Παιδιατρική | Τόμος 72 • Τεύχος 1 • Ιανουάριος - Φεβρουάριος - Μάρτιος 2009

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¢ÈÌËÓÈ·›· ¤Î‰ÔÛË Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜ ¶Úfi‰ÚÔ˜ ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘ ∫. ™ÙÂÊ·Ó›‰Ë˜ M¤ÏË ™. ∞Ó‰ÚÔÓ›ÎÔ˘ ª. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ ¶. ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘ °. µ·ÚÏ¿Ì˘ ∂. °·Ï·Ó¿Î˘ §. £ˆÌ·˝‰Ô˘ ª. ∫·Ó¿ÚÈÔ˘ A. K·ÙÙ¿Ì˘ ™. K›ÙÛÈÔ˘-∆˙¤ÏË ∞. ¶··‰ÔÔ‡ÏÔ˘ µ. ¶··Â˘·ÁÁ¤ÏÔ˘ ∞. ™È·ÌÔÔ‡ÏÔ˘-ª·˘Ú›‰Ô˘ ∞. ™˘Ú›ÁÔ˘-¶··‚·ÛÈÏ›Ԣ ∂. ÷ÚÌ·Ó‰¿ÚË ÀÔ‚ÔÏ‹ ∂ÚÁ·ÛÈÒÓ e-mail: hps@ath.forthnet.gr √‰ËÁ›Â˜ ÚÔ˜ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜: http://www.e-child.gr/Instructions_ to_Authors_GR.pdf

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¶ÂÚȯfiÌÂÓ· ∞¡∞™∫O¶∏™∂π™ 1 ÃÔÚ‹ÁËÛË ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ Û ·È‰È¿ Ì ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘: ·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ∫. ∫·Ú·‚·Ó¿ÎË-∫·Ú·Ó¿ÛÈÔ˘ 10 £ÂÚ·›· Ì ·˘ÍËÙÈ΋ ÔÚÌfiÓË ÛÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi E. ∫Ô‡ÛÙ·, Ã. ÷Ù˙Ë·ı·Ó·Û›Ô˘†, A. ¶··ı·Ó·Û›Ô˘ 16 ¡ÂÔÁÓÈΤ˜ ÏÔÈÌÒÍÂȘ ·fi ‚-·ÈÌÔÏ˘ÙÈÎfi ÛÙÚÂÙfiÎÔÎÎÔ Ù˘ ÔÌ¿‰·˜ µ Ã. ∆۷ηϛ‰Ë˜, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜, ¡. ¡ÈÎÔÏ·˝‰Ë˜ 23 ¶ÔÏ˘Û·Î¯·ÚȉÈο ·ÓÙÈÁfiÓ· – Û˘˙¢Á̤ӷ ÂÌ‚fiÏÈ·: ·ÓÔÛȷ΋ ÌÓ‹ÌË Î·È ·Ó·ÌÓËÛÙÈΤ˜ ‰fiÛÂȘ π.£. ∫·‚·ÏÈÒÙ˘

ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ· ∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ∫. ¶··¯Ú‹ÛÙÔ˘ EÈ̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ™. ¡¿ÎÔ˘ EΉfiÙ˘ K. °ÚÈ‚¤·˜ ™˘ÓÙÔÓÈÛÙ‹˜ ∂ΉfiÛˆ˜ Communications In Practice A.E. ¶ÈÂÚ›·˜ 1∞ 144 51 MÂÙ·ÌfiÚʈÛË TËÏ.: 210 87 78 884 Fax: 210 87 78 822 I‰ÈÔÎÙ‹Ù˘ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·© Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 Aı‹Ó· 115 28 TËÏ.: 210 7771 140 210 7771 663 Fax: 210 7758 354 e-mail: hps@ath.forthnet.gr EÙ‹ÛÈ· ™˘Ó‰ÚÔÌ‹: 40 € EȉÈ΢fiÌÂÓÔÈ, ºÔÈÙËÙ¤˜: 20 € ∫ˆ‰ÈÎfi˜ ¢È‡ı˘ÓÛ˘ ∂ÔÙ›·˜ ªª∂: 3889

ISSN 0377-2551

∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™ 29 ÃÔÚ‹ÁËÛË ‰‡Ô ·ÓÙÈ-TNF Û΢·ÛÌ¿ÙˆÓ (etanercept Î·È adalimumab) Û ·È‰È¿ Ì Ó·ÓÈ΋ ȉÈÔ·ı‹ ·ÚıÚ›Ùȉ·: 5 ¯ÚfiÓÈ· ÌÂÙ¿ ª. ∆Ú·¯·Ó¿, ¶. ¶Ú·ÙÛ›‰Ô˘-°Î¤ÚÙÛË, º. ∫·Ó·ÎÔ‡‰Ë-∆۷ηϛ‰Ô˘, ∫. ¢È¿Ê·, °. ¶·Ú‰·Ïfi˜, ª. ª·ÓÙÔ˘Ú¿ÎË 41 Obesity prevalence in children aged 8-13 years according to IOTF cutoffs, CDC growth charts and Z-BMI scores N. Mantzouranis, H. Douda, T. Pilianidis, S. Tokmakidis 46 ÃÚ‹ÛË Î·È Î·Ù¿¯ÚËÛË ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ Û ÊÔÈÙËÙ¤˜ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜ ™. ∞ÓÙˆÓÈ¿‰Ë˜, Ã. °È·‚Ú‹, ∂. ¶··Ì·Î·Ú›Ô˘, ∫. ™Ù·‡ÚÔ˘, ª. ∑¤Ú‚·, ∂. ¢ÈÔÓ˘ÛÔÔ‡ÏÔ˘, ∂. ª·ÎÚÔÁΛη, µ. ™·ÏˆÓ›‰Ë˜

∂¡¢π∞º∂ƒ√À™∂™ ¶∂ƒπ¶∆ø™∂π™ 51 梉ԸԷωÔÛÙÂÚÔÓÈÛÌfi˜ Ù‡Ô˘ 1 Û ‚Ú¤ÊÔ˜ Ã. ¢·ÛηÏÔÔ‡ÏÔ˘, £. ∆ÛÂÎÔ˘Ú¿, ∞. ∆ÛÈÏÈÌÈÁοÎË 55 π‰ÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË Û Ó·ÚÔ‡˜ ÂÊ‹‚Ô˘˜ Ì ·¯˘Û·ÚΛ· ∞. ºÈÏÈ›‰Ë, ™. ™ÙÂÊ·Ó¿ÎË, ª. ∞Ó·ÙÔÏȈٿÎË, °. µÏ·¯¿ÎË, Ã. º·ÓÔ˘ÚÈ¿Î˘, ∞. ∆ÛÈÏÈÌÈÁοÎË 60 ™˘ÁÁÂÓ‹˜ ¯˘ÏÔıÒڷη˜ Ì ˘Ô‰fiÚÈÔ Ô›‰ËÌ· ¿Óˆ ËÌ›ÛÂÔ˜ ÛÒÌ·ÙÔ˜ Û ÓÂÔÁÓfi Ì ۇӉÚÔÌÔ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ µ. ª·ÎÚ‹, ∞ÈÎ. ∫ˆÓÛÙ·ÓÙÈÓ›‰Ë, ª. ¶··‰ÔÔ‡ÏÔ˘, Ÿ. ¢Â‰Ô‡ÛË, ™. ∆¿ÓÙÚÔ˜, ∂. ∞Á·ËÙfi˜, ∞. °Ô‡Ó·Ú˘ ∫§π¡π∫√ ∫√Àπ∑ 66 ∂. ª·ÓÙ·‰¿Î˘, Õ. ∆Û·ÏΛ‰Ë˜, Õ. ƒ·Ì·Ù¿ÓË, ∂. ¶·Ú·Ûο΢, ∞. ÷Ù˙ËÌȯ·‹Ï ™À°Ãƒ√¡∞ £∂ª∞∆∞ µπ√™∆∞∆π™∆π∫∏™ 67 ¶ÚÔÔÙÈΤ˜ ÏËı˘ÛÌȷΤ˜ ÌÂϤÙ˜: Ë ÂÊ·ÚÌÔÁ‹ Ù˘ ÂȉËÌÈÔÏÔÁ›·˜ ÁÈ· ÙÔÓ Î·ıÔÚÈÛÌfi ÙˆÓ ·ÈÙÈÔÏÔÁÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ ∂. ∫ÚÈÙÛ¤ÏË, ¢.∞. ∫·ÊÂÙ˙‹˜ ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√ 70 http://www.paidiatros.com ∞. ÷Ù˙Ë·Ó·Á‹˜

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Bimonthly Publication of the Greek Paediatric Society

Paediatriki

President A. Constantopoulos Editorial Board Editor-in-Chief C. Stefanidis Members S. Andronikou ª. ∞nthracopoulos P. Augoustides-Savvopoulou G. Varlamis ∂. Galanakis L. Thomaidou M. Kanariou A. Kattamis S. Kitsiou-Tzeli ∞. Papadopoulou V. Papaevagelou A. Siamopoulou-Mavridou A. Syrigou-Papavasiliou E. Charmandari

Volume 72 ñ Number 1 ñ January-February 2009

Contents REVIEW ARTICLES 1 rhGH treatment of children with growth hormone deficiency: factors affecting final height K. Karavanaki-Karanassiou 10 Growth hormone treatment in Prader-Willi syndrome E. Kousta, C. Hadjiathanasiou†, A. Papathanasiou

e-mail: hps@ath.forthnet.gr

16 Newborn infections from group B Streptococcus C. Tsakalidis, A. Tragiannidis, N. Nikolaidis

Instructions to authors: http://www.e-child.gr/paediatriki/ iae.pdf

23 Polysaccharide antigens – conjugated vaccines: immune memory and anamnestic doses π.T. Kavaliotis

Manuscript submission

Manuscript Editing Greek Editing K. Papachristou English Editing S. Nakou Publisher K. Griveas Publishing Coordinator Communications In Practice S.A. 1∞ Pierias St. GR - 144 51, Metamorfossi Tel.: +30 210 87 78 884 Fax: +30 210 87 78 822 Owner Greek Paediatric Society© 92 Michalakopoulou St. GR - 115 28, Athens Tel.: +30 210 7771 140 +30 210 7771 663 Fax: +30 210 7758 354 e-mail: hps@ath.forthnet.gr Annual Subscription All foreign countries: US $ 50

ORIGINAL ARTICLES

CASE REPORTS 51 Type 1 pseudohypoaldosteronism in an infant C. Daskalopoulou, T. Tsekoura, A. Tsilimigaki 55 Idiopathic intracranial hypertension in young adolescents with obesity A. Filippidi, S. Stefanaki, M. Anatoliotaki, G. Vlachaki, C. Fanouriakis, A. Tsilimigaki 60 Congenital chylothorax and subcutaneous oedema in a neonate with superior vena cava syndrome V. Makri, A. Konstantinidi, M. Papadopoulou, O. Dedousi, S. Tantros, E. Agapitos, A. Gounaris CLINICAL QUIZ 66 E. Mantadakis, A. Tsalkidis, A. Ramatani, E. Paraskakis, A. Chatzimichael

29 Administration of two anti-TNF agents (etanercept and adalimumab) in children with juvenile idiopathic arthritis: a 5-year follow-up study M. Trachana, P. Pratsidou-Gertsi, F. Kanakoudi-Tsakalidou, C. Diafa, G. Pardalos, M. Bantouraki

CURRENT ISSUES OF BIOSTATISTICS

41 Obesity prevalence in children aged 8-13 years according to IOTF cutoffs, CDC growth charts and Z-BMI scores N. Mantzouranis, H. Douda, T. Pilianidis, S. Tokmakidis

NEWS FROM THE INTERNET

46 Use and abuse of substances by young adult students of the health professions S. Mantoudis, L. Thomaidis

67 Prospective cohort studies: the application of epidemiology in order to identify the potential determinants of paediatric diseases E. Critselis, D.A. Kafetzis

70 http://www.paidiatros.com A. Hadjipanayis

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REVIEW ARTICLE

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ÃÔÚ‹ÁËÛË ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ Û ·È‰È¿ Ì ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘: ·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ∫. ∫·Ú·‚·Ó¿ÎË-∫·Ú·Ó¿ÛÈÔ˘ ¶ÂÚ›ÏË„Ë: H ·ÚÔ‡Û· ‚È‚ÏÈÔÁÚ·ÊÈ΋ ·Ó·ÛÎfiËÛË ·Ó·Ê¤ÚÂÙ·È ÛÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ Ù˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ (GH) ÛÙ· ·È‰È¿, ÛÙËÓ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· Ì ·ÓıÚÒÈÓË ·Ó·Û˘Ó‰˘·Ṳ̂ÓË ·˘ÍËÙÈ΋ ÔÚÌfiÓË (rhGH), ηıÒ˜ Î·È ÛÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· (∆∞). √ ÂÙ‹ÛÈÔ˜ Ú˘ıÌfi˜ ·‡ÍËÛ˘ (∂ƒ∞) ηٿ ÙÔÓ 1Ô ¯ÚfiÓÔ ·ÁˆÁ‹˜ ·ÚÔ˘ÛÈ¿˙ÂÈ ·ÚÓËÙÈ΋ Û˘Û¯¤ÙÈÛË Ì ÙË ¯ÚÔÓÔÏÔÁÈ΋ Î·È ÔÛÙÈ΋ ËÏÈΛ· ηٿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜. ∞fi ÙȘ ·Ú·Ì¤ÙÚÔ˘˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙÔ ∆∞, ÛËÌ·ÓÙÈÎfiÙÂÚ˜ Â›Ó·È ÙÔ ‡„Ô˜ Î·È Ë ËÏÈΛ· ¤Ó·Ú͢ ·ÁˆÁ‹˜, ÙÔ ‡„Ô˜ ¤Ó·Ú͢ Ù˘ ‹‚˘ Î·È ÙÔ ·Ó¿ÛÙËÌ· ÙˆÓ ÁÔÓ¤ˆÓ. ∆Ô Û˘ÓËı¤ÛÙÂÚÔ ·›ÙÈÔ ÌË ÈηÓÔÔÈËÙÈ΋˜ ·ÓÙ·fiÎÚÈÛ˘ ÛÙË ıÂÚ·›· Ì rhGH Â›Ó·È Ë ÌË Û˘ÌÌfiÚʈÛË ÛÙËÓ ·ÁˆÁ‹. ™˘ÌÂÚ·ÛÌ·ÙÈο, Â›Ó·È ‰˘Ó·Ùfi Ó· ÂÈÙ¢¯ı› Ê˘ÛÈÔÏÔÁÈÎfi ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· Û ·È‰È¿ Ì ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ (GHD) Ì ÙË ¯ÔÚ‹ÁËÛË Û˘Ì‚·ÙÈÎÒÓ ‰fiÛÂˆÓ rhGH, Ì ÙËÓ ÂÓˆÚ›˜ ¤Ó·ÚÍË Ù˘ ·ÁˆÁ‹˜, ÙË ÛÙÂÓ‹ ·Ú·ÎÔÏÔ‡ıËÛË, ÙË Û˘ÌÌfiÚʈÛË ÛÙËÓ Î·ıËÌÂÚÈÓ‹ ¯ÔÚ‹ÁËÛË, ηıÒ˜ Î·È Ì Â›Ù¢ÍË ÈηÓÔÔÈËÙÈÎÔ‡ ‡„Ô˘˜ ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ÂÊ˂›·˜.

B’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: ∫˘Úȷ΋ ∫·Ú·‚·Ó¿ÎË∫·Ú·Ó¿ÛÈÔ˘ kkarav@yahoo.gr B’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡» °Ô˘‰‹ 11527, ∞ı‹Ó·

§¤ÍÂȘ ÎÏÂȉȿ: ∞ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘, ıÂÚ·›· Ì ·ÓıÚÒÈÓË ·Ó·Û˘Ó‰˘·Ṳ̂ÓË ·˘ÍËÙÈ΋ ÔÚÌfiÓË, Ú˘ıÌfi˜ ·‡ÍËÛ˘, ÙÂÏÈÎfi ·Ó¿ÛÙËÌ·.

rhGH treatment of children with growth hormone deficiency: factors affecting final height K. Karavanaki-Karanassiou Abstract: This is a review of the aetiology of growth hormone deficiency (GHD) in childhood, its response to treatment with recombinant human growth hormone (rhGH), and the factors that affect the final height (FH). ∆he growth velocity (GV) during the first year of treatment with rhGH is negatively correlated with chronological and bone age at treatment initiation. Among the factors affecting FH the most important are the genetically determined height potential of the patient, early age at diagnosis, height at initiation of treatment, correction of height deficit prior to the onset of puberty, daily administration of rhGH treatment, higher doses of rhGH and longer duration of treatment. Lack of compliance with the treatment is the most common cause of inadequate response to rhGH therapy in children with GHD. In conclusion, normal FH in children with GHD can be achieved with conventional doses of rhGH when treatment is initiated early, compliance is assured by close follow-up and a satisfactory height is attained prior to the onset of puberty.

2nd Department of Paediatrics, University of Athens, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece Correspondence: Kyriaki KaravanakiKaranassiou kkarav@yahoo.gr 2nd Department of Paediatrics, University of Athens, “P. & A. Kyriakou” Children’s Hospital Goudi 11527, Athens, Greece

Key words: Growth hormone deficiency, human recombinant growth hormone therapy, growth velocity, final height.

AÈÙÈÔÏÔÁ›·-‰È¿ÁÓˆÛË ·ÓÂ¿ÚÎÂÈ·˜ GH ∏ ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ (growth hormone deficiency, GHD), ÂÊfiÛÔÓ ‰ÂÓ ‰È·ÁÓˆÛı› Î·È ‰ÂÓ ·ÓÙÈÌÂÙˆÈÛı› Ì ıÂÚ·›· ˘ÔηٿÛÙ·Û˘ Ì ·ÓıÚÒÈÓË ·Ó·Û˘Ó‰˘·Ṳ̂ÓË ·˘ÍËÙÈ΋ ÔÚÌfiÓË (recombinant human growth hormone, rhGH), Ô‰ËÁ› Û ÛËÌ·ÓÙÈο ¯·ÌËÏfi ·Ó¿ÛÙËÌ·. ∏ ·ÓÂ¿ÚÎÂÈ· GH ‰È·ÎÚ›ÓÂÙ·È Û ȉÈÔ·ı‹ Î·È ÔÚÁ·ÓÈ΋ (1). I‰ÈÔ·ı‹˜ ·ÓÂ¿ÚÎÂÈ· GH (idiopathic growth hormone deficiency, IGHD) ‰È·ÁÈÁÓÒÛÎÂÙ·È fiÙ·Ó Î·Ù¿ ÙÔÓ ¤ÏÂÁ¯Ô ‰ÂÓ ‰È·ÈÛÙÒÓÔÓÙ·È ·Ó·ÙÔÌÈΤ˜ ‹ ÁÂÓÂÙÈΤ˜

·ÓˆÌ·Ï›Â˜, Û ·ÓÙȉȷÛÙÔÏ‹ Ì ÙËÓ ÔÚÁ·ÓÈ΋ ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ (organic growth hormone deficiency, OGHD), ηٿ ÙËÓ ÔÔ›· ‰È·ÈÛÙÒÓÔÓÙ·È ÔÈΛϷ ·›ÙÈ· (¶›Ó·Î·˜ 1). ™Â ·È‰È¿ Ì IGHD ÌÔÚ› Ó· ·ÔÎ·Ï˘ÊıÔ‡Ó ÚÔ‚Ï‹Ì·Ù· ηٿ ÙÔÓ ÙÔÎÂÙfi (ÂÚÈÁÂÓÓËÙÈ΋ ·ÛÊ˘Í›·, Èۯȷ΋ ÚÔ‚ÔÏ‹), ηıÒ˜ Â›Û˘ Î·È Î·Ù¿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË (Â·ÂÈÏÔ‡ÌÂÓË Î‡ËÛË, ·ÈÌÔÚÚ·Á›Â˜, ۷ί·Ú҉˘ ‰È·‚‹Ù˘ ·ËÛ˘, Ïԛ̈ÍË) (2,3). ∂Âȉ‹ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ Ù˘ ˘Ôı·Ï·ÌÔ-˘ÔÊ˘Ûȷ΋˜ ÂÚÈÔ¯‹˜ ‰È·ÈÛÙÒÓÔÓÙ·È Î·È ¯ˆÚ›˜ ÂÚÈÁÂÓÓËÙÈ΋ ¶·È‰È·ÙÚÈ΋ 2009;72:1-9

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∫˘Úȷ΋ ∫·Ú·‚·Ó¿ÎË-∫·Ú·Ó¿ÛÈÔ˘

¶›Ó·Î·˜ 1. ∞›ÙÈ· ·ÓÂ¿ÚÎÂÈ·˜ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ 1. π‰ÈÔ·ı‹˜ ·ÓÂ¿ÚÎÂÈ· GH ·. ªÂ ‹ ¯ˆÚ›˜ ÈÛÙÔÚÈÎfi ÂÚÈÁÂÓÓËÙÈ΋˜ ˘ÔÍ›·˜ ‚. ÕÁÓˆÛÙÔ ÂÚÈÁÂÓÓËÙÈÎfi ÈÛÙÔÚÈÎfi Á. ¡Â˘ÚÔÂÎÎÚÈÙÈ΋ ‰È·Ù·Ú·¯‹ GH 2. OÚÁ·ÓÈ΋ ·ÓÂ¿ÚÎÂÈ· GH ·. ™˘ÁÁÂÓ‹˜ ñ °ÔÓȉȷ΋ ‚Ï¿‚Ë (GH1, GHRHR, POU1F1, PROP1, LHX4, SOX3) ñ ™‡Ó‰ÚÔÌ· (Fanconi, ÂÎÙÔ‰ÂÚÌÈ΋ ‰˘ÛÏ·Û›·) ñ ™˘ÁÁÂÓ›˜ ÏÔÈÌÒÍÂȘ (Û˘ÁÁÂÓ‹˜ ÂÚ˘ıÚ¿ Î.¿.) ñ ∞ӈ̷ϛ˜ Ù˘ ̤Û˘ ÁÚ·ÌÌ‹˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ - ™‡Ó‰ÚÔÌÔ ÎÂÓÔ‡ ÙÔ˘ÚÎÈÎÔ‡ ÂÊÈ›Ԣ - ∞ÁÂÓÂÛ›· ÌÂÛÔÏÔ‚›Ô˘ - ¢˘ÛÏ·Û›· ÙÔ˘ ‰È·Ê·ÓÔ‡˜ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ÙÔ˘ ÌÂÛÔÏÔ‚›Ô˘ Î·È Ù˘ ÔÙÈ΋˜ Û˘Û΢‹˜ (Septooptic dysplasia) - OÏÔÚÔÛÂÁÎÂÊ·Ï›· - ¢È·ÙÔÌ‹ ÙÔ˘ Ì›Û¯Ô˘ Ù˘ ˘fiÊ˘Û˘ - ™‡Ó‰ÚÔÌÔ ÌÔÓ‹ÚÔ˘˜ ÙÔ̤· ¿Óˆ ÁÓ¿ıÔ˘ - ÀÂÚˆÈÔÛ¯ÈÛÙ›· - ™˘ÁÁÂÓ‹˜ ˘‰ÚÔΤʷÏÔ˜ - ∞Ú·¯ÓÔÂȉ›˜ ·ÛÙÂȘ ‚. ∂›ÎÙËÙË ÔÚÁ·ÓÈ΋ ·ÓÂ¿ÚÎÂÈ· GH ñ ŸÁÎÔÈ ˘Ôı·Ï·ÌÔ-˘ÔÊ˘Ûȷ΋˜ ÂÚÈÔ¯‹˜ (ÎÚ·ÓÈÔÊ·Ú˘ÁÁ›ˆÌ·, ‰˘ÛÁÂỨӈ̷, ·Ì¿Úو̷ Î.¿.) ñ ∂Ó‰ÔÎÚ¿ÓÈÔÈ fiÁÎÔÈ ÂÎÙfi˜ ÙÔ˘ÚÎÈÎÔ‡ ÂÊÈ›Ԣ (·ÛÙÚÔ·Ùو̷, ÂÂÓ‰‡ÌˆÌ·, ÁÏÔ›ˆÌ·, Ì˘ÂÏÔ‚Ï¿Ûو̷, fiÁÎÔÈ ÚÈÓÔÊ¿Ú˘ÁÁ· Î.¿.) ñ ∞ÎÙÈÓÔ‚ÔÏ›· Â͈ÎÚ·ÓÈ·ÎÒÓ Î·ÎÔËıÂÈÒÓ (Ï¢¯·ÈÌ›·, ϤÌʈ̷, Û˘Ì·Á›˜ fiÁÎÔÈ) ñ ÕÏÏ· ·›ÙÈ· (ÙÚ·‡Ì· ÎÚ·Ó›Ô˘, Ïԛ̈ÍË ∫¡™, Â›ÎÙËÙÔ˜ ˘‰ÚÔΤʷÏÔ˜, ÈÛÙÈÔ·ÙÙˆÛË, ¯ÚfiÓÈ· ÎÔÎÎȈ̷Ù҉˘ ÓfiÛÔ˜, ·ÁÁÂȷΤ˜ ·ÓˆÌ·Ï›Â˜)

οΈÛË, Ù›ıÂÙ·È Ë ˘fiÓÔÈ· Ì‹ˆ˜ ÔÈ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ Ù˘ ·Ó¿Ù˘Í˘ Ù˘ ˘Ôı·Ï·ÌÔ-˘ÔÊ˘Ûȷ΋˜ ÂÚÈÔ¯‹˜ ‰ÂÓ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ·ÏÏ¿ ·›ÙÈÔ Ù˘ ÂÚÈÁÂÓÓËÙÈ΋˜ οΈÛ˘ (4). ∆· ·È‰È¿ Ì ÔÚÁ·ÓÈ΋ ·ÓÂ¿ÚÎÂÈ· GH ‰È·ÎÚ›ÓÔÓÙ·È Û ÂΛӷ ÌÂ Û˘ÁÁÂÓ‹ ÔÚÁ·ÓÈ΋ ‚Ï¿‚Ë (ÁÔÓȉȷ΋ ‚Ï¿‚Ë, Û‡Ó‰ÚÔÌ·, ·ÓˆÌ·Ï›Â˜ Ù˘ ̤Û˘ ÁÚ·ÌÌ‹˜ Î.Ï.) (3) Î·È Û Â›ÎÙËÙË ÔÚÁ·ÓÈ΋ ‚Ï¿‚Ë (fiÁÎÔÈ, ·ÎÙÈÓÔ‚ÔÏ›· Î.Ï.) (¶›Ó·Î·˜ 1).

H ‰È¿ÁÓˆÛË Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ GH Ù›ıÂÙ·È fiÙ·Ó Û ‰‡Ô ‰È·‰Ô¯ÈΤ˜ ‰˘Ó·ÌÈΤ˜ ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘ GH ÔÈ ÙÈ̤˜ Ù˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ Â›Ó·È ¯·ÌËϤ˜. ∆Ô fiÚÈÔ ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ¤¯ÂÈ ÔÚÈÛı› Ì¿ÏÏÔÓ ·˘ı·›ÚÂÙ· ˆ˜ ̤ÁÈÛÙË ÙÈÌ‹ GH Ï¿ÛÌ·ÙÔ˜ >10 ng/ml ‹ >20 mU/L ηٿ ÙȘ ‰˘Ó·ÌÈΤ˜ ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘ (5).

¶·Ú¿ÌÂÙÚÔÈ ÛˆÌ·ÙÈ΋˜ ·‡ÍËÛ˘ ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ rhGH √È ÌÂÙÚ‹ÛÂȘ ÙÔ˘ ‡„Ô˘˜ Î·È ÙÔ˘ ÂÙ‹ÛÈÔ˘ Ú˘ıÌÔ‡ ·‡ÍËÛ˘ (∂ƒ∞) ÙÔ˘ οı ·È‰ÈÔ‡ ÂÎÊÚ¿˙ÔÓÙ·È Û ‰Â›ÎÙ˜ ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘ (Standard Deviation Score, SDS), Ì ‚¿ÛË ÙË ¯ÚÔÓÔÏÔÁÈ΋ ËÏÈΛ· Î·È ÙÔ Ê‡ÏÔ. √ ‰Â›ÎÙ˘ ·˘Ùfi˜ ‰Â›¯ÓÂÈ fiÛ˜ ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ ·fi ÙÔÓ Ì¤ÛÔ fiÚÔ ‚Ú›ÛÎÂÙ·È ÙÔ ‡„Ô˜ ÙÔ˘ ·È‰ÈÔ‡ Î·È ˘ÔÏÔÁ›˙ÂÙ·È ·fi ÙÔÓ Ù‡Ô SDS = (̤ÛË ÙÈÌ‹ ‡„Ô˘˜-X)/SD [fiÔ˘ à = ‡„Ô˜, ̤ÛË ÙÈÌ‹ Paediatriki 2009;72:1-9

‡„Ô˘˜ = Ë Ì¤ÛË ÙÈÌ‹ ÙÔ˘ ‡„Ô˘˜ Ô˘ ·ÓÙÈÛÙÔȯ› ÛÙËÓ ËÏÈΛ· Î·È ÛÙÔ Ê‡ÏÔ ÙÔ˘ ·È‰ÈÔ‡ (·fi ›Ó·Î˜) Î·È SD = Ë ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË Ù˘ ›‰È·˜ ËÏÈΛ·˜]. ∏ ·ÓÂ¿ÚÎÂÈ· Ù˘ GH (ȉÈÔ·ı‹˜ GHD) ÂΉËÏÒÓÂÙ·È Û˘Ó‹ıˆ˜ Ì ˘ÔÁÏ˘Î·ÈÌ›· ÛÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ ˙ˆ‹˜ Î·È Ì ÂÏ¿ÙÙˆÛË ÙÔ˘ Ú˘ıÌÔ‡ ·Ó¿Ù˘Í˘ (Eƒ∞<3 SDS) ÌÂÙ¿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ. ∏ ¤ÏÏÂÈ„Ë GH ÛÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ ˙ˆ‹˜ ‰ÂÓ ÂËÚ¿˙ÂÈ ÙÔ Ì‹ÎÔ˜/‡„Ô˜ ÙÔ˘ ·È‰ÈÔ‡, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ÛÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ˙ˆ‹˜ Ë ·‡ÍËÛË Â›Ó·È ¿ÌÂÛ· ÂÍ·ÚÙË̤ÓË ·fi ÙË ‰È·ÙÚÔÊ‹. ∞ÓÙ›ıÂÙ·, ÛÙËÓ ÔÚÁ·ÓÈ΋ GHD, ÏfiÁˆ fiÁÎˆÓ ÂÁÎÂÊ¿ÏÔ˘ ‹ ·ÎÙÈÓÔ‚ÔÏ›·˜ ÎÚ·Ó›Ô˘ ˆ˜ Â› Ï¢¯·ÈÌ›·˜, fiÔ˘ ÚÔηÏÂ›Ù·È Â›ÎÙËÙË ‚Ï¿‚Ë ÛÙÔÓ ˘Ôı¿Ï·ÌÔ Î·È ÛÙËÓ ÚfiÛıÈ· ˘fiÊ˘ÛË, Ì ·ÔÙ¤ÏÂÛÌ· ÙË ‰È·Ù·Ú·¯‹ ÛÙËÓ ¤ÎÎÚÈÛË GH, Ë ˘fiÓÔÈ· Ù›ıÂÙ·È ÏfiÁˆ Ì›ˆÛ˘ ÙÔ˘ ∂ƒ∞ Û ·È‰› Ì ÚÔËÁÔ‡ÌÂÓË Ê˘ÛÈÔÏÔÁÈ΋ ·‡ÍËÛË (6). ∏ ˘fiÓÔÈ· ·˘Ù‹ ÂÓÈÛ¯‡ÂÙ·È fiÙ·Ó ‰ÂÓ ˘¿Ú¯ÂÈ ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi (ÔÈ ÁÔÓ›˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜) Î·È ÔÈ ‰È·ÛÙ¿ÛÂȘ ÙˆÓ ·È‰ÈÒÓ Î·Ù¿ ÙË Á¤ÓÓËÛË Î·ıÒ˜ Î·È ÔÈ Û˘Óı‹Î˜ ÙÔ˘ ÙÔÎÂÙÔ‡ Â›Ó·È Ê˘ÛÈÔÏÔÁÈΤ˜ (7). ™ÙÔÓ ¶›Ó·Î· 2 ·Ó·Ê¤ÚÔÓÙ·È Â˘Ú‹Ì·Ù· ‰È·ÊfiÚˆÓ ÌÂÏÂÙÒÓ (8-12) Ô˘ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë Ì¤ÛË ¯ÚÔÓÔÏÔÁÈ΋ ËÏÈΛ· ηٿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜ Ì rhGH Û ·ÛıÂÓ›˜ Ì IGHD Î˘Ì·›ÓÂÙ·È ·fi 1,9 ¤ˆ˜ 10,1 ¤ÙË Î·È Ë Ì¤ÛË ÙÈÌ‹ Ù˘ SDS ÙÔ˘ ‡„Ô˘˜ ·fi -4,5 ¤ˆ˜ -2,5.

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·3

3

¶·È‰È¿ Ì ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘

¶›Ó·Î·˜ 2. ¢Â‰Ô̤ӷ ·ÛıÂÓÒÓ Ì ·ÓÂ¿ÚÎÂÈ· GH ηٿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜ Ì rhGH ªÂϤÙË Ranke (1991) De Muink (1992) Boersma (1995) Karavanaki Î·È Dacou (2001) Ranke (2001)

n 289 21 26 100 265

XH (¤ÙË) SDS ‡„Ô˘˜ (̤ÛË ÙÈÌ‹) (̤ÛË ÙÈÌ‹) 7,0 6,8 1,4 10,1 1,9 7,5

-2,8 -3,6 -4,1 -2,9 -3,1 -2,5

XH = ÃÚÔÓÔÏÔÁÈ΋ ËÏÈΛ· ηٿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜ Ì rhGH SDS ‡„Ô˘˜ = ™Ù·ıÂÚ‹ ·fiÎÏÈÛË ‡„Ô˘˜ ηٿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜

™Â ÛËÌ·ÓÙÈÎfi ·ÚÈıÌfi ·È‰ÈÒÓ, Ë IGHD ‰È·ÁÈÁÓÒÛÎÂÙ·È Û ËÏÈΛ· ÌÂÁ·Ï‡ÙÂÚË ÙˆÓ 6,8 ÂÙÒÓ, ÂÓÒ Û ÌÈÎÚfi ·ÚÈıÌfi ·È‰ÈÒÓ Ë ‰È¿ÁÓˆÛË Ù›ıÂÙ·È ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·. ™Â ·È‰È¿ Ì ÔÚÁ·ÓÈ΋ ·ÓÂ¿ÚÎÂÈ· GH ÏfiÁˆ fiÁÎˆÓ ÂÁÎÂÊ¿ÏÔ˘ Î·È Ù˘ Û˘ÓÔ‰Ô‡ Ï‹„˘ ·ÎÙÈÓÔ‚ÔÏ›·˜, ·Ú·ÙËÚÂ›Ù·È ·Ú¯Èο ÛËÌ·ÓÙÈ΋ Ì›ˆÛË ÙÔ˘ ∂ƒ∞ Î·È ÚÔԉ¢ÙÈο, 2-3 ¯ÚfiÓÈ· ÌÂÙ¿ ÙË ıÂÚ·›· ÙÔ˘ fiÁÎÔ˘, Ì›ˆÛË Ù˘ SDS ‡„Ô˘˜ (¤ˆ˜ -2,5 SDS). ∏ ‰È·›ÛÙˆÛË Ù˘ Ì›ˆÛ˘ ÙÔ˘ ∂ƒ∞ ÛÙ· ·È‰È¿ ·˘Ù¿ ·ÔÙÂÏ› ¤Ó‰ÂÈÍË ÁÈ· ÔÚÌÔÓÈ΋ ‰ÈÂÚ‡ÓËÛË Î·È ·ÓÙÈÌÂÙÒÈÛË (13).

¢ÔÛÔÏÔÁ›· rhGH ¶·Ï·ÈfiÙÂÚ· Ë GH ¯ÔÚËÁÔ‡ÓÙ·Ó 2-3 ÊÔÚ¤˜/ ‚‰ÔÌ¿‰· Î·È Û ÛÙ·ıÂÚ‹ ‰ÔÛÔÏÔÁ›· Û IU (12 IU/‚‰ÔÌ¿‰· Û 3 ‰fiÛÂȘ). ŒÙÛÈ, Ù· ·¯‡Û·Úη ·È‰È¿ ¤·ÈÚÓ·Ó ÌÈÎÚfiÙÂÚË ‰fiÛË Î·È Ù· ÏÂÙfiۈ̷ ÌÂÁ·Ï‡ÙÂÚË ‰fiÛË. ∫·Ù¿ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ë ‰fiÛË ˘ÔÏÔÁ›˙ÂÙ·È ·Ó¿ kg ‹ ·Ó¿ m2 ÂÈÊ·Ó›·˜ ÛÒÌ·ÙÔ˜ (14). M ÙËÓ ÂÈÛ·ÁˆÁ‹ Ù˘ ·ÓıÚÒÈÓ˘ ·Ó·Û˘Ó‰˘·Ṳ̂Ó˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ (rhGH) (3IU/mg), Ë ‰ÔÛÔÏÔÁ›· ÙÚÔÔÔÈ‹ıËΠ۠IU/kg/‚‰ÔÌ¿‰· ‹ Û mg/kg/‚‰ÔÌ¿‰· Î·È ¯ÔÚËÁÂ›Ù·È Î·ıËÌÂÚÈÓ¿. ∏ Û˘ÓÈÛÙÒÌÂÓË ·Ú¯È΋ ‰ÔÛÔÏÔÁ›· Ù˘ rhGH ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì IGHD Â›Ó·È 0,18-0,30 mg/kg/‚‰ÔÌ¿‰· (ÌÂ Û˘ÓËı¤ÛÙÂÚË ÙË ‰ÔÛÔÏÔÁ›· 0,25 mg/kg/‚‰ÔÌ¿‰·) ¯ÔÚËÁÔ‡ÌÂÓË Û 7 ËÌÂÚ‹ÛȘ ‰fiÛÂȘ. Œ¯ÂÈ ·Ô‰Âȯı› fiÙÈ Ë ·‡ÍËÛË Â›Ó·È ·Ó¿ÏÔÁË ÙÔ˘ ÏÔÁ·Ú›ıÌÔ˘ Ù˘ ‰fiÛ˘, ·ÏÏ¿ Ë Î·ıËÌÂÚÈÓ‹ ¯ÔÚ‹ÁËÛË Â›Ó·È ÈÔ ÛËÌ·ÓÙÈ΋ Û˘ÁÎÚÈÙÈο Ì ÙÔ ·Ï·ÈfiÙÂÚÔ ıÂÚ·¢ÙÈÎfi Û¯‹Ì· ¯ÔÚ‹ÁËÛ˘ Ù˘ rhGH 3 ÊÔÚ¤˜/‚‰ÔÌ¿‰·. ŒÙÛÈ, ÌÈ· ÌÂϤÙË (15) ·¤‰ÂÈÍ fiÙÈ ÔÌ¿‰· ·ÛıÂÓÒÓ ˘fi ·ÁˆÁ‹ Ì rhGH Û ‰fiÛË 0,30 mg/kg/‚‰ÔÌ¿‰· Î·È Î·ıËÌÂÚÈÓ‹ ¯ÔÚ‹ÁËÛË Î¤Ú‰ÈÛ 9,7 cm ÂÚÈÛÛfiÙÂÚÔ ‡„Ô˜ Û ‰È¿ÛÙËÌ· 4 ÂÙÒÓ, Û˘ÁÎÚÈÙÈο Ì ÂΛÓÔ˘˜ Ô˘ ÂÏ¿Ì‚·Ó·Ó ÙËÓ ·ÁˆÁ‹ 3 ÊÔÚ¤˜/‚‰ÔÌ¿‰· (38,4 ¤Ó·ÓÙÈ 28,7 cm, p<0,0002), Ì ·ÚfiÌÔÈ·

ÂͤÏÈÍË Ù˘ ÔÛÙÈ΋˜ ËÏÈΛ·˜ Î·È ¯ˆÚ›˜ ÂÈÙ¿¯˘ÓÛË Ù˘ ‹‚˘.

∞ÓÙ·fiÎÚÈÛË ÛÙËÓ ·ÁˆÁ‹ Ì rhGH ∞) ™ÙËÓ ÚÔÂÊË‚È΋ ÂÚ›Ô‰Ô ∏ Ù˘È΋ ·¿ÓÙËÛË ÛÙË ıÂÚ·›· ·È‰ÈÔ‡ Ì IGHD ÛÙË Û˘ÓÈÛÙÒÌÂÓË ‰ÔÛÔÏÔÁ›· Î·È Û ηıËÌÂÚÈÓ‹ ¯ÔÚ‹ÁËÛË Â›Ó·È Ë ·‡ÍËÛË ÙÔ˘ ∂ƒ∞ ·fi 3-4 cm/¯ÚfiÓÔ Û 8,6-13,3 cm/¯ÚfiÓÔ ÙÔÓ 1Ô ¯ÚfiÓÔ ·ÁˆÁ‹˜ (‰ËÏ·‰‹ ÂÈÙ¿¯˘ÓÛË ÙÔ˘ Ú˘ıÌÔ‡ ·‡ÍËÛ˘ ‰ÈÏ¿ÛÈ· ˆ˜ ÙÂÙÚ·Ï¿ÛÈ· ÂΛӢ ÚÔ ·ÁˆÁ‹˜) (16) Î·È Û 7-9 cm/¯ÚfiÓÔ ·ÓÙ›ÛÙÔȯ· ÙÔÓ 2Ô Î·È 3Ô ¯ÚfiÓÔ ·ÁˆÁ‹˜ (17). ªÂ ÙËÓ ¿ÚÔ‰Ô ÙˆÓ ÂÙÒÓ, ·Ú·ÙËÚÂ›Ù·È ÛÙ·‰È·Î‹ Ì›ˆÛË Ùo˘ ∂ƒ∞, Ô˘ ÂÍÈÛÒÓÂÙ·È Ì ÙÔÓ Ê˘ÛÈÔÏÔÁÈÎfi ∂ƒ∞ ÁÈ· ÙËÓ ËÏÈΛ· Î·È ÙÔ Ê‡ÏÔ (17). ¶·ÚfiÌÔÈ· ·ÔÙÂϤÛÌ·Ù· ‰È·ÈÛÙÒıËÎ·Ó Û ̛· ÌÂϤÙË ·fi ÙËÓ ∞ã ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ (11), fiÔ˘ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ‰Â‰Ô̤ӷ ·fi ÙË ¯ÔÚ‹ÁËÛË GH Û 156 ·È‰È¿ Ì GHD (62,2% Ì IGHD Î·È 35,2% Ì OGHD) ηٿ ÙËÓ ÂÚ›Ô‰Ô 1970-1997. ™Ù· ·È‰È¿ ·˘Ù¿, Ô ∂ƒ∞ ÚÔ ·ÁˆÁ‹˜ ‹Ù·Ó 3,8±1,3 cm/¯ÚfiÓÔ ÁÈ· ÙËÓ ÔÌ¿‰· Ì IGHD Î·È 4,0±3,0 cm/¯ÚfiÓÔ ÁÈ· ÂΛӷ Ì OGHD. ∫·Ù¿ ÙÔÓ 1Ô ¯ÚfiÓÔ ·ÁˆÁ‹˜, Ô ∂ƒ∞ ‹Ù·Ó 8,8±3,5 Î·È 8,2±4,0 cm/¯ÚfiÓÔ (p=NS) ÁÈ· ÙȘ ÔÌ¿‰Â˜ Ì ÌÂÌÔӈ̤ÓË IGHD Î·È ÔÏÏ·Ï‹ ˘ÔÊ˘Ûȷ΋ ·ÓÂ¿ÚÎÂÈ·, ·ÓÙ›ÛÙÔȯ· (11). ∂›Û˘, Ô ∂ƒ∞ ·ÚÔ˘Û›·˙ ·ÚÓËÙÈ΋ Û˘Û¯¤ÙÈÛË Ì ÙË ¯ÚÔÓÔÏÔÁÈ΋ (r=-0,53, p=0,001) Î·È ÔÛÙÈ΋ ËÏÈΛ· ηٿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜ (r=-0,51, p=0,001) Î·È ıÂÙÈ΋ Û˘Û¯¤ÙÈÛË Ì ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· (∆∞) (r=0,49, p=0,01) ÌfiÓÔ ÁÈ· ÙËÓ ÔÌ¿‰· Ì ÙËÓ ÔÏÏ·Ï‹ ˘ÔÊ˘Ûȷ΋ ·ÓÂ¿ÚÎÂÈ·. ™˘ÓÂÒ˜, ÛËÌ·ÓÙÈ΋ ·Ú¿ÌÂÙÚÔ˜ ÁÈ· ÙËÓ ·Ú¯È΋ ÂÎÙ›ÌËÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙËÓ ·ÁˆÁ‹ Ì rhGH Â›Ó·È Ë ·‡ÍËÛË ÙÔ˘ ∂ƒ∞ ηٿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ·ÁˆÁ‹˜, Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ Ê·ÈÓfiÌÂÓÔ “catch up” (·Ó·Ù˘ÍÈ·Îfi ¿ÏÌ·). √ ∂ƒ∞ ηٿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ·ÁˆÁ‹˜ ‰ÂÓ ·ÔÙÂÏ› ÚÔÁÓˆÛÙÈÎfi ‰Â›ÎÙË ÙÔ˘ ∆∞, ·ÏÏ¿ Ì›· ηϋ ·Ú¯È΋ ·¿ÓÙËÛË ÛÙËÓ ·ÁˆÁ‹ Ì rhGH Ô˘ ˘Ô‰ËÏÒÓÂÈ ÈηÓÔÔÈËÙÈÎfi ÙÂÏÈÎfi ·ÔÙ¤ÏÂÛÌ· Ù˘ ·ÁˆÁ‹˜ (18).

H ·¿ÓÙËÛË ÛÙËÓ Â͈ÁÂÓ‹ ¯ÔÚ‹ÁËÛË rhGH ÔÈΛÏÏÂÈ ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· (ÌÂÁ·Ï‡ÙÂÚË ·‡ÍËÛË Û ÌÈÎÚfiÙÂÚ· ·È‰È¿), ÙÔ ‚¿ÚÔ˜ ÛÒÌ·ÙÔ˜, ÙËÓ ÔÛfiÙËÙ· Î·È ÙÔ Â›‰Ô˜ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ Ù˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘, fiˆ˜ ˘ÔÏÔÁ›˙ÂÙ·È ·fi Ù· Â›‰· ÙˆÓ ‰ÂÛÌ¢ÙÈÎÒÓ ÚˆÙÂ˚ÓÒÓ Ù˘ GH (GH binding proteins, GHBPs), ηıÒ˜ Â›Û˘ Î·È ·fi ÙËÓ ÂÔ¯‹ ÙÔ˘ ¤ÙÔ˘˜ (·˘ÍË̤ÓÔ˜ ÌÂÙ·‚ÔÏÈÛÌfi˜ ÙˆÓ ÔÛÙÒÓ Î·Ù¿ ÙËÓ ¿ÓÔÈÍË Î·È ÙÔ Î·ÏÔη›ÚÈ) (19,20). ªÈ· ¿ÏÏË ·Ú¿ÌÂÙÚÔ˜ Ô˘ ÂËÚ¿˙ÂÈ ÙËÓ ·ÓÙ·fiÎÚÈÛË ÛÙËÓ ·ÁˆÁ‹ Ì rhGH Û ·È‰È¿ Ì IGHD Â›Ó·È Ë ‚·Ú‡ÙËÙ· Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ Ù˘ GH (Ï‹Ú˘ ‹ ÌÂÚÈ΋ ·ÓÂ¿ÚÎÂÈ·). ¶ÚÈÓ ·fi ÙËÓ ·ÁˆÁ‹, Ô ∂ƒ∞ ‰ÂÓ ‰È·Ê¤ÚÂÈ ¶·È‰È·ÙÚÈ΋ 2009;72:1-9

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·4

4

∫˘Úȷ΋ ∫·Ú·‚·Ó¿ÎË-∫·Ú·Ó¿ÛÈÔ˘

ÛËÌ·ÓÙÈο ÛÙȘ ÔÌ¿‰Â˜ Ì ÛÔ‚·Ú‹ ‹ ÌÂÚÈ΋ ·ÓÂ¿ÚÎÂÈ· GH Î·È Â›Ó·È ÁÂÓÈο ¯·ÌËÏfi˜ (<50Ë ∂£ ÁÈ· ÙËÓ ËÏÈΛ·). ŸÌˆ˜, ÚÔÂÊË‚Èο, Ë ·‡ÍËÛË Î·Ù¿ Ù· 3 ÚÒÙ· ¯ÚfiÓÈ· ·ÁˆÁ‹˜ Ì rhGH Â›Ó·È ·Ó¿ÏÔÁË Ù˘ ‚·Ú‡ÙËÙ·˜ Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ GH. ∫·Ù¿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ·ÁˆÁ‹˜, Ù· ·È‰È¿ Ì ÛÔ‚·Ú‹ ·ÓÂ¿ÚÎÂÈ· GH (<5 ng/ml) ¤¯Ô˘Ó ÛËÌ·ÓÙÈο ηχÙÂÚË ·ÓÙ·fiÎÚÈÛË ÛÙËÓ ·ÁˆÁ‹ (cm/¯ÚfiÓÔ), Û˘ÁÎÚÈÙÈο Ì ÙȘ ¿ÏϘ ÔÌ¿‰Â˜ (14). ∫·Ù¿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ·ÁˆÁ‹˜, Â¿Ó Ô ∂ƒ∞ Â›Ó·È >97 ∂£ ∂ƒ∞ ÁÈ· ÙËÓ ËÏÈΛ· Î·È ÙÔ Ê‡ÏÔ (‹ ¢SDS ‡„Ô˘˜ >0,5), Ë ·ÓÙ·fiÎÚÈÛË ıˆÚÂ›Ù·È ÈηÓÔÔÈËÙÈ΋ (14). ∏ ÔÚ›· ÙˆÓ SDS ‡„Ô˘˜ ηٿ Ù· 5 ÚÒÙ· ¯ÚfiÓÈ· ·ÁˆÁ‹˜ Ì rhGH ·ÚÔ˘ÛÈ¿˙ÂÈ ÛÙ·‰È·Î‹ ‚ÂÏÙ›ˆÛË. ŒÙÛÈ, ·fi Ì›· ÌÂϤÙË Ù˘ ∞ã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ (∞ã ¶¶∫) (11) ‰È·ÈÛÙÒÓÂÙ·È ÌÈ· ÚÔԉ¢ÙÈ΋ ‚ÂÏÙ›ˆÛË ÙˆÓ SDS ‡„Ô˘˜ Ì ÙËÓ ¿ÚÔ‰Ô ÙˆÓ ÂÙÒÓ, ·fi -2,9 SDS Úo ·ÁˆÁ‹˜ Û -2,4, -2,17 Î·È -1,9 ηٿ Ù· ÚÒÙ· 3 ¯ÚfiÓÈ· ·ÁˆÁ‹˜, ·ÓÙ›ÛÙÔȯ·. ∏ SDS ÙÔ˘ ÙÂÏÈÎÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜ ‹Ù·Ó -1,38 Î·È ‰ÂÓ ‰È¤ÊÂÚ ÛËÌ·ÓÙÈο ·fi ÙËÓ SDS ÙÔ˘ ‡„Ô˘˜-ÛÙfi¯Ô˘. ∏ ÛËÌ·Û›· Ù˘ ¤ÁηÈÚ˘ ¤Ó·Ú͢ ·ÁˆÁ‹˜ ·fi ÌÈÎÚ‹ ËÏÈΛ· Ê·›ÓÂÙ·È ÛÙË ÌÂϤÙË ÙˆÓ Gluckman et al.

(6), fiÔ˘ Û˘ÁÎÚ›ÓÂÙ·È Ë ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· Ì rhGH Û ‰‡Ô ÔÌ¿‰Â˜ ·È‰ÈÒÓ: ·. ÔÌ¿‰· ‚ÚÂÊÒÓ Ì ¤Ó·ÚÍË ·ÁˆÁ‹˜ Û ËÏÈΛ· 1,2±0,6 ¯ÚfiÓˆÓ (n=45), ‚. ÔÌ¿‰· ·È‰ÈÒÓ Ì ¤Ó·ÚÍË ·ÁˆÁ‹˜ Û ËÏÈΛ· 7,5±0,3 ¯ÚfiÓˆÓ (n=42) (6). ∏ ·ÓÙ·fiÎÚÈÛË ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ·ÁˆÁ‹˜ ‹Ù·Ó ÛËÌ·ÓÙÈÎfiÙÂÚË ÛÙËÓ ÔÌ¿‰· ÙˆÓ ‚ÚÂÊÒÓ Ì ·‡ÍËÛË Ù˘ SDS ‡„Ô˘˜ ηٿ 1,6 SDS, Û˘ÁÎÚÈÙÈο Ì ÌfiÓÔ 0,5 SDS ÁÈ· ÙËÓ ÔÌ¿‰· ÙˆÓ ·È‰ÈÒÓ. ∞˘Ùfi ÌÔÚ› Ó· ·Ô‰Ôı› ÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ù· ‚Ú¤ÊË ‹Ù·Ó ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚÔ˘ ·Ó·ÛÙ‹Ì·ÙÔ˜ ηٿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜ (-3,7 SDS), Û˘ÁÎÚÈÙÈο Ì ٷ ·È‰È¿ (-2,8 SDS). ∂›Û˘, Ë ÔÌ¿‰· ÙˆÓ ‚ÚÂÊÒÓ Â›¯Â ÛÔ‚·ÚfiÙÂÚË ·ÓÂ¿ÚÎÂÈ· GH Û˘ÁÎÚÈÙÈο Ì ٷ ·È‰È¿. ∏ ·¿ÓÙËÛË ÙˆÓ ‚ÚÂÊÒÓ Î·Ù¿ Ù· ÂfiÌÂÓ· 4 ¯ÚfiÓÈ· ·ÁˆÁ‹˜ ‹Ù·Ó Â›Û˘ ηχÙÂÚË ·fi ÂΛÓË ÙˆÓ ·È‰ÈÒÓ (6). ∞Ó¿ÏÔÁ· ·ÔÙÂϤÛÌ·Ù· ·Ó·Ê¤ÚÔÓÙ·È ÛÙË ÌÂϤÙË ÙˆÓ Ranke et al (8). ∂›Û˘, Û ÌÈ· ÔÌ¿‰· ·È‰ÈÒÓ Ì ¤Ó·ÚÍË ·ÁˆÁ‹˜ Û ËÏÈΛ· 5,8±3,8 ÌËÓÒÓ, ÔÈ ·ÛıÂÓ›˜ ¤Êı·Û·Ó ÌÂÙ¿ ·ÁˆÁ‹ ÁÈ· 8 ¤ÙË Û ·Ó¿ÛÙËÌ· ˘„ËÏfiÙÂÚÔ ·fi ÙÔ ‡„Ô˜-ÛÙfi¯Ô, ÁÂÁÔÓfi˜ Ô˘ ˘ÔÁÚ·ÌÌ›˙ÂÈ ÙË ÛËÌ·Û›· Ù˘ ¤ÁηÈÚ˘ ¤Ó·Ú͢ ·ÁˆÁ‹˜ (21). µ) ∫·Ù¿ ÙËÓ ÂÊ˂›· ∆Ô ‡„Ô˜ Ô˘ ·ÔÎÙ‹ıËΠηٿ Ù· ÚÔÂÊË‚Èο ¯ÚfiÓÈ· ÛÙË ÌÂϤÙË KIGS ‹Ù·Ó ηٿ 1,5 ÊÔÚ¿ ÌÂÁ·Ï‡ÙÂÚÔ ÛÙËÓ ÔÌ¿‰· Ì ÚÔÎÏËÙ‹ ‹‚Ë (ÏfiÁˆ ·‰˘Ó·Ì›·˜ Ê˘ÛÈÔÏÔÁÈ΋˜ ÂÓ‹‚ˆÛ˘), Û˘ÁÎÚÈÙÈο Ì ÂΛÓË Ì ·˘ÙfiÌ·ÙË ‹‚Ë (22). ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ·Ô‰fiıËΠPaediatriki 2009;72:1-9

ÛÙË ¯ÚÔÓÈ΋ ·Ú¿Ù·ÛË Î·Ù¿ 3,5-4,0 ¯ÚfiÓÈ· Ù˘ ‰È¿ÚÎÂÈ·˜ Ù˘ ¯ÔÚ‹ÁËÛ˘ rhGH ÛÙÔ ÚÔÂÊË‚ÈÎfi ÛÙ¿‰ÈÔ ÛÙËÓ ÔÌ¿‰· Ì ÙËÓ ÚÔÎÏËÙ‹ ‹‚Ë. ¶Ú¤ÂÈ fï˜ Ó· ÛËÌÂȈı› fiÙÈ Ë ÔÌ¿‰· Ì ÚÔÎÏËÙ‹ ‹‚Ë Â›¯Â ÌÈÎÚfiÙÂÚË ¯ÚÔÓÈ΋ ‰È¿ÚÎÂÈ· ÂÊË‚È΋˜ ·Ó¿Ù˘Í˘ Î·È ÌÈÎÚfiÙÂÚË ·fiÎÙËÛË ‡„Ô˘˜ ηٿ ÙËÓ ÂÊ˂›· (p<0,001). ∏ ‰È·ÊÔÚ¿ ÙˆÓ SDS ÙÔ˘ ÙÂÏÈÎÔ‡-·Ú¯ÈÎÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜ ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚË ÛÙËÓ ÔÌ¿‰· Ì ÚÔÎÏËÙ‹ ‹‚Ë Û˘ÁÎÚÈÙÈο Ì ÂΛÓË Ì ·˘ÙfiÌ·ÙË ‹‚Ë (2,7 ¤Ó·ÓÙÈ 1,9, p<0,001). ∏ ηχÙÂÚË ·¿ÓÙËÛË ÛÙËÓ ÔÌ¿‰· Ì ÙËÓ ÚÔÎÏËÙ‹ ‹‚Ë ÔÊ›ÏÂÙ·È Èı·ÓfiÓ ÛÙÔ ¯·ÌËÏfiÙÂÚÔ ‡„Ô˜ ÚÔ ·ÁˆÁ‹˜ Î·È Û˘ÓÂÒ˜ ÛÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ‰˘Ó·ÌÈÎfi ·‡ÍËÛ˘, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙË ÛÔ‚·ÚfiÙÂÚË ·ÓÂ¿ÚÎÂÈ· GH (22). √ÌÔ›ˆ˜, ηٿ ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ù˘ ÌÂϤÙ˘ Ù˘ ∞ã ¶¶∫ (11), fiÔ˘ Û˘ÁÎÚ›ÓÂÙ·È ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ÙˆÓ ·È‰ÈÒÓ Ì ÚÔÎÏËÙ‹ ‹ ·˘ÙfiÌ·ÙË ‹‚Ë, ‰È·ÈÛÙÒıËΠfiÙÈ Ë SDS ÙÔ˘ ∆∞ ÛÙ· ·È‰È¿ Ì ÚÔÎÏËÙ‹ ‹‚Ë ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚË ÂÎÂ›ÓˆÓ Ì ·˘ÙfiÌ·ÙË ‹‚Ë (-1,04 ¤Ó·ÓÙÈ -1,7, p=0,001). ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ·Ô‰›‰ÂÙ·È ÛÙÔ fiÙÈ Ù· ·È‰È¿ Ì ÚÔÎÏËÙ‹ ‹‚Ë ‹Ù·Ó ˘„ËÏfiÙÂÚ· ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ‹‚˘ Î·È ÛÙÔ fiÙÈ Ë ¤Ó·ÚÍË Ù˘ ‹‚˘ ¤ÁÈÓ Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ· ·fi fi,ÙÈ ÛÙ· ·È‰È¿ Ì ·˘ÙfiÌ·ÙË ‹‚Ë. ∂Âȉ‹ ÙÔ ∆∞ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÙÔ ·Ó¿ÛÙËÌ· ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ‹‚˘ (17,22,23), Ú¤ÂÈ Ó· Á›ÓÂÈ ÚÔÛ¿ıÂÈ· Ó· ÂÈÙ¢¯ı› ·Ó¿ÛÙËÌ· ÂÓÙfi˜ ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ‹‚˘. ∂›Û˘, ¤¯ÂÈ ‚ÚÂı› fiÙÈ ÙÔ ‡„Ô˜ Ô˘ ·ÔÎÙ¿Ù·È Î·Ù¿ ÙËÓ ÂÊ˂›· Û˘Û¯ÂÙ›˙ÂÙ·È ·ÚÓËÙÈο Ì ÙËÓ ËÏÈΛ· ¤Ó·Ú͢ Ù˘ ‹‚˘ ÙfiÛÔ ÛÙ· Ê˘ÛÈÔÏÔÁÈο ·È‰È¿ fiÛÔ Î·È Û ÂΛӷ Ô˘ Ï·Ì‚¿ÓÔ˘Ó ·ÁˆÁ‹ Ì rhGH (24,25).

¶·Ú¿ÌÂÙÚÔÈ Ô˘ ÂËÚ¿˙Ô˘Ó ÙËÓ ·‡ÍËÛË Î·Ù¿ ÙËÓ ÂÊ˂›· 1. A˘ÍË̤ÓË ‰fiÛË rhGH ηٿ ÙËÓ ÂÊ˂›· ∂›Ó·È ÁÓˆÛÙfi fiÙÈ Ë ¤ÎÎÚÈÛË GH ·˘Í¿ÓÂÙ·È ÛÙ· Ê˘ÛÈÔÏÔÁÈο ·È‰È¿ ηٿ 2 Ì 4 ÊÔÚ¤˜ ηٿ ÙËÓ ÂÊ˂›·, Ì ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (IGF-1). ∏ ·Ú·Ù‹ÚËÛË ·˘Ù‹ Ô‰‹ÁËÛ ÛÙÔ ÂÚÒÙËÌ· Ì‹ˆ˜ Ë ¯Ú‹ÛË ˘„ËÏÒÓ ‰fiÛÂˆÓ rhGH ηٿ ÙËÓ ÂÊ˂›· ı· Û˘Ó¤‚·ÏÏ ÛÙË ‚ÂÏÙ›ˆÛË ÙÔ˘ ∆∞. ™Â ÌÈ· ÌÂϤÙË ‰È·ÈÛÙÒıËΠfiÙÈ Ë ‰ÔÛÔÏÔÁ›· rhGH 30 IU/m2/‚‰ÔÌ¿‰· ÛÙËÓ ÂÊ˂›·, Û˘ÁÎÚÈÙÈο Ì 15 IU/m2/‚‰ÔÌ¿‰· (0,28 ¤Ó·ÓÙÈ 0,14 mg/kg/Ë̤ڷ), ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÌÈÎÚ‹ ‚ÂÏÙ›ˆÛË ÛÙÔ ∆∞ (3 cm) (26,27). ∞ÓÙ›ıÂÙ·, Ë ¯Ú‹ÛË ˘„ËÏÒÓ ‰fiÛÂˆÓ rhGH (0,7 mg/kg/‚‰ÔÌ¿‰·), Û˘ÁÎÚÈÙÈο Ì 0,28 mg/kg/‚‰ÔÌ¿‰·, ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÛÙËÓ SDS ÙÔ˘ ∆∞ (0,0 ¤Ó·ÓÙÈ -0,7, ·ÓÙ›ÛÙÔȯ·) (27). µÚ¤ıËΠfiÙÈ Ë ÌÂÁ·Ï‡ÙÂÚË ‰fiÛË ‰ÂÓ ÂÈÙ¿¯˘Ó ˘¤ÚÌÂÙÚ· ÙËÓ ÔÛÙÈ΋ ˆÚ›Ì·ÓÛË (‰È·ÊÔÚ¿ ÔÛÙÈ΋˜ ËÏÈΛ·˜ 3,0 ¤Ó·ÓÙÈ 3,2 ÂÙÒÓ Û ‰È¿ÛÙËÌ· 3

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·5

5

¶·È‰È¿ Ì ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘

¶›Ó·Î·˜ 3. ∆ÂÏÈÎfi ·Ó¿ÛÙËÌ· ·È‰ÈÒÓ Ì IGHD ˘fi ·ÁˆÁ‹ Ì rhGH ªÂϤÙË ∫πGS ¡CGS

º‡ÏÔ

n

¢fiÛË rhGH (mg/kg/‚‰)

¢È¿ÚÎÂÈ· ·ÁˆÁ‹˜ (¤ÙË)

SDS TA

SDS ∆∞-À™

∞ £ ∞ £

792 466 2.095 1.116

0,18 0,18 0,28 0,29

8,1 7,9 5,2 5,0

-1,0 -1,3 -1,1 -1,3

-0,2 -0,5 -0,7 -0,9

TA = ∆ÂÏÈÎfi ·Ó¿ÛÙËÌ·, À™ = ⁄„Ô˜ ÛÙfi¯Ô˜, ∞ = ÕÚÚÂÓ, £ = £‹Ï˘

¯ÚfiÓˆÓ ıÂÚ·›·˜ ÁÈ· ÙȘ ÔÌ¿‰Â˜ Ì ÙË ÌÂÁ·Ï‡ÙÂÚË Î·È ÙË ÌÈÎÚfiÙÂÚË ‰fiÛË, ·ÓÙ›ÛÙÔȯ·) Î·È ‰ÂÓ Â›¯Â ÛËÌ·ÓÙÈΤ˜ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ (ÂÎÙfi˜ ·fi ÙËÓ ·‡ÍËÛË ÙˆÓ ÂÈ¤‰ˆÓ ÙÔ˘ IGF1). ŸÌˆ˜, ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÙÔ˘ ÎfiÛÙÔ˘˜ ıÂÚ·›·˜. ∏ ¯ÔÚ‹ÁËÛË ˘„ËÏÒÓ ‰fiÛÂˆÓ rhGH ¯ÔÚËÁÂ›Ù·È Û ÂÈÏÂÁ̤Ó˜ ÂÚÈÙÒÛÂȘ Î·È ¤Ó‰ÂÈÍË ·ÔÙÂÏ› ÙÔ ¯·ÌËÏfi ·Ó¿ÛÙËÌ· ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ‹‚˘.

2. ∞Ó·ÛÙÔÏ‹ Ù˘ ÂÊË‚È΋˜ ·Ó¿Ù˘Í˘ (¯Ú‹ÛË ·Ó·ÏfiÁˆÓ Ù˘ Gonadotropin Releasing Hormone) H ¯Ú‹ÛË GnRH analogs (GnRH-an) Û ·È‰È¿ Ì ÚfiˆÚË ‹‚Ë ·ԉ›¯ıËΠfiÙÈ ‚ÂÏÙÈÒÓÂÈ ÛËÌ·ÓÙÈο ÙÔ ∆∞ (28). º·›ÓÂÙ·È, fï˜, fiÙÈ Û ·È‰È¿ Ì IGHD, Ë ·ÁˆÁ‹ ·˘Ù‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì rhGH Ô‰ËÁ› Û ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË ÙÔ˘ ÚÔ‚ÏÂfiÌÂÓÔ˘ ∆∞. ŒÙÛÈ, Û ÌÈ· ÌÂϤÙË ·fi ÙÔ˘˜ Mericq et al. (29) Û 21 ·È‰È¿ Ì ·ÓÂ¿ÚÎÂÈ· GH, ·Ó·Ê¤ÚÂÙ·È fiÙÈ ÙÔ ÚÔ‚ÏÂfiÌÂÓÔ ∆∞ ÛÙËÓ ÔÌ¿‰· Ô˘ ¤Ï·‚Â Û˘Ó‰˘·ÛÌfi GH Î·È GnRH-an ÁÈ· 3 ¤ÙË ‹Ù·Ó -1,3±0,5 SD, ÂÓÒ ÛÙËÓ ÔÌ¿‰· Ô˘ ¤Ï·‚ ÌfiÓÔ GH ‹Ù·Ó -2,7±0,3 SD (26). ∆· Â˘Ú‹Ì·Ù· ·˘Ù¿ ‰ÂÓ ÂȂ‚·ÈÒÓÔÓÙ·È ·fi ÙȘ ÌÂÁ¿Ï˜ ÔÏ˘ÎÂÓÙÚÈΤ˜ ÌÂϤÙ˜ KIGS (30) Î·È NCGS (31). ÃÚÂÈ¿˙ÔÓÙ·È ÂÚÈÛÛfiÙÂÚ˜ ÚÔÔÙÈΤ˜ ÌÂϤÙ˜ ÁÈ· ÙËÓ ÂȂ‚·›ˆÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ ·˘ÙÒÓ. ∏ ·ÓˆÙ¤Úˆ ·ÁˆÁ‹ ·¢ı‡ÓÂÙ·È Î˘Ú›ˆ˜ Û ·È‰È¿ Ì ÚÔ¯ˆÚË̤ÓË ‹ Ù·¯¤ˆ˜ ÂÍÂÏÈÛÛfiÌÂÓË ‹‚Ë.

∆ÂÏÈÎfi ·Ó¿ÛÙËÌ· ∞fi ÙËÓ ÔÏ˘ÎÂÓÙÚÈ΋ ÌÂϤÙË KIGS, Ë ÔÔ›· Â›Ó·È ÌÈ· ÌÂÁ¿ÏË ÂȉËÌÈÔÏÔÁÈ΋ ‚¿ÛË ‰Â‰ÔÌ¤ÓˆÓ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ 3.966 ·ÛıÂÓ›˜ Ì GHD ·fi ‰È¿ÊÔÚ˜ ¯ÒÚ˜, ‰È·ÈÛÙÒıËÎ·Ó ‰È·ÊÔÚ¤˜ ÛÙÔ ıÂÚ·¢ÙÈÎfi ·ÔÙ¤ÏÂÛÌ· ·ÁˆÁ‹˜ Ì rhGH Û ·È‰È¿ Ì IGHD ·Ó¿ÏÔÁ· Ì ÙË Ê˘Ï‹, ÙÔ Ê‡ÏÔ, ηıÒ˜ Î·È ÙËÓ ·ÚÔ˘Û›· ÌÂÌÔӈ̤Ó˘ IGHD ‹ ÔÏÏ·Ï‹˜ ˘ÔÊ˘Ûȷ΋˜ ·ÓÂ¿ÚÎÂÈ·˜ (22).

To TA ·ÛıÂÓÒÓ Ì IGHD ˘fi ·ÁˆÁ‹ Ì rhGH ¤¯ÂÈ ·ÚÔ˘ÛÈ¿ÛÂÈ ÛÙ·‰È·Î‹ ‚ÂÏÙ›ˆÛË Ì ÙËÓ ¿ÚÔ‰Ô ÙˆÓ ÂÙÒÓ. ŒÙÛÈ, ·fi ÙËÓ ·Ó·‰ÚÔÌÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ù˘ ÌÂϤÙ˘ Ù˘ ∞ã ¶¶∫ (11) ÁÈ· Ù· ¯ÚfiÓÈ· 1970 ˆ˜ 1997 (¶›Ó·Î·˜ 3), ‰È·ÈÛÙÒıËΠfiÙÈ Ì ÙËÓ ÚÔԉ¢ÙÈ΋ ·‡ÍËÛË Ù˘ ‰fiÛ˘ Ù˘ GH (·fi

0,4 Û 0,6 IU/kg/‚‰ÔÌ¿‰·) Î·È ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ÂÓ¤ÛˆÓ/‚‰ÔÌ¿‰· (·fi 3,5 Û 6,4/‚‰ÔÌ¿‰·), ÙÔ ∆∞ ‚ÂÏÙÈÒıËΠÛÙ·‰È·Î¿ ·fi -3,9 Û -2,4 SDS. ¢Â‰Ô̤ӷ ·fi ÙȘ ‰‡Ô ÌÂÁ·Ï‡ÙÂÚ˜ ÔÏ˘ÎÂÓÙÚÈΤ˜ ÌÂϤÙ˜ Ô˘ ÂÎÚÔÛˆÔ‡Ó ÙËÓ Â˘Úˆ·˚΋ (KIGS) (27) Î·È ÙËÓ ·ÌÂÚÈηÓÈ΋ ÂÌÂÈÚ›· (NCGS) (32) Ê·›ÓÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 3. ∞fi ÙȘ Ì·ÎÚÔ¯ÚfiÓȘ ·˘Ù¤˜ ÌÂϤÙ˜ ·Ô‰ÂÈÎÓ‡ÂÙ·È fiÙÈ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ·ÛıÂÓ›˜ ‰ÂÓ Êı¿ÓÔ˘Ó ÛÙÔ ÎÏËÚÔÓÔÌÈο ÚÔ‚ÏÂfiÌÂÓÔ ∆∞. AÓ¿ÏÔÁ· ·ÔÙÂϤÛÌ·Ù· Ì ÙȘ ÌÂϤÙ˜ KIGS Î·È NCGS ·Ó·Ê¤ÚÔÓÙ·È ÛÙË ÌÂϤÙË Ù˘ ∞ã ¶¶∫ (11), fiÔ˘ Û 116 ·È‰È¿ Ì IGHD Ô˘ ¤Ï·‚·Ó ·ÁˆÁ‹ Ì rhGH ÌÂÙ¿ ÙÔ 1986 Û ‰fiÛË 0,15-0,18 mg/kg/‚‰ÔÌ¿‰·, ‰È·ÈÛÙÒıËΠfiÙÈ Ë SDS TA ‹Ù·Ó -1,3, ÂÓÒ Ë ‰È·ÊÔÚ¿ ÙˆÓ SDS ÙÂÏÈÎÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜ Î·È ‡„Ô˘˜-ÛÙfi¯Ô˘ ‹Ù·Ó -0,28. ªÈ· ÛԢˉÈ΋ ÌÂϤÙË ¤‰ÂÈÍ fiÙÈ Â›Ó·È ‰˘Ó·Ù‹ Ë Â›Ù¢ÍË ÂÓfi˜ SDS TA -0,3, Ô˘ Â›Ó·È ·Ó¿ÏÔÁË ÙÔ˘ ‡„Ô˘˜-ÛÙfi¯Ô˘ (33). ∆· ÎÚÈÙ‹ÚÈ· ÁÈ· ÙË ‰È·ÎÔ‹ Ù˘ ·ÁˆÁ‹˜ Ì rhGH ‰ÂÓ Â›Ó·È ÛÙ·ıÂÚ¿ Î·È ÔÈΛÏÏÔ˘Ó ÛÙȘ ‰È¿ÊÔÚ˜ ¯ÒÚ˜. ŒÙÛÈ, Û οÔȘ ¯ÒÚ˜ ‰È·ÎfiÙÂÙ·È Ë ·ÁˆÁ‹ fiÙ·Ó ÙÔ ·Ó¿ÛÙËÌ· Êı¿ÛÂÈ ÂÓÙfi˜ ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ ÁÈ· ÙËÓ ËÏÈΛ· Î·È ÙÔ Ê‡ÏÔ, ÂÓÒ Û ¿ÏϘ ¯ÒÚ˜ Ë ·ÁˆÁ‹ Ì rhGH Û˘Ó¯›˙ÂÙ·È Ì¤¯ÚÈ Ó· ÂÈÙ¢¯ı› ∆∞ Î·È Î·ÙfiÈÓ Û˘Ó¯›˙ÂÙ·È Ì ¯·ÌËÏfiÙÂÚ˜ ‰fiÛÂȘ, ˆ˜ ıÂÚ·›· ˘ÔηٿÛÙ·Û˘, ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (22). ™ÙË ÌÂϤÙË ∫IGS ‰È·ÈÛÙÒıËΠfiÙÈ Û 22% ÙˆÓ ·ÛıÂÓÒÓ Ë ·ÁˆÁ‹ Ì rhGH ‰È·ÎfiËΠÚÈÓ ÙËÓ Â›Ù¢ÍË ÙÔ˘ ∆∞ (34). ™Â Ì›· ¿ÏÏË ÌÂϤÙË ·Ó·Ê¤ÚÂÙ·È fiÙÈ 50% ÙˆÓ ·ÛıÂÓÒÓ ‰ÂÓ ¤¯Ô˘Ó Ï‹ÚË Û˘ÌÌfiÚʈÛË Ì ÙËÓ ·ÁˆÁ‹ Î·È Ë Û˘¯Ó‹ ·Ú¿ÏË„Ë ÙˆÓ ‰fiÛÂˆÓ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· Ì›ˆÛË ÙÔ˘ ∂ƒ∞ Î·È ¯·ÌËÏfiÙÂÚÔ ∆∞ (35). ™ÙÔÓ ¶›Ó·Î· 4 ·Ó·Ê¤ÚÔÓÙ·È Ù· Û˘Ó‹ıË ·›ÙÈ· ÁÈ· ÙË ÌÂȈ̤ÓË ·¿ÓÙËÛË ÛÙËÓ ·ÁˆÁ‹ Ì rhGH Û ·È‰È¿ Ì GHD (13). ™ÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜ Û˘ÓÈÛÙ¿Ù·È Ó· ÂÍÂÙ·Ûı› ΢ڛˆ˜ ÙÔ ÂӉ¯fiÌÂÓÔ ÂÏ·Ùو̤Ó˘ Û˘ÌÌfiÚʈÛ˘ Ì ÙËÓ ·ÁˆÁ‹. ªÂÙ¿ ÙËÓ ÔÏÔÎÏ‹ÚˆÛË Ù˘ ıÂÚ·›·˜ Î·È ÙËÓ ·fiÎÙËÛË ÙÔ˘ ÙÂÏÈÎÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜, Â›Ó·È ··Ú·›ÙËÙÔ Ó· Á›ÓÂÈ Â·Ó¤ÏÂÁ¯Ô˜ Ì ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘ GH, Û ‰È¿ÛÙËÌ· 6 ÌËÓÒÓ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ rhGH. ∆Ô ÔÛÔÛÙfi ·Ú·ÌÔÓ‹˜ Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ GH ¶·È‰È·ÙÚÈ΋ 2009;72:1-9

Pediatric Jan-Feb 09

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™ÂÏ›‰·6

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∫˘Úȷ΋ ∫·Ú·‚·Ó¿ÎË-∫·Ú·Ó¿ÛÈÔ˘

¶›Ó·Î·˜ 4. ∞›ÙÈ· ÌÂȈ̤Ó˘ ·ÓÙ·fiÎÚÈÛ˘ ÛÙËÓ ·ÁˆÁ‹ Ì rhGH Û ·È‰È¿ Ì GHD 1. ªË Û˘ÌÌfiÚʈÛË Ì ÙËÓ ·ÁˆÁ‹ 2. ∞ÓÂ·Ú΋˜ ÚÔÂÙÔÈÌ·Û›· Î·È ·Ôı‹Î¢ÛË Ù˘ rhGH 3. §·Óı·Ṳ̂ÓË Ù¯ÓÈ΋ ¯ÔÚ‹ÁËÛ˘ ÙˆÓ ÂÓ¤ÛÂˆÓ 4. ¶ÚfiÁÚ·ÌÌ· ¯ÔÚ‹ÁËÛ˘ rhGH 5. À¤ÚÌÂÙÚË ‰fiÛË ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉÒÓ Û ·È‰È¿ ÌÂ Û˘Ó˘¿Ú¯Ô˘Û· ·ÓÂ¿ÚÎÂÈ· ACTH 6. YÔı˘ÚÂÔÂȉÈÛÌfi˜ Ô˘ ‰ÂÓ ·ÓÙÈÌÂÙˆ›ÛÙËΠ7. ∞ÓÂ·Ú΋˜ ‰È·ÙÚÔÊ‹ 8. ™˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ 9. ∞ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ rhGH 10. πÛÙÔÚÈÎfi ·ÎÙÈÓÔ‚ÔÏ›·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ 11. æ˘¯ÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜ 12. §·Óı·Ṳ̂ÓË ‰È¿ÁÓˆÛË

ÔÈΛÏÏÂÈ. ŒÙÛÈ, ÔÈ Tauber Î·È Û˘Ó. (36) ·Ó·Ê¤ÚÔ˘Ó fiÙÈ, ·fi Ù· ·È‰È¿ Ì ÌÂÚÈ΋ ·ÓÂ¿ÚÎÂÈ· GH ÛÙȘ ·Ú¯ÈΤ˜ ‰˘Ó·ÌÈΤ˜ ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘ GH, 71% ›¯·Ó Ê˘ÛÈÔÏÔÁÈ΋ ·¿ÓÙËÛË Î·Ù¿ ÙÔÓ Â·Ó¤ÏÂÁ¯Ô. ∞ÓÙ›ıÂÙ·, ÌfiÓÔ 36% ÙˆÓ ·È‰ÈÒÓ Ì Ï‹ÚË ·ÓÂ¿ÚÎÂÈ· GH Î·È 3% ÙˆÓ ·È‰ÈÒÓ Ì ÔÏÏ·Ï‹ ˘ÔÊ˘Ûȷ΋ ·ÓÂ¿ÚÎÂÈ· ›¯·Ó Ê˘ÛÈÔÏÔÁÈ΋ ·¿ÓÙËÛË GH ηٿ ÙÔÓ Â·Ó¤ÏÂÁ¯Ô (37). ™Â ÂÚ›ÙˆÛË Ô˘ ı· ‰È·ÈÛÙˆı› ·ÓÂ¿ÚÎÂÈ· GH ηٿ ÙÔÓ Â·Ó¤ÏÂÁ¯Ô, ¯ÚÂÈ¿˙ÂÙ·È Ó· Û˘Ó¯ÈÛı› Ë ıÂÚ·›· Ì rhGH. ∏ ¯ÔÚ‹ÁËÛË rhGH Û ÂÓ‹ÏÈΘ Ì ·ÓÂ¿ÚÎÂÈ· GH ¤¯ÂÈ Û˘˙ËÙËı› ·ÚÎÂÙ¿ ÛÙË ‚È‚ÏÈÔÁÚ·Ê›·, ÏfiÁˆ ÙˆÓ ÚÔ‚ÏËÌ¿ÙˆÓ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ˘˜ ·ÛıÂÓ›˜ ·˘ÙÔ‡˜. ∏ ¤ÏÏÂÈ„Ë GH ÛÙÔ˘˜ ÂÓ‹ÏÈΘ Ô‰ËÁ› Û ‰È·Ù·Ú·¯¤˜ ÛÙË ÛˆÌ·ÙÈ΋ Û‡ÛÙ·ÛË (ÎÂÓÙÚÈ΋ ·¯˘Û·ÚΛ·, ÂÏ·Ùو̤ÓË ¿ÏÈÔ˜ Ì¿˙·) Î·È ÛÙÔÓ ÌÂÙ·‚ÔÏÈÛÌfi ÙˆÓ ÔÛÙÒÓ (ÂÏ·Ùو̤ÓË ÔÛÙÈ΋ ˘ÎÓfiÙËÙ·, ÔÛÙÂÔfiÚˆÛË). ∂›Û˘ Â·˘Í¿ÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· ηډȷÁÁÂȷο ÂÂÈÛfi‰È· ÏfiÁˆ ˘ÂÚ¯ÔÏËÛÙÂÚÔÏ·ÈÌ›·˜, ˘ÂÚÙÚÈÁÏ˘ÎÂÚȉ·ÈÌ›·˜ Î·È ÂÈÙ¿¯˘ÓÛ˘ Ù˘ ·ıËÚÔÛÎÏ‹ÚˆÛ˘ (38,39,40). ∂ÈÚfiÛıÂÙ·, Ô‰ËÁ› Û ¯·ÌËÏfiÙÂÚË ÔÈfiÙËÙ· ˙ˆ‹˜, ÏfiÁˆ ηٷ‚ÔÏ‹˜, ÌÂȈ̤Ó˘ ÂÓÂÚÁËÙÈÎfiÙËÙ·˜ Î·È ÌÂȈ̤Ó˘ ¢ÂÍ›·˜ (41). ∞ÓÙ›ıÂÙ·, Ë ¯ÔÚ‹ÁËÛË rhGH ¤¯ÂÈ ‰È·ÈÛÙˆı› fiÙÈ ‚ÂÏÙÈÒÓÂÈ ÙË ÛˆÌ·ÙÈ΋ Û‡ÛÙ·ÛË Î·È ÙËÓ ÔÈfiÙËÙ· ˙ˆ‹˜ ÛÙÔ˘˜ ÂÚÈÛÛfiÙÂÚÔ˘˜ ·ÛıÂÓ›˜ (42). ŒÙÛÈ, Ë ¯Ú‹ÛË Ù˘ rhGH Û ÌÂȈ̤ÓË ‰fiÛË ÛÙËÓ ÂÓ‹ÏÈÎÔ ˙ˆ‹ ¤¯ÂÈ ·Ú¯›ÛÂÈ Ó· ¯ÔÚËÁÂ›Ù·È Û ÔÚÈṲ̂Ó˜ ¯ÒÚ˜ ÁÈ· ÙËÓ ÚfiÏË„Ë ÙˆÓ ÌÂÙ·‚ÔÏÈÎÒÓ Î·È „˘¯ÔÎÔÈÓˆÓÈÎÒÓ ÂÈÙÒÛÂˆÓ Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ GH.

¶·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· Afi ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙÔ ∆∞ ·È‰ÈÒÓ Ì ȉÈÔ·ı‹ ‹ ÔÚÁ·ÓÈ΋ GHD (¶›Ó·Î·˜ 5) ÛËÌ·ÓÙÈÎfiÙÂÚÔÈ Â›Ó·È ÙÔ ‡„Ô˜ ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÁˆÁ‹˜ Ì rhGH, ηıÒ˜ Î·È ÙÔ ‡„Ô˜ ÛÒÌ·ÙÔ˜ ÁÔÓ¤ˆÓ (22).

Paediatriki 2009;72:1-9

¶›Ó·Î·˜ 5. ¶·Ú¿ÁÔÓÙ˜ Ô˘ ÂËÚ¿˙Ô˘Ó ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ÌÂÙ¿ ·ÁˆÁ‹ Ì rhGH 1. ⁄„Ô˜ ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÁˆÁ‹˜ Ì rhGH 2. ⁄„Ô˜ ÁÔÓ¤ˆÓ 3. ∏ÏÈΛ· ¤Ó·Ú͢ ·ÁˆÁ‹˜ Ì rhGH 4. ™˘ÓÔÏÈ΋ ‰È¿ÚÎÂÈ· ·ÁˆÁ‹˜ Ì rhGH 5. ∏ÏÈΛ· ¤Ó·Ú͢ Ù˘ ‹‚˘ 6. µ¿ÚÔ˜ Î·È Ì‹ÎÔ˜ ηٿ ÙËÓ Á¤ÓÓËÛË 7. ƒ˘ıÌfi˜ ·‡ÍËÛ˘ ηٿ ÙÔÓ 1Ô ¯ÚfiÓÔ ·ÁˆÁ‹˜ Ì rhGH 8. ¢ÔÛÔÏÔÁ›· rhGH

ÕÏϘ ·Ú¿ÌÂÙÚÔÈ Ô˘ Û˘Û¯ÂÙ›˙ÔÓÙ·È ÛËÌ·ÓÙÈο Ì ÙÔ ∆∞ ηٿ ÙËÓ ·ÁˆÁ‹ Ì rhGH Î·È ·ÏÏËÏÂȉÚÔ‡Ó ÌÂٷ͇ ÙÔ˘˜ Â›Ó·È Ë ËÏÈΛ· ¤Ó·Ú͢ ·ÁˆÁ‹˜ Ì rhGH Î·È Ë Û˘ÓÔÏÈ΋ ‰È¿ÚÎÂÈ· ·ÁˆÁ‹˜, ȉȷ›ÙÂÚ· ηٿ ÙÔ ÚÔÂÊË‚ÈÎfi ÛÙ¿‰ÈÔ. ŒÙÛÈ, ¤Ó· ÌÈÎÚfiÙÂÚÔ ·È‰› ı· Ï¿‚ÂÈ ·ÁˆÁ‹ Ì rhGH ÁÈ· ÌÂÁ·Ï‡ÙÂÚÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· Î·È ÙÂÏÈο ı· Ï¿‚ÂÈ Û˘ÓÔÏÈο ÌÂÁ·Ï‡ÙÂÚË ÔÛfiÙËÙ· rhGH ηÈ, ΢ڛˆ˜, ηٿ Ù· ϤÔÓ ·Ô‰ÔÙÈο ˆ˜ ÚÔ˜ ÙÔ ∆∞ ÚÔÂÊË‚Èο ¯ÚfiÓÈ· (13,34). ∂›Û˘, ÛËÌ·ÓÙÈ΋ Û˘Û¯¤ÙÈÛË Ì ÙÔ ∆∞ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙÔ ‡„Ô˜-ÛÙfi¯Ô˜, Ë SDS ‡„Ô˘˜ Î·È Ë ËÏÈΛ· ¤Ó·Ú͢ Ù˘ ‹‚˘, ÙÔ ‚¿ÚÔ˜ Î·È ÙÔ Ì‹ÎÔ˜ Á¤ÓÓËÛ˘ (13,43). √È Ì¤ÁÈÛÙ˜ ÙÈ̤˜ GH ηٿ ÙȘ ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘, ˆ˜ ‰Â›ÎÙ˘ ÛÔ‚·ÚfiÙËÙ·˜ Ù˘ IGHD, ‰ÂÓ Ê·›ÓÂÙ·È Ó· ÂËÚ¿˙Ô˘Ó ÙÔ ∆∞ (13,22). ŒÓ·˜ ¿ÏÏÔ˜ ·Ú¿ÁÔÓÙ·˜ Ô˘ ÂËÚ¿˙ÂÈ ÙÔ ∆∞ Â›Ó·È Ë ‰fiÛË Ù˘ rhGH. ∂›Ó·È fï˜ ·ÍÈÔÛËÌ›ˆÙÔ fiÙÈ Û ·È‰È¿ Ì IGHD, Ë Û˘Ì‚·ÙÈ΋ ‰ÔÛÔÏÔÁ›· rhGH (0,18-0,3 mg/kg/‚‰ÔÌ¿‰·) Ô‰ËÁ› Û ∆∞ ¯·ÌËÏfiÙÂÚÔ ÙÔ˘ ‡„Ô˘˜-ÛÙfi¯Ô˘ (À™) (44,45), ÂÓÒ Ë ¯Ú‹ÛË ˘„ËÏÒÓ ‰fiÛÂˆÓ rhGH (0,7 mg/kg/‚‰ÔÌ¿‰·) (27) ‹ Ë ÂÓˆÚ›˜ ‰È¿ÁÓˆÛË Ù˘ GHD Î·È ¤Ó·ÚÍË ·ÁˆÁ‹˜ Ô‰ËÁÔ‡Ó ÛÙËÓ Â›Ù¢ÍË ÂÓfi˜ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ∆∞ Ô˘ ‰ÂÓ ‰È·Ê¤ÚÂÈ ·fi ÙÔ À™. §·Ì‚¿ÓÔÓÙ·˜, fï˜, ˘’fi„ÈÓ ÙÔ ˘„ËÏfi ÎfiÛÙÔ˜ Î·È ÙȘ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ Ù˘ ˘„ËÏ‹˜ ‰ÔÛÔÏÔÁ›·˜ rhGH, Ë ıÂÚ·›· Ú¤ÂÈ Ó· ÂÍ·ÙÔÌÈ·ÂÙ·È Î·È Ë ¯Ú‹ÛË ˘„ËÏÒÓ ‰fiÛÂˆÓ Ó· ÂÚÈÔÚ›˙ÂÙ·È Û ÂÚÈÙÒÛÂȘ ÌÂÁ·Ï‡ÙÂÚˆÓ ·È‰ÈÒÓ Ì Ôχ ¯·ÌËÏfi ·Ó¿ÛÙËÌ· (46). ¶Ú¤ÂÈ, fï˜, Ó· ÛËÌÂȈı› fiÙÈ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ıÂÚ·›·˜ ÌÂ Û˘Ì‚·ÙÈΤ˜ ‰fiÛÂȘ rhGH ηÈ, Ôχ ÂÚÈÛÛfiÙÂÚÔ, Ì ˘„ËϤ˜ ‰fiÛÂȘ rhGH, Â›Ó·È ··Ú·›ÙËÙË Ë Ì¤ÙÚËÛË ÙˆÓ ÂÈ¤‰ˆÓ ÙˆÓ IGF-I Î·È IGFBP-3 οı 6-12 Ì‹Ó˜, ÁÈ· Ó· ÚÔÛ‰ÈÔÚÈÛı› Ë ··ÈÙÔ‡ÌÂÓË ‰fiÛË rhGH Î·È Ó· ·ÔÊ¢¯ıÔ‡Ó Èı·Ó¤˜ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ (47). ∆· ·È‰È¿ Ì ÔÚÁ·ÓÈ΋ ·ÓÂ¿ÚÎÂÈ· GH ÏfiÁˆ Ï¢¯·ÈÌ›·˜ ‹ fiÁÎˆÓ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ·ÔÙÂÏÔ‡Ó Ì›· ȉȷ›ÙÂÚË ÔÌ¿‰· ·ÛıÂÓÒÓ Ô˘ ‰ÂÓ ··ÓÙÔ‡Ó ÛÙÔÓ ›‰ÈÔ ‚·ıÌfi ÛÙË ıÂÚ·›· Ì rhGH, ÏfiÁˆ Ù˘ ·ÎÙÈÓÔ‚ÔÏ›·˜ ÎÚ·Ó›Ô˘ ‹ Î·È ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ (™™). ŒÙÛÈ, Ù· ·È‰È¿ Ô˘ ¤Ï·‚·Ó ·ÎÙÈÓÔ‚ÔÏ›· ™™ ÌÂÙ¿ ·ÁˆÁ‹ Ì rhGH ÂÈÙ˘Á¯¿ÓÔ˘Ó

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¶·È‰È¿ Ì ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘

¯·ÌËÏfiÙÂÚÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ·fi ÂΛӷ Ô˘ ‰ÂÓ ¤Ï·‚·Ó ·ÎÙÈÓÔ‚ÔÏ›· ™™ (11,48). To ÁÂÁÔÓfi˜ ·˘Ùfi ·Ô‰›‰ÂÙ·È ÛÙËÓ ·Ó·ÛÙÔÏ‹ Ù˘ ·Ó¿Ù˘Í˘ Ù˘ ™™ ÌÂÙ¿ ÙËÓ ·ÎÙÈÓÔ‚ÔÏ›·, Ë ÔÔ›· Â›Ó·È ·Ó¿ÏÔÁË Ù˘ ‰fiÛ˘ Ù˘ (48). ŒÓ· ¿ÏÏÔ Úfi‚ÏËÌ· ÛÙ· ·È‰È¿ ·˘Ù¿ Â›Ó·È fiÙÈ Ë ·ÎÙÈÓÔ‚ÔÏ›· ÎÚ·Ó›Ô˘ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ÂÌÊ¿ÓÈÛË ÚÒÈÌ˘ ‹‚˘. º˘ÛÈÔÏÔÁÈο, Ë ·Ó¿Ù˘ÍË Î·Ù¿ ÙËÓ ÚÔÂÊË‚È΋ ÂÚ›Ô‰Ô ÂÈÙ˘Á¯¿ÓÂÙ·È Ì ÙËÓ ·‡ÍËÛË ÙˆÓ Ì·ÎÚÒÓ ÔÛÙÒÓ, ÂÓÒ Î·Ù¿ ÙËÓ ÂÊ˂›· Ì ÙËÓ ·‡ÍËÛË Ù˘ ™™. ™˘ÓÂÒ˜, Ë ÂÌÊ¿ÓÈÛË ÚÒÈÌ˘ ‹‚˘ ÛÙ· ·È‰È¿ Ì ÔÚÁ·ÓÈ΋ ·ÓÂ¿ÚÎÂÈ· GH Ô‰ËÁ› ÛÙË ‚Ú¿¯˘ÓÛË Ù˘ ÚÔÂÊË‚È΋˜ ÂÚÈfi‰Ô˘ Î·È ÛÙË ÌË ÈηÓÔÔÈËÙÈ΋ ·‡ÍËÛË ÙˆÓ Ì·ÎÚÒÓ ÔÛÙÒÓ. ¶ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó fiÙÈ Ì ÙËÓ ·Ó·ÛÙÔÏ‹ Ù˘ ‹‚˘ ÛÙ· ·È‰È¿ ·˘Ù¿ Ì ¯ÔÚ‹ÁËÛË GnRHan, ·Ú¿ÏÏËÏ· Ì ÙË ¯ÔÚ‹ÁËÛË rhGH, ÂÈÙ˘Á¯¿ÓÂÙ·È Î·Ï‡ÙÂÚÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ·fi ÂΛÓÔ Ô˘ ÂÈÙ˘Á¯¿ÓÂÙ·È ÌfiÓÔ Ì ÙË ¯ÔÚ‹ÁËÛË rhGH (49). ∂›Û˘, ÛËÌ·ÓÙÈ΋ Â›Ó·È Ë ¤ÁηÈÚË ¤Ó·ÚÍË rhGH Û ·È‰È¿ Ì ÂÓ‰ÔÎÚ·ÓÈ·ÎÔ‡˜ fiÁÎÔ˘˜, Ì ‹ ¯ˆÚ›˜ ·ÎÙÈÓÔ‚ÔÏ›· ÎÚ·Ó›Ô˘, Ë ¯ÔÚ‹ÁËÛË ÙˆÓ Û˘Ó‹ıˆÓ (Î·È fi¯È ¯·ÌËÏÒÓ) ‰fiÛÂˆÓ rhGH, ηıÒ˜ Â›Û˘ Î·È Ë ¯ÔÚ‹ÁËÛË Ù˘ ¯·ÌËÏfiÙÂÚ˘ ‰˘Ó·Ù‹˜ ‰fiÛ˘ ·ÎÙÈÓÔ‚ÔÏ›·˜ ™™ (48). ™˘ÌÂÚ·ÛÌ·ÙÈο, Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ‰˘Ó·Ùfi Ó· ÂÈÙ¢¯ı› Ê˘ÛÈÔÏÔÁÈÎfi ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· Û ·È‰È¿ Ì IGHD Ì ÙË ¯ÔÚ‹ÁËÛË Û˘Ì‚·ÙÈÎÒÓ ‰fiÛÂˆÓ rhGH, ÂÊfiÛÔÓ Ë ¤Ó·ÚÍË Ù˘ ·ÁˆÁ‹˜ Á›ÓÂÈ Û ÌÈÎÚ‹ ËÏÈΛ· Î·È ÂÍ·ÛÊ·Ï›˙ÂÙ·È Û˘ÌÌfiÚʈÛË Ì ÙËÓ Î·ıËÌÂÚÈÓ‹ ¯ÔÚ‹ÁËÛË, ηıÒ˜ Î·È Ì·ÎÚ¿ ÚÔÂÊË‚È΋ ¯ÔÚ‹ÁËÛË Ù˘ rhGH (50), Ô‡Ùˆ˜ ÒÛÙ ӷ ÂÈÙ¢¯ı› ÈηÓÔÔÈËÙÈÎfi ‡„Ô˜ ÚÈÓ ÙËÓ ÂÊ˂›·. ŸÌˆ˜, Â¿Ó ˘¿Ú¯Ô˘Ó ηı˘ÛÙÂÚË̤ÓË ‰È¿ÁÓˆÛË, ÁÔÓ›˜ Ì ¯·ÌËÏfi ·Ó¿ÛÙËÌ· ‹ ÂÍ·ÈÚÂÙÈο ¯·ÌËÏfi ‡„Ô˜ ηٿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜, Û˘ÓÈÛÙ¿Ù·È ÌÈ· ÈÔ ÂÍ·ÙÔÌÈÎÂ˘Ì¤ÓË Î·È ÂÈıÂÙÈ΋ ıÂÚ·›·. ∂›Û˘, Ú¤ÂÈ Ó· ˘ÔÁÚ·ÌÌÈÛı› Ë ÛËÌ·Û›· Ù˘ ¤ÁηÈÚ˘ ¤Ó·Ú͢ Ù˘ ·ÁˆÁ‹˜ Ì rhGH Û ·È‰È¿ Ì ÓÂÔÁÓÈ΋ ÌÂÌÔӈ̤ÓË GHD ‹ ÔÏÏ·Ï‹ ˘ÔÊ˘Ûȷ΋ ·ÓÂ¿ÚÎÂÈ·, ηıÒ˜ Î·È Û ·È‰È¿ Ì ÔÚÁ·ÓÈ΋ ·ÓÂ¿ÚÎÂÈ· GH. ™ËÌ·ÓÙÈÎfi˜ Â›Ó·È Â›Û˘ Ô Â·Ó¤ÏÂÁ¯Ô˜ ÙˆÓ ·È‰ÈÒÓ Ì ·ÓÂ¿ÚÎÂÈ· GH ÌÂÙ¿ ÙËÓ ÔÏÔÎÏ‹ÚˆÛË Ù˘ ıÂÚ·›·˜, Ì ‰˘Ó·ÌÈΤ˜ ‰ÔÎÈ̷ۛ˜ ÚfiÎÏËÛ˘ GH, ÁÈ· ÙÔ ÂӉ¯fiÌÂÓÔ Û˘Ó¤¯ÈÛ˘ Ù˘ ·ÁˆÁ‹˜ Ì rhGH ÛÙËÓ ÂÓ‹ÏÈÎÔ ˙ˆ‹.

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42. Drake WM, Howell SJ, Monson JP, Shalet SM. Optimizing gh therapy in adults and children. Endocr Rev 2001;22: 425-450.

43. ¶··‰ÔÔ‡ÏÔ˘ ª, ¡ÙÔ˘Ì¿ ™, ∫›ÙÛÈÔ˜ ∫, ∫·‰fiÁÏÔ˘ ¡, ∫ÒÛÙ· ∫, ∆ÛÈÔ‡Ú˘ π. ∆ÂÏÈÎfi ·Ó¿ÛÙËÌ· Û ·È‰È¿ Ì ·ÓÂ¿ÚÎÂÈ· ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ Ô˘ ¤Ï·‚·Ó ıÂÚ·›· ˘ÔηٿÛÙ·Û˘. ¶·È‰È·ÙÚÈ΋ 2008;71:128-134.

44. Rikken B, Massa GG, Wit JM. Final height in a large cohort of Dutch patients with growth hormone deficiency treated with growth hormone. Dutch Growth Hormone Working Group. Horm Res 1995;43:135-137.

45. Severi F. Final height in children with growth hormone deficiency. Horm Res 1995;43:138-140.

46. Ranke MB, Lindberg A, Martin DD. The mathematical model for total pubertal growth in idiopathic growth hormone (GH) deficiency suggests a moderate role of GH dose. J Clin Endocrinol Metab 2003;88:4748-4753.

47. Cohen P, Bright GM, Rogol AD, Kappelgaard AM, Rosenfeld RG. Effects of dose and gender on the growth and growth factor response to GH in GH-deficient children: implications for efficacy and safety. J Clin Endocrinol Metab 2002;87:90-98.

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Heller G, et al. Factors that affect final height and change in height standard deviation scores in survivors of childhood cancer treated with growth hormone: A report from the Childhood Cancer Survivor Study. J Clin Endocrinol & Metabolism 2004;89:4422-4427.

49. Gleeson HK, Stoeter R, Ogilvy-Stuart AL, Gattamaneni HR, Brennan BM, Shalet SM. Improvements in final

height over 25 years in growth hormone (GH)-deficient childhood survivors of brain tumors receiving GH replacement. J Clin Endocrinol Metab 2003;88:3682-3689.

50. Reiter EO, Cutfield WS. Growth hormone deficiency: puberty and final height. In: Ranke MB, Price DA, Reiter EO, editors. Growth Hormone Therapy in Pediatrics. 20 years of KIGS. Basel: Karger; 2007. pp. 136-144.

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REVIEW ARTICLE

£ÂÚ·›· Ì ·˘ÍËÙÈ΋ ÔÚÌfiÓË ÛÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi ∂Ó‰ÔÎÚÈÓÔÏÔÁÈÎfi ∆Ì‹Ì· ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó· AÏÏËÏÔÁÚ·Ê›·: ∂ϤÓË ∫Ô‡ÛÙ· lkousta@otenet.gr ™. ∞Ú‚·ÓÈÙ¿ÎË 6 ∫¤Ú΢ڷ 49100

E. ∫Ô‡ÛÙ·, Ã. ÷Ù˙Ë·ı·Ó·Û›Ô˘†, A. ¶··ı·Ó·Û›Ô˘ ¶ÂÚ›ÏË„Ë: ∆Ô Û‡Ó‰ÚÔÌÔ Prader-Willi Â›Ó·È ¤Ó· Û¿ÓÈÔ ÁÂÓÂÙÈÎfi Û‡Ó‰ÚÔÌÔ, ÙÔ ÔÔ›Ô ÔÊ›ÏÂÙ·È ÛÙËÓ ·ÒÏÂÈ· Ù˘ ÁÔÓȉȷ΋˜ ¤ÎÊÚ·Û˘ Ù˘ ÂÚÈÔ¯‹˜ 15q11-q13 ÙÔ˘ ·ÙÚÈÎÔ‡ ·ÏÏËÏfiÌÔÚÊÔ˘ ·ÔÙ˘ˆÌ¤ÓÔ˘ ÁÔÓ›‰ÈÔ˘. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ÛÙ·‰È·Î¿ ·˘Í·ÓfiÌÂÓË ˘ÂÚÊ·Á›·, ·¯˘Û·ÚΛ·, ˘ÔÁÔÓ·‰ÈÛÌfi, ¯·ÌËÏfi ·Ó¿ÛÙËÌ· Î·È ¤ÏÏÂÈ„Ë ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘. ∆· ·È‰È¿ Î·È ÔÈ ÂÓ‹ÏÈΘ Ì ÙÔ Û‡Ó‰ÚÔÌÔ ·Ó·Ù‡ÛÛÔ˘Ó ÓÔÛÔÁfiÓÔ ·¯˘Û·ÚΛ·, Ë ÔÔ›· Â›Ó·È Ë Î‡ÚÈ· ·ÈÙ›· Ù˘ ·˘ÍË̤Ó˘ ıÓËÛÈÌfiÙËÙ·˜ Ô˘ ·Ú·ÙËÚ›ٷÈ. ∏ ηı˘ÛÙ¤ÚËÛË Ù˘ ·Ó¿Ù˘Í˘ ÍÂÎÈÓ¿ ÌÂÙ¿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ˙ˆ‹˜, ‰È·ÙËÚÂ›Ù·È ÛÙËÓ ·È‰È΋ ËÏÈΛ· Î·È Î·Ù·Ï‹ÁÂÈ Û˘Ó‹ıˆ˜ Û ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ¯·ÌËÏfiÙÂÚÔ ÙÔ˘ ‡„Ô˘˜-ÛÙfi¯Ô˘ Î·È Û˘Ó‹ıˆ˜ οو ·fi Ù· fiÚÈ· ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡. ¶ÚfiÛÊ·Ù· ·Ú·ÙËÚ‹ıËΠıÂÙÈ΋ Â›‰Ú·ÛË Ù˘ ıÂÚ·›·˜ Ì ·˘ÍËÙÈ΋ ÔÚÌfiÓË, Ë ÔÔ›· ÂÈÙ·¯‡ÓÂÈ ÙÔÓ Ú˘ıÌfi ·‡ÍËÛ˘ Î·È ‚ÂÏÙÈÒÓÂÈ ÙÔ ¤ÏÏÂÈÌÌ· ‡„Ô˘˜ ÙˆÓ ·È‰ÈÒÓ. E›Ó·È Èı·ÓfiÓ Ë ıÂÚ·›·, ¯ÔÚËÁÔ‡ÌÂÓË Ì·ÎÚÔ¯ÚfiÓÈ·, Ó· ‚ÂÏÙÈÒÓÂÈ ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ·. ∫˘Ú›ˆ˜, fï˜, ‚ÂÏÙÈÒÓÂÈ ÙË ÛˆÌ·ÙÈ΋ Û‡ÛÙ·ÛË, ·˘Í¿ÓÔÓÙ·˜ ÙËÓ ¿ÏÈÔ Ì¿˙·, ÌÂÈÒÓÔÓÙ·˜ ÙÔÓ ÏÈÒ‰Ë ÈÛÙfi Î·È ‚ÂÏÙÈÒÓÔÓÙ·˜ ÙȘ ÂÓÂÚÁÂȷΤ˜ ‰·¿Ó˜, ¯ˆÚ›˜ ȉȷ›ÙÂÚ· ÛËÌ·ÓÙÈ΋ ÂÏ¿ÙÙˆÛË ÙÔ˘ BMI. ∂›Û˘, ‚ÂÏÙÈÒÓÂÈ ÙË Ì˘˚΋ ‰‡Ó·ÌË Î·È ÙËÓ ÎÈÓËÙÈÎfiÙËÙ· ÙˆÓ ÌÈÎÚÒÓ ·È‰ÈÒÓ. ∏ ÈÔ Û˘¯Ó‹ ·ÚÂÓ¤ÚÁÂÈ· Ù˘ ıÂÚ·›·˜ Â›Ó·È Ë ÛÎÔÏ›ˆÛË. √È Ì·ÎÚÔÚfiıÂÛ̘ ÂÈÙÒÛÂȘ Ù˘ ıÂÚ·›·˜ Ì ·˘ÍËÙÈ΋ ÔÚÌfiÓË ÛÙ· ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi, ȉȷ›ÙÂÚ· fiÛÔÓ ·ÊÔÚ¿ ÙÔÓ ÌÂÙ·‚ÔÏÈÛÌfi Ù˘ ÁÏ˘Îfi˙˘, Â›Ó·È ·ÎfiÌË ˘fi ‰ÈÂÚ‡ÓËÛË. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ Ì GH ·Ú·ÙËÚ‹ıËÎ·Ó ·ÈÊÓ›‰ÈÔÈ ı¿Ó·ÙÔÈ, ΢ڛˆ˜ ·fi ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Î·È ˘Ô·ÂÚÈÛÌfi. √È ·Ú·ÙËÚ‹ÛÂȘ ·˘Ù¤˜ Ô‰‹ÁËÛ·Ó Û ÚÔ‚ÏËÌ·ÙÈÛÌfi ˆ˜ ÚÔ˜ ÙËÓ ·ÛÊ¿ÏÂÈ· Ù˘ ıÂÚ·›·˜ Î·È ÙÔÓ›˙Ô˘Ó ÙË ÛËÌ·Û›· Ù˘ Ù·ÎÙÈ΋˜ Î·È ÚÔÛÂÎÙÈ΋˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ ηٿ ÙË ıÂÚ·›·. ∆Ô ‡„Ô˜ Û¿ÓÈ· ·ÔÙÂÏ› ÙÔ Î˘Ú›·Ú¯Ô Úfi‚ÏËÌ· ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ Î·È ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ ÙÔ˘˜, ÏfiÁˆ ÙˆÓ ÔÏÏ·ÏÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ Ô˘ ·ÓÙÈÌÂÙˆ›˙Ô˘Ó. ∏ ıÂÚ·›· Ì ·˘ÍËÙÈ΋ ÔÚÌfiÓË Ú¤ÂÈ Ó· ¯ÔÚËÁÂ›Ù·È ÛÙ· ·È‰È¿ ηÙfiÈÓ ÂÈÏÔÁ‹˜, ÁÈ· ‚ÂÏÙ›ˆÛË ÙfiÛÔ Ù˘ ·‡ÍËÛ˘ fiÛÔ Î·È Ù˘ Û‡ÛÙ·Û˘ ÛÒÌ·ÙÔ˜, Èı·ÓfiÓ ‰Â ÛÙÔ Ì¤ÏÏÔÓ Ó· ÁÂÓÈ΢ı›, ÂÊfiÛÔÓ ‚ÂÏÙÈÒÓÂÈ ÙÔÓ ÌÂÙ·‚ÔÏÈÛÌfi Î·È ÂÏ·ÙÙÒÓÂÈ ÙË ÌÂÏÏÔÓÙÈ΋ ÓÔÛËÚfiÙËÙ·. §¤ÍÂȘ ÎÏÂȉȿ: ™‡Ó‰ÚÔÌÔ Prader-Willi, ·˘ÍËÙÈ΋ ÔÚÌfiÓË, ·‡ÍËÛË, ۈ̷ÙÈ΋ Û‡ÛÙ·ÛË.

Growth hormone treatment in Prader-Willi syndrome Department of Endocrinology, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece Correspondence: Eleni Kousta lkousta@otenet.gr 6, Sp. Arvanitaki St. 49100, Kerkyra Greece

E. Kousta, C. Hadjiathanasiou†, A. Papathanasiou Abstract: Prader-Willi syndrome is a rare genetic disorder caused by the lack of expression of the paternally derived imprinted genes on the chromosome region 15q11-q13. It is characterized by gradual development of hyperphagia, obesity, hypogonadism, short stature and growth hormone (GH) deficiency. Children and adults with the syndrome develop morbid obesity, which is the main cause of the associated increased mortality. Growth retardation develops during the first year of life, and persists through childhood, leading to reduction in the final height. Recent observations suggest that GH replacement therapy improves the growth rate and height deficit in these children. Long term therapy is likely to improve final height. GH therapy also improves body composition by increasing fat free mass, decreasing fat mass and improving resting energy expenditure; however it does not seem to affect the body mass index (BMI) significantly. πn addition, it improves muscle strength and motility in younger children. The commonest side effect of the treatment is scoliosis. The long term consequences of GH treatment in children with Prader-Willi syndrome, particularly regarding carbohydrate metabolism, are still under investigation. Sudden death has been reported in children receiving GH, mainly related to hypoventilation and upper airway infection, raising concerns about the safety of GH treatment and indicating the need for regular and careful monitoring. Considering the multiple problems facing children with Prader-Willi syndrome and their families, short stature does not constitute the main health concern.

Treatment with GH is currently recommended in selected children to improve growth and body composition. It is possible that further studies concerning the effects of GH on metabolism and morbidity may lead to generalization of its use.

Key words: Prader-Willi syndrome, growth hormone, growth, body composition.

∞ÊÈÂڈ̤ÓÔ ÛÙË ÌÓ‹ÌË ÙÔ˘ ÷ڿϷÌÔ˘ ÷Ù˙Ë·ı·Ó·Û›Ô˘

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™˘ÓÙÔÌÔÁڷʛ˜ FHtSDS final height SDS - ÛÎÔÚ ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘ ÙÂÏÈÎÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜ GH growth hormone - ·˘ÍËÙÈ΋ ÔÚÌfiÓË HtSDS height SDS - ÛÎÔÚ ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘ ‡„Ô˘˜ IGF-1 insulin-like growth factor 1 - ÈÓÛÔ˘ÏÈÓfiÌÔÚÊÔ˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁˆÓ 1 SDS standard deviation score - ÛÎÔÚ ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘

EÈÛ·ÁˆÁ‹ ∆Ô Û‡Ó‰ÚÔÌÔ Prader-Willi Â›Ó·È ¤Ó· Û¿ÓÈÔ ÁÂÓÂÙÈÎfi Û‡Ó‰ÚÔÌÔ, Ë Û˘¯ÓfiÙËÙ· ÙÔ˘ ÔÔ›Ô˘ ˘ÔÏÔÁ›˙ÂÙ·È Û 1/10.000 - 1/15.000 ÁÂÓÓ‹ÛÂȘ (1). √Ê›ÏÂÙ·È ÛÙËÓ ·ÒÏÂÈ· Ù˘ ÁÔÓȉȷ΋˜ ¤ÎÊÚ·Û˘ Ù˘ ÂÚÈÔ¯‹˜ 15q11-q13, Ë ÔÔ›· ÂÎÊÚ¿˙ÂÙ·È ÌfiÓÔ ·fi ÙÔ ·ÙÚÈÎfi ÁÔÓ›‰ÈÔ (·ÙÚÈÎfi ·ÏÏËÏfiÌÔÚÊÔ ·ÔÙ˘ˆÌ¤ÓÔ ÁÔÓ›‰ÈÔ). ∆· ·ÓÙ›ÛÙÔȯ· ÁÔÓ›‰È· ÌËÙÚÈ΋˜ ÚÔ¤Ï¢Û˘ Â›Ó·È Ê˘ÛÈÔÏÔÁÈο ·ÓÂÓÂÚÁ¿. ™ÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛÂˆÓ (70-75%), Ë ·ÒÏÂÈ· Ù˘ ÁÔÓȉȷ΋˜ ¤ÎÊÚ·Û˘ ·˘Ù‹˜ Ù˘ ÂÚÈÔ¯‹˜ ÔÊ›ÏÂÙ·È Û ·¿ÏÂÈ„Ë ·˘Ù‹˜ Ù˘ ÁÔÓȉȷ΋˜ ÂÚÈÔ¯‹˜ ·fi ÙÔ ·ÙÚÈÎfi ¯ÚˆÌfiۈ̷ 15, Û 20-25% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÎÏËÚÔÓÔÌÔ‡ÓÙ·È Î·È ÔÈ ‰‡Ô ·˘Ù¤˜ ÁÔÓȉȷΤ˜ ÂÚÈÔ¯¤˜ ÙÔ˘ ¯ÚˆÌÔÛÒÌ·ÙÔ˜ 15 ·fi ÙË ÌËÙ¤Ú· (ÌËÙÚÈ΋ ‰Èۈ̛·), Û 2-5% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ˘¿Ú¯ÂÈ ÌÈÎÚÔ·¿ÏÂÈ„Ë ÂÓfi˜ ÁÂÓÂÙÈÎÔ‡ ÛÙÔÈ¯Â›Ô˘ Ô˘ ÔÓÔÌ¿˙ÂÙ·È Î¤ÓÙÚÔ ·ÔÙ‡ˆÛ˘ (imprinting center) (Û¿ÓÈ· ˘¿Ú¯ÂÈ ‰È·Ù·Ú·¯‹ ÛÙË ÁÔÓȉȷ΋ ·ÔÙ‡ˆÛË ·˘Ù‹˜ Ù˘ ÂÚÈÔ¯‹˜ ¯ˆÚ›˜ Ó· ˘¿Ú¯Ô˘Ó ÁÔÓȉȷΤ˜ ÌÂÙ·ÏÏ¿ÍÂȘ) Î·È Û 1% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ˘¿Ú¯Ô˘Ó ÌÂÙ·ı¤ÛÂȘ Û’ ·˘Ù‹ ÙË ÁÔÓȉȷ΋ ÂÚÈÔ¯‹ (1,2). ™ÙË ÁÔÓȉȷ΋ ÂÚÈÔ¯‹ 15q11-q13 ¤¯Ô˘Ó ÂÓÙÔÈÛÙ› ·ÚÎÂÙÔ› ·ÙÚÈο ÂÎÊÚ·˙fiÌÂÓÔÈ ÁÔÓȉȷÎÔ› ÙfiÔÈ (MKRN3, MAGEL2, NDN Î·È SNURFSNRPN). ™ÙËÓ ·Ú·Î›ÌÂÓË ÛÙÔ ÁÔÓ›‰ÈÔ SNURFSNRPN ÂÚÈÔ¯‹ ÂÓÙÔ›˙ÔÓÙ·È ÔÏÏ·Ï¿ ÂÛÒÓÈ·, Ù· ÔÔ›· ÂÚȤ¯Ô˘Ó ÁÔÓ›‰È· ÁÈ· ÌË Îˆ‰ÈÎÔÔÈÔ‡ÓÙ· RNA (C/D box snoRNA - ÌÈÎÚfi RNA ˘ÚËÓ›ÛÎÔ˘ Ù‡Ô˘ C/D box). Œˆ˜ ÙÒÚ·, ‰ÂÓ Â›¯Â ·Ô‰Âȯı› ÔÈÔ ‹ ÔÈ· ·fi Ù· ·ÙÚÈο ÂÎÊÚ·˙fiÌÂÓ· ÁÔÓ›‰È· ·˘Ù‹˜ Ù˘ ÂÚÈÔ¯‹˜ Û˘Ó‰¤ÔÓÙ·È Ì ÙË ÓfiÛÔ Î·È Ì ÔÈÔ Ì˯·ÓÈÛÌfi. ™Â ÚfiÛÊ·ÙË ÌÂϤÙË ÂÓÙÔ›ÛÙËΠÌÈÎÚÔ·¿ÏÂÈ„Ë Ù˘ ÁÔÓȉȷ΋˜ ÂÚÈÔ¯‹˜ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ HBII-85snoRNA Û ¤Ó· ·ÁfiÚÈ Ì ÙÔ Û‡Ó‰ÚÔÌÔ Î·È Â›Ó·È Èı·ÓfiÓ Ë ·¿ÏÂÈ„Ë Ù˘ ÂÚÈÔ¯‹˜ ·˘Ù‹˜ Ó· Û˘Ì‚¿ÏÏÂÈ ÛËÌ·ÓÙÈο ÛÙÔÓ Ê·ÈÓfiÙ˘Ô ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ (3). ∆· Û˘ÁÎÂÎÚÈ̤ӷ ÁÔÓ›‰È· ÙˆÓ snoRNAs ÂÎÊÚ¿˙ÔÓÙ·È Î˘Ú›ˆ˜ ÛÙÔÓ ÂÁΤʷÏÔ, fï˜ Ô ·ÎÚÈ‚‹˜ Ì˯·ÓÈÛÌfi˜ ‰Ú¿Û˘ ÙÔ˘˜ ‰ÂÓ Â›Ó·È ·ÎfiÌË ÁÓˆÛÙfi˜. √È ÏËÚÔÊÔڛ˜ ·˘Ù¤˜ Èı·ÓfiÓ Ó· Ô‰ËÁ‹ÛÔ˘Ó Û ηχÙÂÚË Î·Ù·ÓfiËÛË Ù˘ ·ıÔÁ¤ÓÂÈ·˜ Ù˘ ÓfiÛÔ˘ Î·È ›Ûˆ˜ Û Ӥ˜ ıÂÚ·¢ÙÈΤ˜ ‰˘Ó·ÙfiÙËÙ˜. ∆Ô Û‡Ó‰ÚÔÌÔ Prader-Willi Â›Ó·È ÌÈ· ÔÏ˘Û˘ÛÙË-

Ì·ÙÈ΋ ÓfiÛÔ˜ Ë ÔÔ›· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ˘ÂÚÊ·Á›·, ·¯˘Û·ÚΛ·, ˘ÔÁÔÓ·‰ÈÛÌfi, ¯·ÌËÏfi ·Ó¿ÛÙËÌ· Î·È ¤ÏÏÂÈ„Ë ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘ (GH) ÔÊÂÈÏfiÌÂÓË Û ˘Ôı·Ï·ÌÈ΋ ‰È·Ù·Ú·¯‹ (2,5). O ‚·ıÌfi˜ Ù˘ ¤ÏÏÂȄ˘ GH ÔÈΛÏÏÂÈ ·fi ̤ÙÚÈ· ¤ˆ˜ Ï‹ÚË ·ÓÂ¿ÚÎÂÈ· Î·È Û˘Ó‹ıˆ˜ Ù· Â›‰· IGF-1 (ÈÓÛÔ˘ÏÈÓfiÌÔÚÊÔ˜ ·˘ÍËÙÈÎfi˜ ·Ú¿ÁˆÓ 1) Â›Ó·È ¯·ÌËÏ¿, ·Ú¿ ÙËÓ ·¯˘Û·ÚΛ·. ¶ÔÏÏ¿ ·fi Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ÁÓˆÚ›ÛÌ·Ù· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘, fiˆ˜ Ë ˘ÂÚÊ·Á›·, Ë ˘ÓËÏ›·, Ô ·˘ÙÔÙÚ·˘Ì·ÙÈÛÌfi˜ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ Î·È Ô ˘ÔÁÔÓ·‰ÈÛÌfi˜, ÌÔÚÔ‡Ó Ó· ·Ô‰ÔıÔ‡Ó Û ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ˘Ôı·Ï¿ÌÔ˘. ∏ ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ˘ÔÙÔÓ›· Î·È ·‰˘Ó·Ì›· ÚÔηÏÔ‡Ó Ì›ˆÛË ÙˆÓ ÎÈÓ‹ÛÂˆÓ ÙÔ˘ ÂÌ‚Ú‡Ô˘, ‰˘ÛÎÔϛ˜ Û›ÙÈÛ˘ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, ·‰‡Ó·ÌÔ ÎÏ¿Ì· ‹ ·Ô˘Û›· ÎÏ¿Ì·ÙÔ˜ Î·È ÓˆıÚfiÙËÙ· ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ‚ÚÂÊÈ΋˜ ËÏÈΛ·˜, Ì ηı˘ÛÙ¤ÚËÛË ·Ó¿Ù˘Í˘ Î·È ·ÓÂ·Ú΋ ÚfiÛÏË„Ë ‚¿ÚÔ˘˜ (4,6). √ Ì˘˚Îfi˜ ÙfiÓÔ˜ Î·È ÔÈ ÎÈÓ‹ÛÂȘ ‚ÂÏÙÈÒÓÔÓÙ·È ÚÔ˜ ÙÔ Ù¤ÏÔ˜ ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ Ù˘ ˙ˆ‹˜ ηÈ, ·Ó Î·È Î·ı˘ÛÙÂÚË̤ӷ, ÂÈÙ˘Á¯¿ÓÔÓÙ·È Ù· ·Ó·Ù˘Íȷο ÔÚfiÛËÌ·. ∫·Ù¿ ÙÔ 2Ô ‹ 3Ô ¤ÙÔ˜ Ù˘ ˙ˆ‹˜ ÍÂÎÈÓ¿ Ë Ê¿ÛË Ù˘ ˘ÂÚÊ·Á›·˜, Ë ÔÔ›·, ·Ó ‰ÂÓ ÂÏÂÁ¯ı›, ÂÍÂÏ›ÛÛÂÙ·È Û ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ·¯˘Û·ÚΛ·. ∆· ·È‰È¿ ÂÌÊ·Ó›˙Ô˘Ó ¯·Ú·ÎÙËÚÈÛÙÈ΋ ÂÌÌÔÓ‹ Î·È ÎÚ›ÛÂȘ ı˘ÌÔ‡, ÛÙËÓ ÚÔÛ¿ıÂÈ· ÙˆÓ ÁÔÓ¤ˆÓ Ó· ÂϤÁÍÔ˘Ó Î·È Ó· ÂÚÈÔÚ›ÛÔ˘Ó ÙÔ Ê·ÁËÙfi (4,6). √ ˘ÔÁÔÓ·‰ÈÛÌfi˜ ÂΉËÏÒÓÂÙ·È Ì ηı˘ÛÙ¤ÚËÛË ÙˆÓ ÛËÌ›ˆÓ Ù˘ ‹‚˘ ÛÙ· ÎÔÚ›ÙÛÈ·, Û·ÓÈfiÙÂÚ· ‰Â ÌÔÚ› Ó· ˘¿Ú¯ÂÈ ·Ú·ÈÔÌËÓfiÚÚÔÈ· ‹ ·ÌËÓfiÚÚÔÈ·. ∆· ·ÁfiÚÈ· ·ÚÔ˘ÛÈ¿˙Ô˘Ó Û˘Ó‹ıˆ˜ ÎÚ˘„ÔÚ¯›· ηٿ ÙË Á¤ÓÓËÛË Î·È, ·Ú¿ ÙËÓ ÂÈÙ˘¯‹ ÔÚ¯ÂÔËÍ›·, ¯ÚÂÈ¿˙ÔÓÙ·È ıÂÚ·›· Ì ·Ó‰ÚÔÁfiÓ· ÁÈ· ÙËÓ ¤Ó·ÚÍË Î·È ÚfiÔ‰Ô Ù˘ ‹‚˘. √È ÂÓ‹ÏÈΘ Ì ÙÔ Û‡Ó‰ÚÔÌÔ ·Ó·Ù‡ÛÛÔ˘Ó Û˘Ó‹ıˆ˜ ÓÔÛÔÁfiÓÔ ·¯˘Û·ÚΛ·, Ë ÔÔ›· Â›Ó·È Ë Î‡ÚÈ· ·ÈÙ›· Ù˘ ·˘ÍË̤Ó˘ ıÓËÛÈÌfiÙËÙ·˜. ∏ ·¯˘Û·ÚΛ· Ô‰ËÁ› Û ۷ί·ÚÒ‰Ë ‰È·‚‹ÙË Ù‡Ô˘ II, ηډȷÁÁÂȷ΋ ÓfiÛÔ, ˘¤ÚÙ·ÛË, ˘ÂÚÏÈȉ·ÈÌ›·, ıÚÔÌ‚ÔÊÏ‚›Ùȉ·, ¯ÚfiÓÈÔ Ô›‰ËÌ· οو ¿ÎÚˆÓ, ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ‡ÓÔ˘, ‰È·Ù·Ú·¯¤˜ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È „˘¯ÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜, fiˆ˜ ηٿıÏÈ„Ë Î·È ÂÏ·Ùو̤ÓË ·˘ÙÔÂÎÙ›ÌËÛË (5). ¶ÂÚ›Ô˘ 80% ÙˆÓ ·ÛıÂÓÒÓ ¤¯Ô˘Ó ÛÎÔÏ›ˆÛË. ∂›Û˘, ÂÌÊ·Ó›˙Ô˘Ó Û˘¯Ó¿ ÔÛÙÂÔfiÚˆÛË, Èı·ÓfiÓ ÏfiÁˆ ÙÔ˘ ˘ÔÁÔÓ·‰ÈÛÌÔ‡ Î·È Ù˘ ¤ÏÏÂȄ˘ ·˘ÍËÙÈ΋˜ ÔÚÌfiÓ˘.

∏ ·‡ÍËÛË ÛÙ· ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi ∂ÓÒ ÙÔ ‡„Ô˜ ηٿ ÙË Á¤ÓÓËÛË Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi ‹ ˘ÔÏ›ÂÙ·È ÂÏ¿¯ÈÛÙ·, Ô Ú˘ıÌfi˜ ·‡ÍËÛ˘ ηı˘ÛÙÂÚ› ηٿ ÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, Èı·ÓfiÓ ÏfiÁˆ ÌÂȈ̤Ó˘ ÚfiÛÏ˄˘ ıÂÚÌ›‰ˆÓ. ∆Ô ·Ó¿ÛÙËÌ· ·Ú·Ì¤ÓÂÈ ¯·ÌËÏfi [ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË ‡„Ô˘˜ (∏tSDS) ~ -2] ¶·È‰È·ÙÚÈ΋ 2009;72:10-15

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·12

12

E. ∫Ô‡ÛÙ· Î·È Û˘Ó.

ηı’ fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜, ·Ú¿ ÙÔÓ Û¯ÂÙÈο Ê˘ÛÈÔÏÔÁÈÎfi Ú˘ıÌfi ·‡ÍËÛ˘ (7,8). ™ËÌÂȈ٤ÔÓ fiÙÈ ‰ÂÓ ·Ú·ÙËÚÂ›Ù·È ÂÈÙ¿¯˘ÓÛË ÙÔ˘ Ú˘ıÌÔ‡ ·‡ÍËÛ˘, ·Ú¿ ÙËÓ ˘„ËÏ‹ ÂÓÂÚÁÂȷ΋ ÚfiÛÏË„Ë, Û ·ÓÙ›ıÂÛË Ì fi,ÙÈ Û˘Ì‚·›ÓÂÈ ÛÙËÓ ·Ï‹ ·¯˘Û·ÚΛ·. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÊ˂›·˜, Ô Ú˘ıÌfi˜ ·‡ÍËÛ˘ ÂÏ·ÙÙÒÓÂÙ·È, ÏfiÁˆ ·Ô˘Û›·˜ ÙÔ˘ ÂÊË‚ÈÎÔ‡ ¿ÏÌ·ÙÔ˜ ‡„Ô˘˜ (7,8,9). °È’ ·˘Ùfi, ÛÙ· ¿ÙÔÌ· Ì ۇӉÚÔÌÔ Prader-Willi Ë ÂηÙÔÛÙÈ·›· ı¤ÛË ÙÔ˘ ‡„Ô˘˜ ηٿ ÙËÓ ÂÓ‹ÏÈÎÔ ˙ˆ‹ Â›Ó·È ¯·ÌËÏfiÙÂÚË ·fi ÙËÓ ÂηÙÔÛÙÈ·›· ı¤ÛË ÙÔ˘ ‡„Ô˘˜ ηٿ ÙËÓ ·È‰È΋ ËÏÈΛ·. ∆Ô Ì¤ÛÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ Ô˘ ηٷÁÚ¿ÊËΠÛÙÔ ∏ӈ̤ÓÔ µ·Û›ÏÂÈÔ Â›Ó·È 147 cm (-2,8 SD) ÁÈ· Ù· ÎÔÚ›ÙÛÈ· Î·È 155 cm (-3,2 SD) ÁÈ· Ù· ·ÁfiÚÈ· (7).

™ˆÌ·ÙÈ΋ Û‡ÛÙ·ÛË ™ÙËÓ ·Ï‹ ·¯˘Û·ÚΛ·, Ë ÏÈ҉˘ Ì¿˙· Â›Ó·È ·˘ÍË̤ÓË, ÂÓÒ Ë ¿ÏÈÔ˜ Ì¿˙· Ê˘ÛÈÔÏÔÁÈ΋ ‹ ·˘ÍË̤ÓË. ™ÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi ·Ú·ÙËÚÂ›Ù·È ÂÏ·Ùو̤ÓË ¿ÏÈÔ˜ Î·È ·˘ÍË̤ÓË ÏÈ҉˘ Ì¿˙·, ·ÎfiÌ· Î·È ÛÙ· ÏÂÙ¿ ¿ÙÔÌ· (10,11). ∂ÈϤÔÓ, Ë ÔÛÙÈ΋ ˘ÎÓfiÙËÙ· Â›Ó·È ÂÏ·Ùو̤ÓË Û ۇÁÎÚÈÛË Ì ÙËÓ ÎÔÈÓ‹ ·¯˘Û·ÚΛ· (10). ∞ÎfiÌ· Î·È ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, ηٿ ÙËÓ ÔÔ›· Ë ÛˆÌ·ÙÈ΋ Ì¿˙· ÛÙ· ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi Â›Ó·È ÂÏ·Ùو̤ÓË, Û ۯ¤ÛË Ì ٷ Ê˘ÛÈÔÏÔÁÈο ·È‰È¿, Ë ·Ó·ÏÔÁ›· ÙÔ˘ Ï›Ô˘˜ Â›Ó·È Û¯ÂÙÈο ·˘ÍË̤ÓË (12). ∏ ÂÏ·Ùو̤ÓË ¿ÏÈÔ˜ Ì¿˙· ÌÔÚ› Ó· ·ÓÙ·Ó·ÎÏ¿ ÂÏ·Ùو̤ÓË Ì˘˚΋ Ì¿˙·, Î·È ¤ÙÛÈ Ó· ÂÍËÁÂ›Ù·È ÂÓ Ì¤ÚÂÈ Î·È Ë ˘ÔÙÔÓ›· (5). √È ÂÓÂÚÁÂȷΤ˜ ‰·¿Ó˜ Â›Ó·È ÌÂȈ̤Ó˜, Èı·ÓfiÓ ÏfiÁˆ Ù˘ ÌÂȈ̤Ó˘ ·Ï›Ô˘ Ì¿˙·˜. °È· ÙÔ ÏfiÁÔ ·˘Ùfi, ÛÙ· ¿ÙÔÌ· Ì ۇӉÚÔÌÔ Prader-Willi ÔÈ ÂÓÂÚÁÂȷΤ˜ ·Ó¿ÁΘ ·Ó¿ ÌÔÓ¿‰· ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜ Â›Ó·È ¯·ÌËÏfiÙÂÚ˜, Û ۯ¤ÛË Ì ٷ Ê˘ÛÈÔÏÔÁÈο ¿ÙÔÌ·, ÁÂÁÔÓfi˜ ÙÔ ÔÔ›Ô Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÂÙ·È ˘fi„Ë fiÙ·Ó ˘ÔÏÔÁ›˙ÂÙ·È Ë È‰·ÓÈ΋ ËÌÂÚ‹ÛÈ· ÚfiÛÏË„Ë ÙÚÔÊ‹˜ (13). £ÂÚ·›· Ì ·˘ÍËÙÈ΋ ÔÚÌfiÓË (GH) ∏ Â›‰Ú·ÛË Ù˘ ıÂÚ·›·˜ Ì GH ÛÙËÓ ·‡ÍËÛË ∏ ·ÓÂ·Ú΋˜ ¤ÎÎÚÈÛË GH Î·È Ù· ¯·ÌËÏ¿ Â›‰· IGF-1 ÛÙ· ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ·, Ô‰‹ÁËÛ·Ó ÛÙË ıÂÚ·›· ÙˆÓ ·È‰ÈÒÓ Ì GH. ª¤¯ÚÈ Û‹ÌÂÚ·, ÂÚÈÛÛfiÙÂÚ· ·fi 300 ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi ·ÁÎÔÛÌ›ˆ˜ ¤¯Ô˘Ó ¿ÚÂÈ ıÂÚ·›· Ì GH ÁÈ· ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ·fi 6 Ì‹Ó˜ ¤ˆ˜ >5 ¯ÚfiÓÈ· (5,14). √È ·Ú¯ÈΤ˜ ÌÂϤÙ˜ ıÂÚ·›·˜ ¤‰ÂÈÍ·Ó ÛËÌ·ÓÙÈ΋ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· Ì GH ηٿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ıÂÚ·›·˜ Î·È ‚ÂÏÙ›ˆÛË Ù˘ Ù·¯‡ÙËÙ·˜ ·‡ÍËÛ˘ (15,16,17), ¯ˆÚ›˜ ÂÈÙ¿¯˘ÓÛË Ù˘ ÔÛÙÈ΋˜ ËÏÈΛ·˜ (16). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‰Â‡ÙÂÚÔ˘ ¤ÙÔ˘˜ ıÂÚ·›·˜ Û 35 ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi, ̤Û˘ ËÏÈPaediatriki 2009;72:10-15

Λ·˜ 9,9 ÂÙÒÓ (‡ÚÔ˜ 4-16 ¤ÙË), ·Ú·ÙËÚ‹ıËΠÌÈÎÚ‹ ÙÒÛË ÙÔ˘ Ú˘ıÌÔ‡ ·‡ÍËÛ˘ (6,8 cm/¤ÙÔ˜) Û ۇÁÎÚÈÛË Ì ÙÔÓ ÚÔËÁÔ‡ÌÂÓÔ ¯ÚfiÓÔ (10,1 cm/¤ÙÔ˜), Ô Ú˘ıÌfi˜ fï˜ ·Ú¤ÌÂÈÓ ˘„ËÏfiÙÂÚÔ˜ Û ۯ¤ÛË Ì ÙÔÓ Ú˘ıÌfi ÚÈÓ ÙËÓ ¤Ó·ÚÍË ıÂÚ·›·˜ (4,7 cm/¤ÙÔ˜) (18). ∏ ‰fiÛË Ù˘ GH ‹Ù·Ó 1 mg/m2/24ˆÚÔ (21 IU/m2/‚‰.) Î·È Ë Û˘ÓÔÏÈ΋ ·‡ÍËÛË Ù˘ HtSDS ‹Ù·Ó +0,8 ÌÂÙ¿ ·fi 2 ¯ÚfiÓÈ· ıÂÚ·›·˜ (18). ¶·ÚfiÌÔÈ· Â˘Ú‹Ì·Ù· ·Ó·Ê¤ÚÔÓÙ·È Î·È Û ÂfiÌÂÓË ‰ÈÏ‹ Ù˘ÊÏ‹ ÌÂϤÙË 14 ·ÛıÂÓÒÓ Ô˘ ‹Ú·Ó ıÂÚ·›· Ì GH (Û ‰fiÛË 0,3-0,6 IU/kg/‚‰.) ÁÈ· 3,6 ¯ÚfiÓÈ· (‡ÚÔ˜ 1-9,3 ¤ÙË) Î·È Û˘ÁÎÚ›ıËÎ·Ó Ì 14 ·ÛıÂÓ›˜ Ô˘ ‰ÂÓ Â›¯·Ó Ï¿‚ÂÈ ıÂÚ·›· (19). ™ÙËÓ ÔÌ¿‰· ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ¤Ï·‚·Ó ıÂÚ·›·, Ô Ú˘ıÌfi˜ ·‡ÍËÛ˘ ·ÚÔ˘Û›·Û ÙË Ì¤ÁÈÛÙË ·‡ÍËÛË ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÚÒÙÔ˘ ¤ÙÔ˘˜ Î·È ·Ú¤ÌÂÈÓ ˘„ËÏfiÙÂÚÔ˜ ηı’ fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ¯ÔÚ‹ÁËÛ˘, Û˘ÁÎÚÈÙÈο Ì ÙÔÓ ÚÔ Ù˘ ıÂÚ·›·˜ Ú˘ıÌfi (19). ™Â ¿ÏÏË ÌÂϤÙË 18 ÚÔÂÊË‚ÈÎÒÓ ·ÛıÂÓÒÓ ËÏÈΛ·˜ 0,3-9,5 ÂÙÒÓ Ô˘ ¤Ï·‚·Ó ıÂÚ·›· GH (Û ‰fiÛË 18 IU/m2/‚‰. ÁÈ· ·È‰È¿ <2 ÂÙÒÓ Î·È 24 IU/m2/ ‚‰. ÁÈ· ·È‰È¿ >2 ÂÙÒÓ) ÁÈ· ‰È¿ÛÙËÌ· 4 ÂÚ›Ô˘ ÂÙÒÓ, ·Ó·Ê¤ÚÂÙ·È Ì¤ÛË ·‡ÍËÛË Ù˘ HtSDS ηٿ 1,8 (20). ªÂÙ¿ ·fi Ù· ÚÒÙ· ¯ÚfiÓÈ· ıÂÚ·›·˜, fiˆ˜ Î·È ÛÙȘ ÚÔËÁÔ‡ÌÂÓ˜ ÌÂϤÙ˜ ·Ú·ÙËÚ‹ıËΠۯÂÙÈ΋ ÂÈ‚Ú¿‰˘ÓÛË ÙÔ˘ Ú˘ıÌÔ‡ ·‡ÍËÛ˘. ∏ ÔÛÙÈ΋ ËÏÈΛ· ‰ÂÓ ÂÈÙ·¯‡ÓÂÙ·È Î·Ù¿ ÙË ıÂÚ·›· Ì GH (21). ªÂÙ¿ ·fi ¤ÓÙ ¯ÚfiÓÈ· ıÂÚ·›·˜ Ì GH (0,1 ‹ 0,2 IU/kg/24ˆÚÔ) Û ·È‰È¿ 3-12 ÂÙÒÓ, Ë HtSDS ·Ú¤ÌÂÈÓ ¿Óˆ ·fi ÙÔ Ì¤ÛÔ fiÚÔ ÁÈ· ÙË ¯ÚÔÓÔÏÔÁÈ΋ ËÏÈΛ· (22). ™ÙËÓ ›‰È· ÌÂϤÙË ·Ó·Ê¤ÚÂÙ·È fiÙÈ Ë HtSDS ·˘Í‹ıËΠÛËÌ·ÓÙÈο ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ Ù˘ ıÂÚ·›·˜ Î·È Ë ÂηÙÔÛÙÈ·›· ı¤ÛË Ô˘ ›¯Â ÂÈÙ¢¯ı› ‰È·ÙËÚ‹ıËΠηٿ Ù· ÂfiÌÂÓ· ¯ÚfiÓÈ· Ù˘ ıÂÚ·›·˜. H ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· Â›Ó·È ÁÂÓÈο ηχÙÂÚË ÛÙ· ·È‰È¿ ÚÔÂÊË‚È΋˜ ËÏÈΛ·˜, ·Ú¿ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜, fiˆ˜ Û˘Ì‚·›ÓÂÈ Î·È Ì ÙËÓ ÎÏ·ÛÈ΋ ¤ÏÏÂÈ„Ë (14). ŸÛÔÓ ·ÊÔÚ¿ ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔ Prader-Willi Ô˘ ‹Ú·Ó ıÂÚ·›· Ì GH, ·Ú¯Èο ·Ó·Ê¤ÚıËÎ·Ó ·ÔÙÂϤÛÌ·Ù· ÙÂÏÈÎÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜ ÁÈ· 4 ÌfiÓÔ ·È‰È¿, Ô˘ ‹Ú·Ó ıÂÚ·›· ÁÈ· 5 ¯ÚfiÓÈ·. ∏ SDS ÙÔ˘ ÙÂÏÈÎÔ‡ ·Ó·ÛÙ‹Ì·ÙÔ˜ (FHtSDS) Î˘Ì¿ÓıËΠ۠fiÚÈ· ±2SD, Û˘ÁÎÚÈÙÈο Ì ÙÔ ‡„Ô˜-ÛÙfi¯Ô (‡ÚÔ˜ -1,1 ¤ˆ˜ +0,9 SDS) (22). ¶ÚfiÛÊ·Ù· ·Ó·Ê¤ÚıËÎ·Ó Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ıÂÚ·›·˜ Ì GH (Û ‰fiÛË 0,25±0,06 mg/kg/‚‰ÔÌ¿‰·) ÁÈ· 21 ·È‰È¿ ËÏÈΛ·˜ 8,3±2,7 ÂÙÒÓ Ì ۇӉÚÔÌÔ PraderWilli Î·È ∏tSDS -1,9±1,7, Ù· ÔÔ›· ‹Ú·Ó ıÂÚ·›· ÁÈ· 7,9±1,7 ¯ÚfiÓÈ·, Û ۇÁÎÚÈÛË Ì 39 ÂÓ‹ÏÈΘ ·ÛıÂÓ›˜ Ì ÙÔ Û‡Ó‰ÚÔÌÔ ÔÈ ÔÔ›ÔÈ ‰ÂÓ ‹Ú·Ó ıÂÚ·›· Ì GH Î·È ÔÈ ÔÔ›ÔÈ Â›¯·Ó ·Ó¿ÏÔÁË ∏tSDS ÛÙËÓ ËÏÈΛ· ÙˆÓ 6,8±1,3 ÂÙÒÓ (23). ∏ FHtSDS ‹Ù·Ó ÛËÌ·ÓÙÈο

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·13

13

∞˘ÍËÙÈ΋ ÔÚÌfiÓË ÛÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi

‚ÂÏÙȈ̤ÓË (-0,3±1,2) ÛÙ· ·È‰È¿ Ô˘ ‹Ú·Ó ÙË ıÂÚ·›·, Û ۇÁÎÚÈÛË Ì ÙËÓ SDS ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ‰ÂÓ ‹Ú·Ó ıÂÚ·›· (-3,1±1,0) (23). ™˘ÓÂÒ˜, Ë ¯ÔÚ‹ÁËÛË GH ÁÈ· 1-3 ¯ÚfiÓÈ· ‚ÂÏÙÈÒÓÂÈ ÙÔ ‡„Ô˜ Î·È ÙÔÓ Ú˘ıÌfi ·‡ÍËÛ˘, ¯ˆÚ›˜ ȉȷ›ÙÂÚË ÂÈÙ¿¯˘ÓÛË Ù˘ ÔÛÙÈ΋˜ ËÏÈΛ·˜, ÛÙ· ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi. ¶·ÚfiÏÔ Ô˘ Ô ·ÚÈıÌfi˜ ÙˆÓ ·È‰ÈÒÓ Ì ıÂÚ·›· Ô˘ ¤Êı·Û·Ó ÙÂÏÈÎfi ·Ó¿ÛÙËÌ· Â›Ó·È ÂÚÈÔÚÈṲ̂ÓÔ˜, Ì ٷ ˘¿Ú¯ÔÓÙ· ‰Â‰Ô̤ӷ Ê·›ÓÂÙ·È fiÙÈ Ë Â˘ÂÚÁÂÙÈ΋ Â›‰Ú·ÛË Ù˘ ıÂÚ·›·˜ ‰È·ÙËÚÂ›Ù·È Ì¤¯ÚÈ ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ·. ∏ Â›‰Ú·ÛË Ù˘ ıÂÚ·›·˜ Ì GH ÛÙË ÛˆÌ·ÙÈ΋ Û‡ÛÙ·ÛË ·ÙfiÌˆÓ Ì ۇӉÚÔÌÔ Prader-Willi √È ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë Â›‰Ú·ÛË Ù˘ GH ÛÙË ÛˆÌ·ÙÈ΋ Û‡ÛÙ·ÛË Â›Ó·È Â˘ÂÚÁÂÙÈ΋: ·˘Í¿ÓÂÙ·È Ë ¿ÏÈÔ˜ Ì¿˙· Î·È ÌÂÈÒÓÂÙ·È Ô ÏÈ҉˘ ÈÛÙfi˜ (5,14). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ ıÂÚ·›·˜, ·Ó·Ê¤ÚıËΠÛËÌ·ÓÙÈ΋ ÂÏ¿ÙÙˆÛË Ù˘ ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘ ÙÔ˘ ‰Â›ÎÙË Ì¿˙·˜ ÛÒÌ·ÙÔ˜ (µªπ SDS), ·‡ÍËÛË Î·Ù¿ ÙÔ˘˜ ÂfiÌÂÓÔ˘˜ 6 Ì‹Ó˜ ¯ˆÚ›˜ ıÂÚ·›· Ì GH Î·È ÛÙ·ıÂÚÔÔ›ËÛË ÌÂÙ¿ ÙËÓ Â·Ó¤Ó·ÚÍË Ù˘ ¯ÔÚ‹ÁËÛ˘ (22). ™Â 35 ·ÛıÂÓ›˜ Ì ۇӉÚÔÌÔ Prader-Willi, ·Ú·ÙËÚ‹ıËΠÛËÌ·ÓÙÈ΋ Ì›ˆÛË ÙÔ˘ ۈ̷ÙÈÎÔ‡ Ï›Ô˘˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÚÒÙÔ˘ ¯ÚfiÓÔ˘ Ù˘ ıÂÚ·›·˜, ¯ˆÚ›˜ fï˜ Ó· ·Ú·ÙËÚËı› ÂÚ·ÈÙ¤Úˆ Ì›ˆÛË ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‰Â‡ÙÂÚÔ˘ ¯ÚfiÓÔ˘, ÂÓÒ Ë ¿ÏÈÔ˜ Ì¿˙· ·ÚÔ˘Û›·Û ÛÙ·ıÂÚ‹ ·‡ÍËÛË Î·È ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‰Â‡ÙÂÚÔ˘ ¯ÚfiÓÔ˘ (18). ŸÌˆ˜, ÌÂÙ¿ ·fi 2 ¯ÚfiÓÈ· ıÂÚ·›·˜ Û ÌÂÁ¿ÏÔ ·ÚÈıÌfi ·ÛıÂÓÒÓ, ·Ú¿ ÙË Ì›ˆÛË ÙÔ˘ ÏÈÒ‰Ô˘˜ ÈÛÙÔ‡, ‰ÂÓ ˘¿Ú¯Ô˘Ó ÛËÌ·ÓÙÈΤ˜ ÌÂÙ·‚ÔϤ˜ ÙÔ˘ BMI (14). √È ‚·ÛÈΤ˜ ÂÓÂÚÁÂȷΤ˜ ‰·¿Ó˜, Ô˘ ‹Ù·Ó ÂÏ·Ùو̤Ó˜ ÚÔ ıÂÚ·›·˜ Û˘ÁÎÚÈÙÈο ÌÂ Ê˘ÛÈÔÏÔÁÈο ·È‰È¿ Ì ÙËÓ ›‰È· ÂÈÊ¿ÓÂÈ· ÛÒÌ·ÙÔ˜, ·˘Í‹ıËÎ·Ó ÌÂÙ¿ ·fi ‰‡Ô ¯ÚfiÓÈ· ¯ÔÚ‹ÁËÛ˘ GH (18). ∏ Ì˘˚΋ ÈÛ¯‡˜ Î·È Ë ÎÈÓËÙÈÎfiÙËÙ· ·ÚÔ˘Û›·Û·Ó Â›Û˘ ·‡ÍËÛË ÌÂÙ¿ ·fi ¤Ó· ¯ÚfiÓÔ ·ÁˆÁ‹˜, ÂÓÒ Ë ‚ÂÏÙ›ˆÛË ·˘Ù‹ ‰È·ÙËÚ‹ıËÎÂ Î·È ÛÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‰Â‡ÙÂÚÔ˘ ¯ÚfiÓÔ˘ (18). ∂›Ó·È ÛËÌ·ÓÙÈÎfi fiÙÈ ÛÙËÓ ›‰È· ÌÂϤÙË ÔÈ ÁÔÓ›˜ ·Ó¤ÊÂÚ·Ó ·‡ÍËÛË Ù˘ Ê˘ÛÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÙˆÓ ·È‰ÈÒÓ, Ù˘ ·ÓÙÔ¯‹˜ ÛÙËÓ ¿ÛÎËÛË Î·È Ù˘ ·fiÎÙËÛ˘ Ó¤ˆÓ ·‰ÚÒÓ ÎÈÓËÙÈÎÒÓ ‰ÂÍÈÔÙ‹ÙˆÓ (18). ∏ ‚ÂÏÙ›ˆÛË Ù˘ ۈ̷ÙÈ΋˜ Û‡ÛÙ·Û˘ Î·È ÙˆÓ ‚·ÛÈÎÒÓ ÂÓÂÚÁÂÈ·ÎÒÓ ‰··ÓÒÓ ÙÂÎÌËÚÈÒıËÎÂ Î·È Û ÂfiÌÂÓË ÌÂϤÙË (24). ¶ÚfiÛÊ·Ù·, Ë GH ¯ÔÚËÁ‹ıËÎÂ Î·È Û ÂÓ‹ÏÈΘ ·ÛıÂÓ›˜ Ì ÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi, Ì ¢ÓÔ˚ο ·ÔÙÂϤÛÌ·Ù· ˆ˜ ÚÔ˜ ÙË Û‡ÛÙ·ÛË ÛÒÌ·ÙÔ˜ ¯ˆÚ›˜ ÛËÌ·ÓÙÈΤ˜ ·ÚÂÓ¤ÚÁÂȘ (25,26,27). √ µªπ Î·È Ô ÏfiÁÔ˜ ̤Û˘ ÚÔ˜ ÁÔÊÔ‡˜ (W/∏) ‰ÂÓ ¿ÏÏ·Í ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜. H ¿ÏÈÔ˜ Ì¿˙· ·˘Í‹ıËÎÂ, Ô ÏÈ҉˘ ÈÛÙfi˜ ÌÂÈÒıËΠ(26,27) Î·È Ë ‚ÂÏÙ›ˆ-

ÛË Ù˘ ۈ̷ÙÈ΋˜ Û‡ÛÙ·Û˘ Û˘Ó¯›ÛÙËÎÂ, ÂÎÙfi˜ ·fi ÙÔÓ 1Ô ¯ÚfiÓÔ ıÂÚ·›·˜, Î·È Î·Ù¿ Ù· ÂfiÌÂÓ· 4 ¯ÚfiÓÈ· Ù˘ ıÂÚ·›·˜ (26). ∞ÎfiÌË, Ì ÙË ¯ÔÚ‹ÁËÛË Ù˘ GH ·ÔηٷÛÙ¿ıËÎ·Ó ÔÈ ‹Ș ‰È·Ù·Ú·¯¤˜ Ù˘ ı˘ÚÂÔÂȉÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (¯·ÌËÏ¿ Â›‰· T3) Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó Û ÔÚÈṲ̂ÓÔ˘˜ ·ÛıÂÓ›˜ Ì ÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi (27). ∏ ·ÔηٿÛÙ·ÛË Ù˘ ı˘ÚÂÔÂȉÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ ·˘ÙÔ‡˜ Â›Ó·È Èı·ÓfiÓ Ó· Û˘Ì‚¿ÏÏÂÈ ÛÙË ‚ÂÏÙ›ˆÛË Ù˘ ۈ̷ÙÈ΋˜ Û‡ÛÙ·Û˘ (27). ™˘ÓÔ„›˙ÔÓÙ·˜, Ë ıÂÚ·›· Ì GH ÛÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi ‚ÂÏÙÈÒÓÂÈ ÙË ÛˆÌ·ÙÈ΋ Û‡ÛÙ·ÛË, ·˘Í¿ÓÔÓÙ·˜ ÙËÓ ¿ÏÈÔ Ì¿˙·, ÌÂÈÒÓÔÓÙ·˜ ÙÔÓ ÏÈÒ‰Ë ÈÛÙfi Î·È ‚ÂÏÙÈÒÓÔÓÙ·˜ ÙȘ ÂÓÂÚÁÂȷΤ˜ ‰·¿Ó˜, ¯ˆÚ›˜ Ó· Âȉڿ ÛËÌ·ÓÙÈο ÛÙÔÓ BMI. ∏ ıÂÚ·›· Ì GH ·ÔÎÏÂÈÛÙÈο Ê·›ÓÂÙ·È fiÙÈ ‰ÂÓ ÌÔÚ› Ó· Â·Ó·Ê¤ÚÂÈ ÙË ÛˆÌ·ÙÈ΋ Û‡ÛÙ·ÛË ÛÙ· Ê˘ÛÈÔÏÔÁÈο Â›‰·. ÕÏϘ ÂȉڿÛÂȘ Ù˘ ıÂÚ·›·˜ Ì GH AӷʤÚÂÙ·È fiÙÈ Ë ıÂÚ·›· Ì GH ‚ÂÏÙÈÒÓÂÈ ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· Û ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi, Èı·ÓfiÓ ·˘Í¿ÓÔÓÙ·˜ ÙËÓ ÈÛ¯‡ ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Ì˘ÒÓ (24,28). ∂ÈϤÔÓ ·Ú·ÙËÚ‹ıËΠ‚ÂÏÙ›ˆÛË ÙÔ˘ ·ÂÚÈÛÌÔ‡ Î·È Ù˘ ÂÈÛÓ¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÌÂÙ¿ ·fi 6 Ì‹Ó˜ ıÂÚ·›·˜ Ì GH, Èı·ÓfiÓ ÏfiÁˆ ÎÂÓÙÚÈ΋˜ ‰È¤ÁÂÚÛ˘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ΤÓÙÚÔ˘ (29). ∂›Û˘, Ë ıÂÚ·›· Ì GH Û Ôχ ÌÈÎÚ¿ ·È‰È¿ (<3 ¤ÙË) Ô‰ËÁ›, ÂÎÙfi˜ ·fi ‚ÂÏÙ›ˆÛË Ù˘ ·‡ÍËÛ˘ Î·È Ù˘ ۈ̷ÙÈ΋˜ Û‡ÛÙ·Û˘, Î·È Û ‚ÂÏÙ›ˆÛË Ù˘ ÁÏÒÛÛ·˜ Î·È Ù˘ ·ÓÙ›Ï˄˘ (Ì·ıËÛȷ΋˜ ÈηÓfiÙËÙ·˜), ÔfiÙ ›Ûˆ˜ Â›Ó·È ÚÔÙÈÌfiÙÂÚÔ Ë ·ÁˆÁ‹ Ó· ÍÂÎÈÓ¿ÂÈ Û Ôχ Ó·ڋ ËÏÈΛ· (30). ¶·ÚÂÓ¤ÚÁÂȘ Ù˘ ıÂÚ·›·˜ Ì GH ™Â οÔȘ ÌÂϤÙ˜ (14,22), ·ÏÏ¿ fi¯È Û fiϘ (23), ·Ó·Ê¤ÚıËΠ·Ó¿Ù˘ÍË ·ıÔÏÔÁÈ΋˜ ηÌ‡Ï˘ ÁÏ˘Îfi˙˘ ‹/Î·È ‰È·‚‹ÙË Ù‡Ô˘ ππ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ Ì GH. ∏ ‰È·ÎÔ‹ Ù˘ GH Ô‰‹ÁËÛ Û Â¿ÓÔ‰Ô Ù˘ ÁÏ˘Îfi˙˘ ÛÂ Ê˘ÛÈÔÏÔÁÈο Â›‰· (22). ∏ ÈÔ Û˘¯Ó‹ ·ÚÂÓ¤ÚÁÂÈ· Ù˘ ıÂÚ·›·˜ Ì G∏ ÛÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi Â›Ó·È Ë ÛÎÔÏ›ˆÛË, Ë ÔÔ›· fiÙ·Ó ÚÔ¸¿Ú¯ÂÈ ÌÔÚ› Ó· ÂȉÂÈÓˆı› (14). ∂›Û˘, ·Ó·Ê¤ÚÂÙ·È ÂÌÊ¿ÓÈÛË ÚÒÈÌ˘ ·‰ÚÂÓ·Ú¯‹˜ Û ÔÛÔÛÙfi 57% ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ‹Ú·Ó ıÂÚ·›· Ì GH (23). ™Â ¿ÙÔÌ· Ì ÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi Ù· ÔÔ›· ÂÏ¿Ì‚·Ó·Ó ıÂÚ·›· Ì GH, ·Ó·Ê¤ÚıËÎ·Ó ·ÈÊÓ›‰ÈÔÈ ı¿Ó·ÙÔÈ, ΢ڛˆ˜ ·fi ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Î·È ˘Ô·ÂÚÈÛÌfi ÛÙ· ·È‰È¿ Î·È ·fi ÙËÓ ·¯˘Û·ÚΛ· Î·È ÙȘ ÂÈÏÔΤ˜ Ù˘ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ (31,32). ¶·Ú¿ ÙËÓ Â˘ÂÚÁÂÙÈ΋ Â›‰Ú·ÛË Ù˘ GH ÛÙËÓ ·‡ÍËÛË, ÙË ¶·È‰È·ÙÚÈ΋ 2009;72:10-15

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·14

14

E. ∫Ô‡ÛÙ· Î·È Û˘Ó.

ۈ̷ÙÈ΋ Û‡ÛÙ·ÛË Î·È ÙË Ì·ıËÛȷ΋ ÈηÓfiÙËÙ·, ÔÈ ·Ú·ÙËÚ‹ÛÂȘ ·˘Ù¤˜ Ô‰‹ÁËÛ·Ó ÙÔ˘˜ ıÂÚ¿ÔÓÙ˜ ÁÈ·ÙÚÔ‡˜ Û ÛÎÂÙÈÎÈÛÌfi ˆ˜ ÚÔ˜ ÙË ¯Ú‹ÛË Ù˘ ηÈ, Û ÔÚÈṲ̂Ó˜ ÂÚÈÙÒÛÂȘ, Û ‰È·ÎÔ‹ Ù˘ ıÂÚ·›·˜. ∏ ¯ÔÚ‹ÁËÛË Ù˘ GH ‚ÂÏÙÈÒÓÂÈ ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· (24), fï˜ Â›Ó·È Èı·ÓfiÓ Û ÔÚÈṲ̂Ó˜ ÂÚÈÙÒÛÂȘ (Û ·¯‡Û·Úη ¿ÙÔÌ· Î·È Û ¿ÙÔÌ· Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ¿ÓÔȘ ‡ÓÔ˘) Ó· ÂȉÂÈÓÒÓÂÈ ÙÔÓ ˘Ô·ÂÚÈÛÌfi Ì 3 Ì˯·ÓÈÛÌÔ‡˜: ·) Ô‰ËÁ› Û ·‡ÍËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙÔ˘ Ì·Ï·ÎÔ‡ ÈÛÙÔ‡ ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Î·È ˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Â΂ϷÛÙ‹ÛˆÓ, ‚) ·˘Í¿ÓÂÈ ÙÔÓ ‚·ÛÈÎfi ÌÂÙ·‚ÔÏÈÛÌfi ηÈ, Û˘ÓÂÒ˜, ÙËÓ Î·Ù·Ó¿ÏˆÛË Ô͢ÁfiÓÔ˘ Î·È Á) ·˘Í¿ÓÂÈ ÙÔÓ fiÁÎÔ ÙÔ˘ ·›Ì·ÙÔ˜ Î·È ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÚÔ¸¿Ú¯Ô˘Û· ·Ê˘‰¿ÙˆÛË (32). ¶ÚÔÙ›ÓÂÙ·È, ÏÔÈfiÓ, ÛÙ· ¿ÙÔÌ· Ì ÙÔ Û‡Ó‰ÚÔÌÔ Prader-Willi, ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Ó· Ï·Ì‚¿ÓÂÙ·È ÈÛÙÔÚÈÎfi ÁÈ· ¿ÓÔȘ ‡ÓÔ˘ Î·È Ó· Á›ÓÂÙ·È ÚÔÛÂÎÙÈÎfi˜ ˆÙÔÚÈÓÔÏ·Ú˘ÁÁÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜, ¤ÏÂÁ¯Ô˜ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È Ô͢ÌÂÙÚ›·, ÒÛÙ ӷ ÂÓÙÔÈÛÙÔ‡Ó Ù· ¿ÙÔÌ· Ô˘ ¤¯Ô˘Ó ˘„ËÏfi ΛӉ˘ÓÔ ÂÌÊ¿ÓÈÛ˘ ·ÚÂÓÂÚÁÂÈÒÓ ·fi ÙË ıÂÚ·›· (32). ∂ÈϤÔÓ, ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Û˘ÓÈÛÙ¿Ù·È ·ÒÏÂÈ· ‚¿ÚÔ˘˜, ȉȷ›ÙÂÚ· ÛÙ· Ôχ ·¯‡Û·Úη ¿ÙÔÌ·, ÔÏ˘Î·Ù·ÁÚ·ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘ (polysomnography) ÛÙ· ¿ÙÔÌ· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Î·È ·Ê·›ÚÂÛË ·‰ÂÓÔÂȉÒÓ Â΂ϷÛÙ‹ÛÂˆÓ fiÙ·Ó ˘¿Ú¯ÂÈ ˘ÂÚÙÚÔÊ›· (32). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ Û˘ÓÈÛÙ¿Ù·È Ù·ÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË Ô˘ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ÙËÓ ÔÏ˘Î·Ù·ÁÚ·ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘. ™Ù· ¿ÙÔÌ· Ì ¿ÓÔȘ ‡ÓÔ˘ ‰ÂÓ ·ÔÎÏ›ÂÙ·È Ë ¯Ú‹ÛË Ù˘ GH, ·ÊÔ‡ Á›ÓÂÈ ˘ÔÛÙ‹ÚÈÍË ÙÔ˘ ·ÂÚÈÛÌÔ‡. øÛÙfiÛÔ, ·Ó ·ÔÊ·ÛÈÛÙ› Ë ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, Ú¤ÂÈ Ó· ˘¿Ú¯ÂÈ Ù·ÎÙÈ΋ Î·È Ôχ ÚÔÛÂÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË, Ì ÔÏ˘Î·Ù·ÁÚ·ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘. §fiÁˆ ÙÔ˘ fiÙÈ Ù· ÂÚÈÛÛfiÙÂÚ· ÂÂÈÛfi‰È· (75%) ·ÈÊÓȉ›ˆÓ ı·Ó¿ÙˆÓ Û˘Ó¤‚ËÛ·Ó Î·Ù¿ ÙÔ˘˜ 9 ·Ú¯ÈÎÔ‡˜ Ì‹Ó˜ Ù˘ ıÂÚ·›·˜ Ì GH, Û˘ÓÈÛÙ¿Ù·È Ó· ¯ÔÚËÁÂ›Ù·È Ë GH ·Ú¯Èο Û ÌÈÎÚ¤˜ ‰fiÛÂȘ, Ó· Á›ÓÂÙ·È ÛÙ·‰È·Î‹ ÙÈÙÏÔÔ›ËÛË Ù˘ ‰fiÛ˘ Î·È Ë ·Ú·ÎÔÏÔ‡ıËÛË Ó· Â›Ó·È Ù·ÎÙÈ΋ ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ Ù˘ ıÂÚ·›·˜ (33).

™˘ÌÂÚ¿ÛÌ·Ù· ∏ ıÂÚ·›· Ì GH ÛÙ· ·È‰È¿ Ì ۇӉÚÔÌÔ Prader-Willi ‚ÂÏÙÈÒÓÂÈ ÙÔ ‡„Ô˜, ÙÔÓ Ú˘ıÌfi ·Ó¿Ù˘Í˘ ηÈ, Èı·ÓfiÓ, ÙÔ ÙÂÏÈÎfi ·Ó¿ÛÙËÌ·. ∂›Û˘, Ë ıÂÚ·›· ‚ÂÏÙÈÒÓÂÈ ÙË Û‡ÛÙ·ÛË ÛÒÌ·ÙÔ˜, ·˘Í¿ÓÔÓÙ·˜ ÙËÓ ¿ÏÈÔ Ì¿˙· Î·È ÌÂÈÒÓÔÓÙ·˜ ÙÔÓ ÏÈÒ‰Ë ÈÛÙfi, ¯ˆÚ›˜ fï˜ Ó· ‚ÂÏÙÈÒÓÂÈ ÛËÌ·ÓÙÈο ÙËÓ ·¯˘Û·ÚΛ·. ∏ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· Â›Ó·È Î·Ï‡ÙÂÚË fiÙ·Ó ÍÂÎÈÓ¿ Û ÌÈÎÚ‹ ËÏÈΛ·. ∂ÓÙÔ‡ÙÔȘ, ÙÔ ‡„Ô˜ Û¿ÓÈ· ·ÔÙÂÏ› ·ÚÈ· ·ÓËÛ˘¯›· ÛÙÔ Û‡Ó‰ÚÔÌÔ, ÏfiÁˆ ÙˆÓ ÔÏÏ·ÏÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ Ô˘ ·ÓÙÈÌÂÙˆ›Paediatriki 2009;72:10-15

˙Ô˘Ó Ù· ·È‰È¿ Î·È ÔÈ ÔÈÎÔÁ¤ÓÂȤ˜ ÙÔ˘˜. °È’ ·˘Ùfi, Ë ıÂÚ·›· Ì GH ‰ÂÓ Û˘ÓÈÛÙ¿Ù·È ˆ˜ ‚·ÛÈ΋ Ú·ÎÙÈ΋ ÁÈ· ÙË ‚ÂÏÙ›ˆÛË Ù˘ ·‡ÍËÛ˘ ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ. ∏ ¯ÔÚ‹ÁËÛË GH ÌÔÚ› Ó· Â›Ó·È ¯Ú‹ÛÈÌË ˆ˜ ÚÔ˜ ÙËÓ ·‡ÍËÛË, Û ÂÚÈÙÒÛÂȘ Ô˘ ÙÔ ·È‰› ÂÌÊ·Ó›˙ÂÈ ¯·ÌËÏfi ·Ó¿ÛÙËÌ·, ÂÏ·Ùو̤ÓÔ Ú˘ıÌfi ·‡ÍËÛ˘ ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ‚¿ÚÔ˜ Î·È ÚÔ¤Ú¯ÂÙ·È ·fi ÌÈ· ¢·ÈÛıËÙÔÔÈË̤ÓË ÔÈÎÔÁ¤ÓÂÈ·, ·fi ÙËÓ ÔÔ›· ·Ó·Ì¤ÓÂÙ·È Î·Ï‹ Û˘ÌÌfiÚʈÛË. ™˘ÓÈÛÙ¿Ù·È ·Ú¯È΋ ‰fiÛË 15IU(5mg)/m2/‚‰. ÂÚ›Ô˘, fiˆ˜ ÁÈ· ÙËÓ ÎÏ·ÛÈ΋ ·ÓÂ¿ÚÎÂÈ· GH, Ì ·Ó·ÚÔÛ·ÚÌÔÁ‹ Ù˘ ‰fiÛ˘, Ì ‚¿ÛË ÙËÓ ·ÓÙ·fiÎÚÈÛË Ù˘ ·‡ÍËÛ˘ Î·È Ù· Â›‰· ÙÔ˘ IGF-1. ∆· ÚfiÛÊ·Ù· ‰Â‰Ô̤ӷ Ô˘ ·ÊÔÚÔ‡Ó ÛÙË ‚ÂÏÙ›ˆÛË Ù˘ ۈ̷ÙÈ΋˜ Û‡ÛÙ·Û˘ ‰ÂÓ ÌÔÚÔ‡Ó Ó· Û˘ÓËÁÔÚ‹ÛÔ˘Ó Â·ÚÎÒ˜, ÙÔ˘Ï¿¯ÈÛÙÔÓ ÚÔ˜ ÙÔ ·ÚfiÓ, ˘¤Ú Ù˘ ÂÈÙ·ÎÙÈ΋˜ ¯ÔÚ‹ÁËÛ˘ GH ˆ˜ ıÂÚ·›·˜ ÚÔ˘Ù›Ó·˜, ÂÓÒ Ô ‰È·ÙÚÔÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ ·Ú·Ì¤ÓÂÈ Ô ·ÎÚÔÁˆÓÈ·›Ô˜ Ï›ıÔ˜ Ù˘ ηϋ˜ ·ÓÙÈÌÂÙÒÈÛ˘. ∏ ‚ÂÏÙȈ̤ÓË Ì˘˚΋ ÈÛ¯‡˜ Î·È ÎÈÓËÙÈÎfiÙËÙ·, Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ô˘ ¤Ï·‚·Ó ıÂÚ·›· Ì GH (18,30), ¤¯ÂÈ È‰È·›ÙÂÚÔ ÂӉȷʤÚÔÓ ÁÈ· ÙË ıÂÚ·›· ÙˆÓ ·È‰ÈÒÓ Ì ۇӉÚÔÌÔ Prader-Willi ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜. ∏ ‚ÂÏÙ›ˆÛË Ù˘ ۈ̷ÙÈ΋˜ Û‡ÛÙ·Û˘ Î·È Ù˘ Ì˘˚΋˜ Ì¿˙·˜ ÛÙËÓ ËÏÈÎȷ΋ ·˘Ù‹ ÔÌ¿‰· ı· ÌÔÚÔ‡Û ӷ ¤¯ÂÈ ÛËÌ·ÓÙÈ΋ Â›‰Ú·ÛË ÛÙËÓ ÎÈÓËÙÈÎfiÙËÙ¿ ÙÔ˘˜, Ì ٷ¯‡ÙÂÚË Â›Ù¢ÍË ÙˆÓ ·‰ÚÒÓ ÎÈÓËÙÈÎÒÓ ÔÚfiÛË̈Ó, ηıÒ˜ Î·È ÁÂÓÈÎfiÙÂÚ· ÛÙËÓ ÔÈfiÙËÙ· ˙ˆ‹˜. √È ·ÈÊÓ›‰ÈÔÈ ı¿Ó·ÙÔÈ Ô˘ ·Ú·ÙËÚ‹ıËÎ·Ó Î˘Ú›ˆ˜ ·fi ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Î·È ˘Ô·ÂÚÈÛÌfi ÛÙ· ·È‰È¿ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ Ì GH Ô‰ËÁÔ‡Ó Û ÚÔ‚ÏËÌ·ÙÈÛÌfi ˆ˜ ÚÔ˜ ÙËÓ ·ÛÊ¿ÏÂÈ· Ù˘ ıÂÚ·›·˜. °È· Ó· ÂÓÙÔÈÛÙÔ‡Ó Ù· ¿ÙÔÌ· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Î·È Ó· ÚÔÏËÊı› Ù˘¯fiÓ Âȉ›ӈÛË ÙÔ˘ ˘Ô·ÂÚÈÛÌÔ‡ ηٿ ÙË ıÂÚ·›· ¯ÚÂÈ¿˙ÂÙ·È ÚÔÛÂÎÙÈÎfi˜ ˆÙÔÚÈÓÔÏ·Ú˘ÁÁÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜, ¤ÏÂÁ¯Ô˜ ·Ó·Ó¢ÛÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Î·È Ô͢ÌÂÙÚ›·, ÚÈÓ Î·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·ÒÏÂÈ· ‚¿ÚÔ˘˜, ÔÏ˘Î·Ù·ÁÚ·ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘ Î·È ˘ÔÛÙ‹ÚÈÍË ÙÔ˘ ·ÂÚÈÛÌÔ‡, fiÔ˘ Â›Ó·È ··Ú·›ÙËÙÔ.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Cassidy SB, Dykens E, Williams CA. Prader-Willi and Angelman syndromes: sister imprinted disorders. Am J Med Genet 2000;97:136-146.

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4. Donaldson MD, Chu CE, Cooke A, Wilson A, Greene SA,

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Stephenson JB. The Prader-Willi syndrome. Arch Dis Child 1994;70:58-63.

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14. Craig ME, Cowell CT, Larsson P, Craig ME, Cowell CT, Larsson P, et al. Growth hormone treatment and adverse events in Prader-Willi syndrome: data from KIGS (the Pfizer International Growth Database). Clin Endocrinol (Oxf) 2006;65:178-185.

15. Lindgren AC, Hagenäs L, Müller J, Blichfeldt S, Rosenborg M, Brismar T, et al. Effects of growth hormone treatment on growth and body composition in Prader-Willi syndrome: a preliminary report. Acta Paediatr Suppl 1997; 423:60-62.

16. Hauffa BP. One-year results of growth hormone treatment of short stature in Prader-Willi syndrome. Acta Paediatr Suppl 1997;423:63-65.

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24. Haqq AM, Stadler DD, Jackson RH, Rosenfeld RG, Purnell JQ, LaFranchi SH. Effects of growth hormone on pulmonary function, sleep quality, behavior, cognition, growth velocity, body composition, and resting energy expenditure in Prader-Willi syndrome. J Clin Endocrinol Metab 2003;88:2206-2212.

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32. Stafler P, Wallis C. Prader-Willi syndrome: who can have growth hormone? Arch Dis Child 2008;93:341-345.

33. Tauber M, Diene G, Molinas C, Hébert M. Review of 64 cases of death in children with Prader-Willi syndrome (PWS). Am J Med Genet A 2008;146:881-887.

¶·È‰È·ÙÚÈ΋ 2009;72:10-15

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REVIEW ARTICLE

¡ÂÔÁÓÈΤ˜ ÏÔÈÌÒÍÂȘ ·fi ‚-·ÈÌÔÏ˘ÙÈÎfi ÛÙÚÂÙfiÎÔÎÎÔ Ù˘ ÔÌ¿‰·˜ µ 1 µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£., ¡ÔÛÔÎÔÌÂ›Ô ¶··ÁˆÚÁ›Ô˘, £ÂÛÛ·ÏÔÓ›ÎË 2 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£., ¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞, £ÂÛÛ·ÏÔÓ›ÎË AÏÏËÏÔÁÚ·Ê›·: ÃÚ‹ÛÙÔ˜ ∆۷ηϛ‰Ë˜ zoichr@otenet.gr °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢ «¶··ÁˆÚÁ›Ô˘», £ÂÛÛ·ÏÔÓ›ÎË

Ã. ∆۷ηϛ‰Ë˜1, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜2, ¡. ¡ÈÎÔÏ·˝‰Ë˜1 ¶ÂÚ›ÏË„Ë: ∞Ó Î·È Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ¤¯ÂÈ Á›ÓÂÈ ÙÂÚ¿ÛÙÈ· ÚfiÔ‰Ô˜ ÛÙÔÓ ÙÔ̤· Ù˘ ÚfiÏ˄˘ Ù˘ ÓÂÔÁÓÈ΋˜ Ïԛ̈͢ ·fi ‚-·ÈÌÔÏ˘ÙÈÎfi ÛÙÚÂÙfiÎÔÎÎÔ Ù˘ ÔÌ¿‰·˜ µ (GBS), ÂÓÙÔ‡ÙÔȘ Ë Ïԛ̈ÍË ·˘Ù‹ ·Ú·Ì¤ÓÂÈ Ì›· ·fi ÙȘ ·ÚȘ ·Èٛ˜ ÛË„·ÈÌ›·˜ Î·È ÌËÓÈÁÁ›Ùȉ·˜ Û ·˘Ù‹ ÙËÓ ËÏÈΛ·. √ Á·ÛÙÚÂÓÙÂÚÈÎfi˜ ۈϋӷ˜ ·ÔÙÂÏ› ÙË Ê˘ÛÈ΋ ·ÓıÚÒÈÓË ‰ÂÍ·ÌÂÓ‹ ÁÈ· ÙÔÓ GBS Î·È ·Ó·ÁÓˆÚ›˙ÂÙ·È ˆ˜ Èı·Ó‹ ËÁ‹ ÁÈ· ÙÔÓ ÎÔÏÈÎfi ·ÔÈÎÈÛÌfi. ∏ ÓÂÔÁÓÈ΋ Ïԛ̈ÍË ·fi GBS ÚÔηÏÂ›Ù·È Û˘Ó‹ıˆ˜ ·fi ÙÔÓ ·ÔÈÎÈÛÌfi ÙÔ˘ ·ÌÓÈ·ÎÔ‡ ˘ÁÚÔ‡ ̤ۈ Ù˘ ·ÓÈÔ‡Û·˜ Ô‰Ô‡, Ô˘ Ô‰ËÁ› ÙÂÏÈο Û ÓfiÛÔ, Û ÌÂÚÈο ÓÂÔÁÓ¿. ŸÙ·Ó Ë Ïԛ̈ÍË Ï·Ì‚¿ÓÂÈ ¯ÒÚ· ÙȘ ÚÒÙ˜ 7 Ë̤Ú˜ Ù˘ ˙ˆ‹˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ÚÒÈÌË, ÂÓÒ ÌÂÙ¿ ÙËÓ 7Ë Ë̤ڷ Î·È Ì¤¯ÚÈ ÙÔ˘˜ 3 Ì‹Ó˜ ˆ˜ fi„ÈÌË ÌÔÚÊ‹. ∏ Ó¢ÌÔÓ›· Î·È Ë ÛË„·ÈÌ›· ·ÔÙÂÏÔ‡Ó ÙȘ Û˘¯ÓfiÙÂÚ˜ ÂΉËÏÒÛÂȘ Ù˘ ÚÒÈÌ˘ ÌÔÚÊ‹˜, ÂÓÒ Ë Î‡ÚÈ· ÎÏÈÓÈ΋ ÂΉ‹ÏˆÛË Ù˘ fi„ÈÌ˘ Ïԛ̈͢ Â›Ó·È Ë ÌËÓÈÁÁ›Ùȉ·. ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ·Ó·ÛÎfiËÛ˘ Â›Ó·È Ë Â˘·ÈÛıËÙÔÔ›ËÛË ÙˆÓ ·È‰È¿ÙÚˆÓ Î·È ÙˆÓ ÓÂÔÁÓÔÏfiÁˆÓ ÛÙËÓ ·Ó·ÁÓÒÚÈÛË ÙˆÓ ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘, ηıÒ˜ Î·È ÛÙËÓ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È ıÂÚ·›·, Ô˘ ·ÔÙÂÏÔ‡Ó ··Ú·›ÙËÙ˜ ÚÔ¸Ôı¤ÛÂȘ ÁÈ· ÙËÓ ·ÔÙÚÔ‹ ÙˆÓ ÛÔ‚·ÚÒÓ ÂÈÏÔÎÒÓ Ù˘ ÓfiÛÔ˘. §¤ÍÂȘ ÎÏÂȉȿ: ™ÙÚÂÙfiÎÔÎÎÔ˜ ÔÌ¿‰·˜ µ, ÓÂÔÁÓÈ΋ ËÏÈΛ·, ÛË„·ÈÌ›·.

Newborn infections from group B Streptococcus 1 2nd Department of Neonatology, “Aristoteleio” University of Thessaloniki, “Papageorgiou” Hospital, Thessaloniki, Greece 2 2nd Department of Paediatrics, “Aristoteleio” University of Thessaloniki, “AHEPA” Hospital, Thessaloniki, Greece Correspondence: Christos Tsakalidis zoichr@otenet.gr “Papageorgiou” General Hospital of Thessaloniki, Thessaloniki, Greece

C. Tsakalidis1, A. Tragiannidis2, N. Nikolaidis1 Abstract: Even though great progress has been made in the prevention of neonatal Group B Streptococcus (GBS) infection, it remains one of the main causes of sepsis and meningitis at this age. The gastrointestinal tract represents the natural human source for GBS and constitutes a possible source of vaginal colonization. Neonatal GBS infection is due to the ascent of the bacteria into the amniotic fluid, which leads to neonatal colonization and subsequently to infection in some neonates. Early-onset disease occurs when the infection presents during the first 7 days of life, and late-onset disease presents after the 7th day and up to the 3rd month. Pneumonia and meningitis are the most common infections in neonates developing symptoms during the first 7 days of life, while the main clinical onset of late infection is meningitis. The aim of this review is to alert paediatricians and neonatologists to the risk factors, and to enable them to make timely diagnosis and initiation of treatment, which is necessary in order to avoid the serious consequences of the disease.

Key words: Streptococcus group B, newborn, sepsis.

∂ÈÛ·ÁˆÁ‹ √ ‚-·ÈÌÔÏ˘ÙÈÎfi˜ ÛÙÚÂÙfiÎÔÎÎÔ˜ Ù˘ ÔÌ¿‰·˜ µ (Group B Streptococcus, GBS), ÁÓˆÛÙfi˜ Î·È ˆ˜ Streptococcus agalactiae, Â›Ó·È ¤Ó·˜ gramıÂÙÈÎfi˜ ‰ÈÏfiÎÔÎÎÔ˜, Ô ÔÔ›Ô˜ ·ÔÙÂÏ› Ì›· ·fi ÙȘ ÛËÌ·ÓÙÈÎfiÙÂÚ˜ ·Èٛ˜ Ù˘ ÓÂÔÁÓÈ΋˜ ÛË„·ÈÌ›·˜. ÀÔÏÔÁ›˙ÂÙ·È fiÙÈ ÛÙȘ ∏ӈ̤Ó˜ ¶ÔÏÈÙ›˜ ÙÔ˘Ï¿¯ÈÛÙÔÓ 2.000 ÓÂÔÁÓ¿ ηٷϋÁÔ˘Ó Î¿ı ¯ÚfiÓÔ ·fi ÏÔÈÌÒÍÂȘ Ô˘ ÔÊ›ÏÔÓÙ·È ÛÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ‚·ÎÙ‹ÚÈÔ. ∞Ú¯Èο Ô GBS ÂÓÔ¯ÔÔÈ‹ıËΠÁÈ· ÙËÓ ÚfiÎÏËÛË Ù˘ Ì·ÛÙ›Ùȉ·˜ ÙˆÓ ‚ÔÔÂȉÒÓ Î·È Ô˘ÛÈ·ÛÙÈο ̤¯ÚÈ ÙÔ 1938 ‰ÂÓ ıˆÚ›ÙÔ ·ıÔÁfiÓÔ ÌÈÎÚfi‚ÈÔ ÁÈ· ÙÔÓ ·ÓıÚÒÈÓÔ ÔÚÁ·ÓÈÛÌfi (1,2). ŸÌˆ˜, ÛÙË ‰ÂηÂÙ›· ÙÔ˘ 1970 ·Ó·ÁÓˆÚ›˙ÂÙ·È ˆ˜ ¤Ó·˜ ÛËÌ·ÓÙÈÎfi˜ Paediatriki 2009;72:16-22

·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ù˘ ÓÂÔÁÓÈ΋˜ ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÛÈÌfiÙËÙ·˜, ÊÙ¿ÓÔÓÙ·˜ Û ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ·ÎfiÌË Î·È ·fi ÙËÓ Escherichia coli, ÌÂ Û˘¯ÓfiÙËÙ· 2-3 ÂÚÈÙÒÛÂȘ ·Ó¿ 1000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ÓÂÔÁÓÒÓ Î·È Ì ıÓËÛÈÌfiÙËÙ· ÂÚ›Ô˘ ÛÙÔ 50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (3). ™Ù· ÂfiÌÂÓ· ¯ÚfiÓÈ· ·Ú·ÙËÚ‹ıËΠÛËÌ·ÓÙÈÎfiÙ·ÙË Ì›ˆÛË Ù˘ ıÓËÛÈÌfiÙËÙ·˜, Ë ÔÔ›· ·Ô‰›‰ÂÙ·È ÛÙËÓ ÂÈÛ·ÁˆÁ‹ Î·È ÂÊ·ÚÌÔÁ‹ ÙˆÓ ÂÚÈÁÂÓÓËÙÈÎÒÓ ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ, ÛÙË ‚ÂÏÙ›ˆÛË Ù˘ ÓÂÔÁÓÈ΋˜ ÊÚÔÓÙ›‰·˜ Î·È ÛÙË ¯Ú‹ÛË ·ÓÙÈÌÈÎÚÔ‚È·ÎÒÓ Ê·Ú̿ΈÓ.

∂ȉËÌÈÔÏÔÁ›· ∆Ô Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ· Â›Ó·È Ë Ê˘ÛÈ΋

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·17

17

¡ÂÔÁÓÈΤ˜ ÏÔÈÌÒÍÂȘ ·fi µ-·ÈÌÔÏ˘ÙÈÎfi ÛÙÚÂÙfiÎÔÎÎÔ Ù˘ ÔÌ¿‰·˜ µ

·ÓıÚÒÈÓË ‰ÂÍ·ÌÂÓ‹ ÁÈ· ÙÔÓ GBS Î·È ·ÔÙÂÏ› ÙËÓ Èı·Ó‹ ËÁ‹ ÁÈ· ÙÔÓ ÎÔÏÈÎfi ·ÔÈÎÈÛÌfi. ∏ ÌÂÙ·ÊÔÚ¿ ÙÔ˘ GBS ÛÙËÓ ¤ÁÎ˘Ô ÌÔÚ› Ó· Â›Ó·È ¯ÚfiÓÈ·, ‰È·Ï›Ô˘Û· ‹ ·ÚÔ‰È΋ (4). ™Ù· ÓÂÔÁÓ¿ Ô˘ ÁÂÓÓÈÔ‡ÓÙ·È ·fi ÌËÙ¤Ú˜ ·ÔÈÎÈṲ̂Ó˜ Ì GBS, ÙÔ ÌÈÎÚfi‚ÈÔ ·ÔÌÔÓÒÓÂÙ·È ÛÙÔÓ ÚˆÎÙfi, ÛÙÔ ÔÌÊ·ÏÈÎfi ÎÔÏfi‚ˆÌ· ‹ ÛÙÔ ÛÙfiÌ·, Û ÔÛÔÛÙfi Ô˘ Î˘Ì·›ÓÂÙ·È ·fi 30-70%. Ÿˆ˜ ·Ó·Ê¤ÚıËÎÂ, ηٷÁÚ¿ÊÔÓÙ·È 2-3 ÂÚÈÙÒÛÂȘ ÓÂÔÁÓÈ΋˜ ÛÙÚÂÙÔÎÔÎÎÈ΋˜ Ïԛ̈͢ ·Ó¿ 1.000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ÓÂÔÁÓÒÓ, ·Ó Î·È Ë Â›ÙˆÛË ÌÔÚ› Ó· ·˘ÍËı› Û 10 ÂÚÈÛÙ·ÙÈο ·Ó¿ 1.000 ÁÂÓÓ‹ÛÂȘ Û ÂÚÈÔ¯¤˜ fiÔ˘ fiϘ ÔÈ ¤ÁÎ˘Â˜ Â›Ó·È ·ÔÎÈṲ̂Ó˜ Ì GBS (5-8). øÛÙfiÛÔ, ÌfiÓÔ 1-2% ÙˆÓ ÓÂÔÁÓÒÓ Ù· ÔÔ›· ÁÂÓÓ‹ıËÎ·Ó ·fi ÌËÙ¤Ú˜ ÊÔÚ›˜ ÂΉËÏÒÓÔ˘Ó ÎÏÈÓÈ΋ ÓfiÛÔ. √ ÏfiÁÔ˜ ÁÈ’ ·˘Ùfi ÙÔ ¯·ÌËÏfi ÔÛÔÛÙfi ·Ú·Ì¤ÓÂÈ ¿ÁÓˆÛÙÔ˜ Î·È Â›Ó·È ·ÎfiÌË ˘fi ‰ÈÂÚ‡ÓËÛË. ¶Èı·ÓÔÏÔÁÂ›Ù·È Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙÔ˘ ‚·ıÌÔ‡ ÌËÙÚÈÎÔ‡ ·ÔÈÎÈÛÌÔ‡ Î·È Ù˘ ÌÂÙ¤ÂÈÙ· ÎÏÈÓÈ΋˜ ÔÚ›·˜ ÙˆÓ ÓÂÔÁÓÒÓ. ∆· ÚfiˆÚ· ÓÂÔÁÓ¿ ¤¯Ô˘Ó 15 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚÔ Î›Ó‰˘ÓÔ Ó· ·Ó·Ù‡ÍÔ˘Ó ÛÙÚÂÙÔÎÔÎÎÈ΋ Ïԛ̈ÍË Û ۯ¤ÛË Ì ٷ ÙÂÏÂÈfiÌËÓ· Î·È ·˘Ùfi ÙÔ Ôχ ˘„ËÏfi ÔÛÔÛÙfi Ïԛ̈͢ ›Ûˆ˜ Ó· ·ÓÙ·Ó·ÎÏ¿ ÙÔ ·ÓÒÚÈÌÔ ·Ì˘ÓÙÈÎfi Û‡ÛÙËÌ¿ ÙÔ˘˜ (9). §fiÁˆ Ù˘ ÂÏ·Ùو̤Ó˘ ÂÓÂÚÁÔÔ›ËÛ˘ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ ·fi ÙÔÓ GBS, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ ·ÚÔ˘Û›· ο„·˜ ÛÙÔ ÂÓ ÏfiÁˆ ‚·ÎÙ‹ÚÈÔ, Ô ÚfiÏÔ˜ ÙˆÓ ÂȉÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ˆ˜ ·¿ÓÙËÛË ÛÙË Ïԛ̈ÍË Â›Ó·È ÎÚ›ÛÈÌ˘ ÛËÌ·Û›·˜. Œ¯ÂÈ ·Ô‰Âȯı› fiÙÈ ÙÔ 60% Ù˘ ‰È·Ï·ÎÔ˘ÓÙȷ΋˜ ÌÂÙ·ÊÔÚ¿˜ ÙˆÓ IgG ·ÓÙÈÛˆÌ¿ÙˆÓ Ú·ÁÌ·ÙÔÔÈÂ›Ù·È Î˘Ú›ˆ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÙÚ›ÙÔ˘ ÙÚÈÌ‹ÓÔ˘ Ù˘ ·ËÛ˘ Î·È ÂȉÈÎfiÙÂÚ· ÌÂÙ¿ ÙËÓ 32Ë Â‚‰ÔÌ¿‰·, Ì ·ÔÙ¤ÏÂÛÌ· Ù· ÚfiˆÚ· ÓÂÔÁÓ¿ Ó· ¤¯Ô˘Ó ÂÏ¿¯ÈÛÙÔ˘˜ ‹ ¯·ÌËÏÔ‡˜ Ù›ÙÏÔ˘˜ ÌËÙÚÈÎÒÓ IgG ·ÓÙÈÛˆÌ¿ÙˆÓ (10). ™·ÊÒ˜ Ë ¯·ÌËÏ‹ ·ÓÙÈۈ̷ÙÈ΋ Û˘ÁΤÓÙÚˆÛË ·ÔÙÂÏ› ·ÈÙÈÔÏÔÁÈÎfi Î·È ÚԉȷıÂÛÈÎfi ·Ú¿ÁÔÓÙ· Ïԛ̈͢ ·fi GBS, fï˜ ‰ÂÓ Â›Ó·È Ô ÌÔÓ·‰ÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Î·È ·˘Ùfi ·Ô‰ÂÈÎÓ‡ÂÙ·È ·fi ÙËÓ ÂÚÁ·ÛÙËÚȷ΋ ·Ú·Ù‹ÚËÛË fiÙÈ Ù· ÂÚÈÛÛfiÙÂÚ· ÓÂÔÁÓ¿ Ô˘ ÁÂÓÓÈÔ‡ÓÙ·È ·fi ·ÔÈÎÈṲ̂Ó˜ ÌËÙ¤Ú˜ Î·È ¤¯Ô˘Ó ¯·ÌËÏ‹ ·ÚÔ˘Û›· ·ÓÙÈÛˆÌ¿ÙˆÓ ‰ÂÓ ÓÔÛÔ‡Ó (11). ∏ ÂÈÎÚ¿ÙËÛË ÙÔ˘ GBS, fiÛÔÓ ·ÊÔÚ¿ ÙÔÓ ·ÔÈÎÈÛÌfi ÛÙÔÓ ÎfiÏÔ Î·È ÛÙÔÓ ÚˆÎÙfi ÙˆÓ ÂÁ·ˆÓ Á˘Ó·ÈÎÒÓ, ÔÈΛÏÏÂÈ ÌÂٷ͇ ÙˆÓ ÂıÓÈÎÒÓ ÔÌ¿‰ˆÓ Î·È ÙˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ, ηٿ ÚÔÛ¤ÁÁÈÛË ÌÂٷ͇ 10-40% (7,12-15). √È Á˘Ó·›Î˜ Ô˘ ·Ó‹ÎÔ˘Ó ÛÙË Ì·‡ÚË Ê˘Ï‹ ·Ó·Ê¤ÚÂÙ·È fiÙÈ ·ÔÈΛ˙ÔÓÙ·È Û ˘„ËÏfiÙÂÚÔ ÔÛÔÛÙfi Û ۇÁÎÚÈÛË Ì ÙȘ ˘fiÏÔÈ˜ Ê˘ÏÂÙÈΤ˜ ÔÌ¿‰Â˜ (16). ∞Ó Î·È Ô ·ÔÈÎÈÛÌfi˜ Ì GBS ‰ÂÓ Û˘Ó‰¤ÂÙ·È Ì ÓfiÛËÛË Û ˘ÁÈ›˜ Á˘Ó·›Î˜, ÂÓÙÔ‡ÙÔȘ ÌÔÚ› Ó· ÚÔηϤÛÂÈ ÌfiÏ˘ÓÛË ÛÙËÓ ÂÁ΢ÌÔÛ‡ÓË. §ÔÈÌÒÍÂȘ ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ ·fi GBS ¤¯Ô˘Ó ·-

Ú·ÙËÚËı› Û 2-4% ÙˆÓ Î˘‹ÛÂˆÓ (17). ™ÙȘ Â›ÙÔΘ, ¿ÏϘ ÏÔÈÌÒÍÂȘ Ô˘ ÔÊ›ÏÔÓÙ·È Û GBS Â›Ó·È Ë ÛË„·ÈÌ›·, Ë ·ÌÓÈÔÓ›Ùȉ·, Ë ÂÓ‰ÔÌËÙÚ›Ùȉ· Î·È Û¿ÓÈ· Ë ÌËÓÈÁÁ›Ùȉ· (18). ∞fi ÙÔ˘˜ ÔÚfiÙ˘Ô˘˜ ÙÔ˘ GBS Ô˘ ¤¯Ô˘Ó ·Ó·ÁÓˆÚÈÛÙ›, ˘‡ı˘ÓÔÈ ÁÈ· ÏÔÈÌÒÍÂȘ Â›Ó·È Ô Ù‡Ô˜ Ia, Ô Ù‡Ô˜ Ib, Ô Ù‡Ô˜ ππ, Ô Ù‡Ô˜ πππ Î·È ÙÂÏÂ˘Ù·›· Ô Ù‡Ô˜ V. ∞Ó Î·È Ô ÏËı˘ÛÌfi˜ ÙˆÓ ÌÈÎÚÔ‚›ˆÓ ÛÙȘ ·ÔÈÎÈṲ̂Ó˜ Á˘Ó·›Î˜ ·ÚÔ˘ÛÈ¿˙ÂÈ ›ÛË Î·Ù·ÓÔÌ‹ fiÛÔÓ ·ÊÔÚ¿ ÙÔ˘˜ ÙÚÂȘ ÈÔ ÎÔÈÓÔ‡˜ ÔÚfiÙ˘Ô˘˜ (π, ππ Î·È πππ), ÔÈ ÌÂϤÙ˜ ÛÙ· ÓÂÔÁÓ¿ Ì Ïԛ̈ÍË ‰Â›¯ÓÔ˘Ó ˘ÂÚÔ¯‹ ÙÔ˘ ÔÚfiÙ˘Ô˘ πππ (85%). ¶Èı·ÓÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ ¢ı‡ÓÔÓÙ·È ÁÈ’ ·˘Ùfi Â›Ó·È Ë ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ÂȉÈÎÔ‡ ·ÓÙÈÁfiÓÔ˘ Î·È Ë ÌÂÁ·Ï‡ÙÂÚË ·Ú·ÁˆÁ‹ Ó¢ڷÌÈÓȉ¿Û˘ (19).

¶·ıÔÁ¤ÓÂÈ· ∏ ÈÔ Û˘¯Ó‹ ÌÔÚÊ‹, Ë ÔÔ›· Ù·˘Ùfi¯ÚÔÓ· ¤¯ÂÈ Î·È ÙËÓ Î·Ï‡ÙÂÚË ÚfiÁÓˆÛË, Â›Ó·È Ë ÚÒÈÌË, ηٿ ÙËÓ ÔÔ›· Ë ÂΉ‹ÏˆÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Á›ÓÂÙ·È Ì¤Û· ÛÙȘ ÚÒÙ˜ 7 Ë̤Ú˜ ·fi ÙË Á¤ÓÓËÛË. ™˘¯ÓfiÙÂÚ· Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÂΉËÏÒÓÂÙ·È Ì¤Û· ÛÙȘ ÚÒÙ˜ 24 ÒÚ˜. ™ÙËÓ ÚÒÈÌË ÌÔÚÊ‹ ¤¯Ô˘Ì ›ÛË Â›ÙˆÛË Ù˘ ÓfiÛÔ˘ Û ¿ÚÚÂÓ· Î·È ı‹Ï·. Ÿˆ˜ ηٷ‰ÂÈÎÓ‡ÂÙ·È ·fi ÌÂϤÙË ÙˆÓ ÔÚfiÙ˘ˆÓ ·ÔÈÎÈÛÌ¤ÓˆÓ ÌËÙ¤ÚˆÓ Î·È ÓÂÔÁÓÒÓ, Ë ÌÂÙ¿‰ÔÛË Â›Ó·È Î¿ıÂÙË fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ÚÒÈÌË ÓfiÛÔ. ™‡Ìʈӷ Ì ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ, ¤¯Ô˘Ó ηٷÁÚ·Ê› ‰‡Ô ÌÔÓ٤Ϸ ÌÂÙ¿‰ÔÛ˘ Ù˘ ÓfiÛÔ˘ ·fi ÙË ÌËÙ¤Ú· ÛÙÔ ÓÂÔÁÓfi. ™ÙÔ ÚÒÙÔ, ÙÔ ÓÂÔÁÓfi ÁÂÓÓÈ¤Ù·È Ì ¯·ÌËÏfi Apgar score Î·È ·ÎÔÏÔ˘ı› Ë Ó¢ÌÔÓÈ΋ ‰È‹ıËÛË, ÂÓÒ ÛÙÔ ‰Â‡ÙÂÚÔ ÙÔ ÓÂÔÁÓfi ÁÂÓÓÈ¤Ù·È ˘ÁȤ˜, ·ÏÏ¿ ·Ó·Ù‡ÛÛÂÈ ‚·ıÌÈ·›· Û˘ÌÙÒÌ·Ù· ·ÚÎÂÙ¤˜ Ë̤Ú˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË. ∞˘Ù‹ Ë ‰È·ÊÔÚ¿ Ô‰ËÁ› ÛÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ Ë Ïԛ̈ÍË ÌÔÚ› Ó· Û˘Ì‚Â› ›Ù ‰È·ÌÓȷο, ›Ù ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ‰Èfi‰Ô˘ ̤ۈ ÙÔ˘ ˘ÂÏÔÁÂÓÓËÙÈÎÔ‡ ۈϋӷ Ù˘ ÌËÙ¤Ú·˜. ™Â Ì›· ÌÂϤÙË ÙÔ˘ Garland, ÙÔ 56% ÙˆÓ ÓÂÔÁÓÒÓ Ì Ïԛ̈ÍË ·fi GBS ‹Ù·Ó Û˘Ìو̷ÙÈÎfi ηٿ ÙË Á¤ÓÓËÛË ‹ ̤۷ ÛÙȘ ÚÒÙ˜ 6 ÒÚ˜, ÁÂÁÔÓfi˜ Ô˘ ·Ô‰ÂÈÎÓ‡ÂÈ fiÙÈ Ë ÂÓ‰ÔÌ‹ÙÚÈ· Ïԛ̈ÍË ÌÔÚ› Ó· Â›Ó·È ˘‡ı˘ÓË ÁÈ· ÌÂÁ¿ÏÔ ÔÛÔÛÙfi ÛÙÚÂÙÔÎÔÎÎÈÎÒÓ ÏÔÈÌÒÍÂˆÓ ÚÒÈÌ˘ ÌÔÚÊ‹˜ (20). ∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ∏ Û˘¯ÓfiÙËÙ· Ù˘ ÊÔÚ›·˜ ÙÔ˘ GBS ÛÙËÓ ∂ÏÏ¿‰· Î˘Ì·›ÓÂÙ·È ÂÚ›Ô˘ ÛÙÔ 6,6% Î·È ·fi Ù· ÛÙÔȯ›· Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÌÈ· ÙÂÏÂ˘Ù·›· ·Ó¿Ï˘ÛË, fiÛÔÓ ·ÊÔÚ¿ ÙÔ˘˜ ÔÚfiÙ˘Ô˘˜, ¤¯Ô˘Ì ٷ ÂÍ‹˜ ÔÛÔÛÙ¿: ÔÚfiÙ˘Ô˜ Ia 19%, ÔÚfiÙ˘Ô˜ Ib 12%, ÔÚfiÙ˘Ô˜ ππ 26,9%, ÔÚfiÙ˘Ô˜ πππ 22,4% Î·È ÔÚfiÙ˘Ô˜ V 9% (21). ∏ Ïԛ̈ÍË ·fi GBS ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ· ÌÔÚ› Ó· ÂΉËψı› ›Ù Ì ÙËÓ ÚÒÈÌË Â›Ù Ì ÙËÓ fi„ÈÌË ¶·È‰È·ÙÚÈ΋ 2009;72:16-22

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·18

18

Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

ÌÔÚÊ‹. ∆· ÓÂÔÁÓ¿ Ì Ïԛ̈ÍË ·fi GBS Û˘Ó‹ıˆ˜ ÂÌÊ·Ó›˙Ô˘Ó ÛË„·ÈÌ›· ‹ Ó¢ÌÔÓ›·, ˆÛÙfiÛÔ ÏÈÁfiÙÂÚÔ Û˘¯Ó¿ ÌÔÚ› Ó· ÂΉËÏÒÛÔ˘Ó ÌËÓÈÁÁ›Ùȉ·, ÔÛÙÂÔÌ˘ÂÏ›Ùȉ· ‹ ÛËÙÈ΋ ·ÚıÚ›Ùȉ·. ∏ ÛË„·ÈÌ›· Ì ‹ ¯ˆÚ›˜ Ó¢ÌÔÓ›·, ·ÔÙÂÏ› ÙËÓ Î‡ÚÈ· ÂΉ‹ÏˆÛË Ù˘ ÓfiÛÔ˘ Î·È ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 90% ÙˆÓ ÂÚÈÙÒÛˆÓ, ÂÓÒ Ë ÌËÓÈÁÁ›Ùȉ· ÂÌÊ·Ó›˙ÂÙ·È ÂÚ›Ô˘ ÛÙÔ 10% (22). ŸÛÔÓ ·ÊÔÚ¿ Ù· ÎÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο Ù˘ ÚÒÈÌ˘ ÓÂÔÁÓÈ΋˜ ÛÙÚÂÙÔÎÔÎÎÈ΋˜ Ïԛ̈͢, ¤¯Ô˘Ì ÁÂÓÈο ÛËÌ›· ‹ Î·È Î·Ù·ÛÙÚÔÊÈΤ˜ ÌÔÚʤ˜ ÛË„·ÈÌ›·˜ Ì ˆ¯ÚfiÙËÙ·, shock, ˘ÔıÂÚÌ›· ‹ ˘ÂÚıÂÚÌ›·, ÔÈΛÏÔ˘ ‚·ıÌÔ‡ ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· Ì ·˘ÍË̤Ó˜ ··ÈÙ‹ÛÂȘ Û Ô͢ÁfiÓÔ, Ó¢ÌÔÓ›·, Û˘¯Ó¿ ·Ú·Ì¤ÓÔ˘Û· Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Î·È ÌËÓÈÁÁ›Ùȉ·, Ô˘ Û ÔÚÈṲ̂Ó˜ ÌÂϤÙ˜ ÊÙ¿ÓÂÈ ÛÙÔ 30%. ∞fi Ù· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ¤¯Ô˘Ì ˘ÔÁÏ˘Î·ÈÌ›·, Ï¢ÎÔ΢ÙÙ¿ÚˆÛË ‹ Ï¢ÎÔÂÓ›· Î·È ·˘ÍË̤ÓË Û¯¤ÛË ¿ˆÚˆÓ ÚÔ˜ ÒÚÈÌˆÓ ÔÏ˘ÌÔÚÊÔ‡ÚËÓˆÓ (5,6,15). ∏ fi„ÈÌË ÌÔÚÊ‹ Ù˘ ÛÙÚÂÙÔÎÔÎÎÈ΋˜ Ïԛ̈͢ ÂÌÊ·Ó›˙ÂÙ·È ÌÂÙ¿ ÙËÓ ÚÒÙË Â‚‰ÔÌ¿‰· Ù˘ ˙ˆ‹˜ Î·È Ì¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 3 ÌËÓÒÓ. ™’ ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË, Ô ÛÙÚÂÙfiÎÔÎÎÔ˜ ÌÔÚ› Ó· ÚÔ¤Ú¯ÂÙ·È Î·È ·fi ÌË ÌËÙÚÈ΋ ËÁ‹. À¿Ú¯ÂÈ ÌÂϤÙË fiÔ˘ ·fi ÙȘ 21 ÂÚÈÙÒÛÂȘ ÓÂÔÁÓÒÓ Ì fi„ÈÌË ÓfiÛÔ, ÌfiÓÔ Û 7 ÓÂÔÁÓ¿ ÔÈ ÌËÙ¤Ú˜ ›¯·Ó ·ÓȯÓ‡ÛÈ̘ ·ÔÈ˘ ÙÔ˘ ‚·ÎÙËÚȉ›Ô˘ ηٿ ÙÔÓ ÙÔÎÂÙfi, ÂÓÒ ÛÙ· ˘fiÏÔÈ· 14 ‰ÂÓ ·ÓȯÓ‡ıËÎ·Ó ·ÔÈ˘ (23). ∏ ÚÒÈÌË ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ ÚÔηÏÂ›Ù·È ·fi ¿ÓÔ‰Ô ÙÔ˘ GBS ÛÙÔ ·ÌÓÈ·Îfi ˘ÁÚfi, Ë ÔÔ›· Ô‰ËÁ› Û ·ÔÈÎÈÛÌfi ÙÔ˘ ÓÂÔÁÓÔ‡ Î·È ÛÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ÓfiÛÔ˘ (24). ∏ ÂÚÈÁÂÓÓËÙÈ΋ ÌÂÙ¿‰ÔÛË ÙÔ˘ ÌÈÎÚÔ‚›Ô˘ ÌÔÚ› Ó· Á›ÓÂÈ ‰È· ̤ÛÔ˘ ›Ù ÂÚÚËÁÌ¤ÓˆÓ Â›Ù ¿ıÈÎÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ˘Ì¤ÓˆÓ (25). ™ÙËÓ fi„ÈÌË ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘, ÙÔ ÈÔ Û˘¯Ófi ¯·Ú·ÎÙËÚÈÛÙÈÎfi Â›Ó·È Ë ÌËÓÈÁÁ›Ùȉ·, Ë ÔÔ›· ηٷÏ›ÂÈ ÌfiÓÈÌ· Ó¢ÚÔÏÔÁÈο ˘ÔÏ›ÌÌ·Ù· Û ÔÛÔÛÙfi 50%. ∏ ÌËÓÈÁÁ›Ùȉ· ÂÌÊ·Ó›˙ÂÙ·È Û Ôχ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÛÙËÓ fi„ÈÌË ÛË„·ÈÌ›· Û ۯ¤ÛË Ì ÙËÓ ÚÒÈÌË Î·È ·˘Ùfi ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· Ô Î›Ó‰˘ÓÔ˜ ÁÈ· Ó¢ÚÔÏÔÁÈο ˘ÔÏ›ÌÌ·Ù· Ó· Â›Ó·È ÌÂÁ·Ï‡ÙÂÚÔ˜ ÛÙËÓ fi„ÈÌË. ∏ ÚfiÁÓˆÛË ÙˆÓ ÓÂÔÁÓÒÓ Ì ÛË„·ÈÌ›· ·fi GBS ¤¯ÂÈ ‚ÂÏÙȈı› ÛËÌ·ÓÙÈο Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·. ¡ÂÔÁÓ¿ Ì ËÏÈΛ· ·ËÛ˘ ≥37 ‚‰ÔÌ¿‰Â˜ ÂÈ‚ÈÒÓÔ˘Ó Û ÔÛÔÛÙfi 98%, ÂÓÒ ÓÂÔÁÓ¿ Ì ËÏÈΛ· ·ËÛ˘ 34-36 ‚‰ÔÌ¿‰Â˜ ÂÈ‚ÈÒÓÔ˘Ó Û ÔÛÔÛÙfi 90%. ŸÛÔÓ ·ÊÔÚ¿ ÓÂÔÁÓ¿ Ì ÌÈÎÚfiÙÂÚË ËÏÈΛ· ·ËÛ˘ (≤33 ‚‰ÔÌ¿‰Â˜), Ë ÂÈ‚›ˆÛË ÏËÛÈ¿˙ÂÈ ÙÔ 70% (26).

¢È¿ÁÓˆÛË ∏ ηÏÏȤÚÁÂÈ· ·›Ì·ÙÔ˜ ·ÔÙÂÏ› ÙËÓ ÈÔ ·ÍÈfiÈÛÙË Ì¤ıÔ‰Ô ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù˘ ÛÙÚÂÙÔÎÔÎÎÈ΋˜ Ïԛ̈͢. ∆Ô ÌfiÓÔ ÌÂÈÔÓ¤ÎÙËÌ· Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ Paediatriki 2009;72:16-22

Â›Ó·È ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·, ‰ÈfiÙÈ ¯ÚÂÈ¿˙ÂÙ·È Ó· ÌÂÛÔÏ·‚‹ÛÔ˘Ó 24-48 ÒÚ˜ ̤¯ÚÈ Ó· Á›ÓÔ˘Ó ÁÓˆÛÙ¿ Ù· ·ÔÙÂϤÛÌ·Ù·. ∏ ηٿ gram ¯ÚÒÛË ‰ÂÓ Â›Ó·È Â·ÚÎÒ˜ ¢·›ÛıËÙË Ì¤ıÔ‰Ô˜ ÁÈ· Ù·¯Â›· ·Ó›¯Ó¢ÛË ÙÔ˘ GBS. ¢È·ÁÓˆÛÙÈο test ÁÈ· ÙËÓ ¿ÌÂÛË Ù·˘ÙÔÔ›ËÛË ÙÔ˘ ÔÏ˘Û·Î¯·ÚȉÈÎÔ‡ ·ÓÙÈÁfiÓÔ˘ ÙÔ˘ GBS, fiˆ˜ Ë Ì¤ıÔ‰Ô˜ Û˘ÁÎfiÏÏËÛ˘ Ì latex Î·È Ë ELISA, ¤¯Ô˘Ó ÌÈÎÚfiÙÂÚË Â˘·ÈÛıËÛ›· Û ۇÁÎÚÈÛË Ì ٷ ·ÓÙ›ÛÙÔȯ· ÙˆÓ Î·ÏÏÈÂÚÁÂÈÒÓ (27). ¶ÚfiÛÊ·Ù·, Ë PCR ¤¯ÂÈ ‰Â›ÍÂÈ ÂÍ·ÈÚÂÙÈο ·ÔÙÂϤÛÌ·Ù·, Ì ¢·ÈÛıËÛ›· Ù˘ Ù¿Í˘ ÙÔ˘ 97% Î·È Û ۇÁÎÚÈÛË Ì ٷ ·ÔÙÂϤÛÌ·Ù· ÙˆÓ Î·ÏÏÈÂÚÁÂÈÒÓ ·›Ì·ÙÔ˜, ÂÓÒ Ô ·Ó·ÊÂÚfiÌÂÓÔ˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¯ÚfiÓÔ˜ Î˘Ì·›ÓÂÙ·È ·Ô 40-100 ÏÂÙ¿. ∞˘Ùfi Â›Ó·È ¤Ó· Ôχ ÛËÌ·ÓÙÈÎfi ÛÙÔȯ›Ô, ÙÔ ÔÔ›Ô ÌÔÚ›, ÌÂÏÏÔÓÙÈο, Ó· ·ÏÏ¿ÍÂÈ ÚÈ˙Èο ÙËÓ ÔÏÈÙÈ΋ ÙÔ˘ ÂϤÁ¯Ô˘ ÙˆÓ ÂÁ·ˆÓ Á˘Ó·ÈÎÒÓ fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ÊÔÚ›· ÙÔ˘ GBS (28-32).

¶ÚfiÏË„Ë ∏ Ïԛ̈ÍË ·fi GBS ÚÔηÏ› ÛÔ‚·Ú‹ ÓfiÛÔ ÛÙ· ÓÂÔÁÓ¿ Î·È ÙÔ ÂӉȷʤÚÔÓ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ÂÈÎÂÓÙÚÒÓÂÙ·È Î˘Ú›ˆ˜ ÛÙË Ï‹„Ë ÚÔÊ˘Ï·ÎÙÈÎÒÓ Ì¤ÙÚˆÓ ÁÈ· ÙËÓ ÚfiÏË„Ë Ù˘ Ïԛ̈͢ ·fi ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ÌÈÎÚfi‚ÈÔ. ∆Ô 1996 Î·È ÙÔ 1997, ÙÔ ∞ÌÂÚÈηÓÈÎfi ∫ÔϤÁÈÔ ª·È¢ÙÈ΋˜ Î·È °˘Ó·ÈÎÔÏÔÁ›·˜, ÙÔ ∫¤ÓÙÚÔ ÁÈ· ÙËÓ ¶ÚfiÏË„Ë Î·È ÙÔÓ ŒÏÂÁ¯Ô ÙˆÓ §ÔÈÌÒÍÂˆÓ Î·È Ë ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ Âͤ‰ˆÛ·Ó Ô‰ËÁ›Â˜ ÁÈ· ÙËÓ ÚfiÏË„Ë Ù˘ Ïԛ̈͢ ·fi GBS (33-35). √È Û˘ÛÙ¿ÛÂȘ ·ÊÔÚÔ‡Û·Ó ÙÔÓ ¤ÏÂÁ¯Ô ÁÈ· ·ÔÈÎÈÛÌfi Ì GBS ÙˆÓ ÂÈÙfiÎˆÓ ‹ ÙËÓ ÚÔÛ¤ÁÁÈÛË Ì ‚¿ÛË ÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ÂÌÊ¿ÓÈÛ˘ Ù˘ Ïԛ̈͢. ™‡Ìʈӷ Ì’ ·˘Ù¤˜ ÙȘ Ô‰ËÁ›Â˜, fiϘ ÔÈ Â›ÙÔΘ ÌÂٷ͇ 35˘ Î·È 37˘ ‚‰ÔÌ¿‰·˜ ·ËÛ˘ ÂϤÁ¯ÔÓÙ·È ÁÈ· ·ÔÈÎÈÛÌfi ÙÔ˘ ÎfiÏÔ˘ Î·È ÙÔ˘ ÚˆÎÙÔ‡ Ì GBS. ™ÙȘ ·ÔÈÎÈṲ̂Ó˜ Á˘Ó·›Î˜ ¯ÔÚËÁÂ›Ù·È ·ÓÙÈÌÈÎÚԂȷ΋ ·ÁˆÁ‹ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ÙÔÎÂÙÔ‡. ŸÛÔÓ ·ÊÔÚ¿ ÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘, ·ÍÈÔÏÔÁ‹ıËÎ·Ó Ô ÚfiˆÚÔ˜ ÙÔÎÂÙfi˜ (‰È¿ÚÎÂÈ· ·ËÛ˘ <37 ‚‰ÔÌ¿‰Â˜), Ë Ú‹ÍË ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ˘Ì¤ÓˆÓ >18 ÒÚ˜ Î·È Ë ˘ÚÂÙÈ΋ ΛÓËÛË Ù˘ ÂÈÙfiÎÔ˘ (≥38ÔC). ∞fi Ù· ÛÙÔȯ›· Ô˘ ‰ËÌÔÛȇıËÎ·Ó ·fi ÙȘ ‰‡Ô ÛÙÚ·ÙËÁÈΤ˜ ÚÔÛ¤ÁÁÈÛ˘, Ê·›ÓÂÙ·È fiÙÈ ÂÈÙ‡¯ıËΠÛËÌ·ÓÙÈ΋ Ì›ˆÛË Ù˘ ÓÂÔÁÓÈ΋˜ ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÛÈÌfiÙËÙ·˜ ·fi GBS, ¯ˆÚ›˜ ˆÛÙfiÛÔ Ó· ˘¿Ú¯ÂÈ ¿ÌÂÛË Û‡ÁÎÚÈÛË Û ÌÂÁ¿Ï˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ (36). ¶ÚfiÛÊ·ÙË ÎÏÈÓÈ΋ ÌÂϤÙË ÛÙȘ ∏¶∞ ¤‰ÂÈÍ fiÙÈ Ë ÔÏÈÙÈ΋ ·ÓÙÈÌÂÙÒÈÛ˘ Ì ‚¿ÛË ÙÔÓ ·ÔÈÎÈÛÌfi ‹Ù·Ó ηٿ 50% ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ·fi ÙËÓ ÔÏÈÙÈ΋ ÚÔÛ¤ÁÁÈÛ˘ Ì ‚¿ÛË ÙËÓ ·˘ÍË̤ÓË Èı·ÓfiÙËÙ· ÎÈÓ‰‡ÓÔ˘ (37). ªÂ ‚¿ÛË Ù· Ó¤· ÛÙÔȯ›·, ÙÔ ∫¤ÓÙÚÔ ∂ϤÁ¯Ô˘ Î·È ¶ÚfiÏ˄˘ ÙˆÓ §ÔÈÌÒÍÂˆÓ ·Ó·ıÂÒÚËÛ ÙȘ Ô‰ËÁ›Â˜ ÙÔ˘ 1996 Î·È ·fi ÙÔÓ ∞‡ÁÔ˘ÛÙÔ ÙÔ˘ 2002 Û˘ÓÈÛÙ¿Ù·È Ô Î·ıÔÏÈÎfi˜ ¤ÏÂÁ¯Ô˜

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·19

19

¡ÂÔÁÓÈΤ˜ ÏÔÈÌÒÍÂȘ ·fi µ-·ÈÌÔÏ˘ÙÈÎfi ÛÙÚÂÙfiÎÔÎÎÔ Ù˘ ÔÌ¿‰·˜ µ

fiÏˆÓ ÙˆÓ ÂÁ·ˆÓ ÁÈ· Ù˘¯fiÓ ·ÔÈÎÈÛÌfi ÙÔ˘˜ ·fi GBS Î·È ÌfiÓÔ ÛÙËÓ ÂÚ›ÙˆÛË fiÔ˘ Ù· ·ÔÙÂϤÛÌ·Ù· ‰ÂÓ Â›Ó·È ‰È·ı¤ÛÈÌ· ÚÔ ÙÔ˘ ÙÔÎÂÙÔ‡ Ï·Ì‚¿ÓÂÙ·È ˘fi„Ë Ô ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘ (37). ªÂÙ¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙˆÓ Ó¤ˆÓ Ô‰ËÁÈÒÓ, ¤Ï·‚ ¯ÒÚ· ÌÈ· ÌÂϤÙË Ì ÛÎÔfi ÙËÓ ÂÍ·ÁˆÁ‹ Û˘ÌÂÚ·ÛÌ¿ÙˆÓ Û¯ÂÙÈο Ì ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ Ó¤ˆÓ Ô‰ËÁÈÒÓ, fiÛÔÓ ·ÊÔÚ¿ ÙË Ïԛ̈ÍË ·fi GBS. ™˘ÁÎÂÎÚÈ̤ӷ, ¤ÁÈÓ ۇÁÎÚÈÛË ÌÂٷ͇ Ù˘ ÂÚÈfi‰Ô˘ 2003-2005 (ÌÂÙ¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙˆÓ Ô‰ËÁÈÒÓ) Î·È Ù˘ ÂÚÈfi‰Ô˘ 2000-2001 (ÚÈÓ ÙËÓ ÂÊ·ÚÌÔÁ‹). ∏ Û˘¯ÓfiÙËÙ· Ù˘ ÚÒÈÌ˘ ÌÔÚÊ‹˜ Ù˘ ÛÙÚÂÙÔÎÔÎÎÈ΋˜ Ïԛ̈͢ ‹Ù·Ó ηٿ 33% ¯·ÌËÏfiÙÂÚË Î·Ù¿ ÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô 2003-2005, Û ۯ¤ÛË Ì ÙËÓ ÂÚ›Ô‰Ô 2000-2001. ŸÛÔÓ ·ÊÔÚ¿ ÙË Û˘¯ÓfiÙËÙ· Ù˘ fi„ÈÌ˘ ÌÔÚÊ‹˜, ‰ÂÓ ˘‹Ú¯Â η̛· ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ (38). ∂ÓÒ Ë Û˘¯ÓfiÙËÙ· Ù˘ ÚÒÈÌ˘ ÌÔÚÊ‹˜ Ù˘ ÛÙÚÂÙÔÎÔÎÎÈ΋˜ Ïԛ̈͢ Â›Ó·È ÙÔ˘Ï¿¯ÈÛÙÔÓ 20 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË ·fi ÙË Û˘¯ÓfiÙËÙ· ÙÔ˘ ÓÂÔÁÓÈÎÔ‡ ¤ÚËÙ·, Ù˘ Û‡ÊÈÏ˘ Î·È Ù˘ ÂÚ˘ıÚ¿˜, ÂÓÙÔ‡ÙÔȘ ‰ÂÓ ˘¿Ú¯ÂÈ Ì¤¯ÚÈ Û‹ÌÂÚ· ÙÔ˘Ï¿¯ÈÛÙÔÓ Ô ·Ó¿ÏÔÁÔ˜ ÂÓıÔ˘ÛÈ·ÛÌfi˜ ÁÈ· ÚÔÁÂÓÓËÙÈο screening tests ÁÈ· Ïԛ̈ÍË ·fi GBS, fiˆ˜ Û˘Ì‚·›ÓÂÈ Ì ÙȘ ÚÔ·Ó·ÊÂÚfiÌÂÓ˜ ÏÔÈÌÒÍÂȘ. √ ηχÙÂÚÔ˜ ¯ÚfiÓÔ˜ ÁÈ· ¤ÏÂÁ¯Ô Ù˘ ÂÈÙfiÎÔ˘ Â›Ó·È Ë 28Ë Â‚‰ÔÌ¿‰· ·ËÛ˘ Î·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ¿ Ù˘ Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÔÓÙ·È Î·ÏÏȤÚÁÂȘ ·fi ÙÔÓ ÎfiÏÔ Î·È ÙÔÓ ÚˆÎÙfi. ™Â ÂÚ›ÙˆÛË Ô˘ Á›ÓÂÈ Ï‹„Ë Î·ÏÏȤÚÁÂÈ·˜ ÌfiÓÔ ÁÈ· Ì›· ·fi ÙȘ ‰‡Ô ÂÚÈÔ¯¤˜, ÙÔ ·ÔÙ¤ÏÂÛÌ· ÌÔÚ› Ó· Â›Ó·È „¢‰Ò˜ ·ÚÓËÙÈÎfi -fiÛÔÓ ·ÊÔÚ¿ ÙË ÊÔÚ›· Ù˘ ÂÈÙfiÎÔ˘- Û ÔÛÔÛÙfi ̤¯ÚÈ Î·È 25%. ŸÙ·Ó ˘¿Ú¯ÂÈ ·ÔÈÎÈÛÌfi˜ Ù˘ ÌËÙ¤Ú·˜, Û˘ÓÈÛÙ¿Ù·È Ë ¯ÔÚ‹ÁËÛË ¯ËÌÂÈÔÚÔʇϷ͢ ÙÔ˘Ï¿¯ÈÛÙÔÓ 4 ÒÚ˜ ÚÔ ÙÔ˘ ÙÔÎÂÙÔ‡, ÒÛÙ ӷ ÂÙ‡¯Ô˘Ì ÈηÓÔÔÈËÙÈο ıÂÚ·¢ÙÈο Â›‰·. øÛÙfiÛÔ, ÂÂȉ‹ ˘¿Ú¯ÂÈ Èı·ÓfiÙËÙ· ÙÔ ¤Ì‚Ú˘Ô Ó· ¤¯ÂÈ ÌÔÏ˘Óı› ÂÓ‰ÔÌ‹ÙÚÈ·, Ë Ì¤ıÔ‰Ô˜ ·˘Ù‹ ‰ÂÓ ÂÍ·ÛÊ·Ï›˙ÂÈ ¿ÓÙ· ηϋ ÚfiÁÓˆÛË (20). √È ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ· ·˘ÍË̤ÓË Èı·ÓfiÙËÙ· ÂÚÈÁÂÓÓËÙÈ΋˜ ÛÙÚÂÙÔÎÔÎÎÈ΋˜ Ïԛ̈͢ ›ӷÈ: Ô ÚfiˆÚÔ˜ ÙÔÎÂÙfi˜ (<37 ‚‰ÔÌ¿‰Â˜), Ë ÚfiˆÚË Ú‹ÍË ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ˘Ì¤ÓˆÓ (<37 ‚‰ÔÌ¿‰Â˜), Ë ·Ú·ÙÂٷ̤ÓË Ú‹ÍË ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ˘Ì¤ÓˆÓ (≥18 ÒÚ˜), Ô ˘ÚÂÙfi˜ Ù˘ ÌËÙ¤Ú·˜ (≥38,4ÔC Û ‰‡Ô ˘ÚÂÙÈο ·̷ٷ), ÙÔ ÈÛÙÔÚÈÎfi ÚÔËÁÔ‡ÌÂÓ˘ ·ËÛ˘ Ô˘ ›¯Â ÂÈϷΛ Ì Ïԛ̈ÍË ·fi GBS, Ë Ô˘ÚÔÏԛ̈ÍË Ù˘ ÌËÙ¤Ú·˜ ·fi GBS, Ë ¯ÔÚÈÔ·ÌÓÈÔÓ›Ùȉ· Î·È Ë Ôχ‰˘ÌË Î‡ËÛË (39,40).

∞ÓÙÈÌÂÙÒÈÛË ™Â οı ÚfiˆÚÔ ÓÂÔÁÓfi Ì ÛÔ‚·Ú‹ ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· Ë ÔÔ›· ÂΉËÏÒÓÂÙ·È ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, Ô ·È‰›·ÙÚÔ˜ Â›Ó·È ˘Ô¯Úˆ̤ÓÔ˜ ÛÙË ‰È·-

ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ó· Û˘ÌÂÚÈÏ¿‚ÂÈ Î·È ÙË ÛÙÚÂÙÔÎÔÎÎÈ΋ Ïԛ̈ÍË. ∏ ÎÏÈÓÈ΋ Î·È Ë ·ÎÙÈÓÔÏÔÁÈ΋ ÂÈÎfiÓ· Ù˘ Ïԛ̈͢ ·fi GBS Â›Ó·È ·ÚfiÌÔÈ· Ì ÙÔ Û‡Ó‰ÚÔÌÔ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜ Î·È ÂÔ̤ӈ˜ Ú¤ÂÈ Ó· ¯ÔÚËÁÂ›Ù·È ÂÌÂÈÚÈ΋ ·ÓÙÈÌÈÎÚԂȷ΋ ·ÁˆÁ‹ ̤¯ÚȘ fiÙÔ˘ Ó· ÁÓˆÛÙÔÔÈËıÔ‡Ó Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ Î·ÏÏÈÂÚÁÂÈÒÓ (41). ŸÙ·Ó ˘¿Ú¯Ô˘Ó ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ Î·È Ì¤¯ÚÈ Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ Î·ÏÏÈÂÚÁÂÈÒÓ ·›Ì·ÙÔ˜ Ó· Â›Ó·È ‰È·ı¤ÛÈÌ·, Ù· ÓÂÔÁÓ¿ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ·ÓÙÈ‚ÈoÙÈ΋ ·ÁˆÁ‹. ∆· ÓÂÔÁÓ¿ ÙˆÓ ÔÔ›ˆÓ ÔÈ ÌËÙ¤Ú˜ ¤Ï·‚·Ó ¯ËÌÂÈÔÚÔʇϷÍË Ú¤ÂÈ Ó· ıÂÚ·‡ÔÓÙ·È Û‡Ìʈӷ ÌÂ Û˘ÁÎÂÎÚÈ̤Ó˜ Û˘ÛÙ¿ÛÂȘ. ™Â Û˘Ìو̷ÙÈο ÓÂÔÁÓ¿ ¯ÔÚËÁÔ‡ÓÙ·È ·ÓÙÈ‚ÈÔÙÈο Â˘Ú¤Ô˜ Ê¿ÛÌ·ÙÔ˜ ̤¯ÚȘ fiÙÔ˘ ÁÓˆÛÙÔÔÈËıÔ‡Ó Ù· ·ÔÙÂϤÛÌ·Ù· ÙˆÓ Î·ÏÏÈÂÚÁÂÈÒÓ. ∆· ·Û˘Ìو̷ÙÈο ÙÂÏÂÈfiÌËÓ· ÓÂÔÁÓ¿, ÙˆÓ ÔÔ›ˆÓ ÔÈ ÌËÙ¤Ú˜ ¤Ï·‚·Ó ÂÓÈÎÈÏÏ›ÓË ‹ ·ÌÈÎÈÏÏ›ÓË ÙÔ˘Ï¿¯ÈÛÙÔÓ 4 ÒÚ˜ ÚÔ ÙÔ˘ ÙÔÎÂÙÔ‡, ‰ÂÓ ¯ÚÂÈ¿˙ÔÓÙ·È ÂÚ·ÈÙ¤Úˆ ıÂÚ·›·. ∆· ·Û˘Ìو̷ÙÈο ÚfiˆÚ· (¢∫ ≤34 ‚‰ÔÌ¿‰Â˜), ηıÒ˜ Î·È ÂΛӷ ·ÔÈÎÈÛÌ¤ÓˆÓ ÌËÙ¤ÚˆÓ, ıÂÚ·‡ÔÓÙ·È ÂÌÂÈÚÈο ÙÔ˘Ï¿¯ÈÛÙÔÓ ÁÈ· 72 ÒÚ˜. ∞Ó ÔÈ Î·ÏÏȤÚÁÂȘ Â›Ó·È ÙÂÏÈÎÒ˜ ıÂÙÈΤ˜ ‹ Ù· ÓÂÔÁÓ¿ ÂÌÊ·Ó›˙Ô˘Ó ÎÏÈÓÈο ÛËÌ›·, Ë ıÂÚ·›· Û˘Ó¯›˙ÂÙ·È. ™˘ÁÎÂÎÚÈ̤ӷ, ·Ó ˘¿Ú¯ÂÈ ÌfiÓÔ ÛË„·ÈÌ›· ¯ˆÚ›˜ ÌËÓÈÁÁ›Ùȉ·, Ë ıÂÚ·›· Â›Ó·È ÂÓ‰ÔÊϤ‚È· ÂÓÈÎÈÏÏ›ÓË ÁÈ· ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· 10 ËÌÂÚÒÓ, ÂÓÒ ·Ó ˘¿Ú¯ÂÈ ÌËÓÈÁÁ›Ùȉ· Ë ıÂÚ·›· Â›Ó·È 14 Ë̤Ú˜ ÂÓ‰ÔÊϤ‚È· ÂÓÈÎÈÏÏ›ÓË (42) (¶›Ó·Î·˜ 1). À¿Ú¯ÂÈ ¤ÓÙÔÓË ‰È¯ÔÁӈ̛· ÛÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›· fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ÂÚÈÁÂÓÓËÙÈ΋ ıÂÚ·›· ÙˆÓ ÌËÙ¤ÚˆÓ Ô˘ Â›Ó·È ·ÔÈÎÈṲ̂Ó˜ Ì GBS. ∏ ÂÚÈÁÂÓÓËÙÈ΋ ¯ËÌÂÈÔÚÔʇϷÍË ÌÂÈÒÓÂÈ ÙË ÓÂÔÁÓÈ΋ Î·È ÌËÙÚÈ΋ ÓÔÛËÚfiÙËÙ· ÌfiÓÔ fiÙ·Ó ˘¿Ú¯Ô˘Ó ÌËÙÚÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘. øÛÙfiÛÔ, ˘¿Ú¯Ô˘Ó ÌÂϤÙ˜ ÔÈ Ôԛ˜ ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ Ë ÂÚÈÁÂÓÓËÙÈ΋ ¯ËÌÂÈÔÚÔʇϷÍË ÌÂÈÒÓÂÈ ÛËÌ·ÓÙÈο ÙË Ïԛ̈ÍË ·fi GBS, ·ÎfiÌË Î·È Û ·Ô˘Û›· ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ (43). µÂ‚·›ˆ˜, Ù· ıÂÙÈο ·ÔÙÂϤÛÌ·Ù· ·fi ÙËÓ ÂÎÏÂÎÙÈ΋ ¯ËÌÂÈÔÚÔʇϷÍË ı· Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÔÓÙ·È ˘fi„Ë Û ۇÁÎÚÈÛË Ì ÙËÓ Èı·ÓfiÙËÙ· ·ÏÏÂÚÁÈ΋˜ ·ÓÙ›‰Ú·Û˘ ‹ ·ÎfiÌË Î·È ÙËÓ ·Ó¿Ù˘ÍË ÂÓfi˜ ·ÓıÂÎÙÈÎÔ‡ ÏËı˘ÛÌÔ‡.

™˘ÌÂÚ¿ÛÌ·Ù· ∏ ‰È¿ÁÓˆÛË Î·È Ë ıÂÚ·›· Ù˘ ÂÚÈÁÂÓÓËÙÈ΋˜ Ïԛ̈͢ ·fi GBS Û˘Ó¯›˙ÂÈ Ó· ·ÔÙÂÏ› ¤Ó· ÌÂÁ¿ÏÔ Úfi‚ÏËÌ·. ¡¤Â˜ ‰ÔÎÈ̷ۛ˜ ÁÈ· ÙËÓ ¤ÁηÈÚË ·Ó›¯Ó¢ÛË ÙÔ˘ ÛÙÚÂÙfiÎÔÎÎÔ˘ ÚÔÛ‰›‰Ô˘Ó ÂÏ›‰Â˜ ÁÈ· ηχÙÂÚË ÚfiÁÓˆÛË. øÛÙfiÛÔ, ‰ÂÓ ¤¯Ô˘Ó Á›ÓÂÈ ·ÎfiÌË Î·Ù·ÓÔËÙÔ› ÔÈ Ì˯·ÓÈÛÌÔ› Ì ÙÔ˘˜ ÔÔ›Ô˘˜ οÔÈÔÈ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ› ÚÔηÏÔ‡Ó Ïԛ̈ÍË, ÂÓÒ ¿ÏÏÔÈ ÚÔηÏÔ‡Ó ÌfiÓÔ ·Û˘Ìو̷ÙÈÎfi ·ÔÈÎÈÛÌfi. ¶·È‰È·ÙÚÈ΋ 2009;72:16-22

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·20

20

Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

¶›Ó·Î·˜ 1. ∞ÏÁfiÚÈıÌÔ˜ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ÓÂÔÁÓÔ‡ ∞ÓÙÈ‚ÈÔÙÈ΋ ÚÔʇϷÍË ÛÙË ÌËÙ¤Ú· ÁÈ· GBS1

∞ÓÙÈ‚›ˆÛË ÛÙË ÌËÙ¤Ú· ÁÈ· Èı·Ó‹ ¯ÔÚÈÔ·ÌÓÈÔÓ›Ùȉ·

¡∞π

¡∞π ¡∞π

™ËÌ›· ÓÂÔÁÓÈ΋˜ ÛË„·ÈÌ›·˜

¶Ï‹Ú˘ ¤ÏÂÁ¯Ô˜2 ∂ÌÂÈÚÈ΋ ıÂÚ·›·3

√Ãπ ∏ÏÈΛ· ·ËÛ˘

<35 ‚‰.

≥35 ‚‰. ¢È¿ÚÎÂÈ· ÚÔʇϷ͢ ÚÔ ÙÔÎÂÙÔ‡4

<4 ÒÚ˜

¶ÂÚÈÔÚÈṲ̂ÓË ·ÍÈÔÏfiÁËÛË5 ¶·Ú·Ù‹ÚËÛË ≥ 48 ÒÚ˜ ™Â ˘Ô„›· ÛË„·ÈÌ›·˜, Ï‹Ú˘ ‰È·ÁÓˆÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜2 Î·È ÂÌÂÈÚÈ΋ ıÂÚ·›·3

≥ 4 ÒÚ˜ Ÿ¯È ·ÍÈÔÏfiÁËÛË Ÿ¯È ıÂÚ·›· ¶·Ú·Ù‹ÚËÛË ≥48 ÒÚ˜6 1

∂¿Ó ‰ÂÓ ¤¯ÂÈ ÚÔËÁËı› ÚÔÊ˘Ï·ÎÙÈ΋ ·ÓÙÈ‚ÈÔÙÈ΋ ¯ÔÚ‹ÁËÛË ÛÙË ÌËÙ¤Ú·, ·Ú¿ ÙȘ ÂӉ›ÍÂȘ, ˘¿Ú¯Ô˘Ó ·ÓÂ·Ú΋ ÛÙÔȯ›· ÁÈ· ÙË Û˘ÓÈÛÙÒÌÂÓË ·ÓÙÈÌÂÙÒÈÛË.

2 ¶ÂÚÈÏ·Ì‚¿ÓÂÈ ÁÂÓÈ΋ ·›Ì·ÙÔ˜, ηÏÏȤÚÁÂÈ· ·›Ì·ÙÔ˜ Î·È ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ Â¿Ó ˘¿Ú¯ÂÈ ·Ó·Ó¢ÛÙÈ΋ ·ÓÂ¿ÚÎÂÈ·. ∂¿Ó ˘¿Ú¯Ô˘Ó ÛËÌ›· ÛË„·ÈÌ›·˜, Û˘ÓÈÛÙ¿Ù·È Î·È ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙËÛË.

3 ∏ ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ ÂÍ·ÚÙ¿Ù·È ·fi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ηÏÏȤÚÁÂÈ·˜ ·›Ì·ÙÔ˜ Î·È Ù˘ ηÏÏȤÚÁÂÈ·˜ ∂¡À, ηıÒ˜ Î·È ·fi ÙËÓ ÎÏÈÓÈ΋ ÔÚ›· ÙÔ˘ ÓÂÔÁÓÔ‡. ∂¿Ó Ù· ·ÔÙÂϤÛÌ·Ù· ‰ÂÓ ‰Â›¯ÓÔ˘Ó ÌÈÎÚԂȷ΋ ÌfiÏ˘ÓÛË, Ë ıÂÚ·›· ‰È·ÚΛ 48 ÒÚ˜.

4 ∞ÊÔÚ¿ ¯ÔÚ‹ÁËÛË ÂÓÈÎÈÏÏ›Ó˘, ·ÌÈÎÈÏÏ›Ó˘ ‹ ÎÂÊ·˙ÔÏ›Ó˘.

5 °ÂÓÈ΋ ·›Ì·ÙÔ˜ Î·È Î·ÏÏȤÚÁÂÈ· ·›Ì·ÙÔ˜.

6 ŒÓ· Ê·ÈÓÔÌÂÓÈο ˘ÁȤ˜ ÓÂÔÁÓfi, ÙÔ ÔÔ›Ô ÁÂÓÓ‹ıËΠ̠ËÏÈΛ· ·ËÛ˘ ≥36 ‚‰ÔÌ¿‰Â˜ Î·È ÙÔ˘ ÔÔ›Ô˘ Ë ÌËÙ¤Ú· ¤¯ÂÈ Ï¿‚ÂÈ ·ÓÙÈ‚ÈÔÙÈ΋ ·ÁˆÁ‹ ≥4 ÒÚ˜ ÚÔ ÙÔ˘ ÙÔÎÂÙÔ‡, ÌÔÚ› Ó· ¿ÚÂÈ ÂÍÈÙ‹ÚÈÔ ÌÂÙ¿ ·fi 24 ÒÚ˜ ·Ú·ÎÔÏÔ‡ıËÛ˘, Â¿Ó ÏËÚÔ‡ÓÙ·È ÔÈ ÚÔ¸Ôı¤ÛÂȘ ÁÈ· ÂÍÈÙ‹ÚÈÔ Î·È ˘¿Ú¯ÂÈ ¤Ó· ¿ÙÔÌÔ ÈηÓfi Ó· ·ÎÔÏÔ˘ı‹ÛÂÈ Ï‹Úˆ˜ ÙȘ Ô‰ËÁ›Â˜. ™Â ¿ÏÏË ÂÚ›ÙˆÛË, ı· Ú¤ÂÈ Ó· ·Ú·Ì›ÓÂÈ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 48 ÒÚ˜.

ÕÏψÛÙÂ, ÌfiÓÔ ÙÔ 1-2% ÙˆÓ ÓÂÔÁÓÒÓ Ù· ÔÔ›· ÁÂÓÓÈÔ‡ÓÙ·È ·fi ·ÔÈÎÈṲ̂Ó˜ ÌËÙ¤Ú˜ ÂΉËÏÒÓÔ˘Ó ÎÏÈÓÈο ÓfiÛÔ (44). ªÂÁ¿ÏË Û˘˙‹ÙËÛË Á›ÓÂÙ·È Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ÔÚÈ˙fiÓÙÈ· ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘, ÛÙËÓ ÔÔ›· ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ·›˙ÂÈ Î·È Ë ¯ˆÚÔÙ·ÍÈ΋ ÔÚÁ¿ÓˆÛË ÙˆÓ ÓÂÔÁÓÔÏÔÁÈÎÒÓ ÙÌËÌ¿ÙˆÓ. ŸÙ·Ó ÓÂÔÁÓ¿ ·ÔÈÎÈṲ̂ӷ ÙÔÔıÂÙ‹ıËÎ·Ó ÎÔÓÙ¿ Û ÌË ·ÔÈÎÈṲ̂ӷ, ·Ú·ÙËÚ‹ıËΠfiÙÈ ÙÔ ÔÛÔÛÙfi ÌÂÙ¿‰ÔÛ˘ ·fi ÓÂÔÁÓfi Û ÓÂÔÁÓfi ‹Ù·Ó ·ÚÎÂÙ¿ ˘„ËÏfi. ∏ ˘„ËÏ‹ ˘ÎÓfiÙËÙ· ÓÂÔÁÓÈÎÔ‡ ÏËı˘ÛÌÔ‡, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ۯÂÙÈο Ï›ÁÔ ÓÔÛËÏ¢ÙÈÎfi ÚÔÛˆÈÎfi, ¢ÓÔ› ÙË ÌÂÙ¿‰ÔÛË Ù˘ ÓfiÛÔ˘ (45). ∂Âȉ‹ Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÏÔÈÌÒÍÂˆÓ ·fi GBS ÛÙȘ ¤ÁÎ˘Â˜ Î·È ÛÙ· ÓÂÔÁÓ¿ ·ÊÔÚ¿ Û˘ÁÎÂÎÚÈ̤ÓÔ˘˜ ÔÚfiÙ˘Ô˘˜, Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· Á›ÓÂÙ·È ÚÔÛ¿ıÂÈ· ·fi ÙÔ˘˜ ÂÚ¢ÓËÙ¤˜ ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· ÂÓfi˜ ηÈÓÔ‡ÚÁÈÔ˘ ÂÌ‚ÔÏ›Ô˘. ∏ Â›Ù¢ÍË ·˘ÙÔ‡ ÙÔ˘ ÛÙfi¯Ô˘ ı· ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ó¿Ù˘ÍË Ó¤ˆÓ Paediatriki 2009;72:16-22

ÛÙÚ·ÙËÁÈÎÒÓ Ô˘ ı· ÚÔÛʤÚÔ˘Ó ÌÂÁ·Ï‡ÙÂÚË ÚÔÛÙ·Û›· ·fi ÙËÓ ÚÒÈÌË Î·È ÙËÓ fi„ÈÌË ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘. ∂ȉÈÎfiÙÂÚ·, Ô ÂÌ‚ÔÏÈ·ÛÌfi˜ ÌÔÚ› Ó· ‚ÔËı‹ÛÂÈ ÛÙËÓ ÚfiÏË„Ë Î¿ÔÈˆÓ ·ÓÂÈı‡ÌËÙˆÓ Î·Ù·ÛÙ¿ÛÂˆÓ ÔÈ Ôԛ˜ Û¯ÂÙ›˙ÔÓÙ·È Ì Ïԛ̈ÍË ·fi GBS, fiˆ˜ Ô ÚfiˆÚÔ˜ ÙÔÎÂÙfi˜ Î·È Ë ·Ô‚ÔÏ‹ (46-50). ∏ ÛË„·ÈÌ›· ‹ Î·È Ë ÌËÓÈÁÁ›Ùȉ· ·fi GBS Â›Ó·È ÌÈ· Ôχ ÛÔ‚·Ú‹ ÏÔÈÌ҉˘ ηٿÛÙ·ÛË ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·. ∏ Û˘¯ÓfiÙËÙ· Ù˘ ÓÂÔÁÓÈ΋˜ ÛÙÚÂÙÔÎÔÎÎÈ΋˜ Ïԛ̈͢ ÔÈΛÏÏÂÈ ·fi 0,5-3 ÂÚÈÙÒÛÂȘ ·Ó¿ ¯›ÏȘ ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ÓÂÔÁÓÒÓ (51). øÛÙfiÛÔ, ˘¿Ú¯ÂÈ Û·Ê‹˜ Ù¿ÛË ÁÈ· Ì›ˆÛË Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ ÚÒÈÌ˘ ÌÔÚÊ‹˜ ÌÂÙ¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙˆÓ ÂÚÈÁÂÓÓËÙÈÎÒÓ ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ (52). ¶ÚÔ·ÙÂÈ, Û˘ÓÂÒ˜, fiÙÈ Ë ·Ó·ÁÓÒÚÈÛË ÙˆÓ ÌËÙÚÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘ (Ïԛ̈ÍË Ô˘ÚÔÔÈËÙÈÎÔ‡ ·fi GBS, ÚÔËÁÔ‡ÌÂÓÔ ÓÂÔÁÓfi Ì Ïԛ̈ÍË ·fi GBS, ¯ÔÚÈÔ·ÌÓÈÔÓ›Ùȉ· ‹ ˘ÚÂÙfi˜ ÂÚÈÁÂÓÓËÙÈο, ·Ú·ÙÂٷ̤ÓË

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¡ÂÔÁÓÈΤ˜ ÏÔÈÌÒÍÂȘ ·fi µ-·ÈÌÔÏ˘ÙÈÎfi ÛÙÚÂÙfiÎÔÎÎÔ Ù˘ ÔÌ¿‰·˜ µ

Ú‹ÍË ÂÌ‚Ú˘˚ÎÒÓ ˘Ì¤ÓˆÓ >18 ÒÚ˜, ÚÔˆÚfiÙËÙ· Î·È ÊÔÚ›· ÙÔ˘ GBS) ‰È·‰Ú·Ì·Ù›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÚfiÏË„Ë Ù˘ ·Ó¿Ù˘Í˘ ÚÒÈÌ˘ ÌÔÚÊ‹˜ Ïԛ̈͢ ·fi GBS Û ÓÂÔÁÓ¿ (53). ∆¤ÏÔ˜, ÛËÌ·ÓÙÈÎfi ÚfiÏÔ Î·Ù¤¯ÂÈ Î·È Ë ÂÚÈÁÂÓÓËÙÈ΋ ·ÛÊ˘Í›·, fiˆ˜ ÚÔ·ÙÂÈ ·fi ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ (54-57). øÛÙfiÛÔ, Â›Ó·È ··Ú·›ÙËÙÔ Ó· ·ÍÈÔÏÔÁËıÔ‡Ó Î·È Ó· ÚÔÛ‰ÈÔÚÈÛÙÔ‡Ó Èı·ÓÔ› Ó¤ÔÈ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙË ÓÂÔÁÓÈ΋ ÓfiÛÔ ·fi GBS (40).

BÈ‚ÏÈÔÁÚ·Ê›· 1. Brown JH. Cultural differentiation of beta-hemolytic Streptococci of human and bovine sources. J Exp Med 1920;31:35-45.

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26. Schrag SJ, Zywicki S, Farley MM, Reingold AL, Harrison LH, Lefkowitz LB, et al. Group B streptococcal disease in the era of intrapartum antibiotic prophylaxis. N Engl J Med 2000;342:15-20.

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29. Samadi R, Stek A, Greenspoon JS. Evaluation of a rapid optical immunoassay-based test for group B streptococcus colonization in intrapartum patients. J Matern Fetal Med 2001;10:203-208.

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31. Chan KL, Levi K, Towner KJ, Weston VC, Ramsay MM, ¶·È‰È·ÙÚÈ΋ 2009;72:16-22

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Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

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Paediatriki 2009;72:16-22

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52. Schrag SJ, Zywicki S, Farley MM, Reingold AL, Harrison LH, Lefkowitz LB, et al. Group B streptococcal disease in the era of intrapartum antibiotic prophylaxis. N Engl J Med 2000;342:15-20.

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57. Ledger WJ. Perinatal infections and fetal/neonatal brain injury. Curr Opin Obstet Gynecol 2008;20:120-124.

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¶ÔÏ˘Û·Î¯·ÚȉÈο ·ÓÙÈÁfiÓ· – Û˘˙¢Á̤ӷ ÂÌ‚fiÏÈ·: ·ÓÔÛȷ΋ ÌÓ‹ÌË Î·È ·Ó·ÌÓËÛÙÈΤ˜ ‰fiÛÂȘ π.£. ∫·‚·ÏÈÒÙ˘ ¶ÂÚ›ÏË„Ë: ™˘˙ËÙÔ‡ÓÙ·È Ù· ÚÔ‚Ï‹Ì·Ù· ÙˆÓ ÔÏ˘Û·Î¯·ÚȉÈÎÒÓ ·ÓÙÈÁfiÓˆÓ, ÙÔÓ›˙ÔÓÙ·È Ù· ÌÂÈÔÓÂÎÙ‹Ì·Ù¿ ÙÔ˘˜, ·Ó·Ï‡ÂÙ·È Ë ·ÓÔÛȷ΋ ·¿ÓÙËÛË ÛÙ· ·ÓÙÈÁfiÓ· ·˘Ù¿ Î·È ·Ó·Ê¤ÚÔÓÙ·È Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· ÙˆÓ Û˘˙¢ÁÌ¤ÓˆÓ ·ÓÙÈÁfiÓˆÓ-ÂÌ‚ÔÏ›ˆÓ. ªÂ ‚¿ÛË Ù· ·Ú·¿Óˆ, Á›ÓÔÓÙ·È ÛΤ„ÂȘ ÁÈ· ÚÔÛı‹ÎË ·Ó·ÌÓËÛÙÈÎÒÓ ‰fiÛÂˆÓ ÛÙ· Û˘ÁÎÂÎÚÈ̤ӷ ÂÌ‚fiÏÈ·. §¤ÍÂȘ ÎÏÂȉȿ: ∂Ì‚fiÏÈ·, ·ÓÔÛ›·, ·È‰È¿.

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ∂ȉÈÎÒÓ ¶·ı‹ÛÂˆÓ £ÂÛÛ·ÏÔӛ΢ AÏÏËÏÔÁÚ·Ê›·: π. ∫·‚·ÏÈÒÙ˘ kavagrc@gmail.com ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¡ÔÛÔÎÔÌÂ›Ô ∂ȉÈÎÒÓ ¶·ı‹ÛÂˆÓ °Ú. §·ÌÚ¿ÎË 13 54638 £ÂÛÛ·ÏÔÓ›ÎË

Polysaccharide antigens – conjugated vaccines: immune memory and anamnestic doses π. Kavaliotis Abstract: This is an analysis of the immune response to polysaccharide antigens and conjugated vaccines.

Based on this analysis, there is a rationale for adding more anamnestic doses for conjugated vaccines.

Key words: Vaccines, immunity, children.

∆· Û˘˙¢Á̤ӷ ÔÏ˘Û·Î¯·ÚȉÈο ÂÌ‚fiÏÈ· ›¯·Ó Ì›˙ÔÓ˜ ÂÈÙÒÛÂȘ ÛÙȘ ·ÓÙ›ÛÙÔȯ˜ ‰ÈÂÈÛ‰˘ÙÈΤ˜ ÏÔÈÌÒÍÂȘ ÙˆÓ ·È‰ÈÒÓ fiÙ·Ó ÂÈÛ‹¯ıËÛ·Ó ÛÙ· ÂıÓÈο ÚÔÁÚ¿ÌÌ·Ù· ÂÌ‚ÔÏÈ·ÛÌÒÓ. ∏ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ·˘Ù‹ ›¯Â Û¯¤ÛË Ì ÚÔÛÙ·Û›· ̤ۈ ·Ú·ÁˆÁ‹˜ ·ÓÙÈۈ̿وÓ, ηıÒ˜ Î·È Ì¤Ûˆ Ù˘ ÚÔηÏÔ‡ÌÂÓ˘ ¤ÌÌÂÛ˘ ·ÓÔÛ›·˜. ™ËÌ·ÓÙÈÎfi ÏÂÔÓ¤ÎÙËÌ· ÙˆÓ ÂÌ‚ÔÏ›ˆÓ ·˘ÙÒÓ ‹Ù·Ó Î·È Â›Ó·È Ë ÈηÓfiÙËÙ· ·ÓÔÛȷ΋˜ ‰È¤ÁÂÚÛ˘ (ÚˆÙÔÁÂÓ‹˜ ·¿ÓÙËÛË, priming) ·fi ÙË ‚ÚÂÊÈ΋ ËÏÈΛ· (1-4). ∂›Ó·È ÁÓˆÛÙfi fiÙÈ ÙÔ ·ÓÔÛÈ·Îfi Û‡ÛÙËÌ· ·ÔÙÂÏÂ›Ù·È ·fi ‰‡Ô ·ÏÏËÏÔ-ÂÈÎ·Ï˘ÙfiÌÂÓ· ÙÌ‹Ì·Ù·, ÙÔ ÏÂÌÊÈÎfi Î·È ÙÔ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·Îfi Û‡ÛÙËÌ·. ∆· ÙÌ‹Ì·Ù· ·˘Ù¿ ÊÈÏÔÍÂÓÔ‡Ó Ù· ·ÚÈ· ·ÓÔÛȷο ·ÙÙ·Ú·, Ô˘ Â›Ó·È Ù· Ï¢ÎÔ·ÙÙ·Ú·: Ô˘‰ÂÙÂÚfiÊÈÏ·, ˈÛÈÓfiÊÈÏ·, ‚·ÛÂfiÊÈÏ·, ÌÔÓÔ·ÙÙ·Ú·, Ì·ÎÚÔÊ¿Á·, ·ÙÙ·Ú· ÊÔÓ›˜, T-ÏÂÌÊÔ·ÙÙ·Ú·, B-ÏÂÌÊÔ·ÙÙ·Ú·. ∆· ·ÙÙ·Ú· ÙÔ˘ ·ÓÔÛÈ·ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÂÈÎÔÈÓˆÓÔ‡Ó ÌÂٷ͇ ÙÔ˘˜ ̤ۈ ÌÔÚ›ˆÓ Ô˘ ηÏÔ‡ÓÙ·È Î˘ÙÙ·ÚÔΛÓ˜. H ·¿ÓÙËÛË ÙÔ˘ ·ÓÔÛÈ·ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÚÔ˜ Ù· ·ıÔÁfiÓ·-ÂÈÛ‚ÔÏ›˜ Â›Ó·È ‰ÈÙÙ‹: ÌË ÂȉÈ΋/¤ÌÊ˘ÙË (innate) Î·È ÂȉÈ΋ Â›ÎÙËÙË (adaptive). ∏ ÌË ÂȉÈ΋ Â›Ó·È Ù·¯Â›· Î·È ¿ÌÂÛË, ÛÙÂÚÂ›Ù·È ·ÓÔÛȷ΋˜ ÌÓ‹Ì˘ Î·È Û˘Ì‚·›ÓÂÈ ÛÙËÓ ›‰È· ¤ÎÙ·ÛË, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ fiÛÔ Û˘¯Ó¿ ÙÔ ·ıÔÁfiÓÔ Û˘Ó·ÓÙ¿ ÙÔÓ ÍÂÓÈÛÙ‹. ∞·ÓÙ¿ ·ÎfiÌË Î·È ÛÙ· ·ÏÔ‡ÛÙÂÚ· ›‰Ë ÙÔ˘ ˙ˆÈÎÔ‡ ‚·ÛÈÏ›Ԣ.

Department of Paediatrics, Infectious Diseases Hospital, Thessaloniki, Greece Correspondence: π. Kavaliotis kavagrc@gmail.com Department of Paediatrics Infectious Diseases Hospital 13, Gr. Lampraki St. 54638 Thessaloniki Greece

∏ ÂȉÈ΋ ·¿ÓÙËÛË Â›Ó·È ‚Ú·‰‡ÙÂÚË, ¤¯ÂÈ ÌÓ‹ÌË Î·È ÂÓÈÛ¯‡ÂÙ·È Û Â·ÓÂÈÏËÌ̤Ó˜ ÂÎı¤ÛÂȘ ÙÔ˘ ÍÂÓÈÛÙ‹ ÛÙÔ ›‰ÈÔ ·ıÔÁfiÓÔ. ∞ÔÙÂÏ› ÙËÓ Ô˘Û›· ÙÔ˘ ·ÓÔÛÈ·ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ∫·È ÔÈ ‰‡Ô ·ÓÔÛȷΤ˜ ··ÓÙ‹ÛÂȘ ÂÈÙ˘Á¯¿ÓÔÓÙ·È Ì ÙË ÌÂٷ͇ ÙÔ˘˜ Û˘ÓÂÚÁ·Û›· Î·È ÙÔÓ Û˘ÓÙÔÓÈÛÌfi, ÒÛÙ ӷ ÂÚÈÔÚ›ÛÔ˘Ó ÙÔÓ ÂÈÛ‚ÔϤ· (5-8). ∏ ÌË ÂȉÈ΋ ·ÓÔÛȷ΋ ·¿ÓÙËÛË Â›Ó·È ÌÂÓ Ù·¯Â›· Î·È ¿ÌÂÛË, ‰ÂÓ ÌÔÚ› fï˜ Ó· ÙÚÔÔÔÈËı› Î·È ÛÙÂÚÂ›Ù·È ÂȉÈÎfiÙËÙ·˜. ∏ ÂȉÈ΋ Â›ÎÙËÙË ·ÓÔÛ›· Â›Ó·È ÂÍÂȉÈÎÂ˘Ì¤ÓË, ÌÔÚ› Ó· ‰ÈÂÁÂÚı› ÒÛÙ ӷ ‰ÒÛÂÈ ÛÙË Û˘Ó¤¯ÂÈ· ÌÈ· Ù·¯Â›· Î·È ÚÔÛٷ٢ÙÈ΋ ·ÓÙÈۈ̷ÙÈ΋ ·¿ÓÙËÛË Û Â·ÎfiÏÔ˘ıË ¤ÎıÂÛË ÛÙÔ ›‰ÈÔ ·ıÔÁfiÓÔ (ÂÓÂÚÁËÙÈ΋ ·ÓÔÛ›·), ·ÏÏ¿ ‰ÂÓ Â›Ó·È ¿ÌÂÛË Î·È ··ÈÙ› ·ÚÎÂÙ¤˜ Ë̤Ú˜ ÁÈ· Ó· ·Ó·Ù˘¯ı› (5,7,8,9). ™ÎÔfi˜ ÙˆÓ ÂÌ‚ÔÏÈ·ÛÌÒÓ Â›Ó·È ·ÎÚÈ‚Ò˜ ·˘Ù‹ Ë ‰È¤ÁÂÚÛË Ù˘ ·ÓÔÛ›·˜ Ì ÙËÓ ·ÍÈÔÔ›ËÛË ÙˆÓ ·ÓÙÈÁÔÓÈÎÒÓ È‰ÈÔÙ‹ÙˆÓ ÙÔ˘ ·ıÔÁfiÓÔ˘, ¯ˆÚ›˜ ÙÔ ¿ÙÔÌÔ Ó· ¤¯ÂÈ ¤ÏıÂÈ ÚÔËÁÔ˘Ì¤Óˆ˜ Û Â·Ê‹ Ì ÙÔ ·ıÔÁfiÓÔ. ∏ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·ÓÔÛÔÔ›ËÛË Ô˘ Ô‰ËÁ› Û ̷ÎÚÔ¯ÚfiÓÈ· ÚÔÛÙ·Û›· ··ÈÙ› ÙËÓ ·Ú·ÁˆÁ‹ ÌÓËÌÔÓÈÎÒÓ µ ΢ÙÙ¿ÚˆÓ. ∞˘Ùfi ÂÈÙ˘Á¯¿ÓÂÙ·È Ì ÙȘ ·Ú¯ÈΤ˜ Î·È ÂfiÌÂÓ˜ ‰fiÛÂȘ ÂÌ‚ÔÏ›ˆÓ Ô˘ Û˘Á¯ÚfiÓˆ˜ ‚ÔËıÔ‡Ó Û ·˘ÍË̤ÓË Û˘Ó¿ÊÂÈ·-Û˘ÁÁ¤ÓÂÈ· (affinity-avidity) ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÚÔ˜ ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ·ÓÙÈÁfiÓÔ. øÛÙfiÛÔ, Ù· Ú¿ÁÌ·Ù· Ê·›ÓÂÙ·È fiÙÈ ‰ÂÓ Â›Ó·È ÙfiÛÔ ·Ï¿ Î·È ÔÈ ÁÓÒÛÂȘ ¶·È‰È·ÙÚÈ΋ 2009;72:23-28

Pediatric Jan-Feb 09

16-03-09

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∂ÈÎfiÓ· 5 ∂ÈÎfiÓ˜ 1-5. ∞ÓÔÛȷ΋ ·¿ÓÙËÛË - ·fi ÙË ‚È‚ÏÈÔÁÚ·ÊÈ΋ ·Ú·ÔÌ‹ ·Ú. 2.

Paediatriki 2009;72:23-28

π.£. ∫·‚·ÏÈÒÙ˘

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Pediatric Jan-Feb 09

16-03-09

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¶ÔÏ˘Û·Î¯·ÚȉÈο ·ÓÙÈÁfiÓ· – Û˘˙¢Á̤ӷ ÂÌ‚fiÏÈ·

Ì·˜ ÁÈ· ÙËÓ ·ÓÔÛȷ΋ ÌÓ‹ÌË Û ÌÔÚÈ·Îfi Â›Â‰Ô Â›Ó·È ·ÙÂÏ›˜ (2,3,8,9). ¶ÚÈÓ ÙËÓ ¤ÎıÂÛË Û οÔÈÔ ·ÓÙÈÁfiÓÔ ‰ÂÓ ˘¿Ú¯Ô˘Ó ÌÓËÌÔÓÈο µ ·ÙÙ·Ú· ÁÈ· ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ·ÓÙÈÁfiÓÔ, Ô‡Ù ·˘ÙfiÌ·ÙË ¤ÎÎÚÈÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ÂȉÈÎÒÓ ÁÈ· ÙÔ ·ÓÙÈÁfiÓÔ (5,7). ∆· ÂÚÈÛÛfiÙÂÚ· ·ÓÙÈÁfiÓ· ··ÈÙÔ‡Ó ÙËÓ ·ÏÏËÏÂ›‰Ú·ÛË µ Î·È T ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ ÁÈ· Ó· ÚÔηϤÛÔ˘Ó ·ÓÔÛȷ΋ ·¿ÓÙËÛË (ı˘ÌÔÂÍ·ÚÙÒÌÂÓ· ·ÓÙÈÁfiÓ·, T-thymus-dependent antigens). ªÂÚÈο, fï˜, ·ÓÙÈÁfiÓ· ÂÎÏ‡Ô˘Ó ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ µ ΢ÙÙ¿ÚˆÓ Î·È ÙËÓ ·Ú·ÁˆÁ‹ ·ÓÙÈÛˆÌ¿ÙˆÓ ¯ˆÚ›˜ ÙË ‚Ô‹ıÂÈ· ÙˆÓ ∆ ΢ÙÙ¿ÚˆÓ (ı˘ÌÔ·ÓÂÍ¿ÚÙËÙ· ·ÓÙÈÁfiÓ·, T-independent antigens). ∆· ·ÓÙÈÁfiÓ· ·˘Ù¿ ‰ÂÓ Â›Ó·È Èηӿ ·ÓÔÛÔÁfiÓ· Û ·È‰È¿ ÌÈÎÚfiÙÂÚ· ÙˆÓ 2 ¯ÚfiÓˆÓ (3,10,11). ™ÙËÓ Î·ÙËÁÔÚ›· ·˘Ù‹ ·Ó‹ÎÔ˘Ó Ù· ÔÏ˘Û·Î¯·ÚȉÈο ·ÓÙÈÁfiÓ·. ∏ Û‡˙¢ÍË Ù¤ÙÔÈˆÓ ·ÓÙÈÁfiÓˆÓ Ì ÚˆÙ½ÓË-ÊÔÚ¤· Â›Ó·È ÈηӋ Ó· «ÌÂÙ·‚¿ÏÂÈ» ÙȘ ȉÈfiÙËÙ¤˜ ÙÔ˘˜ Î·È Ó· ‰ÈÂÁ›ÚÂÈ ı˘ÌÔÂÍ·ÚÙÒÌÂÓË ·ÓÔÛȷ΋ ·¿ÓÙËÛË, Ë ÔÔ›· ¤¯ÂÈ ·Ó·Ù˘¯ı› ‹‰Ë ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· (4,8,9). ∏ ¤ÎıÂÛË, ÏÔÈfiÓ, Û ·Ïfi ÔÏ˘Û·Î¯·ÚȉÈÎfi ·ÓÙÈÁfiÓÔ/ÂÌ‚fiÏÈÔ (T-independent antigen) ‰ÈÂÁ›ÚÂÈ Ù· ·ÓÂÓÂÚÁ¿ µ ·ÙÙ·Ú· Ù· ÔÔ›· ·Ú¿ÁÔ˘Ó Î˘Ú›ˆ˜ IgG ·ÓÙÈÛÒÌ·Ù·, ‚Ú·¯˘¯ÚfiÓÈ·. ∆· µ ·˘Ù¿ ·ÙÙ·Ú· ‰ÂÓ ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È Û ÌÓËÌÔÓÈο ·ÙÙ·Ú· (2,8,9,10,12). ∆· Û˘˙¢Á̤ӷ ÔÏ˘Û·Î¯·ÚȉÈο ·ÓÙÈÁfiÓ· (Tdependent antigens) ‰ÈÂÁ›ÚÔ˘Ó Â›Û˘ Ù· ·ÓÂÓÂÚÁ¿ µ ·ÙÙ·Ú· ̤ۈ Ì˯·ÓÈÛÌÔ‡ Ô˘ ··ÈÙ› ΢ÙÙ·ÚÈΤ˜ Î·È ÌÔÚȷΤ˜ ·ÏÏËÏÂȉڿÛÂȘ, ÂÂÍÂÚÁ·Û›· ÙÔ˘ ·ÓÙÈÁfiÓÔ˘ ·fi Ù· ·ÓÙÈÁÔÓÔ·ÚÔ˘ÛÈ·ÛÙÈο ·ÙÙ·Ú· (antigen-presenting cells - ∞PCs), ·ÚÔ˘Û›·ÛË ÙÔ˘ ·ÓÙÈÁfiÓÔ˘ ÛÙ· ∆-·ÙÙ·Ú· ̤ۈ MHC ÌÔÚ›ˆÓ, ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ∆-helper (CD4) ΢ÙÙ¿ÚˆÓ Î·È ¤ÎÎÚÈÛË Î˘ÙÙ·ÚÔÎÈÓÒÓ Ô˘ Ô‰ËÁÔ‡Ó Û ˆÚ›Ì·ÓÛË Ù· Thelper ·ÙÙ·Ú· Î·È ‚ÔËıÔ‡Ó ÙËÓ ÂÈÎÔÈÓˆÓ›· ÌÂٷ͇ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ, ̤ۈ ÈÓÙÂÚÏ¢ÎÈÓÒÓ Ô˘ Ú˘ıÌ›˙Ô˘Ó ÙËÓ ·ÓÔÛȷ΋ ·¿ÓÙËÛË (2,5,6,8,12). ŸÙ·Ó Ù· µ ·ÙÙ·Ú· ÂÓÂÚÁÔÔÈËıÔ‡Ó, ÔÏÏ·Ï·ÛÈ¿˙ÔÓÙ·È Î·È ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È Û Ï·ÛÌ·ÙÔ·ÙÙ·Ú· (ÂÎÎÚ›ÓÔ˘Ó ÂȉÈο ·ÓÙÈÛÒÌ·Ù·) Î·È Û ̷ÎÚfi‚È· ÌÓËÌÔÓÈο µ ·ÙÙ·Ú· Ì ÂÈÊ·ÓÂÈ·ÎÔ‡˜ ˘ԉԯ›˜ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ ÂȉÈÎÒÓ ÁÈ· ÙÔ ·ÓÙÈÁfiÓÔ (2,7,8). ™ÙËÓ ÚˆÙÔÁÂÓ‹ ·ÓÔÛȷ΋ ·¿ÓÙËÛË ÚÔ˜ ·ÓÙÈÁfiÓÔ ÂÌ‚ÔÏ›Ô˘, ˘¿Ú¯ÂÈ ÌÈ· Ï·Óı¿ÓÔ˘Û· ÂÚ›Ô‰Ô˜ ÔÚÈÛÌ¤ÓˆÓ ËÌÂÚÒÓ ÒÛÔ˘ Ó· ÎÈÓËÙÔÔÈËıÔ‡Ó Ë Î˘ÙÙ·ÚÈ΋ Î·È Ë ¯˘ÌÈ΋ ·ÓÔÛ›·. ∆· ·ÓÙÈÛÒÌ·Ù· ÛÙÔÓ ÔÚfi ·ÓȯÓ‡ÔÓÙ·È 7-10 Ë̤Ú˜ ‹ Î·È ϤÔÓ ÌÂÙ¿ ÙÔÓ ÂÌ‚ÔÏÈ·ÛÌfi (6,13). √ Ù‡Ô˜ ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÔÈΛÏÏÂÈ ·Ó¿ÏÔÁ· Ì ÙÔÓ Ù‡Ô ÙÔ˘ ·ÓÙÈÁfiÓÔ˘. ™ÙËÓ ÚÒÙË Â·Ê‹, Ù· ·Ú¯Èο ·ÓÙÈÛÒÌ·Ù· Â›Ó·È IgM. ∆· ÂfiÌÂÓ· ·ÓÙÈÛÒÌ·Ù· Â›Ó·È IgG. ∆· ÚÒÈÌ· IgM Î·È πgG ·ÓÙÈ-

ÛÒÌ·Ù· ÂÎÎÚ›ÓÔÓÙ·È ·fi Ù· B ÏÂÌÊÔ·ÙÙ·Ú· Û ·¿ÓÙËÛË ÚÔ˜ Ù· T-dependent antigens. ¶ÚÒÙ· ·ÓȯÓ‡ÔÓÙ·È Ù· πgM, Ù· ÔÔ›· ‰ÂÛÌÂ‡Ô˘Ó ÙÔ Û˘Ìϋڈ̷ Ô˘ ‰È¢ÎÔχÓÂÈ ÙË Ï‡ÛË Î·È ÙË Ê·ÁÔ΢ÙÙ¿ÚˆÛË ÙˆÓ ÌÈÎÚÔ‚›ˆÓ. ∫·Ù¿ ÙË 2Ë Â‚‰ÔÌ¿‰· ÂÏ·ÙÙÒÓÔÓÙ·È ÔÈ Ù›ÙÏÔÈ ÙˆÓ πgM Î·È ·˘Í¿ÓÔÓÙ·È ÔÈ Ù›ÙÏÔÈ ÙˆÓ IgG Ì ̤ÁÈÛÙ˜ ÙÈ̤˜ ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ ÂÓÙfi˜ 2-6 ‚‰ÔÌ¿‰ˆÓ. ∏ ÌÂÙ·‚ÔÏ‹ ÛÙË Û‡ÓıÂÛË ·fi πgM Û IgG ··ÈÙ› Û˘ÓÂÚÁ·Û›· B Î·È T ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ. ∆· IgG ·Ú¿ÁÔÓÙ·È ÛÙË Û˘Ó¤¯ÂÈ· Û ˘„ËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ Î·È ÚÔÛÙ·ÙÂ‡Ô˘Ó ·fi ÙȘ ÏÔÈÌÒÍÂȘ. √È ÏÂÈÙÔ˘ÚÁ›Â˜ ÙÔ˘˜ ·ÊÔÚÔ‡Ó Û˘Ó¿ÊÂÈ· ÚÔ˜ ÙÔ˘˜ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜, ÂÍÔ˘‰ÂÙ¤ÚˆÛË ÙˆÓ ÈÒÓ, ·ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÓÙÈÁfiÓˆÓ, ·‰Ú·ÓÔÔ›ËÛË ÙÔÍÈÎÒÓ ÚÔ˚fiÓÙˆÓ, ·ÚÂÌfi‰ÈÛË Ù˘ ÚÔÛÎfiÏÏËÛ˘ ÙˆÓ ÌÈÎÚÔ‚›ˆÓ ÛÙÔ˘˜ ‚ÏÂÓÓÔÁfiÓÔ˘˜, ÚÔ·ÁˆÁ‹ Ù˘ Ê·ÁÔ΢ÙÙ¿ÚˆÛ˘, Û‡Ó‰ÂÛË ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ (6,8,14). ªÈ· ‰Â‡ÙÂÚË ¤ÎıÂÛË ÛÙÔ ›‰ÈÔ T-dependent ·ÓÙÈÁfiÓÔ Ô‰ËÁ› Û ÂÓÈÛ¯˘Ì¤ÓË ·ÓÔÛȷ΋ ·¿ÓÙËÛË. ŸÙ·Ó Ù· ÌÓËÌÔÓÈο µ ·ÙÙ·Ú· Û˘Ó·ÓÙÔ‡Ó ÙÔ ÂȉÈÎfi ·ÓÙÈÁfiÓÔ Ô˘ ÚÔοÏÂÛ ÙËÓ ·Ú·ÁˆÁ‹ ÙÔ˘˜, ÔÏÏ·Ï·ÛÈ¿˙ÔÓÙ·È Î·È ‰È·ÊÔÚÔÔÈÔ‡ÓÙ·È Û Ï·ÛÌ·ÙÔ·ÙÙ·Ú· Ù·¯‡ÙÂÚ· ·fi fi,ÙÈ Ù· ·ÓÂÓÂÚÁ¿ ·ÙÙ·Ú·. ∏ ‰Â˘ÙÂÚÔÁÂÓ‹˜ ·˘Ù‹ ·¿ÓÙËÛË Â›Ó·È Ù·¯‡ÙÂÚË Î·È ÂÎχÂÙ·È ÂÓÙfi˜ 4-5 ËÌÂÚÒÓ. ∂›Ó·È ·ÔÙ¤ÏÂÛÌ· ·ÓÔÛȷ΋˜ ÌÓ‹Ì˘ ̤ۈ ÙˆÓ T Î·È B ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ¤ÓÙÔÓÔ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ µ ΢ÙÙ¿ÚˆÓ Ô˘ ·Ú¿ÁÔ˘Ó ·ÓÙÈÛÒÌ·Ù· ‹ ÙˆÓ ‰Ú·ÛÙÈÎÒÓ T ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ. ∆· T-independent ·ÓÙÈÁfiÓ· (·Ï¿ ÔÏ˘Û·Î¯·ÚȉÈο) ‰ÂÓ ÂÎÏ‡Ô˘Ó Ù¤ÙÔÈ· ¤ÓÙÔÓË ·ÓÔÛȷ΋ ·¿ÓÙËÛË (2,6-9). ∆· Ï·ÛÌ·ÙÔ·ÙÙ·Ú· ÌÔÚ› Ó· Â›Ó·È ‚Ú·¯‡‚È·. ÕÚ·, ··ÈÙÂ›Ù·È Û˘Ó¯‹˜ ÔÏÏ·Ï·ÛÈ·ÛÌfi˜ Î·È ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ÌÓËÌÔÓÈÎÒÓ µ-΢ÙÙ¿ÚˆÓ Û Ï·ÛÌ·ÙÔ·ÙÙ·Ú· ÁÈ· Û˘Ó¯‹ ÚÔÛÙ·Û›·. ∞˘Ùfi ÌÔÚ› Ó· Á›ÓÂÙ·È ·˘ÙfiÌ·Ù· ‹ ̤ۈ Û˘Ó¯ԇ˜ ·ÓÙÈÁÔÓÈÎÔ‡ ÂÚÂıÈÛÌÔ‡ [ÊÔÚ›˜, ¤ÎıÂÛË Û ΢ÙÙ·ÚÔΛÓ˜ Ô˘ ·Ú¿ÁÔÓÙ·È ·fi ∆ ·ÙÙ·Ú· ÏfiÁˆ ÂÚÂıÈÛÌÔ‡ ·fi ‰È¿ÊÔÚ· ·ÓÙÈÁfiÓ· (bystander effect)] (EÈÎ. 1-5) (2,6,15,16). ∏ Ì·ÎÚ¿ ·Ú·ÌÔÓ‹ ÚÔÛٷ٢ÙÈÎÒÓ Ù›ÙÏˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÚÔÊ˘Ï¿ÛÛÂÈ ·fi ‰ÈÂÈÛ‰˘ÙÈΤ˜ ÏÔÈÌÒÍÂȘ Î·È Ë ·Ú·ÌÔÓ‹ ·˘Ù‹ Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ·Ó·Áη›·. ¶·ÚfiÏÔ Ô˘ Ë ÌÓ‹ÌË ˘¿Ú¯ÂÈ, Ê·›ÓÂÙ·È fiÙÈ Ù· µ ÌÓËÌÔÓÈο ·ÙÙ·Ú· ÂÓ›ÔÙ ‰ÂÓ Â·ÚÎÔ‡Ó ·fi ÌfiÓ· ÙÔ˘˜, ÂÓÒ ¿ÏϘ ÊÔÚ¤˜ ÚÔÛÙ·ÙÂ‡Ô˘Ó Î·È Ì ÌÈÎÚfiÙÂÚÔ˘˜ ·ÎfiÌË Ù›ÙÏÔ˘˜ (17). ™ÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË, Ë ·¿ÓÙËÛË ÛÙ· ÂÌ‚fiÏÈ· ÂÎÙÈÌ¿Ù·È ·fi ÙÔ˘˜ Ù›ÙÏÔ˘˜ ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙÔÓ ÔÚfi. √È ˘ÎÓfiÙËÙ˜ ·˘Ù¤˜, fï˜, Â›Ó·È ÌfiÓÔ Ì›· ·Ú¿ÌÂÙÚÔ˜ Ù˘ ·ÓÔÛȷ΋˜ ·¿ÓÙËÛ˘. ∏ ΢ÙÙ·ÚÈ΋ ·ÓÔÛ›· ·›˙ÂÈ Â›Û˘ ÚfiÏÔ. ∏ ·Ó·ÌÓËÛÙÈ΋ ·¿ÓÙËÛË Û Â·ÓÂÌ‚ÔÏÈ·ÛÌÔ‡˜ ÛËÌ·›ÓÂÈ ·ÓÔÛȷ΋ ÌÓ‹ÌË ¶·È‰È·ÙÚÈ΋ 2009;72:23-28

Pediatric Jan-Feb 09

16-03-09

14:56

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26

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Î·È ¿Ú· ˘¿Ú¯Ô˘Û· Î·È ÂÌ̤ÓÔ˘Û· ·ÓÔÛ›·. ∂›Û˘, Ë ·Ô˘Û›· ·ÓȯÓ‡ÛÈÌˆÓ Ù›ÙÏˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ‰ÂÓ ÛËÌ·›ÓÂÈ Î·Ù’ ·Ó¿ÁÎË fiÙÈ ÙÔ ¿ÙÔÌÔ ‰ÂÓ ÚÔÛٷهÂÙ·È. √È Ì˯·ÓÈÛÌÔ› Â›Ó·È È‰È·›ÙÂÚ· Û‡ÓıÂÙÔÈ (2, 6,17,18). ªÂϤÙ˜ Û ·È‰È¿ Ô˘ ÂÌ‚ÔÏÈ¿ÛıËÎ·Ó Î·Ù¿ ÙÔ˘ Hib ·ÏÏ¿ Î·È Î·Ù¿ ÙÔ˘ Men C Ì ÙÔ Û˘˙¢Á̤ÓÔ ÂÌ‚fiÏÈÔ ¤‰ÂÈÍ·Ó fiÙÈ Ô ‚·ÛÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ ‰ÂÓ Â·ÚΛ Î·È fiÙÈ ¤Ó· ¯ÚfiÓÔ Î·È ϤÔÓ ÌÂÙ¿ ÙÔÓ ·Ú¯ÈÎfi ÂÌ‚ÔÏÈ·ÛÌfi Ë ÚÔÛÙ·Û›· ‹Ù·Ó ÌÈÎÚfiÙÂÚË, Û ۯ¤ÛË Ì ÙÔ ¿ÌÂÛÔ ÌÂÙ¿ ÙÔÓ ÂÌ‚ÔÏÈ·ÛÌfi ‰È¿ÛÙËÌ·, ·ÚfiÏÔ Ô˘ ˘‹Ú¯Â ·Ú¯È΋ ‰È¤ÁÂÚÛË. ∂›Û˘, Û ÔÏÏ¿ ¿ÙÔÌ· Ù· ·ÓÙÈÛÒÌ·Ù· ‹Ù·Ó ÔÚȷο ·ÎfiÌË Î·È Û ÎÔÓÙÈÓ¿ Ì ÙÔ Ù¤ÏÔ˜ Ù˘ ‚·ÛÈ΋˜ ÛÂÈÚ¿˜ ‰È·ÛÙ‹Ì·Ù·. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ Ù· ¿ÙÔÌ· ·˘Ù¿ ‰ÂÓ ÚÔÛٷهÔÓÙ·È Û ÌÈ· Ù·¯Â›· ÂÈÛ‚ÔÏ‹ ÙˆÓ ·ıÔÁfiÓˆÓ. Ÿˆ˜ ÚԷӷʤÚıËÎÂ, Ë ‰Â˘ÙÂÚÔÁÂÓ‹˜ ·ÓÔÛȷ΋ ·¿ÓÙËÛË Î·È Ë ÂÌÊ¿ÓÈÛË IgG ·ÓÙÈÛˆÌ¿ÙˆÓ ··ÈÙ› ÙÔ˘Ï¿¯ÈÛÙÔÓ 4 Ë̤Ú˜ ·ÎfiÌË Î·È Û ÂΛÓÔ˘˜ Ô˘ ›¯·Ó ··ÓÙ‹ÛÂÈ ÈηÓÔÔÈËÙÈο ÛÙËÓ ÚÒÙË Â·Ê‹ Ì ÙÔ ·ÓÙÈÁfiÓÔ. ™˘ÓÂÒ˜, ÙÔ priming ‰ÂÓ Â·ÚΛ ÌÂÚÈΤ˜ ÊÔÚ¤˜ Î·È Ù· ¿ÙÔÌ· ÌÔÚ› Ó· ÌËÓ ÚÔÛٷهÔÓÙ·È, ·Ó Î·È ÂÓ›ÔÙ ˘¿Ú¯ÂÈ ·ÏÏËÏÔÂÈÎ¿Ï˘„Ë ÚˆÙÔÁÂÓÔ‡˜ Î·È ‰Â˘ÙÂÚÔÁÂÓÔ‡˜ ··ÓÙ‹Ûˆ˜, Ô˘ ηχÙÂÈ ÙÔ ÎÂÓfi ¤ˆ˜ ÙËÓ ·Ú·ÁˆÁ‹ ·ÓÙÈۈ̿وÓ. ∏ ÚˆÙÔÁÂÓ‹˜ ·¿ÓÙËÛË Â›Ó·È ¤Ó· Û‡ÓıÂÙÔ Ê·ÈÓfiÌÂÓÔ, ÙÔ˘ ÔÔ›Ô˘ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ‰ÂÓ ·ÓÙÈηÙÔÙÚ›˙ÂÙ·È Ï‹Úˆ˜ ·fi Ì›· ·Ï‹ ̤ÙÚËÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ÌÂÙ¿ ‰fiÛÂȘ ·Ó·˙ˆ‡ÚˆÛ˘. ™ËÌ·ÓÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ù˘ ‰Â˘ÙÂÚÔÁÂÓÔ‡˜ ·ÓÙÈۈ̷ÙÈ΋˜ ·¿ÓÙËÛ˘ Â›Ó·È Ë ÔÈfiÙËÙ· ÙˆÓ ·Ú·ÁfiÌÂÓˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ (ÈÛÔÙ˘›·, Û˘ÁÁ¤ÓÂÈ·, ÌÈÎÚÔ‚ÈÔÎÙfiÓ· ‰Ú¿ÛË), ηıÒ˜ Î·È Ë ÎÈÓËÙÈ΋ ÙÔ˘˜. ŒÙÛÈ, ‰ÂÓ Â›Ó·È ÂÚ›ÂÚÁÔ Ô˘ Û ÔÚÈṲ̂ӷ ¿ÙÔÌ· Ë ·ÓÔÛȷ΋ ÌÓ‹ÌË ·ÔÙ˘Á¯¿ÓÂÈ Ó· ÚÔÛʤÚÂÈ ÚÔÛÙ·Û›·. ™Ù· ¿ÙÔÌ· ·˘Ù¿, ÛËÌ·ÓÙÈÎfiÙÂÚË ·fi ÙËÓ ·Ú¯È΋ ·¿ÓÙËÛË Â›Ó·È Ë ÈηÓfiÙËÙ¿ ÙÔ˘˜ Ó· Û˘ÓÙËÚÔ‡Ó ÚÔÛٷ٢ÙÈο Â›‰· ·ÓÙÈÛˆÌ¿ÙˆÓ Â› Ì·ÎÚfiÓ. ∏ ηٷÓfiËÛË Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ µ ΢ÙÙ¿ÚˆÓ ›Ûˆ˜ ‚ÔËı‹ÛÂÈ ÛÙËÓ Î·Ù·ÓfiËÛË Ù˘ Û¯¤Û˘ ÂÌ‚fiÏÈÔ-ÚˆÙÔÁÂÓ‹˜ ·¿ÓÙËÛË-Û˘Ó¯‹˜ ·Ú·ÁˆÁ‹ ·ÓÙÈÛˆÌ¿ÙˆÓ (2,5,7,19,20). ∏ Û˘Ó¿ÊÂÈ· (avidity) Â›Ó·È Î·Ïfi˜ ‰Â›ÎÙ˘ Û˘ÁÁ¤ÓÂÈ·˜ (affinity) ÙˆÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÁÈ· ¤Ó· Û˘ÁÎÂÎÚÈ̤ÓÔ ·ÓÙÈÁfiÓÔ. ∏ Û˘Ó¿ÊÂÈ· ·˘Í¿ÓÂÈ ÌÂÙ¿ ¤ÎıÂÛË Û T-dependent ·ÓÙÈÁfiÓ· Î·È Ë ·‡ÍËÛË ·˘Ù‹ ‰ËÏÒÓÂÈ ÙËÓ ÈηÓfiÙËÙ· ·Ú·ÁˆÁ‹˜ µ ΢ÙÙ¿ÚˆÓ ˘„ËÏ‹˜ Û˘ÁÁ¤ÓÂÈ·˜, ‰ËÏ. ÙËÓ ·Ú·ÁˆÁ‹ ÈÔ ÔÈÔÙÈÎÒÓ Î·È ÈÔ ·ÔÙÂÏÂÛÌ·ÙÈÎÒÓ ·ÓÙÈۈ̿وÓ. ¶·Ú·Ì¤ÓÂÈ, fï˜, ·Û·Ê¤˜ ·Ó ÔÈ Û¯ÂÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÛÙËÓ avidity ÌÂÙ·ÊÚ¿˙ÔÓÙ·È Û ‰È·ÊÔÚ¤˜ ÛÙË Ì·ÎÚÔ¯ÚfiÓÈ· ÚÔÛÙ·Û›· (2,5,7,21,22). √ ÂÌ‚ÔÏÈ·ÛÌfi˜ ÚÔηÏ› ÌÂÁ¿ÏË ·‡ÍËÛË Ï·ÛÌ·ÙÔ΢ÙÙ¿ÚˆÓ Î·È ÌÓËÌÔÓÈÎÒÓ µ ΢ÙÙ¿ÚˆÓ. ∏ ·‡Paediatriki 2009;72:23-28

ÍËÛË ÙˆÓ Ï·ÛÌ·ÙÔ΢ÙÙ¿ÚˆÓ Â›Ó·È ·ÚÔ‰È΋ Î·È ÁÈ’ ·˘Ùfi ÛÙ· ÂӉȿÌÂÛ· ÙˆÓ ·ÓÙÈÁÔÓÈÎÒÓ ÂÎı¤ÛÂˆÓ ‰È·ÛÙ‹Ì·Ù· ··ÈÙÂ›Ù·È ·Ó·Ó¤ˆÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÁÈ· ‰È·Ù‹ÚËÛË ÈηÓÔÔÈËÙÈÎÒÓ ÂÈ¤‰ˆÓ. ∆Ô ÙÈ ¯ÚÂÈ¿˙ÂÙ·È Î·È ÔÈ· Â›Ó·È Ù· ÂÚÂı›ÛÌ·Ù· Ô˘ ‚ÔËıÔ‡Ó ÛÙË ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ÌÓËÌÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Û Ï·ÛÌ·ÙÔ·ÙÙ·Ú· Î·È ÛÙË Û˘ÓÙ‹ÚËÛË ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ Â›Ó·È ·Û·Ê¤˜. ¶ÔÈ· Â›Ó·È Ë Ê‡ÛË Ù˘ ÌÓ‹Ì˘ Î·È ÙˆÓ Ï·ÛÌ·ÙÔ΢ÙÙ¿ÚˆÓ Ô˘ ·Ú¿ÁÔÓÙ·È ·fi ÂÌ‚ÔÏÈ·ÛÌfi ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·; ¶Ò˜ ÌÂÙ·‚¿ÏÏÔÓÙ·È ÔÈ ··ÓÙ‹ÛÂȘ ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ· ‹ ÌÂÙ¿ ÙË ‰fiÛË ÂÌ‚ÔÏ›Ô˘ ·Ó·˙ˆ‡ÚˆÛ˘; ¶ÔÈ· µ-·ÙÙ·Ú· ÔÏÏ·Ï·ÛÈ¿˙ÔÓÙ·È Ì¤Ûˆ ÙÔ˘ bystander effect Î·È ÔÈ· ̤ۈ ÙÔ˘ ÂÌ‚ÔÏÈ·ÛÌÔ‡; ∏ ÁÓÒÛË ı· ‹Ù·Ó ÎÏÂȉ› ÁÈ· ÙËÓ Î·Ù·ÓfiËÛË Ù˘ ‚ÈÔÏÔÁ›·˜ ÙˆÓ Û˘˙¢ÁÌ¤ÓˆÓ ÔÏ˘Û·Î¯·ÚȉÈÎÒÓ ÂÌ‚ÔÏ›ˆÓ, ÌÈ·˜ Î·È Ë ÚˆÙÔÁÂÓ‹˜ ·¿ÓÙËÛË Ê·›ÓÂÙ·È fiÙÈ ‰ÂÓ Â·ÚΛ ϤÔÓ ÁÈ· ÚÔÛÙ·Û›· ÔÚÈÛÌ¤ÓˆÓ ·ÙfiÌˆÓ Ì ¤ÏÏÂÈ„Ë ·ÓÙÈÛˆÌ¿ÙˆÓ Û ‰Â‰Ô̤ÓË ÛÙÈÁÌ‹ (2). ¶ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ Ì ·ÓÙÈۈ̷ÙÈÎfi ¤ÏÂÁ¯Ô ÚÔ ÙÔ˘ ÂÌ‚ÔÏÈ·ÛÌÔ‡ Î·È ÌÂÙ¿ ÙÔÓ ÂÌ‚ÔÏÈ·ÛÌfi ÌÂ Û˘˙¢Á̤ÓÔ ÂÌ‚fiÏÈÔ Î·Ù¿ ÙÔ˘ Hib ¤‰ÂÈÍ·Ó ·˘Ùfi ·ÎÚÈ‚Ò˜, fiÙÈ ‰ËÏ. Ë ÚˆÙÔÁÂÓ‹˜ Î·È ÌfiÓÔ ·¿ÓÙËÛË ÌÔÚ› Ó· ÌËÓ ÚÔÛٷهÂÈ Â·ÚÎÒ˜. ∂›Û˘, Â›Ó·È ÁÓˆÛÙfi fiÙÈ Û ¿ÙÔÌ· Ì ¯ÔÚ‹ÁËÛË Û˘Ó‰˘·Ṳ̂ÓÔ˘ ÂÌ‚ÔÏ›Ô˘ DTaP Î·È Hib ¤¯ÂÈ ·Ú·ÙËÚËı› ÌÂȈ̤ÓË ·ÓÔÛȷ΋ ·¿ÓÙËÛË. ∆ÂÏÂ˘Ù·›·, ÛÙË ª. µÚÂÙ·Ó›· ‰È·ÈÛÙÒıËΠÌÂȈ̤ÓË ÚÔÛÙ·Û›· ¤Ó· ¯ÚfiÓÔ Î·È ϤÔÓ ·fi ÙÔ ÂÌ‚fiÏÈÔ ÁÈ· Men C, Û ۯ¤ÛË Ì ÂÚ›Ô‰Ô ÌÈÎÚfiÙÂÚË ÙÔ˘ ¤ÙÔ˘˜ ·fi ÂÌ‚ÔÏÈ·ÛÌfi. √È ·ÔÙ˘¯›Â˜ Î·È ÛÙ· ‰‡Ô ÂÌ‚fiÏÈ· ‹Ù·Ó Û ¿ÙÔÌ· Ô˘ ·Ô‰ÂÈÁ̤ӷ ›¯·Ó ÚˆÙÔÁÂÓ‹ ·¿ÓÙËÛË, ·ÏÏ¿ ÛÙË Û˘Ó¤¯ÂÈ· ÔÈ Ù›ÙÏÔÈ ÙˆÓ ·ÓÙÈη„ȉÈÎÒÓ ÙÔ˘˜ ·ÓÙÈÛˆÌ¿ÙˆÓ ‹Ù·Ó ¯·ÌËÏfiÙÂÚÔÈ ·fi ÙÔ˘˜ ıˆÚÔ‡ÌÂÓÔ˘˜ ˆ˜ ÚÔÛٷ٢ÙÈÎÔ‡˜. º˘ÛÈο, Ù· ¿ÙÔÌ· ·˘Ù¿ Â›Ó·È ·ÚÔÛÙ¿ÙÂ˘Ù· ·fi Ïԛ̈ÍË Ù·¯Â›·˜ ÂͤÏÈ͢ ÚÈÓ ÚÔÏ¿‚ÂÈ Ó· ··ÓÙ‹ÛÂÈ Ô ·ÓÔÛÔÏÔÁÈÎfi˜ Ì˯·ÓÈÛÌfi˜ (2,23,24,25,26). ŸÛÔÓ ·ÊÔÚ¿ ÙÔÓ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ, ÚfiÛÊ·Ù˜ ¤Ú¢Ó˜ ¤‰ÂÈÍ·Ó Ù· ÂÍ‹˜: ‰È¿ÚÎÂÈ· ÚÔÛÙ·Û›·˜ ·fi ÙÔ ÂÌ‚fiÏÈÔ fi¯È ȉȷ›ÙÂÚ· ÁÓˆÛÙ‹, ÔÈ ‰Â›ÎÙ˜ ·ÓÔÛ›·˜ Î·È Ë ÚÔÛÙ·Û›· ÂÍ·ÛıÂÓÔ‡Ó Ù·¯¤ˆ˜ ÌÂÙ¿ ÙÔÓ ·Ú¯ÈÎfi ÂÌ‚ÔÏÈ·ÛÌfi, 4 ¯ÚfiÓÈ· ÌÂÙ¿ ‚·ÛÈÎfi ÂÌ‚ÔÏÈ·ÛÌfi ÙÔ 88% ¤¯ÂÈ Ù›ÙÏÔ˘˜ ¯·ÌËÏfiÙÂÚÔ˘˜ ÙˆÓ ÚÔÛٷ٢ÙÈÎÒÓ, ¯·ÌËÏ¿ Â›‰· (ÌÂÙ¿ 3ÂÙ›·) Û ÂÌ‚ÔÏÈ·Ûı¤ÓÙ˜ ÌÂÙ¿ ÙÔÓ 1Ô ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜, ·˘ÍË̤ÓË ÂÌ‚ÔÏÈ·ÛÙÈ΋ ·Ó·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· 2-3 ¯ÚfiÓÈ· ÌÂÙ¿ ÙÔÓ ‚·ÛÈÎfi ÂÌ‚ÔÏÈ·ÛÌfi (3,20,27). ™Â Ì›· ¤Ú¢ӷ (20), Ì¿ÏÈÛÙ·, ‚Ú¤ıËΠfiÙÈ ÌÂÙ¿ ·Ú¯ÈÎfi Û¯‹Ì· 3 ‰fiÛÂˆÓ (2, 3, 4 ÌËÓÒÓ) Ë ·ÓÙÈÁÔÓÈÎfiÙËÙ· ÂÌ‚ÔÏ›Ô˘, ·fi 93% ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ ÌÂÙ¿ ÂÌ‚ÔÏÈ·ÛÌfi, ˘Ô¯ÒÚËÛ Û -81% ÛÙÔ ‰È¿ÛÙËÌ· ¤Ú·Ó ÙÔ˘ ¤ÙÔ˘˜. µ¤‚·È·, Ô ·ÚÈıÌfi˜ ·˘Ùfi˜ ÛÙËÚ›¯ıËÎÂ

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·27

27

¶ÔÏ˘Û·Î¯·ÚȉÈο ·ÓÙÈÁfiÓ· – Û˘˙¢Á̤ӷ ÂÌ‚fiÏÈ·

Û 19 ÂÚÈÙÒÛÂȘ ‰ÈÂÈÛ‰˘ÙÈ΋˜ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋˜ ÓfiÛÔ˘ C, οÙÈ Ô˘ ÛËÌ·›ÓÂÈ fiÙÈ ÁÈ· Ï‹ÚË ÙÂÎÌËÚ›ˆÛË ¯ÚÂÈ¿˙ÂÙ·È ÌÂÁ·Ï‡ÙÂÚÔ˜ ·ÚÈıÌfi˜. °ÂÓÈο, fï˜, ıˆÚÂ›Ù·È Û‹ÌÂÚ· fiÙÈ ÙfiÛÔ ÁÈ· ÙÔÓ ·ÈÌfiÊÈÏÔ fiÛÔ Î·È ÁÈ· ÙÔÓ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ, Ë ·ÓÔÛÔÏÔÁÈ΋ ÌÓ‹ÌË ‰ÂÓ Â›Ó·È Â·Ú΋˜ ÁÈ· Ó· ÚÔÛٷهÛÂÈ ÔÚÈṲ̂ӷ ÂÌ‚ÔÏÈ·Ṳ̂ӷ ¿ÙÔÌ·. √ ΛӉ˘ÓÔ˜ ÓfiÛËÛ˘ ηıÔÚ›˙ÂÙ·È ·fi ·Ú·Ì¤ÙÚÔ˘˜ Ù˘ Ê˘ÛÈ΋˜ ·ÓÔÛ›·˜, Ù˘ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ ·ÓÔÛ›·˜, Ù˘ ÂÍ¿ÏÂȄ˘ ÊÔÚ›·˜ Î·È Ù˘ ÚfiÎÏËÛ˘ ¤ÌÌÂÛ˘ ·ÓÔÛ›·˜ (2,3). ªÂ ÙÔ Ó¢ÌÔÓÈÔÎÔÎÎÈÎfi Û˘˙¢Á̤ÓÔ ÂÌ‚fiÏÈÔ ‰ÂÓ ¤¯ÂÈ ÚÔ·„ÂÈ ÚÔ˜ ÙÔ ·ÚfiÓ È‰È·›ÙÂÚÔ Úfi‚ÏËÌ·, ÌÈ·˜ Î·È ÔÈ ÂÊ·ÚÌÔ˙fiÌÂÓ˜ ‰fiÛÂȘ Â›Ó·È ·ÚÎÂÙ¤˜, ·ÏÏ¿ Î·È ÁÈ· ÙȘ ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ˘ ˘¿Ú¯ÂÈ ÙÔ 23‰‡Ó·ÌÔ ÔÏ˘Û·Î¯·ÚȉÈÎfi ÂÌ‚fiÏÈÔ. ¶ÚÔ·ÙÂÈ ÏÔÈfiÓ ÙÔ Ú·ÎÙÈÎfi ÂÚÒÙËÌ·: ÔÈÔ Â›Ó·È ÙÔ ÛˆÛÙfi ‰ÔÛÔÏÔÁÈÎfi Û¯‹Ì· ÁÈ· Ù· ÔÏ˘Û·Î¯·ÚȉÈο Û˘˙¢Á̤ӷ ÂÌ‚fiÏÈ·; ∆Ô ÂÚÒÙËÌ· ·ÊÔÚ¿ ΢ڛˆ˜ ÙÔ ÂÌ‚fiÏÈÔ ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘, Ô˘ ÂÍ·Ú¯‹˜ ÂÊ·ÚÌfiÛıËΠ̠ÌÂȈ̤ӷ ‰ÔÛÔÏÔÁÈο Û¯‹Ì·Ù· Ô˘ Ô›ÎÈÏ·Ó ·fi 1 ¤ˆ˜ Î·È 3 ‰fiÛÂȘ. ∏ ·Ú¯È΋ ˘ÈÔı¤ÙËÛË ‹Ù·Ó 3 ‰fiÛÂȘ ÛÙÔÓ ‚·ÛÈÎfi ÂÌ‚ÔÏÈ·ÛÌfi (‚ÚÂÊÈ΋ ËÏÈΛ·) ‹ 1 ‰fiÛË ÌÂÙ¿ ÙËÓ ËÏÈΛ· ÙˆÓ 12 ÌËÓÒÓ (3,20,26,28). ∆· Û¯‹Ì·Ù· ·˘Ù¿ ÛÙËÚ›¯ıËÎ·Ó Û ·Ú¯ÈΤ˜ ÌÂϤÙ˜ ·ÓÔÛÔÁÔÓÈÎfiÙËÙ·˜ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ Î·È ˘ÈÔıÂÙ‹ıËÎ·Ó ·Ó¿ÏÔÁ· Ì ÙȘ ÂȉËÌÈÔÏÔÁÈΤ˜ Û˘Óı‹Î˜ οı ¯ÒÚ·˜.

MÂϤÙ˜ ÛÙË Û˘Ó¤¯ÂÈ· ¤‰ÂÈÍ·Ó fiÙÈ 2 ‰fiÛÂȘ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· ÚÔηÏÔ‡Ó ÈηÓÔÔÈËÙÈÎfi Ù›ÙÏÔ ·ÓÙÈۈ̿وÓ. ∫·Ù¿ Û˘Ó¤ÂÈ·, ·Ó ÂÊ·ÚÌÔ˙fiÙ·Ó ÙÔ Û¯‹Ì· ·˘Ùfi, ı· ÚԤ΢ÙÂ Î·È ÛËÌ·ÓÙÈÎfi ÔÈÎÔÓÔÌÈÎfi fiÊÂÏÔ˜. À‹ÚÍ·Ó Â›Û˘ ‰È·ÈÛÙÒÛÂȘ fiÙÈ Ë ‰È‡ڢÓÛË ÙÔ˘ ÌÂÛԉȷÛÙ‹Ì·ÙÔ˜ ‰fiÛÂˆÓ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ηٿ ÙÔ˘ ·ÈÌÔÊ›ÏÔ˘ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· ‚ÂÏÙÈÒÓÂÈ ÙÔ priming Î·È ÙËÓ avidity Î·È ·˘Í¿ÓÂÈ ÙÔÓ ÙÂÏÈÎfi Ù›ÙÏÔ ·ÓÙÈÛˆÌ¿ÙˆÓ (2).

TÔ fiÙÈ Ë ·ÓÔÛ›· ÂÍ·ÛıÂÓ› ÌÂÙ¿ ¿ÚÔ‰Ô ¯ÚfiÓÔ˘, fiˆ˜ ÚԷӷʤÚıËÎÂ, Ô‰‹ÁËÛ Û Ӥ˜ ÌÂϤÙ˜ 2 ‹ 3 ·Ú¯ÈÎÒÓ ‰fiÛÂˆÓ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ηٿ ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ (ÌfiÓÔ˘ ‹ ÛÂ Û˘Ó‰˘·ÛÌÔ‡˜), ηıÒ˜ Î·È ¯ÔÚ‹ÁËÛ˘ ·Ó·ÌÓËÛÙÈ΋˜ ‰fiÛ˘ (29,30,31). √È ÌÂϤÙ˜ ·˘Ù¤˜ ‰Â›¯ÓÔ˘Ó fiÙÈ ÙÔ Û¯‹Ì· ÙˆÓ 2 ·Ú¯ÈÎÒÓ ‰fiÛÂˆÓ Â›Ó·È ·ÓÔÛÔÁÔÓÈÎfi Î·È ÚÔηÏ› ·ÓÔÛȷ΋ ÌÓ‹ÌË. ∂›Û˘, fiÙÈ Ë ·Ó·ÌÓËÛÙÈ΋ ‰fiÛË Â›Ó·È Î·Ù¿ÏÏËÏË ÁÈ· ÔÔÈÔ‰‹ÔÙ ‚·ÛÈÎfi Û¯‹Ì· ÂÌ‚ÔÏÈ·ÛÌÔ‡ Î·È Â›Û˘ ηٿÏÏËÏË Î·È ·ÓÔÛÔÁÔÓÈ΋ ÌÂÙ¿ ÙÔ Û¯‹Ì· ÙˆÓ 2 ‰fiÛÂˆÓ ÛÙÔÓ ‚·ÛÈÎfi ÂÌ‚ÔÏÈ·ÛÌfi (30,31). ŒÙÛÈ Î·ıÈÂÚÒıËΠÙÔ Û¯‹Ì· 2+1 (·fi ‚ÚÂÊÈ΋ ËÏÈΛ·) ‹ 1 ‰fiÛË fiÙ·Ó ÙÔ ÂÌ‚fiÏÈÔ Á›ÓÂÙ·È Û ËÏÈΛ· ¿Óˆ ÙˆÓ 12 ÌËÓÒÓ. ∞Ó Î·È Â›Ó·È Ôχ ÓˆÚ›˜ Ó· ·ÔÊ·Óı› ηÓ›˜ ÁÈ· ÙÔ ÔÈÔ Û¯‹Ì· ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈÎÔ‡ ÂÌ‚ÔÏ›Ô˘ Â›Ó·È Î·Ï‡ÙÂÚÔ, ˘¿Ú¯Ô˘Ó Ù· ÂÍ‹˜ ‰Â‰Ô̤ӷ: ·) Ë ÌËÓÈÁ-

ÁÈÙȉÔÎÔÎÎÈ΋ ÓfiÛÔ˜ ·˘Ù‹ ηı·˘Ù‹ ‰ÂÓ ÚÔηÏ› ÈÛ¯˘Ú‹ ·ÓÔÛ›· ÛÙȘ ÌÈÎÚ¤˜ ËÏÈ˘, ‚) Ë Û˘¯ÓfiÙËÙ· Ù˘ ÓfiÛÔ˘ Â›Ó·È ·˘ÍË̤ÓË ÛÙËÓ ÂÊË‚È΋ ËÏÈΛ·, fiˆ˜ ·˘ÍË̤ÓË Â›Ó·È Î·È Ë ‚·Ú‡ÙËÙ· Û’ ·˘Ù‹Ó ÙËÓ ËÏÈÎȷ΋ ÂÚ›Ô‰Ô. ™Â Ú·ÎÙÈÎÔ‡˜ fiÚÔ˘˜, ÔÈ ‰fiÛÂȘ ·Ó·˙ˆ‡ÚˆÛ˘ ÁÈ· Ù· Û˘˙¢Á̤ӷ ÂÌ‚fiÏÈ· Â›Ó·È ›Ûˆ˜ Ô ÈÔ Î·Ù¿ÏÏËÏÔ˜ ÙÚfiÔ˜ ‚ÂÏÙ›ˆÛ˘ Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ¿˜ ÙÔ˘˜. ∫¿ÙÈ Ô˘ ‹Ù·Ó ¿ÁÈ· ¿Ô„‹ Ì·˜ ·fi ÂÙÒÓ (32). µ¤‚·È·, ˘¿Ú¯Ô˘Ó ·ÎfiÌË ÚÔ‚Ï‹Ì·Ù· ÛÙÔ fiÙÂ Î·È ÛÙÔ Ò˜ ÁÈ· ÙȘ ‰fiÛÂȘ ·˘Ù¤˜. ∏ ¯Ú‹ÛË ÙˆÓ ÔÏ˘‰‡Ó·ÌˆÓ ÂÌ‚ÔÏ›ˆÓ Ô˘ ÂÍ·ÛıÂÓÔ‡Ó ÙËÓ ·ÓÙÈۈ̷ÙÈ΋ ·¿ÓÙËÛË Î¿ÔÈˆÓ ‰fiÛˆÓ, .¯. Ù˘ ‰Â‡ÙÂÚ˘ ‰fiÛ˘ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ÙÔ˘ ·ÈÌfiÊÈÏÔ˘ ÈÓÊÏÔ˘¤ÓÙ˙·˜ Ù‡Ô˘ b (24,26,33,34), ¯ˆÚ›˜ ÚÔ˜ ÙÔ ·ÚfiÓ ÎÏÈÓÈΤ˜ Û˘Ó¤ÂȘ, Î·È Ë ÌÂȈ̤ÓË ‰˘Ó·ÙfiÙËÙ· Ê˘ÛÈ΋˜ ·Ó·˙ˆ‡ÚˆÛ˘ Ô˘ ı· ·Ú·ÙËÚËı› Ù· ÂfiÌÂÓ· ¯ÚfiÓÈ· (·ÎÚÈ‚Ò˜ ÏfiÁˆ Ù˘ ¯Ú‹Û˘ ÙˆÓ ÂÌ‚ÔÏ›ˆÓ) ‰ËÌÈÔ˘ÚÁÔ‡Ó ÙÔ ÂÚÒÙËÌ· ÙÔ˘ Ò˜ ı· Û˘ÓÙËÚËı› Ë Ì·ÎÚÔ¯ÚfiÓÈ· ·ÓÔÛ›· Ô˘ ··ÈÙÂ›Ù·È ÁÈ· Ù· ÔÏ˘Û·Î¯·ÚȉÈο ·ÓÙÈÁfiÓ·. ª‹ˆ˜ Ì ·ÎfiÌË ÂÚÈÛÛfiÙÂÚ˜ ‰fiÛÂȘ; ¶ÈÛÙ‡ԢÌ fiÙÈ ÔÈ ·Ó·ÌÓËÛÙÈΤ˜ ‰fiÛÂȘ Â›Ó·È ·Ó·Áη›Â˜, ›Ûˆ˜ ÂÚÈÛÛfiÙÂÚÔ ÙˆÓ ·Ú¯ÈÎÒÓ. £· ÌÔÚÔ‡Û·Ì ̿ÏÈÛÙ· Ó· Û˘˙ËÙ‹ÛÔ˘Ì ¤Ó· ÂÓÈ·›Ô Û¯‹Ì· Î·È ÁÈ· Ù· 3 Û˘˙¢Á̤ӷ ÔÏ˘Û·Î¯·ÚȉÈο ÂÌ‚fiÏÈ·: 3 ·Ú¯ÈΤ˜ ‰fiÛÂȘ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· (ı· ÌÔÚÔ‡Û·Ó Ó· Â›Ó·È 2) Û˘Ó 1 ·Ó·ÌÓËÛÙÈ΋ ‰fiÛË ÛÙËÓ ËÏÈΛ· ÙˆÓ 18 ÌËÓÒÓ Û˘Ó ·ÎfiÌË 1 ·Ó·ÌÓËÛÙÈ΋ ηٿ ÙËÓ Â›ÛÔ‰Ô ÛÙÔ ‰ËÌÔÙÈÎfi Û¯ÔÏÂ›Ô (6 ¯ÚfiÓˆÓ). ™Â ·È‰È¿ Ô˘ ÁÈ· οÔÈÔ ÏfiÁÔ ÍÂÎÈÓÔ‡Ó ÙÔÓ ÂÌ‚ÔÏÈ·ÛÌfi Û ËÏÈΛ· ¿Óˆ ÙˆÓ 12-24 ÌËÓÒÓ, ıˆÚԇ̠··Ú·›ÙËÙË ÙË ¯ÔÚ‹ÁËÛË 2 ‰fiÛˆÓ: 1 + 1 ηٿ ÙËÓ Â›ÛÔ‰Ô ÛÙÔ ‰ËÌÔÙÈÎfi Î·È ›Ûˆ˜ 1 ·ÎfiÌË ‰fiÛË Î·Ù¿ ÙËÓ Â›ÛÔ‰Ô ÛÙÔ Á˘ÌÓ¿ÛÈÔ. Œˆ˜ fiÙÔ˘, ‚¤‚·È·, ·˘Ù¿ Á›ÓÔ˘Ó Ú¿ÍË (Â¿Ó ˘ÈÔıÂÙËıÔ‡Ó), Ô ·È‰›·ÙÚÔ˜ ı· Ú¤ÂÈ Ó· ÂÊ·ÚÌfi˙ÂÈ ÙȘ Ô‰ËÁ›Â˜ ÙÔ˘ ÂıÓÈÎÔ‡ ÚÔÁÚ¿ÌÌ·ÙÔ˜ ÂÌ‚ÔÏÈ·ÛÌÔ‡, ‰ÈfiÙÈ ÌfiÓÔÓ ¤ÙÛÈ ÂÈÙ˘Á¯¿ÓÔÓÙ·È ÂÓÈ·›· ·ÔÙÂϤÛÌ·Ù·.

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ÃÔÚ‹ÁËÛË ‰‡Ô ·ÓÙÈ-TNF Û΢·ÛÌ¿ÙˆÓ (etanercept Î·È adalimumab) Û ·È‰È¿ Ì Ó·ÓÈ΋ ȉÈÔ·ı‹ ·ÚıÚ›Ùȉ·: 5 ¯ÚfiÓÈ· ÌÂÙ¿ ª. ∆Ú·¯·Ó¿1, ¶. ¶Ú·ÙÛ›‰Ô˘-°Î¤ÚÙÛË1, º. ∫·Ó·ÎÔ‡‰Ë-∆۷ηϛ‰Ô˘1, ∫. ¢È¿Ê·1, °. ¶·Ú‰·Ïfi˜1, ª. ª·ÓÙÔ˘Ú¿ÎË2 ¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ·ÔÙ›ÌËÛË Ù˘ 5ÂÙÔ‡˜ ÂÌÂÈÚ›·˜ ·fi ÙË ¯Ú‹ÛË ÙˆÓ ‰‡Ô ·ÚÈˆÓ ·ÓÙÈTNF ·Ú·ÁfiÓÙˆÓ Ô˘ ¯ÔÚËÁÔ‡ÓÙ·È Û ·È‰È¿ Ì ÛÔ‚·Ú¤˜ ÌÔÚʤ˜ Ó·ÓÈ΋˜ ȉÈÔ·ıÔ‡˜ ·ÚıÚ›Ùȉ·˜ (NIA). ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ: ªÂÏÂÙ‹ıËÎ·Ó Ë ·ÛÊ¿ÏÂÈ· Î·È Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ etanercept (∂∆) Î·È adalimumab (AD) Û 46 ·ÛıÂÓ›˜ Ì ¡π∞, ËÏÈΛ·˜ 2-16 ÂÙÒÓ. √È 32/46 ¤Ï·‚·Ó ET Î·È ÔÈ 14/46 AD, Û˘Á¯ÚfiÓˆ˜ Ì ¤Ó· ÙÚÔÔÔÈËÙÈÎfi Ù˘ ÓfiÛÔ˘ Ê¿ÚÌ·ÎÔ (44/46), ΢ڛˆ˜ ÌÂıÔÙÚÂÍ¿ÙË Î·È Ú‰ÓÈ˙fiÓË (22/46). √È ·ÛıÂÓ›˜ ˘Ô‚¿ÏÏÔÓÙ·Ó ÛÂ Û˘ÛÙËÌ·ÙÈÎfi ÎÏÈÓÈÎfi, ·ÈÌ·ÙÔÏÔÁÈÎfi, ‚ÈÔ¯ËÌÈÎfi, ·ÓÔÛÔÏÔÁÈÎfi Î·È Î·Ú‰ÈÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÚÈÓ Î·È ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, ·Ó¿ 6ÌËÓÔ. ∏ ÂÎÙ›ÌËÛË Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ ‚·Û›ÛıËΠÛÙ· ÎÚÈÙ‹ÚÈ· «ACR ped 30, 50 Î·È 70». ∞ÔÙÂϤÛÌ·Ù·: ø˜ ÚÔ˜ ÙËÓ ·ÛÊ¿ÏÂÈ·, Û˘Ó‹ıÂȘ ÏÔÈÌÒÍÂȘ ÂÌÊ¿ÓÈÛ·Ó 17/46 ·ÛıÂÓ›˜ (10/32 ˘fi ∂∆ Î·È 3/14 ˘fi AD) Î·È ÛÔ‚·Ú¤˜ ÏÔÈÌÒÍÂȘ 4,7% (1 ˘fi ∂∆, 1 ˘fi AD). ÕÏϘ ÛÔ‚·Ú¤˜ ·ÓÂÈı‡ÌËÙ˜ ·ÓÙȉڿÛÂȘ ‰ÂÓ ·Ú·ÙËÚ‹ıËηÓ. ø˜ ÚÔ˜ ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·, score «ACRped 50-70» ¤Ù˘¯·Ó: ÙÔÓ 1Ô ¯ÚfiÓÔ 88% ÙˆÓ ·ÛıÂÓÒÓ ˘fi ∂∆ Î·È 71% ˘fi AD, ÙÔÓ 2Ô ¯ÚfiÓÔ 81% ˘fi ∂∆ Î·È 66,7% ˘fi AD Î·È ÙÔÓ 3Ô ¯ÚfiÓÔ 83% ˘fi ∂∆ Î·È 100% ˘fi AD. ™ÙË ‰È¿ÚÎÂÈ· Ù˘ 5ÂÙ›·˜, 11/46 ·ÛıÂÓ›˜ (28%) ÌÂÙ·‹‰ËÛ·Ó ·fi ÙÔÓ ¤Ó·Ó ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· ÛÙÔÓ ¿ÏÏÔÓ, 30,5% ‰È¤ÎÔ„·Ó ÏfiÁˆ ‡ÊÂÛ˘ Î·È ¿ÏÏÔÈ 60,8% ·¿ÓÙËÛ·Ó ÈηÓÔÔÈËÙÈο (ACRped 50-70), ÂÓÒ 8,7% ›¯·Ó ÊÙˆ¯‹ ·¿ÓÙËÛË Â›Ù ÛÙÔ ∂∆ ‹ ÛÙÔ AD. ™˘ÌÂÚ¿ÛÌ·Ù·: ∏ ¯ÔÚ‹ÁËÛË ÙˆÓ ·ÓÙÈ-TNF ·Ú·ÁfiÓÙˆÓ Û ·ÓıÂÎÙÈΤ˜ ÛÙË Û˘Ì‚·ÙÈ΋ ıÂÚ·›· NIA ·ÔÙÂÏ› ·ÛÊ·Ï‹ Î·È ÈηÓÔÔÈËÙÈ΋ ÂÓ·ÏÏ·ÎÙÈ΋ χÛË. øÛÙfiÛÔ, ·Ú¿ ÙÔ fiÙÈ Ë ÂÌÊ¿ÓÈÛË Ôχ ÛÔ‚·ÚÒÓ ÏÔÈÌÒÍÂˆÓ Â›Ó·È Û¯ÂÙÈο Û¿ÓÈ·, ··ÈÙÂ›Ù·È Û˘ÛÙËÌ·ÙÈ΋ Â·ÁÚ‡ÓËÛË ·fi ÙÔ˘˜ ıÂÚ¿ÔÓÙ˜ ÁÈ·ÙÚÔ‡˜ ÁÈ· Ó· ÌËÓ ¤¯Ô˘Ó ÌÔÈÚ·›· ¤Î‚·ÛË.

1 ¶·È‰È·ÙÚÈÎfi ∞ÓÔÛÔÏÔÁÈÎfi Î·È ƒÂ˘Ì·ÙÔÏÔÁÈÎfi ∫¤ÓÙÚÔ ∞Ó·ÊÔÚ¿˜, ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£ 2 ¶·È‰Ô·ÎÙÈÓÔÏÔÁÈÎfi ∆Ì‹Ì·, πÔÎÚ¿ÙÂÈÔ °¶¡£ AÏÏËÏÔÁÚ·Ê›·: ª·Ú›· ∆Ú·¯·Ó¿ mtrachan@auth.gr ∫ÏÂÈÔ‡˜ 6 54633 £ÂÛÛ·ÏÔÓ›ÎË

§¤ÍÂȘ ÎÏÂȉȿ: ¡Â·ÓÈ΋ ȉÈÔ·ı‹˜ ·ÚıÚ›Ùȉ·, etanercept, adalimumab, tumor necrosis factor, ıÂÚ·›· Ì ·ÓÙÈTNF ·Ú¿ÁÔÓÙ˜.

Administration of two anti-TNF agents (etanercept and adalimumab) in children with juvenile idiopathic arthritis: a 5-year follow-up study M. Trachana1, P. Pratsidou-Gertsi1, F. Kanakoudi-Tsakalidou1, C. Diafa1, G. Pardalos1, M. Bantouraki2 Abstract Background: The aim of this study was to evaluate the 5-year administration of etanercept (ET) or adalimumab (AD) to children with refractory types of juvenile idiopathic arthritis (JIA).

Methods: The safety and efficacy of two anti-TNF agents, namely ET and AD, were assessed in 46 children, aged 2-16 years. Of these, 32/46 received ET and 14/46 AD, accompanied by a DMARD (44/46), mainly methotrexate and prednisone (22/46). All the children were assessed by clinical, cardiological, haematological, biochemical and immunologic examination, both pre-treatment and at 6-monthly intervals during treatment. Efficacy was assessed by the application of the ACRped criteria 30, 50, and 70.

Results: a) Safety: Common respiratory tract infections were recorded in 17/46 (10/32 on ET and 3/14 on AD). Serious infections were recorded in 2 (4.7%) children (1 on ET, 1 on AD), but no other serious side-effects were observed. b) Efficacy: ACRped 50-70: 1st year: 88% of the ET and 68% of the AD group; 2nd year: 81% of the ET and 71% of the AD group; 3rd year: 83% of the ET and 100% of the AD group. During the 5-year study, 11/46 patients (28%) switched from ET to AD or vice versa, and 30.5% discontinued the anti-TNF treatment due to remission; 60.8% had a satisfactory response to anti-TNF treatment (ACRped 50-70), while 8.7% of patients had a poor response either to ET or to AD.

1 Paediatric Immunology and Rheumatology Referral Center, First Department of Paediatrics, Aristotle University of Thessaloniki, Thessaloniki, Greece 2 Outpatient Paediatric Radiology Clinic, “Ippokration” General Hospital, Thessaloniki, Greece Correspondence: Maria Trachana mtrachan@auth.gr 6, Klious St. 54633 Thessaloniki

∏ ·ÚÔ‡Û· ÂÚÁ·Û›· ‚Ú·‚‡ıËΠ̠ÙÔ ∞’ ÈڤÌÂÈÔ µÚ·‚›Ô, ÛÙÔ 46Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ∫¤Ú΢ڷ, 13-15 πÔ˘Ó›Ô˘ 2008 ¶·È‰È·ÙÚÈ΋ 2009;72:29-40

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ª. ∆Ú·¯·Ó¿ Î·È Û˘Ó.

Conclusions: Long-term administration of anti-TNF agents to patients with JIA refractory to the conventional regimen is a safe and alternative solution. Because of the rare appearance of serious infections, systematic vigilance is warranted in order to avoid a possible fatal outcome.

Key words: Juvenile idiopathic arthritis, etanercept, adalimumab, tumor necrosis factor, anti-TNF treatment.

∂ÈÛ·ÁˆÁ‹ H Ó·ÓÈ΋ ȉÈÔ·ı‹˜ ·ÚıÚ›Ùȉ· (¡π∞) Â›Ó·È Ë ÈÔ Û˘¯Ó‹ ÚÂ˘Ì·ÙÈ΋ ¿ıËÛË ÛÙ· ·È‰È¿, Ì ÌÂÁ¿ÏË ÔÈÎÈÏ›· ÎÏÈÓÈ΋˜ ¤ÎÊÚ·Û˘, ÔÚ›·˜ Î·È ¤Î‚·Û˘. √ÚÈṲ̂Ó˜ ÌÔÚʤ˜, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔÓ Ù‡Ô ¤Ó·Ú͢ (Û˘ÛÙËÌ·ÙÈÎfi, ÔÏÈÁÔ·ÚıÚÈÎfi ‹ ÔÏ˘·ÚıÚÈÎfi), ¤¯Ô˘Ó ÔÏ˘·ÚıÚÈ΋ ÔÚ›·, Â›Ó·È ·ÓıÂÎÙÈΤ˜ ÛÙË Û˘Ì‚·ÙÈ΋ ıÂÚ·›·, ·ÎfiÌË Î·È ÛÙ· ÈÔ ÂÈıÂÙÈο Û¯‹Ì·Ù·, Î·È Î·Ù·Ï‹ÁÔ˘Ó Û ηٷÛÙÚÔÊÈ΋ ·ÚıÚÔ¿ıÂÈ· Î·È Û ÛÔ‚·Ú‹ ÎÈÓËÙÈ΋ ·Ó·ËÚ›· (1). √È Ì˯·ÓÈÛÌÔ› Ô˘ ÂÌϤÎÔÓÙ·È ¿ÌÂÛ· ‹ ¤ÌÌÂÛ· ÛÙËÓ ·ıÔÁ¤ÓÂÛË Î·È Û˘ÓÙ‹ÚËÛË Ù˘ ¯ÚfiÓÈ·˜ ÊÏÂÁÌÔÓ‹˜ ÙÔ˘ ·ÚıÚÈÎÔ‡ ˘Ì¤Ó· ‰ÂÓ ¤¯Ô˘Ó ·ÎfiÌË ‰È¢ÎÚÈÓÈÛı›, Ê·›ÓÂÙ·È fï˜ fiÙÈ ÔÈ Î˘ÙÙ·ÚÔΛÓ˜ Ô˘ ÚÔ¿ÁÔ˘Ó ÙË ÊÏÂÁÌÔÓ‹ (proinflammatory cytokines IL-1, TNF, IL-6) ηÈ, ΢ڛˆ˜, Ô ·Ú¿ÁÔÓÙ·˜ Ó¤ÎÚˆÛ˘ ÙˆÓ fiÁÎˆÓ (TNF) ·›˙Ô˘Ó ÚˆÙ·ÁˆÓÈÛÙÈÎfi ÚfiÏÔ (2,3). °È· ÙÔ ÏfiÁÔ ·˘ÙfiÓ, ηٿ ÙËÓ ÙÂÏÂ˘Ù·›· 10ÂÙ›· ‰ÔÎÈÌ¿ÛÙËÎ·Ó Ó¤Â˜ ıÂÚ·¢ÙÈΤ˜ ÛÙÚ·ÙËÁÈΤ˜ ÛÙ· ·È‰È¿ Ô˘ ¿Û¯Ô˘Ó ·fi ·ÓıÂÎÙÈΤ˜ ÛÙË Û˘Ì‚·ÙÈ΋ ıÂÚ·›· ÌÔÚʤ˜ ¡π∞, ÔÈ Ôԛ˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‚ÈÔÏÔÁÈο ÚÔ˚fiÓÙ· Ô˘ ‰ÚÔ˘Ó ·ÓÙ·ÁˆÓÈÛÙÈο ÚÔ˜ ÙȘ ·Ó·ÊÂÚı›Û˜ ΢ÙÙ·ÚÔΛÓ˜ (4-5). √È ·ÓÙ·ÁˆÓÈÛÙ¤˜ ÙÔ˘ TNF-· ‹Ù·Ó ÔÈ ÚÒÙÔÈ ‚ÈÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ ‰ÔÎÈÌ¿ÛıËÎ·Ó Ì ÂÈÙ˘¯›·, ·Ú¯Èο ÛÙË ÚÂ˘Ì·ÙÔÂȉ‹ ·ÚıÚ›Ùȉ· (ƒ∞) ÙˆÓ ÂÓËÏ›ÎˆÓ Î·È ÛÙË Û˘Ó¤¯ÂÈ· ÛÙË ¡π∞. ™ÙË ¯ÒÚ· Ì·˜ ΢ÎÏÔÊÔÚÔ‡Ó Û‹ÌÂÚ· ÙÚ›· ·ÓÙÈ-TNF Û΢¿ÛÌ·Ù·: ÙÔ etanercept (∂∆, Enbrel©), ÙÔ infliximab (INFL, Remicade©) Î·È ÙÔ adalimumab (AD, Humira©). ∆Ô ∂∆ Â›Ó·È ‰ÈÌÂÚ¤˜ ÌÈ·˜ ¯ÈÌ·ÈÚÈ΋˜ ÚˆÙ½Ó˘ Ô˘ ÚÔ·ÙÂÈ ·fi ÙË Û‡ÓÙËÍË ÙÔ˘ ÂÍˆÎ˘ÙÙ¿ÚÈÔ˘ ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ˘Ô‰Ô¯¤·-2 ÙÔ˘ TNF Ì ÙÔ ÙÌ‹Ì· Fc Ù˘ ·ÓıÚÒÈÓ˘ ·ÓÔÛÔÛÊ·ÈÚ›Ó˘ IgG1. ∆Ô Û··ÛÌ· ‰Ú· ˆ˜ ·Ó·ÛÙÔϤ·˜ Ù˘ Û‡Ó‰ÂÛ˘ ÙÔ˘ TNF Ì ÙÔÓ ÂÈÊ·ÓÂÈ·Îfi ΢ÙÙ·ÚÈÎfi ˘Ô‰Ô¯¤· ÙÔ˘ Î·È Û˘ÓÂÒ˜ ·Ó·ÛÙ¤ÏÏÂÈ ÙË ‰Ú¿ÛË ÙÔ˘ (4-6). ∆Ô INFL Â›Ó·È ¤Ó· ¯ÈÌ·ÈÚÈÎfi ÌÔÓÔÎψÓÈÎfi ·ÓÙÈ-TNF ·Óٛۈ̷ Ô˘ ·ÔÙÂÏÂ›Ù·È Î·Ù¿ 25% ·fi IgG1 ÔÓÙÈÎÔ‡ (Fab ÙÌ‹Ì·) Î·È Î·Ù¿ 75% ·fi IgG1 ·ÓıÚÒÔ˘ (Fc ÙÌ‹Ì·). ∆Ô ÚÒPaediatriki 2009;72:29-40

ÙÔ ·ÔÙÂÏ› ÙÔ ÙÌ‹Ì· Û‡Ó‰ÂÛ˘ ÙÔ˘ INFL Ì ÙÔ ‰È·Ï˘Ùfi Î·È ‰È·ÌÂÌ‚Ú·ÓÈÎfi ∆NF-·, ÂÓÒ ÙÔ ‰Â‡ÙÂÚÔ (Fc ÙÌ‹Ì·) ·Ó·ÛÙ¤ÏÏÂÈ ÙȘ ¿ÏϘ ‚ÈÔÏÔÁÈΤ˜ ‰Ú·ÛÙËÚÈfiÙËÙ˜ ÙÔ˘ TNF-· (4-6). ∆Ô adalimumab (AD) Â›Ó·È ÙÔ ÚÒÙÔ ÂÍ ÔÏÔÎÏ‹ÚÔ˘ ·ÓıÚÒÈÓÔ ÌÔÓÔÎψÓÈÎfi ·ÓÙÈTNF ·Óٛۈ̷ Ô˘ ‰ÔÎÈÌ¿ÛÙËΠ·Ú¯Èο ÛÙË ıÂÚ·›· Ù˘ ƒ∞ ÙˆÓ ÂÓËϛΈÓ. ªÔÈ¿˙ÂÈ ‰ÔÌÈο Î·È ÏÂÈÙÔ˘ÚÁÈο Ì ÙËÓ ·ÓÔÛÔÛÊ·ÈÚ›ÓË IgG1. ∞Ó·ÛÙ¤ÏÏÂÈ Î·È ·˘Ùfi, fiˆ˜ ÙÔ ∂∆, ÙË Û‡Ó‰ÂÛË ÙÔ˘ TNF Ì ÙÔÓ ÂÈÊ·ÓÂÈ·Îfi ΢ÙÙ·ÚÈÎfi ˘Ô‰Ô¯¤· ÙÔ˘. ∂ÈϤÔÓ, ¤¯ÂÈ ¯·ÌËÏ‹ ·ÓÔÛÔÁÔÓÈÎfiÙËÙ·, ȉÈfiÙËÙ· Ô˘ ÙÔ˘ ÂÈÙÚ¤ÂÈ ÙË Ì·ÎÚÔ¯ÚfiÓÈ· ¯ÔÚ‹ÁËÛ‹ ÙÔ˘ ¯ˆÚ›˜ ÙÔ ÂӉ¯fiÌÂÓÔ ÚÒÈÌ˘ ·Ú·ÁˆÁ‹˜ ÂÍÔ˘‰ÂÙÂÚˆÙÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÙÔ˘, ·ÎfiÌË Î·È fiÙ·Ó ‰ÂÓ Ï·Ì‚¿ÓÔÓÙ·È Ù·˘Ùfi¯ÚÔÓ· ·ÓÔÛÔÙÚÔÔÔÈËÙÈο Ê¿Ú̷η, fiˆ˜ Ë ÌÂıÔÙÚÂÍ¿ÙË, ηıÒ˜ Î·È ¯·ÌËÏ‹ Èı·ÓfiÙËÙ· ·ÏÏÂÚÁÈÎÒÓ ·ÓÙȉڿÛÂˆÓ (7). ™Â ÚÔËÁÔ‡ÌÂÓË ÌÂϤÙË Ì·˜ ÂÎÙÈÌ‹ıËÎÂ Ë ·ÛÊ¿ÏÂÈ· Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ‰‡Ô ·ÓÙÈTNF Û΢·ÛÌ¿ÙˆÓ (∂∆ Î·È INFL) Ô˘ ¯ÔÚËÁ‹ıËÎ·Ó ÁÈ· 9 Ì‹Ó˜ Û ÂÈÏÂÁ̤ÓÔ˘˜ ·ÛıÂÓ›˜ Ì ·ÓıÂÎÙÈ΋ ÛÙË Û˘Ì‚·ÙÈ΋ ıÂÚ·›· ¡π∞, Ë ÔÔ›· ·ÔÙ¤ÏÂÛ ÙËÓ ÚÒÙË Î·Ù·ÁÚ·Ê‹ Ù˘ ·ÛÊ¿ÏÂÈ·˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ Ù˘ ·ÓÙÈTNF ıÂÚ·›·˜ Û ·È‰È¿ ÛÙË ¯ÒÚ· Ì·˜ (1). ∏ Û˘ÛÛÒÚ¢ÛË Ù˘ ÂÌÂÈÚ›·˜ Ì·˜ ·fi ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙˆÓ ·ÓÙÈ-TNF ·Ú·ÁfiÓÙˆÓ ÛÙË ¡π∞ Ì·˜ Ô‰‹ÁËÛÂ, ·fi ÙÔ 2003 Î·È ÌÂÙ¿, ÛÙËÓ ÂÈÏÔÁ‹ ¯ÚËÛÈÌÔÔ›ËÛ˘ ÌfiÓÔ ÙÔ˘ ET Î·È ÙÔ˘ AD, ÁÈ·Ù› Ì ÙÔ INFL ηٷÁÚ¿ÊËÎ·Ó ·ÊÂÓfi˜ ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ ÛÔ‚·ÚÒÓ ·ÓÂÈı‡ÌËÙˆÓ ·ÓÙȉڿÛÂˆÓ (΢ڛˆ˜ ·Ó·Ê˘Ï·ÎÙÈÎÔ‡ Ù‡Ô˘) Î·È ·ÊÂÙ¤ÚÔ˘ ÌÈ· Êı›ÓÔ˘Û· ÔÚ›· Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ¿˜ ÙÔ˘ (Èı·ÓÒ˜ ÏfiÁˆ ·Ó¿Ù˘Í˘ ÂÍÔ˘‰ÂÙÂÚˆÙÈÎÒÓ ·ÓÙÈۈ̿وÓ), ‹‰Ë ·fi ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ¯ÔÚ‹ÁËÛ‹˜ ÙÔ˘. ∏ ÙÚ¤¯Ô˘Û· ÌÂϤÙË Û¯Â‰È¿ÛÙËΠ̠ÛÎÔfi Ó· ·ÔÙÈÌ‹ÛÂÈ ÙËÓ 5ÂÙ‹ ÂÌÂÈÚ›· Ì·˜ ·fi ÙË ¯Ú‹ÛË ÙˆÓ ÚÔ·Ó·ÊÂÚı¤ÓÙˆÓ ‰‡Ô ·ÓÙÈ-TNF ·Ú·ÁfiÓÙˆÓ Ô˘ ¯ÚËÛÈÌÔÔÈԇ̠ۋÌÂÚ· Û ·È‰È¿ Ì ÛÔ‚·Ú¤˜ ÌÔÚʤ˜ Ó·ÓÈ΋˜ ȉÈÔ·ıÔ‡˜ ·ÚıÚ›Ùȉ·˜ (NIA) ÛÙÔ Î¤ÓÙÚÔ Ì·˜.

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¶ÂÓÙ·ÂÙ‹˜ ÂÌÂÈÚ›· ·fi ÙË ¯Ú‹ÛË etanercept Î·È adalimumab ÛÙË Ó·ÓÈ΋ ȉÈÔ·ı‹ ·ÚıÚ›Ùȉ·

¶›Ó·Î·˜ 1. ∫˘ÚÈfiÙÂÚ· ÎÏÈÓÈο ‰Â‰Ô̤ӷ ÙˆÓ 46 ·ÛıÂÓÒÓ Ô˘ ÌÂÏÂÙ‹ıËηÓ

∞Ú¯ÈÎfi˜ ·ÚÈıÌfi˜ ·ÛıÂÓÒÓ º‡ÏÔ (∫/∞) ∞ÚÈıÌfi˜ ·ÛıÂÓÒÓ Ô˘ ÌÂÙ·‹‰ËÛ·Ó ·fi ÙË Ì›· ÔÌ¿‰· ÛÙËÓ ¿ÏÏË* ∞ÚÈıÌfi˜ ·ÓÙÈ-TNF ıÂÚ·¢ÙÈÎÒÓ Û˘Ó‰ÚÈÒÓ Ô˘ ηٷÁÚ¿ÊËÎ·Ó ÛÙÔ˘˜ 46 ·ÛıÂÓ›˜ ª¤ÛË ËÏÈΛ· ¤Ó·Ú͢ NIA (Û ¯ÚfiÓÈ·), x(±SD) ª¤ÛË ËÏÈΛ· ¤Ó·Ú͢ ·ÓÙÈ-TNF ıÂÚ·›·˜ (Û ¯ÚfiÓÈ·), x(±SD) ∆‡Ô˜ ·ÚıÚ›Ùȉ·˜ ™˘ÛÙËÌ·ÙÈ΋ → ÔÏ˘·ÚıÚ›Ùȉ· ™˘ÛÙËÌ·ÙÈ΋ → Û˘ÛÙËÌ·ÙÈ΋ ¶ÔÏ˘·ÚıÚ›Ùȉ· (RF ıÂÙÈ΋) √ÏÈÁÔ·ÚıÚ›Ùȉ· ÂÂÎÙ·ı›۷ √ÏÈÁÔ·ÚıÚ›Ùȉ· Â›ÌÔÓË æˆÚÈ·ÛÈ΋ ∞ÚıÚ›Ùȉ· Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÂÓıÂÛ›Ùȉ· ∞ÛıÂÓ›˜ Ì ˘ÔÙÚÔÈ¿˙Ô˘Û· ÈÚȉÔ΢ÎÏ›Ùȉ· ª¤ÛË ‰È¿ÚÎÂÈ· ÓfiÛÔ˘ ÚÈÓ ÙËÓ ¤Ó·ÚÍË ıÂÚ·›·˜ Ì ·ÓÙÈ-TNF - [¤ÙË (‡ÚÔ˜)] ¶ÚÔËÁÔ‡ÌÂÓË ıÂÚ·›· ÛÙÔ Û‡ÓÔÏÔ ÙˆÓ ·ÓÙÈ-TNF Û˘Ó‰ÚÈÒÓ Ô˘ ¯ÔÚËÁ‹ıËÎ·Ó ÛÙÔ˘˜ 46 ·ÛıÂÓ›˜ (¡=59) ∫·Ì›·† ª∆à CSA ª∆à + CSA ª∆à + ¿ÏÏ· DMARDs‡ CSA + ˘‰ÚÔ͢¯ÏˆÚÔΛÓË INFL INFL + MTX INFL + CSA ∂∆ + MTX ‹ CSA AD + MTX ‹ CSA ¢ÔÛÔÏÔÁÈÎfi Û¯‹Ì· ·ÓÙÈ-TNF ·Ú·ÁfiÓÙˆÓ ¶·Ú¿ÏÏËÏË ıÂÚ·›· ÛÙÔ Û‡ÓÔÏÔ ÙˆÓ ıÂÚ·¢ÙÈÎÒÓ Û˘Ó‰ÚÈÒÓ Ô˘ ηٷÁÚ¿ÊËÎ·Ó ÛÙÔ˘˜ 46 ·ÛıÂÓ›˜ (¡=59) PDN (ÛÂ Û˘Ó‰˘·ÛÌfi Ì DMARDS Î·È Ì ·ÓÙÈ-TNF)¨ MTX CSA

∂∆

AD

™‡ÓÔÏÔ

32 25/7 4

14 10/4 7

46 35/11 11/46

37 4,84 (±3,59)** 10,69 (±5,38)**

22 4,69 (±3,38)** 12,92 (±4,96)**

59

4 4 16 (4) 6 1 1 0 6

1 0 6 (1) 4 2 0 1 1

5 4 22 (5) 10 3 1 1 7

5,85 (0-16,5)**

8,23 (1,7-19,7)**

2/37 6/37 0/37 16/37 3/37 2/37 0/37 4/37 0/37

0/22 1/22 1/22 11/22 1/22 0/22 1/22 3/22 1/22 7/22

4/37 0,4 mg/kg ‰È˜/‚‰

25 mg/m2/15ËÌ

14/37 31/37 6/37@

11/22 19/22 3/22

2/59 7/59 1/59 27/59 4/59 2/59 1/59 5/59 1/59 7/59 4/59

25/59 50/59 9/59

¶ÔÏ˘- = ÔÏ˘·ÚıÚÈ΋, √ÏÈÁÔ- = ÔÏÈÁÔ·ÚıÚÈ΋, → = ÌÂÙ¿ÙˆÛË ÛÂ, ™˘ÛÙËÌ·ÙÈ΋ → Û˘ÛÙËÌ·ÙÈ΋ = Û˘ÛÙËÌ·ÙÈÎfi˜ Ù‡Ô˜ ¤Ó·Ú͢ Î·È ÔÚ›·˜, RF = ÚÂ˘Ì·ÙÔÂȉ‹˜ ·Ú¿ÁÔÓÙ·˜ ∂∆=Etanercept, AD=Adalimumab, PDN=Ú‰ÓÈ˙fiÓË, MTX=ÌÂıÔÙÚÂÍ¿ÙË, CsA=∫˘ÎÏÔÛÔÚ›ÓË ∞ * 2/11 ·ÛıÂÓ›˜ ‹Ú·Ó 2 ÊÔÚ¤˜ ›Ù ∂∆ (1) ›Ù AD (1), ·ÓÙ›ÛÙÔȯ· ** p = NS † ∫·Ó¤Ó· DMARD ÏfiÁˆ ‰˘Û·ÓÔ¯‹˜ ‹ ·ÓÂÈı‡ÌËÙˆÓ ÂÓÂÚÁÂÈÒÓ ‡ √Ì¿‰· ∂∆: ÛÔ˘ÏÊ·Û·Ï·˙›ÓË 1, ˘‰ÚÔ͢¯ÏˆÚÔΛÓË (1), ¿Ï·Ù· ¯Ú˘ÛÔ‡ (1). √Ì¿‰· AD: ˘‰ÚÔ͢¯ÏˆÚÔΛÓË (1) ¨ ¢fiÛË <0.5mg/kg/ËÌ. Ì Êı›ÓÔ˘Û· ÂÏ¿ÙÙˆÛË, Ô˘ ‰ÈÂÎfiË ÌÂÙ¿ ‰È¿ÛÙËÌ· 6-12 ÌËÓÒÓ @ 1 ·ÛıÂÓ‹˜ Û˘Ó¤¯ÈÛ Ì ÏÂÊÏÔ˘ÓÔÌ›‰Ë ÌÂÙ¿ ÙÔÓ 2Ô ¯ÚfiÓÔ, ÏfiÁˆ ·ÓÂÈı‡ÌËÙˆÓ ·ÓÙȉڿÛÂˆÓ ÛÙË CsA

∞ÛıÂÓ›˜ Î·È ª¤ıÔ‰ÔÈ ∞ÛıÂÓ›˜ ªÂÏÂÙ‹ıËÎ·Ó ÚÔÔÙÈο 46 ·ÛıÂÓ›˜ (∫/∞ 34/12), ËÏÈΛ·˜ 2-16 ÂÙÒÓ, Ô˘ ¤·Û¯·Ó ·fi NIA. ∫ÚÈÙ‹ÚÈ· ÂÈÛ·ÁˆÁ‹˜ ÛÙË ÌÂϤÙË: ãŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ ›¯·Ó Â›ÌÔÓË ÂÓÂÚÁfiÙËÙ· Ù˘ ÓfiÛÔ˘, ·Ú¿ ÙËÓ ÂÈıÂÙÈ΋ Û˘Ì‚·ÙÈ΋ ıÂÚ·›· ‹ Î·È ÙË ıÂÚ·›· Ì ÙÔÓ ÂÓ‰ÔÊϤ‚È· ¯ÔÚËÁÔ‡ÌÂÓÔ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· (INFL). ∂ȉÈÎfiÙÂÚ·, fiÏÔÈ ÔÈ ·ÛıÂÓ›˜, ÂÎÙfi˜ ·fi 2, ›¯·Ó Ï¿‚ÂÈ ¤Ó· ‹ ÂÚÈÛÛfiÙÂÚ· ÙÚÔÔÔÈËÙÈο Ù˘ ÓfiÛÔ˘ Ê¿Ú̷η (disease modifying antirheumatic

drugs ‹ DMARDs), Ì ‹ ¯ˆÚ›˜ Ú‰ÓÈ˙fiÓË. √È ‰‡Ô ÂÍ·ÈÚÔ‡ÌÂÓÔÈ ·ÛıÂÓ›˜ ›¯·Ó ‰˘Û·ÓÔ¯‹ ÛÙ· DMARDs (¤ÓÙÔÓ˜ Á·ÛÙÚÂÓÙÂÚÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÙÚ·ÓÛ·ÌÈÓ·Û·ÈÌ›· ÛÙË ª∆Ã, ÓÂÊÚÔÙÔÍÈÎfiÙËÙ· ÛÙË CsA) Î·È Â›¯·Ó ‰È·Îfi„ÂÈ ÙËÓ ·ÁˆÁ‹. ∞Ó·Ï˘ÙÈο, ÙÔ Â›‰Ô˜ Ù˘ ÚÔËÁËı›۷˜ ıÂÚ·›·˜ ηٷÁÚ¿ÊÂÙ·È ÛÙÔÓ ¶›Ó·Î· 1. ∂ÈÏÔÁ‹ ·ÓÙÈ-∆¡F ·Ú¿ÁÔÓÙ·: ª¤¯ÚÈ ÙÔ 2003 ÙÔ AD ‰ÂÓ Î˘ÎÏÔÊÔÚÔ‡Û ÛÙË ¯ÒÚ· Ì·˜. ãŒÙÛÈ, ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË Î·Ù·ÁÚ¿ÊÂÙ·È Ë 4¯ÚÔÓË ÂÌÂÈÚ›· ·fi ÙË ¯ÔÚ‹ÁËÛ‹ ÙÔ˘ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ô˘ ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È ÛÙÔ Î¤ÓÙÚÔ Ì·˜. ¶·È‰È·ÙÚÈ΋ 2009;72:29-40

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·32

32

ª. ∆Ú·¯·Ó¿ Î·È Û˘Ó.

∞Ú¯Èο, ÙÔ AD ·ÔÙ¤ÏÂÛ ÙËÓ ÂÓ·ÏÏ·ÎÙÈ΋ χÛË ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì Ùˆ¯‹ ·¿ÓÙËÛË ÛÙÔ ∂∆. øÛÙfiÛÔ, ÙËÓ ÙÂÏÂ˘Ù·›· 2ÂÙ›· Ù˘ ÌÂϤÙ˘, ·ÔÙÂÏÔ‡Û ÙËÓ ÚÒÙË ÂÈÏÔÁ‹ Û ·È‰È¿ Ì ¡π∞ Î·È ÈÚȉÔ΢ÎÏ›Ùȉ· (π∫), ÁÈ·Ù› ÂÓÙˆÌÂٷ͇ ÚԤ΢„·Ó ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ Ô˘ ˘ÔÛÙ‹ÚÈ˙·Ó fiÙÈ ÙÔ ∂∆ ‰ÂÓ ·Ó·ÛÙ¤ÏÏÂÈ ÙËÓ ÂÌÊ¿ÓÈÛË π∫ ηٿ ÙËÓ ÔÚ›· Ù˘ ¡π∞ Î·È ‰ÂÓ ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÔÚ›· Ù˘ ‹‰Ë ÂÁηٷÛÙË̤Ó˘ π∫ (8-10). ¢ËÌÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ: ∆· ΢ÚÈfiÙÂÚ· ÎÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·ÛıÂÓÒÓ ÙˆÓ ‰‡Ô ıÂÚ·¢ÙÈÎÒÓ ÔÌ¿‰ˆÓ Û˘ÓÔ„›˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1 ηÈ, fiˆ˜ Ê·›ÓÂÙ·È, ‰ÂÓ ‰È¤ÊÂÚ·Ó ÛËÌ·ÓÙÈο ÌÂٷ͇ ÙÔ˘˜. √È ·ÛıÂÓ›˜ Ô˘ ÛÙËÓ ÔÚ›· Ù˘ ÌÂϤÙ˘ ›¯·Ó Êı›ÓÔ˘Û· ¤ˆ˜ ÊÙˆ¯‹ ·¿ÓÙËÛË ÌÂÙ·Ë‰Ô‡Û·Ó ·fi ÙË Ì›· ÛÙËÓ ¿ÏÏË ÔÌ¿‰· ıÂÚ·›·˜ (.¯. ·fi ∂∆ Û AD ‹ Î·È ·ÓÙ›ÛÙÚÔÊ·), Û˘Ó‹ıˆ˜ Ì›· ‹ ηÈ, Û·ÓÈfiÙÂÚ·, ‰‡Ô ÊÔÚ¤˜. ∂›Û˘, ÔÈ ·ÛıÂÓ›˜ Ô˘ ‰È¤ÎÔÙ·Ó ÙÔÓ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· ÏfiÁˆ ‡ÊÂÛ˘ Î·È ÛÙËÓ ÔÚ›· ˘ÔÙÚÔ›·˙·Ó ¿Ú¯È˙·Ó Í·Ó¿ ıÂÚ·›· Ì ÙÔÓ ¿ÏÏÔ ·ÓÙÈ- TNF ·Ú¿ÁÔÓÙ·, ÔfiÙ ηٷٿÛÛÔÓÙ·Ó ÛÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ÔÌ¿‰· ıÂÚ·›·˜. ãŒÙÛÈ, ÔÈ 46 ·ÛıÂÓ›˜ ¤Ï·‚·Ó Û˘ÓÔÏÈο 57 ıÂÚ·›˜. ∞Ó·Ï˘ÙÈο, 4 ·ÛıÂÓ›˜ ÌÂÙ·‹‰ËÛ·Ó ·fi ÙËÓ ÔÌ¿‰· ÙÔ˘ AD ÛÙÔ ∂∆ Î·È 7 ·fi ÙÔ ET ÛÙÔ AD. ¢‡Ô ·ÛıÂÓ›˜ Ì ÛÔ‚·Ú‹ ÔÏ˘·ÚıÚÈ΋ ÌÔÚÊ‹ ‹Ú·Ó ÙÔÓ ›‰ÈÔ ·Ú¿ÁÔÓÙ· 2 ÊÔÚ¤˜ ÛÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘ (∂∆, AD, ET ‹ AD, ∂∆, AD) (¶›Ó·Î·˜ 1). ∂Ù¿ ·ÛıÂÓ›˜ ›¯·Ó ˘ÔÙÚÔÈ¿˙Ô˘Û· π∫ ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Ì ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ˜ Î·È Ë Â›‰Ú·Û‹ ÙÔ˘˜ ÛÙËÓ ÔÚ›· Ù˘ π∫ ÂÎÙÈÌ‹ıËΠ‰È·¯ÚÔÓÈο. ™ÙÔÓ ¶›Ó·Î· 1 ·Ó·Ê¤ÚÂÙ·È ÙÔ ‰ÔÛÔÏÔÁÈÎfi Û¯‹Ì· Ì ÙÔ˘˜ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÊ·ÚÌfiÛıËΠÛÙË ÌÂϤÙË. ∂ȉÈÎfiÙÂÚ·, ÙÔ Û··ÛÌ· ∂∆ ¯ÔÚËÁ‹ıËΠ˘Ô‰fiÚÈ· Û ‰fiÛË 0,4 mg/kg, ‰‡Ô ÊÔÚ¤˜ ‚‰ÔÌ·‰È·›ˆ˜ Î·È ÙÔÓ ÙÂÏÂ˘Ù·›Ô ¯ÚfiÓÔ ÛÙË ‰ÈÏ¿ÛÈ· ‰fiÛË Ì›· ÊÔÚ¿ ÙËÓ Â‚‰ÔÌ¿‰· (n=2). ∆Ô AD ¯ÔÚËÁ‹ıËΠ۠‰fiÛË 25 mg/m2 ·Ó¿ 15‹ÌÂÚÔ Î·È Û ÔÚÈṲ̂Ó˜ ÂÚÈÙÒÛÂȘ ·Ó¿ ‚‰ÔÌ¿‰· (n=4). √È ÚÒÙ˜ 2-3 ÂÓ¤ÛÂȘ Á›ÓÔÓÙ·Ó ÛÙÔ ¶·È‰ÔÚÂ˘Ì·ÙÔÏÔÁÈÎfi π·ÙÚÂ›Ô Î·È ÔÈ ÁÔÓ›˜ ÂÎ·È‰Â‡ÔÓÙ·Ó ÛÙËÓ ˘Ô‰fiÚÈ· ¯ÔÚ‹ÁËÛË ÙÔ˘ Û΢¿ÛÌ·ÙÔ˜. ™Â ÂÚ›ÙˆÛË ·‰˘Ó·Ì›·˜ ÙÔ˘˜, ÔÈ ˘Ô‰fiÚȘ ÂÓ¤ÛÂȘ Á›ÓÔÓÙ·Ó Ì ¢ı‡ÓË ÙÔ˘ ·È‰È¿ÙÚÔ˘, ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ ‹ ÙÔ˘ ΤÓÙÚÔ˘ ˘Á›·˜ Ù˘ ÂÚÈÔ¯‹˜ ÙÔ˘ ·ÛıÂÓÔ‡˜, ÌÂÙ¿ ·fi Û˘ÓÂÓÓfiËÛË. ¶·Ú¿ÏÏËÏ· Ì ÙÔ˘˜ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ˜, fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ ¤·ÈÚÓ·Ó ª∆à ‹ ΢ÎÏÔÛÔÚ›ÓË (CSA) ‹ Î·È Ú‰ÓÈ˙fiÓË (PDN Û ¯·ÌËÏ‹ ‰fiÛË Î·È Û ·Ú‹ÌÂÚÔ Û¯‹Ì·), Ô˘ ‰ÈÂÎfiË ÌÂÙ¿ 6-12 Ì‹Ó˜ (¶›Ó·Î·˜ 1). ª¤ıÔ‰ÔÈ ∏ ÌÂıÔ‰ÔÏÔÁ›· Ù˘ ÚÔÔÙÈ΋˜ ·˘Ù‹˜ ÌÂϤÙ˘ ÂÚÈÏ¿Ì‚·Ó ٷ ÂÍ‹˜: 1. ∂ÎÙ›ÌËÛË Ù˘ ÂÓÂÚÁfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ ÚÈÓ ·Ú¯›ÛÂÈ Ë ¯ÔÚ‹ÁËÛË ÙˆÓ ·ÓÙÈ-TNF ·Ú·ÁfiÓÙˆÓ, Ì ‚¿ÛË Ù· ‰ÈÂıÓÒ˜ ·Ô‰ÂÎÙ¿ ÎÚÈÙ‹ÚÈ· ÙˆÓ Giannini Î·È Û˘Ó. (11), Ô˘ ˘ÈÔıÂÙ‹ıËÎ·Ó ·fi ÙÔ ACR (American College of Rheumatologists) Î·È ÔÓoÌ¿˙ÔÓÙ·È ϤÔÓ ACR pediatric criteria (ACRped). ∆· ÎÚÈÙ‹ÚÈ· ·˘Ù¿ ÂÎÙÈÌÔ‡Ó ÙËÓ Î·Ù¿ 30, 50 ‹ 70% ‚ÂÏÙ›ˆÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ (ACRped 30, 50, 70) Î·È ÂÊ·ÚÌfi˙ÔÓÙ·È Û fiϘ ÙȘ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ‰ÈÂıÓÒ˜. ø˜ ÂÏ¿¯ÈÛÙË ‚ÂÏÙ›ˆÛË ıˆÚÂ›Ù·È ÙÔ ACRped 30. √È ÂÚÈÛÛfiÙÂÚÔÈ Û˘ÁÁÚ·Ê›˜, ˆÛÙfiÛÔ, ÚÔÙ›ÓÔ˘Ó ˆ˜ ÈηÓÔÔÈËÙÈ΋ ÙË ‚ÂÏÙ›ˆÛË Î·Ù¿ 50% Î·È ˆ˜ Ôχ ÈηÓÔÔÈËÙÈ΋ ÙË ‚ÂÏÙ›ˆÛË Î·Ù¿ 70% (12). ∆· ÎÚÈÙ‹ÚÈ· ·˘Ù¿ ÂÎÙÈÌÔ‡Ó ÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË Î·È Â˘ÂÍ›· ÙÔ˘ ·ÛıÂÓÔ‡˜, ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ÙÔ˘ ÈηÓfiÙËÙ· Î·È ÙËÓ ÔÈfiÙËÙ· Ù˘ ˙ˆ‹˜ ÙÔ˘ Û ۯ¤ÛË Ì ÙË ¯ÚfiÓÈ· ÓfiÛÔ ÛÙË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ Ô˘ Á›ÓÂÙ·È Ë ÂÎÙ›ÌËÛË. ¶ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙȘ ÂÍ‹˜ 6 ˘ÔÎÂÈÌÂÓÈΤ˜ Î·È ·ÓÙÈÎÂÈÌÂÓÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜: 1. ∆Ë ÛÊ·ÈÚÈ΋ ÂÎÙ›Paediatriki 2009;72:29-40

ÌËÛË Ù˘ ÁÂÓÈ΋˜ ηٿÛÙ·Û˘ (‚·Ú‡ÙËÙ· ÓfiÛÔ˘) ÙÔ˘ ·ÛıÂÓÔ‡˜ ·fi ÙÔÓ ıÂÚ¿ÔÓÙ· È·ÙÚfi, Ì ‚·ıÌÔÏfiÁËÛË ÔÙÈ΋˜ ·Ó·ÏÔÁÈ΋˜ Îϛ̷η˜ (VAS) 0-10cm. 2. ∆Ë ÛÊ·ÈÚÈ΋ ÂÎÙ›ÌËÛË Ù˘ ¢ÂÍ›·˜ (well-being) ·fi ÙÔÓ ›‰ÈÔ ÙÔÓ ·ÛıÂÓ‹ ‹ ÙÔ˘˜ ÁÔÓ›˜ ÙÔ˘, Ì ‚·ıÌÔÏfiÁËÛË VAS 0-10cm. 3. ∆Ë ‚·ıÌÔÏfiÁËÛË Ù˘ ÏÂÈÙÔ˘ÚÁÈ΋˜ ÈηÓfiÙËÙ·˜ ÙÔ˘ ·ÛıÂÓÔ‡˜ ·fi ÙÔÓ ›‰ÈÔ ‹ ÙÔ˘˜ ÁÔÓ›˜ ÙÔ˘, Ì ··ÓÙ‹ÛÂȘ Û ÂȉÈÎfi ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ Child Health Assessment Questionnaire (CHAQ). ∞fi ÙÔ CHAQ ÂÍ¿ÁÂÙ·È Ô ¢Â›ÎÙ˘ ªÂÈÔÓÂÎÙÈÎfiÙËÙ·˜ (Disability Index, score 0-3) ÙÔ˘ ·ÛıÂÓÔ‡˜. À¿Ú¯ÂÈ Ë ÂÏÏËÓÈ΋ ¤Î‰ÔÛË ÙÔ˘ CHAQ, Ë ÔÔ›· ¤¯ÂÈ ÂÁÎÚÈı› ·fi ¢ÈÂıÓ›˜ ¶·È‰ÔÚÂ˘Ì·ÙÔÏÔÁÈÎÔ‡˜ √ÚÁ·ÓÈÛÌÔ‡˜ Î·È ¤¯ÂÈ ‰ËÌÔÛÈ¢ı› ÙÔ 2001 (13). ∆Ô CHAQ ÂÊ·ÚÌfi˙ÂÙ·È ·fi 5ÂÙ›·˜ Î·È ϤÔÓ ÛÙÔ Î¤ÓÙÚÔ Ì·˜, ÛÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË Î¿ı ·ÛıÂÓÔ‡˜ Ì ¡π∞. 4. ∆ËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ·ÚıÚÒÛÂˆÓ Ì ÂÓÂÚÁfi ·ÚıÚ›Ùȉ·. ø˜ ¿ÚıÚˆÛË Ì ÂÓÂÚÁfi ·ÚıÚ›Ùȉ· ÔÚ›˙ÂÙ·È Î¿ı ¿ÚıÚˆÛË Ì ‰ÈfiÁΈÛË Ô˘ ‰ÂÓ ·Ô‰›‰ÂÙ·È Û ÔÛÙÈ΋ ·‡ÍËÛË ‹, Û ·Ô˘Û›· ‰ÈfiÁΈÛ˘, οı ¿ÚıÚˆÛË Ì ÂÚÈÔÚÈṲ̂ÓË ÎÈÓËÙÈÎfiÙËÙ· Ô˘ Û˘Óԉ‡ÂÙ·È Â›Ù ·fi fiÓÔ ÛÙËÓ Î›ÓËÛË Â›Ù ·fi ¢·ÈÛıËÛ›· ÛÙËÓ ÂͤٷÛË. 5. ∆ËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ·ÚıÚÒÛÂˆÓ Ì ÂÚÈÔÚÈÛÌfi Ù˘ ÎÈÓËÙÈÎfiÙËÙ·˜. 6. ∆ËÓ ÂÎÙ›ÌËÛË Ù˘ ∆∫∂ ‹/Î·È Ù˘ CRP (11). ø˜ ‚ÂÏÙ›ˆÛË Ù˘ ÔÚ›·˜ Ù˘ ÓfiÛÔ˘ ÔÚ›˙ÂÙ·È Ë ‚ÂÏÙ›ˆÛË ≥30% Û ≥3/6 ·Ú·Ì¤ÙÚÔ˘˜, ¯ˆÚ›˜ Ù·˘Ùfi¯ÚÔÓË Âȉ›ӈÛË Û >1 ·Ú·Ì¤ÙÚÔ˘˜ ηٿ >30% (14). ø˜ ˘ÔÙÚÔ‹ ÔÚ›˙ÂÙ·È Ë ·ÚÔ˘Û›· ÔÔÈÔ˘‰‹ÔÙÂ Â˘Ú‹Ì·ÙÔ˜ ÂÓÂÚÁfiÙËÙ·˜ ›Ù ˆ˜ ÚÔ˜ ÙËÓ ·ÚıÚ›Ùȉ· ›Ù ˆ˜ ÚÔ˜ ÙȘ Û˘ÛÙËÌ·ÙÈΤ˜ ÂΉËÏÒÛÂȘ (14). ø˜ ‡ÊÂÛË ˘fi ıÂÚ·›· ÔÚ›ÛÙËÎÂ Ë Î·Ù¿ÛÙ·ÛË Ô˘ ÂÎÏËÚÒÓÂÈ Ù· 5 ÎÚÈÙ‹ÚÈ· ‡ÊÂÛ˘, ÂÓÒ Ô ·ÛıÂÓ‹˜ Â›Ó·È ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 6 Û˘Ó¯fiÌÂÓÔ˘˜ Ì‹Ó˜ ˘fi ıÂÚ·›·: 1. ·Ô˘Û›· ÂÓÂÚÁÔ‡ ·ÚıÚ›Ùȉ·˜, 2. ·Ô˘Û›· ˘ÚÂÙÔ‡, ÂÍ·Óı‹Ì·ÙÔ˜, ÔÚÔÁÔÓ›Ùȉ·˜, ÌÂÁ·ÏÔÛÏËÓ›·˜ ‹ ÏÂÌÊ·‰ÂÓ›Ùȉ·˜ Ô˘ ·Ô‰›‰ÂÙ·È ÛÙË NIA, 3. ·Ô˘Û›· ÂÓÂÚÁÔ‡ π∫, 4. ·Ô˘Û›· ·˘ÍË̤Ó˘ ∆∫∂ ‹ Î·È CRP Ô˘ ·Ô‰›‰ÂÙ·È ÛÙË NIA, 5. ÛÊ·ÈÚÈ΋ ÂÎÙ›ÌËÛË Ù˘ ‚·Ú‡ÙËÙ·˜ Ù˘ ηٿÛÙ·Û˘ ÙÔ˘ ·ÛıÂÓÔ‡˜ ·fi ÙÔ ıÂÚ¿ÔÓÙ· È·ÙÚfi, Ô˘ ‰ÂÓ ˘Ô‰ÂÈÎÓ‡ÂÈ ÂÓÂÚÁfiÙËÙ· (max 1 cm) (14).

2. ∂ÎÙ›ÌËÛË Ù˘ ÂÓÂÚÁfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ ÂÚÈÔ‰Èο (1Ô˜, 2Ô˜, 3Ô˜, 6Ô˜ Ì‹Ó·˜ Î·È ·Ó¿ 6 Ì‹Ó˜ ÛÙË Û˘Ó¤¯ÂÈ·), Ì ÙËÓ ÚÔ·Ó·ÊÂÚı›۷ ÌÂıÔ‰ÔÏÔÁ›·.

3. ∂ÎÙ›ÌËÛË Ù˘ ·ÛÊ¿ÏÂÈ·˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜, Ì ‚¿ÛË ÂÚÁ·ÛÙËÚȷο Î·È ÎÏÈÓÈο ‰Â‰Ô̤ӷ: ∂ÎÙfi˜ ·fi ÙËÓ ÂÚÈÔ‰È΋ ÎÏÈÓÈ΋ ÂÎÙ›ÌËÛË, fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ ˘Ô‚¿ÏÏÔÓÙ·Ó Û ÂÚÁ·ÛÙËÚÈ·Îfi, ηډÈÔÏÔÁÈÎfi Î·È ÔÊı·ÏÌÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô Î·È ÂȉÈÎfiÙÂÚ·: ∞. ∞ÈÌ·ÙÔÏÔÁÈÎfi, ‚ÈÔ¯ËÌÈÎfi Î·È ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, ·Ó¿ Ì‹Ó· ÛÙÔ ÚÒÙÔ 6ÌËÓÔ Î·È ·Ó¿ 6ÌËÓÔ ÌÂÙ¤ÂÈÙ·, fiˆ˜ ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÚÔËÁÔ˘Ì¤Óˆ˜.

O ·ÈÌ·ÙÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÂÚÈÏ¿Ì‚·Ó ÙË ÁÂÓÈ΋ ÂͤٷÛË ÙÔ˘ ·›Ì·ÙÔ˜ Î·È ÙËÓ ∆∫∂, Ô ‚ÈÔ¯ËÌÈÎfi˜ ÙÔÓ ¤ÏÂÁ¯Ô Ù˘ ÓÂÊÚÈ΋˜ Î·È Ë·ÙÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (̤ÙÚËÛË ÛÙÔ Ï¿ÛÌ· Ù˘ Ô˘Ú›·˜, ÎÚ·ÙÈÓ›Ó˘, SGPT/SGOT, Á-GT Î·È ÁÂÓÈ΋ ÂͤٷÛË Ô‡ÚˆÓ), ηıÒ˜ Î·È ÙË Ì¤ÙÚËÛË Ù˘ ÁÏ˘Îfi˙˘ Ï¿ÛÌ·ÙÔ˜. √ ·ÓÔÛÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÂÚÈÏ¿Ì‚·Ó ÙË Ì¤ÙÚËÛË Ì ÙȘ ηıÈÂڈ̤Ó˜ Ù¯ÓÈΤ˜ ÙˆÓ ÂÈ¤‰ˆÓ ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ (IgA, IgM, IgG IgE) Î·È ÙˆÓ ˘ÔÙ¿ÍÂˆÓ Ù˘ IgG, ÙˆÓ ÎÏ·ÛÌ¿ÙˆÓ Ùo˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ C3,C4, ÙÔ˘ ÚÂ˘Ì·ÙÔÂȉԇ˜ ·Ú¿ÁÔÓÙ· (RF) Î·È ÙËÓ ·Ó›¯Ó¢ÛË Î·È ÙÔÓ Ù›ÙÏÔ ‰È·ÊfiÚˆÓ ·˘ÙÔ·ÓÙÈۈ̿وÓ: ·ÓÙÈ˘ÚËÓÈο (∞¡∞), ·ÓÙÈ-DNA, ηٿ Âί˘ÏÈÛÌ¿ÙˆÓ ÙÔ˘ ˘Ú‹Ó· (·ÓÙÈ-∂¡∞) Î·È ÂȉÈÎfiÙÂÚ· Ù· ·ÓÙÈ-SSA ‹ Ro, ·ÓÙÈ-SSB ‹ La, ·ÓÙÈ-Sm, ·ÓÙÈ-URNP, ·ÓÙÈ-Jo, ·ÓÙÈ-Scl70, ·ÓÙÈ-Cenp-B,

Pediatric Jan-Feb 09

16-03-09

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33

¶ÂÓÙ·ÂÙ‹˜ ÂÌÂÈÚ›· ·fi ÙË ¯Ú‹ÛË etanercept Î·È adalimumab ÛÙË Ó·ÓÈ΋ ȉÈÔ·ı‹ ·ÚıÚ›Ùȉ·

¶›Ó·Î·˜ 2. ∞ÓÂÈı‡ÌËÙ· Û˘Ì‚¿Ì·Ù· ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ·ÓÙÈ-TNF ıÂÚ·›·˜ (59 ·ÓÙÈ-TNF Û˘Ó‰ڛ˜) ∂›‰Ô˜ ·ÓÂÈı‡ÌËÙ˘ ·ÓÙ›‰Ú·Û˘ ™˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ Ô˘ÚÔÔÈËÙÈÎÔ‡ ™˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ ·Ó·Ó¢ÛÙÈÎÔ‡ ŒÚ˘ ˙ˆÛÙ‹Ú·˜ (·Ú·ÙÂٷ̤ÓË ÔÚ›·) ∂Ú‡ıËÌ· Î·È ÎÓËÛÌfi˜ ÛÙÔ ÛËÌÂ›Ô Ù˘ ¤ÓÂÛ˘ (ÙÔÈ΋ ·ÓÙ›‰Ú·ÛË) ∫Ó›‰ˆÛË, ·ÚıÚ·ÏÁ›Â˜ ÌÂÙ¿ ÙËÓ ¤ÓÂÛË (Û˘ÛÙËÌ·ÙÈ΋ ·ÓÙ›‰Ú·ÛË) ∆ÚȯfiÙˆÛË ∂Ì‡ÚÂÙÔ ÌË Î·ıÔÚÈÛı›۷˜ ·ÈÙÈÔÏÔÁ›·˜ Î·È ı¿Ó·ÙÔ˜ ÂÓÙfi˜ ‚‰ÔÌ¿‰·˜* (·Ô‰fiıËΠ۠Ôχ ÛÔ‚·Ú‹ Ïԛ̈ÍË)

∂∆ (n=37)

AD (n=22)

™‡ÓÔÏÔ (n=59)

1 10 1 0 0 0

0 7 0 1 2 1

1 17 1 1 2 1

0*

1*

1

* 2 Ì‹Ó˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ÙÔ˘ AD Î·È ÌÂÙ¿ ·fi ÚÔËÁËı›۷ ‰ÈÂÙ‹ ıÂÚ·›· Ì AD Î·È 9ÌËÓË ıÂÚ·›· Ì ∂∆ (‚Ϥ Î›ÌÂÓÔ)

·ÓÙÈ-ÚÈ‚Ôۈ̷ÙÈο Î·È ·ÓÙÈ-ÈÛÙfiÓ˜. ∂›Û˘, ·˘ÙÔ·ÓÙÈÛÒÌ·Ù· ηٿ ÙÔ˘ ΢ÙÙ·ÚÔÏ¿ÛÌ·ÙÔ˜ ÙˆÓ Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ (cANCA Î·È p-ANCA) Î·È ·ÓÙÈηډÈÔÏÈȉÈο (Acl). µ. ∫·Ú‰ÈÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Î·È ·Ó¿ 6ÌËÓÔ ÌÂÙ¤ÂÈÙ·. √ ηډÈÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÂÚÈÏ¿Ì‚·Ó ËÏÂÎÙÚÔηډÈÔÁÚ¿ÊËÌ·, ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜ 2 ‰È·ÛÙ¿ÛÂˆÓ Î·È Doppler. °. √Êı·ÏÌÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô (Û¯ÈÛÌÔÂȉ‹˜ Ï˘¯Ó›·, ‚˘ıÔÛÎfiËÛË) ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Î·È ·Ó¿ 4ÌËÓÔ ÌÂÙ¤ÂÈÙ·. ¢. ∫·Ù·ÁÚ·Ê‹ ·fi ÙÔ˘˜ ÁÔÓ›˜, Û ÚÔۯ‰ȷṲ̂ÓÔ ËÌÂÚÔÏfiÁÈÔ, fiÏˆÓ ÙˆÓ ·ÓÂÈı‡ÌËÙˆÓ ·ÓÙȉڿÛÂˆÓ Ô˘ ›¯·Ó Û¯¤ÛË Ì ÙË ¯ÔÚ‹ÁËÛË ÙÔ˘ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· (ÙÔÈΤ˜ Î·È Û˘ÛÙËÌ·ÙÈΤ˜ ·ÓÙȉڿÛÂȘ), ηıÒ˜ Î·È Î·Ù·ÁÚ·Ê‹ οı ÌÂÙ·‚ÔÏ‹˜ Ù˘ ÁÂÓÈ΋˜ ηٿÛÙ·Û˘ ÙÔ˘ ·È‰ÈÔ‡ ÙÔ˘˜ Î·È Î¿ıÂ Â˘Ú‹Ì·ÙÔ˜ ÂÓ‰ÂÈÎÙÈÎÔ‡ Âȉ›ӈÛ˘ Ù˘ ˘ÔΛÌÂÓ˘ ÓfiÛÔ˘ ‹ Ó¤·˜ ÓfiÛÔ˘ (.¯. Ïԛ̈͢ Ô˘ Û˘Ó¤‚Ë ÛÙÔ ÌÂÛԉȿÛÙËÌ· ÌÂٷ͇ ‰‡Ô ÂÈÛΤ„ˆÓ). ™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË: ∫·Ù·ÁÚ¿ÊËÎ·Ó fiϘ ÔÈ ·Ú¿ÌÂÙÚÔÈ Ô˘ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙ· ÎÚÈÙ‹ÚÈ· ÙÔ˘ ACRped, ÛÙËÓ ¤Ó·ÚÍË ıÂÚ·›·˜ Î·È ÛÙËÓ ÔÚ›· Û ÂÙ‹ÛÈ· ‚¿ÛË. ø˜ ÈηÓÔÔÈËÙÈ΋ ·¿ÓÙËÛË ÔÚ›ÛÙËÎÂ Ë ACRped 50, ¿ÚÈÛÙË Ë ACRped 70 ÂÓÒ ÔÚȷ΋ Ë ACRped 30 Î·È Ùˆ¯‹ Ë ACRped<30. ∏ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ¤ÁÈÓ ۇÌʈӷ Ì ÙËÓ ÚÔÙÂÈÓfiÌÂÓË ·fi ÙÔ˘˜ Lovell Î·È Û˘Ó. Î·È Lahdenne Î·È Û˘Ó. ÌÂıÔ‰ÔÏÔÁ›· (12,15). ÀÔÏÔÁ›ÛÙËÎÂ Ë Ì¤ÛË ÙÈÌ‹ (mean) Ù˘ οı ·Ú·Ì¤ÙÚÔ˘, Ë ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË (SD) Î·È Ë ‰È¿ÌÂÛË ÙÈÌ‹ (median) ÛÙË ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ Ù˘ ÂÎÙ›ÌËÛ˘, Ì ÏÔÁÈÛÌÈÎfi ÚfiÁÚ·ÌÌ· (STATISTICA) Î·È Ë % ‚ÂÏÙ›ˆÛ‹ Ù˘ Û˘ÁÎÚÈÙÈο Ì ÙËÓ ÙÈÌ‹ ÛÙËÓ ¤Ó·ÚÍË Ù˘ ÌÂϤÙ˘.

∞ÔÙÂϤÛÌ·Ù· ∞fi ÙÔ˘˜ 46 ·ÛıÂÓ›˜, 8 ˘fi ∂∆ Û˘ÌÏ‹ÚˆÛ·Ó 5 ¯ÚfiÓÈ· ıÂÚ·›·˜ Î·È ÔÈ 2 ˘fi AD ‚Ú›ÛÎÔÓÙ·È ÛÙÔÓ 5Ô ¯ÚfiÓÔ ıÂÚ·›·˜, ÂÓÒ 50% ·fi ÙËÓ Î¿ı ÔÌ¿‰· (¡=18 Î·È ¡=10 ·ÓÙ›ÛÙÔȯ·) Û˘ÌÏ‹ÚˆÛ·Ó ‹‰Ë 3 ¯ÚfiÓÈ· ıÂÚ·›·˜. ∞ÛÊ¿ÏÂÈ· ∫·È ÔÈ 2 ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ˜ ·ԉ›¯ıËÎ·Ó ·ÛÊ·Ï›˜ ÁÈ· ÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ (¶›Ó·Î·˜ 2). ã∏Ș ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ: ™Â 17/46 ·ÛıÂ-

Ó›˜ ÛËÌÂÈÒıËÎ·Ó Û˘¯Ó¤˜, ÎÔÈÓ¤˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ (17) ‹/Î·È ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ (1). ◊Ș, ÙÔÈΤ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ ÛÙÔ ÛËÌÂ›Ô Ù˘ ¤ÓÂÛ˘ ÛËÌÂÈÒıËÎ·Ó Û ¤Ó·Ó ·ÛıÂÓ‹ (AD), Û˘ÛÙËÌ·ÙÈΤ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ Û 2 (AD) Î·È ÙÚȯfiÙˆÛË Û ¤Ó·Ó ¿ÏÏÔ (AD). ™Ô‚·Ú¤˜ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ: ŒÓ·˜ ·ÛıÂÓ‹˜ (∂∆) ·ÚÔ˘Û›·Û ¤ÚËÙ· ˙ˆÛÙ‹Ú· Ì ·Ú·ÙÂٷ̤ÓË ÔÚ›· Î·È ¯ÚÂÈ¿ÛıËΠ·ÁˆÁ‹ Ì ·Î˘ÎÏÔ‚›ÚË. ∆¤ÏÔ˜, Ì›· ¿ÏÏË ·ÛıÂÓ‹˜ η٤ÏËÍÂ, 2 Ì‹Ó˜ ÌÂÙ¿ ÙË ¯ÔÚ‹ÁËÛË ÙÔ˘ AD Î·È ·ÊÔ‡ ›¯Â ÚÔËÁËı› ‰ÈÂÙ‹˜ ıÂÚ·›· ¿ÏÈ Ì AD Î·È ÛÙË Û˘Ó¤¯ÂÈ· 9ÌËÓË ıÂÚ·›· Ì ∂∆. ∏ ·ÛıÂÓ‹˜ ·˘Ù‹ ·ÚÔ˘Û›·Û ÂÌ‡ÚÂÙÔ ÌË Î·ıÔÚÈÛı›Û˘ ·ÈÙÈÔÏÔÁ›·˜ Î·È Î·Ù¤ÏËÍ ¤Ó· 24ˆÚÔ ÌÂÙ¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ Ù˘ Û ÓÔÛÔÎÔÌÂ›Ô Ù˘ ÂÚÈʤÚÂÈ·˜, Ì Èı·Ó‹ ‰È¿ÁÓˆÛË «Ïԛ̈ÍË ÙÔ˘ ∫¡™». ∏ ·ÛıÂÓ‹˜ ‹Ù·Ó ¯·ÌËÏÔ‡ ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎÔ‡ ÂÈ¤‰Ô˘, ›¯Â Ù·Íȉ¤„ÂÈ ÚÔËÁÔ˘Ì¤Óˆ˜ Û ¿ÏÏË, ·Ó·Ù˘ÛÛfiÌÂÓË ¯ÒÚ·, fiÔ˘ Î·È ÍÂΛÓËÛ·Ó Ù· Û˘ÌÙÒÌ·Ù· (˘ÚÂÙfi˜, ÎÂÊ·Ï·ÏÁ›·, ·ÓÔÚÂÍ›· Î·È ÓˆıÚfiÙËÙ·), Ì ·ÔÙ¤ÏÂÛÌ· Ó· ÌÂÛÔÏ·‚‹ÛÂÈ ÛËÌ·ÓÙÈ΋ ηı˘ÛÙ¤ÚËÛË ÚÔÛ¤Ï¢Û˘ Û ÂÏÏËÓÈÎfi ÓÔÛÔÎÔÌ›Ô, ÛÙÔ ÔÔ›Ô Î·È ¤ÁÈÓ ÂÈÛ·ÁˆÁ‹ Û ÙÂÏÈÎfi ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ Ì ·ÓÔ˘Ú›·. ¢˘ÛÙ˘¯Ò˜, ‰ÂÓ ¤ÁÈÓ ÓÂÎÚÔÙÔÌ‹. ∆· ·ÔÙÂϤÛÌ·Ù· ˆ˜ ÚÔ˜ ÙËÓ ÂÌÊ¿ÓÈÛË Ó¤ˆÓ ·˘ÙÔ·ÓÙÈÛˆÌ¿ÙˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ ·ÔÙ¤ÏÂÛ·Ó ·ÓÙÈΛÌÂÓÔ ÚÔËÁÔ‡ÌÂÓ˘ ÌÂϤÙ˘ (16). ∂ÎÙ›ÌËÛË Ù˘ Â›‰Ú·Û˘ Ù˘ ·ÓÙÈ-TNF ıÂÚ·›·˜ ÛÙËÓ ÔÚ›· Ù˘ ÈÚȉÔ΢ÎÏ›Ùȉ·˜: ¢‡Ô ·ÛıÂÓ›˜, ·ÚÔ˘Û›·Û·Ó Â›ÌÔÓË π∫ ÁÈ· ÚÒÙË ÊÔÚ¿ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ NIA, 10 Î·È 15 ¯ÚfiÓÈ· ·ÚÁfiÙÂÚ· Î·È ÂÓÒ ‹Ù·Ó Û ·ÓÙÈ-TNF ıÂÚ·›· Ì ∂∆. ∏ π∫ ‹Ù·Ó ·ÓıÂÎÙÈ΋ ÙfiÛÔ ÛÙËÓ ÙÔÈ΋ ıÂÚ·›· fiÛÔ Î·È ÛÙËÓ ·ÓÙÈTNF, Ì ·ÔÙ¤ÏÂÛÌ· ÙË ‰È·ÎÔ‹ ÙÔ˘ ∂∆ Î·È ÙË ¯ÔÚ‹ÁËÛË AD. ∏ π∫ ˘Ô¯ÒÚËÛÂ Î·È ‰ÂÓ ˘ÔÙÚÔ›·Û Û fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ Ì AD. ™Â ¿ÏÏÔ˘˜ 4 ·ÛıÂÓ›˜ Ì ¡π∞ Î·È ÈÛÙÔÚÈÎfi ˘ÔÙÚÔÈ¿˙Ô˘Û·˜ π∫, ÙÔ ∂∆ ‰ÂÓ Ê¿ÓËΠӷ ÂËÚ¤·Û ÙËÓ ÂÓÂÚÁfiÙËÙ· ¶·È‰È·ÙÚÈ΋ 2009;72:29-40

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·34

34

¶ÔÛÔÛÙfi ·ÛıÂÓÒÓ

-6,5±2,1 -85,7±20,2 -6,5 -85,7 -66,6±40,5 -82,1

N=36

N=32

N=18

N=10

N=8

80% ACR <30 ACR 30 ACR 50 ACR 70

60% 40% 20% 0%

1

2 3 4 ÃÚfiÓÈ· ıÂÚ·›·˜

5

∂ÈÎfiÓ· 1. ∂Ù‹ÛÈ· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙÔ˘ Etanercept Û‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· ACRped.

Ù˘ π∫. ™Â 2/4 Ë π∫ ÂϤÁ¯ıËΠÌÂÙ¿ ·fi Ì·ÎÚÔ¯ÚfiÓÈ· ÙÔÈ΋ ıÂÚ·›· Î·È ÛÙÔ˘˜ ¿ÏÏÔ˘˜ 2 Ì ÙËÓ ·ÏÏ·Á‹ Ù˘ ıÂÚ·›·˜ ·fi ∂∆ Û AD (¶›Ó·Î·˜ 1). ∂ÈϤÔÓ, Û 1 ·ÛıÂÓ‹ Ì ¡π∞ Î·È ÈÛÙÔÚÈÎfi ˘ÔÙÚÔÈ¿˙Ô˘Û·˜ π∫, Ë ¯ÔÚ‹ÁËÛË ÙÔ˘ AD ›¯Â ·Ú¯Èο ÁÈ· 2 ¯ÚfiÓÈ· ¢ÓÔ˚΋ Â›‰Ú·ÛË ÛÙËÓ ÔÚ›· Ù˘ π∫, ·ÏÏ¿ ÛÙË Û˘Ó¤¯ÂÈ· Ë π∫ ˘ÔÙÚÔ›·ÛÂ.

* ·: ª¤ÛË ÌÂÙ·‚ÔÏ‹ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, ‚: % ÌÂÙ·‚ÔÏ‹ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜

-71,4±39,3 -94,3 -4±2,9 -5 -66,8±45,7 -89,4 -72,3±43,4 -92,8

-3,3±2,7 -3,8 -3,1±2,6 -3 4±2,6 3,6

Paediatriki 2009;72:29-40

100%

-3,8±2,7 -4

-0,2±0,3 -61,6±42,8 -0,1 -60 -4±3,2 -75,2±45,6 -3,8 -95 -32,7±77,5 -50 -63,8±33,3 -68,8 -74,3±39,1 -100 -81,3±24,5 -92,9 -0,6±0,6 -0,5 -3,8±2,5 -3,5 -52,1±77,5 -9 -82,2±23,9 -88,9 -0,6±0,7 0,5 -3,5±2,2 -3 0,8±0,7 0,6 4,4±2,3 4

-67,6±49,6 -100 -77,7±34,2 -91,5

-0,7±0,7 -0,5 -4,3±2,4 -4

-0,7±1,1 -0,4 -3,5±2,6 -3,2

-59,2±77,1 -100 -66,2±47,4 -92,9 -49,5±81 -92,8 -46,8±33,6 -55,5 -42,7±72,8 -80,2 -64,5±64 -3,9±2,2 -100 -5 -65,1±62,5 -11±12,4 -91,2 -7 -59±60,2 -6,3±4,3 -79,2 -7 -49,3±44,8 -31,6±24 -60 -35,5 -80,2±24,8 -45,8±31,6 -92,8 -58 -77,2±37,6 -100 -84,2±29 -100 -80,5±29,8 -100 -49,5±61,4 -72,7 -76,5±43,9 -91,6 -4,8±8,5 -3 -9,1±10,7 -5 -7,4±5,1 -5,5 -33,7±29,2 -31 -44,9±43,3 -40 6±9 -4,8±7,5 -72,3±40 4 -3 -100 10,5±12,3 -8,5±10,3 -75,7±35 6 -5 -100 9,7±9 -7,4±6,05 -80,2±25,7 6 -5 -93,8 46,2±26,3 -24,8±25,3 -42,2±58 44 -25 -62,2 42,7±42,3 -34,6±43,8 -65,8±52,9 40 -26 -95

∞Ú. ‰ÈÔÁÎˆÌ¤ÓˆÓ ·ÚıÚÒÛÂˆÓ (x± SD, median) ∞Ú. ÂÒ‰˘ÓˆÓ ·ÚıÚÒÛÂˆÓ (x±SD, median) ∞Ú. ·ÚıÚÒÛÂˆÓ Ì ÂÓÂÚÁfi ÓfiÛÔ (x±SD, median) ∆∫∂ (x± SD, median) CRP (x± SD, median) ¢Â›ÎÙ˘ ªÂÈÔÓÂÎÙÈÎfiÙËÙ·˜ (CHAQ) (0-3) (x±SD, median) ™Ê·ÈÚ. ∂ÎÙ›ÌËÛË π·ÙÚÔ‡ (0-10) (x±SD, median) ™Ê·ÈÚ. ∂ÎÙ›ÌËÛË ∂˘ÂÍ›·˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ (0-10) (x±SD, median)

·

-60±52,3 -92,9 -76,4±36 -97,2 -80,1±30,2 -96,4 -45,5±40,8 -70,4 -59,1±95,1 -94,6

-5±5,6 -4 -9,5±9,5 -7 -7,8±5 -6,5 -41,9±30,1 -40 -49,7±30,2 -51

-6,4±8,8 -5 -11,8±11 -7,5 -6,9±4,5 -5,5 -32,7±24,8 -35,5 -57,3±48,4 -50

‚ (%) 4 (N=10) · ‚ (%) ·

ÃÚfiÓÔ˜ 3 (N=18) 2 (N=32) ‚ (%) 1 (N=37) ·* ‚ (%) ŒÓ·ÚÍË ıÂÚ·›·˜

¶›Ó·Î·˜ 3. ∂Ù‹ÛÈ· ÂÎÙ›ÌËÛË Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ ÙÔ˘ Etanercept, Ì ‚¿ÛË Ù· ÎÚÈÙ‹ÚÈ· ACRped ÛÙÔ˘˜ 36 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘. ™‡ÓÔÏÔ ıÂÚ·¢ÙÈÎÒÓ Û˘Ó‰ÚÈÒÓ: 37

·

5 (N=8) ‚ (%)

ª. ∆Ú·¯·Ó¿ Î·È Û˘Ó.

∞ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ™ÙÔ˘˜ ¶›Ó·Î˜ 3 Î·È 4 ·ÚÔ˘ÛÈ¿˙ÂÙ·È Ë ÌÂÙ·‚ÔÏ‹ ÛÙȘ ÂÈ̤ÚÔ˘˜ ·Ú·Ì¤ÙÚÔ˘˜ Ô˘ ÌÂÏÂÙ‹ıËÎ·Ó ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ‚ÂÏÙ›ˆÛ˘ Ù˘ ÓfiÛÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ 59 ıÂÚ·¢ÙÈÎÒÓ Û˘Ó‰ÚÈÒÓ Ì ٷ ‰‡Ô Û΢¿ÛÌ·Ù·, ∂∆ (¶›Ó·Î·˜ 3) Î·È AD (¶›Ó·Î·˜ 4), ÂÓÒ ÛÙÔÓ ¶›Ó·Î· 5 Î·È ÛÙȘ ∂ÈÎfiÓ˜ 1 Î·È 2 ηٷÁÚ¿ÊÂÙ·È Ë ·Ó¿ ¤ÙÔ˜ ÂÎÙ›ÌËÛË Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ ÙˆÓ ‰‡Ô ·˘ÙÒÓ Û΢·ÛÌ¿ÙˆÓ, Ì ٷ ACRped ÎÚÈÙ‹ÚÈ·, ÛÙÔ˘˜ 46 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘. ∞fi ÙËÓ ·Ó¿Ï˘ÛË ÙÔ˘ ¶›Ó·Î· 5 ÚÔ·ÙÔ˘Ó Ù· ÂÍ‹˜: √Ì¿‰· ∂∆: ™ÙÔÓ 1Ô ¯ÚfiÓÔ ıÂÚ·›·˜ ÙˆÓ ·ÛıÂÓÒÓ, 88,88% ¤Ù˘¯·Ó ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÓfiÛÔ˘ (ACRped 50-70) Î·È Î·Ó¤Ó·˜ ‰ÂÓ ˘ÔÙÚÔ›·ÛÂ. ∆ÔÓ 2Ô ¯ÚfiÓÔ ıÂÚ·›·˜, 81,25% ÙˆÓ ·ÛıÂÓÒÓ ¤Ù˘¯·Ó ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË (ACRped 50-70), 7 ·ÛıÂÓ›˜ ‰È¤ÎÔ„·Ó ÙË ıÂÚ·›·, Ô ¤Ó·˜ ÏfiÁˆ ˘ÔÙÚÔ‹˜, ¤Ó·˜ ¿ÏÏÔ˜ ÏfiÁˆ ·Ó¿Ù˘Í˘ π∫, (15 ¯ÚfiÓÈ· ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ NIA) Î·È ¿ÏÏÔÈ 5 ÏfiÁˆ Ï‹ÚÔ˘˜ ‡ÊÂÛ˘ Ù˘ ÓfiÛÔ˘. ∆ÔÓ 3Ô ¯ÚfiÓÔ ıÂÚ·›·˜, 83,33% ÙˆÓ ·ÛıÂÓÒÓ ¤Ù˘¯·Ó ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË (ACRped 50-70). ∆ÚÂȘ ·ÛıÂÓ›˜ ‰È¤ÎÔ„·Ó ÏfiÁˆ Ï‹ÚÔ˘˜ ‡ÊÂÛ˘ Ù˘ ÓfiÛÔ˘ Î·È Î·Ó¤Ó·˜ ‰ÂÓ ·ÚÔ˘Û›·Û ˘ÔÙÚÔ‹. ∆ÔÓ 4Ô ¯ÚfiÓÔ ıÂÚ·›·˜, 80% ÙˆÓ ·ÛıÂÓÒÓ ¤Ù˘¯·Ó ACRped 50-70, 2 ·ÛıÂÓ›˜ ‰È¤ÎÔ„·Ó ÏfiÁˆ ‡ÊÂÛ˘ Ù˘ ÓfiÛÔ˘ Î·È Î·Ó¤Ó·˜ ‰ÂÓ ˘ÔÙÚÔ›·ÛÂ. ∆¤ÏÔ˜, ÙÔÓ 5Ô ¯ÚfiÓÔ ıÂÚ·›·˜, ACRped 50-70 ¤Ù˘¯·Ó 75% ÙˆÓ ·ÛıÂÓÒÓ Î·È 2 ·ÛıÂÓ›˜ ‰È¤ÎÔ„·Ó ÙË ıÂÚ·›· ›Ù ÏfiÁˆ ‡ÊÂÛ˘ (1) ›Ù ÏfiÁˆ ÂÌÊ¿ÓÈÛ˘ π∫ (1), ÂÓÒ Ô ›‰ÈÔ˜ ¤Ù˘¯Â ACRped 70 ˆ˜ ÚÔ˜ ÙËÓ ·ÚıÚ›Ùȉ·.

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35

-93,2±15 -100 -92,8±16 -100 -89,3±14,4 -100 -40,6±58,7 -64,7 75,6±548,1 -98,7 -73,1±39,8 -92,5 -87,3±15,5 -88,1 -87,3±15,5 -93,9 * ·: ª¤ÛË ÌÂÙ·‚ÔÏ‹ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, ‚: % ÌÂÙ·‚ÔÏ‹ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ ** √È ·ÛıÂÓ›˜ Ô˘ ‚Ú›ÛÎÔÓÙ·È ÛÙÔÓ 5Ô ¯ÚfiÓÔ ıÂÚ·›·˜ ·ÏÏ¿ ‰ÂÓ ÙÔÓ Û˘ÌÏ‹ÚˆÛ·Ó ·ÎfiÌË ‰ÂÓ ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ›Ó·Î·

-3,7±0,6 -4 -9,1±9,1 -6,5 -9±9,7 -5 -23,9±25,5 -22 -49,9±55,8 -38,5 -0,5±0,4 -0,5 -3,2±1,6 -2,2 -3,2±1,5 -3

√Ì¿‰· AD: ¶·ÚfiÌÔÈ· ‹Ù·Ó Î·È Ù· ·ÔÙÂϤÛÌ·Ù· ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ù˘ ÔÌ¿‰·˜ ÙÔ˘ AD. ∆ÔÓ 1Ô ¯ÚfiÓÔ ıÂÚ·›·˜, ACRped 50-70 ¤Ù˘¯·Ó 71,42% ÙˆÓ ·ÛıÂÓÒÓ, ÂÓÒ 3 ·ÛıÂÓ›˜ ‰È¤ÎÔ„·Ó ÙË ıÂÚ·›· ÏfiÁˆ ˘ÔÙÚÔ‹˜ Ù˘ ÓfiÛÔ˘. ∫·È ÔÈ 3 ›¯·Ó ÔÏ˘·ÚıÚ›Ùȉ· (1/3 RF ıÂÙÈÎfi˜) Î·È ÔÈ 2/3 ›¯·Ó ‰ÔÎÈÌ¿ÛÂÈ ÚÔËÁÔ˘Ì¤Óˆ˜ ¿ÏÏÔ˘˜ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ˜ Â› 2 ¯ÚfiÓÈ·, INFL (1) Î·È ∂∆ (1). ∆ÔÓ 2Ô ¯ÚfiÓÔ ıÂÚ·›·˜, ACRped 50-70 ¤Ù˘¯·Ó 66,7% ÙˆÓ ·ÛıÂÓÒÓ Î·È 3 ‰È¤ÎÔ„·Ó ÏfiÁˆ ˘ÔÙÚÔ‹˜ Ù˘ ÓfiÛÔ˘. ∫·È ÔÈ 3 ›¯·Ó ÔÏ˘·ÚıÚ›Ùȉ· (3/3 RF ·ÚÓËÙÈÎfi˜) Î·È ÔÈ 2/3 ›¯·Ó ‰ÔÎÈÌ¿ÛÂÈ ÚÔËÁÔ˘Ì¤Óˆ˜ ¿ÏÏÔ˘˜ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ˜ Â› 2 ¯ÚfiÓÈ·, INFL (1) Î·È ∂∆ (1). ™ÙÔÓ 3Ô ¯ÚfiÓÔ ıÂÚ·›·˜, fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ (100%) ¤Ù˘¯·Ó ACRped 50-70 Î·È 1 ‰È¤ÎÔ„Â ÙË ıÂÚ·›· ÏfiÁˆ ‡ÊÂÛ˘ Ù˘ ÓfiÛÔ˘. √ ·ÛıÂÓ‹˜ ·˘Ùfi˜ ›¯Â ·Ú¯›ÛÂÈ Ì ∂∆ Î·È ÙÔ Â›¯Â ‰È·Îfi„ÂÈ ÏfiÁˆ Ï‹ÚÔ˘˜ ‡ÊÂÛ˘. ªÂÙ¿ 1 ¯ÚfiÓÔ ÂÎÙfi˜ ıÂÚ·›·˜, ˘ÔÙÚÔ›·ÛÂ, ‰ÂÓ ·¿ÓÙËÛ ÛÙË Û˘Ì‚·ÙÈ΋ ıÂÚ·›· Î·È ÌÂÙ·ÎÈÓ‹ıËΠÛÙËÓ ÔÌ¿‰· ÙÔ˘ AD. ∆¤ÏÔ˜, ÛÙÔÓ 4Ô ¯ÚfiÓÔ ıÂÚ·›·˜, 87,5% ÙˆÓ ·ÛıÂÓÒÓ ¤Ù˘¯·Ó ACRped 50-70 Î·È ÔÈ 3 ‰È¤ÎÔ„·Ó ÏfiÁˆ ‡ÊÂÛ˘ Ù˘ ÓfiÛÔ˘ (2) ‹ ˘ÔÙÚÔ‹˜ (1). √ ÙÂÏÂ˘Ù·›Ô˜ ›¯Â ˘ÔÙÚÔÈ¿ÛÂÈ Î·È fiÙ·Ó ‹Ù·Ó ·Ú¯Èο ÛÙËÓ ÔÌ¿‰· ÙÔ˘ ∂∆. ¢‡Ô ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ Û˘Ó¯›˙Ô˘Ó ÙË ıÂÚ·›· ÙÔ˘˜ ÁÈ· 5Ô ¯ÚfiÓÔ (‰ÂÓ ÙÔÓ Û˘ÌÏ‹ÚˆÛ·Ó) Î·È ‰È·ÙËÚÔ‡Ó ÙËÓ Î·Ï‹ ·¿ÓÙËÛË Ô˘ ›¯·Ó ̤¯ÚÈ Û‹ÌÂÚ·. ™ÙÔ Û‡ÓÔÏÔ ÙˆÓ ·ÛıÂÓÒÓ, ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ 3ÂÙ›·˜, ÂÚÈÛÛfiÙÂÚÔÈ ·fi ÙÔ˘˜ ÌÈÛÔ‡˜ ·ÛıÂÓ›˜ (5562%) ¤Ù˘¯·Ó ¿ÚÈÛÙË ·¿ÓÙËÛË (ACRped 70) ›Ù Ì ÙÔ ¤Ó· ›Ù Ì ÙÔ ¿ÏÏÔ Û··ÛÌ·. £ÂÚ·›· Ì 3 Û˘Ó‰ڛ˜ ·ÓÙÈ-TNF: ¢‡Ô ·ÛıÂÓ›˜ ¯ÚËÛÈÌÔÔ›ËÛ·Ó ÙÔÓ ›‰ÈÔ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· ‰‡Ô ÊÔÚ¤˜, Û ‰È·ÊÔÚÂÙÈο ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù·, ÌÂÙ¿ ·fi ·ÔÙ˘¯›· ÛÙÔÓ ¿ÏÏÔ, Ô ¤Ó·˜ ÙÔ ∂∆ Î·È Ô ¿ÏÏÔ˜ ÙÔ AD. √ ÚÒÙÔ˜ ·ÛıÂÓ‹˜ ¯ÚËÛÈÌÔÔ›ËÛ 2 ÊÔÚ¤˜ ÙÔ ∂∆. ∆Ô 2Ô fï˜ ¯ÚfiÓÔ ıÂÚ·›·˜, ÂÌÊ¿ÓÈÛ ÁÈ· ÚÒÙË ÊÔÚ¿ π∫, ÛÙÔÓ 10Ô ¯ÚfiÓÔ Ù˘ ÓfiÛÔ˘, Ë ÔÔ›· Â¤ÌÂÓ ·Ú¿ ÙË Û˘Ó¯‹ ÙÔÈ΋ ·ÁˆÁ‹ Ì ÛÙÂÚÔÂȉ‹

-40,5±80,1 -75 -58,9±42,4 -69,2 -64,5±38,4 -77,1 3,65±124,8 -43,3 37,9±299,7 -70,8 -64,4±47,1 -100 -5±202,5 -66,6 -5±202,4 -66,6

∂ÈÎfiÓ· 2. ∂Ù‹ÛÈ· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙÔ˘ Adalimumab Û‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· ACRped.

-1,6±3,2 -3 -5,3±6,8 -4 -5,4±5,7 -4 -10,8±36,1 -10,9 -16,3±76,7 -21,9 -0,3±0,4 -0,2 -2±3 -3 -2±2,9 -3

4

-61,3±96,9 -100 -63,6±57,2 -83,3 -80,1±26,5 94 -41±47,6 -51,6 -11,6±195,3 -83,5 -58,1±54,9 -85,7 -71,5±39,4 -93,3 -71,5±39,4 -93,3

2 3 ÃÚfiÓÈ· ıÂÚ·›·˜

-3,3±5 -4 -5,7±8 -4 -5,2±6 -4 -19,8±24,5 -17 -25,7±42,8 -24 -0,4±0,5 -0,2 -2,5±1,9 -2,7 -2,4±1,8 -2,7

1

3 (N=10) ‚ (%)

0%

·

20%

ÃÚfiÓÔ˜

40%

·

ACR <30 ACR 30 ACR 50 ACR 70

60%

4±4 4 8,7±8,2 6 9,3±8,3 6 42,9±21,6 42,5 39±41,6 25 0,5±5,5 0,5 3,9±1,8 4 3,9±1,8 4

80%

∞Ú. ‰ÈÔÁÎˆÌ¤ÓˆÓ ·ÚıÚÒÛÂˆÓ (x±SD, median) ∞Ú. ÂÒ‰˘ÓˆÓ ·ÚıÚÒÛÂˆÓ (x±SD, median) ∞Ú. ·ÚıÚÒÛÂˆÓ Ì ÂÓÂÚÁfi ÓfiÛÔ (x±SD, median) ∆∫∂ (x±SD, median) CRP (x±SD, median) ¢Â›ÎÙ˘ ªÂÈÔÓÂÎÙÈÎfiÙËÙ·˜ (CHAQ) (0-3) (x±SD, median) ™Ê·ÈÚ. ∂ÎÙ›ÌËÛË π·ÙÚÔ‡ (0-10) (x±SD, median) ™Ê·ÈÚ. ∂ÎÙ›ÌËÛË ∂˘ÂÍ›·˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ (0-10) (x±SD, median)

4 (N=8)** ‚ (%)

N=8

2 (N=15) · ‚ (%)

N=10

1 (N=22) ·* ‚ (%)

N=15

ŒÓ·ÚÍË ıÂÚ·›·˜

N=21

¶›Ó·Î·˜ 4. ∂Ù‹ÛÈ· ÂÎÙ›ÌËÛË Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ ÙÔ˘ Adalimumab, Ì ‚¿ÛË Ù· ηıÈÂڈ̤ӷ ÎÚÈÙ‹ÚÈ· ÛÙÔ˘˜ 21 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘. ™‡ÓÔÏÔ ıÂÚ·¢ÙÈÎÒÓ Û˘Ó‰ÚÈÒÓ: 22

¶ÔÛÔÛÙfi ·ÛıÂÓÒÓ

100%

-2,4±3,7 -51,5±86 -4 -100 -7,6±10,8 -70,5±48,2 -4 -95,8 -7,3±12 -60,4±63,7 -4 -95,8 -4,4±16,9 -3,6±56,3 2 0 -27±23,7 -71,3±55,1 -30 -86,5 -0,3±0,5 -61,6±42,7 -0,3 -61,6 -2,5±2,4 -24,4±144,3 -4,5 -25 -2,5±2,4 -24,3±144,2 -3 -75

¶ÂÓÙ·ÂÙ‹˜ ÂÌÂÈÚ›· ·fi ÙË ¯Ú‹ÛË etanercept Î·È adalimumab ÛÙË Ó·ÓÈ΋ ȉÈÔ·ı‹ ·ÚıÚ›Ùȉ·

¶·È‰È·ÙÚÈ΋ 2009;72:29-40

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ª. ∆Ú·¯·Ó¿ Î·È Û˘Ó.

¶›Ó·Î·˜ 5. ¢È·¯ÚÔÓÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ 46 ·ÛıÂÓÒÓ ˘fi ·ÓÙÈ-TNF ıÂÚ·›· (Etanercept N=32, Adalimumab N=14)

ŒÓ·ÚÍË ıÂÚ·›·˜ (·Ú. ·ÛıÂÓÒÓ) £ÂÚ·¢ÙÈΤ˜ Û˘Ó‰ڛ˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ 1Ô˜ ¯ÚfiÓÔ˜ ACRped 50-70 ACRped 30 ACRped<30 2Ô˜ ¯ÚfiÓÔ˜ ¢ÂÓ Û˘ÌÏ. 2 ¯ÚfiÓÈ·* ACRped 50-70 ACRped 30 ACRped<30 IK-‰È·ÎÔ‹ ⁄ÊÂÛË-‰È·ÎÔ‹ 3Ô˜ ¯ÚfiÓÔ˜ ¢ÂÓ Û˘ÌÏ. 3 ¯ÚfiÓÈ·* ACRped 50-70 ACRped 30 ⁄ÊÂÛË-‰È·ÎÔ‹ 4Ô˜ ¯ÚfiÓÔ˜ ¢ÂÓ Û˘ÌÏ. 4 ¯ÚfiÓÈ·* ACRped 50-70 ACRped 30 ACRped <30 ⁄ÊÂÛË-‰È·ÎÔ‹ 5Ô˜ ¯ÚfiÓÔ˜ ¢ÂÓ Û˘ÌÏ. 5 ¯ÚfiÓÈ·* ACRped 50-70 ACRped 30 ACRped<30 ⁄ÊÂÛË-‰È·ÎÔ‹ IK-‰È·ÎÔ‹

ªfiÓÔ ∂∆

∂∆ ·fi ·ÏÏ·Á‹ ÔÌ¿‰·˜

™‡ÓÔÏÔ ∂∆

ªfiÓÔ AD

AD ·fi ·ÏÏ·Á‹ ÔÌ¿‰·˜

™‡ÓÔÏÔ AD

32

4

36

14

7

21

32 32 28 4 0 29

5 4 4 0 0 3

37 36 32 4 0 32

14 14 11 1 2 10

24 4 1 0 5 18 4* 15 3 3 10 5* 8 0 2 2 8

2 0 0 1 0 0 3*

26 4 1 1 5 18 7* 15 3 3 10 5* 8 0 2 2 8

7 1 2 0 0 7 1* 7 0 0 6

8 7 4 2 1 5 1* 3 1 1 0 0 3 1* 3 0 1 2 1* 1 0 1 0

22 21 15 3 3¨ 15 3* 10 2 3¨ 0 0 10 2* 10 0 1 8 1* 7 0 1 2 2 2*

6 2 0 2 1

6 0 0 2

6 2 0 2 1

ET: Etanercept, AD: Adalimumab * ¢ÂÓ Û˘ÌÏ‹ÚˆÛ·Ó ÙÔÓ ÚÔ‚ÏÂfiÌÂÓÔ ¯ÚfiÓÔ ·Ú·ÎÔÏÔ‡ıËÛ˘, ·ÏÏ¿ Û˘Ó¯›˙Ô˘Ó ÙË ıÂÚ·›· ¨ ∞ÏÏ·Á‹ ·ÓÙÈ-TNF

Î·È Ì˘‰ÚÈ·ÙÈο Ê¿Ú̷η Â› 3 Ì‹Ó˜ Î·È ·Ó·ÁηÛÙÈο ‰È¤ÎÔ„Â ÙËÓ ·ÁˆÁ‹ Ì ∂∆. ∏ ¿ÏÏË ·ÛıÂÓ‹˜ ‹Ù·Ó ·˘Ù‹ Ô˘ η٤ÏËÍ 2 Ì‹Ó˜ ÌÂÙ¿ ÙËÓ ·ÏÏ·Á‹ ·fi ∂∆ Û AD (ÁÈ· 2Ë ÊÔÚ¿), ÏfiÁˆ ÂÌÊ¿ÓÈÛ˘ π∫. ∂›‰Ú·ÛË Ù˘ ·ÓÙÈ-TNF ıÂÚ·›·˜ ÛÙË Û˘Á¯ÔÚ‹ÁËÛË ÛÙÂÚÔÂȉÒÓ: ™ÙȘ 18/25 (72%) ıÂÚ·¢ÙÈΤ˜ Û˘Ó‰ڛ˜, ÔÈ ·ÛıÂÓ›˜ Ô˘ ¿Ú¯ÈÛ·Ó ·ÓÙÈ-TNF ıÂÚ·›· Ì ·Ú¿ÏÏËÏË ¯ÔÚ‹ÁËÛË PDN ‰È¤ÎÔ„·Ó ÛÙ·‰È·Î¿ Ù· ÛÙÂÚÔÂȉ‹ ̤۷ Û 6 Ì‹Ó˜ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÓÙÈ-TNF ıÂÚ·›·˜ Î·È ÛÙȘ ˘fiÏÔÈ˜ 7/25 Û˘Ó¤¯ÈÛ·Ó Û Ôχ ¯·ÌËÏ‹ ‰fiÛË, 0,1 mg/kg/ËÌ. Û ·Ú‹ÌÂÚÔ Û¯‹Ì·, ̤¯ÚÈ ÙÔ Ù¤ÏÔ˜ Ù˘ ÌÂϤÙ˘, ÏfiÁˆ ÂÈÌÔÓ‹˜ Ù˘ ÓfiÛÔ˘. ∂›‰Ú·ÛË Ù˘ ·ÓÙÈ-TNF ıÂÚ·›·˜ ÛÙËÓ Â›Ù¢ÍË Ï‹ÚÔ˘˜ ‡ÊÂÛ˘: ∆Ô ¤Ó· ÙÚ›ÙÔ ÙˆÓ ·ÛıÂÓÒÓ (30,5%) Ô˘ ¿Ú¯ÈÛ·Ó ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ˜ 14/46 (ET=12, Paediatriki 2009;72:29-40

AD=3) - ¤Ó·˜ ·ÛıÂÓ‹˜ ·Ó‹ÎÂ Î·È ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ ‰È¤ÎÔ„·Ó ÙË ıÂÚ·›· ÏfiÁˆ Ï‹ÚÔ˘˜ ‡ÊÂÛ˘ ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 2 ¯ÚfiÓÈ· (2-4). ∏ ·¿ÓÙËÛË ·˘Ù‹ ÛÙËÓ ·ÓÙÈTNF ·ÁˆÁ‹ ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ ·Ú·ÙËÚ‹ıËΠÛÙ· ÚÒÙ· 2-3 ¯ÚfiÓÈ· Ù˘ ıÂÚ·›·˜. √È 10/14 (71,5%) fï˜, 2-6 Ì‹Ó˜ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ ÙÔ˘ ·ÓÙÈTNF, ÂÌÊ¿ÓÈÛ·Ó ¤Í·ÚÛË Ù˘ ÓfiÛÔ˘, Ì ÎÏÈÓÈ΋ ÂÈÎfiÓ· ËÈfiÙÂÚË Û ۯ¤ÛË Ì ÙËÓ ÂÈÎfiÓ· ÚÈÓ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÓÙÈ-TNF ıÂÚ·›·˜. ∞ÓÙÈÌÂÙˆ›ÛıËÎ·Ó Ì ÙË ¯ÔÚ‹ÁËÛË ÙÔ˘ ¿ÏÏÔ˘ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· (4/10) ‹ ÌÂ Û˘Ó‰˘·ÛÌfi 2 DMARDs Ì ‹ ¯ˆÚ›˜ ÛÙÂÚÔÂȉ‹ (6/10). ∞fi ÙÔ˘˜ ˘fiÏÔÈÔ˘˜ 4 ·ÛıÂÓ›˜ Ô˘ ·Ú·Ì¤ÓÔ˘Ó Û Ï‹ÚË ‡ÊÂÛË ¯ˆÚ›˜ ıÂÚ·›·, ÔÈ 2 ¤¯Ô˘Ó ‹‰Ë Û˘ÌÏËÚÒÛÂÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ 2 ¯ÚfiÓÈ· ‡ÊÂÛ˘. ∂›‰Ú·ÛË Ù˘ ·ÓÙÈ-TNF ıÂÚ·›·˜ ÛÙË Û˘ÛÙËÌ·ÙÈ΋ ÌÔÚÊ‹ Ù˘ ¡π∞: √È 9/46 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘

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¶ÂÓÙ·ÂÙ‹˜ ÂÌÂÈÚ›· ·fi ÙË ¯Ú‹ÛË etanercept Î·È adalimumab ÛÙË Ó·ÓÈ΋ ȉÈÔ·ı‹ ·ÚıÚ›Ùȉ·

›¯·Ó Û˘ÛÙËÌ·ÙÈ΋ ¡IA (4/9 Ì ÚÔ¤¯Ô˘Û˜ ÙȘ Û˘ÛÙËÌ·ÙÈΤ˜ ÂΉËÏÒÛÂȘ Î·È 5/9 Ì ÂÈÎfiÓ· ÔÏ˘·ÚıÚ›Ùȉ·˜ ÛÙËÓ ÔÚ›·. √È 7/9 ¤Ù˘¯·Ó ACRped 50-70 Ì ¤Ó·Ó (5/7) ‹ 2 ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ˜ (2/7) Î·È ÔÈ 2/9 ›¯·Ó Ùˆ¯‹ ·¿ÓÙËÛË (ACRped ≤30). ™˘ÓÔÏÈο, 5 ¯ÚfiÓÈ· ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ·ÁˆÁ‹˜ Ì ∂∆ ‹ AD, Ë ÂÎÙ›ÌËÛË Â›Ó·È fiÙÈ Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ (42/46, 91,3%) ˆÊÂÏ‹ıËÎ·Ó ÛËÌ·ÓÙÈο ·fi Ù· Û˘ÁÎÂÎÚÈ̤ӷ ·ÓÙÈ-TNF Û΢¿ÛÌ·Ù· (›Ù ·fi ÙÔ ¤Ó· ›Ù ·fi ÙÔ ¿ÏÏÔ Â›ÙÂ Î·È ·fi Ù· ‰‡Ô), ·ÊÔ‡ ¤Ù˘¯·Ó Ï‹ÚË ‡ÊÂÛË (30,5%) ‹ ACRped 5070 (60,86%) ÌÂÙ¿ 2-4 ¯ÚfiÓÈ·. √È 4/46 (8,7%) ‰ÂÓ ·¿ÓÙËÛ·Ó ÈηÓÔÔÈËÙÈο (ACRped ≤30) ηÈ, fiˆ˜ ÚԷӷʤÚıËÎÂ, ÔÈ ÌÈÛÔ› ·Ó‹Î·Ó ÛÙÔ Û˘ÛÙËÌ·ÙÈÎfi Ù‡Ô ¤Ó·Ú͢ Ù˘ ÓfiÛÔ˘, Ì ÌÂÙ¿ÙˆÛË Û Â›ÌÔÓË ‰È·‚ÚˆÙÈ΋ ÔÏ˘·ÚıÚ›Ùȉ·.

™˘˙‹ÙËÛË ∏ ÌÂϤÙË ·˘Ù‹ ·ÔÙÂÏ› ÙËÓ ÚÒÙË ·ÔÙ›ÌËÛË Ù˘ 5ÂÙÔ‡˜ ÂÌÂÈÚ›·˜ ˆ˜ ÚÔ˜ ÙËÓ ·ÛÊ¿ÏÂÈ· Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ¯ÔÚ‹ÁËÛ˘ ‰‡Ô ·ÓÙÈ-TNF ·Ú·ÁfiÓÙˆÓ - ∂∆ Î·È AD - Û ·È‰È¿ Ì ¡π∞ ÛÙË ¯ÒÚ· Ì·˜. ∞Ó¿ÏÔÁË Ì·ÎÚÔ¯ÚfiÓÈ· ‰ËÌÔÛÈÂ˘Ì¤ÓË ÂÌÂÈÚ›· ÁÈ· ÌÂÓ ÙÔ ∂∆ ‰ËÌÔÛȇıËΠ·fi ÙÔ˘˜ Lovell Î·È Û˘Ó. (12) Û ·È‰È¿ Ì ÔÏ˘·ÚıÚÈ΋ ÔÚ›· NIA Î·È 4ÂÙ‹ ·Ú·ÎÔÏÔ‡ıËÛË, ÁÈ· ‰Â ÙÔ AD ‰ËÌÔÛȇıËΠÚfiÛÊ·Ù·, ·fi ÙÔ˘˜ Biester Î·È Û˘Ó. (17) Î·È ÙÔ˘˜ Tynjala Î·È Û˘Ó. (18), 3ÂÙ‹˜ ÂÌÂÈÚ›· ˆ˜ ÚÔ˜ ÙËÓ Â›‰Ú·Û‹ ÙÔ˘ ÛÙË ¡π∞ Î·È ÛÙËÓ π∫. ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, Î·È Ù· ‰‡Ô Û΢¿ÛÌ·Ù· ·ԉ›¯ıËÎ·Ó ·ÛÊ·Ï‹ ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ. ™Ô‚·Ú¤˜ ÏÔÈÌÒÍÂȘ ηٷÁÚ¿ÊËÎ·Ó ÌfiÓÔ Û 2/46 ·ÛıÂÓ›˜ (4,34%), ÔÈ ÔÔ›ÔÈ ¯ÚÂÈ¿ÛıËÎ·Ó ÓÔÛËÏ›·. √ ¤Ó·˜ ·ÛıÂÓ‹˜ ˘fi ∂∆ ÓfiÛËÛ ·fi ¤ÚËÙ· ˙ˆÛÙ‹Ú· Ì ·Ú·ÙÂٷ̤ÓË ÔÚ›· Ô˘ ¯ÚÂÈ¿ÛıËΠ·ÁˆÁ‹ Ì ·Î˘ÎÏÔ‚›ÚË. ∏ ¿ÏÏË ·ÛıÂÓ‹˜, ÌÂÙ¿ ·fi 2ÌËÓË ıÂÚ·›· Ì AD Î·È ÚÔËÁÔ‡ÌÂÓË 2ÂÙ‹ ıÂÚ·›· Ì ÙÔÓ ›‰ÈÔ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· (AD) Î·È ÛÙË Û˘Ó¤¯ÂÈ· Ì ∂∆, ÓfiÛËÛ ·fi ÛÔ‚·Ú‹ Ïԛ̈ÍË Èı·ÓfiÓ ÙÔ˘ ∫¡™, Î·È Î·Ù¤ÏËÍ ÂÓÙfi˜ 2 ËÌÂÚÒÓ ·fi ÙËÓ Â›Û΄‹ Ù˘ Û ÓÔÛÔÎÔÌÂ›Ô Ù˘ ÂÚÈʤÚÂÈ·˜, Ì ‹‰Ë ÂÁηÙÂÛÙË̤ÓË ÓÂÊÚÈ΋ ·ÓÂ¿ÚÎÂÈ·. ∏ ·ÎÚÈ‚‹˜ ·ÈÙ›· ÙÔ˘ ı·Ó¿ÙÔ˘ ·Ú·Ì¤ÓÂÈ ·‰È¢ÎÚ›ÓÈÛÙË ÁÈ·Ù›, fiˆ˜ ÚԷӷʤÚıËÎÂ, ‰ÂÓ ¤ÁÈÓ ÓÂÎÚÔÙÔÌ‹. √È Lovell Î·È Û˘Ó.

(12,19,20) ·Ó·Ê¤ÚÔ˘Ó ÛÙË ÌÂϤÙË ÙÔ˘˜ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÛÔ‚·ÚÒÓ ÏÔÈÌÒÍÂˆÓ (12%), Èı·Ófiٷٷ ÏfiÁˆ ÙÔ˘ ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÛıÂÓÒÓ (n=58) Ô˘ ·Ú·ÎÔÏÔ‡ıËÛ·Ó Ì·ÎÚÔ¯ÚfiÓÈ·. ◊Ș ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ, fiˆ˜ ÎÔÈÓ¤˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ‹/Î·È ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡, ÂÌÊ·Ó›ÛıËÎ·Ó Û 17/59 (28,81%) ·ÓÙÈ-TNF Û˘Ó‰ڛ˜ (10/37 Ì ∂∆ Î·È 7/22 Ì AD) Ô˘ ¤Ï·‚·Ó ÔÈ ·ÛıÂÓ›˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, ÂÓÒ ÔÈ ÙÔÈΤ˜ ηÈ

Û˘ÛÙËÌ·ÙÈΤ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ Ô˘ ·ÊÔÚÔ‡Û·Ó ·ÔÎÏÂÈÛÙÈο ÙËÓ ÔÌ¿‰· ÙÔ˘ AD ‹Ù·Ó ÂÏ¿¯ÈÛÙ˜: 1/59 (1,6%) Î·È 2/59 (3,4%). ∏ Û˘¯ÓfiÙËÙ· ÙˆÓ ÏÔÈÌÒÍÂˆÓ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Â›Ó·È ·ÚfiÌÔÈ· (35%) Ì ·˘Ù‹ Ô˘ ·Ó·Ê¤ÚÂÙ·È ÛÙȘ ÌÂϤÙ˜ ÙˆÓ Lovell Î·È Û˘Ó. (12, 19, 20), ÂÓÒ Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ÙÔÈÎÒÓ ·ÏÏÂÚÁÈÎÒÓ ·ÓÙȉڿÛÂˆÓ ‹Ù·Ó Ôχ ÌÈÎÚfiÙÂÚË ·fi ÙËÓ ·Ó·ÊÂÚfiÌÂÓË ÛÙȘ ·Ú·¿Óˆ ÌÂϤÙ˜ (39%) (12,19). ™˘ÛÙËÌ·ÙÈΤ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ ‹ ı¿Ó·ÙÔ˜ ‰ÂÓ ·Ó·Ê¤ÚÔÓÙ·È Û η̛· ·fi ÙȘ ÌÂϤÙ˜ ·˘Ù¤˜. ∞Ó¿ÏÔÁË Û˘¯ÓfiÙËÙ· ·ÓÂÈı‡ÌËÙˆÓ ·ÓÙȉڿÛÂˆÓ Ì ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË Î·Ù·ÁÚ¿ÊÂÙ·È Î·È Û ÁÂÚÌ·ÓÔ-·˘ÛÙÚȷ΋ ÌÂϤÙË (ÔÛÔÛÙfi 3,4% ), ÂÓÒ Û ̛· Á·ÏÏÈ΋ ÌÂϤÙË Î·Ù·ÁÚ¿ÊÂÙ·È 5Ï¿ÛÈ· Û˘¯ÓfiÙËÙ· (20%) (21,22). ∏ ·ÚÔ˘Û›· ·ÓÂÈı‡ÌËÙˆÓ ·ÓÙȉڿÛÂˆÓ ·Ó¿ÁηÛ 4/46 ·ÛıÂÓ›˜ Ó· ‰È·Îfi„Ô˘Ó ÙÔÓ ·ÓÙÈ-TNF, ÙÔ AD ‹ ÙÔ ∂∆ Î·È Ë ·ÈÙ›· ‹Ù·Ó: Û˘ÛÙËÌ·ÙÈΤ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ, fiˆ˜ ÂÍ¿ÓıËÌ· Î·È ÎÓËÛÌfi˜ (2), ‰È·Ù·Ú·¯¤˜ ÂÌÌ‹ÓÔ˘ Ú‡Û˘ (1 ·ÛıÂÓ‹˜ ·fi ÙÔ˘˜ ÚÔ·Ó·ÊÂÚı¤ÓÙ˜ 2) Î·È ÂÌÊ¿ÓÈÛË π∫ (2). ∂›ÌÔÓË π∫, ˘fi ·ÓÙÈ-TNF ıÂÚ·›· Ì ET, ·Ó¤Ù˘Í·Ó 2/46 (4,34%) ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘ Ì·˜, 10 Î·È 15 ¯ÚfiÓÈ· ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ¡π∞, ÁÂÁÔÓfi˜ Ô˘ ·ÔÙ¤ÏÂÛ ÙËÓ ·ÈÙ›· ‰È·ÎÔ‹˜ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘. ∏ ÂÈÏÔ΋ ·˘Ù‹ ηٿ ÙË ‰È¿ÚÎÂÈ· ıÂÚ·›·˜ Ì ∂∆ ·Ú·ÙËÚ‹ıËÎÂ Î·È ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜ (18,23), Û ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi (16,61%), Î·È ÙÔ˘˜ Ô‰‹ÁËÛ Û ·ÏÏ·Á‹ ÙÔ˘ ∂∆ Ì ¿ÏÏÔÓ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· (›Ù INFL ›Ù AD), Ú·ÎÙÈ΋ Ô˘ ·ÎÔÏÔ˘ı‹ıËÎÂ Î·È ÛÙÔ˘˜ 2 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘ Ì·˜ (·fi ∂∆ Û AD). ªÂ ‚¿ÛË ÙËÓ ÂÌÂÈÚ›· ·˘Ù‹ Î·È ¿ÏÏ· ÚfiÛÊ·Ù· ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ, ˘ÈÔıÂÙ‹ıËΠ·fi ÙÔ Î¤ÓÙÚÔ Ì·˜ ˆ˜ ¿ÁÈ· Ù·ÎÙÈ΋ Ù· ÙÂÏÂ˘Ù·›· 2 ¯ÚfiÓÈ· Ù˘ ÌÂϤÙ˘ Ó· ÂÈϤÁÂÙ·È ˆ˜ ·ÓÙÈTNF ·Ú¿ÁÔÓÙ·˜ ÙÔ AD Û ·ÛıÂÓ›˜ Ì NIA Î·È ÂÓÂÚÁfi π∫ (17,18,25,26). ÕÏÏ· ·ÓÂÈı‡ÌËÙ· Û˘Ì‚¿Ì·Ù·, fiˆ˜ Ê˘Ì·Ù›ˆÛË ‹ ÔÙÈ΋ Ó¢ڛÙȉ·, Ô˘ ·Ô‰fiıËÎ·Ó ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜ ÛÙË ıÂÚ·›· Ì INFL Î·È ∂∆ ·ÓÙ›ÛÙÔȯ·, ‰ÂÓ ·Ú·ÙËÚ‹ıËÎ·Ó ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË (27,28). ∏ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ ‰‡Ô ·ÓÙÈ-TNF ·Ú·ÁfiÓÙˆÓ ‹Ù·Ó Ôχ ˘„ËÏ‹ ÛÙËÓ ·ÚÔ‡Û· ÌÂϤÙË. ™˘ÓÔÏÈο 42/46 (91,3%) ·ÛıÂÓ›˜ ¤Ù˘¯·Ó ACRped 50-70 Ì’ ¤Ó·Ó ÙÔ˘Ï¿¯ÈÛÙÔÓ ·fi Ù· 2 ·ÓÙÈ-TNF Û΢¿ÛÌ·Ù· Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËηÓ. ∆Ô ·ÔÙ¤ÏÂÛÌ· ·˘Ùfi ·ÍÈÔÏÔÁÂ›Ù·È ˆ˜ ȉȷ›ÙÂÚ· ÛËÌ·ÓÙÈÎfi, ·Ó Ï¿‚ÂÈ Î·Ó›˜ ˘fi„Ë ÙÔ˘ fiÙÈ fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ ›¯·Ó ÌÂÁ¿ÏË ‰È¿ÚÎÂÈ· ÓfiÛÔ˘ - ¿Óˆ ·fi 5 ¯ÚfiÓÈ· fiÙ·Ó ¿Ú¯ÈÛ·Ó ÙËÓ ·ÓÙÈ-TNF ıÂÚ·›· - Î·È ‹Ù·Ó ·ÓıÂÎÙÈÎÔ› Û ÚÔËÁËı›۷ ¯ÔÚ‹ÁËÛË DMARDs. ¶·ÚfiÌÔÈ· ¢ÓÔ˚ο ·ÔÙÂϤÛÌ·Ù· ÂÚȤÁÚ·„·Ó Î·È ¿ÏÏÔÈ ¶·È‰È·ÙÚÈ΋ 2009;72:29-40

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ª. ∆Ú·¯·Ó¿ Î·È Û˘Ó.

ÂÚ¢ÓËÙ¤˜ (15,21,22,29-31). ∏ ÔÏ˘ÎÂÓÙÚÈ΋ ÁÂÚÌ·ÓÔ-·˘ÛÙÚȷ΋ ÌÂϤÙË, Ô˘ ‰È‹ÚÎÂÛ ÏÈÁfiÙÂÚÔ ·fi ÙËÓ ·ÚÔ‡Û·, ·Ó·Ê¤ÚÂÈ fiÙÈ 80% ÙˆÓ ·ÛıÂÓÒÓ ˘fi ∂∆ ¤Ù˘¯·Ó ACRped 30 (22), ÔÛÔÛÙfi ·Ó¿ÏÔÁÔ Ì’ ·˘Ùfi ÙˆÓ Lovell Î·È Û˘Ó. (20) ÛÙ· 2 ¯ÚfiÓÈ· ·Ú·ÎÔÏÔ‡ıËÛ˘ (81% ¤Ù˘¯·Ó ACRped 30). ∂ÂÎÙ›ÓÔÓÙ·˜, fï˜, ÙË ÌÂϤÙË ÙÔ˘˜ ÔÈ Lovell Î·È Û˘Ó. Û 4 ¯ÚfiÓÈ· ·Ú·ÎÔÏÔ‡ıËÛ˘ Î·È ·ÍÈÔÏÔÁÒÓÙ·˜ ÙȘ Â› ̤ÚÔ˘˜ ·Ú·Ì¤ÙÚÔ˘˜ ‚ÂÏÙ›ˆÛ˘ Ù˘ ÓfiÛÔ˘, Ô˘ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙ· ACRped ÎÚÈÙ‹ÚÈ·, ·Ó·Ê¤ÚÔ˘Ó fiÙÈ, ·Ó¿ÏÔÁ· Ì ÙËÓ ·Ú¿ÌÂÙÚÔ, 16-63% ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ‹Ú·Ó ·ÔÎÏÂÈÛÙÈο ∂∆ ¤Ù˘¯·Ó Ï‹ÚË ‡ÊÂÛË (12). ŸÛÔÓ ·ÊÔÚ¿ ÙÔ ÓÂfiÙÂÚÔ Û ΢ÎÏÔÊÔÚ›· Û··ÛÌ· AD, Ôχ ÚfiÛÊ·Ù· ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ ·fi °ÂÚÌ·Ó›· (17) Î·È ºÈÓÏ·Ó‰›· (18), Ì ÌÈÎÚÔ‡˜ ·ÚÈıÌÔ‡˜ ·ÛıÂÓÒÓ (max 20) Î·È ÌÈÎÚfiÙÂÚÔ ¯ÚfiÓÔ ·Ú·ÎÔÏÔ‡ıËÛ˘, ·Ó·Ê¤ÚÔ˘Ó Â›Û˘ ˘„ËÏ¿ ÔÛÔÛÙ¿ ÂÈÙ˘¯›·˜ (55,6% Î·È 60% ·ÓÙ›ÛÙÔȯ·). ª›· ÂÈϤÔÓ ÔÏ˘ÎÂÓÙÚÈ΋ ÌÂϤÙË Ù˘ ·ÛÊ¿ÏÂÈ·˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ ÙÔ˘ AD ·fi ΤÓÙÚ· Ù˘ ∂˘ÚÒ˘ Î·È ÙˆÓ ∏¶∞ Ô˘ ¤¯ÂÈ ·Ó·ÎÔÈÓˆı› (·‰ËÌÔÛ›Â˘Ù· ‰Â‰Ô̤ӷ), Ì ÌÂÁ·Ï‡ÙÂÚÔ ‰Â›ÁÌ· ·ÛıÂÓÒÓ (¡=148), η٤ÁÚ·„ ·Ó¿ÏÔÁ· ¢ÓÔ˚ο ·ÔÙÂϤÛÌ·Ù· ÌÂÙ¿ ·fi 11 Ì‹Ó˜ ıÂÚ·›·˜ (32). ∆Ô ˘„ËÏfiÙÂÚÔ ÔÛÔÛÙfi ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ ÙˆÓ ·ÓÙÈ-TNF Û΢·ÛÌ¿ÙˆÓ ÛÙË ‰È΋ Ì·˜ ÌÂϤÙË ÔÊ›ÏÂÙ·È Ì¿ÏÏÔÓ ÛÙÔÓ ‰È·ÊÔÚÂÙÈÎfi ÙÚfiÔ ·ÍÈÔÏfiÁËÛ˘, Ë ÔÔ›· ¤ÁÈÓ Ì ‚¿ÛË ÙÔ ÙÂÏÈÎfi ·ÔÙ¤ÏÂÛÌ· Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ¯ÔÚ‹ÁËÛ˘ Ì›·˜, ‰‡Ô ‹ Î·È ÙÚÈÒÓ ıÂÚ·¢ÙÈÎÒÓ Û˘Ó‰ÚÈÒÓ (59 Û˘Ó‰ڛ˜ Û 46 ·ÛıÂÓ›˜). ¢È·¯ÚÔÓÈο, ·Ú·ÙËÚ‹ıËΠÌÈ· ÂÏ·ÊÚÒ˜ Êı›ÓÔ˘Û· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Î·È ÙˆÓ 2 ·ÓÙÈ-TNF Û΢·ÛÌ¿ÙˆÓ. ™ÙËÓ ÂÙ‹ÛÈ· ÂÎÙ›ÌËÛË ÙÔ˘ ∂∆, ÙÔ ACRped 50-70 ÙˆÓ ·ÛıÂÓÒÓ ÂÏ·ÙÙÒıËΠ·fi 88,9% Û 75% ·fi ÙÔÓ 1Ô ÛÙÔÓ 5Ô ¯ÚfiÓÔ, ÔÛÔÛÙfi ˆÛÙfiÛÔ È‰È·›ÙÂÚ· ÈηÓÔÔÈËÙÈÎfi Ì ‚¿ÛË ÙËÓ ÂÈÏÔÁ‹ ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ›¯·Ó ·ÓıÂÎÙÈ΋ ÓfiÛÔ. ∏ ·ÓÙ›ÛÙÔÈ¯Ë ·Ú·Ù‹ÚËÛË ›Û¯˘ÛÂ Î·È ÁÈ· Ù· 2 ÚÒÙ· ¯ÚfiÓÈ· ¯ÔÚ‹ÁËÛ˘ ÙÔ˘ AD (·fi 71,4% Û 66,7%). ∞Ó¿ÏÔÁË ÂÌÂÈÚ›· ÂÚÈÁÚ¿ÊÂÙ·È Î·È ÛÙË Á·ÏÏÈ΋ ÌÂϤÙË ·ÛıÂÓÒÓ ˘fi ∂∆ (22). ¶Èı·ÓÔÏÔÁÂ›Ù·È Ë ·Ó¿Ù˘ÍË ÂÍÔ˘‰ÂÙÂÚˆÙÈÎÒÓ ÚÔ˜ ÙÔÓ ·ÓÙÈ-TNF ·ÓÙÈۈ̿وÓ, Ô˘ ÌÂÈÒÓÔ˘Ó ¤ÙÛÈ ÙË ‰Ú·ÛÙÈÎfiÙËÙ¿ ÙÔ˘ (22). ª›· ÂÈϤÔÓ ¤Ó‰ÂÈÍË Ù˘ ¢ÓÔ˚΋˜ ‰Ú¿Û˘ ÙˆÓ ·ÓÙÈ-TNF Û΢·ÛÌ¿ÙˆÓ ‹Ù·Ó Î·È Ë ÛÙ·‰È·Î‹ ‰È·ÎÔ‹ ÙˆÓ ÛÙÂÚÔÂȉÒÓ Ì¤Û· Û 6 Ì‹Ó˜ ÛÙȘ 18/25 ıÂÚ·¢ÙÈΤ˜ Û˘Ó‰ڛ˜ (72%). √È Lovell Î·È Û˘Ó. ·Ó·Ê¤ÚÔ˘Ó ·Ó¿ÏÔÁÔ ÔÛÔÛÙfi (80%) ÛÙËÓ ÙÂÙÚ·ÂÙ‹ ÂÌÂÈÚ›· ÙÔ˘˜ (12). ∆Ô 1/3 ÂÚ›Ô˘ ÙˆÓ ·ÛıÂÓÒÓ (30,5%) ‰È¤ÎÔ„Â ÙËÓ ·ÓÙÈ-TNF ıÂÚ·›· ÏfiÁˆ Ï‹ÚÔ˘˜ ‡ÊÂÛ˘ ÌÂÙ¿ ·fi 1 (14) ‹ 2 (1) ·ÓÙÈ-TNF Û˘Ó‰ڛ˜ (¤Ó·˜ ·ÛıÂPaediatriki 2009;72:29-40

Ó‹˜ ›¯Â ‡ÊÂÛË Î·È Ì ÙÔ˘˜ ‰‡Ô ·ÓÙÈ-TNF, ÙÔ˘˜ ÔÔ›Ô˘˜ ‹Ú Ì ÌÂÛԉȿÛÙËÌ· 16 ÌËÓÒÓ ÂÚ›Ô˘). √È 10/14 Ô˘ ˘ÔÙÚÔ›·Û·Ó ÌÂÙ¿ ÙË ‰È·ÎÔ‹ ÙÔ˘ ∂∆ ‹ ÙÔ˘ AD ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ 5ÂÙ›·˜ ›¯·Ó ËÈfiÙÂÚË ÂÈÎfiÓ· ·’ ·˘Ù‹Ó Ô˘ ›¯·Ó ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÓÙÈ-TNF ıÂÚ·›·˜. ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi, Ô˘ ·Ú·ÙËÚ‹ıËÎÂ Î·È ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜, (33,34) Ì·˜ ÚÔ‚ÏËÌ·Ù›˙ÂÈ ˆ˜ ÚÔ˜ ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· Ô˘ Ú¤ÂÈ Ó· ‰È·ÙËÚËı› Ë ·ÓÙÈ-TNF ıÂÚ·›· ÌÂÙ¿ ÙËÓ Â›Ù¢ÍË Ï‹ÚÔ˘˜ ‡ÊÂÛ˘, ÚÔÎÂÈ̤ÓÔ˘ Ë ‡ÊÂÛË ·˘Ù‹ Ó· Â›Ó·È Ì·ÎÚÔ¯ÚfiÓÈ·. ∏ ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘ Ô˘ ·ÚÔ˘Û›·Û ȉȷ›ÙÂÚË ·ÓıÂÎÙÈÎfiÙËÙ· ÛÙËÓ ·ÓÙÈ-TNF ·ÁˆÁ‹ ‹Ù·Ó Ë Û˘ÛÙËÌ·ÙÈ΋ ÌÔÚÊ‹ Ì ÔÚ›· ÔÏ˘·ÚıÚ›Ùȉ·˜ (ÂÍ·Ú¯‹˜ ÌË ·¿ÓÙËÛË ‹ ˘ÔÙÚÔ‹ ηٿ ÙËÓ ÔÚ›· ‹ ˘ÔÙÚÔ‹ ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ıÂÚ·›·˜). ŒÙÛÈ, 2/9 ·ÛıÂÓ›˜ ÌÂ Û˘ÛÙËÌ·ÙÈ΋ ÌÔÚÊ‹ ‰ÂÓ ·¿ÓÙËÛ·Ó ÛÙËÓ ·ÓÙÈ-TNF ıÂÚ·›·, ÂÓÒ Ô ·ÓÙ›ÛÙÔȯԘ ·ÚÈıÌfi˜ ÛÙÔ˘˜ 46 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó 4/46 (p=0,003) (35,36). ™˘ÌÂÚ·ÛÌ·ÙÈο, Ù· Â˘Ú‹Ì·Ù· ·˘Ù¿ ‰Â›¯ÓÔ˘Ó: ·) ∆Ë Ì·ÎÚÔ¯ÚfiÓÈ· ·ÛÊ¿ÏÂÈ· Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙÔ˘ ET Î·È AD, ·ÏÏ¿ Î·È ÙËÓ Â›Ù¢ÍË ‡ÊÂÛ˘ Ì ÙË ¯ÔÚ‹ÁËÛË ÙˆÓ 2 ·˘ÙÒÓ ·Ú·ÁfiÓÙˆÓ. øÛÙfiÛÔ ··ÈÙÂ›Ù·È Â·ÁÚ‡ÓËÛË ÁÈ· ÙÔ ÂӉ¯fiÌÂÓÔ ÛÔ‚·ÚÒÓ ÏÔÈÌÒÍÂˆÓ Ô˘ ÌÔÚ› Ó· ¤¯Ô˘Ó ÌÔÈÚ·›· ¤Î‚·ÛË. ‚) ∆Ë ‰˘Ó·ÙfiÙËÙ· Ù˘ ÂÈÙ˘¯Ô‡˜ ÌÂٷΛÓËÛ˘ ·fi ÙÔÓ ¤Ó· ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· ÛÙÔÓ ¿ÏÏÔ Û ÂÚ›ÙˆÛË ·ÔÙ˘¯›·˜ ‹ Êı›ÓÔ˘Û·˜ ·¿ÓÙËÛ˘ ‹ ˘ÔÙÚÔ‹˜ ÌÂÙ¿ ·fi ‡ÊÂÛË. Á) ∆ËÓ Â˘ÓÔ˚΋ Â›‰Ú·ÛË ÙÔ˘ AD ÛÙËÓ π∫. ‰) ø˜ ÚÔ˜ ÙËÓ ÔÏÈÙÈ΋ Â·Ó·¯ÔÚ‹ÁËÛ˘ ÌÂÙ¿ ·fi ¯ÚfiÓÈ· ÙÔ˘ ·Ú¯ÈÎÔ‡ ·ÓÙÈ-TNF ·Ú¿ÁÔÓÙ· Ô˘ ¯ÔÚËÁ‹ıËÎÂ, ‰ÂÓ ÂÍ¿ÁÂÙ·È Î·Ó¤Ó· Û˘Ì¤Ú·ÛÌ·, ÏfiÁˆ ÙÔ˘ ÌÈÎÚÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ ·ÛıÂÓÒÓ (¡=2). ∂ÈϤÔÓ, Ù· Â˘Ú‹Ì·Ù· Ù˘ ÌÂϤÙ˘ ÂȂ‚·ÈÒÓÔ˘Ó ÙË ‰˘Ó·ÙfiÙËÙ· Ù˘ ÂÈÏÔÁ‹˜ ÙÔ˘ ∂∆ Î·È ÙÔ˘ AD ˆ˜ Ì›· ·ÎfiÌË ÂÓ·ÏÏ·ÎÙÈ΋ ıÂÚ·›· ÁÈ· ÔÚÈṲ̂Ó˜ ÌÔÚʤ˜ ¡π∞ (Â›ÌÔÓ· ÔÏÈÁÔ·ÚıÚÈΤ˜, ÔÏ˘·ÚıÚÈΤ˜) Ô˘ Â›Ó·È ·ÓıÂÎÙÈΤ˜ ÛÙË Û˘Ì‚·ÙÈ΋ ıÂÚ·›·, Ë ÔÔ›· Ú¤ÂÈ Ó· ‰ÔÎÈÌ¿˙ÂÙ·È ÈÔ ¤ÁηÈÚ· ÛÙËÓ ÔÚ›· Ù˘ ÓfiÛÔ˘, ÙÔ˘Ï¿¯ÈÛÙÔÓ ÚÈÓ ÂÁηٷÛÙ·ı› ηٷÛÙÚÔÊÈ΋ ·ÚıÚÔ¿ıÂÈ·. ∂¿Ó Ë ·¿ÓÙËÛË ·ÓÙ·ÔÎÚ›ÓÂÙ·È ÛÙ· ‰ÈÂıÓ‹ ÎÚÈÙ‹ÚÈ· Ô˘ ηıÔÚ›˙Ô˘Ó ÙË ‚ÂÏÙ›ˆÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ (ACRped 30-70), Ë ıÂÚ·›· ÌÔÚ› Ó· Û˘Ó¯›˙ÂÙ·È, ·ÏÏ¿ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ¿ Ù˘ Èı·ÓfiÓ Ó· ·ÚÔ˘ÛÈ¿˙ÂÈ Êı›ÓÔ˘Û· ÔÚ›·, fiˆ˜ Ê¿ÓËΠÛÙÔ˘˜ ‰ÈÎÔ‡˜ Ì·˜ ·ÛıÂÓ›˜. ¶·Ú·Ì¤ÓÂÈ ÏÔÈfiÓ Ô ÚÔ‚ÏËÌ·ÙÈÛÌfi˜ ÁÈ· fiÛÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÌÂÙ¿ ÙËÓ ÈηÓÔÔÈËÙÈ΋ ·¿ÓÙËÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ Ú¤ÂÈ Ó· Û˘Ó¯›ÛÔ˘Ì ÙË ¯ÔÚ‹ÁËÛË ÙÔ˘ ∂∆ ‹ AD (·Ó Ï¿‚ÂÈ Ì¿ÏÈÛÙ· ηÓ›˜ ˘fi„Ë ÙÔ˘ ÙÔ ˘„ËÏfi ÎfiÛÙÔ˜ ·˘Ù‹˜ Ù˘ ıÂÚ·›·˜), ÒÛÙ ӷ ÌËÓ

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¶ÂÓÙ·ÂÙ‹˜ ÂÌÂÈÚ›· ·fi ÙË ¯Ú‹ÛË etanercept Î·È adalimumab ÛÙË Ó·ÓÈ΋ ȉÈÔ·ı‹ ·ÚıÚ›Ùȉ·

ÙË ¯¿ÛÔ˘ÌÂ Î·È Ó· ÙË Û˘ÓÙËÚ‹ÛÔ˘Ì ˆ˜ ÌÈ· ÂÓ·ÏÏ·ÎÙÈ΋ ıÂÚ·¢ÙÈ΋ ÛÙÚ·ÙËÁÈ΋ (33,37).

µÈ‚ÏÈÔÁÚ·Ê›· 1. ∫·Ó·ÎÔ‡‰Ë-∆۷ηϛ‰Ô˘ º, ∆Ú·¯·Ó¿ ª, ¶Ú·ÙÛ›‰Ô˘°Î¤ÚÙÛË ¶, µ·ÛÈÏÂÈ¿‰Ë˜ ∫. ∂Ê·ÚÌÔÁ‹ Ó¤·˜ ıÂÚ·¢ÙÈ΋˜ ÛÙÚ·ÙËÁÈ΋˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·ÓıÂÎÙÈÎÒÓ ÌÔÚÊÒÓ Ó·ÓÈ΋˜ ȉÈÔ·ıÔ‡˜ ·ÚıÚ›Ùȉ·˜. ¶·È‰È·ÙÚÈ΋ µÔÚ›Ԣ ∂ÏÏ¿‰Ô˜ 2002;14:48-56.

2. Gutcher I, Becher B. APC-derived cytokines and T-cell polarization in autoimmune inflammation. J Clin Invest 2007;117:1119-1127.

3. Choy CH, Panayi GS. Cytokine pathways and joint inflammation in Rheumatoid Arthritis. N Engl J Med 2001;344:907-916.

4. Chira P, Sandborg CI. Novel therapies in pediatric rheumatic diseases. Curr Opin Pediatr 2003;15:579-585.

5. Wilkinson N, Jackson G, Gardner-Medwin J. Biologic Therapies for juvenile arthritis. Arch Dis Child 2003;88: 186-191.

6. Murray KJ, Lovell DJ. Advanced therapy for juvenile arthritis. Best Pract Res Clin Rheumatol 2002;16:361-378.

7. Furst DE, Schiff MH, Fleischmann RM, Strand V, Birbara CA, Compagnone D, et al. Adalimumab, a fully human antitumor necrosis factor alpha monoclonal antibody, and concomitant standard antirheumatic therapy for the treatment of rheumatoid arthritis: results of STAR (Safety Trial of Adalimumab in Rheumatoid Arthritis). J Rheumatol 2003;30:2569-2571.

8. Hashkes PJ, Shajrawi I. Sarcoid-related uveitis occurring during etanercept therapy. Clin Exp Rheumatol 2003;21: 645-646.

9. Schmeling H, Horneff G. Etanercept and uveitis in patients with juvenile idiopathic arthritis. Rheumatology (Oxford) 2005;44:1008-1011.

10. Taban M, Dupps WJ, Mandell B, Perez VL. Etanercept (enbrel)-associated inflammatory eye disease: case report and review of the literature. Ocul Immuno Inflam 2006;14: 145-150.

11. Giannini EH, Ruperto N, Ravelli A, Lovell DJ, Felson DT, Martini A. Preliminary definition of improvement in juvenile idiopathic arthritis. Arthritis Rheum 1997;40: 1202-1209.

12. Lovell DJ, Reiff A, Olcay Y, Schneider R, Nocton J, Stein LD, et al. Long-term safety and efficacy of etanercept in children with polyarticular course juvenile rheumatoid arthritis. Arthritis Rheum 2006;54:1987-1994.

13. Pratsidou-Gertsi R, Vougiouka O, Tsitsami E, Ruperto N, Siamopoulou-Mavridou A, Dracou C, et al. The Greek version of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ). Clin Exp Rheumatol 2001;19(Suppl 23):S76-S80.

14. Wallace CA, Ruperto N, Giannini EH for the Childhood Arthritis and Rheumatology Research Alliance (CARRA), The Pediatric Rheumatology International Trials Organization (PRINTO), and the Pediatric Rheumatology Collaborative Study Group (PRCSG). Preliminary criteria for clinical remission for selected categories of juvenile idiopathic arthritis. J Rheumatol 2004;2290-2294.

15. Lahdenne P, Vahasalo P, Honkanen V. Infliximab or etanercept in the treatment of children with refractory

juvenile idiopathic arthritis: an open label study. Ann Rheum Dis 2003;62:245-247.

16. Kanakoudi-Tsakalidou F, Tzimouli V, Pratsidou-Gertsi P, Chronopoulou E, Trachana M. The significance of persistent newly developed autoantibodies in JIA patients under long-term anti-TNF treatment. Cytokine 2008; doi:10.1016/j.cyto.2008.01.007, Volume 42 (p. xxx-xxx) (In Press).

17. Biester S, Deuter C, Michels H, Haefner R, KuemmerleDeschner J, Doycheva D, et al. Adalimumab in the therapy of uveitis in childhood. Br J Ophthalmol 2007;91:319-324.

18. Tynjälä P, Kotaniemi K, Lindahl P, Latva K, Aalto K, Honkanen V, et al. Adalimumab in juvenile idiopathic arthritis-associated chronic anterior uveitis. Rheumatology 2008;47:339-344.

19. Lovell DJ, Giannini EH, Reiff A, Jones OY, Schneider R, Olson JC, et al. Long-term efficacy and safety of etanercept in children with polyarticular-course juvenile rheumatoid arthritis: interim results from an ongoing multicenter, open-label, extended-treatment trial. Arthritis Rheum 2003;48:218-226.

20. Lovell DJ, Giannini EH. Etanercept therapy in children with juvenile rheumatoid arthritis. Clin Exp Rheumatol 2004;2:522.

21. Horneff G, Schmeling H, Biedermann T, et al. The German etanercept registry for treatment of juvanile idiopathic arthritis. Ann Rheum Dis 2004;63:1638-1644.

22. Quartier P, Taupin P. Bourdeau F, Lemelle I, Pillet P, Bost M, et al. Efficacy of etanercept for the treatment of juvenile idiopathic arthritis according to the onset type. Arthritis Rheum 2003;48:1093-1101.

23. Tynjälä P, Lindahl P, Honkanen V, Lahdenne P, Kotaniemi K. Infliximab and etanercept in the treatment of chronic uveitis associated with refractory juvenile idiopathic arthritis. Ann Rheum Dis 2007;66:548-550.

24. Karlsson JA, Kristensen LE, Kapetanovic MC, Gulfe A, Saxne T, Geborek P. Treatment response to a second or third TNFinhibitor in RA: results from the South Swedish Arthritis Treatment Group Register. Rheumatololy (Oxford) 2008; 47:507-513.

25. Sarah H, Lightman S. Anti-TNF therapies in the management of acute and chronic uveitis. Cytokine 2006; 33:231-237.

26. Saurenmann RK, Levin AV, Feldman BM, Rose JB, Laxer RM, Schneider R, et al. Prevalence, risk factors and outcome of uveitis in juvenile idiopathic arthritis. Arthritis Rheum 2007;56:647-657.

27. Armbrust W, Kamphuis SS, Wolfs TW, Fiselier TJ, Nikkels PG, Kuis W, et al. Tuberculosis in a nine-year-old girl treated with infliximab for systemic juvenile idiopathic arthritis. Rheumatology (Oxford) 2004;43:527-529.

28. Tauber T, Turetz J, Barash J, Avni I, Morad Y. Optic neuritis associated with Etanercept therapy for Juvenile arthritis. J AAROS 2006;10:26-29.

29. Schmeling H, Mathony K, John V, Keysser G, Burdach S, Horneff G. A combination of etanercept and methotrexate for the treatment of refractory juvenile idiopathic arthritis: a pilot study. Ann Rheum Dis 2001;60:410-412.

30. Kietz DA, Pepmueller PH, Moore TL. Theurapeutic use of etanercept in polyarticular course juvenile idiopathic arthritis over a two year period. Ann Rheum Dis 2002;61: 171-173. ¶·È‰È·ÙÚÈ΋ 2009;72:29-40

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ª. ∆Ú·¯·Ó¿ Î·È Û˘Ó.

31. Lovell DJ. Update on treatment of arthritis in children.

New treatments, new goals. Bull NYU Hosp Joint Dis 2006;64:72-76.

32. Ruperto N. 2006 EULAR meeting proceedings. 48-week data from the study of adalimumab in children with juvenile rheumatoid arthritis (JR). Ann Rheum Dis 65:OP007 (Suppl).

33. Gartlehner G, Hansen RA, Jonas BL, Thieda P, Lohr KN. Biologics for the treatment of juvenile idiopathic arthritis: a systematic review and critical analysis of the evidence.

Clin Rheumatol 2008;27:67-76.

34. Furst DE, Breedveld FC, Kalden JR, Smolen JS, Burmester GR, Emery P, et al. Updated concensus statement on biological agents for the treatment of rheumatic diseases, 2006. Ann Rheum Dis 2006;65(Suppl III):iii2-15.

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35. Russo R∞, Katsicas MM, Zelazco M. Etanercept in systemic juvenile idiopathic arthritis. Clin Exp Rheumatol 2002; 20:723-726.

36. Kimura Y, Pinho P, Walco G, Higgins G, Humell D, Szer I, et al. Etanercept treatment in patients with refractory systemic onset juvenile rheumatoid arthritis. J Rheumatol 2005;32:935-942.

37. Moots R, Taggart A, Walker D. Biologic therapy in clinical practice: enthusiasm must be tempered by caution.

Rheumatology 2003;42:614-616.

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Obesity prevalence in children aged 8-13 years according to IOTF cutoffs, CDC growth charts and Z-BMI scores N. Mantzouranis, H. Douda, T. Pilianidis, S. Tokmakidis Abstract Background: Few epidemiological studies have compared different classification criteria of childhood obesity. The study on Greek Obesity and Lifestyle (GOAL) investigated the prevalence of childhood obesity in a sample of Greek schoolchildren, comparing three classification criteria.

Methods: The study was conducted on 2,056 schoolchildren, aged 8-13 years. The comparisons of obesity were based on the International Obesity Task Force (IOTF) cutoffs, the Centers for Disease Control (CDC) growth charts and body mass index-for-age Z-scores (Z-BMI): median = normal weight, overweight ≥+1SD, obese ≥+2SD. Results: A higher prevalence of obesity and overweight was observed when the CDC growth charts were used [37.6%, 95% CI (35.3-39.9)], while the IOTF cutoffs, revealed slightly lower rates [36.6%, 95% CI (34.3-38.9)]. In relation to both the CDC and IOTF classifications, a significantly lower prevalence was observed when obesity was estimated using Z-BMI [15.6%, 95% CI (13.9-17.3), p<0.05]. ANOVA statistical analysis showed that obesity prevalence was estimated significantly higher in both boys and girls by the CDC and IOTF classification systems than by Z-BMI (p<0.05).

Conclusions: When childhood obesity was based on either the IOTF or the CDC criteria, it was found to be the highest ever recorded in a Greek paediatric population, with results nearing the prevalence rates reported for US children. As compared to Z-BMI, the IOTF and CDC classification systems were found to be more appropriate in specifying the prevalence rates of childhood obesity and should, therefore, be used for future public health applications.

Department of Physical Education and Sports Science, Democritus University of Thrace, Komotini 69100, Greece Correspondence: Savvas P. Tokmakidis stokmaki@phyed.duth.gr Department of Physical Education and Sports Science (T.E.F.A.A.) Democritus University of Thrace Komotini 69100, Greece

Key words: Childhood, preadolescence, obesity, classification criteria.

Abbreviations: BMI CDC CI IOTF WHO

Body Mass Index Centers for Disease Control Confidence Interval International Obesity Task Force World Health Organization

Introduction In many developed societies characterized by physical inactivity and over-consumption, obesity has emerged as an undesirable phenomenon. Obviously, excessive fat accumulation in any individual is the result of a long-term positive energy balance and physical inactivity (1). International health-related organizations regard the prevalence of adult and childhood obesity as a global epidemic (2-4). The global prevalence of childhood and adolescent obesity is rising rapidly and could be characterized as a public-health crisis (5). In the USA, the obesity (including overweight) trends more than doubled between 1976 and 2002 (NHAHES & NHANES II, III) and currently one in six children and adolescents are obese (6).

In Europe, the highest prevalence trends are observed in the southern Mediterranean countries

(7). In Greece, both representative (8-10) and non representative cross-sectional studies (11-14) have shown the prevalence of childhood and adolescence obesity to be extremely high, leading to the conclusion that Greek children are amongst the most obese in Europe.

In epidemiological studies, the evaluation of obesity is based primarily on the use of the practical indirect measure of Body Mass Index (BMI).

Two classification methods are recommended for the evaluation of childhood obesity: the USA Centers for Disease Control (CDC) new growth charts (CDC-2000) based on five nationally representative surveys, conducted between 1963 and 1994 (15), and the International Obesity Task Force (IOTF) recommended BMI cutoff points for the definition of overweight and obesity, using anthropometric data collected from six different populations (16). Studies that have compared the above classification methods in the same population found that the BMI reference cutoffs have relatively high specificity, but lower sensitivity (17-18). For this reason, the Multicentre Growth Reference Study (MGRS) of the ¶·È‰È·ÙÚÈ΋ 2009;72:41-45

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tained prior to participation. The GOAL study sampling model was based on the National 2001 census total population distribution and from biostatistical data obtained from the General Secretary of the Hellenic Statistical Service (Figure 1).

Official approval for the GOAL study protocol was granted by the Ministry of Education of Greece (Division of Management and Health), the Scientific Research Committee of the Pedagogical Institute of Greece and the ethics committee of the Democritus University of Thrace.

Figure 1. Map of Greece. The dots show the schools in which the Greek Obesity and Lifestyle (GOAL) data were collected.

World Health Organization (WHO) proposed the Z-score curves for BMI-for-age (Z-BMI) for boys and girls from birth to 60 months as an innovative criterion for obesity evaluation (19). Taking into consideration the WHO curves for obesity evaluation based on the BMI-for-age and gender Z-score for the age-group 0-60 months, the present study, the Greek Obesity and Lifestyle (GOAL) study, was undertaken in order to assess the prevalence of childhood obesity according to three different classification criteria, namely the CDC-2000 growth charts, the IOTF cutoffs and the Z-BMI of a reference study population of Greek children aged 8-13 years.

Methods This cohort study was conducted on a stratified representative sample of 2,056 schoolchildren (1,148 boys, 908 girls), aged 10.2±1.3 years. The children evaluated were pupils from 19 randomly selected public primary schools (Figure 1). A whole school class from each of the four grades (3rd to 6th) was surveyed in each primary school. In schools with more than 250 students, one-third of all classes were selected by a random procedure, stratified according to city area and school location. In schools with less than 100 students, measurements were applied to the total school population. Written permission and consent from the subjects and their parents were ob-

Anthropometric data Each child’s age was calculated by subtracting the year, month and date of examination in from the year, month and date of the child’s birth. Body mass was measured to the nearest 100g on a calibrated floor scale (Seca 770), with the child standing in the centre with relaxed arms, wearing no shoes and only light sportswear. Height was measured to the nearest 0.1cm in bare feet, with the head in the Frankfort horizontal plane, using a stadiometer (Seca 240). BMI (kg/m2) was calculated as body mass (kg) divided by the squared height (m2). All the anthropometric measurements were duplicated and performed by the same trained researcher. The comparisons of obesity prevalence in the study population were based on IOTF cutoffs, CDC growth charts and Z-BMI, with 0 equivalent to median = normal weight, overweight ≥+1SD, obese ≥+2SD. Statistical analysis The reliability of the anthropometric measurements of the study was evaluated with the use of autocorrelation analysis (ACF) for different time series data. The syntax file calculates Z-scores for the anthropometric indicator of BMI-forage, based on the GOAL reference population data. The prevalence rates, with a 95% confidence interval (CI), according to the three childhood obesity classification criteria, were compared using the chi square non parametric statistic.

Cohen’s Kappa was applied in order to evaluate the classification criteria coefficient of agreement. Statistical significance was set at p <0.05. All statistical analyses were carried out by using the SPSS-PC software, version 16.0 for Windows (SPSS, Inc., Chicago, IL).

Results The Box-Ljung statistics in all lags were significant (p<0.001) and this does not affect the reliability measurements of body mass and height. The anthropometric characteristics of the study children are presented in Table 1.

The Z-BMI cutoffs for the boys and girls were

Table 1. Physical and anthropometric characteristics of children who participated in the GOAL* study, according to gender (mean, 95% CI) Total sample (n=2,056) Age (y) Weight (kg) Height (cm) BMI (kg/m2) * Greek Obesity and Lifestyle Paediatriki 2009;72:41-45

Boys (n=1,148) Mean 95% CI 10.2 39.8 142 19.3

10.1-10.3 39.1-40.5 141.9-143.1 19.1-19.6

Girls (n=908) Mean 95% CI 10.2 40 143 19.2

10.1-10.3 39.1-40.7 142.3-143.7 19-19.5

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Table 2. Simplified field table for Z-BMI according to gender, from the reference data of the GOAL* study Boys Z-BMI Age (Year)

Median

+1 SD

+2 SD

17.88 17.95 19.00 19.76 19.31

21.23 21.46 22.73 23.49 22.61

24.60 25.17 26.54 27.30 26.05

Median

+1 SD

+2 SD

17.25 18.11 19.02 19.56 19.32

20.25 21.33 22.83 23.50 23.03

23.34 24.64 26.78 27.50 26.89

8 9 10 11 12 Girls Z-BMI Age (Year) 8 9 10 11 12

the CDC and the IOTF classifications than to Z-BMI (p<0.05) (Figures 2,3).

The Cohen’s Kappa measure of agreement between the IOTF and CDC classification criteria was as high as 0.86, while the measure of agreement of both of these obesity classification criteria with the Z-BMI was very low (0.35 and 0.28, respectively).

Discussion Over the past three decades, the prevalence of childhood obesity has been evaluated using different BMI classification criteria. In contrast to a multinational comparative study which showed that the prevalence rates of obesity in pre-pubertal children was the same using the CDC and IOTF criteria (17), the present study found a 1% higher prevalence when obesity was defined according to the CDC growth charts. A cohort study in the USA comparing the prevalence of childhood obesity using different criteria showed that the CDC growth charts give in the same population 5% higher obesity rates in boys in relation to IOTF cutoff references (18). Comparing to the above study rates of obesity, the present study showed a 2.8% higher prevalence of obesity in boys but a 0.9% lower prevalence in girls aged 8-13 years using the CDC criterion, compared to the IOTF cutoffs.

In Europe, the prevalence rates of childhood obesity are based mainly on the IOTF definition (20,21). It has recently been demonstrated that BMI has low sensitivity in body fat measurement and estimates the variations from the normal body weight ideal standards (22). For this reason, WHO extended the growth references based on Z-BMI from infants and

* Greek Obesity and Lifestyle, Z-BMI = BMI-for-age score

developed for overweight and obesity classification for the GOAL study reference population (Table 2).

Chi-square statistics exhibited that the higher prevalence of childhood obesity (including overweight) was found when the CDC growth charts were used [37.6%, 95% CI (35.3-39.9)]. The prevalence of obesity as estimated according to the IOTF cutoffs recorded 1% lower rates [36.6%, 95% CI (34.338.9)]. Significantly lower prevalence was recorded when obesity was estimated by Z-BMI [15.6%, 95% CI (13.9-17.3)], compared to the CDC (¯2=2.32, df =4, p<0.05) and IOTF (¯2=1.28, df=4, p<0.05) classifications. Adjusted by gender, obesity prevalence was significantly higher in boys and girls according to both

21.4

CDC

18 .7

25.2

IOTF

11.2 *

GOALS

0

#

5

5

Overweight

12 .1

10

15

20

25

30

35

40

45

Obesity

* p<0.05 vs CDC & IOTF overweight #

p<0.05 vs obesity

Figure 2. Prevalence (%) of overweight and obesity in boys, according to the three BMI-for-age classification criteria: CDC, IOTF and Z-BMI (GOAL). ¶·È‰È·ÙÚÈ΋ 2009;72:41-45

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12.9

22

CDC

25.5

IOTF

GOALS

11.2 * 0

3.8

5 Overweight

10.3

10

#

15

20

25

30

35

40

45

Obesity

* p<0.05 vs CDC & IOTF overweight #

p<0.05 vs obesity

Figure 3. Prevalence (%) of overweight and obesity in girls, according to the three BMI-for-age classification criteria: CDC, IOTF and Z-BMI (GOAL).

toddlers to cover children and adolescents aged 5-19 years (23). The present study is one of the first studies to compare the CDC and IOTF obesity eva-luation criteria with the Z-BMI in primary schoolchildren.

Comparison with earlier representative population studies in Greece using the IOTF criteria shows that the prevalence of childhood obesity in the GOAL study, based on both the IOTF and the CDC classifications, is the highest ever recorded in Greece and approaches the obesity prevalence rates reported in the USA. However, the prevalence of overweight and obesity recorded in the study population using Z-BMI was even lower than the WHO-HBSC study (IOTF-CDC criteria), which was conducted on 11.5 year-old preadolescents, based on self-reported anthropometric data [(overweight: HBSCIOTF 15.2% - HBSCCDC 15.5% vs GOALBMI-for-age Z-scores 11.2%), (obesity: HBSCIOTF 1.5% - HBSCCDC 3.4% vs GOALBMI- for-age Z-scores 15.6%)].

In conclusion, the findings of the present study are in accordance with recent epidemiological studies, confirming that childhood obesity in Greece has increased rapidly in recent years. The comparison of three different classification systems showed that the Z-BMI did not appropriately specify childhood obesity in primary schoolchildren, in contrast with the IOTF and CDC classifications, and should not be used for public health applications. Therefore, a common obesity evaluation standard must be established for widespread use in the assessment of school-age children.

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ORIGINAL ARTICLE

ÃÚ‹ÛË Î·È Î·Ù¿¯ÚËÛË ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ Û ÊÔÈÙËÙ¤˜ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜ ∆Ì‹Ì· ¶ÚÔÛ¯ÔÏÈ΋˜ ∞ÁˆÁ‹˜, ™¯ÔÏ‹ ∂·ÁÁÂÏÌ¿ÙˆÓ ÀÁ›·˜ Î·È ¶ÚfiÓÔÈ·˜, ∞ÓÒÙ·ÙÔ ∆¯ÓÔÏÔÁÈÎfi ∂Î·È‰Â˘ÙÈÎfi ÿ‰Ú˘Ì· ∞ı‹Ó·˜ AÏÏËÏÔÁÚ·Ê›·: ™Ù¤ÏÈÔ˜ ∞ÓÙˆÓÈ¿‰Ë˜ prdrsant@otenet.gr ∏Ú·ÎÏ›ÙÔ˘ 4, ∫ÔψӿÎÈ 106 73 ∞ı‹Ó·

™. ∞ÓÙˆÓÈ¿‰Ë˜, Ã. °È·‚Ú‹, ∂. ¶··Ì·Î·Ú›Ô˘, ∫. ™Ù·‡ÚÔ˘, ª. ∑¤Ú‚·, ∂. ¢ÈÔÓ˘ÛÔÔ‡ÏÔ˘, ∂. ª·ÎÚÔÁΛη, µ. ™·ÏˆÓ›‰Ë˜ ¶ÂÚ›ÏË„Ë ∂ÈÛ·ÁˆÁ‹: ∏ ¯Ú‹ÛË Î·È Ë Î·Ù¿¯ÚËÛË ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ, fiˆ˜ ÙÔ ·ÏÎÔfiÏ, Ô Î·Ófi˜ Î·È Ù· Ó·ÚΈÙÈο, ÌÔÚ› Ó· ‚Ï¿„Ô˘Ó ÛÔ‚·Ú¿ ÙËÓ ˘Á›·. ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ¯Ú‹Û˘ Î·È Ù˘ ηٿ¯ÚËÛ˘ ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ ·fi Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ ÊÔÈÙËÙ¤˜ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ™ÙË ‰È¿ÚÎÂÈ· ÂÓfi˜ ¯ÚfiÓÔ˘, ÌÂÏÂÙ‹Û·ÌÂ, ‚¿ÛÂÈ ÚˆÙÔÎfiÏÏÔ˘ Ì ÎÏÂÈÛÙfi ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ, 954 ÊÔÈÙËÙ¤˜ Û¯ÔÏÒÓ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜ ÙÔ˘ ∞ÓÒÙ·ÙÔ˘ ∆¯ÓÔÏÔÁÈÎÔ‡ ∂Î·È‰Â˘ÙÈÎÔ‡ π‰Ú‡Ì·ÙÔ˜ ∞ı‹Ó·˜. ∞ÔÙÂϤÛÌ·Ù·: ∏ ËÏÈΛ· Î˘Ì·ÈÓfiÙ·Ó ÌÂٷ͇ 17-22 ÂÙÒÓ, 910 (95%) ‹Ù·Ó ŒÏÏËÓ˜, 522 (55%) ·fi ÙËÓ ∞ı‹Ó·, 945 (99%) ›¯·Ó Ê˘ÛÈÎÔ‡˜ ÁÔÓ›˜, 55 (6%) ›¯·Ó ‰È·˙¢Á̤ÓÔ˘˜ ÁÔÓ›˜. √ÈÓÔÓÂ˘Ì·ÙÒ‰Ë ‰ÂÓ Â›¯·Ó ÈÂÈ ÔÙ¤ 227 (24%), 725 (76%) ›¯·Ó ‰ÔÎÈÌ¿ÛÂÈ, 388 (54%) ¤ÈÓ·Ó ÂÚÈÛÙ·Ûȷο, 196 (27%) ¤ÈÓ·Ó Ì›· ÊÔÚ¿ ÙËÓ Â‚‰ÔÌ¿‰·, 119 (16%) ¤ÈÓ·Ó 2-3 ÊÔÚ¤˜ ÙËÓ Â‚‰ÔÌ¿‰·, 17 (2%) ¤ÈÓ·Ó Î¿ı ̤ڷ, 324 (45%) ¤ÈÓ·Ó Û˘Ó‹ıˆ˜ ÂÏ·ÊÚÈ¿ ÔÙ¿, 223 (31%) ¤ÈÓ·Ó ‚·ÚÈ¿ ÔÙ¿, 536 (74%) ¤ÈÓ·Ó 1-2 ÔÙ‹ÚÈ· οı ÊÔÚ¿, 147 (20%) ¤ÈÓ·Ó 3-5 ÔÙ‹ÚÈ· Î·È 31 (4%) ¤ÈÓ·Ó ÂÚÈÛÛfiÙÂÚ·, 339 (47%) ¤ÈÓ·Ó Û˘Ó‹ıˆ˜ Ì ·Ú¤·, 505 (81%) ¤ÈÓ·Ó Û ʿÛË ‰È·ÛΤ‰·Û˘, ‰È·ÎÔÒÓ. ª¤ıË ·Ó¤ÊÂÚ·Ó 400 (55%), Û 113 (18%) ˘‹ÚÍ ÌË ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓË ÛÂÍÔ˘·ÏÈ΋ Ú¿ÍË, Û 36 (5%) ηÎÔÔ›ËÛË, 848 (89%) ‰ÂÓ Ô‰ËÁÔ‡Û·Ó ˘fi ÙËÓ Â‹ÚÂÈ· ÔÈÓÔÓ‡̷ÙÔ˜. √È 629 (66%) ‰ÂÓ Î¿ÓÈ˙·Ó, 322 (34%) οÓÈ˙·Ó, 250 (78%) οÓÈ˙·Ó Û˘ÛÙËÌ·ÙÈο. √È 125 (39%) ·Ó¤ÊÂÚ·Ó ÙË ‰‡Ó·ÌË Ù˘ Û˘Ó‹ıÂÈ·˜ ˆ˜ ·ÈÙ›·, ÂÓÒ 154 (48%) ·Ó¤ÊÂÚ·Ó ÙËÓ ·fiÏ·˘ÛË ˆ˜ ·ÔÙ¤ÏÂÛÌ·. ŸÏÔÈ ÁÓÒÚÈ˙·Ó ÙȘ ‚Ï·‚ÂÚ¤˜ Û˘Ó¤ÂȘ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜ Î·È Â›¯·Ó Â·Ú΋ ÂÓË̤ڈÛË 244 (76%). ∆¤ÏÔ˜, 50 (5%) ‹Ù·Ó ¯Ú‹ÛÙ˜, ÚÔ˚fiÓÙˆÓ Î¿ÓÓ·‚˘ 32 (64%), ÎÔη˝Ó˘ 12 (24%), ¯·ÈÒÓ 4 (8%), ËÚˆ›Ó˘ 2 (4%), 19 (38%) ›¯·Ó ‰ÔÎÈÌ¿ÛÂÈ ÌfiÓÔ 1-2 ÊÔÚ¤˜, 17 (34%) ‹Ù·Ó Û˘ÛÙËÌ·ÙÈÎÔ› ¯Ú‹ÛÙ˜. ªÔÏÔÓfiÙÈ 47 (94%) ÁÓÒÚÈ˙·Ó ÙȘ ÂÈÙÒÛÂȘ, ÌfiÓÔ 16 (32%) ÂÈı˘ÌÔ‡Û·Ó Ó· ‰È·Îfi„Ô˘Ó. ™˘ÌÂÚ¿ÛÌ·Ù·: ∆Ô Û˘ÁÎÚÈÙÈο ÌÈÎÚfiÙÂÚÔ ÔÛÔÛÙfi ÙˆÓ Î·ÓÈÛÙÒÓ Î·Ù¿ ¿Û· Èı·ÓfiÙËÙ· ÔÊ›ÏÂÙ·È ÛÙËÓ ÂÈÙ˘¯›· Ù˘ ·ÓÙÈηÓÈÛÙÈ΋˜ ÂÎÛÙÚ·Ù›·˜, ·fi ÎÚ·ÙÈÎÔ‡˜ Î·È ¿ÏÏÔ˘˜ ÊÔÚ›˜. ∆Ô ›‰ÈÔ Ê·›ÓÂÙ·È Ó· ÈÛ¯‡ÂÈ Î·È ÛÙÔ ı¤Ì· Ù˘ Ô‰‹ÁËÛ˘ ÌÂÙ¿ ·fi ηٷӿψÛË ÔÈÓÔÓ‡̷ÙÔ˜. ¶ÈÛÙ‡ԢÌ fiÙÈ Ú¤ÂÈ Ó· ÂÓÙ·ıÔ‡Ó ÔÈ ÚÔÛ¿ıÂȘ ·fi ÙËÓ ÔÈÎÔÁ¤ÓÂÈ·, ÙÔ Û¯ÔÏÂ›Ô Î·È fiÏÔ˘˜ ÙÔ˘˜ ÊÔÚ›˜, Ô‡Ùˆ˜ ÒÛÙ ӷ ÂÈÙ¢¯ıÔ‡Ó ÔÈ ÛÙfi¯ÔÈ.

§¤ÍÂȘ ÎÏÂȉȿ: ÃÚ‹ÛË-ηٿ¯ÚËÛË ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ, ÊÔÈÙËÙ¤˜.

Use and abuse of substances by young adult students of the health professions Department of Early Childhood Education, Faculty of Health and Caring Professions, Technological Educational Institute of Athens Correspondence: Stelios Antoniadis prdrsant@otenet.gr 4, Iraklitou St., Kolonaki 106 73 Athens Greece

Paediatriki 2009;72:46-50

S. Antoniadis, C. Giavri, E. Papamacariou, K. Stavrou, M. Zerva., E. Dionyssopoulou, E. Makrogkika, V. Salonidis Abstract Introduction: Substance abuse can seriously damage health. The objective of this study was to investigate the use and abuse of substances by students of the health professions.

Methods: During the course of two semesters, 954 students of the Technological Educational Institute (TEI) of Athens were studied, according to a special protocol using a closed questionnaire.

Results: The age of the participants was 17-22 years. Regarding alcohol use, 227 (24%) students had never tried alcohol, 725 (76%) had tried once, 388 (54%) occasionally, 196 (27%) once a week, 119 (16%) 2-3 times weekly, 17 (2%) every day. The type of alcohol was: light drinks 324 (45%), heavy drinks 223 (31%), and the amount was: 1-2 drinks each time 536 (74%), 3-5 drinks 147 (20%), more than 5 drinks 31 (4%). The circumstances of drinking were: during parties 339 (47%) and on vacations 505 (81%); 400 (55%) reported getting drunk, 113 (18%) having unplanned sexual activity and 36 (5%) had been assaulted when drinking. Not driving after drinking was practiced by 848 (89%), and no accidents were reported. Tobacco had never been used by 629 (66%), 322 (34%) were smokers, 250 (78%) were

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∂Í·ÚÙËÛÈÔÁfiÓ˜ Ô˘Û›Â˜ Û ÊÔÈÙËÙ¤˜ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜

regular smokers; among smokers, 125 (39%) smoked because of the force of habit and 154 (48%) for the enjoyment, while 244 (76%) reported being aware of the harmful effects.

Among the students, 50 (5%) were drug users, specifically: marijuana 32 (64%), cocaine 12 (24%), club drugs 4 (8%), heroin 2 (4%). Among the drug users, 19 (38%) took drugs occasionally, and 17 (34%) daily; 47 (94%) knew the consequences, 16 (32%) were motivated to quit.

Conclusions: The relatively small number of smokers is most probably due to the effectiveness of the smoking cessation programs. The success of preventing driving after alcohol use is likely to be due to the information strategies that have been used. The researchers believe that students’ families and the community should try harder in order to meet the targets for reduction of substance abuse.

Key words: Substance abuse, students, health professions studies.

∂ÈÛ·ÁˆÁ‹ ∏ ¯Ú‹ÛË Î·È Î·Ù¿¯ÚËÛË ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ, fiˆ˜ ÙÔ ·ÏÎÔfiÏ, Ô Î·Ófi˜ Î·È Ù· ¿ÏÏ· ÂÍ·ÚÙËÛÈÔÁfiÓ· (ÚÔ˚fiÓÙ· οÓÓ·‚˘, ÎÔη˝ÓË, ËÚˆ›ÓË, ¯¿È· Î.Ù.Ï.), Â›Ó·È ÁÓˆÛÙfi fiÙÈ ÌÔÚ› Ó· ¤¯Ô˘Ó ÛÔ‚·Ú¤˜ ÂÈÙÒÛÂȘ ÛÙËÓ ˘Á›·. ∏ ¤Ó·ÚÍË Ù˘ ¯Ú‹Û˘ ÔÈÓÔÓ‡̷ÙÔ˜ ·fi ÙËÓ ÂÊË‚È΋ ‹ ÙËÓ ÚÒÙË Ó·ÓÈ΋ ËÏÈΛ·, ÛÂ Û˘Ó¿ÚÙËÛË Ì ÙÔ Â›‰Ô˜ ÙÔ˘ ÔÙÔ‡ ·ÏÏ¿ Î·È ÙË Û˘¯ÓfiÙËÙ· ηٷӿψÛ˘, ¤¯ÂÈ È‰È·›ÙÂÚË ÛËÌ·Û›·. ∆Ô Î¿ÓÈÛÌ· Â›Ó·È Ôχ ηϿ ÁÓˆÛÙfi fiÙÈ ÌÔÚ› Ó· ‚Ï¿„ÂÈ ÛÔ‚·Ú¿ ÙËÓ ˘Á›·, ÂÂȉ‹ fï˜ ÔÈ ‚Ï¿‚˜ ‰ÂÓ Â›Ó·È ÔÚ·Ù¤˜ ÛÙȘ ËÏÈ˘ Ô˘ ÔÈ ¿ÓıÚˆÔÈ Â›Ó·È ÊÔÈÙËÙ¤˜, ÔÏÏÔ› ‰˘ÛÎÔχÔÓÙ·È Ó· ÈÛÙ¤„Ô˘Ó ·˘Ù¿ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ȷÙÚÈο ¿ÚıÚ·, ·ÏÏ¿ Î·È ÛÙȘ ÂÊËÌÂÚ›‰Â˜, Ù· ÂÚÈÔ‰Èο, ηıÒ˜ Î·È Ù· ¿ÏÏ· ̤۷ Ì·˙È΋˜ ÂÓË̤ڈÛ˘. ∞ÓÙ›ıÂÙ·, Ë ¯Ú‹ÛË ¿ÏÏˆÓ ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ ‰ËÌÈÔ˘ÚÁ› ۈ̷ÙÈΤ˜, „˘¯ÔÏÔÁÈΤ˜, ηıÒ˜ Î·È ÎÔÈÓˆÓÈΤ˜ ÂÈÏÔΤ˜, ÔÈ Ôԛ˜ οÓÔ˘Ó ÙËÓ ÂÌÊ¿ÓÈÛ‹ ÙÔ˘˜ Ôχ ÁÚ‹ÁÔÚ·. §fiÁˆ ÙÔ˘ È·ÙÚÈÎÔ‡ ·ÏÏ¿ Î·È ÙÔ˘ ÎÔÈÓˆÓÈÎÔ‡ ÙÔ˘˜ ÂӉȷʤÚÔÓÙÔ˜, Ù· ·Ú·¿Óˆ ı· Ú¤ÂÈ Ó· ·Ó·ÁÓˆÚ›˙ÔÓÙ·È ¤ÁηÈÚ· Î·È Ó· ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È ˆ˜ ÛÔ‚·Ú¿ ÚÔ‚Ï‹Ì·Ù· ‰ËÌfiÛÈ·˜ ˘Á›·˜ (1,2). ™ÎÔfi˜ Ù˘ ÂÚÁ·Û›·˜ Ì·˜ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ¯Ú‹Û˘ Î·È Ù˘ ηٿ¯ÚËÛ˘ ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ ·fi Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ ÊÔÈÙËÙ¤˜ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ™ÙË ‰È¿ÚÎÂÈ· ÂÓfi˜ ¯ÚfiÓÔ˘, ÌÂÏÂÙ‹Û·ÌÂ, ‚¿ÛÂÈ ÚˆÙÔÎfiÏÏÔ˘ Ì ÎÏÂÈÛÙfi ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ, 954 ÊÔÈÙËÙ¤˜ Û¯ÔÏÒÓ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜ (ÓÔÛËÏ¢ÙÈ΋, Ì·È¢ÙÈ΋, Ê˘ÛÈÔıÂÚ·›·, ÂÚÁÔıÂÚ·›·, ‚ÚÂÊÔÓËÈÔÎÔÌ›·, ÂÈÛÎÂÙÒÓ ˘Á›·˜), ÙÔ˘ ∞ÓÒÙ·ÙÔ˘ ∆¯ÓÔÏÔÁÈÎÔ‡ ∂Î·È‰Â˘ÙÈÎÔ‡ π‰Ú‡Ì·ÙÔ˜ ∞ı‹Ó·˜. ∆· ·ÔÙÂϤÛÌ·Ù· Ù˘ ¤Ú¢ӷ˜ ÚԤ΢„·Ó ̤ۈ ·Ó¿Ï˘Û˘ ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ÙˆÓ ÂÚˆÙËÌ·ÙÔÏÔÁ›ˆÓ Û ËÏÂÎÙÚÔÓÈÎfi ˘ÔÏÔÁÈÛÙ‹, Ì ÙË ‚Ô‹ıÂÈ· ÙÔ˘ ÚÔÁÚ¿ÌÌ·ÙÔ˜ SPSS.

∞ÔÙÂϤÛÌ·Ù· ∏ ËÏÈΛ· Î˘Ì·ÈÓfiÙ·Ó ÌÂٷ͇ 17-22 ÂÙÒÓ, Ì ̤ÛË ËÏÈΛ· Ù· 19,5 ¯ÚfiÓÈ· (ÂÎÙfi˜ ·fi ÌÈ· ÌÈÎÚ‹ ÔÌ¿‰· ÊÔÈÙËÙÒÓ ·ÚÂÏıfiÓÙˆÓ ÂÙÒÓ, Ô˘ ›¯·Ó ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ·). ∞fi ·˘ÙÔ‡˜, ÔÈ 522 (55%) ηٿÁÔÓÙ·Ó ·fi ÙËÓ ∞ı‹Ó· Î·È ÔÈ 425 (45%) ·fi ÙËÓ ÂÚÈʤÚÂÈ·, 814 (85%) ‰‹ÏˆÛ·Ó ˆ˜ ÙfiÔ ÌfiÓÈÌ˘ ηÙÔÈΛ·˜ ÙËÓ ∞ı‹Ó· Î·È 135 (14%) ·ÛÙÈο Î·È ÂÚÈ·ÛÙÈο ΤÓÙÚ· Ù˘ ÂÚÈʤÚÂÈ·˜. ∞fi ÙÔ Û‡ÓÔÏÔ ÙˆÓ ÊÔÈÙËÙÒÓ, 910 (95%) ‹Ù·Ó ŒÏÏËÓ˜ Î·È 38 (4%) ·ÏÏÔ‰·Ô›, (6 ‰ÂÓ ·¿ÓÙËÛ·Ó). ™Â fiÙÈ ·ÊÔÚ¿ ÙÔ ÌÔÚʈÙÈÎfi Â›Â‰Ô ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘˜, 417 (44%) ‹Ù·Ó ·fiÊÔÈÙÔÈ ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ ÂÎ·›‰Â˘Û˘, 360 (38%) ÙÚÈÙÔ‚¿ıÌÈ·˜, 158 (17%) ÚˆÙÔ‚¿ıÌÈ·˜, ÂÓÒ 945 (99%) ‹Ù·Ó Ê˘ÛÈÎÔ› ÁÔÓ›˜, 55 (6%) ·fi ÙÔ˘˜ ÔÔ›Ô˘˜ ‹Ù·Ó ‰È·˙¢Á̤ÓÔÈ. √È 875 (92%) ÊÔÈÙËÙ¤˜ ›¯·Ó ¤Ó· ‹ ‰‡Ô ·‰¤ÏÊÈ·, ÂÓÒ 113 (12%) ÂÚÈÛÛfiÙÂÚ·, 927 (97%) ‹Ù·Ó ·fiÊÔÈÙÔÈ ‰ËÌfiÛÈ·˜ ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ ÂÎ·›‰Â˘Û˘ Î·È 21 (2%) ȉȈÙÈ΋˜, 385 (40%) ·Û¯ÔÏÔ‡ÓÙ·Ó Ì ÙÔÓ ·ıÏËÙÈÛÌfi, 226 (24%) Ì ÙË ÌÔ˘ÛÈ΋, 101 (11%) ·Ó¤ÊÂÚ·Ó ÂӉȷʤÚÔÓ ÁÈ· ÙȘ Ù¤¯Ó˜, ÂÓÒ 242 (22%) ‰ÂÓ ·¿ÓÙËÛ·Ó. ™Â fiÙÈ ·ÊÔÚ¿ ÙË ¯Ú‹ÛË ÔÈÓÔÓÂ˘Ì·Ùˆ‰ÒÓ ÔÙÒÓ, 725 ÊÔÈÙËÙ¤˜ (76%) ›¯·Ó ‰ÔÎÈÌ¿ÛÂÈ ¤ÛÙˆ Î·È Ì›· ÊÔÚ¿ οÔÈÔ ÔÈÓÔÓÂ˘Ì·Ù҉˜ ÔÙfi, ÂÓÒ 227 (24%) ‰ÂÓ Â›¯·Ó ÈÂÈ ÔÙ¤. ∏ ÚÒÙË Â·Ê‹ ÙÔ˘˜ Ì ÙÔ ÔÙfi ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ ÍÂÎÈÓÔ‡Û ÌÂÙ¿ ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 15 ¯ÚfiÓˆÓ [499 (69%)]. √È ÂÚÈÛÛfiÙÂÚÔÈ ·fi ·˘ÙÔ‡˜ [388 (54%)] Î·Ù·Ó¿ÏˆÓ·Ó ·ÏÎÔfiÏ ÂÚÈÛÙ·Ûȷο, ÔÈ 196 (27%) Ì›· ÊÔÚ¿ ÙËÓ Â‚‰ÔÌ¿‰·, 119 (16%) 2-3 ÊÔÚ¤˜ ÙËÓ Â‚‰ÔÌ¿‰·, 17 (2%) οı ̤ڷ. √È 324 (45%) Î·Ù·Ó¿ÏˆÓ·Ó Û˘Ó‹ıˆ˜ ÂÏ·ÊÚÈ¿ ÔÙ¿, fiˆ˜ Ì›Ú·, ÎÚ·Û› (ÂÚÈÂÎÙÈÎfiÙËÙ˜ ÔÈÓÔÓ‡̷ÙÔ˜ 5-14%) Î·È ÔÈ 223 (31%) ‚·ÚÈ¿ ÔÙ¿ Ì ˘„ËÏ‹ ÂÚÈÂÎÙÈÎfiÙËÙ· Û ÔÈÓfiÓÂ˘Ì·, fiˆ˜ Ô˘›ÛÎÈ, ‚fiÙη, Ù˙ÈÓ ¶·È‰È·ÙÚÈ΋ 2009;72:46-50

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™. ∞ÓÙˆÓÈ¿‰Ë˜ Î·È Û˘Ó.

Î.Ù.Ï. (ÂÚÈÂÎÙÈÎfiÙËÙ· ÔÈÓÔÓ‡̷ÙÔ˜ 40%), ÂÓÒ 178 (24%) ‰ÂÓ ·¿ÓÙËÛ·Ó. ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ ÔÛfiÙËÙ·, 536 (74%) ¤ÈÓ·Ó Î¿ı ÊÔÚ¿ 1-2 ÔÙ‹ÚÈ·, 147 (20%) 3-5 ÔÙ‹ÚÈ· Î·È 31 (4%) ÂÚÈÛÛfiÙÂÚ· ·fi 5. ∏ ¯Ú‹ÛË ÙÔ˘ ÔÈÓÔÓÂ˘Ì·ÙÒ‰Ô˘˜ ÁÈÓfiÙ·Ó Û˘¯ÓfiÙÂÚ· Ì ·Ú¤· [339 (47%)], Û˘Ó‹ıˆ˜ Û ʿÛË ‰È·ÛΤ‰·Û˘ Î·È ‰È·ÎÔÒÓ [505 (81%)], ÂÓÒ 63 (8%) ÊÔÈÙËÙ¤˜ ‰ÂÓ ı¤ÏËÛ·Ó Ó· ··ÓÙ‹ÛÔ˘Ó. ¶·ÚfiÏÔ Ô˘ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ‰ÂÓ ¤ÈÓ·Ó ÌfiÓÔÈ ÙÔ˘˜, 547 (75%) ‰‹ÏˆÛ·Ó fiÙÈ ‰ÂÓ ÙÔ˘˜ ·Ú¤Û˘Ú·Ó ÔÈ Û˘Ó·Ó·ÛÙÚÔʤ˜ ÙÔ˘˜. ™Â fiÙÈ ·ÊÔÚ¿ ÙȘ ÂÈÙÒÛÂȘ Ù˘ ˘ÂÚ‚ÔÏÈ΋˜ ηٷӿψÛ˘, 400 (55%) ·Ó¤ÊÂÚ·Ó Ì¤ıË Ì ·ÓÂÈı‡ÌËÙ· Â·ÎfiÏÔ˘ı·: ÙË ˙¿ÏË [260 (36%)], ÙËÓ ÎÂÊ·Ï·ÏÁ›· [196 (27%)], ÙÔÓ ÂÌÂÙfi [156 (22%)] ‹ ÛÔ‚·ÚfiÙÂÚ˜ ηٷÛÙ¿ÛÂȘ [7 (1%)]. ™Â 113 (18%) ˘‹ÚÍ ÌË ÚÔÁÚ·ÌÌ·ÙÈṲ̂ÓË ÛÂÍÔ˘·ÏÈ΋ Ú¿ÍË Î·È Û 36 (5%) ηÎÔÔ›ËÛË ·fi ¿ÏÏÔ ¿ÙÔÌÔ. ™ÙË Û˘ÓÙÚÈÙÈ΋ ÙÔ˘˜ ÏÂÈÔ„ËÊ›· [848 (89%)] ·Ó¤ÊÂÚ·Ó fiÙÈ ‰ÂÓ Ô‰ËÁÔ‡Û·Ó ˘fi ÙËÓ Â‹ÚÂÈ· ÔÈÓÔÓ‡̷ÙÔ˜ Î·È ‰ÂÓ Â›¯·Ó ÙÚÔ¯·›Ô ·Ù‡¯ËÌ·. ™ÙË Û‡ÁÎÚÈÛË ÙˆÓ Û˘ÓËıÂÈÒÓ ÙˆÓ ÊÔÈÙËÙÒÓ Ì ·˘Ù¤˜ ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘˜, ‰ÂÓ ˘‹ÚÍ·Ó ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜, ·ÊÔ‡ 64% (467) ÙˆÓ ÁÔÓ¤ˆÓ ¤Î·Ó·Ó ۯ‰fiÓ ÂÚÈÛÙ·Ûȷ΋ ¯Ú‹ÛË ÂÏ·ÊÚÒÓ ÔÈÓÔÓÂ˘Ì·Ùˆ‰ÒÓ ÔÙÒÓ, ÂÓÒ Û 118 (25%) ÂÚˆÙËÌ·ÙÔÏfiÁÈ· ‰ÂÓ ˘‹Ú¯Â ·¿ÓÙËÛË. ø˜ ÚÔ˜ ÙÔ Î¿ÓÈÛÌ·, Ê¿ÓËΠfiÙÈ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ [629 (66%)] ‰ÂÓ ¤Î·Ó·Ó ¯Ú‹ÛË Î·ÓÔ‡, Ô‡ÙÂ Î·È Â›¯·Ó ‰ÔÎÈÌ¿ÛÂÈ ÛÙÔ ·ÚÂÏıfiÓ. ∞fi ·˘ÙÔ‡˜ fï˜ Ô˘ ηÓ›˙Ô˘Ó [322 (34%)], ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ [250 (78%)] ·Ó¤ÊÂÚ·Ó fiÙÈ Â›Ó·È Û˘ÛÙËÌ·ÙÈÎÔ› ηÓÈÛÙ¤˜, Ì ËÌÂÚ‹ÛÈ· ηٷӿψÛË ÂÚÈÛÛfiÙÂÚˆÓ ÙˆÓ 10 ÙÛÈÁ¿ÚˆÓ (51%), ÂÓÒ 65 (20%) ‰‹ÏˆÛ·Ó fiÙÈ Î¿ÓÈ˙·Ó ÂÚÈÛÙ·Ûȷο. ø˜ ÚÒÙË ËÏÈΛ· ¯Ú‹Û˘ ÙÛÈÁ¿ÚˆÓ, ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi [277 (86%)] ·Ó¤ÊÂÚ fiÙÈ ¿Ú¯ÈÛ ÙÔ Î¿ÓÈÛÌ· ÌÂÙ¿ ÙËÓ ËÏÈΛ· ÙˆÓ 15 ¯ÚfiÓˆÓ, 201 (62%) ›ÛÙ¢·Ó fiÙÈ ‰ÂÓ Â›¯·Ó ·Ú·ÎÈÓËı› ·fi ÙȘ Û˘Ó·Ó·ÛÙÚÔʤ˜ ÙÔ˘˜, ·Ó Î·È ÛÙËÓ ·Ú¤· ÙÔ˘˜ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ [262 (81%)] οÓÈ˙·Ó. ∂›Û˘, Ê¿ÓËΠfiÙÈ Û˘ÓËı¤ÛÙÂÚ· ‰ÂÓ Û˘Ó‰‡·˙·Ó ÙÔ Î¿ÓÈÛÌ· Ì ·ÏÎÔfiÏË ‹ ¿ÏϘ Ô˘Û›Â˜ [177 (55%)], ÌÔÏÔÓfiÙÈ 110 (34%) ¤Î·Ó·Ó Ù·˘Ùfi¯ÚÔÓË ¯Ú‹ÛË ·ÏÎÔfiÏ Î·È 11 (3%) ¿ÏÏˆÓ Ô˘ÛÈÒÓ. ø˜ ·ÈÙ›· ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ·Ó¤ÊÂÚ·Ó ÙË ‰‡Ó·ÌË Ù˘ Û˘Ó‹ıÂÈ·˜ [125 (39%)], ÂÓÒ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÌfiÓÔ ÔÈ 154 (48%) ·Ó¤ÊÂÚ·Ó ÙËÓ ·fiÏ·˘ÛË - ÔÈ ˘fiÏÔÈÔÈ ‰ÂÓ ·¿ÓÙËÛ·Ó. ™ÙË Û‡ÁÎÚÈÛË Ù˘ Û˘Ó‹ıÂÈ·˜ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜ ÌÂٷ͇ ÙˆÓ ÊÔÈÙËÙÒÓ Î·È ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘˜, Ù· ÔÛÔÛÙ¿ ÙˆÓ Î·ÓÈÛÙÒÓ ÁÔÓ¤ˆÓ ‹Ù·Ó ¯·ÌËÏ¿ ÙfiÛÔ ÁÈ· ÙȘ ÌËÙ¤Ú˜ [120 (37%)] fiÛÔ Î·È ÁÈ· ÙÔ˘˜ ·Ù¤Ú˜ [178 (55%)]. ŸÏÔÈ ÁÓÒÚÈ˙·Ó ÙȘ ‚Ï·‚ÂÚ¤˜ Û˘Ó¤ÂȘ ÙÔ˘ ηÓ›ÛÌ·ÙÔ˜ Î·È ·Ó¤ÊÂÚ·Ó fiÙÈ Â›¯·Ó Â·Ú΋ ÂÓË̤ڈÛË [244 (76%)]. ¶·ÚfiÏ· Ù·‡Ù·, 168 (52%) ‰ÂÓ Â›¯·Ó Paediatriki 2009;72:46-50

οÓÂÈ ÚÔÛ¿ıÂȘ Ó· ‰È·Îfi„Ô˘Ó, ÂÓÒ ·fi ·˘ÙÔ‡˜ Ô˘ ›¯·Ó ÂȯÂÈÚ‹ÛÂÈ [142 (44%)] ηӤӷ˜ ‰ÂÓ Â›¯Â ˙ËÙ‹ÛÂÈ ÂÓË̤ڈÛË Î·È ‚Ô‹ıÂÈ· ·fi È·ÙÚÈΤ˜ ‹ ¿ÏϘ ËÁ¤˜. ™¯ÂÙÈο Ì ÙÔ Úfi‚ÏËÌ· Ù˘ ¯Ú‹Û˘ ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ, 50 (5%) ÊÔÈÙËÙ¤˜ ·Ó¤ÊÂÚ·Ó fiÙÈ ¤Î·Ó·Ó ¯Ú‹ÛË Î¿ÔÈ·˜ Ô˘Û›·˜, Û˘ÓËı¤ÛÙÂÚ· ÚÔ˚fiÓÙˆÓ Î¿ÓÓ·‚˘ [32 (64%)], ·ÏÏ¿ Î·È ÎÔη˝Ó˘ [12 (24%)], ¯·ÈÒÓ, LSD, Ecstasy Î.Ù.Ï. [4 (8%)] Î·È ËÚˆ›Ó˘ [2 (4%)]. ø˜ ËÏÈΛ· ÚÒÙ˘ ¯Ú‹Û˘ ·Ó·Ê¤ÚıËΠ·˘Ù‹ ÙˆÓ 15 ÂÙÒÓ [38 (76%)], ÌÂÙ¿ ·fi Û˘Ó·Ó·ÛÙÚÔʤ˜ Ì ¿ÏÏÔ˘˜ ¯Ú‹ÛÙ˜ (Ê›ÏÔ˘˜) [7(14%)], ¯ˆÚ›˜ Ó· ·Ó·ÊÂÚı› οÔÈÔ˜ Û˘ÁÎÂÎÚÈ̤ÓÔ˜ ‹ ȉȷ›ÙÂÚÔ˜ ÏfiÁÔ˜, fiˆ˜ ¿Á¯Ô˜, ηٿıÏÈ„Ë, Û˘Ó‹ıÂÈ· Î.Ï. ø˜ ÚÔ˜ ÙÔ ı¤Ì· Ù˘ Û˘¯ÓfiÙËÙ·˜ ¯Ú‹Û˘, ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ [19 (38%)] ·Ó¤ÊÂÚ·Ó fiÙÈ ‰ÔÎ›Ì·Û·Ó ÌfiÓÔ 1-2 ÊÔÚ¤˜, ÂÓÒ ˘‹Ú¯·Ó Î·È ·˘ÙÔ› Ô˘ ¤Î·Ó·Ó Û˘ÛÙËÌ·ÙÈ΋ ¯Ú‹ÛË 3-4 ÊÔÚ¤˜ ÙË ‚‰ÔÌ¿‰· [17 (34%)]. ªÔÏÔÓfiÙÈ Ë ÌÂÁ¿ÏË ÏÂÈÔ„ËÊ›· [47 (94%)] ÁÓÒÚÈ˙ ÙȘ ÔϤıÚȘ ÂÈÙÒÛÂȘ ÙˆÓ Ô˘ÛÈÒÓ ·˘ÙÒÓ ÛÙËÓ ˘Á›·, ÌfiÓÔ 16 (32%) ‰‹ÏˆÛ·Ó fiÙÈ ÂÈı˘ÌÔ‡Û·Ó Ó· ‰È·Îfi„Ô˘Ó, ÂÓÒ 7 (14%) ›¯·Ó ÙËÓ ¿Ô„Ë fiÙÈ ÔÈ ÂÍ·ÚÙËÛÈÔÁfiÓ˜ Ô˘Û›Â˜ ‰ÂÓ ÌÔÚÔ‡Û·Ó Ó· ‚Ï¿„Ô˘Ó ÙËÓ ˘Á›· ÙÔ˘˜.

™˘˙‹ÙËÛË ∞fi Ù· ·Ú·¿Óˆ Ê·›ÓÂÙ·È fiÙÈ ÂÚÈÛÛfiÙÂÚÔÈ ·fi ÙÔ˘˜ ÌÈÛÔ‡˜ ÊÔÈÙËÙ¤˜ ηٿÁÔÓÙ·Ó ·fi ÙËÓ ∞ı‹Ó·, ÛÙËÓ ÔÔ›· fï˜ ‰È¤ÌÂÓ ÌfiÓÈÌ· Ë ÏÂÈÔ„ËÊ›· (85%), οÙÈ ÙÔ ÔÔ›Ô Û›ÁÔ˘Ú· ·›˙ÂÈ ÙÔ ÚfiÏÔ ÙÔ˘, ÌÈ·˜ Î·È ÛÙȘ ÌÈÎÚfiÙÂÚ˜ ÎÔÈӈӛ˜ Ù· ¿ÓÙ· Á›ÓÔÓÙ·È ÈÔ Â‡ÎÔÏ· ÁÓˆÛÙ¿. ™¯Â‰fiÓ fiÏÔÈ (99%) ›¯·Ó Ê˘ÛÈÎÔ‡˜ ÁÔÓ›˜, ·fiÊÔÈÙÔ˘˜ ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ ‹ ÙÚÈÙÔ‚¿ıÌÈ·˜ ÂÎ·›‰Â˘Û˘ (81%), ·fi ÙÔ˘˜ ÔÔ›Ô˘˜ ÌfiÓÔ 55 (6%) ‹Ù·Ó ‰È·˙¢Á̤ÓÔÈ. ™ÙË Û˘ÓÙÚÈÙÈ΋ ÙÔ˘˜ ÏÂÈÔ„ËÊ›·, ÔÈ ÊÔÈÙËÙ¤˜ ‹Ù·Ó ·fiÊÔÈÙÔÈ ‰ËÌfiÛÈ·˜ ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ ÂÎ·›‰Â˘Û˘, ÂÓÒ ÙÔ 1/3 ›¯Â Â͈ۯÔÏÈο ÂӉȷʤÚÔÓÙ·, fiˆ˜ Ô ·ıÏËÙÈÛÌfi˜, Ë ÌÔ˘ÛÈ΋, ÔÈ Ù¤¯Ó˜. ∫¿ı ¤Ó·˜ ·fi ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ·˘ÙÔ‡˜ Û›ÁÔ˘Ú· ¤¯ÂÈ ÙË ‰È΋ ÙÔ˘ ÛËÌ·Û›·. ™ÙÔ ı¤Ì· Ù˘ ηٷӿψÛ˘ ÔÈÓÔÓ‡̷ÙÔ˜, ¤Ó·˜ ÛÙÔ˘˜ Ù¤ÛÛÂÚȘ ‰ÂÓ Â›¯Â ÈÂÈ ÔÙ¤, ÂÓÒ, ·fi ·˘ÙÔ‡˜ Ô˘ ¤ÈÓ·Ó, ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ Â›¯·Ó ·Ú¯›ÛÂÈ ÌÂÙ¿ ÙËÓ ËÏÈΛ· ÙˆÓ 15 ¯ÚfiÓˆÓ, ¯ˆÚ›˜ Ó· ¤¯Ô˘Ó ·Ú·Û˘Úı› ·fi Ê›ÏÔ˘˜. ∫·Ù·Ó¿ÏˆÓ·Ó ÂÚÈÛÙ·Ûȷο ÂÏ·ÊÚÈ¿ ÔÙ¿, ΢ڛˆ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ‰È·ÛΤ‰·Û˘ Î·È ‰È·ÎÔÒÓ, ˘‹Ú¯·Ó fï˜ Î·È 17 (2%) Ô˘ ¿ÁÁÈ˙·Ó Ù· fiÚÈ· ÙÔ˘ ·ÏÎÔÔÏÈÛÌÔ‡ (3-6). √È ÌÈÛÔ› ÂÚ›Ô˘ ·Ó¤ÊÂÚ·Ó Ì¤ıË, ¤Ó·˜ ÌÈÎÚfiÙÂÚÔ˜ ·ÚÈıÌfi˜ ·ÓÂÈı‡ÌËÙË ÛÂÍÔ˘·ÏÈ΋ Ú¿ÍË Î·È ¤Ó·˜ ·ÎfiÌ· ÌÈÎÚfiÙÂÚÔ˜ ·ÚÈıÌfi˜ [36 (5%)] ηÎÔÔ›ËÛË ·fi ¿ÏÏÔ ¿ÙÔÌÔ. ¶Ôχ ÛËÌ·ÓÙÈÎfi Â›Ó·È ÙÔ fiÙÈ ‰ÂÓ Ô‰ËÁÔ‡Û·Ó ÌÂÙ¿ ·fi ηٷӿψÛË ÔÈÓÔÓ‡̷ÙÔ˜ Î·È ‰ÂÓ Â›¯·Ó ÚÔηϤÛÂÈ

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·49

49

∂Í·ÚÙËÛÈÔÁfiÓ˜ Ô˘Û›Â˜ Û ÊÔÈÙËÙ¤˜ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜

ÙÚÔ¯·›· ·Ù˘¯‹Ì·Ù· (7,8). ™Â fiÙÈ ·ÊÔÚ¿ ÙËÓ Î·Ù·Ó¿ÏˆÛË ÔÈÓÔÓ‡̷ÙÔ˜, Ù· ‰Èο Ì·˜ ·ÔÙÂϤÛÌ·Ù· ·ÚÔ˘ÛÈ¿˙Ô˘Ó Â› Ù· ‚ÂÏÙ›ˆ ‰È·ÊÔÚ¤˜, fiˆ˜ 10% ÌÈÎÚfiÙÂÚÔ ÔÛÔÛÙfi ·˘ÙÒÓ Ô˘ ‰ÂÓ Â›¯·Ó ÔÙ¤ ηٷӷÏÒÛÂÈ ÔÈÓfiÓÂ˘Ì· Û ۇÁÎÚÈÛË Ì ÚÔËÁÔ‡ÌÂÓË ·Ó·ÛÎfiËÛË ÙˆÓ ∆ÚÈ·ÓÙ·Ê˘ÏÏ›‰Ô˘-∆ÛÔ˘Ì¿Î·˜ (5) Î·È 20% ÌÈÎÚfiÙÂÚÔ ÔÛÔÛÙfi ·fi ÙË ÛÙ·ÙÈÛÙÈ΋ ÙÔ˘ ∂∫∆∂¶¡ (∂ıÓÈÎfi ∫¤ÓÙÚÔ ∆ÂÎÌËÚ›ˆÛ˘-¶ÏËÚÔÊfiÚËÛ˘ ÁÈ· Ù· ¡·ÚΈÙÈο Î·È ÙËÓ ∆ÔÍÈÎÔÌ·Ó›·), ÂÓÒ 17% ÏÈÁfiÙÂÚÔÈ ‹Ù·Ó ·˘ÙÔ› Ô˘ ›¯·Ó ÌÂı‡ÛÂÈ. ™ËÌ·ÓÙÈÎfiÙÂÚÔ Â›Ó·È ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÛÙË ‰È΋ Ì·˜ ÌÂϤÙË, fiˆ˜ ·Ó·Ê¤ÚıËÎÂ, ηӤӷ˜ ‰ÂÓ Ô‰ËÁÔ‡Û ÌÂı˘Ṳ̂ÓÔ˜. √È ‰È·ÊÔÚ¤˜ Ì ÙÔ ·Ú·¿Óˆ ¿ÚıÚÔ Èı·Ófiٷٷ ÔÊ›ÏÔÓÙ·È ÛÙÔ fiÙÈ ÂÚÈÏ·Ì‚¿ÓÂÈ ÌÈÎÚfiÙÂÚ˜ ËÏÈ˘. ™Â fiÙÈ ·ÊÔÚ¿ ÙËÓ Â›‰Ú·ÛË ÙˆÓ Û˘ÓËıÂÈÒÓ ÙˆÓ ÁÔÓ¤ˆÓ, ·ÚfiÏÔ Ô˘ ÔÈ Malone Î·È Û˘Ó.

(7) ‰›ÓÔ˘Ó ÌÂÁ¿ÏË ÛËÌ·Û›· ÛÙËÓ ÔÈÎÔÁÂÓÂȷ΋ ηٿÛÙ·ÛË, ηıÒ˜ Î·È ÛÙËÓ Î·Ù¿¯ÚËÛË Ô˘ÛÈÒÓ ·fi ÙÔ˘˜ ÁÔÓ›˜, ÛÙȘ ‰ÈΤ˜ Ì·˜ ÂÚÈÙÒÛÂȘ ‰ÂÓ ˘‹Ú¯·Ó ÔÛÔÛÙ¿ Ô˘ ı· ÌÔÚÔ‡Û·Ó Ó· ıˆÚËıÔ‡Ó ÂÈ‚·Ú˘ÓÙÈο. ∆· Â˘Ú‹Ì·Ù¿ Ì·˜, Û ۇÁÎÚÈÛË Ì ·˘Ù¿ Ù˘ ÌÂϤÙ˘ ÙˆÓ Rimza Î·È Û˘Ó. (9), fiÔ˘ ·Ó·Ê¤ÚÂÙ·È fiÙÈ 80% ÙˆÓ ÊÔÈÙËÙÒÓ Ù˘ ÙÚÈÙÔ‚¿ıÌÈ·˜ ÂÎ·›‰Â˘Û˘ ÛÙȘ ∏¶∞ ηٷӷÏÒÓÔ˘Ó ÔÈÓfiÓÂ˘Ì·, ηıÒ˜ Î·È fiÙÈ 29% Ô‰ËÁÔ‡Ó ˘fi ÙËÓ Â‹ÚÂÈ· ÔÈÓÔÓ‡̷ÙÔ˜, ·ÔÙÂÏÒÓÙ·˜ ¤ÙÛÈ ÙËÓ ÚÒÙË ·ÈÙ›· ı·Ó¿ÙÔ˘ ÛÙȘ ËÏÈ˘ ÙˆÓ 15-24 ÂÙÒÓ, Ì·˜ οÓÔ˘Ó Ó· ·ÈÛı·ÓfiÌ·ÛÙ οˆ˜ ηχÙÂÚ·. ∆Ô ·›ÛıËÌ· ·˘Ùfi ÂÓÈÛ¯‡ÂÙ·È Î·È ·fi ¿ÏϘ ÂÚÁ·Û›Â˜, fiˆ˜ ·˘Ù‹ ÙˆÓ Noel Î·È Fromme (10) ÔÈ ÔÔ›ÔÈ, Û ÌÈ· ‰ÈÂıÓ‹ ÌÂϤÙË Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ÊÔÈÙËÙ¤˜ ·fi ÔÏϤ˜ ¯ÒÚ˜, ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÔÛÔÛÙfi ÚÔ‚ÏËÌ¿ÙˆÓ Û˘ÌÂÚÈÊÔÚ¿˜ Û 8 ÛÙÔ˘˜ 10 ÊÔÈÙËÙ¤˜ Ô˘ ηٷӷÏÒÓÔ˘Ó ÔÈÓfiÓÂ˘Ì·, ηıÒ˜ Â›Û˘ Î·È ·fi ÙËÓ ÂÚÁ·Û›· ÙˆÓ Lorente Î·È Û˘Ó. (11) Ô˘ ·Ó·Ê¤ÚÂÈ fiÙÈ 20,4% ÙˆÓ ÊÔÈÙËÙÒÓ Á˘ÌÓ·ÛÙÈ΋˜ ·Î·‰ËÌ›·˜ ÛÙË °·ÏÏ›· ·ÚÔ˘Û›·˙·Ó ÂÚÈÛÛfiÙÂÚ· ·fi 6 ÂÂÈÛfi‰È· ˘ÂÚηٷӿψÛ˘ ÔÈÓÔÓ‡̷ÙÔ˜, ÊÙ¿ÓÔÓÙ·˜ Ù· fiÚÈ· Ù˘ ÙÔÍÈ΋˜ ‰Ú¿Û˘. ™Â fiÙÈ ·ÊÔÚ¿ ÙÔ Î¿ÓÈÛÌ·, Â›Ó·È È‰È·›ÙÂÚ· ·Ú‹ÁÔÚÔ fiÙÈ ÂÚÈÛÛfiÙÂÚÔÈ ·fi ‰‡Ô ÛÙÔ˘˜ ÙÚÂȘ ÊÔÈÙËÙ¤˜ ‰ÂÓ Î¿ÓÈ˙·Ó (12,13). ªÔÏÔÓfiÙÈ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ‰ÂÓ ·Ô‰¤¯ÔÓÙ·Ó ÙËÓ Â›‰Ú·ÛË ÙˆÓ Û˘Ó·Ó·ÛÙÚÔÊÒÓ, Ë Û˘¯ÓfiÙËÙ· ÙˆÓ Î·ÓÈÛÙÒÓ Û ·˘Ù¤˜ ÍÂÂÚÓÔ‡Û ÙÔ 80% (14,15). ∆· ÔÛÔÛÙ¿ ·˘Ù¿ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÈÎÚ‹ ÌfiÓÔ (1,5%) Â› Ù· ‚ÂÏÙ›ˆ ·fiÎÏÈÛË Û ۇÁÎÚÈÛË Ì ÚÔËÁÔ‡ÌÂÓË ÂÚ¢ÓËÙÈ΋ ÂÚÁ·Û›· Ô˘ ›¯·Ì ·ÚÔ˘ÛÈ¿ÛÂÈ ÚÈÓ ·fi 4 ÂÚ›Ô˘ ¯ÚfiÓÈ· (16). ŸÌˆ˜, Â¿Ó Û˘ÁÎÚ›ÓÔ˘Ì ÙÔ ‰ÈÎfi Ì·˜ 34% Ì ÙÔ 28,5% ÙˆÓ ∏¶∞ (9) ‹ ÙÔ 15,3-27,6% Ù˘ ∫ÔÚ¤·˜, Ô˘ ·Ó·Ê¤ÚÔ˘Ó ÔÈ Ham Î·È Û˘Ó. (17), ηıÒ˜ Î·È Ì ÙȘ ÚÔÛ¿ıÂȘ Ô˘ Á›ÓÔÓÙ·È ÛÙÔÓ ∫·Ó·‰¿ [Kischuk Î·È Û˘Ó. (18)] ‹ ÛÙȘ ∏¶∞ [Staten Î·È Û˘Ó.

(19]), Ê·›ÓÂÙ·È fiÙÈ Ë ÌÈÎÚ‹ ‚ÂÏÙ›ˆÛË Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÙ·È ÛÙÔ˘˜ ‰ÈÎÔ‡˜ Ì·˜ ÊÔÈÙËÙ¤˜ ‰ÂÓ Ú¤ÂÈ Ó· Ì·˜ ÈηÓÔÔÈ›, ·ÏÏ¿ ÙÔ˘Ó·ÓÙ›ÔÓ Ó· Ì·˜ οÓÂÈ Ó· ·˘Í‹ÛÔ˘Ì ÙȘ ÚÔÛ¿ıÂȤ˜ Ì·˜ ÁÈ· ÙËÓ Î·Ï‡ÙÂÚË ÂÓË̤ڈۋ ÙÔ˘˜. ø˜ ÚÔ˜ ÙÔ Úfi‚ÏËÌ· Ù˘ ¯Ú‹Û˘ ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ, ÌÔÏÔÓfiÙÈ ÙÔ ÔÛÔÛÙfi 5% Â›Ó·È Û¯ÂÙÈο ÌÈÎÚfi, ‰ÂÓ Â›Ó·È Â˘Î·Ù·ÊÚfiÓËÙÔ, ÂÓÒ ÂÓÙ‡ˆÛË ÚÔηÏ› Î·È Ë ÌÈÎÚ‹ ËÏÈΛ· ¤Ó·Ú͢, Ô˘ ‰Â›¯ÓÂÈ ÙË ÛËÌ·Û›· ·˘ÙÔ‡ Ô˘ ·Ó·Ê¤ÚÂÈ Ô Patton (20), fiÙÈ Ë Î·Ù¿¯ÚËÛË ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ ·fi ÂÊ‹‚Ô˘˜ Â›Ó·È Ë ·È‰È·ÙÚÈ΋ ‰È¿ÁÓˆÛË Ô˘ Û˘¯ÓfiÙÂÚ· ‰È·Ê‡ÁÂÈ. √È ÂÚÈÛÛfiÙÂÚÔÈ ¯ÚËÛÈÌÔÔÈÔ‡Û·Ó ÚÔ˚fiÓÙ· οÓÓ·‚˘ ‹ ÎÔη˝ÓË (21-23), ÂÈÏÔÁ¤˜ Ô˘ Û˘ÌʈÓÔ‡Ó Ì ·˘Ù¤˜ Ô˘ ·Ó·Ê¤ÚÂÈ Ô ª·ÚÛ¤ÏÔ˜ (24) ÛÙÔ ‚È‚Ï›Ô ÙÔ˘. ∂›Û˘, ¤¯ÂÈ ÛËÌ·Û›· ˆ˜ Ë ¯Ú‹ÛË ÁÈÓfiÙ·Ó ÌÂÙ¿ ·fi Û˘Ó·Ó·ÛÙÚÔÊ‹ Ì ¯Ú‹ÛÙ˜, ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ ¯ˆÚ›˜ οÔÈÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ÏfiÁÔ, fiˆ˜ Î·È fiÙÈ ·Ó Î·È Û¯Â‰fiÓ fiÏÔÈ ÁÓÒÚÈ˙·Ó ÙȘ ÔϤıÚȘ ÂÈÙÒÛÂȘ ÛÙËÓ ˘Á›·, ÌfiÓÔ ¤Ó·˜ ÛÙÔ˘˜ ÙÚÂȘ ‰‹ÏˆÓ fiÙÈ ÂÈı˘ÌÔ‡Û ӷ ‰È·Îfi„ÂÈ, ‰›¯ˆ˜ Ó· ¤¯ÂÈ ˙ËÙ‹ÛÂÈ È·ÙÚÈ΋ ‹ ¿ÏÏË ‚Ô‹ıÂÈ·, οÙÈ Ô˘ ›Û¯˘Â Î·È ÁÈ’ ·˘ÙÔ‡˜ Ô˘ οÓÈ˙·Ó (25-26). ∆· ·Ú·¿Óˆ ÔÛÔÛÙ¿ Â›Ó·È ÂÓı·ÚÚ˘ÓÙÈο (fi¯È ÂÊËÛ˘¯·ÛÙÈο), Û ۇÁÎÚÈÛË Ì ·˘Ù¿ Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÔÈ Gledhill-Hoyt Î·È Û˘Ó. (27) ÁÈ· ÙȘ ∏¶∞, fiÔ˘ ÛÙË ÌÂϤÙË Ô˘ ÂÚÈÏ¿Ì‚·Ó 119 Û¯ÔϤ˜ ÙÚÈÙÔ‚¿ıÌÈ·˜ ÂÎ·›‰Â˘Û˘, ÙÔ ÔÛÔÛÙfi ¯ÚËÛÙÒÓ Ì·ÚÈ¯Ô˘¿Ó·˜ ¤ÊÙ·Ó ÙÔ 15,7%, οÙÈ Ô˘ ÂȂ‚·ÈÒÓÔ˘Ó Î·È ÔÈ Johnston Î·È Û˘Ó. (28), ·Ó·Ê¤ÚÔÓÙ·˜ fiÙÈ Ë Ì·ÚÈ¯Ô˘¿Ó· ·ÔÙÂÏ› ÙËÓ ÚÒÙË Û ÚÔÙ›ÌËÛË ÂÍ·ÚÙËÛÈÔÁfiÓÔ Ô˘Û›·, fiˆ˜ ·Ú·ÙËÚ‹ıËÎÂ Î·È ÛÙÔ˘˜ ÊÔÈÙËÙ¤˜ Ê·Ú̷΢ÙÈ΋˜ Ù˘ ∆Û¯›·˜, ÛÙËÓ ÂÚÁ·Û›· ÙˆÓ Kavalirova Î·È Visnovsky (29). ∆¤ÏÔ˜, ·ÚfiÏÔ Ô˘ ÁÈ· ÙÔ fiÛÔ Â›Î·ÈÚÔ Â›Ó·È ÙÔ ı¤Ì· Ù˘ ÂÚÁ·Û›·˜ ·˘Ù‹˜ ‰ÂÓ ÓÔÌ›˙Ô˘Ì ˆ˜ ˘¿Ú¯Ô˘Ó ·ÌÊÈ‚Ôϛ˜, ÈÛÙ‡ԢÌ fiÙÈ ·Í›˙ÂÈ Ó· ·Ó·ÊÂÚı› fiÙÈ ÙËÓ Ë̤ڷ Ô˘ ¤ÁÈÓ·Ó ÔÈ ÙÂÏÂ˘Ù·›Â˜ ‰ÈÔÚıÒÛÂȘ, ‰ËÌÔÛȇÙËÎ·Ó ÙÚ›· ÔÏÔÛ¤Ïȉ· ¿ÚıÚ· Ì ·ÚfiÌÔÈÔ ı¤Ì· Û ‰‡Ô ʇÏÏ· ÙÔ˘ ϤÔÓ ¤ÁÎÚÈÙÔ˘ ΢ÚȷοÙÈÎÔ˘ Ù‡Ô˘ (30-32).

™˘ÌÂÚ¿ÛÌ·Ù· ∆Ô Û¯ÂÙÈο ÌÈÎÚfiÙÂÚÔ ÔÛÔÛÙfi ÙˆÓ Î·ÓÈÛÙÒÓ Û ۯ¤ÛË Ì ÚÔËÁÔ‡ÌÂÓ˜ ÌÂϤÙ˜ ηٿ ¿Û· Èı·ÓfiÙËÙ· ÔÊ›ÏÂÙ·È ÛÙËÓ ÂÈÙ˘¯›· Ù˘ ·ÓÙÈηÓÈÛÙÈ΋˜ ÂÎÛÙÚ·Ù›·˜ Ô˘ ÂÊ·ÚÌfiÛıËΠ·fi ÎÚ·ÙÈÎÔ‡˜ Î·È ¿ÏÏÔ˘˜ ÊÔÚ›˜, ÛÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ ÂÈÎÔÛÈ¤ÓÙ ¯ÚfiÓˆÓ. ŸÌˆ˜, fiˆ˜ Ê·›ÓÂÙ·È ÛÙË Û‡ÁÎÚÈÛË Ì ٷ ÔÛÔÛÙ¿ ¿ÏÏˆÓ ¯ˆÚÒÓ, ÔÈ ÚÔÛ¿ıÂȤ˜ Ì·˜ Ú¤ÂÈ Ó· Û˘Ó¯ÈÛÙÔ‡Ó Î·È Ó· ÂÈÙ·ıÔ‡Ó. ™Â fiÙÈ ·ÊÔÚ¿ ÛÙËÓ Ô‰‹ÁËÛË ÌÂÙ¿ ·fi ηٷӿψÛË ÔÈÓÔÓ‡̷ÙÔ˜, Ê·›ÓÂÙ·È fiÙÈ Ë Û˘ÛÙËÌ·ÙÈ΋ ¶·È‰È·ÙÚÈ΋ 2009;72:46-50

Pediatric Jan-Feb 09

16-03-09

14:56

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50

™. ∞ÓÙˆÓÈ¿‰Ë˜ Î·È Û˘Ó.

ÂÓË̤ڈÛË ÁÈ· ÙÔ˘˜ Ôχ ÛÔ‚·ÚÔ‡˜ ÎÈÓ‰‡ÓÔ˘˜, Ù· Û˘¯Ó¿ ·ÏÎÔÙ¤ÛÙ, ηıÒ˜ Î·È Ë ·˘ÛÙËÚ‹ ÓÔÌÔıÂÛ›·, ›¯·Ó ηϿ ·ÔÙÂϤÛÌ·Ù·. ¶ÈÛÙ‡ԢÌ fiÙÈ ı· Ú¤ÂÈ Ó· ÂÓÙ·ıÔ‡Ó ÔÈ ÚÔÛ¿ıÂȘ ÙfiÛÔ ·fi ÙËÓ ÔÈÎÔÁ¤ÓÂÈ· fiÛÔ Î·È ·fi ÙÔ Û¯ÔÏ›Ô, fiˆ˜ Î·È ·fi fiÏÔ˘˜ ÙÔ˘˜ ÎÚ·ÙÈÎÔ‡˜ Î·È ÌË ÎÚ·ÙÈÎÔ‡˜ ÊÔÚ›˜ ˘Á›·˜, Ô‡Ùˆ˜ ÒÛÙ ӷ ÂÈÙ¢¯ı› Ô ÛÙfi¯Ô˜ Û fiÙÈ ·ÊÔÚ¿ ÙÔ ·ÏÎÔfiÏ Î·È ÙÔ Î¿ÓÈÛÌ·, ·ÏÏ¿ Î·È ÙË ¯Ú‹ÛË ¿ÏÏˆÓ ÂÍ·ÚÙËÛÈÔÁfiÓˆÓ Ô˘ÛÈÒÓ. ¢ÂÓ Ú¤ÂÈ ‰Â Ó· ‰È·Ê‡ÁÂÈ Ë ÛËÌ·Û›· ÙÔ˘ fiÙÈ ÙÔ ˘ÏÈÎfi Ù˘ ÂÚÁ·Û›·˜ Ì·˜ ·ÊÔÚ¿ ÊÔÈÙËÙ¤˜ ÙÚÈÙÔ‚¿ıÌÈ·˜ ÂÎ·›‰Â˘Û˘ Î·È Ì¿ÏÈÛÙ· Û¯ÔÏÒÓ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜.

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2. Bower AM. Are college students alcoholics? J Am Coll Health 2002;50:253-255.

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5. ∆ÚÈ·ÓÙ·Ê˘ÏÏ›‰Ô˘ ∞, ∆ÛÔ˘Ì¿Î·˜ ∫. ∞ÏÎÔfiÏ Î·È ÂÊ˂›·. ¢ÂÏÙ. ∞ã ¶·È‰È·ÙÚ. ∫ÏÈÓ. ¶·ÓÂ. ∞ıËÓÒÓ 2006;53:33-40.

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16. ∞ÓÙˆÓÈ¿‰Ë˜ ™, ∫˘ÚÈ·ÎÔÔ‡ÏÔ˘ ™, ∞ÚÁ˘ÚÔÔ‡ÏÔ˘ µ, ∫Ú›ÎÔ˜ •. µ·ıÌfi˜ ÁÓÒÛ˘ ÙˆÓ ÂÈ¤‰ˆÓ ÏÈȉ›ˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ Û ÊÔÈÙËÙ¤˜ Â·ÁÁÂÏÌ¿ÙˆÓ ˘Á›·˜- ÚfiÓÔÈ·˜. ¶·È‰È·ÙÚÈ΋ 2004;67:195-199.

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23. American Academy of Pediatrics Committee on substance abuse. A guide for health professionals. 2nd ed. Elk Grove Village (IL): American Academy of Pediatrics; 2002.

24. ª·ÚÛ¤ÏÔ˜ ª. ∂Í·ÚÙËÛÈÔÁfiÓ˜ Ô˘Û›Â˜. ∞ı‹Ó·: ∂ΉfiÛÂȘ ∆˘ˆı‹Ùˆ; 2005.

25. ∞ÓÙˆÓÈ¿‰Ë˜ ™. ¡ÙfiÈÓÁÎ Û ·È‰È¿, ÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ. ¶·È‰È·ÙÚÈ΋ 2006;69:351-356.

26. Baltimore American College Health Associasion Survey.

National College Health Assesment: reference group executive summary. Baltimore (MD): American College Health Association; 2004.

27. Gledhill-Hoyt J, Lee H, Strote S, Wechsler H. Increased use of marijuana and other illicid drugs at US colleges in the 1990s: results in three national surveys. Addiction 2000;95:1655-1657.

28. Johnston LD, O’Malley PM, Bachman JG, editors.

Monitoring the future. In: National results on adolescent drug use: overview of key findings, 2001. Bethesda (MD): National Institute on Drug Abuse; 2002. p. 1-61.

29. Kavalirova A, Visnovsky P. Use of marijuana in pharmacy students (2000-2005). Biomed Pop Med Fac Univ Palackej Olomouc Chech Repub 2005;149:477-480.

30. ∫·Ú¿Ï˘ ™. ∞Ó‹ÏÈÎÔÈ ÛÙ· ‰›¯Ù˘· ÙÔ˘ ÛÎÏËÚÔ‡ ·ÏÎÔfiÏ. ∫·ıËÌÂÚÈÓ‹ Ù˘ ∫˘Úȷ΋˜, 18/5/2008, ÛÂÏ›‰· 39.

31. º˘ÓÙ·Ó›‰Ô˘ ∂. ∂ÎÙfi˜ ÓfiÌÔ˘ ÙÔ ÙÛÈÁ¿ÚÔ. ∆Ô µ‹Ì·, ∫˘Úȷ΋ 18/5/2008, ÛÂÏ›‰· 10 32. ¡ÈÎÔÏfiÔ˘ÏÔ˜ π, ª›ÙÛÈη ¶. √È ÁÂÈÙÔÓȤ˜ Ù˘ ËÚˆ›Ó˘. ∆Ô µ‹Ì·, ∫˘Úȷ΋ 18/5/2008, ÛÂÏ›‰· 44.

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CASE REPORT

51

梉ԸԷωÔÛÙÂÚÔÓÈÛÌfi˜ Ù‡Ô˘ 1 Û ‚Ú¤ÊÔ˜ Ã. ¢·ÛηÏÔÔ‡ÏÔ˘, £. ∆ÛÂÎÔ˘Ú¿, ∞. ∆ÛÈÏÈÌÈÁοÎË ¶ÂÚ›ÏË„Ë: √ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi˜ Ù‡Ô˘ 1 Â›Ó·È ÌÈ· Û¿ÓÈ· ÎÏËÚÔÓÔÌÈ΋ ÓfiÛÔ˜ Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·ÓÙ›ÛÙ·ÛË ÛÙË ‰Ú¿ÛË Ù˘ ·Ï‰ÔÛÙÂÚfiÓ˘. ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ·ÁÔÚÈÔ‡ 37 ËÌÂÚÒÓ Ì „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi Ù‡Ô˘ 1, Ô˘ ÚÔÛ‹Ïı ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÏfiÁˆ ÌÂȈ̤Ó˘ Û›ÙÈÛ˘, ·ÓÂ·ÚÎÔ‡˜ ÚfiÛÏ˄˘ ‚¿ÚÔ˘˜, ‰˘ÛÙÚÔÊ›·˜ Î·È ·Ê˘‰¿ÙˆÛ˘. √ ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍ ˘ÔÓ·ÙÚÈ·ÈÌ›· ˘ÂÚηÏÈ·ÈÌ›·, ·˘ÍË̤ÓÔ Ó¿ÙÚÈÔ Ô‡ÚˆÓ Î·È ˘„ËϤ˜ ÙÈ̤˜ ÚÂÓ›Ó˘ Î·È ·Ï‰ÔÛÙÂÚfiÓ˘. √ ÏÔÈfi˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜, Ë ÎÚ·ÙÈÓ›ÓË, ÔÈ Î·ÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜ Î·È Ô‡ÚˆÓ, ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ, Ë ACTH, Ë ÎÔÚÙÈ˙fiÏË Î·È Ë 17-√∏ ÚÔÁÂÛÙÂÚfiÓË ‹Ù·Ó ÂÓÙfi˜ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ. √ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÁÔÓÈÒÓ ¤‰ÂÈÍ ·˘ÍË̤ÓË ÙÈÌ‹ ·Ï‰ÔÛÙÂÚfiÓ˘ ÛÙË ÌËÙ¤Ú·. ∏ ÌÔÚȷ΋ ÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ ¤‰ÂÈÍ ÙÚÂȘ ÔÏ˘ÌÔÚÊÈÛÌÔ‡˜ ÛÙÔ ÂÍfiÓÈÔ 2 ÛÙÔ ÁÔÓ›‰ÈÔ Ô˘ Έ‰ÈÎÔÔÈ› ÙÔÓ ˘Ô‰Ô¯¤· ÙˆÓ ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ, Ô Û˘Ó‰˘·ÛÌfi˜ ÙˆÓ ÔÔ›ˆÓ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÎÏÈÓÈ΋ ¤ÎÊÚ·ÛË ÙÔ˘ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌÔ‡ Ù‡Ô˘ 1.

B’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, µÂÓÈ˙¤ÏÂÈÔ °ÂÓÈÎfi ¡ÔÛÔÎÔÌ›Ô, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘ AÏÏËÏÔÁÚ·Ê›·: ∞Ì·Ï›· ∆ÛÈÏÈÌÈÁοÎË tsiliman@yahoo.gr ∏ÚÔ‰fiÙÔ˘ 214 ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘ 71601

§¤ÍÂȘ ÎÏÂȉȿ: 梉ԸԷωÔÛÙÂÚÔÓÈÛÌfi˜, ·ÒÏÂÈ· ¿Ï·ÙÔ˜, ·Ï‰ÔÛÙÂÚfiÓË, ˘ÔÓ·ÙÚÈ·ÈÌ›·, ˘ÂÚηÏÈ·ÈÌ›·, ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË, ·ÓÂ·Ú΋˜ ÚfiÛÏË„Ë ‚¿ÚÔ˘˜.

Type 1 pseudohypoaldosteronism in an infant C. Daskalopoulou, T. Tsekoura, A. Tsilimigaki Abstract: Type 1 pseudohypoaldosteronism is a rare inherited disorder characterized by insensitivity to the action of aldosterone. The case is reported of a 37 day-old boy with type 1 pseudohypoaldosteronism, admitted because of poor feeding, failure to thrive, dystrophy and dehydration. Biochemical studies revealed hyponatraemia, hyperkalaemia, increased urinary sodium concentration and increased plasma concentrations of aldosterone and renin. Serum levels of creatinine, cortisol, ACTH and 17OH progesterone, blood and urine cultures and ultrasound of the kidneys were normal. Molecular genetic studies showed three polymorphisms within the exon 2 of the mineralocorticoid receptor gene. The combination of these polymorphisms can induce the clinical expression of type 1 pseudohypoaldosterinism.

2nd Department of Paediatrics, “Venizelio” General Hospital, Heraklion, Crete Correspondence: Amalia Tsilimigaki tsiliman@yahoo.gr 214, Irodotou St. Heraklion, Crete, 71601, Greece

Key words: type 1 pseudohypoaldosteronism, salt wasting, plasma aldosterone, hyponatraemia, hyperkalaemia, metabolic acidosis, failure to thrive.

∂ÈÛ·ÁˆÁ‹ √ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi˜ Â›Ó·È ¤Ó· ÎÏÈÓÈÎfi Û‡Ó‰ÚÔÌÔ Ì ÂÙÂÚoÁ¤ÓÂÈ· Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙË ÓÂÊÚÈ΋ ‹ Î·È Û˘ÛÙËÌ·ÙÈ΋ ·ÓÙ›ÛÙ·ÛË ÛÙË ‰Ú¿ÛË Ù˘ ·Ï‰ÔÛÙÂÚfiÓ˘. √È ·Èٛ˜ ·ÓÙ›ÛÙ·Û˘ ÛÙËÓ ·Ï‰ÔÛÙÂÚfiÓË ÌÔÚ› Ó· Â›Ó·È ÚˆÙÔ·ı›˜ (ÎÏËÚÔÓÔÌÈΤ˜) ‹ ‰Â˘ÙÂÚÔ·ı›˜ (Â›ÎÙËÙ˜). √È ÚˆÙÔ·ı›˜ ÌÔÚʤ˜ ·ÔÙÂÏÔ‡ÓÙ·È ·fi ‰‡Ô ‰È·ÎÚÈÙ¿ ÎÏËÚÔÓÔÌÈο Û‡Ó‰ÚÔÌ·, ÙÔÓ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi Ù‡Ô˘ 1 (PHA 1) Î·È ÙÔÓ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi Ù‡Ô˘ 2 (PHA 2 ‹ Û‡Ó‰ÚÔÌÔ GÔrdon) (1). ∆· Û˘¯ÓfiÙÂÚ· ·›ÙÈ· ÙÔ˘ ‰Â˘ÙÂÚÔ·ıÔ‡˜ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌÔ‡ Â›Ó·È ÏÔÈÌÒÍÂȘ ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, Ì ‹ ¯ˆÚ›˜ ·ÔÊÚ·ÎÙÈΤ˜ ÓÂÊÚÈΤ˜ ‚Ï¿‚˜, Ô˘ ‰È·ÈÛÙÒÓÂÙ·È Û¿ÓÈ· Û ‚Ú¤ÊË (2).

¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ‚Ú¤ÊÔ˘˜ Ì „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi Ù‡Ô˘ 1 ÁÈ· Ó· ÙÔÓÈÛı› Ë Û·ÓÈfiÙËÙ· Î·È Ë ÛËÌ·Û›· Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ ÛÙËÓ ÚÒÙË ‚ÚÂÊÈ΋ ËÏÈΛ·.

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ AÁfiÚÈ ËÏÈΛ·˜ 37 ËÌÂÚÒÓ ÚÔÛ‹Ïı ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÏfiÁˆ ÌÂȈ̤Ó˘ Û›ÙÈÛ˘ Î·È ÛÙ·ÛÈÌfiÙËÙ·˜ ‚¿ÚÔ˘˜. ¶ÚfiÎÂÈÙ·È ÁÈ· ÙÔ ÚÒÙÔ ·È‰› Ê·ÈÓÔÙ˘Èο ˘ÁÈÒÓ ÁÔÓ¤ˆÓ, Ô˘ ÁÂÓÓ‹ıËΠ̠ηÈÛ·ÚÈ΋ ÙÔÌ‹ ÌÂÙ¿ ·fi ·ËÛË 36 ‚‰ÔÌ¿‰ˆÓ, Ì ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ 2880 g.

TË 14Ë Ë̤ڷ ˙ˆ‹˜ ÓÔÛËχÙËΠ۠ªÔÓ¿‰· ¡ÂÔÁÓÒÓ ÁÈ· ›ÎÙÂÚÔ. £‹Ï·Û ÁÈ· 14 Ë̤Ú˜ Î·È ÛÙË Û˘Ó¤¯ÂÈ· ¤Ï·‚ ÙÚÔÔÔÈË̤ÓÔ Á¿Ï·. ∆Ô ÔÈÎÔÁÂÓÂÈ·Îfi ·Ó·ÌÓËÛÙÈÎfi ·Ó·Ê¤ÚÂÙ·È ÂχıÂÚÔ. ¶·È‰È·ÙÚÈ΋ 2009;72:51-54

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Ã. ¢·ÛηÏÔÔ‡ÏÔ˘ Î·È Û˘Ó.

∆Ô ‚Ú¤ÊÔ˜ ÛÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘ ‹Ù·Ó ‰˘ÛÙÚÔÊÈÎfi, Ì ÎÏÈÓÈο Â˘Ú‹Ì·Ù· ·Ê˘‰¿ÙˆÛ˘, ı: 37ÔC, ÛʇÍÂȘ 144/min Î·È ∞¶: 85/45 mmHg. µ¿ÚÔ˜ ÂÈÛfi‰Ô˘ 2810 g (<3Ë ∂£), Ì‹ÎÔ˜ ÛÒÌ·ÙÔ˜ 53 cm (50‹ ∂£) Î·È ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜ 35 cm (10Ë-25Ë ∂£). ∞fi ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË ÙˆÓ Û˘ÛÙËÌ¿ÙˆÓ ‰ÂÓ ÚԤ΢„·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. √ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ: ÁÂÓÈ΋ ·›Ì·ÙÔ˜ Î·È TKE ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜, CRP 0,01 mg/dl, ÁÏ˘Îfi˙Ë 77 mg/dl, ÎÚ·ÙÈÓ›ÓË 0,5 mg/dl, Ô˘Ú›· 59 mg, Ê˘ÛÈÔÏÔÁÈ΋ Ë·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·, Ca 10,7 mg/dl, Na 129 mmol/l Î·È K 6,6 mmol/l/dl, Cl 97 mmol/l, ‰ÈÙÙ·ÓıÚ·ÎÈο HCO3: 18 mmol/l Î·È ¡a Ô‡ÚˆÓ: 56 mmol/l (·˘ÍË̤ÓÔ). √È Î·ÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜ Î·È Ô‡ÚˆÓ ‹Ù·Ó ÛÙ›Ú˜ Î·È ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÙˆÓ ÓÂÊÚÒÓ Ê˘ÛÈÔÏÔÁÈÎfi. ∆Ô ‚Ú¤ÊÔ˜ Ù¤ıËΠ۠ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ˘ÁÚÒÓ Î·È ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘. ∆Ș ÂfiÌÂÓ˜ Ë̤Ú˜, Ë Û›ÙÈÛ‹ ÙÔ˘ ‚ÂÏÙÈÒıËÎÂ Î·È ‰ÈÂÎfiË Ë ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ˘ÁÚÒÓ. ∆Ô 6Ô ÂÈÎÔÛÈÙÂÙÚ¿ˆÚÔ Ù˘ ÓÔÛËÏ›·˜, ÙÔ ‚Ú¤ÊÔ˜ ·ÚÔ˘Û›·Û ÂÎ Ó¤Ô˘ ÌÂȈ̤ÓË Û›ÙÈÛË, ÂÓÒ Ô ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍ ˘ÔÓ·ÙÚÈ·ÈÌ›· Î·È ˘ÂÚηÏÈ·ÈÌ›·, ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ÎÔÚÙÈ˙fiÏ˘, 17-√∏ ÚÔÁÂÛÙÂÚfiÓ˘ Î·È ACTH Ì ˘„ËϤ˜ ÙÈ̤˜ ÚÂÓ›Ó˘ >310 pg/ml (º∆ <130 pg/ml ÁÈ· ‚Ú¤ÊË) Î·È ·Ï‰ÔÛÙÂÚfiÓ˘ 16000 pg/ml (º∆ <50-900 pg/ml ÁÈ· ‚Ú¤ÊË). √ ·ÛıÂÓ‹˜ ·ÓÙÈÌÂÙˆ›ÛÙËΠ̠¯ÏˆÚÈÔ‡¯Ô Ó¿ÙÚÈÔ ·fi ÙÔ ÛÙfiÌ·, Ì ı·̷ÙÈ΋ ‚ÂÏÙ›ˆÛË ÛÙË Û›ÙÈÛË Î·È ÛÙËÓ ÚfiÛÏË„Ë ‚¿ÚÔ˘˜. √ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÁÔÓ¤ˆÓ ¤‰ÂÈÍ ·˘ÍË̤ÓË ÙÈÌ‹ ·Ï‰ÔÛÙÂÚfiÓ˘ ÛÙË ÌËÙ¤Ú· (1909 pg/ml, Ì º∆ 35-310 pg/ml ÁÈ· ÂÓ‹ÏÈΘ), ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ËÏÂÎÙÚÔÏ˘ÙÒÓ Î·È ÚÂÓ›Ó˘. ∞fi ÙË Ï‹„Ë ·Ó·Ï˘ÙÈÎfiÙÂÚÔ˘ ÈÛÙÔÚÈÎÔ‡ ÚԤ΢„ fiÙÈ Ë ÌËÙ¤Ú· ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· ›¯Â ÚÔ‚Ï‹Ì·Ù· Û›ÙÈÛ˘ Î·È ·ÓÂ·Ú΋ ÚfiÛÏË„Ë ‚¿ÚÔ˘˜, ÂÓÒ ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ù˘ ‰È·ÙÚÔÊ‹ ÚÔÛı¤ÙÂÈ ÌÂÁ¿Ï˜ ÔÛfiÙËÙ˜ ·Ï·ÙÈÔ‡. ∏ ÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË ÙÔ˘ DNA ÙÔ˘ ‚Ú¤ÊÔ˘˜ Û ΤÓÙÚÔ ÙÔ˘ Â͈ÙÂÚÈÎÔ‡ ¤‰ÂÈÍ ÙËÓ ·ÚÔ˘Û›· ÔÏ˘ÌÔÚÊÈÛÌÒÓ Û ÙÚÂȘ ÁÂÓÂÙÈÎÔ‡˜ ÙfiÔ˘˜ ÛÙÔ ÂÍfiÓÈÔ 2 ÙÔ˘ ÁÔÓȉ›Ô˘ ÙÔ˘ ˘Ô‰Ô¯¤· ÙˆÓ ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ (MR). ™˘ÓÂÛÙ‹ıË ¤ÏÂÁ¯Ô˜ Î·È ÙˆÓ ÁÔÓ¤ˆÓ ÁÈ· Ó· ‰È·ÈÛÙˆı› ·Ó ÚfiÎÂÈÙ·È ÁÈ· ÛÔÚ·‰È΋ ÌÔÚÊ‹ ‹ ÁÈ· ·˘ÙÔۈ̷ÙÈ΋ ÂÈÎÚ·ÙËÙÈ΋ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·. ∆Ô ‚Ú¤ÊÔ˜ ÂÍ‹ÏıÂ, Ì ԉËÁ›Â˜ ÁÈ· Ï‹„Ë ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ ·fi ÙÔ ÛÙfiÌ· Î·È ÁÈ· Ù·ÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË. ∂·ÓÂÎÙÈÌ‹Û·Ì ÙÔ ·È‰› Û ËÏÈΛ· 20 ÌËÓÒÓ Î·È Ù· ۈ̷ÙÔÌÂÙÚÈο ÙÔ˘ ÛÙÔȯ›· ‹Ù·Ó ‚¿ÚÔ˜ ÛÒÌ·ÙÔ˜ 11.600 g (25Ë ∂£), ‡„Ô˜ ÛÒÌ·ÙÔ˜ 85 cm (50‹ ∂£) Î·È ÂÚ›ÌÂÙÚÔ˜ ÎÂÊ·Ï‹˜ 48 cm (25Ë ∂£), ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ „˘¯ÔÎÈÓËÙÈ΋ ÂͤÏÈÍË. √ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ËÏÂÎÙÚÔÏ˘ÙÒÓ Î·È ÚÂÓ›Ó˘, ÂÓÒ Ù· Â›‰· Ù˘ ·Ï‰ÔÛÙÂÚfiÓ˘ ·Ú¤ÌÂÈÓ·Ó ˘„ËÏ¿ (4937 pg/ml, º∆ <50900 pg/ml ÁÈ· ‚Ú¤ÊË). ¢ÈÂÎfiË Ë ¯ÔÚ‹ÁËÛË ¯ÏˆÚÈPaediatriki 2009;72:51-54

Ô‡¯Ô˘ Ó·ÙÚ›Ô˘ Î·È ÙÔ ·È‰› ·Ú·Ì¤ÓÂÈ ·Û˘Ìو̷ÙÈÎfi ÌÂÙ¿ ·fi 6 Ì‹Ó˜.

™˘˙‹ÙËÛË √ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi˜ Ù‡Ô˘ 1 (PHA 1) Â›Ó·È ÌÈ· Û¿ÓÈ· ÎÏËÚÔÓÔÌÈ΋ ÓfiÛÔ˜ Ô˘ ÂΉËÏÒÓÂÙ·È Ì ·ÒÏÂÈ· Ó·ÙÚ›Ô˘ ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·, Ì Â·ÎfiÏÔ˘ı· ÙËÓ ˘ÔÓ·ÙÚÈ·ÈÌ›·, ˘ÂÚηÏÈ·ÈÌ›·, ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË, ·Ê˘‰¿ÙˆÛË Î·È ·ÓÂ·Ú΋ ·‡ÍËÛË, Ì ·˘ÍË̤ӷ Â›‰· ·Ï‰ÔÛÙÂÚfiÓ˘ Î·È ÚÂÓ›Ó˘. √ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi˜ Ù‡Ô˘ 1 ÂÚÈÁÚ¿ÊËΠÁÈ· ÚÒÙË ÊÔÚ¿ ·fi ÙÔ˘˜ Cheek Î·È Perry ÙÔ 1958 (1). ∆Ô ‚Ú¤ÊÔ˜ ‚ÂÏÙÈÒıËΠı·̷ÙÈο Ì ÙË ¯ÔÚ‹ÁËÛË ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ ·fi ÙÔ ÛÙfiÌ· ÁÈ· ¤Ó· ¤ÙÔ˜. √È Armanini Î·È Û˘Ó. ÌÂϤÙËÛ·Ó ÙËÓ ÎÏÈÓÈ΋ ÔÚ›· ÙÔ˘ ·ÓˆÙ¤Úˆ ·ÛıÂÓÔ‡˜ ÛÙËÓ ËÏÈΛ· ÙˆÓ 28 ÂÙÒÓ, ηıÒ˜ Î·È ‰‡Ô Ӥ˜ ÂÚÈÙÒÛÂȘ ·‰ÂÚÊÒÓ (8 Î·È 2 ÂÙÒÓ) Ì ·ÚfiÌÔÈ· Û˘Ìو̷ÙÔÏÔÁ›·. ∏ ·Ó¿Ï˘ÛË ÙˆÓ ÌÔÓÔ΢ÙÙ¿ÚˆÓ ÙˆÓ ·ÓˆÙ¤Úˆ ·ÛıÂÓÒÓ ¤‰ÂÈÍ ¤Ó· ¤ÏÏÂÈÌÌ· ÛÙÔÓ ˘Ô‰Ô¯¤· ÙˆÓ ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ. ™ÙȘ ·Ú·¿Óˆ ÂÚÈÙÒÛÂȘ, ÔÈ ÁÔÓ›˜ ‹Ù·Ó ·Û˘Ìو̷ÙÈÎÔ› (1,8). ¢È·ÎÚ›ÓÔÓÙ·È ‰‡Ô ÌÔÚʤ˜ ÎÏËÚÔÓÔÌÔ‡ÌÂÓÔ˘ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌÔ‡ Ù‡Ô˘ 1: Ë ·˘ÙÔۈ̷ÙÈ΋ ÂÈÎÚ·ÙËÙÈ΋ ÌÔÚÊ‹ (adPHA 1, ÓÂÊÚÈ΋ ÌÔÚÊ‹) Î·È Ë ·˘ÙÔۈ̷ÙÈ΋ ˘ÔÏÂÈfiÌÂÓË ÌÔÚÊ‹ (arPHA 1, ÔÏ˘Û˘ÛÙËÌ·ÙÈ΋ ÌÔÚÊ‹) (3-6). ∏ ÌÔÚÊ‹ Ô˘ ÎÏËÚÔÓÔÌÂ›Ù·È Ì ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ÙÚfiÔ ÔÊ›ÏÂÙ·È Û ÂÙÂÚfi˙˘Á˜ ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ Ô˘ Έ‰ÈÎÔÔÈ› ÙÔÓ ˘Ô‰Ô¯¤· ÙˆÓ ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ (NR3C2) Ô˘ ·ÊÔÚ¿ ÌfiÓÔ ÙÔ˘˜ ÓÂÊÚÔ‡˜ (4). ∆Ô ÁÔÓ›‰ÈÔ ·˘Ùfi ·ÔÙÂÏÂ›Ù·È ·fi 10 ÂÍfiÓÈ· Î·È ‚Ú›ÛÎÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 4q31.1. ∏ Û˘Ìو̷ÙÔÏÔÁ›· ÂÌÊ·Ó›˙ÂÙ·È ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·, Ì ·ÒÏÂÈ· ¿Ï·ÙÔ˜ ÛÙ· Ô‡Ú·, Â›Ó·È ‹È· Î·È ˘¿Ú¯ÂÈ ·ÓÙ·fiÎÚÈÛË ÛÙËÓ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ¯ÔÚ‹ÁËÛË ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘. ™ÙË ÌÔÚÊ‹ ·˘Ù‹, Û˘ÓÈÛÙ¿Ù·È Ë ¯ÔÚ‹ÁËÛË ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ ·fi ÙÔ ÛÙfiÌ·, Ô˘ ÌÔÚ› Ó· ‰È·ÎÔ› ÌÂÙ¿ ·fi 1 ¤ˆ˜ 3 ¯ÚfiÓÈ· ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜, ηıÒ˜ Ë Î·Ù¿ÛÙ·ÛË ‚ÂÏÙÈÒÓÂÙ·È. ∏ ·ÔηٿÛÙ·ÛË ·˘Ù‹ Û˘Ì‚·›ÓÂÈ Ì Ì˯·ÓÈÛÌÔ‡˜ Ô˘ ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ηٷÓÔËÙÔ›. ¶Èı·ÓÔÏÔÁÂ›Ù·È ·˘ÍË̤ÓË Â·Ó·ÚÚfiÊËÛË Ó·ÙÚ›Ô˘ ÛÙ· ¿ˆ ÂÛÂÈڷ̤ӷ ÛˆÏËÓ¿ÚÈ· ‹ ·Ô‰›‰ÂÙ·È Û ̷ÎÚÔ¯ÚfiÓÈ· ˘ÂÚÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ÚÂÓ›Ó˘-·ÁÁÂÈÔÙ·Û›Ó˘·Ï‰ÔÛÙÂÚfiÓ˘ (1). √ Ù‡Ô˜ ·˘Ùfi˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ‹È· ÌÔÚÊ‹, ÁÈ·Ù› Ë Û˘Ìو̷ÙÔÏÔÁ›· ˘Ô¯ˆÚ› ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹, ·ÚfiÏÔ Ô˘ ÔÈ ÙÈ̤˜ ÚÂÓ›Ó˘ Î·È ·Ï‰ÔÛÙÂÚfiÓ˘ ÌÔÚ› Ó· ·Ú·Ì¤ÓÔ˘Ó ˘„ËϤ˜ (3,6,7). ∏ ·˘ÙÔۈ̷ÙÈ΋ ˘ÔÏÂÈfiÌÂÓË ÌÔÚÊ‹ ÔÊ›ÏÂÙ·È Û ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ÁÔÓ›‰È· Ô˘ Έ‰ÈÎÔÔÈÔ‡Ó ÙÔ˘˜ ¢·›ÛıËÙÔ˘˜ ÛÙËÓ ·ÌÈÏÔÚ›‰Ë ÂÈıËÏÈ·ÎÔ‡˜ ‰È·‡ÏÔ˘˜ Ó·ÙÚ›Ô˘ (ENaC). √È ÌÂÙ·ÏÏ¿ÍÂȘ Â›Ó·È ÔÌfi˙˘Á˜ ‹

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·53

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梉ԸԷωÔÛÙÂÚÔÓÈÛÌfi˜ Ù‡Ô˘ 1 Û ‚Ú¤ÊÔ˜

Û‡ÓıÂÙ˜ ÂÙÂÚfi˙˘Á˜ ·ÓÂÓÂÚÁ¤˜ ÛÙ· ÁÔÓ›‰È· SCNN1A, SCNN1B Î·È SCNN1G (4). √È ˘ÔÌÔÓ¿‰Â˜ ·˘Ù¤˜ ÂÎÊÚ¿˙ÔÓÙ·È ÛÙ· ÂÈıËÏȷο ·ÙÙ·Ú· ÙˆÓ ÓÂÊÚÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ, Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡, ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘, ÙˆÓ ÛÈÂÏÔÁfiÓˆÓ Î·È È‰ÚˆÙÔÔÈÒÓ ·‰¤ÓˆÓ. ∏ ·Ô˘Û›· Â·Ó·ÚÚfiÊËÛ˘ Ó·ÙÚ›Ô˘ ·Ó·ÛÙ¤ÏÏÂÈ ÙË Ê˘ÛÈÔÏÔÁÈ΋ ·¤ÎÎÚÈÛË Î·Ï›Ô˘ Î·È ˘‰ÚÔÁfiÓÔ˘, Ì ·ÔÙ¤ÏÂÛÌ· ÙË ¯·Ú·ÎÙËÚÈÛÙÈ΋ ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË Î·È ÙËÓ ÂÈΛӉ˘ÓË ÁÈ· ÙË ˙ˆ‹ ˘ÂÚηÏÈ·ÈÌ›·. ∏ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ÂÈıËÏÈ·ÎÒÓ ‰È·‡ÏˆÓ Ó·ÙÚ›Ô˘ ÛÙ· Â͈ÓÂÊÚÈο ÂÈı‹ÏÈ· ÚÔηÏ› ÛÔ‚·Ú‹ ·ÒÏÂÈ· ¿Ï·ÙÔ˜ ‰È·Ì¤ÛÔ˘ ÙÔ˘ ȉÚÒÙ· Î·È ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÔÁfiÓÓÔ˘, ηıÒ˜ Î·È ¿ıÚÔÈÛË ˘ÁÚÔ‡ ÛÙȘ ·ÂÚÔÊfiÚ˜ Ô‰Ô‡˜, ÁÂÁÔÓfi˜ Ô˘ Ô‰ËÁ› ÛÂ Û˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡. √È ·ÛıÂÓ›˜ ¤¯Ô˘Ó ·Ó¿ÁÎË ·fi Û˘Ó¯‹ ¯ÔÚ‹ÁËÛË Û˘ÌÏËÚˆÌ¿ÙˆÓ ¿Ï·ÙÔ˜ ÁÈ· ÙÔÓ ¤ÏÂÁ¯Ô Ù˘ ÛÔ‚·Ú‹˜ ˘ÂÚηÏÈ·ÈÌ›·˜. ∂ÌÊ·Ó›˙ÂÙ·È Â›Û˘ ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ· Ì ÂÓÙÔÓfiÙÂÚË Û˘Ìو̷ÙÔÏÔÁ›·, ˘„ËÏ‹ ıÓËÛÈÌfiÙËÙ· Î·È ÂÈ̤ÓÂÈ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ ¯ˆÚ›˜ ȉȷ›ÙÂÚË ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· (5,6). ∞ӷʤÚÔÓÙ·È ÂÚÈÙÒÛÂȘ ÛÔÚ·‰ÈÎo‡ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌÔ‡ Ù‡Ô˘ 1 Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ‹È· Û˘Ìو̷ÙÔÏÔÁ›·, Ë ÔÔ›· ˘Ô¯ˆÚ› Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ Î·È ÙËÓ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ¯ÔÚ‹ÁËÛË Ó·ÙÚ›Ô˘ (6,7). ™Ù· ¿ÙÔÌ· ·˘Ù¿ ¤¯Ô˘Ó ‚ÚÂı› ÌÂÌÔӈ̤ÓÔÈ ÔÏ˘ÌÔÚÊÈÛÌÔ› ÛÙÔ ÁÔÓ›‰ÈÔ NR3C2 ‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÔÏ˘ÌÔÚÊÈÛÌÔ‡˜ ÛÙÔ ÁÔÓ›‰ÈÔ Ô˘ Έ‰ÈÎÔÔÈ› ÙÔ˘˜ ÂÈıËÏÈ·ÎÔ‡˜ ‰È·‡ÏÔ˘˜ Ó·ÙÚ›Ô˘ (∂¡aC) Ô˘ ÌÔÚ› Ó· ÂËÚ¿ÛÔ˘Ó ·ÚÓËÙÈο ÙËÓ Â·Ó·ÚÚfiÊËÛË ÙÔ˘ Ó·ÙÚ›Ô˘ (3,6,9,10). ∆· ¿ÙÔÌ· ·˘Ù¿ Â›Ó·È ÎÏÈÓÈο ˘ÁÈ‹, fiˆ˜ Î·È Ù· ̤ÏË Ù˘ ˘fiÏÔÈ˘ ÔÈÎÔÁ¤ÓÂÈ·˜ (7,9,10). ∂ÈϤÔÓ, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› Î·È ‰Â˘ÙÂÚÔ·ı›˜ ÌÔÚʤ˜ Ù˘ ÓfiÛÔ˘ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ·ÓˆÌ·ÏÈÒÓ ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ (1,11). ™ÙÔÓ ·ÛıÂÓ‹ Ì·˜, Ë ‰È¿ÁÓˆÛË ÙÔ˘ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌÔ‡ Ù‡Ô˘ 1 Ù¤ıËΠ·fi Ù· ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·, ÙËÓ ·Ó¿Ï˘ÛË DNA Î·È ÙÔÓ ·ÔÎÏÂÈÛÌfi ¿ÏÏˆÓ ÓfiÛˆÓ. ™Ù· Û‡Ó‰ÚÔÌ· ÛˆÏËÓ·Úȷ΋˜ ÔͤˆÛ˘ Î·È Bartter ·Ó¢ڛÛÎÂÙ·È ˘ÔηÏÈ·ÈÌ›· Î·È ˘ÂڷωÔÛÙÂÚÔÓÈÛÌfi˜. √È ‰È·Ù·Ú·¯¤˜ Ù˘ ÛÙÂÚÔÂȉÔÁ¤ÓÂÛ˘ ηÈ, ΢ڛˆ˜, Ë ·ÓÂ¿ÚÎÂÈ· Ù˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ ·ÔÎÏ›ÛÙËÎ·Ó ·fi ÙȘ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ Ù˘ ÎÔÚÙÈ˙fiÏ˘, ACTH Î·È 17-OH ÚÔÁÂÛÙÂÚfiÓ˘. √ ÂÎÏÂÎÙÈÎfi˜ ˘ԷωÔÛÙÂÚÔÓÈÛÌfi˜ Ô˘ Ú¤ÂÈ Ó· Ù›ıÂÙ·È ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ‚ÚÂÊÒÓ Ì ˘ÂÚηÏÈ·ÈÌ›· Î·È ·ÒÏÂÈ· ¿Ï·ÙÔ˜ ·ÔÎÏ›ÛÙËΠÛÙË Û˘Ó¤¯ÂÈ· ÏfiÁˆ Ù˘ ·˘ÍË̤Ó˘ ÙÈÌ‹˜ ·Ï‰ÔÛÙÂÚfiÓ˘ Ï¿ÛÌ·ÙÔ˜. √ ˘ÔÚÂÓÈÓ·ÈÌÈÎfi˜ ˘ԷωÔÛÙÂÚÔÓÈÛÌfi˜, ¿ÏÏË Ì›· ·ÈÙ›· ˘ÂÚηÏÈ·ÈÌ›·˜, ·ÔÎÏ›ÛÙËΠÏfiÁˆ ÙˆÓ ·˘ÍËÌ¤ÓˆÓ ÙÈÌÒÓ Ù˘ ·Ï‰ÔÛÙÂÚfiÓ˘ Î·È ÚÂÓ›Ó˘. ™ÙÔÓ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi

¶›Ó·Î·˜ 1. ∞ÍÈÔÏfiÁËÛË ÙˆÓ ÙÈÌÒÓ ÚÂÓ›Ó˘ Î·È ·Ï‰ÔÛÙÂÚfiÓ˘ Û ÓÂÔÁÓ¿ Î·È ‚Ú¤ÊË Ì ˘ÂÚηÏÈ·ÈÌ›· (11) ƒÂÓ›ÓË ∞ωÔÛÙÂÚfiÓË æ¢‰Ô¸ԷωÔÛÙÂÚÈÓÈÛÌfi˜ (æÀ∞) Ù‡Ô˘ 1 Î·È ·ÚÔ‰ÈÎfi˜ æÀ∞ ÀԷωÔÛÙÂÚÔÓÈÛÌfi˜ 梉ԸԷωÔÛÙÂÚÈÓÈÛÌfi˜ (æÀ∞) Ù‡Ô˘ 2 ÀÔÚÂÓÈÓ·ÈÌÈÎfi˜ ˘ԷωÔÛÙÂÚÔÓÈÛÌfi˜

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Ù‡Ô˘ 2 ‰ÂÓ ˘¿Ú¯ÂÈ ˘ÔÓ·ÙÚÈ·ÈÌ›· Î·È Ù· Â›‰· ÚÂÓ›Ó˘ Â›Ó·È ¯·ÌËÏ¿, ÂÓÒ Ù˘ ·Ï‰ÔÛÙÂÚfiÓ˘ Â›Ó·È ˘„ËÏ¿ ‹ Ê˘ÛÈÔÏÔÁÈο (¶›Ó·Î·˜ 1) (11,12). ∏ ı·̷ÙÈ΋ ·ÓÙ·fiÎÚÈÛË ÛÙË ¯ÔÚ‹ÁËÛË ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ Î·È Ë ÔÚ›· ÙÔ˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó ÂÓ‰ÂÈÎÙÈο ‹È·˜ ÌÔÚÊ‹˜ Ù˘ ÓfiÛÔ˘. √ ÁÔÓȉȷÎfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍ ÙÚÂȘ ÔÏ˘ÌÔÚÊÈÛÌÔ‡˜ ÛÙÔ ÂÍfiÓÈÔ 2, ηı¤Ó·˜ ·fi ÙÔ˘˜ ÔÔ›Ô˘˜ ·ÓÂÍ¿ÚÙËÙ· Âȉڿ ÂÏ¿¯ÈÛÙ· ÛÙË ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ˘Ô‰Ô¯¤· ÙˆÓ ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ (MR), ·ÏÏ¿ ÌÂϤÙ˜ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ Ô Û˘Ó‰˘·ÛÌfi˜ ‰‡Ô ‹ ·Ú·¿Óˆ Ù¤ÙÔÈˆÓ ÔÏ˘ÌÔÚÊÈÛÌÒÓ ÌÔÚ› Ó· ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÎÏÈÓÈ΋ ¤ÎÊÚ·ÛË ÙÔ˘ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌÔ‡ Ù‡Ô˘ 1 (7,10). ™ÙȘ ̤¯ÚÈ Û‹ÌÂÚ· ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ÔÈÎÔÁÂÓÂÈÒÓ Ì PHA 1 ÎÏËÚÔÓÔÌÔ‡ÌÂÓÔ Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ÙÚfiÔ, Ë ·ÓÙ·fiÎÚÈÛË ÙˆÓ ·ÛıÂÓÒÓ ÛÙË ¯ÔÚ‹ÁËÛË ¯ÏˆÚÈÔ‡¯Ô˘ Ó·ÙÚ›Ô˘ ·fi ÙÔ ÛÙfiÌ· ‹Ù·Ó ı·̷ÙÈ΋, ·ÚfiÏÔ Ô˘ ÔÈ ÙÈ̤˜ ·Ï‰ÔÛÙÂÚfiÓ˘ ·Ú·Ì¤ÓÔ˘Ó ˘„ËϤ˜, fiˆ˜ ÛÙÔÓ ‰ÈÎfi Ì·˜ ·ÛıÂÓ‹. ∞fi ÙÔ ÈÛÙÔÚÈÎfi ÙˆÓ ÁÔÓ¤ˆÓ ‰ÂÓ ÚÔ·ÙÂÈ ¿ÓÙÔÙ οÔÈÔ ÛÙÔÈ¯Â›Ô ·Ó¿ÏÔÁ˘ Û˘Ìو̷ÙÔÏÔÁ›·˜, ÂÓÒ ÌÂÚÈΤ˜ ÊÔÚ¤˜ Ô ¤Ó·˜ ·fi ÙÔ˘˜ ‰‡Ô ÁÔÓ›˜ ›¯Â ÚÔ‚Ï‹Ì·Ù· Û›ÙÈÛ˘ Î·È ·ÓÂ·ÚÎÔ‡˜ ·Ó¿Ù˘Í˘ ÛÙË ‚ÚÂÊÈ΋ ÙÔ˘ ËÏÈΛ·, Ô˘ ÍÂÂÚ¿ÛÙËÎ·Ó ¯ˆÚ›˜ ıÂÚ·›·. ∆· Â›‰· ·Ï‰ÔÛÙÂÚfiÓ˘ ÙˆÓ ÁÔÓ¤ˆÓ ÌÔÚ› Ó· ‚ÚÂıÔ‡Ó ˘„ËÏ¿, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ ·Ó ·ÚÔ˘Û›·Û·Ó Û˘ÌÙÒÌ·Ù· ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, ÂÓÒ Ù· Â›‰· ÚÂÓ›Ó˘ Â›Ó·È Ê˘ÛÈÔÏÔÁÈο ‹ ·˘ÍË̤ӷ (3,6,7). ™ÙËÓ ÂÚ›ÙˆÛË ÙÔ˘ ‰ÈÎÔ‡ Ì·˜ ·ÛıÂÓÔ‡˜, Ë ÌËÙ¤Ú· ›¯Â Û˘Ìو̷ÙÔÏÔÁ›· Î·È Ù· Â›‰· ·Ï‰ÔÛÙÂÚfiÓ˘ ‹Ù·Ó ˘„ËÏ¿. Œ¯ÂÈ ‚ÚÂı› fiÙÈ ÁÔÓ›˜ Ô˘ ‹Ù·Ó ÊÔÚ›˜ Ù˘ ›‰È·˜ ÌÂÙ¿ÏÏ·Í˘ Ì ÙÔ ·È‰› ÙÔ˘˜, Ì ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙËÙÈÎfi ÙÚfiÔ, ‰ÂÓ ÂΉ‹ÏˆÛ·Ó ÔÙ¤ ÎÏÈÓÈο Û˘ÌÙÒÌ·Ù·, ·ÚfiÏÔ Ô˘ ›¯·Ó ·˘ÍË̤ӷ Â›‰· ·Ï‰ÔÛÙÂÚfiÓ˘ (7). ∆Ô ÁÂÁÔÓfi˜ ·˘Ùfi ˘Ô‰ËÏÒÓÂÈ ÙËÓ Èı·ÓfiÙËÙ· Â›‰Ú·Û˘ ÂÚÈ‚·ÏÏÔÓÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ‹ ·ÎfiÌ· ÙËÓ ·ÚÔ˘Û›· ÌÂÙ·ÏÏ¿ÍÂˆÓ Î·È Û ¿ÏÏ· ÁÔÓ›‰È· Ô˘ ‰ÂÓ ¤¯Ô˘Ó ·ÎfiÌË ÌÂÏÂÙËı›. ™Â ·˘Ùfi Û˘ÓËÁÔÚ› Î·È Ë ÂÚÈÁÚ·Ê‹ ÂÚÈÙÒÛÂˆÓ ¶·È‰È·ÙÚÈ΋ 2009;72:51-54

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Ã. ¢·ÛηÏÔÔ‡ÏÔ˘ Î·È Û˘Ó.

„¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌÔ‡ Ù‡Ô˘ 1 ‹È·˜ ÌÔÚÊ‹˜ ÛÙȘ Ôԛ˜ ‰ÂÓ ‚Ú¤ıËΠη̛· ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ÁÔÓ›‰ÈÔ NR3C2 Î·È ‰Â›¯ÓÂÈ ÙË ÁÂÓÂÙÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· ÙÔ˘ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌÔ‡ Ù‡Ô˘ 1 (3,7,13). Œ¯Ô˘Ó ÂÚÈÁÚ·ÊÙ› 22 ·ÓÂÓÂÚÁ¤˜ ÌÂÙ·ÏÏ¿ÍÂȘ ·˘ÙÔ‡ ÙÔ˘ ÁÔÓȉ›Ô˘ (6). ™ÙËÓ ÂÚ›ÙˆÛË ÙÔ˘ ‰ÈÎÔ‡ Ì·˜ ·ÛıÂÓÔ‡˜ ‰ÂÓ ¤ÁÈÓ ÁÔÓȉȷÎfi˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÁÔÓ¤ˆÓ. ™˘ÌÂÚ·ÛÌ·ÙÈο, fiÙ·Ó Ô ·È‰›·ÙÚÔ˜ ·ÓÙÈÌÂÙˆ›˙ÂÈ ÓÂÔÁÓ¿ ‹ ÌÈÎÚ¿ ‚Ú¤ÊË Ì ·ÓÂ·Ú΋ ÚfiÛÏË„Ë ‚¿ÚÔ˘˜, ·Ê˘‰¿ÙˆÛË, ˘ÔÓ·ÙÚÈ·ÈÌ›·, ˘ÂÚηÏÈ·ÈÌ›·, Ô˘ ·ÔÙÂÏÔ‡Ó ÎÏÈÓÈο Â˘Ú‹Ì·Ù· ÎÔÈÓ¿ Û ÔÏÏ¿ ·È‰È·ÙÚÈο ÓÔÛ‹Ì·Ù·, ı· Ú¤ÂÈ Ó· Û˘ÌÂÚÈÏ·Ì‚¿ÓÂÈ ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÙÔÓ „¢‰Ô¸ԷωÔÛÙÂÚÔÓÈÛÌfi Ù‡Ô˘ 1, ΢ڛˆ˜ fiÙ·Ó Û˘Ó‰˘¿˙ÔÓÙ·È Ì ÓÂÊÚÈ΋ ·ÒÏÂÈ· ¿Ï·ÙÔ˜ Î·È ¤¯ÂÈ ·ÔÎÏÂÈÛÙ› Ë Û˘ÁÁÂÓ‹˜ ˘ÂÚÏ·Û›· ÂÈÓÂÊÚȉ›ˆÓ.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Proctor G, Linas S. Type 2 Pseudohypoaldosteronism: new insights into renal potassium, sodium, and chloride handling. Am J of Kidney Dis 2006;48:674-693.

2. Rodriguez-Soriano J. Tubular disorders of electrolyte regulation. In: Holiday M, Barrat M, Anver E, editors.

Pediatric nephrology. Baltimore: Williams and Wilkins; 1994. p. 631-632.

3. Viemann M, Peter M, Lopez-Siguero JP, Simic-Schleicher G, Sippel GW. Evidence for genetic heterogeneity of pseudohypoaldosteronism type 1: Identification of a novel mutation in the human mineralocorticoid receptor in one sporadic case and no mutation in two autosomal dominant kindrends. J Clin Endocrinol Metab 2001;86:2056-2059.

4. Riepe GF, Finkeldei J, Sanctis L, Einaudi S, Testa A, Karges B, et al. Elucidating the underlying molecular pathogenesis of NR3C2 mutants causing autosomal dominant pseudohypoaldosteronism type 1. J of Clin Endocrinol and Metab 2006;91:4552-4561.

Paediatriki 2009;72:51-54

5. Geller D, Zhang J, Zennaro M, Vallo-Boado A, RodriguezSoriano J, Furu L, et al. Autosomal dominant pseudohypoaldosteronism type 1: mechanisms, evidence for neonatal lethality and phenotypic expression in adults. J Am Soc Nephrol 2006;17:1429-1436.

6. Fernandes-Rosa F, Castro M, Latronico AC, Sippel W, Riepe FG, Antonini S. Recurrence of the R947X mutation in unrelated families with autosomal dominant pseudohypoaldosteronism type 1: evidence for a mutational hot spot in the mineralocorticoid receptor gene. J of Clinic Endocrinol and Metab 2006;91:3671-3675.

7. Balsamo A, Cicognari A, Gennari M, Sippell W, Menabo S, Baronio F et al. Functional characterization of naturally occurring NR3C2 gene mutations in Italian patients suffering from pseudohypoaldosterinism type 1. Eur J of Endocrinol 2007;156:249-256.

8. Armanini D, Kuhnle U, Strasser T, Dorr H, Butenandt I, Weber P, et al. Aldosterone-receptor deficiency in pseudohypoaldosteronism. N Engl J Med 1985;313:1178-1181.

9. Arai K, Zachman K, Tamotsu S, Chrousos GP. Polymorphisms of amiloride-sensitive sodium channel subunits in five sporadic cases of pseudohypoaldosteronism: Do they have pathologic potential? J of Clin Endocrinol and Metab 1999;84:2434-2437.

10. Arai K, Nakagomi Y, Iketami M, Shimura Y, Amemiya S, Ohyama K, et al. Functional polymorphisms in the mineralocorticoid receptor and amirolide-sensitive sodium channel genes in a patient with sporadic pseudohypoaldosteronism. Hum Genet 2003;112:91-97.

11. Giapros VI, Tsatsoulis AA, Drougia EE, Kollios KD, Siomou EC, Andronikou SK. Rare causes of acute hyperkalemia in the 1st week of life - Three case reports.

Pediatr Nephrol 2004;19:1046-1049.

12. Forest M. Adrenal steroid deficiency states. In: Brook C, editor. Clinical pediatric endocrinology. London: Blackwell; 1989. p. 389-390.

13. Riepe F, Holterhus PM. Exclusion of serum and glucocorticoid induced kinase (SGK1) as a candidate gene for genetically heterogeneous renal pseudohypoaldosteronism type 1 in eight families. Am J Nephr 2007;27:164-169.

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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

CASE REPORT

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π‰ÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË Û Ó·ÚÔ‡˜ ÂÊ‹‚Ô˘˜ Ì ·¯˘Û·ÚΛ· ∞. ºÈÏÈ›‰ËÅ, ™. ™ÙÂÊ·Ó¿ÎËÅ, ª. ∞Ó·ÙÔÏȈٿÎËÅ, °. µÏ·¯¿ÎËÅ, Ã. º·ÓÔ˘ÚÈ¿Î˘Ç, ∞. ∆ÛÈÏÈÌÈÁοÎËÅ ¶ÂÚ›ÏË„Ë: ∏ ȉÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË ‹ „¢‰ofiÁÎÔ˜ ÂÁÎÂÊ¿ÏÔ˘ ÂÌÊ·Ó›˙ÂÙ·È Û fiϘ ÙȘ ËÏÈ˘, ·ÏÏ¿ Â›Ó·È Û˘¯ÓfiÙÂÚË Û ·¯‡Û·ÚΘ Á˘Ó·›Î˜ ·Ó··Ú·ÁˆÁÈ΋˜ ËÏÈΛ·˜. ™ÙÔÓ ·È‰È·ÙÚÈÎfi ÏËı˘ÛÌfi ÂÌÊ·Ó›˙ÂÙ·È ·fi ÙË ‚ÚÂÊÈ΋ ¤ˆ˜ Î·È ÙËÓ ÂÊË‚È΋ ËÏÈΛ·, Ì ÌÂÁ·Ï‡ÙÂÚË Â›ÙˆÛË Û ·¯‡Û·ÚÎÔ˘˜ ÂÊ‹‚Ô˘˜. ∆· Û˘Ó‹ıË Û˘ÌÙÒÌ·Ù· Ù˘ ÓfiÛÔ˘ Â›Ó·È ÎÂÊ·Ï·ÏÁ›·, Ó·˘Ù›·, ¤ÌÂÙÔÈ Î·È ÔÊı·ÏÌÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜. ¶ÂÚÈÁÚ¿ÊÔÓÙ·È Ù¤ÛÛÂÚȘ ÂÚÈÙÒÛÂȘ ·¯‡Û·ÚÎˆÓ ‹ ˘¤Ú‚·ÚˆÓ Ó·ÚÒÓ ÂÊ‹‚ˆÓ, ËÏÈΛ·˜ ‰Ò‰Âη ¤ˆ˜ ‰ÂηÙÚÈÒÓ ÂÙÒÓ, Ì ȉÈÔ·ı‹ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË, Ô˘ ÓÔÛËχÙËÎ·Ó ÛÙËÓ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ Ù· ÙÂÏÂ˘Ù·›· ‰‡Ô ¯ÚfiÓÈ·. ∏ ‰È¿ÁÓˆÛË Ù¤ıËΠ̠‚¿ÛË Ù· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÁÈ· ÙËÓ È‰ÈÔ·ı‹ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË ÛÙ· ·È‰È¿, Ù· ÔÔ›· ‚·Û›˙ÔÓÙ·È ÛÙ· ÙÚÔÔÈË̤ӷ ÎÚÈÙ‹ÚÈ· Dandy ÁÈ· ÂÓ‹ÏÈΘ.

T· ·ÚÈ· Û˘ÌÙÒÌ·Ù· ‹Ù·Ó Ë ‰Èψ›· Î·È Ë ÎÂÊ·Ï·ÏÁ›·. ŸÏ· Ù· ·È‰È¿ Ô˘ ÌÂÏÂÙ‹ıËÎ·Ó Â›¯·Ó BMI ÌÂÁ·Ï‡ÙÂÚÔ ·fi ÙËÓ ÂÓÂÓËÎÔÛÙ‹ (90‹) ÂηÙÔÛÙÈ·›· ı¤ÛË ÁÈ· ÙËÓ ËÏÈΛ· ÙÔ˘˜. ™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ ‰È·ÈÛÙÒıËΠԛ‰ËÌ· ÔÙÈÎÒÓ ıËÏÒÓ. ∆Ô ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi ›¯Â ·˘ÍË̤ÓË ›ÂÛË Î·È ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋˜ Û‡ÛÙ·Û˘. √ Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì ̷ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÂÁÎÂÊ¿ÏÔ˘ ‰ÂÓ ·Ó¤‰ÂÈÍ ¯ˆÚÔηٷÎÙËÙÈ΋ ÂÍÂÚÁ·Û›·, ‰È¿Ù·ÛË ÙˆÓ ÎÔÈÏÈÒÓ ‹ ÊÏ‚È΋ ·ÔÊÚ·ÎÙÈ΋ ÓfiÛÔ. ∏ Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ Ì ·ÎÂÙ·˙ÔÏ·Ì›‰Ë ‹Ù·Ó ·ÔÙÂÏÂÛÌ·ÙÈ΋ Û fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ŒÓ· ¤ˆ˜ ‰‡Ô ¯ÚfiÓÈ· ÌÂÙ¿, ÔÈ ¤ÊË‚ÔÈ ·Ú·Ì¤ÓÔ˘Ó ·Û˘Ìو̷ÙÈÎÔ›. ∏ ·¯˘Û·ÚΛ· Û Ó·ÚÔ‡˜ ÂÊ‹‚Ô˘˜ ıˆÚÂ›Ù·È ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÔÏϤ˜ ÓÔÛËÚ¤˜ ηٷÛÙ¿ÛÂȘ, fiˆ˜ Ë È‰ÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË.

1 ∞’ Î·È µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, µÂÓÈ˙¤ÏÂÈÔ °ÂÓÈÎfi ¡ÔÛÔÎÔÌ›Ô, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘ 2 √Êı·ÏÌÔÏÔÁÈ΋ ∫ÏÈÓÈ΋, µÂÓÈ˙¤ÏÂÈÔ °ÂÓÈÎfi ¡ÔÛÔÎÔÌ›Ô, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘ AÏÏËÏÔÁÚ·Ê›·: ∞Ì·Ï›· ∆ÛÈÏÈÌÈÁοÎË tsilimam@yahoo.gr ∏ÚÔ‰fiÙÔ˘ 214, ∆.∫. 71601, ¡. ∞ÏÈηÚÓ·ÛÛfi˜, ∏Ú¿ÎÏÂÈÔ ∫Ú‹Ù˘

§¤ÍÂȘ ÎÏÂȉȿ: π‰ÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË, ·¯˘Û·ÚΛ·.

Idiopathic intracranial hypertension in young adolescents with obesity A. FilippidiÅ, S. StefanakiÅ, M. AnatoliotakiÅ, G. VlachakiÅ, C. FanouriakisÇ, A. TsilimigakiÅ Abstract: Idiopathic intracranial hypertension or pseudotumor cerebri appears at all ages, but it is most frequent in obese women of childbearing age. πn the paediatric population it appears from infancy to puberty, with a high incidence among obese adolescents. ∏eadache, nausea, vomiting, and ophthalmological abnormalities are common symptoms of the disease. The cases are reported of four obese or overweight young adolescents, aged 12 to 13 years, with idiopathic intracranial hypertension newly diagnosed during the last two years. The diagnosis was based on the diagnostic criteria of idiopathic intracranial hypertension for children, which were developed from the modified Dandy criteria for adults. The main symptoms were diplopia and headache. All children had body mass index (BMI) >90th percentile for age, and papilloedema was found in all cases. ∆he cerebrospinal fluid (CSF) pressure was increased and the CSF composition was normal. Neuroimaging study with magnetic resonance imaging (MRI) of the brain showed no intracranial mass lesion, ventricular dilation or venous obstructive disease. The treatment with acetazolamide was effective and all four adolescents remain asymptomatic one and two years later. Obesity in young adolescents is considered to be a risk factor for a variety of medical conditions, including idiopathic intracranial hypertension.

1 1st and 2nd Departments of Paediatrics, "Venizelio" General Hospital, Heraklion, Crete, Greece 2 Department of Ophthalmology, “Venizelio” General Hospital, Heraklion, Crete, Greece Correspondence: Amalia Tsilimigaki tsilimam@yahoo.gr 214, Irodotou St., ¡. Alikarnassos, 71601, Heraklion, Crete, Greece

Key words: Idiopathic intracranial hypertension, obesity.

™˘ÓÙÔÌÔÁڷʛ˜: π∂À √¡¶

ȉÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙËÛË

∂ÈÛ·ÁˆÁ‹ ∏ ȉÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË (π∂À) ‹ „¢‰ÔfiÁÎÔ˜ ÂÁÎÂÊ¿ÏÔ˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·˘ÍË̤ÓË ÂÓ‰ÔÎÚ¿ÓÈ· ›ÂÛË, Ë ÔÔ›· ‰ÂÓ ÔÊ›-

ÏÂÙ·È Û ˘‰ÚÔΤʷÏÔ ‹ ¯ˆÚÔηٷÎÙËÙÈ΋ ÂÍÂÚÁ·Û›· (1,2). ∆Ô ∂¡À ¤¯ÂÈ Ê˘ÛÈÔÏÔÁÈ΋ Û‡ÛÙ·ÛË Î·È ÔÈ ÎÔÈϛ˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Â›Ó·È Ê˘ÛÈÔÏÔÁÈΤ˜ (3). ∏ ‰È¿ÁÓˆÛË Ù›ıÂÙ·È Ì ‚¿ÛË Ù· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÁÈ· ÙËÓ È‰ÈÔ·ı‹ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË ÛÙ· ·È‰È¿ (4) (¶›Ó·Î·˜ 1), Ù· ÔÔ›· ‚·Û›˙ÔÓÙ·È ÛÙ· ÙÚÔÔÔÈË̤ӷ ÎÚÈÙ‹ÚÈ· Dandy. ∆· Û˘Ó‹ıË Û˘ÌÙÒÌ·Ù· Ù˘ ÓfiÛÔ˘ ¶·È‰È·ÙÚÈ΋ 2009;72:55-59

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·56

56

∞. ºÈÏÈ›‰Ë Î·È Û˘Ó.

Â›Ó·È ÎÂÊ·Ï·ÏÁ›·, Ó·˘Ù›·, ¤ÌÂÙÔÈ Î·È ÔÊı·ÏÌÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜ ¤ˆ˜ Î·È ·ÒÏÂÈ· fiÚ·Û˘ (4-8). ∏ ·¯˘Û·ÚΛ· ıˆÚÂ›Ù·È ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· π∂À ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, ȉȷ›ÙÂÚ· Û ·¯‡Û·ÚΘ Á˘Ó·›Î˜ ·Ó··Ú·ÁˆÁÈ΋˜ ËÏÈΛ·˜ (1,9,10). ™ÙÔÓ ·È‰È·ÙÚÈÎfi ÏËı˘ÛÌfi, Ë π∂À ‰ÂÓ Ê·›ÓÂÙ·È Ó· ÂÌÊ·Ó›˙ÂÙ·È Û˘¯ÓfiÙÂÚ· Û ·¯‡Û·Úη ·È‰È¿ ÚÈÓ ÙËÓ ‹‚Ë. øÛÙfiÛÔ, fiÛÔ ·˘Í¿ÓÂÈ Ë ËÏÈΛ· ÙÔ˘ ·È‰ÈÔ‡, ˘¿Ú¯ÂÈ ÌÂÁ·Ï‡ÙÂÚË Û˘Û¯¤ÙÈÛË ·¯˘Û·ÚΛ·˜ Î·È π∂À Î·È ÛÙËÓ ÂÚ›Ô‰Ô Ù˘ ÂÊ˂›·˜ Êı¿ÓÂÈ Ù· ÔÛÔÛÙ¿ ÙˆÓ ÂÓËÏ›ÎˆÓ (2). √ ÂÈÔÏ·ÛÌfi˜ Ù˘ ·¯˘Û·ÚΛ·˜ ¤¯ÂÈ Û¯Â‰fiÓ ‰ÈÏ·ÛÈ·ÛÙ› ÙȘ ‰‡Ô ÙÂÏÂ˘Ù·›Â˜ ‰ÂηÂٛ˜ ÛÙȘ ∏¶∞ Î·È ÛÙËÓ ∂˘ÚÒË, ·ÎÔÏÔ˘ıÒÓÙ·˜ ·Ú¿ÏÏËÏË ÔÚ›· Ì ÙËÓ ·˘ÍË̤ÓË Â›ÙˆÛË Ù˘ π∂À (11). ∞ÎÔÏÔ˘ı› ÂÚÈÁÚ·Ê‹ 4 ÂÚÈÙÒÛÂˆÓ ·¯‡Û·ÚÎˆÓ ‹ ˘¤Ú‚·ÚˆÓ ÂÊ‹‚ˆÓ Ì π∂À, Ô˘ ÓÔÛËχÙËÎ·Ó ÛÙËÓ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ Ù· ÙÂÏÂ˘Ù·›· 2 ¯ÚfiÓÈ·.

¶ÚÒÙË ÂÚ›ÙˆÛË ∞ÁfiÚÈ, ËÏÈΛ·˜ 13 ÂÙÒÓ, ÚÔÛ‹Ïı ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÏfiÁˆ ·Ó·ÊÂÚfiÌÂÓˆÓ ÂÂÈÛÔ‰›ˆÓ ·ÚÔ‰È΋˜ ‰Èψ›·˜ ·fi 10Ë̤ÚÔ˘ Î·È ·˘¯ÂÓ·ÏÁ›·˜. ∆Ô ·È‰› ‹Ù·Ó ˘fi ·Ú·ÎÔÏÔ‡ıËÛË ÛÙÔ È·ÙÚÂ›Ô ·¯˘Û·ÚΛ·˜ Ù˘ ÎÏÈÓÈ΋˜ Ì·˜ ·fi ¤ÙÔ˘˜. ∞fi ÙÔ ÚfiÛÊ·ÙÔ ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi ·Ó·Ê¤ÚÂÙ·È Ï‹„Ë ÛÂÙÈÚÈ˙›Ó˘, ÚÈÓ 15 Ë̤Ú˜, ÏfiÁˆ ÎÓ›‰ˆÛ˘. ∆Ô ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ‹Ù·Ó ÂχıÂÚÔ. ∆Ô ·È‰› ‹Ù·Ó ·‡ÚÂÙÔ Î·È Ë Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∞fi Ù· ˘fiÏÔÈ· Û˘ÛÙ‹Ì·Ù· ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∞¶ = 111/74 mmHg, ÛÊ›ÍÂȘ = 61/min. ∆· ۈ̷ÙÔÌÂÙÚÈο ÛÙÔȯ›· ‹Ù·Ó: µ™ = 85 kg (>97Ë ∂£), À™ = 161 cm (50‹-75Ë ∂£), BMI = 32,8 (>97Ë ∂£). ¶ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ = 119 cm (>95Ë ∂£) (12). ™Ù¿‰ÈÔ Tanner ÁÂÓÓËÙÈο fiÚÁ·Ó· Î·È ÂÊ‹‚·ÈÔ πππ. ∞fi ÙËÓ ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËÎÂ: Ô›‰ËÌ· ÔÙÈÎÒÓ ıËÏÒÓ, Ì ·Û¿ÊÂÈ· ÙˆÓ ÔÚ›ˆÓ ÙÔ˘ ÔÙÈÎÔ‡ Ó‡ÚÔ˘, ÂÍȉڈ̷ÙÈο ÛÙÔȯ›· ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ Ô›ÛıÈÔ˘ fiÏÔ˘, ηıÒ˜ Â›Û˘ Î·È ÌÈÎÚÔ·ÈÌÔÚÚ·Á›Â˜ ‰ÂÍÈÔ‡ Î·È ·ÚÈÛÙÂÚÔ‡ ÔÊı·ÏÌÔ‡. ∂›Û˘, ÛËÌÂÈÒıËΠ·‰˘Ó·Ì›· ÚÔÛ‰ÈÔÚÈÛÌÔ‡ ÊÏ‚ÈÎÔ‡ ÛÊ˘ÁÌÔ‡. ∞fi ÙËÓ ÂͤٷÛË ÙˆÓ ÔÙÈÎÒÓ ‰›ˆÓ ‰È·ÈÛÙÒıËΠ‰È‡ڢÓÛË Ù˘ÊÏ‹˜ ÎËÏ›‰·˜ ·ÌÊfiÙÂÚˆÓ ÙˆÓ ÔÊı·ÏÌÒÓ. ∏ ÔÙÈ΋ Ô͇ÙËÙ·, Ù· ÚfiÛıÈ· ÌfiÚÈ·, Ô ÎÂÚ·ÙÔÂȉ‹˜ Î·È Ù· ·ÓÙ·Ó·ÎÏ·ÛÙÈο Ù˘ ÎfiÚ˘ ‹Ù·Ó ηٿ ʇÛË. ∂Ï‹ÊıË Ï‹Ú˘ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ (ÁÂÓÈ΋ ·›Ì·ÙÔ˜, ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜, ÁÂÓÈ΋ Ô‡ÚˆÓ, ÏÈ›‰È·, ÏÈÔÚˆÙ½Ó˜, ÔÚÌfiÓ˜ ı˘ÚÂÔÂȉԇ˜, ACTH, ÎÔÚÙÈ˙fiÏË, ÈÓÛÔ˘Ï›ÓË), Ô˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. ∆Ô ·È‰› ˘Ô‚Ï‹ıËΠ۠MRI ÂÁÎÂÊ¿ÏÔ˘, Ô˘ ‰ÂÓ ·Ó¤‰ÂÈÍ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ŒÁÈÓ √¡¶ Ô˘ ¤‰ÂÈÍÂ: ›ÂÛË ∂¡À 870 mm H2O, ÏÂ˘Î¿ = 0/mmÑ, ۿί·ÚÔ Paediatriki 2009;72:55-59

= 78 mg/dl Î·È Ï‡Έ̷ = 20 mg/dl. ªÂÙ¿ ÙËÓ √¡¶, Ô ·ÛıÂÓ‹˜ ‰ÂÓ ·Ó¤ÊÂÚ ϤÔÓ ‰Èψ›·, ÏËÓ 2 ÂÂÈÛÔ‰›ˆÓ ‚Ú·¯Â›·˜ ‰È¿ÚÎÂÈ·˜. ªÂ ÙË ‰È¿ÁÓˆÛË π∂À, Ô ·ÛıÂÓ‹˜ ÂÙ¤ıË Û ıÂÚ·›· Ì ·ÎÂÙ·˙ÔÏ·Ì›‰Ë Ì ‰fiÛË 750 mg/24h. ∂Í‹Ïı ÌÂÙ¿ ·fi 6 Ë̤Ú˜ ÓÔÛËÏ›·˜, Ì ʷÚ̷΢ÙÈ΋ ·ÁˆÁ‹ ·ÎÂÙ·˙ÔÏ·Ì›‰Ë Î·È Î¿ÏÈÔ. ™˘ÓÂÛÙ‹ıË Ù·ÎÙÈ΋ ÔÊı·ÏÌÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË Î·È ‰fiıËÎ·Ó Ô‰ËÁ›Â˜ ‰È·ÙÚÔÊ‹˜ ÁÈ· ·ÒÏÂÈ· ‚¿ÚÔ˘˜. ªÂÙ¿ ·fi 2 Ì‹Ó˜, ÙÔ Ô›‰ËÌ· ÙˆÓ ÔÙÈÎÒÓ ıËÏÒÓ ˘Ô¯ÒÚËÛÂ Î·È ‰ÈÂÎfiË Ë ıÂÚ·›·. ∆Ô ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜ ›¯Â ÂÏ·ÙÙˆı› ηٿ 4 kg. ¢‡Ô ¯ÚfiÓÈ· ÌÂÙ¿, ÙÔ ·È‰› Â›Ó·È ·Û˘Ìو̷ÙÈÎfi, Ì ÂÏ·Ùو̤ÓÔ ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜ Î·È Û ηϋ ÁÂÓÈ΋ ηٿÛÙ·ÛË.

¢Â‡ÙÂÚË ÂÚ›ÙˆÛË ∫ÔÚ›ÙÛÈ, ËÏÈΛ·˜ 13 ÂÙÒÓ, ÚÔÛ‹Ïı ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÏfiÁˆ ‰Èψ›·˜ Î·È ÂÌ¤ÙˆÓ ·fi 24ÒÚÔ˘, ηıÒ˜ Î·È ÎÂÊ·Ï·ÏÁ›·˜ ·fi 15Ë̤ÚÔ˘. ∆Ô ·È‰› ‹Ù·Ó ·‡ÚÂÙo Î·È Ë Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∞¶ = 120/63 mmHg, ÛÊ›ÍÂȘ = 75/min. ∆· ۈ̷ÙÔÌÂÙÚÈο ÙÔ˘ ÛÙÔȯ›· ‹Ù·Ó: µ™ = 69 kg (90‹-97Ë ∂£), À™ = 157 cm (50‹ ∂£), BMI = 28 (90‹-97Ë ∂£). ¶ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ = 95 cm (>95Ë ∂£) (12). ™Ù¿‰ÈÔ Tanner ÛÙ‹ıÔ˜ Î·È ÂÊ‹‚·ÈÔ πππ. ∞fi ÙËÓ ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠԛ‰ËÌ· ÔÙÈÎÒÓ ıËÏÒÓ, Ì ·ÈÌÔÚÚ·ÁÈο ÛÙÔȯ›· ¿Ìʈ. ∏ ÔÙÈ΋ Ô͇ÙËÙ·, Ù· ÚfiÛıÈ· ÌfiÚÈ·, Ô ÎÂÚ·ÙÔÂȉ‹˜ Î·È Ù· ·ÓÙ·Ó·ÎÏ·ÛÙÈο Ù˘ ÎfiÚ˘ ‹Ù·Ó ηٿ ʇÛË. ∂Ï‹ÊıË Ï‹Ú˘ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜, Ô˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. ŒÁÈÓ MRI ÂÁÎÂÊ¿ÏÔ˘, Ë ÔÔ›· ¤‰ÂÈÍ ÂÏ·ÊÚ¿ ‰È‡ڢÓÛË ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ˘·Ú·¯ÓÔÂȉԇ˜ ¯ÒÚÔ˘, ¯ˆÚ›˜ ÛËÌ›· ˘‰ÚÔÎÂÊ¿ÏÔ˘ ‹ ¯ˆÚÔηٷÎÙËÙÈ΋˜ ‚Ï¿‚˘. ∏ √¡¶ ¤‰ÂÈÍÂ: ›ÂÛË ∂¡À = 420 mm H2O, ÏÂ˘Î¿ = 3/mmÑ, ۿί·ÚÔ = 68 mg/dl Î·È Ï‡Έ̷ = 22,5 mg/dl. ∏ ·ÛıÂÓ‹˜ ÂÙ¤ıË Û ıÂÚ·›· Ì ·ÎÂÙ·˙ÔÏ·Ì›‰Ë Ì ‰fiÛË 500 mg/24h Î·È ·ÚÔ˘Û›·Û ‡ÊÂÛË ÙˆÓ Û˘Ìو̿وÓ. ∆Ô 8Ô 24ˆÚÔ ÓÔÛËÏ›·˜ ˘‹ÚÍ ۷ʋ˜ ‚ÂÏÙ›ˆÛË Ù˘ ÂÈÎfiÓ·˜ ÙˆÓ ÔÙÈÎÒÓ ıËÏÒÓ, Ì ·ÔÚÚfiÊËÛË ÙˆÓ ·ÈÌÔÚÚ·ÁÈÒÓ. ∏ ·ÛıÂÓ‹˜ ÂÍ‹Ïı Ì ·ÁˆÁ‹ ·ÎÂÙ·˙ÔÏ·Ì›‰Ë Î·È ‰È·ÈÙËÙÈΤ˜ Ô‰ËÁ›Â˜ ÁÈ· ·ÒÏÂÈ· ‚¿ÚÔ˘˜. ∆Ô Ô›‰ËÌ· ÙˆÓ ÔÙÈÎÒÓ ıËÏÒÓ ˘Ô¯ÒÚËÛÂ Î·È ‰ÈÂÎfiË Ë ıÂÚ·›· 2 Ì‹Ó˜ ÌÂÙ¿. ∆Ô ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜ ›¯Â ÂÏ·ÙÙˆı› ηٿ 3 kg. ŒÓ· ¯ÚfiÓÔ ÌÂÙ¿, ÙÔ ·È‰› Â›Ó·È ·Û˘Ìو̷ÙÈÎfi. ∆Ú›ÙË ÂÚ›ÙˆÛË ∞ÁfiÚÈ, ËÏÈΛ·˜ 13 ÂÙÒÓ, ÚÔÛ‹Ïı ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÏfiÁˆ ‰Èψ›·˜ ·fi 10Ë̤ÚÔ˘ Î·È ÎÂÊ·Ï·ÏÁ›·˜ ·fi 24ÒÚÔ˘. ∏ Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∞fi Ù· ˘fiÏÔÈ· Û˘ÛÙ‹Ì·Ù· ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∞¶ = 102/63 mmHg,

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·57

57

π‰ÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË Î·È ·¯˘Û·ÚΛ·

¶›Ó·Î·˜ 1. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÁÈ· ÙËÓ È‰ÈÔ·ı‹ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË (4) 1. ™˘ÌÙÒÌ·Ù· ‹/Î·È ÛËÌ›· ·˘ÍË̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›ÂÛ˘, Ì ԛ‰ËÌ· ÔÙÈÎÒÓ ıËÏÒÓ Û ·ÛıÂÓ‹ ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi Â›Â‰Ô Û˘Ó›‰ËÛ˘ 2. º˘ÛÈÔÏÔÁÈ΋ Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË, ÂÎÙfi˜ ·fi „¢‰Ò˜ ÂÛÙȷο Â˘Ú‹Ì·Ù·, fiˆ˜ ¿ÚÂÛË ÙÔ˘ ··ÁˆÁÔ‡ ‹ ÙÔ˘ ÚÔÛˆÈÎÔ‡ Ó‡ÚÔ˘ 3. ∞˘ÍË̤ÓË ÂÓ‰ÔÎÚ¿ÓÈ· ›ÂÛË (>250 mm H2O), Ì ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙËÛË Û Ï¿ÁÈ· ηٷÎÂÎÏÈ̤ÓË ı¤ÛË Ì ¤ÎÙ·ÛË ÙˆÓ Î¿Ùˆ ¿ÎÚˆÓ 4. º˘ÛÈÔÏÔÁÈ΋˜ Û‡ÛÙ·Û˘ ∂¡À 5. º˘ÛÈÔÏÔÁÈ΋ ·ÂÈÎfiÓÈÛË ÂÁÎÂÊ¿ÏÔ˘ ÛÙȘ Ó¢ÚÔ·ÂÈÎÔÓÈÛÙÈΤ˜ ÌÂıfi‰Ô˘˜ (·Ô˘Û›· ÂÓ‰ÔÎÚ¿ÓÈ·˜ Ì¿˙·˜, ˘‰ÚÔÎÂÊ¿ÏÔ˘, ÂÁÎÂÊ·ÏÈ΋˜ ÊÏ‚È΋˜ ıÚfiÌ‚ˆÛ˘) 6. ∫·Ì›· ¿ÏÏË ·ÈÙ›· ·˘ÍË̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›ÂÛ˘

ÛÊ›ÍÂȘ = 80/min, ıÂÚÌÔÎÚ·Û›· = 36,6ÔC. ∆· ۈ̷ÙÔÌÂÙÚÈο ÛÙÔȯ›· ‹Ù·Ó: µ™ = 76 kg (90‹-97Ë ∂£), À™ = 161 cm (50‹-75Ë ∂£), BMI = 29,3 (90‹-97Ë ∂£). ¶ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ = 89 cm (90‹ ∂£) (12). ™Ù¿‰ÈÔ Tanner ÁÂÓÓËÙÈο fiÚÁ·Ó· Î·È ÂÊ‹‚·ÈÔ πππ. ∞fi ÙËÓ ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠ‰Èψ›· ÛÙËÓ ÚˆÙÂ‡Ô˘Û· ‚ÏÂÌÌ·ÙÈ΋ ı¤ÛË Î·È ·Ú·Ï˘ÙÈÎfi˜ ÛÙÚ·‚ÈÛÌfi˜, ÏfiÁˆ ¿ÚÂÛ˘ ÙÔ˘ ··ÁˆÁÔ‡ Ó‡ÚÔ˘ ·ÚÈÛÙÂÚ¿. ∏ ÔÙÈ΋ Ô͇ÙËÙ·, Ù· ÚfiÛıÈ· ÌfiÚÈ·, Ô ÎÂÚ·ÙÔÂȉ‹˜ Î·È Ù· ·ÓÙ·Ó·ÎÏ·ÛÙÈο Ù˘ ÎfiÚ˘ ‹Ù·Ó ηٿ ʇÛË. H ‚˘ıÔÛÎfiËÛË ¤‰ÂÈÍ ԛ‰ËÌ· ÔÙÈÎÒÓ ıËÏÒÓ ¿Ìʈ. ∂Ï‹ÊıË Ï‹Ú˘ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ Î·È ¤ÁÈÓ MRI ÂÁÎÂÊ¿ÏÔ˘, Ô˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. ∏ √¡¶ ¤‰ÂÈÍÂ: ›ÂÛË ∂¡À 280 mm H2O, ÏÂ˘Î¿ = 5/mmÑ, ۿί·ÚÔ = 65 mg/dl Î·È Ï‡Έ̷ = 25 mg/dl. O ·ÛıÂÓ‹˜ ÂÙ¤ıË Û ıÂÚ·›· Ì ·ÎÂÙ·˙ÔÏ·Ì›‰Ë Ì ‰fiÛË 500 mg/24h. ªÂÙ¿ ÙËÓ √¡¶ ·ÚÔ˘Û›·Û ‚ÂÏÙ›ˆÛË Ù˘ ‰Èψ›·˜. ∂Í‹Ïı ÌÂÙ¿ ·fi 7 Ë̤Ú˜ ÓÔÛËÏ›·˜, Ì ·ÁˆÁ‹ ·ÎÂÙ·˙ÔÏ·Ì›‰Ë Î·È ‰È·ÈÙËÙÈΤ˜ Ô‰ËÁ›Â˜. ∆Ô Ô›‰ËÌ· ÙˆÓ ÔÙÈÎÒÓ ıËÏÒÓ ˘Ô¯ÒÚËÛ 1 Ì‹Ó· ÌÂÙ¿ Î·È Ë Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ ‰È·ÎfiËÎÂ. ∆Ô ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜ ›¯Â ÂÏ·ÙÙˆı› ηٿ 5 kg. ŒÓ· ¯ÚfiÓÔ ÌÂÙ¿ ·Ú·Ì¤ÓÂÈ ·Û˘Ìو̷ÙÈÎfi˜ Î·È Ì ÛËÌ·ÓÙÈ΋ Ì›ˆÛË ÙÔ˘ ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜.

∆¤Ù·ÚÙË ÂÚ›ÙˆÛË ∞ÁfiÚÈ, ËÏÈΛ·˜ 12 ÂÙÒÓ, ÚÔÛ‹Ïı ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÏfiÁˆ ‰Èψ›·˜ ·fi 7Ë̤ÚÔ˘. ∆Ô ·È‰› ‹Ù·Ó ·‡ÚÂÙÔ Î·È Ë Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∞¶ = 114/80 mmHg, ÛÊ›ÍÂȘ = 75/min. ∆· ۈ̷ÙÔÌÂÙÚÈο ÛÙÔȯ›· ‹Ù·Ó Ù· ÂÍ‹˜: µ™ = 52 kg (75Ë ∂£), À™ = 142 cm (10Ë ∂£), BMI = 25,8 (90‹-97Ë ∂£). ¶ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ = 85 cm (90‹ ∂£) (12). ™Ù¿‰ÈÔ Tanner ÁÂÓÓËÙÈο fiÚÁ·Ó· Î·È ÂÊ‹‚·ÈÔ πππ. To ÚÔÂÍ¿Ú¯ÔÓ Û‡Ìو̷ ‹Ù·Ó Ë ‰Èψ›·, Ë ÔÔ›· ÂÈÙÂÈÓfiÙ·Ó ÛÙËÓ ·ÚÈÛÙÂÚ‹ Ï¿ÁÈ·

‚ÏÂÌÌ·ÙÈ΋ ı¤ÛË. ∞fi ÙËÓ ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠԛ‰ËÌ· ÔÙÈÎÒÓ ıËÏÒÓ. ™ÙËÓ MRI ÂÁÎÂÊ¿ÏÔ˘ ‰ÂÓ ˘‹Ú¯·Ó ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∏ √¡¶ ¤‰ÂÈÍÂ: ›ÂÛË ∂¡À 420 mm H2O, ÏÂ˘Î¿ = 0/mmÑ, ۿί·ÚÔ = 70 mg/dl Î·È Ï‡Έ̷ = 30 mg/dl. O ·ÛıÂÓ‹˜ ÂÙ¤ıË Û ıÂÚ·›· Ì ·ÎÂÙ·˙ÔÏ·Ì›‰Ë Ì ‰fiÛË 500 mg/24h. ∏ ‰Èψ›· ˘Ô¯ÒÚËÛÂ. ∆Ô ·È‰› ÂÍ‹Ïı ÌÂÙ¿ ·fi 6 Ë̤Ú˜ ÓÔÛËÏ›·˜ Ì ʷÚ̷΢ÙÈ΋ ·ÁˆÁ‹ ·ÎÂÙ·˙ÔÏ·Ì›‰Ë Î·È Î¿ÏÈÔ. ∂›Û˘, ‰fiıËÎ·Ó ‰È·ÈÙËÙÈΤ˜ Ô‰ËÁ›Â˜. TÔ Ô›‰ËÌ· ÙˆÓ ÔÙÈÎÒÓ ıËÏÒÓ ˘Ô¯ÒÚËÛ ÌÂÙ¿ 2 Ì‹Ó˜ Î·È ¤ÁÈÓ ‰È·ÎÔ‹ Ù˘ ·ÎÂÙ·˙ÔÏ·Ì›‰Ë˜. ∆Ô ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜ ›¯Â ÂÏ·ÙÙˆı› ηٿ 3 kg. ŒÓ· ¯ÚfiÓÔ ÌÂÙ¿ ÙËÓ ¤ÍÔ‰fi ÙÔ˘, ÙÔ ·È‰› ·Ú·Ì¤ÓÂÈ ·Û˘Ìو̷ÙÈÎfi.

™˘˙‹ÙËÛË ∏ π∂À Â›Ó·È ÌÈ· Û¯ÂÙÈο Û˘¯Ó‹ ÓfiÛÔ˜, Ì ÂÙ‹ÛÈ· Â›ÙˆÛË 1-2 ÂÚÈÙÒÛÂȘ ·Ó¿ 100.000 ÛÙÔÓ ÁÂÓÈÎfi ÏËı˘ÛÌfi. ∏ ÓfiÛÔ˜ ÂÌÊ·Ó›˙ÂÙ·È Û fiϘ ÙȘ ËÏÈ˘, ·ÏÏ¿ Â›Ó·È Û˘¯ÓfiÙÂÚË Û ·¯‡Û·ÚΘ Á˘Ó·›Î˜ ·Ó··Ú·ÁˆÁÈ΋˜ ËÏÈΛ·˜. ∏ ηٷÓÔÌ‹ Â›Ó·È ·ÚfiÌÔÈ· ÛÙ· ‰‡Ô ʇϷ ÛÙËÓ ÚÔË‚È΋ ÂÚ›Ô‰Ô, ·ÏÏ¿ ÛÙËÓ ÂÊ˂›· ·Ú·ÙËÚÂ›Ù·È ¯·Ú·ÎÙËÚÈÛÙÈ΋ ˘ÂÚÔ¯‹ ÙˆÓ ·¯‡Û·ÚÎˆÓ ÎÔÚÈÙÛÈÒÓ (5). ¶ÔÏÏÔ› ·ÈÙÈÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜ ¤¯Ô˘Ó Û˘Û¯ÂÙÈÛı› Ì π∂À (3,4). √ÚÈṲ̂ÓÔÈ ·fi ·˘ÙÔ‡˜ Â›Ó·È Î·Ï¿ ÙÂÎÌËÚȈ̤ÓÔÈ Î·È ¿ÏÏÔÈ ·Ó·Ê¤ÚÔÓÙ·È Û ÌÈÎÚ¤˜ ÛÂÈÚ¤˜ ‹ Û ÌÂÌÔӈ̤ӷ ÂÚÈÛÙ·ÙÈο. ∏ ·¯˘Û·ÚΛ· ıˆÚÂ›Ù·È ÚԉȷıÂÛÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÁÈ· π∂À, ·Ó Î·È Ô ÚfiÏÔ˜ Ù˘ ·Ú·Ì¤ÓÂÈ ¿ÁÓˆÛÙÔ˜. ¶Èı·ÓfiÓ Ô‰ËÁ› Û ·˘ÍË̤ÓË ÂÓ‰ÔÎÔÈÏȷ΋ ›ÂÛË, Ë ÔÔ›· ·˘Í¿ÓÂÈ ·ÓÙ›ÛÙÔȯ· ÙËÓ ÂÓ‰ÔıˆÚ·ÎÈ΋ Î·È ÙËÓ ÂÓ‰ÔÂÁÎÂÊ·ÏÈ΋ ÊÏ‚È΋ ›ÂÛË (4). ªÂÙ·‚ÔÏÈÎÔ› Î·È ÔÚÌÔÓÈÎÔ› ·Ú¿ÁÔÓÙ˜, ÔÈ ÔÔ›ÔÈ ˘Ê›ÛÙ·ÓÙ·È ¤ÓÙÔÓ˜ ·ÏÏ·Á¤˜ Ì ÙËÓ ¤Ó·ÚÍË Ù˘ ‹‚˘ Î·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÊ˂›·˜, Èı·ÓfiÓ Ó· ¢ı‡ÓÔÓÙ·È ÁÈ· ÙË Û˘¯ÓfiÙÂÚË ÂÌÊ¿ÓÈÛË π∂À Û ·¯‡Û·ÚÎÔ˘˜ Ó·ÚÔ‡˜ ¤ÊË‚Ô˘˜ (10). ∞fi ÙÔ˘˜ Genizi J Î·È Û˘Ó. (13) ¤ÁÈÓ ÌÂÙ·Ó¿Ï˘ÛË Û 244 ÂÚÈÙÒÛÂȘ ·È‰ÈÒÓ Ì π∂À Ô˘ ¤¯Ô˘Ó ·Ó·ÊÂÚı› ·fi ÙÔ 1996 ¤ˆ˜ ÙÔ 2006. ¢ÈÂÚ¢ӋıËÎÂ Ë Î·Ù·ÓÔÌ‹ ÙÔ˘ ʇÏÔ˘ Î·È Ë Û˘¯ÓfiÙËÙ· ·¯˘Û·ÚΛ·˜ ÌÂٷ͇ Ù˘ ÔÌ¿‰·˜ ·È‰ÈÒÓ ¤ˆ˜ 11 ÂÙÒÓ Î·È Ù˘ ÔÌ¿‰·˜ ÂÊ‹‚ˆÓ 12-17 ÂÙÒÓ. ¢È·ÈÛÙÒıËΠfiÙÈ Ë π∂À ÂÌÊ·Ó›˙ÂÙ·È ÛÙ· ·È‰È¿ ¤ˆ˜ 11 ÂÙÒÓ Ì ·ÚfiÌÔÈ· ηٷÓÔÌ‹ ʇÏÔ˘, ÂÓÒ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ ÂÌÊ·Ó›˙ÂÙ·È Û˘¯ÓfiÙÂÚ· Û ·¯‡Û·Úη ÎÔÚ›ÙÛÈ·. ™Â 147 ·ÛıÂÓ›˜ ‰ÈÂÚ¢ӋıËÎÂ Ë ·¯˘Û·ÚΛ·. ™Â 74 ·È‰È¿ ËÏÈΛ·˜ ¤ˆ˜ 11 ÂÙÒÓ, ÌfiÓÔ 19 (26%) ‹Ù·Ó ·¯‡Û·Úη, ÂÓÒ Û 73 ÂÊ‹‚Ô˘˜ ÔÈ 47 (64%) ‹Ù·Ó ·¯‡Û·ÚÎÔÈ. ™Â ÌÂϤÙË ÙˆÓ Balcer LJ Î·È Û˘Ó. (10) Û 40 ·È‰È¿ ËÏÈΛ·˜ 3-18 ÂÙÒÓ Ì π∂À, 81% ‹Ù·Ó ·¯‡Û·Úη ÛÙȘ ËÏÈ˘ 12-14 ÂÙÒÓ Î·È 91% ÛÙȘ ËÏÈ˘ 15-17 ÂÙÒÓ. ¶·È‰È·ÙÚÈ΋ 2009;72:55-59

Pediatric Jan-Feb 09

16-03-09

14:56

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∞. ºÈÏÈ›‰Ë Î·È Û˘Ó.

™Â ÌÂϤÙË ÙˆÓ Youroukos S Î·È Û˘Ó. (14) Û 36 ·È‰È¿ Ì π∂À ËÏÈΛ·˜ 3,5 ÌËÓÒÓ ¤ˆ˜ 14 ÂÙÒÓ, ·Ó¢ڤıË Ë ·ÈÙ›· Ù˘ ÓfiÛÔ˘ Û 28 (77,7%) ·È‰È¿, Ì ‰Â‡ÙÂÚÔ ÛÂ Û˘¯ÓfiÙËÙ· ÚԉȷıÂÛÈÎfi ·Ú¿ÁÔÓÙ· ÙËÓ ·¯˘Û·ÚΛ·. ™ÙË ÌÂϤÙË ÙˆÓ Kesler A Î·È Û˘Ó. (2) Û 27 ·ÛıÂÓ›˜ ËÏÈΛ·˜ ≤16 ÂÙÒÓ Ì π∂À, 72% ‹Ù·Ó ·¯‡Û·ÚÎÔÈ fiÛÔÓ ·ÊÔÚ¿ ÙÔ˘˜ ÂÊ‹‚Ô˘˜. ∞˘Ù¿ Ù· ·ÔÙÂϤÛÌ·Ù· Û˘ÌʈÓÔ‡Ó Ì ٷ ‰Â‰Ô̤ӷ Ù˘ ÚfiÛÊ·Ù˘ ·Ó¿Ï˘Û˘ fiÏˆÓ ÙˆÓ ÌÂÏÂÙÒÓ ÁÈ· π∂À Û ·È‰È¿ (13). ™Â ÌÂϤÙË ÙˆÓ Daniels A Î·È Û˘Ó. ·Ú·ÙËÚ‹ıËΠfiÙÈ ·ÎfiÌË Î·È ÌÈÎÚ‹ ·‡ÍËÛË ÙÔ˘ ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜ (515%) ·˘Í¿ÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· π∂À (11). ∆· Û˘Ó‹ıË Û˘ÌÙÒÌ·Ù· Ù˘ ÓfiÛÔ˘ Â›Ó·È ÎÂÊ·Ï·ÏÁ›·, Ó·˘Ù›·, ¤ÌÂÙÔÈ Î·È ÔÊı·ÏÌÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜ (1-4,9). ∏ ÎÂÊ·Ï·ÏÁ›· ÂÓÙÔ›˙ÂÙ·È Û˘Ó‹ıˆ˜ ÌÂÙˆÈ·›·, ÂȉÂÈÓÒÓÂÙ·È Ì ÙËÓ Î·Ù¿ÎÏÈÛË Î·È ÌÔÚ› Ó· ·Ê˘Ó›˙ÂÈ ÙÔ ·È‰›. (9). ™Â ÈÔ ÌÈÎÚ¿ ·È‰È¿, Ù· ÚÔÂÍ¿Ú¯ÔÓÙ· Û˘ÌÙÒÌ·Ù· Â›Ó·È ˘ÓËÏ›·, ÓˆıÚfiÙËÙ· ‹ ·ÓËÛ˘¯›· (8). ∞fi ÙËÓ ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË Û˘Ó‹ıˆ˜ ·ÚÔ˘ÛÈ¿˙ÂÙ·È ‰Èψ›·, ÏfiÁˆ ¿ÚÂÛ˘ Ù˘ VI ÂÁÎÂÊ·ÏÈ΋˜ Û˘˙˘Á›·˜, Î·È ‰È·Ù·Ú·¯¤˜ ÔÙÈÎÒÓ ‰›ˆÓ (8,9). ∂›Û˘, ÌÔÚ› Ó· ·Ú·ÙËÚËı› ʈÙÔÊÔ‚›·, ÂÛˆÙÚÔ›· ηÈ, Û ·È‰È¿ ÚÈÓ ÙËÓ ‹‚Ë, ÛÙÚ·‚ÈÛÌfi˜ (9,15). ∏ ·ÒÏÂÈ· fiÚ·Û˘ ·ÔÙÂÏ› ÛÔ‚·Ú‹ ÂÈÏÔ΋ (4,9). ™˘¯Ófi ‡ÚËÌ· ·ÔÙÂÏ› ÙÔ Ô›‰ËÌ· ÙˆÓ ÔÙÈÎÒÓ ıËÏÒÓ, ÙÔ ÔÔ›Ô ÌÔÚ› Ó· Â›Ó·È ÂÙÂÚfiÏ¢ÚÔ ‹ ·ÌÊÔÙÂÚfiÏ¢ÚÔ (1,9,10). ∆Ô ‚Ú¤ÊÔ˜ ¯ˆÚ›˜ Û‡ÁÎÏÂÈÛË Ú·ÊÒÓ ÌÔÚ› Ó· ÌËÓ ·ÚÔ˘ÛÈ¿ÛÂÈ Ô›‰ËÌ· ÔÙÈÎÒÓ ıËÏÒÓ, fiÙ·Ó Ë ·‡ÍËÛË Ù˘ ÂÓ‰ÔÎÚ·Óȷ΋˜ ›ÂÛ˘ Â›Ó·È ‹È· (16). ™·ÓÈfiÙÂÚ· Û˘ÌÙÒÌ·Ù· Ù˘ π∂À ·ÔÙÂÏÔ‡Ó Ë ·˘¯ÂÓ·ÏÁ›·, ÙÔ Ú·È‚fiÎÚ·ÓÔ, ÙÔ ¿ÏÁÔ˜ ˆÌÈ΋˜ ˙ÒÓ˘, ÔÈ Û·ÛÌÔ›, Ô Ó˘ÛÙ·ÁÌfi˜ Î·È Ë ·ÓˆÙÚÔ›·, ÏfiÁˆ ¿ÚÂÛ˘ IV ÂÁÎÂÊ·ÏÈ΋˜ Û˘˙˘Á›·˜ (6,7,9,13). ∏ ıÂÚ·›· Ù˘ π∂À Â›Ó·È Û˘Ìو̷ÙÈ΋, Ì ÛÙfi¯Ô ÙËÓ ÂÏ¿ÙÙˆÛË Ù˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›ÂÛ˘. ∏ ·ÎÂÙ·˙ÔÏ·Ì›‰Ë, ˆ˜ ·Ó·ÛÙÔϤ·˜ Ù˘ ηڂÔÓÈ΋˜ ·Ó˘‰Ú¿Û˘, ÂÏ·ÙÙÒÓÂÈ ÙËÓ ·Ú·ÁˆÁ‹ ∂¡À Î·È ¯ÚËÛÈÌÔÔÈÂ›Ù·È ÛÙË ıÂÚ·›· Ù˘ ÓfiÛÔ˘ ˆ˜ Ê¿ÚÌ·ÎÔ ÚÒÙ˘ ÂÎÏÔÁ‹˜. ™Â ·ÛıÂÓ›˜ Ô˘ Ï·Ì‚¿ÓÔ˘Ó ·ÎÂÙ·˙ÔÏ·Ì›‰Ë ¿Óˆ ·fi 6 Ì‹Ó˜, Â›Ó·È ··Ú·›ÙËÙÔ˜ Ô ¤ÏÂÁ¯Ô˜ ÁÈ· Èı·Ó‹ ÓÂÊÚ·Û‚¤ÛÙˆÛË. ∂¿Ó Ù· Û˘ÌÙÒÌ·Ù· ·Ú·Ì¤ÓÔ˘Ó ÌÂÙ¿ ÙË ıÂÚ·›· Ì ·ÎÂÙ·˙ÔÏ·Ì›‰Ë ÁÈ· Ì›· ‚‰ÔÌ¿‰·, ÙfiÙ ÌÔÚ› Ó· Á›ÓÂÈ Â·Ó·ÏËÙÈ΋ √¡¶ (9,13,16). º·Ú̷΢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ì ÛÙÂÚÔÂȉ‹, fiˆ˜ ‰ÂÍ·ÌÂı·˙fiÓË ‹ Ú‰ÓÈ˙ÔÏfiÓË, ¤¯ÂÈ ¤Ó‰ÂÈÍË Û ÌË ·ÓÙ·fiÎÚÈÛË ‹ ‰˘Û·ÓÂÍ›· ÛÙËÓ ·ÎÂÙ·˙ÔÏ·Ì›‰Ë, ηıÒ˜ Â›Û˘ Î·È Û ÛÔ‚·Úfi Ô›‰ËÌ· ÔÙÈÎÒÓ ıËÏÒÓ. ∂Ó·ÏÏ·ÎÙÈο, ÌÔÚ› Ó· ÚÔÛÙÂı› ÛÙË Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ Ë ÊÔ˘ÚÔÛÂÌ›‰Ë (4,9,13,16). ÃÂÈÚÔ˘ÚÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË ¤¯ÂÈ ¤Ó‰ÂÈÍË Û ÚÔԉ¢ÙÈ΋ ‹ Ôχ ÛÔ‚·Ú‹ ÔÙÈ΋ Ó¢ÚÔ¿ıÂÈ· Î·È Û Paediatriki 2009;72:55-59

ÂÈ̤ÓÔ˘Û· ÎÂÊ·Ï·ÏÁ›· Ô˘ ÂËÚ¿˙ÂÈ ÙËÓ Î·ıËÌÂÚÈÓ‹ ‰Ú·ÛÙËÚÈfiÙËÙ·, ·Ú¿ ÙË Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹. ¶ÂÚÈÏ·Ì‚¿ÓÂÈ ÎÔÈÏÈÔÂÚÈÙÔÓ·˚΋ ·ÚÔ¯¤Ù¢ÛË Î·È ‰ËÌÈÔ˘ÚÁ›· ·Ú·ı‡ÚÔ˘ ÛÙÔ ¤Ï˘ÙÚÔ ÙÔ˘ ÔÙÈÎÔ‡ Ó‡ÚÔ˘ (4,9,13,16). ∏ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘ Â›Ó·È Î·Ï‡ÙÂÚË ÛÙ· ·È‰È¿ Û ۯ¤ÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ Î·È Ë ·˘ÙfiÌ·ÙË ˘Ô¯ÒÚËÛË Û˘ÓËı¤ÛÙÂÚË (1,2). √È ÁÔÓ›˜ Ú¤ÂÈ Ó· ÂÓËÌÂÚÒÓÔÓÙ·È fiÙÈ ÌÔÚ› Ó· ˘¿ÚÍÂÈ Ó¤Ô ÂÂÈÛfi‰ÈÔ π∂À Ì‹Ó˜ ‹ ¤ÙË ÌÂÙ¿ ÙÔ ÚÒÙÔ. ∞ÛıÂÓ›˜ Ì ˘ÔÙÚÔ‹ Ù˘ π∂À Ú¤ÂÈ Ó· ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È ˆ˜ Ó¤· ÂÚÈÛÙ·ÙÈο (9). ™ËÌ·ÓÙÈÎfi ‚‹Ì· ÛÙË ıÂÚ·›· Ù˘ ÓfiÛÔ˘ ·ÔÙÂÏ› Ë ·ÒÏÂÈ· ‚¿ÚÔ˘˜ Û ·¯‡Û·Úη ‹ ˘¤Ú‚·Ú· ·È‰È¿ (17). ∞ÎfiÌË Î·È ÌÈÎÚ‹ ·ÒÏÂÈ· ‚¿ÚÔ˘˜ ÌÔÚ› Ó· ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ˘Ô¯ÒÚËÛË ÙÔ˘ Ôȉ‹Ì·ÙÔ˜ ÔÙÈ΋˜ ıËÏ‹˜ (9,11). ¶ÚfiÁÚ·ÌÌ· ‰›·ÈÙ·˜ Ì ·ÒÏÂÈ· ‚¿ÚÔ˘˜ ≥2,5 kg ÂÓÙfi˜ 3 ÌËÓÒÓ ¤¯ÂÈ Û˘Û¯ÂÙÈÛı› Ì ٷ¯‡ÙÂÚË ‚ÂÏÙ›ˆÛË ÙÔ˘ Ôȉ‹Ì·ÙÔ˜ ÔÙÈ΋˜ ıËÏ‹˜ Û ·ÛıÂÓ›˜ Ì π∂À (5). ™Â ÔÚÈṲ̂Ó˜ ÂÚÈÙÒÛÂȘ Ó·ÓÈ΋˜ ·¯˘Û·ÚΛ·˜, fiÔ˘ ˘¿Ú¯ÂÈ ÛÔ‚·Ú‹ ÓÔÛËÚfiÙËÙ· (۷ί·Ú҉˘ ‰È·‚‹Ù˘ Ù‡Ô˘ ππ, ·ÔÊÚ·ÎÙÈ΋ ˘ÓÈ΋ ¿ÓÔÈ·, π∂À) Î·È BMI ≥40, Û˘ÓÈÛÙ¿Ù·È ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ·¯˘Û·ÚΛ·˜ (18). ∆· ·È‰È¿ Ô˘ ÌÂÏÂÙ‹ıËÎ·Ó ‹Ù·Ó ÛÙËÓ ÂÊ˂›· Î·È ‹Ù·Ó ·¯‡Û·Úη ‹ ˘¤Ú‚·Ú·. ∏ ˘ÂÚÔ¯‹ ÙˆÓ ·ÁÔÚÈÒÓ (3:1) ‰ÂÓ ·ÍÈÔÏÔÁ›ٷÈ, ÏfiÁˆ ÙÔ˘ ÌÈÎÚÔ‡ ·ÚÈıÌÔ‡. ∆Ô ·˘ÍË̤ÓÔ ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜ ıˆڋıËÎÂ Ô Î‡ÚÈÔ˜ ÚԉȷıÂÛÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÁÈ· ÙËÓ ÂΉ‹ÏˆÛË Ù˘ ÓfiÛÔ˘. ∏ ÛÂÙÈÚÈ˙›ÓË Ô˘ ÂÏ¿Ì‚·ÓÂ Ô ÚÒÙÔ˜ ·ÛıÂÓ‹˜ ‰ÂÓ ¤¯ÂÈ ·Ó·ÊÂÚı› ˆ˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ù˘ π∂À. ∏ ·¯˘Û·ÚΛ· ÛÙ· ·È‰È¿ ·ÚÔ˘ÛÈ¿˙ÂÈ ÂȉËÌÈ΋ ¤Í·ÚÛË ·fi ÙÔ 1980. √ ÂÈÔÏ·ÛÌfi˜ ·˘ÍË̤ÓÔ˘ ‚¿ÚÔ˘˜ Î·È ·¯˘Û·ÚΛ·˜ Û ·È‰È¿ 5-17 ÂÙÒÓ ÛÙËÓ ∂˘ÚÒË ˘ÔÏÔÁ›˙ÂÙ·È ÛÙÔ 20% ÙË ‰ÂηÂÙ›· 1990. ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ ÂÚÈÔ¯‹ Ù˘ ∫Ú‹Ù˘, Û ÌÂϤÙË ÙˆÓ ∞. ∫·Ê¿ÙÔ˘ Î·È Û˘Ó. Û ·È‰È¿ ËÏÈΛ·˜ 5-15 ÂÙÒÓ, Ô ÂÈÔÏ·ÛÌfi˜ ÙˆÓ ˘¤Ú‚·ÚˆÓ ·È‰ÈÒÓ ·˘Í‹ıËΠ·fi 21,3% Û 24,7% Î·È Ù˘ ·¯˘Û·ÚΛ·˜ ·fi 5,4% Û 10,4%, ÌÂٷ͇ ÙˆÓ ÂÚÈfi‰ˆÓ 1989-1993 Î·È 1999-2003 (19). ∞·ÈÙÂ›Ù·È Â·ÁÚ‡ÓËÛË ÙÔ˘ ·È‰È¿ÙÚÔ˘ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÙÔ˘ ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜ ÙˆÓ Ó·ÚÒÓ ÂÊ‹‚ˆÓ ÁÈ· ÙËÓ ÚfiÏË„Ë Ù˘ ·¯˘Û·ÚΛ·˜ Î·È ÙˆÓ ÓÔÛËÚÒÓ Î·Ù·ÛÙ¿ÛÂˆÓ Ô˘ ÙË Û˘Óԉ‡ԢÓ, ÛÙȘ Ôԛ˜ ÂÚÈÏ·Ì‚¿ÓÂÙ·È Î·È Ë È‰ÈÔ·ı‹˜ ÂÓ‰ÔÎÚ¿ÓÈ· ˘¤ÚÙ·ÛË.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Warman R. Management of pseudotumor cerebri in children. Int Pediatr 2000;15:147-150.

2. Kesler A, Fattal-Valevski A. Idiopathic intracranial hypertension in the pediatric population. J Child Neurol 2002; 17:745-748.

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3. Haslam RHA. Pseudotumor cerebri. In: Berman RE, Kliegman RM, Jenson HB, editors. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: WB Saunders; 2007. p. 2525.

4. Mercille G, Ospina LH. Pediatric idiopathic intracranial hypertension: a review. Pediatr Rev 2007; 28:77-86.

5. Skau M, Brennum J, Gjerris F, Jensen R. What is new about idiopathic intracranial hypertension? An updated review of mechanism and treatment. Cephalalgia 2006;26:384-399.

6. Speer C, Pearlman J, Phillips PH, Cooney M, Repka MX. Fourth cranial nerve palsy in pediatric patients with pseudotumor cerebri. Am J Ophthalmol 1999;127:236-237.

7. Straussberg R, Harel L, Amir J. Pseudotumor cerebri manifesting as stiff neck and torticollis. Pediatr Neurol 2002;26:225-227.

8. Lim M, Kurian M, Penn A, Calver D, Lin JP. Visual failure without headache in idiopathic intracranial hypertension.

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9. Soler D, Cox T, Bullock P, Calver DM, Robinson RO. Diagnosis and management of benign intracranial hypertension. Arch Dis Child 1998;78:89-94.

10. Balcer LJ, Liu GT, Forman S, Pun K, Volpe NJ, Galetta SL, et al. Idiopathic intracranial hypertension: relation of age and obesity in children. Neurology 1999;52:870-872.

11. Daniels AB, Liu GT, Volpe NJ, Galetta SL, Moster ML, Newman NJ, et al. Profiles of obesity, weight gain, and quality of life in idiopathic intracranial hypertension (pseudotumor cerebri). Am J Ophthalmol 2007;143:635-641.

12. §ÈÓ·Ú‰¿Î˘ ∂, µ·Ú‰¿‚·˜ ∞, ∫·Ê¿ÙÔ˜ ∞. ∂ηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘ ·È‰ÈÒÓ Ù˘ ∫Ú‹Ù˘ ËÏÈΛ·˜ 3 ¤ˆ˜ 16 ÂÙÒÓ. ¶·È‰È·ÙÚÈ΋ 2007;70:300-307.

13. Genizi J, Lahat E, Zelnik N, Mahajnah M, Ravid S, Shahar E. Childhood-onset idiopathic intracranial hypertension: relation of sex and obesity. Pediatr Neurol 2007;36:247-249.

14. Youroukos S, Psychou F, Fryssiras S, Paikos P, Nicolaidou P. Idiopathic intracranial hypertension in children. J Child Neurol 2000;15:453-457.

15. Cinciripini GS, Donahue S, Borchert MS. Idiopathic intracranial hypertension in prepubertal pediatric patients: characteristics, treatment, andoutcome. Am J Ophthalmol 1999;127:178-182.

16. Baker RS, Baumann RJ, Buncic JR. Idiopathic intracranial hypertension (pseudotumor cerebri) in pediatric patients.

Pediatr Neurol 1989;5:5-11.

17. Stevenson SB. Pseudotumor cerebri: yet another reason to fight obesity. J Pediatr Health Care 2008;22:40-43.

18. Xanthakos SA, Inge TH. Extreme pediatric obesity: weighing the health dangers. J Pediatr 2007;150:3-5.

19. §ÈÓ·Ú‰¿Î˘ ª, ªÂÚÙÛÈ¿˜ °, ™·ÚÚ‹ ∫, ∫·Ê¿ÙÔ˜ ∞. ªÂÙ·‚ÔÏ‹ ۈ̷ÙÔÌÂÙÚÈÎÒÓ ÌÂÁÂıÒÓ, ·ÚÙËÚȷ΋˜ ›ÂÛ˘, ۷ί¿ÚÔ˘ Î·È ÏÈȉ›ˆÓ ÔÚÔ‡ ·›Ì·ÙÔ˜ ·È‰ÈÒÓ Ù˘ ∫Ú‹Ù˘ ÌÂٷ͇ ÙˆÓ ¯ÚÔÓÈÎÒÓ ÂÚÈfi‰ˆÓ 1989-1993 Î·È 19992003. ¶·È‰È·ÙÚÈ΋ 2007;70:205-216.

¶·È‰È·ÙÚÈ΋ 2009;72:55-59

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CASE REPORT

™˘ÁÁÂÓ‹˜ ¯˘ÏÔıÒڷη˜ Ì ˘Ô‰fiÚÈÔ Ô›‰ËÌ· ¿Óˆ ËÌ›ÛÂÔ˜ ÛÒÌ·ÙÔ˜ Û ÓÂÔÁÓfi Ì ۇӉÚÔÌÔ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ 1 ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ ¡ÔÛËÏ›·˜ ¡ÂÔÁÓÒÓ, °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¡›Î·È·˜ «ÕÁÈÔ˜ ¶·ÓÙÂÏ‹̈ӻ 2 ∂ÚÁ·ÛÙ‹ÚÈÔ ¶·ıÔÏÔÁÈ΋˜ ∞Ó·ÙÔÌÈ΋˜, ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ AÏÏËÏÔÁÚ·Ê›·: µ·ÛÈÏÈ΋ ª·ÎÚ‹ vanessmakri@yahoo.gr À„ËÏ¿ÓÙÔ˘ 45, ¶Â‡ÎË, ∞ı‹Ó·

µ. ª·ÎÚ‹1, ∞ÈÎ. ∫ˆÓÛÙ·ÓÙÈÓ›‰Ë1, ª. ¶··‰ÔÔ‡ÏÔ˘1, Ÿ. ¢Â‰Ô‡ÛË1, ™. ∆¿ÓÙÚÔ˜1, ∂. ∞Á·ËÙfi˜2, ∞. °Ô‡Ó·Ú˘1 ¶ÂÚ›ÏË„Ë: ∏ Û˘Ó‡·ÚÍË ¯˘ÏÔıÒڷη Ì ˘Ô‰fiÚÈÔ Ô›‰ËÌ· ¿Óˆ ËÌ›ÛÂÔ˜ ÛÒÌ·ÙÔ˜ ·ÔÙÂÏ› ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜, ÙÔ ÔÔ›Ô ÂÌÊ·Ó›˙ÂÙ·È Û˘Ó‹ıˆ˜ ›Ù ˆ˜ ÂÈÏÔ΋ ηډÈÔ¯ÂÈÚÔ˘ÚÁÈÎÒÓ ÂÂÌ‚¿ÛÂˆÓ Â›Ù ÛÙ· Ï·›ÛÈ· ÓÂÔÏ·ÛÌ·ÙÈ΋˜ ÓfiÛÔ˘ Ì ÂÈÓ¤ÌÂÛË ÛÙÔ ÌÂÛÔıˆÚ¿ÎÈÔ. π‰È·›ÙÂÚ· Û¿ÓÈ·, ÙÔ Û‡Ó‰ÚÔÌÔ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ ÌÔÚ› Ó· ÂÌÊ·ÓÈÛı› Û˘ÁÁÂÓÒ˜. ¶ÂÚÈÁÚ¿ÊÂÙ·È ÂÚ›ÙˆÛË ÚfiˆÚÔ˘ ÓÂÔÁÓÔ‡ 33 ‚‰ÔÌ¿‰ˆÓ ·ËÛ˘ Ô˘ ÁÂÓÓ‹ıËΠÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ÙÔÎÂÙfi Î·È ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ¯ÚÂÈ¿ÛÙËΠηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÓË„Ë, ÂÓÒ ÛÙË Û˘Ó¤¯ÂÈ·, ÏfiÁˆ ÛËÌ·ÓÙÈ΋˜ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜, ÙÔÔıÂÙ‹ıËΠ۠Ì˯·ÓÈ΋ ˘ÔÛÙ‹ÚÈÍË Ì ˘„ËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ·ÂÚÈÛÌfi Ì ٷϷÓÙÒÛÂȘ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÓÙÈÌÂÙˆÈÛı› Ë ÛËÌ·ÓÙÈÎÔ‡ ‚·ıÌÔ‡ ·Ó·Ó¢ÛÙÈ΋ Î·È ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË. ∫·Ù¿ ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠÂÈÎfiÓ· Û˘Ó‰ÚfiÌÔ˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜. ™˘ÁÎÂÎÚÈ̤ӷ, ÙÔ ÓÂÔÁÓfi ÂÌÊ¿ÓÈ˙ ÂÎÛÂÛËÌ·Ṳ̂ÓÔ ˘Ô‰fiÚÈÔ Ô›‰ËÌ· ¿Óˆ ËÌ›ÛÂÔ˜ ÛÒÌ·ÙÔ˜ Î·È ·fi ÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ·ÔηχÊıËÎÂ Û˘ÏÏÔÁ‹ Ï¢ÚÈÙÈÎÔ‡ ˘ÁÚÔ‡ ÛÙÔ ‰ÂÍÈfi ËÌÈıˆÚ¿ÎÈÔ, Ë ·Ó¿Ï˘ÛË ÙÔ˘ ÔÔ›Ô˘ ¤‰ÂÈÍ fiÙÈ ‹Ù·Ó Û˘Ì‚·Ù‹ Ì ¯˘ÏÔıÒڷη. ¶·Ú¿ ÙËÓ ÚÔÛˆÚÈÓ‹ ‚ÂÏÙ›ˆÛË Ô˘ ·ÚÔ˘Û›·Û ÙÔ ÓÂÔÁÓfi ÌÂÙ¿ ·fi ÙËÓ ·ÚÔ¯¤Ù¢ÛË ÙÔ˘ ¯˘ÏÔıÒڷη, 12 ÒÚ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ÂÌÊ¿ÓÈÛ ڷÁ‰·›· Âȉ›ӈÛË Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜ ÏfiÁˆ Ó¢ÌÔıÒڷη ˘fi Ù¿ÛË Î·È, ·Ú¿ ÙȘ ÚÔÛ¿ıÂȘ ·Ó¿Ó˄˘, η٤ÏËÍÂ. ∏ ·ıÔÏÔÁÔ·Ó·ÙÔÌÈ΋ ÂͤٷÛË ÙÔ˘ ÓÂÔÁÓÔ‡ ¤‰ÂÈÍ ıÚfiÌ‚ˆÛË Ù˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜.

§¤ÍÂȘ ÎÏÂȉȿ: Ã˘ÏÔıÒڷη˜, Ô›‰ËÌ·, ıÚfiÌ‚ˆÛË, ¿Óˆ ÎÔ›ÏË ÊϤ‚·.

Congenital chylothorax and subcutaneous oedema in a neonate with superior vena cava syndrome 1 NICU, “Aghios Panteleimon” General Hospital of Nikaia-Piraeus, Piraeus, Greece 2 Department of Pathology, University of Athens, Athens, Greece Correspondence: Vassiliki Makri vanessmakri@yahoo.gr 45, Ipsilantou St., Pefki, Athens, Greece

V. Makri1, A. Konstantinidi1, M. Papadopoulou1, O. Dedousi1, S. Tantros1, E. Agapitos2, A. Gounaris1 Abstract: Chylothorax and venous congestion leading to subcutaneous oedema of the upper half of the body are clinical characteristics of the superior vena cava syndrome, which usually presents either as a complication after cardiopulmonary surgery or due to local compression by underlying neoplasms of the mediastinum. In extremely rare cases, superior vena cava syndrome can occur due to congenital vascular thrombosis. This is a case report of a premature infant of 33 weeks gestation, which was delivered vaginally and needed cardiorespiratory resuscitation immediately after birth. The neonate showed signs of severe respiratory distress and arterial blood samples revealed both respiratory and metabolic acidosis, which was treated with High Frequency Oscillatory Ventilation. Physical examination revealed subcutaneous oedema of the upper half of the body, and the chest X-ray demonstrated pleural effusion of the right hemithorax, which on drainage and analysis was biochemically consistent with chylothorax. Despite temporary improvement after the chest tube drainage of the chylothorax, about 12 hours after birth the condition worsened dramatically, with the development of tension pneumothorax, which did not respond to resuscitation efforts, and the infant died. Post-mortem examination confirmed the diagnosis of superior vena cava thrombosis.

Key words: Chylothorax, subcutaneous oedema, thrombosis, superior vena cava.

∂ÈÛ·ÁˆÁ‹ ø˜ ¯˘ÏÔıÒڷη˜ ÔÚ›˙ÂÙ·È Ë Ï‹ÚˆÛË Ù˘ ˘Â˙ˆÎÔÙÈ΋˜ ÎÔÈÏfiÙËÙ·˜ Ì ϤÌÊÔ ÚÔÂÚ¯fiÌÂÓË ·fi ÙÚ·‡Ì· ‹ ·fiÊÚ·ÍË ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ fiÚÔ˘. √ ıˆÚ·ÎÈÎfi˜ fiÚÔ˜ ‰È·ÎÚ›ÓÂÙ·È Û Ì›˙ÔÓ· Î·È ÂÏ¿ÛÛÔÓ· Î·È Â΂¿ÏÏÂÈ ·Ú¯Èο ÛÙËÓ ·ÓÒÓ˘ÌË Î·È ÙÂÏÈο ÛÙËÓ ¿Óˆ ÎÔ›ÏË ÊϤ‚·, Ë ÔÔ›· ‰¤¯ÂÙ·È Â›Û˘ fiÏÔ ÙÔ ÊÏ‚ÈÎfi ·›Ì· ·fi Paediatriki 2009;72:60-65

ÙËÓ ÎÂÊ·Ï‹, ÙÔÓ ÙÚ¿¯ËÏÔ Î·È ·ÌÊfiÙÂÚ· Ù· ¿Óˆ ¿ÎÚ·. ø˜ ÂÎ ÙÔ‡ÙÔ˘, ÛÙÔ Û‡Ó‰ÚÔÌÔ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ (™∞∫º), Ë ·fiÊÚ·ÍË Ù˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ ÂÌÊ·Ó›˙ÂÙ·È ÎÏÈÓÈο Ì ÊÏ‚È΋ Û˘ÌÊfiÚËÛË Î·È Ô›‰ËÌ· ÎÂÊ·Ï‹˜-ÙÚ·¯‹ÏÔ˘-¿Óˆ ¿ÎÚˆÓ, ηıÒ˜ Î·È ‰ËÌÈÔ˘ÚÁ›· ¯˘ÏÔıÒڷη, ÏfiÁˆ Ù˘ ˘„ËÏ‹˜ ÊÏ‚È΋˜ ›ÂÛ˘ ÚÔ ÙÔ˘ Έχ̷ÙÔ˜, ÙÔ ÔÔ›Ô ·ÚÂÌÔ‰›˙ÂÈ ÙËÓ ÔÌ·Ï‹

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·61

61

™˘ÁÁÂÓ¤˜ Û‡Ó‰ÚÔÌÔ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜

ÏÂÌÊÈ΋ ÚÔ‹ ÂÓÙfi˜ ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ fiÚÔ˘, ÚÔηÏÒÓÙ·˜ ¤ÙÛÈ ‚Ï¿‚Ë ÛÙ· Ó¢ÌÔÓÈο ÏÂÌÊ·ÁÁ›· Î·È ÂÎÚÔ‹ ϤÌÊÔ˘ ÛÙËÓ ˘Â˙ˆÎÔÙÈ΋ ÎÔÈÏfiÙËÙ·. ∂ÈϤÔÓ, Â›Ó·È ‰˘Ó·ÙfiÓ ÁÈ· ÙÔÓ ›‰ÈÔ ÏfiÁÔ Ó· ÂÌÊ·ÓÈÛı› ÂÁÎÂÊ·ÏÈ΋ ·ÈÌÔÚÚ·Á›· Î·È Ó· ÌÂȈı› ÛËÌ·ÓÙÈο Ë Î·Ú‰È·Î‹ ·ÚÔ¯‹. ™Ù· Û˘Ó‹ıË ·›ÙÈ· ÙÔ˘ ™∞∫º ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ıÚÔÌ‚ˆÙÈο Û˘Ì‚¿Ì·Ù· ÌÂÙ¿ ·fi ηډÈÔıˆÚ·ÎÈΤ˜ ÂÂÌ‚¿ÛÂȘ, Ë ÙÔÔı¤ÙËÛË ÎÂÓÙÚÈÎÒÓ ÊÏ‚ÈÎÒÓ Î·ıÂÙ‹ÚˆÓ Î·È Ë ÙÔÈ΋ ›ÂÛË Ô˘ ÌÔÚ› Ó· ·ÛÎÂ›Ù·È ÛÙËÓ ¿Óˆ ÎÔ›ÏË ÊϤ‚· ·fi ÚˆÙÔ·ı›˜ ‹ ÌÂÙ·ÛÙ·ÙÈÎÔ‡˜ fiÁÎÔ˘˜ ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘ (1-4). ¶ÂÚÈÁÚ¿ÊÂÙ·È È‰È·›ÙÂÚ· Û¿ÓÈ· ÂÚ›ÙˆÛË Û˘ÁÁÂÓÔ‡˜ ™∞∫º Ô˘ ÂΉËÏÒıËΠÎÏÈÓÈο Ì ¯˘ÏÔıÒڷη Î·È ˘Ô‰fiÚÈÔ Ô›‰ËÌ· ¿Óˆ ËÌ›ÛÂÔ˜ ÛÒÌ·ÙÔ˜.

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ÕÚÚÂÓ ÓÂÔÁÓfi, Ì ‚¿ÚÔ˜ Á¤ÓÓËÛ˘ 2.500 g, ÁÂÓÓ‹ıËΠÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ÙÔÎÂÙfi ÙËÓ 33Ë Â‚‰ÔÌ¿‰· ·ËÛ˘, ÏfiÁˆ ÚfiˆÚˆÓ Û˘Û¿ÛÂˆÓ Ù˘ ·ÏÏÔ‰·‹˜ ÌËÙ¤Ú·˜ ÙÔ˘. ™ÙË Á¤ÓÓËÛË ÙÔ ÓÂÔÁÓfi ‹Ù·Ó ΢·ÓˆÙÈÎfi, ·ÓÔ˚Îfi, Ì ηډȷÎfi Ú˘ıÌfi 70-80 ÛÊ›ÍÂȘ/ÏÂÙfi. ŒÁÈÓ ·Ú¯Èο ·Ó¿ÓË„Ë Ì ·ÛÎfi Î·È ¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘, ÂÓÒ ÛÙÔ 3Ô ÏÂÙfi ‰È·ÛˆÏËÓÒıËÎÂ Î·È ·ÎÔÏÔ‡ıˆ˜ ÌÂٷʤÚıËΠÛÙÔ ÙÌ‹Ì· Ì·˜ ÁÈ· ÂÚ·ÈÙ¤Úˆ ·ÓÙÈÌÂÙÒÈÛË. ∆Ô Apgar-score ·Ó·Ê¤ÚÂÙ·È 2 ÛÙÔ 1Ô ÏÂÙfi Î·È 5 ÛÙÔ 5Ô ÏÂÙfi. ∞fi ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È fiÙÈ Î·È ÔÈ ‰‡Ô ÁÔÓ›˜ Â›Ó·È ˘ÁÈ›˜, fiˆ˜ Î·È ÙÔ 3 ÂÙÒÓ ı‹Ï˘ Ó‹ÈÔ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. ∞fi ÙÔÓ ÚÔÁÂÓÓËÙÈÎfi ¤ÏÂÁ¯Ô, Ù· ˘ÂÚ˯ÔÁÚ·Ê‹Ì·Ù·, Ô ¤ÏÂÁ¯Ô˜ ÁÈ· Û˘ÁÁÂÓ›˜ ÏÔÈÌÒÍÂȘ Î·È Ë ·ÌÓÈÔ·Ú·Î¤ÓÙËÛË ·Ó·Ê¤ÚÔÓÙ·È ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠ÙÚÈÙÔ‚¿ıÌÈÔ ÓÔÛÔÎÔÌÂ›Ô Ù˘ ∞ı‹Ó·˜, fiÔ˘ ÌÂٷʤÚıËÎÂ Ë ÌËÙ¤Ú· ÏfiÁˆ ÚfiˆÚˆÓ Û˘Û¿ÛˆÓ, ·Ó¤ÊÂÚ ÙËÓ ‡·ÚÍË ÔÏ˘˘‰Ú·ÌÓ›Ô˘ Ì Èı·Ófi ˘‰ÚÔıÒڷη, ÏËÚÔÊÔÚ›· Ô˘ ‰ÂÓ ¤ÁÈÓ ÁÓˆÛÙ‹ Û ÂÌ¿˜ ÂÁη›Úˆ˜. ∞fi ÙÔ Ì·È¢ÙÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È Â›Û˘ fiÙÈ ÏfiÁˆ ·Ófi‰Ô˘ Ù˘ CRP, 18 ÒÚ˜ ÚÔ ÙÔ˘ ÙÔÎÂÙÔ‡, Ë ÌËÙ¤Ú· ÂÙ¤ıË Û ÂÓ‰ÔÊϤ‚È· ·ÁˆÁ‹ Ì ·ÌÈÎÈÏÏ›ÓË-ÛÔ˘ÏÌ·ÎÙ¿ÌË Î·È ¤Ï·‚ 1 ‰fiÛË ‚ËÙ·ÌÂı·˙fiÓ˘. ∫·Ù¿ ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠfiÙÈ ÚfiÎÂÈÙ·È ÁÈ· ÔȉËÌ·Ù҉˜ ÓÂÔÁÓfi, Ô˘ ¿ÊËÓ ÂÓÙ‡ˆÌ· ÛÙËÓ ›ÂÛË ÛÙËÓ ÎÂÊ·Ï‹, ÛÙÔÓ ÙÚ¿¯ËÏÔ, ÛÙ· ¿Óˆ ¿ÎÚ· Î·È ÛÙÔ ¿Óˆ ‹ÌÈÛ˘ ÙÔ˘ ıÒڷη, ÂÓÒ ÙÔ Î¿Ùˆ ‹ÌÈÛ˘ ÙÔ˘ ÛÒÌ·ÙÔ˜ ‹Ù·Ó ÂÏ¿¯ÈÛÙ· ÔȉËÌ·Ù҉˜. ¶·Ú·ÙËÚ‹ıËÎ·Ó Â›Û˘ ÌÂÁ¿ÏË ‰˘ÛÌÔÚÊ›· ÙˆÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ ÙÔ˘ ÚÔÛÒÔ˘ ÏfiÁˆ ÙÔ˘ ÂÎÛÂÛËÌ·Ṳ̂ÓÔ˘ Ôȉ‹Ì·ÙÔ˜, ÌÂȈ̤ÓË Â›ÛÔ‰Ô˜ ·¤Ú· ÛÙÔ˘˜ Ó‡ÌÔÓ˜, ¢ÎÚÈÓ›˜ ηډȷÎÔ› ÙfiÓÔÈ, ¯ˆÚ›˜ ηډȷÎfi ʇÛËÌ·, „ËÏ·ÊËÙ¤˜ ÌËÚÈ-

·›Â˜ ·ÌÊÔÙÂÚfiÏ¢ڷ, „ËÏ·ÊËÙfi ‹·Ú 1,5 cm οوıÂÓ ÙÔ˘ ‰ÂÍÈÔ‡ ˘Ô¯ÔÓ‰Ú›Ô˘, ¤Íˆ ÁÂÓÓËÙÈο fiÚÁ·Ó· ¿ÚÚÂÓÔ˜. ™ÙÔ ÓÂÔÁÓfi, ÌÂÙ¿ ÙËÓ ¿ÊÈÍ‹ ÙÔ˘ ÛÙÔ ÙÌ‹Ì· Ì·˜, Û˘Ó¯›ÛÙËÎÂ Ë Î·Ú‰ÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÓË„Ë, Ô˘ ‰È‹ÚÎËÛ ÂÚ›Ô˘ 20 min. ™ÙË Û˘Ó¤¯ÂÈ·, ÙÔ ÓÂÔÁÓfi Û˘Ó‰¤ıËΠ۠·Ó·Ó¢ÛÙ‹Ú· SLE-5000 ÛÂ Û˘Á¯ÚÔÓÈṲ̂ÓÔ ‰È·Ï›ÔÓÙ· ˘Ô¯ÚˆÙÈÎfi ·ÂÚÈÛÌfi (SIMV), Ì ÛÙÔȯ›·: ̤ÁÈÛÙË ÂÈÛÓ¢ÛÙÈ΋ ›ÂÛË (PIP): 30 cm H2O, ıÂÙÈ΋ ÙÂÏÔÂÎÓ¢ÛÙÈ΋ ›ÂÛË (PEEP): 5 cm H2O, Û˘¯ÓfiÙËÙ·: 80 ·Ó·ÓÔ¤˜/ÏÂÙfi, ¯ÚfiÓÔ˜ ÂÈÛÓÔ‹˜: 0,35 sec Î·È ÎÏ¿ÛÌ· ÂÈÛÓÂfiÌÂÓÔ˘ Ô͢ÁfiÓÔ˘ (FiO2): 0,1. ∞fi ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô Ô˘ ‰ÈÂÓÂÚÁ‹ıËÎÂ, Ù· ·¤ÚÈ· ·ÚÙËÚÈ·ÎÔ‡ ·›Ì·ÙÔ˜ ·ÔÎ¿Ï˘„·Ó ÛËÌ·ÓÙÈÎÔ‡ ‚·ıÌÔ‡ ÌÂÈÎÙ‹ ·Ó·Ó¢ÛÙÈ΋ Î·È ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË, ÂÓÒ Ë ÁÂÓÈ΋ ·›Ì·ÙÔ˜ Î·È ÙÔ ÈÔÓÙfiÁÚ·ÌÌ· ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο (¶›Ó·Î·˜ 1). ∞ÎÔÏÔ‡ıˆ˜ ¤ÁÈÓ ·ÏÏ·Á‹ Ù˘ Ì˯·ÓÈ΋˜ ˘ÔÛÙ‹ÚÈ͢ Û ˘„ËÏ‹˜ Û˘¯ÓfiÙËÙ·˜ ·ÂÚÈÛÌfi Ì ٷϷÓÙÒÛÂȘ (HFOV), ÚÔÎÂÈ̤ÓÔ˘ Ó· ÌÂȈı› Ë ÌÂÚÈ΋ Ù¿ÛË ‰ÈÔÍÂȉ›Ô˘ ÙÔ˘ ¿Óıڷη ÛÙÔ ·›Ì· (PaCO2), ¯ˆÚ›˜ Ó· ·˘ÍËıÔ‡Ó ÂÚ·ÈÙ¤Úˆ ÔÈ Ó¢ÌÔÓÈΤ˜ ȤÛÂȘ ÚÔηÏÒÓÙ·˜ ‚·ÚfiÙÚ·˘Ì·. H ·/· ıÒÚ·ÎÔ˜ η٤‰ÂÈÍ ıÔÏÂÚfiÙËÙ· ‰ÂÍÈÔ‡ ËÌÈıˆÚ·Î›Ô˘ Û˘Ì‚·Ù‹ Ì ˘‰ÚÔıÒڷη ‰ÂÍÈ¿ Ô˘ ·ÚÂÎÙfiÈ˙ ÙËÓ Î·Ú‰È·Î‹ ÛÈÏÔ˘¤Ù· ·ÚÈÛÙÂÚ¿ (∂ÈÎfiÓ· 1). ŒÁÈÓ ·Ú·Î¤ÓÙËÛË Î·È ·ÚÔ¯ÂÙ‡ıËÎ·Ó ¿ÌÂÛ· 95 mL ÎÈÙÚÈÓˆÔ‡ ˘ÁÚÔ‡. ∏ ·Ó¿Ï˘ÛË ÙÔ˘ Ï¢ÚÈÙÈÎÔ‡ ˘ÁÚÔ‡ ¤‰ÂÈÍ fiÙÈ ÂÚfiÎÂÈÙÔ ÁÈ· ϤÌÊÔ (¶›Ó·Î·˜ 2). ™ÙË Û˘Ó¤¯ÂÈ· ÙÔÔıÂÙ‹ıËΠıˆÚ·ÎÈÎfi˜ ۈϋӷ˜ ÛÙÔ 6Ô ÌÂÛÔχÚÈÔ ‰È¿ÛÙËÌ· ‰ÂÍÈ¿, Ô˘ Û˘Ó¤¯ÈÛ ӷ ·ÚÔ¯ÂÙ‡ÂÈ Ï¤ÌÊÔ Î·È ÙÔ ÓÂÔÁÓfi ÂÙ¤ıË Û ·ÁˆÁ‹ Ì ÈÓfiÙÚÔ·, ‰ÈÔ˘ÚËÙÈο Î·È ·ÓÙÈ‚ÈÔÙÈο. ™Ù·‰È·Î¿ ˘Ô¯ÒÚËÛÂ Ë ·Ó·Ó¢ÛÙÈ΋ ÔͤˆÛË Î·È Ë ÔÍÂÔ‚·ÛÈ΋ ÈÛÔÚÚÔ›· ‚ÂÏÙÈÒıËΠÛËÌ·ÓÙÈο (¶›Ó·Î·˜ 1). ™Â ÂfiÌÂÓ˜ ·/· ıÒÚ·ÎÔ˜ ‰È·ÈÛÙÒıËΠ̛ˆÛË ÙÔ˘ ˘ÁÚÔ‡ ÛÙÔ ‰ÂÍÈfi ËÌÈıˆÚ¿ÎÈÔ, Ë Î·Ú‰È·Î‹ ÛÎÈ¿ Â·Ó‹Ïı ÛÙÔ Î¤ÓÙÚÔ ÙÔ˘ ıÒڷη, ˆÛÙfiÛÔ ·Ú¿ÏÏËÏ· ¤ÁÈÓ ÂÌÊ·Ó‹˜ ÌÈÎÚfiÙÂÚË ÔÛfiÙËÙ· ˘ÁÚÔ‡ ÛÙÔ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ. ¶ÂÚ›Ô˘ 12 ÒÚ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, ÙÔ ÓÂÔÁÓfi ÂÌÊ¿ÓÈÛ ڷÁ‰·›· Âȉ›ӈÛË Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜, Ì ·˘Í·ÓfiÌÂÓË ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, Ì›ˆÛË ÙÔ˘ ηٿ ÏÂÙfi ·ÂÚÈÛÌÔ‡ Î·È ÙÒÛË ÙÔ˘ ·ÚÙËÚÈ·ÎÔ‡ ÎÔÚÂÛÌÔ‡ Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ Ì Ô͢ÁfiÓÔ, Ú¿ÁÌ· Ô˘ Û˘Ì‚¿‰È˙ Ì ·ÎÙÈÓÔÏÔÁÈ΋ Âȉ›ӈÛË ÙÔ˘ ˘‰ÚÔıÒڷη, Ì ·‡ÍËÛË Ù˘ ÔÛfiÙËÙ·˜ ÙÔ˘ ˘ÁÚÔ‡ ÛÙÔÓ ·ÚÈÛÙÂÚfi ˘Â˙ˆÎfiÙ·. ŒÁÈÓ ÚÔÛ¿ıÂÈ· ·Ó¿Ó˄˘ Ì ÂÎ Ó¤Ô˘ ·ÚÔ¯¤Ù¢ÛË ÙÔ˘ ¯˘ÏÔıÒڷη ·ÚÈÛÙÂÚ¿ Î·È Î·Ú‰ÈÔ·Ó·Ó¢ÛÙÈ΋ ˘ÔÛÙ‹ÚÈÍË, ¯ˆÚ›˜ fï˜ ·ÔÙ¤ÏÂÛÌ·, ÂÓÒ ·fi ÙËÓ ÙÂÏÂ˘Ù·›· ·/· ıÒÚ·ÎÔ˜ ‰È·ÈÛÙÒıËΠÓ¢ÌÔıÒڷη˜ ˘fi Ù¿ÛË ·ÚÈÛÙÂÚ¿. ¶·È‰È·ÙÚÈ΋ 2009;72:60-65

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·62

62

µ. ª·ÎÚ‹ Î·È Û˘Ó.

¶›Ó·Î·˜ 1. ∂ÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜

pH pO2 pCO2 BE HCO3 §Â˘Î¿

∂Ú˘ıÚ¿ Hb Ht ¢∂∫ ∞ÈÌÔÂÙ¿ÏÈ· CRP ∆ÚÔÔÓ›ÓË I CK CK-MB √ÏÈο Ï¢ÎÒÌ·Ù· ∞Ï‚Ô˘Ì›ÓË Ca ÔÏÈÎfi Mg °Ï˘Îfi˙Ë

1Ë ÒÚ· ˙ˆ‹˜

4Ë ÒÚ· ˙ˆ‹˜

6,87 [7,35-7,50] 61 mmHg [55-80] 103 mmHg [27-40] -13 mmol/L [(-10)-(-2)] 10 mmmol/L [21-28] 18,8x109/L [9,0-30,0x109] (18,8x103/mm3) (¶: 36%, §: 53%, M: 8%, ∏: 3%) 4,08x1012/L [4,0-6,6x1012] (4,08x106/mm3) 2,48 mmol/L [2,25-3,49] (16 g/dL) 0,50 [0,48-0,69] (50%) 0,07 [0,004-0,06] (7%) 204x109/L [84-478x109] (204x103/mm3) <0,5 mg/L [<3,5] 0,18 Ìg/L [0,088-1,12] 214 U/L [214-1175] 4,7 U/L [1,7-7,9%] 33 g/L [43-76] (3,3 g/dL) 20 g/L [18-30] (2,0 g/dL) 2,22 mmol/L [2,3-2,65] (8,9 mg/dL) 1,15 mmol/L [0,48-1,05] (2,8 mg/dL) 9,8 mmol/L [1,1-3,3] (176 mg/dL)

7,50 [7,35-7,50] 191 mmHg [55-80] 20 mmHg [27-40] -7 mmol/L [(-10)-(-2)] 20,9 mmmol/L [21-28]

∂ÓÙfi˜ ·Á·Ï˘ ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ÁÈ· ÙËÓ ËÏÈΛ·, Û‡Ìʈӷ Ì ÙÔ ‚È‚Ï›Ô: ¡elson Textbook of Pediatrics, 17th edition.

™ÙËÓ ÙÂÏÈ΋ ‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË ÙÔ˘ ÂÚÈÛÙ·ÙÈÎÔ‡ ·˘ÙÔ‡ Û˘Ó¤‚·Ï ԢÛÈ·ÛÙÈο Ë ¯·Ú·ÎÙËÚÈÛÙÈ΋ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ Ôȉ‹Ì·ÙÔ˜ ·ÔÎÏÂÈÛÙÈο ÙÔ˘ ¿Óˆ ËÌ›ÛÂÔ˜ ÙÔ˘ ÛÒÌ·ÙÔ˜, Ë ÔÔ›·, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÔÓ ¯˘ÏÔıÒڷη, ·Ú¤ÂÌ Û ۇӉÚÔÌÔ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜, οÙÈ Ô˘ ÙÂÏÈο ÂȂ‚·›ˆÛ ÙÔ ·ÔÙ¤ÏÂÛÌ· Ù˘ ·ıÔÏÔÁÔ·Ó·ÙÔÌÈ΋˜ ÂͤٷÛ˘, fiÔ˘ ‰È·ÈÛÙÒıËΠıÚfiÌ‚ˆÛË ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ ¶›Ó·Î·˜ 2. ∞Ó¿Ï˘ÛË Ï¢ÚÈÙÈÎÔ‡ ˘ÁÚÔ‡ §Â˘Î¿ ∂Ú˘ıÚ¿ CRP LDH °Ï˘Îfi˙Ë §Â‡ÎˆÌ· ∆ÚÈÁÏ˘ÎÂÚ›‰È· ÃÔÏËÛÙÂÚfiÏË Paediatriki 2009;72:60-65

0,48x109/L (0,48x103/mm3) (¶: 27%, §: 70%, ∏: 3%) ·ÚÎÂÙ¿ (95% ÚfiÛÊ·Ù·) 1,6 mg/L 123 IU/L 4,3 mmol/L (77 mg/dL) 24 g/L (2,4g/dL) 0,07 g/L (7 mg/dL) 0,70 mmol/L (27 mg/dL)

Î·È Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· ÏfiÁˆ Û˘Ì›ÂÛ˘ ·fi ÙÔÓ ¯˘ÏÔıÒڷη, ηıÒ˜ Î·È ·ÏÏÔÈÒÛÂȘ Û˘Ó‰ÚfiÌÔ˘ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜.

™˘˙‹ÙËÛË √ ¯˘ÏÔıÒڷη˜ Â›Ó·È Û˘Ó‹ıˆ˜ Â›ÎÙËÙÔ˜, ÌÔÓfiÏ¢ÚÔ˜ Î·È ÂÌÊ·Ó›˙ÂÙ·È Û˘¯ÓfiÙÂÚ· ·ÚÈÛÙÂÚ¿. ∆fiÛÔ ÛÙÔÓ ÂÓ‹ÏÈÎÔ fiÛÔ Î·È ÛÙÔÓ ·È‰ÈÎfi ÏËı˘ÛÌfi ÔÊ›ÏÂÙ·È Û ÔÈΛϷ ·›ÙÈ·, ÌÂ Û˘¯ÓfiÙÂÚÔ ÙËÓ ÂÈÏÔ΋ ÌÂÙ¿ ·fi ηډÈÔ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË, Ô˘ ¤¯ÂÈ ÚÔηϤÛÂÈ Ú‹ÍË ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ fiÚÔ˘. ÕÏÏ· ·›ÙÈ· Â›Ó·È ÙÔ ıˆÚ·ÎÈÎfi ÙÚ·‡Ì·, Ë ÚˆÙÔ·ı‹˜ ‹ ‰Â˘ÙÂÚÔ·ı‹˜ ÂÓ‰ÔıˆÚ·ÎÈ΋ ηÎÔ‹ıÂÈ·, Ë ÏÂÌÊ·ÁÁÂȈ̿وÛË, ÔÈ ÂÚÈÔÚÈÛÙÈΤ˜ Ó¢ÌÔÓÔ¿ıÂȘ ηÈ, Ù¤ÏÔ˜, Ë Â›ÎÙËÙË ıÚfiÌ‚ˆÛË ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ fiÚÔ˘, Ù˘ ˘ÔÎÏÂȉ›Ô˘ ‹ Ù˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ (™∞∫º), ÏfiÁˆ ‡·Ú͢ ÎÂÓÙÚÈÎÔ‡ ÊÏ‚ÈÎÔ‡ ηıÂÙ‹Ú· (1,4). ™ÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÔÓÙ·È Û¿ÓÈ· ÂÚÈÛÙ·ÙÈο Û˘ÁÁÂÓÔ‡˜ ¯˘ÏÔıÒڷη, Ô ÔÔ›Ô˜ ÂÌÊ·Ó›˙ÂÙ·È

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·63

63

™˘ÁÁÂÓ¤˜ Û‡Ó‰ÚÔÌÔ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜

∂ÈÎfiÓ· 1. ∞/· ıÒÚ·ÎÔ˜, fiÔ˘ ·ÂÈÎÔÓ›˙ÂÙ·È Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡ ÛÙÔ ‰ÂÍÈfi ËÌÈıˆÚ¿ÎÈÔ (ÏÂÙ¿ ‚¤ÏË), Û˘Ì‚·Ù‹ Ì ˘‰ÚÔıÒڷη ‰ÂÍÈ¿, Ô˘ ·ÚÂÎÙÔ›˙ÂÈ ÙËÓ Î·Ú‰È·Î‹ ÛÈÏÔ˘¤Ù· ·ÚÈÛÙÂÚ¿. ¢È·ÎÚ›ÓÂÙ·È Â›Û˘ ÂÎÛÂÛËÌ·Ṳ̂ÓÔ Ô›‰ËÌ· ÎÂÊ·Ï‹˜-ÙÚ·¯‹ÏÔ˘ (¯ÔÓÙÚ¿ ‚¤ÏË).

›Ù ÌÂÌÔӈ̤ӷ [ÛÔÚ·‰ÈÎfi˜ ‹ ÎÏËÚÔÓÔÌÔ‡ÌÂÓÔ˜ Ì ·˘ÙÔÛˆÌÈÎfi ‹ Ê˘ÏÔÛ‡Ó‰ÂÙÔ ÙÚfiÔ (5)] ›Ù ÛÙ· Ï·›ÛÈ·: ·) ȉÈÔ·ıÔ‡˜ ÂÌ‚Ú˘˚ÎÔ‡ ‡‰Úˆ· ÌË ·ÓfiÛÔ˘ ·ÈÙÈÔÏÔÁ›·˜ (6,7), ‚) Û˘ÁÁÂÓÔ‡˜ Ó¢ÌÔÓÈ΋˜ ÏÂÌÊ·ÁÁÂÈÂÎÙ·Û›·˜ ›Ù ȉÈÔ·ıÔ‡˜ ›Ù ÎÏËÚÔÓÔÌÔ‡ÌÂÓ˘ (8) ›Ù ÛÙ· Ï·›ÛÈ· Û˘Ó‰ÚfiÌˆÓ Ì ÔÏÏ·Ϥ˜ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜, fiˆ˜ Ù· Û‡Ó‰ÚÔÌ· Noonan, Turner, Down, Hennekam (9,10), Á) ÚˆÙÔ·ıÔ‡˜ ÏÂÌÊÈ΋˜ ‰˘ÛÏ·Û›·˜ (11), ‰) Û˘ÁÁÂÓÔ‡˜ ˘Ôı˘ÚÂÔÂȉÈÛÌÔ‡ (12) Î·È Â) Û˘ÁÁÂÓÔ‡˜ ·fiÊڷ͢ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ (™∞∫º) (13-15). ∞Û¯¤Ùˆ˜ Ì ÙËÓ ·ÈÙ›· ÚfiÎÏËÛ˘ ÙÔ˘ ¯˘ÏÔıÒڷη, Ë ÎÏÈÓÈ΋ ÙÔ˘ ÂÈÎfiÓ· ·Ú·Ì¤ÓÂÈ ¯·Ú·ÎÙËÚÈÛÙÈ΋, ÌÂ Û˘ÌÙÒÌ·Ù· ›ÂÛ˘ ÙfiÛÔ Â› ÙÔ˘ Ó¢ÌÔÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜ fiÛÔ Î·È Â› Ù˘ ηډȿ˜, Ì ·ÔÙ¤ÏÂÛÌ· ÙÔ ÓÂÔÁÓfi, fiˆ˜ Î·È ÛÙËÓ ÚÔÎÂÈ̤ÓË ÂÚ›ÙˆÛË, Ó· ÂÌÊ·Ó›˙ÂÈ ‰‡ÛÓÔÈ·, ΢¿ÓˆÛË, ·Ó·Ó¢ÛÙÈ΋ Î·È Î·Ú‰È·Î‹ ·ÓÂ¿ÚÎÂÈ·, Ì ÌÂȈ̤ÓË Î·Ú‰È·Î‹ ·ÚÔ¯‹ (1). ∏ ÔÚÈÛÙÈ΋ ‰È¿ÁÓˆÛË ÙÔ˘ ¯˘ÏÔıÒڷη Ù›ıÂÙ·È Ì ıˆÚ·ÎÔΤÓÙËÛË Î·È ·Ó¿Ï˘ÛË ÙÔ˘ Ï¢ÚÈÙÈÎÔ‡ ˘ÁÚÔ‡, ÙÔ ÔÔ›Ô Â›Ó·È Á·Ï·ÎÙ҉˜ Î·È ÂÌÂÚȤ¯ÂÈ Ï›-

Ë (ÙÚÈÁÏ˘ÎÂÚ›‰È·, ¯ÔÏËÛÙÂÚfiÏË, ¯˘ÏÔÌÈÎÚ¿), ÚˆÙ½Ó˜, ÏÂÌÊÔ·ÙÙ·Ú· Î·È ¿ÏÏ· Û˘ÛÙ·ÙÈο Ù˘ ϤÌÊÔ˘. ™Â ·˘Ùfi ÙÔ ÛËÌÂ›Ô Ú¤ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÛÙ· ÓÂÔÁÓ¿ Ô˘ ‰ÂÓ ¤¯Ô˘Ó ÛÈÙÈÛı› ·ÎfiÌË, ÙÔ Ï¢ÚÈÙÈÎfi ˘ÁÚfi ÌÔÚ› Ó· Â›Ó·È ‰È·˘Á¤˜ (1,13). øÛÙfiÛÔ, ÛÙËÓ ÚÔÎÂÈ̤ÓË ÂÚ›ÙˆÛË, Ë Û˘Ó‡·ÚÍË ¯˘ÏÔıÒڷη Ì ˘Ô‰fiÚÈÔ Ô›‰ËÌ· ¿Óˆ ËÌ›ÛÂÔ˜ ÛÒÌ·ÙÔ˜ Û˘Ì‚·‰›˙ÂÈ Ì ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ ™∞∫º, οÙÈ Ô˘ ÂȂ‚·›ˆÛÂ Ë ·ıÔÏÔÁÔ·Ó·ÙÔÌÈ΋ ÂͤٷÛË ÙÔ˘ ÓÂÔÁÓÔ‡. ™¯ÂÙÈο Ì ٷ ·›ÙÈ· ÙÔ˘ ™∞∫º, Û˘Ó‹ıˆ˜ ÚfiÎÂÈÙ·È ÁÈ· Â›ÎÙËÙË ‰È·Ù·Ú·¯‹, ·ÔÙ¤ÏÂÛÌ· ÚÔËÁËı›Û˘ ηډÈÔ¯ÂÈÚÔ˘ÚÁÈ΋˜ Â¤Ì‚·Û˘, Â͈ÙÂÚÈ΋˜ ›ÂÛ˘ ·fi ÓÂfiÏ·ÛÌ· ‹ ÙÔÔı¤ÙËÛ˘ ÎÂÓÙÚÈÎÒÓ ÊÏ‚ÈÎÒÓ Î·ıÂÙ‹ÚˆÓ (2,3). ∞fi fiÛÔ ÁÓˆÚ›˙Ô˘ÌÂ, ¤ˆ˜ ÙÒÚ· ‰ÂÓ ¤¯ÂÈ ·Ó·ÊÂÚı› ÂÚ›ÙˆÛË Û˘ÁÁÂÓÔ‡˜ ıÚfiÌ‚ˆÛ˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜, ·Ú¿ ÌfiÓÔ ¤Ó· ÂÚÈÛÙ·ÙÈÎfi Û˘ÁÁÂÓÔ‡˜ ÛÙ¤ÓˆÛ˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜ ·ÁÓÒÛÙÔ˘ ·ÈÙÈÔÏÔÁ›·˜, fiÔ˘ Ë ‰È¿ÁÓˆÛË ÂÙ¤ıË Ì ·ÁÁÂÈÔÁÚ·Ê›· Î·È Ë ·ÓˆÌ·Ï›· ·ÔηٷÛÙ¿ıËΠ̠‰È¿ÓÔÈÍË ÙÔ˘ ·ÁÁ›Ԣ Ì Ì·ÏÔÓ¿ÎÈ Î·È ÙÔÔı¤ÙËÛË ÂÓ‰·ÁÁÂÈ·ÎÔ‡ stent (13), ηıÒ˜ Î·È Û¿ÓÈ· ÂÚÈÛÙ·ÙÈο Û˘ÁÁÂÓÔ‡˜ ·Ï·Û›·˜ (14) ‹ ˘ÔÏ·Û›·˜ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜, ¯ˆÚ›˜ Û˘ÓÔ‰fi Û˘ÁÁÂÓ‹ ηډȷ΋ ·ÓˆÌ·Ï›· (15). ∂ÓÒ, fï˜, ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ‰ÂÓ ·Ó·Ê¤ÚÂÙ·È ·ÚfiÌÔÈÔ ÂÚÈÛÙ·ÙÈÎfi, ˘¿Ú¯Ô˘Ó ·Ó·ÊÔÚ¤˜ Û Û¿ÓȘ ÂÚÈÙÒÛÂȘ Û˘ÁÁÂÓÔ‡˜ ıÚfiÌ‚ˆÛ˘ ¿ÏÏˆÓ ·ÁÁ›ˆÓ. ™˘ÁÎÂÎÚÈ̤ӷ, ·Ó·Ê¤ÚÔÓÙ·È ÂÚÈÛÙ·ÙÈο ÂÓ‰ÔÌËÙÚ›Ô˘ ıÚfiÌ‚ˆÛ˘ Ù˘ οو ÎÔ›Ï˘ Î·È ÙˆÓ ÓÂÊÚÈÎÒÓ ÊÏ‚ÒÓ, Ô˘ ·Ô‰fiıËÎ·Ó Û ÁÂÓÂÙÈο ÎÏËÚÔÓÔÌÔ‡ÌÂÓË ıÚÔÌ‚ÔÊÈÏ›· ÙfiÛÔ Ù˘ ÌËÙ¤Ú·˜ fiÛÔ Î·È ÙÔ˘ ÂÌ‚Ú‡Ô˘, ηıÒ˜ Î·È ÛÂ Û˘Ó‰˘·ÛÌfi ·˘ÙÒÓ Ì ¤Ó· ÚÔıÚÔÌ‚ˆÙÈÎfi ÂÓ‰ÔÌ‹ÙÚÈÔ ÂÚÈ‚¿ÏÏÔÓ Ô˘ ‰ËÌÈÔ˘ÚÁ› Ë ˘¤ÚÙ·ÛË Ù˘ ÌËÙ¤Ú·˜, Ô ‰È·‚‹Ù˘ ·ËÛ˘, Ë ÚÔÂÎÏ·Ì„›· Î·È Ë ÂÓ‰ÔÌ‹ÙÚÈ· Ïԛ̈ÍË, ·ÏÏ¿ Î·È Ù· ·˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù· ÏfiÁˆ Ù˘ ·ÚÔ˘Û›·˜ ·˘ÙÔ·ÓÙÈۈ̿وÓ, fiˆ˜ ÙÔ ·ÓÙÈËÎÙÈÎfi ÙÔ˘ χÎÔ˘ Î·È Ù· ·ÓÙÈʈÛÊÔÏÈȉÈο ·ÓÙÈÛÒÌ·Ù· (16-19). ¶·Ú·‰Â›ÁÌ·Ù· ÎÏËÚÔÓÔÌÔ‡ÌÂÓ˘ ıÚÔÌ‚ÔÊÈÏ›·˜ (20,21), Ô˘ ÌÔÚ› Ó· ÂÌÊ·Ó›˙ÔÓÙ·È Â›Ù ÌÂÌÔӈ̤ӷ ›ÙÂ Î·È ÛÂ Û˘Ó‰˘·ÛÌfi, ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 3. ∆¤ÏÔ˜, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ ·›ÙÈÔ Ô˘ ÚÔοÏÂÛ ÙË ıÚfiÌ‚ˆÛË Ù˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜, Ë ıÂÚ·›· Â›Ó·È Î·Ù·Ú¯‹Ó Ë ÛÙ·ıÂÚÔÔ›ËÛË ÙÔ˘ ·ÛıÂÓÔ‡˜. ∂ÊfiÛÔÓ Ë Ï¢ÚÈÙÈ΋ Û˘ÏÏÔÁ‹ ϤÌÊÔ˘ Â›Ó·È ÌÂÁ¿ÏË, ı· ÚÔηϤÛÂÈ ÛÔ‚·Ú‹ ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· Î·È ·ÈÌÔ‰˘Ó·ÌÈ΋ ·ÛÙ¿ıÂÈ· ÏfiÁˆ Ì›ˆÛ˘ Ù˘ ηډȷ΋˜ ·ÚÔ¯‹˜, ÔfiÙ ı· ¯ÚÂÈ·ÛÙ› Â›ÁÔ˘Û· ıˆÚ·ÎÔΤÓÙËÛË, ηٿ ÚÔÙ›ÌËÛË ‹‰Ë ÛÙËÓ ·›ıÔ˘Û· ÙÔÎÂÙÔ‡, Î·È ·ÚÔ¯¤Ù¢ÛË ÙÔ˘ ˘ÁÚÔ‡ Ì ÙÔÔı¤ÙËÛË ıˆÚ·ÎÈÎÔ‡ ۈϋӷ (13). ™ÙË Û˘Ó¤¯ÂÈ·, ı· Ú¤ÂÈ Ó· ÙÂÎÌËÚȈı› ·ÂÈÎÔÓÈÛÙÈο Ë ıÚfiÌ‚ˆÛË ÙÔ˘ ·ÁÁ›Ԣ ›Ù Ì ˘ÂÚ˯ÔÁÚ¿ÊËÌ· (Doppler) ›Ù Ì ·ÁÁÂÈÔÁÚ·Ê›· ¶·È‰È·ÙÚÈ΋ 2009;72:60-65

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·64

64

µ. ª·ÎÚ‹ Î·È Û˘Ó.

¶›Ó·Î·˜ 3. ∞ÈÙÈÔÏÔÁ›· ÎÏËÚÔÓÔÌÔ‡ÌÂÓ˘ ıÚÔÌ‚ÔÊÈÏ›·˜ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô 1. ∞ÓÂ¿ÚÎÂÈ· ÙˆÓ ÚˆÙÂ˚ÓÒÓ C Î·È S, Ë ÔÔ›·, ÂÓÒ Û˘Ó‹ıˆ˜ ·ÊÔÚ¿ ÌfiÓÔ Ì›· ·fi ÙȘ ‰‡Ô ·˘Ù¤˜ ÚˆÙ½Ó˜ Î·È Â›Ó·È ÂÙÂÚfi˙˘ÁÔ˜, Û·ÓÈfiÙÂÚ· ÌÔÚ› Ó· ·ÊÔÚ¿ Î·È ÙȘ ‰‡Ô (‰ÈÏ‹ ÂÙÂÚfi˙˘ÁÔ˜) ‹ Ó· Â›Ó·È ÔÌfi˙˘ÁÔ˜, ÔfiÙ Úԉȷı¤ÙÂÈ Û ÊÏ‚ÈΤ˜ ıÚÔÌ‚ÒÛÂȘ Ô˘ ÌÔÚ› Ó· ÂΉËψıÔ‡Ó ÓˆÚ›˜ ÛÙË ˙ˆ‹, ·ÎfiÌË Î·È ÂÓ‰ÔÌËÙÚ›ˆ˜ 2. ∞ÓÂ¿ÚÎÂÈ· ·ÓÙÈıÚÔÌ‚›Ó˘-πππ 3. ÀÔ- ‹ ‰˘ÛÏ·ÛÌÈÓÔÁÔÓ·ÈÌ›· 4. ∞ÓÙ›ÛÙ·ÛË ÛÙËÓ ÂÓÂÚÁÔÔÈË̤ÓË ÚˆÙ½ÓË-C (·Ú¿ÁˆÓ-V-Leiden) 5. ∞ÓÂ¿ÚÎÂÈ· ÙÔ˘ Û˘Ì·Ú¿ÁÔÓÙ·-ππ Ù˘ Ë·Ú›Ó˘ 6. ∞‡ÍËÛË ÂÈ¤‰ˆÓ ÚÔıÚÔÌ‚›Ó˘ ÛÙÔ Ï¿ÛÌ· (ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ÁfiÓÔ G20210∞) 7. ∞‡ÍËÛË ÂÈ¤‰ˆÓ ÔÌÔ΢ÛÙ½Ó˘ ÛÙÔ Ï¿ÛÌ·, ÛÙ· Ï·›ÛÈ· ÎÏ·ÛÈ΋˜ ÔÌÔ΢ÛÙÈÓÔ˘Ú›·˜ ‹ ıÂÚÌÈ΋˜ ·ÛÙ¿ıÂÈ·˜ ÙÔ˘ ÂÓ˙‡ÌÔ˘ MTHFR ÏfiÁˆ ÔÌfi˙˘ÁÔ˘ ÔÏ˘ÌÔÚÊÈÛÌÔ‡ (677C→ T)

(„ËÊȷ΋ ‹ Ì·ÁÓËÙÈ΋). ∞fi ÂΛ Î·È ¤Ú·, ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ÚÔÙ›ÓÂÙ·È Ë ¯ÔÚ‹ÁËÛË ·ÓÙÈËÎÙÈÎÒÓ, Ë ÂÊ·ÚÌÔÁ‹ ıÚÔÌ‚ÔÏ˘ÙÈ΋˜ ·ÁˆÁ‹˜, Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ıÚÔÌ‚ÂÎÙÔÌ‹ ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔηٷÛÙ·ı› Ë ‚·ÙfiÙËÙ· ÙÔ˘ ·ÁÁ›Ԣ ‹ Î·È Û˘Ó‰˘·ÛÌfi˜ ÙˆÓ ·Ú·¿Óˆ ηٿ ÂÚ›ÙˆÛË, ¯ˆÚ›˜ Ó· ˘¿Ú¯ÂÈ ÔÌÔʈӛ· (18,19,22,23). ¶ÚÔÎÂÈ̤ÓÔ˘ Ó· ÌÂȈı› Ë ÏÂÌÊÈ΋ ÚÔ‹ ÂÓÙfi˜ ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ fiÚÔ˘ Î·È ˆ˜ ÂÎ ÙÔ‡ÙÔ˘ Ë ÂÎÚÔ‹ ϤÌÊÔ˘ ·fi Ù· ÙÚ·˘Ì·ÙÈṲ̂ӷ Ó¢ÌÔÓÈο ÏÂÌÊ·ÁÁ›· ÛÙËÓ ˘Â˙ˆÎÔÙÈ΋ ÎÔÈÏfiÙËÙ·, ÚÔÙ›ÓÂÙ·È Ë ÂÊ·ÚÌÔÁ‹ ‰›·ÈÙ·˜ ÏÔ‡ÛÈ·˜ Û ÙÚÈÁÏ˘ÎÂÚ›‰È· ̤Û˘ ·Ï‡ÛÔ˘ (MCT) Ô˘ ·ÔÚÚÔÊÒÓÙ·È ·¢ı›·˜ ̤ۈ Ù˘ ˘Ï·›·˜ ÊϤ‚·˜ (13). ¶ÂÚÈÁÚ¿ÊÂÙ·È È‰È·›ÙÂÚ· Û¿ÓÈÔ ÂÚÈÛÙ·ÙÈÎfi Û˘ÁÁÂÓÔ‡˜ ıÚfiÌ‚ˆÛ˘ ¿Óˆ ÎÔ›Ï˘ ÊϤ‚·˜, Ô˘ ¤ÁÈÓ ÎÏÈÓÈο ¤Î‰ËÏË Ì ÂÌÊ¿ÓÈÛË ˘Ô‰fiÚÈÔ˘ Ôȉ‹Ì·ÙÔ˜ ¿Óˆ ËÌ›ÛÂÔ˜ ÛÒÌ·ÙÔ˜, ηıÒ˜ Î·È Ì ¤ÓÙÔÓË ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· ÔÊÂÈÏfiÌÂÓË ÛÙËÓ ‡·ÚÍË ÂÓ‰ÔÌ‹ÙÚÈÔ˘ ¯˘ÏÔıÒڷη, Ô ÔÔ›Ô˜ Ô‰‹ÁËÛ Û ˘ÔÏ·Û›· ÙÔ˘ Ó¢ÌÔÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜. ø˜ Èı·Ó‹ ·ÈÙ›· ÌÂÏÂÙ¿Ù·È Ë ‡·ÚÍË ÁÂÓÂÙÈο ÎÏËÚÔÓÔÌÔ‡ÌÂÓ˘ ıÚÔÌ‚ÔÊÈÏ›·˜, ›Ûˆ˜ ÛÂ Û˘Ó‰˘·ÛÌfi ÌÂ Û˘Óı‹Î˜ ÂÓ‰ÔÌËÙÚ›Ô˘ ÚÔıÚÔÌ‚ˆÙÈÎÔ‡ ÂÚÈ‚¿ÏÏÔÓÙÔ˜, Ì ÌfiÓÔ ÛÙÔÈ¯Â›Ô ·fi ÙÔ ÈÛÙÔÚÈÎfi ÙËÓ Èı·Ó‹ ‡·ÚÍË Ïԛ̈͢ Ï›Á˜ Ë̤Ú˜ ÚÔ ÙÔ˘ ÙÔÎÂÙÔ‡. √È ÁÔÓ›˜ ‰ÂÓ ‰¤¯ıËÎ·Ó Ó· Á›ÓÂÈ ¤ÏÂÁ¯Ô˜ ıÚÔÌ‚ÔÊÈÏ›·˜ ÛÙÔ˘˜ ›‰ÈÔ˘˜, ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰Ôı› ÁÂÓÂÙÈ΋ Û˘Ì‚Ô˘Ï‹ ÁÈ· Èı·Ó¿ ÂfiÌÂÓ· ΢‹Ì·Ù·.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Winnie GB. Chylothorax. In: Behrman RE, Kliegman RM, Jenson HB, editors. ¡elson Textbook of Pediatrics. 17th ed. International edition: Saunders; 2004. p. 1466.

2. Kramer SS, Taylor GA, Garfinkel DJ, Simmons MA. Lethal chylothoraces due to superior vena cava thrombosis in infants. Am J Roentgenol 1981;137:559-563.

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3. Dittrich S, Schlensak C, Kececioglu D. Successful thrombectomy of the superior vena cava thrombosis in a newborn after cardiopulmonary bypass surgery. Interact Cardiovasc Thorac Surg 2003;2:692-693.

4. Warren WH, Altman SAG. Chylothorax secondary to obstruction of the superior vena cava: a complication of the LeVeen shunt. Thorax 1990; 45:978-979.

5. King PA, Ghosh A, Tang MH, Lam SK. Recurrent congenital chylothorax. Prenat Diagn 1991;11:809-811.

6. Coulter DM. Hydrops fetalis. In: Spitzer AR, editor.

Intensive care of the fetus and neonate. 2nd ed. Philadelphia: Elsevier Mosby; 2005. p. 149-157.

7. Kolben M, Zimmermann A, Schneider KT. Acute nonimmunologic hydrops fetalis with bilateral chylothorax in the 36th week of pregnancy. Geburtshilfe Frauenheilkd.

1992;52:698-701.

8. Stevenson DA, Pysher TJ, Ward RM, Carey JC. Familial congenital non-immune hydrops, chylothorax, and pulmonary lymphangiectasia. Am J Med Genet A 2006; 140:368-372.

9. Moerman P, Vandenberghe K, Devlieger H, Van Hole C, Fryns JP, Lauweryns JM. Congenital pulmonary lymphangiectasis with chylothorax: a heterogeneous lymphatic vessel abnormality. Am J Med Genet 1993;47: 54-58.

10. Bellini C, Mazzella M, Arioni C, Campisi C, Taddei G, Toma P, et al. Hennekam syndrome presenting as nonimmune hydrops fetalis, congenital chylothorax, and congenital pulmonary lymphangiectasia. Am J Med Genet A 2003;120:92-96.

11. Hanssler L, Metz KA, Roll C, Hennecke KH. Primary lymphatic dysplasia in a newborn infant. Montsschr Kinderheilkd 1990;138:772-774.

12. Kessel I, Makhoul IR, Suvoj P. Congenital hypothyroidism and nonimmune hydrops fetalis: associated? Pediatrics 1999;103:E9.

13. Ro PS, Hill SL, Cheatham JP. Congenital superior vena cava obstruction causing anasarca and respiratory failure in a newborn: successful transcatheter therapy. Catheter Cardiovasc Interv 2005;65:60-65.

14. Römer S, Opgen-Rhein B, Chaoui R, Scheer I, Czernik C, Obladen M. Bilateral agenesis of the superior vena cava associated with congenital hydrothorax. Ultrasound Obstet Gynecol 2006;28:842-844.

15. Lee CY, Jan SL, Wang TM, Chi CS. Congenital chylothorax associated with isolated congenital hypoplastic superior caval vein: a case report. Acta Paediatr 2005;94:1840-1843.

16. Smorgick N, Herman A, Wiener Y, Halperin R, Sherman D. Prenatal thrombosis of the inferior vena cava and the renal veins. Prenat Diagn 2007;27:603-607.

17. Boffa MC, Lachassinne E. Infant perinatal thrombosis and antiphospholipid antibodies: a review. Lupus 2007;16: 634-641.

18. Khan JU, Takemoto CM, Casella JF, Streiff MB, Nwankwo IJ, Kim HS. Catheter-directed thrombolysis of inferior vena cava thrombosis in a 13-day-old nenate and review of literature. Cardiovasc Intervent Radiol 2008;31:153-160.

19. Messinger Y, Watterson Sheaffer J, Mrozek J, Smith CM, Sinaiko AR. Renal outcome of neonatal renal venous thrombosis: review of 28 patients and effectiveness of fibrinolytics and heparin in 10 patients. Pediatrics 2006; 118:1478-1484.

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20. Formstone CJ, Hallam PJ, Tuddenham EGD, Voke J, Layton M, Nicolaides K, et al. Severe perinatal thrombosis in double and triple heterozygous offspring of a family segregating two independent protein S mutations and a protein C mutation. Blood 1996;87:3731-3737.

21. Florell SR, Rodgers GM. Inherited thrombotic disorders: an update. Am J Hematol 1997;54:53-60.

22. Pfluger T, Czekalla R, Hundt C, Schubert M, Graubner U,

Leinsinger G, et al. MR angiography versus color Doppler sonography in the evaluation of renal vessels and the inferior vena cava in abdominal masses of pediatric patients. AJR Am J Roentgenol 1999;173:103-108.

23. Kobayashi T, Kobayashi T, Mayuzumi H, Morikawa A. Percutaneous hydrodynamic thrombectomy for congenital deep vein thrombosis in a neonate. Pediatr Cardiol 2006;27:170-174.

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¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌÂ›Ô ∞ÏÂÍ·Ó‰ÚÔ‡ÔÏ˘, ∞ÏÂÍ·Ó‰ÚÔ‡ÔÏË

∂. ª·ÓÙ·‰¿Î˘, Õ. ∆Û·ÏΛ‰Ë˜, Õ. ƒ·Ì·Ù¿ÓË, ∂. ¶·Ú·Ûο΢, ∞. ÷Ù˙ËÌȯ·‹Ï

AÏÏËÏÔÁÚ·Ê›·: ∂ÏȉÔÊfiÚÔ˜ ª·ÓÙ·‰¿Î˘ emantada@med.duth.gr ¢ËÌÔÎÚ›ÙÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £Ú¿Î˘, ∞ÏÂÍ·Ó‰ÚÔ‡ÔÏË

Department of Paediatrics, University Hospital of Alexandroupolis, Alexandroupolis, Greece Correspondence: Elpidoforos Mantadakis emantada@med.duth.gr Democritus University of Thrace, Alexandroupolis, Greece

CLINICAL QUIZ

E. Mantadakis, A. Tsalkidis, A. Ramatani, E. Paraskakis, A. Chatzimichael ∞ÁfiÚÈ ËÏÈΛ·˜ 38 ËÌÂÚÒÓ ÚÔÛÎÔÌ›˙ÂÙ·È ÌÈ· ‰ÚÔÛÂÚ‹ ηÏÔηÈÚÈÓ‹ Ë̤ڷ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ÏfiÁˆ ÚÔ˘ÎÂÙÔÂȉÒÓ ÂÌÂÙÒÓ ·fi 15Ë̤ÚÔ˘ Î·È ÌË ÚfiÛÏ˄˘ ‚¿ÚÔ˘˜. ∆Ô ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi Â›Ó·È ÂχıÂÚÔ, ÂÓÒ ÙÔ ‚Ú¤ÊÔ˜ Â›Ó·È ÙÔ ‰Â‡ÙÂÚÔ ·È‰› Ê·ÈÓÔÙ˘Èο ˘ÁÈÒÓ ÁÔÓ¤ˆÓ, ·Ï‚·ÓÈ΋˜ ηٷÁˆÁ‹˜. ∏ ÙÚ›¯ÚÔÓË ·‰ÂÏÊ‹ ÙÔ˘ Â›Ó·È ˘ÁÈ‹˜. √È ÂÌÂÙÔ› ÍÂΛÓËÛ·Ó ÂÓÒ ÙÔ ‚Ú¤ÊÔ˜ ÛÈÙÈ˙fiÙ·Ó ·ÔÎÏÂÈÛÙÈο Ì ÌËÙÚÈÎfi Á¿Ï·, ·ÏÏ¿ Û˘Ó¯›ÛÙËÎ·Ó Î·È ÌÂÙ¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÂÍ·ÓıÚˆÈṲ̂ÓÔ˘ Á¿Ï·ÙÔ˜ ˆ˜ Û˘ÌÏËÚÒÌ·ÙÔ˜ ÛÙË ‰È·ÙÚÔÊ‹. √È ÎÂÓÒÛÂȘ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ÂÚÈÁÚ¿ÊÔÓÙ·È ˆ˜ Ê˘ÛÈÔÏÔÁÈΤ˜. ™ÙËÓ ÂÈÛ·ÁˆÁ‹, ÙÔ ‚Ú¤ÊÔ˜ ˙˘Á›˙ÂÈ 2.750 g, fiÛÔ Î·È ÛÙË Á¤ÓÓËÛË, Ô˘ ¤ÁÈÓ ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ÙÔÎÂÙfi ÌÂÙ¿ ·fi ·ËÛË ‰È¿ÚÎÂÈ·˜ 40 ‚‰ÔÌ¿‰ˆÓ. ∫·Ù¿ ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË, ÙÔ ·È‰› Â›Ó·È ÛÔ‚·Ú¿ ·ÈÛ¯Ó·Ṳ̂ÓÔ, Ì ÌÂȈ̤ÓË ·Ú·ÁˆÁ‹ Ô‡ÚˆÓ Î·È ‰·ÎÚ‡ˆÓ, ·‰‡Ó·ÌÔ ÎÏ¿Ì·, ÌÂȈ̤ÓË Û·ÚÁ‹ ‰¤ÚÌ·ÙÔ˜ Î·È ÂÈÛ¤¯Ô˘Û· ÚfiÛıÈ· ËÁ‹. ∏ ·ÚÙËÚȷ΋ ›ÂÛË Â›Ó·È 85/50 mmHg, ÔÈ ÛÊ›ÍÂȘ 170/min Î·È Ë ıÂÚÌÔÎÚ·Û›· ÛÒÌ·ÙÔ˜ Ê˘ÛÈÔÏÔÁÈ΋. ∏ ÏÔÈ‹ ÂͤٷÛË ·Ó·‰ÂÈÎÓ‡ÂÈ Ì·ÚÌ·ÚÔÂȉ¤˜ ‰¤ÚÌ· ¯ˆÚ›˜ ÂÍ·Óı‹Ì·Ù·, ÂÓÒ Ë ÂͤٷÛË Ù˘ ηډȿ˜ Î·È ÙˆÓ Ó¢ÌfiÓˆÓ Â›Ó·È ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∏ ÎÔÈÏÈ¿ Â›Ó·È Â˘›ÂÛÙË, ¯ˆÚ›˜ Ë·ÙÔ-

ÛÏËÓÔÌÂÁ·Ï›·, Ì ηÏÔ‡˜ ÂÓÙÂÚÈÎÔ‡˜ ‹¯Ô˘˜. ∆· Ì·ÏÏÈ¿ Î·È Ù· Ó‡¯È· Â›Ó·È Ê˘ÛÈÔÏÔÁÈο. ∆· ÁÂÓÓËÙÈο fiÚÁ·Ó· Â›Ó·È Ê˘ÛÈÔÏÔÁÈο ¿ÚÚÂÓÔ˜ Î·È ÔÈ fiÚ¯ÂȘ Â›Ó·È ÛÙÔ fiÛ¯ÂÔ. §fiÁˆ Ù˘ ·Ê˘‰¿ÙˆÛ˘, Ô˘ ÎÏÈÓÈο ÂÎÙÈÌ‹ıËΠ۠10%, ÂÓ˘‰·ÙÒıËΠÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ÔÚfi, ÂÓ ·Ó·ÌÔÓ‹ ÙÔ˘ ·Ú¯ÈÎÔ‡ ÂÚÁ·ÛÙËÚÈ·ÎÔ‡ ÂϤÁ¯Ô˘, Ô˘ ¤‰ÂÈÍ ÏÂ˘Î¿ 10.160/mm3 Ì ÏÂÌÊÔ΢ÙÙ·ÚÈÎfi Ù‡Ô, ·ÈÌÔÛÊ·ÈÚ›ÓË 17,1 g/dl, ·ÈÌ·ÙÔÎÚ›ÙË 45%, ·ÈÌÔÂÙ¿ÏÈ· 567.000/mm3, CRP 0,1 mg/dl, ÁÏ˘Îfi˙Ë 69 mg/dl, Ô˘Ú›· 27 mg/dl, ÎÚ·ÙÈÓ›ÓË 0,5 mg/dl, Ó¿ÙÚÈÔ 104 mEq/l, οÏÈÔ 5,5 mEq/l, ¯ÏÒÚÈÔ 77 mEq/l, ·Û‚¤ÛÙÈÔ 10,2 mg/dl, ÊÒÛÊÔÚÔ 6 mg/dl, Ì·ÁÓ‹ÛÈÔ 2,1 mg/dl, SGOT 65 U/l, SGPT 74 U/l, ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË 315 U/l, Á-GT 444 U/l, LDH 435 U/l Î·È ÔÏÈ΋ ¯ÔÏÂÚ˘ıÚ›ÓË 1,2 mg/dl. ∞¤ÚÈ· ÊÏ‚ÈÎÔ‡ ·›Ì·ÙÔ˜ ¤‰ÂÈÍ·Ó pH 7,25, pCO2 38 mmHg, Ó¿ÙÚÈÔ 107 mEq/l Î·È ‰ÈÙÙ·ÓıÚ·ÎÈο 16,7 mmol/l.

¶Èı·Ó¤˜ ‰È·ÁÓÒÛÂȘ: 1. ¶˘ÏˆÚÈ΋ ÛÙ¤ÓˆÛË 2. πÓÔ΢ÛÙÈ΋ ÓfiÛÔ˜ 3. ™˘ÁÁÂÓ‹˜ ˘ÂÚÏ·Û›· ÂÈÓÂÊÚȉ›ˆÓ 4. ∞ÓȉڈÙÈ΋ ÂÎÙÔ‰ÂÚÌÈ΋ ‰˘ÛÏ·Û›· 5. ¢È·ÙÚÔÊÈÎfi ÛÊ¿ÏÌ· ÏfiÁˆ ·Ú·›ˆÛ˘ Á¿Ï·ÙÔ˜

∏ ·¿ÓÙËÛË ÛÙË ÛÂÏ›‰· 71

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CURRENT ISSUES OF BIOSTATISTICS

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¶ÚÔÔÙÈΤ˜ ÏËı˘ÛÌȷΤ˜ ÌÂϤÙ˜: Ë ÂÊ·ÚÌÔÁ‹ Ù˘ ÂȉËÌÈÔÏÔÁ›·˜ ÁÈ· ÙÔÓ Î·ıÔÚÈÛÌfi ÙˆÓ ·ÈÙÈÔÏÔÁÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ ∂. ∫ÚÈÙÛ¤ÏË, ¢.∞. ∫·ÊÂÙ˙‹˜ ¶ÂÚ›ÏË„Ë: √È ÚÔÔÙÈΤ˜ ÏËı˘ÛÌȷΤ˜ ÌÂϤÙ˜ Â›Ó·È Ô Î‡ÚÈÔ˜ ÙÚfiÔ˜ ÌÂϤÙ˘ Ô˘ ÂÊ·ÚÌfi˙ÂÙ·È ÁÈ· ÙËÓ ·ÍÈÔÏfiÁËÛË ·ÈÙÈÔÏÔÁÈÎÒÓ Û˘Û¯ÂÙ›ÛÂˆÓ ·Ó¿ÌÂÛ· ÛÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ Î·È ÛÙËÓ ÂΉ‹ÏˆÛË ÓÔÛËÌ¿ÙˆÓ. ¶·ÚÔ˘ÛÈ¿˙ÂÙ·È ·Ó·ÛÎfiËÛË Ù˘ ‰ÔÌ‹˜ Î·È Ù˘ ÌÂıÔ‰ÔÏÔÁ›·˜ ÙˆÓ ÚÔÔÙÈÎÒÓ ÏËı˘ÛÌÈ·ÎÒÓ ÌÂÏÂÙÒÓ, fiˆ˜ ·˘Ù¤˜ ÂÊ·ÚÌfi˙ÔÓÙ·È ÁÈ· ÙËÓ ·ÍÈÔÏfiÁËÛË ÙˆÓ ·ÈÙÈÔÏÔÁÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÏÔÈ̈‰ÒÓ Î·È ¯ÚfiÓÈˆÓ ÓÔÛËÌ¿ÙˆÓ. ∂ÈϤÔÓ, ·Ó·Ï‡ÔÓÙ·È ÂӉ›ÍÂȘ ÁÈ· ÙËÓ ÂÊ·ÚÌÔÁ‹ ÚÔÔÙÈÎÒÓ ÏËı˘ÛÌÈ·ÎÒÓ ÌÂÏÂÙÒÓ Î·È Ù· Ù˘¯fiÓ ÂȉËÌÈÔÏÔÁÈο ÛÊ¿ÏÌ·Ù· Ù· ÔÔ›· ‰˘Ó·ÙfiÓ Ó· ÚÔ·„Ô˘Ó Î·Ù¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘˜. ∆¤ÏÔ˜, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ú·ÎÙÈο ·Ú·‰Â›ÁÌ·Ù· ÁÈ· ÙËÓ ·ÍÈÔÏfiÁËÛË Ù˘ ·ÈÙÈÔÏÔÁÈ΋˜ Û˘Û¯¤ÙÈÛ˘ ·Ó¿ÌÂÛ· Û ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ Î·È ÛÙËÓ Â›ÙˆÛË ·È‰È·ÙÚÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ.

µ’ ¶·ÓÂÈÛÙËÌȷ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ AÏÏËÏÔÁÚ·Ê›·: ŒÏÂÓ· ∫ÚÈÙÛ¤ÏË ecritselis@yahoo.com µ’ ¶·ÓÂÈÛÙËÌȷ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

§¤ÍÂȘ ÎÏÂȉȿ: ¶ÚÔÔÙÈ΋ ÌÂϤÙË, ÂȉËÌÈÔÏÔÁ›·, ۯ‰ȷÛÌfi˜ ÌÂϤÙ˘, ·È‰È·ÙÚÈο ÓÔÛ‹Ì·Ù·.

Prospective cohort studies: the application of epidemiology in order to identify the potential determinants of paediatric diseases E. Critselis, D.A. Kafetzis Abstract: Prospective cohort studies constitute a primary means for elucidating aetiological associations between potential risk factors and the clinical manifestation of infectious and chronic diseases. A review of the structure and methodological design of prospective cohort studies within the context of investigating the determinants of infectious and chronic paediatric diseases is presented. In addition, recommended indications for the implementation of prospective cohort studies are discussed. Potential systematic epidemiological biases which may be inherently introduced are addressed. Finally, practical tutorials for evaluating the association between the exposure to risk factors and the occurrence of paediatric diseases are presented.

Second Department of Paediatrics, “P. & A. Kyriakou” Children’s Hospital, University of Athens, Athens, Greece Correspondence: Elena Critselis ecritselis@yahoo.com Second Department of Paediatrics, “P. & A. Kyriakou” Children’s Hospital, Athens, Greece

Key words: Prospective cohort study, epidemiology, study design, pediatric disease.

∂ÈÛ·ÁˆÁ‹ √ ۯ‰ȷÛÌfi˜ ÌÂϤÙ˘ ·ÔÙÂÏ› ÙÔÓ ·ÎÚÔÁˆÓÈ·›Ô Ï›ıÔ Ù˘ ¤Ú¢ӷ˜. ∏ ÔÚı‹ ÂÈÏÔÁ‹ ÙÔ˘ ηٿÏÏËÏÔ˘ ۯ‰ȷÛÌÔ‡ Ì ÛÙfi¯Ô ÙË ‰ÈÂÚ‡ÓËÛË ÂÈÛÙËÌÔÓÈÎÒÓ ˘Ôı¤ÛÂˆÓ ‰È·ÛÊ·Ï›˙ÂÈ ÙËÓ ÂÁ΢ÚfiÙËÙ· Î·È ÙËÓ ÔÈfiÙËÙ· ÌÈ·˜ ÂÚ¢ÓËÙÈ΋˜ ÚÔÛ¿ıÂÈ·˜ Î·È ÙˆÓ Â·ÎfiÏÔ˘ıˆÓ Û˘ÌÂÚ·ÛÌ¿ÙˆÓ. °È· ÙË ‰ÈÂÚ‡ÓËÛË Ù˘ ·ÈÙÈÔÏÔÁ›·˜ Î·È ÙˆÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ ·È‰È·ÙÚÈÎÒÓ ÏÔÈ̈‰ÒÓ Î·È ¯ÚfiÓÈˆÓ ÓÔÛËÌ¿ÙˆÓ, ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Ù¤ÛÛÂÚȘ ‚·ÛÈΤ˜ ÌÔÚʤ˜ ۯ‰ȷÛÌÔ‡ ÌÂϤÙ˘: (1) Ë ÚÔÔÙÈ΋ ‹ ·Ó·‰ÚÔÌÈ΋ ÏËı˘ÛÌȷ΋ ÌÂϤÙË (prospective or retrospective cohort study), (2) Ë ÌÂϤÙË ·ÛıÂÓÒÓ-Ì·ÚÙ‡ÚˆÓ (case-control study), (3) Ë Û˘Á¯ÚÔÓÈ΋ ÌÂϤÙË (cross-sectional study) Î·È (4) Ë ÎÏÈÓÈ΋ ‰ÔÎÈÌ·Û›· (randomized trial). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ·Ó·ÛÎÔÔ‡ÓÙ·È Ë ‰ÔÌ‹ Î·È Ë ÌÂıÔ‰ÔÏÔÁ›· ÙˆÓ ÚÔÔÙÈÎÒÓ

ÏËı˘ÛÌÈ·ÎÒÓ ÌÂÏÂÙÒÓ, fiˆ˜ ·˘Ù¤˜ ÂÊ·ÚÌfi˙ÔÓÙ·È ÁÈ· ÙËÓ ·ÍÈÔÏfiÁËÛË ÙˆÓ ·ÈÙÈÔÏÔÁÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÏÔÈ̈‰ÒÓ Î·È ¯ÚfiÓÈˆÓ ÓÔÛËÌ¿ÙˆÓ. ∂ÈϤÔÓ, ·Ó·Ï‡ÔÓÙ·È ÂӉ›ÍÂȘ ÁÈ· ÙËÓ ÂÊ·ÚÌÔÁ‹ ÚÔÔÙÈÎÒÓ ÏËı˘ÛÌÈ·ÎÒÓ ÌÂÏÂÙÒÓ, Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· Î·È ÌÂÈÔÓÂÎÙ‹Ì·Ù· ÙÔ˘ ۯ‰ȷÛÌÔ‡ ÌÂϤÙ˘ Î·È Ù· Ù˘¯fiÓ ÂȉËÌÈÔÏÔÁÈο ÛÊ¿ÏÌ·Ù· Ù· ÔÔ›· ‰˘Ó·ÙfiÓ Ó· ÚÔ·„Ô˘Ó Î·Ù¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘˜. ∆¤ÏÔ˜, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ú·ÎÙÈο ·Ú·‰Â›ÁÌ·Ù· ÁÈ· ÙËÓ ·ÍÈÔÏfiÁËÛË Ù˘ ·ÈÙÈÔÏÔÁÈ΋˜ Û˘Û¯¤ÙÈÛ˘ ·Ó¿ÌÂÛ· ÛÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ Î·È ÛÙËÓ Â›ÙˆÛË ·È‰È·ÙÚÈÎÒÓ ÓÔÛËÌ¿ÙˆÓ (¶›Ó·Î·˜ 1).

™ÎÔfi˜ ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ µ·ÛÈÎfi˜ ÛÎÔfi˜ οı ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ Â›Ó·È Ë ·ÍÈÔÏfiÁËÛË Ù˘ ¶·È‰È·ÙÚÈ΋ 2009;72:67-69

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·68

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∂. ∫ÚÈÙÛ¤ÏË, ¢.∞. ∫·ÊÂÙ˙‹˜

¶›Ó·Î·˜ 1. ¶·Ú¿‰ÂÈÁÌ· ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ ªÈ· ÚÔÔÙÈ΋ ÏËı˘ÛÌȷ΋ ÌÂϤÙË ı· ÌÔÚÔ‡Û ӷ ¤¯ÂÈ ˆ˜ ÛÎÔfi ÙËÓ ·ÍÈÔÏfiÁËÛË Ù˘ Â›‰Ú·Û˘ ÙÔ˘ ·ıËÙÈÎÔ‡ ηÓ›ÛÌ·ÙÔ˜ ÛÙËÓ Â›ÙˆÛË ‚ÚÔÁ¯ÈÎÔ‡ ¿ÛıÌ·ÙÔ˜ ÛÙ· ·È‰È¿.

1. ∆Ô ÏËı˘ÛÌÈ·Îfi ‰Â›ÁÌ· Ù˘ ÌÂϤÙ˘ ı· Ú¤ÂÈ Ó· ·ÔÙÂÏÂ›Ù·È ·fi ¤Ó· Ù˘¯·›Ô ‰Â›ÁÌ· ·È‰ÈÒÓ, 4-6 ÂÙÒÓ, ¯ˆÚ›˜ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ‹/Î·È Û˘ÌÙÒÌ·Ù· ‚ÚÔÁ¯ÈÎÔ‡ ¿ÛıÌ·ÙÔ˜, ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ÌÂϤÙ˘.

2. ∏ ÔÌ¿‰· Ì·ÚÙ‡ÚˆÓ Ù˘ ÌÂϤÙ˘ ı· ÂÚÈÏ·Ì‚¿ÓÂÈ ·È‰È¿ Ù· ÔÔ›· ‰ÂÓ ¤¯Ô˘Ó ÂÎÙÂı› ÔÙ¤ Û ·ıËÙÈÎfi οÓÈÛÌ·.

3. ∏ ÚÒÙË ÔÌ¿‰· Û‡ÁÎÚÈÛ˘ ı· ÂÚÈÏ·Ì‚¿ÓÂÈ ·È‰È¿ Ô˘ ¤¯Ô˘Ó ÂÎÙÂı› ÌfiÓÔ ÂÚÈÛÙ·Ûȷο Û ·ıËÙÈÎfi οÓÈÛÌ·.

4. ∏ ‰Â‡ÙÂÚË ÔÌ¿‰· Û‡ÁÎÚÈÛ˘ ı· ÂÚÈÏ·Ì‚¿ÓÂÈ ·È‰È¿ Ô˘ ¤¯Ô˘Ó ÂÎÙÂı› Û˘ÛÙËÌ·ÙÈο Û ·ıËÙÈÎfi οÓÈÛÌ·.

5. √ ÂÚ¢ÓËÙ‹˜ Ú¤ÂÈ Ó· ·Ú·ÎÔÏÔ˘ı‹ÛÂÈ ‰È·¯ÚÔÓÈο ·˘Ù¤˜ ÙȘ ÙÚÂȘ ÔÌ¿‰Â˜ ̤¯ÚÈ ÙËÓ ÂÊ˂›·, ÒÛÙ ӷ ηٷÁÚ¿„ÂÈ Î·È Ó· Û˘ÁÎÚ›ÓÂÈ ÙËÓ Â›ÙˆÛË ‚ÚÔÁ¯ÈÎÔ‡ ¿ÛıÌ·ÙÔ˜ ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ Ô˘ ¤¯Ô˘Ó ÂÎÙÂı› ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘, ˆ˜ ÚÔ˜ ÙË ÌË ÂÎÙÂıÂÈ̤ÓË Û’ ·˘ÙfiÓ ÔÌ¿‰· Ì·ÚÙ‡ÚˆÓ.

6. ∏ ·ÈÙÈÔÏÔÁÈ΋ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ Ù˘ ¤ÎıÂÛ˘ ηٿ ÙËÓ ·È‰È΋ ËÏÈΛ· Û ·ıËÙÈÎfi οÓÈÛÌ· Î·È ÙÔ˘ ‚ÚÔÁ¯ÈÎÔ‡ ¿ÛıÌ·ÙÔ˜ ı· ‰ڷȈı› ÂÊfiÛÔÓ Ë Â›ÙˆÛË ÓfiÛÔ˘ Ô˘ ·Ú·ÙËÚÂ›Ù·È ÙfiÛÔ ÛÙËÓ ÚÒÙË fiÛÔ Î·È ÛÙË ‰Â‡ÙÂÚË ÔÌ¿‰· Û‡ÁÎÚÈÛ˘ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË ·fi ·˘Ù‹Ó Ô˘ ·Ú·ÙËÚÂ›Ù·È ÛÙËÓ ÔÌ¿‰· Ì·ÚÙ‡ÚˆÓ.

·ÈÙÈÔÏÔÁÈ΋˜ Û˘Û¯¤ÙÈÛ˘ ·Ó¿ÌÂÛ· Û ¤Ó·Ó ‹ ÂÚÈÛÛfiÙÂÚÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ (risk factors) Î·È ÙËÓ Â›ÙˆÛË Ù˘ ÓfiÛÔ˘ (incidence rate) ‹ ÙËÓ ÂͤÏÈÍ‹ Ù˘. √È ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ÌÔÚ› Ó· Û˘ÌÂÚÈÏ·Ì‚¿ÓÔ˘Ó ¤Ó· ¢ڇ Ê¿ÛÌ· ·Ú·Ì¤ÙÚˆÓ, fiˆ˜ ·Ú¿ÁÔÓÙ˜ ‰ËÌÔÁÚ·ÊÈÎÔ‡˜, ÎÔÈÓˆÓÈÎÔ-ÔÈÎÔÓÔÌÈÎÔ‡˜, ÂÚÈ‚·ÏÏÔÓÙÔÏÔÁÈÎÔ‡˜, ÁÂÓÂÙÈÎÔ‡˜ Î.Ï.

™¯Â‰È·ÛÌfi˜ Î·È ‰ÈÂÍ·ÁˆÁ‹ ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ °È· ÙËÓ Ú·ÎÙÈ΋ ÂÊ·ÚÌÔÁ‹ ÌÈ·˜ ÚÔÔÙÈ΋˜ ¤Ú¢ӷ˜, ÙÔ ‰Â›ÁÌ· Ù˘ ÌÂϤÙ˘ ·ÔÙÂÏÂ›Ù·È ·fi ÔÌ¿‰· ·ÙfiÌˆÓ Ô˘ ‰ÂÓ ¤¯Ô˘Ó ÂÎÙÂı› ÛÙÔÓ ÂÓ ÏfiÁˆ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ (Ì¿ÚÙ˘Ú˜, non-exposed group) Î·È Ì›· ÔÌ¿‰· ·ÙfiÌˆÓ Ô˘, ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ ÌÂϤÙ˘, ¤¯Ô˘Ó ‹‰Ë ÂÎÙÂı› ÛÙÔÓ ›‰ÈÔ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ (ÔÌ¿‰· Û‡ÁÎÚÈÛ˘, exposed group). ™ËÌÂÈÒÓÂÙ·È fiÙÈ, ÛÙÔÓ Û˘ÁÎÂÎÚÈ̤ÓÔ Û¯Â‰È·ÛÌfi ÌÂϤÙ˘, ÌÔÚÔ‡Ó Ó· ÂÍÂÙ¿˙ÔÓÙ·È Û˘Á¯ÚfiÓˆ˜ ‰‡Ô ‹ Î·È ÂÚÈÛÛfiÙÂÚ˜ ÔÌ¿‰Â˜ Û‡ÁÎÚÈÛ˘, fiˆ˜ ·˘Ù¤˜ ηıÔÚ›˙ÔÓÙ·È Â›Ù ·fi ÙËÓ ¤ÎıÂÛË Û ·ÓÂÍ¿ÚÙËÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘, ›Ù ·fi ÙËÓ ÔÛfiÛÙˆÛË Ù˘ ¤ÎıÂÛ˘ (.¯. ¯·ÌËÏ‹ ‹ ˘„ËÏ‹ ¤ÎıÂÛË) Û ¤Ó·Ó Û˘ÁÎÂÎÚÈ̤ÓÔ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘. ∂›Ó·È ÛËÌ·ÓÙÈÎfi Ó· ÙÔÓÈÛı› fiÙÈ Î·Ù¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ÌÂϤÙ˘, ÙfiÛÔ ÛÙËÓ ÔÌ¿‰· Ì·ÚÙ‡ÚˆÓ fiÛÔ Î·È ÛÙȘ ÔÌ¿‰Â˜ Û‡ÁÎÚÈÛ˘ ı· Ú¤ÂÈ Ó· ¤¯ÂÈ ·ÔÎÏÂÈÛı› Ë ·ÚÔ˘Û›· Ù˘ ÂÍÂÙ·˙fiÌÂÓ˘ ÓfiÛÔ˘ Û ˘ÔÎÏÈÓÈ΋, Ï·Óı¿ÓÔ˘Û· ‹ Paediatriki 2009;72:67-69

ÂÓÂÚÁfi ÌÔÚÊ‹. √È ÔÌ¿‰Â˜ ˘fi ÌÂϤÙË ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È ‰È·¯ÚÔÓÈο ÒÛÙ ӷ ηٷÁÚ·Ê›, Û οı ÔÌ¿‰· ͯˆÚÈÛÙ¿, Ë Â›ÙˆÛË, ıÓËÛÈÌfiÙËÙ· ‹ ıÓËÙfiÙËÙ· Ù˘ ÓfiÛÔ˘.

¶·ı‹ÛÂȘ Ô˘ ÂӉ›ÎÓ˘Ù·È Ó· ÌÂÏÂÙËıÔ‡Ó ™ÙÔ Ï·›ÛÈÔ ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÌÂÏÂÙËıÔ‡Ó Ë Â›ÙˆÛË, ıÓËÛÈÌfiÙËÙ· ‹ ıÓËÙfiÙËÙ· ÙfiÛÔ ÙˆÓ ¯ÚfiÓÈˆÓ fiÛÔ Î·È ÙˆÓ ÏÔÈ̈‰ÒÓ ·ı‹ÛˆÓ. ¶·ÚfiÏ· ·˘Ù¿, ‰‡Ô Ú·ÎÙÈο ÛËÌ›· Ú¤ÂÈ Ó· ÛËÌÂȈıÔ‡Ó. ¶ÚÒÙÔÓ, Û˘ÓÈÛÙ¿Ù·È Ë ·Ó·ÌÂÓfiÌÂÓË ¯ÚÔÓÈ΋ ‰È¿ÚÎÂÈ· ÌÂٷ͇ Ù˘ ¤ÎıÂÛ˘ ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ Î·È Ù˘ ÎÏÈÓÈ΋˜ ÂΉ‹ÏˆÛ˘ Ù˘ ÓfiÛÔ˘ Ó· Â›Ó·È Ë Û˘ÓÙÔÌfiÙÂÚË ‰˘Ó·ÙfiÓ. ¢Â‡ÙÂÚÔÓ, ÁÈ· ÏfiÁÔ˘˜ ÔÈÎÔÓÔÌ›·˜ ÎfiÛÙÔ˘˜ Ù˘ ÌÂϤÙ˘, Û˘ÓÈÛÙ¿Ù·È Ó· ÌËÓ ·ÍÈÔÏÔÁÔ‡ÓÙ·È Û¿ÓÈ· ÓÔÛ‹Ì·Ù·, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ÛÙËÓ ÂÚ›ÙˆÛË ·˘Ù‹ ı· Ú¤ÂÈ Ó· Û˘Ïϯı› Î·È Ó· ·Ú·ÎÔÏÔ˘ıËı› ÌÂÁ¿ÏÔ ÏËı˘ÛÌÈ·Îfi ‰Â›ÁÌ·, ÒÛÙ ӷ ηٷÁÚ·Ê› Â·Ú΋˜ ·ÚÈıÌfi˜ ÂÚÈÙÒÛÂˆÓ ÓfiÛÔ˘. ∂›Ó·È ÛÎfiÈÌÔ Ë ·ÍÈÔÏfiÁËÛË Û·Ó›ˆÓ ÓÔÛËÌ¿ÙˆÓ Ó· Á›ÓÂÙ·È Ì ˘ÏÈÎfi ·fi ÔÏ˘ÎÂÓÙÚÈΤ˜ ÌÂϤÙ˜. ¶ÏÂÔÓÂÎÙ‹Ì·Ù· Ù˘ ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ π‰È·›ÙÂÚ· ÛËÌ·ÓÙÈÎfi Â›Ó·È fiÙÈ, Ì ÙÔÓ Û˘ÁÎÂÎÚÈ̤ÓÔ Û¯Â‰È·ÛÌfi ÌÂϤÙ˘, Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÙÂÎÌËÚȈı› Ë ·ÈÙÈÔÏÔÁÈ΋ Û˘Û¯¤ÙÈÛË ¤ÎıÂÛ˘-ÓfiÛÔ˘, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ÂÍÂÙ¿˙ÔÓÙ·È Ó¤Â˜ ÂÚÈÙÒÛÂȘ ÛÙȘ Ôԛ˜ ¤¯ÂÈ ÚÔËÁËı› Ë ¤ÎıÂÛË ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘. ªÂÈÔÓÂÎÙ‹Ì·Ù· Ù˘ ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ ø˜ ÌÂÈÔÓ¤ÎÙËÌ· Ù˘ ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ ·Ó·Ê¤ÚÂÙ·È Ë Ì·ÎÚÔ¯ÚfiÓÈ· ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘, ηْ ·ÎÔÏÔ˘ı›· ÙÔ ˘„ËÏfi ÎfiÛÙÔ˜ Î·È Ë Èı·Ó‹ ·‰˘Ó·Ì›· ·Ú·ÎÔÏÔ‡ıËÛ˘ (lost to follow-up) ÙÔ˘ ÏËı˘ÛÌÈ·ÎÔ‡ ‰Â›ÁÌ·ÙÔ˜. ∂Ó·ÏÏ·ÎÙÈÎfi˜ ۯ‰ȷÛÌfi˜ ÁÈ· ÙÔÓ ÂÚÈÔÚÈÛÌfi Ù˘ ‰È¿ÚÎÂÈ·˜ Î·È ÙÔ˘ ÎfiÛÙÔ˘˜ ∂Ó·ÏÏ·ÎÙÈÎfi˜ ۯ‰ȷÛÌfi˜ Ù˘ ÚÔÔÙÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ Â›Ó·È ·˘Ùfi˜ Ù˘ ·Ó·‰ÚÔÌÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ (retrospective cohort study), ÛÙ· Ï·›ÛÈ· ÙÔ˘ ÔÔ›Ô˘ ÂÈϤÁÂÙ·È ˘ÁÈ‹˜ ÏËı˘ÛÌfi˜, Ô˘ ¤¯ÂÈ Î·ıÈÂÚˆı› ÛÙÔ ·ÚÂÏıfiÓ (historical cohort), fiˆ˜ .¯. ÌÈ· Ù¿ÍË Ì·ıËÙÒÓ ‰ËÌÔÙÈÎÔ‡ Û¯ÔÏ›Ԣ ÚÔËÁÔ‡ÌÂÓˆÓ ÂÙÒÓ. ∏ ·ÍÈÔÏfiÁËÛË Ù˘ ¤ÎıÂÛ˘ ÙˆÓ ÂÍÂÙ·˙fiÌÂÓˆÓ ÛÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ Á›ÓÂÙ·È ‚¿ÛÂÈ È·ÙÚÈÎÒÓ ‹ ¿ÏÏˆÓ ·Ú¯Â›ˆÓ. ∆· ̤ÏË ÙÔ˘ ÏËı˘ÛÌÔ‡ ·Ó¢ڛÛÎÔÓÙ·È, ÛÙËÓ ·ÚÔ‡Û· ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô Î·È Û ÌÂÙ·ÁÂÓ¤ÛÙÂÚË ËÏÈΛ·, ÒÛÙÂ

Pediatric Jan-Feb 09

16-03-09

14:56

™ÂÏ›‰·69

69

¶ÚÔÔÙÈΤ˜ ÏËı˘ÛÌȷΤ˜ ÌÂϤÙ˜

Ó· ηٷÁÚ·Ê› Ë Â›ÙˆÛË ÓfiÛÔ˘. ªÂ ÙËÓ ÂÊ·ÚÌÔÁ‹ ·˘ÙÔ‡ ÙÔ˘ ۯ‰ȷÛÌÔ‡ ÌÂÈÒÓÂÙ·È ÛËÌ·ÓÙÈο Ë ¯ÚÔÓÈ΋ ‰È¿ÚÎÂÈ· Î·È ÙÔ ÎfiÛÙÔ˜ Ù˘ ÌÂϤÙ˘. ™ËÌ·ÓÙÈÎfi Â›Ó·È Ó· ÛËÌÂȈı› fiÙÈ, Ë ‚·ÛÈ΋ ‰ÔÌ‹ Ù˘ ·Ó·‰ÚÔÌÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘ ‰ÂÓ ‰È·Ê¤ÚÂÈ ·fi ·˘Ù‹Ó Ù˘ ÚÔÔÙÈ΋˜ ÌÂϤÙ˘. ™˘ÁÎÂÎÚÈ̤ӷ, Î·È ÛÙȘ ‰‡Ô ÌÂϤÙ˜ Û˘ÁÎÚ›ÓÔÓÙ·È Ë ÔÌ¿‰· Ô˘ ¤¯ÂÈ ÂÎÙÂı› ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ÚÔ˜ ÙËÓ ÔÌ¿‰· Ô˘ ‰ÂÓ ¤¯ÂÈ ÂÎÙÂı› Û’ ·˘ÙfiÓ, ˆ˜ ÚÔ˜ ÙËÓ Â›ÙˆÛË Ù˘ ÓfiÛÔ˘. ∏ Û‡ÁÎÚÈÛË ·˘Ù‹ ·ÔÙÂÏ› Î·È ÙËÓ È‰È·›ÙÂÚË Ù·˘ÙfiÙËÙ· οı ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘.

¶Èı·Ó¿ Û˘ÛÙËÌ·ÙÈο ÛÊ¿ÏÌ·Ù· ÌÂϤÙ˘ ∫·Ù¿ ÙË ‰ÈÂÍ·ÁˆÁ‹ ÌÈ·˜ ÚÔÔÙÈ΋˜ ‹ ·Ó·‰ÚÔÌÈ΋˜ ÏËı˘ÛÌȷ΋˜ ÌÂϤÙ˘, ÂÓ‰¤¯ÂÙ·È Ó· ÚÔ·„Ô˘Ó Ù· ·ÎfiÏÔ˘ı· Û˘ÛÙËÌ·ÙÈο ÛÊ¿ÏÌ·Ù·.

1. ™Ê¿ÏÌ· ·ÍÈÔÏfiÁËÛ˘ ·ÔÙÂϤÛÌ·ÙÔ˜ (bias in the assessment of outcome) ∂Ó‰¤¯ÂÙ·È Ó· ÚÔ·„ÂÈ fiÙ·Ó Ô ÂÚ¢ÓËÙ‹˜ Ô˘ ·ÍÈÔÏÔÁ› ÙËÓ Â›ÙˆÛË ÓfiÛÔ˘ ÁÓˆÚ›˙ÂÈ ÔÈ· ¿ÙÔÌ· ¤¯Ô˘Ó ÂÎÙÂı› ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘, ÔfiÙ ÂËÚ¿˙ÂÙ·È Ë ÎÚ›ÛË ÙÔ˘. ∞˘Ùfi ÙÔ ÛÊ¿ÏÌ· ÌÔÚ› Ó· ·ÔÊ¢¯ı› ÂÊfiÛÔÓ Â›ÙÂ Ô ÂÚ¢ÓËÙ‹˜ ‰ÂÓ ÂÓËÌÂÚˆı› Û¯ÂÙÈο Ì ÙËÓ ¤ÎıÂÛË ÙˆÓ ·ÙfiÌˆÓ ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ (Ù˘ÊÏ‹ ·ÍÈÔÏfiÁËÛË, blind assessment) ›Ù ÎÏËıÔ‡Ó ‰‡Ô ‰È·ÊÔÚÂÙÈÎÔ› ÂÚ¢ÓËÙ¤˜ Ó· ·ÍÈÔÏÔÁ‹ÛÔ˘Ó ÙËÓ Â›ÙˆÛË ÓfiÛÔ˘ ·ÓÂÍ¿ÚÙËÙ·.

2. ™Ê¿ÏÌ· ÏËÚÔÊfiÚËÛ˘ (information bias) ∂Ó‰¤¯ÂÙ·È Ó· ÚÔ·„ÂÈ Û ÂÚÈÙÒÛÂȘ ÛÙȘ Ôԛ˜ Ë ¤ÎÙ·ÛË Î·È Ë ÔÈfiÙËÙ· ÙˆÓ ÏËÚÔÊÔÚÈÒÓ ·fi ÙÔ ÏËı˘ÛÌÈ·Îfi ‰Â›ÁÌ· ‰È·Ê¤ÚÂÈ ÛËÌ·ÓÙÈο, ·Ó¿ÏÔÁ· Ì ÙÔ ·Ó ÔÈ Û˘ÌÌÂÙ¤¯ÔÓÙ˜ ÛÙË ÌÂϤÙË ¤¯Ô˘Ó ÂÎÙÂı› ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘. ∆Ô ÛÊ¿ÏÌ· ÏËÚÔÊfiÚËÛ˘ Â›Ó·È ÈÔ Èı·Ófi Ó· ÚÔ·„ÂÈ Î·Ù¿ ÙË ‰ÈÂÍ·ÁˆÁ‹ ·Ó·‰ÚÔÌÈÎÒÓ ÏËı˘ÛÌÈ·ÎÒÓ ÌÂÏÂÙÒÓ.

3. ™Ê¿ÏÌ· ÏfiÁˆ ·‰˘Ó·Ì›·˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ (bias from non-responses and lost to follow-up) ∂ËÚ¿˙ÂÈ ¿ÌÂÛ· ÙËÓ ·ÍÈÔÈÛÙ›· Ù˘ ÌÂϤÙ˘, ‰Â‰Ô̤ÓÔ˘ Ù˘ ·‰˘Ó·Ì›·˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ ·ÙfiÌˆÓ Ô˘ ÓÔÛÔ‡Ó, Ì Â·ÎfiÏÔ˘ıÔ ÙÔÓ Ï·Óı·Ṳ̂ÓÔ ˘ÔÏÔÁÈÛÌfi Ù˘ Â›ÙˆÛ˘ ÓfiÛÔ˘.

∂ȉËÌÈÔÏÔÁÈΤ˜ Î·È ‚ÈÔÛÙ·ÙÈÛÙÈΤ˜ ·Ú¿ÌÂÙÚÔÈ ∫‡ÚÈÔ˜ ÛÎÔfi˜ οı ÚÔÔÙÈ΋˜ ÌÂϤÙ˘ Â›Ó·È Ë ÛÙ·ÙÈÛÙÈ΋ ‰ڷ›ˆÛË Ù˘ ·ÈÙÈÔÏÔÁÈ΋˜ Û˘Û¯¤ÙÈÛ˘ ·Ó¿ÌÂÛ· Û ¤Ó·Ó ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ Î·È ÙËÓ ˘fi ÌÂϤÙË ÓfiÛÔ. °È· ÙÔÓ ÛÎÔfi ·˘Ùfi, Á›ÓÔÓÙ·È ÔÈ ·ÎfiÏÔ˘ıÔÈ ˘ÔÏÔÁÈÛÌÔ›, Ì ‚¿ÛË ÙÔÓ ¶›Ó·Î· 2.

¶›Ó·Î·˜ 2. ÀÔıÂÙÈ΋ ηٷÓÔÌ‹ ÏËı˘ÛÌÔ‡ ÚÔÔÙÈ΋˜ ÌÂϤÙ˘

ŒÎıÂÛË ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ªË ¤ÎıÂÛË ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘

¶·ÚÔ˘Û›· ÓfiÛÔ˘

∞Ô˘Û›· ÓfiÛÔ˘

∂›ÙˆÛË ÓfiÛÔ˘

a

b

πÔÛ = a / (a + b)

c

d

πÔÌ = c / (c+d)

∂›ÙˆÛË ÓfiÛÔ˘ ÛÙËÓ ÔÌ¿‰· Û‡ÁÎÚÈÛ˘ (πÔÛ) ∂›Ó·È Ô ÏfiÁÔ˜ ÙˆÓ Ó¤ˆÓ ÂÚÈÙÒÛÂˆÓ ÓfiÛÔ˘ Û ¿ÙÔÌ· Ô˘ ¤¯Ô˘Ó ÂÎÙÂı› ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ÚÔ˜ ÙÔ Û‡ÓÔÏÔ ÙˆÓ ·ÙfiÌˆÓ Ô˘ ¤¯Ô˘Ó ÂÎÙÂı› Û’·˘ÙfiÓ: πÔÛ = a / (a+b). ∂›ÙˆÛË ÓfiÛÔ˘ ÛÙËÓ ÔÌ¿‰· Ì·ÚÙ‡ÚˆÓ (πÔÌ) ∂›Ó·È Ô ÏfiÁÔ˜ ÙˆÓ Ó¤ˆÓ ÂÚÈÙÒÛÂˆÓ ÓfiÛÔ˘ Û ¿ÙÔÌ· Ô˘ ‰ÂÓ ¤¯Ô˘Ó ÂÎÙÂı› ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ÚÔ˜ ÙÔ Û‡ÓÔÏÔ ÙˆÓ ·ÙfiÌˆÓ Ô˘ ‰ÂÓ ¤¯Ô˘Ó ÂÎÙÂı› Û’·˘ÙfiÓ: πÔÌ = c / (c+d). ™¯ÂÙÈÎfi˜ ∫›Ó‰˘ÓÔ˜ (Relative Risk, RR) ∂›Ó·È Ô ÏfiÁÔ˜ Ù˘ Â›ÙˆÛ˘ ÓfiÛÔ˘ ÛÙËÓ ÔÌ¿‰· Û‡ÁÎÚÈÛ˘ (ÂÎÙÂıÂÈ̤ÓÔ˘˜) ÚÔ˜ ÙËÓ Â›ÙˆÛË ÛÙËÓ ÔÌ¿‰· Ì·ÚÙ‡ÚˆÓ (ÌË ÂÎÙÂıÂÈ̤ÓÔ˘˜): RR = πÔÛ / πÔÌ. ∂¿Ó Ë ÙÈÌ‹ ÙÔ˘ Û¯ÂÙÈÎÔ‡ ÎÈÓ‰‡ÓÔ˘ Â›Ó·È ›ÛË Ì ÙË ÌÔÓ¿‰· (RR=1), ÙfiÙÂ Ô Î›Ó‰˘ÓÔ˜ (Â›ÙˆÛË) ÓfiÛÔ˘ Û ÂÎÙÂıÂÈ̤ӷ ¿ÙÔÌ· ÈÛÔ‡Ù·È Ì ÙÔÓ Î›Ó‰˘ÓÔ ÓfiÛÔ˘ Û ÌË ÂÎÙÂıÂÈ̤ӷ ¿ÙÔÌ·, ‰ËÏ·‰‹ ‰ÂÓ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ·ÈÙÈÔÏÔÁÈ΋˜ Û˘Û¯¤ÙÈÛ˘ ·Ó¿ÌÂÛ· ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ Î·È ÙËÓ Â›ÙˆÛË ÓfiÛÔ˘. ∂¿Ó Ë ÙÈÌ‹ ÙÔ˘ Û¯ÂÙÈÎÔ‡ ÎÈÓ‰‡ÓÔ˘ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË ·fi ÙË ÌÔÓ¿‰· (RR>1), ÙfiÙÂ Ô Î›Ó‰˘ÓÔ˜ ÓfiÛÔ˘ Û ÂÎÙÂıÂÈ̤ӷ ¿ÙÔÌ· Â›Ó·È ÌÂÁ·Ï‡ÙÂÚÔ˜ ·fi ·˘ÙfiÓ Û ÌË ÂÎÙÂıÂÈ̤ӷ ¿ÙÔÌ·, ÔfiÙ ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË ıÂÙÈ΋˜ ·ÈÙÈÔÏÔÁÈ΋˜ Û˘Û¯¤ÙÈÛ˘ ·Ó¿ÌÂÛ· ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ Î·È ÙËÓ Â›ÙˆÛË ÓfiÛÔ˘. ∆¤ÏÔ˜, Â¿Ó Ë ÙÈÌ‹ ÙÔ˘ Û¯ÂÙÈÎÔ‡ ÎÈÓ‰‡ÓÔ˘ Â›Ó·È ÌÈÎÚfiÙÂÚË ·fi ÙË ÌÔÓ¿‰· (RR<1), ÙfiÙÂ Ô Î›Ó‰˘ÓÔ˜ ÓfiÛÔ˘ Û ÂÎÙÂıÂÈ̤ӷ ¿ÙÔÌ· Â›Ó·È ÌÈÎÚfiÙÂÚÔ˜ ·fi ·˘ÙfiÓ Û ÌË ÂÎÙÂıÂÈ̤ӷ ¿ÙÔÌ·, ‰ËÏ·‰‹ ˘¿Ú¯ÂÈ ·ÚÓËÙÈ΋ (ÚÔÛٷ٢ÙÈ΋) Û˘Û¯¤ÙÈÛË ·Ó¿ÌÂÛ· ÛÙÔÓ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ Î·È ÙËÓ Â›ÙˆÛË ÓfiÛÔ˘.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Gordis L. 1996. Epidemiology. WB Saunders Company.

Pennsylvania, USA. 2. Rothman KJ, Greenland S. 1998. Modern Epidemiology, Second Edition. Lippincott Williams & Wilkins, Pennsylvania, USA. 3. Szklo M, Nieto FJ. 2000. Epidemiology: Beyond the Basics.

Aspen Publishers. Maryland, USA. ¶·È‰È·ÙÚÈ΋ 2009;72:67-69

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¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√

NEWS FROM THE INTERNET

http://www.paidiatros.com ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¡ÔÛÔÎÔÌ›Ԣ §¿Úӷη˜, §¿Úӷη, ∫‡ÚÔ˜ AÏÏËÏÔÁÚ·Ê›·: ∞‰¿ÌÔ˜ ÷Ù˙Ë·Ó·Á‹˜ adamos@paidiatros.com ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¡ÔÛÔÎÔÌÂ›Ô §¿Úӷη˜ 6301, §¿Úӷη ∫‡ÚÔ˜

Pediatric Department, Larnaca General Hospital, Larnaca, Cyprus Correspondence: Adamos Hadjipanayis adamos@paidiatros.com Pediatric Department Larnaca General Hospital 6301, Larnaca Cyprus

Paediatriki 2009;72:70

∞. ÷Ù˙Ë·Ó·Á‹˜

∏ ÈÛÙÔÛÂÏ›‰· www.paidiatros.com ·¢ı‡ÓÂÙ·È ÛÙÔ˘˜ ÁÔÓ›˜, ·ÏÏ¿ Î·È Û Â·ÁÁÂÏ̷ٛ˜ ˘Á›·˜ (·È‰›·ÙÚÔ˘˜, ÁÂÓÈÎÔ‡˜ ÁÈ·ÙÚÔ‡˜ Î.Ù.Ï.). ™ÙËÓ Î‡ÚÈ· ÛÂÏ›‰· ı· ‚Ú›Ù ÙÔ «∂›Î·ÈÚÔ ı¤Ì·», ÙÔ ÔÔ›Ô Ú·ÁÌ·Ù‡ÂÙ·È Ù· ÙÚ¤¯ÔÓÙ· ı¤Ì·Ù· Ô˘ ··Û¯ÔÏÔ‡Ó ÙÔÓ ÁÔÓÈfi. ∆Ô ı¤Ì· ·˘Ùfi, ·Ó¿ÏÔÁ· Ì ÙË ÚÔ‹ ÙˆÓ Âȉ‹ÛˆÓ, ·Ó·ÓÂÒÓÂÙ·È Î¿ı 8-12 ̤Ú˜. ™ÙÔ Î˘Ú›ˆ˜ ÌÂÓÔ‡ ·ÚÈÛÙÂÚ¿, ÌÔÚ› Ó· ‚ÚÂÈ Ô ¯Ú‹ÛÙ˘ ÙȘ ηÙËÁÔڛ˜ ÙˆÓ ıÂÌ¿ÙˆÓ. ∫¿ı ηÙËÁÔÚ›· ÂÚÈÏ·Ì‚¿ÓÂÈ ÏËıÒÚ· ¿ÚıÚˆÓ Ô˘ ·Ó·Ï‡Ô˘Ó Ì ÙÚfiÔ ÂÎÏ·˚ÎÂ˘Ì¤ÓÔ ı¤Ì·Ù· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔ ·È‰› (·fi ÙË Û‡ÏÏË„Ë Ì¤¯ÚÈ Î·È ÙËÓ ÂÓËÏÈΛˆÛË). À¿Ú¯Ô˘Ó ›ÎÔÛÈ ÎÂÊ¿Ï·È· Ù· ÔÔ›· ÂÚȤ¯Ô˘Ó ÂÚÈÛÛfiÙÂÚ· ·fi 400 ‰È·ÊÔÚÂÙÈο ¿ÚıÚ·. ∏ Ì˯·Ó‹ ·Ó·˙‹ÙËÛ˘ οÓÂÈ ÙËÓ ·Ó‡ÚÂÛË ÙÔ˘ ¿ÚıÚÔ˘ Ô˘ „¿¯ÓÂÙ ¿Ú· Ôχ ‡ÎÔÏË. ∆·˘Ùfi¯ÚÔÓ·, fï˜, Â›Ó·È Î·Ù·¯ˆÚË̤ӷ fiÏ· Ù· ¿ÚıÚ· Û ·ÏÊ·‚ËÙÈ΋ ÛÂÈÚ¿. √ ÁÔÓÈfi˜ ¤¯ÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ÚˆÙ‹ÛÂÈ ÙËÓ Â›ÏÂÎÙË ÔÌ¿‰· ÂȉÈÎÒÓ ÁÈ· ÔÔÈÔ‰‹ÔÙ ı¤Ì· ÙÔÓ ··Û¯ÔÏ›, Ô˘ ·ÊÔÚ¿ ÙÔ ·È‰› ÙÔ˘. ∏ ÂÚÒÙËÛË ı· ··ÓÙËı› ÙÔ Û˘ÓÙÔÌfiÙÂÚÔ ‰˘Ó·ÙfiÓ ·fi ÙÔÓ ·ÚÌfi‰ÈÔ ÁÈ· ÙÔ ı¤Ì· ÂȉÈÎfi. ™ÙÔ ·Ú¯Â›Ô È·ÙÚÒÓ ˘¿Ú¯Ô˘Ó ηٷ¯ˆÚË̤ӷ Ù· ÛÙÔȯ›· fiÏˆÓ ÙˆÓ ·È‰È¿ÙÚˆÓ Ù˘ ∫‡ÚÔ˘ Î·È Û˘Ó·ÊÒÓ ÂȉÈÎÔÙ‹ÙˆÓ (·È‰Ô„˘¯ÔÏfiÁˆÓ, ÔÚıÔ‰ÔÓÙÈÎÒÓ Î.Ù.Ï.). ∏ ÈÛÙÔÛÂÏ›‰· Û¿˜ ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ‚Ú›Ù ÙÔ Ê·ÚÌ·ÎÂ›Ô Ô˘ ÂÊËÌÂÚ‡ÂÈ Û ÔÔÈ·‰‹ÔÙ Â·Ú¯›· Ù˘ ∫‡ÚÔ˘. ∂›Û˘, ‰È·ı¤ÙÂÈ ·›ıÔ˘Û· Û˘˙‹ÙËÛ˘ ÛÙËÓ ÔÔ›· ÔÈ ÁÔÓ›˜ ·ÓÙ·ÏÏ¿ÛÛÔ˘Ó ·fi„ÂȘ ÁÈ· ÎÔÈÓ¿ ı¤Ì·Ù· Ô˘ ÙÔ˘˜ ··Û¯ÔÏÔ‡Ó. ∂›Ó·È ¤Ó·˜ ¯ÒÚÔ˜ fiÔ˘ ÔÈ ÁÔÓ›˜ ÌÔÈÚ¿˙ÔÓÙ·È Ù· ÚÔ‚Ï‹Ì·Ù· Î·È ÙȘ ·ÓËÛ˘¯›Â˜ ÙÔ˘˜. ¶·ÙÒÓÙ·˜ ÛÙÔ Û‡Ó‰ÂÛÌÔ «°È· ·È‰È¿ÙÚÔ˘˜», ı· Ô‰ËÁËı›Ù ÛÙËÓ ÎÂÓÙÚÈ΋ ÛÂÏ›‰· Ù˘ ÈÛÙÔÛÂÏ›‰·˜ ÙˆÓ Â·ÁÁÂÏÌ·ÙÈÒÓ ˘Á›·˜ (http://www.peds.paidiatros.com). °È· Ó· ÙËÓ ÂÈÛÎÂÊı›Ù Ú¤ÂÈ Ó· ›ÛÙ ̤ÏÔ˜ - ÌÔÚ›Ù ӷ Á›ÓÂÙ ̤ÏÔ˜ ¯ˆÚ›˜ η̛· ·Ôχو˜ ÂÈ‚¿Ú˘ÓÛË. √ ¯Ú‹ÛÙ˘ Û ·˘Ù‹ ÙË ÈÛÙÔÛÂÏ›‰· ÌÔÚ› Ó· ‰È·‚¿ÛÂÈ ÂÚÈÏ‹„ÂȘ ·fi ÙËÓ ÙÚ¤¯Ô˘Û· ‚È‚ÏÈÔÁÚ·Ê›·, Ó· ‚ÚÂÈ ÙÔ Û˘Ó¤‰ÚÈÔ Ô˘ ÙÔÓ ÂӉȷʤÚÂÈ ÛÙËÓ ∫‡ÚÔ, ÛÙËÓ ∂ÏÏ¿‰·, ·ÏÏ¿ Î·È Û ÔÔÈ·‰‹ÔÙ ¯ÒÚ· ÙÔ˘ ÎfiÛÌÔ˘. ∂›Û˘, Û·˜ ‰›ÓÂÙ·È Ë ‰˘Ó·ÙfiÙËÙ· Ó· ηٷ¯ˆÚ‹ÛÂÙ ÙÔ ‰ÈÎfi Û·˜ Û˘Ó¤‰ÚÈÔ. ∂ӉȷʤÚÔÓ Â›Ó·È ÙÔ ÎÏÈÓÈÎfi Quiz ÙÔ˘ Ì‹Ó·, fiÔ˘ ÂÚÈÁÚ¿ÊÂÙ·È ¤Ó· Û¯ÂÙÈο Û¿ÓÈÔ ÂÚÈÛÙ·ÙÈÎfi Î·È ÔÈ ¯Ú‹ÛÙ˜ Ô˘ ı· ‚ÚÔ˘Ó ÙË ‰È¿ÁÓˆÛË Ì·›ÓÔ˘Ó ÛÙË ÎÏ‹ÚˆÛË ÁÈ· ÙËÓ ·fiÎÙËÛË ÂÓfi˜ ‚È‚Ï›Ô˘ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ¶·È‰È·ÙÚÈ΋. ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ÈÛÙÔÛÂÏ›‰· www.paidiatros.com ·ÔÙÂÏ› ÌÈ· ·ÍÈfiÈÛÙË ËÁ‹ ÏËÚÔÊfiÚËÛ˘ ÁÈ· ı¤Ì·Ù· ˘Á›·˜ ÙˆÓ ·È‰ÈÒÓ, ÙfiÛÔ ÁÈ· ÙÔ˘˜ ÁÔÓ›˜ fiÛÔ Î·È ÁÈ· ÙÔ˘˜ Â·ÁÁÂÏ̷ٛ˜ ˘Á›·˜.

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CLINICAL QUIZ

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∞¶∞¡∆∏™∏ ¢È¿ÁÓˆÛË: ™˘ÁÁÂÓ‹˜ ˘ÂÚÏ·Û›· ÂÈÓÂÊÚȉ›ˆÓ

∆Ô ÚÔÂͤ¯ÔÓ ·fi Ù· ÎÏÈÓÈο Â˘Ú‹Ì·Ù· ÙÔ˘ ‚Ú¤ÊÔ˘˜ ‹Ù·Ó Ë ÛÔ‚·Ú‹ ·›Û¯Ó·ÛË Î·È Ë ·Ê˘‰¿ÙˆÛË. ∞fi Ù· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·, ÚÔ›¯·Ó Ë ·ÂÈÏËÙÈ΋ ÁÈ· ÙË ˙ˆ‹ ˘ÔÓ·ÙÚÈ·ÈÌ›· Î·È ÏÈÁfiÙÂÚÔ Ë ˘ÂÚηÏÈ·ÈÌ›·. H ÙÂÏÂ˘Ù·›·, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙË ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË, ηıÒ˜ Î·È Ë ·ÚÓËÙÈ΋ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË Ù˘ ÎÔÈÏÈ¿˜ ÁÈ· „ËÏ·ÊËÙ‹ Ì¿˙· Ù‡Ô˘ «ÂÏ·›·˜» ÛÙÔ ÂÈÁ¿ÛÙÚÈÔ ·ÔÌ·ÎÚ‡ÓÔ˘Ó ÙËÓ Èı·ÓfiÙËÙ· ˘ÏˆÚÈ΋˜ ÛÙ¤ÓˆÛ˘, ÛÙËÓ ÔÔ›· ı· ·Ó·ÌÂÓfiÙ·Ó ˘ÔηÏÈ·ÈÌ›· Î·È ·ÏοψÛË. ∏ ÈÓÔ΢ÛÙÈ΋ ÓfiÛÔ˜ ÌÔÚ› Ó· ÂΉËψı› Ì ·›Û¯Ó·ÛË ÏfiÁˆ ‰˘Û·ÔÚÚfiÊËÛ˘ (ÔÈ ÎÂÓÒÛÂȘ ÙÔ˘ ‚Ú¤ÊÔ˘˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜), ÌÂ Û˘ÓÔ‰fi ˘ÔÓ·ÙÚÈ·ÈÌ›· Î·È ˘ԯψÚÈ·ÈÌ›· ÏfiÁˆ ·ÒÏÂÈ·˜ ¿Ï·ÙÔ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ôχ ˙ÂÛÙÒÓ ËÌÂÚÒÓ, Û˘Ó‹ıˆ˜ fï˜ Û ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ· ·fi ·˘Ù‹Ó ÙÔ˘ ·ÛıÂÓ‹ Ì·˜. ∏ ·Ó¿ÌÈÍË ÙÔ˘ ‚ÚÂÊÈÎÔ‡ Á¿Ï·ÙÔ˜ ·fi ÙË ÌËÙ¤Ú· ÁÈÓfiÙ·Ó ÛˆÛÙ¿, ·ÔÌ·ÎÚ‡ÓÔÓÙ·˜ ÙÔ ÂӉ¯fiÌÂÓÔ «‰ËÏËÙËÚ›·Û˘ ·fi ÓÂÚfi», ÂÓÒ Ë ·Ô˘Û›· ˘ÚÂÙÔ‡ Î·È ‰˘ÛÌÔÚÊÈÎÒÓ ¯·Ú·ÎÙËÚÈÛÙÈÎÒÓ Î·È Ë Ê˘ÛÈÔÏÔÁÈ΋ ÂͤٷÛË ‰¤ÚÌ·ÙÔ˜, Ì·ÏÏÈÒÓ Î·È Ó˘¯ÈÒÓ ·ÔÌ·ÎÚ‡ÓÂÈ ÙÔ ÂӉ¯fiÌÂÓÔ ·ÓȉڈÙÈ΋˜ ÂÎÙÔ‰ÂÚÌÈ΋˜ ‰˘ÛÏ·Û›·˜, ‰È·Ù·Ú·¯‹ Ô˘ ÌÔÚ› Ó· ÂΉËψı› Ì ˘ÔıÚ„›· Î·È Û˘ÓÔ‰fi ˘ÂÚ˘ÚÂÍ›·, Ô˘ ‰ÂÓ ˘‹Ú¯Â ÛÙÔÓ ·ÛıÂÓ‹ Ì·˜. ∆Ô ÎÏÈÓÈÎfi ÛÂÓ¿ÚÈÔ ÙÔ˘ ‚Ú¤ÊÔ˘˜ Ô˘ ÂÚÈÁÚ¿ÊÔ˘ÌÂ, ÌÂ Û˘Ó‰˘·ÛÌfi ˘ÔÓ·ÙÚÈ·ÈÌ›·˜, ˘ÂÚηÏÈ·ÈÌ›·˜ Î·È ÌÂÙ·‚ÔÏÈ΋˜ ÔͤˆÛ˘, Â›Ó·È Û˘Ì‚·Ùfi Ì ¤ÏÏÂÈ„Ë ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ, Ô˘ ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È ÛÂ Û˘ÁÁÂÓ‹ ˘ÂÚÏ·Û›· ÂÈÓÂÊÚȉ›ˆÓ, Û ˘ԷωÔÛÙÂÚÔÓÈÛÌfi Î·È Û ·ÓÙ›ÛÙ·ÛË ÛÙË ‰Ú¿ÛË Ù˘ ·Ï‰ÔÛÙÂÚfiÓ˘ ‹ „¢‰Ô¸ԷωÂÛÙÂÚÔÓÈÛÌfi Ù‡Ô˘ π. √È ÙÂÏÂ˘Ù·›Â˜ ‰‡Ô ÔÓÙfiÙËÙ˜ Â›Ó·È Û·ÓÈfiÙÂÚ˜. §fiÁˆ Ù˘ ÂÍ·ÈÚÂÙÈο ‰˘Û¯ÂÚÔ‡˜ ·ÈÌÔÏË„›·˜, ·Ú¯Èο ¤ÁÈÓ ̤ÙÚËÛË ÌfiÓÔ Ù˘ 17-˘‰ÚÔ͢-ÚÔÁÂÛÙÂÚfiÓ˘ Î·È Ù˘ ÎÔÚÙÈ˙fiÏ˘. √ ¤ÏÂÁ¯Ô˜ ·Ó¤‰ÂÈÍ 17-˘‰ÚÔ͢-ÚÔÁÂÛÙÂÚfiÓË 17,9 nmol/l (º.∆.: 2-6,05) Î·È ÎÔÚÙÈ˙fiÏË 241,5 nmol/l (º.∆.: 28-662). ªÂ ÙËÓ ¿ÌÂÛË ¤Ó·ÚÍË ˘‰ÚÔÎÔÚÙÈ˙fiÓ˘ Î·È ÙË ¯ÔÚ‹ÁËÛË ÎÚ˘ÛÙ·ÏÏÔÂȉÒÓ ‰È·Ï˘Ì¿ÙˆÓ Ì ¯ÏˆÚÈÔ‡¯Ô Ó¿ÙÚÈÔ, Â‹Ïı ÛÙ·‰È·Î‹ ·ÔηٿÛÙ·ÛË Ù˘ ˘ÔÓ·ÙÚÈ·ÈÌ›·˜, Ì ¿ÓÔ‰Ô Ù˘ Û˘ÁΤÓÙÚˆÛ˘ Ó·ÙÚ›Ô˘ ÔÚÔ‡ ÛÙÔ 130 mEq/l Û ‰È¿ÛÙËÌ· 60 ÂÚ›Ô˘ ˆÚÒÓ.

∏ ˘„ËÏ‹ ÙÈÌ‹ 17-˘‰ÚÔ͢-ÚÔÁÂÛÙÂÚfiÓ˘ ÛÙÔÓ ·ÛıÂÓ‹ Ì·˜ Â›Ó·È ÂÓ‰ÂÈÎÙÈ΋ Û˘ÁÁÂÓÔ‡˜ ˘ÂÚÏ·Û›·˜ ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ (1-3), ·ÚfiÏÔ Ô˘ Ù· ÂÚÈÛÛfiÙÂÚ· ÚÔۂ‚ÏË̤ӷ ÓÂÔÁÓ¿ ¤¯Ô˘Ó ÛÙËÓ ÚˆÙԉȿÁÓˆÛË Û˘ÁÎÂÓÙÚÒÛÂȘ >105 nmol/l (4). ªÂ Ù· ‰Â‰Ô̤ӷ ·˘Ù¿ ¤ÁÈÓ ¿ÌÂÛ·, ÌÂÙ¿ ÙË ‰È·›ÛÙˆÛË ÙˆÓ ˘„ËÏÒÓ ÂÈ¤‰ˆÓ 17-˘‰ÚÔ͢-ÚÔÁÂÛÙÂÚfiÓ˘, ¯ÔÚ‹ÁËÛË ˘‰ÚÔÎÔÚÙÈ˙fiÓ˘ 25 mg ÂÓ‰ÔÊÏ‚›ˆ˜ Î·È Û˘Ó¤¯ÈÛË Ù˘ ·ÁˆÁ‹˜ ·fi ÙÔ ÛÙfiÌ· 20 mg/m2/Ë̤ڷ Û ÙÚÂȘ ‰fiÛÂȘ. ∆ËÓ ÙÚ›ÙË Ì¤Ú· ÓÔÛËÏ›·˜ ÚÔÛÙ¤ıËΠÛÙË ıÂÚ·¢ÙÈ΋ ·ÁˆÁ‹ Î·È ÊÏÔ˘ÔÚÔÎÔÚÙÈ˙fiÓË 0,05 mg/Ë̤ڷ, Ì ÛÙ·‰È·Î‹ ·‡ÍËÛË Ù˘ ‰fiÛ˘ Û 0,1 mg/Ë̤ڷ, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙË ¯ÔÚ‹ÁËÛË ÂÈÚfiÛıÂÙÔ˘ ¿Ï·ÙÔ˜ ÛÙÔ ‚ÚÂÊÈÎfi Á¿Ï·. ª›· ‚‰ÔÌ¿‰· ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ıÂÚ·›·˜, ¤ÁÈÓ ̤ÙÚËÛË ACTH, ·Ï‰ÔÛÙÂÚfiÓ˘, ÚÂÓ›Ó˘ Î·È DHEA-S Ô˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜, ÂÓÒ Ë 17-˘‰ÚÔ͢-ÚÔÁÂÛÙÂÚfiÓË ‹Ù·Ó ϤÔÓ ÂÓÙfi˜ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÙÈÌÒÓ. ∆Ô ‚Ú¤ÊÔ˜ Ô˘ ¿Ú¯ÈÛ ӷ ÛÈÙ›˙ÂÙ·È ÈηÓÔÔÈËÙÈο ÌÂÙ¿ ÙËÓ ÙÚ›ÙË Ë̤ڷ ÓÔÛËÏ›·˜ ÂÍ‹Ïı Û ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË ÌÂÙ¿ ·fi ÓÔÛËÏ›· 3 ‚‰ÔÌ¿‰ˆÓ, Ì ‚¿ÚÔ˜ ÂÍfi‰Ô˘ 3.615 g, ˘fi ·ÁˆÁ‹ Ì ˘‰ÚÔÎÔÚÙÈ˙fiÓË Î·È ÊÏÔ˘ÔÚÔÎÔÚÙÈ˙fiÓË. ŒÎÙÔÙÂ, ÔÈ ÔÚÌfiÓ˜ 17-˘‰ÚÔ͢-ÚÔÁÂÛÙÂÚfiÓË Î·È ·Ó‰ÚÔÛÙÂÓ‰ÈfiÓË, ηıÒ˜ Î·È Ë ‰Ú·ÛÙÈÎfiÙËÙ· ÚÂÓ›Ó˘ ÔÚÔ‡, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ Ù·ÎÙÈ΋ ηٷÁÚ·Ê‹ ÙˆÓ ·Ú·Ì¤ÙÚˆÓ ÛˆÌ·ÙÈ΋˜ ·Ó¿Ù˘Í˘ ÙÔ˘ ‚Ú¤ÊÔ˘˜, ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·ÓÙ·fiÎÚÈÛ˘ ÛÙË ıÂÚ·›· ˘ÔηٿÛÙ·Û˘. ∏ ÌÂȈ̤ÓË ÌÂÙ·ÙÚÔ‹ Ù˘ 17-˘‰ÚÔ͢ÚÔÁÂÛÙÂÚfiÓ˘ Û 11-‰ÂÔ͢-ÎÔÚÙÈ˙fiÏË Â›Ó·È ˘‡ı˘ÓË ÁÈ· >90% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û˘ÁÁÂÓÔ‡˜ ÂÈÓÂÊÚȉȷ΋˜ ˘ÂÚÏ·Û›·˜. ∏ ÌÂÙ·ÙÚÔ‹ ·˘Ù‹ Á›ÓÂÙ·È ·fi ÙÔ ¤Ó˙˘ÌÔ 21-˘‰ÚÔÍ˘Ï¿ÛË (CYP21A2) (1-3). ∏ ÌÂȈ̤ÓË Û‡ÓıÂÛË 11-‰ÂÔ͢-ÎÔÚÙÈ˙fiÏ˘ Ô‰ËÁ› Û ÌÂȈ̤ÓË ‚ÈÔÛ‡ÓıÂÛË ÎÔÚÙÈ˙fiÏ˘ Î·È ·ÓÙÈÚÚÔÈÛÙÈο Û ·˘ÍË̤ÓË ¤ÎÎÚÈÛË ACTH. H ÔÊÂÈÏfiÌÂÓË ÛÙËÓ ÎÔÚÙÈÎÔÙÚÔ›ÓË ‰È¤ÁÂÚÛË ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ Ô‰ËÁ› Û ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ·Ó‰ÚÔÁfiÓˆÓ. ¢Â‰Ô̤ӷ ·fi ‰ÈÂıÓ‹ ÚÔÁÚ¿ÌÌ·Ù· ÚÒÈÌ˘ ‰È¿ÁÓˆÛ˘ Û 6,5 ÂηÙÔÌ̇ÚÈ· ÓÂÔÁÓ¿ ‰›ÓÔ˘Ó ÌÈ· Â›ÙˆÛË ÂÚ›Ô˘ 1 ÛÙ· 15.000 ˙ÒÓÙ· ÓÂÔÁ¤ÓÓËÙ·, Ì ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ·Ó¿ÏÔÁ· Ì ÙË ¯ÒÚ· Î·È ÙÔÓ ÏËı˘ÛÌfi ÌÂϤÙ˘ (5). √È

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¶·È‰È·ÙÚÈ΋ 2009;72:66,71-73

Pediatric Jan-Feb 09

16-03-09

72

14:56

™ÂÏ›‰·72

∫§π¡π∫√ ∫√Àπ∑ ·ÛıÂÓ›˜ Ì ÎÏ·ÛÈÎÔ‡ Ù‡Ô˘ Û˘ÁÁÂÓ‹ ÂÈÓÂÊÚȉȷ΋ ˘ÂÚÏ·Û›· ÏfiÁˆ ¤ÏÏÂȄ˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ Ù˘Èο ÂÌÊ·Ó›˙ÔÓÙ·È Î·Ù¿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô ‹ Ï›ÁÔ ÌÂÙ¿, Ì ·ÓÂ·Ú΋ ÚfiÛÏË„Ë ‚¿ÚÔ˘˜, ·›Û¯Ó·ÛË, ·Ê˘‰¿ÙˆÛË, Ì ‹ ¯ˆÚ›˜ ·ÒÏÂÈ· ¿Ï·ÙÔ˜, ‹ ÛÙË ÓËȷ΋ ËÏÈΛ· Ì ·ÚÚÂÓÔÔ›ËÛË. ∆· ı‹Ï· ‚Ú¤ÊË ÂÌÊ·Ó›˙Ô˘Ó ·ÌÊ›‚ÔÏ· ¤Íˆ ÁÂÓÓËÙÈο fiÚÁ·Ó· (·ÚÚÂÓÔÔ›ËÛË ¤Íˆ ÁÂÓÓËÙÈÎÒÓ ÔÚÁ¿ÓˆÓ). ∆· ·ÁfiÚÈ· ÂÌÊ·Ó›˙ÔÓÙ·È Ê˘ÛÈÔÏÔÁÈο ‹ ÌÔÚ› Ó· ¤¯Ô˘Ó ·˘ÍË̤ÓË ˘¤Ú¯ÚˆÛË ÙÔ˘ ÔÛ¯¤Ô˘ Î·È ÌÂÁ¿ÏÔ ¤Ô˜. ¶ÂÚ›Ô˘ ÙÔ 67% ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÏ·ÛÈ΋ ¤ÏÏÂÈ„Ë 21-˘‰ÚÔÍ˘Ï¿Û˘ ÂÌÊ·Ó›˙ÂÈ ·ÒÏÂÈ· ¿Ï·ÙÔ˜, ÏfiÁˆ ¤ÏÏÂȄ˘ ·Ï‰ÔÛÙÂÚfiÓ˘, ÂÓÒ ÙÔ ˘fiÏÔÈÔ ¤Ó· ÙÚ›ÙÔ ÂÌÊ·Ó›˙ÂÈ ÙË ÌÔÚÊ‹ Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ÌfiÓÔ ·fi ·ÚÚÂÓÔÔ›ËÛË. √ ÛÙfi¯Ô˜ Ù˘ ·Ú¯È΋˜ ıÂÚ·›·˜ Û ÂÈÓÂÊÚȉȷ΋ ÎÚ›ÛË Â›Ó·È Ë ÁÚ‹ÁÔÚË ‰ÈfiÚıˆÛË Ù˘ ·Ê˘‰¿ÙˆÛ˘ Î·È Ù˘ ˘fiÙ·Û˘, Ë ÛÙ·‰È·Î‹ ·ÔηٿÛÙ·ÛË Ù˘ ˘ÔÓ·ÙÚÈ·ÈÌ›·˜ Î·È Ë ‰ÈfiÚıˆÛË Ù˘ ¤ÏÏÂȄ˘ ÎÔÚÙÈ˙fiÏ˘. ªÈ· ·Ú¯È΋ ‰fiÛË stress ˘‰ÚÔÎÔÚÙÈ˙fiÓ˘ 50-100 mg/m2 (Ù˘È΋ ÓÂÔÁÓÈ΋ ‰fiÛË 25 mg) Ú¤ÂÈ Ó· ·ÎÔÏÔ˘ıÂ›Ù·È ·fi ¯ÔÚ‹ÁËÛË ËÌÂÚ‹ÛÈˆÓ ‰fiÛÂˆÓ 50100 mg/m2 Û ‰È·ÈÚÂ̤Ó˜ ‰fiÛÂȘ Î·È Ì¤¯ÚÈ ÙË ÛÙ·ıÂÚÔÔ›ËÛË ÙÔ˘ ÓÂÔÁÓÔ‡ Î·È ÙËÓ ¤Ó·ÚÍË ÈηÓÔÔÈËÙÈ΋˜ Û›ÙÈÛ˘. ™ÙËÓ ÔÍ›· Ê¿ÛË Ô˘ ¯ÔÚËÁÂ›Ù·È ˘‰ÚÔÎÔÚÙÈ˙fiÓË Û ‰fiÛÂȘ stress ‰ÂÓ Â›Ó·È ÙÂÏ›ˆ˜ ··Ú·›ÙËÙË Ë ¯Ú‹ÛË ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ, ·ÚfiÏÔ Ô˘ ÔÈ ÂÚÈÛÛfiÙÂÚÔÈ ·È‰ÔÂÓ‰ÔÎÚÈÓÔÏfiÁÔÈ Ù· ÍÂÎÈÓÔ‡Ó ·Ì¤Ûˆ˜ Û ‰fiÛË 0,15 mg/m2/Ë̤ڷ. ™ÙË Û˘Ó¤¯ÂÈ· ¯ÔÚËÁÂ›Ù·È ˘‰ÚÔÎÔÚÙÈ˙fiÓË Û ‰fiÛË 12-18 mg/m2/Ë̤ڷ Û ‰È·ÈÚÂ̤Ó˜ ‰fiÛÂȘ Î·È Û˘Ó¯›˙ÂÙ·È Ë ·ÁˆÁ‹ Ì ÊÏÔ˘ÔÚÔÎÔÚÙÈ˙fiÓË. H ·ÓÂ·Ú΋˜ ˘ÔηٿÛÙ·ÛË ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ̷ÎÚÔ¯ÚfiÓÈ· Ì›ˆÛË ÙÔ˘ ÂÓ‰·ÁÁÂÈ·ÎÔ‡ fiÁÎÔ˘, ¯ˆÚ›˜ ȉȷ›ÙÂÚ· ÎÏÈÓÈο Â˘Ú‹Ì·Ù·, ÌÂ Û˘ÓÔ‰fi ˘ÂÚ·Ú·ÁˆÁ‹ ÂÈÓÂÊÚȉȷÎÒÓ ·Ó‰ÚÔÁfiÓˆÓ ÏfiÁˆ ˘ÂÚ·Ú·ÁˆÁ‹˜ ÚÂÓ›Ó˘ Î·È ·ÁÁÂÈÔÙÂÓÛ›Ó˘ ππ Ô˘ ‰ÈÂÁ›ÚÔ˘Ó ÙËÓ ÂÈÓÂÊÚȉȷ΋ ÛÙÂÚÔÂȉÔÁ¤ÓÂÛË (6). °È· ÙÔ˘˜ ÏfiÁÔ˘˜ ·˘ÙÔ‡˜, Ù· ·Ï·ÙÔÎÔÚÙÈÎÔÂȉ‹ Â›Ó·È ˆÊ¤ÏÈÌ· ÛÙËÓ ÎÏ·ÛÛÈ΋ ¤ÏÏÂÈ„Ë 21-˘‰ÚÔÍ˘Ï¿Û˘, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔ ·Ó ·˘Ù‹ Û˘Óԉ‡ÂÙ·È ‹ fi¯È ·fi ·ÒÏÂÈ· ¿Ï·ÙÔ˜. ∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Û˘ÁÁÂÓÔ‡˜ ÂÈÓÂÊÚȉȷ΋˜ ˘ÂÚÏ·Û›·˜ ÏfiÁˆ ¤ÏÏÂȄ˘ 21-˘‰ÚÔÍ˘Ï¿Û˘ ÂÓ¤¯ÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ˘ÂÚıÂÚ·›·˜, Ì ÂÌÊ¿ÓÈÛË È·ÙÚÔÁÂÓÔ‡˜ Û˘Ó‰ÚfiÌÔ˘ Cushing Î·È Î·ı˘ÛÙ¤ÚËÛ˘ Ù˘ ·Ó¿Ù˘Í˘. ∏ ÌË ÎÏ·ÛÈ΋ ‹ fi„ÈÌË ¤ÏÏÂÈ„Ë 21-˘‰ÚÔÍ˘Ï¿Û˘ ÂÌÊ·Ó›˙ÂÙ·È ÌÂÙ·ÁÂÓ¤ÛÙÂÚ·, Ì ÎÏÈÓÈο

Paediatriki 2009;72:66,71-73

ÛËÌ›· ÂÚ›ÛÛÂÈ·˜ ·Ó‰ÚÔÁfiÓˆÓ Î·È ¯ˆÚ›˜ ÈÛÙÔÚÈÎfi ·ÚÚÂÓÔÔ›ËÛ˘ ÙˆÓ ¤Íˆ ÁÂÓÓËÙÈÎÒÓ ÔÚÁ¿ÓˆÓ ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô (7). ∫ÏÈÓÈο ÛËÌ›· Î·È Û˘ÌÙÒÌ·Ù¿ Ù˘ Â›Ó·È Ë ÚÒÈÌË ‹‚Ë, Ë ÚÔ¯ˆÚË̤ÓË ÔÛÙÈ΋ ËÏÈΛ·, Ë ˘ÂÚÙÚ›¯ˆÛË, ÔÈ ‰È·Ù·Ú·¯¤˜ ÂÚÈfi‰Ô˘, Ë ·ÎÌ‹ Î·È Ë ˘ÔÁÔÓÈÌfiÙËÙ·, ·ÚfiÏÔ Ô˘ ·ÚÎÂÙÔ› ·ÛıÂÓ›˜ ·Ú·Ì¤ÓÔ˘Ó ·Û˘Ìو̷ÙÈÎÔ›. ∏ ¤ÏÏÂÈ„Ë 11-‚-˘‰ÚÔÍ˘Ï¿Û˘ (CYP11B1) Â›Ó·È Ë ‰Â‡ÙÂÚË ÛÂ Û˘¯ÓfiÙËÙ· ·ÈÙ›· Û˘ÁÁÂÓÔ‡˜ ÂÈÓÂÊÚȉȷ΋˜ ˘ÂÚÏ·Û›·˜, ηıÒ˜ ¢ı‡ÓÂÙ·È ÁÈ· ÙÔ 5-8% ÙˆÓ ÂÚÈÙÒÛˆÓ, Ì Â›ÙˆÛË 1 ÛÙȘ 100.000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ÓÂÔÁ¤ÓÓËÙˆÓ (1-3). ∆Ô ÂÓ˙˘ÌÈÎfi ¤ÏÏÂÈÌÌ· ÚÔηÏ› Ì›ˆÛË ÛÙË ÌÂÙ·ÙÚÔ‹ Ù˘ 11-‰ÂÔ͢-ÎÔÚÙÈ˙fiÏ˘ Î·È 11-‰ÂÔ͢-ÎÔÚÙÈÎÔÛÙÂÚfiÓ˘ Û ÎÔÚÙÈ˙fiÏË Î·È ÎÔÚÈÎÔÛÙÂÚfiÓË, ·ÓÙ›ÛÙÔȯ·, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·ÓÙÈÚÚÔÈÛÙÈ΋ ·‡ÍËÛË Ù˘ ÎÔÚÙÈÎÔÙÚÔ›Ó˘ Î·È ÙËÓ Â·ÎfiÏÔ˘ıË ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ÂÈÓÂÊÚȉȷÎÒÓ ·Ó‰ÚÔÁfiÓˆÓ. √È ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ Ù˘ ¤ÏÏÂȄ˘ 11-‚-˘‰ÚÔÍ˘Ï¿Û˘ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ù˘ ÂÚ›ÛÛÂÈ·˜ ·Ó‰ÚÔÁfiÓˆÓ Î·È Ù˘ ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÈ΋˜ ‰Ú¿Û˘ Ù˘ 11-‰ÂÔ͢-ÎÔÚÙÈÎÔÛÙÂÚfiÓ˘. ∏ ÙÂÏÂ˘Ù·›· ÌÔÚ› Ó· ÂΉËψı› Ì ˘ÔηÏÈ·ÈÌ›· Î·È ˘¤ÚÙ·ÛË Î·È ·˘Ù¿ ÙË ‰È·ÎÚ›ÓÔ˘Ó ·fi ÙË Û˘¯ÓfiÙÂÚË ¤ÏÏÂÈ„Ë 21-˘‰ÚÔÍ˘Ï¿Û˘. √ ˘ԷωÔÛÙÂÚÔÓÈÛÌfi˜, Ô˘ ı· Ú¤ÂÈ Ó· Û˘ÌÂÚÈÏËÊı› ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÙÔ˘ ·ÛıÂÓ‹ Ì·˜, ÔÊ›ÏÂÙ·È Û ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ¤Ó˙˘ÌÔ CYP11B2 (Û˘ÓıÂÙ¿ÛË ·Ï‰ÔÛÙÂÚfiÓ˘), ÌÈ· Û¿ÓÈ· ‰È·Ù·Ú·¯‹ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ‰‡Ô ˘fiÙ˘Ô˘˜, ·Ó¿ÏÔÁ· Ì ٷ Â›‰· ·Ï‰ÔÛÙÂÚfiÓ˘ Î·È ÙˆÓ ÚÔ‰ÚfiÌˆÓ Ù˘. ∞ÛıÂÓ›˜ Ì ¤ÏÏÂÈ„Ë ÌÂı˘Ï-ÔÍÂȉ¿Û˘ ÎÔÚÙÈÎÔÛÙÂÚfiÓ˘ Ù‡Ô˘ π ¤¯Ô˘Ó ·Ó‚·Ṳ̂ӷ Â›‰· ÎÔÚÙÈÎÔÛÙÂÚfiÓ˘ Î·È ¯·ÌËÏ¿ Â›‰· 18-˘‰ÚÔ͢-ÎÔÚÙÈÎÔÛÙÂÚfiÓ˘ Î·È ·Ï‰ÔÛÙÂÚfiÓ˘. ∞ÓÙÈı¤Ùˆ˜, ÔÈ ·ÛıÂÓ›˜ Ì ¤ÏÏÂÈ„Ë ÌÂı˘Ï-ÔÍÂȉ¿Û˘ ÎÔÚÙÈÎÔÛÙÂÚfiÓ˘ Ù‡Ô˘ ππ ¤¯Ô˘Ó ˘„ËÏ‹ 18-˘‰ÚÔ͢ÎÔÚÙÈÎÔÛÙÂÚfiÓË, ÙËÓ ¿ÌÂÛË Úfi‰ÚÔÌË Ô˘Û›· Ù˘ ·Ï‰ÔÛÙÂÚfiÓ˘ (8). √ „¢‰Ô¸ԷωÂÛÙÂÚÔÓÈÛÌfi˜ Ù‡Ô˘ π, Ô˘ Â›Û˘ ı· Ú¤ÂÈ Ó· Û˘ÌÂÚÈÏËÊı› ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË, ¤¯ÂÈ ·Ó·ÁÓˆÚÈÛÙ› ˆ˜ ¤Ó· ÂÙÂÚÔÁÂÓ¤˜ Û‡Ó‰ÚÔÌÔ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙÔ˘Ï¿¯ÈÛÙÔ ‰‡Ô ÎÏÈÓÈο ‰È·ÎÚÈÙ¤˜ ÔÓÙfiÙËÙ˜ Ì ÓÂÊÚÈ΋ ‹ ÔÏ˘ÔÚÁ·ÓÈ΋ Û˘ÌÌÂÙÔ¯‹, ·ÓÙ›ÛÙÔȯ·. ∆˘Èο ÂÌÊ·Ó›˙ÔÓÙ·È Û‡ÓÙÔÌ· ÌÂÙ¿ ÙË Á¤ÓÓËÛË, Ì ÛËÌ›· ¤ÏÏÂȄ˘ ·Ï·ÙÔÎÔÚÙÈÎÔÂȉÒÓ, ·ÏÏ¿ Ì ·ıÔÏÔÁÈο ˘„ËϤ˜ ÙÈ̤˜ Ï¿ÛÌ·ÙÔ˜ ·Ï‰ÔÛÙÂÚfiÓ˘ Î·È ÚÂÓ›Ó˘. ∆· ÛËÌ›· Î·È Û˘ÌÙÒÌ·Ù· ·ÓÙ›ÛÙ·Û˘ ÛÙËÓ ·Ï‰ÔÛÙÂÚfiÓË

Pediatric Jan-Feb 09

16-03-09

14:56

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CLINICAL QUIZ ˘Ô¯ˆÚÔ‡Ó Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ Û ÔÏÏÔ‡˜ ·ÛıÂÓ›˜, ÌÂÚÈÎÔ› fï˜ ηٷϋÁÔ˘Ó ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ· ·fi ˘ÂÚηÏÈ·ÈÌ›· (8). ªÂÚÈΤ˜ ¯ÒÚ˜ ÂÊ·ÚÌfi˙Ô˘Ó ÚÔÁÚ¿ÌÌ·Ù· ÚÒÈÌ˘ ‰È¿ÁÓˆÛ˘ Ù˘ Û˘ÁÁÂÓÔ‡˜ ˘ÂÚÏ·Û›·˜ ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ ÛÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Â›Ó·È Ì›· ·fi ÙȘ Û˘¯ÓfiÙÂÚ˜ ÎÏËÚÔÓÔÌÔ‡ÌÂÓ˜ ‰È·Ù·Ú·¯¤˜ Ì ·˘ÙÔÛˆÌÈÎfi ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. ∆· ÚÔÁÚ¿ÌÌ·Ù· ·˘Ù¿ ÌÂÈÒÓÔ˘Ó ÙËÓ Èı·ÓfiÙËÙ· ÚÔÛ¤Ï¢Û˘ ÓÂÔÁÓÒÓ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô Ì ‚·ÚÈ¿ ·Ê˘‰¿ÙˆÛË Î·È ·ÒÏÂÈ· ¿Ï·ÙÔ˜ Î·È Èı·ÓfiÓ ÌÂÈÒÓÔ˘Ó Î·È ÙË ıÓËÙfiÙËÙ· ÏfiÁˆ ηı˘ÛÙÂÚË̤Ó˘ ‰È¿ÁÓˆÛ˘. ™˘ÌÂÚ·ÛÌ·ÙÈο, οı ÓÂÔÁÓfi ‹ ‚Ú¤ÊÔ˜ ÔÏ›ÁˆÓ ‚‰ÔÌ¿‰ˆÓ Ô˘ ÂÌÊ·Ó›˙ÂÙ·È Ì ˘ÔıÚ„›·, ·ÓÂ·Ú΋ ·Ó¿Ù˘ÍË, ·Ê˘‰¿ÙˆÛË Î·È ÂÌÂÙÔ‡˜ ı· Ú¤ÂÈ Ó· ÂϤÁ¯ÂÙ·È ÁÈ· ËÏÂÎÙÚÔÏ˘ÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ ηÈ, ÂÊfiÛÔÓ ˘¿Ú¯ÂÈ ˘ÔÓ·ÙÚÈ·ÈÌ›· Î·È ˘ÂÚηÏÈ·ÈÌ›·, Ó· Á›ÓÂÙ·È ¿ÌÂÛ· ̤ÙÚËÛË 17-˘‰ÚÔ͢-ÚÔÁÂÛÙÂÚfiÓ˘, ηıÒ˜ Î·È ÎÔÚÙÈ˙fiÏ˘, 11-‰ÂÔ͢-ÎÔÚÙÈ˙fiÏ˘, 17˘‰ÚÔ͢-ÚÂÁÓÂÓÔÏfiÓ˘ Î·È ·Ó‰ÚÔÛÙÂÓ‰ÈfiÓ˘ ÁÈ· ·ÔÎÏÂÈÛÌfi Û˘ÁÁÂÓÔ‡˜ ÂÈÓÂÊÚȉȷ΋˜ ˘ÂÚÏ·Û›·˜ Î·È Î·ıÔÚÈÛÌfi ÙÔ˘ ÂÓ˙˘ÌÈÎÔ‡ ÂÏÏ›ÌÌ·ÙÔ˜.

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µÈ‚ÏÈÔÁÚ·Ê›· 1. Merke DP, Bornstein SR. Congenital adrenal hyperplasia. Lancet 2005;365:2125-2136.

2. Speiser PW, White PC. Congenital adrenal hyperplasia. N Engl J Med 2003;349:776-788.

3. Clayton PE, Miller WL, Oberfield SE, Ritzén EM, Sippell WG, Speiser PW; ESPE/LWPES CAH Working Group. Consensus statement on 21-hydroxylase deficiency from the European Society for Paediatric Endocrinology and the Lawson Wilkins Pediatric Endocrine Society. Horm Res 2002;58:188-195.

4. Witchel SF, Nayak S, Suda-Hartman M, Lee PA. Newborn screening for 21-hydroxylase deficiency: results of CYP21 molecular genetic analysis. J Pediatr 1997;131:328-331.

5. Pang S, Clark A. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: Newborn screening and its relationship to the diagnosis and treatment of the disorder. Screening 1993;2:105-139.

6. Schaison G, Couzinet B, Gourmelen M, Elkik F, Bougneres P. Angiotensin and adrenal steroidogenesis: study of 21-hydroxylase-deficient congenital adrenal hyperplasia. J Clin Endocrinol Metab 1980;51: 1390-1394.

7. Kohn B, Levine LS, Pollack MS, Pang S, Lorenzen F, Levy D, et al. Late-onset steroid 21-hydroxylase deficiency: a variant of classical congenital adrenal hyperplasia. J Clin Endocrinol Metab 1982;55:817-827.

8. White PC. Abnormalities of aldosterone synthesis and action in children. Curr Opin Pediatr 1997;9: 424-430.

¶·È‰È·ÙÚÈ΋ 2009;72:66,71-73