Παιδιατρική | Τόμος 64 • Τεύχος 1 • Ιανουάριος - Φεβρουάριος 2001

Page 1

E•øº.¶AI¢IATP.TEL TE§O™ 17-10-01 15:38 ™ÂÏ›‰· 1

ISSN 0377-2551

TOMO™ 64

TEYXO™ 1

ETO™ 2001

π·ÓÔ˘¿ÚÈÔ˜ - ºÂ‚ÚÔ˘¿ÚÈÔ˜ 2001 . ∆fiÌÔ˜ 64 . ∆‡¯Ô˜ 1

∞ƒ£ƒ√ ™À¡∆∞•∏™ ¶·Ú¿ÁÔÓÙ˜ Ô˘ ÂȉÚÔ‡Ó ÛÙËÓ ÂÓ‰ÔÌ‹ÙÚÈ· ·Ó¿Ù˘ÍË ÙˆÓ Ó¢ÌfiÓˆÓ

EDITORIAL 1

£. ∆ÛÈÏÈÁÈ¿ÓÓ˘

T. Tsiligiannis

ANA™KO¶H™∂π™ √Í›· ‰È¿ÚÚÔÈ·

REVIEW ARTICLES 4

∫. ™È·Ê¿Î·˜, π. ¶·Ó·ÁÈÒÙÔ˘-∞ÁÁÂÏ·ÎÔÔ‡ÏÔ˘

∏ Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ÛÙËÓ ·È‰È΋ ËÏÈΛ·

Factors affecting intrauterine lung growth

Acute diarrhea C. Siafakas, J. Panayiotou-Angelakopoulou

14

Nocturnal enuresis in children

∞. ∏Ï›·˜

A. Elias

™À¡∂Ãπ∑√ª∂¡∏ ¶∞π¢π∞∆ƒπ∫∏ ∂∫¶∞π¢∂À™∏

CONTINUING MEDICAL EDUCATION

™À¡¢ƒ√ª∞ ªÀ∂§π∫∏™ ∞¡∂¶∞ƒ∫∂π∞™

™˘ÁÁÂÓ›˜ √˘‰ÂÙÂÚÔÂӛ˜

25 26

Õ. ∫·Ú·ÎfiÏË

£ÚÔÌ‚ÔÂӛ˜ ÔÊÂÈÏfiÌÂÓ˜ Û ·ÓÂ·Ú΋ ·Ú·ÁˆÁ‹ ·ÈÌÔÂÙ·Ï›ˆÓ

30

Thrombocytopenias resulting from deficient platelet production H. Platokouki-Komitopoulou

34

µ. ∫›ÙÚ·

∞ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ıÂÚ·›· Û ·È‰È¿ Ì ‚·ÚÈ¿ ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·

Congenital neutropenias ∞. Karakoli

∂. ¶Ï·ÙÔÎÔ‡ÎË-∫ÔÌÈÙÔÔ‡ÏÔ˘

∞ÌÈÁ‹˜ ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜

THE BONE MARROW FAILURE SYNDROMES

Pure red cell aplasia of childhood V. Kitra

39

J.P. Panagiotou

¶AI¢IATPIKH

π.¶. ¶·Ó·ÁÈÒÙÔ˘

Immunosuppressive therapy in children with severe aplastic anemia

ªÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (ªª√) Û ·È‰È¿ Ì ۇӉÚÔÌ· Ì˘ÂÏÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜

45

¢. ¶ÂÙÚfiÔ˘ÏÔ˜

D. Petropoulos

∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™ £ÂÚ·›· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· ÛÙËÓ ÔÍ›· ‰È¿ÚÚÔÈ· Î·È ·Ó·ÛÙ·ÏÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘

Bone marrow transplantation in children with bone marrow failure syndromes

ORIGINAL PAPERS 49

Oral rehydration therapy for acute diarrhea and factors that inhibit its application

∂. ∞ÏÂÍfiÔ˘ÏÔ˜, ¡. ™ÎÂÓÙ¤Ú˘, ¡. ™Ô‡ÎÈ·˜, ∂. ∫ˆÛÙ·‰‹Ì·, ™. ÷˚‰¿˜

E. Alexopoulos, N. Skenteris, N. Soukias, E. Kostadima, S. Haidas

∏ Û˘Ó¤¯ÂÈ· ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÛÙËÓ ÚÒÙË ÂÛˆÙÂÚÈ΋ ÛÂÏ›‰·

Continuation of table of contents inside title page

January - February 2001 . Volume 64 . No 1


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 5

π·ÓÔ˘¿ÚÈÔ˜ - ºÂ‚ÚÔ˘¿ÚÈÔ˜ 2001 . ∆fiÌÔ˜ 64 . ∆‡¯Ô˜ 1

™˘Ó¤¯ÂÈ· ÂÚȯÔ̤ӈÓ

Continuation of table of contents

∂›‰· §ÈÔÚˆÙ½Ó˘ (·) Û 3298 ∂ÏÏËÓfiÔ˘Ï·, ËÏÈΛ·˜ 6-14 ÂÙÒÓ

55

Serum Lipoprotein (a) concentration in 3298 healthy school aged Greek children K.H. Schulpis, G.A. Karikas, S. Gavrili

∫. ™Ô‡ÏË, °.∞. ∫·Ú›Î·˜, ™. °·‚Ú›ÏË

∆ÔÈ΋ ·ÓÙÈÊÏÂÁÌÔÓ҉˘ ·ÁˆÁ‹ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ Ï·Ú˘ÁÁÔÙÚ·¯ÂÈÔ‚ÚÔÁ¯›Ùȉ·˜

59

¶. ™Ù·ÌÔ‡Ï˘, Ã. ÃÚ˘Û·ÓıfiÔ˘ÏÔ˜, ª. ∂ÌÔÚÈ¿‰Ô˘, ¡. ∆Û¤˘, ∂. °ÂˆÚÁÈ¿‰Ô˘, ¡. ª·ÁÎÏ·‚¤Ú·˜

Topical anti-inflammatory therapy in the management of acute laryngotracheitis P. Stamoulis, C. Chryssanthopoulos, M. Emporiadou, N. Tsepis, E. Georgiadou, N. Maglaveras

∂›‰· ‚ÈÙ·ÌÈÓÒÓ ∞ Î·È ∂ ÛÙÔÓ ÔÚfi ·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË Û ıÂÚ·›· ˘ÔηٿÛÙ·Û˘ Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì· Î·È ÏÈÔ‰È·Ï˘Ù¤˜ ‚Èٷ̛Ә

65

S. Nousia-Arvanitakis, A. Galli-Tsinopoulou, N. RÔubies, K. Tentzidou, M. Xefteri

™. ¡Ô‡ÛÈ·-∞Ú‚·ÓÈÙ¿ÎË, ∞. °·ÏÏ‹-∆ÛÈÓÔÔ‡ÏÔ˘, ¡. ƒÔ˘ÌȤ˜, ∫. ∆ÂÓÙ˙›‰Ô˘, ª. •ÂÊÙ¤ÚË

∂¡¢π∞º∂ƒ√À™∂™ ¶∂ƒπ¶∆ø™∂π™ “∫ÚÂÌ¿ÌÂÓÔ˜” ÛÏ‹Ó·˜ Û ÎÔÚ›ÙÛÈ 7 ÂÙÒÓ

72

CASE REPORTS “Wandering” spleen in a 7 year old girl K. Chaidopoulou, M. Stamou-Tsiaprazi, F. Athanasiadou, K. Kouskouras, A. Antoniadis

∫. ÷˚‰ÔÔ‡ÏÔ˘, ª. ™Ù¿ÌÔ˘-∆ÛÈ·Ú¿˙Ë, º. ∞ı·Ó·ÛÈ¿‰Ô˘, ∫. ∫Ô‡ÛÎÔ˘Ú·˜, ∞. ∞ÓÙˆÓÈ¿‰Ë˜

∫ÏËÚÔÓÔÌÈ΋ ‰È·Ù·Ú·¯‹ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (ÁÈÁ·ÓÙÈ·›·) Ô˘ ÂΉËÏÒıËÎÂ Û·Ó “„¢‰ÔıÚÔÌ‚ÔÂÓ›·” ¶ÂÚÈÁÚ·Ê‹ ‰‡Ô ÂÚÈÙÒÛˆÓ

Serum vitamin A and E levels in cystic fibrosis patients supplemented with pancreatic enzymes and liposoluble vitamins

76

Hereditary giant platelet disorder presenting as "pseudothrombocytopenia" A report of two cases E. Hatzipantelis, M. Athanassiou-Metaxa, H. Tsantali, K. Avramidou, D. Zambuli, N. Gombakis

∂. ÷Ù˙Ë·ÓÙÂÏ‹˜, ª. ∞ı·Ó·Û›Ô˘-ªÂÙ·Í¿, Ã. ∆Û¿ÓÙ·ÏË, ∫. ∞‚Ú·Ì›‰Ô˘, ¢. ∑·ÌÔ‡ÏË, ¡. °ÔÌ¿Î˘

ŒÏÏÂÈ„Ë ÙÔ˘ ÎÏ¿ÛÌ·ÙÔ˜ C6 ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ Î·È ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈΤ˜ ÏÔÈÌÒÍÂȘ

81

M. Theodoridou, M. Ioannou, G. Mostrou, A. Germenis

ª. £ÂÔ‰ˆÚ›‰Ô˘, ª. πˆ¿ÓÓÔ˘, °. ªÔÛÙÚÔ‡, ∞. °ÂÚÌÂÓ‹˜

¶Ó¢ÌÔÓÈÔÎÔÎÎÈ΋ ÌËÓÈÁÁ›Ùȉ· Ì ·Ú¯ÈÎÒ˜ Ê˘ÛÈÔÏÔÁÈÎfi ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi Û ÌË ·ÓÔÛÔηٷÛÙ·Ï̤ÓÔ ¿ÚÚˆÛÙÔ

C6 complement deficiency and meningococcal infections

85

Streptococcus pneumoniae meningitis with initial normal cerebrospinal fluid findings in a non-immunocompomised patient

∞. ÷Ù˙Ë·Ó·Á‹˜, ª. ™È‰ÂÚ¿

A. Hadjipanayis, M. Sidera

™˘Ó¯›˙ÔÓÙ·È

Continued

January - February 2001 . Volume 64 . No 1


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 6

π·ÓÔ˘¿ÚÈÔ˜ - ºÂ‚ÚÔ˘¿ÚÈÔ˜ 2001 . ∆fiÌÔ˜ 64 . ∆‡¯Ô˜ 1

™˘Ó¤¯ÂÈ· ÂÚȯÔ̤ӈÓ

Continuation of table of contents

K·ÏÔ‹ı˘ ·ÚÔ‰È΋ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›· Û ·ÁfiÚÈ 16 ÌËÓÒÓ

87

¡. °Î·Ì›Ï˘, °. ∆ÛfiÏ·˜

N. Gamilis, G. Tsolas

¶∞π¢π∞∆ƒπ∫∏ ∂¡∏ª∂ƒø™∏

92

∑. ¶··‰ÔÔ‡ÏÔ˘-∫Ô˘ÏÔ˘Ì‹ 92

¶∂ƒπ§∏æ∂π™ ∞ƒ£ƒø¡ ∞¶√ ∆∏ µπµ§π√°ƒ∞ºπ∞ µÚÔÁ¯È΋ ÚÔÛÙ·Û›· Ì ¤Ó· ·ÓÙ·ÁˆÓÈÛÙ‹ Ï¢ÎÔÙÚÈÂÓÈÎÒÓ ˘Ô‰Ô¯¤ˆÓ Û ·ÛıÌ·ÙÈο ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜

24

H. Bisgaard, K. Nielsen 71

C. Bosken, WC. Hunt, WE. Lambert, JM. Samet ∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: £. ∆ÛÈÏÈÁÈ¿ÓÓ˘

LITERATURE ABSTRACTS Bronchoprotection with a leukotriene receptor antagonist in asthmatic preschool children

A parental history of asthma is a risk factor for wheezing and nonwheezing respiratory illnesses in infants younger than 18 months of age C. Bosken, WC. Hunt, WE. Lambert, JM. Samet Greek translation: Th. Tsiligiannis

91

Hyams JS, Markowitz J, Wyllie R ∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: °.π. ∫·ÊÚ›ÙÛ·

§Ô›ÌˆÍË ·fi ÂÏÈÎÔ‚·ÎÙËÚ›‰ÈÔ ÙÔ˘ ˘ÏˆÚÔ‡ Û ·Û˘Ìو̷ÙÈο ·È‰È¿: Û‡ÁÎÚÈÛË ‰È·ÁÓˆÛÙÈÎÒÓ ‰ÔÎÈÌ·ÛÈÒÓ

Communication

H. Bisgaard, K. Nielsen Greek translation: Th. Tsiligiannis

∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: £. ∆ÛÈÏÈÁÈ¿ÓÓ˘

£ÂÚ·›· Ù˘ ÓfiÛÔ˘ ÙÔ˘ Crohn Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ·ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ ÙÔ˘ ·Ú¿ÁÔÓÙ· Ó¤ÎÚˆÛ˘ ÙˆÓ fiÁÎˆÓ - ¿ÏÊ·

PAEDIATRIC NEWS ∑. Papadopoulou-Couloumbis

∞Ó·ÎÔ›ÓˆÛË

∆Ô ÈÛÙÔÚÈÎfi ¿ÛıÌ·ÙÔ˜ ÛÙÔ˘˜ ÁÔÓ›˜ Â›Ó·È ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘ ÂΉ‹ÏˆÛ˘ ·Ó·Ó¢ÛÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ Ì ‹ ¯ˆÚ›˜ Û˘ÚÚ›ÙÔ˘Û· ·Ó·ÓÔ‹ Û ‚Ú¤ÊË ËÏÈΛ·˜ οو ÙˆÓ 18 ÌËÓÒÓ

Benign transient hyperphosphatasaemia in a 16month old boy

Use of infliximab in the treatment of Crohn’s disease in children and adolescents Hyams JS, Markowitz J, Wyllie R Greek translation: G.I. Kafritsa

91

Malaty HM, Logan ND, Graham DY, Ramchatesingh JE, Reddy SG ∞fi‰ÔÛË ÛÙ· ∂ÏÏËÓÈο: °.π. ∫·ÊÚ›ÙÛ·

Helicobacter pylori infection in asymptomatic children: comparison of diagnostic tests Malaty HM, Logan ND, Graham DY, Ramchatesingh JE, Reddy SG Greek translation: G.I. Kafritsa

¶ÚÔÛ¯‹ ™˘Ó¤‰ÚÈ·

xi

Scheduled Medical Meetings

™˘ÓÙÔÌÔÁڷʛ˜

xv

Abbreviations

January - February 2001 . Volume 64 . No 1


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 7

π·ÓÔ˘¿ÚÈÔ˜ - ºÂ‚ÚÔ˘¿ÚÈÔ˜ 2001

. ∆fiÌÔ˜

64

. ∆‡¯Ô˜

1

¢ÈÌËÓÈ·›· ¤Î‰ÔÛË E›ÛËÌÔ ¶ÂÚÈÔ‰ÈÎfi Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜

Bimonthly Publication The Official Journal of the Hellenic Paediatric Society

EΉfiÙ˘ K. °ÚÈ‚¤·˜

Publisher K. Griveas

I‰ÈÔÎÙ‹Ù˘© EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›· Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 Aı‹Ó·È 115 28

Owner© Hellenic Paediatric Society Michalakopoulou 92 Athens 115 28

TËÏ:. (01) 7771 140 / 7771 663, Fax: 7758 354

Tel. (01) 7771 140 / 7771 663, Fax: 7758 354

EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ¶Úfi‰ÚÔ˜ : ∫. ª·Ï·Î¿ ¢È¢ı˘ÓÙ‹˜ : ∑. ¶··‰ÔÔ‡ÏÔ˘-∫Ô˘ÏÔ˘Ì‹ M¤ÏË : ∂. ∞ÁÁÂÏ¿ÎË-°ÂˆÚÁ¿ÎË : º. Aı·Ó·ÛÈ¿‰Ô˘-¶ÈÂÚÔÔ‡ÏÔ˘ : ª. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ : °. µ·ÚÏ¿Ì˘ : ¶. ∫·ÊÚ›ÙÛ· : ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ : £. ∆ÛÈÏÈÁÈ¿ÓÓ˘ : ∫. ∆ÛÔ˘Ì¿Î·˜ : ™. ºˆÙfiÔ˘ÏÔ˜ : A. ÷Ù˙‹˜

Scientific President Editor Members

ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ· ∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈÌ¤ÓˆÓ º. ª·˘ÚÔÂȉ‹

Manuscript Editing

∂È̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ∑. ¶··‰ÔÔ‡ÏÔ˘-∫Ô˘ÏÔ˘Ì‹

English Editing Z. Papadopoulou-Couloumbis

∞ÏÏËÏÔÁÚ·Ê›· ∂ÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ ∂Ù·ÈÚ›· ªÈ¯·Ï·ÎÔÔ‡ÏÔ˘ 92 ∞ı‹Ó·È 115 28 ∆ËÏ: (01) 7771 140, Fax: 7758 354

Correspondence Hellenic Paediatric Society Michalakopoulou 92 Athens 115 28 Tel: (01) 7771 140, Fax: 7758 354

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Editorial Board : K. Malaka : Z. Papadopoulou-Couloumbis : E. Agelaki-Georgaki : F. Athanassiadou-Piperopoulou : M. Anthrakopoulos : G. Varlamis : P. Kafritsa : A. Konstadopoulos : Th. Tsiligiannis : K. Tsoumakas : S. Fotopoulos : ∞. Hatzis

. Volume

64

.

No 1


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 9

O‰ËÁ›Â˜ ÚÔ˜ ÙÔ˘˜ ™˘ÁÁÚ·Ê›˜ A. °ÂÓÈΤ˜ ¶ÏËÚÔÊÔڛ˜ ∆Ô ÂÚÈÔ‰ÈÎfi "¶∞π¢π∞∆ƒπ∫∏" Â›Ó·È Ë Â›ÛËÌË ÂÈÛÙËÌÔÓÈ΋ ¤Î‰ÔÛË Î·È È‰ÈÔÎÙËÛ›· Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜. Œ¯ÂÈ ˆ˜ ‚·ÛÈÎÔ‡˜ ÛÙfi¯Ô˘˜ ÙËÓ ·ÔÙ‡ˆÛË ÙÔ˘ ·È‰È·ÙÚÈÎÔ‡ ¤ÚÁÔ˘ ÛÙËÓ ∂ÏÏ¿‰· Î·È ÙË Û˘Ó¯‹ È·ÙÚÈ΋ ÂÓË̤ڈÛË Î·È ÂÈÌfiÚʈÛË ÙˆÓ EÏÏ‹ÓˆÓ ¶·È‰È¿ÙÚˆÓ. °È· ÙÔ ÛÎÔfi ·˘Ùfi ‰¤¯ÂÙ·È ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÔÈÎÈÏ›· ¿ÚıÚˆÓ Î·È Û˘ÁÎÂÎÚÈ̤ӷ: ¿ÚıÚ· Û‡ÓÙ·Í˘ (ÌÂÙ¿ ·fi ÚfiÛÎÏËÛË Ù˘ EÈÛÙËÌÔÓÈ΋˜ ™˘ÓÙ·ÎÙÈ΋˜ EÈÙÚÔ‹˜), ·Ó·ÛÎÔ‹ÛÂȘ, Â›Î·ÈÚ· ı¤Ì·Ù·, ÂÍÂÏ›ÍÂȘ ÛÙËÓ π·ÙÚÈ΋ ∂ÈÛÙ‹ÌË, ÚˆÙfiÙ˘˜ ÂÚ¢ÓËÙÈΤ˜ ÌÂϤÙ˜ Ì ·È‰È·ÙÚÈÎfi ‹ È·ÙÚÔÎÔÈÓˆÓÈÎfi ÂÚȯfiÌÂÓÔ, ÁÂÓÈο ı¤Ì·Ù· Ô˘ ·ÊÔÚÔ‡Ó ÙÔÓ ÚÔÁÚ·ÌÌ·ÙÈÛÌfi Î·È ÙËÓ ÔÚÁ¿ÓˆÛË Ù˘ È·ÙÚÈ΋˜ ÂÎ·›‰Â˘Û˘ Î·È ˘Á›·˜, ‚Ú·‚Â˘Ì¤Ó˜ ÂÚÁ·Û›Â˜, ÂÈÏÂÁ̤Ó˜ Û˘˙ËÙ‹ÛÂȘ ÛÙÚÔÁÁ˘ÏÒÓ ÙÚ·Â˙ÒÓ, ÂӉȷʤÚÔ˘Û˜ ÎÏÈÓÈΤ˜ Î·È ·ÂÈÎÔÓÈÛÙÈΤ˜ ÂÚÈÙÒÛÂȘ, ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ, ‚È‚ÏÈÔÎÚÈۛ˜, ÂÈÛÙÔϤ˜ ÚÔ˜ ÙË Û‡ÓÙ·ÍË, ÂÚÈÏ‹„ÂȘ Ù˘ ÂÏÏËÓÈ΋˜ Î·È Í¤Ó˘ ‚È‚ÏÈÔÁÚ·Ê›·˜, ·Ó·ÎÔÈÓÒÛÂȘ ÚÔÛ¯ÒÓ Û˘ÌÔÛ›ˆÓ Î·È Û˘Ó‰ڛˆÓ Û ı¤Ì·Ù· Û¯ÂÙÈο Ì ÙËÓ ¶·È‰È·ÙÚÈ΋ Î·È ·Ó·ÊÔÚ¤˜ Ó¤ˆÓ ÂΉfiÛˆÓ, ÂÏÏËÓÈÎÒÓ Î·È Í¤ÓˆÓ, ·È‰È·ÙÚÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜. H EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‰È·ÙËÚ› ÙÔ ‰Èη›ˆÌ· Ó· ‰ËÌÔÛȇÂÈ ¿ÚıÚ· Ì ȉȷ›ÙÂÚÔ ÂÈÛÙËÌÔÓÈÎfi ÂӉȷʤÚÔÓ ¯ˆÚ›˜ Ó· ÙËÚÂ›Ù·È Ë ÛÂÈÚ¿ ˘Ô‚ÔÏ‹˜ Î·È Ó· ·ÔÊ·Û›˙ÂÈ ÁÈ· ÙË ‰ËÌÔÛ›Â˘ÛË ÂÚÁ·ÛÈÒÓ Ô˘ ¤¯Ô˘Ó ‚Ú·‚¢ı› ÛÙ· Ï·›ÛÈ· ÙÔ˘ ÂÙ‹ÛÈÔ˘ ¶·È‰È·ÙÚÈÎÔ‡ ™˘Ó‰ڛԢ, ÙËÓ ÔÏÈ΋ ‹ ÌÂÚÈ΋ ‰ËÌÔÛ›Â˘ÛË ÛÙÚÔÁÁ˘ÏÒÓ ÙÚ·Â˙ÒÓ È‰È·›ÙÂÚÔ˘ ÂӉȷʤÚÔÓÙÔ˜, Î·È ÙËÓ ÔÏÈ΋ ‹ ÌÂÚÈ΋ ‰ËÌÔÛ›Â˘ÛË ÂÈÛÙÔÏÒÓ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Û ‰ËÌÔÛÈÂ˘Ì¤Ó· ÂÈÛÙËÌÔÓÈο ¿ÚıÚ· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. µ. ÀÔ‚ÔÏ‹ Î·È ¢ËÌÔÛ›Â˘ÛË ÙˆÓ ∂ÚÁ·ÛÈÒÓ OÈ ÂÚÁ·Û›Â˜ Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È ÚÔ˜ ÎÚ›ÛË ÁÈ· ‰ËÌÔÛ›Â˘ÛË ÛÙÔ ÂÚÈÔ‰ÈÎfi "¶∞π¢π∞∆ƒπ∫∏" ·ÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó ÛÙË ‰È‡ı˘ÓÛË: EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›· Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 115 28 Aı‹Ó· ŸÏ˜ ÔÈ ÂÚÁ·Û›Â˜ Ú¤ÂÈ Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ÂÈÛÙÔÏ‹, Ë ÔÔ›· ı· Û˘Ó˘ÔÁÚ¿ÊÂÙ·È ·fi fiÏÔ˘˜ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ ÛÙËÓ ÔÔ›· ı· ‰ËÏÒÓÂÙ·È fiÙÈ Ë ÂÚÁ·Û›· ‰ÂÓ ¤¯ÂÈ ÂÓ Ì¤ÚÂÈ ‹ ÂÍ ÔÏÔÎÏ‹ÚÔ˘ ‰ËÌÔÛÈ¢ı› ‹ ˘Ô‚ÏËı› ÁÈ· ÎÚ›ÛË Û ¿ÏÏÔ ÂÚÈÔ‰ÈÎfi Î·È fiÙÈ ÔÈ Û˘ÁÁÚ·Ê›˜ ÂÁÎÚ›ÓÔ˘Ó ÙË ‰ËÌÔÛ›Â˘Û‹ Ù˘ ÛÙÔ ÂÚÈÔ‰ÈÎfi "¶∞π¢π∞∆ƒπ∫∏". ∂ÈϤÔÓ, ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È Ù˘¯fiÓ ÂȉÔÙ‹ÛÂȘ - ¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢. ŸÏ˜ ÔÈ ÎÏÈÓÈΤ˜ ¤Ú¢Ó˜ Ú¤ÂÈ Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ÁÚ·Ù‹ ‰‹ÏˆÛË ÙˆÓ Û˘ÁÁڷʤˆÓ fiÙÈ ¤ÁÈÓ·Ó ÌÂÙ¿ ·fi ÏËÚÔÊÔÚË̤ÓË Û˘Ó·›ÓÂÛË ÙˆÓ ÌÂÙ¯fiÓÙˆÓ ‹ ÙˆÓ ÓÔÌ›ÌˆÓ ÂÎÚÔÛÒˆÓ ÙÔ˘˜ Û‡Ìʈӷ Ì ÙȘ ‰È·ÎËÚ‡ÍÂȘ ÙÔ˘ EÏÛ›ÓÎÈ Î·È ÙÔ˘ TfiÎÈÔ. ŸÏ˜ ÔÈ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ıˆÚÔ‡ÓÙ·È È‰ÈÔÎÙËÛ›· ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ "¶∞π¢π∞∆ƒπ∫∏" Î·È Ë ÔÏÈ΋ ‹ ÌÂ-

ÚÈ΋ ·Ó·‰ËÌÔÛ›Â˘Û‹ ÙÔ˘˜ ÂÈÙÚ¤ÂÙ·È ÌfiÓÔ ÌÂÙ¿ ·fi ¤ÁÁÚ·ÊË Û˘ÁηٿıÂÛË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ∏ ¶∞π¢π∞∆ƒπ∫∏ ÚÔÙ›ÓÂÈ Ó· ·ÎÔÏÔ˘ıÔ‡ÓÙ·È ÔÈ “∫ÔÈÓ¤˜ ¶ÚԉȷÁڷʤ˜ ÁÈ· ÃÂÈÚfiÁÚ·Ê· Ô˘ ˘Ô‚¿ÏÏÔÓÙ·È Û µÔ˚·ÙÚÈο ¶ÂÚÈÔ‰Èο” (Uniform Requirements for Manuscripts Submitted to Biomedical Journals) Ô˘ ‰ËÌÔÛȇÙËÎ·Ó ÛÙÔ JAMA 1997;277:927934, http://jama.ama-assn.org/info/auinst_req.html. OÈ ·fi„ÂȘ Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ Ô˘ ‰È·Ù˘ÒÓÔÓÙ·È ÛÙȘ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ ‰ÂÓ ·ÓÙÈηÙÔÙÚ›˙Ô˘Ó ··Ú·›ÙËÙ· ·˘Ù¤˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. H ηٷ¯ÒÚËÛË ‰È·ÊËÌ›ÛÂˆÓ ÛÙÔ ÂÚÈÔ‰ÈÎfi ‰ÂÓ ˘Ô‰ËÏÒÓÂÈ ÔˆÛ‰‹ÔÙ ¤ÁÎÚÈÛË ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘˜ ·fi ÙËÓ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·, ÙËÓ EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ‹ ·fi ÙÔÓ EΉfiÙË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. EÊfiÛÔÓ Ë ÂÚÁ·Û›· Á›ÓÂÈ ·Ô‰ÂÎÙ‹, ÙÔ ‰ÈÔÚıˆÌ¤ÓÔ Û‡Ìʈӷ Ì ÙȘ ˘ԉ›ÍÂȘ ÙˆÓ ÎÚÈÙÒÓ Î·È ÂÎ Ó¤Ô˘ ‰·ÎÙ˘ÏÔÁÚ·ÊË̤ÓÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ˘Ô‚¿ÏÂÙ·È ÛÙËÓ EÈÛÙËÌÔÓÈ΋ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ Û˘Óԉ¢fiÌÂÓÔ ·fi ‰ÈÛΤٷ Ô˘ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÚÁ·Û›· Û ÚfiÁÚ·ÌÌ· Word .doc ‹ MacWrite Î·È ÙÔ˘˜ ›Ó·Î˜, ·Ó Â›Ó·È ‰˘Ó·ÙfiÓ, Û ÚfiÁÚ·ÌÌ· Exel ηıÒ˜ Î·È ÂÈÛÙÔÏ‹ fiÔ˘ Ó· ·Ó·Ê¤ÚÔÓÙ·È ·Ó·Ï˘ÙÈο ÔÈ ÙÚÔÔÔÈ‹ÛÂȘ ‹ ÔÈ ·ÓÙÈÚÚ‹ÛÂȘ ÛÙȘ ÚÔÙ¿ÛÂȘ ÙˆÓ ÎÚÈÙÒÓ. H ηı˘ÛÙ¤ÚËÛË Ù˘ Â·Ó˘Ô‚ÔÏ‹˜ ÙÔ˘ ÙÚÔÔÔÈË̤ÓÔ˘ ÎÂÈ̤ÓÔ˘ ¤Ú·Ó ÙˆÓ 4 ÌËÓÒÓ Û˘ÓÂ¿ÁÂÙ·È Ó¤· ˘Ô‚ÔÏ‹. °È· Ó· ÂÈÙ¢¯ı› Ë ¤ÁηÈÚË ‰ËÌÔÛ›Â˘ÛË Ù˘ ÂÚÁ·Û›·˜ Â›Ó·È ··Ú·›ÙËÙÔ Ó· ÂÈÛÙÚ¤ÊÂÙ·È ÙÔ ‰ÈÔÚıˆÌ¤ÓÔ Ù˘ÔÁÚ·ÊÈÎfi ‰ÔΛÌÈÔ ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ ̤۷ Û ÂÙ¿ (7) Ë̤Ú˜. T· ¤ÍÔ‰· ÙˆÓ ÊÈÏ̘ Î·È ÙˆÓ ·Ó·Ù‡ˆÓ ‚·Ú‡ÓÔ˘Ó ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ Î·È Ú¤ÂÈ Ó· ÏËÚÒÓÔÓÙ·È Ì ÙËÓ ·ÔÛÙÔÏ‹ ÙÔ˘ ÚÒÙÔ˘ ‰ÔÎÈÌ›Ô˘ ηÙ¢ı›·Ó ÛÙÔÓ Ù˘ÔÁÚ¿ÊÔ. T· ΛÌÂÓ· ÙˆÓ ÂÚÁ·ÛÈÒÓ Ô˘ ‰ÂÓ Á›ÓÔÓÙ·È ·Ô‰ÂÎÙ¤˜ ÁÈ· ‰ËÌÔÛ›Â˘ÛË ‰ÂÓ ÂÈÛÙÚ¤ÊÔÓÙ·È. MÔÚ› Ó· ÂÈÛÙÚ·ÊÔ‡Ó, ÂÊfiÛÔÓ ˙ËÙËı›, Ù· Û¯‹Ì·Ù· Î·È ÔÈ ÊˆÙÔÁڷʛ˜ Ô˘ Ù· Û˘Óԉ‡ԢÓ. °. ™‡ÓÙ·ÍË ÙˆÓ ∂ÚÁ·ÛÈÒÓ TÔ Û‡ÓÔÏÔ Ù˘ ÂÚÁ·Û›·˜ (Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ ÙˆÓ ÈÓ¿ÎˆÓ Î·È ÙˆÓ Ù›ÙÏˆÓ ÙˆÓ ÂÈÎfiÓˆÓ) Ú¤ÂÈ Ó· Â›Ó·È ‰·ÎÙ˘ÏÔÁÚ·ÊË̤ÓÔ ÛÙË ÌÈ· fi„Ë Ï¢ÎÔ‡ ¯·ÚÙÈÔ‡ ÌÂÁ¤ıÔ˘˜ A4 (22x28 cm) Ì ‰ÈÏfi ‰È¿ÛÙËÌ· ÌÂٷ͇ ÙˆÓ ÁÚ·ÌÌÒÓ Î·È Ê·Ú‰È¿ ÂÚÈıÒÚÈ· (2,5 cm) ÛÙȘ ‰‡Ô Ï¢ڤ˜. TÔ Î›ÌÂÓÔ Ú¤ÂÈ Ó· ¤¯ÂÈ ÙËÓ ·ÎfiÏÔ˘ıË ‰È¿Ù·ÍË: ÛÂÏ›‰· Ù›ÙÏÔ˘, ‚Ú·¯‡˜ Ù›ÙÏÔ˜, ÂÚ›ÏË„Ë ÛÙ· ÂÏÏËÓÈο Î·È Ù· ·ÁÁÏÈο, ΛÌÂÓÔ, ¢¯·ÚÈÛٛ˜ ηıÒ˜ Î·È ·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢, ‚È‚ÏÈÔÁÚ·Ê›·, ›Ó·Î˜, ÂÈÎfiÓ˜, Ù›ÙÏÔÈ ÂÈÎfiÓˆÓ, ηٿÏÔÁÔ˜ Û˘ÓÙÌ‹ÛˆÓ. K¿ı ¤Ó· ·fi Ù· ·Ú·¿Óˆ ÛÙÔȯ›· Ú¤ÂÈ Ó· ·Ú¯›˙ÂÈ Û ¯ˆÚÈÛÙfi ʇÏÏÔ Î·È ÔÈ ÛÂÏ›‰Â˜ Ó· ·ÚÈıÌÔ‡ÓÙ·È ‰È·‰Ô¯Èο ·Ú¯›˙ÔÓÙ·˜ Ì ÙË ÛÂÏ›‰· Ù›ÙÏÔ˘.


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 10

H ¤ÎÙ·ÛË ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ÁÈ· ÙȘ ·Ó·ÛÎÔ‹ÛÂȘ Û 15-25, ÁÈ· ÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ Û 12-15 ÛÂÏ›‰Â˜, ÁÈ· Ù· Â›Î·ÈÚ· ı¤Ì·Ù· Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ Û 3-5 ‰·ÎÙ˘ÏÔÁÚ·ÊË̤Ó˜ ÛÂÏ›‰Â˜, ÂÓÒ ÁÈ· ÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ Û 1000 ϤÍÂȘ Î·È ÁÈ· ÙȘ ÂÈÛÙÔϤ˜ Û 250-300 ϤÍÂȘ. ™ÂÏ›‰· Ù›ÙÏÔ˘ ¶Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ: - ÙÔÓ Ù›ÙÏÔ (<14 ϤÍÂȘ) ηıÒ˜ Î·È ÙÔÓ ‚Ú·¯‡ Ù›ÙÏÔ (<5 ϤÍÂȘ) ÙÔ˘ ¿ÚıÚÔ˘. ¢ÂÓ ÂÈÙÚ¤ÔÓÙ·È Û˘ÓÙÌ‹ÛÂȘ, - ÙÔ fiÓÔÌ· Î·È ÙÔ ÂÒÓ˘ÌÔ Î¿ıÂ Û˘ÁÁڷʤ· ÛÙËÓ ÔÓÔÌ·ÛÙÈ΋, - ÙÔ ÂÈÛÙËÌÔÓÈÎfi ΤÓÙÚÔ (›‰Ú˘Ì·, ÎÏÈÓÈ΋, ÂÚÁ·ÛÙ‹ÚÈÔ) ·fi fiÔ˘ ÚÔ¤Ú¯ÂÙ·È Ë ÂÚÁ·Û›·. ™Â ÂÚ›ÙˆÛË ¤ÏÏÂȄ˘ Û˘ÓÂÚÁ·Û›·˜ ÌÂ Û˘ÁÎÂÎÚÈ̤ӷ ΤÓÙÚ·, Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÂÙ·È Ë È‰ÈfiÙËÙ· ÙÔ˘/ÙˆÓ Û˘ÁÁڷʤˆÓ (.¯. ȉÈÒÙ˘ ·È‰›·ÙÚÔ˜) Î·È Ô ÙfiÔ˜ ‰È·ÌÔÓ‹˜ ÙÔ˘/ÙˆÓ, - ÙËÓ Ï‹ÚË ‰È‡ı˘ÓÛË Î·È ÙÔ ÙËϤʈÓÔ ÙÔ˘ Û˘ÁÁڷʤ· Ì ÙÔÓ ÔÔ›Ô Á›ÓÂÙ·È Ë ·ÏÏËÏÔÁÚ·Ê›·. ¶ÂÚÈÏ‹„ÂȘ H ÂÚ›ÏË„Ë Ú¤ÂÈ Ó· ·Ó·ÎÂÊ·Ï·ÈÒÓÂÈ ÙÔ˘˜ ÛÙfi¯Ô˘˜ Ù˘ ÂÚÁ·Û›·˜, ÙË ÌÂıÔ‰ÔÏÔÁ›·, Ù· ΢ÚÈfiÙÂÚ· ·ÔÙÂϤÛÌ·Ù· Î·È Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ù˘ ÌÂϤÙ˘. ¢ÂÓ Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÂÈ ÙȘ 250 ϤÍÂȘ. ™ÙËÓ ÂÚ›ÏË„Ë ÛÙ· ·ÁÁÏÈο Ú¤ÂÈ Ó· ·Ó·ÁÚ¿ÊÔÓÙ·È ÛÙËÓ ·Ú¯‹ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Ô Ù›ÙÏÔ˜ Ù˘ ÂÚÁ·Û›·˜ Î·È Ù· ÔÓfiÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ ÛÙ· ·ÁÁÏÈο. TÔ ÂÚȯfiÌÂÓÔ ÙÔ˘ ·ÁÁÏÈÎÔ‡ ÎÂÈ̤ÓÔ˘ ‰ÂÓ Ú¤ÂÈ Ó· ‰È·Ê¤ÚÂÈ ·fi ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô ÂÏÏËÓÈÎfi. T· ¿ÚıÚ· Û‡ÓÙ·Í˘ Î·È ÔÈ ·ÂÈÎÔÓÈÛÙÈΤ˜ ÂÚÈÙÒÛÂȘ ¤¯Ô˘Ó ÌfiÓÔ ·ÁÁÏÈ΋ ÂÚ›ÏË„Ë. K¿Ùˆ ·fi ÙËÓ ÂÏÏËÓÈ΋ Î·È ÙËÓ ·ÁÁÏÈ΋ ÂÚ›ÏË„Ë ÛËÌÂÈÒÓÔÓÙ·È ÙÚÂȘ ¤ˆ˜ ¤ÓÙ ϤÍÂȘ ÎÏÂȉȿ Ô˘ ı· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ÁÈ· ÙÔ ıÂÌ·ÙÈÎfi ¢ÚÂÙ‹ÚÈÔ. K›ÌÂÓÔ OÈ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ÂÈÛ·ÁˆÁ‹, ÌÂıfi‰Ô˘˜, ·ÔÙÂϤÛÌ·Ù· Î·È Û˘˙‹ÙËÛË. H ∂ÈÛ·ÁˆÁ‹ ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· ÙÂÏÂ˘Ù·›· ‰Â‰Ô̤ӷ Ù˘ ¤Ú¢ӷ˜ ÛÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ı¤Ì· Ì ÙȘ ΢ÚÈfiÙÂÚ˜ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ Î·È ÙÔ ÛÎÔfi Ù˘ ÂÚÁ·Û›·˜. H ÂÚÈÁÚ·Ê‹ ÙˆÓ ªÂıfi‰ˆÓ Ú¤ÂÈ Ó· Â›Ó·È ·ÎÚÈ‚‹˜ Î·È ·ÚÎÂÙ¿ ÏÂÙÔÌÂÚ‹˜ ÒÛÙ ӷ ÂÈÙÚ¤ÂÈ ÙËÓ ·Ó··Ú·ÁˆÁ‹ ÙÔ˘˜ ·fi ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜. T· ∞ÔÙÂϤÛÌ·Ù· Ú¤ÂÈ Ó· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ۷ʋÓÂÈ· Î·È Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ÙËÓ ··Ú·›ÙËÙË ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË. H ™˘˙‹ÙËÛË Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· Û˘ÌÂÚ¿ÛÌ·Ù· Ô˘ ÚÔ·ÙÔ˘Ó ·fi ÙËÓ ÂÚÁ·Û›·, ÙË ÛËÌ·Û›· Ô˘ ÌÔÚ› Ó· ¤¯Ô˘Ó Î·È ÙËÓ Èı·Ó‹ Û˘Û¯¤ÙÈÛ‹ ÙÔ˘˜ Ì ·Ú·ÙËÚ‹ÛÂȘ ¿ÏÏˆÓ ÂÚ¢ÓËÙÒÓ. OÈ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û‡ÓÙÔÌË ÂÈÛ·ÁˆÁ‹, ÂÚÈÁÚ·Ê‹ Ù˘ ÂÚ›ÙˆÛ˘ Î·È ‚Ú·¯Â›· Û˘˙‹ÙËÛË. OÈ ·ÂÈÎÔÓÈÛÙÈΤ˜ ÂÚÈÙÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û‡ÓÙÔÌË ÂÚÈÁÚ·Ê‹ Ù˘ ÂÚ›ÙˆÛ˘ ηÈ

‚Ú·¯Â›· Û˘˙‹ÙËÛË Ì ¤ÌÊ·ÛË ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË. T· ˘fiÏÔÈ· ı¤Ì·Ù· ¤¯Ô˘Ó ÂχıÂÚË ‰ÔÌ‹ ηٿ ÙËÓ ÎÚ›ÛË ÙˆÓ Û˘ÁÁڷʤˆÓ ÙÔ˘˜. ∂˘¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ - ¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢) ı· Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Ù¤ÏÔ˜ ÙÔ˘ ÎÂÈ̤ÓÔ˘ Î·È ÚÈÓ ·fi ÙȘ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜. TÈ̤˜ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ OÈ ÙÈ̤˜ ÙˆÓ ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÂÍÂÙ¿ÛÂˆÓ Ú¤ÂÈ Ó· ÂÎÊÚ¿˙ÔÓÙ·È ÛÙÔ ¢ÈÂıÓ¤˜ ™‡ÛÙËÌ· MÔÓ¿‰ˆÓ (SI Units) Î·È ÛÙÔ MÂÙÚÈÎfi (Conventional-™˘Ì‚·ÙÈÎfi) ™‡ÛÙËÌ· ̤۷ Û ·Ú¤ÓıÂÛË. ¶›Ó·Î˜ ÌÂÙ·ÙÚÔ‹˜ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÛÙÔ JAMA 1986;255:2329-2339. ™˘ÓÙÔÌÔÁڷʛ˜ OÈ Û˘ÓÙÔÌÔÁڷʛ˜ Ô˘ ¤¯Ô˘Ó ÂÈ‚ÏËı› ‰ÈÂıÓÒ˜ ‰ËÌÔÛȇÔÓÙ·È Û οı Ù‡¯Ô˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡. ™‡ÓıÂÙÔÈ Î·È Ì·ÎÚÔÛÎÂÏ›˜ fiÚÔÈ Ô˘ Â·Ó·Ï·Ì‚¿ÓÔÓÙ·È Û˘¯Ó¿ ÛÙÔ Î›ÌÂÓÔ ÌÔÚÔ‡Ó Ó· ·ÓÙÈηı›ÛÙ·ÓÙ·È ·fi Û˘ÓÙÔÌÔÁڷʛ˜ Ô˘ ÂÂÍËÁÔ‡ÓÙ·È ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ Û ÂȉÈÎfi ηٿÏÔÁÔ Ô ÔÔ›Ô˜ ˘Ô‚¿ÏÏÂÙ·È Ì·˙› Ì ÙËÓ ÂÚÁ·Û›·. OÈ Û˘ÓÙÔÌÔÁڷʛ˜ ·Ó·Ê¤ÚÔÓÙ·È Û ·Ú¤ÓıÂÛË ÌfiÓÔ ÛÙȘ ÂÚÈÏ‹„ÂȘ. BÈ‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ ™ÙÔ ÙÌ‹Ì· Ù˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ ηٷ¯ˆÚÔ‡ÓÙ·È fiϘ ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ™ÙÔ Î›ÌÂÓÔ ÔÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ ·Ó·Ê¤ÚÔÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Û ·Ú¤ÓıÂÛË. OÈ ‚È‚ÏÈÔÁÚ·ÊÈΤ˜ ·Ú·ÔÌ¤˜ ‰ÂÓ Ú¤ÂÈ Ó· ˘ÂÚ‚·›ÓÔ˘Ó ÙȘ 70 ÛÙȘ ·Ó·ÛÎÔ‹ÛÂȘ, ÙȘ 30 ÛÙȘ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜, ÙȘ 12 ÛÙ· Â›Î·ÈÚ· ı¤Ì·Ù· Î·È ÙȘ ÂӉȷʤÚÔ˘Û˜ ÂÚÈÙÒÛÂȘ, ÙȘ 5 ÛÙȘ ‚Ú·¯Â›Â˜ ‰ËÌÔÛȇÛÂȘ. H Û‡ÓÙ·ÍË ÙˆÓ ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ Á›ÓÂÙ·È Û‡Ìʈӷ Ì ÙȘ ÚԉȷÁڷʤ˜ Ù˘ International Committee of Medical Journal Editors / Uniform Requirements for Manuscripts Submitted to Biomedical Journals (JAMA 1997;277:927-934), http://jama.amaassn.org/info/auinst_req.html. OÈ Û˘ÓÙÌ‹ÛÂȘ ÙˆÓ Ù›ÙÏˆÓ ÙˆÓ ÂÚÈÔ‰ÈÎÒÓ Á›ÓÔÓÙ·È Ì ‚¿ÛË ÙÔ Cumulated Index Medicus (List of Journals Indexed in Index Medicus, http://www.nlm.nih.gov). ¶·Ú·‰Â›ÁÌ·Ù· ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ú·ÔÌÒÓ I. ¶ÂÚÈÔ‰Èο AÓ ÔÈ Û˘ÁÁÚ·Ê›˜ Â›Ó·È ¤ˆ˜ ¤ÍÈ ·Ó·ÁÚ¿ÊÔÓÙ·È fiÏÔÈ, ·Ó Â›Ó·È ÂÙ¿ ‹ ÂÚÈÛÛfiÙÂÚÔÈ ·Ó·ÁÚ¿ÊÔÓÙ·È ÔÈ ÚÒÙÔÈ ¤ÍÈ Î·È ÚÔÛÙ›ıÂÙ·È “et al” ‹ “Î·È Û˘Ó”. T·ÎÙÈ΋ ¤Î‰ÔÛË ÂÚÈÔ‰ÈÎÔ‡: ∞ÏÂ͛Ԣ ¢, ª¿Ú· Ã, °È·ÓÓ·ÎÔÔ‡ÏÔ˘ Ã. ∂Ì‚fiÏÈ·, ·ÚfiÓ Î·È Ì¤ÏÏÔÓ: Â›‰Ú·ÛË ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ÙˆÓ ÏÔÈ̈‰ÒÓ ÓÔÛËÌ¿ÙˆÓ Î·È ÙËÓ ·È‰È·ÙÚÈ΋ Ú¿ÍË.


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¶·È‰È·ÙÚÈ΋ 1996;59:272-279. Proesmans W. Bartter syndrome and its neonatal variant. Eur J Pediatr 1997;156:669-679. ™˘ÌÏËڈ̷ÙÈÎfi Ù‡¯Ô˜ ÂÚÈÔ‰ÈÎÔ‡: Flyvbjerg A. Role of growth hormone, insulin-like growth factors (IGFs) and IGF-binding proteins in the renal complications of diabetes. Kidney Int 1997;52 (60 Suppl):S12-S19. Èڛ˜ Û˘ÁÁڷʤ·: National Institutes of Health Consensus Development Conference. Neurofibromatosis conference statement. Arch Neurol 1988;45:575-578. ¶ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù‡Ô˘ ¿ÚıÚÔ˘: ∫Ô˘Ú›‰Ë˜ Ã, ∫Ô˘Ú›‰Ë˜ °. ¶·¯˘Û·ÚΛ· Î·È ˘ÂÚÏÈȉ·ÈÌ›· Û ·È‰È¿ ‚ÔÚÂÈÔ‰˘ÙÈ΋˜ ∫‡ÚÔ˘ [¶ÂÚ›ÏË„Ë]. 36Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ; 1998 5-7 πÔ˘Ó›Ô˘; ¶¿ÊÔ˜, ∫‡ÚÔ˜;1998. ÛÂÏ. 172. Schreiner GF, Lange L. Ethanol modulation of macrophage influx in glomerulonephritis [abstract]. J Am Soc Nephrol 1991;2:562. Should antileukotriene therapies be used instead of inhaled corticosteroids in asthma? [Editorial]. Am J Respir Crit Care Med 1998;158:1697-1701. Laux-End R, Inaebnit D, Gerber HA, Bianchetti MG. Vasculitis associated with levamisole and circulating autoantibodies [letter]. Arch Dis Child 1996;75:355-356. II. µÈ‚Ï›· ∫ÂÊ¿Ï·ÈÔ Û ‚È‚Ï›Ô: Clark AG, Barratt TM. Steroid-responsive nephrotic syndrome. In: Barratt TM, Arner ED, Harmon WE, editors. Pediatric Nephrology. 4th ed. Baltimore: Lippincott William Wilkins; 1999. p. 742. ™‡ÁÁÚ·ÌÌ· ‹ ÌÔÓÔÁÚ·Ê›·: Gorlin RJ, Cohen MM, Levin LS. Syndromes of the head and neck. 3rd ed. New York: Oxford University Press; 1990. ¢ËÌÔÛ›Â˘ÛË Û ÙfiÌÔ Ú·ÎÙÈÎÒÓ: Bauer AW. The two definitions of bacterial resistance. In: Smith AJ, Rogers CA, eds. Proceedings of the Third International Congress of Chemotherapy; 1962 May 29-31; New York: International Society of Chemotherapy; 1963. p. 484-500. ¢È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹: ¡ÙÔ˘ÓÙÔ˘Ï¿Î˘ π. ∏ ¯ÂÈÚÔ˘ÚÁÈ΋ ıÂÚ·›· ÙÔ˘ Û˘ÁÁÂÓÔ‡˜ ÂÍ·ÚıÚ‹Ì·ÙÔ˜ ÙÔ˘ ÈÛ¯›Ô˘ Û ·È‰È¿ οو ÙÔ˘ ÂÓfi˜ ¤ÙÔ˘˜ [‰È‰·ÎÙÔÚÈ΋ ‰È·ÙÚÈ‚‹]. ∞ı‹Ó·: ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ; 1979.

Kaplan SJ. Post hospital home health care: the elderly’s access and utilization [dissertation]. St. Louis (Mo): Washington Univ.; 1995. ¶›Ó·Î˜ Î·È ÂÈÎfiÓ˜ AÔÛÙ¤ÏÏÔÓÙ·È ÂȘ ÙÚÈÏÔ‡Ó (¤Ó· ÚˆÙfiÙ˘Ô+‰‡Ô ʈÙÔ·ÓÙ›ÁÚ·Ê·). ¶Ú¤ÂÈ Ó· ¤¯Ô˘Ó Ï¿ÙÔ˜ ›ÛÔ Ì ÙÔ Ï¿ÙÔ˜ ÙÔ˘ ÌÔÓfiÛÙËÏÔ˘ (8 cm) ‹ Ì ÙÔ Ï¿ÙÔ˜ fiÏ˘ Ù˘ ÛÂÏ›‰·˜ (16,8 cm). TÔ Ì¤ÁÈÛÙÔ Ì‹ÎÔ˜ ÙÔ˘˜, Ì·˙› Ì ÙÔ˘˜ Ù›ÙÏÔ˘˜, ‰ÂÓ Ú¤ÂÈ Ó· Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ ·fi 22 cm. OÈ ›Ó·Î˜ ·ÚÈıÌÔ‡ÓÙ·È Ì ·Ú·‚ÈÎÔ‡˜ ·ÚÈıÌÔ‡˜ Ì ÙË ÛÂÈÚ¿ Ô˘ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ Î›ÌÂÓÔ. ¶ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ‚Ú·¯‡ Ù›ÙÏÔ Î·È ÂÂÍ‹ÁËÛË ÙˆÓ Û˘ÓÙÌ‹ÛÂˆÓ ÛÙÔ Î¿Ùˆ ̤ÚÔ˜. ™ÙÔ˘˜ ›Ó·Î˜ Ú¤ÂÈ Ó· ·ÔʇÁÔÓÙ·È ÔÈ Î¿ıÂÙ˜ ÁÚ·Ì̤˜. ŸÏÔ ÙÔ ·ÂÈÎÔÓÈÛÙÈÎfi ˘ÏÈÎfi (Û¯‹Ì·Ù·, ‰È·ÁÚ¿ÌÌ·Ù·, ʈÙÔÁڷʛ˜, ÎÏ.) ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ÂÈÎfiÓ˜. £· Ú¤ÂÈ Ó· Â›Ó·È ¿ÚÈÛÙ˘ ÔÈfiÙËÙ·˜ Ì ÌÔÚÊ‹ ʈÙÔÁÚ·ÊÈÒÓ ‹ Î·È ÚˆÙÔÙ‡ˆÓ. ™ÙÔ ›Ûˆ ̤ÚÔ˜ ÙˆÓ ÂÈÎfiÓˆÓ Ú¤ÂÈ Ó· ÛËÌÂÈÒÓÂÙ·È Ì ÌÔχ‚È Ô ·ÚÈıÌfi˜ Ù˘ ÂÈÎfiÓ·˜ Î·È ÙÔ fiÓÔÌ· ÙÔ˘ ÚÒÙÔ˘ Û˘ÁÁڷʤ·, ηıÒ˜ Î·È ¤Ó· ‚¤ÏÔ˜ ÙÔ ÔÔ›Ô Ó· ‰Â›¯ÓÂÈ ÙÔ ¿Óˆ ̤ÚÔ˜ Ù˘ ÂÈÎfiÓ·˜. ™ÙȘ ʈÙÔÁڷʛ˜ ·ÛıÂÓÒÓ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·ÁÓˆÚ›˙ÂÙ·È Ë Ù·˘ÙfiÙËÙ¿ ÙÔ˘˜. OÈ ·ÛıÂÓ›˜ ‰ÂÓ Ú¤ÂÈ Ó· ·Ó·Ê¤ÚÔÓÙ·È ÔÓÔÌ·ÛÙÈο. ¶ÚÈÓ ·ÔÛÙ›ÏÂÙ ÙËÓ ÂÚÁ·Û›· Û·˜ ‚‚·Èˆı›Ù fiÙÈ ÂÚÈÏ·Ì‚¿ÓÂÈ: - ÂÈÛÙÔÏ‹ ˘Ô‚ÔÏ‹˜ - ‰‹ÏˆÛË ÌË ÚÔÁÂÓ¤ÛÙÂÚ˘ ‰ËÌÔÛ›Â˘Û˘ Ù˘ ÂÚÁ·Û›·˜ - 3 ·ÓÙ›ÁÚ·Ê· ÙÔ˘ ÎÂÈ̤ÓÔ˘ Ù˘ ÂÚÁ·Û›·˜ - ÛÂÏ›‰· Ù›ÙÏÔ˘ (¯ˆÚÈÛÙ‹ ÛÂÏ›‰·): ÂÚÈÏ·Ì‚¿ÓÂÈ - Ù›ÙÏÔ Î·È ‚Ú·¯‡ Ù›ÙÏÔ ÂÚÁ·Û›·˜ - fiÓÔÌ· Î·È ÂÒÓ˘ÌÔ Û˘ÁÁڷʤˆÓ (ÔÏÔÁڿʈ˜), ÂÈÛÙËÌÔÓÈÎfi/¿ ΤÓÙÚÔ/· fiÔ˘ ¤ÁÈÓÂ Ë ÂÚÁ·Û›· - fiÓÔÌ·, ‰È‡ı˘ÓÛË Î·È ÙËϤʈÓÔ Û˘ÁÁڷʤ· ÁÈ· ·ÏÏËÏÔÁÚ·Ê›· - ÂÚ›ÏË„Ë ÂÏÏËÓÈ΋-·ÁÁÏÈ΋, ϤÍÂȘ ÎÏÂȉȿ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) - ηٿÏÔÁÔ Û˘ÓÙÔÌÔÁÚ·ÊÈÒÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) - ΛÌÂÓÔ (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) - ¢¯·ÚÈÛٛ˜ ‹ ·Ó·ÁÓˆÚ›ÛÂȘ (·Ó·ÊÔÚ¿ Û ÂȉÔÙ‹ÛÂȘ-¯ÔÚËÁ›Â˜ ‹ ¿ÏϘ ËÁ¤˜ ˘ÔÛÙ‹ÚÈ͢) - ‚È‚ÏÈÔÁÚ·Ê›· (‰ÈÏfi ‰È¿ÛÙËÌ· - ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) - ›Ó·Î˜ (Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó - ÂÈÎfiÓ˜, Ì ‚¤ÏÔ˜ ÛÙÔ ›Ûˆ ̤ÚÔ˜ Ó· ‰Â›¯ÓÂÈ ÚÔ˜ Ù· Â¿Óˆ, ·ÚÈıÌË̤Ó˜, ÂȘ ÙÚÈÏÔ‡Ó - Ù›ÙÏÔÈ ÂÈÎfiÓˆÓ (‰ÈÏfi ‰È¿ÛÙËÌ· - Ô Î·ı¤Ó·˜ Û ¯ˆÚÈÛÙ‹ ÛÂÏ›‰·) ÂȘ ÙÚÈÏÔ‡Ó.


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 1

¶·È‰È·ÙÚÈ΋ 2001;64:1-3

Aƒ£ƒ√ ™À¡∆∞•∏™

Paediatriki 2001;64:1-3

EDITORIAL

¶·Ú¿ÁÔÓÙ˜ Ô˘ ÂȉÚÔ‡Ó ÛÙËÓ ÂÓ‰ÔÌ‹ÙÚÈ· ·Ó¿Ù˘ÍË ÙˆÓ Ó¢ÌfiÓˆÓ £. ∆ÛÈÏÈÁÈ¿ÓÓ˘

Factors affecting intrauterine lung growth T. Tsiligiannis

Abstract: The mature lung is a complex organ, comprised of more than 60 different cell types, of a total expansion area of 80 m2 and thickness of 0.2 Ìm. The development and growth of the lung is a remarkable blend of genetic, biochemical, hormonal and physical factors, many of which are still under investigation. Aberrations in development in the

various stages of this process lead to congenital malformations. A better knowledge of lung growth regulation has already led to new treatments, especially to reverse lung hypoplasia.

∏ ηٷ‚ÔÏ‹ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÂÌÊ·Ó›˙ÂÙ·È ÂÚ›Ô˘ 24 Ë̤Ú˜ ÌÂÙ¿ ÙË ÁÔÓÈÌÔÔ›ËÛË, ˆ˜ ÚÔÛÂ΂ÔÏ‹ ·fi ÙÔ ÎÔÈÏÈ·Îfi ÙÔ›¯ˆÌ· ÙÔ˘ ÚfiÛıÈÔ˘ ÂÓÙ¤ÚÔ˘. ™ÙË Û˘Ó¤¯ÂÈ· ÔÈ Ó‡ÌÔÓ˜ ÚÔԉ¢ÙÈο ÂÍÂÏ›ÛÛÔÓÙ·È Û ¤Ó· ÔχÏÔÎÔ fiÚÁ·ÓÔ Ô˘ ·ÔÙÂÏÂ›Ù·È ·fi 60 ÙÔ˘Ï¿¯ÈÛÙÔÓ ‰È·ÊÔÚÂÙÈο ·ÙÙ·Ú· Î·È ¤¯ÂÈ Û·Ó ÛÎÔfi ÙËÓ ·ÓÙ·ÏÏ·Á‹ ÙˆÓ ·ÂÚ›ˆÓ, Ì ÂÈÊ¿ÓÂÈ· Û˘ÓÔÏÈ΋˜ ¤ÎÙ˘Í˘ ÂÚ›Ô˘ 80 m2 Î·È ¿¯Ô˜ 0,2 Ìm (1). ∆¤ÛÛÂÚ· Â›‰· ¤¯Ô˘Ó ηıÔÚÈÛÙ› ÛÙËÓ ‰È·‰Èηۛ· ·Ó¿Ù˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ. ∏ ÂÌ‚Ú˘ÔÁ¤ÓÂÛË, ˘Ô‰ËÏÒÓÂÈ ÙËÓ ÂÌÊ¿ÓÈÛË ÙÔ˘ ·Ú¯¤ÁÔÓÔ˘ ÔÚÁ¿ÓÔ˘. ∏ ÌÔÚÊÔÁ¤ÓÂÛË, Â›Ó·È Ë ‰È·ÌfiÚʈÛË ÙÔ˘ Û¯‹Ì·ÙÔ˜ ÙˆÓ Ó¢ÌfiÓˆÓ Â͈ÙÂÚÈο ·ÏÏ¿ Î·È ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Î·È ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÌÔÓ¿‰ˆÓ, Ì ÙËÓ ÔÏ˘ÏÔÎfiÙËÙ· Î·È ÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ ÈÛÙÒÓ. ∏ ‰È·ÊÔÚÔÔ›ËÛË Â›Ó·È Ë ·Ó¿Ù˘ÍË ÂÍÂȉÈÎÂ˘Ì¤ÓˆÓ Î˘ÙÙ¿ÚˆÓ ·fi Ù· Úfi‰ÚÔÌ· ·ÙÙ·Ú·, fiˆ˜ Ù· Ó¢ÌÔÓÔ·ÙÙ·Ú· Ù‡Ô˘ ππ. ∏ ·‡ÍËÛË, Â›Ó·È Ë ‰ÈÂÚÁ·Û›· Ù˘ ÌÂÁ¤ı˘ÓÛ˘ ÙÔ˘ fiÁÎÔ˘ Î·È Ù˘ ÈÛÙÈ΋˜ Ì¿˙·˜ ÙˆÓ Ó¢ÌfiÓˆÓ Ì¤Ûˆ ˘ÂÚÏ·Û›·˜ Î·È ˘ÂÚÙÚÔÊ›·˜ (2). ªÂÙ¿ ÙËÓ ·Ú¯È΋ ÂÌ‚Ú˘˚΋ ÂÚ›Ô‰Ô ÙˆÓ ÚÒÙˆÓ Â‚‰ÔÌ¿‰ˆÓ Ô˘ ·ÎÔÏÔ˘ıÔ‡Ó ÙË ÁÔÓÈÌÔÔ›ËÛË, Ë ·Ó¿Ù˘ÍË ÙˆÓ Ó¢ÌfiÓˆÓ ÂÍÂÏ›ÛÛÂÙ·È ÛÂ

Ù¤ÛÛÂÚ· ¯ÚÔÓÈο ÛÙ¿‰È· ‹ ÂÚÈfi‰Ô˘˜ Ô˘ ¯ÚÔÓÈο ·ÏÏËÏÂÈηχÙÔÓÙ·È (3).

¶·È‰È·ÙÚÈÎfi ∆Ì‹Ì· - ¡·˘ÙÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ∞ıËÓÒÓ

Key words: lung development, growth factors.

1. ∏ 梉Է‰ÂÓÈ΋ ÂÚ›Ô‰Ô˜ (Pseudogladular phase) ™ÙÔ ÛÙ¿‰ÈÔ ·˘Ùfi Ô˘ ‰È·ÚΛ ·fi ÙËÓ 5Ë Ì¤¯ÚÈ ÙË 17Ë Â‚‰ÔÌ¿‰· Ù˘ ·ËÛ˘, ·Ó·Ù‡ÛÛÂÙ·È ÙÔ ÚˆÙfiÁÔÓÔ ·Ó·Ó¢ÛÙÈÎfi Û‡ÛÙËÌ· Î·È ‰È·ÊÔÚÔÔÈÂ›Ù·È Û ‚ÚfiÁ¯Ô˘˜ Î·È ‚ÚÔÁ¯ÈfiÏÈ· Ì ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë ·ÁÁ›ˆÛË, ̤¯ÚÈ ÙÔ ÚÔÏԂȉȷÎfi Â›‰Ô. 2. ∏ ÂÚ›Ô‰Ô˜ ÙˆÓ ¶fiÚˆÓ (Canalicular phase) ∫·Ù¿ ÙËÓ ÂÚ›Ô‰Ô ·˘Ù‹ Ô˘ ‰È·ÚΛ ·fi ÙË 16Ë Ì¤¯ÚÈ ÙËÓ 26Ë Â‚‰ÔÌ¿‰· Ù˘ ·ËÛ˘, ·˘Í¿ÓÂÈ Û ÛËÌ·ÓÙÈÎfi ‚·ıÌfi Ë ·ÁÁ›ˆÛË ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ ÚÔ˜ ÙËÓ ÂÚÈʤÚÂÈ· Î·È Ù· ÙÚȯÔÂȉ‹ Û˘ÌÏËÛÈ¿˙Ô˘Ó Ì ÙÔ ÂÈÊ·ÓÂÈ·Îfi ÂÈı‹ÏÈÔ ÙˆÓ ·Ó·Ù˘ÛÛfiÌÂÓˆÓ ·ÂÚ·ÁˆÁÒÓ. √ ·˘Ïfi˜ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ ·˘Í¿ÓÂÈ Û ̤ÁÂıÔ˜ Î·È ÙÔ ÙÔ›¯ˆÌ¿ ÙÔ˘˜ ÏÂÙ·›ÓÂÈ, ηıÒ˜ ÂÏ·ÙÙÒÓÂÙ·È ÙÔ ÌÂÛ¤Á¯˘Ì·. 3. ∏ ÂÚ›Ô‰Ô˜ ÙˆÓ ∆ÂÏÈÎÒÓ ™¿ÎÎˆÓ (Terminal sac or Saccular phase) ∏ ÂÚ›Ô‰Ô˜ ·˘Ù‹ Ô˘ ÂÎÙ›ÓÂÙ·È ·fi ÙËÓ 24Ë Ì¤¯ÚÈ ÙËÓ 36Ë Â‚‰ÔÌ¿‰· Î·È Û˘Ó¯›˙ÂÈ Ì¤¯ÚÈ ÙÔ Ù¤ÏÔ˜ Ù˘ ·ËÛ˘, ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ÂÈϤÔÓ Division of Paediatrics - Naval Hospital of Athens

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2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 2

¶·È‰È·ÙÚÈ΋ 2001;64:1-3

·Ó¿Ù˘ÍË ·Ó·Ó¢ÛÙÈÎÒÓ ·ÂÚ·ÁˆÁÒÓ, ÙËÓ ·‡ÍËÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ‚ÚÔÁ¯ÈÔÏ›ˆÓ Î·È ÙˆÓ ÌÂÏÏÔÓÙÈÎÒÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÌÔÓ¿‰ˆÓ, ‰ËÏ·‰‹ ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÏԂȉ›ˆÓ (acini). 4. ∏ ∫˘„ÂÏȉÈ΋ ÂÚ›Ô‰Ô˜ (Alveolar phase) ∏ ÂÚ›Ô‰Ô˜ ·˘Ù‹ Ô˘ ÂÎÙ›ÓÂÙ·È ·fi ÙËÓ 36Ë Â‚‰ÔÌ¿‰· ̤¯ÚÈ ÙÔ Ù¤ÏÔ˜ Ù˘ ·ËÛ˘ Î·È Û˘Ó¯›˙ÂÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ Ì¤¯ÚÈ Ù· ÚÒÙ· 3 ¯ÚfiÓÈ· Ù˘ ˙ˆ‹˜, ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ·Ó¿Ù˘ÍË Î·È ÙËÓ ·‡ÍËÛË ÙÔ˘ ·ÚÈıÌÔ‡ Î·È ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ Î˘„ÂÏ›‰ˆÓ. ∆Ô 85% ÙˆÓ Î˘„ÂÏ›‰ˆÓ ·Ó·Ù‡ÛÛÂÙ·È Ì ÔÏÏ·Ï·ÛÈ·ÛÌfi ÌÂÙ¿ ÙË Á¤ÓÓËÛË, ÂÓÒ ·ÚÁfiÙÂÚ· Ë ·‡ÍËÛË ÙÔ˘ fiÁÎÔ˘ ÙˆÓ Ó¢ÌfiÓˆÓ ÂÈÙ˘Á¯¿ÓÂÙ·È Î˘Ú›ˆ˜ Ì ÙËÓ ·‡ÍËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ Î˘„ÂÏ›‰ˆÓ (4). ∏ ‰È·‰Èηۛ· Ù˘ ·Ó¿Ù˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ Â›Ó·È È‰È·›ÙÂÚ· ÔχÏÔÎË Î·È Î·ıÔÚ›˙ÂÙ·È ·fi ÌÈ· ÔÈÎÈÏ›· ·Ú·ÁfiÓÙˆÓ Ô˘ Â›Ó·È ÁÂÓÂÙÈÎÔ›, ‚ÈÔ¯ËÌÈÎÔ›, ÔÚÌÔÓÈÎÔ› Î·È Ì˯·ÓÈÎÔ›. √È ÁÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ¤¯Ô˘Ó ÌÂÏÂÙËı› Û ÂÈÚ·Ì·Ùfi˙ˆ· ‹ Û ηÏÏȤÚÁÂȘ ÂÌ‚Ú˘˚ÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È ·ÊÔÚÔ‡Ó Ù· ÁÔÓ›‰È·, Ù· ÔÔ›· Û˘ÓÙÔÓ›˙ÔÓÙ·È Î·È ÂÓÂÚÁÔÔÈÔ‡ÓÙ·È ÛÙÔÓ "ηٿÏÏËÏÔ ÙfiÔ Î·È ¯ÚfiÓÔ" Î·È Î·ıÔÚ›˙Ô˘Ó ÙȘ ‰È·‰Èηۛ˜ Ù˘ ·Ó¿Ù˘Í˘ Î·È ‰È·ÊÔÚÔÔ›ËÛ˘ ÙˆÓ Î˘ÙÙ¿ÚˆÓ. ∏ Â›‰Ú·ÛË ÁÂÓÂÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ‰È·Ê·›ÓÂÙ·È ·fi ÙËÓ ·ÚÔ˘Û›· ÔÈÎÔÁÂÓÒÓ ÔÌÔÈÔÙ‹ÙˆÓ ÛÙÔÓ ÙÚfiÔ Ù˘ ·ÚÙËÚȷ΋˜ ‰È·ÎÏ¿‰ˆÛ˘ (1). √È ‚ÈÔ¯ËÌÈÎÔ› ‹ Ú˘ıÌÈÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ (regulatory factors) Â›Ó·È Ì›· ÌÂÁ¿ÏË ÔÌ¿‰· ÂÙȉ›ˆÓ Ù· ÔÔ›· ÂÎχÔÓÙ·È ·fi Ù· ÂÈıËÏȷο ·ÙÙ·Ú· Î·È Ù· ·ÙÙ·Ú· ÙÔ˘ ÌÂÛÂÁ¯‡Ì·ÙÔ˜ Î·È Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ¤Ó·ÚÍË Ù˘ ÌÔÚÊÔÁ¤ÓÂÛ˘ ÙˆÓ Ó¢ÌfiÓˆÓ, fiˆ˜ Ô TTF-1 (thyroid transcription factor-1), ÙÔ Û·Ê‹ ‰È·¯ˆÚÈÛÌfi Î·È ÙËÓ ·Û˘ÌÌÂÙÚ›· ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ Î·È ÙÔ˘ ‰ÂÍÈÔ‡ Ó‡ÌÔÓ· lrd (leftright dyenin), TGF-‚ (transforming growth factor –‚), ÙÔÓ Î·ıÔÚÈÛÌfi ÙÔ˘ ÙÚfiÔ˘ ‰È·ÎÏ¿‰ˆÛ˘ ÙÔ˘ ‚ÚÔÁ¯ÈÎÔ‡ ‰¤Ó‰ÚÔ˘ Î·È ÙÔ˘ ‰È·¯ˆÚÈÛÌÔ‡ ÙÔ˘ ·fi ÙÔÓ ÔÈÛÔÊ¿ÁÔ, FGF (fibroblast growth factor pathway), ÙÔÓ ·ÚÌÔÓÈÎfi Û˘Ó‰˘·ÛÌfi Ù˘ ‰È·ÎÏ¿‰ˆÛ˘ ÙÔ˘ ‚ÚÔÁ¯ÈÎÔ‡ ‰¤Ó‰ÚÔ˘ Ì ÙËÓ ·ÁÁ›ˆÛË ÙˆÓ Ó¢ÌfiÓˆÓ Î·È ÙËÓ Î˘„ÂÏȉÔÁ¤ÓÂÛË Î·È ÙËÓ ÙÂÏÈ΋ ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ (5). º˘ÛÈÎÔ› ‹ Ì˯·ÓÈÎÔ› ·Ú¿ÁÔÓÙ˜, Â›Û˘, ÂÓ¤¯ÔÓÙ·È ÛÙË ‰È·‰Èηۛ· Ù˘ ·Ó¿Ù˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ. √ ÂÚÈÔÚÈÛÌfi˜ ÙÔ˘ ··Ú·›ÙËÙÔ˘ ‰È·ı¤ÛÈÌÔ˘ ¯ÒÚÔ˘ Û ÚÒÈÌ· ÛÙ¿‰È· Ù˘ ·ËÛ˘, ·fi ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ ÙÔȯÒÌ·ÙÔ˜, ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, Û˘ÁÁÂÓ‹ ˘‰ÚÔıÒڷη ‹ ‚·ÚÈ¿ ˘‰ÚÔÓ¤ÊÚˆÛË, Ô‰ËÁ› Û Ì›ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Î·È Û ·Ó¿ÏÔÁÔ˘ ‚·ıÌÔ‡ Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· (6).

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∆Ô ÂÌ‚Ú˘˚Îfi ˘ÁÚfi ÙˆÓ Ó¢ÌfiÓˆÓ Ô˘ ·Ú¿ÁÂÙ·È ·fi Ù· ÂÈıËÏȷο ·ÙÙ·Ú· Â›Ó·È ˙ˆÙÈ΋˜ ÛËÌ·Û›·˜ ÛÙË Ê˘ÛÈÔÏÔÁÈ΋ ·Ó¿Ù˘ÍË. ∏ ·ÚÔ¯¤Ù¢ÛË ÙÔ˘ ˘ÁÚÔ‡ ›Ù ÂÈÚ·Ì·ÙÈο ›ÙÂ Û·Ó ·ÔÙ¤ÏÂÛÌ· ÚfiˆÚ˘ ڋ͢ ÙˆÓ ÌÂÌ‚Ú·ÓÒÓ (7), Ô‰ËÁ› Û ˘ÔÏ·ÛÙÈÎÔ‡˜ Ó‡ÌÔÓ˜, ÂÓÒ Ë ÂÈÚ·Ì·ÙÈ΋ ·ÔϛӈÛË Ù˘ ÙÚ·¯Â›·˜ Û ÂÈÚ·Ì·Ùfi˙ˆ· ‹ Û·Ó Â·ÎfiÏÔ˘ıÔ ·ÙÚËÛ›·˜ ÙÔ˘ Ï¿Ú˘ÁÁ·, Ô‰ËÁ› Û ·˘ÍË̤ÓÔ Ì¤ÁÂıÔ˜ Ó¢ÌfiÓˆÓ (8,9). ∞Ó¿ÏÔÁ·, Ë Ì›ˆÛË Ù˘ ÔÛfiÙËÙ·˜ ÙÔ˘ ·ÌÓÈ·ÎÔ‡ ˘ÁÚÔ‡ Û·Ó ·ÔÙ¤ÏÂÛÌ· ÔÏÈÁÔ¸‰Ú¿ÌÓÈÔ˘, Ô‰ËÁ› Û Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· Û˘ÓÂ›· ÂÏÏÈÔ‡˜ ‰È·ÎÏ¿‰ˆÛ˘ ÙÔ˘ ‚ÚÔÁ¯ÈÎÔ‡ ‰¤Ó‰ÚÔ˘ ‹ Ì›ˆÛ˘ ÙÔ˘ ·ÚÈıÌÔ‡ ‹ Î·È ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ Î˘„ÂÏ›‰ˆÓ Î·È Â›Ó·È ‚·Ú‡ÙÂÚË, fiÙ·Ó ÙÔ ÔÏÈÁÔ¸‰Ú¿ÌÓÈÔ ÂÌÊ·Ó›˙ÂÙ·È ÂÓˆÚ›˜ ηٿ ÙËÓ Î‡ËÛË ‹ ·Ú·Ì¤ÓÂÈ ÁÈ· ÌÂÁ¿ÏÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·. √È ÂÌ‚Ú˘˚Τ˜ ÎÈÓ‹ÛÂȘ ·Ó·ÓÔ‹˜ Û·Ó ·Ú¿ÁÔÓÙ˜ ¤ÎÙ·Û˘ ÙÔ˘ Ó¢ÌÔÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜, Ê·›ÓÂÙ·È fiÙÈ Â›Û˘ ÚÔ¿ÁÔ˘Ó ÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ Ó¢ÌfiÓˆÓ Î·È ÂËÚ¿˙Ô˘Ó Ùo ÙÂÏÈÎfi ‚¿ÚÔ˜ Î·È ÙÔÓ fiÁÎÔ ÙÔ˘˜ ηıÒ˜ Î·È ÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ Î˘„ÂÏ›‰ˆÓ (10). ŒÓ·˜ ÛËÌ·ÓÙÈÎfi˜ ·ÚÈıÌfi˜ ÔÚÌÔÓÒÓ, fiˆ˜ ÔÈ ÔÚÌfiÓ˜ Ù˘ ˘ÔʇÛˆ˜, ÙÔ˘ ı˘ÚÂÔÂȉԇ˜ Î·È ÙˆÓ ÂÈÓÂÊÚȉ›ˆÓ ÂÓ¤¯ÔÓÙ·È ÛÙË ‰È·‰Èηۛ· ·Ó¿Ù˘Í˘ Î·È ˆÚ›Ì·ÓÛ˘ ÙˆÓ Ó¢ÌfiÓˆÓ. π‰È·›ÙÂÚÔ˜ Â›Ó·È Ô ÚfiÏÔ˜ ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙÔ ¯ÚfiÓÔ ÂÌÊ¿ÓÈÛ˘ ÙˆÓ ÂÙ·ÏÏȈ‰ÒÓ ÛˆÌ·Ù›ˆÓ (lamellar bodies) ÛÙ· Ó¢ÌÔÓÔ·ÙÙ·Ú· Ù‡Ô˘ ππ Î·È ÛÙËÓ ¤Ó·ÚÍË Ù˘ ·˘ÍË̤Ó˘ ·Ú·ÁˆÁ‹˜ ‰Ú·ÛÙÈ΋˜ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈ΋˜ Ô˘Û›·˜ (surfactant) (11). √È ‰È¿ÊÔÚ˜ ·ÚÂÎÎÏ›ÛÂȘ Ô˘ ÌÔÚ› Ó· ÂÈÛ˘Ì‚Ô‡Ó Î·Ù¿ ÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ Ó¢ÌfiÓˆÓ ¤¯Ô˘Ó Û·Ó Â·ÎfiÏÔ˘ıÔ ·ÓÙ›ÛÙÔȯ˜ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜. ∆Ô ÛÙ¿‰ÈÔ Ù˘ ·Ó¿Ù˘Í˘ ηٿ ÙÔ ÔÔ›Ô ÂÈÛ˘Ì‚·›ÓÂÈ Ë ·Ú¤ÎÎÏÈÛË ·fi ÙËÓ ÔÌ·Ï‹ ‰È·‰Èηۛ·, ηıÔÚ›˙ÂÈ ÙÔ Ì¤ÁÂıÔ˜, ÙË ı¤ÛË Î·È ÙËÓ ÎÏÈÓÈ΋ ÛËÌ·Û›· Ù˘ ·ÓˆÌ·Ï›·˜. ∏ Ó¢ÌÔÓÈ΋ ·ÁÂÓÂÛ›· Î·È ·Ï·Û›· Â›Ó·È Â·ÎfiÏÔ˘ı· Ù˘ ·‡Û˘ Ù˘ ·Ó¿Ù˘Í˘ Û ÚÒÈÌ· ÛÙ¿‰È· Ù˘ ·ËÛ˘. √È ÎÂÓÙÚÈÎÔ‡ Ù‡Ô˘ ·ÛÙÂȘ ·Ó·Ù‡ÛÛÔÓÙ·È ÛÙ· ÚÒÈÌ· ÛÙ¿‰È·, ÂÓÒ ÔÈ ÂÚÈÊÂÚÈÎÔ‡ Ù‡Ô˘ ÛÙ· ÙÂÏÈο ÛÙ¿‰È·. √È ‚ÚÔÁ¯ÔÁÂÓ›˜ ·ÛÙÂȘ Û¯ËÌ·Ù›˙ÔÓÙ·È ·fi ·ÓÒÌ·ÏË ¤ÎÊ˘ÛË ‚ÚÔÁ¯ÈÎÔ‡ ÈÛÙÔ‡, ÂÓÒ ÔÈ Ó¢ÌÔÓÈΤ˜ ·ÛÙÂȘ ÂΉËÏÒÓÔÓÙ·È Î·Ù¿ ÙËÓ Î˘„ÂÏȉÔÔ›ËÛË Î·È Ë Î˘ÛÙÈ΋ ·‰Âӈ̷Ù҉˘ ‰˘ÛÏ·Û›· Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ˘ÂÚÏ·Û›·˜ ÙˆÓ ÙÂÏÈÎÒÓ ‚ÚÔÁ¯ÈÔÏ›ˆÓ (12). √È Û˘ÁÁÂÓ›˜ ·ÁÁÂȷΤ˜ ‹ Î·È Î·Ú‰È·Î¤˜ ·ÓˆÌ·Ï›Â˜ Ô˘ ¤¯Ô˘Ó Û·Ó ·ÔÙ¤ÏÂÛÌ· ÌÂȈ̤ÓË Ó¢ÌÔÓÈ΋ ·ÈÌ¿ÙˆÛË Û¯ÂÙ›˙ÔÓÙ·È Ì ÌÈÎÚfiÙÂÚÔ ·ÚÈıÌfi Î·È Ì¤ÁÂıÔ˜ ΢„ÂÏ›‰ˆÓ,


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fiˆ˜ Û˘Ì‚·›ÓÂÈ ÛÙËÓ ÂÙÂÚfiÏ¢ÚË ·ÁÂÓÂÛ›· Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜ (13). ∏ ÁÓÒÛË ÙˆÓ ÔÈÎ›ÏˆÓ ·Ú·ÁfiÓÙˆÓ Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ·Ó¿Ù˘ÍË Î·È ÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ Ó¢ÌfiÓˆÓ, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙȘ Û‡Á¯ÚÔÓ˜ ·ÂÈÎÔÓÈÛÙÈΤ˜ Î·È ‰È·ÁÓˆÛÙÈΤ˜ ÌÂıfi‰Ô˘˜ Î·È ÙËÓ ·Ó¿Ù˘ÍË Ù˘ ÂÌ‚Ú˘˚΋˜ ¯ÂÈÚÔ˘ÚÁÈ΋˜, ¤¯Ô˘Ó Ô‰ËÁ‹ÛÂÈ Û ÈÔ ¤ÁηÈÚË Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË Û ·ÚÎÂÙ¤˜ Ù¤ÙÔȘ ·ıÔÏÔÁÈΤ˜ ηٷÛÙ¿ÛÂȘ (14,15). µÈ‚ÏÈÔÁÚ·Ê›· 1. Jeffery P. The development of the large and small airways. Am J Respir Crit Care Med 1998;157:174-180. 2. Thurlbeck WM. Pre- and postnatal organ development. In: Chernick V, Mellins RB, eds. µasic Mechanisms of Pediatric Respiratory Disease: Cellular and Integrative. 1st ed. Philadelphia: Decker; 1991. p. 23-36. 3. Merkus PJ, ten Have-Opbroek AA, Quanjer PH. Human Lung Growth: A Review. Pediatr Pulmonol 1996;21:383397. 4. Thurlbeck WM. Quantitative anatomy of the lung. In: Thurlbeck WM, ed. Pathology of the lung. Stuttgart: Thieme; 1988. p. 51-55. 5. Warburton D, Lee MK. Current concepts on lung development. Curr Opin Pediatr 1999;11:188-192. 6. Reid LM. Lung growth in health and disease. Br J Dis Chest 1984;78:113-114. 7. Perlman M, Williams J, Hirsch M. Neonatal pulmonary hypoplasia after prolonged leakage of amniotic fluid. Arch Dis Child 1976;51:349-353.

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8. Alcorn D, Adamson TM, Lambert TF. Morphologic effects of tracheal ligation and drainage in the fetal lamb lung. J Anat 1977;123:649-660. 9. Wigglesworth JS, Hislop A, Desai R. Fetal lung growth in laryngeal atresia. Pediatr Pathol 1987;7:515-525. 10. Wigglesworth JS, Desai R. Is fetal respiratory function a major determinant of perinatal survival? Lancet 1982;1:264-267. 11. Difiore JW, Wilson JM. Lung Development. Semin Pediatr Surg 1994;3:221-232. 12. Kravitz RM. Congenital malformations of the lung. Pediatr Clin N Am 1994;41:453-473. 13. Haworth SG, McKenzie SA, Fitzpatrick ML. Alveolar development after ligation of left pulmonary artery in newborn pig: Clinical relevance to unilateral pulmonary artery. Thorax 1981;36:938-943. 14. Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998;179:884-889. 15. Hubbarb AM, Crombleholme TM. Anomalies and malformations affecting the fetal/neonatal chest. Semin Roentenol 1998;33:117-125.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 29-11-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 18-12-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: £. ∆ÛÈÏÈÁÈ¿ÓÓ˘ ¡·˘ÙÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ∞ıËÓÒÓ, ¶·È‰È·ÙÚÈÎfi ∆Ì‹Ì· ¢ÂÈÓÔÎÚ¿ÙÔ˘˜ 70 - ∞ı‹Ó· 115 21

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¶·È‰È·ÙÚÈ΋ 2001;64:4-13

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Paediatriki 2001;64:4-13

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√Í›· ‰È¿ÚÚÔÈ· ∫. ™È·Ê¿Î·˜1, π. ¶·Ó·ÁÈÒÙÔ˘-∞ÁÁÂÏ·ÎÔÔ‡ÏÔ˘2

Acute diarrhea C. Siafakas1, J. Panayiotou-Angelakopoulou2

¶ÂÚ›ÏË„Ë: ∏ ÔÍ›· ‰È¿ÚÚÔÈ· Â›Ó·È ¤Ó· ÎÔÈÓfi Úfi‚ÏËÌ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜. ∏ ηٷÓfiËÛË ÙˆÓ Ì˯·ÓÈÛÌÒÓ Ô˘ ˘ÂÈÛ¤Ú¯ÔÓÙ·È ÛÙËÓ ·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ‰È¿ÚÚÔÈ·˜ ÌÔÚ› Ó· ‚ÔËı‹ÛÂÈ ÙÔÓ ÁÈ·ÙÚfi ÛÙÔ Ó· ÚԂϤ„ÂÈ ÙËÓ ·ÈÙ›· Ù˘ ‰È¿ÚÚÔÈ·˜ ÛÙËÓ Î¿ı ÂÚ›ÙˆÛË. √È ÈÔ Û˘¯ÓÔ› ·ÈÙÈÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ Â›Ó·È ÏÔÈÌÒ‰ÂȘ (ÈÔ› ‹ ‚·ÎÙ‹ÚÈ·). ∏ ÎÏÈÓÈ΋ ÂÎÙ›ÌËÛË ÙÔ˘ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛ˘ Â›Ó·È ıÂÌÂÏÈÒ‰Ô˘˜ ÛËÌ·Û›·˜ ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ ÔÛÔ‡ ÙˆÓ ¯ÔÚËÁÔ‡ÌÂÓˆÓ ˘ÁÚÒÓ. ∏ ıÂÚ·›· Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ Ú¤ÂÈ Ó· ηÙ¢ı‡ÓÂÙ·È ÚÔ˜ ÙË ‰ÈfiÚıˆÛË Ù˘ ·Ê˘‰¿ÙˆÛ˘, ΢ڛˆ˜ Ì ÙË ¯ÔÚ‹ÁËÛË ‰È·Ï˘Ì¿ÙˆÓ ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· Ô˘ ÂÚȤ¯Ô˘Ó ÙȘ ηٿÏÏËϘ Û˘ÁÎÂÓÙÚÒÛÂȘ ˘‰·Ù·ÓıÚ¿ÎˆÓ Î·È ËÏÂÎÙÚÔÏ˘ÙÒÓ. ™‹ÌÂÚ· Û˘ÓÈÛÙ¿Ù·È Ë ¤ÁηÈÚË Â·Ó·Û›ÙÈÛË ¯ˆÚ›˜ Ó· ·Ó·ÁÓˆÚ›˙ÂÙ·È Ë ·Ó¿ÁÎË ÁÈ· ‰È·ÎÔ‹ Ù˘ Û›ÙÈÛ˘ ÛÙÔ ·Ú¯ÈÎfi ÛÙ¿‰ÈÔ, ·Ú·›ˆÛË ÙÔ˘ Á¿Ï·ÎÙÔ˜ ‹ ÂÍ¿ÏÂÈ„Ë Ù˘ Ï·ÎÙfi˙˘ ·fi ÙË ‰›·ÈÙ·. ∆· ÂÚÈÛÛfiÙÂÚ· ÂÂÈÛfi‰È· ‰È¿ÚÚÔÈ·˜ Â›Ó·È ·˘ÙÔÂÚÈÔÚÈ˙fiÌÂÓ· Î·È ‰ÂÓ ¯Ú‹˙Ô˘Ó ·ÓÙÈÌÈÎÚԂȷ΋˜ ıÂÚ·›·˜. ∆Ô Î˘ÚÈfiÙÂÚÔ ‚‹Ì· ÛÙÔÓ ¤ÏÂÁ¯Ô Ù˘ ‰È¿ÚÚÔÈ·˜ Â›Ó·È Ë ÚfiÏË„Ë, Ë ÔÔ›· ÌÔÚ› Ó· ÂÈÙ¢¯ı› Ì ÙËÓ Î·Ï‹ Ù‹ÚËÛË ÙˆÓ Î·ÓfiÓˆÓ ˘ÁÈÂÈÓ‹˜, ÙË ‚ÂÏÙ›ˆÛË ÙˆÓ ·Ô¯ÂÙ‡ÛˆÓ, ÙÔ ÛˆÛÙfi Ì·Á›ÚÂÌ· Î·È ÙËÓ Î·Ù¿„˘ÍË ÙˆÓ ÙÚÔÊÒÓ.

Abstract: Acute diarrhea is a common problem in children. Understanding the different pathophysiologic processes implicated in diarrhea can help the physician to predict the etiology of diarrhea in an individual patient. The most common etiologic agents in acute diarrhea are infectious (viruses or bacteria). The clinical evaluation of the degree of dehydration is of primary importance in the estimation of the amount of fluids needed to be administered. The treatment of acute diarrhea should be directed towards the correction of dehydration, principally with the use of oral rehydration solutions which contain appropriate concentrations of carbohydrates and electrolytes. Early refeeding is currently recommended without the need of discontinuing oral feeding in the initial stages, formula dilution or elimination of lactose from the diet. Most diarrheal episodes are self-limited and do not require antimicrobial therapy. The most important step in controlling diarrhea is prevention which can be accomplished by the maintenance of good personal hygiene, improved sanitation, adequate cooking and refrigeration of food.

§¤ÍÂȘ ÎÏÂȉȿ: ÔÍ›· ‰È¿ÚÚÔÈ·, ·Ê˘‰¿ÙˆÛË, ÂÓ˘‰¿ÙˆÛË.

Key words: rehydration.

1 ÷ÚÔÎfiÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ 2 ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ∆Ì‹Ì· ¶·È‰È·ÙÚÈ΋˜ °·ÛÙÚÂÓÙÂÚÔÏÔÁ›·˜ Î·È ¢È·ÙÚÔÊ‹˜

1 Charokopio University 2 ∞’ Pediatric Clinic of University of Athens, Division of Pediatric Gastroenterology and Nutrition

4

acute

diarrhea,

dehydration,


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 5

¶·È‰È·ÙÚÈ΋ 2001;64:4-13

Paediatriki 2001;64:4-13

∂ÈÛ·ÁˆÁ‹ ∏ ‰È¿ÚÚÔÈ· Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÔÚÈÛÙ›. √È ·ÛıÂÓ›˜ ‹ ÔÈ ÁÔÓ›˜ ¯ÚËÛÈÌÔÔÈÔ‡Ó ÙÔÓ fiÚÔ ·˘Ùfi fiÙ·Ó ·Ó·Ê¤ÚÔÓÙ·È ÛÂ Û˘¯Ó¤˜ ‹ ˘‰·Ú›˜ ÎÂÓÒÛÂȘ. ∂Âȉ‹ fï˜ ˘¿Ú¯ÂÈ ÌÂÁ¿ÏË ÔÈÎÈÏÔÌÔÚÊ›· fiÛÔÓ ·ÊÔÚ¿ ÙÔÓ ·ÚÈıÌfi, ÙÔÓ fiÁÎÔ Î·È ÙË Û‡ÛÙ·ÛË ÙˆÓ ÎÔÚ¿ÓˆÓ, fi¯È ÌfiÓÔ ÌÂٷ͇ ‰È·ÊÔÚÂÙÈÎÒÓ ·ÙfiÌˆÓ ·ÏÏ¿ Î·È ÙÔ˘ ›‰ÈÔ˘ ·ÙfiÌÔ˘ ÛÙȘ ‰È¿ÊÔÚ˜ Ê¿ÛÂȘ Ù˘ ˙ˆ‹˜ ÙÔ˘, ¤Ó·˜ ¯Ú‹ÛÈÌÔ˜ ÔÚÈÛÌfi˜ Â›Ó·È ·˘Ùfi˜ Ù˘ ·˘ÍË̤Ó˘ ·Ú·ÁˆÁ‹˜ ÎÔÚ¿ÓˆÓ Ù· ÔÔ›· Û˘Ó‹ıˆ˜ ¤¯Ô˘Ó ·˘ÍË̤ÓË ÂÚÈÂÎÙÈÎfiÙËÙ· ‡‰·ÙÔ˜. ŒÙÛÈ ÏÔÈfiÓ, Ë ‰È¿ÚÚÔÈ· ÔÚ›˙ÂÙ·È Û·Ó ‚¿ÚÔ˜ ÎÔÚ¿ÓˆÓ ÌÂÁ·Ï‡ÙÂÚÔ ÙˆÓ 10 g/kg/24 h ÁÈ· Ù· ‚Ú¤ÊË ‹ 200 g/24 h ÁÈ· Ù· ·È‰È¿ (1). ∏ ‰È¿ÚÚÔÈ· ‰È·ÎÚ›ÓÂÙ·È ·˘ı·›ÚÂÙ· Û ÔÍ›·, fiÙ·Ó Ë ‰È¿ÚÎÂÈ¿ Ù˘ ‰ÂÓ ˘ÂÚ‚·›ÓÂÈ ÙȘ 2 ‚‰ÔÌ¿‰Â˜ Î·È Û ¯ÚfiÓÈ·, fiÙ·Ó ÙȘ ˘ÂÚ‚·›ÓÂÈ. ∏ ÔÍ›· ‰È¿ÚÚÔÈ· Â›Ó·È ÌÈ· ·fi ÙȘ Û˘¯ÓfiÙÂÚ˜ ·Èٛ˜ ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÛÈÌfiÙËÙ·˜ ÛÙȘ ·Ó·Ù˘ÛÛfiÌÂÓ˜ ¯ÒÚ˜. ∂›Ó·È ˘‡ı˘ÓË ÁÈ· 4 ÂηÙÔÌ̇ÚÈ· ı·Ó¿ÙÔ˘˜ ·È‰ÈÒÓ, ËÏÈΛ·˜ ÌÈÎÚfiÙÂÚ˘ ÙˆÓ 5 ÂÙÒÓ, ÂÙËÛ›ˆ˜ (2). ™ÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜, Ë ÔÍ›· ‰È¿ÚÚÔÈ· ¢ı‡ÓÂÙ·È ÁÈ· ÙÔ 10% ÙˆÓ ÂÈÛ·ÁˆÁÒÓ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ·È‰ÈÒÓ ËÏÈΛ·˜ οو ÙˆÓ 5 ÂÙÒÓ (3). ∞ÈÙÈÔÏÔÁ›· √È ÏÔÈÌÒÍÂȘ ·ÔÙÂÏÔ‡Ó ÙËÓ Î‡ÚÈ· ·ÈÙ›· ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ÛÙ· ·È‰È¿. ∏ ÂÈÎÚ¿ÙËÛË ÙˆÓ ÈÒÓ Û·Ó ÙÔ Î‡ÚÈÔ ·ıÔÁfiÓÔ ·›ÙÈÔ Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜, Ì ÙË ‚ÂÏÙ›ˆÛË ÙˆÓ Û˘ÓıËÎÒÓ ˘ÁÈÂÈÓ‹˜ Î·È ·Ô¯¤Ù¢Û˘, ¤¯ÂÈ ÌÂÙ·ÙÔ›ÛÂÈ ÙËÓ Â›ÙˆÛË Ù˘ ÓfiÛÔ˘ ·fi ÙÔ Î·ÏÔη›ÚÈ ÚÔ˜ ÙÔ ¯ÂÈÌÒÓ·. ∂ȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ ÛÙËÓ ∂ÏÏ¿‰· ¤¯Ô˘Ó ·Ó·‰Â›ÍÂÈ ÙÔ ÚfiÙ·-Èfi Û·Ó ÙÔ ÈÔ Û˘¯Ófi ·›ÙÈÔ ÔÍ›·˜ Á·ÛÙÚÂÓÙÂÚ›Ùȉ·˜ ÛÙ· ·È‰È¿ ÌÂ Û˘¯ÓfiÙËÙ· Ô˘ Î˘Ì·›ÓÂÙ·È ·fi 19,6% (4) ¤ˆ˜ 26,6% (5). πÔ› ÚÔηÏÔ‡Ó 400 ı·Ó¿ÙÔ˘˜ ÙÔ ¯ÚfiÓÔ ·fi Á·ÛÙÚÂÓÙÂÚ›Ùȉ· ÛÙȘ ∏¶∞ (3). √ ›Ó·Î·˜ 1 ‰Â›¯ÓÂÈ ÙÔ˘˜ ÈÔ‡˜ Ô˘ ÚÔηÏÔ‡Ó ÔÍ›· ÂÓÙÂÚ›Ùȉ· ÛÙ· ·È‰È¿ ηıÒ˜ Î·È ÌÂÚÈο ·fi Ù·

ÈÔ ÎÔÈÓ¿ ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘˜. ªÂÙ·‰›‰ÔÓÙ·È Ì ÙËÓ ÂÓÙÂÚÔÛÙÔÌ·ÙÈ΋ Ô‰fi, ÂÓÒ ÁÈ· ÙÔ ÚfiÙ· Èfi ˘¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ fiÙÈ ÌÔÚ› Ó· ÌÂÙ·‰Ôı› Î·È Ì ÙËÓ ·Ó·Ó¢ÛÙÈ΋ Ô‰fi. ∆· Û˘ÌÙÒÌ·Ù· Ô˘ ÚÔηÏÔ‡Ó Â›Ó·È ÂÌÂÙfi˜, ‰È¿ÚÚÔÈ·, ÎÔÏÈÎÔÂȉ¤˜ ÎÔÈÏÈ·Îfi ¿ÏÁÔ˜ Î·È ˘ÚÂÙfi˜. ∂Âȉ‹ fï˜ ÔÈ ÈÔ› ·˘ÙÔ› ÚÔÛ‚¿ÏÏÔ˘Ó Ù· ·ÙÙ·Ú· ÙˆÓ Ï·¯ÓÒÓ ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘, Û ‚·ÚȤ˜ ηٷÛÙ¿ÛÂȘ ÚÔηÏÔ‡Ó ÙËÓ ÂÈ¤‰ˆÛ‹ ÙÔ˘˜ Ì ·ÔÙ¤ÏÂÛÌ· Ë ÔÍ›· ‰È¿ÚÚÔÈ· Ó· ÌÂÙ·¤ÛÂÈ Û ¯ÚfiÓÈ· Î·È Ó· ·Ú·Ù·ı› ̤¯ÚÈ Î·È ‰‡Ô Ì‹Ó˜ ¤ˆ˜ fiÙÔ˘ Ô ‚ÏÂÓÓÔÁfiÓÔ˜ ·ÔηٷÛÙ·ı› (ÌÂÙ·ÏÔÈÌ҉˘ ÂÓÙÂÚ›Ùȉ·). √È ‚·ÎÙËÚȉȷΤ˜ ÏÔÈÌÒÍÂȘ ÌÂÙ·‰›‰ÔÓÙ·È Ì ÙËÓ ÂÓÙÂÚÔÛÙÔÌ·ÙÈ΋ Ô‰fi ‹ Ì ÙË Ï‹„Ë ÌÔÏ˘Ṳ̂ÓÔ˘ ÓÂÚÔ‡ ‹ ÙÚÔÊ‹˜ (6,7). ™ÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜ Ù· ΢ÚÈfiÙÂÚ· ·›ÙÈ· Â›Ó·È Ë Û·ÏÌÔÓ¤ÏÏ·, Ë ÛÈÁΤÏÏ·, ÙÔ ÎÔÏÔ‚·ÎÙËÚ›‰ÈÔ, ÙÔ Î·Ì˘ÏÔ‚·ÎÙËÚ›‰ÈÔ jejuni, Ë ˘ÂÚÛ›ÓÈ·. √È ‚·ÎÙËÚȉȷΤ˜ ÂÓÙÂÚ›Ùȉ˜ ÂΉËÏÒÓÔÓÙ·È Â›Ù Ì ÙË ÌÔÚÊ‹ ÂȉËÌÈÒÓ Â›Ù Ì ÙË ÌÔÚÊ‹ ÛÔÚ·‰ÈÎÒÓ ÎÚÔ˘ÛÌ¿ÙˆÓ. ™ÙËÓ ·ÙÚ›‰· Ì·˜ ÔÈ Û·ÏÌÔÓ¤ÏϘ Î·È ÔÈ ÛÈÁΤÏϘ ·ÔÙÂÏÔ‡Ó ÙÔ Î‡ÚÈÔ ·›ÙÈÔ Ù˘ ‰È¿ÚÚÔÈ·˜ ÙÔ˘ ηÏÔηÈÚÈÔ‡, ÂÓÒ ÙÔ ÂÓÙÂÚÔ·ıÔÁfiÓÔ ÎÔÏÔ‚·ÎÙËÚ›‰ÈÔ (EPEC) Î·È ÙÔ Î·Ì˘ÏÔ‚·ÎÙËÚ›‰ÈÔ ‰ÂÓ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Âԯȷ΋ ηٷÓÔÌ‹ (4,8). ∆· Û˘ÌÙÒÌ·Ù· ÛÙȘ ‚·ÎÙËÚȉȷΤ˜ ÏÔÈÌÒÍÂȘ ¤¯Ô˘Ó ·fiÙÔÌË ¤Ó·ÚÍË Î·È Û˘Ó›ÛÙ·ÓÙ·È Û ˘ÚÂÙfi, ÎÔÈÏÈ·Îfi ¿ÏÁÔ˜, ‚ÏÂÓÓÔ·ÈÌ·ÙËÚ¤˜ ÎÂÓÒÛÂȘ Î·È ÙÂÈÓÂÛÌfi. √ ›Ó·Î·˜ 2 ‰Â›¯ÓÂÈ ÌÂÚÈο ·fi Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ‚·ÎÙËÚȉȷÎÒÓ ·ıÔÁfiÓˆÓ Ô˘ ÚÔηÏÔ‡Ó ‰È¿ÚÚÔÈ· ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜. ªÈ· ȉȷ›ÙÂÚË ÔÌ¿‰· ·ÔÙÂÏ› Ë ‰È¿ÚÚÔÈ· Ô˘ ÔÊ›ÏÂÙ·È Û ÙÔ͛Ә ‚·ÎÙËÚȉ›ˆÓ Ô˘ ÌÔχÓÔ˘Ó ÙÔÓ ¿ÓıÚˆÔ Ì ÙȘ ÙÚÔʤ˜ (9). √È ÙÔ͛Ә ›ÙÂ Â›Ó·È ÚÔÛ¯ËÌ·ÙÈṲ̂Ó˜, fiˆ˜ Ë ÙÔÍ›ÓË ÙÔ˘ ¯Ú˘Û›˙ÔÓÙÔ˜ ÛÙ·Ê˘ÏfiÎÔÎÎÔ˘, ›Ù ·Ú¿ÁÔÓÙ·È ·fi ÙÔ ‚·ÎÙËÚ›‰ÈÔ ÌÂÙ¿ ÙËÓ Â›ÛÔ‰fi ÙÔ˘ ÛÙÔÓ ·ÓıÚÒÈÓÔ ÔÚÁ·ÓÈÛÌfi, fiˆ˜ ÙÔ ‰ÔÓ¿ÎÈÔ Ù˘ ¯ÔϤڷ˜ Ô˘ ·ÔÙÂÏ› ÙËÓ Î‡ÚÈ· ·ÈÙ›· Ù˘ ‰È¿ÚÚÔÈ·˜ ÛÙÔÓ ÙÚ›ÙÔ ÎfiÛÌÔ, ÙÔ ÎψÛÙËÚ›‰ÈÔ Ù˘ ·ÏÏ·ÓÙ›·Û˘, ÙÔ ÂÓÙÂÚÔÙÔÍÈÓÔÁfiÓÔ ÎÔÏÔ‚·ÎÙËÚ›‰ÈÔ (ETEC) Ô˘

¶›Ó·Î·˜ 1. ÷ڷÎÙËÚÈÛÙÈο ÈÒÓ Ô˘ ÚÔÛ‚¿ÏÏÔ˘Ó ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi.

πfi˜

∏ÏÈΛ· ÔÌ¿‰·˜ ÚÔÛ‚ÔÏ‹˜

ÃÚfiÓÔ˜

∂Ô¯ÈÎfiÙËÙ· ÂÒ·Û˘

¢È¿ÚÎÂÈ· Û˘Ìو̿وÓ

ƒfiÙ·-Èfi˜ Norwalk/calici-ÈÔ›

6-24 ÌËÓÒÓ > 3 ÂÙÒÓ

2-3 ̤Ú˜ 1-2 ̤Ú˜

2-8 ̤Ú˜ 12-48 ÒÚ˜

∂ÓÙÂÚÔ·‰ÂÓÔ-ÈÔ› ∞ÛÙÚÔ-ÈÔ›

< 2 ÂÙÒÓ 1-3 ÂÙÒÓ

7-8 ̤Ú˜ 3-4 ̤Ú˜

ÃÂÈÌÒÓ·˜ ÃÂÈÌÒÓ·˜ Î·È Î·ÏÔη›ÚÈ ∫·ÏÔη›ÚÈ ÕÁÓˆÛÙË

̤¯ÚÈ 14 ̤Ú˜ 1-4 ̤Ú˜

5


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 6

¶·È‰È·ÙÚÈ΋ 2001;64:4-13

Paediatriki 2001;64:4-13

¶›Ó·Î·˜ 2. ÷ڷÎÙËÚÈÛÙÈο ‚·ÎÙËÚȉ›ˆÓ Ô˘ ÚÔηÏÔ‡Ó ‰È¿ÚÚÔÈ·.

µ·ÎÙ‹ÚÈ·

™·ÏÌÔÓ¤ÏÏ·

™·ÏÌÔÓ¤ÏÏ·

(ÌË Ù˘ÊÔÂȉ‹˜)

Ù‡ÊÔ˘

ªÂÙ¿‰ÔÛË

¤ÓÙÂÚÔÛÙÔÌ·ÙÈ΋

¶ËÁ‹ ÌfiÏ˘ÓÛ˘

∫Ú¤·˜, ·˘Á¿, Á¿Ï·

ÃÚfiÓÔ˜ ÂÒ·Û˘ ºÔÚ›·

6-72 ÒÚ˜

µ·ÎÙËÚÈ·ÈÌ›·

5-40% (‚Ú¤ÊË) 2-7 ̤Ú˜

¢È¿ÚÎÂÈ· ÓfiÛÔ˘ ∂ÈÏÔΤ˜

¡·È

¤ÓÙÂÚÔÛÙÔÌ·ÙÈ΋

¡ÂÚfi

7-14 ̤Ú˜

™ÈÁΤÏÏ·

ÀÂÚÛ›ÓÈ·

ÎÙËÚ›‰ÈÔ jejuni

enterocolitica

¤ÓÙÂÚÔÛÙÔÌ·ÙÈ΋

¤ÓÙÂÚÔÛÙÔÌ·ÙÈ΋ Â·Ê‹ Ì ˙Ò·

∆ÚÔʤ˜, ÓÂÚfi

¡ÂÚfi, Á¿Ï·, (΢ڛˆ˜ Ô˘ÏÂÚÈο)

12-48 ÒÚ˜

2-4 ̤Ú˜ Ÿ¯È

∞ÂÚÔÌÔÓ¿‰·

¤ÓÙÂÚÔÛÙÔÌ·ÙÈ΋ Â·Ê‹ Ì ˙Ò·, ÌÂÙ¿ÁÁÈÛË ·›Ì·ÙÔ˜ ¡ÂÚfi, Á¿Ï·, ÎÚ¤·˜ (΢ڛˆ˜ ¯ÔÈÚÈÓfi)

§‹„Ë ÌÔÏ˘Ṳ̂ÓÔ˘ ÓÂÚÔ‡

1-3 ‚‰ÔÌ¿‰Â˜

ÕÁÓˆÛÙÔ˜

Ÿ¯È

¡ÂÚfi

¡·È

Ÿ¯È

™˘¯Ó‹

™¿ÓÈ·

™¿ÓÈ·

2-3 ‚‰.

2-5 ̤Ú˜

2-7 ̤Ú˜

ªËÓÈÁÁ›Ùȉ·,

∞ÈÌÔÚÚ·Á›·

™·ÛÌÔ›,

™·ÛÌÔ›,

∞ÚıÚ›Ùȉ·

™˘Ó‹ıˆ˜

ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·, ·ÚıÚ›Ùȉ·, „¢‰ÔÛΈÏËÎÔÂȉ›Ùȉ·,

ÂÙÈÎÔ‡, ·ÈÌÔÏ˘ÙÈÎfi Ë·Ù›Ùȉ·, -Ô˘Ú·ÈÌÈÎfi ¯ÔÏÔ΢ÛÙ›Ùȉ· Û‡Ó‰ÚÔÌÔ, ‰È¿ÙÚËÛË ÂÓÙ¤ÚÔ˘

Û‡Ó‰ÚÔÌÔ Guillain-Barre

„¢‰ÔÛΈÏËÎÔÂȉ›Ùȉ·, ÂÁÎÔÏ·ÛÌfi˜ ÙÔÍÈÎfi ÌÂÁ¿ÎÔÏÔ

ÚÔÛ‚¿ÏÏÂÈ ·È‰È¿ οو ÙˆÓ 3 ÂÙÒÓ

·ÔÙÂÏ› ÙËÓ ÈÔ Û˘¯Ó‹ ·ÈÙ›· Ù˘ ‰È¿ÚÚÔÈ·˜ ÙˆÓ Ù·ÍȉȈÙÒÓ Î·È ÙÔ ÂÓÙÂÚÔ·ÈÌÔÚÚ·ÁÈÎfi ÎÔÏÔ‚·ÎÙËÚ›‰ÈÔ (O157:H7) Ô˘ Â›Ó·È ¤Ó· ·fi Ù· ·ÚÈ· ·›ÙÈ· ÙÔ˘ ·ÈÌÔÏ˘ÙÈÎÔ‡-Ô˘Ú·ÈÌÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘. ∏ ÓfiÛÔ˜ Ô˘ ÚÔηÏÂ›Ù·È ·fi ÚÔÛ¯ËÌ·ÙÈṲ̂Ó˜ ÙÔ͛Ә ¤¯ÂÈ ÌÈÎÚfiÙÂÚÔ ¯ÚfiÓÔ ÂÒ·Û˘ (1 ¤ˆ˜ 6 ÒÚ˜) Î·È ÌÈÎÚfiÙÂÚË ‰È¿ÚÎÂÈ· (<12 ÒÚ˜) Û ۇÁÎÚÈÛË Ì ÙË ÓfiÛÔ Ô˘ ÚÔηÏÂ›Ù·È ·fi in vivo ·Ú·ÁˆÁ‹ ÙÔÍÈÓÒÓ Ë ÔÔ›· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÌÂÁ·Ï‡ÙÂÚÔ ¯ÚfiÓÔ ÂÒ·Û˘ (6 ¤ˆ˜ 73 ÒÚ˜) Î·È ÌÂÁ·Ï‡ÙÂÚË ‰È¿ÚÎÂÈ· (1 ¤ˆ˜ 10 ̤Ú˜). ∆¤ÏÔ˜, ÙÔ Clostridium difficile Â›Ó·È Î˘Ú›ˆ˜ ÓÔÛÔÎÔÌÂÈ·Îfi˜ ÔÚÁ·ÓÈÛÌfi˜ Ô˘ ÚÔηÏ› ‰È¿ÚÚÔÈ· ̤ۈ ·Ú·ÁˆÁ‹˜ ÙÔÍÈÓÒÓ. ∏ ÓfiÛÔ˜ ÔÊ›ÏÂÙ·È ÛÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙÔ˘ ‚·ÎÙËÚȉ›Ô˘ ÌÂÙ¿ ·fi ÙËÓ ·ÏÏ·Á‹ Ù˘ ÂÓÙÂÚÈ΋˜ ¯ÏˆÚ›‰·˜ Ô˘ ¤¯ÂÈ ÂÈʤÚÂÈ Ë ÚÔËÁÔ‡ÌÂÓË ¯ÔÚ‹ÁËÛË ·ÓÙÈ‚ÈÔÙÈÎÒÓ. ∏ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ Â›Ó·È ·Û˘Ìو̷ÙÈÎÔ› ÊÔÚ›˜ ‹ ¤¯Ô˘Ó ˘‰·Ú‹ ‰È¿ÚÚÔÈ·, ÂÓÒ Ë ÎÏ·ÛÛÈ΋ ÂÈÎfiÓ· Ù˘ „¢‰ÔÌÂÌ‚Ú·ÓÒ‰Ô˘˜ ÎÔÏ›Ùȉ·˜ Ì ÎÔÏÈÎÔÂȉ‹ ÎÔÈÏȷο ¿ÏÁË, ˘ÚÂÙfi Î·È ‚ÏÂÓÓÔ·ÈÌ·ÙËÚ¤˜ ÎÂÓÒÛÂȘ ·ÚÔ˘ÛÈ¿˙ÂÙ·È ÛÙÔ 10% ÙˆÓ ÂÚÈÙÒ-

6

∫·Ì˘ÏÔ‚·-

™¿ÓÈ· (‚Ú¤ÊË) 14 ̤Ú˜

<1% ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜ ™¿ÓÈ· 7-14 ̤Ú˜

ÛÂˆÓ (10). √È ·Ú·ÛÈÙÈΤ˜ ·Èٛ˜ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ¤¯Ô˘Ó ÛËÌ·ÓÙÈΤ˜ Û˘Ó¤ÂȘ ÁÈ· ÙË ‰ËÌfiÛÈ· ˘Á›· (6,7). ∏ Ï¿Ì‚ÏÈ· Î·È ÙÔ ÎÚ˘ÙÔÛÔÚ›‰ÈÔ Â›Ó·È Ù· ÈÔ Û˘¯Ó¿ ·›ÙÈ· ·Ú·ÛÈÙÈ΋˜ ‰È¿ÚÚÔÈ·˜ ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜. ™ÙËÓ ·ÙÚ›‰· Ì·˜, Ë Û˘¯ÓfiÙËÙ· Ù˘ ÔÍ›·˜ Á·ÛÙÚÂÓÙÂÚ›Ùȉ·˜ ·fi ÎÚ˘ÙÔÛÔÚ›‰ÈÔ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË Û ·È‰È¿ ËÏÈΛ·˜ ÌÂٷ͇ 6 ÌËÓÒÓ Î·È 4 ¯ÚfiÓˆÓ, Ì ÙÔ 44% ÙˆÓ ·ÛıÂÓÒÓ Î¿Ùˆ ÙˆÓ 24 ÌËÓÒÓ (11). ∆fiÛÔ ÙÔ ÎÚ˘ÙÔÛÔÚ›‰ÈÔ fiÛÔ Î·È Ë Ï¿Ì‚ÏÈ· ÚÔÛÎÔÏÏÒÓÙ·È ‹ ‰ÈÂÈÛ‰‡Ô˘Ó ÛÙÔ ‚ÏÂÓÓÔÁfiÓÔ ÙÔ˘ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘ Î·È ÌÔÚÔ‡Ó, ·Ó ¯ÚÔÓ›ÛÔ˘Ó, Ó· ÚÔηϤÛÔ˘Ó Û‡Ó‰ÚÔÌÔ ‰˘Û·ÔÚÚfiÊËÛ˘. ªÂÙ·‰›‰ÔÓÙ·È Ì ÙËÓ ÎÔÚ·ÓÔÛÙÔÌ·ÙÈ΋ Ô‰fi ·fi ¿ÙÔÌÔ Û ¿ÙÔÌÔ ‹ Ì ÌfiÏ˘ÓÛË ÙˆÓ ˘‰·ÙÈÎÒÓ ËÁÒÓ ·fi ÙȘ ·ÛÙÂȘ ÙˆÓ ·Ú·Û›ÙˆÓ Ô˘ Â›Ó·È Ì¿ÏÈÛÙ· ·ÓıÂÎÙÈΤ˜ ÛÙË ¯ÏˆÚ›ˆÛË ÙÔ˘ ÓÂÚÔ‡. ∏ ‰È·ÎÔ‹ Ù˘ ÌÂÙ¿‰ÔÛ˘, ΢ڛˆ˜ fiÙ·Ó ˘¿Ú¯Ô˘Ó ÎÚÔ‡ÛÌ·Ù· Û ‚ÚÂÊÔÓËÈ·ÎÔ‡˜ ÛÙ·ıÌÔ‡˜, Â›Ó·È ÚˆÙ·Ú¯È΋˜ ÛËÌ·Û›·˜. ∏ ‰È¿ÚÚÔÈ· Ô˘ ÚÔηÏÔ‡Ó ‰ÂÓ Â›Ó·È ‚ÏÂÓÓÔ·ÈÌ·ÙËÚ‹, ÂÓÒ ·˘Ù‹ ÙÔ˘ ÎÚ˘ÙÔÛÔÚȉ›Ô˘ Â›Ó·È Ôχ


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 7

¶·È‰È·ÙÚÈ΋ 2001;64:4-13

˘‰·Ú‹˜. H ·ÌÔÈ‚¿‰· Â›Ó·È Û˘¯Ófi ·›ÙÈÔ ·Ú·ÛÈÙÈ΋˜ ‰È¿ÚÚÔÈ·˜ ÛÙȘ ·Ó·Ù˘ÛÛfiÌÂÓ˜ ¯ÒÚ˜ Î·È ÂΉËÏÒÓÂÙ·È ÌÂ Û˘ÌÙÒÌ·Ù· ‰˘ÛÂÓÙÂÚÈ΋˜ ÎÔÏ›Ùȉ·˜ (˘ÚÂÙfi, ÎÔÈÏÈ·Îfi ¿ÏÁÔ˜, ÌÈÎÚ¤˜ Î·È Û˘¯Ó¤˜ ‚ÏÂÓÓÔ·ÈÌ·ÙËÚ¤˜ ÎÂÓÒÛÂȘ, ÙÂÈÓÂÛÌfi). ¶·ıÔÊ˘ÛÈÔÏÔÁ›· ¶·ıÔÊ˘ÛÈÔÏÔÁÈο, Ë ‰È¿ÚÚÔÈ· ‰È·ÎÚ›ÓÂÙ·È Û 4 Ù‡Ô˘˜: ÂÎÎÚÈÙÈ΋, ΢ÙÙ·ÚÔÙÔÍÈ΋, ÔÛ̈ÙÈ΋ Î·È ‰˘ÛÂÓÙÂÚ›·. ∏ ‰È¿ÚÚÔÈ· ÌÔÚ› Ó· ·ÔÙÂÏ› Û˘Ó‰˘·ÛÌfi ÂÓfi˜ ‹ ÂÚÈÛÛÔÙ¤ÚˆÓ ·fi ÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ ·˘ÙÔ‡˜ (1,12). ∏ ÂÎÎÚÈÙÈ΋ ‰È¿ÚÚÔÈ· ÔÊ›ÏÂÙ·È Û ÂÓÙÂÚÔÙÔ͛Ә ÔÈ Ôԛ˜ ·Ú¿ÁÔÓÙ·È ·fi ÏÔÈÌÔÁfiÓÔ˘˜ ÔÚÁ·ÓÈÛÌÔ‡˜. ∏ ÂÓÙÂÚÔÙÔÍ›ÓË Û˘Ó‰¤ÂÙ·È Ì ÂȉÈÎÔ‡˜ ˘ԉԯ›˜ Ô˘ ‚Ú›ÛÎÔÓÙ·È: ·) ÛÙ· ·ÙÙ·Ú· ÙˆÓ ÎÚ˘ÙÒÓ ÙÔ˘ ‚ÏÂÓÓÔÁfiÓÔ˘ Î·È Ô‰ËÁ› ÛÙËÓ ¤ÎÎÚÈÛË ‡‰·ÙÔ˜ Î·È ËÏÂÎÙÚÔÏ˘ÙÒÓ, ‚) ÛÙ· ·ÙÙ·Ú· ÙˆÓ Ï·¯ÓÒÓ ÙÔ˘ ‚ÏÂÓÓÔÁfiÓÔ˘ Î·È ·Ó·ÛÙ¤ÏÏÂÈ ÙËÓ ·ÔÚÚfiÊËÛË ‡‰·ÙÔ˜ Î·È ËÏÂÎÙÚÔÏ˘ÙÒÓ. ∏ ‰È·‰Èηۛ˜ ·˘Ù¤˜ ÂÈÙÂÏÔ‡ÓÙ·È ÂÓ‰Ô΢ÙÙ¿ÚÈ· Ì ÙË ÌÂÛÔÏ¿‚ËÛË Ù˘ ΢ÎÏÈ΋˜ ÌÔÓÔʈÛÊÔÚÈ΋˜ ·‰ÂÓÔÛ›Ó˘ ‹ ÁÔ˘·ÓÔÛ›Ó˘. §ÔÈÌÔÁfiÓÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ‰ÚÔ˘Ó Ì¤Ûˆ ÙÔ˘ ÂÎÎÚÈÙÈÎÔ‡ Ì˯·ÓÈÛÌÔ‡ Â›Ó·È ÙÔ ÎÔÏÔ‚·ÎÙËÚ›‰ÈÔ, Ë Û·ÏÌÔÓ¤ÏÏ·, Ë ÛÈÁΤÏÏ·, Ë ˘ÂÚÛ›ÓÈ·, Ë ·ÂÚÔÌÔÓ¿‰·, Ë Ï¿Ì‚ÏÈ·, Ë ¯ÔϤڷ, Ô ¯Ú˘Û›˙ˆÓ ÛÙ·Ê˘ÏfiÎÔÎÎÔ˜, ÙÔ Clostridium difficile Î·È ÙÔ ÎψÛÙËÚ›‰ÈÔ Ù˘ ·ÏÏ·ÓÙ›·Û˘ (Clostridium botulinum). ∏ ΢ÙÙ·ÚÔÙÔÍÈ΋ ‰È¿ÚÚÔÈ· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ Î·Ù·ÛÙÚÔÊ‹ ÙˆÓ ÂÈÊ·ÓÂÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘ Ô˘ Ô‰ËÁ› Û ÌÂÚÈ΋ ‹ Ï‹ÚË ÂÈ¤‰ˆÛË ÙˆÓ Ï·¯ÓÒÓ. ™·Ó Û˘Ó¤ÂÈ·, Â¤Ú¯ÂÙ·È Ì›ˆÛË Ù˘ ·ÔÚÚÔÊËÙÈ΋˜ ÂÈÊ¿ÓÂÈ·˜ ÙÔ˘ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘ Î·È ·ÒÏÂÈ· ‡‰·ÙÔ˜ Î·È ËÏÂÎÙÚÔÏ˘ÙÒÓ. ∂ÈϤÔÓ, Ù· ÂÚÈÛÛfiÙÂÚ· ·ÙÙ·Ú· Ô˘ ·Ô̤ÓÔ˘Ó Â›Ó·È ·˘Ù¿ ÙˆÓ ÎÚ˘ÙÒÓ ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ÂÈıËÏ›Ô˘ Ô˘ Â›Ó·È Î·ÙÂÍÔ¯‹Ó ÂÎÎÚÈÙÈο ·ÙÙ·Ú·. ∆· ÈÔ Û˘¯Ó¿ ·›ÙÈ· ·˘ÙÔ‡ ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ·ÔÙÂÏÔ‡Ó ÔÈ ÈÔ› (ƒfiÙ·, Norwalk). §ÈÁfiÙÂÚÔ Û˘¯Ó¿ Â›Ó·È ÙÔ ÎÚ˘ÙÔÛÔÚ›‰ÈÔ Î·È ÙÔ ÎÔÏÔ‚·ÎÙËÚ›‰ÈÔ. ∏ ÔÛ̈ÙÈ΋ ‰È¿ÚÚÔÈ· ··ÓÙ¿Ù·È ÛÙ· Û‡Ó‰ÚÔÌ· ‰˘Û·ÔÚÚfiÊËÛ˘, ·fi Ù· ÔÔ›· ÙÔ ÈÔ Û˘¯Ófi Â›Ó·È Ë ‰˘Û·ÓÂÍ›· ÛÙË Ï·ÎÙfi˙Ë, Ë ÔÔ›· ·Ú·ÙËÚÂ›Ù·È Ì ÙË ‚Ï¿‚Ë ÙˆÓ ÂÈÊ·ÓÂÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ‚ÏÂÓÓÔÁfiÓÔ˘ ÙÔ˘ ÂÓÙ¤ÚÔ˘ Ô˘ ÂÚȤ¯Ô˘Ó ÙÔ ¤Ó˙˘ÌÔ Ï·ÎÙ¿ÛË. ∞˘Ùfi ÌÔÚ› Ó· Û˘Ì‚Â› ÌÂÙ¿ ·fi ÏÔÈÌÒÍÂȘ Ô˘ ÚÔÛ‚¿ÏÏÔ˘Ó ÙÔ ÏÂÙfi ¤ÓÙÂÚÔ (.¯. ƒfiÙ·-Èfi˜, §¿Ì‚ÏÈ·). ∆· ‰˘Û·ÔÚÚÔÊÔ‡ÌÂÓ· ÌfiÚÈ· fiÙ·Ó Êı¿ÛÔ˘Ó Û ÌÈ· Û˘ÁΤÓÙÚˆÛË ÛÙÔÓ ·˘Ïfi ÙÔ˘ ÂÓÙ¤ÚÔ˘ Ô˘ Â›Ó·È ÔÛ̈ÙÈο ÂÓÂÚÁ‹, Û˘Ì·Ú·Û‡ÚÔ˘Ó ÌfiÚÈ· ÓÂÚÔ‡ ̤۷ ÛÙÔÓ ·˘Ïfi Î·È Î·Ù¿ Û˘Ó¤ÂÈ· Ô‰ËÁÔ‡Ó Û ·‡ÍËÛË ÙÔ˘ ÂÚÈÛÙ·Ï-

Paediatriki 2001;64:4-13

ÙÈÛÌÔ‡ ÙÔ˘ ÂÓÙ¤ÚÔ˘ Î·È ‰È¿ÚÚÔÈ·. √È ‰˘Û·ÔÚÚÔÊÔ‡ÌÂÓÔÈ ˘‰·Ù¿ÓıڷΘ ˙˘ÌÒÓÔÓÙ·È ·fi ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜ ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘. ∆· ÚÔ˚fiÓÙ· ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ ·˘ÙÔ‡ Â›Ó·È Ù· CO2, H2, Ù· ÔÔ›· ÚÔηÏÔ‡Ó ·¤ÚÈ· Î·È ÌÂÙˆÚÈÛÌfi ÛÙÔÓ ·ÛıÂÓ‹ Î·È Ù· ÏÈ·Ú¿ Ôͤ· ÌÈÎÚ‹˜ ·Ï‡ÛÔ˘, Ù· ÔÔ›· Â›Ó·È ˘‡ı˘Ó· ÁÈ· ÙÔ ¯·ÌËÏfi pH ÙˆÓ ÎÔÚ¿ÓˆÓ (οو ÙÔ˘ 5,5). ∏ ‰˘ÛÂÓÙÂÚ›· ÚÔηÏÂ›Ù·È ·fi ÊÏÂÁÌÔÓ‹ ÙÔ˘ ‚ÏÂÓÓÔÁfiÓÔ˘ Î·È ˘Ô‚ÏÂÓÓÔÁfiÓÈÔ˘ ÙÔ˘ ÙÂÏÈÎÔ‡ ÂÈÏÂÔ‡ Î·È ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘ ·fi ÙË ‰È›ۉ˘ÛË ‚·ÎÙËÚ›ˆÓ. ∏ ‰È›ۉ˘ÛË ·˘Ù‹ ÚÔηÏ› Ô›‰ËÌ·, ·ÈÌÔÚÚ·Á›· Î·È ‰È‹ıËÛË ÙÔ˘ ‚ÏÂÓÓÔÁfiÓÔ˘ ·fi Ï¢ÎÔ·ÙÙ·Ú·. ∆· Ï¢ÎÔ·ÙÙ·Ú· Î·È ÙÔ ·›Ì· ‰È·¯¤ÔÓÙ·È ÛÙÔÓ ·˘Ïfi ÙÔ˘ ÂÓÙ¤ÚÔ˘ Î·È ·Ô‚¿ÏÏÔÓÙ·È ÛÙ· ÎfiÚ·Ó·. √ ÂÚÂıÈÛÌfi˜ ·fi ÙË ÊÏÂÁÌÔÓ‹ ÚÔηÏ› ·˘ÍË̤ÓË ÎÈÓËÙÈÎfiÙËÙ· ÙÔ˘ ÎfiÏÔ˘ Î·È Û˘¯Ó¤˜ ÎÂÓÒÛÂȘ, Û˘¯Ó¿ Ì ÙÂÈÓÂÛÌfi. §ÔÈÌÔÁfiÓÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ‰ÚÔ˘Ó Ì¤Ûˆ ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ·˘ÙÔ‡ Â›Ó·È Ë Û·ÏÌÔÓ¤ÏÏ·, Ë ÛÈÁΤÏÏ·, ÙÔ Î·Ì˘ÏÔ‚·ÎÙËÚ›‰ÈÔ, Ë ˘ÂÚÛ›ÓÈ·, Ë ·ÌÔÈ‚¿‰· Î·È ÙÔ Clostridium difficile. ¢ÈÂÚ‡ÓËÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ πÛÙÔÚÈÎfi: ∆Ô Î·Ïfi ÈÛÙÔÚÈÎfi ı· ÚÔÛ·Ó·ÙÔÏ›ÛÂÈ ÙË ‰ÈÂÚ‡ÓËÛË ÛÙË ÛˆÛÙ‹ ηÙ‡ı˘ÓÛË Î·È ı· ·ÔÙÚ¤„ÂÈ ¿ÛÎÔ˜ ÂÚÁ·ÛÙËÚȷΤ˜ ÂÍÂÙ¿ÛÂȘ. √È ÂÚˆÙ‹ÛÂȘ ·ÊÔÚÔ‡Ó ÚfiÛÊ·ÙË ¯Ú‹ÛË ·ÓÙÈ‚ÈÔÙÈÎÒÓ, ˘ÔΛÌÂÓ˜ ÓfiÛÔ˘˜ (.¯. ·ÓÔÛÔÏÔÁÈ΋ ·ÓÂ¿ÚÎÂÈ·), Ù·Í›‰È· Û ¿ÏϘ ¯ÒÚ˜ ‹ ÂÚÈÔ¯¤˜ fiÔ˘ ÔÈ Û˘Óı‹Î˜ ·Ô¯¤Ù¢Û˘ ‹ fiÛÈÌÔ˘ ÓÂÚÔ‡ ‰ÂÓ Â›Ó·È ÔÈ Î·Ù¿ÏÏËϘ, ·Ú·ÎÔÏÔ‡ıËÛË ‚ÚÂÊÔÓËÈ·ÎÔ‡ ÛÙ·ıÌÔ‡ ηıÒ˜ Î·È ÏËÚÔÊÔڛ˜ ÁÈ· ÙÚÔʤ˜ Ô˘ ηٷӷÏÒıËÎ·Ó ÚfiÛÊ·Ù·. ¢‡Ô ·Ú¿ÁÔÓÙ˜ Â›Ó·È È‰È·›ÙÂÚ· ¯Ú‹ÛÈÌÔÈ ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË Ù˘ ·ÈÙÈÔÏÔÁ›·˜ Ù˘ ‰È¿ÚÚÔÈ·˜, ÙÔ ÈÛÙÔÚÈÎfi ÂÌÂÙÔ‡ Î·È Ë Û‡ÛÙ·ÛË Î·È Û˘¯ÓfiÙËÙ· ÙˆÓ ÎÂÓÒÛˆÓ. ∏ ¯ÚÔÓÈ΋ ÛÙÈÁÌ‹ Ù˘ ÂÌÊ¿ÓÈÛ˘ ÙÔ˘ ÂÌÂÙÔ‡ ÌÔÚ› Ó· ‚ÔËı‹ÛÂÈ ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÓfiÛÔ˘ ÙÔ˘ ÏÂÙÔ‡ ·fi ÙÔ ·¯‡ ¤ÓÙÂÚÔ. ∏ ÓfiÛÔ˜ ÙÔ˘ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘, Ë ÔÔ›· ΢ڛˆ˜ ÔÊ›ÏÂÙ·È Û ÈÔ‡˜, ÚÔηÏ› ÂÈÏÂfi ÙÔ˘ ÂÓÙ¤ÚÔ˘ Ì ‰È¿Ù·ÛË ÙÔ˘ ·˘ÏÔ‡ Î·È ÂÈ‚Ú¿‰˘ÓÛË Ù˘ ΤӈÛ˘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Ô˘ Ô‰ËÁÔ‡Ó Û ÂÌÂÙfi ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ‰È¿ÚÚÔÈ·˜. √È ÎÂÓÒÛÂȘ Â›Ó·È ˘‰·Ú›˜, ÌÂÁ¿ÏÔ˘ fiÁÎÔ˘ Î·È ÏÈÁfiÙÂÚÔ Û˘¯Ó¤˜ ·fi ÙȘ ÓfiÛÔ˘˜ ÙÔ˘ ·¯¤Ô˜ ÂÓÙ¤ÚÔ˘ fiÔ˘ Â›Ó·È ÂÚÈÛÛfiÙÂÚ˜, ÌÈÎÚfiÙÂÚ˜ Î·È ÏÈÁfiÙÂÚÔ ˘‰·Ú›˜. º˘ÛÈ΋ ÂͤٷÛË: √ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙÔ˘ ‚·ıÌÔ‡ Ù˘ ·Ê˘‰¿ÙˆÛ˘ Â›Ó·È ÙÔ ÚˆÙ‡ÔÓ Ì¤ÏËÌ· Ù˘ Ê˘ÛÈ΋˜ ÂͤٷÛ˘. √ ›Ó·Î·˜ 3 ·ÚÔ˘ÛÈ¿˙ÂÈ Ù· ÎÏÈÓÈο ÛËÌ›· Ô˘ ‚ÔËıÔ‡Ó ÛÙËÓ ÂÎÙ›ÌËÛ‹ Ù˘. ∫·Ù¿ ÙËÓ ÂͤٷÛË Ù˘ ÎÔÈÏÈ¿˜, ÂÎÙÈÌ¿Ù·È ·Ó ˘¿Ú¯ÂÈ Â˘·ÈÛıËÛ›·, ÌÂÙˆÚÈÛÌfi˜ Î·È ·ÍÈÔÏÔÁÔ‡ÓÙ·È ÔÈ

7


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 8

¶·È‰È·ÙÚÈ΋ 2001;64:4-13

Paediatriki 2001;64:4-13

¶›Ó·Î·˜ 3. ∂ÎÙ›ÌËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ Ù˘ ·Ê˘‰¿ÙˆÛ˘. µ·ıÌfi˜ ·Ê˘‰¿ÙˆÛ˘ ◊È· (3-5%)

™˘ÌÙÒÌ·Ù· Î·È ÎÏÈÓÈο ÛËÌ›· º˘ÛÈÔÏÔÁÈο ˙ˆÙÈο ÛËÌ›· ∆Ô ·È‰› Â›Ó·È Û ÂÁÚ‹ÁÔÚÛË µÏÂÓÓÔÁfiÓÔÈ (ÛÙfiÌ·ÙÔ˜ Î·È ¯ÂÈÏÈÒÓ) Ì ‹È· ÍËÚfiÙËÙ·, ¤¯ÂÈ ‰¿ÎÚ˘· fiÙ·Ó ÎÏ·›ÂÈ ◊ÈÔ ·›ÛıËÌ· ‰›„·˜ º˘ÛÈÔÏÔÁÈ΋ Û·ÚÁ‹ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ ÃÚÔÓÈ΋ ‰È¿ÚÎÂÈ· Â·Ó·Ï‹ÚˆÛ˘ ÙÚȯÔÂȉÒÓ* ÌË ·Ú·ÙÂٷ̤ÓË (οو ÙˆÓ 2 sec) ™Â ‚Ú¤ÊË Ë ÚfiÛıÈ· ËÁ‹ ‰ÂÓ Â›Ó·È ‚˘ıÈṲ̂ÓË

ª¤ÙÚÈ· (6-9%)

√È ÛÊ˘ÁÌÔ› Î·È Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ·Ó·ÓÔÒÓ Â›Ó·È ·˘ÍË̤Ó˜, Ë ·ÚÙËÚȷ΋ ›ÂÛË Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋ ‹ ÂÏ·ÊÚÒ˜ ÂÏ·Ùو̤ÓË ∆Ô ·È‰› Â›Ó·È ÓˆıÚfi ‹ ¢ÂÚ¤ıÈÛÙÔ √È ‚ÏÂÓÓÔÁfiÓÔÈ (ÛÙfiÌ·ÙÔ˜ Î·È ¯ÂÈÏÈÒÓ) Â›Ó·È ÍËÚÔ›, ‰ÂÓ ¤¯ÂÈ ‰¿ÎÚ˘· fiÙ·Ó ÎÏ·›ÂÈ ™ËÌ·ÓÙÈÎfi ·›ÛıËÌ· ‰›„·˜ ∏ Û·ÚÁ‹ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ Â›Ó·È ÂÏ·ÊÚ¿ ÂÏ·Ùو̤ÓË ÃÚÔÓÈ΋ ‰È¿ÚÎÂÈ· Â·Ó·Ï‹ÚˆÛ˘ ÙÚȯÔÂȉÒÓ ·Ú·ÙÂٷ̤ÓË (2-3 sec) ™Â ‚Ú¤ÊË Ë ÚfiÛıÈ· ËÁ‹ Â›Ó·È ‚˘ıÈṲ̂ÓË

™Ô‚·Ú‹ (≥10%)

√È ÛÊ˘ÁÌÔ› Î·È Ë Û˘¯ÓfiÙËÙ· ÙˆÓ ·Ó·ÓÔÒÓ Â›Ó·È ·˘ÍË̤Ó˜, Ë ·ÚÙËÚȷ΋ ›ÂÛË Â›Ó·È ÂÏ·Ùو̤ÓË ∆Ô ·È‰› ‚Ú›ÛÎÂÙ·È Û ϋı·ÚÁÔ ‹ ÎÒÌ· √È ‚ÏÂÓÓÔÁfiÓÔÈ (ÛÙfiÌ·ÙÔ˜ Î·È ¯ÂÈÏÈÒÓ) Â›Ó·È ÍËÚÔ›, ‰ÂÓ ¤¯ÂÈ ‰¿ÎÚ˘· fiÙ·Ó ÎÏ·›ÂÈ ™ËÌ·ÓÙÈÎfi ·›ÛıËÌ· ‰›„·˜ ∏ Û·ÚÁ‹ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ Â›Ó·È Ôχ ÂÏ·Ùو̤ÓË ∆Ô ‰¤ÚÌ· Â›Ó·È „˘¯Úfi ÃÚÔÓÈ΋ ‰È¿ÚÎÂÈ· Â·Ó·Ï‹ÚˆÛ˘ ÙÚȯÔÂȉÒÓ Ôχ ·Ú·ÙÂٷ̤ÓË (>3 sec) ™Â ‚Ú¤ÊË Ë ÚfiÛıÈ· ËÁ‹ Â›Ó·È Ôχ ‚˘ıÈṲ̂ÓË

* √ ¯ÚfiÓÔ˜ Â·Ó·Ï‹ÚˆÛ˘ ÙÚȯÔÂȉÒÓ Â›Ó·È Ë ¯ÚÔÓÈ΋ ‰È¿ÚÎÂÈ· Ô˘ ··ÈÙÂ›Ù·È ÁÈ· ÙËÓ ÂÈÛÙÚÔÊ‹ ÙÔ˘ Úfi‰ÈÓÔ˘ ¯ÚÒÌ·ÙÔ˜ Ô˘ ‰ËÌÈÔ˘ÚÁ› ÙÔ ÂÚÈÊÂÚÈÎfi ‰›ÎÙ˘Ô ÙˆÓ ÙÚȯÔÂȉÒÓ ÛÙȘ ¿ÎÚ˜ ÙˆÓ ‰·ÎÙ‡ÏˆÓ ÌÂÙ¿ ÙËÓ ÂÏ·ÊÚ¿ ¿ÛÎËÛË ›ÂÛ˘ ÛÙ· ÛËÌ›· ·˘Ù¿ ÒÛÙ ӷ ÚÔÎÏËı› ˆ¯ÚfiÙËÙ· (25).

ÂÓÙÂÚÈÎÔ› ‹¯ÔÈ. √ ÌÂÙˆÚÈÛÌfi˜ Ù˘ ÎÔÈÏÈ¿˜ ÌÔÚ› Ó· Û¯ÂÙ›˙ÂÙ·È Ì ÂÈÏÂfi ‹ ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ·ÂÚ›ˆÓ ÏfiÁˆ ‰˘Û·ÔÚÚfiÊËÛ˘. ∞ÓÙ›ıÂÙ·, Ë ÛηÊÔÂȉ‹˜ ÎÔÈÏÈ¿ ÌÔÚ› Ó· ˘Ô‰ËÏÒÓÂÈ ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛË. ∞‡ÍËÛË ÙÔ˘ ÂÚÈÛÙ·ÏÙÈÛÌÔ‡ ÙÔ˘ ÂÓÙ¤ÚÔ˘ ·Ú·ÙËÚÂ›Ù·È ÛÙËÓ ÂÓÙÂÚ›Ùȉ· Î·È ÙËÓ ÂÎÎÚÈÙÈ΋ ‰È¿ÚÚÔÈ·, ÂÓÒ ÛÙË ‰˘ÛÂÓÙÂÚ›· ÔÈ ÂÓÙÂÚÈÎÔ› ‹¯ÔÈ Â›Ó·È ÏÈÁfiÙÂÚÔ ÂÓÂÚÁÔ›. ªÔÏÔÓfiÙÈ Ù· ÛËÌ›· ÂÚÂıÈÛÌÔ‡ ÙÔ˘ ÂÚÈÙÔÓ·›Ô˘ ÌÔÚ› Ó· Â›Ó·È ·ÚfiÓÙ· Û ÂÚÈÙÒÛÂȘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ·fi ÌÂÁ¿ÏË ÊÏÂÁÌÔÓ‹ ÙÔ˘ ÂÓÙ¤ÚÔ˘ (E.coli 0157:H7, ™ÈÁΤÏÏ·, ·ÌÔÈ‚¿‰·), ‰ÂÓ ı· Ú¤ÂÈ Ó· ·Ú·‚ÏÂÊı› Ë Èı·ÓfiÙËÙ· Ë ‰È¿ÚÚÔÈ· Ó· Â›Ó·È ‰Â˘ÙÂÚÔ·ı‹˜, ‰ËÏ·‰‹ ·ÔÙ¤ÏÂÛÌ· ˘¿Ú¯Ô˘Û·˜ ÂÚÈÙÔÓ›Ùȉ·˜. ∏ ·Ï‹ ·Ú·Ù‹ÚËÛË ÙˆÓ ÎÔÚ¿ÓˆÓ ÁÈ· ·ÚÔ˘Û›· ‚ϤÓÓ·˜ Î·È ·›Ì·ÙÔ˜ Â›Ó·È ÛËÌ·ÓÙÈ΋˜ ÛËÌ·Û›·˜ ÁÈ· ÙË ‰ÈÂÚ‡ÓËÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜.

8

∂ÚÁ·ÛÙËÚȷ΋ ‰ÈÂÚ‡ÓËÛË: ∏ ·ÚÔ˘Û›· Ï¢ÎÔ΢ÙÙ¿ÚˆÓ ÛÙ· ÎfiÚ·Ó· Â›Ó·È ¯Ú‹ÛÈÌË ÛÙË ‰È¿ÁÓˆÛË Ù˘ ‰È¿ÚÚÔÈ·˜ ·fi ‚·ÎÙËÚ›‰È·, ΢ڛˆ˜ ·˘ÙÒÓ Ô˘ ÚÔηÏÔ‡Ó ‰˘ÛÂÓÙÂÚ›·. √È ·ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó Ï¢ÎÔ·ÙÙ·Ú· ÛÙ· ÎfiÚ·Ó¿ ÙÔ˘˜ ¤¯Ô˘Ó ‚·ÎÙËÚȉȷ΋ ‰È¿ÚÚÔÈ· Ì Èı·ÓfiÙËÙ· 70%. ∏ ·ÚÔ˘Û›· ÔÏÏÒÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ Ì ·ÓÙÂÏ‹ ·Ô˘Û›· Ï¢ÎÔ΢ÙÙ¿ÚˆÓ Â›Ó·È ‡ÔÙË ÁÈ· Ïԛ̈ÍË ·fi ·ÌÔÈ‚¿‰·, ÌÈ· Î·È ÙÔ ·Ú¿ÛÈÙÔ ·˘Ùfi Ù›ÓÂÈ Ó· ηٷÛÙÚ¤ÊÂÈ Ù· ÏÂ˘Î¿ ·ÈÌÔÛÊ·›ÚÈ·. √È Î·ÏÏȤÚÁÂȘ ÎÔÚ¿ÓˆÓ Û˘ÓÈÛÙÒÓÙ·È ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ ÂΛÓÔ˘˜ Ô˘: ·) Ë ‰È¿ÚÚÔÈ¿ ÙÔ˘˜ Û˘Ó¯›˙ÂÙ·È ÁÈ· ÂÚÈÛÛfiÙÂÚ˜ ·fi 5 ̤Ú˜ ·Ú¿ ÙËÓ ÂÓ˘‰¿ÙˆÛË Î·È Â·Ó·Û›ÙÈÛË, ‚) ¤¯Ô˘Ó ÛËÌ›· ÊÏÂÁÌÔÓÒ‰Ô˘˜ ÓfiÛÔ˘ fiˆ˜ ˘ÚÂÙfi, ·ÚıÚ·ÏÁ›Â˜, Ì˘·ÏÁ›Â˜, Á) ¤¯Ô˘Ó Ï¢ÎÔ·ÙÙ·Ú· ‹ ÔÚ·Ùfi ·›Ì· ÛÙ· ÎfiÚ·Ó·, ‰) Â›Ó·È ·ÓÔÛÔηٷÛÙ·Ï̤ÓÔÈ, Â) ¤¯Ô˘Ó


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 9

¶·È‰È·ÙÚÈ΋ 2001;64:4-13

Paediatriki 2001;64:4-13

¶›Ó·Î·˜ 4. ∂ÚÁ·ÛÙËÚȷΤ˜ ÂÍÂÙ¿ÛÂȘ ÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË ÂÓÙÂÚÔ·ıÔÁfiÓˆÓ ÌÈÎÚÔ‚›ˆÓ. ∂ÚÁ·ÛÙËÚȷΤ˜ ÂÍÂÙ¿ÛÂȘ ªÈÎÚÔÛÎÔÈ΋ ÂͤٷÛË ÎÔÚ¿ÓˆÓ §Â˘ÎÔ·ÙÙ·Ú· ∆ÚÔÊÔ˙ˆ˝Ù˜, ·ÛÙÂȘ, ˆÔ·ÛÙÂȘ ™ÂÈÚÔÂȉ‹ ‚·ÎÙ‹ÚÈ· ∫·ÏÏȤÚÁÂÈ· ÎÔÚ¿ÓˆÓ ™˘Ó‹ı˘ ∂ȉÈ΋ ∞Ó›¯Ó¢ÛË ·ÓÙÈÁfiÓÔ˘ ‹ ÙÔ͛Ӣ (ELISA ‹ latex Û˘ÁÎfiÏÏËÛË)

√ÚÁ·ÓÈÛÌÔ› µ·ÎÙ‹ÚÈ· Ô˘ ÚÔηÏÔ‡Ó ‰˘ÛÂÓÙÂÚ›· §¿Ì‚ÏÈ·, ∞ÌÔÈ‚¿‰·, ∫Ú˘ÙÔÛÔÚ›‰ÈÔ ∫·Ì˘ÏÔ‚·ÎÙËÚ›‰ÈÔ

™·ÏÌÔÓ¤ÏÏ·, ™ÈÁΤÏÏ·, ∫·Ì˘ÏÔ‚·ÎÙËÚ›‰ÈÔ ÀÂÚÛ›ÓÈ·, ∞ÂÚÔÌÔÓ¿‰·, Clostridium difficile, E. coli 0157:H7 ∞ÓÙÈÁfiÓÔ (ƒfiÙ·-Èfi˜, §¿Ì‚ÏÈ·), ∆ÔÍ›ÓË (Clostridium difficile)

‚·ÚÈ¿˜ ÌÔÚÊ‹˜ ‰È¿ÚÚÔÈ· ÒÛÙ ӷ Â›Ó·È ·Ó·Áη›· Ë ÂÈÛ·ÁˆÁ‹ ÙÔ˘˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô. ∆¤ÏÔ˜, ηÏÏȤÚÁÂȘ ÎÔÚ¿ÓˆÓ Û˘ÓÈÛÙÒÓÙ·È ÁÈ· ÙÔ˘˜ Û˘Ìو̷ÙÈÎÔ‡˜ ·ÛıÂÓ›˜ Û ÂÚÈÙÒÛÂȘ ÂȉËÌÈÒÓ Û ·È‰ÈÎÔ‡˜ ÛÙ·ıÌÔ‡˜, ÓÔÛÔÎÔÌ›· ‹ ¿ÏÏ· ȉڇ̷ٷ fiÔ˘ ·Ú·ÙËÚÂ›Ù·È Û˘Á¯ÚˆÙÈÛÌfi˜. ∏ ηÏÏȤÚÁÂÈ· ÎÔÚ¿ÓˆÓ ı· Ú¤ÂÈ ¿ÓÙ· Ó· ÚÔËÁÂ›Ù·È Ù˘ Ï‹„˘ ·ÓÙÈ‚ÈÔÙÈÎÔ‡. ∏ ‰ÈÂÚ‡ÓËÛË ·Ú¯Èο ÛÙÚ¤ÊÂÙ·È ÛÙ· ÈÔ ÎÔÈÓ¿ ·›ÙÈ· ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ Ì ÙȘ Û˘Ó‹ıÂȘ ηÏÏȤÚÁÂȘ ÎÔÚ¿ÓˆÓ ÁÈ· Û·ÏÌÔÓ¤ÏÏ·, ÛÈÁΤÏÏ·, ηÌ˘ÏÔ‚·ÎÙËÚ›‰ÈÔ, ÙËÓ ·Ú·ÛÈÙÔÏÔÁÈ΋ ÂͤٷÛË Î·È ÙËÓ ·Ó›¯Ó¢ÛË ÙÔ˘ ·ÓÙÈÁfiÓÔ˘ ÙÔ˘ ÚfiÙ·-ÈÔ‡. ∆Ô ÈÛÙÔÚÈÎfi ı· ηÙ¢ı‡ÓÂÈ ÙË ‰ÈÂÚ‡ÓËÛË Û ÈÔ ÂȉÈΤ˜ ÂÍÂÙ¿ÛÂȘ. √ ›Ó·Î·˜ 4 ‰Â›¯ÓÂÈ ÙȘ ÂÚÁ·ÛÙËÚȷΤ˜ ÂÍÂÙ¿ÛÂȘ ÎÔÚ¿ÓˆÓ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙË ‰È¿ÁÓˆÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜. ∞ÚÎÂÙ¤˜ ÊÔÚ¤˜ Â›Ó·È ¯Ú‹ÛÈÌÔ ·fi ‰È·ÁÓˆÛÙÈ΋˜ ÛÎÔÈ¿˜ Ó· ‰È·ÎÚÈı› Ë ‰È¿ÚÚÔÈ· Û ÔÛ̈ÙÈ΋ ‹ ÂÎÎÚÈÙÈ΋. ∏ ÔÛ̈ÙÈ΋ ‰È¿ÚÚÔÈ· ÛÙ·Ì·Ù¿ fiÙ·Ó Ë Û›ÙÈÛË ‰È·ÎÔ›, ÂÓÒ ·ÓÙ›ıÂÙ· Ë ÂÎÎÚÈÙÈ΋ ·Ú·Ì¤ÓÂÈ ·ÎfiÌË Î·È ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ Û›ÙÈÛ˘. ™ÙË ‰È¿ÎÚÈÛË ·˘Ù‹ ‚ÔËı¿ Ë Ì¤ÙÚËÛË ÙˆÓ ËÏÂÎÙÚÔÏ˘ÙÒÓ ÛÙ· ÎfiÚ·Ó· (Riedel, 1995 #12). ∏ ÂÎÎÚÈÙÈ΋ ‰È¿ÚÚÔÈ· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ˘„ËÏ‹ ÂÚÈÂÎÙÈÎfiÙËÙ· ÙˆÓ ÎÔÚ¿ÓˆÓ Û ӿÙÚÈÔ, Û˘Ó‹ıˆ˜ ˘„ËÏfiÙÂÚË ÙˆÓ 50 mEq/L Î·È ¯·ÌËÏfi ÔÛ̈ÙÈÎfi ¯¿ÛÌ· ÎÔÚ¿ÓˆÓ, ÌÈÎÚfiÙÂÚÔ ÙˆÓ 50 mOsm. To ÔÛ̈ÙÈÎfi ¯¿ÛÌ· ÎÔÚ¿ÓˆÓ ˘ÔÏÔÁ›˙ÂÙ·È ˆ˜ ÂÍ‹˜: ÔÛ̈ÙÈÎfi ¯¿ÛÌ· ÎÔÚ¿ÓˆÓ = 290 – 2 x (mEq Na ÎÔÚ¿ÓˆÓ + mEq K ÎÔÚ¿ÓˆÓ). ∞ÓÙ›ıÂÙ·, ÛÙËÓ ÔÛ̈ÙÈÎÔ‡ Ù‡Ô˘ ‰È¿ÚÚÔÈ· ÙÔ ÔÛ̈ÙÈÎfi ¯¿ÛÌ· ÎÔÚ¿ÓˆÓ Â›Ó·È ·˘ÍË̤ÓÔ, Û˘Ó‹ıˆ˜ ÌÂÁ·Ï‡ÙÂÚÔ ÙÔ˘ 100 mOsm, ÁÈ·Ù› Ù· ÌË ÚÔÛ‰ÈÔÚÈ˙fiÌÂÓ· ÔÛ̈ÙÈÎÒ˜ ÂÓÂÚÁ¿ ÌfiÚÈ· ·˘Í¿ÓÔ˘Ó ÙËÓ ÔÛ̈ÙÈÎfiÙËÙ· ÙˆÓ ÎÔÚ¿ÓˆÓ. ∏ÏÂÎÙÚÔχÙ˜ ÔÚÔ‡, ‰ÈÙÙ·ÓıÚ·ÎÈο, Ô˘Ú›· Î·È ÎÚ·ÙÈÓ›ÓË ı· Ú¤ÂÈ Ó· ·Ú·ÁÁ¤ÏÏÔÓÙ·È ÁÈ· οıÂ

·È‰› Ì ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛË. ∂›Û˘, ‰ÂÓ ı· Ú¤ÂÈ Ó· ·Ú·Ï›ÂÙ·È Ë ÁÂÓÈ΋ Ô‡ÚˆÓ, ·ÊÔ‡ ÔÈ Ô˘ÚÔÏÔÈÌÒÍÂȘ ÌÔÚ› Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ‰È¿ÚÚÔÈ·. £ÂÚ·›· ∂Ó˘‰¿ÙˆÛË: √ ÚˆÙ·Ú¯ÈÎfi˜ ÛÎÔfi˜ Ù˘ ıÂÚ·›·˜ Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ·ÔÛÎÔ› ÛÙËÓ ·Ó·Ï‹ÚˆÛË ÙÔ˘ ÂÏÏ›ÌÌ·ÙÔ˜ ‡‰·ÙÔ˜ Î·È ËÏÂÎÙÚÔÏ˘ÙÒÓ Î·È ÛÙË ‰È·Ù‹ÚËÛË Ù˘ ÂÓ˘‰¿ÙˆÛ˘ fiÛÔ Ë ‰È¿ÚÚÔÈ· Û˘Ó¯›˙ÂÙ·È (13). ∏ ÂÓ‰ÔÊϤ‚È· ÂÓ˘‰¿ÙˆÛË ÂӉ›ÎÓ˘Ù·È ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛË, ·Ú·Ï˘ÙÈÎfi ÂÈÏÂfi, ·Î·Ù¿Û¯ÂÙÔ˘˜ Â̤ÙÔ˘˜. ™ÙÔ˘˜ ˘fiÏÔÈÔ˘˜ ÌÔÚÔ‡Ó Ó· ‰ÔıÔ‡Ó ·fi ÙÔ ÛÙfiÌ· ηٿ ÚÔÙ›ÌËÛË ÈÛfiÙÔÓ· ‹ ˘fiÙÔÓ· ‰È·Ï‡Ì·Ù· ˘‰·Ù·ÓıÚ¿ÎˆÓ Ô˘ ÂÚȤ¯Ô˘Ó ËÏÂÎÙÚÔχÙ˜. ∏ ‚¿ÛË Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ ‰È·Ï˘Ì¿ÙˆÓ ·˘ÙÒÓ ÛÙËÚ›˙ÂÙ·È ÛÙË Û˘Ó‰Â‰Â̤ÓË ÌÂÙ·ÊÔÚ¿ Ó·ÙÚ›Ô˘ Î·È ÁÏ˘Îfi˙˘ ‰È· ̤ÛÔ˘ ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ÂÈıËÏ›Ô˘. √ Ì˯·ÓÈÛÌfi˜ ·˘Ùfi˜ ‰È·ÙËÚÂ›Ù·È ÂÓ Ì¤ÚÂÈ ·ÎfiÌË Î·È ÛÙȘ ÂÚÈÙÒÛÂȘ Ô˘ ÙÔ Î‡ÙÙ·ÚÔ ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘ ¤¯ÂÈ ˘ÔÛÙ› ÛËÌ·ÓÙÈ΋ ‚Ï¿‚Ë, fiˆ˜ .¯. ÛÙË Ïԛ̈ÍË ·fi ÚfiÙ·-Èfi. ∏ ̤ÁÈÛÙË ·ÔÚÚfiÊËÛË ‡‰·ÙÔ˜ Î·È ËÏÂÎÙÚÔÏ˘ÙÒÓ ·fi ÙÔ ¤ÓÙÂÚÔ ÂÈÙÂÏÂ›Ù·È fiÙ·Ó o ÌÔÚÈ·Îfi˜ ÏfiÁÔ˜ ˘‰·Ù·ÓıÚ¿ÎˆÓ ÚÔ˜ Ó¿ÙÚÈÔ ÛÙÔ ‰È¿Ï˘Ì· ÂÓ˘‰¿ÙˆÛ˘ ‰ÂÓ ˘ÂÚ‚·›ÓÂÈ ÙÔ 2:1 (14). ∆Ô ‰È¿Ï˘Ì· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· Ô˘ Û˘ÓÈÛÙ¿ Ë ¶·ÁÎfiÛÌÈ· √ÚÁ¿ÓˆÛË ÀÁ›·˜ (¶√À) Î·È Ë UNICEF ¤¯ÂÈ ÌÔÚÈ·Îfi ÏfiÁÔ ˘‰·Ù·ÓıÚ¿ÎˆÓ ÚÔ˜ Ó¿ÙÚÈÔ 1,4:1 Î·È Û˘ÁΤÓÙÚˆÛË Ó·ÙÚ›Ô˘ 90 mmol/L ÂÓÒ ·˘Ù¿ Ô˘ Û˘ÓÈÛÙ¿ Ë ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ ¤¯Ô˘Ó ÏfiÁÔ ÌÈÎÚfiÙÂÚÔ ÙÔ˘ 2:1 Î·È Û˘ÁΤÓÙÚˆÛË Ó·ÙÚ›Ô˘ ÌÂÁ·Ï‡ÙÂÚË ÙˆÓ 55 mmol/L. ¢˘ÛÙ˘¯Ò˜, ·ÎfiÌË Î·È Û‹ÌÂÚ· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ú¿ÍË ˘ÁÚ¿ ÁÈ· ÂÓ˘‰¿ÙˆÛË (ÙÛ¿˚, ÊÚÔ˘ÙÔ¯˘ÌÔ›, Coca Cola) Ô˘ ÂÚȤ¯Ô˘Ó ¯·ÌËÏ‹ Û˘ÁΤÓÙÚˆÛË

9


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 10

¶·È‰È·ÙÚÈ΋ 2001;64:4-13

Paediatriki 2001;64:4-13

Ó·ÙÚ›Ô˘ Î·È ·˘ÍË̤ÓË Û˘ÁΤÓÙÚˆÛË ·ÏÒÓ Û·Î¯¿ÚˆÓ Ô˘ ÌÔÚÔ‡Ó Ó· Ô‰ËÁ‹ÛÔ˘Ó Û ˘ÔÓ·ÙÚÈ·ÈÌ›· Î·È Âȉ›ӈÛË Ù˘ ‰È¿ÚÚÔÈ·˜ ÂÍ·ÈÙ›·˜ Ù˘ ˘„ËÏ‹˜ ÙÔ˘˜ ÔÛ̈ÙÈÎfiÙËÙ·˜. √ ›Ó·Î·˜ 5 ‰Â›¯ÓÂÈ ÙË Û‡ÓıÂÛË ‰È·Ï˘Ì¿ÙˆÓ Ô˘ ÛˆÛÙ¿ ‹ Ï¿ıÔ˜ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙË ıÂÚ·›· Ù˘ ‰È¿ÚÚÔÈ·˜. ŒÓ· ÛËÌ·ÓÙÈÎfi ÌÂÈÔÓ¤ÎÙËÌ· ÙˆÓ ‰È·Ï˘Ì¿ÙˆÓ ÂÓ˘‰¿ÙˆÛ˘ Ô˘ ÂÚȤ¯Ô˘Ó ÁÏ˘Îfi˙Ë Î·È ËÏÂÎÙÚÔχÙ˜ Â›Ó·È fiÙÈ ‰ÂÓ ÂÚÈÔÚ›˙Ô˘Ó ÙË Û˘¯ÓfiÙËÙ· ‹ ÙÔÓ fiÁÎÔ ÙˆÓ ÎÔÚ¿ÓˆÓ Ô‡Ù ÌÂÈÒÓÔ˘Ó ÙË ‰È¿ÚÎÂÈ· Ù˘ ‰È¿ÚÚÔÈ·˜. °È· ÙÔ ÏfiÁÔ ·˘Ùfi, ¤¯Ô˘Ó ‰ÔÎÈÌ·ÛÙ› ‰È·Ï‡Ì·Ù· ÂÓ˘‰¿ÙˆÛ˘ Ô˘ ÂÚȤ¯Ô˘Ó ‰ËÌËÙÚȷο ‹ fiÛÚÈ·. ∞fi ·˘Ù¿ Ù· ηχÙÂÚ· ÌÂÏÂÙË̤ӷ Â›Ó·È ·˘Ù¿ Ô˘ ÂÚȤ¯Ô˘Ó Ú‡˙È (50 gr/L)

·ÓÙ› ÁÈ· ÁÏ˘Îfi˙Ë (15). ∆· ‰È·Ï‡Ì·Ù· ·˘Ù¿ Ê·›ÓÂÙ·È fiÙÈ ÌÂÈÒÓÔ˘Ó ÙÔÓ fiÁÎÔ ÙˆÓ ÎÔÚ¿ÓˆÓ Î·È ÙË ‰È¿ÚÎÂÈ· Ù˘ ‰È¿ÚÚÔÈ·˜. √È Û‡ÓıÂÙÔÈ ˘‰·Ù¿ÓıڷΘ Ô˘ ÂÚȤ¯Ô˘Ó .¯. ¿Ì˘ÏÔ, ˘‰ÚÔχÔÓÙ·È ·fi Ù· ¤Ó˙˘Ì· ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ·˘ÏÔ‡ Û ÌÈÎÚfiÙÂÚ· ÌfiÚÈ· Ù· ÔÔ›· ·Ú·Ï·Ì‚¿ÓÔÓÙ·È Ôχ ÁÚ‹ÁÔÚ· ·fi Ù· ÂÈÊ·ÓÂȷο ·ÙÙ·Ú· ÙÔ˘ ÂÓÙÂÚÈÎÔ‡ ‚ÏÂÓÓÔÁfiÓÔ˘ ÚÈÓ ·˘ÍËı› ÛËÌ·ÓÙÈο Ë ÔÛ̈ÙÈÎfiÙËÙ· ̤۷ ÛÙÔÓ ÂÓÙÂÚÈÎfi ·˘Ïfi. °È· ÙÔ Ú‡˙È ¤¯ÂÈ ‚ÚÂı› fiÙÈ ·Ó·ÛÙ¤ÏÏÂÈ ÙËÓ ÂÎÎÚÈÙÈ΋ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ÂÓÙÂÚÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì¤Ûˆ Ù˘ ‰Ú¿Û˘ ÙÔ˘ ÛÙËÓ Î˘ÎÏÈ΋ ÌÔÓÔʈÛÊÔÚÈ΋ ·‰ÂÓÔÛ›ÓË (cAMP)(16). ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ ÔÛfiÙËÙ· ÙˆÓ ¯ÔÚËÁÔ‡ÌÂ-

¶›Ó·Î·˜ 5. ™‡ÓıÂÛË ˘ÁÚÒÓ Ô˘ ÛˆÛÙ¿ ‹ Ï·Óı·Ṳ̂ӷ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ÂÓ˘‰¿ÙˆÛË. ¢È¿Ï˘Ì·

À‰·Ù¿ÓıڷΘ (g/L)

¡¿ÙÚÈÔ (mmol/L)

∫¿ÏÈÔ (mmol/L)

µ¿ÛË (‰ÈÙÙ·ÓıÚ·ÎÈÎfi ‹ ÎÈÙÚÈÎfi)

√Û̈ÙÈÎfiÙËÙ· (mOsm/L)

¢È¿Ï˘Ì· ¶√À/UNICEF

20 (ÁÏ˘Îfi˙Ë)

90

20

30

310

Orisel (NUTRICIA)

20 (ÁÏ˘Îfi˙Ë)

60

25

10

269

Almora (ELPEN)

20 (ÁÏ˘Îfi˙Ë)

35

13,5

18

Soparyx (FARMANIC)

30 (¿Ï¢ÚÔ Ú˘˙ÈÔ‡)

60

20

10

140

Rizolyte (UNI PHARM)

30 (¿Ì˘ÏÔ Ú˘˙ÈÔ‡)

60

20

10

200

ElectroRice (PANAX)

30 (¿Ì˘ÏÔ Ú˘˙ÈÔ‡)

60

20

10

140

Gatorade

45 (ÁÏ˘Îfi˙Ë, ÊÚÔ˘ÎÙfi˙Ë, ÛÔ˘ÎÚfi˙Ë)

20

3

3

330

Ã˘Ìfi˜ Ì‹ÏÔ˘

100-150 (ÁÏ˘Îfi˙Ë, ÊÚÔ˘ÎÙfi˙Ë)

3

20

0

700

Cola

50-150 (ÁÏ˘Îfi˙Ë, ÊÚÔ˘ÎÙfi˙Ë)

2

0,1

13

550

∆Û¿È

0

0

0

0

0

∑ˆÌfi˜ ·fi ÎÔÙfiÔ˘ÏÔ

0

250

5

5

450

∏ ÔÛ̈ÙÈÎfiÙËÙ· ÙÔ˘ Almora ‰ÂÓ ·Ó·Ê¤ÚÂÙ·È. Ã˘Ìfi˜ Ì‹ÏÔ˘, Cola, ÙÛ¿È, ˙ˆÌfi˜ ·fi ÎÔÙfiÔ˘ÏÔ ·Ó·‰ÂÈÎÓ‡ÔÓÙ·È ÌfiÓÔ Î·È ÌfiÓÔ ÁÈ· Û‡ÁÎÚÈÛË. ¢ÂÓ Û˘ÓÈÛÙÒÓÙ·È ÁÈ· ÂÓ˘‰¿ÙˆÛË.

10


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 11

¶·È‰È·ÙÚÈ΋ 2001;64:4-13

ÓˆÓ ˘ÁÚÒÓ, ÁÈ· ÙËÓ ‹È· ·Ê˘‰¿ÙˆÛË (3-5%) Û˘ÓÈÛÙ¿Ù·È Ë ¯ÔÚ‹ÁËÛË 50 ml/kg ‚¿ÚÔ˘˜ ÂÓ˘‰·ÙÈÎÔ‡ ‰È·Ï‡Ì·ÙÔ˜ Û ‰È¿ÛÙËÌ· 6 ˆÚÒÓ. °È· ÌÂÙÚ›Ô˘ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛË (5-9%) Û˘ÓÈÛÙ¿Ù·È Ë ¯ÔÚ‹ÁËÛË 100 ml/kg ‚¿ÚÔ˘˜ ‰È·Ï‡Ì·ÙÔ˜ ÛÙÔ ›‰ÈÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·. ∆¤ÏÔ˜, ÁÈ· ÙË ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛË (10% Î·È ¿Óˆ) Û˘ÓÈÛÙ¿Ù·È Ë ·Ú¯È΋ ·Ó·˙ˆÔÁfiÓËÛË Ì ÂÓ‰ÔÊϤ‚È· ˘ÁÚ¿ (20-60 ml/kg) ÛÙȘ ÚÒÙ˜ ‰‡Ô ÒÚ˜ ̤¯ÚÈ Ó· ·ÔηٷÛÙ·ıÔ‡Ó Ù· ˙ˆÙÈο ÛËÌ›· Î·È Ô Ú˘ıÌfi˜ Ô‡ÚËÛ˘. ™ÙË Û˘Ó¤¯ÂÈ· ÌÔÚÔ‡Ó Ó· ‰ÔıÔ‡Ó ‰È·Ï‡Ì·Ù· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ·. √È Û˘Ó¯È˙fiÌÂÓ˜ ·ÒÏÂȘ ˘ÁÚÒÓ ÌÔÚÔ‡Ó Ó· ·Ó·ÏËÚÒÓÔÓÙ·È Ì ÙË ¯ÔÚ‹ÁËÛË 10 ml/kg ‚¿ÚÔ˘˜ ÂÓ˘‰·ÙÈÎÔ‡ ‰È·Ï‡Ì·ÙÔ˜ ÁÈ· οı ‰È·ÚÚÔ˚΋ ΤӈÛË. ŸÌˆ˜, ·ÚfiÏÔ Ô˘ Ù· ‰È·Ï‡Ì·Ù· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· ¤¯Ô˘Ó ÛÒÛÂÈ ÂηÙÔÌ̇ÚÈ· ˙ˆ¤˜ ÛÙÔÓ ÙÚ›ÙÔ ÎfiÛÌÔ, Ë ¯ÚËÛÈÌÔÔ›ËÛ‹ ÙÔ˘˜ ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜ Â›Ó·È Î·ÙÒÙÂÚË ÙÔ˘ ·Ó·ÌÂÓfiÌÂÓÔ˘. ¶ÔÏϤ˜ ÊÔÚ¤˜ ÚÔÙÈÌ¿Ù·È Ë ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ˘ÁÚÒÓ ·ÎfiÌË Î·È Û ÂÚÈÙÒÛÂȘ ̤ÙÚÈ·˜ ·Ê˘‰¿ÙˆÛ˘. ªÂϤÙË Ù˘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ Û ·È‰È¿ ËÏÈΛ·˜ ̤¯ÚÈ 3 ÂÙÒÓ Ô˘ ÓÔÛËχıËÎ·Ó ÁÈ· ÔÍ›· Á·ÛÙÚÂÓÙÂÚ›Ùȉ· ¤‰ÂÈÍ fiÙÈ ÛÙÔ 52% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ¯ÔÚËÁ‹ıËÎ·Ó ÂÓ‰ÔÊÏ‚›ˆ˜ ˘ÁÚ¿ ¯ˆÚ›˜ Ó· ˘¿Ú¯ÂÈ ¤Ó‰ÂÈÍË (17). ∆· ÌÂÈÔÓÂÎÙ‹Ì·Ù· ÙˆÓ ‰È·Ï˘Ì¿ÙˆÓ ÂÓ˘‰¿ÙˆÛ˘ Â›Ó·È Ë ··›ÙËÛË Ù˘ Û˘Ó¯ԇ˜ ·ÚÔ˘Û›·˜ ÙÔ˘ ÁÔÓ¤· ÛÙÔ Ï¢Úfi ÙÔ˘ ·È‰ÈÔ‡ ÁÈ· Ó· Ù· ¯ÔÚËÁ› ηıÒ˜ Î·È Ë Á‡ÛË ÙÔ˘˜, ·ÊÔ‡ Û ÔÏÏ¿ ·È‰È¿ ‰ÂÓ Â›Ó·È ·ÚÂÛÙ‹. ¶ÏÂÔÓÂÎÙ‹Ì·Ù· ·ÔÙÂÏÔ‡Ó Ë È‰·ÓÈÎfiÙÂÚË ·Ó·Ï‹ÚˆÛË ÙˆÓ ˘ÁÚÒÓ ·fi ÏÂ˘Ú¿˜ Ê˘ÛÈÔÏÔÁ›·˜, Ë ·ÔÊ˘Á‹ Ù˘ ÂÈÛ·ÁˆÁ‹˜ ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô Î·È ÙÔ ÌÈÎÚfiÙÂÚÔ ÎfiÛÙÔ˜. ™›ÙÈÛË: ∏ Û›ÙÈÛË ı· Ú¤ÂÈ Ó· ·Ú¯›ÛÂÈ ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙËÓ ÂÓ˘‰¿ÙˆÛË. ∏ ·Ï·È¿ ·ÓÙ›ÏË„Ë fiÙÈ Ë Û›ÙÈÛË Â›Ó·È ÚÔÙÈÌfiÙÂÚÔ Ó· ηı˘ÛÙÂÚ› ÁÈ· Ó· ‰Ôı› οÔÈÔ˜ ¯ÚfiÓÔ˜ ·Ó¿·˘Û˘ ÛÙÔ «ÙÚ·˘Ì·ÙÈṲ̂ÓÔ» ¤ÓÙÂÚÔ, ÙÔ ÔÔ›Ô ‰˘Û·ÔÚÚÔÊ¿ Î·È Û˘ÓÂÒ˜ ÂÈÙ›ÓÂÈ ÙË ‰È¿ÚÚÔÈ·, ‰ÂÓ Â˘ÛÙ·ı›. ∞ÓÙ›ıÂÙ·, ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ ÙÔ 80 Ì 95% ÙˆÓ ˘‰·Ù·ÓıڿΈÓ, ÙÔ 70% ÙÔ˘ Ï›Ô˘˜ Î·È ÙÔ 75% Ù˘ ÚˆÙ½Ó˘ ·ÔÚÚÔÊÔ‡ÓÙ·È (18). ∏ ÚfiÛÏË„Ë ÙÚÔÊ‹˜ ·fi ÙÔ ¤ÓÙÂÚÔ ‚ÔËı¿ ÛÙËÓ ÂԇψÛË ÙÔ˘ ÙÚ·˘Ì·ÙÈṲ̂ÓÔ˘ ‚ÏÂÓÓÔÁfiÓÔ˘, ÂÓÒ Ë ·ÛÈÙ›· Ô‰ËÁ› ÛÙËÓ ˘ÔÏ·Û›· ÙÔ˘ Î·È ÛÙË ÌÂȈ̤ÓË ‰Ú·ÛÙÈÎfiÙËÙ· ÙˆÓ ÂÙÈÎÒÓ ÂÓ˙‡ÌˆÓ. ŸÙ·Ó ÙÔ ·È‰› ·ÚÁ‹ÛÂÈ Ó· ÛÈÙÈÛÙ› ¤¯ÂÈ Û˘¯Ó¤˜, ÌÈÎÚÔ‡ fiÁÎÔ˘, Î·È ‚ÏÂÓÓÒ‰ÂȘ ÎÂÓÒÛÂȘ (ÎÂÓÒÛÂȘ «›ӷ˜»). ™Ù· ‚Ú¤ÊË Ô˘ ıËÏ¿˙Ô˘Ó, Ô ÌËÙÚÈÎfi˜ ıËÏ·ÛÌfi˜ ı· Ú¤ÂÈ Ó· ·Ú¯›ÛÂÈ ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙËÓ ÂÓ˘‰¿ÙˆÛË. ™Ù· ‚Ú¤ÊË Ô˘ ‰ÂÓ ıËÏ¿˙Ô˘Ó ÌÔÚ› Ó· ‰Ôı› ÙÔ ÎÔÓÈÔÔÈË̤ÓÔ Á¿Ï· Ô˘ ¤·ÈÚÓ·Ó ÚÔËÁÔ‡ÌÂÓ· ¯ˆÚ›˜ ·Ú·›ˆÛË (15). ∏ ¯ÔÚ‹ÁËÛË ·Ú·ÈˆÌ¤ÓÔ˘ Á¿Ï·ÎÙÔ˜, fiˆ˜ Û˘¯Ó¿ Á›ÓÂÙ·È

Paediatriki 2001;64:4-13

ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ú¿ÍË, ‰ÂÓ Û˘ÓÈÛÙ¿Ù·È ÁÈ·Ù› Ù· ‚Ú¤ÊË ˘ÔÛÈÙ›˙ÔÓÙ·È Ì¤¯ÚÈ Ó· ÙÔ˘˜ ‰Ôı› ÙÔ Ï‹Ú˜ Á¿Ï· (19). ∆Ô Á¿Ï· ÂχıÂÚÔ Ï·ÎÙfi˙˘ ‰ÂÓ ı· Ú¤ÂÈ Ó· ‰›ÓÂÙ·È ÂÍ·Ú¯‹˜, ÁÈ·Ù› ÔÏϤ˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ ÛÙËÓ ÔÍ›· ‰È¿ÚÚÔÈ· ÙˆÓ ‚ÚÂÊÒÓ ‰ÂÓ ˘¿Ú¯ÂÈ ÛËÌ·ÓÙÈÎÔ‡ ‚·ıÌÔ‡ ‰˘Û·ÓÂÍ›· ÛÙË Ï·ÎÙfi˙Ë. °¿Ï· ÂχıÂÚÔ Ï·ÎÙfi˙˘ (Á¿Ï· ·ÁÂÏ¿‰·˜ ‹ ÛfiÁÈ·˜) Û˘ÓÈÛÙ¿Ù·È fiÙ·Ó ÔÈ ·ÛıÂÓ›˜ Â›Ó·È ÛÔ‚·Ú¿ ·Ê˘‰·ÙˆÌ¤ÓÔÈ Î·Ù¿ ÙËÓ ÚÒÙË Â›Û΄Ë, ¤¯Ô˘Ó ÎÏÈÓÈο ÛËÌ›· ˘ÔÛÈÙÈÛÌÔ‡ ‹ Ë ‰È¿ÚÚÔÈ· ÙÔ˘˜ ¯ÂÈÚÔÙÂÚ‡ÂÈ ÂÌÊ·ÓÒ˜ Ì ÙËÓ Â·ÓÂÈÛ·ÁˆÁ‹ Ù˘ Ï·ÎÙfi˙˘ (19). ∆Ô ÊÔÚÙ›Ô Ù˘ Ï·ÎÙfi˙˘ ÚÔ˜ ÙÔ ÏÂÙfi ¤ÓÙÂÚÔ ÌÔÚ› Ó· ÂÏ·ÙÙˆı› Ì ÙË ¯Ú‹ÛË Á¿Ï·ÎÙÔ˜ Ô˘ ¤¯ÂÈ ˘ÔÛÙ› ˙‡ÌˆÛË ‹ ¤¯ÂÈ ÚÔÛÙÂı› ÙÔ ¤Ó˙˘ÌÔ ‚-Á·Ï·ÎÙÔÛȉ¿ÛË fiˆ˜ Û˘Ì‚·›ÓÂÈ Ì ÙÔ ÁÈ·Ô‡ÚÙÈ. £· Ú¤ÂÈ Ó· ÛËÌÂȈı› fiÙÈ Ë Û˘Ó¤¯ÈÛË Ù˘ ‰›·ÈÙ·˜ Ô˘ ÂÚȤ¯ÂÈ Ï·ÎÙfi˙Ë ÌÔÚ› Ó· Û˘Óԉ‡ÂÙ·È ·fi ÌÈÎÚÔ‡ ‚·ıÌÔ‡ ·‡ÍËÛË ÙˆÓ ÎÂÓÒÛÂˆÓ Î·È ·Ú¿Ù·ÛË Ù˘ ‰È¿ÚÚÔÈ·˜ Û˘ÁÎÚÈÓfiÌÂÓË Ì ÌÈ· ‰›·ÈÙ· ÂχıÂÚË Ï·ÎÙfi˙˘ (20). ∞˘Ùfi ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ ·Ó˘ÔÌÔÓËÛ›· ÙˆÓ ÁÔÓ¤ˆÓ οÓÂÈ ÙÔ˘˜ ·È‰›·ÙÚÔ˘˜ ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜ Ó· ÚÔ¯ˆÚÔ‡Ó ÛÙÔÓ ÚfiˆÚÔ ‰È·ÈÙËÙÈÎfi ·ÔÎÏÂÈÛÌfi Ù˘. ™Â ÂÚÈÙÒÛÂȘ ÛÔ‚·Ú‹˜ ‚Ï¿‚˘ ÙÔ˘ ÂÈÊ·ÓÂÈ·ÎÔ‡ ÂÓÙÂÚÈÎÔ‡ ÂÈıËÏ›Ô˘ Ô˘ Ë ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ ÂÙÈÎÒÓ ÂÓ˙‡ÌˆÓ ¤¯ÂÈ ÌÂȈı› ηٿ Ôχ, fiˆ˜ Û˘Ì‚·›ÓÂÈ ÛÙȘ ÂÚÈÙÒÛÂȘ ÌÂÙ·ÏÔÈÌÒ‰Ô˘˜ ÂÓÙÂÚ›Ùȉ·˜, Ô ·ÛıÂÓ‹˜ ÌÔÚ› Ó· ˆÊÂÏËı› ·fi ÙË ¯ÔÚ‹ÁËÛË ÛÙÔȯÂÈ·ÎÒÓ Á·Ï¿ÙˆÓ, ÂχıÂÚˆÓ Ï·ÎÙfi˙˘ Ì ˘‰ÚÔÏ˘Ì¤ÓË ÚˆÙ½ÓË. ∂Âȉ‹ Ù· Á¿Ï·Ù· ·˘Ù¿ ¤¯Ô˘Ó Û˘Ó‹ıˆ˜ ˘„ËÏ‹ ÔÛ̈ÙÈÎfiÙËÙ· Ô˘ ÌÔÚ› Ó· ÂȉÂÈÓÒÛÂÈ ÙË ‰È¿ÚÚÔÈ·, ı· Ú¤ÂÈ Ó· ÂÈÏÂÁ› ·˘Ùfi Ì ÙË ÌÈÎÚfiÙÂÚË ‰˘Ó·Ù‹ ÔÛ̈ÙÈÎfiÙËÙ· (19). °È· Ù· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿, Ë ∞ÌÂÚÈηÓÈ΋ ∞η‰ËÌ›· ¶·È‰È·ÙÚÈ΋˜ Û˘ÓÈÛÙ¿ ÙËÓ ¤ÁηÈÚË Â·Ó·Û›ÙÈÛË Ì ÌÈ· ÈÛÔÚÚÔË̤ÓË ‰›·ÈÙ· ‚·ÛÈṲ̂ÓË Û ÙÚÔʤ˜ Ô˘ ÂÚȤ¯Ô˘Ó Û‡ÓıÂÙÔ˘˜ ˘‰·Ù¿ÓıڷΘ (Ú‡˙È, ‰ËÌËÙÚȷο, „ˆÌ›, ·Ù¿Ù˜), „·¯Ófi ÎÚ¤·˜, Ì·ÁÂÈÚÂ̤ӷ ÊÚÔ‡Ù· Î·È Ï·¯·ÓÈο, ÁÈ·Ô‡ÚÙÈ Î·È ·ÔÊ˘Á‹ ÙÚÔÊÒÓ ¯·ÌËÏÒÓ Û ϛË Î·È ·Ï¿ ۿί·Ú· (¤ÙÔÈÌÔÈ ¯˘ÌÔ› ÊÚÔ‡ÙˆÓ, ·Ó·„˘ÎÙÈο) (15). ∞ÓÙÈ‚ÈÔÙÈο: ∆· ·ÓÙÈ‚ÈÔÙÈο Û¿ÓÈ· ÂӉ›ÎÓ˘ÓÙ·È ÛÙË ıÂÚ·›· Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜, ·ÊÔ‡ ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ë ÓfiÛÔ˜ ·Ô‰Ú¿ÌÂÈ ·fi ÌfiÓË Ù˘ (21). ™Â ÂÚ›ÙˆÛË ·ÓÙÈ‚ÈÔÙÈ΋˜ ·ÁˆÁ‹˜ ηÏfi ı· Â›Ó·È Ó· Á›ÓÂÙ·È ·ÓÙÈ‚ÈfiÁÚ·ÌÌ·, ηıÒ˜ ¤¯ÂÈ ·Ú·ÙËÚËı› ·Ó¿Ù˘ÍË ·ÓıÂÎÙÈÎÒÓ ÛÙÂϯÒÓ. ™ÙË Ïԛ̈ÍË ·fi ÛÈÁΤÏÏ· Ù· ·ÓÙÈ‚ÈÔÙÈο Û˘ÓÙÔÌÂ‡Ô˘Ó ÙËÓ ÔÚ›· Ù˘ ÓfiÛÔ˘ Î·È ÙÔ ¯ÚfiÓÔ ·Ô‚ÔÏ‹˜ ÙÔ˘ ‚·ÎÙËÚ›Ô˘ ÛÙ· ÎfiÚ·Ó·. ∏ ÎÔÙÚÈÌÔÍ·˙fiÏË (ÙÚÈÌÂıÔÚ›ÌË - ÛÔ˘ÏÊÔÌÂıÔÍ·˙fiÏË) ·Ú·Ì¤ÓÂÈ ÙÔ Ê¿ÚÌ·ÎÔ ÂÎÏÔÁ‹˜ ÛÙȘ ·Ó·Ù˘Á̤Ó˜ ¯ÒÚ˜, ·ÏÏ¿

11


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 12

¶·È‰È·ÙÚÈ΋ 2001;64:4-13

Ë ·˘Í·ÓfiÌÂÓË ·ÓıÂÎÙÈÎfiÙËÙ· ÌÔÚ› Ó· ··ÈÙ‹ÛÂÈ ÙË ¯ÔÚ‹ÁËÛË ¿ÏÏˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ, fiˆ˜ ÔÈ ÎÂÊ·ÏÔÛÔÚ›Ó˜ ÙÚ›Ù˘ ÁÂÓ¿˜ (.¯. Ë ÎÂÊÈÍ›ÌË) Î·È ÔÈ ·ÌÈÓÔÁÏ˘ÎÔÛ›‰Â˜. ªÂÁ¿ÏË ÌÂϤÙË Ô˘ ·ÊÔÚÔ‡Û ÙË ÛÈÁΤÏψÛË Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ÛÙË µ. ∂ÏÏ¿‰· ηٿ ÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô 1971-1994 ¤‰ÂÈÍ fiÙÈ ˘‹Ú¯Â ÛËÌ·ÓÙÈ΋ ·ÓÙÔ¯‹ ÙÔ˘ ‚·ÎÙËÚ›Ô˘ ÛÙËÓ ÎÔÙÚÈÌÔÍ·˙fiÏË (24%) Î·È ÛÙËÓ ·ÌÈÎÈÏÏ›ÓË (16%) (22). ∏ ıÂÚ·›· ÂÓÙÂÚ›Ùȉ·˜ ·fi Û·ÏÌÔÓ¤ÏÏ· Û˘ÓÈÛÙ¿Ù·È ÁÈ· ÙȘ ·Ú·Î¿Ùˆ ηÙËÁÔڛ˜ ·ÛıÂÓÒÓ: ·) ·ÓÔÛÔηٷÛÙ·Ï̤ӷ ¿ÙÔÌ·, ‚) ·È‰È¿ Ì ·ÈÌÔÛÊ·ÈÚÈÓÔ¿ıÂȘ, Á) ·È‰È¿ ÌÂ Ù˘ÊÔÂȉ‹ ˘ÚÂÙfi, ‰) ‚·ÎÙËÚÈ·ÈÌ›· ·fi Û·ÏÌÔÓ¤ÏÏ·, Â) ‚Ú¤ÊË Î¿Ùˆ ÙˆÓ 3 ÌËÓÒÓ. º¿ÚÌ·ÎÔ ÚÒÙ˘ ÂÎÏÔÁ‹˜ Â›Ó·È ÔÈ ÎÂÊ·ÏÔÛÔÚ›Ó˜, ΢ڛˆ˜ ·˘Ù¤˜ Ù˘ 3˘ ÁÂÓ¿˜, ÂÓÒ Ë ·ÌÈÎÈÏÏ›ÓË Î·È Ë ÎÔÙÚÈÌÔÍ·˙fiÏË ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È fiÙ·Ó ‚ÚÂı› ¢·ÈÛıËÛ›· ÛÙÔ ·ÓÙÈ‚ÈfiÁÚ·ÌÌ·. ™Â ÌÈ· ¤Ú¢ӷ Ù˘ ∞’ ¶·È‰È·ÙÚÈ΋˜ ÎÏÈÓÈ΋˜ ÙÔ˘ ·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ (23) Ô˘ ¤ÁÈÓ ÛÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô 1993-1995 ‚Ú¤ıËΠfiÙÈ ÔÈ ÈÔ Û˘¯Ó¿ ··ÓÙÒÌÂÓ˜ Û·ÏÌÔÓ¤ÏϘ ÛÙÔÓ ÂÏÏËÓÈÎfi ¯ÒÚÔ S. typhimurium Î·È S. enteritidis ·ÚÔ˘Û›·˙·Ó ÚÔԉ¢ÙÈο ·˘Í·ÓfiÌÂÓË ·ÓÙÔ¯‹ ÛÙËÓ ·ÌÈÎÈÏÏ›ÓË Ô˘ ¤Êı·Ó·Ó ÙÔ 43 Î·È 65% ·ÓÙ›ÛÙÔȯ·, ÂÓÒ Ë S. typhimurium ÛÙËÓ ÎÔÙÚÈÌÔÍ·˙fiÏË (42%) Î·È ¯ÏˆÚ·ÌÊÂÓÈÎfiÏË (51%). ∞ÓÙ›ıÂÙ·, Ë ·ÓÙÔ¯‹ ÛÙȘ ÎÂÊ·ÏÔÛÔÚ›Ó˜ 1˘, 2˘ Î·È 3˘ ÁÂÓ¿˜ Î˘Ì¿ÓıËΠ۠Ôχ ¯·ÌËÏ¿ Â›‰· (0-5%). °È· ÙË ¯ˆÚ›˜ ÂÈÏÔΤ˜ ÂÓÙÂÚ›Ùȉ· ·fi Û·ÏÌÔÓ¤ÏÏ· ‰ÂÓ Û˘ÓÈÛÙ¿Ù·È ıÂÚ·›· ÁÈ·Ù› Ë ¯Ú‹ÛË ÙˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ÌÔÚ› Ó· ·Ú·Ù›ÓÂÈ ÙË ÊÔÚ›·. ∏ ¯ÔϤڷ ıÂÚ·‡ÂÙ·È Ì ÙÂÙڷ΢ÎϛӘ ‹ ÎÔÙÚÈÌÔÍ·˙fiÏË. √È ÂÚÈÙÒÛÂȘ ·Ú·ÙÂٷ̤Ó˘ ‹ ÛÔ‚·Ú‹˜ ‰È¿ÚÚÔÈ·˜ ·fi ηÌ˘ÏÔ‚·ÎÙËÚ›‰ÈÔ ıÂÚ·‡ÔÓÙ·È Ì ÂÚ˘ıÚÔÌ˘Î›ÓË. ∏ ·ÓÙÈ‚ÈÔÙÈ΋ ·ÁˆÁ‹ Û˘ÓÈÛÙ¿Ù·È Î·È ÁÈ· ÙË Ïԛ̈ÍË Ì ÂÓÙÂÚÔ‰ÈÂÈÛ‰˘ÙÈÎfi ‹ ÂÓÙÂÚÔÙÔÍÈÓÔÁfiÓÔ ÎÔÏÔ‚·ÎÙËÚ›‰ÈÔ. ∆Ô ÙÂÏÂ˘Ù·›Ô ·ÔÙÂÏ› Î·È ÙËÓ Î˘Ú›· ·ÈÙ›· Ù˘ ‰È¿ÚÚÔÈ·˜ ÙˆÓ Ù·ÍȉȈÙÒÓ. ∆Ô Ê¿ÚÌ·ÎÔ ÂÎÏÔÁ‹˜ Â›Ó·È Ë ÎÔÙÚÈÌÔÍ·˙fiÏË. ∆Ô ÚˆÙ·Ú¯ÈÎfi ‚‹Ì· ÛÙË ıÂÚ·›· Ù˘ ÎÔÏ›Ùȉ·˜ ·fi Clostridium difficile Â›Ó·È Ë ‰È·ÎÔ‹ ÙˆÓ ‹‰Ë ¯ÔÚËÁÔ‡ÌÂÓˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ. ∞ÓÙÈ‚ÈÔÙÈο Ô˘ ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› Ì ÂÈÙ˘¯›· ·ÔÙÂÏÔ‡Ó Ë ÌÂÙÚÔÓȉ·˙fiÏË Î·È Ë ‚·ÓÎÔÌ˘Î›ÓË ·fi ÙÔ ÛÙfiÌ·. √ ÚfiÏÔ˜ Ù˘ ·ÓÙÈ‚ÈÔÙÈ΋˜ ·ÁˆÁ‹˜ ÁÈ· ÙȘ Á·ÛÙÚÂÓÙÂÚ›Ùȉ˜ ·fi ÂÓÙÂÚÔ·ÈÌÔÚÚ·ÁÈÎfi, ÂÓÙÂÚÔÚÔÛÎÔÏÏËÙÈÎfi ÎÔÏÔ‚·ÎÙËÚ›‰ÈÔ, ·ÂÚÔÌÔÓ¿‰·, Î·È ˘ÂÚÛ›ÓÈ· ‰ÂÓ ¤¯ÂÈ ‰È·Ï¢ηÓı› Ï‹Úˆ˜. ∏ ÎÚ˘ÙÔÛÔÚȉ›·ÛË Û ÌË ·ÓÔÛÔηٷÛÙ·Ï̤ӷ ¿ÙÔÌ· ·Ô‰Ú¿ÌÂÈ ·fi ÌfiÓË Ù˘ ¯ˆÚ›˜ ıÂÚ·›·. ∏ ÌÂÙÚÔÓȉ·˙fiÏË ·ÔÙÂÏ› ÙÔ Ê¿ÚÌ·ÎÔ ÂÎÏÔÁ‹˜ ÁÈ· ÙËÓ ÂÓÙÂÚÈ΋ ·ÌÔÈ‚¿‰ˆÛË. ¶ÚÔ‚ÈÔÙÈο: ¶ÚfiÎÂÈÙ·È ÁÈ· ÌË ·ıÔÁfiÓÔ˘˜ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜ Ô˘ fiÙ·Ó Î·Ù·ÔıÔ‡Ó ÌÔÚÔ‡Ó

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Ó· ÚÔÏ¿‚Ô˘Ó ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ‰È¿ÚÚÔÈ·˜ ‹ Ó· ‚ÔËı‹ÛÔ˘Ó ÛÙË ıÂÚ·›· Ù˘ (24). ∏ ηÙËÁÔÚ›· ·˘Ù‹ ÂÚÈÏ·Ì‚¿ÓÂÈ Â›‰Ë Lactobacillus (.¯. GG Î·È acidophilus), ›‰Ë Saccharomyces (.¯. boulardii), Bifidobacterium, Streptococcus thermophilus. √È Ì˯·ÓÈÛÌÔ› ‰Ú¿Û˘ ÙÔ˘˜ Â›Ó·È ÔÈ ·ÎfiÏÔ˘ıÔÈ: ·) ·ÓÙ·ÁˆÓ›˙ÔÓÙ·È ÙËÓ ÚÔÛÎfiÏÏËÛË ·ıÔÁfiÓˆÓ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ ¿Óˆ ÛÙÔÓ ÂÓÙÂÚÈÎfi ‚ÏÂÓÓÔÁfiÓÔ, Ô˘ Â›Ó·È ··Ú·›ÙËÙË ÁÈ· ÙËÓ ÂΉ‹ÏˆÛË Ù˘ ‚Ï·ÙÈ΋˜ ‰Ú¿Û˘ ÙÔ˘˜, ‚) ·ÓÙ·ÁˆÓ›˙ÔÓÙ·È ÙÔ˘˜ ·ıÔÁfiÓÔ˘˜ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡˜ ˆ˜ ÚÔ˜ ÙËÓ ÚfiÛÏË„Ë ÙÚÔÊ‹˜ ‹ ·Ú¿ÁÔ˘Ó Ô˘Û›Â˜ Ô˘ ·Ó·ÛÙ¤ÏÏÔ˘Ó ÙËÓ ·Ó¿Ù˘Í‹ ÙÔ˘˜, Á) ¤¯Ô˘Ó ·ÓÔÛÔÙÚoÔÔÈËÙÈΤ˜ ȉÈfiÙËÙ˜, .¯ Ô Lactobacillus GG ‰ÈÂÁ›ÚÂÈ ÙËÓ ·Ú·ÁˆÁ‹ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ ÚfiÙ·-ÈÔ‡. ∆· ÚÔ‚ÈÔÙÈο Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈο ÛÙËÓ ÚfiÏË„Ë Î·È ıÂÚ·›· Ù˘ ÏÔÈÌÒ‰Ô˘˜ ‰È¿ÚÚÔÈ·˜, Ù˘ ‰È¿ÚÚÔÈ·˜ ÙˆÓ Ù·ÍȉȈÙÒÓ Î·È Ù˘ ‰È¿ÚÚÔÈ·˜ ·fi ·ÓÙÈ‚ÈÔÙÈο. ÃÚÂÈ¿˙ÔÓÙ·È fï˜ ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜ ÁÈ· Ó· ηÙÔ¯˘Úˆı› Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Î·È Ë ·ÛÊ¿ÏÂÈ¿ ÙÔ˘˜ ÌÔÏÔÓfiÙÈ ‰ÂÓ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ÙÔÍÈΤ˜ ·ÓÙȉڿÛÂȘ. µÈٷ̛Ә Î·È È¯ÓÔÛÙÔȯ›·: Y¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ ·fi ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ Ô˘ ¤Ï·‚·Ó ¯ÒÚ· Û ·Ó·Ù˘ÛÛfiÌÂÓ˜ ¯ÒÚ˜, fiÔ˘ ÂÓ‰ËÌ› Ô ˘ÔÛÈÙÈÛÌfi˜ Î·È Ë ˘Ô‚Èٷ̛ӈÛË, fiÙÈ Ë ¯ÔÚ‹ÁËÛË „¢‰·ÚÁ‡ÚÔ˘, ‚ÈÙ·Ì›Ó˘ ∞ ‹ Ê˘ÏÏÈÎÔ‡ ÔͤԘ ÌÔÚ› Ó· ÂÏ·ÙÙÒÛÂÈ ÙË Û˘¯ÓfiÙËÙ· ÙˆÓ ÂÂÈÛÔ‰›ˆÓ ÛÔ‚·Ú‹˜ ‰È¿ÚÚÔÈ·˜ ‹ fiÙ·Ó ·˘Ù‹ Â¤ÏıÂÈ Ó· ÌÂÈÒÛÂÈ ÙË ‚·Ú‡ÙËÙ· Î·È ÙË ‰È¿ÚÎÂÈ¿ Ù˘ (19). ∞˘Ù‹ ÙË ÛÙÈÁÌ‹ fï˜ Â›Ó·È Ôχ ÚfiˆÚÔ Ó· ԇ̠fiÙÈ fiÏ· Ù· ·È‰È¿ Ì ÔÍ›· ‰È¿ÚÚÔÈ· ı· Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÔ˘Ó ÙȘ Ô˘Û›Â˜ ·˘Ù¤˜. ÕÏÏ· Ê¿Ú̷η ηٿ Ù˘ ‰È¿ÚÚÔÈ·˜: ∆· Ê¿Ú̷η Ô˘ ÂÌÔ‰›˙Ô˘Ó ÙÔÓ ÂÚÈÛÙ·ÏÙÈÛÌfi ÙÔ˘ ÂÓÙ¤ÚÔ˘, fiˆ˜ Ë ÏÔÂÚ·Ì›‰Ë Î·È Ë ‰ÈÊ·ÈÓÔÍ˘Ï¿ÙË Ì ÙËÓ ·ÙÚÔ›ÓË, ‰ÂÓ ¤¯Ô˘Ó ı¤ÛË ÛÙË ıÂÚ·›· Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ÛÙ· ·È‰È¿, ÁÈ·Ù› ÌÔÚÔ‡Ó Ó· ÚÔηϤÛÔ˘Ó ÂÈÏÂfi Î·È ˘ÂÚ·Ó¿Ù˘ÍË ÙˆÓ ·ıÔÁfiÓˆÓ ÔÚÁ·ÓÈÛÌÒÓ (bacterial overgrowth). Œ¯Ô˘Ó Â›Û˘ ·Ó·ÊÂÚı› ηٷÛÙÔÏ‹ Ù˘ ·Ó·ÓÔ‹˜ Î·È ÎÒÌ· (15). º¿Ú̷η Ô˘ ÂÌÔ‰›˙Ô˘Ó ÙËÓ ·ÔÚÚfiÊËÛË, fiˆ˜ ¯ÔÏÂÛÙ˘Ú·Ì›ÓË ‹ Ì›ÁÌ· ηÔÏ›Ó˘ ËÎÙ›Ó˘, ‰ÂÓ Û˘ÓÈÛÙÒÓÙ·È Â›Û˘, ÁÈ·Ù› ÌÔÏÔÓfiÙÈ ‚ÂÏÙÈÒÓÔ˘Ó ÙË Û‡ÛÙ·ÛË ÙˆÓ ÎÔÚ¿ÓˆÓ, ÌÂÈÒÓÔ˘Ó ÙËÓ ÎÈÓËÙÈÎfiÙËÙ· ÙÔ˘ ÂÓÙ¤ÚÔ˘ Î·È ÌÔÚ› Ó· Ô‰ËÁ‹ÛÔ˘Ó Û ˘ÂÚ·Ó¿Ù˘ÍË ÙˆÓ ÌÈÎÚÔ‚›ˆÓ (13,15). ¶ÚfiÏË„Ë: ∆Ô ÚˆÙ·Ú¯ÈÎfi ̤ÏËÌ· fï˜, ·ÔÙÂÏ› Ë ÚfiÏË„Ë Ë ÔÔ›· ÌÔÚ› Ó· ÂÈÙ¢¯ı› Ì ÙËÓ Î·Ï‹ Ù‹ÚËÛË ÙˆÓ Î·ÓfiÓˆÓ ˘ÁÈÂÈÓ‹˜, ΢ڛˆ˜ ÙÔ χÛÈÌÔ ÙˆÓ ¯ÂÚÈÒÓ, ÙË ‚ÂÏÙ›ˆÛË ÙˆÓ ·Ô¯ÂÙ‡ÛˆÓ, ÙÔ ÌËÙÚÈÎfi ıËÏ·ÛÌfi, ÙËÓ ·ÔÊ˘Á‹ ÌË ·ÛÙÂÚȈ̤ÓÔ˘ Á¿Ï·ÎÙÔ˜, ÌË Â·ÚÎÒ˜ „ËÌ¤ÓˆÓ ÎÚ¿-


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ÙˆÓ, Ô˘ÏÂÚÈÎÒÓ Î·È ·ÙÂÏÒ˜ ‚Ú·ÛÌ¤ÓˆÓ (οو ÙˆÓ 6 ÏÂÙÒÓ) ·˘ÁÒÓ Î·È ÙËÓ Â·Ú΋ ηٿ„˘ÍË ÙˆÓ ÙÚÔÊÒÓ. ∏ ¤Ú¢ӷ ·Ó·Ù‡ÛÛÂÙ·È ÚÔ˜ ÙËÓ Î·Ù‡ı˘ÓÛË ÙˆÓ ÂÌ‚ÔÏ›ˆÓ Ô˘ ÚÔÊ˘Ï¿ÛÛÔ˘Ó ·fi ÙË Ïԛ̈ÍË Ì ÂÓÙÂÚÔ·ıÔÁfiÓ· ÌÈÎÚfi‚È·. µÈ‚ÏÈÔÁÚ·Ê›· 1. Riedel B, Ghishan F. Acute diarrhea. In: Walker W, Durie P, Hamilton J, Walker-Smith J, Watkins J, editors. Pediatric gastrointestinal disease; pathophysiology, diagnosis, management. Vol. 1. St Louis, MO: Mosby; 1996. p. 251. 2. Claeson M, Merson M. Global progress in the control of diarrheal diseases. Pediatr Infect Dis 1990;9:345-355. 3. Glass R, Lew J, Gangarosa R, LeBaron C, Ho MS. Estimates of morbidity and mortality for diarrheal diseases in American children. J Pediatr 1991;118:S27-S33. 4. ∫ÔÓ‰‡Ï˘ ∞, ¶Ú›ÊÙ˘ ∫, ∫·ÓÂÏÏÔÔ‡ÏÔ˘ ª, ™ÌÒÎÔ˘ ¢, ª·Ï¿ÌÔ˘ ∂, ÷˚‰¿˜ ∞. ∂ԯȷ΋ ηٷÓÔÌ‹ Ù˘ ÔÍ›·˜ Á·ÛÙÚÂÓÙÂÚ›ÙȉԘ [¶ÂÚ›ÏË„Ë]. 30Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ; 1992 πÔ‡ÓÈÔ˜ 13-14; §ÂÌÂÛfi˜, ∫‡ÚÔ˜; 1992: ∞50. 5. ∞ÚÛ¤ÓË ∞, ª·Ï¿ÌÔ˘-§·‰¿ ∂, ∫Ô‡ÙÛÈ·-∫·ÚÔ‡ÛÔ˘ ∫, ÷ÚÈÛÈ¿‰Ô˘ ∞. ∞Ó·˙‹ÙËÛË Ó¤ˆÓ Î·È ·ÏÈÒÓ ÂÓÙÂÚÔ·ıÔÁfiÓˆÓ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ ÛÙ· ÎfiÚ·Ó· ‰È·ÚÚÔ˚ÎÒÓ ·È‰ÈÒÓ. π·ÙÚÈ΋ 1986;50:361-364. 6. ª·ÙÛ·ÓÈÒÙ˘ ¡, ∫·Ú¿ıÈÔ˜ £. ™ÙÔ: ¶·È‰È·ÙÚÈ΋. ∆fiÌÔ˜ ∞’. ∞ı‹Ó·: π·ÙÚÈΤ˜ ÂΉfiÛÂȘ §›ÙÛ·˜; 1995. ÛÂÏ. 234. 7. Pickering L, Cleary T. Approach to patients with gastrointestinal tract infections and food poisoning. In: Feigin R, Cherry J, eds. Textbook of pediatric infectious diseases. Vol. 1. Philadelphia PN: WB Saunders Company; 1998. p. 567. 8. ÷˚‰¿˜ ∞. ¢È¿ÚÚÔȘ ÙÔ˘ ηÏÔηÈÚÈÔ‡ (‹ ÌÈÎÚԂȷΤ˜ Á·ÛÙÚÂÓÙÂÚ›Ùȉ˜). ¶·È‰È·ÙÚÈ΋ 1997;60:355-362. 9. Afghani B, Stutman H. Toxin-related diarrheas. Pediatr ∞nn 1994;23:549-555. 10. Knoop F, Owens M, Crocker I. Clostridium difficile: clinical disease and diagnosis. Clin Microbiol Rev 1993;6:251-265. 11. ºÈÏ›Ô˘ √, °ÈÔ˘Ï·Ì¿ÎË µ, ∫·‚·ÏÈÒÙ˘ π, ∫·ÓÛÔ˘˙›‰Ô˘ ∞, ∏ ∆. √Í›· Á·ÛÙÚÂÓÙÂÚ›Ùȉ· ·fi ÎÚ˘ÙÔÛÔÚ›‰ÈÔ ÛÙ· ·È‰È¿. ¶·È‰È·ÙÚÈ΋ µÔÚ›Ԣ ∂ÏÏ¿‰Ô˜ 1998;10:25-29. 12. Mehta D, Lebenthal E. New developments in acute diarrhea. Curr Prob Pediatr 1994; 24:95-107. 13. ƒÒÌ· ∂. √Í›· ‰È¿ÚÚÔÈ· ÛÙ· ·È‰È¿. ¢ÂÏÙ ∞' ¶·È‰È·ÙÚ ∫ÏÈÓ ¶·ÓÂ ∞ıËÓÒÓ 1983; 30:183-189. 14. Committee on Nutrition. Use of oral fluid therapy and posttreatment feeding following enteritis in children in a

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developed country. Pediatrics 1985;75:358-361. 15. Provisional committee on quality improvement. Practice parameter: the management of acute gastroenteritis in young children. Pediatrics 1996;97:424-436. 16. Macleod R, Bennett H, Hamilton JR . Inhibition of intestinal secretion by rice. Lancet 1995;346:90-92. 17. ∞‰·Ì›‰Ë˜ ¢, µ·Ï¿ÛÛË-∞‰¿Ì ∂, ƒÒÌ·-°È·ÓÓ›ÎÔ˘ ∂. √Í›· Á·ÛÙÚÂÓÙÂÚ›Ùȉ·: ·ÍÈÔÏfiÁËÛË Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ Û ÌÔÓ¿‰· ÙÚÈÙÔ‚¿ıÌÈ·˜ ÂÚ›ı·Ï„˘. ¢ÂÏÙ ∞' ¶·È‰È·ÙÚ ∫ÏÈÓ ¶·ÓÂ ∞ıËÓÒÓ 1993;40:35-44. 18. Molla A, Rahim A, Sarker S, Mozaffar Z, Rahaman M. Intake and absorption of nutrients in children with cholera and rotavirus infection during acute diarrhea and after recovery. Nutr Res 1982;2:233-242. 19. Duggan C, Nurko S. "Feeding the gut": the scientific basis for continued enteral nutrition during acute diarrhea. J Pediatr 1997;131:801-808. 20. Brown K, Peerson J, Fontaine O. Use of non-human milks in the dietary management of young children with acute diarrhea: a meta-analysis of clinical trials. Pediatrics 1994;93:17-27. 21. Richards L, Claeson M, Pierce N. Management of acute diarrhea in children: lessons learned. Pediatr Infect Dis J 1993;12:5-9. 22. ∫·‚·ÏÈÒÙ˘ π, ™·˚Ó›‰Ô˘ ∞, ∫ˆÓÛÙ·ÓÙԇϷ £, ∆˙ÈÓȤÚ˘ ¢, ∫·ÓÛÔ˘˙›‰Ô˘ ∞, ∆Û·ÁηÚÔÔ‡ÏÔ˘ ∏. ™ÈÁΤÏψÛË Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ÛÙË µ. ∂ÏÏ¿‰·: ÂȉËÌÈÔÏÔÁÈο ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Ù˘ ÂÚÈfi‰Ô˘ 1971-1994. ¶·È‰È·ÙÚÈ΋ 1997;60:372-383. 23. ™ˆÙËÚÔÔ‡ÏÔ˘ º, ÃÚ˘Û¿ÎË ∞, ∑·¯·ÚÈ¿‰Ô˘ §, ∫·Ú‡‰· ∞, ∆ÛÈÏ¿ÎÔ˘ ™, ∞Ó·ÛÙ·Û¤·-µÏ¿¯Ô˘ ¶. ∞ÔÌfiÓˆÛË Û·ÏÌÔÓÂÏÏÒÓ ·fi ηÏÏȤÚÁÂȘ ÎÔÚ¿ÓˆÓ ·È‰ÈÒÓ Ì ‰È¿ÚÚÔÈ· ηٿ ÙËÓ ÙÚÈÂÙ›· 1993-1995. ∞ÓÙÔ¯‹ ÛÙ· ·ÓÙÈ‚ÈÔÙÈο [¶ÂÚ›ÏË„Ë]. 34Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ; 1996 31 ª·˝Ô˘ - 2 πÔ˘Ó›Ô˘; ƒfi‰Ô˜; 1996:µ∞101. 24. Vanderhoof J, Young R. Use of probiotics in childhood gastrointestinal disorders. J Pediatr Gastroenterol Nutr 1997;27:323-332. 25. Meyers A. Fluid and electrolyte therapy for children. Curr Opin Pediatr 1994;6:303-309. HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 26-06-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 20-11-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∫ˆÓÛÙ·ÓÙ›ÓÔ˜ ™È·Ê¿Î·˜, ¶‡ÚÁÔ˘ 1, ∏ÏÈÔ‡ÔÏË 16345 E-mail: csiafaks@ath.forthnet.gr

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¶·È‰È·ÙÚÈ΋ 2001;64:14-24

A¡∞™∫√¶∏™H

Paediatriki 2001;64:14-24

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H Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ÛÙËÓ ·È‰È΋ ËÏÈΛ· ∞. ∏Ï›·˜

Nocturnal enuresis in children A. Elias

¶ÂÚ›ÏË„Ë: H Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË Â›Ó·È Û˘¯Ófi Úfi‚ÏËÌ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜. ∏ ·ÈÙÈÔÏÔÁ›· Ù˘ ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈṲ̂ÓË, ·Ó Î·È ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÂÓÂÙÈο ηıÔÚÈṲ̂Ó˜ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ·Ê‡ÓÈÛ˘ ηıÒ˜ Î·È ÌÂȈ̤ÓË ¤ÎÎÚÈÛË ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ ÙË Ó‡¯Ù·. ¶·ÚfiÏÔ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ·˘ÍË̤ÓÔ Ú˘ıÌfi ÂÙ‹ÛÈ·˜ ·˘ÙfiÌ·Ù˘ ˘Ô¯ÒÚËÛ˘, Âȉڿ ·ÚÓËÙÈο ÛÙËÓ ·˘ÙÔÂÎÙ›ÌËÛË ÙÔ˘ ·È‰ÈÔ‡, ·ÔÙÂÏÒÓÙ·˜ ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ÁÈ· „˘¯ÈΤ˜ ‰È·Ù·Ú·¯¤˜ Î·È ÚÔ‚Ï‹Ì·Ù· ÎÔÈÓˆÓÈ΋˜ ÚÔÛ·ÚÌÔÁ‹˜. ∂Ô̤ӈ˜, Ë ÛÙ¿ÛË ·Ó·ÌÔÓ‹˜ Î·È ·Ú·ÎÔÏÔ‡ıËÛ˘ ‰ÂÓ Â›Ó·È ‰ÈηÈÔÏÔÁË̤ÓË, ·ÏÏ¿ ·ÓÙ›ıÂÙ· ··ÈÙÂ›Ù·È ‰Ú·ÛÙÈ΋ ·Ú¤Ì‚·ÛË, ÂÊfiÛÔÓ ‚¤‚·È· ÙÔ ÂÈı˘Ì› ÙÔ ·È‰› Î·È ÔÈ ÁÔÓ›˜, Ë Û˘ÓÂÚÁ·Û›· ÙˆÓ ÔÔ›ˆÓ Â›Ó·È ··Ú·›ÙËÙË. √È ϤÔÓ ·Ô‰ÂÎÙ¤˜ ıÂÚ·›˜ Â›Ó·È Ë Û˘Û΢‹ ·Ê‡ÓÈÛ˘ Î·È Ë ‰ÂÛÌÔÚÂÛÛ›ÓË, ¯ˆÚ›˜ ˆÛÙfiÛÔ Ó· ·ÔÙÂÏÔ‡Ó ÙËÓ È‰·ÓÈ΋ χÛË. ∏ ·Ô˘Û›· ÎÔÈÓ¿ ·Ô‰ÂÎÙÔ‡ ÙÚfiÔ˘ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘, ÂÈ‚¿ÏÏÂÈ ÙËÓ Â·Ó·ÙÔÔı¤ÙËÛË ÙÔ˘ È·ÙÚÈÎÔ‡ ÎfiÛÌÔ˘ ÛÙÔ Úfi‚ÏËÌ·.

Abstract: Nocturnal enuresis is a common problem in children. The etiology remains unclear and is probably genetically determined, involving poor arousal from sleep and insufficient antidiuretic hormone secretion at night. Enuresis can cause poor self-esteem and is a risk factor for psychiatric disorders and social adjustment problems, despite the high annual spontaneous cure rate. Therefore, a "wait and see" attitude is not justifiable, but on the contrary immediate treatment is indicated with a close co-operation of child, family and physician. The most acceptable treatment is the use of enuresis alarm and desmopressin although they are not the ideal solution to the problem. The experts’ opinions vary widely, indicating a need for a consensus regarding the management of nocturnal enuresis.

§¤ÍÂȘ ÎÏÂȉȿ: Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË, Û˘Û΢‹ ·Ê‡ÓÈÛ˘, ‰ÂÛÌÔÚÂÛÛ›ÓË, DD∞VP.

Key words: nocturnal enuresis, enuresis alarm, vasopressin, DDAVP.

∂ÈÛ·ÁˆÁ‹ ∏ Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ·ÔÙÂÏ› Û˘¯ÓfiÙ·ÙÔ ·È‰È·ÙÚÈÎfi Úfi‚ÏËÌ·, ·ÊÔ‡ ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 15-20% ÙˆÓ ·È‰ÈÒÓ ËÏÈΛ·˜ 5 ÂÙÒÓ. ∞·ÓÙ¿Ù·È Û˘¯ÓfiÙÂÚ· ÛÙ· ·ÁfiÚÈ· Û ۯ¤ÛË Ì ٷ ÎÔÚ›ÙÛÈ· Î·È Ë ·Ó·ÏÔÁ›· Â›Ó·È 2:1 (1). £ÂˆÚÂ›Ù·È Î·ÏÔ‹ı˘ ‰È·Ù·Ú·¯‹ ÁÈ·Ù› ·ÊÂÓfi˜ ÌÂÓ ‰ÂÓ ÚÔηÏ› ‚Ï¿‚Ë ÛÙË ÛˆÌ·ÙÈ΋ ˘Á›· ÙÔ˘ ·È‰ÈÔ‡ Î·È ·ÊÂÙ¤ÚÔ˘ ·ÚÔ˘ÛÈ¿˙ÂÈ Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ ÂÓÙ˘ˆÛÈ·-

΋ Ù¿ÛË ·˘ÙfiÌ·Ù˘ ˘Ô¯ÒÚËÛ˘. Œ¯ÂÈ ˘ÔÏÔÁÈÛı› fiÙÈ Ô ÂÙ‹ÛÈÔ˜ Ú˘ıÌfi˜ ·˘ÙfiÌ·Ù˘ ˘Ô¯ÒÚËÛ‹˜ Ù˘ Â›Ó·È 15%, ÂÓÒ ÛÙËÓ ËÏÈΛ· ÙˆÓ 15 ÂÙÒÓ Ë Û˘¯ÓfiÙËÙ¿ Ù˘ ÂÚÈÔÚ›˙ÂÙ·È ÌfiÏȘ ÛÙÔ 1% (2). °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, Ô È·ÙÚÈÎfi˜ ÎfiÛÌÔ˜ ηٿ ÙÔ ·ÚÂÏıfiÓ, ‰È·Ù‹ÚËÛ ··ı‹ ÛÙ¿ÛË ·¤Ó·ÓÙÈ ÛÙÔ Úfi‚ÏËÌ·, Ì ·ÔÙ¤ÏÂÛÌ· ÁÈ· ¿Ú· ÔÏÏ¿ ¯ÚfiÓÈ· Ë ÂÓÔ‡ÚËÛË Ó· ·ÔÙÂÏ› ‚·Ú‡ ÊÔÚÙ›Ô ÁÈ· ÙÔ ·È‰› Î·È ÙËÓ ÔÈÎÔÁ¤ÓÂÈ·, ·ÏÏ¿ fi¯È ÁÈ· ÙÔ ÁÈ·ÙÚfi. ŸÌˆ˜,

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ª·Î¿ÚÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô §Â˘ÎˆÛ›·˜

Pediatric Clinic, Makario Hospital of Nicosia, Cyprus

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2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 15

¶·È‰È·ÙÚÈ΋ 2001;64:14-24

ÙÔ 1985, ÌÈ· ÂÚ¢ÓËÙÈ΋ ÂÚÁ·Û›· ÚÔÂÚ¯fiÌÂÓË ·fi ÙË ¢·Ó›·, ‹Ïı ӷ Ù·Ú¿ÍÂÈ Î˘ÚÈÔÏÂÎÙÈο Ù· ÏÈÌÓ¿˙ÔÓÙ· ‡‰·Ù·. ™ÙËÓ ÂÚÁ·Û›· ·˘Ù‹, ‰È·ÈÛÙÒıËΠfiÙÈ Ù· Â›‰· Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ ÛÙ· ¿ÙÔÌ· Ì Ӣ¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ‰ÂÓ ·˘Í¿ÓÔÓÙ·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, fiˆ˜ Û˘Ì‚·›ÓÂÈ ÛÙ· ÌË ÂÓÔ˘ÚËÙÈο ·È‰È¿, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÚfiÎÏËÛË Ó˘¯ÙÂÚÈÓ‹˜ ÔÏ˘Ô˘Ú›·˜ (3). ŒÎÙÔÙÂ, ÙÔ ÎÏÈÓÈÎfi Î·È ÂÚ¢ÓËÙÈÎfi ÂӉȷʤÚÔÓ ·Ó·˙ˆ˘ÚÒıËΠ̠·ÔÙ¤ÏÂÛÌ· Ó· ·ÎÔÏÔ˘ı‹ÛÔ˘Ó ÂηÙÔÓÙ¿‰Â˜ ÌÂϤÙ˜, ÚÔÛ‰›‰ÔÓÙ·˜ ÛÙÔ ı¤Ì· ÙËÓ Ú·ÁÌ·ÙÈ΋ ÙÔ˘ ‰È¿ÛÙ·ÛË. ∂ÈϤÔÓ, ·ÚÎÂÙ¤˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ·ԉ›ÍÂÈ ÙËÓ ·ÚÓËÙÈ΋ Â›‰Ú·ÛË Ù˘ ÂÓÔ‡ÚËÛ˘ ÛÙËÓ „˘¯È΋ ˘Á›· ÙÔ˘ ·È‰ÈÔ‡ Î·È ÙË ‰È·Ù·Ú·¯‹ ÙˆÓ Û¯¤ÛÂÒÓ ÙÔ˘ Ì ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi Î·È ÎÔÈÓˆÓÈÎfi ÂÚÈ‚¿ÏÏÔÓ. ™˘ÓÂÒ˜, Ë ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË ıˆÚÂ›Ù·È ÂȂ‚ÏË̤ÓË, ȉȷ›ÙÂÚ· ÛÙ· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿, Ô˘ ÁÂÌ¿Ù· ÂÓÔ¯¤˜ ¯¿ÓÔ˘Ó ÙËÓ ·˘ÙÔÂÎÙ›ÌËÛË ÙÔ˘˜, Ì ·ÚÓËÙÈ΋ Â›‰Ú·ÛË ÛÙËÓ ·Ó¿Ù˘ÍË Ù˘ ÚÔÛˆÈÎfiÙËÙ¿˜ ÙÔ˘˜ (4). OÚÈÛÌÔ› ø˜ ÌÔÓÔÛ˘Ìو̷ÙÈ΋ Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË, ÔÚ›˙ÂÙ·È Ë ·ÎÔ‡ÛÈ· Ô‡ÚËÛË Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ ÙÔ˘Ï¿¯ÈÛÙÔÓ 2 ÊÔÚ¤˜ ÙÔÓ Ì‹Ó·, ÌÂÙ¿ ÙËÓ ËÏÈΛ· ÙˆÓ 5 ÂÙÒÓ, ¯ˆÚ›˜ Û˘ÁÁÂÓ‹ ‹ Â›ÎÙËÙË ‰È·Ù·Ú·¯‹ ÙÔ˘ Ô˘ÚÔÔÈÔÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜. ¢È·ÎÚ›ÓÂÙ·È Û ÚˆÙÔ·ı‹ Î·È ‰Â˘ÙÂÚÔ·ı‹. ∏ ÚˆÙÔ·ı‹˜ ··ÓÙ¿Ù·È ÛÙÔ 75-80% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ÂÚÈÙÒÛÂˆÓ Î·È ÙÔ ·È‰› Ô˘‰¤ÔÙ ·¤ÎÙËÛ ÙÔÓ ¤ÏÂÁ¯Ô Ù˘ ·ÛÙÂÒ˜ ÙÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ (1). ∞ÓÙ›ıÂÙ·, ÛÙË ‰Â˘ÙÂÚÔ·ı‹, ÚÔËÁÂ›Ù·È ÂÚ›Ô‰Ô˜ 6 ÙÔ˘Ï¿¯ÈÛÙÔÓ ÌËÓÒÓ, ÛÙËÓ ÔÔ›· ÙÔ ·È‰› ÛÙ·Ì¿ÙËÛ ӷ ‚Ú¤¯ÂÙ·È. ∞ÒÏÂÈ· Ô‡ÚˆÓ Î·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ë̤ڷ˜ ÌÔÚ› Ó· ·Ú·ÙËÚËı› ÛÙÔ 15-20% ÙˆÓ ·È‰ÈÒÓ Ì Ӣ¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË. ∏ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ ·ÔÎÙ¿ ÙÔÓ ¤ÏÂÁ¯Ô Ù˘ Ô‡ÚËÛ˘, ÚÒÙ· ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ë̤ڷ˜ Î·È ÛÙË Û˘Ó¤¯ÂÈ· Î·È ÙË Ó‡¯Ù·. ∂ÈÙ˘¯‹˜ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· ÔÚ›˙ÂÙ·È Ë ‰È·Ù‹ÚËÛË ÛÙÂÁÓÔ‡ ÙÔ˘ ·È‰ÈÔ‡ ÁÈ· 14 Û˘Ó¯fiÌÂÓ˜ Ó‡¯Ù˜ Û ‰È¿ÛÙËÌ· 16 ‚‰ÔÌ¿‰ˆÓ ıÂÚ·›·˜. ÀÔÙÚÔ‹ ÔÚ›˙ÂÙ·È Ë ÂÌÊ¿ÓÈÛË 2 ÂÂÈÛÔ‰›ˆÓ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Û ‰È¿ÛÙËÌ· 2 ‚‰ÔÌ¿‰ˆÓ. ™˘Ó¯È˙fiÌÂÓË ÂÈÙ˘¯›· ÔÚ›˙ÂÙ·È Ë ·Ô˘Û›· ˘ÔÙÚÔ‹˜ ÌÂÙ¿ ·fi 6 Ì‹Ó˜, ÂÓÒ Ï‹Ú˘ ÂÈÙ˘¯›·, Ë ·Ô˘Û›· ˘ÔÙÚÔ‹˜ ÌÂÙ¿ 2 ¯ÚfiÓÈ· ·fi ÙËÓ ·Ú¯È΋ ÂÈÙ˘¯‹ ·ÓÙ·fiÎÚÈÛË (5). øڛ̷ÓÛË ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ÂϤÁ¯Ô˘ Ù˘ Ô‡ÚËÛ˘ ∏ ˆÚ›Ì·ÓÛË ÙÔ˘ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙÔ ÛÙ·‰È·Îfi ¤ÏÂÁ¯Ô Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ Ù˘ ·ÛÙˆ˜ Î·È ÙÔ˘ ÂÓÙ¤ÚÔ˘, Ì ÙËÓ ·ÎfiÏÔ˘ıË ÛÂÈÚ¿:

Paediatriki 2001;64:14-24

- ŒÏÂÁ¯Ô˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘. - ŒÏÂÁ¯Ô˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘ ηٿ ÙËÓ ÂÁÚ‹ÁÔÚÛË. - ŒÏÂÁ¯Ô˜ Ù˘ ·ÛÙˆ˜ ηٿ ÙËÓ ÂÁÚ‹ÁÔÚÛË. - ŒÏÂÁ¯Ô˜ Ù˘ ·ÛÙˆ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘. ™ÙÔ ÚÒÙÔ ¤ÙÔ˜ Ù˘ ˙ˆ‹˜, Ë Ô‡ÚËÛË Á›ÓÂÙ·È ·ÎÔ‡ÛÈ· (·ÓÙ·Ó·ÎÏ·ÛÙÈο), ¯ˆÚ›˜ ÙÔ ‚Ú¤ÊÔ˜ Ó· ¤¯ÂÈ ÙËÓ ·›ÛıËÛË Ï‹ÚˆÛ˘ ‹ ΤӈÛ˘ Ù˘ ·ÛÙˆ˜. ∏ Ï‹ÚˆÛË Ù˘ ·ÛÙˆ˜ ÚÔηÏ› ·ÓÙ·Ó·ÎÏ·ÛÙÈ΋ Û‡Û·ÛË ÙÔ˘ Â͈ÛÙ‹Ú· Ì˘fi˜ Ì ٷ˘Ùfi¯ÚÔÓË ¯·Ï¿ÚˆÛË ÙÔ˘ ÛÊÈÁÎÙ‹Ú·, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂχıÂÚË Î¤ÓˆÛ‹ Ù˘. ™ÙËÓ ËÏÈΛ· ·˘Ù‹, Ë ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜ Â›Ó·È ÌÈÎÚ‹ (ÂÚ›Ô˘ 60 ml), ÂÓÒ Ô ·ÚÈıÌfi˜ ÙˆÓ Ô˘Ú‹ÛÂˆÓ ÌÂÁ¿ÏÔ˜ (ÂÚ›Ô˘ 20 ËÌÂÚËÛ›ˆ˜) (6). ™Ù· ÂfiÌÂÓ· 2 ¯ÚfiÓÈ·, Ë Ô‡ÚËÛË ÂÍ·ÎÔÏÔ˘ı› Ó· Á›ÓÂÙ·È ·ÓÙ·Ó·ÎÏ·ÛÙÈο, Ô ËÌÂÚ‹ÛÈÔ˜ fï˜ ·ÚÈıÌfi˜ ÙˆÓ Ô˘Ú‹ÛÂˆÓ Â›Ó·È ÛËÌ·ÓÙÈο ÌÈÎÚfiÙÂÚÔ˜. ∆Ô‡ÙÔ ÔÊ›ÏÂÙ·È Î˘Ú›ˆ˜, ÛÙË ‚·ıÌÈ·›· ·‡ÍËÛË Ù˘ ¯ˆÚËÙÈÎfiÙËÙ·˜ Ù˘ ·ÛÙˆ˜ ·ÏÏ¿ Î·È ÛÙËÓ ˆÚ›Ì·ÓÛË ÙÔ˘ ÚfiÛıÈÔ˘ Î·È ÙˆÓ Ï·Á›ˆÓ ÂÁÎÂÊ·ÏÈÎÒÓ ÏÔ‚ÒÓ, Ë ÔÔ›· ÚÔηÏ› ÌÂÚÈ΋ ÎÂÓÙÚÈ΋ ·Ó·ÛÙÔÏ‹ ÛÙÔ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎfi Ù˘ Ô‡ÚËÛ˘ (7). ŒÙÛÈ, ÛÙÔ ‰Â‡ÙÂÚÔ ¤ÙÔ˜ Ù˘ ˙ˆ‹˜, ÙÔ ·È‰› ·Ú¯›˙ÂÈ Ó· ·ÓÙÈÏ·Ì‚¿ÓÂÙ·È ÙËÓ Ï‹ÚˆÛË Ù˘ ·ÛÙˆ˜ Î·È ·Ó·ÁÓˆÚ›˙ÂÈ ÙËÓ ÂÈΛÌÂÓË Ô‡ÚËÛË, ¯ˆÚ›˜ fï˜, Ó· ÙËÓ ÂϤÁ¯ÂÈ. ∫·Ù¿ ÙÔ ÙÚ›ÙÔ ¤ÙÔ˜ Ù˘ ˙ˆ‹˜, ÙÔ ·È‰› ÂϤÁ¯ÂÈ Î·Ï‡ÙÂÚ· ÙËÓ Î‡ÛÙË ÙÔ˘ Î·È ·Ú·Ì¤ÓÂÈ ÛÙÂÁÓfi ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ë̤ڷ˜. ™ÙËÓ ËÏÈΛ· ÙˆÓ 4-6 ÂÙÒÓ, Ë ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜ ·˘Í¿ÓÂÙ·È ÂÚÈÛÛfiÙÂÚÔ Î·È ·Ú¿ÏÏËÏ· ·ÔÎÙ¿Ù·È ÂÎÔ‡ÛÈÔ˜ ¤ÏÂÁ¯Ô˜ Â› ÙÔ˘ ¤Íˆ ÛÊÈÁÎÙ‹Ú·, Ô ÔÔ›Ô˜ Â›Ó·È ··Ú·›ÙËÙÔ˜ ÁÈ· ÙËÓ ÂÎÔ‡ÛÈ· ¤Ó·ÚÍË Î·È ÙÔÓ ÙÂÚÌ·ÙÈÛÌfi Ù˘ Ô‡ÚËÛ˘. ◊‰Ë ÛÙËÓ ËÏÈΛ· ÙˆÓ 4-5 ÂÙÒÓ, Ù· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ Â›Ó·È Èηӿ Ó· ‰È·ÙËÚÔ‡Ó ÈηÓÔÔÈËÙÈÎfi ¤ÏÂÁ¯Ô ÙˆÓ Ô‡ÚˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ (1). AÈÙÈÔÏÔÁ›· ∏ ·ÈÙÈÔÏÔÁ›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Ê·›ÓÂÙ·È Ó· Â›Ó·È ÔÏ˘·Ú·ÁÔÓÙÈ΋ Î·È fi¯È Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈṲ̂ÓË. ™ÙËÓ ·ıÔÁ¤ÓÂÈ¿ Ù˘ ÂÌϤÎÔÓÙ·È ÔÈΛÏÔÈ ·Ú¿ÁÔÓÙ˜ fiˆ˜ Ë Î·ı˘ÛÙ¤ÚËÛË Ù˘ ˆÚ›Ì·ÓÛ˘ ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ÂϤÁ¯Ô˘ Ù˘ ·ÛÙˆ˜, ÁÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜, ‰È·Ù·Ú·¯¤˜ ÛÙÔ Ú˘ıÌfi ¤ÎÎÚÈÛ˘ Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ Î·È ‰È·Ù·Ú·¯¤˜ ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ‡ÓÔ˘-·Ê‡ÓÈÛ˘. K·ı˘ÛÙ¤ÚËÛË ˆÚ›Ì·ÓÛ˘ ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ÂϤÁ¯Ô˘ Ù˘ ·ÛÙˆ˜ ∏ ÏÂÈÙÔ˘ÚÁÈ΋ ηı˘ÛÙ¤ÚËÛË Ù˘ ˆÚ›Ì·ÓÛ˘ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ÌÂÈÒÓÂÈ ÙËÓ

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2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 16

¶·È‰È·ÙÚÈ΋ 2001;64:14-24

ÈηÓfiÙËÙ· ·Ó·ÛÙÔÏ‹˜ ÙˆÓ Û˘Û¿ÛÂˆÓ Ù˘ ·ÛÙˆ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜. ∏ ¿Ô„Ë ·˘Ù‹ ÂÓÈÛ¯‡ÂÙ·È ¤ÓÙÔÓ· ·fi ÙÔÓ ·˘ÍË̤ÓÔ Ú˘ıÌfi ·˘ÙfiÌ·Ù˘ ˘Ô¯ÒÚËÛ˘ Ù˘ ÂÓÔ‡ÚËÛ˘ Ì ÙËÓ ¿ÚÔ‰Ô Ù˘ ËÏÈΛ·˜. ∂Í¿ÏÏÔ˘, Ë Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË Â›Ó·È Û˘¯ÓfiÙÂÚË ÛÙ· ·È‰È¿ Ì ÓÔËÙÈ΋ ηı˘ÛÙ¤ÚËÛË ‹ ηı˘ÛÙ¤ÚËÛË ÛÙËÓ „˘¯ÔÎÈÓËÙÈ΋ ÙÔ˘˜ ·Ó¿Ù˘ÍË (1,8). °ÂÓÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ∞fi ÔÏÏ¿ ¯ÚfiÓÈ· Â›Ó·È ÁÓˆÛÙfi fiÙÈ Ë Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ·Ú·ÙËÚÂ›Ù·È Ì ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· Û ̤ÏË Ù˘ ›‰È·˜ ÔÈÎÔÁ¤ÓÂÈ·˜. ŒÙÛÈ, Â¿Ó ÎÈ ÔÈ ‰‡Ô ÁÔÓ›˜ ¤¯Ô˘Ó ÈÛÙÔÚÈÎfi ÂÓÔ‡ÚËÛ˘, ÔÈ Èı·ÓfiÙËÙ˜ ÁÈ· ÙÔ ·È‰› Â›Ó·È ÂÚ›Ô˘ 77%, ÂÓÒ fiÙ·Ó Ô ¤Ó·˜ ÁÔÓ¤·˜ ¤¯ÂÈ ÈÛÙÔÚÈÎfi, ÔÈ Èı·ÓfiÙËÙ˜ ÁÈ· ÙÔ ·È‰› Â›Ó·È 44% (1). ™Â Û¯ÂÙÈ΋ ÌÂϤÙË ‚Ú¤ıËΠfiÙÈ Ù· ·È‰È¿ Ì ÂÓÔ˘ÚËÙÈÎÔ‡˜ ·Ù¤Ú˜, ¤¯Ô˘Ó 7,1 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË Èı·ÓfiÙËÙ· Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘, ÂÓÒ Ë Èı·ÓfiÙËÙ· ÙˆÓ ·È‰ÈÒÓ ÙˆÓ ÂÓÔ˘ÚËÙÈÎÒÓ ÌËÙ¤ÚˆÓ Â›Ó·È 5,2 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË (9). √ ÙÚfiÔ˜ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜ ÌÂÏÂÙ‹ıËΠ۠392 ÔÈÎÔÁ¤ÓÂȘ Ì ÈÛÙÔÚÈÎfi Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Î·È ‚Ú¤ıËΠӷ Â›Ó·È ÂÈÎÚ·ÙÒÓ ÛÙÔ 43%, ˘ÔÏÂÈfiÌÂÓÔ˜ ÛÙÔ 9%, ÂÓÒ ÛÙÔ 48% Â›Ó·È ÛÔÚ·‰ÈÎfi˜ (10). ∂Ô̤ӈ˜, Ô ÁÔÓfiÙ˘Ô˜ Î·È Ô ÙÚfiÔ˜ Ô˘ ÂÎÊÚ¿˙ÂÙ·È, Ê·›ÓÂÙ·È Ó· Â›Ó·È Û‡ÓıÂÙÔ˜ Î·È Ì ÌÂÁ¿ÏË ÂÙÂÚÔÁ¤ÓÂÈ·. ∞˘Ùfi ÂÓÈÛ¯‡ÂÙ·È ÎÈ ·fi ÙÔ ÁÂÁÔÓfi˜ Ù˘ ÂÓÙfiÈÛ˘ ÙÔ˘ ÁÔÓȉ›Ô˘ Ù˘ ÂÓÔ‡ÚËÛ˘ Û ‰È¿ÊÔÚ· ¯ÚˆÌÔÛÒÌ·Ù· (¯ÚˆÌÔÛÒÌ·Ù· 8, 12, 13 Î·È 22) (11,12). ∞ÓÙȉÈÔ˘ÚËÙÈ΋ ÔÚÌfiÓË ∏ ·ıÔÏÔÁÈ΋ ¤ÎÎÚÈÛË Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ ¤¯ÂÈ ÂÓÔ¯ÔÔÈËı› ˆ˜ ¤Ó· ·fi Ù· ΢ÚÈfiÙÂÚ· ·›ÙÈ· Ù˘ ·ıÔÁ¤ÓÂÛ˘ Ù˘ ÚˆÙÔ·ıÔ‡˜ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘. ™Ù· Ê˘ÛÈÔÏÔÁÈο ¿ÙÔÌ· Ê·›ÓÂÙ·È fiÙÈ ˘¿Ú¯ÂÈ ·ÚÌÔÓÈ΋ ÂÈÎÔÈÓˆÓ›· ÌÂٷ͇ ÓÂÊÚÒÓ Î·È Ô˘ÚÔ‰fi¯Ô˘ ·ÛÙˆ˜, Û ‚·ıÌfi Ô˘ Ë Ï‹ÚˆÛË Ù˘ ·ÛÙˆ˜, ÌÂÈÒÓÂÈ ÙËÓ ·Ú·ÁˆÁ‹ ÙˆÓ Ô‡ÚˆÓ ·fi Ù· ÓÂÊÚ¿. ∞˘Ùfi ÈÛÙ‡ÂÙ·È fiÙÈ ÂÈÙ˘Á¯¿ÓÂÙ·È Ì ÙË ‚Ô‹ıÂÈ· Ù˘ ÚˆÙ½Ó˘ ˘‰·ÙÔÔÚ›Ó˘ 2, Ë ÔÔ›· ‚Ú›ÛÎÂÙ·È ˘fi ÙËÓ Â›‰Ú·ÛË Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘. ∏ ˘‰·ÙÔÔÚ›ÓË 2, ‰ËÌÈÔ˘ÚÁ› ηӿÏÈ· ‡‰·ÙÔ˜ ‰È·Ì¤ÛÔ˘ ÙˆÓ ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ ÙˆÓ ·ıÚÔÈÛÙÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ, ÂÈÙÚ¤ÔÓÙ·˜ ÙË ‰È·Ê˘Á‹ ‡‰·ÙÔ˜ ÚÔ˜ ÙËÓ Î˘ÎÏÔÊÔÚ›· ÙÔ˘ ·›Ì·ÙÔ˜, ‰È·‰Ú·Ì·Ù›˙ÔÓÙ·˜ ¤ÙÛÈ, ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ÂÓÔ‡ÚËÛ˘ (13). ¶ÔÏϤ˜ ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ ¤¯Ô˘Ó ·ԉ›ÍÂÈ fiÙÈ Ë ¤ÎÎÚÈÛË Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ Â›Ó·È ·˘ÍË̤ÓË Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ ÛÂ Ê˘ÛÈÔÏÔÁÈο ¿ÙÔÌ·, ÂÓÒ Û ÂÓÔ˘ÚËÙÈο Ë ¤ÎÎÚÈÛ‹ Ù˘ Â›Ó·È ÛË-

16

Paediatriki 2001;64:14-24

Ì·ÓÙÈο ÌÂȈ̤ÓË ÌÂٷ͇ ÙˆÓ ˆÚÒÓ 23:00 Î·È 04:00 (3,14,15). ∏ ÌÂȈ̤ÓË ·˘Ù‹ ¤ÎÎÚÈÛË Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜, Ô‰ËÁ› Û ·˘ÍË̤ÓÔ fiÁÎÔ Ô‡ÚˆÓ, Ô ÔÔ›Ô˜ Â¿Ó ˘Âڂ› ÙË ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜ ¤¯ÂÈ Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙË Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË. ªÂÙ·ÁÂÓ¤ÛÙÂÚË ÌÂϤÙË ·¤‰ÂÈÍ fiÙÈ Ë ¤ÎÎÚÈÛË Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜, Â›Ó·È ÌÂȈ̤ÓË ÌfiÓÔ ÛÙ· ÂÓÔ˘ÚËÙÈο ¿ÙÔÌ· Ì Ӣ¯ÙÂÚÈÓ‹ ÔÏ˘Ô˘Ú›·, ÂÓÒ ‰ÂÓ ·Ú·ÙËÚÂ›Ù·È ÌÂȈ̤ÓË ¤ÎÎÚÈÛË ÛÙ· ÂÓÔ˘ÚËÙÈο ¿ÙÔÌ· Ì ·Ú·ÁˆÁ‹ ÌÈÎÚÒÓ fiÁÎˆÓ Ô‡ÚˆÓ Î·Ù¿ ÙË Ó‡¯Ù· Î·È Ê˘ÛÈÔÏÔÁÈ΋ ÔÛ̈ÙÈ΋ ›ÂÛË ÛÙ· ÚˆÈÓ¿ Ô‡Ú· (16). ∂›Ó·È, ÂÔ̤ӈ˜, Ê·ÓÂÚfi fiÙÈ Ë Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ‰ÂÓ ÌÔÚ› Ó· ·Ô‰Ôı› ·ÔÎÏÂÈÛÙÈο ÛÙË ÌÂȈ̤ÓË ¤ÎÎÚÈÛË Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜. ∞˘Ùfi, ¿ÏψÛÙÂ, ÂȂ‚·ÈÒÓÂÙ·È ÎÈ ·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÌfiÓÔ ÙÔ 10-70% ÂÚ›Ô˘ ÙˆÓ ·È‰ÈÒÓ Ì Ӣ¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ·ÓÙ·ÔÎÚ›ÓÂÙ·È ÛÙË ıÂÚ·›· Ì ·ÓÙȉÈÔ˘ÚËÙÈ΋ ÔÚÌfiÓË. ¢È·Ù·Ú·¯¤˜ ‡ÓÔ˘ – ·Ê‡ÓÈÛ˘ √È ÂÚÈÛÛfiÙÂÚ˜ ·fi ÙȘ ·Ï·ÈfiÙÂÚ˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó fiÙÈ Ë Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË Â›Ó·È ‰˘Ó·ÙfiÓ Ó· Û˘Ì‚Â› Û fiÏ· Ù· ÛÙ¿‰È· ÙÔ˘ ‡ÓÔ˘. ∞˘Ùfi Ô‰‹ÁËÛ ÛÙËÓ ¿Ô„Ë fiÙÈ ÔÈ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ‡ÓÔ˘ Î·È Ù˘ ·Ê‡ÓÈÛ˘ ‰ÂÓ ‰È·‰Ú·Ì·Ù›˙Ô˘Ó ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ·ÈÙÈÔÏÔÁ›· Ù˘ ÂÓÔ‡ÚËÛ˘. ∏ ¿Ô„Ë ·˘Ù‹ ÎÏÔÓ›ÛıËΠÛÙË Û˘Ó¤¯ÂÈ·, ·fi ÌÂϤÙË ÙˆÓ ÂÓÔ˘ÚËÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ Ì ٷ˘Ùfi¯ÚÔÓË Î·Ù·ÁÚ·Ê‹ ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ·Ê‹Ì·ÙÔ˜ Î·È Î˘ÛÙÂÔÌ·ÓÔÌÂÙÚ›·˜. ™‡Ìʈӷ Ì ٷ ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜, ÙÔ 60% Î·È ϤÔÓ ÙˆÓ ÂÓÔ˘ÚËÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ Û˘Ì‚·›ÓÔ˘Ó Û ÛÙ¿‰ÈÔ ÌÂÚÈ΋˜ ·Ê‡ÓÈÛ˘, ¯ˆÚ›˜, fï˜, ÙÔ ·È‰› Ó· ¤¯ÂÈ Ï‹Úˆ˜ ·Ê˘ÓÈÛı› (17). ™‹ÌÂÚ·, Â›Ó·È ÁÓˆÛÙfi fiÙÈ ÙÔ˘Ï¿¯ÈÛÙÔÓ 4 ÂÁÎÂÊ·ÏÈο ‰›ÎÙ˘· Ó¢ÚÒÓˆÓ Â›Ó·È ˘‡ı˘Ó· ÁÈ· ÙËÓ ·Ê‡ÓÈÛË. ∞fi ·˘Ù¿, ÙÔ ‰›ÎÙ˘Ô Ô˘ ÚÔ¤Ú¯ÂÙ·È ·fi ÙÔÓ ˘Ú‹Ó· locus coeruleus (LC) Ô ÔÔ›Ô˜ ‰ڿ˙ÂÙ·È ÛÙÔÓ ˘ı̤ӷ Ù˘ 4˘ ÎÔÈÏ›·˜ ÙÔ˘ ÂÁÎÂÊ¿ÏÔ˘ Î·È ÂÓÂÚÁÔÔÈÂ›Ù·È ·fi ÙË ÓÔÚ·‰ÚÂÓ·Ï›ÓË, ÈÛÙ‡ÂÙ·È fiÙÈ ·ÔÙÂÏ› ÙÔ ÛËÌ·ÓÙÈÎfiÙÂÚÔ Û‡ÛÙËÌ· ÛÙË ‰È¤ÁÂÚÛË Ù˘ ·Ê‡ÓÈÛ˘ (18,19). ∏ Ï‹ÚˆÛË Ù˘ Ô˘ÚÔ‰fi¯Ô˘ ·ÛÙˆ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ·ÔÙÂÏ› ÙÔ ÂÚ¤ıÈÛÌ· ÁÈ· ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÂÁÎÂÊ·ÏÈÎÒÓ ‰ÈÎÙ‡ˆÓ Ù˘ ·Ê‡ÓÈÛ˘, ΢ڛˆ˜ ÙÔ˘ LC ‰ÈÎÙ‡Ô˘ Ô˘ ÌÂÙ·ÙÚ¤ÂÈ ÙÔÓ ‚·ı‡ ‡ÓÔ Û ÂÏ·ÊÚ‡. ∆Ô Î¤ÓÙÚÔ ÙÔ˘ ‡ÓÔ˘ ÙÔ ÔÔ›Ô Â‰Ú¿˙ÂÙ·È Èı·ÓÒ˜ ÛÙÔ ı¿Ï·ÌÔ ‹ ÛÙÔÓ ˘Ôı¿Ï·ÌÔ, ‰Ú· ·ÓÙ·ÁˆÓÈÛÙÈο ηٷÛÙ¤ÏÏÔÓÙ·˜ ÙËÓ ·Ê‡ÓÈÛË. °È· ÙËÓ ·Ê‡ÓÈÛË ÙÔ˘ ·È‰ÈÔ‡, ¤Ó· ·ÓÒÙÂÚÔ Î¤ÓÙÚÔ ÙÔ˘ ÂÁÎÂÊ·ÏÈÎÔ‡ ÊÏÔÈÔ‡, ı· ‰ÒÛÂÈ ‹ fi¯È ÙËÓ ÙÂÏÈ΋ ÂÓÙÔÏ‹ (19). ¢È·Ù·Ú·¯‹ ÙˆÓ Ì˯·ÓÈÛÌÒÓ


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 17

¶·È‰È·ÙÚÈ΋ 2001;64:14-24

·˘ÙÒÓ, ¤¯ÂÈ Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙË ÌË ·Ê‡ÓÈÛË Î·È ÙËÓ ÂÓÔ‡ÚËÛË. ªÂ ‚¿ÛË ÙË ‰È·Ù·Ú·¯‹ ÛÙÔ˘˜ Ì˯·ÓÈÛÌÔ‡˜ ·Ê‡ÓÈÛ˘, ÙÔ 1989 ÚÔÙ¿ıËΠ¤Ó· Ó¤Ô Û‡ÛÙËÌ· ‰È·¯ˆÚÈÛÌÔ‡ Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Û 3 Ù‡Ô˘˜. ∆ÔÓ Ù‡Ô π, ÙÔÓ ππ· Î·È ÙÔÓ ππ‚ (17). ∏ ηٿٷÍË Á›ÓÂÙ·È Ì ÙËÓ Ù·˘Ùfi¯ÚÔÓË Î·Ù·ÁÚ·Ê‹ ÙÔ˘ ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ·Ê‹Ì·ÙÔ˜ Î·È Ù˘ ΢ÛÙÂÔÌ·ÓÔÌÂÙÚ›·˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜, Ú¿ÁÌ· Ô˘ ηıÈÛÙ¿ ‰‡ÛÎÔÏË ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ú¿ÍË (20). ∏ ÂÓÔ‡ÚËÛË Ù‡Ô˘ π Â›Ó·È Ô ÈÔ Û˘¯Ófi˜ Ù‡Ô˜, ·ÊÔÚ¿ ÙÔ 60% ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ ÂÓÔ˘ÚËÙÈÎÒÓ Î·È ÚÔηÏÂ›Ù·È ·fi ‹Ș ‰È·Ù·Ú·¯¤˜ Ù˘ ·Ê‡ÓÈÛ˘. ŸÙ·Ó Ë Ô˘ÚÔ‰fi¯Ô˜ ·ÛÙË ÁÂÌ›ÛÂÈ, ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‚·ı¤ˆ˜ ‡ÓÔ˘ (ÛÙ¿‰ÈÔ ‡ÓÔ˘ 3 ‹ 4), Ë ·Ê‡ÓÈÛË ·Ú¯›˙ÂÈ Ó· ηٷÁÚ¿ÊÂÙ·È ÛÙÔ ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ· ÌÂÙ·ÙÚ¤ÔÓÙ·˜ ÙÔ ‚·ı‡ ‡ÓÔ Û ÂÏ·ÊÚ‡ (ÛÙ¿‰ÈÔ 1 ‹ 2). ¶ÂÚ›Ô˘ 5-15 ÏÂÙ¿ ÌÂÙ¿ ÙËÓ ·ÏÏ·Á‹ ·˘Ù‹, Ë ÂÓÔ‡ÚËÛË Â¤Ú¯ÂÙ·È ¯ˆÚ›˜ ÙËÓ ·Ê‡ÓÈÛË ÙÔ˘ ·ÙfiÌÔ˘. √ Ù‡Ô˜ ππ· ·ÊÔÚ¿ ÛÙÔ 10% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ·È‰ÈÒÓ Ì ÂÓÔ‡ÚËÛË Î·È ÔÊ›ÏÂÙ·È Û ·‰˘Ó·Ì›· ‰È¤ÁÂÚÛ˘ ÙÔ˘ ΤÓÙÚÔ˘ Ù˘ ·Ê‡ÓÈÛ˘ ·Ú¿ ÙËÓ Ï‹ÚˆÛË Ù˘ Ô˘ÚÔ‰fi¯Ô˘ ·ÛÙˆ˜. √ Ù‡Ô˜ ππ‚ ·ÊÔÚ¿ ÛÙÔ 30% ÙÔ˘ Û˘ÓfiÏÔ˘ Î·È ÔÊ›ÏÂÙ·È Û ϷÓı¿ÓÔ˘Û· Ó¢ÚÔÁÂÓ‹ ·ÛÙË Ë ÔÔ›· Â›Ó·È Ê·ÓÂÚ‹ ÌfiÓÔ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘. √È Û˘¯Ó¤˜ Û˘Û¿ÛÂȘ Ù˘ Ô˘ÚÔ‰fi¯Ô˘ ·ÛÙˆ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜, ·ÚfiÏÔ Ô˘ Ë Î˘ÛÙÂÔÌ·ÓÔÌÂÙÚ›· Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋ ÙËÓ Ë̤ڷ, Ô‰ËÁÔ‡Ó Û ·‰˘Ó·Ì›· ‰È¤ÁÂÚÛ˘ ÙÔ˘ ΤÓÙÚÔ˘ Ù˘ ·Ê‡ÓÈÛ˘ fiÙ·Ó Ë Î‡ÛÙË Â›Ó·È ÁÂÌ¿ÙË, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÓÔ‡ÚËÛË. ∂›Ó·È, ÂÔ̤ӈ˜, Ê·ÓÂÚfi fiÙÈ ˘¿Ú¯ÂÈ ÈÛ¯˘Ú‹ Û¯¤ÛË ÌÂٷ͇ Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Î·È ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ‡ÓÔ˘–·Ê‡ÓÈÛ˘. ∂ÈÙÒÛÂȘ ÛÙÔÓ „˘¯ÈÛÌfi ∏ Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË, ÛÙËÓ ÏÂÈÔ„ËÊ›· Ù˘, ‰ÂÓ ÚÔηÏ›ٷÈ, fiˆ˜ ·Ï·ÈfiÙÂÚ· ÈÛÙ¢fiÙ·Ó, ·fi „˘¯ÔÏÔÁÈÎÔ‡˜, ÎÔÈÓˆÓÈÎÔ‡˜ ‹ ÂÚÈ‚·ÏÏÔÓÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜. ∞ÓÙ›ıÂÙ·, ÌÔÚ› Ó· ÚÔηϤÛÂÈ ‰Â˘ÙÂÚÔ·ıÒ˜ „˘¯ÔÏÔÁÈο ÚÔ‚Ï‹Ì·Ù· ÛÙÔ ·È‰›, Ì ȉȷ›ÙÂÚ· ·ÚÓËÙÈ΋ Â›‰Ú·ÛË ÛÙËÓ ·˘ÙÔÂÎÙ›ÌËÛ‹ ÙÔ˘ (21,22). ™Â ÌÂϤÙË, ·Ó·Ê¤ÚÂÙ·È fiÙÈ Ë ·˘ÙÔÂÎÙ›ÌËÛË ÙˆÓ ·È‰ÈÒÓ Ì ÂÓÔ‡ÚËÛË ‰ÂÓ Â›Ó·È ¯·ÌËÏfiÙÂÚË ÌfiÓÔ ·fi ÙËÓ ÔÌ¿‰· ÙˆÓ Ì·ÚÙ‡ÚˆÓ, ·ÏÏ¿ Â›Ó·È ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚË Û˘ÁÎÚÈÙÈο Ì ·È‰È¿ Ì ¯ÚfiÓÈ· ÓÔÛ‹Ì·Ù· fiˆ˜ Ë ÚÂ˘Ì·ÙÔÂȉ‹˜ ·ÚıÚ›Ùȉ· Î·È Ë Î˘ÛÙÈ΋ ›ÓˆÛË (4). ∞ÚÎÂÙ¤˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ·ԉ›ÍÂÈ fiÙÈ Ë ¯·Ì¤ÓË ·˘ÙÔÂÎÙ›ÌËÛË ÙˆÓ ·È‰ÈÒÓ Ì Ӣ¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË Â·Ó·ÔÎÙ¿Ù·È ÌÂÙ¿ ·fi ÂÈÙ˘¯‹ ıÂÚ·›· ÙÔ˘ ÚÔ‚Ï‹Ì·Ùfi˜ ÙÔ˘˜ (23-25). ™‡Ìʈӷ Ì ٷ ·ÔÙÂϤÛÌ·Ù· ÙˆÓ ÌÂÏÂÙÒÓ ·˘ÙÒÓ,

Paediatriki 2001;64:14-24

Ú¤ÂÈ Ó· ‰Ôı› ȉȷ›ÙÂÚË ‚·Ú‡ÙËÙ· ÛÙË ıÂÚ·›· ÙˆÓ ·È‰ÈÒÓ Ì Ӣ¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË. °È·Ù›, fiˆ˜ Â›Ó·È ÁÓˆÛÙfi, Ë ¯·Ì¤ÓË ·˘ÙÔÂÎÙ›ÌËÛË ÛÙË Ó·ڋ ËÏÈΛ· ·ÔÙÂÏ› ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘ ÁÈ· „˘¯ÈΤ˜ ‰È·Ù·Ú·¯¤˜ Î·È ÚÔ‚Ï‹Ì·Ù· ÎÔÈÓˆÓÈ΋˜ ÚÔÛ·ÚÌÔÁ‹˜. ¢ÈÂÚ‡ÓËÛË ∏ ·Ú¯È΋ ÂÎÙ›ÌËÛË ÙˆÓ ·È‰ÈÒÓ Ì Ӣ¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ÂÚÈÏ·Ì‚¿ÓÂÈ ÙÔ ÏÂÙÔÌÂÚ¤˜ ÈÛÙÔÚÈÎfi, ÙË Ê˘ÛÈ΋ ÂͤٷÛË Î·È ÙË ÁÂÓÈ΋, ηıÒ˜ Î·È Î·ÏÏȤÚÁÂÈ· Ô‡ÚˆÓ. ∆Ô ÈÛÙÔÚÈÎfi Ú¤ÂÈ Ó· ÚÔÛ‰ÈÔÚ›˙ÂÈ ÙË Û˘¯ÓfiÙËÙ·, ÙË ‰È¿ÚÎÂÈ· Î·È ÙÔ Â›‰Ô˜ Ù˘ ÂÓÔ‡ÚËÛ˘. ™˘Ó˘¿Ú¯ÔÓÙ· Û˘ÌÙÒÌ·Ù· ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ë̤ڷ˜ fiˆ˜ ·ÎÚ¿ÙÂÈ· Ô‡ÚˆÓ, ÔÍ›· ¤ÂÈÍË, ‰È·ÎÂÎÔÌ̤ÓË Ô‡ÚËÛË Î·È ·‰‡Ó·ÙË ÚÔ‹ Ô‡ÚˆÓ, Ú¤ÂÈ Ó· ÛËÌÂÈÒÓÔÓÙ·È. ∂›Û˘, ‰ÈÂÚ¢ÓÒÓÙ·È Èı·Ó¿ ÚÔËÁÔ‡ÌÂÓ· ÂÂÈÛfi‰È· Ô˘ÚÔÏԛ̈͢, Û˘Ó˘¿Ú¯Ô˘Û· ‰˘ÛÎÔÈÏÈfiÙËÙ· ‹ ÂÁÎfiÚÈÛË Î·È ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ·Ó·ÌÓËÛÙÈÎfi. ∏ Ê˘ÛÈ΋ ÂͤٷÛË ÂÚÈÏ·Ì‚¿ÓÂÈ „ËÏ¿ÊËÛË Ù˘ ÎÔÈÏȷ΋˜ ¯ÒÚ·˜, ÂÈÛÎfiËÛË Î·È „ËÏ¿ÊËÛË Ù˘ ÔÛÊ˘˚΋˜ ¯ÒÚ·˜ ÁÈ· ¤ÏÂÁ¯Ô ·ÓˆÌ·ÏÈÒÓ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘ Î·È ÂÈÛÎfiËÛË ÙˆÓ ¤Íˆ ÁÂÓÓËÙÈÎÒÓ ÔÚÁ¿ÓˆÓ. ∏ ÂͤٷÛË ÔÏÔÎÏËÚÒÓÂÙ·È Ì ÙËÓ ·‰Ú‹ Ó¢ÚÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË Ë ÔÔ›· ÂÚÈÏ·Ì‚¿ÓÂÈ ¤ÏÂÁ¯Ô ÂÚÈÊÂÚÈÎÒÓ Î·È ÂÓ Ùˆ ‚¿ıÂÈ ÙÂÓÔÓÙ›ˆÓ ·ÓÙ·Ó·ÎÏ·ÛÙÈÎÒÓ Î·È ¤ÏÂÁ¯Ô ÂÚÈÓÂ˚΋˜ ·ÈÛıËÙÈÎfiÙËÙ·˜ Î·È ÙÔ˘ ÙfiÓÔ˘ ÙÔ˘ ÛÊÈÁÎÙ‹Ú· ÙÔ˘ ÔÚıÔ‡. °ÂÓÈ΋ Î·È Î·ÏÏȤÚÁÂÈ· Ô‡ÚˆÓ Ú¤ÂÈ Ó· Á›ÓÂÙ·È Û fiÏ· Ù· ·È‰È¿. ™ÙË ÁÂÓÈ΋ Ô‡ÚˆÓ Ú¤ÂÈ Ó· ÂϤÁ¯ÂÙ·È Ì ÚÔÛÔ¯‹ ÙÔ ÂȉÈÎfi ‚¿ÚÔ˜ ηıÒ˜ Î·È Ë Ù˘¯fiÓ ·ÚÔ˘Û›· ۷ί¿ÚÔ˘ ‹ Ï¢ÎÒÌ·ÙÔ˜. ªÂ ‚¿ÛË ÙËÓ ·Ú¯È΋ ·˘Ù‹ ÂÎÙ›ÌËÛË, ÔÈ ·ÛıÂÓ›˜ ‰È·ÎÚ›ÓÔÓÙ·È Û 2 ηÙËÁÔڛ˜: 1) Û ·˘ÙÔ‡˜ Ì ÌË ÂÈÏÂÁ̤ÓË ÂÓÔ‡ÚËÛË Î·È 2) Û ·˘ÙÔ‡˜ Ì ÂÈÏÂÁ̤ÓË ÂÓÔ‡ÚËÛË (∂ÈÎfiÓ· 1) (26). ∆· ·È‰È¿ Ì ·ÚÓËÙÈ΋ Ê˘ÛÈ΋ ÂͤٷÛË Î·È ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ·fi ÙË ÁÂÓÈ΋ Î·È ÙËÓ Î·ÏÏȤÚÁÂÈ· Ô‡ÚˆÓ, ¤¯Ô˘Ó ÌË ÂÈÏÂÁ̤ÓË ÂÓÔ‡ÚËÛË. AÔÙÂÏÔ‡Ó ÙË Û˘ÓÙÚÈÙÈ΋ ÏÂÈÔ„ËÊ›· Î·È ‰ÂÓ ¯Ú‹˙Ô˘Ó ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛ˘. ∞ÓÙ›ıÂÙ·, Ù· ·È‰È¿ Ì ÈÛÙÔÚÈÎfi ÛËÌ·ÓÙÈÎÒÓ ‰˘ÛÏÂÈÙÔ˘ÚÁÈÒÓ Ù˘ Ô‡ÚËÛ˘ (Ôχ Û˘¯Ó‹ Ô‡ÚËÛË, ‰È·ÎÂÎÔÌ̤ÓË Ô‡ÚËÛË, ·ÎÚ¿ÙÂÈ· Ô‡ÚˆÓ, ·ÛıÂÓ‹ ÚÔ‹ Ô‡ÚˆÓ), ÂÁÎfiÚÈÛË, ıÂÙÈ΋ ηÏÏȤÚÁÂÈ· Ô‡ÚˆÓ ‹ ÈÛÙÔÚÈÎfi Ô˘ÚÔÏÔÈÌÒ͈Ó, ·ıÔÏÔÁÈ΋ ÁÂÓÈ΋ Ô‡ÚˆÓ ‹ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ·fi ÙË Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË, ¤¯Ô˘Ó ÂÈÏÂÁ̤ÓË ÂÓÔ‡ÚËÛË. ∆· ·È‰È¿ ·˘Ù¿, Ù· ÔÔ›· ·ÔÙÂÏÔ‡Ó ÙË ÌÂÈÔ„ËÊ›·, ¯Ú‹˙Ô˘Ó ÂÚ·ÈÙ¤Úˆ ÂϤÁ¯Ô˘ Ì ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÓÂÊÚÒӖ·ÛÙˆ˜, ÚÈÓ Î·È ÌÂÙ¿ ÙËÓ Ô‡ÚËÛË, ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· ¡√∫ Î·È ·ÓÈÔ‡Û· ΢ÛÙÂÔÁÚ·Ê›·. √

17


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 18

¶·È‰È·ÙÚÈ΋ 2001;64:14-24

Paediatriki 2001;64:14-24

¤ÏÂÁ¯Ô˜ ·˘Ùfi˜ ·ÔÛÎÔ› ÛÙÔÓ ·ÔÎÏÂÈÛÌfi ·ÔÊÚ·ÎÙÈ΋˜ Ô˘ÚÔ¿ıÂÈ·˜, ˘‰ÚÔÓ¤ÊÚˆÛ˘, ‰Èۯȉԇ˜ Ú¿¯Ë˜, ΢ÛÙÂÔÔ˘ÚËÙËÚÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘, ¿¯˘ÓÛ˘ ÙÔ˘ ÙÔȯÒÌ·ÙÔ˜ Ù˘ ·ÛÙˆ˜ ‹ ˘ÔÏ›ÌÌ·ÙÔ˜ Ô‡ÚˆÓ ÛÙËÓ Î‡ÛÙË. ∆· ÙÂÏÂ˘Ù·›· Â˘Ú‹Ì·Ù·, Èı·ÓÒ˜ Ó· ··ÈÙ‹ÛÔ˘Ó Ô˘ÚÔ‰˘Ó·ÌÈÎfi ¤ÏÂÁ¯Ô Ù˘ ·ÛÙˆ˜. ™Ù· ·È‰È¿ Ì ÂÈÏÂÁ̤ÓË Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË, ÌÈ· ·‰Ú‹ ÂÎÙ›ÌËÛË Ù˘ ÓÂÊÚÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ Ì ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ Ô˘Ú›·˜ Î·È Ù˘ ÎÚ·ÙÈÓ›Ó˘ ÔÚÔ‡, ÎÚ›ÓÂÙ·È ··Ú·›ÙËÙË. TÂÏÂ˘Ù·›·, Û˘ÓÈÛÙ¿Ù·È Ë ·ÎÚÈ‚‹˜ ηٷ̤ÙÚËÛË ·fi ÙÔ˘˜ ÁÔÓ›˜, ÙÔ˘ fiÁÎÔ˘ ÙˆÓ Ô‡ÚˆÓ Î¿ı ԇÚËÛ˘, ÙfiÛÔ ÙËÓ Ë̤ڷ fiÛÔ Î·È ÙË Ó‡¯Ù· ÁÈ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 48 ÒÚ˜. ªÂ ÙÔÓ ÙÚfiÔ ·˘Ùfi, ÌÔÚ› Ó· ÂÎÙÈÌËı› Ë ÏÂÈÙÔ˘ÚÁÈ΋ ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜ Î·È Ë ·ÚÔ˘Û›· ‹ fi¯È Ó˘¯ÙÂÚÈÓ‹˜ ÔÏ˘Ô˘Ú›·˜, ‰Â‰Ô̤ӷ Ô˘ ηıÔ‰ËÁÔ‡Ó ÙËÓ ÂÚ·ÈÙ¤Úˆ ‰ÈÂÚ‡ÓËÛË Î·È ıÂÚ·›· (7,27). ∏ ÏÂÈÙÔ˘ÚÁÈ΋ ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜ ˘ÔÏÔÁ›˙ÂÙ·È Î·Ù¿ ÚÔÛ¤ÁÁÈÛË Ì ÙÔÓ Ù‡Ô: xˆÚË-

ÙÈÎfiÙËÙ· ·ÛÙˆ˜ (ml) = [ËÏÈΛ· (¤ÙË) +2] x 32ml (± 64ml SD) (2,28). ¡˘¯ÙÂÚÈÓ‹ ÔÏ˘Ô˘Ú›· ÔÚ›˙ÂÙ·È, fiÙ·Ó Ô ÏfiÁÔ˜ fiÁÎÔ˘ Ô‡ÚˆÓ Ë̤ڷ˜ ÚÔ˜ fiÁÎÔ Ô‡ÚˆÓ Ó‡¯Ù·˜ Â›Ó·È <1 (29,30). £ÂÚ·›· ∏ ηÏÔ‹ı˘ ʇÛË Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Î·È Ô ˘„ËÏfi˜ ÂÙ‹ÛÈÔ˜ Ú˘ıÌfi˜ Ù˘ ·˘ÙfiÌ·Ù˘ ˘Ô¯ÒÚËÛ˘, ÂÈ‚¿ÏÏÔ˘Ó Î¿ı ›‰Ô˜ ıÂÚ·¢ÙÈ΋˜ ·Ú¤Ì‚·Û˘ Ó· ÛÙÂÚÂ›Ù·È ÔÔÈÔ˘‰‹ÔÙ ÎÈÓ‰‡ÓÔ˘ ‹ ÛÔ‚·ÚÒÓ ·ÓÂÈı‡ÌËÙˆÓ ·ÚÂÓÂÚÁÂÈÒÓ. ∂ÈϤÔÓ, οı ıÂÚ·¢ÙÈÎfi ̤ÙÚÔ ıˆÚÂ›Ù·È ÈηÓÔÔÈËÙÈÎfi, ÌfiÓÔ Â¿Ó ÙÔ ·ÔÙ¤ÏÂÛÌ¿ ÙÔ˘ Â›Ó·È ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚÔ ·fi ÙÔÓ ·Ó·ÌÂÓfiÌÂÓÔ Ú˘ıÌfi ·˘ÙfiÌ·Ù˘ ˘Ô¯ÒÚËÛ˘ Ù˘ ÂÓÔ‡ÚËÛ˘. ∏ ıÂÚ·›· Ú¤ÂÈ Ó· ÚÔÙ›ÓÂÙ·È ÌfiÓÔ ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ ÙÔ ·È‰› ‹/Î·È ÔÈ ÁÔÓ›˜ ÙÔ˘ ÂÈı˘ÌÔ‡Ó Ó· ··ÏÏ·ÁÔ‡Ó ·fi ÙÔ ÂÓÔ¯ÏËÙÈÎfi ·˘Ùfi Úfi‚ÏËÌ· Î·È ÙÔ‡ÙÔ ÁÈ·Ù› Ë Û˘ÓÂÚÁ·Û›· ÙÔ˘˜ ·ÔÙÂÏ› ÙÔÓ ·ÎÚÔÁˆÓÈ·›Ô Ï›ıÔ Ù˘

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∞ÚÓËÙÈ΋ Ê˘ÛÈ΋ ÂͤٷÛË

£ÂÙÈÎfi ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi

∞ÚÓËÙÈÎfi ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi

(.¯. Ô˘ÚÔÏԛ̈ÍË, ÂÁÎfiÚÈÛË)

°ÂÓÈ΋ Î·È Î·ÏÏȤÚÁÂÈ· Ô‡ÚˆÓ ¡Â˘ÚÔÏÔÁÈ΋ ÂͤٷÛË

∞ÚÓËÙÈ΋

£ÂÙÈ΋

¢ÂÓ ··ÈÙÂ›Ù·È ÂÚ·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô˜

∂ÈÎfiÓ· 1. ™¯ËÌ·ÙÈ΋ ·ÂÈÎfiÓÈÛË Ù˘ ‰ÈÂÚ‡ÓËÛ˘ Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘.

18

U/S ÓÂÊÚÒÓ-·ÛÙ˘ (ÚÈÓ Î·È ÌÂÙ¿ Ô‡ÚËÛË) ∞Ï‹ ¡√∫ ∞ÓÈÔ‡Û· ΢ÛÙÂÔÁÚ·Ê›· √˘Ú›· Î·È ÎÚ·ÙÈÓ›ÓË ÔÚÔ‡ √˘ÚÔ‰˘Ó·ÌÈÎfi˜ ¤ÏÂÁ¯Ô˜; ¡Â˘ÚÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË;


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 19

¶·È‰È·ÙÚÈ΋ 2001;64:14-24

Paediatriki 2001;64:14-24

ÂÈÙ˘¯Ô‡˜ ηٿÏË͢ ÔÔÈÔ˘‰‹ÔÙ ıÂÚ·¢ÙÈÎÔ‡ ̤ÙÚÔ˘ (31). ∆Ô ıÂÚ·¢ÙÈÎfi ·ÔÙ¤ÏÂÛÌ· ηٷÁÚ¿ÊÂÙ·È ·fi ÙÔ ·È‰› Û ËÌÂÚÔÏfiÁÈÔ fiÔ˘ ÛËÌÂÈÒÓÔÓÙ·È ÔÈ ÛÙÂÁÓ¤˜ Ó‡¯Ù˜ Î·È ·ÍÈÔÏÔÁÂ›Ù·È ·fi ÙÔÓ ıÂÚ¿ÔÓÙ· È·ÙÚfi ·Ó¿ Ù·ÎÙ¿ ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù·, ÔfiÙÂ Î·È ÙÔ ·È‰› ÂÈ‚Ú·‚‡ÂÙ·È Î·È ÂÓı·ÚÚ‡ÓÂÙ·È ÁÈ· ÙËÓ ÚÔÛ¿ıÂÈ· Î·È ÙÔ ·Ó¿ÏÔÁÔ ·ÔÙ¤ÏÂÛÌ·. ∆· ÔÏÏ¿ ıÂÚ·¢ÙÈο ̤ÙÚ·, Ê·Ú̷΢ÙÈο Î·È ÌË, Ô˘ ÚÔÙ›ÓÔÓÙ·È, Ì·ÚÙ˘ÚÔ‡Ó fiÙÈ Î·Ó¤Ó· ̤ÙÚÔ ıÂÚ·›·˜ ‰ÂÓ ıˆÚÂ›Ù·È È‰·ÓÈÎfi ÁÈ· ÙË Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË. ∞˘Ùfi ÂȂ‚·ÈÒÓÂÙ·È ·fi ÙÔ ‰È·ÊÔÚÂÙÈÎfi ÙÚfiÔ ıÂÚ·¢ÙÈ΋˜ ·Ú¤Ì‚·Û˘ Ô˘ ÂÊ·ÚÌfi˙ÂÙ·È Û ‰È¿ÊÔÚ˜ ¯ÒÚ˜. ™ÙȘ ∏.¶.∞. ÁÈ· ·Ú¿‰ÂÈÁÌ·, Ë Û˘¯ÓfiÙÂÚ· ÚÔÙÂÈÓfiÌÂÓË ıÂÚ·›· Â›Ó·È Ë ‰ÂÛÌÔÚÂÛÛ›ÓË, ÂÓÒ Ë Û˘Û΢‹ ·Ê‡ÓÈÛ˘ ¯ÚËÛÈÌÔÔÈÂ›Ù·È Û ÔÛÔÛÙfi <5% (26,32). ™ÙË °·ÏÏ›·, ÙÔ 20% ÙˆÓ È·ÙÚÒÓ ‰ÂÓ ÚÔÙ›ÓÂÈ Î·Ì›· ıÂÚ·›·. ∏ ÏÂÈÔ„ËÊ›· ÚÔÙ›ÓÂÈ ·Ú·ÎÔÏÔ‡ıËÛË Î·È ·ÏÏ·Á‹ ÙÔ˘ ÙÚfiÔ˘ ˙ˆ‹˜. ∞fi ·˘ÙÔ‡˜ Ô˘ ÚÔÙ›ÓÔ˘Ó ıÂÚ·›·, ÙÔ 48% ¯ÔÚËÁÔ‡Ó Ô͢‚Ô˘ÙÈÓ›ÓË, ÙÔ 22% ‰ÂÛÌÔÚÂÛÛ›ÓË, ÙÔ 12% ÈÌÈÚ·Ì›ÓË Î·È ÙÔ 5% Û˘Û΢‹ ·Ê‡ÓÈÛ˘ (33). ∞ÓÙ›ıÂÙ·, ÛÙÔ ∏ӈ̤ÓÔ µ·Û›ÏÂÈÔ, Ë Û˘Û΢‹ ·Ê‡ÓÈÛ˘ Î·È Ë ‰ÂÛÌÔÚÂÛÛ›ÓË Â›Ó·È ÔÈ Û˘¯ÓfiÙÂÚ· ÚÔÙÂÈÓfiÌÂÓ˜ ıÂÚ·›˜ (34). °ÂÓÈο ÌË Ê·Ú̷΢ÙÈο ̤ÙÚ· fiˆ˜ ÂÚÈÔÚÈÛÌfi˜ Ù˘ Ï‹„˘ ˘ÁÚÒÓ ÙÔ ‚Ú¿‰˘, ·Ê‡ÓÈÛË ÙÔ˘ ·È‰ÈÔ‡ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜ ÁÈ· Ó· Ô˘Ú‹ÛÂÈ ‹ ·ÎfiÌË ¿ÛÎËÛË „˘¯ÔÏÔÁÈ΋˜ ‚›·˜ ÛÙÔ ·È‰›, ‰ÂÓ ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ Û˘Ì‚¿ÏÏÔ˘Ó ÛÙËÓ ˘Ô¯ÒÚËÛË Ù˘ ÂÓÔ‡ÚËÛ˘ (1,35). ¶¿ÓÙˆ˜, Ô ÂÚÈÔÚÈÛÌfi˜ Ù˘ Ï‹„˘ ˘ÁÚÒÓ ÙË Ó‡¯Ù· Î·È Ë Ô‡ÚËÛË ·Ì¤Ûˆ˜ ÚÈÓ ÙËÓ Î·Ù¿ÎÏÈÛË, ÚÔÙ›ÓÔÓÙ·È ·fi ·ÚÎÂÙÔ‡˜ Ô˘ ·Û¯ÔÏÔ‡ÓÙ·È Ì ÙÔ ı¤Ì· Û·Ó Û˘ÌÏË-

ڈ̷ÙÈÎfi ıÂÚ·¢ÙÈÎfi ̤ÙÚÔ ÔÔÈ·Û‰‹ÔÙ ¿ÏÏ˘ ·Ú¤Ì‚·Û˘, ȉȷ›ÙÂÚ· fiÙ·Ó ÛÙÔ ·È‰› ¯ÔÚËÁÂ›Ù·È ‰ÂÛÌÔÚÂÛÛ›ÓË (32). √‰ËÁ›Â˜ ÁÈ· ·˘ÍË̤ÓË ÚfiÛÏË„Ë ˘ÁÚÒÓ Î·È ·Ó·‚ÔÏ‹ ÙˆÓ Ô˘Ú‹ÛÂˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ë̤ڷ˜ Ì ÛÎÔfi ÙËÓ ÚÔԉ¢ÙÈ΋ ·‡ÍËÛË ÙÔ˘ fiÁÎÔ˘ ÙˆÓ Ô‡ÚˆÓ ÎÈ ÂÈÌ‹Î˘ÓÛË ÙˆÓ ÌÂÛԉȷÛÙËÌ¿ÙˆÓ ÌÂٷ͇ ÙˆÓ Ô˘Ú‹ÛˆÓ, ‰ÂÓ Ê·›ÓÂÙ·È Ó· ¤¯Ô˘Ó ηÌÈ¿ ıÂÚ·¢ÙÈ΋ ·Í›·, ·ÚfiÏÔ Ô˘ Ë ÏÂÈÙÔ˘ÚÁÈ΋ ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜ ·˘Í¿ÓÂÙ·È (36). ∏ ÂÈÏÔÁ‹ ÙÔ˘ ›‰Ô˘˜ Ù˘ ıÂÚ·›·˜ Ô˘ ı· ·ÎÔÏÔ˘ıËı› Á›ÓÂÙ·È ·ÊÔ‡ ÏËÊı› ÛÔ‚·Ú¿ ˘’ fi„ÈÓ Ë ÂÈı˘Ì›· ÙˆÓ ÁÔÓ¤ˆÓ ‹/Î·È ÙÔ˘ ·È‰ÈÔ‡, ÛÙÔ˘˜ ÔÔ›Ô˘˜ ÂÍËÁÔ‡ÓÙ·È ÏÂÙÔÌÂÚÒ˜ Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· Î·È Ù· ÌÂÈÔÓÂÎÙ‹Ì·Ù· οı ÌÂıfi‰Ô˘. √È Î˘ÚÈfiÙÂÚ˜ ¢ڤˆ˜ ·Ô‰ÂÎÙ¤˜ ıÂÚ·›˜ Ù˘ Ó˘ÎÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Â›Ó·È Ë Û˘Û΢‹ ·Ê‡ÓÈÛ˘, Ë ‰ÂÛÌÔÚÂÛÛ›ÓË Î·È Ë ÈÌÈÚ·Ì›ÓË. ™‡ÁÎÚÈÛË ÙˆÓ ıÂÚ·¢ÙÈÎÒÓ ·˘ÙÒÓ Ì¤ÙÚˆÓ ·Ú·Ù›ıÂÙ·È ÛÙÔÓ ¶›Ó·Î· 1. ™˘Û΢‹ ·Ê‡ÓÈÛ˘ ∏ ̤ıÔ‰Ô˜ ·˘Ù‹ ¯ÚËÛÈÌÔÔÈ‹ıËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ ÙÔ 1938 ·fi ÙÔ˘˜ Mowrer Î·È Mowrer. ∏ Û˘Û΢‹ ÛÙË Û˘Ó¤¯ÂÈ· ÙÂÏÂÈÔÔÈ‹ıËÎÂ Î·È Û‹ÌÂÚ· ‰È·Ù›ıÂÙ·È ÛÙËÓ ·ÁÔÚ¿ Û ÌÈÎÚfi Ú·ÎÙÈÎfi ̤ÁÂıÔ˜ Î·È Û¯ÂÙÈο ¯·ÌËÏfi ÎfiÛÙÔ˜. ∞ÔÙÂÏÂ›Ù·È ·fi ¤Ó· ÌÈÎÚfi ·ÈÛıËÙ‹Ú· Ô˘ ÂÊ·ÚÌfi˙ÂÙ·È ÛÙÔ ÂÛÒÚÔ˘¯Ô ÎÈ ·fi ¤Ó· ÌÈÎÚfi ͢ÓËÙ‹ÚÈ Ô˘ ÂÊ·ÚÌfi˙ÂÙ·È ÛÙÔ ¿Óˆ ̤ÚÔ˜ Ù˘ ˘Ù˙¿Ì·˜ ÙÔ˘ ·È‰ÈÔ‡. ŸÙ·Ó Ô ·ÈÛıËÙ‹Ú·˜ ‚Ú·¯Â›, ÙfiÙ ÙÔ Í˘ÓËÙ‹ÚÈ ÎÙ˘¿ÂÈ Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ê‡ÓÈÛË ÙÔ˘ ·È‰ÈÔ‡ Î·È ÙË ‰È·ÎÔ‹

¶›Ó·Î·˜ 1. ™‡ÁÎÚÈÛË ÙˆÓ Î˘ÚÈÔÙ¤ÚˆÓ ıÂÚ·¢ÙÈÎÒÓ Ì¤ÙÚˆÓ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘. ™˘Û΢‹ ·Ê‡ÓÈÛ˘ ∞Ú¯È΋ ·ÓÙ·fiÎÚÈÛË

¢ÂÛÌÔÚÂÛÛ›ÓË

πÌÈÚ·Ì›ÓË

65-100%

10-70%

10-40%

∂ÈÙ˘¯‹˜ ·ÓÙ·fiÎÚÈÛË Û ۯ¤ÛË Ì placebo

13,3 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË

4,5 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË

4,2 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚË

¶ÔÛÔÛÙfi ˘ÔÙÚÔ‹˜ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ ıÂÚ·›·˜

20-30%

30-45%

40-60%

3-6 Ì‹Ó˜

3-6 Ì‹Ó˜

3-6 Ì‹Ó˜

∫·Ì›·

™¿ÓȘ

™¿ÓȘ ·ÏÏ¿ ‰˘Ó·ÙfiÓ ÛÔ‚·Ú¤˜

ªÂÙ¿ ·fi 2-3 ‚‰ÔÌ¿‰Â˜

ÕÌÂÛË

ÕÌÂÛË

¢È¿ÚÎÂÈ· ıÂÚ·›·˜ ∞ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ ∂ÌÊ¿ÓÈÛË ·ÔÙÂϤÛÌ·ÙÔ˜

19


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 20

¶·È‰È·ÙÚÈ΋ 2001;64:14-24

Ù˘ Ô‡ÚËÛ˘ (26). √ Ì˯·ÓÈÛÌfi˜ Ì ÙÔÓ ÔÔ›Ô Ë Û˘Û΢‹ ·Ó·ÛÙ¤ÏÏÂÈ ÙËÓ Ô‡ÚËÛË ‰ÂÓ Â›Ó·È ·ÎfiÌË Î·È Û‹ÌÂÚ· Ï‹Úˆ˜ ηٷÓÔËÙfi˜. ¶ÈÛÙ‡ÂÙ·È fiÙÈ ÔÊ›ÏÂÈ ÙÔÓ ÙÚfiÔ ‰Ú¿Û˘ Ù˘ ÛÙÔ Û˘Ó‰˘·ÛÌfi ÙÔ˘ ÂÚÂı›ÛÌ·ÙÔ˜ Ô˘ ÚÔηÏÂ›Ù·È ·fi ÙË ÁÂÌ¿ÙË Î‡ÛÙË, Ì ÙÔ ÂÚ¤ıÈÛÌ· Ù˘ ·Ê‡ÓÈÛ˘ Ô˘ ·Ú¿ÁÂÙ·È ·fi ÙË Û˘Û΢‹ fiÙ·Ó ·Ú¯›ÛÂÈ Ë Ô‡ÚËÛË. ∆ÂÏÈο, ÙÔ ÂÚ¤ıÈÛÌ· Ù˘ ÁÂÌ¿Ù˘ ·ÛÙˆ˜ ÂÈÙ˘Á¯¿ÓÂÈ Ó· ·Ê˘Ó›ÛÂÈ ·ÓÙ·Ó·ÎÏ·ÛÙÈο ÙÔ ·È‰› ÚÔÙÔ‡ Ô˘Ú‹ÛÂÈ ‹ ·Ó·ÛÙ¤ÏÏÂÈ ·˘ÙfiÌ·Ù· ÙËÓ Ô‡ÚËÛË ¯ˆÚ›˜ ÙÔ ·È‰› Ó· ·Ê˘ÓÈÛı› (2). ∏ ÂÈÙ˘¯›· Ù˘ ÌÂıfi‰Ô˘ ·˘Ù‹˜ ÛÙËÚ›˙ÂÙ·È Û ÌÂÁ¿ÏÔ ‚·ıÌfi ÛÙË Î·Ï‹ Û˘ÓÂÚÁ·Û›· ÙÔ˘ ·È‰ÈÔ‡ Î·È ÙˆÓ ÁÔÓ¤ˆÓ. ªÂÚÈο ·È‰È¿ ‰ÂÓ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙÔÓ ‹¯Ô Ù˘ Û˘Û΢‹˜ ÎÈ ÂÔ̤ӈ˜ Ú¤ÂÈ Ó· ˘¿Ú¯ÂÈ Î¿ÔÈÔ˜ ÂÓ‹ÏÈÎÔ˜ Ô ÔÔ›Ô˜, fiÙ·Ó ÎÙ˘‹ÛÂÈ Ë Û˘Û΢‹, Ó· ·Ê˘Ó›ÛÂÈ ÙÔ ·È‰›. ŸÙ·Ó ÙÔ ·È‰› ͢Ó‹ÛÂÈ Ú¤ÂÈ Ó· ¿ÂÈ Ó· Ô˘Ú‹ÛÂÈ (37). ∏ ÂÈÙ˘¯‹˜ ·ÓÙ·fiÎÚÈÛË Ù˘ ÌÂıfi‰Ô˘ Î˘Ì·›ÓÂÙ·È ·fi 65-100% ÌÂÙ¿ ·fi 4-6 Ì‹Ó˜ ıÂÚ·›·˜ (1), Ì ̤ÛÔ fiÚÔ Û 2 ÌÂÁ¿Ï˜ Û˘ÁÎÂÓÙÚˆÙÈΤ˜ ÌÂϤÙ˜ 82% (1) Î·È 67,7% ·ÓÙ›ÛÙÔȯ· (38). ∏ Û˘Û΢‹ Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË Â¿Ó Ë ÂÓÔ‡ÚËÛË Û˘Ì‚·›ÓÂÈ Î¿ı Ӈ¯Ù·. £ÂˆÚÂ›Ù·È ÏÈÁfiÙÂÚÔ ·ÔÙÂÏÂÛÌ·ÙÈ΋ fiÛÔ ÌÈÎÚfiÙÂÚÔ˜ Â›Ó·È Ô ·ÚÈıÌfi˜ ÙˆÓ ÂÓÔ˘ÚËÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ, ÂÓÒ ÙÔ ·ÔÙ¤ÏÂÛÌ· Ù˘ ‰ÂÓ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ËÏÈΛ· ‹ ÙÔ Ê‡ÏÔ (37). ∏ ‰È¿ÚÎÂÈ· Ù˘ ıÂÚ·›·˜ Ì ÙË Û˘Û΢‹ Î˘Ì·›ÓÂÙ·È ·fi 3-6 Ì‹Ó˜, ÂÓÒ Ë ıÂÚ·›· Ú¤ÂÈ Ó· Û˘Ó¯›˙ÂÙ·È ÙÔ˘Ï¿¯ÈÛÙÔÓ Ì¤¯ÚÈ Ó· ÂÈÙ¢¯ıÔ‡Ó 4 Û˘Ó¯fiÌÂÓ˜ ÛÙÂÁÓ¤˜ ‚‰ÔÌ¿‰Â˜ (1,26). ∏ Û˘Û΢‹ ·Ê‡ÓÈÛ˘ ¤¯ÂÈ 13,3 ÊÔÚ¤˜ ÌÂÁ·Ï‡ÙÂÚ˜ Èı·ÓfiÙËÙ˜ Ó· ÚÔηϤÛÂÈ 14 Û˘Ó¯fiÌÂÓ˜ ÛÙÂÁÓ¤˜ Ó‡¯Ù˜ Û ۯ¤ÛË Ì ÌË ıÂÚ·›·, ¤Ó·ÓÙÈ 4,5 ÊÔÚ¤˜ Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ Î·È 4,2 ÊÔÚ¤˜ Ù˘ ÈÌÈÚ·Ì›Ó˘ (34). ÀÔÙÚÔ‹ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ ıÂÚ·›·˜ ÌÔÚ› Ó· ·Ú·ÙËÚËı› ÛÙÔ 20-30% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (39) Î·È ·ÓÙÈÌÂÙˆ›˙ÂÙ·È Ì Â·Ó¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Î·È Â› ÌË ÈηÓÔÔÈËÙÈÎÔ‡ ·ÔÙÂϤÛÌ·ÙÔ˜, ÚÔÛı‹ÎË ‰Â‡ÙÂÚ˘ ıÂÚ·›·˜ (Û˘Ó‹ıˆ˜ ‰ÂÛÌÔÚÂÛÛ›Ó˘). ¶·Ú¿ Ù· Û·Ê‹ ÏÂÔÓÂÎÙ‹Ì·Ù·, Ë ·Ó·ÊÂÚı›۷ ̤ıÔ‰Ô˜ ‰ÂÓ Î·Ù¤¯ÂÈ ‰ÈÂıÓÒ˜ ÙËÓ ·Ó¿ÏÔÁË ı¤ÛË ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘, ·ÚfiÏÔ Ô˘ ÚÔÙ›ÓÂÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ˆ˜ Ë ıÂÚ·›· ÚÒÙ˘ ÂÎÏÔÁ‹˜, ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ·fi οı ¿ÏÏË (2,40-42). ∏ Û˘Û΢‹ ÛÙÂÚÂ›Ù·È Î¿ı ·ÚÂÓ¤ÚÁÂÈ·˜ ÁÈ·Ù› ‰ÂÓ Â›Ó·È Ê¿ÚÌ·ÎÔ, ¤¯ÂÈ ÌÂÁ¿ÏÔ ÔÛÔÛÙfi ÂÈÙ˘¯›·˜ Î·È Û¯ÂÙÈο ¯·ÌËÏfi ÎfiÛÙÔ˜. ∞ÓÙ›ıÂÙ·, ÛÙ· ÌÂÈÔÓÂÎÙ‹Ì·Ù· Ù˘ ·Ó·Ê¤ÚÂÙ·È ÙÔ fi¯È ¿ÌÂÛÔ ·ÔÙ¤ÏÂÛÌ· ÙÔ ÔÔ›Ô Â›Ó·È ÔÚ·Ùfi ÌÂÙ¿ ·fi ¯Ú‹ÛË 2-3 ‚‰ÔÌ¿‰ˆÓ Î·È ÙÔ ˘„ËÏfi ÔÛÔÛÙfi ˘ÔÙÚÔ‹˜ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ ıÂÚ·›·˜. ∂›Û˘, ÌÂÈÔÓ¤ÎÙËÌ· ·ÔÙÂÏ› Ë ÂÓfi¯ÏËÛË Ô˘ ÚÔηÏ› Ë ·fiÙÔÌË ·Ê‡ÓÈÛË ÙÔ˘ ·È‰ÈÔ‡ Î·È Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜, ȉȷ›ÙÂÚ·

20

Paediatriki 2001;64:14-24

fiÙ·Ó ˘¿Ú¯Ô˘Ó Î·È ¿ÏÏ· ·È‰È¿ ÛÙÔ ›‰ÈÔ ‰ˆÌ¿ÙÈÔ. ∆ÂÏÂ˘Ù·›·, ¤¯ÂÈ ·Ó·Ù˘¯ı› Û˘Û΢‹ Ë ÔÔ›· ·ÔÙÂÏÂ›Ù·È ·fi ¤Ó·Ó ·Ïfi ˘ÂÚ˯ÔÁÚ¿ÊÔ Î·È ¤Ó·Ó ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊÔ (18). ∏ Û˘Û΢‹ ηٷÁÚ¿ÊÂÈ ˘ÂÚ˯ÔÁÚ·ÊÈο ÙËÓ Ï‹ÚˆÛË Ù˘ Ô˘ÚÔ‰fi¯Ô˘ ·ÛÙˆ˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, Ì ÙË ‚Ô‹ıÂÈ· ÂÓfi˜ ·ÈÛıËÙ‹Ú· Ô ÔÔ›Ô˜ ÛÙÂÚÂÒÓÂÙ·È ÛÙËÓ Î·ÙÒÙÂÚË ÎÔÈÏȷ΋ ¯ÒÚ·. ¶·Ú¿ÏÏËÏ· ηٷÁÚ¿ÊÂÙ·È Ë ÂÁÎÂÊ·ÏÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ·. ∆ËÓ Ï‹ÚˆÛË Ù˘ Ô˘ÚÔ‰fi¯Ô˘ ·ÛÙˆ˜, ·ÎÔÏÔ˘ı› ÌÂÙ·ÙÚÔ‹ ÙÔ˘ ‚·ı¤ˆ˜ ‡ÓÔ˘ Û ÂÏ·ÊÚ‡ Î·È Ô Û˘Ó‰˘·ÛÌfi˜ ·˘Ùfi˜ ÂÓÂÚÁÔÔÈ› ÙË Û˘Û΢‹, Ë ÔÔ›· ÎÙ˘¿ÂÈ Î·È ·Ê˘Ó›˙ÂÈ ÙÔÓ ·ÛıÂÓ‹ ÚÈÓ ·˘Ùfi˜ Ô˘Ú‹ÛÂÈ. ∏ Û˘Û΢‹ Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙË ıÂÚ·›· Ù˘ ÂÓÔ‡ÚËÛ˘ Ù‡Ô˘ π Î·È ·Ó·Ì¤ÓÂÙ·È Ó· ΢ÎÏÔÊÔÚ‹ÛÂÈ ÌÂÏÏÔÓÙÈο ÛÙÔ ÂÌfiÚÈÔ. ¢ÂÛÌÔÚÂÛÛ›ÓË ∏ ‰ÂÛÌÔÚÂÛÛ›ÓË, ¤Ó· Û˘ÓıÂÙÈÎfi ·Ó¿ÏÔÁÔ Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘, ¯ÚËÛÈÌÔÔÈÂ›Ù·È Â˘Ú¤ˆ˜ ·fi ÔÏÏ¿ ¯ÚfiÓÈ· ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘. Ÿˆ˜ ·Ó·Ê¤ÚıËΠÈÔ ¿Óˆ, Ù· ÂÚÈÛÛfiÙÂÚ· ÂÓÔ˘ÚËÙÈο ¿ÙÔÌ· ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÂȈ̤ÓË ¤ÎÎÚÈÛË ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜ Û ۯ¤ÛË Ì ٷ ÌË ÂÓÔ˘ÚËÙÈο. ∞˘Ùfi Ô‰ËÁ› Û ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ Ô‡ÚˆÓ, Ë ÔÔ›· fiÙ·Ó ˘Âڂ› ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜, ÚÔηÏ› ÂÓÔ‡ÚËÛË. ∏ ¯ÔÚ‹ÁËÛË Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ ÌÂÈÒÓÂÈ ÙÔÓ fiÁÎÔ ÙˆÓ ·Ú·ÁÔÌ¤ÓˆÓ Ô‡ÚˆÓ Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·ÔÙÚÔ‹ Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘. °È· ÙÔ ÏfiÁÔ ·˘Ùfi, Ë ıÂÚ·›· ÎÚ›ÓÂÙ·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚË ÛÙ· ·È‰È¿ Ì Ӣ¯ÙÂÚÈÓ‹ ÔÏ˘Ô˘Ú›· (43,44). ∏ ‰ÂÛÌÔÚÂÛÛ›ÓË ‰È·Ù›ıÂÙ·È ÛÙÔ ÂÌfiÚÈÔ ˘fi ÌÔÚÊ‹ spray ÁÈ· ÂÓ‰ÔÚÈÓÈ΋ ¯Ú‹ÛË Î·È ÚfiÛÊ·Ù· ˘fi ÌÔÚÊ‹ ¯·ÈÒÓ ÁÈ· ¯ÔÚ‹ÁËÛË ·fi ÙÔ ÛÙfiÌ·. ∞ÔÚÚÔÊ¿Ù·È Ù·¯¤ˆ˜ ·fi ÙÔ ÚÈÓÈÎfi Î·È Á·ÛÙÚÈÎfi ‚ÏÂÓÓÔÁfiÓÔ, Ë ‚ÈԉȷıÂÛÈÌfiÙËÙ¿ Ù˘, fï˜, ÌÂÈÒÓÂÙ·È Î·Ù¿ 90% fiÙ·Ó ·ÔÚÚÔÊ¿Ù·È ·fi ÙÔ ÛÙÔÌ¿¯È Î·È ÛÙËÓ ÂÚ›ÙˆÛË ·˘Ù‹ ¯ÔÚËÁÂ›Ù·È Û ÌÂÁ·Ï‡ÙÂÚË ‰fiÛË. ∂ÌÊ·Ó›˙ÂÈ Ì¤ÁÈÛÙË Û˘ÁΤÓÙÚˆÛË ÛÙÔ Ï¿ÛÌ· ÂÚ›Ô˘ 50-60 ÏÂÙ¿ ÌÂÙ¿ ÙË Ï‹„Ë Ù˘, Ô ¯ÚfiÓÔ˜ ËÌ›ÛÂÈ·˜ ˙ˆ‹˜ Â›Ó·È 4-6 ÒÚ˜ Î·È Ë ‰È¿ÚÎÂÈ· ‰Ú¿Û˘ Ù˘ Â›Ó·È ÂÚ›Ô˘ 10 ÒÚ˜ (45). ∏ ·Ú¯È΋ Û˘ÓÈÛÙÒÌÂÓË ÂÓ‰ÔÚÈÓÈ΋ ‰fiÛË Â›Ó·È 10Ìg ÂÚ›Ô˘ 1 ÒÚ· ÚÈÓ ÙËÓ Î·Ù¿ÎÏÈÛË, Ë ÔÔ›· ·˘Í¿ÓÂÙ·È ·Ó¿ÏÔÁ· Ì ÙËÓ ·ÓÙ·fiÎÚÈÛË Î¿ı 3 Ë̤Ú˜ ̤¯ÚÈ Ù· 40Ìg (46) Ô˘ Â›Ó·È Ë Ì¤ÁÈÛÙË ¯ÔÚËÁÔ‡ÌÂÓË ·ÏÏ¿ Î·È ÈÔ Û˘ÓËıÈṲ̂ÓË ‰fiÛË (47). ∆Ô ·ÔÙ¤ÏÂÛÌ· Ù˘ ¯ÔÚ‹ÁËÛ˘ Ù˘ ‰ÂÛÌÔÚÂÛÛ›Ó˘ Â›Ó·È Ê·ÓÂÚfi ·fi ÙȘ ÚÒÙ˜ Ë̤Ú˜. ∏ ·Ú¯È΋ ·ÓÙ·fiÎÚÈÛË ÛÙÔ Ê¿ÚÌ·ÎÔ Î˘Ì·›ÓÂÙ·È Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ ·fi 10-70% (1), ÂÓÒ Ï‹Ú˘ ÂÈÙ˘¯›· ÛÙË ıÂÚ·›· ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 10-30%. √È Moffatt


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 21

¶·È‰È·ÙÚÈ΋ 2001;64:14-24

Î·È Û˘Ó, ÁÈ· Ó· ÂÎÙÈÌ‹ÛÔ˘Ó ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙÔ˘ Ê·ÚÌ¿ÎÔ˘, ·Ó¤Ï˘Û·Ó 18 ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÌÂϤÙ˜ ÌÂ Û˘ÓÔÏÈο 580 ·È‰È¿ Ì Ӣ¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ÛÙ· ÔÔ›· ¯ÔÚËÁ‹ıËΠ‰ÂÛÌÔÚÂÛÛ›ÓË (41). ¶·Ú¿ ÙË ÛËÌ·ÓÙÈ΋ Ì›ˆÛË ÙˆÓ ÂÓÔ˘ÚËÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ ÌfiÓÔ ÙÔ 25% ·ÚÔ˘Û›·Û Ï‹ÚË ·ÓÙ·fiÎÚÈÛË, ÂÓÒ Û ¿ÏÏË ·ÚfiÌÔÈ· ÌÂϤÙË ÙÔ ÔÛÔÛÙfi Ï‹ÚÔ˘˜ ÂÈÙ˘¯›·˜ ‹Ù·Ó 21% (40). ™Â ÚfiÛÊ·ÙË ÌÂϤÙË ·fi ÙË ™Ô˘Ë‰È΋ √Ì¿‰· ∂ÓÔ‡ÚËÛ˘, ÂÎÙÈÌ‹ıËÎÂ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ Ì·ÎÚfi¯ÚÔÓ˘ ıÂÚ·›·˜ (1 ¯ÚfiÓÔ) Ì ‰ÂÛÌÔÚÂÛÛ›ÓË. ™‡Ìʈӷ Ì ÙÔ ·ÔÙ¤ÏÂÛÌ·, ÙÔ 61% ÙˆÓ ·È‰ÈÒÓ ·ÚÔ˘Û›·Û·Ó ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· (>50% Ì›ˆÛË ÙˆÓ ‚ÚÂÁÌ¤ÓˆÓ ÂÂÈÛÔ‰›ˆÓ), ÂÓÒ ÌfiÓÔ ÙÔ 22% ·ÚÔ˘Û›·Û Ï‹ÚË ÂÈÙ˘¯›· (47). ∆· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ ·ÓÙ·ÔÎÚ›ıËÎ·Ó ÌÂÙ¿ ·fi ıÂÚ·›· 3-6 ÌËÓÒÓ ÎÈ ¤ÙÛÈ ÚÔÙ›ÓÂÙ·È Û·Ó ‰È¿ÚÎÂÈ· ıÂÚ·›·˜ ÔÈ 6 Ì‹Ó˜. ∆Ô Ê¿ÚÌ·ÎÔ ·Ó·Ê¤ÚÂÙ·È ·ÛʷϤ˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfi Î·È ÛÙ· ·È‰È¿ ËÏÈΛ·˜ 6-7 ÂÙÒÓ, ·ÚfiÏÔ Ô˘ ·Ï·ÈfiÙÂÚ˜ ÌÂϤÙ˜ ηٷϋÁÔ˘Ó fiÙÈ Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚÔ Û ·È‰È¿ ÌÂÁ·Ï‡ÙÂÚ· ÙˆÓ 10 ÂÙÒÓ. √È ÂÚÈÛÛfiÙÂÚ˜ ÚfiÛÊ·Ù· ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÁ·Û›Â˜ Û˘ÌʈÓÔ‡Ó fiÙÈ Ë ‰È¿ÚÎÂÈ· ıÂÚ·›·˜ Â›Ó·È 3-6 Ì‹Ó˜ (48). ∂Âȉ‹ Ë Èı·ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ Â›Ó·È ÌÂÁ¿ÏË (30-45%), ÚÔÙ›ÓÂÙ·È ÛÙ·‰È·Î‹ Ì›ˆÛ‹ ÙÔ˘ ·Ó¿ÏÔÁ· Ì ÙÔ ·ÔÙ¤ÏÂÛÌ· (27,48,49). √È ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ Â›Ó·È ÔÈ ·ÎfiÏÔ˘ı˜: ÎÔÈÓfi ÎÚ˘ÔÏfiÁËÌ·, ˘ÚÂÙfi˜, ÚÈÓ›ÙȘ, ÔÓÔΤʷÏÔ˜ Î·È Á·ÛÙÚÂÓÙÂÚ›ÙȘ. ™Â ÂÚÈÙÒÛÂȘ ¯ÚfiÓÈ·˜ ÚÈÓ›Ùȉ·˜, ÙÔ Ê¿ÚÌ·ÎÔ ¯ÔÚËÁÂ›Ù·È ·fi ÙÔ ÛÙfiÌ· (48). ¶·ÚfiÏÔ Ô˘ Ô Î›Ó‰˘ÓÔ˜ ηٷÎÚ¿ÙËÛ˘ ÓÂÚÔ‡ Î·È ˘ÔÓ·ÙÚÈ·ÈÌ›·˜ Â›Ó·È ˘·ÚÎÙfi˜, ÂÓÙÔ‡ÙÔȘ ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ÂÏ¿¯ÈÛÙ· ÂÚÈÛÙ·ÙÈο Û˘Ìو̷ÙÈ΋˜ ˘ÔÓ·ÙÚÈ·ÈÌ›·˜ ·Ó·Ê¤ÚÔÓÙ·È (50-52). ¶¿ÓÙˆ˜, Ú¤ÂÈ Ó· ÙÔÓ›˙ÂÙ·È Ì ¤ÌÊ·ÛË ÛÙÔ˘˜ ÁÔÓ›˜ Ë ÛËÌ·Û›· Ù˘ ÛÙ¤ÚËÛ˘ Ï‹„˘ ˘ÁÚÒÓ ÙÔ˘Ï¿¯ÈÛÙÔÓ 1 ÒÚ· ÚÈÓ ·fi ÙË Ï‹„Ë ÙÔ˘ Ê·ÚÌ¿ÎÔ˘, ÁÈ· ÙËÓ ·ÔÊ˘Á‹ ÙÔ˘ ÌÈÎÚÔ‡, ¤ÛÙˆ, ·˘ÙÔ‡ ÎÈÓ‰‡ÓÔ˘ (48). ∏ ‰ÂÛÌÔÚÂÛÛ›ÓË ıˆÚÂ›Ù·È Ê¿ÚÌ·ÎÔ ÚÒÙ˘ ÂÎÏÔÁ‹˜ ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ ·Ó Î·È ÙÔ ÔÛÔÛÙfi ÂÈÙ˘¯›·˜ Ù˘ ‰ÂÓ Â›Ó·È ÙÔ ·Ó·ÌÂÓfiÌÂÓÔ Î·È Â›Ó·È Û·ÊÒ˜ ÌÈÎÚfiÙÂÚÔ, Û˘ÁÎÚÈÓfiÌÂÓÔ Ì ·˘Ùfi Ù˘ Û˘Û΢‹˜ ·Ê‡ÓÈÛ˘. ∂ÈϤÔÓ, ÛÙ· ÌÂÈÔÓÂÎÙ‹Ì·Ù¿ Ù˘ ·Ó·Ê¤ÚÂÙ·È ÙÔ ˘„ËÏfi ÔÛÔÛÙfi ˘ÔÙÚÔ‹˜ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ ıÂÚ·›·˜. ∞ÓÙ›ıÂÙ·, ÏÂÔÓ¤ÎÙËÌ¿ Ù˘ ıˆÚÂ›Ù·È Ë ¿ÌÂÛË ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ¿ Ù˘ Î·È Û˘ÓÂÒ˜ ·ÔÙÂÏ› ÙË Ï‡ÛË ÁÈ· ÙÔ ÂÓÔ˘ÚËÙÈÎfi ·È‰› Ô˘ Ú¤ÂÈ Ó· ‰È·Ó˘ÎÙÂÚ‡ÛÂÈ ÂÎÙfi˜ ÛÈÙÈÔ‡ (.¯. ‰È·ÌÔÓ‹ Û ·È‰È΋ ηٷÛ΋ӈÛË) Î·È ¯ÚÂÈ¿˙ÂÙ·È ¤Ó· Ê¿ÚÌ·ÎÔ Ô˘ ‰Ú· ÁÚ‹ÁÔÚ· Î·È ·ÔÙÂÏÂÛÌ·ÙÈο.

Paediatriki 2001;64:14-24

™˘Ó‰˘·ÛÌfi˜ Û˘Û΢‹˜ ·Ê‡ÓÈÛ˘ Î·È ‰ÂÛÌÔÚÂÛÛ›Ó˘ ∏ Û˘Û΢‹ ·Ê‡ÓÈÛ˘ Î·È Ë ·ÓÙȉÈÔ˘ÚËÙÈ΋ ÔÚÌfiÓË fiÙ·Ó ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Ù·˘Ùfi¯ÚÔÓ·, Ê·›ÓÂÙ·È Ó· ¤¯Ô˘Ó Û˘ÓÂÚÁÈ΋ ‰Ú¿ÛË. √È Bradbury Î·È Meadow ·Ó·Ê¤ÚÔ˘Ó Û ÌÂϤÙË ÙÔ˘˜ ηχÙÂÚÔ ·ÔÙ¤ÏÂÛÌ· ÛÙËÓ ÔÌ¿‰· ÙˆÓ ·È‰ÈÒÓ Ì Ӣ¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË ÛÙ· ÔÔ›· ÂÊ·ÚÌfiÛıËÎÂ Û˘Ó‰˘·ÛÌfi˜ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ Î·È Û˘Û΢‹˜ ·Ê‡ÓÈÛ˘ Û ۯ¤ÛË Ì ÙËÓ ÔÌ¿‰· Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËΠÌfiÓÔ ÌÂ Û˘Û΢‹ ·Ê‡ÓÈÛ˘ (53). ŒÙÛÈ, ÚÔÙ›ÓÂÙ·È -Â› ·ÔÙ˘¯›·˜ Ì›·˜ ·fi ÙȘ 2 ÌÂıfi‰Ô˘˜- Ë ÚÔÛı‹ÎË Ù˘ ‰Â‡ÙÂÚ˘ Û·Ó Û˘ÌÏËڈ̷ÙÈÎfi ̤ÙÚÔ ıÂÚ·›·˜ (42,48). πÌÈÚ·Ì›ÓË ∏ ÈÌÈÚ·Ì›ÓË, ¤Ó· ÙÚÈ΢ÎÏÈÎfi ·ÓÙÈηٷıÏÈÙÈÎfi, ¯ÚËÛÈÌÔÔÈ‹ıËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÙÔ 1960 ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘. √ ·ÎÚÈ‚‹˜ Ì˯·ÓÈÛÌfi˜ ‰Ú¿Û˘ Ù˘ ‰ÂÓ Â›Ó·È ÁÓˆÛÙfi˜, ·Ó Î·È ¤¯Ô˘Ó ÚÔÙ·ı› ‰È¿ÊÔÚ˜ ıˆڛ˜ Û¯ÂÙÈ˙fiÌÂÓ˜ Ì ÙËÓ ·ÓÙÈηٷıÏÈÙÈ΋ Î·È ·ÓÙȯÔÏËÓÂÚÁÈ΋ Ù˘ ‰Ú¿ÛË Î·ıÒ˜ Î·È ÙȘ ÌÂÙ·‚ÔϤ˜ Ô˘ ÚÔηÏ› ÛÙÔ Ì˯·ÓÈÛÌfi ‡ÓÔ˘ – ·Ê‡ÓÈÛ˘. ÃÔÚËÁÂ›Ù·È Û ‰fiÛË 25mg ÁÈ· ·È‰È¿ 6-8 ÂÙÒÓ Î·È 50-75mg ÁÈ· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿, 1-2 ÒÚ˜ ÚÈÓ ÙËÓ Î·Ù¿ÎÏÈÛË (26). ªÂ ‚¿ÛË ÙÔ ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜, Ë Û˘Ó‹ı˘ ÚÔÙÂÈÓfiÌÂÓË ‰fiÛË Â›Ó·È 0,9-1,5 mg/kg/Ë̤ڷ. ∏ ÎÏÈÓÈ΋ ·ÓÙ·fiÎÚÈÛË ¤¯ÂÈ Û˘Û¯ÂÙÈÛı› Ì ٷ Â›‰· Ù˘ ÛÙÔ Ï¿ÛÌ· (54), ··ÈÙÂ›Ù·È fï˜ ȉȷ›ÙÂÚË ÚÔÛÔ¯‹ ÛÙË ¯ÔÚ‹ÁËÛË, ÁÈ·Ù› ÌÂÚÈΤ˜ ÊÔÚ¤˜ Ù· ıÂÚ·¢ÙÈο Â›‰· Â›Ó·È ÎÔÓÙ¿ ÛÙ· ÙÔÍÈο. ¶·ÚfiÏÔ Ô˘ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ Â›Ó·È ¿ÌÂÛ· ÔÚ·Ù‹, ÂÓÙÔ‡ÙÔȘ ı· ‹Ù·Ó ÚÔÙÈÌfiÙÂÚÔ Ó· Û˘Ó¯ÈÛı› Ë ıÂÚ·›· ÁÈ· 1-2 ‚‰ÔÌ¿‰Â˜ ÚÔÙÔ‡ ÂÎÙÈÌËı› ÙÔ ·ÔÙ¤ÏÂÛÌ· Î·È ÙÚÔÔÔÈËı› Ë ‰fiÛË. ∏ ·Ú¯È΋ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂÚ·›· Î˘Ì·›ÓÂÙ·È ·fi 10-40%, ÂÓÒ Ê·›ÓÂÙ·È ÙÔ Ê¿ÚÌ·ÎÔ Ó· Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚÔ Û ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ (26). ¶Ú¤ÂÈ, ¿ÓÙˆ˜, Ó· ÙÔÓÈÛı› Ë ·Ô˘Û›· ·fi ÙË ‚È‚ÏÈÔÁÚ·Ê›· Û‡Á¯ÚÔÓ˘ ‰ÈÏ‹˜-Ù˘ÊÏ‹˜ ÌÂϤÙ˘ Ô˘ Ó· ·ÍÈÔÏÔÁ› ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Î·È ÙȘ ·ÚÂÓ¤ÚÁÂȘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘. ∏ ÚÔÙÂÈÓfiÌÂÓË ‰È¿ÚÎÂÈ· ıÂÚ·›·˜ Â›Ó·È 3-6 Ì‹Ó˜, ÂÓÒ ˘ÔÙÚÔ‹ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ ıÂÚ·›·˜ ÌÔÚ› Ó· ·Ú·ÙËÚËı› ÛÙÔ 40-60% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (55,56). ÀÔÛÙËÚ›˙ÂÙ·È fiÙÈ Ë ÛÙ·‰È·Î‹ Ì›ˆÛË Î·È ‰È·ÎÔ‹ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ÌÔÚ› Ó· ÌÂÈÒÛÂÈ ÙËÓ Èı·ÓfiÙËÙ· ˘ÔÙÚÔ‹˜. ∂› ÂÌÊ¿ÓÈÛ˘ ˘ÔÙÚÔ‹˜, Ë ıÂÚ·›· ÌÔÚ› Ó· Â·Ó·ÏËÊı›. √È ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ‰ÂÓ Â›Ó·È Û˘¯Ó¤˜ Î·È ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ·ÓËÛ˘¯›·, ·¸Ó›·, ÍËÚÔÛÙÔÌ›·, Ó·˘Ù›·, ÎÂÊ·Ï·ÏÁ›·, ·ÒÏÂÈ· ‚¿ÚÔ˘˜ Î·È ‰È·Ù·Ú·¯¤˜ Ù˘

21


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 22

¶·È‰È·ÙÚÈ΋ 2001;64:14-24

ÚÔÛˆÈÎfiÙËÙ·˜. ∏ ˘ÂÚ‰ÔÛÔÏÔÁ›· ÌÔÚ› Ó· ·Ԃ› ı·Ó·ÙËÊfiÚ·, ÚÔηÏÒÓÙ·˜ ηډȷ΋ ·ÚÚ˘ıÌ›·, ˘fiÙ·ÛË, Û·ÛÌÔ‡˜ Î·È ÎÒÌ·. √ ÚfiÏÔ˜ Ù˘ ÈÌÈÚ·Ì›Ó˘ ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ ¤¯ÂÈ ÂÚÈÔÚÈÛı› ÏfiÁÔ ÙˆÓ ÛÔ‚·ÚÒÓ ·Ó Î·È Û¿ÓȈÓ- ·ÓÂÈı‡ÌËÙˆÓ ÂÓÂÚÁÂÈÒÓ Ù˘ (42). ªÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› ηٿ ÚÔÙ›ÌËÛË ·fi ·È‰Ô„˘¯›·ÙÚÔ Û ·È‰È¿ ÌÂÁ·Ï‡ÙÂÚ· ÙˆÓ 7 ÂÙÒÓ Ì ÂÓÔ‡ÚËÛË Î·È ‰È·Ù·Ú·¯¤˜ ÛÙËÓ ÚÔÛÔ¯‹, ·ÓÙ›ÏË„Ë ‹ ÎÈÓËÙÈÎfiÙËÙ· (4,48). ™Â οıÂ, fï˜, ÂÚ›ÙˆÛË, ·ÔÙÂÏ› Ê¿ÚÌ·ÎÔ ‰Â‡ÙÂÚ˘ ‹ ÙÚ›Ù˘ ÂÎÏÔÁ‹˜ ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ (1). ÕÏϘ ıÂÚ·›˜ ¢È¿ÊÔÚ˜ ıÂÚ·›˜ Ê·Ú̷΢ÙÈΤ˜ (Ô͢‚Ô˘ÙÈÓ›ÓË, ηڂ·Ì·˙Â›ÓË) Î·È ÌË („˘¯ÔıÂÚ·›·, ‰È·ÈÙËÙÈÎÔ› ÂÚÈÔÚÈÛÌÔ›, ˘ÓÔıÂÚ·›·), ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÂÓÔ‡ÚËÛ˘, Ì ‰È·ÊÔÚÂÙÈο ·ÔÙÂϤÛÌ·Ù·. ∏ Ô͢‚Ô˘ÙÈÓ›ÓË, ηıÒ˜ Î·È ¿ÏÏ· ·ÓÙȯÔÏËÓÂÚÁÈο Ê¿Ú̷η, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û˘¯Ó¿ ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘, ·ÚfiÏÔ Ô˘ ‰ÂÓ ·ÔÙÂÏÔ‡Ó Ê¿Ú̷η ÂÎÏÔÁ‹˜. ∞Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ Ë Ô͢‚Ô˘ÙÈÓ›ÓË Â›Ó·È ÙÔ Û˘¯ÓfiÙÂÚ· Û˘ÓÙ·ÁÔÁÚ·ÊË̤ÓÔ Ê¿ÚÌ·ÎÔ ÁÈ· ÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ ÛÙË °·ÏÏ›· (33). ∏ Ô͢‚Ô˘ÙÈÓ›ÓË ÌÂÈÒÓÂÈ ÙË Û˘¯ÓfiÙËÙ· ÙˆÓ Û˘Û¿ÛÂˆÓ ÙÔ˘ Â͈ÛÙ‹Ú· ÎÈ ÂÔ̤ӈ˜, ·˘Í¿ÓÂÈ ÙË ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜. ∏ Û˘Ó‹ı˘ ‰fiÛË ÁÈ· ·È‰È¿ ÌÂÁ·Ï‡ÙÂÚ· ÙˆÓ 7 ÂÙÒÓ Â›Ó·È 5mg 2 ‹ 3 ÊÔÚ¤˜ ÙËÓ Ë̤ڷ (1). √È Û˘Ó‹ıÂȘ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ Â›Ó·È ÍËÚÔÛÙÔÌ›·, ÂÚ˘ıÚfiÙËÙ· ÙÔ˘ ÚÔÛÒÔ˘ Î·È ı¿Ì‚Ô˜ Ù˘ fiÚ·Û˘ Û ÌÂÁ·Ï‡ÙÂÚ˜ ‰fiÛÂȘ. ∏ Ô͢‚Ô˘ÙÈÓ›ÓË ‰ÂÓ Ê·›ÓÂÙ·È Ó· Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙË ıÂÚ·›· Ù˘ ÚˆÙÔ·ıÔ‡˜ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Î·È Û˘ÓÂÒ˜ Ô ÚfiÏÔ˜ Ù˘ Â›Ó·È ÂÚÈÔÚÈṲ̂ÓÔ˜ (57). ∞ÓÙ›ıÂÙ·, ‰È·‰Ú·Ì·Ù›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙË ıÂÚ·›· Ù˘ ÂÓÔ‡ÚËÛ˘, fiÙ·Ó ·˘Ù‹ Û˘Ó‰˘¿˙ÂÙ·È Ì ·ÛÙ·ı‹ ‹ Ó¢ÚÔÁÂÓ‹ ·ÛÙË. ∏ ηڂ·Ì·˙Â›ÓË ·˘Í¿ÓÂÈ ÙË ¯ˆÚËÙÈÎfiÙËÙ· Ù˘ ·ÛÙˆ˜ Î·È ÙËÓ Â˘·ÈÛıËÛ›· ÙˆÓ ÓÂÊÚÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ ÛÙËÓ ·ÓÙȉÈÔ˘ÚËÙÈ΋ ÔÚÌfiÓË, ÌÂÈÒÓÔÓÙ·˜ ÙËÓ ·Ú·ÁˆÁ‹ ÙˆÓ Ô‡ÚˆÓ. Œ¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ Ì ÂÓı·ÚÚ˘ÓÙÈο ·ÔÙÂϤÛÌ·Ù· (58), ÏËÓ fï˜, Ë ÂÌÂÈÚ›· ·fi ÙË ¯Ú‹ÛË Ù˘ Â›Ó·È Ôχ ÂÚÈÔÚÈṲ̂ÓË. ∏ „˘¯ÔıÂÚ·›· ‰ÂÓ Ê·›ÓÂÙ·È Ó· Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙË ıÂÚ·›· Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ (26). √È ‰È·ÈÙËÙÈÎÔ› ÂÚÈÔÚÈÛÌÔ› (Á·Ï·ÎÙÔÎÔÌÈο ÚÔ˚fiÓÙ·, ÛÔÎÔÏ¿Ù·, ·Ó·„˘ÎÙÈο Ù‡Ô˘ ÎfiÏ·˜ Î·È ¯˘ÌÔ›), ·Ó·Ê¤ÚÂÙ·È Û ÌÂϤÙË, fiÙÈ ÌÂÈÒ-

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Paediatriki 2001;64:14-24

ÓÔ˘Ó ÙÔÓ ·ÚÈıÌfi ÙˆÓ ÂÓÔ˘ÚËÙÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ (26,59), ·ÚfiÏÔ Ô˘ ·˘Ùfi ¤¯ÂÈ ·ÌÊÈÛ‚ËÙËı›. ∫·Ï¿ ·ÔÙÂϤÛÌ·Ù· ·Ó·Ê¤ÚÔÓÙ·È Î·È ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÂÓÔ‡ÚËÛ˘ Ì ˘ÓÔıÂÚ·›·, ·ÚfiÏÔ Ô˘ ‰ÂÓ Â›Ó·È ·Ô‰ÂÎÙ‹ Û·Ó Ì¤ıÔ‰Ô˜ ıÂÚ·›·˜ Ù˘ Ó˘ÎÙÂÚÈÓ‹˜ ÂÓÔ‡ÚËÛ˘ (26,60). ™˘ÌÂÚ¿ÛÌ·Ù· ∏ Ó˘¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË Â›Ó·È Û˘¯Ófi Úfi‚ÏËÌ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ Ô˘ ‰˘Ó·ÙfiÓ Â›Ó·È Ó· ÚÔηϤÛÂÈ ÛÔ‚·Ú¤˜ ÂÈÙÒÛÂȘ ÛÙËÓ ·˘ÙÔÂÎÙ›ÌËÛË ÙÔ˘ ·È‰ÈÔ‡. ∏ ·ÈÙÈÔÏÔÁ›· ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ηٷÓÔËÙ‹. ∂ÓÔ¯ÔÔÈÔ‡ÓÙ·È Î˘Ú›ˆ˜, ‰È·Ù·Ú·¯¤˜ ÙÔ˘ Ì˯·ÓÈÛÌÔ‡ ·Ê‡ÓÈÛ˘ fiÙ·Ó Ë Î‡ÛÙË Â›Ó·È Ï‹Ú˘, ηıÒ˜ Î·È ÌÂȈ̤ÓË ¤ÎÎÚÈÛË Ù˘ ·ÓÙȉÈÔ˘ÚËÙÈ΋˜ ÔÚÌfiÓ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜. √È ‰È·Ù·Ú·¯¤˜ ·˘Ù¤˜ Ê·›ÓÂÙ·È Ó· Â›Ó·È ÁÂÓÂÙÈο ηıÔÚÈṲ̂Ó˜. √È ÔÏϤ˜ ÚÔÙÂÈÓfiÌÂÓ˜ ıÂÚ·¢ÙÈΤ˜ χÛÂȘ Ê·ÓÂÚÒÓÔ˘Ó fiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ È‰·ÓÈ΋ ıÂÚ·›· ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜. √ ‰È·ÊÔÚÂÙÈÎfi˜ ÙÚfiÔ˜ ÚÔÛ¤ÁÁÈÛ˘ Î·È ·ÓÙÈÌÂÙÒÈÛ˘ ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ Ô˘ ·Ú·ÙËÚÂ›Ù·È Û ÔÏϤ˜ ¯ÒÚ˜, ‰Â›¯ÓÂÈ ÙËÓ ·Ô˘Û›· ÎÔÈÓ¿ ·Ô‰ÂÎÙÔ‡ ‰ÈÂıÓÔ‡˜ ÚˆÙÔÎfiÏÏÔ˘. √È ÂÈÎÚ·ÙÔ‡Û˜ ¿ÓÙˆ˜ ıÂÚ·›˜ Â›Ó·È Ë Û˘Û΢‹ ·Ê‡ÓÈÛ˘ Î·È Ë ‰ÂÛÌÔÚÂÛÛ›ÓË, ÌfiÓ˜ ‹ ÛÂ Û˘Ó‰˘·ÛÌfi. ∏ Û˘ÓÂÚÁ·Û›· ÙÔ˘ ·È‰ÈÔ‡ Î·È ÙˆÓ ÁÔÓ¤ˆÓ Ì ÙÔÓ È·ÙÚfi ÎÚ›ÓÂÙ·È ··Ú·›ÙËÙË ÁÈ· ÙËÓ ÂÈÙ˘¯‹ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÂÓÔ‡ÚËÛ˘. °È· ÙÔ ÏfiÁÔ ·˘Ùfi, Ë ÂÈÏÔÁ‹ ÙÔ˘ ›‰Ô˘˜ Ù˘ ıÂÚ·›·˜ Á›ÓÂÙ·È Ï·Ì‚¿ÓÔÓÙ·˜ ˘’ fi„ÈÓ Î·È ÙËÓ ÚÔÙ›ÌËÛ‹ ÙÔ˘˜, ·ÊÔ‡ ÙÔ˘˜ ·Ú·ÙÂıÔ‡Ó ·Ó·Ï˘ÙÈο Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· Î·È Ù· ÌÂÈÔÓÂÎÙ‹Ì·Ù· οı ıÂÚ·›·˜. ∏ ÛÙ¿ÛË Ù˘ ÏÂÈÔ„ËÊ›·˜ ÙÔ˘ È·ÙÚÈÎÔ‡ ÎfiÛÌÔ˘ Ó· ·Ú·ÎÔÏÔ˘ı› ÙÔ Úfi‚ÏËÌ· ·Ó·Ì¤ÓÔÓÙ·˜ ·˘ÙfiÌ·ÙË ˘Ô¯ÒÚËÛË ‰ÂÓ Â›Ó·È ‰ÈηÈÔÏÔÁË̤ÓË. ∞ÓÙ›ıÂÙ·, ÂÈ‚¿ÏÏÂÙ·È ‰Ú·ÛÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÁÈ· ÙËÓ ··ÏÏ·Á‹ ÙÔ˘ ·È‰ÈÔ‡ Î·È Ù˘ ÔÈÎÔÁ¤ÓÂÈ¿˜ ÙÔ˘ ·fi ÙÔ ‚·Ú‡ ·˘Ùfi ÊÔÚÙ›Ô. µÈ‚ÏÈÔÁÚ·Ê›· 1. Mark S, Frank J. Nocturnal enuresis. Br J Urol 1995;75:427-434. 2. Alon S. Nocturnal enuresis. Pediatr Nephrol 1995;9:94-103. 3. Norgaard JP, Petersen EB, Djurhuus JC. Diurnal anti-diuretic hormone levels in enuretics. J Urol 1985;134:1029-1031. 4. Hjalmas K. Nocturnal enuresis: Basic facts and new horizons. Eur Urol 1998;33(3 Suppl):53-57. 5. Butler RJ. Establishment of working definitions in nocturnal enuresis. Arch Dis Child 1991;66:267-271. 6. Goellner H, Ziegler E, Foman J. Urination during the first three years of life. Nephron 1981;28:174-178. 7. Koff A. Estimating bladder capacity in children. Urology 1988;21:248-251.


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55. Martin IG. Imipramine pamoate in the treatment of childhood enuresis. Am J Dis Child 1971;122:42-47. 56. Shaffer D, Costello AJ, Hill ID. Control of enuresis with imipramine. Arch Dis Child 1968;43:665-671. 57. Lovering JS, Tallet SE, Mc Kendry JB. Oxybutynin efficacy in the treatment of primary enuresis. Pediatrics 1988;82:104-106. 58. Al-Waili NS. Carbamazepine to treat primary nocturnal enuresis: Double-blind study. Eur J Med Res 2000;5:40-44. 59. Esperanca M, Gerrard JW. Nocturnal enuresis: Comparison of the effect of imipramine and dietary restriction of bladder capacity. Can Med Assoc J 1969;101:721-724. 60. Olness K. The use of self-hypnosis in the treatment of childhood nocturnal enuresis. Clin Pediatr 1975;14:273-275.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 13-09-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 27-11-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∞‚Ú·¿Ì ∏Ï›·˜ ∫ËÊÈÛ›·˜ 23, ∞Á. ¢Ô̤ÙÈÔ˜ 2369, §Â˘ÎˆÛ›·, ∫‡ÚÔ˜

¶ÂÚ›ÏË„Ë ¿ÚıÚÔ˘ ·fi ÙË µÈ‚ÏÈÔÁÚ·Ê›· µÚÔÁ¯È΋ ÚÔÛÙ·Û›· Ì ¤Ó· ·ÓÙ·ÁˆÓÈÛÙ‹ Ï¢ÎÔÙÚÈÂÓÈÎÒÓ ˘Ô‰Ô¯¤ˆÓ Û ·ÛıÌ·ÙÈο ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜1 ™ÎÔfi˜: ÀÔı¤Û·Ì fiÙÈ ¤Ó·˜ ·ÓÙ·ÁˆÓÈÛÙ‹˜ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ ÙˆÓ Ï¢ÎÔÙÚÈÂÓ›ˆÓ (LTRA) ı· ÌÔÚÔ‡Û ӷ ÚÔÛٷهÛÂÈ ÙÔ˘˜ ·ÂÚ·ÁˆÁÔ‡˜ ·fi ÙËÓ ·ÓÙ›‰Ú·ÛË Û ÂÈÛÓÔ‹ ÍËÚÔ‡„˘¯ÚÔ‡ ·¤Ú· Û ·ÛıÌ·ÙÈο ·È‰È¿ ÚÔÛ¯ÔÏÈ΋˜ ËÏÈΛ·˜. ª¤ıÔ‰Ô˜: ™Â Ì›· Ù˘¯·ÈÔÔÈË̤ÓË, ‰ÈÏ‹ Ù˘ÊÏ‹ ÂÏÂÁ¯fiÌÂÓË Ì placebo, ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ÌÂϤÙË, ÂÍÂÙ¿Û·Ì ٷ ·ÔÙÂϤÛÌ·Ù· ÙÔ˘ ÂȉÈÎÔ‡ ·Ó·ÛÙÔϤ· ÙˆÓ ˘Ô‰Ô¯¤ˆÓ ÙˆÓ Ï¢ÎÔÙÚÈÂÓ›ˆÓ montelukast Û ‰fiÛË 5mg ËÌÂÚËÛ›ˆ˜ ÁÈ· ‰‡Ô Ë̤Ú˜, ÛÙÔÓ ‚ÚÔÁ¯fiÛ·ÛÌÔ Ô˘ ÚÔηÏÂ›Ù·È ·fi ÙÔÓ ˘ÂÚ·ÂÚÈÛÌfi Ì ÍËÚfi-„˘¯Úfi ·¤Ú· Û 13 ·ÛıÌ·ÙÈο ·È‰È¿ ËÏÈΛ·˜ 3 ¤ˆ˜ 5 ¯ÚÔÓÒÓ. √ ‚ÚÔÁ¯fiÛ·ÛÌÔ˜ ÌÂÙÚ‹ıËΠ̠ÙË ÌÔÚÊ‹ Ù˘ ÂȉÈ΋˜ ·ÓÙ›ÛÙ·Û˘ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ (sRaw) Û ÏËı˘ÛÌÔÁÚ¿ÊÔ ÔÏÈÎÔ‡ ÛÒÌ·ÙÔ˜ (whole-body plethesmograph) ÚÈÓ, Î·È 4 ÏÂÙ¿ ÌÂÙ¿ ÙË ‰ÔÎÈÌ·Û›· ÚfiÎÏËÛ˘ Ì ÍËÚfi „˘¯Úfi-·¤Ú·. ∏ ‰˘Ó·ÙfiÙËÙ· Â·Ó¿Ï˄˘ Ù˘ ‚ÚÔÁ¯È΋˜ ÚÔÛÙ·Û›·˜ ÂϤÁ¯ıËΠ̠ÙËÓ Â·Ó¿ÏË„Ë Ù˘ ÌÂϤÙ˘ Ì placebo Û 6 ·fi Ù· 13 ·È‰È¿. ∞ÔÙÂϤÛÌ·Ù·: ∏ ÂȉÈ΋ ·ÓÙ›ÛÙ·ÛË ÙˆÓ ·ÂÚ·ÁˆÁÒÓ sRaw ·˘Í‹ıËΠηٿ ̤ÛÔ fiÚÔ 46% (95% confidence interval, (CI): 30 ¤ˆ˜ 63%) ÌÂÙ¿ ·fi ıÂÚ·›· Ì placebo, Î·È 17% (95% CI: 3 ¤ˆ˜ 31%) ÌÂÙ¿ ıÂÚ·›· Ì montelukast. √ÎÙÒ ·fi Ù· ·È‰È¿ ÂÏ¿Ì‚·Ó·Ó ıÂÚ·›· Û˘ÓÙ‹ÚËÛ˘ Ì ‚Ô˘‰ÂÛÔÓ›‰Ë ¯ÔÚËÁÔ‡ÌÂÓË Ì ·ÂÚÔı¿Ï·ÌÔ Û ̛· ̤ÛË ËÌÂÚ‹ÛÈ· ‰fiÛË 350 mcg, ·ÏÏ¿ Ë ‚ÚÔÁ¯È΋ ÚÔÛÙ·Û›· ·fi ÙÔ montelukast ‹Ù·Ó ·ÓÂÍ¿ÚÙËÙË ·fi ÙËÓ Ù·˘Ùfi¯ÚÔÓË ıÂÚ·›· Ì ÛÙÂÚÔÂȉ‹. ¢ÂÓ ˘‹ÚÍ ·ÍÈfiÈÛÙË ¤Ó‰ÂÈÍË ·ÔÙ˘¯›·˜ ÛÙËÓ ·ÓÙ·fiÎÚÈÛË, Î·È Ô ÚÔÛٷ٢ÙÈÎfi˜ ÚfiÏÔ˜ ÙÔ˘ montelukast ‹Ù·Ó ÛÙ·ıÂÚfi˜ Ì ÙËÓ Â·Ó¿ÏË„Ë Ù˘ ‰ÔÎÈÌ·Û›·˜ (p=0,02).

24

™˘ÌÂÚ¿ÛÌ·Ù·: √ ·ÓÙ·ÁˆÓÈÛÙ‹˜ Ï¢ÎÔÙÚÈÂÓ›ˆÓ montelukast ·Ú¤Û¯Â ÎÏÈÓÈο ÛËÌ·ÓÙÈ΋ ‚ÚÔÁ¯È΋ ÚÔÛÙ·Û›· ¤Ó·ÓÙÈ ÙˆÓ ÂÈÙÒÛÂˆÓ ÙÔ˘ ˘ÂÚ·ÂÚÈÛÌÔ‡ Ì ÍËÚfi-„˘¯Úfi ·¤Ú· Û ·ÛıÌ·ÙÈο ·È‰È¿ ËÏÈΛ·˜ 3 ¤ˆ˜ 5 ÂÙÒÓ. ∏ ‚ÚÔÁ¯È΋ ÚÔÛÙ·Û›· Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ÔÌÔÈÔÁÂÓ‹˜ ÌÂٷ͇ ÙˆÓ ·È‰ÈÒÓ Î·È ·ÓÂÍ¿ÚÙËÙË ·fi ÙËÓ Ù·˘Ùfi¯ÚÔÓË ıÂÚ·›· Ì ÛÙÂÚÔÂȉ‹. ∞˘Ùfi ˘Ô‰ËÏÒÓÂÈ fiÙÈ ÔÈ ·Ó·Ù·ÁˆÓÈÛÙ¤˜ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ ÙˆÓ Ï¢ÎÔÙÚÈÂÓ›ˆÓ ÌÔÚ› Ó· ¤¯Ô˘Ó ıÂÚ·¢ÙÈ΋ ÂÊ·ÚÌÔÁ‹ ÛÙÔÓ ÂÚÈÔÚÈÛÌfi ÙˆÓ ÎÏÈÓÈÎÒÓ Û˘ÌÙˆÌ¿ÙˆÓ ÙÔ˘ ¿ÛıÌ·ÙÔ˜ Û ÌÈÎÚ¿ ·È‰È¿.

1H.

Bisgaard, K. Nielsen Bronchoprotection with a leukotriene receptor antagonist in asthmatic preschool children Am J Respir Crit Care Med 2000;162:187-190

£ÂÔÊ¿Ó˘ ∆ÛÈÏÈÁÈ¿ÓÓ˘ ¢È¢ı˘ÓÙ‹˜ ¶·È‰È·ÙÚÈÎÔ‡ ∆Ì‹Ì·ÙÔ˜ ¡·˘ÙÈÎÔ‡ ¡ÔÛÔÎÔÌ›Ԣ ∞ıËÓÒÓ


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 25

¶·È‰È·ÙÚÈ΋ 2001;64:25-48

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™À¡¢ƒ√ª∞ ªÀ∂§π∫∏™ ∞¡∂¶∞ƒ∫∂π∞™

Paediatriki 2001;64:25-48

CONTINUING MEDICAL EDUCATION

THE BONE MARROW FAILURE SYNDROMES

∆Ô ı¤Ì· Ù˘ ™˘Ó¯È˙fiÌÂÓ˘ ¶·È‰È·ÙÚÈ΋˜ ∂Î·›‰Â˘Û˘ Â›Ó·È «™‡Ó‰ÚÔÌ· ª˘ÂÏÈ΋˜ ∞ÓÂ¿ÚÎÂÈ·˜». ¶·ÚÔ˘ÛÈ¿ÛÙËΠˆ˜ ‰‡Ô ™ÙÚÔÁÁ˘Ï¤˜ ∆Ú¿Â˙˜ ÛÙÔ 12Ô ∂Ù‹ÛÈÔ ™˘Ó¤‰ÚÈÔ Ù˘ ∂ÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ ∞ÈÌ·ÙÔÏÔÁÈ΋˜ ∂Ù·ÈÚ›·˜, ∞ı‹Ó·, 20.11.1999

∂È̤ÏÂÈ· °Ú·ÙÔ‡ ™˘ÌÔÛ›Ô˘:

Editing of Symposium Manuscripts:

º. ∞ı·Ó·ÛÈ¿‰Ô˘-¶ÈÂÚÔÔ‡ÏÔ˘ ∞Ó·Ï. ∫·ıËÁ‹ÙÚÈ· ¶·È‰È·ÙÚÈ΋˜ ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢

F. Athanasiadou-Piperopoulou Assoc. Professor of Pediatrics Aristotelion University of Thessaloniki

™˘ÓÙÔÓÈÛÙ‹˜ ∞’ ™ÙÚÔÁÁ˘Ï‹˜ ∆Ú¿Â˙·˜:

Coordinator of Round Table A’:

Ã. ∫·ÙÙ¿Ì˘ √ÌfiÙÈÌÔ˜ ∫·ıËÁËÙ‹˜ ¶·È‰È·ÙÚÈ΋˜ ¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ

Ch. Kattamis Professor Emeritus of Pediatrics University of Athens

ªË¯·ÓÈÛÌÔ› –·ÈÙÈÔÏÔÁ›· ·Ï·ÛÙÈ΋˜ ·Ó·ÈÌ›·˜

∂tiology and pathogenetic mechanisms of acquired and inherited aplastic anemia in childhood

™. ¶ÔÏ˘¯ÚÔÓÔÔ‡ÏÔ˘-∞Ó‰ÚÔ˘Ï·Î¿ÎË

S. Polychronopoulou-Androulakaki

∞Ï·ÛÙÈο Û‡Ó‰ÚÔÌ· Î·È Ì˘ÂÏÔ‰˘ÛÏ·Û›·

Aplastic syndromes and myelodysplasia

∞. ¶Ô˘ÚÙÛ›‰Ë˜

∞. Pourtsidis

∞Ï·ÛÙÈο Û‡Ó‰ÚÔÌ· Î·È ·˘ÍËÙÈÎÔ› ·ÈÌÔÔÈËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜

Aplastic syndromes and hematopoietic growth factors

ª. ªÔÛ¯fi‚Ë

M. Moschovi

™˘ÁÁÂÓ›˜ √˘‰ÂÙÂÚÔÂӛ˜

Congenital neutropenias

Õ. ∫·Ú·ÎfiÏË

∞. Karakoli

™˘ÓÙÔÓ›ÛÙÚÈ· µ’ ™ÙÚÔÁÁ˘Ï‹˜ ∆Ú¿Â˙·˜:

Coordinator of Round Table µ’:

º. ∞ı·Ó·ÛÈ¿‰Ô˘-¶ÈÂÚÔÔ‡ÏÔ˘ ∞Ó·Ï. ∫·ıËÁ‹ÙÚÈ· ¶·È‰È·ÙÚÈ΋˜ ∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ £ÂÛÛ·ÏÔӛ΢

F. Athanasiadou-Piperopoulou Assoc. Professor of Pediatrics Aristotelion University of Thessaloniki

£ÚÔÌ‚ÔÂӛ˜ ÔÊÂÈÏfiÌÂÓ˜ Û ·ÓÂ·Ú΋ ·Ú·ÁˆÁ‹ ·ÈÌÔÂÙ·Ï›ˆÓ

Thrombocytopenias resulting from deficient platelet production

∂. ¶Ï·ÙÔÎÔ‡ÎË-∫ÔÌÈÙÔÔ‡ÏÔ˘

H. Platokouki-Komitopoulou

∞ÌÈÁ‹˜ ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜

Pure red cell aplasia of childhood

µ. ∫›ÙÚ·

V. Kitra

∞ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ıÂÚ·›· Û ·È‰È¿ Ì ‚·ÚÈ¿ ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·

Immunosuppressive therapy in children with severe aplastic anemia

π.¶. ¶·Ó·ÁÈÒÙÔ˘

J.P. Panagiotou

ªÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (ªª√) Û ·È‰È¿ Ì ۇӉÚÔÌ· Ì˘ÂÏÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜

Bone marrow transplantation in children with bone marrow failure syndromes

¢. ¶ÂÙÚfiÔ˘ÏÔ˜

D. Petropoulos

25


2001 paid(teliko) 17-10-01 15:45 ™ÂÏ›‰· 26

¶·È‰È·ÙÚÈ΋ 2001;64:26-29

™À¡∂Ãπ∑√ª∂¡∏ ¶∞π¢π∞∆ƒπ∫∏ ∂∫¶∞π¢∂À™∏

Paediatriki 2001;64:26-29

CONTINUING MEDICAL EDUCATION

™˘ÁÁÂÓ›˜ Ô˘‰ÂÙÂÚÔÂӛ˜ Õ. ∫·Ú·ÎfiÏË

Congenital neutropenias ∞. Karakoli

¶ÂÚ›ÏË„Ë: ∏ Ì›ˆÛË ÙÔ˘ ·fiÏ˘ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÎÏÔÊÔÚÔ‡ÓÙˆÓ ÛÙÔ ·›Ì· Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ Î¿Ùˆ ·fi 1500/mm3 ÔÚ›˙ÂÙ·È ˆ˜ Ô˘‰ÂÙÂÚÔÂÓ›·. ∏ Ô˘‰ÂÙÂÚÔÂÓ›· ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È ÛÂ Û˘ÁÁÂÓ‹ ‹ Â›ÎÙËÙ· ·›ÙÈ·. ∆· ΢ÚÈfiÙÂÚ· Û˘ÁÁÂÓ‹ ÓÔÛ‹Ì·Ù· Ì ‚·ÚÈ¿ Ô˘‰ÂÙÂÚÔÂÓ›· Â›Ó·È Ë ·ÁÂÓÂÛ›· ÙÔ˘ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·ÎÔ‡ ÈÛÙÔ‡, ÙÔ Û‡Ó‰ÚÔÌÔ Kostmann, Ë ª˘ÂÏÔοıÂÍË, ÙÔ Û‡Ó‰ÚÔÌÔ Shwachman ÎÏ., Ù· ÔÔ›· ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ÙËÓ ÚÒÈÌË ÂÌÊ¿ÓÈÛË ·ÂÈÏËÙÈÎÒÓ ÁÈ· ÙË ˙ˆ‹ ÏÔÈÌÒ͈Ó. ∏ ΢ÎÏÈ΋ Ô˘‰ÂÙÂÚÔÂÓ›· Î·È Ë ÔÈÎÔÁÂÓ‹˜ ηÏÔ‹ı˘ Ô˘‰ÂÙÂÚÔÂÓ›· ¤¯Ô˘Ó ηχÙÂÚË ÚfiÁÓˆÛË Î·È ¯Ú‹˙Ô˘Ó ÌfiÓÔÓ ÂÏ·¯›ÛÙ˘ ‹ η̛·˜ È·ÙÚÈ΋˜ ·Ú¤Ì‚·Û˘. √È Û˘ÁÁÂÓ›˜ Ô˘‰ÂÙÂÚÔÂӛ˜ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Û‹ÌÂÚ· Ì ÙË ¯ÔÚ‹ÁËÛË ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ÙˆÓ ÎÔÎÎÈÔ΢ÙÙ¿ÚˆÓ (G-CSF) Î·È ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ.

Abstract: Neutropenia is defined as an absolute decrease in the number of circulating neutrophils in the blood to less than 1500/mm3. Congenital or extrinsic factors may cause neutropenia. The main congenital disorders associated with severe neutropenia are reticular dysgenesis, Kostmann’s syndrome, Myelokathexis and Shwachman’s syndrome and which are characterized by an early onset of life-threatening pyogenic infections. Patients with cyclic neutropenia and familial benign neutropenia have a good prognosis requiring only minimal or no medical intervention. The use of granulocyte colony-stimulating factor (G-CSF) and bone marrow transplantation have dramatically improved the outcome for many patients with severe congenital neutropenia.

§¤ÍÂȘ ÎÏÂȉȿ: Û˘ÁÁÂÓ›˜ Ô˘‰ÂÙÂÚÔÂӛ˜, ·˘ÍËÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÙˆÓ ÎÔÎÎÈÔ΢ÙÙ¿ÚˆÓ (G-CSF), ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ.

Key words: congenital neutropenias, granulocyte colony-stimulating factor (G-CSF), bone marrow transplantation.

™˘ÁÁÂÓ›˜ Ô˘‰ÂÙÂÚÔÂӛ˜ ø˜ Ô˘‰ÂÙÂÚÔÂÓ›· ÔÚ›˙ÂÙ·È Ë Ì›ˆÛË ÙÔ˘ ·fiÏ˘ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÎÏÔÊÔÚÔ‡ÓÙˆÓ Ô˘‰ÂÙÂÚoÊ›ÏˆÓ ÛÙÔ ·›Ì· οو ·fi 1000 Ô˘‰ÂÙÂÚfiÊÈÏ·/mm3 Û ‚Ú¤ÊË ËÏÈΛ·˜ ‰‡Ô ‚‰ÔÌ¿‰ˆÓ ¤ˆ˜ ÂÓfi˜ ¤ÙÔ˘˜ Î·È Î¿Ùˆ ·fi 1500 ÌÂÙ¿ ÙÔÓ ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜ (1). ∞Ó¿ÏÔÁ· Ì ÙË ‚·Ú‡ÙËÙ·, Ë Ô˘‰ÂÙÂÚÔÂÓ›· ‰È·ÎÚ›ÓÂÙ·È Û ÂÏ·ÊÚ¿, Ì ·ÚÈıÌfi Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ ·fi 1000 ̤¯ÚÈ 1500/mm3, ̤ÙÚÈ·, Ì ·ÚÈıÌfi Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ ·fi 500 ̤¯ÚÈ 1000/mm3 Î·È ‚·ÚÈ¿,

Ì ·ÚÈıÌfi Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ ÌÈÎÚfiÙÂÚÔ ·fi 500/mm3. √È ·ÛıÂÓ›˜ Ì ‚·ÚÈ¿ Ô˘‰ÂÙÂÚÔÂÓ›· ¤¯Ô˘Ó ·˘ÍË̤ÓË Â˘·ÈÛıËÛ›· Û ·ÂÈÏËÙÈΤ˜, ÁÈ· ÙË ˙ˆ‹, ÏÔÈÌÒÍÂȘ. √È Û˘¯ÓfiÙÂÚ˜ ÌÔÚʤ˜ ÏÔÈÌÒÍÂˆÓ ·fi ˘ÔÁfiÓÔ˘˜ ÎfiÎÎÔ˘˜ Û ·ÛıÂÓ›˜ Ì ‚·ÚÈ¿ Ô˘‰ÂÙÂÚÔÂÓ›· ›ӷÈ: ‰ÂÚÌ·ÙÈ΋ ΢ÙÙ·Ú›ÙȘ, ÂÈÔÏ‹˜ ‹ ÂÓ Ùˆ ‚¿ıÂÈ ‰ÂÚÌ·ÙÈο ·ÔÛÙ‹Ì·Ù·, ‰ÔıÈ‹ÓˆÛË, Ó¢ÌÔÓ›· Î·È ÛË„·Ì›·. ∂›Û˘, Û˘Ì‚·›ÓÔ˘Ó ÛÙÔÌ·Ù›ÙȘ, Ô˘ÏÔÛÙÔÌ·Ù›ÙȘ, ÂÚÈÚˆÎÙÈ΋ ÊÏÂÁÌÔÓ‹ Î·È Ì¤ÛË ˆÙ›ÙȘ. √È ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ› Ô˘ Û˘Ó‹ıˆ˜ ·ÔÌÔÓÒÓÔÓÙ·È ·fi Ô˘‰ÂÙÂÚÔÂÓÈ-

¶·È‰ÔÔÁÎÔÏÔÁÈ΋ ∫ÏÈÓÈ΋ ÙÔ˘ πÔÎÚ·Ù›Ԣ ¡ÔÛÔÎÔÌ›Ԣ £ÂÛÛ·ÏÔӛ΢

Pediatric Oncology Clinic Ippokration Hospital of Thessaloniki

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¶·È‰È·ÙÚÈ΋ 2001;64:26-29

¶›Ó·Î·˜ 1. ∆·ÍÈÓfiÌËÛË Ô˘‰ÂÙÂÚÔÂÓÈÒÓ ™˘ÁÁÂÓ›˜ Ô˘‰ÂÙÂÚÔÂӛ˜ ∞ÁÂÓÂÛ›· ÙÔ˘ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·ÎÔ‡ ÈÛÙÔ‡ µ·ÚÈ¿ Û˘ÁÁÂÓ‹˜ Ô˘‰ÂÙÂÚÔÂÓ›· (Û. Kostmann) ∫˘ÎÏÈ΋ Ô˘‰ÂÙÂÚÔÂÓ›· ª˘ÂÏÔοıÂÍË (Myelokathexis) ™‡Ó‰ÚÔÌÔ Shwachman ™˘ÁÁÂÓ‹˜ ‰˘ÛÎÂÚ¿ÙˆÛË ™‡Ó‰ÚÔÌÔ Chediak-Higashi √ÈÎÔÁÂÓ‹˜ ηÏÔ‹ı˘ Ô˘‰ÂÙÂÚÔÂÓ›· Î.·. √˘‰ÂÙÂÚÔÂÓ›· ÔÊÂÈÏfiÌÂÓË Û Â͈ÁÂÓ›˜ ·Ú¿ÁÔÓÙ˜ √˘‰ÂÙÂÚÔÂÓ›· Û¯ÂÙÈ˙fiÌÂÓË Ì ÌÂÙ·‚ÔÏÈο ÓÔÛ‹Ì·Ù· ¢È‹ıËÛË ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·fi ÓÂÔÏ·ÛÌ·ÙÈο ·ÙÙ·Ú· ªË ·ÔÙÂÏÂÛÌ·ÙÈ΋ ˆÚ›Ì·ÓÛË ÙˆÓ Ì˘ÂÏÔ΢ÙÙ¿ÚˆÓ (ÌÂÁ·ÏÔ‚Ï·ÛÙÈ΋ ·Ó·ÈÌ›·) §ÔÈÌÒÍÂȘ πÛÔ¿ÓÔÛË ÓÂÔÁÓÈ΋ Ô˘‰ÂÙÂÚÔÂÓ›· ∞˘ÙÔ¿ÓÔÛË Ô˘‰ÂÙÂÚÔÂÓ›· √˘‰ÂÙÂÚÔÂÓ›· ÔÊÂÈÏfiÌÂÓË Û ʿÚ̷η ÀÂÚÛÏËÓÈÛÌfi˜

ÎÔ‡˜ ·ÛıÂÓ›˜ Â›Ó·È Ô ¯Ú˘Û›˙ˆÓ ÛÙ·Ê˘ÏfiÎÔÎÎÔ˜ Î·È gram-·ÚÓËÙÈο ‚·ÎÙËÚ›‰È· (2). ∏ Ù·ÍÈÓfiÌËÛË Ù˘ Ô˘‰ÂÙÂÚÔÂÓ›·˜ ·Ó¿ÏÔÁ· Ì ٷ ·›ÙÈ· Ê·›ÓÂÙ·È ÛÙÔÓ ›Ó·Î· 1. ∞ÁÂÓÂÛ›· ÙÔ˘ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·ÎÔ‡ ÈÛÙÔ‡ ∏ ·‰˘Ó·Ì›· ˆÚ›Ì·ÓÛ˘ ÙÔ˘ ·Ú¯¤ÁÔÓÔ˘ ÔÏ˘‰‡Ó·ÌÔ˘ ·ÈÌÔÔÈËÙÈÎÔ‡ ΢ÙÙ¿ÚÔ˘ (stem cell) Û ÚÔÁÔÓÈÎfi ÎÔÎÎÈÔ·ÙÙ·ÚÔ Î·È Î‡ÙÙ·ÚÔ Ù˘ ÏÂÌÊÈ΋˜ ÛÂÈÚ¿˜, ÔÚ›˙ÂÙ·È ˆ˜ ·ÁÂÓÂÛ›· ÙÔ˘ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·ÎÔ‡ ÈÛÙÔ‡. ∫ÏËÚÔÓÔÌÂ›Ù·È Ì ·˘ÙÔۈ̷ÙÈÎfi ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. ∂ÎÙfi˜ ·fi ÙË ‚·ÚÈ¿ Ô˘‰ÂÙÂÚÔÂÓ›·, Û˘Ó˘¿Ú¯ÂÈ ÏÂÌÊÔÂÓ›· Î·È ·Á·ÌÌ·ÛÊ·ÈÚÈÓ·ÈÌ›·, Ù· ‰Â ¿Û¯ÔÓÙ· ‚Ú¤ÊË ‰ÂÓ ÂÈ˙Ô‡Ó ÌÂÙ¿ ÙÔ˘˜ ÚÒÙÔ˘˜ Ì‹Ó˜ Ù˘ ˙ˆ‹˜. ∏ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ¤¯ÂÈ ÂÊ·ÚÌÔÛı› Ì ηϿ ·ÔÙÂϤÛÌ·Ù· Û ÌÈÎÚfi ·ÚÈıÌfi ·ÛıÂÓÒÓ (2,3). µ·ÚÈ¿ Û˘ÁÁÂÓ‹˜ Ô˘‰ÂÙÂÚÔÂÓ›· (Û‡Ó‰ÚÔÌÔ Kostmann) ∆Ô Û‡Ó‰ÚÔÌÔ ·˘Ùfi ÂÚÈÁÚ¿ÊËΠ·fi ÙÔÓ Kostmann ÙÔ 1956 Û·Ó Ì›· ‰È·Ù·Ú·¯‹ ÎÏËÚÔÓÔÌÔ‡ÌÂÓË Ì ·˘ÙÔۈ̷ÙÈÎfi ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. √È ¿Û¯ÔÓÙ˜ ¤¯Ô˘Ó ·fiÏ˘ÙÔ ·ÚÈıÌfi Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ ÌÈÎÚfiÙÂÚÔ ·fi 200/mm3 Î·È Û˘¯Ó¿ ¤¯Ô˘Ó ÂΉËÏÒÛÂȘ ˘ÚÂÙÔ‡, ÏÔÈÌÒÍÂˆÓ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜, ÛÙÔÌ·Ù›Ùȉ·˜ Î·È ÂÚÈÚˆÎÙÈÎÒÓ Û˘ÚÈÁÁ›ˆÓ Ô˘ Û˘Ó‹ıˆ˜ ÂÌÊ·Ó›˙ÔÓÙ·È ·fi ÙÔÓ ÚÒÙÔ Ì‹Ó· Ù˘ ˙ˆ‹˜. ∏ ÂͤٷÛË ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ‰Â›¯ÓÂÈ Ì›· Ê˘ÛÈÔÏÔÁÈ΋ ÂͤÏÈÍË ÙˆÓ Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ Ì¤¯ÚÈ ÙÔ ÛÙ¿‰ÈÔ ÙÔ˘ ÚÔÌ˘ÂÏÔ΢ÙÙ¿ÚÔ˘ ‹ Ì˘ÂÏÔ-

Paediatriki 2001;64:26-29

΢ÙÙ¿ÚÔ˘, ÂÓÒ ·Ô˘ÛÈ¿˙Ô˘Ó Ù· ÒÚÈÌ· Ô˘‰ÂÙÂÚfiÊÈÏ·. ∏ ·Ú·ÁˆÁ‹ G-CSF ÛÙÔ˘˜ ¿Û¯ÔÓÙ˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋, ·Ó fi¯È ·˘ÍË̤ÓË. ∏ ‰È·Ù·Ú·¯‹ ‚Ú›ÛÎÂÙ·È ÛÙÔ Â›Â‰Ô ÙÔ˘ ˘Ô‰Ô¯¤· ÙÔ˘ G-CSF (GCSF receptor, G-CSF-R) ‹ ÛÙËÓ ÂÓ‰Ô΢ÙÙ¿ÚÈ· ÌÂÙ·ÁˆÁ‹ Û‹Ì·ÙÔ˜ Ô˘ Ù›ıÂÙ·È Û ÂÓ¤ÚÁÂÈ· ·fi ÙÔÓ G-CSF-R ‹ Î·È ÛÙ· ‰‡Ô (4). √È ¿Û¯ÔÓÙ˜ ·fi Û‡Ó‰ÚÔÌÔ Kostmann ¤¯Ô˘Ó ·˘ÍË̤ÓË Èı·ÓfiÙËÙ· Ó· ÂΉËÏÒÛÔ˘Ó ÔÍ›· Ì˘ÂÏÔÁÂÓ‹ Ï¢¯·ÈÌ›·. ∏ ÛÏËÓÂÎÙÔÌ‹, Ù· ÎÔÚÙÈÎÔÂȉ‹ Î·È Ù· ·Ó‰ÚÔÁfiÓ· ‰ÔÎÈÌ¿ÛÙËÎ·Ó ÛÙÔ ·ÚÂÏıfiÓ ¯ˆÚ›˜ ÂÈÙ˘¯›·. ∏ ¯Ú‹ÛË ·ÓÙÈ‚ÈÔÙÈÎÒÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÏÔÈÌÒÍÂˆÓ ‚ÔËı¿ ÛÙËÓ ·Ú¿Ù·ÛË Ù˘ ˙ˆ‹˜ ÙˆÓ ·ÛıÂÓÒÓ. ∏ ¯ÔÚ‹ÁËÛË G-CSF ‚ÔËı¿ ÛËÌ·ÓÙÈο ÛÙÔÓ ÂÚÈÔÚÈÛÌfi ÙˆÓ ÏÔÈÌÒÍÂˆÓ Î·È ÙËÓ Ì·ÎÚ‡ÙÂÚË ÂÈ‚›ˆÛË ÙˆÓ ·Û¯fiÓÙˆÓ, ·Ó Î·È ‰ÂÓ ··ÓÙÔ‡Ó fiÏÔÈ ÔÈ ·ÛıÂÓ›˜. ∏ ‰fiÛË ÙÔ˘ G-CSF ÁÈ· ÙÔÓ Î¿ı ·ÛıÂÓ‹ Î˘Ì·›ÓÂÙ·È ·fi 3 ¤ˆ˜ 6 Ìg/kgµ™/∏ (2). ∏ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ÂÈÙ˘Á¯¿ÓÂÈ ÈÔ ÌfiÓÈÌ· ·ÔÙÂϤÛÌ·Ù· ÛÙË ‰ÈfiÚıˆÛË Ù˘ ·ÎÔÎÎÈÔ΢ÙÙ·Ú·ÈÌ›·˜ ÎÈ ·ÔÙÚ¤ÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÂΉ‹ÏˆÛ˘ ÔÍ›·˜ Ì˘ÂÏÔÁÂÓÔ‡˜ Ï¢¯·ÈÌ›·˜ (5). ∫˘ÎÏÈ΋ Ô˘‰ÂÙÂÚÔÂÓ›· ∏ ΢ÎÏÈ΋ Ô˘‰ÂÙÂÚÔÂÓ›· Â›Ó·È Ì›· Û¿ÓÈ· ‰È·Ù·Ú·¯‹ Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÂÚÈÔ‰È΋ -·Ó¿ 21- Ë̤Ú˜ ‰È·Î‡Ì·ÓÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÎÏÔÊÔÚÔ‡ÓÙˆÓ Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ ·fi Ù· Ê˘ÛÈÔÏÔÁÈο ̤¯ÚÈ Ù· Ô˘‰ÂÙÂÚÔÂÓÈο Â›‰·. ™Â ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ Ù· ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ÙÙ·Ú·, Ù· ·ÈÌÔÂÙ¿ÏÈ· Î·È ÔÈ ¿ÏϘ ÌÔÚʤ˜ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ ¤¯Ô˘Ó, Â›Û˘, ÌÈ· ΢ÎÏÈ΋ ‰È·Î‡Ì·ÓÛË. ∏ ΢ÎÏÈ΋ Ô˘‰ÂÙÂÚÔÂÓ›· ÎÏËÚÔÓÔÌÂ›Ù·È Ì ۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú·, ·ÏÏ¿ Û ÔÏϤ˜ ÂÚÈÙÒÛÂȘ Ë ÓfiÛÔ˜ ÂΉËÏÒÓÂÙ·È ÛÔÚ·‰Èο. ™ÙÔÓ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Ù· ÚÔÁÔÓÈο ÎÔÎÎÈÔ-/ÌÔÓÔ- ·ÙÙ·Ú· Â›Ó·È ·fiÓÙ· 1 ‚‰ÔÌ¿‰· ÚÈÓ ·fi ÙÔ Ô˘‰ÂÙÂÚÔÂÓÈÎfi Ó·‰›Ú, ÙÔ ÔÔ›Ô ‰È·ÚΛ 3-10 ̤Ú˜. ∏ ˘fiÓÔÈ· Ù˘ ÓfiÛÔ˘ Ù›ıÂÙ·È ·fi Â·Ó·Ï·Ì‚·ÓfiÌÂÓ˜ ·Ó¿ 21 ̤Ú˜ ÏÔÈÌÒÍÂȘ, ÂÓÒ ÁÈ· ÙËÓ ÂȂ‚·›ˆÛË ¯ÚÂÈ¿˙ÂÙ·È Ó· Á›ÓÂÙ·È ÁÂÓÈ΋ ÂͤٷÛË ·›Ì·ÙÔ˜ 2-3 ÊÔÚ¤˜ ÙËÓ Â‚‰ÔÌ¿‰· ÁÈ· ‰È¿ÛÙËÌ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 2 ÌËÓÒÓ. ∞fi ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ Ê·›ÓÂÙ·È fiÙÈ ˘¿Ú¯ÂÈ ‰È·Ù·Ú·¯‹ ÛÙÔ˘˜ Ú˘ıÌÈÛÙÈÎÔ‡˜ Ì˯·ÓÈÛÌÔ‡˜ Ô˘ ·ÊÔÚÔ‡Ó ÙȘ ÚÔÁÔÓÈΤ˜ ÌÔÚʤ˜ ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ Ô˘‰ÂÙÂÚÔÂÓÈÎÒÓ ÂÚÈfi‰ˆÓ, Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ ÂÌÊ·Ó›˙ÂÈ ÛÙÔÌ·ÙÈο ¤ÏÎË, ÛÙÔÌ·Ù›Ùȉ· Î·È Ê·Ú˘ÁÁ›Ùȉ· Û˘Óԉ¢fiÌÂÓË ·fi ‰ÈfiÁΈÛË ÙˆÓ ÏÂÌÊ·‰¤ÓˆÓ. ¶ÈÔ ÛÔ‚·Ú¤˜ ÏÔÈÌÒÍÂȘ ÌÔÚ›, Â›Û˘, Ó· ÂÈÛ˘Ì‚Ô‡Ó. √È ¿Û¯ÔÓÙ˜ ·fi ΢ÎÏÈ΋ Ô˘‰ÂÙÂÚÔÂÓ›· Â›Ó·È ÂÈÚÚÂ›˜ Û ÏÔÈÌÒÍÂȘ ·fi clostridium per-

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fringens. ªÂٷ͇ ÙˆÓ ÚÔÛ‚ÔÏÒÓ ÔÈ ¿Û¯ÔÓÙ˜ ¤¯Ô˘Ó ηϋ ˘Á›·. ™Â ÌÂÚÈÎÔ‡˜ ·ÛıÂÓ›˜ Ì ÙËÓ ¿ÚÔ‰Ô Ù˘ ËÏÈΛ·˜ ˘¿Ú¯ÂÈ ‚ÂÏÙ›ˆÛË Ù˘ ÎÏÈÓÈ΋˜ ÂÈÎfiÓ·˜, ηıfiÛÔÓ ÔÈ Ô˘‰ÂÙÂÚÔÂÓÈÎÔ› ·ÎÏÔÈ Á›ÓÔÓÙ·È ÏÈÁfiÙÂÚÔ ÂÎÛÂÛËÌ·Ṳ̂ÓÔÈ. ∏ ΢ÎÏÈ΋ Ô˘‰ÂÙÂÚÔÂÓ›· ‰ÂÓ Û¯ÂÙ›˙ÂÙ·È Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ·Ó¿Ù˘ÍË Ì˘ÂÏÔÁÂÓÔ‡˜ Ï¢¯·ÈÌ›·˜, Û ·ÓÙ›ıÂÛË Ì ÙËÓ ‚·ÚÈ¿ Û˘ÁÁÂÓ‹ Ô˘‰ÂÙÂÚÔÂÓ›·. °È· Ó· ÌÂȈı› Ô Î›Ó‰˘ÓÔ˜ ÛÔ‚·ÚÒÓ ÏÔÈÌÒÍÂˆÓ Î·Ù¿ ÙȘ Ê¿ÛÂȘ Ù˘ Ô˘‰ÂÙÂÚÔÂÓ›·˜, ÔÈ ·ÛıÂÓ›˜ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì G-CSF (3-10Ìg/kg µ™/∏) (6). √ GM-CSF ‰ÂÓ ¤¯ÂÈ ÙÔ ›‰ÈÔ Î·Ï¿ ·ÔÙÂϤÛÌ·Ù·. Myelokathexis (ª˘ÂÏÔοıÂÍȘ) √ fiÚÔ˜ ÚÔ¤Ú¯ÂÙ·È ·fi ÙȘ ÂÏÏËÓÈΤ˜ ϤÍÂȘ «Ì˘ÂÏfi˜» Î·È «Î¿ıÂÍȘ» Ô˘ ÛËÌ·›ÓÂÈ ÂÚÈÔÚÈÛÌfi˜, ·Ó·¯·›ÙÈÛË (·Ó·¯·›ÙÈÛË ÙˆÓ Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ ÂÓÙfi˜ ÙÔ˘ Ì˘ÂÏÔ‡). ∂›Ó·È Ì›· Û˘ÁÁÂÓ‹˜ ‰È·Ù·Ú·¯‹ Ô˘ ÚÔηÏ› ‚·ÚÈ¿ ¯ÚfiÓÈ· Ï¢ÎÔÂÓ›· Î·È Ô˘‰ÂÙÂÚÔÂÓ›·. ∆· Ô˘‰ÂÙÂÚfiÊÈÏ· ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· ¤¯Ô˘Ó ΢ÙÙ·ÚÔÏ·ÛÌ·ÙÈο ÎÂÓÔÙfiÈ·, ÔÈ ‰Â ˘Ú‹Ó˜ ÙÔ˘˜ ¤¯Ô˘Ó ÔÏÏ¿ Ïfi‚È· Û˘Ó‰Â‰Â̤ӷ ÌÂٷ͇ ÙÔ˘˜ Ì Á¤Ê˘Ú˜. √ Ì˘ÂÏfi˜ ÙˆÓ ÔÛÙÒÓ ·ÚÔ˘ÛÈ¿˙ÂÈ ÂÈÎfiÓ· ÚÒÈÌ˘ ·fiÙˆÛ˘ ÙˆÓ Úfi‰ÚÔÌˆÓ ÌÔÚÊÒÓ ÙˆÓ Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ (˘ÂÚÏ·Û›· Ì ˘ÂÚηٿÙÌËÛË ÙÔ˘ ˘Ú‹Ó· ÙˆÓ Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ Î·È ÂÓ‰ÔÌ˘ÂÏÈ΋ ηٷÛÙÚÔÊ‹ ·˘ÙÒÓ) (7). ∏ Ô˘‰ÂÙÂÚÔÂÓ›· ÛÙË ÓfiÛÔ ·˘Ù‹ ÌÔÚ› ÌÂÚÈÎÒ˜ Ó· ‰ÈÔÚıˆı› Ì ÙË ¯ÔÚ‹ÁËÛË G-CSF ‹ GMCSF. ∞ӷʤÚÂÙ·È Î·È Ì˘ÂÏÔοıÂÍË Ì ˘ÔÁ·ÌÌ·ÛÊ·ÈÚÈÓ·ÈÌ›·. ™‡Ó‰ÚÔÌÔ Shwachman ∂›Ó·È Ì›· Û¿ÓÈ· ÔÏ˘ÔÚÁ·ÓÈ΋ ÓfiÛÔ˜ Ô˘ ÎÏËÚÔÓÔÌÂ›Ù·È Ì ·˘ÙÔۈ̷ÙÈÎfi ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ÌÂÙ·Ê˘Ûȷ΋ ¯ÔÓ‰ÚÔ‰˘ÛÏ·Û›·, Ó·ÓÈÛÌfi, ·ÓÂ¿ÚÎÂÈ· Ù˘ Â͈ÎÚÈÓÔ‡˜ ÌÔ›Ú·˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜ Î·È Ô˘‰ÂÙÂÚÔÂÓ›·. √ ·ÚÈıÌfi˜ ÙˆÓ Ô˘‰ÂÙÂÚÔÊ›ÏˆÓ ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· ÔÈΛÏÏÂÈ, Î˘Ì·ÈÓfiÌÂÓÔ˜ ·fi 200 ̤¯ÚÈ 400 ·ÙÙ·Ú·/mm3. §fiÁˆ Ù˘ ÛÙ·ÙfiÚÚÔÈ·˜ Î·È ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ÏÔÈÌÒÍÂˆÓ ÌÔÚ› Ó· ıˆÚËı›, ÛÙ· ·Ú¯Èο ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘, ˆ˜ ΢ÛÙÈ΋ ›ÓˆÛË, ·fi ÙËÓ ÔÔ›·, fï˜, ‡ÎÔÏ· ‰È·ÎÚ›ÓÂÙ·È ÏfiÁˆ ÙÔ˘ ·ÚÓËÙÈÎÔ‡ test ȉÚÒÙ·. ŒÓ· ̤ÚÔ˜ ÙˆÓ ·ÛıÂÓÒÓ, ÂÚ›Ô˘ 25%, ÂÌÊ·Ó›˙ÂÈ ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· ÛÙËÓ ÔÚ›· Ù˘ ÓfiÛÔ˘. √È ˘ÔÙÚÔÈ¿˙Ô˘Û˜ ‚·ÚȤ˜ ‚·ÎÙËÚȷΤ˜ ÏÔÈÌÒÍÂȘ ÎÈ Ë ÂΉ‹ÏˆÛË Ì˘ÂÏÔÁÂÓÔ‡˜ Ï¢¯·ÈÌ›·˜ Â›Ó·È ÔÈ Î‡ÚȘ ·Èٛ˜ ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÙfiÙËÙ·˜ ÛÙÔ˘˜ ¿Û¯ÔÓÙ˜ ·fi Û‡Ó‰ÚÔÌÔ Shwachman.

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Paediatriki 2001;64:26-29

°È· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Ô˘‰ÂÙÂÚÔÂÓ›·˜ ¯ÔÚËÁÂ›Ù·È G-CSF Ì ηϿ ·ÔÙÂϤÛÌ·Ù·. °È· ÙÔ Û‡Ó‰ÚÔÌÔ Shwachman ıÂÚ·›· ÂÎÏÔÁ‹˜ ıˆÚÂ›Ù·È Ë ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ, ‰ÈfiÙÈ ÂÎÙfi˜ ·fi ÙË ‰ÈfiÚıˆÛË ÙˆÓ ·ÈÌ·ÙÔÏÔÁÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ ·Ó·¯·ÈÙ›˙ÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÂÌÊ¿ÓÈÛ˘ Ì˘ÂÏÔÁÂÓÔ‡˜ Ï¢¯·ÈÌ›·˜ (8). ™˘ÁÁÂÓ‹˜ ‰˘ÛÎÂÚ¿ÙˆÛË ∫ÏËÚÔÓÔÌÂ›Ù·È Ì ˘ÔÏÂÈfiÌÂÓÔ ÁfiÓÔ Â˘ÚÈÛÎfiÌÂÓÔ ÛÙÔ ¯ÚˆÌfiۈ̷ Ã. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ‰˘ÛÙÚÔÊ›· ÔÓ‡¯ˆÓ, Ï¢ÎÔϷΛ·, ˘¤Ú¯ÚˆÛË ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ Î·È Ô˘‰ÂÙÂÚÔÂÓ›·. √È ·ÛıÂÓ›˜ Û˘Ó‹ıˆ˜ Êı¿ÓÔ˘Ó ÛÙËÓ ÂÓËÏÈΛˆÛË. £ÂÚ·¢ÙÈο ¯ÔÚËÁÂ›Ù·È G-CSF (9). ™‡Ó‰ÚÔÌÔ Chediak - Higashi ∂›Ó·È Ì›· Û¿ÓÈ· ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ÌÂÚÈÎfi ÔÊı·ÏÌÔ‰ÂÚÌ·ÙÈÎfi ·ÏÊÈÛÌfi, ·ÚÔ˘Û›· ÁÈÁ¿ÓÙÈˆÓ (2 - 5Ìm) ÁÎÚÈ-ÌÏ ÎÔÎΛˆÓ ÛÙÔ Î˘ÙÙ·ÚfiÏ·ÛÌ· ÙˆÓ Ô˘‰ÂÙÂÚÔʛψÓ, ˈÛÈÓÔʛψÓ, ‚·ÛÂÔÊ›ÏˆÓ Î·È ·ÈÌÔÂÙ·Ï›ˆÓ. √È ·ÛıÂÓ›˜ Ì ·˘Ùfi ÙÔ Û‡Ó‰ÚÔÌÔ ¤¯Ô˘Ó ·˘ÍË̤ÓË Â˘·ÈÛıËÛ›· Û ÏÔÈÌÒÍÂȘ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ Î·È Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡. √È ÂÚÈÛÛfiÙÂÚÔÈ Î·Ù·Ï‹ÁÔ˘Ó ÚÈÓ ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 10 ÂÙÒÓ, ·Ó Î·È ÔÚÈṲ̂ÓÔÈ ·ÛıÂÓ›˜ ¤Ù˘¯·Ó Ì›· Ì·ÎÚ¿ ÂÈ‚›ˆÛË ÌÂÙ¿ ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (1). √ÈÎÔÁÂÓ‹˜ ηÏÔ‹ı˘ Ô˘‰ÂÙÂÚÔÂÓ›· ‹ ¯ÚfiÓÈ· ȉÈÔ·ı‹˜ Ô˘‰ÂÙÂÚÔÂÓ›· ™Â ·˘Ù‹ ÙËÓ ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ· ÂÚÈÏ·Ì‚¿ÓÂÙ·È Ì›· ÂÙÂÚÔÁÂÓ‹˜ ÔÌ¿‰· ·ÛıÂÓÒÓ. ∏ ·ÈÙ›· ‰ÂÓ Â›Ó·È ·fiÏ˘Ù· ‰È¢ÎÚÈÓÈṲ̂ÓË. À¿Ú¯ÂÈ ÌÂȈ̤ÓË ‹ ÌË ·ÔÙÂÏÂÛÌ·ÙÈ΋ ·Ú·ÁˆÁ‹ Ô˘‰ÂÙÂÚÔʛψÓ, ÁÈ· ÙËÓ ÔÔ›· Èı·ÓfiÓ Â˘ı‡ÓÔÓÙ·È ‰È·ÊÔÚÂÙÈÎÔ› Ì˯·ÓÈÛÌÔ› ·fi ·ÛıÂÓ‹ Û ·ÛıÂÓ‹. ∞ӷʤÚÔÓÙ·È ÂÚÈÙÒÛÂȘ ·˘ÙfiÌ·Ù˘ ›·Û˘ Û ËÏÈΛ· 2-4 ÂÙÒÓ, ·ÏÏ¿ Î·È ÌÂÙ¿ ·fi ·˘Ù‹ ÙËÓ ËÏÈΛ· (2). √È ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ Ù˘ ÓfiÛÔ˘ Â›Ó·È ÛÔÚ·‰ÈΤ˜. Œ¯ÂÈ ÂÚÈÁÚ·Ê› ÎÏËÚÔÓÔÌÈÎfiÙËÙ· Ì ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú· Û ÌÂÚÈΤ˜ ÔÈÎÔÁ¤ÓÂȘ, ΢ڛˆ˜ ÛÙÔ˘˜ ∂‚Ú·›Ô˘˜ Ù˘ ÀÂ̤Ó˘. ∏ ÌÔÚÊÔÏÔÁ›· ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ÔÈΛÏÏÂÈ ·fi ·ÛıÂÓ‹ Û ·ÛıÂÓ‹, ·ÏÏ¿ Î·È ÛÙÔÓ ›‰ÈÔ ÙÔÓ ·ÛıÂÓ‹ ·fi Ï‹„ÂȘ Ô˘ Á›ÓÔÓÙ·È Û ‰È·ÊÔÚÂÙÈÎÔ‡˜ ¯ÚfiÓÔ˘˜. √ ·ÚÈıÌfi˜ ÙˆÓ ÚÔ‰ÚÔÌÈÎÒÓ ÎÔÎÎÈÔ-/ÌÔÓÔ- ΢ÙÙ¿ÚˆÓ ÛÙÔÓ Ì˘ÂÏfi Â›Ó·È Â·Ú΋˜, ·Ú·ÙËÚÂ›Ù·È ‰È·ÎÔ‹ Ù˘ ˆÚ›Ì·ÓÛ˘ Û ‰È¿ÊÔÚ· ÛÙ¿‰È· ·fi ÙÔ ÚÔÌ˘ÂÏÔ·ÙÙ·ÚÔ Ì¤¯ÚÈ ÙȘ ÒÚÈ̘ ÌÔÚʤ˜ ÙˆÓ Ô˘‰ÂÙÂÚÔʛψÓ. £ÂÚ·¢ÙÈο ÔÚÈṲ̂ÓÔÈ ·ÛıÂÓ›˜ ··ÓÙÔ‡Ó


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¶·È‰È·ÙÚÈ΋ 2001;64:26-29

ÛÙ· ÎÔÚÙÈÎÔÂȉ‹. ∏ ¯ÔÚ‹ÁËÛË G-CSF ¤¯ÂÈ, Â›Û˘, ηϿ ·ÔÙÂϤÛÌ·Ù· (1,2). ÀÔÏ·Û›· ¯fiÓ‰ÚˆÓ – ÙÚȯÒÓ (Cartilage – hair hypoplasia) ∫ÏËÚÔÓÔÌÂ›Ù·È Ì ·˘ÙÔۈ̷ÙÈÎfi ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. √È ¿Û¯ÔÓÙ˜ ÂÌÊ·Ó›˙Ô˘Ó Ó·ÓÈÛÌfi Ì ÎÔÓÙ¿ οو ¿ÎÚ·, ÏÂÙ¤˜ ÙÚ›¯Â˜, ̤ÙÚÈ· Ô˘‰ÂÙÂÚÔÂÓ›· (100–2000 Ô˘‰ÂÙÂÚfiÊÈÏ·/mm3) Î·È ‰È·Ù·Ú·¯‹ Ù˘ ΢ÙÙ·ÚÈ΋˜ ·ÓÔÛ›·˜. ∞ӷʤÚÂÙ·È ·ÔηٿÛÙ·ÛË ÙˆÓ ·ÓÔÛÈ·ÎÒÓ ÏÂÈÙÔ˘ÚÁÈÒÓ Î·È Ù˘ Ô˘‰ÂÙÂÚÔÂÓ›·˜ ÌÂÙ¿ ·fi ·ÏÏÔÁÂÓ‹ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·fi HLA–ÈÛÙÔÛ˘Ì‚·ÙÔ‡˜ ‰fiÙ˜ (2,10). ÕÏÏ· Û˘ÁÁÂÓ‹ ÓÔÛ‹Ì·Ù· Ô˘ Û˘Óԉ‡ÔÓÙ·È ·fi Ô˘‰ÂÙÂÚÔÂÓ›· ›ӷÈ: Ë ·Ó·ÈÌ›· Fanconi, Ë ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, Ù· Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈο Û‡Ó‰ÚÔÌ·, Ë ·Á·ÌÌ·ÛÊ·ÈÚÈÓ·ÈÌ›· (1/3 ÙˆÓ ·ÛıÂÓÒÓ), Ë ‰˘ÛÁ·ÌÌ·ÛÊ·ÈÚÈÓ·ÈÌ›· Ù‡Ô˘ I, ÎÏ. µÈ‚ÏÈÔÁÚ·Ê›· 1. Bernini JC. Diagnosis and management of chronic neutropenia during childhood. Pediatr Clin N Am 1996; 43:773-775. 2. ¡athan DG, Oski FA. Hematology of infancy and childhood. 4th ed. Philadelphia: Saunders Co; 1993. p. 939-945. 3. Levinsky RJ, Tiedeman K. Successful bone - marrow transplantation for reticular dysgenesis. Lancet 1983;1:671.

Paediatriki 2001;64:26-29

4. Deshpande RV, Lalezari P, Pergolizzi RG, Moore MA. Structural abnormalities in the G-CSF receptor in severe congenital neutropenia. J Hematother Stem Cell Res 1999;8:411-420. 5. Zeidler C, Welte K, Barak Y, Barriga F, Bolyard AA, Boxer L et al. Stem cell transplantation in patients with severe congenital neutropenia without evidence of leukemic transformation. Blood 2000;95:1195-1198. 6. Dale DC, Hammond WP. Cyclic neutropenia: A clinical review. Blood Rev 1998;2:178. 7. ∞prikyan AA, Liles WC, Park JR, Jonas M, Chi EY, Dale DC. Myelokathexis, a congenital disorder of severe neutropenia characterized by accelerated apoptosis and defective expression of bcl-x in neutrophil precursors. Blood 2000;95:320-327. 8. Faber J, Lauener R, Wick F, Betts D, Filgueira L, Seger RA et al. Shwachman–Diamond syndrome: early bone marrow transplantation in a high risk patient and new clues to pathogenesis. Eur J Pediatr 1999;158:995-1000. 9. Dokal I. Dyskeratosis congenita: recent advances and future directions. J Pediatr Hematol Oncol 1999;21:344-350. 10. Makitie O, Juvonen E, Dunkel L, Kaitila I, Siimes MA. Anemia in children with cartilage – hair hypoplasia is related to body growth and to the insulin – like growth HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 02-10-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 21-11-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∞. ∫·Ú·ÎfiÏË £. ™ÔÊÔ‡ÏË 50, 54655 £ÂÛÛ·ÏÔÓ›ÎË

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¶·È‰È·ÙÚÈ΋ 2001;64:30-33

™À¡∂Ãπ∑√ª∂¡∏ ¶∞π¢π∞∆ƒπ∫∏ ∂∫¶∞π¢∂À™∏

Paediatriki 2001;64:30-33

CONTINUING MEDICAL EDUCATION

£ÚÔÌ‚ÔÂӛ˜ ÔÊÂÈÏfiÌÂÓ˜ Û ·ÓÂ·Ú΋ ·Ú·ÁˆÁ‹ ·ÈÌÔÂÙ·Ï›ˆÓ ∂. ¶Ï·ÙÔÎÔ‡ÎË - ∫ÔÌÈÙÔÔ‡ÏÔ˘

Thrombocytopenias resulting from deficient platelet production H. Platokouki - Komitopoulou

¶ÂÚ›ÏË„Ë: ∂›‰· ·ÈÌÔÂÙ·Ï›ˆÓ ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· οو ·fi Ù· ηÙÒÙÂÚ· Ê˘ÛÈÔÏÔÁÈο ÔÚ›˙ÂÙ·È ˆ˜ ıÚÔÌ‚ÔÂÓ›·. ¶·Ú¿ ÙÔ ÌÂÁ¿ÏÔ ·ÚÈıÌfi Î·È ÙËÓ ÔÈÎÈÏ›· ÙˆÓ ·ÈÙ›ˆÓ Ô˘ ÙËÓ ÚÔηÏÔ‡Ó, Ë ıÚÔÌ‚ÔÂÓ›· Â›Ó·È ÙÔ ·ÔÙ¤ÏÂÛÌ· ÙÚÈÒÓ ‰ÈÂÚÁ·ÛÈÒÓ: ÌÂȈ̤Ó˘ ·Ú·ÁˆÁ‹˜ ·ÈÌÔÂÙ·Ï›ˆÓ ·fi ÙÔÓ Ì˘ÂÏfi, ·˘ÍË̤Ó˘ ηٷÛÙÚÔÊ‹˜ ‹ ·ÓÒÌ·Ï˘ ηٷÓÔÌ‹˜ ÛÙËÓ ÂÚÈʤÚÂÈ·. ™ÙËÓ ÂÈÛ‹ÁËÛË ·˘Ù‹ ηٷÁÚ¿ÊÂÙ·È Ë Î·Ù¿Ù·ÍË ÙˆÓ ıÚÔÌ‚ÔÂÓÈÒÓ Ô˘ ÔÊ›ÏÔÓÙ·È Û ·ÓÂ¿ÚÎÂÈ· ·Ú·ÁˆÁ‹˜ ·ÈÌÔÂÙ·Ï›ˆÓ ‚¿ÛÂÈ ÙÔ˘ ·ıÔÊ˘ÛÈÔÏÔÁÈÎÔ‡ Ì˯·ÓÈÛÌÔ‡ Ô˘ ÂÌϤÎÂÙ·È Î·È Û˘˙ËÙÂ›Ù·È ‚Ú·¯¤ˆ˜ Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· Î·È Ù· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·.

Abstract: Thrombocytopenia is defined as a subnormal platelet count in the circulating blood. Despite the number and the diversity of disorders that may be associated aetiologically, thrombocytopenia results from only three processes: deficient platelet production, accelerated platelet destruction and abnormal distribution or pooling of the platelets within the body. ∞ classification of thrombocytopenia resulting from deficient platelet production based on pathophysiologic, clinical and laboratory criteria is presented and briefly discussed in this paper.

§¤ÍÂȘ ÎÏÂȉȿ: ıÚÔÌ‚ÔÂÓ›·, ÌÂÁ·Î·Ú˘Ô·ÙÙ·ÚÔ, ·Ï·Û›·, ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ıÚÔÌ‚ÔÔ›ËÛË.

Key words: thrombocytopenia, megakaryocyte, aplasia, ineffective thrombopoiesis.

ø˜ ıÚÔÌ‚ÔÂÓ›· ÔÚ›˙ÂÙ·È ·ÚÈıÌfi˜ ·ÈÌÔÂÙ·Ï›ˆÓ (∞ª¶) ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· <150.000/mm3, Ô˘ Â›Ó·È Ù· ηÙÒÙÂÚ· fiÚÈ· ÁÈ· οı ËÏÈΛ·. ∏ ÛÔ‚·Ú‹ ıÚÔÌ‚ÔÂÓ›· (∞ª¶ <50.000/mm3 ) ·ÔÙÂÏ› ÙË Û˘¯ÓfiÙÂÚË ·ÈÙ›· ·ÈÌÔÚÚ·Á›·˜ Ô˘ ·Ô‰›‰ÂÙ·È Û ·ıÔÏÔÁÈο ·›ÙÈ·(1,2). ¶·Ú¿, fï˜, ÙÔ ÌÂÁ¿ÏÔ ·ÚÈıÌfi Î·È ÙËÓ ÔÈÎÈÏ›· ÙˆÓ ·ÈÙ›ˆÓ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ıÚÔÌ‚ÔÂÓ›·, ·˘Ù‹ Û ÙÂÏÈ΋ ·Ó¿Ï˘ÛË ·ÔÙÂÏ› ÙÔ ÚÔ˚fiÓ ÙÚÈÒÓ ‰ÈÂÚÁ·ÛÈÒÓ, ‰ËÏ·‰‹: ·ÓÂ·ÚÎÔ‡˜ ·Ú·ÁˆÁ‹˜ ∞ª¶, ·˘ÍË̤Ó˘ ηٷÛÙÚÔÊ‹˜ Î·È ‰È·Ù·Ú·¯‹˜ ηٷÓÔÌ‹˜ ÛÙËÓ ÂÚÈʤÚÂÈ· (3-5). °È· ÙË ‰È¿ÎÚÈÛË ÌÂٷ͇ ÙÔ˘˜, ¯Ú‹ÛÈÌË ·Ú¿ÌÂÙÚÔ˜ ı· ‹Ù·Ó Ë ·¢ı›·˜ ̤ÙÚËÛË ÙÔ˘ Ú˘ıÌÔ‡ ·Ú·ÁˆÁ‹˜ Ó¤ˆÓ ∞ª¶, fiˆ˜ Û˘Ì‚·›ÓÂÈ Ì ٷ ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ·ٷÚÚ· ÙˆÓ ÂÚ˘ıÚÒÓ. ∂ÏÏ›„ÂÈ Ù¤ÙÔÈ·˜ ‰˘-

Ó·ÙfiÙËÙ·˜ ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ¤ÌÌÂÛÔÈ ‰Â›ÎÙ˜, fiˆ˜ Ë Û˘ÓÔ‰fi˜ ·Ï·Û›· Î·È ÙˆÓ ¿ÏÏˆÓ ÛÂÈÚÒÓ ‹ ÔÈ ¯·ÌËϤ˜ ÙÈ̤˜ ÙÔ˘ ̤ÛÔ˘ fiÁÎÔ˘ ·ÈÌÔÂÙ·Ï›ˆÓ (mean platelet volume, MPV) Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È Û ıÚÔÌ‚ÔÂӛ˜ ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜. ∏ ΢ÙÙ·ÚÔÌÂÙÚ›· ÚÔ‹˜ ·Ô‰ÂÈÎÓ‡ÂÙ·È, ·ÓÙÈı¤Ùˆ˜, ¯Ú‹ÛÈÌË Ì¤ıÔ‰Ô˜ ÁÈ· ÙË Ì¤ÙÚËÛË ÌÂÁ¿ÏˆÓ Ó·ÚÒÓ ∞ª¶ Ô˘ ÂÚȤ¯Ô˘Ó RNA Î·È ÙÔ ÔÔ›Ô ˘fi ηÓÔÓÈΤ˜ Û˘Óı‹Î˜ ‰ÂÓ ÂÌÂÚȤ¯ÂÙ·È ÛÙ· ∞ª¶ Ô˘ ΢ÎÏÔÊÔÚÔ‡Ó ÛÙÔ ·›Ì·. µÂ‚·›ˆ˜, ÙÔ Ì˘ÂÏfiÁÚ·ÌÌ· Â›Ó·È Ë ÂͤٷÛË ÂÎÏÔÁ‹˜ (2,4,6). ∆· ·ÈÌÔÂÙ¿ÏÈ·, ˆ˜ ÁÓˆÛÙfiÓ, ·ÔÙÂÏÔ‡Ó ÙÔ ÙÂÏÈÎfi ÚÔ˚fiÓ Ì›·˜ ‰È·‰Èηۛ·˜ Ô˘ ÍÂÎÈÓ¿ ·fi ÙÔ ÔÏ˘‰‡Ó·ÌÔ ·Ú¯¤ÁÔÓÔ ·ÈÌÔÔÈËÙÈÎfi ·ÙÙ·ÚÔ Ô˘ ‰È·ÊÔÚÔÔÈÂ›Ù·È ÛÙ·‰È·Î¿ Û ÚÔÁÔÓÈο ·ÙÙ·Ú· ηÈ

ªÔÓ¿‰· ∞ÈÌÔÚÚ·ÁÈÎÒÓ ¢È·ı¤ÛˆÓ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó·

Division of Hemorrhagic Diathesis “Saint Sophia” Children’s Hospital, Athens

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 31

¶·È‰È·ÙÚÈ΋ 2001;64:30-33

Úfi‰ÚÔ̘ ÌÔÚʤ˜. ∞Ó·ÏfiÁˆ˜ ÙÔ˘ ÛÙ·‰›Ô˘ Ù˘ ˆÚ›Ì·ÓÛ˘, ‰È·Ê¤ÚÂÈ Ë ÈηÓfiÙËÙ· ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÁÈ· ·Ó·Ó¤ˆÛË, ·Ó¿Ù˘ÍË Î·È ‰Ú·ÛÙËÚÈfiÙËÙ· (7). ™ÙË Ú‡ıÌÈÛË Ù˘ ÌÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÔÔ›ËÛ˘ ÂÌϤÎÔÓÙ·È ÔÈΛÏÔÈ ·Ú¿ÁÔÓÙ˜: ¯˘ÌÈÎÔ›, ΢ÙÙ·ÚÈÎÔ›, ·˘ÍËÙÈÎÔ› Î·È Î˘ÙÙ·ÚÔΛÓ˜ (ÔÈ ÈÓÙÂÚÏ¢ΛÓ˜ -IL 1,3,6,11, Ô GMCSF, Ë ÂÚ˘ıÚÔÔÈËÙ›ÓË (EPO) Î·È Ë ıÚÔÌ‚ÔÔÈËÙ›ÓË (TPO), Ô˘ ÛÎÔfi ¤¯Ô˘Ó Ó· ÚÔ¿ÁÔ˘Ó ÙËÓ ÂͤÏÈÍË ·fi ÙÔ ¤Ó· ÛÙ¿‰ÈÔ ÛÙÔ ¿ÏÏÔ. ∂ÈϤÔÓ, ¤¯Ô˘Ó ÙËÓ ÈηÓfiÙËÙ· (.¯. Ë ∆ƒ√) Ó· ·˘Í‹ÛÔ˘Ó ÙÔÓ ·ÚÈıÌfi, ÙÔ Ì¤ÁÂıÔ˜, ÙËÓ ÏÔȉ›· Î·È ÙË Û˘ÓıÂÙÈ΋ ÈηÓfiÙËÙ· ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ. ∞ÓÙÈı¤Ùˆ˜, ÔΛϘ ÚˆÙ½Ó˜, fiˆ˜ Ô ·ÈÌÔÂÙ·ÏÈ·Îfi˜ ·Ú¿ÁÔÓÙ·˜ 4 (PF4), Ë ÈÓÙÂÚÊÂÚfiÓË (INF) ÎÏ, ·Ó·ÛÙ¤ÏÏÔ˘Ó ÙÔ Û¯ËÌ·ÙÈÛÌfi Î·È ÙËÓ ·Ó¿Ù˘ÍË ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ (1,7,8). ™Â οı ÂÚ›ÙˆÛË, ¿ÓÙˆ˜, Ô ÙÂÏÈÎfi˜ ·ÚÈıÌfi˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Ô˘ ΢ÎÏÔÊÔÚ› ·ÓÙ·Ó·ÎÏ¿ ¤Ó·Ó ηϿ Ú˘ıÌÈṲ̂ÓÔ Ì˯·ÓÈÛÌfi ÈÛÔÚÚÔ›·˜ ÌÂٷ͇ ·Ú·ÁˆÁ‹˜ Î·È Î·Ù·ÛÙÚÔÊ‹˜. ∏ ‰Â ·ÓÂ·Ú΋˜ ·Ú·ÁˆÁ‹ ·ÈÌÔÂÙ·Ï›ˆÓ, ÚÔ·ÙÂÈ ·fi ‰È·Ù·Ú·¯‹, Û ÔÔÈÔ‰‹ÔÙ ·fi Ù· ÛÙ¿‰È· Ù˘ ıÚÔÌ‚ÔÔ›ËÛ˘. £ÚÔÌ‚ÔÂÓ›· ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜ (2,5,8), Û˘¯ÓfiÙÂÚ· ÔÊ›ÏÂÙ·È Û ˘ÔÏ·Û›· ‹ ηٷ›ÂÛË ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ, ÏfiÁˆ ηٷÛÙÔÏ‹˜ ÙÔ˘ Ì˘ÂÏÔ‡ ·fi Ê¿Ú̷η Î·È ¿ÏÏÔ˘˜ ¯ËÌÈÎÔ‡˜/ Ê˘ÛÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ‹ ηٿÏË„‹˜ ÙÔ˘ ·fi ͤӷ ·ÙÙ·Ú·. ∏ ıÚÔÌ‚ÔÂÓ›· Ô˘ ÔÊ›ÏÂÙ·È Û ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ıÚÔÌ‚ÔÔ›ËÛË (·ÓÂ¿ÚÎÂÈ· ‚ÈÙ·Ì›Ó˘ µ12/Ê˘ÏÏÈÎÔ‡ ÔͤԘ, ·ÚÔ͢ÛÌÈ΋ Ó˘ÎÙÂÚÈÓ‹ ·ÈÌÔÛÊ·ÈÚÈÓÔ˘Ú›·), Û·Ó›ˆ˜ Â›Ó·È ÛÔ‚·Ú‹. ™Â ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË Ë ·Ú·ÁˆÁ‹ ·ÈÌÔÂÙ·Ï›ˆÓ ˘ÔÏ›ÂÙ·È, ›Ù ÏfiÁˆ ·ÓÂ·ÚÎÔ‡˜ Û‡ÓıÂÛ˘ DNA, ›Ù ÏfiÁˆ ˘ÂÚ¢·ÈÛıËÛ›·˜ Ù˘ ÌÂÌ‚Ú¿Ó˘ ÙÔ˘˜ Û ·ÓÙÈÛÒÌ·Ù· Î·È ÙÔ Û˘Ìϋڈ̷, ·Ú¿ ÙÔÓ ·˘ÍË̤ÓÔ ·ÚÈıÌfi ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ. √È ‰È·Ù·Ú·¯¤˜ ÙˆÓ Ú˘ıÌÈÛÙÈÎÒÓ Ì˯·ÓÈÛÌÒÓ Ù˘ ıÚÔÌ‚ÔÔ›ËÛ˘ (·ÓÂ¿ÚÎÂÈ· ıÚÔÌ‚ÔÔÈËÙ›Ó˘, ΢ÎÏÈ΋ ıÚÔÌ‚ÔÂÓ›·, ÂÚÈÔ‰È΋ ‰˘ÛÁÂÓÂÛ›· ·ÈÌÔÂÙ·Ï›ˆÓ) ›ӷÈ, Ô‡Ùˆ˜ ‹ ¿Ïψ˜, Û¿ÓȘ Î·È Î·Ù¿ ηÓfiÓ· ‰ÂÓ ÚÔηÏÔ‡Ó Ôχ ÛÔ‚·Ú¤˜ ıÚÔÌ‚ÔÂӛ˜. √ ·ıÔÊ˘ÛÈÔÏÔÁÈÎfi˜ Ì˯·ÓÈÛÌfi˜ Ô˘ ÂÌϤÎÂÙ·È ÛÙȘ ‰È¿ÊÔÚ˜ ÎÏËÚÔÓÔÌÈΤ˜ ıÚÔÌ‚ÔÂӛ˜ Â›Ó·È ÂÚÈÛÛfiÙÂÚÔ Û‡ÓıÂÙÔ˜ Î·È ÔÈΛÏÔ˜ (5). ∞Ó Î·È Ô ‰È·¯ˆÚÈÛÌfi˜ ÙˆÓ ıÚÔÌ‚ÔÂÓÈÒÓ ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜, ‚¿ÛÂÈ ÙÔ˘ ·ÈÙ›Ô˘ Ô˘ ÙȘ ÚÔηÏ›, Û ÚˆÙÔ·ı›˜ (ÎÏËÚÔÓÔÌÈΤ˜, Û˘ÁÁÂÓ›˜ ·ÌÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÈΤ˜, ΢ÎÏÈΤ˜, ÎÏ) Î·È ‰Â˘ÙÂÚÔ·ı›˜ (·Ϸۛ˜, Ì˘ÂÏÔ‰˘ÛϷۛ˜, Ì˘ÂÏfiÊıÈÛË, ÏÔÈÌÒÍÂȘ, ˘Ô‚ÈÙ·ÌÈÓÒÛÂȘ, ÎÏ) ÌÔÚ› Ó· Â›Ó·È ÎÏÈÓÈο ¯Ú‹ÛÈÌÔ˜, ÂÓÙÔ‡ÙÔȘ, ÔÈ fiÚÔÈ ÚˆÙÔ·ı›˜/ȉÈÔ·ı›˜ ‰ÂÓ ¤¯Ô˘Ó Ô˘ÛÈ·ÛÙÈÎfi ÓfiËÌ· ÛÙË Û‡Á¯ÚÔÓË È·ÙÚÈ΋. ¶ÚÔÎÂÈ̤ÓÔ˘ ÁÈ· Ù· ·È‰È¿, Ù· ÔÔ›· Î·È Ì¿˜ ÂӉȷʤÚÔ˘Ó, ı· ‹Ù·Ó ›Ûˆ˜ ÚÔÙÈÌfiÙÂÚÔ ÔÈ ıÚÔÌ‚ÔÂӛ˜ ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜ Ó· ‰È·¯ˆÚÈÛıÔ‡Ó ÎÏÈÓÈο ÛÂ

Paediatriki 2001;64:30-33

¶›Ó·Î·˜ 1: ∫ÏÈÓÈ΋ ηٿٷÍË ıÚÔÌ‚ÔÂÓÈÒÓ ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜ ™˘ÁÁÂÓ‹˜ ıÚÔÌ‚ÔÂÓ›· ·Ó·ÈÌ›· Fanconi Û‡Ó‰ÚÔÌÔ TAR ·ÓÂ¿ÚÎÂÈ· ıÚÔÌ‚ÔÔÈËÙ›Ó˘ ∫ÏËÚÔÓÔÌÈ΋ ıÚÔÌ‚ÔÂÓ›· Û‡Ó‰ÚÔÌÔ Wiskott-Aldrich ·ÓˆÌ·Ï›· May-Hegglin Û‡Ó‰ÚÔÌÔ Alport ªÂÛÔÁÂȷ΋ Ì·ÎÚÔıÚÔÌ‚ÔÂÓ›· ÔÈÎ›ÏˆÓ Ù‡ˆÓ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜ ¡ÂÔÁÓÈ΋ ıÚÔÌ‚ÔÂÓ›· ‰È‹ıËÛË Ì˘ÂÏÔ‡ ÈÔÁÂÓ‹˜ Ïԛ̈ÍË (ÂÚ˘ıÚ¿, CMV) Ï‹„Ë ıÂÈ·˙ȉÒÓ ·fi ÙË ÌËÙ¤Ú· ∂›ÎÙËÙË ıÚÔÌ‚ÔÂÓ›·

Û˘ÁÁÂÓ›˜, ÎÏËÚÔÓÔÌÈΤ˜, ÓÂÔÁÓÈΤ˜ Î·È Â›ÎÙËÙ˜ (¶›Ó·Î·˜ 1). ∏ Û˘ÁÁÂÓ‹˜ ·ÌÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÈ΋ ıÚÔÌ‚ÔÂÓ›· (5,8,9) Â›Ó·È ·ÌÊ›‚ÔÏÔ ·Ó ·ÔÙÂÏ› ÌÔÓ‹ÚË ‰È·Ù·Ú·¯‹ ‹ ·ÏÒ˜ Ì›· Ê¿ÛË ÛÙËÓ ÂͤÏÈÍË Ù˘ ·Ï·ÛÙÈ΋˜ ·Ó·ÈÌ›·˜. ™Â ÌÈÎÚfi ·ÚÈıÌfi ·ÛıÂÓÒÓ ÂÚÈÁÚ¿ÊÂÙ·È ıÚÔÌ‚ÔÂÓ›· ÔÈΛÏ˘ ‚·Ú‡ÙËÙ·˜ ÚÔ Ù˘ ËÏÈΛ·˜ ÙˆÓ 12-18 ÌËÓÒÓ Î·È ·Ó¿Ù˘ÍË ·Á΢ÙÙ·ÚÔÂÓ›·˜ ·ÚÁfiÙÂÚ·. ∞Ó Î·È ‰ÂÓ ¤¯Ô˘Ó ·Ó·ÁÓˆÚÈÛı› Â·ÎÚÈ‚Ò˜ ÔÈ ÁÔÓȉȷΤ˜ ‚Ï¿‚˜ (fiˆ˜ ¯ ÛÙËÓ ·Ó·ÈÌ›· Fanconi), ¿ÓÙˆ˜ ˘¿Ú¯ÂÈ Û·Ê¤˜ ÁÂÓÂÙÈÎfi ˘fiÛÙڈ̷. ∞ӷʤÚÂÙ·È ÌÂÁ¿ÏË ˘ÂÚÔ¯‹ ÙˆÓ ·ÁÔÚÈÒÓ (ÙÚÈÏ¿ÛÈ· Û˘¯ÓfiÙËÙ· ·fi Ù· ÎÔÚ›ÙÛÈ·). ªfiÓÔ Û ÛÔÚ·‰ÈΤ˜ ÂÚÈÙÒÛÂȘ Ê·›ÓÂÙ·È Ó· Û˘Ó˘¿Ú¯Ô˘Ó ¿ÏϘ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜. ª¤ÙÚËÛË ·ÈÌÔÂÙ·Ï›ˆÓ ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÛÙÔ Ì¤ÛÔÓ Ù˘ ΢‹Ûˆ˜ ÚÔÙ›ÓÂÙ·È ˆ˜ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË. ∏ ÚfiÁÓˆÛË Â›Ó·È Î·Î‹, ›ÙÂ Â›Ó·È ÌÔÓ‹Ú˘ Ë ‰È·Ù·Ú·¯‹ ›ÙÂ Û˘Ó‰˘·Ṳ̂ÓË. ∏ Û˘ÁÁÂÓ‹˜ ·Ô˘Û›· ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ Ô˘ Û˘Óԉ‡ÂÙ·È ·fi ¿ÏϘ ·ÓˆÌ·Ï›Â˜, ·ÔÙÂÏ› Û¿ÓÈÔ ·›ÙÈÔ ÛÔ‚·Ú‹˜, fï˜, ıÚÔÌ‚ÔÂÓ›·˜ Û ÓÂÔÁÓ¿ (5). ¶ÂÚÈÁÚ¿ÊÔÓÙ·È ÔÈΛϘ Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ (ÛÎÂÏÂÙÈΤ˜ ‹ Ô˘ÚÔÔÈËÙÈÎÔ‡ Î·È Î·Ú‰È·ÁÁÂÈ·ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜), Ë Û˘¯ÓfiÙÂÚ·, fï˜, ··ÓÙÒÌÂÓË ‰È·Ù·Ú·¯‹ (~90% ÙˆÓ ÂÚÈÙÒÛˆÓ) Â›Ó·È Ë ·ÌÊÔÙÂÚfiÏ¢ÚË ·Ô˘Û›· ÙˆÓ ÔÛÙÒÓ Ù˘ ÎÂÚΛ‰·˜ (ÂÍ Ô˘ Î·È Û‡Ó‰ÚÔÌÔ ∆∞R - thrombocytopenia with absent radii syndrome). ¶¿ÓÙÔÙÂ, fï˜, ˘¿Ú¯Ô˘Ó ÔÈ ·ÓÙ›¯ÂÈÚ˜, ÁÂÁÔÓfi˜ ¯Ú‹ÛÈÌÔ ÁÈ· ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ¿ÏÏˆÓ Û˘Ó‰ÚfiÌˆÓ (·Ó·ÈÌ›· Fanconi ‹ ÙÚÈۈ̛· 18). ¢Â‰Ô̤ÓÔ˘ fiÙÈ ÔÈ ÎÂÚΛ‰Â˜, Ë Î·Ú‰È¿ Î·È Ù· ÌÂÁ·Î·Ú˘Ô·ÙÙ·Ú· ·Ó·Ù‡ÛÛÔÓÙ·È fiÏ· ÌÂٷ͇ 6˘-8˘ ‚‰ÔÌ¿‰·˜ ΢‹Ûˆ˜, ÂÓ‰¤¯ÂÙ·È ÂÓ‰ÔÌ‹ÙÚÈ· ‚Ï¿‚Ë Û’ ·˘Ù‹ ÙËÓ ËÏÈΛ· Ó· ÚÔηÏ› ÙË ‰È·Ù·Ú·¯‹. ∞Ó ÎÈ ¤¯ÂÈ

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 32

¶·È‰È·ÙÚÈ΋ 2001;64:30-33

ÂÓÔ¯ÔÔÈËı› Ïԛ̈ÍË ·fi ÂÚ˘ıÚ¿, Ë ‚Ï¿‚Ë ıˆÚÂ›Ù·È Ì¿ÏÏÔÓ ÁÂÓÂÙÈ΋, ·Ú¿ Â›ÎÙËÙË. ¶Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ÌÂÙ·‚È‚¿˙ÂÙ·È Ì ˘ÔÏÂÈfiÌÂÓÔ ÛˆÌ·ÙÈÎfi ÁÔÓ›‰ÈÔ, ·Ó Î·È ·Ó·Ê¤ÚÂÙ·È ÂÏ·ÊÚ¿ ˘ÂÚÔ¯‹ ÙˆÓ ÎÔÚÈÙÛÈÒÓ. ¶¿ÓÙˆ˜, ˘ÂÚ˯ÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÂÚ› ÙË 16Ë-20Ë Â‚‰ÔÌ¿‰· ΢‹Ûˆ˜ ‚ÔËı¿ ÛÙËÓ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË. ∞·ÈÙ›ٷÈ, fï˜, ·ÂÈÎfiÓÈÛË Î·È ÙˆÓ ‰‡Ô ¿Óˆ ¿ÎÚˆÓ, ÁÈ·Ù› ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› Ï›Á˜ ÂÚÈÙÒÛÂȘ ÂÙÂÚfiÏ¢Ú˘ ·Ï·Û›·˜ ÎÂÚΛ‰Ô˜ (5,8). ∏ ÚfiÁÓˆÛË Â›Ó·È Î·Î‹. £¿Ó·ÙÔ˜ ÛÙË ‚ÚÂÊÈ΋ ËÏÈΛ·, ˆ˜ ·ÔÙ¤ÏÂÛÌ· ·ÈÌÔÚÚ·Á›·˜, Û˘Ì‚·›ÓÂÈ ÛÙÔ 25% ÙˆÓ ÂÚÈÙÒÛˆÓ. ŸÛÔÈ, fï˜, ÂÈ‚ÈÒÛÔ˘Ó, ·Ó‚¿˙Ô˘Ó ÙÔÓ ·ÚÈıÌfi ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ >100.000/mm3 Û˘Ó‹ıˆ˜, Â›‰· ¯Ú‹ÛÈÌ· ÁÈ· ÙȘ ÔÚıÔ‰ÈΤ˜ ÂÂÌ‚¿ÛÂȘ Ô˘ ı· ·ÎÔÏÔ˘ı‹ÛÔ˘Ó. ªÂÙ¿ÁÁÈÛË ∞ª¶ ·fi ‰fiÙË HLA Û˘Ì‚·Ùfi ÚÔÙ›ÓÂÙ·È ˆ˜ ıÂÚ·›· ÙˆÓ ·ÈÌÔÚÚ·ÁÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ, ÚÔÊ˘Ï·ÎÙÈ΋, fï˜, ¯ÔÚ‹ÁËÛË Û ·È‰È¿ οو ÙÔ˘ ¤ÙÔ˘˜ ‰ÂÓ Â›Ó·È ÎÔÈÓ¿ ·Ô‰ÂÎÙ‹. °È· ÙË ıÚÔÌ‚ÔÔÈËÙ›ÓË Ì·ı·›ÓÔ˘Ì ÔÏÏ¿ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, ·ÏÏ¿ ·ÁÓÔԇ̠ÔÏÏ¿ ÂÚÈÛÛfiÙÂÚ·. ∏ ˘fiıÂÛË ÁÈ· ÙËÓ ‡·ÚÍ‹ Ù˘ Î·È ÙÔ fiÓÔÌ· ‰fiıËΠ‹‰Ë ·fi ÙÔ 1958, ·ÏÏ¿ ÌfiÏȘ ÙÔ 1994 ÎψÓÔÔÈ‹ıËÎÂ. ∆Ô ÁÔÓ›‰ÈÔ Ô˘ ÙËÓ Îˆ‰ÈÎÔÔÈ› ‰ڿ˙ÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 1 (1p34). ∏ ∆ƒ√ Î·È Ô Î˘ÙÙ·ÚÈÎfi˜ ˘Ô‰Ô¯¤·˜ Ù˘, ÙÔ ÚˆÙÔÔÁÎÔÁÔÓ›‰ÈÔ c-mpl, ÙÔ˘ ÔÔ›Ô˘ ÙÔ ÁÔÓ›‰ÈÔ ¤¯ÂÈ Â›Û˘ ¯·ÚÙÔÁÚ·ÊËı› (3q26.3-q27), Ê·›ÓÂÙ·È fiÙÈ ·›˙Ô˘Ó ‚·ÛÈÎfi ÚfiÏÔ ÛÙË Ú‡ıÌÈÛË Ù˘ ·Ú·ÁˆÁ‹˜ ∞ª¶. ™Â Û˘Óı‹Î˜ ıÚÔÌ‚ÔÂÓ›·˜, ÁÂÓÈÎÒ˜, ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ ∆ƒ√ ÛÙÔ Ï¿ÛÌ· Â›Ó·È ·˘ÍË̤Ó˜, ÏfiÁˆ ÂÓÂÚÁÔÔ›ËÛ˘ Ì˯·ÓÈÛÌÔ‡ ·Ï›Ó‰ÚÔÌ˘ ·ÏÏËÏÔÚÚ‡ıÌÈÛ˘ (feed-back). ∂ÈϤÔÓ, Ê·›ÓÂÙ·È fiÙÈ Ù· ·ÈÌÔÂÙ¿ÏÈ· ·ÚÂÌ‚·›ÓÔ˘Ó ÛÙË ÛÙ¿ıÌË Ù˘ ∆ƒ√, ‰ÈfiÙÈ ¤¯Ô˘Ó ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ÙË ‰ÂÛÌÂ‡Ô˘Ó Ì ÙË ‚Ô‹ıÂÈ· ÂȉÈÎÔ‡ ˘Ô‰Ô¯¤·. ∂ÚÂ˘Ó¿Ù·È Î·Ù¿ fiÛÔ ÛÙȘ ÂÚÈÙÒÛÂȘ ·Ï·Û›·˜ ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ ˘¿Ú¯ÂÈ ·‰˘Ó·Ì›· ÚfiÛ‰ÂÛ˘ Ù˘ ∆ƒ√ ÛÙÔÓ ˘Ô‰Ô¯¤· Ù˘ (10). √È ÎÏËÚÔÓÔÌÈΤ˜ ıÚÔÌ‚ÔÂӛ˜ (5), ·ÔÙÂÏÔ‡Ó Ì›· ÔÌ¿‰· ıÚÔÌ‚ÔÂÓÈÒÓ ÛÙȘ Ôԛ˜ Ë ‚·ÛÈ΋ ‚Ï¿‚Ë Â›Ó·È ¿ÁÓˆÛÙË, Ë ÂÈÎfiÓ· ÙÔ˘ Ì˘ÂÏÔ‡ ¯ˆÚ›˜ ȉȷ›ÙÂÚ· ¯·Ú·ÎÙËÚÈÛÙÈο Î·È Ë ÁÓÒÛË Ì·˜ ÁÈ· ÙËÓ ÎÈÓËÙÈ΋ ÙˆÓ ∞ª¶ ·ÙÂÏ‹˜. √È ÂÚÈÛÛfiÙÂÚ˜ Û˘Óԉ‡ÔÓÙ·È ÂÈÚÔÛı¤Ùˆ˜, ·fi ÏÂÈÙÔ˘ÚÁÈΤ˜ ‰È·Ù·Ú·¯¤˜ ∞ª¶. ¢È·¯ˆÚ›˙ÔÓÙ·È Î·Ï‡ÙÂÚ· ‚¿ÛÂÈ ÙÔ˘ ÙÚfiÔ˘ ÌÂÙ·‚›‚·Û˘ Ù˘ ‰È·Ù·Ú·¯‹˜. ∆Ô Û‡Ó‰ÚÔÌÔ Wiskott-Aldrich Â›Ó·È ‰È·Ù·Ú·¯‹ Ô˘ ÌÂÙ·‚È‚¿˙ÂÙ·È ÌÂ Ê˘ÏÔÛ‡Ó‰ÂÙÔ ¯ÚˆÌfiۈ̷, Ô˘ ÛËÌ·›ÓÂÈ fiÙÈ Î·Ù¿ ηÓfiÓ· ¿Û¯Ô˘Ó ·ÁfiÚÈ·. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ¤Î˙ÂÌ·, ıÚÔÌ‚ÔÂÓ›· ÎÈ Â˘·ÈÛıËÛ›· ÛÙȘ ÏÔÈÌÒÍÂȘ, ÏfiÁˆ Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ Ù˘ ¯˘ÌÈ΋˜, ·ÏÏ¿ Î·È Î˘ÙÙ·ÚÈ΋˜, ·ÓÔÛ›·˜. √ ·fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ ÛÙÔ Ì˘ÂÏfi ›ӷÈ

32

Paediatriki 2001;64:30-33

Ê˘ÛÈÔÏÔÁÈÎfi˜ ‹/Î·È ·˘ÍË̤ÓÔ˜, ˘¿Ú¯ÂÈ, fï˜, ‰È·Ù·Ú·¯‹ ÛÙËÓ ˆÚ›Ì·ÓÛ‹ ÙÔ˘˜. ∞˘Ùfi Ô‰ËÁ› ÙfiÛÔ Û ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ·Ú·ÁˆÁ‹ ∞ª¶, fiÛÔ Î·È Û ‚Ú¿¯˘ÓÛË Ù˘ ˙ˆ‹˜ ÙÔ˘˜ (30% ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡), ÂӉ¯Ô̤ӈ˜ ÏfiÁˆ ÂÓ‰ÔÌ˘ÂÏÈ΋˜ ηٷÛÙÚÔÊ‹˜. £ÂÚ·›· Ì ÎÔÚÙÈÎÔÂȉ‹, ÂÓ‰ÔÊϤ‚È· ¯ÔÚ‹ÁËÛË ÁÛÊ·ÈÚ›Ó˘ ‹ ÛÏËÓÂÎÙÔÌ‹ ÂÏ¿¯ÈÛÙ· ·Ô‰›‰ÂÈ (5). √È ıÚÔÌ‚ÔÂӛ˜ Ô˘ ÌÂÙ·‚È‚¿˙ÔÓÙ·È Ì ÂÈÎÚ·ÙÔ‡ÓÙ· ۈ̷ÙÈÎfi ÁfiÓÔ ¤¯Ô˘Ó, ·Ú·‰fi͈˜, ‰È·ÊÔÚÂÙÈÎfi ̤ÁÂıÔ˜ ∞ª¶ ·fi ÙȘ ˘fiÏÔÈ˜ ıÚÔÌ‚ÔÂӛ˜ ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜ -Î˘Ì·›ÓÂÙ·È ·fi ·ÏÒ˜ ÌÂÁ·Ï‡ÙÂÚÔ ¤ˆ˜ ÙÔ˘Ï¿¯ÈÛÙÔÓ ‰ÈÏ¿ÛÈÔ. ∫·È ·˘Ù¤˜ ÔÈ ıÚÔÌ‚ÔÂӛ˜ ÂÓ‰¤¯ÂÙ·È Ó· Û˘Óԉ‡ÔÓÙ·È ·fi ÏÂÈÙÔ˘ÚÁÈΤ˜ ‰È·Ù·Ú·¯¤˜ ∞ª¶. ∏ ·ÓˆÌ·Ï›· May-Hegglin ·Ô‰›‰ÂÙ·È Û ‰È·Ù·Ú·¯‹ ηٿ ÙËÓ ÔÏÔÎÏ‹ÚˆÛË ÙÔ˘ ηٷÎÂÚÌ·ÙÈÛÌÔ‡ ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ Ô˘ ηٷϋÁÂÈ Û ۇÓÙËÍË/ Û˘Á¯ÒÓ¢ÛË ∞ª¶, Ù· ÔÔ›· ÂÚȤ¯Ô˘Ó ÙÂÚ¿ÛÙÈ· ÎÔÎΛ·. µ·ÛÂfiÊÈÏ· ÎÔÎΛ· (Dohle bodies) ·ÓȯÓ‡ÔÓÙ·È, Â›Û˘, ÛÙ· ÔÏ˘ÌÔÚÊÔ‡ÚËÓ·. ∏ ıÚÔÌ‚ÔÂÓ›· Â›Ó·È ÂÓ›ÔÙ ÛÔ‚·Ú‹. ™ÙÔ Û‡Ó‰ÚÔÌÔ Alport (ıÚÔÌ‚ÔÂÓ›·, ÎÒʈÛË, ÓÂÊÚ›ÙȘ), Ë Ì·ÎÚÔıÚÔÌ‚ÔÂÓ›· ·Ô‰›‰ÂÙ·È Û ·Ú·ÁˆÁ‹ ∞ª¶ Ï›ÁˆÓ Û ·ÚÈıÌfi, ÌÂÁ¿ÏˆÓ Û ̤ÁÂıÔ˜, ·ÏÏ¿ Ê˘ÛÈÔÏÔÁÈ΋˜ ‰ÔÌ‹˜, ·fi ÌÂÁ·Î·Ú˘Ô·ÙÙ·Ú· ÌË Ê˘ÛÈÔÏÔÁÈ΋˜ ‰ÔÌ‹˜. ™ÙË ªÂÛÔÁÂȷ΋ ıÚÔÌ‚ÔÂÓ›· ÂÚÈÁÚ¿ÊÂÙ·È ·Ú·ÁˆÁ‹ ÏÈÁfiÙÂÚˆÓ ∞ª¶ ·Ó¿ ÌÂÁ·Î·Ú˘Ô·ÙÙ·ÚÔ, ÏfiÁˆ ‚Ï¿‚˘ ÛÙË ‰È·›ÚÂÛË ÙÔ˘ ΢ÙÙ·ÚÔÏ¿ÛÌ·ÙÔ˜ ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ Î·Ù¿ ÙËÓ ·Ú·ÁˆÁ‹ ∞ª¶. ∂›Ó·È, ¿ÓÙˆ˜, ηÏÔ‹ı˘ ‰È·Ù·Ú·¯‹ Ô˘ ‰ÂÓ Û˘Óԉ‡ÂÙ·È ·fi ·ÈÌÔÚÚ·ÁÈ΋ ‰È¿ıÂÛË. ∆¤ÏÔ˜, Û ÂÏ¿¯ÈÛÙ˜ ÔÈÎÔÁ¤ÓÂȘ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ıÚÔÌ‚ÔÂӛ˜ Ô˘ ıˆÚÂ›Ù·È fiÙÈ ÎÏËÚÔÓÔÌÔ‡ÓÙ·È Ì ˘ÔÏÂÈfiÌÂÓÔ ÛˆÌ·ÙÈÎfi ÁÔÓ›‰ÈÔ. ∞ÔÙÂÏÔ‡Ó ÂÙÂÚÔÁÂÓ‹ ÔÌ¿‰· Ì ÔÈΛϷ ¯·Ú·ÎÙËÚÈÛÙÈο. ∆Ô Î˘ÚÈfiÙÂÚÔ Â›Ó·È Ë ÔÛÔÙÈ΋ ‹/Î·È ÔÈÔÙÈ΋ ·ÓÂ¿ÚÎÂÈ· ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ Ô˘ ·Ô‰›‰Ô˘Ó ∞ª¶ Ì Ôχ ‚Ú·¯‡ ¯ÚfiÓÔ ˙ˆ‹˜ ÛÙËÓ ÂÚÈʤÚÂÈ· ηÈ, Ê˘ÛÈο ÏÂÈÙÔ˘ÚÁÈο ·ÓÂ·Ú΋. ™Â ·˘Ù‹ ÙËÓ Î·ÙËÁÔÚ›· ˘¿ÁÂÙ·È Ë Bernard-Soulier, ÙÔ gray platelet syndrome, ÎÏ (5). ∏ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ı· ·ÔÙÂÏÔ‡Û ÙË ıÂÚ·›· ÂÎÏÔÁ‹˜ ÛÙÔ ¿ÌÂÛÔ Ì¤ÏÏÔÓ, ·Ó fi¯È ÛÙÔ ·ÚfiÓ, ÁÈ· ÔÏϤ˜ ·fi ÙȘ ıÚÔÌ‚ÔÂӛ˜ Ì ÌfiÓÈ̘ ‚Ï¿‚˜. ∏ ‰È‹ıËÛË ÙÔ˘ Ì˘ÂÏÔ‡ (ÈÛÙÈÔ΢ÙÙ¿ÚˆÛË, Û˘ÁÁÂÓ‹˜ Ï¢¯·ÈÌ›·) Â›Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈ· ·ÈÙ›· ıÚÔÌ‚ÔÂÓ›·˜ ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô (2,4,5). ∞ÓÙÈı¤Ùˆ˜, ÛÔ‚·Ú‹ ıÚÔÌ‚ÔÂÓ›· Â›Ó·È Û˘¯Ófi ‡ÚËÌ· Û ÓÂÔÁÓ¿ Ô˘ ¿Û¯Ô˘Ó ·fi Û˘ÁÁÂÓ›˜ ÏÔÈÌÒÍÂȘ, ΢ڛˆ˜ ÂÚ˘ıÚ¿ Î·È ÓfiÛÔ ·fi ΢ÙÙ·ÚÔÌÂÁ·ÏÔÈfi. ∏ ıÚÔÌ‚ÔÂÓ›· ·Ô‰›‰ÂÙ·È Û ηٷÛÙ·ÏÙÈ΋ ‰Ú¿ÛË


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 33

¶·È‰È·ÙÚÈ΋ 2001;64:30-33

¶›Ó·Î·˜ 2: ∞›ÙÈ· Â›ÎÙËÙ˘ ıÚÔÌ‚ÔÂÓ›·˜ ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜. ∞Ï·ÛÙ΋ ·Ó·ÈÌ›· ªÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÈ΋ ·Ï·Û›· ¢È‹ıËÛË Ì˘ÂÏÔ‡ ∞ÎÙÈÓÔ‚ÔÏ›· ª˘ÂÏÔηٷÛÙ·ÏÙÈο Ê¿Ú̷η ∞ÏÎÔfiÏ, ıÂÈ·˙›‰Â˜, ÈÓÙÂÚÊÂÚfiÓË, ÔÈÛÙÚÔÁfiÓ· ∫‡ËÛË ∫˘ÎÏÈ΋ ıÚÔÌ‚ÔÂÓ›· ∞ÓÂ¿ÚÎÂÈ· µ12/Ê˘ÏÏÈÎÔ‡ ÔͤԘ, Ûȉ‹ÚÔ˘ πÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ ¶·ÚÔ͢ÛÌÈ΋ Ó˘ÎÙÂÚÈÓ‹ ·ÈÌÔÛÊ·ÈÚÈÓÔ˘Ú›· ŒÎıÂÛË Û ˘ÂÚ‚·ÚÈÎfi Ô͢ÁfiÓÔ

ÙÔ˘ ÈÔ‡ ÛÙËÓ ·Ú·ÁˆÁ‹ ∞ª¶. ™Ù· ÓÂÔÁÓ¿ Ô˘ ÂÈ˙Ô‡Ó, Ë ıÚÔÌ‚ÔÂÓ›· ·Ôηı›ÛÙ·Ù·È ÔÏÏÔ‡˜ Ì‹Ó˜ ·ÚÁfiÙÂÚ·. ŒÁÎ˘Â˜, ÂÍ¿ÏÏÔ˘, Ô˘ Ï·Ì‚¿ÓÔ˘Ó ıÂÈ·˙›‰Â˜ ÂÓ‰¤¯ÂÙ·È Ó· ÁÂÓÓ‹ÛÔ˘Ó ÓÂÔÁÓ¿ Ì ‚·ÚÈ¿ ıÚÔÌ‚ÔÂÓ›· Ô˘ ÌÔÚ› Ó· ηٷϋÍÔ˘Ó ·fi ÂÁÎÂÊ·ÏÈ΋ ·ÈÌÔÚÚ·Á›·, ¯ˆÚ›˜ ÔÈ ›‰È˜ Ó· ¤¯Ô˘Ó ıÚÔÌ‚ÔÂÓ›·. ∏ ıÚÔÌ‚ÔÂÓ›· ·Ô‰›‰ÂÙ·È Û ÙÔÍÈ΋ ‰Ú¿ÛË ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ÛÙ· ÌÂÁ·Î·Ú˘Ô·ÙÙ·Ú· Î·È ·Ôηı›ÛÙ·Ù·È Ôχ ÁÚ‹ÁÔÚ· (~10 Ë̤Ú˜). ™ÙÔÓ ¶›Ó·Î· 2 ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Â›ÎÙËÙ˜ ηٷÛÙ¿ÛÂȘ Ô˘ Û˘Óԉ‡ÔÓÙ·È ·fi ıÚÔÌ‚ÔÂÓ›· ÎÂÓÙÚÈ΋˜ ·ÈÙÈÔÏÔÁ›·˜ (5). ∂Í ·˘ÙÒÓ, Ë ÂÈÏÂÎÙÈ΋ ·Ï·Û›· ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ Â›Ó·È ÂÍ·ÈÚÂÙÈÎÒ˜ Û¿ÓÈ·, ÂÓ‰¤¯ÂÙ·È, fï˜, Ó· ·ÔÙÂÏ› ÚÒÈÌË ÂΉ‹ÏˆÛË ÁÂÓÈÎÂ˘Ì¤Ó˘ ‰È·Ù·Ú·¯‹˜ ÙÔ˘ ·Ú¯¤ÁÔÓÔ˘ ·ÈÌÔÔÈËÙÈÎÔ‡ ΢ÙÙ¿ÚÔ˘ (stem cell), Ë ÔÔ›· ı· ÂΉËψı› ·ÚÁfiÙÂÚ·. ™Â ·˘Ù‹ ÙËÓ ÂÚ›ÙˆÛË, Ë ·ÔηٿÛÙ·ÛË Ù˘ ıÚÔÌ‚ÔÂÓ›·˜ Â›Ó·È Ôχ ‚Ú·‰‡ÙÂÚË ÙˆÓ ¿ÏÏˆÓ ÛÂÈÚÒÓ. ¶·ÚfiÙÈ Ô ÂÌÏÂÎfiÌÂÓÔ˜ Ì˯·ÓÈÛÌfi˜ ÁÈ· ÙËÓ Î·Ù·ÛÙÚÔÊ‹ ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ ‹ ÙËÓ Î¿ı·ÚÛË ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ ıˆÚÂ›Ù·È fiÙÈ Â›Ó·È ·ÓÔÛÔÏÔÁÈÎfi˜, ÂÓÙÔ‡ÙÔȘ, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ¯ÚˆÌÔÛˆÌȷΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ Ì˘ÂÏÔ‡. ∏ ÙÔÍÈ΋ ‰Ú¿ÛË Ù˘ ÔÏfiÛˆÌ˘ ·ÎÙÈÓÔ‚ÔÏ›·˜ ÛÙȘ Úfi‰ÚÔ̘ ÌÔÚʤ˜ ÙˆÓ ∞ª¶, ·ÏÏ¿ fi¯È ÛÙ· ΢ÎÏÔÊÔÚÔ‡ÓÙ· ∞ª¶, ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÏ·ÊÚ¿ ηı˘ÛÙ¤ÚËÛË ÛÙËÓ ÂÌÊ¿ÓÈÛË ıÚÔÌ‚ÔÂÓ›·˜. ∏ ΢ÎÏÈ΋, ·Ó¿ 30 Ë̤Ú˜ ÂÚ›Ô˘, ıÚÔÌ‚ÔÂÓ›·, ÔÈΛÏ˘ ‚·Ú‡ÙËÙ·˜, Û˘Ì‚·›ÓÂÈ ÛÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÂÊ‹‚Ô˘˜ Î·È Á˘Ó·›Î˜ ·Ó··Ú·ÁˆÁÈ΋˜ ËÏÈΛ·˜, ~2 ‚‰ÔÌ¿‰Â˜ ÚÔ Ù˘ ÂÚÈfi‰Ô˘ (·ÎÚÈ‚Ò˜ ÙÔ ·ÓÙ›ıÂÙÔ Ù˘ ‰È·Î‡Ì·ÓÛ˘ ÙˆÓ ∞ª¶, ÏfiÁˆ ÔÚÌÔÓÈÎÔ‡ ·ÎÏÔ˘). ™·Ó›ˆ˜ Û˘Óԉ‡ÂÙ·È ·fi ÔÚʇڷ. ∞Ô‰›‰ÂÙ·È Û ·ÔÚÚ‡ıÌÈÛË ÙˆÓ Ì˯·ÓÈÛÌÒÓ feed-back. √È ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ Â›Ó·È Û˘¯Ófi ·›ÙÈÔ ‹È·˜, Û˘Ó‹-

Paediatriki 2001;64:30-33

ıˆ˜, ıÚÔÌ‚ÔÂÓ›·˜ ÛÙ· ·È‰È¿, Ë ÔÔ›· ·Ôηı›ÛÙ·Ù·È Ì¤Û· Û 1-2 ‚‰ÔÌ¿‰Â˜. √È ÈÔ› ‚Ú›ÛÎÔ˘Ó ÛÙ· ÌÂÁ·Î·Ú˘Ô·ÙÙ·Ú· ȉ·ÓÈΤ˜ ı¤ÛÂȘ ÁÈ· ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙÔ˘˜, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ÚÔηÏÔ‡Ó Ì›ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙÔ˘˜ Î·È ·ÓÂ¿ÚÎÂÈ· Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙÔ˘˜ (2-5). µÈ‚ÏÈÔÁÚ·Ê›· 1. Bithell TC. Pathophysiology and classification of thrombocytopenia. In: Wintrobe’s Clinical Hematology. 9th ed. Philadelphia: Lea and Febiger; 1993. p. 1325-1328. 2. Beardsley DS. Platelet abnormalities in infancy and childhood. In: Nathan DJ, Oski FA, eds. Hematology of infancy and childhood. 5th ed. Philadelphia: WB Saunders; 1997. p. 1561-1588. 3. Bithell TC. Deficient platelet production. In: Wintrobe’s Clinical Hematology. 9th ed. Philadelphia, London: Lea and Febiger; 1993. p. 1363-1367. 4. McMillan CW. Decreased or ineffective production of platelets. In: Miller DR, editor. Blood diseases of infancy and childhood. 7th ed. St Louis, Toronto, Princeton: Mosby; 1996. p. 815-820. 5. George JN, Aster RH. Thrombocytopenia due to diminished or defective platelet production. In: Williams, Beutler, Erslev, Lichtman, eds. Hematology. 4th International ed. McGraw-Hill Book Company; 1991. p. 1343-1348. 6. Nesthammer AG, Forman EN. Use of platelet histogram maximum in evaluating thrombocytopenia. Am J Hematol 1999;60:19-23. 7. Gewirtz AM, Schick B. Megakaryocytopoiesis. In: Colman, Hirsh, Marder, Salzman, eds. Hemostasis and Thrombosis: Basic principles and clinical practice. 3rd ed. Philadelphia: Lippincott Company; 1994. p. 353-383. 8. Alter BP, Young NS. The bone marrow failure syndromes. In: Nathan, Oski, eds. Hematology of infancy and childhood. 5th ed. Philadelphia, London, Toronto, Montreal, Sydney, Tokyo: WB Saunders; 1993. p. 216-286. 9. Hardisty RM. Platelet disorders. In: Hoffbrand AV, Lewis SM, editors. Postgraduate Haematology. 3rd ed. London: Heineman Medical Books; 1989. p. 598-626. 10. Wolber EM, Dame C, Fahnenstich H, Hofmann D, Bartmann P, Jelkmann W et al. Expression of the thrombopoietin gene in human fetal and neonatal tissues. Blood 1999;94:97-105.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 02-10-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 14-12-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∂ϤÓË ¶Ï·ÙÔÎÔ‡ÎË-∫ÔÌÈÙÔÔ‡ÏÔ˘ ¶·‰›·ÙÚÔ˜, ∞Ó·ÏËÚÒÙÚÈ· ¢È¢ı‡ÓÙÚÈ· ∂™À ªÔÓ¿‰· ∞ÈÌÔÚÚ·ÁÈÎÒÓ ¢È·ı¤ÛÂˆÓ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ "∞Á›· ™ÔÊ›·" 115 27, ∞ı‹Ó·

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 34

¶·È‰È·ÙÚÈ΋ 2001;64:34-38

™À¡∂Ãπ∑√ª∂¡∏ ¶∞π¢π∞∆ƒπ∫∏ ∂∫¶∞π¢∂À™∏

Paediatriki 2001;64:34-38

CONTINUING MEDICAL EDUCATION

∞ÌÈÁ‹˜ ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ µ. ∫›ÙÚ·

Pure red cell aplasia of childhood V. Kitra

¶ÂÚ›ÏË„Ë: ∏ ·ÌÈÁ‹˜ ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ (PRCA) ·ÔÙÂÏ› ÂȉÈÎfi Û‡Ó‰ÚÔÌÔ ¯·Ú·ÎÙËÚÈ˙fiÌÂÓÔ ·fi ·Ó·ÈÌ›·, ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ·ÚÔÂÓ›· Î·È ÂÎÛÂÛËÌ·Ṳ̂ÓË ÂÏ¿ÙÙˆÛË Ì¤¯ÚÈ ÂÍ·Ê¿ÓÈÛË Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ ÛÙÔ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ˆÚ›Ì·ÓÛË Î·È ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ˘ÔÏÔ›ˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ ÛÂÈÚÒÓ, ‰ËÏ·‰‹ Ù˘ ÎÔÎÎÈÒ‰Ô˘˜ Î·È Ù˘ ÌÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÈ΋˜. ™ÙËÓ ·È‰È΋ ËÏÈΛ· ˘¿Ú¯Ô˘Ó 2 ÎÏÈÓÈο ‰È·ÊÔÚÂÙÈÎÔ› Ù‡ÔÈ, Ë Û˘ÁÁÂÓ‹˜ ·Ó·ÈÌ›· Diamond-Blackfan (DBA) Î·È Ë ·ÚÔ‰È΋ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›· (TEC). ∏ DBA Â›Ó·È ¤Ó· Û‡Ó‰ÚÔÌÔ Ì ÌÂÁ¿ÏË ÂÙÂÚÔÁ¤ÓÂÈ·, ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛˆÓ, ÛÔÚ·‰ÈÎÔ‡ ¯·Ú·ÎÙ‹Ú· ÂÓÒ ÌfiÓÔ ÙÔ 20% ıˆÚÂ›Ù·È ÎÏËÚÔÓÔÌÔ‡ÌÂÓÔ Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ‹ ÙÔÓ ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. ∏ ·ÚÔ‰È΋ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›· (TEC) Â›Ó·È Â›ÎÙËÙË ·Ó·ÈÌ›· ÂÌÊ·ÓÈ˙fiÌÂÓË ÛÙÔ 85% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û ÚÔËÁÔ‡ÌÂÓ· ˘ÁȤ˜ ·È‰› ËÏÈΛ·˜ 1-4 ÂÙÒÓ Î·È Û˘Ó‹ıˆ˜ ¤¯ÂÈ ÚÔËÁËı› Ì›· ÈÔÁÂÓ‹˜ Ïԛ̈ÍË. ∫˘Ú›ˆ˜, ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·˘ÙfiÌ·Ù˜ ˘Ê¤ÛÂȘ ÂÓÙfi˜ 4-8 ‚‰ÔÌ¿‰ˆÓ Î·È ·Ô˘Û›· ˘ÔÙÚÔÒÓ. ∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ PRCA ··ÈÙ› ÚÔÛÂÎÙÈ΋ ‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË ÁÈ· ÙËÓ ¤ÁηÈÚË Î·È ÛˆÛÙ‹ ‰È¿ÁÓˆÛË Î·È ÙË ‰È·ÊÔÚÔÔ›ËÛË ÌÂٷ͇ ÎÏËÚÔÓÔÌÈ΋˜ ‹ Â›ÎÙËÙ˘ ÓfiÛÔ˘, ηıÒ˜ Î·È ÌÂٷ͇ ÙÔ˘ ÚˆÙÔ·ıÔ‡˜ ‹ ‰Â˘ÙÂÚÔ·ıÔ‡˜ Ù‡Ô˘ ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘. ∏ ·Ú¯È΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ PRCA ··ÈÙ›, ۯ‰fiÓ ¿ÓÙÔÙÂ, ÌÂÙ·ÁÁ›ÛÂȘ ·›Ì·ÙÔ˜. ∞˘Ù‹ Ë ÚÔÛ¤ÁÁÈÛË ‰›ÓÂÈ ÙËÓ Â˘Î·ÈÚ›· Ó· ÔÏÔÎÏËÚˆı› Ô ¤ÏÂÁ¯Ô˜, ηıÒ˜ Î·È ÙË ‰˘Ó·ÙfiÙËÙ· ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙÔ˘ ·ÛıÂÓÔ‡˜ ÁÈ· ÙËÓ Èı·ÓfiÙËÙ· ·˘ÙfiÌ·Ù˘ ›·Û˘, Û ÂÚ›ÙˆÛË Ô˘ ¿Û¯ÂÈ ·fi ·ÚÔ‰È΋ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›·. ∏ ıÂÚ·¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Ù˘ DBA ÂÚÈÏ·Ì‚¿ÓÂÈ ÌÂÙ·ÁÁ›ÛÂȘ ·›Ì·ÙÔ˜, ¯ÔÚ‹ÁËÛË ·Ó‰ÚÔÁfiÓˆÓ ‹

Abstract: Pure red cell aplasia (PRCA) is a specific syndrome characterized by anaemia, reticulocytopenia, and strikingly reduced or absent erythroid precursor cells in the bone marrow with preservation of the lymphoid and other hematopoietic lineages. There are two main clinical forms of PRCA that are absolutely unique to pediatrics, the Diamond–Blackfan anaemia (DBA) and the transient erythroblastopenia of childhood (TEC), which are clearly distinguishable from each other. DBA is congenital and presents in early infancy; although it may be associated with autosomal dominant or recessive patterns of inheritance (20%), most reported cases are sporadic. TEC is an acquired self-limited form of PRCA, which occurs gradually in previously healthy children 1-4 years of age (85% of cases) following a viral infection. It is characterized by spontaneous recovery, usually within 4-8 weeks, without episodes of recurrences. Management of childhood PRCA begins with a thorough inve- stigation of the diagnostic possibilities that may underlie the syndrome being careful to distinguish between the congenital and acquired causes as well as the primary and secondary forms of the syndrome. Until a firm diagnosis is established, the initial treatment requires almost always blood transfusions. This allows flexibility to complete the investigations and to observe for a spontaneous remission if the anaemia is due to TEC or other selflimited condition. Several therapeutic approaches have been attempted for DBA, such as administration of androgens or cyclosporine; however the treatment of choice is the use of steroids. Bone marrow transplantation is a possibility for those

ªÔÓ¿‰· ªÂÙ·ÌfiÛ¯Â˘Û˘ ª˘ÂÏÔ‡ ÙˆÓ √ÛÙÒÓ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, ∞ı‹Ó·

Division of Bone Marrow Transplantation “Saint Sophia” Children’s Hospital, Athens

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¶·È‰È·ÙÚÈ΋ 2001;64:34-38

Paediatriki 2001;64:34-38

΢ÎÏÔÛÔÚ›Ó˘, ·ÏÏ¿ ıÂÚ·›· ÂÎÏÔÁ‹˜ ·ÔÙÂÏ› Ë ¯ÚËÛÈÌÔÔ›ËÛË ÙˆÓ ÎÔÚÙÈÎÔÂȉÒÓ. ™Â ·ÛıÂÓ›˜ ·ÓıÂÎÙÈÎÔ‡˜ ÛÙ· ÎÔÚÙÈÎÔÂȉ‹ ‹ Û ·˘ÙÔ‡˜ Ô˘ ÂÍ·ÚÙÒÓÙ·È ·fi ÌÂÁ¿Ï˜ ‰fiÛÂȘ ‹ ÌÂÙ·ÁÁ›ÛÂȘ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ, Ë ÌÂÙ·ÌfiÛ¯Â˘ÛË ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ, fiÙ·Ó ˘¿Ú¯ÂÈ Î·Ù¿ÏÏËÏÔ˜ ‰fiÙ˘, Â›Ó·È Ë ÂӉ‰ÂÈÁ̤ÓË ıÂÚ·›·.

patients who do not respond to reasonable doses of steroids.

§¤ÍÂȘ ÎÏÂȉȿ: ·ÌÈÁ‹˜ ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, ·È‰È΋ ËÏÈΛ·.

Key words: pure red cell aplasia, childhood.

∏ ·ÌÈÁ‹˜ ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ ·ÔÙÂÏ› ÂȉÈÎfi Û‡Ó‰ÚÔÌÔ Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·Ó·ÈÌ›·, ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ·ÚÔÂÓ›· Î·È ÂÎÛÂÛËÌ·Ṳ̂ÓË ÂÏ¿ÙÙˆÛË Ì¤¯ÚÈ Ï‹ÚÔ˘˜ ¤ÏÏÂȄ˘ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ ÛÙÔ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ˆÚ›Ì·ÓÛË Î·È ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ˘ÔÏÔ›ˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ ÛÂÈÚÒÓ, ‰ËÏ·‰‹ Ù˘ ÎÔÎÎÈÒ‰Ô˘˜ Î·È Ù˘ ÌÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÈ΋˜.

∏ ·ÈÙÈÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË (¶›Ó·Î·˜ 1), fiˆ˜ ¤¯ÂÈ ÚÔÙ·ı› ·fi ÙÔ˘˜ Alter Î·È Young, ‰Â›¯ÓÂÈ fiÙÈ Ë ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ÔÈÎ›ÏˆÓ ·Ú·ÁfiÓÙˆÓ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ Û ·ÓÙ›ıÂÛË Ì ٷ ·È‰È¿ (1). ™ÙËÓ ·È‰È΋ ËÏÈΛ· ˘¿Ú¯Ô˘Ó 2 Ù‡ÔÈ ·ÌÈÁÔ‡˜ ·Ï·Û›·˜ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, Ë Û˘ÁÁÂÓ‹˜ ·Ó·ÈÌ›· Diamond - Blackfan (DBA) Î·È Ë ·ÚÔ‰È΋ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›· (transient erythroblastopenia of childhood TEC).

¶›Ó·Î·˜ 1. ∞ÈÙÈÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË Ù˘ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›·˜.

™˘ÁÁÂÓ‹˜ ·Ó·ÈÌ›· Diamond – Blackfan ∏ DBA Â›Ó·È ¤Ó· Û‡Ó‰ÚÔÌÔ Ì ÌÂÁ¿ÏË ÂÙÂÚÔÁ¤ÓÂÈ· ˆ˜ ÚÔ˜ ÙËÓ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·, Ù· ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·, ÙË ıÂÚ·¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Î·È ÙËÓ ¤Î‚·ÛË. ∆Ô 80% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Â›Ó·È ÛÔÚ·‰ÈΤ˜ Î·È ÌfiÓÔ ÙÔ 20% ıˆÚÔ‡ÓÙ·È ÎÏËÚÔÓÔÌÔ‡ÌÂÓ˜ Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ‹ ÙÔÓ ˘ÔÏÂÈfiÌÂÓÔ ¯·Ú·ÎÙ‹Ú·. ¶Èı·ÓfiÓ Ó· ˘¿Ú¯ÂÈ Î·È Ê˘ÏÔÛ‡Ó‰ÂÙË (x-linked) ÎÏËÚÔÓÔÌÈÎfiÙËÙ·, ÁÂÁÔÓfi˜ Ô˘ ÂÍËÁ› ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ÓfiÛÔ˘ ÛÙ· ¿ÚÚÂÓ· ·È‰È¿ Û ÔÚÈṲ̂Ó˜ ÔÈÎÔÁ¤ÓÂȘ. √È ÂÚÈÛÛfiÙÂÚ˜ ΢‹ÛÂȘ Ì ÚÔۂ‚ÏË̤ӷ ¤Ì‚Ú˘· ‰ÂÓ Â›Ó·È ÙÂÏÂÈfiÌËÓ˜ Î·È ÙÔ 5% ÙˆÓ ÌËÙ¤ÚˆÓ ·Ó·Ê¤ÚÔ˘Ó ÚÔËÁÔ‡ÌÂÓ˜ ·Ô‚ÔϤ˜. ¶ÚÔ‚Ï‹Ì·Ù· ÂÌÊ·ÓÈ˙fiÌÂÓ· ηٿ ÙËÓ Î‡ËÛË Â›Ó·È Ë ÚÔÂÎÏ·Ì„›·, Ë ÙÔÍÈÓ·ÈÌ›·, Ë ÚÔˆÚfiÙËÙ· Î·È Ô ÚÔ‰ÚÔÌÈÎfi˜ Ï·ÎÔ‡ÓÙ·˜. ∏ ¤ÎıÂÛË, ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔÛ‡Ó˘, Û ÙÔÍÈΤ˜ Ô˘Û›Â˜ fiˆ˜ ¯ÏˆÚÔıÂÈ·˙›‰Ë, ¯ÏˆÚ·ÌÊÂÓÈÎfiÏË, ÚÂ˙ÂÚ›ÓË, ı˘ÚÂÔÂȉÈΤ˜ ÔÚÌfiÓ˜ Î·È ÎÔÚÙÈ˙fiÓË Úԉȷı¤ÙÂÈ ÛÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ÓfiÛÔ˘. ™˘Ó‹ıˆ˜ Ù· ÚÔۂ‚ÏË̤ӷ ·È‰È¿ ÁÂÓÓÈÔ‡ÓÙ·È ÏÈÔ‚·Ú‹ Î·È ÏÈÁfiÙÂÚÔ Û˘¯Ó¿ Ì ÂËÚ·Ṳ̂ÓÔ ÙÔ Ì‹ÎÔ˜ ÙÔ˘˜. ∂ÎÙfi˜ ÙˆÓ ·ÈÌ·ÙÔÏÔÁÈÎÒÓ ÚÔ‚ÏËÌ¿ÙˆÓ ‰ÂÓ ·Ó·Ê¤ÚÔÓÙ·È ¿ÏϘ ‰È·Ù·Ú·¯¤˜, ·Ú¿ ÌfiÓÔ Û¿ÓȘ ÂÚÈÙÒÛÂȘ Ì ¢ÂÚÂıÈÛÙfiÙËÙ·, Ï‹ı·ÚÁÔ ‹ ηډȷ΋ ·ÓÂ¿ÚÎÂÈ·. ∆· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÂȂ‚·›ˆÛ˘ Ù˘ ÓfiÛÔ˘ ·ÔÙÂÏÔ‡Ó: ·) ÔÚıÔ-Ì·ÎÚÔ΢ÙÙ·ÚÈ΋ ·Ó·ÈÌ›· ÂÌÊ·ÓÈ˙fiÌÂÓË ÛÙÔ 90% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÛÙÔ˘˜ ‰‡Ô ÚÒÙÔ˘˜ Ì‹Ó˜ Ù˘ ˙ˆ‹˜, ‚) ÂÎÛÂÛËÌ·Ṳ̂ÓË ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ΢ÙÙ·ÚÔÂÓ›·, Á) Ê˘ÛÈÔÏÔÁÈ΋ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ· ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ

™˘ÁÁÂÓ‹˜ Diamond–Blackfan ·Ó·ÈÌ›· (DBA) ∂›ÎÙËÙË

¶ÚˆÙÔ·ı‹˜

¶·ÚÔ‰È΋ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›· ÀÔÙÚÔÈ¿˙Ô˘Û· ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ∞˘ÙÔ¿ÓÔÛË π‰ÈÔ·ı‹˜

¢Â˘ÙÂÚÔ·ı‹˜

§ÔÈÌ҉˘

πÔÁÂÓ‹˜ (Parvovirus B19, EBV, HBV) ªÈÎÚԂȷ΋ (ÛÙ·Ê˘ÏfiÎÔÎÎÔ˜, ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˜, Ì˘ÎfiÏ·ÛÌ·)

∞˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù· (™∂§, ƒ∞) §Â˘¯·È̛˜, Û˘Ì·Á›˜ fiÁÎÔÈ (£‡ÌˆÌ·, ÓÂÔϷۛ˜ ÏÂÌÊÈ΋˜ ÛÂÈÚ¿˜, ηÚÎÈÓÒÌ·Ù·) º¿Ú̷η – ∆Ô͛Ә

¢È¿ÊÔÚ·

∫‡ËÛË ∞µ√ ·Û‡Ì‚·ÙË ªª√ ¡ÂÊÚÈ΋ ·ÓÂ¿ÚÎÂÈ·

35


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 36

¶·È‰È·ÙÚÈ΋ 2001;64:34-38

ÔÛÙÒÓ Ì ÂÓÙ˘ˆÛȷ΋ ·ÒıËÛË Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, ‰) ·˘ÍË̤ӷ Â›‰· ÂÚ˘ıÚÔÔÈËÙ›Ó˘ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ, ·˘ÍË̤ÓÔ ·ÓÙÈÁfiÓÔ i Î·È ·ÈÌÔÛÊ·ÈÚ›ÓË F, Ô˘ ˘Ô‰ËÏÒÓÔ˘Ó ÂÌ‚Ú˘˚ÎÔ‡ Ù‡Ô˘ ·ÈÌÔÔ›ËÛË, Â) ·˘ÍË̤ÓË ··ÌÈÓ¿ÛË Ù˘ ·‰ÂÓÔÛ›Ó˘ (ADA) ÙˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ, ÛÙ) Ê˘ÛÈÔÏÔÁÈΤ˜ (‹ ÂÏ·ÊÚÒ˜ ÂÏ·Ùو̤Ó˜) ÙÈ̤˜ ÙˆÓ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ Î·È ˙) Ê˘ÛÈÔÏÔÁÈο ‹ ·˘ÍË̤ӷ ·ÈÌÔÂÙ¿ÏÈ·. ™ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ·ÚÎÂÙ¿ ·fi ·˘Ù¿ Ù· ·È‰È¿ Î·È Î˘Ú›ˆ˜ Ù· ·ÁfiÚÈ· ÂÌÊ·Ó›˙Ô˘Ó ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ (¶›Ó·Î·˜ 2), fiˆ˜ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÚÔÛˆÂ›Ô (Cathie) Ì ηı›˙ËÛË Ù˘ Ú›˙·˜ Ù˘ ÚÈÓfi˜, ˘ÂÚÙÂÏÔÚÈÛÌfi, ·¯‡ ¿Óˆ ¯Â›ÏÔ˜ ÎÈ ¤Í˘ÓË ¤ÎÊÚ·ÛË. ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ Ì DBA ÂÍ ÔÚÈÛÌÔ‡ ¤¯Ô˘Ó ·Ó·ÈÌ›· Ì ÙÈ̤˜ Hb ηٿ ÙË Á¤ÓÓËÛË 2,6-14,8 gr/dl (̤ÛË ÙÈÌ‹ 7), Ì·ÎÚÔ΢ÙÙ¿ÚˆÛË ÛÙË ÌÔÚÊÔÏÔÁ›· ÙˆÓ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ, ÂÏ·Ùو̤Ó˜ ¤ˆ˜ ÌˉÂÓÈΤ˜ ÙÈ̤˜ ÙˆÓ ¢∂∫, ÂÌ‚Ú˘˚ÎÔ‡ Ù‡Ô˘ ·ÈÌÔÔ›ËÛË Ì ·˘ÍË̤Ó˜ ÙÈ̤˜ HbF, Ag i, ADA Î·È Ì˘ÂÏfiÁÚ·ÌÌ· Ì ÂÎÏÂÎÙÈ΋ ·Ô˘Û›· ÙˆÓ ÂÚ˘ıÚÔ‚Ï·ÛÙÒÓ. ∆· Â˘Ú‹Ì·Ù· Ù˘ Ì˘ÂÏÈ΋˜ ‚ÈÔ„›·˜ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ˘ÔÏ·Û›· ̤¯ÚÈ Ï‹ÚË ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ ÛÙÔ 90% ÙˆÓ ÂÚÈÙÒÛˆÓ, ˘ÂÚÏ·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ Ì ·Ó·ÛÙÔÏ‹ Ù˘ ˆÚ›Ì·ÓÛ˘ Î·È ·‡ÍËÛË ÙˆÓ ·ÒÚˆÓ ÂÚ˘ıÚÔ‚Ï·ÛÙÒÓ ÛÙÔ 5%, ÂÓÒ ÛÙÔ ˘fiÏÔÈÔ 5% ·Ú·ÙËÚÂ›Ù·È Ê˘ÛÈÔÏÔÁÈÎfi˜ ·ÚÈıÌfi˜ Î·È ˆÚ›Ì·ÓÛË ÙˆÓ ÂÚ˘ıÚÔ‚Ï·ÛÙÒÓ. ∏ ‰È·Ù·Ú·¯‹ Ù˘ ÂÚ˘ıÚÔÔ›ËÛ˘ ÛÙË DBA ¤¯ÂÈ ÌÂÏÂÙËı› ÂÎÙÂÓÒ˜ Ì ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ Î·ÏÏÈÂÚÁÂÈÒÓ, ÙË ¯Ú‹ÛË ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Î·È ÙˆÓ ÌÔÚÈ·ÎÒÓ Ù¯ÓÈÎÒÓ Î·È ¤¯Ô˘Ó ηٷÓÔËı› ·ÚÎÂÙ¿ Ô˘ ·ÊÔÚÔ‡Ó ÙËÓ Î˘ÙÙ·ÚÈ΋ ‚¿ÛË Û Ï›ÛÙ˜ ÂÚÈÙÒÛÂȘ Ì DBA. ŸÌˆ˜, ·Ú·Ì¤ÓÂÈ ÙÔ ÂÚÒÙËÌ· ·Ó Ë ‚Ï¿‚Ë ·ÊÔÚ¿ ÙÔ ÂÚ˘ıÚfi ·ÈÌÔÛÊ·›ÚÈÔ ‹ ÙÔ ÌÈÎÚÔÂÚÈ‚¿ÏÏÔÓ ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ‹ ·Ó ÂÚÈÔÚ›˙ÂÙ·È Û ·Ó·ÛÙÔÏ›˜ Ù˘ ¯˘ÌÈ΋˜ ·ÓÔÛ›·˜. ∏ ·ÓÔÛÔÏÔÁÈÎÔ‡ Ù‡Ô˘ ηٷÛÙÔÏ‹ Ù˘ ÂÚ˘ıÚÔÔ›ËÛ˘ ˘ÔÛÙËÚ›˙ÂÙ·È ·fi ·ÚÎÂÙÔ‡˜ ÂÚ¢ÓËÙ¤˜. ¶ÔÏÏÔ›, fï˜, ·Ô‰›‰Ô˘Ó ÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ DBA Û ‰È·Ù·Ú·¯¤˜ ÙˆÓ ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ΢ÙÙ·ÚÈΤ˜ ηÏÏȤÚÁÂȘ ·˘ÙÒÓ (CFU-E, BFU-E). ∏ ·Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Û ηÏÏȤÚÁÂȘ Û ·ÛıÂÓ›˜ Ì DBA ‹Ù·Ó ÂÏ¿¯ÈÛÙË Ì¤¯ÚÈ ÌˉÂÓÈ΋ ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ. ¶ÚfiÛÊ·Ù·, Ë ÂÈÎÚ·ÙÔ‡Û· ¿Ô„Ë ÁÈ· ÙËÓ ·ıÔÁ¤ÓÂÈ· Ù˘ DBA Û¯ÂÙ›˙ÂÙ·È Ì ‰È·Ù·Ú·¯¤˜ ·ÓÙ›‰Ú·Û˘ ÙˆÓ ÂÚ˘ıÚÔ‚Ï·ÛÙÒÓ ÛÙÔ˘˜ Â·ÁˆÁ›˜ ÙÔ˘ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡ Î·È Ù˘ ‰È·ÊÔÚÔÔ›ËÛ‹˜ ÙÔ˘˜. ∏ ‚Ï¿‚Ë ÂÓÙÔ›˙ÂÙ·È Û ·‰˘Ó·Ì›· ÌÂÙ·‚›‚·Û˘ ÙÔ˘ Û‹Ì·ÙÔ˜ ÙˆÓ Â·ÁˆÁ¤ˆÓ Ù˘ ÂÚ˘ıÚÔÔ›ËÛ˘ {∂Ú˘ıÚÔÔÈËÙ›ÓË (Epo), πÓÙÂÚÏ¢ΛÓË 3 (IL-3), ∞˘ÍËÙÈÎfi˜ ·Ú¿-

36

Paediatriki 2001;64:34-38

¶›Ó·Î·˜ 2. ∂ȉÈΤ˜ ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÛÙËÓ D.B. ∞Ó·ÈÌ›·. ∫ÂÊ·Ï‹ (Cathie Facies) ª¿ÙÈ· (˘ÂÚÙÂÏÔÚÈÛÌfi˜, ÁÏ·‡ÎˆÌ·, ÌÏ ÛÎÏËÚÔ›) ∞˘¯¤Ó·˜ (ÎÔÓÙfi˜ Ï·ÈÌfi˜, ·Ó‡„ˆÛË ˆÌÔÏ¿Ù˘) ÕÓˆ ¿ÎÚ· (·ÓˆÌ·Ï›Â˜ ΢ڛˆ˜ ÙÔ˘ ·ÓÙ›¯ÂÈÚ·, ‚Ú·¯›ÔÓ·) ª‡ÙË (Ï·ÙÈ¿, Â›‰Ë, ηı›˙ËÛË ÚÈ˙ÔÚÚÈÓ›Ô˘) ÷ÌËÏfi ·Ó¿ÛÙËÌ· ¡ÔËÙÈ΋ ˘ÛÙ¤ÚËÛË ¡ÂÊÚÔ› (¢ÈÏ·ÛÈ·ÛÌfi˜ Ô˘ÚËÙ‹ÚˆÓ, ÂÙ·ÏÔÂȉ‹˜ ÓÂÊÚfi˜, Ô˘ÚÔÏÔÈÌÒÍÂȘ) °ÂÓÓËÙÈο fiÚÁ·Ó· (ÀÔÏ·Û›·, ‚Ô˘‚ˆÓÔ΋ÏË, ˘ÔÛ·‰›·˜, ˘‰ÚÔ΋ÏË) ∫·Ú‰È¿ (™Ù¤ÓˆÛË ·ÔÚÙ‹˜, ÛÙ¤ÓˆÛË Ó¢ÌÔÓÈ΋˜ ÎÏ) ™ÎÂÏÂÙfi˜ (™ÎÔÏ›ˆÛË, ·¯ÔÓ‰ÚÔÏ·Û›·, ÎÔÓÙ¿ ¿ÎÚ·)

ÁÔÓÙ·˜ ÙÔ˘ ·Ú¯¤ÁÔÓÔ˘ ·ÈÌÔÔÈËÙÈÎÔ‡ ΢ÙÙ¿ÚÔ˘ (Stem Cell Factor, SCF)} ·fi ÙÔ˘˜ ˘ԉԯ›˜ (receptor-ligand) ÙˆÓ ÂÚ˘ıÚÔ‚Ï·ÛÙÒÓ. ∆Ô ·ÓˆÙ¤Úˆ ÂÓÈÛ¯‡ÂÙ·È ·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ù· ÏÂÌÊÔ·ÙÙ·Ú· ·˘ÙÒÓ ÙˆÓ ·ÛıÂÓÒÓ ·Ú¿ÁÔ˘Ó IL-3, ÙÔ ÛÙÚÒÌ· ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ÂÎÊÚ¿˙ÂÈ ÙÔÓ (SCF) Î·È ˘¿Ú¯Ô˘Ó ÌÂÙÚËÙ¿ Â›‰· ÙÔ˘ SCF ÛÙÔÓ ÔÚfi ηıÒ˜, Â›Û˘, ˘ԉԯ›˜ ·˘Ùo‡ ÛÙ· Ì˘ÂÏÈο ·ÙÙ·Ú·. ∂ÈϤÔÓ, ‰ÂÓ ¤¯Ô˘Ó ‚ÚÂı› ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙ· ÁÔÓ›‰È· ÙÔ˘ ˘Ô‰Ô¯¤· SCF. ¶·Ú’fiÏÔ Ô˘ Ë ·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ DBA ‰ÂÓ ¤¯ÂÈ Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈÛÙ›, ÏËÚÔÊÔڛ˜ ·fi ÙȘ in vitro ÌÂϤÙ˜ ¤¯Ô˘Ó ÂÊ·ÚÌÔÛı› ÂÈÙ˘¯Ò˜ ÛÙËÓ ÎÏÈÓÈ΋ Ú·ÎÙÈ΋ Î·È ÛÙË ıÂÚ·¢ÙÈ΋ ÛÙÚ·ÙËÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÓfiÛÔ˘. ∏ ıÂÚ·›· ·ÓÙÈÌÂÙÒÈÛ˘ ÂÚÈÏ·Ì‚¿ÓÂÈ: ·) ªÂÙ·ÁÁ›ÛÂȘ Û˘Ì˘ÎÓˆÌ¤ÓˆÓ ÂÚ˘ıÚÒÓ Î·È ‰È·Ù‹ÚËÛË Ù˘ ·ÈÌÔÛÊ·ÈÚ›Ó˘ ¿Óˆ ·fi 8 gr/dl ηٿ ÙË ‰È¿ÁÓˆÛË. ∏ ÛÔ‚·ÚfiÙÂÚË ÂÈÏÔ΋ ÙˆÓ ÌÂÙ·ÁÁ›ÛˆÓ, Ë ·ÈÌÔÛȉ‹ÚˆÛË, ··ÈÙ› Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Ù·ÎÙÈ΋ ·ÔÛȉ‹ÚˆÛË Ì ‰ÂÛÊÂÚÈÔÍ·Ì›ÓË. ∏ ÛÏËÓÂÎÙÔÌ‹ ‰ÂÓ Û˘ÓÈÛٿٷÈ. ‚) ∆· ÎÔÚÙÈÎÔÂȉ‹, Ú‰ÓÈ˙fiÓË ‹ Ú‰ÓÈ˙ÔÏfiÓË, ·ÔÙÂÏÔ‡Ó Ê¿Ú̷η ÚÒÙ˘ ÂÎÏÔÁ‹˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ DBA. ∏ ‰fiÛË ¤Ó·Ú͢ Â›Ó·È 2mg/kg/∏ Û 3 ‹ 4 ‰fiÛÂȘ. ∆· ¢∂∫ Û˘Ó‹ıˆ˜ ·˘Í¿ÓÔÓÙ·È Û 1-2 ‚‰ÔÌ¿‰Â˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Î·È Ë Hb ÌÂÙ¿ ÙÔÓ 1Ô3Ô Ì‹Ó·. ∏ ˘„ËÏ‹ ‰fiÛË ÎÔÚÙÈ˙fiÓ˘ Û˘Ó¯›˙ÂÙ·È Ì¤¯ÚÈ Ë Hb Ó· ‰È·ÙËÚËı› Û Â›‰· 10gr/dl Î·È Ë ‰È·ÎÔ‹ Ù˘ Á›ÓÂÙ·È ·ÚÁ¿ Î·È ÛÙ·ıÂÚ¿ Ì ÚÔÔÙÈ΋ ÙË ¯ÔÚ‹ÁËÛË ÌÈÎÚÒÓ ‰fiÛÂˆÓ Û ·Ú‹ÌÂÚÔ Û¯‹Ì·, ‰È·ÙËÚÒÓÙ·˜ ÛÙ·ıÂÚ‹ ÙËÓ Hb. ∞˘ÙÔ› ÔÈ ·ÛıÂÓ›˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÂÓÙ˘ˆÛȷ΋ ¢·ÈÛıËÛ›· Û ÌÈÎÚ¤˜ ÙÚÔÔÔÈ‹ÛÂȘ Ù˘ ‰fiÛ˘ ÙˆÓ ÎÔÚÙÈÎÔÂȉÒÓ. ∞Ó¿ÏÔÁ· Ì ÙËÓ ·¿ÓÙËÛË ÛÙ· ÎÔÚÙÈÎÔÂȉ‹


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 37

¶·È‰È·ÙÚÈ΋ 2001;64:34-38

‰È·ÎÚ›ÓÔÓÙ·È ÔÈ ·ÎfiÏÔ˘ı˜ ηÙËÁÔڛ˜ ·ÛıÂÓÒÓ: 1) ÁÚ‹ÁÔÚÔÈ ··ÓÙËÙ¤˜ Ì ÎÔÚÙÈ˙ÔÓÔÂÍ·ÚÙÒÌÂÓË ‡ÊÂÛË (Û˘Ì‚·›ÓÂÈ ÛÙÔ 5% ÙˆÓ ·ÛıÂÓÒÓ) 2) ÂӉȿÌÂÛÔÈ ··ÓÙËÙ¤˜ <5% 3) ÙÔ 60% ÙˆÓ ·ÛıÂÓÒÓ ··ÓÙÔ‡Ó Î·Ï¿. ∞fi ·˘ÙÔ‡˜, ÙÔ 40% Á›ÓÔÓÙ·È ÎÔÚÙÈ˙ÔÓÔÂÍ·ÚÙÒÌÂÓÔÈ Î·È ÙÔ ˘fiÏÔÈÔ 20% ‰È·ÙËÚÔ‡Ó Î·Ï¿ Â›‰· ·ÈÌÔÛÊ·ÈÚ›Ó˘ ·ÂÍ·ÚÙÒÌÂÓÔÈ Ù˘ ÎÔÚÙÈ˙fiÓ˘ 4) ·Ú¯È΋ ·¿ÓÙËÛË ÛÙËÓ ÎÔÚÙÈ˙fiÓË ·ÎÔÏÔ˘ıÔ‡ÌÂÓË ·fi ÂÍ¿ÚÙËÛË Î·È ÌÂÙ¿ ˘ÔÙÚÔ‹ Î·È ·ÔÙ˘¯›· ·¿ÓÙËÛ˘ ÛÙËÓ ›‰È· ‹ ÌÂÁ·Ï‡ÙÂÚË ‰fiÛË ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 5% 5) ÔÛÔÛÙfi οو ÙÔ˘ 5% ·ÚÔ˘ÛÈ¿˙ÂÈ ÂÍ¿ÚÙËÛË ·fi Ôχ ˘„ËϤ˜ ‰fiÛÂȘ ÎÔÚÙÈÎÔÂȉÒÓ Î·È ¤¯Ô˘Ó ·Ó¿ÁÎË Û˘¯ÓÒÓ ÌÂÙ·ÁÁ›ÛÂˆÓ 6) ·ÚÎÂÙÔ› ·ÛıÂÓ›˜ (30-40%) ‰ÂÓ ··ÓÙÔ‡Ó Î·ıfiÏÔ˘ ÛÙ· ÎÔÚÙÈÎÔÂȉ‹. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ¯ÔÚ‹ÁËÛ˘ ÙˆÓ ÎÔÚÙÈÎÔÂȉÒÓ ı· Ú¤ÂÈ Ó· ·ÔʇÁÔÓÙ·È Î·Ù·ÛÙ·ÏÙÈο Ê¿Ú̷η ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ fiˆ˜ ÔÈÛÙÚÔÁfiÓ·, Ê·ÈÓÔ‚·Ú‚ÈÙ¿ÏË, Ê·ÈÓ˘ÙÔ˝ÓË. ∞˘ÙfiÌ·Ù˜ ˘Ê¤ÛÂȘ, ΢ڛˆ˜ Û˘Ì‚·›ÓÔ˘Ó ÛÙËÓ ÂÊ˂›· Î·È ¤¯Ô˘Ó ·Ú·ÙËÚËı› ÛÙÔ 20% ·˘ÙÒÓ ÙˆÓ ·ÛıÂÓÒÓ. Á) ∏ ¯ÔÚ‹ÁËÛË ·Ó‰ÚÔÁfiÓˆÓ, 6-ÌÂÚηÙÔÔ˘Ú›Ó˘, ·ÓÙÈÏÂÌÊÔ΢ÙÙ·ÚÈÎÔ‡ ÔÚÔ‡ Î·È Î˘ÎÏÔÛÔÚ›Ó˘ ∞, ‰ÂÓ Ê·›ÓÂÙ·È Ó· ¤¯Ô˘Ó ı¤ÛË ÛÙË ıÂÚ·›· Ù˘ DBA. ‰) ∏ ÚÔÛı‹ÎË ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, fiˆ˜ Epo Î·È IL-3, Û˘Ó¤‚·Ï ÌfiÓÔ Û ·ÚÔ‰ÈΤ˜ ˘Ê¤ÛÂȘ. √ SCF ÏfiÁˆ ÙˆÓ ÂÓı·ÚÚ˘ÓÙÈÎÒÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ in vitro, ÙÂÏÂ˘Ù·›· ‰ÔÎÈÌ¿˙ÂÙ·È Û ÂÚ¢ÓËÙÈο ÚˆÙfiÎÔÏÏ·. ™ÙË ¯ÔÚ‹ÁËÛ‹ ÙÔ˘ ··ÈÙÂ›Ù·È ÚÔÛÔ¯‹, ·Ó ÏËÊı› ˘fi„Ë Ë ÚԉȿıÂÛË ÙˆÓ ·ÛıÂÓÒÓ Ì DBA ۠Ϣ¯·È̛˜ Î·È Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈο Û‡Ó‰ÚÔÌ·, ÁÈ·Ù› Ë ¯Ú‹ÛË ÂÓfi˜ ‰˘Ó·ÌÈÎÔ‡ Â·ÁˆÁÈÎÔ‡ ·˘ÍËÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·, fiˆ˜ Ô SCF, ›Ûˆ˜ Ô‰ËÁ‹ÛÂÈ Û ÌË ·Ó·ÌÂÓfiÌÂÓ· ÂÈΛӉ˘Ó· ·ÈÌ·ÙÔÏÔÁÈο ÚÔ‚Ï‹Ì·Ù·. Â) ªÂÙ·ÌfiÛ¯Â˘ÛË ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ∂›Ó·È ıÂÚ·›· ÂÎÏÔÁ‹˜ Û ·ÛıÂÓ›˜ Ô˘ ‰ÂÓ ··ÓÙÔ‡Ó ÛÙ· ÎÔÚÙÈÎÔÂȉ‹ ‹ ÂÍ·ÚÙÒÓÙ·È ·fi ÌÂÁ¿Ï˜ ‰fiÛÂȘ Î·È ÌÂÙ·ÁÁ›ÛÂȘ ÂÚ˘ıÚÔ΢ÙÙ¿ÚˆÓ Î·È ‰È·ı¤ÙÔ˘Ó Û˘Ì‚·Ùfi ‰fiÙË. ∏ ÁÚ‹ÁÔÚË Î·È ¿ÌÂÛË ÚfiÛÏË„Ë ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ Î·È Ë Ï‹Ú˘ ·ÔηٿÛÙ·ÛË Ù˘ ·ÈÌÔÔ›ËÛ˘ ·˘ÙÒÓ ÙˆÓ ·ÛıÂÓÒÓ Û ˘„ËÏfi ÔÛÔÛÙfi, ·Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Ë ‚Ï¿‚Ë Ù˘ DBA Ì¿ÏÏÔÓ ÔÊ›ÏÂÙ·È ÛÙ· ·Ú¯¤ÁÔÓ· ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· Î·È fi¯È ÛÙÔ ÌÈÎÚÔÂÚÈ‚¿ÏÏÔÓ Ù˘ ÂÚ˘ıÚÔÔ›ËÛ˘. ∏ ÚfiÁÓˆÛË Â›Ó·È ·ÌÊ›‚ÔÏË. ∆Ô 50% ÙˆÓ ·ÛıÂÓÒÓ ·˘ÙÒÓ Ô˘ ·¿ÓÙËÛ·Ó ÛÙ· ÎÔÚÙÈÎÔÂȉ‹ ÂÌÊ·Ó›˙Ô˘Ó Ì·ÎÚÔ¯ÚfiÓÈ· ÂÈ‚›ˆÛË Ì Ôχ ηϋ ÔÈfiÙËÙ· ˙ˆ‹˜, ȉȷ›ÙÂÚ· fiÛÔÈ Â›¯·Ó ·˘ÙfiÌ·Ù˜ ˘Ê¤ÛÂȘ ‹ ÂÏ¿Ì‚·Ó·Ó Ôχ ÌÈÎÚ‹ ‰fiÛË ÎÔÚÙÈ˙fiÓ˘. ∆Ô ¿ÏÏÔ 50% ›Ù ¯ÚÂÈ¿˙ÂÙ·È ÌÂÙ·ÁÁ›ÛÂȘ, ›Ù ˘Ô‚¿ÏÏÂÙ·È Û ÌÂÙ·ÌfiÛ¯Â˘ÛË

Paediatriki 2001;64:34-38

Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·Ó ‰È·ı¤ÙÂÈ Û˘Ì‚·Ùfi ‰fiÙË. ∏ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ηÎÔ‹ıÂÈ·˜ Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Â›Ó·È ˘„ËÏfiÙÂÚË Ù˘ ÂÌÊ·ÓÈ˙fiÌÂÓ˘ ÛÂ Ê˘ÛÈÔÏÔÁÈÎfi ÏËı˘ÛÌfi. ¢Â˘ÙÂÚÔ·ı›˜ ÓÂÔϷۛ˜ ¤¯Ô˘Ó ·Ó·ÊÂÚı› Û 10 ·ÛıÂÓ›˜: Û 2 ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·, Û 5 ÔÍ›· Ì˘ÂÏÔÁÂÓ‹˜ Ï¢¯·ÈÌ›·, Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ Û 2 Î·È Ë·ÙÔ΢ÙÙ·ÚÈÎfi ηÚΛӈ̷ Û 1. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Â›Ó·È Ôχ ÂÚÈÔÚÈṲ̂ÓË. ™Â ÔÈÎÔÁ¤ÓÂȘ Ì ÚÔËÁÔ‡ÌÂÓÔ ÈÛÙÔÚÈÎfi, Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ ·˘ÍË̤Ó˘ ∞DA, Ë ·Ô˘Û›· Û¯ËÌ·ÙÈÛÌÔ‡ ·ÔÈÎÈÒÓ Û ΢ÙÙ·ÚÈΤ˜ ηÏÏȤÚÁÂȘ BFU-E, CFU-E ÙˆÓ ÂÚ˘ıÚÔ‚Ï·ÛÙÒÓ ÛÙÔ ¤Ì‚Ú˘Ô ‹ Î·È ÙÔ ˘ÂÚ˯ÔηډÈÔÁÚ¿ÊËÌ· Doppler Ì ·Ó·Û‡ÓıÂÛË ÂÈÎfiÓ·˜ Û ÂÚÈÙÒÛÂȘ ·ÈÌÔ‰˘Ó·ÌÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ, ·ÔÙÂÏÔ‡Ó ÂӉ›ÍÂȘ Ù˘ ÓfiÛÔ˘. ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë DBA ·Ú·Ì¤ÓÂÈ Ì›· ‰È·Ù·Ú·¯‹ Ì ۯÂÙÈο ηϋ ÚfiÁÓˆÛË Û ۯ¤ÛË Ì ¿ÏÏ· Û‡Ó‰ÚÔÌ· Ì˘ÂÏÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜. ¶·ÚÔ‰È΋ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ (Transient erythroblastopenia of childhood, TEC) ∏ ·ÚÔ‰È΋ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂÓ›· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ Â›Ó·È Ì›· Â›ÎÙËÙË ·Ó·ÈÌ›· ÂÌÊ·ÓÈ˙fiÌÂÓË ÛÙÔ 85% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û ÚÔËÁÔ‡ÌÂÓ· ˘ÁȤ˜ ·È‰› ËÏÈΛ·˜ 1-4 ÂÙÒÓ. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ÛÙ·‰È·Î‹ ÂÌÊ¿ÓÈÛË Î·È Û ¿Óˆ ·fi 50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ¤¯ÂÈ ÚÔËÁËı› ÌÈ· ÈÔÁÂÓ‹˜ Ïԛ̈ÍË. ∏ ·Ó·ÈÌ›· Â›Ó·È ÔÚıԯڈ̷ÙÈ΋, ÔÚıÔ΢ÙÙ·ÚÈ΋ Ì ÔÈΛÏË ‰ÈÎÙ˘ÔÂÚ˘ıÚÔ-΢ÙÙ·ÚÔÂÓ›· Î·È Û ÂÚÈÛÛfiÙÂÚ˜ ·fi 60% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÛÙÔ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ ·Ó¢ڛÛÎÂÙ·È ˘ÔÏ·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜. ∏ ÂÌÊ¿ÓÈÛË Ù˘ ÓfiÛÔ˘ ¤¯ÂÈ Âԯȷ΋ ηٷÓÔÌ‹ Î·È Ì ÂÏ¿¯ÈÛÙ˜ ÂÍ·ÈÚ¤ÛÂȘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·˘ÙfiÌ·Ù˜ ˘Ê¤ÛÂȘ ÂÓÙfi˜ 4-8 ‚‰ÔÌ¿‰ˆÓ Î·È ·Ô˘Û›· ˘ÔÙÚÔÒÓ. ∂ÈϤÔÓ, ÌÔÚ› Ó· ÂÌÊ·ÓÈÛı› Û˘Á¯ÚfiÓˆ˜ ÛÙ· ·‰¤ÏÊÈ· Ì›·˜ ÔÈÎÔÁ¤ÓÂÈ·˜ Î·È Ó· Û˘Óԉ‡ÂÙ·È ·fi ·ÚÔ‰ÈΤ˜ Ó¢ÚÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜, fiˆ˜ ÂÍÔÊı¿ÏÌȘ ÎÈÓ‹ÛÂȘ, ·ÛÙ¿ıÂÈ·, ËÌÈ¿ÚÂÛË Î·È Û·ÛÌÔ‡˜. √˘ÛÈ·ÛÙÈο, Ë TEC ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙȘ ˘fiÏÔÈ˜ ÛÂÈÚ¤˜, ÎÔÎÎÈÒ‰Ë Î·È ÌÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÈ΋. ∞ӷʤÚÔÓÙ·È, fï˜, ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ Ô˘ Û˘Óԉ‡ÔÓÙ·È ·fi ˘Ô΢ÙÙ·ÚÈÎfi Ì˘ÂÏfi ‹ ·Ó·ÛÙÔÏ‹ ˆÚ›Ì·ÓÛ˘ Ù˘ ÎÔÎÎÈÒ‰Ô˘˜ ÛÂÈÚ¿˜ (maturation arrest). ∏ ·ıÔÁ¤ÓÂÈ· ‰ÂÓ Â›Ó·È ·ÎÚÈ‚Ò˜ ÁÓˆÛÙ‹. √ parvo-Èfi˜ ηıÒ˜ Î·È ¿ÏÏÔÈ ÈÔ› ÂÓ¤¯ÔÓÙ·È ÛÙËÓ TEC, ÚÔηÏÒÓÙ·˜ ·ÚÔ‰È΋ ηٷÛÙÔÏ‹ Ù˘ ÂÚ˘ıÚÔÔ›ËÛ˘. ¶·Ú·ÙËÚ‹ıËΠfiÙÈ ¿Óˆ ·fi ÙÔ 60% ÙˆÓ ·ÛıÂÓÒÓ Ì TEC ›¯·Ó ·Ó·Ù‡ÍÂÈ ·˘ÙÔ- ‹ ·ÏÏÔ- ·ÓÙÈÛÒÌ·Ù· ηٿ ·ÔÈÎÈÒÓ ÙˆÓ ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ (CFU-E, BFU-E).

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 38

¶·È‰È·ÙÚÈ΋ 2001;64:34-38

Paediatriki 2001;64:34-38

¶›Ó·Î·˜ 3. ∫ÚÈÙ‹ÚÈ· ‰È·ÊÔÚÈ΋˜ ‰È¿ÁÓˆÛ˘. PRCA ∞ÈÙ›· ¶ÚÔËÁÔ‡ÌÂÓÔ ÈÛÙÔÚÈÎfi

™˘ÁÁÂÓ‹˜ -

∞ӈ̷ϛ˜ ¡Â˘ÚÔÏÔÁÈο Â˘Ú‹Ì·Ù· ∞ÓÔÛÔÏÔÁÈο Â˘Ú‹Ì·Ù· ¢È¿ÚÎÂÈ· ª¤ÛË ËÏÈΛ·

30% Û¿ÓÈ· ¯ÚfiÓÈ· 6 ÌËÓ (1-72)

TEC ∂›ÎÙËÙË ÈÔÁÂÓ‹˜ Ïԛ̈ÍË 0% ÂÓ›ÔÙÂ Û˘Ó‹ıË ·ÚÔ‰È΋ 27 ÌËÓ (1-192)

∂ÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù·: HbF ∫º ADA ∫º Ag i Ag I ∞ÈÌÔÔ›ËÛË ÂÌ‚Ú˘ïÎÔ‡ Ù‡Ô˘

∏ ·ÓÔÛÔÏÔÁÈÎÔ‡ Ù‡Ô˘ ·ÈÙÈÔÏfiÁËÛË Ù˘ TEC ηıÒ˜ Î·È Ë ·ÚÔ‰È΋ ÂÌÊ¿ÓÈÛ‹ Ù˘ ÛÙËÓ ·È‰È΋ ËÏÈΛ· Û˘ÓËÁÔÚÔ‡Ó fiÙÈ Â›Ó·È ·ÚfiÌÔÈÔ ÓfiÛËÌ· Ì ¿ÏÏ· ·˘ÙÔ¿ÓÔÛ· ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù·, fiˆ˜ Ë È‰ÈÔ·ı‹˜ ıÚÔÌ‚ÔÂÓÈ΋ ÔÚʇڷ (ITP) Î·È Ë ·˘ÙÔ¿ÓÔÛË ·ÈÌÔÏ˘ÙÈ΋ ·Ó·ÈÌ›·. ¶·ÚfiÏÔ Ô˘ Ë ·ÌÈÁ‹˜ ·Ï·Û›· Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ (PRCA) fiÛÔ Î·È Ë TEC ÂÌÊ·Ó›˙Ô˘Ó ÎÔÈÓ¿ ÎÏÈÓÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÛÙËÓ ·Ú¯È΋ ‰È¿ÁÓˆÛË, ÂÓÙÔ‡ÙÔȘ ˘¿Ú¯Ô˘Ó Û·Ê‹ ÛÙÔȯ›· Ô˘ ͯˆÚ›˙Ô˘Ó ÙȘ ‰‡Ô ÓÔÛÔÏÔÁÈΤ˜ ÔÓÙfiÙËÙ˜ (¶›Ó·Î·˜ 3). √ parvo-Èfi˜ Â›Ó·È Ô ÈÔ Û˘¯Ófi˜ Ù‡Ô˜ ÈÔ‡ Ô˘ ÚÔηÏ› ·ÚÔ‰ÈΤ˜ ·Ï·ÛÙÈΤ˜ ÎÚ›ÛÂȘ ÛÙËÓ ·È‰È΋ ËÏÈΛ· Î·È Î˘Ú›ˆ˜ Û ·ÛıÂÓ›˜ Ì ¯ÚfiÓÈ· ·ÈÌÔÏ˘ÙÈ΋ ·Ó·ÈÌ›·, fiˆ˜ ‰ÚÂ·ÓÔ΢ÙÙ·ÚÈ΋ ·Ó·ÈÌ›· Î·È ÎÏËÚÔÓÔÌÈ΋ ÛÊ·ÈÚÔ΢ÙÙ¿ÚˆÛË. ∞ÓıÂÎÙÈΤ˜ ÂÚ˘ıÚÔ‚Ï·ÛÙÔÂӛ˜ ·fi parvo-Èfi ·Ú·ÙËÚÔ‡ÓÙ·È Û ¤ÓÙÔÓ· ·ÓÔÛÔηٷÛÙ·Ï̤ӷ ·È‰È¿ Ì AIDS, ÔÍ›· Ï¢¯·ÈÌ›· Î·È ·ÓÔÛÔ·ÓÂ¿ÚÎÂȘ. ÕÏÏÔÈ ÈÔ› fiˆ˜ Ù˘ Ë·Ù›Ùȉ·˜, Epstein Barr, HIV-1, HTLV-1, ¤¯Ô˘Ó ÂÓÔ¯ÔÔÈËı› ÁÈ· ηٷÛÙÔÏ‹ Ù˘ ÂÚ˘ıÚÔÔ›ËÛ˘. ∞ÛıÂÓ›˜ Ì ·˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù· fiˆ˜ Û˘ÛÙËÌ·ÙÈÎfi ÂÚ˘ıËÌ·ÙÒ‰Ë Ï‡ÎÔ ‹ ÚÂ˘Ì·ÙÔÂȉ‹ ·ÚıÚ›Ùȉ·, ÂÌÊ·Ó›˙Ô˘Ó ‰Â˘ÙÂÚÔ·ı‹ PRCA, ÏfiÁˆ ηٷÛÙ·ÏÙÈ΋˜ ‰Ú¿Û˘ ·˘ÙÔ·ÓÙÈÛˆÌ¿ÙˆÓ Î·Ù¿ ÙˆÓ ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ù˘ ÂÚ˘ıÚ¿˜. ∂›Û˘, ‰Â˘ÙÂÚÔ·ı‹˜ PRCA ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙ› ÌÂÙ¿ ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ Û ÂÚÈÙÒÛÂȘ ∞µ√ ·Û˘Ì‚·ÙfiÙËÙ·˜ ‰fiÙË-Ï‹ÙË. ∏ ·Ï·Û›· Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ·ÓıÂÎÙÈÎÒÓ ÈÛÔ·ÈÌÔÛ˘ÁÎÔÏÏËÙÈÓÒÓ ÙÔ˘ Ï‹ÙË Î·Ù¿ ÙˆÓ ∞ Î·È µ ·ÓÙÈÁfiÓˆÓ ÙˆÓ BFU-E Î·È CFU-E ÙÔ˘ ‰fiÙË. ™˘ÌÂÚ·ÛÌ·ÙÈο, Ë ·ÓÙÈÌÂÙÒÈÛË Ù˘ PRCA Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ ··ÈÙ› ÚÔÛÂÎÙÈ΋ ‰È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË (¶›Ó·Î·˜ 4). ∏ Ï‹„Ë ÏÂÙÔÌÂÚÔ‡˜ ÈÛÙÔÚÈÎÔ‡ Ì ÂÎÙ›ÌËÛË ÙˆÓ ÎÏÈÓÈÎÒÓ Î·È ÂÚÁ·ÛÙËÚÈ·ÎÒÓ

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¶›Ó·Î·˜ 4. ¢È·ÁÓˆÛÙÈ΋ ÚÔÛ¤ÁÁÈÛË ÙˆÓ ·È‰ÈÒÓ Ì PRCA. ™˘ÁÁÂÓ‹˜ (ËÏÈΛ·) ∂›ÎÙËÙË ¶ÚˆÙÔ·ı‹˜

¢Â˘ÙÂÚÔ·ı‹˜ πÛÙÔÚÈÎfi (ÙÔÍÈο Ê¿Ú̷η, ÈÔ›) ∫ÏÈÓÈ΋ ÂͤٷÛË ∞ÓÔÛÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ª˘ÂÏfiÁÚ·ÌÌ· ∫˘ÙÙ·ÚÈΤ˜ ηÏÏȤÚÁÂȘ ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ PCR + in situ ˘‚ÚȉÈÛÌfi˜ ÁÈ· ÚÔÛ‰ÈÔÚÈÛÌfi Parvovirus

‰Â‰ÔÌ¤ÓˆÓ ı· Ô‰ËÁ‹ÛÂÈ ÛÙËÓ ¤ÁηÈÚË Î·È ÛˆÛÙ‹ ‰È¿ÁÓˆÛË Î·ıÒ˜ Î·È ÛÙËÓ ·ÎÚÈ‚‹ ıÂÚ·¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ·ÛıÂÓÔ‡˜. µÈ‚ÏÈÔÁÚ·Ê›· 1. Alter BP, Young NS. The Bone Marrow failure syndromes. In: Nathan DJ, Oski FA, editors. Hematology of infancy and childhood. Philadelphia: WB Saunders; 1992. p. 216-236. 2. Alter BP, Knobloch ME, He L, Gillio AP, O’Reilly RJ, Reilly LK et al. Effect of stem cell factor on in vitro erythropoiesis in patients with bone marrow failure syndromes. Blood 1192;80:3000-3008. 3. Bagnara GP, Zauli G, Vitale L, Rosito P, Vecchi V, Paolucci G et al. In vitro growth and regulation of bone marrow enriched CD34+ hematopoietic progenitors in DiamondBlackfan anemia. Blood 1991;78:2203-2210. 4. Bhambhani K, Inoue S, Sarnaik S. Seasonal clustering of transient erythroblastopenia of childhood. Am J Dis Child 1998;142:175-177. 5. Chan HS, Saunders EF, Freedman MH. Diamond-Blackfan syndrome. I. Erythropoiesis in prednisone responsive and resistant disease. Pediatric Res 1982; 16:474-476. 6. Chan HS, Saunders EF, Freedman MH. Diamond-Blackfan syndrome. II. In vitro corticosteroid effect on erythropoiesis. Pediatric Res 1982;16:477-478. 7. Freedman MH, Saunders EF. Diamond-Blackfan syndrome: evidence against cell-mediated erythropoietic suppression. Blood 1978;51:1125-1128. 8. Freedman MH, Saunders EF. Transient erythroblastopenia of childhood: varied pathogenesis. Am J Hematol 1983;14:247-254. 9. Gussetis ES, Peristeri J, Kitra V, Liakopoulou T, Kattamis A, Grafakos S. Clinical value of bone marrow culture in childhood pure red cell aplasia. J Pediatr Hematol Oncol 1998;2:120-124. 10. Halperin DS, Freedman MH. Diamond-Blackfan anemia: etiology, pathophysiology, and treatment. Am J Pediatr Hematol Oncol 1989;11:380-394. HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 02-10-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 19-12-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: µ. ∫›ÙÚ· ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”


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¶·È‰È·ÙÚÈ΋ 2001;64:39-44

™À¡∂Ãπ∑√ª∂¡∏ ¶∞π¢π∞∆ƒπ∫∏ ∂∫¶∞π¢∂À™∏

Paediatriki 2001;64:39-44

CONTINUING MEDICAL EDUCATION

∞ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ıÂÚ·›· Û ·È‰È¿ Ì ‚·ÚÈ¿ ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· π.¶. ¶·Ó·ÁÈÒÙÔ˘

Immunosuppressive therapy in children with severe aplastic anemia J.P. Panagiotou

¶ÂÚ›ÏË„Ë: ∏ ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· ·Ó‹ÎÂÈ ÛÙ· Û¿ÓÈ· ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·Ï·Û›· ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ Î·È ˘Ô΢ÙÙ·ÚÈÎfi Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ. ∏ ÓfiÛÔ˜ ·ÓÙÈÌÂÙˆÈ˙fiÌÂÓË ÌfiÓÔ ÌÂ Û˘ÓÙËÚËÙÈ΋ ·ÁˆÁ‹ ¤¯ÂÈ ÊÙˆ¯‹ ÚfiÁÓˆÛË, ηıfiÛÔÓ ÌfiÓÔ ÙÔ 20% ÂÚ›Ô˘ ÙˆÓ ·ÛıÂÓÒÓ ÂÈ‚ÈÒÓÂÈ ¤Ú·Ó ÙÔ˘ ¤ÙÔ˘˜. ∞ÓÙ›ıÂÙ·, Ì ÙËÓ ÂÙÂÚfiÏÔÁË ÌÂÙ·ÌfiÛ¯Â˘ÛË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ë ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂÓÒÓ ‚ÂÏÙÈÒÓÂÙ·È ‰Ú·Ì·ÙÈο. ™ËÌ·ÓÙÈÎfi, fï˜, ÔÛÔÛÙfi ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ·ÛıÂÓÒÓ Ì ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· ·‰˘Ó·Ù› Ó· ‚ÚÂÈ Û˘Ì‚·Ùfi ‰fiÙË ÁÈ· ÙË ‰ÈÂÓ¤ÚÁÂÈ· Ù˘ ÌÂÙ·ÌfiÛ¯Â˘Û˘. °È· ÙÔ˘˜ ·ÛıÂÓ›˜ ·˘ÙÔ‡˜ Ë ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ıÂÚ·›· ·ÔÙÂÏ› ÙË ıÂÚ·›· ÂÎÏÔÁ‹˜. ∏ ·ÓÔÛÔηٷÛÙÔÏ‹ ÂÈÙ˘Á¯¿ÓÂÙ·È Ì ÙË ¯ÔÚ‹ÁËÛË ·ÓÙÈı˘ÌÔ΢ÙÙ·ÚÈÎÔ‡/ ·ÓÙÈÏÂÌÊÔ΢ÙÙ·ÚÈÎÔ‡ ÔÚÔ‡, ÌÂÌÔӈ̤ӷ ‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ΢ÎÏÔÛÔÚ›ÓË ∞, ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, ·Ó‰ÚÔÁfiÓ· ‹ ·˘ÍËÙÈÎÔ‡˜ ·ÈÌÔÔÈËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Î·È ÙÔ 60% ÂÚ›Ô˘ ÙˆÓ ·ÛıÂÓÒÓ ·ÓÙ·ÔÎÚ›ÓÂÙ·È Û ·˘Ù‹Ó. ™ÙÔ ·ÚfiÓ ¿ÚıÚÔ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ù· ÚfiÛÊ·Ù· ‰Â‰Ô̤ӷ ·Ó·ÊÔÚÈο Ì ÙË ı¤ÛË, ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Î·È ÙȘ ·ÚÂÓ¤ÚÁÂȘ Ù˘ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜.

Abstract: Aplastic anemia is a rare hematologic disorder in children characterized by blood pancytopenia and bone marrow hypoplasia. Disease have a poor prognosis with supportive care alone, with only 20% surviving one year from diagnosis. On the contrary, bone marrow transplantation is the more effective therapeutic approach, but a significant percentage of pediatric patients lack suitable donors. For these patients immunosuppressive treatment comprises an effective alternative treatment option. Immunosuppression is mainly based on antithymocyte/ antilymphocyte globulin administration, which is used either alone or in combination with cyclosporine A, corticosteroids, androgens or hemopoietic growth factors with an overall response in approximately 60%. In this review article, the role, efficacy, and side effects of immunosuppressive treatment is presented.

§¤ÍÂȘ ÎÏÂȉȿ: ·ÓÔÛÔηٷÛÙÔÏ‹, ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, ·È‰È΋ ËÏÈΛ·.

Key words: immunosuppression, aplastic anemia, childhood.

∏ ÈÛÙÔÚ›· Ù˘ ·Ï·ÛÙÈ΋˜ ·Ó·ÈÌ›·˜ (AA) Û·Ó ‰È·ÎÚÈÙ‹ ÓÔÛÔÏÔÁÈ΋ ÔÓÙfiÙËÙ·, ÍÂÎÈÓ¿ ÚÈÓ ·fi 112 ¯ÚfiÓÈ·, fiÙ·Ó ÁÈ· ÚÒÙË ÊÔÚ¿ Ô Paul Ehrlich ÂÚȤÁÚ·„ ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ ÓÔÛ‹Ì·ÙÔ˜. ∞fi ÙfiÙ ¤¯Ô˘Ó Â-

Ú¿ÛÂÈ ·ÚÎÂÙ¤˜ ‰ÂηÂٛ˜, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ÂηÙÔÓÙ¿‰Â˜ Ӥ˜ ÂÚÈÙÒÛÂȘ, Û ·È‰È¿ Î·È ÂÓ‹ÏÈΘ ÎÈ ¤¯Ô˘Ó ‰ÔÎÈÌ·Ûı› ÔÈΛϷ ıÂÚ·¢ÙÈο Û¯‹Ì·Ù·, ¿ÏÏÔÙ ·ÔÙÂÏÂÛÌ·ÙÈο Î·È ¿ÏÏÔÙ fi¯È.

∂ÈÌÂÏËÙ‹˜ ∞’ ∆Ì‹Ì·ÙÔ˜ ¶·È‰È·ÙÚÈ΋˜ ∞ÈÌ·ÙÔÏÔÁ›·˜ / √ÁÎÔÏÔÁ›·˜ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∏ ∞Á›· ™ÔÊ›·”, ∞ı‹Ó·

Registrar Pediatric Hematology/Oncology Division “Saint Sophia” Children’s Hospital, Athens

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 40

¶·È‰È·ÙÚÈ΋ 2001;64:39-44

∆· ÚÒÙ· ¯ÚfiÓÈ·, Ô˘ Ë ıÂÚ·›· Ù˘ ∞∞ ÛÙËÚÈ˙fiÙ·Ó ÛÙËÓ ˘ÔÛÙËÚÈÎÙÈ΋ ·ÁˆÁ‹ Ì ÌÂÙ·ÁÁ›ÛÂȘ ·›Ì·ÙÔ˜ Î·È ÛÙÔȯÂÈÒ‰Ë ·ÓÙÈÌÈÎÚԂȷ΋ Î¿Ï˘„Ë, Ë ÔÚ›· ÙˆÓ ·ÛıÂÓÒÓ ‹Ù·Ó ·ÔÁÔËÙ¢ÙÈ΋ Ì ‰ÈÂÙ‹ ÂÈ‚›ˆÛË Û ÔÛÔÛÙfi Î˘Ì·ÈÓfiÌÂÓÔ ÌÂٷ͇ 20-30%. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, ¿ÏψÛÙÂ, ÛÙÔ ÂÁ¯ÂÈÚ›‰ÈÔ Wintrobe’s Clinical Haematology ÙÔ˘ 1956, Ë ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ «ÓfiÛÔ˜ Ô˘ ÚÔԉ¢ÙÈο, ·Ì›ÏÈÎÙ· Î·È Î·Ù¿ ÙÔ Ì¿ÏÏÔÓ ‹ ‹ÙÙÔÓ Ù·¯¤ˆ˜ Ô‰ËÁ› ÛÙÔ ı¿Ó·ÙÔ. √ ı¿Ó·ÙÔ˜ Â¤Ú¯ÂÙ·È ÂÓÙfi˜ ÔÏ›ÁˆÓ ‚‰ÔÌ¿‰ˆÓ ‹ ˘¿Ú¯ÂÈ ·Ú¿Ù·ÛË ˙ˆ‹˜ ̤¯ÚÈ 6 Ì‹Ó˜». ∏ ÚÒÙË ·Ó·ÊÔÚ¿ Û 40 ·È‰È·ÙÚÈÎÔ‡˜ ·ÛıÂÓ›˜ Ì ∞∞ Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÌÂ Û˘ÛÙËÌ·ÙÈ΋ ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Î·È ÙÂÛÙÔÛÙÂÚfiÓ˘ Á›ÓÂÙ·È ÙÔ 1959 ·fi ÙÔ˘˜ Shahidi Î·È Diamond Î·È ÔÈ ÔÔ›ÔÈ ·Ó·Ê¤ÚÔ˘Ó ÔÛÔÛÙfi Ì·ÎÚfi¯ÚÔÓ˘ ÂÈ‚›ˆÛ˘ Û ÔÛÔÛÙfi 4% ÙˆÓ ·ÛıÂÓ›˜. ∆˘ ‰ËÌÔÛ›Â˘Û˘ ·˘Ù‹˜, ›¯Â ÚÔËÁËı› Ë ·Ó·ÎÔ›ÓˆÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Û 334 ·ÛıÂÓÒÓ, ÂÎ ÙˆÓ ÔÔ›ˆÓ ÙÔ 24,3% ·ÊÔÚÔ‡Û ·È‰È¿. ∏ Û˘ÓÔÏÈ΋ Ì·ÎÚfi¯ÚÔÓË ÂÈ‚›ˆÛË ÙˆÓ ·ÓÂÚ¯fiÙ·Ó ÌfiÏȘ ÛÙÔ 3,3% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ·ÛıÂÓÒÓ. ™Ù· Ù¤ÏË Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ ’60, ·fi ÙÔÓ Mathé G Î·È Û˘Ó, Á›ÓÂÙ·È Ë ÚÒÙË ·Ó·ÊÔÚ¿ ÛÙÔ ıÂÚ·¢ÙÈÎfi ÚfiÏÔ Ô˘ ÂӉ¯fiÌÂÓ· ı· ÌÔÚÔ‡Û ӷ ¤¯ÂÈ Ë ·ÓÙÈÏÂÌÊÔ΢ÙÙ·ÚÈ΋ ÛÊ·ÈÚ›ÓË (ALG) ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ∞∞, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙËÓ ALG Û·Ó ÚÔ·Ú·Û΢·ÛÙÈÎfi Û¯‹Ì· Û ·ÏÏÔÁÂÓ‹ ÌÂÙ·ÌfiÛ¯Â˘ÛË ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ∆· ¯ÚfiÓÈ· Ô˘ ·ÎÔÏÔ‡ıËÛ·Ó ÂȂ‚·›ˆÛ·Ó ÙȘ ıÂÚ·¢ÙÈΤ˜ ‰˘Ó·ÙfiÙËÙ˜ ÙÔ˘ Û΢¿ÛÌ·ÙÔ˜ ÎÈ ¤ÎÙÔÙ ·Ú·Ì¤ÓÂÈ ¤Ó·˜ ‚·ÛÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÛÙË ıÂÚ·›· ·˘ÙÔ‡ ÙÔ˘ ÛÔ‚·ÚÔ‡ ÓÔÛ‹Ì·ÙÔ˜. °È· ÙËÓ Î·Ï‡ÙÂÚË ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ∞∞ ‹Ù·Ó ··Ú·›ÙËÙÔ Ó· ‚ÚÂı› ÎÔÈÓ‹ ÁÏÒÛÛ· ÂÈÎÔÈÓˆÓ›·˜. ¶ÚÔ˜ ÙËÓ Î·Ù‡ı˘ÓÛË ·˘Ù‹ ÎÈÓÔ‡ÌÂÓÔÈ ÔÈ Camitta Î·È Û˘Ó, ı¤ÛÈÛ·Ó Ù· ÎÚÈÙ‹ÚÈ· Ù˘ ‚·ÚÈ¿˜ ∞∞ Ì ‚¿ÛË Ù· ÔÔ›· ÔÈ ·ÛıÂÓ›˜ ÂÓÙ¿ÛÛÔÓÙ·È Û ÔÌ¿‰Â˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Î·È ¯Ú‹˙Ô˘Ó ÂÓÙ·ÙÈ΋˜ Î·È ÂÈıÂÙÈ΋˜ ıÂÚ·¢ÙÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘. ∆· ÎÚÈÙ‹ÚÈ· ·˘Ù¿ ·Ó·Ï˘ÙÈο ›ӷÈ: ∫˘ÙÙ·ÚÈÎfiÙËÙ· Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (ª√) οو ·fi ÙÔ 25% Ù˘ ·Ó·ÌÂÓfiÌÂÓ˘ ÁÈ· ÙËÓ ËÏÈΛ· ÙÔ˘ ·ÛıÂÓÔ‡˜, Ì ÙË Ì˘ÂÏÈ΋ ÛÂÈÚ¿ Ó· ·ÔÙÂÏ› ÏÈÁfiÙÂÚÔ ·fi ÙÔ 30% Î·È ‚Ï·ÛÙÈο ·ÙÙ·Ú· οو ÙÔ˘ ÔÚ›Ô˘ ÙˆÓ 5%. ∆·˘Ùfi¯ÚÔÓ·, ı· Ú¤ÂÈ Ó· Û˘Ó˘¿Ú¯Ô˘Ó 2, ÙÔ˘Ï¿¯ÈÛÙÔÓ, ·fi Ù· ηو٤ڈ ·Ó·ÊÂÚfiÌÂÓ· Â˘Ú‹Ì·Ù· ·fi ÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· Î·È Ù· ÔÔ›· ›ӷÈ: ∞fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ Ô˘‰ÂÙÂÚfiÊÈÏˆÓ ÔÏ˘ÌÔÚÊÔ˘Ú‹ÓˆÓ <500/mm3 ∞ÚÈıÌfi˜ ·ÈÌÔÂÙ·Ï›ˆÓ <2x104/mm3 ¢ÈÔÚıˆÌ¤ÓÔ˜ ‰ÈÎÙ˘Ô-ÂÚ˘ıÚÔ΢ÙÙ·ÚÈÎfi˜ ‰Â›ÎÙ˘ <1%. ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, ÛÙ· ÚÔ·Ó·ÊÂÚı¤ÓÙ· ÎÚÈÙ‹ÚÈ· Û˘ÌÂÚÈÏ‹ÊıËΠ̛· ·ÎfiÌË ÔÌ¿‰· ÙˆÓ ·ÛıÂÓÒÓ ÌÂ

40

Paediatriki 2001;64:39-44

Ôχ ‚·ÚÈ¿ ∞∞ Î·È Ì Ôχ η΋ ¤Î‚·ÛË, ÛÙËÓ ÔÔ›· ÂÓÙ¿ÛÛÔÓÙ·È ÔÈ ·ÛıÂÓ›˜ ÙˆÓ ÔÔ›ˆÓ Ô ·fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ ÙˆÓ Ô˘‰ÂÙÂÚfiÊÈÏˆÓ ÔÏ˘ÌÔÚÊÔ˘Ú‹ÓˆÓ ·Ó¢ڛÛÎÂÙ·È <200/mm3. °È· ÙËÓ Î·Ï‡ÙÂÚË ÚÔÛ¤ÁÁÈÛË Ù˘ Ê˘ÛÈ΋˜ ÈÛÙÔÚ›·˜ ÙÔ˘ ÓÔÛ‹Ì·ÙÔ˜ Î·È ÙË ‰È¢ÎÚ›ÓÈÛË ÙÔ˘ ‹ ÙˆÓ ·ÈÙ›ˆÓ Ô˘ ÂȉÚÔ‡Ó ¿Óˆ ÛÙË Ê˘ÛÈÔÏÔÁÈ΋ ΢ÙÙ·ÚÈÎfiÙËÙ· Î·È ÙË ‰È·‰Èηۛ· ·ÈÌÔÔ›ËÛ˘ ÙÔ˘ ª√, ·ÔÁ˘ÌÓÒÓÔÓÙ·˜ ÙÔÓ ÙÂÏÂ˘Ù·›Ô ·fi Ù· ΢ÙÙ·ÚÈο ÙÔ˘ ÛÙÔȯ›·, ¤ÁÈÓ·Ó ÔÏϤ˜ Â›ÔÓ˜ Î·È ¯ÚÔÓÔ‚fiÚ˜ ÌÂϤÙ˜. ™˘ÓÔÙÈο, Ù· ·ÔÙÂϤÛÌ·Ù· ·˘ÙÒÓ ÙˆÓ ÚÔÛ·ıÂÈÒÓ Û˘Ó¤ÎÏÈÓ·Ó ÛÙÔ fiÙÈ Ë ∞∞ ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È: 1. ™Â ‚Ï¿‚Ë ÙÔ˘ ·Ú¯¤ÁÔÓÔ˘ ÔÏ˘‰‡Ó·ÌÔ˘ ·ÈÌÔÔÈËÙÈÎÔ‡ ΢ÙÙ¿ÚÔ˘ (stem cell-SC), ÔÊÂÈÏfiÌÂÓË Û ÂÓ‰ÔÁÂÓ¤˜ ¤ÏÏÂÈÌÌ· ÙÔ˘ SC ‹ Û ÙÔÍÈ΋ ‰Ú¿ÛË Â͈ÁÂÓÒÓ ·Ú·ÁfiÓÙˆÓ (.¯. ΢ÎÏÈÎÔ› ˘‰ÚÔÁÔÓ¿ÓıڷΘ, Èfi˜ Ë·Ù›Ùȉ·˜ µ Î.¿.). ∫¿Ùˆ ·fi ·˘Ùfi ÙÔ Ú›ÛÌ·, ÂȯÂÈÚÂ›Ù·È Ë ·ÓÙÈηٿÛÙ·ÛË ÙˆÓ ·ıÔÏÔÁÈÎÒÓ SC ̤ۈ Ù˘ ·ÏÏÔÁÂÓÔ‡˜ ÌÂÙ·ÌfiÛ¯Â˘Û˘ ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È Ë ÔÔ›· ¤¯ÂÈ Ôχ ηϿ ıÂÚ·¢ÙÈο ·ÔÙÂϤÛÌ·Ù·. 2. ™Â ηٷÛÙÚÔÊÈ΋ ‰Ú¿ÛË ÔÈÎ›ÏˆÓ ·Ú·ÁfiÓÙˆÓ Ì¤Ûˆ ‰Ú·ÛÙËÚÈÔÔ›ËÛ˘ ¿ÓÔÛˆÓ Ì˯·ÓÈÛÌÒÓ Î·È Ù˘ ηٷÛÙÚÔÊ‹˜ ÙˆÓ SC ̤ۈ ·ÓÔÛÔÚÔÎÏËÙÒÓ ·ÓÙȉڿÛˆÓ, ÛÙȘ Ôԛ˜ ÎÂÓÙÚÈ΋, ˘„›ÛÙ˘ ÛËÌ·Û›·˜ ı¤ÛË Î·Ù¤¯ÂÈ ÙÔ ÂÓÂÚÁÔÔÈË̤ÓÔ ÏÂÌÊÔ·ÙÙ·ÚÔ. ∏ ALG, Ë ·ÓÙÈı˘ÌÔ΢ÙÙ·ÚÈ΋ ÛÊ·ÈÚ›ÓË (ATG), Ù· ÎÔÚÙÈÎÔÂȉ‹ Î·È Ë Î˘ÏÔÛÔÚ›ÓË-∞ ÂÓÙ¿ÛÛÔÓÙ·È ÛÙË ÌÂÁ¿ÏË ÔÌ¿‰· Ù˘ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜, Ù˘ ÔÔ›·˜ Ô ÚfiÏÔ˜ Û˘Ó›ÛÙ·Ù·È ÛÙË ‰È·ÎÔ‹ Î·È ·Ó·ÛÙÚÔÊ‹ ·˘Ù‹˜ Ù˘ ηٷÛÙÚÔÊÈ΋˜ ‰È·‰Èηۛ·˜. ∆· ·ÔÙÂϤÛÌ·Ù· Â›Ó·È ÈηÓÔÔÈËÙÈο, ·ÏÏ¿ Û·ÊÒ˜ ˘ÔÏ›ÔÓÙ·È Ù˘ ·ÏÏÔÁÂÓÔ‡˜ ªª√. 3. ™Â ‚Ï¿‚Ë ÙÔ˘ ÛÙÚÒÌ·ÙÔ˜, ÚÔηÏÔ‡ÌÂÓË ·fi ÙË ‰Ú¿ÛË ÙÔÍÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. H ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹ ÍÂΛÓËÛ ӷ ÂÊ·ÚÌfi˙ÂÙ·È ÛÙȘ ·Ú¯¤˜ Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ ’50 Ì ÙË ¯ÔÚ‹ÁËÛË Û΢·ÛÌ¿ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ. ∏ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ Û˘ÁÎÂÎÚÈÌ¤ÓˆÓ ıÂÚ·¢ÙÈÎÒÓ Û¯ËÌ¿ÙˆÓ Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÎÙÈÌËı›, ‰Â‰Ô̤Ó˘ Ù˘ ¤ÏÏÂȄ˘ ·ÓÙÈÎÂÈÌÂÓÈÎÒÓ ÎÚÈÙËÚ›ˆÓ ηٿٷ͢ Î·È ·ÓÙ·fiÎÚÈÛ˘ ÙˆÓ ·ÛıÂÓÒÓ. ¶·ÚfiÙÈ Ù· ÛÙÂÚÔÂȉ‹ Â›Ó·È Û΢¿ÛÌ·Ù· Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÔÈΛÏ˘ ·ÈÙÈÔÏÔÁ›·˜ ÓÔÛËÌ¿ÙˆÓ Â› ÔÏϤ˜ ‰ÂηÂٛ˜, ̤¯ÚÈ Û‹ÌÂÚ· Ô ·ÎÚÈ‚‹˜ ÙÚfiÔ˜ ‰Ú¿Û˘ ÙÔ˘˜ Â›Ó·È ·‰È¢ÎÚ›ÓÈÛÙÔ˜. ∂›Ó·È Èı·Ófi Ë ‰Ú¿ÛË ÙÔ˘˜ Ó· Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ·Ú¤Ì‚·Û‹ ÙÔ˘˜ Û ·ÓÔÛÔÏÔÁÈΤ˜ ·ÓÙȉڿÛÂȘ ΢ÙÙ·ÚÔÌÂÛÔÏ·‚ËÙÈÎÔ‡ Ù‡Ô˘ ‹ Ì ÙË Ì·ÎÚÔ¯ÚfiÓÈ· ¯ÔÚ‹ÁËÛ‹ ÙÔ˘˜ Â›Ó·È ÂӉ¯fiÌÂÓÔ Ó· ηٷÛÙÚ¤ÊÔ˘Ó ‹ Ó· ÂÌÔ‰›˙Ô˘Ó ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ ∆-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ. ∞˘Ùfi ¿ÓÙˆ˜, Ô˘ ¤¯ÂÈ ‰È·ÈÛÙˆı› Â›Ó·È fiÙÈ ¯ÔÚËÁÔ‡ÌÂÓ· Û ¯·ÌËϤ˜ ‰fiÛÂȘ ‰ÂÓ ¤¯Ô˘Ó ‰Ú¿ÛË Û ·ÛıÂÓ›˜ Ì ∞∞. °È· ÙÔÓ


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 41

¶·È‰È·ÙÚÈ΋ 2001;64:39-44

ÏfiÁÔ ·˘Ùfi, ÔÈ ÚÔÙÂÈÓfiÌÂÓ˜ ‰fiÛÂȘ Â›Ó·È ˘„ËϤ˜ Î·È ·Ó¿ÏÔÁ· Ì ÙÔ Î¤ÓÙÚÔ ÚÔ¤Ï¢Û˘ ÙˆÓ ÌÂÏÂÙÒÓ, Î˘Ì·›ÓÂÙ·È ·fi 20-30 mg/Kg ·Ú¯Èο Î·È ÛÙË Û˘Ó¤¯ÂÈ· ÌÂÈÒÓÂÙ·È ÛÙ·‰È·Î¿. ∏ Ì›ˆÛË ‰È·ÚΛ ÂÚ›Ô˘ 30 Ë̤Ú˜ Î·È ·ÎÔÏÔ‡ıˆ˜ ¯ÔÚËÁÂ›Ù·È Û ‰fiÛË Û˘ÓÙ‹ÚËÛ˘, Î˘Ì·ÈÓfiÌÂÓË ·fi 0,1-0,2 mg/Kg/24ˆÚÔ. ∆Ô ÔÛÔÛÙfi ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ·ÓÙ·ÔÎÚ›ÓÂÙ·È Û ·˘Ù¿ Ù· ıÂÚ·¢ÙÈο Û¯‹Ì·Ù· Î˘Ì·›ÓÂÙ·È ·fi 25-60%. ™‹ÌÂÚ·, ÛÙ· Û˘Ó‰˘·Ṳ̂ӷ Û¯‹Ì·Ù· ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜ Ô˘ ¯ÔÚËÁÔ‡ÓÙ·È ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ∞∞, ÔÈ ˘„ËϤ˜ ‰fiÛÂȘ ÙˆÓ ÛÙÂÚÔÂȉÒÓ ¤¯Ô˘Ó ÙË ı¤ÛË ÙÔ˘˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ALG/ATG ‹ Î·È Î˘ÎÏÔÛÔÚ›ÓË-∞ (CsA). √È ·ÚÂÓ¤ÚÁÂȘ ÙˆÓ ÛÙÂÚÔÂȉÒÓ Â›Ó·È ÁÓˆÛÙ¤˜ Î·È ÔÈ ÈÔ Û˘¯Ó¿ ·Ó·ÊÂÚfiÌÂÓ˜ Â›Ó·È Ë ˘¤ÚÙ·ÛË, Ë ˘ÂÚÁÏ˘Î·ÈÌ›·, Ë ·˘ÍË̤ÓË ·ÒÏÂÈ· Î·Ï›Ô˘, Ë Â˘·ÈÛıËÛ›· ÛÙȘ ÏÔÈÌÒÍÂȘ, Ë „‡¯ˆÛË Î·È Ë ¿ÛËÙË Ó¤ÎÚˆÛË Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘ ÌËÚÈ·›Ô˘. ∏ ΢ÎÏÔʈÛÊ·Ì›‰Ë (CP), ÂÚÈÛÛfiÙÂÚÔ ÁÓˆÛÙ‹ Û·Ó ·ÓÙÈÓÂÔÏ·ÛÌ·ÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜, ‰ÔÎÈÌ¿ÛıËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ∞∞ ÛÙ· ̤۷ Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ ’70, ¯ˆÚ›˜ fï˜, ÙÂÏÈο, Ó· Ù‡¯ÂÈ Â˘Ú›·˜ ·Ô‰Ô¯‹˜ ˆ˜ Ê¿ÚÌ·ÎÔ ÚÒÙ˘ ÂÈÏÔÁ‹˜ Û ·ÛıÂÓ›˜ ¯ˆÚ›˜ Û˘Ì‚·Ùfi ‰fiÙË. ∏ ‰Ú¿ÛË Ù˘ CP Ê·›ÓÂÙ·È Ó· ÛÙËÚ›˙ÂÙ·È ÛÙË ÌË ÂȉÈ΋ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ‰Ú¿ÛË Ô˘ ÂÌÊ·Ó›˙ÂÈ, ÂΉËÏÔ‡ÌÂÓË ÛÙ· Ï·›ÛÈ· Ù˘ Ì˘ÂÏÔÙÔÍÈ΋˜ Ù˘ ‰Ú¿Û˘. ∏ Èı·ÓfiÙËÙ· ‰Â, Ó· ηٷÛÙÚ¤ÊÂÈ Ù· SC Ô˘ ¤¯Ô˘Ó ·Ú·‚Ï·Êı›, ÛÙ· ÔÔ›· Â›Ó·È ÂӉ¯fiÌÂÓÔ Ó· ÔÊ›ÏÂÙ·È Ë ÌÂÏÏÔÓÙÈ΋ ÂÌÊ¿ÓÈÛË Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘, ·ÚÔ͢ÛÌÈ΋˜ Ó˘ÎÙÂÚÈÓ‹˜ ·ÈÌÔÛÊ·ÈÚÈÓÔ˘Ú›·˜ ‹ ÔÍ›·˜ Ï¢¯·ÈÌ›·˜, Ù˘ ÚÔÛ‰›‰ÂÈ, fiˆ˜ ÙÔ˘Ï¿¯ÈÛÙÔÓ ·Ó·Ê¤ÚÔ˘Ó ÔÈ Û˘ÁÁÚ·Ê›˜ Ù˘ ÌÂϤÙ˘, ÏÂÔÓ¤ÎÙËÌ· ¤Ó·ÓÙÈ ÙˆÓ ¿ÏÏˆÓ ·ÓÔÛÔηٷÛÙ·ÏÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ∆Ô ‰ÔÛÔÏÔÁÈÎfi Û¯‹Ì· Ô˘ ÂÊ·ÚÌfiÛÙËΠÛÙË ÌÂϤÙË ‹Ù·Ó 45 mg/Kg ÙËÓ Ë̤ڷ, Â› 4 Û˘Ó¯›˜ Ë̤Ú˜, ÙÔ Ê¿ÚÌ·ÎÔ ¯ÔÚËÁ‹ıËΠ̠‹ ¯ˆÚ›˜ Cs∞ Î·È 7/10 ·ÛıÂÓ›˜ ¤Ù˘¯·Ó Ï‹ÚË ‡ÊÂÛË Ù˘ ÓfiÛÔ˘. ∏ ÙÔÍÈÎfiÙËÙ· Ù˘ CP ÂΉËÏÒÓÂÙ·È, ΢ڛˆ˜ ·fi ÙÔ Ô˘ÚÔÔÈËÙÈÎfi Û‡ÛÙËÌ· (·ÈÌÔÚÚ·ÁÈ΋ ΢ÛÙ›Ùȉ·, ÓÂÊÚÈ΋ ‚Ï¿‚Ë), ÙÔ Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Ì ·Ï·Û›· fiÏˆÓ ÙˆÓ ÛÂÈÚÒÓ Î·È ÙÔ˘˜ Ó‡ÌÔÓ˜ Ì ‰È¿ÌÂÛË Ó¢ÌÔÓ›Ùȉ·. √È ALG Î·È ATG Â›Ó·È ÎÂηı·Ṳ́Ó˜ Î·È Û˘Ì˘Îӈ̤Ó˜ ÛÊ·ÈÚ›Ó˜, ÚÔÂÚ¯fiÌÂÓ˜ ·fi ¢·ÈÛıËÙÔÔÈË̤ӷ ˙Ò· (¿ÏÔÁÔ, ÎÔ˘Ó¤ÏÈ), ÛÙ· ÔÔ›· ¤¯ÂÈ Á›ÓÂÈ ¤Á¯˘ÛË ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ ·fi ÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì· ‹ ÙÔ ıˆÚ·ÎÈÎfi fiÚÔ ·ÓÙ›ÛÙÔȯ·. ∞ÔÙÂÏÔ‡Ó ÙÔ˘˜ ϤÔÓ Î·Ï¿ ÌÂÏÂÙË̤ÓÔ˘˜ Î·È ÂÚÈÛÛfiÙÂÚÔ ¯ÚËÛÈÌÔÔÈË̤ÓÔ˘˜ ·ÓÔÛÔηٷÛÙ·ÏÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ∞∞, ÁÈ· ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· 30 ÂÚ›Ô˘ ÂÙÒÓ. ∞ÓÂÍ¿ÚÙËÙ· Ù˘ ÂÌÂÈÚ›·˜ Ô˘ ¤¯ÂÈ Î¿ı ıÂÚ·¢ÙÈÎfi ΤÓÙÚÔ, fiÏÔÈ Û˘ÌʈÓÔ‡Ó fiÙÈ Û ·ÛıÂÓ›˜ Ì ‚·ÚÈ¿ ∞∞ Î·È ¯ˆÚ›˜ Û˘Ì‚·Ùfi ‰fiÙË Ú¤ÂÈ Ó· ¯ÔÚËÁÂ›Ù·È Û¯‹-

Paediatriki 2001;64:39-44

Ì· Ô˘ ı· ÂÌÂÚȤ¯ÂÈ ÙËÓ ALG/∞TG. ∏ ¯Ú‹ÛË ÙÔ˘˜ ÛÙËÚ›˙ÂÙ·È ÛÙËÓ ˘fiıÂÛË fiÙÈ Û ÛËÌ·ÓÙÈÎfi ·ÚÈıÌfi ·ÛıÂÓÒÓ Ì ∞∞ Ë ÓfiÛÔ˜ ÔÊ›ÏÂÙ·È ÛÙËÓ ·ÚÔ˘Û›· ΢ÙÙ·ÚÔÙÔÍÈÎÒÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ, Ù· ÔÔ›· ·ÛÎÔ‡Ó Î·Ù·ÛÙ·ÏÙÈ΋ ‰Ú¿ÛË ÛÙË Ê˘ÛÈÔÏÔÁÈ΋ ‰È·‰Èηۛ· Ù˘ ·ÈÌÔÔ›ËÛ˘. ∫·Ù¿ Û˘Ó¤ÂÈ·, Ô ÂÚÈÔÚÈÛÌfi˜ ÙÔ˘˜ ̤ۈ ÙˆÓ ÛÊ·ÈÚÈÓÒÓ, ¯ˆÚ›˜ Ó· ·ÔηıÈÛÙ¿ ÙËÓ ÂӉ¯fiÌÂÓË ‚Ï¿‚Ë ÙˆÓ SC, ‚ÔËı¿ ÛÙËÓ Â·Ó¤Ó·ÚÍË Ù˘ ·Ô‰ÔÙÈ΋˜ ·ÈÌÔÔ›ËÛ˘. ∆· Û΢¿ÛÌ·Ù· ÙˆÓ ÛÊ·ÈÚÈÓÒÓ ÂÚȤ¯Ô˘Ó ÔÈΛϷ ·ÓÙÈÛÒÌ·Ù· ÛÙÚÂÊfiÌÂÓ· ¤Ó·ÓÙÈ ÔÏÏÒÓ ÏÂÌÊÔ΢ÙÙ·ÚÈÎÒÓ ·ÓÙÈÁfiÓˆÓ (.¯. CD2, CD3, CD4, Î.¿.), Ì ·ÔÙ¤ÏÂÛÌ· Ë ‰Ú¿ÛË ÙÔ˘˜ Ó· Â›Ó·È Û‡ÓıÂÙË Î·È Ó· ·ÊÔÚ¿ ÛÂ: ∫·Ù·ÛÙÚÔÊ‹ Î·È ·Ó·ÛÙÔÏ‹ Ù˘ ‰Ú¿Û˘ fiψÓ, ۯ‰fiÓ, ÙˆÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ, Ì ÂȉÈÎfiÙËÙ· ΢ڛˆ˜ ÛÙ· ΢ÙÙ·ÚÔÙÔÍÈο, Ô˘ η٤¯Ô˘Ó ÚfiÏÔ Î·Ù·ÛÙ·ÏÙÈÎfi Ù˘ ·ÈÌÔÔ›ËÛ˘. ªÂ ÙÔÓ ÙÚfiÔ ·˘Ùfi, ÂÈÙÚ¤Ô˘Ó ÙÔÓ ·ÚfiÛÎÔÙÔ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ SC Î·È ÙËÓ Â·ÓÂÁηٿÛÙ·ÛË Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ·ÈÌÔÔ›ËÛ˘. ¶·ÚÂÌfi‰ÈÛË Ù˘ ·Ú·ÁˆÁ‹˜ ηٷÛÙ·ÏÙÈÎÒÓ Ù˘ ·ÈÌÔÔ›ËÛ˘ ÏÂÌÊÔÎÈÓÒÓ, Ì ·Ó¿ÏÔÁÔ Ì ÙÔÓ ·ÓˆÙ¤Úˆ Ì˯·ÓÈÛÌfi. ∞ÓÔÛԉȤÁÂÚÛË ÙˆÓ ∆-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ Î·È ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ÛÙÚÒÌ·ÙÔ˜ ÚÔ˜ ·Ú·ÁˆÁ‹ ·˘ÍËÙÈÎÒÓ Ù˘ ·ÈÌÔÔ›ËÛ˘ ·Ú·ÁfiÓÙˆÓ Î·È ÙËÓ, ˆ˜ ÂÎ ÙÔ‡ÙÔ˘, ·ÔηٿÛÙ·ÛË Ù˘ ‚Ï¿‚˘. ∞ÓÔÛԉȤÁÂÚÛË Ì¤Ûˆ ·Ú·ÁˆÁ‹˜ ·ÓÙÈ-·ÓÙÈ-ȉÈÔÙ˘ÈÎÒÓ ÌÔÚ›ˆÓ, Ù· ÔÔ›· ÌÈÌÔ‡ÓÙ·È ÙË Û‡Ó‰ÂÛË Ì ٷ ÚˆÙfiÙ˘· ·ÓÙÈÛÒÌ·Ù· Ù· ¢ÚÈÛÎfiÌÂÓ· ÛÙÔÓ ›ÂÈÔ ÔÚfi. ÕÌÂÛË ‰Ú¿ÛË ÛÙ· SC, Ù· ÔÔ›· Î·È ‰Ú·ÛÙËÚÈÔÔÈÔ‡Ó. ¶Ú¤ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÔÈ ‰‡Ô ÙÂÏÂ˘Ù·›ÔÈ Ì˯·ÓÈÛÌÔ› ·ÊÔÚÔ‡Ó ÂÈÚ·Ì·ÙÈο ‰Â‰Ô̤ӷ ¯ˆÚ›˜ Ó· ¤¯Ô˘Ó ÂȂ‚·Èˆı› in vitro. ∞fi ÙÔ 1970, fiÙ·Ó ÂÊ·ÚÌfiÛıËÎÂ Ë ¯ÔÚ‹ÁËÛË ÙˆÓ ALG/ATG, ¤¯Ô˘Ó ‰ÔÎÈÌ·Ûı› ÔÈΛϷ ıÂÚ·¢ÙÈο Û¯‹Ì·Ù· Ì ‰fiÛÂȘ Ô˘ Î˘Ì·›ÓÔÓÙ·Ó ·fi 15-40 mg/Kg/24ˆÚÔ Î·È ‰È¿ÚÎÂÈ·˜ ·fi 5-28 Ë̤Ú˜. ªÂÙ¿ ·fi ÂÚ¢ÓËÙÈΤ˜ ÌÂϤÙ˜ Ô˘ ÛÙfi¯Â˘·Ó ÛÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ Î·È Ù˘ ÙÔÍÈÎfiÙËÙ·˜, Û‹ÌÂÚ· ÚÔÙÈÌÒÓÙ·È Ù· ‚Ú·¯Â›·˜ ¯ÚÔÓÈ΋˜ ‰È¿ÚÎÂÈ·˜ Û¯‹Ì·Ù·, ‰È¿ÚÎÂÈ·˜ 4-5 ËÌÂÚÒÓ, Ì ‰fiÛË ÂÎÏÔÁ‹˜ Ù· 40 mg/Kg/24ˆÚÔ Ì ·Ú¿ÏÏËÏË ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÂȉÒÓ ‹ Î·È CsA. H ¤Á¯˘ÛË ÙˆÓ Û΢·ÛÌ¿ÙˆÓ ‰È·ÚΛ 6-8 ÒÚ˜ Î·È ÚÔ˜ ·ÔÊ˘Á‹ ¤ÓÙÔÓˆÓ ·ÏÏÂÚÁÈÎÒÓ ·ÓÙȉڿÛÂˆÓ ÚÔËÁÂ›Ù·È ‰ÔÎÈÌ·Û›· ¢·ÈÛıËÛ›·˜. °È· ÙËÓ ÈÔ ·ÛÊ·Ï‹ ¯ÔÚ‹ÁËÛË ÙˆÓ ÛÊ·ÈÚÈÓÒÓ Î·È ÙËÓ Î·Ï‡ÙÂÚË ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÚÂÔÌ¤ÓˆÓ Ù˘ ¯ÔÚ‹ÁËÛ˘, ··Ú·›ÙËÙË ÎÚ›ÓÂÙ·È Ë ·ÚÔ˘Û›· ÎÂÓÙÚÈÎÔ‡ ÊÏ‚ÈÎÔ‡ ηıÂÙ‹Ú· Î·È Ë ÂÍ·ÛÊ¿ÏÈÛË ÈηÓÔ‡ ·ÚÈıÌÔ‡ ÌÔÓ¿‰ˆÓ Û˘Ì˘ÎÓˆÌ¤ÓˆÓ ÂÚ˘ıÚÒÓ Î·È Î˘Ú›ˆ˜ ·ÈÌÔÂÙ·Ï›ˆÓ.

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 42

¶·È‰È·ÙÚÈ΋ 2001;64:39-44

√È Î‡ÚȘ ·ÚÂÓ¤ÚÁÂȘ ÙˆÓ ALG/ATG ·ÊÔÚÔ‡Ó Û ¿ÌÂÛ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ, ÔÚÔÓÔÛ›· Î·È ·ÚÔ‰È΋ ·Ï·Û›· ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜. √ ˘ÚÂÙfi˜, Ù· Ú›ÁË Î·È ÙÔ ÎÓȉˆÙÈÎfi ÂÍ¿ÓıËÌ· ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ ·ÔÙÂÏÔ‡Ó ÙȘ Û˘ÓËı¤ÛÙÂÚ˜ ·ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ, ÔÈ Ôԛ˜ ÂÌÊ·Ó›˙ÔÓÙ·È 1-2 Ë̤Ú˜ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ¯ÔÚ‹ÁËÛ˘, ·ÓÙÈÌÂÙˆÈ˙fiÌÂÓ˜ Ì ·ÓÙÈÈÛÙ·ÌÈÓÈο Û΢¿ÛÌ·Ù·. £ÂÙÈ΋ ‰ÂÚÌ·ÙÈ΋ ‰ÔÎÈÌ·Û›· ÌÔÚ› Ó· ÚÔ·ÁÁ¤ÏÏÂÈ ‚·ÚȤ˜ Î·È ÂÈΛӉ˘Ó˜ ÁÈ· ÙËÓ ˙ˆ‹ ÙˆÓ ·ÚÚÒÛÙˆÓ ·ÓÙȉڿÛÂȘ. ™ÙȘ ÂÚÈÙÒÛÂȘ ·˘Ù¤˜ ÂȯÂÈÚÂ›Ù·È ·¢·ÈÛıËÙÔÔ›ËÛË ÙˆÓ ·ÛıÂÓÒÓ ÌÂ Û˘Ó¯Ҙ ·˘Í·ÓfiÌÂÓ˜ ‰fiÛÂȘ ¯ÔÚËÁÔ‡ÌÂÓ˜ ÂÓ‰Ô‰ÂÚÌÈο, ˘Ô‰fiÚÈ· Î·È ÙÂÏÈο ÂÓ‰ÔÊϤ‚È·. ªÂ ÙÔÓ ÙÚfiÔ ·˘Ùfi, ÛÙËÓ ÏÂÈÔÓfiÙËÙ· ÙˆÓ ÂÚÈÙÒÛÂˆÓ Á›ÓÂÙ·È Î·ÏÒ˜ ·ÓÂÎÙ‹ Ë ÂÚ·ÈÙ¤Úˆ ¯ÔÚ‹ÁËÛË ÙˆÓ ALG/ATG. ∏ ÔÚÔÓÔÛ›· ÔÊ›ÏÂÙ·È ÛÙËÓ ·Ú·ÁˆÁ‹ ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙÚÂÊfiÌÂÓˆÓ Î·Ù¿ ÙˆÓ ›ÂÈˆÓ ÚˆÙÂ˚ÓÒÓ Î·È ÂÌÊ·Ó›˙ÂÙ·È Û¯Â‰fiÓ Û fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Ô˘ Ï·Ì‚¿ÓÔ˘Ó ATG. ™˘Ó‹ıˆ˜ ÂÌÊ·Ó›˙ÂÙ·È 5-11 Ë̤Ú˜ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Î·È ‰È·ÚΛ ·fi Ï›Á˜ Ë̤Ú˜ ̤¯ÚÈ, Û·ÓÈfiÙÂÚ·, ÌÂÚÈΤ˜ ‚‰ÔÌ¿‰Â˜. ∆· ÚÔ¤¯ÔÓÙ· Û˘ÌÙÒÌ·Ù· Â›Ó·È ˘ÚÂÙfi˜, ÎfiˆÛË, ÎËÏȉԂϷÙȉ҉˜ ÂÍ¿ÓıËÌ·, ·ÚıÚ·ÏÁ›· Î·È Ì˘·ÏÁ›·. ¶·ıÔÁÓˆÌÔÓÈÎfi ‡ÚËÌ· Û˘ÓÈÛÙ¿ ÙÔ ÂÚËÙÔÂȉ¤˜ ÂÍ¿ÓıËÌ· ÙˆÓ ¿ÎÚˆÓ ¯ÂÈÚÒÓ Î·È Ô‰ÒÓ. ¶ÚÔ˜ ·ÔÊ˘Á‹ Ù˘ ÔÚÔÓÔÛ›·˜ ¯ÔÚËÁ›ٷÈ, ·Ú¿ÏÏËÏ· Ì ÙÔÓ ›ÂÈÔ ÔÚfi, ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfiÓË ¯ˆÚ›˜, fï˜, Ó· Â›Ó·È ·ԉ‰ÂÈÁ̤ÓÔ˜ Ô Â˘ÂÚÁÂÙÈÎfi˜ ÚfiÏÔ˜ Ù˘, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Ë ÔÚÔÓÔÛ›· Â›Ó·È ÓfiÛÔ˜ ·˘ÙÔÂÚÈÔÚÈ˙fiÌÂÓË. ∏ ÙÒÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ÎÔÎÎÈÔ΢ÙÙ¿ÚˆÓ Î·È ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ηٿ ÙÔ ¿ÌÂÛÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ¯ÔÚ‹ÁËÛ˘ ÙˆÓ ALG/ATG Â›Ó·È Ôχ Û˘¯Ófi Î·È ·ÚÔ‰ÈÎfi Ê·ÈÓfiÌÂÓÔ. ¶Èı·ÓÒ˜ Ó· ÔÊ›ÏÂÙ·È ÛÙËÓ ÚÔÛÎfiÏÏËÛË ÙˆÓ ›ÂÈˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ Â› ÙˆÓ Î˘ÙÙ·ÚÈÎÒÓ ÌÂÌ‚Ú·ÓÒÓ, ¯ˆÚ›˜ ¿ÓÙˆ˜ Ó· ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ ÔÈ ALG/ATG η٤¯Ô˘Ó ÂȉÈ΋ ·ÓÔÛÔÏÔÁÈ΋ ·ÓÙÈÁÔÓÈÎfiÙËÙ·. ∏ ¯ÔÚ‹ÁËÛË ÙˆÓ ALG/ATG Û ·ÛıÂÓ›˜ Ì ∞∞ ¤¯ÂÈ ÈηÓÔÔÈËÙÈο ·ÔÙÂϤÛÌ·Ù·. ∏ ·¿ÓÙËÛË ÛÙË ıÂÚ·›·, Û˘¯Ó¿, Â›Ó·È Î·ı˘ÛÙÂÚË̤ÓË Î·È ÔÏϤ˜ ÊÔÚ¤˜ ¯ÚÂÈ¿˙ÂÙ·È ·Ó·ÌÔÓ‹ ̤¯ÚÈ Î·È 4 Ì‹Ó˜ ÚÔÎÂÈ̤ÓÔ˘ Ó· ÂÎÙÈÌËı› ÙÔ ıÂÚ·¢ÙÈÎfi ·ÔÙ¤ÏÂÛÌ·. ™ÙÔ˘˜ ηÎÔ‡˜ ··ÓÙËÙ¤˜ ÌÔÚ› Ó· ÂȯÂÈÚËı› Ë ¯ÔÚ‹ÁËÛË Î·È ‰Â‡ÙÂÚÔ˘ ıÂÚ·¢ÙÈÎÔ‡ ·ÎÏÔ˘ Ì ATG Ì Èı·ÓfiÙËÙ· ·ÓÙ·fiÎÚÈÛ˘ Î˘Ì·ÈÓfiÌÂÓË ÌÂٷ͇ 50 Î·È 63%. ™ÙÔ˘˜ ÙÂÏÂ˘Ù·›Ô˘˜ ‰ÂÓ Û˘ÓÈÛÙ¿Ù·È Ë ·ÏÏÔÁÂÓ‹˜ ¤Á¯˘ÛË ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·fi Û˘Ì‚·Ùfi ÌË Û˘ÁÁÂÓ‹ ‰fiÙË, ‰Â‰Ô̤ÓÔ˘ fiÙÈ ˘¿Ú¯ÂÈ ˘„ËÏfi ÔÛÔÛÙfi ·ÔÙ˘¯›·˜, ÔÊÂÈÏfiÌÂÓÔ Û ·fiÚÚÈ„Ë ÙÔ˘ ÌÔۯ‡̷ÙÔ˜, ÓfiÛÔ ÌÔۯ‡̷ÙÔ˜ ηٿ ÍÂÓÈÛÙ‹ Î·È Û ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ. ∆Ô Û˘ÓÔÏÈÎfi ÔÛÔÛÙfi ·¿ÓÙËÛ˘ Î˘Ì·›ÓÂÙ·È ÌÂٷ͇ 50 Î·È 60% ÙˆÓ ·ÛıÂÓÒÓ, ÂÎ ÙˆÓ ÔÔ›ˆÓ ÙÔ 1/3 ÂÚ›Ô˘ ÂÌÊ·Ó›˙ÂÈ Ï‹ÚË Î·È Ù· 2/3 ÌÂÚÈ΋ ‡ÊÂÛË, fiÏÔÈ fï˜, ·Ô‰ÂÛ̇ÔÓÙ·È ·fi ÙȘ ÌÂÙ·ÁÁ›ÛÂȘ. ¶Ú¤ÂÈ Ó· ÙÔÓÈ-

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Ûı›, fiÙÈ ‰˘ÛÌÂÓ›˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ‰Â›ÎÙ˜ ÁÈ· ÙËÓ ÙÂÏÈ΋ ·ÓÙ·fiÎÚÈÛË ·ÔÙÂÏÔ‡Ó Ô ·fiÏ˘ÙÔ˜ ·ÚÈıÌfi˜ Ô˘‰ÂÙÂÚfiÊÈÏˆÓ ÔÏ˘ÌÔÚÊÔ˘Ú‹ÓˆÓ οو ÙˆÓ 200/mm3, Ë ÛË„·ÈÌ›·, ÔÈ ·ÈÌÔÚÚ·ÁÈΤ˜ ÂΉËÏÒÛÂȘ Î·È ÙÔ ı‹Ï˘ ʇÏÔ. ∏ CsA Â›Ó·È ¤Ó· ΢ÎÏÈÎfi ÂÓÙÂηÂÙ›‰ÈÔ ·Ú·ÁfiÌÂÓÔ ·fi ̇ÎËÙ˜, ÙÔ ÔÔ›Ô ÂÌÏÔ‡ÙÈÛ ÙË ıÂÚ·¢ÙÈ΋ Ê·Ú¤ÙÚ· ηٿ Ù˘ ∞∞ ÙÔ 1984, fiÙ·Ó ÁÈ· ÚÒÙË ÊÔÚ¿ ¯ÚËÛÈÌÔÔÈ‹ıËÎÂ Û·Ó ·ÓÔÛÔηٷÛÙ·ÏÙÈÎfi Ê¿ÚÌ·ÎÔ Û ·ÛıÂÓ›˜ Ì ∞∞. ∏ ÂÊ·ÚÌÔÁ‹ Ù˘ CsA ÛÙËÚ›˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ ¯ˆÚ›˜ Ó· Â›Ó·È Î˘ÙÙ·ÚÔÙÔÍÈ΋, ·ÚÂÌÔ‰›˙ÂÈ ÂÎÏÂÎÙÈο ÙËÓ ÂÓÂÚÁÔÔ›ËÛË Î·È ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ ∆-΢ÙÙ·ÚÔÙÔÍÈÎÒÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ. °È· ÙËÓ Â›Ù¢ÍË Ù˘ ‰Ú¿Û˘ Ù˘ Û˘Ó‰¤ÂÙ·È Ì ΢ÙÙ·ÚÈÎÔ‡˜ ˘ԉԯ›˜ (immunophilins), ÙˆÓ ÔÔ›ˆÓ Ô ·ÎÚÈ‚‹˜ ÚfiÏÔ˜ ÛÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ÂÍ·ÎÚÈ‚ˆÌ¤ÓÔ˜. ¶·Ú¿ÏÏËÏ·, ·ÚÂÌÔ‰›˙ÂÈ ÙÔÓ Û¯ËÌ·ÙÈÛÌfi Ù˘ ÈÓÙÂÚÏ¢ΛÓ˘-2 Î·È ÙˆÓ ˘Ô‰Ô¯¤ˆÓ Ù˘ ηıÒ˜ Î·È Ù˘ ÈÓÙÂÚÊÂÚfiÓ˘-Á, ·Ó·ÛÙ¤ÏÏÔÓÙ·˜ ÙËÓ ·ÓÔÛÔÚÔÎÏËÙ‹ ηٷÛÙÚÔÊ‹ ÙˆÓ SC Î·È ÂÈÙÚ¤ÔÓÙ·˜ ÙËÓ ·ÔηٿÛÙ·ÛË Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ·ÈÌÔÔ›ËÛ˘. ∆Ô Ê¿ÚÌ·ÎÔ ¯ÔÚËÁÂ›Ù·È ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜, Ë ‰fiÛË ÙÔ˘ Â›Ó·È 10-12 mg/Kg/24ˆÚÔ ¯ÔÚËÁÔ˘Ì¤ÓË ·Ó¿ 12ˆÚÔ ÁÈ· ‰È¿ÛÙËÌ· ÙÔ˘Ï¿¯ÈÛÙÔÓ 6 ÌËÓÒÓ. ¶ÚÔ˜ ·ÔÊ˘Á‹Ó ·ÓÂÈı‡ÌËÙˆÓ ÂÓÂÚÁÂÈÒÓ Î·È ÌË ·Ó·ÛÙÚ¤„ÈÌˆÓ ‚Ï·‚ÒÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ¯ÔÚ‹ÁËÛ˘, Ú¤ÂÈ Û ٷÎÙ¿ ¯ÚÔÓÈο ‰È·ÛÙ‹Ì·Ù· Ó· ÂϤÁ¯ÔÓÙ·È Ù· Â›‰· ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ÛÙÔ ·›Ì·, Ù· ÔÔ›· Ú¤ÂÈ Ó· Î˘Ì·›ÓÔÓÙ·È ÌÂٷ͇ 200 Î·È 400 ng/mL. ∞Ó¿ÏÔÁ· Ì ٷ Â›‰· ·˘ÍÔÌÔÈÒÓÔÓÙ·È ÔÈ ¯ÔÚËÁÔ‡ÌÂÓ˜ ‰fiÛÂȘ, ÂÓÒ ·Ú¿ÏÏËÏ· ̤ۈ ‚ÈÔ¯ËÌÈÎÒÓ ‰ÂÈÎÙÒÓ ÂϤÁ¯ÂÙ·È Ë ÓÂÊÚÈ΋ ÏÂÈÙÔ˘ÚÁ›·. ∏ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ Ê·›ÓÂÙ·È ÌÂÙ¿ ·fi 3-6 Ì‹Ó˜ ¯ÔÚ‹ÁËÛ‹˜ ÙÔ˘, ÂÓÒ Û ÔÚÈṲ̂ÓÔ˘˜ ·ÛıÂÓ›˜ ÂÌÊ·Ó›˙ÂÙ·È ‚ÂÏÙ›ˆÛË Ù˘ ·ÈÌÔÔ›ËÛ˘ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ ÙÔ˘. ¢ÂÓ Û˘ÓÈÛÙ¿Ù·È Ë ¯ÔÚ‹ÁËÛË Ù˘ CsA Û·Ó ÚÒÙ˘ ÂÈÏÔÁ‹˜ ıÂÚ·¢ÙÈÎfi Û¯‹Ì· Î·È Î·Ù¿ ηÓfiÓ· ¯ÔÚËÁÂ›Ù·È ÌÂÙ¿ ·fi ·ÔÙ˘¯›· ÙˆÓ ALG/ATG ‹, ÙÔ Û˘ÓËı¤ÛÙÂÚÔ, ÂÓÙ¿ÛÛÂÙ·È Û ÚˆÙfiÎÔÏÏ· ÂÓÙ·ÙÈ΋˜ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜. ™Â Â·ÓÂÌÊ¿ÓÈÛË Ù˘ ∞∞ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘, ÌÔÚ› Ó· Â·Ó·¯ÔÚËÁËı› ÛÙȘ ·Ú¯ÈΤ˜ ‰fiÛÂȘ ‹ Î·È Ó· ··ÈÙËı› Ì·ÎÚfi¯ÚÔÓË Ï‹„Ë Ù˘ CsA Û ‰fiÛÂȘ Û˘ÓÙ‹ÚËÛ˘. ∏ ÙÔÍÈÎfiÙËÙ· Ù˘ CsA ‰ÂÓ Â›Ó·È ·ÌÂÏËÙ¤·. √È Û˘Ó‹ıÂȘ ·ÚÂÓ¤ÚÁÂȘ ·ÊÔÚÔ‡Ó Û ÂÌÊ¿ÓÈÛË ˘¤ÚÙ·Û˘, Ô˘Ú·ÈÌ›·˜, ˘ÂÚÙÚ›¯ˆÛ˘ Î·È ˘ÂÚÙÚÔÊ›·˜ ÙˆÓ Ô‡ÏˆÓ. ∏ ¿ÓÔ‰Ô˜ Ù˘ ÙÈÌ‹˜ Ù˘ ÎÚ·ÙÈÓ›Ó˘ ÙÔ˘ ÔÚÔ‡ ·ÔÙÂÏ› ·fiÏ˘ÙË ¤Ó‰ÂÈÍË Ì›ˆÛ˘ ÙˆÓ ¯ÔÚËÁÔ˘Ì¤ÓˆÓ ‰fiÛˆÓ, ηıfiÛÔÓ Â›Ó·È È‰È·›ÙÂÚ· ÓÂÊÚÔÙÔÍÈÎfi Ê¿ÚÌ·ÎÔ ÎÈ ÂÎÙfi˜ ·fi ÔÍ›·, ÌÔÚ› Ó· ÚÔηϤÛÂÈ Î·È ¯ÚfiÓÈ· ÌË ·Ó·ÛÙÚ¤„ÈÌË ‚Ï¿‚Ë, ¯·Ú·ÎÙËÚÈ˙fiÌÂÓË ·fi ‰È¿ÌÂÛË ›ÓˆÛË Î·È ·ÙÚÔÊ›· ÙˆÓ ÓÂÊÚÈÎÒÓ ÛˆÏËÓ·Ú›ˆÓ. ∂È‚·Ú˘ÓÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË ÓÂÊÚÈ-


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 43

¶·È‰È·ÙÚÈ΋ 2001;64:39-44

Paediatriki 2001;64:39-44

΋˜ ‚Ï¿‚˘ ·ÔÙÂÏÔ‡Ó ÔÈ ˘„ËϤ˜ ‰fiÛÂȘ Ù˘ CsA, Ë Ì·ÎÚfi¯ÚÔÓË ¯ÔÚ‹ÁËÛ‹ Ù˘ Î·È Ë ÌÂÁ¿ÏË ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ (ÔÈ ÂÓ‹ÏÈΘ ·ÛıÂÓ›˜ Â›Ó·È ÈÔ ÂÈÚÚÂ›˜ ·fi Ù· ·È‰È¿ ÛÙËÓ ÂÌÊ¿ÓÈÛË Ù¤ÙÔÈˆÓ ‚Ï·‚ÒÓ). √ Û˘Ó‰˘·ÛÌfi˜ Ù˘ CsA Ì ÎÔÚÙÈÎÔÂȉ‹ Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÚÔηϤÛÂÈ ·ÚÔ‰È΋ ·ÓÔÛÔ·ÓÂ¿ÚÎÂÈ· Ì ·ÔÙ¤ÏÂÛÌ· ÔÈ ¿Û¯ÔÓÙ˜ Ó· Â›Ó·È Â˘·›ÛıËÙÔÈ Û ÔÈΛÏÔ˘˜ ÏÔÈÌÔÁfiÓÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÛÙÔ˘˜ ÔÔ›Ô˘˜ Û˘ÌÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÙÔ Ì˘ÎÔ‚·ÎÙËÚ›‰ÈÔ Ù˘ Ê˘Ì·Ù›ˆÛ˘ Î·È Ë Ó¢ÌÔÓÔ·ÛÙË carinii. ™Ô‚·Ú‹ ·ÚÂÓ¤ÚÁÂÈ· Ù˘ ıÂÚ·›·˜ Ì CsA Â›Ó·È Ù· ÂÂÈÛfi‰È· Û·ÛÌÒÓ. ∂Ó›ÔÙ ‰Â, Ë ·Ú·ÙËÚÔ‡ÌÂÓË ÏfiÁˆ ÓÂÊÚÈ΋˜ ‚Ï¿‚˘ ˘ÔÌ·ÁÓËÛÈ·ÈÌ›·, Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ÌÂÈÒÛÂÈ ÙÔÓ Ô˘‰fi Ì ·ÔÙ¤ÏÂÛÌ· Ó· ·˘Í¿ÓÂÙ·È Î·È Ë Èı·ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ Û·ÛÌÒÓ. ∞fi ÙȘ ·Ú¯¤˜ Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ ’90 Á›ÓÂÙ·È ÚÔÛ¿ıÂÈ· ÂÚ·ÈÙ¤Úˆ ‚ÂÏÙ›ˆÛ˘ ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Ù˘ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜ ̤ۈ ‰ËÌÈÔ˘ÚÁ›·˜ ÚˆÙÔÎfiÏψÓ, Ù· ÔÔ›· ÂÌÂÚȤ¯Ô˘Ó Û˘Ó‰˘·ÛÌÔ‡˜ ÙˆÓ ·ÓˆÙ¤Úˆ Û΢·ÛÌ¿ÙˆÓ (ÂÓÙ·ÙÈ΋ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹). √È ÏfiÁÔÈ Ô˘ Ô‰‹ÁËÛ·Ó Û ·˘Ù‹ ÙËÓ ÚÔÛ¿ıÂÈ· ‹Ù·Ó ·Ê’ ÂÓfi˜ Ë ‰˘ÛÎÔÏ›· ·Ó‡ÚÂÛ˘ Ï‹Úˆ˜ Û˘ÁÁÂÓÔ‡˜ Û˘Ì‚·ÙÔ‡ ‰fiÙË Î·È ·Ê’ ÂÙ¤ÚÔ˘ Ë ıÂÚ·¢ÙÈ΋ Î¿Ï˘„Ë ÙˆÓ ÌÂÁ¿Ï˘ ËÏÈΛ·˜ ·ÛıÂÓÒÓ, ÔÈ ÔÔ›ÔÈ ‰ÂÓ Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ˘Ô‚ÏËıÔ‡Ó ÛÙË ‰È·‰Èηۛ· Ù˘ ·ÏÏÔÁÂÓÔ‡˜ ¤Á¯˘Û˘ ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ∆· ÏÂÔÓÂÎÙ‹Ì·Ù· Ù˘ ¤ÓÙÔÓ˘ ıÂÚ·›·˜, Ë ÔÔ›· ‰Â›¯ÓÂÈ Ó· ¤¯ÂÈ Û¯Â‰fiÓ ‰ÈÏ·ÛÈ¿ÛÂÈ ÙËÓ ·ÓÙ·fiÎÚÈÛË ÙˆÓ ·ÛıÂÓÒÓ, ·ÊÔÚÔ‡Ó Î˘Ú›ˆ˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ‚·ÚÈ¿ ‹ Î·È Ôχ ‚·ÚÈ¿ ∞∞ Î·È Ê·›ÓÂÙ·È fiÙÈ Ôχ ÛËÌ·ÓÙÈ΋ ‚Ô‹ıÂÈ· ÛÙËÓ Â›Ù¢ÍË ÙÔ˘ ÛÙfi¯Ô˘ ·˘ÙÔ‡

¤‰ˆÛÂ Ë ¤ÓÙ·ÍË Ù˘ CsA ÛÙ· ÚˆÙfiÎÔÏÏ· ·˘Ù¿. ∏ ıˆÚËÙÈ΋ ‚¿ÛË Ù˘ ÂÓÙ·ÙÈÎÔÔÈË̤Ó˘ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜ Â›Ó·È fiÙÈ ÛÙԯ‡ÂÈ ÛÙËÓ ·ıÚÔÈÛÙÈ΋ ‹ Î·È Û˘ÓÂÚÁÈ΋ ‰Ú¿ÛË ÙˆÓ ÂÈ̤ÚÔ˘˜ Ê·ÚÌ¿ÎˆÓ Î·È Ë ÔÔ›· ÂÈÙ˘Á¯¿ÓÂÙ·È Ì¤Ûˆ: Û˘Ó‰˘·Ṳ̂Ó˘ ‰Ú¿Û˘ Â› ÂÓfi˜ ·ıÔÁÂÓÂÙÈÎÔ‡ Ì˯·ÓÈÛÌÔ‡ ‹ ‰È·ÊÔÚÂÙÈ΋˜ ‰Ú¿Û˘ ÙˆÓ ·ÓÔÛÔηٷÛÙ·ÏÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Â› ÂÚÈÛÛÔÙ¤ÚˆÓ ÙÔ˘ ÂÓfi˜ ·ıÔÁÂÓÂÙÈÎÒÓ Ì˯·ÓÈÛÌÒÓ. Ÿˆ˜ ÚÔ·ÙÂÈ ·fi ÙȘ ̤¯ÚÈ Û‹ÌÂÚ· ÌÂϤÙ˜, Ë Û˘Ó‰˘·Ṳ̂ÓË ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹ ÌÔÚ› Ó· ÂÙ‡¯ÂÈ Î·Ï¿ ·ÔÙÂϤÛÌ·Ù· Ì ÌÈÎÚfiÙÂÚ˜ ‰fiÛÂȘ ÙˆÓ Ê·Ú̿ΈÓ, Ì ¿ÌÂÛÔ ÛÙfi¯Ô ÙËÓ Èı·Ó‹ Ì›ˆÛË Ù˘ ÙÔÍÈÎfiÙËÙ·˜. ŒÙÛÈ Û‹ÌÂÚ·, Â›Ó·È Û ÂͤÏÈÍË ıÂÚ·›˜ ÌÂ Û˘Ó‰˘·ÛÌÔ‡˜ ÙˆÓ ·ÓˆÙ¤Úˆ ·ÓÔÛÔηٷÛÙ·ÏÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, Û ÔÚÈṲ̂Ó˜ ÙˆÓ ÔÔ›ˆÓ ÚԂϤÂÙ·È Î·È Ë ÚÔÛı‹ÎË ·˘ÍËÙÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ∞ÔÙÂÏÂÛÌ·ÙÈÎfi˜ ‰Â›¯ÓÂÈ Ó· Â›Ó·È Ô Û˘Ó‰˘·ÛÌfi˜ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ALG, CsA Î·È ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfiÓË (¶›Ó·Î·˜ 1). ŸÛÔÓ ·ÊÔÚ¿ ‰Â, ÙËÓ Â·ÓÂÌÊ¿ÓÈÛË Ù˘ ∞∞, Ù˘ ÔÔ›·˜ Ë Èı·ÓfiÙËÙ· ÛÙ· 14 ¤ÙË ·Ú·ÎÔÏÔ‡ıËÛ˘ ÍÂÂÚÓ¿ ÙÔ 35%, ÌÂÙ¿ ÙËÓ ÚÒÙË Ï‹ÚË ‹ ÌÂÚÈ΋ ‡ÊÂÛË, Ë ·ÓÙÈÌÂÙÒÈÛ‹ Ù˘ ÔÈΛÏÏÂÈ Î·È ÂÍ·ÚÙ¿Ù·È Î˘Ú›ˆ˜ ·fi ÙËÓ ÂÌÂÈÚ›· Î·È ÙȘ ‰˘Ó·ÙfiÙËÙ˜ ÙÔ˘ ıÂÚ·¢ÙÈÎÔ‡ ΤÓÙÚÔ˘. ™Â ÁÂÓÈΤ˜, ¿ÓÙˆ˜, ÁÚ·Ì̤˜ Â›Ó·È ‰˘Ó·ÙfiÓ Ó· ‰ÔÎÈÌ·ÛıÔ‡Ó Ù· οوıÈ: ¢Â‡ÙÂÚÔ˜ ·ÎÏÔ˜ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜ Ì Èı·ÓfiÙËÙ· ·ÓÙ·fiÎÚÈÛ˘ ÂÚ› ÙÔ 50% ªÂÌÔӈ̤ÓË ¯ÔÚ‹ÁËÛË CsA, CP Ì ‹ ¯ˆÚ›˜

¶›Ó·Î·˜ 1. ∏ ‰È·¯ÚÔÓÈ΋ ÂͤÏÈÍË ÛÙË ıÂÚ·›· Î·È ÂÈ‚›ˆÛË ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ·ÛıÂÓÒÓ Ì ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· Î·È ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹. ™˘ÁÁÚ·Ê›˜

ŒÙË

∞ÚÈıÌfi˜ ·ÛıÂÓÒÓ

£ÂÚ·›·

∂È‚›ˆÛË (%)

ÃÚfiÓÔ˜ ·Ú·ÎÔÏÔ‡ıËÛ˘

Cairo MS et al

¶ÚÔ ÙÔ˘ 1982

23

ATG+PDN

48

12 Ì‹Ó˜

Bayever E et al

1977 – 1982

22

ATG+PDN

45

2 ¤ÙË

Warner EJ et al

1981 – 1986

9

ATG+PDN+CsA (2)

88

2 ¤ÙË

Halpérin DS et al

1977 – 1987

12

hATG ‹ rATG+MePDN

42

15 Ì‹Ó˜

Locasciulli A at al

1970 – 1988

133

ALG+PDN+AÓ‰ÚÔÁfiÓ·

48

10 ¤ÙË

Weble DKH et al

1974 – 1988

18

ALG+√͢ÌÂıÔÏfiÓË

30

5 ¤ÙË

Matloub Y et al

1990 – 1993

22

ATG+PDN+CsA

80

43 Ì‹Ó˜

Lawlor ER et al

1982 – 1994

27

ATG+MePDN+CsA

92

33,5 Ì‹Ó˜

Pitcher IA et al

1989 – 1996

25

ATG+CsA+MePDN

86

8 ¤ÙË

Fürher M et al

1993 – 1997

114

ALG+CsA+G-CSF

54 (EFS)

4 ¤ÙË

Kojima S et al

1992 - 1997

111

ALG+CsA+Danazole 87

5 ¤ÙË

+G-CSF

43


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 44

¶·È‰È·ÙÚÈ΋ 2001;64:39-44

·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÀÔÛÙËÚÈÎÙÈ΋ ·ÁˆÁ‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ‹ ¯ˆÚ›˜ ·Ó‰ÚÔÁfiÓ· ∞ÏÏÔÁÂÓ‹˜ ¤Á¯˘ÛË ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·fi Û˘Ì‚·Ùfi ÌË Û˘ÁÁÂÓ‹ ‰fiÙË. ∆Ô ÌÂÁ¿ÏÔ ÌÂÈÔÓ¤ÎÙËÌ· Ù˘ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜, ÂÓÙ·ÙÈ΋˜ ‹ ÌË, Â›Ó·È fiÙÈ ·ÔηıÈÛÙ¿ ÙËÓ ·ÈÌÔÔ›ËÛË ¯ˆÚ›˜, fï˜, Ó· ·ÓÙÈηıÈÛÙ¿ Ù· ·ıÔÏÔÁÈο SC. ∆Ô‡ÙÔ Ú·ÎÙÈο ηٷϋÁÂÈ ÛÙËÓ ÌÂÚÈ΋ ·ÔηٿÛÙ·ÛË ÙˆÓ ·ÈÌ·ÙÔÏÔÁÈÎÒÓ ·Ú·Ì¤ÙÚˆÓ Û ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ·ÛıÂÓÒÓ, ·ÚfiÙÈ, ‚‚·›ˆ˜, Â›Ó·È ÂχıÂÚÔÈ ÌÂÙ·ÁÁ›ÛˆÓ. ¢Â‡ÙÂÚË ÛËÌ·ÓÙÈ΋ Û˘Ó¤ÂÈ· ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∞∞ Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó Ì ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹, Â›Ó·È Ô ·˘ÍË̤ÓÔ˜ ΛӉ˘ÓÔ˜ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ÁÈ· ÎψÓÈÎÔ‡ Ù‡Ô˘ ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· Î·È Û˘ÁÎÂÎÚÈ̤ӷ Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ, ÔÍ›· Ì˘ÂÏÔÁÂÓ‹ Ï¢¯·ÈÌ›· Î·È ·ÚÔ͢ÛÌÈ΋ Ó˘ÎÙÂÚÈÓ‹ ·ÈÌÔÛÊ·ÈÚÈÓÔ˘Ú›·. ∆Ô ÔÛÔÛÙfi ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó Ù¤ÙÔÈÔ˘ Ù‡Ô˘ ·ÈÌ·ÙÔÏÔÁÈΤ˜ ‰È·Ù·Ú·¯¤˜ ÁÈ· ÌÂÓ ÙÔ˘˜ ÂÓ‹ÏÈΘ ·ÛıÂÓ›˜ ·Ó¤Ú¯ÂÙ·È ÛÙ· 10 ¤ÙË ÛÙÔ 30-50%, ÂÓÒ ÛÙ· ·È‰È¿ ÙÔ ÔÛÔÛÙfi Â›Ó·È ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚÔ Î˘Ì·ÈÓfiÌÂÓÔ ÂÚ› ÙÔ 10% ÛÙÔ ›‰ÈÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ·Ú·ÎÔÏÔ‡ıËÛ˘. √ÏÔÎÏËÚÒÓÔÓÙ·˜ ÙË Û‡ÓÙÔÌË ·˘Ù‹ ·Ó·ÊÔÚ¿ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ‚·ÚÈ¿˜ ·Ï·ÛÙÈ΋˜ ·Ó·ÈÌ›·˜ Ì ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹, ı· Ú¤ÂÈ Û˘ÌÂÚ·ÛÌ·ÙÈο Ó· ˘ÔÁÚ·Ì̛ۈ fiÙÈ: ∏ ıÂÚ·›· ÂÎÏÔÁ‹˜ ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ‚·ÚÈ¿ ∞∞ ‹Ù·Ó Î·È ·Ú·Ì¤ÓÂÈ Ë ·ÏÏÔÁÂÓ‹˜ ¤Á¯˘ÛË ÚÔÁÔÓÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ™ÙËÓ ÂÚ›ÙˆÛË Ô˘ ‰ÂÓ ˘¿Ú¯ÂÈ Û˘Ì‚·Ùfi˜ Û˘ÁÁÂÓ‹˜ ‰fiÙ˘, ÂȯÂÈÚÂ›Ù·È Ë ¯ÔÚ‹ÁËÛË ÂÓÙ·ÙÈ΋˜ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ·ÁˆÁ‹˜ Î·È Ë ÔÔ›· ηٿ ηÓfiÓ· ÛÙËÚ›˙ÂÙ·È ÛÙË Û˘Ó‰˘·Ṳ̂ÓË ¯ÔÚ‹ÁËÛË ALG/CsA/ ÌÂı˘ÏÚ‰ÓÈ˙ÔÏfiÓ˘. µÈ‚ÏÈÔÁÚ·Ê›· 1. Young NS. Aplastic anaemia. Lancet 1995;346:228-232. 2. Definitive treatment of acquired aplastic anemia. In: Young

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NS, Alter BP, eds. Aplastic anemia acquired and inherited. 1st edition. Philadelphia: WB Saunders; 1994. p. 159-200. Camitta BM, Storb R, Thomas ED. Aplastic Anemia: pathogenesis, diagnosis, treatment and prognosis. N Engl J Med 1982;306:645-652. Brodsky RA, Sensenbrenner LL, Jones RJ. Complete remission in severe aplastic anemia after high-dose cyclophosphamide without bone marrow transplantation. Blood 1996;87:491-494. Werner EJ, Stout RD, Valdez LP, Harris RE. Immunosuppressive therapy versus bone marrow transplantation for children with aplastic anemia. Pediatrics 1989;83:61-65. Halperin DS, Grisaru D, Freedman MH, Saunders EF. Severe acquired aplastic anemia in children: 11-year experience with bone marrow transplantation and immunosuppressive therapy. Am J Pediatr Hematol Oncol 1989;11:304-309. Bayever E, Champlin R, Ho W, Lenarsky C, Storch S, Ladiscch S et al. Comparison between bone marrow trasplantation and antithymocyte globulin in treatment of young patients with severe aplastic anemia. J Pediatr 1984;105:920-925. Cairo MS, Baehner RL. The use of antithymocyte globulin in the treatment of severe aplastic anemia in children. J Pediatr 1982;100:307-311. Bielory L, Wright L, Nienhuis AW, Young NS. Antithymocyte globulin hypersensitivity in bone marrow failure patients. JAMA 1988;260:3164-3167. Bacigalupo A, Broccia G, Corda G, Arcese W, Carotenuto M, Gallamini A et al. Antilymphocyte globulin, cyclosporin, and granulocyte colony-stimulating factor in patients with acquired severe aplastic anemia (SAA); A pilot study of the EBMT SAA Working Party. Blood 1995;85:1348-1353.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 02-10-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 21-11-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: π.¶. ¶·Ó·ÁÈÒÙÔ˘ °ÂÓÓ·‰›Ô˘ 1, 184-52 ¡›Î·È·


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 45

¶·È‰È·ÙÚÈ΋ 2001;64:45-48

™À¡∂Ãπ∑√ª∂¡∏ ¶∞π¢π∞∆ƒπ∫∏ ∂∫¶∞π¢∂À™∏

Paediatriki 2001;64:45-48

CONTINUING MEDICAL EDUCATION

ªÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (ªª√) Û ·È‰È¿ Ì ۇӉÚÔÌ· Ì˘ÂÏÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜ ¢. ¶ÂÙÚfiÔ˘ÏÔ˜

Bone marrow transplantation in children with bone marrow failure syndromes D. Petropoulos

¶ÂÚ›ÏË„Ë: ∏ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (ªª√) ·ÔÙÂÏ› ıÂÚ·›· ÂÎÏÔÁ‹˜ ÁÈ· ·È‰È¿ Ì ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, fiÙ·Ó ˘¿Ú¯ÂÈ HLA-Û˘Ì‚·Ùfi˜ ·‰ÂÏÊfi˜ ‰fiÙ˘. ∏ ¤Î‚·ÛË Ù˘ ·ÏÏÔÁÂÓÔ‡˜ ªª√ ¤¯ÂÈ ÛËÌ·ÓÙÈο ‚ÂÏÙȈı› ηٿ ÙËÓ ÙÂÏÂ˘Ù·›· ÂÈÎÔÛ·ÂÙ›· Î·È ÙÔ ÔÛÔÛÙfi Ì·ÎÚ¿˜ ÂÈ‚›ˆÛ˘ ÙˆÓ ·ÛıÂÓÒÓ ÚÔÛÂÁÁ›˙ÂÈ ÙÔ 90%. √È Î˘ÚÈfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ¤¯Ô˘Ó Û˘ÓÙÂϤÛÂÈ Û ·˘Ù‹ ÙËÓ ÚfiÔ‰Ô Â›Ó·È Ë ÌÂÙ·ÌfiÛ¯Â˘ÛË Û ÚÒÈÌË Ê¿ÛË Ù˘ ÓfiÛÔ˘, Ë ÚÔÛı‹ÎË ·ÓÙÈı˘ÌÔ΢ÙÙ·ÚÈ΋˜ ÛÊ·ÈÚ›Ó˘ ÛÙÔ Û¯‹Ì· ÚÔÂÙÔÈÌ·Û›·˜ Î·È Ë ¯Ú‹ÛË Ù˘ ΢ÎÏÔÛÔÚ›Ó˘ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÌÈÎÚ¤˜ ‰fiÛÂȘ ÌÂıÔÙÚÂÍ¿Ù˘ ÁÈ· ÙËÓ ÚfiÏË„Ë ÙÔ˘ GVHD. √È ÌÂÙ·ÌÔۯ‡ÛÂȘ ·fi Û˘Ì‚·ÙÔ‡˜ ÌË Û˘ÁÁÂÓ›˜ ‹ ÌË ·fiÏ˘Ù· Û˘Ì‚·ÙÔ‡˜ Û˘ÁÁÂÓ›˜ ‰fiÙ˜, Â›Ó·È ÏÈÁfiÙÂÚÔ ÂÈÙ˘¯Â›˜ ÎÈ ¤¯Ô˘Ó ¤Ó‰ÂÈÍË ÌfiÓÔ Û ·ÛıÂÓ›˜ Ô˘ ‰ÂÓ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹. ∏ ·ÏÏÔÁÂÓ‹˜ ªª√ ¤¯ÂÈ Â›Û˘, ηıÈÂÚˆı› ˆ˜ ıÂÚ·›· Û ·ÛıÂÓ›˜ Ì ·Ó·ÈÌ›· Fanconi, Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· ‹ ΢ÙÙ·ÚÔÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ Ô˘ ÚÔÌËÓ‡Ô˘Ó ÂÍ·ÏÏ·Á‹ ÛÂ Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ ‹ ÔÍ›· Ï¢¯·ÈÌ›·. ÕÏÏ· Û¿ÓÈ· Û‡Ó‰ÚÔÌ· Ì˘ÂÏÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜ Ô˘ ¤¯Ô˘Ó ·ÓÙÈÌÂÙˆÈÛı› ÂÈÙ˘¯Ò˜ Ì ·ÏÏÔÁÂÓ‹ ªª√, Â›Ó·È Ë ·Ó·ÈÌ›· Diamond-Blackfan Ì ·ÓÙÔ¯‹ ÛÙ· ÛÙÂÚÔÂȉ‹, ÙÔ Û‡Ó‰ÚÔÌÔ Schwachman-Diamond, Ë ·ÌÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÈ΋ ıÚÔÌ‚ÔÂÓ›· Î·È ÙÔ Û‡Ó‰ÚÔÌÔ Kostmann.

Abstract: Bone marrow transplantation (BMT) is the treatment of choice for children with severe aplastic anemia when an HLA-compatible sibling donor is available. The outcome of allogeneic transplantation has greatly improved over the last two decades with long-term survival approaching 90%. Factors that have contributed to this advance include transplantation earlier in the course of the disease, the addition of antithymocyte globulin in the conditioning regimen, and the use of cyclosporine in combination with shortdose methotrexate for GVHD prophylaxis. Transplants from matched unrelated or mismatched related donors is associated with an inferior outcome, and is indicated only in cases refractory to immuno- suppression. Allogeneic BMT is also an established treatment for patients with Fanconi anemia who develop aplastic anemia or cytogenetic abnormalities which are suggestive of impending myelodysplastic syndrome or acute leukemia. Other less common bone marrow failure syndromes, in which successful treatment with allogeneic BMT has been reported include steroidrefractory Diamond-Blackfan anemia, SchwachmanDiamond syndrome, amegakaryocytic thrombocytopenia, and Kostmann syndrome.

§¤ÍÂȘ-ÎÏÂȉȿ: Û‡Ó‰ÚÔÌ· Ì˘ÂÏÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜, ·ÏÏÔÁÂÓ‹˜ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ, ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, ·Ó·ÈÌ›· Fanconi, ·Ó·ÈÌ›· Diamond-Blackfan.

Key words: bone marrow failure syndromes, allogeneic bone marrow transplantation, aplastic anemia, Fanconi anemia, Diamond-Blackfan anemia.

∂ÈÌÂÏËÙ‹˜ µ’ ªÔÓ¿‰·˜ ªÂÙ·ÌfiÛ¯Â˘Û˘ ª˘ÂÏÔ‡ ÙˆÓ √ÛÙÒÓ

Register, 2nd Division of Bone Marrow Transplantation

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¶·È‰È·ÙÚÈ΋ 2001;64:45-48

∞Ï·ÛÙÈ΋ ·Ó·ÈÌ›· (∞∞) ∏ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·ÔÙÂÏ› ÙË ıÂÚ·›· ÂÎÏÔÁ‹˜ Û ·È‰È¿ Î·È Ó·ÚÔ‡˜ ·ÛıÂÓ›˜ Ì ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, fiÙ·Ó ˘¿Ú¯ÂÈ HLA-Û˘Ì‚·Ùfi˜ ·‰ÂÏÊfi˜ ‰fiÙ˘. √È ·Ú¯ÈΤ˜ ÌÂÙ·ÌÔۯ‡ÛÂȘ Û ·È‰È¿ Î·È ÂÓ‹ÏÈΘ Ì ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· ‰ÂÓ ‹Ù·Ó Û˘Ó‹ıˆ˜ ÂÈÙ˘¯Â›˜, ÏfiÁˆ ·ÓÂ¿ÚÎÂÈ·˜ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜, ÓfiÛÔ˘ ÌÔۯ‡̷ÙÔ˜ ηٿ ÙÔ˘ ÍÂÓÈÛÙ‹ (GVHD) Î·È ÌÂÁ¿Ï˘ ıÓËÙfiÙËÙ·˜ ·fi ÏÔÈÌÒÍÂȘ. ∏ ·ÓÂ¿ÚÎÂÈ· ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ ‰È·ÎÚ›ÓÂÙ·È ÛÂ: - ÚˆÙÔ·ı‹ (fiÙ·Ó ‰ÂÓ ·Ú·ÙËÚÂ›Ù·È Î·ıfiÏÔ˘ ·ÔηٿÛÙ·ÛË Ù˘ ·ÈÌÔÔ›ËÛ˘ ÌÂÙ¿ ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ) Î·È - ‰Â˘ÙÂÚÔ·ı‹ (fiÙ·Ó Û˘Ì‚·›ÓÂÈ ·Ú¯Èο, ÂÁηٿÛÙ·ÛË ÙÔ˘ ÌÔۯ‡̷ÙÔ˜, ·ÎÔÏÔ˘ı›ٷÈ, fï˜, ·fi ·fiÚÚÈ„Ë). Ÿˆ˜ ¤ÁÈÓ ·ÓÙÈÏËÙfi ·fi ÙȘ ÚÒÙ˜ ÌÂÙ·ÌÔۯ‡ÛÂȘ Û ·ÛıÂÓ›˜ Ì ∞∞, fiÙ·Ó ÙÔ Û¯‹Ì· ÚÔÂÙÔÈÌ·Û›·˜ ‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÂÈ ÈÛ¯˘Ú‹ ·ÓÔÛÔηٷÛÙÔÏ‹, ‰ÂÓ ÂÈÙ˘Á¯¿ÓÂÙ·È Û˘Ó‹ıˆ˜ ÂÁηٿÛÙ·ÛË ÙÔ˘ ÌÔۯ‡̷ÙÔ˜, ÁÂÁÔÓfi˜ Ô˘ Èı·ÓfiÓ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔÓ ˘ÔΛÌÂÓÔ ·ÓÔÛÔÏÔÁÈÎfi Ì˯·ÓÈÛÌfi ÙÔ˘ ÓÔÛ‹Ì·ÙÔ˜. ∏ ȉȷÈÙÂÚfiÙËÙ· Ù˘ ªª√ Û ∞∞ ¤ÁÈÓ ۷ʤÛÙÂÚË ·fi ÙËÓ ÂÌÂÈÚ›· ÌÂÙ·ÌÔۯ‡ÛÂˆÓ ·fi ÌÔÓÔ˙˘ÁˆÙÈÎÔ‡˜ ·‰ÂÏÊÔ‡˜ ‰fiÙ˜. £ÂˆÚËÙÈο, fiÙ·Ó Ô ‰fiÙ˘ Â›Ó·È ÌÔÓÔ˙˘ÁˆÙÈÎfi˜ ‰›‰˘ÌÔ˜ ·‰ÂÏÊfi˜, ‰ËÏ·‰‹ ÁÂÓÂÙÈο ·fiÏ˘Ù· Ù·˘ÙfiÛËÌÔ˜ Ì ÙÔ ‰¤ÎÙË, ‰ÂÓ ˘¿Ú¯ÂÈ Î›Ó‰˘ÓÔ˜ ·fiÚÚȄ˘ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ ‹ GVHD. ¶·Ú·ÙËÚ‹ıËΠfï˜, fiÙÈ ·ÎfiÌË Î·È Û ·˘Ù¤˜ ÙȘ ÂÚÈÙÒÛÂȘ, Î·È Ì¿ÏÈÛÙ· Û ÔÛÔÛÙfi 50%, ‰ÂÓ ÂÈÙ˘Á¯·ÓfiÙ·Ó ÂÁηٿÛÙ·ÛË ÙÔ˘ ÌÔۯ‡̷ÙÔ˜. ™ÙË Û˘Ó¤¯ÂÈ·, Ë ¯ÔÚ‹ÁËÛË ˘„ËÏ‹˜ ‰fiÛ˘ ΢ÎÏÔʈÛÊ·Ì›‰Ë˜ 200 mg/kg ¤Ï˘Û ÙÔ Úfi‚ÏËÌ· Ù˘ ÂÁηٿÛÙ·Û˘ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ Û ÌÔÓÔ˙˘ÁˆÙÈÎÔ‡˜ ‰È‰‡ÌÔ˘˜, Ì ·ÔÙ¤ÏÂÛÌ· ÂÈ‚›ˆÛË Û ÔÛÔÛÙfi ÌÂÁ·Ï‡ÙÂÚÔ ÙÔ˘ 90% ÙˆÓ ·ÛıÂÓÒÓ. ™ÙȘ ÌÂÙ·ÌÔۯ‡ÛÂȘ ·fi HLA ÈÛÙÔÛ˘Ì‚·ÙÔ‡˜ ·‰ÂÏÊÔ‡˜, Ë ·ÓÂ¿ÚÎÂÈ· ÌÔۯ‡̷ÙÔ˜ ÂÍ·ÎÔÏÔ˘ıÔ‡Û ӷ ·ÔÙÂÏ› Úfi‚ÏËÌ· fiÙ·Ó ÙÔ Û¯‹Ì· ÚÔÂÙÔÈÌ·Û›·˜ ÂÚÈ›¯Â ÌfiÓÔ Î˘ÎÏÔʈÛÊ·Ì›‰Ë. °È· ÙÔÓ ÏfiÁÔ ·˘Ùfi, ‰fiıËΠ·ÎfiÌË ÌÂÁ·Ï‡ÙÂÚË ¤ÌÊ·ÛË ÛÙÔ ·ÓÔÛÔηٷÛÙ·ÏÙÈÎfi ÙÌ‹Ì· ÙÔ˘ Û¯‹Ì·ÙÔ˜ ÚÔÂÙÔÈÌ·Û›·˜. ™¯‹Ì·Ù· ÚÔÂÙÔÈÌ·Û›·˜ Ô˘ ¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› Â›Ó·È Ô Û˘Ó‰˘·ÛÌfi˜ ΢ÎÏÔʈÛÊ·Ì›‰Ë˜ Ì ·ÎÙÈÓÔ‚ÔÏ›·, Ô˘ ÌÔÚ› Ó· Â›Ó·È ıˆÚ·ÎÔÎÔÈÏȷ΋, ÔÏÔۈ̷ÙÈ΋ ‹ ÔÏÔ·‰ÂÓÈ΋. º·›ÓÂÙ·È, fï˜, fiÙÈ Ù· ηχÙÂÚ· ·ÔÙÂϤÛÌ·Ù· ‰›ÓÂÈ Ô Û˘Ó‰˘·ÛÌfi˜ ΢ÎÏÔʈÛÊ·Ì›‰Ë˜ Ì ·ÓÙÈı˘ÌÔ΢ÙÙ·ÚÈ΋ ÛÊ·ÈÚ›ÓË Ô˘ ·Ó‚¿˙ÂÈ ÙÔ ÔÛÔÛÙfi ÂÁηٿÛÙ·Û˘ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ Û 95%. ∏ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹ Ô˘ ¯ÔÚËÁÂ›Ù·È ÁÈ· ÙËÓ ÚfiÏË„Ë ÙÔ˘ ÔͤԘ GVHD Û˘Ì‚¿ÏÏÂÈ ÂÈ-

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ÚfiÛıÂÙ·, ÛÙËÓ ·ÔÙÚÔ‹ Ù˘ ·fiÚÚȄ˘ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜. ∏ ¯Ú‹ÛË Ù˘ ΢ÎÏÔÛÔÚ›Ó˘ (ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÌÈÎÚ¤˜ ‰fiÛÂȘ ÌÂıÔÙÚÂÍ¿Ù˘) ÁÈ· ÙËÓ ÚfiÏË„Ë ÙÔ˘ ÔͤԘ GVHD, ·ÔÙÂÏ› ÙÔÓ ÛËÌ·ÓÙÈÎfiÙÂÚÔ ·Ú¿ÁÔÓÙ· Ô˘ ‚ÂÏÙ›ˆÛ ÙËÓ ¤Î‚·ÛË Ù˘ ÌÂÙ·ÌfiÛ¯Â˘Û˘ ÛÙËÓ ∞∞. Œ¯ÂÈ Ì¿ÏÈÛÙ· ·Ú·ÙËÚËı› fi„ÈÌË ·ÓÂ¿ÚÎÂÈ· ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ ÌÂÙ¿ ÙË ‰È·ÎÔ‹ Ù˘ ΢ÎÏÔÛÔÚ›Ó˘, ÁÂÁÔÓfi˜ Ô˘ Ô‰‹ÁËÛ οÔÈÔ˘˜ ÌÂÙ·ÌÔÛ¯Â˘Ù¤˜ Ó· Û˘Ó¯›˙Ô˘Ó ÙË ¯ÔÚ‹ÁËÛË Ù˘ ΢ÎÏÔÛÔÚ›Ó˘ Â› 12 ‹ ÂÚÈÛÛfiÙÂÚÔ˘˜ Ì‹Ó˜ ÌÂÙ¿ ·fi ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË. ∞˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ·ÓÂ¿ÚÎÂÈ·˜ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ ·Ú·ÙËÚ›ٷÈ, Â›Û˘, ÛÙȘ ÂÚÈÙÒÛÂȘ Ô˘ Ô ·ÚÈıÌfi˜ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÛÙÔ ÌfiÛ¯Â˘Ì· Â›Ó·È ÌÈÎÚfiÙÂÚÔ˜ ·fi 2x108 ÌÔÓÔ‡ÚËÓ· ·ÙÙ·Ú·/kg ‚¿ÚÔ˘˜ ÛÒÌ·ÙÔ˜ ÙÔ˘ Ï‹ÙË. ∆¤ÏÔ˜, Ë ·ÏÏÔ·ÓÔÛÔÔ›ËÛË ·fi ÚÔËÁÔ‡ÌÂÓ˜ ÌÂÙ·ÁÁ›ÛÂȘ ¤¯ÂÈ ÂÓÔ¯ÔÔÈËı› ÁÈ· ÙËÓ ·fiÚÚÈ„Ë ÙÔ˘ ÌÔۯ‡̷ÙÔ˜. Œ¯ÂÈ, Ì¿ÏÈÛÙ·, ‰È·ÈÛÙˆı› Û˘Û¯¤ÙÈÛË ·Ó¿ÌÂÛ· ÛÙÔÓ ·ÚÈıÌfi ÙˆÓ ÌÂÙ·ÁÁ›ÛÂˆÓ Ô˘ ¤¯Ô˘Ó ÚÔËÁËı› Î·È ÙË Û˘¯ÓfiÙËÙ· Ù˘ ·fiÚÚȄ˘. ∏ ¯Ú‹ÛË ÊÈÏÙÚ·ÚÈÛÌ¤ÓˆÓ Î·È ·ÎÙÈÓÔ‚ÔÏËÌ¤ÓˆÓ ·Ú·ÁÒÁˆÓ ·›Ì·ÙÔ˜ Û ·ÛıÂÓ›˜ Ì ∞∞ Â›Ó·È ÂÈÙ·ÎÙÈ΋ ÚÈÓ ·fi ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË, ÁÈ· ÙËÓ ÚfiÏË„Ë ÙfiÛÔ Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ fiÛÔ Î·È ÙÔ˘ GVHD. √ ΛӉ˘ÓÔ˜ ÔͤԘ GVHD (‚·ıÌÔ‡ II-IV) ÌÂÙ¿ ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ·fi HLAÛ˘Ì‚·Ùfi ·‰ÂÏÊfi Â›Ó·È 15-30%. ∂ÈϤÔÓ, 30-45% ÙˆÓ ·ÛıÂÓÒÓ ·Ó·Ù‡ÛÛÔ˘Ó ¯ÚfiÓÈÔ GVHD. √ ÈÔ ÛËÌ·ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË GVHD Â›Ó·È Ë ËÏÈΛ· ÙÔ˘ ·ÛıÂÓÔ‡˜, Ì ¯·ÌËÏfiÙÂÚË Â›ÙˆÛË Û Ó·ÚfiÙÂÚÔ˘˜ ·ÛıÂÓ›˜. ∏ Û˘Ó¯‹˜ ·‡ÍËÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ÌÂÙ·ÌÔۯ‡ÛÂˆÓ ÛÙËÓ ∞∞ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· ‹Ù·Ó Ë ·ÊÔÚÌ‹ ÁÈ· ÌÈ· Û˘ÁÎÚÈÙÈ΋ ÌÂϤÙË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ Û ‰È¿ÊÔÚ˜ ¯ÚÔÓÈΤ˜ ÂÚÈfi‰Ô˘˜ ·fi ÙÔ ¢ÈÂıÓ¤˜ ∞Ú¯Â›Ô ∫·Ù·ÁÚ·Ê‹˜ ªÂÙ·ÌÔۯ‡ÛÂˆÓ ª˘ÂÏÔ‡ ÙˆÓ √ÛÙÒÓ. ∞˘Ùfi Ô˘ ‰È·ÈÛÙÒıËÎÂ Â›Ó·È Ì›· Û˘Ó¯‹˜ Ù¿ÛË ·‡ÍËÛ˘ Ù˘ ÂÈ‚›ˆÛ˘ ÙˆÓ ·ÛıÂÓÒÓ (Ì·ÎÚÔ¯ÚfiÓÈ· ÂÈ‚›ˆÛË Û ÔÛÔÛÙfi 8090% ÙˆÓ ·ÛıÂÓÒÓ). ¢ÂÓ Â›Ó·È Û·Ê¤˜ ÔÈÔ› ·Ú¿ÁÔÓÙ˜ Â›Ó·È ˘‡ı˘ÓÔÈ ÁÈ’ ·˘Ù‹ ÙË ‚ÂÏÙ›ˆÛË. ™ÙË ÌÂϤÙË ·˘Ù‹ ‰ÂÓ ‰È·ÈÛÙÒıËΠ‰È·ÊÔÚ¿ ÛÙË Û˘¯ÓfiÙËÙ· ·ÓÂ¿ÚÎÂÈ·˜ ÙÔ˘ ÌÔۯ‡̷ÙÔ˜ ÛÙȘ ‰È¿ÊÔÚ˜ ¯ÚÔÓÈΤ˜ ÂÚÈfi‰Ô˘˜ Î·È Ë ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÂÈ‚›ˆÛ˘ ÔÊ›ÏÂÙ·È ÛÙËÓ ÂÏ¿ÙÙˆÛË Ù˘ ÚÒÈÌ˘ ıÓËÙfiÙËÙ·˜ ·fi ªª√. ŒÙÛÈ, Ë ‚ÂÏÙ›ˆÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ ·Ô‰fiıËΠ΢ڛˆ˜: - ÛÙË ¯Ú‹ÛË Î˘ÎÏÔÛÔÚ›Ó˘, - ÛÙËÓ ÂÈÏÔÁ‹ ÓÂfiÙÂÚˆÓ ·ÛıÂÓÒÓ ÁÈ· ÌÂÙ·ÌfiÛ¯Â˘ÛË, - ÛÙËÓ ÚˆÈÌfiÙÂÚË ÂÊ·ÚÌÔÁ‹ Ù˘ ªª√ Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ÂÏ¿ÙÙˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ÚÔËÁËıÂÈÛÒÓ ÌÂÙ·ÁÁ›ÛˆÓ,


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- ÛÙË ¯Ú‹ÛË ÂÓ‰ÔÊϤ‚È·˜ Á-ÛÊ·ÈÚ›Ó˘ Î·È ÎÔÙÚÈÌÔÍ·˙fiÏ˘ ÁÈ· ÙËÓ ÚfiÏË„Ë Â˘Î·ÈÚÈ·ÎÒÓ ÏÔÈÌÒ͈Ó. ∏ Û˘Ó‰˘·Ṳ̂ÓË ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹ Ì ΢ÎÏÔÛÔÚ›ÓË Î·È ·ÓÙÈı˘ÌÔ΢ÙÙ·ÚÈ΋ ÛÊ·ÈÚ›ÓË Â›Ó·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÂÓ·ÏÏ·ÎÙÈ΋ ıÂÚ·¢ÙÈ΋ ÂÈÏÔÁ‹ Û ·ÛıÂÓ›˜ Ì ∞∞ Ô˘ ‰ÂÓ ‰È·ı¤ÙÔ˘Ó ÈÛÙÔÛ˘Ì‚·Ùfi ·‰ÂÏÊfi ‰fiÙË. ¶ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ·fi ‰È¿ÊÔÚ· ΤÓÙÚ· ·Ó·Ê¤ÚÔ˘Ó ‚ÂÏÙ›ˆÛË Ù˘ ÂÈ‚›ˆÛ˘ ÌÂÙ¿ ·fi ·ÓÔÛÔηٷÛÙÔÏ‹ Û Â›‰· ·ÚfiÌÔÈ· Ì ·˘Ù¿ Ù˘ ÌÂÙ·ÌfiÛ¯Â˘Û˘ Ì˘ÂÏÔ‡. ∏ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹, fï˜, ‰ÂÓ ·ÔÙÚ¤ÂÈ ÙËÓ ÎψÓÈ΋ ÂÍ·ÏÏ·Á‹ ÙÔ˘ ÓÔÛ‹Ì·ÙÔ˜, Ì ·ÔÙ¤ÏÂÛÌ· Ë Ì·ÎÚÔ¯ÚfiÓÈ· ÂÈ‚›ˆÛË Ó· ·ÚÔ˘ÛÈ¿˙ÂÈ ÙˆÙÈ΋ Ù¿ÛË. ™Â 860 ·ÛıÂÓ›˜ Ì ‚·ÚÈ¿ ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›·, Ô˘ ηٷÁÚ¿ÊËÎ·Ó ·fi ÙËÓ ∂˘Úˆ·˚΋ √Ì¿‰· ∂ÚÁ·Û›·˜ ÁÈ· ÙË ªª√ Î·È Â›¯·Ó ·ÓÙÈÌÂÙˆÈÛı› Ì ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹, Ë Û˘¯ÓfiÙËÙ· ·Ó¿Ù˘Í˘ Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ‹ Ï¢¯·ÈÌ›·˜ Û ‰È¿ÛÙËÌ· 10 ÂÙÒÓ ‹Ù·Ó 9,6% Î·È 6,6% ·ÓÙ›ÛÙÔȯ·. ∞ÓÙ›ıÂÙ·, Ë Î·Ì‡ÏË Ù˘ ÂÈ‚›ˆÛ˘ ÌÂÙ¿ ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ·Ú·Ì¤ÓÂÈ ÛÙ·ıÂÚ‹, ȉȷ›ÙÂÚ· ÛÙ· ·È‰È¿, ‰ÈfiÙÈ ÙÔ ¯ÚfiÓÈÔ GVHD, Ô˘ ·ÔÙÂÏ› ÙË ÛÔ‚·ÚfiÙÂÚË ÂÈÏÔ΋, ¤¯ÂÈ ÌÈÎÚ‹ Û˘¯ÓfiÙËÙ· (5%). ¢Â˘ÙÂÚÔ·ı›˜ ÓÂÔϷۛ˜ ·Ó·Ê¤ÚÔÓÙ·È Î·È Û ÌÈÎÚfi ÔÛÔÛÙfi ·ÛıÂÓÒÓ ÌÂÙ¿ ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË. ™˘ÓÂÚÁ·ÙÈ΋ ÌÂϤÙË ·fi ÙÔ Seattle Î·È ÙÔ ¡ÔÛÔÎÔÌÂ›Ô St. Louis ÛÙÔ ¶·Ú›ÛÈ, ·Ó·Ê¤ÚÂÙ·È Û 621 ·ÛıÂÓ›˜ Ì ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· Ì ‰È¿ÌÂÛË ËÏÈΛ· Ù· 19 ¤ÙË, Ô˘ ÌÂÙ·ÌÔۯ‡ıËÎ·Ó ·fi HLA-Û˘Ì‚·Ùfi ·‰ÂÏÊfi. ∞Ó·Ù‡¯ıËÎ·Ó 18 ÓÂÔϷۛ˜: - 5 ÙÔ˘ ÏÂÌÊÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ (3 ÏÂÌÊÔ¸ÂÚÏ·ÛÙÈο – 2 ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·) ̤۷ ÛÙÔ˘˜ ÚÒÙÔ˘˜ 15 Ì‹Ó˜, - 13 Û˘Ì·Á›˜ fiÁÎÔÈ (6 ÎÂÊ·Ï‹˜-ÙÚ·¯‹ÏÔ˘, 6 ‰¤ÚÌ·ÙÔ˜ Î·È 1 ·È‰Ô›Ô˘). ¶·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Û˘Ì·ÁÒÓ fiÁÎˆÓ ‹Ù·Ó: - Ë ¯ÔÚ‹ÁËÛË ·˙·ıÂÈÔÚ›Ó˘ ÁÈ· ıÂÚ·›· ¯ÚfiÓÈÔ˘ GVHD, - Ë ÌÂÁ·Ï‡ÙÂÚË ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ Î·È - Ë ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔ‚ÔÏ›·˜ ÛÙÔ Û¯‹Ì· ÚÔÂÙÔÈÌ·Û›·˜. ∆Ô ¯ÚfiÓÈÔ GVHD ‰ÂÓ ·ÔÙÂÏ› ȉȷ›ÙÂÚÔ Úfi‚ÏËÌ· ÛÙ· ·È‰È¿ Î·È Ù· Û¯‹Ì·Ù· ÚÔÂÙÔÈÌ·Û›·˜ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È Û‹ÌÂÚ· ‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ·ÎÙÈÓÔ‚ÔÏ›·. ∂Ô̤ӈ˜, Ô Î›Ó‰˘ÓÔ˜ ‰Â˘ÙÂÚÔ·ıÒÓ ÓÂÔÏ·ÛÈÒÓ ÛÙ· ·È‰È¿ Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ·ÌÂÏËÙ¤Ô˜. ∏ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·fi ÂÓ·ÏÏ·ÎÙÈÎÔ‡˜ ‰fiÙ˜ (ÌË Û˘ÁÁÂÓ›˜ Û˘Ì‚·ÙÔ‡˜ ‹ Û˘ÁÁÂÓ›˜ Ê·ÈÓÔÙ˘Èο Û˘Ì‚·ÙÔ‡˜ ‹ Ì 1 ·ÓÙÈÁfiÓÔ ‰È·ÊÔÚ¿) ‰ÂÓ ·ÔÙÂÏ› ıÂÚ·›· ÚÒÙ˘ ÁÚ·ÌÌ‹˜, ¤¯ÂÈ

Paediatriki 2001;64:45-48

fï˜ ¤Ó‰ÂÈÍË Û ·È‰È¿ Ì ∞∞ Ô˘ ‰ÂÓ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ ·ÓÔÛÔηٷÛÙ·ÏÙÈ΋ ·ÁˆÁ‹. ∞Ó·ÈÌ›· Fanconi ¢Â˘ÙÂÚÔ·ı‹˜ ∞∞ ·Ú·ÙËÚÂ›Ù·È Û ·È‰È¿ Ì ·Ó·ÈÌ›· Fanconi, fiÔ˘ ˘¿Ú¯ÂÈ ·ÚÎÂÙ‹ ÂÌÂÈÚ›· Ì ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ. π‰È·›ÙÂÚÔ Úfi‚ÏËÌ· ηٿ ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË Û ·Ó·ÈÌ›· Fanconi ·ÔÙÂÏ› Ë ÂÁÁÂÓ‹˜ ˘ÂÚ¢·ÈÛıËÛ›· ÙˆÓ Î˘ÙÙ¿ÚˆÓ Û ·Ú¿ÁÔÓÙ˜ Ô˘ ‰È·Ù·Ú¿ÛÛÔ˘Ó ÙË ‰ÔÌ‹ ÙÔ˘ DNA, fiˆ˜ Â›Ó·È Ë Î˘ÎÏÔʈÛÊ·Ì›‰Ë Î·È Ë ·ÎÙÈÓÔ‚ÔÏ›· ÛÙȘ ‰fiÛÂȘ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÛÙ· Û˘Ó‹ıË Û¯‹Ì·Ù· ÚÔÂÙÔÈÌ·Û›·˜. °È’ ·˘ÙfiÓ ÙÔ ÏfiÁÔ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È: - ÌfiÓÔ Î˘ÎÏÔʈÛÊ·Ì›‰Ë, - ΢ÎÏÔʈÛÊ·Ì›‰Ë Û ‰fiÛË 20-40 mg/kg + ıˆÚ·ÎÔÎÔÈÏȷ΋ ·ÎÙÈÓÔ‚ÔÏ›· 400-500cGy, - ΢ÎÏÔʈÛÊ·Ì›‰Ë + ·ÓÙÈı˘ÌÔ΢ÙÙ·ÚÈ΋ ÛÊ·ÈÚ›ÓË + ÊÏÔ˘ÓÙ·Ú·Ì›ÓË, - ‚Ô˘ÛÔ˘ÏÊ¿ÓË + ΢ÎÏÔʈÛÊ·Ì›‰Ë. ∏ ÌÂÁ·Ï‡ÙÂÚË ÔÌ¿‰· ·ÛıÂÓÒÓ Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó Ì ÌÂÙ·ÌfiÛ¯Â˘ÛË ÚÔ¤Ú¯ÂÙ·È ·fi ÙÔ IBMTR (International Bone Marrow Transplantation Registry). ∫·Ù¿ ÙËÓ ÂÚ›Ô‰Ô 1978-1992, ÌÂÙ·ÌÔۯ‡ıËÎ·Ó 199 ·ÛıÂÓ›˜ Ì ·Ó·ÈÌ›· Fanconi. ™ÙÔ˘˜ 151 Ô˘ ˘Ô‚Ï‹ıËÎ·Ó Û ÌÂÙ·ÌfiÛ¯Â˘ÛË ·fi HLAÛ˘Ì‚·Ùfi ·‰ÂÏÊfi, Ë ‰ÈÂÙ‹˜ ÂÈ‚›ˆÛË ‹Ù·Ó 66%. ™ÙÔ˘˜ 48 Ô˘ ¤Ï·‚·Ó ÌfiÛ¯Â˘Ì· ·fi ÂÓ·ÏÏ·ÎÙÈÎÔ‡˜ ‰fiÙ˜, Ë ‰ÈÂÙ‹˜ ÂÈ‚›ˆÛË ‹Ù·Ó 29%. ¶·Ú¿ÁÔÓÙ˜ Ô˘ Û˘Û¯ÂÙ›ÛıËÎ·Ó Ì ÛËÌ·ÓÙÈο ÂÏ·Ùو̤ÓË ıÓËÙfiÙËÙ· ·fi ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË ‹Ù·Ó: - ËÏÈΛ· ·ÛıÂÓÔ‡˜ <10 ¤ÙË - ·ÚÈıÌfi˜ ·ÈÌÔÂÙ·Ï›ˆÓ ÚÈÓ ·fi ÙË ªª√ - ¯Ú‹ÛË ·ÓÙÈı˘ÌÔ΢ÙÙ·ÚÈ΋˜ ÛÊ·ÈÚ›Ó˘ - ¯Ú‹ÛË Î˘ÎÏÔʈÛÊ·Ì›‰Ë˜ (15-25mg/kg) + ·ÎÙÈÓÔ‚ÔÏ›· ÂÚÈÔÚÈṲ̂ÓÔ˘ ‰›Ô˘ - ¯Ú‹ÛË Î˘ÎÏÔÛÔÚ›Ó˘ ÁÈ· ÚÔʇϷÍË ·fi GVHD. ª¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 40 ÂÙÒÓ, ÂÚÈÛÛfiÙÂÚÔÈ ·fi ÙÔ˘˜ ÌÈÛÔ‡˜ ·ÛıÂÓ›˜ Ì ·Ó·ÈÌ›· Fanconi ·Ó·Ù‡ÛÛÔ˘Ó Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ ‹ Ï¢¯·ÈÌ›·. ∂›Û˘, ·˘ÍË̤ÓË Â›Ó·È Ë Û˘¯ÓfiÙËÙ· Û˘Ì·ÁÒÓ fiÁΈÓ. Œ¯Ô˘Ó ·Ó·ÊÂÚı› ÂÚÈÙÒÛÂȘ ÔÍ›·˜ Ì˘ÂÏÔÁÂÓÔ‡˜ Ï¢¯·ÈÌ›·˜ ÌÂÙ¿ ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ Û ·Ó·ÈÌ›· Fanconi, ¯ˆÚ›˜ fï˜ Ó· Â›Ó·È Û·Ê¤˜ ·Ó ÔÈ ·ÛıÂÓ›˜ ·˘ÙÔ› ›¯·Ó ‹‰Ë ·Ó·Ù‡ÍÂÈ Ï¢¯·ÈÌ›· ‹ Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ ÚÈÓ ·fi ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË. ™ÙË ÛÂÈÚ¿ ÙˆÓ ·ÛıÂÓÒÓ ·fi ÙÔ Seattle Î·È ÙÔ ¡ÔÛÔÎÔÌÂ›Ô St. Louis, Û 79 ·ÛıÂÓ›˜ Ì ·Ó·ÈÌ›· Fanconi Î·È ‰È¿ÌÂÛË ËÏÈΛ· Ù· 10 ¤ÙË, ·Ó·Ù‡¯ıËÎ·Ó 5 Û˘Ì·Á›˜ fiÁÎÔÈ ÎÂÊ·Ï‹˜ Î·È ÙÚ·¯‹ÏÔ˘ Û ‰È¿ÛÙËÌ· 6-11 ÂÙÒÓ ÌÂÙ¿ ·fi ÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË, ÂÓÒ ‰ÂÓ ·Ú·ÙËÚ‹ıËΠη̛· ÓÂÔÏ·Û›·

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ÙÔ˘ ÏÂÌÊÔ·ÈÌÔÔÈËÙÈÎÔ‡ ÈÛÙÔ‡. ∆Ô Â‡ÚËÌ· ·˘Ùfi Â›Ó·È Û˘Ì‚·Ùfi Ì ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ÌÂÙ·ÌfiÛ¯Â˘ÛË ·Ôη٤ÛÙËÛÂ Ê˘ÛÈÔÏÔÁÈ΋ ·ÈÌÔÔÈ›· ÛÙÔ˘˜ ·ÛıÂÓ›˜, ·ÏÏ¿ ‰ÂÓ ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ ÙËÓ Â˘¿ıÂÈ· ÙˆÓ ¿ÏÏˆÓ ÈÛÙÒÓ ÛÙËÓ ·Ó¿Ù˘ÍË ÓÂÔÏ·ÛÈÒÓ. √È ÌÂÙ·ÌÔÛ¯Â˘Ì¤ÓÔÈ ·ÛıÂÓ›˜ Ì ·Ó·ÈÌ›· Fanconi ‹Ù·Ó ËÏÈΛ·˜ 17-29 ÂÙÒÓ fiÙ·Ó ‰È·ÁÓÒÛıËÎ·Ó ÔÈ Û˘Ì·Á›˜ fiÁÎÔÈ, ËÏÈΛ· ·ÚfiÌÔÈ· Ì ÙËÓ ·Ó·ÊÂÚfiÌÂÓË ÁÈ· ÌË ÌÂÙ·ÌÔÛ¯Â˘Ì¤ÓÔ˘˜ ·ÛıÂÓ›˜. ÕÏÏ· Û‡Ó‰ÚÔÌ· Ì˘ÂÏÈ΋˜ ·ÓÂ¿ÚÎÂÈ·˜ ∏ ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·ÛıÂÓÒÓ Ì ·Ó·ÈÌ›· Diamond-Blackfan, Ô˘ ‰ÂÓ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙË ıÂÚ·›· Ì ÛÙÂÚÔÂȉ‹ ÎÈ ¤¯Ô˘Ó ·Ó¿ÁÎË ·fi Û˘¯Ó¤˜ ÌÂÙ·ÁÁ›ÛÂȘ. ¶·ÚfiÙÈ Ë ‰ÈÂıÓ‹˜ ÂÌÂÈÚ›· Â›Ó·È ÂÚÈÔÚÈṲ̂ÓË, ÏfiÁˆ Ù˘ Û·ÓÈfiÙËÙ·˜ ÙÔ˘ ÓÔÛ‹Ì·ÙÔ˜, Ù· ·ÔÙÂϤÛÌ·Ù· Â›Ó·È ÂÓı·ÚÚ˘ÓÙÈο. ÕÏÏ· Û·ÓÈfiÙÂÚ· Û‡Ó‰ÚÔÌ· Ì˘ÂÏÈ΋˜ ·Ï·Û›·˜ Û ·È‰È¿ fiÔ˘ ˘¿Ú¯Ô˘Ó ÌÂÌÔӈ̤Ó˜ ·Ó·ÊÔÚ¤˜ ıÂÚ·›·˜ Ì ÌÂÙ·ÌfiÛ¯Â˘ÛË Â›Ó·È Ë Û˘ÁÁÂÓ‹˜ ‰˘ÛÎÂÚ¿ÙˆÛË, ÙÔ Û‡Ó‰ÚÔÌÔ Schwachman-Diamond, Ë ·ÌÂÁ·Î·Ú˘Ô΢ÙÙ·ÚÈ΋ ıÚÔÌ‚ÔÂÓ›·, ÙÔ Û‡Ó‰ÚÔÌÔ ıÚÔÌ‚ÔÂÓ›·˜ Ì ·Ô˘Û›· ÎÂÚΛ‰ˆÓ Î·È ÙÔ Û‡Ó‰ÚÔÌÔ Kostmann. ∞Ó·ÎÂÊ·Ï·ÈÒÓÔÓÙ·˜, Ë ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·ÔÙÂÏ› ÙË ıÂÚ·›· ÂÎÏÔÁ‹˜ ÁÈ· ·È‰È¿ Ì ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· Ì ÈÛÙÔÛ˘Ì‚·Ùfi ·‰ÂÏÊfi ‰fiÙË. ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ ·Ó·ÈÌ›· Fanconi, ÂÈ‚¿ÏÏÂÙ·È Ó· ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È Ù·ÎÙÈο ÔÈ ·ÛıÂÓ›˜

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ÌÂ Ì˘ÂÏfiÁÚ·ÌÌ· Î·È Ó· Á›ÓÂÙ·È ÌÂÙ·ÌfiÛ¯Â˘ÛË fiÛÔ ÙÔ ‰˘Ó·ÙfiÓ ÂÓˆÚ›ÙÂÚ· fiÙ·Ó ·Ó·Ù˘¯ı› ·Ï·ÛÙÈ΋ ·Ó·ÈÌ›· ‹ fiÙ·Ó ·ÓȯÓ‡ÔÓÙ·È Î˘ÙÙ·ÚÔÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜, ÚÔÙÔ‡ ÂΉËψı› Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎfi Û‡Ó‰ÚÔÌÔ ‹ ÔÍ›· Ï¢¯·ÈÌ›·. ∆¤ÏÔ˜, Ë ÌÂÙ·ÌfiÛ¯Â˘ÛË Â›Ó·È ÂÈÙ˘¯‹˜ Û ·ÛıÂÓ›˜ Ì ·Ó·ÈÌ›· Diamond-Blackfan, Ô˘ Â›Ó·È ·ÓıÂÎÙÈ΋ ÛÙ· ÛÙÂÚÔÂȉ‹. µÈ‚ÏÈÔÁÚ·Ê›· 1. Young NS, Barrett AJ. The treatment of severe aplastic anemia. Blood 1995;85:3367-3377. 2. Gluckman E, Auerbach AD, Horowitz MM, Sobocinski KA, Ash RC, Bortin MM et al. Bone marrow transplantation for Fanconi anemia. Blood 1995;86:2856-2862. 3. Deeg HJ, Socié G, Schoch G, Henry-Amar M, Witherspoon RP, Devergie A et al. Malignancies after marrow transplantation for aplastic anemia and Fanconi anemia: a joint Seattle and Paris analysis of results in 700 patients. Blood 1996;87:386-392. 4. Passweg JR, Socié G, Hinterberger W, Bacigalupo A, Biggs JC, Camitta BM et al. Bone marrow transplantation for severe aplastic anemia: has outcome improved? Blood 1997;90:858-864.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 02-10-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 21-11-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ¢ËÌ‹ÙÚÈÔ˜ ¶ÂÙÚfiÔ˘ÏÔ˜ ª.ª.ª.√., ¶.°.¡.∞. “∞Á›· ™ÔÊ›·” £Ë‚ÒÓ Î·È §Â‚·‰Â›·˜, ∞ı‹Ó· 115 27


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Paediatriki 2001;64:49-54

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£ÂÚ·›· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· ÛÙËÓ ÔÍ›· ‰È¿ÚÚÔÈ· Î·È ·Ó·ÛÙ·ÏÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ ∂. ∞ÏÂÍfiÔ˘ÏÔ˜, ¡. ™ÎÂÓÙ¤Ú˘, ¡. ™Ô‡ÎÈ·˜, ∂. ∫ˆÛÙ·‰‹Ì·, ™. ÷˚‰¿˜

Oral rehydration therapy for acute diarrhea and factors that inhibit its application E. Alexopoulos, N. Skenteris, N. Soukias, E. Kostadima, S. Haidas

¶ÂÚ›ÏË„Ë: ∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ·Ê˘‰¿ÙˆÛ˘ Û˘ÓÂ›· ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ Â›Ó·È Û˘¯Ófi Úfi‚ÏËÌ· ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ú·ÎÙÈ΋ ÙÔ˘ ·È‰›·ÙÚÔ˘. ∏ ·ÚÔ‡Û· ÌÂϤÙË ÚÔÛ·ı› Ó· ‰ÈÂÚ¢ӋÛÂÈ ÙÔ ‚·ıÌfi ·Ô‰Ô¯‹˜ ·fi ÙÔ˘˜ ·È‰›·ÙÚÔ˘˜ ÙˆÓ ÚÔÙ¿ÛÂˆÓ Ù˘ ‰ÈÂıÓÔ‡˜ ‚È‚ÏÈÔÁÚ·Ê›·˜ ÁÈ· ÙË ıÂÚ·›· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· (£∂™) Î·È ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ·Ó·ÛÙ¤ÏÏÔ˘Ó ÙË Û˘ÓÙ·ÁÔÁÚ¿ÊËÛ‹ Ù˘. ÃÚËÛÈÌÔÔÈ‹ıËΠ¤Ó· ·ÓÒÓ˘ÌÔ ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ ÚÔ˜ ÙÔ˘˜ ·È‰›·ÙÚÔ˘˜ Ô˘ ·ÊÔÚ¿ ÙË ÁÓÒÛË, ÙË ÛÙ¿ÛË Î·È ÙËÓ Ú·ÎÙÈ΋ ÙÔ˘˜ Û ۯ¤ÛË Ì ÙËÓ ÂÓ˘‰¿ÙˆÛË ·fi ÙÔ ÛÙfiÌ· Û Ôͤ· ‰È·ÚÚÔ˚ο Û‡Ó‰ÚÔÌ·. √È ÂÚÈÛÛfiÙÂÚÔÈ ·È‰›·ÙÚÔÈ (90%) ¤¯Ô˘Ó ıÂÙÈ΋ ÛÙ¿ÛË ˆ˜ ÚÔ˜ ÙËÓ ÂÓ˘‰¿ÙˆÛË ·fi ÙÔ ÛÙfiÌ·. ªfiÓÔ ÙÔ 13% ÈÛÙ‡ÂÈ fiÙÈ Ë ÂÎ·›‰Â˘ÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ Â›Ó·È ‰‡ÛÎÔÏË ‹ ·‰‡Ó·ÙË Î·È fiÙÈ ÔÈ ÁÔÓ›˜ ‰ÂÓ ¤¯Ô˘Ó ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ·ÓÙÈÌÂÙˆ›ÛÔ˘Ó ·ÔÙÂÏÂÛÌ·ÙÈο Ù· ·È‰È¿ ÛÙÔ Û›ÙÈ. ŸÏÔÈ ÔÈ ·È‰›·ÙÚÔÈ ÚÔÙ›ÓÔ˘Ó, ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÚ›ÙˆÛË, ÙfiÛÔ ÙËÓ ÂÓ˘‰¿ÙˆÛË ·fi ÙÔ ÛÙfiÌ· fiÛÔ Î·È ÙËÓ ÂÓ‰ÔÊϤ‚È· ÂÓ˘‰¿ÙˆÛË. ŸÌˆ˜, ÙÔ 43% ıˆÚ› ÙÔ˘˜ Â̤ÙÔ˘˜ ·ÓÙ¤Ó‰ÂÈÍË ÛÙËÓ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ÂÓ˘‰¿ÙˆÛË. √È ÂÚÈÛÛfiÙÂÚÔÈ ·È‰›·ÙÚÔÈ (87%) Û˘ÓÙ·ÁÔÁÚ·ÊÔ‡Ó ÂȉÈο ‰È·Ï‡Ì·Ù· ÁÈ· ÂÓ˘‰¿ÙˆÛË ·fi ÙÔ ÛÙfiÌ·, ·Ó Î·È ÙÔ 17% ·fi ·˘ÙÔ‡˜ ÚÔÙ›ÓÔ˘Ó ÌÂÚÈΤ˜ ÊÔÚ¤˜ Î·È ÌË ÂÓ‰ÂÈÎÓ˘fiÌÂÓ· ˘ÁÚ¿. ªfiÓÔ ÙÔ 13% ÚÔÙ›ÓÂÈ ÛÙÔ˘˜ ÁÔÓ›˜ Ó· ¯ÚËÛÈÌÔÔÈÔ‡Ó ‰ÔÛÔÌÂÙÚËÙ‹ (ÎÔ˘Ù·Ï¿ÎÈ ‹ Û‡ÚÈÁÁ·). √È ÂÚÈÛÛfiÙÂÚÔÈ (55%) Û˘ÛÙ‹ÓÔ˘Ó Â·Ó·Û›ÙÈÛË ÛÙÔ ÚÒÙÔ 24ˆÚÔ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘. √ ÛËÌ·ÓÙÈÎfiÙÂÚÔ˜ ·Ó·ÛÙ·ÏÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ £∂™ Â›Ó·È Ë ÛÙ¿ÛË ÙˆÓ ·È‰›·ÙÚˆÓ Û ۯ¤ÛË Ì ÙË ‰˘ÛÎÔÏ›· ÂÎ·›‰Â˘Û˘ ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ. ∞·ÈÙÔ‡ÓÙ·È ÚfiÛıÂÙ˜

Abstract: Management of dehydration resulting from acute diarrhea is a common problem in pediatric practice. This complication can be prevented and managed with prompt administration of appropriate oral solutions. This study attempts to determine the degree of acceptance by the pediatricians of the recommendations published in the international literature regarding the use of oral rehydration therapy (ORT), as well as the factors responsible for its non implementation. By using an anonymous questionnaire addressed to pediatricians, we investigated their knowledge, attitudes and practices in relation to the ORT. Almost all pediatricians (90%) have a positive attitude as far as ORT is concerned. Only 13% believe that training the families is a difficult or impossible process due to their inability to cope successfully with their sick child at home. All the pediatricians, depending on the case, suggest the use of either ORT or intravenous rehydration. However 43% consider vomiting as a contraindication to oral rehydration. Most pediatricians (87%) prescribe appropriate oral rehydration solutions, although 17% of them propose occasionally the use of inappropriate liquids. Only 13% suggest to the parents the use of volumetric measurements for feeding (spoon or syringe) and 55% suggest refeeding in the first 24 hours from the beginning of the illness. The most important inhibiting factor for its application is the difficulty in training the families. Additional efforts are required to encourage the use of the recommended guidelines for the outpatient management of children with acute diarrhea.

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ £ÂÛÛ·Ï›·˜, ¶.¶.°.¡.§¿ÚÈÛ·˜

Pediatric Clinic of the University of Thessalia University General Hospital of Larisa

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 50

¶·È‰È·ÙÚÈ΋ 2001;64:49-54

Paediatriki 2001;64:49-54

ÚÔÛ¿ıÂȘ ÁÈ· ÙËÓ ÂÈÎÚ¿ÙËÛË Ù˘ ÂÓ‰ÂÈÎÓ˘fiÌÂÓ˘ Â͈ÓÔÛÔÎÔÌÂȷ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì ÔÍ›· ‰È¿ÚÚÔÈ·. §¤ÍÂȘ ÎÏÂȉȿ: ÔÍ›· ‰È¿ÚÚÔÈ·, ·Ê˘‰¿ÙˆÛË, ıÂÚ·›· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ·.

∂ÈÛ·ÁˆÁ‹ ∏ ÔÍ›· ‰È¿ÚÚÔÈ· Û˘Ó¯›˙ÂÈ Ó· ·ÔÙÂÏ› ·ÚÈÔ ·›ÙÈÔ Ù˘ ·È‰È΋˜ ÓÔÛËÚfiÙËÙ·˜ Î·È ıÓËÛÈÌfiÙËÙ·˜ ÛÙÔÓ ·Ó·Ù˘ÛÛfiÌÂÓÔ, ΢ڛˆ˜, ÎfiÛÌÔ (1). ™ÙË ¯ÒÚ· Ì·˜ ÔÈ Û˘Ó¤ÂȤ˜ Ù˘ ÛÙËÓ ˘Á›· ÙˆÓ ·È‰ÈÒÓ Â›Ó·È ËÈfiÙÂÚ˜, ˆÛÙfiÛÔ ‰ÂÓ ·‡ÂÈ Ó· ·ÔÙÂÏ› ηıËÌÂÚÈÓfi Úfi‚ÏËÌ· ÁÈ· ÙÔÓ ·È‰›·ÙÚÔ. π‰È·›ÙÂÚ· ··Û¯ÔÏ› Ë ÂÈÏÔÁ‹ Ù˘ Ô‰Ô‡ ·fi ÙËÓ ÔÔ›· ı· ¯ÔÚËÁËıÔ‡Ó Ù· ··Ú·›ÙËÙ· ˘ÁÚ¿ (p.o. ‹ i.v.) ÁÈ· ÙË ‰ÈfiÚıˆÛË Ù˘ ·Ê˘‰¿ÙˆÛ˘, Ù˘ Û˘¯ÓfiÙÂÚ˘ ÂÈÏÔ΋˜ Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜. ŸÙ·Ó ÔÈ Schultz Î·È Zalusky (2) ·¤‰ÂÈÍ·Ó Û ÏËı˘ÛÌfi ÎÔ˘ÓÂÏÈÒÓ Î·È Ô Binder (3) Û ·ÓıÚÒÈÓÔ ÏËı˘ÛÌfi, fiÙÈ Ë ÁÏ˘Îfi˙Ë ÂÓÈÛ¯‡ÂÈ ÙËÓ ÚfiÛÏË„Ë ÙÔ˘ Ó·ÙÚ›Ô˘ ·fi ÙË Ó‹ÛÙȉ·, Ù¤ıËÎ·Ó ÔÈ ‚¿ÛÂȘ ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· ÙˆÓ Î·Ù¿ÏÏËÏˆÓ ‰È·Ï˘Ì¿ÙˆÓ ÁÈ· ÂÓ˘‰¿ÙˆÛË ·fi ÙÔ ÛÙfiÌ·. ™‹ÌÂÚ· ‰È¿ÊÔÚÔÈ ‰ÈÂıÓ›˜ ÔÚÁ·ÓÈÛÌÔ› (WHO, AAP, European Society of Pediatric Gastroenterology and Nutrition) ÚÔÙ›ÓÔ˘Ó ‰È·Ï‡Ì·Ù· Ô˘ ıˆÚÔ‡Ó Î·Ù¿ÏÏËÏ· ÁÈ· ÂÓ˘‰¿ÙˆÛË p.o. Î·È Ô˘ ÂÚȤ¯Ô˘Ó ËÏÂÎÙÚÔχÙ˜ Î·È ˘‰·Ù¿ÓıڷΘ (ÁÏ˘Îfi˙Ë, ۷ί·Úfi˙Ë, Ú˘˙¿Ï¢ÚÔ ÎÏ). ∏ ¯Ú‹ÛË ·Ó¿ÏÔÁˆÓ ‰È·Ï˘Ì¿ÙˆÓ Â›Ó·È ·ÛʷϤÛÙÂÚË, ÏÈÁfiÙÂÚÔ ÂÒ‰˘ÓË Î·È ÔÈÎÔÓÔÌÈÎfiÙÂÚË ·fi ÙËÓ i.v. ÂÓ˘‰¿ÙˆÛË (4-9). ¶ÚÔÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÌÊ¿ÓÈÛË ·Ê˘‰¿ÙˆÛ˘ (6-10), ÂÏ·ÙÙÒÓÂÈ ÙÔÓ ·ÚÈıÌfi ÙˆÓ ÂÈÛΤ„ÂˆÓ ÛÙÔÓ ·È‰›·ÙÚÔ (11) Î·È Â›Ó·È ÂÍ›ÛÔ˘ ·ÔÙÂÏÂÛÌ·ÙÈ΋ Ì ÙËÓ i.v. ÂÓ˘‰¿ÙˆÛË, ÙfiÛÔ ÁÈ· ÙÔ˘˜ ÓÔÛËÏ¢fiÌÂÓÔ˘˜ fiÛÔ Î·È ÁÈ· ÙÔ˘˜ Â͈ÓÔÛÔÎÔÌÂÈ·ÎÔ‡˜ ·ÛıÂÓ›˜ (12). º·›ÓÂÙ·È fiÙÈ Ù· ‰È·Ï‡Ì·Ù· Ô˘ ÂÚȤ¯Ô˘Ó ¿Ì˘ÏÔ Ú˘˙ÈÔ‡ Â›Ó·È ÈÔ ·ÔÙÂÏÂÛÌ·ÙÈο ·fi ·˘Ù¿ Ô˘ ÂÚȤ¯Ô˘Ó ÁÏ˘Îfi˙Ë, ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ˆÏÂÈÒÓ ·fi ÙȘ ‰È¿ÚÚÔȘ (13). ™Â ·ÓÙ›ıÂÛË Ì ÙȘ ÚÔÙ¿ÛÂȘ ÙˆÓ ‰ÈÂıÓÒÓ ÔÚÁ·ÓÈÛÌÒÓ Î·È ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›·, Ë ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ÛÙÔÓ ·ÓÂÙ˘Á̤ÓÔ ÎfiÛÌÔ ÂÍ·ÎÔÏÔ˘ı› Ó· ÛÙËÚ›˙ÂÙ·È, ÂÚÈÛÛfiÙÂÚÔ ·fi ÙÔ ÂӉ‰ÂÈÁ̤ÓÔ, ÛÙËÓ i.v. ÂÓ˘‰¿ÙˆÛË (14). ™ËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ıÂÚ·›· ÙÔ˘ ·È‰ÈÔ‡ Ì ÔÍ›· ‰È¿ÚÚÔÈ· η٤¯ÂÈ Î·È Ë ¤ÁηÈÚË Â·Ó·Û›ÙÈÛË Ë ÔÔ›· Â›Ó·È ˆÊ¤ÏÈÌË (15,16) ηÈ, ÂÊfiÛÔÓ Â›Ó·È Ë ÂÓ‰ÂÈÎÓ˘fiÌÂÓË, ÌÔÚ› Ó· ÂÏ·ÙÙÒÛÂÈ ÙË Û˘¯ÓfiÙËÙ· Î·È ÙÔÓ fiÁÎÔ ÙˆÓ ÎÂÓÒÛÂˆÓ Î·È Ó· ÂÈÛ‡ÛÂÈ ÙËÓ ›·ÛË (17-21). ∫·Ù¿ÏÏËϘ

50

Key words: acute diarrhea, dehydration, oral rehydration therapy.

ÙÚÔʤ˜, ηϿ ·ÓÂÎÙ¤˜ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ‰È¿ÚÚÔÈ·˜ Â›Ó·È ·˘Ù¤˜ Ô˘ ÂÚȤ¯Ô˘Ó ¿Ì˘ÏÔ (ÔÏ˘ÌÂÚ‹ ÁÏ˘Îfi˙˘) fiˆ˜ ÙÔ Ú‡˙È, Ô ·Ú·‚fiÛÈÙÔ˜, ÙÔ ÛÈÙ¿ÚÈ Î·È Ë ·Ù¿Ù· (19,22-23). ∏ ·ÚÔ‡Û· ÌÂϤÙË ÚÔÛ·ı› Ó· ‰ÈÂÚ¢ӋÛÂÈ: ·) ÙÔ ‚·ıÌfi ·Ô‰Ô¯‹˜ ·fi ÙÔ˘˜ ·È‰›·ÙÚÔ˘˜ ÙˆÓ ÚÔÙ¿ÛÂˆÓ Ù˘ ‰ÈÂıÓÔ‡˜ ‚È‚ÏÈÔÁÚ·Ê›·˜ ÁÈ· ÙËÓ ıÂÚ·›· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· (£∂™) Î·È ‚) ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ·Ó·ÛÙ¤ÏÏÔ˘Ó ÙË Û˘¯ÓfiÙÂÚË Û˘ÓÙ·ÁÔÁÚ¿ÊËÛ‹ Ù˘. °È· ÙÔ ÛÎÔfi ·˘Ùfi ¯ÚËÛÈÌÔÔÈ‹Û·Ì ¤Ó· ·ÓÒÓ˘ÌÔ ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ Ô˘ ‰fiıË ¶›Ó·Î·˜ 1. ¶ÂÚȯfiÌÂÓÔ ÙÔ˘ ÂÚˆÙËÌ·ÙÔÏÔÁ›Ô˘ I. °ÓÒÛË ÙˆÓ ·È‰›·ÙÚˆÓ ∂ÚˆÙ‹ÛÂȘ Ì ··ÓÙ‹ÛÂȘ ÔÏÏ·Ï‹˜ ÂÈÏÔÁ‹˜ Ô˘ ·ÊÔÚÔ‡Ó: ∆Ș ÂӉ›ÍÂȘ Ù˘ £∂™ ∆Ș ·ÓÙÂӉ›ÍÂȘ Ù˘ £∂™ ∆· Û˘ÛÙ·ÙÈο ÙˆÓ ‰È·Ï˘Ì¿ÙˆÓ ÂÓ˘‰¿ÙˆÛ˘ ∆Ô ¯ÚfiÓÔ Â·Ó·Û›ÙÈÛ˘ II. ™Ù¿ÛË ÙˆÓ ·È‰›·ÙÚˆÓ ∂ÚˆÙ‹ÛÂȘ Ì ··ÓÙ‹ÛÂȘ ۯ‰ȷṲ̂Ó˜ Ì ÙËÓ Îϛ̷η Likert 5 ÛËÌ›ˆÓ Ô˘ ·ÊÔÚÔ‡Ó: ∆ËÓ ·ÛÊ¿ÏÂÈ· Î·È ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ £∂™ ∆Ë ı¤ÛË Ù˘ £∂™ ÛÙËÓ ÚfiÏË„Ë Ù˘ ·Ê˘‰¿ÙˆÛ˘ ∆ËÓ Â˘ÎÔÏ›· ÂÎ·›‰Â˘Û˘ ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ ∆ËÓ ÚÔÙ›ÌËÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ ‹ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ÁÈ· £∂™ ‹ ÂÓ‰ÔÊϤ‚È· ÂÓ˘‰¿ÙˆÛË ∆ËÓ ÚÔÙ›ÌËÛË ÙÔ˘ ÓÔÛËÏ¢ÙÈÎÔ‡ ÚÔÛˆÈÎÔ‡ ÁÈ· £∂™ ‹ ÂÓ‰ÔÊϤ‚È· ÂÓ˘‰¿ÙˆÛË III. ¶Ú·ÎÙÈ΋ ÙˆÓ ·È‰›·ÙÚˆÓ ∂ÚˆÙ‹ÛÂȘ Ì ÂχıÂÚ˜ ··ÓÙ‹ÛÂȘ Ô˘ ·ÊÔÚÔ‡Ó: ∆Ș ÂӉ›ÍÂȘ Ù˘ £∂™ ∆Ș ·ÓÙÂӉ›ÍÂȘ Ù˘ £∂™ ∆· ‰È·Ï‡Ì·Ù· ÂÓ˘‰¿ÙˆÛ˘ Ô˘ Û˘ÛÙ‹ÓÔ˘Ó ∆Ș Ô‰ËÁ›Â˜ Â·Ó·Û›ÙÈÛ˘ Ô˘ ÚÔÙ›ÓÔ˘Ó IV. ∏ Û˘Ìʈӛ· Ì ÙË ı¤ÛË: «∏ £∂™ η٤¯ÂÈ ÛËÌ·ÓÙÈ΋ ı¤ÛË ÛÙËÓ Î·ıËÌÂÚÈÓ‹ È·ÙÚÈ΋ Ú¿ÍË ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜» ÂϤÁ¯ıËΠ̠Îϛ̷η Likert 5 ÛËÌ›ˆÓ


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 51

¶·È‰È·ÙÚÈ΋ 2001;64:49-54

ÛÙÔ˘˜ ·È‰›·ÙÚÔ˘˜ Î·È ·ÊÔÚ¿: ·) ÛÙË ÁÓÒÛË, ‚) ÙË ÛÙ¿ÛË Î·È Á) ÙËÓ Ú·ÎÙÈ΋ ÙÔ˘˜ Û ۯ¤ÛË Ì ÙËÓ ÂÓ˘‰¿ÙˆÛË p.o. Û Ôͤ· ‰È·ÚÚÔ˚ο Û‡Ó‰ÚÔÌ·. ÀÏÈÎfi Î·È Ì¤ıÔ‰Ô˜ ∆Ô ‰Â›ÁÌ· Ù˘ ÌÂϤÙ˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ·È‰›·ÙÚÔ˘˜ Ô˘ ·ÛÎÔ‡Ó ÙÔ Â¿ÁÁÂÏÌ· ÛÙË £ÂÛÛ·Ï›·. ∆Ô˘˜ ˙ËÙ‹ıËΠӷ Û˘ÌÏËÚÒÛÔ˘Ó ¤Ó· ·ÓÒÓ˘ÌÔ ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ Û¯ÂÙÈÎfi Ì ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ‰È·ÚÚÔ˚΋˜ ÓfiÛÔ˘. ŸÏ· Ù· ÂÚˆÙËÌ·ÙÔÏfiÁÈ· Ô˘ Û˘ÌÏËÚÒıËÎ·Ó ·fi ÙÔ˘˜ ·È‰›·ÙÚÔ˘˜ (47 ·fi ÙÔ˘˜ 49 Ô˘ ·¢ı˘Óı‹Î·ÌÂ Û˘ÓÔÏÈο) Û˘ÌÂÚÈÂÏ‹ÊıËÛ·Ó ÛÙË ÌÂϤÙË. ∞fi ÙÔ˘˜ ·È‰›·ÙÚÔ˘˜, ÔÈ 40 (85%) ·ÛÎÔ‡Û·Ó ÂχıÂÚÔ Â¿ÁÁÂÏÌ·, ÔÈ 5 (11%) ˘ËÚÂÙÔ‡Û·Ó Û ÓÔÛÔÎÔÌ›· Î·È ÔÈ 2 (4%) Û ΤÓÙÚ· ÀÁ›·˜. ∫·Ù¿ÏÏËÏ· ‰È·Ï‡Ì·Ù· ÂÓ˘‰¿ÙˆÛ˘ ıˆڋıËÎ·Ó ·˘Ù¿ Ô˘ ÂÚȤ¯Ô˘Ó Na 35-90 mEq/l, ˘‰·Ù¿ÓıڷΘ 1-3% ÎÈ ¤¯Ô˘Ó ˆÛ̈ÙÈÎfiÙËÙ· <310 mmol/l. ∆¤ÙÔÈ· ‰È·Ï‡Ì·Ù· Â›Ó·È ÙÔ ÚÔÙÂÈÓfiÌÂÓÔ ·fi ÙoÓ WHO ÎÈ ·˘Ù¿ Ô˘ ΢ÎÏÔÊÔÚÔ‡Ó ÛÙÔ ÂÌfiÚÈÔ. ∆Ô ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ Ô˘ ¯ÚËÛÈÌÔÔÈ‹Û·Ì (¶›Ó·Î·˜ 1) ¯ˆÚ›ÛÙËΠ۠٤ÛÛÂÚ· ̤ÚË Ô˘ ·ÊÔÚÔ‡Ó: ·) ™ÙË ÁÓÒÛË ÙÔ˘ ·È‰›·ÙÚÔ˘ ÁÈ· ÙȘ ÚÔÙ¿ÛÂȘ Ù˘ Û‡Á¯ÚÔÓ˘ ‚È‚ÏÈÔÁÚ·Ê›·˜ Û¯ÂÙÈο Ì ÙË £∂™. ‚) ™ÙË ÛÙ¿ÛË ÙˆÓ ·È‰›·ÙÚˆÓ ·¤Ó·ÓÙÈ ÛÙË £∂™ ηıÒ˜ Î·È ÛÙËÓ ÚÔı˘Ì›· ·Ô‰Ô¯‹˜ Ô˘ ıˆÚÔ‡Ó ÔÈ ·È‰›·ÙÚÔÈ fiÙÈ ‰Â›¯ÓÔ˘Ó ÔÈ ·ÛıÂÓ›˜, ÔÈ ÔÈÎÔÁ¤ÓÂȤ˜ ÙÔ˘˜ Î·È ÙÔ ÓÔÛËÏ¢ÙÈÎfi ÚÔÛˆÈÎfi ÁÈ· ÙÔ ›‰ÈÔ ı¤Ì·. Á) ™ÙËÓ Ú·ÎÙÈ΋ Ô˘ ¯ÚËÛÈÌÔÔÈÔ‡Ó ÔÈ ·È‰›·ÙÚÔÈ ÁÈ· ÙË ¯ÔÚ‹ÁËÛË ÙˆÓ ··Ú·›ÙËÙˆÓ ˘ÁÚÒÓ Î·È ÙËÓ Â·Ó·Û›ÙÈÛË ÛÙÔ Ô͇ ‰È·ÚÚÔ˚Îfi Û‡Ó‰ÚÔÌÔ Ì ·Ê˘‰¿ÙˆÛË. ‰) ∆¤ÏÔ˜, ÂϤÁ¯ıËÎÂ Â¿Ó ÔÈ ·È‰›·ÙÚÔÈ ıˆÚÔ‡Ó ÛËÌ·ÓÙÈ΋ ÙË £∂™ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ÛÙËÓ Î·ıËÌÂÚÈÓ‹ È·ÙÚÈ΋ Ú¿ÍË. °È· Ó· ‰ÈÂÚ¢ӋÛÔ˘Ì ÙÔ˘˜ Èı·ÓÔ‡˜ ·Ó·ÛÙ·ÏÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ £∂™, Û˘Û¯ÂÙ›Û·Ì ÛÙ·ÙÈÛÙÈο ÙÔ˘˜ ÙÔÌ›˜ Ù˘ ÁÓÒÛ˘ Î·È Ù˘ ÛÙ¿Û˘ ÙˆÓ ·È‰›·ÙÚˆÓ Ì ÙË Û˘Ó‹ıË Ú·ÎÙÈ΋ Ô˘ ·ÎÔÏÔ˘ıÔ‡Ó, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙË ‰ÔÎÈÌ·Û›· ¯2 ‹ ÙË ‰ÔÎÈÌ·Û›· Fisher’s Exact.

∞ÔÙÂϤÛÌ·Ù· °ÓÒÛË Ù˘ ıÂÚ·›·˜ ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ·. ∏ ÂÏ·ÊÚÈ¿ ·Ê˘‰¿ÙˆÛË ıˆڋıËΠÔÚıÒ˜ ˆ˜ ¤Ó‰ÂÈÍË ¯ÔÚ‹ÁËÛ˘ £∂™ ·fi ÙÔ 98% ÙˆÓ ·È‰›·ÙÚˆÓ Î·È Ë Ì¤Û˘ ‚·Ú‡ÙËÙ·˜ ·Ê˘‰¿ÙˆÛË ·fi ÙÔ 70%. ∆ËÓ ÚÔÏËÙÈ΋ ¯ÔÚ‹ÁËÛË ‰È·Ï˘Ì¿ÙˆÓ ÂÓ˘‰¿ÙˆÛ˘ ·Ó·ÁÓˆÚ›˙ÂÈ ˆ˜ ¤Ó‰ÂÈÍË ÙÔ 47% ÙˆÓ ·È‰›·ÙÚˆÓ. √È ÂÚÈÛÛfiÙÂÚÔÈ (53%), ÛˆÛÙ¿, ‰Â ıˆÚÔ‡Ó ÙÔ˘˜ Â̤ÙÔ˘˜ ˆ˜ ·ÓÙ¤Ó‰ÂÈÍË £∂™, ÂÓÒ ÙÔ 4% ıˆÚ›, Ï·Óı·Ṳ̂ӷ, ÙË Ì¤ÙÚÈ· ·Ê˘‰¿ÙˆÛË ˆ˜ ·ÓÙ¤Ó‰ÂÈÍË ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ·. ªfiÓÔ ÙÔ 6%, ÔÚı¿, Â¤ÏÂÍ ÙÔ Ó¿ÙÚÈÔ Î·È ÙÔ˘˜ ˘‰·Ù¿ÓıڷΘ ˆ˜ Ù· Û˘ÛÙ·ÙÈο ÙˆÓ ‰È·Ï˘Ì¿ÙˆÓ Ô˘ Ú¤ÂÈ Ó· ‚Ú›ÛÎÔÓÙ·È Û ηٿÏÏËÏË ·Ó·ÏÔÁ›·, ÚÔÎÂÈ̤ÓÔ˘ Ó· ·ÔÚÚÔÊÒÓÙ·È Î·Ï‡ÙÂÚ·. ∆¤ÏÔ˜, ÙÔ 77%,

Paediatriki 2001;64:49-54

ÔÚı¿, ıˆÚ› fiÙÈ Ë Â·Ó·Û›ÙÈÛË Ú¤ÂÈ Ó· ·Ú¯›˙ÂÈ ·fi ÙÔ ÚÒÙÔ 24ˆÚÔ ¤Ó·Ú͢ Ù˘ ‰È¿ÚÚÔÈ·˜. ™Ù¿ÛË ¤Ó·ÓÙÈ Ù˘ ıÂÚ·›·˜ ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· ™¯Â‰fiÓ fiÏÔÈ ÔÈ ·È‰›·ÙÚÔÈ (90%) Ô˘ Û˘ÌÌÂÙ›¯·Ó ÛÙË ÌÂϤÙË, ¤¯Ô˘Ó ıÂÙÈ΋ ÛÙ¿ÛË ˆ˜ ÚÔ˜ ÙË £∂™ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ·Ê˘‰¿ÙˆÛ˘ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÔͤˆÓ ‰È·ÚÚÔ˚ÎÒÓ Û˘Ó‰ÚfiÌˆÓ ÛÙ· ·È‰È¿ Î·È ÙËÓ Â·ÎfiÏÔ˘ıË ·ÔÊ˘Á‹ Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘˜ Û ÓÔÛÔÎÔÌ›Ô. ∆Ô 90% ÙˆÓ ·È‰›·ÙÚˆÓ ÈÛÙ‡ÂÈ fiÙÈ ÔÈ ÔÈÎÔÁ¤ÓÂȘ Î·È ÔÈ ·ÛıÂÓ›˜ ÚÔÙÈÌÔ‡Ó ÙË £∂™. ∆Ô 13% ÈÛÙ‡ÂÈ fiÙÈ Ë ÂÎ·›‰Â˘ÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ Â›Ó·È ‰‡ÛÎÔÏË ‹ ·‰‡Ó·ÙË Î·È fiÙÈ ÔÈ ÁÔÓ›˜ ‰ÂÓ ¤¯Ô˘Ó ÙË ‰˘Ó·ÙfiÙËÙ· Ó· ·ÓÙÈÌÂÙˆ›ÛÔ˘Ó ·ÔÙÂÏÂÛÌ·ÙÈο ÙÔ ·È‰› ÛÙÔ Û›ÙÈ. ∆Ô 4% ÈÛÙ‡ÂÈ fiÙÈ Ë £∂™ ‰ÂÓ Â›Ó·È ·ÛÊ·Ï‹˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈ΋ ̤ıÔ‰Ô˜. √È ÂÚÈÛÛfiÙÂÚÔÈ ·È‰›·ÙÚÔÈ (76%) ·fi fiÛÔ˘˜ ‰È·ı¤ÙÔ˘Ó ‹ ‰È¤ıÂÙ·Ó ÓÔÛËÏ¢ÙÈÎfi ÚÔÛˆÈÎfi, ıˆÚÔ‡Ó fiÙÈ ·˘Ùfi ÚÔÙÈÌ¿ ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ·Ê˘‰¿ÙˆÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì £∂™. ∆¤ÏÔ˜, ÙÔ 4% ·˘ÙÒÓ ıˆÚ› fiÙÈ Ë £∂™ ¤¯ÂÈ ÌÂÁ¿ÏÔ ÔÈÎÔÓÔÌÈÎfi ÎfiÛÙÔ˜ ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Î·È ÙȘ ÔÈÎÔÁ¤ÓÂȤ˜ ÙÔ˘˜. ∏ ıÂÚ·›· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· ÛÙËÓ Ú¿ÍË √ ¶›Ó·Î·˜ 2 ÂÚÈÏ·Ì‚¿ÓÂÈ ÙȘ ··ÓÙ‹ÛÂȘ Ô˘ ¤‰ˆÛ·Ó ÔÈ ·È‰›·ÙÚÔÈ ÛÙȘ ÂÚˆÙ‹ÛÂȘ ÂχıÂÚ˘ ¤ÎÊÚ·Û˘, ÁÈ· ÙËÓ Î·ıËÌÂÚÈÓ‹ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ÔÍ›· ‰È¿ÚÚÔÈ·. ŸÏÔÈ ÔÈ ·È‰›·ÙÚÔÈ ÚÔÙ›ÓÔ˘Ó, ·Ó¿ÏÔÁ· Ì ÙËÓ ÂÚ›ÙˆÛË, ÙfiÛÔ ÙËÓ ÂÓ˘‰¿ÙˆÛË p.o. fiÛÔ Î·È ÙËÓ i.v. ÂÓ˘‰¿ÙˆÛË. ∞ÎfiÌË, fiÏÔÈ ÔÈ ·È‰›·ÙÚÔÈ ·ÓÙÈÌÂÙˆ›˙Ô˘Ó ÙËÓ ÂÏ·ÊÚÈ¿ ·Ê˘‰¿ÙˆÛË Ì ÂÓ˘‰¿ÙˆÛË p.o. Î·È ÌfiÓÔ ÙÔ 2% ıˆÚ› ÙËÓ ÌÂÙÚ›Ô˘ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛË ·ÓÙ¤Ó‰ÂÈÍË ÂÓ˘‰¿ÙˆÛ˘ p.o. ŸÌˆ˜, ÙÔ 43% ıˆÚ› ÙÔ˘˜ Â̤ÙÔ˘˜ ·ÓÙ¤Ó‰ÂÈÍË. √È ÂÚÈÛÛfiÙÂÚÔÈ ·È‰›·ÙÚÔÈ (87%) Û˘ÓÙ·ÁÔÁÚ·ÊÔ‡Ó Î·Ù¿ÏÏËÏ· ‰È·Ï‡Ì·Ù· ÁÈ· ÂÓ˘‰¿ÙˆÛË p.o., ·Ó Î·È ÙÔ 17% ÚÔÙ›ÓÂÈ ÌÂÚÈΤ˜ ÊÔÚ¤˜ Î·È ÌË ÂÓ‰ÂÈÎÓ˘fiÌÂÓ· ˘ÁÚ¿ (¯˘ÌÔ‡˜, ·Ó·„˘ÎÙÈο, ÙÛ¿È, sport drinks). ªfiÓÔ ÙÔ 2% ‰›ÓÂÈ Ô‰ËÁ›Â˜ ·Ú·Û΢‹˜ ‰È·Ï‡Ì·ÙÔ˜ ÂÓ˘‰¿ÙˆÛ˘ ·fi ˘ÏÈο Ô˘ Û˘Ó‹ıˆ˜ ˘¿Ú¯Ô˘Ó ÛÙÔ Û›ÙÈ (·Ï¿ÙÈ, ˙¿¯·ÚË ÎÏ.). ∆Ô 13% ÚÔÙ›ÓÂÈ ·ÔÎÏÂÈÛÙÈο ÌË ÂÓ‰ÂÈÎÓ˘fiÌÂÓ· ˘ÁÚ¿. ŸÌˆ˜, ÌfiÓÔ ÙÔ 13% ÚÔÙ›ÓÂÈ ÛÙÔ˘˜ ÁÔÓ›˜ Ó· ¯ÚËÛÈÌÔÔÈÔ‡Ó ‰ÔÛÔÌÂÙÚËÙ‹ (ÎÔ˘Ù·Ï¿ÎÈ ‹ Û‡ÚÈÁÁ·), ¤ÙÛÈ ÒÛÙ ӷ ÂϤÁ¯ÂÙ·È Ô Ú˘ıÌfi˜ Î·È Ë ÔÛfiÙËÙ· ÙˆÓ ˘ÁÚÒÓ Ô˘ ¯ÔÚËÁÔ‡ÓÙ·È. √È ÂÚÈÛÛfiÙÂÚÔÈ (55%), ÔÚı¿ Û˘ÛÙ‹ÓÔ˘Ó Â·Ó·Û›ÙÈÛË Ì¤Û· ÛÙÔ ÚÒÙÔ 24ˆÚÔ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘. √È ÈÔ Û˘¯Ó¿ ÚÔÙÂÈÓfiÌÂÓ˜ ÙÚÔʤ˜ ÁÈ· Â·Ó·Û›ÙÈÛË Â›Ó·È ÙÔ ÌËÙÚÈÎfi Á¿Ï·, ÙÔ ·Ú·ÈˆÌ¤ÓÔ Á¿Ï· ÁÈ· Ù· ‚Ú¤ÊË Ô˘ ‰Â ıËÏ¿˙Ô˘Ó

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 52

¶·È‰È·ÙÚÈ΋ 2001;64:49-54

Paediatriki 2001;64:49-54

¶›Ó·Î·˜ 2: ¶Ú·ÎÙÈ΋ ÙˆÓ ·È‰È¿ÙÚˆÓ ∂Ӊ›ÍÂȘ ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· ∂Ï·ÊÚÈ¿ ·Ê˘‰¿ÙˆÛË ª¤ÙÚÈ· ·Ê˘‰¿ÙˆÛË µ·ÚÈ¿ ·Ê˘‰¿ÙˆÛË ∞ÓÙÂӉ›ÍÂȘ ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· µ·ÚÈ¿ ·Ê˘‰¿ÙˆÛË ∫ÔÈÓˆÓÈÎÔ› / ÔÈÎÔÓÔÌÈÎÔ› ÏfiÁÔÈ ŒÌÂÙÔÈ ™˘ÓÙ·ÁÔÁÚ·ÊÔ‡ÌÂÓ· ˘ÁÚ¿ Almora Soparyx Orisel Risolyte ¢È¿Ï˘Ì· WHO/Unicef Sport drinks Ã˘ÌÔ›, ÙÛ¿È, ·Ó·„˘ÎÙÈο ƒ˘ıÌfi˜ ¯ÔÚ‹ÁËÛ˘ ªÈÎÚ‹ ÔÛfiÙËÙ· ™˘¯Ó‹ ¯ÔÚ‹ÁËÛË ∫Ô˘Ù·Ï¿ÎÈ / Û‡ÚÈÁÁ· ÃÚfiÓÔ˜ Â·Ó·Û›ÙÈÛ˘ <24 ÒÚ˜ 24-48 ÒÚ˜ >48 ÒÚ˜

100% 85% 6% 68% 30% 43% 70% 40% 26% 26% 2% 21% 25% 4% 15% 13% 55% 40% 5%

Î·È ÙÔ Ú‡˙È, ÙÔ ÎÔÙfiÔ˘ÏÔ, Ë ·Ù¿Ù· Î·È Ë Ì·Ó¿Ó· ÁÈ· Ù· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿. ∆¤ÏÔ˜, ۯ‰fiÓ fiÏÔÈ (98%) ˘ÈÔıÂÙÔ‡Ó ÙÔ fiÙÈ Ë £∂™ ¤¯ÂÈ ÛËÌ·ÓÙÈ΋ ı¤ÛË ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ÛÙËÓ Î·ıËÌÂÚÈÓ‹ È·ÙÚÈ΋ Ú¿ÍË. ∞Ó·ÛÙ·ÏÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· °È· Ó· ‰ÈÂÚ¢ӋÛÔ˘Ì ·Ó·ÛÙ·ÏÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÌÔ‰›˙Ô˘Ó ÙÔ˘˜ ·È‰›·ÙÚÔ˘˜ Ó· ÚÔÙ›ÓÔ˘Ó ÙËÓ ÂÓ‰ÂÈÎÓ˘fiÌÂÓË ÂÓ˘‰¿ÙˆÛË p.o., ¤ÁÈÓÂ Û˘Û¯¤ÙÈÛË Ù˘ ÁÓÒÛ˘ Î·È Ù˘ ÛÙ¿Û˘ ÙˆÓ ·È‰È¿ÙÚˆÓ Ì ÙËÓ Ú·ÎÙÈ΋ Ô˘ ·ÎÔÏÔ˘ıÔ‡Ó. ™ËÌ·ÓÙÈ΋ ÛÙ·ÙÈÛÙÈ΋ Û¯¤ÛË (P<0,01 ) ‚Ú¤ıËΠÌÂٷ͇ Ù˘ ¯ÔÚ‹ÁËÛ˘ Ù˘ ÂÓ˘‰¿ÙˆÛ˘ p.o., Û ·È‰È¿ ÌfiÓÔ Ì ÂÏ·ÊÚÈ¿ ‹ Ì ÂÏ·ÊÚÈ¿ Î·È Ì¤ÙÚÈ· ·Ê˘‰¿ÙˆÛË Î·È Ù˘ ÛÙ¿Û˘ ÙˆÓ ·È‰›·ÙÚˆÓ Û¯ÂÙÈο Ì ÙËÓ Â˘ÎÔÏ›· ÂÎ·›‰Â˘Û˘ ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ ÛÙËÓ ÂÓ˘‰¿ÙˆÛË p.o. ŒÙÛÈ, ÙÔ 75% ÙˆÓ ·È‰È¿ÙÚˆÓ Ô˘ ıˆÚ› ‡ÎÔÏË ÙËÓ ÂÎ·›‰Â˘ÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ Û˘ÓÈÛÙ¿ ÂÓ˘‰¿ÙˆÛË p.o. Î·È Û ·È‰È¿ Ì ÌÂÙÚ›Ô˘ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛË, ÂÓÒ ÌfiÓÔ ÙÔ 25% fiÛˆÓ ıˆÚ› ‰‡ÛÎÔÏË ÙËÓ ÂÎ·›‰Â˘ÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ ·ÎÔÏÔ˘ı› ÙËÓ ›‰È· Ú·ÎÙÈ΋. ¢ÂÓ ‚Ú¤ıËΠÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ Û¯¤ÛË ÌÂٷ͇ Ù˘ ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ· Û ·È‰È¿ Ìfi-

52

ÓÔ Ì ÂÏ·ÊÚÈ¿ ‹ Ì ÂÏ·ÊÚÈ¿ Î·È Ì¤ÙÚÈ· ·Ê˘‰¿ÙˆÛË Î·È Ù˘ ÛÙ¿Û˘ ‹ Ù˘ ÁÓÒÛ˘ ÙˆÓ ·È‰›·ÙÚˆÓ, fiˆ˜ ·˘Ù‹ ÂϤÁ¯ÂÙ·È Ì ÙÔ ÂÚˆÙËÌ·ÙÔÏfiÁÈÔ. ™˘˙‹ÙËÛË ∏ ÂÈÎfiÓ· Ô˘ ·ÔÙ˘ÒÓÂÙ·È ÛÙËÓ ÂÚÁ·Û›· Ì·˜ Â›Ó·È ÌfiÓÔ ÂÓ Ì¤ÚÂÈ Û‡ÌʈÓË Ì ÙȘ ÚÔÙ¿ÛÂȘ ÙˆÓ ‰ÈÂıÓÒÓ ÔÚÁ·ÓÈÛÌÒÓ Î·È ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›·, ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜. H Ú·ÎÙÈ΋ ÙˆÓ ·È‰›·ÙÚˆÓ Ù˘ £ÂÛÛ·Ï›·˜ Û ۯ¤ÛË Ì ÙÔ˘˜ Û˘Ó·‰¤ÏÊÔ˘˜ ÙÔ˘˜ ÙˆÓ ∏.¶.∞., Û˘ÓÙ¿ÛÛÂÙ·È Ôχ ÂÚÈÛÛfiÙÂÚÔ Ì ÙȘ ÚÔÙ¿ÛÂȘ Ù˘ ‰ÈÂıÓÔ‡˜ ‚È‚ÏÈÔÁÚ·Ê›·˜ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ (24, 25). øÛÙfiÛÔ, fiˆ˜ ‰Â›¯ÓÔ˘Ó Î·È ¿ÏϘ ÌÂϤÙ˜ (24-26), ˘¿Ú¯Ô˘Ó ÂÚÈıÒÚÈ· ηχÙÂÚ˘, ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚ˘ Î·È ÏÈÁfiÙÂÚÔ Ô‰˘ÓËÚ‹˜ ·ÓÙÈÌÂÙÒÈÛ˘, ¯ˆÚ›˜ Ó· ˘ÔÛÙÔ‡Ó ÔÈ ÔÈÎÔÁ¤ÓÂȘ ÙˆÓ ÌÈÎÚÒÓ ·ÛıÂÓÒÓ ÙËÓ ·Ó·ÛٿوÛË Ô˘ Û˘ÓÂ¿ÁÂÙ·È Ë ÂÈÛ·ÁˆÁ‹ ÙÔ˘˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô. ∏ ÂÏ·ÊÚÔ‡ ‚·ıÌÔ‡ ·Ê˘‰¿ÙˆÛË ·ÓÙÈÌÂÙˆ›˙ÂÙ·È ·fi ÙÔ 100% Î·È Ë Ì¤ÙÚÈ· ·fi ÙÔ 85% ÙˆÓ ·È‰›·ÙÚˆÓ Ì ÂÓ˘‰¿ÙˆÛË p.o. ∆Ô 87% Û˘ÓÙ·ÁÔÁÚ·Ê› (fi¯È ·ÔÎÏÂÈÛÙÈο) ÂÓ‰ÂÈÎÓ˘fiÌÂÓ· ‰È·Ï‡Ì·Ù·, ÙÔ 98% ˘ÈÔıÂÙ› ÙË ¿Ô„Ë fiÙÈ Ë £∂™ η٤¯ÂÈ ÛËÌ·ÓÙÈ΋ ı¤ÛË ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ ‰È¿ÚÚÔÈ·˜ ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ú·ÎÙÈ΋ ÙÔ˘ È·ÙÚ›Ԣ, ÂÓÒ Ë ÏÂÈÔÓfiÙËÙ· ÙˆÓ ·È‰›·ÙÚˆÓ Â·Ó·ÛÈÙ›˙ÂÈ Ù· ·È‰È¿ Ì ηٿÏÏËϘ ÙÚÔʤ˜. ∞ÓÙ›ıÂÙ·, ÙÔ 43% ÙˆÓ ·È‰›·ÙÚˆÓ ÚÔÙÈÌ¿ ÙËÓ i.v. ÂÓ˘‰¿ÙˆÛË fiÙ·Ó Û˘Ó˘¿Ú¯Ô˘Ó ¤ÌÂÙÔÈ. ∆Ô 45%, Â›Û˘, ‰ÂÓ Â·Ó·ÛÈÙ›˙ÂÈ ÙÔ˘˜ ÌÈÎÚÔ‡˜ ·ÛıÂÓ›˜ ̤۷ ÛÙÔ ÚÒÙÔ 24ˆÚÔ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘, ÂÓÒ ÙÔ 87% ‰ÂÓ ÚÔÙ›ÓÂÈ ÛÙÔ˘˜ ÁÔÓ›˜ Ó· ¯ÚËÛÈÌÔÔÈÔ‡Ó ‰ÔÛÔÌÂÙÚËÙ‹ (ÎÔ˘Ù·Ï¿ÎÈ ‹ Û‡ÚÈÁÁ·), ÒÛÙ ӷ ÂϤÁ¯ÂÙ·È Ô Ú˘ıÌfi˜ Î·È Ë ÔÛfiÙËÙ· ÙˆÓ ˘ÁÚÒÓ Ô˘ ¯ÔÚËÁÔ‡ÓÙ·È. ∞fi Ù· ‰È·Ï‡Ì·Ù· Ô˘ ΢ÎÏÔÊÔÚÔ‡Ó ÛÙÔ ÂÌfiÚÈÔ, Û˘ÓÙ·ÁÔÁÚ·ÊÂ›Ù·È Û˘¯ÓfiÙÂÚ· ÙÔ ‰È¿Ï˘Ì· Ì ÙË ¯·ÌËÏfiÙÂÚË ÂÚÈÂÎÙÈÎfiÙËÙ· Û ӿÙÚÈÔ (35 mEq/l), Ú¿ÁÌ· Ô˘ ÙÔ Î·ıÈÛÙ¿ ÏÈÁfiÙÂÚÔ ·ÔÙÂÏÂÛÌ·ÙÈÎfi ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ·Ê˘‰¿ÙˆÛ˘. ∆· ˘ÁÚ¿ Ô˘ ˘¿Ú¯Ô˘Ó ÛÙÔ Û›ÙÈ Î·È Û˘¯Ó¿ ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ÙËÓ ÚfiÏË„Ë Î·È ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ·Ê˘‰¿ÙˆÛ˘ (˙ˆÌfi˜ ÎÚ¤·ÙÔ˜ ‹ ÎÔÙfiÔ˘ÏÔ˘, ¯˘ÌÔ›, ÙÛ¿È, ·ÓıÚ·ÎÔ‡¯· ·Ó·„˘ÎÙÈο) ‰ÂÓ ÂӉ›ÎÓ˘ÓÙ·È, ÁÈ·Ù› ¿ÏÏ· ÂÚȤ¯Ô˘Ó ÂÏ¿¯ÈÛÙ˜ Î·È ¿ÏÏ· Ôχ ÌÂÁ¿Ï˜ ÔÛfiÙËÙ˜ Ó·ÙÚ›Ô˘ Î·È Ù· ÂÚÈÛÛfiÙÂÚ· Â›Ó·È ˘ÂÚˆÛ̈ÙÈο, ÂȉÂÈÓÒÓÔÓÙ·˜ ¤ÙÛÈ ÙȘ ‰È¿ÚÚÔȘ (27) (¶›Ó·Î·˜ 3). ∏ ‚ÂÏÙ›ˆÛË ÙˆÓ ÁÓÒÛÂˆÓ ÙˆÓ ·È‰›·ÙÚˆÓ ÈÛÙ‡ԢÌ fiÙÈ ı· Ô‰ËÁÔ‡Û Û ηχÙÂÚË ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÌÈÎÚÒÓ ·ÛıÂÓÒÓ. √È 3 ·È‰›·ÙÚÔÈ Ô˘ ÁÓˆÚ›˙Ô˘Ó Ù· Û˘ÛÙ·ÙÈο ÙˆÓ ‰È·Ï˘Ì¿ÙˆÓ Ô˘ Ú¤-


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 53

¶·È‰È·ÙÚÈ΋ 2001;64:49-54

Paediatriki 2001;64:49-54

¶›Ó·Î·˜ 3. ™‡ÓıÂÛË ÌË ÂÓ‰ÂÈÎÓ˘fiÌÂÓˆÓ ˘ÁÚÒÓ ÁÈ· ÙË ıÂÚ·›· ÂÓ˘‰¿ÙˆÛ˘ ·fi ÙÔ ÛÙfiÌ·. ÀÁÚ¿ ∑ˆÌfi˜ ÎÚ¤·ÙÔ˜ ∑ˆÌfi˜ ÎfiÙ·˜ Ã˘Ìfi˜ Ì‹ÏÔ˘ ∆Û¿È (¯ˆÚ›˜ ˙¿¯·ÚË) ∫fiη-ÎfiÏ· 7up Gatorade

Na+ (mEq/l)

K+ (mEq/l)

À‰·Ù¿ÓıڷΘ (gr/100ml)

øÛ̈ÙÈÎfiÙËÙ· (mmol/l)

110-248 140-251 3 1,7 5,5 20

2,5-17 1,5-8,2 32 5 0,1 2 3

6 11 7,4 4,6

300-390 380-500 700 550 550 330

ÂÈ Ó· ‚Ú›ÛÎÔÓÙ·È Û ηٿÏÏËÏË ·Ó·ÏÔÁ›· ÁÈ· Ó· ·ÔÚÚÔÊÒÓÙ·È Î·Ï‡ÙÂÚ·, Û˘ÓÙ·ÁÔÁÚ·ÊÔ‡Ó ·ÔÎÏÂÈÛÙÈο ÂÓ‰ÂÈÎÓ˘fiÌÂÓ· ‰È·Ï‡Ì·Ù·, ÂÓÒ ÙÔ 64% ·fi ÙÔ˘˜ ˘fiÏÔÈÔ˘˜ 44 Ú¿ÙÙÂÈ ÙÔ ›‰ÈÔ. ∞Ó Î·È ÙÔ ·ÔÙ¤ÏÂÛÌ· ·˘Ùfi ‰ÂÓ Â›Ó·È ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈÎfi (P=0,54) Â›Ó·È ÂÓ‰ÂÈÎÙÈÎfi fiÙÈ Ë ÁÓÒÛË Ù˘ £∂™ Ô‰ËÁ› ÛÙË ¯Ú‹ÛË Î·Ù¿ÏÏËÏˆÓ ‰È·Ï˘Ì¿ÙˆÓ ÂÓ˘‰¿ÙˆÛ˘. √ ÈÔ Î·Ù¿ÏÏËÏÔ˜, fï˜, ÙÚfiÔ˜ ·Ú¤Ì‚·Û˘ ÁÈ· ÙËÓ ·ÚÔ¯‹ Ù˘ ··ÈÙÔ‡ÌÂÓ˘ ÁÓÒÛ˘ Â›Ó·È ·ÌÊÈÛ‚ËÙ‹ÛÈÌÔ˜. ∞ÚÎÂÙ¤˜ ÌÂϤÙ˜ (28-29) ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ Ë ·Ï‹ ·ÚÔ¯‹ ¤ÓÙ˘Ô˘ ˘ÏÈÎÔ‡ ‰ÂÓ ¤¯ÂÈ ÈηÓÔÔÈËÙÈÎfi ·ÔÙ¤ÏÂÛÌ· ÛÙËÓ ·ÏÏ·Á‹ Ù˘ Ú·ÎÙÈ΋˜ ÙÔ˘ ÁÈ·ÙÚÔ‡, ÂÓÒ ¤¯ÂÈ ÌÈÎÚfi ‹ ηӤӷ ·ÔÙ¤ÏÂÛÌ· ÛÙË ‰ÈfiÚıˆÛË ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜ ÙÔ˘ ·ÛıÂÓÔ‡˜. ∏ ·ÏÏ·Á‹ Ù˘ Ú·ÎÙÈ΋˜, fiÙ·Ó ÚÔÙ›ÓÔÓÙ·È Ó¤Â˜ ̤ıÔ‰ÔÈ, ¤¯ÂÈ ·Ô‰Âȯı› ÈÛÙÔÚÈο ‰‡ÛÎÔÏË (30) Î·È Ì¿ÏÈÛÙ· fiÛÔÓ ·ÊÔÚ¿ ÛÙË ıÂÚ·›· Ù˘ ‰È¿ÚÚÔÈ·˜, ÁÈ· ÙËÓ ÔÔ›· Ë i.v. ·ÓÙÈÌÂÙÒÈÛË ¤¯ÂÈ, ÁÈ· ÌÂÁ¿ÏÔ ‰È¿ÛÙËÌ·, ÂÊ·ÚÌÔÛı› Ì ÂÈÙ˘¯›·. ∏ ÌÂϤÙË Ì·˜ ¤‰ÂÈÍ fiÙÈ Ô ·ÚÈıÌfi˜ ÙˆÓ ·È‰ÈÒÓ Ô˘ Ì ÙË ‰È¿ÁÓˆÛË Ì¤ÙÚÈ·˜ ·Ê˘‰¿ÙˆÛ˘ ηٷϋÁÔ˘Ó ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ÁÈ· Ó· ÙÔ˘˜ ¯ÔÚËÁËı› i.v. ÂÓ˘‰¿ÙˆÛË, ‰ÂÓ ÂÍ·ÚÙ¿Ù·È ·fi ÙË ÁÓÒÛË ·ÏÏ¿ ÌfiÓÔ ·fi ÙË ÛÙ¿ÛË ÙˆÓ ·È‰›·ÙÚˆÓ ·¤Ó·ÓÙÈ ÛÙËÓ Â˘ÎÔÏ›· ‹ ÌË ÂÎ·›‰Â˘Û˘ ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ ÛÙË ¯ÔÚ‹ÁËÛË Ù˘ £∂™. √ ·Ú¿ÁÔÓÙ·˜ ·˘Ùfi˜ ‰ÂÓ Â›Ó·È Â‡ÎÔÏÔ Ó· ÙÚÔÔÔÈËı›, ÒÛÙ ӷ Ô‰ËÁ‹ÛÂÈ ÛÙË Ì›ˆÛË ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ·È‰ÈÒÓ Ô˘ ·‰ÈηÈÔÏfiÁËÙ· ηٷϋÁÔ˘Ó ÛÙÔ ÓÔÛÔÎÔÌ›Ô. √È ·ÚÌfi‰ÈÔÈ ÊÔÚ›˜ Ú¤ÂÈ Ó· ˘ÈÔıÂÙ‹ÛÔ˘Ó ÚÔÛ¿ıÂȘ Ô˘ ı· ·ԂϤÔ˘Ó ÛÙË ‰È¿‰ÔÛË Ù˘ ÛˆÛÙ‹˜ Â͈ÓÔÛÔÎÔÌÂȷ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì ÔÍ›· ‰È¿ÚÚÔÈ·, Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· ÂȉÈο ‰È·Ï‡Ì·Ù· ÂÓ˘‰¿ÙˆÛ˘ Î·È ÙËÓ ¤ÁηÈÚË ÛˆÛÙ‹ Â·Ó·Û›ÙÈÛË (£∂™). µÈ‚ÏÈÔÁÚ·Ê›· 1. Snyder JD, Merson MH. The magnitude of the global problem of acute diarrhoeal disease: a review of active surveillance data. Bull WHO 1982;60:605-613. 2. Shultz S, Zalusky R. Ion transport in isolated rabbit ileum. The interaction between active sodium and active sugar

transport. J Gen Physiol 1964;47:1043. 3. Binder HJ. Sodium transport across isolated human jejunum. Gastroenterology 1974;67:231. 4. Listernick R, Zieserl E, Davis AT. Outpatient oral rehydration in the United States. Am J Dis Child 1986;140:211-215. 5. Phillips M, Kumate-Rodriquez J, Mota-Hernandez F. Costs of treating diarrhoea in a children’s hospital in Mexico City. Bull WHO 1989;67:373-380. 6. Santosham M, Greenough WB 3rd. Oral rehydration therapy: a global perspective. J Pediatr 1991;118:S44-S51. 7. Santosham M, Brown KH, Sack RB. Oral rehydration therapy and dietary therapy for acute childhood diarrhea. Pediatr Rev 1987;8:273-278. 8. Goepp JG, Katz SA. Oral rehydration therapy. Am Fam Physician 1993;47:843-851. 9. Richards L, Claeson M, Pierce NF. Management of acute diarrhea in children: lessons learned. Paediatr Infect Dis J 1993;12:5-9. 10. ƒÒÌ· ∂. §¿ıË ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ‰È¿ÚÚÔÈ·˜. ¢ÂÏÙ›Ô ∞’ ¶·È‰. ∫ÏÈÓ. ¶·Ó/Ì›Ô˘ ∞ıËÓÒÓ 1981;28:315-320. 11. Duggan C, Lasche J, McCarty M, Mitchell K, Dershewitz R, Lerman SJ et al. Oral rehydration solution for acute diarrhea prevents subsequent unscheduled follow-up visits. Pediatrics 1999;104:3,e29. 12. Finberg L, Harper PA, Harrison HE, Sack RB. Oral rehydration for diarrhea. J Pediatr 1982;101:497-499. 13. Gracey M. Nutritional effects and management of diarrhea in infancy. Acta Paediatr Suppl 1999;430:110-126. 14. Avery M, Snyder J. Oral therapy for acute diarrhea. The underused simple solution. N Engl J Med 1990;323: 891-894. 15. Brown KH, MacLean WC Jr. Nutritional management of acute diarrhea: an appraisal of the alternatives. Pediatrics 1984;73:119-125. 16. Subcommittee on Nutrition and Diarrheal Disease Control Committee on International Nutritional Programs, Nutrition Research Council. Nutritional Management of Acute Diarrhea in Infants and Children. Washington, DC: National Academy Press; 1985. 17. Santosham M, Foster S, Reid R, Bertrando R, Yolken R, Bums B et al. Role of soy-based, lactose-free formula during treatment of acute diarrhea. Pediatrics 1985; 76:292-298. 18. Khin-Maung-v, Nyut-Nyut-Wai, Myo-Khin, Mu Mu Khin, Tin U Thane Toe. Effect on clinical outcome of breast

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feeding during acute diarrhea. Br Med J 1985;290:587-589. 19. Molla AM, Molla A, Nath SK, Khatun M. Food-based oral rehydration salt solution for acute childhood diarrhea. Lancet 1989;2:429-431. 20. Walker Smith JA, Sandhu BK, Isolauri E, Banchini G, van Caillie Bertrand M, Dias JA et al. Guidelines prepared by the ESPGAN working group on acute diarrhoea. Recommendations for feeding in childhood gastroenteritis. J Pediatr Gastroenterol Nutr 1997;24:619-620. 21. Burkhart DM. Management of acute gastroenteritis in children. Am Fam Physician 1999;60:2555-2566. 22. Brown KH, Lopez de Romana G, Graham GG, MacLean WC. Experience with mixture of wheat-noodles and casein in the initial dietary therapy of infants and young children with protein-calorie malnutrition of acute diarrhea. J Hum Nutr 1982;3A:354-366. 23. Mahalanabis D, Merson M. Development of an improved formulation of oral rehydration salts (ORS) with antidiarrheal and nutritional properties: a super ORS. In: Holmgren J, Lindberg A, Molly RR, eds. Development of Vaccines and Drugs Against Diarrhea. Lund, Sweden: Studentlitteratur; 1986. 24. Reis EC, Goepp JG, Katz S, Santosham M. Barriers to use of oral rehydration therapy. Pediatrics 1994;93:708-711. 25. Snyder JD. Use and Misuse of Oral Therapy for diarrhea: Comparison of US practices with American Academy of Pediatrics Recommendations. Pediatrics 1991;87:28-33. 26. Szajewska H, Hoekstra JH, Sandhu B. Management of

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27.

28.

29. 30.

acute gastroenteritis in Europe and the impact of the new recommendations: a multicenter study. The Working Group on acute Diarrhoea of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 2000;30;522-527. §·ÁÎÒÓ·–™Ù¿ıË ∂. ∞ÔÛÙÔÏ‹ ·ÚÚÒÛÙˆÓ Û ÓÔÛÔÎÔÌ›· ‰Â˘ÙÂÚÔ‚¿ıÌÈ·˜ Î·È ÙÚÈÙÔ‚¿ıÌÈ·˜ ÂÚ›ı·Ï„˘: ÂӉ›ÍÂȘ–ÚÔÂÙÔÈÌ·Û›·. ¢ÂÏÙ›Ô ∞’ ¶·È‰. ∫ÏÈÓ. ¶·Ó/Ì›Ô˘ ∞ıËÓÒÓ 1995;42(Û˘ÌÏËڈ̷ÙÈÎfi Ù‡¯Ô˜):71-76. Eisenberg JM. Doctors’ decisions and the cost of medical care: the reasons for doctors’ practice patterns and ways to change them. Ann Arbor, MI: Health Administration Press; 1986. Davis DA, Thomson MA, Oxman AD, Haynes RB. Evidence for the effectiveness of CME. JAMA 1992;286:1111-1117. Soumerai SB, McLaughlin TJ, Avorn J. Improving drug prescribing in primary care: a critical analysis of the experimental literature. Milbank Mem Fund Q Health Soc 1989;67:268-317.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 25-07-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 04-12-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∂ÌÌ·ÓÔ˘‹Ï ∞ÏÂÍfiÔ˘ÏÔ˜ ÃÚ˘ÛÔÛÙfiÌÔ˘ ™Ì‡ÚÓ˘ 2, ¶ÂÚÈÔ¯‹ ™Ù·ıÌÔ‡, 412 23 §¿ÚÈÛ·.


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∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞

Paediatriki 2001;64:55-58

ORIGINAL PAPER

∂›‰· §ÈÔÚˆÙ½Ó˘(·) Û 3298 ∂ÏÏËÓfiÔ˘Ï·, ËÏÈΛ·˜ 6-14 ÂÙÒÓ ∫. ∂. ™Ô‡ÏË1, °.∞. ∫·Ú›Î·˜2, ™. °·‚Ú›ÏË

Serum Lipoprotein (a) concentration in 3298 healthy school aged Greek children K.H. Schulpis1, G.A. Karikas2, S. Gavrili

¶ÂÚ›ÏË„Ë: ∏ §ÈÔÚˆÙ½ÓË (·), Lp(·), Â›Ó·È Ì›· ÏÈÔÚˆÙ½ÓË ÁÂÓÂÙÈο ÚÔÛ‰ÈÔÚÈṲ̂ÓË Î·ıÒ˜, Â›Û˘, ·Ú¿ÁˆÓ ÔÛÔÙÈο ηıÔÚÈṲ̂ÓÔ˜ Î·È Û˘Û¯ÂÙÈ˙fiÌÂÓÔ˜ Ì ÙËÓ ·Ó¿Ù˘ÍË Î·Ú‰È·ÁÁÂȷ΋˜ ÓfiÛÔ˘. ™ÎÔfi˜ Ù˘ ÂÚÁ·Û›·˜ Ì·˜ ‹Ù·Ó Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ ÂÈ¤‰ˆÓ Ù˘ Lp(·) Î·È Ë Î·Ù·ÓÔÌ‹ ÙÔ˘˜ Û ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ ·Ó¿ÏÔÁ· Ì ÙÔ Ê‡ÏÔ Î·È ÙËÓ ËÏÈΛ·, ÒÛÙ ӷ ¯ÚËÛÈ̇ÛÔ˘Ó ˆ˜ ÙÈ̤˜ ·Ó·ÊÔÚ¿˜ ΢ڛˆ˜ ÙÔ˘ ·È‰ÈÎÔ‡ ÏËı˘ÛÌÔ‡ 6-14 ÂÙÒÓ ÛÙËÓ ∂ÏÏ¿‰·. ∆Ô ‰Â›ÁÌ· ÙÔ˘ ÏËı˘ÛÌÔ‡ Ì·˜ ÂÚȤϷ‚ 3298 ·È‰È¿, 1590 ·ÁfiÚÈ· Î·È 1708 ÎÔÚ›ÙÛÈ·, ËÏÈΛ·˜ 6-14 ÂÙÒÓ ‰È·Ì¤ÓÔÓÙ· ÛÙËÓ ÂÚÈÔ¯‹ ÙÔ˘ ÓÔÌÔ‡ ∞ÙÙÈ΋˜. ∏ ̤ıÔ‰Ô˜ ̤ÙÚËÛ˘ Ù˘ Lp(·) ‹Ù·Ó ·ÓÔÛÔÂÓ˙˘ÌÈ΋. ∆· ·ÔÙÂϤÛÌ·Ù· Ì¿˜ ¤‰ÂÈÍ·Ó fiÙÈ Ô Ì¤ÛÔ˜ fiÚÔ˜ ÙˆÓ ÂÈ¤‰ˆÓ Ù˘ Î˘Ì·ÈÓfiÙ·Ó ·fi 153 ¤ˆ˜ 159 (mg/L) ÁÈ· Ù· ·ÁfiÚÈ· Î·È ·fi 146 ¤ˆ˜ 151 (mg/L) ÁÈ· Ù· ÎÔÚ›ÙÛÈ·. ∏ ‰È¿ÌÂÛË ÙÈÌ‹ Ù˘ ÌÂÙÚ‹ıËΠ·fi 133 ¤ˆ˜ 157 (mg/L) ÁÈ· Ù· ·ÁfiÚÈ· Î·È ·fi 100 ¤ˆ˜ 108 (mg/L) ÁÈ· Ù· ÎÔÚ›ÙÛÈ·. ™‡Ìʈӷ Ì ٷ Â˘Ú‹Ì·Ù¿ Ì·˜, Ù· Â›‰· Ù˘ Lp(·) ÛÙ· ∂ÏÏËÓfiÔ˘Ï· Û¯ÔÏÈ΋˜ ËÏÈΛ·˜, ‹Ù·Ó Ù· ¯·ÌËÏfiÙÂÚ· Ô˘ ¤¯Ô˘Ó ‰ËÌÔÛÈ¢ı› ̤¯ÚÈ Û‹ÌÂÚ·.

Abstract: Lipoprotein (a), Lp (a), is a lipoprotein which is genetically determined, as well as a factor quantitatively defined, that is associated with the development of cardiovascular disease. ∆he aim of this study was to measure the serum Lp(a) levels in Greek school-aged children and determine their centile distribution according to age and sex so that they can be used as reference points. We studied a total of 3298 children (1590 boys, 1708 girls) aged 614 yrs, all of whom resided in the greater Athens area. Lp(a) levels were evaluated using an enzyme linked immunorbent assay. Mean Lp(a) levels ranged from 153 to 159 mg/L for boys and from 146 to 151 mg/L for girls. The median levels ranged from 133 to 157 mg/L for boys and from 100 to 108 mg/L for girls. The results of our study indicate that the Lp(a) levels in the school-aged Greek children, were the lowest ever reported up to the present time.

§¤ÍÂȘ ÎÏÂȉȿ: §ÈÔÚˆÙ½ÓË (·), Lp(·), ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ.

Key words: Lp(a), Lipoprotein (a), centiles.

∂ÈÛ·ÁˆÁ‹ ∏ §ÈÔÚˆÙ½ÓË (·), Lp(·), ıˆÚÂ›Ù·È ¤Ó·˜ -·ÓÂÍ¿ÚÙËÙÔ˜ Ù˘ ‰È·ÙÚÔÊ‹˜- ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘

·Ó¿Ù˘Í˘ ÛÙÂÊ·ÓÈ·›·˜ ÓfiÛÔ˘ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹. ¶ÚÔÛ‰ÈÔÚ›ÛıËΠÁÈ· ÚÒÙË ÊÔÚ¿ ÛÙÔ ·ÓıÚÒÈÓÔ Ï¿ÛÌ· ·fi ÙÔÓ Berg (1), Ô˘ ¤‰ÂÈÍ fiÙÈ ÚfiÎÂÈÙ·È

1 πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, 11527 ∞ı‹Ó· 2 ªÔÓ¿‰· ¶·ÚÂÓÙÂÚÈ΋˜ ¢È·ÙÚÔÊ‹˜ Î·È º·ÚÌ·ÎÔÎÈÓËÙÈ΋˜ (ª∂¢º) ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·”, 11527 ∞ı‹Ó·

1 Institute of Child Health “St. Sophia” Children’s Hospital, Athens 2 Division of Parenteral Nutrition and Pharmacokinetics “St. Sophia” Children’s Hospital, Athens

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ÁÈ· Ì›· ·ÔÏÈÔÚˆÙ½ÓË (·) Î·È Ì›· ¯·ÌËÏ‹˜ ˘ÎÓfiÙËÙ·˜ ÏÈÔÚˆÙ½ÓË (LDL) (2). ∞ÚÎÂÙ¤˜ ÌÂϤÙ˜ ·¤‰ÂÈÍ·Ó fiÙÈ Ù· ·˘ÍË̤ӷ Â›‰· Ù˘ Lp(·) Û˘Ì‚¿ÏÏÔ˘Ó ÛÙËÓ ÂÌÊ¿ÓÈÛË ·ıËڈ̿وÛ˘ Î·È ÂÁÎÂÊ·ÏÈÎÒÓ ÂÂÈÛÔ‰›ˆÓ (3,4). ŸÙ·Ó Ù· Â›‰¿ Ù˘ ˘ÂÚ‚·›ÓÔ˘Ó Ù· 300 mg/L, ÙfiÙÂ Ô Î›Ó‰˘ÓÔ˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Î·Ú‰È·ÁÁÂÈ·ÎÒÓ ÂÂÈÛÔ‰›ˆÓ ‰ÈÏ·ÛÈ¿˙ÂÙ·È (5,6). ∏ ÂÓÙfiÈÛË Ù˘ Lp(·) fi¯È ÌfiÓÔ ÛÙ· ÙÔȯÒÌ·Ù· ·ÁÁ›ˆÓ, ·ÏÏ¿ ÛÙ· ÌÔۯ‡̷ٷ, ¤‰ˆÛ ÙÔÓ ¯·Ú·ÎÙËÚÈÛÌfi Ù˘ ˆ˜ ·ıËڈ̷ÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·, ·Ô‰ÂÈÎÓ‡ÔÓÙ·˜ fiÙÈ Ë ·ÔÏÈÔÚˆÙ½ÓË (·) ÚÔÛÔÌÔÈ¿˙ÂÈ ÌÔÚȷο Ì ÙÔ Ï·ÛÌÈÓÔÁfiÓÔ. Œ¯ÂÈ ıÚÔÌ‚ÔÁÂÓÂÙÈΤ˜ Î·È ·ıËڈ̷ÙÔÁfiÓ˜ ȉÈfiÙËÙ˜ (7,8). ™ÙÔ˘˜ ÂÓ‹ÏÈÎÔ˘˜ ·Ú·ÙËÚ‹ıËΠ·‡ÍËÛË Ù˘ Lp(·) ÌÂÙ¿ ·fi ¤ÌÊÚ·ÁÌ· ÙÔ˘ Ì˘Ôηډ›Ô˘, ¯ÂÈÚÔ˘ÚÁÈ΋ Â¤Ì‚·ÛË, fiˆ˜ Î·È Î·Ù¿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË (9, 10). ™‡Ìʈӷ Ì ÙË ÌÂϤÙË Ù˘ Bogalusa ·Ú·ÙËÚ‹ıËÎ·Ó ‰È·ÊÔÚ¤˜ ÛÙ· Â›‰· Ù˘ Lp(·) fiÛÔÓ ·ÊÔÚ¿ ÛÙÔ Ê‡ÏÔ Î·È ÙÔ ÛÙ¿‰ÈÔ Ù˘ ‹‚˘ (11,12). ∞Ó ÎÈ ¤Ó·˜ ÂÚÈÔÚÈṲ̂ÓÔ˜ ·ÚÈıÌfi˜ ÌÂÏÂÙÒÓ ‰ÂÓ ÂȂ‚·›ˆÛ ÙË Û¯¤ÛË ÌÂٷ͇ ÂÈ¤‰ˆÓ Lp(·) Î·È ÛÙÂÊ·ÓÈ·›·˜ ÓfiÛÔ˘, Èı·ÓfiÓ ¤ÓÂη Ù˘ ‰È·ÊÔÚÂÙÈ΋˜ ÌÂıÔ‰ÔÏÔÁ›·˜ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËΠ(13). ™Â ·ÛıÂÓ›˜ Ì ÔÈÎÔÁÂÓ‹ ˘ÂÚ¯ÔÏËÛÙÂÚÈÓ·ÈÌ›· ÌÂÙÚ‹ıËÎ·Ó ˘„ËÏfiÙÂÚ· Â›‰· Lp(·), Û˘ÁÎÚÈÓfiÌÂÓ· Ì ÂΛӷ ÙˆÓ ˘ÁÈÒÓ (14,15). ∞fi ¿ÏϘ ÌÂϤÙ˜ ‰È¢ÎÚÈÓ›ÛÙËΠfiÙÈ Ë ÂÌÊ¿ÓÈÛË ÛÙÂÊ·ÓÈ·›·˜ ÓfiÛÔ˘ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÙÔÓ ÁfiÓÔ LDLR (16). Œ¯ÔÓÙ·˜ ˘fi„Ë fiÙÈ Ë ÛÙ¿ıÌË Ù˘ Lp(·) ÂËÚ¿˙ÂÙ·È ÁÂÓÂÙÈο, ۯ‰ȿ۷Ì ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ÂÎÙ›ÌËÛ˘ ÙˆÓ ÂÈ¤‰ˆÓ Ù˘ ÏÈÔÚˆÙ½Ó˘ Û ¤Ó· ÌÂÁ¿ÏÔ ·ÚÈıÌfi ∂ÏÏËÓÔ·›‰ˆÓ Û¯ÔÏÈ΋˜ ËÏÈΛ·˜ 6-14 ÂÙÒÓ, ÒÛÙ ӷ ‰È·ÌÔÚÊÒÛÔ˘Ì ÙȘ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ (ÙÈ̤˜ ·Ó·ÊÔÚ¿˜) ÙÔ˘ Ó·ÓÈÎÔ‡ Ì·˜ ÏËı˘ÛÌÔ‡.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ ∞fi ÙËÓ 1Ë π·ÓÔ˘·Ú›Ô˘ 1996 ¤ˆ˜ ÙËÓ 31Ë ¢ÂÎÂÌ‚Ú›Ô˘ 1997, ÂÏ‹ÊıËÛ·Ó ‰Â›ÁÌ·Ù· ·›Ì·ÙÔ˜ ·fi 3298 ˘ÁÈ‹ ∂ÏÏËÓfiÔ˘Ï· Ô˘ ηÙÔÈÎÔ‡Û·Ó ÛÙÔ ÓÔÌfi ∞ÙÙÈ΋˜. ∞fi ·˘Ù¿, Ù· 1590 ‹Ù·Ó ·ÁfiÚÈ· Î·È Ù· 1708 ÎÔÚ›ÙÛÈ·. ™Â οı ÂÚ›ÙˆÛË, ·Ó·ÁÚ·ÊfiÙ·Ó Ë ËÏÈΛ· ÙÔ˘ ·È‰ÈÔ‡, fiÙ·Ó ÁÈÓfiÙ·Ó Ë ÎÏÈÓÈ΋ ÂͤٷÛË Î·È Ë ·ÈÌÔÏË„›·, ηÙfiÈÓ ¤ÁÁÚ·Ê˘ ÏËÚÔÊfiÚËÛ˘ ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘˜. ∏ ÛÙ¿ıÌË Ù˘ Lp(·) ÛÙÔ ·›Ì· ÚÔÛ‰ÈÔÚ›ÛÙËΠ̠kit-test ÂÌÔÚ›Ô˘ (·ÓÔÛÔÏÔÁÈ΋ ̤ıÔ‰Ô˜ Elisa, Biopool AB, Umea, Sweden) (17,18). ∏ ηٷ¯ÒÚËÛË ÙˆÓ ÙÈÌÒÓ ·Ó¿ ËÏÈΛ· Î·È Ê‡ÏÔ Û ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ ¤ÁÈÓ Û ÚfiÁÚ·ÌÌ· SPSS 7,5 Ì ÙË ‚Ô‹ıÂÈ· ËÏÂÎÙÚÔÓÈÎÔ‡ ˘ÔÏÔÁÈÛÙ‹.

∞ÔÙÂϤÛÌ·Ù· √È Ì¤Û˜ Î·È ÔÈ ‰È¿ÌÂÛ˜ ÙÈ̤˜ ·Ó¿ ËÏÈΛ· Î·È Ê‡ÏÔ Î·Ù·ÓÂÌË̤Ó˜ Û ÂηÙÔÛÙÈ·›Â˜ ·Ó·ÏÔÁ›Â˜, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1. √È Ì¤Û˜ ÙÈ̤˜ Ù˘ Lp(·) ÌÂÙÚ‹ıËÎ·Ó ·fi 152 ¤ˆ˜ 159 (mg/L) ÁÈ· Ù· ·ÁfiÚÈ· Î·È ·fi 146 ¤ˆ˜ 151 (mg/L) ÁÈ· Ù· ÎÔÚ›ÙÛÈ·. √È ‰È¿ÌÂÛ˜ ÙÈ̤˜ Î˘Ì·›ÓÔÓÙ·Ó ·fi 133 ¤ˆ˜157 (mg/L) ÁÈ· Ù· ·ÁfiÚÈ· Î·È ·fi 100 ¤ˆ˜ 108 (mg/L) ÁÈ· Ù· ÎÔÚ›ÙÛÈ·. ™˘˙‹ÙËÛË ªÂÙ¿ ÙËÓ ·Ó·Î¿Ï˘„Ë Ù˘ Lp(·) ·fi ÙoÓ Berg (1) Î·È ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜ (19,20,21), ·¤‰ÂÈÍ·Ó fiÙÈ ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ Ù˘ ÛÙÔ Ï¿ÛÌ· Â›Ó·È ÔÛÔÙÈο ηıÔÚÈṲ̂Ó˜. √È ÁÂÓÂÙÈο ηıÔÚÈṲ̂Ó˜ ÙÈ̤˜ Ù˘ Lp(·) Ô‰‹ÁËÛ·Ó ÛÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ Â›Ó·È ¯·Ú·ÎÙËÚÈÛÙÈΤ˜ ÁÈ· οı ¯ÒÚ· (20,21). ∞ÎfiÌË, Ë Ê˘ÏÂÙÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· Ô˘ ‚Ú¤ıËΠ۠ÔÏϤ˜ ¯ÒÚ˜ ÙÔ˘ ‰˘ÙÈÎÔ‡ ÎfiÛÌÔ˘ (∞ÁÁÏ›·, °·ÏÏ›·) ·ÚÔ˘Û›·Û ÔÏϤ˜ ·ÓÙÈÊ¿ÛÂȘ, .¯. ÔÈ πÓ‰Ô› Ô˘ ‰È·Ì¤ÓÔ˘Ó ÛÙÔ §ÔÓ‰›ÓÔ ¤¯Ô˘Ó ˘„ËÏfiÙÂÚ· Â›‰· Lp(·), Û˘ÁÎÚÈ-

¶›Ó·Î·˜ 1. ∂›‰· §ÈÔÚˆÙ½Ó˘ Lp(·) (mg/L) Û 3298 ∂ÏÏËÓfiÔ˘Ï· ËÏÈΛ·˜ 6-14 ÂÙÒÓ. ∏ÏÈΛ·

AÁfiÚÈ·

¡

6-8 8 -10 10 - 12 10 - 14

405 414 388 383

™‡ÓÔÏÔ ∫ÔÚ›ÙÛÈ·

∂ηÙ. £¤ÛÂȘ (Lp(a))

(¯Ú.)

™∞

¢È·Ì

10

25

50

75

95

153 152 159 157

90 105 104 116

133 134 135 157

85 96 90 95

109 97 95 100

133 138 130 139

176 195 180 240

264 257 264 284

146 149 150 151

107 99 108 120

100 106 107 108

91 99 77 89

100 104 95 108

115 126 120 128

256 240 256 260

270 268 278 298

1.590 6-8 8 - 10 10 - 12 12 - 14

394 405 415 494

™‡ÓÔÏÔ

1.708

°ÂÓÈÎfi ™‡ÓÔÏÔ ™∞: ™Ù·ıÂÚ‹ ∞fiÎÏÈÛË

3.298

56

ª¤ÛË


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¶›Ó·Î·˜ 2. ª¤ÛË ∆ÈÌ‹ §ÈÔÚˆÙ½Ó˘ (·) (mg/L) fiˆ˜ ·Ó·Ê¤ÚÂÙ·È ÛÙË Û¯ÂÙÈ΋ ‚È‚ÏÈÔÁÚ·Ê›·. ÃÒÚ· ¡ÈÁËÚ›· °·ÏÏ›· π·ˆÓ›· ∏¶∞ (ÃÈÔ‡ÛÙÔÓ-§Â˘ÎÔ›) (¡. √ÚÏ¿ÓË-¡¤ÁÚÔÈ) (™ÈοÁÔ-§Â˘ÎÔ›) (ªÔÁηÏÔ‡Û·-§Â˘ÎÔ›) (ªÔÁηÏÔ‡Û·-¡¤ÁÚÔÈ) ∂§§∞™

ÕÓ‰Ú˜

°˘Ó·›Î˜

µÈ‚ÏÈÔÁÚ·Ê›·

256 220 170 157 239 160 170 240 152

243 245 150 173 260 150 168 230 127

(22) (23) (24) (25) (25) (25) (11) (11)

∆· Â›‰· Ù˘ §ÈÔÚˆÙ½Ó˘ (·) ÌÂÙÚ‹ıËÎ·Ó Ì ̤ıÔ‰Ô ELISA.

ÓfiÌÂÓ· Ì ÂΛӷ ¿ÏÏˆÓ ÂıÓÈÎÔÙ‹ÙˆÓ Ô˘ ‰È·Ì¤ÓÔ˘Ó ÛÙËÓ ›‰È· ÂÚÈÔ¯‹ (20). √ ˘ÔÏÔÁÈÛÌfi˜ ÙˆÓ ÂÈ¤‰ˆÓ Ù˘ Lp(·) Û ۯÔÏÈ΋˜ ËÏÈΛ·˜ ∂ÏÏËÓfiÔ˘Ï· ¤ÁÈÓ ÂÈϤÔÓ, Ì ÛÎÔfi Ó· ÂÓÙÔ›ÛÔ˘Ì ¤Ó·Ó ·ÎfiÌË ÁÂÓÂÙÈÎfi ·Ú¿ÁÔÓÙ· ÎÈÓ‰‡ÓÔ˘, Ô˘ ı· ÌÔÚÔ‡Û ӷ ÚÔηϤÛÂÈ ¤ÌÊÚ·ÁÌ· ÙÔ˘ Ì˘Ôηډ›Ô˘ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹. ∆· ‰Â›ÁÌ·Ù· ·›Ì·ÙÔ˜ ÂÏ‹ÊıËÛ·Ó ÌÂÙ¿ 12ˆÚË ÓËÛÙ›· ·ÚfiÙÈ ˘¿Ú¯Ô˘Ó ÌÂϤÙ˜ Ô˘ ‰ÂÓ ‰È·ÊÔÚÔÔÈÔ‡Ó Ù· Â›‰· Ù˘ Lp(·) ÌÂÙ¿ ·fi Ê·ÁËÙfi (17). √È Rifai Î·È Û˘Ó (21), Û ÌÂϤÙË ÙÔ˘˜ ·Ó·Ê¤ÚÔ˘Ó fiÙÈ ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ Lp(·) Û ‚Ú¤ÊË, ·È‰È¿ Î·È ÂÓ‹ÏÈΘ ÛÙȘ ∏¶∞ ‰Â›¯ÓÔ˘Ó Ì›· ·‡ÍËÛË ·fi ÙË Á¤ÓÓËÛË ¤ˆ˜ ÙÔ 2o ¤ÙÔ˜ ˙ˆ‹˜ Î·È Â›Ó·È ›‰È˜ Î·È ÛÙËÓ ÂÓËÏÈΛˆÛË. √È Ì¤Û˜ Î·È ‰È¿ÌÂÛ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ Ù˘ Lp(·) Û 3298 ˘ÁÈ‹ ·È‰È¿, Ô˘ ÂÍÂÙ¿ÛıËÎ·Ó Ì ÙËÓ ›‰È· ̤ıÔ‰Ô, ‹Ù·Ó Î·È ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ ¯·ÌËϤ˜. ™˘ÓÂÒ˜, ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ Lp(·) Û ·È‰È¿ ËÏÈΛ·˜ 6-14 ÂÙÒÓ Ô˘ ‹Ù·Ó ۯ‰fiÓ ·ÌÂÙ¿‚ÏËÙ˜, ı· ÌÔÚÔ‡Û·Ó Ó· ıˆÚËıÔ‡Ó ·ÚfiÌÔȘ Î·È ÛÙÔ˘˜ ÂÓ‹ÏÈÎÔ˘˜ (21). °È· ÙÔÓ ›‰ÈÔ ÏfiÁÔ, ıˆڋ۷Ì ˆ˜ ·ÓÒÙ·ÙÔ Ê˘ÛÈÔÏÔÁÈÎfi fiÚÈÔ Ù˘ Lp(·) ÙËÓ ÙÈÌ‹ ÙˆÓ 300 mg/L. √È Ì¤Û˜ ÙÈ̤˜, fiˆ˜ ÂÚÈÁÚ¿ÊËÎ·Ó Û ‰È¿ÊÔÚ˜ ÂÚÁ·Û›Â˜ Î·È ·Ó·Ê¤ÚÔÓÙ·È Û ‰È¿ÊÔÚ˜ ÂıÓfiÙËÙ˜, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 2. ∏ ¤ÏÏÂÈ„Ë ‰ÈfiÚıˆÛ˘ ÙˆÓ ÙÈÌÒÓ Ù˘ Lp(·), Ô˘ ‰ÂÓ ¤¯Ô˘Ó ηÓÔÓÈ΋ ηٷÓÔÌ‹ (non gaussian) Î·È Ë ¯Ú‹ÛË ‰È·ÊfiÚˆÓ ÂȉÒÓ ÙÈÌÒÓ ·Ó·ÊÔÚ¿˜, οÓÂÈ ÙË ÌÂٷ͇ ÙÔ˘˜ Û‡ÁÎÚÈÛË ÙˆÓ ÙÈÌÒÓ ‰‡ÛÎÔÏË ·ÏÏ¿ ·Ô‰ÂÎÙ‹, ·ÊÔ‡ Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ ÂÈ¤‰ˆÓ Ù˘ Lp(·) ¤ÁÈÓ Ì ÙËÓ ›‰È· ̤ıÔ‰Ô (ELISA). ∂›Û˘, ÔÈ ·Ú·ÙËÚÔ‡ÌÂÓ˜ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ Ì¤ÛˆÓ Î·È ‰È·Ì¤ÛˆÓ ÙÈÌÒÓ Ù˘ Lp(a) Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ù˘ ÚÔÂÚÈÁÚ·Ê›۷˜ ÌË Î·ÓÔÓÈ΋˜ ηٷÓÔÌ‹˜ ÙˆÓ ÙÈÌÒÓ Ù˘ ÏÈÔÚˆÙ½Ó˘. ∞ÎfiÌË, ·ÊÔ‡ ÔÈ ÌÂÙÚ‹ÛÂȘ Ù˘ Lp(·) ÛÙ· ∂ÏÏËÓfiÔ˘Ï· ¤ÁÈÓ·Ó Î¿Ùˆ ·fi ÙȘ ›‰È˜ Û˘Óı‹Î˜ ÛÙÔ ›‰ÈÔ ÂÚÁ·ÛÙ‹ÚÈÔ, ı· ÌÔÚÔ‡Û ӷ Á›ÓÂÈ ·Ô‰ÂÎÙ‹ Ë Û‡-

ÁÎÚÈÛ‹ ÙÔ˘˜ Ì ÂΛӘ ¿ÏÏˆÓ ¯ˆÚÒÓ. ªÂ ‚¿ÛË Ù· ·ÔÙÂϤÛÌ·Ù·, Ë Lp(·) ÛÙË ¯ÒÚ· Ì·˜ Ê·›ÓÂÙ·È Ó· ‚Ú›ÛÎÂÙ·È ÛÙ· ¯·ÌËÏfiÙÂÚ· Â›‰·, Û˘ÁÎÚÈÓfiÌÂÓ· Ì ÂΛӷ ¿ÏÏˆÓ ¯ˆÚÒÓ (¶›Ó·Î·˜ 2). ™˘ÌÂÚ·ÛÌ·ÙÈο ı· ÌÔÚÔ‡Û ӷ ϯı›, fiÙÈ ·ÚfiÏÔ Ô˘ ÔÈ Î·Ú‰È·ÁÁÂȷΤ˜ ÓfiÛÔÈ ·ÔÙÂÏÔ‡Ó ÙËÓ ÚÒÙË ·ÈÙ›· ı·Ó¿ÙÔ˘ ÛÙËÓ ∂ÏÏ¿‰·, Ù· ·ÚfiÓÙ· Â˘Ú‹Ì·Ù· ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÂΛӷ Ô˘ ·ÊÔÚÔ‡Ó ÛÙ· Â›‰· ÙˆÓ ÏÈȉ›ˆÓ ·È‰ÈÒÓ ›‰È·˜ ËÏÈΛ·˜ (26), ı· ÌÔÚÔ‡Û·Ó Ó· Ô‰ËÁ‹ÛÔ˘Ó ÛÙÔ Û˘Ì¤Ú·ÛÌ· fiÙÈ Ô ÂÏÏËÓÈÎfi˜ ÏËı˘ÛÌfi˜ ¢ڛÛÎÂÙ·È Û ÏÂÔÓÂÎÙÈ΋ ı¤ÛË Û ۯ¤ÛË Ì ÙÔÓ ÚÔÂÚ¯fiÌÂÓÔ Î›Ó‰˘ÓÔ ·fi ÙȘ ÚÔ·Ó·ÊÂÚfiÌÂÓ˜ ‚ÈÔ¯ËÌÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜. ∂˘¯·ÚÈÛٛ˜ ∂˘¯·ÚÈÛÙԇ̠ÙËÓ Î. ∫·ÏÏÈfiË ∆·ÛÛÔÔ‡ÏÔ˘ ÁÈ· ÙËÓ ÂÈÌÂÏË̤ÓË ‰·ÎÙ˘ÏÔÁÚ¿ÊËÛË Ù˘ ÂÚÁ·Û›·˜ ·˘Ù‹˜.

µÈ‚ÏÈÔÁÚ·Ê›· 1. Berg R. A new serum type system in man – the Lp (a) system. Acta Pathol Microbiol Scand 1963;59: 369–382. 2. Utermann G. The Mysteries of lipoprotein (a). Science 1989;246: 904–910. 3. Dahlen GH, Guyton JR, Attar M. Farmer JA, Kauz JA. Association of levels of lipoprotein (a), plasma lipids and others lipoproteins with coronary artery disease documented by angiography. Circulation 1986;14:758–765. 4. Rhonds GG, Dahlen GH, Bera K, Morton NE, Dannenberg A. Lp(a) lipoprotein as a risk factor in myocardial infarction. JAMA 1986;256:2540–2544. 5. Murai A, Miyahara I, Fujinoto N, Matsuda M. Lp(a) lipoprotein as a risk factor for coronary heart disease and cerebral infarction. Atherosclerosis 1986;59:199–206. 6. Armstrong VW, Cremer P, Eberle E. The association between serum Lp(a) concentrations and angiographically assessed coronary atherosclerosis: dependence on serum LDL levels. Atherosclerosis 1986;62:249–257. 7. Walton KW, Hitchens I, Magnani HN, Khan M. A study of methods of identification and estimation of Lp(a)

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18. Fless GM, Snyder ML, Doetsch R. Enzyme Linked immunoassay for Lp(a). J Lipid Res 1989;30:651–62. 19. Bowden JF, Pritchard PH, Frohlich JJ. Lp(a) concentration and apo (a) isoform size. Relation to the presence of coronary artery disease in familial hypercholesterolemia. Arterioscler Thromb 1994;14:1561-1568. 20. Krempler G, Kostner G, Bolzano K. Lipoprotein (a) is not a metabolic product of other lipoproteins containing apoprotein B. Biochim Biophys Acta 1979;575:63–70. 21. Rifai N, Heiss G, Doetsch K. Lipoprotein (a) at birth, in blacks and whites. Atherosclerosis 1992;92:123–129. 22. Kesteloot H, Oviasu VO, Obasohan AO. Serum lipid and apolipoprotein levels in a Nigerian population trait. Atherosclerosis 1989;78:33–38. 23. Janhiainen H, Koskinen P, Ehnholm C. Lipoprotein (a) and coronary heart disease risk. A case – control study of the Helsinki. Heart study participants. Atherosclerosis 1991;89:59-67. 24. Taro Ohji, Chiba K, Kohri T, Yamagishi M. Serum lipoprotein (a) in healthy Japanese children 5 yrs. of age. Acta Paediatr Jpn 1993;35:302–306. 25. Para H, Luyeye J, Bouramne J. Black, white differences in serum Lipoprotein (a) levels. Clin Chim Acta 1987;168:27–31. 26. Schulpis KH, Karikas GA. Serum cholesterol and triglycerides distribution in 7767 school–aged Greek children. Pediatrics 1998;101:861–864.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 30-06-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 01-12-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∫ÏÂÔ¿ÙÚ· ™Ô‡ÏË πÓÛÙÈÙÔ‡ÙÔ ÀÁ›·˜ ÙÔ˘ ¶·È‰ÈÔ‡ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∞Á›· ™ÔÊ›·», 11527 ∞ı‹Ó·


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∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞

Paediatriki 2001;64:59-64

ORIGINAL PAPER

∆ÔÈ΋ ·ÓÙÈÊÏÂÁÌÔÓ҉˘ ·ÁˆÁ‹ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÔÍ›·˜ Ï·Ú˘ÁÁÔÙÚ·¯ÂÈÔ‚ÚÔÁ¯›Ùȉ·˜ ¶. ™Ù·ÌÔ‡Ï˘1, Ã. ÃÚ˘Û·ÓıfiÔ˘ÏÔ˜2, ª. ∂ÌÔÚÈ¿‰Ô˘3, ¡. ∆Û¤˘4, ∂. °ÂˆÚÁÈ¿‰Ô˘5, ¡. ª·ÁÎÏ·‚¤Ú·˜6

Topical anti-inflammatory therapy in the management of acute laryngotracheitis P. Stamoulis1, C. Chryssanthopoulos2, M. Emporiadou3, N. Tsepis4, E. Georgiadou5, N. Maglaveras6

¶ÂÚ›ÏË„Ë: ¶ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ·ԉ›ÍÂÈ fiÙÈ Ë ÂÓ‰ÔÌ˘˚΋ ¯ÔÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ (¢ª) Û Â͈ÓÔÛÔÎÔÌÂÈ·ÎÔ‡˜ ·ÛıÂÓ›˜ Ì ÔÍ›· Ï·Ú˘ÁÁÔÙÚ·¯ÂÈÔ‚ÚÔÁ¯›Ùȉ· (§∆µ) ‹È·˜-̤ÙÚÈ·˜ ‚·Ú‡ÙËÙ·˜ Û¯ÂÙ›˙ÂÙ·È Ì ÂÏ¿ÙÙˆÛË Ù˘ ‚·Ú‡ÙËÙ·˜ Ù˘ ÓfiÛÔ˘ ̤۷ Û 24 ÒÚ˜. ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ Ì·˜ ‹Ù·Ó Ó· ‰È·ÈÛÙˆı› ·Ó Ë ÙÔÈ΋ ·ÓÙÈÊÏÂÁÌÔÓ҉˘ ıÂÚ·›· Ì ·ÂÚÔÔÈË̤ÓË ‰ÈÚÔÈÔÓÈ΋ ÌÂÎÏÔÌÂı·˙fiÓË (¢¶ª) Â›Ó·È ÂÍ›ÛÔ˘ ‹ ÂÚÈÛÛfiÙÂÚÔ ·ÔÙÂÏÂÛÌ·ÙÈ΋ ÛÙË ıÂÚ·›· Â͈ÓÔÛÔÎÔÌÂÈ·ÎÒÓ ·ÛıÂÓÒÓ Ì ‹ÈÔ˘-̤ÙÚÈÔ˘ ‚·ıÌÔ‡ ÔÍ›·-§∆µ. ™ÙËÓ ·ÚÔ‡Û· ‰ÈÏ‹ Ù˘ÊÏ‹ Û˘ÁÎÚÈÙÈ΋ ÌÂϤÙË ÌÂÏÂÙ‹ıËÎ·Ó 62 ·È‰È¿ ËÏÈΛ·˜ ÌÂٷ͇ 6 ÌËÓÒÓ Î·È 5 ÂÙÒÓ, ÛÙ· ÔÔ›· ¯ÔÚËÁ‹ıËΠ›Ù ·‰ÚÂÓ·Ï›ÓË (LE) Ì ÙË Ì¤ıÔ‰Ô Ù˘ ÓÂÊÂÏÔÔ›ËÛ˘, ›Ù ÌÈ· ÂÊ¿·Í ÂÓ‰ÔÌ˘˚΋ xoÚ‹ÁËÛË ‰ÂÍ·ÌÂı·˙fiÓ˘ (¢ª) 0,6mg/Kg B™ (max 8mg), ›Ù ·ÂÚÔÔÈË̤ÓË ¢¶ª 200 mcg Ì ·ÂÚÔı¿Ï·ÌÔ. ∂ÎÙÈÌ‹ıËÎ·Ó Ô ‚·ıÌfi˜ ‰‡ÛÓÔÈ·˜ (µ¢),Ô ·ÚÈıÌfi˜ ÙˆÓ ·Ó·ÓÔÒÓ (∞∞) Î·È ÙˆÓ Î·Ú‰È·ÎÒÓ ÛʇÍÂˆÓ (∫™), Ë ·ÚÙËÚȷ΋ ›ÂÛË (∞¶) Î·È Ô ÎÔÚÂÛÌfi˜ Ù˘ Ô͢·ÈÌÔÛÊ·ÈÚ›Ó˘ (SO2) Û ¯ÚfiÓÔ˘˜ 0, 15, 30, 60, 90 & 120 ÏÂÙ¿ Ù˘ ÒÚ·˜ ÌÂÙ¿ ÙË ıÂÚ·›·. √È ·ÛıÂÓ›˜ Î·È ÙˆÓ ÙÚÈÒÓ ıÂÚ·¢ÙÈÎÒÓ ÔÌ¿‰ˆÓ ·ÚÔ˘Û›·Û·Ó ÛËÌ·ÓÙÈ΋ ÂÏ¿ÙÙˆÛË ÙÔ˘ µ¢ ÛÙÔ Ù¤ÏÔ˜ ÙÔ˘ ¯ÚfiÓÔ˘ ·Ú·ÎÔÏÔ‡ıËÛ˘. ªÂÙ¿ ÙËÓ ·ÔÌ¿ÎÚ˘ÓÛ‹ ÙÔ˘˜ ·fi Ù· Â͈ÙÂÚÈο È·ÙÚ›· (∂π) ηÓ›˜ ·fi ·˘ÙÔ‡˜ ‰ÂÓ Â·Ó‹Ïı ̤۷ ÛÙȘ ÂfiÌÂÓ˜ 24 ÒÚ˜. ∏ ÔÌ¿‰· Ù˘

Abstract: Recent studies have demonstrated that the intramuscular administration of dexamethasone in children in the outpatient department with acute viral laryngotracheitis (AVL) of mild to moderate severity, is associated with a reduction in the severity of illness within 24 hours after treatment. The purpose of our study was to determine if topical anti-inflammatory therapy with aerolized beclomethazone dipropionate-(BD) is similarly or even more effective in the outpatient management of mild to moderate severity AVL. Sixty two children aged 6 months to 5 years ,who came to the emergency room (ER) with AVL and a croup score of at least 2 (range 0 to 10), were assigned, in a randomised double-blind fashion, to receive either aerolized L-epinephrine, or a single intramuscular injection of dexamethasone 0,6 mg/kg, or aerolized BD 200 mcg via aerochamber, before discharge from the ER. Croup score (CS), heart rate (HR),blood pressure (BP),respiratory rate (RR) and oxygen saturation (SO2) were recorded before treatment and at 15, 30, 60, 90 and 120 minutes after treatment. Patients in all groups demonstated a significant transient reduction of the CS at the end of the observation period. After discharge from the ER none of the patients returned for additional therapy during the next 24 hours. The L-epinephrine group

1 ¶·È‰›·ÙÚÔ˜, ∫.À. ™ÎÈ¿ıÔ˘, ™ÎÈ¿ıÔ˜ 2 ¶·È‰›·ÙÚÔ˜-∞ÏÏÂÚÁÈÔÏfiÁÔ˜, £ÂÛ/Ó›ÎË 3 ∞Ó·Ï. ηıËÁ‹ÙÚÈ· ¶·È‰È·ÙÚÈ΋˜, 4Ë ¶·È‰È·ÙÚÈ΋ ÎÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞, ∞¶£ 4 ¶·È‰›·ÙÚÔ˜, ¡ÔÛÔÎÔÌÂ›Ô “°. °ÂÓÓËÌ·Ù¿˜” £ÂÛ/Ó›ÎË 5 Œ‰Ú· ™Ù·ÙÈÛÙÈ΋˜, ∞¶£ 6 ∞Ó·ÏËÚˆÙ‹˜ ηıËÁËÙ‹˜, ¤‰Ú· π·ÙÚÈ΋˜ ™Ù·ÙÈÛÙÈ΋˜, ∞¶£

1 Pediatrician, Health Center, Skiathos 2 Pediatric-Allergiologist, Thessaloniki 3 Assoc. Professor of Pediatrics, 4th Pediatric Clinic, AHEPA Hospital, Thessaloniki 4 Pediatrician,“G. Gennimatas” Hospital, Thessaloniki 5 Department of Statistics, Aristotelion University of Thessaloniki 6 Assoc. Professor, Department of Statistics, Aristotelion University of Thessaloniki

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¶·È‰È·ÙÚÈ΋ 2001;64:59-64

Paediatriki 2001;64:59-64

LE ÂÌÊ¿ÓÈÛ ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË ÙˆÓ ·Ú·Ì¤ÙÚˆÓ µ¢, ∫™ Î·È ∞∞ Û ۯ¤ÛË Ì ÙȘ ¿ÏϘ ‰‡Ô ÔÌ¿‰Â˜. ¢ÂÓ ‰È·ÈÛÙÒıËΠÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÛÙȘ ·ÓÙ›ÛÙÔȯ˜ ·Ú·Ì¤ÙÚÔ˘˜ ÌÂٷ͇ ÙˆÓ ÔÌ¿‰ˆÓ Ù˘ ¢ª Î·È Ù˘ ¢¶ª. ™˘ÌÂÚ·ÛÌ·ÙÈο Ë ¯ÔÚ‹ÁËÛË ÓÂÊÂÏÔÔÈË̤Ó˘ LE ˘ÂÚ¤¯ÂÈ ıÂÚ·¢ÙÈο Ù˘ ÂÓ‰ÔÌ˘˚΋˜ ¯ÔÚ‹ÁËÛ˘ ¢ª Î·È Ù˘ ·ÂÚÔÔÈË̤Ó˘ ¢¶ª ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÂÏ·ÊÚÈ¿˜ Î·È ÌÂÙÚ›·˜ ‚·Ú‡ÙËÙ·˜ ∞∆µ. øÛÙfiÛÔ, Ë ¯Ú‹ÛË Ù˘ ¢¶ª ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË Â͈ÓÔÛÔÎÔÌÂÈ·ÎÒÓ ·ÛıÂÓÒÓ Ì ÈÔÁÂÓ‹ ÔÍ›· §∆µ (croup) Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ÂÏ¿ÙÙˆÛË Ù˘ ‚·Ú‡ÙËÙ·˜ Ù˘ ÓfiÛÔ˘ ̤۷ ÛÙÔ ÂfiÌÂÓÔ 24ˆÚÔ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜. ∏ ·Ú·¿Óˆ ̤ıÔ‰Ô˜ ¯ÔÚ‹ÁËÛ˘ ÙÔÈ΋˜ ·ÓÙÈÊÏÂÁ̈ÓÒ‰Ô˘˜ ·ÁˆÁ‹˜ ·ÔÙÂÏ› ÌÈ· ·ÔÙÂÏÂÛÌ·ÙÈ΋, ·ÛÊ·Ï‹, ·ÓÒ‰˘ÓË Î·È ÔÈÎÔÓÔÌÈ΋ ıÂÚ·›· Ù˘ ÔÍ›·˜ §∆µ ÛÙ· Â͈ÙÂÚÈο È·ÙÚ›· ‹ ÛÙÔ Û›ÙÈ ÙÔ˘ ·ÛıÂÓÔ‡˜.

showed significant improvement of CS, HR and RR in comparison to the other two groups. There was no significant difference in the measured parameters between the dexamethasone and beclome- thasone groups. In summary, the use of aerolized L-epinephrine is superior to intramuscular dexamethasone and aerolized beclomethazone in the management of ALV in the ER setting. However, the use of aerolized beclomethasone in the outpatient management of viral croup is associated with a significant reduction in severity of illness within 24 hrs after treatment.This mode of administration of local steroids provides a safe and cost effective method of treatment of AVL in ER or at home.

§¤ÍÂȘ-ÎÏÂȉȿ: Ï·Ú˘ÁÁÔÙÚ·¯ÂÈÔ‚ÚÔÁ¯›Ùȉ·, ÙÔÈο ·ÓÙÈÊÏÂÁÌÔÓÒ‰Ë, ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹, ÌÂÎÏÔÌÂı·˙fiÓË, ‚·ıÌfi˜ ‰‡ÛÓÔÈ·˜.

Key words: croup (laryngotracheobronchitis), local antiinflammatory drugs, beclomethazone, croup score.

™˘ÓÙÔÌÔÁڷʛ˜ ¢ª: ¢¶ª: §∆µ: LE: B¢: ∂π: SO2: ∞∞: K™: ∞¶:

¢ÂÍ·ÌÂı·˙fiÓË ¢ÈÚÔÈÔÓÈ΋ ªÂÎÏÔÌÂı·˙fiÓË §·Ú˘ÁÁÔÙÚ·¯ÂÈÔ‚ÚÔÁ¯›ÙȘ L-ÂÈÓÂÊÚ›ÓË, ·‰ÚÂÓ·Ï›ÓË µ·ıÌfi˜ ¢‡ÛÓÔÈ·˜ ∂͈ÙÂÚÈο π·ÙÚ›· ∫ÔÚÂÛÌfi˜ Ô͢·ÈÌÔÛÊ·ÈÚ›Ó˘ ∞ÚÈıÌfi˜ ·Ó·ÓÔÒÓ ∫·Ú‰È·Î¤˜ ™Ê‡ÍÂȘ ∞ÚÙËÚȷ΋ ¶›ÂÛË

∂ÈÛ·ÁˆÁ‹ ∏ ÔÍ›· Ï·Ú˘ÁÁ›Ùȉ· (Ï·Ú˘ÁÁÔÙÚ·¯ÂÈÔ‚ÚÔÁ¯›Ùȉ·) ‹ ÈÔÁÂÓ¤˜ croup ‹ croup Â›Ó·È ¤Ó· Û‡Ó‰ÚÔÌÔ ·fiÊڷ͢ ÙˆÓ ·ÓÒÙÂÚˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Ô‰ÒÓ. ÷ڷÎÙËÚ›˙ÂÙ·È ·fi ˘Ï·ÎÒ‰Ë ‚‹¯·, ‚Ú¿Á¯Ô˜ ʈӋ˜ Î·È ÂÈÛÓ¢ÛÙÈÎfi Û˘ÚÈÁÌfi. ¶·ıÔÏÔÁÔ·Ó·ÙÔÌÈο, ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ˘ÔÁψÙÙȉÈ΋ ÊÏÂÁÌÔÓ‹, Û˘Ó‹ıˆ˜ ÈÔÁÂÓÔ‡˜ ·ÈÙÈÔÏÔÁ›·˜ (1). ¡ÔÛÔÎÔÌÂÈ·ÎÔ› ·ÛıÂÓ›˜ Ì croup ¤¯Ô˘Ó ·ÓÙÈÌÂÙˆÈÛı› Ì ÓÂÊÂÏÔÔÈË̤ÓË ·‰ÚÂÓ·Ï›ÓË(LE) ‹/Î·È Û˘ÛÙËÌ·ÙÈ΋ ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ (2). ∏ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ÓÂÊÂÏÔÔÈË̤Ó˘ LE ÛÙË ıÂÚ·›· Ù˘ ÔÍ›·˜ §∆µ Â›Ó·È ·ԉ‰ÂÈÁ̤ÓË Î·È Ë ¯Ú‹ÛË Ù˘ ¢Ú›· Û ·ÁÎfiÛÌÈÔ Â›Â‰Ô (3-7). ªÂÙ¿ ·fi ·ÌÊÈÛ‚‹ÙËÛË 40 ¯ÚfiÓˆÓ (8,9) ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ÙÂÎÌËÚÈÒÛÂÈ ÙËÓ

60

·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙË ıÂÚ·›· ÓÔÛÔÎÔÌÂÈ·ÎÒÓ ·ÛıÂÓÒÓ Ì croup (10,16). ∂›Ó·È ϤÔÓ ·ԉ‰ÂÈÁ̤ÓÔ fiÙÈ ·È‰È¿ Ì ÂÏ·ÊÚÈ¿˜, ̤ÙÚÈ·˜ Î·È ‚·ÚÈ¿˜ ÌÔÚÊ‹˜ croup ÂˆÊÂÏÔ‡ÓÙ·È ·fi ÙËÓ ¤ÁηÈÚË ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ (17). ∏ ÓÂÊÂÏÔÔÈË̤ÓË ‚Ô˘‰ÂÛÔ‰›ÓË ˘ÂÚÙÂÚ› ıÂÚ·¢ÙÈο Û ·È‰È¿ Ì ÂÏ·ÊÚ‡-̤ÙÚÈÔ (14) Î·È Ì¤ÙÚÈÔ-‚·Ú‡ croup (10,15) Û ۯ¤ÛË Ì ÙÔ ¯ÔÚËÁÔ‡ÌÂÓÔ ÓÂÊÂÏÔÔÈË̤ÓÔ Ê˘ÛÈÔÏÔÁÈÎfi ÔÚfi (placebo), ·ÏÏ¿ Â›Ó·È ÙÔ ›‰ÈÔ ·ÔÙÂÏÂÛÌ·ÙÈ΋ Ì ÙË ¯ÔÚËÁÔ‡ÌÂÓË ÓÂÊÂÏÔÔÈË̤Ó˘ LE ÛÙÔ Ì¤ÙÚÈÔ-‚·Ú‡ croup (2). ∞’ fi,ÙÈ ÁÓˆÚ›˙Ô˘ÌÂ, ˘¿Ú¯ÂÈ ÂÚÈÔÚÈṲ̂ÓË ·Ó·ÊÔÚ¿ ÛÙËÓ ·ÁÁÏÔÛ·ÍÔÓÈ΋ È·ÙÚÈ΋ ‚È‚ÏÈÔÁÚ·Ê›· fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Î·È ·ÛÊ¿ÏÂÈ· ÙˆÓ ·ÂÚÔÔÈËÌ¤ÓˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ Ù· ÔÔ›· ¯ÔÚËÁÔ‡ÓÙ·È ÙÔÈο ÌÂ Û˘Û΢¤˜ ·ÂÚÔı·Ï¿ÌˆÓ, Û ۯ¤ÛË Ì ÙËÓ ˘¿Ú¯Ô˘Û· ‚È‚ÏÈÔÁÚ·Ê›· Û¯ÂÙÈο Ì ÙËÓ ∂ª ¯ÔÚ‹ÁËÛË Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘ (¢ª) Î·È Ù˘ ÓÂÊÂÏÔÔÈË̤Ó˘ LE ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ Â͈ÓÔÛÔÎÔÌÂÈ·ÎÒÓ ·ÛıÂÓÒÓ Ì ÔÍ›· §∆µ(9). ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ÂÎÙ›ÌËÛË Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ Î·È ·ÛÊ¿ÏÂÈ·˜ Ù˘ ·ÂÚÔÔÈË̤Ó˘ ¢¶ª ÛÙË ıÂÚ·›· Ù˘ ÔÍ›·˜ §∆µ Û Â͈ÓÔÛÔÎÔÌÂÈ·ÎÔ‡˜ ·ÛıÂÓ›˜. ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ ∆Ô ˘ÏÈÎfi Ù˘ ÌÂϤÙ˘ Ì·˜ ·ÔÙ¤ÏÂÛ·Ó 62 ·ÛıÂÓ›˜ ËÏÈΛ·˜ 6 ÌËÓÒÓ Ì¤¯ÚÈ 5 ÂÙÒÓ Ô˘ ÚÔÛ‹Ïı·Ó ÛÙ· ∂.π. ÙÔ˘


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 61

¶·È‰È·ÙÚÈ΋ 2001;64:59-64

Paediatriki 2001;64:59-64

ÓÔÛÔÎÔÌ›Ԣ Ì·˜ ÙËÓ ÂÚ›Ô‰Ô √ÎÙˆ‚Ú›Ô˘ 1996-¡ÔÂÌ‚Ú›Ô˘1998 Ì ÙË ‰È¿ÁÓˆÛË Ù˘ ÔÍ›·˜ §∆µ. ∆· ÎÚÈÙ‹ÚÈ· ÂÈÏÔÁ‹˜ ÙˆÓ ·ÛıÂÓÒÓ ‹Ù·Ó: 1) ¶ÚÔËÁËı›۷ (12-72 ÒÚ˜) ÈÔÁÂÓ‹˜ Ïԛ̈ÍË ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Ì ÎfiÚ˘˙·, ‚Ú¿Á¯Ô˜ ʈӋ˜ Î·È ˘Ï·ÎÒ‰Ë ‚‹¯· Î·È 2) ∫ÏÈÓÈ΋ ‰È·›ÛÙˆÛË ‚Ú¿Á¯Ô˘˜ ʈӋ˜, ˘Ï·ÎÒ‰Ô˘˜ ‚‹¯· Î·È ÂÈÛÓ¢ÛÙÈÎÔ‡ Û˘ÚÈÁÌÔ‡ ηٿ ÙËÓ ÚÔÛ¤ÏÂ˘Û‹ ÙÔ˘˜ ÛÙ· ∂.π. ∏ ‚·Ú‡ÙËÙ· Ù˘ Ïԛ̈͢ ÂÎÙÈÌ‹ıËÎÂ, 1) Ì ÙÔ ‚·ıÌfi ‰‡ÛÓÔÈ·˜ (µ.¢), croup score ÙÔ˘Ï¿¯ÈÛÙÔÓ >2, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙÔ ÙÚÔÔÔÈË̤ÓÔ Û¯‹Ì· ηٿ Downes Î·È Raphaely (18) (¶›Ó·Î·˜ 1) Î·È 2) Ì ÙÔ ·Ó Ë Î·Ù¿ÛÙ·ÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ ‰ÂÓ ··ÈÙÔ‡Û ÂÈÛ·ÁˆÁ‹ ÛÙÔ ¡ÔÛÔÎÔÌ›Ô. ∞ÔÎÏ›ÛıËÎ·Ó ·fi ÙË ÌÂϤÙË ·È‰È¿ Ô˘ ›¯·Ó ·Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Ô‰ÒÓ, ÔÍ›· ÂÈÁψÙÙ›Ùȉ·, ‚·ÎÙËÚȷ΋ ÙÚ·¯ÂÈ›Ùȉ·, Ó¢ÌÔÓ›·, ÂÈÛÚfiÊËÛË Í¤ÓÔ˘ ÛÒÌ·ÙÔ˜, ÈÛÙÔÚÈÎfi Ï·Ú˘ÁÁÔÛÎfiËÛ˘ Î·È ÂÓ‰ÔÙÚ·¯Âȷ΋˜ ‰È·ÛˆÏ‹ÓˆÛ˘, ¯ÚfiÓÈ· ·fiÊÚ·ÍË ÙˆÓ ·ÓˆÙ¤ÚˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ, ÂΛӷ Ô˘ ¤Ï·‚·Ó ÂÚÈÛÛfiÙÂÚ˜ Ù˘ Ì›·˜ ÊÔÚ¿˜ ıÂÚ·›· Ì ÓÂÊÂÏÔÔÈË̤ÓË LE, ÂΛӷ Ô˘ ›¯·Ó Û˘¯Ó¤˜ ÓÔÛËÏ›˜ ÛÙ· ÓÔÛÔÎÔÌ›· ηٿ ÙÔ ÚfiÛÊ·ÙÔ ·ÚÂÏıfiÓ, ÂΛӷ Ô˘ ÂÏ¿Ì‚·Ó·Ó ıÂÚ·›· Ì ‚-‰ÈÂÁ¤ÚÙ˜ Î·È ÂΛӷ Ô˘ ‹Û·Ó ˘fi ıÂÚ·›· Ì ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ÙȘ ÚÔËÁÔ‡ÌÂÓ˜ 24 ÒÚ˜. ∏ ÎÏÈÓÈ΋ Ì·˜ ‹Ù·Ó Ë ÌfiÓË ÂÊËÌÂÚÂ‡Ô˘Û· ·È‰È·ÙÚÈ΋ ÎÏÈÓÈ΋ Â› 24ÒÚÔ˘ ‚¿Ûˆ˜ Î·È Î¿Ï˘Ù ٷ ·È‰È·ÙÚÈο ÂÚÈÛÙ·ÙÈο ÌÈ·˜ ÂÚÈÔ¯‹˜ ÂÚ›Ô˘ 2,5 ÂηÙÔÌÌ˘Ú›ˆÓ ηÙԛΈÓ. ∏ ‰ÈÂÍ·ÁˆÁ‹ Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜ ÂÁÎÚ›ıËΠ·fi ÙËÓ ∂ÈÙÚÔ‹ ¶·È‰È·ÙÚÈ΋˜ ŒÚ¢ӷ˜ ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ Ì·˜. √ ‚·ıÌfi˜ ‰‡ÛÓÔÈ·˜ (µ¢) ÙÔ˘ croup (croupscore) ˘ÔÏÔÁ›ÛÙËΠ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙÔ ÙÚÔÔÔÈË̤ÓÔ Û¯‹Ì· ηٿ Downes Î·È Raphaly (8) (¶›Ó·Î·˜ 1), ÙÔ ÔÔ›Ô ÂÎÙÈÌ¿ 5 ÎÏÈÓÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜, ÔÈ Ôԛ˜ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙÔ ‚·ıÌfi ·fiÊڷ͢ ÙˆÓ ·ÓˆÙ¤ÚˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Ô‰ÒÓ. °È· ηχÙÂÚË ·ÓÙÈÎÂÈÌÂÓÈÎfiÙËÙ·, Ë ·Ú¿ÌÂÙÚÔ˜ Ù˘ ΢¿ÓˆÛ˘ ÙÚÔÔÔÈ‹ıËÎÂ Î·È ÔÚ›ÛÙËÎÂ Û·Ó SO2 <95%. ∏ ‰È·‚¿ıÌÈÛË ÙÔ˘ µ¢ Î˘Ì·›ÓÂÙ·È ÌÂٷ͇ 0 Î·È 10, fiÔ˘ 10 ˘Ô‰ÂÈÎÓ‡ÂÈ ÙË ‚·Ú‡ÙÂÚË Î·Ù¿ÛÙ·ÛË. ÃÚËÛÈÌÔÔÈ‹Û·Ì ÙÔ µ¢≥6 ÁÈ· ·ÛıÂÓ›˜ Ì ̤ÙÚÈÔ-‚·Ú‡ croup. OÈ ·ÛıÂÓ›˜ ¯ˆÚ›ÛıËÎ·Ó Ù˘¯·›· Û ÙÚÂȘ ÔÌ¿‰Â˜: √Ì¿‰· ∞: ¡ÂÊÂÏÔÔÈË̤ÓË LE 1/1000 (1amp:1ml/1mg) Û ‰fiÛË 5 ml. √Ì¿‰· µ: ¢ÂÍ·ÌÂı·˙fiÓË (¢ª) 0,6 mg/kg B™, ∂ª(max 8 mg)

√Ì¿‰· °: ∞ÂÚÔÔÈË̤ÓË ¢¶ª, 200 mcg Ì ·ÂÚÔı¿Ï·ÌÔ. ∏ ÛΤ„Ë ÁÈ· ¯ÔÚ‹ÁËÛË ·ÂÚÔÔÈË̤Ó˘ ¢¶ª ‚·Û›ÛÙËΠÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ Â›Ó·È ÔÈÎÔÓÔÌÈο ÊıËÓfiÙÂÚË ·fi ¿ÏÏ· Û΢¿ÛÌ·Ù·, Â›Ó·È ‰È·ı¤ÛÈÌË ·ÁÎfiÛÌÈ·, fiˆ˜ Î·È ÛÙË ¯ÒÚ· Ì·˜ Î·È ÌÔÚ› Ó· ¯ÔÚËÁËı› Î·È ÛÙÔ Û›ÙÈ ÙÔ˘ ·ÛıÂÓÔ‡˜ Ì ÂȉÈΤ˜ Û˘Û΢¤˜ (.¯ ·ÂÚÔı¿Ï·ÌÔÈ) (19,25). ∏ ÚÔ‹ Ô͢ÁfiÓÔ˘ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËΠÁÈ· ÙË ÓÂÊÂÏÔÔ›ËÛË Ù˘ LE ‹Ù·Ó 4-5 L/min Î·È Ë ‰È¿ÚÎÂÈ· 5-10min. ∏ ¢¶ª ¯ÔÚËÁ‹ıËΠÌÂ Û˘Û΢‹ ·ÂÚÔı·Ï¿ÌÔ˘, Û‡Ìʈӷ Ì ÙȘ ˘ԉ›ÍÂȘ ÙÔ˘ ıÂÚ¿ÔÓÙÔ˜ È·ÙÚÔ‡. ∂ÎÙÈÌ‹ıËÎ·Ó ÔÈ ·Ú¿ÌÂÙÚÔÈ: ‚·ıÌfi˜ ‰‡ÛÓÔÈ·˜ (µ¢),ηډȷΤ˜ ÛʇÍÂȘ (∫™), ·ÚÈıÌfi˜ ·Ó·ÓÔÒÓ (∞∞), ·ÚÙËÚȷ΋ ›ÂÛË (∞¶), Î·È Ô ÎÔÚÂÛÌfi˜ Ô͢·ÈÌÔÛÊ·ÈÚ›Ó˘ (SO2). H ÂͤٷÛË ÙˆÓ ·Ú·Ì¤ÙÚˆÓ ·˘ÙÒÓ ¤ÁÈÓ ÛÙÔ˘˜ ¯ÚfiÓÔ˘˜ 0, ‰ËÏ. ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜, Î·È ÛÙË Û˘Ó¤¯ÂÈ· ÛÙ· 15, 30, 60, 90 Î·È 120 ÏÂÙ¿ ÌÂÙ¿ ÙË ¯ÔÚ‹ÁËÛË. ¶·Ú¿ÏÏËÏ·, ÁÈÓfiÓÙ·Ó Û˘Ó¯Ҙ ηٷÁÚ·Ê‹ Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ Î·È ÙˆÓ ·ÚÂÓÂÚÁÂÈÒÓ ÙˆÓ Ê·ÚÌ¿ÎˆÓ ÛÙÔ˘˜ ÚÔ·Ó·ÊÂÚı¤ÓÙ˜ ¯ÚfiÓÔ˘˜. ∞Ó Ô µ¢ ‰ÂÓ ‚ÂÏÙȈÓfiÙ·Ó Î·Ù¿ ÙÔ˘Ï¿¯ÈÛÙÔÓ 3 ‚·ıÌÔ‡˜, Ô ·ÛıÂÓ‹˜ ıˆÚ›ÙÔ ıÂÚ·¢ÙÈ΋ ·ÔÙ˘¯›· Î·È ÂÈÛ·ÁfiÙ·Ó ÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ÁÈ· ¯ÔÚ‹ÁËÛË Ù˘ Û˘ÓËıÈṲ̂Ó˘ ıÂÚ·¢ÙÈ΋˜ ·ÁˆÁ‹˜. ∞ÎÙÈÓÔÁڷʛ˜ ·˘¯ÂÓÈ΋˜ ÌÔ›Ú·˜ Ù˘ ÛÔÓ‰˘ÏÈ΋˜ ÛÙ‹Ï˘, ıÒڷη Î·È ÁÂÓÈ΋ ÂͤٷÛË ·›Ì·ÙÔ˜ ‰ÈÂÓÂÚÁÔ‡ÓÙ·Ó ÌfiÓÔ fiÙ·Ó ˘‹Ú¯·Ó ÎÏÈÓÈΤ˜ ÂӉ›ÍÂȘ ·ÔÎÏÂÈÛÌÔ‡ ¿ÏÏˆÓ Î·Ù·ÛÙ¿ÛÂˆÓ ˘‡ı˘ÓˆÓ Ù˘ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÂÈ·˜. ∆ÔÓ Î¿ı ·ÛıÂÓ‹ ÂÎÙÈÌÔ‡ÛÂ Ô ›‰ÈÔ˜ È·ÙÚfi˜ ηı’ fiÏË ÙË ‰È¿ÚÎÂÈ· ÙˆÓ 2 ˆÚÒÓ Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘. ™˘ÌÏËڈ̷ÙÈ΋ ¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ ¯ÔÚËÁ‹ıËΠÛÙ· ·È‰È¿ Ì SO2>95%. ªÂÙ¿ ÙÔ ¤Ú·˜ ÙˆÓ 2 ˆÚÒÓ Ù· ·È‰È¿ ·Ô¯ˆÚÔ‡Û·Ó ÁÈ· ÙÔ Û›ÙÈ ÙÔ˘˜, ÂÎÙfi˜ ÂÎÂ›ÓˆÓ Ô˘ ÎÚ›ÓÔÓÙ·Ó Û·Ó ıÂÚ·¢ÙÈΤ˜ ·ÔÙ˘¯›Â˜, ÔfiÙ ÂÈÛ¿ÁÔÓÙ·Ó ÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ÁÈ· ÂÚ·ÈÙ¤Úˆ ıÂÚ·›·. ∑ËÙ‹ıËΠ·fi ÙÔ˘˜ ·Ô¯ˆÚ‹Û·ÓÙ˜ ·ÛıÂÓ›˜ Ó· Â·Ó¤ÏıÔ˘Ó ÛÙ· ∂π Û ÂÚ›ÙˆÛË ˘ÔÙÚÔ‹˜ Ù˘ ÓfiÛÔ˘ ̤۷ ÛÙȘ ÂfiÌÂÓ˜ 24 ÒÚ˜. ∆· ∂π ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ Ì·˜ ‹Ù·Ó Ù· ÌfiÓ· Ô˘ ÂÊË̤Ú¢·Ó Â› 24ˆÚÔ˘ ‚¿Ûˆ˜ Î·È Î·Ù¿ Û˘Ó¤ÂÈ· fiϘ ÔÈ ıÂÚ·¢ÙÈΤ˜ ·ÔÙ˘¯›Â˜ ÂÚfiÎÂÈÙÔ Ó· Â·ÓÂÎÙÈÌËıÔ‡Ó ·fi ÂÌ¿˜ ÙÔ˘˜ ›‰ÈÔ˘˜. ∏ ·ÓÙ·fiÎÚÈÛË ÙˆÓ ·ÛıÂÓÒÓ Î·È ÙˆÓ 3 ÔÌ¿‰ˆÓ ÛÙËÓ Ê·Ú̷΢ÙÈ΋ ·ÁˆÁ‹ Û˘ÁÎÚ›ıËΠ¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙȘ ÌÂÙÚ‹ÛÂȘ ÙÔ˘ µ¢. ∏ Û‡ÁÎÚÈÛË ¤ÁÈÓ Ì ÙËÓ Ì¤ıÔ‰Ô ∞¡√VA. ∂·Ó·Ï·Ì‚·ÓfiÌÂÓ˜ ÌÂÙÚ‹ÛÂȘ ¯ÚËÛÈÌÔÔÈÒ-

¶›Ó·Î·˜ 1. µ·ıÌÔÏÔÁ›· ÛÔ‚·ÚfiÙËÙ·˜ ÙÔ˘ ‚·ıÌÔ‡ ‰‡ÛÓÔÈ·˜ (µ¢) ηٿ Downes & Raphaely (ÙÚÔÔÔÈË̤ÓÔ). µ·ıÌÔÏÔÁ›· ÛÔ‚·ÚfiÙËÙ·˜ ‰‡ÛÓÔÈ·˜* ¶·Ú¿ÌÂÙÚÔ˜ ∂ÈÛÓ¢ÛÙÈο Â˘Ú‹Ì·Ù· ™˘ÚÈÁÌfi˜(Stridor) µ‹¯·˜ ∂ÈÛÔÏΤ˜/∞Ó·¤Ù·ÛË ÚÈÓ. ÙÂÚ.

0 Ÿ¯È Ÿ¯È Ÿ¯È Ÿ¯È

1 ∆Ú·¯‡ÙËÙ·-ÚfiÁ¯ÔÈ ∂ÈÛÓ¢ÛÙÈÎfi˜ ∆Ú·¯‡ ÎÏ¿Ì· ÀÂÚÛÙÂÚÓÈΤ˜/¡∞π

∫˘¿ÓˆÛË**

Ÿ¯È

™Â ·¤Ú·

2 ¶·Ú¿Ù·ÛË ∞ÌÊ›¯ÚÔÓÔ˜ ÀÏ·Î҉˘ ÀÂÚÛÙÂÚÓÈΤ˜ & ÌÂÛÔχÚȘ/¡∞π ™Â F1O2: 0,4***

* ¶ËÁ‹:Downes & Raphaely (18) ** √Ú›˙ÂÙ·È ˆ˜ √2sat<95% *** ∫Ï¿ÛÌ· ÂÈÛÓÂfiÌÂÓÔ˘ √2

61


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 62

¶·È‰È·ÙÚÈ΋ 2001;64:59-64

Paediatriki 2001;64:59-64

L-¢ÂÍ·ÌÂı·˙fiÓË ∂ÈÓÂÊÚ›ÓË ªÂÎÏÔÌÂı·˙fiÓË ¢ÂÍ·ÌÂı·˙fiÓË

µ·ıÌfi˜ ¢‡ÛÓÔÈ·˜ (µ¢)

ªÂÎÏÔÌÂı·˙fiÓË L- ∂ÈÓÂÊÚ›ÓË

6 5 4 3 2 1 0 15

30

60

90

120

Ã√¡√™(min) ∂ÈÎfiÓ· 1. °Ú·ÊÈ΋ ·ÂÈÎfiÓÈÛË ÙÔ˘ ‚·ıÌÔ‡ ‰‡ÛÓÔÈ·˜ (µ¢) ÙˆÓ ÙÚÈÒÓ ÔÌ¿‰ˆÓ Û ۯ¤ÛË Ì ÙÔÓ ¯ÚfiÓÔ, ∏ ¯ÔÚ‹ÁËÛË ·ÂÚÔÔÈË̤Ó˘ ÌÂÎÏÔÌÂı·˙fiÓ˘ ‚ÂÏÙ›ˆÛ ÛËÌ·ÓÙÈο ÙÔ µ¢. ÓÙ·˜ ÙËÓ ·Ú·¿Óˆ ̤ıÔ‰Ô ¤ÁÈÓ·Ó ÚÔÎÂÈ̤ÓÔ˘ Ó· Û˘ÁÎÚÈıÔ‡Ó Î·È ÔÈ ˘fiÏÔÈ˜ ·Ú¿ÌÂÙÚÔÈ (∞∞, ∫™, ∞¶, SO2) ÌÂٷ͇ ÙˆÓ 3 ÔÌ¿‰ˆÓ. ∏ ÛÙ·ÙÈÛÙÈ΋ ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ¤ÁÈÓ Ì ƒ<0,05.

∞ÔÙÂϤÛÌ·Ù· ∆Ô ˘ÏÈÎfi Ù˘ ÌÂϤÙ˘ Ì·˜ ·ÔÙ¤ÏÂÛ·Ó 62 ·ÛıÂÓ›˜. √È ıÂÚ·¢ÙÈΤ˜ ÔÌ¿‰Â˜ ‹Û·Ó ÔÌÔÈÔÁÂÓ›˜ ˆ˜ ÚÔ˜ ÙËÓ ËÏÈΛ·, ÙÔ Ê‡ÏÔ Î·È ÙË Û˘Ìو̷ÙÔÏÔÁ›·. ∏ ÌfiÓË ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ Ô˘ ‰È·ÈÛÙÒıËΠÌÂٷ͇ ÙˆÓ 3 ÔÌ¿‰ˆÓ ‹Ù·Ó ·˘Ù‹ ÙÔ˘ ‚·ıÌÔ‡ ‰‡ÛÓÔÈ·˜ (µ¢) ÚÔ Ù˘ ıÂÚ·›·˜ ÛÙËÓ ÔÌ¿‰· µ (‰ÂÍ·ÌÂı·˙fiÓ˘), Ô ÔÔ›Ô˜ ‹Ù·Ó ÂÏ·ÊÚÒ˜ ˘„ËÏfiÙÂÚÔ˜ Ù˘ ÔÌ¿‰·˜ ∞ (·‰ÚÂÓ·Ï›Ó˘).(Ã: 5,89 vs 5,13, P<0,05) (¶›Ó·Î·˜ 2). ¢È·ÈÛÙÒıËΠÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË ÙÔ˘ µ¢ Î·È ÛÙȘ 3 ÔÌ¿‰Â˜ ÌÂÙ¿ ÙÔ ¤Ú·˜ ÙÔ˘ ¯ÚfiÓÔ˘ ·Ú·ÎÔÏÔ‡ıËÛ˘. ∫·Ó¤Ó·˜ ·ÛıÂÓ‹˜ ‰ÂÓ Â¤ÛÙÚ„ ÛÙÔ ∂π ÙËÓ ›‰È· ̤ڷ (∂ÈÎfiÓ· 1). ∏ ÔÌ¿‰· Ù˘ LE ·ÚÔ˘Û›·Û ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË ÛÙȘ ·Ú·Ì¤ÙÚÔ˘˜ µ¢, ∫™ Î·È ∞∞ Û ۯ¤ÛË Ì ÙȘ ¿ÏϘ 2 ÔÌ¿‰Â˜. ¢ÂÓ ‰È·ÈÛÙÒıËÎÂ, ˆÛÙfiÛÔ, ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÛÙȘ ÌÂÙÚËı›Û˜ ·Ú·Ì¤¶›Ó·Î·˜ 2. ¢ËÌÔÁÚ·ÊÈÎfi ÚÔÊ›Ï* ·ÛıÂÓÒÓ. ÷ڷÎÙËÚÈÛÙÈο ª¤ÛË ËÏÈΛ·(¯Ú) º‡ÏÏÔ(∞/∫) ª¤ÛË ÙÈÌ‹ µ¢ ÂÈÛfi‰Ô˘

√Ì¿‰· ∞ √Ì¿‰· µ √Ì¿‰· ° 2,6 3,2 3,4 20:3 11:8 13:7 5,13 5,89** 3,95

* M¤Û˜ ÙÈ̤˜(SD) **P<0,05 ÌfiÓÔ ÌÂٷ͇ ÔÌ¿‰ˆÓ ∞ & µ

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ÙÚÔ˘˜ ÌÂٷ͇ ÙˆÓ ÔÌ¿‰ˆÓ Ù˘ ¢ª Î·È Ù˘ ¢¶ª. ™˘˙‹ÙËÛË ∏ ÔÍ›· Ï·Ú˘ÁÁ›Ùȉ· (Ï·Ú˘ÁÁÔÙÚ·¯ÂÈÔ‚ÚÔÁ¯›Ùȉ·) ‹ ÈÔÁÂÓ¤˜ croup ‹ croup Â›Ó·È Ë ÈÔ Û˘¯Ó‹ ·ÈÙ›· ÔÍ›·˜ ·fiÊڷ͢ ÙˆÓ ·ÓˆÙ¤ÚˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Ô‰ÒÓ Û ·È‰È¿ ËÏÈΛ·˜ 6 ÌËÓÒÓ ¤ˆ˜ 6 ÂÙÒÓ(1). ∏ ¯Ú‹ÛË ÓÂÊÂÏÔÔÈË̤Ó˘ LE ¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ ÂÏ·ÙÙÒÓÂÈ ÛËÌ·ÓÙÈο ÙË ‚·Ú‡ÙËÙ· Ù˘ ÓfiÛÔ˘, ˆÛÙfiÛÔ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ¿ Ù˘ ÂÚÈÔÚ›˙ÂÙ·È ÛÙȘ ÂfiÌÂÓ˜ 2 ÒÚ˜ ·fi ÙË ¯ÔÚ‹ÁËÛ‹ Ù˘. ∏ ¯ÔÚ‹ÁËÛË ÌÈ·˜ ÂÊ¿·Í ∂.ª ‰fiÛ˘ ¢ª Û Â͈ÓÔÛÔÎÔÌÂÈ·ÎÔ‡˜ ·ÛıÂÓ›˜ Ì ÔÍ›· §∆µ ÂÏ·ÙÙÒÓÂÈ ÙË ‚·Ú‡ÙËÙ· Î·È ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓfiÛÔ˘(15). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ‰È·ÈÛÙÒıËΠÛËÌ·ÓÙÈ΋ ÂÏ¿ÙÙˆÛË Ù˘ ÎÏÈÓÈ΋˜ ‚·Ú‡ÙËÙ·˜ ÙÔ˘ croup Û fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Î·È ÙˆÓ ÙÚÈÒÓ ÔÌ¿‰ˆÓ. ∞Ó Î·È ÔÈ ‰È·ÊÔÚ¤˜ ÛÙȘ ÙÈ̤˜ ı· ÌÔÚÔ‡Û·Ó Ó· ÔÊ›ÏÔÓÙ·È Û ·Ú¿ÁÔÓÙ˜ ‰È·ÊÔÚÂÙÈÎÔ‡˜ ÙˆÓ Ê·Ú̿ΈÓ, ·˘ÙÔ› ÔÈ ·Ú¿ÁÔÓÙ˜ Ú¤ÂÈ Ó· ¤¯Ô˘Ó ηٷÓÂÌËı› ÔÌÔÈfiÌÔÚÊ· ÌÂٷ͇ ÙˆÓ ÔÌ¿‰ˆÓ. ∏ ÛΤ„Ë ÁÈ· ¯ÔÚ‹ÁËÛË ·ÂÚÔÔÈË̤Ó˘ ¢¶ª ‚·Û›ÛÙËΠÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ë ÌÔÚÊ‹ ·˘Ù‹ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ηÙ¢ı‡ÓÂÙ·È ·Ì¤Ûˆ˜ ÛÙÔ fiÚÁ·ÓÔ ÛÙfi¯Ô˜, ·Ú·Î¿ÌÙÔÓÙ·˜ ÙËÓ ·ÈÌ·ÙÈ΋ ΢ÎÏÔÊÔÚ›·, Â›Ó·È ·Ï‹ ÛÙË ¯ÔÚ‹ÁËÛ‹ Ù˘, ¤¯ÂÈ ¯·ÌËÏfi ÔÈÎÔÓÔÌÈÎfi ÎfiÛÙÔ˜ Î·È Â›Ó·È ‰È·ı¤ÛÈÌË ·ÁÎfiÛÌÈ·. ∏ ıÂÚ·›· Ì ÂÓ‰ÔÌ˘˚΋ ¢ª, ›¯Â Û·Ó ·ÔÙ¤ÏÂÛÌ· ÙËÓ ›‰È· ‚ÂÏÙ›ˆÛË ÙÔ˘ µ¢ Û ۇÁÎÚÈÛË Ì ÙË ıÂÚ·›· Ì ·ÂÚÔÔÈË̤ÓË ¢¶ª. ∞Ó Î·È Ë ÎÏÈÓÈ΋ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙˆÓ ÚÔ·Ó·ÊÂÚı¤ÓÙˆÓ Û¯ËÌ¿ÙˆÓ ÌÔÚ› Ó· ηı˘ÛÙÂÚ‹ÛÂÈ (∂ÈÎfiÓ· 1), Ë ¢¶ª ¤¯ÂÈ Ù¤ÙÔÈÔ ¯ÚfiÓÔ ËÌ›ÛÂÈ·˜ ˙ˆ‹˜, ÒÛÙ ÌÈ· ·Ï‹ ‰fiÛË ÙˆÓ 200 mcg ‹ Î·È ÌÂÁ·Ï‡ÙÂÚË Ó· Â›Ó·È ·ÚÎÂÙ‹ ÚÔÎÂÈ̤ÓÔ˘ Ô ·ÛıÂÓ‹˜ Ó· ÍÂÂÚ¿ÛÂÈ ÙËÓ ÔÍ›· Ê¿ÛË Ù˘ ÓfiÛÔ˘ Î·È Ó· ·ÔʇÁÂÈ ÙËÓ ÂÓ‰ÔÓÔÛÔÎÔÌÂȷ΋ ÓÔÛËÏ›·. ∏ ¢ª ÂÓ‹ÚÁËÛ ٷ¯‡ÙÂÚ· ·’ fi,ÙÈ Ë ¢¶ª. ∞˘Ùfi ÙÔ Â‡ÚËÌ· ‹Ù·Ó ·ÚÔÛ‰fiÎËÙÔ, ηıfiÛÔÓ Ë Û˘ÁΤÓÙÚˆÛË Ù˘ ¢¶ª ÛÙÔ˘˜ ÈÛÙÔ‡˜ ÙÔ˘ ·ÓˆÙ¤ÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ·˘Í¿ÓÂÈ ÁÚËÁÔÚfiÙÂÚ· ÌÂÙ¿ ÙË ¯ÔÚ‹ÁËÛ‹ Ù˘ Ì ·ÂÚÔı¿Ï·ÌÔ ·’ fi,ÙÈ ·˘Í¿ÓÂÈ Ë Û˘ÁΤÓÙÚˆÛË Ù˘ ¢ª ÌÂÙ¿ ÙËÓ ∂ª ¯ÔÚ‹ÁËÛ‹ Ù˘ (21). øÛÙfiÛÔ, ÙÔ Â‡ÚËÌ· ·˘Ùfi Â›Ó·È ·›ı·ÓÔ Ó· ÔÊ›ÏÂÙ·È Û ·ÓÂ·Ú΋ ÌÂÙ·ÊÔÚ¿ Ù˘ ¢¶ª ÛÙÔ˘˜ ÈÛÙÔ‡˜ ÙˆÓ ·ÓˆÙ¤ÚˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ. √È ·ÛıÂÓ›˜ ‚Ú›ÛÎÔÓÙ·Ó ˘fi ·˘ÛÙËÚ‹ ÂÈÙ‹ÚËÛË ·fi ÙÔÓ ıÂÚ¿ÔÓÙ· È·ÙÚfi ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰È·ÛÊ·ÏÈÛı› Ë Û˘ÌÌfiÚʈۋ ÙÔ˘˜ fiÛÔÓ ·ÊÔÚ¿ ÙË ÛˆÛÙ‹ ¯Ú‹ÛË Ù˘ Û˘Û΢‹˜(22,23). ¶ÈÛÙ‡ԢÌ fiÙÈ Ë ¯ÔÚ‹ÁËÛË ˘„ËÏfiÙÂÚˆÓ ‰fiÛÂˆÓ ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ ı· ÌÔÚÔ‡Û ӷ ÂÈʤÚÂÈ Ù·¯‡ÙÂÚ· Î·È Î·Ï‡ÙÂÚ· ·ÔÙÂϤÛÌ·Ù·. ∂›Ó·È ·ԉ‰ÂÈÁ̤ÓÔ fiÙÈ Ë ÌÂÁ·Ï‡ÙÂÚË ÔÛfiÙËÙ· ÙÔ˘ ÂÈÛÓÂfiÌÂÓÔ˘ Ê·ÚÌ¿ÎÔ˘ ·Ú·Ì¤ÓÂÈ ÛÙ· ÙÔÈ-


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¯ÒÌ·Ù· ÙˆÓ ·ÂÚÔÊfiÚˆÓ ÙÔ˘ ·ÓˆÙ¤ÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Î·È ÌfiÓÔ 1-5% Ù˘ ¯ÔÚËÁÔ‡ÌÂÓ˘ ‰fiÛ˘ Êı¿ÓÂÈ ÛÙÔ Î·ÙÒÙÂÚÔ ‚ÚÔÁ¯ÈÎfi ‰¤Ó‰ÚÔ. µÂ‚·›ˆ˜, ·˘Ùfi ·ÔÙÂÏ› ¤Ó· ÛËÌ·ÓÙÈÎfi ÌÂÈÔÓ¤ÎÙËÌ· ÛÙË ıÂÚ·›· ÙˆÓ ·ÛıÂÓÒÓ Ì ¿ÛıÌ·, ·ÏÏ¿ ȉ·ÓÈÎfi ÏÂÔÓ¤ÎÙËÌ· ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·ÛıÂÓÒÓ Ì ÔÍ›· §∆µ. ¶Èı·ÓÔÏÔÁԇ̠fiÙÈ Ì›· ÂÊ¿·Í ‰fiÛË ·ÂÚÔÔÈË̤Ó˘ ¢¶ª, ·ÓÂÍ¿ÚÙËÙ· Ù˘ ÔÛfiÙËÙ·˜, Â›Ó·È Ôχ ηϿ ·ÓÂÎÙ‹ ·fi ÙÔÓ ·ÛıÂÓ‹, ¯ˆÚ›˜ ·ÚÂÓ¤ÚÁÂȘ. ∞Ó Î·È ‰ÂÓ ÌÂÏÂÙ‹Û·Ì ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· ÙÔ˘ Û˘Ó‰˘·ÛÌÔ‡ Ù˘ LE Ì ÙËÓ ¢¶ª, ı· Û˘ÓÈÛÙÔ‡Û·ÌÂ Ë ¢¶ª Ó· ·ÎÔÏÔ˘ı› ÙË ¯ÔÚ‹ÁËÛË Ù˘ LE, ÚÔÎÂÈ̤ÓÔ˘ Ó· ÚÔÏ¿‚Ô˘Ì ÙËÓ ˘ÔÙÚÔ‹ Ù˘ ÓfiÛÔ˘, ÏfiÁˆ Ù˘ ÂÏ·Ùو̤Ó˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ Ù˘ LE Û ‚¿ıÔ˜ ¯ÚfiÓÔ˘. ∏ ȉ·ÓÈ΋ ‰ÔÛÔÏÔÁ›· Ù˘ ¢¶ª ‹ ¿ÏÏˆÓ ÂÈÛÓÂfiÌÂÓˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ·Ô̤ÓÂÈ Ó· ‰È¢ÎÚÈÓÈÛÙ› ÛÙÔ Ì¤ÏÏÔÓ. ™Â ·˘Ù‹ ÙË ÌÂϤÙË ‰ÂÓ ·Ú·ÙËÚ‹Û·Ì ηÌÈ¿ ‰È·ÊÔÚ¿ fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Î·È ÙËÓ ·ÛÊ¿ÏÂÈ· ÙˆÓ ¯ÔÚËÁÔ‡ÌÂÓˆÓ Ê·ÚÌ¿ÎˆÓ ÌÂٷ͇ ÙˆÓ ·È‰ÈÒÓ ÙˆÓ ÔÌ¿‰ˆÓ Ù˘ ¢¶ª Î·È Ù˘ ¢ª Ì ̤ÙÚÈÔ-ÛÔ‚·Úfi croup. ∏ ·ÂÚÔÔÈË̤ÓË ¢¶ª ›¯Â ÂÚÈÔÚÈṲ̂ÓË ‰Ú¿ÛË Î·Ù¿ Ù· ÚÒÙ· 30 min ÌÂÙ¿ ÙË ¯ÔÚ‹ÁËÛ‹ Ù˘, ·ÏÏ¿ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ·˘Í‹ıËΠ‚·ıÌÈ·›· ÙȘ ÂfiÌÂÓ˜ ÒÚ˜, ÌÈ· ÔÚ›· ·ÚfiÌÔÈ· Ù˘ ‰ÂÍ·ÌÂı·˙fiÓ˘. ∏ Ù·¯Â›· ¤Ó·ÚÍË ‰Ú¿Û˘ Ù˘ LE Â›Ó·È ÂÌÊ·Ó‹˜ ·fi ÙÔ ÁÂÁÔÓfi˜ Ù˘ ¿ÌÂÛ˘ Ì›ˆÛ˘ ÙÔ˘ µ¢ (croupscore) Û ۯ¤ÛË Ì ÚÈÓ ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂÚ·›·˜ Î·È ÙÔ˘ ˘„ËÏÔ‡ ÎÔÚÂÛÌÔ‡ Ù˘ Ô͢·ÈÌÔÛÊ·ÈÚ›Ó˘ (SO2) ÌÂÙ¿ 30 min. ∏ ÌÂÁ·Ï‡ÙÂÚË ÎÏÈÓÈ΋ ‚ÂÏÙ›ˆÛË, fiˆ˜ ·˘Ù‹ ÌÂÙÚ‹ıËΠ̠ÙËÓ ÙÒÛË ÙÔ˘ ̤ÛÔ˘ ÔÏÈÎÔ‡ µ¢, ÛËÌÂÈÒıËΠ2 ÒÚ˜ ÌÂÙ¿ Î·È ÁÈ· ÙȘ 3 ÔÌ¿‰Â˜ ÚÈÓ ·ÔÊ·ÛÈÛı› Ë ÂÚ·ÈÙ¤Úˆ Û˘ÛÙËÌ·ÙÈ΋ ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ. ¶Èı·ÓÔÏÔÁÂ›Ù·È ˆÛÙfiÛÔ, fiÙÈ ·˘ÙÔ‡ ÙÔ˘ ›‰Ô˘˜ Ë ıÂÚ·›· ÂÓ‰¤¯ÂÙ·È Ó· ÌËÓ ¤¯ÂÈ ÂÈʤÚÂÈ Î·ÌÈ¿ ·Ôχو˜ ÙÚÔÔÔ›ËÛË ÛÙËÓ ÔÚ›· Ù˘ ÓfiÛÔ˘. ∞˘Ù‹ Ë ˘fiıÂÛË ‰ÂÓ ÌÔÚ› Ó· ÙÂÎÌËÚȈı› ÏfiÁˆ ¤ÏÏÂȄ˘ ÛËÌ·ÓÙÈÎfiÙËÙ·˜ ÌÂٷ͇ ÙˆÓ ÙÚÈÒÓ ıÂÚ·¢ÙÈÎÒÓ Û¯ËÌ¿ÙˆÓ Û ÔÔÈÔ‰‹ÔÙ ÛÙ¿‰ÈÔ Ù˘ ·Ú·ÎÔÏÔ‡ıËÛ˘. ∏ ÚÔÛı‹ÎË ÌÈ·˜ ÔÌ¿‰·˜ placebo ı· ÌÔÚÔ‡Û ӷ ‰È·ÏÂ˘Î¿ÓÂÈ ÙËÓ ·¿ÓÙËÛË. øÛÙfiÛÔ, ‰ÂÓ Â›Ó·È ËıÈο ·Ô‰ÂÎÙfi Ó· ¯ÔÚËÁËı› ıÂÚ·›· placebo Û ·ÛıÂÓ›˜ Ì ÌÂÙÚ›Ô˘-ÛÔ‚·Ú‹˜ ‚·Ú‡ÙËÙ·˜ croup. H ÌÂϤÙË Ì·˜, ·Ó Î·È ÌÈÎÚ‹ ÛÙÔ Ì¤ÁÂıfi˜ Ù˘, Â›Ó·È Ë ÚÒÙË Ô˘ ı¤ÙÂÈ ÙÔ ÂÚÒÙËÌ·, ·Ó ÔÈ ·ÂÚÔÔÈË̤Ó˜ ÌÔÚʤ˜ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈËıÔ‡Ó ÛÙË ‚·ÛÈ΋ ıÂÚ·›· Â͈ÓÔÛÔÎÔÌÂÈ·ÎÒÓ ·ÛıÂÓÒÓ Ì ÌÂÙÚ›Ô˘-‚·Ú¤ˆ˜ ‚·ıÌÔ‡ §∆µ. ∞ԉ›¯ıËΠfiÙÈ ÌÈ· ÂÊ¿·Í ‰fiÛË ·ÂÚÔÔÈ-

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Ë̤Ó˘ ¢¶ª 200 mcg Â·ÚΛ Î·È ‰ÂÓ ˘¿Ú¯ÂÈ ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÛÙ· ·ÔÙÂϤÛÌ·Ù· ÌÂٷ͇ ÙˆÓ 2 ÔÌ¿‰ˆÓ ÙˆÓ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ (¢ª-¢¶ª) fiˆ˜ ·˘Ù‹ ÚÔ·ÙÂÈ ·fi ÙȘ ÌÂÙÚ‹ÛÂȘ ÙˆÓ ·Ú·Ì¤ÙÚˆÓ µ¢, ∞∞, ∫™ Î·È SO2 . ™ÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË Ù· Â˘Ú‹Ì·Ù· Ù˘ ÛÙ·ıÂÚ‹˜ ‚ÂÏÙ›ˆÛ˘ ÙˆÓ ·ÛıÂÓÒÓ Ì ÔÍ›· §∆µ ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ·ÂÚÔÔÈË̤Ó˘ ¢¶ª ¤¯ÂÈ Ù· ·ÎfiÏÔ˘ı· ÏÂÔÓÂÎÙ‹Ì·Ù·: ·) ∂›Ó·È ·Ï‹ ÛÙË ¯Ú‹ÛË Î·È ‰È·ı¤ÛÈÌË ·ÁÎfiÛÌÈ·, fiˆ˜ Î·È ÛÙË ¯ÒÚ· Ì·˜. ‚) ¢ÂÓ ··ÈÙ› ÂÍÂȉÈÎÂ˘Ì¤ÓÔ ÚÔÛˆÈÎfi ÁÈ· ÙË ¯ÔÚ‹ÁËÛ‹ Ù˘. Á) ∂›Ó·È ·ÓÒ‰˘ÓË ÁÈ· ÙÔÓ ·ÛıÂÓ‹. ‰) ªÔÚ› Ó· ¯ÔÚËÁËı› ÛÙÔ Û›ÙÈ ÙÔ˘ ·ÛıÂÓÔ‡˜. Â) Œ¯ÂÈ ÂÏ¿¯ÈÛÙË ‹ ηÌÈ¿ „˘¯ÔÏÔÁÈ΋ ÂÈ‚¿Ú˘ÓÛË ÁÈ· ÙÔ ·È‰›. ÛÙ) Œ¯ÂÈ ¯·ÌËÏfi ÎfiÛÙÔ˜. ∏ ÂÍ·ÎÚ›‚ˆÛË Ù˘ ȉ·ÓÈ΋˜ ‰ÔÛÔÏÔÁ›·˜ Î·È Ë ¯Ú‹ÛË Î·È ¿ÏÏˆÓ ÂÈÛÓÂfiÌÂÓˆÓ ÙÔÈÎÒÓ ÛÙÂÚÔÂȉÒÓ Ì ÙÔÓ ›‰ÈÔ ÙÚfiÔ ¯ÔÚ‹ÁËÛ˘, ÌÔÚ› Ó· ηıÈÂÚÒÛÂÈ ¤Ó· ηÈÓÔ‡ÚÁÈÔ ıÂÚ·¢ÙÈÎfi Û¯‹Ì·, ‰ËÌÈÔ˘ÚÁÒÓÙ·˜ Ӥ˜ ÚÔÔÙÈΤ˜ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·È‰ÈÒÓ Ì ‹ÈÔ-̤ÙÚÈÔ Î·È Ì¤ÙÚÈÔ-‚·Ú‡ croup ÛÙÔ Û›ÙÈ ÙÔ˘˜. À„ËÏfiÙÂÚ˜ ‰fiÛÂȘ ¢¶ª ı· ÌÔÚÔ‡Û·Ó ÂӉ¯Ô̤ӈ˜, Ó· Â›Ó·È ÈÔ ·ÔÙÂÏÂÛÌ·ÙÈΤ˜. µÈ‚ÏÈÔÁÚ·Ê›· 1. Jaff DM. The treatment of crÔup with glucocorticoids. N Engl J Med 1998;339:553. 2. Fitzqerald D, Melliw C, Johnson M, Allen H, Cooper P, Van Asperen P. Nebulized budesonide is as effective as nebulized adrenaline in moderately severe croup. Pediatrics 1996;97:722-725. 3. Taussing LM, Castro O, Beaudry PH, Fox WW, Bureau M. Treatment of laryngotracheobronchitis (croup): use of intermittent positive pressure breathing and racemic epinephrine AJDC 1975;129:790-793. 4. Fogel JM, Berg IZ, Gerer MA, Shorter CB. Racemic epinephrine in the treatment of croup: nebulization alone versus nebulization with intermittent positive pressure breathing J Pediatr 1982;101:1028-1031. 5. Waisman Y, Klein BL, Boenning DA, Younp GM, Chamberlain JM, O’Donnell R, Ochsenschlager RW. Prospective randomized double-blind study comparing Lepinephrine and racemic epinephrine aerosols in the treatment of laryngotr·cheitis (Croup) Pediatrics 1992;89:302-306. 6. Kristiansson S, Berg-Kelly, Winso E. Inhalation of racemic adrenaline in the treatment of mild and moderately severe croup. Acta Paediatr 1994;83:1156-1160. 7. Prendergast M, Jones JS, Hartman D. Racemic epinephrine in the treatment of laryngotracheitis: can we identify children for outpatient therapy? Am J Emerg Med 1994;12:613-616. 8. Martenson B, Nilson G, Torbjat J. The effect of corti-

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costeroids in the treatment of pseudo-croup. Acta Otolaryngl Suppl 1969;158:6271. Kairns S, Olmstead EM, O’Connor GT. Steroid treatment of laryngotracheitis: a meta-analysis of the evidence from randomized trials. Pediatrics 1989;83:683-693. Husby S, Agertort L, Mortensen, Pedersen S. Treatment of croup with nebulised steroid (budesonide): a double blind,placebo controlled study. Arch Dis Child 1993;68:352-325. Tibbals J, Shann FA, Landau LI. Placebo-controlled trial of prednisolone in children incubated for croup. Lancet 1992;340:745-748. Super DM, Cartelli NA, Brooks LJ, Lembo RM, Kumar ML. A prospective randomized double-blind study to evaluate the effect of dexamethasone in acute laryngotracheitis. J Pediatr 1989;115:323-329. Kuusela AL, Vesikari T. A randomized double-blind, placebo-controlled trial of dexamethasone and racemic epinephrine in the treatment of croup. Acta Paediatr Scand 1988;77:99-104. Klassen TP, Feldam ME, Watters LK, Sutclife T, Rowe PC, et al. Nebulized budesonide for children with mild-tomoderate croup. N Engl J Med 1994;331:285-289. Johnson DW, Jacobson S, Edney PC, Hadfield P, Mundy ME, Schuh S. A comparison of nebulized budesonide, intramuscular dexamethasone, and placebo for moderately severe croup. N Engl J.Med 1998;339:498-503. Klassen TP, Watters LK, Feldman ME, Sutcliffet RW*, Rowe PC. The efficacy of nebulized budesonide in dexamethasone-treated outpatients with croup. Pediatrics

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1998;97:463-466. 17. Geelhoed C. Croup. Pediatr Pulmonol 1997;23:370-374. 18. Downes JJ, Raphaely RC. Pediatric intensive care. Anesthesiology 1975;43:238-250. 19. Freelander M, Van Asperen PP. Nebulizer versus spacer in children with acute asthma Br Med J 1984;288:1873-1874. 20. O’Callaghan C, Milner AD, Swarbrick A. Spacer device with face mask attachment for giving bronchodilators to infants with asthma. Br Med J 1989;298:160-161. 21. Loew D, Schuster O, Graul EH. Dose-dependent pharmacokinetics, of dexamethoxasone. Eur J Clin Pharmacol 1986;30:225-230. 22. Hess D, Fisher D, Williams P, Pooler S, Kacmaker RM. Medication nebulizer performance effects of diluent volume, nebulizer flow, and nebulizer brand. Chest 1996;110:498-505. 23. Simonson B. Anatomical and pathophysiological consideration in aerosol therapy. Eur J Respir Dis 1982;119:7-14. 24. Leipzig B, Oski F, Cummngs CW, Stockman JA, Swender P. A prospective randomized study to determine the efficacy of steroids in the treatment of croup. J Pediatr 1979;94:194-196. 25. Newhouse MT, Dolovich MB. Control of asthma by aerosols. N Engl J Med 1986;315:870-874. HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 11-09-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 11-12-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ¶·Ó·ÁÈÒÙ˘ ™Ù·ÌÔ‡Ï˘ ∫.À. ™ÎÈ¿ıÔ˘ 37002 ™ÎÈ¿ıÔ˜


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∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞

Paediatriki 2001;64:65-71

ORIGINAL PAPER

∂›‰· ‚ÈÙ·ÌÈÓÒÓ ∞ Î·È ∂ ÛÙÔÓ ÔÚfi ·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË Û ıÂÚ·›· ˘ÔηٿÛÙ·Û˘ Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì· Î·È ÏÈÔ‰È·Ï˘Ù¤˜ ‚Èٷ̛Ә ™. ¡Ô‡ÛÈ·-∞Ú‚·ÓÈÙ¿ÎË1, ∞. °·ÏÏ‹-∆ÛÈÓÔÔ‡ÏÔ˘2, ¡. ƒÔ˘ÌȤ˜3, ∫. ∆ÂÓÙ˙›‰Ô˘4, ª. •ÂÊÙ¤ÚË4

Serum vitamin A and E levels in cystic fibrosis patients supplemented with pancreatic enzymes and liposoluble vitamins S. Nousia-Arvanitakis1, A. Galli-Tsinopoulou2, N. RÔubies3, K. Tentzidou4, M. Xefteri4

¶ÂÚ›ÏË„Ë: √È ‰È·Ù·Ú·¯¤˜ ıÚ¤„˘ ÛÙËÓ Î˘ÛÙÈ΋ ›ÓˆÛË ÔÊ›ÏÔÓÙ·È ÛÙËÓ ·ÓÂ¿ÚÎÂÈ· Ù˘ Â͈ÎÚÈÓÔ‡˜ ÌÔ›Ú·˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜. ™ÙÔÓ ÔÚfi 41 ·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË Î·È ·ÁÎÚ·ÙÈ΋ ·ÓÂ¿ÚÎÂÈ·, ËÏÈΛ·˜ 8 ˆ˜ 32 ¯ÚfiÓˆÓ, ÌÂÙÚ‹ıËÎ·Ó Ù· Â›‰· ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ Î·È ÙˆÓ ‚ÈÙ·ÌÈÓÒÓ ∞ Î·È ∂. √È ·ÛıÂÓ›˜ ÂÏ¿Ì‚·Ó·Ó Ì ÙË ‰È·ÙÚÔÊ‹ ÙÔ˘˜ 150% ÙˆÓ ··ÈÙÔ‡ÌÂÓˆÓ ËÌÂÚ‹ÛÈˆÓ ·Ó·ÁÎÒÓ Î·È 300% ÙˆÓ ·Ó·ÁÎÒÓ ÙÔ˘˜ Û ‚Èٷ̛Ә ∞ Î·È ∂, 6000 IU (1800Ìg) Î·È 100mg, ·ÓÙ›ÛÙÔȯ·. ªÂÏÂÙ‹ıËÎ·Ó ÔÈ ›‰ÈÔÈ ·Ú¿ÁÔÓÙ˜ Û 41 ·ÛıÂÓ›˜ Ì ÎÔÈÏÈÔοÎË, Û ‰›·ÈÙ· ÂχıÂÚË ÁÏÔ˘Ù¤Ó˘ Î·È Ê˘ÛÈÔÏÔÁÈÎfi ÂÓÙÂÚÈÎfi ‚ÏÂÓÓÔÁfiÓÔ, ηıÒ˜ Â›Û˘ Î·È 41 ˘ÁÈ‹ ·È‰È¿ ·ÓÙ›ÛÙÔȯ˘ ËÏÈΛ·˜. ∆· Â›‰· ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ Î·È Ù˘ ‚ÈÙ·Ì›Ó˘ ∂ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο (1,21±0,22 Î·È 10,7±4,0 mg/L, ·ÓÙ›ÛÙÔȯ·) ·ÏÏ¿ ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· Û ۇÁÎÚÈÛË Ì ·˘Ù¿ ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÔÈÏÈÔοÎË (1,02±0,10 Î·È 7,6±3,0 mg/L, ·ÓÙ›ÛÙÔȯ·) Î·È ÙˆÓ ˘ÁÈÒÓ ·È‰ÈÒÓ (0,94±0,12 Î·È 10,9±5,0, ·ÓÙ›ÛÙÔȯ·). ∞ÓÙ›ıÂÙ·, Ù· Â›‰· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ΢ÛÙÈ΋ ›ÓˆÛË (0,30±0,09 mg/L) ‹Ù·Ó ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ· Û ۇÁÎÚÈÛË ÙfiÛÔ Ì ٷ Â›‰· ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÔÈÏÈÔοÎË (0,39±0,16 mg/L), fiÛÔ Î·È Ì ٷ Â›‰· ÙˆÓ ˘ÁÈÒÓ ·È‰ÈÒÓ (0,56±0,16 mg/L). ∏ ıÂÚ·›· ˘ÔηٿÛÙ·Û˘ Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì·, 6000 IU (1800 Ìg) ‚ÈÙ·Ì›Ó˘ ∞ Î·È 100 mg ‚ÈÙ·Ì›Ó˘ ∂ Û¯ÂÙ›˙ÂÙ·È ÌÂ Ê˘ÛÈÔÏÔÁÈο Â›‰· „¢‰·ÚÁ‡ÚÔ˘ Î·È Ù˘

Abstract: Malnutrition in cystic fibrosis is associated with exocrine pancreatic insufficiency. We measured serum zinc and vitamins A and E in the serum of 41 patients (age range: 8-32 years) with cystic fibrosis and exocrine pancreatic insufficiency. The patients were on a closely monitored pancreatic enzyme replacement therapy and a diet providing 150% of daily requirements and 300% of vitamins A and E requirements (6000 IU or 1800 Ìg and 100 mg, respectively). Similar measurements were performed in 41 patients with celiac disease (matched for age) who were on gluten free diet and had a normal intestinal morphology, documented by biopsy, as well as in 41 normal individuals matched for age who served as controls. Serum zinc and vitamin E levels were normal in the cystic fibrosis group (1.21±0.22 and 10.7±4.0 mg/L, respectively) but significantly higher compared to the celiac disease group (1.02±0.10 and 7.6±3.0 mg/L, respectively) and to normal controls (0.94±0.12 and 10.9±5.0, respectively). However, vitamin A serum concentration was low in cystic fibrosis (0.30±0.09 mg/L) and significantly lower in comparison to that in the group with celiac disease (0.39±0.16 mg/L) and the group of normal children (0.55±0.16 mg/L). Adequate supplementation with pancreatic enzymes, 6000 IU (1800Ìg) of vitamin A and 100mg

1 ∞Ó·ÏËÚÒÙÚÈ· ∫·ıËÁ‹ÙÚÈ· ¶·È‰È·ÙÚÈ΋˜°·ÛÙÚÂÓÙÂÚÔÏÔÁ›·˜, ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£ 2 ¶·È‰›·ÙÚÔ˜, EÈÌÂÏ‹ÙÚÈ· ∂™À ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£ 3 ∂›ÎÔ˘ÚÔ˜ ∫·ıËÁËÙ‹˜, ∂ÚÁ·ÛÙ‹ÚÈÔ ∫ÏÈÓÈ΋˜ ¢È·ÁÓˆÛÙÈ΋˜ Î·È ¶ÚÔ·È‰Â˘ÙÈ΋˜ ¶·ıÔÏÔÁ›·˜, ∆Ì‹Ì· ∫ÙËÓÈ·ÙÚÈ΋˜ ∞¶£ 4 ¶·È‰›·ÙÚÔ˜, ∂ÈÛÙËÌÔÓÈÎfi˜ ™˘ÓÂÚÁ¿Ù˘, ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£

1 Assoc. Professor of Pediatric Gastroenterology 4th Pediatric Clinic, Aristotelion University of Thessaloniki 2 Staff Pediatrician, Register 4th Pediatric Clinic Aristotelion University of Thessaloniki 3 Assistant Professor, Division of Veterinary Medicine Laboratories of Clinical Diagnostic and Academic Pathology Aristotelion University of Thessaloniki 4 Pediatrician, Scientific Collaborator, 4th Department Aristotelion University of Thessaloniki

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Paediatriki 2001;64:65-71

‚ÈÙ·Ì›Ó˘ ∂, ·ÏÏ¿ ¯·ÌËÏ¿ Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙÔÓ ÔÚfi ·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË ËÏÈΛ·˜ >8 ¯ÚfiÓˆÓ. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ Ë ¯ÔÚËÁÔ‡ÌÂÓË ÔÛfiÙËÙ· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ‰ÂÓ ·ÓÙ·ÔÎÚ›ÓÂÙ·È ÛÙȘ ··ÈÙ‹ÛÂȘ ·˘ÙÒÓ ÙˆÓ ·ÛıÂÓÒÓ.

of vitamin E is associated with normal serum levels of zinc and vitamin E but low levels of vitamin A in patients with cystic fibrosis aged >8 years. This finding indicates that the usual supplementation doses of vitamin A in this group of CF patients do not fulfill their daily requirements.

§¤ÍÂȘ ÎÏÂȉȿ: ΢ÛÙÈ΋ ›ÓˆÛË, ‚ÈÙ·Ì›ÓË ∞, ‚ÈÙ·Ì›ÓË ∂, „¢‰¿ÚÁ˘ÚÔ˜.

Key words: cystic fibrosis, zinc, vitamin A, vitamin E.

∂ÈÛ·ÁˆÁ‹ ∞ÓÂ¿ÚÎÂÈ· Ù˘ Â͈ÎÚÈÓÔ‡˜ ÌÔ›Ú·˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜ Î·È ÏËÌÌÂÏ‹˜ ·ÔÚÚfiÊËÛË ÙˆÓ ÏÈÒÓ Î·È ÏÈÔ‰È·Ï˘ÙÒÓ ‚ÈÙ·ÌÈÓÒÓ ¯·Ú·ÎÙËÚ›˙ÂÈ 85% ÙˆÓ ·ÛıÂÓÒÓ Ì ΢ÛÙÈ΋ ›ÓˆÛË (∫π). ∏ ¯ÔÚ‹ÁËÛË ·ÁÎÚ·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ Î·È ‚ÈÙ·ÌÈÓÒÓ Â›Ó·È ·Ó·Áη›· Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ¶·Ú¿ ÙË ıÂÚ·›· ˘ÔηٿÛÙ·Û˘, ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÔÓÙ·È ¯·ÌËÏ¿ Â›‰· ‚ÈÙ·Ì›Ó˘ ∞ Î·È ∂ ÛÙÔÓ ÔÚfi ÔÚÈÛÌ¤ÓˆÓ ·ÛıÂÓÒÓ Ì ∫π (1,2). ∏ ‚ÈÙ·Ì›ÓË ∞ Â›Ó·È ·ÓÙÈÔÍÂȉˆÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜, Ô ÔÔ›Ô˜ ÚÔ¤Ú¯ÂÙ·È ·fi ÚÔÛ¯ËÌ·ÙÈṲ̂ÓË ‚ÈÙ·Ì›ÓË ∞ Î·È ·fi ηÚÔÙ¤ÓÈ·. ∏ ÚÔÛ¯ËÌ·ÙÈṲ̂ÓË ‚ÈÙ·Ì›ÓË ∞ Â›Ó·È ÁÓˆÛÙ‹ ˆ˜ ÚÂÙÈÓfiÏË. ∆· ηÚÔÙ¤ÓÈ· Â›Ó·È Î·Ù·ÓÂÌË̤ӷ Û ÌÂÌ‚Ú¿Ó˜, Ì·˙› Ì ÙË ‚ÈÙ·Ì›ÓË ∂, ¿ÏÏÔÓ ·ÓÙÈÔÍÂȉˆÙÈÎfi ·Ú¿ÁÔÓÙ·, Ô ÔÔ›Ô˜ Ê·›ÓÂÙ·È fiÙÈ ÚÔÛٷهÂÈ ÙËÓ ‚ÈÙ·Ì›ÓË ∞ ·fi ÙËÓ ÔÍ›‰ˆÛË (3). √È Ê˘ÛÈÔÏÔÁÈΤ˜ ËÌÂÚ‹ÛȘ ·Ó¿ÁΘ Û ‚ÈÙ·Ì›ÓË ∞ Â›Ó·È 2000 IU (600 Ìg). ™ÙËÓ ∫π, Û˘ÓÈÛÙ¿Ù·È ËÌÂÚ‹ÛÈ· ‰fiÛË 5000 ¤ˆ˜ 10000 IU (1200-3000 Ìg) ‚ÈÙ·Ì›Ó˘ ∞ Û ·ÛıÂÓ›˜ ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ 8 ¯ÚfiÓˆÓ (4-6). ∏ ‚ÈÙ·Ì›ÓË ∂ (·-ÙÔÎÔÊÂÚfiÏË) Â›Ó·È ·ÓÙÈÔÍÂȉˆÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜, Ô ÔÔ›Ô˜ ÚÔÛٷهÂÈ ÙȘ ÌÂÌ‚Ú¿Ó˜ ·fi ÙËÓ Î·Ù·ÛÙÚÔÊ‹ ÙËÓ ÔÔ›· ÂÈʤÚÔ˘Ó ÔÈ ÂχıÂÚ˜ ÔÍÂȉˆÙÈΤ˜ Ú›˙˜. ∏ ·-ÙÔÎÔÊÂÚfiÏË Û˘Ì‚¿ÏÏÂÈ ÛÙËÓ ÂÓÙÂÚÈ΋ ·ÔÚÚfiÊËÛË ÙˆÓ ·Î¤Ú·ÈˆÓ ‚-ηÚÔÙ¤ÓÈˆÓ Î·È Èı·ÓfiÓ ÙÚÔÔÔÈ› ÙË ÌÂÙ·ÙÚÔ‹ ÙˆÓ ‚-ηÚÔÙ¤ÓÈˆÓ Û ÚÂÙÈÓfiϘ (7). º·›ÓÂÙ·È fiÙÈ Ë ‚ÈÙ·Ì›ÓË ∂ ¤¯ÂÈ Û˘ÓÂÚÁÈ΋ ‰Ú¿ÛË Ì ¿ÏÏ· ·ÓÙÈÔÍÂȉˆÙÈο fiˆ˜ ÙÔ ÛÂÏ‹ÓÈÔ, ÙÔ ¯ÚÒÌÈÔ, ÙÔ ‚-ηÚÔÙ¤ÓÈÔ Î·È ÙË ‚ÈÙ·Ì›ÓË C. ∆· ·ÓÙÈÔÍÂȉˆÙÈο ȯÓÔÛÙÔȯ›·, fiˆ˜ ÔÈ ‚Èٷ̛Ә ∞ Î·È ∂, Ê·›ÓÂÙ·È fiÙÈ ·›˙Ô˘Ó ÚÔÛٷ٢ÙÈÎfi ÚfiÏÔ Û ηٷÛÙ¿ÛÂȘ ηٿ ÙȘ Ôԛ˜ Ë ·ÂÏ¢ı¤ÚˆÛË ÂÏ¢ı¤ÚˆÓ ÚÈ˙ÒÓ ÚÔηÏ› ηٷÛÙÚÔÊ‹ ÈÛÙÒÓ fiˆ˜ Û˘Ì‚·›ÓÂÈ ÛÙËÓ ∫π (8). √È ËÌÂÚ‹ÛȘ ·Ó¿ÁΘ Ù˘ ‚ÈÙ·Ì›Ó˘ ∂ ‰È·Ê¤ÚÔ˘Ó Ì ÙËÓ ËÏÈΛ· Î·È Î˘Ì·›ÓÔÓÙ·È ·fi 10 ˆ˜ 30 mg ËÌÂÚËÛ›ˆ˜ (9). √È ·Ó¿ÁΘ Û ‚ÈÙ·Ì›ÓË ∂ Â›Ó·È ·˘ÍË̤Ó˜ ÛÙËÓ ∫π ÏfiÁˆ Ù˘ ˘ÔÏÂÈfiÌÂÓ˘ ÏËÌÌÂÏÔ‡˜ ·ÔÚÚfiÊËÛ˘, ÌÂÙ¿ ÙË ¯ÔÚ‹ÁË-

ÛË ·ÁÎÚ·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ, ȉȷ›ÙÂÚ· fiÙ·Ó Ë ËÌÂÚ‹ÛÈ· ÚfiÛÏË„Ë Â›Ó·È ÏÔ‡ÛÈ· Û ÏÈ›‰È· Î·È ÔÏ˘·ÎfiÚÂÛÙ· ÏÈ·Ú¿ Ôͤ·. ™ÙËÓ ∫π Û˘ÓÈÛÙ¿Ù·È ËÌÂÚ‹ÛÈ· ‰fiÛË 100 mg ‚ÈÙ·Ì›Ó˘ ∂, Ì ÙËÓ ÔÔ›· ÂÈÙ˘Á¯¿ÓÔÓÙ·È ÈηÓÔÔÈËÙÈο Â›‰· ÛÙÔ ·›Ì· (10). √ „¢‰¿ÚÁ˘ÚÔ˜ Â›Ó·È ¤Ó· ÛËÌ·ÓÙÈÎfi ȯÓÔÛÙÔÈ¯Â›Ô ÙÔ ÔÔ›Ô ‚Ú›ÛÎÂÙ·È Û ÔÏÏ¿ fiÚÁ·Ó· (‹·Ú, ̇˜, ÔÛÙ¿, ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹˜) Î·È ÈÛÙÔ‡˜ (ÏÂ˘Î¿ Î·È ÂÚ˘ıÚ¿ ·ÈÌÔÛÊ·›ÚÈ·). ªÈÎÚ‹ ÔÛfiÙËÙ· Â›Ó·È ·ÔıËÎÂ˘Ì¤ÓË ÛÙÔ ‹·Ú Î·È ÎÈÓËÙÔÔÈÂ›Ù·È Û ηٷÛÙ¿ÛÂȘ ¤Ó‰ÂÈ·˜. ∞ÔÙÂÏ› Û˘ÛÙ·ÙÈÎfi ÔÏÏÒÓ ÂÓ˙‡ÌˆÓ (ηڂÔ͢ÂÙȉ¿ÛË, ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË, 5-ÓÔ˘ÎÏÂÔÙȉ¿ÛË). ∏ Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ÛÙË ‚ÈԉȷıÂÛÈÌfiÙËÙ· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ Â›Ó·È ÌÈ· ·fi ÙȘ ÏÂÈÙÔ˘ÚÁ›Â˜ ÙÔ˘. ∏ Û˘ÓÈÛÙÒÌÂÓË ËÌÂÚ‹ÛÈ· ÚfiÛÏË„Ë „¢‰·ÚÁ‡ÚÔ˘ Î˘Ì·›ÓÂÙ·È ·fi 5 ˆ˜ 15 mg (11). ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ó· ÂÍÂÙ¿ÛÂÈ Ù· Â›‰· ÙˆÓ ‚ÈÙ·ÌÈÓÒÓ ∞ Î·È ∂ Î·È ÙÔ˘ Zn ÂÈÏÂÁ̤Ó˘ ÔÌ¿‰·˜ ·ÛıÂÓÒÓ Ì ∫π, ÔÈ ÔÔ›ÔÈ ‚Ú›ÛÎÔÓÙ·Ó Û ıÂÚ·›· ˘ÔηٿÛÙ·Û˘ Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì·, Û ‰È·ÙÚÔÊ‹ Ë ÔÔ›· ·ÓÙ·ÔÎÚ›ÓÔÓÙ·Ó ÛÙÔ 150% ÙˆÓ ËÌÂÚ‹ÛÈˆÓ ·Ó·ÁÎÒÓ, Û 6000 IU (1800 Ìg) ‚ÈÙ·Ì›Ó˘ ∞ Î·È Û 100 mg ‚ÈÙ·Ì›Ó˘ ∂. ∏ ÌÂϤÙË Û¯Â‰È¿ÛÙËΠÂ›Û˘, Ì ÛÎÔfi Ó· Á›ÓÂÈ Û‡ÁÎÚÈÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ ÙˆÓ ·ÛıÂÓÒÓ Ì ∫π Ì ÙȘ ÙÈ̤˜ ‰‡Ô ÔÌ¿‰ˆÓ Ì·ÚÙ‡ÚˆÓ, ÔÌ¿‰·˜ ·ÙfiÌˆÓ Ì ÎÔÈÏÈÔοÎË Û ‰È·ÙÚÔÊ‹ ÂχıÂÚË ÁÏÔ˘Ù¤Ó˘ Î·È Ê˘ÛÈÔÏÔÁÈÎfi ÂÓÙÂÚÈÎfi ‚ÏÂÓÓÔÁfiÓÔ Î·È ÔÌ¿‰·˜ Ê˘ÛÈÔÏÔÁÈÎÒÓ ·ÙfïÓ.

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ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ ∂ÍÂÙ¿ÛÙËÎ·Ó 41 ·ÛıÂÓ›˜ Ì ∫π, ËÏÈΛ·˜ 8 ˆ˜ 32 ¯ÚfiÓˆÓ (25 ·ÁfiÚÈ·, 16 ÎÔÚ›ÙÛÈ·) ÌÂ Û˘ÁΤÓÙÚˆÛË ¯ÏˆÚÈÔ‡¯ˆÓ ȉÚÒÙ· >60meq/L Î·È ·ÓÂ¿ÚÎÂÈ· Ù˘ Â͈ÎÚÈÓÔ‡˜ ÌÔ›Ú·˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜ (·ÁÎÚ·ÙÈ΋ ÂÏ·ÛÙ¿ÛË ÎÔÚ¿ÓˆÓ <200Ìg/g Î·È Ï›Ô˜ ÎÔÚ¿ÓˆÓ 24ÒÚÔ˘ >4g. §¿Ì‚·Ó·Ó ıÂÚ·›· ˘ÔηٿÛÙ·Û˘ Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì· (60008000 ÌÔÓ¿‰Â˜ ÏÈ¿Û˘/Kg/µ™ ËÌÂÚËÛ›ˆ˜), ‰›·ÈÙ· Ë ÔÔ›· ·Ú›¯Â 150% ÙˆÓ ËÌÂÚËÛ›ˆÓ ·Ó·ÁÎÒÓ, 6000 πU (1800Ìg) ‚ÈÙ·Ì›Ó˘ ∞ Î·È 100mg ‚ÈÙ·Ì›Ó˘ ∂ ËÌÂÚËÛ›ˆ˜. ∏ Û¯¤ÛË ÚˆÙÂ˚ÓÒÓ, ÏÈÒÓ Î·È ˘‰·Ù·ÓıÚ¿ÎˆÓ ‹Ù·Ó 20, 40, 40%, ·ÓÙ›ÛÙÔȯ·. √ ÎÏÈÓÈÎfi˜ ‰Â›ÎÙ˘ Shwachman Î˘Ì·›ÓÔÓÙ·Ó


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 67

¶·È‰È·ÙÚÈ΋ 2001;64:65-71

·fi 65 ̤¯ÚÈ 98 Î·È ÙÔ ÛˆÌ·ÙÈÎfi ‚¿ÚÔ˜ ÙˆÓ ·ÛıÂÓÒÓ Î˘Ì·›ÓÔÓÙ·Ó ·fi ÙËÓ ‰¤Î·ÙË ˆ˜ ÙËÓ Â‚‰ÔÌËÎÔÛÙ‹ ¤ÌÙË ÂηÙÔÛÙÈ·›· ı¤ÛË. ∆· Â›‰· ÏÂ˘ÎˆÌ·Ù›Ó˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο (>3,8 g/dl) Î·È Ù· Â›‰· Ù˘ ¯ÔÏËÛÙÂÚfiÏ˘ Î˘Ì·›ÓÔÓÙ·Ó ·fi 120 ̤¯ÚÈ 180 mg/dl. ¶¤ÓÙ ·ÛıÂÓ›˜ ›¯·Ó Ë·ÙÈ΋ ‚Ï¿‚Ë Î·È ÂÏ¿Ì‚·Ó·Ó ·ÁˆÁ‹ Ì ԢÚÛÔ‰ÂÔ͢¯ÔÏÈÎfi Ô͇ (UDCA 20mg/Kg/µ™ ËÌÂÚËÛ›ˆ˜). √Ì¿‰Â˜ Ì·ÚÙ‡ÚˆÓ ·ÔÙ¤ÏÂÛ·Ó 41 ·ÛıÂÓ›˜ Ì ÎÔÈÏÈÔοÎË ·ÚfiÌÔÈ·˜ ËÏÈΛ·˜ Î·È Ê‡ÏÔ˘, ÔÈ ÔÔ›ÔÈ ‚Ú›ÛÎÔÓÙ·Ó Û ‰›·ÈÙ· ¯ˆÚ›˜ ÁÏÔ˘Ù¤ÓË Î·È Â›¯·Ó Ê˘ÛÈÔÏÔÁÈÎfi ‚ÏÂÓÓÔÁfiÓÔ ÛÙË ‚ÈÔ„›· ÂÓÙ¤ÚÔ˘ Î·È 41 Ê˘ÛÈÔÏÔÁÈο ¿ÙÔÌ·. ™Â fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜ Î·È Ì¿ÚÙ˘Ú˜ ¤ÁÈÓ ·ÈÌÔÏË„›· ÊÏ‚ÈÎÔ‡ ·›Ì·ÙÔ˜ ÙÔ ÔÔ›Ô ÙÔÔıÂÙ‹ıËΠ۠Á˘¿ÏÈÓ· ÛˆÏËÓ¿ÚÈ· ÁÈ· Ê˘ÁÔΤÓÙÚËÛË. √ ÔÚfi˜ „‡¯ıËΠÛÙÔ˘˜ -20ÔC ̤¯ÚÈ ÙËÓ Ë̤ڷ ÙÔ˘ ÚÔÛ‰ÈÔÚÈÛÌÔ‡. ∏ ÌÂϤÙË ‰ÈÂÓÂÚÁ‹ıËΠۇÌʈӷ Ì ÙË ‰È·Î‹Ú˘ÍË ÙÔ˘ Helsinki. 梉¿ÚÁ˘ÚÔ˜ ∏ Û˘ÁΤÓÙÚˆÛË ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ ÚÔÛ‰ÈÔÚ›ÛÙËÎÂ, ÌÂÙ¿ ·fi ·Ú·›ˆÛË ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜ Û ·ÔÛÙ·Á̤ÓÔ ÓÂÚfi (1:4), Û ʷÛÌ·ÙfiÌÂÙÚÔ ·ÙÔÌÈ΋˜ ·ÔÚÚfiÊËÛ˘ (Perkin Elmer Model 430) (12). µÈÙ·Ì›ÓË ∞ ∏ Û˘ÁΤÓÙÚˆÛË Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙÔÓ ÔÚfi ÚÔÛ‰ÈÔÚ›ÛÙËΠ̠ÙË ¯ÚˆÌ·ÙÔÌÂÙÚÈ΋ ̤ıÔ‰Ô ÙˆÓ Neeld Î·È Pearson, fiˆ˜ ·˘Ù‹ ÙÚÔÔÔÈ‹ıËΠ·fi ÙÔ˘˜ Roels Î·È Trout (13). √ ÔÚfi˜ ıÂÚÌ¿ÓıËΠÛÙÔ˘˜ 60ÔC Ì ·Èı·ÓÔÏÈÎfi ‰È¿Ï˘Ì· ˘‰ÚÔÍÂȉ›Ô˘ ÙÔ˘ Î·Ï›Ô˘ 1¡, ÁÈ· ÙËÓ Î·Ù·ÎÚ‹ÌÓÈÛË ÙˆÓ ÚˆÙÂ˚ÓÒÓ Î·È ÙËÓ Û·ˆÓÔÔ›ËÛË ÔÚÈÛÌ¤ÓˆÓ ÏÈȉ›ˆÓ, Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ Î·È ÙˆÓ ÂÛÙ¤ÚˆÓ Ù˘ ÚÂÙÈÓfiÏ˘ (·Ú¿ÁˆÁ· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞). ∏ ÚÂÙÈÓfiÏË, Ë ÔÔ›· ÚÔ‹Ïı ·fi ÙÔ˘˜ ÂÛÙ¤Ú˜ Ù˘ ÚÂÙÈÓfiÏ˘, ·Ú·Ï‹ÊıËΠ̠Âί‡ÏÈÛË Ì n- ÂÍ¿ÓÈÔ. √ ‰È·Ï‡Ù˘ ÂÍ·ÙÌ›ÛÙËΠ̠ÙËÓ Â›‰Ú·ÛË ÏÂÙÔ‡ Ú‡̷ÙÔ˜ ·˙ÒÙÔ˘ Î·È ÙÔ ˘fiÏÔÈÔ ‰È·Ï‡ıËΠ۠¯ÏˆÚÔÊfiÚÌÈÔ. ªÂÙ¿ ·fi ·ÓÙ›‰Ú·ÛË ·˘ÙÔ‡ ÙÔ˘ ‰È·Ï‡Ì·ÙÔ˜ Ì ÙÚÈÊıÔÚÔÍÈÎfi Ô͇, Ë ·ÔÚÚfiÊËÛË ÙÔ˘ ¯ÚÒÌ·ÙÔ˜ ÌÂÙÚ‹ıËΠÛÙ· 620 nm (Ê·ÛÌ·ÙÔʈÙfiÌÂÙÚÔ Hitachi 2000) ÁÈ· ÙÔÓ ÔÛÔÙÈÎfi ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ ‚ÈÙ·Ì›Ó˘ ∞. µÈÙ·Ì›ÓË ∂ ∏ ‚ÈÙ·Ì›ÓË ∂ (·-ÙÔÎÔÊÂÚfiÏË) ÛÙÔÓ ÔÚfi ÚÔÛ‰ÈÔÚ›ÛÙËΠ̠ÙË ÊıÔÚÈÛÌÔʈÙÔÌÂÙÚÈ΋ ̤ıÔ‰Ô ÙˆÓ Hansen Î·È Warwick (14). √È ÙÔÎÔÊÂÚfiϘ ·Ú·Ï·Ì‚¿ÓÔÓÙ·Ó ·fi ÙÔÓ ÔÚfi Ì Âί‡ÏÈÛË Ì n- ÂÍ¿ÓÈÔ, ÌÂÙ¿ ·fi ηٷÎÚ‹ÌÓÈÛË ÙˆÓ ÚˆÙÂ˚ÓÒÓ Ì ·Èı·ÓfiÏË. √ ÊıÔÚÈÛÌfi˜ ÙÔ˘ ‰È·Ï‡ÙË ÌÂÙÚÈfiÙ·Ó Û ∂à 295 nm Î·È ∂ª 340 nm Ì ʷÛÌ·ÙÔÊıÔÚÈÛÌfiÌÂÙÚÔ Hitachi Model F-2000. ™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË √È ÙÈ̤˜ ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘, Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ Î·È ∂ Û˘ÁÎÚ›ıËÎ·Ó Ì ÙȘ ·ÓÙ›ÛÙÔȯ˜ ÙˆÓ Ì·ÚÙ‡ÚˆÓ (t-student’s test).

∞ÔÙÂϤÛÌ·Ù· ∆· Â›‰· ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ ÛÙÔÓ ÔÚfi ÙˆÓ ˘ÁÈÒÓ ·ÙfiÌˆÓ Î˘Ì¿ÓıËÎ·Ó ·fi 0,70 ̤¯ÚÈ 1,15 mg/L Ì ̤ÛË ÙÈÌ‹ Î·È ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË 0,94±0,12mg/L. ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π, ÔÈ ÙÈ̤˜ ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ Î˘Ì¿ÓıËÎ·Ó ·fi 0,75 ̤¯ÚÈ

Paediatriki 2001;64:65-71

1,70 (1,21±0,22) mg/L Î·È ÛÙËÓ ÔÌ¿‰· Ù˘ ∫ÔÈÏÈÔο΢ ·fi 0,82 ̤¯ÚÈ 1,20 (1,02±0,10) mg/L. √È ÙÈ̤˜ ‹Ù·Ó ˘„ËÏfiÙÂÚ˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π ·’ fi,ÙÈ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÎÔÈÏÈÔοÎË Î·È ÛÙ· ˘ÁÈ‹ ·È‰È¿. ∏ ‰È·ÊÔÚ¿ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ (tind 5,1453, p<0,001 Î·È tind 7,1174, p<0,001) (∂ÈÎfiÓ· 1). ∆· Â›‰· Ù˘ ‚ÈÙ·Ì›Ó˘ ∂ ÛÙÔÓ ÔÚfi ÙˆÓ ˘ÁÈÒÓ ·ÙfiÌˆÓ Î˘Ì¿ÓıËÎ·Ó ·fi 5 ̤¯ÚÈ 20 mg/L Ì ̤ÛË ÙÈÌ‹ Î·È ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË 10,9±5,0 mg/L. ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π, ÔÈ ÙÈ̤˜ Ù˘ ‚ÈÙ·Ì›Ó˘ ∂ Î˘Ì¿ÓıËÎ·Ó ·fi 4 ˆ˜ 24 (10,7±4,0) mg/L Î·È ÛÙËÓ ÔÌ¿‰· Ù˘ ∫ÔÈÏÈÔο΢ ·fi 4 ˆ˜ 12 (7,6±3,0) mg/L. √È Ì¤Û˜ ÙÈ̤˜ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ˘„ËÏfiÙÂÚ˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π ·fi ·˘Ù¤˜ ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÔÈÏÈÔοÎË Î·È Û˘ÁÎÚ›ÛÈ̘ Ì ÂΛӘ ÙˆÓ ˘ÁÈÒÓ ·ÙfiÌˆÓ (tind 4,4512, p<0,001 Î·È tind 0,7308 mg/L Ì p=0,2335, ·ÓÙ›ÛÙÔȯ·) (∂ÈÎfiÓ· 2). ∆· Â›‰· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙÔÓ ÔÚfi ÙˆÓ ˘ÁÈÒÓ ·ÙfiÌˆÓ Î˘Ì¿ÓıËÎ·Ó ·fi 0,35 ̤¯ÚÈ 0,80 Ì ̤ÛË ÙÈÌ‹ Î·È ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË 0,55±0,16 mg/L. ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π, ÔÈ ÙÈ̤˜ Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ Î˘Ì¿ÓıËÎ·Ó ·fi 0,16 ˆ˜ 0,58 (0,30±0,09) mg/L Î·È ÛÙËÓ ÔÌ¿‰· Ù˘ ∫ÔÈÏÈÔο΢ ·fi 0,18 ˆ˜ 0,71 (0,39±0,16) mg/L. √È Ì¤Û˜ ÙÈ̤˜ ‹Ù·Ó ¯·ÌËÏfiÙÂÚ˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π Û˘ÁÎÚÈÓfiÌÂÓ˜ Ì ·˘Ù¤˜ ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÔÈÏÈÔοÎË Î·È ÙˆÓ ˘ÁÈÒÓ ·È‰ÈÒÓ. ∏ ‰È·ÊÔÚ¿ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ (tind -3,6345, p<0,001 Î·È tind 9,1881, p<0,001, ·ÓÙ›ÛÙÔȯ·) (∂ÈÎfiÓ· 3). ™˘˙‹ÙËÛË ∆· Â˘Ú‹Ì·Ù· Ù˘ ÌÂϤÙ˘ ·˘Ù‹˜ ‰Â›¯ÓÔ˘Ó ¯·ÌËÏ¿ Â›‰· ‚ÈÙ·Ì›Ó˘ ∞ Û ·ÛıÂÓ›˜ Ì ∫π ÔÈ ÔÔ›ÔÈ ‚Ú›ÛÎÔÓÙ·Ó Û ıÂÚ·›· Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì· Î·È ‚ÈÙ·ÌÈÓÔ‡¯Ô Û··ÛÌ·, ÂÓÒ ÔÈ ÙÈ̤˜ Ù˘ ‚ÈÙ·Ì›Ó˘ ∂ Î·È ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜. √ „¢‰¿ÚÁ˘ÚÔ˜ ¤¯ÂÈ ÂÚÈÏËÊı› ÛÙË ÌÂϤÙË ÁÈ·Ù› ÂËÚ¿˙ÂÈ ÙËÓ ·ÔÚÚfiÊËÛË, ÙË ÌÂÙ·ÊÔÚ¿ Î·È ÙË ¯ÚËÛÈÌÔÔ›ËÛË Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ (15). ∞ÔÚÚÔÊ¿Ù·È ÛÙÔ ÏÂÙfi ¤ÓÙÂÚÔ Î·È Î˘Ú›ˆ˜ ÛÙÔ ‰ˆ‰Âη‰¿ÎÙ˘ÏÔ (11). À¿Ú¯ÂÈ Â˘Ú›· ¤ÓÙÂÚÔ-·ÁÎÚ·ÙÈ΋ ΢ÎÏÔÊÔÚ›· ÛÙËÓ ÔÔ›· Ë ÂÓÙÂÚÈ΋ ¤ÎÎÚÈÛË ÙÔ˘ ÂÓ‰ÔÁÂÓÔ‡˜ „¢‰·ÚÁ‡ÚÔ˘ ÌÔÚ› Ó· Â›Ó·È ÙÂÏÈο ‰ÈÏ¿ÛÈ· ·fi ÙËÓ ÔÛfiÙËÙ· Ë ÔÔ›· ηٷӷÏÒÓÂÙ·È ËÌÂÚËÛ›ˆ˜ Î·È ÁÈ· ÙÔ ÏfiÁÔ ·˘Ùfi Ë ¿ˆ ÂÓÙÂÚÈ΋ ·ÔÚÚfiÊËÛË ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ Â›Ó·È ÏÂÈÙÔ˘ÚÁÈο Ôχ ÛËÌ·ÓÙÈ΋ (10). ™ÙËÓ Î˘ÎÏÔÊÔÚ›· Ô „¢‰¿ÚÁ˘ÚÔ˜ Û˘Ó‰¤ÂÙ·È Ì ÏÂ˘ÎˆÌ·Ù›ÓË (80%), Ì ÙËÓ ·-2 Ì·ÎÚÔÛÊ·ÈÚ›ÓË (15%) Î·È Ì ¿ÏϘ ÚˆÙ½Ó˜. ∆Ô ‹·Ú ·›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙÔÓ ¤ÏÂÁ¯Ô ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘. ∏

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 68

¶·È‰È·ÙÚÈ΋ 2001;64:65-71

Zn mg/L

∫À™∆π∫∏ π¡ø™∏

2,0 1,9 1,8 1,7 1,6 1,5 1,4 1,3 1,2 1,1 1,0 0,9 0,8 0,7 0,6 0,5 0,4 0,3 0,2 0,1 0,0

Paediatriki 2001;64:65-71

∫√π§π√∫∞∫∏

t=5,1453 p<0,001

ª∞ƒ∆Àƒ∂™

t=7,1174 p<0,001

∂ÈÎfiÓ· 1. ™ÙÔÓ Î¿ıÂÙÔ ¿ÍÔÓ· ·Ó·ÁÚ¿ÊÔÓÙ·È Ù· Â›‰· ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ ÛÙÔÓ ÔÚfi. ∆· Â›‰· ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ ÛÙÔÓ ÔÚfi ·ÛıÂÓÒÓ Ì ∫π Î˘Ì·›ÓÔÓÙ·Ó ·fi 0,70 ̤¯ÚÈ 1,70 mg/L. ∞˘Ù¤˜ ÔÈ ÙÈ̤˜ Â›Ó·È ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ˜ ·fi ·˘Ù¤˜ ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÔÈÏÈÔοÎË ·ÏÏ¿ Î·È ÙˆÓ ˘ÁÈÒÓ ·È‰ÈÒÓ.

Zn mg/L 24 23 22 21 20 19 18 17 16 15 14 13 12 11 10 9 8 7 6 5 4 3 2 1 0

∫À™∆π∫∏ π¡ø™∏

∫√π§π√∫∞∫∏

t=4,4512 p<0,001

ª∞ƒ∆Àƒ∂™

t=0,7308 p<0,001

∂ÈÎfiÓ· 2. √È ÙÈ̤˜ Ù˘ ‚ÈÙ·Ì›Ó˘ ∂ ·Ó·ÁÚ¿ÊÔÓÙ·È ÛÙÔÓ Î¿ıÂÙÔ ¿ÍÔÓ·. √È ÙÈ̤˜ Ù˘ ‚ÈÙ·Ì›Ó˘ ∂ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Ì ∫π Î˘Ì·›ÓÔÓÙ·Ó ·fi 4 ̤¯ÚÈ 24 mg/L. ∞˘Ù¤˜ ÔÈ ÙÈ̤˜ ‹Ù·Ó Û˘ÁÎÚ›ÛÈ̘ Ì ÂΛӘ ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ·ÙfiÌˆÓ Î·È ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ˜ ·fi ·˘Ù¤˜ ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÔÈÏÈÔοÎË.

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µπ∆∞ªπ¡∏ mg/L

∫À™∆π∫∏ π¡ø™∏

Paediatriki 2001;64:65-71

∫√π§π√∫∞∫∏

ª∞ƒ∆Àƒ∂™

1,0 0,9 0,8 0,7 0,6 0,5 0,4 0,3 0,2 0,1 0,0

t=3,6345 p<0,001

t=9,1881 p<0,001

∂ÈÎfiÓ· 3. √È ÙÈ̤˜ Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ·Ó·ÁÚ¿ÊÔÓÙ·È ÛÙÔÓ Î¿ıÂÙÔ ¿ÍÔÓ·. √È ÙÈ̤˜ Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Ì ∫π Î˘Ì·›ÓÔÓÙ·Ó ·fi 0,16 ̤¯ÚÈ 0,58 mg/L. ∞˘Ù¤˜ ÔÈ ÙÈ̤˜ Â›Ó·È ÛËÌ·ÓÙÈο ÌÈÎÚfiÙÂÚ˜ ·fi ·˘Ù¤˜ ÙˆÓ ·ÛıÂÓÒÓ Ì ÎÔÈÏÈÔοÎË Î·È ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ·È‰ÈÒÓ.

ÌÂÙ·ÏÏÔıÂÈÔÓ›ÓË Û˘Ì‚¿ÏÏÂÈ ÛÙȘ ÂÓ‰ÔË·ÙÈΤ˜ ·Ôı‹Î˜ „¢‰·ÚÁ‡ÚÔ˘ ÁÈ·Ù› Û˘Ó‰¤ÂÈ ¿ÙÔÌ· „¢‰·ÚÁ‡ÚÔ˘. ™ÙËÓ ∫π, Ë ·ÔÚÚfiÊËÛË ÙÔ˘ „¢‰·ÚÁ‡ÚÔ˘ ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ·ÁÎÚ·ÙÈ΋ ·ÓÂ¿ÚÎÂÈ· (16) Î·È ‚ÂÏÙÈÒÓÂÙ·È Ì ÙË ¯ÔÚ‹ÁËÛË ·ÁÎÚ·ÙÈÎÒÓ Âί˘ÏÈÛÌ¿ÙˆÓ (17-19). ™‡Ìʈӷ Ì ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË, ÔÈ ÙÈ̤˜ „¢‰·ÚÁ‡ÚÔ˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ∫π. ∆· Â˘Ú‹Ì·Ù· ·˘Ù¿ ‹Ù·Ó Û‡Ìʈӷ Ì ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÈ ·ÛıÂÓ›˜ Ï¿Ì‚·Ó·Ó Â·Ú΋ ıÂÚ·›· ˘ÔηٿÛÙ·Û˘ Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì· Î·È ÙÚÈÏ¿ÛÈ· ‰fiÛË ÙˆÓ ËÌÂÚËÛ›ˆÓ ·Ó·ÁÎÒÓ Û „¢‰¿ÚÁ˘ÚÔ Ì ÙË ÌÔÚÊ‹ Û΢·ÛÌ¿ÙˆÓ ·ÏÏ¿ Î·È Ì ˙ˆÈΤ˜ ÚˆÙ½Ó˜ ÛÙË ‰›·ÈÙ¿ ÙÔ˘˜. ™‡Ìʈӷ Ì ÙË ÌÂϤÙË ·˘Ù‹, Ë ‚ÈÙ·Ì›ÓË ∂ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋ ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ Ì ∫π, ÔÈ ÔÔ›ÔÈ ÂÏ¿Ì‚·Ó·Ó Â·ÚΛ˜ ÔÛfiÙËÙ˜ ·ÁÎÚ·ÙÈÎÒÓ ÂÓ˙‡ÌˆÓ Î·È ‚ÈÙ·ÌÈÓÒÓ. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ Ë Â·Ú΋˜ ıÂÚ·›· ˘ÔηٿÛÙ·Û˘ Ì ·ÁÎÚ·ÙÈο ¤Ó˙˘Ì· Î·È ‚ÈÙ·Ì›ÓË ∂ Ô‰ËÁ› ÛÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ‚ÈÙ·Ì›Ó˘ ∂ ÛÙÔÓ ÔÚfi. À¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ fiÙÈ Ù· Â›‰· Ù˘ ‚ÈÙ·Ì›Ó˘ ∂ ÛÙËÓ Î˘ÎÏÔÊÔÚ›· Â›Ó·È ‰ÔÛÔÂÍ·ÚÙÒÌÂÓ· (20). ™‡Ìʈӷ Ì ٷ ·ÔÙÂϤÛÌ·Ù· Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘, Ë ‚ÈÙ·Ì›ÓË ∞ ‹Ù·Ó ¯·ÌËÏ‹ ÛÙÔÓ ÔÚfi ·ÛıÂÓÒÓ Ì ∫π ·Ú¿ ÙËÓ Î·ıËÌÂÚÈÓ‹ ¯ÔÚ‹ÁËÛË ‰fiÛ˘ 6000 IU (1800 Ìg) ‚ÈÙ·Ì›Ó˘ ∞. ∞˘Ùfi ÙÔ Â‡ÚËÌ· ÌÔÚ› Ó· ·Ô‰Ôı› ÛÙËÓ ¿Ô„Ë fiÙÈ Ë ‚ÈÙ·-

Ì›ÓË ∞ Â›Ó·È Î˘Ú›ˆ˜ ·ÔıËÎÂ˘Ì¤ÓË ÛÙÔ ‹·Ú Î·È ÛÙÔ˘˜ ÈÛÙÔ‡˜. °È· Ó· ·˘ÍËıÔ‡Ó Ù· Â›‰· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙËÓ Î˘ÎÏÔÊÔÚ›· Â›Ó·È ··Ú·›ÙËÙË Ë ¯ÔÚ‹ÁËÛË ÌÂÁ¿ÏˆÓ ‰fiÛÂˆÓ ‚ÈÙ·Ì›Ó˘. √ ΛӉ˘ÓÔ˜ Ó· ‰ËÌÈÔ˘ÚÁËı› ˘ÂÚ‚Èٷ̛ӈÛË ∞ Ê·›ÓÂÙ·È Ó· Â›Ó·È ÌÈÎÚfi˜ ·Ú¿ ÙËÓ ˘ÔηٿÛÙ·ÛË Ì ÌÂÁ¿Ï˜ ‰fiÛÂȘ (21). ∏ ·ÔÚÚfiÊËÛË Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÂÍ·ÚÙ¿Ù·È ·fi ÙË ¯ÔÏÈ΋ ¤ÎÎÚÈÛË, ÙËÓ ·ÔÚÚfiÊËÛË ÙˆÓ ÏÈÒÓ, ÙËÓ ÚfiÛÏË„Ë ÚˆÙÂ˚ÓÒÓ Ì ÙË ‰È·ÙÚÔÊ‹, ÙËÓ ·ÚÔ˘Û›· „¢‰·ÚÁ‡ÚÔ˘ Î·È ÙȘ ·Ó¿ÁΘ ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡ Û „¢‰¿ÚÁ˘ÚÔ (22). ∏ ‚ÈԉȷıÂÛÈÌfiÙËÙ· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ Â›Ó·È ÂÏ·Ùو̤ÓË Û ÔÚÈṲ̂Ó˜ ηٷÛÙ¿ÛÂȘ (Ë·ÙÈ΋ ÓfiÛÔ˜, ·ÁÎÚ·ÙÈ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·, ¤ÏÏÂÈ„Ë „¢‰·ÚÁ‡ÚÔ˘, ˘ÔÚˆÙÂ˚Ó·ÈÌ›·), ÔÈ Ôԛ˜ ıˆÚËÙÈο Â›Ó·È Û˘¯Ó¤˜ ÛÙËÓ ∫π ·ÏÏ¿ ›¯·Ó ·ÔÎÏÂÈÛÙ› ÛÙÔ˘˜ ·ÛıÂÓ›˜ ·˘Ù‹˜ Ù˘ ÌÂϤÙ˘. ∏ ÚÂÙÈÓfiÏË Î˘ÎÏÔÊÔÚ› ÛÙÔ Ï¿ÛÌ· Ì ٷ ¯˘ÏÔÌÈÎÚ¿ Î·È ·ÔıË·ÂÙ·È ÛÙÔ ‹·Ú ̤ۈ ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ ÙˆÓ ÏÈÔÚˆÙÂ˚ÓÒÓ (23). À¿Ú¯ÂÈ ‰È¿ÛÙ·ÛË ·fi„ÂˆÓ Û¯ÂÙÈο Ì ٷ Ë·ÙÈο ·Ôı¤Ì·Ù· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙËÓ ∫π (4,24). √ ΛӉ˘ÓÔ˜ ¤ÏÏÂȄ˘ ‚ÈÙ·Ì›Ó˘ ∞ ·˘Í¿ÓÂÈ Ì ÙËÓ ËÏÈΛ· (4). ∞ӷʤÚÂÙ·È ÌÂÁ¿ÏË ·ÒÏÂÈ· ‚ÈÙ·Ì›Ó˘ ∞ Ì ٷ ÎfiÚ·Ó· (25). √È ·ÛıÂÓ›˜ Ì ∫π Î·È Ë·ÙÈ΋ ÚÔÛ‚ÔÏ‹ ›¯·Ó Ê˘ÛÈÔÏÔÁÈο Â›‰· ‚ÈÙ·Ì›Ó˘ ∞. ∞˘Ùfi ÙÔ Â‡ÚËÌ· Èı·ÓfiÓ Ó· ÔÊ›ÏÂÙ·È ÛÙÔ ÁÂÁÔÓfi˜ fiÙÈ ÔÈ

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·ÛıÂÓ›˜ Ì Ë·ÙÈ΋ ÚÔÛ‚ÔÏ‹ ÂÏ¿Ì‚·Ó·Ó ÌÂÁ¿Ï˜ ‰fiÛÂȘ Ô˘ÚÛÔ‰ÂÔ͢¯ÔÏÈÎÔ‡ ÔͤԘ, ÙÔ ÔÔ›Ô ¤¯ÂÈ Ë·ÙÔÚÔÛٷ٢ÙÈ΋ ‰Ú¿ÛË Î·È ‚ÂÏÙÈÒÓÂÈ ÙÔÓ Ë·ÙÈÎfi ÌÂÙ·‚ÔÏÈÛÌfi ÙˆÓ ··Ú·ÈÙ‹ÙˆÓ ÏÈ·ÚÒÓ ÔͤˆÓ Î·È Ù˘ ÚÂÙÈÓfiÏ˘ ÛÙ· ·È‰È¿ Ì ∫π (26). ∂ÊfiÛÔÓ Ï·Ì‚¿ÓÔ˘Ó Â·ÚΛ˜ ÔÛfiÙËÙ˜ ·ÁÎÚ·ÙÈÎÒÓ Âί˘ÏÈÛÌ¿ÙˆÓ, 100 mg ‚ÈÙ·Ì›Ó˘ ∂ Î·È 6000 IU (1800 Ìg) ‚ÈÙ·Ì›Ó˘ ∞ ËÌÂÚËÛ›ˆ˜, ·ÛıÂÓ›˜ Ì ∫π ËÏÈΛ·˜ ÌÂÁ·Ï‡ÙÂÚ˘ ÙˆÓ ÔÎÙÒ ¯ÚfiÓˆÓ Î·È ·ÓÂ¿ÚÎÂÈ· Ù˘ Â͈ÎÚÈÓÔ‡˜ ÌÔ›Ú·˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜ ¤¯Ô˘Ó Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ „¢‰·ÚÁ‡ÚÔ˘ Î·È ‚ÈÙ·Ì›Ó˘ ∂ ·ÏÏ¿ ¯·ÌËϤ˜ ÙÈ̤˜ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙÔÓ ÔÚfi. ∆· Â›‰· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ÛÙËÓ Î˘ÎÏÔÊÔÚ›· ·ÔÙÂÏÔ‡Ó ÊÙˆ¯fi ‰Â›ÎÙË Ù˘ Â¿ÚÎÂÈ·˜ ‹ ·ÓÂ¿ÚÎÂÈ·˜ Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ (27), ÁÈ·Ù› ‰ÂÓ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙ· Ë·ÙÈο ·Ôı¤Ì·Ù· (28-30). ªÔÚԇ̠ӷ Û˘ÌÂÚ¿ÓÔ˘Ì fiÙÈ ÙÔ Â‡ÚËÌ· ·˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ ÔÈ ··ÈÙ‹ÛÂȘ ÙˆÓ ·ÛıÂÓÒÓ Ì ∫π ËÏÈΛ·˜ >8 ¯ÚfiÓˆÓ Û ‚ÈÙ·Ì›ÓË ∞ Â›Ó·È ˘„ËÏfiÙÂÚ˜ Î·È fiÙÈ Ë ËÌÂÚ‹ÛÈ· ‰fiÛË ÙˆÓ 6000 IU (1800 Ìg) ‰ÂÓ Â·ÚΛ. µÈ‚ÏÈÔÁÚ·Ê›· 1. Rayner RJ, Tyrrell JC, Hiller EJ, Marenah C, Neugebauer MA, Vernon SA et al. Night blindness and conjunctiva xerosis caused by vitamin A deficiency in patients with cystic fibrosis. Arch Dis Child 1989;64:1151-1156. 2. Congden PJ, Bruce G, Rothburn MM, Clarke PC, Littlewood JM, Kelleher J et al. Vitamin status in treated patients with cystic fibrosis. Arch Dis Child 1981;56:708-714. 3. Olson JA. Vitamin A and carotenoids as antioxidant in a physiological context. J Nutr Sci Vitaminol 1993; 39(Suppl):57-65. 4. Ramsey BW, Farrell PM, Pencharz P, and the Consensus Committee. Nutritional assessment and management in cystic fibrosis: a consensus report. Am J Clin Nutr 1992;55:108-116. 5. Carr SB, Bratney J. The role of vitamins in cystic fibrosis. J R Soc Med 2000;93(Sup 38):14-19. 6. Lindlblad A, Diczfalusy U, Hultcrantz R, Thorell A, Strandvik B. Vitamin A concentrations in the liver decrease with age in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1997;24;264-270. 7. Wang XD, Marini RP, Hebuterne X, Fox JG, Krinsky NI, Russell RM. Vitamin E enhances the lymphatic transport of beta-carotene and its conversion to vitamin A in the ferret. Gastroenterology 1995;108:719-726. 8. Rock CL, Jacob RA, Bowen PE. Update on the biological characteristics of the antioxidant micronutrients: vitamin C, vitamin E and the carotenoids. J Am Diet Assoc 1996;96: 693-702. 9. Sokol R. Metabolism and disorders of vitamin E in infancy and childhood. Internat Sem Pediatr Gastroenterol Nutr 1995;4:8-15. 10. Peters SA, Kelly FJ. Vitamin E supplementation in cystic

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fibrosis. J Pediatr Gastroenterol Nutr 1996; 22: 341-345. 11. Aggett PJ. Zinc. Annales Nesle 1994;52:94-106. 12. The Perkin-Elmer Corporation. Analytical Methods for Atomic Absorption Spectroscopy 1996;160-161. 13. Roels OA, Trout M. Vitamin A and carotene. In: Cooper GR and Kings JS, editors. Standard methods of clinical chemistry. New York and London: Academic Press; 1972. p. 215-230. 14. Hansen LG, Warnick WJ. A fluorometric micro method for serum tocopherol. Tech Bull Reg Med Tech 1966;36:131-136. 15. Christian P, West KP Jr. Interactions between zinc and vitamin A: an update. Am J Clin Nutr 1998;68(2 Suppl):435-441. 16. Jacob RA, Sandstead H H, Solomons NW, Rieger C, Rothberg R. Zinc status and vitamin A transport in cystic fibrosis. Am J Clin Nutr 1978;31:638-644. 17. Krebs N, Sontag M, Accurso F, Hambidge KM. Low plasma zinc concentrations in young infants with cystic fibrosis. J Pediatr 1998;133:761-764. 18. Easley D, Krebs N, Jefferson M, Miller L, Erskine J, Accurso F et al. Effect of pancreatic enzyme on zinc absorption in cystic fibrosis. J Pediatr GastrÔenterol Nutr 1998;26:136-139. 19. Safai-Kutti S, Selin E, Larsson E, Jagenburg R, Denfors I, Sten G et al. Zinc therapy in children with cystic fibrosis. Beitr Infusionsther 1991;27:104-114. 20. Borel P, Mekki N, Boirie Y, Partier A, Groiler P, AlexandreCouabau MC et al. Postprandial chylomicron vitamin E responses in healthy older subjects compared with younger ones. Eur J Clin Invest 1997;27:812-821. 21. James DR, Owen G, Campbell IA, Goodchild MC. Vitamin A absorption in cystic fibrosis: risk of hyper- vitaminosis A. Gut 1992;33:707-710. 22. Herrera MG. Vitamin A deficiency: Prevention and treatment. Internat Sem Paediatr Gastroenterol Nutr 1995;4:2-8. 23. ∆raber MG, Diamond SR, Lane JC, Brody RI, Kayden HJ. Beta-carotene- transport in human lipoproteins. Comparisons with a-tocopherol. Lipids 1994;29:665-669. 24. Underwood BA, Denning CR. Blood and liver concentrations of vitamins A and E in children with cystic fibrosis of the pancreas. Pediatr Res 1972;6:26-31 25. Ahmed F, Ellis J, Murphy J, Wootton S, Jackson AA. Excessive fecal losses of vitamin A (retinol) in cystic fibrosis. Arch Dis Child 1990;65:589-593. 26. Lepage G, Paradis K, Lacaille F, Sénéchal L, Ronco N, Champagne J et al. Ursodeoxycholic acid improves the hepatic metabolism of essential fatty acids and retinol in children with cystic fibrosis. J Pediatr 1997;130:52-58. 27. Amedee-Manesme O, Furr HC, Alvarez F et al. Biochemical indicators of vitamin A depletion in children with cholestasis. Hepatology 1985;5:1143-1148. 28. Mourey MS, Siegenthaler G, Amedee-Manesme O. Regulation of metabolism of retinol-binding protein by vitamin A status in children with biliary atresia. Am J Clin Nutr 1990;51:638-643. 29. Lancellotti L, D’Orazio C, Mastella G et al. Deficiency of


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vitamins A and E in cystic fibrosis is independent of pancreatic function and current enzyme and vitamin supplementation. Euro P J Pediatr1996;155:281-285. 30. Dodge JA, O’Rawe AM. nutritional aspects of cystic fibrosis (Review). Europ J Gastroenterol Hepatol 1006;8:739-743.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 27-06-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 27-11-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ™. ¡Ô‡ÛÈ·-∞Ú‚·ÓÈÙ¿ÎË ∆£ 322, £¤ÚÌË, 57001 £ÂÛÛ·ÏÔÓ›ÎË e-mail: sarvanit@med.auth.gr

¶ÂÚ›ÏË„Ë ¿ÚıÚÔ˘ ·fi ÙË µÈ‚ÏÈÔÁÚ·Ê›· ∆Ô ÈÛÙÔÚÈÎfi ¿ÛıÌ·ÙÔ˜ ÛÙÔ˘˜ ÁÔÓ›˜ Â›Ó·È ·Ú¿ÁÔÓÙ·˜ ÎÈÓ‰‡ÓÔ˘ ÂΉ‹ÏˆÛ˘ ·Ó·Ó¢ÛÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ Ì ‹ ¯ˆÚ›˜ Û˘ÚÚ›ÙÔ˘Û· ·Ó·ÓÔ‹ Û ‚Ú¤ÊË ËÏÈΛ·˜ οو ÙˆÓ 18 ÌËÓÒÓ1 ™ÎÔfi˜: ∏ Û¯¤ÛË ÌÂٷ͇ ·Ó·Ó¢ÛÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ Î·È ·ÏÏÂÚÁ›·˜ Û·Ó ·Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ÓfiÛÔ˘ ÌÂ Û˘ÚÚ›ÙÔ˘Û· ·Ó·ÓÔ‹ (wheezing) Û ‚Ú¤ÊË, ¤¯ÂÈ ·ÌÊÈÛ‚ËÙËı›. ÀÔı¤Û·Ì fiÙÈ Ë ‡·ÚÍË ÈÛÙÔÚÈÎÔ‡ ·ÏÏÂÚÁÈ΋˜ ÓfiÛÔ˘ ÛÙÔ˘˜ ÁÔÓ›˜ ı· ÌÔÚÔ‡Û ӷ Û¯ÂÙ›˙ÂÙ·È Ì ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ·Ó·Ó¢ÛÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ ÛÙ· ‚Ú¤ÊË, fiˆ˜ Â›Û˘ Î·È Ì ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· Û˘ÚÚ›ÙÔ˘Û·˜ ·Ó·ÓÔ‹˜ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÏÔÈÌÒÍÂˆÓ ·˘ÙÒÓ. ª¤ıÔ‰Ô˜: ∂ÎÙÈÌ‹ıËÎ·Ó ÚÔÔÙÈο 1.193 ‚Ú¤ÊË, ·fi ÙË Á¤ÓÓËÛË Ì¤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 18 ÌËÓÒÓ, Ì ÙËÏÂʈÓÈ΋ ÂÈÎÔÈÓˆÓ›· ‰‡Ô ÊÔÚ¤˜ ‚‰ÔÌ·‰È·›ˆ˜, Ì ÛÎÔfi ÙËÓ Î·Ù·ÁÚ·Ê‹ fiÏˆÓ ÙˆÓ ÂÂÈÛÔ‰›ˆÓ ÏÔÈÌÒÍÂˆÓ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡. ∞ÔÙÂϤÛÌ·Ù·: ∏ Û˘ÓÔÏÈ΋ Û˘¯ÓfiÙËÙ· ·Ó·Ó¢ÛÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ (all RI) ·ÚÔ˘Û›·˙ ̛· ÚÔԉ¢ÙÈ΋ ·‡ÍËÛË, ˆ˜ ÙË Ì¤ÁÈÛÙË ÙÈÌ‹ ÙˆÓ 10,6 ÓÔÛ‹ÛÂˆÓ /‚Ú¤ÊÔ˜/¤ÙÔ˜, ÛÙ· ‚Ú¤ÊË ËÏÈΛ·˜ 7 ¤ˆ˜ 9 ÌËÓÒÓ Î·È ÛÙ·ıÂÚÔÔÈ‹ıËΠÛÙ· ÌÂÁ·Ï‡ÙÂÚ· ‚Ú¤ÊË. ªÂ ÙË ¯Ú‹ÛË ÔÏ˘·Ú·ÁÔÓÙÈ΋˜ ÂÍÔÌ¿Ï˘ÓÛ˘ ÁÈ· ‰ËÌÔÁÚ·ÊÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜, ÌËÙÚÈÎfi ıËÏ·ÛÌfi, Ì‹Ó· ÓfiÛËÛ˘, ·ÚÈıÌfi ·‰ÂÏÊÒÓ, Î·È ·Ú·ÎÔÏÔ‡ıËÛË ·È‰ÈÎÔ‡ ÛÙ·ıÌÔ‡, ‰È·ÈÛÙÒıËΠ̛· ·‡ÍËÛË ÛÙË Û˘¯ÓfiÙËÙ· fiÏˆÓ ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ Û ‚Ú¤ÊË ¿Óˆ ÙˆÓ 7 ÌËÓÒÓ Ô˘ ›¯·Ó ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ¿ÛıÌ·ÙÔ˜ (Odds Ratio (OR)= 1,24, Confidence Interval, (CI) 1,09 ¤ˆ˜ 1,41), ‹ ÈÛÙÔÚÈÎfi ·ÙÔ›·˜ ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘˜ (OR= 1,14, CI 1,03 ¤ˆ˜ 1,26). ∏ Û˘¯ÓfiÙËÙ· ÙˆÓ ÏÔÈÌÒÍÂˆÓ ÙÔ˘ ηÙÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Ô˘ Û˘Óԉ¢fiÙ·Ó ·fi Û˘ÚÚ›ÙÔ˘ÛÛ· ·Ó·ÓÔ‹ Û¯ÂÙÈ˙fiÙ·Ó ÌfiÓÔÓ Ì ÙËÓ ·ÚÔ˘Û›· ¿ÛıÌ·ÙÔ˜ ÛÙÔ˘˜ ÁÔÓ›˜ (OR= 2,06, CI = 1,36 ¤ˆ˜ 3,11).

™˘ÌÂÚ¿ÛÌ·Ù·: ŸÏ˜ ÔÈ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡, Ô˘ ÛÙËÓ ÏÂÈÔÓfiÙËÙ¿ ÙÔ˘˜ Â›Ó·È ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ, Â›Ó·È ·˘ÍË̤Ó˜ Û ˘ÁÈ‹ ηٿ Ù· ¿ÏÏ· ‚Ú¤ÊË, Ì ÈÛÙÔÚÈÎfi ¿ÛıÌ·ÙÔ˜ ‹ ·ÙÔ›·˜ ÙˆÓ ÁÔÓ¤ˆÓ ÙÔ˘˜, ÂÓÒ Ë Û˘ÚÚ›ÙÔ˘Û· ·Ó·ÓÔ‹ (wheezing) Û¯ÂÙ›˙ÂÙ·È ÌfiÓÔ Ì ÙËÓ ·ÚÔ˘Û›· ¿ÛıÌ·ÙÔ˜ ÛÙÔ˘˜ ÁÔÓ›˜.

1Bosken CH, Hunt WC, Lambert WE, Samet JM A parental history of asthma is a risk factor for wheezing and nonwheezing respiratory illnesses in infants younger than 18 months of age Am J Respir Crit Care Med 2000; 161:1810-1815

£ÂÔÊ¿Ó˘ ∆ÛÈÏÈÁÈ¿ÓÓ˘ ¢È¢ı˘ÓÙ‹˜ ¶·È‰È·ÙÚÈÎÔ‡ ∆Ì‹Ì·ÙÔ˜ ¡·˘ÙÈÎÔ‡ ¡ÔÛÔÎÔÌ›Ԣ ∞ıËÓÒÓ

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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

Paediatriki 2001;64:72-75

CASE REPORT

"∫ÚÂÌ¿ÌÂÓÔ˜" ÛÏ‹Ó·˜ Û ÎÔÚ›ÙÛÈ 7 ÂÙÒÓ ∫. ÷˚‰ÔÔ‡ÏÔ˘1, ª. ™Ù¿ÌÔ˘-∆ÛÈ·Ú¿˙Ë2, º. ∞ı·Ó·ÛÈ¿‰Ô˘2, ∫. ∫Ô‡ÛÎÔ˘Ú·˜3, ∞. ∞ÓÙˆÓÈ¿‰Ë˜4

"Wandering" spleen in a 7 year old girl K. Haidopoulou1, M. Stamou-Tsiaprazi2, F. Athanassiadou2, K. Kouskouras3, A. Antoniadis4

¶ÂÚ›ÏË„Ë: √ fiÚÔ˜ "ÎÚÂÌ¿ÌÂÓÔ˜" ÂÚÈÁÚ¿ÊÂÈ ÌÈ· Ì¿ÏÏÔÓ Û¿ÓÈ· ÛÙ· ·È‰È¿ ÂÎÙÔ›· ÙÔ˘ ÛÏ‹Ó·, Ë ÔÔ›· ÔÊ›ÏÂÙ·È ÛÙËÓ ·Ô˘Û›· ‹ ÙË ¯·Ï¿ÚˆÛË ÙˆÓ Û˘Ó‰¤ÛÌˆÓ Ô˘ Ê˘ÛÈÔÏÔÁÈο ÙÔÓ ‰È·ÙËÚÔ‡Ó Î·ıËψ̤ÓÔ ÛÙË ı¤ÛË ÙÔ˘. ∏ ‰È¿ÁÓˆÛË Ù›ıÂÙ·È ‰‡ÛÎÔÏ· ÎÏÈÓÈο. ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ÂÓfi˜ ÎÔÚÈÙÛÈÔ‡ ËÏÈΛ·˜ 7 ÂÙÒÓ Ì „ËÏ·ÊËÙ‹ Ì¿˙· ÛÙËÓ ÎÔÈÏÈ¿. ªÂ ÙÔÓ ·ÂÈÎÔÓÈÛÙÈÎfi ¤ÏÂÁ¯Ô ‰È·ÈÛÙÒıËΠfiÙÈ ÂÚfiÎÂÈÙÔ ÁÈ· ÎÚÂÌ¿ÌÂÓÔ ÛÏ‹Ó·. ∏ ·ÛıÂÓ‹˜ ˘Ô‚Ï‹ıËΠ۠Ϸ·ÚÔÙÔÌ›· Î·È Ô ÛÏ‹Ó·˜ ηıËÏÒıËΠÛÙËÓ ·ÚÈÛÙÂÚ‹ ˘ԉȷÊÚ·ÁÌ·ÙÈ΋ ÂÚÈÔ¯‹ Ì ÂÈÙ˘¯›·.

Abstract: The wandering spleen is a rare condition characterized by splenic malposition due to congenital maldevelopment or laxity of the supporting ligamentous structures that usually fixate the spleen in its anatomical position. The diagnosis is often difficult to establish, clinically. We report the case of a 7 years old girl with an abdominal mass that proved to be a wandering spleen. The patient underwent laparotomy and the spleen was successfully repositioned in the left phrenorenal angle.

§¤ÍÂȘ ÎÏÂȉȿ: ÛÏ‹Ó·˜, ÎÚÂÌ¿ÌÂÓÔ˜ ÛÏ‹Ó·˜.

Key words: spleen, wandering spleen.

ªÂ ÙÔÓ fiÚÔ «ÎÚÂÌ¿ÌÂÓÔ˜» (wandering, ectopic, floating, aberrant spleen, lien mobile, splenoptosis) ÛÏ‹Ó·˜, ÂÚÈÁÚ¿ÊÂÙ·È Ë ÌÂÙ·Ó¿ÛÙ¢ÛË ÙÔ˘ ÛÏ‹Ó· ·fi ÙËÓ ·Ó·ÙÔÌÈ΋ ı¤ÛË ÙÔ˘, ÏfiÁˆ ‰È·Ì·ÚÙ›·˜ ηٿ ÙË ‰È¿Ï·ÛË ‹ ¯·Ï¿ÚˆÛ˘ ÙˆÓ Û˘Ó‰¤ÛÌˆÓ Ô˘ Ê˘ÛÈÔÏÔÁÈο ÙÔÓ Î·ıËÏÒÓÔ˘Ó ÛÙÔ ·ÚÈÛÙÂÚfi ¿Óˆ ÙÂÙ·ÚÙËÌfiÚÈÔ Ù˘ ÎÔÈÏ›·˜. √ ÎÚÂÌ¿ÌÂÓÔ˜ ÛÏ‹Ó·˜ Â›Ó·È Û˘Ó‹ıˆ˜ ‰ÈÔÁΈ̤ÓÔ˜ Î·È ¤¯ÂÈ Ì·ÎÚ‡ ·ÁÁÂÈ·Îfi ̛ۯÔ, Ì ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ Û˘ÛÙÚÔÊ‹˜ ‹ ڋ͢, Û ‹Ș ηÎÒÛÂȘ Ù˘ ÂÚÈÔ¯‹˜. ªÂ ÙËÓ Â˘Î·ÈÚ›· ÓÔÛËÏ›·˜ ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜

ÎÔÚÈÙÛÈÔ‡ 7 ÂÙÒÓ Ì ÎÔÈÏȷο ¿ÏÁË Î·È „ËÏ·ÊËÙ‹ Ì¿˙· ÛÙÔÓ ·ÚÈÛÙÂÚfi Ï·ÁfiÓÈÔ ‚fiıÚÔ, ÂÚÈÁÚ¿ÊÔ˘Ì ÙË Û¿ÓÈ· ·˘Ù‹ ÂÚ›ÙˆÛË ÎÚÂÌ¿ÌÂÓÔ˘ ÛÏ‹Ó· Î·È Á›ÓÂÙ·È Â˘Ú›· Û˘˙‹ÙËÛË Ì ‚¿ÛË Ù· Û‡Á¯ÚÔÓ· ‚È‚ÏÈÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ.

1 ¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£., ¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞, £ÂÛÛ·ÏÔÓ›ÎË 2 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£., ¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞, £ÂÛÛ·ÏÔÓ›ÎË 3 ∞ÎÙÈÓÔÏÔÁÈÎfi ∂ÚÁ·ÛÙ‹ÚÈÔ ∞.¶.£., ¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞, £ÂÛÛ·ÏÔÓ›ÎË 4 ÃÂÈÚÔ˘ÚÁÈ΋ ∫ÏÈÓÈ΋ ªÂÙ·ÌÔۯ‡ÛÂˆÓ ∞.¶.£., πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔÓ›ÎË

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¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ∫ÔÚ›ÙÛÈ ËÏÈΛ·˜ 7 ÂÙÒÓ ÂÈÛ‹¯ıË ÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ÁÈ· ÂÚ·ÈÙ¤Úˆ ¤ÏÂÁ¯Ô Î·È ·ÓÙÈÌÂÙÒÈÛË „ËÏ·ÊËÙ‹˜ ·ÓÒ‰˘Ó˘ Ì¿˙·˜ ÛÙÔÓ ·ÚÈÛÙÂÚfi Ï·ÁfiÓÈÔ ‚fiıÚÔ. ∆Ô ·Ú·¿Óˆ ‡ÚËÌ· ‹Ù·Ó Ù˘¯·›Ô ηٿ ÙËÓ „ËÏ¿ÊËÛË Ù˘ ÎÔÈÏ›·˜ Û ÂͤٷÛË ÚÔ˘Ù›Ó·˜. 1 4th Pediatric Clinic, AHEPA Hospital, Thessaloniki 2 2nd Pediatric Clinic, AHEPA Hospital, Thessaloniki 3 Radiology Laboratory, AHEPA Hospital, Thessaloniki 4 Department of Surgery, Division of Transplantation, Ippokration Hospital, Aristotelion University of Thessaloniki


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∂ÈÎfiÓ· 1. ∏ ·Ó·ÙÔÌÈ΋ ı¤ÛË ÙÔ˘ ÛÏ‹Ó·, Ë ÔÔ›· ÙÒÚ· ηٷϷ̂¿ÓÂÙ·È ·fi ÙÔÓ ıfiÏÔ ÙÔ˘ ÛÙÔÌ¿¯Ô˘.

∂›Ó·È ÙÔ ÙÚ›ÙÔ ·È‰› Ê·ÈÓÔÙ˘Èο ˘ÁÈÒÓ ÁÔÓ¤ˆÓ Î·È ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ·Ó·ÌÓËÛÙÈÎfi Ù˘ ·ÛıÂÓÔ‡˜ Â›Ó·È ÂχıÂÚÔ. ∞fi ÙÔ ·ÙÔÌÈÎfi ·Ó·ÌÓËÛÙÈÎfi ÙÔ˘ ·È‰ÈÔ‡ ·Ó·Ê¤ÚÔÓÙ·È ÂÂÈÛfi‰È· ¤ÓÙÔÓÔ˘ ÎÔÈÏÈ·ÎÔ‡ ¿ÏÁÔ˘˜ ÌÂÙ¿ ·fi ÎfiˆÛË. ∏ ·ÓÙÈÎÂÈÌÂÓÈ΋, ηٿ Ù· ÏÔÈ¿ Û˘ÛÙ‹Ì·Ù· ÂͤٷÛË, ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. √ ·ÈÌ·ÙÔÏÔÁÈÎfi˜ Î·È ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜, ÛÙÔÓ ÔÔ›Ô ˘Ô‚Ï‹ıËΠ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. ∏ ·ÂÈÎfiÓÈÛË Ù˘ ÎÔÈÏÈ¿˜, Ì ˘ÂÚ˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô, ¤ıÂÛ ÙË ‰È¿ÁÓˆÛË ¤ÎÙÔÔ˘ ‰ÈÔÁΈ̤ÓÔ˘ ÛÏ‹Ó·. √ ÂÚ·ÈÙ¤Úˆ ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì CT ÎÔÈÏ›·˜ Î·È ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÛÏ‹Ó· ÎÚ›ıËΠ·Ó·Áη›Ô˜, ÙfiÛÔ ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·ÁÁ›ˆÛ˘ ÙÔ˘ ÛÏ‹Ó·, fiÛÔ Î·È ÁÈ· ÙËÓ ÏËÚ¤ÛÙÂÚË ·ÚÂÁ¯˘Ì·ÙÈ΋ Î·È ÏÂÈÙÔ˘ÚÁÈ΋ ·ÂÈÎfiÓÈÛË ÙÔ˘ ÔÚÁ¿ÓÔ˘. ∞fi ÙËÓ CT ÎÔÈÏ›·˜ ‚Ú¤ıËΠÂÎÙÔ›· ÛÏ‹Ó· Ì ‰ÈfiÁΈÛË ·˘ÙÔ‡ Î·È Ê˘ÛÈÔÏÔÁÈ΋ ·ÂÈÎfiÓÈÛË ÙˆÓ ÛÏËÓÈÎÒÓ ·ÁÁ›ˆÓ, Ù· ÔÔ›· ÂÍÔÚÌÒÓÙ·È ·fi ÙËÓ ·Ó·ÙÔÌÈο ·Ó·ÌÂÓfiÌÂÓË ı¤ÛË Î·È ÔÚ‡ÔÓÙ·È Î·Ù·ÎfiÚ˘Ê· ÌÚÔÛÙ¿ ·fi ÙÔÓ ·ÚÈÛÙÂÚfi ÓÂÊÚfi Î·È ·Ú¿ÏÏËÏ· Ì ÙËÓ ·ÚÈÛÙÂÚ‹ ·Ú·ÎÔÏÈ΋ ·‡Ï·Î· (∂ÈÎfiÓ˜ 1-4). ∆Ô ÛÈÓıËÚÔÁÚ¿ÊËÌ· ¤‰ÂÈÍ fiÙÈ ÚfiÎÂÈÙ·È ÁÈ· ¤ÎÙÔÔ ÛÏ‹Ó·, ÌÂÁ¿ÏÔ˘ ÌÂÁ¤ıÔ˘˜ Ì ηٷÎÚ¿ÙËÛË ÙÔ˘ Ú·‰ÈÔÊ·ÚÌ¿ÎÔ˘ ÂÓÙfi˜ ·˘ÙÔ‡ (ıÂÚÌfi˜ ÛÏ‹Ó·˜) (∂ÈÎfiÓ· 5). ªÂ ‰Â‰Ô̤ÓË ÙË ‰È¿ÁÓˆÛË Ù˘ ÛÏËÓÈ΋˜ ÂÎÙÔ›·˜ ÌÂÏÂÙ‹Û·Ì ÙË ‚È‚ÏÈÔÁÚ·Ê›· Î·È ÏfiÁˆ ÎÈÓ‰‡ÓÔ˘ ڋ͢ ‹ Û˘ÛÙÚÔÊ‹˜ ÙÔ˘ ¤ÎÙÔÔ˘ ÛÏ‹Ó· Û˘ÛÙ‹ıËΠÛÙÔ˘˜ ÁÔÓ›˜ Ë ¿ÌÂÛË ¯ÂÈÚÔ˘ÚÁÈ΋ ÂÎÙ›ÌËÛË. ∞ÔÊ·Û›ÛıËΠ·ÓÔÈÎÙ‹ Ï··ÚÔÙÔÌ›· Î·È ·ÓÙÈÌÂÙÒÈÛË ‹ Ì ÛÏËÓÂÎÙÔÌ‹, ‹ ÂÊfiÛÔÓ ‹Ù·Ó ¯ÂÈÚÔ˘ÚÁÈο ÂÊÈÎÙfi, Ì Â·Ó·ÙÔÔı¤ÙËÛË ÙÔ˘ ÛÏ‹Ó· ÛÙËÓ ·Ó·ÙÔÌÈ΋ ÙÔ˘ ı¤ÛË. °È· ÙÔ ÂӉ¯fiÌÂÓÔ ÛÏËÓÂÎÙÔÌ‹˜ ¤ÁÈÓ ÂÌ‚ÔÏÈ·ÛÌfi˜ ¤Ó·ÓÙÈ ÙÔ˘ Ó¢ÌÔÓÈÔÎfiÎÎÔ˘, ÙÔ˘ ÌËÓÈÁÁÈÙȉÔÎfiÎÎÔ˘ Î·È ÙÔ˘ ·ÈÌfiÊÈÏÔ˘ Ù˘ ÈÓÊÏÔ˘¤ÓÙ˙·˜. ∆Ô ·È‰› ˘Ô‚Ï‹ıËΠ۠·ÓÔÈÎÙ‹ Ï··ÚÔÙÔ-

Paediatriki 2001;64:72-75

∂ÈÎfiÓ· 2. ∂›Â‰Ô ÂÌÊ¿ÓÈÛ˘ ÙÔ˘ ¿Óˆ fiÏÔ˘ ÙÔ˘ ÛÏ‹Ó· οو ·fi ÙÔ ÛËÌÂ›Ô ¤ÎÊ˘Û˘ Ù˘ ¿Óˆ ÌÂÛÂÓÙÂÚ›Ô˘ ·ÚÙËÚ›·˜ (ı12-√1).

Ì›·, fiÔ˘ ‰È·ÈÛÙÒıËΠfiÙÈ Ô ÛÏ‹Ó·˜ "ÎÚÂÌfiÙ·Ó" ·fi ·ÁÁÂÈ·Îfi ̛ۯÔ, ‹Ù·Ó ‰ÈÔÁΈ̤ÓÔ˜ Î·È ÂÓÙÔÈ˙fiÙ·Ó ÛÙÔÓ ·ÚÈÛÙÂÚfi Ï·ÁfiÓÈÔ ‚fiıÚÔ. ªÂÙ¿ ÙÔÓ ÂÍÂÏ΢ÛÌfi ÙÔ˘ ‰È·ÈÛÙÒıËΠfiÙÈ Ô Ì›Û¯Ô˜ ‹Ù·Ó ÂÚÈÛÙÚ·Ì̤ÓÔ˜ ηٿ ÙÔÓ ÂÈÌ‹ÎË ¿ÍÔÓ· ηٿ 180Ô. ªÂÙ¿ ÙËÓ ·Ó¿Ù·ÍË Ù˘ ÛÙÚÔÊ‹˜ ÙÔ˘ Ì›Û¯Ô˘ Ô ÛÏ‹Ó·˜ ·ÔÛ˘ÌÊÔÚ‹ıËÎÂ Î·È Ì›ÎÚ˘Ó ÛËÌ·ÓÙÈο. ™ÙË Û˘Ó¤¯ÂÈ· ÙÔÔıÂÙ‹ıËΠÛÙËÓ ·ÚÈÛÙÂÚ‹ ˘ԉȷÊÚ·ÁÌ·ÙÈ΋ ÂÚÈÔ¯‹ Î·È Î·ıËÏÒıËΠÂΛ Ì ·Ó·Û¯ËÌ·ÙÈÛÌfi ÙÔ˘ ÊÚÂÓÔÎÔÏÈÎÔ‡ Û˘Ó‰¤ÛÌÔ˘ ¿Óˆ ÛÙÔÓ ÔÔ›Ô ÂÈοıËÛÂ Ô Î¿Ùˆ fiÏÔ˜ ÙÔ˘ ÛÏ‹Ó·. ™˘˙‹ÙËÛË ∏ ÚÒÙË Â›ÛËÌË ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ÎÚÂÌ¿ÌÂÓÔ˘ ÛÏ‹Ó· ¤ÁÈÓ ÙÔ 1667 ·fi ÙÔÓ Van Horne Î·È ·ÊÔÚÔ‡ÛÂ Ù˘¯·›Ô ‡ÚËÌ· ÓÂÎÚÔ„›·˜, ·Ó Î·È ÔÈ ·ÓˆÌ·Ï›Â˜ Ù˘ ı¤Û˘ ÙÔ˘ ÛÏ‹Ó· ‹Ù·Ó ÁÓˆÛÙ¤˜ ·ÎfiÌ· ·fi ÙÔ˘˜ πÔÎÚ¿ÙË Î·È °·ÏËÓfi(1). ∏ ÚÒÙË ÛÏËÓÂÎÙÔÌ‹ Û ·ÛıÂÓ‹ Ì ÎÚÂÌ¿ÌÂÓÔ ÛÏ‹Ó· Ú·ÁÌ·ÙÔÔÈ‹ıËΠ̠ÂÈÙ˘¯›· ÙÔ 1878 ·fi ÙÔÓ Martin(2). ¶ÚfiÎÂÈÙ·È ÁÈ· Û¿ÓÈ· ηٿÛÙ·ÛË, ÌÂ Û˘¯ÓfiÙËÙ· Ô˘ ‰‡ÛÎÔÏ· ˘ÔÏÔÁ›˙ÂÙ·È Ì ·ÎÚ›‚ÂÈ·. ∏ ‰È¿ÁÓˆÛË Û˘¯ÓfiÙÂÚ· Ù›ıÂÙ·È ÌÂÙ¿ ÙËÓ ÂÌÊ¿ÓÈÛË ÂÈÏÔÎÒÓ, fiˆ˜ Ë Û˘ÛÙÚÔÊ‹, Ù˘ ÔÔ›·˜ Ë Û˘¯ÓfiÙËÙ· ˘ÔÏÔÁ›˙ÂÙ·È ÌÈÎÚfiÙÂÚË ÙÔ˘ 0,2%(3). ∆Ô 1989, Û ·Ó·ÛÎfiËÛË Ù˘ ̤¯ÚÈ ÙfiÙ µÚÂÙ·ÓÈ΋˜ ‚È‚ÏÈÔÁÚ·Ê›·˜ ·fi ÙÔÓ Allen, ‚Ú¤ıËÎ·Ó Û˘ÓÔÏÈο 35 ÂÚÈÙÒÛÂȘ ÎÚÂÌ¿ÌÂÓÔ˘ ÛÏ‹Ó· Û ·È‰È¿ ËÏÈΛ·˜ οو ÙˆÓ 10 ÂÙÒÓ(4), ÂÓÒ Û ÛÂÈÚ¿ 1413 ·ÛıÂÓÒÓ Ô˘ ˘Ô‚Ï‹ıËÎ·Ó Û ÛÏËÓÂÎÙÔÌ‹, ÔÈ ·ÛıÂÓ›˜ Ì ÎÚÂÌ¿ÌÂÓÔ ÛÏ‹Ó· ‹Ù·Ó ÌfiÓÔ 4(5). √È ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÈÙÒÛÂȘ Â›Ó·È ÂÚ›Ô˘ 400(6). ∆Ô Â‡ÚÔ˜ Ù˘ ËÏÈΛ·˜ ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ÂÚÈÁÚ¿ÊËÎ·Ó Â›Ó·È ·fi 3 Ì‹Ó˜ ¤ˆ˜ 82 ¯ÚfiÓÈ·. √È ÌÈÛ¤˜ ·fi ÙȘ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÈÙÒÛÂȘ ·ÊÔ-

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∂ÈÎfiÓ· 3. ™ÏËÓÔÌÂÁ·Ï›· Ì ÛÙÚÔÊ‹ ÙÔ˘ ÛÏ‹Ó· (∏ ‡ÏË ‚ϤÂÈ ÛÙÔÓ ÔÈÛıÔÂÚÈÙÔÓ·˚Îfi ¯ÒÚÔ).

ÚÔ‡Ó ·È‰È¿(7), ÂÓÒ ˘¿Ú¯ÂÈ ÈÛfiÙÈÌË Î·Ù·ÓÔÌ‹ ÛÙ· ‰‡Ô ʇϷ (6,7). ∞fi ÙËÓ ÚÔÛÈÙ‹ ÂÏÏËÓÈ΋ ‚È‚ÏÈÔÁÚ·Ê›·, ·Ó·ÊÔÚÈο Ì ·È‰È¿, ÂÚÈÁÚ¿ÊÔÓÙ·È ‰‡Ô ÂÚÈÙÒÛÂȘ ¤ÎÙÔÔ˘ ÛÏ‹Ó· (8). ∏ ·ÈÙÈÔÏÔÁ›· ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ·ÔÛ·ÊËÓÈṲ̂ÓË. √È ÂÚÈÛÛfiÙÂÚÔÈ Û˘ÁÁÚ·Ê›˜ ıˆÚÔ‡Ó ˆ˜ ÚfiÎÂÈÙ·È ÁÈ· Û˘ÁÁÂÓ‹ ‰È·Ì·ÚÙ›· ÂÚ› ÙË ‰È¿Ï·ÛË ÙˆÓ Î˘ÚÈfiÙÂÚˆÓ Û˘Ó‰ÂÛÌÈÎÒÓ ÛÙÔȯ›ˆÓ, Ô˘ Ê˘ÛÈÔÏÔÁÈο ηıËÏÒÓÔ˘Ó ÙÔÓ ÛÏ‹Ó· ÛÙÔ ¿Óˆ ·ÚÈÛÙÂÚfi ÎÔÈÏÈ·Îfi ÙÂÙ·ÚÙËÌfiÚÈÔ Î·È Ù· ÔÔ›· ·Ó·Ù‡ÛÛÔÓÙ·È Î·Ù¿ ÙÔÓ 2Ô Ì‹Ó· Ù˘ ·ËÛ˘ (6,7,911). √È Û‡Ó‰ÂÛÌÔÈ ·˘ÙÔ› Â›Ó·È Ô Á·ÛÙÚÔÛÏËÓÈÎfi˜, Ô˘ ÂÓÒÓÂÈ ÙÔ Ì›˙ÔÓ ÙfiÍÔ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Ì ÙËÓ ‡ÏË ÙÔ˘ ÛÏ‹Ó· Î·È Ô ÛÏËÓÔÓÂÊÚÈÎfi˜, Ô˘ ÂÚȤ¯ÂÈ Ù· ·ÁÁ›· Î·È Ó‡ڷ Ù˘ ‡Ï˘ Î·È Û˘ÁÎÚ·Ù› ÙÔÓ ÛÏ‹Ó· ÛÙÔ Ô›ÛıÈÔ ÂÚÈÙÔÓ·˚Îfi ÙÔ›¯ˆÌ· (7,9). ŸÙ·Ó ÙÔ Ú·¯È·›Ô ÙÌ‹Ì· ÙÔ˘ Á·ÛÙÚÈÎÔ‡ ÌÂÛÂÓÙÂÚ›Ô˘ ·ÔÙ‡¯ÂÈ Ó· ÂÓˆı› Ì ÙÔ Ú·¯È·›Ô ÙÌ‹Ì· ÙÔ˘ ÂÚÈÙÔÓ·›Ô˘ ηٿ ÙË ‰È¿Ï·ÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘, Û¯ËÌ·Ù›˙ÂÙ·È Â›ÌËΘ ÛÏËÓÈÎfi ÌÂÛÂÓÙ¤ÚÈÔ, Ô ÛÏ‹Ó·˜ Â›Ó·È Â˘Î›ÓËÙÔ˜ Î·È Ë fiÏË Î·Ù¿ÛÙ·ÛË Úԉȷı¤ÙÂÈ Û ÔÍ›·, ˘ÔÍ›· ‹ ¯ÚfiÓÈ· Û˘ÛÙÚÔÊ‹ ηıÒ˜ Î·È Û ÙÚ·˘Ì·ÙÈÛÌÔ‡˜ ·Ó Ô ÛÏ‹Ó·˜ ÌÂÙ·ÙÔÈÛı› ·fi ÙË ı¤ÛË ÙÔ˘ (7,9). ∂Ó›ÔÙ ·Ô˘ÛÈ¿˙ÂÈ ·ÓÙÂÏÒ˜ οı ÛÙËÚÈÎÙÈÎfi Û˘Ó‰ÂÛÌÈÎfi ÛÙÔÈ¯Â›Ô (9,11,12). Œ¯Ô˘Ó Â›Û˘ ÂÚÈÁÚ·Ê› ÙÔ˘Ï¿¯ÈÛÙÔÓ ‰‡Ô ÂÚÈÙÒÛÂȘ ÂÓ‰ÔıˆÚ·ÎÈ΋˜ ÂÓÙfiÈÛ˘ ÎÚÂÌ¿ÌÂÓÔ˘ ÛÏ‹Ó· ÛÂ Û˘Ó‰˘·ÛÌfi Ì ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ·ÏÏ¿ ·ÔÙÂÏ› ‰È·ÊÔÚÂÙÈ΋ ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ· (6). ÕÏÏÔÈ Û˘ÁÁÚ·Ê›˜, ÂÓÙÔ›˙Ô˘Ó ·˘ÍË̤ÓË Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ÎÚÂÌ¿ÌÂÓÔ˘ ÛÏ‹Ó· Û Á˘Ó·›Î˜ ·Ó··Ú·ÁˆÁÈ΋˜ ËÏÈΛ·˜ Î·È ÙÔ ·Ô‰›‰Ô˘Ó ÛÙË ¯·Ï¿ÚˆÛË ÙˆÓ ÛÏËÓÈÎÒÓ Û˘Ó‰¤ÛÌˆÓ Î·Ù¿ Î·È ÌÂÙ¿ ÙËÓ ÂÁ΢ÌÔÛ‡ÓË (6,10). ÕÏÏÔÈ Â›ÎÙËÙÔÈ ÚԉȷıÂÙÈÎÔ› ·Ú¿ÁÔÓÙ˜, Ô˘ ·Ó·Ê¤ÚÔÓÙ·È Â›Ó·È ÔÈ ÙÚ·˘Ì·ÙÈÛÌÔ›, ÔÈ ¯ÂÈÚÔ˘ÚÁÈΤ˜ ÂÂÌ‚¿ÛÂȘ Î·È Î·Ù·ÛÙ¿ÛÂȘ Ô˘ Û˘Óԉ‡ÔÓÙ·È ·fi ÛÏËÓÔÌÂÁ·-

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∂ÈÎfiÓ· 4. ∫¿Ùˆ fiÏÔ˜ ÛÏ‹Ó·, ›ÛÔ‰Ô˜ ÂÏ¿ÛÛÔÓÔ˜ ˘¤ÏÔ˘ (π1-π2).

Ï›·, fiˆ˜ Ë ÂÏÔÓÔÛ›·, ÔÈ ÛÏËÓÈΤ˜ ·ÛÙÂȘ, Ù· ·ÔÛÙ‹Ì·Ù·, ÚˆÙÔ·ı›˜ ‹ ÌÂÙ·ÛÙ·ÙÈÎÔ› fiÁÎÔÈ, ·ÈÌ·ÁÁÂÈÒÌ·Ù·, Ë ÓfiÛÔ˜ ÙÔ˘ Hodgkin, ·ÈÌÔÏ˘ÙÈΤ˜ ·Ó·È̛˜ ÎÏ. (6,9,11). øÛÙfiÛÔ ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÂÚÈÙÒÛÂȘ Ô ÛÏ‹Ó·˜ ·˘Ùfi˜ ηı·˘Ùfi˜ Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi˜ (6). √È ·ÛıÂÓ›˜ ÌÔÚ› Ó· Â›Ó·È ·Û˘Ìو̷ÙÈÎÔ› ‹ Û˘ÓËı¤ÛÙÂÚ· Ó· ·Ú·ÔÓÔ‡ÓÙ·È ÁÈ· ¯ÚfiÓÈ· ÎÔÈÏȷο ¿ÏÁË, ÌÂÙÚ›·˜ ÂÓÙ¿Ûˆ˜, ÏfiÁˆ ‰È·Ï›Ô˘Û·˜ Û˘ÛÙÚÔÊ‹˜ (6,9) ‹ ÌÂÙ·Ó¿ÛÙ¢Û˘ ÙÔ˘ ÛÏ‹Ó· (10). ÕÏÏÔÙ ¿ÏÈ, Ô ÛÏ‹Ó·˜ „ËÏ·Ê¿Ù·È ÂÎÙfi˜ Ù˘ ·Ó·ÙÔÌÈ΋˜ ÙÔ˘ ı¤Û˘ Î·È ‰›ÓÂÈ ÙËÓ ÂÓÙ‡ˆÛË ÂÓ‰ÔÎÔÈÏȷ΋˜ Ì¿˙·˜ (6,7,9). ∆¤ÏÔ˜, Û ·ÚÎÂÙÔ‡˜ ·ÛıÂÓ›˜ Ë ‰È¿ÁÓˆÛË Ù›ıÂÙ·È ÌfiÓÔ ·ÊÔ‡ ÛËÌÂȈıÔ‡Ó ÂÈÏÔΤ˜ (.¯. Û˘ÛÙÚÔÊ‹ ‹ Ú‹ÍË) Î·È ÙfiÙ ٷ Û˘ÌÙÒÌ·Ù· Û˘Ó‹ıˆ˜ Â›Ó·È Ô͇ ÎÔÈÏÈ·Îfi ¿ÏÁÔ˜, Ì ‹ ¯ˆÚ›˜ shock (6,7,11,12), ηٿÛÙ·ÛË Ô˘ Ô‰ËÁ› Û Â›ÁÔ˘Û· ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÓÙÈÌÂÙÒÈÛË. ∏ ·ÚÔ˘Û›· ÂÈÌË΢Ṳ̂ÓÔ˘ ·ÁÁÂÈ·ÎÔ‡ Ì›Û¯Ô˘ ‰È¢ÎÔχÓÂÈ ÙËÓ ÂÌÊ¿ÓÈÛË Û˘ÛÙÚÔÊ‹˜, Ë ÔÔ›· ÌÔÚ› Ó· Â›Ó·È ÔÍ›· , ‰È·Ï›Ô˘Û· ‹ ¯ÚfiÓÈ·. ∏ Û˘ÛÙÚÔÊ‹ ÂÌÔ‰›˙ÂÈ ·Ú¯Èο ÙË ÊÏ‚È΋ ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜ ‰ËÌÈÔ˘ÚÁÒÓÙ·˜ Û˘ÌÊfiÚËÛË, ÂÓÒ Ë ·ÚÙËÚȷ΋ ·ÈÌ¿ÙˆÛË ÙÔ˘ ÛÏ‹Ó· ÂËÚ¿˙ÂÙ·È ·ÚÁfiÙÂÚ· (ÈÛ¯·ÈÌ›·). √ ‚·ıÌfi˜ Ù˘ Û˘ÛÙÚÔÊ‹˜ ÔÈΛÏÂÈ ÌÂٷ͇ 90Ô, 360Ô ‹ Î·È ÔÏÏ·Ï¿ÛÈÔ˘ ·˘ÙÒÓ. √ fiÓÔ˜ ÔÊ›ÏÂÙ·È ÛÙË ‰È¿Ù·ÛË Ù˘ ο„·˜ ÙÔ˘ ÛÏ‹Ó· Î·È ÙËÓ ·‡ÍËÛË ÙˆÓ ‰È·ÛÙ¿ÛÂÒÓ ÙÔ˘, ·ÏÏ¿ Î·È Û ÙÔÈ΋ ·ÓÙ›‰Ú·ÛË ÙÔ˘ ÂÚÈÙÔÓ·›Ô˘ (6). ∞Ó Ë ‰È¿ÁÓˆÛË – ·ÓÙÈÌÂÙÒÈÛË Î·ı˘ÛÙÂÚ‹ÛÂÈ, ÌÔÚ› Ó· ·Ú·ÙËÚËıÔ‡Ó Á¿ÁÁÚ·ÈÓ· ‹ ·ÔÛÙ‹Ì·Ù· ÙÔ˘ ÛÏ‹Ó· ηıÒ˜ Î·È ·ÈÌÔÚÚ·Á›· ·fi ÎÈÚÛÔ‡˜ ÔÈÛÔÊ¿ÁÔ˘. ™·ÓÈfiٷٷ ÂÚÈÁÚ¿ÊÔÓÙ·È Û˘ÛÙÚÔÊ‹ (ÔÏÈ΋ ‹ ÌÂÚÈ΋) ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Î·È Û˘ÛÙÚÔÊ‹ Ù˘ Ô˘Ú¿˜ ÙÔ˘ ·ÁÎÚ¤·ÙÔ˜, ̤¯ÚÈ Î·È ·ÁÎÚ·ÙÈ΋ Ó¤ÎÚˆÛË (6,13). ∆ÔÓ Î‡ÚÈÔ ÏfiÁÔ ÛÙË ‰È¿ÁÓˆÛË ¤¯Ô˘Ó ÔÈ ·ÂÈÎÔ-


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ÛÙÈΤ˜ ÌÂıfi‰Ô˘˜. ∏ ·Ô˘Û›· ÂÈÏÔÎÒÓ ·fi ÙÔ ÛÏËÓÈÎfi ·Ú¤Á¯˘Ì· Î·È Ë Ê˘ÛÈÔÏÔÁÈ΋ ÙÔ˘ ·ÈÌ¿ÙˆÛË ‹Ù·Ó ‰‡Ô ÛÙÔȯ›· Ô˘ Û˘ÓÂÎÙÈÌ‹ıËηÓ, ÒÛÙ ӷ Á›ÓÂÈ ÚÔÛ¿ıÂÈ· ‰È·Ù‹ÚËÛ˘ ÙÔ˘ ÔÚÁ¿ÓÔ˘ Î·È ·ÔηٿÛÙ·Û‹˜ ÙÔ˘ ÛÙË Ê˘ÛÈÔÏÔÁÈ΋ ·Ó·ÙÔÌÈ΋ ÙÔ˘ ı¤ÛË. ™‹ÌÂÚ·, ¤Ó· ¯ÚfiÓÔ ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË, ÙÔ ·È‰› ‰ÂÓ ·ÚÔ˘ÛÈ¿˙ÂÈ ÎÔÈÏȷο ¿ÏÁË Î·È Ô ˘ÂÚ˯ÔÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ÛÏ‹Ó· Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi˜. µÈ‚ÏÈÔÁÚ·Ê›·

∂ÈÎfiÓ· 5. ŒÎÙÔÔ˜ ‰ÈÔÁΈ̤ÓÔ˜ ÛÏ‹Ó·˜ Ì ηٷÎÚ¿ÙËÛË ÙÔ˘ Ú·‰ÈÔÊ·ÚÌ¿ÎÔ˘ ÂÓÙfi˜ ·˘ÙÔ‡. (£ÂÚÌfi˜ ÛÏ‹Ó·˜).

ÓÈÛÙÈΤ˜ ̤ıÔ‰ÔÈ. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÎÔÈÏ›·˜ ·ÔÙÂÏ› ÙË Ì¤ıÔ‰Ô ÂÎÏÔÁ‹˜. ÷ڷÎÙËÚÈÛÙÈÎfi ‡ÚËÌ· Â›Ó·È Ë ·Ô˘Û›· ÙÔ˘ ÛÏ‹Ó· ·fi ÙËÓ «ÎÔÈÙ›‰·» ÙÔ˘ ÛÙÔ ·ÚÈÛÙÂÚfi ˘Ô¯fiÓ‰ÚÈÔ Î·È Ë ·Ó‡ÚÂÛË «Ì¿˙·˜» ÔÔ˘‰‹ÔÙ ÂÓ‰ÔÎÔÈÏȷο, Ì ۷ʤ˜ ÂÚ›ÁÚ·ÌÌ·, ο„· Î·È ˘ÂÚ˯ÔÁÂÓ‹ ‰ÔÌ‹ ‹·ÙÔ˜ÛÏ‹Ó·(6,7). ∞ÓÙ›ÛÙÔȯ·, ÛËÌ·ÓÙÈ΋ ‰È·ÁÓˆÛÙÈ΋ ‚Ô‹ıÂÈ· ÚÔÛʤÚÂÈ Î·È ÙÔ ¤Á¯ÚˆÌÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ·, Ì ÙÔ ÔÔ›Ô ÌÔÚ› Ó· ÂÎÙÈÌËı› Î·È Ë ·ÈÌ¿ÙˆÛË ÙÔ˘ ÛÏ‹Ó· Î·È Ó· ‰È·ÈÛÙˆı› Ë ·ÚÔ˘Û›· Û˘ÌÊfiÚËÛ˘ ‹ ÈÛ¯·ÈÌ›·˜ ÙÔ˘ ÛÏËÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜, ηıÒ˜ Î·È Ë ÔÚ›· ÙˆÓ ÛÏËÓÈÎÒÓ ·ÁÁ›ˆÓ ÙÔ˘ ¤ÎÙÔÔ˘ ÛÏ‹Ó· (6,11). ∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÂӉ›ÎÓ˘Ù·È Î˘Ú›ˆ˜ Û ÂÚÈÙÒÛÂȘ Û˘ÛÙÚÔÊ‹˜ ‹ ڋ͢, ÚÔÛʤÚÔÓÙ·˜ ÂÎÙfi˜ ·fi ÙË ‰È¿ÁÓˆÛË, ÂÈÚfiÛıÂÙ˜ ÏËÚÔÊÔڛ˜ ÁÈ· ÙËÓ ·ÈÌ¿ÙˆÛË Î·È ÙËÓ ˘Ê‹ ÙÔ˘ ÛÏËÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜ ÛÙÔÓ ¯ÂÈÚÔ˘ÚÁfi (6,7,9). ∏ ·ÓÙÈÌÂÙÒÈÛË ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ·ÚÔ˘Û›· ‹ fi¯È ÂÈÏÔÎÒÓ Î·È ÙË ‚ȈÛÈÌfiÙËÙ· ‹ ÌË ÙÔ˘ ÛÏËÓÈÎÔ‡ ·ÚÂÁ¯‡Ì·ÙÔ˜. ∆· ÂÚÈÛÛfiÙÂÚ· ÂÚˆÙËÌ·ÙÈο ÁÂÓÓ¿ Ë ·ÓÙÈÌÂÙÒÈÛË ·ÚÚÒÛÙˆÓ ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ηٿ Ù· ¿ÏÏ· ÛÏ‹Ó·, fiˆ˜ ÛÙË ‰È΋ Ì·˜ ÂÚ›ÙˆÛË, ηıÒ˜ - ÔÈ ·fi„ÂȘ ηٿ ηÈÚÔ‡˜ ·ÏÏ¿˙Ô˘Ó Ê¤ÚÔÓÙ·˜ ¿ÏÏÔÙ ˆ˜ ̤ıÔ‰Ô ÂÎÏÔÁ‹˜ ÙËÓ ÛÏËÓÂÎÙÔÌ‹ Î·È ¿ÏÏÔÙ ÙËÓ ÛÏËÓÔËÍ›·. ¶ÚÔÎÂÈ̤ÓÔ˘ ÁÈ· ·È‰È¿, fiÔ˘ Ô Î›Ó‰˘ÓÔ˜ ÂÌÊ¿ÓÈÛ˘ ÏÔÈÌÒÍÂˆÓ Î·È ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÌÂÙ¿ ÛÏËÓÂÎÙÔÌ‹ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚÔ˜, ηٷ‚¿ÏÏÂÙ·È ÚÔÛ¿ıÂÈ· ÁÈ· Ó· ‰È·ÙËÚËı› Ô ÛÏ‹Ó·˜. ™Â ÂÚÈÙÒÛÂȘ ÈÛ¯·ÈÌÈ΋˜ Ó¤ÎÚˆÛ˘ ‹ ˘ÔΛÌÂÓ˘ ÓÂÔÏ·ÛÌ·ÙÈ΋˜ ÓfiÛÔ˘ ˘¿Ú¯ÂÈ ·fiÏ˘ÙË ¤Ó‰ÂÈÍË ÛÏËÓÂÎÙÔÌ‹˜ (6). ™ÙË ‰È΋ Ì·˜ ÂÚ›ÙˆÛË, Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· ¤ıÂÛ ÙË ‰È¿ÁÓˆÛË, Ë ÔÔ›· ÂȂ‚·ÈÒıËΠ̠ÙȘ ·ÂÈÎÔÓÈ-

1. Robinson AP. Wandering spleen: case report and review. Mt Sin J Med 1988;55:428-434. 2. Martin A. A successful case of splenotomy. Br J Med 1978;61:191-192. 3. Fujiwara T, Takehara Y, Isoda H, Ichijo K, Tooyama N, Kodaira N et al. Torsion of a wandering spleen: CT and angiographic appearance. Comput Assist Tomogr 1995;19:84-86. 4. Allen KB, Gibbs A. Pediatric wandering spleen, the case of splenopexy. J Pediatr Surg 1989;17:373. 5. Eraklis AJ, Filler RM. Splenectomy in childhood, a review of 1413 cases. J Pediatr Surg 1972;7:382-388. 6. Peitgen K, Scweden K. Management of intermittent splenic torsion ("wandering spleen"): a review. Eur J Surg 1995;161:49-52. 7. Raissaki M, Prassopoulos P, Daskalogiannaki M, Magkanas E, Gourtsoyiannis N. Acute abdomen pain due to torsion of wandering spleen: CT diagnosis. Eur Radiol 1998;8:1409-1412. 8. ¶ÚˆÙÔÛ˘ÁÁÂÏ›‰Ô˘ ª, °·ÚÔ‡ÊË ∞, µÏfiÓÙ˙Ô˘ ∫, ∫·ÙÛÈοÚË ª, ª·ÚÔ‡¯·˜ °, ªÔ˘ÙÛÔ‡Ú˘ Ã. ¶Ï¿ÓËÙ·˜ ÛÏ‹Ó·˜ Û 2 ·È‰È¿. [¶ÂÚ›ÏË„Ë ∞Ú. 153∞∞]. 32Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ; 1994 17-19 πÔ˘Ó›Ô˘; ∫¤Ú΢ڷ; 1994. 9. Saadaoui H, Toppet V, Hubloux G, Horth M, Cadiere G, Spehl M. Acute torsion of a wandering spleen in a child: preoperative diagnosis by ultrasonography and computed tomography. Eur J Radiol 1998;26:205-209. 10. Possilico L, Shah A. A wandering spleen detection by In111 leucocyte imaging. Clin Nucl Med 1996;4:287-289. 11. Newman B, Bowen A, Eggli KD. Recognition of malposition of the liver and spleen: CT, MRI, nuclear scan and fluoroscopic imaging. Pediatr Radiol 1994;24:274-279. 12. Papakyriacou K, Nicolaou N, Symeonides P. Wandering spleen: a rare emergency condition. Br J Surg 1996;83:46-50. 13. Sheflin JR, Lee CM, Kretchmar KA. Torsion of wandering spleen and distal pancreas. Am J Roentgenol 1984; 142:100-101.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 10-04-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 06-12-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: º. ∞ı·Ó·ÛÈ¿‰Ô˘-¶ÈÂÚÔÔ‡ÏÔ˘ µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞, £ÂÛÛ·ÏÔÓ›ÎË

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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

Paediatriki 2001;64:76-80

CASE REPORT

∫ÏËÚÔÓÔÌÈ΋ ‰È·Ù·Ú·¯‹ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (ÁÈÁ·ÓÙÈ·›·) Ô˘ ÂΉËÏÒıËÎÂ Û·Ó “„¢‰ÔıÚÔÌ‚ÔÂÓ›·” ¶ÂÚÈÁÚ·Ê‹ ‰‡Ô ÂÚÈÙÒÛÂˆÓ ∂. ÷Ù˙Ë·ÓÙÂÏ‹˜, ª. ∞ı·Ó·Û›Ô˘-ªÂÙ·Í¿, Ã. ∆Û¿ÓÙ·ÏË, ∫. ∞‚Ú·Ì›‰Ô˘, ¢. ∑·ÌÔ‡ÏË*, ¡. °ÔÌ¿Î˘

Hereditary giant platelet disorder presenting as "pseudothrombocytopenia" A report of two cases E. Hatzipantelis, M. Athanassiou-Metaxa, H. Tsantali, K. Avramidou, D. Zambuli*, N. Gombakis

¶ÂÚ›ÏË„Ë: OÈ ÎÏËÚÔÓÔÌÈΤ˜ ‰È·Ù·Ú·¯¤˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Ô˘ ÂΉËÏÒÓÔÓÙ·È Ì ÙËÓ ÂÌÊ¿ÓÈÛË ÁÈÁ·ÓÙÈ·›ˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Î·È ıÚÔÌ‚ÔÂÓ›· ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ¿ÏÏÔÙ ˆ˜ ÔÛÔÙÈΤ˜ (·ÓˆÌ·Ï›· MayHegglin, Û‡Ó‰ÚÔÌ· Fechtner Î·È Sebastian) Î·È ¿ÏÏÔÙ ˆ˜ ÔÈÔÙÈΤ˜ (Û‡Ó‰ÚÔÌ· Bernard-Soulier, Epstein, Montreal, ÓfiÛÔ˜ Von Willebrand Ù‡Ô˘ ππb). ™Â ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ fï˜, Ë ·ÎÚÈ‚‹˜ ηٿٷÍË Ù˘ ÎÏËÚÔÓÔÌÈ΋˜ ·˘Ù‹˜ ‰È·Ù·Ú·¯‹˜ ‰ÂÓ Â›Ó·È ‰˘Ó·Ù‹ ÏfiÁˆ Ù˘ ·Ô˘Û›·˜ ÂȉÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ. ¶ÂÚÈÁÚ¿ÊÔÓÙ·È ‰‡Ô ÂÚÈÙÒÛÂȘ (ÌËÙ¤Ú· Î·È ÎfiÚË), ¯ˆÚ›˜ ÈÛÙÔÚÈÎfi ·ÈÌÔÚÚ·ÁÈ΋˜ ‰È¿ıÂÛ˘, Ì ‰È·Ù·Ú·¯‹ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (ÁÈÁ·ÓÙÈ·›·) Ô˘ ÂΉËÏÒıËÎÂ Û·Ó „¢‰‹˜ ıÚÔÌ‚ÔÂÓ›·. √ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ·È‰ÈÔ‡ Î·È Ù˘ ÌËÙ¤Ú·˜ ¤‰ÂÈÍ ıÚÔÌ‚ÔÂÓ›· (·ÚÈıÌfi˜ ·ÈÌÔÂÙ·Ï›ˆÓ 50-120 x 109/L) Û Â·ÓÂÈÏËÌ̤Ó˜ ÌÂÙÚ‹ÛÂȘ ÛÙÔÓ ·ÈÌ·ÙÔÏÔÁÈÎfi ·Ó·Ï˘Ù‹ Î·È ·˘ÍË̤ÓÔ Ì¤ÛÔ fiÁÎÔ ·ÈÌÔÂÙ·Ï›ˆÓ (12-16 fl). ∞fi ÙË ÌÈÎÚÔÛÎÔÈ΋ ÂͤٷÛË ÙÔ˘ ·ÚÈıÌÔ‡ Î·È Ù˘ ÌÔÚÊÔÏÔÁ›·˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Û Â›¯ÚÈÛÌ· ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ‰È·ÈÛÙÒıËÎÂ Ê˘ÛÈÔÏÔÁÈÎfi˜ ·ÚÈıÌfi˜ ·ÈÌÔÂÙ·Ï›ˆÓ, ·ÚÔ˘Û›· ÌÈÎÚÒÓ ·ÈÌÔÂÙ·ÏÈ·ÎÒÓ ÛˆÚÒÓ Î·ıÒ˜ Î·È ÌÂÁ¿Ï·-ÁÈÁ·ÓÙÈ·›· ·ÈÌÔÂÙ¿ÏÈ·. ™ÙÔ Ì˘ÂÏfiÁÚ·ÌÌ· ÙÔ˘ ·È‰ÈÔ‡ ‚Ú¤ıËΠ·˘ÍË̤ÓÔ˜ ·ÚÈıÌfi˜ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ÌÔÚÊÔÏÔÁ›·. ∞ÓÙÈ·ÈÌÔÂÙ·Ïȷο ·ÓÙÈÛÒÌ·Ù· ·ÓȯÓ‡ıËÎ·Ó Ì ÙËÓ ¿ÌÂÛË Ì¤ıÔ‰Ô (ÔÏÈÎfi ·›Ì·) Î·È ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ. ª¤¯ÚÈ Û‹ÌÂÚ· ÌËÙ¤Ú· Î·È ÎfiÚË ‰ÂÓ ¤¯Ô˘Ó ÂÌÊ·Ó›ÛÂÈ Î·Ó¤Ó· ÎÏÈÓÈÎfi

Abstract: Hereditary giant platelet disorders are characterized either as quantitative (May-Hegglin anomaly, Fechtner and Sebastian platelet syndromes), or as qualitative (Bernard-Soulier, Epstein and Montreal syndromes, type IIb von Willebrand disease). However, a number of giant platelet disorders cannot be classified as yet, due to the absence of specific findings. We describe two cases (mother and daughter) with non specific hereditary giant platelet disorder, that presented as "pseudothrombocytopenia", in the absence of any history of bleeding diathesis. Platelet counts (Coulter),which were performed several times over an eight-month period were found to be decrea- sed ranging from 50 to 120 x 109/L,while the mean platelet volume (MPV) was always elevated (12-16 fl). Microscopic examination of platelet morpho- logy stained peripheral blood films revealed the presence of small platelet aggregates as well as giant thrombocytes while the platelet count was within normal limits. Examination of the child’s bone marrow aspirate revealed an increased number of megacaryocytes with normal morpho- logy. Platelet associated IgG antibodies (PAIgG) were increased in both patients. Up to the present time both mother and daughter have not developed any bleeding symptoms and have not required any specific treatment. It is postulated that the diagnosis is that of a non specific hereditary giant platelet disorder

∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞.¶.£., “πÔÎÚ¿ÙÂÈÔ” °.¶.¡.£. *∞ÈÌ·ÙÔÏÔÁÈÎfi ∂ÚÁ·ÛÙ‹ÚÈÔ, “£Â·Á¤ÓÂÈÔ” ∞ÓÙÈηÚÎÈÓÈÎfi ¡ÔÛÔÎÔÌ›Ô, £ÂÛÛ·ÏÔÓ›ÎË

1st Pediatric Clinic of the Aristotelion University of Thessaloniki, “Ippokration” Hospital *Hematology Laboratory, “Theagenio” ∞nticancer Hospital, Thessaloniki

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Paediatriki 2001;64:76-80

Û‡Ìو̷ Î·È ‰ÂÓ ¯ÚÂÈ¿ÛıËΠη̛· ıÂÚ·¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË. ¶Èı·ÓÔÏÔÁԇ̠fiÙÈ Ë ‰È¿ÁÓˆÛË Â›Ó·È ·˘Ù‹ Ù˘ ÎÏËÚÔÓÔÌÈ΋˜, ÌË ÂȉÈ΋˜ ‰È·Ù·Ú·¯‹˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ, Ì ÂÌÊ¿ÓÈÛË ÁÈÁ·ÓÙÈ·›ˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ, ‰È¿ÁÓˆÛË Ô˘ ÌÔÚ› Ó· ·Ó·ÙÚ·› ·Ó ÔÈ ÂÚÈÙÒÛÂȘ Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È Î·È ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È Û˘ÛÙËÌ·ÙÈο ÂÌÊ·Ó›ÛÔ˘Ó Î¿ÔÈ· ÎÏÈÓÈ΋ Û˘Ìو̷ÙÔÏÔÁ›· ÛÙÔ Ì¤ÏÏÔÓ.

However, this diagnosis may change, if the cases we describe develop any clinical symptoms on follow up.

§¤ÍÂȘ ÎÏÂȉȿ: ÁÈÁ·ÓÙÈ·›· ·ÈÌÔÂÙ¿ÏÈ·, ÎÏËÚÔÓÔÌÈ΋ ‰È·Ù·Ú·¯‹ ·ÈÌÔÂÙ·Ï›ˆÓ, „¢‰ÔıÚÔÌ‚ÔÂÓ›·.

Key words: giant platelets, hereditary platelet disorder, pseudothrombocytopenia.

∂ÈÛ·ÁˆÁ‹ OÈ ÎÏËÚÔÓÔÌÈΤ˜ ‰È·Ù·Ú·¯¤˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Ô˘ ÂΉËÏÒÓÔÓÙ·È Ì ÙËÓ ÂÌÊ¿ÓÈÛË ÁÈÁ·ÓÙÈ·›ˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ‰˘Ó·ÙfiÓ Ó· Â›Ó·È ÔÛÔÙÈΤ˜ Î·È ÔÈÔÙÈΤ˜. √È ÔÛÔÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, fiˆ˜ Ë ·ÓˆÌ·Ï›· May-Hegglin Î·È Ù· Û‡Ó‰ÚÔÌ· Fechtner Î·È Sebastian, ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ıÚÔÌ‚ÔÂÓ›· Î·È ÙËÓ ·ÚÔ˘Û›· Ï¢ÎÔ΢ÙÙ·ÚÈÎÒÓ ÂÁÎÏ›ÛÙˆÓ Î·È ÎÏËÚÔÓÔÌÔ‡ÓÙ·È Ì ÙÔÓ ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú·. ™ÙȘ ÔÈÔÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÛÙȘ Ôԛ˜ Ô ¯·Ú·ÎÙ‹Ú·˜ Ù˘ ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜ ÔÈΛÏÏÂÈ, ·Ó‹ÎÔ˘Ó Ù· Û‡Ó‰ÚÔÌ· Bernard-Soulier, Epstein, Montreal Î·È Ë ÓfiÛÔ˜ Von Willebrand Ù‡Ô˘ ππb. ™Â ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ Ë ·ÚÔ˘Û›· ÌÂÁ¿ÏˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Û˘Óԉ¢fiÌÂÓË ·fi ıÚÔÌ‚ÔÂÓ›· ¤¯ÂÈ Û˘Û¯ÂÙÈÛı› Ì ÙÔ Û‡Ó‰ÚÔÌÔ Alport (1-5). ¶·ÚfiÏ· ·˘Ù¿, Û ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ Ë ·ÎÚÈ‚‹˜ ηٿٷÍË Ù˘ ÎÏËÚÔÓÔÌÈ΋˜ ·˘Ù‹˜ ‰È·Ù·Ú·¯‹˜ ‰ÂÓ Â›Ó·È ‰˘Ó·Ù‹ ÏfiÁˆ Ù˘ ·Ô˘Û›·˜ ÂȉÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ. 梉‹˜ ıÚÔÌ‚ÔÂÓ›· ηٿ ÙË Ì¤ÙÚËÛË ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ·fi ÙÔÓ ·Ó·Ï˘Ù‹ ÌÔÚ› Ó· Û˘Ì‚Â› Û ηٷÛÙ¿ÛÂȘ Ô˘ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi Û˘ÛÛÒÚ¢ÛË ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ·Ú¿ ÙËÓ ·ÚÔ˘Û›· ·ÓÙÈËÎÙÈÎÔ‡ ‹ ·fi ÙËÓ ·ÚÔ˘Û›· ÌÂÁ¿ÏˆÓ-ÁÈÁ·ÓÙÈ·›ˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (6-9). ¶ÂÚÈÁÚ¿ÊÔÓÙ·È ‰‡Ô ÂÚÈÙÒÛÂȘ (ÌËÙ¤Ú· Î·È ÎfiÚË), ¯ˆÚ›˜ ÈÛÙÔÚÈÎfi ·ÈÌÔÚÚ·ÁÈ΋˜ ‰È¿ıÂÛ˘, Ì „¢‰‹ ıÚÔÌ‚ÔÂÓ›·, Ô˘ ·Ó·Î·Ï‡ÊıËΠÛÂ Ù˘¯·›Ô ¤ÏÂÁ¯Ô Î·È ‰È·Ù·Ú·¯‹ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (ÁÈÁ·ÓÙÈ·›·), ÏfiÁˆ Ù˘ Û·ÓÈfiÙËÙ¿˜ ÙÔ˘˜ Î·È ÙÔ˘ ÌÂÁ¿ÏÔ˘ ‰È·ÊÔÚԉȷÁÓˆÛÙÈÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜ Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó.

1510 gr Î·È ·Ú¤ÌÂÈÓ ÛÙË ªÔÓ¿‰· ∂ÓÙ·ÙÈ΋˜ ¡ÔÛËÏ›·˜ ¡ÂÔÁÓÒÓ (ª∂¡¡) ÁÈ· ¤ÍÈ Â‚‰ÔÌ¿‰Â˜ ÏfiÁˆ ÚÔ‚ÏËÌ¿ÙˆÓ Û›ÙÈÛ˘. ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘ ÛÙË ª∂¡¡ ‚Ú¤ıËΠ‹È· ıÚÔÌ‚ÔÂÓ›· (·ÈÌÔÂÙ¿ÏÈ· 50-100 x 109/L) ¯ˆÚ›˜ ·ÈÌÔÚÚ·ÁÈ΋ ‰È¿ıÂÛË Î·È ·Ó·ÈÌ›· ÁÈ· ÙËÓ ÔÔ›· ˘Ô‚Ï‹ıËΠ۠ÌÂÙ¿ÁÁÈÛË ÌÂ Û˘Ì˘Îӈ̤ӷ ÂÚ˘ıÚ¿. ∞fi ÙÔ ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È fiÙÈ Û 2-3 Ù˘¯·›Â˜ ÌÂÙÚ‹ÛÂȘ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ‚Ú¤ıËΠ‹È· ıÚÔÌ‚ÔÂÓ›· (·ÈÌÔÂÙ¿ÏÈ· 80-150 à 109/L), Ë ÔÔ›· ‰ÂÓ ‰ÈÂÚ¢ӋıËΠÂÚ·ÈÙ¤Úˆ. ∞fi ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ‰È·ÈÛÙÒıËΠfiÙÈ Ë ÌËÙ¤Ú· ·Ó·Ê¤ÚÂÈ Î·È ·˘Ù‹ ‹È· ıÚÔÌ‚ÔÂÓ›· ¯ˆÚ›˜ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ, Ë ÔÔ›· ·Ó·Î·Ï‡ÊıËÎÂ Ù˘¯·›· Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·. ∞fi ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ÙÔ˘ ·È‰ÈÔ‡, ÌÂÙ¿ ÙËÓ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Ïԛ̈͢ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡, ‰ÂÓ ‰È·ÈÛÙÒıËΠٛÔÙ· ·ıÔÏÔÁÈÎfi, ÂÓÒ Ë ÛˆÌ·ÙÈ΋ Î·È „˘¯ÔÎÈÓËÙÈ΋ ·Ó¿Ù˘ÍË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∞fi ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË Ù˘ ÌËÙ¤Ú·˜ ‰È·ÈÛÙÒıËΠfiÙÈ ÂÚfiÎÂÈÙÔ ÁÈ· Á˘Ó·›Î· 33 ¯ÚÔÓÒÓ, ˘ÁÈ‹, ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ۈ̷ÙÈ΋ Î·È ÓÔËÙÈ΋ ·Ó¿Ù˘ÍË, ¯ˆÚ›˜ ÈÛÙÔÚÈÎfi ·ÈÌÔÚÚ·ÁÈ΋˜ ‰È¿ıÂÛ˘ ̤¯ÚÈ Û‹ÌÂÚ·. ∞fi ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ÙÔ˘ ·È‰ÈÔ‡ Î·È Ù˘ ÌËÙ¤Ú·˜ ‰È·ÈÛÙÒıËΠıÚÔÌ‚ÔÂÓ›· (·ÚÈıÌfi˜ ·ÈÌÔÂÙ·Ï›ˆÓ 50-120 x 109/L) Û Â·ÓÂÈÏËÌ̤Ó˜ ÌÂÙÚ‹ÛÂȘ Û ·ÈÌ·ÙÔÏÔÁÈÎÔ‡˜ ·Ó·Ï˘Ù¤˜, ÂÓÒ ÔÈ ˘fiÏÔÈ˜ ·ÈÌ·ÙÔÏÔÁÈΤ˜ ÛÂÈÚ¤˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜. ªÂÙÚ‹ÛÂȘ ÙÔ˘ ·ÚÈıÌÔ‡ Î·È ÙÔ˘ fiÁÎÔ˘ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ¤ÁÈÓ·Ó ¤ÓÙ ÊÔÚ¤˜ Û ‰È¿ÛÙËÌ· Ô¯ÙÒ ÌËÓÒÓ Î·È ÛÙȘ ‰‡Ô ÂÚÈÙÒÛÂȘ. ™ÙȘ Ï‹„ÂȘ ‰Â›ÁÌ·ÙÔ˜ ·›Ì·ÙÔ˜ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ˆ˜ ·ÓÙÈËÎÙÈο K3-EDTA, Ë·Ú›ÓË Î·È ÎÈÙÚÈÎfi Ó¿ÙÚÈÔ. √È ÌÂÙÚ‹ÛÂȘ ¤ÁÈÓ·Ó ÛÙÔ ¯ÚfiÓÔ 0, 60, 180 Î·È 240 min. T·˘Ùfi¯ÚÔÓ· ÁÈÓfiÙ·Ó Î·È ÌÈÎÚÔÛÎÔÈ΋ ÂͤٷÛË ÙÔ˘ ·ÚÈıÌÔ‡ Î·È Ù˘ ÌÔÚÊÔÏÔÁ›·˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Û Â›¯ÚÈÛÌ· ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ÌÂ Î·È ¯ˆÚ›˜ ÙË ¯ÚËÛÈÌÔÔ›ËÛË ·ÓÙÈËÎÙÈÎÔ‡. √ ·ÚÈıÌfi˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ÛÙÔÓ ·Ó·Ï˘Ù‹ Î˘Ì·ÈÓfiÙ·Ó ¿ÓÙÔÙ ÌÂٷ͇ 50-120 x 109/L Î·È Ô Ì¤ÛÔ˜ fiÁÎÔ˜ ÙÔ˘˜ ÌÂٷ͇ 12-16 fl. ∞ÓÙ›ıÂÙ·, Ô ·ÚÈıÌfi˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ‚Ú¤ıËΠ̤۷

¶ÂÚÈÁÚ·Ê‹ ÂÚÈÙÒÛÂˆÓ ¶ÚfiÎÂÈÙ·È ÁÈ· ÎÔÚ›ÙÛÈ ÂÙ¿ ¯ÚÔÓÒÓ Ô˘ ÚÔÛÎÔÌ›ÛıËΠÛÙËÓ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ÁÈ· Èı·Ó‹ Ïԛ̈ÍË ÙÔ˘ ηÙÒÙÂÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Î·È ÛÙÔ ÔÔ›Ô ‰È·ÈÛÙÒıËΠ‹È· ıÚÔÌ‚ÔÂÓ›· ¯ˆÚ›˜ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ. ∞fi ÙÔ ÂÚÈÁÂÓÓËÙÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È fiÙÈ ÁÂÓÓ‹ıËΠÚfiˆÚ·, Ì ‚¿ÚÔ˜ Á¤ÓÓËÛ˘

77


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 78

¶·È‰È·ÙÚÈ΋ 2001;64:76-80

ÛÙ· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· (150-400 à 109/L) fiÙ·Ó ÔÈ ÌÂÙÚ‹ÛÂȘ ¤ÁÈÓ·Ó Ì ¿ÌÂÛÔ ÙÚfiÔ ÛÙÔ ÌÈÎÚÔÛÎfiÈÔ. ªfiÓÔ Û ¤Ó· ‰Â›ÁÌ· Ô ·ÚÈıÌfi˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ‚Ú¤ıËΠÔÚȷο ÂÏ·Ùو̤ÓÔ˜. £¤ÚÌ·ÓÛË ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ ÛÙÔ˘˜ 37ÔC ‰ÂÓ ÙÚÔÔÔ›ËÛ ٷ Â˘Ú‹Ì·Ù· ·fi ÙȘ ÌÂÙÚ‹ÛÂȘ. ∆Ô Â›¯ÚÈÛÌ· ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ¤‰ÂÈÍ ÙËÓ ·ÚÔ˘Û›· ÛˆÚÒÓ Î·È ÌÂÁ¿ÏˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (∂ÈÎfiÓ· 1). ∏ Ï‹Ú˘ ÂÚÁ·ÛÙËÚȷ΋ ‰ÈÂÚ‡ÓËÛË ÙˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ Ê·›ÓÂÙ·È ÛÙÔÓ ›Ó·Î· 1. ™ÙÔ Ì˘ÂÏfiÁÚ·ÌÌ· ÙÔ˘ ·È‰ÈÔ‡ ‚Ú¤ıËΠ·˘ÍË̤ÓÔ˜ ·ÚÈıÌfi˜ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ÌÔÚÊÔÏÔÁ›· Î·È ÔÏÏ¿ ·ÈÌÔÂÙ¿ÏÈ· (∂ÈÎfiÓ˜ 2, 3). ∞ÓÙÈ·ÈÌÔÂÙ·Ïȷο ·ÓÙÈÛÒÌ·Ù· ·ÓȯÓ‡ıËÎ·Ó ÌfiÓÔ Ì ÙËÓ ¿ÌÂÛË Ì¤ıÔ‰Ô, ‰ËÏ·‰‹ ÛÙÔ ÔÏÈÎfi ·›Ì· Î·È fi¯È ÛÙÔÓ ÔÚfi ÙˆÓ ·ÛıÂÓÒÓ, ÂÓÒ Ô Ù›ÙÏÔ˜ ÙˆÓ ÎÚ˘ÔÛÊ·ÈÚÈÓÒÓ ÛÙÔÓ ÔÚfi (ÌËÙ¤Ú·) ‹Ù·Ó ÂÏ·ÊÚ¿ ·˘ÍË̤ÓÔ˜. ª¤¯ÚÈ Û‹ÌÂÚ· ÌËÙ¤Ú· Î·È ÎfiÚË ‰ÂÓ ¤¯Ô˘Ó ÂÌÊ·Ó›ÛÂÈ Î·Ó¤Ó· ÎÏÈÓÈÎfi Û‡Ìو̷ Î·È ‰ÂÓ ¯ÚÂÈ¿ÛıËΠη̛· ıÂÚ·¢ÙÈ΋ ·Ú¤Ì‚·ÛË.

Paediatriki 2001;64:76-80

∂ÈÎfiÓ· 1. ¢Â›ÁÌ· ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ÙÔ˘ ·È‰ÈÔ‡ Ì ۈÚÔ‡˜ Î·È ÌÂÁ¿Ï· ·ÈÌÔÂÙ¿ÏÈ·.

™˘˙‹ÙËÛË ∏ «„¢‰ÔıÚÔÌ‚ÔÂÓ›·» ·ÔÙÂÏ› Ì›· ÂÚÁ·ÛÙË-

¶›Ó·Î·˜ 1. ∂ÚÁ·ÛÙËÚȷ΋ ‰ÈÂÚ‡ÓËÛË ÙˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ (ÌËÙ¤Ú·-ÎfiÚË). ∞ÈÌ·ÙÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ªËÙ¤Ú· ∞ÈÌÔÛÊ·ÈÚ›ÓË (g/dl) 12 AÚÈıÌfi˜ Ï¢ÎÔ΢ÙÙ¿ÚˆÓ (X109/L) 5,5 ∞Ú. ∞ÈÌÔÂÙ·Ï›ˆÓ (x109/L) 50-90 (·ÈÌ·ÙÔÏ. ·Ó·Ï˘Ù‹˜ Ì ¯Ú‹ÛË ‰È·ÊfiÚˆÓ ·ÓÙÈËÎÙÈÎÒÓ)

¶·È‰› 11,2 4,7 60-120

∞Ú. ∞ÈÌÔÂÙ·Ï›ˆÓ (x109/L) (̤ÙÚËÛË Û Ͽη Neubauer Î·È Â›¯ÚÈÛÌ· ÂÚ. ·›Ì·ÙÔ˜)

150-400

ª¤ÛÔ˜ fiÁÎÔ˜ ·ÈÌÔÂÙ·Ï›ˆÓ (Fl) ªÔÚÊÔÏÔÁ›· ·ÈÌÔÂÙ·Ï›ˆÓ ŒÏÂÁ¯Ô˜ ·Ó·ÈÌ›·˜ (Û›‰ËÚÔ˜, ÊÂÚÚÈÙ›ÓË, µ12, Ê˘ÏÏÈÎfi Ô͇, ¢∂∫, G6PD, ÌÔÚÊÔÏÔÁ›· ÂÚ˘ıÚÒÓ, ËÏÂÎÙÚÔÊfiÚËÛË Hb)

~ 16 ~ 12 ÌÂÁ¿Ï· ·ÈÌÔÂÙ¿ÏÈ· Ô˘ Û¯ËÌ·Ù›˙Ô˘Ó ÛˆÚÔ‡˜ ÎÊ ÎÊ

ŒÏÂÁ¯Ô˜ ·ÈÌfiÛÙ·Û˘ PT-APTT, ÈÓˆ‰ÔÁfiÓÔ, ·Ú¿ÁÔÓÙ·˜ VIII, vWF, vWFAg, ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ· ·ÈÌÔÂÙ·Ï›ˆÓ (Û˘ÛÛÒÚ¢ÛË ÌÂÙ¿ ÚÔÛı‹ÎË ADP, ÎÔÏÏ·ÁfiÓÔ˘, ÚÈÛÙÔÛÂÙ›Ó˘) ÃÚfiÓÔ˜ ÚÔ‹˜ (IVY method) ª˘ÂÏfiÁÚ·ÌÌ· (‰ÂÓ ¤ÁÈÓ ÛÙË ÌËÙ¤Ú·)

7,5 min 4 min ·˘ÍË̤ÓÔ˜ ·ÚÈıÌfi˜ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ÌÔÚÊÔÏÔÁ›· Î·È ÔÏÏ¿ ·ÈÌÔÂÙ¿ÏÈ·

U/S ‹·ÙÔ˜-ÛÏËÓfi˜

‰ÂÓ ¤ÁÈÓÂ

µ·ÛÈÎfi˜ ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜

ÎÊ

150-400

ÎÊ

∞ÓÔÛÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ∞ÓÔÛÔÛÊ·ÈÚ›Ó˜-˘ÔÙ¿ÍÂȘ, ∞¡∞-DNA-C3-C4-CIC ÎÊ ∫Ú˘ÔÛÊ·ÈÚ›Ó˜ ÔÚÔ‡ (ng/100ml) 18 AÓÙÈ·ÈÌÔÂÙ·Ïȷο ·ÓÙÈÛÒÌ·Ù· (¿ÌÂÛË Ì¤ıÔ‰Ô˜, ÔÏÈÎfi ·›Ì·) ıÂÙÈο (¤ÌÌÂÛË Ì¤ıÔ‰Ô˜, ÔÚfi˜) ·ÚÓËÙÈο √ÚÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ·ÚÓËÙÈÎfi˜ (Ë·Ù›Ùȉ· B Î·È C, HIV, ÂÚ˘ıÚ¿, ÙÔÍfiÏ·ÛÌ·, ΢ÙÙ·ÚÔÌÂÁ·ÏÔ˚fi˜) ∞ÎÔ˘ÔÏÔÁÈÎfi˜-ÔÊı·ÏÌÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÎÊ

78

ÎÊ

ÎÊ ÎÊ

ÎÊ 4 ıÂÙÈο ·ÚÓËÙÈο ·ÚÓËÙÈÎfi˜ ÎÊ


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Paediatriki 2001;64:76-80

∂ÈÎfiÓ· 2. ¢Â›ÁÌ· Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ Ì ¿ÊıÔÓ· ¿ˆÚ· ÌÂÁ·Î·Ú˘Ô·ÙÙ·Ú·.

∂ÈÎfiÓ· 3. ¢Â›ÁÌ· Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ fiÔ˘ ‰È·ÎÚ›ÓÔÓÙ·È ÌÂÁ·Î·Ú˘Ô·ÙÙ·Ú· Î·È ÛˆÚÔ› ·fi ÌÂÁ¿Ï· ·ÈÌÔÂÙ¿ÏÈ·.

Úȷ΋ ÔÓÙfiÙËÙ· Ô˘ ‰È·ÈÛÙÒıËΠÌÂÙ¿ ÙËÓ ¤ÓÙ·ÍË ÙˆÓ ·ÈÌ·ÙÔÏÔÁÈÎÒÓ ·Ó·Ï˘ÙÒÓ ÛÙËÓ Î·ıËÌÂÚÈÓ‹ Ú¿ÍË Î·È ÔÊ›ÏÂÙ·È ÛÙË Û˘ÛÛÒÚ¢ÛË ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ·Ú¿ ÙË ¯ÚËÛÈÌÔÔ›ËÛË ·ÓÙÈËÎÙÈÎÒÓ. ∏ «„¢‰ÔıÚÔÌ‚ÔÂÓ›·» ·Ó¢ڛÛÎÂÙ·È Û ÔÛÔÛÙ¿ 0,01-0,1% ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ ·›Ì·ÙÔ˜ Ô˘ Ï·Ì‚¿ÓÔÓÙ·È Ì ·ÓÙÈËÎÙÈÎfi ÙÔ EDTA, Ô˘ ·ÔÙÂÏ› ÙË Û˘¯ÓfiÙÂÚË ·ÈÙ›· ·˘ÙÔ‡ ÙÔ˘ Ê·ÈÓÔ̤ÓÔ˘ (6-9). ¶ÚfiÎÂÈÙ·È Ì¿ÏÏÔÓ ÁÈ· ·˘ÙÔ·ÓÙÈÛÒÌ·Ù· Ô˘ ηÙ¢ı‡ÓÔÓÙ·È ÂÓ·ÓÙ›ÔÓ ÎÔÈÓÒÓ ¯ËÌÈÎÒÓ Ô˘ÛÈÒÓ Û˘ÁÁÂÓÒÓ ÚÔ˜ ÙÔ EDTA Î·È Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ·ÓÙ›‰Ú·ÛË (6). "梉ÔıÚÔÌ‚ÔÂÓ›·" ÌÔÚ› Â›Û˘ Ó· ·ÚÔ˘ÛÈ·ÛÙ› Î·È ·fi ¿ÏϘ ·Èٛ˜, fiˆ˜ ÌÂÙ¿ ·fi Ï‹„Ë ·›Ì·ÙÔ˜ Ì η΋ Ù¯ÓÈ΋ ‹ ÏfiÁˆ Û¯ËÌ·ÙÈÛÌÔ‡ ‹ÁÌ·ÙÔ˜, ÂÓÒ ÙÔ Ê·ÈÓfiÌÂÓÔ ·˘Ùfi ¤¯ÂÈ Û˘Û¯ÂÙÈÛı› Î·È Ì ‰È¿ÊÔÚ˜ ·ıÔÏÔÁÈΤ˜ ηٷÛÙ¿ÛÂȘ ÛÙȘ Ôԛ˜ ·Ú·ÙËÚÂ›Ù·È ·ÓÔÛÔÏÔÁÈ΋ ‰È¤ÁÂÚÛË (ηÎÔ‹ıÂȘ, Ë·ÙÈ΋ ÓfiÛÔ˜, ·˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù·). ™˘Û¯ÂÙ›˙ÂÙ·È ·ÎfiÌË Î·È Ì Ïԛ̈ÍË ·fi CMV, HIV, ÂÚ˘ıÚ¿ ÎÏ (6,9). √ ÏÂÙÔÌÂÚ‹˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ ·¤ÎÏÂÈÛ ÙËÓ ÂÚ›ÙˆÛË «„¢‰ÔıÚÔÌ‚ÔÂÓ›·˜» ÔÊÂÈÏfiÌÂÓ˘ Û EDTA ‹ ¿ÏÏ· ·ÓÙÈËÎÙÈο, fiˆ˜ Î·È ÙËÓ ‡·ÚÍË ÙˆÓ ·ıÔÏÔÁÈÎÒÓ Î·Ù·ÛÙ¿ÛÂˆÓ Ô˘ ¤¯Ô˘Ó Û˘Û¯ÂÙÈÛı› Ì ÙÔ Ê·ÈÓfiÌÂÓÔ ·˘Ùfi. «æ¢‰ÔıÚÔÌ‚ÔÂÓ›·» Û˘Ì‚·›ÓÂÈ Â›Û˘ Û ηٷÛÙ¿ÛÂȘ Ô˘ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ÌÂÁ¿Ï· ‹ Î·È ÁÈÁ¿ÓÙÈ· ·ÈÌÔÂÙ¿ÏÈ·, ÁÈ·Ù› Ù· ·ÈÌÔÂÙ¿ÏÈ· ·˘Ù¿ ÏfiÁˆ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙÔ˘˜ ηٷÌÂÙÚÒÓÙ·È Ï·Óı·Ṳ̂ӷ ·fi ÙÔÓ ·Ó·Ï˘Ù‹ (6-9). ™ÙȘ ‰ÈΤ˜ Ì·˜ ÂÚÈÙÒÛÂȘ ÛÙË ÌÈÎÚÔÛÎÔÈ΋ ÂͤٷÛË ÙˆÓ ÂȯÚÈÛÌ¿ÙˆÓ ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ·Ú·ÙËÚ‹ıËÎ·Ó ÌÈÎÚÔ› ÛˆÚÔ› ·ÈÌÔÂÙ·Ï›ˆÓ Î·È ÌÂÁ¿Ï·-ÁÈÁ·ÓÙÈ·›· ·ÈÌÔÂÙ¿ÏÈ· (∂ÈÎfiÓ· 1), ηٷÛÙ¿ÛÂȘ Ô˘ ‰ÈηÈÔÏÔÁÔ‡Ó Èı·ÓÒ˜ ÙȘ ¯·ÌËϤ˜ ÙÈ̤˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ÛÙȘ ‰È¿ÊÔÚ˜ ÌÂÙÚ‹ÛÂȘ. ∞’ fiÛ· ÁÓˆÚ›˙Ô˘Ì ÔÈ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Û˘Óԉ‡ÔÓÙ·È ·fi ‹È·

¤ˆ˜ ÛÔ‚·Ú‹ ıÚÔÌ‚ÔÂÓ›· Ì ·Ó¿ÏÔÁ· Û˘ÌÙÒÌ·Ù· (1,2,5). ™ÙȘ ÂÚÈÙÒÛÂȘ Ô˘ ÂÚÈÁÚ¿ÊËÎ·Ó fiϘ ÔÈ ÁÓˆÛÙ¤˜ ‰È·Ù·Ú·¯¤˜ Ì ÁÈÁ·ÓÙÈ·›· ·ÈÌÔÂÙ¿ÏÈ· ·ÔÎÏ›ÛıËÎ·Ó ÏfiÁˆ Ù˘ ·Ô˘Û›·˜ Ï¢ÎÔ΢ÙÙ·ÚÈÎÒÓ ÂÁÎÏ›ÛÙˆÓ, Ù˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ·˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ, ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ¯ÚfiÓÔ˘ ÚÔ‹˜, Ù˘ ·Ô˘Û›·˜ ÎÏÈÓÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ Î·È ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ‚ÈÔ¯ËÌÈÎÔ‡, ÔÊı·ÏÌÔÏÔÁÈÎÔ‡ Î·È ·ÎÔ˘ÔÏÔÁÈÎÔ‡ ÂϤÁ¯Ô˘. ™·ÓÈfiÙÂÚ˜ ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ, fiˆ˜ 1) Ë ÔÈÎÔÁÂÓ‹˜ ıÚÔÌ‚ÔÂÓ›· Paris-Trousseau Ô˘ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ·ÚÔ˘Û›· ÁÈÁ·ÓÙÈ·›ˆÓ ·-ۈ̷ٛˆÓ Û ¤Ó·Ó ˘ÔÏËı˘ÛÌfi ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (12), 2) ÌÈ· ·Ú·ÏÏ·Á‹ ·˘Ù‹˜ (12) Î·È 3) ÙÔ ÔÈÎÔÁÂÓ¤˜ Û‡Ó‰ÚÔÌÔ ÁÈÁ·ÓÙÈ·›ˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Ô˘ ÂÚÈÁÚ¿ÊËΠ·fi ÙÔÓ Greaves Î·È ÙÔ˘˜ Û˘ÓÂÚÁ¿Ù˜ ÙÔ˘ (13), ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ‰ÔÌÈΤ˜, ÌÂÙ·‚ÔÏÈΤ˜ Î·È ÏÂÈÙÔ˘ÚÁÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ, fiˆ˜ Î·È ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ (11). ™ÙȘ ‰ÈΤ˜ Ì·˜ ÂÚÈÙÒÛÂȘ ÔÈ ·Ú·¿Óˆ ηٷÛÙ¿ÛÂȘ ·ÔÎÏ›ÔÓÙ·È ÏfiÁˆ Ê˘ÛÈÔÏÔÁÈ΋˜ ÌÔÚÊÔÏÔÁ›·˜ ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ Î·È Ê˘ÛÈÔÏÔÁÈ΋˜ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ·˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ. ∏ ‰È¿ÁÓˆÛË Ù˘ ÔÈÎÔÁÂÓÔ‡˜ 𣶠ÏfiÁˆ ·‡ÍËÛ˘ ÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ Î·È ·Ó‡ÚÂÛ˘ ıÂÙÈÎÒÓ ·ÓÙÈ·ÈÌÔÂÙ·ÏÈ·ÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ·ÔÎÏ›ÛıËÎÂ, ‰ÈfiÙÈ ÛÙ· ‰Â›ÁÌ·Ù· ÂÚÈÊÂÚÈÎÔ‡ ·›Ì·ÙÔ˜ ÌÈÎÚÔÛÎÔÈο ‰ÂÓ ‰È·ÈÛÙÒıËΠıÚÔÌ‚ÔÂÓ›·. ∂›Û˘, ÛÙËÓ π£¶ Ù· ·ÈÌÔÂÙ¿ÏÈ· ÌÔÚ› Ó· Â›Ó·È ÌÂÁ¿Ï· ·ÏÏ¿ ‰ÂÓ ·ıÚÔ›˙ÔÓÙ·È Û ۈÚÔ‡˜ (3,14). ∆¤ÏÔ˜, ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ̤¯ÚÈ Û‹ÌÂÚ· ·ÚÎÂÙ¤˜ ÂÚÈÙÒÛÂȘ ıÚÔÌ‚ÔÂÓ›·˜ Ì ÌÂÁ¿Ï· ·ÈÌÔÂÙ¿ÏÈ· Ô˘ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ηٷٷÁÔ‡Ó Û η̛· ·fi ÙȘ ÚÔ·Ó·ÊÂÚı›Û˜ ηٷÛÙ¿ÛÂȘ Î·È ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ·˘ÙÔۈ̷ÙÈÎfi ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú· ÎÏËÚÔÓÔÌÈÎfiÙËÙ·˜ ‹ ¿ÁÓˆÛÙÔ Û ¿ÏϘ ÂÚÈÙÒÛÂȘ, ·Ó‡ÚÂÛË ÌÂÁ¿ÏˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Î·È ·˘ÍË̤ÓÔ˘

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¶·È‰È·ÙÚÈ΋ 2001;64:76-80

̤ÛÔ˘ fiÁÎÔ˘ ÙÔ˘˜, ‹È· ‹ η̛· ·ÈÌÔÚÚ·ÁÈ΋ ‰È¿ıÂÛË, Ê˘ÛÈÔÏÔÁÈÎfi ·ÚÈıÌfi ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ, ·Ô˘Û›· Ï¢ÎÔ΢ÙÙ·ÚÈÎÒÓ ÂÁÎÏ›ÛÙˆÓ, Ê˘ÛÈÔÏÔÁÈÎfi ¯ÚfiÓÔ ÚÔ‹˜, Ê˘ÛÈÔÏÔÁÈ΋ Û˘Ó‹ıˆ˜ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ· ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Î·È ·ÚÔ˘Û›· ·ÓÙÈ·ÈÌÔÂÙ·ÏÈ·ÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ Û ¤Ó· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi (12,9-10). ∏ ·ÚÔ˘Û›· ÙˆÓ ·ÓÙÈ·ÈÌÔÂÙ·ÏÈ·ÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ Èı·ÓfiÓ ·ÓÙ·Ó·ÎÏ¿ ÌfiÓÔ ÛÙËÓ ·‡ÍËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (1), ÂÓÒ ÌÔÚ› Ó· Û¯ÂÙ›˙ÂÙ·È Ì ¿ÏϘ ηٷÛÙ¿ÛÂȘ, fiˆ˜ ·ÓÔÛÔ·ÓÂ¿ÚÎÂȘ, Ïԛ̈ÍË ·fi ÙÔÓ Èfi Ù˘ ·ÓÔÛÔ·ÓÂ¿ÚÎÂÈ·˜, Û˘ÛÙËÌ·ÙÈÎfi ÂÚ˘ıËÌ·ÙÒ‰Ë Ï‡ÎÔ, ·˘ÙÔ¿ÓÔÛË ı˘ÚÂÔÂȉ›Ùȉ· Î·È Ï‹„Ë Ê·ÚÌ¿ÎˆÓ (3,5). √È Î·Ù·ÛÙ¿ÛÂȘ ·˘Ù¤˜ ·ÔÎÏ›ÛıËÎ·Ó ÚÔ˜ ÙÔ ·ÚfiÓ ÛÙȘ ‰ÈΤ˜ Ì·˜ ÂÚÈÙÒÛÂȘ ·fi ÙÔ ÈÛÙÔÚÈÎfi Î·È ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô. ∏ ·Ó‡ÚÂÛË ·˘ÍË̤ÓÔ˘ ·ÚÈıÌÔ‡ ÌÂÁ·Î·Ú˘Ô΢ÙÙ¿ÚˆÓ ÌÂ Ê˘ÛÈÔÏÔÁÈ΋ ÌÔÚÊÔÏÔÁ›· ÛÙÔ ·È‰›, Ù· ÛÙ·ıÂÚ¿ Â˘Ú‹Ì·Ù· ÙˆÓ ÁÈÁ·ÓÙÈ·›ˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Î·È Ù˘ Ù·¯Â›·˜ Û˘ÛÒÚ¢Û˘ ·˘ÙÒÓ, Ë ·ÚÔ˘Û›· ·ÓÙÈ·ÈÌÔÂÙ·ÏÈ·ÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙË ÌËÙ¤Ú· Î·È ÛÙÔ ·È‰›, Ë ÌÈÎÚ‹ ·‡ÍËÛË ÙÔ˘ Ù›ÙÏÔ˘ ÙˆÓ ÎÚ˘ÔÛÊ·ÈÚÈÓÒÓ ÛÙË ÌËÙ¤Ú·, fiˆ˜ Î·È Ë ·Ó‡ÚÂÛË ÔÚȷο ÂÏ·Ùو̤ÓÔ˘ ·ÚÈıÌÔ‡ ·ÈÌÔÂÙ·Ï›ˆÓ Û ̛· ̤ÙÚËÛË, ¯ˆÚ›˜ ÎÏÈÓÈο Â˘Ú‹Ì·Ù·, ‰ÂÓ ‰‡Ó·Ù·È Ó· ‰ÈηÈÔÏÔÁËıÔ‡Ó Ï‹Úˆ˜. £· ÌÔÚÔ‡Û·Ó Ó· ‰ÈηÈÔÏÔÁËıÔ‡Ó Èı·ÓÒ˜ ·fi οÔÈ· ·ÓÔÛÔÏÔÁÈ΋ ‰È·Ù·Ú·¯‹ Ô˘ Ô‰ËÁ› ÛÙÔ Û¯ËÌ·ÙÈÛÌfi ·˘ÙÔ·ÓÙÈۈ̿وÓ, Ì ·ÔÙ¤ÏÂÛÌ· ÙË Û˘Ó¯‹ ηٷÛÙÚÔÊ‹ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Î·È ÙËÓ ˘ÂÚÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ, ¤ÙÛÈ ÒÛÙÂ Ô ·ÚÈıÌfi˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ ÛÙËÓ ÂÚÈʤÚÂÈ· Ó· Â›Ó·È ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ÌÂÙÚ‹ÛÂȘ Ê˘ÛÈÔÏÔÁÈÎfi˜. ™ÙËÓ ÂÚ›ÙˆÛË ·˘Ù‹ ı· ÌÔÚÔ‡Û ӷ ÂÍËÁËı› Ë ·ÚÔ˘Û›· ÙˆÓ ÌÂÁ¿ÏˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ, Ë Ù·¯Â›· Û˘ÁÎfiÏÏËÛË ·˘ÙÒÓ Î·È Ô Û¯ËÌ·ÙÈÛÌfi˜ ÌÈÎÚÒÓ ÛˆÚÒÓ, ÁÂÁÔÓfi˜ Ô˘ ÂÍËÁ› ÙËÓ «„¢‰ÔıÚÔÌ‚ÔÂÓ›·». ™ÙȘ ÂÚÈÙÒÛÂȘ Ô˘ ÂÚÈÁÚ¿ÊÔ˘Ì ‰ÂÓ ‚Ú¤ıËΠ·fi ÙÔÓ ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÂÁ¯Ô οÔÈ· ‰È·Ù·Ú·¯‹ Î·È Èı·ÓÔÏÔÁԇ̠fiÙÈ Ë ‰È¿ÁÓˆÛË Â›Ó·È ·˘Ù‹ Ù˘ ÎÏËÚÔÓÔÌÈ΋˜, ÌË ÂȉÈ΋˜ ‰È·Ù·Ú·¯‹˜ ÙˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ Ì ÂÌÊ¿ÓÈÛË ÁÈÁ·ÓÙÈ·›ˆÓ ·ÈÌÔÂÙ·Ï›ˆÓ (2), ‰È¿ÁÓˆÛË Ô˘ ÌÔÚ› Ó· ·Ó·ÙÚ·› ·Ó ÔÈ ÂÚÈÙÒÛÂȘ Ô˘ ÂÚÈÁÚ¿ÊÔÓÙ·È Î·È ·Ú·ÎÔÏÔ˘ıÔ‡ÓÙ·È Û˘ÛÙËÌ·ÙÈο ÂÌÊ·Ó›ÛÔ˘Ó ÎÏÈÓÈ΋ Û˘Ìو̷ÙÔÏÔÁ›· ÛÙÔ Ì¤ÏÏÔÓ. ∂˘¯·ÚÈÛٛ˜ ∂˘¯·ÚÈÛÙԇ̠ıÂÚÌ¿ ÙÔ ÚÔÛˆÈÎfi ÙÔ˘ ∞ÈÌ·ÙÔÏÔÁÈÎÔ‡ ∂ÚÁ·ÛÙËÚ›Ô˘ Ù˘ µ’ ¶ÚÔ·È‰Â˘ÙÈ΋˜ ¶·ıÔÏÔÁÈ΋˜ ÙÔ˘ ∞.¶.£. ÁÈ· ÙËÓ ÂÈÛÙËÌÔÓÈ΋ ÙÔ˘˜ ‚Ô‹ıÂÈ· ÛÙË ‰ÈÂÚ‡ÓËÛË ÙÔ˘ ÂÚÈÛÙ·ÙÈÎÔ‡ Î·È ÙË ‰ÈÂÓ¤ÚÁÂÈ· ÙÔ˘ ÂϤÁ¯Ô˘ Ù˘ ·ÈÌfiÛÙ·Û˘.

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µÈ‚ÏÈÔÁÚ·Ê›· 1. Fabris F, Cordiano I, Salvan F, Ramon R, Valente M, Luzzatto G et al. Cronic isolated macrothrom- bocytopenia with autosomal dominant transmission: a morphological and qualitative platelet disorder. Eur J Haematol 1997;58:40-45. 2. Young G, Luban N, White GJ. Giant platelet disorders in African-American children misdiagnosed as idiopathic thrombocytopenic purpura. J Pediatr Hematol Oncol 1999;21:231-236. 3. µÔÚÁÈ¿˜ ¡π, §·Ô˘Ù¿Ú˘ ¡¶. ¢È·Ù·Ú·¯¤˜ ÙˆÓ ·ÈÌÔÌÂÙ·Ï›ˆÓ. ∞ÈÌÔÚÚ·ÁÈο ÓÔÛ‹Ì·Ù· Î·È ıÚfiÌ‚ˆÛË. ™ÙËÓ: ∞ÈÌ·ÙÔÏÔÁ›·. ∆fiÌÔ˜ µ’. 1Ë ¤Î‰. ∞ı‹Ó·: 1990. ÛÂÏ. 582-615. 4. Murphy S, Nepo A, Sills R. Thrombocytopenia. Paediatr in Review 1999;2:64-69. 5. Schultz-Beardsley D, Nathan GD. Platelet abnormalities in infancy and childhood. πn: Nathan GD, Oski AF, eds. Paediatric Haematology and Oncology. 4th ed. Philadelphia: WB Saunders Company; 1998. p.1585-1630. 6. µ˘˙·ÓÙÈ¿‰Ë˜ ∞∆, µ·Î·ÏÔÔ‡ÏÔ˘ ™, ª·Ó‰·Ï¿ ∂, §È¿ÏÈ· ∞, °·Ú˘›‰Ô˘ µ. 梉‹˜ ıÚÔÌ‚ÔÂÓ›·: ‰‡Ô ÂÚÈÙÒÛÂȘ. πÔÎÚ¿ÙÂÈ· 1998;2:91-95. 7. Hoyt HR, Durie GMB. Pseudothrombocytopenia induced by a monoclonal IgM kappa platelet agglutinin. Am J Hematol 1989;31:50-52. 8. Silvestri F, Virgolini L, Savignano C, Zaja F, Velisig M, Baccarani M. Incidence and diagnosis of EDTA-dependent pseudothrombocytopenia in a consecutive outpatient population referred for isolated thrombocytopenia. Vox Sang 1995;68:35-39. 9. Silvestri F, Masotti A, Pradella P, Zaja F, Barillari G, Marco L. More on false thrombocytopenias: EDTA-dependent pseudothrombocytopenia associated with a congenital platelet release defect. Vox Sang 1996;71:27-29. 10. Najean Y, Lecompte T. Genetic thrombocytopenia with autosomal dominant transmission: a review of 54 cases. J Haematol 1990;74:203-208. 11. Najean Y, Lecompte T. Hereditary thrombocytopenias in childhood. Semin Thromb Hemost 1995;21:294-304. 12. Breton-Gorius J, Favier R, Guichard J, Cherif D, Berger R, Debili N et al. A new congenital dysmegakaryopoietic thrombocytopenia (Paris-Trousseau) associated with giant platelet a-granules and chromosome 11 deletion at 11q23. Blood 1995;7:1805-1814. 13. Greaves M, Pickering C, Martin J, Cartwright I, Preston EF. A new familial ‘giant platelet syndrome’ with structural, metabolic and functional abnormalities due to a primary megacaryocyte defect. Br J Haematol 1987;65:429-435. 14. Sanchez-Fayos J, Olavarrva E, Roman A, Cabello A, Soto C, Panigua C. Idiopathic thrombocytopenic purpura in 5 members of a family. Sangre (Barc) 1994;39:215-217.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 22-06-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 13-10-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ÷Ù˙Ë·ÓÙÂÏ‹˜ ∂ÌÌ·ÓÔ˘‹Ï ¶. ¶··ÁˆÚÁ›Ô˘ 8, 546 35 £ÂÛÛ·ÏÔÓ›ÎË


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∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

Paediatriki 2001;64:81-84

CASE REPORT

ŒÏÏÂÈ„Ë ÙÔ˘ ÎÏ¿ÛÌ·ÙÔ˜ C6 ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ Î·È ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈΤ˜ ÏÔÈÌÒÍÂȘ ª. £ÂÔ‰ˆÚ›‰Ô˘, ª. πˆ¿ÓÓÔ˘, °. ªÔÛÙÚÔ‡, ∞. °ÂÚÌÂÓ‹˜

C6 complement deficiency and meningococcal infections M. Theodoridou, M. Ioannou, G. Mostrou, A. Germenis

¶ÂÚ›ÏË„Ë: ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ·ÁÔÚÈÔ‡ ËÏÈΛ·˜ 11 ÂÙÒÓ Ì ‰‡Ô ÚÔÛ‚ÔϤ˜ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋˜ ÌËÓÈÁÁ›Ùȉ·˜ Î·È Ì›· ÛË„·ÈÌ›·˜ Û ‰È¿ÛÙËÌ· 3 ¯ÚfiÓˆÓ Ì ÎÏÈÓÈο Î·È ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Û˘Ì‚·Ù¿ Ì Ïԛ̈ÍË ·fi ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ. √È ÂÚÈÙÒÛÂȘ Â·ÓÂÈÏËÌÌ¤ÓˆÓ Û˘ÛÙËÌ·ÙÈÎÒÓ ÏÔÈÌÒÍÂˆÓ ‰ÂÓ Â›Ó·È Û˘Ó‹ıÂȘ, ÁÈ’ ·˘Ùfi Ú¤ÂÈ Ó· ·Ó·˙ËÙÂ›Ù·È ˘fiÛÙڈ̷ ·ÓÔÛÔÏÔÁÈ΋˜ ‹ ·Ó·ÙÔÌÈ΋˜ ‰È·Ù·Ú·¯‹˜. √ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ Ô˘ ¤ÁÈÓÂ, ¤‰ÂÈÍ fiÙÈ ÙÔ ·È‰› ›¯Â Û˘ÁÁÂÓ‹ ÔÌfi˙˘ÁÔ, ¤ÏÏÂÈ„Ë ÙÔ˘ ÎÏ¿ÛÌ·ÙÔ˜ C6 ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜. ™˘˙ËÙÔ‡ÓÙ·È ÔÈ È‰È·ÈÙÂÚfiÙËÙ˜ Ù˘ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋˜ ÓfiÛÔ˘ Î·È Ë ·ıÔÁ¤ÓÂÈ· (1) Ù˘ Ïԛ̈͢ ÛÙ· ¿ÙÔÌ· Ì ¤ÏÏÂÈ„Ë ÂÓfi˜ ·fi ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ÙÔ˘ ÙÂÏÈÎÔ‡ Û˘ÌϤÁÌ·ÙÔ˜ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ C5-C9.

Abstract: The case of an 11ó year old child is described who had two episodes of meningococcal meningitis and one episode of meningococcal septicemia over a three-year period. Recurrent meningococcal disease is rare in childhood and if present, a full investigation should be performed in order to detect an immunologic or anatomic defect. Examination of the terminal pathway of the com- plement system in our patient revealed C6 deficiency, a rare autosomal recessive disorder. The patient’s parents had halfnormal levels of C6 and were carriers for the deficiency. The peculiarities of meningococcal infection and the pathogenesis of infection in complement deficiency states are discussed.

§¤ÍÂȘ ÎÏÂȉȿ: Â·ÓÂÈÏËÌ̤Ó˜ ÌËÓÈÁÁ›Ùȉ˜, ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈΤ˜ ÏÔÈÌÒÍÂȘ, ¤ÏÏÂÈ„Ë Û˘ÌÏËÚÒÌ·ÙÔ˜.

Key words: recurrent meningitis, recurrent meningococcal disease, complement deficiency.

∂ÈÛ·ÁˆÁ‹ ∏ ·ÓÂ¿ÚÎÂÈ· (1-3) ÙˆÓ ÎÏ·ÛÌ¿ÙˆÓ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ Û˘Ó‰˘¿˙ÂÙ·È Ì ÛÔ‚·Ú¤˜ ÌÈÎÚԂȷΤ˜ ÏÔÈÌÒÍÂȘ. ÕÙÔÌ· Ì ·ÓÂ¿ÚÎÂÈ· ÙˆÓ ÎÏ·ÛÌ¿ÙˆÓ Ù˘ ÎÏ·ÛÈ΋˜ Ô‰Ô‡ (4) (C1,C4,C2) ¤¯Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ÓÔÛ‹Ì·Ù· ÎÔÏÏ·ÁfiÓÔ˘ Î·È ÏÈÁfiÙÂÚÔ Û˘¯Ó¿ Û˘ÛÙËÌ·ÙÈΤ˜ ÏÔÈÌÒÍÂȘ ·fi Ó¢ÌÔÓÈfiÎÔÎÎÔ (Pn), ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ (ªn) Î·È ·ÈÌfiÊÈÏÔ ÈÓÊÏÔ˘¤ÓÙ˙·˜ Ù‡Ô˘ ‚ (Hib) (5). ÕÙÔÌ· Ì ·ÓÂ¿ÚÎÂÈ· Ù˘ ÂÓ·ÏÏ·ÎÙÈ΋˜ Ô‰Ô‡ (ÚÔÂÚ‰›Ó˘ (6), ·Ú¿ÁÔÓÙ· D) ¤¯Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ

ÁÈ· Û˘ÛÙËÌ·ÙÈΤ˜ ÏÔÈÌÒÍÂȘ ΢ڛˆ˜ ·fi ¡. Ù˘ ÌËÓÈÁÁ›Ùȉ·˜, ÂÓÒ Ë ·ÓÂ¿ÚÎÂÈ· ÙÔ˘ C3, ÙÔ˘ ·Ú¿ÁÔÓÙ· ∏ Î·È π Úԉȷı¤ÙÂÈ ÁÈ· ÏÔÈÌÒÍÂȘ ·fi ÌÈÎÚfi‚È· Ì ο„·, fiˆ˜ Ó¢ÌÔÓÈfiÎÔÎÎÔ, ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ Î·È ·ÈÌfiÊÈÏÔ ÈÓÊÏÔ˘¤ÓÙ˙·˜ Ù‡Ô˘ b. ∂›Û˘, Ë ·ÓÂ¿ÚÎÂÈ· ÙˆÓ ÙÂÏÈÎÒÓ ÎÏ·ÛÌ¿ÙˆÓ (4) ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ (C5-C9) ·˘Í¿ÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· Û˘ÛÙËÌ·ÙÈ΋ ÌËÓÔÁÁÈÙȉÔÎÔÎÎÈ΋ Ïԛ̈ÍË. ∆· ¿ÙÔÌ· ·˘Ù¿ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·‰˘Ó·Ì›· Û¯ËÌ·ÙÈÛÌÔ‡ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ C5-C9, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ¤¯Ô˘Ó ¤ÏÏÂÈ„Ë Ù˘ ‚·ÎÙËÚÈÔÏ˘ÙÈ΋˜ ‰Ú¿Û˘

∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, ¶ÂÚÈÊÂÚÂÈ·Îfi °ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô “°. °ÂÓÓËÌ·Ù¿˜”

1st Pediatric Clinic of Athens University, “G. Gennimatas” Hospital, Athens

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ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ ·˘ÙÔ‡, Ë ÔÔ›· ¤¯ÂÈ ÌÂÁ¿ÏË ÛËÌ·Û›· ÛÙËÓ ¿Ì˘Ó· ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡, ηÙÂÍÔ¯‹Ó ¤Ó·ÓÙÈ ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ Î·È fi¯È ¿ÏÏˆÓ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ. ªÂ ÙËÓ Â›ÛÔ‰Ô ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi ¤¯Ô˘Ì ÂÓÂÚÁÔÔ›ËÛË ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜, ·Ú·ÁˆÁ‹ Î·È ÚÔÛÎfiÏÏËÛË ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ C5-C9 ÛÙËÓ Â͈ÙÂÚÈ΋ ÌÂÌ‚Ú¿ÓË ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ Ô˘ Ô‰ËÁ› Û χÛË ÙÔ˘ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ‡ Î·È ·ÂÏ¢ı¤ÚˆÛË ÂÓ‰ÔÙÔ͛Ӣ (2,3). ™Ù· ¿ÙÔÌ· ·˘Ù¿ ‰ÂÓ ÂËÚ¿˙ÂÙ·È Ë ¯ËÌÂÈÔÙ·Í›·, Ë Ô„ˆÓÈÓÔÔ›ËÛË, Ë Ê·ÁÔ΢ÙÙ¿ÚˆÛË Î·È Ë ÂÓÂÚÁÔÔ›ËÛË Ù˘ ÂÓ·ÏÏ·ÎÙÈ΋˜ Ô‰Ô‡ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜.

Paediatriki 2001;64:81-84

¶›Ó·Î·˜ 1 CT ÂÁÎÂÊ¿ÏÔ˘ = Ê˘ÛÈÔÏÔÁÈÎfi

IgG1 = 710 mg/dl

MRI ÂÁÎÂÊ¿ÏÔ˘ = Ê˘ÛÈÔÏÔÁÈ΋ ∞ÓÔÛÔÛÊ·ÈÚ›Ó˜ IgG = 1150 mmg/dl IgA = 127 mg/dl IgM = 425 mg/dl C3 = 74 mg/dl (50-90) C4 = 14 mg/dl (10-40) C5 = 6 mg/dl (6-9) C6 = 8 mg/dl (45-95)

IgG2 = 152 mg/dl IgG3 = 98 mg/dl IgG4 = 55 mg/dl ANA = (-) antiDNA = (-) SMA = (-) AMA = (-) VIII Ag (-)

∞ÈÌÔÏ˘ÙÈÎfi˜ Ù›ÙÏÔ˜ ÎÏ·ÛÈ΋˜ Ô‰Ô‡ = 0 ∞ÈÌÔÏ˘ÙÈÎfi˜ Ù›ÙÏÔ˜ ÂÓ·ÏÏ·ÎÙÈ΋˜ Ô‰Ô‡ = 0

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ∞ÁfiÚÈ ËÏÈΛ·˜ 9 ÂÙÒÓ ÌÂٷʤÚıËΠÛÙËÓ ÎÏÈÓÈ΋ Ì·˜ ·fi ÓÔÛÔÎÔÌÂ›Ô Ù˘ Â·Ú¯›·˜ ÏfiÁˆ ˘„ËÏÔ‡ ˘ÚÂÙÔ‡ 39oC ·fi 24ÒÚÔ˘, Â̤ÙÔ˘˜ Î·È ·ÈÌÔÚÚ·ÁÈÎfi ÂÍ¿ÓıËÌ· ÛÙÔÓ ÎÔÚÌfi Î·È ÛÙ· ¿ÎÚ·. ™ÙÔÓ ·ÛıÂÓ‹ Ì ÙËÓ ÎÏÈÓÈ΋ ˘Ô„›· ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋˜ ÛË„·ÈÌ›·˜ ¯ÔÚËÁ‹ıËΠ·ÓÙÈ‚›ˆÛË. ¶ÚfiÎÂÈÙ·È ÁÈ· ÙÔ ÙÚ›ÙÔ ·È‰› Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜. ∆· 2 ·‰¤ÏÊÈ· ÙÔ˘ Â›Ó·È ˘ÁÈ‹. ∆Ô ·È‰› ·˘Ùfi ‰ÂÓ Â›¯Â ÓÔÛ‹ÛÂÈ ÛÙÔ ·ÚÂÏıfiÓ Î·È Â›¯Â οÓÂÈ Î·ÓÔÓÈο fiÏ· Ù· ÂÌ‚fiÏÈ·. ∫·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹, ÙÔ ·È‰› ‹Ù·Ó Û ηϋ ηٿÛÙ·ÛË, ¯ˆÚ›˜ ·˘¯ÂÓÈ΋ ‰˘Ûη̄›·, Ì ηϿ ˙ˆÙÈο ÛËÌ›· Î·È Ê˘ÛÈÔÏÔÁÈ΋ Ó¢ÚÔÏÔÁÈ΋ ÂͤٷÛË. ¶·ÚÔ˘Û›·˙ ÛÙÈÎÙfi Ì ·Û‡ÌÌÂÙÚË Î·Ù·ÓÔÌ‹ ÂÙ¯ÂÈ҉˜ ÂÍ¿ÓıËÌ· ÛÙÔÓ ÎÔÚÌfi Î·È Ù· ¿ÎÚ·. ∏ ηٿ Û˘ÛÙ‹Ì·Ù· ÂͤٷÛË ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈ΋. ∞fi ÙÔ ÁÂÓfiÌÂÓÔ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ‚Ú¤ıËÎ·Ó Ù· ÂÍ‹˜: Hb=13,3 g/dl, Ht=31%, §Â˘Î¿=28.900/Ìl (¶=88%, §=5%), ∞ª¶=198.000/Ìl, ∆∫∂=44 mm/h, CRP=174 mg/dl, P.T.=16’’. ∆· Â˘Ú‹Ì·Ù· ÙÔ˘ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘ ˘ÁÚÔ‡ ‹Ù·Ó: ∫‡ÙÙ·Ú·=930/mm3 (¶=70%, §=26%), §Â‡ÎˆÌ·=128mg/dl, ™¿Î¯·ÚÔ=60 mg/dl (™¿Î¯·ÚÔ ·›Ì·ÙÔ˜=97 mg/dl). To ¿ÌÂÛÔ ·Ú·Û··ÛÌ· ‹Ù·Ó ·ÚÓËÙÈÎfi, fiˆ˜ Î·È Ë ·Ó·˙‹ÙËÛË ÙˆÓ ÌÈÎÚÔ‚È·ÎÒÓ ·ÓÙÈÁfiÓˆÓ (Latex test). √È Î·ÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜, ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘ ˘ÁÚÔ‡ Î·È ÚÈÓÔÊ¿Ú˘ÁÁÔ˜ ‰ÂÓ ·Ó¤Ù˘Í·Ó ·ıÔÁfiÓ· ÌÈÎÚfi‚È·. ªÂ ‚¿ÛË ÙËÓ ÎÏÈÓÈ΋ ÂÈÎfiÓ· Î·È Ù· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· ¯ÔÚËÁ‹ıËΠÛÙÔÓ ·ÛıÂÓ‹ ÂÓÈÎÈÏ›ÓË Î·È ¯ÏˆÚ·ÌÊÂÓÈÎfiÏË ÂÓ‰ÔÊÏ‚›ˆ˜. ∆Ô ·È‰›, ‹‰Ë ·fi ÙÔ ‰Â‡ÙÂÚÔ 24ˆÚÔ ÓÔÛËÏ›·˜, ‹Ù·Ó Û ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË Î·È ÂÍ‹Ïı ÙÔ fiÁ‰ÔÔ 24ˆÚÔ ÌÂÙ¿ ÙË Û˘ÌÏ‹ÚˆÛË Ù˘ ıÂÚ·›·˜. ¢‡Ô ¯ÚfiÓÈ· ·ÚÁfiÙÂÚ·, Û ËÏÈΛ· 11 ¯ÚfiÓˆÓ, ÙÔ ·È‰› ·ÚÔ˘Û›·Û ˘ÚÂÙfi ¤ˆ˜ 39,5ÔC ·fi 24ÒÚÔ˘, Ì ڛÁÔ˜, ÎÂÊ·Ï·ÏÁ›· Î·È ·ÈÌÔÚÚ·ÁÈÎfi ÂÍ¿ÓıËÌ· ÛÙËÓ ÂÎÙ·ÙÈ΋ ÂÈÊ¿ÓÂÈ· ÙÔ˘ ‰ÂÍÈÔ‡

82

‚Ú·¯›ÔÓ·. √ ·ÛıÂÓ‹˜ ÌÂٷʤÚÂÙ·È Î·È ¿ÏÈ ÛÙËÓ ∞ı‹Ó·, ·ÊÔ‡ ÚÒÙ· ¯ÔÚËÁ‹ıËΠÂÓ‰ÔÊÏ‚›ˆ˜ ÎÂÊ·ÏÔÛÔÚ›ÓË 3˘ ÁÂÓÈ¿˜. ∫·Ù¿ ÙË 2Ë Â›ÛÔ‰Ô, ÙÔ ·È‰› ›¯Â ηϿ ˙ˆÙÈο ÛËÌ›·, ‹È· ÓˆıÚfiÙËÙ·, ÂÙ¯ÂÈ҉˜ ÂÍ¿ÓıËÌ· ΢ڛˆ˜ ¿ÎÚˆÓ Î·È ÏÔÈ¿ Û˘ÛÙ‹Ì·Ù· ¯ˆÚ›˜ ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·. ∆· Â˘Ú‹Ì·Ù· ·fi ÙÔÓ ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ÙÔ˘ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘ ˘ÁÚÔ‡ ‹Ù·Ó ·ÚÓËÙÈο ÁÈ· ÌËÓÈÁÁ›Ùȉ· (·ÙÙ·Ú·=0 mm3, χΈ̷=22 mgr/dl, ۿί·ÚÔ=67 mgr/dl, ۿί·ÚÔ ·›Ì·ÙÔ˜=106 mgr/dl). ∏ ¿ÌÂÛË Î·Ù¿ Gram ¯ÚÒÛË ‹Ù·Ó ·ÚÓËÙÈ΋ fiˆ˜ Î·È Ë ‰ÔÎÈÌ·Û›· Û˘ÁÎfiÏÏËÛ˘ ÁÈ· ·Ó›¯Ó¢ÛË ÌÈÎÚÔ‚È·ÎÔ‡ ·ÓÙÈÁfiÓÔ˘. √È Î·ÏÏȤÚÁÂȘ ·fi ÙÔ ÚÈÓÔÊ¿Ú˘ÁÁ·, ÙÔ ·›Ì· Î·È ÙÔ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi ‰ÂÓ ·Ó¤Ù˘Í·Ó ÌÈÎÚfi‚ÈÔ. √ ÏÔÈfi˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ: Hb=13,5 gr/dl, Ht=40%, §Â˘Î¿=19.000/ÌL (¶=84%, §=13%), ∆∫∂=15 mm/1Ë h, CRP=85 mgr/dl Î·È PT=17’’. ªÂ ÙËÓ Èı·Ó‹ ‰È¿ÁÓˆÛË Ù˘ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋˜ ÛË„·ÈÌ›·˜ ¯ÔÚËÁ‹ıËΠÂÓÈÎÈÏ›ÓË Î·È ¯ÏˆÚ·ÌÊÂÓÈÎfiÏË ÁÈ· ÔÎÙÒ 24ˆÚ·, ÂÓÒ Û fiÏ· Ù· ¿ÙÔÌ· ÙÔ˘ ¿ÌÂÛÔ˘ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ ¯ÔÚËÁ‹ıËΠ¯ËÌÂÈÔÚÔʇϷÍË Ì ÚÈÊ·ÌÈΛÓË. ∂Âȉ‹ ÔÈ ÂÚÈÙÒÛÂȘ Â·ÓÂÈÏËÌ̤Ó˘ Û˘ÛÙËÌ·ÙÈ΋˜ Ïԛ̈͢ Ì ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ ‰ÂÓ Â›Ó·È Û˘Ó‹ıÂȘ, ¤ÁÈÓ ÏÂÙÔÌÂÚ‹˜ ·ÓÔÛÔÏÔÁÈÎfi˜ Î·È ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ (¶›Ó·Î·˜ 1). √È ·ÈÌÔÏ˘ÙÈÎÔ› Ù›ÙÏÔÈ Ù˘ ÎÏ·ÛÈ΋˜ Î·È Ù˘ ÂÓ·ÏÏ·ÎÙÈ΋˜ Ô‰Ô‡ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ ‚Ú¤ıËÎ·Ó ÌˉÂÓÈÎÔ›, ÁÂÁÔÓfi˜ Ô˘ Â¤‚·Ï ÙÔÓ ·ÓÔÛÔ¯ËÌÈÎfi ÚÔÛ‰ÈÔÚÈÛÌfi ÙˆÓ ·Ú·ÁfiÓÙˆÓ Ù˘ ÙÂÏÈ΋˜ Ô‰Ô‡. ∞fi ÙÔÓ ¤ÏÂÁ¯Ô ·˘Ùfi, ‚Ú¤ıËÎ·Ó Ê˘ÛÈÔÏÔÁÈΤ˜ ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ ÙˆÓ ·Ú·ÁfiÓÙˆÓ C5, C7, C8 Î·È C9, ÂÓÒ Ë Û˘ÁΤÓÙÚˆÛË ÙÔ˘ C6 ‹Ù·Ó ÌfiÏȘ ·ÓȯÓ‡ÛÈÌË. ∫·ÙfiÈÓ ÙÔ‡ÙÔ˘, Â·Ó·Ï‹ÊıËÎ·Ó ÔÈ ·ÈÌÔÏ˘ÙÈÎÔ› Ù›ÙÏÔÈ Ù˘ ÎÏ·ÛÈ΋˜ Î·È Ù˘ ÂÓ·ÏÏ·ÎÙÈ΋˜ Ô‰Ô‡ ÌÂÙ¿ ·fi ÚÔÛı‹ÎË ÎÂηı·Ṳ́ÓÔ˘ C6 ÛÙÔÓ ÔÚfi ÙÔ˘ ·ÛıÂÓÔ‡˜, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·ÔηٿÛÙ·Û‹ ÙÔ˘˜ ÛÙ·


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 83

¶·È‰È·ÙÚÈ΋ 2001;64:81-84

Ê˘ÛÈÔÏÔÁÈο fiÚÈ·. √ ·ÓÔÛÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙˆÓ ÁÔÓ¤ˆÓ Î·È ÙÔ˘ ÂÓfi˜ ·fi Ù· ‰‡Ô ·‰¤ÏÊÈ· ¤‰ÂÈÍ ÙÈ̤˜ ÎÏ¿ÛÌ·ÙÔ˜ C6, ÂÚ› ÙÔ 50% ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ, ÁÂÁÔÓfi˜ Ô˘ ·ÔÙÂÏ› ¤Ó‰ÂÈÍË ÂÙÂÚfi˙˘Á˘ ÌÔÚÊ‹˜ ·ÓÂ¿ÚÎÂÈ·˜ ÙÔ˘ C6. ™ÙÔ ·È‰›, ÚÈÓ ÙËÓ ¤ÍÔ‰fi ÙÔ˘ ·fi ÙÔ ÓÔÛÔÎÔÌ›Ô, ¤ÁÈÓ ÂÌ‚fiÏÈÔ Ó¢ÌÔÓÈfiÎÔÎÎÔ˘, ·ÈÌfiÊÈÏÔ˘ ÈÓÊÏÔ˘¤ÓÙ˙·˜ Ù‡Ô˘ b Î·È ‰È‰‡Ó·ÌÔ ÂÌ‚fiÏÈÔ ÌËÓÈÁÁÈÙȉfiÎÔÎÎˆÓ A+C. ∂›Û˘, Û˘ÛÙ‹ıËΠÛÙÔ˘˜ ‹‰Ë ¢·ÈÛıËÙÔÔÈË̤ÓÔ˘˜ ÁÔÓ›˜ Ë ¿ÌÂÛË È·ÙÚÈ΋ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ·È‰ÈÔ‡ Ì ÙËÓ ÂÌÊ¿ÓÈÛË Û˘Ìو̷ÙÔÏÔÁ›·˜ Û˘Ì‚·Ù‹˜ Ì Ïԛ̈ÍË. ∂Ù¿ Ì‹Ó˜ ·ÚÁfiÙÂÚ· Î·È Û ËÏÈΛ· 11ó ¯ÚfiÓˆÓ, ÙÔ ·È‰› ÁÈ· 3Ë ÊÔÚ¿ ÏfiÁˆ ˘„ËÏÔ‡ ˘ÚÂÙÔ‡, Â̤وÓ, ÎÂÊ·Ï·ÏÁ›·˜ Î·È ÂÙ¯ÂÈÒ‰Ô˘˜ ÂÍ·Óı‹Ì·ÙÔ˜ ÛÙ· οو ¿ÎÚ·, ÌÂٷʤÚÂÙ·È ÛÙËÓ ∞ı‹Ó·. ™ÙÔ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi ‚Ú¤ıËÎ·Ó Î‡ÙÙ·Ú·=874/mm3 (¶=98%, §=2%), §Â‡ÎˆÌ·=67 mgr/dl, ™¿Î¯·ÚÔ=65 mgr/dl (™¿Î¯·ÚÔ ·›Ì·ÙÔ˜=156 mgr/dl), ÂÓÒ ÛÙÔ ¿ÌÂÛÔ ·Ú·Û··ÛÌ· ÙÔ˘ ∂¡À ·Ú·ÙËÚ‹ıËÎ·Ó gram (-) ‰ÈÏfiÎÔÎÎÔÈ. √È Î·ÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜, ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘, ÚÈÓÔÊ¿Ú˘ÁÁ· Î·È ‰ÂÚÌ·ÙÈ΋˜ ‚Ï¿‚˘ ‰ÂÓ ·Ó¤Ù˘Í·Ó ÌÈÎÚfi‚È·. §ÔÈfi˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜: Hb=12,6 g/dl, Ht=36%, §Â˘Î¿=23.000/ÌL (¶=91%, §=3%), ∆∫∂=12 mm/1Ë h, CRP=98 mgr/dl, PT=16,5’’. ∆Ô ·È‰› ·ÓÙÈÌÂÙˆ›ÛÙËΠˆ˜ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋ ÌËÓÈÁÁ›Ùȉ· Ì ÙËÓ ›‰È· ıÂÚ·¢ÙÈ΋ ·ÁˆÁ‹, Ì ¿ÚÈÛÙË ¤Î‚·ÛË. ™˘˙‹ÙËÛË ∏ Û˘¯ÓfiÙËÙ· (8,2) ÙˆÓ ÎÏËÚÔÓÔÌÈÎÒÓ ·ÓÂ·ÚÎÂÈÒÓ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ ÔÈΛÏÏÂÈ ·fi ÙËÓ ÂıÓÈÎfiÙËÙ· ÙÔ˘ ÂÍÂÙ·˙fiÌÂÓÔ˘ ÏËı˘ÛÌÔ‡. ÀÔÏÔÁ›˙ÂÙ·È fiÙÈ Û ÂÚ›Ô˘ 0,03% ÙÔ˘ ∂˘Úˆ·˚ÎÔ‡ ÏËı˘ÛÌÔ‡ ‰È·ÈÛÙÒÓÂÙ·È Î¿ÔÈÔ˘ Ù‡Ô˘ ·ÓÂ¿ÚÎÂÈ· Û˘ÌÏËÚÒÌ·ÙÔ˜. °ÂÓÈο fï˜, ÛÙȘ ÂÚÈÙÒÛÂȘ Â·ÓÂÈÏËÌÌ¤ÓˆÓ ÂÂÈÛÔ‰›ˆÓ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÔ΋˜ Ïԛ̈͢, fiˆ˜ ÛÙÔ ÂÚÈÛÙ·ÙÈÎfi Ì·˜, Ë Û˘¯ÓfiÙËÙ· ·Ó‡ÚÂÛ˘ ·ÓÂ¿ÚÎÂÈ·˜ Û˘ÌÏËÚÒÌ·ÙÔ˜ Êı¿ÓÂÈ Î·È ÙÔ 30%. ∏ ¤ÏÏÂÈ„Ë ÙÔ˘ C6, fiˆ˜ Î·È ÙˆÓ ˘fiÏÔÈˆÓ ÙÂÏÈÎÒÓ ÎÏ·ÛÌ¿ÙˆÓ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜, ÎÏËÚÔÓÔÌÂ›Ù·È Ì ÙÔÓ ·˘ÙfiÛˆÌÔ ˘ÔÏÂÈfiÌÂÓÔ ÙÚfiÔ. √ ¤ÏÂÁ¯Ô˜ Ù˘ ÔÈÎÔÁ¤ÓÂÈ·˜ ÙÔ˘ ·ÛıÂÓÔ‡˜ Ì·˜ ¤‰ÂÈÍ fiÙÈ ÔÈ ÁÔÓ›˜ ÙÔ˘ ‹Ù·Ó ÂÙÂÚfi˙˘ÁÔÈ Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ C6, ·ÊÔ‡ ‚Ú¤ıËÎ·Ó Ó· ¤¯Ô˘Ó ¯·ÌËϤ˜ ÙÈ̤˜ ÎÏ¿ÛÌ·ÙÔ˜ C6 ÛÙÔÓ ÔÚfi ÙÔ˘˜, ÂÓÒ Ù· ·‰¤ÏÊÈ· ÙÔ˘ ›¯·Ó Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜. £· Ú¤ÂÈ Ó· ·Ó·ÊÂÚı› fiÙÈ Ù· ÂÙÂÚfi˙˘Á· ¿ÙÔÌ· ‰ÂÓ ¤¯Ô˘Ó ·˘ÍË̤ÓË Â˘¿ıÂÈ· Û ÏÔÈÌÒÍÂȘ. ∏ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋ ÓfiÛÔ˜ ÛÙ· ¿ÙÔÌ· Ì ·ÓÂ¿ÚÎÂÈ· ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ C5-C6 ·ÚÔ˘ÛÈ¿˙ÂÈ

Paediatriki 2001;64:81-84

ÔÚÈṲ̂Ó˜ ȉȷÈÙÂÚfiÙËÙ˜ Û ۇÁÎÚÈÛË Ì ÙÔ ÁÂÓÈÎfi ÏËı˘ÛÌfi. ŒÙÛÈ, Ë Û˘¯ÓfiÙËÙ· ˘ÔÙÚÔ‹˜ (7,2,1) Ù˘ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋˜ Ïԛ̈͢ ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 40-45% Û ¿ÙÔÌ· Ì ·ÓÂ¿ÚÎÂÈ· ÙˆÓ ÙÂÏÈÎÒÓ ÎÏ·ÛÌ¿ÙˆÓ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜, fiˆ˜ Û˘Ó¤‚Ë Î·È ÛÙÔÓ ·ÛıÂÓ‹ Ì·˜, ÂÓÒ ÛÙ· Ê˘ÛÈÔÏÔÁÈο ¿ÙÔÌ· ‰ÂÓ ÂÌÊ·Ó›˙ÂÙ·È Û¯Â‰fiÓ Ô˘‰ÂÌ›· ˘ÔÙÚÔ‹. ∞˘Ùfi ÂȂ‚·ÈÒÓÂÈ fiÙÈ Ë ·Ú¯È΋ Ïԛ̈ÍË ÛÙ· ¿ÙÔÌ· ·˘Ù¿ ‰ÂÓ Î·Ù·Ï›ÂÈ ·ÓÔÛ›·. ∫¿ı ÂÂÈÛfi‰ÈÔ Ïԛ̈͢ Â›Ó·È ·ÓÂÍ¿ÚÙËÙÔ ÁÂÁÔÓfi˜, Ô˘ ‰ÂÓ ÌÂÈÒÓÂÈ ÙÔÓ Î›Ó‰˘ÓÔ ÁÈ· ÂfiÌÂÓË Ïԛ̈ÍË ·fi ÙÔÓ ›‰ÈÔ Ù‡Ô ÌÈÎÚÔ‚›Ô˘. ∂›Û˘, Ë ıÓËÙfiÙËÙ· ·fi ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋ Ïԛ̈ÍË ÙˆÓ ·ÙfiÌˆÓ Ì ·ÓÂ¿ÚÎÂÈ· ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ Û ۇÁÎÚÈÛË Ì ÙÔ ÁÂÓÈÎfi ÏËı˘ÛÌfi, Â›Ó·È ¯·ÌËÏ‹ (2,3,9). ∏ ıÓËÙfiÙËÙ· Ù˘ ÓfiÛÔ˘ ÛÙ· Ê˘ÛÈÔÏÔÁÈο ¿ÙÔÌ· Î˘Ì·›ÓÂÙ·È ÌÂٷ͇ 10-19%, ÂÓÒ ÛÙ· ¿ÙÔÌ· Ì ·ÓÂ¿ÚÎÂÈ· ÙˆÓ ÙÂÏÈÎÒÓ ÎÏ·ÛÌ¿ÙˆÓ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ Î˘Ì·›ÓÂÙ·È ÌÂٷ͇ 1,5-2,4%. ∆Ô ·È‰› Ì·˜, ·fi ÙÔ ‰Â‡ÙÂÚÔ 24ˆÚÔ ·ÁˆÁ‹˜, ‹Ù·Ó Û ¿ÚÈÛÙË ÁÂÓÈ΋ ηٿÛÙ·ÛË. ∏ ηÏÔ‹ı˘ ¤Î‚·ÛË Û˘Ó‰¤ÂÙ·È Èı·ÓfiÓ Ì ÌÂȈ̤ÓË ·Ú·ÁˆÁ‹ ÂÓ‰ÔÙÔ͛Ӣ (ÏÈÔÔÏ˘Û·Î¯·Ú›Ù˘) ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘, Ë ÔÔ›· Û¯ÂÙ›˙ÂÙ·È ¿ÌÂÛ· Ì ÙËÓ ·ÂÏ¢ı¤ÚˆÛË Î˘ÙÙ·ÚÔÎÈÓÒÓ Î·È ¿ÏÏˆÓ ÌÂÛÔÏ·‚ËÙÒÓ ÊÏÂÁÌÔÓ‹˜, ÔÈ ÔÔ›ÔÈ Â˘ı‡ÓÔÓÙ·È ÁÈ· ÈÛÙÈ΋ ‚Ï¿‚Ë. º·›ÓÂÙ·È, ‰ËÏ·‰‹, fiÙÈ Ù· ¿ÙÔÌ· Ì ·ÓÂ¿ÚÎÂÈ· ÙˆÓ ÙÂÏÈÎÒÓ ÎÏ·ÛÌ¿ÙˆÓ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ (9,3) ‰ÂÓ ÂÌÊ·Ó›˙Ô˘Ó Û˘¯Ó¿ ı·Ó·ÙËÊfiÚÔ ¤Î‚·ÛË. √È ¯·ÌËϤ˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ÂÓ‰ÔÙÔ͛Ӣ ÛÙÔ Ï¿ÛÌ·, Ë ÂÚÈÔÚÈṲ̂ÓË ÈÛÙÈ΋ ‚Ï¿‚Ë Î·È Ô ÌÈÎÚfi˜ ·ÚÈıÌfi˜ ÌÈÎÚÔ‚›ˆÓ ÁÈ· ÚfiÎÏËÛË Ù˘ ÓfiÛÔ˘ ÔÊ›ÏÔÓÙ·È Î·Ù¿ ·ÚÈÔ ÏfiÁÔ ÛÙËÓ ·‰˘Ó·Ì›· Û¯ËÌ·ÙÈÛÌÔ‡ ÙÔ˘ Û˘ÌϤÁÌ·ÙÔ˜ C5-C9. ∂›Û˘, Ë ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋ Ïԛ̈ÍË Û ¿ÙÔÌ· Ì ·˘Ù¤˜ ÙȘ ·ÓÂ¿ÚÎÂȘ ÂÌÊ·Ó›˙ÂÙ·È Û fiϘ ÙȘ ËÏÈ˘, Û˘Ó‹ıˆ˜ fï˜ ÛÙȘ ËÏÈ˘ 14-17 ¯ÚfiÓˆÓ. ¶Èı·Ó‹ ÂÚÌËÓ›· ·˘ÙÔ‡ ÙÔ˘ ÁÂÁÔÓfiÙÔ˜ Â›Ó·È fiÙÈ Ô Ê˘ÛÈÔÏÔÁÈÎfi˜ ÏËı˘ÛÌfi˜ ·ÚÔ˘ÛÈ¿˙ÂÈ Â˘¿ıÂÈ· ÛÙȘ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈΤ˜ ÏÔÈÌÒÍÂȘ ΢ڛˆ˜ ÛÙȘ ËÏÈ˘ ·fi 1 Ì‹Ó· ¤ˆ˜ 4 ¯ÚfiÓˆÓ. ™ÙÔ ‰È¿ÛÙËÌ· ·˘Ùfi, ÙÔ ·ÓÔÛÔÔÈËÙÈÎfi Û‡ÛÙËÌ· ÙÔ˘ ·È‰ÈÔ‡ ÂÎÙ›ıÂÙ·È Û ÌË ·ıÔÁfiÓÔ˘˜ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘˜ Î·È ¿ÏÏ· Gram(-) ‚·ÎÙËÚ›‰È· Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ú·ÁˆÁ‹ ÚÔÛٷ٢ÙÈÎÒÓ ·ÓÙÈۈ̿وÓ. ∆· ¿ÙÔÌ· Ì ·ÓÂ¿ÚÎÂÈ· ÙˆÓ ÙÂÏÈÎÒÓ ÎÏ·ÛÌ¿ÙˆÓ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜ ·Ú¿ÁÔ˘Ó ·ÓÙÈÌËÓÈÁÁÈÙȉÔÎÔÎÎÈο ·ÓÙÈÛÒÌ·Ù· (1,2,3), fiˆ˜ Î·È Ô Ê˘ÛÈÔÏÔÁÈÎfi˜ ÏËı˘ÛÌfi˜. ∂›Ó·È ÂӉȷʤÚÔÓ fiÙÈ Ù· ¿ÙÔÌ· Ì ·ÓÂ¿ÚÎÂȘ ÓÔÛÔ‡Ó ·fi Û¿ÓÈÔ˘˜ ÔÚfiÙ˘Ô˘˜ (3,8) ÌËÓÈÁÁÈÙȉfiÎÔÎΈÓ, fiˆ˜ X,Y,Z 2qE, W135. ¶Èı·Ó‹

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 84

¶·È‰È·ÙÚÈ΋ 2001;64:81-84

ÂÚÌËÓ›· Â›Ó·È Ë ÂÈÎÚ¿ÙËÛË ÙˆÓ ÔÚÔÔÌ¿‰ˆÓ ·˘ÙÒÓ ÛÙ· ÌÂÁ·Ï‡ÙÂÚ· ¿ÙÔÌ·. °È· ·Ú¿‰ÂÈÁÌ·, Ë Û˘¯ÓfiÙËÙ· Ù˘ ÊÔÚ›·˜ ÁÈ· Ù· ÛÙÂϤ¯Ë Ù˘ ÔÚÔÔÌ¿‰·˜ À ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ ·˘Í¿ÓÂÙ·È Ì ÙËÓ ËÏÈΛ· Î·È ·ÊÔ‡ Ë ÚÒÙË Ïԛ̈ÍË ÛÙ· ¿ÙÔÌ· ·˘Ù¿ ·ÚÔ˘ÛÈ¿˙ÂÙ·È Û ÌÂÁ¿ÏË ËÏÈΛ·, ·ÔÙÂÏ› ÙË Û˘¯ÓfiÙÂÚË ÔÚÔÔÌ¿‰· Ô˘ ÚÔηÏ› ÙËÓ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋ Ïԛ̈ÍË. ∏ ÌË ·Ó¿Ù˘ÍË ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ ÛÙȘ ηÏÏȤÚÁÂȘ ÙÔ˘ ·ÛıÂÓÔ‡˜, Èı·Ófiٷٷ ÏfiÁˆ Ù˘ ¯ÔÚ‹ÁËÛ˘ ·ÓÙÈ‚›ˆÛ˘, ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· Ó· ÌËÓ Î·ıÔÚÈÛÙ› Ô ÔÚfiÙ˘Ô˜. ∏ ˘ÔÙÚÔ‹ (1) ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋˜ Ïԛ̈͢ ÂÈ‚¿ÏÏÂÈ ÙÔÓ ¤ÏÂÁ¯Ô Ù˘ ·Ï˘Û›‰·˜ ÙˆÓ ÎÏ·ÛÌ¿ÙˆÓ ÙÔ˘ Û˘ÌÏËÚÒÌ·ÙÔ˜. ™ÙËÓ ÂÚ›ÙˆÛË Ô˘ ‰È·ÈÛÙˆı› Ë ·ÓÂ¿ÚÎÂÈ·, Ô ÛËÌ·ÓÙÈÎfiÙÂÚÔ˜ ÙÚfiÔ˜ ÚÔÛÙ·Û›·˜ ÙÔ˘ ·ÙfiÌÔ˘ Â›Ó·È Ë Â˘·ÈÛıËÙÔÔ›ËÛË ÛÙÔ Úfi‚ÏËÌ· ÙÔ˘ ›‰ÈÔ˘ ÙÔ˘ ·ÛıÂÓÔ‡˜ Î·È ÙÔ˘ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ ÂÚÈ‚¿ÏÏÔÓÙÔ˜. ∂ÓÙÔ‡ÙÔȘ, ÚÔÙ›ÓÔÓÙ·È ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ÎÈ ¿ÏÏ· ̤ÙÚ·, fiˆ˜ Ô ÂÌ‚ÔÏÈ·ÛÌfi˜ (10) ÙÔ˘ ·ÛıÂÓÔ‡˜ Ì ÙÂÙÚ·‰‡Ó·ÌÔ ÂÌ‚fiÏÈÔ ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ (A,C,Y,W135) ·ÏÏ¿ Î·È Ù˘ ÔÈÎÔÁ¤ÓÂÈ¿˜ ÙÔ˘. £· Ú¤ÂÈ Ó· ÛËÌÂȈı› fiÙÈ Ì¤¯ÚÈ Û‹ÌÂÚ· ‰ÂÓ ˘¿Ú¯Ô˘Ó Â·ÚΛ˜ ÌÂϤÙ˜ ÁÈ· ÙËÓ ÎÏÈÓÈ΋ ·Í›· ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ¤Ó·ÓÙÈ ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ Û ¿ÙÔÌ· Ì ·ÓÂ¿ÚÎÂȘ Û˘ÌÏËÚÒÌ·ÙÔ˜. ¶Èı·ÓfiÓ Ù· ¿ÙÔÌ· ·˘Ù¿ Ó· ‚ÔËıËıÔ‡Ó Ì ÙÔÓ ÂÌ‚ÔÏÈ·ÛÌfi, ÁÈ·Ù› Ù· ÔÏ˘Û·Î¯·ÚȉÈο ·ÓÙÈÛÒÌ·Ù· ‰ÈÂÁ›ÚÔ˘Ó ÙÔ Ì˯·ÓÈÛÌfi Ù˘ Ô„ˆÓÔÊ·ÁÔ΢ÙÙ¿ÚˆÛ˘ Ô˘ ·Ú·Ì¤ÓÂÈ Ô ÌÔÓ·‰ÈÎfi˜ Ì˯·ÓÈÛÌfi˜ ¿Ì˘Ó·˜. √ ÂÌ‚ÔÏÈ·ÛÌfi˜ Ì ÙÔ ÂÌ‚fiÏÈÔ ÙÔ˘ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ ¿ÓÙˆ˜, ‰ÂÓ ·ÔÎÏ›ÂÈ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋ Ïԛ̈ÍË. ∏ Ì·ÎÚfi¯ÚÔÓË ¯ÔÚ‹ÁËÛË ·ÓÙÈ‚›ˆÛ˘ ÁÈ· ÚÔʇϷÍË (1,2,11) ·Ú·Ì¤ÓÂÈ ·ÌÊÈÏÂÁfiÌÂÓË. ™Â ÂÚÈÙÒÛÂȘ Ô˘ ¤¯ÂÈ ‰Ôı›, ηıÔÚÈÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Â›Ó·È Ë ÂÓ‰ËÌÈÎfiÙËÙ· Ù˘ Ïԛ̈͢ ÛÙËÓ ÂÚÈÔ¯‹ fiˆ˜ Î·È Ë ·ÓÙÔ¯‹ ÌËÓÈÁÁÈÙȉfiÎÔÎÎÔ˘ ÛÙËÓ ÂÓÈÎÈÏ›ÓË. ∞fi ÔÚÈṲ̂ÓÔ˘˜ ÂÚ¢ÓËÙ¤˜ ˘ÔÛÙËÚ›˙ÂÙ·È fiÙÈ Ë ¯ÔÚ‹ÁËÛË ¯ËÌÂÈÔÚÔʇϷ͢, Ë ‰È¿ÚÎÂÈ· Ù˘ ÔÔ›·˜ ‰ÂÓ Î·ıÔÚ›˙ÂÙ·È, Èı·ÓfiÓ Ó· ÌÂÈÒÓÂÈ ÙÔÓ ·ÚÈıÌfi ÙˆÓ ˘ÔÙÚÔÒÓ. ∆Ô ÁÂÁÔÓfi˜ fiÙÈ Û ̛· ÎÏÈÓÈ΋ Ô˘ ÓÔÛËχÂÈ ÌÂÁ¿ÏÔ ·ÚÈıÌfi ÌÈÎÚÔ‚È·ÎÒÓ ÌËÓÈÁÁ›ÙȉˆÓ ÂÚÈÁÚ¿ÊÂÙ·È ÁÈ· ÚÒÙË ÊÔÚ¿ ˘ÔÙÚÔÈ¿˙Ô˘Û· ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋ Ïԛ̈ÍË, ˘Ô‰ËÏÒÓÂÈ ÙË Û·ÓÈfiÙËÙ· ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜. Ÿˆ˜ ÚÔ·ÙÂÈ Î·È ·fi ÙË ‚È‚ÏÈÔÁÚ·Ê›·, Ë Û·Ê‹˜ Û˘Û¯¤ÙÈÛË Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ ÙˆÓ ÙÂÏÈÎÒÓ ÎÏ·ÛÌ¿ÙˆÓ Ì ˘ÔÙÚÔÈ¿˙Ô˘Û˜, ÛÔ‚·Ú¤˜ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈΤ˜ ÏÔÈÌÒÍÂȘ, ÂÈ‚¿ÏÏÂÈ ÙËÓ ·Ó·˙‹ÙËÛË Ù˘ ·ÓÂ¿ÚÎÂÈ·˜ ·˘Ù‹˜ Û ·ÛıÂÓ›˜ Ì ÌË ÂȉËÌÈ΋ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈ΋ ÌËÓÈÁÁ›ÙÈ-

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‰· ‹ Û ·ÛıÂÓ›˜ Ì ˘ÔÙÚÔÈ¿˙Ô˘Û˜ ÌËÓÈÁÁÈÙȉÔÎÔÎÎÈΤ˜ ÏÔÈÌÒÍÂȘ. µÈ‚ÏÈÔÁÚ·Ê›· 1. Ross SC, Densen P. Complement deficiency states and infection: Epidemiology, pathogenesis and consequences of Neisseria and other infections in an immune deficiency. Medicine 1984;63:243-267. 2. Figueroa JE, Densen P. Infectious diseases associated with complement deficiencies. Cl Microbiol Rev 1991: 359-395. 3. Figueroa JE, Andreoni J, Densen P. Complement deficiency states and meningococcal disease. Immunol Res 1993;12:295-311. 4. Richard B, Johnston JR. The complement system in host defense and inflammation: the cutting edges of a double edged sword. Pediatr Inf Dis J 1993;12:933-941. 5. Pallares D, Figueroa E, Julio E, Densen R, Marchall GS. Invasive Haemophilus influenzae type b infection in a child with familial deficiency of the beta subunit of the eighth component of complement. J Pediatr 1996;128:102-103. 6. Gelfand Erwin W, Pandu RC, Minta JD, Purkall DB, Shuan R. Inherited deficiency of properdin and C2 in a patient with recurrent bacteremia. Am J Med 1987;82:671-675. 7. Vogler LB, Johnston RB. Recurrent meningococcal meningitis with absence of the sixth component of complement: An evaluation of underlying immunologic mechanisms. Pediatrics 1979;64:465-467. 8. Fijen Cees AP, Kuijper EJ, van Putten Jos PM. Complement deficiencies in patients over ten years old with meningococcal disease due to uncommon serogroups. Lancet 1989;2:585-588. 9. Derkx HHF, Kuijper EJ, Fijen CAP, Dankert J. Inherited complement deficiency in children surviving fulminant meningococcal septic shock. Eur J Paediatr 1995;154: 735-738. 10. Andreoni J, Kayhty H, Densen P. Vaccination and the role of capsular polysaccharide antibody in prevention of recurrent meningococcal disease in late complement component deficient individuals. J Infect Dis 1993;168: 227-231. 11. Potter PC, Frasch CE, Van der Sande WJM. Profylaxis against Neisseria meningitidis infections and antibody responses in patients with deficiency of the sixth component of complement. J Infect Dis 1990;161:932-937. 12. Platonov AE, Vershinina IV, Kayhty H. Vaccination of patients deficient in a late complement component with tetravalent meningococcal capsular polysaccharide vaccine. Clin Exp Immunol 1995;100:32-39.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 17-07-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 27-11-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ª·Ú›· £ÂÔ‰ˆÚ›‰Ô˘ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ “∞Á›· ™ÔÊ›·” ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 85

¶·È‰È·ÙÚÈ΋ 2001;64:85-86

∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

Paediatriki 2001;64:85-86

CASE REPORT

¶Ó¢ÌÔÓÈÔÎÔÎÎÈ΋ ÌËÓÈÁÁ›Ùȉ· Ì ·Ú¯ÈÎÒ˜ Ê˘ÛÈÔÏÔÁÈÎfi ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi Û ÌË ·ÓÔÛÔηٷÛÙ·Ï̤ÓÔ ¿ÚÚˆÛÙÔ ∞. ÷Ù˙Ë·Ó·Á‹˜, ª. ™È‰ÂÚ¿

Streptococcus pneumoniae meningitis with initial normal cerebrospinal fluid findings in a nonimmunocompromised patient A. Hadjipanayis, M. Sidera

¶ÂÚ›ÏË„Ë: ¶ÂÚÈÁÚ¿ÊÂÙ·È ÂÚ›ÙˆÛË Ó¢ÌÔÓÈÔÎÔÎÎÈ΋˜ ÌËÓÈÁÁ›Ùȉ·˜, Ë ‰È¿ÁÓˆÛË Ù˘ ÔÔ›·˜ ηı˘ÛÙ¤ÚËÛÂ, ÁÈ·Ù› Ù· Â˘Ú‹Ì·Ù· Ù˘ ·Ú¯È΋˜ ÔÛÊ˘ÔÓˆÙÈ·›·˜ ·Ú·Î¤ÓÙËÛ˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. ∆Ô ·È‰› ÂÈÛ‹¯ıË ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ÁÈ·Ù› ·ÚÔ˘Û›·Û ˘ÚÂÙÈÎÔ‡˜ Û·ÛÌÔ‡˜. ∆· Â˘Ú‹Ì·Ù· Ù˘ ÚÒÙ˘ ÔÛÊ˘ÔÓˆÙÈ·›·˜ ·Ú·Î¤ÓÙËÛ˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο, ÂÓÒ Ë ‰Â‡ÙÂÚË ¤‰ÂÈÍ 1350 ÏÂ˘Î¿ ·ÈÌÔÛÊ·›ÚÈ·/mm3. √È Î·ÏÏȤÚÁÂȘ ÙÔ˘ ÚÒÙÔ˘ Î·È ‰Â‡ÙÂÚÔ˘ ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô˘ ˘ÁÚÔ‡, ηıÒ˜ Â›Û˘ Î·È ÔÈ Î·ÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜, ·Ó¤Ù˘Í·Ó Ó¢ÌÔÓÈfiÎÔÎÎÔ. ∆Ô Ê˘ÛÈÔÏÔÁÈÎfi ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi ‰ÂÓ ·ÔÎÏ›ÂÈ ÙËÓ Èı·ÓfiÙËÙ· ÌËÓÈÁÁ›Ùȉ·˜.

Abstract: A case of Streptococcus pneumoniae meningitis is reported in a child in whom the diagnosis was delayed because of an initially normal cerebrospinal fluid analysis. The child was admitted to the hospital because of febrile seizures. The initial lumbar puncture revealed normal cerebrospinal fluid findings but a second one, performed 24h later, showed 1350 white blood cells/mm3. Streptococcus pneumoniae was cultured from the CSF obtained from the initial and the second lumbar puncture as well as from blood cultures. Normal cerebrospinal fluid findings do not always exclude the possibility of meningitis.

§¤ÍÂȘ ÎÏÂȉȿ: Ó¢ÌÔÓÈÔÎÔÎÎÈ΋ ÌËÓÈÁÁ›Ùȉ·, ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi.

Key words: Streptococcus pneumoniae meningitis, cerebrospinal fluid.

∂ÈÛ·ÁˆÁ‹ ∏ ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙËÛË (√¡¶) Â›Ó·È Ë ·Ó·Áη›· ÂͤٷÛË ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù˘ ÌËÓÈÁÁ›Ùȉ·˜ Û ÔÔÈÔÓ‰‹ÔÙ ¿ÚÚˆÛÙÔ ˘¿Ú¯ÂÈ ˘Ô„›· ÁÈ· ÙË ÓfiÛÔ ·˘Ù‹. ∂ÓÙÔ‡ÙÔȘ, Ê˘ÛÈÔÏÔÁÈÎfi ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi (∂¡À) ‰ÂÓ ·ÔÎÏ›ÂÈ ÙË ‰È¿ÁÓˆÛË Ù˘ ÌËÓÈÁÁ›Ùȉ·˜ ·fi ÙË ÛÙÈÁÌ‹ Ô˘ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› ·ÚÎÂÙ¿ ÂÚÈÛÙ·ÙÈο ÌËÓÈÁÁ›Ùȉ·˜ ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ∂¡À (1-4). ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ∫ÔÚ›ÙÛÈ ËÏÈΛ·˜ ‰‡Ô ¯ÚfiÓˆÓ ÚÔÛ‹Ïı ÛÙÔ ÓÔÛÔÎÔÌÂ›Ô ÁÈ·Ù› ·ÚÔ˘Û›·Û ˘ÚÂÙÈÎÔ‡˜ Û·ÛÌÔ‡˜ ‰È¿ÚÎÂÈ·˜ 10-15 ÏÂÙÒÓ. ™ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË Î·Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘ ‰È·ÈÛÙÒıËΠfiÙÈ ÂÚfiÎÂÈÙÔ ÁÈ· ÓˆıÚfi ·È‰›, ¯ˆÚ›˜ fï˜ Ù›ÔÙ· ÙÔ ·ıÔ-

ÏÔÁÈÎfi ·fi ÙËÓ Î·Ù¿ Û˘ÛÙ‹Ì·Ù· ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË. ™ÙÔ ·ÙÔÌÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È ·ÎfiÌ· ¤Ó· ÂÂÈÛfi‰ÈÔ ˘ÚÂÙÈÎÒÓ Û·ÛÌÒÓ ÚÈÓ ¤Ó· ¯ÚfiÓÔ. ∆· ·ÔÙÂϤÛÌ·Ù· ÙÔ˘ ÂÚÁ·ÛÙËÚÈ·ÎÔ‡ ÂϤÁ¯Ô˘ ηٿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘ Â›Ó·È Ù· ·ÎfiÏÔ˘ı·: §Â˘Î¿=10.000/mm3 /73 Ï/19 Ì/8, HB=10,4 gr%, ∞ÈÌÔÂÙ¿ÏÈ·=403.000/mm3, °Ï˘Îfi˙Ë=201 mg%, √˘Ú›·=24 mg%, ∫Ú·ÙÈÓ›ÓË=0,4 mg%, Ca=8,9 mg%, P=3,4 mg%, Na=132 mEq/L, K=5,5 mEq/L, Cl=106 mEq/L. ∏ √¡¶ ¤‰ÂÈÍ ‰È·˘Á¤˜ ∂¡À Ì ηӤӷ ÂÚ˘ıÚfi ·ÈÌÔÛÊ·›ÚÈÔ, 5 ÏÂ˘Î¿/mm3, ÁÏ˘Îfi˙Ë=92 mg/dl, Î·È ÚˆÙ½ÓË=16 mg/dl. ŸÏÔ˜ Ô ÏËı˘ÛÌfi˜ ÙˆÓ Ï¢ÎÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ ÛÙÔ ∂¡À ‹Ù·Ó ÏÂÌÊÔ΢ÙÙ·ÚÈÎfi˜. ¶·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ‰ÂÓ ‚Ú¤ıËΠÂÛÙ›· Ïԛ̈͢, ¯ÔÚËÁ‹ıËΠÛÙÔ ·È‰› ÎÂÊoÙ·Í›ÌË 200 mg/kg, ÂÚÈ̤ÓÔÓÙ·˜ Ù· ·ÔÙÂϤÛÌ·Ù·

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋, ª·Î¿ÚÈÔ ¡ÔÛÔÎÔÌÂ›Ô §¿Úӷη˜, §¿Úӷη, K‡ÚÔ˜

Pediatric Clinic, Makario Hospital of Larnaka Larnaka, Cyprus

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·fi ÙȘ ηÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜ Î·È ∂¡À. ∂ÈÎÔÛÈÙ¤ÛÛÂÚȘ ÒÚ˜ ·fi ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙÔ˘, ÙÔ ·È‰› ¿Ú¯ÈÛ ӷ οÓÂÈ ÂÌÂÙÔ‡˜ Î·È ÛÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËΠ·˘¯ÂÓÈ΋ ‰˘Ûη̄›·. ∏ ηÈÓÔ‡ÚÁÈ· √¡¶ ¤‰ÂÈÍ 1350 ÏÂ˘Î¿/mm 3 (/95%, Ï/5%), ÁÏ˘Îfi˙Ë=59 mg/dl Î·È ÚˆÙ½ÓË=45 mg/dl. √ Ó¤Ô˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ¤‰ÂÈÍÂ: §Â˘Î¿= 23100/mm3 /85 Ï/7 Ì/7, HB=10,7 gr%, ∞ÈÌÔÂÙ¿ÏÈ·=259.000/mm 3, ∆∫∂=72 mm/1h, °Ï˘Îfi˙Ë= 125 mg%, √˘Ú›·=13 mg%, ∫Ú·ÙÈÓ›ÓË=0,4 mg%, Na=136 mEq/L, K=3,7 mEq/L, Cl=102 mEq/L. ∏ ÎÂÊÔÙ·Í›ÌË ‰È·ÎfiËÎÂ Î·È ¯ÔÚËÁ‹ıËΠÛÙÔ ·È‰› ÂÓÈÎÈÏ›ÓË-¯ÏˆÚ·ÌÊÂÓÈÎfiÏË. √È Î·ÏÏȤÚÁÂȘ ÙÔ˘ ÚÒÙÔ˘ Î·È ‰Â‡ÙÂÚÔ˘ ∂¡À, ηıÒ˜ Î·È ÔÈ Î·ÏÏȤÚÁÂȘ ·›Ì·ÙÔ˜, ·Ó¤Ù˘Í·Ó Ó¢ÌÔÓÈfiÎÔÎÎÔ Ô˘ ‹Ù·Ó ¢·›ÛıËÙÔ˜ ÛÙËÓ ÂÓÈÎÈÏ›ÓË, ÎÂÊÔÙ·Í›ÌË Î·È ¯ÏˆÚ·ÌÊÂÓÈÎfiÏË. ªfiÏȘ ‹Ú·Ì ٷ ·ÔÙÂϤÛÌ·Ù· Ù˘ ηÏÏȤÚÁÂÈ·˜ ÙÔ˘ ‰Â‡ÙÂÚÔ˘ ∂¡À, ‰ÈÂÎfiË Ë ¯ÔÚ‹ÁËÛË Ù˘ ¯ÏˆÚ·ÌÊÂÓÈÎfiÏ˘. ∏ ‰È·ÎÔ‹ Ù˘ ÎÂÊÔÙ·Í›Ì˘ ‰ÂÓ ‹Ù·Ó ÔÚı‹, ÁÈ·Ù› ·ÎfiÌ· ÎÈ ·Ó Ô Ó¢ÌÔÓÈfiÎÔÎÎÔ˜ ‹Ù·Ó ·ÓıÂÎÙÈÎfi˜ ÛÙËÓ ÎÂÊÔÙ·Í›ÌË ÙÔ ·ÓÙÈ‚ÈÔÙÈÎfi Ô˘ ı· ¤ÚÂ ӷ ¯ÚËÛÈÌÔÔÈËı› ‹Ù·Ó Ë ‚·ÓÎÔÌ˘Î›ÓË. √ ¤ÏÂÁ¯Ô˜ Ù˘ ΢ÙÙ·ÚÈ΋˜ Î·È ¯˘ÌÈ΋˜ ·ÓÔÛ›·˜ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎfi˜. ™˘˙‹ÙËÛË ∏ ‰È¿ÁÓˆÛË Ù˘ ÌËÓÈÁÁ›Ùȉ·˜ Ù›ıÂÙ·È Ì ÙËÓ ÂͤٷÛË ÙÔ˘ ∂¡À Û οı ·È‰› Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÛË„·ÈÌ›·˜ ‹ ·Ó Ë ·ÓÙÈÎÂÈÌÂÓÈ΋ Âͤٷۋ ÙÔ˘ ·Ó·‰ÂÈÎÓ‡ÂÈ ÛËÌ›· Ïԛ̈͢ ÙÔ˘ ∫ÂÓÙÚÈÎÔ‡ ¡Â˘ÚÈÎÔ‡ ™˘ÛÙ‹Ì·ÙÔ˜. ∂ÓÙÔ‡ÙÔȘ, Ê˘ÛÈÔÏÔÁÈÎfi ∂¡À ‰ÂÓ ·ÔÎÏ›ÂÈ ÙË ‰È¿ÁÓˆÛË Ù˘ ÌËÓÈÁÁ›Ùȉ·˜ (1-4). À¿Ú¯Ô˘Ó ‰È¿ÊÔÚ˜ ˘Ôı¤ÛÂȘ Ô˘ ÚÔÛ·ıÔ‡Ó Ó· ÂÍËÁ‹ÛÔ˘Ó ÙÔ Ê·ÈÓfiÌÂÓÔ ·˘Ùfi. ∏ ÚÒÙË ÂÈÛËÁÂ›Ù·È ÙËÓ Èı·ÓfiÙËÙ· Ë √¡¶ Ó· ¤¯ÂÈ Á›ÓÂÈ ÛÙ· ·Ú¯Èο ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘ Î·È Ó· ÌËÓ ¤¯ÂÈ ‰Ôı› ¯ÚfiÓÔ˜ ÁÈ· Ï‹ÚË Î˘ÙÙ·ÚÈ΋ ·ÓÙ·fiÎÚÈÛË. ™ÙÔ ÂÚÈÛÙ·ÙÈÎfi Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È Ë √¡¶ ¤ÁÈÓ ٤ÛÛÂÚȘ ÒÚ˜ ·fi ÙËÓ ¤Ó·ÚÍË ÙÔ˘ ˘ÚÂÙÔ‡. ªÂϤÙ˜ ¤¯Ô˘Ó ·ԉ›ÍÂÈ fiÙÈ Ô Ì¤ÛÔ˜ ¯ÚfiÓÔ˜ Ù˘ √¡¶ ·fi ÙËÓ ¤Ó·ÚÍË ÙÔ˘ ˘ÚÂÙÔ‡ Â›Ó·È 6-24 ÒÚ˜ ÛÙ· ·È‰È¿ ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ∂¡À Î·È ÌËÓÈÁÁ›Ùȉ·, ÂÓÒ ÛÙ· ·È‰È¿ Ì ·ıÔÏÔÁÈÎfi ∂¡À Ô Ì¤ÛÔ˜ ¯ÚfiÓÔ˜ Â›Ó·È 43 ÒÚ˜ (1,2). ∆· ·ÓÔÛÔηٷÛÙ·Ï̤ӷ ¿ÙÔÌ· ÌÔÚ› Ó· ¤¯Ô˘Ó ÌËÓÈÁÁ›Ùȉ· ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ∂¡À. √ ¿ÚÚˆÛÙÔ˜ Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È ¤¯ÂÈ

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Ê˘ÛÈÔÏÔÁÈ΋ ¯˘ÌÈ΋ Î·È Î˘ÙÙ·ÚÈ΋ ·ÓÔÛ›·. ∏ ÙÚ›ÙË ˘fiıÂÛË ÂÈÛËÁÂ›Ù·È ÙËÓ Èı·ÓfiÙËÙ· Ë ›‰È· Ë √¡¶ Ó· ¤¯ÂÈ ÚÔηϤÛÂÈ ÌËÓÈÁÁ›Ùȉ· Û ·ÚÚÒÛÙÔ˘˜ Ì ÛË„·ÈÌ›· (5). ™Â ÂÚ›ÙˆÛË ‰ËÏ·‰‹ Ô˘ Ë √¡¶ Â›Ó·È ÙÚ·˘Ì·ÙÈ΋ Û ¿ÚÚˆÛÙÔ Ì ÛË„·ÈÌ›·, ÌÔÚ› Ó· ÌÂÙ·ÊÂÚıÔ‡Ó ÌÈÎÚfi‚È· Ì ÙÔ ·›Ì· ÛÙÔ ∂¡À. ∏ √¡¶ fï˜, Ô˘ ¤ÁÈÓ ÛÙÔ ¿ÚÚˆÛÙÔ ·È‰› ‰ÂÓ ‹Ù·Ó ÙÚ·˘Ì·ÙÈ΋ Î·È Î·Ó¤Ó· ÂÚ˘ıÚfi ·ÈÌÔÛÊ·›ÚÈÔ ‰ÂÓ ‚Ú¤ıËΠÛÙË ÌÈÎÚÔÛÎÔÈ΋ ÂͤٷÛË ÙÔ˘ ∂¡À. ÃÚÒÛË Î·Ù¿ Gram ‰˘ÛÙ˘¯Ò˜ ‰ÂÓ Á›ÓÂÙ·È ÛÙÔ Ê˘ÛÈÔÏÔÁÈÎfi ∂¡À. ∏ ÁÓÒÛË fï˜ fiÙÈ ˘¿Ú¯ÂÈ ÌËÓÈÁÁ›Ùȉ· ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi ∂¡À ÂÈ‚¿ÏÏÂÈ ÙËÓ ·Ó¿ÁÎË Ó· Á›ÓÂÙ·È ¯ÚÒÛË Î·Ù¿ Gram Û οı ∂¡À, ȉȷ›ÙÂÚ· ÛÙ· ·È‰È¿ Ì Û·ÛÌÔ‡˜. ∞˘Ù‹ Ë ·Ï‹ ÂͤٷÛË ÛÂ Û˘Ó‰˘·ÛÌfi Ì ηÈÓÔ‡ÚÁȘ Ù¯ÓÈΤ˜ ·Ó›¯Ó¢Û˘ ·ÓÙÈÁfiÓˆÓ ÙˆÓ ÌÈÎÚÔ‚›ˆÓ ı· ·˘Í‹ÛÔ˘Ó ÙËÓ ÈηÓfiÙËÙ· ‰È¿ÁÓˆÛ˘ ·Ù‡ˆÓ ÂÚÈÛÙ·ÙÈÎÒÓ ÌËÓÈÁÁ›Ùȉ·˜. √ ÎÏÈÓÈÎfi˜ ÁÈ·ÙÚfi˜ ‰ÂÓ Ú¤ÂÈ Ó· ‚·Û›˙ÂÙ·È ÌfiÓÔ ÛÙ· ·ÔÙÂϤÛÌ·Ù· ÙÔ˘ ∂¡À ÁÈ· ÙËÓ ¤Ó·ÚÍË ‹ fi¯È ·ÓÙÈÌÈÎÚԂȷ΋˜ ıÂÚ·›·˜, ·ÏÏ¿ ÈÛ¯˘Úfi ÎÚÈÙ‹ÚÈÔ ·ÔÙÂÏ› ¿ÓÙÔÓÙÂ Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ ·ÚÚÒÛÙÔ˘. ∂›Û˘, Â›Ó·È ¯Ú‹ÛÈÌÔ Ó· Á›ÓÂÙ·È ‰Â‡ÙÂÚË √¡¶, fiÙ·Ó Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· Â›Ó·È ‡ÔÙË ÌËÓÈÁÁ›Ùȉ·˜, ·Ú¿ ÙÔ Ê˘ÛÈÔÏÔÁÈÎfi ∂¡À ÛÙËÓ ÚÒÙË √¡¶. µÈ‚ÏÈÔÁÚ·Ê›· 1. Uchihara T, Ichikawa K, Yoshida S, Tsukagoshi H. Positive culture from normal CSF of Streptococcus pneumoniae meningitis. Eur Neurol 1996;36:234. 2. Rosenthal J, Golan A, Dagan R. Bacterial meningitis with initial normal cerebrospinal fluid findings. Isr J Med Sci 1989;25:186-188. 3. Coll MT, Uriz MS, Pineda V, Fontanals D, Bella F, Nava JM et al. Meningococcal meningitis with "normal": cerebrospinal fluid. J Infect 1994;29:289-294. 4. Polk B, Steele R. Bacterial meningitis presenting with normal cerebrospinal fluid. Pediatr Infect Dis J 1987;6:1040-1042. 5. Fischer GW, Brenz RW, ∞lden ER, Beckwith JB. Lumbar puncture and meningitis. Am J Dis Child 1975;129:590-592.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 06-06-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 06-12-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ∞‰¿ÌÔ˜ ÷Ù˙Ë·Ó·Á‹˜ 25˘ ª·ÚÙ›Ô˘ 6, ¢ÂÚ‡ÓÂÈ·, 5380, ∫‡ÚÔ˜


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 87

¶·È‰È·ÙÚÈ΋ 2001;64:87-90

∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏

Paediatriki 2001;64:87-90

CASE REPORT

∫·ÏÔ‹ı˘ ·ÚÔ‰È΋ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›· Û ·ÁfiÚÈ 16 ÌËÓÒÓ ¡. °Î·Ì›Ï˘1, °. ∆ÛfiÏ·˜2

Benign transient hyperphosphatasaemia in a 16-month old boy N. Gamilis1, G. Tsolas2

¶ÂÚ›ÏË„Ë: ¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ÂÓfi˜ ÎÏÈÓÈο ˘ÁÈÔ‡˜ ÓË›Ô˘, ËÏÈΛ·˜ 16 ÌËÓÒÓ, Ì ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË ÙˆÓ ÂÈ¤‰ˆÓ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡, ˆ˜ Ù˘¯·›Ô˘ Î·È ÌÔÓ·‰ÈÎÔ‡ Â˘Ú‹Ì·ÙÔ˜ ÛÙ· Ï·›ÛÈ· ÂÚÁ·ÛÙËÚÈ·ÎÔ‡ ÂϤÁ¯Ô˘ ÚÔ˘Ù›Ó·˜. ∏ ÙÈÌ‹ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡, Ë ÔÔ›· ¤Êı·Û ÛÙȘ 3.568 ÌÔÓ¿‰Â˜/L, Â·Ó‹Ïı ÛÂ Ê˘ÛÈÔÏÔÁÈο Â›‰· ÌÂÙ¿ ·fi ¤ÍÈ Ì‹Ó˜, ¯ˆÚ›˜ η̛· ·Ú¤Ì‚·ÛË. ∂ÎÙÂÓ‹˜ ÎÏÈÓÈÎfi˜ Î·È ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ ·¤‚Ë ·ÚÓËÙÈÎfi˜ ÁÈ· ÔÛÙÈ΋, Ë·ÙÈ΋ ‹ ¿ÏÏË ÓfiÛÔ Ë ÔÔ›· Ó· ‰‡Ó·Ù·È Ó· ÂÚÌËÓ‡ÛÂÈ ÙËÓ ÙfiÛÔ ÌÂÁ¿ÏË ·‡ÍËÛË Ù˘ ÙÈÌ‹˜ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘. ªÂ ·ÊÔÚÌ‹ ÙËÓ ÂÚ›ÙˆÛË ·˘Ù‹, ·Ó·Ï‡ÔÓÙ·È Ë Èı·Ó‹ ·ÈÙÈÔÏÔÁ›· Î·È Ë ·ıÔÁ¤ÓÂÈ· Ù˘ ÂӉȷʤÚÔ˘Û·˜ ·˘Ù‹˜ ÔÓÙfiÙËÙ·˜.

Abstract: A previously well 16-month-old boy is presented who on routine examination was found to have marked, transient hyperphosphatasaemia of infancy and early childhood. The diagnosis was based on the finding of increased activity of serum alkaline phosphatase, more than fivefold the upper reference limit (3568 u/l), without evidence of liver and bone disease, and also by its subsequent return to normal levels within 6 months after diagnosis. The etiology of the condition and the possible mechanisms of the enzyme increase are discussed.

§¤ÍÂȘ ÎÏÂȉȿ: ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË, ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·, ·ÚÔ‰È΋ ·‡ÍËÛË Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘, ÈÛÔ¤Ó˙˘Ì· Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘.

Key words: alkaline phosphatase, hyperphosphatasaemia, transient elevation of alkaline phosphatase, isoenzymes of alkaline phosphatase.

∂ÈÛ·ÁˆÁ‹ ∏ ·ÚÔ‰È΋ ÂÎÛÂÛËÌ·Ṳ̂ÓË ·‡ÍËÛË Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÙÔ˘ ÔÚÔ‡ ηٿ ÙË ‚ÚÂÊÈ΋ Î·È ÓËȷ΋ ËÏÈΛ·, ·ÁÓÒÛÙÔ˘ ·ÈÙÈÔÏÔÁ›·˜, ›¯Â ÂÚÈÁÚ·Ê› ÁÈ· ÚÒÙË ÊÔÚ¿, ÚÈÓ ·fi ÌÈÛfi ÂÚ›Ô˘ ·ÈÒÓ·, ·fi ÙÔÓ Bach (1). H ηÏÔ‹ı˘ ·˘Ù‹ ηٿÛÙ·ÛË, ·ÔÙÂÏ› ηٿ ηÓfiÓ· Ù˘¯·›Ô ‡ÚËÌ·. ¶·Ú·ÙËÚÂ›Ù·È Î·Ù¿ ÙË ‚ÚÂÊÈ΋ Î·È ÓËȷ΋ ËÏÈΛ· Ì ·ÎÚ·›· ËÏÈÎȷο fiÚÈ· ·fi 2 ¤ˆ˜ 54 ÌËÓÒÓ Î·È ˘Ô¯ˆÚ› ηٿ ηÓfiÓ· Û 3-6 Ì‹Ó˜. ∏ ·ÈÙÈÔ·ıÔÁ¤-

ÓÂÈ¿ Ù˘ ·Ú·Ì¤ÓÂÈ ¿ÁÓˆÛÙË, Èı·ÓÔÏÔÁÂ›Ù·È fï˜ fiÙÈ Û¯ÂÙ›˙ÂÙ·È Ì ÚÔËÁËı›۷ Ïԛ̈ÍË, ÈÔÁÂÓÔ‡˜, ηٿ ηÓfiÓ·, ·ÈÙÈÔÏÔÁ›·˜. ∂ÎÙÂÓ‹˜ ÎÏÈÓÈÎfi˜ Î·È ·Ú·ÎÏÈÓÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‰ÂÓ ·ÔηχÙÂÈ Ë·ÙÈ΋ ‹ ÔÛÙÈ΋ ·ıÔÏÔÁ›· Ë ÔÔ›· Ó· ÂÍËÁ› ÙËÓ ·‡ÍËÛË Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘, ÔÚÈṲ̂Ó˜ Ì¿ÏÈÛÙ· ÊÔÚ¤˜, ¤ˆ˜ Î·È 30 ÊÔÚ¤˜ ¿Óˆ ·fi Ù· ·ÓÒÙÂÚ· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· (2). ¶ÂÚÈÁÚ¿ÊÂÙ·È ÛÙË Û˘Ó¤¯ÂÈ·, Ë ÂÚ›ÙˆÛË ¿ÚÚÂÓÔ˜ ÓË›Ô˘, ËÏÈΛ·˜ 16 ÌËÓÒÓ, Ì Ôχ ·˘ÍË̤ÓË

1 π‰ÈÒÙ˘ ¶·È‰›·ÙÚÔ˜ 2 ∂ÈÌÂÏËÙ‹˜ µ' ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ¶ÂÚÈÊÂÚÂÈ·ÎÔ‡ °ÂÓÈÎÔ‡ ¡ÔÛÔÎÔÌ›Ԣ ¡›Î·È·˜, ¶ÂÈÚ·È¿˜ “ÕÁÈÔ˜ ¶·ÓÙÂÏ‹̈Ӕ

1 Private practicing Pediatrician 2 Registrar 2nd Pediatric Clinic General Hospital of Nikea, Piraeus “Saint Panteleimon”

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 88

¶·È‰È·ÙÚÈ΋ 2001;64:87-90

ÙÈÌ‹ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡, Ë ÔÔ›· ·Ó¢ڤıËΠÛÙ· Ï·›ÛÈ· Ù˘¯·›Ô˘ ÂÚÁ·ÛÙËÚÈ·ÎÔ‡ ÂϤÁ¯Ô˘ Î·È ˘Ô¯ÒÚËÛ Ï‹Úˆ˜ Û ‰È¿ÛÙËÌ· 6 ÌËÓÒÓ. ¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ ∞ÁfiÚÈ, ËÏÈΛ·˜ 16 ÌËÓÒÓ, Ì ÂχıÂÚÔ ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi Î·È Ê˘ÛÈÔÏÔÁÈΤ˜ ۈ̷ÙÔÌÂÙÚÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜, ÂÍÂÙ¿ÛıËΠ‰‡Ô ÊÔÚ¤˜ ̤۷ Û ‰È¿ÛÙËÌ· ÂÓfi˜ ÌËÓfi˜ ·fi ÙÔÓ ·È‰›·ÙÚfi ÙÔ˘, ÏfiÁˆ ˘ÚÂÙÔ‡ ÙËÓ ÚÒÙË ÊÔÚ¿, Ô ÔÔ›Ô˜ ·Ô‰fiıËΠ۠ÈÔÁÂÓ‹ Î˘Ó¿Á¯Ë Î·È ÏfiÁˆ ÂÌ‡ÚÂÙ˘ Á·ÛÙÚÂÓÙÂÚ›Ùȉ·˜ ÙË ‰Â‡ÙÂÚË ÊÔÚ¿, Ì ¯·Ú·ÎÙ‹Ú˜ ÈÔÁÂÓÔ‡˜ Ïԛ̈͢. ∆Ô ·È‰› ‰ÂÓ ¤Ï·‚ ʿÚ̷η, ÂÎÙfi˜ ·fi ·Ú·ÎÂÙ·ÌfiÏË ˆ˜ ·ÓÙÈ˘ÚÂÙÈÎfi Û ηÓÔÓÈΤ˜ ‰fiÛÂȘ. ™Â ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô ÚÔ˘Ù›Ó·˜, Ô ÔÔ›Ô˜ ·ÎÔÏÔ‡ıËÛ ‰‡Ô ‚‰ÔÌ¿‰Â˜ ÌÂÙ¿ ÙËÓ ·Ô‰ÚÔÌ‹ Ù˘ ‰Â‡ÙÂÚ˘ Ïԛ̈͢, ·Ó¢ڤıËΠÔχ ·˘ÍË̤ÓË ÙÈÌ‹ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡, ۯ‰fiÓ ‰ÂηÏ¿ÛÈ· Ù˘ ·ÓÒÙÂÚ˘ Ê˘ÛÈÔÏÔÁÈ΋˜ ÙÈÌ‹˜ (ÙÈÌ‹ ·ÛıÂÓÔ‡˜ 3490 u/l, Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ÁÈ· ÙËÓ ËÏÈΛ· ·˘Ù‹ 150-420 u/l). ∫·Ù¿ ÙË ÏÂÙÔÌÂÚ‹ Ê˘ÛÈ΋ ÂͤٷÛË ÙÔ˘ ÓË›Ô˘, ‰ÂÓ ·Ó¢ڤıËÎ·Ó ÛËÌ›· ÂÓ‰ÂÈÎÙÈο Ú·¯›Ùȉ·˜, Ô‡Ù Ë·ÙÔÌÂÁ·Ï›· ‹ ¿ÏÏÔ ·ÍÈÔÛËÌ›ˆÙÔ ·ıÔÏÔÁÈÎfi ‡ÚËÌ·. √ ·ÛıÂÓ‹˜ ˘Ô‚Ï‹ıËΠ۠Ï‹ÚË ÂÚÁ·ÛÙËÚÈ·Îfi ¤ÏÂÁ¯Ô Ô ÔÔ›Ô˜ ·¤ÎÏÂÈÛ ÔÛÙÈ΋, Ë·ÙÈ΋ ‹ ¿ÏÏË ÓfiÛÔ Ô˘ Ó· ‰ÈηÈÔÏÔÁ› ÙÔ ·Ú·¿Óˆ ÂÚÁ·ÛÙËÚÈ·Îfi ‡ÚËÌ·. ™˘ÁÎÂÎÚÈ̤ӷ, ·Ó¢ڤıËηÓ: Hb: 12,2 g/dl, Ht: 36,1%, MCV: 77,5 fl, MCH: 26,1 pg, RDW-CV: 13,8%, ÏÂ˘Î¿ ·ÈÌÔÛÊ·›ÚÈ·: 12.480/mm3 (ÔÏ˘ÌÔÚÊÔ‡ÚËÓ·: 49%, ÏÂÌÊÔ·ÙÙ·Ú·: 37%, ÌÂÁ¿Ï· ÌÔÓÔ‡ÚËÓ·: 8%, ˈÛÈÓfiÊÈÏ·: 6%), ·ÈÌÔÂÙ¿ÏÈ·: 365.000/mm3, AST(SGOT): 41 u/l (ÊÙ <65 u/l), ALT(SGPT): 32 u/l (ÊÙ <54 u/l), Á-GT (Á-ÁÏÔ˘Ù·Ì˘ÏÈ΋ ÙÚ·ÓÛÂÙȉ¿ÛË): 9u/l (ÊÙ: 0-23 u/l), ÔÏÈο Ï¢ÎÒÌ·Ù· ÔÚÔ‡: 6,1 g/dl (ÊÙ: 3,77,5 g/dl), ÏÂ˘ÎˆÌ·Ù›ÓË ÔÚÔ‡: 4,2 g/dl (ÊÙ: 1,9-5 g/dl), ÛÊ·ÈÚ›Ó˜ ÔÚÔ‡: 1,9 g/dl (ÊÙ: 1,3-5 g/dl), ÔÏÈÎfi ·Û‚¤ÛÙÈÔ ·›Ì·ÙÔ˜: 9,5 mg/dl (ÊÙ: 8-10,5 mg/dl), ÈÔÓÈṲ̂ÓÔ ·Û‚¤ÛÙÈÔ ·›Ì·ÙÔ˜: 0,96 mMol/l (ÊÙ: 1,051,3 mMol/l), ʈÛÊfiÚÔ˜ ·›Ì·ÙÔ˜: 4,8 mg/dl (ÊÙ: 3,2-6,3 mg/dl), ·Ú·ıÔÚÌfiÓË ÔÚÔ‡: 13 pg/ml (ÊÙ: 10-65 pg/ml), ÔÛÙ¿ÛË (ÔÛÙÈ΋ ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË) ÔÚÔ‡: 121 Ìg/L (ÊÙ: 6-183,5 Ìg/l), 25(OH) ‚ÈÙ·Ì›ÓË D3: 51,8 ng/ml (ÊÙ: 16-74 ng/ml). ™Â Ó¤· ̤ÙÚËÛË, Ì›· ‚‰ÔÌ¿‰· ÌÂÙ¿ ÙËÓ ·Ú¯È΋, Ë ÙÈÌ‹ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡ ‚ÚÈÛÎfiÙ·Ó ÛÙ· ›‰È· ÂÚ›Ô˘ Â›‰·, ÛÙȘ 3568 u/l. ∏ ̤ÙÚËÛË ÙˆÓ ÈÛÔÂÓ˙‡ÌˆÓ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ Ì ËÏÂÎÙÚÔÊfiÚËÛË ¤‰ÂÈÍÂ Ê˘ÛÈÔÏÔÁÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· ÙÔ˘ ÔÛÙÈÎÔ‡ ÎÏ¿ÛÌ·ÙÔ˜ ·ÏÏ¿ ·˘ÍË̤ÓÔ ÙÔ Ë·ÙÈÎfi ÎÏ¿ÛÌ· L1: 57,1% + L2: 20,7% = 77,8% (ÊÙ: 30-40%). ∏ ÙÈÌ‹ Ù˘ 5-ÓÔ˘ÎÏÂÔÙȉ¿Û˘

88

Paediatriki 2001;64:87-90

‹Ù·Ó Â›Û˘ ·˘ÍË̤ÓË, 23,1 u/l Û 30ÔC ÌÂ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ›Û˜ ‹ ÌÈÎÚfiÙÂÚ˜ ÙˆÓ 5 u/l Û 30ÔC. ∏ ·ÎÙÈÓÔÁÚ·Ê›· Ù˘ ‰ÂÍÈ¿˜ ˯ÂÔηÚÈ΋˜ ¿ÚıÚˆÛ˘ ‰ÂÓ ·ÔÎ¿Ï˘„Â Â˘Ú‹Ì·Ù· ÂÓ‰ÂÈÎÙÈο Ú·¯›Ùȉ·˜ ‹ ¿ÏÏ˘ ÔÛÙÈ΋˜ ·ıÔÏÔÁ›·˜. ¶ÚÔÛ‰ÈÔÚ›ÛıËΠÂ›Û˘, Ë ÙÈÌ‹ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡ ÙˆÓ ÁÔÓ¤ˆÓ, Ë ÔÔ›· ‹Ù·Ó ̤۷ ÛÂ Ê˘ÛÈÔÏÔÁÈο fiÚÈ·. √ ·ÚÓËÙÈÎfi˜ ÁÈ· Ë·ÙÈ΋ Î·È ÔÛÙÈ΋ ÓfiÛÔ ·Ú·ÎÏÈÓÈÎfi˜ ¤ÏÂÁ¯Ô˜, Ë ËÏÈΛ· ÙÔ˘ ·È‰ÈÔ‡, ÔÈ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡ ÙˆÓ ÁÔÓ¤ˆÓ, ÔÈ ÚÔËÁËı›Û˜ ÎÏÈÓÈο ÙÔ˘Ï¿¯ÈÛÙÔÓ ‚¤‚·È˜ ÈÔÁÂÓ›˜ ÏÔÈÌÒÍÂȘ (ÔÚÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ¤Ó·ÓÙÈ ÈÒÓ ‰ÂÓ Î·Ù¤ÛÙË ‰˘Ó·ÙfiÓ Ó· Ú·ÁÌ·ÙÔÔÈËı›) ηıÒ˜ Î·È Ë ¿ÚÈÛÙË, ·fi οı ¿Ô„Ë, ηٿÛÙ·ÛË ÙÔ˘ ÓË›Ô˘, ¤ÛÙÚ„·Ó ÙË ‰È·ÁÓˆÛÙÈ΋ Ì·˜ ÛΤ„Ë ÚÔ˜ ÙËÓ ÏÂ˘Ú¿ Ù˘ ·ÚÔ‰È΋˜ ηÏÔ‹ıÔ˘˜ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·˜, ÌÂÙ·ÏÔÈÌÒ‰Ô˘˜ Èı·Ófiٷٷ ·ÈÙÈÔÏÔÁ›·˜ Î·È ·ÔÊ·Û›ÛıËΠ·Ó·ÌÔÓ‹ Î·È Â·Ó¤ÏÂÁ¯Ô˜ Û ¤ÍÈ Ì‹Ó˜. ¶Ú¿ÁÌ·ÙÈ, ÛÙÔÓ Â·Ó¤ÏÂÁ¯Ô, Ô ÔÔ›Ô˜ ¤ÁÈÓ ÌÂÙ¿ ·fi ¤ÍÈ Ì‹Ó˜, Ë ÙÈÌ‹ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡ ›¯Â Â·Ó¤ÏıÂÈ Û ·Ôχو˜ Ê˘ÛÈÔÏÔÁÈο Â›‰· (275 u/l), ÛÙÔÈ¯Â›Ô ÙÔ ÔÔ›Ô Û˘Ó¤‚·Ï ÛÙËÓ ÔÚÈÛÙÈ΋ ÙÂÎÌËÚ›ˆÛË Ù˘ ‰È¿ÁÓˆÛ˘ Ù˘ ηÏÔ‹ıÔ˘˜ ·ÚÔ‰È΋˜ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·˜. ™˘˙‹ÙËÛË ∏ ηÏÔ‹ı˘ ·ÚÔ‰È΋ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›· (·‡ÍËÛË Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡) Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ·ÚÎÂÙ¿ Û˘¯Ó‹ ÓÔÛÔÏÔÁÈ΋ ÔÓÙfiÙËÙ·, ·Ó ÎÚ›ÓÂÈ Î·Ó›˜ ·fi ÙÔÓ ·ÚÈıÌfi ÙˆÓ Û¯ÂÙÈÎÒÓ ‚È‚ÏÈÔÁÚ·ÊÈÎÒÓ ·Ó·ÊÔÚÒÓ, ·ÏÏ¿ Î·È ·fi ÙÔ ÂÍ·ÈÚÂÙÈο Èı·Ófi ÁÂÁÔÓfi˜ ÌÂÁ¿ÏÔ˜ ·ÚÈıÌfi˜ ÂÚÈÙÒÛÂˆÓ Ó· ‰È·Ê‡ÁÔ˘Ó ·‰È¿ÁÓˆÛÙ˜, ηıÒ˜ Ì¿ÏÈÛÙ· ÚfiÎÂÈÙ·È ÂÚ› ·Û˘Ìو̷ÙÈ΋˜ ηٷÛÙ¿Ûˆ˜, Ô ‰Â ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù˘ ÙÈÌ‹˜ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÙÔ˘ ÔÚÔ‡ ‰ÂÓ Û˘ÓËı›˙ÂÙ·È Ó· ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙȘ ÂÚÁ·ÛÙËÚȷΤ˜ ÂÍÂÙ¿ÛÂȘ ÚÔ˘Ù›Ó·˜ ÛÙȘ Ôԛ˜ ˘Ô‚¿ÏÏÔÓÙ·È Ù· ‚Ú¤ÊË Î·È Ù· Ó‹È·. ¶ÚfiÎÂÈÙ·È ÁÈ· ηÏfiËı˜, ·ÚÔ‰ÈÎfi Ê·ÈÓfiÌÂÓÔ, ÙÔ ÔÔ›Ô ·Ú·ÙËÚÂ›Ù·È Û ‚Ú¤ÊË Î·È Ó‹È· ËÏÈΛ·˜ ¤ˆ˜ 5 ÂÙÒÓ, ÂÍ›ÛÔ˘ ÛÙ· ‰‡Ô ʇϷ, ¯ˆÚ›˜ ÙËÓ ·ÚÔ˘Û›· Ë·ÙÈ΋˜ ‹ ÔÛÙÈ΋˜ ·ıÔÏÔÁ›·˜ (1, 2), Â·Ó¤Ú¯ÂÙ·È ‰Â ÛÙÔ Ê˘ÛÈÔÏÔÁÈÎfi Û ÙÚÂȘ ¤ˆ˜ ¤ÍÈ Ì‹Ó˜. ∏ ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË Â›Ó·È ‰ÈÌÂÚ‹˜ ÁÏ˘ÎÔÚˆÙ½ÓË, ÏÔ‡ÛÈ· Û „¢‰¿ÚÁ˘ÚÔ, Ë ÔÔ›· ·Ú¿ÁÂÙ·È Î˘Ú›ˆ˜ ÛÙÔ ‹·Ú Î·È Ù· ÔÛÙ¿. ∞ÔÙÂÏ› ÙÔÓ Î‡ÚÈÔ ÂÎÚfiÛˆÔ Ù˘ ÔÌ¿‰·˜ ÙˆÓ ‚ÈÔÏÔÁÈÎÒÓ ÂÍÂÙ¿ÛÂˆÓ Ô˘ ¯·Ú·ÎÙËÚ›˙Ô˘Ó ÙË ¯ÔÏfiÛÙ·ÛË. ∞˘Í¿ÓÂÈ Û οı ÂÚ›ÙˆÛË ¯ÔÏÈ΋˜ ÛÙ¿Û˘, Ï‹ÚÔ˘˜ ‹ ·ÙÂÏÔ‡˜, Û ÔÔÈÔ‰‹ÔÙ ÛËÌÂ›Ô ÙÔ˘ ¯ÔÏËÊfiÚÔ˘ ‰¤Ó‰ÚÔ˘ Î·È ÏÈÁfiÙÂÚÔ Û ÂÚÈÙÒÛÂȘ


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 89

¶·È‰È·ÙÚÈ΋ 2001;64:87-90

‚Ï¿‚˘ ÙˆÓ Ë·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ∂Âȉ‹ fï˜, Ë ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË ·Ú¿ÁÂÙ·È Î·È ÛÙ· ÔÛÙ¿ (·fi ÙÔ˘˜ ÔÛÙÂÔ‚Ï¿ÛÙ˜), Ë ÙÈÌ‹ Ù˘ ‚Ú›ÛÎÂÙ·È ·˘ÍË̤ÓË ÛÙËÓ ÂÚ›Ô‰Ô Ù˘ ÔÛÙÈ΋˜ ·Ó¿Ù˘Í˘ ηıÒ˜ Î·È Û ÔÔÈ·‰‹ÔÙ ηٿÛÙ·ÛË Ì ·˘ÍË̤ÓË ÔÛÙÂÔ‚Ï·ÛÙÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· (Ú·¯›Ùȉ·, ˘ÂÚ·Ú·ı˘ÚÂÔÂȉÈÛÌfi˜, ÔÛÙÂÔ‚Ï·ÛÙÈΤ˜ ÌÂÙ·ÛÙ¿ÛÂȘ, ÔÛÙÂÔÏ˘ÙÈΤ˜ ÂÂÍÂÚÁ·Û›Â˜ Î.Ï.). ∞Ó Î·È Ô ‚ÏÂÓÓÔÁfiÓÔ˜ ÙˆÓ ÂÓÙ¤ÚˆÓ Î·È Ô ÓÂÊÚfi˜ ¤¯Ô˘Ó ˘„ËÏ‹ ÂÚÈÂÎÙÈÎfiÙËÙ· Û ·ÏηÏÈ΋ ʈÛÊ·Ù¿ÛË, ÓfiÛÔÈ ÙˆÓ ÔÚÁ¿ÓˆÓ ·˘ÙÒÓ ‰ÂÓ ÂËÚ¿˙Ô˘Ó ÙȘ ÂÚÈÊÂÚÈΤ˜ ÙÈ̤˜. ™Â ·ËÛË ÂÍ¿ÏÏÔ˘, ·˘ÍË̤ÓË ÙÈÌ‹ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È Û ·Ú·ÁˆÁ‹ ·fi ÙÔÓ Ï·ÎÔ‡ÓÙ·. ™Â Ê˘ÛÈÔÏÔÁÈο ·È‰È¿, Ë ‰Ú·ÛÙËÚÈfiÙËÙ· Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ Â›Ó·È ·˘ÍË̤ÓË Û ۯ¤ÛË Ì ·˘Ù‹ ÙˆÓ ÂÓËϛΈÓ. ∞˘Ùfi ÔÊ›ÏÂÙ·È Û ·‡ÍËÛË ÙÔ˘ ÔÛÙÈÎÔ‡ ÈÛÔÂÓ˙‡ÌÔ˘, ˆ˜ Û˘Ó¤ÂÈ· Ù˘ Ê˘ÛÈÔÏÔÁÈο ·˘ÍË̤Ó˘ ÔÛÙÂÔ‚Ï·ÛÙÈ΋˜ ‰Ú·ÛÙËÚÈfiÙËÙ·˜ Û ¤Ó·Ó ÁÚ‹ÁÔÚ· ·˘Í·ÓfiÌÂÓÔ ÔÚÁ·ÓÈÛÌfi. ∂ȉÈÎfiÙÂÚ·, ηٿ ÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô Ë ‰Ú·ÛÙËÚÈfiÙËÙ· ÙÔ˘ ÂÓ˙‡ÌÔ˘ Â›Ó·È ·ÛÙ·ı‹˜, ÒÛÙÂ Â›Ó·È ‰˘Û¯ÂÚ‹˜ Ô Î·ıÔÚÈÛÌfi˜ ·ÛÊ·ÏÒÓ ÔÚ›ˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÙÈÌÒÓ. ª¤¯ÚÈ ÙÔÓ ¤ÎÙÔ Ì‹Ó·, Ë ÂÓ˙˘ÌÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· ·˘Í¿ÓÂÙ·È Û Â›‰· ÙÚÈÏ¿ÛÈ· ¤ˆ˜ ÂÍ·Ï¿ÛÈ· ·˘ÙÒÓ ÙˆÓ ÂÓËϛΈÓ, ÂÓÒ ·ÎÔÏÔ‡ıˆ˜ ˘Ô¯ˆÚ› ÚÔԉ¢ÙÈο ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 2 ÂÙÒÓ, ÔfiÙÂ Ë ÙÈÌ‹ ÙÔ˘ ÂÓ˙‡ÌÔ˘ Â›Ó·È ‰ÈÏ¿ÛÈ· ¤ˆ˜ ÙÂÙÚ·Ï¿ÛÈ· ·fi ÂΛÓË ÙˆÓ ÂÓËϛΈÓ. ™ÙË Û˘Ó¤¯ÂÈ·, Ù· Â›‰· Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÙÔ˘ ÔÚÔ‡ ·Ú·Ì¤ÓÔ˘Ó ÛÙ·ıÂÚ¿ ̤¯ÚÈ ÙËÓ ÂÊ˂›·, ¿ÓÙÔÙ fï˜ Â›Ó·È ·˘ÍË̤ӷ Û ۯ¤ÛË Ì ٷ Â›‰· ÙˆÓ ÂÓËϛΈÓ, Èı·Ófiٷٷ ÏfiÁˆ Ù˘ Ê˘ÛÈÔÏÔÁÈο ·˘ÍË̤Ó˘ ¤ÎÎÚÈÛ˘ ÙÔ˘ ÔÛÙÈÎÔ‡ Ù˘ ÈÛÔÂÓ˙‡ÌÔ˘ ·fi ÙÔ˘˜ ÔÛÙÂÔ‚Ï¿ÛÙ˜ ηٿ ÙËÓ ·Û‚ÂÛÙÔÔ›ËÛË ÙÔ˘ ·˘Í·ÓfiÌÂÓÔ˘ ÛÎÂÏÂÙÔ‡. ∫·Ù¿ ÙËÓ ÂÊ˂›·, ÏfiÁˆ Ù˘ Ù·¯Â›·˜ ·‡ÍËÛ˘ ÙÔ˘ ÛÎÂÏÂÙÔ‡, ÙÈ̤˜ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÔÚÔ‡ ÌÂÁ·Ï‡ÙÂÚ˜ ·ÎfiÌË Î·È ÙˆÓ 1000 u/l ıˆÚÔ‡ÓÙ·È ·Ó·ÌÂÓfiÌÂÓ˜, ȉ›ˆ˜ ÛÙ· ·ÁfiÚÈ·. °ÂÓÈο ¿ÓÙˆ˜, οı ·‡ÍËÛË Ù˘ ÙÈÌ‹˜ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÙÔ˘ ÔÚÔ‡, Û Â›‰· ÂÓÙ·Ï¿ÛÈ· ‹ Î·È ÌÂÁ·Ï‡ÙÂÚ· ÙˆÓ ·ÓˆÙ¤ÚˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÙÈÌÒÓ Û ‚Ú¤ÊÔ˜ ‹ Ó‹ÈÔ, ›ıÈÛÙ·È Ó· ‰ÈÂÚÂ˘Ó¿Ù·È ‰ÈÂÍÔ‰Èο Î·È ÂÊfiÛÔÓ ·ÔÎÏÂÈÛı› ÔÛÙÈ΋ ‹ Ë·ÙÈ΋ ·ıÔÏÔÁ›· Ó· ·Ô‰›‰ÂÙ·È Û ηÏÔ‹ıË ·ÚÔ‰È΋ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·, ‰È¿ÁÓˆÛË Ë ÔÔ›· ı· Ú¤ÂÈ Ó· ÂȂ‚·Èˆı› Ì ÙËÓ Â¿ÓÔ‰Ô ÙˆÓ ÙÈÌÒÓ ÛÂ Ê˘ÛÈÔÏÔÁÈο Â›‰· ̤۷ Û 36 Ì‹Ó˜. ∏ ·ÈÙÈÔÏÔÁ›· Ù˘ ηÏÔ‹ıÔ˘˜ ·ÚÔ‰È΋˜ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·˜ ·Ú·Ì¤ÓÂÈ ·‰È¢ÎÚ›ÓÈÛÙË. ¶Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ Â›Ó·È ÙÔ ·ÔÙ¤ÏÂÛÌ· ÚÔËÁËı›۷˜

Paediatriki 2001;64:87-90

Ïԛ̈͢, ηٿ ηÓfiÓ· ÈÔÁÂÓÔ‡˜, fiˆ˜ Û˘Ó¿ÁÂÙ·È ·fi ÌÂÁ¿ÏÔ ·ÚÈıÌfi ·Ú·ÙËÚ‹ÛÂˆÓ (3-10). £ÂˆÚÂ›Ù·È fiÙÈ Ô ˘‡ı˘ÓÔ˜ ÏÔÈÌÔÁfiÓÔ˜ ·Ú¿ÁÔÓÙ·˜ ÂËÚ¿˙ÂÈ Èı·Ófiٷٷ ÙËÓ ·Ú·ÁˆÁ‹, ÙË ÛÙ·ıÂÚfiÙËÙ· ‹ ÙËÓ Î¿ı·ÚÛË ÙÔ˘ ÂÓ˙‡ÌÔ˘ ·fi ÙËÓ Î˘ÎÏÔÊÔÚ›·. ¶Èı·ÓfiÙÂÚË ıˆÚÂ›Ù·È Ë ·ÚÂÌfi‰ÈÛË Ù˘ οı·ÚÛ˘ ÙÔ˘ ÂÓ˙‡ÌÔ˘ ̤ۈ ·¢ı›·˜ ‰Ú¿Û˘ ÙÔ˘ ÏÔÈÌÔÁfiÓÔ˘ ·Ú¿ÁÔÓÙ· ÛÙÔÓ ÂÓ˙˘ÌÈÎfi ÌÂÙ·‚ÔÏÈÛÌfi Î·È ÛÙËÓ Ë·ÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·. ∂‰Ò Ú¤ÂÈ Ó· ÛËÌÂÈÒÛÔ˘Ì fiÙÈ Ë ÙÈÌ‹ ÙÔ˘ Ë·ÙÈÎÔ‡ ÈÛÔÂÓ˙‡ÌÔ˘ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘, Ù˘ 5ÓÔ˘ÎÏÂÔÙȉ¿Û˘, ·˘Í¿ÓÂÙ·È ÛÙÔÓ ÔÚfi ÛÙȘ ÂÚÈÙÒÛÂȘ Ë·ÙÔ¯ÔÏÈÎÒÓ ·ı‹ÛˆÓ, ÂÓÒ ·Ú·Ì¤ÓÂÈ ÂÓÙfi˜ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÔÚ›ˆÓ Û ÂÚÈÙÒÛÂȘ ÔÛÙÈ΋˜ ‚Ï¿‚˘. ™ÙË ‰È΋ Ì·˜ ÂÚ›ÙˆÛË ‹Ù·Ó ·˘ÍË̤ӷ ÙfiÛÔ Ë 5-ÓÔ˘ÎÏÂÔÙȉ¿ÛË, fiÛÔ Î·È Ù· ÏÔÈ¿ Ë·ÙÈο ÈÛÔ¤Ó˙˘Ì· Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘, ÛÙÔÈ¯Â›Ô ÙÔ ÔÔ›Ô ¤¯ÂÈ ·Ó¢ÚÂı› Î·È Û ¿ÏϘ ÂÚÈÙÒÛÂȘ ηÏÔ‹ıÔ˘˜ ·ÚÔ‰È΋˜ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·˜ (4). ∏ ·Ô˘Û›· ÈÎÙ¤ÚÔ˘ Î·È ÔÈ Ê˘ÛÈÔÏÔÁÈΤ˜ ÙÈ̤˜ ÙˆÓ ÙÚ·ÓÛ·ÌÈÓ·ÛÒÓ Î·È Ù˘ Á-ÁÏÔ˘Ù·Ì˘ÏÈ΋˜ ÙÚ·ÓÛÂÙȉ¿Û˘ (Á-GT) Û˘ÓËÁÔÚÔ‡Ó ˘¤Ú Ù˘ ·Ô˘Û›·˜ Ë·ÙÈ΋˜ ÓfiÛÔ˘. º·›ÓÂÙ·È ÏÔÈfiÓ fiÙÈ ˘fi ÙËÓ Â›‰Ú·ÛË ÂÓfi˜ ÏÔÈÌÔÁfiÓÔ˘ ·Ú¿ÁÔÓÙ· Â¤Ú¯ÂÙ·È ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ·fi ÙÔ ‹·Ú (fiˆ˜ ¿ÏψÛÙÂ Û˘Ì‚·›ÓÂÈ Î·È Ì ¿ÏϘ ÚˆÙ½Ó˜ ¯. C-·ÓÙȉÚÒÛ· ÚˆÙ½ÓË, ÊÂÚÚÈÙ›ÓË, ÎÏ.) ‹ ‰ÔÌÈ΋ ÌÂÙ¿ÏÏ·ÍË Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ‹ Ù¤ÏÔ˜ ·ÚÔ‰Èο ÌÂȈ̤ÓË Ë·ÙÈ΋ οı·ÚÛË ÙÔ˘ ÂÓ˙‡ÌÔ˘ (¯ˆÚ›˜ ÙËÓ ·ÚÔ˘Û›· ÂÌÊ·ÓÔ‡˜ Ë·ÙÈ΋˜ ÓfiÛÔ˘) ÌÂ Ê˘ÛÈÔÏÔÁÈÎfi Û οı ÂÚ›ÙˆÛË ÔÛÙÈÎfi ÌÂÙ·‚ÔÏÈÛÌfi (5,6). ∏ ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË Ù˘ ηÏÔ‹ıÔ˘˜ ·ÚÔ‰È΋˜ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·˜, ¤Ú·Ó ‚‚·›ˆ˜ ÙˆÓ ÓÔÛËÌ¿ÙˆÓ ÙÔ˘ ‹·ÙÔ˜ Î·È ÙÔ˘ ÛÎÂÏÂÙÔ‡, Ú¤ÂÈ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ÙËÓ ÔÈÎÔÁÂÓ‹ ηÏÔ‹ıË ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›· Ë ÔÔ›· ÎÏËÚÔÓÔÌÂ›Ù·È Ì ÙÔÓ ·˘ÙfiÛˆÌÔ ÂÈÎÚ·ÙÔ‡ÓÙ· ¯·Ú·ÎÙ‹Ú·. ŸÌˆ˜, Ë Î·Ù¿ÛÙ·ÛË ·˘Ù‹ Â›Ó·È Â›ÌÔÓË Î·È ‰ÂÓ ˘Ô¯ˆÚ› Û ‰È¿ÛÙËÌ· Ï›ÁˆÓ ÌËÓÒÓ (7). ÕÏψÛÙÂ, ÛÙË ‰È΋ Ì·˜ ÂÚ›ÙˆÛË Ô ¤ÏÂÁ¯Ô˜ ÙˆÓ ÁÔÓ¤ˆÓ ·¤‚Ë ·ÚÓËÙÈÎfi˜. ∂›Û˘, ÔÚÈṲ̂ӷ Ê¿Ú̷η, ·ÓÙÈÂÈÏËÙÈο, ·ÓÙÈ‚ÈÔÙÈο Î·È ¿ÏÏ·, ÌÔÚ› Ó· Ô‰ËÁ‹ÛÔ˘Ó Û ·ÚÔ‰Èο ·˘ÍË̤Ó˜ ÙÈ̤˜ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ̤ۈ Èı·Ófiٷٷ Ë·ÙÔÙÔÍÈ΋˜ ‰Ú¿Û˘ ÌÂ Û˘ÓÔ‰fi ¯ÔÏfiÛÙ·ÛË (8). ™ÙË ‰È΋ Ì·˜ ÂÚ›ÙˆÛË ‰ÂÓ Â›¯·Ó ¯ÔÚËÁËı› ÛÙÔ ·È‰› Ê¿Ú̷η, ÂÎÙfi˜ Ù˘ ·Ú·ÎÂÙ·ÌfiÏ˘ Û ηÓÔÓÈΤ˜ ·ÓÙÈ˘ÚÂÙÈΤ˜ ‰fiÛÂȘ, ÔÈ Ôԛ˜ ‰ÂÓ ı· ‰ÈηÈÔÏÔÁÔ‡Û·Ó Û η̛· ÂÚ›ÙˆÛË ÙËÓ ·Ú·ÙËÚËı›۷ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·. ∂Ó Î·Ù·ÎÏ›‰È, Ù· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ÁÈ· ÙËÓ ÙÂÎÌËÚ›ˆÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Ù˘ ηÏÔ‹ıÔ˘˜

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 90

¶·È‰È·ÙÚÈ΋ 2001;64:87-90

·ÚÔ‰È΋˜ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›·˜ Â›Ó·È (3,10): ·. ∏ÏÈΛ· <5 ÂÙÒÓ. ‚. ∞Ô˘Û›· ÎÏÈÓÈÎÒÓ Û˘ÌÙˆÌ¿ÙˆÓ ‹ ÛËÌ›ˆÓ ÂÓ‰ÂÈÎÙÈÎÒÓ Ë·ÙÈ΋˜ ‹ ÔÛÙÈ΋˜ ·ıÔÏÔÁ›·˜ ‹ ·Ó·ÊÔÚ¿ ÛÙÔ ÚfiÛÊ·ÙÔ ÈÛÙÔÚÈÎfi ÙÔ˘ ·ÛıÂÓÔ‡˜ Û˘ÌÙˆÌ¿ÙˆÓ ÂÓ‰ÂÈÎÙÈÎÒÓ ÚÔËÁËı›۷˜ Ïԛ̈͢, Ù· ÔÔ›· fï˜ ‰ÂÓ Û¯ÂÙ›˙ÔÓÙ·È Ì Ë·ÙÈ΋ ‹ ÔÛÙÈ΋ ÓfiÛÔ. Á. ∫ÏÈÓÈ΋ ÂͤٷÛË ·ÚÓËÙÈ΋ ÁÈ· ÔÛÙÈ΋ ‹ Ë·ÙÈ΋ ·ıÔÏÔÁ›·. ‰. ¶·Ú·ÎÏÈÓÈÎfi˜ ¤ÏÂÁ¯Ô˜ ·ÚÓËÙÈÎfi˜ ÁÈ· ÔÛÙÈ΋ ‹ Ë·ÙÈ΋ ÓfiÛÔ. Â. ∏ÏÂÎÙÚÔÊfiÚËÛË ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ Û˘Ì‚·Ù‹ Ì ηÏÔ‹ıË ·ÚÔ‰È΋ ˘ÂÚʈÛÊ·Ù·Û·ÈÌ›· (·‡ÍËÛË ÙˆÓ Ë·ÙÈÎÒÓ ÈÛÔÂÓ˙‡ÌˆÓ) Î·È Ù¤ÏÔ˜, ÛÙ. ∂¿ÓÔ‰Ô˜ ÙˆÓ ÂÈ¤‰ˆÓ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÛÙÔ Ê˘ÛÈÔÏÔÁÈÎfi ÂÓÙfi˜ ÙÚÈÒÓ ¤ˆ˜ ¤ÍÈ ÌËÓÒÓ. ™˘ÓÂÒ˜, Ë ‰È΋ Ì·˜ ÂÚ›ÙˆÛË ·ÔÙÂÏ› ¯·Ú·ÎÙËÚÈÛÙÈÎfi ·Ú¿‰ÂÈÁÌ· Ù˘ ηÏÔ‹ıÔ˘˜ ·˘Ù‹˜ ÔÓÙfiÙËÙ·˜. ∂›Ó·È ¿ÓÙˆ˜ Ê·ÓÂÚfi fiÙÈ Ô ·È‰›·ÙÚÔ˜ ‚Ú›ÛÎÂÙ·È ÂÓÒÈÔÓ ÂÓfi˜ ÛÔ‚·ÚÔ‡ ‰ÈÏ‹ÌÌ·ÙÔ˜, ˆ˜ ÚÔ˜ ÙËÓ ·Ó·ÁηÈfiÙËÙ· Î·È ÙÔ ‚¿ıÔ˜ ÙÔ˘ ÂÚÁ·ÛÙËÚÈ·ÎÔ‡ ÂϤÁ¯Ô˘ Ô ÔÔ›Ô˜ ı· ··ÈÙËı›, fiÙ·Ó Î·ÏÂ›Ù·È Ó· ·ÓÙÈÌÂÙˆ›ÛÂÈ ÙËÓ ÂÚ›ÙˆÛË ÂÓfi˜ ÎÏÈÓÈο ˘ÁÈÔ‡˜ ·È‰ÈÔ‡ Ì ÛËÌ·ÓÙÈ΋ ·‡ÍËÛË Ù˘ ÙÈÌ‹˜ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÙÔ˘ ÔÚÔ‡. ¶ÈÛÙ‡ԢÌ fiÙÈ ÂÊfiÛÔÓ Ë ·Ó¿Ù˘ÍË ÙÔ˘ ·È‰ÈÔ‡ Â›Ó·È Ê˘ÛÈÔÏÔÁÈ΋, Ë ÎÏÈÓÈ΋ ÂͤٷÛË ·ÚÓËÙÈ΋, Â›Û˘ ·ÚÓËÙÈÎfi˜ Î·È Ô ·‰Úfi˜ ÂÚÁ·ÛÙËÚÈ·Îfi˜ ¤ÏÂÁ¯Ô˜ (·Û‚¤ÛÙÈÔ, ʈÛÊfiÚÔ˜, ÙÚ·ÓÛ·ÌÈÓ¿Û˜, Á-GT, ·ÎÙÈÓÔÁÚ·Ê›· ‰ÂÍÈ¿˜ ˯ÂÔηÚÈ΋˜ ¿ÚıÚˆÛ˘) ÁÈ· ÔÛÙÈ΋ ‹ Ë·ÙÈ΋ ÓfiÛÔ, Â¿Ó Ì¿ÏÈÛÙ· ÙÔ ·È‰› ÏËÚÔ› Ù· ËÏÈÎȷο ÎÚÈÙ‹ÚÈ· ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ Î·È Ôχ ÂÚÈÛÛfiÙÂÚÔ Â¿Ó Û˘Ó˘¿Ú¯ÂÈ ÈÛÙÔÚÈÎfi ÚÔËÁËı›۷˜ ÈÔÁÂÓÔ‡˜ ‹ ¿ÏÏ˘ Ïԛ̈͢ (ÌÔÏÔÓfiÙÈ ÙÔ ÙÂÏÂ˘Ù·›Ô ‰ÂÓ Â›Ó·È ·Ôχو˜ ··Ú·›ÙËÙÔ, ‰Ôı¤ÓÙÔ˜ fiÙÈ ·ÚÎÂÙ¤˜ ÏÔÈÌÒÍÂȘ ¤¯Ô˘Ó ˘ÔÎÏÈÓÈ΋ ÔÚ›·), ÙfiÙÂ Ë ·Ó·ÌÔÓ‹ Â› ÂÍ¿ÌËÓÔ Î·È Ô Â·Ó·ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù˘ ÙÈÌ‹˜ Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÙÔ˘ ÔÚÔ‡ Â›Ó·È Ë ϤÔÓ ÂӉ‰ÂÈÁ̤ÓË Ù·ÎÙÈ΋. ∂¿Ó, ‚‚·›ˆ˜, ÛÙÔÓ ÌÂÙ¿ ÂÍ¿ÌËÓÔ Â·Ó¤ÏÂÁ¯Ô Ù· Â›‰· Ù˘ ·ÏηÏÈ΋˜ ʈÛÊ·Ù¿Û˘ ÙÔ˘ ÔÚÔ‡ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ·Ó¢ڛÛÎÔÓÙ·È ·˘ÍË̤ӷ, ÙfiÙ ı· ··ÈÙËı› ÂÚ·ÈÙ¤Úˆ ÏÂÙÔÌÂÚ‹˜ ¤ÏÂÁ¯Ô˜ ÙfiÛÔ ÙÔ˘ ·ÛıÂÓÔ‡˜, fiÛÔ Î·È ÙˆÓ ÌÂÏÒÓ Ù˘ ÔÈÎÔÁ¤ÓÂÈ¿˜ ÙÔ˘.

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Paediatriki 2001;64:87-90

∏ ÂÚ›ÙˆÛË ÂÚÈÁÚ¿ÊÂÙ·È ÂÂȉ‹ Â›Ó·È ÛËÌ·ÓÙÈ΋ Ë ÁÓÒÛË Ù˘ ηÏÔ‹ıÔ˘˜ ·˘Ù‹˜ ÔÓÙfiÙËÙ·˜ ·fi ÙÔÓ ·ÛÎÔ‡ÓÙ· ·È‰›·ÙÚÔ, Ô ÔÔ›Ô˜ ‰ÂÓ ·ÔÎÏ›ÂÙ·È Ó· ÙË Û˘Ó·ÓÙ‹ÛÂÈ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÛÙ·‰ÈÔ‰ÚÔÌ›·˜ ÙÔ˘, ·ÊÔ‡ Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ·ÚÎÂÙ¿ Û˘¯Ó‹. ∞˘Ùfi ı· Û˘Ì‚¿ÏÂÈ ÛÙËÓ ·ÔÊ˘Á‹ Ù˘ ¿ÛÎÔ˘ Ù·Ï·ÈˆÚ›·˜ ÙfiÛÔ ÙÔ˘ ·È‰ÈÔ‡, fiÛÔ Î·È ÙÔ˘ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ ÙÔ˘ ÂÚÈ‚¿ÏÏÔÓÙÔ˜ Ì ÂÚÈÙÙ¤˜ ÂÍÂÙ¿ÛÂȘ, ·ÏÏ¿ Î·È Ì ÙÔ ¿Á¯Ô˜ ÂÓfi˜ Èı·ÓÔ‡, ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ· fï˜ ·Ó‡·ÚÎÙÔ˘ ÛÔ‚·ÚÔ‡ ÓÔÛ‹Ì·ÙÔ˜. µÈ‚ÏÈÔÁÚ·Ê›· 1. Bach U. Das Verhalten der alkalischen serum phosphatase bei Fruhgeborenen, Rachitikern and Spasmophilen. Z Kinderheilk 1954;74:593-603. 2. Schönau E, Herzog KH, Böhles HJ. Transient hyperphosphatasaemia of infancy. Eur J Pediatr 1988; 148:264-266. 3. Kruse K, Kurz N. Further evidence for infectious origin of isolated transient hyperphosphatasaemia. Eur J Pediatr 1989;148:453-454. 4. Crofton PM. What is the cause of benign transient hyperphosphatasaemia? A study of 35 cases. Clin Chem 1988;34/2:335-340. 5. Stein P, Rosalki SB, Foo Y, Hjelm M. Transient hyperphosphatasaemia of infancy and early childhood: Clinical and biochemical features of 21 cases and literature review. Clin Chem 1987;33/2:313-318. 6. Kruse K. Normal bone turnover in isolated hyperphosphatasaemia. J Pediatr 1985;106:946-948. 7. Nathan E. Transient hyperphosphatasaemia of infancy. Acta Paediatr Scand 1980;69:235-238. 8. Lockitch G, Pudek MR, Haltstead AC. Isolated elevation of serum alkaline phosphatase. J Pediatr 1984;105:773-775. 9. Frank U, Kruse K. Evidence for infectious origin of isolated transient hyperphosphatasemia. Eur J Pediatr 1985;143:323-324. 10. Krant JR, Metrick M, Maxwell NR, Kaplan MM. Isoenzyme studies in transient hyperphosphatasemia of infancy. Ten new cases and a review of the literature. AJDC 1985;139:736-740.

HÌÂÚÔÌËÓ›· ˘Ô‚ÔÏ‹˜: 22-06-2000 HÌÂÚÔÌËÓ›· ¤ÁÎÚÈÛ˘: 13-10-2000 ¢È‡ı˘ÓÛË ·ÏÏËÏÔÁÚ·Ê›·˜: ¡ÈÎfiÏ·Ô˜ °Î·Ì›Ï˘ ∫ÔÏÔÎÔÙÚÒÓË 28 187 56, ∫ÂÚ·ÙÛ›ÓÈ, ¶ÂÈÚ·È¿˜


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 91

¶ÂÚ›ÏË„Ë ¿ÚıÚÔ˘ ·fi ÙË µÈ‚ÏÈÔÁÚ·Ê›· £ÂÚ·›· Ù˘ ÓfiÛÔ˘ ÙÔ˘ Crohn Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ·ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ ÙÔ˘ ·Ú¿ÁÔÓÙ· Ó¤ÎÚˆÛ˘ ÙˆÓ fiÁÎˆÓ - ¿ÏÊ·1 ∂ÈÛ·ÁˆÁ‹: H ÓfiÛÔ˜ ÙÔ˘ Crohn ¤¯ÂÈ ·ÚÎÂÙ¿ Û˘¯Ó¿ Ùˆ¯‹ ·ÓÙ·fiÎÚÈÛË ÛÙË Û˘Ó‹ıË ıÂÚ·›· Ì ÎÔÚÙÈÎÔÂȉ‹ Î·È ·ÓÔÛÔηٷÛÙ·ÏÙÈο. ∏ ¯Ú‹ÛË ¯ÈÌ·ÈÚÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ ·Ú¿ÁÔÓÙ· Ó¤ÎÚˆÛ˘ ÙˆÓ fiÁÎˆÓ -¿ÏÊ· (TNF·) Û ÂÓ‹ÏÈΘ Ì ·ÓıÂÎÙÈ΋ ÛÙË ıÂÚ·›· ÓfiÛÔ ÙÔ˘ Crohn ¤¯ÂÈ ‰ÒÛÂÈ ÂÓı·ÚÚ˘ÓÙÈο ·ÔÙÂϤÛÌ·Ù·. ™ÎÔfi˜: ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ÂÎÙ›ÌËÛË Ù˘ ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ Ù˘ ¯ÔÚ‹ÁËÛ˘ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ TNF· (·ÓÙÈ-TNF·) Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ÂÓÂÚÁfi ÓfiÛÔ ÙÔ˘ Crohn. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: 19 ·ÛıÂÓ›˜, 9 ̤¯ÚÈ 19 ÂÙÒÓ, (̤ÛË ËÏÈΛ· 14,4 ¤ÙË) ¤Ï·‚·Ó 1 ˆ˜ 3 ÂÁ¯‡ÛÂȘ ·ÓÙÈ-TNF· (5mg/kg) Û ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· 12 ‚‰ÔÌ¿‰ˆÓ. ∂Ù¿ ·È‰È¿ ›¯·Ó ÎÔÚÙÈÎÔ·ÓıÂÎÙÈ΋ ÓfiÛÔ ÂÓÒ Ù· ˘fiÏÔÈ· ›¯·Ó ÎÔÚÙÈÎÔÂÍ·ÚÙÒÌÂÓË ÓfiÛÔ. ∏ ·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ ·È‰ÈÒÓ ¤ÁÈÓ Ì ηٷÁÚ·Ê‹ Ù˘ ÎÏÈÓÈ΋˜ ÂͤٷÛ˘ Î·È ÙÔ˘ ·È‰È·ÙÚÈÎÔ‡ ‰Â›ÎÙË ÂÓÂÚÁfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ (Pediatric Crohn Activity Index). ∞ÔÙÂϤÛÌ·Ù·: ™ÙȘ ÚÒÙ˜ 4 ‚‰ÔÌ¿‰Â˜ ÌÂÙ¿ ÙËÓ ÚÒÙË ¤Á¯˘ÛË ÔÈ ÙÈ̤˜ ÙÔ˘ ‰Â›ÎÙË ÂÓÂÚÁfiÙËÙ·˜ ÌÂÈÒıËÎ·Ó ÛËÌ·ÓÙÈο ·fi 42,1±13,7 Û 10±5,6 (p<0,001). ∆Ș ÂfiÌÂÓ˜ 8 ‚‰ÔÌ¿‰Â˜, 8 ·fi ÙÔ˘˜ 19 ·ÛıÂÓ›˜ ·ÚÔ˘Û›·Û·Ó Û¯ÂÙÈ΋ Âȉ›ӈÛË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Î·È Ë Ì¤ÛË ÙÈÌ‹ ÙÔ˘ ‰Â›ÎÙË ÂÓÂÚÁfiÙËÙ·˜ ÙË 12Ë Â‚‰ÔÌ¿‰· ‹Ù·Ó 26,8±16,4, ¯ˆÚ›˜ fï˜ ηÓ›˜ ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ Ó· ·ÚÔ˘ÛÈ¿ÛÂÈ ‚·-

ÚÂÈ¿ ÓfiÛÔ. ∏ ̤ÛË ËÌÂÚ‹ÛÈ· ‰fiÛË Ú‰ÓÈ˙fiÓ˘ ·Ú¯Èο, ÙËÓ 4Ë Â‚‰ÔÌ¿‰· Î·È ÙËÓ 12Ë Â‚‰ÔÌ¿‰· ‹Ù·Ó 28±14 mg, 20±12 mg Î·È 8±12 mg, ·ÓÙ›ÛÙÔȯ· (p<0,01). ∞ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ, fiˆ˜ ÂÍ¿ÓıËÌ· ‹ ‰‡ÛÓÔÈ·, ·ÚÔ˘Û›·Û·Ó 3 ·ÛıÂÓ›˜ ηٷ ÙË ‰È¿ÚÎÂÈ· Ù˘ ¤Á¯˘Û˘ ¯ˆÚ›˜ Ó· ¯ÚÂÈ·ÛÙ› ȉȷ›ÙÂÚË ·ÓÙÈÌÂÙÒÈÛË. ™˘ÌÂÚ¿ÛÌ·Ù·: ∏ ¯ÔÚ‹ÁËÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ TNF· Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ Ì ÂÓÂÚÁfi ÓfiÛÔ ÙÔ˘ Crohn ÚÔηÏ› ‚Ú·¯˘ÚfiıÂÛÌË ÎÏÈÓÈ΋ ‚ÂÏÙ›ˆÛË. ∏ ÁÚ‹ÁÔÚË Âȉ›ӈÛË Ù˘ ÂÓÂÚÁfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Û ÌÂÚÈÎÔ‡˜ ·ÛıÂÓ›˜ Û˘ÓËÁÔÚ› ÁÈ· ‰È·ÊÔÚÂÙÈο ‰ÔÛÔÏÔÁÈο Û¯‹Ì·Ù·, Ô˘ ›Ûˆ˜ Ú¤ÂÈ Ó· ÂÊ·ÚÌÔÛÙÔ‡Ó. ª·ÎÚ˘ÚfiıÂÛÌ· Ë ·ÛÊ¿ÏÂÈ· ÙÔ˘ Ê·ÚÌ¿ÎÔ˘ Â›Ó·È ¿ÁÓˆÛÙË Î·È ÁÈ· ·˘Ùfi Â›Ó·È ··Ú·›ÙËÙË Ë Û˘¯Ó‹ ·Ú·ÎÔÏÔ‡ıËÛË Î·È ¤ÏÂÁ¯Ô˜ ÙˆÓ ·ÛıÂÓÒÓ.

1Hyams

JS, Markowitz J, Wyllie R Use of infliximab in the treatment of Crohn’s disease in children and adolescents J Pediatr 2000;137:192-196 °ÈÒÙ· π. ∫·ÊÚ›ÙÛ· ¶·È‰›·ÙÚÔ˜ ¢È‰¿ÎÙˆÚ ¶·Ó/Ì›Ô˘ ∞ıËÓÒÓ

¶ÂÚ›ÏË„Ë ¿ÚıÚÔ˘ ·fi ÙË µÈ‚ÏÈÔÁÚ·Ê›· §Ô›ÌˆÍË ·fi ÂÏÈÎÔ‚·ÎÙËÚ›‰ÈÔ ÙÔ˘ ˘ÏˆÚÔ‡ Û ·Û˘Ìو̷ÙÈο ·È‰È¿: Û‡ÁÎÚÈÛË ‰È·ÁÓˆÛÙÈÎÒÓ ‰ÔÎÈÌ·ÛÈÒÓ1 ∂ÈÛ·ÁˆÁ‹: ∏ ·È‰È΋ ËÏÈΛ· ·ÔÙÂÏ› ÂÚ›Ô‰Ô ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÁÈ· Ïԛ̈ÍË ·fi ÂÏÈÎÔ‚·ÎÙËÚ›‰ÈÔ ÙÔ˘ ˘ÏˆÚÔ‡ (H. pylori). EȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ ÛÙÔÓ ·È‰ÈÎfi ÏËı˘ÛÌfi ÁÈ· ÙË Û˘¯ÓfiÙËÙ· Ù˘ Ïԛ̈͢ ÛÙËÚ›˙ÔÓÙ·È Û ‰È·ÁÓˆÛÙÈΤ˜ ‰ÔÎÈ̷ۛ˜ Ì ÔÈΛÏË ·ÍÈÔÈÛÙ›·. ™ÎÔfi˜: ∏ ·ÚÔ‡Û· ÌÂϤÙË ¤¯ÂÈ ÛÎÔfi ÙË Û‡ÁÎÚÈÛË ‰È·ÊfiÚˆÓ ‰È·ÁÓˆÛÙÈÎÒÓ ÌÂıfi‰ˆÓ ÁÈ· ÙËÓ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ Û˘¯ÓfiÙËÙ·˜ Ù˘ Ïԛ̈͢ ·fi H. pylori Û ·Û˘Ìو̷ÙÈο ·È‰È¿. ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ∆Ô ˘ÏÈÎfi Ù˘ ÌÂϤÙ˘ ·ÔÙ¤ÏÂÛ·Ó 287 ·È‰È¿ (151 ·ÁfiÚÈ·-136 ÎÔÚ›ÙÛÈ·) ËÏÈΛ·˜ 2-18 ÂÙÒÓ ¯ˆÚ›˜ Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ÂÙÈÎfi Û‡ÛÙËÌ·. ™Â οı ·È‰› ¤ÁÈÓ ÙËÓ ›‰È· Ë̤ڷ ÂͤٷÛ˘, ‰ÔÎÈÌ·Û›· ·Ó·ÓÔ‹˜ Ì C13 , ̤ÙÚËÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙÔ ·›Ì· Î·È Ì¤ÙÚËÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙË Û›ÂÏÔ. ∞ÔÙÂϤÛÌ·Ù·: §ÔÈ̈ÍË ·fi H. pylori ·ÓȯÓ‡ıËΠÛÙÔ 32%, 22% Î·È 18% ÙˆÓ ·È‰ÈÒÓ Ì ÙË ‰ÔÎÈÌ·Û›· ·Ó·ÓÔ‹˜, ·ÓÙÈÛÒÌ·Ù· ÛÙÔ ·›Ì· Î·È ·ÓÙÈÛÒÌ·Ù· ÛÙË Û›ÂÏÔ ·ÓÙ›ÛÙÔȯ·. 103 ·fi Ù· 287 ·È‰È¿ (35%) ›¯·Ó ¤ÛÙˆ Î·È Ì›· ‰ÔÎÈÌ·Û›· ıÂÙÈ΋ (92 ıÂÙÈ΋ ‰ÔÎÈÌ·Û›· ·Ó·ÓÔ‹˜, 8 ·ÓÙÈÛÒÌ·Ù· ÛÙÔ ·›Ì·, 3 ·ÓÙÈÛÒÌ·Ù· ÛÙË Û›ÂÏÔ). ªfiÓÔ 39 ·È‰È¿ (14%) ›¯·Ó Î·È ÙȘ ÙÚ›˜ ‰ÔÎÈ̷ۛ˜ ıÂÙÈΤ˜. £ÂˆÚÒÓÙ·˜ ÙË ‰ÔÎÈÌ·Û›· ·Ó·ÓÔ‹˜ Û·Ó Ì¤ıÔ‰Ô ÂÎÏÔÁ‹˜ Ë Â˘·ÈÛıËÛ›· Ù˘ ÌÂıfi‰Ô˘ ̤ÙÚËÛ˘ ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙÔ ·›Ì· Î·È ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙË Û›ÂÏÔ ‹Ù·Ó 48% Î·È 65% ·ÓÙ›ÛÙÔȯ·, ÂÓÒ

Ë ÂȉÈÎfiÙËÙ· Î·È ÙˆÓ ‰‡Ô ‰ÔÎÈÌ·ÛÈÒÓ ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚË ÙÔ˘ 95%. √ Û˘Ó‰˘·ÛÌfi˜ Ù˘ ̤ÙÚËÛ˘ ·ÓÙÈÛˆÌ¿ÙˆÓ ÛÙÔ ·›Ì· Î·È Û›ÂÏÔ Û˘ÁÎÚÈÙÈο Ì ÙË Ì¤ıÔ‰Ô ·Ó·ÓÔ‹˜ ›¯Â ÌÂÁ·Ï‡ÙÂÚË Â˘·ÈÛıËÛ›· (71%) Î·È ÂȉÈÎfiÙËÙ· (95%) ·’ fi,ÙÈ Ë Î¿ı ÂͤٷÛË Í¯ˆÚÈÛÙ¿. ™˘ÌÂÚ¿ÛÌ·Ù· : ™Â ·Û˘Ìو̷ÙÈο ·È‰È¿ , Ë Û˘¯ÓfiÙËÙ· Ù˘ Ïԛ̈͢ ·fi H. pylori ÔÈΛÏÂÈ Â˘Ú¤ˆ˜ ·Ó¿ÏÔÁ· Ì ÙË ‰È·ÁÓˆÛÙÈ΋ ‰ÔÎÈÌ·Û›· Ô˘ ¯ÚËÛÈÌÔÔÈ›ٷÈ. √È Ì¤ıÔ‰ÔÈ Ô˘ ‚·Û›˙ÔÓÙ·È Û ̤ÙÚËÛË ·ÓÙÈÛˆÌ¿ÙˆÓ Â›Ó·È ÏÈÁÒÙÂÚÔ ·ÍÈfiÈÛÙ˜ ·fi ÙË ‰ÔÎÈÌ·Û›· ·Ó·ÓÔ‹˜ Ì C13. ¶·Ú’ fiÏ· ·˘Ù¿, οو ·fi ÔÚÈṲ̂Ó˜ Û˘Óı‹Î˜, fiˆ˜ Ô Û˘Ó‰˘·ÛÌfi˜ ÌÂıfi‰ˆÓ Û ·›Ì· Î·È Û›ÂÏÔ, Ë Ì¤ÙÚËÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ÌÔÚ› Ó· ·ÔÙÂϤÛÂÈ ÌÈ· ÊıËÓ‹ ÂÓ·ÏÏ·ÎÙÈ΋ ÂͤٷÛË Û ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Û ·È‰È¿.

1Malaty

HM, Logan ND, Graham DY, Ramchatesingh JE, Reddy SG Helicobacter pylori infection in asymptomatic children: comparison of diagnostic tests Helicobacter 2000;5:155-159 °ÈÒÙ· π. ∫·ÊÚ›ÙÛ· ¶·È‰›·ÙÚÔ˜ ¢È‰¿ÎÙˆÚ ¶·Ó/Ì›Ô˘ ∞ıËÓÒÓ

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¶∞π¢π∞∆ƒπ∫∏ ∂¡∏ª∂ƒø™∏

PEADIATRIC NEWS

∞Á·ËÙÔ› ™˘Ó¿‰ÂÏÊÔÈ, ¶ÚÈÓ ·fi ÌÂÚÈο ¯ÚfiÓÈ· ÍÂΛÓËÛÂ Ë È‰¤· ÁÈ· ÙËÓ ¤ÓÙ·ÍË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ «¶∞π¢π∞∆ƒπ∫∏» ÛÙÔÓ Î·Ù¿ÏÔÁÔ ÙˆÓ ·Ó·ÁÓˆÚÈÛÌ¤ÓˆÓ Í¤ÓˆÓ ÂÚÈÔ‰ÈÎÒÓ ÛÙËÓ ∂ıÓÈ΋ µÈ‚ÏÈÔı‹ÎË π·ÙÚÈ΋˜ ÙˆÓ ∏.¶.∞. (National Library of Medicine). ª·˙› Ì ÙÔÓ ∫·ıËÁËÙ‹ Î. £ÂÌÈÛÙÔÎÏ‹ ∫·Ú¿ıÈÔ (ÙÔÓ ÙfiÙ ¢È¢ı˘ÓÙ‹ ŒÎ‰ÔÛ˘ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡) ηٷ‚¿ÏÏ·Ì οı ÚÔÛ¿ıÂÈ· ÁÈ· Ó· ÂÎÏËÚÒÛÔ˘Ì ÙÔ˘Ï¿¯ÈÛÙÔÓ ÌÂÚÈΤ˜ ·fi ÙȘ ÚÔ¸Ôı¤ÛÂȘ Ô˘ ··ÈÙÔ‡ÓÙ·Ó ÁÈ· ÙËÓ ·Ó·ÁÓÒÚÈÛË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ Ì·˜. ¢˘ÛÙ˘¯Ò˜, fï˜, ÔÈ ÚÔÛ¿ıÂȤ˜ Ì·˜ ‰ÂÓ Â˘Ô‰ÒıËηÓ. ™‹ÌÂÚ·, Ì ÙËÓ ¤Ó·ÚÍË ÌÈ·˜ ηÈÓÔ‡ÚÁÈ·˜ ¯ÈÏÈÂÙ›·˜, Ë Ó¤· ™˘ÓÙ·ÎÙÈ΋ ∂ÈÙÚÔ‹ ·ÔÊ¿ÛÈÛ ӷ Û˘Ó¯›ÛÂÈ ÙËÓ ÚÔÛ¿ıÂÈ·. ∞ÒÙÂÚÔ˜ ÛÎÔfi˜ Ì·˜ Â›Ó·È Ó· Û˘ÌÂÚÈÏËÊı› ÙÔ ÂÚÈÔ‰ÈÎfi Ì·˜ ÛÙÔ Science Citation Index (SCI) Î·È Ó· ·ÔÎÙ‹ÛÂÈ ÂÈÙ¤ÏÔ˘˜ ÙÔ ÁÓˆÛÙfi Impact Factor (™˘ÓÙÂÏÂÛÙ‹ ∂Ì‚¤ÏÂÈ·˜). ∫·Ù’·Ú¯¿˜, Ë ÂÌÊ¿ÓÈÛË ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ ı· Ú¤ÂÈ Ó· Â›Ó·È «ÊÈÏÈ΋» ÚÔ˜ ÙÔÓ Í¤ÓÔ ÂÈÛÙ‹ÌÔÓ· Ô˘ ‰ÂÓ ÁÓˆÚ›˙ÂÈ ÙËÓ ÂÏÏËÓÈ΋ ÁÏÒÛÛ·. ø˜ ÂÎ ÙÔ‡ÙÔ˘, Ô ¶›Ó·Î·˜ ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÛÙÔ ÂÍÒÊ˘ÏÏÔ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡ ı· ÂÚȤ¯ÂÈ Î·È ÛÙËÓ ∞ÁÁÏÈ΋ ÁÏÒÛÛ· ÙÔ˘˜ Ù›ÙÏÔ˘˜ ÙˆÓ ‰ËÌÔÛÈÂ˘Ì¤ÓˆÓ ¿ÚıÚˆÓ Î·ıÒ˜ Î·È Ù· ÔÓfiÌ·Ù· ÙˆÓ Û˘ÁÁڷʤˆÓ. ∂ÈϤÔÓ, ıˆÚÂ›Ù·È ··Ú·›ÙËÙÔ Ó· ÂΉ›‰ÂÙ·È ¤ÁηÈÚ· ÙÔ Î¿ı Ù‡¯Ô˜ ÙÔ˘ ÂÚÈÔ‰ÈÎÔ‡, Ô˘ ÛËÌ·›ÓÂÈ fiÙÈ Ë fiÏË ‰È·‰Èηۛ· ·ÍÈÔÏfiÁËÛ˘ ÙˆÓ ÂÚÁ·ÛÈÒÓ Î·È ÔÈ Û¯ÂÙÈΤ˜ ‰ÈÔÚıÒÛÂȘ ·fi ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜ ı· Ú¤ÂÈ Ó· ÎÈÓÂ›Ù·È ÂÌÚfiıÂÛÌ·. ∆¤ÏÔ˜, ÁÈ· Ó· Á›ÓÂÈ ÙÔ ÂÚÈÔ‰ÈÎfi Ì·˜ ÈÔ ÚÔÛÈÙfi ÛÙÔÓ Â˘Ú‡ÙÂÚÔ ÂÈÛÙËÌÔÓÈÎfi ¯ÒÚÔ, ÛÎÔ‡ԢÌ ӷ ÙÔ ÂÓÙ¿ÍÔ˘Ì Ôχ ÁÚ‹ÁÔÚ· Î·È ÛÙÔ Internet. °È· ÙËÓ Â›Ù¢ÍË ÙˆÓ ·Ú·¿Óˆ ÛÙfi¯ˆÓ Ì·˜ ¯ÚÂÈ·˙fiÌ·ÛÙ ÙËÓ ˘ÔÛÙ‹ÚÈÍË Î·È Û˘ÓÂÚÁ·Û›· fiÏˆÓ Û·˜. √È ÌÂÓ ÎÚÈÙ¤˜ ı· Ú¤ÂÈ Ó· ·ÔÛÙ¤ÏÏÔ˘Ó ÙȘ ·Ú·ÙËÚ‹ÛÂȘ ÙÔ˘˜ ¤ÁηÈÚ·, ÔÈ ‰Â Û˘ÁÁÚ·Ê›˜ ı· Ú¤ÂÈ Ó· ÙËÚÔ‡Ó ·˘ÛÙËÚ¿ Ù· ÚÔÙÂÈÓfiÌÂÓ· ¯ÚÔÓÈο Ï·›ÛÈ· ÁÈ· ÙË ‰ÈfiÚıˆÛË ÙÔ˘ ÎÂÈ̤ÓÔ˘ ÙÔ˘˜ ηıÒ˜ Î·È ÙÔ˘ ÚÒÙÔ˘ Ù˘ˆÌ¤ÓÔ˘ ‰ÔÎÈÌ›Ô˘. ∂¿Ó ·ÓÙÈÌÂÙˆ›ÛÔ˘Ì fiÏÔÈ Ì·˙› ·˘Ù‹ ÙËÓ ÚfiÎÏËÛË, Â›Ì·È Û›ÁÔ˘ÚË fiÙÈ ı· ÂÈÙ‡¯Ô˘ÌÂ. ∂Π̤ÚÔ˘˜ Ù˘ ™˘ÓÙ·ÎÙÈ΋˜ ∂ÈÙÚÔ‹˜ ∑ˆ‹ ¶··‰ÔÔ‡ÏÔ˘-∫Ô˘ÏÔ˘Ì‹

∞Ó·ÎÔ›ÓˆÛË ¶ÚÔ˜ fiÏ· Ù· ̤ÏË Ù˘ ∂ÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ ∂Ù·ÈÚ›·˜ ∫‡ÚÈÔÈ ™˘Ó¿‰ÂÏÊÔÈ, °È· Ó· ÌÔÚ¤ÛÂÈ Ë ÂÙ·ÈÚ›· Ì·˜ Ó· ÂÎÏËÚÒÛÂÈ ÙÔ˘˜ ÛÎÔÔ‡˜ Ù˘ Î·È Ó· ηχ„ÂÈ ÙȘ ·Ó¿ÁΘ ÏÂÈÙÔ˘ÚÁ›·˜ Ù˘ ‚·Û›˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙÔ˘˜ fiÚÔ˘˜ ·fi ÙȘ Û˘Ó‰ÚÔ̤˜ ÙˆÓ ÌÂÏÒÓ Ù˘. ∏ ·ÓÂÍ·ÚÙËÛ›· Î·È Ë ·˘ÙÔÓÔÌ›· Ù˘ ¤¯Ô˘Ó Û·Ó ·˘ÙÔÓfiËÙË ÚÔ¸fiıÂÛË ÙËÓ ÔÈÎÔÓÔÌÈ΋ ·ÓÂÍ·ÚÙËÛ›· Ù˘ Î·È ÁÈ· ÙÔÓ ÏfiÁÔ ·˘Ùfi, ÙÔ ¢ÈÔÈÎËÙÈÎfi ™˘Ì‚Ô‡ÏÈÔ Ù˘ ∂ÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ ∂Ù·ÈÚ›˜ (∂.¶.∂.) Ì ÁÚ·Ù‹ ˘fiÌÓËÛ‹ ÙÔ˘, ˙‹ÙËÛ ·fi Ù· ̤ÏË ÙÔ˘, Ì ÂÈÛÙÔϤ˜ Ô˘ ÙÔ˘˜ ·ˇı˘ÓÂ, ÙËÓ ÔÈÎÔÓÔÌÈ΋ ÙÔ˘˜ Ù·ÎÙÔÔ›ËÛË ÚÔÎÂÈ̤ÓÔ˘ Ë ∂Ù·ÈÚ›· Ì·˜ Ó· ÌÔÚ› ·˘ÙÔ‰‡Ó·ÌË Ó· ηχ„ÂÈ ÙȘ ‰·¿Ó˜ ÏÂÈÙÔ˘ÚÁ›·˜ Ù˘ Î·È Ó· ÂȉÈÒÎÂÈ Ù˘ ÂÎÏ‹ÚˆÛË ÙˆÓ ÛÎÔÒÓ Ù˘. ∫‡ÚÈÔÈ ™˘Ó¿‰ÂÏÊÔÈ, ∆Ô ¢ÈÔÈÎËÙÈÎfi ™˘Ì‚Ô‡ÏÈÔ Ù˘ ∂.¶.∂. Û·˜ ηÏ› Ó· ·ÓÙ·ÔÎÚÈı›Ù ÛÙËÓ ¤ÎÎÏËÛË Ô˘ Û·˜ ·ˇı˘Ó ̤ۈ ÙˆÓ ˆ˜ ¿Óˆ ÂÈÛÙÔÏÒÓ.

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¶PO™EXH ™YNE¢PIA

2-4 ºÂ‚ÚÔ˘·Ú›Ô˘

First World Congress on the Fetal Origins of Adult Disease ¶ÏËÚÔÊÔڛ˜: Ms Alifiya Motiwala Tel.: +91 22 651 6439 Fax: +91 22 6516438 e-mail: mrcssc@vsnl.com website: www.sneha-india.org

Bombay, India

10-11 ºÂ‚ÚÔ˘·Ú›Ô˘

International Symposium on the West Syndrome and Related Infantile Epileptic Encephalopathies (ISWS) ¶ÏËÚÔÊÔڛ˜: Yukio Fukuyama Secretariat, ISWS C/o Child Neurology Institute Samban-cho TY Plaza, SFL 24 Samban-cho, Chiyoda-Ku Tokyo 102-0075 Japan Tel.: +81 3 3238 1580 Fax: +81 3 3238 1502 e-mail: yfukuyam@sc4.so-net.ne.jp

Tokyo, Japan

8-10 ª·ÚÙ›Ô˘

4Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ∞ÏÏÂÚÁÈÔÏÔÁ›·˜ Î·È ∫ÏÈÓÈ΋˜ ∞ÓÔÛÔÏÔÁ›·˜ ¶ÏËÚÔÊÔڛ˜: ∂æπ§√¡ ∆ËÏ.: 01 7254 360-2 Fax: 01 7254 363 e-mail: epsilonb@hol.gr

Holiday Inn, ∞ı‹Ó·

15-17 ª·ÚÙ›Ô˘

5th European Postgraduate Course in Neonatal and Pediatric Intensive care ¶ÏËÚÔÊÔڛ˜: BBS Congress GmbH Barbara Bülmann P.O. Box, CH-3000 Barn 25, Switzerland ∆ËÏ.: +41 31 331 8275 Fax: +41 31 332 9879 e-mail: bbscongress@swissonline.ch

Barn, ∂Ï‚ÂÙ›·

26-28 ª·ÚÙ›Ô˘

European Society for Pediatric Infectious Diseases (ESPID) Secretariat: PO Box 50006 Tel Aviv 61500, Israel Tel.: +972 3 5140000 Fax: +972 3 51 40077 e-mail: espid2001@kenes.com website: http://www.kenes.com/espid2001

Eilat, Israel


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 94

¶PO™EXH ™YNE¢PIA

17-20 ∞ÚÈÏ›Ô˘

22nd Course of Pediatric Dermatology ¶ÏËÚÔÊÔڛ˜: e-mail: alain.taieb@dermatol.u-bordeaux2.fr

Arcachon, °·ÏÏ›·

28 ∞ÚÈÏ›Ô˘ – 1 ª·˝Ô˘

Pediatric Academic Societies Annual Meeting Baltimore Convention Center Baltimore, Maryland ¶ÏËÚÔÊÔڛ˜: Program Office ∆ËÏ.: ++281 419 0052 Fax: ++281 419 0082 e-mail: info@aps-spr.org

µ·ÏÙÈÌfiÚË, ∏¶∞

5-6 ª·˝Ô˘

10Ë ¢ÈËÌÂÚ›‰· ¶·È‰È·ÙÚÈÎÒÓ ∞Ó·Ó¢ÛÙÈÎÒÓ ¶·ı‹ÛÂˆÓ ∂Î·È‰Â˘ÙÈÎfi ∫¤ÓÙÚÔ ∞ÁÚÔÙÈ΋˜ ∆Ú¿Â˙·˜, ¡. ∂Ú˘ıÚ·›·, ∞ı‹Ó· √ÚÁ¿ÓˆÛË: ∞ÏÏÂÚÁÈÔÏÔÁÈÎfi ∆Ì‹Ì· °¡¶¶ ™˘ÓÂÚÁ·Û›·: ∂ÏÏËÓÈ΋ ¶·È‰ÔÓ¢ÌÔÏÔÁÈ΋ ∂Ù·ÈÚ›· ¶ÏËÚÔÊÔڛ˜: ∞ÏÏÂÚÁÈÔÏÔÁÈÎfi ∆Ì‹Ì· °¡¶¶ 152 36, ¶. ¶ÂÓÙ¤ÏË ∆ËÏ.: 01 80 36 472 Fax: 01 61 31 173 e-mail: kpriftis@otenet.gr

∞ı‹Ó·

18-20 ª·˝Ô˘

13Ô ¶·ÓÂÏÏ‹ÓÈÔ ™˘Ó¤‰ÚÈÔ ∂ÏÏËÓÈ΋˜ ∂Ù·ÈÚ›·˜ ∫ÔÈÓˆÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ Î·È ¶ÚÔ·ÁˆÁ‹˜ Ù˘ ÀÁ›·˜ £¤Ì·: π·ÙÚÔÎÔÈÓˆÓÈΤ˜ ÂÈÙÒÛÂȘ ÙˆÓ ÏÔÈ̈‰ÒÓ ÓÔÛËÌ¿ÙˆÓ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ¶ÏËÚÔÊÔڛ˜: ∂ϤÓË ¶ÂÙÚ›‰Ô˘ ∂ÚÁ·ÛÙ‹ÚÈÔ ÀÁÂÈÈÓ‹˜ Î·È ∂ȉËÌÈÔÏÔÁ›·˜ π·ÙÚÈ΋ ™¯ÔÏ‹ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ª. ∞Û›·˜ 75, 115 27, ∞ı‹Ó· ∆ËÏ.: 775 9962 – 0944 974249 Fax: 777 3840 – 748 5872 ∏ÏÂÎÙÚÔÓÈ΋ ‰È‡ı˘ÓÛË Ù˘ ∂Ù·ÈÚ›·˜ ÁÈ· ˘Ô‚ÔÏ‹ ÂÚÈÏ‹„ˆÓ: http://www.socped.gr ‹ e-mail: epetrid@cc.uoa.gr

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2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 95

™YNTOMO°PAºIE™

ABBREVIATIONS

™˘ÓÙÔÌÔÁڷʛ˜

EÏÏËÓÈÎÔ› fiÚÔÈ

AÁÁÏÈÎÔ› fiÚÔÈ

AIDS

Û‡Ó‰ÚÔÌÔ Â›ÎÙËÙ˘ ·ÓÔÛÔ·ÓÂ¿ÚÎÂÈ·˜

AMP, ADP, ATP

ÌÔÓÔ-, ‰È-, ÙÚÈʈÛÊÔÚÈ΋ ·‰ÂÓÔÛ›ÓË

ATPase cal CSF ‹ ENY CNS ‹ ∫¡™ cm CoA cmÑ cm2 cAMP ÆC CRP DNA ECG ‹ ∏∫° EEG ‹ ∏∂° ELISA ESR ‹ ∆∫∂ EDTA g Hct Hb HLA

ÙÚÈʈÛÊ·Ù¿ÛË Ù˘ ·‰ÂÓÔÛ›Ó˘ ıÂÚÌ›‰· ÂÁÎÂÊ·ÏÔÓˆÙÈ·›Ô ˘ÁÚfi ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ· ÂηÙÔÛÙfi Û˘Ó¤Ó˙˘ÌÔ ∞ ΢‚ÈÎfi ÂηÙÔÛÙfi ÙÂÙÚ·ÁˆÓÈÎfi ÂηÙÔÛÙfi ·ÎÏÈÎfi AMP ‚·ıÌfi˜ KÂÏÛ›Ô˘ C-·ÓÙȉÚÒÛ· ÚˆÙ½ÓË ‰ÂÔ͢ÚÈ‚Ô˙ÔÓÔ˘ÎÏÂ˚ÓÈÎfi Ô͇ ËÏÂÎÙÚÔηډÈÔÁÚ¿ÊËÌ· ËÏÂÎÙÚÔÂÁÎÂÊ·ÏÔÁÚ¿ÊËÌ· ¤Ó˙˘ÌÔ-Û˘Ó‰¤Ù˘ ·ÓÔÛÔÚÔÛÚfiÊËÛ˘ ÚÔÛ‰ÈÔÚÈÛÌfi˜ Ù·¯‡ÙËÙ· ηıÈ˙‹Ûˆ˜ ÂÚ˘ıÚÒÓ Âı˘ÏÂÓԉȷÌÈÓÔÙÂÙÚ·ÔÍÂ˚Îfi Ô͇ ÁÚ·ÌÌ¿ÚÈo ·ÈÌ·ÙÔÎÚ›Ù˘ ·ÈÌÔÛÊ·ÈÚ›ÓË ·ÓÙÈÁfiÓÔ ÈÛÙÔÛ˘Ì‚·ÙfiÙËÙ·˜

Ig ICU ‹ ª∂£ IU i.m. ‹ ∂.ª. i.p. ‹ ∂.¶. i.v. ‹ ∂.º. L log mol ªW ‹ MB MCH MCV mRNA m min n N NS ‹ ª™ Ôsm % PCR pH PCO2 PO2 p

·ÓÔÛÔÛÊ·ÈÚ›ÓË ÌÔÓ¿‰· ÂÓÙ·ÙÈ΋˜ ıÂÚ·›·˜ ‰ÈÂıÓ‹˜ ÌÔÓ¿‰· ÂÓ‰ÔÌ˘˚ο ÂÓ‰ÔÂÚÈÙÔÓ·˚ο ÂÓ‰ÔÊϤ‚È· Ï›ÙÚÔ(·) ÏÔÁ¿ÚÈıÌÔ˜ ÁÚ·ÌÌÔÌfiÚÈÔ ÌÔÚÈ·Îfi ‚¿ÚÔ˜ ̤ÛË ·ÈÌÔÛÊ·ÈÚ›ÓË ÂÚ˘ıÚÒÓ Ì¤ÛÔ˜ fiÁÎÔ˜ ÂÚ˘ıÚÒÓ ·ÁÁÂÏÈÔÊfiÚÔ˜ RNA ̤ÙÚÔ ÏÂÙfi ·ÚÈıÌfi˜ Ó¢ÙÒÓÈÔ ÌË ÛËÌ·ÓÙÈÎfi ÔÛÌÒÏÈÔ Â› ÙÔȘ ÂηÙfi ·Ï˘ÛȉˆÙ‹ ·ÓÙ›‰Ú·ÛË ÔÏ˘ÌÂÚ¿Û˘ Û˘ÁΤÓÚˆÛË ÈfiÓÙˆÓ ˘‰ÚÔÁfiÓÔ˘ ÌÂÚÈ΋ Ù¿ÛË CO2 ÌÂÚÈ΋ Ù¿ÛË Ô͢ÁfiÓÔ˘ Èı·ÓfiÙËÙ·

acquired immunodeficiency syndrome adenosine 5mono-, di-, triphosphate adenosine triphosphatase calorie cerebrospinal fluid central nervous system centimeter coenzyme A cubic centimeter square centimeter cyclic AMP degree(s) Celsius C-reactive protein deoxyribonucleic acid electrocardiogram electroencephalogram enzyme-linked immunoabsorbent assay erythrocyte sedimentation rate ethylenediaminetetracetate gram haematocrit haemoglobin (human) histocompatibility leucocyte antigen immunoglobulin intensive care unit international Unit intramuscular intraperitoneal intravenous liter logarithm mole molecular weight mean corpuscular haemoglobin mean corpuscular volume messenger RNA meter minute number newton not significant osmole percent polymerase chain reaction potentia hydrogenii carbon dioxide pressure oxygen pressure probability


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 96

™˘ÓÙÔÌÔÁڷʛ˜

EÏÏËÓÈÎÔ› fiÚÔÈ

AÁÁÏÈÎÔ› fiÚÔÈ

p.o. RIA RNA RDS sec s.c. ‹ À.¢. SD SE U

·fi ÙÔ ÛÙfiÌ· Ú·‰ÈÔ·ÓÔÛÔ·ÔÚÚÔÊËÙÈ΋ ‰ÔÎÈÌ·Û›· ÚÈ‚ÔÓÔ˘ÎÏÂ˚ÓÈÎfi Ô͇ Û‡Ó‰ÚÔÌÔ ·Ó·Ó¢ÛÙÈ΋˜ ‰˘Û¯¤ÚÈ·˜ ‰Â˘ÙÂÚfiÏÂÙÔ ˘Ô‰fiÚÈ· ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË ÛÙ·ıÂÚfi ÛÊ¿ÏÌ· ÌÔÓ¿‰·

by mouth radioimmunoassay ribonucleic acid respiratory distress syndrome second subcutaneous standard deviation standard error unit

™˘Ó‰˘·˙fiÌÂÓ· ÚÔı¤Ì·Ù· 12

tera- (10 ) 9 giga- (10 ) 6 mega-(10 ) 3 kilo- (10 ) 2 hecto(10 ) 1 deca (10 ) -1 deci (10 ) -2 centi- (10 ) -3 milli(10 ) -5 micro (10 ) -9 nano (10 ) -12 pico (10 ) -15 femto (10 ) -18 atto (10 )

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


2001 paid(teliko) 17-10-01 15:46 ™ÂÏ›‰· 97

™˘ÓÙ·ÁÔÁÚ·ÊÈΤ˜ ¶ÏËÚÔÊÔڛ˜ ¢È·ÊËÌÈ˙fiÌÂÓˆÓ ¶ÚÔ˚fiÓÙˆÓ

CECLOR: ¶∂ƒπ§∏æ∏ Ã∞ƒ∞∫∆∏ƒπ™∆π∫ø¡ ¶ƒO´O¡∆O™ ∂N¢EI•EI™: ∆Ô CECLOR® ÂӉ›ÎÓ˘Ù·È ÛÙË ıÂÚ·›· ÙˆÓ ·ÎfiÏÔ˘ıˆÓ ÏÔÈÌÒ͈Ó, fiÙ·Ó ·˘Ù¤˜ ÚÔηÏÔ‡ÓÙ·È ·fi Ù· ·Ó·ÊÂÚfiÌÂÓ· ηو٤ڈ ÛÙÂϤ¯Ë ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ ÂÊfiÛÔÓ ·˘Ù¿ Â›Ó·È Â˘·›ÛıËÙ· Û ·˘Ùfi: §ÔÈÌÒÍÂȘ ÙˆÓ ·ÓˆÙ¤ÚˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Ô‰ÒÓ. ª¤ÛË ˘Ò‰Ë˜ ˆÙ›Ùȉ·: ÚÔηÏÔ‡ÌÂÓË ·fi S. pneumoniae, H. influenzae, Staphylococci (ÂÍ·ÈÚÔ˘Ì¤ÓˆÓ ÙˆÓ ÛÙÂϯÒÓ Ô˘ Â›Ó·È ·ÓıÂÎÙÈο ÛÙË ÌÂıÈÎÈÏÏ›ÓË), ‚-·ÈÌÔÏ˘ÙÈÎfi ™ÙÚÂÙfiÎÔÎÎÔ ÔÌ¿‰·˜ ∞ Î·È ª. catarrhalis. º·Ú˘ÁÁ›Ùȉ· Î·È ·Ì˘Á‰·Ï›Ùȉ·, ·fi ‚-·ÈÌÔÏ˘ÙÈÎfi ™ÙÚÂÙfiÎÔÎÎÔ ÔÌ¿‰·˜ ∞.¶·Ú·ÚÚÈÓÔÎÔÏ›Ùȉ· ·fi ¢·›ÛıËÙ· ÛÙÂϤ¯Ë H. influenzae ( ‚-Ï·ÎÙ·Ì¿ÛË ±), ‚-·ÈÌÔÏ˘ÙÈÎfi ÛÙÚÂÙfiÎÔÎÎÔ ÔÌ¿‰·˜ ∞, S. pneumoniae, M. catarrhalis Î·È S. aureus ( ‚-Ï·ÎÙ·Ì¿Û˘ ±). ™ÙË ¯ÚfiÓÈ· ÌÔÚÊ‹ ··ÈÙÂ›Ù·È Û˘Ó‹ıˆ˜ ÚÔÛı‹ÎË Î·È ÂÓfi˜ ¿ÏÏÔ˘ ·ÓÙÈÌÈÎÚÔ‚È·ÎÔ‡ ·Ú¿ÁÔÓÙ·, ‰Ú·ÛÙÈÎÔ‡ ÛÙÔ˘˜ ·Ó·ÂÚfi‚ÈÔ˘˜ ÔÚÁ·ÓÈÛÌÔ‡˜. §ÔÈÌÒÍÂȘ ÙˆÓ Î·ÙˆÙ¤ÚˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ Ô‰ÒÓ: √Í›· ‚ÚÔÁ¯›Ùȉ·, Î·È ÔÍ›˜ ÂÍ¿ÚÛÂȘ ¯ÚfiÓÈ·˜ ‚ÚÔÁ¯›Ùȉ·˜ Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi S. pneumoniae, H. influenzae (‚-Ï·ÎÙ·Ì¿ÛË ±), H. parainfluenzae, M. catarrhalis (‚-Ï·ÎÙ·Ì¿ÛË ±), S. aureus. ¶Ó¢ÌÔÓ›· Û ·ÛıÂÓ›˜ Ù˘ ÎÔÈÓfiÙËÙ·˜ Ô˘ ÚÔηÏÂ›Ù·È ·Ô S. pneumoniae, H. influenzae (‚-Ï·ÎÙ·Ì¿Û˱) Î·È M. catarrhalis(‚-Ï·ÎÙ·Ì¿Û˱). ªË ÂÈÂÏÂÁ̤Ó˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ Ô˘ÚÔÔÈÔÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, Û˘ÌÂÚÈÏ·Ì‚·ÓÔÌ¤ÓˆÓ Ù˘ ˘ÂÏÔÓÂÊÚ›Ùȉ·˜ Î·È Î˘ÛÙ›Ùȉ·˜, (Ì ٷ˘Ùfi¯ÚÔÓË Î¿Ï˘„Ë ÁÈ· Ù· ·Ó·ÂÚfi‚È· ) Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi ∂. coli, P. mirabilis, Klebsiella spp. §ÔÈÌÒÍÂȘ ÙÔ˘ ‰¤ÚÌ·ÙÔ˜ Î·È Ì·Ï·ÎÒÓ ÌÔÚ›ˆÓ, Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi Staphylococcus aureus(‚-Ï·ÎÙ·Ì¿Û˱) Î·È ‚-·ÈÌÔÏ˘ÙÈÎÒÓ ÛÙÚÂÙÔÎfiÎÎˆÓ ÔÌ¿‰·˜ ∞. ANTEN¢EI•EI™: °ÓˆÛÙ‹ ˘ÂÚ¢·ÈÛıËÛ›· ÛÙË ÎÂÊ·ÎÏfiÚË Î·È ¿ÏϘ ÎÂÊ·ÏÔÛÔÚ›Ó˜ ηıÒ˜ Î·È ÈÛÙÔÚÈÎfi ·Ó·Ê˘Ï·ÎÙÈ΋˜ ·ÓÙ›‰Ú·Û˘ ÛÙȘ ÂÓÈÎÈÏϛӘ. ∞¡∂¶π£Àª∏∆∂™ ∂¡∂ƒ°∂π∂™: ∞ÏÏÂÚÁÈΤ˜ ·ÓÙȉڿÛÂȘ (‰È·ÛÙ·˘ÚÔ‡ÌÂÓË ·ÏÏÂÚÁ›· Ì ÙȘ ÂÓÈÎÈÏϛӘ10%). ¡·˘Ù›·,¤ÌÂÙÔÈ, ‰È¿ÚÚÔȘ, ÂÈÁ·ÛÙÚÈÎfi˜ ÊfiÚÙÔ˜. ∞§§∏§∂¶π¢ƒ∞™∂π™: ∏ Û‡Á¯ÚÔÓË ¯ÔÚ‹ÁËÛË ÚÔ‚ÂÓÂÛ›‰Ë˜ ·Ó·ÛÙ¤ÏÏÂÈ ÙËÓ ·¤ÎÎÚÈÛË ÙÔ˘ CECLOR® ·fi ÙÔ˘˜ ÓÂÊÚÔ‡˜ fiˆ˜ Û˘Ì‚·›ÓÂÈ Î·È Ì ¿ÏÏ· ·ÓÙÈ‚ÈÔÙÈο. ™‡Á¯ÚÔÓË ¯ÔÚ‹ÁËÛË Ì ÙÂÙڷ΢ÎϛӘ ‹ ¯ÏˆÚ·ÌÊÂÓÈÎfiÏË, ¤¯ÂÈ ·ÓÙ·ÁˆÓÈÛÙÈÎfi ·ÔÙ¤ÏÂÛÌ·. ŸÙ·Ó ¯ÔÚËÁÂ›Ù·È Û ÌÂÁ¿Ï˜ ‰fiÛÂȘ Ì·˙› Ì ¿ÏÏ· ÓÂÊÚÔÙÔÍÈο Ê¿Ú̷η, fiˆ˜ ·ÌÈÓÔÁÏ˘ÎÔÛ›‰Â˜, ·˘Í¿ÓÂÙ·È Ô Î›Ó‰˘ÓÔ˜ ÓÂÊÚÔÙÔÍÈÎfiÙËÙ·˜ ¢√™√§√°π∞: XÔÚËÁÂ›Ù·È ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜, Ì ‹ ¯ˆÚ›˜ Ê·ÁËÙfi. ∂Ó‹ÏÈΘ: 250 mg οı 8 ÒÚ˜, ‰ÈÏ·ÛÈ·˙fiÌÂÓË Û ÛÔ‚·Ú¤˜ ÏÔÈÌÒÍÂȘ. M¤ÁÈÛÙË ‰fiÛË ÁÈ· ÙÔ˘˜ ÂÓ‹ÏÈΘ 4 gr ËÌÂÚËÛ›ˆ˜ ¶·È‰È¿: 20-40 mg/kg ËÌÂÚËÛ›ˆ˜ ‰È·ÈÚÂ̤ÓË Û ÙÚÂȘ ›Û˜ ‰fiÛÂȘ. ™ÙË ıÂÚ·›· Ù˘ ̤Û˘ ˆÙ›Ùȉ·˜ Î·È Ê·Ú˘ÁÁ›Ùȉ·˜, Ë Û˘ÓÔÏÈ΋ ËÌÂÚ‹ÛÈ· ‰fiÛË ÌÔÚ› Ó· ‰È·ÈÚÂı› Î·È Ó· ¯ÔÚËÁËı› ·Ó¿ 12ˆÚÔ. ª¤ÁÈÛÙË Û˘ÓÈÛÙÒÌÂÓË ‰fiÛË ÛÙ· ·È‰È¿ Â›Ó·È 1 gr ËÌÂÚËÛ›ˆ˜. ªOƒº∂™: Susp 125mg/5ml, 60ml, Susp 250mg/5ml, 60ml, Susp 375mg/5ml, 60ml, Caps 500mg Û ÎÔ˘Ù› ÙˆÓ 12. ¶ÔÛÔÛÙfi ÂȯÔÚ‹ÁËÛ˘ ·fi Ù· Ù·Ì›·: 75 %


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