤ÓÔ Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfi ÚfiÁÚ·ÌÌ· ÚfiÏ˄˘. ∏ ÂÌÂÈÚ›· ¿ÏÏˆÓ ¯ˆÚÒÓ Ì¿˜ ‰È‰¿ÛÎÂÈ fiÙÈ ÌÔÚÔ‡Ó Ó· Á›ÓÔ˘Ó ÛËÌ·ÓÙÈο Ú·ÎÙÈο ‚‹Ì·Ù· ̤۷ ·fi ÂÎ·È‰Â˘ÙÈΤ˜, ‰ÈÔÈÎËÙÈΤ˜ Î·È ÓÔÌÔıÂÙÈΤ˜ ·ÚÂÌ‚¿ÛÂȘ. ∏ ÂÎ·È‰Â˘ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Ê·›ÓÂÙ·È Ó· ·Ô‰›‰ÂÈ ÂÚÈÛÛfiÙÂÚÔ fiÙ·Ó ·Â˘ı‡ÓÂÙ·È Û ·È‰È¿ ÚÔÂÊË‚È΋˜ ËÏÈΛ·˜, fiÔ˘ ÔÈ Î·Ù¿ÏÏËÏÔÈ ·È‰·ÁˆÁÈÎÔ› ¯ÂÈÚÈÛÌÔ› ÌÔÚÔ‡Ó Ó· ÂȉڿÛÔ˘Ó ıÂÙÈο ‰È·ÌÔÚÊÒÓÔÓÙ·˜ Û˘ÁÎÚÔÙË̤ÓÔ˘˜ ÙÚfiÔ˘˜ Û˘ÌÂÚÈÊÔÚ¿˜. °È· ÙÔ˘˜ ÂÊ‹‚Ô˘˜, Ë ÚfiÏË„Ë ÂÚÈÔÚ›˙ÂÙ·È ÛÙË ‰È·ÌfiÚʈÛË ·ÛÊ·ÏÔ‡˜ ÂÚÈ‚¿ÏÏÔÓÙÔ˜, ÒÛÙ ӷ ÂÏ·¯ÈÛÙÔÔÈËıÔ‡Ó ÔÈ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘. ¶¤Ú· ·fi ·˘Ù¤˜ ÙȘ ÛÙÚ·ÙËÁÈΤ˜ ÚfiÏ˄˘, ÁÈ· ÙÔ Û¯Â‰È·ÛÌfi ÙˆÓ ÔÔ›ˆÓ ÙË ‚·ÛÈ΋ ¢ı‡ÓË ¤¯ÂÈ Ë ÔÏÈÙ›·, Ë ÔÈÎÔÁ¤ÓÂÈ· Î·È Ë ÎÔÈÓˆÓ›· ı· Ú¤ÂÈ Ó· ‰È·‰Ú·Ì·Ù›ÛÔ˘Ó Ô˘ÛÈ·ÛÙÈÎfi ÚfiÏÔ ÛÙË ‰ËÌÈÔ˘ÚÁ›· ÂÚÂÈÛÌ¿ÙˆÓ, ÒÛÙÂ Ô Â˘¿ÏˆÙÔ˜ ¤ÊË‚Ô˜ Ó· ‚Ú›ÛÎÂÈ ÂÔÈÎÔ‰ÔÌËÙÈΤ˜ ‰ÈÂÍfi‰Ô˘˜ ÛÙ· ‰ÈÏ‹ÌÌ·Ù¿ ÙÔ˘, ÌÂÈÒÓÔÓÙ·˜ ¤ÙÛÈ ÙËÓ Èı·ÓfiÙËÙ· Ó· ¯¿ÛÂÈ ÙËÓ ·˘ÙÔÂÎÙ›ÌËÛ‹ ÙÔ˘ Î·È Ó· Ô‰ËÁËı› ÛÙË ¯Ú‹ÛË ÂıÈÛÙÈÎÒÓ Ô˘ÛÈÒÓ, ÛÙËÓ ˘ÈÔı¤ÙËÛË ‚›·È˘ Û˘ÌÂÚÈÊÔÚ¿˜ ‹ Û ٿÛÂȘ ·˘ÙÔÎÙÔÓ›·˜. ¶ÈÛÙ‡ԢÌ fiÙÈ fiÛÔ ·˘Í¿ÓÔ˘Ó ÔÈ ÁÓÒÛÂȘ Ì·˜ Á‡Úˆ ·fi ÙËÓ ÂȉËÌÈÔÏÔÁ›· ÙˆÓ Î·ÎÒÛÂˆÓ ÛÙËÓ ÂÊ˂›·, ı· Ì·˜ ‰›ÓÂÙ·È Ë ‰˘Ó·ÙfiÙËÙ· Ó· ÂÊ·ÚÌfi˙Ô˘Ì ·ÔÙÂÏÂÛÌ·ÙÈο ÌÔÓ٤Ϸ ÚfiÏ˄˘, ÛÙԯ‡ÔÓÙ·˜ ÛÙÔÓ ÂÚÈÔÚÈÛÌfi ÙˆÓ ·Ù˘¯ËÌ¿ÙˆÓ.
Paediatriki 2001;64:493-498
Emerg Med 1996;3:213-220. 5. Foltin E. Pediatric and adolescent accident victims (ICD-E 800 to 829) in Austria 1980 to 1989. Unfallchirurgie 1996;22:99-109. 6. Fraser JJ Jr. Nonfatal injuries in adolescents: United States, 1988. J Adolesc Health 1996;19:166-170. 7. Huth O. Accident incidence at various ages. Versicherungsmedizin 1997;49:36-40. 8. Kelton GM, Shank JC. Adolescent injury and death: The plagues of accident, self-infliction and violence. Prim Care 1998;25:163-179. 9. Millar WJ. Accidents in Canada, 1988 and 1993. Health Rep 1995;7:7-16. 10. ŒÎıÂÛË Ù˘ ‰È·ÎÔÌÌ·ÙÈ΋˜ ∫ÔÈÓÔ‚Ô˘Ï¢ÙÈ΋˜ ∂ÈÙÚÔ‹˜ ÁÈ· ÙË ÌÂϤÙË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ÙˆÓ ÙÚÔ¯·›ˆÓ ·Ù˘¯ËÌ¿ÙˆÓ. ∞Ú›ÏÈÔ˜ 1996: ∆fiÌÔÈ π-πππ. 11. ∞Ì·Ù˙fiÁÏÔ˘ °. OÈÎÔÁ¤ÓÂÈ· Î·È ¤ÊË‚Ô˜. ¶·È‰/ÎË µ. ∂ÏÏ¿‰Ô˜ 1995;7:278-283. 12. Pelech L, Roth Z. Schoolchildren and accidents. Rozhl Chir 1997;76:400-404. 13. WHO. World Health Statistics (Annual) Geneva: WHO; 1980-1994. 14. ¶ÂÙÚ›‰Ô˘ ∂, ∆ÚȯfiÔ˘ÏÔ˜ ¢. ∆Ô ∂ıÓÈÎfi Úfi‚ÏËÌ· ˘Á›·˜ Ù˘ ∂ÏÏ¿‰Ô˜: ∆ÚÔ¯·›· ·Ù˘¯‹Ì·Ù·. ¶·È‰È·ÙÚÈ΋ 1998;61:31-35. 15. Behrman RE, ed. Nelson Textbook of Pediatrics. 14th ed. Philadelphia: WB Saunders; 1992. p. 524. 16. Bass JL, Gallaher SS, Mehta KA. Injuries in teenagers and young adults. Pediatr Clin N Am 1975;32:59-69. 17. Uitenbroek DG. Sports, exercise, and other causes of injuries: results of a population survey. Res Q Exerc Sport 1996;67:380-385. 18. Montella N, Borrell C, Brugal MT, Plasencia A. Mortality trends in the young people of the city of Barcelona, 19831993. Med Clin (Barc) 1997;108:241-247. 19. Emanuelson I, v Wendt L. Epidemiology of traumatic brain injury in children and adolescents in southwestern Sweden. Acta Paediatr 1997;86:730-735. 20. Hillier SL, Hiller JE, Metzer J. Epidemiology of traumatic brain injury in South Australia. Brain Inj 1997;11:649-659. 21. Dufort VM, Kotch JB, Marshall SW, Waller AE, Langley JD. Occupational injuries among adolescents in Dunedin, New Zealand, 1990-1993. Ann Emerg Med 1997;30:266273. 22. Behrman RE, ed. Nelson Textbook of Pediatrics. 14th ed. Philadelphia: WB Saunders; 1992. p. 526. 23. ∆Û›ÎÔ˘Ï·˜ π. ∆· ·›ÙÈ· Ù˘ ·˘ÙÔÎÙÔÓ›·˜ ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ·. ¶·È‰/΋ µ. ∂ÏÏ¿‰Ô˜ 1995;7:273-277.
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CASE REPORT
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∆oxic shock syndrome in children with burns. Report of 2 cases M. Narlioglou, P. Kalabalikis, G. Briassoulis, A. Hatzis
¶ÂÚ›ÏË„Ë: ∆Ô Û‡Ó‰ÚÔÌÔ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜ ·ÔÙÂÏ› ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ÓfiÛÔ Î·È ÔÊ›ÏÂÙ·È ÛÙË ‰Ú¿ÛË Â͈ÙÔÍÈÓÒÓ Ô˘ ·Ú¿ÁÔÓÙ·È ·fi ÙÔ ¯Ú˘Û›˙ÔÓÙ· ÛÙ·Ê˘ÏfiÎÔÎÎÔ ‹ ÙÔÓ ˘ÔÁfiÓÔ ÛÙÚÂÙfiÎÔÎÎÔ. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ¤Ó· ¢ڇ Ê¿ÛÌ· ÎÏÈÓÈÎÒÓ ÂΉËÏÒÛˆÓ, ÌÂٷ͇ ÙˆÓ ÔÔ›ˆÓ Â›Ó·È o ˘ÚÂÙfi˜, ÙÔ ÂÍ¿ÓıËÌ· Î·È Ë Î·Ù·ÏËÍ›·. ¶ÂÚÈÁÚ¿ÊÔÓÙ·È 2 ÂÚÈÙÒÛÂȘ ·È‰ÈÒÓ ËÏÈΛ·˜ 9 Î·È 18 ÌËÓÒÓ Ì ÂÁη‡Ì·Ù· 2% Î·È 35% Ù˘ ÂÈÊ¿ÓÂÈ·˜ ÙÔ˘ ÛÒÌ·ÙÔ˜ ·ÓÙ›ÛÙÔȯ·, Ô˘ ·ÚÔ˘Û›·Û·Ó Û‡Ó‰ÚÔÌÔ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜. ∞fi ÙȘ ÂÁη˘Ì·ÙÈΤ˜ ÂÈÊ¿ÓÂȘ ÙˆÓ 2 ·È‰ÈÒÓ ·ÔÌÔÓÒıËΠ¯Ú˘Û›˙ˆÓ ÛÙ·Ê˘ÏfiÎÔÎÎÔ˜. ∞fi ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜, Ô˘ ÂÎÙfi˜ ÙˆÓ ¿ÏÏˆÓ ÂΉËÏÒÛÂˆÓ ·ÚÔ˘Û›·Û·Ó Î·È Û‡Ó‰ÚÔÌÔ ÔÍ›·˜ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜ (ARDS), Ô ¤Ó·˜ η٤ÏËÍÂ.
Abstract: ∆oxic shock syndrome (TSS) is a multisystem illness that is caused by exotoxins produced by Staphylococcus aureus or Streptococcus pyogenes. It is characterized by a broad spectrum of clinical manifestations that include fever, rash and shock. We describe two children, aged 9 and 18 months, with burns of 2 and 35 per cent of total body surface area respectively, who developed TSS. Staphylococcus aureus was isolated from burn swabs from both children. Of our patients who, in addition to the TSS manifestations, developed adult respiratory distress syndrome, one succumbed to the illness.
§¤ÍÂȘ ÎÏÂȉȿ: Û‡Ó‰ÚÔÌÔ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜, ÛÙ·Ê˘ÏfiÎÔÎÎÔ˜, ηٷÏËÍ›·, ¤Áη˘Ì·.
∫ey words: toxic shock Staphylococcus, shock, burn.
∂ÈÛ·ÁˆÁ‹ ∆Ô Û‡Ó‰ÚÔÌÔ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜ (™∆∫) ·Ú·ÙËÚ‹ıËÎÂ Î·È ÂÚÈÁÚ¿ÊËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÙÔ 1978 ·fi ÙÔÓ Todd, Û 7 ·È‰È¿ ËÏÈΛ·˜ 8-17 ÂÙÒÓ (1). ∆Ô 1979 ‰fiıËΠÌÂÁ¿ÏË ‰ËÌÔÛÈfiÙËÙ· ÛÙÔ Û‡Ó‰ÚÔÌÔ, ‰ÈfiÙÈ Û¯ÂÙ›ÛÙËΠ̠ÙË ¯Ú‹ÛË ÎÔÏÈÎÒÓ Ù·ÌfiÓ ÛÙȘ Á˘Ó·›Î˜ ·Ó··Ú·ÁˆÁÈ΋˜ ËÏÈΛ·˜ (2). TÔ ™∆∫ Â›Ó·È ÔÏ˘Û˘ÛÙËÌ·ÙÈÎfi ÓfiÛËÌ·, ¯·Ú·ÎÙËÚÈ˙fiÌÂÓÔ ·fi ¤Ó· ÂÙÂÚÔÁÂÓ¤˜ Ê¿ÛÌ· ÎÏÈÓÈÎÒÓ ÂΉËÏÒÛÂˆÓ Î·È ÔÊ›ÏÂÙ·È ÛÙË ‰Ú¿ÛË Â͈ÙÔÍÈÓÒÓ Ô˘ ·Ú¿ÁÔÓÙ·È Î˘Ú›ˆ˜ ·fi ÙÔ ¯Ú˘Û›˙ÔÓÙ· ÛÙ·Ê˘ÏfiÎÔÎÎÔ ·ÏÏ¿ Î·È ·fi ÙÔÓ ˘ÔÁfiÓÔ ÛÙÚÂÙfiÎÔÎÎÔ (3) (¶›Ó·Î·˜ 1). OÈ Û˘ÁÎÂÎÚÈ̤Ó˜ ÙÔ͛Ә, ‰ÚÒÓÙ·˜ ˆ˜ ˘ÂÚ·ÓÙÈÁfiÓ·, ‰ÈÂÁ›ÚÔ˘Ó ¤Ó· ÌÂÁ¿ÏÔ
·ÚÈıÌfi ∆-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ, Ô˘ Ì ÙË ÛÂÈÚ¿ ÙÔ˘˜ ÚÔηÏÔ‡Ó ·Ú·ÁˆÁ‹ ΢ÙÙ·ÚÔÎÈÓÒÓ, ÔÈ Ôԛ˜ ¢ı‡ÓÔÓÙ·È ÁÈ· ÙȘ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Î·È Î˘Ú›ˆ˜ ÁÈ· ÙËÓ Î·Ù·ÏËÍ›· (4,5). ∏ ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ ‰ÂÓ ÂÍ·ÚÙ¿Ù·È Ô‡Ù ÂȂ‚·ÈÒÓÂÙ·È ·fi ÙËÓ ·ÔÌfiÓˆÛË ÌÈÎÚÔ‚›Ô˘, ÙËÓ ·Ó›¯Ó¢ÛË Â͈ÙÔÍÈÓÒÓ ‹ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Â͈ÙÔÍÈÓÒÓ, ·ÏÏ¿ Ù›ıÂÙ·È ·ÔÎÏÂÈÛÙÈο Ì ÎÏÈÓÈο ÎÚÈÙ‹ÚÈ· (6,7) (¶›Ó·Î·˜ 2). ¢È·ÎÚ›ÓÔ˘Ì ‰‡Ô ÌÔÚʤ˜ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘: ÙËÓ ÂÌÌËÓÔÚÚ˘Ûȷ΋ ÌÔÚÊ‹, Ë ÔÔ›· ·Ú·ÙËÚÂ›Ù·È Û Á˘Ó·›Î˜ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡Ó ÂÓ‰ÔÎÔÏÈο Ù·ÌfiÓ Î·Ù¿ ÙËÓ ¤ÌÌËÓÔ Ú‡ÛË Î·È ÙË ÌË ÂÌÌËÓÔÚÚ˘Ûȷ΋ ÌÔÚÊ‹, Ô˘ ÚÔÛ‚¿ÏÏÂÈ Û˘Ó‹ıˆ˜ ·È‰È¿ ηÈ
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Division of Pediatric Intensive Care Children’s Hospital “St. Sophia”, Athens
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¶·È‰È·ÙÚÈ΋ 2001;64:499-503
¶›Ó·Î·˜ 1. E͈ÙÔ͛Ә Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ· ÙÔ Û‡Ó‰ÚÔÌÔ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜ ∂͈ÙÔÍ›ÓË ªÈÎÚÔÔÚÁ·ÓÈÛÌfi˜ ∆ÔÍ›ÓË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÃÚ˘Û›˙ˆÓ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜ (TSST-1) ÛÙ·Ê˘ÏfiÎÎÔÎÔ˜ ∂ÓÙÂÚÔÙÔÍ›ÓË ∞ (SEA) >> ∂ÓÙÂÚÔÙÔÍ›ÓË µ (S∂µ) >> ∂ÓÙÂÚÔÙÔÍ›ÓË C (SEC) (SEC1 - SEC2 - SEC3) >> ∂ÓÙÂÚÔÙÔÍ›ÓË D (SED) >> ∂ÓÙÂÚÔÙÔÍ›ÓË E (SEE) >> ∂ÓÙÂÚÔÙÔÍ›ÓË G (SEG) >> ∂ÓÙÂÚÔÙÔÍ›ÓË H (SEH) >> ∂ÓÙÂÚÔÙÔÍ›ÓË π (SEπ) >> ∞ÔÊÔÏȉˆÙÈ΋ ÙÔÍ›ÓË ∞ (ExF-A) >> ∞ÔÊÔÏȉˆÙÈ΋ ÙÔÍ›ÓË B (ExF-B) >> ™ÙÚÂÙÔÎÔÎÎÈ΋ ¶˘ÔÁfiÓÔ˜ ˘ÚÂÙÔÁfiÓÔ˜ ÙÔÍ›ÓË ∞ (SPE-A) ÛÙÚÂÙfiÎÔÎÎÔ˜ >> >> >> B (SPE-B) >> >> >> >> C (SPE-C) >>
Ó¤Ô˘˜ ÂÓ‹ÏÈΘ Î·È ÌÔÚ› Ó· ·Ú·ÙËÚËı› ÌÂÙ¿ ·fi ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË, ÙÚ·˘Ì·ÙÈÛÌfi ‹ ¤Áη˘Ì· (8). ™ÙËÓ ÂÚÁ·Û›· Ì·˜ ÂÚÈÁÚ¿ÊÔÓÙ·È ‰‡Ô ÂÚÈÙÒÛÂȘ ·È‰ÈÒÓ Ô˘ ·ÚÔ˘Û›·Û·Ó ™∆∫ ÌÂÙ¿ ·fi ¤Áη˘Ì·, ÙÔ ÔÔ›Ô Â›¯Â ÂÈÌÔÏ˘Óı› ·fi ¯Ú˘Û›˙ÔÓÙ· ÛÙ·Ê˘ÏfiÎÔÎÎÔ. ¶ÂÚÈÁÚ·Ê‹ ÂÚÈÙÒÛÂˆÓ ∏ ÚÒÙË ÂÚ›ÙˆÛË ·ÊÔÚ¿ ÎÔÚ›ÙÛÈ ËÏÈΛ·˜ 9 ÌËÓÒÓ Ô˘ ÂÈÛ‹¯ıË ÛÙË ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ £Âڷ›·˜ (ª∂£) ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ Ì·˜ ÏfiÁˆ ˘ÚÂÙÔ‡, ΢¿ÓˆÛ˘ Î·È ÎˆÌ·ÙÒ‰Ô˘˜ ηٿÛÙ·Û˘. ∂ÚfiÎÂÈÙÔ ÁÈ· ÙÔ ‰Â‡ÙÂÚÔ ·È‰› ·ı›ÁÁ·ÓˆÓ, Ì ÂχıÂÚÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi. ™ÙÔ ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi ·Ó·ÊÂÚfiÙ·Ó ÌËÓÈÁÁ›Ùȉ· Û ËÏÈΛ· 45 ËÌÂÚÒÓ Î·È ÎÔÎ·Ù˘ Û ËÏÈΛ· 4 ÌËÓÒÓ. ∏ ·ÛıÂÓ‹˜, 7 Ë̤Ú˜ ÚÔ Ù˘ ÂÈÛ·ÁˆÁ‹˜ Ù˘ ÛÙÔ ÙÌ‹Ì· Ì·˜, ›¯Â ˘ÔÛÙ› ¤Áη˘Ì· ÛÙÔ ‰ÂÍÈfi ·ÓÙÈ‚Ú¿¯ÈÔ ·fi η˘Ùfi ÓÂÚfi, ÙÔ ÔÔ›Ô ¤Ù˘¯Â ÏËÌÌÂÏÔ‡˜ ·ÓÙÈÌÂÙÒÈÛ˘. ∞fi ÂÓıË̤ÚÔ˘ ·ÚÔ˘Û›·˙ ˘„ËÏfi ˘ÚÂÙfi Î·È ·fi ‰ÈË̤ÚÔ˘ ¿ÚÓËÛË Ï‹„˘ ÙÚÔÊ‹˜, Â̤ÙÔ˘˜ Î·È ‰È·ÚÚÔ˚Τ˜ ÎÂÓÒÛÂȘ. ∫·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÛÙË ª∂£ ‹Ù·Ó Û Έ̷ÙÒ‰Ë Î·Ù¿ÛÙ·ÛË (ÎÒÌ· ÛÙ¿‰ÈÔ ππ), Ì ÂÈfiÏ·È· ·Ó·ÓÔ‹, Ù·¯‡ÓÔÈ· Î·È Î˘¿ÓˆÛË, ·‡ÚÂÙË (ıÂÚÌÔÎÚ·Û›· ÔÚıÔ‡: 36,0ÔC), Ì ÂÈÎfiÓ· ηٷÏËÍ›·˜ (ÛʇÍÂȘ: 160/min, ∞¶: 65/40 mmHg, η΋ ÙÚȯÔÂȉÈ΋ ΢ÎÏÔÊÔÚ›·) Î·È ·Ê˘‰¿ÙˆÛË ÂÚ›Ô˘ 7%. À‹Ú¯Â ¤Áη˘Ì· 2Ô˘ ‚·ıÌÔ‡ ÂÎÙ¿Ûˆ˜ 2% ÛÙÔ ‰ÂÍÈfi ·ÓÙÈ‚Ú¿¯ÈÔ Î·È ‰È¿¯˘ÙÔ ÌÈÎÚÔÎËÏȉ҉˜ ÂÍ¿ÓıËÌ· ÛÙÔ ÚfiÛˆÔ, ÙÔÓ ÎÔÚÌfi Î·È Ù· ¿ÎÚ·,
500
Paediatriki 2001;64:499-503
ηıÒ˜ Î·È ˘ÂÚ·ÈÌ›· ÙˆÓ ÂÈÂÊ˘ÎfiÙˆÓ. O ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÂÈÛfi‰Ô˘ ¤‰ÂÈÍ ·Ó·ÈÌ›· Î·È ÂËÚ·Ṳ̂Ó˜ ‚ÈÔ¯ËÌÈΤ˜ ÂÍÂÙ¿ÛÂȘ: ·ÈÌÔÛÊ·ÈÚ›ÓË: 7 gr/dl, ·ÈÌ·ÙÔÎÚ›Ù˘: 22%, ÏÂ˘Î¿ ·ÈÌÔÛÊ·›ÚÈ·: 5500/mm3 (¶: 20%, §: 80%), ·ÈÌÔÂÙ¿ÏÈ·: 93000/mm3, Ô˘Ú›·: 83 mg/dl, ÎÚ·ÙÈÓ›ÓË: 0,6 mg/dl, SGOT: 210 U/L, SGPT: 45 U/L, CPK: 43 U/L, ÔÏÈο Ï¢ÎÒÌ·Ù·: 4,3 gr/dl, ·Ï‚Ô˘Ì›ÓË: 2,3 gr/dl, CRP: 198 mg/dl. ∏ O¡¶ ‹Ù·Ó ·ÚÓËÙÈ΋ ÁÈ· ÌËÓÈÁÁ›Ùȉ·. OÈ Î·ÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜ ‹Ù·Ó ÛÙ›Ú˜, ÂÓÒ ·fi ÙËÓ ÂÁη˘Ì·ÙÈ΋ ÂÈÊ¿ÓÂÈ· ·ÔÌÔÓÒıËΠSt·phylococcus aureus ·ÓıÂÎÙÈÎfi˜ ÛÙË ÌÂıÈÎÈÏÏ›ÓË. ∏ ·ÛıÂÓ‹˜, ÂÎÙfi˜ Ù˘ ¯ÔÚ‹ÁËÛ˘ ·ÓÙÈ‚·ÎÙËÚȷ΋˜ ·ÁˆÁ‹˜ (ÎÂÊÔÙ·Í›ÌË-ÎÏÈÓ‰·Ì˘Î›ÓË) Î·È Ù˘ ÂÚÈÔ›ËÛ˘ Ù˘ ÂÁη˘Ì·ÙÈ΋˜ ÂÈÊ¿ÓÂÈ·˜, ·ÓÙÈÌÂÙˆ›ÛÙËΠ̠Ì˯·ÓÈ΋ ˘ÔÛÙ‹ÚÈÍË Ù˘ ·Ó·ÓÔ‹˜, Ì ¯ÔÚ‹ÁËÛË ˘ÁÚÒÓ (ÊÔÚÙ›ÛÂȘ Ì ÎÔÏÏÔÂȉ‹ ‰È·Ï‡Ì·Ù·) Î·È ÈÓÔÙÚfiˆÓ (ÓÙÔ·Ì›ÓËÓÙÔÔ˘Ù·Ì›ÓË Â› 12 Ë̤Ú˜) Î·È ÌÂÙ¿ÁÁÈÛË ·›Ì·ÙÔ˜. ∆Ë 2Ë Ë̤ڷ ÓÔÛËÏ›·˜ ·ÚÔ˘Û›·Û ÂÈÎfiÓ· Û˘Ó‰ÚfiÌÔ˘ ÔÍ›·˜ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜ (ARDS) Ì ÛÔ‚·Ú‹ Âȉ›ӈÛË ÙfiÛÔ Ù˘ ·ÎÙÈÓÔÁÚ·Ê›·˜ ıÒÚ·ÎÔ˜, fiÛÔ Î·È ÙˆÓ ÙÈÌÒÓ ÙˆÓ ·ÂÚ›ˆÓ ·›Ì·ÙÔ˜. ™ÙËÓ ÚÔÛ¿ıÂÈ· ·ÓÙÈÌÂÙÒÈÛ˘ ÙÔ˘ ARDS ¤ÁÈÓ ÙÚÔÔÔ›ËÛË ÙÔ˘ Ì˯·ÓÈÎÔ‡ ·ÂÚÈÛÌÔ‡ (·ÓÙÈηٿÛÙ·ÛË ÙÔ˘ Û˘Ì‚·ÙÈÎÔ‡ ·ÂÚÈÛÌÔ‡ Ì ˘„›Û˘¯ÓÔ, Ì ÙËÓ ÂÊ·ÚÌÔÁ‹ ·Ó·Ó¢ÛÙ‹Ú· Ù·Ï·ÓÙÒÛˆÓ) Î·È ¯ÔÚËÁ‹ıËÎ·Ó 2 ‰fiÛÂȘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· (surfactant) ÂÓ‰ÔÙÚ·¯Âȷο. µ·ıÌÈ·›·, Ë ·ÛıÂÓ‹˜ ·ÚÔ˘Û›·Û ‚ÂÏÙ›ˆÛË. ∆Ô ÌÈÎÚÔÎËÏȉ҉˜ ÂÍ¿ÓıËÌ· ÛÙ·‰È·Î¿ ÂÍ·Ê·Ó›ÛÙËÎÂ Î·È ÙË 10Ë Ë̤ڷ ¿Ú¯ÈÛ ·ÔϤÈÛË Û fiÏÔ ÙÔ ÛÒÌ·, ȉȷ›ÙÂÚ· ¤Î‰ËÏË ÛÙȘ ·Ï¿Ì˜ Î·È Ù· ¤ÏÌ·Ù·. ∂Í‹Ïı ·fi ÙÔ ÙÌ‹Ì· Ì·˜ ÙË 16Ë Ë̤ڷ Ù˘ ÓfiÛÔ˘ Û ηϋ ηٿÛÙ·ÛË. ∏ ‰Â‡ÙÂÚË ÂÚ›ÙˆÛË ·ÊÔÚ¿ ÎÔÚ›ÙÛÈ ËÏÈΛ·˜ 18 ÌËÓÒÓ ·fi ·ı›ÁÁ·ÓÔ˘˜ ÁÔÓ›˜ Ì ÂχıÂÚÔ ÔÈÎÔÁÂÓÂÈ·Îfi Î·È ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi Ô˘ ÂÈÛ‹¯ıË ÛÙË ª∂£ ÏfiÁˆ ‰È·Ù·Ú·¯‹˜ ÙÔ˘ ÂȤ‰Ô˘ Û˘Ó›‰ËÛ˘. ∆Ô ·È‰› ›¯Â ˘ÔÛÙ› ÚÔ 24ÒÚÔ˘ ¤Áη˘Ì·, ÙÔ ÔÔ›Ô ·ÓÙÈÌÂÙˆ›ÛÙËΠ·fi ÙÔ˘˜ ÁÔÓ›˜ Ì ÙËÓ ÂÊ·ÚÌÔÁ‹ οÔÈ·˜ ·ÏÔÈÊ‹˜. ∫·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘ ÛÙÔ ÙÌ‹Ì· Ì·˜ ‹Ù·Ó Û ‚˘ıÈfiÙËÙ·, Ë ıÂÚÌÔÎÚ·Û›· ÔÚıÔ‡ ‹Ù·Ó 39,0ÔC, ›¯Â ηϋ ·ÚÙËÚȷ΋ ›ÂÛË Î·È Ê˘ÛÈÔÏÔÁÈÎfi ·Ó·Ó¢ÛÙÈÎfi „Èı‡ÚÈÛÌ·. ¶·ÚÔ˘Û›·˙ ¤Áη˘Ì· 2Ô˘ ‚·ıÌÔ‡ ÂÎÙ¿Ûˆ˜ 35%, ÛÙ· οو ¿ÎÚ·, ÙÔ ÂÚ›ÓÂÔ Î·È ÙÔ˘˜ ÁÏÔ˘ÙÔ‡˜. O ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍ ٷ ·ÎfiÏÔ˘ı·: °ÂÓÈ΋ ·›Ì·ÙÔ˜: ·ÈÌÔÛÊ·ÈÚ›ÓË: 9,6 gr/dl, ·ÈÌ·ÙÔÎÚ›Ù˘: 29%, ÏÂ˘Î¿ ·ÈÌÔÛÊ·›ÚÈ·: 3000/mm3 (¶: 69%, §: 25%, ª: 6%), ·ÈÌÔÂÙ¿ÏÈ·: 157000/mm3, Na: 127 meq/L, Ca: 6,2
¶·È‰È·ÙÚÈ΋ 2001;64:499-503
Paediatriki 2001;64:499-503
¶›Ó·Î·˜ 2. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· Û˘Ó‰ÚfiÌÔ˘ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜ (7) ¶˘ÚÂÙfi˜: £ÂÚÌÔÎÚ·Û›· ≥38,9ÔC EÍ¿ÓıËÌ·: ¢È¿¯˘ÙÔ ÎËÏȉ҉˜ ÂÍ¿ÓıËÌ· ∞ÔϤÈÛË: 1-2 ‚‰ÔÌ¿‰Â˜ ÌÂÙ¿ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘, ȉȷ›ÙÂÚ· Û ¤ÏÌ·Ù· Î·È ·Ï¿Ì˜ ÀfiÙ·ÛË: ™˘ÛÙÔÏÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË ≤90 mmHg ÁÈ· ÂÓËÏ›ÎÔ˘˜ ‹ <5Ë ∂£ ÁÈ· ÙËÓ ËÏÈΛ· ÙÔ˘˜ Û ·È‰È¿ <16 ÂÙÒÓ, ÙÒÛË Ù˘ ‰È·ÛÙÔÏÈ΋˜ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ ≥15 mmHg ηٿ ÙË ÌÂÙ¿‚·ÛË ·fi ÙËÓ Î·Ù·ÎÂÎÏÈ̤ÓË ÛÙËÓ fiÚıÈ· ı¤ÛË, ÔÚıÔÛÙ·ÙÈÎfi Û˘ÁÎÔÈÎfi ÂÂÈÛfi‰ÈÔ ‹ ˙¿ÏË ÛÙËÓ fiÚıÈ· ı¤ÛË 5. ¶ÚÔÛ‚ÔÏ‹ ÙÚÈÒÓ ‹ ÂÚÈÛÛfiÙÂÚˆÓ ·fi Ù· ·ÎfiÏÔ˘ı·: ñ °·ÛÙÚÂÓÙÂÚÈÎfi: ŒÌÂÙÔÈ ‹ ‰È·ÚÚÔ˚Τ˜ ÎÂÓÒÛÂȘ ÛÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘ ñ ª˘˚Îfi: ™ËÌ·ÓÙÈ΋ Ì˘·ÏÁ›· ‹ CPK ÔÚÔ‡ >2Ï¿ÛÈÔ Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ÙÈÌ‹˜ ñ µÏÂÓÓÔÁfiÓÔÈ: ÀÂÚ·ÈÌ›· ÂÈÂÊ˘ÎfiÙˆÓ, ÛÙÔÌ·ÙÔÊ¿Ú˘ÁÁ·, ÎfiÏÔ˘ ñ O˘ÚÔÔÈËÙÈÎfi: O˘Ú›· ‹ ÎÚ·ÙÈÓ›ÓË ÔÚÔ‡ >2Ï¿ÛÈÔ Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ÙÈÌ‹˜ ‹ ˘ÔÛÊ·›ÚÈ· ≥5 ÎÔ Â› ·Ô˘Û›·˜ Ô˘ÚÔÏԛ̈͢ ñ ◊·Ú: OÏÈ΋ ¯ÔÏÂÚ˘ıÚ›ÓË, SGOT ‹ SGPT >2Ï¿ÛÈÔ Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ÙÈÌ‹˜ ñ ∞ÈÌÔÔÈËÙÈÎfi: ∞ÈÌÔÂÙ¿ÏÈ· <100.000/mm3 ñ ∫¡™: ¢È·Ù·Ú·¯¤˜ ÚÔÛ·Ó·ÙÔÏÈÛÌÔ‡ ‹ ÙÔ˘ ÂȤ‰Ô˘ Û˘Ó›‰ËÛ˘ ¯ˆÚ›˜ ÂÛÙȷο Ó¢ÚÔÏÔÁÈο ÛËÌ›·, › ·Ô˘Û›·˜ ˘ÚÂÙÔ‡ Î·È ˘fiÙ·Û˘ 6. ∞ÚÓËÙÈο ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ·ÎfiÏÔ˘ıˆÓ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛˆÓ, Â¿Ó ÏËÊıÔ‡Ó: ñ ∫·ÏÏȤÚÁÂÈ· ·›Ì·ÙÔ˜, Ê·Ú˘ÁÁÈÎÔ‡ ÂȯڛÛÌ·ÙÔ˜ Î·È ∂¡À (Ë Î·ÏÏȤÚÁÂÈ· ·›Ì·ÙÔ˜ ÌÔÚ› Ó· Â›Ó·È ıÂÙÈ΋ ÁÈ· ¯Ú˘Û›˙ÔÓÙ· ÛÙ·Ê˘ÏfiÎÔÎÎÔ) ñ OÚÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÁÈ· ÎËÏÈ‰Ò‰Ë ˘ÚÂÙfi ÙˆÓ ‚Ú·¯ˆ‰ÒÓ ÔÚ¤ˆÓ, ÏÂÙÔÛ›ڈÛË ‹ ÈÏ·Ú¿
1. 2. 3. 4.
¶Èı·Ó‹ ‰È¿ÓˆÛË: ŸÙ·Ó ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È 5 ·fi Ù· 6 ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· µÂ‚·›· ‰È¿ÁÓˆÛË: ŸÙ·Ó ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Î·È Ù· 6 ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ·, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Ù˘ ·ÔϤÈÛ˘, ÂÎÙfi˜ Â¿Ó Ô ·ÛıÂÓ‹˜ ηٷϋÍÂÈ ÚÈÓ ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ·ÔϤÈÛ˘
mg/dl, ÁÏ˘Îfi˙Ë: 38 mg/dl, Ô˘Ú›·: 32 mg/dl, ÎÚ·ÙÈÓ›ÓË: 0,7 mg/dl, SGOT: 145 U/L, SGPT: 53 U/L, CPK: 3020 U/L, ÔÏÈο Ï¢ÎÒÌ·Ù·: 3,8 gr/dl, ·Ï‚Ô˘Ì›ÓË: 1,8 gr/dl, CRP: 382 mg/dl. ∏ ηÏÏȤÚÁÂÈ· ·›Ì·ÙÔ˜ ‹Ù·Ó ÛÙ›ڷ, ÂÓÒ ·fi ÙËÓ Î·ÏÏȤÚÁÂÈ· ˘ÏÈÎÔ‡ ·Ô ÙËÓ ÂÁη˘Ì·ÙÈ΋ ÂÈÊ¿ÓÂÈ· ·ÔÌÔÓÒıËÎ·Ó St. aureus, Enterobacter cloacae Î·È Klebsiella oxytoca. ÃÔÚËÁ‹ıËÎ·Ó ÂÓ‰ÔÊϤ‚È· ÔÈ ËÌÂÚ‹ÛȘ ·Ó¿ÁΘ ÙÔ˘ Û ˘ÁÚ¿ Î·È Î·Ï‡ÊıËΠ̠ÂÓÈÎÈÏÏ›ÓË G. ∆Ô Â›Â‰Ô Û˘Ó›‰ËÛ˘ ·ÔηٷÛÙ¿ıËΠÁÚ‹ÁÔÚ· Î·È ·fi ÙË 2Ë Ë̤ڷ ÓÔÛËÏ›·˜ Ô ˘ÚÂÙfi˜ ÛËÌ›ˆÛ ‡ÊÂÛË. ∆ËÓ 3Ë Ë̤ڷ ¿Ú¯ÈÛ ӷ ·ÚÔ˘ÛÈ¿˙ÂÈ ÔÏ˘¿ÚÈı̘ ‰È·ÚÚÔ˚Τ˜ ÎÂÓÒÛÂȘ Î·È ·Ú¿ÏÏËÏ· ·Ú·ÙËÚ‹ıËΠ̛ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (50000/mm3). To 6o 24ˆÚÔ Î·È ÂÓÒ ‹Ù·Ó Û ηϋ ÁÂÓÈ΋ ηٿÛÙ·ÛË, ÂÌÊ¿ÓÈÛ ÎËÏȉԂϷÙȉ҉˜ ÂÍ¿ÓıËÌ· ΢ڛˆ˜ ÛÙÔ ÚfiÛˆÔ Î·È ÙÔÓ ÎÔÚÌfi Î·È ıÂÚÌÔÎÚ·Û›· 38,2ÔC. ∆Ș ÂfiÌÂÓ˜ ÒÚ˜ ÛËÌ›ˆÛ ڷÁ‰·›· Âȉ›ӈÛË, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·˜ ÂÈÎfiÓ· ηٷÏËÍ›·˜ Î·È ÔÍ›·˜ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜. ¶·Ú¿ ÙËÓ ˘ÔÛÙ‹ÚÈÍË ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Ì Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi Î·È ÙÔ˘ ΢ÎÏÔÊÔÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Ì ÈÓfiÙÚÔ·, ÙÔ ·È‰› η٤ÏËÍ ÙËÓ ›‰È· Ë̤ڷ. ™ÙËÓ ÙÂÏÂ˘Ù·›·
ηÏÏȤÚÁÂÈ· ·›Ì·ÙÔ˜ ·ÔÌÔÓÒıËΠS. aureus. O ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ ÙÔÍÈÓÒÓ ÙÔ˘ Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ÛÙÂϤ¯Ô˘˜ ¤‰ÂÈÍ ÙÔÍ›ÓË Û˘Ó‰ÚfiÌÔ˘ ÙÔÍÈ΋˜ ηٷÏËÍ›·˜ (TSST-1) ηıÒ˜ Î·È ·ÔÊÔÏȉˆÙÈ΋ ÙÔÍ›ÓË (ExF-A). ™˘˙‹ÙËÛË ∆Ô ™∆∫ ·ÔÙÂÏ› Û¿ÓÈ· ÂÈÏÔ΋ Ù˘ ÛÙ·Ê˘ÏÔÎÔÎÎÈ΋˜ Ïԛ̈͢ (3). ∏ Û˘¯ÓfiÙËÙ¿ ÙÔ˘ ·Ó¤Ú¯ÂÙ·È ÛÙȘ ∏¶∞ Û 0,50 ÂÚÈÙÒÛÂȘ ·Ó¿ 100.000 ηÙÔ›ÎÔ˘˜ Î·È ·fi ·˘Ù¤˜ ÙÔ 20-40% ·ÊÔÚ¿ ÙË ÌË ÂÌÌËÓÔÚÚ˘Ûȷ΋ ÌÔÚÊ‹ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, Ô˘ Û˘Ì‚·›ÓÂÈ ÌÂÙ¿ ·fi ¯ÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ, ÙÚ·˘Ì·ÙÈÛÌÔ‡˜ ‹ ÂÁη‡Ì·Ù· (9). H Û˘¯ÓfiÙËÙ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Û ÌÈÎÚ¿ ·È‰È¿ Ì ¤Áη˘Ì· Â›Ó·È ÛËÌ·ÓÙÈ΋ Î·È Î˘Ì·›ÓÂÙ·È ÂÚ›Ô˘ ÛÙÔ 10% ÙˆÓ ·ÛıÂÓÒÓ (6,10). ™˘ÁÎÂÎÚÈ̤ӷ, ·fi Ù· 64 ·È‰È¿ ËÏÈΛ·˜ οو ÙˆÓ 24 ÌËÓÒÓ Ô˘ ÂÈÛ‹¯ıËÛ·Ó Û ªÔÓ¿‰· ∂Áη˘Ì·ÙÈÒÓ Ù· 6 (9,4%) ·ÚÔ˘Û›·Û·Ó ™∆∫ (10). OÈ ‰‡Ô ·ÛıÂÓ›˜ Ù˘ ÂÚÁ·Û›·˜ Ì·˜ ÏËÚÔ‡Û·Ó Ù· ÎÚÈÙ‹ÚÈ· ÙÔ˘ ™∆∫ (7). ∏ ÚÒÙË ÂÚ›ÙˆÛË ÏËÚÔ‡ÛÂ Î·È Ù· ¤ÍÈ ÎÚÈÙ‹ÚÈ·, ÂÓÒ Ë ‰Â‡ÙÂÚË ÂÚ›ÙˆÛË Ù· ¤ÓÙ ·fi Ù· ¤ÍÈ, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ·Ô˘Û›·˙ ÙÔ ÎÚÈÙ‹ÚÈÔ Ù˘ ·ÔϤÈÛ˘. ∫·Ù¿ ÙËÓ ·Ó·ÛÎfiËÛË ÙˆÓ de Saxe Î·È Û˘Ó, ÌfiÓÔ ÔÈ ÌÈÛ¤˜ ·fi
501
¶·È‰È·ÙÚÈ΋ 2001;64:499-503
ÙȘ ÂÚÈÙÒÛÂȘ ÌË ÂÌÌËÓÔÚÚ˘Ûȷ΋˜ ÌÔÚÊ‹˜ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÏËÚÔ‡Ó fiÏ· Ù· ÎÚÈÙ‹ÚÈ· (11). ∞˘Ù¿ Ô˘ ·Ô˘ÛÈ¿˙Ô˘Ó Û ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· Â›Ó·È Ë ˘fiÙ·ÛË Î·È Ë ·ÔϤÈÛË. ŒÙÛÈ, Û‡Ìʈӷ Ì ÙÔÓ ÚfiÛÊ·ÙÔ ÔÚÈÛÌfi ÙÔ˘ ∫¤ÓÙÚÔ˘ ∂ϤÁ¯Ô˘ Î·È ¶ÚfiÏ˄˘ ¡ÔÛËÌ¿ÙˆÓ (CDC) ÙˆÓ ∏¶∞, Û ÂÚ›ÙˆÛË ı·Ó¿ÙÔ˘ ÙÔ˘ ·ÛıÂÓÔ‡˜, Ë ·ÔϤÈÛË ‰ÂÓ Û˘ÌÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙ· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÙÔ˘ ™∆∫ (7). °È· ÙËÓ ÂΉ‹ÏˆÛË ÙÔ˘ ™∆∫ ··ÈÙÂ›Ù·È Ô Û˘Ó‰˘·ÛÌfi˜ ÙÚÈÒÓ ·Ú·ÁfiÓÙˆÓ (21): ¤Ó· ¢ÓÔ˚Îfi ÂÚÈ‚¿ÏÏÔÓ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ÂÓfi˜ ·ıÔÁfiÓÔ˘ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡, Ô ÔÔ›Ô˜ ı· ·Ú¿ÁÂÈ Â·Ú΋ ÔÛfiÙËÙ· ÙÔ͛Ӣ, ÒÛÙ ӷ ÂΉËÏÒÛÂÈ Û˘ÌÙÒÌ·Ù· ¤Ó·˜ ¢·ı‹˜ ÍÂÓÈÛÙ‹˜. ŸÛÔÓ ·ÊÔÚ¿ ÙÔÓ ÍÂÓÈÛÙ‹, Ë Â˘¿ıÂÈ¿ ÙÔ˘ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ‡·ÚÍË ‹ fi¯È ÚÔÛٷ٢ÙÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙˆÓ ·Ú·ÁfiÌÂÓˆÓ Â͈ÙÔÍÈÓÒÓ Î·È Î˘Ú›ˆ˜ Ù˘ TSST-1 (22). ∏ ·Ô˘Û›· ·ÓÔÛ›·˜ ÔÊ›ÏÂÙ·È ÛÙËÓ ¤ÏÏÂÈ„Ë ÚÔËÁÔ‡ÌÂÓ˘ ¤ÎıÂÛ˘ ‹ ÛÙËÓ ·‰˘Ó·Ì›· ·Ú·ÁˆÁ‹˜ ·˘ÙÒÓ ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ (14,16). O Ù›ÙÏÔ˜ ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ù˘ TSST-1 ·˘Í¿ÓÂÈ Ì ÙËÓ ËÏÈΛ·. ŒÙÛÈ ÂÍËÁÂ›Ù·È ÁÈ·Ù› ÚÔÛ‚¿ÏÏÔÓÙ·È Û˘¯ÓfiÙÂÚ· ·È‰È¿ ÌÈÎÚ‹˜ ËÏÈΛ·˜. ™‡Ìʈӷ Ì ÙÔ˘˜ Vergerant Î·È Û˘Ó, ÌfiÓÔ ÙÔ 30% ˘ÁÈÒÓ ·È‰ÈÒÓ ËÏÈΛ·˜ 1 ¤ˆ˜ 2 ÂÙÒÓ Â›¯Â ÚÔÛٷ٢ÙÈÎÔ‡˜ Ù›ÙÏÔ˘˜ ·ÓÙÈÛˆÌ¿ÙˆÓ (≥1:100) ¤Ó·ÓÙÈ Ù˘ TSST-1 (13). ∞ÓÙ›ıÂÙ·, Û‡Ìʈӷ Ì ÙË ÌÂϤÙË ÙˆÓ Notermans Î·È Û˘Ó, ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô ÔÛÔÛÙfi ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ ¤Êı·Ó ÙÔ 90% (12). £· Ú¤ÂÈ Ó· ÛËÌÂȈı›, ›Û˘, fiÙÈ ÌËÙÚÈο ·ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ Ù˘ TSST-1 ·ÓȯÓ‡ÔÓÙ·È Û ‚Ú¤ÊË ËÏÈΛ·˜ ̤¯ÚÈ 9 ÌËÓÒÓ (13,14). º·›ÓÂÙ·È, ÏÔÈfiÓ, fiÙÈ ÔÈ ‰‡Ô ÂÁη˘Ì·Ù›Â˜ Ù˘ ÂÚÁ·Û›·˜ Ì·˜, 9 Î·È 18 ÌËÓÒÓ, ·Ó‹Î·Ó ÏfiÁˆ ËÏÈΛ·˜ Û ÔÌ¿‰· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ˆ˜ ÚÔ˜ ÙËÓ ÂΉ‹ÏˆÛË ™∆∫. π‰È·›ÙÂÚË ÛËÌ·Û›· ¤¯ÂÈ ÙÔ fiÙÈ Ë ÂÌÊ¿ÓÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ‰ÂÓ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ¤ÎÙ·ÛË Ù˘ ÂÁη˘Ì·ÙÈ΋˜ ÂÈÊ¿ÓÂÈ·˜. Œ¯Ô˘Ó ·Ó·ÊÂÚı› ÂÚÈÙÒÛÂȘ ·È‰ÈÒÓ Ô˘ ÂÌÊ¿ÓÈÛ·Ó ™∆∫ Ì ¤Áη˘Ì· ÂÎÙ¿Ûˆ˜ 2% (15). ∏ ÂÚÁ·Û›· Ì·˜, ÛÙËÓ ÔÔ›· Ô ¤Ó·˜ ·ÛıÂÓ‹˜ ›¯Â ÂÁη˘Ì·ÙÈ΋ ÂÈÊ¿ÓÂÈ· ÂÎÙ¿Ûˆ˜ 2%, ÂȂ‚·ÈÒÓÂÈ ÙË ‰È·›ÛÙˆÛË ·˘Ù‹. ™ÙÔÓ ¤Ó· ·ÛıÂÓ‹ Ì·˜ ·ÓȯÓ‡ıËÎ·Ó ÔÈ Â͈ÙÔ͛Ә TSST-1 Î·È ExF-A. ∏ TSST-1 ·ÓȯÓ‡ÂÙ·È Û˘¯ÓfiÙÂÚ· Û ۯ¤ÛË Ì ÙȘ ¿ÏϘ Â͈ÙÔ͛Ә ÛÙÔ˘˜ ·ÚÚÒÛÙÔ˘˜ Ì ™∆∫ (16). Œ¯ÂÈ ‰È·ÈÛÙˆı› fiÙÈ ÛÙȘ ÂÚÈÙÒÛÂȘ ÂÌÌËÓÔÚÚ˘Ûȷ΋˜ ÌÔÚÊ‹˜ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, ÂÚÈÛÛfiÙÂÚÔ ·fi 90% ÙˆÓ ÛÙÂϯÒÓ ÙÔ˘ ÛÙ·Ê˘ÏfiÎÔÎÎÔ˘ ·Ú¿ÁÔ˘Ó ÙËÓ TSST-1, ÂÓÒ ÛÙȘ ÂÚÈÙÒÛÂȘ ÌË ÂÌÌËÓÔÚÚ˘Ûȷ΋˜ ÌÔÚÊ‹˜ ÙÔ 6065% ÙˆÓ ÛÙÂϯÒÓ Û˘Óı¤ÙÔ˘Ó ÙË Û˘ÁÎÂÎÚÈ̤ÓË ÙÔÍ›ÓË (11). ∏ ·Ó›¯Ó¢ÛË ÙÔÍÈÓÒÓ, fiˆ˜ ·Ó·Ê¤Ú-
502
Paediatriki 2001;64:499-503
ıËΠÛÙËÓ ÂÈÛ·ÁˆÁ‹, ‰ÂÓ ÂȂ‚·ÈÒÓÂÈ ÙË ‰È¿ÁÓˆÛË ÙÔ˘ ™∆∫, ·ÏÏ¿ ÌÔÚ› Ó· ¯ÚËÛÈ̇ÛÂÈ ÌfiÓÔ ÁÈ· ÂȉËÌÈÔÏÔÁÈÎÔ‡˜ ‹ ÂÚ¢ÓËÙÈÎÔ‡˜ ÏfiÁÔ˘˜ (6). ¶Ú¿ÁÌ·ÙÈ, ¤¯ÂÈ ‚ÚÂı› fiÙÈ ÙÔ 25-30% ÙˆÓ ÛÙÂϯÒÓ ÙÔ˘ ¯Ú˘Û›˙ÔÓÙ· ÛÙ·Ê˘ÏfiÎÔÎÎÔ˘ ·Ú¿ÁÔ˘Ó Â͈ÙÔ͛Ә (12). ¶ÚÔÛ‚ÔÏ‹ ·fi Ù· Û˘ÁÎÂÎÚÈ̤ӷ ÌÈÎÚԂȷο ÛÙÂϤ¯Ë (Ì ٷ˘Ùfi¯ÚÔÓË ·Ú·ÁˆÁ‹ ÙÔÍÈÓÒÓ) ‰ÂÓ ÚÔηÏ› ··Ú·›ÙËÙ· ™∆∫ (17). ∂›Ó·È ·ÍÈÔÛËÌ›ˆÙÔ fiÙÈ Î·È ÔÈ ‰‡Ô ·ÛıÂÓ›˜ Ì·˜ ·ÚÔ˘Û›·Û·Ó ÂÈÎfiÓ· ÔÍ›·˜ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜. ™ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· Ê·›ÓÂÙ·È fiÙÈ ÔÈ ·ÛıÂÓ›˜ Ì ™∆∫ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Û ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ARDS. ™‡Ìʈӷ, Ì¿ÏÈÛÙ·, Ì ÙË ÌÂϤÙË ÙˆÓ Stevens Î·È Û˘Ó, Û 20 ·ÛıÂÓ›˜ Ì ™∆∫ ÔÊÂÈÏfiÌÂÓÔ Û Â͈ÙÔ͛Ә ·Ú·ÁfiÌÂÓ˜ ·fi ÛÙÚÂÙfiÎÔÎÎÔ, ÔÈ 11 (55%) ÂΉ‹ÏˆÛ·Ó ARDS (18). ∂ÈϤÔÓ, Â›Ó·È ÁÓˆÛÙfi fiÙÈ ÙÔ ARDS ˘ÚÔ‰ÔÙÂ›Ù·È ·fi Û˘ÁÎÂÎÚÈ̤ÓÔ˘˜ ÂÎÏ˘ÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ïԛ̈ÍË, ÙÚ·˘Ì·ÙÈÛÌfi˜, ¤Áη˘Ì·, ÌÂÙ·ÁÁ›ÛÂȘ (19). ¶ÚÔÊ·ÓÒ˜, ÔÈ ‰‡Ô ÂÁη˘Ì·Ù›Â˜ Ù˘ ÂÚÁ·Û›·˜ Ì·˜ ·ÚÔ˘Û›·˙·Ó ÔÏÏÔ‡˜ ÂÎÏ˘ÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, ¤Ú·Ó ÙÔ˘ ™∆∫, Ô˘ ı· ÌÔÚÔ‡Û·Ó Ó· ÚÔηϤÛÔ˘Ó ÙËÓ ÂΉ‹ÏˆÛË ARDS. ∏ ıÂڷ›· Ô˘ ÂÊ·ÚÌfiÛÙËΠÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜, ÂÎÙfi˜ ·fi ÙËÓ ÙÔÈ΋ ÂÚÈÔ›ËÛË Ù˘ ÂÁη˘Ì·ÙÈ΋˜ ÂÈÊ¿ÓÂÈ·˜, ÂÚÈÂÏ¿Ì‚·Ó ÙË ¯ÔÚ‹ÁËÛË ·ÓÙÈÛÙ·Ê˘ÏÔÎÔÎÎÈ΋˜ ·ÁˆÁ‹˜, ÊÔÚÙ›ÛÂˆÓ Ì ÎÔÏÏÔÂȉ‹ ‰È·Ï‡Ì·Ù· Î·È ÈÓÔÙÚfiˆÓ Ê·Ú̿ΈÓ. ∏ ¯ÔÚ‹ÁËÛË ÛÙÂÚÔÂȉÒÓ Î·È Á-ÛÊ·ÈÚ›Ó˘, ·Ó Î·È ÂÊ·ÚÌfi˙ÂÙ·È ·fi ÔÚÈṲ̂ÓÔ˘˜, ıˆÚÂ›Ù·È ·ÌÊÈÛ‚ËÙÔ‡ÌÂÓË (8). O ¤Ó·˜ ·fi ÙÔ˘˜ ‰‡Ô ·ÛıÂÓ›˜ Ù˘ ÂÚÁ·Û›·˜ Ì·˜ η٤ÏËÍÂ. °ÂÓÈο, Ë ıÓËÙfiÙËÙ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Î˘Ì·›ÓÂÙ·È ·fi 2-15%, ÛÙÔ˘˜ ·ÚÚÒÛÙÔ˘˜ fï˜ Ì ¤Áη˘Ì· Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË Î·È ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ÂΉ‹ÏˆÛË Î·Ù·ÏËÍ›·˜, ÔfiÙ ÌÔÚ› Ó· ·Ó¤ÏıÂÈ ¤ˆ˜ 57% (6,8,20). ∞fi ÙË ÛÙÈÁÌ‹ Ô˘ ÙÔ Û‡Ó‰ÚÔÌÔ ¤¯ÂÈ ÛËÌ·ÓÙÈ΋ ıÓËÙfiÙËÙ·, Â›Ó·È ÂÈı˘ÌËÙfi Ó· ·Ó·ÁÓˆÚ›˙ÂÙ·È Î·È Ó· ıÂڷ‡ÂÙ·È ¯ˆÚ›˜ ηı˘ÛÙ¤ÚËÛË (6). ∆Ô ÛËÌ·ÓÙÈÎfiÙÂÚÔ ÛËÌÂ›Ô Ù˘ ıÂڷ›·˜ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Â›Ó·È Èı·ÓfiÓ Ë ÁÓÒÛË Ù˘ ‡·ÚÍ‹˜ ÙÔ˘. ∏ ‰È¿ÁÓˆÛË Ù˘ ÛÙ·Ê˘ÏÔÎÔÎÎÈ΋˜ ÙÔÍÈÓ·ÈÌ›·˜ Ú¤ÂÈ Ó· ÙÂı› ÚÔÙÔ‡ Á›ÓÂÈ ÂÌÊ·Ó¤˜ ÔÏfiÎÏËÚÔ ÙÔ Û‡Ó‰ÚÔÌÔ, ‰ËÏ·‰‹ ¯ˆÚ›˜ Ó· ¤¯Ô˘Ì fiÏ· Ù· ÎÚÈÙ‹ÚÈ· Ù˘ ÔÚÈÛÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Î·È ÙfiÙ ӷ ·Ú¯›ÛÂÈ Î·È Ë ·ÓÙÈÌÂÙÒÈÛË. ∞˘Ù¿ Â›Ó·È ÛËÌ·ÓÙÈÎfi Ó· Á›ÓÔ˘Ó ÚÈÓ ÙËÓ ÂΉ‹ÏˆÛË Ù˘ ηٷÏËÍ›·˜ (10). ∏ ÚÒÈÌË ‰È¿ÁÓˆÛË ÙÔ˘ ™∆∫ ÛÙ· ·È‰È¿ Ì ¤Áη˘Ì·, Ô˘ÛÈ·ÛÙÈο ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË ÙÔ˘ shock, ı· Ô‰ËÁ‹ÛÂÈ ÛÙËÓ ¤ÁηÈÚË ¤Ó·ÚÍË ıÂڷ›·˜, Ì ·ÔÙ¤ÏÂÛÌ· ÙË Ì›ˆÛË Ù˘ ıÓËÛÈÌfiÙËÙ·˜ ÛÙ· ·È‰È¿ ·˘Ù¿.
¶·È‰È·ÙÚÈ΋ 2001;64:499-503
Paediatriki 2001;64:499-503
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HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 03-11-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 17-04-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ª·Ú›· ¡·ÚÏ›ÔÁÏÔ˘ ¶ª∂¡ (4Ô˜ fiÚÔÊÔ˜), ¡ÔÛ. ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” £Ë‚ÒÓ & §Â‚·‰›·˜, 115 27, ∞ı‹Ó·
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CASE REPORT
™˘ÁÁÂÓ‹˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË Ì ·˘ÍË̤ӷ ›‰· ÂÚ˘ıÚÔÔÈËÙ›Ó˘. ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ª. ∞ı·Ó·Û›Ô˘-ªÂÙ·Í¿, ª. OÈÎÔÓfiÌÔ˘, Ã. ∆Û¿ÓÙ·ÏË, ∞. ∫Ô‡ÛË, ¡. °ÔÌ¿Î˘
Congenital erythrocytosis with increased erythropoietin levels. Case report M. Athanassiou-Metaxa, M. Oikonomou, C. Tsantali, A. Kousi, N. Gombakis
¶ÂÚ›ÏË„Ë: O fiÚÔ˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË ÂÚÈÏ·Ì‚¿ÓÂÈ Ì›· ÂÙÂÚÔÁÂÓ‹ ÔÌ¿‰· ‰È·Ù·Ú·¯ÒÓ, ÔÈ Ôԛ˜ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ·‡ÍËÛË Ù˘ Ì¿˙·˜ ÙˆÓ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ, Û¯ÂÙÈ΋ ‹ ·fiÏ˘ÙË. ∏ ·fiÏ˘ÙË ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË ÌÔÚ› Ó· Â›Ó·È ÚˆÙÔ·ı‹˜ ‹ ‰Â˘ÙÂÚÔ·ı‹˜, Û˘ÁÁÂÓ‹˜ ‹ ›ÎÙËÙË. ¶ÂÚÈÁÚ¿ÊÂÙ·È ÂÚ›ÙˆÛË ‚Ú¤ÊÔ˘˜ ËÏÈΛ·˜ 20 ÌËÓÒÓ Ì ·fiÏ˘ÙË ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË, Èı·Ófiٷٷ Û˘ÁÁÂÓÔ‡˜ ·ÈÙÈÔÏÔÁ›·˜, Ì›· ·ÈÌ·ÙÔÏÔÁÈ΋ ‰È·Ù·Ú·¯‹ Û¿ÓÈ· ÛÙËÓ ·È‰È΋ Î·È È‰È·›ÙÂÚ· ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·. ÷ڷÎÙËÚÈÛÙÈ΋ ‹Ù·Ó, ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È, Ë È‰È·›ÙÂÚ· ·˘ÍË̤ÓË ÙÈÌ‹ Ù˘ ÂÚ˘ıÚÔÔÈËÙ›Ó˘ ÙÔ˘ ÔÚÔ‡, ¯ˆÚ›˜ Ó· Â›Ó·È ‰˘Ó·Ù‹ Ë ‰È·›ÛÙˆÛË, ·Ú¿ ÙÔÓ ÂÎÙÂٷ̤ÓÔ ¤ÏÂÁ¯Ô, οÔÈ·˜ ÂÌÊ·ÓÔ‡˜ ·ÈÙ›·˜ ˘ÂÚ¤ÎÎÚÈÛ‹˜ Ù˘. ∂ӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ, ›Û˘, Ë ·Ó›¯Ó¢ÛË ·˘ÙfiÌ·Ù˘ ·Ó¿Ù˘Í˘ ·ÔÈÎÈÒÓ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ (CFU-E) ÛÙËÓ Î·ÏÏȤÚÁÂÈ· ΢ÙÙ¿ÚˆÓ Ì˘ÂÏÔ‡, ¯ˆÚ›˜ ÙËÓ ·ÚÔ˘Û›· ÂÚ˘ıÚÔÔÈËÙ›Ó˘. ¶·Ú¿ ÙÔ fiÙÈ ÙÔ Â‡ÚËÌ· ·˘Ùfi ıˆÚÂ›Ù·È ÂÓ‰ÂÈÎÙÈÎfi ÚˆÙÔ·ıÔ‡˜ Ì˘ÂÏÔ¸ÂÚÏ·ÛÙÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ Î·È ÂȉÈÎfiÙÂÚ· Ù˘ ·ÏËıÔ‡˜ ÔÏ˘Î˘ÙÙ·Ú·ÈÌ›·˜ (polycythemia vera), Ë ·ÛıÂÓ‹˜ ‰ÂÓ ÏËÚÔ‡Û ٷ ÎÚÈÙ‹ÚÈ· ‰È¿ÁÓˆÛ˘ Ù˘ ÓÔÛÔÏÔÁÈ΋˜ ·˘Ù‹˜ ÔÓÙfiÙËÙ·˜.
Abstract: The term erythrocytosis denotes a heterogenous group of disorders characterized by an increased red blood cell mass, absolute or relative. Absolute erythrocytocis may be primary or secondary, congenital or acquired. We report the case of a 20 month old girl who presented with absolute erythrocytosis, probably congenital, a disorder that is rarely seen in children. Erythropoietin levels were found to be significantly increased, although extensive evaluation failed to reveal a cause for such an inappropriate elevation. Of interest is the finding of spontaneous erythroid colony formation (CFU-E) in the patient’s bone marrow cultures. Although such a finding is considered typical of polycythemia vera, the diagnostic criteria of this myeloproliferative disorder were not met.
§¤ÍÂȘ ÎÏÂȉȿ: Û˘ÁÁÂÓ‹˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË, ‚ÚÂÊÈ΋ ËÏÈΛ·, ÂÚ˘ıÚÔÔÈËÙ›ÓË.
Key words: congenital erythrocytosis, infancy, erythropoietin.
∂ÈÛ·ÁˆÁ‹ H ·fiÏ˘ÙË ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË Â›Ó·È Ì›· ‰È·Ù·Ú·¯‹ ÂÍ·ÈÚÂÙÈο Û¿ÓÈ· ÛÙËÓ ·È‰È΋ ËÏÈΛ· (1). ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ·‡ÍËÛË Ù˘ Ì¿˙·˜ ÙˆÓ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ Î·È ÌÔÚ› Ó· Â›Ó·È ÚˆÙÔ·ı‹˜ ‹ ‰Â˘ÙÂÚÔ·ı‹˜, Û˘ÁÁÂÓ‹˜ ‹ ›ÎÙËÙË. ™ÙËÓ
ÚˆÙÔ·ı‹ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË ·Ú·ÙËÚÂ›Ù·È ·˘ÍË̤ÓË Â˘·ÈÛıËÛ›· ÙˆÓ ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÛÙË Ê˘ÛÈÔÏÔÁÈο ÂÎÎÚÈÓfiÌÂÓË ÂÚ˘ıÚÔÔÈËÙ›ÓË ÂÓÒ ÛÙË ‰Â˘ÙÂÚÔ·ı‹, ·˘ÍË̤ÓË ‰È¤ÁÂÚÛË ÙˆÓ ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ˘ÂÚ¤ÎÎÚÈÛ˘ ÂÚ˘ıÚÔÔÈËÙ›Ó˘.
∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£, °¶¡£ πÔÎÚ¿ÙÂÈÔ £ÂÛÛ·ÏÔÓ›ÎË
1st Pediatric Department of Aristotelion University of Thessaloniki, Ippokration General Hospital, Thessaloniki
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¶·È‰È·ÙÚÈ΋ 2001;64:504-507
¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ‚Ú¤ÊÔ˘˜ ËÏÈΛ·˜ 20 ÌËÓÒÓ Ì ·fiÏ˘ÙË ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË, ÏfiÁˆ Ù˘ Û·ÓÈfiÙËÙ·˜ ÂΉ‹ÏˆÛ˘ Ù˘ ‰È·Ù·Ú·¯‹˜ ·˘Ù‹˜ ÛÙËÓ ·È‰È΋ Î·È È‰È·›ÙÂÚ· ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·. ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ µÚ¤ÊÔ˜ ı‹Ï˘, ËÏÈΛ·˜ 20 ÌËÓÒÓ, ÚÔÛ‹Ïı ÛÙËÓ ∞’ ¶/¢ ∫ÏÈÓÈ΋ ÙÔ˘ πÔÎÚ¿ÙÂÈÔ˘ ¡ÔÛÔÎÔÌ›Ԣ £ÂÛÛ·ÏÔӛ΢ ÁÈ· ¤ÏÂÁ¯Ô ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛ˘, Ë ÔÔ›· ‰È·ÈÛÙÒıËΠÛÂ Ù˘¯·›Ô ·ÈÌ·ÙÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô. ¶ÚfiÎÂÈÙ·È ÁÈ· ÙÔ ÌÔÓ·‰ÈÎfi ·È‰› Ê·ÈÓÔÌÂÓÈο ˘ÁÈÒÓ ÁÔÓ¤ˆÓ, ÔÈ ÔÔ›ÔÈ ‰ÂÓ ·Ó·Ê¤ÚÔ˘Ó ÌÂٷ͇ ÙÔ˘˜ Û˘ÁÁ¤ÓÂÈ·. ∆fiÛÔ ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi, fiÛÔ Î·È ÙÔ ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi Ù˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó ÂχıÂÚ·. ∫·Ù¿ ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠÂÚ˘ıÚfiÙËÙ· ÚÔÛÒÔ˘, ¿ÎÚˆÓ ‰·¯Ù‡ÏˆÓ Î·È ‚ÏÂÓÓÔÁfiÓˆÓ, Ë ÔÔ›· ·Ó·Ê¤ÚÂÙ·È fiÙÈ ˘‹Ú¯Â ·fi ÙË Á¤ÓÓËÛË. O ÛÏ‹Ó·˜ ‹Ù·Ó ·„ËÏ¿ÊËÙÔ˜ Î·È Ë ·ÚÙËÚȷ΋ ›ÂÛË Î˘Ì·ÈÓfiÙ·Ó ÛÂ Ê˘ÛÈÔÏÔÁÈο - ÁÈ· ÙËÓ ËÏÈΛ· Ù˘ ·ÛıÂÓÔ‡˜ - ›‰·. ∏ ˘fiÏÔÈË ÎÏÈÓÈ΋ ÂͤٷÛË ‹Ù·Ó ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∞fi ÙÔÓ ·Ú¯ÈÎfi ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ‰È·ÈÛÙÒıËΠȉȷ›ÙÂÚ· ˘„ËÏ‹ ÙÈÌ‹ ·ÈÌÔÛÊ·ÈÚ›Ó˘, ·ÈÌ·ÙÔÎÚ›ÙË Î·È ·ÚÈıÌÔ‡ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ (∏b 17,3 g/dl, Ht 61,5%, RBC 9,3x106/Ìl) ηıÒ˜ Î·È ¯·ÌËÏÔ› ÁÈ· ÙËÓ ËÏÈΛ· Ù˘ ·ÛıÂÓÔ‡˜ ÂÚ˘ıÚÔ΢ÙÙ·ÚÈÎÔ› ‰Â›ÎÙ˜ (MCV 67,4 Ìm3, MCH 18,9 pg, MCHC 28,1%). O ·ÚÈıÌfi˜ ‰ÈÎÙ˘ÔÂÚ˘ÚıÔ΢ÙÙ¿ÚˆÓ ‹Ù·Ó 2,5%, Ô ·ÚÈıÌfi˜ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ 8,3x103/Ìl, Ô ·ÚÈıÌfi˜ ·ÈÌÔÂÙ·Ï›ˆÓ 277x103/Ìl Î·È Ë ÊÂÚÚÈÙ›ÓË 21 ng/ml. ∏ ÌÔÚÊÔÏÔÁ›· ÂÚ˘ıÚÒÓ ¤‰ÂÈÍ ˘Ô¯ÚˆÌ›·, ÌÈÎÚÔ΢ÙÙ¿ÚˆÛË, ·ÓÈÛÔ΢ÙÙ¿ÚˆÛË Î·È ÔÏ˘¯ÚˆÌ·Û›·. O ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‹Ù·Ó ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. O ·ÈÌ·ÙÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÁÔÓ¤ˆÓ ‹Ù·Ó ›Û˘ Ê˘ÛÈÔÏÔÁÈÎfi˜. O Û˘ÓÔÏÈÎfi˜ fiÁÎÔ˜ ÂÚ˘ıÚÒÓ, ÌÂÙ¿ ·fi Ú·‰ÈÔ˚ÛÔÙÔÈ΋ ̤ÙÚËÛË Ì ÛÂÛËÌ·Ṳ̂ӷ ÂÚ˘ıÚ¿, ‚Ú¤ıËΠȉȷ›ÙÂÚ· ·˘ÍË̤ÓÔ˜ (937 ml), ÂÓÒ Ë Ì¿˙· ÙˆÓ ÂÚ˘ıÚÒÓ ‹Ù·Ó 85 ml/kg (Ê˘ÛÈÔÏÔÁÈ΋ ÙÈÌ‹ <35 ml/kg). ∏ ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË ÙˆÓ Ï¢ÎÒÓ ‚Ú¤ıËΠ·˘ÍË̤ÓË (LAPA score=276, Ê˘ÛÈÔÏÔÁÈ΋ ÙÈÌ‹=25-180), ÂÓÒ Ë ÙÈÌ‹ Ù˘ ‚ÈÙ·Ì›Ó˘ µ12 ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∆· ›‰· ÂÚ˘ıÚÔÔÈËÙ›Ó˘ ‚Ú¤ıËÎ·Ó ÂÍ·ÈÚÂÙÈο ·˘ÍË̤ӷ Û ·ÓÂÈÏËÌ̤Ó˜ ÌÂÙÚ‹ÛÂȘ, ÙfiÛÔ ÚÈÓ fiÛÔ Î·È ÌÂÙ¿ ÙËÓ Ú·ÁÌ·ÙÔÔ›ËÛË ·Ê·›Ì·Í˘, Ì ‰È·Î‡Ì·ÓÛË ·fi 800 IU/L ̤¯ÚÈ Î·È 2.500 IU/L (Ê˘ÛÈÔÏÔÁÈ΋ ÙÈÌ‹ 9,4±4,1 IU/L). ∏ ËÏÂÎÙÚÔÊfiÚËÛË Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘, fiˆ˜ Î·È Ô ¤ÏÂÁ¯Ô˜ ÙÔ˘ DNA Ì ÌÔÚȷΤ˜ Ù¯ÓÈΤ˜ ‹Ù·Ó ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∆· ›‰· ÙÔ˘ P50 Î·È ÙÔ˘ 2,3-DPG ‹Ù·Ó ›Û˘ Ê˘ÛÈÔÏÔÁÈο (P50=28 mmHg, 2,3 DPG=5,4 mmol/L).
Paediatriki 2001;64:504-507
∫·Ù¿ ÙËÓ ÂͤٷÛË ÂȯڛÛÌ·ÙÔ˜ ·Ú·Î¤ÓÙËÛ˘ Ì˘ÂÏÔ‡ Î·È ‰Â›ÁÌ·ÙÔ˜ ÔÛÙÂÔÌ˘ÂÏÈ΋˜ ‚ÈÔ„›·˜, ‰È·ÈÛÙÒıËΠ·ÚÔ˘Û›· Î·È ÙˆÓ ÙÚÈÒÓ ÛÂÈÚÒÓ Û fiÏ· Ù· ÛÙ¿‰È· ˆÚ›Ì·ÓÛ˘, Ì ˘ÂÚÏ·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ Î·È ·‡ÍËÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ÂÚ˘ıÚÔ‚Ï·ÛÙÒÓ. ™ÙËÓ Î·ÏÏȤÚÁÂÈ· Ì˘ÂÏÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·Ú·ÙËÚ‹ıËΠ·˘ÙfiÌ·ÙË ·Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, ÌfiÓÔ CFU-E, ¯ˆÚ›˜ ÙËÓ ·ÚÔ˘Û›· ÂÚ˘ıÚÔÔÈËÙ›Ó˘ (4 CFU-E/105 ÌÔÓÔ‡ÚËÓ· ·ÙÙ·Ú· Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ). ∏ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη Ù˘ ·ÛıÂÓÔ‡˜ ·ÔÎ¿Ï˘„ ̛· ‹È· ÂÚÈ‚ÚÔÁ¯È΋ ·ÓÙ›‰Ú·ÛË ·ÌÊÔÙÂÚfiÏ¢ڷ, ÂÓÒ Ì ÙËÓ ·ÏÌÈ΋ Ô͢ÌÂÙÚ›· ‰È·ÈÛÙÒıËΠSaO2 99%. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ¿Óˆ Î·È Î¿Ùˆ ÎÔÈÏ›·˜, ÙÔ Ú·‰ÈÔ˚ÛÔÙÔÈÎfi ÓÂÊÚfiÁÚ·ÌÌ· Î·È Ë Ì·ÁÓËÙÈ΋ ·ÁÁÂÈÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘ ‹Ù·Ó ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. §fiÁˆ Ù˘ Èı·ÓfiÙËÙ·˜ ÂΉ‹ÏˆÛ˘ ıÚÔÌ‚ÔÂÌ‚ÔÏÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ, Ë ·ÛıÂÓ‹˜ Ù¤ıËΠ۠ÚfiÁÚ·ÌÌ· Ù·ÎÙÈÎÒÓ ·Ê·ÈÌ¿ÍÂˆÓ (10-15 ml/kg). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÍ¿ÌËÓ˘ ·Ú·ÎÔÏÔ‡ıËÛ‹˜ Ù˘, ·Ú·Ì¤ÓÂÈ Û ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË Ì¤¯ÚÈ Û‹ÌÂÚ· ¯ˆÚ›˜ ÂÚ·ÈÙ¤Úˆ ÚÔ‚Ï‹Ì·Ù·, ÂÓÒ Ë ÙÈÌ‹ ÙÔ˘ ·ÈÌ·ÙÔÎÚ›ÙË ‰È·ÙËÚÂ›Ù·È ÛÂ Â›Â‰Ô ~55%. ™˘˙‹ÙËÛË ∏ ÚˆÙÔ·ı‹˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË ÛÙ· ·È‰È¿ Â›Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈ·, ÂÓÒ Ë ‰Â˘ÙÂÚÔ·ı‹˜ ··ÓÙ¿Ù·È Û˘¯ÓfiÙÂÚ·, Û˘Ó‹ıˆ˜ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ¯ÚfiÓÈ·˜ ·ÚÙËÚȷ΋˜ ˘ÔÍ›·˜ ‹ ˘ÂÚ¤ÎÎÚÈÛ˘ ÂÚ˘ıÚÔÔÈËÙ›Ó˘ ·fi οÔÈÔÓ fiÁÎÔ (1,2) (¶›Ó·Î·˜ 1). ™ÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È, Ë ·ÛıÂÓ‹˜ ‰ÂÓ ÏËÚÔ‡Û ٷ ÎÚÈÙ‹ÚÈ· Ù˘ ÚˆÙÔ·ıÔ‡˜ ÔÏ˘Î˘ÙÙ·Ú·ÈÌ›·˜ (¶›Ó·Î·˜ 2) (3), ÂÓÒ Ë ·˘ÍË̤ÓË ÙÈÌ‹ Ù˘ ÂÚ˘ıÚÔÔÈËÙ›Ó˘ ‹Ù·Ó Û˘Ì‚·Ù‹ Ì ÙË ‰È¿ÁÓˆÛË ‰Â˘ÙÂÚÔ·ıÔ‡˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛ˘. øÛÙfiÛÔ, ·Ú¿ ÙÔÓ ÂÎÙÂٷ̤ÓÔ ¤ÏÂÁ¯Ô, ‰ÂÓ ‰È·ÈÛÙÒıËΠη̛· ·fi ÙȘ ›ÎÙËÙ˜ ·Èٛ˜ ‰Â˘ÙÂÚÔ·ıÔ‡˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛ˘. ∂Í¿ÏÏÔ˘, ηıÒ˜ ÛÙÔ ÈÛÙÔÚÈÎfi Ù˘ ·ÛıÂÓÔ‡˜ ·Ó·Ê¤ÚÂÙ·È ¯·Ú·ÎÙËÚÈÛÙÈ΋ ÂÚ˘ıÚfiÙËÙ· ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ ·fi ÙË Á¤ÓÓËÛË, Èı·ÓÔÏÔÁԇ̠fiÙÈ Ë ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË ‹Ù·Ó Û˘ÁÁÂÓÔ‡˜ ·ÈÙÈÔÏÔÁ›·˜. OÈ ‰Â˘ÙÂÚÔ·ı›˜ ÌÔÚʤ˜ Ù˘ Û˘ÁÁÂÓÔ‡˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛ˘ ÔÊ›ÏÔÓÙ·È Û˘Ó‹ıˆ˜ ÛÙËÓ ‡·ÚÍË ·ıÔÏÔÁÈÎÒÓ ·ÈÌÔÛÊ·ÈÚÈÓÒÓ (ÌÂı·ÈÌÔÛÊ·ÈÚ›ÓË, ·ÓıÚ·Î˘Ï·ÈÌÔÛÊ·ÈÚ›ÓË, ·ÈÌÔÛÊ·ÈÚ›Ó˜ Ì ·˘ÍË̤ÓË Û˘ÁÁ¤ÓÂÈ· ÚÔ˜ ÙÔ Ô͢ÁfiÓÔ) ‹ Û ÂÓ˙˘ÌÈΤ˜ ‰È·Ù·Ú·¯¤˜ ÙˆÓ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ (ÂÏ·Ùو̤ӷ ›‰· 2,3-DPG) (1,2). ™ÙËÓ ÂÚ›ÙˆÛË ÙÔ˘ ‚Ú¤ÊÔ˘˜ Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È, Ô ¤ÏÂÁ¯Ô˜ ‹Ù·Ó ·ÚÓËÙÈÎfi˜ ÁÈ· ÙËÓ ‡·ÚÍË ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂÈ·˜ ‹ ÂÓ˙˘ÌÔ¿ıÂÈ·˜. ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÔÓÙ·È ÂÚÈÙÒÛÂȘ Û˘ÁÁÂÓÔ‡˜ ‹ ÔÈÎÔÁÂÓÔ‡˜
505
¶·È‰È·ÙÚÈ΋ 2001;64:504-507
Paediatriki 2001;64:504-507
¶›Ó·Î·˜ 1. ∆·ÍÈÓfiÌËÛË ÂÚ˘ıÚÔ΢ÙÙ·ÚÒÛÂˆÓ (11) ¶ÚˆÙÔ·ı‹˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË ñ ™˘ÁÁÂÓ‹˜ - ¢È·Ù·Ú·¯‹ ˘Ô‰Ô¯¤· ÂÚ˘ıÚÔÔÈËÙ›Ó˘
ñ ∂›ÎÙËÙË - ∞ÏËı‹˜ ÔÏ˘Î˘ÙÙ·Ú·ÈÌ›· (polycythemia vera) - π‰ÈÔ·ı‹˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË
¢Â˘ÙÂÚÔ·ı‹˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛË ñ ™˘ÁÁÂÓ‹˜ - ¶·ıÔÏÔÁÈ΋ ·ÈÌÔÛÊ·ÈÚ›ÓË (ÌÂı·ÈÌÔÛÊ·ÈÚ›ÓË, ·ÓıÚ·Î˘Ï·ÈÌÔÛÊ·ÈÚ›ÓË, ·ÈÌÔÛÊ·ÈÚ›ÓË ÌÂ Û˘ÁÁ¤ÓÂÈ· ÚÔ˜ ÙÔ O2) - ∂Ó˙˘ÌÔ¿ıÂÈ· ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ ( 2,3-DPG) - ∞˘ÙfiÓÔÌË ˘ÂÚ¤ÎÎÚÈÛË ÂÚ˘ıÚÔÔÈËÙ›Ó˘(‚Ï¿‚Ë Ô‰Ô‡ ·›ÛıËÛ˘ O2;) ñ ∂›ÎÙËÙË - ÀÔÍ›· (Û˘ÁÁÂÓ‹˜ ηډÈÔ¿ıÂÈ·, Ó¢ÌÔÓÔ¿ıÂÈ·) - ŸÁÎÔÈ (ÓÂÊÚÔ‡, ‹·ÙÔ˜, ·ÚÂÁÎÂÊ·Ï›‰·˜)
¶›Ó·Î·˜ 2. ¡ÂfiÙÂÚ· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ·ÏËıÔ‡˜ ÔÏ˘Î˘ÙÙ·Ú·ÈÌ›·˜ (3) ∞1 ∞2 ∞3 ∞4
∞˘ÍË̤ÓË Ì¿˙· ÂÚ˘ıÚÒÓ (>25% ÊÙ) ∞Ô˘Û›· ·ÈÙ›·˜ ‰Â˘ÙÂÚÔ·ıÔ‡˜ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛ˘ æËÏ·ÊËÙ‹ ÛÏËÓÔÌÂÁ·Ï›· ¶·ÛÚÔ˘Û›· ‰Â›ÎÙË ÎψÓÈÎfiÙËÙ·˜
¢È·ÁÓˆÛÙÈο: ∞1 + ∞2 + ∞3 ‹ ∞4
µ1 µ2
£ÚÔÌ‚Ô΢ÙÙ¿ÚˆÛË (>400 x 109/l) O˘‰ÂÙÂÚÔÊÈÏ›· (>10 x 109/l)
µ3 µ4
™ÏËÓÔÌÂÁ·Ï›· Ô˘ ‰È·ÈÛÙÒÓÂÙ·È Ì ·ÂÈÎÔÓÈÛÙÈΤ˜ ÌÂıfi‰Ô˘˜ ∂Ó‰ÔÁÂÓ›˜ ÂÚ˘ıÚÔÂȉ›˜ ·ÔÈ˘ ‹/Î·È ÂÏ·Ùو̤ÓË ∂ƒO ÔÚÔ‡
∞1 + ∞2 + 2 ·fi Ù· µ
ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛ˘, ÔÈ Ôԛ˜ ‰ÂÓ ÔÊ›ÏÔÓÙ·È ÛÙȘ ÚÔ·Ó·ÊÂÚı›Û˜ ‰È·Ù·Ú·¯¤˜, ·ÏÏ¿ Û ˘ÂÚ¤ÎÎÚÈÛË ÂÚ˘ıÚÔÔÈËÙ›Ó˘ ·Î·ıfiÚÈÛÙ˘ ·ÈÙÈÔÏÔÁ›·˜ (4-7). ¶Èı·ÓÔÏÔÁÂ›Ù·È Ë ‡·ÚÍË ‚Ï¿‚˘ ÛÙÔ ÓÂÊÚÈÎfi ·ÈÛıËÙ‹Ú· ÙÔ˘ Ô͢ÁfiÓÔ˘ ‹ Û οÔÈÔ ÛËÌÂ›Ô Ù˘ Ô‰Ô‡ ·›ÛıËÛ˘ ÙÔ˘ Ô͢ÁfiÓÔ˘ (oxygen sensing pathway) (6-8), Èı·ÓfiÓ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ˘ÂÚ¤ÎÎÚÈÛ˘ ÙÔ˘ hypoxia-inducable factor-1 (HIF-1) (7). ∂›Ó·È ÁÓˆÛÙfi fiÙÈ Ë ÂÓ‰ÔÁÂÓ‹˜ ·Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ CFU-E ÛÙËÓ Î·ÏÏȤÚÁÂÈ· ΢ÙÙ¿ÚˆÓ, ¯ˆÚ›˜ ÙËÓ ·ÚÔ˘Û›· ÂÚ˘ıÚÔÔÈËÙ›Ó˘, Â›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈÎfi Ù˘ ÚˆÙÔ·ıÔ‡˜ ÔÏ˘Î˘ÙÙ·Ú·ÈÌ›·˜ (polycythemia vera) (1,2). ™ÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È fiˆ˜ Î·È Û ¿ÏϘ ·Ó·ÊÂÚfiÌÂÓ˜ ÛÙË ‰ÈÂıÓ‹ ‚È‚ÈÏÈÔÁÚ·Ê›· ÂÚÈÙÒÛÂȘ, ‰È·ÈÛÙÒıËΠ·˘ÙfiÌ·ÙË ·Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ (CFU-E) ¯ˆÚ›˜ Ó· ÏËÚÔ‡ÓÙ·È Ù· ÎÚÈÙ‹ÚÈ· ‰È¿ÁÓˆÛ˘ Ù˘ ÚˆÙÔ·ıÔ‡˜ ÔÏ˘Î˘ÙÙ·Ú·ÈÌ›·˜ (4,9,10). ∞ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ Ì˯·ÓÈÛÌfi ˘ÂÚ¤ÎÎÚÈÛ˘ ÂÚ˘ıÚÔÔÈËÙ›Ó˘, Ô Î›Ó‰˘ÓÔ˜ ÂΉ‹ÏˆÛ˘ ıÚÔÌ‚ÔÂÌ‚ÔÏÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ˘ÂÚÁÏÔÈfiÙËÙ·˜ ÙÔ˘ ·›Ì·ÙÔ˜ ·Ú·Ì¤ÓÂÈ. ∏ ıÂڷ¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÛ˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ Ú·ÁÌ·ÙÔÔ›ËÛË ·Ê·È͈̿Ó, ÙË ¯ÔÚ‹ÁËÛË ˘‰ÚÔ͢Ԣڛ·˜ ‹ Ì˘ÂÏÔηٷÛÙ·ÏÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (1,2). ™ÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜, ÏfiÁˆ ÙÔ˘ ÌÈÎÚÔ‡ Ù˘ ËÏÈΛ·˜ Ù˘ ·ÛıÂÓÔ‡˜, ˆ˜ ıÂڷ›· ÂÎÏÔÁ‹˜ ÎÚ›ıËÎÂ Ë Ú·ÁÌ·ÙÔÔ›ËÛË Ù·ÎÙÈÎÒÓ ·Ê·È͈̿Ó, Ì ÛÎÔfi ÙË ‰È·Ù‹ÚËÛË ÙÔ˘ ·ÈÌ·ÙÔÎÚ›ÙË Û ¯·ÌËÏ¿ ›‰·, Ì ٷ˘Ùfi¯ÚÔÓÔ ¤ÏÂÁ¯Ô ÙˆÓ ÂȤ‰ˆÓ Ù˘ ÊÂÚÚÈÙ›Ó˘, ηıÒ˜ ÔÈ Û˘¯Ó¤˜ ·Ê·ÈÌ¿ÍÂȘ Ô‰ËÁÔ‡Ó Û˘¯Ó¿ Û ÛȉËÚÔÂÓ›·.
506
∏ ‰È¢ÎÚ›ÓÈÛË Ù˘ ·ÎÚÈ‚Ô‡˜ ÌÔÚȷ΋˜ ‚Ï¿‚˘ Ô˘ Ô‰ËÁ› ÛÙËÓ ˘ÂÚ¤ÎÎÚÈÛË Ù˘ ÂÚ˘ıÚÔÔÈËÙ›Ó˘, ı· ÌÔÚ¤ÛÂÈ ›Ûˆ˜ ÛÙÔ Ì¤ÏÏÔÓ Ó· ‚ÔËı‹ÛÂÈ ÛÙÔÓ Î·ıÔÚÈÛÌfi Ù˘ ÛˆÛÙ‹˜ ·ÓÙÈÌÂÙÒÈÛ˘ fiˆ˜ Î·È Ù˘ ÚfiÁÓˆÛ˘, ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙË ‰È·Ù·Ú·¯‹ ·˘Ù‹. ∂˘¯·ÚÈÛٛ˜ ∂˘¯·ÚÈÛÙԇ̠ÙÔÓ ¶·È‰›·ÙÚÔ-°ÂÓÂÙÈÛÙ‹ ∞Ó·ÏËÚˆÙ‹ ∫·ıËÁËÙ‹ Î. ª. ∫·Ó·‚¿ÎË ·fi ÙÔ ∂ÚÁ·ÛÙ‹ÚÈÔ π·ÙÚÈ΋˜ °ÂÓÂÙÈ΋˜ ÙÔ˘ ∂ıÓÈÎÔ‡ Î·È ∫·Ô‰ÈÛÙÚÈ·ÎÔ‡ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ηıÒ˜ Î·È ÙËÓ ∞ÈÌ·ÙÔÏfiÁÔ Î. ∂. πˆ·ÓÓ›‰Ô˘, ∂›ÎÔ˘ÚË ∫·ıËÁ‹ÙÚÈ· Ù˘ µ’ ¶/£ ∫ÏÈÓÈ΋˜ ∞¶£ ÁÈ· ÙË Û˘Ì‚ÔÏ‹ ÙÔ˘˜ ÛÙËÓ ÂÚÁ·ÛÙËÚȷ΋ ‰ÈÂÚ‡ÓËÛË Ù˘ ·ÛıÂÓÔ‡˜ Ì·˜.
µÈ‚ÏÈÔÁÚ·Ê›· 1. Nathan DG, Oski FA, eds. Therapeutic plasma exchange and cytapheresis. In: Hematology of Infancy and Childhood. 4th ed. W.B. Saunders Company; 1993. p. 1823-1824. 2. Hoffman R, Benz EJ, Shattil SJ, Furil B, Cohen HJ, Silberstein LE, eds. Diseases of the blood: Polycythemia. In: Hematology. Basic Principles and Practice. 2nd ed. Churchill Livingstone Inc; 1995. p. 1056-1057. 3. Pearson TC, Messinezy H. The diagnostic criteria of polycythemia rubra vera. Leuk Lymphoma 1996;22(Suppl 1):87-93. 4. Borgna-Pignatti C, Liberato NL, Marradi P, Rosti V, Barosi G. Regulation of erythropoietin production in a case of congenital erythropoietin-dependent pure erythrocytosis. Am J Hematol 1994;46:348-353. 5. Yonemitsu H, Yamaghuchi K, Shigeta H, Okuda K, Takaku F. Two cases of familial erythrocytosis with increased erythropoietin activity in plasma and urine. Blood 1973;42:793-797. 6. Manglani M, DeGroff C, Dukes P, Ettinger L. Congenital
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erythrocytosis with elevated erythropoietin level: An incorrectly set “erythrostat?”. J Pediatr Hematol Oncol 1998;20:560-562. Sokol L, Kralovics R, Ren S, Luhovy M, Prchal JF, Prchal JT. Secondary polycythemia with putative defect in HIF-1 transcription activation pathway. Blood 1995;86:312a. Whitcomb WH, Peschle C, Moore M, Nitschke R, Adamson J. Congenital erythrocytocis: a new form associated with an erythropoietin-dependant mechanism. Br J Haematol 1980;44:17-24. Mankad V, Moore B, Mc Ryan D, Zuckerman K. Erythrocytosis associated with spontaneous erythroid colony formation and idiopathic hypererythropoietinemia. J Pediatr 1987;111:743-745. Dainiak N, Hoffman R, Lewbowitz AI, Solomon L, Maffei L, Ritsey K. Erythropoietin-dependent primary pure
erythrocytosis. Blood 1979;53:1076-1084. 11. ¶·ÓÙÂÏ›‰Ô˘ ¢, ∏ÏÈ¿Î˘ ¶. ¢È¿ÁÓˆÛË-∫ÏÈÓÈΤ˜ ∂ΉËÏÒÛÂȘ-∂ͤÏÈÍË Ù˘ ∞ÏËıÔ‡˜ ¶ÔÏ˘Î˘ÙÙ·Ú·ÈÌ›·˜. ª˘ÂÏÔ¸ÂÚÏ·ÛÙÈο ™‡Ó‰ÚÔÌ·. ∂Ù‹ÛÈÔ ªÂÙÂÎ·È‰Â˘ÙÈÎfi ™ÂÌÈÓ¿ÚÈÔ. £ÂÛÛ·ÏÔÓ›ÎË, ∞ı‹Ó·; ª¿ÈÔ˜-πÔ‡ÓÈÔ˜ 2000. ÛÂÏ. 72-77.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 18-01-2001 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 05-06-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ªÈÚ¿ÓÙ· ∞ı·Ó·Û›Ô˘ ∞Á. ™ÔÊ›·˜ 30, 546 22, £ÂÛÛ·ÏÔÓ›ÎË
¶ÂÚ›ÏË„Ë ¿ÚıÚÔ˘ ·fi ÙË µÈ‚ÏÈÔÁÚ·Ê›· ™È‰ËÚÔÂÓ›· Î·È ÁÓˆÛÙÈ΋ ·ÓÙ›ÏË„Ë Û ·È‰È¿ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ Î·È ÂÊ‹‚Ô˘˜ ÛÙȘ ∏¶∞1 ∂ÈÛ·ÁˆÁ‹: OÈ Halterman Î·È Û˘Ó ·fi ÙËÓ π·ÙÚÈ΋ ™¯ÔÏ‹ Rochester Ù˘ ¡¤·˜ ÀfiÚ΢ ÛÙȘ ∏¶∞, ·Ó·Ê¤ÚÔ˘Ó fiÙÈ Ù· ·È‰È¿ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ Î·È ÔÈ ¤ÊË‚ÔÈ Ì ÛȉËÚÔÂÓ›·, ¯ˆÚ›˜ ··Ú·›ÙËÙ· Ì ·Ó·ÈÌ›·, ¤¯Ô˘Ó ¯·ÌËϤ˜ ÂȉfiÛÂȘ ÛÂ Ù˘ÔÔÈË̤Ó˜ Ì·ıËÌ·ÙÈΤ˜ ‰ÔÎÈ̷ۛ˜. ™Ù· ‚Ú¤ÊË, Â›Ó·È ÁÓˆÛÙfi fiÙÈ Ë ÛȉËÚÔÂÓÈ΋ ·Ó·ÈÌ›· ÚÔηÏ› ‰È·Ù·Ú·¯¤˜ ÛÙËÓ ·Ó¿Ù˘ÍË. ∏ ›‰Ú·ÛË, fï˜, Ù˘ ÛȉËÚÔÂÓÈ΋˜ ·Ó·ÈÌ›·˜ ‹ ·ÎfiÌË Î·È Ù˘ ÛȉËÚÔÂÓ›·˜, ÛÙË ÁÓˆÛÙÈ΋ ·ÓÙ›ÏË„Ë ·È‰ÈÒÓ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ Î·È ÂÊ‹‚ˆÓ ·Ú·Ì¤ÓÂÈ Û¯ÂÙÈο ·‰È¢ÎÚ›ÓÈÛÙË. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: OÈ ÂÚ¢ÓËÙ¤˜ Halterman Î·È Û˘Ó ¯ÚËÛÈÌÔÔ›ËÛ·Ó ‰Â‰Ô̤ӷ ·fi ÙËÓ ÂıÓÈ΋ ÌÂϤÙË “National Health and Nutrition Examination Survey III1988-1994” Û¯ÂÙÈο Ì ÙËÓ Â¿ÚÎÂÈ· Ûȉ‹ÚÔ˘ ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi ·È‰ÈÒÓ ËÏÈΛ·˜ 6-16 ÂÙÒÓ, fiˆ˜ Â›Ó·È Ô ÎÔÚÂÛÌfi˜ ÙÚ·ÓÛÊÂÚÚ›Ó˘, Ë ÂχıÂÚË ÚˆÙÔÔÚÊ˘Ú›ÓË ÂÚ˘ıÚÒÓ Î·È Ë ÊÂÚÚÈÙ›ÓË ÛÙÔÓ ÔÚfi. ∂¿Ó ‰‡Ô ·fi ÙȘ ÙÚÂȘ ·Ú·¿Óˆ ·Ú·Ì¤ÙÚÔ˘˜ ‹Ù·Ó ·ıÔÏÔÁÈΤ˜ ÁÈ· ÙËÓ ËÏÈΛ· Î·È ÙÔ Ê‡ÏÔ ÙÔ˘ ·È‰ÈÔ‡, ÙfiÙ ÙÔ ·È‰› ·˘Ùfi ÔÚÈ˙fiÙ·Ó fiÙÈ Â›¯Â ÛȉËÚÔÂÓ›·. ∏ ÙÈÌ‹ Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ ¯ÚËÛÈÌÔÔÈ‹ıËΠÁÈ· ÙÔÓ Î·ıÔÚÈÛÌfi Ù˘ ·Ó·ÈÌ›·˜. ∞ÔÙÂϤÛÌ·Ù·: ∞fi Ù· 5398 ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ù˘ ÂıÓÈ΋˜ ÌÂϤÙ˘, ¤Ó· ÔÛÔÛÙfi 3% ›¯Â ÛȉËÚÔÂÓ›·. ∆Ô ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÛȉËÚÔÂÓ›·˜ (8,7%) ·Ú·ÙËÚ‹ıËΠÛÙ· ÎÔÚ›ÙÛÈ· ÂÊË‚È΋˜ ËÏÈΛ·˜. OÈ ÂÚ¢ÓËÙ¤˜ Û˘Ó¤ÎÚÈÓ·Ó ÙȘ ‚·ıÌÔÏÔÁ›Â˜ ·fi Ù˘ÔÔÈË̤Ó˜ Ì·ıËÌ·ÙÈΤ˜ ‰ÔÎÈ̷ۛ˜, ÛÙȘ Ôԛ˜ ˘Ô‚Ï‹ıËÎ·Ó ·È‰È¿ ¯ˆÚ›˜ ÛȉËÚÔÂÓ›·, ·È‰È¿ Ì ÛȉËÚÔ-
ÂÓ›· ·ÏÏ¿ ¯ˆÚ›˜ ·Ó·ÈÌ›· Î·È ·È‰È¿ Ì ÛȉËÚÔÂÓÈ΋ ·Ó·ÈÌ›·. ¢È·ÈÛÙÒıËΠfiÙÈ Ù· ·È‰È¿ Ì ÛȉËÚÔÂÓ›· (Ì ‹ ¯ˆÚ›˜ ·Ó·ÈÌ›·) ›¯·Ó ¿Óˆ ·fi ‰‡Ô ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË Èı·ÓfiÙËÙ· Ó· ‚·ıÌÔÏÔÁËıÔ‡Ó Î¿Ùˆ ·fi ÙÔ Ì¤ÛÔ fiÚÔ ÛÙȘ Ì·ıËÌ·ÙÈΤ˜ ‰ÔÎÈ̷ۛ˜ Û ۇÁÎÚÈÛË Ì ٷ ·È‰È¿ ¯ˆÚ›˜ ÛȉËÚÔÂÓ›·. ∞˘Ù‹ Ë Û˘Û¯¤ÙÈÛË ·Ú¤ÌÂÈÓ ÈÛ¯˘Ú‹, ·ÎfiÌË Î·È ÛÙ· ·È‰È¿ Ì ÛȉËÚÔÂÓ›· Ù· ÔÔ›· ‰ÂÓ Â›¯·Ó ·Ó·ÈÌ›·. ™˘ÌÂÚ¿ÛÌ·Ù·: OÈ Halterman Î·È Û˘Ó Û˘ÌÂÚ·›ÓÔ˘Ó fiÙÈ ¯ÚÂÈ¿˙ÂÙ·È ÂÚ·ÈÙ¤Úˆ ÌÂϤÙË ÁÈ· Ó· ηıÔÚÈÛÙ› Â¿Ó Ô Ì·˙ÈÎfi˜ ¤ÏÂÁ¯Ô˜ (screening) ·È‰ÈÒÓ Û ˘„ËÏfi ΛӉ˘ÓÔ ÁÈ· ÛȉËÚÔÂÓ›· Î·È Ë ıÂڷ›· ÙÔ˘˜ ·ÔÙÂÏ› ÂӉ‰ÂÈÁ̤ÓË Ù·ÎÙÈ΋ Î·È ÙÔÓ›˙Ô˘Ó fiÙÈ ·Ó Ù· Â˘Ú‹Ì·Ù· Ù˘ ÂÚÁ·Û›·˜ ÙÔ˘˜ ÂȂ‚·ÈˆıÔ‡Ó Û ÌÂÏÏÔÓÙÈΤ˜ ÚÔÔÙÈΤ˜ ÌÂϤÙ˜, ›Ûˆ˜ ¯ÚÂÈ·ÛÙ› Ó· Á›ÓÂÙ·È ¤ÏÂÁ¯Ô˜ ÛÙ· ÎÔÚ›ÙÛÈ· ÂÊË‚È΋˜ ËÏÈΛ·˜ ÁÈ· ÛȉËÚÔÂÓ›·.
1
Halterman JS, Kaczorowski JM, Allgne CA, Auinger P, Szilagyl PG Iron deficiency and cognitive achievement among school-aged children and adolescents in the United States Pediatrics 2001;107:1381-1386
∞ϤͷӉÚÔ˜ Ã. ª¿Î˘ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ πˆ·ÓÓ›ÓˆÓ
507
¶·È‰È·ÙÚÈ΋ 2001;64:508-513
∂¡¢π∞º∂ƒOÀ™∞ ¶∂ƒπ¶∆ø™∏
Paediatriki 2001;64:508-513
CASE REPORT
™˘ÁÁÂÓ‹˜ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· ·fi ¤ÏÏÂÈ„Ë Ï·ÌÈÓ›Ó˘ ·2 Ã. ¶. ¶·ÓÙÂÏÈ¿‰Ë˜1, ¡. ºÏ¿Ú˘2, ∂. ∫·Ú·Ù˙¿-•ÈÊÈÏ›‰Ô˘1, π. •˘ÓÈ¿˜1
Congenital muscular dystrophy with laminin-a2 deficiency C. Panteliadis1, N. Flaris2, E. Karatza-Xifilidou1, J. Xinias1
¶ÂÚ›ÏË„Ë: ∏ Û˘ÁÁÂÓ‹˜ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· Â›Ó·È ÂÙÂÚÔÁÂÓ‹˜ ÔÌ¿‰· Ó¢ÚÔÌ˘˚ÎÒÓ ·ı‹ÛÂˆÓ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi Ì˘˚΋ ·‰˘Ó·Ì›· Î·È ˘ÔÙÔÓ›· ·fi ÙË Á¤ÓÓËÛË ‹ ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ Ù˘ ˙ˆ‹˜. ªÂÙ·‚È‚¿˙ÂÙ·È Ì ÙÔ ÛˆÌ·ÙÈÎfi ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú· Î·È ÛÙÔ 50% ÂÚ›Ô˘ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÔÊ›ÏÂÙ·È ÛÙËÓ ¤ÏÏÂÈ„Ë Ù˘ Ï·ÌÈÓ›Ó˘ ·2 ‹ ÌÂÚÔÛ›Ó˘. ¶ÂÚÈÁÚ¿ÊÂÙ·È ÂÚ›ÙˆÛË ‚Ú¤ÊÔ˘˜ ÌÂ Û˘ÁÁÂÓ‹ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· ·fi ¤ÏÏÂÈ„Ë Ï·ÌÈÓ›Ó˘ ·2 Ì ¤Ó·ÚÍË Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜ ·fi ÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·. ∏ ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ ÛÙËÚ›¯ıËΠÛÙ· ÎÏÈÓÈο Û˘ÌÙÒÌ·Ù·, ÛÙ· ÂÓ‰ÂÈÎÙÈο ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Î·È ÂȂ‚·ÈÒıËΠ̠ÙËÓ ÈÛÙÔÏÔÁÈ΋ Î·È ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈ΋ ÌÂϤÙË Ù˘ ‚ÈÔ„›·˜ Ì˘fi˜.
Abstract: Congenital muscular dystrophy is a heterogeneous group of neuromuscular disorders characterized by muscle weakness and hypotonia at birth or within the first few months of life. It is inherited in an autosomal recessive pattern. About half of the patients have a deficiency of the alpha 2-chain of laminin-2 (merosin). We describe a case of congenital muscular dystrophy in an infant with laminin a2-chain deficiency, whose onset of symptoms appeared in early infancy. The diagnosis was based on clinical symptoms as well as on laboratory findings, and was confirmed by histological and immunohistochemical studies on muscle biopsy.
§¤ÍÂȘ ÎÏÂȉȿ: Ì˘˚΋ ‰˘ÛÙÚÔÊ›·, Ï·ÌÈÓ›ÓË ·2.
Key words: muscular dystrophy, laminin a2.
∂ÈÛ·ÁˆÁ‹ OÈ Ì˘˚Τ˜ ‰˘ÛÙÚÔʛ˜ Â›Ó·È Ì›· ÔÌ¿‰· ÂÎÊ˘ÏÈÛÙÈÎÒÓ ÓfiÛˆÓ ÙˆÓ Ì˘ÒÓ Ô˘ ÌÂÙ·‰›‰ÔÓÙ·È ÁÂÓÂÙÈο. ∏ Û˘ÁÁÂÓ‹˜ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· (™ª¢) ÂΉËÏÒÓÂÙ·È Ì ÙË Á¤ÓÓËÛË ‹ Ï›ÁÔ˘˜ Ì‹Ó˜ ÌÂÙ¿ (1-4). O ¯·Ú·ÎÙËÚÈÛÌfi˜ Ù˘ ™ª¢ ‰›ÓÂÙ·È Û ·È‰È¿ Ô˘ ÁÂÓÓÈÔ‡ÓÙ·È Ì ˘ÔÙÔÓ›· Î·È ·‰˘Ó·Ì›·, Û˘¯Ó¿ Ì ·ÚıÚÔÁÚ‡ˆÛË ‹ Û˘Áο̄ÂȘ Ì˘ÒÓ, ÙˆÓ ÔÔ›ˆÓ Ë ÎÈÓËÙÈ΋ ·Ó¿Ù˘ÍË ˘ÔÏ›ÂÙ·È ÛËÌ·ÓÙÈο Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ (1-4). ∏ ÎÏÈÓÈ΋ ÔÚ›· ÙˆÓ ·ÛıÂÓÒÓ ÂȉÂÈÓÒÓÂÙ·È ÚÔԉ¢ÙÈο. ¶ÂÚÈÁڷʤ˜ Ù˘ ÓfiÛÔ˘ ·fi ÙË ‰ÂηÂÙ›· ÙÔ˘ 1970 Ù·ÍÈÓÔÌÔ‡Ó ÙË ™ª¢ fi¯È ·Ó¿ÏÔÁ· Ì ÙÔ Ú˘ıÌfi ÂͤÏÈÍ‹˜ Ù˘, ·ÏÏ¿ Ì ÙÔ Î·Ù¿ fiÛÔ Û˘Ó˘¿Ú¯ÂÈ ÚÔÛ‚ÔÏ‹ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·-
ÙÔ˜ (∫¡™). ¶ÚÒÙ· ÔÈ Fukuyama Î·È Û˘Ó (5) Î·È ·ÚÁfiÙÂÚ· ÔÈ Fowler Î·È Manson (6) ÂÚȤÁÚ·„·Ó ·È‰È¿ Ù· ÔÔ›· ÁÂÓÓ‹ıËÎ·Ó ˘ÔÙÔÓÈο ÌÂ Û˘Áο̄ÂȘ Ì˘ÒÓ, ÓÂ˘Ì·ÙÈ΋ ηı˘ÛÙ¤ÚËÛË Î·È ·ÚÔ˘Û›·˙·Ó ÂÎÙÂٷ̤Ó˜ ·Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÛÙÔ ∫¡™. OÈ Santavuori Î·È Û˘Ó (7) ÂÚȤÁÚ·„·Ó ÙË ÓfiÛÔ ÔÊı·ÏÌÒÓ, Ì˘ÒÓ, ÂÁÎÂÊ¿ÏÔ˘, Ë ÔÔ›· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ™ª¢, ÓÔËÙÈ΋ ηı˘ÛÙ¤ÚËÛË Î·È ÔÊı·ÏÌÈΤ˜ ·ÏÏÔÈÒÛÂȘ Û ·ÛıÂÓ›˜ ·fi ÙË ºÈÓÏ·Ó‰›·. ∏ Û˘ÓÔ‰fi˜ Û·ÛÙÈÎfiÙËÙ· Î·È Ù· ÔÊı·ÏÌÈο Â˘Ú‹Ì·Ù· ÙÔ ‰È·¯ˆÚ›˙Ô˘Ó ·fi ÙË ÌÔÚÊ‹ Fukuyama Ù˘ ™ª¢ (™ª¢F). ∆Ô Û‡Ó‰ÚÔÌÔ WalkerWarburg (™WW) ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ™ª¢, ˘‰ÚÔΤʷÏÔ, Ì·ÎÚÔÎÂÊ·Ï›·, ÏÈÛÂÁÎÂÊ·Ï›· Ù‡Ô˘ 2, ·Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ Ù˘ ·ÚÂÁÎÂÊ·Ï›‰·˜, ÙÔ˘
1 °’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£ πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢ 2 ¶·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎfi ∂ÚÁ·ÛÙ‹ÚÈÔ πÔÎÚ·Ù›Ԣ ¡ÔÛÔÎÔÌ›Ԣ £ÂÛÛ·ÏÔӛ΢
1 3rd Pediatric Department of Aristotelion University Ippokration General Hospital, Thessaloniki 2 Pathology Laboratory Ippokration General Hospital, Thessaloniki
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·ÌÊÈ‚ÏËÛÙÚÔÂȉԇ˜ Î·È ÁÈ’ ·˘Ùfi ıˆÚÂ›Ù·È ‰È·ÊÔÚÂÙÈÎfi Ù˘ ™ª¢F (8). ∆Ô 1986, Ô Brooke ‰È·ÈÛÙÒÓÂÈ fiÙÈ ÁÈ· ¤Ó· ÌÂÁ¿ÏÔ ·ÚÈıÌfi ·È‰ÈÒÓ Ô˘ ÁÂÓÓÈÔ‡ÓÙ·È Ì ˘ÔÙÔÓ›·, Û˘Û¿ÛÂȘ Ì˘ÒÓ Î·È ¿ÏÏ· ÛÙÔȯ›· ÂÓ‰ÂÈÎÙÈο ÚÔÛ‚ÔÏ‹˜ ÙˆÓ Ì˘ÒÓ, ¯ÚÂÈ¿˙ÂÙ·È Ì›· ·‰È·ÌÊÈÛ‚‹ÙËÙË ‚ÈÔ¯ËÌÈ΋ ‰ÔÎÈÌ·Û›·, Ë ÔÔ›· ı· ÌÔÚ› Ó· ‰È·¯ˆÚ›ÛÂÈ ÙȘ ‰È¿ÊÔÚ˜ ÌÔÚʤ˜ Ù˘ ÓfiÛÔ˘ (1,2). ŒÙÛÈ, ÚfiÛÊ·Ù· ÂÚÈÁÚ¿ÊËÎÂ Ë ¤ÏÏÂÈ„Ë Ù˘ ÌÂÚÔÛ›Ó˘ (Ï·ÌÈÓ›ÓË-2) ÛÙË ‚·ÛÈ΋ ÌÂÌ‚Ú¿ÓË ÙˆÓ Ì˘˚ÎÒÓ Î˘ÙÙ¿ÚˆÓ, ÛÙË ÌÔÚÊ‹ Ù˘ ™M¢ Ô˘ ÁÂÓÂÙÈο Û˘Ó‰¤ÂÙ·È Ì ÙÔ ¯ÚˆÌfiۈ̷ 6q22-23 Î·È Ë ÔÔ›· ·Ú·ÙËÚÂ›Ù·È Û˘¯ÓfiÙÂÚ· ÛÙÔ˘˜ ∂˘Úˆ·˚ÎÔ‡˜ Ï·Ô‡˜. ∏ ¤ÏÏÂÈ„Ë ·˘Ù‹ ÔÊ›ÏÂÙ·È Û ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ÌÂÚÔÛ›Ó˘ (9,10). ªÂ ÙËÓ ·Ó·Î¿Ï˘„Ë ·˘Ù‹ ‰È·¯ˆÚ›ÛÙËÎ·Ó ÔÈ ÌÔÚʤ˜ ™ª¢ Û ·ÚÓËÙÈΤ˜ Î·È ıÂÙÈΤ˜ ÁÈ· ÌÂÚÔÛ›ÓË (11-15). ™ÙËÓ ·ÚÔ‡Û· ÂÚÁ·Û›· ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ‚Ú¤ÊÔ˘˜ Ì ™ª¢ ·ÚÓËÙÈ΋ ÁÈ· ÌÂÚÔÛ›ÓË Î·È Á›ÓÂÙ·È Û˘˙‹ÙËÛË ÁÈ· ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ·, ÙÔ˘˜ ÙÚfiÔ˘˜ ‰È¿ÁÓˆÛ˘, ÙËÓ ÚfiÁÓˆÛË Î·È ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ·˘Ù‹ ÙË ÌÔÚÊ‹ Ù˘ Û˘ÁÁÂÓÔ‡˜ Ì˘˚΋˜ ‰˘ÛÙÚÔÊ›·˜. ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ £‹Ï˘ ‚Ú¤ÊÔ˜ 57 ËÌÂÚÒÓ ÂÈÛ¿ÁÂÙ·È ÁÈ· ¤ÏÂÁ¯Ô ÁÂÓÈÎÂ˘Ì¤Ó˘ ˘ÔÙÔÓ›·˜, ÓˆıÚfiÙËÙ·˜ Î·È ÂÏ·Ùو̤Ó˘ ÚfiÛÏ˄˘ ‚¿ÚÔ˘˜. ∏ ·ËÛË, Ô ÙÔÎÂÙfi˜ Î·È Ë ÂÚÈÁÂÓÓËÙÈ΋ ÂÚ›Ô‰Ô˜ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ‹Ù·Ó ¯ˆÚ›˜ ÚÔ‚Ï‹Ì·Ù·. ∆Ô ·È‰› ÁÂÓÓ‹ıËΠÙÂÏÂÈfiÌËÓÔ, Ì ‚¿ÚÔ˜ 3450 gr, Ì‹ÎÔ˜ 52 cm Î·È ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜ 35,5 cm. ™ÙËÓ ËÏÈΛ· ÙˆÓ 57 ËÌÂÚÒÓ Â›¯Â ‚¿ÚÔ˜ 4060 gr (10Ë ∂£), Ì‹ÎÔ˜ 58 cm (75Ë ∂£) Î·È ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜ 38 cm (25Ë ∂£). ∏ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË Î·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈο Â˘Ú‹Ì·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi, ΢ÎÏÔÊÔÚÈÎfi Î·È Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ·. ∫·Ù¿ ÙË Ó¢ÚÔ-·Ó·Ù˘Íȷ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠÁÂÓÈÎÂ˘Ì¤ÓË ˘ÔÙÔÓ›· ÌÂ Ì˘˚΋ ·‰˘Ó·Ì›· ÛÙ· ¿Óˆ ¿ÎÚ· Î·È Ì¤ÙÚÈ· ÛÙ· οو Ì ÂÏ¿¯ÈÛÙ˜ ÂÓÂÚÁËÙÈΤ˜ ÎÈÓ‹ÛÂȘ, ·‰˘Ó·Ì›· ÛÙ‹ÚÈ͢ ÎÂÊ·Ï‹˜, ·Ô˘Û›· ·Ú¯¤ÁÔÓˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ. OÈ ÙÂÓfiÓÙȘ ·ÓÙ·Ó·ÎÏ¿ÛÂȘ ÛÙ· ¿Óˆ Î·È Î¿Ùˆ ¿ÎÚ· ‹Ù·Ó ÂÏ·Ùو̤Ó˜. ∞fi ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ‰È·ÈÛÙÒıËΠ̤ÙÚÈ· ·‡ÍËÛË ÙˆÓ ÙÚ·ÓÛ·ÌÈÓ·ÛÒÓ (SGOT 205 mU/ml, SGPT 150 mU/ml, ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ¤ˆ˜ 40 mU/ml) Î·È Ôχ ˘„ËϤ˜ ÙÈ̤˜ ÎÚ·ÙÈÓ›Ó˘ ʈÛÊÔÎÈÓ¿Û˘ (CPK), 8400 mU/ml Î·È Á·Ï·ÎÙÈ΋˜ ‰Â¸‰ÚÔÁÂÓ¿Û˘ LDH, 1373 mU/ml (Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ 25-190 mU/ml Î·È 225-400 mU/ml, ·ÓÙ›ÛÙÔȯ·). O ¤ÏÂÁ¯Ô˜ ÁÈ· Ù· Û˘ÓËı¤ÛÙÂÚ· ÓÔÛ‹Ì·Ù· ÌÂÙ·‚ÔÏÈÛÌÔ‡, fiˆ˜ ·ÌÈÓfiÁÚ·ÌÌ·, ۷ί·ÚfiÁÚ·ÌÌ· ÔÚÔ‡ Î·È Ô‡ÚˆÓ, ÔÚÁ·ÓÈο Ôͤ·, ·Ì̈ӛ·, Á·Ï·-
Paediatriki 2001;64:508-513
ÎÙÈÎfi Ô͇ ÔÚÔ‡ Î·È ÔÍÂÔ‚·ÛÈ΋ ÈÛÔÚÚÔ›· ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. ∆· ·ÎÔ˘ÛÙÈο Î·È ÔÙÈο ÚÔÎÏËÙ¿ ‰˘Ó·ÌÈο ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο Î·È ÙÔ ËÏÂÎÙÚÔÌ˘ÔÁÚ¿ÊËÌ· (ÚfiÛıÈÔ˜ ÎÓËÌÈ·›Ô˜ Î·È ÙÂÙڷΤʷÏÔ˜) Ì˘Ô·ıËÙÈÎÔ‡ Ù‡Ô˘. O ¤ÏÂÁ¯Ô˜ ÙÔ˘ ∫¡™, ·Ú¯Èο Ì ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· (CT) ÂÁÎÂÊ¿ÏÔ˘, ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜ Î·È ÛÙË Û˘Ó¤¯ÂÈ·, Ì ̷ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· (MRI), ¤‰ÂÈÍ ÂÈÎfiÓ· ˘ÔÌ˘ÂϛӈÛ˘, Èı·Ó¿ ÛÙ· Ï·›ÛÈ· ÓfiÛÔ˘ Û˘ÁÁÂÓÔ‡˜ Ì˘˚΋˜ ‰˘ÛÙÚÔÊ›·˜. ™ÙËÓ ∆2 ·ÎÔÏÔ˘ı›· ηٷÁÚ¿ÊËΠÂÚÈÎÔÈÏȷο ˘„ËÏfi Ì·ÁÓËÙÈÎfi Û‹Ì·, ÂÓÒ ÛÙËÓ ∆1 ·ÎÔÏÔ˘ı›· ¯·ÌËÏfi. ∏ MRI ÂÁÎÂÊ¿ÏÔ˘ ¤‰ÂÈÍ ·ÎfiÌË ÔÏÈÁÔÁ˘Ú›· Î·È ·Á˘Ú›· ÛÙÔ˘˜ ÈÓÈ·ÎÔ‡˜ ÏÔ‚Ô‡˜, ηıÒ˜ Î·È ÊÏÔÈ˚΋ ‰˘ÛÏ·Û›· Á‡Úˆ ·fi ÙËÓ ·ÚÈÛÙÂÚ‹ Û¯ÈÛÌ‹ ÙÔ˘ Silvius, ·Û˘ÌÌÂÙÚ›· ÙˆÓ ÂÁÎÂÊ·ÏÈÎÒÓ ËÌÈÛÊ·ÈÚ›ˆÓ, ˘ÂÚÛÎËÓȉȷÎÒÓ Î·È ˘·Ú·¯ÓÔÂȉÒÓ ¯ÒÚˆÓ (∂ÈÎfiÓ· 1). ∆· ·ÓˆÙ¤Úˆ ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Û˘ÓËÁÔÚÔ‡Û·Ó ˘¤Ú Ù˘ Û˘ÁÁÂÓÔ‡˜ Ì˘˚΋˜ ‰˘ÛÙÚÔÊ›·˜. ŒÙÛÈ, ·ÎÔÏÔ‡ıËÛ ‚ÈÔ„›· Ì˘fi˜ ·fi ÙÔÓ ÙÂÙڷΤʷÏÔ ÁÈ· ÔÏÔÎÏ‹ÚˆÛË Ù˘ ‰È¿ÁÓˆÛ˘. ™ÙË ‚ÈÔ„›· ÙÔ˘ Ì˘fi˜ ·Ú·ÙËÚ‹ıËΠ¤ÓÙÔÓË ›ÓˆÛË Î·È ÏÈ҉˘ ‰È‹ıËÛË ÙÔ˘ ÛÙÚÒÌ·ÙÔ˜. OÈ ›Ó˜ ·ÚÔ˘Û›·˙·Ó ‰È·Î‡Ì·ÓÛË ÙÔ˘ ‡ÚÔ˘˜ ÙÔ˘˜, ·ÔÛÙÚÔÁÁ‡ÏˆÛË ÙÔ˘ ÂÚÈÁÚ¿ÌÌ·Ùfi˜ ÙÔ˘˜, ·ÙÚÔÊ›·, ˘ÂÚÙÚÔÊ›· Î·È ÂÛÙȷ΋ ÂÎʇÏÈÛË (EÈÎfiÓ˜ 2·, 2‚, 2Á Î·È 2‰). ¢È·ÎÚ›ıËÎ·Ó Ï›Á˜
∂ÈÎfiÓ· 1. ª·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘ Ù˘ ¿ÚÚˆÛÙ˘. ·) ∂ÈÎfiÓ· ˘ÔÌ˘ÂϛӈÛ˘ ÂÚÈÎÔÈÏȷο. ‚) OÏÈÁÔÁ˘Ú›·, ·Á˘Ú›·. Á) ∞Û˘ÌÌÂÙÚ›· ÂÁÎÂÊ·ÏÈÎÒÓ ËÌÈÛÊ·ÈÚ›ˆÓ.
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‰È¿Û·ÚÙ˜ ›Ó˜ Ì ‚·ÛÂfiÊÈÏÔ ‹ ÎÔÎÎÈ҉˜ ΢ÙÙ·ÚfiÏ·ÛÌ·. ™Â ¯ÚÒÛÂȘ ÁÈ· ÔÍÂȉˆÙÈο ¤Ó˙˘Ì· ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ·Ú¯ÈÙÂÎÙÔÓÈΤ˜ ‰È·Ù·Ú·¯¤˜ ‹ Â˘Ú‹Ì·Ù· ÂÓ‰ÂÈÎÙÈο ÌÈÙÔ¯ÔÓ‰ÚÈ·ÎÒÓ ·ÏÏÔÈÒÛˆÓ. ™Â ¯ÚÒÛÂȘ ÁÈ· fiÍÈÓË ÊˆÛÊ·Ù¿ÛË Î·È ÌË ÂȉÈ΋ ÂÛÙÂÚ¿ÛË, ·Ú·ÙËÚ‹ıËΠÂÛÙȷ΋ ‰È‹ıËÛË ·fi Ì·ÎÚÔÊ¿Á· Î·È Ï›Á˜ ÌÂÌÔӈ̤Ó˜ ÂÎÊ˘ÏÈ˙fiÌÂÓ˜ ›Ó˜. ¢È·ÎÚ›ıËÎ·Ó ÔÏϤ˜ ‰È¿Û·ÚÙ˜ ›Ó˜, ıÂÙÈΤ˜ ÁÈ· ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË. ¢ÂÓ ·Ú·ÙËÚ‹ıËΠ·‡ÍËÛË Ù˘ ÂÚÈÂÎÙÈÎfiÙËÙ·˜ ÙˆÓ ÈÓÒÓ Û ÁÏ˘ÎÔÁfiÓÔ ‹ ϛԘ Û ¯ÚÒÛÂȘ ƒ∞S Î·È Ì ÌÂÏ·Ófi ÙÔ˘ ™Ô˘‰¿Ó, ·ÓÙ›ÛÙÔȯ·. ™Â ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈΤ˜ ¯ÚÒÛÂȘ ‰ÂÓ ·Ú·ÙËÚ‹ıËΠÂÏ¿ÙÙˆÛË Ù˘ ÌÂÌ‚Ú·ÓÈ΋˜ ¯ÚÒÛ˘ ÙˆÓ ÈÓÒÓ ÁÈ· ‰˘ÛÙÚÔÊ›ÓË, Û·ÚÎÔÁÏ˘Î¿Ó˜ Î·È ‰˘ÛÙÚÔÁÏ˘Î¿ÓË Û ۇÁÎÚÈÛË Ì ̿ÚÙ˘Ú·. ∞ÓÙ›ıÂÙ·, ·Ú·ÙËÚ‹ıËΠϋÚ˘ ¤ÏÏÂÈ„Ë ¯ÚÒÛ˘ ÁÈ· ÌÂÚÔÛ›ÓË (·2 Ï·ÌÈÓ›ÓË). ∆· ÈÛÙÔÏÔÁÈο Î·È ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈο Â˘Ú‹Ì·Ù· Ù˘ ‚ÈÔ„›·˜
Â›Ó·È Û˘Ì‚·Ù¿ ÌÂ Û˘ÁÁÂÓ‹ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· ·fi ¤ÏÏÂÈ„Ë Ï·ÌÈÓ›Ó˘ ·2, ÚˆÙ½Ó˘ Ô˘ Ê˘ÛÈÔÏÔÁÈο ‚Ú›ÛÎÂÙ·È ÛÙË ‚·ÛÈ΋ ÌÂÌ‚Ú¿ÓË ÙˆÓ Ì˘˚ÎÒÓ Î˘ÙÙ¿ÚˆÓ (ÁÚ·Ì̈ÙÔ› ̇˜ Î·È Ì˘ÔοډÈÔ), ÛÙ· ÂÚÈÊÂÚÈο Ó‡ڷ (·ÙÙ·Ú· Schwan), ÛÙ· ·ÈÌÔÊfiÚ· ·ÁÁ›· ÂÁÎÂÊ¿ÏÔ˘ (‚·ÛÈ΋ ÌÂÌ‚Ú¿ÓË), ÛÙÔÓ Ï·ÎÔ‡ÓÙ·, ÛÙÔ ÓÂÊÚfi Î·È ÛÙÔ ‰¤ÚÌ· (16-18). O ÁÔÓȉȷÎfi˜ ¤ÏÂÁ¯Ô˜ ·¤ÎÏÂÈÛ ÙËÓ ÚÔ˚Ô‡Û· ÓˆÙÈ·›· Ì˘˚΋ ‰˘ÛÙÚÔÊ›· (Werdnig-Hoffmann) Î·È ÙË Ì˘˚΋ ‰˘ÛÙÚÔÊ›· Ù‡Ô˘ Duchenne, Ô˘ ·ÔÎÏ›ÛÙËÎÂ Î·È Ì ÙËÓ ·Ó‡ÚÂÛË Ê˘ÛÈÔÏÔÁÈ΋˜ ‰˘ÛÙÚÔÊ›Ó˘. O ÁÔÓȉȷÎfi˜ ¤ÏÂÁ¯Ô˜ Ù˘ Û˘ÁÎÂÎÚÈ̤Ó˘ Û˘ÁÁÂÓÔ‡˜ Ì˘˚΋˜ ‰˘ÛÙÚÔÊ›·˜ ‹Ù·Ó ‰‡ÛÎÔÏÔ ¤ˆ˜ ·‰‡Ó·ÙÔ Ó· Á›ÓÂÈ ÛÙËÓ ∂ÏÏ¿‰·, ·ÏÏ¿ Î·È Û ÂÚ¢ÓËÙÈο ΤÓÙÚ· ÙÔ˘ Â͈ÙÂÚÈÎÔ‡ ÛÙ· ÔÔ›· ·Â˘ı˘Óı‹Î·ÌÂ. ∏ ‰È¿ÁÓˆÛË ÙÂÎÌËÚÈÒıËΠ̠ٷ ·ÔÙÂϤÛÌ·Ù· Ù˘ ÈÛÙÔÏÔÁÈ΋˜ Î·È ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈ΋˜
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∂ÈÎfiÓ· 2. ªÔÚÊÔÏÔÁÈο Î·È ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈο Â˘Ú‹Ì·Ù· ‚ÈÔ„›·˜ ÙÂÙÚ·ÎÂÊ¿ÏÔ˘ Ù˘ ¿ÚÚˆÛÙ˘. ·. ∞ÈÌ·ÙÔÍ˘Ï›ÓË-∏ˆÛ›ÓË (x 350). ¶·Ú·ÙËÚÂ›Ù·È ¤ÓÙÔÓË ›ÓˆÛË ÙÔ˘ ‰È¿ÌÂÛÔ˘ ÛÙÚÒÌ·ÙÔ˜ Î·È Â˘Ú›· ‰È·Î‡Ì·ÓÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ÈÓÒÓ Ì ¤ÓÙÔÓË ·ÙÚÔÊ›· Î·È ˘ÂÚÙÚÔÊ›· ÙˆÓ ÈÓÒÓ. ‚. ∞ÓÔÛÔ˚ÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË ÁÈ· ‰˘ÛÙÚÔÊ›ÓË (x 85) (¡CL-Dys 2). ¶·Ú·ÙËÚÂ›Ù·È Ê˘ÛÈÔÏÔÁÈ΋ ¯ÚÒÛË Î·È ‰È·ÁÚ·Ê‹ ÙÔ˘ ÂÚÈÁÚ¿ÌÌ·ÙÔ˜ ÙˆÓ ÈÓÒÓ Ì ¢Ú›· ‰È·Î‡Ì·ÓÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙÔ˘˜. Á. ∞ÓÔÛÔ˚ÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË ÁÈ· ÌÂÚÔÛ›ÓË (x 350) ‚ÈÔ„›·˜ ·ÛıÂÓÔ‡˜. ¶·Ú·ÙËÚÂ›Ù·È Û¯Â‰fiÓ ·ÓÙÂÏ‹˜ ¤ÏÏÂÈ„Ë ¯ÚÒÛ˘ ÙˆÓ ÈÓÒÓ ÁÈ· ÌÂÚÔÛ›ÓË Û ۇÁÎÚÈÛË Ì ̿ÚÙ˘Ú·. ‰. ∞ÓÔÛÔ˚ÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË ÁÈ· ÌÂÚÔÛ›ÓË ‚ÈÔ„›·˜ Ê˘ÛÈÔÏÔÁÈÎÔ‡ Ì¿ÚÙ˘Ú· (x 350).
510
¶·È‰È·ÙÚÈ΋ 2001;64:508-513
ÌÂϤÙ˘ Ù˘ ‚ÈÔ„›·˜ Ì˘fi˜, fiˆ˜ ·Ó·Ê¤ÚÂÙ·È Î·È Û ·Ó¿ÏÔÁ· ÂÚÈÛÙ·ÙÈο Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ (18). ∆Ô ·È‰› Û˘Ó¯›˙ÂÈ Ó· ·Ú·ÎÔÏÔ˘ıÂ›Ù·È ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜. ™ÙÔ˘˜ ·ÓÂϤÁ¯Ô˘˜, Û ËÏÈΛ· 15, 18 Î·È 27 ÌËÓÒÓ, ÂÌÊ·Ó›˙ÂÈ ‚·ÚÈ¿ ÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ÂÈÎfiÓ· ÁÂÓÈÎÂ˘Ì¤Ó˘ ˘ÔÙÔÓ›·˜ ÌÂ Ì˘˚΋ ·‰˘Ó·Ì›· ¯ˆÚ›˜ η̛· ÂÓÂÚÁËÙÈ΋ ΛÓËÛË, Ï‹ÚË Î·Ù¿ÚÁËÛË ÙˆÓ ÙÂÓfiÓÙÈˆÓ ·ÓÙ·Ó·ÎÏ¿ÛˆÓ, ·Ú·ÎÔÏÔ˘ı› Î·È Ï¤ÂÈ ·ÚÎÂÙ¤˜ ϤÍÂȘ. ÀÔÏ›ÂÙ·È ÛÙËÓ ·Ó¿Ù˘ÍË Ì ‚¿ÚÔ˜ Î·È ‡„Ô˜ οو ·fi ÙËÓ 3Ë ∂£. O ηډÈÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì ËÏÂÎÙÚÔηډÈÔÁÚ¿ÊËÌ· Î·È ˘ÂÚ˯ÔηډÈÔÁÚ¿ÊËÌ·, ÙfiÛÔ Î·Ù¿ ÙË ‰È¿ÁÓˆÛË fiÛÔ Î·È ÛÙÔ˘˜ ·ÓÂϤÁ¯Ô˘˜, ¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ· ·ÚÈÛÙÂÚ¿˜ ÎÔÈÏ›·˜ Î·È Ê˘ÛÈÔÏÔÁÈÎfi ¿¯Ô˜ ÙÔ˘ ÌÂÛÔÎÔÈÏÈ·ÎÔ‡ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ Î·È ÙÔ˘ Ô›ÛıÈÔ˘ ÙÔȯÒÌ·ÙÔ˜ ·ÚÈÛÙÂÚ¿˜ ÎÔÈÏÈ¿˜. ∆Ô ·È‰›, ¤ˆ˜ ÙËÓ ËÏÈΛ· ÙˆÓ 27 ÌËÓÒÓ, ‰ÂÓ ÂÌÊ¿ÓÈÛ ÂÂÈÛfi‰ÈÔ Û·ÛÌÒÓ Î·È ÛÙÔ ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ¿ ÙÔ˘ ηٷÁÚ¿ÊËÎ·Ó ¯·ÌËÏ¿ ‰˘Ó·ÌÈο, ηıÒ˜ Î·È ·ÛÙ¿ıÂÈ· Ú˘ıÌÔ‡ Î·È ‰˘Ó·ÌÈÎÔ‡. ∏ ‰Â‡ÙÂÚË MRI ÂÁÎÂÊ¿ÏÔ˘, ÛÙËÓ ËÏÈΛ· ÙˆÓ 18 ÌËÓÒÓ, ·ÂÈÎÔÓ›˙ÂÈ Ù· ›‰È· ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· Ì ÙËÓ ÚÒÙË, Ì ÈÔ ÂÎÙÂٷ̤ÓË ÂÈÎfiÓ· ‰˘ÛÌ˘ÂϛӈÛ˘. ∆Ô ÂÎ Ó¤Ô˘ ËÏÂÎÙÚÔÌ˘ÔÁÚ¿ÊËÌ· Â›Ó·È Û·ÊÒ˜ Ì˘Ô·ıËÙÈÎÔ‡ Ù‡Ô˘ Ì ηٷÛÙÚÔÊ‹ Ù˘ Ì˘˚΋˜ ›Ó·˜. ™Ù· ۈ̷ÙÔ·ÈÛıËÙÈο ÚÔÎÏËÙ¿ ‰˘Ó·ÌÈο ·Ú·ÙËÚÂ›Ù·È ‰È·ÊÔÚ¿ ÌÂٷ͇ ·ÚÈÛÙÂÚÔ‡ Î·È ‰ÂÍÈÔ‡ ËÌÈÌÔÚ›Ô˘, Ì ۷ʋ ηı˘ÛÙ¤ÚËÛË Î˘Ì·ÙÔÌÔÚÊÒÓ ÛÙÔ ‰ÂÍÈfi ÎÓËÌÈ·›Ô Ì˘. ∆· ·ÎÔ˘ÛÙÈο ‰˘Ó·ÌÈο Â›Ó·È ·ÚÈÛÙÂÚ¿ Ê˘ÛÈÔÏÔÁÈο, ÂÓÒ ‰ÂÍÈ¿ ·Ú·ÙËÚÂ›Ù·È Î·ı˘ÛÙ¤ÚËÛË ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÎÔ¯ÏÈ·ÎÔ‡ ˘Ú‹Ó· Î·È ¿Óˆ ÂÏ·›·˜. ∆· Ì˘˚ο ¤Ó˙˘Ì·, ÛÙÔÓ Â·Ó¤ÏÂÁ¯Ô, ‰ÂÓ Â˘Ú›ÛÎÔÓÙ·È Ï¤ÔÓ ÛÙȘ ›‰È˜ ˘„ËϤ˜ ÙÈ̤˜ (CPK 304 mU/ml, LDH 609 mU/ml), Û ۇÁÎÚÈÛË Ì ÙȘ ÚÒÙ˜, Èı·Ófiٷٷ ÏfiÁˆ Ù˘ ÂÎÙÂٷ̤Ó˘ ÂÎʇÏÈÛ˘, ÏÈÒ‰Ô˘˜ ‰È‹ıËÛ˘ Î·È ·ÙÚÔÊ›·˜ ÙÔ˘ Ì˘˚ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. £Âڷ¢ÙÈο Á›ÓÂÙ·È ˘ÔÛÙËÚÈÎÙÈ΋ Ê˘ÛÈÎÔıÂڷ›· ÁÈ· ÙËÓ ÂÓ›Û¯˘ÛË Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È ·Ú·ÎÔÏÔ˘ıÂ›Ù·È ·fi ÙËÓ ÎÏÈÓÈ΋ Ì·˜. ™˘˙‹ÙËÛË ∏ Û˘ÁÁÂÓ‹˜ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· ÎÏËÚÔÓÔÌÂ›Ù·È Ì ÙÔÓ ˘ÔÏÂÈfiÌÂÓÔ ·˘ÙÔۈ̷ÙÈÎfi ¯·Ú·ÎÙ‹Ú·, ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi Ì˘˚΋ ·‰˘Ó·Ì›· ‹ ˘ÔÙÔÓ›· ·fi ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ Ù˘ ˙ˆ‹˜ Î·È Ë Û˘¯ÓfiÙËÙ¿ Ù˘ ·Ó¤Ú¯ÂÙ·È Û 1/10.000 ÁÂÓÓ‹ÛÂȘ (19). ™ÙÔ 50% ÂÚ›Ô˘ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ˘¿Ú¯ÂÈ Ï‹Ú˘ ¤ÏÏÂÈ„Ë Ù˘ ·2 ·Ï˘Û›‰·˜ Ù˘ Ï·ÌÈÓ›Ó˘ 2 ‹ ÌÂÚÔÛ›Ó˘, ÁÓˆÛÙ‹ ˆ˜ Û˘ÁÁÂÓ‹˜ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· ·fi ¤ÏÏÂÈ„Ë Ï·ÌÈÓ›Ó˘. ∏ Ï·ÌÈÓ›ÓË 2, Ë ÔÔ›· ·Ï·ÈfiÙÂÚ· ÔÓÔÌ·˙fiÙ·Ó Î·È ÌÂÚÔÛ›ÓË ‹ Ï·ÌÈÓ›ÓË-ª, Â›Ó·È ¤Ó· ÂÙÂÚÔÙÚÈÌÂ-
Paediatriki 2001;64:508-513
Ú¤˜, Ô˘ ·ÔÙÂÏÂ›Ù·È ·fi Ì›· ‚·ÚÈ¿ ·Ï˘Û›‰·, ÙËÓ ·2 Î·È ‰‡Ô ÂÏ·ÊÚ¤˜, ÙË ‚1 Î·È Á1. ∆Ô ÌfiÚÈfi Ù˘ ʤÚÂÈ ¤Ó· Ì·ÎÚ‡ Î·È ‰‡Ô ‚Ú·¯¤· ÛΤÏË Î·È Û˘Ó‰¤ÂÙ·È ¤ÌÌÂÛ· Ì ÙËÓ ÚˆÙ½ÓË Ù˘ ‰˘ÛÙÚÔÊ›Ó˘ ‰È·Ì¤ÛÔ˘ Ì›·˜ ÔÌ¿‰·˜ ÁÏ˘ÎÔÚˆÙÂ˚ÓÒÓ (16,19). OÈ ÚˆÙ½Ó˜ ·˘Ù¤˜ ‚Ú›ÛÎÔÓÙ·È ÛÙÔ Û·ÚΛÏËÌ· ÙˆÓ Î˘ÙÙ¿ÚˆÓ Î·È ÛÙ· ÂÁοÚÛÈ· ÛˆÏËÓ¿ÚÈ· ÙÔ˘ ηډȷÎÔ‡ Ì˘fi˜ (3). O ˘Â‡ı˘ÓÔ˜ ÁfiÓÔ˜ Ù˘ Ï·ÌÈÓ›Ó˘ ·2 ÂÓÙÔ›ÛÙËΠÛÙÔ ¯ÚˆÌ·Ùfiۈ̷ 6q22-23 Î·È Ë ¤ÏÏÂÈ„Ë ·˘ÙÔ‡ ‹ ÔÈ ÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ Â›Ó·È ˘Â‡ı˘Ó˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ÓfiÛÔ˘ (19,20). ∞ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó ¤ÏÏÂÈ„Ë Ù˘ ·2 ·Ï˘Û›‰·˜ ·Ó·Ê¤ÚÔÓÙ·È ˆ˜ ¤¯ÔÓÙ˜ ¤ÏÏÂÈ„Ë Ù˘ ÌÂÚÔÛ›Ó˘ ‹ Ï·ÌÈÓ›Ó˘ ·2 ÂÍ·ÈÙ›·˜ Ù˘ ·Ù¤ÏÂÈ·˜ ÙÔ˘ ÂÙÂÚÔÙÚÈÌÂÚÔ‡˜. OÈ ·ÛıÂÓ›˜ ·˘ÙÔ› ¤¯Ô˘Ó ‚·Ú‡ÙÂÚÔ Ê·ÈÓfiÙ˘Ô, Û˘ÁÎÚÈÓfiÌÂÓÔÈ Ì ıÂÙÈÎÔ‡˜ ÁÈ· ÌÂÚÔÛ›ÓË ·ÚÚÒÛÙÔ˘˜ Ì ™ª¢ Î·È Û˘Ó‹ıˆ˜ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ‚·‰›ÛÔ˘Ó ¯ˆÚ›˜ ‚Ô‹ıÂÈ·, ÁÈ·Ù› ¿Û¯Ô˘Ó ÙfiÛÔ ÔÈ ÛÎÂÏÂÙÈÎÔ› ̇˜ fiÛÔ Î·È ÙÔ ÂÚÈÊÂÚÈÎfi Î·È ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· (19,21-23). ™ÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜, Ë ¿ÚÚˆÛÙË ÂÌÊ¿ÓÈÛ ˘ÔÙÔÓ›· Î·È Ì˘˚΋ ·‰˘Ó·Ì›· ·fi ÙË ÓÂÔÁÓÈ΋ ËÏÈΛ· Î·È ‰ÂÓ Â›¯Â „˘¯ÔÓÔËÙÈ΋ ηı˘ÛÙ¤ÚËÛË ‹ ‰È·Ù·Ú·¯¤˜ ·fi Ù· Ì¿ÙÈ·. ∞fi ÙË ‚È‚ÏÈÔÁÚ·Ê›· Â›Ó·È ÁÓˆÛÙfi fiÙÈ Ì ÙËÓ ¿ÚÔ‰Ô Ù˘ ËÏÈΛ·˜ ·Ó·Ù‡ÛÛÂÙ·È ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ Î·È Û ÌÂÁ¿Ï˘ ËÏÈΛ·˜ ·ÚÚÒÛÙÔ˘˜, ‰È·Ù·ÙÈÎÔ‡ Ù‡Ô˘ Ì˘ÔηډÈÔ¿ıÂÈ· Û ÔÛÔÛÙfi 40%, Ì ÂÏ·Ùو̤ÓÔ ÎÏ¿ÛÌ· ÂÍÒıËÛ˘ Û ÔÛÔÛÙfi ÂÚ›Ô˘ 50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (24,25). ¶ÚÔ˜ ÙÔ ·ÚfiÓ, Ë ·ÛıÂÓ‹˜, ÂÍ·ÈÙ›·˜ Ù˘ ËÏÈΛ·˜ Ù˘, ‰ÂÓ ÂÌÊ¿ÓÈÛ Úfi‚ÏËÌ· ·fi ÙËÓ Î·Ú‰È¿. ∆· Ì˘˚ο ¤Ó˙˘Ì· (CPK, LDH, SGOT Î·È SGPT) ‹Ù·Ó Û ˘„ËϤ˜ ÙÈ̤˜, fiˆ˜ ·Ó¢ڛÛÎÔÓÙ·È Û fiÏÔ˘˜ ۯ‰fiÓ ÙÔ˘˜ ¿Û¯ÔÓÙ˜ ·fi ÙË ÓfiÛÔ. ∆Ô ËÏÂÎÙÚÔÌ˘ÔÁÚ¿ÊËÌ· ‹Ù·Ó Ì˘Ô·ıËÙÈÎÔ‡ Ù‡Ô˘, Ù· ۈ̷ÙÔ·ÈÛıËÙÈο ÚÔÎÏËÙ¿ ‰˘Ó·ÌÈο Î·È Ù· ·ÎÔ˘ÛÙÈο ‰˘Ó·ÌÈο ·ıÔÏÔÁÈο fiˆ˜ ·Ó·Ê¤ÚÂÙ·È Î·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·, ηı’ fiÛÔÓ ˘¿Ú¯ÂÈ ÂÏ·Ùو̤ÓË Ù·¯‡ÙËÙ· ·ÁˆÁ‹˜ ÙˆÓ ÂÚÈÊÂÚÈÎÒÓ ÓÂ‡ÚˆÓ (4,19). ∏ ÚÔÛ‚ÔÏ‹ ·˘Ù‹ ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Â›Ó·È Èı·Ófi Ó· Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ¤ÎÊÚ·ÛË Ù˘ ·2 ·Ï˘Û›‰·˜ Ù˘ Ï·ÌÈÓ›Ó˘ ÛÙ· ·ÙÙ·Ú· ÙÔ˘ Schwan (4,19). O ¤ÏÂÁ¯Ô˜ ÙÔ˘ ∫¡™ Ì ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· Û˘Ó‹ıˆ˜ ‰›ÓÂÈ Ê˘ÛÈÔÏÔÁÈο Â˘Ú‹Ì·Ù·, ÂÓÒ Ë ªRI ·ÔηχÙÂÈ ÛÙËÓ ∆2 ·ÎÔÏÔ˘ı›·, ·ÏÏÔÈÒÛÂȘ Ù˘ ÂÚÈÎÔÈÏȷ΋˜ Ï¢΋˜ Ô˘Û›·˜ Ì ˘„ËÏfi Ì·ÁÓËÙÈÎfi Û‹Ì· (15,16,19,26). OÈ ›‰È˜ ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ‚Ï¿‚˜ ÛÙÔ ∫¡™ Ô˘ ·ÂÈÎÔÓ›˙ÔÓÙ·È Ì ÙËÓ MRI ÂÁÎÂÊ¿ÏÔ˘ (·ÏÏÔÈÒÛÂȘ Ù˘ ÂÚÈÎÔÈÏȷ΋˜ Ï¢΋˜ Ô˘Û›·˜ Î·È ˘„ËÏfi Ì·ÁÓËÙÈÎfi Û‹Ì· ÛÙËÓ ∆2 ·ÎÔÏÔ˘ı›·) ·Ú·ÙËÚ‹ıËÎ·Ó Î·È ÛÙË ‰È΋ Ì·˜ ÂÚ›ÙˆÛË. ∏ ‰Â‡ÙÂÚË MRI Ô˘ ¤ÁÈÓ Û ËÏÈΛ· 18 ÌËÓÒÓ ¤‰ÂÈÍ ÂÎÙÂٷ̤ÓË Û˘ÌÌÂÙÚÈ΋ Ï¢ÎÔÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÌÂ Û˘ÌÌÂÙÔ¯‹ ηÈ
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¶·È‰È·ÙÚÈ΋ 2001;64:508-513
ÙˆÓ ‰ÂÌ·Ù›ˆÓ, ¯ˆÚ›˜ Û˘ÌÌÂÙÔ¯‹ Ù˘ ·ÚÂÁÎÂÊ·Ï›‰·˜ Î·È ÙˆÓ ‚·ÛÈÎÒÓ Á·ÁÁÏ›ˆÓ. ∏ ·ÈÙ›· ÚÔÛ‚ÔÏ‹˜ ÙÔ˘ ∫¡™ ‰ÂÓ ¤¯ÂÈ ‰È·Û·ÊËÓÈÛÙ› Ï‹Úˆ˜ (19), ÁÈ·Ù› Ê˘ÛÈÔÏÔÁÈο Ë ¿Ï˘ÛÔ˜ Ù˘ Ï·ÌÈÓ›Ó˘ ·2 ˘¿Ú¯ÂÈ ÛÙ· ·ÈÌÔÊfiÚ· ·ÁÁ›· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È fi¯È ÛÙÔ˘˜ ÔÏÈÁÔ‰ÂÓ‰Ú›Ù˜. ™‡Ìʈӷ Ì ̛· ¿Ô„Ë, ÔÈ ‚Ï¿‚˜ Ù˘ Ï¢΋˜ Ô˘Û›·˜, ÔÈ ÂÌÊ·Ó›˜ Ì ÙËÓ MRI ÂÁÎÂÊ¿ÏÔ˘, ÔÊ›ÏÔÓÙ·È ÛÙË ‰È·Ù·Ú·¯‹ ÙÔ˘ ·ÈÌ·ÙÔÂÁÎÂÊ·ÏÈÎÔ‡ ÊÚ·ÁÌÔ‡ ÏfiÁˆ ¤ÏÏÂȄ˘ Ù˘ Ï·ÌÈÓ›Ó˘ (4,9,27). ¢ÂÓ ˘¿Ú¯ÂÈ Û¯¤ÛË ·Ó¿ÌÂÛ· ÛÙËÓ ¤ÎÙ·ÛË Ù˘ ‚Ï¿‚˘ Ù˘ Ï¢΋˜ Ô˘Û›·˜ Î·È ÙÔ˘ Ê·ÈÓfiÙ˘Ô˘ ‹ Ù˘ ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈ΋˜ ÂÌÊ¿ÓÈÛ˘ ÙˆÓ ‰È·ÊfiÚˆÓ ÎÏ·ÛÌ¿ÙˆÓ Ù˘ Ï·ÌÈÓ›Ó˘ ·2. ªÂϤÙ˜ Û ·ÚÚÒÛÙÔ˘˜ Ì ÂÎÙÂٷ̤Ó˜ ·ÏÏÔÈÒÛÂȘ Ù˘ Ï¢΋˜ Ô˘Û›·˜ ‰Â›¯ÓÔ˘Ó ‹ÈÔ Ê·ÈÓfiÙ˘Ô, Û ·ÓÙ›ıÂÛË Ì ¿ÏÏÔ˘˜, fiÔ˘ ÂÓÒ ˘‹Ú¯·Ó ‹È˜ ‚Ï¿‚˜ ÛÙË Ï¢΋ Ô˘Û›·, ¤¯Ô˘Ó ‚·Ú‡ÙÂÚÔ Ê·ÈÓfiÙ˘Ô ·ÎfiÌË Î·È Ì ÂÌÊ¿ÓÈÛË ÂÂÈÛÔ‰›ˆÓ Û·ÛÌÒÓ (18,19). ∏ ‰È¿ÁÓˆÛË Ù˘ Û˘ÁÁÂÓÔ‡˜ ‰˘ÛÙÚÔÊ›·˜ Ù›ıÂÙ·È ÎÏÈÓÈο Î·È ÂȂ‚·ÈÒÓÂÙ·È Ì ÙË ‚ÈÔ„›· Ì˘fi˜, fiÔ˘ ·Ú·ÙËÚÂ›Ù·È ÏÈ҉˘ ‰È‹ıËÛË, ›ÓˆÛË Î·È ·Ô‰ÈÔÚÁ¿ÓˆÛË ÙˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ, ·ÙÚÔÊ›·, ˘ÂÚÙÚÔÊ›· Î·È ÂÛÙȷ΋ ÂÎʇÏÈÛË ÙÔ˘ Ì˘fi˜ (25). ™Ù· ÈÛÙÔÏÔÁÈο Î·È ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈο ·Ú·Û΢¿ÛÌ·Ù· Ì˘fi˜ ‰È·ÈÛÙÒÓÂÙ·È Ë ¤ÏÏÂÈ„Ë Ù˘ Ï·ÌÈÓ›Ó˘ ·2. ™ÙË ‚ÈÔ„›· Ì˘fi˜ Ù˘ ÂÚ›ÙˆÛ‹˜ Ì·˜ ·Ú·ÙËÚ‹ıËΠϋÚ˘ ¤ÏÏÂÈ„Ë ¯ÚÒÛ˘ ÁÈ· ÌÂÚÔÛ›ÓË (·2 Ï·ÌÈÓ›ÓË). ¢ÂÓ ·Ú·ÙËÚ‹ıËΠÂÏ¿ÙÙˆÛË Ù˘ ÌÂÌ‚Ú·ÓÈ΋˜ ¯ÚÒÛ˘ ÁÈ· ‰˘ÛÙÚÔÊ›ÓË, Û·ÚÎÔÁÏ˘Î¿Ó˜ Î·È ‰˘ÛÙÚÔÁÏ˘Î¿Ó˜, Ô˘ ¯·Ú·ÎÙËÚ›˙Ô˘Ó ¿ÏϘ Ì˘Ô¿ıÂȘ (4,25) (∂ÈÎfiÓ· 2). ™˘ÓÂÒ˜, Ù· Â˘Ú‹Ì·Ù· Ù˘ ‚ÈÔ„›·˜ Â›Ó·È Û˘Ì‚·Ù¿ Ì ™ª¢, ÏfiÁˆ ¤ÏÏÂȄ˘ Ù˘ ÌÂÚÔÛ›Ó˘ ‹ ·2 Ï·ÌÈÓ›Ó˘ (4,18,25). ªÂ ÙÔÓ ÁÔÓȉȷÎfi ¤ÏÂÁ¯Ô ·ÔÎÏ›ÛÙËÎÂ Ë ÓˆÙÈ·›· Ì˘˚΋ ·ÙÚÔÊ›· (Werdnig Hoffmann) Î·È Ë Ì˘˚΋ ‰˘ÛÙÚÔÊ›· Ù‡Ô˘ Duchenne. ªÂÚÈ΋ ¤ÏÏÂÈ„Ë Ù˘ ·2 ·Ï˘Û›‰·˜ Ù˘ Ï·ÌÈÓ›Ó˘ ¤¯ÂÈ ·Ú·ÙËÚËı›: ·) ÛÙÔÓ Ù‡Ô Ù˘ Û˘ÁÁÂÓÔ‡˜ Ì˘˚΋˜ ‰˘ÛÙÚÔÊ›·˜ ÛÙË ÓfiÛÔ “ÔÊı·ÏÌÒÓ Ì˘ÒÓ ÂÁÎÂÊ¿ÏÔ˘” (muscle-eye-brain), fiÔ˘ ˘¿Ú¯Ô˘Ó Ù¤ÛÛÂÚȘ ˘ÔÔÌ¿‰Â˜ Û‡Ìʈӷ Ì ÙÔ ‚·ıÌfi ÚÔÛ‚ÔÏ‹˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È ÙˆÓ ÔÊı·ÏÌÒÓ Î·È ‚) ÛÙË Û˘ÁÁÂÓ‹ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· Ù‡Ô˘ Fukuyama, Ë ÔÔ›· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÓÔËÙÈ΋ ˘ÛÙ¤ÚËÛË Î·È ·ÓˆÌ·Ï›Â˜ ·fi ÙÔÓ ÂÁΤʷÏÔ (19,23). ∏ ı¤ÛË ÙÔ˘ ÁfiÓÔ˘ Û ·˘Ù‹ ÙË ÓfiÛÔ Â›Ó·È ÛÙÔ ¯ÚˆÌfiۈ̷ 9q3133 Î·È Ë ·Ó¿ÚÎÂÈ· Ù˘ Ï·ÌÈÓ›Ó˘ ·2 Â›Ó·È ‰Â˘ÙÂÚÔ·ı‹˜, Û ·ÓÙ›ıÂÛË Ì ÙË ÌÔÚÊ‹ Ù˘ ™ª¢ ÏfiÁˆ ¤ÏÏÂȄ˘ Ï·ÌÈÓ›Ó˘, fiÔ˘ Ë ¤ÏÏÂÈ„Ë ·˘Ù‹ Â›Ó·È ÚˆÙÔ·ı‹˜ (19,20,23). ™ÙȘ ÌÔÚʤ˜ Ì˘˚΋˜ ‰˘ÛÙÚÔÊ›·˜ Ù‡Ô˘ Fukuyama Î·È ªuscle-eye-brain Û˘Ó˘¿Ú¯Ô˘Ó ÂÎÙÂٷ̤Ó˜ ·Ó·ÙÔÌÈΤ˜ ‰È·Ù·Ú·-
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¯¤˜ ÙÔ˘ ∫¡™ (Fukuyama, Muscle-eye-brain) Î·È ÙÔ˘ ÔÊı·ÏÌÔ‡ (Muscle-eye-brain) Î·È Û˘ÓÔ‰fi˜ ÓÔËÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ¯·Ú·ÎÙËÚÈÛÙÈο Ù· ÔÔ›· ‰ÂÓ ¤¯ÂÈ Ë ·ÛıÂÓ‹˜ Ì·˜. ∏ ÂͤÏÈÍË ÙˆÓ ·ÚÚÒÛÙˆÓ ÌÂ Û˘ÁÁÂÓ‹ Ì˘˚΋ ‰˘ÛÙÚÔÊ›· ·fi ¤ÏÏÂÈ„Ë Ï·ÌÈÓ›Ó˘ Â›Ó·È ‚·Ú‡ÙÂÚË. ™˘Ó‹ıˆ˜ ‰ÂÓ Î·ÙÔÚıÒÓÔ˘Ó Ó· ÛÙ·ıÔ‡Ó fiÚıÈÔÈ ‹ Ó· ÂÚ·Ù‹ÛÔ˘Ó, Ô‡Ù ӷ ηı›ÛÔ˘Ó ¯ˆÚ›˜ ‚Ô‹ıÂÈ·. ∏ ‚·ıÌÈ·›· ÂÍÂÏÈÛÛfiÌÂÓË Ì˘ÔηډÈÔ¿ıÂÈ· Î·È ÔÈ Û˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ ·Ó·Ó¢ÛÙÈÎÔ‡ ÂÈ‚·Ú‡ÓÔ˘Ó ‰Ú·Ì·ÙÈο ÙËÓ ÚfiÁÓˆÛ‹ ÙÔ˘˜ (4,19,24). ∏ ıÂڷ¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛ‹ ÙÔ˘˜ Â›Ó·È Û˘Ìو̷ÙÈ΋ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ ÙÔÓ ¤ÏÂÁ¯Ô Ù˘ ηډȿ˜ Û ٷÎÙ¿ ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù·, ÙËÓ ¤ÁηÈÚË ·ÓÙÈÌÂÙÒÈÛË ÏÔÈÌÒÍÂˆÓ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Î·È ÙËÓ ˘ÔÛÙËÚÈÎÙÈ΋ Ê˘ÛÈÎÔıÂڷ›· (4,25). ∏ ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ Á›ÓÂÙ·È Ì ÙËÓ ·Ó·˙‹ÙËÛË Ù˘ Ï·ÌÈÓ›Ó˘ ·2 ÛÙÔ˘˜ ÛÎÂÏÂÙÈÎÔ‡˜ Ì˘˜ Î·È ÛÙÔ ‰¤ÚÌ· ÌÂÙ¿ ·fi ‚ÈÔ„›· Î·È Ì ÁÔÓȉȷÎfi ¤ÏÂÁ¯Ô, fiÔ˘ ·ÔηχÙÂÙ·È ¤ÏÏÂÈ„Ë ‹ ÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ˘Â‡ı˘ÓÔ˘ ÁfiÓÔ˘ (18-21). ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË ÂӉ›ÎÓ˘Ù·È Û ΢‹ÛÂȘ Ì ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi Î·È Á›ÓÂÙ·È Ì ÙË ‚ÈÔ„›· ¯ÔÚÈ·ÎÒÓ Ï·¯ÓÒÓ ÙÔ˘ Ï·ÎÔ‡ÓÙ· (12Ë Â‚‰ÔÌ¿‰· ·ËÛ˘), fiÔ˘ ·ÓȯÓ‡ÂÙ·È Ë ·Ï˘Û›‰· Ù˘ ·2 Ï·ÌÈÓ›Ó˘, ›Ù Ì ÙË ¯Ú‹ÛË ÂȉÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ù˘ Ï·ÌÈÓ›Ó˘ ·2, ›Ù Ì ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈΤ˜ ¯ÚÒÛÂȘ ‰ÂÈÁÌ¿ÙˆÓ ¯ÔÚÈ·ÎÒÓ Ï·¯ÓÒÓ (18,25,28). ™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÂÓ›˜ Ì˘˚Τ˜ ‰˘ÛÙÚÔʛ˜ Â›Ó·È Ì›· ÔÌ¿‰· ÓÔÛËÌ¿ÙˆÓ Ì ÚÔ˚Ô‡Û· ÂͤÏÈÍË Î·È ¯ˆÚ›˜ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·ÈÙÈÔÏÔÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÚÔ˜ ÙÔ ·ÚfiÓ. ∏ ‰È¿ÁÓˆÛ‹ ÙÔ˘˜, fï˜, ¤¯ÂÈ ÌÂÁ¿ÏË ÛËÌ·Û›·, ÙfiÛÔ ÁÈ· ÙÔÓ ·ÔÎÏÂÈÛÌfi ¿ÏÏˆÓ ÓÔÛËÌ¿ÙˆÓ fiÛÔ Î·È ÁÈ· ÙË ÁÂÓÂÙÈ΋ ηıÔ‰‹ÁËÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ ÙˆÓ ·Û¯fiÓÙˆÓ. µÈ‚ÏÈÔÁÚ·Ê›· 1. Brooke MH, ed. A clinician’s view of neuromuscular diseases. 2nd ed. Baltimore: Williams & Wilkins; 1986. p. 345-354. 2. Banker BQ. The congenital muscular dystrophies. In: Engel AG, Franzini-Armstrong C, eds. Myology. 2nd ed. New York: Mc Graw Hill; 1994. p. 1275-1289. 3. Dubowitz V. Muscle disorders in childhood. 2nd ed. WB Saunders: London; 1995. p. 93-105. 4. Darras TB. Neuromuscular disorders in the hypotonic infant. In: Panteliadis PC, editor. Encyclopedia of Paediatric Neurology. 2nd ed. Thessaloniki: Giahoudi Giapouli; 1999. p. 752-773. 5. Fukuyama Y, Haruna H, Kawazura M. A peculiar form of congenital progressive muscular dystrophy. Pediatr Univ Tokyo 1960;4:4-5.
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6. Fowler MC, Manson JL. Congenital muscular dystrophy with malformation of the central nervous system. In: Kakulas BA, editor. Clinical studies in myology. Amsterdam: Elsevier; 1973. p. 192-197. 7. Santavuori P, Leisti J, Kruus S. Muscle eye, brain disease: A new syndrome. Neuropädiatrie 1977;8:553-558. 8. Dobyns WB, Pagon RA, Armstrong D, Curry CJR, Greenberg F, Grix A et al. Diagnostic criteria for WalkerWarburg Syndrome. Am J Med Genet 1989;32:195-210. 9. Tome FMS, Evangelista T, Leclerc A, Sunada Y, Mande E, Esturnet B et al. Congenital muscular dystrophy with merosin deficiency CR. Acad Sci Paris 1994;317:351-357. 10. Helbling-Leclerc A, Zhang X, Topaloglu H, Gruand C, Tesson F, Weissenbach J et al. Mutations in the laminin a2chain gene (LAMA-2) cause merosin-deficient congenital muscular dystrophy. Nat Genet 1995;11:216-218. 11. Kobayashi K, Nakahori Y, Miyake M, Matsumura K, Kandolida E, Mormura Y et al. An ancient retrotransposal insertion causes Fukuyama type 6 congenital muscular dystrophy. Nature 1998;394:388-392. 12. Toba T, Kobayashi K, Kondo-lida E, Sasaki J, Nakamura Y. The fukuyama congenital muscular dystrophy story. Neuromuscul Disord 2000;10:153-159. 13. Cormand B, Avela K, Pihko H, Santavuori P, Talim P, Topaloglu H et al. Assignment of the muscle-eye-brain disease gene to 1p32-34 by linkage analysis and homozygosity mapping. Am J Hum Genet 1999;64:126-135. 14. Moghadaszadeh B, Desguerre I, Topaloglu H, Muntoni F, Pavek S, Sewrg C et al. Identification of a new locus for a peculiar form of congenital muscular dystrophy with early rigidity of the spine on chromosome 1p35-36. Am J Hum Genet 1998;62:1439-1445. 15. Flannigan KM, Kerr L, Bromberg MB, Leonard C, Tsuruda J, Zhang P et al. Congenital muscular dystrophy with rigid spine syndrome: a clinical, pathological, radiological and genetic study. Ann Neurol 2000;47:147-161. 16. Dubowitz V. Congenital muscular dystrophy: An exparding clinical syndrome. Annals Neurol 2000;47:143-144. 17. Engrail E. Laminin variants: why where and when? Kidney Internat 1993;43:2-6. 18. Sewry CA, Philpot J, Sorokin LM, Wilson LA, Naom L, Goodwin F et al. Diagnosis of merosin (Laminin 2) deficient congenital muscular dystrophy by skin biopsy. Lancet 1996;347:582-584. 19. Cohn RD, Herrmann R, Sorokin L, Wener MU, Volt T. Laminin a2 chain-deficient congenital muscular epitage expression in severe and mild cases. Neurology 1998;51:94-100. 20. Hillaire D, Leclerc A, Faure S, Topaloglou H,
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Chiannilkulchai N, Guicheney P et al. Localisation of merosin-negative congenital muscular dystrophy to chromosome 6q22 by homozygosity mapping. Hum Mol Genet 1994;3:1657-1661. Dubowitz V. The muscular dystrophies. Postgrad Med J 1992;68:500-506. Burgeson RE, Chiquet ª, Deutzmann R, Ekblome P, Engel J, Kleinman H et al. A new nomenclature for the laminins. Matrix Biol 1994;14:209-211. Toda T, Segawa ª, Nomura À, Nonaka I, Masuda K, Ishihara T et al. Localisation of a gene for Fukuyama type congenital muscular dystrophy to chromosome 9q31-33. Nat Genet 1993;5:283-286. Spyrou ¡, Philpot J, Foale R, Camici ƒ, Muntoni F. Evidence of left ventricular dysfunction in children with merosin-deficient congenital muscular dystrophy. Am Heart J 1998;136:474-476. Klietsch R, Ervasti JM, Arnold W, Campobell KP, Jorgensen AO. Dystrophin-glycoprotein complex and laminin localize to the sarcolemma and transverse tubules of cardiac muscle. Circ Res 1993;72:349-360. Tsao CY, Mendell JR, Rusin J, Laquette M. Congenital muscular dystrophy with complete laminin-alpha 2deficiency cortical dysplasia and cerebral white matter changes in children. J Child Neurol 1988;13:253-256. Caro PA, Scavina M, Hoffman E, Pergoraro E, Marks HG. MR imaging findings in children with merosin deficient congenital muscular dystrophy. Am J Neuroradiol 1999;20:324-326. Muntoni F, Sewry C, Wilson L, Angelini C, Trevisan CP, Brabati B et al. Prenatal diagnosis in congenital muscular dystrophy [Letter]. Lancet 1995;345:591.
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CASE REPORT
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Crossed renal ectopia with fusion and hydronephrosis in a girl with congenital adrenal hyperplasia D. Karakaidos, P. Zosi, M. Karafezi, A. Moutafi, G. Kaniaris, C. Karis
¶ÂÚ›ÏË„Ë: ¶ÂÚÈÁÚ¿ÊÂÙ·È ÂÚ›ÙˆÛË ÎÔÚÈÙÛÈÔ‡ 17 ÂÙÒÓ Ì ηÙÒÙÂÚË ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ÓÂÊÚÈ΋ ÂÎÙÔ›· ÌÂ Û˘ÁÎfiÏÏËÛË. ∏ ‰È¿ÁÓˆÛË ·ÔÙ¤ÏÂÛÂ Ù˘¯·›Ô ˘ÂÚ˯ÔÁÚ·ÊÈÎfi ‡ÚËÌ· ÛÙ· Ï·›ÛÈ· ÂϤÁ¯Ô˘ ÁÈ· ˘ÂÚ·Ó‰ÚÔÁÔÓ·ÈÌ›· Ô˘ ·Â‰Â›¯ıË fiÙÈ ÔÊÂÈÏfiÙ·Ó ÛÂ Û˘ÁÁÂÓ‹ ˘ÂÚÏ·Û›· ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ. O ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍ ÙËÓ ·ÚÔ˘Û›· ˘‰ÚÔÓ¤ÊÚˆÛ˘ Î·È ÙˆÓ ‰‡Ô ÓÂÊÚÒÓ Ì ÌÂȈ̤ÓË ·ÚÔ¯¤Ù¢ÛË Ô‡ÚˆÓ, ȉ›ˆ˜ ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi, ηıÒ˜ Î·È ÂÚÈÔ¯¤˜ ÂÏϷو̤Ó˘ ÚfiÛÏ˄˘ Ú·‰ÈÔÊ·ÚÌ¿ÎÔ˘ ÛÙÔ ÛÙ·ÙÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· Î·È ÛÙÔ˘˜ ‰‡Ô ÓÂÊÚÔ‡˜. ∞Ó·ÛÎÔÂ›Ù·È Ë ‚È‚ÏÈÔÁÚ·Ê›· ˆ˜ ÚÔ˜ ÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ· ·˘Ù‹˜ Ù˘ Û¿ÓÈ·˜ Û˘ÁÁÂÓÔ‡˜ ·ÓˆÌ·Ï›·˜, ηıÒ˜ Î·È ÙËÓ ÂȉËÌÈÔÏÔÁ›·, ÙËÓ ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ÚÔ‚ÔÏ‹ Î·È ÙËÓ ÚfiÁÓˆÛË.
Abstract: A case of a 17 year old girl with crossed renal ectopia with fusion is described. The diagnosis was based on an incidental ultrasonographic finding during the course of a thorough investigation for hyperadrogenemia, which ultimately proved to be due to congenital adrenal hyperplasia. Imaging studies confirmed the presence of hydronephrosis of both kidneys with decreased urinary drainage which was more prominent on the ectopic kidney. DMSA revealed segments of reduced uptake of the radioisotope in both kidneys. The etiology of this rare condition as well as the clinical presentation, diagnosis and prognosis are reviewed.
§¤ÍÂȘ ÎÏÂȉȿ: ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ÓÂÊÚÈ΋ ÂÎÙÔ›·, ˘‰ÚÔÓ¤ÊÚˆÛË, Û˘ÁÁÂÓ‹˜ ˘ÂÚÏ·Û›· ÂÈÓÂÊÚȉ›ˆÓ.
Key words: crossed renal ectopia, hydronephrosis of both kidneys, congenital adrenal hyperplasia.
∂ÈÛ·ÁˆÁ‹ ŒÎÙÔÔ˜ ÔÓÔÌ¿˙ÂÙ·È o ÓÂÊÚfi˜ Ô˘ ηٿ ÙËÓ ÂÌ‚Ú˘˚΋ ˙ˆ‹ ‰È·ÎfiÙÂÙ·È Ë ¿ÓÔ‰fi˜ ÙÔ˘ ÚÔ˜ ÙË Ê˘ÛÈÔÏÔÁÈ΋ ÙÔ˘ ı¤ÛË, Ë ÔÔ›· Â›Ó·È ·Ú·ÛÔÓ‰˘ÏÈο ÛÙÔ ‡„Ô˜ ÙÔ˘ £12 ¤ˆ˜ O3 ÛÔÓ‰‡ÏÔ˘ Î·È ·Ú·Ì¤ÓÂÈ Û ·ÓÒÌ·ÏË ı¤ÛË (1). ∏ ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ÓÂÊÚÈ΋ ÂÎÙÔ›· (¢¡∂) Â›Ó·È Ì›· Û¿ÓÈ· Û˘ÁÁÂÓ‹˜ ·ÓˆÌ·Ï›·, ÛÙËÓ ÔÔ›· Ô ¤ÎÙÔÔ˜ ÓÂÊÚfi˜
‚Ú›ÛÎÂÙ·È ÛÙÔ ·ÓÙ›ıÂÙÔ ËÌÈÌfiÚÈÔ ÙÔ˘ ÛÒÌ·ÙÔ˜, ÂÓÒ Ô Ô˘ÚËÙ‹Ú·˜ ÙÔ˘ ¯·Ú·ÎÙËÚÈÛÙÈο ‰È·ÛÙ·˘ÚÒÓÂÙ·È Ì ÙË Ì¤ÛË ÁÚ·ÌÌ‹ Î·È ÂÈÛ¤Ú¯ÂÙ·È Û ÔÚıfiÙÔË ı¤ÛË ÛÙËÓ Ô˘ÚÔ‰fi¯Ô ·ÛÙË (1-4). OÈ ªc Donald Î·È Mc Clellan Î·Ù¤Ù·Í·Ó ÙË ¢¡∂ ÛÙȘ ·ÎfiÏÔ˘ı˜ ÌÔÚʤ˜ (1,2,5): 1) ¢È·ÛÙ·˘ÚÔ‡ÌÂÓË ÂÎÙÔ›· ÌÂ Û˘ÁÎfiÏÏËÛË (¢¡∂ª™), Ô˘ Â›Ó·È Ë Û˘¯ÓfiÙÂÚË ÌÔÚÊ‹ Ì ÔÛÔÛÙfi 90%, 2) ¢È·ÛÙ·˘ÚÔ‡ÌÂÓË ÂÎÙÔ›· ¯ˆÚ›˜ Û˘ÁÎfiÏÏËÛË, Ô˘ ·ÎÔÏÔ˘ı› ÛÂ Û˘¯ÓfiÙËÙ·, 3) ªÔÓ‹Ú˘ ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ÂÎÙÔ›· Î·È 4) ∞ÌÊÔÙÂÚfiÏ¢ÚË ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ÂÎÙÔ›·, Ô˘ Â›Ó·È Ë Û·ÓÈfiÙÂÚË ÌÔÚÊ‹.
¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¶ÂÚÈÊÂÚÂÈ·Îfi °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¡›Î·È·˜-¶ÂÈÚ·È¿ “ÕÁÈÔ˜ ¶·ÓÙÂÏ‹̈Ӕ
Department of Pediatrics General Hospital of Nikea-Piraeus “St. Panteleimon”, Athens
™˘ÓÙÔÌÔÁڷʛ˜ ¢¡∂ ¢È·ÛÙ·˘ÚÔ‡ÌÂÓË ÓÂÊÚÈ΋ ÂÎÙÔ›· ¢¡∂ª™ ¢È·ÛÙ·˘ÚÔ‡ÌÂÓË ÓÂÊÚÈ΋ ÂÎÙÔ›· ÌÂ Û˘ÁÎfiÏÏËÛË
514
¶·È‰È·ÙÚÈ΋ 2001;64:514-517
∏ ¢¡∂ª™ Ì ÙË ÛÂÈÚ¿ Ù˘ ‰È·ÎÚ›ÓÂÙ·È ÛÙȘ ·Ú·Î¿Ùˆ ÌÔÚʤ˜ (1-3): ·) ∫·ÙÒÙÂÚË ÂÎÙÔ›·, fiÙ·Ó Ô Î¿Ùˆ fiÏÔ˜ ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ÓÂÊÚÔ‡ ÂÓÒÓÂÙ·È Ì ÙÔÓ ¿Óˆ fiÏÔ ÙÔ˘ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡ (ÂÚ›Ô˘ ÙÔ 60%), ‚) ™ÈÁÌÔÂȉ‹˜ ÂÎÙÔ›·, Á) ™¯‹Ì·ÙÔ˜ -L-ÂÎÙÔ›·, fiÙ·Ó Ô ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ Â›Ó·È Î¿ıÂÙÔ˜, ‰) ¡ÂÊÚfi˜ Û¯‹Ì·ÙÔ˜ ‚ÒÏÔ˘ (Lump-kindey), fiÙ·Ó ¤¯Ô˘Ì ÂÎÙÂٷ̤ÓË ¤ÓˆÛË ÙˆÓ ‰‡Ô ÓÂÊÚÒÓ, Â) ¢ÈÛÎÔÂȉ‹˜ ÓÂÊÚfi˜, fiÙ·Ó ¤¯Ô˘Ì ‰È·ÊfiÚÔ˘ ‚·ıÌÔ‡ ¤ÓˆÛË ÛÙȘ ¤Ûˆ ÂÈÊ¿ÓÂȘ ÙˆÓ ÓÂÊÚÒÓ Î·È ÛÙ) ∞ÓÒÙÂÚË ÂÎÙÔ›·, fiÙ·Ó Ô ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ ‚Ú›ÛÎÂÙ·È Û ˘„ËÏfiÙÂÚË ·fi ÙÔÓ ÔÚıfiÙÔÔ, Ô˘ Â›Ó·È Î·È Ë Û·ÓÈfiÙÂÚË ÌÔÚÊ‹. ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ∫ÔÚ›ÙÛÈ ËÏÈΛ·˜ 16 ÂÙÒÓ ·Ú·¤ÌÊıËΠÛÙÔ ∂Ó‰ÔÎÚÈÓÔÏÔÁÈÎfi π·ÙÚÂ›Ô Ù˘ ÎÏÈÓÈ΋˜ Ì·˜ ÏfiÁˆ ˘ÂÚ·Ó‰ÚÔÁÔÓ·ÈÌ›·˜, Ô˘ ‰È·ÈÛÙÒıËΠ۠¤ÏÂÁ¯Ô ÁÈ· ˘ÂÚÙÚ›¯ˆÛË ¿ÎÚˆÓ Î·È ·Ú·›ˆÛË ÙÚ›¯ˆÛ˘ ÎÂÊ·Ï‹˜. ∞fi ÙÔ ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi ‰ÂÓ ·Ó·Ê¤ÚıËÎ·Ó Ô˘ÚÔÏÔÈÌÒÍÂȘ, fï˜ ÛËÌÂÈÒÓÂÙ·È Ë ·ÚÔ˘Û›· ·ÚÎÂÙÒÓ ÂÌ˘Ú¤ÙˆÓ Ù· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜ Ô˘ ·Ô‰fiıËÎ·Ó Û ÏÔÈÌÒÍÂȘ ·Ó·Ó¢ÛÙÈÎÔ‡. ∞fi ÙËÓ ËÏÈΛ· ÙˆÓ 14 ÂÙÒÓ ÙÔ ·È‰› ¿Ú¯ÈÛ ӷ ·ÚÔ˘ÛÈ¿˙ÂÈ ÎÚ›ÛÂȘ Ù‡Ô˘ ·Ê·ÈÚ¤Ûˆ˜ ÌÂ Û˘ÓÔ‰¤˜ ·ÎÔ˘ÛÙÈΤ˜ „¢‰·ÈÛı‹ÛÂȘ, Ô˘ ·ÓÙ·ÔÎÚ›ıËÎ·Ó ÈηÓÔÔÈËÙÈο ÛÙËÓ ·ÁˆÁ‹ Ì Ô͢ηڂ·Ì·˙›ÓË. ™ÙÔÓ ¤ÏÂÁ¯Ô ÙˆÓ ÂÈÏËÙÈÎÒÓ ÎÚ›ÛÂˆÓ ‰È·ÈÛÙÒıËΠÌÈÎÚfi ÊÏ‚҉˜ ·ÁÁ›ˆÌ· ÛÙÔ ‰ÂÍÈfi ÌÂوȷ›Ô ÏÔ‚fi Ô˘ ‰ÂÓ Û˘Û¯ÂÙ›ÛÙËΠ̠ÙȘ ÎÚ›ÛÂȘ. ∫·Ù¿ ÙËÓ Â›ÛÔ‰Ô ÛÙËÓ ÎÏÈÓÈ΋, Ë ·ÛıÂÓ‹˜ ·-
∂ÈÎfiÓ· 1. I.V ˘ÂÏÔÁÚ·Ê›·: Ë ‡ÂÏÔ˜ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ÓÂÊÚÔ‡ ÂÏ·ÊÚ¿ ‰È·ÙÂٷ̤ÓË ‰È·ÎÚ›ÓÂÙ·È Û ‰ÂÍÈ¿ ÂÓ‰Ô˘ÂÏÈ΋ ı¤ÛË, ÂÓÒ Ô Û‡ÛÙÔȯԘ Ô˘ÚËÙ‹Ú·˜ ÂÈÛ¤Ú¯ÂÙ·È Û ÔÚıfiÙÔË ı¤ÛË ÛÙËÓ Ô˘ÚÔ‰fi¯Ô ·ÛÙË. ∏ ‡ÂÏÔ˜ ÙÔ˘ ‰ÂÍÈÔ‡ ÓÂÊÚÔ‡ Û ÔÚıfiÙÔË ı¤ÛË Ì ‰È¿Ù·ÛË Ô˘ Èı·ÓÒ˜ ˘ÂÚÂÎÙÈÌ¿Ù·È ÏfiÁˆ Ù˘ ¤Íˆ ÛÙÚÔÊ‹˜ ÙÔ˘ Û‡ÛÙÔÈ¯Ô˘ ÓÂÊÚÔ‡.
Paediatriki 2001;64:514-517
ÚÔ˘Û›·˙ ·Ú·›ˆÛË ÙÚ›¯ˆÛ˘ ÎÂÊ·Ï‹˜, ȉ›ˆ˜ ÛÙË ÌÂوȷ›· ¯ÒÚ·, ˘ÂÚÙÚ›¯ˆÛË ¿ÎÚˆÓ, Ê˘ÛÈÔÏÔÁÈο ¤Íˆ ÁÂÓÓËÙÈο fiÚÁ·Ó· ı‹ÏÂÔ˜ Î·È Ô ‚·ıÌfi˜ ÂÓ‹‚ˆÛ˘ ‹Ù·Ó ÛÙÔ 5Ô ÛÙ¿‰ÈÔ Î·Ù¿ Tanner. ∞fi Ù· ˘fiÏÔÈ· Û˘ÛÙ‹Ì·Ù·, Ë ÂͤٷÛË ‹Ù·Ó ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. O ÔÚÌÔÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍ ·˘ÍË̤ӷ ›‰· ·Ó‰ÚÔÁfiÓˆÓ (ÙÂÛÙÔÛÙÂÚfiÓ˘, ‰È¸‰ÚÔÂÈ·Ó‰ÚÔÛÙÂÚfiÓ˘, ·Ó‰ÚÔÛÙÂÓ‰ÈfiÓ˘, 17 O∏-ÚÔÁÂÛÙÂÚfiÓ˘) Ì ·‡ÍËÛË ÙfiÛÔ ÙˆÓ ‚·ÛÈÎÒÓ ÙÈÌÒÓ, fiÛÔ Î·È ÙˆÓ ÙÈÌÒÓ ÌÂÙ¿ ·fi ‰È¤ÁÂÚÛË Ì ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ACTH. OÈ ˘fiÏÔȘ ÔÚÌfiÓ˜ Ô˘ ÂÍÂÙ¿ÛÙËÎ·Ó Â›¯·Ó Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜. ∏ ˘ÂÚ·Ó‰ÚÔÁÔÓ·ÈÌ›· ·Ô‰fiıËΠ۠ÌË ÎÏ·ÛÈ΋ ÌÔÚÊ‹ Û˘ÁÁÂÓÔ‡˜ ˘ÂÚÏ·Û›·˜ ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ Î·È Ë ·ÛıÂÓ‹˜ ÂÙ¤ıË Û ·ÁˆÁ‹ Ì ˘‰ÚÔÎÔÚÙÈ˙fiÓË (20 mg/m2/24h). ™Ù· Ï·›ÛÈ· ÙÔ˘ ÂϤÁ¯Ô˘ ÙˆÓ ¤Ûˆ ÁÂÓÓËÙÈÎÒÓ ÔÚÁ¿ÓˆÓ ¤ÁÈÓ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÎÔÈÏ›·˜ Ô˘ ¤‰ÂÈÍ ÙÔÓ ·ÚÈÛÙÂÚfi ÓÂÊÚfi Ó· ‚Ú›ÛÎÂÙ·È Û ¤ÎÙÔË ı¤ÛË ‰ÂÍÈ¿ ÂÓ‰Ô˘ÂÏÈο Î·È Èı·ÓfiÓ Ô ¿Óˆ fiÏÔ˜ ÙÔ˘ Ó· Û˘Ó‰¤ÂÙ·È Ì ÙÔÓ Î¿Ùˆ fiÏÔ ÙÔ˘ ‰ÂÍÈÔ‡ ÓÂÊÚÔ‡, Ô ÔÔ›Ô˜ ·ÚÔ˘Û›·˙ ¤Íˆ ÛÙÚÔÊ‹. ∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ‰ÂÓ ·ÂÎ¿Ï˘„ ·ıÔÏÔÁ›· ·fi Ù· ÂÈÓÂÊÚ›‰È·, ÂȂ‚·›ˆÛ fï˜ Ù· Â˘Ú‹Ì·Ù· ÙÔ˘ ˘ÂÚ˯ÔÁÚ·ÊÈÎÔ‡ ÂϤÁ¯Ô˘. ∞ÎÔÏÔ‡ıˆ˜, ¤ÁÈÓ ÂÓ‰ÔÊϤ‚È· ˘ÂÏÔÁÚ·Ê›·, ÛÙËÓ ÔÔ›· Ë ‡ÂÏÔ˜ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡ ÂÌÊ·ÓÈ˙fiÙ·Ó ÂÏ·ÊÚÒ˜ ‰È·ÙÂٷ̤ÓË Û ‰ÂÍÈ¿ ÂÓ‰Ô˘ÂÏÈ΋ ı¤ÛË, ÂÓÒ Ô Û‡ÛÙÔȯԘ Ô˘ÚËÙ‹Ú·˜ ÂÈÛÂÚ¯fiÙ·Ó Û ÔÚıfiÙÔË ı¤ÛË ÛÙËÓ Ô˘ÚÔ‰fi¯Ô ·ÛÙË. ∏ ‡ÂÏÔ˜
∂ÈÎfiÓ· 2. ¢˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· DTPA: ÈηÓfiÙËÙ· ·ÚÔ¯¤Ù¢Û˘ ÂËÚ·Ṳ̂ÓË Î·È ÛÙÔ˘˜ ‰‡Ô ÓÂÊÚÔ‡˜, ȉȷ›ÙÂÚ· fï˜ ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi, ÛÙÔÓ ÔÔ›Ô ‰ÂÓ ˘¿Ú¯ÂÈ ·ÓÙ·fiÎÚÈÛË ÛÙË ¯ÔÚ‹ÁËÛË ÊÔ˘ÚÔÛÂÌ›‰Ë˜.
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Î·È ÔÈ Î¿Ï˘Î˜ ÙÔ˘ ‰ÂÍÈÔ‡ ÓÂÊÚÔ‡ Û ÔÚıfiÙÔË ı¤ÛË ·ÚÔ˘Û›·˙·Ó ‰È¿Ù·ÛË, Ë ÔÔ›· Èı·ÓÒ˜ Ó· ˘ÂÚÂÎÙÈÌ‹ıËΠÏfiÁˆ Ù˘ ¤Íˆ ÛÙÚÔÊ‹˜ ÙÔ˘ Û‡ÛÙÔÈ¯Ô˘ ÓÂÊÚÔ‡ (∂ÈÎfiÓ· 1). ∂ÈϤÔÓ, ·Ú·ÙËÚ‹ıËÎÂ Ë ·ÚÔ˘Û›· ÛÎÈ·ÁÚ·ÊÈÎÔ‡ Û ·ÌÊfiÙÂÚ˜ ÙȘ ˘¤ÏÔ˘˜ ÛÙȘ ·Ú·ÙÂٷ̤Ó˜ Ï‹„ÂȘ. O ¤ÏÂÁ¯Ô˜ Û˘Ó¯›ÛÙËΠ̠‰˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ (DTPA) Ô˘ ¤‰ÂÈÍ ÂËÚ·Ṳ̂ÓË ·Ô¯ÂÙ¢ÙÈ΋ ÈηÓfiÙËÙ· Î·È ÛÙÔ˘˜ ‰‡Ô ÓÂÊÚÔ‡˜, ȉȷ›ÙÂÚ· fï˜ ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi, Ô ÔÔ›Ô˜ ‰ÂÓ ·ÓÙ·ÔÎÚ›ıËΠÛÙË ¯ÔÚ‹ÁËÛË ÊÔ˘ÚÔÛÂÌ›‰Ë˜ (∂ÈÎfiÓ· 2). ∆Ô ÛÙ·ÙÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ (DMSA) ·ÔÎ¿Ï˘„ ÂÚÈÔ¯¤˜ ÂÏϷو̤Ó˘ ÚfiÛÏ˄˘ Ú·‰ÈÔÊ·ÚÌ¿ÎÔ˘ Î·È ÛÙÔ˘˜ ‰‡Ô ÓÂÊÚÔ‡˜ (∂ÈÎfiÓ· 3) Ì ۯÂÙÈ΋ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ· 62% ÛÙÔÓ ‰ÂÍÈfi ÓÂÊÚfi Î·È 38% ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi, Î·È Ì ‰È·ÛÙ¿ÛÂȘ 115 mm ÛÙÔÓ ‰ÂÍÈfi ÓÂÊÚfi Î·È 129 mm ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi. ∏ Ú·‰ÈÔ˚ÛÔÙÔÈ΋ ΢ÛÙÂÔÔ˘ÚËıÚÔÁÚ·Ê›· ‹Ù·Ó ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. O ˘fiÏÔÈÔ˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜, fiˆ˜ Î·È Ë ·ÚÙËÚȷ΋ ›ÂÛË, ‹Ù·Ó ÂÓÙfi˜ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ (Ô˘Ú›· 24 mg%, ÎÚ·ÙÈÓ›ÓË 0,6 mg%, χΈ̷ Ô‡ÚˆÓ 24ÒÚÔ˘ ·ÚÓËÙÈÎfi, οı·ÚÛË ÎÚ·ÙÈÓ›Ó˘ 148 ml/min/1,73 mÇ). ∞ÎÔÏÔ‡ıˆ˜, ¤ÁÈÓ ԢÚÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË Î·È Û˘ÛÙ‹ıËΠٷÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË ÁÈ· Ù˘¯fiÓ ÂÌÊ¿ÓÈÛË ÂÈÏÔÎÒÓ (ÏÈı›·ÛË, Ïԛ̈ÍË) Î·È Â·ÓÂÎÙ›ÌËÛË ·Ô¯ÂÙ¢ÙÈ΋˜ ÈηÓfiÙËÙ·˜ ÓÂÊÚÒÓ ÌÂ Ó¤Ô ‰˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· Û 6 Ì‹Ó˜. ™˘˙‹ÙËÛË ∏ Û˘¯ÓfiÙËÙ· Ù˘ ¢¡∂ª™ Û ÓÂÎÚÔÙÔÌÈÎfi ˘ÏÈÎfi ÔÈΛÏÏÂÈ ·fi 1/1000 ¤ˆ˜ 1/7000 (1-3,5). ¶·Ú·ÙËÚÂ›Ù·È Ì›· ˘ÂÚÔ¯‹ Ù˘ Û˘¯ÓfiÙËÙ·˜ ÛÙÔ˘˜ ¿Ó‰Ú˜ (2/1 ¤ˆ˜ 3/2), ÂÓÒ Â›Ó·È 3 ÊÔÚ¤˜ Û˘¯ÓfiÙÂÚË Ë ÌÂÙ·Ó¿ÛÙ¢ÛË ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ ÓÂÊÚÔ‡ ÛÙË ‰ÂÍÈ¿ ÏÂ˘Ú¿ (1,4) fiˆ˜ Û˘Ì‚·›ÓÂÈ Î·È ÛÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜. ∏ ·ÈÙÈÔÏÔÁ›· Ù˘ ¢¡∂ ·Ú·Ì¤ÓÂÈ ·Û·Ê‹˜ (1). ∞ÚÎÂÙ¤˜ ıˆڛ˜ ¤¯Ô˘Ó ‰È·Ù˘ˆı› Ì ÂÈÎÚ·Ù¤ÛÙÂÚ˜ ÙȘ ·ÎfiÏÔ˘ı˜: ∞) ªË¯·ÓÈÎÔ› ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ì›· ·ÓÒÌ·ÏË ÔÌÊ·ÏÈ΋ ·ÚÙËÚ›·, ÂÌÔ‰›˙Ô˘Ó ÙËÓ ¿ÓÔ‰Ô ÙÔ˘ ÓÂÊÚÔ‡ Ô‰ËÁÒÓÙ·˜ ÙÔÓ ÛÙÔ ¿ÏÏÔ ËÌÈÌfiÚÈÔ ÙÔ˘ ÛÒÌ·ÙÔ˜ (1,2,4,5). µ) ∞ÓÒÌ·ÏË ÙÔÔı¤ÙËÛË Ù˘ Ô˘ÚËÙËÚÈ΋˜ ηٷ‚ÔÏ‹˜ ¿ÁÂÈ ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ÂÙÂÚfiÏ¢ÚÔ˘ ÓÂÊÚÔÁfiÓÔ˘ ‚Ï·ÛÙ‹Ì·ÙÔ˜. ∞ӈ̷ϛ˜ ÛÙË ÛÙÚÔÊ‹ Î·È ÙËÓ Î¿Ì„Ë ÙÔ˘ ÂÌ‚Ú‡Ô˘ Èı·ÓfiÓ Ó· ·ÔÙÂÏÔ‡Ó ÙËÓ ·ÈÙ›· Ù˘ ÙÔÔı¤ÙËÛ˘ Î·È ÙˆÓ ‰‡Ô Ô˘ÚËÙËÚÈÎÒÓ Î·Ù·‚ÔÏÒÓ ÛÙËÓ ›‰È· ÏÂ˘Ú¿ (1,2,4,5). °) ¢È¿ÊÔÚÔÈ ÙÂÚ·ÙÔÁfiÓÔÈ ·Ú¿ÁÔÓÙ˜ ÂȉÚÔ‡Ó ÛÙÔ ÂÌ‚Ú˘˚Îfi ÂÚÈ‚¿ÏÏÔÓ, ¤¯ÔÓÙ·˜ Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙfiÛÔ ÙË ¢¡∂, fiÛÔ Î·È ÙȘ ¿ÏϘ Û˘Á-
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Paediatriki 2001;64:514-517
∂ÈÎfiÓ· 3. ™Ù·ÙÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· DMSA: ·ÓÔÌÔÈÔÁÂÓ‹˜ ηı‹ÏˆÛË ÙÔ˘ Ú·‰ÈÔÊ·ÚÌ¿ÎÔ˘ Î·È ÛÙÔ˘˜ ‰‡Ô ÓÂÊÚÔ‡˜.
ÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ Ô˘ Û˘¯Ó¿ ÙË Û˘ÓÔ‰Â‡Ô˘Ó (1,2,4). OÈ ÂÚÈÙÒÛÂȘ ¢¡∂ ·Ú·Ì¤ÓÔ˘Ó ÛÙËÓ ÏÂÈÔÓfiÙËÙ¿ ÙÔ˘˜ ·Û˘Ìو̷ÙÈΤ˜, fiˆ˜ Ê·›ÓÂÙ·È ·fi ÙË Û¯ÂÙÈο ˘„ËÏ‹ Û˘¯ÓfiÙËÙ¿ Ù˘ ÛÙȘ ÓÂÎÚÔÙÔ̤˜ Û ۯ¤ÛË Ì ÙȘ ·Ó·ÊÂÚfiÌÂÓ˜ ÂÚÈÙÒÛÂȘ ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· (1). ∞fi ÙȘ ÂÚÈÙÒÛÂȘ Ô˘ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È, ¤Ó· ÔÛÔÛÙfi 60% ÂÚ›Ô˘ ÚÔ‚¿ÏÏÂÈ Ì ÂÈÎfiÓ· ÏÈı›·Û˘ ‹ Ïԛ̈͢ Ô˘ÚÔÔÈËÙÈÎÔ‡ (ÎÔÈÏÈ·Îfi ¿ÏÁÔ˜, ·ÈÌ·ÙÔ˘Ú›·, ‰˘ÛÔ˘ÚÈο ÂÓԯϋ̷ٷ, ˘Ô˘Ú›·), ÂÓÒ Û ÔÛÔÛÙfi 30% ÂÚ›Ô˘ ÚÔ‚¿ÏÏÂÈ ˆ˜ ÎÔÈÏȷ΋ Ì¿˙·. ∆¤ÏÔ˜, Û ÔÛÔÛÙfi 10-20% Ë ‰È¿ÁÓˆÛË ·ÔÙÂÏ› Ù˘¯·›Ô ‡ÚËÌ·, fiˆ˜ Î·È ÛÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜ (1,3). ™ÙȘ ̤Ú˜ Ì·˜, Ë Â˘Ú›· ÂÊ·ÚÌÔÁ‹ ÙˆÓ ·ÂÈÎÔÓÈÛÙÈÎÒÓ ÌÂıfi‰ˆÓ ı· ·˘Í‹ÛÂÈ ·ÎfiÌ· ÂÚÈÛÛfiÙÂÚÔ ÙÔ ÔÛÔÛÙfi ÙˆÓ Ù˘¯·›· ·Ó¢ÚÈÛÎfiÌÂÓˆÓ ÂÚÈÙÒÛÂˆÓ (1). O ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ ÌÔÚ› Ó· ·ÚÔ˘ÛÈ¿˙ÂÈ ‰È¿Ù·ÛË ÙÔ˘ ˘ÂÏÔÎ·Ï˘ÎÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ÛÙ¤ÓˆÛË Ù˘ ˘ÂÏÔÔ˘ÚËÙËÚÈ΋˜ Û˘Ì‚ÔÏ‹˜, ΢ÛÙÂÔÔ˘ÚËÙËÚÈ΋ ·ÏÈÓ‰ÚfiÌËÛË Î·È Î˘ÛÙÈ΋ ‰˘ÛÏ·Û›· (1,4,5,6). ∏ ·ÓÒÌ·ÏË ı¤ÛË ÙÔ˘ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡, Ë ˘„ËÏ‹ ›ÛÔ‰Ô˜ ÙÔ˘ Ô˘ÚËÙ‹Ú· ÛÙËÓ ‡ÂÏÔ, Ô ·ÓÒÌ·ÏÔ˜ ÂÚÈÛÙ·ÏÙÈÛÌfi˜ Ù˘ ˘¤ÏÔ˘ ÙÔ˘ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡, ›ÂÛË ·fi ·Ú·Î›ÌÂÓ· ÌfiÚÈ· (fiˆ˜ ¤ÎÙÔ· ·ÁÁ›·), ‰ËÌÈÔ˘ÚÁÔ‡Ó Û˘Óı‹Î˜ η΋˜ ·ÚÔ¯¤Ù¢Û˘ ÙˆÓ Ô‡ÚˆÓ Î·È ÚÔ¸Ôı¤ÛÂȘ ÁÈ· ‰ËÌÈÔ˘ÚÁ›· Ï›ıˆÓ Î·È ÏÔÈÌÒÍÂˆÓ Ô˘ÚÔÔÈËÙÈÎÔ‡ (4). ™ÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜, Ë ·ÚÔ¯¤Ù¢ÛË ÙˆÓ Ô‡ÚˆÓ ˘ÔÏÂÈfiÙ·Ó Î·È ÛÙÔ˘˜ ‰‡Ô ÓÂÊÚÔ‡˜, Û ÛËÌ·ÓÙÈο fï˜ ÌÂÁ·Ï‡ÙÂÚÔ ‚·ıÌfi ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi, ¯ˆÚ›˜ Ó· ÌÔÚ› Ó· ÚÔÛ‰ÈÔÚÈÛÙ› Ë ·ÎÚÈ‚‹˜ ·ÈÙÈÔÏÔÁ›·. ∂˘Ú‹Ì·Ù· ÏÈı›·Û˘ ·Ô˘Û›·˙·Ó, ÂÓÒ ÔÈ ·ÏÏÔÈÒÛÂȘ Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙÔ ÛÙ·ÙÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ DMSA Èı·ÓÒ˜ ÔÊ›ÏÔÓÙ·Ó Û ˘ÂÏÔÓÂÊÚÈÙȉÈΤ˜ Ô˘Ï¤˜ ·fi ÚÔËÁÔ‡ÌÂÓ˜ Ô˘ÚÔÏÔÈÌÒÍÂȘ ‹ ‹Ù·Ó ÙÔ ·ÔÙ¤ÏÂÛÌ·
¶·È‰È·ÙÚÈ΋ 2001;64:514-517
‰˘ÛÏ·Û›·˜. ¶Ú¤ÂÈ Ó· ÛËÌÂÈÒÛÔ˘Ì fiÙÈ ÔÈ ÓÂÊÚÔ› ÛÙË ¢¡∂ Û˘¯Ó¿ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ÓˆÌ·Ï›Â˜ ÛÙÚÔÊ‹˜, Ô˘ ¤¯Ô˘Ó Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙËÓ ˘ÂÚÂÎÙ›ÌËÛË Ù˘ ‰È¿Ù·Û˘ ÙÔ˘ ˘ÂÏÔÎ·Ï˘ÎÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ηٿ ÙÔÓ ·ÂÈÎÔÓÈÛÙÈÎfi ¤ÏÂÁ¯Ô, fiˆ˜ Û˘Ó¤‚Ë ÛÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜ (1). O ÌÔÓ‹Ú˘ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜, Û ۯ¤ÛË Ì ÙȘ ¿ÏϘ ÌÔÚʤ˜ ¢¡∂, ·ÚÔ˘ÛÈ¿˙ÂÈ ÙË ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· Û˘ÁÁÂÓÒÓ ·ÓˆÌ·ÏÈÒÓ, ÌÂ Û˘¯ÓfiÙÂÚ˜ ÙȘ ÛÎÂÏÂÙÈΤ˜ (50%), ÙÔ˘ ÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (40%) (ÎÚ˘„ÔÚ¯›·, ·ÙÚËÛ›· ÎfiÏÔ˘, ·ÓˆÌ·Ï›Â˜ Û¯‹Ì·ÙÔ˜ Ì‹ÙÚ·˜, ˘ÔÛ·‰›·˜ Î.Ï.), ÙËÓ ·ÙÚËÛ›· ÔÚıÔ‡ (20%) (1,2,4). ™ÙȘ ¿ÏϘ ÌÔÚʤ˜ ¢¡∂, Ë Û˘¯ÓfiÙËÙ· ÙˆÓ Û˘ÁÁÂÓÒÓ ·ÓˆÌ·ÏÈÒÓ Â›Ó·È Ôχ ÌÈÎÚfiÙÂÚË, ÌÂ Û˘¯ÓfiÙÂÚ˜ ÙËÓ ·ÙÚËÛ›· ÔÚıÔ‡ (4%), ÔÚıÔ‰ÈΤ˜ Î·È ÛÎÂÏÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ (ËÌÈÛfiÓ‰˘ÏÔ˜, ÛÎÔÏ›ˆÛË, ·ʈÛË, Û˘ÁÁÂÓ¤˜ ÂÍ¿ÚıËÌ· ÙÔ˘ ÈÛ¯›Ô˘), ÂÏÏ›ÌÌ·Ù· ÙÔ˘ ÎÔÏÔÎÔÈÏÈ·ÎÔ‡ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ¿ÏϘ ·ÓˆÌ·Ï›Â˜ Á·ÛÙÚÂÓÙÂÚÈÎÔ‡ (ÔÈÛÔÊ·ÁÈ΋ ·ÙÚËÛ›·, ÙÚ·¯ÂÈÔÔÈÛÔÊ·ÁÈÎfi Û˘Ú›ÁÁÈÔ), ·ÓˆÌ·Ï›Â˜ ÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (1,4-6). ∞ÛıÂÓ›˜ ÌÂ Ì˘ÂÏÔ‰˘ÛÏ·Û›· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ¢¡∂ ÛÂ Û˘¯ÓfiÙËÙ· Ô˘ Êı¿ÓÂÈ ¤ˆ˜ 3,3%, ÔÛÔÛÙfi Ôχ ˘„ËÏfiÙÂÚÔ ·fi ÂΛÓÔ ÙÔ˘ ÁÂÓÈÎÔ‡ ÏËı˘ÛÌÔ‡ (6,7). ™ÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜ Û˘Ó˘‹Ú¯·Ó Â˘Ú‹Ì·Ù· ·fi ÙÔ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· (ÊÏ‚È΋ ·ÁÁÂȷ΋ ‰˘ÛÏ·Û›· ÛÙÔ ‰ÂÍÈfi ÌÂوȷ›Ô ÏÔ‚fi), ηıÒ˜ Î·È ·fi Ù· ÂÈÓÂÊÚ›‰È· (Û˘ÁÁÂÓ‹˜ ˘ÂÚÏ·Û›· ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ ηı˘ÛÙÂÚË̤Ó˘ ¤Ó·Ú͢), Ù· ÔÔ›· fï˜ ‰Â ÁÓˆÚ›˙Ô˘Ì ηٿ fiÛÔ ÌÔÚÔ‡Ó Ó· Û˘Û¯ÂÙÈÛÙÔ‡Ó Ì ÙË ¢¡∂. ™ÙÔ ·ÚÂÏıfiÓ, Ë ÂÓ‰ÔÊϤ‚È· ˘ÂÏÔÁÚ·Ê›· ‹Ù·Ó Ë Ì¤ıÔ‰Ô˜ ÂÎÏÔÁ‹˜ ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù˘ ¢¡∂. ™‹ÌÂÚ·, ÙfiÛÔ ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· fiÛÔ Î·È ÙÔ ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ (ÛÙ·ÙÈÎfi Î·È ‰˘Ó·ÌÈÎfi) ¤¯Ô˘Ó ·Ôηχ„ÂÈ ·ÚÎÂÙ¤˜ ·Û˘Ìو̷ÙÈΤ˜ ÂÚÈÙÒÛÂȘ ¢¡∂. ∏ ·ÓÈÔ‡Û· ΢ÛÙÂÔÔ˘ÚËıÚÔÁÚ·Ê›· ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙÔÓ ·ÔÎÏÂÈÛÌfi Ù˘ ΢ÛÙÂÔÔ˘ÚËÙËÚÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘, ÂÓÒ ÙÔ ‰˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ ‚ÔËı¿ÂÈ ÛËÌ·ÓÙÈο Û ÂÚÈÙÒÛÂȘ ˘‰ÚÔÓ¤ÊÚˆÛ˘ ÒÛÙ ӷ ηıÔÚÈÛÙ› Ë ÈηÓfiÙËÙ· ·ÚÔ¯¤Ù¢Û˘ ÙˆÓ Ô‡ÚˆÓ, fiˆ˜ ¤ÁÈÓÂ Î·È ÛÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜ (1). ¶ÔÏϤ˜ ÊÔÚ¤˜, Ô ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‰ÂÓ ÌÔÚ› Ó· ‰È·ÎÚ›ÓÂÈ ·Ó ˘¿Ú¯ÂÈ ‹ fi¯È Û˘ÁÎfiÏÏËÛË ÙˆÓ ‰‡Ô ÓÂÊÚÒÓ Î·È Ë ‰È¿ÁÓˆÛË Û˘¯Ó¿ Ù›ıÂÙ·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘
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¯ÂÈÚÔ˘ÚÁ›Ԣ. ∆· ·ÁÁ›· ÛÙË ¢¡∂, ȉ›ˆ˜ ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi, ¤¯Ô˘Ó Ôχ Û˘¯Ó¿ ·ÓÒÌ·ÏË ÚԤϢÛË, ÁÂÁÔÓfi˜ Ô˘ οÓÂÈ ·Ó·Áη›· ÙË ‰ÈÂÓ¤ÚÁÂÈ· ·ÁÁÂÈÔÁÚ·Ê›·˜ ÚÈÓ ·fi ÂÎÙÂٷ̤ӷ ¯ÂÈÚÔ˘ÚÁ›· (1,2). OÈ ÂÚÈÛÛfiÙÂÚÔÈ ·ÛıÂÓ›˜ Ì ¢¡∂ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Î·Ï‹ ÚfiÁÓˆÛË Î·È Ê˘ÛÈÔÏÔÁÈÎfi ÚÔÛ‰fiÎÈÌÔ ÂÈ‚›ˆÛ˘. ŸÛÔÈ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Èı·Ó‹ ·fiÊÚ·ÍË ÙÔ˘ ·Ô¯ÂÙ¢ÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ‚Ú›ÛÎÔÓÙ·È Û ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· Ïԛ̈ÍË Ô˘ÚÔÔÈËÙÈÎÔ‡ Î·È ÏÈı›·ÛË. ¶ÂÚ›Ô˘ ÙÔ 1/3 ÙˆÓ Û˘Ìو̷ÙÈÎÒÓ ·ÛıÂÓÒÓ ··ÈÙ› ˘ÂÏÔÏÈıÔÙÔÌ‹ ÁÈ· Ï›ıÔ Ô˘ ÚÔηÏ› ·fiÊÚ·ÍË (1). ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ¢¡∂ Â›Ó·È Ì›· Û¿ÓÈ· ÔÓÙfiÙËÙ·. ¢È·ÁÈÁÓÒÛÎÂÙ·È ·ÎfiÌ· Û·ÓÈfiÙÂÚ· ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙˆÓ ÂÈÏÔÎÒÓ Ù˘, ÔÈ Ôԛ˜ fiÙ·Ó ·Ó¢ڛÛÎÔÓÙ·È ··ÈÙÔ‡Ó ¿ÌÂÛË ·ÓÙÈÌÂÙÒÈÛË. µÈ‚ÏÈÔÁÚ·Ê›· 1. Bauer S. Anomalies of the kidney and ureteropelvic junction. In: Campbell’s Urology. 7th ed. Philadelphia: WB Saunders Company; 1998. p. 1720-1725. 2. Baniel J, Glezerson G, Tobiaw M, Manning AJ. Crossed renal ectopia. Scad J Urol Nephrol 1991;25:241-244. 3. Fowlis GA, Mee AD, Colbeck R, Twomey B. Acute abdomen, an unusual presentation of crossed renal ectopia. Br J Urol 1994;73:328-329. 4. Felzenberg J, Nasrallah PF. Crossed renal ectopia without fusion associated with hydronephrosis in an infant. Urology 1991;38:450-452. 5. Marshal FF, Freedman MT. Crossed renal ectopia. J Urol 1978;119:188-191. 6. Dragun MJ, Flemming D, Knorr A, Borden T, Smith A. Crossed fused renal ectopy in children with myelodysplasia. Urology 1994;44:562-564. 7. Robson WLM, Leung AKC. Crossed fused renal ectopy and myelodysplasia [letter]. Urology 1995;45:1087.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 03-11-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 23-03-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ÃÚ›ÛÙÔ˜ ∫·Ú‹˜ ∑·Î‡ÓıÔ˘ 2, ÷ϿӉÚÈ
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∂•∂§π•∂π™ ™∆∏¡ π∞∆ƒπ∫∏ ∂¶π™∆∏ª∏
Paediatriki 2001;64:518-526
PROGRESS IN MEDICAL SCIENCE
™ÔÁÁÒ‰ÂȘ ÂÁÎÂÊ·ÏÔ¿ıÂȘ. ¡ÂfiÙÂÚ˜ ·fi„ÂȘ ∫. ∫·Ú·‚·Ó¿ÎË - ∫·Ú·Ó¿ÛÈÔ˘
Spongiform encephalopathies. Recent advances K. Karavanaki - Karanasiou
¶ÂÚ›ÏË„Ë: OÈ ÛÔÁÁÒ‰ÂȘ ÂÁÎÂÊ·ÏÔ¿ıÂȘ ·ÔÙÂÏÔ‡Ó Ì›· ÔÌ¿‰· ÌÂÙ·‰È‰fiÌÂÓˆÓ, ÚÔԉ¢ÙÈο ÂÍÂÏÈÛÛfiÌÂÓˆÓ Ó¢ÚÔÂÎÊ˘ÏÈÛÙÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ, ·fi ÙȘ Ôԛ˜ Ë Û˘ÓËı¤ÛÙÂÚ· ÂÌÊ·ÓÈ˙fiÌÂÓË ÛÙÔÓ ¿ÓıÚˆÔ Â›Ó·È Ë ÓfiÛÔ˜ Creutzfeldt-Jakob (CJD). ∏ CJD ‰È·ÎÚ›ÓÂÙ·È Û ÎÏËÚÔÓÔÌÈ΋, ÏÔÈÌÒ‰Ë (Kuru, È·ÙÚÔÁÂÓ‹ CJD) Î·È ÛÔÚ·‰È΋ (ȉÈÔ·ı‹). ∏ Ó¤· ÔÈÎÈÏ›· Ù˘ ÛÔÚ·‰È΋˜ CJD (new variant CJD) ·ÔÙÂÏ› ÙËÓ ·ÓıÚÒÈÓË ÛÔÁÁÒ‰Ë ÂÁÎÂÊ·ÏÔ¿ıÂÈ·, Ë ¤Í·ÚÛË Ù˘ ÔÔ›·˜ Û˘Û¯ÂÙ›ÛÙËΠ̠ÙËÓ ÂȉËÌ›· Ù˘ ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ ÙˆÓ ‚ÔÔÂȉÒÓ (™∂µ). O ˘Â‡ı˘ÓÔ˜ ·Ú¿ÁÔÓÙ·˜ Â›Ó·È Ë ·ıÔÏÔÁÈ΋ ÚˆÙ½ÓË prion (proteinaceous infectious), Ë ÔÔ›· ‰ÂÓ ÂÚȤ¯ÂÈ DNA ‹ RNA. ∏ ÚˆÙ½ÓË prion, ÌÂÙ¿ ÙËÓ Â›ÛÔ‰fi Ù˘ ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi, ·Ú¯Èο ÂÓ·ÔÙ›ıÂÙ·È ÛÙÔ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·Îfi Û‡ÛÙËÌ· Î·È ÛÙË Û˘Ó¤¯ÂÈ· ÚÔÛ‚¿ÏÏÂÈ Ù· ÂÚÈÊÂÚÈο Ó‡ڷ Î·È ÙÔÓ ÂÁΤʷÏÔ, ÚÔηÏÒÓÙ·˜ ÙËÓ ÚÔԉ¢ÙÈ΋ ÂÎʇÏÈÛ‹ ÙÔ˘˜. ∏ ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ Á›ÓÂÙ·È Ì ÙËÓ ·ÔÌfiÓˆÛË ÙˆÓ ÚˆÙÂ˚ÓÒÓ prions ÛÙÔÓ ÂÁΤʷÏÔ ‹ ÙȘ Ê·Ú˘ÁÁÈΤ˜ ·Ì˘Á‰·Ï¤˜, ÂÓÒ Ù· Â˘Ú‹Ì·Ù· ·fi ÙÔ ÏÔÈfi ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ‰ÂÓ Â›Ó·È ÂȉÈο. ∏ CJD ÌÂÙ·‰›‰ÂÙ·È ÛÙÔÓ ¿ÓıÚˆÔ È·ÙÚÔÁÂÓÒ˜, Ì ÙË ¯Ú‹ÛË ÙˆÌ·ÙÈ΋˜ ·ÓıÚÒÈÓ˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘, Ì ÙË ÌÂÙ¿ÁÁÈÛË ÌÔÏ˘Ṳ̂ÓÔ˘ ·›Ì·ÙÔ˜ ‹ ·Ú·ÁÒÁˆÓ Î·È ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË ÔÚÁ¿ÓˆÓ. ∂›Û˘, ÌÂÙ·‰›‰ÂÙ·È ‰È¿ Ù˘ ÙÚÔÊÈ΋˜ Ô‰Ô‡, Ì ÙË ‚ÚÒÛË ÎÚ¤·ÙÔ˜ ‚Ô‰ÈÓÔ‡, ÚÔÂÚ¯fiÌÂÓÔ˘ ·fi ˙ÒÔ ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 2 ÂÙÒÓ Ô˘ ¿Û¯ÂÈ ·fi ™Eµ. ∆· Úfi‚·Ù·, Ù· ¯ÔÈÚÈÓ¿, Ù· Ô˘ÏÂÚÈο Î·È ÙÔ Á¿Ï· ‰ÂÓ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ· ÙË ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘. ∞Ó·ÊÔÚÈο Ì ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙË ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ ™Eµ ·Ó¿ÌÂÛ· ÛÙ· ‚ÔÔÂȉ‹,
Abstract: The human transmissible spongiform encephalopathies are a group of rapidly progressive neurodegenerative disorders. The most common human type is Creutzfeldt-Jakob disease (CJD). CJD is classified as a genetic, or infectious (Kuru, iatrogenic) or a sporadic (idiopathic) form. The new variant CJD (nvCJD) is considered to be the human type of the bovine spongiform encephalopathy (BSE). The factor responsible is the abnormal prion (proteinaceous infectious), which is a protein without DNA or RNA. After ingestion, prion initially accumulates in the lymphoreticular tissues and progressively causes neuroinvasion and fatal neurodegeneration. The diagnosis of CJD is based on the demonstration of prion in the brain parenchyma or in biopsy specimens of the pharyngeal tonsil, while the rest of the laboratory findings are non-specific for the disease. CJD is transmissible iatrogenically, through cadaveric human growth hormone, human blood and blood products and organ transplantation. It is also transmissible orally through the consumption of bovine meat originating from cattle, older than 2 years of age, with confirmed BSE. Sheep, pigs, poultry and bovine milk are not sources of transmission of the disease. Age, sex and maternal transmission are not considered responsible for the transmission of BSE among cattle, while the use of commercial concentrated feed containing animal derived products is considered the major source of epidemics among cattle. The pronounced resistance of prions to proteinases, to heat and to the host defense mechanisms are factors facilitating the transmission through the oral route. In Greece, up
µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” ∞ı‹Ó·
µ’ Pediatric Clinic, Children’s Hospital “Aghia Sophia”, Athens
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¶·È‰È·ÙÚÈ΋ 2001;64:518-526
Paediatriki 2001;64:518-526
Ë ËÏÈΛ·, ÙÔ Ê‡ÏÔ Î·È Ë ‰È·Ï·ÎÔ˘ÓÙȷ΋ ÌÂÙ¿‰ÔÛË ‰ÂÓ Â˘ı‡ÓÔÓÙ·È ÁÈ· ÙË ‰È·ÛÔÚ¿ Ù˘ ÓfiÛÔ˘, ÂÓÒ ÛÙ·ıÂÚfi ·›ÙÈÔ ıˆÚÂ›Ù·È Ë ‚ÚÒÛË ÂȉÈο ÂÂÍÂÚÁ·ÛÌ¤ÓˆÓ ˙ˆÔÙÚÔÊÒÓ. ∏ ÌfiÏ˘ÓÛË ÙÔ˘ ·ÓıÚÒÔ˘ ·fi ÌÔÏ˘Ṳ̂ÓÔ ÎÚ¤·˜ Â›Ó·È ÂÊÈÎÙ‹ ÏfiÁˆ Ù˘ ÌÂÁ¿Ï˘ ·ÓÙÔ¯‹˜ ÙˆÓ prions ÛÙȘ ÚˆÙ¿Û˜, ÙË ı¤ÚÌ·ÓÛË Î·È ÙÔ˘˜ ·Ì˘ÓÙÈÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜ ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡. ™ÙË ¯ÒÚ· Ì·˜ ‰ÂÓ ·Ó·Ê¤ÚÔÓÙ·È Ì¤¯ÚÈ Û‹ÌÂÚ· ÂÚÈÛÙ·ÙÈο Ù˘ Ó¤·˜ ÛÔÚ·‰È΋˜ ÌÔÚÊ‹˜. ¶·Ú¿ Ù·‡Ù·, Û·Ê›˜ ÂӉ›ÍÂȘ ÁÈ· ÙËÓ ¤ÎÙ·ÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ı· ¤¯Ô˘Ì ÌÂÙ¿ ·fi ¯ÚfiÓÈ·, ÏfiÁˆ ÙÔ˘ ÌÂÁ¿ÏÔ˘ ¯ÚfiÓÔ˘ ÂÒ·Û˘ Ù˘ ÓfiÛÔ˘.
to now, there have been no reported cases of the new sporadic form of the disease (nvCJD). However, clear indications on the incidence of the nvCJD will not be available for a considerable period of time, due to the long incubation period of the disease.
§¤ÍÂȘ ÎÏÂȉȿ: ÛÔÁÁ҉˘ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·, ÓfiÛÔ˜ Creutzfeldt-Jakob (CJD), Ó¤· ÔÈÎÈÏ›· ÓfiÛÔ˘ CJD, ÛÔÁÁ҉˘ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ‚ÔÔÂȉÒÓ.
Key words: spongiform encephalopathy, Creutzfeldt-Jakob disease (CJD), new variant CJD, bovine spongiform encephalopathy.
∂ÈÛ·ÁˆÁ‹ ∞˘Ùfi Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÈ ÙË ÛÔÁÁÒ‰Ë ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ‚ÔÔÂȉÒÓ (™∂µ), ÁÓˆÛÙ‹ ˆ˜ ÓfiÛÔ ÙˆÓ “ÙÚÂÏÒÓ ·ÁÂÏ¿‰ˆÓ”, Â›Ó·È Ë ÂÈÛÙËÌÔÓÈ΋ ·‚‚·ÈfiÙËÙ·. ∏ ÂȉËÌ›· Ù˘ ™∂µ ͤ۷Û ÙÔ 1986 ÛÙËÓ ∞ÁÁÏ›·. °È· ÙË ÌfiÏ˘ÓÛË ÙˆÓ ·ÁÂÏ¿‰ˆÓ ÂÓÔ¯ÔÔÈ‹ıËÎ·Ó ˙ˆÔÙÚÔʤ˜ Ô˘ ÂÚÈ›¯·Ó ÌÔÏ˘Ṳ̂ӷ ÂÓÙfiÛıÈ· ÚÔ‚¿ÙˆÓ Ô˘ ¤·Û¯·Ó ·fi ÙË ÓfiÛÔ scrapie (ÙÚÔÌÒ‰Ë ÓfiÛÔ ÙˆÓ ÚÔ‚¿ÙˆÓ). À¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ fiÙÈ Ë ÓfiÛÔ˜ ¤Ú·Û ·fi Ù· Úfi‚·Ù· ÛÙȘ ·ÁÂÏ¿‰Â˜ Î·È ·fi ·˘Ù¤˜ ÛÙÔÓ ¿ÓıÚˆÔ. ∫‡ÚÈÔ˜ ·ÓÙÈÚfiÛˆÔ˜ ÙˆÓ ÛÔÁÁˆ‰ÒÓ ÂÁÎÂÊ·ÏÔ·ıÂÈÒÓ ÛÙÔÓ ¿ÓıÚˆÔ Â›Ó·È Ë ÓfiÛÔ˜ CreutzfeldtJakob (CJD) (1).
ÔÈÎÔÁÂÓ‹˜ ·¸Ó›· Î·È Ë prion ·Ì˘ÏÔÂȉÈ΋ ·ÁÁÂÈÔ¿ıÂÈ· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ (1). ™Ù· ˙Ò· ¤¯Ô˘Ó ·Ó·ÊÂÚı› 6 ·Ûı¤ÓÂȘ (¶›Ó·Î·˜ 1): Ë ÓfiÛÔ˜ Scrapie, Ô˘ ÚÔÛ‚¿ÏÏÂÈ Ù· ·ÈÁÔÚfi‚·Ù·, Ë ÛÔÁÁ҉˘ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ‚ÔÔÂȉÒÓ, Ô˘ ÚÔÛ‚¿ÏÏÂÈ ÙȘ ·ÁÂÏ¿‰Â˜, Ë ¯ÚfiÓÈ· ÂÎÊ˘ÏÈÛÙÈ΋ ÓfiÛÔ˜, Ô˘ ÂÌÊ·Ó›˙ÂÙ·È ÛÙ· ÂÏ¿ÊÈ· Î·È ÙÔ˘˜ Ù·Ú¿Ó‰Ô˘˜, Ë ÌÂÙ·‰È‰fiÌÂÓË ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ÈÎÙȉÒÓ, Ô˘ ÂÌÊ·Ó›˙ÂÙ·È ÛÙȘ Ó˘Ê›ÙÛ˜, Ë ÛÔÁÁ҉˘ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ·ÈÏÔ˘ÚÔÂȉÒÓ, Ô˘ ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÛÙȘ Á¿Ù˜ Î·È Ù¤ÏÔ˜, Ë Â͈ÙÈ΋ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ÔÏËÊfiÚˆÓ ıËÏ·ÛÙÈÎÒÓ, Ô˘ ÚÔÛ‚¿ÏÏÂÈ ÙȘ ·ÓÙÈÏfi˜ ÛÙË ¡fiÙÈ· ∞ÊÚÈ΋ (1). ∏ ÓfiÛÔ˜ CJD Â›Ó·È Ô Û˘ÓËı¤ÛÙÂÚÔ˜ Ù‡Ô˜ ·fi ÙȘ ·ÓıÚÒÈÓ˜ ÛÔÁÁÒ‰ÂȘ ÂÁÎÂÊ·ÏÔ¿ıÂȘ (¶›Ó·Î·˜ 2). ∞˘Ù‹ ηٷٿÛÛÂÙ·È ˆ˜ ÂÍ‹˜ (2): ∞) ∫ÏËÚÔÓÔÌÈ΋ (ÔÈÎÔÁÂÓ‹˜ CJD), ÌÂÙ·‰È‰fiÌÂÓË Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú·, Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ÁÔÓȉ›Ô˘ PrP
∫·Ù¿Ù·ÍË ÛÔÁÁˆ‰ÒÓ ÂÁÎÂÊ·ÏÔ·ıÂÈÒÓ O fiÚÔ˜ “ÛÔÁÁÒ‰ÂȘ ÂÁÎÂÊ·ÏÔ¿ıÂȘ” ‹ ·Ûı¤ÓÂȘ prion ÂÚÈÏ·Ì‚¿ÓÂÈ Ù·¯¤ˆ˜ ÂÍÂÏÈÛÛfiÌÂÓ· ÓÔÛ‹Ì·Ù· ÊıÔÚ¿˜ ÙÔ˘ ∫¡™, Ô˘ ÚÔÛ‚¿ÏÏÔ˘Ó ·ÓıÚÒÔ˘˜ Î·È ˙Ò·. ∆Ô Î¿ı ›‰Ô˜ ˙ÒÔ˘ (Úfi‚·Ù·, ·ÁÂÏ¿‰Â˜, Ó˘Ê›ÙÛ˜, ÂÏ¿ÊÈ· Î.Ï.) ·ı·›ÓÂÈ ÙË ‰È΋ ÙÔ˘ ÌÔÚÊ‹ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜, Ô˘ ¤¯ÂÈ Ù· ͯˆÚÈÛÙ¿ ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ›‰Ô˘˜. ªÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ ÂȉÒÓ, ˘¿Ú¯ÂÈ Ô ÊÚ·ÁÌfi˜ ÙÔ˘ ›‰Ô˘˜, Ô ÔÔ›Ô˜ Ê·›ÓÂÙ·È fiÙÈ ÌÔÚ› Ó· Î·Ù·Ï˘ı› fiÙ·Ó Ë ÌÔÏ˘ÛÌ·ÙÈ΋ ‰fiÛË Â›Ó·È ÌÂÁ¿ÏË ‹ fiÙ·Ó Ô ÌÔÏ˘ÛÌ·ÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Â›Ó·È ÂÈıÂÙÈÎfi˜ (1). ∏ ÂÚ¢ÓËÙÈ΋ ÚÔÛ¤ÁÁÈÛË ÙˆÓ ÔÓÙÔÙ‹ÙˆÓ ·˘ÙÒÓ ·ÔÙÂÏ› ·Ú¿‰ÂÈÁÌ· Û˘ÓÂÚÁ·Û›·˜ ÔÏÏÒÓ ÎÏ¿‰ˆÓ Ù˘ È·ÙÚÈ΋˜ Î·È Ù˘ ‚ÈÔÏÔÁ›·˜. ∏ ¤Ú¢ӷ Û˘Ó¯›˙ÂÙ·È Î·È ‰ÂÓ ¤¯Ô˘Ó ·ÎfiÌ· ‰Ôı› fiϘ ÔÈ ··ÓÙ‹ÛÂȘ ÁÈ· ÙË ÌÂϤÙË ÙˆÓ ·ı‹ÛÂˆÓ ·˘ÙÒÓ. ™ÙÔÓ ¿ÓıÚˆÔ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ¤ÓÙ ÔÓÙfiÙËÙ˜ ·ÛıÂÓÂÈÒÓ prion (¶›Ó·Î·˜ 1): Ë ÓfiÛÔ˜ Kuru, Ë ÓfiÛÔ˜ Creutzfeldt-Jakob, ÙÔ Û‡Ó‰ÚÔÌÔ Gerstmann-Straussler-Scheinker, Ë ı·Ó·ÙËÊfiÚÔ˜
¶›Ó·Î·˜ 1. ¡ÔÛ‹Ì·Ù· prion ¡fiÛËÌ· ñ Kuru ñ Creutzfeldt-Jacob ñ Gerstmann-Straussler-Scheinker ñ £·Ó·ÙËÊfiÚÔ˜ ÔÈÎÔÁÂÓ‹˜ ·¸Ó›· ñ Prion ·Ì˘ÏÔÂȉÈ΋ ·ÁÁÂÈÔ¿ıÂÈ· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ ñ Scrapie ñ ™ÔÁÁ҉˘ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ‚ÔÔÂȉÒÓ ñ ÃÚfiÓÈ· ÂÎÊ˘ÏÈÛÙÈ΋ ÓfiÛÔ˜ ñ ªÂÙ·‰È‰fiÌÂÓË ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ÈÎÙȉÒÓ ñ ™ÔÁÁ҉˘ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ·ÈÏÔ˘ÚÔÂȉÒÓ ñ ∂͈ÙÈ΋ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ÔÏËÊfiÚˆÓ ıËÏ·ÛÙÈÎÒÓ
º˘ÛÈÎfi˜ ÍÂÓÈÛÙ‹˜ ÕÓıÚˆÔ˜ ÕÓıÚˆÔ˜ ÕÓıÚˆÔ˜ ÕÓıÚˆÔ˜ ÕÓıÚˆÔ˜ ∞ÈÁÔÚfi‚·Ù· ∞ÁÂÏ¿‰Â˜ ∂Ï¿ÊÈ·, Ù¿Ú·Ó‰ÔÈ ¡˘Ê›ÙÛ˜ °¿Ù˜ ∞ÓÙÈÏfiË, Nyala, Tragelaphus angasi
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¶·È‰È·ÙÚÈ΋ 2001;64:518-526
Ô˘ ‰ڿ˙ÂÙ·È ÛÙÔ ‚Ú·¯‡ ÛΤÏÔ˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 20 Î·È ÂϤÁ¯ÂÈ ÙËÓ ·Ú·ÁˆÁ‹ Ù˘ ÚˆÙ½Ó˘ prion. Œ¯Ô˘Ó ·Ú·ÙËÚËı› ÌÂÙ·ÏÏ¿ÍÂȘ Û 12 ÛËÌ›· Î·È ·ÚÎÂÙ¤˜ ÂÈÛ·ÁˆÁ¤˜ ˙¢ÁÒÓ ‚¿ÛˆÓ, ÔÈ Ôԛ˜ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ÔÈÎÔÁÂÓ‹ CJD (3). OÈ ‰‡Ô ÁÓˆÛÙfiÙÂÚ˜ ÂÓÙÔ›ÛÂȘ Ù˘ ÔÈÎÔÁÂÓÔ‡˜ CJD ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙȘ ·ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜ Ù˘ ™ÏÔ‚·Î›·˜ Î·È ÛÙÔ˘˜ §È‚˘Î‹˜ ηٷÁˆÁ‹˜ ∂‚Ú·›Ô˘˜ Ô˘ ηÙÔÈÎÔ‡Ó ÛÙÔ πÛÚ·‹Ï. ∏ ÔÈÎÔÁÂÓ‹˜ CJD ·Ú·ÙËÚÂ›Ù·È ÛÂ Û˘¯ÓfiÙËÙ· 6-15% ÙˆÓ ÂÚÈÙÒÛÂˆÓ CJD Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ (3). µ) §ÔÈÌ҉˘: ·. π·ÙÚÔÁÂÓ‹˜, Ë ÔÔ›· ·Ú·ÙËÚ‹ıËΠÙÔ 1985 ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ·ÓıÚÒÈÓ˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ Î·È ÁÔÓ·‰ÔÙÚÔÈÓÒÓ, ÚÔÂÚ¯fiÌÂÓˆÓ ·fi Âί˘Ï›ÛÌ·Ù· و̷ÙÈÎÒÓ ˘ÔʇÛˆÓ. ‚. NfiÛÔ˜ ∫uru (ÂȉËÌ›· ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ ·Ó¿ÌÂÛ· ÛÙÔ˘˜ ηÓÈ‚¿ÏÔ˘˜ ÙˆÓ Papua Ù˘ ¡¤·˜ °Ô˘˚Ó¤·˜). °) π‰ÈÔ·ı‹˜: ·. ™ÔÚ·‰È΋ ÌÔÚÊ‹, Ô˘ Â›Ó·È ÁÓˆÛÙ‹ ·fi ÙÔ 1920 Î·È ÂÌÊ·Ó›˙ÂÈ Ù˘¯·›· ηٷÓÔÌ‹. ∏ ÓfiÛÔ˜ Â›Ó·È ¿ÁÓˆÛÙ˘ ·ÈÙÈÔÏÔÁ›·˜, Ì ̤ÛË ËÏÈΛ· ¤Ó·Ú͢ Ù· 63 ¤ÙË (‡ÚÔ˜ ËÏÈÎÈÒÓ: 55-70 ¤ÙË). ∏ Û˘¯ÓfiÙËÙ¿ Ù˘ Â›Ó·È 0,5-1,5 ÂÚÈÙÒÛÂȘ/1.000.000/¤ÙÔ˜ Î·È ‰ÂÓ ¤¯ÂÈ ·˘ÍËı› Ë Û˘¯ÓfiÙËÙ· ·˘Ù‹ ·fi ÙÔ 1968, fiÙ·Ó ÚˆÙԉȷÈÛÙÒıËΠÛÙȘ ∏ӈ̤Ó˜ ¶ÔÏÈÙ›˜ (4). ∏ ÂÌÊ¿ÓÈÛË Ù˘ ÓfiÛÔ˘ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÂÈ Âԯȷ΋ ‹ ÁˆÁÚ·ÊÈ΋ ηٷÓÔÌ‹ Î·È ‰ÂÓ ¤¯ÂÈ ·Ú·ÙËÚËı› Ë ÌÂÙ¿‰ÔÛË ·fi ¿ÙÔÌÔ Û ¿ÙÔÌÔ ÌÂٷ͇ ÙˆÓ ÌÂÏÒÓ Ù˘ ›‰È·˜ ÔÈÎÔÁ¤ÓÂÈ·˜. ‚. ∏ Ó¤· ÔÈÎÈÏ›· Ù˘ CJD (4), fiÔ˘ Ë Ì¤ÛË ËÏÈΛ· ÙˆÓ ·Û¯fiÓÙˆÓ Â›Ó·È Ù· 28 ¤ÙË (‡ÚÔ˜ ËÏÈÎÈÒÓ: 10-39 ¤ÙË). ∆· ÚÒÙ· ÂÚÈÛÙ·ÙÈο Ù˘ ÓfiÛÔ˘ ·ÚÔ˘ÛÈ¿ÛÙËÎ·Ó ÛÙË ª. µÚÂÙ·Ó›· ÙÔ 1994. Œ¯Ô˘Ó ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ Ó¢ÚÔ·ıÔÏÔÁÔ·Ó·ÙÔÌÈΤ˜ ·ÏÏÔÈÒÛÂȘ Î·È Ë ÎÏÈÓÈ΋ ÔÚ›· Ù˘ ÓfiÛÔ˘ ‰ÂÓ Â›Ó·È Ë Ù˘È΋ Ù˘ ÛÔÚ·‰È΋˜ CJD. ∏ ÓfiÛÔ˜ ·˘Ù‹ Û˘Û¯ÂÙ›ÛÙËΠ·ÈÙÈÔÏÔÁÈο Ì ÙË ÛÔÁÁÒ‰Ë ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ‚ÔÔÂȉÒÓ (™∂µ), ‰ÈfiÙÈ ·ÚÔ˘ÛÈ¿ÛÙËΠ̠·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· Û ÎÙËÓÔÙÚfiÊÔ˘˜ Ô˘ ·Û¯ÔÏÔ‡ÓÙ·Ó Ì ÙËÓ ÂÎÙÚÔÊ‹ ‚ÔÔÂȉÒÓ ÛÙË ª. µÚÂÙ·Ó›·. ∏ ʇÛË ÙÔ˘ ÏÔÈÌÔÁfiÓÔ˘ ·Ú¿ÁÔÓÙ· - Ë ıˆڛ· ÙˆÓ Prions ∏ ÓfiÛÔ˜ CJD Î·È Ë ÛÔÁÁ҉˘ ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙˆÓ ‚ÔÔÂȉÒÓ ÔÊ›ÏÔÓÙ·È Û ¤Ó· ÌÔÏ˘ÛÌ·ÙÈÎfi ¶›Ó·Î·˜ 2. ∫·Ù¿Ù·ÍË Ù˘ ÓfiÛÔ˘ Creutzfeldt-Jacob (CJD) 1. ∫ÏËÚÔÓÔÌÈ΋ (ÌÂÙ¿ÏÏ·ÍË ÙÔ˘ ÁÔÓȉ›Ô˘ Ô˘ ÂϤÁ¯ÂÈ ÙËÓ ·Ú·ÁˆÁ‹ ÙÔ˘ prion) 2. §ÔÈÌ҉˘: ·) π·ÙÚÔÁÂÓ‹˜ (·ÓıÚÒÈÓË ·˘ÍËÙÈ΋ ÔÚÌfiÓË) ‚) ¡fiÛÔ˜ Kuru 3. π‰ÈÔ·ı‹˜: ·) ™ÔÚ·‰È΋ (variant CJD) ‚) ¡¤· ÔÈÎÈÏ›· ÛÔÚ·‰È΋˜ ÌÔÚÊ‹˜ (new variant CJD)
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Paediatriki 2001;64:518-526
·Ú¿ÁÔÓÙ·, ÙÔ prion (proteinaceous infectious), Ô˘ Â›Ó·È Ì›· ÚˆÙ½ÓË Ô˘ ‰ÂÓ ÂÚȤ¯ÂÈ DNA ‹ RNA. ∏ ÚˆÙ½ÓË ·˘Ù‹ Â›Ó·È È‰È·›ÙÂÚ· ·ÓıÂÎÙÈ΋ ÛÙȘ ÚˆÙ¿Û˜ Î·È ‰ÂÓ Î·Ù·ÛÙÚ¤ÊÂÙ·È ·fi ÙËÓ ˘ÂÚÈÒ‰Ë ·ÎÙÈÓÔ‚ÔÏ›· ‹ Ù· ¤Ó˙˘Ì· ÚÈ‚ÔÓÔ˘ÎÏ¿Û˜ (ÂÊfiÛÔÓ ‰ÂÓ ÂÚȤ¯ÂÈ DNA ‹ RNA) (5). ∏ ÌÔÏ˘ÛÌ·ÙÈ΋ ·˘Ù‹ ÚˆÙ½ÓË Â›Ó·È ÈÛfiÌÔÚÊË Ì ̛· Ê˘ÛÈÔÏÔÁÈ΋ ÚˆÙ½ÓË PrPC, ÌÔÚÈ·ÎÔ‡ ‚¿ÚÔ˘˜ 33-35 Kd, Ô˘ Έ‰ÈÎÔÔÈÂ›Ù·È ·fi ¤Ó· ÁÔÓ›‰ÈÔ Ô˘ ‰ڿ˙ÂÙ·È ÛÙÔ ‚Ú·¯‡ ÛΤÏÔ˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 20 (1). ∏ ÚˆÙ½ÓË ·˘Ù‹ ˘¿Ú¯ÂÈ ÛÙ· Ê˘ÛÈÔÏÔÁÈο ·ÙÙ·Ú·, ·ÏÏ¿ ΢ڛˆ˜ ÛÙËÓ Â͈ÙÂÚÈ΋ ÛÙÈ‚¿‰· ÙˆÓ Ó¢ÚÒÓˆÓ (1). ™ÙÔÓ ¿ÓıÚˆÔ Ô ÚfiÏÔ˜ Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ÚˆÙ½Ó˘ Â›Ó·È ¿ÁÓˆÛÙÔ˜. ™Ù· ÎÔÙfiÔ˘Ï· Ê·›ÓÂÙ·È fiÙÈ Û˘ÌÌÂÙ¤¯ÂÈ ÛÙËÓ ÔÌ·Ï‹ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ Û˘Ó¿„ÂˆÓ (6). ªÂÙ·ÏÏ¿ÍÂȘ ÛÙË Ê˘ÛÈÔÏÔÁÈ΋ ÚˆÙ½ÓË, ›Ù ÙÔ˘ Ù‡Ô˘ Ù˘ ÛËÌÂȷ΋˜ ÌÂÙ¿ÏÏ·Í˘, ›Ù ÙÔ˘ Ù‡Ô˘ Ù˘ ÚÔÛı‹Î˘ ˙¢ÁÒÓ ‚¿ÛˆÓ, ¤¯Ô˘Ó ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÌÊ¿ÓÈÛË ÙˆÓ prion ·ÛıÂÓÂÈÒÓ ÙÔ˘ ·ÓıÚÒÔ˘ (¶›Ó·Î·˜ 1), fiˆ˜ ÙÔ Û‡Ó‰ÚÔÌÔ GerstmannStraussler-Scheinker, Ë ı·Ó·ÙËÊfiÚÔ˜ ÔÈÎÔÁÂÓ‹˜ ·¸Ó›·, Ë prion ·Ì˘ÏÔÂȉÈ΋ ·ÁÁÂÈÔ¿ıÂÈ· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È ÔÈ ÎÏËÚÔÓÔÌÔ‡ÌÂÓÔÈ Ù‡ÔÈ Ù˘ ÓfiÛÔ˘ Creutzfeldt-Jakob (7). ∏ ·ıÔÏÔÁÈ΋ ÚˆÙ½ÓË PrPCJD ·ÚÔ˘ÛÈ¿˙ÂÈ ÙËÓ ›‰È· ¯ËÌÈ΋ Û‡ÛÙ·ÛË Ì ÙË Ê˘ÛÈÔÏÔÁÈ΋, ·ÏÏ¿ ¤¯ÂÈ ‰È·ÊÔÚÂÙÈ΋ ÛÙÂÚÂÔ¯ËÌÈ΋ ‰ÔÌ‹. ∏ PrPCJD ÂÈÛ¤Ú¯ÂÙ·È ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi ·fi ‰È¿ÊÔÚ˜ Ô‰Ô‡˜ (ÂÙÈÎfi, ·ÚÂÓÙÂÚÈο Î.Ï.). ∏ ›ÛÔ‰Ô˜ Ù˘ ·ıÔÏÔÁÈ΋˜ ÚˆÙ½Ó˘ ·fi ÙËÓ ÂÙÈ΋ Ô‰fi ·ÎÔÏÔ˘ı› ÙËÓ ÂÍ‹˜ ÔÚ›·: ·ÊÔ‡ ·ÓÙÈÛÙ·ı› ÛÙÔ˘˜ ¯ËÌÈÎÔ‡˜ Î·È ÂÓ˙˘ÌÈÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Î·È ÙÔ˘ ‰ˆ‰Âη‰·ÎÙ‡ÏÔ˘, ‰È·ÂÚÓ¿ ÙÔÓ ÂÓÙÂÚÈÎfi ÊÚ·ÁÌfi ‰È·Ì¤ÛÔ˘ ÙˆÓ Ï·ÎÒÓ ÙÔ˘ Payer ÙÔ˘ ÂÓÙ¤ÚÔ˘. ∫·ÙfiÈÓ ÚÔÛÏ·Ì‚¿ÓÂÙ·È ·fi ÙÔ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·Îfi Û‡ÛÙËÌ· (·Ì˘Á‰·Ï¤˜, ÛΈÏËÎÔÂȉ‹˜ ·fiÊ˘ÛË, ÏÂÌÊ·‰¤Ó˜) Î·È ÙÔ ÛÏ‹Ó·, fiÔ˘ Î·È ÂÓ·ÔÙ›ıÂÙ·È ¯ÚÔÓ›ˆ˜. ™ÙË Û˘Ó¤¯ÂÈ·, ÚÔÛ‚¿ÏÏÂÈ Ù· ÂÚÈÊÂÚÈο Ó‡ڷ, fiÔ˘ ÔÏÏ·Ï·ÛÈ¿˙ÂÙ·È Î·È Ô‰ËÁ› ÛÙËÓ ÂÎʇÏÈÛ‹ ÙÔ˘˜. ∆ÂÏÈο, ÂÁηı›ÛÙ·Ù·È ÛÙÔÓ ÂÁΤʷÏÔ, ·ÎÔÏÔ˘ıÒÓÙ·˜ ÙËÓ ÎÂÓÙÚÔÌfiÏÔ Ô‰fi, Ì ‚¿ÛË ÙÔ ÌÔÓÙ¤ÏÔ Ù˘ ÔÏÈÔÌ˘ÂÏ›Ùȉ·˜ (8). ∏ fiÏË ‰È·‰Èηۛ· Ù˘ ÂÓ·fiıÂÛ˘ Ù˘ ·ıÔÏÔÁÈ΋˜ ÚˆÙ½Ó˘ ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi ‰È·ÚΛ ÔÏÏ¿ ¤ÙË, fiˆ˜ ÛÙ· Ó¢ÚÔÂÎÊ˘ÏÈÛÙÈο ÓÔÛ‹Ì·Ù· Î·È Ô ·ÛıÂÓ‹˜ ·Ú·Ì¤ÓÂÈ ·Û˘Ìو̷ÙÈÎfi˜. O Ì˯·ÓÈÛÌfi˜ ÙÔ˘ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡ Ù˘ ·ıÔÏÔÁÈ΋˜ ÚˆÙ½Ó˘ prion Î·È Ù˘ ÌfiÏ˘ÓÛ˘ ÙˆÓ Ó¢ÚÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·ÂÈÎÔÓ›˙ÂÙ·È ÛÙÔ ™¯‹Ì· 1. ∏ ·ıÔÏÔÁÈ΋ ÚˆÙ½ÓË, Û ·Ï‹ ·ʋ Ì ÙË Ê˘ÛÈÔÏÔÁÈ΋ ÚˆÙ½ÓË, ÙË ÌÂÙ·ÙÚ¤ÂÈ Û ·ıÔÏÔÁÈ΋ (8). ∆· prions Ù›ıÂÓÙ·È ‰ÔÌÈο ÙÔ ¤Ó· ‰›Ï· ÛÙÔ ¿ÏÏÔ Î·È ‰ËÌÈÔ˘ÚÁÂ›Ù·È ¤ÙÛÈ Ì›· ·Ï˘Û›‰·
¶·È‰È·ÙÚÈ΋ 2001;64:518-526
Paediatriki 2001;64:518-526
·ıÔÏÔÁÈÎÒÓ prions (Ͽη), Ë ÔÔ›· ηٷÛÙÚ¤ÊÂÈ Ù· Ó¢ÚÈο ·ÙÙ·Ú· Î·È Ì·ÎÚÔÛÎÔÈο Ô ÂÁΤʷÏÔ˜ ·ÔÎÙ¿ ÙËÓ ÂÈÎfiÓ· ÛÊÔ˘ÁÁ·ÚÈÔ‡. ∏ Û˘ÛÛÒÚ¢ÛË Ù˘ ·ıÔÏÔÁÈ΋˜ ÚˆÙ½Ó˘ ÛÙÔÓ ÂÁΤʷÏÔ Â›Ó·È ‚Ú·‰Â›· Î·È ÙÔ ¿ÙÔÌÔ Â›Ó·È ·Û˘Ìو̷ÙÈÎfi. ªÂ ÙËÓ ¿ÚÔ‰Ô ÙˆÓ ÂÙÒÓ, Ë Û˘ÛÛÒÚ¢ÛË Ù˘ ÚˆÙ½Ó˘ ·˘Í¿ÓÂÙ·È ‰›ÎËÓ ¯ÈÔÓÔÛÙÈ‚¿‰·˜ Î·È Ô ÂÁΤʷÏÔ˜ ·Ô‰ÈÔÚÁ·ÓÒÓÂÙ·È Ï‹Úˆ˜ (8). ∏ Ó¤· ÛÔÚ·‰È΋ ÌÔÚÊ‹ ÂΉËÏÒÓÂÙ·È Ì „˘¯È·ÙÚÈο Ê·ÈÓfiÌÂÓ· Î·È ‰È·Ù·Ú·¯¤˜ Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜, ÛÙȘ Ôԛ˜ ÌÂÙ¿ ·fi Ì‹Ó˜ ÚÔÛÙ›ıÂÙ·È Ó¢ÚÔÏÔÁÈ΋ ÛËÌÂÈÔÏÔÁ›·, fiˆ˜ Ì˘ÔÎÏÔӛ˜, ‰˘Û·ÈÛıËۛ˜, ·ÚÂÁÎÂÊ·ÏȉÈ΋ ·Ù·Í›· Î·È ÚÔԉ¢ÙÈ΋ ÂÌÊ¿ÓÈÛË ¿ÓÔÈ·˜ (10). O ·ÛıÂÓ‹˜ Ô‰ËÁÂ›Ù·È ÛÙÔ ı¿Ó·ÙÔ Ì¤Û· Û ¤Ó· ¯ÚfiÓÔ (6-22 Ì‹Ó˜) ·fi ÙËÓ ÂΉ‹ÏˆÛË ÙˆÓ Û˘Ìو̿وÓ. O ¯ÚfiÓÔ˜ ÂÒ·Û˘ Ù˘ ÓfiÛÔ˘ Â›Ó·È ¿ÁÓˆÛÙÔ˜. ∏ ÎÏ·ÛÈ΋ ÛÔÚ·‰È΋ ÌÔÚÊ‹ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ¿ÓÔÈ·, Ì˘fiÎÏÔÓÔ ‹ ÏÈÁfiÙÂÚÔ Û˘¯Ó¿ ¯ÔÚ›· ‹ ‰˘ÛÙÔÓ›·, ˘Ú·ÌȉÈ΋ ‹ Â͈˘Ú·ÌȉÈ΋ Û˘Ó‰ÚÔÌ‹ Î·È ·ÚÂÁÎÂÊ·ÏȉÈ΋ ·Ù·Í›· Î·È ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ·ÛıÂÓ›˜ ηٷϋÁÔ˘Ó 1 ¯ÚfiÓÔ ÌÂÙ¿ ·fi ÙËÓ ÂÌÊ¿ÓÈÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Ù˘ ÓfiÛÔ˘, ¤¯ÔÓÙ·˜ ÂÚȤÛÂÈ Û ·Ê·Û›· Î·È Ï‹ÚË ·ÎÈÓËÛ›· (9). ∞Ó¿Ï˘ÛË Ù˘ ÚˆÙ½Ó˘ PrPCJD Ù˘ Ó¤·˜ ÛÔ-
ªË¯·ÓÈÛÌfi˜ ÌfiÏ˘ÓÛ˘
¡Â˘ÚÈÎfi ·ÙÙ·ÚÔ
ÀÁÈ›˜ ÚˆÙ½Ó˜ prions
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ªÔÏ˘Ṳ̂Ó˜ ÚˆÙ½Ó˜
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ªË¯·ÓÈÛÌfi˜ ÌfiÏ˘ÓÛ˘: 1. ∂·Ê‹ ·ıÔÏÔÁÈ΋˜ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ÚˆÙ½ÓË prion 2. ªÂÙ·ÙÚÔ‹ Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ Û ·ıÔÏÔÁÈ΋ 3. ¢ËÌÈÔ˘ÚÁ›· ·Ï˘Û›‰·˜ prions (Ͽη) Ô˘ ηٷÛÙÚ¤ÊÂÈ Ù· Ó¢ÚÈο ·ÙÙ·Ú· ™¯‹Ì· 1. ∏ ‰Ú¿ÛË ÙˆÓ prions.
Ú·‰È΋˜ ÌÔÚÊ‹˜ Ì ÌÂıfi‰Ô˘˜ ÌÔÚȷ΋˜ ‚ÈÔÏÔÁ›·˜ ηٿ Western, ¤‰ÂÈÍ ÔÌÔÈfiÙËÙ˜ Ì ·ÓÙ›ÛÙÔȯ· Â˘Ú‹Ì·Ù· ·fi ÙȘ ·ÓÒ̷Ϙ ÚˆÙ½Ó˜ Ô˘ ·ÔÌÔÓÒıËÎ·Ó ·fi ÂÚÈÙÒÛÂȘ ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ ÙˆÓ ‚ÔÔÂȉÒÓ, fi¯È fï˜ Ì ÂΛӷ ·fi ÙȘ ÛÔÚ·‰ÈΤ˜ ÂÚÈÙÒÛÂȘ Creutzfeld-Jakob (11). ∂ÚÁ·ÛÙËÚȷΤ˜ ̤ıÔ‰ÔÈ ‰È¿ÁÓˆÛ˘ Ù˘ ÓfiÛÔ˘ Creutzfeldt-Jakob OÈ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÁ·ÛÙËÚȷΤ˜ ̤ıÔ‰ÔÈ ‰ÂÓ Â›Ó·È ¯Ú‹ÛÈ̘ ÛÙË ‰È¿ÁÓˆÛË Ù˘ CJD. ∏ ÂͤٷÛË ÙÔ˘ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘ ˘ÁÚÔ‡ (∂¡À) ÌÔÚ› Ó· ·ÚÔ˘ÛÈ¿ÛÂÈ ÌÈÎÚ‹ ·‡ÍËÛË ÙˆÓ ÚˆÙÂ˚ÓÒÓ, ÂÓÒ Î·Ù¿ Ù· ¿ÏÏ· Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋. ªÂ ÙË ¯Ú‹ÛË Ù·¯¤ˆÓ Ú·‰ÈÔ·ÓÔÛÔÏÔÁÈÎÒÓ ÂÍÂÙ¿ÛÂˆÓ ¤¯Ô˘Ó ·ÔÌÔÓˆı› ÛÙÔ ∂¡À ·ÚÎÂÙ¤˜ Ê˘ÛÈÔÏÔÁÈΤ˜ ÚˆÙ½Ó˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, ÔÈ Ôԛ˜ ‰ÂÓ ·Ó¢ڛÛÎÔÓÙ·È Ê˘ÛÈÔÏÔÁÈο ÛÙÔ ∂¡À Î·È ¯ÚËÛÈÌÂ‡Ô˘Ó ÛÙË ‰È¿ÁÓˆÛË Ù˘ CJD ÚÈÓ ÙÔ ı¿Ó·ÙÔ (9). ∏ ÛËÌ·ÓÙÈÎfiÙÂÚË ·fi ·˘Ù¤˜ ÙȘ ÚˆÙ½Ó˜ Â›Ó·È Ë 14-3-3, ÂÓÒ ‰ÂÓ ·Ó¢ڛÛÎÂÙ·È Ë ÚˆÙ½ÓË PrPCJD. ∏ ÚˆÙ½ÓË 14-3-3 ¤¯ÂÈ ·Ó¢ÚÂı› Î·È Û ¿ÏϘ Ó¢ÚÔÏÔÁÈΤ˜ ηٷÛÙ¿ÛÂȘ, fiˆ˜ ÛÙÔ ∂¡À ·ÛıÂÓÒÓ Ì ÔÍ›· ÈÔÁÂÓ‹ ÌËÓÈÁÁÔÂÁÎÂÊ·Ï›Ùȉ· ‹ Û ·ÛıÂÓ›˜ Ì ÎÚ·ÓÈÔÂÁÎÂÊ·ÏÈΤ˜ ηÎÒÛÂȘ. ∆Ô ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ·, ÛÙËÓ ·Ú¯È΋ Ê¿ÛË Ù˘ ÎÏ·ÛÈ΋˜ CJD, Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi ‹ ·ÚÔ˘ÛÈ¿˙ÂÈ ÌË ÂȉÈο ¯·ÌËÏ¿ ·̷ٷ, ÂÓÒ ÛÙ·‰È·Î¿ ÂÍÂÏ›ÛÛÂÙ·È Û ÂÚÈÔ‰È΋ ÂÌÊ¿ÓÈÛË ‰ÈÊ·ÛÈÎÒÓ ‹ ÙÚÈÊ·ÛÈÎÒÓ Û˘ÌÏÂÁÌ¿ÙˆÓ ·È¯ÌËÚÒÓ Î˘Ì¿ÙˆÓ ÛÙ· 2/3 ÙˆÓ ·ÛıÂÓÒÓ (9). ™Â ϤÔÓ ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È· Ù˘ CJD, ÛÙËÓ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ·Ú·ÙËÚÂ›Ù·È ÁÂÓÈÎÂ˘Ì¤ÓË ·ÙÚÔÊ›·, ÂÓÒ ÛÙË Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ·Ú·ÙËÚÔ‡ÓÙ·È ˘„ËÏ‹˜ ¤ÓÙ·Û˘ Û‹Ì·Ù· ÛÙ· ‚·ÛÈο Á¿ÁÁÏÈ· (9). ∆· Â˘Ú‹Ì·Ù· ·˘Ù¿ ‰È·ÈÛÙÒÓÔÓÙ·È ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ·ÛıÂÓÒÓ Û ÚÔ¯ˆÚË̤ÓÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘. ¡Â˘ÚÔ·ıÔÏÔÁÔ·Ó·ÙÔÌÈο, ÔÈ ÂÁΤʷÏÔÈ ÙˆÓ ·ÛıÂÓÒÓ Ì CJD Ì·ÎÚÔÛÎÔÈο Û˘Ó‹ıˆ˜ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Ô˘ÛÈ·ÛÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ, ÂÓÒ Û ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘ ·Ú·ÙËÚÂ›Ù·È ‰È·ÊfiÚÔ˘ ‚·ıÌÔ‡ ·ÙÚÔÊ›· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, Ô ÔÔ›Ô˜ ˙˘Á›˙ÂÈ ÌÂٷ͇ 850-1000 gr. ªÂ ÙË ÌÈÎÚÔÛÎÔÈ΋ ÂͤٷÛË ‰ÂÈÁÌ¿ÙˆÓ ·fi ‚ÈÔ„›· ÂÁÎÂÊ¿ÏÔ˘ ·fi ·ÓıÚÒÔ˘˜ Ì fiϘ ÙȘ ÌÔÚʤ˜ CJD ‰È·ÈÛÙÒÓÔÓÙ·È ÛÔÁÁÒ‰ÂȘ ·ÏÏÔÈÒÛÂȘ Ô˘ Û˘Óԉ‡ÔÓÙ·È ·fi ·ÒÏÂÈ· Ó¢ÚÒÓˆÓ Î·È ÁÏÔ›ˆÛË (13). OÈ ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ·ıÔÏÔÁÔ·Ó·ÙÔÌÈΤ˜ ·ÏÏÔÈÒÛÂȘ Ù˘ Ó¤·˜ ÛÔÚ·‰È΋˜ ÌÔÚÊ‹˜ CJD Â›Ó·È ÔÈ ‰È¿Û·ÚÙ˜ ϿΘ ·Ì˘ÏÔÂȉԇ˜ ÛÙË Ê·È¿ Ô˘Û›· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È È‰È·›ÙÂÚ· Ù˘ ·ÚÂÁÎÂÊ·Ï›‰·˜, Ô˘ ÂÚÈ‚¿ÏÏÔÓÙ·È ·fi ÎÂÓÔÙÔÈÒ‰ÂȘ ‚Ï¿‚˜. ÷ڷÎÙËÚÈÛÙÈ΋ Â›Ó·È Î·È Ë ·ÓÙȉڷÛÙÈ΋ ·ÛÙÚÔ·ÙÙˆÛË. ∏
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¶·È‰È·ÙÚÈ΋ 2001;64:518-526
Ï¢΋ Ô˘Û›· ·Ú·Ì¤ÓÂÈ ·Ó¤·ÊË. OÈ ÛÔÁÁÈÒ‰ÂȘ ‚Ï¿‚˜ Â›Ó·È ÈÔ ¤ÓÙÔÓ˜ ÛÙ· ‚·ÛÈο Á¿ÁÁÏÈ· Î·È ÙÔ ı¿Ï·ÌÔ (13). ∏ ‰È¿ÁÓˆÛË Ù˘ CJD ‰È¢ÎÔχÓÂÙ·È ·fi ÙË ‰È·›ÛÙˆÛË Ù˘ ‡·Ú͢ ÙˆÓ ÚˆÙÂ˚ÓÒÓ PrP ÛÙÔ ·Ú¤Á¯˘Ì· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Ì ÙË ¯Ú‹ÛË ·ÓÔÛÔ˚ÛÙÔ¯ËÌÈÎÒÓ ÌÂıfi‰ˆÓ. ∂ÈϤÔÓ, ÌÔÚ› Ó· ·Ó¢ÚÂıÔ‡Ó Ï¿Î˜ PrP, ·Ó¿ÏÔÁ· Ì ÙÔ Â›‰Ô˜ Ù˘ ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜. ™ÙË Ó¤· ÛÔÚ·‰È΋ ÌÔÚÊ‹, ¯·Ú·ÎÙËÚÈÛÙÈÎfi ‡ÚËÌ· ·ÔÙÂÏÔ‡Ó ÔÈ “·ÓıÔÛÙfiÏÈÛÙ˜” ϿΘ, ‰ËÏ·‰‹ ϿΘ ·Ì˘ÏÔÂȉԇ˜ ‰›ÎËÓ ·Óı¤ˆÓ, Ô˘ ÂÚÈ‚¿ÏÏÔÓÙ·È ·fi ÎÂÓÔÙfiÈ· (12). ∏ ‚ÈÔ„›· ·Ì˘Á‰·ÏÒÓ Â›Ó·È ¯Ú‹ÛÈÌË ÛÙË ‰È¿ÁÓˆÛË Ù˘ Ó¤·˜ ÛÔÚ·‰È΋˜ ÌÔÚÊ‹˜ Ù˘, ‰ÈfiÙÈ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË Ù˘ ÚˆÙ½Ó˘ PrPCJD (12). ™ÙË Û‡Á¯ÚÔÓË ‚È‚ÏÈÔÁÚ·Ê›· ‰ÂÓ ·Ó·Ê¤ÚÂÙ·È ¤ˆ˜ Û‹ÌÂÚ· ̤ıÔ‰Ô˜ ÂÚÁ·ÛÙËÚȷ΋˜ ‰È·›ÛÙˆÛ˘ Ù˘ ÓfiÛÔ˘ ÛÙÔ ·›Ì· ·ÛıÂÓÒÓ Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙË Ó¤· ÛÔÚ·‰È΋ ÌÔÚÊ‹ CJD. π·ÙÚÔÁÂÓ‹˜ ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ ∏ ¯ÔÚ‹ÁËÛË ·ÓıÚÒÈÓ˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ (¶›Ó·Î·˜ 3), ÚÔÂÚ¯fiÌÂÓ˘ ·fi Âί˘Ï›ÛÌ·Ù· و̷ÙÈÎÒÓ ˘ÔʇÛˆÓ, ÚÔοÏÂÛ ٷ ÚÒÙ· ÎÚÔ‡ÛÌ·Ù· Ù˘ ÓfiÛÔ˘ ÙÔ 1985 (14), Ì ·ÔÙ¤ÏÂÛÌ· Ó· ·ÔÛ˘Úı› ·ÁÎÔÛÌ›ˆ˜ ·fi ÙËÓ Î˘ÎÏÔÊÔÚ›· Î·È Ó· ·ÓÙÈηٷÛÙ·ı› ·fi ÙË ‚ÈÔÛ˘ÓıÂÙÈ΋ ·˘ÍËÙÈ΋ ÔÚÌfiÓË, ·Ú·Û΢·˙fiÌÂÓË Ì ÙËÓ Ù¯ÓÈ΋ ÙÔ˘ ·Ó·Û˘Ó‰˘·Ṳ̂ÓÔ˘ DNA. ∂›Û˘, ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È Î·È ÔÈ ·ÓıÚÒÈÓ˜ ÁÔÓ·‰ÔÙÚÔ›Ó˜ ÁÈ· ÙË ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ (14). °È· ÙËÓ Èı·ÓfiÙËÙ· ÌÂÙ¿‰ÔÛ˘ Ù˘ CJD ̤ۈ ÌÂÙ·ÌÔۯ‡ÛˆÓ, ÌÂÙ¿ÁÁÈÛ˘ ·›Ì·ÙÔ˜ Î·È ·Ú·ÁÒÁˆÓ Â›Ó·È ÁÓˆÛÙ¿ Ù· ÂÍ‹˜: Œ¯Ô˘Ó ·Ó·ÊÂÚı› 4 ÂÚÈÙÒÛÂȘ CJD ·fi ÌÂÙ¿ÁÁÈÛË ·›Ì·ÙÔ˜, Ì›· ÌÂÙ¿ ·fi ¤Á¯˘ÛË ·Ï‚Ô˘Ì›Ó˘, Ì›· ÌÂÙ¿ ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ‹·ÙÔ˜ Î·È ‡ÛÙÂÚ· ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ÎÂÚ·ÙÔÂȉԇ˜ (15,16). ∂›Û˘, ·Ó·Ê¤ÚÂÙ·È ÂÚ›ÙˆÛË CJD ÌÂÙ¿ ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ÛÎÏËÚ¿˜ Ì‹ÓÈÁÁ·˜ (14). ∂ÍÂÙ¿˙ÂÙ·È, ›Û˘, ÙÔ ÂӉ¯fiÌÂÓÔ ÌÂÙ¿‰ÔÛ˘ Ù˘ ÓfiÛÔ˘ ·fi ηıÂÙ‹Ú˜ ·ÁÁÂÈÔÏ·ÛÙÈ΋˜, ‚ËÌ·ÙÔ‰fiÙ˜, ηıÒ˜ Î·È Ì ٷ Ú¿ÌÌ·Ù· catgut. ¢ÂÓ ¤¯ÂÈ ·Ó·ÊÂÚı› ÂÚ›ÙˆÛË ·fi ¯ÔÚ‹ÁËÛË ·Ú¿ÁÔÓÙ· VIII ‹ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ, ·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Û˘ÌÌÂÙ¤¯Ô˘Ó 20.000-60.000 ·ÈÌÔ‰fiÙ˜ ÛÙË ¶›Ó·Î·˜ 3. π·ÙÚÔÁÂÓ‹˜ ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ ñ ÃÔÚ‹ÁËÛË ·ÓıÚÒÈÓ˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ ·fi و̷ÙÈΤ˜ ˘ÔʇÛÂȘ ñ ªÂÙ¿ÁÁÈÛË ·›Ì·ÙÔ˜ Î·È ·Ú·ÁÒÁˆÓ (·Ï‚Ô˘Ì›Ó˘, ·Ú·ÁfiÓÙˆÓ ‹Íˆ˜, ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ) ñ ∂·Ó·¯ÚËÛÈÌÔÔ›ËÛË Î·ıÂÙ‹ÚˆÓ ·ÁÁÂÈÔÏ·ÛÙÈ΋˜ ‹ ‚ËÌ·ÙÔ‰ÔÙÒÓ ñ ªÂÙ·ÌfiÛ¯Â˘ÛË ‹·ÙÔ˜, ÎÂÚ·ÙÔÂȉÒÓ ‹ ÛÎÏËÚ¿˜ Ì‹ÓÈÁÁ·˜
522
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‰ÂÍ·ÌÂÓ‹ Ï¿ÛÌ·ÙÔ˜, ·fi ÙËÓ ÔÔ›· ÊÙÈ¿¯ÓÔÓÙ·È ÔÈ Û˘Ì˘Îӈ̤ÓÔÈ ·Ú¿ÁÔÓÙ˜ Î·È Ë Èı·ÓfiÙËÙ· ÌfiÏ˘ÓÛ˘ Â›Ó·È ˘„ËÏ‹ (17). ø˜ ̤ÛÔ ÚÔÛÙ·Û›·˜ ÙˆÓ ÌÂÙ·ÁÁÈ˙fiÌÂÓˆÓ, ÙÔ FDA (US Food and Drug Administration) ¤¯ÂÈ ÚÔÙ›ÓÂÈ ÙËÓ ·fiÛ˘ÚÛË fiÏˆÓ ÙˆÓ ÔÛÔÙ‹ÙˆÓ ·›Ì·ÙÔ˜ ‹ ·Ú·ÁÒÁˆÓ (Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤ÓÔ˘ Î·È ÙÔ˘ Ï¿ÛÌ·ÙÔ˜ ‰ÔÙÒÓ, ÚÈÓ ·˘Ùfi ÙÔÔıÂÙËı› Û ‰ÂÍ·ÌÂÓ¤˜ ÁÈ· ÙËÓ ·Ú·ÁˆÁ‹ ÚÔ˚fiÓÙˆÓ ·›Ì·ÙÔ˜), Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ¿ÙÔÌ· Ù· ÔÔ›· ·ÚÁfiÙÂÚ· ·Ó¤Ù˘Í·Ó ÛÔÚ·‰È΋ CJD ‹ Ó¤· ÛÔÚ·‰È΋ CJD, fiˆ˜ Î·È ·fi ·ÈÌÔ‰fiÙ˜ Ô˘ ¤¯Ô˘Ó ÂÎÙÂı› ÛÙÔÓ Î›Ó‰˘ÓÔ Ó· ·Ó·Ù‡ÍÔ˘Ó CJD. ∆· ̤ÙÚ· Â›Ó·È ‰Ú·ÛÙÈο, ‰ÈfiÙÈ ÌÂÙ¿ ÙËÓ ¤ÎıÂÛË ÛÙÔ ÏÔÈÌÔÁfiÓÔ ·Ú¿ÁÔÓÙ·, ‰ÂÓ ˘¿Ú¯Ô˘Ó ÚÔ˜ ÙÔ ·ÚfiÓ ·ÍÈfiÈÛÙÔÈ ÙÚfiÔÈ ÁÈ· Ó· ·ÚÂÌÔ‰ÈÛÙ› Ë Â¤ÎÙ·ÛË Ù˘ ÓfiÛÔ˘, Ô‡Ù ˘¿Ú¯ÂÈ ·ÍÈfiÈÛÙË ÂÚÁ·ÛÙËÚȷ΋ ̤ıÔ‰Ô˜ ÁÈ· Ó· ·ÓȯÓ¢ı› Ë ÓfiÛÔ˜ ÚÈÓ ÙËÓ ¤Ó·ÚÍË ÙˆÓ Û˘Ìو̿وÓ. ªÂÙ¿‰ÔÛË Ù˘ ™∂µ ‰È¿ Ù˘ ÙÚÔÊÈ΋˜ Ô‰Ô‡ °È· ÙË ÌÂÙ¿‰ÔÛË Ù˘ ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ ÙˆÓ ‚ÔÔÂȉÒÓ ÛÙÔÓ ¿ÓıÚˆÔ ÂÓÔ¯ÔÔÈÂ›Ù·È Ë ‚ÚÒÛË ‚Ô‰ÈÓÔ‡ ÎÚ¤·ÙÔ˜ ˙ÒÔ˘ ¿Û¯ÔÓÙÔ˜ ·fi ÙË ÓfiÛÔ. O ·Ú¿ÁÔÓÙ·˜ ™∂µ ‰ÂÓ ·ÓȯÓ‡ÂÙ·È Û ÛËÌ·ÓÙÈΤ˜ ÔÛfiÙËÙ˜ ÛÙÔ˘˜ ÈÛÙÔ‡˜ ÙˆÓ ‚ÔÔÂȉÒÓ, fiˆ˜ ·ÓȯÓ‡ÂÙ·È Ô ·Ú¿ÁÔÓÙ·˜ Ù˘ ÓfiÛÔ˘ scrapie ÛÙ· Úfi‚·Ù· (¶›Ó·Î·˜ 4). ªÂϤÙ˜ Û ÈÛÙÔ‡˜ ·ÁÂÏ¿‰ˆÓ Ô˘ ¤¯Ô˘Ó ÌÔÏ˘Óı› Ì ÙÔÓ ·Ú¿ÁÔÓÙ· ™∂µ ¤‰ÂÈÍ·Ó ÌÔÏ˘ÛÌ·ÙÈÎfiÙËÙ· ÌfiÓÔ ÛÙÔÓ ÂÁΤʷÏÔ, ÙÔ ÓˆÙÈ·›Ô Ì˘ÂÏfi, ÙÔÓ ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹ Î·È ÙÔÓ ÂÈÏÂfi, 6-18 Ì‹Ó˜ ÌÂÙ¿ ÙËÓ Â·Ê‹ Ì ÙË ÓfiÛÔ. ∂›Û˘, ÌÂÏÂÙ‹ıËÎ·Ó ÛÏ‹Ó·˜ Î·È ÏÂÌÊ·‰¤Ó˜ ‚ÔÔÂȉÒÓ 40 ‚‰ÔÌ¿‰Â˜ ÌÂÙ¿ ÙË ÌfiÏ˘ÓÛ‹ ÙÔ˘˜ ·fi ÙÔÓ ·Ú¿ÁÔÓÙ· ™∂µ Î·È ‰ÂÓ ·ÓȯÓ‡ıËÎ·Ó ÌÔÏ˘ÛÌ·ÙÈÎÔ› ·Ú¿ÁÔÓÙ˜. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ ÔÈ Ù›ÙÏÔÈ ÙÔ˘ ÌÔÏ˘ÛÌ·ÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ™∂µ Â›Ó·È Ôχ ¯·ÌËÏÔ› ÛÙÔ˘˜ ÈÛÙÔ‡˜ ·˘ÙÔ‡˜ Î·È ·Ó·Ì¤ÓÂÙ·È fiÙÈ ÛÙÔ˘˜ Ì˘˜ Î·È ÙÔ Á¿Ï· ı· Â›Ó·È Ôχ ¯·ÌËÏfiÙÂÚÔÈ (18). ∂ÎÙfi˜ ·fi Ù· ‚ÔÔÂȉ‹, ÂÈÚ¿Ì·Ù· Ô˘ ¤ÁÈÓ·Ó Û ¿ÏÏ· ›‰Ë ˙ÒˆÓ ¤‰ÂÈÍ·Ó fiÙÈ fiÙ·Ó Ô ·Ú¿ÁÔÓÙ·˜ ™∂µ ¯ÔÚËÁÂ›Ù·È ·ÚÂÓÙÂÚÈο ÁÈ· ÂÈÚ·Ì·ÙÈÎÔ‡˜ ÛÎÔÔ‡˜ Û Úfi‚·Ù·, ηÙۛΘ (19) Î·È ¯Ô›ÚÔ˘˜ (20), ·˘Ù¿ ÌÔχÓÔÓÙ·È Î·È ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÛÔÁÁÒ‰Ë ÂÁÎÂÊ·ÏÔ¿ıÂÈ·, Ì ÂÍ·›ÚÂÛË Ù· ÎÔÙfiÔ˘Ï·, Ù· ÔÔ›· 3 ¯ÚfiÓÈ· ÌÂÙ¿ ÙËÓ Â·Ê‹ Ì ÙË ÓfiÛÔ, ‰ÂÓ ÓfiÛËÛ·Ó. ∂›Û˘, ¯ÔÚËÁÒÓÙ·˜ ˆÌ¿ Ì˘·Ï¿ ·Û¯fiÓÙˆÓ ‚ÔÔÂȉÒÓ Û Úfi‚·Ù·, ηÙۛΘ Î·È ¯Ô›ÚÔ˘˜, ÌÂÙ·‰fiıËÎÂ Ë ÓfiÛÔ˜ Û ·˘Ù¿, ÂÓÒ ‰ÂÓ ÌÂÙ·‰fiıËΠÛÙ· ÎÔÙfiÔ˘Ï· (21). ¶·Ú¿ ÙËÓ ÂÈÙ˘¯‹ ÂÈÚ·Ì·ÙÈ΋ ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ ™∂µ Û Úfi‚·Ù·, ηÙۛΘ Î·È ¯Ô›ÚÔ˘˜, ·˘Ùfi ‰ÂÓ Û˘Ó¿ÁÂÙ·È ÙË ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ ‰È¿ ÙˆÓ ˙ˆÔÙÚÔÊÒÓ, Ô‡ÙÂ
¶·È‰È·ÙÚÈ΋ 2001;64:518-526
Paediatriki 2001;64:518-526
¶›Ó·Î·˜ 4. µ·ıÌfi˜ ÌÂÙ·‰ÔÙÈÎfiÙËÙ·˜ ÓfiÛÔ˘ ·fi ‰È¿ÊÔÚÔ˘˜ ÈÛÙÔ‡˜ µ·ıÌfi˜ ÌÂÙ·‰ÔÙÈÎfiÙËÙ·˜ +++
++
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ªË ·ÓȯÓ‡ÛÈÌÔ
πÛÙÔ› ÚÔ‚¿ÙˆÓ ÌÂ ÓfiÛÔ Scrapie
πÛÙÔ› ‚ÔÔÂȉÒÓ Ì BSE
∂ÁΤʷÏÔ˜, ÓˆÙÈ·›Ô˜ Ì˘ÂÏfi˜
∂ÁΤʷÏÔ˜, ÓˆÙÈ·›Ô˜ Ì˘ÂÏfi˜, ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹˜
∂ÈÏÂfi˜, ÏÂÌÊ·‰¤Ó˜, ·ÓÈfiÓ ÎfiÏÔÓ, ÛÏ‹Ó·˜, ·Ì˘Á‰·Ï¤˜
∂ÈÏÂfi˜
πۯȷÎfi Ó‡ÚÔ, ı‡ÌÔ˜, Ó‡ÌÔÓ·˜, ¿ÁÎÚ·˜, ÓˆÙÈ·›Ô˜ Ì˘ÂÏfi˜, ηÙÈfiÓ ÎfiÏÔÓ £ÚfiÌ‚ÔÈ ·›Ì·ÙÔ˜, ηډ›·, ÓÂÊÚfi˜, Ì·˙ÈÎfi˜ ·‰¤Ó·˜, ÛÎÂÏÂÙÈÎÔ› ̇˜, Á¿Ï·, ÔÚfi˜, fiگȘ
˘¿Ú¯Ô˘Ó ÂȉËÌÈÔÏÔÁÈο ‰Â‰Ô̤ӷ Ô˘ Ó· ÂȂ‚·ÈÒÓÔ˘Ó ÙÔ ÁÂÁÔÓfi˜ ·˘Ùfi. °È· ÙËÓ ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛË Ù˘ ÌÂÙ¿‰ÔÛ˘ Ù˘ ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ Ì ÙȘ ˙ˆÔÙÚÔʤ˜ Û Úfi‚·Ù·, ηÙۛΘ Î·È ¯Ô›ÚÔ˘˜, ¯ÔÚËÁ‹ıËÎ·Ó Û ·˘Ù¿ ˙ˆÔÙÚÔʤ˜ Ì ˘„ËÏ‹ Û˘ÁΤÓÙÚˆÛË ÙÔ˘ ·Ú¿ÁÔÓÙ· ™∂µ. ª¤¯ÚÈ Û‹ÌÂÚ·, ‰ËÏ·‰‹ ÂÙ¿ ¯ÚfiÓÈ· ÌÂÙ¿ ÙË ÌfiÏ˘ÓÛ‹ ÙÔ˘˜, Ù· ˙Ò· ·˘Ù¿ ‰ÂÓ ¤¯Ô˘Ó ÂÌÊ·Ó›ÛÂÈ Û˘ÌÙÒÌ·Ù· ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜. §fiÁˆ fï˜ ÙÔ˘ ÌÂÁ¿ÏÔ˘ ¯ÚfiÓÔ˘ ÂÒ·Û˘ Ù˘ ÓfiÛÔ˘, Ù· ·ÔÙÂϤÛÌ·Ù· ·˘Ù¿ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ıˆÚËıÔ‡Ó ÔÚÈÛÙÈο Î·È Ë ·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ ˙ÒˆÓ Û˘Ó¯›˙ÂÙ·È. ŸÛÔÓ ·ÊÔÚ¿ ÙÔ Á¿Ï·, ıˆÚÂ›Ù·È ·ÛʷϤ˜ ̤¯ÚÈ ÛÙÈÁÌ‹˜, ‰ÈfiÙÈ ·fi ÂÈÚ¿Ì·Ù· Û ÔÓÙ›ÎÈ· Ô˘ ÙÔ˘˜ ¯ÔÚËÁ‹ıËΠ› Ì·ÎÚfiÓ Î·È Û ÌÂÁ¿Ï˜ ÔÛfiÙËÙ˜ Á¿Ï· ÌÔÏ˘ÛÌ¤ÓˆÓ ‚ÔÔÂȉÒÓ, ‰ÂÓ ‚Ú¤ıËÎ·Ó ÎÏÈÓÈο Û˘ÌÙÒÌ·Ù· ‹ ÈÛÙÔÏÔÁÈο Â˘Ú‹Ì·Ù· Ù˘ ÓfiÛÔ˘ (22). ∂›Û˘, ·ÌÓÔ› Ô˘ ı‹Ï·Û·Ó ·fi ÚÔ‚·Ù›Ó˜ ÌÔÏ˘Ṳ̂Ó˜ ÂÈÚ·Ì·ÙÈο Ì ÙÔÓ ·Ú¿ÁÔÓÙ· ™∂µ, ‰ÂÓ ÂÌÊ¿ÓÈÛ·Ó Û˘ÌÙÒÌ·Ù· ‹ ÈÛÙÔÏÔÁÈο Â˘Ú‹Ì·Ù· Ù˘ ÓfiÛÔ˘ (22). ™‡ÓÙÔÌ· ı· ÍÂÎÈÓ‹ÛÂÈ ÛÂÈÚ¿ ÂÈÚ·Ì¿ÙˆÓ ·Ó›¯Ó¢Û˘ Ù˘ ÚˆÙ½Ó˘ prion Û Á¿Ï· ‚ÔÔÂȉÒÓ Ô˘ ÌÔχÓıËÎ·Ó ÂÈÚ·Ì·ÙÈο Ì ÙÔÓ ·Ú¿ÁÔÓÙ· ™∂µ. ªË¯·ÓÈÛÌÔ› ÌÂÙ¿‰ÔÛ˘ Ù˘ ÓfiÛÔ˘ ™∂µ ·Ó¿ÌÂÛ· ÛÙ· ‚ÔÔÂȉ‹ °È· ÙË ÌfiÏ˘ÓÛË ÙˆÓ ‚ÔÔÂȉÒÓ ¤¯Ô˘Ó ‰ÈÂÚ¢ÓËı› ‰È¿ÊÔÚÔÈ ·Ú¿ÁÔÓÙ˜ (23), fiˆ˜: ·) Ë ¯Ú‹ÛË ÂÌ‚ÔÏ›ˆÓ, Ê·ÚÌ¿ÎˆÓ ‹ Ê˘ÙÔÊ·Ú̿ΈÓ, ‚) Ë ¿ÌÂÛË Â·Ê‹ ÙˆÓ ‚ÔÔÂȉÒÓ Ì ˙ÒÓÙ· Úfi‚·Ù·, Á) Ë ËÏÈΛ· ÙˆÓ ‚ÔÔÂȉÒÓ, ÙÔ Ê‡ÏÔ, Ë ‰È·Ï·ÎÔ˘ÓÙȷ΋ ÌÂÙ¿‰ÔÛË, Ë ‰È·ÙÚÔÊ‹ ÙˆÓ ÌÔÛ¯·ÚÈÒÓ Ì ıËÏ·ÛÌfi ‹ Ì ˙ˆÔÙÚÔʤ˜. ∞fi fiϘ ÙȘ ·ÓˆÙ¤Úˆ ·Ú·Ì¤ÙÚÔ˘˜, ÌfiÓÔ Ë ‰È·ÙÚÔÊ‹ ÙˆÓ ÌÔÛ¯·ÚÈÒÓ Ì ˙ˆÔÙÚÔʤ˜ ·ÔÙÂÏÔ‡Û ÙÔÓ ÎÔÈÓfi ·Ú¿ÁÔÓÙ·, ˘Â‡ı˘ÓÔ ÁÈ· ÙË ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ ™∂µ ·Ó¿ÌÂ-
πۯȷÎfi Ó‡ÚÔ, ı‡ÌÔ˜, ÓˆÙÈ·›Ô˜ Ì˘ÂÏfi˜, ‹·Ú, Ó‡̈Ó, ¿ÁÎÚ·˜, ηډ›·, ÓÂÊÚfi˜, Ì·˙ÈÎfi˜ ·‰¤Ó·˜, Á¿Ï·, ·›Ì·, ÔÚfi˜, ÛÎÂÏÂÙÈÎÔ› ̇˜, fiگȘ
Û· ÛÙ· ‚ÔÔÂȉ‹. OÈ ˙ˆÔÙÚÔʤ˜ ·˘Ù¤˜ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙËÓ ÂÂÍÂÚÁ·Û›· ÎÚ¿وÓ, Ï›Ô˘˜ Î·È ÔÛÙÒÓ ‚ÔÔÂȉÒÓ ·fi Ù· ÛÊ·Á›· Î·È Ù· ÎÚÂԈϛ·. ∏ ÂȉÈ΋ ÂÂÍÂÚÁ·Û›· ÂÚÈÏ·Ì‚¿ÓÂÈ ·Ó¿ÌÂÈÍË, Û‡ÓıÏÈ„Ë, ‚Ú·ÛÌfi Î·È Ê˘ÁÔΤÓÙÚÈÛË ‹ ·ÎfiÌ· Î·È ÚÔÛı‹ÎË ‰È·Ï˘Ì¿ÙˆÓ ˘‰ÚÔÁÔÓ·ÓıÚ¿ÎˆÓ ÁÈ· Ó· ·Ê·ÈÚÂı› ÙÔ Ï›Ô˜ (23). ∆Ô Â›‰Ô˜ Ù˘ ÂÂÍÂÚÁ·Û›·˜ ÙˆÓ ˙ˆÔÙÚÔÊÒÓ Â›Ó·È ÛËÌ·ÓÙÈÎfi ÁÈ· ÙË ‰È·Ù‹ÚËÛË Ù˘ ÌÔÏ˘ÛÌ·ÙÈÎfiÙËÙ¿˜ ÙÔ˘˜. ŒÙÛÈ, Ë ÌÔÏ˘ÛÌ·ÙÈÎfiÙËÙ· ·˘Í¿ÓÂÙ·È Ì ÙËÓ ÂÂÍÂÚÁ·Û›· ÙÔ˘˜ Ì ˘‰ÚÔÁÔÓ¿ÓıڷΘ Û ÂÚÈÔÚÈṲ̂ÓÔ ‚·ıÌfi, ÒÛÙ ӷ ‰È·ÙËÚÂ›Ù·È ˘„ËÏfi ÔÛÔÛÙfi Ï›Ô˘˜ (7-14%), ÙÔ ÔÔ›Ô ÚÔÊ˘Ï¿ÛÛÂÈ Ù· prions ·fi ÙËÓ Î·Ù·ÛÙÚÔÊ‹ Ì Ì˯·ÓÈο ̤۷ (14). ∂›Û˘, Ë ˘ÁÚ‹ ı¤ÚÌ·ÓÛË ÙˆÓ ˙ˆÔÙÚÔÊÒÓ Â›Ó·È Ï¤ÔÓ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙË Ì›ˆÛË Ù˘ ÌÔÏ˘ÛÌ·ÙÈÎfiÙËÙ·˜ ·fi ÙË ÓfiÛÔ scrapie, Û ۇÁÎÚÈÛË Ì ÙËÓ ÍËÚ‹ ı¤ÚÌ·ÓÛË (14). ∂›Ó·È ÚÔÊ·Ó¤˜ fiÙÈ Ù· ‚ÔÔÂȉ‹ ‹Ù·Ó ¿ÓÙ· ÂÎÙÂıÂÈ̤ӷ ÛÙË ÓfiÛÔ scrapie, ·ÏÏ¿ ÔÈ Ì¤ıÔ‰ÔÈ ÂÂÍÂÚÁ·Û›·˜ ÙˆÓ ˙ˆÔÙÚÔÊÒÓ ÙÚÔÔÔÈ‹ıËÎ·Ó Î·È ¤ÙÛÈ ‰ËÌÈÔ˘ÚÁ‹ıËÎ·Ó ÔÈ Û˘Óı‹Î˜ ÁÈ· ÙË ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ ™∂µ ÛÙ· ‚ÔÔÂȉ‹ (24). ∞Ó·ÊÔÚÈο Ì ÙȘ ¿ÏϘ ·Ú·Ì¤ÙÚÔ˘˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË ÌfiÏ˘ÓÛË ÙˆÓ ‚ÔÔÂȉÒÓ, fiˆ˜ Ë ËÏÈΛ·, ÙÔ Ê‡ÏÔ, Ë ‰È·Ï·ÎÔ˘ÓÙȷ΋ ÌÂÙ¿‰ÔÛË, Ë ‰È·ÙÚÔÊ‹ ÙˆÓ ÌÔÛ¯·ÚÈÒÓ, ·Ó·Ê¤ÚÔÓÙ·È Ù· ÂÍ‹˜: Ë Û˘¯ÓfiÙËÙ· Ù˘ ÓfiÛÔ˘ ™∂µ ·Ó¿ÌÂÛ· ÛÙ· ‚ÔÔÂȉ‹ Â›Ó·È ÌˉÂÓÈ΋ Û ÌÔÛ¯¿ÚÈ· ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 2 ÂÙÒÓ (18), ÂÓÒ Ë Ì¤ÁÈÛÙË Û˘¯ÓfiÙËÙ· ·Ú·ÙËÚÂ›Ù·È Û ËÏÈ˘ 4 Î·È 5 ÂÙÒÓ (™¯‹Ì· 2). ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ÂÚÌËÓ‡ÂÙ·È ˆ˜ ÂÍ‹˜: Ë ‰È·ÙÚÔÊ‹ ÙˆÓ ÌÔÛ¯·ÚÈÒÓ ÁÈ· ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ˙ˆ‹˜ Á›ÓÂÙ·È ·ÔÎÏÂÈÛÙÈο Ì Á¿Ï·. ∂¿Ó ÙÔ ÌÔÛ¯¿ÚÈ ÚÔ¤Ú¯ÂÙ·È ·fi ÌË Á·Ï·ÎÙÔÊfiÚ· ·ÁÂÏ¿‰·, ÙfiÙ ıËÏ¿˙ÂÈ ·ÔÎÏÂÈÛÙÈο. ∂¿Ó, ·ÓÙÈı¤Ùˆ˜, Ë ·ÁÂÏ¿‰· Â›Ó·È Á·Ï·ÎÙÔÊfiÚ· (Ù˘ ÔÔ›·˜ ÙÔ Á¿Ï· ÚÔÔÚ›˙ÂÙ·È ÁÈ· ÂÎÌÂÙ¿ÏÏ¢ÛË), ÙfiÙ ¯ÔÚËÁÂ›Ù·È ÛÙÔ ÌÔÛ¯¿ÚÈ Á¿Ï·
523
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ÎÔÓÈÔÔÈË̤ÓÔ ‹ Î·È ÏÔ‡ÛÈ· Û ڈÙ½Ó˜ Û˘Ì˘Îӈ̤ÓË ˙ˆÔÙÚÔÊ‹ Û ÔÛÔÛÙfi 5%. ŒÙÛÈ, Ë Û˘¯ÓfiÙËÙ· Ù˘ ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ ÙˆÓ ‚ÔÔÂȉÒÓ ÛÙ· ÎÔ¿‰È· Á·Ï·ÎÙÔÊfiÚˆÓ ·ÁÂÏ¿‰ˆÓ Ô˘ ‰ÂÓ ı‹Ï·˙·Ó Ù· ÌÔÛ¯¿ÚÈ· ÙÔ˘˜ ·Ó·Ê¤ÚıËΠÛÙË ª. µÚÂÙ·Ó›· Û ÔÛÔÛÙfi 45,6% (πÔ‡ÏÈÔ˜ 1993) Î·È ‹Ù·Ó ÛËÌ·ÓÙÈο ·˘ÍË̤ÓË Û ۇÁÎÚÈÛË Ì ÂΛÓË ÙˆÓ ÌË Á·Ï·ÎÙÔÊfiÚˆÓ ·ÁÂÏ¿‰ˆÓ Ô˘ ı‹Ï·˙·Ó Ù· ÌÔÛ¯¿ÚÈ· ÙÔ˘˜ (9,9%) (21). ¢Â‰Ô̤ÓÔ˘ fiÙÈ ÙÔ ÌÔÛ¯¿ÚÈ ÙÚ¤ÊÂÙ·È ·ÔÎÏÂÈÛÙÈο Ì Á¿Ï· ÁÈ· ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ˙ˆ‹˜ Î·È Ô ¯ÚfiÓÔ˜ ÂÒ·Û˘ Ù˘ ÓfiÛÔ˘ ÛÙ· ‚ÔÔÂȉ‹ Â›Ó·È 4-5 ¯ÚfiÓÈ·, Ë Ì¤ÁÈÛÙË Û˘¯ÓfiÙËÙ· Ù˘ ™∂µ ÛÙ· ‚ÔÔÂȉ‹ ÂÌÊ·Ó›˙ÂÙ·È Û ˙Ò· 4-5 ÂÙÒÓ, ÂÓÒ ÛÙ· ÌÈÎÚfiÙÂÚ· ÙˆÓ 2 ÂÙÒÓ ÌÔÛ¯¿ÚÈ· Â›Ó·È ÌˉÂÓÈ΋ (™¯‹Ì· 2). ∞Ó·ÊÔÚÈο Ì ÙÔ Ê‡ÏÔ ÙˆÓ ·Û¯fiÓÙˆÓ ‚ÔÔÂȉÒÓ, Ë ÏÂÈÔ„ËÊ›· Â›Ó·È ·ÁÂÏ¿‰Â˜, ‰ÈfiÙÈ Ù· ‚fi‰È· Ô˘ ÚÔÔÚ›˙ÔÓÙ·È ÁÈ· ÎÚ·ÙÔ·Ú·ÁˆÁ‹ ı·Ó·ÙÒÓÔÓÙ·È Û ËÏÈΛ· 12-14 ÌËÓÒÓ, ÂÓÒ ÂΛӷ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ·Ó··Ú·ÁˆÁ‹, ı·Ó·ÙÒÓÔÓÙ·È Û ËÏÈΛ· 2 ÂÙÒÓ (21). ∏ ‰È·Ï·ÎÔ˘ÓÙȷ΋ ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ scrapie ÛÙ· Úfi‚·Ù· (25) Â›Ó·È ÛËÌ·ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ οıÂÙ˘ ·ÏÏ¿ Î·È ÔÚÈ˙fiÓÙÈ·˜ ÌÂÙ¿‰ÔÛ˘ Ù˘ ÓfiÛÔ˘ (‰È¿ Ù˘ ‚ÚÒÛ˘ ÙÔ˘ Ï·ÎÔ‡ÓÙ· ·fi ¿ÏÏ· ˙Ò·). ™ÙËÓ ÂÚ›ÙˆÛË ·˘Ù‹, ÙÔ fiÚÁ·ÓÔ ÌÂÙ¿‰ÔÛ˘ Â›Ó·È Ô Ï·ÎÔ‡ÓÙ·˜. OÈ Pattison Î·È Û˘Ó (25) ¤‰ÂÈÍ·Ó fiÙÈ ¯ÔÚ‹ÁËÛË Ï·ÎÔ‡ÓÙ· ·Û¯fiÓÙˆÓ ÚÔ‚¿ÙˆÓ Û ˘ÁÈ‹ ‰È¿ Ù˘ ÙÚÔÊÈ΋˜ Ô‰Ô‡ ‹ ÔÚÔÏÔÁÈο, ÚÔοÏÂÛ ÙË ÌfiÏ˘ÓÛË ÙˆÓ ÚÔ‚¿ÙˆÓ ·˘ÙÒÓ. ∞ÓÙ›ıÂÙ·, Ë ¯ÔÚ‹ÁËÛË ÔÚÔ‡ ·fi Ï·ÎÔ‡ÓÙ· ·Û¯fiÓÙˆÓ ·ÁÂÏ¿‰ˆÓ Û ˘ÁÈ›˜ ·ÁÂÏ¿‰Â˜, ‰ÂÓ ÚÔοÏÂÛ ÙË ÓfiÛÔ ÌÂÙ¿ 3,5 ¤ÙË ·fi ÙËÓ ÚfiÎÏËÛË. ∂›Û˘, ‚ÔÔÂȉ‹ Ô˘ ı·Ó·ÙÒıËÎ·Ó 2 ¤ÙË ÌÂÙ¿ ÙËÓ ÚfiÎÏËÛË ‰ÂÓ ·ÚÔ˘Û›·Û·Ó ÈÛÙÔÏÔÁÈΤ˜ ‚Ï¿‚˜ Ù˘ ÓfiÛÔ˘. ∏ ÛËÌ·Û›· Ù˘ οıÂÙ˘ ‹ Ù˘ ÔÚÈ-
™¯‹Ì· 2. ∂Ù‹ÛÈ· Û˘¯ÓfiÙËÙ· ™∂µ ÛÙ· ‚ÔÔÂȉ‹ ·Ó¿ ËÏÈΛ· (21).
524
Paediatriki 2001;64:518-526
˙fiÓÙÈ·˜ ÌÂÙ¿‰ÔÛ˘ Ù˘ ÓfiÛÔ˘ scrapie ·Ó¿ÌÂÛ· ÛÙ· Úfi‚·Ù· ‰È¿ Ù˘ ‚ÚÒÛ˘ ÙÔ˘ Ï·ÎÔ‡ÓÙ· ·Û¯fiÓÙˆÓ ˙ÒˆÓ Â›Ó·È ÛËÌ·ÓÙÈ΋ ÁÈ· ÙËÓ ÚfiÎÏËÛË ÂȉËÌÈÒÓ Ù˘ ÓfiÛÔ˘ Û ÎÔ¿‰È· ÚÔ‚¿ÙˆÓ. ∏ ‰È·Ï·ÎÔ˘ÓÙȷ΋ ÌÂÙ¿‰ÔÛË ·fi ÙËÓ ·ÁÂÏ¿‰· ÛÙÔ ÌÔÛ¯¿ÚÈ Â›Ó·È ¤Ó·˜ ·Ú¿ÁÔÓÙ·˜ Ô˘ ÂÍÂÙ¿˙ÂÙ·È ÂÎÙÂÓÒ˜, ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰È·Û·ÊËÓÈÛÙ› Ë ÚԤϢÛË Ù˘ ÂȉËÌ›·˜ Ù˘ ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ ÙˆÓ ‚ÔÔÂȉÒÓ. ¶ÚÔ˜ ÙÔ ·ÚfiÓ ˘¿Ú¯Ô˘Ó ‰‡Ô ÌÂϤÙ˜ Û ÂͤÏÈÍË (21). ∏ ÚÒÙË ÂÚÈÏ·Ì‚¿ÓÂÈ 316 ˙‡ÁË ÌÔÛ¯·ÚÈÒÓ Ô˘ ÌÂÏÂÙÒÓÙ·È Â› 7 ¯ÚfiÓÈ·. ∆Ô Î¿ı ˙‡ÁÔ˜ ·ÔÙÂÏÂ›Ù·È ·fi ¤Ó· ÌÔÛ¯¿ÚÈ Ô˘ ÁÂÓÓ‹ıËΠ·fi ·ÁÂÏ¿‰· ¿Û¯Ô˘Û· ·fi ™∂µ, ÂÓÒ ÙÔ ¿ÏÏÔ ÚÔ¤Ú¯ÂÙ·È ·fi ·ÁÂÏ¿‰· ˘ÁÈ‹. ªÂÙ¿ 5 ¯ÚfiÓÈ· ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÌÂϤÙ˘, ‰È·ÈÛÙÒıËÎ·Ó 10 ÂÚÈÙÒÛÂȘ ™∂µ, ȉȷ›ÙÂÚ· ·Ó¿ÌÂÛ· ÛÙ· ÌÂÁ·Ï‡ÙÂÚ· ÌÔÛ¯¿ÚÈ·. ¶ÚÔ˜ ÙÔ ·ÚfiÓ, ÏÔÈfiÓ, Ë Î¿ıÂÙË ÌÂÙ¿‰ÔÛË ‰ÂÓ Á›ÓÂÙ·È Û ÛËÌ·ÓÙÈÎfi ‚·ıÌfi, ·ÏÏ¿ ̤¯ÚÈ ÙËÓ ÔÏÔÎÏ‹ÚˆÛË Ù˘ ÌÂϤÙ˘ Î·È ÙË ı·Ó¿ÙˆÛË fiÏˆÓ ÙˆÓ ˙ÒˆÓ, ÙËÓ ÈÛÙÔÏÔÁÈ΋ ÌÂϤÙË ÙˆÓ ÂÁÎÂÊ¿ÏˆÓ Î·È ÙËÓ ·ÔΈ‰ÈÎÔÔ›ËÛË ÙˆÓ ÛÙÔȯ›ˆÓ ÙˆÓ ÌËÙ¤ÚˆÓ, ‰ÂÓ ÌÔÚÔ‡Ó Ó· ÂÍ·¯ıÔ‡Ó Û·Ê‹ Û˘ÌÂÚ¿ÛÌ·Ù·. ™ÙË ‰Â‡ÙÂÚË ¤Ú¢ӷ (21), ÌÂÏÂÙ¿Ù·È Ë ÂÙ‹ÛÈ· Û˘¯ÓfiÙËÙ· Ù˘ ™∂µ ÛÙÔ˘˜ ·ÔÁfiÓÔ˘˜ ·Û¯Ô˘ÛÒÓ ·ÁÂÏ¿‰ˆÓ Î·È Û˘ÁÎÚ›ÓÂÙ·È Ì ÙËÓ ·Ó·ÌÂÓfiÌÂÓË ÂÙ‹ÛÈ· Û˘¯ÓfiÙËÙ· ÌÂÙ¿‰ÔÛ˘ ‰È¿ Ù˘ ÙÚÔÊÈ΋˜ Ô‰Ô‡. T· ÚÒÙ· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë Û˘¯ÓfiÙËÙ· ÛÙÔ˘˜ ·ÔÁfiÓÔ˘˜ ÙˆÓ ·ÁÂÏ¿‰ˆÓ Ì ™∂µ ‰ÂÓ ‰È·Ê¤ÚÂÈ ·fi ÂΛÓË Ù˘ ÌÂÙ¿‰ÔÛ˘ ‰È¿ Ù˘ ÙÚÔÊÈ΋˜ Ô‰Ô‡. ™¯ÂÙÈο Ì ÙËÓ ·ÙÚÈ΋ ÌÂÙ¿‰ÔÛË, ‰ÂÓ ‰È·ÈÛÙÒıËΠ·˘ÍË̤ÓÔ˜ ΛӉ˘ÓÔ˜ ÁÈ· ™∂µ ÛÙÔ˘˜ ·ÔÁfiÓÔ˘˜ ·Û¯fiÓÙˆÓ Ù·‡ÚˆÓ ηÈ, Û˘ÓÂÒ˜, Ë ÌÂÙ¿‰ÔÛË ‰È¿ ÙÔ˘ Û¤ÚÌ·ÙÔ˜ Â›Ó·È ·ÌÂÏËÙ¤· (21). ∂¿Ó ÏÔÈfiÓ ıˆÚËı› Ë Î¿ıÂÙË ÌÂÙ¿‰ÔÛË ˆ˜ ÌË ÛËÌ·ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ‰È¿‰ÔÛ˘ ÛÙ· ‚ÔÔÂȉ‹, ÙfiÙÂ Ô ÌfiÓÔ˜ ÛËÌ·ÓÙÈÎfi˜ ÙÚfiÔ˜ ÌÂÙ¿‰ÔÛ˘ Ù˘ ÓfiÛÔ˘ Á›ÓÂÙ·È Ì ÙȘ ˙ˆÔÙÚÔʤ˜. ™Â ·˘Ùfi ÙÔ ÛËÌ›Ô, ϤÔÓ, ÂÓÙ›ÓÔÓÙ·È Ù· ̤ÙÚ· ÁÈ· ÙÔÓ ÂÚÈÔÚÈÛÌfi Ù˘ ‰È¿‰ÔÛ˘ Ù˘ ÓfiÛÔ˘. ªÂÙ·‰ÔÙÈÎfiÙËÙ· Î·È ·ÓÙÔ¯‹ ÙˆÓ prions ∏ ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ ÂÈÚ·Ì·ÙÈο ÂÈÙ˘Á¯¿ÓÂÙ·È Ì ÙË ¯ÔÚ‹ÁËÛË Âί˘ÏÈÛÌ¿ÙˆÓ ÂÁÎÂÊ¿ÏÔ˘ ‹ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡, ÔÊı·ÏÌÒÓ, Ó¢ÌfiÓˆÓ, ‹·ÙÔ˜, ÓÂÊÚÒÓ Î·È ÛÏ‹Ó·, ηıÒ˜ Î·È ÏÂÌÊ·‰¤ÓˆÓ ·fi ¿Û¯ÔÓÙ˜. ∂›Û˘, ∂¡À ·ÛıÂÓÒÓ Ì CJD ÌÔÚ› Ó· ÌÔχÓÂÈ ıËÏ·ÛÙÈο (9). OÈ ÂÎÎÚ›ÛÂȘ ÙˆÓ ·‰¤ÓˆÓ (Á¿Ï·, Û¤ÚÌ·) Î·È ÔÈ ·ÂÎÎÚ›ÛÂȘ (Ô‡Ú·, ÎfiÚ·Ó·, ‰¿ÎÚ˘·, Û¿ÏÈÔ, ȉÚÒÙ·˜) ‰ÂÓ ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ ÌÂÙ·‰›‰Ô˘Ó ÙË ÓfiÛÔ. ™˘ÓÂÒ˜, ‰ÂÓ ¯ÚÂÈ¿˙ÂÙ·È ·ÔÌfiÓˆÛË ÙˆÓ ·ÛıÂÓÒÓ Ì CJD. O ΛӉ˘ÓÔ˜ ÌÂÙ¿‰ÔÛ˘ Û ¿ÙÔÌ· Ù˘ ›‰È·˜ ÔÈÎÔÁ¤ÓÂÈ·˜ ‹ ÛÙÔ ˘ÁÂÈÔÓÔÌÈÎfi ÚÔÛˆÈÎfi Â›Ó·È ¯·ÌËÏfi˜. ÃÚÂÈ¿˙ÂÙ·È, fï˜,
¶·È‰È·ÙÚÈ΋ 2001;64:518-526
ÚÔÛÔ¯‹ ÛÙËÓ Â·Ê‹ Ì ÙÔ ∂¡À ‹ Ù· ÈÛÙÔÏÔÁÈο ·Ú·Û΢¿ÛÌ·Ù· ÙˆÓ ·ÛıÂÓÒÓ Ì CJD (9). °È· ÙËÓ ·Ôχ̷ÓÛË ÙˆÓ ÂÚÁ·Ï›ˆÓ Û˘ÓÈÛÙ¿Ù·È Ë ÙÔÔı¤ÙËÛ‹ ÙÔ˘˜ Û ‰È¿Ï˘Ì· ¡·O∏ >/1¡ ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 1 h Î·È ÛÙË Û˘Ó¤¯ÂÈ· ·ÔÛÙ›ڈÛË ÛÙÔ˘˜ 134ÔC ÁÈ· 1 h. ¶ÚÔÙÈÌ¿Ù·È Ë ¯Ú‹ÛË ÂÚÁ·Ï›ˆÓ Ì›·˜ ¯Ú‹Û˘ Î·È ÙÔ Î¿„ÈÌÔ ÙˆÓ ˘ÏÈÎÒÓ Ô˘ ¤¯Ô˘Ó ÌÔÏ˘Óı› ·fi ÈÛÙÔ‡˜ ·Û¯fiÓÙˆÓ (9). ∆›ıÂÙ·È ÙÔ ÂÚÒÙËÌ· Â¿Ó ÙÂÏÈο ˘¿Ú¯ÂÈ ‹ fi¯È ΛӉ˘ÓÔ˜ ηٿ ÙË ‚ÚÒÛË ÙÔ˘ ‚Ô‰ÈÓÔ‡ ÎÚ¤·ÙÔ˜. ∏ ÌfiÏ˘ÓÛË ÙˆÓ ˙ÒˆÓ ‰È¿ Ù˘ ÙÚÔÊÈ΋˜ Ô‰Ô‡ Â›Ó·È Ôχ ‡ÎÔÏË, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ··ÈÙÂ›Ù·È Ì›· ÎÔ˘Ù·ÏÈ¿ ˙ˆÔÙÚÔÊ‹˜ Ì ˘„ËÏ‹ Û˘ÁΤÓÙÚˆÛË ÙÔ˘ ·Ú¿ÁÔÓÙ· ™∂µ ÁÈ· Ó· ÌÔÏ˘Óı› Ì›· ·ÁÂÏ¿‰· (26). ∏ ÂÏ¿¯ÈÛÙË ‰fiÛË ÌÂÙ¿‰ÔÛ˘ Ù˘ ÓfiÛÔ˘ ÛÙÔÓ ¿ÓıÚˆÔ ‰ÂÓ Â›Ó·È ÁÓˆÛÙ‹. ª¤¯ÚÈ ÙȘ ·Ú¯¤˜ Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ 1990, Ë Î·Ù·Ó¿ÏˆÛË ‚Ô‰ÈÓÔ‡ Ì˘·ÏÔ‡ ‹ ÂÓÙÔÛı›ˆÓ ‹Ù·Ó ÂχıÂÚË Î·È Ì ÙÔÓ ÙÚfiÔ ·˘Ùfi ÌÔÚ› Ó· ‰È·‰fiıËÎ·Ó Ù· prions ÛÙÔ ·ÓıÚÒÈÓÔ ÂÙÈÎfi Û‡ÛÙËÌ·. ∏ ÌÂÙ·‰ÔÙÈÎfiÙËÙ· ‰È¿ Ù˘ ÙÚÔÊÈ΋˜ Ô‰Ô‡ ‰È¢ÎÔχÓÂÙ·È ·fi ÙËÓ ·ÓÙÔ¯‹ ÙˆÓ prions ÛÙȘ Û˘Óı‹Î˜ ÙÔ˘ Ì·ÁÂÈڤ̷ÙÔ˜ Î·È ÛÙÔ˘˜ ·Ì˘ÓÙÈÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜ ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡. ∆· prions ¤¯Ô˘Ó Ôχ ÌÂÁ¿ÏË ·ÓÙÔ¯‹ ÛÙË ı¤ÚÌ·ÓÛË, ÙËÓ ÈÔÓ›˙Ô˘Û· ·ÎÙÈÓÔ‚ÔÏ›·, ÙË ÊÔÚÌfiÏË Î·È, fiˆ˜ ÚԷӷʤÚıËÎÂ, ÛÙȘ ÚˆÙ¿Û˜ Î·È ÚÈ‚ÔÓÔ˘ÎÏ¿Û˜. ∞ÓÙ¤¯Ô˘Ó ÛÙȘ Û˘Ó‹ıÂȘ ıÂÚÌÔÎڷۛ˜ Ì·ÁÂÈڤ̷ÙÔ˜, ÛÙȘ ¯‡ÙÚ˜ ·ÙÌÔ‡ Î·È ÛÙÔ˘˜ ÊÔ‡ÚÓÔ˘˜ ÌÈÎÚÔÎ˘Ì¿ÙˆÓ. ∂›Û˘, ¤¯Ô˘Ó ¯ËÌÈ΋ ·ÓÙÔ¯‹ Î·È ÔÈ ÚˆÙ½Ó˜ ÙÔ˘ Á‡̷ÙÔ˜ Ù· ÚÔÛÙ·ÙÂ‡Ô˘Ó ·fi ÙÔ˘˜ ·Ì˘ÓÙÈÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ (27,28). ∂Ӊ¯Ô̤ӈ˜, ·Ú¿ÁÔÓÙ˜ ÙÔÈÎÔ›, fiˆ˜ ·ÏÎÔfiÏË, ÌË ÛÙÂÚÔÂȉ‹ ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë, ÂËÚ¿˙Ô˘Ó ÙË ‰È·‚·ÙfiÙËÙ· ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ÂÈıËÏ›Ô˘ (26). ∂›Û˘, ÔÈ ·Ì˘ÓÙÈÎÔ› Ì˯·ÓÈÛÌÔ› ÙÔ˘ ÂÓÙ¤ÚÔ˘ ÌÔÚ› Ó· ηٷÛÙ·ÏÔ‡Ó ·fi ÏÔÈÌÒ‰ÂȘ ·Ú¿ÁÔÓÙ˜, fiˆ˜ ÏÔÈÌ҉˘ ÌÔÓÔ˘Ú‹ÓˆÛË, ÈÏ·Ú¿ ‹ influenza, ηٷÛÙ¤ÏÏÔÓÙ·˜ ÙË ‰Ú¿ÛË ÙˆÓ Ì·ÎÚÔÊ¿ÁˆÓ (26). ∆ÂÏÈο, ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÍÔ˘‰ÂÙÂÚˆı› Ô ·Ú¿ÁˆÓ ™∂µ ÛÙ· ˙ˆÈο ÚÔÈfiÓÙ·, ··ÈÙÂ›Ù·È ı¤ÚÌ·ÓÛ‹ ÙÔ˘˜ ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 20 min ÛÙÔ˘˜ 130ÔC Î·È Û ›ÂÛË 3 atm, ÒÛÙ ӷ Â›Ó·È ·ÛÊ·Ï‹ ÁÈ· ÙÔÓ Î·Ù·Ó·ÏˆÙ‹ (29). ™ÙȘ ¯‡ÙÚ˜ Ù·¯‡ÙËÙ·˜ ·Ó·Ù‡ÛÛÂÙ·È ıÂÚÌÔÎÚ·Û›· 115ÔC Î·È ›ÂÛË 1,5 atm. ™˘ÓÂÒ˜, ÔÈ ˘¿Ú¯Ô˘Û˜ ¯‡ÙÚ˜ Ù·¯‡ÙËÙ·˜ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ηٷÛÙÚ¤„Ô˘Ó Ù· prions. ŒÎÙ·ÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ™Â fi,ÙÈ ·ÊÔÚ¿ ÙËÓ ¤ÎÙ·ÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜, ˆ˜ ÙȘ 3/11/2000 ¤¯Ô˘Ó ·Ó·ÎÔÈÓˆı› 77 ÂȂ‚·ÈˆÌ¤Ó· ÂÚÈÛÙ·ÙÈο ·ÛıÂÓÒÓ ·Û¯fiÓÙˆÓ ·fi ÙË Ó¤· ÔÈÎÈÏ›· ÛÔÚ·‰È΋˜ ÌÔÚÊ‹˜ CJD, Ô˘ ¤ı·Ó·Ó ÛÙË ª. µÚÂÙ·Ó›· ÌÂٷ͇ ÙˆÓ ÂÙÒÓ 1995-2000, ÂÓÒ Î·Ó¤Ó· ÂÚÈÛÙ·ÙÈÎfi ‰ÂÓ ·Ó·Ê¤ÚıËΠηٿ ÙË
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‰È¿ÚÎÂÈ· Ù˘ ÂÓÙ·ÂÙ›·˜ 1990-1995 (30,31). ™ÙË µfiÚÂÈ· ∞ÌÂÚÈ΋ ‰ÂÓ ¤¯ÂÈ ‰È·ÈÛÙˆı› η̛· ÂÚ›ÙˆÛË Ó¤·˜ ÛÔÚ·‰È΋˜ CJD, Ô‡Ù ÛÔÁÁÒ‰Ô˘˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜ ‚ÔÔÂȉÒÓ Î·È ‰ÂÓ ·˘Í‹ıËÎÂ Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ÎÏ·ÛÈÎÒÓ ÌÔÚÊÒÓ Ù˘ CJD ηٿ Ù· ÙÂÏÂ˘Ù·›· 20 ¯ÚfiÓÈ· (9). ™ÙË ¯ÒÚ· Ì·˜, fiˆ˜ ÚÔ·ÙÂÈ ·fi ÛÙÔȯ›· ·fi ÙËÓ ¤‰Ú· Ù˘ ∂ȉËÌÈÔÏÔÁ›·˜ ÙÔ˘ ¶·Ó/Ì›Ô˘ ∞ıËÓÒÓ (¡Ô¤Ì‚ÚÈÔ˜ 2000), ‰ÂÓ ˘¿Ú¯Ô˘Ó ÎÚÔ‡ÛÌ·Ù· Ù˘ Ó¤·˜ ÛÔÚ·‰È΋˜ ÌÔÚÊ‹˜ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙË ‚ÚÒÛË ‚Ô‰ÈÓÔ‡ ÎÚ¤·ÙÔ˜. À¿Ú¯Ô˘Ó 20 ‚‚·ÈˆÌ¤Ó· ÎÚÔ‡ÛÌ·Ù· Ù˘ ÓfiÛÔ˘ Î·È 7 ˘fi ‰ÈÂÚ‡ÓËÛË, Ù· ÔÔ›· ‰È·ÁÓÒÛÙËÎ·Ó ÌÂÙ¿ ÙÔ 1997. ŸÏ˜ ÔÈ ÂÚÈÙÒÛÂȘ ·ÊÔÚÔ‡Û·Ó ËÏÈÎȈ̤ӷ ¿ÙÔÌ· Î·È ·Ó‹Î·Ó ÛÙË ÛÔÚ·‰È΋ ÌÔÚÊ‹. ™ËÌÂÈÒÓÂÙ·È fiÙÈ Ú¤ÂÈ Ó· ˘¿Ú¯ÂÈ ÎÏËÚÔÓÔÌÈ΋ ÚԉȿıÂÛË ÁÈ· ÙËÓ ÂΉ‹ÏˆÛË Ù˘ ÛÔÚ·‰È΋˜ Î·È Ù˘ È·ÙÚÔÁÂÓÔ‡˜ ÌÔÚÊ‹˜ Ù˘ ÓfiÛÔ˘. ŸÏÔÈ ÔÈ ¿Û¯ÔÓÙ˜, ‰ËÏ·‰‹, ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙÔÓ ›‰ÈÔ ÁÔÓfiÙ˘Ô, Ô ÔÔ›Ô˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ‡·ÚÍË ÌÂıÂÈÔÓ›Ó˘ Û ÔÌfi˙˘ÁË ÌÔÚÊ‹ ÛÙÔ Îˆ‰ÈÎfiÓÈÔ 129 ÙÔ˘ ÁÔÓȉ›Ô˘ PrP (31). ø˜ ÙÒÚ·, fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ Ì ÙË Ó¤· ÛÔÚ·‰È΋ ÌÔÚÊ‹ Â›Ó·È ÔÌfi˙˘ÁÔÈ ÁÈ· ÙË ÌÂıÂÈÔÓ›ÓË ÛÙË ı¤ÛË ·˘Ù‹, ÂÓÒ ¿ÏÏÔÈ ÁÔÓfiÙ˘ÔÈ ÌÔÚ› Ó· Â›Ó·È ·ÓıÂÎÙÈÎÔ› ÛÙË ÓfiÛÔ ‹ Ó· ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚÔ ¯ÚfiÓÔ ÂÒ·Û˘ (32). ¶Ú¤ÂÈ Ó· ÛËÌÂȈı› fiÙÈ Ë Û˘¯ÓfiÙËÙ· ·˘ÙÔ‡ ÙÔ˘ ÁÔÓfiÙ˘Ô˘ ÛÙÔÓ ÏËı˘ÛÌfi Ù˘ ª. µÚÂÙ·Ó›·˜ Â›Ó·È 40% (32). ¶ÚfiÛÊ·Ù·, ·Ó·ÎÔÈÓÒıËÎÂ Ë Èı·ÓfiÙËÙ· ‡·Ú͢ ·Û˘Ìو̷ÙÈÎÒÓ ÊÔÚ¤ˆÓ Ù˘ ÓfiÛÔ˘. ∆Ô Û˘Ì¤Ú·ÛÌ· ·˘Ùfi ÚԤ΢„ ·fi ÌÂϤÙ˜ Û ÔÓÙ›ÎÈ· Ô˘ ÌÔχÓıËÎ·Ó Ì prions, ÙˆÓ ÔÔ›ˆÓ Ë Û˘ÁΤÓÙÚˆÛË ÛÙÔÓ ÂÁΤʷÏÔ ‹Ù·Ó Ôχ ˘„ËÏ‹ Î·È ÂÓÙÔ‡ÙÔȘ ‰ÂÓ ·ÚÔ˘Û›·˙·Ó Û˘Ìو̷ÙÔÏÔÁ›· (33). ∏ ‡·ÚÍË ·Û˘Ìو̷ÙÈÎÒÓ ÊÔÚ¤ˆÓ ·˘Í¿ÓÂÈ ÙȘ Èı·ÓfiÙËÙ˜ ÌÂÙ¿‰ÔÛ˘ ̤ۈ ÌÂÙ¿ÁÁÈÛ˘ ·›Ì·ÙÔ˜ ‹ ·Ú·ÁÒÁˆÓ Î·È Î¿ÓÂÈ ÙËÓ ¤ÎÙ·ÛË Ù˘ ÓfiÛÔ˘ ·ÚÔÛ‰ÈfiÚÈÛÙË. ∏ ÌfiÓË ·¿ÓÙËÛË Ô˘ ÌÔÚ› Ó· ‰Ôı› ÛÙÔ ÌÂÁ¿ÏÔ ÂÚÒÙËÌ· Ù˘ ηٷӿψÛ˘ ‹ fi¯È ‚Ô‰ÈÓÔ‡ ÎÚ¤·ÙÔ˜, Â›Ó·È ÙÔ “·Ó ̤ÙÚÔÓ ¿ÚÈÛÙÔÓ”. ª›· ‰È·ÙÚÔÊ‹ Ì ÔÈÎÈÏ›· ÙÚÔÊÒÓ Î·È ÎÔÓÙ¿ ÛÙËÓ ·Ú·‰ÔÛȷ΋ ÌÂÛÔÁÂȷ΋ ‰›·ÈÙ· Â›Ó·È Ë ÌfiÓË Ï‡ÛË ÁÈ· ÙËÓ ÚÔÛÙ·Û›· Ì·˜ ·fi ¤Ó·Ó ÌÂÁ¿ÏÔ ·ÚÈıÌfi ÓÔÛËÌ¿ÙˆÓ Î·È fi¯È ÌfiÓÔ ·fi ÙË ÓfiÛÔ ÙˆÓ “ÙÚÂÏÒÓ ·ÁÂÏ¿‰ˆÓ”. µÈ‚ÏÈÔÁÚ·Ê›· 1. Ghetti B, Piccardo P, Frangione B, Bugiani O, Giaccone G, Young K et al. Prion protein amyloidosis. Brain Pathol 1996;6:127-145. 2. Collinge J, Rossor M. Less beef, more brain. Lancet 1996;347:915-917. 3. Brown P, Cathala F, Raubertas RF, Gajdusek DC, Cataigne P. The epidemiology of Creutzfeldt-Jakob disease:
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1989. ISBN. 185197-405-9. 21. Bradley R, Wilesmith JW. Epidemiology and control of bovine spongiform encephalopathy (BSE). Br Med Bull 1993;49:932-959. 22. Colle JG, Bradley R. BSE: a decade on-part 2. Lancet 1997;349:715-721. 23. Wilesmith JW, Wells GAH, Cranwell MP, Ryan JBM. Bovine spongiform encephalopathy. Epidemiological studies. Vet Rec 1988;123:638-644. 24. Collinge J, Palmer MS, Dryden AJ. Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease. Lancet 1991;337:1441-1442. 25. Pattison IH, Hoare MN, Jebbett JN, Watson WA. Further observations on the introduction of scrapie in sheep by oral dosing with foetal membranes from scrapie-affected sheep. Br Vet J 1974;130:LXV-LXVII. 26. Gerald-Coffee J. A dreadful challenge. Lancet 1996;347:917-918. 27. Taylor DM. Inactivation of SE agents. Br Med Bull 1993;49:810-821. 28. Taylor DM. Deactivation of BSE and scrapie agents: rendering and other UK studies. In: Bradley R, Marchand B, eds. Transmissible spongiform encephalopathies. Brussels: C.E.C; 1995. 29. Taylor DM. Inactivation of the unconventional agents of scrapie, bovine spongiform encephalopathy and Creutzfeldt-Jakob disease. J Hosp Infect 1991;18(Suppl A):141-146. 30. Newby MJ, Thomas PJ. Witness Statement No 313B to the BSE Enquiry Jan 2000. 31. Collinge J. Human prion diseases and bovine spongiform encephalopathy (BSE). Hum Mol Genet 1997;6:1699-1705. 32. Ghani AC, Ferguson NM, Donnelly CA, Anderson RM. Predicted vCJD mortality in Great Britain. Nature 2000;406:583-584. 33. Bonn D. Healthy carriers could increase vCJD risk, scientists say. Lancet 2000;356:833.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 14-02-2001 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 14-06-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∫. ∫·Ú·‚·Ó¿ÎË-∫·Ú·Ó¿ÛÈÔ˘ ∂ÏÏËÓÈÎÔ‡ ™ÙÚ·ÙÔ‡ 22∞, 152 37 ºÈÏÔı¤Ë, ∞ı‹Ó·
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∂•∂§π•∂π™ ™∆∏¡ π∞∆ƒπ∫∏ ∂¶π™∆∏ª∏
Paediatriki 2001;64:527-534
PROGRESS IN MEDICAL SCIENCE
¶·Ú¿ÁÔÓÙ˜ Ó¢ÚÔÁ¤ÓÂÛ˘ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô. ¡ÂfiÙÂÚ˜ ·fi„ÂȘ ∫. ∂. ™Ô‡ÏË1, Õ. ¢Ô˘ÏÁÂÚ¿ÎË2, ™. ∆۷ΛÚ˘2
Factors affecting neurogenesis during the neonatal period. A review K. H. Schulpis1, A. Doulgeraki2, S. Tsakiris2
¶ÂÚ›ÏË„Ë: O Û¯ËÌ·ÙÈÛÌfi˜ Î·È Ë ÔÚÁ¿ÓˆÛË ÙˆÓ Ó¢ÚÒÓˆÓ Û ‰›ÎÙ˘·, ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, Â›Ó·È ÔχÏÔη Ê·ÈÓfiÌÂÓ· Ô˘ Ú˘ıÌ›˙ÔÓÙ·È ·fi Ì›· ÔÈÎÈÏ›· ·Ú·ÁfiÓÙˆÓ. ¢›ÓÂÙ·È ¤ÌÊ·ÛË ÛÙÔ ÚfiÏÔ ÙˆÓ Ó¢ÚÔÙÚÔÊÈÓÒÓ, Ù˘ Ó¢ÚÔÁÏÔ›·˜, ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, Ù˘ Û˘Ó·ÙÈ΋˜ ËÏÂÎÙÚÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜ Î·È ÙˆÓ Ó¢Úԉȷ‚È‚·ÛÙÒÓ. ∏ ÌÂٷ͇ ÙÔ˘˜ Û˘ÓÂÚÁ·Û›· Ú˘ıÌ›˙ÂÈ ÙËÓ ÂÈÌ‹Î˘ÓÛË Î·È ‰È·ÎÏ¿‰ˆÛË ÙˆÓ Ó¢ÚÒÓˆÓ, ·ÏÏ¿ Î·È ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ÙÔ˘˜ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·. ∂ӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ Î·È Ë ıÂÙÈ΋ ›‰Ú·ÛË ‰È·ÙÚÔÊÈÎÒÓ ÛÙÔȯ›ˆÓ, fiˆ˜ Ë ÁÏ˘Îfi˙Ë, Ô Û›‰ËÚÔ˜ Î·È ÙÔ ÌËÙÚÈÎfi Á¿Ï· ÛÙË ‰È·ÌfiÚʈÛË Î·È ˆÚ›Ì·ÓÛË ÙˆÓ ÓÂ˘ÚˆÓÈÎÒÓ Û˘Ó‰¤ÛˆÓ. ™¯ÔÏÈ¿˙ÂÙ·È Ë ÈÛÔÚÚÔ›· ÌÂٷ͇ Ù˘ ÂÈ‚›ˆÛ˘ Î·È ÙÔ˘ ı·Ó¿ÙÔ˘ ÙˆÓ Ó¢ÚÒÓˆÓ, ηıÒ˜ Î·È ÔÈ Èı·Ó¤˜ ÂÊ·ÚÌÔÁ¤˜ ÙˆÓ Ó¤ˆÓ ·fi„ÂˆÓ ÛÙË ‰ÈÂÚ‡ÓËÛË Ù˘ Ï·ÛÙÈÎfiÙËÙ·˜ ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ηıÒ˜ ›Û˘ Î·È ÛÙË ıÂڷ›· ÙˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ Î·È ÂÎÊ˘ÏÈÛÙÈÎÒÓ ·ı‹ÛˆÓ.
Abstract: The most recent data regarding the structure of the neuronal circuits during postnatal development are reviewed. These phenomena are rather complex because they include processes regulated by many different factors. The role of neurotrophins, neuroglia, growth factors, synaptic electrical activity and neurotransmitters are strongly emphasized. The mutual interactions of the above factors control the elongation and branching of the neurons, as well as their functional efficacy. Of great interest is the positive influence of nutritional elements, such as glucose, iron and maternal milk, on the morphology and maturation of the neuronal connections. Finally, the balance between neuronal survival and death is discussed, as well as the application of known data on the study of neural plasticity and the therapeutic approach of the neurodegenerative diseases.
§¤ÍÂȘ ÎÏÂȉȿ: ·Ó¿Ù˘ÍË, Ó¢ÚÔÁ¤ÓÂÛË, Û˘Ó·ÙÔÁ¤ÓÂÛË, ·˘ÍËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜.
Key words: development, neurogenesis, synaptogenesis, growth factors, newborns.
™˘ÓÙÔÌÔÁڷʛ˜ ACh AChE BDGF
·ÎÂÙ˘ÏÔ¯ÔÏ›ÓË ·ÎÂÙ˘ÏÔ¯ÔÏÈÓÂÛÙÂÚ¿ÛË ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ô˘ ·Ú¿ÁÂÙ·È ·fi ÙÔÓ ÂÁΤʷÏÔ cAMP ΢ÎÏÈ΋ ÌÔÓÔʈÛÊÔÚÈ΋ ·‰ÂÓÔÛ›ÓË CAMs ÌfiÚÈ· ΢ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ DSCAM ÌfiÚÈÔ Î˘ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Down EGF ÂȉÂÚÌÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜
1 πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ¶·È‰ÈÔ‡, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó· 2 ∂ÚÁ·ÛÙ‹ÚÈÔ ¶ÂÈÚ·Ì·ÙÈ΋˜ º˘ÛÈÔÏÔÁ›·˜, π·ÙÚÈ΋ ™¯ÔÏ‹ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ
Fe FGF 5-HT ICAMs IFN-Á IGF-I IL-1 IL-2 IL-3
Û›‰ËÚÔ˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÈÓÔ‚Ï·ÛÙÒÓ 5-˘‰ÚÔ͢ÙÚ˘Ù·Ì›ÓË (ÛÂÚÔÙÔÓ›ÓË) ‰È·Î˘ÙÙ·ÚÈο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ ÈÓÙÂÚÊÂÚfiÓË-Á ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ô˘ ÌÔÈ¿˙ÂÈ Ì ÙËÓ ÈÓÛÔ˘Ï›ÓË, Ù‡Ô˜ π ÈÓÙÂÚÏ¢ΛÓË-1 ÈÓÙÂÚÏ¢ΛÓË-2 ÈÓÙÂÚÏ¢ΛÓË-3
1 Institute of Child Health “Saint Sophia” Children’s Hospital, Athens 2 Laboratory of Experimental Physiology University of Athens Medical School
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IL-6 LTP MAGs MOG
ÈÓÙÂÚÏ¢ΛÓË-6 Ì·ÎÚÔÚfiıÂÛÌË ÂÓ›Û¯˘ÛË ÙˆÓ Û˘Ó¿„ÂˆÓ ÁÏ˘ÎÔÚˆÙ½Ó˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË Ì˘ÂÏ›ÓË ÁÏ˘ÎÔÚˆÙ½ÓË Ù˘ Ì˘ÂÏ›Ó˘ ÙˆÓ ÔÏÈÁÔ‰ÂÓ‰ÚÔ΢ÙÙ¿ÚˆÓ NCAMs ÓÂ˘ÚˆÓÈο ÌfiÚÈ· ΢ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ NgCAMs Ó¢ÚÔÁÏÔȷο ÌfiÚÈ· ΢ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ NGF ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÙˆÓ ÓÂ‡ÚˆÓ ¡™ Ó¢ÚÈÎfi Û‡ÛÙËÌ· ¡-∆ Ó¢ÚÔÙÚÔÊ›ÓË ƒ∂∆ ÙÔÌÔÁÚ·Ê›· ÂÎÔÌ‹˜ Ô˙ÈÙÚÔÓ›ˆÓ TNF-· ·Ú¿ÁÔÓÙ·˜ Ó¤ÎÚˆÛ˘ ÙˆÓ fiÁΈÓ, Ù‡Ô˘ ·
∂ÈÛ·ÁˆÁ‹ °È· ÔÏÏ¿ ¯ÚfiÓÈ· ıˆÚ›ÙÔ fiÙÈ Ô ÂÁΤʷÏÔ˜ Î·È Ô ÓˆÙÈ·›Ô˜ Ì˘ÂÏfi˜ ‹Ù·Ó ·Ó›Î·ÓÔÈ ÁÈ· ·‡ÍËÛË ‹ ‰ÈÂÚÁ·Û›Â˜ ‰ÈfiÚıˆÛ˘. ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, fï˜, Â›Ó·È ÂÌÊ·Ó¤˜ fiÙÈ Î¿ÙÈ Ù¤ÙÔÈÔ ‰Â Û˘Ì‚·›ÓÂÈ, ·ÊÔ‡ Ì ÙÔ Î·Ù¿ÏÏËÏÔ ÂÚ¤ıÈÛÌ·, Ô ÂÁΤʷÏÔ˜ ÌÔÚ› Ó· ·Ó·ÁÂÓÓ‹ÛÂÈ Ù· ΢ÎÏÒÌ·Ù¿ ÙÔ˘. ∆Ô ÂÚ¤ıÈÛÌ· ÌÔÚ› Ó· Â›Ó·È ÙÚ·‡Ì· ‹ ÌÂÙ·‚ÔÏÈ΋ ‰È·Ù·Ú·¯‹ ‹ ·ÎfiÌË, Ô ÂÍ·Ó·ÁηÛÌfi˜ ÁÈ· ÙËÓ ÂÎÌ¿ıËÛË Ó¤Ô˘ ηı‹ÎÔÓÙÔ˜ ‹ Û˘ÌÂÚÈÊÔÚ¿˜. ªÔÚ›, ›Û˘, Ó· Â›Ó·È ÌfiÛ¯Â˘Ì· Ó¢ÚÈÎÔ‡ ÈÛÙÔ‡. ∆ÒÚ· ÁÓˆÚ›˙Ô˘Ì fiÙÈ ÔÈ ÎÂÓÙÚÈÎÔ› ÓÂ˘Ú¿ÍÔÓ˜, Ô˘ ÚÈÓ ÓÔÌ›˙·Ì fiÙÈ ‰ÂÓ ·Ó·ÁÂÓÓÈÔ‡ÓÙ·È, ÌÔÚÔ‡Ó Ó· ÌÂÁ·ÏÒÛÔ˘Ó Ì¤Ûˆ ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ Ó‡ÚÔ˘ ‹ Ì Ù¯ÓËÙ¤˜ “Á¤Ê˘Ú˜” Î·È Ó· Û¯ËÌ·Ù›ÛÔ˘Ó Û˘Ó‰¤ÛÂȘ Ì ·ÔÓÂ˘ÚˆÌ¤ÓÔ˘˜ ÛÙfi¯Ô˘˜. ∂ÈÚfiÛıÂÙ·, ÔÏfiÎÏËÚ· ÓÂ˘ÚˆÓÈο ‰›ÎÙ˘· ÌÔÚÔ‡Ó Ó· ·ÓÙÈηٷÛÙ·ıÔ‡Ó Ì ÌÂÙ·ÌfiÛ¯Â˘ÛË ÂÌ‚Ú˘˚ÎÒÓ ‹ ·Ó·Ù˘ÛÛfiÌÂÓˆÓ Ó¢ÚÒÓˆÓ ÛÙÔÓ ÂÓ‹ÏÈÎÔ ÂÁΤʷÏÔ. ∆ÂÏÈο, Ì ÙËÓ Â˘Ú‡ÙÂÚË ¤ÓÓÔÈ·, Ë ·Ó¿Ù˘ÍË Û˘Ó¯›˙ÂÙ·È Î·È ÌÂÙ¿ ÙË Á¤ÓÓËÛË. ¶ÂÚÈÏ·Ì‚¿ÓÂÈ Ì›· Û˘Ó¯‹ ·Ôı‹Î¢ÛË ‚Ú·¯˘ÚfiıÂÛÌˆÓ Î·È Ì·ÎÚÔÚfiıÂÛÌˆÓ ÏËÚÔÊÔÚÈÒÓ, fiÔ˘ Û οı ‚‹Ì· Ù˘ ·Ó¿Ù˘Í˘, ÁÂÓÂÙÈΤ˜ Î·È ÂÚÈ‚·ÏÏÔÓÙÈΤ˜ ÂȉڿÛÂȘ ·ÓÙÈÚÔۈ‡ÔÓÙ·È ÛÙÔ ÂÍÂÏÈÛÛfiÌÂÓÔ ‰ÔÌÈÎfi Î·È ÏÂÈÙÔ˘ÚÁÈÎfi Û¯¤‰ÈÔ ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (1). ∆· ·Ó·Ù˘ÛÛfiÌÂÓ· ·ÙÙ·Ú· ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (¡™) ÂÌϤÎÔÓÙ·È Û ÔχÏÔη ‰›· Ì˯·ÓÈ΋˜ Î·È ËÏÂÎÙÚÈ΋˜ Ù¿Û˘, fiˆ˜ ›Û˘ ‚ÈÔ¯ËÌÈÎÒÓ ‰ÈÂÚÁ·ÛÈÒÓ. ∞˘Ù¤˜ ÔÈ Û˘Ó¯Ҙ ÌÂÙ·‚·ÏÏfiÌÂÓ˜ ÏËÚÔÊÔڛ˜ ÁÈ· ÙÔ ¯ÒÚÔ Î·È ÙÔ ¯ÚfiÓÔ ÁÈ· οı ·ÙÙ·ÚÔ, ‰ËÌÈÔ˘ÚÁÔ‡ÓÙ·È, ηٿ ÌÂÁ¿ÏÔ Ì¤ÚÔ˜, ·fi ÙË ¯ËÌ›· ÙˆÓ ›‰ÈˆÓ ÙˆÓ Ó¢ÚÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È Â›Ó·È ÔÈ ‚·ÛÈΤ˜ ÂÚÈ‚·ÏÏÔÓÙÈΤ˜ ‰˘Ó¿ÌÂȘ Ô˘ ηıÔÚ›˙Ô˘Ó ÙËÓ ·ÏÏËÏÔ˘¯›· ÙˆÓ ·Ó·Ù˘ÍÈ·ÎÒÓ ‰È·‰ÈηÛÈÒÓ. ∞˘ÍËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÁÈ· ÙË Ó¢ÚÔÁ¤ÓÂÛË O ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÙˆÓ ÓÂ‡ÚˆÓ (NGF), Ô˘ ·ÔÌÔÓÒıËΠ·fi ÙÔ ÛÈÂÏÔÁfiÓÔ ·‰¤Ó· ÔÓÙÈÎÔ‡, ÂÌÊ·Ó›˙ÂÙ·È Ì ÙË ÌÔÚÊ‹ Û˘ÌÏfiÎÔ˘, Ô˘ ·ÔÙÂÏÂ›Ù·È ·fi ÙÚÂȘ ˘ÔÌÔÓ¿‰Â˜ ·, ‚ Î·È Á ÛÂ
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·Ó·ÏÔÁ›· ‚:2·:2Á. ∏ ˘ÔÌÔÓ¿‰· ‚ Â›Ó·È Ô ÌfiÓÔ˜ Ó¢ÚÔ‰Ú·ÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ·fi ÙÔ˘˜ ÚÔ·Ó·ÊÂÚfiÌÂÓÔ˘˜ ÙÚÂȘ. OÈ ÂȉڿÛÂȘ ÙÔ˘ NGF ıˆÚÂ›Ù·È fiÙÈ ·Ú¯›˙Ô˘Ó Ì ÙÔ Û¯ËÌ·ÙÈÛÌfi Û˘ÌÏÂÁÌ¿ÙˆÓ NGF-˘Ô‰Ô¯¤· ÛÙËÓ Î˘ÙÙ·ÚÈ΋ ÂÈÊ¿ÓÂÈ· Î·È ·ÎÔÏÔ˘ı› ÌÂÙ·ÙfiÈÛË ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ ÛÙÔ Î˘ÙÙ·ÚfiÏ·ÛÌ·. ∆· ·ÙÙ·Ú·-ÛÙfi¯ÔÈ ‰È·ı¤ÙÔ˘Ó Î·È ˘„ËÏ‹˜ Î·È ¯·ÌËÏ‹˜ Û˘ÁÁ¤ÓÂÈ·˜ ˘Ô‰Ô¯Â›˜. OÈ ¯·ÌËÏ‹˜ Û˘ÁÁ¤ÓÂÈ·˜ ˘Ô‰Ô¯Â›˜, ·ÚÔ˘Û›· ÙÔ˘ NGF, ÌÔÚÔ‡Ó Ó· ·ÏÏËÏÂȉڿÛÔ˘Ó, Ó· Û˘Ûۈ̷وıÔ‡Ó Î·È Ó· ÌÂÙ·ÙÚ·Ô‡Ó Û ˘„ËÏ‹˜ Û˘ÁÁ¤ÓÂÈ·˜ ˘Ô‰Ô¯Â›˜, ˆ˜ ̤ÚÔ˜ ÌÈ·˜ ‚ÈÔÏÔÁÈ΋˜ ·¿ÓÙËÛ˘. ™Ù· Û˘Ì·ıËÙÈο Ó¢ÚÈο ·ÙÙ·Ú· Ô˘ ¤¯Ô˘Ó ·ÍÔÓÈΤ˜ ·ÔʇÛÂȘ, Ô NGF Û˘Ó‰¤ÂÙ·È ÂÎÏÂÎÙÈο Û ˘Ô‰Ô¯Â›˜ ÙˆÓ ·ÍÔÓÈÎÒÓ ·ÔÏ‹ÍÂˆÓ Î·È ÂÛˆÙÂÚÈÎÔÔÈÂ›Ù·È ÁÈ· Ó· ÌÂÙ·ÊÂÚı› ηٿ Ì‹ÎÔ˜ ÙÔ˘ ¿ÍÔÓ· ÛÙÔ Î˘ÙÙ·ÚÈÎfi ÛÒÌ·. ª›· ÛÂÈÚ¿ ΢ÙÙ·ÚÔÏ·ÛÌ·ÙÈÎÒÓ Î·È ˘ÚËÓÈÎÒÓ ÁÂÁÔÓfiÙˆÓ Û˘Ì‚·›ÓÔ˘Ó Ì ¯ÚÔÓÈ΋ ·ÏÏËÏÔ˘¯›· Ô˘ Î˘Ì·›ÓÂÙ·È ·fi ÂÏ¿¯ÈÛÙ· ‰Â˘ÙÂÚfiÏÂÙ· ¤ˆ˜ ·ÚÎÂÙ¤˜ Ë̤Ú˜. OÈ Ê˘ÛÈÔÏÔÁÈΤ˜ ÂȉڿÛÂȘ ÙÔ˘ NGF (2,3) Ù·ÍÈÓÔÌÔ‡ÓÙ·È ÛÂ: ·) Ó¢ÚÔÙÚÔÊÈΤ˜ Î·È ıÚÂÙÈΤ˜ ηٿ ÙËÓ ·Ú¯È΋ ·Ó¿Ù˘ÍË, ‚) ‰Ú·ÛÙÈΤ˜ ÛÙË ‚ÈÔ¯ËÌÈ΋ Î·È ÌÔÚÊÔÏÔÁÈ΋ ‰È·ÊÔÚÔÔ›ËÛË, Á) ÈÛ¯˘Ú¿ Ó¢ÚÔÙÚÔÈΤ˜ ÛÙËÓ Â›‰Ú·ÛË Î·È ÙËÓ Î·Ù‡ı˘ÓÛË Ù˘ ·‡ÍËÛ˘ ÙÔ˘ Ó¢ڛÙË. ∏ ÙÔÈ΋ ‰Ú¿ÛË ÙÔ˘ NGF ¤¯ÂÈ ÛÎÔfi Ó· ÂϤÁ¯ÂÈ ÙË ‰È·Ù‹ÚËÛË ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ‰ÈÂÚÁ·ÛÈÒÓ Î·È ÌÔÚ› Ó· ÌÂÙ·ÊÂÚı› ÁÈ· Ó· ÂÍ·ÛÊ·Ï›ÛÂÈ ÙËÓ ÂÈ‚›ˆÛË ÙÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ÛÒÌ·ÙÔ˜ Ô˘ ‚Ú›ÛÎÂÙ·È Û οÔÈ· ·fiÛÙ·ÛË, Û ÂÚÈ‚¿ÏÏÔÓ, ‰ËÏ·‰‹, Ô˘ ÛÙÂÚÂ›Ù·È NGF. ¶ÚfiÛÊ·Ù˜ ¤Ú¢Ó˜ (4) ¤‰ÂÈÍ·Ó fiÙÈ Ô NGF ÚÔηÏ› ·‡ÍËÛË Ù˘ ÚÔ‹˜ ÙÔ˘ ÈÔÓÙÈṲ̂ÓÔ˘ Ca2+, Ô‰ËÁÒÓÙ·˜ Û ·‡ÍËÛË ÙÔ˘ ÚÔÛ˘Ó·ÙÈÎÔ‡ Ca2+. ∞˘Ù‹ Ë ·‡ÍËÛË Â›Ó·È ÛËÌ·ÓÙÈÎfi ÛÙÔÈ¯Â›Ô Ù˘ ‰Ú¿Û˘ ÙÔ˘ NGF ÛÙËÓ ·ÂÏ¢ı¤ÚˆÛË ÙˆÓ Ó¢Úԉȷ‚È‚·ÛÙÒÓ Î·È Ô‰ËÁ› ÛÙÔ Ê·ÈÓfiÌÂÓÔ Ù˘ Ì·ÎÚÔÚfiıÂÛÌ˘ ÂÓ›Û¯˘Û˘ ÙˆÓ Û˘Ó¿„ÂˆÓ (LTP). ∂›Û˘, Ô ¤ÏÂÁ¯Ô˜ Ù˘ ‰È·Î›ÓËÛ˘ ÙÔ˘ Ca2+ ·fi ÙÔÓ NGF, ÂËÚ¿˙ÂÈ ÙȘ Ì·ÎÚÔÚfiıÂÛ̘ Û˘Ó¤ÂȘ ÙÔ˘ ·Ú¿ÁÔÓÙ· ·˘ÙÔ‡ ÛÙË ÓÂ˘ÚˆÓÈ΋ ÂÈ‚›ˆÛË Î·È ÚÔÛÙ·Û›·. ∞fi ÙÔÓ ÂÁΤʷÏÔ Î·È ÙËÓ ˘fiÊ˘ÛË (5) ¤¯ÂÈ ·ÔÌÔÓˆı› Ô ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÙˆÓ ÈÓÔ‚Ï·ÛÙÒÓ (FGF), Ì ‰Ú¿ÛË Ó¢ÚÔÙÚÔÊÈ΋ Î·È Ó¢ÚÔÙÚÔÈ΋ ÛÙÔ˘˜ Ó¢ÚÒÓ˜ Î·È ÛÙË Ó¢ÚÔÁÏÔ›·. ¶Èı·ÓfiÓ ‰Ú· Î·È ÛÙËÓ ·ÁÁÂÈÔÁ¤ÓÂÛË ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘, ηıÒ˜ Î·È Û ÂÌ‚Ú˘˚ÎÔ‡˜ ÌÂÙ·Û¯ËÌ·ÙÈÛÌÔ‡˜. ∂›Ó·È ÂӉȷʤÚÔ˘Û· Ë ·Ú·Ù‹ÚËÛË fiÙÈ Ë Ó¢ÚÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· Ú˘ıÌ›˙ÂÈ ÙËÓ ¤ÎÊÚ·ÛË ÙˆÓ ÙÚÔÊÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ∆· ›‰· ÙÔ˘ FGF ·˘Í¿ÓÔÓÙ·È Î·Ù¿ ÙË ‰È·‰Èηۛ· ÂÎÌ¿ıËÛ˘ ÂÓfi˜ ηı‹ÎÔÓÙÔ˜. ŸÙ·Ó Ë ‰È·‰Èηۛ· ·˘Ù‹ Û˘Óԉ‡ÂÙ·È Î·È ·fi
¶·È‰È·ÙÚÈ΋ 2001;64:527-534
Paediatriki 2001;64:527-534
ÌÔ, ‰Ú· ÚÔÛ˘Ó·ÙÈο, ÒÛÙ ӷ ÚÔηϤÛÂÈ Ì·ÎÚÔÚfiıÂÛÌË ÂÓ›Û¯˘ÛË ÙˆÓ Û˘Ó¿„ˆÓ, Û˘Ì‚¿ÏÏÔÓÙ·˜ ÛÙË ‰È·ÌfiÚʈÛË Ù˘ ÌÓ‹Ì˘. ∂›Û˘, ·Ó·Ê¤ÚÂÙ·È Î·È ˆ˜ Ú˘ıÌÈÛÙ‹˜ Ù˘ ¤ÎÎÚÈÛ˘ ÙˆÓ Ó¢Úԉȷ‚È‚·ÛÙÒÓ (10).
·˘ÍË̤ÓË Ê˘ÛÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·, ÙfiÙÂ Ô FGF ‚Ú›ÛÎÂÙ·È ·ÎfiÌ· ÈÔ ·˘ÍË̤ÓÔ˜ (6). ∆· ˘„ËÏ¿ ›‰· ÙÔ˘ FGF ÚÔηÏÔ‡Ó ·‡ÍËÛË Ù˘ ˘ÎÓfiÙËÙ·˜ ÙˆÓ ·ÛÙÚÔ΢ÙÙ¿ÚˆÓ ÛÙÔÓ ÈfiηÌÔ. ∂Ô̤ӈ˜, Ë Ì¿ıËÛË ÂÓÈÛ¯‡ÂÈ ÙȘ ÂȉڿÛÂȘ Ù˘ Ê˘ÛÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÛÙË ‰È¤ÁÂÚÛË ÙˆÓ Ó¢ÚÔÙÚÔÊÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ŒÓ·˜ ¿ÏÏÔ˜ ÛËÌ·ÓÙÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Â›Ó·È Ô ÂȉÂÚÌÈÎfi˜ (EGF). ¢Ú· ˆ˜ ÌÈÙÔÁfiÓÔ ÁÈ· ÔÏÏ¿ ›‰Ë ΢ÙÙ¿ÚˆÓ, fiˆ˜ Ù· ·ÛÙÚÔ·ÙÙ·Ú· Î·È ‰ÈÂÁ›ÚÂÈ ÙË ‰È·ÊÔÚÔÔ›ËÛË Ù˘ Ó¢ÚÔÁÏÔ›·˜. O EGF ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ Ó¢ÚÒÓ· Ì ¿ÌÂÛÔ ÙÚfiÔ. ∂›Û˘, ÂÌϤÎÂÙ·È Î·È ÛÙËÓ ÂÌ‚Ú˘˚΋ ·Ó¿Ù˘ÍË. OÈ Ó¢ÚÔ‰Ú·ÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÚÔ¿ÁÔ˘Ó Ì›· ÔÈÎÈÏ›· ·Ó·Ù˘ÍÈ·ÎÒÓ ‰È·‰ÈηÛÈÒÓ, Ô˘ ÂÍ·ÚÙÒÓÙ·È ·fi ÙÔÓ Î˘ÙÙ·ÚÈÎfi Ù‡Ô, ÙÔ ÛÙ¿‰ÈÔ Ù˘ ΢ÙÙ·ÚÈ΋˜ ·Ó¿Ù˘Í˘ Î·È ÙÔ ÂÚÈ‚¿ÏÏÔÓ (¶›Ó·Î·˜ 1). O ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ô˘ ÌÔÈ¿˙ÂÈ Ì ÙËÓ ÈÓÛÔ˘Ï›ÓË, Ù‡Ô˘ π (IGF-I), ·ÚÔ˘ÛÈ¿˙ÂÈ ÔÏϤ˜ ‰Ú¿ÛÂȘ, ÂÓÂÚÁÒÓÙ·˜ Ì ·˘ÙÔÎÚÈÓ‹ ‹/Î·È ·Ú·ÎÚÈÓ‹ ÙÚfiÔ, Û ÂȉÈο ÛÙ¿‰È· Ù˘ ·Ó¿Ù˘Í˘ (7). ƒ˘ıÌ›˙ÂÈ ÂÓ‰Ô΢ÙÙ¿ÚÈ· ÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ Ó¢ÚÒÓˆÓ Î·È ÙË Ó¢ÚÔÁÏÔ›·. ∂›Û˘, ·˘Í¿ÓÂÈ ÙÔ ÂÓ‰Ô΢ÙÙ¿ÚÈÔ Ca2+ ÛÙ· ·ÙÙ·Ú· Purkinje Ù˘ ·ÚÂÁÎÂÊ·Ï›‰·˜. ∞fi ÙÔÓ ÂÁΤʷÏÔ ·Ú¿ÁÂÙ·È ¤Ó·˜ ÂȉÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜, Ô BDGF, Ì ÔÏϷϤ˜ ÏÂÈÙÔ˘ÚÁ›Â˜. ∞˘Í¿ÓÂÈ ÙËÓ ÔÏ˘ÏÔÎfiÙËÙ· Ù˘ ‰È¿Ù·Í˘ ÙˆÓ ‰ÂÓ‰ÚÈÙÒÓ (8) Î·È Û˘Ì‚¿ÏÏÂÈ ÛÙÔ Û¯ËÌ·ÙÈÛÌfi ‰ÈÂÁÂÚÙÈÎÒÓ Î·È ·Ó·ÛÙ·ÏÙÈÎÒÓ Û˘Ó¿„ˆÓ. ∂ËÚ¿˙ÂÈ ÙË ‰È·ÎÏ¿‰ˆÛË ÙˆÓ Ó¢ڷÍfiÓˆÓ, ηıÒ˜ Î·È ÙÔ Û˘ÓÔÏÈÎfi ÙÔ˘˜ Ì‹ÎÔ˜ (9). ™ÙÔÓ Èfiη-
¡Â˘ÚÔÙÚÔʛӘ (¡-∆) ¶ÚfiÎÂÈÙ·È ÁÈ· Ì›· ÔÌ¿‰· ÚˆÙÂ˚ÓÒÓ, Ì ΢ÚÈfiÙÂÚÔ˘˜ ÂÎÚÔÛÒÔ˘˜ ÙȘ ¡-∆-3 Î·È ¡-∆-4, Ô˘ ‰ÚÔ˘Ó Ì¤Ûˆ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ Trk (11). ƒ˘ıÌ›˙Ô˘Ó ÙË Ì·ÎÚÔÚfiıÂÛÌË ÂÈ‚›ˆÛË Î·È ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ Ó¢ÚÒÓˆÓ. ∏ Ó¢ÚÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· (.¯. Û˘Ó·ÙÈ΋ Ï·ÛÙÈÎfiÙËÙ· Î·È ·Ó¿Ù˘ÍË) ·˘Í¿ÓÂÈ Ù· ›‰¿ ÙÔ˘˜ ÛÙÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ· (12). OÈ ¡-∆ ÚÔηÏÔ‡Ó ÔÍ›· ·‡ÍËÛË Ù˘ Û˘Ó·ÙÈ΋˜ ‰È·‚›‚·Û˘. ∂›Û˘, Ú˘ıÌ›˙Ô˘Ó Ù· ›‰· ÙÔ˘ ÂÓ‰Ô΢ÙÙ¿ÚÈÔ˘ Ca2+, Ô˘ Â›Ó·È ÛËÌ·ÓÙÈÎfi ÁÈ· ÙËÓ ÂÈ‚›ˆÛË ÙˆÓ Ó¢ÚÒÓˆÓ Î·È ÙËÓ Ï·ÛÙÈÎfiÙËÙ· ÙˆÓ Û˘Ó¿„ˆÓ. ∂ËÚ¿˙Ô˘Ó Î·È ÙË Ó¢ÚÔÁÏÔ›·, Ú˘ıÌ›˙ÔÓÙ·˜ ÙËÓ ·Ú·ÁˆÁ‹ Î·È ÙËÓ ÂÈ‚›ˆÛË ÙˆÓ Ó¢ÚÔÁÏÔÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ. ™¯ÂÙ›˙ÔÓÙ·È ÛÙÂÓ¿ Ì ÙÔ ‚·ıÌfi ËÏÂÎÙÚÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÙˆÓ ÓÂ˘ÚˆÓÈÎÒÓ ‰ÈÎÙ‡ˆÓ (13). ∫˘ÙÔΛÓ˜ OÈ ÈÓÙÂÚÏ¢ΛÓ˜ 1‚, 3 Î·È 6 (IL-1‚, IL-3, IL-6), Ë ÈÓÙÂÚÊÂÚfiÓË-Á (IFN-Á) Î·È Ô ·Ú¿ÁÔÓÙ·˜ Ó¤ÎÚˆÛ˘ ÙˆÓ fiÁΈÓ-· (TNF·), ·ÚÔ˘ÛÈ¿˙Ô˘Ó Ó¢ÚÔÙÚÔÊÈ΋ ‰Ú¿ÛË, ·ÏÏ¿ fiÙ·Ó ‚Ú›ÛÎÔÓÙ·È Û ˘„ËÏ¿ ›‰·, ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ· Ó¢ÚÔÏÔÁÈο ÓÔÛ‹Ì·Ù· fiˆ˜ Ë ÛÎÏ‹Ú˘ÓÛË Î·Ù¿ Ͽη˜, Ë ÓfiÛÔ˜ Alzheimer Î·È ÙÔ ·ÁÁÂÈ·Îfi ÂÁÎÂÊ·ÏÈÎfi ÂÂÈÛfi‰ÈÔ. ∏ ›‰Ú·Û‹ ÙÔ˘˜ ÛÙÔ ÔÓÙ›ÎÈ ‰È·ÊÔÚÔÔÈ›ٷÈ,
¶›Ó·Î·˜ 1. ∞˘ÍËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙË ÓÂ˘ÚˆÓÈ΋ ·Ó¿Ù˘ÍË (1) ∞˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜
ªÔÚÈ·Îfi ‚¿ÚÔ˜ (kDa)
∫‡ÙÙ·Ú·ÛÙfi¯ÔÈ
∂ÁÎÂÊ·ÏÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁoÓÙ·˜ ÃÔÏÈÓÂÚÁÈÎfi˜ Ó¢ÚÔÙÚÔÊÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ∂ȉÂÚÌÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ πÓÔ‚Ï·ÛÙÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ (fiÍÈÓÔ˜)
12 20 6 16
¡ ¡ ∞,¡ ∞,¡
πÓÔ‚Ï·ÛÙÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ (‚·ÛÈÎfi˜)
16
∞,O,¡
∞˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ó¢ÚÔÁÏÔ›·˜ ∞˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ô˘ ÌÔÈ¿˙ÂÈ Ì ÙËÓ ÈÓÛÔ˘Ï›ÓË-π ∞˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ô˘ ÌÔÈ¿˙ÂÈ Ì ÙËÓ ÈÓÛÔ˘Ï›ÓË-ππ
31 7 7
∞,S O,N
πÓÙÂÚÏ¢ΛÓË π (·,‚)
16
A,O
πÓÙÂÚÏ¢ΛÓË ππ ¡Â˘ÚÈÎfi˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ¡Â˘ÚÔÏ¢ΛÓË ∞˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÚÔÂÚ¯fiÌÂÓÔ˜ ·fi Ù· ·ÈÌÔÂÙ¿ÏÈ·
15 26 56 32
O N N A,O
¢Â›ÁÌ· ÈÛÙÔ‡ ∂ÁΤʷÏÔ˜ OÊı·ÏÌfi˜, ÈۯȷÎfi Ó‡ÚÔ ÀÔÁÓ¿ıÈÔ˜ ·‰¤Ó·˜ ∂ÁΤʷÏÔ˜, ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹˜, ˘Ôı¿Ï·ÌÔ˜ ∂ÁΤʷÏÔ˜, ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹˜, ˘Ôı¿Ï·ÌÔ˜, ˘fiÊ˘ÛË ÀfiÊ˘ÛË, ÎÂÚÎÔÊfiÚÔ˜ ˘Ú‹Ó·˜ ◊·Ú, ¿ÏÏ· ·ÙÙ·Ú· ∂Ì‚Ú˘˚Îfi ‹·Ú, ÂÌ‚Ú˘˚Îfi˜ ÂÁΤʷÏÔ˜ ª·ÎÚÔÊ¿Á·, ·ÛÙÚÔ·ÙÙ·Ú·, ÌÈÎÚÔÁÏÔ›· ∆-·ÙÙ·Ú· ÀÔÁÓ¿ıÈÔ˜ ·‰¤Ó·˜, Ï·ÎÔ‡ÓÙ·˜ ÀÔÁÓ¿ıÈÔ˜ ·‰¤Ó·˜ ∞ÈÌÔÂÙ¿ÏÈ·, Ï·ÎÔ‡ÓÙ·˜, ·ÛÙÚÔ·ÙÙ·Ú·
∞: ·ÛÙÚÔ·ÙÙ·Ú·, ¡: Ó¢ÚÒÓ˜, O: ÔÏÈÁÔ‰ÂÓ‰ÚÔ·ÙÙ·Ú·, S: ·ÙÙ·Ú· Schwann
529
¶·È‰È·ÙÚÈ΋ 2001;64:527-534
·Ó¿ÏÔÁ· Ì ÙÔ ·Ó ‰ÚÔ˘Ó ÂÚÈÊÂÚÈο ‹ ÛÙ· ·ÛÙÚÔ·ÙÙ·Ú· ‹ ÛÙÔ˘˜ Ó¢ÚÒÓ˜ (14). ªfiÚÈ· ΢ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ (CAMs) (1) ¶ÔÏÏÔ› ·Ú¿ÁÔÓÙ˜ ˘ÔÛÙÚÒÌ·ÙÔ˜ Î·È Î˘ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ ÌÔÚÔ‡Ó Ó· ÂËÚ¿ÛÔ˘Ó ÙË Ó¢ÚÈ΋ ·Ó¿Ù˘ÍË Ì¤Ûˆ Ù˘ Ú‡ıÌÈÛ˘ ÙˆÓ È‰ÈÔÙ‹ÙˆÓ ÚÔÛÎfiÏÏËÛ˘ ÙˆÓ Î˘ÙÙ¿ÚˆÓ. OÈ ·ÏÏËÏÂȉڿÛÂȘ ÌÔÚÔ‡Ó Ó· Û˘Ì‚Ô‡Ó ¿ÌÂÛ· ÌÂٷ͇ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ‹ ÌÂٷ͇ ÂÓfi˜ ΢ÙÙ¿ÚÔ˘ Î·È Ù˘ ÂÍˆÎ˘ÙÙ¿ÚÈ·˜ Ô˘Û›·˜ ÙÔ˘ ÌÈÎÚÔÂÚÈ‚¿ÏÏÔÓÙÔ˜. ∆· ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ ÂËÚ¿˙Ô˘Ó ÙËÓ ÈηÓfiÙËÙ· ÙˆÓ Î˘ÙÙ¿ÚˆÓ Ó· ÌÂÙ·Ó·ÛÙ‡ԢÓ, Ó· Ù·ÍÈÓÔÌÔ‡ÓÙ·È Î·È Ó· ÛÙ·ıÂÚÔÔÈÔ‡Ó ÙȘ ¯ˆÚÔÙ·ÍÈΤ˜ ÙÔ˘˜ Û¯¤ÛÂȘ, Ô˘ ıˆÚÔ‡ÓÙ·È ÛËÌ·ÓÙÈΤ˜ ÁÈ· ÙËÓ ÔÚ›· Ù˘ ‰È·ÊÔÚÔÔ›ËÛ˘ (15,16). ™˘ÌÌÂÙ¤¯Ô˘Ó ÛÙËÓ Ï·ÛÙÈÎfiÙËÙ· Î·È ÛÙ·ıÂÚfiÙËÙ· ÙˆÓ Û˘Ó¿„ÂˆÓ (ÂËÚ¿˙ÔÓÙ·˜ ÙÔ Ê·ÈÓfiÌÂÓÔ LTP), ηıÒ˜ Î·È ÛÙËÓ ·Ó·Á¤ÓÓËÛË ÙˆÓ Ó¢ÚÒÓˆÓ (17). ∞˘Ù¿ Ù· ÌfiÚÈ· ÔÓÔÌ¿˙ÔÓÙ·È ÌfiÚÈ· ΢ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ (CAMs) Î·È ‰È·Î˘ÙÙ·ÚÈο ÌfiÚÈ· ÚÔÛÎfiÏÏËÛ˘ (ICAMs). OÈ ÈÓÙÂÁÎÚ›Ó˜ (integrins) Â›Ó·È Ì›· ÔÈÎÔÁ¤ÓÂÈ· ‰ÔÌÈο Û¯ÂÙÈÎÒÓ Û˘ÌÏÂÁÌ¿ÙˆÓ ÁÏ˘ÎÔÚˆÙ½Ó˘, Ô˘ ‰ÚÔ˘Ó ˆ˜ ˘Ô‰Ô¯Â›˜ ÁÈ· ÔÈÎÈÏ›· CAMs Î·È ICAMs (18). ∞ÔÙÂÏÔ‡ÓÙ·È ·fi · Î·È ‚ ˘ÔÌÔÓ¿‰Â˜ Î·È Èı·ÓfiÓ ·›˙Ô˘Ó ÚfiÏÔ ÛÙË Û‡Ó‰ÂÛË ÙÔ˘ ΢ÙÙ·ÚÔÛÎÂÏÂÙÔ‡ Î·È ÙÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ÌÂÙ·‚ÔÏÈÛÌÔ‡ Ì ÙÔ ÂÍˆÎ˘ÙÙ¿ÚÈÔ ÂÚÈ‚¿ÏÏÔÓ. ∏ Ï·ÌÈÓ›ÓË ·Ó‹ÎÂÈ ÛÙ· ICAMs Î·È ÂÓÂÚÁ› ÁÈ· Ó· Û˘Ó‰¤ÛÂÈ Î˘ÙÙ·ÚÈΤ˜ ÂÈÊ¿ÓÂȘ Î·È ÙȘ ÁÏ˘ÎÔÚˆÙ½Ó˜ Ù˘ ÌÂÛÔ΢ÙÙ¿ÚÈ·˜ Ô˘Û›·˜ (19,20). ª¤Ûˆ ‰È·Î˘ÙÙ·ÚÈÎÒÓ ·ÏÏËÏÂȉڿÛˆÓ, Ë Ï·ÌÈÓ›ÓË ÙÚÔÔÔÈ› ÙË Ó¢ÚÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·. À¿Ú¯ÂÈ ÔÌfiÏÔÁÔ ÂÙ›‰ÈÔ ÙÔ˘ ηڂÔ͢ÙÂÏÈÎÔ‡ ¿ÎÚÔ˘ Ù˘ Á1-·Ï˘Û›‰·˜ Ù˘ Ï·ÌÈÓ›Ó˘, Ô˘ Ú˘ıÌ›˙ÂÈ ÙËÓ ËÏÂÎÙÚÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ ˘Ú·ÌȉÈÎÒÓ Ó¢ÚÒÓˆÓ ÛÙÔ ÊÏÔÈfi ÙÔ˘ ÂÓ‹ÏÈη. ªÂ ·˘Ùfi ÙÔÓ ÙÚfiÔ, Ë Ï·ÌÈÓ›ÓË ·˘Í¿ÓÂÈ ÙË ÓÂ˘ÚˆÓÈ΋ ‰ÈÂÁÂÚÛÈÌfiÙËÙ· (21). Œ¯ÂÈ, ›Û˘, ·Ô‰Âȯı› Ë ÛÙÂÓ‹ Û¯¤ÛË Ï·ÌÈÓ›Ó˘ Î·È ı˘ÚÔ͛Ӣ. ¶ÙÒÛË ÙˆÓ ÂȤ‰ˆÓ Ù˘ ÔÚÌfiÓ˘ Ô‰ËÁ› Û ÂÏ¿ÙÙˆÛË ÙˆÓ Û˘ÁÎÂÓÙÚÒÛÂˆÓ Ù˘ Ï·ÌÈÓ›Ó˘ Î·È ·ÒÏÂÈ· ÙÔ˘ ÛˆÛÙÔ‡ Û˘Á¯ÚÔÓÈÛÌÔ‡ Ù˘ ÓÂ˘ÚˆÓÈ΋˜ ·‡ÍËÛ˘, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ô‰ÈÔÚÁ¿ÓˆÛË ÙˆÓ Ó¢ÚÒÓˆÓ (22). ∏ ÊÈÌÚÔÓÂÎÙ›ÓË (‹ ÈÓÔ‰ÂÛÌ›ÓË) ·Ó‹ÎÂÈ Â›Û˘ ÛÙ· ICAM. ∂ȉڿ ΢ڛˆ˜ ÛÙËÓ Î˘ÙÙ·ÚÈ΋ ΛÓËÛË Î·È ÙË ‰È·Î˘ÙÙ·ÚÈ΋ Û˘Ûۈ̿وÛË Î·È ‰È·ÊÔÚÔÔ›ËÛË Î·Ù¿ ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ¡™. ∆· CAMs Â›Ó·È ÁÏ˘ÎÔÚˆÙ½Ó˜ Ì ÌÔÚÊÔÚÚ˘ıÌÈÛÙÈÎfi ÚfiÏÔ ÛÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ¡™ (15). À¿Ú¯Ô˘Ó Ó¢ÚÈο CAMs (NCAMs) Ô˘ ÂÓÙÔ›˙ÔÓÙ·È ÓˆÚ›˜ ÛÙËÓ ÂÌ‚Ú˘ÔÁ¤ÓÂÛË Î·È Î·Ù¿ ÙËÓ ·Ó¿-
530
Paediatriki 2001;64:527-534
Ù˘ÍË ÙÔ˘ ¡™ Î·È ÛÙ· Ó¢ÚÈο Î·È ÛÙ· Ó¢ÚÔÁÏÔȷο ·ÙÙ·Ú·. À¿Ú¯Ô˘Ó Î·È Ó¢ÚÔÁÏÔȷο CAMs (NgCAMs), Ô˘ ‚Ú›ÛÎÔÓÙ·È ÌfiÓÔ ÛÙ· Ó¢ÚÈο ·ÙÙ·Ú· Î·È Èı·ÓfiÓ Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙË Û‡Ó‰ÂÛË ÌÂٷ͇ ÙˆÓ Ó¢ÚÈÎÒÓ Î·È Ó¢ÚÔÁÏÔÈ·ÎÒÓ ÌÂÌ‚Ú·ÓÒÓ. OÈ ÁÏ˘ÎÔÚˆÙ½Ó˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË Ì˘ÂÏ›ÓË (MAGs), ıˆÚÂ›Ù·È fiÙÈ Â›Ó·È ˘Â‡ı˘Ó˜ ÁÈ· ÙËÓ ·ÏÏËÏ›‰Ú·ÛË Ó¢ÚÔÁÏÔ›·˜-·ÍfiÓˆÓ Î·Ù¿ ÙË Ì˘ÂÏÈÓÔÔ›ËÛË. ∂ӉȷʤÚÔÓ ·ÚÔ˘ÛÈ¿˙ÂÈ ÙÔ ÌfiÚÈÔ Î˘ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ ÛÙÔ Û‡Ó‰ÚÔÌÔ Down (DSCAM) (23). ∂ÓÙÔ›˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙÔ Ó¢ÚÈÎfi ۈϋӷ, ÙÔ ÊÏÔÈfi, ÙÔÓ ÈfiηÌÔ, ÙÔ ÓˆÙÈ·›Ô Ì˘ÂÏfi Î·È ÛÙÔ˘˜ ÈÛÙÔ‡˜ Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙË Ó¢ÚÈ΋ ·ÎÚÔÏÔÊ›·. ∂ËÚ¿˙ÂÈ ‰˘ÛÌÂÓÒ˜ ÙË ‰È·ÊÔÚÔÔ›ËÛË Î·È ÙË ÌÂÙ·Ó¿ÛÙ¢ÛË ÙˆÓ Ó¢ÚÒÓˆÓ, ηıÒ˜ Î·È ÙË Û˘Ó·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·. π‰È·›ÙÂÚË ·Ó·ÊÔÚ¿ Ú¤ÂÈ Ó· Á›ÓÂÈ ÛÙȘ ÚˆÙÂÔÁÏ˘Î¿Ó˜ (24). ¶ÚfiÎÂÈÙ·È ÁÈ· Ì·ÎÚÔÌfiÚÈ· Ì ÔÏϷϤ˜ ÏÂÈÙÔ˘ÚÁ›Â˜, Ô˘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ¤Ó· ÔÏ˘ÂÙȉÈÎfi ΤÓÙÚÔ Î·È ÔÈΛϘ ·Ï˘Û›‰Â˜ ÁÏ˘ÎÔ˙·ÌÈÓÔÁÏ˘Î·ÓÒÓ. OÈ ÚˆÙÂÔÁÏ˘Î¿Ó˜ Ú˘ıÌ›˙Ô˘Ó ÙË ‰ÔÌÈ΋ ÔÚÁ¿ÓˆÛË Ù˘ ÂÍˆÎ˘ÙÙ¿ÚÈ·˜ Ô˘Û›·˜, ÙÚÔÔÔÈÔ‡Ó ÙȘ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ηıÒ˜ Î·È Ù· Ê·ÈÓfiÌÂÓ· Ù˘ ΢ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ Î·È ÎÈÓËÙÈÎfiÙËÙ·˜, fiˆ˜ Ë ÌÂÙ·Ó¿ÛÙ¢ÛË ÙˆÓ Ó¢ÚÒÓˆÓ Î·È Ë ·‡ÍËÛË ÙˆÓ Ó¢ڷÍfiÓˆÓ. ∂Ô̤ӈ˜, ÔÈ ÚˆÙÂÔÁÏ˘Î¿Ó˜ ·ÔÙÂÏÔ‡Ó ÛËÌ·ÓÙÈÎÔ‡˜ ÙÚÔÔÔÈËÙ¤˜ Ù˘ Ó¢ÚÈÙÈ΋˜ ·‡ÍËÛ˘ Î·È Ù˘ ÔÏÈÎfiÙËÙ·˜ ÙˆÓ Ó¢ÚÒÓˆÓ. øÛÙfiÛÔ, ÂȉÈΤ˜ ˘ÔηÙËÁÔڛ˜ ·˘ÙÒÓ ÙˆÓ Ì·ÎÚÔÌÔÚ›ˆÓ Ê·›ÓÂÙ·È fiÙÈ ·Ó·ÛÙ¤ÏÏÔ˘Ó ÙËÓ Â¤ÎÙ·ÛË ÙˆÓ Ó¢ÚÈÙÒÓ, ÙfiÛÔ Î·Ù¿ ÙËÓ ·Ó¿Ù˘ÍË, fiÛÔ Î·È Î·Ù¿ ÙËÓ ·Ó·Á¤ÓÓËÛË ÙÔ˘ ¡™. ∫·ıÒ˜ Ë ·Ó¿Ù˘ÍË ÚÔ¯ˆÚ›, ·Ú·ÙËÚÂ›Ù·È ÈÛÔÚÚÔ›· ÌÂٷ͇ ΢ÙÙ·ÚÈ΋˜ ÂÈ‚›ˆÛ˘ Î·È Î˘ÙÙ·ÚÈÎÔ‡ ı·Ó¿ÙÔ˘. ¶ÔÈÔÈ Â›Ó·È ÔÈ ·Ú¿ÁÔÓÙ˜, ÏÔÈfiÓ, Ô˘ ÂËÚ¿˙Ô˘Ó ·˘Ù‹ ÙËÓ ÈÛÔÚÚÔ›·; O ¤ÏÂÁ¯Ô˜ ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ·ˆÏÂÈÒÓ Î·Ù¿ ÙËÓ ·Ó¿Ù˘ÍË Â›Ó·È Èı·Ófi Ó· Á›ÓÂÙ·È ·fi ÙÔ Î˘ÙÙ·ÚÈÎfi ÌÈÎÚÔÂÚÈ‚¿ÏÏÔÓ. ™ÙË Ú‡ıÌÈÛË ÙÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ı·Ó¿ÙÔ˘ Û˘Ì‚¿ÏÏÔ˘Ó Ù· ÂÏ·Ùو̤ӷ ›‰· ‰È·Ï˘ÙÒÓ ·Ú·ÁfiÓÙˆÓ Ô˘ ÂÎÎÚ›ÓÔÓÙ·È ·fi Ù· ·ÙÙ·Ú·-ÛÙfi¯Ô˘˜, Ù· ÔÔ›· Ô‰ËÁÔ‡Ó Û ·ÓÙ·ÁˆÓÈÛÌfi ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÁÈ· ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ·˘ÙÔ‡˜ (1). ∂ÈÚfiÛıÂÙ·, Ë ËÏÂÎÙÚÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· Î·È Ô NGF (2) ÌÔÚÔ‡Ó Ó· ÂËÚ¿ÛÔ˘Ó ÙË ÓÂ˘ÚˆÓÈ΋ ÂÈ‚›ˆÛË. ¶ÈÔ Û˘ÁÎÂÎÚÈ̤ӷ, ¤¯ÂÈ ˘ÔÏÔÁÈÛÙ› fiÙÈ ÔÈ ÌÈÛÔ› Ó¢ÚÒÓ˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Âı·›ÓÔ˘Ó fiÙ·Ó Û¯ËÌ·Ù›˙ÔÓÙ·È ÔÈ Û˘Ó‰¤ÛÂȘ ÙÔ˘˜ (25). Œ¯ÂÈ, ›Û˘, ·Ú·ÙËÚËı› ¤Ó· ·ÚÁfi “Û‹Ì· ÂÈ‚›ˆÛ˘” (̤ۈ ÙˆÓ ¡-∆) ÎÈ ¤Ó· ÁÚ‹ÁÔÚÔ “Û‹Ì· ı·Ó¿ÙÔ˘” (›ÛÔ‰Ô˜
¶·È‰È·ÙÚÈ΋ 2001;64:527-534
Ca2+ ÛÙÔ Î‡ÙÙ·ÚÔ), Ô˘ ÔÊ›ÏÂÙ·È ÛÙËÓ ËÏÂÎÙÚÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ ÚÔÛ˘Ó·ÙÈÎÒÓ ·Ôϋ͈Ó. ªÂ ÙËÓ ÙÚÔÔÔ›ËÛË ·˘ÙÒÓ ÙˆÓ ÛËÌ¿ÙˆÓ, ¤گÂÙ·È Â›Ù ÚÔÛÙ·Û›· ÙˆÓ Ó¢ÚÒÓˆÓ Â›Ù ÓÂ˘ÚˆÓÈÎfi˜ ı¿Ó·ÙÔ˜. ™¯ÂÙÈο Ì ÙÔÓ NGF, Ê·›ÓÂÙ·È fiÙÈ ·Ó·ÛÙ¤ÏÏÂÈ ÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ ÙÔ˘ ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ı·Ó¿ÙÔ˘ (·fiÙˆÛË), ÔÈ ÔÔ›ÔÈ ÂÓÂÚÁÔÔÈÔ‡ÓÙ·È ÛÙÔ˘˜ Ó¢ÚÒÓ˜ Ô˘ ‰ÂÓ ¤¯Ô˘Ó ¡-∆ (26). ∂Í¿ÏÏÔ˘, Ë ÂÎfiψÛË ÙˆÓ Ó¢ÚÒÓˆÓ ·fi ÙÔ ∫+ ·Ú·Ù›ÓÂÈ ÙË ÓÂ˘ÚˆÓÈ΋ ÂÈ‚›ˆÛË, ÔfiÙÂ, ÔÈ ¯·ÌËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ∫+ ÚÔηÏÔ‡Ó ·fiÙˆÛË, Ì ¤Ó·Ó “ηٷÚÚ¿ÎÙË” ΢ÙÙ·ÚÈÎÒÓ ÁÂÁÔÓfiÙˆÓ. ¶ÚfiÛÊ·ÙË ‰ËÌÔÛ›Â˘ÛË (27) Û˘Û¯ÂÙ›˙ÂÈ ÙÔÓ NGF Ì ÙÔ Ê·ÈÓfiÌÂÓÔ ·Ú·ÁˆÁ‹˜ ÂÏ¢ı¤ÚˆÓ ÚÈ˙ÒÓ. ¶ÂÈÚ¿Ì·Ù· ÛÂ Â›Ì˘˜ ¤‰ÂÈÍ·Ó fiÙÈ Ô Û˘Ó‰˘·ÛÌfi˜ Ù˘ ˘ÂÚÔÍ›·˜ (Ô˘ ÂÏ·ÙÙÒÓÂÈ Ù· ›‰· ÙÔ˘ NGF) Î·È ÙˆÓ ·Ó·ÛÙÔϤˆÓ Ù˘ Û‡ÓıÂÛ˘ Ù˘ ÁÏÔ˘Ù·ıÂÈfiÓ˘ (Ô˘ ÚÔηÏÔ‡Ó ÂÔ̤ӈ˜ ÂÏ¿ÙÙˆÛË Ù˘ ÁÏÔ˘Ù·ıÂÈfiÓ˘), Ô‰ËÁ› Û ‰Ú·Ì·ÙÈ΋ ·‡ÍËÛË Ù˘ ·fiÙˆÛ˘ ÛÙÔÓ ÂÁΤʷÏÔ, ·ÔÙÂÏÒÓÙ·˜ Ì›· Èı·Ó‹ ÂÍ‹ÁËÛË ÁÓˆÛÙÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ Ô˘ ¤گÔÓÙ·È Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘. ŒÏÂÁ¯Ô˜ Ù˘ ÂÈÌ‹Î˘ÓÛ˘ Î·È Ù˘ ‰È·ÎÏ¿‰ˆÛ˘ ÙˆÓ Ó¢ÚÒÓˆÓ ∫·Ù¿ ÙËÓ ·È‰È΋ ËÏÈΛ·, Ë Û˘Ó·ÙÔÁ¤ÓÂÛË Â˘Ô‰ÒÓÂÙ·È ·Ú¿ÏÏËÏ· Ì ÙËÓ ·‡ÍËÛË ÙˆÓ ‰ÂÓ‰ÚÈÙÒÓ, ÙˆÓ Ó¢ڷÍfiÓˆÓ Î·È Ù˘ Ì˘ÂÏÈÓÔÔ›ËÛ˘, fiˆ˜ ›Û˘ ·˘Í¿ÓÔÓÙ·È ÔÈ ‰È·ÎÏ·‰ÒÛÂȘ ÙˆÓ Ó¢ÚÈÎÒÓ ‰ÈÎÙ‡ˆÓ Î·È Ë ‰È¿ÌÂÙÚÔ˜ ÙˆÓ Ó¢ڷÍfiÓˆÓ. ∏ Ï¢΋ Ô˘Û›· Á›ÓÂÙ·È ÈÔ ˘ÎÓ‹ Î·È ·˘Í¿ÓÂÙ·È Ë Ì˘ÂÏ›ÓË. ∫·Ù¿ ÙËÓ ÂÊ˂›· Î·È ÙËÓ ÂÓËÏÈΛˆÛË, ÂÍ·Ï›ÊÔÓÙ·È Û¯Â‰fiÓ ÔÈ ÌÈÛ¤˜ ·fi ÙȘ Û˘Ó¿„ÂȘ, ÔÈ ÈÔ “·‰‡Ó·Ì˜” ÏÂÈÙÔ˘ÚÁÈο, ÂÓÒ ÂΛӘ Ô˘ ÂÈ‚ÈÒÓÔ˘Ó ÙÚÔÔÔÈÔ‡ÓÙ·È ‚¿ÛÂÈ ÙÔ˘ fiÁÎÔ˘ ÙˆÓ ÏËÚÔÊÔÚÈÒÓ Ô˘ ‰¤¯ÔÓÙ·È ÛÙ· Ï·›ÛÈ· Ù˘ Û˘Ó·ÙÈ΋˜ Ï·ÛÙÈÎfiÙËÙ·˜. ∏ ˆÚ›Ì·ÓÛË ÙˆÓ Ó¢ÚÈÎÒÓ Ô‰ÒÓ Á›ÓÂÙ·È ÛÙ·‰È·Î¿, ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ÙÔ˘ ·ÔÛÙÔÏ‹ (28-30). ∫·Ù¿ ̤ÛÔ fiÚÔ, ηı¤Ó· ·fi Ù· ‰ÈÛÂηÙÔÌ̇ÚÈ· Ó¢ÚÈο ·ÙÙ·Ú· Û¯ËÌ·Ù›˙ÂÈ ÂÚ›Ô˘ 10.000 ÂȉÈΤ˜ ‰È·ÓÂ˘ÚˆÓÈΤ˜ Û˘Ó‰¤ÛÂȘ (1). ∞˘Ù‹ Ë ‰È·‰Èηۛ· ÌÔÚÊÔÏÔÁÈ΋˜ ‰È·ÊÔÚÔÔ›ËÛ˘ ··ÈÙ› ÙËÓ Î·Ù¢ı˘ÓfiÌÂÓË ·‡ÍËÛË ÛËÌ·ÓÙÈÎÔ‡ ·ÚÈıÌÔ‡ ΢ÙÙ·ÚÈÎÒÓ ·Ôϋ͈Ó. ¶Èı·ÓfiÓ ÔÈ ‰È¿ÊÔÚ˜ Ê¿ÛÂȘ Ù˘ ÂÓÂÚÁËÙÈ΋˜ ÂÈÌ‹Î˘ÓÛ˘ Ì›·˜ ΢ÙÙ·ÚÈ΋˜ ·fiÏË͢ ‰ËÌÈÔ˘ÚÁÔ‡Ó ÙÔ ÙÂÏÈÎfi ̤ÁÂıÔ˜ Î·È ÙË ÁˆÌÂÙÚ›· ÙÔ˘ ÓÂ˘Ú¿ÍÔÓ· Î·È ÙˆÓ ‰ÂÓ‰ÚÈÙÒÓ (31,32). ∂›Ó·È ÁÓˆÛÙfi fiÙÈ ÔÈ ·˘ÍËÙÈÎÔ› ÎÒÓÔÈ ‚Ú›ÛÎÔÓÙ·È ÛÙËÓ ·Ú¯‹ ÙÔ˘ Ó¢ڛÙË. ŸÙ·Ó fï˜ ÂÓÂÚÁÔÔÈÔ‡-
Paediatriki 2001;64:527-534
ÓÙ·È, Ê·ÓÂÚÒÓÔ˘Ó Â˘Î›ÓËÙ˜ ‰Ô̤˜, Ù· ÓËÌ·ÙÔÂȉ‹ Î·È ÂÙ·ÏÔÂȉ‹ „¢‰Ôfi‰È·. ∞˘Ù¤˜ ÔÈ ÏÂÙ¤˜, Ô‰ÔÂȉ›˜ ‰Ô̤˜ ‚Ú›ÛÎÔ˘Ó “ÌÔÓÔ¿ÙÈ·” ÁÈ· ÙËÓ ·‡ÍËÛË Î·È ÙË ‰È·ÎÏ¿‰ˆÛË ÙÔ˘ Ó¢ÚÒÓ·. O ¤ÏÂÁ¯Ô˜ Ù˘ ÌÔÚÊÔÏÔÁÈ΋˜ ‰È·ÊÔÚÔÔ›ËÛ˘ Â›Ó·È ÔÏ˘·Ú·ÁÔÓÙÈÎfi˜. ŒÙÛÈ, ÙÔÈΤ˜ ÎÏ›ÛÂȘ Û˘ÁΤÓÙÚˆÛ˘ ÙÔ˘ NGF ηÙ¢ı‡ÓÔ˘Ó ÙËÓ ·‡ÍËÛË Î·È ÙËÓ Î›ÓËÛË. ™ËÌ·ÓÙÈÎfi ÚfiÏÔ ·›˙ÂÈ, ›Û˘ Î·È Ë ËÏÂÎÙÚÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·, ·ÊÔ‡ Ë ·˘ıfiÚÌËÙË ˘ÚÔ‰fiÙËÛË ÙˆÓ Ó¢ÚÒÓˆÓ Î·Ù¿ ÙËÓ ·Ó¿Ù˘ÍË Û¯ËÌ·Ù›˙ÂÈ ÓÂ˘ÚˆÓÈο ΢ÎÏÒÌ·Ù· Î·È Û˘Ó‰¤ÛÂȘ Û fiÏÔ ÙÔÓ ÂÁΤʷÏÔ (33). ∆· ÌfiÚÈ· ΢ÙÙ·ÚÈ΋˜ ÚÔÛÎfiÏÏËÛ˘ (CAMs) Û˘Ì‚¿ÏÏÔ˘Ó ÛËÌ·ÓÙÈο ÛÙËÓ ·‡ÍËÛË ÙÔ˘ Ó¢ڛÙË, ÙÚÔÔÔÈÒÓÙ·˜ ÙȘ ¿ÏÏËÏÂȉڿÛÂȘ Ó¢ڛÙË-Ó¢ڛÙË Î·È Ó¢ڛÙË-Ó¢ÚÔÁÏÔ›·˜. ∂ȉÈο ÁÈ· ÙË Ï·ÌÈÓ›ÓË, ¤¯ÂÈ ‚ÚÂı› fiÙÈ Û˘ÓÂÚÁ¿˙ÂÙ·È ÛÙÂÓ¿ Ì ÙË ı˘ÚÔÍ›ÓË, Ë ÔÔ›· ÔÏ˘ÌÂÚ›˙ÂÈ ÙËÓ ·ÎÙ›ÓË ÙÔ˘ ΢ÙÙ·ÚÈÎÔ‡ ÛÎÂÏÂÙÔ‡ ÛÙ· ·ÛÙÚÔ·ÙÙ·Ú·. ªÂ ·˘Ùfi ÙÔÓ ÙÚfiÔ, Èı·ÓfiÓ Ú˘ıÌ›˙ÔÓÙ·È ÔÈ Û˘Ó‰¤ÛÂȘ ÙˆÓ Î˘ÙÙ¿ÚˆÓ Î·È Î·ıÔ‰ËÁÔ‡ÓÙ·È ÛˆÛÙ¿ ÚÔ˜ ÙȘ ÂÚÈÔ¯¤˜ ÌÂÙ·Ó¿ÛÙ¢Û˘ (34). ™ÙËÓ ·‡ÍËÛË ÂÌϤÎÂÙ·È Î·È Ë ·ÏÏËÏ›‰Ú·ÛË Ó¢ÚÒÓˆÓ-Ó¢ÚÔÁÏÔ›·˜. OÈ Û˘Ì·ıËÙÈÎÔ› Ó¢ÚÒÓ˜ Ô˘ ÌÂÁ·ÏÒÓÔ˘Ó ¯ˆÚ›˜ ·ÙÙ·Ú· Schwann, ÂÂÎÙ›ÓÔÓÙ·È ¯ˆÚ›˜ ‰È·ÎÏ·‰ÒÛÂȘ Ì ÙÔ˘˜ Ó¢ڛÙ˜ ˆ˜ ¿ÍÔÓ·. ¶·ÚÔ˘Û›· Ó¢ÚÔÁÏÔÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ, fï˜, ·Ú·ÙËÚÂ›Ù·È ÂÎÙÂٷ̤ÓË ‰È·ÎÏ¿‰ˆÛË ÙˆÓ ·Ôϋ͈Ó, Ô˘ ı˘Ì›˙ÂÈ ‰ÂÓ‰Ú›Ù˜. ∂ÈÚfiÛıÂÙ·, ÂÓ‰Ô΢ÙÙ¿ÚȘ Ô‰Ô› Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙË Ú‡ıÌÈÛË Ù˘ ·‡ÍËÛ˘ ÙÔ˘ Ó¢ڛÙË. ºˆÛÊÔÏÈ›‰È· ÈÓÔÛÈÙfiÏ˘ Î·È cAMP ¤¯Ô˘Ó ·Ó·ÊÂÚı› ˆ˜ ÂÓ‰Ô΢ÙÙ¿ÚÈÔÈ Ú˘ıÌÈÛÙ¤˜. ¶·Ú¿ÏÏËÏ·, ÚÔÛ˘Ó·ÙÈÎÔ› Ù‡ÔÈ ‰È·‡ÏˆÓ Ca2+, Ô˘ ÂÓÂÚÁÔÔÈÔ‡Ó ÙËÓ ·ÂÏ¢ı¤ÚˆÛË Ó¢Úԉȷ‚È‚·ÛÙÒÓ, ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ÍÈfiÏÔÁË ·Ó¿Ù˘ÍË ÛÙËÓ ÚÒÈÌË ÌÂÙ·ÁÂÓÓËÙÈ΋ ÂÚ›Ô‰Ô (35). ∂ÈϤÔÓ, ÙÔ Ca2+ Ú˘ıÌ›˙ÂÈ ÙËÓ ÂÈÌ‹Î˘ÓÛË ÙÔ˘ Ó¢ڛÙË Î·È ÙȘ ÎÈÓ‹ÛÂȘ ÙÔ˘ ·˘ÍËÙÈÎÔ‡ ÎÒÓÔ˘ (36). ∂ȉÈÎÔ› Ó¢Úԉȷ‚È‚·ÛÙ¤˜ ÌÔÚÔ‡Ó Ó· ÙÚÔÔÔÈ‹ÛÔ˘Ó ÙË Ó¢ÚÈÙÈ΋ ÂÈÌ‹Î˘ÓÛË Î·È ÙȘ ÎÈÓ‹ÛÂȘ ÙÔ˘ ·˘ÍËÙÈÎÔ‡ ÎÒÓÔ˘. ∏ ÛÂÚÔÙÔÓ›ÓË (5-∏∆) ÂÏ·ÙÙÒÓÂÈ ÙÔ Ú˘ıÌfi Ù˘ Û˘Ó·ÙÔÁ¤ÓÂÛ˘ Î·È ÙË Û˘Ó·ÙÈ΋ ˘ÎÓfiÙËÙ·, ‰ÚÒÓÙ·˜ ̤ۈ Ù˘ ·ÛÙÚÔÁÏÔȷ΋˜ ÚˆÙ½Ó˘ S-100‚ (37). ∞˘Ùfi˜ Ô Ó¢Úԉȷ‚È‚·ÛÙ‹˜ ÂËÚ¿˙ÂÈ ÙË ÓÂ˘ÚˆÓÈ΋ ΢ÙÙ·ÚÈ΋ ‰È·›ÚÂÛË (38), ÌÂÙ·Ó¿ÛÙ¢ÛË (39) Î·È ÚÔηÏ› ‚ÈÔ¯ËÌÈΤ˜ Î·È ÌÔÚÊÔÏÔÁÈΤ˜ ·ÏÏ·Á¤˜ ÛÙÔ˘˜ ·˘ÍËÙÈÎÔ‡˜ ÎÒÓÔ˘˜. ∏ ·ÎÂÙ˘ÏÔ¯ÔÏ›ÓË (ACh) ‰ÈÂÁ›ÚÂÈ ÙËÓ ·Ú·ÁˆÁ‹ ÚÔ‰ÚfiÌˆÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ÊÏÔÈÔ‡, ̤ۈ ÂÓÂÚÁÔÔ›ËÛ˘ ÙˆÓ ÌÔ˘ÛηÚÈÓÈÎÒÓ ˘Ô‰Ô¯¤ˆÓ (40). ∏ ¯ÔÏ›ÓË ·›˙ÂÈ ÚfiÏÔ ·ÁÁÂÏÈÔÊfiÚÔ˘ ηٿ ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ¡™ Î·È Î·Ù¿ ÙȘ ÂÚÈfi‰Ô˘˜ ·˘ÍË̤Ó˘
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Û˘Ó·ÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (41). ∂Í¿ÏÏÔ˘, Ì ·ÊÔÚÌ‹ ÙËÓ ·Ú·Ù‹ÚËÛË fiÙÈ Ë ·ÎÂÙ˘ÏÔ¯ÔÏÈÓÂÛÙÂÚ¿ÛË (AChE) Â›Ó·È È‰È·›ÙÂÚ· ·˘ÍË̤ÓË ÛÙË Ì¤Ï·ÈÓ· Ô˘Û›· (42), ÚÔÙ¿ıËΠ¤Ó·˜ ÂÓ·ÏÏ·ÎÙÈÎfi˜ ÚfiÏÔ˜ ÁÈ· ÙËÓ ACh, ÂÎÙfi˜ ·fi ÙÔ ÚfiÏÔ ÙÔ˘ Ó¢Úԉȷ‚È‚·ÛÙ‹. À¿Ú¯Ô˘Ó οÔÈÔÈ Ù‡ÔÈ AChE (ÌÔÓÔÌÂÚ‹ ¯·ÌËÏÔ‡ ÌÔÚÈ·ÎÔ‡ ‚¿ÚÔ˘˜) Ô˘ ·˘Í¿ÓÔ˘Ó ÙËÓ ÂÈ‚›ˆÛË Î·È ·‡ÍËÛË ÙˆÓ Ó¢ÚÒÓˆÓ, ·›˙Ô˘Ó ÂÔ̤ӈ˜ ÙÚÔÊÈÎfi (ÌË ¯ÔÏÈÓÂÚÁÈÎfi) ÚfiÏÔ. ∞Í›˙ÂÈ ·ÎfiÌË Ó· ÛËÌÂȈı› Ë Û¯¤ÛË ÙÔ˘ stress Ì ÙËÓ Ï·ÛÙÈÎfiÙËÙ· ÙˆÓ Ó¢ÚÒÓˆÓ (43). O ÈfiηÌÔ˜ ÙÔ˘ Â›Ì˘Ô˜ ·ÓÙ·ÔÎÚ›ÓÂÙ·È ÛÙȘ ÔÚÌfiÓ˜ ÙÔ˘ stress, ηıÒ˜ ›Û˘ ÛÙȘ ı˘ÚÂÔÂȉÈΤ˜, ÁÔÓ·‰ÈΤ˜ Î·È ÂÈÓÂÊÚȉȷΤ˜ ÔÚÌfiÓ˜, ·ÏÏ¿˙ÔÓÙ·˜ ÙÔ Û¯ËÌ·ÙÈÛÌfi ÙˆÓ Û˘Ó¿„ÂˆÓ Î·È ÙË ‰ÔÌ‹ ÙˆÓ ‰ÂÓ‰ÚÈÙÒÓ. ¢‡Ô Ù‡ÔÈ Û˘Ó·ÙÈ΋˜ Ï·ÛÙÈÎfiÙËÙ·˜ ÂËÚ¿˙ÔÓÙ·È ·fi ÙÔ stress: ·) ·ӷϷ̂·ÓfiÌÂÓÔ stress ÚÔηÏ› ·ÙÚÔÊ›· ÙˆÓ ‰ÂÓ‰ÚÈÙÒÓ ÛÙËÓ ÂÚÈÔ¯‹ CA3 ÙÔ˘ ÈÔοÌÔ˘ Î·È ‚) Ô͇ Î·È ¯ÚfiÓÈÔ stress ηٷÛÙ¤ÏÏÔ˘Ó ÙË Ó¢ÚÔÁ¤ÓÂÛË ÛÙËÓ Ô‰ÔÓوً ¤ÏÈη ÙÔ˘ ÈÔοÌÔ˘. ∏ ·Ú·ÁˆÁ‹ Ó¢ÚÒÓˆÓ ÛÙ·Ì·Ù¿ ÓˆÚ›˜ ηٿ ÙËÓ ÂÌ‚Ú˘ÔÁ¤ÓÂÛË (1), ÂÓÒ Ù· Ó¢ÚÔÁÏÔȷο ·ÙÙ·Ú· Û˘Ó¯›˙Ô˘Ó Ó· ‰È·ÈÚÔ‡ÓÙ·È, ·ÎfiÌ· Î·È ÛÙË ÌÂÙ·ÁÂÓÓËÙÈ΋ ÂÚ›Ô‰Ô ·Ó¿Ù˘Í˘. ∂ȉÈÎfiÙÂÚ·, Ù· ·ÛÙÚÔ·ÙÙ·Ú· π Î·È ππ ÂÌÊ·Ó›˙ÔÓÙ·È Ôχ ÓˆÚ›˜ ηٿ ÙËÓ ·Ó¿Ù˘ÍË, ÂÓÒ Ë ÁÂÓÈ¿ ÙˆÓ ÔÏÈÁÔ‰ÂÓ‰ÚÔ΢ÙÙ¿ÚˆÓ ·ÎÔÏÔ˘ı› ÙÚ›· ÛÙ¿‰È· ·Ó¿Ù˘Í˘: ·) ·Ú¯Èο ÂÌÊ·Ó›˙ÂÙ·È ÙÔ ÚÔ-ÔÏÈÁÔ‰ÂÓ‰ÚÔ·ÙÙ·ÚÔ, ‚) ·ÎÔÏÔ˘ı› ÙÔ ·ÓÒÚÈÌÔ (ÚÒÈÌÔ) ÔÏÈÁÔ‰ÂÓ‰ÚÔ·ÙÙ·ÚÔ Î·È Á) ÙÂÏÈο ÚÔ·ÙÂÈ ÙÔ ÒÚÈÌÔ ÔÏÈÁÔ‰ÂÓ‰ÚÔ·ÙÙ·ÚÔ, Ô˘ ·Ú¯›˙ÂÈ ÙÔ Û¯ËÌ·ÙÈÛÌfi Ù˘ Ì˘ÂÏÈÓÈ΋˜ ÌÂÌ‚Ú¿Ó˘. ∆· ÛÙÚÒÌ·Ù· Ì˘ÂÏ›Ó˘ ÚÔÛÙ·ÙÂ‡Ô˘Ó ÙÔ ÓÂ˘Ú¿ÍÔÓ· ·fi ÙËÓ ÂÎʇÏÈÛË (44). ∆· ÚÔ·Ó·ÊÂÚı¤ÓÙ· ÛÙ¿‰È· ·Ó¿Ù˘Í˘ Â›Ó·È ·Ó·ÛÙÚ¤„ÈÌ·, ·Ó¿ÏÔÁ· Ì ÙËÓ ·ÚÔ˘Û›· ‹ ·Ô˘Û›· ÔÚÈÛÌ¤ÓˆÓ ‰ÂÈÎÙÒÓ. ŒÓ·˜ ·fi ·˘ÙÔ‡˜ Â›Ó·È Ë ÁÏ˘ÎÔÚˆÙ½ÓË Ù˘ Ì˘ÂÏ›Ó˘ ÙˆÓ ÔÏÈÁÔ‰ÂÓ‰ÚÔ΢ÙÙ¿ÚˆÓ (MOG) Ô˘ ·ÔÙÂÏ› Î·È ‰Â›ÎÙË Ù˘ ˆÚ›Ì·ÓÛ‹˜ ÙÔ˘˜, ÂϤÁ¯ÂÈ Î·È ‰È·ÙËÚ› ÙË Ì˘ÂÏÈÓÔÔ›ËÛË, fiˆ˜ ›Û˘ Î·È ÙȘ ‰È·Î˘ÙÙ·ÚÈΤ˜ ÂȉڿÛÂȘ (45). ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ ·Ó·Á¤ÓÓËÛË ÙˆÓ Ó¢ÚÒÓˆÓ, Ô Martin Schwab Î·È ÔÈ Û˘ÓÂÚÁ¿Ù˜ ÙÔ˘ ¤‰ÂÈÍ·Ó fiÙÈ ˘¿Ú¯Ô˘Ó ‰‡Ô ÚˆÙ½Ó˜ ÛÙÔ ˘fiÛÙڈ̷ Ù˘ Ì˘ÂÏ›Ó˘ Ô˘ ÂÚÈ‚¿ÏÏÂÈ Ù· Ó‡ڷ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡, ÔÈ Ôԛ˜ ÂÌÔ‰›˙Ô˘Ó ÙËÓ ·Ó·Á¤ÓÓËÛË ·˘ÙÒÓ ÙˆÓ ÓÂ‡ÚˆÓ (46). ∞Ó ·˘Ù¤˜ ÔÈ ÚˆÙ½Ó˜ ·Ô˘ÛÈ¿ÛÔ˘Ó ‹ ·ÂÓÂÚÁÔÔÈËıÔ‡Ó, Â›Ó·È ‰˘Ó·ÙfiÓ Ó· Û˘Ì‚Â› Ë ·Ó·Á¤ÓÓËÛË ÙˆÓ ÓÂ‡ÚˆÓ ÙÔ˘ ÓˆÙÈ·›Ô˘ Ì˘ÂÏÔ‡, ÙÔ˘Ï¿¯ÈÛÙÔÓ ÂÓ Ì¤ÚÂÈ, ·ÊÔ‡ ˘¿Ú¯Ô˘Ó ÎÈ ¿ÏÏ· ÂÌfi‰È· ÁÈ· ÙË ÓÂ˘ÚˆÓÈ΋ ÙÔ˘˜ ·Ó¿Ù˘ÍË. ŒÙÛÈ, Ù· Ó¢ÚÈο ·ÙÙ·Ú· Èı·ÓfiÓ Ó· ·ÔÙ˘Á¯¿ÓÔ˘Ó Ó· ·Ó·ÁÂÓÓËıÔ‡Ó, ÏfiÁˆ ÙˆÓ ‰˘ÛÓfiËÙˆÓ ÎÏË-
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ÚÔÓÔÌÔ‡ÌÂÓˆÓ È‰ÈÔÙ‹ÙˆÓ ÙÔ˘˜ ‹ ÂÂȉ‹ ÛÙÂÚÔ‡ÓÙ·È ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ‹, ·ÎfiÌË, ÂÂȉ‹ ÏÂ›Ô˘Ó ÂΛӷ Ù· ÌfiÚÈ· Ô˘ ı· Ù· ‚ÔËı‹ÛÔ˘Ó Ó· Û˘ÁÎÚ·ÙËıÔ‡Ó Û ̛· ÂÈÊ¿ÓÂÈ· ηıÒ˜ ÌÂÁ·ÏÒÓÔ˘Ó. ¡ÂfiÙÂÚ˜ ·fi„ÂȘ Û¯ÂÙÈο Ì ÙË Ó¢ÚÔÁ¤ÓÂÛË ªÂϤÙ˜ Ì ÙÔÌÔÁÚ·Ê›· ÂÎÔÌ‹˜ Ô˙ÈÙÚÔÓ›ˆÓ (ƒ∂∆) ¤‰ÂÈÍ·Ó fiÙÈ Ë ·Ó¿Ù˘ÍË ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Û¯ÂÙ›˙ÂÙ·È ÛÙÂÓ¿ Ì ÙË ¯ÚËÛÈÌÔÔ›ËÛË Ù˘ ÁÏ˘Îfi˙˘. ¢È·ÈÛÙÒıËÎ·Ó ÌÂÙ·‚ÔÏÈΤ˜ ·ÏÏ·Á¤˜, Ô˘ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ ÂͤÏÈÍË Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜ ηٿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜. ∏ ˆÚ›Ì·ÓÛË ÙÔ˘ ¡™ Â›Ó·È Ì›· ‰˘Ó·ÌÈ΋ ‰È·‰Èηۛ·. ∂›Û˘, ˘¿Ú¯ÂÈ Û¯¤ÛË ÌÂٷ͇ Û˘Ó·ÙÔÁ¤ÓÂÛ˘ Î·È ÂȤ‰ˆÓ ÁÏ˘Îfi˙˘, Ô˘ Â›Ó·È Ê·ÓÂÚ‹ Û ÂÚÈfi‰Ô˘˜ Ì¿ıËÛ˘, fiÔ˘ Ë Ï·ÛÙÈÎfiÙËÙ· ÙˆÓ Û˘Ó¿„ÂˆÓ Â›Ó·È ¤ÓÙÔÓË (47). ª›· ¿ÏÏË ÛËÌ·ÓÙÈ΋ ·Ú¿ÌÂÙÚÔ˜ ÁÈ· ÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ Û˘Ó¿„ˆÓ, Â›Ó·È Ù· ›‰· ÙÔ˘ Ûȉ‹ÚÔ˘ (Fe) ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi. O Fe Û˘Ì‚¿ÏÏÂÈ ÛÙÔ Û¯ËÌ·ÙÈÛÌfi Î·È ÙË Û˘ÓÙ‹ÚËÛË Ù˘ Ì˘ÂÏ›Ó˘. ∂Ô̤ӈ˜, ÛÙË ÛȉËÚÔÂÓÈ΋ ·Ó·ÈÌ›·, ·Ó·Ì¤ÓÂÙ·È ·ÏÏ·Á‹ ÛÙË Ì˘ÂÏÈÓÔÔ›ËÛË, ‰ËÏ·‰‹ ÂÏ¿ÙÙˆÛË Ù˘ Ù·¯‡ÙËÙ·˜ ·ÁˆÁ‹˜ ÙˆÓ ÂÚÂıÈÛÌ¿ÙˆÓ, ηıÒ˜ ›Û˘ Ù˘ Ù·¯‡ÙËÙ·˜ ˆÚ›Ì·ÓÛ˘ ÙˆÓ Û˘Ó¿„ˆÓ, Û ‚Ú¤ÊË Ì ËÏÈΛ· ̤¯ÚÈ 6 ÌËÓÒÓ (48). ™¯ÂÙÈο Ì ÙÔ ÌËÙÚÈÎfi Á¿Ï·, ·Ó·Ê¤ÚÂÙ·È fiÙÈ Ù· ÏÈ›‰È· Ô˘ ÂÚȤ¯ÂÈ ·ÔÙÂÏÔ‡Ó Úfi‰ÚÔ̘ Ô˘Û›Â˜ ÚÔÛÙ·ÁÏ·Ó‰ÈÓÒÓ Î·È ÚÔÛÙ·Î˘ÎÏÈÓÒÓ, Ô˘ Û˘Ì‚¿ÏÏÔ˘Ó ÛÙË ‰fiÌËÛË ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ÌÂÌ‚Ú·ÓÒÓ Î·È ÂÍ·ÛÊ·Ï›˙Ô˘Ó ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ‚ÂÏÙ›ˆÛË ÙˆÓ Ó¢ÚÒÓˆÓ (49). ∂ӉȷʤÚÔ˘Û˜ Â›Ó·È ÔÈ ‚Ï·ÙÈΤ˜ ÂȉڿÛÂȘ ÙÔ˘ ·ÏÎÔfiÏ ÛÙË Ó¢ÚÔÁ¤ÓÂÛË. ∏ ÌÂÙ·Ó¿ÛÙ¢ÛË Î·È Ë ÛˆÛÙ‹ ηıÔ‰‹ÁËÛË ÙˆÓ Ó¢ڷÍfiÓˆÓ ·ÚÂÌÔ‰›˙ÔÓÙ·È, ÏfiÁˆ Ù˘ ·Ó·ÛÙÔÏ‹˜ Ù˘ Ï·ÌÈÓ›Ó˘ ·fi ÙÔ ·ÏÎÔfiÏ (50). ∂›Û˘, ÙÔ ·ÏÎÔfiÏ ÂÓÙ›ÓÂÈ ÙËÓ ·fiÙˆÛË Î·È ÙËÓ ÂÎʇÏÈÛË ÙˆÓ Ó¢ÚÒÓˆÓ, ÛÙË Ê¿ÛË Ù˘ Û˘Ó·ÙÔÁ¤ÓÂÛ˘ (·fi ÙÔÓ 6Ô Ì‹Ó· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ ̤¯ÚÈ Î·È ÌÂÚÈο ¯ÚfiÓÈ· ÌÂÙ¿ ÙË Á¤ÓÓËÛË). ∂›Û˘, ÚÔηÏ› ÙËÓ ÂÍ¿ÏÂÈ„Ë ÂηÙÔÌÌ˘Ú›ˆÓ Ó¢ÚÒÓˆÓ ·fi ÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ ÂÁΤʷÏÔ, ÂÏ·ÙÙÒÓÔÓÙ·˜ ÙË Û˘ÓÔÏÈ΋ Ì¿˙· ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È ÚÔ¿ÁÔÓÙ·˜ ‰È·Ù·Ú·¯¤˜ Ù˘ Û˘ÌÂÚÈÊÔÚ¿˜, fiˆ˜ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔ Û‡Ó‰ÚÔÌÔ ÙÔ˘ ·ÏÎÔÔÏÈÎÔ‡ ÓÂÔÁÓÔ‡-‚Ú¤ÊÔ˘˜ (51) (Ô˘ Â›Ó·È Ë Û˘Ó¤¯ÂÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Ù˘ ·ÏÎÔÔÏÈ΋˜ ÂÌ‚Ú˘Ô¿ıÂÈ·˜). ¶ÚÔÔÙÈΤ˜ ÛÙËÓ ¤Ú¢ӷ OÈ ·˘ÍËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ·Ô‰ÂÈÎÓ‡ÔÓÙ·È ¯Ú‹ÛÈÌ· ÂÚÁ·Ï›· ·ÏÏ¿ Î·È Û‡Á¯ÚÔÓ· ıÂڷ¢ÙÈο ̤۷ ÁÈ· ÙË ‰ÈÂÚ‡ÓËÛË Î·È ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘
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HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 20-02-2001 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 07-06-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ™Ù˘ÏÈ·Ófi˜ ∆۷ΛÚ˘ ∂ÚÁ·ÛÙ‹ÚÈÔ ¶ÂÈÚ·Ì·ÙÈ΋˜ º˘ÛÈÔÏÔÁ›·˜ π·ÙÚÈ΋ ™¯ÔÏ‹ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ∆.£. 65257, 154 01 ∞ı‹Ó· e-mail: stsakir@cc.uoa.gr
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PROGRESS IN MEDICAL SCIENCE
™‡Á¯ÚÔÓ˜ ·ÈÙÈÔÏÔÁÈΤ˜ Û˘Û¯ÂÙ›ÛÂȘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ∞. ¶Ô˘ÚÙÛ›‰Ë˜
Recent etiological correlations of childhood leukemia A. G. Pourtsidis
¶ÂÚ›ÏË„Ë: O ηÚΛÓÔ˜ Â›Ó·È Ë ‰Â‡ÙÂÚË ÛÂ Û˘¯ÓfiÙËÙ· ·ÈÙ›· ı·Ó¿ÙÔ˘ ·È‰ÈÒÓ, ÌÂÙ¿ Ù· ·Ù˘¯‹Ì·Ù·, ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜. OÈ Ï¢¯·È̛˜ ·ÔÙÂÏÔ‡Ó ÙË Û˘¯ÓfiÙÂÚË ÌÔÚÊ‹ ·È‰ÈÎÔ‡ ηÚΛÓÔ˘ ÛÙȘ ¯ÒÚ˜ ·˘Ù¤˜ Î·È ˘ÔÏÔÁ›˙ÔÓÙ·È ÛÙÔ ¤Ó· ÙÚ›ÙÔ ÙˆÓ ÂÚÈÙÒÛˆÓ. ¶·Ú¿ ÙȘ ÂÎÙÂٷ̤Ó˜ ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜, Ë ·ÈÙÈÔÏÔÁ›· Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ·Ú·Ì¤ÓÂÈ ˆ˜ › ÙÔ Ï›ÛÙÔÓ ·‰È¢ÎÚ›ÓÈÛÙË. ∞Ó·ÁÓˆÚÈṲ̂ÓÔÈ ·ÈÙÈÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ¤ÎıÂÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ Û ·ÎÙÈÓÔ‚ÔÏ›· Î·È ÔÚÈṲ̂Ó˜ ÁÂÓÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÂÍËÁÔ‡Ó ÌfiÓÔ ¤Ó· ÌÈÎÚfi ÔÛÔÛÙfi ÂÚÈÙÒÛÂˆÓ Ï¢¯·ÈÌ›·˜ Û ·È‰È¿. MÂϤÙ˜ ÛÙÔ ·ÚÂÏıfiÓ ÚfiÙÂÈÓ·Ó Î¿ÔÈÔ˘˜ ·ÈÙÈÔÏÔÁÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÁÈ· ÙËÓ ·È‰È΋ Ï¢¯·ÈÌ›·, ·ÏÏ¿ Ù· ·ÔÙÂϤÛÌ·Ù· ‹Ù·Ó ·ÛÙ·ı‹ Î·È ÌË ÂÈÛÙÈο. ∆Ô È‰È·›ÙÂÚÔ ÂӉȷʤÚÔÓ ÙˆÓ ÂÚ¢ÓËÙÒÓ ¤¯ÂÈ ÂÓÙÔÈÛÙ› ÛÙËÓ ·ÎÙÈÓÔ‚ÔÏ›· Î·È ÛÙÔ ÚfiÏÔ ÙˆÓ ÏÔÈÌÒÍÂˆÓ ÛÙÔ ·È‰› Î·È ÙË ÌËÙ¤Ú· ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ·ËÛ˘. Œ¯ÂÈ ÌÂÏÂÙËı› Ë Â›‰Ú·ÛË Ù˘ ¤ÎıÂÛ˘ Û ¿ÏϘ ÌÔÚʤ˜ ·ÎÙÈÓÔ‚ÔÏ›·˜, ÂÎÙfi˜ ·fi ÙËÓ ÈÔÓ›˙Ô˘Û·, fiˆ˜ Ë ¤ÎıÂÛË Û ÂÍ·ÈÚÂÙÈο ¯·ÌËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ËÏÂÎÙÚÈο Î·È Ì·ÁÓËÙÈο ‰›·, Û ÌÈÎÚÔ·̷ٷ ÙËÏÂÈÎÔÈÓˆÓÈ·ÎÒÓ Û˘ÛÙËÌ¿ÙˆÓ Î·È Ú·‰ÈÔÙËÏÂÔÙÈÎÒÓ ÂÎÔÌÒÓ, Û ʈÙÔıÂڷ›· Î·È ¯Ú‹ÛË ˘ÂÚ‹¯ˆÓ ÁÈ· ‰È·ÁÓˆÛÙÈÎÔ‡˜ ÏfiÁÔ˘˜. ∂›Û˘, ÌÂÏÂÙÒÓÙ·È ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜, ÂÚÈÁÂÓÓËÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ·È‰ÈÔ‡, ÂÚÈ‚·ÏÏÔÓÙÈΤ˜ ÂÎı¤ÛÂȘ ÙÔ˘ ·È‰ÈÔ‡ Î·È ÙˆÓ ÁÔÓ¤ˆÓ, ÂȉÈÎfiÙÂÚ· ‰Â, ÂÎı¤ÛÂȘ ÙÔ˘ ÂÌ‚Ú‡Ô˘ Î·È Ù˘ ÂÁ·Ԣ, ˆ˜ Î·È Ë Â·ÁÁÂÏÌ·ÙÈ΋ ¤ÎıÂÛË ÙˆÓ ÁÔÓ¤ˆÓ ÚÈÓ ÙË Û‡ÏÏË„Ë. ∏ ·Ó·ÛÎfiËÛË ·˘Ù‹ ÂÍÂÙ¿˙ÂÈ ÏÂÙÔÌÂÚÂȷο ÙËÓ ÚfiÔ‰Ô Ù˘ ÂȉËÌÈÔÏÔÁÈ΋˜ ¤Ú¢ӷ˜ ÛÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜.
Abstract: Cancer is the second most common cause of death, following accidents, among children in developed countries. The leukemias constitute the most common form of childhood cancer in these countries, accounting for about one third of the cases. In spite of extensive epidemiologic studies the etiology of childhood leukemia remains for the most part unclarified. Established causes such as exposure of the fetus to ionizing radiation, and certain genetic disorders explain only a small fraction of leukemia cases in children. Past studies suggested some causative factors but the results were unconvincing. Researchers today have focused their interest on the effect of radiation and the role of infections in the child and the mother during pregnancy. The effect of exposure to certain types of radiation, other than ionizing, have also been investigated, including extremely low frequency electric and magnetic fields, microwaves of telecommunication systems and radiotelevision transmission, phototherapy and use of ultrasound for diagnostic purposes. In addition, the role of socioeconomic factors of the family, perinatal characteristics of the child, environmental exposure of the child and the parents, especially exposure of the fetus and mother during pregnancy, as well as parental occupational exposure before conception are being investigated. This review surveys in detail the progress of epidemiologic research on the etiology of childhood leukemia.
∂ÈÌÂÏËÙ‹˜ OÁÎÔÏÔÁÈÎÔ‡ ∆Ì‹Ì·ÙÔ˜ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “¶ & ∞ ∫˘ÚÈ·ÎÔ‡”
Registrar, Division of Oncology Children’s Hospital of “P & A Kyriakou”, Athens
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¶·È‰È·ÙÚÈ΋ 2001;64:535-546
Paediatriki 2001;64:535-546
§¤ÍÂȘ ÎÏÂȉȿ: ·È‰È΋ Ï¢¯·ÈÌ›·, ÏÔÈÌ҉˘ ·ÈÙÈÔÏÔÁ›·, ËÏÂÎÙÚÔÌ·ÁÓËÙÈ΋ ·ÎÙÈÓÔ‚ÔÏ›·, ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎÔ› ·Ú¿ÁÔÓÙ˜.
Key words: childhood leukemia, infectious etiology, electromagnetic radiation, socioeconomic factors.
™˘ÓÙÔÌÔÁڷʛ˜
¶›Ó·Î·˜ 1: ∂Ȃ‚·ÈˆÌ¤ÓÔÈ Î·È ‡ÔÙÔÈ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜
O§§ Oª§ ∂Ù ∏ª¶ Ì∆ (microTesla)
ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· ÔÍ›· Ì˘ÂÏÔÁÂÓ‹˜ Ï¢¯·ÈÌ›· ÂÍ·ÈÚÂÙÈο ¯·ÌËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ËÏÂÎÙÚÈο Î·È Ì·ÁÓËÙÈο ‰›· ÌÔÓ¿‰· ̤ÙÚËÛ˘ Ì·ÁÓËÙÈÎÒÓ Â‰›ˆÓ
O ηÚΛÓÔ˜ Â›Ó·È Ë ‰Â‡ÙÂÚË ÛÂ Û˘¯ÓfiÙËÙ· ·ÈÙ›· ı·Ó¿ÙÔ˘ ·È‰ÈÒÓ, ÌÂÙ¿ Ù· ·Ù˘¯‹Ì·Ù·, ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜. OÈ Ï¢¯·È̛˜ ·ÔÙÂÏÔ‡Ó ÙË Û˘¯ÓfiÙÂÚË ÌÔÚÊ‹ ·È‰ÈÎÔ‡ ηÚΛÓÔ˘ ÛÙȘ ¯ÒÚ˜ ·˘Ù¤˜ Î·È ˘ÔÏÔÁ›˙ÔÓÙ·È ÛÙÔ ¤Ó· ÙÚ›ÙÔ ÙˆÓ ÂÚÈÙÒÛˆÓ. ∏ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· (O§§) Î·È Ë ÔÍ›· Ì˘ÂÏÔÁÂÓ‹˜ Ï¢¯·ÈÌ›· (Oª§) Â›Ó·È ÔÈ Û˘¯ÓfiÙÂÚÔÈ Ù‡ÔÈ, Ì ÙËÓ ÚÒÙË Ó· ·ÔÙÂÏ› ÙÔ 7075% Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ÛÙËÓ ∞ÌÂÚÈ΋ Î·È ÙËÓ ∂˘ÚÒË. ∏ Oª§ ·ÔÙÂÏ› ÙÔ 30-40% Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ÛÙËÓ ∞Û›·, ·ÏÏ¿ Â›Ó·È Û¯ÂÙÈο ·Û˘Ó‹ı˘ ÛÙȘ ‰˘ÙÈΤ˜ ¯ÒÚ˜ (1). ¶·Ú¿ ÙȘ ÂÎÙÂٷ̤Ó˜ ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Ô˘ ·Ó·ÎÂÊ·Ï·ÈÒıËÎ·Ó ÚfiÛÊ·Ù· ·fi ÙȘ Chow Î·È Û˘Ó (2) Î·È Linet Î·È Cartwright (3), Ë ·ÈÙÈÔÏÔÁ›· Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ·Ú·Ì¤ÓÂÈ ˆ˜ › ÙÔ Ï›ÛÙÔÓ ·‰È¢ÎÚ›ÓÈÛÙË. ∞Ó·ÁÓˆÚÈṲ̂ÓÔÈ ·ÈÙÈÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ¤ÎıÂÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ Û ·ÎÙÈÓÔ‚ÔÏ›· Î·È ÔÚÈṲ̂Ó˜ ÁÂÓÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, fiˆ˜ ÙÔ Û‡Ó‰ÚÔÌÔ Down Î·È Ë ·Ó·ÈÌ›· Fanconi, ÂÍËÁÔ‡Ó ÌfiÓÔ ¤Ó· ÌÈÎÚfi ÔÛÔÛÙfi ÂÚÈÙÒÛÂˆÓ Ï¢¯·ÈÌ›·˜ Û ·È‰È¿. MÂϤÙ˜ ÛÙÔ ·ÚÂÏıfiÓ ÚfiÙÂÈÓ·Ó Î¿ÔÈÔ˘˜ ·ÈÙÈÔÏÔÁÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÁÈ· ÙËÓ ·È‰È΋ Ï¢¯·ÈÌ›·, ·ÏÏ¿ Ù· ·ÔÙÂϤÛÌ·Ù· ‹Ù·Ó ·ÛÙ·ı‹ Î·È ÌË ÂÈÛÙÈο (¶›Ó·Î·˜ 1) (4). OÈ ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ ·Ó·˙‹ÙËÛ˘ ·ÈÙÈÔÏÔÁÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ¤¯Ô˘Ó ÛËÌ·ÓÙÈο ÌÂıÔ‰ÔÏÔÁÈο ÏÂÔÓÂÎÙ‹Ì·Ù·. ∆Ô ‰È¿ÛÙËÌ· ÌÂٷ͇ ¤ÎıÂÛ˘ Î·È ÂÌÊ¿ÓÈÛ˘ Ù˘ ÓfiÛÔ˘ Â›Ó·È ‚Ú·¯‡, Ë ·Ó¿ÌÓËÛË ÙˆÓ ÂÎı¤ÛÂˆÓ Â›Ó·È Î·Ï‡ÙÂÚË Î·È Ë Â›‰Ú·ÛË Û˘Á¯˘ÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÌÈÎÚfiÙÂÚË. ¢ÈÂıÓÒ˜, Ë Â›ÙˆÛË Ù˘ Ï¢¯·ÈÌ›·˜ ÛÙ· ·È‰È¿ ÔÈΛÏÏÂÈ, Î˘Ì·ÈÓfiÌÂÓË ·fi 15 ¤ˆ˜ 65 ÂÚÈÙÒÛÂȘ ·Ó¿ ÂηÙÔÌ̇ÚÈÔ. ¶ÚfiÛÊ·Ù· ·Ó·Ê¤ÚıËΠ·ÓÔ‰È΋ Ù¿ÛË ÛÙËÓ ∞ÌÂÚÈ΋ Î·È ¿ÏϘ ¯ÒÚ˜. ∫·Ù¿ ÙËÓ ÂÚ›Ô‰Ô ÙˆÓ ÂÙÒÓ 1974 ¤ˆ˜ 1991, Ë Â›ÙˆÛË Ù˘ O§§ ÛÙ· ·È‰È¿ ÁÈ· ÙËÓ ∞ÌÂÚÈ΋ ·˘Í·ÓfiÙ·Ó Î¿ı ¯ÚfiÓÔ 1,6% (5). ∞Ó Î·È Ë ·‡ÍËÛË ·˘Ù‹ Èı·ÓfiÓ Ó· Â›Ó·È Ú·ÁÌ·ÙÈ΋, ‰Â›¯ÓÔÓÙ·˜ Ì›· Èı·Ó‹ ·‡ÍËÛË ÂÎı¤ÛÂˆÓ ÛÙÔ˘˜ ·ÈÙÈÔÏÔÁÈο Û¯ÂÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘, ÔÈ ·ÏÏ·Á¤˜ ÛÙÔÓ ÙÚfiÔ Î·-
536
∂Ȃ‚·ÈˆÌ¤ÓÔÈ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ñ In utero ¤ÎıÂÛË Û ·ÎÙÈÓÔ‚ÔÏ›· ñ ªÂÙ¿ ÙË Á¤ÓÓËÛË ˘„ËÏ‹ ‰fiÛË ·ÎÙÈÓÔ‚ÔÏ›·˜ (.¯ ·ÎÙÈ ÓÔıÂڷ›· ) ñ ™‡Ó‰ÚÔÌÔ Down ñ ∞Ó·ÈÌ›· Fanconi ñ ∞Ù·Í›· - ÙËÏ·ÁÁÂÈÂÎÙ·Û›· ñ ¡Â˘ÚÔ˚ӈ̿وÛË ñ OÈÎÔÁÂÓ‹˜ ÌÔÓÔۈ̛· 7 ñ ÃËÌÂÈÔıÂڷ¢ÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ⁄ÔÙÔÈ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ñ ¶ÚÔ¯ˆÚË̤ÓË ËÏÈΛ· ÌËÙ¤Ú·˜ ñ À„ËÏfi ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎfi Â›Â‰Ô ñ ªÂÁ¿ÏÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ (>4000 gr) ñ ¶ÚˆÙfiÙÔη ·È‰È¿ ñ πÛÙÔÚÈÎfi ıÓËÛÈÁÂÓÔ‡˜ ÂÌ‚Ú‡Ô˘ ñ ∫¿ÓÈÛÌ· ÙˆÓ ÁÔÓ¤ˆÓ ηٿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË ñ ÃÚ‹ÛË ·ÏÎÔfiÏ ·fi ÙË ÌËÙ¤Ú· ηٿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË ñ ÃÚ‹ÛË Ì·ÚÈ¯Ô˘¿Ó·˜ ·fi ÙË ÌËÙ¤Ú· ηٿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË ñ ∏ÏÂÎÙÚÔÌ·ÁÓËÙÈο ‰›· ñ ∂ÓÙÔÌÔÎÙfiÓ· ñ ∂·ÁÁÂÏÌ·ÙÈ΋ ¤ÎıÂÛË ÙˆÓ ÁÔÓ¤ˆÓ (.¯ ‰È·Ï˘ÙÈο, ̤ٷÏÏ·, ˘‰ÚÔÁÔÓ¿ÓıڷΘ) ñ ¢È·ÈÙËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜
Ù·ÁÚ·Ê‹˜ ‹/Î·È Ë ‚ÂÏÙȈ̤ÓË ·Ó·ÊÔÚ¿ ÙˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ ›Ûˆ˜ ÂÍËÁÔ‡Ó, ÂÓ Ì¤ÚÂÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ, ÙËÓ ·‡ÍËÛË. ∏ ›وÛË Ù˘ Oª§ ÎÚ·Ù‹ıËΠÂÚ›Ô˘ ÛÙ·ıÂÚ‹ ·Ó Î·È ·Ó·Ê¤ÚıËΠοÔÈ· ·‡ÍËÛË Û Ó·ڿ ÎÔÚ›ÙÛÈ·. ¶ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó ÌÈÎÚ¤˜ Û¯ÂÙÈο ·ÔÎÏ›ÛÂȘ ÛÙË Û˘¯ÓfiÙËÙ· Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ÛÙ· ÎÚ¿ÙË Ù˘ ∂˘ÚÒ˘. OÈ ÌÂϤÙ˜ ·˘Ù¤˜, Ì ÛËÌ·ÓÙÈ΋ ÏËÚfiÙËÙ· ÛÙËÓ ÂÍ·ÎÚ›‚ˆÛË ÙˆÓ ÛÙÔȯ›ˆÓ, ·Ó¤ÊÂÚ·Ó Â›ÙˆÛË ·fi 31,0 ¤ˆ˜ 49,2 ·Ó¿ ÂηÙÔÌ̇ÚÈÔ (6). ∂›Ó·È Èı·Ófi, ÌÂÙ·‚ÔϤ˜ ÛÙÔÓ ÂÈÔÏ·ÛÌfi ‹ ÛÙ· ›‰· ¤ÎıÂÛ˘ ÔÚÈÛÌ¤ÓˆÓ ÂÚÈ‚·ÏÏÔÓÙÈÎÒÓ Ï¢¯·ÈÌÔÁfiÓˆÓ, ȉȷ›ÙÂÚ· ·˘Ù¿ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ O§§ Û ÌÈÎÚ¿ ·È‰È¿, Ó· ¢ı‡ÓÔÓÙ·È ÁÈ· ÙȘ ÚfiÛηÈÚ˜ Ù¿ÛÂȘ ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ ¯ÚfiÓˆÓ. ™ÙËÓ ∞ÌÂÚÈ΋ Î·È Û ¿ÏϘ ·ÓÂÙ˘Á̤Ó˜ ¯ÒÚ˜, ˘¿Ú¯ÂÈ ÛËÌ·ÓÙÈ΋ ÎÔڇʈÛË ÛÙËÓ Â›ÙˆÛË Ù˘ ·È‰È΋˜ O§§ ÌÂٷ͇ ÙˆÓ ËÏÈÎÈÒÓ 2 Î·È 5 ÂÙÒÓ, ÁÓˆÛÙ‹ Î·È Û·Ó ÎÔÈÓÔ‡ Ù‡Ô˘ (common) O§§. ™ÙËÓ ·È‰È΋ ËÏÈΛ·, Ë Â›ÙˆÛË Ù˘ O§§ ÛÙÔ˘˜ Ó¤ÁÚÔ˘˜ Â›Ó·È ÛÙ·ıÂÚ¿ ÛÙÔ ‹ÌÈÛ˘ ÂΛӢ ÙˆÓ Ï¢ÎÒÓ. ∂›Û˘, ÙÔ ˘„ËÏfi ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎfi Â›Â‰Ô Û˘Û¯ÂÙ›ÛÙËΠ̠·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ O§§
¶·È‰È·ÙÚÈ΋ 2001;64:535-546
Paediatriki 2001;64:535-546
ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ÌÂÏÂÙÒÓ. OÈ ·Ú·ÙËÚ‹ÛÂȘ ·˘Ù¤˜ ‰Â›¯ÓÔ˘Ó fiÙÈ ÔÈ Û˘Û¯ÂÙ›ÛÂȘ Ì ÙË ‚ÈÔÌ˯·ÓÔÔ›ËÛË Ù˘ ˙ˆ‹˜ ›Ûˆ˜ Ô‰ËÁÔ‡Ó ÛÙËÓ ËÏÈÎȷ΋ ·˘Ù‹ ¤Í·ÚÛË Î·È ¤ÙÛÈ Â›¯·Ó Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙË ‰È·ÌfiÚʈÛË ÔÚÈÛÌ¤ÓˆÓ ÛËÌ·ÓÙÈÎÒÓ ˘Ôı¤ÛÂˆÓ Û¯ÂÙÈο Ì ÙËÓ Î·ı˘ÛÙÂÚË̤ÓË ¤ÎıÂÛË Û ÏÔÈÌÒ‰ÂȘ ·Ú¿ÁÔÓÙ˜ (7,8). ∏ ÔÍ›· Ï¢¯·ÈÌ›·, fiˆ˜ Î·È ¿ÏÏÔÈ Î·ÚΛÓÔÈ, Â›Ó·È Ì›· ÂÍÂÏÈÛÛfiÌÂÓË ÎψÓÈ΋ ‰È·Ù·Ú·¯‹, Ë ÔÔ›· ηıÔ‰ËÁÂ›Ù·È ·fi ÌÂÙ·ÏÏ¿ÍÂȘ (9). ¶¿ÓÙˆ˜, ¤Ó· ÌÈÎÚfi ·ÏÏ¿ ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi (̤¯ÚÈ Î·È 5%) ÙˆÓ ÔͤˆÓ Ï¢¯·ÈÌÈÒÓ Û¯ÂÙ›˙ÔÓÙ·È Ì ÎÏËÚÔÓÔÌÔ‡ÌÂÓ·, ÚԉȷıÂÛÈο ÁÂÓÂÙÈο Û‡Ó‰ÚÔÌ·, fiˆ˜ Û‡Ó‰ÚÔÌÔ Down, Û‡Ó‰ÚÔÌÔ Bloom, Û‡Ó‰ÚÔÌÔ Schwachman, Ó¢ÚÔ˚ӈ̿وÛË, ·Ù·Í›· ÙËÏ·ÁÁÂÈÂÎÙ·Û›· Î·È Èı·ÓfiÓ ÙÔ Û‡Ó‰ÚÔÌÔ Klinefelter’s (10). O ·ÈÙÈÔÏÔÁÈÎfi˜ Ì˯·ÓÈÛÌfi˜ Â›Ó·È Èı·Ófiٷٷ ÔÏ˘·Ú·ÁÔÓÙÈÎfi˜ (™¯‹Ì· 1). OÈ Ì˯·ÓÈÛÌÔ› Ô˘ ÂÌϤÎÔÓÙ·È ÛÙËÓ ·Ú¯È΋ ÌÂÙ¿ÏÏ·ÍË ‰È·Ê¤ÚÔ˘Ó ·fi ÂΛÓÔ˘˜ Ô˘ ÚÔηÏÔ‡Ó ‹ ÚÔˆıÔ‡Ó ·ÎÔÏÔ˘ı›Û˜ ÌÂÙ·ÏÏ¿ÍÂȘ Î·È Ù· ¯ÚÔÓÈο fiÚÈ· Ô˘ ηχÙÔ˘Ó ÙȘ Û¯ÂÙÈΤ˜ ÂÎı¤ÛÂȘ, ÙȘ Èı·Ó¤˜ ÌÂÙ·ÏÏ¿ÍÂȘ Î·È ÙËÓ ÎÏÈÓÈ΋ ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ ÌÔÚ› Ó· Â›Ó·È Ôχ ÂÎÙÂٷ̤ӷ. ø˜ ÂÎ ÙÔ‡ÙÔ˘, ÙÔ ˙ËÙÔ‡ÌÂÓÔ Â›Ó·È Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ - Èı·Ófiٷٷ ÔÏÏ·ÏÒÓ - ·ÈÙ›ˆÓ ‹ Ì˯·ÓÈÛÌÒÓ Ù˘ ÌÂÙ¿ÏÏ·Í˘ ÛÙÔ Û‡ÓÔÏÔ Ù˘ Ê˘ÛÈ΋˜ ÈÛÙÔÚ›·˜ Ù˘
ÓfiÛÔ˘. ∞Ó Î·È Ë ·ÈÙÈÔÏÔÁ›· Ù˘ ÔÍ›·˜ Ï¢¯·ÈÌ›·˜ Â›Ó·È ¿ÁÓˆÛÙË, ÔÚÈṲ̂ÓÔÈ ·Ú¿ÁÔÓÙ˜ ¤¯Ô˘Ó ÂÓÔ¯ÔÔÈËı› Û ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ, fiˆ˜ Ë ·ÎÙÈÓÔ‚ÔÏ›·, ¯ËÌÈο ΢ڛˆ˜ ÔÚÁ·ÓÈÎÔ› ‰È·Ï‡Ù˜ (‚ÂÓ˙›ÓË) Î·È ÙÔ˘Ï¿¯ÈÛÙÔÓ ¤Ó·˜ Èfi˜, Ô Èfi˜ Ù˘ ∆ Ï¢¯·ÈÌ›·˜ ÙˆÓ ÂÓËÏ›ÎˆÓ (HTLV-I). ∆Ô È‰È·›ÙÂÚÔ ÂӉȷʤÚÔÓ ÙˆÓ ÂÚ¢ÓËÙÒÓ ¤¯ÂÈ ÂÓÙÔÈÛÙ› ÛÙËÓ ·ÎÙÈÓÔ‚ÔÏ›· ÈÔÓ›˙Ô˘Û· Î·È ÌË - Î·È ÛÙÔ ÚfiÏÔ ÙˆÓ ÏÔÈÌÒÍÂˆÓ ÛÙÔ ·È‰›, ÙË ÌËÙ¤Ú· Î·È ÙÔ ÂÚÈ‚¿ÏÏÔÓ Ô˘ ˙ÂÈ. ÕÏÏÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ÌÂÏÂÙÒÓÙ·È Â›Ó·È ÁÔÓÂ˚ο ¯·Ú·ÎÙËÚÈÛÙÈο, ÂÚÈ‚·ÏÏÔÓÙÈΤ˜ ÂÎı¤ÛÂȘ ÙÔ˘ ·È‰ÈÔ‡ Î·È ÙˆÓ ÁÔÓ¤ˆÓ, ÂȉÈÎfiÙÂÚ· ‰Â ÂÎı¤ÛÂȘ ÙÔ˘ ÂÌ‚Ú‡Ô˘ Î·È Ù˘ ÂÁ·Ԣ ˆ˜ Î·È Ë Â·ÁÁÂÏÌ·ÙÈ΋ ¤ÎıÂÛË ÙˆÓ ÁÔÓ¤ˆÓ. πÔÓ›˙Ô˘Û· ·ÎÙÈÓÔ‚ÔÏ›· H ÌÂϤÙË Ù˘ ηÚÎÈÓÔÁfiÓÔ˘ ‰Ú¿Û˘ Ù˘ ÈÔÓ›˙Ô˘Û·˜ ·ÎÙÈÓÔ‚ÔÏ›·˜ ÚÔÛÂÏ·ÂÈ ÙÔ ÂӉȷʤÚÔÓ ÙÔ˘ È·ÙÚÈÎÔ‡ Î·È ÂÈÛÙËÌÔÓÈÎÔ‡ ÎfiÛÌÔ˘. ∏ ηÚÎÈÓÔÁfiÓÔ˜ ›‰Ú·ÛË Û¯ÂÙÈο ÌÂÁ¿ÏˆÓ ‰fiÛÂˆÓ ·ÎÙÈÓÔ‚ÔÏ›·˜ ÛÙÔÓ ¿ÓıÚˆÔ ¤¯ÂÈ ÙÂÎÌËÚȈı› Ï‹Úˆ˜, ÂÓÒ ÔÈ ÌÈÎÚ¤˜ ‰fiÛÂȘ Â›Ó·È Ôχ Èı·ÓfiÓ, ·ÏÏ¿ fi¯È ·Ô‰Â‰ÂÈÁ̤ÓÔ, fiÙÈ Â›Û˘ ·ÛÎÔ‡Ó Î·ÚÎÈÓÔÁfiÓÔ Â›‰Ú·ÛË. ∏ Û˘Û¯¤ÙÈÛË ¯·ÌËÏÒÓ ‰fiÛÂˆÓ ·ÎÙÈÓÔ‚ÔÏ›·˜ Î·È ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ·Ú·Ì¤ÓÂÈ ·ÌÊÈÏÂÁfiÌÂÓË Î·È ÁÂÓÈο ‰ÂÓ ÌÔÚԇ̠ӷ ·ÔÎÏ›ÛÔ˘Ì ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÚÈṲ̂Ó˜ È·ÙÚÈΤ˜
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∆˘¯·›Ô ÁÂÁÔÓfi˜ ™¯‹Ì· 1. ∞ÈÙÈÔÏÔÁÈÎfi˜ Ì˯·ÓÈÛÌfi˜ ηÚΛÓÔ˘.
537
¶·È‰È·ÙÚÈ΋ 2001;64:535-546
ÂÊ·ÚÌÔÁ¤˜ Î·È ÂÍÂÙ¿ÛÂȘ Û¯ÂÙ›˙ÔÓÙ·È Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ Î·ÚΛÓÔ˘ ÛÙ· ·È‰È¿. ∏ Èı·ÓfiÙËÙ· ·˘ÍË̤ÓÔ˘ ÎÈÓ‰‡ÓÔ˘ Ï¢¯·ÈÌ›·˜ ·fi ¯·ÌËϤ˜ ‰fiÛÂȘ ‰È·ÁÓˆÛÙÈ΋˜ ·ÎÙÈÓÔ‚ÔÏ›·˜, ·ÁÁÂÏÌ·ÙÈ΋ ¤ÎıÂÛË ÙˆÓ ÂÚÁ·˙ÔÌ¤ÓˆÓ Û ˘ÚËÓÈο ÂÚÁÔÛÙ¿ÛÈ·, ¤ÎıÂÛË ·fi ˘ÚËÓÈΤ˜ ‰ÔÎÈ̤˜ ‹ ‰È·ÚÚÔ¤˜ ÌÂÙ¿ ·fi ·Ù‡¯ËÌ· ˘ÚËÓÈÎÒÓ ÛÙ·ıÌÒÓ Î·È ·fi Ê˘ÛÈ΋ ‹ ÙÔ˘ ˘Â‰¿ÊÔ˘˜ ·ÎÙÈÓÔ‚ÔÏ›·, ‰ËÌÈÔ‡ÚÁËÛ ÔÏϤ˜ ‰È·Ì¿¯Â˜ (11). ∏ ¤ÎıÂÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ Û ·ÎÙÈÓÔÁڷʛ˜ Â›Ó·È ¤Ó·˜ ·fi ÙÔ˘˜ ÂÏ¿¯ÈÛÙÔ˘˜ ÂȂ‚·ÈˆÌ¤ÓÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ·È‰È΋ Ï¢¯·ÈÌ›· Î·È ÔÈ ÈÔ ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó 40 ¤ˆ˜ 50% ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÌÂÙ¿ ·fi 2-3 ·ÎÙÈÓÔÁڷʛ˜ ÎÔÈÏ›·˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ (12). O ΛӉ˘ÓÔ˜ ·˘Ùfi˜, fï˜, Û˘Ó¯Ҙ ÌÂÈÒÓÂÙ·È, Èı·Ófiٷٷ ÏfiÁˆ Ì›ˆÛ˘ Ù˘ ¯Ú‹Û˘ ÙˆÓ ·ÎÙÈÓÔÁÚ·ÊÈÒÓ ÛÙË Ì·È¢ÙÈ΋ Ú·ÎÙÈ΋ Î·È ÙˆÓ Û˘Ó‹ıˆ˜ ¯ÚËÛÈÌÔÔÈÔ‡ÌÂÓˆÓ ‰fiÛÂˆÓ ·ÎÙÈÓÔ‚ÔÏ›·˜. ªÂÙ¿ ÙÔ 1996 ‰ËÌÔÛȇıËÎ·Ó ·ÚÎÂÙ¤˜ ÌÂϤÙ˜ Û¯ÂÙÈο Ì ÙË ‰È·Ù˘ˆı›۷ ˘fiıÂÛË fiÙÈ Ë ¤ÎıÂÛË ÙˆÓ ·È‰ÈÒÓ ÛÙËÓ ·ÎÙÈÓÔ‚ÔÏ›· ·fi ÙÔ ˘ÚËÓÈÎfi ·Ù‡¯ËÌ· ÙÔ˘ Chernobyl ÙÔ 1986 Û¯ÂÙ›˙ÂÙ·È Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· Ï¢¯·ÈÌ›·. OÈ ÌÂϤÙ˜ ·˘Ù¤˜ ‰ÂÓ ·Ó·Ê¤ÚÔ˘Ó Î¿ÔÈ· ˘ÔÏÔÁ›ÛÈÌË ·‡ÍËÛË ÛÙÔÓ Î›Ó‰˘ÓÔ Ï¢¯·ÈÌ›·˜ ÛÙ· ·È‰È¿ Ô˘ ˙Ô‡Û·Ó Û ÂÚÈÔ¯¤˜ Ô˘ ¤‰Ú·Û ÙÔ Ú·‰ÈÂÓÂÚÁfi Ó¤ÊÔ˜ ηٿ ÙË ÛÙÈÁÌ‹ ÙÔ˘ ·Ù˘¯‹Ì·ÙÔ˜ (13). ∂ÓÙÔ‡ÙÔȘ, Ì›· ÚfiÛÊ·ÙË ÌÂϤÙË ÛÙË ¯ÒÚ· Ì·˜ ·Ó·Ê¤ÚÂÈ ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ‚ÚÂÊÈ΋˜ Ï¢¯·ÈÌ›·˜ ηٿ 2,6 ÊÔÚ¤˜ Ô˘ Û¯ÂÙ›ÛÙËΠ̠ÙËÓ ¤ÎıÂÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÛÙÔ Ú·‰ÈÂÓÂÚÁfi Ó¤ÊÔ˜ ÙÔ˘ Chernobyl (14). ŒÎıÂÛË Û ·ÎÙÈÓÔ‚ÔÏ›· ÏfiÁˆ ‰È·ÁÓˆÛÙÈÎÒÓ ·ÎÙÈÓÔÁÚ·ÊÈÒÓ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ‰ÂÓ Ê·›ÓÂÙ·È Ó· Û¯ÂÙ›˙ÂÙ·È Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· Ï¢¯·ÈÌ›· (15). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÙÂÏÂ˘Ù·›·˜ ‰ÂηÂÙ›·˜, Û˘¯Ó¿ ÂÌÊ·Ó›˙ÂÙ·È ÛÔ‚·Ú‹ ‰È·Ì¿¯Ë fiÛÔÓ ·ÊÔÚ¿ ÛÙËÓ Â›‰Ú·ÛË Ô˘ ¤¯ÂÈ ÁÈ· ÙÔÓ Î›Ó‰˘ÓÔ Ï¢¯·ÈÌ›·˜ ÛÙ· ·È‰È¿ Ë ¤ÎıÂÛË ÙÔ˘ ·Ù¤Ú· Û ¯·ÌËϤ˜ ‰fiÛÂȘ ·ÎÙÈÓÔ‚ÔÏ›·˜ ηٿ ÙËÓ ÂÚ›Ô‰Ô ÚÈÓ ÙË Û‡ÏÏË„Ë. ∆Ô˘Ï¿¯ÈÛÙÔÓ ‰‡Ô ÌÂϤÙ˜ ·Ó¤ÊÂÚ·Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ·fi ¤ÎıÂÛË ÙˆÓ ·Ù¤ÚˆÓ Û ·ÎÙÈÓÔ‚ÔÏ›·, ›Ù ÁÈ· ·ÁÁÂÏÌ·ÙÈÎÔ‡˜ ›Ù ÁÈ· ‰È·ÁÓˆÛÙÈÎÔ‡˜ ÏfiÁÔ˘˜ (16). ∆¤ÏÔ˜, ‰ÂÓ ˘¿Ú¯Ô˘Ó ÂÈÚ·Ì·ÙÈο ‰Â‰Ô̤ӷ Ô˘ Ó· ÂÓÔ¯ÔÔÈÔ‡Ó ÙË Ê˘ÛÈ΋ Á‹ÈÓË ‹ ÎÔÛÌÈ΋ ·ÎÙÈÓÔ‚ÔÏ›· Û·Ó ÂȂ‚·ÈˆÌ¤ÓË ·ÈÙ›· Ï¢¯·ÈÌ›·˜, ·ÏÏ¿ Ë Ì¤ÛË ÂÙ‹ÛÈ· ¤ÎıÂÛË (~1 mSv) ÈÛÙ‡ÂÙ·È fiÙÈ ÚÔηÏ› ¤Ó· ÌÈÎÚfi, ·ÏÏ¿ ÛËÌ·ÓÙÈÎfi, (~5%) ÔÛÔÛÙfi ·fi ÙË Û˘ÓÔÏÈ΋ ›وÛË Ù˘ Ï¢¯·ÈÌ›·˜ (11). §Ô›ÌˆÍË ∏ ‰È·Ù˘ˆı›۷ ȉ¤·, ÁÈ· ÙËÓ ÔÔ›· ˘¿Ú¯Ô˘Ó Û·Ê›˜ - ·Ó Î·È ¤ÌÌÂÛ˜ - ÂȉËÌÈÔÏÔÁÈΤ˜ ÂӉ›-
538
Paediatriki 2001;64:535-546
ÍÂȘ, Â›Ó·È fiÙÈ ÔÏϤ˜ ÂÚÈÙÒÛÂȘ Ï¢¯·ÈÌ›·˜ ÎÔÈÓÔ‡ Ù‡Ô˘ ÛÙ· ·È‰È¿ ÂÌÊ·Ó›˙ÔÓÙ·È Û·Ó Û˘Ó¤ÂÈ· Û¿ÓÈ·˜ ·ıÔÏÔÁÈ΋˜ ·¿ÓÙËÛ˘ Û οÔÈ· ÎÔÈÓ‹ Ïԛ̈ÍË (17,18). ∏ Ïԛ̈ÍË ÌÔÚ› Ó· Â›Ó·È ·Û˘Ó‹ı˘ Î·È fi¯È ÙfiÛÔ ÛÔ‚·Ú‹ ÙË ÛÙÈÁÌ‹ Ô˘ Û˘Ì‚·›ÓÂÈ, ·ÏÏ¿ ÁÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ‹ Ë È‰È·›ÙÂÚË Â˘·ÈÛıËÛ›· ÙÔ˘ ÍÂÓÈÛÙ‹ ›Ûˆ˜ ÂËÚ¿˙Ô˘Ó ÙËÓ ·ÓÒÌ·ÏË ·¿ÓÙËÛË Ô˘ Ô‰ËÁ› ÛÙË Ï¢¯·ÈÌ›· (19). ∂›Û˘, ¤¯ÂÈ ‰È·Ù˘ˆı› Ë ¿Ô„Ë fiÙÈ Ë ·È‰È΋ Ï¢¯·ÈÌ›· Î·È È‰È·›ÙÂÚ· Ë O§§ Û ·È‰È¿ 2 ¤ˆ˜ 5 ¯ÚfiÓˆÓ, Èı·ÓfiÓ ÔÊ›ÏÂÙ·È ÛÙËÓ ¤ÏÏÂÈ„Ë ÚÔÛÙ·Û›·˜ ¤Ó·ÓÙÈ ÎÔÈÓÒÓ ÏÔÈÌÒÍÂˆÓ Ô˘ ÚԂϤÂÙ·È ·fi ÙËÓ ·ıËÙÈ΋ ·ÓÔÛ›· ÔÌ¿‰·˜ (8) ‹ fiÙÈ ÔÊ›ÏÂÙ·È ÛÙËÓ Î·ı˘ÛÙÂÚË̤ÓË ·ÓÙÈÁÔÓÈ΋ ÚfiÎÏËÛË Û ÎÔÈÓ¤˜ ÏÔÈÌÒÍÂȘ Ù˘ Û˘Ó‹ıÔ˘˜ ¤ÎıÂÛ˘ ηٿ ÙË ‚ÚÂÊÈ΋ ÂÚ›Ô‰Ô (17). ∆Ô 1988, Ô Kinlen (8) ‰È·Ù‡ˆÛ ÙËÓ È‰¤· fiÙÈ Ë ·È‰È΋ Ï¢¯·ÈÌ›· Èı·ÓfiÓ ÔÊ›ÏÂÙ·È Û ̛· Û¿ÓÈ· ·ÓÙ›‰Ú·ÛË Û οÔÈ· ·ÚÔÛ‰ÈfiÚÈÛÙË Ïԛ̈ÍË ‹ ÏÔÈÌÒÍÂȘ. OÈ ÂȉË̛˜ ·fi ÈÒÛÂȘ Â›Ó·È Èı·ÓfiÙÂÚ˜ fiÙ·Ó ÊÔÚ›˜ Î·È Â˘·›ÛıËÙ· ¿ÙÔÌ· ¤Ú¯ÔÓÙ·È Û ·ʋ ÌÂÙ¿ ·fi ÌÂÁ¿ÏË ·Ó¿ÌÂÈÍË ÏËı˘ÛÌÒÓ (ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÌÂÙ·Ó¿ÛÙ¢Û˘). ªÂ ·˘Ù¤˜ ÙȘ Û˘Óı‹Î˜ ı· ÌÔÚÔ‡Û ӷ ˘¿ÚÍÂÈ ·ÓÙ›ÛÙÔȯ· ˘„ËÏ‹ ›وÛË Ï¢¯·ÈÌ›·˜ Û ·È‰È¿, ÙˆÓ ÔÔ›ˆÓ Ë ·ÓÔÛ›· Â›Ó·È ÂÚÈÔÚÈṲ̂ÓË. ¡¤· ‰Â‰Ô̤ӷ ‰Â›¯ÓÔ˘Ó fiÙÈ Ì·˙È΋ ·Ó¿ÌÂÈÍË ·ÔÌÔÓˆÌ¤ÓˆÓ ‹ ·ÁÚÔÙÈÎÒÓ ÏËı˘ÛÌÒÓ Ì ·ÛÙÈΤ˜ ÔÌ¿‰Â˜ ·˘Í¿ÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜, fiˆ˜ Û˘Ì‚·›ÓÂÈ Û ÓÂÔ·ÛÙÈÎÔÔÈË̤Ó˜ ·ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜, fiÏÂȘ Ì ÌÂÁ¿ÏË ·‡ÍËÛË ÙˆÓ ı¤ÛÂˆÓ ÂÚÁ·Û›·˜ Î·È ÂÚÈÔ¯¤˜ Ì ÌÂÁ¿ÏÔ ·ÚÈıÌfi ÛÙÚ·ÙȈÙÒÓ, ÙÔ˘ÚÈÛÙÒÓ Î·È ÚÔÛʇÁˆÓ ÔϤÌÔ˘ (20). ™ËÌ·ÓÙÈ΋ Û˘ÛÛÒÚ¢ÛË - Û ¯ÚfiÓÔ Î·È Û ÙfiÔ - Ó¤ˆÓ ÂÚÈÙÒÛÂˆÓ O§§ Û ·È‰È¿ 0 ¤ˆ˜ 4 ¯ÚfiÓˆÓ, ·Ó·ÎÔÈÓÒıËÎÂ Î·È ÛÙË ¯ÒÚ· Ì·˜ fiÙ·Ó ÌÂÏÂÙ‹ıËÎ·Ó 872 ÂÚÈÙÒÛÂȘ ÂȉËÌÈÔÏÔÁÈο, ¤ÌÌÂÛ· ˘ÔÛÙËÚ›˙ÔÓÙ·˜ ÙËÓ ˘fiıÂÛË ÁÈ· ÎÔÈÓfi ÏÔÈÌÔÁfiÓÔ ·Ú¿ÁÔÓÙ· (21). ¶ÔÏϷϤ˜ ÂӉ›ÍÂȘ, fiˆ˜ ‰ÈÂıÓ›˜ Û˘ÁÎÚ›ÛÂȘ ›وÛ˘/Û˘¯ÓfiÙËÙ·˜, ‰È·¯ÚÔÓÈΤ˜ Ù¿ÛÂȘ, ÁˆÁÚ·ÊÈΤ˜ ηٷÓÔ̤˜, Û˘Û¯ÂÙ›ÛÂȘ Ì ¯·Ú·ÎÙËÚÈÛÙÈο Ù˘ ÎÔÈÓfiÙËÙ·˜ fiˆ˜ ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈ΋ ηٿÛÙ·ÛË Î·È ‚ÈÔÏÔÁÈΤ˜ ·Ú·ÙËÚ‹ÛÂȘ, ‰Â›¯ÓÔ˘Ó Û˘ÓÔÏÈο fiÙÈ Ë ÎÔÈÓÔ‡ Ù‡Ô˘ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Â›Ó·È ÓfiÛÔ˜ ÏÔ‡ÛÈˆÓ ÎÔÈÓˆÓÈÒÓ Û·Ó ÁÂÓÈ΋ ·ÓÙ›ÏË„Ë, ÂÌÂÚȤ¯ÔÓÙ·˜ ÛÙËÓ ·ÈÙÈÔ·ıÔÁ¤ÓÂÈ¿ Ù˘ Ì›· Û¿ÓÈ· ·¿ÓÙËÛË Û ÎÔÈÓ‹ Ïԛ̈ÍË/ÏÔÈÌÒÍÂȘ. ∏ ˘fiıÂÛË ·˘Ù‹, Ô˘ Û¯ËÌ·ÙÈο ‰›ÓÂÙ·È ÛÙÔ ™¯‹Ì· 2 (9), ÙÔÓ›˙ÂÈ ÙË ÛÔ‚·ÚfiÙËÙ· ÙÔ˘ ÙÚfiÔ˘ Î·È ÙÔ˘ ¯ÚfiÓÔ˘ ÙˆÓ ÏÔÈÌÒÍÂˆÓ ÛÙËÓ ÚÒÙË ·È‰È΋ ËÏÈΛ· Û ۯ¤ÛË Ì ÙËÓ ·Ó·Ù˘Íȷ΋ ‰È·‰Èηۛ· ÙÔ˘ ·ÓÔÛÔÏÔÁÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ∆Ô ‚ÈÔÏÔÁÈο “Ê˘ÛÈÔÏÔÁÈÎfi” ÁÈ· ÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÎÔÈÓ¤˜ ‹ ÂÓ‰ËÌÈΤ˜
¶·È‰È·ÙÚÈ΋ 2001;64:535-546
Paediatriki 2001;64:535-546
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∞Ô˘Û›· ·ÓÔÛÔÏÔÁÈ΋˜ Ú‡ıÌÈÛ˘ ÛÙ· ‚Ú¤ÊË
“∫·ı˘ÛÙÂÚË̤ÓË” ¤ÎıÂÛË Û ÏÔÈÌÒÍÂȘ
➝
➝
“À„ËÏ‹” ··ÓÙËÙÈ΋ ηٿÛÙ·ÛË
➝ ➝
ÀÂÚ·ÓÙȉڷÛÙÈ΋ ·ÓÔÛÔÏÔÁÈ΋ ·¿ÓÙËÛË
∞ÓÔÛÔÏÔÁÈ΋ ·ÔÚ‡ıÌÈÛË Ù˘ ÏÂÌÊÔÔ›ËÛ˘ ÙˆÓ µ-΢ÙÙ¿ÚˆÓ: ŒÓÙÔÓË ˘ÂÚÏ·Û›· ÙˆÓ µ ·Ú¯¤ÁÔÓˆÓ Î˘ÙÙ¿ÚˆÓ
➝ “∂ÈÏÔÁ‹” ÙÔ˘ ˘ÂÚÏ·ÛÙÈÎÔ‡ ÎÏÒÓÔ˘ Û ·Ó·ÛÙÔÏ‹ ‰È·ÊÔÚÔÔ›ËÛ˘
➝ ∫ÔÈÓÔ‡ Ù‡Ô˘ O§§ (common)
™¯‹Ì· 2. ∞ÓÔÛÔÏÔÁÈÎfi ÚfiÙ˘Ô ÁÈ· ÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ ·È‰È΋˜ ÎÔÈÓÔ‡ Ù‡Ô˘ ÔÍ›·˜ ÏÂÌÊÔ‚Ï·ÛÙÈ΋˜ Ï¢¯·ÈÌ›·˜.
ÏÔÈÌÒÍÂȘ Â›Ó·È Ë Ù˘¯·›· ·ʋ Ì ·˘Ù¤˜ ÂÚÈÁÂÓÓËÙÈο, ‰È¿ ̤ÛÔ˘ Ù˘ ÌËÙ¤Ú·˜ ‹ ηٿ ÙË ‚ÚÂÊÈ΋ ËÏÈΛ· ‰È¿ ÙÔ˘ ÌËÙÚÈÎÔ‡ ıËÏ·ÛÌÔ‡, ÙˆÓ ·‰ÂÏÊÒÓ ‹ ¿ÏÏˆÓ ·È‰ÈÒÓ (‚ÚÂÊÔÓËÈ·ÎÔ› ÛÙ·ıÌÔ›). ¶ÚÒÈ̘ ÂÎı¤ÛÂȘ ı· Ú˘ıÌ›ÛÔ˘Ó ‹ ı· ÚÔÂÙÔÈÌ¿ÛÔ˘Ó ÙËÓ ·ÓÔÛÔÏÔÁÈ΋ ·¿ÓÙËÛË ÙÔ˘ ‚Ú¤ÊÔ˘˜ ÛÙȘ Èı·Ó¤˜ ÌÂÏÏÔÓÙÈΤ˜ ÂÎı¤ÛÂȘ. OÈ ·ÏÏ·Á¤˜ ·˘Ù¤˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Â¤ÎÙ·ÛË Î·È Î·Ù·ÛÙÔÏ‹ ÔÚÈÛÌ¤ÓˆÓ ˘ÔÔÌ¿‰ˆÓ ∆ ΢ÙÙ¿ÚˆÓ ‹ ÎÏÒÓˆÓ. ™˘Ó‹ıÂȘ Î·È ÙÚfiÔ˜ ˙ˆ‹˜ ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜, fiˆ˜ Ô ıËÏ·ÛÌfi˜ Î·È Ë ·Ó·ÙÚÔÊ‹ ÙÔ˘ ·È‰ÈÔ‡, ·fi ·Ì¤ÏÂÈ· ÂÎı¤ÙÔ˘Ó Û ΛӉ˘ÓÔ ·˘Ù‹ ÙËÓ ÂÍÂÏÈÎÙÈ΋ ÚÔÛ·ÚÌÔÁ‹ ÙÔ˘ ·ÓÔÛÔÏÔÁÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ∫·Ù’ ·Ú¯¿˜, ¤ÁÎ˘Â˜ ÌËÙ¤Ú˜ ›Ûˆ˜ ‰ÂÓ ¤¯Ô˘Ó ÂÎÙÂı› Î·È ¤ÙÛÈ ‰ÂÓ Â›Ó·È ÈηӤ˜ Ó· ‰ÒÛÔ˘Ó ·ÓÔÛÔÏÔÁÈ΋ ÚÔÛÙ·Û›· ‹ Ú‡ıÌÈÛË. ∂ÈÚfiÛıÂÙ·, ÔÈ ÂÎı¤ÛÂȘ ÙˆÓ ›‰ÈˆÓ ÙˆÓ ·È‰ÈÒÓ Û ÏÔÈÌÒÍÂȘ ¤¯Ô˘Ó ÂÏ·ÙÙˆı› ÏfiÁˆ Ù˘ ‚ÂÏÙ›ˆÛ˘ ÙˆÓ Û˘ÓıËÎÒÓ ˘ÁÈÂÈÓ‹˜ Î·È ÙˆÓ ·ÏÏ·ÁÒÓ ÛÙÔ˘˜ ÙÚfiÔ˘˜ ÎÔÈÓˆÓÈÎÒÓ Â·ÊÒÓ. OÈ ÌÂÙ·‚ÔϤ˜ ·˘Ù¤˜ Û˘Ì‚·›ÓÔ˘Ó ÙËÓ ›‰È· ÛÙÈÁÌ‹ Ô˘ ‰ÈÂıÓÒ˜ ·Ú·ÙËÚ›ٷÈ, ÁÈ· ÎÔÈÓˆÓÈÎÔ‡˜ ÏfiÁÔ˘˜, ÂÚÈÔÚÈÛÌfi˜ ÙÔ˘ ·Ú·ÙÂٷ̤ÓÔ˘ ıËÏ·ÛÌÔ‡. ∂›Ó·È ÁÓˆÛÙfi fiÙÈ Ô ÌËÙÚÈÎfi˜ ıËÏ·ÛÌfi˜ ¤¯ÂÈ ‰È·ÙÚÔÊÈΤ˜ Î·È ·ÓÔÛÔÏÔÁÈΤ˜ ÂȉڿÛÂȘ
ÛÙÔ ‚Ú¤ÊÔ˜, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ÌÂÙ¿‰ÔÛ˘ ‚·ÎÙËÚȉ›ˆÓ Î·È ÈÒÓ, Ô˘ Èı·ÓfiÓ ·ÔÙÂÏ› ÙËÓ ÚÒÈÌË ·Ú¯È΋ ·ʋ. ª›· ÂÓ·ÏÏ·ÎÙÈ΋ ÂÍ‹ÁËÛË ÙÔ˘ ™¯‹Ì·ÙÔ˜ 2 ‰fiıËΠ·fi ÙÔ˘˜ Greaves Î·È Û˘Ó (17). ∆Ô Úfi‚ÏËÌ· ‰ÂÓ Â›Ó·È Ë ¤ÏÏÂÈ„Ë ·ÓÔÛÔÏÔÁÈ΋˜ ·¿ÓÙËÛ˘, ·ÏÏ¿ Ì›· ˘ÂÚ‚ÔÏÈ΋ ‹ ÌË ÂÍÈÛÔÚÚÔË̤ÓË ·ÓÙ›‰Ú·ÛË Û ÔÚÈṲ̂ӷ ÏÔÈÌÒ‰Ë ·ÓÙÈÁfiÓ· ‹ ˘ÂÚ·ÓÙÈÁfiÓ·. À¿Ú¯Ô˘Ó ÔÏÏÔ› Èı·ÓÔ› Ì˯·ÓÈÛÌÔ›, ·ÓÔÛÔÏÔÁÈÎÔ› ‹ ‰È·Ì¤ÛÔ˘ ΢ÙÔÎÈÓÒÓ, Ô˘ ¿ÁÔ˘Ó ˘ÂÚϷۛ˜ ÛÙ· ·Ú¯¤ÁÔÓ· ÏÂÌÊÔÔÈËÙÈο ·ÙÙ·Ú· ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ. ∏ ˘ÂÚÏ·ÛÙÈ΋ ·˘Ù‹ ·ÓÙ›‰Ú·ÛË Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÌÂÙ·ÙÚ¤„ÂÈ Ê˘ÛÈÔÏÔÁÈο ·ÙÙ·Ú· ۠Ϣ¯·ÈÌÈο ‰È·Ì¤ÛÔ˘ ÌÂÙ¿ÏÏ·Í˘ ‹ ÂÓ·ÏÏ·ÎÙÈο ÌÔÚ› Ó· ÏÂÈÙÔ˘ÚÁ‹ÛÂÈ Û·Ó ÚÔ·ÁˆÁ¤·˜ ÚÔ¸·Ú¯fiÓÙˆÓ ÌÂÙ·ÏÏ·ÁÌ¤ÓˆÓ ·˘ÙÔÌ¿Ùˆ˜ ÎÏÒÓˆÓ. ∏ ·È‰È΋ Ï¢¯·ÈÌ›· ›Ûˆ˜ Â›Ó·È ÙÔ ·ÔÙ¤ÏÂÛÌ· ‰‡Ô ÌÂÙ·ÏϿ͈Ó, Ë ÚÒÙË ÂÓ‰ÔÌËÙÚÈο Î·È Ë ‰Â‡ÙÂÚË ÌÂÙ¿ ÙË Á¤ÓÓËÛË. ∏ ÙÂÏÂ˘Ù·›· ·Ô‰›‰ÂÙ·È Û ‰È·Ù·Ú·¯¤˜ ηٿ ÙËÓ ÂͤÏÈÍË ÙÔ˘ ·ÓÔÛÔÏÔÁÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. O Greaves, ÙÔ 1988, ‰ËÌÔÛ›Â˘Û ̛· ˘fiıÂÛË (22) Û‡Ìʈӷ Ì ÙËÓ ÔÔ›· Ë ÈÔ ÎÔÈÓ‹ ÌÔÚÊ‹ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ (Ë ÎÔÈÓÔ‡ Ù‡Ô˘ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·, Ô˘ Û˘Ó‹ıˆ˜ ÂÌÊ·Ó›˙ÂÙ·È Î·Ù¿ ÙËÓ ÚÒÈÌË ·È‰È΋ ËÏÈΛ·) ›Ûˆ˜ ÔÊ›ÏÂÙ·È Û ‰‡Ô ͯˆÚÈÛÙ¿ ÁÂÓÂÙÈο Û˘Ì‚¿Ì·Ù· (·˘ÙfiÌ·Ù˜ ÌÂÙ·ÏÏ¿ÍÂȘ). ∏ ÚÒÙË ıˆÚÂ›Ù·È ‰Â‰Ô̤ÓÔ fiÙÈ Â›Ó·È ·˘ÙfiÌ·ÙË Î·È Û˘Ì‚·›ÓÂÈ Î·Ù¿ ÙËÓ ÂÌ‚Ú˘˚΋ ˙ˆ‹, fiÙ·Ó Ù· µ ·Ú¯¤ÁÔÓ· ·ÙÙ·Ú· ÔÏÏ·Ï·ÛÈ¿˙ÔÓÙ·È Ù·¯‡Ù·Ù· ÛÙÔ ‹·Ú Î·È ÙÔ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ. O ›‰ÈÔ˜ ıˆÚ› fiÙÈ ·˘Ùfi˜ Ô ÌÂÙ·ÏÏ·Á̤ÓÔ˜ ÎÏÒÓÔ˜ ÙÔ˘ ÚÒÈÌÔ˘ µ ΢ÙÙ¿ÚÔ˘ ı· ˘ÂÚÔÏÏ·Ï·ÛÈ·ÛÙ› fiÙ·Ó ·ÚÁfiÙÂÚ· ÂÎÙÂı› Û οÔÈÔ ·ÓÙÈÁÔÓÈÎfi ÂÚÂıÈÛÌfi-ÚfiÎÏËÛË (.¯. ¤Ó· ÏÔÈÌÒ‰Ë ·Ú¿ÁÔÓÙ·). ∏ ηı˘ÛÙÂÚË̤ÓË ¤ÎıÂÛË Û ÏÔÈÌÒ‰ÂȘ ·Ú¿ÁÔÓÙ˜ Î·È Ë ÌÂÁ·Ï‡ÙÂÚË ·ÓÔÛÔÏÔÁÈ΋ ÚfiÎÏËÛË ¤¯Ô˘Ó Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙÔ ÌË ÂÏÂÁ¯fiÌÂÓÔ Î˘ÙÙ·ÚÈÎfi ÔÏÏ·Ï·ÛÈ·ÛÌfi. ∞˘Ùfi, ÛÙË Û˘Ó¤¯ÂÈ·, Ô‰ËÁ› ÛÙË ‰Â‡ÙÂÚË ÌÂÙ¿ÏÏ·ÍË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ÎÏÒÓÔ˘ Ô˘ ‹‰Ë ›¯·Ó ÂÂÎÙ·ı› Û·Ó Û˘Ó¤ÂÈ· Ù˘ ÚÒÙ˘ ÌÂÙ¿ÏÏ·Í˘ Î·È ÙÔ ·ÔÙ¤ÏÂÛÌ· Â›Ó·È Ë ÎÏÈÓÈ΋ ÂÌÊ¿ÓÈÛË Ù˘ Ï¢¯·ÈÌ›·˜. ¶ÈÛÙ‡ÂÈ ‰Â fiÙÈ ·˘Ù‹ Ë ÎÚ›ÛÈÌË ÂÚ›Ô‰Ô˜ ÚÔ·ÁˆÁ‹˜ ›Ûˆ˜ Û˘Ì‚·›ÓÂÈ ÙÔ ‰Â‡ÙÂÚÔ ‹ ÙÚ›ÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜ ·ÎÔÏÔ˘ıÒÓÙ·˜ ÙËÓ ·Ú¯È΋ ·ʋ ÙÔ˘ ‚Ú¤ÊÔ˘˜ Ì ‰È¿ÊÔÚ· ·ÓÙÈÁfiÓ·. ∂›Û˘, Ô ›‰ÈÔ˜ ıˆÚ› fiÙÈ ·È‰È¿ Ô˘ ¤¯Ô˘Ó ÂÎÙÂı› Û ÏÔÈÌÒÍÂȘ ηٿ ÙË ‚ÚÂÊÈ΋ ËÏÈΛ· ¤¯Ô˘Ó ‹‰Ë ·Ó·Ù‡ÍÂÈ ÌÂÚÈ΋ ·ÓÔÛ›· Î·È ¤ÙÛÈ Ù· µ ·ÙÙ·Ú¿ ÙÔ˘˜ ‰ÂÓ Â˘Ú›ÛÎÔÓÙ·È Û ¤ÓÙÔÓË ˘ÂÚÏ·ÛÙÈ΋ Ê¿ÛË fiÙ·Ó ÂÎÙ›ıÂÓÙ·È Û ÏÔÈÌÒ‰ÂȘ ·Ú¿ÁÔÓÙ˜ ·ÚÁfiÙÂÚ· ÛÙËÓ ÚÒÈÌË ·È‰È΋ ËÏÈΛ·. ∆Ô˘Ï¿¯ÈÛÙÔÓ Ì›· ÌÂϤÙË (23) Ô˘ Â›Ó·È Û˘Ì‚·Ù‹ Ì ÙËÓ ·ÓˆÙ¤Úˆ ˘fiıÂÛË,
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¤‰ÂÈÍ fiÙÈ Ë ·Ú·ÎÔÏÔ‡ıËÛË ·È‰ÈÎÔ‡ ÛÙ·ıÌÔ‡ Û ÚÒÈÌË ËÏÈΛ· Û¯ÂÙ›˙ÂÙ·È Ì ¯·ÌËÏfiÙÂÚÔ Î›Ó‰˘ÓÔ. ∂›Û˘, Û˘Û¯ÂÙ›ÛÂȘ Ì ÙË ÛÂÈÚ¿ Á¤ÓÓËÛ˘ Î·È ÙÔ ·ÓÒÙÂÚÔ ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎfi Â›Â‰Ô ›Ûˆ˜ ÂÍËÁÔ‡ÓÙ·È ·fi ÙËÓ Î·ı˘ÛÙÂÚË̤ÓË ¤ÎıÂÛË Û ÏÔÈÌÒÍÂȘ, ÏfiÁˆ ÌÈÎÚÔ‡ ·ÚÈıÌÔ‡ ·‰ÂÏÊÒÓ Î·È/‹ ηı˘ÛÙÂÚË̤ÓË ÂÈÛ·ÁˆÁ‹ Û ‚ÚÂÊÔÓËÈ·Îfi ÛÙ·ıÌfi (24). ¶·Ú¿ÁÔÓÙ˜ fiˆ˜ Ô ÌËÙÚÈÎfi˜ ıËÏ·ÛÌfi˜, ÂÌ‚ÔÏÈ·ÛÌÔ›, ·ÏÏÂÚÁ›Â˜, ÏÔÈÌÒÍÂȘ, ¤ÎıÂÛË Û ˙Ò·, ÛÂÈÚ¿ Á¤ÓÓËÛ˘ ¤¯Ô˘Ó ‹‰Ë ÂÚ¢ÓËı› ÛÂ Û˘Û¯¤ÙÈÛË Ì ÙËÓ ˘fiıÂÛË Greaves. ∆· ·ÔÙÂϤÛÌ·Ù· ·˘ÙÒÓ ÙˆÓ ÌÂÏÂÙÒÓ Â›Ó·È ÁÂÓÈο ·ÓÙÈÊ·ÙÈο. ™Â Ì›· ÌÂϤÙË ÂÚÈÙÒÛÂˆÓ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜, Ô˘ ‰È·ÁÓÒÛÙËÎ·Ó ÌÂÙ¿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜, ‚Ú¤ıËΠÈÛ¯˘Ú¿ ıÂÙÈ΋ Û˘Û¯¤ÙÈÛË Ì ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ÚÒÙÔ˘ ÂÍ·Ì‹ÓÔ˘ Ù˘ ˙ˆ‹˜, ÂÓÒ ¿ÏÏË ÌÂϤÙË ‰ÂÓ ¤‰ÂÈÍ ÔÔÈ·‰‹ÔÙÂ Û˘Û¯¤ÙÈÛË Ì ÎÔÈÓ¿ ÎÚ˘ÔÏÔÁ‹Ì·Ù·, ˘ÚÂÙÈο ÂÂÈÛfi‰È·, ·È‰ÈΤ˜ ·ÚÚÒÛÙȘ (ÈÏ·Ú¿, ·ÓÂÌÔ‚ÏÔÁÈ¿, ·ÚˆÙ›Ùȉ·, ÂÚ˘ıÚ¿) Î·È ÛÔ‚·Ú¤˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ Ù˘ ˙ˆ‹˜ ÁÈ· ÙȘ Ôԛ˜ ··ÈÙ‹ıËΠ·Ú·ÌÔÓ‹ Û ÓÔÛÔÎÔÌÂ›Ô (25). ∏ ˘fiıÂÛË fiÙÈ ÔÈ ÂÌ‚ÔÏÈ·ÛÌÔ› Ì BCG, DTP ‹ ÔÈ ÂÌ‚ÔÏÈ·ÛÌÔ› ÁÈ· ¿ÏÏ· ÈÔÁÂÓ‹ ÓÔÛ‹Ì·Ù· Èı·ÓfiÓ ÚÔÛÙ·ÙÂ‡Ô˘Ó ·fi ÙËÓ ·È‰È΋ Ï¢¯·ÈÌ›· ‰ÂÓ ÂȂ‚·ÈÒıËΠÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜. ∆¤ÏÔ˜, Û˘Û¯¤ÙÈÛË Î·ÎÔËıÂÈÒÓ ÙÔ˘ ÏÂÌÊÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÛÙ· ·È‰È¿ Î·È ¤ÎıÂÛË Û ¿ÚÚˆÛÙË Á¿Ù· ‹ Û˘Ó‡·ÚÍË Ì ˙Ò· ÛÙÔ Û›ÙÈ ¤¯ÂÈ ·Ó·ÊÂÚı›, ·ÏÏ¿ Û ÌÈÎÚfi ·ÚÈıÌfi ·ÛıÂÓÒÓ Î·È ¯ˆÚ›˜ ÛˆÛÙ‹ ÂÍ·ÎÚ›‚ˆÛË ÙˆÓ ÛÙÔȯ›ˆÓ (26). °ÔÓÂ˚ÎÔ› Î·È ¿ÏÏÔÈ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ∫·ÓÈÛÙÈΤ˜ Û˘Ó‹ıÂȘ ÁÔÓ¤ˆÓ ¢ÂÓ ˘¿Ú¯ÂÈ ÛÙ·ıÂÚfiÙËÙ· ¢ÚËÌ¿ÙˆÓ ÌÂٷ͇ ηӛÛÌ·ÙÔ˜ ÙˆÓ ÁÔÓ¤ˆÓ Î·È Ï¢¯·ÈÌ›·˜ ÛÙÔ˘˜ ·ÔÁfiÓÔ˘˜. ∞ÚÎÂÙ¤˜ ÌÂϤÙ˜ ·Ó¤ÊÂÚ·Ó ıÂÙÈ΋ Û˘Û¯¤ÙÈÛË Î·È ¿ÏϘ fi¯È (27). OÈ ·Ú¯ÈΤ˜, fï˜, ÌÂϤÙ˜ ÂÛÙ›·Û·Ó ΢ڛˆ˜ ÙÔ ÂӉȷʤÚÔÓ ÙÔ˘˜ ÛÙËÓ ¤ÎıÂÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ Î·È ‰ÂÓ ÌÔÚÔ‡Û·Ó Ó· ‰È·¯ˆÚ›ÛÔ˘Ó ÙȘ ‰˘ÓËÙÈο ‚Ï·‚ÂÚ¤˜ Û˘Ó¤ÂȘ ·fi ÙÔ Î¿ÓÈÛÌ· ÙˆÓ ÁÔÓ¤ˆÓ Î·È ÙÔ ·ıËÙÈÎfi οÓÈÛÌ· ÙÔ˘ ·È‰ÈÔ‡ (28). ¶ÚfiÛÊ·Ù·, ‰‡Ô ÌÂϤÙ˜ ÛÙËÓ ∞ÁÁÏ›· Î·È ÙËÓ ∫›Ó· ·Ó¤ÊÂÚ·Ó fiÙÈ ÙÔ Î¿ÓÈÛÌ· ÙÔ˘ ·Ù¤Ú· ÙËÓ ÂÚ›Ô‰Ô ÚÈÓ ÙË Û‡ÏÏË„Ë Û˘Û¯ÂÙÈ˙fiÙ·Ó Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ·È‰ÈÎÔ‡ ηÚΛÓÔ˘. ∏ ÌÂϤÙË ·fi ÙËÓ ∫›Ó· (29) ‹Ù·Ó ͯˆÚÈÛÙ‹ ÁÈ·Ù› ̤¯ÚÈ ÂΛÓË ÙË ÛÙÈÁÌ‹ Ï›Á˜ Á˘Ó·›Î˜ οÓÈ˙·Ó Î·È ¤ÙÛÈ Ë Â›‰Ú·ÛË ÙÔ˘ ηӛÛÌ·ÙÔ˜ ·fi ÙÔÓ ·Ù¤Ú· ‹Ù·Ó ‰˘Ó·Ùfi Ó· ÂÎÙÈÌËı› ·ÓÂÍ¿ÚÙËÙ· ·fi οıÂ Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ÌËÙÚÈÎÔ‡ ηӛÛÌ·ÙÔ˜. OÈ Û˘ÁÁÚ·Ê›˜ ÙˆÓ ‰‡Ô ·˘ÙÒÓ ÌÂÏÂÙÒÓ ıˆÚÔ‡Ó fiÙÈ ÙÔ Î¿ÓÈÛÌ· ›Ûˆ˜ ÚÔηÏ› ÁÂÓÂÙÈ΋ ‚Ï¿‚Ë ÛÙ· ÛÂÚÌ·ÙÔ˙ˆ¿ÚÈ· Î·È ·˘Ùfi Ô‰ËÁ› Û ۇÌÊ˘Ù˜
540
Paediatriki 2001;64:535-546
ÌÂÙ·ÏÏ¿ÍÂȘ, Ô˘ Â›Ó·È Ë ·ÈÙ›· ‹ Úԉȷı¤ÙÔ˘Ó Û ηÚΛÓÔ, ÛÙÔ˘˜ ·ÔÁfiÓÔ˘˜. ∏ ¤Ó‰ÂÈÍË ·˘Ù‹˜ Ù˘ ˘fiıÂÛ˘ ÈÛ¯˘ÚÔÔÈÂ›Ù·È ·fi ÛÙÔȯ›· ÂÚ¢ÓÒÓ in vitro, ÛÙȘ Ôԛ˜ ÙÔ Î¿ÓÈÛÌ· ‚Ú¤ıËΠӷ ·˘Í¿ÓÂÈ ÙËÓ Î·Ù·ÛÙÚÔÊ‹ ÙÔ˘ DNA ÛÙ· ·ÓıÚÒÈÓ· ÛÂÚÌ·ÙÔ˙ˆ¿ÚÈ· Î·È Ó· ÚÔηÏ› ÌÂÙ¿ÏÏ·ÍË ÛÙ· ÁÂÓÓËÙÈο ·ÙÙ·Ú· (30). ∫·Ù·Ó¿ÏˆÛË ·ÏÎÔfiÏ ∞ÛÙ·ı‹ Â˘Ú‹Ì·Ù· ·Ó·Ê¤ÚıËÎ·Ó Î·È ÛÙË Û˘Û¯¤ÙÈÛË Î·Ù·Ó¿ÏˆÛ˘ ·ÏÎÔfiÏ Î·Ù¿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË Î·È ÎÈÓ‰‡ÓÔ˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜. ™Â ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚıËÎÂ Û˘Û¯¤ÙÈÛË Î·Ù·Ó¿ÏˆÛ˘ ·ÏÎÔfiÏ ·fi ÙË ÌËÙ¤Ú· Î·È ÎÈÓ‰‡ÓÔ˘ Oª§ Û ÌÈÎÚ¿ ·È‰È¿, ıˆÚÒÓÙ·˜ fiÙÈ Ë ¤ÎıÂÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÛÙÔ ·ÏÎÔfiÏ ›Ûˆ˜ ¤¯ÂÈ Î¿ÔÈ· ËÏÈÎȷ΋ ›‰Ú·ÛË ÁÈ· ÙËÓ Oª§ (31). O ·˘ÍË̤ÓÔ˜ ΛӉ˘ÓÔ˜ Û˘Û¯ÂÙ›ÛÙËΠ̠fiÏÔ˘˜ ÙÔ˘˜ Ù‡Ô˘˜ ·ÏÎÔÔÏÔ‡¯ˆÓ ÔÙÒÓ Î·È ‹Ù·Ó ȉȷ›ÙÂÚ· ˘„ËÏfi˜ ÁÈ· ·È‰È¿ ÙˆÓ ÔÔ›ˆÓ ÔÈ ÌËÙ¤Ú˜ ¤ÈÓ·Ó Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‰Â‡ÙÂÚÔ˘ ‹ ÙÚ›ÙÔ˘ ÙÚÈÌ‹ÓÔ˘ Ù˘ ·ËÛ˘. OÈ ‚ÈÔÏÔÁÈÎÔ› Ì˯·ÓÈÛÌÔ› ÁÈ· ÙËÓ ·Ú·ÙËÚËı›۷ Û˘Û¯¤ÙÈÛË Â›Ó·È ·Û·Ê›˜, ·ÏÏ¿ ÙÔ ·ÏÎÔfiÏ ÌÔÚ› Ó· ·˘Í‹ÛÂÈ Ù· ›‰· ÔÚÈÛÌ¤ÓˆÓ ÂÓ˙‡ÌˆÓ, fiˆ˜ ÙÔ Î˘ÙÙfi¯ÚˆÌ· P450, Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÛÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÚÔηÚÎÈÓÔÁfiÓˆÓ. ¢È·ÈÙËÙÈΤ˜ Û˘Ó‹ıÂȘ O Èı·Ófi˜ ÚfiÏÔ˜ ÙˆÓ ‰È·ÈÙËÙÈÎÒÓ Û˘ÓËıÂÈÒÓ Ù˘ ÌËÙ¤Ú·˜ Î·È ÙÔ˘ ·È‰ÈÔ‡ ÛÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ‰ÂÓ ÌÂÏÂÙ‹ıËΠ̤¯ÚÈ ÙÔ 1994, fiÙ·Ó ‰‡Ô Û˘ÁÎÚÈÙÈΤ˜ ÌÂϤÙ˜ ·Ó¤ÊÂÚ·Ó Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ηٷӿψÛ˘ ηÓÈÛÙÔ‡ ÎÚ¤·ÙÔ˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ ‹ Ù· ·È‰È¿ Î·È ·˘ÍË̤ÓÔ˘ ÎÈÓ‰‡ÓÔ˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜. ∏ ʇÛË ·˘Ù‹˜ Ù˘ Û˘Û¯¤ÙÈÛ˘ ·Ú·Ì¤ÓÂÈ ÚÔ˜ ‰È¢ÎÚ›ÓÈÛË, ÁÈ·Ù› Û ̛· ¿ÏÏË ÌÂϤÙË ‰È·ÈÛÙÒıËΠÌÂȈ̤ÓÔ˜ ΛӉ˘ÓÔ˜ ÁÈ· Oª§ Î·È O§§ Û ·È‰È¿ Ì ̷ÎÚÔ¯ÚfiÓÈ· ¯Ú‹ÛË ÌÔ˘ÚÔ˘Ó¤Ï·ÈÔ˘. ¶ÚfiÛÊ·Ù·, Û ̛· Û˘ÁÎÚÈÙÈ΋ ÌÂϤÙË 84 ÂÚÈÙÒÛÂˆÓ ‚ÚÂÊÈ΋˜ Ï¢¯·ÈÌ›·˜ Î·È 97 ‚ÚÂÊÒÓ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó Û·Ó ÔÌ¿‰· ÂϤÁ¯Ô˘ (32), Ë ÚfiÛÏË„Ë ·fi ÙË ÌËÙ¤Ú· ÙÚÔÊ›ÌˆÓ Ô˘ ÂÚȤ¯Ô˘Ó ·Ó·ÛÙÔÏ›˜ ÙÔ˘ ÂÓ˙‡ÌÔ˘ ÙÔÔ˚ÛÔÌÂÚ¿ÛË ππ ‚Ú¤ıËΠӷ Û¯ÂÙ›˙ÂÙ·È ıÂÙÈο Ì ΛӉ˘ÓÔ ‚ÚÂÊÈ΋˜ Oª§, ÂÓÒ ‰ÂÓ ‚Ú¤ıËÎÂ Û˘Û¯¤ÙÈÛË Ì O§§. ™Ù· ÙÚfiÊÈÌ· ·˘Ù¿ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ù· Ê·ÛfiÏÈ·, ÊÚ¤Ûη Ï·¯·ÓÈο, ÎÔÓÛÂÚ‚ÔÔÈË̤ӷ Ï·¯·ÓÈο, ÊÚÔ‡Ù·, ÛfiÁÈ·, ηÓÔÓÈÎfi˜ ηʤ˜, Ì·‡ÚÔ ÙÛ¿È, Ú¿ÛÈÓÔ ÙÛ¿È, Ù‡ÔÈ ÎfiÏ·˜ Î·È ÎÚ·Û›. ∆· Â˘Ú‹Ì·Ù· ·˘Ù¿ Â›Ó·È ·ÓËÛ˘¯ËÙÈο, ·ÊÔ‡ Â›Ó·È ÁÓˆÛÙfi fiÙÈ ¯ËÌÂÈÔıÂڷ¢ÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ ·Ó·ÛÙ¤ÏÏÔ˘Ó ÙËÓ ÙÔÔ˚ÛÔÌÂÚ¿ÛË ππ ÙÔ˘ DNA ÚÔηÏÔ‡Ó ÂÓ›ÔÙ ‰Â˘ÙÂÚÔ·ı‹ Oª§ Ì ·ÓˆÌ·Ï›· ÛÙÔ ÁÔÓ›‰ÈÔ MLL (mixed lineage leukemia) ÛÙË ı¤ÛË 11q23. ∏ ·ÓˆÌ·Ï›· ·˘Ù‹ ·Ó¢ڛÛÎÂÙ·È ÛÙÔ
¶·È‰È·ÙÚÈ΋ 2001;64:535-546
80% ÙˆÓ ÓÂԉȷÁÓˆÛÌ¤ÓˆÓ ÂÚÈÙÒÛÂˆÓ ‚ÚÂÊÈ΋˜ Ï¢¯·ÈÌ›·˜. ∞Í›˙ÂÈ Ó· ÛËÌÂȈı›, fï˜, fiÙÈ ÔÏÏ¿ ·fi Ù· ÙÚfiÊÈÌ· Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È, fiˆ˜ Ù· ÊÚ¤Ûη Ï·¯·ÓÈο Î·È ÊÚÔ‡Ù·, ›Ûˆ˜ ÂÚȤ¯Ô˘Ó Î·È ·˘ÍË̤ӷ ›‰· Ê˘ÙÔÊ·ÚÌ¿ÎˆÓ Ô˘, ›Û˘, Â›Ó·È ‡ÔÙ· Ï¢¯·ÈÌÔÁfiÓ·. ∂Âȉ‹ ·˘Ù‹ Ë ÌÂϤÙË ‹Ù·Ó ÌÈÎÚÔ‡ ÌÂÁ¤ıÔ˘˜ Î·È Ì ÂÏ¿¯ÈÛÙË ÂÎÙ›ÌËÛË Ù˘ ‰È·ÈÙËÙÈ΋˜ ÚfiÛÏ˄˘, ÔÈ Û˘ÁÁÚ·Ê›˜ ÈÛÙÂ‡Ô˘Ó fiÙÈ Ù· ·ÔÙÂϤÛÌ·Ù· Ú¤ÂÈ Ó· ıˆÚËıÔ‡Ó ÚÔηٷÚÎÙÈο. ¶ÂÚÈÁÂÓÓËÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο ∆Ô ÌÂÁ¿ÏÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ ›¯Â ÛÙÔ ·ÚÂÏıfiÓ Û˘Û¯ÂÙÈÛÙ› Ì ΛӉ˘ÓÔ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜, ȉȷ›ÙÂÚ· ÁÈ· ·È‰È¿ ÌÈÎÚfiÙÂÚ· ÙˆÓ 2 ¯ÚfiÓˆÓ Î·Ù¿ ÙË ‰È¿ÁÓˆÛË. ∞ÚÎÂÙ¤˜ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ, ÙÔ˘Ï¿¯ÈÛÙÔÓ ‰ÈÏ¿ÛÈÔ, Û ·È‰È¿ Ì ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ ¿Óˆ ·fi 4000 gr, ÂÓÒ ¿ÏϘ ‰ÂÓ ·Ó¤ÊÂÚ·Ó Û˘Û¯¤ÙÈÛË (33). O ˘ÔΛÌÂÓÔ˜ Ì˯·ÓÈÛÌfi˜ Â›Ó·È ¿ÁÓˆÛÙÔ˜. ªÂ ‚¿ÛË ÙȘ ·Ú·ÙËÚ‹ÛÂȘ fiÙÈ 1) ÙÔ ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ Û¯ÂÙ›˙ÂÙ·È Ì ٷ ›‰· ÙÔ˘ ·Ú¿ÁÔÓÙ· π insuline-like Ù˘ ·‡ÍËÛ˘ (IGF-1), 2) Ô ·Ú¿ÁÔÓÙ·˜ ·˘Ùfi˜ ‰È·‰Ú·Ì·Ù›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙȘ ‰ÈÂÚÁ·Û›Â˜ Ù˘ ·ÈÌÔÔ›ËÛ˘ Î·È 3) Ë ‚ÚÂÊÈ΋ Ï¢¯·ÈÌ›· ÚÔηÏÂ›Ù·È Î˘Ú›ˆ˜ ·fi ÂÎı¤ÛÂȘ ÙÔ˘ ÂÌ‚Ú‡Ô˘, ÔÈ Ross Î·È Û˘Ó (34) ‰ÈÂÙ‡ˆÛ·Ó ÙËÓ ˘fiıÂÛË fiÙÈ ˘„ËÏ¿ ›‰· ÙÔ˘ ·Ú¿ÁÔÓÙ· IGF-1 ‰˘ÓËÙÈο ÌÔÚÔ‡Ó fi¯È ÌfiÓÔ Ó· ‰ËÌÈÔ˘ÚÁÔ‡Ó ¤Ó· ÌÂÁ¿ÏÔ ÌˆÚfi, ·ÏÏ¿ Î·È Ó· Û˘Ó‰Ú¿ÌÔ˘Ó ÛÙË Ï¢¯·ÈÌÔÁ¤ÓÂÛË. AÓ··Ú·ÁˆÁÈÎfi ÈÛÙÔÚÈÎfi ÌËÙ¤Ú·˜ ¶ÚÔËÁÔ‡ÌÂÓ˜ ·ÒÏÂȘ ÂÌ‚Ú‡ˆÓ Ê·›ÓÂÙ·È Ó· Û¯ÂÙ›˙ÔÓÙ·È Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜, ·Ó Î·È Ì›· ÌÂϤÙË ·Ó¤ÊÂÚ ·Ó¿ÛÙÚÔÊË Û˘Û¯¤ÙÈÛË (32). ™Â ÌÈ· ÚfiÛÊ·ÙË Û¯ÂÙÈο ÌÂϤÙË ·ÛıÂÓÒÓ/Ì·ÚÙ‡ÚˆÓ (35) Û 1507 ÂÚÈÙÒÛÂȘ O§§ ‰È·ÁÓˆÛı¤ÓÙˆÓ Û ËÏÈΛ· <2 ¯ÚfiÓˆÓ, ÙÔ ÈÛÙÔÚÈÎfi ‰‡Ô ‹ ÂÚÈÛÛÔÙ¤ÚˆÓ ·Ô‚ÔÏÒÓ, Û ۇÁÎÚÈÛË Ì ÙËÓ ÔÌ¿‰· ÂϤÁ¯Ô˘, Û¯ÂÙÈ˙fiÙ·Ó Ì ÂÓÙ·Ï¿ÛÈÔ Î›Ó‰˘ÓÔ (p<0,001). ∆Ô ÈÛÙÔÚÈÎfi ıÓËÛÈÁÂÓÒÓ ÂÌ‚Ú‡ˆÓ ‹ ·˘ÙfiÌ·ÙˆÓ ·Ô‚ÔÏÒÓ ›Ûˆ˜ ‰Â›¯ÓÂÈ Î¿ÔÈ· ÎÔÈÓ‹ ÂÚÈ‚·ÏÏÔÓÙÈ΋ ¤ÎıÂÛË Î·È/‹ οÔÈ· ÎÏËÚÔÓÔÌÔ‡ÌÂÓË ÁÂÓÂÙÈ΋ ·ÓˆÌ·Ï›· Ô˘ ‰‡Ó·Ù·È Ó· ¤¯ÂÈ ÔÈΛϘ ÂȉڿÛÂȘ ÛÙÔ ¤Ì‚Ú˘Ô (.¯. ·fi ıÓËÛÈÁÂÓ¤˜ ̤¯ÚÈ Ï¢¯·ÈÌ›·). ÕÏÏÔÈ ·Ú¿ÁÔÓÙ˜ À¿Ú¯Ô˘Ó ‰È¿ÊÔÚ˜ ¿ÏϘ Û˘Û¯ÂÙ›ÛÂȘ, Ì ‹ ¯ˆÚ›˜ ȉȷ›ÙÂÚË ÛËÌ·ÓÙÈÎfiÙËÙ·, Ì ÙËÓ ·È‰È΋ Ï¢¯·ÈÌ›·, fiˆ˜ Ë ËÏÈΛ· Ù˘ ÌËÙ¤Ú·˜, Ë ÛÂÈÚ¿ Á¤ÓÓËÛ˘ ÙÔ˘ ·È‰ÈÔ‡, Ë ¤ÎıÂÛË Û ÂÓÙÔÌÔÎÙfiÓ· (·ÁÁÂÏÌ·ÙÈ΋ ‹ ÔÈÎȷ΋), Ë ¤ÎıÂÛË ÙˆÓ ÁÔÓ¤ˆÓ Û ‰È·Ï˘ÙÈο Î·È ·Ú¿ÁˆÁ· ‚ÂÓ˙›Ó˘, Ë ¯Ú‹ÛË, Ì·ÚÈ¯Ô˘¿Ó·˜, ·ÓÙÈÛ˘ÏÏËÙÈÎÒÓ Î·È ¿ÏÏˆÓ ÔÚÌÔÓÒÓ ·fi ÙË ÌËÙ¤Ú· ηٿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË (34,36,37).
Paediatriki 2001;64:535-546
ªË ÈÔÓ›˙Ô˘Û· ·ÎÙÈÓÔ‚ÔÏ›· ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ÂÎÊÚ¿ÛÙËÎ·Ó ÛΤ„ÂȘ ÁÈ· Èı·Ó‹ ηÚÎÈÓÔÁfiÓÔ Â›‰Ú·ÛË Ù˘ ÌË ÈÔÓ›˙Ô˘Û·˜ ·ÎÙÈÓÔ‚ÔÏ›·˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ Î·È Ù· ·È‰È¿. Œ¯ÂÈ ÌÂÏÂÙËı› Ë Â›‰Ú·ÛË Ù˘ ¤ÎıÂÛ˘ Û ÂÍ·ÈÚÂÙÈο ¯·ÌËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ËÏÂÎÙÚÈο Î·È Ì·ÁÓËÙÈο ‰›· (∂Ù ∏ª¶) (38), Û ÌÈÎÚÔ·̷ٷ ÙËÏÂÈÎÔÈÓˆÓÈ·ÎÒÓ Û˘ÛÙËÌ¿ÙˆÓ Î·È Ú·‰ÈÔÙËÏÂÔÙÈÎÒÓ ÂÎÔÌÒÓ (39), Û ʈÙÔıÂڷ›· (40) Î·È ¯Ú‹ÛË ˘ÂÚ‹¯ˆÓ ÁÈ· ‰È·ÁÓˆÛÙÈÎÔ‡˜ ÏfiÁÔ˘˜ (41). ∆· Â˘Ú‹Ì·Ù· ÙˆÓ ÌÂÏÂÙÒÓ ·˘ÙÒÓ Â›Ó·È ÙÔ˘Ï¿¯ÈÛÙÔÓ ·ÓÙÈÊ·ÙÈο. ∂ȉÈÎfiÙÂÚ·, ÙËÓ ÙÂÏÂ˘Ù·›· 20ÂÙ›· ¤¯Ô˘Ó Á›ÓÂÈ ·ÚÎÂÙ¤˜ ÌÂÁ¿Ï˜ ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ ÛÙËÓ ∞ÌÂÚÈ΋ Î·È ÙËÓ ∂˘ÚÒË ·Ó·ÊÔÚÈο Ì ÙË Û¯¤ÛË Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ Î·È ÙËÓ ¤ÎıÂÛË Û ÂÍ·ÈÚÂÙÈο ¯·ÌËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ Ì·ÁÓËÙÈο ‰›· 50-60 Hz Ô˘ ‰ËÌÈÔ˘ÚÁÔ‡ÓÙ·È ·fi ÂÓ·¤ÚȘ ÁÚ·Ì̤˜ ÌÂÙ·ÊÔÚ¿˜ ˘„ËÏ‹˜ Ù¿Û˘, ·fi ηψ‰ÈÒÛÂȘ ÛÙȘ ηÙÔÈ˘ Î·È ·fi ÔÈÎȷΤ˜ ËÏÂÎÙÚÈΤ˜ Û˘Û΢¤˜. ∏ ÂÚ¢ÓËÙÈ΋ ·˘Ù‹ ÚÔÛ¿ıÂÈ· ηχÊıËΠ·fi Ù· ̤۷ Ì·˙È΋˜ ÂÓË̤ڈÛ˘ Ì ¤Ó· ¤ÏÔ Ì˘ÛÙËÚ›Ô˘, ·ÓÙÈı¤ÛÂˆÓ Î·È Û‡Á¯˘Û˘. ∏ ‰È·Ì¿¯Ë ÛÙËÓ ÂÈÛÙËÌÔÓÈ΋ ÎÔÈÓfiÙËÙ· ‹Ù·Ó ¤ÓÙÔÓË Î·È ÌÂÚÈΤ˜ ÊÔÚ¤˜ ˘ÂÚ‚ÔÏÈ΋. ∞ÎfiÌË Î·È Ë ∂ıÓÈ΋ ∞η‰ËÌ›· ∂ÈÛÙËÌÒÓ Ù˘ ∞ÌÂÚÈ΋˜ ÙÔ ¡Ô¤Ì‚ÚÈÔ ÙÔ˘ 1997, ÂÓÒ ‰ËÌÔÛÈÔÔ›ËÛ fiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ÎÈÓ‰‡ÓÔ˘, ˘ÔÛÙ‹ÚÈÍÂ Û˘Á¯ÚfiÓˆ˜ ÙËÓ ÂÚ·ÈÙ¤Úˆ ÂÚ¢ÓËÙÈ΋ ÚÔÛ¿ıÂÈ· ÁÈ· ÙËÓ ·È‰È΋ Ï¢¯·ÈÌ›· (42). “∏ÏÂÎÙÚÈΤ˜ ÁÚ·Ì̤˜, ηÚΛÓÔ˜ Î·È Êfi‚Ô˜” ‹Ù·Ó Ô Ù›ÙÏÔ˜ ·ÚÈÔ˘ ¿ÚıÚÔ˘ ÛÙÔ New England Journal of Medicine ÛÙȘ 3 πÔ˘Ï›Ô˘ 1997 (43). OÚÈṲ̂ÓÔÈ ÂÚ¢ÓËÙ¤˜ ÈÛ¯˘Ú›ÛÙËÎ·Ó fiÙÈ Ë ‰È·ÌÔÓ‹ ÎÔÓÙ¿ Û ÁÚ·Ì̤˜ ˘„ËÏÔ‡ ÊÔÚÙ›Ô˘ (˘ÏÒÓ˜, ÁÚ·Ì̤˜ ˘„ËÏ‹˜ Ù¿Û˘ Î.Ï.) ÚÔηÏ› ηÚΛÓÔ Î·È ÂȉÈÎfiÙÂÚ· Ï¢¯·ÈÌ›· ÛÙ· ·È‰È¿ (44). ÕÌÂÛ·, ÔÈÎÔÏÔÁÈΤ˜ ÔÚÁ·ÓÒÛÂȘ Î·È Ù· ̤۷ Ì·˙È΋˜ ÂÓË̤ڈÛ˘ ¿Ú¯ÈÛ·Ó Ó· ‰È·‰›‰Ô˘Ó ÙËÓ Â›‰ËÛË fiÙÈ Ù· ∂Ù ∏ª¶ ÚÔηÏÔ‡Ó Î·ÚΛÓÔ. OÈ Ù˘¯ÈÔ‡¯ÔÈ º˘ÛÈ΋˜ ÁÓˆÚ›˙Ô˘Ó ·˘Ù¿ Ù· ·fiÚ·Ù· ‰›· ηϿ, ·ÏÏ¿ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ÁÈ·ÙÚÔ›, ÁÔÓ›˜ Î·È ·ÛıÂÓ›˜ fi¯È. ∏ ΛÓËÛË ÙˆÓ ËÏÂÎÙÚÈÎÒÓ ÊÔÚÙ›ˆÓ ‰ËÌÈÔ˘ÚÁ› ¤Ó· Ì·ÁÓËÙÈÎfi ÊÔÚÙ›Ô Ô˘ ÌÔÚ› Ó· ÌÂÙÚËı›. ∞ÎfiÌË Î·È ÙˆÓ 60 Hz (ÛÙËÓ ∞ÌÂÚÈ΋) ‹ 50 Hz (ÛÙËÓ ∂˘ÚÒË) ËÏÂÎÙÚÈÎfi Ú‡̷ ÙˆÓ ¯ÒÚˆÓ ‰È·ÌÔÓ‹˜ ‰ËÌÈÔ˘ÚÁ› ¤Ó· Ôχ ·ÛıÂÓ¤˜ ‰›Ô, ÙÔ ÔÔ›Ô - fiˆ˜ fiÏ· Ù· Ì·ÁÓËÙÈο ‰›· - ‰È·ÂÚÓ¿ ˙ˆÓÙ·ÓÔ‡˜ ÈÛÙÔ‡˜. ∞˘Ù¿ Ù· ∂Ù ∏ª¶ Â›Ó·È ÁÓˆÛÙ¿ Û·Ó ÌË ÈÔÓ›˙Ô˘Û· ·ÎÙÈÓÔ‚ÔÏ›·, ·ÊÔ‡ ÙÔ ÔÛÔÛÙfi ÂÓ¤ÚÁÂÈ·˜ Ô˘ ʤÚÔ˘Ó Â›Ó·È Î·Ù¿ Ôχ ¯·ÌËÏfiÙÂÚÔ ·fi ÂΛÓÔ Ô˘ ¯ÚÂÈ¿˙ÂÙ·È ÁÈ· Ó· ‰È·Û¿ÛÂÈ ÌÔÚÈ·ÎÔ‡˜ ‰ÂÛÌÔ‡˜, fiˆ˜ ·˘ÙÔ‡˜ ÛÙÔ DNA. ¶Ú·ÁÌ·ÙÈο, Â›Ó·È ÂÓÙ˘ˆÛÈ·Îfi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ·ÓËÛ˘¯Ô‡Ì ÁÈ· Ù· ÂÍ·ÈÚÂÙÈο ¯·ÌËÏ‹˜
541
¶·È‰È·ÙÚÈ΋ 2001;64:535-546
Û˘¯ÓfiÙËÙ·˜ ËÏÂÎÙÚÈο Î·È Ì·ÁÓËÙÈο ‰›· fiÙ·Ó ˙ԇ̠̤۷ ÛÙÔ Á‹ÈÓÔ ÛÙ·ÙÈÎfi Ì·ÁÓËÙÈÎfi ‰›Ô ¤ÓÙ·Û˘ 50 Ì∆ Ô˘ Â›Ó·È ÂηÙÔÓÙ¿‰Â˜ ÊÔÚ¤˜ ÈÛ¯˘ÚfiÙÂÚÔ ·fi ÙÔ Ì·ÁÓËÙÈÎfi ‰›Ô ¤ÓÙ·Û˘ 0,01 ¤ˆ˜ 0,05 Ì∆ Ô˘ ·Ú¿ÁÂÙ·È ·fi ÙÔ ËÏÂÎÙÚÈÎfi Ú‡̷ 110/220 V ÛÙ· Û›ÙÈ·. ∞ÎfiÌË Î·È ·ÎÚÈ‚Ò˜ οو ·fi Ì›· ÁÚ·ÌÌ‹ ˘„ËÏ‹˜ Ù¿Û˘, ÙÔ Ì·ÁÓËÙÈÎfi ‰›Ô Â›Ó·È ÌfiÓÔ 3 ¤ˆ˜ 10 Ì∆ ¯·ÌËÏfiÙÂÚÔ ·fi ÂΛÓÔ Ì¤Û· Û ¤Ó· fi¯ËÌ· ËÏÂÎÙÚÈÎÔ‡ ÙÚ¤ÓÔ˘ Î·È Î·Ù¿ Ôχ ·ÛıÂÓ¤ÛÙÂÚÔ ÂΛÓÔ˘ Ô˘ ·Ú¿ÁÂÙ·È ÛÙÔ ÎÂÊ¿ÏÈ Î·Ù¿ ÙË ¯Ú‹ÛË ËÏÂÎÙÚÈ΋˜ ͢ÚÈÛÙÈ΋˜ Ì˯·Ó‹˜ (ÂÚ›Ô˘ 60 Ì∆). ∂ÓÒ Ë ÏÂÈÔ„ËÊ›· ÙˆÓ º˘ÛÈÎÒÓ ıˆÚÔ‡Ó ·›ÛÙ¢ÙË ÙËÓ ¿Ô„Ë fiÙÈ Ù· ∂Ù ∏ª¶ Â›Ó·È ·ÂÈÏ‹ ÁÈ· ÙËÓ ˘Á›· ÙˆÓ ·ÓıÚÒˆÓ, ‰Âο‰Â˜ ÂȉËÌÈÔÏÔÁÈÎÒÓ ÌÂÏÂÙÒÓ ·Ó¤ÊÂÚ·Ó ·ÛıÂÓ›˜ ıÂÙÈΤ˜ Û˘Û¯ÂÙ›ÛÂȘ ÌÂٷ͇ ÙÔ˘ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ηÚΛÓÔ Î·È Ù˘ ÁÂÈÙÓ›·Û˘ Ì ÁÚ·Ì̤˜ ˘„ËÏ‹˜ Ù¿Û˘. OÈ ·ÚÓËÙÈΤ˜ ‹ ·Û·Ê›˜ ÌÂϤÙ˜ ‰ÂÓ ¤ÎÏÂÈÛ·Ó ÙËÓ ·ÓÙ›ıÂÛË. ™ËÌÂÈÒÓÂÙ·È fiÙÈ, ÂÂȉ‹ ‰ÂÓ ˘¿Ú¯Ô˘Ó Ô˘ÛÈ·ÛÙÈο ÌË ÂÎÙÂıÂÈ̤ÓÔÈ Û ‰›· ËÏÂÎÙÚÈÎÒÓ ÁÚ·ÌÌÒÓ, Ë Ú·ÁÌ·ÙÈ΋ Û‡ÁÎÚÈÛË Â›Ó·È ÌÂٷ͇ “˘„ËÏ¿ ÂÎÙÂıÂÈ̤ӈӔ Î·È “¯·ÌËÏ¿ ÂÎÙÂıÂÈ̤ӈӔ. OÈ ·Ú¯ÈΤ˜ ÌÂϤÙ˜ Ô˘ ˘ÔÛÙ‹ÚÈÍ·Ó ÙË Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ËÏÂÎÙÚÈÎÒÓ ÁÚ·ÌÌÒÓ Î·È ·È‰ÈÎÔ‡ ηÚΛÓÔ˘ ¯ÚËÛÈÌÔÔ›ËÛ·Ó ÙÔ Û˘Ó‰˘·ÛÌfi ÙÔ˘ Ù‡Ô˘ ÙˆÓ Î·Ïˆ‰ÈÒÛÂˆÓ Î·È ÙËÓ ·fiÛÙ·ÛË ·fi ÙÔ Û›ÙÈ Û·Ó ÂÌÂÈÚÈÎfi ̤ÙÚÔ ¤ÎıÂÛ˘, ¤Ó· Û‡ÛÙËÌ· Ô˘ ÔÓÔÌ¿ÛÙËΠ“Έ‰ÈÎÔ› ηψ‰›ˆÓ” (44). ÕÏϘ ÌÂϤÙ˜ ¯ÚËÛÈÌÔÔ›ËÛ·Ó Û·Ó Ì¤ÙÚÔ ¤ÎıÂÛ˘ ÙËÓ ·fiÛÙ·ÛË ·fi ÙȘ ÁÚ·Ì̤˜ ÌÂÙ·ÊÔÚ¿˜ ‹ ÙÔ˘˜ ˘ÔÛÙ·ıÌÔ‡˜, ¿ÌÂÛ˜ ÌÂÙÚ‹ÛÂȘ ‰›ˆÓ ‹ ˘ÔÏÔÁÈÛı¤ÓÙ· ·Ó·‰ÚÔÌÈο ‰›· (ÚÔÙ˘ÔÔÈË̤Ó˜ ÌÂÙÚ‹ÛÂȘ) (45). ∫·Ì›· ·fi ÙȘ ÌÂÙÚ‹ÛÂȘ ¤ÎıÂÛ˘ ‰ÂÓ ˘ÂÚ¤¯ÂÈ Û·ÊÒ˜ ÙˆÓ ¿ÏÏˆÓ Î·È Î·Ì›· ‰ÂÓ Â›Ó·È ÎÔÈÓ‹ ÁÈ· ÙȘ ÌÂÁ·Ï‡ÙÂÚ˜ ÌÂϤÙ˜. πÛÙÔÚÈο, ¤Ó· ·fi Ù· ÂÚÈÛÛfiÙÂÚÔ ·ÈÓÈÁÌ·ÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ÌÂÏÂÙÒÓ ÁÈ· ÙËÓ ·È‰È΋ Ï¢¯·ÈÌ›· ‹Ù·Ó ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë Û˘Û¯¤ÙÈÛË Ù˘ “¤ÎıÂÛ˘” Ì ÙËÓ Â›ÙˆÛË ÙÔ˘ ηÚΛÓÔ˘ ÂÌÊ·ÓÈ˙fiÙ·Ó ˘„ËÏfiÙÂÚË fiÙ·Ó Ô Ù‡Ô˜ ÙˆÓ Î·Ïˆ‰›ˆÓ ‹ Ë ÁÂÈÙÓ›·ÛË Ì ÙȘ ÁÚ·Ì̤˜ ‹Ù·Ó ÙÔ Ì¤ÙÚÔ ¤ÎıÂÛ˘ Î·È fi¯È fiÙ·Ó ÁÈÓfiÙ·Ó ¿ÌÂÛË Ì¤ÙÚËÛË ÙˆÓ Â‰›ˆÓ ÛÙ· Û›ÙÈ·. ∞˘Ùfi Ô‰‹ÁËÛ ÛÙËÓ ˘fiıÂÛË fiÙÈ Ë Û˘Û¯¤ÙÈÛË ÙÔ˘ ·È‰ÈÎÔ‡ ηÚΛÓÔ˘ Ì ÙË ‰È·ÌÔÓ‹ ÎÔÓÙ¿ Û ËÏÂÎÙÚÔÊfiÚ˜ ÁÚ·Ì̤˜ ı· ÌÔÚÔ‡Û ӷ ÔÊ›ÏÂÙ·È Û ¿ÏÏÔ ·Ú¿ÁÔÓÙ· Î·È fi¯È ÛÙËÓ ¤ÎıÂÛË ÛÙ· ‰›· ·˘Ù¿. °È· ·Ú¿‰ÂÈÁÌ·, ˘ÔÛÙËÚ›¯ıËΠfiÙÈ Ë ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈ΋ Ù¿ÍË ı· ÌÔÚÔ‡Û ӷ Â›Ó·È ¤Ó·˜ Û˘Ì·Ú¿ÁÔÓÙ·˜ Î·È “ÂÎÙÂıÂÈ̤Ó˜” Î·È “ÌË ÂÎÙÂıÂÈ̤Ó˜” ÔÌ¿‰Â˜ ›Ûˆ˜ ÚÔ¤Ú¯ÔÓÙ·È ·fi ‰È·ÊÔÚÂÙÈΤ˜ ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈΤ˜ Ù¿ÍÂȘ. ∞˘Ùfi ·ÊÔÚ¿ ȉȷ›ÙÂÚ· ÙȘ ÌÂϤÙ˜ ÔÈÎȷ΋˜ ¤ÎıÂÛ˘ ÛÙËÓ ∞ÌÂÚÈ΋ Ô˘ ‚·Û›˙ÔÓÙ·È
542
Paediatriki 2001;64:535-546
ÛÙËÓ Îˆ‰ÈÎÔÔ›ËÛË ÙˆÓ Î·Ïˆ‰ÈÒÛÂˆÓ Û·Ó Ì¤ÙÚÔ ¤ÎıÂÛ˘ Î·È Â›Ó·È ÁÓˆÛÙfi fiÙÈ ÔÈ Ù‡ÔÈ Î·Ïˆ‰›ˆÓ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔÓ ·È‰ÈÎfi ηÚΛÓÔ ·Ó¢ڛÛÎÔÓÙ·È Î˘Ú›ˆ˜ Û ·ÏȤ˜ ÊÙˆ¯È¤˜ Û˘ÓÔÈ˘ ηÈ/‹ ÛÂ Û˘ÓÔÈ˘ Ì „ËÏfi ÔÛÔÛÙfi ÂÓÔÈÎÈ·˙fiÌÂÓˆÓ ÛÈÙÈÒÓ (46). ¶ÚÔÛ¿ıÂȘ Ô˘ ¤ÁÈÓ·Ó ÁÈ· Ì›· ÁÂÓÈ΋ ·Ó·ÛÎfiËÛË ·˘ÙÒÓ ÙˆÓ ÌÂÏÂÙÒÓ ·¤Ù˘¯·Ó, ÂÂȉ‹ ‰ÂÓ ‹Ù·Ó ‰˘Ó·Ù‹ Ë “ÔÌÔÁÂÓÔÔ›ËÛË” ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ÂÊ’ fiÛÔÓ ÔÈ ‰È¿ÊÔÚÔÈ ÙÚfiÔÈ ÂÎÙ›ÌËÛ˘ Ù˘ ¤ÎıÂÛ˘ ‰ÂÓ ÂÈÙÚ¤Ô˘Ó ÙË Û˘ÓÔÏÈ΋ ·ÔÙ‡ˆÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜. ∆Ô 1996, ÙÔ ∂ıÓÈÎfi ™˘Ì‚Ô‡ÏÈÔ ŒÚ¢ӷ˜ Ù˘ ∞ÌÂÚÈ΋˜ (NRC) ·ÚÔ˘Û›·Û ÙȘ ›ÛË̘ ı¤ÛÂȘ ÌÂÙ¿ ·fi ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ·fi 500 Î·È Ï¤ÔÓ ÌÂϤÙ˜ ¤ÎıÂÛ˘ Û ËÏÂÎÙÚÈο Î·È Ì·ÁÓËÙÈο ‰›·. ∏ ÂÈÙÚÔ‹ ÂÂÍÂÚÁ¿ÛÙËΠÙËÓ Ï¤ÔÓ ÏÂÙÔÌÂÚ‹ Î·È ÂÚ›ÏÔÎË ÌÂÙ·-·Ó¿Ï˘ÛË ÌÂÏÂÙÒÓ, ̤¯ÚÈ ÙÔ 1996, Û ÂÈÚ·Ì·Ùfi˙ˆ·, in vitro ÌÂϤÙ˜ Î·È ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Û ·ÓıÚÒÔ˘˜. O Úfi‰ÚÔ˜ Ù˘ ÂÈÙÚÔ‹˜ ·Ó·ÎÔ›ÓˆÛ (47) fiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË fiÙÈ Ù· ËÏÂÎÙÚÈο Î·È Ì·ÁÓËÙÈο ‰›· ÂÍ·ÈÚÂÙÈο ¯·ÌËÏÒÓ Û˘¯ÓÔًوÓ, ‰ËÏ·‰‹ ÛÙÔ Â›Â‰Ô ÙˆÓ ÔÈÎÈ·ÎÒÓ ÂÎı¤ÛˆÓ, ÚÔηÏÔ‡Ó Î·ÚΛÓÔ ‹ ¤¯Ô˘Ó ÂÈ‚Ï·‚›˜ Û˘Ó¤ÂȘ ÛÙÔ Ó¢ÚÈÎfi Û‡ÛÙËÌ·, ÙËÓ ·Ó··Ú·ÁˆÁ‹ Î·È ·Ó¿Ù˘ÍË. ∂ÓÙÔ‡ÙÔȘ, ·Ú·Ì¤ÓÂÈ ·ÓÂÍ‹ÁËÙË Ì›· ·ÛıÂÓ‹˜, ·ÏÏ¿ ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋, Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ Î·È ¤ÎıÂÛ˘ Û Ôχ ˘„ËÏ¿ (>0,4 Ì∆) ›‰· ¤ÓÙ·Û˘ Ì·ÁÓËÙÈÎÒÓ Â‰›ˆÓ, Ù· ÔÔ›· fï˜ ·ÊÔÚÔ‡Ó ÂÏ¿¯ÈÛÙÔ ·ÚÈıÌfi ÔÈÎÈ·ÎÒÓ ÂÎı¤ÛˆÓ. ∏ ÂÈÙÚÔ‹ ‰ÂÓ ˘ÔÛÙËÚ›˙ÂÈ fiÙÈ Ë ¤ÎıÂÛË ÚÔηÏ› ÙË Ï¢¯·ÈÌ›· ÛÙ· ·È‰È¿, ·ÏÏ¿ ÙÔ Â‡ÚËÌ· ¯ÚÂÈ¿˙ÂÙ·È ÂÚ·ÈÙ¤Úˆ ÌÂϤÙË ÛÂ Û˘Ó‰˘·ÛÌfi Ì ¿ÏÏÔ˘˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ ÙÚfiÔÈ ÂÎÙ›ÌËÛ˘ Ù˘ ¤ÎıÂÛ˘, ËÏÈΛ· ÙÔ˘ ÛÈÙÈÔ‡, Ú‡·ÓÛË Ù˘ ·ÙÌfiÛÊ·ÈÚ·˜, ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈ΋ ηٿÛÙ·ÛË Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. ∏ ÚfiÛÊ·ÙË ÌÂϤÙË ·fi ÙËÓ ∞ÌÂÚÈ΋ ÙˆÓ Linet Î·È Û˘Ó (48) Â›Ó·È Ë ÌÂÁ·Ï‡ÙÂÚË Û ̤ÁÂıÔ˜ Û˘ÓÂÚÁ·ÙÈ΋ ÌÂϤÙË ÙÔ˘ πÓÛÙÈÙÔ‡ÙÔ˘ ∫·ÚΛÓÔ˘ Ù˘ ∞ÌÂÚÈ΋˜ (NCI) Î·È Ù˘ ÔÌ¿‰·˜ ÌÂϤÙ˘ ·È‰ÈÎÔ‡ ηÚΛÓÔ˘ (CCSG). ∏ ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ù˘ ÌÂϤÙ˘ ¤‰ÂÈÍ fiÙÈ ‰ÂÓ ˘Ê›ÛÙ·Ù·È ¤Ó‰ÂÈÍË ÔÔÈ·Û‰‹ÔÙÂ Û˘Û¯¤ÙÈÛ˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ Î·È ¤ÎıÂÛ˘ Û ÂÍ·ÈÚÂÙÈο ¯·ÌËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ËÏÂÎÙÚÈο Î·È Ì·ÁÓËÙÈο ‰›·, ·ÎfiÌË Î·È ÁÈ· Ôχ ˘„ËϤ˜ ÂÓÙ¿ÛÂȘ ¿Óˆ ÙˆÓ 0,4 Ì∆. ™ÙË Û˘ÁÎÚÈÙÈ΋ ·˘Ù‹ ÌÂϤÙË, ÔÈ ÂÚ¢ÓËÙ¤˜ ·Ó¤ÊÂÚ·Ó fiÙÈ ·È‰È¿ Ô˘ ‰È¤ÌÂÓ·Ó Û ۛÙÈ· Ù· ÔÔ›· Ù·ÍÈÓÔÌ‹ıËÎ·Ó Û ˘„ËϤ˜ ηÙËÁÔڛ˜ ¤ÎıÂÛ˘ Ì ‚¿ÛË ÙÔ˘˜ “Έ‰ÈÎÔ‡˜ ηψ‰ÈÒÛˆӔ Ô˘ Â›Ó·È Î·Ù¿ ÚÔÛ¤ÁÁÈÛË Ì¤ÙÚÔ ¤ÎıÂÛ˘ Û ̷ÁÓËÙÈο ‰›· Î·È ‚·Û›˙ÂÙ·È ÛÙÔ ¿¯Ô˜, ÙË ‰È¿Ù·ÍË Î·È ÙËÓ ·fiÛÙ·ÛË ÙˆÓ ËÏÂÎÙÚÈÎÒÓ ÁÚ·ÌÌÒÓ ·fi ÙÔ ¯ÒÚÔ ‰È·ÌÔÓ‹˜, ‰ÂÓ ÂÌÊ¿ÓÈÛ·Ó
¶·È‰È·ÙÚÈ΋ 2001;64:535-546
·˘ÍË̤ÓË Â›ÙˆÛË Ï¢¯·ÈÌ›·˜. ∆· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ ¤Ú¯ÔÓÙ·È Û ۷ʤÛÙ·ÙË ·ÓÙ›ıÂÛË Ì ÙÔ ÙÂÏÈÎfi Û˘Ì¤Ú·ÛÌ· Ù˘ ·Ó¿Ï˘Û˘ Ù˘ ∂ıÓÈ΋˜ ∞η‰ËÌ›·˜ ∂ÈÛÙËÌÒÓ (42) Ô˘ η٤ÏËÁ Ì ÙËÓ ¿Ô„Ë fiÙÈ Ë ÂÏ¿¯ÈÛÙË ÂȉËÌÈÔÏÔÁÈ΋ ¤Ó‰ÂÈÍË ÁÈ· Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ηÚΛÓÔ˘ Î·È ËÏÂÎÙÚÈÎÒÓ ÁÚ·ÌÌÒÓ, Â¿Ó ˘¿Ú¯ÂÈ, ‹Ù·Ó ·˘Ù‹ ÌÂٷ͇ Ôχ ˘„ËÏÒÓ ÂÎı¤ÛÂˆÓ Î·È Ï¢¯·ÈÌ›·˜ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜. ∏ ·ÓˆÙ¤Úˆ ·Ó¿Ï˘ÛË ·Ó¤ÊÂÚ ۯÂÙÈÎfi ΛӉ˘ÓÔ 1,5 Ì fiÚÈ· ·ÍÈÔÈÛÙ›·˜ (O∞) 1,2 ¤ˆ˜ 1,8. ªÂ ·ÚfiÌÔÈÔ ÔÚÈÛÌfi ¤ÎıÂÛ˘, Ë ÌÂϤÙË ·fi ÙÔ ∂ıÓÈÎfi πÓÛÙÈÙÔ‡ÙÔ ∫·ÚΛÓÔ˘ (NCI / CCSG, 48) ‰›ÓÂÈ Û¯ÂÙÈÎfi ΛӉ˘ÓÔ 1,0 (O∞ 0,7-1,3). ™Ê¿ÏÌ·Ù· ηٿ ÙËÓ ÂÈÏÔÁ‹ ÙˆÓ Ì·ÚÙ‡ÚˆÓ, ΢ڛˆ˜ fiÙ·Ó ¯ÚËÛÈÌÔÔÈÂ›Ù·È Ë Ì¤ıÔ‰Ô˜ Ù˘ Ù˘¯·›·˜ ÙËÏÂʈÓÈ΋˜ ÂÈÏÔÁ‹˜, Â›Ó·È Èı·ÓfiÓ Ó· ÂËÚ¤·Û·Ó Ù· ‰Â‰Ô̤ӷ Ì ÙËÓ ÂÈÛ·ÁˆÁ‹ Ì·ÚÙ‡ÚˆÓ ·fi ˘„ËÏfiÙÂÚ˜ ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈο Ù¿ÍÂȘ. ∏ Ù·˘Ùfi¯ÚÔÓË Â›‰Ú·ÛË Î·È ¿ÏÏˆÓ ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ·È‰È΋ Ï¢¯·ÈÌ›· Ú¤ÂÈ Ó· ÂÎÙÈÌËı› Ì ȉȷ›ÙÂÚË ÚÔÛÔ¯‹. ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ‰È¿ÊÔÚ˜ ÂÈÙÚÔ¤˜ Î·È ÔÌ¿‰Â˜ ÂȉÈÎÒÓ ¤¯Ô˘Ó ηٷϋÍÂÈ ÛÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ ÂÈÛÙÈ΋ ¤Ó‰ÂÈÍË ÁÈ· ÂÈ‚Ï·‚›˜ ÂȉڿÛÂȘ ÛÙËÓ ·ÓıÚÒÈÓË ˘Á›· ‹ ÚfiÎÏËÛË Î·ÚΛÓÔ˘ ·fi ÙȘ ÁÚ·Ì̤˜ ÌÂÙ·ÊÔÚ¿˜ ˘„ËÏ‹˜ Ù¿Û˘. OÈ ÛˆÛÙ¿ ۯ‰ȷṲ̂Ó˜ ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜, fiˆ˜ Ë ·ÓˆÙ¤Úˆ ·fi ÙËÓ Linet Î·È Û˘Ó (48) ηٷϋÁÔ˘Ó ÛÙÔ ›‰ÈÔ ·ÎÚÈ‚Ò˜ Û˘Ì¤Ú·ÛÌ·. ∆Ë ‰ÈÂÙ›· 1993-94 Ú·ÁÌ·ÙÔÔÈ‹ıËΠÛÙË ¯ÒÚ· Ì·˜ ·ÚfiÌÔÈ· ÌÂϤÙË, Û ÂıÓÈÎfi ›‰Ô, Ì ÛÎÔfi ÙË ‰È·›ÛÙˆÛË ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ·È‰È΋ Ï¢¯·ÈÌ›· ÛÙË ¯ÒÚ· Ì·˜. ∆Ô ÚˆÙfiÎÔÏÏÔ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó ·ÚfiÌÔÈÔ Ì ÂΛÓÔ Ô˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È Û ·Ú¿ÏÏËϘ ÌÂϤÙ˜ ·ÛıÂÓÒÓ-Ì·ÚÙ‡ÚˆÓ Ô˘ ‰ÈÂÓÂÚÁ‹ıËÎ·Ó Û˘Á¯ÚfiÓˆ˜ Û ¿ÏϘ ∂˘Úˆ·˚Τ˜ ¯ÒÚ˜. π‰È·›ÙÂÚË ¤ÌÊ·ÛË ‰fiıËΠÛÙÔÓ ÙÔ̤· ÙˆÓ ËÏÂÎÙÚÔÌ·ÁÓËÙÈÎÒÓ Â‰›ˆÓ ÛÂ Û˘ÓÂÚÁ·Û›· Ì ÙË ¢ËÌfiÛÈ· ∂ȯ›ÚËÛË ∏ÏÂÎÙÚÈÛÌÔ‡ (¢∂∏) Ô˘ ÂÎÙ›ÌËÛ ÙÔ˘˜ ‰Â›ÎÙ˜ ¤ÎıÂÛ˘ ÌÂ Ù˘ÊÏfi ¤ÏÂÁ¯Ô. O˘ÛÈ·ÛÙÈο ‰ÂÓ ‚Ú¤ıËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈο ·˘ÍË̤ÓÔÈ Î›Ó‰˘ÓÔÈ ÌÂٷ͇ ÙˆÓ ·È‰ÈÒÓ Ô˘ ‰È·Ì¤ÓÔ˘Ó Û ÂÚÈÔ¯¤˜ ˘„ËÏ‹˜ ηÙËÁÔÚ›·˜ ¤ÎıÂÛ˘ ËÏÂÎÙÚÈÎÒÓ Î·È Ì·ÁÓËÙÈÎÒÓ Â‰›ˆÓ Î·È Ë Û˘ÓÔÏÈ΋ ¤ÎÊÚ·ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Â›Ó·È Û˘Ì‚·Ù‹ Ì ٷ ‰Â‰Ô̤ӷ ·Ï·ÈfiÙÂÚˆÓ ÂÚ¢ÓËÙÈÎÒÓ ÌÂÏÂÙÒÓ Ô˘ ·ÚÔ˘ÛÈ¿ÛÙËÎ·Ó Û ÚfiÛÊ·ÙË ∞ÌÂÚÈηÓÈ΋ ¤ÎıÂÛË. ™·Ê¤ÛÙÂÚ· ‹Ù·Ó Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ Û ۯ¤ÛË Ì ÙȘ ÂÚÈ‚·ÏÏÔÓÙÈΤ˜ ÂÎı¤ÛÂȘ Ô˘ ÌÂÏÂÙ‹ıËηÓ, ÔÈ Ôԛ˜ ‹Ù·Ó Û·ÊÒ˜ ·Ó‡·ÚÎÙ˜. O‡Ù ÔÈ ·ÎÙÈÓÔÁڷʛ˜ ηٿ ÙËÓ Î‡ËÛË, Ô˘ Á›ÓÔÓÙ·È Û¿ÓÈ·, Ô‡ÙÂ Ë Ôχ Û˘¯Ó‹ ˘ÂÚ˯ÔÁÚ·ÊÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË Û¯ÂÙ›ÛÙËÎ·Ó Ì ·È‰È΋ Ï¢¯·ÈÌ›· Î·È Ë ‰È·ÌÔÓ‹ ÛÙÔ ÈÛfiÁÂÈÔ ˆ˜
Paediatriki 2001;64:535-546
‰Â›ÎÙ˘ Èı·Ó‹˜ ¤ÎıÂÛ˘ Û ڷ‰fiÓÈÔ ‰ÂÓ ‚Ú¤ıËÎ·Ó Ó· ·ÔÙÂÏÔ‡Ó ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ·È‰È΋ Ï¢¯·ÈÌ›·. ∂ÈϤÔÓ, ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜ Ô˘ ÂÚ‡ÓËÛ·Ó ÙË Û¯¤ÛË ˘ÂÚ˯ÔÁÚ·Ê‹Ì·ÙÔ˜ Î·È ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ‰ÂÓ ·Ó¤ÊÂÚ·Ó Û˘Û¯¤ÙÈÛË. ∏ ¯Ú‹ÛË ËÏÂÎÙÚÈ΋˜ ı¤ÚÌ·ÓÛ˘ Î·È ÛÙÂÁÓˆÙ‹Ú· Ì·ÏÏÈÒÓ ·Ú¿ÁÂÈ ˘„ËÏ‹˜ ¤ÓÙ·Û˘ ËÏÂÎÙÚÔÌ·ÁÓËÙÈο ‰›·, ·ÏÏ¿ ÔÈ Û˘Û¯ÂÙ›ÛÂȘ Ô˘ ÛËÌÂÈÒıËÎ·Ó ÛÙË ÌÂϤÙË ‹Ù·Ó ÂÏ¿¯ÈÛÙ˜ Î·È ÛÙ·ÙÈÛÙÈο ÌË ÛËÌ·ÓÙÈΤ˜, fiˆ˜ ·Ó¤ÊÂÚ·Ó Î·È ÚÔËÁÔ‡ÌÂÓ˜ ÌÂϤÙ˜ (49). ∏ ·Ó·ÌÂÓfiÌÂÓË, Â‰Ò Î·È ¯ÚfiÓÈ·, ÌÂϤÙË ·fi ÙË µÚÂÙ·ÓÈ΋ ÔÌ¿‰· ÌÂϤÙ˘ ·È‰ÈÎÔ‡ ηÚΛÓÔ˘ (UKCCSG) ‰ËÌÔÛȇıËΠÛÙȘ 4 ¢ÂÎÂÌ‚Ú›Ô˘ ÙÔ˘ 1999 (50) ÛÙÔ Lancet. ¶ÚfiÎÂÈÙ·È ÁÈ· ÌÂÁ¿ÏË Û˘ÓÂÚÁ·ÙÈ΋ ÌÂϤÙË Ô˘ ·ÊÔÚ¿ 2226 ‚·ÛÈΤ˜ ÂÚÈÙÒÛÂȘ Ï¢¯·ÈÌ›·˜ ·È‰È΋˜ ËÏÈΛ·˜ Î·È ›‰ÈÔ ·ÚÈıÌfi Ì·ÚÙ‡ÚˆÓ. ∏ ÂÎÙ›ÌËÛË Ù˘ ¤ÎıÂÛ˘ ¤ÁÈÓ Ì ÌÂÙÚ‹ÛÂȘ ÛÙÔ Û›ÙÈ Î·È ÙÔ Û¯ÔÏÂ›Ô Î·È ÛÙË Û˘Ó¤¯ÂÈ· ÛÙ¿ıÌÈÛË ÙˆÓ ÌÂÙÚ‹ÛÂˆÓ ·˘ÙÒÓ Ì ÚÔÙ˘ÔÔÈË̤Ó˜ ÌÂÙÚ‹ÛÂȘ ÙˆÓ ÊÔÚÙ›ˆÓ ÙˆÓ ËÏÂÎÙÚÈÎÒÓ ÁÚ·ÌÌÒÓ Î·È ÙˆÓ Â‰›ˆÓ ÙˆÓ ËÏÂÎÙÚÈÎÒÓ Û˘Û΢ÒÓ ÁÈ· ¤Ó· ¯ÚfiÓÔ ÚÈÓ ÙË ‰È¿ÁÓˆÛË. °È· ·È‰È¿ Ì ‰È¿ÌÂÛË ¤ÎıÂÛË >0,2 Ì∆ Û ۇÁÎÚÈÛË Ì ·È‰È¿ Ì ‰È¿ÌÂÛË ¤ÎıÂÛË <0,1 Ì∆, Ô Û¯ÂÙÈÎfi˜ ΛӉ˘ÓÔ˜ ‹Ù·Ó: - 0,92 (95% O∞ 0,47-1,79) ÁÈ· ÙËÓ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· - 0,90 (95% O∞ 0,49-1,63) ÁÈ· fiϘ ÙȘ Ï¢¯·È̛˜ - 0,46 (95% O∞ 0,11-1,86) ÁÈ· fiÁÎÔ˘˜ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ - 0,97 (95% O∞ 0,46-2,05) ÁÈ· ¿ÏÏÔ˘˜ Û˘Ì·Á›˜ fiÁÎÔ˘˜ - 0,87 (95% O∞ 0,47-1,79) ÁÈ· ·È‰ÈÎfi ηÚΛÓÔ ÁÂÓÈο. À„ËϤ˜ ÂÎı¤ÛÂȘ (>0,4 Ì∆) ·Ó·Ê¤ÚıËÎ·Ó ÌfiÓÔ Û 17 (<0,4%) ·È‰È¿ (8 ÂÚÈÙÒÛÂȘ, 9 Ì¿ÚÙ˘Ú˜). ∏ ÌÂϤÙË ˘ÔÛÙËÚ›˙ÂÈ fiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ÁÈ· Û˘Û¯¤ÙÈÛË ¤ÎıÂÛ˘ Û ̷ÁÓËÙÈο ‰›·, ÔÊÂÈÏfiÌÂÓ· Û ·ÚÔ¯¤˜ ËÏÂÎÙÚÈ΋˜ ÂÓ¤ÚÁÂÈ·˜ Î·È ·˘ÍËÌ¤ÓˆÓ ÎÈÓ‰‡ÓˆÓ ÁÈ· Ï¢¯·ÈÌ›·, fiÁÎÔ˘˜ ÂÁÎÂÊ¿ÏÔ˘ ‹ ÔÔÈ·‰‹ÔÙ ¿ÏÏË ÌÔÚÊ‹ ·È‰ÈÎÔ‡ ηÚΛÓÔ˘. ∏ ¶·ÁÎfiÛÌÈ· OÚÁ¿ÓˆÛË ÀÁ›·˜ (WHO), Û ۯfiÏÈfi Ù˘ Ô˘ ‰ËÌÔÛȇÂÙ·È Û˘Á¯ÚfiÓˆ˜ (51), ‰¤¯ÂÙ·È fiÙÈ ÚfiÎÂÈÙ·È ÁÈ· ÌÂÁ¿ÏË Î·È ÛˆÛÙ¿ ‰ÔÌË̤ÓË ÌÂϤÙË, ·ÏÏ¿ ‰ÂÓ ÙË ıˆÚ› “ηıÔÚÈÛÙÈ΋” fiˆ˜ ›ÛÙ¢·Ó ÔÏÏÔ› ÂÚ¢ÓËÙ¤˜ Î·È ÂȉËÌÈÔÏfiÁÔÈ. O ÚÒÙÔ˜ ÏfiÁÔ˜ Â›Ó·È fiÙÈ Ë ÌÂϤÙË Û¯Â‰È¿ÛÙËΠÔÏÏ¿ ¯ÚfiÓÈ· ÚÈÓ (·fi ÙÔ 1988) Î·È ¤ÙÛÈ Ë ÂÎÙ›ÌËÛË Ù˘ ¤ÎıÂÛ˘ ‚·Û›ÛÙËΠ̤۠Û˜ - ‰È·¯ÚÔÓÈο - ÙÈ̤˜ ‰›ˆÓ (time-weighted average, TWA). ∏ ̤ıÔ‰Ô˜ ·˘Ù‹ ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› Û ·ÚÎÂÙ¤˜ ÌÂϤÙ˜ ηٿ ÙÔ ·ÚÂÏıfiÓ ·ÏÏ¿ ‰ÂÓ Û˘Û¯ÂÙ›˙ÂÙ·È Ì οÔÈÔ Ì˯·ÓÈÛÌfi ›‰Ú·Û˘ ÙˆÓ
543
¶·È‰È·ÙÚÈ΋ 2001;64:535-546
¯·ÌËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ‰›ˆÓ ÛÙÔ˘˜ ÈÛÙÔ‡˜. ∏ WHO ÁÈ· ÌÂÏÏÔÓÙÈΤ˜ ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Î·È ÁÈ· ÏfiÁÔ˘˜ Û‡ÁÎÚÈÛ˘ Û˘ÓÈÛÙ¿ ÙËÓ TWA Û·Ó Ì¤ıÔ‰Ô ÂÎÙ›ÌËÛ˘, ·ÏÏ¿ ··Ú·Èًو˜ ÙÔ ÚˆÙfiÎÔÏÏÔ ÂÎÙ›ÌËÛ˘ Ù˘ ¤ÎıÂÛ˘ Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ¿ÏÏÔ˘˜ ÙÚfiÔ˘˜ ̤ÙÚËÛ˘ Ù˘ ¤ÎıÂÛ˘ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔ˘˜ ÁÓˆÛÙÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜ ›‰Ú·Û˘ ÙˆÓ Â‰›ˆÓ ·˘ÙÒÓ (52). O ‰Â‡ÙÂÚÔ˜ ÏfiÁÔ˜ Â›Ó·È Ô ¯·ÌËÏfi˜ ·ÚÈıÌfi˜ ·È‰ÈÒÓ ÛÙȘ ˘„ËÏfiÙÂÚ˜ ηÙËÁÔڛ˜ ¤ÎıÂÛ˘. ™‡Ìʈӷ Ì ÙËÓ ·ÓˆÙ¤Úˆ ÌÂϤÙË, ÌfiÓÔ 2,3% ÙˆÓ Ì·ÚÙ‡ÚˆÓ ÂÎÙ¤ıËΠ̷۠ÁÓËÙÈο ‰›· >0,2 Ì∆. ∞Ó Î·È ·˘Ùfi ÙÔ ÔÛÔÛÙfi Â›Ó·È ·ÚfiÌÔÈÔ Ì ÂΛÓÔ Ù˘ °ÂÚÌ·Ó›·˜ (2%) (53), ÛÙË ÌÂϤÙË Ù˘ ∞ÌÂÚÈ΋˜ (48) ‹Ù·Ó 11,4% Î·È ÛÙËÓ ∫·Ó·‰È΋ ÌÂϤÙË 15,4% (54). ∏ ‰È·ÊÔÚ¿ ·˘Ù‹ ÌÂٷ͇ ÙˆÓ ‰‡Ô ËÂ›ÚˆÓ ·ÓÙ·Ó·ÎÏ¿ fi¯È ÌfiÓÔ ÙË ‰È·ÊÔÚ¿ Ù¿Û˘ ÛÙ· ‰‡Ô Û˘ÛÙ‹Ì·Ù· ·ÚÔ¯‹˜ ËÏÂÎÙÚÈ΋˜ ÂÓ¤ÚÁÂÈ·˜ (110 V ¤Ó·ÓÙÈ 220 V) ·ÏÏ¿ Î·È ÙË ‰È·ÓÔÌ‹ ÙÔ˘ Ú‡̷ÙÔ˜ Û ۯ¤ÛË Ì ÙȘ ηψ‰ÈÒÛÂȘ Î·È ÙË Á›ˆÛË ÙˆÓ ·ÚÔ¯ÒÓ. ¶ÚfiÛÊ·Ù·, ›Û˘, ‰ËÌÔÛȇÙËÎ·Ó Ù· ‰Â‰Ô̤ӷ ‰‡Ô Ó¤ˆÓ ÌÂÏÂÙÒÓ ·fi ÙË ¡¤· ∑ËÏ·Ó‰›· (55) Î·È ÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ OÓÙ¿ÚÈÔ ÙÔ˘ ∫·Ó·‰¿ (56). ∏ ÚÒÙË ·ÚÔ˘ÛÈ¿˙ÂÈ ÙȘ ›‰È˜ ·Ù¤ÏÂȘ fiˆ˜ Î·È Ë µÚÂÙ·ÓÈ΋, Û‡Ìʈӷ Ì ÙÔ Û¯ÔÏÈ·ÛÌfi Ù˘ WHO, ·ÏÏ¿ Ù·˘Ùfi¯ÚÔÓ· ˘ÔÛÙËÚ›˙ÂÈ fiÙÈ ‰ÂÓ ˘Ê›ÛÙ·Ù·È ÛËÌ·ÓÙÈ΋ Û˘Û¯¤ÙÈÛË Ï¢¯·ÈÌ›·˜ Î·È Ì¤ÛˆÓ - ‰È·¯ÚÔÓÈο - ÙÈÌÒÓ Ì·ÁÓËÙÈÎÒÓ ‹ ËÏÂÎÙÚÈÎÒÓ Â‰›ˆÓ 50 Hz ÛÙÔ ˘ÓÔ‰ˆÌ¿ÙÈÔ ÙÔ˘ ·È‰ÈÔ‡ Î·È ÙÔ Î·ıÈÛÙÈÎfi Û˘Ó‰˘·Ṳ̂ӷ. ∏ ‰Â‡ÙÂÚË ·Ó·Ê¤ÚÂÈ fiÙÈ Ù· Â˘Ú‹Ì·Ù¿ ÙÔ˘˜ ‰ÂÓ ˘ÔÛÙËÚ›˙Ô˘Ó Û˘Û¯¤ÙÈÛË ÌÂٷ͇ Ï¢¯·ÈÌ›·˜ Î·È ÁÂÈÙÓ›·Û˘ Ì ËÏÂÎÙÚÈΤ˜ ÁÚ·Ì̤˜ ˘„ËÏ‹˜ Î·È ˘ÂÚ˘„ËÏ‹˜ Ù¿Û˘. ™ÙÔ ¿ÌÂÛÔ Ì¤ÏÏÔÓ ·Ó·Ì¤ÓÔÓÙ·È Ù· ·ÔÙÂϤÛÌ·Ù· Ì›·˜ ·Ó¿Ï˘Û˘ ·fi ÙËÓ ∂˘Úˆ·˚΋ ŒÓˆÛË Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È Ù· ‰Â‰Ô̤ӷ Ù˘ µÚÂÙ·ÓÈ΋˜ ÌÂϤÙ˘. ∂›Û˘, Ì›· ÌÂÁ¿ÏË ÌÂϤÙË ·Ó·Ì¤ÓÂÙ·È ·fi ÙÔ ∂ıÓÈÎfi πÓÛÙÈÙÔ‡ÙÔ ¶ÂÚÈ‚·ÏÏÔÓÙÈÎÒÓ ÌÂÏÂÙÒÓ Ù˘ π·ˆÓ›·˜. ∏ ÌÂϤÙË ·˘Ù‹ ı· Ï¿‚ÂÈ ˘’ fi„ÈÓ Ù˘ ÙËÓ ÂÚÈÔ‰ÈÎfiÙËÙ· ÙˆÓ Ì·ÁÓËÙÈÎÒÓ Â‰›ˆÓ ηٿ ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ¤ÎıÂÛ˘. ¶ÂÚ›Ô˘ 1500 ‚·ÛÈΤ˜ ÂÚÈÙÒÛÂȘ (1000 Ï¢¯·ÈÌ›·˜ Î·È 500 fiÁÎˆÓ ÂÁÎÂÊ¿ÏÔ˘) ı· Û˘ÌÂÚÈÏËÊıÔ‡Ó Î·È ·Ó·Ì¤ÓÂÙ·È ˘„ËÏfi˜ ·ÚÈıÌfi˜ ÂÎÙÂıÂÈÌ¤ÓˆÓ ÛÙȘ Ôχ ˘„ËϤ˜ ηÙËÁÔڛ˜ ¤ÎıÂÛ˘, ÏfiÁˆ Ù˘ ‚·ÚÈ¿˜ ‚ÈÔÌ˯·Ó›·˜ ÛÙË ¯ÒÚ· ·˘Ù‹. ∏ ÌÂϤÙË ·˘Ù‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙȘ ÌÂϤÙ˜ Ô˘ Û˘Ó¯›˙ÔÓÙ·È ÛÙËÓ ∞ÌÂÚÈ΋, °ÂÚÌ·Ó›·, πÙ·Ï›· ›Ûˆ˜ ‚ÔËı‹ÛÔ˘Ó ÛÙËÓ Â›Ï˘ÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜, ··ÓÙÒÓÙ·˜ ÛÙË ‰È·Ù˘ˆı›۷ ˘fiıÂÛË Â¿Ó Ú·ÁÌ·ÙÈο ˘Ê›ÛÙ·Ù·È ·˘ÍË̤ÓÔ˜ ΛӉ˘ÓÔ˜ Ï¢¯·ÈÌ›·˜ ·È‰È΋˜ ËÏÈΛ·˜ ·fi ÙËÓ ¤ÎıÂÛË ÛÙ· Ì·ÁÓËÙÈο ‰›· ‹ Â¿Ó Ë
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·ÛıÂÓ‹˜ Û˘Û¯¤ÙÈÛË Â›Ó·È ı¤Ì· Ù‡¯Ë˜. ¶ÔÏÏÔ› ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ ÔÈ Ï¢¯·È̛˜ ÛÙ· ·È‰È¿ Â›Ó·È Ì›· ÔÌ¿‰· ‚ÈÔÏÔÁÈο ·ÏÏ¿ Î·È ·ÈÙÈÔÏÔÁÈο ÂÙÂÚÔÁÂÓ‹˜ Î·È fiÙÈ ÔÈ ÂȉڿÛÂȘ ·fi ÙȘ ÂÎı¤ÛÂȘ Û ÂÚÈ‚·ÏÏÔÓÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÂÍ·ÚÙÒÓÙ·È fi¯È ÌfiÓÔ ·fi Ù· ›‰· ¤ÎıÂÛ˘ ·ÏÏ¿ Î·È ·fi ÙË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ Ô˘ Û˘Ì‚·›ÓÂÈ (.¯. ÚÈÓ ÙË Û‡ÏÏË„Ë, ηٿ ÙËÓ Î‡ËÛË, ÌÂÙ¿ ÙË Á¤ÓÓËÛË) (57). OÈ ÌÂÏÏÔÓÙÈΤ˜ ÌÂϤÙ˜ ÁÈ· ÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ ı· Ú¤ÂÈ Ó· ۯ‰ȿ˙ÔÓÙ·È Ì ÌÂÁ¿ÏÔ ·ÚÈıÌfi ‰Â›ÁÌ·ÙÔ˜, ¤ÙÛÈ ÒÛÙ ÔÈ ÂȉڿÛÂȘ ·fi ÙȘ ‰È¿ÊÔÚ˜ ÂÎı¤ÛÂȘ Û ‰È·ÊÔÚÂÙÈο ÛÙ¿‰È· ·Ó¿Ù˘Í˘ Ó· ÂÎÙÈÌÒÓÙ·È Ì¤Û· Û ηÏÒ˜ ۯ‰ȷṲ̂Ó˜ ˘ÔÔÌ¿‰Â˜ Ï¢¯·ÈÌÈÒÓ. ∏ ·Ô˘Û›· ¿ÌÂÛ˘ ‚ÈÔÏÔÁÈ΋˜ ¤Ó‰ÂÈ͢ ÁÈ· ÏÔÈÌÒ‰Ë ·ÈÙ›· Ù˘ ·È‰È΋˜ Ï¢¯·ÈÌ›·˜ Ô‰ËÁ› ÛÙËÓ ·Ó·˙‹ÙËÛË Ó¤ˆÓ ·ÈÙÈÔÏÔÁÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Ì ÂÚ·ÈÙ¤Úˆ ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜. ™ÙËÓ ∞ÌÂÚÈ΋, Ë ÔÌ¿‰· ÌÂϤÙ˘ ÙÔ˘ ·È‰ÈÎÔ‡ ηÚΛÓÔ˘ (CCSG) ÂÂÍÂÚÁ¿˙ÂÙ·È Ù· ‰Â‰Ô̤ӷ 2500 ÂÚÈÙÒÛÂˆÓ ÔÍ›·˜ Ï¢¯·ÈÌ›·˜ ·fi ÙÔ 1988 (58). ∂ÈÎÂÓÙÚÒÓÔ˘Ó ÙËÓ ¤ÚÂ˘Ó¿ ÙÔ˘˜ ȉȷ›ÙÂÚ· ÛÙ· ËÏÂÎÙÚÔÌ·ÁÓËÙÈο ‰›·, ÙÔ Ú·‰fiÓÈÔ Î·È Û ÂÎı¤ÛÂȘ ÙˆÓ ÁÔÓ¤ˆÓ Î·È ÙˆÓ ·È‰ÈÒÓ ÙÔ˘˜ Û ¯ËÌÈο Î·È ÂÓÙÔÌÔÎÙfiÓ·, ·ÏÏ¿ Û˘Á¯ÚfiÓˆ˜ Û˘ÏϤÁÔ˘Ó ÏËÚÔÊÔڛ˜ Î·È ‰Â‰Ô̤ӷ Û¯ÂÙÈο Ì ÙË ÏÔÈÌÒ‰Ë ˘fiıÂÛË. ™ÙË °ÂÚÌ·Ó›·, ›Û˘, ÍÂΛÓËÛ ÙÔ 1988 (59) Ì›· ·ÓÂıÓÈ΋ ÂȉËÌÈÔÏÔÁÈ΋ Û˘ÁÎÚÈÙÈ΋ ÌÂϤÙË ÙÔ˘ ηÚΛÓÔ˘ ÛÙ· ·È‰È¿, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ Ï¢¯·ÈÌ›·˜. ∏ ÌÂϤÙË ·˘Ù‹ ÂÂÍÂÚÁ¿˙ÂÙ·È ‰È¿ÊÔÚ˜ ˘Ôı¤ÛÂȘ ÁÈ· ÙË Ï¢¯·ÈÌ›· Î·È ¿ÏÏÔ˘˜ ηÚΛÓÔ˘˜ ÛÙ· ·È‰È¿, fiˆ˜ ¤ÎıÂÛË ÛÙË Ê˘ÛÈ΋ ÈÔÓ›˙Ô˘Û· ·ÎÙÈÓÔ‚ÔÏ›· (Ú·‰fiÓÈÔ, ·ÎÙ›Ó˜ Á), ËÏÂÎÙÚÈο Î·È Ì·ÁÓËÙÈο ‰›·, ¤ÎıÂÛË ÙˆÓ ÁÔÓ¤ˆÓ Î·È ÙÔ˘ ·ÛıÂÓÔ‡˜ Û ¯ËÌÈο ‹ ·ÎÙÈÓÔ‚ÔÏ›·, ÎÏËÚÔÓÔÌÔ‡ÌÂÓË ÚԉȿıÂÛË Î·È ÙËÓ ¤ÎıÂÛË Û ÏÔÈÌÒÍÂȘ. ∏ ÚfiÔ‰Ô˜ ÛÙȘ ÌÂıfi‰Ô˘˜ ˘ÔÏÔÁÈÛÌÔ‡ Ù˘ ÂÚÈ‚·ÏÏÔÓÙÈ΋˜ ¤ÎıÂÛ˘, fiˆ˜ ›Û˘ Î·È Ë ·Ó‡ÚÂÛË ÁÂÓÂÙÈÎÒÓ ÚԉȷıÂÛÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Î·È ÙˆÓ ·ÏÏËÏÂȉڿÛÂÒÓ ÙÔ˘˜ Ì ÙÔ˘˜ ÂÚÈ‚·ÏÏÔÓÙÈÎÔ‡˜, Â›Ó·È Èı·Ófi Ó· ‚ÂÏÙÈÒÛÂÈ ÙȘ ÁÓÒÛÂȘ Ì·˜ Û¯ÂÙÈο Ì ÙË Û˘¯Ó‹ ·˘Ù‹ ÔÌ¿‰· ηÎÔËıÂÈÒÓ ÛÙ· ·È‰È¿. ∂›Û˘, Â›Ó·È Èı·Ófi Ó· ˘¿ÚÍÔ˘Ó ÂÚÈÛÛfiÙÂÚ· ÛÙÔȯ›· ÁÈ· ÙË ‰È·Ù˘ˆı›۷ ˘fiıÂÛË Ù˘ “›‰Ú·Û˘ ÙˆÓ ÏÔÈÌÒÍÂˆÓ Ù˘ ÚÒÙ˘ ·È‰È΋˜ ËÏÈΛ·˜” ÛÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ Ï¢¯·ÈÌ›·˜ ÛÙ· ·È‰È¿. À¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ Ô˘ ·ÎÔÏÔ˘ıÔ‡Ó ÂΛӘ Ù˘ ηı˘ÛÙÂÚË̤Ó˘ ¤ÎıÂÛ˘ Û ÏÔÈÌÒÍÂȘ Ô˘ ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ Ô Î›Ó‰˘ÓÔ˜ ÁÈ· ÙËÓ ÎÔÈÓÔ‡ Ù‡Ô˘ O§§ ·˘Í¿ÓÂÙ·È ÛÂ Û˘Û¯¤ÙÈÛË Ì ÔÚÈṲ̂ÓÔ˘˜ ˘fiÙ˘Ô˘˜ (60) ÙÔ˘ ªÂ›˙ÔÓÔ˜ ™˘ÌϤÁÌ·ÙÔ˜ πÛÙÔÛ˘Ì‚·ÙfiÙËÙ·˜, fiÙ·Ó Û˘Ó˘¿Ú¯ÂÈ ·Ô˘Û›· ·Ú·ÙÂٷ̤ÓÔ˘ ıËÏ·ÛÌÔ‡, ÌÂȈ̤Ó˜ ÏÔÈÌÒÍÂȘ ÛÙË
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52. McKinlay AF, Repacholi MH, eds. Exposure metrics and dosimetry for EMF epidemiology: Proceedings of an International Workshop held at the National Radiological Protection Board, Chilton, UK, Sept 7-9, 1998. Radiation Protection Dosimetry 1999;83:1-194. 53. Michaelis J, Schuz H, Meiner R, Zemann E, Grigat JP, Kaatsch et al. Combined risk estimates for two German population-based case-control studies on residential magnetic fields and childhood adult leukemia. Epidemiology 1998;9:92-94. 54. McBride ML, Gallagher RP, Thériault G, Armstrong BG, Tamaro S, Spinelli JJ et al. Power-frequency electric and magnetic fields and risk of childhood leukemia in Canada. Am J Epidemiol 1999;149:831-842. 55. Dockerty JD, Elwood JM, Skegg DC, Herbison GP. Electromagnetic field exposures and childhood cancers in New Zealand. Cancer Causes Control 1998;9:299-300. 56. Green L, Miller B, Villeneuve P, Greenberg M, Donnelly E. A case-control study of childhood leukemia in southern Ontario, Canada, and exposure to magnetic fields in residences. Internat J Cancer 1999;82:161-170. 57. Cnattingius S, Zack M, Ekbom A, Gunnarskog J, Linet M, Adami HO. Prenatal and neonatal risk factors for childhood myeloid leukemia. Cancer Epidemiol Biomarkers Prev 1995;4:441-445. 58. Robison LL, Buckley JD, Bumin G. Assessment of environmental and genetic factors in the etiology of childhood cancers: the children’s cancer group epidemiology programm. Env Health Perspectives 1995;103:111-116. 59. Kaatsch P, Kaletsch U, Krummenauer F, Meinert R, Miesner A, Haaf G et al. Case control study on childhood leukemia in Lower Saxony, Germany. Basic considerations, methodology, and summary of results. Klin Pediatr 1996;208:179-185. 60. Taylor GM, Birch JM. The hereditary basis of human leukemia. In: Henderson ES, Lister TA, Greaves MF, eds. Leukemia. 6th ed. WB Saunders; 1996. p. 34-45.
HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 13-12-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 30-04-2001 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∞fiÛÙÔÏÔ˜ ¶Ô˘ÚÙÛ›‰Ë˜ ∞Ê·›·˜ 14, 154 54 æ˘¯ÈÎfi
¶·È‰È·ÙÚÈ΋ 2001;64:132-135
Paediatriki 2001;64:132-135
¶PO™EXH ™YNE¢PIA 9-12 ™ÂÙÂÌ‚Ú›Ô˘ 2001
European Asthma Congress Congress Secretariat Dr. T. Slavyanskaya 16/10 Miklukho-Maklaya street Moscow, 117997 Russia Tel.: (++7 -095) 336-5000 & 429-9620 Fax: (++7 -095) 336-5000 E-mail: acicis@ibch.ru Web site: http://www.isir.ru
ªfiÛ¯·, ƒˆÛ›·
9-14 ™ÂÙÂÌ‚Ú›Ô˘ 2001
11th International Clinical Genetics Seminar "The Genetics of Diabetes Mellitus" ¶ÏËÚÔÊÔڛ˜: Triaena Tours and Congress S.A ∆ËÏ.: 01 7499 300 Fax: 01 7713 795 E-mail: congress@triaenatours.gr
∏Ú¿ÎÏÂÈÔ
9-14 ™ÂÙÂÌ‚Ú›Ô˘ 2001
23rd International Congress of Pediatrics ¶ÏËÚÔÊÔڛ˜: Chinese Medical Association 42 Dongsi Xidajie, Beijing 100710, China ∆ËÏ.: 86-10-6527 8804 / 6527 8803 Fax: 86-10-65-123-754 E-mail: 231CP@chinamed.com.cn.
¶ÂΛÓÔ, ∫›Ó·
23-27 ™ÂÙÂÌ‚Ú›Ô˘ 2001
World Congress of Perinatal Medicine ¶ÏËÚÔÊÔڛ˜: Santiago Dexeus Font Foundation Paeso Bonanova 67, 08017 Barcelona ∆ËÏ.: +34 (93) 227 4709 Fax: +34 (93) 418 78 32 E-mail: Fundacio@indexus.uab.es
µ·ÚÎÂÏÒÓË, πÛ·Ó›·
27-30 ™ÂÙÂÌ‚Ú›Ô˘ 2001
24Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ÃÂÈÚÔ˘ÚÁÈ΋˜ ¶·›‰ˆÓ °Ú·ÌÌ·Ù›·: ªÂıÒÓ˘ 64 106 83, ∞ı‹Ó· ∆ËÏ.: (01) 330 62 50 Fax: (01) 330 62 40 E-mail: charissi@her.forthnet.gr
∞ı‹Ó·
xiii
¶PO™EXH ™YNE¢PIA 2-6 OÎÙˆ‚Ú›Ô˘ 2001
IXth Congress of European Society for the study and prevention of infant death ESPID, Prenatal Health Care and Postnatal Development Organizing Secretariat: Figure Congress Organization Ayazmaderesi Cad. Karadut Sk. No: 780888 Dikilitas, Istanbul, Turkey Tel.: +90 212 258 60 20 Fax: +90 212 258 60 78 E-mail: espid@figur.net
6-7 OÎÙˆ‚Ú›Ô˘ 2001
11Ë ∂ÈÛÙËÌÔÓÈ΋ ∂Ή‹ÏˆÛË πˆ¿ÓÓÈÓ· ¶·È‰È·ÙÚÈ΋˜ ∆Ô̤·˜ YÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡, π·ÙÚÈ΋ ™¯ÔÏ‹ ¶·ÓÂÈÛÙËÌ›Ô˘ πˆ·ÓÓ›ÓˆÓ •ÂÓÔ‰Ô¯Â›Ô Du Lac °Ú·ÌÌ·Ù›·: ∆ËÏ.: (0651) 97 546 & 97 544 Fax: (0651) 97 032 Web site: http://www.uoi.gr/conf_sem/11paediatr
20-21 OÎÙˆ‚Ú›Ô˘ 2001
¢ÈÂÈÛÙËÌÔÓÈÎfi ™˘Ó¤‰ÚÈÔ πÓÛÙÈÙÔ‡ÙÔ˘ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ °Ú·ÌÌ·Ù›·: C&C International A.E ¶·Ú·‰Â›ÛÔ˘ 16, 151 24 ∞ı‹Ó· ∆ËÏ.: (01) 68 89 100 Fax: (01) 68 44 777 E-mail: congress@cnc.gr
∞ı‹Ó·
31 OÎÙ.-3 ¡ÔÂÌ‚Ú›Ô˘ 2001
¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ∞ÓÔÛÔÏÔÁ›·˜ °Ú·ÌÌ·Ù›·: AFEA Congress S.A §˘Î·‚ËÙÔ‡ 39-41, 106 72 ∞ı‹Ó· ∆ËÏ.: (01) 36 04 413 Fax: (01) 36 43 511 E-mail: info@afea.gr
∞ı‹Ó·
10 ¡ÔÂÌ‚Ú›Ô˘ 2001
14Ë ∂ÈÛÙËÌÔÓÈ΋ ∏ÌÂÚ›‰· ∂ÏÏËÓÈ΋ ∂Ù·ÈÚ›· ¶·È‰È·ÙÚÈ΋˜ ∞ÈÌ·ÙÔÏÔÁ›·˜-OÁÎÔÏÔÁ›·˜ “¡ÂÒÙÂÚ· ‰Â‰Ô̤ӷ ÛÙË ‰È¿ÁÓˆÛË Î·È ıÂڷ›· ∞ÈÌ·ÙÔÏÔÁÈÎÒÓ - OÁÎÔÏÔÁÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ Ù˘ ·È‰È΋˜ ËÏÈΛ·˜” ¶ÏËÚÔÊÔڛ˜: ∂ÏÏËÓÈ΋ ∂Ù·ÈÚ›· ¶·È‰È·ÙÚÈ΋˜ ∞ÈÌ·ÙÔÏÔÁ›·˜/OÁÎÔÏÔÁ›·˜ ∆ËÏ.: 7467 303-6 & 7792 200 Fax: 7792 200
∞ı‹Ó·
xiv
∫ˆÓ/ÔÏË, ∆Ô˘ÚΛ·
™YNTOMO°PAºIE™
ABBREVIATIONS
™˘ÓÙÔÌÔÁڷʛ˜
EÏÏËÓÈÎÔ› fiÚÔÈ
AÁÁÏÈÎÔ› fiÚÔÈ
AIDS
Û‡Ó‰ÚÔÌÔ Â›ÎÙËÙ˘ ·ÓÔÛÔ·Ó¿ÚÎÂÈ·˜
AMP, ADP, ATP
ÌÔÓÔ-, ‰È-, ÙÚÈʈÛÊÔÚÈ΋ ·‰ÂÓÔÛ›ÓË
ATPase cal CSF ‹ ENY CNS ‹ ∫¡™ cm CoA cmÑ cm2 cAMP ÆC CRP DNA ECG ‹ ∏∫° EEG ‹ ∏∂° ELISA ESR ‹ ∆∫∂ EDTA g Hct Hb HLA
ÙÚÈʈÛÊ·Ù¿ÛË Ù˘ ·‰ÂÓÔÛ›Ó˘ ıÂÚÌ›‰· ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· ÂηÙÔÛÙfi Û˘Ó¤Ó˙˘ÌÔ ∞ ΢‚ÈÎfi ÂηÙÔÛÙfi ÙÂÙÚ·ÁˆÓÈÎfi ÂηÙÔÛÙfi ΢ÎÏÈÎfi AMP ‚·ıÌfi˜ KÂÏÛ›Ô˘ C-·ÓÙȉÚÒÛ· ÚˆÙ½ÓË ‰ÂÔ͢ÚÈ‚Ô˙ÔÓÔ˘ÎÏÂ˚ÓÈÎfi Ô͇ ËÏÂÎÙÚÔηډÈÔÁÚ¿ÊËÌ· ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ· ¤Ó˙˘ÌÔ-Û˘Ó‰¤Ù˘ ·ÓÔÛÔÚÔÛÚfiÊËÛ˘ ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù·¯‡ÙËÙ· ηıÈ˙‹Ûˆ˜ ÂÚ˘ıÚÒÓ Âı˘ÏÂÓԉȷÌÈÓÔÙÂÙÚ·ÔÍÂ˚Îfi Ô͇ ÁÚ·ÌÌ¿ÚÈo ·ÈÌ·ÙÔÎÚ›Ù˘ ·ÈÌÔÛÊ·ÈÚ›ÓË ·ÓÙÈÁfiÓÔ ÈÛÙÔÛ˘Ì‚·ÙfiÙËÙ·˜
Ig ICU ‹ ª∂£ IU i.m. ‹ ∂.ª. i.p. ‹ ∂.¶. i.v. ‹ ∂.º. L log mol ªW ‹ MB MCH MCV mRNA m min n N NS ‹ ª™ Ôsm % PCR pH PCO2 PO2 p
·ÓÔÛÔÛÊ·ÈÚ›ÓË ÌÔÓ¿‰· ÂÓÙ·ÙÈ΋˜ ıÂڷ›·˜ ‰ÈÂıÓ‹˜ ÌÔÓ¿‰· ÂÓ‰ÔÌ˘˚ο ÂÓ‰ÔÂÚÈÙÔÓ·˚ο ÂÓ‰ÔÊϤ‚È· Ï›ÙÚÔ(·) ÏÔÁ¿ÚÈıÌÔ˜ ÁÚ·ÌÌÔÌfiÚÈÔ ÌÔÚÈ·Îfi ‚¿ÚÔ˜ ̤ÛË ·ÈÌÔÛÊ·ÈÚ›ÓË ÂÚ˘ıÚÒÓ Ì¤ÛÔ˜ fiÁÎÔ˜ ÂÚ˘ıÚÒÓ ·ÁÁÂÏÈÔÊfiÚÔ˜ RNA ̤ÙÚÔ ÏÂÙfi ·ÚÈıÌfi˜ Ó¢ÙÒÓÈÔ ÌË ÛËÌ·ÓÙÈÎfi ÔÛÌÒÏÈÔ Â› ÙÔȘ ÂηÙfi ·Ï˘ÛȉˆÙ‹ ·ÓÙ›‰Ú·ÛË ÔÏ˘ÌÂÚ¿Û˘ Û˘ÁΤÓÙÚˆÛË ÈfiÓÙˆÓ ˘‰ÚÔÁfiÓÔ˘ ÌÂÚÈ΋ Ù¿ÛË CO2 ÌÂÚÈ΋ Ù¿ÛË Ô͢ÁfiÓÔ˘ Èı·ÓfiÙËÙ·
acquired immunodeficiency syndrome adenosine 5mono-, di-, triphosphate adenosine triphosphatase calorie cerebrospinal fluid central nervous system centimeter coenzyme A cubic centimeter square centimeter cyclic AMP degree(s) Celsius C-reactive protein deoxyribonucleic acid electrocardiogram electroencephalogram enzyme-linked immunoabsorbent assay erythrocyte sedimentation rate ethylenediaminetetracetate gram haematocrit haemoglobin (human) histocompatibility leucocyte antigen immunoglobulin intensive care unit international Unit intramuscular intraperitoneal intravenous liter logarithm mole molecular weight mean corpuscular haemoglobin mean corpuscular volume messenger RNA meter minute number newton not significant osmole percent polymerase chain reaction potentia hydrogenii carbon dioxide pressure oxygen pressure probability
xvii
™˘ÓÙÔÌÔÁڷʛ˜
EÏÏËÓÈÎÔ› fiÚÔÈ
AÁÁÏÈÎÔ› fiÚÔÈ
p.o. RIA RNA RDS sec s.c. ‹ À.¢. SD SE U
·fi ÙÔ ÛÙfiÌ· Ú·‰ÈÔ·ÓÔÛÔ·ÔÚÚÔÊËÙÈ΋ ‰ÔÎÈÌ·Û›· ÚÈ‚ÔÓÔ˘ÎÏÂ˚ÓÈÎfi Ô͇ Û‡Ó‰ÚÔÌÔ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÈ·˜ ‰Â˘ÙÂÚfiÏÂÙÔ ˘Ô‰fiÚÈ· ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË ÛÙ·ıÂÚfi ÛÊ¿ÏÌ· ÌÔÓ¿‰·
by mouth radioimmunoassay ribonucleic acid respiratory distress syndrome second subcutaneous standard deviation standard error unit
™˘Ó‰˘·˙fiÌÂÓ· ÚÔı¤Ì·Ù· 12
tera- (10 ) giga- (10 ) mega-(10 ) kilo- (10 ) hecto(10 ) deca (10 ) deci (10 ) centi- (10 ) milli(10 ) micro (10 ) nano (10 ) pico (10 ) femto (10 ) atto (10 ) 9
6
3
2
1
-1
-2
-3
-6
-9
-12
-15
-18
xviii
Combining prefixes T G M k h da d c m Ì n p f a