86
¢ÈÌËÓÈ·›· ¤Î‰ÔÛË Ù˘ EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜ ¶Úfi‰ÚÔ˜ ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ ™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹ ¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘ ∫. ™ÙÂÊ·Ó›‰Ë˜ M¤ÏË ™. ∞Ó‰ÚÔÓ›ÎÔ˘ ª. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜ ¶. ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘ °. µ·ÚÏ¿Ì˘ ∂. °·Ï·Ó¿Î˘ §. £ˆÌ·˝‰Ô˘ ª. ∫·Ó¿ÚÈÔ˘ A. K·ÙÙ¿Ì˘ ™. K›ÙÛÈÔ˘-∆˙¤ÏË ∞. ¶··‰ÔÔ‡ÏÔ˘ µ. ¶··Â˘·ÁÁ¤ÏÔ˘ ∞. ™È·ÌÔÔ‡ÏÔ˘-ª·˘Ú›‰Ô˘ ∞. ™˘Ú›ÁÔ˘-¶··‚·ÛÈÏ›Ԣ ∂. ÷ÚÌ·Ó‰¿ÚË ÀÔ‚ÔÏ‹ ∂ÚÁ·ÛÈÒÓ e-mail: [email protected] √‰ËÁ›Â˜ ÚÔ˜ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜: http://www.e-child.gr/Instructions_ to_Authors_GR.pdf ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ· ∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ∫. ¶··¯Ú‹ÛÙÔ˘ EÈ̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈÌ¤ÓˆÓ ™. ¡¿ÎÔ˘ EΉfiÙ˘ K. °ÚÈ‚¤·˜ ™˘ÓÙÔÓÈÛÙ‹˜ ∂ΉfiÛˆ˜ Communications In Practice ∞.∂. ¶ÈÂÚ›·˜ 1∞ 144 51 MÂÙ·ÌfiÚʈÛË TËÏ.: 210 87 78 884 Fax: 210 87 78 822 I‰ÈÔÎÙ‹Ù˘ EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›· © Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92 Aı‹Ó· 115 28 TËÏ.: 210 7771 140 210 7771 663 Fax: 210 7758 354 e-mail: [email protected] EÙ‹ÛÈ· ™˘Ó‰ÚÔÌ‹: 40 EȉÈ΢fiÌÂÓÔÈ, ºÔÈÙËÙ¤˜: 20 ¶ÂÚȯfiÌÂÓ· ∞¡∞™∫O¶∏™∂π™ 75 √Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹ 82 §ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ - ¡ÂfiÙÂÚ· ıÂڷ¢ÙÈο ‰Â‰Ô̤ӷ µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹ 89 ∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË Ã. ∫Ô˘ÙÛ·˘Ù›ÎË, ∂. ª¿ÓÙ˙ÈÔ˘, ¡. ª˘ÚÈÔÎÂÊ·ÏÈÙ¿Î˘ 97 ¡ÂfiÙÂÚ· ‰Â‰Ô̤ӷ ÁÈ· ÙË Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ã. ∆۷ηϛ‰Ë˜, ∂. √˘ÚÁηÓÙ˙fiÁÏÔ˘, ¡. ¡ÈÎÔÏ·˝‰Ë˜ ∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™ 109 ∂›‰Ú·ÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì˘ÂÏÔ‡ ÔÛÙÒÓ ÛÙËÓ ex vivo ¤ÎÙ˘ÍË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜ π. ¶ÂÏ·ÁÈ¿‰Ë˜, ∂. ¢ËÌËÙÚ›Ô˘, ∂. ™ÙÂȷοÎË, Ã. ¶ÂÚ‰ÈÎÔÁÈ¿ÓÓË, ∂. ÷Ù˙ˉ¿ÎË, ª. ∫·ÏÌ·ÓÙ‹ 117 ¶·ÓÂÏÏ‹ÓÈ· ÔÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞ Û ·È‰È¿ ËÏÈΛ·˜ 0-14 ÂÙÒÓ µ. ¶··Â˘·ÁÁ¤ÏÔ˘, ∞. ∫‡Úη, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜, ¢. ∫·Û›ÌÔ˜, ∫. ¶·ÓÙÂÏ¿ÎË, ∂. ¶·‡ÏÔ˘, ª. ∆˙Ô‡ÊË, ª. ª·˘ÚÔÎÒÛÙ·, ∞. ∆˙È‚¿Ú·˜, •. ¶Â‰¤ÏË, ∞. ÷Ù˙ËÌȯ·‹Ï, º. ∞ı·Ó·ÛÈ¿‰Ô˘, ¢. ∫·ÊÂÙ˙‹˜, ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜ 123 §¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿: ÂÌÂÈÚ›· 28 ÂÙÒÓ ∞. ¶Ô˘ÚÙÛ›‰Ë˜, ¢. ¢ÔÁ¿Ó˘, ª. ª¿Î·, ¢. ªÔ˘¯Ô‡ÙÛÔ˘, ª. µ·Ú‚Ô˘ÙÛ‹, ª. ™˘ÓÔ‰ÈÓÔ‡, ∞ÈÎ. ªÈ¯·‹Ï-™ÙÚ¿ÓÙ˙È·, ∂. ∫ÔÛÌ›‰Ë ¶ƒ∞∫∆π∫√ £∂ª∞ 132 √È ÂÈÙÒÛÂȘ Ù˘ ˘ÂÚÙÚÔÊ›·˜ ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ ÛÙËÓ Î·Ú‰È·Î‹ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ ∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘ 139 √Ú›˙ÔÓÙ·˜ ÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ·¯˘Û·ÚΛ· ÛÙËÓ Ú¿ÍË: ÌÂıÔ‰ÔÏÔÁÈο ‰ÈÏ‹ÌÌ·Ù· ¶. ¶ÂÚ‚·Ó›‰Ô˘ ∂¡¢π∞º∂ƒ√À™A ¶∂ƒπ¶∆ø™H 145 ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘ Ã. ∆۷ηϛ‰Ë˜, µ. §·ÌÚfiÔ˘ÏÔ˜, Œ. √˘ÚÁηÓÙ˙fiÁÏÔ˘, ¢. ƒ¿ÏÏ˘, ∞. ¶ÂÙÚfiÔ˘ÏÔ˜, ¡. ¡ÈÎÔÏ·˝‰Ë˜ ∫§π¡π∫√ ∫√Àπ∑ 150 ∞ÁfiÚÈ 13 ÂÙÒÓ Ì ·ӷϷ̂·ÓfiÌÂÓ· ÂÂÈÛfi‰È· ÂÒ‰˘Ó˘ Ô‰ÔÎÓËÌÈ΋˜ ¿ÚıÚˆÛ˘ ¡. ª·ÚΤ·˜, π. ¶··¯Ú‹ÛÙÔ˜ ™À¡∆√ª∞ ¶∞π¢π∞∆ƒπ∫∞ ¡∂∞ 151 ¡¤· ·fi ÙÔÓ ¯ÒÚÔ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÏÔÈÌÒÍÂˆÓ ¡. ™˘Ú›‰Ë˜ ∂§§∏¡π∫∏ ∂ƒ∂À¡∞ ™∆∏ ¢π∂£¡∏ µπµ§π√°ƒ∞ºπ∞ 154 ∂ÏÏËÓÈ΋ ·È‰È·ÙÚÈ΋ ¤Ú¢ӷ Û ‰ÈÂıÓ‹ ÂÚÈÔ‰Èο, 2008 ∂. °·Ï·Ó¿Î˘ ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√ 156 πÛÙÔÛÂÏ›‰· Ù˘ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ ™. ∫¯·ÁÈ¿˜, ¢. ∞Ó·ÁÓÒÛÙÔ˘ ¶·È‰È·ÙÚÈ΋ ∆fiÌÔ˜ 72 ñ ∆‡¯Ô˜ 2 ñ ª¿ÚÙÈÔ˜-∞Ú›ÏÈÔ˜ 2009 ∫ˆ‰ÈÎfi˜ ¢È‡ı˘ÓÛ˘ ∂ÔÙ›·˜ ªª∂: 3889 ISSN 0377-2551

Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

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Page 1: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

¢ÈÌËÓÈ·›· ¤Î‰ÔÛË Ù˘

EÏÏËÓÈ΋˜ ¶·È‰È·ÙÚÈ΋˜ EÙ·ÈÚ›·˜

¶Úfi‰ÚÔ˜

∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜

™˘ÓÙ·ÎÙÈ΋ EÈÙÚÔ‹

¢È¢ı˘ÓÙ‹˜ ™‡ÓÙ·Í˘

∫. ™ÙÂÊ·Ó›‰Ë˜

M¤ÏË

™. ∞Ó‰ÚÔÓ›ÎÔ˘

ª. ∞ÓıÚ·ÎfiÔ˘ÏÔ˜

¶. ∞˘ÁÔ˘ÛÙ›‰Ô˘-™·‚‚ÔÔ‡ÏÔ˘

°. µ·ÚÏ¿Ì˘

∂. °·Ï·Ó¿Î˘

§. £ˆÌ·˝‰Ô˘

ª. ∫·Ó¿ÚÈÔ˘

A. K·ÙÙ¿Ì˘

™. K›ÙÛÈÔ˘-∆˙¤ÏË

∞. ¶··‰ÔÔ‡ÏÔ˘

µ. ¶··Â˘·ÁÁ¤ÏÔ˘

∞. ™È·ÌÔÔ‡ÏÔ˘-ª·˘Ú›‰Ô˘

∞. ™˘Ú›ÁÔ˘-¶··‚·ÛÈÏ›Ԣ

∂. ÷ÚÌ·Ó‰¿ÚË

ÀÔ‚ÔÏ‹ ∂ÚÁ·ÛÈÒÓ

e-mail: [email protected]

√‰ËÁ›Â˜ ÚÔ˜ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜:

http://www.e-child.gr/Instructions_

to_Authors_GR.pdf

ºÈÏÔÏÔÁÈ΋ EÈ̤ÏÂÈ·

∂È̤ÏÂÈ· ÂÏÏËÓÈÎÒÓ ÎÂÈ̤ӈÓ

∫. ¶··¯Ú‹ÛÙÔ˘

EÈ̤ÏÂÈ· ·ÁÁÏÈÎÒÓ ÎÂÈ̤ӈÓ

™. ¡¿ÎÔ˘

EΉfiÙ˘

K. °ÚÈ‚¤·˜

™˘ÓÙÔÓÈÛÙ‹˜ ∂ΉfiÛˆ˜

Communications In Practice ∞.∂.

¶ÈÂÚ›·˜ 1∞

144 51 MÂÙ·ÌfiÚʈÛË

TËÏ.: 210 87 78 884

Fax: 210 87 78 822

I‰ÈÔÎÙ‹Ù˘

EÏÏËÓÈ΋ ¶·È‰È·ÙÚÈ΋ EÙ·ÈÚ›·©

Mȯ·Ï·ÎÔÔ‡ÏÔ˘ 92

Aı‹Ó· 115 28

TËÏ.: 210 7771 140

210 7771 663

Fax: 210 7758 354

e-mail: [email protected]

EÙ‹ÛÈ· ™˘Ó‰ÚÔÌ‹: 40 €EȉÈ΢fiÌÂÓÔÈ, ºÔÈÙËÙ¤˜: 20 €

¶ÂÚȯfiÌÂÓ·

∞¡∞™∫O¶∏™∂π™

75 √Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Û ·È‰È¿

Î·È ÂÊ‹‚Ô˘˜

∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹

82 §ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ - ¡ÂfiÙÂÚ·

ıÂڷ¢ÙÈο ‰Â‰Ô̤ӷ

µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹

89 ∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË

Ã. ∫Ô˘ÙÛ·˘Ù›ÎË, ∂. ª¿ÓÙ˙ÈÔ˘, ¡. ª˘ÚÈÔÎÂÊ·ÏÈÙ¿Î˘

97 ¡ÂfiÙÂÚ· ‰Â‰Ô̤ӷ ÁÈ· ÙË Û˘ÁÁÂÓ‹

‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

Ã. ∆۷ηϛ‰Ë˜, ∂. √˘ÚÁηÓÙ˙fiÁÏÔ˘, ¡. ¡ÈÎÔÏ·˝‰Ë˜

∂ƒ∂À¡∏∆π∫∂™ ∂ƒ°∞™π∂™

109 ∂›‰Ú·ÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

Ì˘ÂÏÔ‡ ÔÛÙÒÓ ÛÙËÓ ex vivo ¤ÎÙ˘ÍË

·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜

π. ¶ÂÏ·ÁÈ¿‰Ë˜, ∂. ¢ËÌËÙÚ›Ô˘, ∂. ™ÙÂȷοÎË, Ã. ¶ÂÚ‰ÈÎÔÁÈ¿ÓÓË, ∂. ÷Ù˙ˉ¿ÎË, ª. ∫·ÏÌ·ÓÙ‹

117 ¶·ÓÂÏÏ‹ÓÈ· ÔÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË

Ë·Ù›Ùȉ·˜ ∞ Û ·È‰È¿ ËÏÈΛ·˜ 0-14 ÂÙÒÓ

µ. ¶··Â˘·ÁÁ¤ÏÔ˘, ∞. ∫‡Úη, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜, ¢. ∫·Û›ÌÔ˜, ∫. ¶·ÓÙÂÏ¿ÎË, ∂. ¶·‡ÏÔ˘, ª. ∆˙Ô‡ÊË,ª. ª·˘ÚÔÎÒÛÙ·, ∞. ∆˙È‚¿Ú·˜, •. ¶Â‰¤ÏË, ∞. ÷Ù˙ËÌȯ·‹Ï, º. ∞ı·Ó·ÛÈ¿‰Ô˘, ¢. ∫·ÊÂÙ˙‹˜, ∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜

123 §¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿:

ÂÌÂÈÚ›· 28 ÂÙÒÓ

∞. ¶Ô˘ÚÙÛ›‰Ë˜, ¢. ¢ÔÁ¿Ó˘, ª. ª¿Î·, ¢. ªÔ˘¯Ô‡ÙÛÔ˘, ª. µ·Ú‚Ô˘ÙÛ‹, ª. ™˘ÓÔ‰ÈÓÔ‡,∞ÈÎ. ªÈ¯·‹Ï-™ÙÚ¿ÓÙ˙È·, ∂. ∫ÔÛÌ›‰Ë

¶ƒ∞∫∆π∫√ £∂ª∞

132 √È ÂÈÙÒÛÂȘ Ù˘ ÂÚÙÚÔÊ›·˜ ÙˆÓ

·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ ÛÙËÓ Î·Ú‰È·Î‹

ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ

∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘

139 √Ú›˙ÔÓÙ·˜ ÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋

·¯˘Û·ÚΛ· ÛÙËÓ Ú¿ÍË:

ÌÂıÔ‰ÔÏÔÁÈο ‰ÈÏ‹ÌÌ·Ù·

¶. ¶ÂÚ‚·Ó›‰Ô˘

∂¡¢π∞º∂ƒ√À™A ¶∂ƒπ¶∆ø™H

145 ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘

Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜

ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘

Ã. ∆۷ηϛ‰Ë˜, µ. §·ÌÚfiÔ˘ÏÔ˜, Œ. √˘ÚÁηÓÙ˙fiÁÏÔ˘, ¢. ƒ¿ÏÏ˘, ∞. ¶ÂÙÚfiÔ˘ÏÔ˜, ¡. ¡ÈÎÔÏ·˝‰Ë˜

∫§π¡π∫√ ∫√Àπ∑

150 ∞ÁfiÚÈ 13 ÂÙÒÓ Ì ·ӷϷ̂·ÓfiÌÂÓ·

ÂÂÈÛfi‰È· ÂÒ‰˘Ó˘ Ô‰ÔÎÓËÌÈ΋˜

¿ÚıÚˆÛ˘

¡. ª·ÚΤ·˜, π. ¶··¯Ú‹ÛÙÔ˜

™À¡∆√ª∞ ¶∞π¢π∞∆ƒπ∫∞ ¡∂∞

151 ¡¤· ·fi ÙÔÓ ÒÚÔ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ

ÏÔÈÌÒ͈Ó

¡. ™˘Ú›‰Ë˜

∂§§∏¡π∫∏ ∂ƒ∂À¡∞ ™∆∏ ¢π∂£¡∏µπµ§π√°ƒ∞ºπ∞

154 ∂ÏÏËÓÈ΋ ·È‰È·ÙÚÈ΋ ¤Ú¢ӷ Û ‰ÈÂıÓ‹

ÂÚÈÔ‰Èο, 2008

∂. °·Ï·Ó¿Î˘

¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√

156 πÛÙÔÛÂÏ›‰· Ù˘ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘

¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ

™. ∫¯·ÁÈ¿˜, ¢. ∞Ó·ÁÓÒÛÙÔ˘

¶·È‰È·ÙÚÈ΋∆fiÌÔ˜ 72 ñ ∆‡¯Ô˜ 2 ñ ª¿ÚÙÈÔ˜-∞Ú›ÏÈÔ˜ 2009

∫ˆ‰ÈÎfi ¢È‡ı˘ÓÛË ∂ÔÙ›· ªª∂: 3889

ISSN 0377-2551

Pediatri Mar-Apr 09 10-04-09 15:35 ™ÂÏ›‰·1

Page 2: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

Bimonthly Publication of

the Greek Paediatric Society

President

A. Constantopoulos

Editorial Board

Editor-in-Chief

C. Stefanidis

Members

S. Andronikou

ª. ∞nthracopoulos

P. Augoustides-Savvopoulou

G. Varlamis

∂. Galanakis

L. Thomaidou

M. Kanariou

A. Kattamis

S. Kitsiou-Tzeli

∞. Papadopoulou

V. Papaevagelou

A. Siamopoulou-Mavridou

A. Syrigou-Papavasiliou

E. Charmandari

Manuscript submission

e-mail: [email protected]

Instructions to authors:

http://www.e-child.gr/paediatriki/

iae.pdf

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Greek Editing

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English Editing

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Publishing Coordinator

Communications In Practice S.∞.

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Contents

REVIEW ARTICLES

75 Acute lymphoblastic leukaemia in children

and adolescents

E. Stiakaki, M. Kalmanti

82 Childhood lymphomas - Novel therapeutic

approaches

V. Danilatou, M. Kalmanti

89 Haemophagocytic lymphohistiocytosis - HLH

C. Koutsaftiki, E. Mantziou, N. Myriokefalitakis

97 Current data on congenital diaphragmatic

hernia

C. Tsakalidis, E. Ourgantzoglou, N. Nikolaidis

ORIGINAL ARTICLES

109 The effect of paediatric bone-marrow-

derived stromal cells on the ex-vivo

expansion of cord-blood haemopoietic

stem cells

I. Pelagiadis, H. Dimitriou, E. Stiakaki, C. Perdikogianni, E. Hatzidaki, M. Kalmanti

117 Seroepidemiological study of hepatitis A in

Greek children aged 0-14 years

V. Papaevagelou, A. Kyrka, A. Tragiannidis, D. Kassimos, K. Pantelaki, E. Pavlou, M. Tzoufi,M. Mavrokosta, A. Tzivaras, X. Pedeli, A. Hatzimichail, F. Athanassiadou, D. Kafetzis, A. Konstantopoulos

123 Hodgkin’s lymphoma in children:

28 years of experience

A. Pourtsidis, D. Doganis, M. Baka, D. Bouhoutsou, M. Varvoutsi, M. Synodinou, A. Michael-Strantzia, E. Kosmidi

PRACTICAL ISSUES

132 Cardiovascular consequences of

adenotonsillar hypertrophy in children

K. Papadopoulou-Legbelou, G. Varlamis

139 Defining obesity in children and

adolescents: methodological dilemmas

P. Pervanidou

CASE REPORT

145 Bochdalek congenital diaphragmatic

hernia with intrathoracic ectopic kidney -

a case report

C. Takalidis, V. Lampropoulos, E. Ourgantzoglou,D. Rallis, A. Petropoulos, N. Nikolaidis

CLINICAL QUIZ

150 Thirteen-year-old boy with recurrent

episodes of painful ankle joint

¡. Markeas, π. Papachristos

PAEDIATRIC NEWS IN BRIEF

151 News on paediatric infections

N. Spyridis

HELLENIC RESEARCH IN THEINTERNATIONAL LITERATURE

154 Hellenic paediatric research in

international journals, 2008

E. Galanakis

NEWS FROM THE INTERNET

156 Site of the 2nd Department of Paediatrics

of the University of Athens

S. Kehagias, D. Anagnostou

PaediatrikiVolume 72 ñ Number 2 ñ March-April 2009

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·3

Page 3: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

75∞¡∞™∫√¶∏™∏ REVIEW ARTICLE

¶·È‰È·ÙÚÈ΋ 2009;72:75-000

√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜

∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹

¶ÂÚ›ÏË„Ë: ∏ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· Â›Ó·È Ë Û˘¯ÓfiÙÂÚË Î·ÎÔ‹ıÂÈ· ÛÙ· ·È‰È¿. ∏ ÚfiÔ‰Ô˜ ÛÙˉȿÁÓˆÛË Î·È Ë ıÂڷ›· Û‡Ìʈӷ Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ ÙÔ˘ οı ·È‰ÈÔ‡ ¤¯Ô˘Ó ‰ÒÛÂÈ ÔÛÔÛÙ¿ ›·-Û˘ Ô˘ ÏËÛÈ¿˙Ô˘Ó ÙÔ 90%. ∏ ΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ÌÂϤÙË ÙˆÓ ‚Ï·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·›˙ÂÈ ÛËÌ·ÓÙÈÎfi Úfi-ÏÔ ÛÙËÓ ÚÔÛ·ÚÌÔÁ‹ Ù˘ ıÂڷ›·˜ Î·È ÛÙËÓ ·Ó·˙‹ÙËÛË Ó¤ˆÓ ıÂڷ¢ÙÈÎÒÓ ÛÙfi¯ˆÓ. ∏ ηٿٷÍË ÛÂÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ ‚·Û›˙ÂÙ·È Û ÎÏÈÓÈο Î·È Î˘ÙÙ·ÚÔÁÂÓÂÙÈο ‰Â‰Ô̤ӷ, ηıÒ˜ Î·È ÛÙËÓ ÂÎÙ›ÌËÛË Ù˘ ÂÏ¿-¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘, Ô˘ Ê·›ÓÂÙ·È Ó· ·ÔÙÂÏ› ¤Ó·Ó ·fi ÙÔ˘˜ ÈÛ¯˘ÚfiÙÂÚÔ˘˜ ÚÔÁÓˆÛÙÈÎÔ‡˜·Ú¿ÁÔÓÙ˜ Î·È ·Ú¤¯ÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ÁÈ· ۯ‰fiÓ ÂÍ·ÙÔÌÈÎÂ˘Ì¤ÓË ıÂڷ›·. ∂ÈÚfiÛıÂÙ·, ÚfiÛÊ·Ù˜ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó fiÙÈ ÔÈ ¤ÊË‚ÔÈ Î·È ÔÈ Ó·ÚÔ› ÂÓ‹ÏÈΘ ¤¯Ô˘Ó Ôχ ÌÂÁ·Ï‡ÙÂÚ· ÔÛÔÛÙ¿ ›·Û˘ fiÙ·Ó ıÂ-ڷ‡ÔÓÙ·È Ì ·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ·, Û˘ÁÎÚÈÙÈο Ì ÂΛÓÔ˘˜ Ô˘ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ıÂڷ¢ÙÈοۯ‹Ì·Ù· ÙˆÓ ÂÓËϛΈÓ.

§¤ÍÂȘ ÎÏÂȉȿ: √Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·, ·È‰È¿, ¤ÊË‚ÔÈ.

Acute lymphoblastic leukaemia in children and adolescents

E. Stiakaki, M. Kalmanti

Abstract: Acute lymphoblastic leukaemia is the most common malignancy in childhood. Progress indiagnosis and treatment according to risk classification have produced cure rates that now approach 90%.Molecular genetics of the blasts plays a crucial role in tailoring therapy and identifying new targets forspecific treatment. Current risk classification schemes are based on clinical characteristics and cytogeneticfeatures, and on minimal residual disease assessment, which appears to be among the strongestprognostic factors and provides an opportunity for more precise risk tailored treatments. Recent studieshave shown that adolescents and young adults have a much better outcome when treated with paediatricthan with adult protocols.

Key words: Acute lymphoblastic leukaemia, children, adolescents

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘

AÏÏËÏÔÁÚ·Ê›·:

∂˘Ù˘¯›· ™ÙÂȷοÎË[email protected]¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜,¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô∏Ú·ÎÏ›Ԣ, 71110 ∏Ú¿ÎÏÂÈÔ∫Ú‹Ù˘

Department of PaediatricHaematology/Oncology,University of Crete, Greece

Correspondence:

Eftichia [email protected] of PaediatricHaematology/Oncology,University Hospital ofHeraklion, 71110 Heraklion,Crete, Greece

™˘ÓÙÔÌÔÁڷʛ˜:

ALL acute lymphoblastic leukemia

BFM Berlin-Frankfurt-Muenster

CCG Children’s Cancer Group

COG Children’s Oncology Group

DFCI Dana-Farber Cancer Institute

MRD Minimal Residual Disease (ÂÏ¿¯ÈÛÙË

˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ˜)

NCI National Cancer Institute

POG Pediatric Oncology Group

√§§ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·

∫¡™ ∫ÂÓÙÚÈÎfi ¡Â˘ÚÈÎfi ™‡ÛÙËÌ·

∂ÈÛ·ÁˆÁ‹

∏ ÔÍ›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›· (√§§)

Â›Ó·È Ë Û˘¯ÓfiÙÂÚË Î·ÎÔ‹ıÂÈ· ÛÙ· ·È‰È¿ ηÈ

·ÔÙÂÏ› ÙÔ 75-80% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ Ó¤ˆÓ Â-

ÚÈÙÒÛÂˆÓ Ï¢¯·ÈÌ›·˜. ∏ Û˘¯ÓfiÙËÙ¿ Ù˘ ›ӷÈ

35 Ì 40 Ӥ˜ ÂÚÈÙÒÛÂȘ ÙÔ ¯ÚfiÓÔ ·Ó¿

1000.000 ·È‰È¿ ËÏÈΛ·˜ <15 ÂÙÒÓ, Ì ̤ÁÈÛÙË

›وÛË ÛÙËÓ ËÏÈΛ· ÙˆÓ 4 ÂÙÒÓ (1).

∏ ÚfiÔ‰Ô˜ ÛÙË ıÂڷ›· Ù˘ √§§ ÛÙ· ·È-

‰È¿ ·ÔÙÂÏ› ÌÂÁ¿ÏÔ Â›Ù¢ÁÌ· Ù˘ Û‡Á¯ÚÔÓ˘

ÎÏÈÓÈ΋˜ ÔÁÎÔÏÔÁ›·˜, ηı’ fiÛÔÓ ÙÔ 1970 ÙÔ Ô-

ÛÔÛÙfi ›·Û˘ ÌfiÏȘ ¿ÁÁÈ˙ ÙÔ 30%, ÂÓÒ Û‹ÌÂÚ·

¤¯ÂÈ ˘Âڂ› ÙÔ 80% (2). ∏ ÚfiÔ‰Ô˜ Ô˘ ¤¯ÂÈ

ÂÈÙ¢¯ı› ÔÊ›ÏÂÙ·È ÛÙË ¯Ú‹ÛË ·ÔÙÂÏÂÛÌ·-

ÙÈÎÒÓ ¯ËÌÂÈÔıÂڷ¢ÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÛÙ·

Ï·›ÛÈ· Ù˘¯·ÈÔÔÈËÌ¤ÓˆÓ ÌÂÏÂÙÒÓ, ÛÙË ıÂÚ·-

›· Ù˘ ˘ÔÎÏÈÓÈ΋˜ ÓfiÛÔ˘ ÙÔ˘ ∫¡™, ÛÙËÓ

ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂڷ›·˜ ηÈ, ‚¤‚·È·,

ÛÙËÓ Ù·ÍÈÓfiÌËÛË Î·È ıÂڷ›· Ì ‚¿ÛË ÙÔ˘˜

·Ú¿ÁÔÓÙ˜ ÎÈÓ‰‡ÓÔ˘ (3).

∂Ú¢ÓËÙÈο, ÙÔ ÂӉȷʤÚÔÓ ¤¯ÂÈ ÂÛÙÈ·ÛÙ›

ÛÙË ÌÂϤÙË ÙÔ˘ ÚfiÏÔ˘ ÙˆÓ ÌÔÚÈ·ÎÒÓ ‰È·Ù·Ú·-

¯ÒÓ ÛÙË Ï¢¯·ÈÌÔÁ¤ÓÂÛË, Û ʷÚÌ·ÎÔÁÂÓÂÙÈ-

Τ˜ ÌÂϤÙ˜ Î·È ÛÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÚfiÏÔ˘ Ù˘

˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘.

¶·Ú¿ ÙËÓ ÚfiÔ‰Ô Ô˘ ¤¯ÂÈ ÂÈÙ¢¯ı›, ¤Ó·

ÔÛÔÛÙfi ·È‰ÈÒÓ Ï·Ì‚¿ÓÂÈ ›Ûˆ˜ ÂÚÈÛÛfiÙÂÚË

ıÂڷ›· ·fi ÂΛÓË Ô˘ Ú·ÁÌ·ÙÈο ¯ÚÂÈ¿˙Â-

Ù·È ÁÈ· Ó· È·ı›, 10-20% ÙˆÓ ·È‰ÈÒÓ Ì ·Ú¯Èο

¢ÓÔ˚ÎÔ‡˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÛÙË

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·75

Page 4: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

76 ∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹

Paediatriki 2009;72:76-000

Û˘Ó¤¯ÂÈ· ˘ÔÙÚÔÈ¿˙Ô˘Ó, ÂÓÒ ˘¿Ú¯ÂÈ Â›Û˘ ÌÈ·

ÔÌ¿‰· ·ÛıÂÓÒÓ Ì ÓfiÛÔ ·ÓıÂÎÙÈ΋ ÛÙË ıÂڷ›·.

∂Âȉ‹ Ë ÂÚ·ÈÙ¤Úˆ ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂڷ›·˜

‰ÂÓ Ê·›ÓÂÙ·È Ó· ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÚfiÁÓˆÛË, ÙÔ ÂӉȷ-

ʤÚÔÓ ¤¯ÂÈ ÛÙÚ·Ê› Û ӤԢ˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ·-

ÚÂÌ‚·›ÓÔ˘Ó ÛÙË Ï¢¯·ÈÌÔÁ¤ÓÂÛË Î·È ÛÙËÓ ·ÓÙÔ¯‹

ÛÙ· Ê¿Ú̷η, ηıÒ˜ Î·È ÛÙËÓ ·Ó·ÁÓÒÚÈÛË ÂÈÚfi-

ÛıÂÙˆÓ ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ¤ÙÛÈ ÒÛÙÂ Ë Ù·-

ÍÈÓfiÌËÛË Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ Ó· Â›Ó·È ÈÔ ·ÎÚÈ‚‹˜.

∆·ÍÈÓfiÌËÛË Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘

°È· ÔÏϤ˜ ‰ÂηÂٛ˜, Ë ıÂڷ›· ‚·Û›ÛÙËΠÛÂ

ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ËÏÈΛ·, ÙÔ Ê‡-

ÏÔ, Ô ·ÚÈıÌfi˜ ÙˆÓ Ï¢ÎÒÓ ÛÙË ‰È¿ÁÓˆÛË, Ë ·ÚÔ˘-

Û›· ÓfiÛÔ˘ ÛÙÔ ∫¡™, Ô ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô˜ Î·È ÔÈ Î˘Ù-

Ù·ÚÔÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ÙˆÓ ‚Ï·ÛÙÒÓ, ηÈ, ÌÂÙ·ÁÂ-

Ó¤ÛÙÂÚ·, Ë ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· (4,5).

™Ù· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ıÂڷ›·˜ ¤¯ÂÈ ÚÔ-

ÛÙÂı› Ë ÂÎÙ›ÌËÛË Ù˘ ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘. ∏ ËÏÈ-

Λ· Î·È Ô ·ÚÈıÌfi˜ ÙˆÓ Ï¢ÎÒÓ ÛÙË ‰È¿ÁÓˆÛË ·Ú·-

̤ÓÔ˘Ó ‰È·¯ÚÔÓÈο ÛÙ·ıÂÚÔ› Î·È ·ÓÂÍ¿ÚÙËÙÔÈ ÚÔ-

ÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Û fiÏ· Ù· ÚˆÙfiÎÔÏÏ· ıÂÚ·-

›·˜. ™‡Ìʈӷ Ì ٷ ÎÚÈÙ‹ÚÈ· ÙÔ˘ NCI, Ù· ·È‰È¿

Ì √§§ µ-ÛÂÈÚ¿˜, ËÏÈΛ·˜ 1-9 ÂÙÒÓ Î·È Ì ÏÂ˘Î¿

<50.000 Îί ıˆÚÔ‡ÓÙ·È ÛÙ·ıÂÚÔ‡ ÎÈÓ‰‡ÓÔ˘, ÂÓÒ Ù·

˘fiÏÔÈ· ·È‰È¿ ηٷٿÛÛÔÓÙ·È ÛÙËÓ ÔÌ¿‰· ˘„Ë-

ÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (4). ∞ÓÙ›ÛÙÔȯ·, Î·È ÛÙ· ÚˆÙfiÎÔÏÏ·

BFM ̤¯ÚÈ Î·È ÙÔ 1995, Ë ËÏÈΛ· 1-6 ÂÙÒÓ Î·È Ô ·ÚÈı-

Ìfi˜ Ï¢ÎÒÓ <20.000 Îί ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÁÈ· ÙËÓ

ηٿٷÍË ÙˆÓ ·ÛıÂÓÒÓ ÛÙËÓ ÔÌ¿‰· ÛÙ·ıÂÚÔ‡ ÎÈÓ-

‰‡ÓÔ˘ (5).

∏ Û˘ÌÌÂÙÔ¯‹ ÙÔ˘ ∫¡™ ÛÙË ‰È¿ÁÓˆÛË (¶›Ó·Î·˜

1) ¤¯ÂÈ Â›Û˘ ·Ô‰Âȯı› ÛËÌ·ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜

ÛÙËÓ ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ (∂ÈÎfiÓ· 1). ∞Ú¯Èο, Ë

·Ó·ÁÓÒÚÈÛË ÙÔ˘ ÚfiÏÔ˘ Ù˘ ÚÔʇϷ͢ ÙÔ˘ ∫¡™ ‹

-ÈÔ ÛˆÛÙ¿- Ë ıÂڷ›· Ù˘ ˘ÔÎÏÈÓÈ΋˜ ÓfiÛÔ˘,

·fi Ù· Ù¤ÏË Ù˘ ‰ÂηÂÙ›·˜ ÙÔ˘ 1960, Û˘Ó¤‚·Ï ٷ

̤ÁÈÛÙ· ÛÙË ‚ÂÏÙ›ˆÛË Ù˘ ÂÈ‚›ˆÛ˘/›·Û˘. ™‹ÌÂ-

Ú·, Ë ıÂڷ›· Ù˘ ˘ÔÎÏÈÓÈ΋˜ ÓfiÛÔ˘ ‚·Û›˙ÂÙ·È

ÛÙËÓ ÂÈıÂÙÈ΋ Û˘ÛÙËÌ·ÙÈ΋ ¯ËÌÂÈÔıÂڷ›·

(HDMTX ) Î·È ÛÙËÓ ÂÓ‰ÔÚÚ·¯È·›· ¯ÔÚ‹ÁËÛË ÌÂıÔ-

ÙÚÂÍ¿Ù˘ ÛÙÔ 80-90% ÙˆÓ ·È‰ÈÒÓ. ™ÙÔ˘˜ ·ÛıÂÓ›˜

˘„ËÏÔ‡ ‹ Ôχ „ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÔÚËÁÂ›Ù·È ÙÚÈÏ‹

ÂÓ‰ÔÚÚ·¯È·›· ¯ËÌÂÈÔıÂڷ›· Î·È ·ÎÙÈÓÔ‚ÔÏ›· ÎÚ·-

Ó›Ô˘ (12Gy) Û fiÏ· ۯ‰fiÓ Ù· ΤÓÙÚ·, Ù·ÎÙÈ΋ Ô˘

¤¯ÂÈ ÌÂÈÒÛÂÈ ÙÔ ÔÛÔÛÙfi ÙˆÓ ˘ÔÙÚÔÒÓ ÌÂ Û˘ÌÌÂ-

ÙÔ¯‹ ÙÔ˘ ∫¡™ Û <5% Î·È ÙˆÓ ÌÂÌÔÓˆÌ¤ÓˆÓ ˘Ô-

ÙÚÔÒÓ ∫¡™ ÛÙÔ 1,1%. ∂Í·›ÚÂÛË ·ÔÙÂÏ› ÙÔ St.

Jude, ÛÙ· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ÙÔ˘ ÔÔ›Ô˘ ‰ÂÓ ¯Ô-

ÚËÁÂ›Ù·È ·ÎÙÈÓÔ‚ÔÏ›· Ô‡Ù ÛÙÔ˘˜ ·ÛıÂÓ›˜ ˘„ËÏÔ‡

ÎÈÓ‰‡ÓÔ˘ (6,7). ∆ËÓ ÙÂÏÂ˘Ù·›· ‰ÂηÂÙ›· ·Ó·ÁÓˆÚ›-

ÛÙËΠfiÙÈ Ë ÙÚ·˘Ì·ÙÈ΋ ÔÛÊ˘ÔÓˆÙÈ·›· ·Ú·Î¤ÓÙË-

ÛË ÛÙË ‰È¿ÁÓˆÛË ÂÈ‚·Ú‡ÓÂÈ ÙËÓ ÙÂÏÈ΋ Â΂·ÛË ÙˆÓ

·ÛıÂÓÒÓ, Ì ÔÛÔÛÙfi ˘ÔÙÚÔÒÓ ÙÔ˘ ∫¡™ ÛÙ· Â-

ÚÈÛÙ·ÙÈο ·˘Ù¿ ÂÚ›Ô˘ 8% (8). ŒÙÛÈ, ‹‰Ë ÛÙ· Úˆ-

ÙfiÎÔÏÏ· BFM -95 Î·È St. Jude XIII ·˘Í‹ıËÎÂ Ô ·ÚÈı-

Ìfi˜ ÙˆÓ ÂÓ‰ÔÚÚ·¯È·›ˆÓ ¯ÔÚËÁ‹ÛÂˆÓ ÛÙȘ ÂÚÈÙÒ-

ÛÂȘ ·˘Ù¤˜.

∞ÓÔÛÔÊ·ÈÓfiÙ˘Ô˜

∏ ÌÂϤÙË ÙÔ˘ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô˘ ÙˆÓ ‚Ï·ÛÙÒÓ

ÛÙË ‰È¿ÁÓˆÛË Ù˘ √§§ ¤¯ÂÈ ·ÚÔ˘ÛÈ¿ÛÂÈ ÙÂÚ¿ÛÙÈ·

ÚfiÔ‰Ô Ù· ÙÂÏÂ˘Ù·›· ÚfiÓÈ·, Ì ÙË ‰È¿ıÂÛË ÌÂÁ¿ÏÔ˘

·ÚÈıÌÔ‡ ÌÔÓÔÎψÓÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ï¢ÎÔ-

΢ÙÙ·ÚÈÎÒÓ ·ÓÙÈÁfiÓˆÓ Î·È ÙÔ Û¯Â‰È·ÛÌfi ¢·›ÛıËÙ˘

Î·È ·ÍÈfiÈÛÙ˘ ΢ÙÙ·ÚÔÌÂÙÚ›·˜ ÚÔ‹˜. ¢Â›ÎÙ˜ ȉȷ›-

ÙÂÚ· ¢·›ÛıËÙÔÈ ÁÈ· ÙË µ Î·È ∆ ÛÂÈÚ¿ ·ÓÙ›ÛÙÔȯ· ›-

Ó·È Ù· CD19 Î·È CD7 Î·È ‰Â›ÎÙ˜ Ì ÌÂÁ¿ÏË ÂȉÈÎfi-

ÙËÙ· Ù· ΢ÙÙ·ÚÔÏ·ÛÌ·ÙÈο CD79a, CD3 ÁÈ· µ ηÈ

∆ ÏÂÌÊÔ·ÙÙ·Ú·, ·ÓÙ›ÛÙÔȯ·. ªÂ ÙÔÓ ·ÓÔÛÔÊ·ÈÓfi-

Ù˘Ô ÌfiÓÔ, Ë ‰È¿ÁÓˆÛË ÌÔÚ› Ó· ÙÂı› ÛÙÔ 99% ÙˆÓ

ÂÚÈÙÒÛÂˆÓ (9).

ªÂ ‚¿ÛË ÙÔÓ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô ÙˆÓ ‚Ï·ÛÙÒÓ, Ë

·ÚÈ· ‰È¿ÎÚÈÛË Â›Ó·È ÌÂٷ͇ √§§ B-΢ÙÙ·ÚÈ΋˜

ÚÔ¤Ï¢Û˘ (µ-lineage ‹ µ-precursor ALL) (85%

ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ÛÙ· ·È‰È¿) Î·È ∆-

√§§, Ô˘ ·ÔÙÂÏ› ÙÔ ˘fiÏÔÈÔ 15%. ∏ √§§ B-

΢ÙÙ·ÚÈ΋˜ ÚÔ¤Ï¢Û˘ ÌÔÚ› Ó· ‰È·ÎÚÈı› ÂÚ·È-

Ù¤Úˆ Û ÚÒÈÌË ÚÔ-µ, ÚÔ-µ, ÌÂÙ·‚·ÙÈ΋ ÚÔ-µ

∂ÈÎfiÓ· 1. §ÂÌÊÔ‚Ï¿ÛÙ˜ Û ΢ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ∂¡À.

¶›Ó·Î·˜ 1. ∆·ÍÈÓfiÌËÛË Ù˘ Û˘ÌÌÂÙÔ¯‹˜ ÙÔ˘ ∫¡™ ÛÙËÓ √§§

¡fiÛÔ˜ ÛÙÔ ∫¡™ñ CNS 1

∞Ô˘Û›· ‚Ï·ÛÙÒÓ ÛÙËÓ Î˘ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ÙÔ˘ ∂¡Àñ CNS 2

<5 WBC/Ìl ÛÙËÓ Î˘ÙÙ·ÚÔÊ˘ÁÔΤÓÙÚËÛË ÌÔÚÊÔÏÔÁÈο‚Ï¿ÛÙ˜

ñ CNS 3≥5 WBC/Ìl ÌÔÚÊÔÏÔÁÈο ‚Ï¿ÛÙ˜

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·76

Page 5: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

77√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·

¶·È‰È·ÙÚÈ΋ 2009;72:10-15

Î·È µ-√§§ (10). ŸÛÔÓ ·ÊÔÚ¿ ÙÔÓ Û¯Â‰È·ÛÌfi Ù˘ ıÂ-

ڷ›·˜ Ù˘ √§§ µ-ÛÂÈÚ¿˜, Ë ÌfiÓË ÛËÌ·ÓÙÈ΋ ‰È¿-

ÎÚÈÛË Â›Ó·È ÌÂٷ͇ ÒÚÈÌˆÓ µ Î·È Úfi‰ÚÔÌˆÓ µ-΢Ù-

Ù¿ÚˆÓ. ∞Ó¿ÏÔÁ·, Ë ∆-√§§ ÌÔÚ› Ó· Ù·ÍÈÓÔÌËı› ÌÂ

‚¿ÛË ÙÔ ÛÙ¿‰ÈÔ ‰È·ÊÔÚÔÔ›ËÛ˘ ÙˆÓ ı˘ÌÔ΢ÙÙ¿ÚˆÓ

‹ ÙÔ ÛÙ¿‰ÈÔ ¤ÎÊÚ·Û˘ ÙÔ˘ ˘Ô‰Ô¯¤· ÙÔ˘ ∆-΢ÙÙ¿-

ÚÔ˘, ·ÏÏ¿ ·˘Ù‹ Ë Ù·ÍÈÓfiÌËÛË ¤¯ÂÈ ÂÚÈÔÚÈṲ̂ÓË

ÎÏÈÓÈ΋ ¯ÚËÛÈÌfiÙËÙ·.

∫˘ÙÙ·ÚÔÁÂÓÂÙÈο ·Ú·ÎÙËÚÈÛÙÈο

™ÙÔ 90% Ù˘ √§§ ·Ú·ÙËÚÔ‡ÓÙ·È ÂȉÈΤ˜ ΢ÙÙ·-

ÚÔÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜, ·ÚÈıÌËÙÈΤ˜ ‹ ‰ÔÌÈΤ˜. ∏

΢ÙÙ·ÚÔÁÂÓÂÙÈ΋ ·Ó¿Ï˘ÛË ÙˆÓ ‚Ï·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

¤¯ÂÈ ÌÂÁ¿ÏË ÛËÌ·Û›· ÛÙË ‰È¿ÁÓˆÛË, ıÂڷ›· ηÈ

ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ (11). ∫ÏÈÓÈο ÛËÌ·ÓÙÈΤ˜ ΢Ù-

Ù·ÚÔÁÂÓÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜ Â›Ó·È Ô ˘ÂÚ‰ÈÏÔÂȉÈ-

ÛÌfi˜, Ô ˘Ô‰ÈÏÔÂȉÈÛÌfi˜, ÔÈ ¯ÚˆÌÔÛˆÌÈΤ˜ ÌÂÙ·-

ı¤ÛÂȘ fiˆ˜ t(12;21), t(1;19), t(9;22), Ì ·ÓÙ›ÛÙÔȯ·

ÚÔ˚fiÓÙ· Û‡ÓÙË͢ ÙˆÓ ÁÔÓȉ›ˆÓ TEL-AML1, E2A-

PBX1, BCR-ABL Î·È ÌÈ· ÔÈÎÈÏ›· ·Ó·‰È·Ù¿ÍÂˆÓ ÙÔ˘

MLL ÁÔÓȉ›Ô˘. √ ˘ÂÚ‰ÈÏÔÂȉÈÛÌfi˜ (>50 ¯ÚˆÌÔ-

ÛÒÌ·Ù· ‹ DNA index ≥1,16 ), ηıÒ˜ Î·È ÔÈ ÙÚÈÛˆ-

̛˜ 4,10,17 ·ÏÏ¿ ΢ڛˆ˜ Ë ÙÚÈÏ‹ ÙÚÈۈ̛· 4,10,17,

¤¯Ô˘Ó ıÂÙÈ΋ ›وÛË ÛÙËÓ ¤Î‚·ÛË, ÂÓÒ Ô ˘Ô‰È-

ÏÔÂȉÈÛÌfi˜ (<45 ¯ÚˆÌÔÛÒÌ·Ù· Ì DI <0,95 ‹ <44

¯ÚˆÌÔÛÒÌ·Ù· Î·È DI <0,81) Û¯ÂÙ›˙ÂÙ·È Ì η΋

ÚfiÁÓˆÛË (12-14) (∂ÈÎfiÓ· 2).

∏ ÌÂÙ¿ıÂÛË t(12;21) ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 25% Ù˘

√§§ ΢ڛˆ˜ µ ÛÂÈÚ¿˜ Î·È Ô ÚÔÁÓˆÛÙÈÎfi˜ Ù˘ ÚfiÏÔ˜

‹Ù·Ó ÁÈ· ÔÏÏ¿ ¯ÚfiÓÈ· ·ÌÊÈÏÂÁfiÌÂÓÔ˜. ∞Ú¯Èο ıˆ-

Ú‹ıËΠ¢ÓÔ˚Îfi˜, Ì ÂÈ‚›ˆÛË >90% ÛÙËÓ ÔÌ¿‰·

·˘Ù‹, ÂÓÒ ·ÎÔÏÔ‡ıËÛ·Ó ·Ó·ÊÔÚ¤˜ ÁÈ· ·˘ÍË̤ÓË Û˘-

¯ÓfiÙËÙ· fi„ÈÌˆÓ ˘ÔÙÚÔÒÓ, ¤ÙÛÈ ÒÛÙÂ Ë ÂÈ‚›ˆÛË

Ó· ÌË ‰È·Ê¤ÚÂÈ ÌÂÙ¿ ·fi Ì·ÎÚ¿ ÂÚ›Ô‰Ô ·Ú·ÎÔ-

ÏÔ‡ıËÛ˘ (15,16). ¶ÚfiÛÊ·Ù·, ÔÈ ‰È·ÊÔÚ¤˜ ÛÙËÓ

¤Î‚·ÛË ·Ô‰›‰ÔÓÙ·È ÛÙÔ Â›‰Ô˜ Î·È ÙËÓ ÂÈıÂÙÈÎfiÙË-

Ù· ÙˆÓ ıÂڷ¢ÙÈÎÒÓ ÚˆÙÔÎfiÏψÓ. ∫·Ï‡ÙÂÚË

¤Î‚·ÛË Û¯ÂÙ›˙ÂÙ·È Ì ˘„ËϤ˜ ‰fiÛÂȘ ÌÂıÔÙÚÂÍ¿Ù˘

Î·È L-·Û·Ú·ÁÈÓ¿Û˘. ¶ÚÔÔÙÈ΋ ÌÂϤÙË ¤‰ÂÈÍ fiÙÈ

Ì ÂÈıÂÙÈ΋ ¯ËÌÂÈÔıÂڷ›· ÛÙ· ·È‰È¿ ·˘Ù¿, Ë

5ÂÙ‹˜ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÊÙ¿ÓÂÈ ÙÔ 97%, ¯ˆÚ›˜

fï˜ Ë ÌÂÙ¿ıÂÛË TEL-AML1 Ó· ·Ô‰ÂÈÎÓ‡ÂÙ·È ·ÓÂ-

Í¿ÚÙËÙÔ˜ ÚÔÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ fiÙ·Ó Ï·Ì‚¿ÓÔ-

ÓÙ·È ˘fi„Ë Ë ËÏÈΛ· Î·È Ô ·ÚÈıÌfi˜ Ï¢ÎÒÓ (17).

∏ ‰Â‡ÙÂÚË ÛÂ Û˘¯ÓfiÙËÙ· ÌÂÙ¿ıÂÛË ÛÙËÓ √§§

ÛÙ· ·È‰È¿, Ë t(1;19), Û¯ÂÙ›˙ÂÙ·È Ì ÔÛÔÛÙ¿ ›·Û˘

̤¯ÚÈ 90%, ·ÚfiÏÔ Ô˘ ÛÙÔ ·ÚÂÏıfiÓ Â›¯Â ıˆÚË-

ı› ‰˘ÛÌÂÓ‹˜ ÚÔÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ (18).

∏ t(9;22) ·Ú·ÙËÚÂ›Ù·È ÛÙÔ 3% ÂÚ›Ô˘ ÙˆÓ ·È-

‰ÈÒÓ Ì √§§, ·ÔÙÂÏ› ‰˘ÛÌÂÓ‹ ÚÔÁÓˆÛÙÈÎfi ·-

Ú¿ÁÔÓÙ· Î·È Û¯ÂÙ›˙ÂÙ·È Ì ¯·ÌËÏ¿ ÔÛÔÛÙ¿ ›Ù¢-

͢ ‡ÊÂÛ˘, Û˘¯Ó¤˜ Î·È ÚÒÈ̘ ˘ÔÙÚÔ¤˜ ηÈ

ÊÙˆ¯‹ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË (19). ™Ù· ·È‰È¿ ·˘Ù¿, Ë

ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ, ·ÎfiÌ· Î·È ·fi

ÌË Û˘ÁÁÂÓ‹ ‰fiÙË, ıˆÚÂ›Ù·È ıÂڷ›· ÂÎÏÔÁ‹˜ ÁÈ·

›·ÛË, ÂÓÒ Ë ¯ÔÚ‹ÁËÛË ÈÌ·ÙÈÓ›Ì˘ Û˘ÓÈÛÙ¿Ù·È Û˘Á-

¯ÚfiÓˆ˜ Ì ÙË ¯ËÌÂÈÔıÂڷ›· (20).

√È ·Ó·‰È·Ù¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ MLL ·Ú·ÙËÚÔ‡-

ÓÙ·È ÛÙÔ 8% ÙˆÓ ·È‰ÈÒÓ Ì √§§, ÂÓÒ ·ÔÙÂÏÔ‡Ó

ÙËÓ ÈÔ Û˘¯Ó‹ ÁÂÓÂÙÈ΋ ‰È·Ù·Ú·¯‹ ÛÙ· ‚Ú¤ÊË. ∏ ÌÂ-

Ù¿ıÂÛË t(4;11) [MLL-AF4] Â›Ó·È Ë ÈÔ Û˘¯Ó‹ ÛÙËÓ

ÔÌ¿‰· ·˘Ù‹ Î·È Û¯ÂÙ›˙ÂÙ·È Ì ‰˘ÛÌÂÓ‹ ÚfiÁÓˆÛË

(21). ∞Ó Î·È ÛÙÔ ·ÚÂÏıfiÓ Ë √§§ ÛÙ· ‚Ú¤ÊË ÁÂÓÈο

Û˘Û¯ÂÙ›ÛÙËΠ̠η΋ ÚfiÁÓˆÛË, Û‹ÌÂÚ· ÁÓˆÚ›-

˙Ô˘Ì fiÙÈ, ÌÂÙ¿ ÙË ¯Ú‹ÛË ÂȉÈÎÒÓ ÈÔ ÂÈıÂÙÈÎÒÓ

ÚˆÙÔÎfiÏÏˆÓ ÁÈ· Ù· ‚Ú¤ÊË, η΋ ÚfiÁÓˆÛË ¤¯Ô˘Ó

ÌfiÓÔ ÂΛӷ Ì MLL ·Ó·‰È·Ù¿ÍÂȘ (22,23).

∆Ô 12%-14% Ù˘ √§§ ÛÙ· ·È‰È¿ Â›Ó·È ∆- ÛÂÈ-

Ú¿˜. ∏ ∆-√§§ Â›Ó·È ÈÔ Û˘¯Ó‹ ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ ηÈ

Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ Ì ˘„ËÏfi ·ÚÈıÌfi Ï¢ÎÒÓ Î·È Â›-

Û˘ Û˘Óԉ‡ÂÙ·È Û˘¯ÓfiÙÂÚ· ·fi ‰È‡ڢÓÛË ÌÂÛÔ-

ıˆÚ·Î›Ô˘, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·

Î·È ‰È‹ıËÛË ∫¡™. ªÂ ÙË ¯ÔÚ‹ÁËÛË ÂÈıÂÙÈ΋˜ ¯Ë-

ÌÂÈÔıÂڷ›·˜, Ë ‰˘ÛÌÂÓ¤ÛÙÂÚË ÚfiÁÓˆÛË Û ۯ¤-

ÛË Ì ÙËÓ √§§ µ-ÛÂÈÚ¿˜ ¤¯ÂÈ ‚ÂÏÙȈı›, Ì ÔÛÔÛÙ¿

›·Û˘ 75% (24,25). ™ÙËÓ ÔÌ¿‰· ·˘Ù‹, Ë ËÏÈΛ· Î·È Ô

·ÚÈıÌfi˜ Ï¢ÎÒÓ ‰ÂÓ Ê·›ÓÂÙ·È Ó· ¤¯Ô˘Ó ÈÛ¯˘Ú‹ ÚÔ-

ÁÓˆÛÙÈ΋ ·Í›·, fiˆ˜ ›Û˘ ‰ÂÓ Â›Ó·È Û·Ê‹˜ Ô ÚÔ-

ÁÓˆÛÙÈÎfi˜ ÚfiÏÔ˜ ÙˆÓ Î˘ÙÙ·ÚÔÁÂÓÂÙÈÎÒÓ ‰È·Ù·Ú·-

¯ÒÓ (24). ªÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ NOTCH ¤¯Ô˘Ó

·Ú·ÙËÚËı› ÛÙÔ 50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ∆-√§§,

·ÏÏ¿ Ë ÚÔÁÓˆÛÙÈ΋ ÙÔ˘˜ ÛËÌ·Û›· Â›Ó·È ·Û·Ê‹˜

(27-28).

¶ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· Î·È ÂÏ¿¯ÈÛÙË

˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ˜

∏ ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· ·ÔÙÂÏ›

ÈÛ¯˘Úfi ÚÔÁÓˆÛÙÈÎfi ·Ú¿ÁÔÓÙ·, ÁÈ·Ù› Û¯ÂÙ›˙ÂÙ·È ÌÂ

ÙËÓ Â˘·ÈÛıËÛ›· ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙ· Ê¿Ú̷η, ηıÒ˜

Î·È Ì ٷ Ê·ÚÌ·ÎÔ‰˘Ó·ÌÈο-Ê·ÚÌ·ÎÔÁÂÓÂÙÈο ¯·-

Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ·ÛıÂÓÔ‡˜ (29). ¶·Ú¿ÌÂÙÚÔÈ Ô˘

¤¯Ô˘Ó ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·¿-

ÓÙËÛ˘ ÛÙË ıÂڷ›· Â›Ó·È Ô ·ÚÈıÌfi˜ ÙˆÓ ‚Ï·ÛÙÒÓ

ÛÙÔ ·›Ì· ÙËÓ 8Ë Ë̤ڷ ıÂڷ›·˜, Ô Ì˘ÂÏfi˜ ÙËÓ 7Ë ‹

14Ë Ë̤ڷ ηÈ, ÚfiÛÊ·Ù·, Ë ÂÎÙ›ÌËÛË Ù˘ ÂÏ¿¯ÈÛÙ˘

˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ (30-34).

∏ ÔÌ¿‰· ÙÔ˘ BFM ÂÈÛ‹Á·Á ·fi ÙÔ 1983 ÙËÓ

ÚÔÁÓˆÛÙÈ΋ ·Í›· Ù˘ ·¿ÓÙËÛ˘ ÛÙËÓ Ú‰ÓÈ˙fiÓË.

∆· ÚˆÙfiÎÔÏÏ· BFM ¯ÚËÛÈÌÔÔÈÔ‡Ó ÙË Ì›ˆÛË ÙÔ˘

·ÚÈıÌÔ‡ ÙˆÓ ‚Ï·ÛÙÒÓ Û <1000/Ìl ÙËÓ 8Ë Ë̤ڷ, ÌÂ-

Ù¿ ·fi ¯ÔÚ‹ÁËÛË Ú‰ÓÈ˙fiÓ˘ Î·È ÌÈ·˜ ÂÓ‰ÔÚÚ·¯È-

·›·˜ ¤Á¯˘Û˘ MTX, ˆ˜ ÎÚÈÙ‹ÚÈÔ ÁÈ· ¢ÓÔ˚΋ Úfi-

ÁÓˆÛË (35). √ Ú˘ıÌfi˜ οı·ÚÛ˘ ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙÔ

ÂÚÈÊÂÚÈÎfi ·›Ì· ¤¯ÂÈ ÚÔÁÓˆÛÙÈ΋ ·Í›· Û ∆- Î·È µ-

ÛÂÈÚ¿˜ √§§. √ ·ÚÈıÌfi˜ ÙˆÓ ‚Ï·ÛÙÒÓ ÛÙÔ ·›Ì· 7 Ë̤-

Ú˜ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË ÔÏ˘·Ú·ÁÔÓÙÈ΋˜ ıÂڷ›·˜

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·77

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·ÔÙÂÏ› ›Û˘ ÚÔÁÓˆÛÙÈÎfi ·Ú¿ÁÔÓÙ· Û ¿ÏÏ·

ÚˆÙfiÎÔÏÏ· (32).

ŸÛÔÓ ·ÊÔÚ¿ ÙËÓ ·¿ÓÙËÛË ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ

ÔÛÙÒÓ, ÔÈ ·ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó Ì›ˆÛË ÙˆÓ ‚Ï·ÛÙÒÓ

Û <5% Û 7-14 Ë̤Ú˜ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ÔÏ˘-

·Ú·ÁÔÓÙÈ΋˜ ¯ËÌÂÈÔıÂڷ›·˜ ¤¯Ô˘Ó ȉȷ›ÙÂÚ· ¢-

ÓÔ˚΋ ÚfiÁÓˆÛË. ∏ ÌÔÚÊÔÏÔÁÈ΋ ·¿ÓÙËÛË ÛÙË ıÂ-

ڷ›· ÂÍ·ÎÔÏÔ˘ı› Ó· ¤¯ÂÈ ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›·

Î·È ÛÙ· ÙÚ¤¯ÔÓÙ· ÚˆÙfiÎÔÏÏ· ÙÔ˘ COG (37).

∏ ÂÎÙ›ÌËÛË Ù˘ ÚÒÈÌ˘ ·¿ÓÙËÛ˘ ÛÙË ıÂڷ›·,

Ì ÙÔÓ ˘ÔÏÔÁÈÛÌfi Ù˘ MRD ÛÙÔÓ Ì˘ÂÏfi, ·ÔÙÂÏ›

Û‹ÌÂÚ· ÙÔÓ ÈÔ ÈÛ¯˘Úfi ›Ûˆ˜ ÚÔÁÓˆÛÙÈÎfi ·Ú¿ÁÔÓÙ·,

Ô˘ ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ÁÈ· ·ÎÚÈ‚‹ Ù·ÍÈÓfiÌËÛË ÛÂ

ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘ Î·È ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂڷ›·˜

(34,38-40). √È ·ÛıÂÓ›˜ Ô˘ ÂÈÙ˘Á¯¿ÓÔ˘Ó ·ÓÔÛÔÏÔÁÈ-

΋ ‹ ÌÔÚȷ΋ ‡ÊÂÛË, Ô˘ ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ˆ˜ Ì›ˆÛË

ÙˆÓ ‚Ï·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Û <0,01% ÙˆÓ Â̇ÚËÓˆÓ

΢ÙÙ¿ÚˆÓ ÙÔ˘ Ì˘ÂÏÔ‡ ÛÙÔ Ù¤ÏÔ˜ Ù˘ ·ÁˆÁ‹˜ ÛÙËÓ

‡ÊÂÛË, ¤¯Ô˘Ó ÂÍ·ÈÚÂÙÈ΋ ÚfiÁÓˆÛË (41,42).

√§§ Û ÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ

πÛÙÔÚÈο, Ù· ÌÂÁ·Ï‡ÙÂÚ· ·È‰È¿ Î·È ÔÈ ¤ÊË‚ÔÈ ÌÂ

√§§ ¤¯Ô˘Ó ¯ÂÈÚfiÙÂÚË ÚfiÁÓˆÛË, Û ۯ¤ÛË Ì ٷ ÌÈ-

ÎÚfiÙÂÚ· ·È‰È¿. µÈÔÏÔÁÈο, ÔÈ ¤ÊË‚ÔÈ ‰È·Ê¤ÚÔ˘Ó

·fi Ù· ÌÈÎÚfiÙÂÚ· ·È‰È¿ Î·È ÚÔÛ¤Ú¯ÔÓÙ·È Ì ‰˘-

ÛÌÂÓ›˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ηٿ ÙË ‰È¿-

ÁÓˆÛË, fiˆ˜ ˘„ËÏfi˜ ·ÚÈıÌfi˜ Ï¢ÎÒÓ, ∆ ·ÓÔÛÔÊ·È-

ÓfiÙ˘Ô˜ Î·È ÌÂÁ·Ï‡ÙÂÚË Û˘¯ÓfiÙËÙ· ‰˘ÛÌÂÓÒÓ Î˘Ù-

Ù·ÚÔÁÂÓÂÙÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ (43). √È ‰È·ÊÔÚ¤˜, fï˜,

ÛÙËÓ ¤Î‚·ÛË ·Ô‰›‰ÔÓÙ·È Î·È ÛÙË ÏÈÁfiÙÂÚÔ ÂÈıÂÙÈ-

΋ ıÂڷ›· Ô˘ Ï·Ì‚¿ÓÔ˘Ó fiÙ·Ó ıÂڷ‡ÔÓÙ·È ÌÂ

ÚˆÙfiÎÔÏÏ· ÂÓËϛΈÓ. ¶ÚfiÛÊ·Ù· ‰ËÌÔÛȇÙËÎ·Ó 3

·Ó·‰ÚÔÌÈΤ˜ ÌÂϤÙ˜ ·fi ÙËÓ ∂˘ÚÒË Î·È Ì›· ÚÔ-

‰ÚÔÌÈ΋ ·fi ÙÔ DFCI ÛÙȘ ∏¶∞, Ô˘ ‰Â›¯ÓÔ˘Ó fiÙÈ ÔÈ

¤ÊË‚ÔÈ Î·È ÔÈ Ó·ÚÔ› ÂÓ‹ÏÈΘ Ì √§§ ¤¯Ô˘Ó ÛËÌ·-

ÓÙÈο ηχÙÂÚ· ÔÛÔÛÙ¿ ›·Û˘ fiÙ·Ó ıÂڷ‡ÔÓÙ·È

Ì ·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ· (44-47). ∏ Û‡ÁÎÚÈÛË

Ù˘ 5ÂÙÔ‡˜ ÂÈ‚›ˆÛ˘ ·ÛıÂÓÒÓ 15-21 ÂÙÒÓ ¤‰ÂÈÍÂ

̤¯ÚÈ Î·È 30% ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ›·Û˘ Ì ٷ

121086420

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E2A-PXB1 (n=19)Hyperdiploidy >50 chromosomes (n=112)

Other subtypes (n=148)

T-cell ALL (n=78)

MLL-AF4 (n=15)BCR-AµL (n=14)

TEL-AML1 (n=81)

∂ÈÎfiÓ· 2. ∫·Ì‡Ï˜ ÂÈ‚›ˆÛ˘ ˘ÔÔÌ¿‰ˆÓ Ì ‚¿ÛË Ù· ΢ÙÙ·ÚÔÁÂÓÂÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο (∞fi Pui C et al. NEJM 2004).

78 ∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹

Paediatriki 2009;72:10-15

¶›Ó·Î·˜ 2. ∞ÓÔÛÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË √§§

ÀÔÔÌ¿‰· ŒÎÊÚ·ÛË ·ÓÙÈÁfiÓˆÓ ™˘¯ÓfiÙËÙ· (%)

¶ÚÒÈÌË ÚÔ-B CD19+, CD22+, CD79a+, CD10+/-, CD7-, CD3-a, 57-65%cIgÌ-, sIgÎ-, sIgÏ-

¶ÚÔ-µ CD19+, CD22+, CD79a+,CD10+/-, CD7-,CD3-a, 20-25%cIgÌ+, sIgÌ-, sIgÎ-, sIgÏ-

ªÂÙ·‚·ÙÈ΋ ÚÔ-µ CD19+, CD22+, CD79a+, CD10+/-b, CD7-, CD3-a, 2-3%cIgÌ+, sIgÌ+, sIgÎ-, sIgÏ-

B- √§§ CD19+, CD22+, CD79a+, CD10+/-b, CD7-, CD3-a, 2-3%cIgÌ+, sIgÌ+, sIgÎ+, sIgÏ+

T- √§§ CD19+/-c, CD22-, CD79a-, CD10+/-b, 13-15%CD7+, CD3+a, cIgÌ-, sIgÌ-, sIgÎ-, sIgÏ-

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·78

Page 7: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

79√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·

¶·È‰È·ÙÚÈ΋ 2009;72:10-15

·È‰È·ÙÚÈο ÚˆÙfiÎÔÏÏ· ıÂڷ›·˜. ∞Ó Î·È ÔÈ ‰È·-

ÊÔÚ¤˜ ·˘Ù¤˜ ‰ÂÓ ÌÔÚÔ‡Ó Ó· ÂÚÌËÓ¢ıÔ‡Ó ÌfiÓÔ

·fi Ù· ‚ÈÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο Î·È ÙȘ ÁÂÓÂÙÈΤ˜

‰È·Ù·Ú·¯¤˜, Â›Ó·È È‰È·›ÙÂÚ· ·ÍÈÔÛËÌ›ˆÙÔ ÙÔ fiÙÈ ÔÈ

¤ÊË‚ÔÈ Î·È ÔÈ Ó·ÚÔ› ÂÓ‹ÏÈΘ, fiÙ·Ó ÂÓÙ¿ÛÔÓÙ·È ÛÂ

ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ Î·È ıÂڷ‡ÔÓÙ·È Ì ·È‰È·ÙÚÈο

ÚˆÙfiÎÔÏÏ·, ÌÔÚ› Ó· ÂÈÙ‡¯Ô˘Ó ÔÛÔÛÙ¿ ›·Û˘

Ô˘ Êı¿ÓÔ˘Ó ÙÔ 70%.

∆Ú¤¯Ô˘Û· ıÂڷ¢ÙÈ΋ ÛÙÚ·ÙËÁÈ΋ Î·È Ó¤Â˜

ÚÔÁÓˆÛÙÈΤ˜ ÔÌ¿‰Â˜

H Ù·ÍÈÓfiÌËÛË ÙˆÓ ·È‰ÈÒÓ Û ÔÌ¿‰Â˜ ÎÈÓ‰‡ÓÔ˘

¤¯ÂÈ ˆ˜ ÛÙfi¯Ô ÙËÓ ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂڷ›·˜, ¤ÙÛÈ

ÒÛÙ ÈÔ ÂÈıÂÙÈ΋ ηÈ, ÂÔ̤ӈ˜, ÈÔ ÙÔÍÈ΋ ıÂÚ·-

›· Ó· Ï·Ì‚¿ÓÔ˘Ó ÌfiÓÔ Ù· ·È‰È¿ Ô˘ ÙË ¯ÚÂÈ¿˙Ô-

ÓÙ·È ÁÈ· Ó· È·ıÔ‡Ó.

∏ ÚfiÔ‰Ô˜ ÛÙË ÌÔÚȷ΋ Ù·ÍÈÓfiÌËÛË Î·È ÙËÓ ·Ó›-

¯Ó¢ÛË ÁÂÓÂÙÈÎÒÓ ‰È·Ù·Ú·¯ÒÓ Ô‰ËÁ› ÛÙËÓ ·Ó·˙‹-

ÙËÛË Ô‰ÒÓ Ô˘ ÂÓ¤¯ÔÓÙ·È ÛÙËÓ ·ÓÙ›ÛÙ·ÛË ÛÙË ıÂÚ·-

›·, ·ÏÏ¿ Î·È Ó¤ˆÓ ıÂڷ¢ÙÈÎÒÓ ÛÙfi¯ˆÓ (48). ™Ë-

Ì·ÓÙÈÎfi ·Ú¿‰ÂÈÁÌ· ·ÔÙÂÏ› Ë ¯ÔÚ‹ÁËÛË ÙÔ˘

imatinib mesylate ÛÙ· ·È‰È¿ Ì √§§ Î·È Û‡ÓÙËÍË

ÙˆÓ bcr-abl ÁÔÓȉ›ˆÓ (20).

∆· ÚˆÙfiÎÔÏÏ· ÙÔ˘ BFM ηٷٿÛÛÔ˘Ó ÛÙËÓ

ÔÌ¿‰· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Ù· ·È‰È¿ Ì ÊÙˆ¯‹ ·¿-

ÓÙËÛË ÛÙËÓ Ú‰ÓÈ˙fiÓË, ηıÒ˜ Î·È Ù· ·È‰È¿ Ô˘

¤¯Ô˘Ó ÙȘ ÌÂÙ·ı¤ÛÂȘ t(9;22) Î·È t(4;11). ™ÙÔ ÚˆÙfi-

ÎÔÏÏÔ ALL BFM 2000, ÔÈ ·ÛıÂÓ›˜ Ù·ÍÈÓÔÌÔ‡ÓÙ·È ÌÂ

‚¿ÛË fi¯È ÌfiÓÔ ÙËÓ ·¿ÓÙËÛË ÛÙËÓ Ú‰ÓÈ˙fiÓË Î·È

ÙȘ ΢ÙÙ·ÚÔÁÂÓÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, ·ÏÏ¿ Î·È ÙËÓ

MRD ÙËÓ Â‚‰ÔÌ¿‰· 4 Î·È 12. ŒÙÛÈ, ˜ ÛÙ·ıÂÚÔ‡ ÎÈÓ-

‰‡ÓÔ˘ ηٷٿÛÛÔÓÙ·È Ù· ·È‰È¿ Ì ·ÚÓËÙÈ΋ MRD

ÙËÓ 4Ë Î·È 12 ‚‰ÔÌ¿‰·, ÂӉȿÌÂÛÔ˘ ÎÈÓ‰‡ÓÔ˘ Ù·

·È‰È¿ Ì MRD <10-3 Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Ù· ·È-

‰È¿ Ì MRD ≥10-3 ÙË 12Ë Â‚‰ÔÌ¿‰·. ∆· ·È‰È¿ ÌÂ

ÊÙˆ¯‹ ·¿ÓÙËÛË ÛÙËÓ Ú‰ÓÈ˙fiÓË ıˆÚÔ‡ÓÙ·È

˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔÓ ÚÔÛ‰ÈÔÚÈ-

ÛÌfi Ù˘ MRD, ÂÓÒ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ›Û˘ ıˆ-

ÚÔ‡ÓÙ·È ÔÈ ·ÛıÂÓ›˜ Ì ÌÂÙ¿ıÂÛË t(9;22) Î·È t(4;11),

·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ ·¿ÓÙËÛË ÛÙË ıÂڷ›· (49).

∞fi ÙÔ DFCI ¤¯ÂÈ ÚÔÙ·ı› ›Û˘ Ó¤Ô Û‡ÛÙËÌ·

Ù·ÍÈÓfiÌËÛ˘ ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì √§§ µ-ÛÂÈÚ¿˜. √È

·ÛıÂÓ›˜ ·Ú¯Èο Ù·ÍÈÓÔÌÔ‡ÓÙ·È ˆ˜ ÛÙ·ıÂÚÔ‡ ‹ ˘„Ë-

ÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Ì ‚¿ÛË ÙËÓ ËÏÈΛ·, ÙÔÓ ·ÚÈıÌfi ÙˆÓ

Ï¢ÎÒÓ Î·È ÙËÓ ·ÚÔ˘Û›· ÓfiÛÔ˘ ÛÙÔ ∫¡™. ªÂÙ¿ ÙË

¯ÔÚ‹ÁËÛË ÙˆÓ 4 ‚‰ÔÌ¿‰ˆÓ Ù˘ ıÂڷ›·˜ ·Áˆ-

Á‹˜ ÛÙËÓ ‡ÊÂÛË, ÔÈ ·ÛıÂÓ›˜ Ì MRD ≥0,1% Ù·ÍÈÓÔ-

ÌÔ‡ÓÙ·È ˆ˜ Ôχ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÂÓÒ ÂΛÓÔÈ ÌÂ

MRD <0,1% Û˘Ó¯›˙Ô˘Ó fiˆ˜ Ù·ÍÈÓÔÌ‹ıËÎ·Ó ·Ú¯È-

ο. ™Ùfi¯Ô˜ Â›Ó·È Ó· ÚÔÛ‰ÈÔÚÈÛı› Â¿Ó Ë ÂÓÙ·ÙÈÎÔ-

Ô›ËÛË Ù˘ ıÂڷ›·˜ ı· ‚ÔËı‹ÛÂÈ ÙÔ˘˜ ·ÛıÂÓ›˜

Ì ˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ ÛÙÔ Ù¤ÏÔ˜ Ù˘ ·ÁˆÁ‹˜

ÛÙËÓ ‡ÊÂÛË. √È ·ÛıÂÓ›˜ Ì ∆-√§§ Ï·Ì‚¿ÓÔ˘Ó ıÂ-

ڷ›· ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÓÂÍ¿ÚÙËÙ· ·fi ÙËÓ MRD

ÛÙË Ê¿ÛË ·˘Ù‹. ø˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÓÙÈÌÂÙˆ›˙Ô-

ÓÙ·È Â›Û˘ ÔÈ ·ÛıÂÓ›˜ Ì ˘Ô‰ÈÏÔÂȉÈÛÌfi, ·ÓÂ-

Í¿ÚÙËÙ· ·fi ÙÔÓ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô ‹ ÙËÓ ÂÏ¿¯ÈÛÙË

˘ÔÏÂÈfiÌÂÓË ÓfiÛÔ.

™ÙÔ St. Jude Children’s Research Hospital, Ë Ù·ÍÈ-

ÓfiÌËÛË Â›Û˘ ‚·Û›˙ÂÙ·È Î˘Ú›ˆ˜ ÛÙ· ›‰· Ù˘

MRD ÌÂÙ¿ ·fi 6 ‚‰ÔÌ¿‰Â˜ ıÂڷ›·˜ ·ÁˆÁ‹˜

ÛÙËÓ ‡ÊÂÛË Î·È ‰È·ÎÚ›ÓÂÈ ÙÔ˘˜ ·ÛıÂÓ›˜ Û ¯·ÌËÏÔ‡

(MRD <0,01%), ÛÙ·ıÂÚÔ‡ (MRD 0,01% ¤ˆ˜ <1%)

Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (MRD >1%).

To 2000, Ë ‰ËÌÈÔ˘ÚÁ›· ÙÔ˘ COG ·fi ÙË Û˘Ó¤Óˆ-

ÛË ÙÔ˘ CCG Î·È ÙÔ˘ POG ¤‰ˆÛ ÙË ‰˘Ó·ÙfiÙËÙ· Ó·

·Ó·Ï˘ıÔ‡Ó ·Ó·‰ÚÔÌÈο Ù· ÎÏÈÓÈο ‚ÈÔÏÔÁÈο ‰Â‰Ô-

̤ӷ Î·È Ë ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· 18.000

·ÛıÂÓÒÓ Ì √§§, ËÏÈΛ·˜ 1-21.99 ÂÙÒÓ, Î·È Ó· ÚÔ-

Ù·ı› ¤Ó· Ó¤Ô Û‡ÛÙËÌ· Ù·ÍÈÓfiÌËÛ˘ (37). ªÂ ‚¿ÛË

ÙÔ ÚÔÙÂÈÓfiÌÂÓÔ Û‡ÛÙËÌ·, ÔÈ ·ÛıÂÓ›˜ Ì √§§ µ-

ÛÂÈÚ¿˜ ‰È·ÎÚ›ÓÔÓÙ·È Û ¯·ÌËÏÔ‡, ÛÙ·ıÂÚÔ‡, ˘„ËÏÔ‡

Î·È Ôχ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ·,

ÙÔÓ ·ÚÈıÌfi ÙˆÓ Ï¢ÎÒÓ, ÙȘ ΢ÙÙ·ÚÔÁÂÓÂÙÈΤ˜ ‰È·-

Ù·Ú·¯¤˜, ÙÔÓ Ì˘ÂÏfi Ù˘ Ë̤ڷ˜ 14 Î·È ÙËÓ MRD ÛÙÔ

Ù¤ÏÔ˜ Ù˘ ·ÁˆÁ‹˜ ÛÙËÓ ‡ÊÂÛË.

∞Ú¯Èο ‰È·ÎÚ›ÓÔÓÙ·È Û ÛÙ·ıÂÚÔ‡ Î·È ˘„ËÏÔ‡

ÎÈÓ‰‡ÓÔ˘ Ì ‚¿ÛË ÙËÓ ËÏÈΛ· Î·È ÙÔÓ ·ÚÈıÌfi ÙˆÓ

Ï¢ÎÒÓ. √È ·ÛıÂÓ›˜ Ì ÌÂÙ¿ıÂÛË t(12;21) ‹ ÙÚÈÛˆ-

̛˜ 4, 10 Î·È 17 ıˆÚÔ‡ÓÙ·È "standard risk-low".

ŸÏ· Ù· ·È‰È¿ Ì ∆-√§§ Ù·ÍÈÓÔÌÔ‡ÓÙ·È ˆ˜ ˘„ËÏÔ‡

ÎÈÓ‰‡ÓÔ˘, ·ÓÂÍ¿ÚÙËÙ· ·fi ÙÔÓ ·ÚÈıÌfi ÙˆÓ Ï¢ÎÒÓ.

∏ ÙÂÏÈ΋ Ù·ÍÈÓfiÌËÛË ÛÙË Û˘Ó¤¯ÂÈ· ‚·Û›˙ÂÙ·È ÛÙËÓ

ÚÒÈÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· Î·È ÛÙ· ΢ÙÙ·ÚÔÁÂ-

ÓÂÙÈο ¯·Ú·ÎÙËÚÈÛÙÈο. ∏ ÔÌ¿‰· ÙˆÓ Ôχ ˘„ËÏÔ‡

ÎÈÓ‰‡ÓÔ˘ ÂÚÈÏ·Ì‚¿ÓÂÈ ·ÛıÂÓ›˜ Ì t(9;22), ˘Ô‰È-

ÏÔÂȉÈÛÌfi (<44 ¯ÚˆÌÔÛÒÌ·Ù·), MLL ·Ó·‰È·Ù¿-

ÍÂȘ, ·ÚÁ‹ ÌÔÚÊÔÏÔÁÈ΋ ·¿ÓÙËÛË, ª3 Ì˘ÂÏfi ÙËÓ

Ë̤ڷ 29 ‹ ª2 Ì˘ÂÏfi ‹/Î·È MRD >1% ÙȘ Ë̤Ú˜ 29

Î·È 43.

ŸÏ· Ù· Û˘ÛÙ‹Ì·Ù· Ù·ÍÈÓfiÌËÛ˘ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô

Ó· ‚ÂÏÙÈÒÛÔ˘Ó Ù· ÔÛÔÛÙ¿ ›·Û˘ ÙˆÓ ·ÛıÂÓÒÓ Ô˘

‰È·ÙÚ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚÔ Î›Ó‰˘ÓÔ ˘ÔÙÚÔ‹˜ Ì ÙËÓ

ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂڷ›·˜ ÙÔ˘˜ Î·È Ó· ÂÚÈÔÚ›-

ÛÔ˘Ó ÙËÓ ÙÔÍÈÎfiÙËÙ· Ù˘ ıÂڷ›·˜ Û ·ÛıÂÓ›˜ ¯·-

ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÌÂÈÒÓÔÓÙ·˜ ÂÚ·ÈÙ¤Úˆ ÙË ¯ËÌÂÈÔ-

ıÂڷ›·.

µÈ‚ÏÈÔÁÚ·Ê›·

1. Carrol WL, Bhojwani DJ, et al. Pediatric acute lympho-

blastic leukemia. Hematology (Am Soc ∏ematol Educ

Program) 2003;102-131.

2. Pui CH, Evans WE. Treatment of acute lymphoblastic

leukemia. N Engl J Med 2006;354:166-178.

3. Winick NJ, Carroll WL, Hunger SP. Childhood leukemia-

new advances and challenges. N Engl J Med 2004;351:

601-603.

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·79

Page 8: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

80 ∂. ™ÙÂȷοÎË, M. ∫·ÏÌ·ÓÙ‹

Paediatriki 2009;72:10-15

4. Smith M, Arthur D, Camitta B, et al. Uniform approach to

risk classification and treatment assignment for children

with acute lymphoblastic leukemia. J Clin Oncol 1996;14:

18-24.

5. Moricke A, Zimmermann M, Reiter A, et al. Prognostic

impact of age in children and adolescents with acute

lymphoblastic leukemia: Data from the trials ALL- BFM

86, 90 and 95. Klin Paediatr 2005;217:310-320.

6. Mahmoud HH, Rivera GK, Hancock ML, et al. Low

leukocyte counts with blast cells in cerebrospinal fluid of

children with newly diagnosed acute lymphoblastic

leukemia. N Engl J Med 1993;329:314-319.

7. Pui CH, Mahmoud HH, Rivera GK, et al. Early

intensification of intrathecal chemotherapy virtually

eliminates central nervous system relapse in children with

acute lymphoblastic leukemia. Blood 1998;92:411-415.

8. Gajjar A, Harrison PL, Sandlund JT, et al. Traumatic

lumbar puncture at diagnosis adversely affects outcome in

childhood acute lymphoblastic leukemia. Blood 2000;96:

3381-3384.

9. Borowitz MJ, Bray R, Gascoyne R, et al. US-Canadian

Consensus recommendations on the immunophenotypic

analysis of hematologic neoplasia by flow cytometry: data

analysis and interpretation. Consensus Development

Conference. Cytometry 1997;30:236-244.

10. Pui CH, Behm FG, Crist WM. Clinical and biologic

relevance of immunologic marker studies in childhood

acute lymphoblastic leukemia. Blood 1993;82:343-362.

11. Trueworthy R, Shuster J, Look T, et al. Ploidy of

lymphoblasts is the strongest predictor of treatment

outcome in B-progenitor cell acute lymphoblastic

leukemia of childhood: a Pediatric Oncology Group study.

J Clin Oncol 1992;10:606-613.

12. Arico M, Valsecchi MG, Rizzari C, et al. Long-term results of

the AIEOP-ALL-95 trial for childhood acute lymphoblastic

leukemia: Insight on the prognostic value of DNA index in

the framework of Berlin-Frankfurt-Muenster-based chemo-

therapy. J Clin Oncol 2008;26:283-289.

13. Sutcliffe MJ, Shuster JJ, Sather HN, et al.: High

concordance from independent studies by the Children's

Cancer Group (CCG) and Pediatric Oncology Group

(POG) associating favorable prognosis with combined

trisomies 4, 10, and 17 in children with NCI Standard-Risk

B-precursor Acute Lymphoblastic Leukemia: a COG

initiative. Leukemia 2005;19:734-740.

14. Nachman JB, Heerema NA, Sather H, et al. Outcome of

treatment in children with hypodiploid acute lympho-

blastic leukaemia. Blood 2007;110:1112-1115.

15. Loh ML, Rubnitz JE. TEL/AML1-positive pediatric leukemia:

prognostic significance and therapeutic approaches. Curr

Opin Hematol 2002;9:345-352.

16. Seeger K, Adams HP, Buchwald D, et al. TEL-AML1 fusion

transcript in relapsed childhood acute lymphoblastic

leukemia: the BFM Study Group. Blood 1998;91:1716-

1722.

17. Loh ML, Goldwasser MA, Silverman LB, et al. Prospective

analysis of TEL/AML1-positive patients treated on Dana-

Farber Cancer Institute Consortium Protocol 95-01. Blood

2006;107:4508-4513.

18. Uckun FM, Sensel MG, Sather HN, et al. Clinical

significance of translocation t(1;19) in childhood acute

lymphoblastic leukemia in the context of contemporary

therapies: a report from the Children's Cancer Group. J

Clin Oncol 1998;16:527-535.

19. Arico M, Valsecchi MG, Camitta B, et al. Outcome of

treatment in children with Philadelphia chromosome-

positive acute lymphoblastic leukemia. N Engl J Med

2000;342:998-1006.

20. Jones LK, Saha V. Philadelphia positive acute lympho-

blastic leukaemia of childhood. Br J Haematol 2005;130:

489-500.

21. Pui CH, Gaynon PS, Boyett JM, et al. Outcome of

treatment in childhood acute lymphoblastic leukaemia

with rearrangements of the 11q23 chromosomal region.

Lancet 2002;359:1909-1915.

22. Kosaka Y, Koh K, Kinukawa N, et al. Infant acute

lymphoblastic leukemia with MLL gene rearrangements:

outcome following intensive chemotherapy and hemato-

poietic stem cell transplantation. Blood 2004;104:3527-3534.

23. Hilden JM, Dinndorf PA, Meerbaum SO, et al. Analysis of

prognostic factors of acute lymphoblastic leukemia in

infants: report on CCG 1953 from the Children's Oncology

Group. Blood 2006;108:441-451.

24. Uckun FM, Sensel MG, Sun L, et al. Biology and treatment

of childhood T-lineage acute lymphoblastic leukemia.

Blood 1998;91:735-746.

25. Goldberg JM, Silverman LB, Levy DE, et al. Childhood T-

cell acute lymphoblastic leukemia: the Dana-Farber

Cancer Institute acute lymphoblastic leukemia consortium

experience. J Clin Oncol 2003;21:3616-3622.

26. van Grotel M, Meijerink JP, van Wering ER, et al. Prognostic

significance of molecular-cytogenetic abnormalities in

pediatric T-ALL is not explained by immunophenotypic

differences. Leukemia 2008;22:124-131.

27. Weng AP, Ferrando AA, Lee W, et al. Activating mutations

of NOTCH1 in human T cell acute lymphoblastic

leukemia. Science 2004;306:269-271.

28. Breit S, Stanulla M, Flohr T, et al. Activating NOTCH1

mutations predict favorable early treatment response and

long-term outcome in childhood precursor T-cell

lymphoblastic leukemia. Blood 2006;108:1151-1157.

29. Gaynon PS, Desai AA, Bostrom BC, et al. Early response to

therapy and outcome in childhood acute lymphoblastic

leukemia: a review. Cancer 1997;80:1717-1726.

30. Rautonen J, Hovi L, Siimes MA. Slow disappearance of

peripheral blast cells: an independent risk factor indicating

poor prognosis in children with acute lymphoblastic

leukemia. Blood 1988;71: 989-991.

31. Steinherz PG, Gaynon PS, Breneman JC, et al. Cyto-

reduction and prognosis in acute lymphoblastic leukaemia

- the importance of early marrow response: report from the

Childrens Cancer Group. J Clin Oncol 1996;14:389-398.

32. Gajjar A, Ribeiro R, Hancock ML, et al. Persistence of

circulating blasts after 1 week of multiagent chemotherapy

confers a poor prognosis in childhood acute lymphoblastic

leukemia. Blood 1995;86:1292-1295.

33. Griffin TC, Shuster JJ, Buchanan GR, et al. Slow

disappearance of peripheral blood blasts is an adverse

prognostic factor in childhood T cell acute lymphoblastic

leukemia: a Pediatric Oncology Group study. Leukemia

2000;14:792-795.

34. Cavé H, van der Werff ten Bosch J, Suciu S, et al. Clinical

significance of minimal residual disease in childhood acute

lymphoblastic leukemia. European Organization for

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·80

Page 9: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

81√Í›· ÏÂÌÊÔ‚Ï·ÛÙÈ΋ Ï¢¯·ÈÌ›·

¶·È‰È·ÙÚÈ΋ 2009;72:10-15

Research and Treatment of Cancer - Childhood LeukemiaCooperative Group. N Engl J Med 1998;339:591-598.

35. Reiter A, Schrappe M, Ludwig W-D, et al. Chemotherapyin 998 unselected childhood acute lymphoblastic leukemiapatients. Results and conclusions of the multicenter trialALL-BFM 86. Blood 1994;84:3122-3133.

36. Schrappe M, Reiter A, Zimmermann M, et al. Long termresults of four consecutive trials in childhood ALLperformed by the ALL-BFM study group from 1981 to1995. Leukemia 2000;14:2205-2222.

37. Schultz KR, Pullen DJ, Sather HN, et al. Risk- andresponse-based classification of childhood B-precursoracute lymphoblastic leukemia: a combined analysis ofprognostic markers from the POG and CCG. Blood 2007;109:926-935.

38. van Dongen JJ, Seriu T, Panzer-Gruemayer ER, et al.Prognostic value of minimal residual disease in acutelymphoblastic leukaemia in childhood. Lancet 1998;352:1731-1738.

39. Coustan-Smith E, Sancho J, Hancock ML, et al. Clinicalimportance of minimal residual disease in childhood acutelymphoblastic leukemia. Blood 2000;96:2691-2696.

40. Björklund E, Mazur J, Söderhäll S, et al. Flow cytometricfollow-up of minimal residual disease in bone marrowgives prognostic information in children with acutelymphoblastic leukemia. Leukemia 2003;17:138-148.

41. Panzer-Grümayer ER, Schneider M, Panzer S, et al. Rapidmolecular response during early induction chemotherapypredicts a good outcome in childhood acute lymphoblasticleukemia. Blood 2000;95:790-794.

42. Zhou J, Goldwasser MA, Li A, et al. Quantitative analysis of

minimal residual disease predicts relapse in children withB-lineage acute lymphoblastic leukemia in DFCI ALLConsortium Protocol 95-01. Blood 2007;110:1607-1611.

43. Santana VM, Dodge RK, Crist WM, et al. Presentingfeatures and treatment outcome of adolescents with acutelymphoblastic leukemia. Leukemia 1990;4:87-90.

44. Boissel N, Auclerc MF, Lhéritier V, et al. Should adolescentswith acute lymphoblastic leukemia be treated as old childrenor young adults? Comparison of the French FRALLE-93 andLALA-94 trials. J Clin Oncol 2003;21:774-780.

45. de Bont JM, Holt B, Dekker AW, et al. Significant differencein outcome for adolescents with acute lymphoblasticleukemia treated on pediatric vs adult protocols in theNetherlands. Leukemia 2004;18:2032-2035.

46. Ramanujachar R, Richards S, Hann I, et al. Adolescentswith acute lymphoblastic leukaemia: outcome on UKnational paediatric (ALL97) and adult (UKALLXII/E2993)trials. Pediatr Blood Cancer 2007;48:254-261.

47. Barry E, Deangelo DJ, Neuberg D, et al. Favorable outcomefor adolescents with acute lymphoblastic leukemia treated onDana-Farber Cancer Institute acute lymphoblastic leukemiaConcortium Protocols. J Clin Oncol 2007;25:813-819.

48. Bhojwani D, Kang H, Moskowitz NP, et al. Biologicpathways associated with relapse in childhood acutelymphoblastic leukemia: a Children's Oncology Groupstudy. Blood 2006;108:711-717.

49. Moricke A, Reiter A, Zimmermann M, et al. Risk-adjustedtherapy of acute lymphoblastic leukemia can decreasetreatment burden and improve survival: treatment results of2169 unselected pediatric and adolescent patients enrolledin the trial ALL-BFM 95. Blood 2008;111:4477-4489.

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82 ∞¡∞™∫√¶∏™∏ REVIEW ARTICLE

Paediatriki 2009;72:82-000

§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ - ¡ÂfiÙÂÚ· ıÂڷ¢ÙÈο ‰Â‰Ô̤ӷ

µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹

¶ÂÚ›ÏË„Ë: ∆· ÏÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ ·ÔÙÂÏÔ‡Ó ÙËÓ 3Ë ÈÔ Û˘¯Ó‹ ηÎÔ‹ıÂÈ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜. ¢È·-ÎÚ›ÓÔÓÙ·È Û ‰‡Ô Ù‡Ô˘˜, Ù· ÌË-Hodgkin Ô˘ Â›Ó·È Î·È ÈÔ Û˘¯Ó¿ Î·È ·ÔÙÂÏÔ‡Ó ÙÔ 60% ÙÔ˘ Û˘ÓfiÏÔ˘,Î·È Ù· Hodgkin ÏÂÌÊÒÌ·Ù·. ∏ ıÂڷ›· ÙÔ˘˜ ÂÚÈÏ·Ì‚¿ÓÂÈ Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂڷ¢ÙÈÎÒÓ Ê·ÚÌ¿-ÎˆÓ Î·È ·ÎÙÈÓÔıÂڷ›·˜ Î·È Ë ÚfiÁÓˆÛË Û˘Ó‹ıˆ˜ Â›Ó·È ÂÍ·ÈÚÂÙÈο ηϋ, Û ·ÓÙ›ıÂÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ.¶·ÚfiÏ· ·˘Ù¿, οÔÈ· ·È‰È¿ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ˘ÔÙÚÔÈ¿˙Ô˘Ó ‹ Ó· ÂÌÊ·Ó›˙Ô˘Ó ·ÒÙÂÚ˜ ÂÈÏÔΤ˜·fi ÙË ıÂڷ›·. √È ÓÂfiÙÂÚ˜ ÎÏÈÓÈΤ˜ ÌÂϤÙ˜ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô ÙËÓ ·Ó·ÁÓÒÚÈÛË ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·Áfi-ÓÙˆÓ, ÙËÓ ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂڷ¢ÙÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ Î·È ÙËÓ ·ÓÙ·-fiÎÚÈÛË ÛÙË ıÂڷ›· οı ·ÛıÂÓÔ‡˜, ¤ÙÛÈ ÒÛÙ ӷ ‚ÂÏÙȈı› Ë ÂÈ‚›ˆÛË, Ì ٷ˘Ùfi¯ÚÔÓË ÂÏ¿ÙÙˆÛË Ù˘̷ÎÚÔ¯ÚfiÓÈ·˜ ÙÔÍÈÎfiÙËÙ·˜ Ù˘ ıÂڷ›·˜. ∏ ·Ó·Î¿Ï˘„Ë Ó¤ˆÓ Ê·ÚÌ¿ÎˆÓ Ô˘ ÛÙÔ¯Â‡Ô˘Ó ÂȉÈο Û ÌÔ-ÚÈ·Îfi Â›Â‰Ô ·Ó·Ì¤ÓÂÙ·È Ó· Û˘Ì‚¿ÏÂÈ ÛËÌ·ÓÙÈο ÛÙÔÓ ÙÔ̤· ·˘Ùfi. ¶·Ú¿ÏÏËÏ·, Ë ·Ó¿Ù˘ÍË Â˘·›ÛıË-ÙˆÓ ÌÂıfi‰ˆÓ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂڷ›· Î·È Ù˘ ¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ Ù˘˘ÔÙÚÔ‹˜ Î·È Ë ·Ó›¯Ó¢ÛË ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ ı· ‚ÔËı‹ÛÔ˘Ó ÛÙËÓ ÚÒÈÌË ·Ó·ÁÓÒÚÈÛËÙ˘ ¯ËÌÂÈÔ-¢·ÈÛıËÛ›·˜ ‹ ·ÓıÂÎÙÈÎfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Î·È ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ηٷÏÏËÏfiÙÂÚÔ˘ ıÂڷ¢ÙÈ-ÎÔ‡ Û¯‹Ì·ÙÔ˜. ∏ ¯Ú‹ÛË Û‡Á¯ÚÔÓˆÓ ‚ÈÔÏÔÁÈÎÒÓ ÌÂÏÂÙÒÓ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È ÂÎÙÂÓ‹ ÁÔÓȉȷ΋ ηÈÚˆÙÂ˚ÓÈ΋ ·Ó¿Ï˘ÛË ı· ‰ÒÛÂÈ Ó¤· ‰È¿ÛÙ·ÛË ÛÙËÓ Î·Ù·ÓfiËÛË ÙˆÓ ·ıÔÁÂÓÂÙÈÎÒÓ Ì˯·ÓÈÛÌÒÓ, ÛÙËÓ Ù·-ÍÈÓfiÌËÛË ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Î·È ÛÙË ‚ÂÏÙÈÛÙÔÔ›ËÛË ÙˆÓ ıÂڷ¢ÙÈÎÒÓ ÌÂıfi‰ˆÓ.

§¤ÍÂȘ ÎÏÂȉȿ: §¤Ìʈ̷, ·È‰È΋ ËÏÈΛ·, Hodgkin, non-Hodgkin, ¯ËÌÂÈÔıÂڷ›·, ·ÎÙÈÓÔıÂڷ›·.

Childhood lymphomas - Novel therapeutic approaches

V. Danilatou, M. Kalmanti

Abstract: Childhood lymphoma is the third most common paediatric malignancy. Non-Hodgkinlymphoma accounts for approximately 60% of all lymphomas and Hodgkin’s disease for the rest.Combined chemotherapy and radiotherapy is the treatment of choice and the prognosis is excellent forchildren and adolescents, in contrast with that for adults. However, some patients relapse or suffer fromlong-term toxicity. πn order to improve the overall survival, the aims of current clinical trials are theidentification of prognostic markers and the individualization of treatment according to risk group andtreatment response. Targeted therapies directed against tumour-characteristic molecules will probablycontribute to the reduction of toxicity. The development of sensitive methods for monitoring minimalresidual disease and early diagnosis of relapse will assist in choosing the appropriate treatment schedulefor each patient. Genomic and proteomic analysis will provide new dimensions in the understanding ofthe pathogenetic mechanisms, in disease classification and in the optimization of treatment strategies.

Key words: Lymphoma, childhood, Hodgkin, non-Hodgkin, chemotherapy, radiotherapy.

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜/√ÁÎÔÏÔÁ›·˜,¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ∫Ú‹Ù˘

AÏÏËÏÔÁÚ·Ê›·:

µ·ÛÈÏÈ΋ ¢·ÓËÏ¿ÙÔ˘[email protected]@gmail.com¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜/√ÁÎÔÏÔÁ›·˜,¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô∏Ú·ÎÏ›Ԣ ∫Ú‹Ù˘, ∆.∫. 71110

Department of PaediatricHematology/Oncology,University Hospital ofHeraklion, Crete, Greece

Correspondence:

Vassiliki [email protected]@gmail.comDepartment of PaediatricHematology/Oncology,University Hospital ofHeraklion, 71110 Crete,Greece

™˘ÓÙÔÌÔÁڷʛ˜:

∞§ª∫ ·Ó·Ï·ÛÙÈÎfi ϤÌʈ̷ ·fi ÌÂÁ¿Ï·Î‡ÙÙ·Ú·

µ§ Burkitt ϤÌʈ̷¢§ªµ§ ‰È¿¯˘ÙÔ Ï¤Ìʈ̷ ·fi ÌÂÁ¿Ï·

µ-ÏÂÌÊÔ·ÙÙ·Ú· ∏§ Hodgkin ÏÂÌÊÒÌ·Ù·∫¡™ ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ·§∂ ÏÂÌÊÔÂÈÎÚ·Ù¤˜ §§ ÏÂÌÊÔ‚Ï·ÛÙÈÎfi ϤÌʈ̷§¶ ÏÂÌÊÔÂÓÈÎfiª∏§ ÌË-Hodgkin ÏÂÌÊÒÌ·Ù·ª∫ ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ √§∂∏§ Ô˙҉˜ ÏÂÌÊÔÂÈÎÚ·Ù¤˜ √™ Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË

∂ÈÛ·ÁˆÁ‹

∆· ÏÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿ ·ÔÙÂÏÔ‡Ó ÂÚ›-Ô˘ ÙÔ 13% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ Î·ÎÔËıÂÈÒÓ Ù˘·È‰È΋˜ ËÏÈΛ·˜ (1). ¢È·ÎÚ›ÓÔÓÙ·È Û ‰‡Ô Ù‡-Ô˘˜: Ù· ÌË-Hodgkin (ª∏§), Ô˘ Â›Ó·È Î·È ÈÔÛ˘¯Ó¿ Î·È ·ÔÙÂÏÔ‡Ó ÙÔ 60% ÙÔ˘ Û˘ÓfiÏÔ˘, Î·È Ù·Hodgkin ÏÂÌÊÒÌ·Ù· (∏§). ¶ÚfiÎÂÈÙ·È ÁÈ· ÎψÓÈ-Τ˜ ηÎÔ‹ıÂȘ ˘ÂÚϷۛ˜ ÙˆÓ µ Î·È ∆-ÏÂÌÊÔ-΢ÙÙ¿ÚˆÓ Û ‰È¿ÊÔÚ· ÛÙ¿‰È· ˆÚ›Ì·ÓÛ˘ (2).

∞. MË Hodgkin §ÂÌÊÒÌ·Ù· (ª∏§)

™Â ·ÓÙ›ıÂÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ Ô˘ ·Ó·-Ù‡ÛÛÔ˘Ó ÈÔ Û˘¯Ó¿ ‚Ú·‰¤ˆ˜ ·Ó·Ù˘ÛÛfiÌÂÓ·,

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·82

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83§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿

¶·È‰È·ÙÚÈ΋ 2009;72:83-000

¯·ÌËÏÔ‡ ‚·ıÌÔ‡ ηÎÔ‹ıÂÈ·˜ ÏÂÌÊÒÌ·Ù·, Ù· ª∏§

ÛÙ· ·È‰È¿ Â›Ó·È ÈÔ Û˘¯Ó¿ ÂÈıÂÙÈο ÏÂÌÊÒÌ·Ù·

˘„ËÏÔ‡ ‚·ıÌÔ‡ ηÎÔ‹ıÂÈ·˜ (1). ∆Ô ÈÔ Û˘¯Ófi ϤÌ-

ʈ̷ ÛÙ· ·È‰È¿ Â›Ó·È ÙÔ Ï¤Ìʈ̷ Burkitt (µ§) ηÈ

·ÎÔÏÔ˘ıÔ‡Ó ÙÔ ÏÂÌÊÔ‚Ï·ÛÙÈÎfi ϤÌʈ̷ (§§), ÙÔ

‰È¿¯˘ÙÔ Ï¤Ìʈ̷ ·fi ÌÂÁ¿Ï· µ-ÏÂÌÊÔ·ÙÙ·Ú·

(¢§ªµ§) Î·È ÙÔ ·Ó·Ï·ÛÙÈÎfi ϤÌʈ̷ ·fi ÌÂÁ¿Ï·

·ÙÙ·Ú· (∞§ª∫). √È ˘fiÙ˘ÔÈ ª∏§ ¯·Ú·ÎÙËÚ›˙Ô-

ÓÙ·È ·fi ‰È·ÊÔÚÂÙÈΤ˜ ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ, fiˆ˜

ÔÈΛÏÔ˘ ‚·ıÌÔ‡ ÚԉȿıÂÛË ÁÈ· ‰È‹ıËÛË ÙÔ˘ Ì˘Â-

ÏÔ‡ ÙˆÓ ÔÛÙÒÓ Î·È/‹ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘-

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ÁÂÓÂÙÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ·, Ì ·ÔÙ¤ÏÂÛÌ· Ó· ··ÈÙÔ‡Ó

‰È·ÊÔÚÂÙÈΤ˜ ıÂڷ¢ÙÈΤ˜ ÚÔÛÂÁÁ›ÛÂȘ (3).

ª¤¯ÚÈ ÚfiÛÊ·Ù·, ˘‹Ú¯·Ó ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜

ÚÔÛÂÁÁ›ÛÂȘ ÁÈ· ÙÔÓ ‰È·¯ˆÚÈÛÌfi ÙˆÓ ıÂڷ¢ÙÈÎÒÓ

ÔÌ¿‰ˆÓ: ·Ó¿ÏÔÁ· Ì ÙËÓ ÈÛÙÔÏÔÁÈ΋ ÔÌ¿‰· Î·È ·Ó¿-

ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ (4). ¶Ùˆ¯Ô› ÚÔÁÓˆ-

ÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Â›Ó·È Ë ˘„ËÏ‹ LDH ÔÚÔ‡, Ë ËÏÈ-

Λ· >15 ÂÙÒÓ, Ë ÌË ·¿ÓÙËÛË ÛÙË ıÂڷ›· ÚfiÊ·-

Û˘ Ì ΢ÎÏÔʈÛÊ·Ì›‰Ë, ‚ÈÓÎÚÈÛÙ›ÓË Î·È Ú‰ÓÈ˙Ô-

ÏfiÓË Î·È ÔÈ Â͈ÏÂÌÊ·‰ÂÓÈΤ˜ ÂÓÙÔ›ÛÂȘ (1,5). ∞Í›-

˙ÂÈ Ó· ·Ó·ÊÂÚı› fiÙÈ ÚfiÛÊ·Ù· ·Ó·ÁÓˆÚ›ÛÙËÎ·Ó Û˘-

ÁÎÂÎÚÈ̤ÓÔÈ ÔÏ˘ÌÔÚÊÈÛÌÔ› ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ

«·Ú¿ÁÔÓÙ· ı·Ó¿ÙˆÛ˘ fiÁΈӻ (TNF) Î·È ÏÂÌÊÔ-

ÙÔ͛Ӣ-· (LT-·) Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ÚfiÁÓˆÛË

ÙˆÓ ·ÛıÂÓÒÓ Ì µ§ (6).

ªÂ ‚¿ÛË ·˘Ù¿ Ù· ÎÚÈÙ‹ÚÈ·, ˘¿Ú¯Ô˘Ó ÙÚÂȘ ÔÌ¿-

‰Â˜ ıÂڷ¢ÙÈ΋˜ ·ÓÙÈÌÂÙÒÈÛ˘. ∏ ÚÒÙË ÔÌ¿‰·

·ÊÔÚ¿ Ù· §§, Ô˘ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È Ì ڈÙfiÎÔÏ-

Ï· Ù‡Ô˘ ÔÍ›·˜ ÏÂÌÊÔ‚Ï·ÛÙÈ΋˜ Ï¢¯·ÈÌ›·˜ ηÈ

‚·Û›˙ÔÓÙ·È ÛÂ Û˘Ó¯‹ ¤ÎıÂÛË Û ΢ÙÙ·ÚÔÛÙ·ÙÈο

Ê¿Ú̷η ÁÈ· Ì·ÎÚfi ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·. ∏ ‰Â‡ÙÂÚË

ÔÌ¿‰· ·ÊÔÚ¿ Ù· µ§ Î·È ¢§ªµ§, Ô˘ ·ÓÙÈÌÂÙˆ›˙Ô-

ÓÙ·È Ì ‚Ú·¯Â›·˜ ‰È¿ÚÎÂÈ·˜ ·ӷϷ̂·ÓfiÌÂÓÔ˘˜

·ÎÏÔ˘˜ ÂÓÙ·ÙÈ΋˜ Û˘Ó‰˘·Ṳ̂Ó˘ ¯ËÌÂÈÔıÂڷ›-

·˜. ∆¤ÏÔ˜, Ë ÙÚ›ÙË ÔÌ¿‰· ·ÊÔÚ¿ Ù· ∞§ª∫, ÁÈ· Ù·

ÔÔ›·, ·Ú¿ ÙÔ ÁÂÁÔÓfi˜ fiÙÈ ·Ú¯Èο ·ÓÙÈÌÂÙˆ›ÛÙË-

Î·Ó ÂÈÙ˘¯Ò˜ Ì ڈÙfiÎÔÏÏ· Î·È ·fi ÙȘ ‰‡Ô ÔÌ¿-

‰Â˜, ÙÂÏÈο ·Ó·Ù‡¯ıËÎ·Ó Í¯ˆÚÈÛÙ¿ ıÂڷ¢ÙÈο

ÚˆÙfiÎÔÏÏ· (4). ∂Âȉ‹ Ë ÚÒÙË ÔÌ¿‰· ¯ÚËÛÈÌÔ-

ÔÈ› ÚˆÙfiÎÔÏÏ· ıÂڷ›·˜ Ù‡Ô˘ Ï¢¯·ÈÌ›·˜, ı·

Á›ÓÂÈ ·Ó·ÊÔÚ¿ ÌfiÓÔ ÁÈ· ÙȘ ‰‡Ô ÙÂÏÂ˘Ù·›Â˜ ÔÌ¿‰Â˜.

∆Ô µ§ ·ÔÙÂÏ› ÙÔ 50% ÙˆÓ ª∏§ ÛÙ· ·È‰È¿ ηÈ

¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÒÚÈÌÔ µ-Ê·ÈÓfiÙ˘Ô, ÌÂ Tdt-,

CD10+, CD19+, CD20+, sIg+, CD22+ (4). ™Â ÔÛÔ-

ÛÙfi 25% ·ÓȯÓ‡ÂÙ·È ÁÔÓȉ›ˆÌ· ÙÔ˘ ÈÔ‡ Epstein-Barr

(EB). ¶ÈÔ Û˘¯Ó¿ ·ÓȯÓ‡ÂÙ·È Ë ¯ÚˆÌÔÛˆÌÈ΋ ÌÂÙ¿-

ıÂÛË t(8;14) Î·È Û·ÓÈfiÙÂÚ· Ë t(8;22) ‹ Ë t(2;8), ÌÂ

·Ó·‰È¿Ù·ÍË ÙÔ˘ c-myc. ∏ ÓfiÛÔ˜ Û˘Ó‹ıˆ˜ ÂÓÙÔ›˙Â-

Ù·È ÛÙËÓ ÎÔÈÏÈ¿, ÙÔ ∫¡™, ÙËÓ øƒ§ ÂÚÈÔ¯‹ Î·È ÙÔÓ

Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ. ∆Ô Û‡Ó‰ÚÔÌÔ Ï‡Û˘ fiÁÎÔ˘, Ô˘

·ÔÙÂÏÔ‡Û ÛËÌ·ÓÙÈÎfi Úfi‚ÏËÌ· ·Ï·ÈfiÙÂÚ· ηٿ

ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ¤¯ÂÈ ÂÏ·¯ÈÛÙÔÔÈËı› ÌÂ-

Ù¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÚfiÊ·Û˘ ÛÙË ıÂڷ›· (Ì ÎÔÚÙÈ-

ÎÔÛÙÂÚÔÂȉ‹ Î·È ¯·ÌËÏ‹ ‰fiÛË Î˘ÎÏÔʈÛÊ·Ì›‰Ë˜)

Î·È ÙË ¯Ú‹ÛË Ù˘ Ú·Û‚Ô˘ÚÈοÛ˘ Ì ÂÓÙ·ÙÈ΋ ÂÓ˘-

‰¿ÙˆÛË. ∏ ·ÎÙÈÓÔıÂڷ›· Î·È Ë ÂÎÙÂٷ̤ÓË ¯ÂÈ-

ÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ‰ÂÓ ¤¯Ô˘Ó ϤÔÓ ı¤ÛË ÛÙË ıÂÚ·-

¢ÙÈ΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÓfiÛÔ˘ (7,8).

∆· ¢§ªµ§ ·ÔÙÂÏÔ‡Ó ÙÔ 10-20% ÙˆÓ ª∏§ ÛÙ·

·È‰È¿ Î·È ÂÌÊ·Ó›˙ÔÓÙ·È ÈÔ Û˘¯Ó¿ ÙË 2Ë ‰ÂηÂÙ›·

Ù˘ ˙ˆ‹˜. ™˘Ó‹ıˆ˜ Â›Ó·È ÂÓÙÔÈṲ̂ӷ Î·È Û·ÓÈfiÙÂ-

Ú· ÂÌÊ·Ó›˙Ô˘Ó Â͈ÏÂÌÊ·‰ÂÓÈΤ˜ ÂÓÙÔ›ÛÂȘ ÛÙÔÓ

Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Î·È ÙÔ ∫¡™. À¿Ú¯ÂÈ ‚ÈÔÏÔÁÈ΋

‰È·ÊÔÚ¿ Ù˘ ÓfiÛÔ˘ Ì ·˘Ù‹Ó ÙˆÓ ÂÓËϛΈÓ. ¶ÈÔ Û˘-

ÁÎÂÎÚÈ̤ӷ, ·ÓȯÓ‡ÂÙ·È Û¿ÓÈ· Ë ¯ÚˆÌÔÛˆÌÈ΋ ÌÂ-

Ù¿ıÂÛË t(14;18) [IgH/bcl-2] Ì ·Ó·‰È¿Ù·ÍË ÙÔ˘ bcl-2

ÛÙËÓ ÂÚÈÔ¯‹ Ù˘ ‚·ÚÈ¿˜ ·Ï‡ÛÔ˘ ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈ-

ÓÒÓ, ÂÓÒ ÈÔ Û˘¯Ó¿ ·ÓȯÓ‡ÔÓÙ·È ·Ó·‰È·Ù¿ÍÂȘ ÙÔ˘

c-myc. ∏ ÛËÌ·Û›· ÙˆÓ ÛˆÌ·ÙÈÎÒÓ ˘ÂÚÌÂÙ·ÏÏ¿ÍÂ-

ˆÓ ÙˆÓ ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ, Ô˘ Â›Ó·È ÁÓˆÛÙ‹ ÛÙÔ˘˜

ÂÓ‹ÏÈΘ, ÛÙ· ·È‰È¿ ‰ÂÓ ¤¯ÂÈ ·ÔÛ·ÊËÓÈÛÙ›. ∏ Â-

ÓÙ·ÂÙ‹˜ ÂÈ‚›ˆÛË Â›Ó·È Ôχ ηϋ Î·È Î˘Ì·›ÓÂÙ·È

Û ÔÛÔÛÙfi 90%. ∂Í·›ÚÂÛË ·ÔÙÂÏ› ÙÔ ÚˆÙÔ·-

ı¤˜ ϤÌʈ̷ ·fi µ-·ÙÙ·Ú· ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘,

Ô˘ ¤¯ÂÈ ¯ÂÈÚfiÙÂÚË ÚfiÁÓˆÛË (9).

√È ˘¿Ú¯Ô˘Û˜ ·ÚȘ ıÂڷ¢ÙÈΤ˜ ÂÈÏÔÁ¤˜ ÁÈ·

Ù· B§ Î·È ¢§ªµ§ Â›Ó·È ‚Ú·¯Â›·˜ ‰È¿ÚÎÂÈ·˜ ÂÓÙ·ÙÈο

¯ËÌÂÈÔıÂڷ¢ÙÈο ÚˆÙfiÎÔÏÏ· (8). ∆· ÚˆÙfiÎÔÏ-

Ï· ¯ÚËÛÈÌÔÔÈÔ‡Ó ¤Ó· Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂڷ¢ÙÈ-

ÎÒÓ Ê·ÚÌ¿ÎˆÓ Ì ‰È·ÊÔÚÂÙÈ΋ ΢ÙÙ·ÚÔÙÔÍÈ΋ ‰Ú¿-

ÛË Î·È ‰È·ÊÔÚÂÙÈ΋ ÙÔÍÈÎfiÙËÙ· Î·È ¤¯Ô˘Ó ˆ˜ ÊÈÏÔÛÔ-

Ê›· ÙË Û˘Ó¯‹ ‹ ÎÏ·ÛÌ·ÙÔÔÈË̤ÓË ¤ÎıÂÛË ÙˆÓ

˘„ËÏ¿ ÔÏÏ·Ï·ÛÈ·˙fiÌÂÓˆÓ Î·ÎÔËıÒÓ Î˘ÙÙ¿ÚˆÓ

Û ΢ÙÙ·ÚÔÙÔÍÈο Ê¿Ú̷η. ¶ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Â·Ó·-

Ï·Ì‚·ÓfiÌÂÓ˜ Û˘Ó‰ڛ˜ ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ, ‚ÈÓÎÚÈ-

ÛÙ›Ó˘, ΢ÎÏÔʈÛÊ·Ì›‰Ë˜ ‹ ÈʈÛÊ·Ì›‰Ë˜, ˘„ËÏ‹˜

‰fiÛ˘ ÌÂıÔÙÚÂÍ¿Ù˘, Î˘Ù·Ú·‚›Ó˘, ·‰ÚÈ·Ì˘Î›Ó˘ ηÈ

ÂÙÔÔÛ›‰Ë˜, Û˘Ó‹ıˆ˜ Û ·ÎÏÔ˘˜ ÙˆÓ 3 ‚‰ÔÌ¿‰ˆÓ,

Ì Ôχ ηϿ ·ÔÙÂϤÛÌ·Ù· (ÂÈ‚›ˆÛË ÂχıÂÚË

Û˘Ì‚·Ì¿ÙˆÓ ÛÙ· 3-5 ¤ÙË 77-91%). ∂ÈϤÔÓ, ÏfiÁˆ

Ù˘ ÂÍ·ÈÚÂÙÈ΋˜ Ù¿Û˘ ÙÔ˘ µ§ ÁÈ· ‰È‹ıËÛË ÙÔ˘ ∫¡™,

Á›ÓÔÓÙ·È ÂÓ‰ÔÚÚ·¯È·›Â˜ ÂÁ¯‡ÛÂȘ Ê·ÚÌ¿ÎˆÓ (4,10).

¢Â‰Ô̤ÓÔ˘ fiÙÈ ÔÈ ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ¤¯Ô˘Ó

ÂÍ·ÈÚÂÙÈο ηϋ ÚfiÁÓˆÛË Î·È ÂÈ‚›ˆÛË, Û‡Á¯ÚÔÓ˜

ÔÏ˘ÎÂÓÙÚÈΤ˜ ÌÂϤÙ˜ ÚÔÛ¿ıËÛ·Ó Ó· ÂÏ·ÙÙÒ-

ÛÔ˘Ó ÙË ‰fiÛË Î·È ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜. ŒÙÛÈ,

¤ÁÈÓ ÚÔÛ¿ıÂÈ· Ó· ÂÏ·ÙÙˆı› Ë ‰fiÛË Ù˘ ËÌÂÈÔıÂ-

ڷ›·˜ Û ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, ÌÂ

·ÚÓËÙÈÎfi fï˜ ·ÔÙ¤ÏÂÛÌ· ÛÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË,

ÂÓÒ Û ÂӉȷ̤ÛÔ˘ ÎÈÓ‰‡ÓÔ˘ ·ÛıÂÓ›˜ ÂÏ·ÙÙÒıËΠÌÂ

ÂÈÙ˘¯›· Ë ‰fiÛË Ù˘ ¯ËÌÂÈÔıÂڷ›·˜, ¯ˆÚ›˜ ·ÚÓËÙÈ-

ο ·ÔÙÂϤÛÌ·Ù· ÛÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË (11-13).

Ÿˆ˜ ·Ó·Ê¤ÚıËÎÂ, ÔÈ ·ÛıÂÓ›˜ Ì µ§ ¤¯Ô˘Ó ·˘-

ÍË̤ÓË Èı·ÓfiÙËÙ· ÓfiÛÔ˘ ÛÙÔ ∫¡™. ∏ Û˘ÛÙËÌ·ÙÈ΋

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·83

Page 12: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

84 µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹

Paediatriki 2009;72:1-9

¯ÔÚ‹ÁËÛË ˘„ËÏ‹˜ ‰fiÛ˘ ÌÂıÔÙÚÂÍ¿Ù˘ Î·È ÙÚÈÏ‹˜

ÂÓ‰ÔÚÚ·¯È·›·˜ ıÂڷ›·˜ ÂÏ·ÙÙÒÓÂÈ ÙËÓ Èı·ÓfiÙË-

Ù· ˘ÔÙÚÔ‹˜ ÛÙÔ ∫¡™ Û ·ÛıÂÓ›˜ Ô˘ ‹Ù·Ó ·ÚÓË-

ÙÈÎÔ› ÁÈ· ÓfiÛÔ ÛÙÔ ∫¡™ ηٿ ÙË ‰È¿ÁÓˆÛË. ∞ÓÙ›ıÂÙ·,

Ë ÚfiÁÓˆÛË Â›Ó·È ¯ÂÈÚfiÙÂÚË ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ô˘

¤¯Ô˘Ó ÚÔÛ‚ÔÏ‹ ÙÔ˘ ∫¡™ ηٿ ÙË ‰È¿ÁÓˆÛË (14).

¢ÂÓ ˘¿Ú¯Ô˘Ó ·ÎfiÌË Ù˘¯·ÈÔÔÈË̤Ó˜ ÌÂϤÙ˜ Ô˘

Ó· Û˘ÁÎÚ›ÓÔ˘Ó ÙÔÓ ÚfiÏÔ Ù˘ ·ÎÙÈÓÔıÂڷ›·˜ ÛÙÔ˘˜

·ÛıÂÓ›˜ Ì ÚÔÛ‚ÔÏ‹ ∫¡™ (4).

∞Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÙÂÏÂ˘Ù·›· ¤¯ÂÈ ÂÓۈ̷-

Ùˆı› Ë ÌÂϤÙË ÙÔ˘ ÁÔÓȉȷÎÔ‡ ÚÔÊ›Ï ÙˆÓ ÏÂÌʈ-

Ì¿ÙˆÓ ÛÙ· ·È‰È¿ Ì ¢§ªµ§ Î·È B§ (8). Ÿˆ˜ ›-

Ó·È ÁÓˆÛÙfi, Ù· ¢§ªµ§ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÌÂÁ¿ÏË ÎÏÈ-

ÓÈ΋ Î·È ‚ÈÔÏÔÁÈ΋ ÂÙÂÚÔÁ¤ÓÂÈ· ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, Ë

ÔÔ›· ·ÓÙ·Ó·ÎÏ¿Ù·È Û ‰È·ÊÔÚÂÙÈ΋ ÚfiÁÓˆÛË Î·È

·ÓÙ·fiÎÚÈÛË ÛÙË ıÂڷ›·. √È Alizadeh Î·È Û˘Ó.,

¯ÚËÛÈÌÔÔÈÒÓÙ·˜ ÙËÓ Ù¯ÓÈ΋ ÁÔÓȉȷ΋˜ ·Ó¿Ï˘Û˘

Ì ÌÈÎÚÔÛ˘ÛÙÔȯ›Â˜ DNA, ηٿÊÂÚ·Ó Ó· ·Ó·ÁÓˆÚ›-

ÛÔ˘Ó ‰‡Ô ‰È·ÊÔÚÂÙÈΤ˜ ˘Ô-ÔÌ¿‰Â˜ Ì ÚÔÁÓˆÛÙÈ-

΋ ÛËÌ·Û›·, ÙËÓ «Ù‡Ô˘ ‚Ï·ÛÙÈÎÔ‡ ΤÓÙÚÔ˘ µ-ÏÂÌ-

ÊÔ΢ÙÙ¿ÚˆÓ» Î·È ÙËÓ «ÂÓÂÚÁÔÔÈË̤ÓË», ÂÓÒ ·ÚÁfi-

ÙÂÚ· ÔÈ Rosenwald Î·È Û˘Ó. ÂȂ‚·›ˆÛ·Ó ÙËÓ ‡·Ú-

ÍË ÙˆÓ ‰‡Ô ˘Ô-ÔÌ¿‰ˆÓ Î·È ·Ó·Î¿Ï˘„·Ó Î·È Ì›·

ÙÚ›ÙË ÔÌ¿‰· «Ù‡Ô˘ 3» (15,16). ¶·ÚfiÌÔȘ ÌÂϤÙ˜

‰ÂÓ ¤¯Ô˘Ó ·ÎfiÌË ‰ËÌÔÛÈ¢ı› ÁÈ· ·È‰È¿ ÌÂ

¢§ªµ§. ¶·ÚfiÏ· ·˘Ù¿, ÚfiÛÊ·ÙË ÌÂϤÙË, ¯ÚËÛÈ-

ÌÔÔÈÒÓÙ·˜ ·ÓÔÛÔ˚ÛÙÔ¯ËÌ›· Î·È «Â› ÙfiÔ˘»

˘‚ÚȉÈÛÌfi ÊıÔÚÈÛÌÔ‡ (FISH), η٤‰ÂÈÍ fiÙÈ ÛÙ·

·È‰È¿ Ù· ¢§ªµ§ Â›Ó·È ÈÔ Û˘¯Ó¿ «Ù‡Ô˘ ‚Ï·ÛÙÈ-

ÎÔ‡ ΤÓÙÚÔ˘ µ-ÏÂÌÊÔ΢ÙÙ¿ÚˆÓ» Î·È ÁÈ’ ·˘Ùfi ¤¯Ô˘Ó

Î·È Î·Ï‡ÙÂÚË ¤Î‚·ÛË (17).

™Â ÂÚÈÙÒÛÂȘ ˘ÔÙÚÔ‹˜ ÙÔ˘ µ§, Ë ÚfiÁÓˆÛË

·Ú·Ì¤ÓÂÈ ‰˘ÛÙ˘¯Ò˜ ÂÍ·ÈÚÂÙÈο ‰˘ÛÌÂÓ‹˜. H ÌÂÙ·-

ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·ÔÙÂÏ› ÁÈ· ÔÏÏÔ‡˜

ıÂڷ›· ÂÎÏÔÁ‹˜ Ô˘ ›Ûˆ˜ ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÂÈ‚›ˆ-

ÛË, ÂÓÒ ·Ú¿ÏÏËÏ· Á›ÓÔÓÙ·È Î·È ÂÈÚ·Ì·ÙÈΤ˜ ÛÙÔ-

¯Â˘Ì¤Ó˜ ıÂڷ›˜ (18). ¶Ôχ ÛËÌ·ÓÙÈÎfi˜ ÚÔ-

ÁÓˆÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Û ÂÚ›ÙˆÛË ˘ÔÙÚÔ‹˜

Â›Ó·È Ë Â›Ù¢ÍË ‰Â‡ÙÂÚ˘ ‡ÊÂÛ˘ Î·È Ë ·¿ÓÙËÛË

ÛÙË ıÂڷ›· ‰È¿ÛˆÛ˘ (19,20).

T· ∞§ª∫ ·ÔÙÂÏÔ‡Ó ÙÔ 10% ÙˆÓ ª∏§ ÛÙ· ·È-

‰È¿. ™˘Ó‹ıˆ˜ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ∆-Ê·ÈÓfiÙ˘Ô ÌÂ

ÁÂÓÈÎÂ˘Ì¤ÓË ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· Î·È µ-Û˘ÌÙÒÌ·Ù·,

ÂÓÒ ‰ÂÓ Â›Ó·È Û¿ÓȘ Î·È ÔÈ Â͈ÏÂÌÊ·‰ÂÓÈΤ˜ ÂÓÙÔ-

›ÛÂȘ ÛÙÔ ‰¤ÚÌ·, ÙÔ˘˜ Ó‡ÌÔÓ˜, ÙÔ ‹·Ú, Ù· ÔÛÙ¿

Î·È Ù· ̷Ϸο ÌfiÚÈ·. ™Â ÔÛÔÛÙfi 90%, ·ÓȯÓ‡ÂÙ·È Ô

·ÓÔÛÔÊ·ÈÓÔÙ˘ÈÎfi˜ ‰Â›ÎÙ˘ CD30+ Î·È Ë ¯ÚˆÌÔÛˆ-

ÌÈ΋ ÌÂÙ¿ıÂÛË t(2;5)(p23;q35) Ô˘ Ô‰ËÁ› ÛÙÔ Úˆ-

ÙÂ˚ÓÈÎfi Û‡ÌÏÂÁÌ· NPM/ALK Ì ‰Ú·ÛÙËÚÈfiÙËÙ· Ù˘-

ÚÔÛÈÓÈ΋˜ ÎÈÓ¿Û˘ Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ¢ÓÔ˚Îfi-

ÙÂÚË ÚfiÁÓˆÛË, Û ·ÓÙ›ıÂÛË Ì ÙÔ˘˜ ÂÓ‹ÏÈΘ. ∆Ô

Û‡ÌÏÂÁÌ· ·˘Ùfi Ô‰ËÁ› Û ÂÏ·Ùو̤ÓË ·fiÙˆÛË,

̤ۈ ÙˆÓ ÌÔÓÔ·ÙÈÒÓ PI3K Î·È PKB/AKT, ηıÒ˜ ηÈ

Û ÂÓ›Û¯˘ÛË ÙÔ˘ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡, ̤ۈ ÙˆÓ ÌÔÓÔ-

·ÙÈÒÓ JAK/STAT Î·È ÙÔ˘ ÔÁÎÔÁÔÓȉ›Ô˘ myc (3,7).

¢È¿ÊÔÚ· ¯ËÌÂÈÔıÂڷ¢ÙÈο ÚˆÙfiÎÔÏÏ· ¤¯Ô˘Ó

¯ÚËÛÈÌÔÔÈËı› Ì Ôχ ηϿ ·ÔÙÂϤÛÌ·Ù· (ÂÈ-

‚›ˆÛË ÂχıÂÚË ÓfiÛÔ˘ 65-75%) (21-23). ∂›Ó·È ·Ú·-

ÎÙËÚÈÛÙÈÎfi fiÙÈ Ù· ıÂڷ¢ÙÈο ÚˆÙfiÎÔÏÏ· ›ӷÈ

ÂÙÂÚÔÁÂÓ‹ Î·È ÂÔ̤ӈ˜ Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÍ·¯ıÔ‡Ó

Û˘ÌÂÚ¿ÛÌ·Ù· Û¯ÂÙÈο Ì ÙÔÓ ÚfiÏÔ Î¿ı ʷÚÌ·-

΢ÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· ͯˆÚÈÛÙ¿. ¶Èı·ÓÒ˜ Ë ‰ÔÍÔ-

ÚÔ˘ÌÈΛÓË, Ë ‚ÈÓÎÚÈÛÙ›ÓË Î·È Ù· ÛÙÂÚÔÂȉ‹ Ó· ·Ô-

ÙÂÏÔ‡Ó ÛËÌ·ÓÙÈο Ê¿Ú̷η ÛÙË ıÂڷ›· (4). ∂›-

Û˘, Û ·ÓÙ›ıÂÛË Ì ٷ ¿ÏÏ· ª∏§, Ë ‰È·ÛÙڈ̿و-

ÛË Ù˘ ıÂڷ›·˜ ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘

Î·È ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ ‰ÂÓ Ê·›ÓÂÙ·È Ó· Â›Ó·È ÛËÌ·-

ÓÙÈ΋ ÛÙÔ ∞§ª∫. ∂Í·›ÚÂÛË ›Ûˆ˜ ·ÔÙÂÏÔ‡Ó ÔÈ

·ÛıÂÓ›˜ Ì ϋڈ˜ ¯ÂÈÚÔ˘ÚÁÈο ÂÍ·ÈÚ¤ÛÈÌÔ fiÁÎÔ,

Ô˘ Ê·›ÓÂÙ·È Ó· ¯ÚÂÈ¿˙ÔÓÙ·È ÏÈÁfiÙÂÚË ıÂڷ›·, ηÈ

Ë ÂÓÙ·ÙÈÎÔÔ›ËÛË Ù˘ ıÂڷ›·˜ Û ·ÚÔ˘Û›· ·-

Ú·ÁfiÓÙˆÓ ÎÈÓ‰‡ÓÔ˘ (.¯. ÚÔÛ‚ÔÏ‹ ‰¤ÚÌ·ÙÔ˜, ÌÂ-

ÛÔıˆÚ·Î›Ô˘, ÛÏ¿¯ÓˆÓ) Ô˘ ‚Ú›ÛÎÂÙ·È ˘fi ÌÂϤÙË.

∆¤ÏÔ˜, ·Í›˙ÂÈ Ó· ÛËÌÂȈı› fiÙÈ ÔÈ Û‡Á¯ÚÔÓ˜ ÌÂϤÙ˜

ÂÓۈ̷ÙÒÓÔ˘Ó ‰È¿ÊÔÚ˜ ‚ÈÔÏÔÁÈΤ˜ ·Ú·Ì¤ÙÚÔ˘˜,

fiˆ˜ ·ÓÔÛÔÊ·ÈÓfiÙ˘Ô, ›‰· ΢ÙÔÎÈÓÒÓ, ÚÔÛ-

‰ÈÔÚÈÛÌfi ÙÔ˘ CD30 ÛÙÔ Ï¿ÛÌ·, ÌÂϤÙË ˘ÔÏÂÈfi-

ÌÂÓ˘ ÓfiÛÔ˘ Î·È ÁÔÓȉȷÎÒÓ ÌÈÎÚÔÛ˘ÛÙÔȯÈÒÓ DNA,

Ì ÛÙfi¯Ô ÙËÓ ·ÍÈÔÏfiÁËÛ‹ ÙÔ˘˜ ˆ˜ ‰È·ÁÓˆÛÙÈÎÔ‡˜ ‹

ÚÔÁÓˆÛÙÈÎÔ‡˜ ‰Â›ÎÙ˜ (24,25). ¶Úfi‰ÚÔÌ· ·ÔÙÂ-

ϤÛÌ·Ù· ·Ó·Ê¤ÚÔ˘Ó fiÙÈ ÙÔ ÁÔÓ›‰ÈÔ clusterin ÂÎÊÚ¿-

˙ÂÙ·È ÂȉÈο Û ∞§ª∫, ÂÓÒ Â›Ó·È ·ÚÓËÙÈÎfi Û ¿ÏÏ·

ÏÂÌÊÒÌ·Ù· Î·È Èı·ÓÒ˜ Ó· ·ÔÙÂϤÛÂÈ ‰È·ÁÓˆÛÙÈÎfi

ÎÚÈÙ‹ÚÈÔ ÌÂÏÏÔÓÙÈο (26).

√È ÂÚÈÛÛfiÙÂÚ˜ ˘ÔÙÚÔ¤˜ Û˘Ì‚·›ÓÔ˘Ó Û¯ÂÙÈ-

ο ÓˆÚ›˜, ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÒÙˆÓ 15 ÌËÓÒÓ.

™Â ÂÚ›ÙˆÛË ˘ÔÙÚÔ‹˜, Ë ÚfiÁÓˆÛË ÙˆÓ ∞§ª∫

Â›Ó·È ÂÍ·ÈÚÂÙÈο Ùˆ¯‹. ™Â ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ Á›-

ÓÂÙ·È Î·È ‰È¿ÛˆÛË Ì ·˘ÙfiÏÔÁË ‹ ·ÏÏÔÁÂÓ‹ ÌÂÙ·Ìfi-

Û¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (4,27).

µ. Hodgkin §ÂÌÊÒÌ·Ù· (∏§)

H ÓfiÛÔ˜ ÙÔ˘ Hodgkin ÂÚÈÁÚ¿ÊËÎÂ ÁÈ· ÚÒÙË

ÊÔÚ¿ ·fi ÙÔÓ Thomas Hodgkin (1832). ∆Ô ¯·Ú·ÎÙË-

ÚÈÛÙÈÎfi ·ıÔÁÓˆÌÔÓÈÎfi ·ÙÙ·ÚÔ Â›Ó·È ÙÔ Reed-

Sternberg (R-S), Ô˘ ¤¯ÂÈ µ-΢ÙÙ·ÚÈ΋ ÚԤϢÛË.

™Â ÔÛÔÛÙfi 30% ·ÓȯÓ‡ÂÙ·È Ô Èfi˜ ∂µ. ∆fiÛÔ ÛÙÔ EB

ıÂÙÈÎfi fiÛÔ Î·È ÛÙÔ ·ÚÓËÙÈÎfi ∏§, ·Ú·ÙËÚÂ›Ù·È ÂÓÂÚ-

ÁÔÔ›ËÛË ÙˆÓ ÌÂÙ·ÁÚ·ÊÈÎÒÓ ·Ú·ÁfiÓÙˆÓ STAT6

Î·È NfkB, Ô˘ Ô‰ËÁÔ‡Ó Û ÂÓ›Û¯˘ÛË ÙÔ˘ ÔÏÏ·Ï·-

ÛÈ·ÛÌÔ‡ Î·È Ù˘ ÂÈ‚›ˆÛ˘ ÙˆÓ ÓÂÔÏ·ÛÌ·ÙÈÎÒÓ

΢ÙÙ¿ÚˆÓ R-S. ™ÙËÓ ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ÌÂÙ·ÁÚ·ÊÈ-

ÎÒÓ ·˘ÙÒÓ ·Ú·ÁfiÓÙˆÓ Û˘ÌÌÂÙ¤¯Ô˘Ó ‰È¿ÊÔÚ˜

΢ÙÙ·ÚÔΛÓ˜, fiˆ˜ Ë IL-13 Î·È Ô TNF-·, ÂÓÒ ˘¿Ú-

¯ÂÈ ·ÏÏËÏ›‰Ú·ÛË Î·È Ì ˈÛÈÓfiÊÈÏ· Î·È ÏÂÌÊÔ-

·ÙÙ·Ú·, ̤ۈ ÙˆÓ ˘Ô‰Ô¯¤ˆÓ CD30L Î·È CD40L.

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·84

Page 13: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

85§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿

¶·È‰È·ÙÚÈ΋ 2009;72:1-9

∏ ÚˆÙ½ÓË LMP1ÙÔ˘ ÈÔ‡ EB ÂÓÂÚÁÔÔÈ› ·Â˘ı›·˜

ÙÔÓ ÌÂÙ·ÁÚ·ÊÈÎfi ·Ú¿ÁÔÓÙ· NfkB (28-30).

∏ ÈÛÙÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË ÙˆÓ ∏§ Û‡Ìʈӷ ÌÂ

ÙËÓ International Lymphoma Study Group, REAL ηÈ

ÙÔÓ WHO (31) Û˘ÓÔ„›˙ÂÙ·È ÛÙÔÓ ¶›Ó·Î· 1.

∆Ô ∏§ ÂÌÊ·Ó›˙ÂÙ·È ÈÔ Û˘¯Ó¿ Û ËÏÈ˘ ÌÂÁ·Ï‡-

ÙÂÚ˜ ÙˆÓ 10 ÂÙÒÓ. √È ˘fiÙ˘ÔÈ ÏÂÌÊÔÂÈÎÚ·ÙÔ‡-

ÓÙÔ˜ Î·È ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ ¤¯Ô˘Ó ÙËÓ Î·Ï‡ÙÂ-

ÚË ÚfiÁÓˆÛË, ÂÓÒ Ù· ÏÂÌÊÔÂÓÈο ¤¯Ô˘Ó ÙË ¯ÂÈÚfi-

ÙÂÚË ÚfiÁÓˆÛË.

∏ ‰È¿ÁÓˆÛË Ù›ıÂÙ·È Ì ‚ÈÔ„›· Ù˘ Ì¿˙·˜. ∞ÂÈÎÔ-

ÓÈÛÙÈΤ˜ ÂÍÂÙ¿ÛÂȘ Ô˘ Û˘ÓÂÈÎÔ˘ÚÔ‡Ó Â›Ó·È Ë ·ÎÙÈ-

ÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜, ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ·, Ë ·ÍÔÓÈ΋

(CT) Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· (MRI), ÙÔ ÛÈÓıË-

ÚÔÁÚ¿ÊËÌ· Ì Á¿ÏÏÈÔ Î·È Ë ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›·

(FDP-PET). H ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· Ù›ÓÂÈ Ó·

·ÓÙÈηٷÛÙ‹ÛÂÈ ÙÔ ÛÈÓıËÚÔÁÚ¿ÊËÌ· Ì Á¿ÏÏÈÔ, η-

ıÒ˜ ‰È·ı¤ÙÂÈ ˘„ËÏfiÙÂÚË ‰È·ÎÚÈÙÈ΋ ÈηÓfiÙËÙ· ηÈ

¢·ÈÛıËÛ›·, ¯·ÌËÏfiÙÂÚË ‰fiÛË ·ÎÙÈÓÔ‚ÔÏ›·˜ ÁÈ· ÙÔÓ

·ÛıÂÓ‹, ÌÈÎÚfiÙÂÚÔ ¯ÚfiÓÔ ÂͤٷÛ˘ Î·È ·ÔÙÂÏÂÛÌ·-

ÙÈÎfiÙÂÚË ·Ó›¯Ó¢ÛË Ù˘ ÛÏËÓÈ΋˜ ÚÔÛ‚ÔÏ‹˜. ∏

ÔÛÈÙÚÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ÌÔÚ› Ó· ·ÏÏ¿ÍÂÈ ÙÔ ÛÙ¿-

‰ÈÔ Ù˘ ÓfiÛÔ˘ Û ÔÛÔÛÙfi 15-20% ÙˆÓ ·ÛıÂÓÒÓ. ¶·-

ÚfiÏ· ·˘Ù¿, ¤¯ÂÈ ¯·ÌËÏ‹ ¢·ÈÛıËÛ›· ÛÙËÓ ·Ó›¯Ó¢ÛË

Ù˘ Ì˘ÂÏÈ΋˜ Û˘ÌÌÂÙÔ¯‹˜ Î·È ‰ÂÓ ÌÔÚ› Ó· ·ÓÙÈη-

Ù·ÛÙ‹ÛÂÈ ÙË ‚ÈÔ„›· Ì˘ÂÏÔ‡. À¿Ú¯Ô˘Ó, fï˜, ÂÚÈ-

ÙÒÛÂȘ Ì ıÂÙÈÎfi PET Î·È ·ÚÓËÙÈ΋ ‚ÈÔ„›· Ì˘ÂÏÔ‡.

∆Ô PET ‰ÂÓ ÌÔÚ› Ó· ·ÓÙÈηٷÛÙ‹ÛÂÈ ÙË CT. √ Û˘Ó-

‰˘·ÛÌfi˜ ƒ∂∆/CT ¤¯ÂÈ ÌÂÁ·Ï‡ÙÂÚË ‰È·ÁÓˆÛÙÈ΋ ·Í›·.

¢ÂÓ ¯ÚÂÈ¿˙ÂÙ·È ÁÈ· ÙË ÛÙ·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘ ÚÈÓ

ÙËÓ ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ηıÒ˜ ¤¯ÂÈ Ù¿ÛË ÁÈ·

˘ÂÚ-ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ·ÛıÂÓÒÓ (28,32-37).

∏ ÛÙ·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘ ηٿ Ann-Arbor Á›ÓÂ-

Ù·È Û ٤ÛÛÂÚ· ÛÙ¿‰È· I-IV, ·Ó¿ÏÔÁ· Ì ÙËÓ ¤ÎÙ·ÛË

Ù˘ ÓfiÛÔ˘, Î·È Î·ÙËÁÔÚÈÔÔÈÂ›Ù·È Û A Î·È µ ·Ó¿ÏÔÁ·

Ì ÙËÓ ·ÚÔ˘Û›· ‹ fi¯È µ-Û˘ÌÙˆÌ¿ÙˆÓ (ηÎÔ˘¯›·,

˘ÚÂÙfi˜), Û à Û ÂÚÈÙÒÛÂȘ ÌÂÁ¿Ï˘ Ì¿˙·˜ (>10

cm), Û ∂ Û ·ÚÔ˘Û›· Â͈ÏÂÌÊ·‰ÂÓÈÎÒÓ Ì·˙ÒÓ Î·È

Û S Û ÂÚÈÙÒÛÂȘ ÛÏËÓÈ΋˜ ÚÔÛ‚ÔÏ‹˜ (38).

∏ ÚfiÁÓˆÛË Ù˘ ÓfiÛÔ˘ ¤¯ÂÈ ‚ÂÏÙȈı› ÛËÌ·ÓÙÈ-

ο Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ· Ì ÙË ¯Ú‹ÛË Û˘Ó‰˘·Ṳ̂Ó˘

¯ËÌÂÈÔıÂڷ›·˜ Î·È ·ÎÙÈÓÔıÂڷ›·˜ Î·È ÙËÓ ÚÔ-

Û·ÚÌÔÁ‹ Ù˘ ıÂڷ›·˜ ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘

ÓfiÛÔ˘. ∆· Û˘Ó‹ıË ¯ËÌÂÈÔıÂڷ¢ÙÈο Û¯‹Ì·Ù· Ô˘

¯ÔÚËÁÔ‡ÓÙ·È Û ·È‰È¿ Î·È ÂÓ‹ÏÈΘ Ì ϤÌʈ̷

Hodgkin Â›Ó·È Û˘Ó‰˘·ÛÌfi˜ Ê·ÚÌ¿ÎˆÓ Ô˘ ÂÚȤ¯ÂÈ

·Óıڷ΢ÎϛӘ Î·È ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜. ªÂ-

ÚÈο ·Ú·‰Â›ÁÌ·Ù· ›ӷÈ: ABVD (‰ÔÍÔÚÔ˘ÌÈΛÓË,

ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÌÏ·ÛÙ›ÓË Óٷηڂ·˙›ÓË),

OPPA±COPP Û ı‹Ï· (‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ˙fiÓË,

ÚÔηڂ·˙›ÓË, ‰ÔÍÔÚÔ˘ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë,

Ú‰ÓÈ˙fiÓË, ÚÔηڂ·˙›ÓË), OEPA±COPP Û ¿ÚÚÂ-

Ó˜ (‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ˙fiÓË, ÂÙÔÔÛ›‰Ë, ‰ÔÍÔÚÔ˘-

ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë, Ú‰ÓÈ˙fiÓË, ÚÔηڂ·-

˙›ÓË), BEACOPP (ÌÏÂÔÌ˘Î›ÓË, ÂÙÔÔÛ›‰Ë, ‰ÔÍÔ-

ÚÔ˘ÌÈΛÓË, ΢ÎÏÔʈÛÊ·Ì›‰Ë, ‚ÈÓÎÚÈÛÙ›ÓË, Ú‰ÓÈ-

˙fiÓË, ÚÔηڂ·˙›ÓË), DBVE-PC (‰ÔÍÔÚÔ˘ÌÈΛÓË,

ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÎÚÈÛÙ›ÓË, ÂÙÔÔÛ›‰Ë, Ú‰ÓÈ˙fiÓË,

΢ÎÏÔʈÛÊ·Ì›‰Ë), MOPP/ABV (̯ψڷÈı·Ì›ÓË,

‚ÈÓÎÚÈÛÙ›ÓË, ÚÔηڂ·˙›ÓË, Ú‰ÓÈ˙fiÓË, ‰ÔÍÔÚÔ˘-

ÌÈΛÓË, ÌÏÂÔÌ˘Î›ÓË, ‚ÈÓÌÏ·ÛÙ›ÓË) (39). ∏ ıÂÚ·-

›· Á›ÓÂÙ·È ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ ηÈ

ÌÔÚ› Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ Û˘Ó‰˘·ÛÌfi ·ÎÙÈÓÔıÂڷ›·˜

ÚÔۂ‚ÏËÌ¤ÓˆÓ Â‰›ˆÓ, Ë ‰Â ÂÈ‚›ˆÛË Â›Ó·È Ôχ

˘„ËÏ‹ Î·È ÔÈΛÏÏÂÈ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡-

ÓÔ˘ (¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘: ÔÏÈ΋ ÂÈ‚›ˆÛË 96-100%,

ÂӉȷ̤ÛÔ˘ Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘: 85-100%).

¶·ÚfiÏÔ Ô˘ Ë ÂÈ‚›ˆÛË Â›Ó·È Ôχ ηϋ, Ë ÙÔÍÈ-

ÎfiÙËÙ· ÙfiÛÔ ·fi ÙË ¯ËÌÂÈÔıÂڷ›· fiÛÔ Î·È ·fi

ÙËÓ ·ÎÙÈÓÔıÂڷ›· ·Ú·Ì¤ÓÔ˘Ó ÛÔ‚·Ú¤˜. ¶ÈÔ Û˘-

ÁÎÂÎÚÈ̤ӷ, ÔÈ ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜ Ô‰ËÁÔ‡Ó

Û ÛÙ›ڈÛË Î·È ˘ÔÁÔÓÈÌfiÙËÙ·, ÔÈ ·Ï΢ÏÈÔ‡ÓÙ˜

·Ú¿ÁÔÓÙ˜ Î·È Ë ÂÙÔÔÛ›‰Ë ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È ÁÈ·

ÂÌÊ¿ÓÈÛË ‰Â˘ÙÂÚÔ·ıÔ‡˜ Ï¢¯·ÈÌ›·˜, Ë ÌÏÂÔÌ˘Î›-

ÓË Ô‰ËÁ› Û ‰Â˘ÙÂÚÔ·ı‹ ›ÓˆÛË Î·È ÔÈ ·Óıڷ΢ÎÏ›-

Ó˜ Û ηډÈÔÌ˘Ô¿ıÂÈ·. ∏ ·ÎÙÈÓÔıÂڷ›· ¢ı‡-

ÓÂÙ·È ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Û˘Ì·ÁÒÓ fiÁΈÓ, Ï¢¯·È-

ÌÈÒÓ Î·È Ì˘ÂÏÔ‰˘ÛÏ·ÛÙÈÎÒÓ Û˘Ó‰ÚfïÓ, ·ıËÚÔ-

ÛÎÏËÚ˘ÓÙÈ΋˜ ηډȷ΋˜ ÓfiÛÔ˘, ‰˘ÛÏÂÈÙÔ˘ÚÁÈÒÓ

ÙˆÓ ‚·Ï‚›‰ˆÓ, Ó¢ÌÔÓÈ΋˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜, ÛÙ›-

ÚˆÛ˘, ˘ÔÁÔÓÈÌfiÙËÙ·˜ Î·È ˘Ôı˘ÚÂÔÂȉÈÛÌÔ‡/ Ca

ı˘ÚÂÔÂȉԇ˜ (39).

°È· ÙÔ ÏfiÁÔ ·˘Ùfi, Ù· Û‡Á¯ÚÔÓ· ıÂڷ¢ÙÈο

ÚˆÙfiÎÔÏÏ· ÚÔÛ·ıÔ‡Ó Ó· ÚÔÛ‰ÈÔÚ›ÛÔ˘Ó ÙË

‚¤ÏÙÈÛÙË ‰fiÛË ¯ËÌÂÈÔıÂڷ›·˜ ηÈ/‹ ·ÎÙÈÓÔıÂÚ·-

›·˜ ‹ Ó· ·ÔʇÁÔ˘Ó ÙËÓ ·ÎÙÈÓÔıÂڷ›· ÚÔÎÂÈ-

̤ÓÔ˘ Ó· ÂÏ·¯ÈÛÙÔÔÈ‹ÛÔ˘Ó ÙËÓ ÙÔÍÈÎfiÙËÙ·. ŒÙÛÈ,

ÔÈ Nachman Î·È Û˘Ó. ¯ÔÚ‹ÁËÛ·Ó ¯ËÌÂÈÔıÂڷ›·

·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ Î·È Â¤ÏÂÍ·Ó Ù˘-

¯·›· ÙÔ˘˜ ·ÛıÂÓ›˜ ÁÈ· Ó· Ï¿‚Ô˘Ó ‹ fi¯È ·ÎÙÈÓÔıÂÚ·-

›·, ·Ó¿ÏÔÁ· Ì ÙËÓ ·¿ÓÙËÛË ÛÙË ¯ËÌÂÈÔıÂÚ·-

›· (40). √È ·ÛıÂÓ›˜ Ì ϋÚË ·ÓÙ·fiÎÚÈÛË ÛÙË

¯ËÌÂÈÔıÂڷ›· Ô˘ ¤Ï·‚·Ó ·ÎÙÈÓÔıÂڷ›· ·ÎÔ-

ÏÔ‡ıˆ˜ ‰ÂÓ Â›¯·Ó ηӤӷ ÏÂÔÓ¤ÎÙËÌ· ÛÙË Û˘ÓÔ-

ÏÈ΋ ÂÈ‚›ˆÛË. ¶·ÚÔÌÔ›ˆ˜, ÔÈ Rühl Î·È Û˘Ó. ‰ÂÓ

¶›Ó·Î·˜ 1. ∏ ÈÛÙÔÏÔÁÈ΋ Ù·ÍÈÓfiÌËÛË ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Hodgkin

1. Hodgkin ÎÏ·ÛÈÎfi ϤÌʈ̷ ñ √˙҉˘ ÛÎÏ‹Ú˘ÓÛË - √™ [nodular sclerosis (NS)]ñ §ÂÌÊÔÂÈÎÚ·Ù¤˜ - §∂ [lymphocyte rich classical

Hodgkin’s lymphoma (LRCHD)]ñ §ÂÌÊÔÂÓÈÎfi - §¶ [lymphocyte depleted subtypes (LD)]ñ ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ - ª∫ [mixed cellularity (MC)]

2. √˙҉˜ §ÂÌÊÔÂÈÎÚ·Ù¤˜ - √§∂∏§ [nodular lymphocytepredominance type (¡LPHD)]

3. ∞Ù·ÍÈÓfiÌËÙ· [unclassifiable cases (UC)]

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·85

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‰È·›ÛÙˆÛ·Ó ˘ÂÚÔ¯‹ ÛÙËÓ ÂÈ‚›ˆÛË ÌÂÙ¿ ·fi Û˘-

ÌÏËڈ̷ÙÈ΋ ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔıÂڷ›·˜ Û ¯·-

ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ·ÛıÂÓ›˜ (41). ŸÌˆ˜, ÛÙÔ˘˜ ·ÛıÂ-

Ó›˜ ÂӉȷ̤ÛÔ˘ Î·È ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘, Ë ¯ÔÚ‹ÁËÛË

·ÎÙÈÓÔıÂڷ›·˜ ÚÔۂ‚ÏË̤ÓÔ˘ ‰›Ô˘ Û˘ÌÏË-

ڈ̷ÙÈο ÌÂÙ¿ ÙË ¯ÔÚ‹ÁËÛË ¯ËÌÂÈÔıÂڷ›·˜ ‚ÂÏ-

Ù›ˆÛ ÛËÌ·ÓÙÈο ÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂ-

ÓÒÓ. ∞ÓÙ›ıÂÙ·, Ì›· ÈÔ ÚfiÛÊ·ÙË ÌÂϤÙË ·Ó·Ê¤ÚÂÈ

·ÚfiÌÔÈ· ÂÈ‚›ˆÛË ÙfiÛÔ ÛÙÔ˘˜ ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡

fiÛÔ Î·È ÛÙÔ˘˜ ·ÛıÂÓ›˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (42). ∞fi

ÙȘ ÌÂϤÙ˜ ·˘Ù¤˜ ÚÔ·ÙÂÈ fiÙÈ Ë ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔ-

ıÂڷ›·˜ ÚÔۂ‚ÏË̤ÓÔ˘ ‰›Ô˘ ÌÂÙ¿ ·fi ¯ËÌÂÈ-

ÔıÂڷ›·, Ô˘ ÚÔÛ·ÚÌfi˙ÂÙ·È ·Ó¿ÏÔÁ· Ì ÙÔ ÛÙ¿-

‰ÈÔ Ù˘ ÓfiÛÔ˘, ‚ÂÏÙÈÒÓÂÈ ÙËÓ ÂÈ‚›ˆÛË ÂχıÂÚË

Û˘Ì‚·Ì¿ÙˆÓ, fi¯È fï˜ Î·È ÙË Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË. ∏

‰Â ¯ÔÚ‹ÁËÛË ÈÛ¯˘ÚfiÙÂÚ˘ ¯ËÌÂÈÔıÂڷ›·˜ ÚÔÎÂÈ-

̤ÓÔ˘ Ó· ·ÔÊ¢¯ı› Ë ¯ÔÚ‹ÁËÛË ·ÎÙÈÓÔıÂڷ›·˜

‰ÂÓ ÛËÌ·›ÓÂÈ fiÙÈ ¤¯ÂÈ Î·È ÏÈÁfiÙÂÚË ÙÔÍÈÎfiÙËÙ·. ¶¿-

ÓÙˆ˜, Ë ¯ÔÚ‹ÁËÛË ¯·ÌËÏfiÙÂÚ˘ ‰fiÛ˘ ¯ËÌÂÈÔıÂÚ·-

›·˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·ÎÙÈÓÔıÂڷ›· ÚÔۂ‚ÏË-

̤ÓÔ˘ ‰›Ô˘ ¤¯ÂÈ ·ÚfiÌÔÈ· ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·

Ì ÙË ¯ÔÚ‹ÁËÛË ÌfiÓÔ ¯ËÌÂÈÔıÂڷ›·˜ ˘„ËÏfiÙÂÚ˘

‰fiÛ˘, ·ÏÏ¿ ·ÚÔ˘ÛÈ¿˙ÂÈ ÏÈÁfiÙÂÚË ÙÔÍÈÎfiÙËÙ·.

πÛÙÔÚÈο, Ô Û¯Â‰È·ÛÌfi˜ Ù˘ ¯ËÌÂÈÔıÂڷ›·˜

·Ï·ÈfiÙÂÚ· ÂÚÈÂÏ¿Ì‚·Ó ¢ڇÙÂÚ· ‰›· ·ÎÙÈÓÔ-

‚fiÏËÛ˘ Ù‡Ô˘ Ì·Ó‰‡·, Ô˘ ¤¯Ô˘Ó ηٷÚÁËı› ηÈ

·ÓÙÈηٷÛÙ·ı› ·fi ·ÎÙÈÓÔ‚fiÏËÛË ÚÔۂ‚ÏË̤-

ÓÔ˘ ‰›Ô˘. ª¿ÏÈÛÙ·, Ë Û‡Á¯ÚÔÓË Ù¿ÛË ÙˆÓ ·ÎÙÈÓÔ-

ıÂڷ¢ÙÒÓ Â›Ó·È Ó· ·ÎÙÈÓÔ‚ÔÏÔ‡Ó ÌfiÓÔ ÙÔ˘˜ ÚÔ-

ۂ‚ÏË̤ÓÔ˘˜ ÏÂÌÊ·‰¤Ó˜, Ì ·ÔÙ¤ÏÂÛÌ· Ë ‰fiÛË

·ÎÙÈÓÔ‚fiÏËÛ˘ ÛÙÔ˘˜ Ê˘ÛÈÔÏÔÁÈÎÔ‡˜ ÈÛÙÔ‡˜ Ó· ›-

Ó·È ÂÚÈÔÚÈṲ̂ÓË (43).

∏ Û‡Á¯ÚÔÓË Ù¿ÛË ÙˆÓ ÓÂfiÙÂÚˆÓ ıÂڷ¢ÙÈÎÒÓ

ÚˆÙÔÎfiÏÏˆÓ Â›Ó·È Ë ÚÔÛ·ÚÌÔÁ‹ Ù˘ ıÂڷ›·˜

·Ó¿ÏÔÁ· Ì ÙËÓ Ù·¯Â›· ‹ ‚Ú·‰Â›· ÚÒÈÌË ·¿ÓÙËÛË

ÛÙË ıÂڷ›·. ™Ùfi¯Ô˜ Â›Ó·È Ë ·Ó·ÁÓÒÚÈÛË ·ÛıÂÓÒÓ

Ì ¢ÓÔ˚ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Î·È Ë ÂÏ¿ÙÙˆÛË Ù˘ ¤ÓÙ·-

Û˘ Ù˘ ıÂڷ›·˜ Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ª›·

·fi ÙȘ ÚÒÙ˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÂÈ Î·Ï¿ ·ÔÙÂϤ-

ÛÌ·Ù· Û ·È‰È¿ Ì ÂÓÙÔÈṲ̂ÓÔ ∏§ Ô˘ ··ÓÙÔ‡Ó

ηϿ ÛÙË ¯ËÌÂÈÔıÂڷ›·, Ì ¯ÔÚ‹ÁËÛË ¯ËÌÂÈÔıÂ-

ڷ›·˜ ¯ˆÚ›˜ ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜ Î·È ·ÓıÚ·-

΢ÎϛӘ Î·È ·ÎÔÏÔ‡ıˆ˜ ¯·ÌËÏ‹ ‰fiÛË ·ÎÙÈÓÔıÂÚ·-

›·˜ (44). ¶·ÚÔÌÔ›ˆ˜, Ì›· ¿ÏÏË ÌÂϤÙË ÚÔÛ¿ıË-

Û ӷ ÂÍ·ÙÔÌÈ·ÛÂÈ ÙË ıÂڷ›· ·Ó¿ÏÔÁ· Ì ÙËÓ

ÚÒÈÌË ·¿ÓÙËÛË Û ·ÛıÂÓ›˜ ¯·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘,

Ì ηϿ ·ÔÙÂϤÛÌ·Ù· (45). ™‡Á¯ÚÔÓË Ù¿ÛË ÙˆÓ

ÎÏÈÓÈÎÒÓ ÌÂÏÂÙÒÓ Â›Ó·È Ë ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂÚ·-

›·˜ ·Ó¿ÏÔÁ· Ì ÙËÓ Ù·¯Â›· ‹ ‚Ú·‰Â›· ·¿ÓÙËÛË

Î·È ÙËÓ Ï‹ÚË ‹ ÌÂÚÈ΋ ·ÓÙ·fiÎÚÈÛË. ¶ÚÔÎÂÈ̤ÓÔ˘

Ó· ‚ÂÏÙȈı› Ë Ù·ÍÈÓfiÌËÛË ÙˆÓ ·ÛıÂÓÒÓ Û ÔÌ¿‰Â˜

ÎÈÓ‰‡ÓÔ˘, ÚfiÛÊ·Ù· ¤¯ÂÈ ¯ÚËÛÈÌÔÔÈËı› ÌÈ· ̤ıÔ-

‰Ô˜ ÂÎÙÂٷ̤Ó˘ ÌÂϤÙ˘ ÚˆÙÂ˚ÓÈÎÒÓ ‰ÂÈÎÙÒÓ ÛÙÔ

·›Ì· (surface enhanced laser desorption/ionization,

SELDI), Û ÌÈ· ÚÔÛ¿ıÂÈ· ·Ó·ÁÓÒÚÈÛ˘ ÌÈ·˜ Úˆ-

ÙˆÌÈ΋˜ ˘ÔÁÚ·Ê‹˜ Ô˘ ‰È·¯ˆÚ›˙ÂÈ Ù· ‰È¿ÊÔÚ·

ÎÏÈÓÈο ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘ (47). ∏ ÌÂϤÙË ÙˆÓ ÁÔÓÈ-

‰È·ÎÒÓ ˘ÔÁÚ·ÊÒÓ Û 29 ·ÛıÂÓ›˜ Ì ÎÏ·ÛÛÈÎfi ∏§

·Ó¤‰ÂÈÍÂ Û˘ÁÎÂÎÚÈ̤ӷ ÁÔÓ›‰È· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ

ÙËÓ ·¿ÓÙËÛË ÛÙË ıÂڷ›· (48). ªÂÏÏÔÓÙÈο, Ë

·Ó·ÁÓÒÚÈÛË ‚ÈÔÏÔÁÈÎÒÓ ‰ÂÈÎÙÒÓ ÂÓ‰¤¯ÂÙ·È Ó· ·Ô-

ÙÂϤÛÂÈ ÛËÌ·ÓÙÈÎfi ÂÚÁ·ÏÂ›Ô ÁÈ· ÙËÓ ¤ÁηÈÚË Úfi-

ÁÓˆÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂڷ›·, ÙËÓ ·Ó›¯Ó¢ÛË

ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ Î·È ÙËÓ Úfi‚Ï„Ë

Èı·ÓÒÓ ÌÂÏÏÔÓÙÈÎÒÓ ˘ÔÙÚÔÒÓ.

∆Ô √§∂∏§ Â›Ó·È Í¯ˆÚÈÛÙ‹ ÔÓÙfiÙËÙ· ·fi ÙÔ

ÎÏ·ÛÈÎfi ∏§, Ì ‰È·ÊÔÚÂÙÈο ÈÛÙÔ·ıÔÏÔÁÈο ηÈ

·ÓÔÛÔÊ·ÈÓÔÙ˘Èο ¯·Ú·ÎÙËÚÈÛÙÈο, ¢ÓÔ˚ÎfiÙÂÚË

ÚfiÁÓˆÛË, ·ÏÏ¿ Î·È Û˘¯ÓfiÙÂÚË ÂÌÊ¿ÓÈÛË ˘ÔÙÚÔ-

ÒÓ Î·È ‰Â˘ÙÂÚÔ·ıÒÓ ª∏§. ∏ ‰˘Ó·ÙfiÙËÙ· ÌfiÓÔ

¯ÂÈÚÔ˘ÚÁÈ΋˜ ÂÎÙÔÌ‹˜ Û ¯·ÌËÏÔ‡ ÛÙ·‰›Ô˘ ÓfiÛÔ

‚Ú›ÛÎÂÙ·È ˘fi ÌÂϤÙË (49).

∆¤ÏÔ˜, Ë ÌÂϤÙË ÔÏ˘ÌÔÚÊÈÛÌÒÓ Û ÁÔÓ›‰È· Ô˘

Έ‰ÈÎÔÔÈÔ‡Ó ¤Ó˙˘Ì· ÌÂÙ·‚ÔÏÈÛÌÔ‡ ÙˆÓ Ê·ÚÌ¿-

ΈÓ, fiˆ˜ ÙÔ Î˘Ùfi¯ÚˆÌ· P450, Î·È ·ÔÙÔ͛ӈÛ˘

ÙÔ˘ ÔÚÁ·ÓÈÛÌÔ‡, fiˆ˜ Ë S-ÙÚ·ÓÛÊÂÚ¿ÛË Ù˘ ÁÏÔ˘-

Ù·ıÂÈfiÓ˘, ¤¯ÂÈ ˆ˜ ÛÙfi¯Ô ÙËÓ ·Ó·ÁÓÒÚÈÛË ·ÛıÂÓÒÓ

Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÂÌÊ¿ÓÈÛ˘

ÙÔÍÈÎfiÙËÙ·˜ ·fi ÙË ıÂڷ›·. ∆Ú¤¯Ô˘Û˜ ÌÂϤÙ˜

¤¯Ô˘Ó ÂÈÛ·Á¿ÁÂÈ ÙËÓ ÂÊ·ÚÌÔÁ‹ Ê·ÚÌ·ÎÔÁÂÓˆÌÈÎÒÓ

ÌÂıfi‰ˆÓ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ·Ó·ÁÓˆÚ›ÛÔ˘Ó ‚ÈÔÏÔÁÈ-

ÎÔ‡˜ ‰Â›ÎÙ˜ Ô˘ ı· ÂÈÙÚ¤„Ô˘Ó ÌÂÏÏÔÓÙÈο ÙËÓ

ÂÈÏÔÁ‹ ÙÔ˘ ηٿÏÏËÏÔ˘ ıÂڷ¢ÙÈÎÔ‡ Û¯‹Ì·ÙÔ˜

Î·È ı· ÂÏ·ÙÙÒÛÔ˘Ó ÙËÓ ÂÌÊ¿ÓÈÛË ÂÈÏÔÎÒÓ (50).

™˘ÌÂÚ·ÛÌ·ÙÈο, Ë Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ÙˆÓ ·È-

‰ÈÒÓ Ì ÏÂÌÊÒÌ·Ù· Â›Ó·È Ôχ ηϋ, fï˜ οÔÈ·

·È‰È¿ ÂÍ·ÎÔÏÔ˘ıÔ‡Ó Ó· ˘ÔÙÚÔÈ¿˙Ô˘Ó ‹ Ó· ÂÌÊ·-

Ó›˙Ô˘Ó ·ÒÙÂÚ˜ ÂÈÏÔΤ˜. √È ÓÂfiÙÂÚ˜ ÎÏÈÓÈΤ˜

ÌÂϤÙ˜ ¤¯Ô˘Ó ˆ˜ ÛÙfi¯Ô ÙËÓ ·Ó·ÁÓÒÚÈÛË ÚÔÁÓˆÛÙÈ-

ÎÒÓ ·Ú·ÁfiÓÙˆÓ, ÙËÓ ÂÍ·ÙƠ̂΢ÛË Ù˘ ıÂڷ¢ÙÈ-

΋˜ ÚÔÛ¤ÁÁÈÛ˘ ·Ó¿ÏÔÁ· Ì ÙËÓ ÔÌ¿‰· ÎÈÓ‰‡ÓÔ˘ ηÈ

ÙËÓ ·ÓÙ·fiÎÚÈÛË ÛÙË ıÂڷ›· οı ·ÛıÂÓÔ‡˜, ¤ÙÛÈ

ÒÛÙ ӷ ‚ÂÏÙȈı› Ë ÂÈ‚›ˆÛË, Ì ٷ˘Ùfi¯ÚÔÓË ÂÏ¿Ù-

ÙˆÛË Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ÙÔÍÈÎfiÙËÙ·˜ Ù˘ ıÂڷ›·˜.

∏ ·Ó·Î¿Ï˘„Ë Ó¤ˆÓ Ê·ÚÌ¿ÎˆÓ Ô˘ ÛÙÔ¯Â‡Ô˘Ó ÂȉÈο

Û ÌÔÚÈ·Îfi Â›Â‰Ô ı· Û˘Ì‚¿ÏÂÈ ÛËÌ·ÓÙÈο. ¶·Ú¿Ï-

ÏËÏ·, Ë ·Ó¿Ù˘ÍË Â˘·›ÛıËÙˆÓ ÌÂıfi‰ˆÓ ÁÈ· ÙËÓ ·-

Ú·ÎÔÏÔ‡ıËÛË Ù˘ ·¿ÓÙËÛ˘ ÛÙË ıÂڷ›· Î·È Ù˘

¤ÁηÈÚ˘ ‰È¿ÁÓˆÛ˘ Ù˘ ˘ÔÙÚÔ‹˜ Î·È Ë ·Ó›¯Ó¢ÛË

ÂÏ¿¯ÈÛÙ˘ ˘ÔÏÂÈfiÌÂÓ˘ ÓfiÛÔ˘ ı· ‚ÔËı‹ÛÔ˘Ó ÛÙËÓ

ÚÒÈÌË ·Ó·ÁÓÒÚÈÛË Ù˘ ¯ËÌÂÈÔ-¢·ÈÛıËÛ›·˜ ‹ ·ÓıÂ-

ÎÙÈÎfiÙËÙ·˜ Ù˘ ÓfiÛÔ˘ Î·È ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ηٷÏÏË-

ÏfiÙÂÚÔ˘ ıÂڷ¢ÙÈÎÔ‡ Û¯‹Ì·ÙÔ˜. ∏ ¯Ú‹ÛË Û‡Á¯ÚÔ-

ÓˆÓ ‚ÈÔÏÔÁÈÎÒÓ ÌÂÏÂÙÒÓ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ Î·È

ÂÎÙÂÓ‹ ÁÔÓȉȷ΋ Î·È ÚˆÙÂ˚ÓÈ΋ ·Ó¿Ï˘ÛË ı· ‰ÒÛÂÈ

Ó¤· ‰È¿ÛÙ·ÛË ÛÙËÓ Î·Ù·ÓfiËÛË ÙˆÓ ·ıÔÁÂÓÂÙÈÎÒÓ

86 µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹

Paediatriki 2009;72:1-9

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·86

Page 15: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

87§ÂÌÊÒÌ·Ù· ÛÙ· ·È‰È¿

¶·È‰È·ÙÚÈ΋ 2009;72:1-9

Ì˯·ÓÈÛÌÒÓ, ÛÙËÓ Ù·ÍÈÓfiÌËÛË ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Î·ÈÛÙË ‚ÂÏÙÈÛÙÔÔ›ËÛË ÙˆÓ ıÂڷ¢ÙÈÎÒÓ ÌÂıfi‰ˆÓ.

∂˘¯·ÚÈÛٛ˜

∂˘¯·ÚÈÛÙԇ̠ȉȷÈÙ¤Úˆ˜ ÙÔÓ ∂›ÎÔ˘ÚÔ ∫·ıËÁËÙ‹ ¶·È-

‰È·ÙÚÈ΋˜ ÙÔ˘ ¢ËÌÔÎÚ›ÙÂÈÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘ £Ú¿Î˘ Î.

∂ÏȉÔÊfiÚÔ ª·ÓÙ·‰¿ÎË ÁÈ· ÙȘ ÏÂÙÔÌÂÚ›˜ ÏËÚÔÊÔڛ˜

Ô˘ Ì·˜ ¤‰ˆÛ ۯÂÙÈο Ì ٷ ÙÚ¤¯ÔÓÙ· ·ÌÂÚÈηÓÈο ıÂÚ·-

¢ÙÈο ÚˆÙfiÎÔÏÏ·.

µÈ‚ÏÈÔÁÚ·Ê›·

1. Sandlund JT, Downing JR, Crist WM. Non-Hodgkin's

lymphoma in childhood. N Engl J Med 1996;334:1238-1248.

2. Anagnostopoulos I. Individualized target therapy of

malignant lymphomas: an outlook. Recent Results Cancer

Res 2007;176:177-188.

3. Cairo MS, Raetz E, Lim MS, Davenport V, Perkins SL.

Childhood and adolescent non-Hodgkin lymphoma: new

insights in biology and critical challenges for the future.

Pediatr Blood Cancer 2005;45:753-769.

4. Reiter A. Diagnosis and treatment of childhood non-

Hodgkin lymphoma. Hematology Am Soc Hematol Educ

Program 2007;2007:285-296.

5. Reiter A, Schrappe M, Tiemann M, Ludwig WD, Yakisan

E, Zimmermann M et al. Improved treatment results in

childhood B-cell neoplasms with tailored intensification of

therapy: A report of the Berlin-Frankfurt-Münster Group

Trial NHL-BFM 90. Blood 1999;94:3294-3306.

6. Seidemann K, Zimmermann M, Book M, Meyer U,

Burkhardt B, Welte K, et al. Tumor necrosis factor and

lymphotoxin alfa genetic polymorphisms and outcome in

pediatric patients with non-Hodgkin's lymphoma: results

from Berlin-Frankfurt-Münster Trial NHL-BFM 95. Clin

Oncol 2005;23:8414-8421.

7. National Cancer Institute, U.S. National Institutes of Health

[Webpage, Internet]. http://www.cancer.gov/cancertopics/

pdq/treatment/child-non-hodgkins/ healthprofessional.

8. Raetz E, Perkins S, Davenport V, Cairo MS. B large-cell

lymphoma in children and adolescents. Cancer Treat Rev

2003;29:91-98.

9. Heerema NA, Bernheim A, Lim MS, Look AT, Pasqualucci

L, Raetz E, et al. State of the Art and Future Needs in

Cytogenetic/Molecular Genetics/Arrays in childhood

lymphoma: summary report of workshop at the First

International Symposium on childhood and adolescent

non-Hodgkin lymphoma, April 9, 2003, New York City,

NY. Pediatr Blood Cancer 2005;45:616-622.

10. Patte C. Treatment of mature B-ALL and high grade B-NHL

in children. Best Pract Res Clin Haematol 2002;15: 695-711.

11. Cairo MS, Gerrard M, Sposto R, Auperin A, Pinkerton CR,

Michon J, et al. FAB LMB96 International Study Committee.

Results of a randomized international study of high-risk

central nervous system B non-Hodgkin lymphoma and B

acute lymphoblastic leukemia in children and adolescents.

Blood 2007;109:2736-2743.

12. Patte C, Auperin A, Gerrard M, Michon J, Pinkerton R,

Sposto R, et al. FAB/LMB96 International Study Committee.

Results of the randomized international FAB/LMB96 trial for

intermediate risk B-cell non-Hodgkin lymphoma in children

and adolescents: it is possible to reduce treatment for the

early responding patients. Blood 2007;109:2773-2780.

13. Woessmann W, Seidemann K, Mann G, Zimmermann M,

Burkhardt B, Oschlies I, et al. BFM Group. The impact of

the methotrexate administration schedule and dose in the

treatment of children and adolescents with B-cell

neoplasms: a report of the BFM Group Study NHL-

BFM95. Blood 2005;105:948-958.

14. Patte C, Auperin A, Michon J, Behrendt H, Leverger G,

Frappaz D, et al. The Société Française d'Oncologie

Pédiatrique LMB89 protocol: highly effective multiagent

chemotherapy tailored to the tumor burden and initial

response in 561 unselected children with B-cell

lymphomas and L3 leukemia. Blood 2001;97:3370-3379.

15. Alizadeh AA, Eisen MB, Davis RE, Ma C, Lossos IS,

Rosenwald A, et al. Distinct types of diffuse large B-cell

lymphoma identified by gene expression profiling. Nature

2000;403:503-511.

16. Rosenwald A, Wright G, Chan WC, Connors JM, Campo

E, Fisher RI, et al. The use of molecular profiling to predict

survival after chemotherapy for diffuse large-B-cell

lymphoma. N Engl J Med 2002;346:1937-1947.

17. Oschlies I, Klapper W, Zimmermann M, Krams M,

Wacker HH, Burkhardt B, et al. Diffuse large B-cell

lymphoma in pediatric patients belongs predominantly to

the germinal-center type B-cell lymphomas: a clinico-

pathologic analysis of cases included in the German BFM

(Berlin-Frankfurt-Munster) Multicenter Trial. Blood

2006;107:4047-4052.

18. Cooney-Qualter E, Krailo M, Angiolillo A, Fawwaz RA,

Wiseman G, Harrison L, et al. Children's Oncology Group.

A phase I study of 90yttrium-ibritumomab-tiuxetan in

children and adolescents with relapsed/refractory CD20-

positive non-Hodgkin's lymphoma: a Children's Oncology

Group study. Clin Cancer Res. 2007;13:5652s-5660s.

19. Fujita N, Mori T, Mitsui T, Inada H, Horibe K, Tsurusawa

M; for the Lymphoma Committee of the Japanese

Pediatric Leukemia/Lymphoma Study Group. The role of

hematopoietic stem cell transplantation with relapsed or

primary refractory childhood B-cell non-Hodgkin

lymphoma and mature B-cell leukemia: A retrospective

analysis of enrolled cases in Japan. Pediatr Blood Cancer.

2008 Apr 21. [Epub ahead of print].

20. Attarbaschi A, Dworzak M, Steiner M, Urban C, Fink FM,

Reiter A, et al. Outcome of children with primary resistant

or relapsed non-Hodgkin lymphoma and mature B-cell

leukemia after intensive first-line treatment: a population-

based analysis of the Austrian Cooperative Study Group.

Pediatr Blood Cancer 2005;44:70-76.

21. Seidemann K, Tiemann M, Schrappe M, Yakisan E,

Simonitsch I, Janka-Schaub G, Dörffel W, Zimmermann M,

Mann G, Gadner H, Parwaresch R, Riehm H, Reiter A.

Short-pulse B-non-Hodgkin lymphoma-type chemo-

therapy is efficacious treatment for pediatric anaplastic large

cell lymphoma: a report of the Berlin-Frankfurt-Münster

Group Trial NHL-BFM 90. Blood 2001;97:3699-3706.

22. Brugières L, Deley MC, Pacquement H, Meguerian-Bedoyan

Z, Terrier-Lacombe MJ, Robert A, Pondarré C, Leverger G,

Devalck C, Rodary C, Delsol G, Hartmann O. CD30(+)

anaplastic large-cell lymphoma in children: analysis of 82

patients enrolled in two consecutive studies of the French

Society of Pediatric Oncology. Blood 1998;92:3591-3598.

23. Laver JH, Kraveka JM, Hutchison RE, Chang M, Kepner J,

Schwenn M, Tarbell N, Desai S, Weitzman S, Weinstein

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·87

Page 16: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

HJ, Murphy SB. Advanced-stage large-cell lymphoma inchildren and adolescents: results of a randomized trialincorporating intermediate-dose methotrexate and high-dose cytarabine in the maintenance phase of the APOregimen: a Pediatric Oncology Group phase III trial. J ClinOncol 2005;23:541-547.

24. Nadali G, Vinante F, Stein H, Todeschini G, Tecchio C,Morosato L, et al. Serum levels of the soluble form of CD30molecule as a tumor marker in CD30+ anaplastic large-celllymphoma. J Clin Oncol 1995;13:1355-1360.

25. Franchina M, Woo AJ, Dods J, Karimi M, Ho D, WatanabeT, et al. The CD30 gene promoter microsatellite bindstranscription factor Yin Yang 1 (YY1) and shows geneticinstability in anaplastic large cell lymphoma. J Pathol2008;214:65-74.

26. Wellmann A, Thieblemont C, Pittaluga S, Sakai A, Jaffe ES,Siebert P, et al. Detection of differentially expressed genesin lymphomas using cDNA arrays: identification ofclusterin as a new diagnostic marker for anaplastic large-cell lymphomas. Blood 2000;96:398-404.

27. Brugières L, Quartier P, Le Deley MC, Pacquement H,Perel Y, Bergeron C, et al. Relapses of childhood anaplasticlarge-cell lymphoma: treatment results in a series of 41children - a report from the French Society of PediatricOncology. Ann Oncol 2000;11:53-58.

28. Hodgson DC, Hudson MM, Constine LS. Pediatrichodgkin lymphoma: maximizing efficacy and minimizingtoxicity. Semin Radiat Oncol 2007;17:230-242.

29. Skinnider BF, Mak TW. The role of cytokines in classicalHodgkin lymphoma. Blood 2002;99:4283-4297.

30. Re D, Küppers R, Diehl V. Molecular pathogenesis ofHodgkin's lymphoma. J Clin Oncol 2005;23:6379-6386.

31. Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J, et al. The World HealthOrganization classification of neoplasms of the hemato-poietic and lymphoid tissues: report of the ClinicalAdvisory Committee meeting - Airlie House, Virginia,November, 1997. Hematol J 2000;1:53-66.

32. Bar-Shalom R. Gallium SPECT/CT in lymphoma: the upsand downs of functional imaging. Eur J Nucl Med MolImaging 2005;32:1247-1249.

33. Kaste SC, Howard SC, McCarville EB, Krasin MJ, Kogos PG,Hudson MM. 18F-FDG-avid sites mimicking active diseasein pediatric Hodgkin's. Pediatr Radiol 2005;35:141-154.

34. Furth C, Denecke T, Steffen I, Ruf J, Voelker T, Misch D, etal. Correlative imaging strategies implementing CT, MRI,and PET for staging of childhood Hodgkin disease. JPediatr Hematol Oncol 2006;28:501-512.

35. Hutchings M, Loft A, Hansen M, Berthelsen AK, Specht L.Clinical impact of FDG-PET/CT in the planning ofradiotherapy for early-stage Hodgkin lymphoma. Eur JHaematol 2007;78:206-212.

36. Montravers F, McNamara D, Landman-Parker J, GrahekD, Kerrou K, Younsi N, et al. [(18)F]FDG in childhoodlymphoma: clinical utility and impact on management.Eur J Nucl Med Mol Imaging 2002;29:1155-1165.

37. Menzel C, Döbert N, Mitrou P, Mose S, Diehl M, Berner U,Grünwald F. Positron emission tomography for the stagingof Hodgkin's lymphoma - increasing the body of evidencein favor of the method. Acta Oncol. 2002;41:430-436.

38. Prosnitz LR. The Ann Arbor staging system for Hodgkin's

disease: does E stand for error? Int J Radiat Oncol Biol Phys1977;2:1039.

39. Schwartz CL. The management of Hodgkin disease in theyoung child. Curr Opin Pediatr 2003;15:10-16.

40. Nachman JB, Sposto R, Herzog P, Gilchrist GS, WoldenSL, Thomson J, et al; Children's Cancer Group.Randomized comparison of low-dose involved-fieldradiotherapy and no radiotherapy for children withHodgkin's disease who achieve a complete response tochemotherapy. J Clin Oncol 2002;20:3765-3771.

41. Rühl U, Albrecht M, Dieckmann K, Lüders H, MarciniakH, Schellenberg D, et al. Response-adapted radiotherapy inthe treatment of pediatric Hodgkin's disease: an interimreport at 5 years of the German GPOH-HD 95 trial. Int JRadiat Oncol Biol Phys 2001;51:1209-1218.

42. Kung FH, Schwartz CL, Ferree CR, London WB, TernbergJL, Behm FG, et al; Children's Oncology Group. POG8625: a randomized trial comparing chemotherapy withchemoradiotherapy for children and adolescents withStages I, IIA, IIIA1 Hodgkin Disease: a report from theChildren’s Oncology Group. J Pediatr Hematol Oncol2006;28:362-368.

43. Girinsky T, van der Maazen R, Specht L, Aleman B,Poortmans P, Lievens Y, et al. Involved-node radiotherapy(INRT) in patients with early Hodgkin lymphoma:concepts and guidelines. Radiother Oncol 2006;79:270-277.

44. Landman-Parker J, Pacquement H, Leblanc T, HabrandJL, Terrier-Lacombe MJ, Bertrand Y, et al. Localizedchildhood Hodgkin's disease: response-adapted chemo-therapy with etoposide, bleomycin, vinblastine, andprednisone before low-dose radiation therapy-results ofthe French Society of Pediatric Oncology Study MDH90. JClin Oncol 2000;18:1500-1507.

45. Schwartz CL, Constine LS, London W, Marcus R, KampsW, Hutchinson R, et al. POG-9425: response-based,intensively timed therapy for intermediate/high stage(IS/HS) pediatric Hodgkin’s disease. Proc Am Soc ClinOncol 2002;21:abstr 1555.

46. Hudson MM, Krasin M, Link MP, Donaldson SS, BillupsC, Merchant TE, et al. Risk-adapted, combined-modalitytherapy with VAMP/COP and response-based, involved-field radiation for unfavorable pediatric Hodgkin's disease.J Clin Oncol 2004;22:4541-4550.

47. Qi L, Cazares L, Johnson C, de Alarcon P, Kupfer GM,Semmes OJ. Serum protein expression profiling inpediatric Hodgkin lymphoma: A report from theChildren's Oncology Group. Pediatr Blood Cancer. 2008.[Epub ahead of print].

48. Saãnchez-Aguilera A, Montalbaãn C, de la Cueva P, Saãnchez-Verde L, Morente MM, Garc›a-Cos›o M, et al. Tumormicroenvironment and mitotic checkpoint are key factorsin the outcome of classic Hodgkin lymphoma. Blood.2006;108:662-668.

49. Cincinati Children’s Hospital Medical Center [Webpage,Internet]. http://www.cincinnatichildrens.org/svc/alpha/c/cancer/trials/hodgkins/ahod03p1-pf.htm.

50. Kelly KM, Perentesis JP; Children's Oncology Group.Polymorphisms of drug metabolizing enzymes andmarkers of genotoxicity to identify patients with Hodgkin'slymphoma at risk of treatment-related complications. AnnOncol. 2002;13(Suppl 1):34-39.

88 µ. ¢·ÓËÏ¿ÙÔ˘, ª. ∫·ÏÌ·ÓÙ‹

Paediatriki 2009;72:1-9

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∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË

Ã. ∫Ô˘ÙÛ·˘Ù›ÎË, ∂. ª¿ÓÙ˙ÈÔ˘, ¡. ª˘ÚÈÔÎÂÊ·ÏÈÙ¿Î˘

¶ÂÚ›ÏË„Ë: ∏ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË ·ÔÙÂÏ› Û¿ÓÈÔ Û‡Ó‰ÚÔÌÔ, fiÔ˘ ÙÔ ÚÔÂÍ¿Ú¯ÔÓ¯·Ú·ÎÙËÚÈÛÙÈÎfi Â›Ó·È Ë ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÌÔÓÔ˘ÚËÓÈÎÔ‡ Ê·ÁÔ΢ÙÙ·ÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓηÏÔ‹ıË ÁÂÓÈÎÂ˘Ì¤ÓË ÈÛÙÈÔ΢ÙÙ·ÚÈ΋ ‰È‹ıËÛË ‰È·ÊfiÚˆÓ ÔÚÁ¿ÓˆÓ Ì ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË. ∆· Û˘ÌÙÒÌ·Ù·Ô˘ ·Ú·¤ÌÔ˘Ó ÛÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ Â›Ó·È Ô Â›ÌÔÓÔ˜ ˘ÚÂÙfi˜ (100%), ÔÈ Î˘ÙÙ·ÚÔÂӛ˜ (95%), ËË·ÙÈ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· (96%), Ë Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›· (84%), Ô ›ÎÙÂÚÔ˜ (55%), Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· (43%),ÔÈ ‰È·Ù·Ú·¯¤˜ Ù˘ ‹Í˘ (75%) Î·È ÙÔ˘ ∫¡™ (32%) Î·È Ë ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË Ì ·ÈÌÔÊ·ÁÔ·ÙÙ·Ú· ÛÙÔÓÌ˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Î·È Û fiÚÁ·Ó· ÙÔ˘ ‰ÈÎÙ˘ÔÂÓ‰ÔıËÏÈ·ÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ Û fiÏÔ˘˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ∆· ÂÚÁ·ÛÙË-Úȷο Â˘Ú‹Ì·Ù· Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È Â›Ó·È ·ÔÙ¤ÏÂÛÌ· ˘ÂÚÊÏÂÁÌÔÓÒ‰Ô˘˜ ‰È·‰Èηۛ·˜ Î·È Û˘Ó›ÛÙ·ÓÙ·È Û·‡ÍËÛË ÛÙÔ ·›Ì· ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ, Ù˘ ÊÂÚÚÈÙ›Ó˘, Ù˘ Á·Ï·ÎÙÈ΋˜ ·Ê˘‰ÚÔÁoÓ¿Û˘, ÙˆÓ ÙÚÈÁÏ˘ÎÂÚȉ›ˆÓ,ÙˆÓ ÙÚ·ÓÛ·ÌÈÓ·ÛÒÓ Î·È Ù˘ ÔÏÂÚ˘ıÚ›Ó˘, ηıÒ˜ Î·È Û ÂÏ¿ÙÙˆÛË ÙÔ˘ ÈÓˆ‰ÔÁfiÓÔ˘. ¢È·ÎÚ›ÓÂÙ·È Û ÔÈÎÔÁÂÓ¤˜‹ ÚˆÙÔ·ı¤˜ Î·È Û ‰Â˘ÙÂÚÔ·ı¤˜, Ô˘ ‰È·ÎÚ›ÓÔÓÙ·È ‰‡ÛÎÔÏ· ÌÂٷ͇ ÙÔ˘˜ ÂÂȉ‹ Î·È ÛÙȘ ‰‡Ô ÌÔÚʤ˜ ÌÔ-ÚÔ‡Ó Ó· ·Ó¢ÚÂıÔ‡Ó ÔÈ ›‰È˜ ÌÂÙ·ÏÏ¿ÍÂȘ, ÂÓÒ ¤¯Ô˘Ó ·ÚfiÌÔÈ· ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Î·È ÚfiÁÓˆ-ÛË. ∂›Û˘, ·ÊÔÚÌ‹ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Î·È ÙˆÓ ‰‡Ô ÌÔÚ› Ó· ·ÔÙÂÏ› οÔÈ· Ïԛ̈ÍË. ™˘¯Ó¿ Ë ‰È¿ÁÓˆÛˉȷʇÁÂÈ, ηıÒ˜ Ë ÓfiÛÔ˜ ÌÈÌÂ›Ù·È ÔÈΛϘ ÓÔÛÔÏÔÁÈΤ˜ ÔÓÙfiÙËÙ˜, οÙÈ Ô˘ ÌÔÚ› Ó· ·Ô‚› ÌÔÈÚ·›Ô, ·ÊÔ‡¯ˆÚ›˜ ıÂڷ›· Â›Ó·È Ôχ Èı·Ó‹ Ë Î·Ù¿ÏËÍË ÙÔ˘ ·ÛıÂÓÔ‡˜, ÂÓÒ Ì ÙËÓ Î·Ù¿ÏÏËÏË ·ÁˆÁ‹ ηÈ, ΢ڛˆ˜, Ì ÙËÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ¤¯ÂÈ ÂÈÙ¢¯ı› ›·ÛË Û ˘„ËÏ¿ ÔÛÔÛÙ¿.

§¤ÍÂȘ ÎÏÂȉȿ: ∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË, ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ, ·È‰È¿.

Haemophagocytic lymphohistiocytosis - HLH

C. Koutsaftiki, E. Mantziou, N. Myriokefalitakis

Abstract: Haemophagocytic lymphÔhistiocytosis is a rare syndrome characterized by a reactive disorderof the mononuclear phagocytic system which gives rise to benign, generalized histiocytic proliferation inmany organs, with marked haemophagocytosis. The symptoms and signs that are associated with thediagnosis, along with their frequency of appearance, are: persistent fever (100%), cytopenia (96%), liverdysfunction (96%), hepatosplenomegaly (84%), jaundice (55%), lymphadenopathy (43%),coagulopathy (75%) and central nervous system symptoms (32%). Lymphohistiocytosis, with thepresence of haemophagocytes in the bone marrow and the reticuloendothelial system, is observed in allcases. The laboratory findings that are usually present are due to the associated hyperinflammatoryprocess and usually consist of hypercytokinaemia, hyperferritinaemia, elevated blood levels of lactatedehydrogenase, hypertriglyceridaemia, hypofibrinogenaemia, transaminasaemia, hyperbilirubinaemia,etc. Two types of haemophagocytic lymphÔhistiocytosis have been identified, the familial or primarysyndrome and the secondary, but there is difficulty in distinguishing between them, as similar mutationsand clinical and laboratory findings may be observed in both, and they have roughly the same prognosis.In addition, the triggering factor for both can be, and is in most cases, an infection. It is easy for diagnosis tobe delayed, because the syndrome can mimic a variety of diseases. Delay in diagnosis can be fatal, sinceprompt treatment, specifically bone marrow transplantation, can achieve a cure in most cases.

Key words: ∏aemophagocytic lymphÔhistiocytosis, haemophagocytic syndrome, children.

89∞¡∞™∫√¶∏™∏ REVIEW ARTICLE

∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ¶ÂÓÙ¤Ï˘

AÏÏËÏÔÁÚ·Ê›·:

ÃÚ˘Û‹ ∫Ô˘ÙÛ·˘Ù›ÎË[email protected]∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ¶ÂÓÙ¤Ï˘, 152 36, ¶. ¶ÂÓÙ¤ÏË

1st Department ofPaediatrics, GeneralChildren’s Hospital ofPenteli, Athens, Greece

Correspondence:

Chryssie [email protected] Department ofPaediatrics, GeneralChildren’s Hospital ofPenteli, Palaia Penteli 152 36,Athens, Greece

¶·È‰È·ÙÚÈ΋ 2009;72:89-000

∂ÈÛ·ÁˆÁ‹ - √ÚÈÛÌÔ›

∏ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿Úˆ-ÛË ‹ ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈÎfi Û‡Ó‰ÚÔÌÔ (∞º™)·ÓÙÈÚÔۈ‡ÂÈ ¤Ó· Ê¿ÛÌ· ·fi ÎÏËÚÔÓÔÌÔ‡-ÌÂÓ˜ Î·È Â›ÎÙËÙ˜ ηٷÛÙ¿ÛÂȘ, Ì ¿ÏÏÔÙ¿ÏÏË ‚·Ú‡ÙËÙ· ÛÙË ‰È·Ù·Ú·¯‹ Ù˘ ·ÓÔÛÔÏÔÁÈ-΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ (1).

¶ÂÚÈÏ·Ì‚¿ÓÂÈ ‰‡Ô ·ÚȘ ηٷÛÙ¿ÛÂȘ, ÌÂÎÔÈÓ¿ ÎÏÈÓÈο Î·È ·ıÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο:

ÙÔ ÚˆÙÔ·ı¤˜ ‹ ÔÈÎÔÁÂÓ¤˜ ∞º™ (Familial

Haemophagocytic Lymphoistiocytosis, FHL) ηÈ

ÙÔ ‰Â˘ÙÂÚÔ·ı¤˜ ∞º™ (secondary Haemo-

phagocytic Lymphoistiocytosis, HLH) (1).

∏ ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ·ÔÙÂÏ› ·ıÔÏÔÁÔ-

·Ó·ÙÔÌÈÎfi ‡ÚËÌ·, fiÔ˘ ÂÓÂÚÁÔÔÈË̤ӷ Ì·-

ÎÚÔÊ¿Á· Ê·ÁÔ΢ÙÙ·ÚÒÓÔ˘Ó Î·È Î·Ù·ÛÙÚ¤ÊÔ˘Ó

ÂÚ˘ıÚÔ·ÙÙ·Ú·, Ï¢ÎÔ·ÙÙ·Ú·, ¿ÏÏ· Ì·ÎÚÔÊ¿-

Á·, ηıÒ˜ Î·È Úfi‰ÚÔÌ· ·ÙÙ·Ú¿ ÙÔ˘˜ (2).

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·89

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∆Ô ∏LH, ÚˆÙÔ·ı¤˜ Î·È ‰Â˘ÙÂÚÔ·ı¤˜, ÌÔÚ›

Ó· Â›Ó·È ı·Ó·ÙËÊfiÚÔ, ÙfiÛÔ ÁÈ· Ù· ·È‰È¿ fiÛÔ Î·È ÁÈ·

ÙÔ˘˜ ÂÓ‹ÏÈΘ, ȉȷ›ÙÂÚ· ·Ó ·Ú·Ì›ÓÂÈ ·‰È¿ÁÓˆÛÙÔ

Î·È ‰ÂÓ ·ÓÙÈÌÂÙˆÈÛÙ› (3).

™˘¯ÓfiÙËÙ·

∏ Û˘¯ÓfiÙËÙ· Ù˘ ÓfiÛÔ˘ ‰È·Ê¤ÚÂÈ ·fi ¯ÒÚ· ÛÂ

¯ÒÚ·, ÂÓÒ, ÂÍ ·ÈÙ›·˜ ÙˆÓ ‰˘ÛÎÔÏÈÒÓ Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ

ÛÙË ‰È¿ÁÓˆÛË, Â›Ó·È Èı·Ófi ÔÈ ·ÚÈıÌÔ› Ô˘ ·Ó·Ê¤ÚÔ-

ÓÙ·È Ó· ÌËÓ ·ÓÙ·ÔÎÚ›ÓÔÓÙ·È ÛÙËÓ Ú·ÁÌ·ÙÈÎfiÙËÙ·.

™ÙË ™Ô˘Ë‰›·, Ë Â›ÙˆÛË ÙÔ˘ FLH ÔÏÔÁ›˙ÂÙ·È

Û 1:50.000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ‚ÚÂÊÒÓ, Ì ·Ó·ÏÔÁ›·

·ÚÚ¤ÓˆÓ:ıËϤˆÓ ~1:1 ‹ 1,2 ÂÚÈÙÒÛÂȘ:1.000.000

·È‰È¿ ÙÔ ÚfiÓÔ (1,4). ªÂÁ¿Ï˜ ÛÂÈÚ¤˜ ÂÚÈÙÒÛˆÓ

Ì ∞º™ ¤¯Ô˘Ó ·Ó·ÎÔÈÓˆı› ·fi ÙËÓ π·ˆÓ›· ηÈ

ÙËÓ ∆·˚‚¿Ó, ·ÏÏ¿ ‰ÂÓ Â›Ó·È ÁÓˆÛÙfi ·Ó Ë Û˘¯ÓfiÙËÙ·

Â›Ó·È ˘„ËÏfiÙÂÚË ÛÙÔ˘˜ ∞ÛÈ¿Ù˜ ·fi fi,ÙÈ ÛÙÔ˘˜ ∂˘-

Úˆ·›Ô˘˜ (5).

™Â ÌÂϤÙË ·fi ¤Ó· ΤÓÙÚÔ ÛÙËÓ ∆Ô˘ÚΛ·, Û ‰È¿-

ÛÙËÌ· 7 ÂÙÒÓ, ‰È·ÈÛÙÒıËΠHLH Û 18 ·ÛıÂÓ›˜

Î·È FHL Û 19 ·ÛıÂÓ›˜, ·fi 34.250 Ô˘ ÓÔÛËχÙË-

Î·Ó ÛÙÔ ›‰ÈÔ ‰È¿ÛÙËÌ· (0,052% Î·È 0,055% ·ÓÙÈÛÙÔ›-

¯ˆ˜) (6).

°ÂÓÈο ÁÈ· ÙÔ FHL

¶ÂÚ›Ô˘ 25% fiÏˆÓ ÙˆÓ ÂÚÈÙÒÛÂˆÓ ∞º™ ›ӷÈ

ÚˆÙÔ·ı›˜-ÔÈÎÔÁÂÓ›˜ Î·È Û¯ÂÙ›˙ÔÓÙ·È Ì ÙË Û˘-

¯ÓfiÙËÙ· Û˘ÁÁ¤ÓÂÈ·˜ ÌÂٷ͇ ÙˆÓ ÁÔÓ¤ˆÓ.

¶ÚÒÙË ÂÚÈÁÚ·Ê‹ ÙÔ˘ FLH ¤ÁÈÓ ÙÔ 1952 (7).

¶ÚfiÎÂÈÙ·È ÁÈ· ·˘ÙÔۈ̷ÙÈ΋ ˘ÔÏÂÈfiÌÂÓË ‰È·Ù·-

Ú·¯‹ Ô˘ ÚÔÛ‚¿ÏÏÂÈ Î˘Ú›ˆ˜ ‚Ú¤ÊË, ·ÏÏ¿ Î·È ·È-

‰È¿ ÌÈÎÚ‹˜ ËÏÈΛ·˜, ÂÓÒ ¤¯Ô˘Ó ·Ó·ÊÂÚı› Î·È ÛÔÚ·-

‰ÈΤ˜ ÂÚÈÙÒÛÂȘ. ∂ΉËÏÒÓÂÙ·È Û˘¯ÓfiÙÂÚ· ÛÙÔÓ

ÚÒÙÔ ¯ÚfiÓÔ Ù˘ ˙ˆ‹˜ Î·È Û¯Â‰fiÓ ¿ÓÙ· ÚÈÓ ÙÔÓ

¤‚‰ÔÌÔ (7,8,9,10,11,12).

∏ ¤Î‚·ÛË Â›Ó·È ÌÔÈÚ·›·, Â¿Ó Ë ÓfiÛÔ˜ ‰ÂÓ ·ÓÙÈÌÂ-

ÙˆÈÛÙ›. ∏ ̤ÛË ÂÈ‚›ˆÛË ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ‰ÂÓ

˘Ô‚¿ÏÏÔÓÙ·È Û ıÂڷ›· Â›Ó·È 2 Ì‹Ó˜, ÁÂÁÔÓfi˜

Ô˘ ‰Â›¯ÓÂÈ ÙËÓ ¤ÌÊ·ÛË Ô˘ Ú¤ÂÈ Ó· ‰Ôı› ÛÙËÓ

¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È ıÂڷ›· (12).

¶ÚfiÛÊ·Ù· ¤ÁÈÓ ٷÍÈÓfiÌËÛË Ù˘ FHL Û FHL-1,

FHL-2, FHL-3, ηıÒ˜ Î·È ¿ÏÏÔ˘˜ ÔÙ‡Ô˘˜, Ì ‚¿-

ÛË ÙȘ ÂοÛÙÔÙ ¢ÚÈÛÎfiÌÂÓ˜ ÁÂÓÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜

(10). ∏ ÁÂÓÂÙÈ΋ ·ÓˆÌ·Ï›· ÌÔÚ› Ó· ·ÊÔÚ¿ ÙËÓ

ÚˆÙ½ÓË ÂÚÊÔÚ›ÓË, Ë ÔÔ›· Û˘ÓÙÂÏ› ÛÙËÓ Î˘ÙÙ·-

ÚÔÙÔÍÈ΋ ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Ê˘ÛÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

ÊÔÓ¤ˆÓ (natural killers ‹ NK) Î·È ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆

(13). √ ˘Â‡ı˘ÓÔ˜ ÁÔÓȉȷÎfi˜ ÙfiÔ˜ ‚Ú›ÛÎÂÙ·È ÛÙÔ

¯ÚˆÌfiۈ̷ 10q21-22, ¯·Ú·ÎÙËÚ›˙ÂÈ ÙËÓ FHL-2 ηÈ

··ÓÙ¿Ù·È ÛÙÔ 20-30% ÙˆÓ ·ÛıÂÓÒÓ (10).

√È ÌÂÙ·ÏÏ¿ÍÂȘ ÛÙÔ ÁÔÓ›‰ÈÔ MUNC 13-4, Ô˘

‰ڿ˙ÂÙ·È ÛÙÔ ¯ÚˆÌfiۈ̷ 17q25, Ô‰ËÁÔ‡Ó ÛÙËÓ ÂÎ-

‰‹ÏˆÛË Ù˘ FHL-3, ÛÙËÓ ÔÔ›· ˘¿Ú¯ÂÈ ·Ó¿ÚÎÂÈ·

Ù˘ ÚˆÙ½Ó˘ Munc 13-4, Ë ÔÔ›· ÂÌϤÎÂÙ·È Â›-

Û˘ ÛÙÔÓ Ì˯·ÓÈÛÌfi ηٷÛÙÚÔÊ‹˜ (10).

∆¤ÏÔ˜, ÛÙËÓ FHL-1 ¤¯Ô˘Ó ·Ó·ÊÂÚı› ÌÂÙ·ÏÏ¿-

ÍÂȘ ÛÙÔ 9q21.3-22, ÂÓÒ ˘¿Ú¯Ô˘Ó Î·È ÂÚÈÙÒÛÂȘ

ÛÙȘ Ôԛ˜ ‰ÂÓ ·Ó¢ڛÛÎÂÙ·È Î·Ì›· ·fi ÙȘ ·Ú·-

¿Óˆ ÌÂÙ·ÏÏ¿ÍÂȘ. ∏ ÌÔÚÊ‹ ·˘Ù‹ ¯·Ú·ÎÙËÚ›˙ÂÙ·È

ˆ˜ non FHL-2/3, ¯ˆÚ›˜ Ó· ¤¯ÂÈ ‰È¢ÎÚÈÓÈÛı› Ë Û¯¤-

ÛË Ù˘ Ì ÙËÓ FHL-1 ‹ Ì ¿ÏϘ Ӥ˜ ÁÔÓȉȷΤ˜ ÌÂ-

Ù·ÏÏ¿ÍÂȘ (10).

∆ÂÏÂ˘Ù·›·, Á›ÓÂÙ·È ÏfiÁÔ˜ ÁÈ· ÙËÓ FHL-4, fiÔ˘

˘¿Ú¯ÂÈ ÌÂÙ¿ÏÏ·ÍË ÛÙÔ ¯ÚˆÌfiۈ̷ 6q24, ‰ËÏ·‰‹

ÛÙÔ ÁÔÓ›‰ÈÔ Ù˘ Û˘ÓÙ·Í›Ó˘ 11 Ô˘ ÂËÚ¿˙ÂÈ ÙÔ Û‡-

ÛÙËÌ· Ê·ÁÔ΢ÙÙ¿ÚˆÛ˘, Ë ÔÔ›· ÂÓÙÔ›ÛÙËΠÛÂ

ÌÈÎÚ‹ ÔÌ¿‰· ∫Ô‡Ú‰ˆÓ ·ÛıÂÓÒÓ (14).

°ÂÓÈο ÁÈ· ÙÔ ‰Â˘ÙÂÚÔ·ı¤˜ ∏LH

∆Ô ∏LH ·Ó·Ê¤ÚÂÙ·È Î˘Ú›ˆ˜ ÛÙË ‰Â˘ÙÂÚÔ·ı‹

ÌÔÚÊ‹ Ù˘ ÓfiÛÔ˘, fiÔ˘ Ë ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚȷ΋ ÂÈ-

ÎfiÓ· Â›Ó·È ·ÚfiÌÔÈ· Ì Ù˘ FHL, ‰ÂÓ ˘¿Ú¯Ô˘Ó

fï˜ Û¯ÂÙÈΤ˜ ÏËÚÔÊÔڛ˜ ·fi ÙÔ ÔÈÎÔÁÂÓÂÈ·Îfi

ÈÛÙÔÚÈÎfi. ™˘Ó‹ıˆ˜ ÂÌÊ·Ó›˙ÂÙ·È Û ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ-

˘ ·’ fi,ÙÈ Ë FHL Î·È ÌÔÚ› Ó· ·Ó·Î¿Ì„ÂÈ ·˘Ùfi-

Ì·Ù·. ∂›Ó·È ۷ʤ˜ fiÙÈ Û¯ÂÙ›˙ÂÙ·È Ì ÏÔÈÌÒÍÂȘ, Ï‹„Ë

Ê·Ú̿ΈÓ, Û˘ÛÙËÌ·ÙÈΤ˜ ÓfiÛÔ˘˜ (.¯. ™∂§, ¡ƒ∞)

Î·È ÁÂÓÂÙÈο ‹ ·ÎfiÌ· Î·È Î·ÎÔ‹ıË ÓÔÛ‹Ì·Ù·

(1,2,3,13,15,16,17,18,19).

√È ÏÔÈÌÔÁfiÓÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÓÔ¯ÔÔÈÔ‡ÓÙ·È

ÁÈ· ÙËÓ ÚfiÎÏËÛË HLH, ·ÏÏ¿ ÂÓ›ÔÙÂ ˘ÚÔ‰ÔÙÔ‡Ó

Î·È ÂÓÂÚÁÔÔÈÔ‡Ó ÙËÓ FLH, Â›Ó·È Ô EBV (ηٿ ·ÚÈÔ

ÏfiÁÔ), Ô CMV, ¿ÏÏÔÈ ÂÚËÙÔ˚Ô›, Ô Èfi˜ ·ÓÂÌ¢ÏÔÁÈ¿˜-

¤ÚËÙ· ˙ˆÛÙ‹Ú·, Ô Èfi˜ Ù˘ ÈÏ·Ú¿˜, Ù˘ ÂÚ˘ıÚ¿˜, Ô

·Ú‚Ô˚fi˜ µ-19, Ô Èfi˜ Ù˘ Áڛ˘ ∞, ÔÈ ÈÔ› ∂CHO,

Arbo, ηıÒ˜ Î·È ÙÔ Ì˘ÎÔ‚·ÎÙËÚ›‰ÈÔ Ù˘ Ê˘Ì·Ù›ˆ-

Û˘, ÙÔ Ì˘ÎfiÏ·ÛÌ·, Ë ‚ÚԢΤÏÏ·, Ë Û·ÏÌÔÓ¤ÏÏ·

ÙÔ˘ Ù‡ÊÔ˘, Ë ÏÂ˚ÛÌ¿ÓÈ·, Ë ÚÈΤÙÛÈ· Coxiella burnetti

Î·È ‰È¿ÊÔÚÔÈ Ì‡ÎËÙ˜ (9,20,21,22,23,24,25).

∆¤ÏÔ˜, HLH ÌÔÚ› Ó· ÂΉËψı› ‡ÛÙÂÚ· ·fi

HIV Ïԛ̈ÍË ‹/Î·È ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÔÓ ·ÓıÚÒÈÓÔ

ÂÚËÙÔ˚fi 8, ·ÏÏ¿ Î·È Ï›ÁÔ ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘ ·ÓÙÈ-

ÚÂÙÚÔ˚΋˜ ıÂڷ›·˜ ηٿ ÙÔ˘ ∏πV-AIDS (26).

∏ ÏËıÒÚ· ÙˆÓ ˘Â‡ı˘ÓˆÓ ÌÈÎÚÔÔÚÁ·ÓÈÛÌÒÓ

οÓÂÈ ÈÔ Â‡ÎÔÏ· ηٷÓÔËÙfi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Ô ·Ú¯È-

Îfi˜ fiÚÔ˜ (1979) «HLH Û¯ÂÙÈ˙fiÌÂÓÔ Ì ›ˆÛË» (virus-

associated haemophagocytic syndrome, VAHS) ¿Ï-

Ï·Í ·ÚÁfiÙÂÚ· Û «HLH Û¯ÂÙÈ˙fiÌÂÓÔ Ì Ïԛ̈ÍË»

(infection-associated haemophagocytic syndrome,

IAHS). ∆Ô IAHS ÂÌÊ·Ó›˙ÂÈ ıÓËÙfiÙËÙ· ̤¯ÚÈ Î·È 50%

(18,27,28,29).

∆Ô HLH ÌÔÚ› Ó· ÂΉËψı› Î·È ‡ÛÙÂÚ· ·fi

¯ÔÚ‹ÁËÛË Ê·Ú̿ΈÓ, fiˆ˜ ·ÓÙÈÂÈÏËÙÈÎÒÓ (Ê·È-

Ó˘ÙÔ˝ÓË, Ï·ÌÔÙÚÈÁ›ÓË), ·ÓÙÈ‚ÈÔÙÈÎÒÓ (ÁÏ˘ÎÔÂÙ›-

‰È·, ÎÔÙÚÈÌÔÍ·˙fiÏË) Î·È ÂӉ¯Ô̤ӈ˜ ÌÂÊ·ÈÓ·ÌÈ-

ÎÔ‡ ÔͤԘ (2,30,31).

90 Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

Paediatriki 2009;72:90-000

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√È Î·ÎÔ‹ıÂȘ Ì ÙȘ Ôԛ˜ Û˘Ó‰¤ÂÙ·È ÙÔ HLH

·ÊÔÚÔ‡Ó Î˘Ú›ˆ˜ ÙÔ ·ÈÌÔÔÈËÙÈÎfi Û‡ÛÙËÌ· (Ï¢¯·È-

̛˜-ÏÂÌÊÒÌ·Ù·) Î·È ÌÔÚ› Ó· ¤ÏıÂÈ ÚÈÓ ‹ ηٿ

ÙË ‰È¿ÚÎÂÈ· Ù˘ ıÂڷ›·˜, Ó· Û¯ÂÙ›˙ÂÙ·È Ì Ïԛ̈-

ÍË ‹ Ó· ¤گÂÙ·È ¯ˆÚ›˜ ÂÌÊ·Ó‹ ÏfiÁÔ (32,33,34,

35,36).

∆Ô HLH, ·ÏÏ¿ Î·È ÙÔ FLH, ¤¯ÂÈ ‰È·ÈÛÙˆı› fiÙÈ

Û¯ÂÙ›˙ÔÓÙ·È Ì ÔÚÈṲ̂Ó˜ ·ÓÔÛÔ·Ó¿ÚÎÂȘ, fiˆ˜

ÙÔ Ê˘ÏÔÛ‡Ó‰ÂÙÔ ÏÂÌÊÔÔÏÏ·Ï·ÛÈ·ÛÙÈÎfi Û‡Ó‰ÚÔ-

ÌÔ (X-linked lymphoproliferative syndrome: XLP),

ÙÔ Û‡Ó‰ÚÔÌÔ Griscelli, ÙÔ Û‡Ó‰ÚÔÌÔ Hermansky-

Pudlak type II, Ë ÓfiÛÔ˜ Kawasaki Î·È ÙÔ Û‡Ó‰ÚÔÌÔ

Chediak- Higashi. ∆¤ÏÔ˜, HLH ÌÔÚ› Ó· ÂΉËψ-

ı› ‡ÛÙÂÚ· ·fi ÌÂÙ·ÌfiÛ¯Â˘ÛË ÓÂÊÚÒÓ-‹·ÙÔ˜ ηÈ

ÌÔÚ› Ó· Û˘Óԉ‡ÂÈ ÏÂÌÊÔ¸ÂÚÏ·ÛÙÈÎfi Û‡Ó-

‰ÚÔÌÔ Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔÓ EBV (37,38,39,40,

41,42).

∞ÓÙ›ÛÙÚÔÊ·, ÙÔ HLH Ô˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙÔÓ EBV

ÌÈÌÂ›Ù·È ‚ÈÔÏÔÁÈο ÙÔ Ï¤Ìʈ̷ ∆, ÂÓÒ ·Ú·ÙËÚ›ٷÈ

Û˘¯Ó¿ ÂͤÏÈÍË ÙÔ˘ HLH Û ϤÌʈ̷ ∆ (5,36).

∂›Û˘, Û ·ÚÎÂÙ¤˜ ÂÓ‰ÔÁÂÓ›˜ ‰È·Ù·Ú·¯¤˜ ÙÔ˘

ÌÂÙ·‚ÔÏÈÛÌÔ‡ ¤¯ÂÈ ÂÚÈÁÚ·Ê› ÎÏÈÓÈ΋ ÂÈÎfiÓ· Ô˘

ÌÔÈ¿˙ÂÈ ÌÂ HLH (43,44).

π‰È·›ÙÂÚÔ˜ ÏfiÁÔ˜ Ú¤ÂÈ Ó· Á›ÓÂÈ ÁÈ· ÙÔ Û‡Ó‰ÚÔ-

ÌÔ ÙˆÓ ÂÓÂÚÁÔÔÈËÌ¤ÓˆÓ Ì·ÎÚÔÊ¿ÁˆÓ (MAS:

macrophage activation syndrome), Ô˘ ·Ó·Ê¤ÚÂÙ·È

ÛÙË ÌÔÚÊ‹ ÂΛÓË ÙÔ˘ ‰Â˘ÙÂÚÔ·ıÔ‡˜ HLH Ô˘

Û¯ÂÙ›˙ÂÙ·È Ì ÓÔÛ‹Ì·Ù· ÙÔ˘ Û˘Ó‰ÂÙÈÎÔ‡ ÈÛÙÔ‡. ∞·-

ÓÙ¿Ù·È ÈÔ Û˘¯Ó¿ ηٿ ÙËÓ ¤Ó·ÚÍË Ù˘ Û˘ÛÙËÌ·ÙÈ-

΋˜ Ó·ÓÈ΋˜ ÚÂ˘Ì·ÙÔÂȉԇ˜ ·ÚıÚ›Ùȉ·˜ (™¡ƒ∞) ηÈ

Û·ÓÈfiÙÂÚ· Ì ¿ÏÏ· ÓÔÛ‹Ì·Ù· ÙÔ˘ Û˘Ó‰ÂÙÈÎÔ‡

ÈÛÙÔ‡ ÛÙ· ·È‰È¿, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤ÓÔ˘ ÙÔ˘ Û˘-

ÛÙËÌ·ÙÈÎÔ‡ ÂÚ˘ıËÌ·ÙÒ‰Ô˘˜ χÎÔ˘ (15).

∏ Û˘¯ÓfiÙËÙ· ÙÔ˘ MAS ÛÙË ™¡ƒ∞ ·Ó¤Ú¯ÂÙ·È ÛÂ

6,7%-13% (45,46,47). ªÔÚ› Ó· ÂΉËψı› ÔÔÙÂ-

‰‹ÔÙ ηٿ ÙËÓ ÔÚ›· Ù˘ ÓfiÛÔ˘, ·Ó Î·È Ë ÏÂÈÔ-

„ËÊ›· ÙˆÓ ·È‰ÈÒÓ ¤¯ÂÈ ÂӉ›ÍÂȘ ÂÓÂÚÁÔ‡ ÓfiÛÔ˘

ηٿ ÙË ‰È¿ÁÓˆÛË ÙÔ˘ MAS. ¢ÂÓ ˘¿Ú¯ÂÈ ÔÌÔʈӛ·

ˆ˜ ÚÔ˜ ÙÔ ·Ó ÙÔ MAS ı· Ú¤ÂÈ Ó· ÂÍÂÙ¿˙ÂÙ·È ˆ˜

ͯˆÚÈÛÙ‹ ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ· ‹ ˆ˜ ÌÔÚÊ‹ ÙÔ˘ ‰Â˘ÙÂ-

ÚÔ·ıÔ‡˜ HLH. £ÂˆÚÂ›Ù·È fiÙÈ ı· ˘¿ÚÍÂÈ ÂÈÙ˘¯‹˜

·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ÌfiÓÔ fiÙ·Ó Á›ÓÂÈ ¯Ú‹-

ÛË ÙˆÓ ‰È·ı¤ÛÈÌˆÓ ıÂڷ¢ÙÈÎÒÓ Û¯ËÌ¿ÙˆÓ Ô˘

˘¿Ú¯Ô˘Ó ÁÈ· ÙÔ HLH (15).

¶·Ú¿ÁÔÓÙ˜ Ô˘ ÌÔÚÔ‡Ó Ó· ˘ÚÔ‰ÔÙ‹ÛÔ˘Ó ÙËÓ

ÂΉ‹ÏˆÛË ÙÔ˘ MAS Û ·ÛıÂÓ›˜ Ì ™¡ƒ∞ Â›Ó·È ‰È¿-

ÊÔÚ˜ ÏÔÈÌÒÍÂȘ (EBV, Coxsackie, Parvo B-19,

Varicella, ∏epatitis A, Salmonella, Pneumocystis

carinii, Enterococcus), Ê¿Ú̷η (·ÛÈÚ›ÓË, ª™∞º,

ÌÂıÔÙÚÂÍ¿ÙË, etanercept, anakinra, ¿Ï·Ù· ¯Ú˘ÛÔ‡,

ÛÔ˘ÏÊ·Û·Ï·˙›ÓË Î.¿.), ηıÒ˜ Î·È Ë ·˘ÙfiÏÔÁË ÌÂÙ·-

ÌfiÛ¯Â˘ÛË ·Ú¯¤ÁÔÓˆÓ Î˘ÙÙ¿ÚˆÓ Û ·ÛıÂÓ›˜ Ì ·Ó-

ıÂÎÙÈ΋ ÌÔÚÊ‹ ™¡ƒ∞ (15,45,48).

∫ÏÈÓÈ΋ ÂÈÎfiÓ·

∏ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ÙÔ˘ HLH (ÚˆÙÔ·ıÔ‡˜ ηÈ

‰Â˘ÙÂÚÔ·ıÔ‡˜) ÂÚÈÏ·Ì‚¿ÓÂÈ Â›ÌÔÓÔ ˘ÚÂÙfi,

Ë·ÙÔÛÏËÓÔÌÂÁ·Ï›·, ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ÂÍ¿ÓıË-

Ì· Î·È ÔÈÎÈÏ›· Ó¢ÚÔÏÔÁÈÎÒÓ Û˘Ìو̿وÓ, fiˆ˜

¢ÂÚÂıÈÛÙfiÙËÙ·, Û·ÛÌÔ‡˜, ·Ú¿Ï˘ÛË ÎÚ·ÓÈ·ÎÒÓ

Ó‡ڈÓ, ·Ù·Í›·, Ó˘ÛÙ·ÁÌfi, ‰˘Û¯¤ÚÂÈ· ÛÙË ‚¿‰ÈÛË,

„˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË, ËÌÈÏËÁ›·, ÙÂÙÚ·ÏË-

Á›·, ÔÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, Â̤ÙÔ˘˜, ·˘¯ÂÓÈ΋ ‰˘-

Ûη̄›· Î·È ÛËÌ›· ·˘ÍË̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›Â-

Û˘ (8,49). ∏ Ó¢ÌÔÓÈ΋ Û˘ÌÌÂÙÔ¯‹ Â›Ó·È Â›Û˘

Û˘¯Ó‹ (4).

∂ȉÈÎfiÙÂÚ· ÁÈ· ÙËÓ ÂÈÓ¤ÌÂÛË ÙÔ˘ ∫¡™ Û ¿-

Û¯ÔÓÙ˜ ·fi HLH, Ú¤ÂÈ Ó· ˘ÔÁÚ·ÌÌÈÛÙ› fiÙÈ Ù·

Ó¢ÚÔ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· Â›Ó·È ‰‡ÛÎÔÏÔ Ó·

·ÍÈÔÏÔÁËıÔ‡Ó, ΢ڛˆ˜ ·Ó Ë ‰È¿ÁÓˆÛË Á›ÓÂÈ ÌÂÙ¿ ÙÔ

ı¿Ó·Ùfi ÙÔ˘˜. ∂ÈϤÔÓ, Û οÔÈÔ˘˜ ·fi ·˘ÙÔ‡˜

ÙÔ˘˜ ·ÛıÂÓ›˜, Ù· Â˘Ú‹Ì·Ù· ÛÙÔÓ ÂÁΤʷÏÔ Â›¯·Ó

·Ô‰oı› Û ÚÔÛ‚ÔÏ‹ ·fi οÔÈÔÓ Èfi ‹ Û ÌË ÂÈ-

‰È΋ ÌËÓÈÁÁÔÂÁÎÂÊ·Ï›Ùȉ· (8). ∏ Û˘¯ÓfiÙËÙ· ÂÌÊ¿-

ÓÈÛ˘ ‰È·Ù·Ú·¯ÒÓ ·fi ÙÔ ∫¡™ ·Ó¤Ú¯ÂÙ·È Û 20-

100% (8). ªÔÚ› Ó· ·Ú·ÙËÚËıÔ‡Ó ·ÈÌÔÚÚ·Á›Â˜

ÛÙÔÓ ·ÌÊÈ‚ÏËÛÙÚÔÂȉ‹, Û·ÛÌÔ›, ÎÒÌ·, ‰ËÏ·‰‹

ÛÙÔȯ›· Ô˘ ‰˘ÓËÙÈο Ô‰ËÁÔ‡Ó ÛÙËÓ ÂÛÊ·Ï̤ÓË

‰È¿ÁÓˆÛË Ù˘ ηÎÔÔ›ËÛ˘, ÚÔηÏÒÓÙ·˜ ‰˘ÛÌÂ-

Ó›˜ Û˘Ó¤ÂȘ ÁÈ· ÙËÓ ÔÈÎÔÁ¤ÓÂÈ·, ·ÏÏ¿ ΢ڛˆ˜ η-

ı˘ÛÙÂÚÒÓÙ·˜ ÂÈΛӉ˘Ó· ÙË ÛˆÛÙ‹ ‰È¿ÁÓˆÛË Î·È

ıÂڷ›· (50).

¢ÂÓ ˘¿Ú¯ÂÈ Û·Ê‹˜ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ÙˆÓ Ó¢-

ÚÔ·ıÔÏÔÁÈÎÒÓ Â˘ÚËÌ¿ÙˆÓ Î·È ÙˆÓ ÈÛÙÔ·ıÔÏÔÁÈ-

ÎÒÓ ‚Ï·‚ÒÓ Û ¿ÏÏ· fiÚÁ·Ó·, ÂÓÒ ¤¯ÂÈ ÂÚÈÁÚ·Ê›

ÂÚ›ÙˆÛË HLH Ì ·ÔÎÏÂÈÛÙÈ΋ ÂÓÙfiÈÛË ÛÙÔ

∫¡™ (8,11).

¶·ıÔÊ˘ÛÈÔÏÔÁ›·

√È ˘Â‡ı˘ÓÔÈ ·ıÔÊ˘ÛÈÔÏÔÁÈÎÔ› Ì˯·ÓÈÛÌÔ› ‰ÂÓ

Â›Ó·È ·Ôχو˜ ÁÓˆÛÙÔ› Î·È Î·Ù·ÓÔËÙÔ›, ¤¯Ô˘Ó ·Ô-

Î·Ï˘Êı› fï˜ ÔÏϤ˜ ·fi ÙȘ ·ÏÏ·Á¤˜ Ô˘ Û˘Ì-

‚·›ÓÔ˘Ó Û ΢ÙÙ·ÚÈÎfi Î·È ÌÔÚÈ·Îfi Â›Â‰Ô (12).

£ÂˆÚÂ›Ù·È Û‡Ó‰ÚÔÌÔ ˘ÂÚÊÏÂÁÌÔÓ‹˜, fiˆ˜ Û·-

ÊÒ˜ ÚÔ·ÙÂÈ ·fi ÙË ‰È·›ÛÙˆÛË ˘ÂÚ΢ÙÙ·ÚÔÎÈ-

Ó·ÈÌ›·˜, ηıÒ˜ Î·È ÙËÓ ·‡ÍËÛË ¿ÏÏˆÓ ÚÔÊÏÂÁÌÔ-

Óˆ‰ÒÓ ÌÂÛÔÏ·‚ËÙÒÓ, fiˆ˜ Ô ·Ú¿ÁÔÓÙ·˜ Ó¤ÎÚˆ-

Û˘ ÙˆÓ fiÁÎˆÓ (TNF-·), ÔÈ ÈÓÙÂÚÏ¢ΛÓ˜ 6-8-12-18

(IL-6, IL-8, IL-12, IL-18 Î.¿.), Ô ‰È·Ï˘Ùfi˜ ˘Ô‰Ô¯¤·˜

Ù˘ ÈÓÙÂÚÏ¢ΛÓ˘ 2 (sCD25), Ë ÊÏÂÁÌÔÓ҉˘ Úˆ-

Ù½ÓË ÙˆÓ Ì·ÎÚÔÊ¿ÁˆÓ (macrophage inflammatory

protein: MIP-·), Ë ÈÓÙÂÚÊÂÚfiÓË Á (INF-Á) Î·È Ï‹-

ıÔ˜ ·ÈÌÔÔÈËÙÈÎÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Ô˘

·ÂÏ¢ıÂÚÒÓÔÓÙ·È ·fi ÂÓÂÚÁÔÔÈË̤ӷ ÏÂÌÊÔ·Ù-

Ù·Ú· Î·È ÈÛÙÈÔ·ÙÙ·Ú· Û ÔÏÏ¿ fiÚÁ·Ó· (51-52).

™ËÌ·ÓÙÈÎfi˜ ÚfiÏÔ˜ ·Ô‰›‰ÂÙ·È Î·È Û ·ıÔÏÔ-

ÁÈΤ˜ ÚˆÙ½Ó˜ Ô˘ Έ‰ÈÎÔÔÈÔ‡ÓÙ·È ·fi Ù·

91∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË

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ÌÂÙ·ÏÏ·Á̤ӷ ÁÔÓ›‰È· Ù· ÔÔ›· ˘¿Ú¯Ô˘Ó Û ¿ÙÔÌ·

Ì FHL, ·ÏÏ¿ Î·È ÛÙÔ HLH, Ì ΢ÚÈfiÙÂÚÔ ÂÎÚfiÛˆ-

Ô ÙËÓ ÂÚÊÔÚ›ÓË.

∏ INF-Á ·ÚÔ˘ÛÈ¿˙ÂÈ ·ÚÎÂÙ¿ „ËÏfiÙÂÚ˜ Û˘ÁÎÂ-

ÓÙÚÒÛÂȘ ÛÙÔ Ï¿ÛÌ· ηٿ ÙËÓ ÂÓÂÚÁfi Ê¿ÛË Ù˘ Ófi-

ÛÔ˘, ·fi fi,ÙÈ Û ·ÛıÂÓ›˜ Ì ÛË„·ÈÌ›· ‹ ·˘ÙÔ¿ÓÔÛ˜

·ı‹ÛÂȘ. √È Î˘ÙÙ·ÚÔΛÓ˜ ¢ı‡ÓÔÓÙ·È ÁÈ· ÙȘ ·Ú·-

ÙËÚÔ‡ÌÂÓ˜ ηٷÛÙ¿ÛÂȘ Ù˘ ÓfiÛÔ˘, fiˆ˜ ÔÈ Î˘ÙÙ·-

ÚÔÂӛ˜, Ë ˘ÂÚÙÚÈÁÏ˘ÎÂÚȉ·ÈÌ›· Î·È ÔÈ ‰È·Ù·Ú·¯¤˜

Ù˘ ‹Í˘. ∏ ÊÏÂÁÌÔÓ҉˘ ΢ÙÙ·ÚÔΛÓË IL-1‚ ‰ÂÓ

Â›Ó·È ·˘ÍË̤ÓË ÛÙȘ ÎÏ·ÛÈΤ˜ ÂÚÈÙÒÛÂȘ HLH,

ÂÓÒ ·ÓÙÈı¤Ùˆ˜ Ôχ ·˘ÍË̤ӷ ›‰· sCD-25

(>10000 U/ml) ‰ËÏÒÓÔ˘Ó ‰˘ÛÌÂÓ‹ ÚfiÁÓˆÛË ÁÈ·

fiϘ ÙȘ ηÙËÁÔڛ˜ ·ÛıÂÓÒÓ (13,10,60).

∏ ÊÂÚÚÈÙ›ÓË, ›Û˘ ÛËÌ·ÓÙÈÎfi˜ ‰Â›ÎÙ˘, ·˘Í¿-

ÓÂÙ·È ÏfiÁˆ ÙÔ˘ ÔÍÂȉˆÙÈÎÔ‡ stress Î·È ÌÔÚ› Ó·

Û¯ÂÙ›˙ÂÙ·È Ì ·ÓÙÈÛÙ·ıÌÈÛÙÈ΋ ·ÓÔÛÔÏÔÁÈ΋ ·¿-

ÓÙËÛË ÛÙÔ HLH.

∏ ·ÓÂͤÏÂÁÎÙË ÂÓÂÚÁÔÔ›ËÛË ÙˆÓ ·ÓÔÛÔÏÔÁÈÎÒÓ

΢ÙÙ¿ÚˆÓ Á›ÓÂÙ·È Èı·ÓÒ˜ ÏfiÁˆ ·Ó·ÔÙÂÏÂÛÌ·ÙÈ-

΋˜ ·ÓÔÛÔÏÔÁÈ΋˜ ·¿ÓÙËÛ˘ Ô˘ ˘ÚÔ‰ÔÙ›ٷÈ

·fi Â͈ÁÂÓ‹ ÂÚÂı›ÛÌ·Ù·, fiˆ˜ Â›Ó·È ÔÈ ÏÔÈÌÔÁfiÓÔÈ

ÌÈÎÚÔÔÚÁ·ÓÈÛÌÔ› Î·È ÔÈ ÙÔ͛Ә, ‹ ·fi ÂÓ‰ÔÁÂÓ›˜

·Ú¿ÁÔÓÙ˜, fiˆ˜ ÙÔ ÔÍÂȉˆÙÈÎfi stress, Ë ÈÛÙÈ΋ η-

Ù·ÛÙÚÔÊ‹ ‹ ÚÔ˚fiÓÙ· ÙÔ˘ ÌÂÙ·‚ÔÏÈÛÌÔ‡ (13).

∆Ô ˘ÔΛÌÂÓÔ ·ÓÔÛÔÏÔÁÈÎfi ¤ÏÏÂÈÌÌ· Ô˘ Û˘Ì-

‚¿ÏÏÂÈ ÛÙËÓ ·Ó¿Ù˘ÍË HLH ÌÔÚ› Ó· Â›Ó·È ÎÏËÚÔ-

ÓÔÌÔ‡ÌÂÓË ‰È·Ù·Ú·¯‹ ÙˆÓ ·ÓÔÛÔ˚ηÓÒÓ Î˘ÙÙ¿ÚˆÓ

‹ ›ÎÙËÙË, fiˆ˜ .¯. È·ÙÚÔÁÂÓ¤˜ ·ÓÔÛÔÏÔÁÈÎfi ¤Ï-

ÏÂÈÌÌ· ηÙfiÈÓ ÌÂÙ·ÌfiÛ¯Â˘Û˘ ‹ ΢ÙÙ·ÚÔÙÔÍÈ΋˜

ıÂڷ›·˜. ∆Ô Â›ÎÙËÙÔ ¤ÏÏÂÈÌÌ· ÛÙËÓ ·ÓÔÛ›· ÌÔ-

Ú› ›Û˘ Ó· Â›Ó·È ·ÔÙ¤ÏÂÛÌ· Ïԛ̈͢ ÙˆÓ ·ÓÔ-

ÛÔ˚ηÓÒÓ Î˘ÙÙ¿ÚˆÓ ‹ Ó· ÚÔηÏÂ›Ù·È ¤ÌÌÂÛ· ̤ۈ

·ÓÔÛÔηٷÛÙ·ÏÙÈ΋˜ ‰Ú¿Û˘ ÙˆÓ ÊÏÂÁÌÔÓˆ‰ÒÓ ÌÂ-

ÛÔÏ·‚ËÙÒÓ.

°ÂÓÈο, ÙÔ HLH Â›Ó·È ˘ÂÚÊÏÂÁÌÔÓ҉˜ Û‡Ó‰ÚÔ-

ÌÔ, Ô˘ ˘Ô‰È·ÁÈÁÓÒÛÎÂÙ·È Î·È ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi

˘ÂÚ‰Ú·ÛÙ‹ÚÈ· Ê·ÁÔ·ÙÙ·Ú·, ÌË ·ÔÙÂÏÂÛÌ·ÙÈ΋

¤Ó·ÚÍË Ù˘ ·fiÙˆÛ˘ Î·È ÌÂȈ̤ÓË ‰Ú·ÛÙËÚÈfiÙË-

Ù· ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆ Î·È ¡∫, Ô˘ Â›Ó·È ÌfiÓÈÌË ÛÙÔ˘˜

ÚˆÙÔ·ı›˜ ÁÂÓÂÙÈο Ù‡Ô˘˜ (5,13).

∏ ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ‰Ú¿ÛË ÙˆÓ ¡∫ Î·È Ù˘ ΢Ù-

Ù·ÚÔÙÔÍÈÎfiÙËÙ·˜ ÙˆÓ ∆, Û‡ÌÊ˘ÙË ‹ ›ÎÙËÙË, ÌÔ-

Ú› Ó· Â›Ó·È ÎÔÈÓfi˜ Ì˯·ÓÈÛÌfi˜ ÛÙÔ˘˜ ·ÛıÂÓ›˜ ÌÂ

HLH, Ô˘ Ô‰ËÁ› Û ·ÚfiÌÔȘ ÎÏÈÓÈΤ˜ ÂΉËÏÒ-

ÛÂȘ (13).

¢˘ÛÙ˘¯Ò˜, ÔÈ ÂÍÂÙ¿ÛÂȘ Ô˘ ÌÔÚÔ‡Ó Ó· ·Ôη-

χ„Ô˘Ó ÙÔ˘˜ ÂοÛÙÔÙ ÂÌÏÂÎfiÌÂÓÔ˘˜ Ì˯·ÓÈ-

ÛÌÔ‡˜, Á›ÓÔÓÙ·È ÌfiÓÔ Û ΤÓÙÚ· Ì ˘„ËÏ‹ ÂÍÂȉ›-

΢ÛË Î·È Ù· ·ÔÙÂϤÛÌ·Ù¿ ÙÔ˘˜ Û¿ÓÈ· Â›Ó·È ‰È·-

ı¤ÛÈÌ· ÛÙËÓ ¤Ó·ÚÍË Ù˘ ÓfiÛÔ˘, ÙfiÙ ‰ËÏ·‰‹ Ô˘

Ú¤ÂÈ Ó· ÏËÊıÔ‡Ó ·ÔÊ¿ÛÂȘ ÁÈ· ÙË ıÂڷ›· ηÈ

ÙËÓ ÂÚ·ÈÙ¤Úˆ ·ÓÙÈÌÂÙÒÈÛË ÙÔ˘ ·ÛıÂÓÔ‡˜ (53).

∂ÚÁ·ÛÙËÚȷο - ÈÛÙÔÏÔÁÈο Â˘Ú‹Ì·Ù·

∆· ÂÚÁ·ÛÙËÚȷο Â˘Ú‹Ì·Ù· Ô˘ ˘ÔÛÙËÚ›˙Ô˘Ó

ÙË ‰È¿ÁÓˆÛË ·Ó·Ê¤ÚÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1

(13,28,50). ∏ ÔÌ¿‰· ÌÂϤÙ˘ ÙÔ˘ 1994 (HLH-94)

ıˆÚ› fiÙÈ Ù· ›‰· ÊÂÚÚÈÙ›Ó˘ >500 Ìg/L ‰È·ı¤-

ÙÔ˘Ó ÂȉÈÎfiÙËÙ· 80% ÁÈ· ÙË ‰È¿ÁÓˆÛË HLH. ∂›-

Û˘, ·Ú·ÙËÚÔ‡ÓÙ·È ·˘ÍË̤Ó˜ ÙÈ̤˜ Û ΢ÙÙ·ÚÔ-

ΛÓ˜, INF-Á, ∆¡F-· Î·È ÌÂȈ̤ÓË ‰Ú·ÛÙÈÎfiÙËÙ·

ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¡∫ Î·È ÌÂȈ̤ÓË Î˘ÙÙ·ÚÔÙÔÍÈ΋

‰Ú¿ÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆.

∆· ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙÔ HLH Â›Ó·È ·fiÚ-

ÚÔÈ· ÙÔ˘ ÂÈıÂÙÈÎÔ‡ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡ ÙˆÓ Î·Ù¿ Ù·

¿ÏÏ· Ê˘ÛÈÔÏÔÁÈÎÒÓ ÈÛÙÈÔ΢ÙÙ¿ÚˆÓ Î·È ÏÂÌÊÔ΢ÙÙ¿-

ÚˆÓ ∆ ÛÙÔ˘˜ ‰È¿ÊÔÚÔ˘˜ ÈÛÙÔ‡˜. ∆Ô Ô˘ÛÈ·ÛÙÈÎfiÙÂÚÔ

‰È·ÁÓˆÛÙÈÎfi ‡ÚËÌ· Â›Ó·È Ë ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË

ÙˆÓ ÂÚ˘ıÚÒÓ ·ÈÌÔÛÊ·ÈÚ›ˆÓ, ¿ÏÏˆÓ Ï¢ÎÒÓ ·ÈÌÔ-

ÛÊ·ÈÚ›ˆÓ ‹ ·ÈÌÔÂÙ·Ï›ˆÓ ̤۷ ÛÙÔÓ Ì˘ÂÏfi ÙˆÓ

ÔÛÙÒÓ, ÛÙÔÓ ÛÏ‹Ó· Î·È ÛÙÔ˘˜ ÏÂÌÊ·‰¤Ó˜, Ô˘

ÌÔÚ› Ó· ÌËÓ Â›Ó·È ÂÌÊ·Ó‹˜ ·fi ÙËÓ ·Ú¯‹ Î·È Ó·

¯ÚÂÈ·ÛÙÔ‡Ó Â·Ó·Ï·Ì‚·ÓfiÌÂÓ˜ ÂÍÂÙ¿ÛÂȘ ÙˆÓ

ÈÛÙÒÓ ·˘ÙÒÓ. ™Â ·ÛıÂÓ›˜ ·fi ÙË °ÂÚÌ·Ó›·, ·ÈÌÔ-

Ê·ÁÔ΢ÙÙ¿ÚˆÛË ‹Ù·Ó ·ÚÔ‡Û· ÌfiÓÔ ÛÙÔ 32% ÙˆÓ

92 Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

Paediatriki 2009;72:16-22

¶›Ó·Î·˜ 1. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ·ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋˜ ÏÂÌÊÔ˚-ÛÙÈÔ΢ÙÙ¿ÚˆÛ˘ (28)

1. ªÔÚȷ΋ ‰È¿ÁÓˆÛË Û˘Ì‚·Ù‹ Ì ∞º™2. ¢È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ· ∞º™: Ú¤ÂÈ Ó· ÏËÚÔ‡ÓÙ·È 5 ‹

ÂÚÈÛÛfiÙÂÚ· ·fi Ù· ·ÎfiÏÔ˘ı· 8: i) ¶˘ÚÂÙfi˜

ii) ™ÏËÓÔÌÂÁ·Ï›·iii) ∫˘ÙÙ·ÚÔÂӛ˜ (Ó· ÂËÚ¿˙ÔÓÙ·È ≥2 ΢ÙÙ·ÚÈΤ˜

ÛÂÈÚ¤˜ ÛÙÔ ÂÚÈÊÂÚÈÎfi ·›Ì·) - ∞ÈÌÔÛÊ·ÈÚ›ÓË <90 g/L (ÓÂÔÁÓ¿ <4 ‚‰ÔÌ¿‰ˆÓ:

·ÈÌÔÛÊ·ÈÚ›ÓË <100 g/L)- ∞ÈÌÔÂÙ¿ÏÈ· <100x109/L- √˘‰ÂÙÂÚfiÊÈÏ· <1,0x109/L

iv) ÀÂÚÙÚÈÁÏ˘ÎÂÚȉ·ÈÌ›· ηÈ/‹ ˘Ô˚Óˆ‰ÔÁÔÓ·ÈÌ›·:- ∆ÚÈÁÏ˘ÎÂÚ›‰È· Ó‹ÛÙˆ˜ ≥265 mg/dl- πÓˆ‰ÔÁfiÓÔ ≤1,5 g/L

Ó) ∞ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ÛÂ: Ì˘ÂÏfi ÔÛÙÒÓ, ÛÏ‹Ó·, ÏÂÌÊ·‰¤Ó˜ ‹ ∂¡À: ¯ˆÚ›˜ ÂӉ›ÍÂȘ ηÎÔ‹ıÂÈ·˜

vi) ∂Ï·Ùو̤ÓË ‹ Ï‹Ú˘ ¤ÏÏÂÈ„Ë ‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÙˆÓ΢ÙÙ¿ÚˆÓ ¡∫ (Û‡Ìʈӷ Ì ÙȘ ÙÈ̤˜ ·Ó·ÊÔÚ¿˜ ÙÔ˘ÂοÛÙÔÙ ÂÚÁ·ÛÙËÚ›Ô˘)

vii) ∞˘ÍË̤ӷ ›‰· ÊÂÚÚÈÙ›Ó˘ (≥500 Ìg/L)viii) ¢È·Ï˘Ùfi˜ ˘Ô‰Ô¯¤·˜ Ù˘ ÈÓÙÂÚÏ¢ΛÓ˘ 2 (sCD25)

¿Óˆ ·fi Ù· Ê˘ÛÈÔÏÔÁÈο fiÚÈ· ÁÈ· ÙËÓ ËÏÈΛ·

∂ÈÎÔ˘ÚÈΤ˜ ∂Ӊ›ÍÂȘ:ñ ™˘ÌÙÒÌ·Ù· ∫¡™ Î·È ∂˘Ú‹Ì·Ù· ∂¡À: ·)̤ÙÚÈ· ÏÂÈÔ΢Ù-

Ù¿ÚˆÛË 20-80 ÏÂÌÊÔ·ÙÙ·Ú·/ÌL ENY (΢ڛˆ˜ ∆ ÏÂÌÊÔ-·ÙÙ·Ú·) ‚) ·‡ÍËÛË Ï¢ÎÒÌ·ÙÔ˜ Û 0,5-1 g/L ENY Á) ·ÈÌÔ-Ê·ÁÔ΢ÙÙ¿ÚˆÛË ÛÙ· ÈÛÙÈÔ·ÙÙ·Ú·-Ì·ÎÚÔÊ¿Á· ÙÔ˘ ∂¡À

ñ ∆Ú·ÓÛ·ÌÈÓ·Û·ÈÌ›·ñ ÃÔÏÂÚ˘ıÚÈÓ·ÈÌ›·ñ LDH ÔÚÔ‡ >1000 U/L

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·92

Page 21: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

ÂÚÈÙÒÛÂˆÓ ÛÙËÓ ÚÒÙË Ï‹„Ë Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ

Î·È ÛÙÔ 85% ÙË ÛÙÈÁÌ‹ Ù˘ ‰È¿ÁÓˆÛ˘ (13).

∏ Ë·ÙÈ΋ ·Ó¿ÚÎÂÈ· (ÏfiÁˆ ‰È‹ıËÛ˘ ÙÔ˘ ‹·-

ÙÔ˜ Î·È Ù˘ ‰Ú¿Û˘ ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ) Â›Ó·È Âͤ¯ÔÓ

¯·Ú·ÎÙËÚÈÛÙÈÎfi Û ÔÏÏÔ‡˜ ·ÛıÂÓ›˜, Ô˘ ÌÔÚ›

Ó· ÂÌÊ·Ó›˙Ô˘Ó È‰È·ÈÙ¤Úˆ˜ ·˘ÍË̤ӷ ›‰· Ë·ÙÈ-

ÎÒÓ ÂÓ˙‡ÌˆÓ, ¯ÔÏÂÚ˘ıÚ›Ó˘, ·ÏÏ¿ Î·È ˘ÔÏÂ˘ÎˆÌ·-

ÙÈÓ·ÈÌ›· Î·È ÌÂȈ̤ÓË ‰Ú·ÛÙËÚÈfiÙËÙ· ÔÏÏÒÓ ·fi

ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ ‹Í˘ (54).

∏ ÙÂÎÌËÚ›ˆÛË Ù˘ ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛ˘ ÛÙÔ

ÚÒÙÔ ‰Â›ÁÌ· Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ, Û ÂÚ›Ô˘ 20%

ÙˆÓ ÂÚÈÙÒÛˆÓ, Â›Ó·È ‰‡ÛÎÔÏË ‹ Î·È ·‰‡Ó·ÙË,

fï˜ ÙÔ ÁÂÁÔÓfi˜ ·˘Ùfi ‰ÂÓ ı· Ú¤ÂÈ Ó· ·ÔÙÂÏ›

ÂÌfi‰ÈÔ ÛÙËÓ ¿ÌÂÛË ¤Ó·ÚÍË Ù˘ ıÂڷ›·˜, ÂÊfiÛÔÓ

ÏËÚÔ‡ÓÙ·È Ù· ¿ÏÏ· ‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ·

(13,15,28). ∂›Û˘, Â¿Ó ‰È·ÈÛÙˆı› ÌÂÙ¿ÏÏ·ÍË ÛÙÔ

ÁÔÓ›‰ÈÔ Ù˘ ÂÚÊÔÚ›Ó˘, ÙfiÙ ‰ÂÓ ¯ÚÂÈ¿˙ÔÓÙ·È ¿ÏÏ·

‰È·ÁÓˆÛÙÈο ÎÚÈÙ‹ÚÈ·.

∂ÈϤÔÓ, ˘ÔÛÙËÚ›˙ÂÙ·È fiÙÈ ÁÈ· ÙË ‰È¿ÁÓˆÛË

HLH Ú¤ÂÈ Ó· Û˘Ó˘¿Ú¯ÂÈ Ï‹Ú˘ ¤ÏÏÂÈ„Ë ‰Ú·-

ÛÙËÚÈfiÙËÙ·˜ ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¡∫, ÌÂ Û˘ÁÎÂÓÙÚÒÛÂȘ

ÊÂÚÚÈÙ›Ó˘ >500 Ìg/L Î·È sCD25 >2400 U/ml

(15,28). √ ‰È·Ï˘Ùfi˜ ˘Ô‰Ô¯¤·˜ ·ÈÌÔÛÊ·ÈÚ›Ó˘-

·ÙÔÛÊ·ÈÚ›Ó˘ CD163, Ô˘ Â›Ó·È ÂȉÈÎfi˜ ÁÈ· Ù· Ì·-

ÎÚÔÊ¿Á·, ·ÔÙÂÏ› Ó¤Ô ‰Â›ÎÙË ÁÈ· ÙÔÓ ¤ÏÂÁ¯Ô Ù˘

‰Ú·ÛÙËÚÈfiÙËÙ·˜ ÙÔ˘ HLH. ∆· ›‰¿ ÙÔ˘ Û¯ÂÙ›˙Ô-

ÓÙ·È ÛÙÂÓ¿ Ì ÙË ‰Ú·ÛÙËÚÈfiÙËÙ· Ù˘ ÓfiÛÔ˘ Î·È Â›-

Û˘ Ì ÙË ÊÂÚÚÈÙ›ÓË ÙÔ˘ ÔÚÔ‡.

∏ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ÎÏÈÓÈÎÒÓ Î·È ÈÛÙÔÏÔÁÈÎÒÓ

¢ÚËÌ¿ÙˆÓ Û˘ÓÔ„›˙ÂÙ·È ÛÙÔ˘˜ ¶›Ó·Î˜ 2 Î·È 3 (8).

¶ÚÔÛ‚ÔÏ‹ ÙÔ˘ ∫¡™

ŸÙ·Ó ˘¿Ú¯ÂÈ ÂÈÓ¤ÌÂÛË ÙÔ˘ ∫¡™, ÙÔ Î˘ÚÈfiÙÂÚÔ

ÈÛÙÔ·ıÔÏÔÁÈÎfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi Â›Ó·È Ë ÂÎÙÂٷ̤-

ÓË, ÌË Î·ÎÔ‹ı˘ ‰È‹ıËÛË ·fi ÏÂÌÊÔ·ÙÙ·Ú· ηÈ

Ì·ÎÚÔÊ¿Á·, Ù˘ÈÎfiÙÂÚ· Ì ·ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË,

Û˘Ó‹ıˆ˜ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ‰È‹ıËÛË Î·È Û ¿ÏÏ· fiÚ-

Á·Ó·, fiˆ˜ ÛÏ¿¯Ó·, ÏÂÌÊ·‰¤Ó˜, Ì˘ÂÏfi˜ ÙˆÓ

ÔÛÙÒÓ Î.¿. (55). ªÔÚ› Ó· ˘¿Ú¯ÂÈ ÂÈÓ¤ÌËÛË Î·È

ÙˆÓ ÂÚÈÊÂÚÈÎÒÓ ÓÂ‡ÚˆÓ (8).

∂›Û˘, Â›Ó·È ÂӉȷʤÚÔÓ fiÙÈ ¤¯ÂÈ ‰È·ÈÛÙˆı›

Û˘Û¯¤ÙÈÛË Ù˘ ÎÏÈÓÈ΋˜ ‚·Ú‡ÙËÙ·˜ Ù˘ ÚÔÛ‚ÔÏ‹˜

ÙÔ˘ ∫¡™ Î·È ÙˆÓ Ó¢ÚÔ·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎÒÓ ·Ï-

ÏÔÈÒÛÂˆÓ (8).

∏ ÓfiÛÔ˜ ÙÔ˘ ∫¡™ Ê·›ÓÂÙ·È fiÙÈ Â›Ó·È ÈÔ Û˘¯Ó‹

ÛÙ· ¿ÙÔÌ· Ô˘ ʤÚÔ˘Ó ÙË ÌÂÙ¿ÏÏ·ÍË ªunc 13-4

(FHL-3) (56).

√È ·ÛıÂÓ›˜ Ì ÚÔÛ‚ÔÏ‹ ÙÔ˘ ∫¡™ ÌÔÚÔ‡Ó Ó·

Ù·ÍÈÓÔÌËıÔ‡Ó ˆ˜ ¿Û¯ÔÓÙ˜ ›Ù ·fi ÏÂÌÊÔ΢ÙÙ·-

ÚÈ΋ ÌËÓÈÁÁ›Ùȉ·/ÂÁÎÂÊ·Ï›Ùȉ·, fiÔ˘ ‰ÂÓ ˘¿Ú¯ÂÈ

ηӤӷ ¿ÏÏÔ Ó¢ÚÔÏÔÁÈÎfi Û‡Ìو̷ ÂÎÙfi˜ ·fi ÌË-

ÓÈÁÁÈÛÌfi Î·È ·ıÔÏÔÁÈÎfi ∂¡À, ›Ù ·fi «Ó¢ÚÔÏÔÁÈ-

΋ Û˘Ìو̷ÙÔÏÔÁ›·», ‰ËÏ·‰‹ ÔÔÈÔ‰‹ÔÙ Ó¢-

ÚÔÏÔÁÈÎfi Úfi‚ÏËÌ· ‹ Û·ÛÌÔ‡˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ

‰È·Ù·Ú·¯¤˜ ÙÔ˘ ∫¡™ (55).

∆· Â˘Ú‹Ì·Ù· ·fi ÙÔ ∂¡À ÛÙË ÏÂÌÊÔ΢ÙÙ·ÚÈ΋

ÌËÓÈÁÁ›Ùȉ· ÙÔ˘ ∞º™ Û˘ÓÔ„›˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 4.

∂›Û˘, Â›Ó·È Ú‹ÛÈÌË Ë Ì¤ÙÚËÛË ∂¡À ΢ÙÙ·ÚÔΛÓ˘

Î·È ÓÂÔÙÂÚ›Ó˘ ÛÙÔ ∂¡À (8,55).

∆Ô Î˘ÙÙ·ÚÈÎfi ÂÚȯfiÌÂÓÔ ÙÔ˘ ∂¡À ‰ÂÓ Ê·›ÓÂ-

Ù·È fiÙÈ ·ÔÙÂÏ› ȉȷ›ÙÂÚ· ¯Ú‹ÛÈÌÔ ‰Â›ÎÙË Ù˘ ‚·-

Ú‡ÙËÙ·˜ ÂÈÓ¤ÌËÛ˘ ÙÔ˘ ∫¡™, Û ·ÓÙ›ıÂÛË Ì ÙÔ

χΈ̷, ÙÔ ÔÔ›Ô ‹Ù·Ó ȉȷ›ÙÂÚ· ·˘ÍË̤ÓÔ ÛÂ

·È‰È¿ Ì ÈÔ ÛÔ‚·Úfi ÎÏÈÓÈÎfi ÛÙ¿‰ÈÔ (ÛÙ¿‰ÈÔ 3),

ηıÒ˜ Î·È Ì ÙËÓ ÈÔ ÛÔ‚·Ú‹ Ó¢ÚÔ·ıÔÏÔÁÔ·Ó·-

ÙÔÌÈ΋ ÛÙ·‰ÈÔÔ›ËÛË (ÛÙ¿‰ÈÔ πππ) (8). ¶·ÚfiÏ· ·˘-

Ù¿, ÙÔ ∂¡À ÌÔÚ› Ó· Â›Ó·È Ê˘ÛÈÔÏÔÁÈÎfi, ·ÎfiÌ· ηÈ

¶›Ó·Î·˜ 4. ∂˘Ú‹Ì·Ù· ·fi ÙÔ ∂¡À ÛÙË ÏÂÌÊÔ΢ÙÙ·ÚÈ΋ ÌËÓÈÁÁ›-Ùȉ· ÙÔ˘ ∞º™

i) ª¤ÙÚÈ· ÏÂÈÔ΢ÙÙ¿ÚˆÛË: 20-80 ÏÂÌÊÔ·ÙÙ·Ú·/ÌL ∂¡À(΢ڛˆ˜ ÏÂÌÊÔ·ÙÙ·Ú· ∆)

ii) ∞‡ÍËÛË Ù˘ Û˘ÁΤÓÙÚˆÛ˘ ÙÔ˘ Ï¢ÎÒÌ·ÙÔ˜ 0,5-1 g/L∂¡À

iii) ∞ÈÌÔÊ·ÁÔ΢ÙÙ¿ÚˆÛË ÛÙ· ÈÛÙÈÔ·ÙÙ·Ú· Î·È Ì·ÎÚÔÊ¿Á·ÙÔ˘ ∂¡À (Û¿ÓÈÔ Â‡ÚËÌ·, ·ÏÏ¿ ÈÛ¯˘Ú¿ ‰ËψÙÈÎfi ÁÈ· ÙËÓfiÛÔ)

93∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË

¶·È‰È·ÙÚÈ΋ 2009;72:16-22

¶›Ó·Î·˜ 2. ∫ÏÈÓÈ΋ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ Ó¢ÚÔÏÔÁÈÎÒÓ Û˘Ìو̿-ÙˆÓ ÛÙÔ ∫¡™-∞º™

™Ù¿‰ÈÔ 0: ¶Ï‹Ú˘ ¤ÏÏÂÈ„Ë Ó¢ÚÔÏÔÁÈÎÒÓ ÂΉËÏÒÛÂˆÓ ‹ÌË ÂȉÈΤ˜ Ó¢ÚÔÏÔÁÈΤ˜ ·ÓˆÌ·Ï›Â˜

™Ù¿‰ÈÔ 1: ªË ÂȉÈο Ó¢ÚÔÏÔÁÈο ÛËÌ›·, fiˆ˜ Ûʇ˙Ô˘-Û· ËÁ‹ ‹ ˘ÔÙÔÓ›·

™Ù¿‰ÈÔ 2: ∂ȉÈο Ó¢ÚÔÏÔÁÈο ÛËÌ›·, fiˆ˜ ·˘¯ÂÓÈ΋‰˘Ûη̄›·, Û·ÛÌÔ›, ·Ú¤ÛÂȘ

™Ù¿‰ÈÔ 3: ™Ô‚·Ú‹ Ó¢ÚÔÏÔÁÈ΋ ÓfiÛÔ˜, fiˆ˜ ÚÔԉ¢ÙÈ-΋ „˘¯ÔÎÈÓËÙÈ΋ ηı˘ÛÙ¤ÚËÛË ‹ ·ÒÏÂÈ· Û˘-Ó›‰ËÛ˘ (ÎÒÌ·)

¶›Ó·Î·˜ 3. πÛÙÔÏÔÁÈ΋ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ Â˘ÚËÌ¿ÙˆÓ ÛÙÔ ∫¡™-∞º™ (8)

™Ù¿‰ÈÔ 0: ¶Ï‹Ú˘ ¤ÏÏÂÈ„Ë Ó¢ÚÔ-·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎÒÓ¢ÚËÌ¿ÙˆÓ

™Ù¿‰ÈÔ π: ªfiÓÔ ÏÂÙÔÌËÓÈÁÁÈΤ˜ ‰ÈËı‹ÛÂȘ ·fi ÏÂÌÊÔ-·ÙÙ·Ú· Î·È ÈÛÙÈÔ·ÙÙ·Ú· /Ì·ÎÚÔÊ¿Á·. ¶ÂÚÈ-ÛÙ·Ûȷο ÂÚÈ·ÁÁÂȷ΋ ‰È‹ıËÛË.

™Ù¿‰ÈÔ ππ: ◊È· ·ÚÂÁ¯˘Ì·ÙÈ΋ Û˘ÌÌÂÙÔ¯‹ Ì ÂÓÙÔÓfiÙÂ-ÚË ÂÚÈ·ÁÁÂȷ΋ ‰È‹ıËÛË, ·ÏÏ¿ Î·È ÂÓÙÔÓfiÙÂ-ÚË ‰È‹ıËÛË ÙˆÓ ÌËÓ›ÁÁˆÓ, Ô˘ ÂÓ›ÔÙ ÂÚÈ-Ï·Ì‚¿ÓÂÈ Î·È ÙÔÓ ˘ÔΛÌÂÓÔ ÂÁÎÂÊ·ÏÈÎfi ÈÛÙfi.

™Ù¿‰ÈÔ πππ: ∂ÈϤÔÓ Ù˘ ¤ÓÙÔÓ˘ Ì·˙È΋˜ ÈÛÙÈ΋˜ ‰È‹ıË-Û˘ ·fi ÏÂÌÊÔ·ÙÙ·Ú· Î·È ÈÛÙÈÔ·ÙÙ·Ú·/Ì·-ÎÚÔÊ¿Á·, ·Ó¢ڛÛÎÂÙ·È Ó¤ÎÚˆÛË ÙÔ˘ ÂÁÎÂÊ·-ÏÈÎÔ‡ ÈÛÙÔ‡ Ì ÛËÌ·ÓÙÈ΋ ·ÛÙÚÔÁÏÔ›ˆÛË Î·ÈÛ¯ÂÙÈο Ï›Á· ·ÊÚÒ‰Ë Ì·ÎÚÔÊ¿Á·. ªÔÚ› Ó·˘¿Ú¯Ô˘Ó ·ۂÂÛÙÒÛÂȘ.

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·93

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ηٿ ÙË ‰È¿ÚÎÂÈ· ÎÏÈÓÈο ÚÔ¯ˆÚË̤Ó˘ ÓfiÛÔ˘ ÙÔ˘

∫¡™ (8).

∞ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù·

ÀÂÚ˯ÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ηٿ ÙËÓ ·Ú¯È΋

ÚÔۤϢÛË ÙˆÓ ·ÛıÂÓÒÓ Â›Ó·È (Ì Êı›ÓÔ˘Û· ÛÂÈ-

Ú¿ Û˘¯ÓfiÙËÙ·˜): ·) ÛÏËÓÔÌÂÁ·Ï›·, ‚) Ë·ÙÔÌÂ-

Á·Ï›·, Á) ·ÛΛÙ˘, ‰) ¿¯˘ÓÛË ÙÔ˘ ÙÔȯÒÌ·ÙÔ˜ Ù˘

¯ÔÏˉfi¯Ô˘ ·ÛÙ˘, Â) ·˘ÍË̤ÓË ÂÚÈ˘Ï·›· ˯Ô-

Á¤ÓÂÈ·, ÛÙ) ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, ˙) Ï¢ÚÈÙÈÎfi ÂÍ›-

‰ÚˆÌ· ηÈ, Û·ÓÈfiÙÂÚ·, Ë) ÔÏϷϤ˜ ˘fi˯˜

ÂÚÈÔ¯¤˜ ÛÙÔÓ ÛÏ‹Ó·, ÓÂÊÚÔÌÂÁ·Ï›·, Ï›ıÔÈ ÛÙË

¯ÔÏˉfi¯Ô ·ÛÙË, ·˘ÍË̤ÓË Ë¯ÔÁ¤ÓÂÈ· ÙÔ˘ ÊÏÔÈÔ‡

ÙˆÓ ÓÂÊÚÒÓ Î·È ¿¯˘ÓÛË ÙÔ˘ ÙÔȯÒÌ·ÙÔ˜ ÙÔ˘ ‰ˆ-

‰Âη‰·ÎÙ‡ÏÔ˘ (12).

™ÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜, Â›Ó·È Èı·Ófi Ó·

˘¿Ú¯Ô˘Ó ΢„ÂÏȉÈΤ˜ Î·È ‰È¿ÌÂÛ˜ ÛÎȤ˜ ̠ϢÚÈ-

ÙÈ΋ Û˘ÏÏÔÁ‹, Û˘¯Ó¿ Ì ٷ¯Â›· ÂͤÏÈÍË Î·È ·Ôηٿ-

ÛÙ·ÛË-χÛË, Ô˘ ÔÊ›ÏÔÓÙ·È Û ‰ÈËı‹ÛÂȘ ΢ڛˆ˜ ÙÔ˘

ÂÚÈ·ÁÁÂÈ·ÎÔ‡, ÙÔ˘ ÂÚÈ‚ÚÔÁ¯ÈÎÔ‡ ÈÛÙÔ‡ Î·È ÙÔ˘ ÂÓ‰Ô-

΢„ÂÏȉÈÎÔ‡ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ (57). ∆· ·Ú·¿Óˆ ¢ڋ-

Ì·Ù· ÌÔÚ› Ó· ·Ú·¤ÌÔ˘Ó Û SARS ‹ ARDS (57).

™ÙËÓ ˘ÔÏÔÁÈÛÙÈ΋ Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·, Ù·

Â˘Ú‹Ì·Ù· ÔÈΛÏÏÔ˘Ó Î·È ÌÔÚ› Ó· Â›Ó·È ·ÎfiÌ· ηÈ

Ê˘ÛÈÔÏÔÁÈο, ·Ú¿ Ù· ¤ÓÙÔÓ· Û˘ÌÙÒÌ·Ù· ·fi ÙÔ

∫¡™ (8). ÕÏÏÔÙ ÌÔÚ› Ó· ¿Ú¯Ô˘Ó ÂӉ›ÍÂȘ ·˘ÍË-

̤Ó˘ ÂÓ‰ÔÎÚ¿ÓÈ·˜ ›ÂÛ˘, ·ÓÒÌ·ÏË ÏÂÙÔÌËÓÈÁÁÈ΋

ÂÓ›Û¯˘ÛË, ÂÛÙȷ΋ Ó¤ÎÚˆÛË Î·È ˘fi˘ÎÓ˜ ÓÂÎÚˆÙÈ-

Τ˜ ÂÚÈÔ¯¤˜, Ì ·ÒÏÂÈ· ·ÚÂÁ¯˘Ì·ÙÈÎÔ‡ fiÁÎÔ˘ ηÈ

·ÙÚÔÊ›· (8,50). ™ÙË Û˘Ó¤¯ÂÈ·, ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó

ÂÚÈÔ¯¤˜ Ì ӤÎÚˆÛË Î·È ÂÛÙȷ΋ ‹ ‰È¿¯˘ÙË ·ÔÌ˘Â-

ϛӈÛË. ∂›Û˘, ÂÚÈÁÚ¿ÊÔÓÙ·È ÂÛÙȷΤ˜ ·ÈÌÔÚÚ·Á›Â˜

Î·È ‰È¿Ù·ÛË ÙÔ˘ ˘ÔÛÎÏËÚ›‰ÈÔ˘ ¯ÒÚÔ˘ (50). ™ÙÔ˘˜

·ÛıÂÓ›˜ Ì ÌËÓÈÁÁ›Ùȉ· ÌfiÓÔ, ÌÔÚ› Ó· ·ÓȯÓ‡ÔÓÙ·È

ÌfiÓÔ ·ÓˆÌ·Ï›Â˜ ÛÙË Ï¢΋ Ô˘Û›· (55).

¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË

∆Ô ∞º™ ÌÔÚ› Ó· ÌÈÌÂ›Ù·È ‰È¿ÊÔÚ· ÓÔÛ‹Ì·Ù·

Î·È ÁÈ’ ·˘Ùfi Ú¤ÂÈ Ó· ‰È·ÊÔÚԉȷÁÓˆÛı› ·fi ÔÏ-

Ï·Ï‹ ·Ó¿ÚÎÂÈ· ÔÚÁ¿ÓˆÓ, ˘ÚÂÙfi ·ÁÓÒÛÙÔ˘ ·È-

ÙÈÔÏÔÁ›·˜, ÛË„·ÈÌ›·, Ë·Ù›Ùȉ·, ÔÏÏ·Ï‹ ÛÎÏ‹-

Ú˘ÓÛË, Û‡Ó‰ÚÔÌÔ Reye, ARDS, SARS, ÔÍ›· ÓÂÎÚˆÙÈ-

΋ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·, ÂÁÎÂÊ·ÏÔ¿ıÂÈ· ÙÔ˘ Leigh, η-

ÎfiËı˜ ÁÏÔ›ˆÌ·, ÔÍ›· ·ÈÌÔÚÚ·ÁÈ΋ Ï¢ÎÔÂÁÎÂÊ·-

Ï›Ùȉ·, ۈ̷ÙÈ΋ ηÎÔÔ›ËÛË, ·ÏÏÂÚÁÈ΋ ÔÚʇڷ,

ÈÛÙÈÔ΢ÙÙ¿ÚˆÛË ·fi ·ÙÙ·Ú· ÙÔ˘ Langerhans, ϤÌ-

ʈ̷ ‹ ¿ÏϘ ηÎÔ‹ıÂȘ (3,11,12,49,50,61).

∞fi Ù· ·Ú·¿Óˆ Á›ÓÂÙ·È Î·Ù·ÓÔËÙfi ÁÈ·Ù› Ë ‰È¿-

ÁÓˆÛË Û˘Ó‹ıˆ˜ ηı˘ÛÙÂÚ› Î·È Á›ÓÂÙ·È Û˘Ó‹ıˆ˜ ÌÂ-

Ù¿ ÙÔ ı¿Ó·ÙÔ.

£Âڷ›·

√ ¿ÌÂÛÔ˜ ÛÙfi¯Ô˜ Ù˘ ıÂڷ›·˜ Â›Ó·È Ë Î·Ù·ÛÙÔ-

Ï‹ Ù˘ ÛÔ‚·Ú‹˜ ˘ÂÚÊÏÂÁÌÔÓ‹˜, Ô˘ ıˆÚ›ٷÈ

˘Â‡ı˘ÓË ÁÈ· Ù· ·ÂÈÏËÙÈο ÁÈ· ÙË ˙ˆ‹ Û˘ÌÙÒÌ·-

Ù·. ¢Â˘ÙÂÚ¢fiÓÙˆ˜, ÛÎÔfi˜ Â›Ó·È Ë Î·Ù·ÛÙÚÔÊ‹ ÙˆÓ

·Û¯fiÓÙˆÓ ·ÓÙÈÁÔÓÔ·ÚÔ˘ÛÈ·ÛÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È

Ë ÂÍÔ˘‰ÂÙ¤ÚˆÛË Ì ÙÔÓ ÙÚfiÔ ·˘Ùfi ÙÔ˘ ÂÚÂı›ÛÌ·ÙÔ˜

ÁÈ· ¤ÓÙÔÓË ·ÏÏ¿ ·Ó·ÔÙÂÏÂÛÌ·ÙÈ΋ ‰Ú·ÛÙËÚÈÔÔ›Ë-

ÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ¡∫ Î·È Î·Ù·ÛÙ·ÏÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

∆. ™ÙȘ ÂÚÈÙÒÛÂȘ Ì ÁÂÓÂÙÈÎfi ˘fiÛÙڈ̷, Ô ·fi-

Ï˘ÙÔ˜ ÛÙfi¯Ô˜ ı· Ú¤ÂÈ Ó· Â›Ó·È Ë ·ÓÙÈηٿÛÙ·ÛË

ÙÔ˘ ÌÂÈÔÓÂÎÙÈÎÔ‡ ·Ì˘ÓÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ·fi ·ÙÙ·-

Ú· Ê˘ÛÈÔÏÔÁÈ΋˜ ·ÓÔÛÔÏÔÁÈ΋˜ ηٿÛÙ·Û˘ (13).

∆Ô 1994, Ë πÛÙÈÔ΢ÙÙ·ÚÈ΋ ∂Ù·ÈÚ›· (Histiocyte

Society), ÍÂΛÓËÛ ÚÔÔÙÈ΋ ıÂڷ¢ÙÈ΋ ¤Ú¢ӷ

Û˘ÓÂÚÁ·Û›·˜ (ÙÔ ÚˆÙfiÎÔÏÏÔ HLH-94), ÌÂ ÛÙfi¯Ô ÙË

‚ÂÏÙ›ˆÛË ÙÔ˘ ÔÛÔÛÙÔ‡ ÂÈ‚›ˆÛ˘ (1,28).

ª¤¯ÚÈ ÙfiÙÂ, Ë ıÓËÙfiÙËÙ· ·ÓÂÚ¯fiÙ·Ó Û >90%. ∆Ô

2004 ÍÂΛÓËÛÂ Ë ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ÚˆÙÔÎfiÏÏÔ˘ HLH-

2004, ÌÈ·˜ Ó¤·˜ ¤Î‰ÔÛ˘ ÙÔ˘ ·Ï·ÈfiÙÂÚÔ˘ (58).

™Ù· ÚˆÙfiÎÔÏÏ· ·˘Ù¿ Û˘Ó‰˘¿˙ÔÓÙ·È Ë ¯ËÌÂÈÔ-

ıÂڷ›· Î·È Ë ·ÓÔÛÔıÂڷ›·, ÌÂ Û˘Ó·ÎfiÏÔ˘ıË

ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ÁÈ· ÙȘ ›ÌÔÓ˜,

˘ÔÙÚÔÈ¿˙Ô˘Û˜ ‹/Î·È ÔÈÎÔÁÂÓ›˜ ÌÔÚʤ˜ (1).

∏ ıÂڷ›· ¤Ó·Ú͢ ÂÚÈÏ·Ì‚¿ÓÂÈ ÙË ¯ÔÚ‹ÁËÛË

‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È ÂÙÔÔÛ›‰Ë˜, Ô˘ ·ÎÔÏÔ˘ıÔ‡-

ÓÙ·È ·fi Û˘Ó¯‹ ıÂڷ›· Ì ΢ÎÏÔÛÔÚ›ÓË ∞, Ì·˙›

Ì ÒÛÂȘ ‰ÂÍ·ÌÂı·˙fiÓ˘ Î·È ÂÙÔÔÛ›‰Ë˜, ÁÈ· ÂÚ›-

Ô˘ 1 ÚfiÓÔ. ∞˘Ù‹ Ë ıÂڷ›· ÌÔÚ› Ó· Â›Ó·È Â·Ú-

΋˜ ÁÈ· ·È‰È¿ ¯ˆÚ›˜ ÂÏÏ›ÌÌ·Ù· ÛÙ· ·ÙÙ·Ú· ¡∫,

¯ˆÚ›˜ ÂӉ›ÍÂȘ HLH ‹ fiÙ·Ó ÙÔ HLH Â›Ó·È Î·ı·Ú¿

·ÔÙ¤ÏÂÛÌ· Ïԛ̈͢, ΢ڛˆ˜ ·fi ÙÔÓ EBV (59).

∏ ÂÙÔÔÛ›‰Ë Â›Ó·È ¤Ó· ÂÍ·ÈÚÂÙÈÎfi Ê¿ÚÌ·ÎÔ ÁÈ·

Ó· ÚÔηϤÛÂÈ ¤Ó·ÚÍË Ù˘ ·fiÙˆÛ˘, Ì ·ÚÂÓ¤Ú-

ÁÂȘ ÙË ÛÔ‚·Ú‹ Ô˘‰ÂÙÂÚÔÂÓ›· ηÈ, Û·ÓÈfiÙÂÚ·, ÙË

‰ËÌÈÔ˘ÚÁ›· ÔÍ›·˜ Ì˘ÂÏÔÁÂÓÔ‡˜ Ï¢¯·ÈÌ›·˜ Î·È Ì˘Â-

ÏÔ‰˘ÛÏ·ÛÙÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ (1).

∏ ‰ÂÍ·ÌÂı·˙fiÓË ‰ÈÂÈÛ‰‡ÂÈ ÛÙÔÓ ·ÈÌ·ÙÂÁÎÂÊ·ÏÈ-

Îfi ÊÚ·ÁÌfi Ì ηϤ˜ ·ÓÙÈÊÏÂÁÌÔÓÒ‰ÂȘ Î·È ÚÔ·Ô-

ÙˆÙÈΤ˜ ȉÈfiÙËÙ˜, ÂÓÒ Ë Î˘ÎÏÔÛÔÚ›ÓË ∞ ÌÂÈÒÓÂÈ

ÙË ‰Ú·ÛÙËÚÈfiÙËÙ· ÙˆÓ Î˘ÙÙ¿ÚˆÓ ∆, Ô˘ Â›Ó·È ·˘ÍË-

̤ÓË ÛÙÔ HLH Î·È ÌÔÚ› Ó· ÌÂÈÒÛÂÈ ÙË Û˘¯ÓfiÙËÙ·

ÙˆÓ ı·Ó·ÙËÊfiÚˆÓ ÏÔÈÌÒÍÂˆÓ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ

ÙËÓ Ô˘‰ÂÙÂÚÔÂÓ›· (1,60).

™ÙË ÁÂÓÈÎfiÙÂÚË ·ÓÙÈÌÂÙÒÈÛË Ù˘ ÓfiÛÔ˘ ¤¯Ô˘Ó

¯ÚËÛÈÌÔÔÈËı› Ï·ÛÌ·Ê·›ÚÂÛË, ·Ê·ÈÌ·ÍÔÌÂÙ¿ÁÁÈ-

ÛË, ·ÈÌԉȇÏÈÛË Î·È ¯ÔÚ‹ÁËÛË ·ÓÔÛÔÛÊ·ÈÚÈÓÒÓ, ÂÓÒ

·ÌÊÈÏÂÁfiÌÂÓË ·Ú·Ì¤ÓÂÈ Ë ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·

Ù˘ ÂÓ‰ÔÚÚ·¯È·›·˜ ¤Á¯˘Û˘ ÌÂıÔÙÚÂÍ¿Ù˘ (3,59).

™ÙȘ ‹È˜ ÌÔÚʤ˜ HLH ·fi ÏÔÈÌ҉˜ ·›ÙÈÔ, ·Ú-

Λ ÌfiÓÔ ·ÓÙÈÏÔÈÌ҉˘ ·ÁˆÁ‹, fiˆ˜ Û ·ÛıÂÓ›˜ ÌÂ

ÏÂ˚ÛÌ·Ó›·ÛË.

∏ ÂÙÂÚfiÏÔÁË ÌÂÙ·ÌfiÛ¯Â˘ÛË ·ÈÌÔÔÈËÙÈÎÒÓ Î˘Ù-

Ù¿ÚˆÓ ·Ú¤¯ÂÈ Ù· ηχÙÂÚ· ıÂڷ¢ÙÈο ·ÔÙÂϤ-

ÛÌ·Ù· (3,11,50,55,56). ∞ӷʤÚÂÙ·È ÂÈ‚›ˆÛË 62% ÛÂ

94 Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

Paediatriki 2009;72:16-22

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·94

Page 23: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

‰È¿ÊÔÚ˜ ÌÂϤÙ˜, ÔÛÔÛÙfi ۯ‰fiÓ ·ÓÔÌÔÈfiÙ˘Ô

(62%±12%) Ì ÙËÓ ÙÚÈÂÙ‹ ÂÈ‚›ˆÛË ·È‰ÈÒÓ (¿-

Û¯ÔÓÙ· ·fi FHL ‹ ›ÌÔÓ˜-˘ÔÙÚÔÈ¿˙Ô˘Û˜ ÌË

ÔÈÎÔÁÂÓ›˜ ÌÔÚʤ˜) Ô˘ ·ÎÔÏÔ‡ıËÛ·Ó ÙÔ HLH-94

ÚˆÙfiÎÔÏÏÔ (1,13). ∫·ıÒ˜ Ë ‰È¿ÁÓˆÛË Û˘¯Ó¿ ηı˘-

ÛÙÂÚ› Î·È Ë ÓfiÛÔ˜ Â›Ó·È ·ÚÎÂÙ¿ ÂÈıÂÙÈ΋, ·ÚÎÂÙÔ›

·ÛıÂÓ›˜ ηٷϋÁÔ˘Ó ÚÈÓ Ï¿‚Ô˘Ó HCT. ∞˘Ù‹ Ë ıÂ-

ڷ›· ·ÊÔÚ¿ ÔÌÔ˙˘ÁÒÙ˜ ÁÈ· ÌÂÙ·ÏÏ¿ÍÂȘ ‰È·Êfi-

ÚˆÓ ÁÔÓȉ›ˆÓ Û¯ÂÙÈο Ì ÙÔ ∞º™, ·ÛıÂÓ›˜ Ô˘

··ÓÙÔ‡Ó ·Ó·ÚÎÒ˜ ÛÙËÓ ·Ú¯È΋ ıÂڷ›· ηÈ

ÂΛÓÔ˘˜ Ì ÓfiÛÔ ÙÔ˘ ∫¡™.

√È ÂÚÈÛÛfiÙÂÚÔÈ ÂȉÈÎÔ› ıˆÚÔ‡Ó fiÙÈ Ë ¿ÌÂÛË

¤Ó·ÚÍË ·ÓÔÛÔ¯ËÌÂÈÔıÂڷ›·˜, ¯ÚËÛÈÌÔÔÈÒÓÙ·˜

ÙÔ˘˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÚԷӷʤÚıËηÓ, ·ÎÔÏÔ˘ıÔ‡-

ÌÂÓË ·fi HCT, Ê·›ÓÂÙ·È ˆ˜ Ë ÌfiÓË ıÂڷ¢ÙÈ΋ ‰È·-

‰Èηۛ· Ô˘ Â›Ó·È ÈηӋ Ó· ·Ó·ÛÙ›ÏÂÈ ÙËÓ ÂͤÏÈÍË Ù˘

ÓfiÛÔ˘ ÙÔ˘ ∫¡™ Î·È Ó· ÂÈʤÚÂÈ ÙËÓ ›·ÛË, ·Ó Á›ÓÂÈ Û¯Â-

ÙÈο ÓˆÚ›˜, ·ÊÔ‡ ÚÒÙ· ÂÈÙ¢¯ı› ‡ÊÂÛË (12,55).

∆ÂÏÂÈÒÓÔÓÙ·˜, ˘ÔÁÚ·ÌÌ›˙ÂÙ·È Ë ·Ó¿ÁÎË ÁÈ· ÙËÓ

¢·ÈÛıËÙÔÔ›ËÛË ÙˆÓ È·ÙÚÒÓ ÚÔ˜ ÙË ÓfiÛÔ Î·È ÙËÓ

¤ÁηÈÚË ‰È¿ÁÓˆÛ‹ Ù˘, ÁÈ·Ù› ηٷϋÁÂÈ Û¯Â‰fiÓ ¿-

ÓÙ· Û ı¿Ó·ÙÔ, ·Ó ‰ÂÓ ¯ÔÚËÁËı› ıÂڷ›·.

µÈ‚ÏÈÔÁÚ·Ê›·

1. Henter JI, Samuelsson-Horne AC, Arico M, Egeler M,

Elinder G, Filipovich A, et al. Treatment of hemo-

phagocytic lymphohistiocytosis with HLH-94 immuno-

therapy and bone marrow transplantation. Blood 2002;100:

2367-2373.

2. Yang Y-C, Jou S-T, Chang Y-H, Liang J-S, Lee W-T.

Hemophagocytic syndrome associated with antiepileptic

drug. Pediatr Neurol 2004;30:358-356.

3. DiCarlo J, Lui W, Frankel L, Howell W, Schiffman J,

Alexander S. The hemophagocytic syndrome: titrating

continuous hemofiltration to the degree of lactic acidosis. J

Pediatr Hematol Oncol 2006;23:599-610.

4. Henter JI, Elinder G, Soder O, Ost A. Incidence in Sweden

and clinical features of familial hemophagocytic lympho-

histiocytosis. Acta Paediatr Scand 1991;80:428-435.

5. van der Woude HJ, van der Werf TS, Verschuuren EAM,

Tamminga RYJ, Rosati S, Meertens JHJM, et al. An 18-

year-old man with rapidly progressive multiorgan failure

after a positive mononucleosis spot test result. Chest

2006;130:291-295.

6. Gurgey A, Secmeer G, Tavil B, Ceyhan M, Kuskonmaz B,

Cengiz B, et al. Secondary hemophagocytic lympho-

histiocytosis in Turkish children. J Pediatr Infect Dis 2005;

24:1116-1117.

7. Farquhar JW, Claireaux AE. Familial hemophagocytic

reticulosis. Arch Dis Child 1952;27:519-525.

8. Henter JI, Nennesmo I. Neurophathologic findings and

neurologic symptoms in twenty-three children with hemo-

phagocytic lymphohistiocytosis. J Pediatr 1997;130:358-365.

9. Henter JI, Ehrnst A, Andersson J, Elinder G. Familial

hemophagocytic lymphohistiocytosis and viral infections.

Acta Paediatr 1993;82:369-372.

10. Ueda I, Ishii E, Morimoto A, Ohga S, Sako M, Imashuku S.

Correlation between phenotypic heterogeneity and gene

mutational characteristics in familial hemophagocytic

lymphohistiocytosis. Pediatr Blood Cancer 2006;46:482-488.

11. Shinoda J, Murase S, Takenaka K, Sakai N. Isolated central

nervous system hemophagocytic lymphohistiocytosis: case

report. Neurosurgery 2005;56:187-190.

12. Schmidt MH, Sung L, Shuckett BM. Hemophagocytic

lymphohistiocytosis in children: abdominal US findings

within 1 week of presentation. Radiology 2004;230:685-689.

13. Janka GE, Schneider EM. Modern management of children

with haemophagocytic lymphohistiocytosis. Br J Haematol

2004;124:4-14

14. Marcenaro S, Gallo F, Martini S, Santoro A, Griffiths GM,

AricÔ M, et al. Analysis of natural killer-cell function in

familial hemophagocytic lymphohistiocytosis (FHL):

defective CD 107a surface expression heralds Munc 13-4

defect and discriminates between genetic subtypes of the

disease. Blood 2006;108:2316-2323.

15. Kelly A, Ramanan AV. Recognition and management of

macrophage activation syndrome in juvenile arthritis.

Curr Opin Rheumatol 2007;19:477-481.

16. Henter JI, Elinder G, Ost A. Diagnostic guidelines for

hemophagocytic lymphohistiocytosis: The FHL Study Group

of the Histiocyte Society. Semin Oncol 1991;18;29-33.

17. Arico M, Janka G, Fischer A, Henter JI, Blanche S, Elinder J,

et al Hemophagic histiocytosis. Report of 122 children from

the International Registry. Leukemia 1996;10:197-203.

18. Reiner AP, Spivak JL. Hematophagic histiocytosis. A

report of 23 new patients and a review of the literature.

Medicine 1988;67:369-388.

19. Clementi R, Emmi L, Maccario R, Liotta F, Moretta L,

Danesino C, et al. Adult onset and atypical presentation of

hemophagocytic lymphohistiocytosis in siblings carrying

PRF1 mutations. Blood 2002;100:2266-2267.

20. Ishida Y, Hiroi K, Tauchi H, Oto Y, Tokuda K, Kida K.

Hemophagocytic lymphohistiocytosis secondary to Myco-

plasma pneumoniae infection. Pediatrics International

2004;46:174-177.

21. McClain K, Gehrz R, Grierson H, Purtilo D, Filipovich A.

Virus-associated histiocytic proliferations in children.

Frequent association with Epstein-Barr virus and

congenital or acquired immunodeficiencies. Am J Pediatr

Hematol Oncol 1988;10:196-205.

22. Chen TL, Wong WW, Chiou TJ. Hematophagocytic

syndrome: an unusual manifestation of acute human

immunodeficiency virus infection. Int J Hematol 2003;78:

450-452.

23. Fardet L, Blum L, Kerob D, Aqbalika F, Galicier L, Dupuy

A, et al. Human herpesvirus 8-associated lymphohistio-

cytosis in human immunodeficiency virus-infected

patients. Clin Infect Dis 2003;37:285-291.

24. Grossman WJ, Radhi M, Schauer D, Gerday E, Grose C,

Goldman FD. Development of hemophagocytic lympho-

histiocytosis in triplets infected with HHV-8. Blood

2005;106:1203-1206.

25. Mou SS, Nakagawa TA, Riemer EC, McLean TW, Hines

MH, Shetty AK. Hemophagocytic lymphohistiocytosis

complicating influenza A infection. Pediatrics 2006;118:

e216-e219.

26. Huang DB, Wu JJ, Hamill RJ. Reactive hemophagocytosis

associated the initiation of highly active antiretroviral

therapy (HAART) in a patient with AIDS. Scand J Infect

Dis 2004;36:516-519.

95∞ÈÌÔÊ·ÁÔ΢ÙÙ·ÚÈ΋ ÏÂÌÊÔ˚ÛÙÈÔ΢ÙÙ¿ÚˆÛË

¶·È‰È·ÙÚÈ΋ 2009;72:16-22

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·95

Page 24: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

27. Risdall RJ, McKenna RW, Nesbit ME, Krivit W, BalfourHH, Simmons RL, et al. Virus-associated hemophagocyticsyndrome: a benign histiocytic proliferation distinct frommalignant histiocytosis. Cancer 1979;44:993-1002.

28. Filipovich AH. Hemophagocytic lymphohistiocytosis andrelated disorders. Cur Opin Allergy Clin Immunol 2006;6:410-415.

29. Janka G, Imashuku S, Elinder G, Schneider M, Henter JL.Infection- and malignancy- associated hematophagocyticsyndromes. Secondary hematophagocytic lymphohistio-cytosis. Hematol/Oncol Clin North Am 1998;12:435-444.

30. Stanley J, Fallon-Pelicci V. Phenytoin hypersensitivityreaction. Arch Dermatol 1978;114:1350-1353.

31. Akiyoshi K, Hamada Y, Yamada H, Kojo M, Izumi T. Acutenecrotizing encephalopathy associated with hemophagocyticsyndrome. Pediatr Neurol 2006;34:315-318.

32. Falini B, Pileri S, De Solas I, Martelli MF, Mason DY,Delson G, et al. Peripheral T-Cell lymphoma associatedwith hemophagocytic syndrome. Blood 1990;75:434-444.

33. Okuda T, Sakamoto S, Deguchi T, Misawa S, Kashima K,Yoshihara T, et al. Hemophagocytic syndrome associatedwith agressive natular killer cell leukemia. Am J Hematol1991;38:321-323.

34. Miyahara M, Sano M, Shibata K, Matsuzaki M, Ibaraki K,Shimamoto Y, et al. B-cell lymphoma-associated hemo-phagocytic syndrome: clinicopathological characteristics.Ann Hematol 2000;79:378-388.

35. Shimazaki C, Inaba T, Nakagawa M. B-cell lymphoma-associated hemophagocytic syndrome. Leuk Lymphoma2000;38:121-130.

36. Chuang HC, Lay JD, Hsieh WC, Su IJ. Pathogenesis andmechanism of disease progression from hemophagocyticlymphohistiocytosis to Epstein-Barr Virus-associated T-celllymphoma: nuclear factor-kappa B pathway as a potentialtherapeutic target. Cancer Sci 2007;98:1281-1287.

37. Ezdinli EZ, Kucuk O, Chedid A, Sinclair TF, Thomas K,Singh S, et al. Hypogammaglobulinemia and hemophago-cytic syndrome associated with lymphoproliferativedisorders. Cancer 1986;57:1024-1037.

38. Janka G , Zur Stadt U. Familial and aquired hemophago-cytic lymphohistiocytosis. Hamatology Am Soc HematolEduc Program 2005:82-8.

39. Enders A, Zieger B, Schwarz K, Yoshimi A, Speckmann C,Knoepfle EM, et al. Lethal hemophagocytic lympho-histiocytosis in Hermansky-Pudlak syndrome type II.Blood 2006;108:81-87.

40. Karras A, Thervet E, Legendre C. Hemophagocytic syndromein renal transplant recipients: report of 17 cases and review ofthe literature. Transplantation 2004;77:238-243.

41. George TI, Jeng M, Berquist W, Cherry AM, Link MP,Arber DA. Epstein-Barr virus-associated peripheral T-celllymphoma and hemophagocytic syndrome arising afterliver transplantation: case report and review of theliterature. Pediatr Blood Cancer 2005;44:270-276.

42. Dufourcq-Lagelouse R, Pastural E, Barrat FJ, Feldmann J,Le Deist F, Fisher A, et al. Genetic basis of hemophagocyticlymphohistiocytosis syndrome. Int J Mol Med 1999;4:127-133.

43. Duval M, Fenneteau O, Doireau V, Faye A, Emilie D,Yotnda P, et al. Intermittent hemophagocytic lympho-histiocytosis is a regular feature of lysinuric proteinintolerance. J Pediatr 1999;134:236-239.

44. Wu S, Gonzalez-Gomez I, Coates T, Yano S. Cobalamin C

disease presenting with hemophagocytic lympho-histiocytosis. Pediatr Hematol Oncol 2005;22:717-721.

45. Sawhney S, Woo P, Murray KJ. Macrophage activationsyndrome: a potentially fatal complication of rheumaticdisorders. Arch Dis Child 2001;85:421-426.

46. Behrens EM, Beukelman T, Paessler M, Cron RQ. Occultmacrophage activation syndrome in patients with systemicjuvenile idiopathic arthritis. J Rheumatol 2007;34:1133-1138.

47. Katoh N, Gono T, Mitsuhashi S, Fukushima K, Takei Y,Matsuda M, et al. Hemophagocytic syndrome associatedwith rheumatoid arthritis. Intern Med 2007;46:1809-1813.

48. Brinkman DM, de Kleer IM, ten Cate R, van Rossum Ma,Bekkering WP, Fasth A, et al. Autologus stem celltransplantation in children with severe progressivesystemic or polyarticular juvenile idiopathic arthritis: long-term follow-up of a prospective clinical trial. ArthritisRheum 2007;56:2410-2421.

49. Henter JI, Elinder G. Cerebromeningeal haemophagocyticlymphohistiocytosis. Lancet 1992;339:104-107.

50. Rooms L, Fitzgerald N, McClain KL. Haemophagocyticlymphohistiocytosis masquerading as child abuse:presentation of three cases and review of central nervoussystem findings in haemophagocytic lymphohistiocytosis.Pediatrics 2003;111:e636-e640.

51. Henter JI, Elinder G, Söder O, Hansson M, Andersson B,Andersson U. Hypercytokinemia in familial hemophagocyticlymphohistiocytosis. Blood 1991;78:2918-2922.

52. Osugi Y, Hara J, Tagawa S, Takai K, Hosoi G, Matsuda Y,et al. Cytokine production regulating Th1 and Th2cytokines in hemophagocytic lymphohistiocytosis. Blood1997;89:4100-4103.

53. Astigarraga I, Prats JM, Navajas A, Fernandez-Teijeiro A,Urberuaga A. Near fatal cerebellar swelling in familialhemophagocytic lymphohistiocytosis. Pediatr Neurol2004;30:361-364.

54. de Kerguenec C, Hillaire S, Molinie V, Gardin C, Deqott C,Erlinger S, et al. Hepatic manifestations of hemophago-cytic syndrome: a study of 30 cases. Am J Gastroenterol2001; 96:852-857.

55. Haddad E, Sulis ML, Jabado N, Blanche S, Fisher A,Tardieu M. Frequency and severity of central nervoussystem lesions in haemophagocytic lymphohistiocytosis.Blood 1997;89:794-800.

56. Imashuku S, Iwai A. Isolated central nervous system haemo-phagocytic lymphohistiocytosis: case report. Neurosurgery2006;58:e590.

57. Ishii H, Asai S, Suzuki T, Eguchi K, Miyachi H. Virus-associated hemophagocytic syndrome in an internationaltraveler as a differential diagnosis of SARS. InternalMedicine 2005;44:342-345.

58. Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH,Imashuku S, et al. HLH-2004: Diagnostic and therapeuticguidelines for hemophagocytic lymphohistiocytosis.Pediatr Blood Cancer 2007;48:124-131.

59. Imashuku S, Hibi S, Todo S. Hemophagocytic lympho-histiocytosis in infancy and childhood. J Pediatr 1997;130:352-357.

60. Imashuku S, Kuriyama K, Teramura T, Ishii E, KinugawaN, Kato M, et al. Requirement for etoposide in thetreatement of Epstein-Barr virus-associated hemophago-cytic lymphohistiocytosis. J Clin Oncol 2001;19:2665-2673.

61. Tardieu M, Mikaeloff Y. Multiple Sclerosis in children. TheInternational MS Journal 2004;11:36-42.

96 Ã. ∫Ô˘ÙÛ·˘Ù›ÎË Î·È Û˘Ó.

Paediatriki 2009;72:16-22

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¡ÂfiÙÂÚ· ‰Â‰Ô̤ӷ ÁÈ· ÙË Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

Ã. ∆۷ηϛ‰Ë˜1, ∂. √˘ÚÁηÓÙ˙fiÁÏÔ˘2, ¡. ¡ÈÎÔÏ·˝‰Ë˜1

¶ÂÚ›ÏË„Ë: ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) ·ÔÙÂÏ› ¤Ó· ÛËÌ·ÓÙÈÎfi Úfi‚ÏËÌ· ÙˆÓ ÓÂÔÁÓÒÓ, Ì¢„ËÏ‹ ÓÔÛËÚfiÙËÙ· Î·È ıÓËÙfiÙËÙ·. ∫·Ù·Ù¿ÛÛÂÙ·È Û ‰È¿ÊÔÚÔ˘˜ Ù‡Ô˘˜, ·Ó¿ÏÔÁ· Ì ÙȘ ˘¿Ú¯Ô˘Û˜·Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜. ∏ Û˘¯ÓfiÙËÙ· Ù˘ ™¢∫ Â›Ó·È 1 ·Ó¿ 2.000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ÓÂÔÁÓÒÓ. ∆Ș ÙÂÏ¢-Ù·›Â˜ ‰‡Ô ‰ÂηÂٛ˜, Ù· ÔÛÔÛÙ¿ ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ¤¯Ô˘Ó ·˘ÍËı›, Ë ·ıÔÊ˘ÛÈÔÏÔÁ›· Ù˘ ™¢∫¤¯ÂÈ Î·Ù·ÓÔËı› ηχÙÂÚ· Î·È Ë ÎÏÈÓÈ΋ ·ÓÙÈÌÂÙÒÈÛË ¤¯ÂÈ ‚ÂÏÙȈı›. ∆· ÓÂÔÁÓ¿ Ì ™¢∫ Û˘¯Ó¿ ¤¯Ô˘Ó ·Ó¿-ÁÎË ÂÓÙ·ÙÈ΋˜ ıÂڷ›·˜ Î·È ·Ú·ÙÂٷ̤Ó˘ ÓÔÛËÏ›·˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË Î·È ÂÌÊ·Ó›˙Ô˘Ó Û˘Ó˘¿Ú¯Ô˘-Û˜ ·ÓˆÌ·Ï›Â˜. √È ÛËÌ·ÓÙÈÎfiÙÂÚÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘ Â›Ó·È ˘Â‡ı˘ÓÔÈ ÁÈ· ÙËÓ Î·Î‹ ÚfiÁÓˆÛË Â›Ó·È Ë˘ÔÏ·Û›· ÙˆÓ Ó¢ÌfiÓˆÓ Î·È Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙÔ˘˜ È·ÙÚÔÁÂÓ›˜ ÙÚ·˘Ì·ÙÈ-ÛÌÔ‡˜ Ô˘ ÚÔηÏÔ‡ÓÙ·È ·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi. ∂ÓÙÔ‡ÙÔȘ, ÓÂfiÙÂÚ· ‰Â‰Ô̤ӷ Ù˘ ·ıÔÊ˘ÛÈÔÏÔ-Á›·˜ Ù˘ ÓfiÛÔ˘ Î·È ÙˆÓ Ì˯·ÓÈÛÌÒÓ Ô˘ ÂÚÌËÓÂ‡Ô˘Ó ÙËÓ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· ¤¯Ô˘Ó Ô‰ËÁ‹ÛÂÈ ÛÂÛËÌ·ÓÙÈΤ˜ ·ÏÏ·Á¤˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ÓÂÔÁÓÒÓ Ì ™¢∫.

§¤ÍÂȘ ÎÏÂȉȿ: ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÓÂÔÁÓfi, ıÂڷ›·.

Current data on congenital diaphragmatic hernia

C. Tsakalidis1, E. Ourgantzoglou2, N. Nikolaidis1

Abstract: Congenital diaphragmatic hernia (CDH) is considered a serious problem of the newborns,associated with high mortality and morbidity. It can be subdivided into several different types,depending on the affected anatomical structures. The incidence of CDH may be as high as 1 in 2,000of living births. Over the past two decades, antenatal diagnosis rates have increased, thepathophysiology of CDH has become better understood and advances in clinical care have occurred.Infants with CDH often have other congenital anomalies and require intensive treatment after birth,leading to prolonged hospitalisation. The major factors which are associated with poor prognosis arepersistent pulmonary hypertension and lung hypoplasia in combination with iatrogenic injuriescaused by the mechanical ventilation. However, newer data of pathophysiology of the disease andthe mechanisms of the respiratory distress have resulted in improving the treatment of newbornswith CDH.

Key words: Congenital diaphragmatic hernia, neonate, treatment.

97∞¡∞™∫√¶∏™∏ REVIEW ARTICLE

1 µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋,∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ£ÂÛÛ·ÏÔӛ΢,¡ÔÛÔÎÔÌ›ԫ¶··ÁˆÚÁ›Ô˘»,£ÂÛÛ·ÏÔÓ›ÎË

2 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ£ÂÛÛ·ÏÔӛ΢,¡ÔÛÔÎÔÌÂ›Ô «∞Ã∂¶∞»,£ÂÛÛ·ÏÔÓ›ÎË

AÏÏËÏÔÁÚ·Ê›·:

ÃÚ‹ÛÙÔ˜ ∆۷ηϛ‰Ë˜[email protected]µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋,∞ÚÈÛÙÔÙ¤ÏÂÈÔ ¶·ÓÂÈÛÙ‹ÌÈÔ£ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌ›ԫ¶··ÁˆÚÁ›Ô˘»,£ÂÛÛ·ÏÔÓ›ÎË

1 2nd Department ofNeonatal Paediatrics,“Aristotelio” University ofThessaloniki,“Papageorgiou” Hospital,Thessaloniki, Greece

2 2nd Department ofPaediatrics, “Aristotelio”University of Thessaloniki,“AHEPA” Hospital,Thessaloniki, Greece

Correspondence:

Christos [email protected] Department of NeonatalPaediatrics, “Aristotelio”University of Thessaloniki,“Papageorgiou” Hospital,Thessaloniki, Greece

¶·È‰È·ÙÚÈ΋ 2009;72:97-000

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Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·97

Page 26: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

Ì ÙËÓ ·ÚÈÛÙÂÚ‹ Ó· ·Ó¤Ú¯ÂÙ·È ÛÙÔ 73-90%, ÙË ‰ÂÍÈ¿ÛÙÔ 8-24% Î·È ÙËÓ ·ÌÊÔÙÂÚfiÏ¢ÚË ‹ ÎÂÓÙÚÈ΋ ÛÙÔ1,2-4,6% (3-5). √È ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜Ô˘ ‰ÂÓ ÂÓÙÔ›˙ÔÓÙ·È ÛÙ· Ï¿ÁÈ· Â›Ó·È Û¿ÓȘ ηÈÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 1-6% ÙˆÓ ·ÛıÂÓÒÓ. √È ÏÈÁfiÙÂÚÔÛ˘¯ÓÔ› Ù‡ÔÈ ™¢∫ ·ÊÔÚÔ‡Ó ÙËÓ ÔÈÛıÔÛÙÂÚÓÈ΋‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ‹ ΋ÏË ÙÔ˘ Morgagni, ÙËÓ Úfi-ÛıÈ· ‹ ÂÁοÚÛÈ· ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÙËÓ ÎÂÓÙÚÈ΋‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÙËÓ ·Ó‡„ˆÛË ‹ Ï¤Ù˘ÓÛË ÙÔ˘‰È·ÊÚ¿ÁÌ·ÙÔ˜ Î·È ÙËÓ ·ÁÂÓÂÛ›· ·˘ÙÔ‡ (1) (∂ÈÎfiÓ·1). ∫ËÏÈÎfi˜ Û¿ÎÔ˜ ·ÔÙÂÏÔ‡ÌÂÓÔ˜ ·fi ÏÂÙ‹ ÌÂ-ÛÔıËÏȷ΋ ÌÂÌ‚Ú¿ÓË ÂÌÊ·Ó›˙ÂÙ·È Û ÏÈÁfiÙÂÚÔ ·fi10% ÙˆÓ ÂÚÈÙÒÛˆÓ. ™ÙËÓ ·ÚÈÛÙÂÚ‹ ™¢∫, Ù· ÎÔÈ-Ïȷο ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó ÛÙÔÓ ıÒڷη ›-Ó·È ÙÔ ÛÙÔÌ¿¯È, ÙÔ ÏÂÙfi ¤ÓÙÂÚÔ, ÙÔ ÎfiÏÔÓ, Ô ÛÏ‹-Ó·˜ Î·È Ô ·ÚÈÛÙÂÚfi˜ ÏÔ‚fi˜ ÙÔ˘ ‹·ÙÔ˜. ¶ÂÚÈÛÙ·ÛÈ·-ο ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÙÔ ¿ÁÎÚ·˜, Ô ÓÂÊÚfi˜ Î·È ÙÔ·ÚÈÛÙÂÚfi ÂÈÓÂÊÚ›‰ÈÔ. ™ÙË ‰ÂÍÈ¿ ™¢∫, Ôχ ÏÈÁfiÙÂ-ÚÔ ¤ÓÙÂÚÔ ‚Ú›ÛÎÂÙ·È ÛÙÔÓ ıÒڷη, ‰ÈfiÙÈ ÙÔ ‹·Ú·ÔÊÚ¿ÛÛÂÈ ÙÔ ¤ÏÏÂÈÌÌ·.

™˘¯ÓfiÙËÙ·

∏ Û˘¯ÓfiÙËÙ· Ù˘ ™¢∫ ·Ó¤Ú¯ÂÙ·È ·fi 1 ÛÙȘ 2.000¤ˆ˜ 1 ÛÙȘ 4.000 ÁÂÓÓ‹ÛÂȘ ˙ÒÓÙˆÓ ÓÂÔÁÓÒÓ Î·È ·Ô-ÙÂÏ› ÙÔ 8% fiÏˆÓ ÙˆÓ Û˘ÁÁÂÓÒÓ ‰˘ÛÏ·ÛÈÒÓ (6). ∆·ÂÚÈÛÛfiÙÂÚ· ÓÂÔÁÓ¿ Â›Ó·È ÙÂÏÂÈfiÌËÓ·. §›Á˜ ‰ËÌÔ-ÛȇÛÂȘ ·Ó·Ï‡Ô˘Ó ÙË ‰È·ÊÔÚ¿ ʇÏÔ˘ ÛÙË ™¢∫. ∏Û¯¤ÛË ¿ÚÚÂÓ·:ı‹Ï· ˘ÔÏÔÁ›˙ÂÙ·È Û 1:1,25 (7), 3:2(8), ÂÓÒ ¿ÏϘ ‰ËÌÔÛȇÛÂȘ ‰ÂÓ ·Ó·Ê¤ÚÔ˘Ó ‰È·ÊÔ-Ú¿ fiÛÔÓ ·ÊÔÚ¿ ÙÔ Ê‡ÏÔ (9). √È ·Ó·ÊÔÚ¤˜ ÂÚÈÛÙ·ÙÈ-ÎÒÓ ÂÌÊ¿ÓÈÛ˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ Û ‰È‰‡ÌÔ˘˜,Û˘ÁÁÂÓ›˜ Î·È ·ÔÁfiÓÔ˘˜ ÙˆÓ ·ÛıÂÓÒÓ Â›Ó·È ÛÔÚ·-‰ÈΤ˜. ™Â ÔÈÎÔÁ¤ÓÂȘ Ô˘ ·Ú·ÙËÚ‹ıËΠϋÚ˘·ÁÂÓÂÛ›· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ‚Ú¤ıËΠÎÏËÚÔÓÔÌÈÎfi˜·˘ÙÔۈ̷ÙÈÎfi˜ ˘ÔÏÂÈfiÌÂÓÔ˜ ¯·Ú·ÎÙ‹Ú·˜.

™˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜

∂ÎÙfi˜ ·fi ÙȘ ηٷÛÙ¿ÛÂȘ Ô˘ ıˆÚÔ‡ÓÙ·È fiÙÈ·ÔÙÂÏÔ‡Ó Ì¤ÚÔ˜ ÙÔ˘ «Û˘Ó‰ÚfiÌÔ˘ Ù˘ ‰È·ÊÚ·ÁÌ·-ÙÔ΋Ï˘» (Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·, ·ÓÔȯÙfi˜ ·ÚÙË-ÚÈ·Îfi˜ fiÚÔ˜, ·ÓÔȯÙfi ÔÂȉ¤˜ ÙÚ‹Ì· Î·È ·ÙÂÏ‹˜ Û˘-ÛÙÚÔÊ‹ ÙÔ˘ ÂÓÙ¤ÚÔ˘), ÂÚ›Ô˘ ÙÔ 40% ÙˆÓ ˙ÒÓÙˆÓ

ÓÂÔÁÓÒÓ Ì ™¢∫ ¤¯Ô˘Ó Ì›· ‹ ÂÚÈÛÛfiÙÂÚ˜ Û˘ÓÔ‰¤˜

‰È·Ì·Úٛ˜, Û˘¯Ó¿ ˆ˜ ̤ÚÔ˜ ÂÓfi˜ ·Ó·ÁÓˆÚÈṲ̂ÓÔ˘

Û˘Ó‰ÚfiÌÔ˘ ‹ ¯ÚˆÌ·ÙÔÛˆÌÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ (¶›Ó·-

η˜ 1) (10,2). ™ÙÔ 60% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Û˘Ó˘¿Ú-

¯Ô˘Ó ηډȷΤ˜ ‰È·Ì·Úٛ˜, ÂÓÒ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ Ô˘-

ÚÔÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ··ÓÙÔ‡Ó ÛÙÔ 15% ÙˆÓ

ÛÔÚ·‰ÈÎÒÓ Î·È ÛÙÔ 18% ÙˆÓ ÔÈÎÔÁÂÓÒÓ ÂÚÈÙÒÛÂ-

ˆÓ (11). ∞ӈ̷ϛ˜ ÙÔ˘ Á·ÛÙÚÂÓÙÂÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜

ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 17% ÙˆÓ ÂÚÈÙÒÛˆÓ, ÙÔ˘ ∫¡™

ÛÙÔ 14%, ÂÓÒ ÛÙÔ 10% Û˘Ó˘¿Ú¯Ô˘Ó ¯ÚˆÌÔۈ̷ÙÈ-

Τ˜ ·ÓˆÌ·Ï›Â˜. ∆Ô Û‡Ó‰ÚÔÌÔ Ù˘ ˘ÔÏ·ÛÙÈ΋˜ ·ÚÈ-

ÛÙÂÚ‹˜ ηډ›·˜ ·ÚÔ˘ÛÈ¿˙ÂÙ·È Ì ¤Ó· ¢ڇ Ê¿ÛÌ·

·ÓˆÌ·ÏÈÒÓ, Ô˘ Î˘Ì·›ÓÂÙ·È ·fi ÙËÓ ˘ÔÏ·ÛÙÈ΋

·ÚÈÛÙÂÚ‹ ηډȿ ¤ˆ˜ ÙËÓ ·Ó·ÙÔÌÈο Ê˘ÛÈÔÏÔÁÈ΋

ηډȿ, Ë ÔÔ›· fï˜ ÛÙÔ Û‡ÓÔÏfi Ù˘ Â›Ó·È ˘ÔÏ·-

ÛÙÈ΋ (3). ¢ÂÓ ¤¯ÂÈ ‰È¢ÎÚÈÓÈÛÙ› ÁÈ·Ù› Û˘Ì‚·›ÓÂÈ ·˘-

Ùfi. ª›· ıˆڛ· Â›Ó·È fiÙÈ Ë ÂÈÛÙÚÔÊ‹ ÙÔ˘ ÂÌ‚Ú˘˚ÎÔ‡

ÊÏ‚ÈÎÔ‡ ·›Ì·ÙÔ˜ ‰È·Ì¤ÛÔ˘ ÙÔ˘ ˆÔÂȉԇ˜ ÙÚ‹Ì·ÙÔ˜

ÛÙËÓ ·ÚÈÛÙÂÚ‹ ÏÂ˘Ú¿ Ù˘ ηډȿ˜ ÌÔÚ› Ó· ÂÏ·Ù-

Ùˆı› ·fi ÙË ÌÂÙ·ÙfiÈÛË Ù˘ ηډȿ˜ ̤۷ ÛÙÔÓ ıÒ-

ڷη, ÔfiÙÂ Ë ·ÏÏ·Á‹ ÛÙË ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜ ÌÔÚ›

Ó· ÚÔηϤÛÂÈ ˘ÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜

(12). ¢˘ÛÌÔÚÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο Î·È ·ÓˆÌ·Ï›Â˜

ÙˆÓ ¿ÎÚˆÓ Â›Ó·È Û˘¯ÓfiÙÂÚ· ÛÙËÓ ·ÚÈÛÙÂÚ¿ ÂÓÙÔÈ˙fi-

ÌÂÓË ™¢∫ (13). ∆· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, Ë Û˘¯ÓfiÙËÙ·

ÙˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡ Ó¢ÚÈÎÔ‡

Î·È ÙÔ˘ Á·ÛÙÚÂÓÙÂÚÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÌÂÈÒıËΠÛË-

Ì·ÓÙÈο Î·È ·˘Ùfi ÌÔÚ› Ó· ÔÊ›ÏÂÙ·È ÛÙË ‚ÂÏÙ›ˆÛË

ÙÔ˘ ÚÔÁÂÓÓËÙÈÎÔ‡ ÂϤÁ¯Ô˘ Ì ÙË ¯Ú‹ÛË ÙˆÓ ˘ÂÚ‹-

¯ˆÓ. ∏ Ì›ˆÛË, fï˜, ÙˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ÛÙ·

‰‡Ô ·˘Ù¿ Û˘ÛÙ‹Ì·Ù· ÂÈÛËÁÂ›Ù·È Â›Û˘ fiÙÈ Ë Â›Ùˆ-

ÛË ÙˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ¤¯ÂÈ ·ÏÏ¿ÍÂÈ Ù· ÙÂÏ¢-

Ù·›· ¯ÚfiÓÈ· (9).

98 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:98-000

¶›Ó·Î·˜ 1. ∞Ó·ÙÔÌÈΤ˜ ·ÓˆÌ·Ï›Â˜ Ô˘ Û˘ÓÔ‰Â‡Ô˘Ó ÙË ™¢∫

™‡ÛÙËÌ· ∫‡ÚÈ· ·ÓˆÌ·Ï›·

∫·Ú‰È·ÁÁÂÈ·Îfi ªÂÛÔÎÔÏÈ΋ ÂÈÎÔÈÓˆÓ›·ªÂÛÔÎÔÈÏȷ΋ ÂÈÎÔÈÓˆÓ›·™Ù¤ÓˆÛË ·ÔÚÙ‹˜∞ӈ̷ϛ· EbsteinÀÔÏ·ÛÙÈ΋ ·ÚÈÛÙÂÚ‹ ηډ›·

°·ÛÙÚÂÓÙÂÚÈÎfi °·ÛÙÚfiÛ¯ÈÛË√ÌÊ·ÏÔ΋ÏË∞ÙÚËÛ›· ÔÈÛÔÊ¿ÁÔ˘

√˘ÚÔÁÂÓÓËÙÈÎfi À‰ÚÔÓ¤ÊÚˆÛ˶ÔÏ˘Î˘ÛÙÈÎÔ› ÓÂÊÚÔ›

∫¡™ À‰ÚÔÎÂÊ·Ï›·¢Èۯȉ‹˜ Ú¿¯Ë

™ÎÂÏÂÙÔ‡-ÚÔÛÒÔ˘ ŒÏÏÂÈ„Ë Ï¢ÚÒÓ¶ÔÏ˘‰·ÎÙ˘Ï›·™˘Ó‰·ÎÙ˘Ï›·¶ÚÔ¤¯oÓ Ì¤ÙˆÔµÚ·¯˘ÎÂÊ·Ï›·¶Ï·ÁÈÔÎÂÊ·Ï›·

∂ÈÎfiÓ· 1. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË: ·) Ù‡Ô˘ Bochdalec, ‚) Ù‡-Ô˘ Morgagni Î·È ÚfiÛıÈ· ‹ ÂÁοÚÛÈ· ™¢∫, Á) ÎÂÓÙÚÈ΋ ™¢∫.

Ù‡Ô˘ MorgagniÚfiÛıÈ· ‹ ÂÁοÚÛÈ· ™¢∫

· ‚ Á

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·98

Page 27: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

°ÂÓÂÙÈ΋

∏ ™¢∫ ¯ˆÚ›˜ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ÂÌÊ·Ó›˙ÂÙ·ÈÛÔÚ·‰Èο. √ÈÎÔÁÂÓ›˜ ÂÚÈÙÒÛÂȘ ··ÓÙÔ‡Ó ÛÙÔ2% (14,15), Û˘Ó‹ıˆ˜ ‰ÂÓ Û˘Óԉ‡ÔÓÙ·È ·fi ¿ÏÏÂ˜Û˘ÁÁÂÓ›˜ ·ÓˆÌ·Ï›Â˜ Î·È ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚË Û˘¯Ófi-ÙËÙ· ·ÌÊÔÙÂÚfiÏ¢Ú˘ ÂÓÙfiÈÛ˘. √ ΛӉ˘ÓԘ·ÓÂÌÊ¿ÓÈÛ˘ ˘ÔÏÔÁ›˙ÂÙ·È Î·Ù¿ ÚÔÛ¤ÁÁÈÛË ÛÂ2% › ·Ô˘Û›·˜ ÔÈÎÔÁÂÓÂÈ·ÎÔ‡ ÈÛÙÔÚÈÎÔ‡ (16). ∏™¢∫ ÌÔÚ› Ó· ··ÓÙ¿Ù·È ˆ˜ ̤ÚÔ˜ ÂÓfi˜ Û˘Ó‰Úfi-ÌÔ˘ (17). ∆Ô˘Ï¿¯ÈÛÙÔÓ ÙÔ 10% ÙˆÓ ·ÛıÂÓÒÓ Ì ™¢∫Î·È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ·Ó‹ÎÂÈ Û οÔÈÔ ÁÓˆÛÙfiÛ‡Ó‰ÚÔÌÔ (18) (¶›Ó·Î·˜ 2), fiˆ˜ ÙÔ Û‡Ó‰ÚÔÌÔFryns (19,20). ¶Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ÛÙÔ ¯ÚˆÌfiۈ̷1q41-q42.12 ‚Ú›ÛÎÔÓÙ·È ¤Ó· ‹ ÂÚÈÛÛfiÙÂÚ· ÁÔÓ›‰È·Ô˘ ··ÈÙÔ‡ÓÙ·È ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·-ÙÔ˜. ∆· ·ÔÙÂϤÛÌ·Ù· ÁÂÓÂÙÈÎÒÓ ÌÂÏÂÙÒÓ ‰Â›¯ÓÔ˘ÓfiÙÈ ÛÙÔ ÛËÌÂ›Ô Û‡Ó‰ÂÛ˘ ÙˆÓ ÂÚÈÔ¯ÒÓ 1q41 ηÈ1q42.11 ‚Ú›ÛÎÂÙ·È Ô ÁÂÓÂÙÈÎfi˜ ÙfiÔ˜ Ô˘ Â›Ó·È˘Â‡ı˘ÓÔ˜ ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË ™¢∫ Î·È Û˘Ó‰ÚfiÌÔ˘Fryns (19). À¿Ú¯Ô˘Ó ÌÂÌÔӈ̤Ó˜ ·Ó·ÊÔÚ¤˜ ÂÚÈ-ÙÒÛÂˆÓ ™¢∫ Ì ʷÈÓfiÙ˘Ô Ù‡Ô˘ Û˘Ó‰ÚfiÌÔ˘Fryns Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ‰ÈÏ·ÛÈ·ÛÌfi ÙÔ˘ 1q24-q31.2, ÂÍ¿ÏÂÈ„Ë ÙÔ˘ ÙÂÏÈÎÔ‡ ÙÌ‹Ì·ÙÔ˜ ÙˆÓ 6q,8p23.1, 15q26 ¯ÚˆÌ·ÙÔÛˆÌ¿ÙˆÓ Î·È ÌÂÚÈ΋ ÙÚÈÛˆ-Ì›· 22 (21,22,23). √ Ì˯·ÓÈÛÌfi˜ ‰ËÌÈÔ˘ÚÁ›·˜ Ù˘‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ÛÙ· Û‡Ó‰ÚÔÌ· ·˘Ù¿ ·Ú·Ì¤ÓÂÈ·‰È¢ÎÚ›ÓÈÛÙÔ˜. ∞ÚÎÂÙ¿ Û‡Ó‰ÚÔÌ· Ô˘ Û¯ÂÙ›˙ÔÓÙ·ÈÌ ™¢∫ ÂÌÊ·Ó›˙Ô˘Ó ÌÂÓ‰¤ÏÂÈ· ÎÏËÚÔÓÔÌÈÎfiÙËÙ·Î·È Û ÌÂÚÈΤ˜ ÂÚÈÙÒÛÂȘ Ë ÂÚÈÔ¯‹ Ô˘ ‰ڿ˙Ô-ÓÙ·È Ù· ÁÔÓ›‰È· ·˘Ù¿ ‹ Î·È Ë Ù·˘ÙfiÙËÙ· ÙˆÓ ÁÔÓȉ›ˆÓ·˘ÙÒÓ Â›Ó·È ÁÓˆÛÙ¤˜. ∏ ‡·ÚÍË ÁÂÓÂÙÈÎÒÓ Û˘Ó‰Úfi-ÌˆÓ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È Ì ™¢∫ ·ÔÙÂÏ› ÈÛ¯˘Ú‹ ·fi-‰ÂÈÍË fiÙÈ ÛÙËÓ ÂÌÊ¿ÓÈÛ‹ Ù˘ ·›˙Ô˘Ó ÚfiÏÔ ÁÂÓÂÙÈÎÔ›·Ú¿ÁÔÓÙ˜. ∆Ô ÁÔÓ›‰ÈÔ WT1 ·›˙ÂÈ ÚfiÏÔ ÛÙË Ê˘-ÛÈÔÏÔÁÈ΋ ·Ó¿Ù˘ÍË ÙÔ˘ Ô˘ÚÔÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·-ÙÔ˜. ∞fi ÌÂϤÙ˜ Û ÔÓÙ›ÎÈ· ÔÌÔ˙˘ÁÒÙ˜ ÁÈ· ÙȘÌÂÙ·ÏÏ¿ÍÂȘ ÙÔ˘ ÁÔÓȉ›Ô˘ WT1 ‚Ú¤ıËΠfiÙÈ ÔÈ ÌÂ-Ù·ÏÏ¿ÍÂȘ ÙÔ˘ ¢ı‡ÓÔÓÙ·È ÁÈ· ·ÓˆÌ·Ï›Â˜ ÙÔ˘ Ô˘ÚÔ-

ÁÂÓÓËÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, ÙÔ˘ ıˆÚ·ÎÈÎÔ‡ ÙÔȯÒÌ·-

ÙÔ˜ Î·È ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ Ô˘ Ô‰ËÁÔ‡Ó ÛÙË ‰ËÌÈ-

Ô˘ÚÁ›· ™¢∫. ∫¿ÙÈ ·ÓÙ›ÛÙÔÈ¯Ô ‰ÂÓ ·Ô‰Â›¯ıËΠÛÂ

·ÓıÚÒÔ˘˜ (24). À¿Ú¯Ô˘Ó ·˘Í·ÓfiÌÂÓ· ÛÙÔȯ›·

Ô˘ ‰Â›¯ÓÔ˘Ó fiÙÈ ÙÔ Ì·ÎÚ‡ ÛΤÏÔ˜ ÙÔ˘ ¯ÚˆÌ·ÙÔÛÒ-

Ì·ÙÔ˜ 15 ‰È·‰Ú·Ì·Ù›˙ÂÈ ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÂÌÊ¿-

ÓÈÛË ™¢∫ Î·È ÔÚÈÛÌ¤ÓˆÓ Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ. ∏ Â-

ÚÈÔ¯‹ ·˘Ù‹ ÙÔ˘ ¯ÚˆÌ·ÙÔÛÒÌ·ÙÔ˜ 15 Έ‰ÈÎÔÔÈ›

ÙËÓ Î˘ÙÙ·ÚÈ΋ ÚˆÙ½ÓË-1 Ô˘ ‰ÂÛ̇ÂÈ ÙÔ ÚÂÙÈÓÔ˚-

Îfi Ô͇ (25,26,27). √È ÙÚÈۈ̛˜ 13, 18, 21 Î·È 45à ›-

Ó·È ÔÈ ÈÔ Û˘¯Ó¤˜ ·Ó¢ÏÔÂȉ›Â˜ Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ

™¢∫ (9). ∞ӈ̷ϛ˜ fiˆ˜ ÂÍ·Ï›„ÂȘ, ‰ÈÏ·ÛÈ·-

ÛÌÔ›, ·Ó·ÛÙÚÔʤ˜ Î·È ÌÂÙ·ı¤ÛÂȘ ۯ‰fiÓ fiÏˆÓ ÙˆÓ

¯ÚˆÌ·ÙÔÛˆÌ¿ÙˆÓ ¤¯Ô˘Ó ÂÚÈÁÚ·Ê› Û ۯ¤ÛË Ì ÙË

™¢∫ (28,29,18) Î·È ¤¯Ô˘Ó ·Ó·ÁÓˆÚÈÛÙ› ˆ˜ ÛÔ˘‰·›-

Ô˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜, ÌÂ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜

Ô˘ ··ÓÙÔ‡Ó ÛÙÔ 33% ÙˆÓ ÂÚÈÙÒÛÂˆÓ (18). ∫·-

Ú˘fiÙ˘Ô˜ ı· Ú¤ÂÈ Ó· Á›ÓÂÙ·È Û οı ·È‰› ÌÂ

™¢∫ Î·È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ Ô˘ ‰ÂÓ ·ÔÙÂÏÔ‡Ó

‰Â˘ÙÂÚ‡ԢÛ˜ Û˘Ó¤ÂȘ ·˘Ù‹˜.

¶·ıÔÊ˘ÛÈÔÏÔÁ›·

∏ ·ıÔÁ¤ÓÂÈ· Ù˘ ™¢∫ ‰ÂÓ Â›Ó·È ·fiÏ˘Ù· ηٷ-

ÓÔËÙ‹ ¤ˆ˜ Û‹ÌÂÚ·. ∏ ıˆڛ· Ù˘ ·ÔÙ˘¯Ë̤Ó˘ Û‡-

ÁÎÏÂÈÛ˘ Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘¯‹˜ Â›Ó·È Â˘-

Ú¤ˆ˜ ·Ô‰ÂÎÙ‹. ∏ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ·Ú-

¯›˙ÂÈ ·fi ÙÔ ÚfiÛıÈÔ ÙÌ‹Ì· ÙÔ˘, ˆ˜ ¤Ó· ¯ÒÚÈÛÌ· ÌÂ-

ٷ͇ Ù˘ ηډȿ˜ Î·È ÙÔ˘ ‹·ÙÔ˜, Î·È ÚÔ¯ˆÚ¿ÂÈ

ÚÔ˜ Ù· ›Ûˆ ÁÈ· Ó· ÎÏ›ÛÂÈ ÙÂÏÈο ÚÔ˜ ÙÔ ·ÚÈÛÙÂÚfi

ÙÚ‹Ì· ÙÔ˘ Bochdalek, Á‡Úˆ ÛÙËÓ 8Ë Ì 10Ë Â‚‰ÔÌ¿-

‰· ·ËÛ˘. ∆Ô ¤ÓÙÂÚÔ ÌÂÙ·Ó·ÛÙ‡ÂÈ ·fi ÙÔÓ ÏÂÎÈıÈ-

Îfi Û¿ÎÔ ÂÚ›Ô˘ ÙË 10Ë Â‚‰ÔÌ¿‰· ·ËÛ˘ ηÈ, ·Ó

ÊÙ¿ÛÂÈ ÚÈÓ ÙÔ ÎÏ›ÛÈÌÔ ÙÔ˘ ÙÚ‹Ì·ÙÔ˜, ‰ËÌÈÔ˘ÚÁ›-

Ù·È Ë Î‹ÏË. ¶ÈÛÙ‡ÂÙ·È fiÙÈ fiÙ·Ó Û˘Ó˘¿Ú¯ÂÈ ·ÓÒÌ·-

ÏË ÛÙÚÔÊ‹ ÙÔ˘ ÂÓÙ¤ÚÔ˘, ·˘Ù‹, ÏfiÁˆ ı¤Û˘, ‰ÂÓ ÂÈ-

ÙÚ¤ÂÈ ÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ÌÂ Û˘Ó¤-

ÂÈ· ÙË ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË. ¢ÂÓ Â›Ó·È ÁÓˆÛÙfi ÔȘ

·Èٛ˜ Ô‰ËÁÔ‡Ó ÛÙËÓ ·ÔÙ˘¯›· Û‡ÁÎÏÂÈÛ˘ Ù˘ Ï¢-

ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘¯‹˜. ¢Â‰Ô̤ӷ ·fi ¤Ú¢Ó˜ ÛÂ

˙Ò· Ô‰ËÁÔ‡Ó ÛÙËÓ ˘fiıÂÛË ˆ˜ ¤Ó· ÌË Ì˘˚Îfi, ÌÂ-

ÛÂÁ¯˘Ì·ÙÈÎfi ÛÙÔÈ¯Â›Ô Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘-

¯‹˜ Â›Ó·È ÂÏ·Ùو̷ÙÈÎfi Î·È ‰ÂÓ ÚÔ¿ÁÂÈ ÙÔÓ Û¯ËÌ·-

ÙÈÛÌfi ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. ∞˘Ù‹ Ë ˘fiıÂÛË ÂÍËÁ› ÙË

‰È·Ù·Ú·¯‹ ÛÙËÓ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ·ÏÏ¿

‰ÂÓ ‰ÈηÈÔÏÔÁ› ÙȘ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ (9).

√ Aderson ÙÔ 1941 ·Ú·Ù‹ÚËÛ fiÙÈ Ë ·Ó¿Ú-

ÎÂÈ· Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ ·˘Í¿ÓÂÈ ÙËÓ Â›ÙˆÛË Ù˘

™¢∫. ÕÏÏ· ‰Â‰Ô̤ӷ ˘ÔÛÙËÚ›˙Ô˘Ó fiÙÈ ÙÔ Û‡ÛÙËÌ·

Ù˘ ‚ÈÙ·Ì›Ó˘ ∞ Û˘ÌÌÂÙ¤¯ÂÈ ÛÙË ‰ËÌÈÔ˘ÚÁ›· ‰È·-

ÊÚ·ÁÌ·ÙÔ΋Ï˘, Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜ Î·È Î·Ú-

‰È·ÎÒÓ ·ÓˆÌ·ÏÈÒÓ (30,31,26). ªÂϤÙ˜ ÙÔ˘ Major

(1998) ¤‰ÂÈÍ·Ó fiÙÈ ÔÈ ÌËÙ¤Ú˜ Î·È Ù· ÓÂÔÁÓ¿ Ì ™¢∫

›¯·Ó ¯·ÌËÏfiÙÂÚ· ›‰· ÚÂÙÈÓfiÏ˘ ÛÙÔ Ï¿ÛÌ·,

99™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

¶·È‰È·ÙÚÈ΋ 2009;72:99-000

¶›Ó·Î·˜ 2. ™‡Ó‰ÚÔÌ· Ô˘ Û¯ÂÙ›˙ÔÓÙ·È ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·-ÙÔ΋ÏË

ñ ™‡Ó‰ÚÔÌÔ Frynsñ ™‡Ó‰ÚÔÌÔ Beckwith-Wiedemannñ ™‡Ó‰ÚÔÌÔ Brachman-de Langeñ ™‡Ó‰ÚÔÌÔ Simpson-Golabi-Behmelñ ™‡Ó‰ÚÔÌÔ Donnaiñ ™‡Ó‰ÚÔÌÔ Denys-Drashñ ™‡Ó‰ÚÔÌÔ Perlmanñ ™‡Ó‰ÚÔÌÔ Craniofrontonasalñ ™‡Ó‰ÚÔÌÔ Spondylocostalñ ™‡Ó‰ÚÔÌÔ Marfanñ ™‡Ó‰ÚÔÌÔ ™‡Ó‰ÚÔÌÔ Donnai-Barrowñ ™‡Ó‰ÚÔÌÔ Gershoni-Baruchñ ™‡Ó‰ÚÔÌÔ Matthew-Wood

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·99

Page 28: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

Û ۇÁÎÚÈÛË Ì ÂΛӷ ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÌËÙ¤ÚˆÓ

Î·È ÓÂÔÁÓÒÓ (32).

¶ÚfiÛÊ·Ù· Ë ™¢∫ Û¯ÂÙ›ÛÙËΠÁÂÓÂÙÈο Ì ÙÔ˘˜

·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ 10 (FGF-10) Î·È 18 (FGF-

18) Ù˘ ÊÈÌÚÔÏ·ÛÙ›Ó˘, Ô˘ ÂÎÎÚ›ÓÔÓÙ·È ·fi ÙÔ

¿ˆ Ó¢ÌÔÓÈÎfi ÌÂÛ¤Á¯˘Ì·. √ ·Ú¿ÁÔÓÙ·˜ FGF-10

ÚÔ¿ÁÂÈ ÙË ‰È·ÊÔÚÔÔ›ËÛË ÙˆÓ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ

΢ÙÙ¿ÚˆÓ Û ·ÙÙ·Ú· ÙˆÓ Ï›ˆÓ Ì˘˚ÎÒÓ ÈÓÒÓ ÙˆÓ

·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ Ô˘ ·›˙Ô˘Ó ÚfiÏÔ ÛÙË ‰È·Ù‹ÚËÛË

Ù˘ ÂÓ‰Ô·˘ÏÈ΋˜ ›ÂÛ˘ ÙˆÓ ·ÂÚ·ÁˆÁÒÓ Î·Ù¿ ÙËÓ

ÚÒÈÌË Ê¿ÛË Ù˘ ·Ó¿Ù˘Í˘ ÙˆÓ Ó¢ÌfiÓˆÓ Î·È

ÛÙË Ú‡ıÌÈÛË Ù˘ ¢ÂÓ‰ÔÙfiÙËÙ·˜ ÙˆÓ Ó¢ÌfiÓˆÓ

ÛÙ· ÙÂÏÂ˘Ù·›· ÛÙ¿‰È· Ù˘ ·ËÛ˘ (33). ∏ ·Ó¿Ú-

ÎÂÈ¿ ÙÔ˘ ÌÔÚ› Ó· ·˘Í‹ÛÂÈ ÙËÓ Â˘¿ıÂÈ· ÙˆÓ Ó¢-

ÌfiÓˆÓ ÛÙÔÓ ÙÚ·˘Ì·ÙÈÛÌfi ·fi Ù· ÂÓ‰ÔÎÔÈÏȷο

ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó. √ ·Ú¿ÁÔÓÙ·˜ FGF-18

ÚÔ¿ÁÂÈ ÙËÓ ·Ú·ÁˆÁ‹ ÂÏ·ÛÙÈÎÒÓ ÈÓÒÓ Î·È Ë ·ÓÂ-

¿ÚÎÂÈ¿ ÙÔ˘ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ Û ÂÏ·Ùو̤ÓË ·-

Ú·ÁˆÁ‹ ·˘ÙÒÓ (34).

√ Ì˯·ÓÈÛÌfi˜ Ù˘ Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜ ÛÙË

™¢∫ ·ÔÙÂÏ› ÛËÌÂ›Ô ‰È¯ÔÁӈ̛·˜. ™‡Ìʈӷ Ì ÙËÓ

ÈÔ ·Ô‰Â¯Ù‹ ¿Ô„Ë, ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÓ‰ÔÌ‹-

ÙÚÈ·˜ ·Ó¿Ù˘Í˘, Ù· ÂÓÙfi˜ ÙÔ˘ ıÒڷη ÎÔÈÏȷο

ÛÏ¿¯Ó· Ȥ˙Ô˘Ó ÙÔÓ Û‡ÛÙÔÈ¯Ô Ó‡ÌÔÓ·, ÁÂÁÔÓfi˜

Ô˘ ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ Û ÌÈÎÚfiÙÂÚÔ ‚·ıÌfi ηÈ

ÙÔÓ ÂÙÂÚfiÏ¢ÚÔ Ó‡ÌÔÓ·, ÏfiÁˆ Ù˘ ÎÈÓËÙÈÎfiÙË-

Ù·˜ ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘ ÛÙÔ ¤Ì‚Ú˘Ô. ∞˘Ù‹ Ë ¿Ô„Ë

¤¯ÂÈ ÂȂ‚·Èˆı› ÂÈÚ·Ì·ÙÈο Û ÔÓÙÈÎÔ‡˜ ηÈ

·ÌÓÔ‡˜ (35). ™‡Ìʈӷ Ì ÙËÓ ˘fiıÂÛË ÙˆÓ «‰‡Ô

¯Ù˘ËÌ¿ÙˆÓ», ÙÔ «ÚÒÙÔ ¯Ù‡ËÌ·» ¤گÂÙ·È Óˆ-

Ú›˜ ÛÙËÓ ·Ó¿Ù˘ÍË Î·È ÂËÚ¿˙ÂÈ ÂÍ›ÛÔ˘ ÙËÓ ·Ó¿-

Ù˘ÍË ÙˆÓ ‚ÚfiÁ¯ˆÓ Î·È ÙˆÓ ‰‡Ô Ó¢ÌfiÓˆÓ. ∆Ô

«‰Â‡ÙÂÚÔ ¯Ù‡ËÌ·» Û˘Ì‚·›ÓÂÈ ÛÙ· ÙÂÏÂ˘Ù·›· ÛÙ¿‰È·

·Ó¿Ù˘Í˘ Î·È ÚÔ¤Ú¯ÂÙ·È Î˘Ú›ˆ˜ ·fi ÙÔ ‰È¿ÊÚ·Á-

Ì·, Ô˘ Ì·˙› Ì ٷ ÂÓ‰ÔÎÔÈÏȷο ÛÏ¿¯Ó· Ô˘ ÚÔ-

Û›ÙÔ˘Ó ÂËÚ¿˙ÂÈ ÙÔÓ Û‡ÛÙÔÈ¯Ô Ó‡ÌÔÓ·. ∞˘Ùfi

Ô‰ËÁ› Û Ì›ˆÛË ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÎÈÓ‹ÛÂˆÓ ÙÔ˘

ÂÌ‚Ú‡Ô˘ Î·È ÂÏ¿ÙÙˆÛË ÙÔ˘ Ú˘ıÌÔ‡ ·Ú·ÁˆÁ‹˜

Ó¢ÌÔÓÈÎÔ‡ ˘ÁÚÔ‡ (36). ŸÙ·Ó Ù· Û˘ÌÙÒÌ·Ù· ÂÌ-

Ê·Ó›˙ÔÓÙ·È ÓˆÚ›˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, Ë ˘ÔÏ·Û›· ηÈ

ÙˆÓ ‰‡Ô Ó¢ÌfiÓˆÓ Â›Ó·È ¤Ó· ÛÙ·ıÂÚfi ‡ÚËÌ·. ∏

·ÌÊÔÙÂÚfiÏ¢ÚË Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· Â›Ó·È ÛË-

Ì·ÓÙÈο ·˘ÍË̤ÓË Û ·ÛıÂÓ›˜ Ì ‰ÂÍÈ¿ ™¢∫. √È

Ó‡ÌÔÓ˜ ¤¯Ô˘Ó ÂÏ·Ùو̤ÓÔ ·ÚÈıÌfi ‚ÚfiÁ¯ˆÓ, ÏÈ-

ÁfiÙÂÚ· ‚ÚÔÁ¯ÈfiÏÈ· (Ù˘Èο 6-8 ÁÂÓ¤˜ ‚ÚfiÁ¯ˆÓ,

¤Ó·ÓÙÈ ÙˆÓ 15 ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÓÂÔÁ¤ÓÓËÙˆÓ ‚ÚÂ-

ÊÒÓ) Î·È ÂÏ·Ùو̤ÓË Î˘„ÂÏȉÈ΋ ÂÈÊ¿ÓÂÈ· (37). ∏

·ÁÁ›ˆÛË ÙˆÓ Ó¢ÌfiÓˆÓ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ·‡-

ÍËÛË ÙÔ˘ ¿¯Ô˘˜ ÙÔ˘ Ï›Ԣ Ì˘˚ÎÔ‡ ¯ÈÙÒÓ· ÙˆÓ ·Á-

Á›ˆÓ, Ë ÔÔ›· ÂÂÎÙ›ÓÂÙ·È ¤ˆ˜ Î·È ÙÔ Â›Â‰Ô ÙˆÓ

ÙÚȯÔÂȉÒÓ ÙˆÓ Î˘„ÂÏ›‰ˆÓ. √ ·ÚÈıÌfi˜ ÙˆÓ ·ÚÙËÚÈ-

‰›ˆÓ Â›Ó·È Î·Ù¿ ·Ó·ÏÔÁ›· ÂÏ·Ùو̤ÓÔ˜ (38). √È ™¢∫

Ô˘ ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Î·Ù¿ ÙËÓ ÂÓ‰ÔÌ‹ÙÚÈ· Î·È ¿ÌÂ-

ÛË ÌÂÙ·ÁÂÓÓËÙÈ΋ ÂÚ›Ô‰Ô Û¯ÂÙ›˙ÔÓÙ·È Ì Ì›ˆÛË

Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜, ÂÓÒ ·˘Ù¤˜ Ô˘ ÂÌÊ·-

Ó›˙ÔÓÙ·È ·ÚÁfiÙÂÚ· Û¿ÓÈ· Û¯ÂÙ›˙ÔÓÙ·È Ì Ó¢ÌÔÓÈ-

΋ ˘ÔÏ·Û›· (39,40). ∏ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›·

ÂÏ·ÙÙÒÓÂÙ·È ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ ·fi ÙËÓ ·˘Í·Ófi-

ÌÂÓË ›ÂÛË ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›·, Ì ·ÔÙ¤ÏÂ-

ÛÌ· ÙË ‰ÂÍÈÔ-·ÚÈÛÙÂÚ‹ ‰È·Ê˘Á‹ (41). ™Ù· ÓÂÔÁÓ¿

·˘Ù¿, Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Â›Ó·È Û¯Â‰fiÓ Î·ıÔÏÈ-

Îfi ‡ÚËÌ· Î·È Ë ·Ó·Ó¢ÛÙÈ΋ ‹ ÌÂÙ·‚ÔÏÈ΋ Ôͤˆ-

ÛË ÙËÓ ÂȉÂÈÓÒÓÂÈ. ∏ ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË Û˘Ó‹ıˆ˜

ÔÊ›ÏÂÙ·È ÛÙËÓ ÂÏ·Ùو̤ÓË Î·Ú‰È·Î‹ ·ÚÔ¯‹ Ô˘

ÚÔηÏÂ›Ù·È ·fi ÙËÓ ˘ÔÍ›· Î·È ÙËÓ ˘ÔÏ·ÛÙÈ΋

·ÚÈÛÙÂÚ‹ ÎÔÈÏ›· (42,43).

™Â ¿ÙÔÌ· Ì ™¢∫, Ë ‚ÈÔ¯ËÌÈ΋ ·ÓˆÚÈÌfiÙËÙ· ÙˆÓ

Ó¢ÌfiÓˆÓ ¤¯ÂÈ ·ÍÈÔÏÔÁËı› Û Ó‡ÌÔÓ˜ ·Ôı·-

ÓfiÓÙˆÓ ·ÙfiÌˆÓ (44), ÛÙÔ ·ÌÓÈ·Îfi ÁÚfi ηÈ, ÈÔ Úfi-

ÛÊ·Ù·, ÛÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· (45). ™ÙÔÓ

Ó¢ÌÔÓÈÎfi ÈÛÙfi ÓÂÔÁÓÒÓ Ì ™¢∫, Ë Û˘ÁΤÓÙÚˆÛË

ʈÛÊ·ÙȉԯÔÏ›Ó˘ Â›Ó·È ¯·ÌËÏfiÙÂÚË ÛÙ· ÂÚÈÛÛfi-

ÙÂÚ· ¿ÙÔÌ· (44). ™ÙÔ ·ÌÓÈ·Îfi ÁÚfi ¤¯Ô˘Ó ·Ó·ÊÂÚı›

ÌÂȈ̤ÓË ·Ó·ÏÔÁ›· ÏÂÎÈı›Ó˘/ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ ηÈ

ÂÏ·Ùو̤Ó˜ Û˘ÁÎÂÓÙÚÒÛÂȘ ʈÛÊ·ÙȉԯÔÏ›Ó˘ ηÈ

ÚˆÙ½Ó˘ ∞ ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ·

(46,47), ÂÓÒ ¿ÏÏÔÈ ÂÚ¢ÓËÙ¤˜ ·Ó·Ê¤ÚÔ˘Ó Ê˘ÛÈÔÏÔÁÈ-

Τ˜ ·Ó·ÏÔÁ›Â˜ ÏÂÎÈı›Ó˘/ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ Î·È Ê˘ÛÈÔ-

ÏÔÁÈ΋ Û˘ÁΤÓÙÚˆÛË Ù˘ ʈÛÊ·ÙȉÔÁÏ˘ÎÂÚfiÏ˘

(48). ™ÙÔ ‚ÚÔÁ¯Ô΢„ÂÏȉÈÎfi ¤ÎÏ˘Ì· ÓÂÔÁÓÒÓ ÌÂ

™¢∫, ÌÈ· ÚfiÛÊ·ÙË ÌÂϤÙË ÙˆÓ Ijsselstijn Î·È Û˘Ó.

·Ó·Ê¤ÚÂÈ fiÙÈ ÔÈ Û˘ÁÎÂÓÙÚÒÛÂȘ ʈÛÊ·ÙȉԯÔÏ›Ó˘

Î·È ÊˆÛÊ·ÙȉÔÁÏ˘ÎÂÚfiÏ˘, Î·È ÔÈ ·Ó·ÏÔÁ›Â˜ ÏÂÎÈı›-

Ó˘/ÛÊÈÁÁÔÌ˘ÂÏ›Ó˘ Â›Ó·È ·ÚfiÌÔȘ Ì ÂΛӘ ÓË-

›ˆÓ ›‰È·˜ ËÏÈΛ·˜ Ô˘ ·Ó‹Î·Ó ÛÙËÓ ÔÌ¿‰· ÙˆÓ Ì·Ú-

Ù‡ÚˆÓ (45) Î·È ÚÔÙ›ÓÂÈ fiÙÈ ÌÈ· ÚfiˆÚË ·Ó¿Ú-

ÎÂÈ· ÙÔ˘ ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· Â›Ó·È Ì¿Ï-

ÏÔÓ ·›ı·ÓË ÛÙ· ‚Ú¤ÊË Ì ™¢∫. ÕÏÏË ÌÂϤÙË (49)

·Ó·Ê¤ÚÂÈ fiÙÈ Ù· ÓÂÔÁÓ¿ Ì ™¢∫ ¤¯Ô˘Ó ÛËÌ·ÓÙÈο ·-

ÌËÏfiÙÂÚ· ›‰· ʈÛÊ·ÙȉÔÁÏ˘ÎÂÚfiÏ˘ Î·È Úˆ-

Ù½Ó˘ ∞ ÛÙÔ ˘ÏÈÎfi ·fi ·Ó·ÚÚfiÊËÛË Ù˘ ÙÚ·¯Â›·˜.

∞˘Ù¿ Ù· ›‰· ÌÂÈÒÓÔÓÙ·È ·ÎfiÌË ÂÚÈÛÛfiÙÂÚÔ

ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÌÂϤÙ˘, Û ۇÁÎÚÈÛË Ì ·˘Ù¿

ÙˆÓ Ê˘ÛÈÔÏÔÁÈÎÒÓ ÓÂÔÁÓÒÓ ›‰È·˜ ËÏÈΛ·˜ Ô˘ ·Ô-

Ù¤ÏÂÛ·Ó ÙËÓ ÔÌ¿‰· ÙˆÓ Ì·ÚÙ‡ÚˆÓ. ∞˘Ù¿ Ù· Û˘ÌÂ-

Ú¿ÛÌ·Ù· ‰ËÏÒÓÔ˘Ó ÙËÓ ‡·ÚÍË ·Ó¿ÚÎÂÈ·˜ ÙÔ˘

ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡ ·Ú¿ÁÔÓÙ· Û ·ÛıÂÓ›˜ ÌÂ

™¢∫, Ë ÔÔ›· ı· ÌÔÚÔ‡Û ӷ ÔÊ›ÏÂÙ·È ÛÙËÓ ‡·Ú-

ÍË ÌÈÎÚfiÙÂÚ˘ ΢„ÂÏȉÈ΋˜ ÂÈÊ¿ÓÂÈ·˜ ÛÙÔ˘˜ ÂË-

Ú·Ûı¤ÓÙ˜ Ó‡ÌÔÓ˜ ‹ ÛÙË ÌÂȈ̤ÓË Û‡ÓıÂÛË ‹

ÛÙÔÓ ·˘Í·ÓfiÌÂÓÔ Î·Ù·‚ÔÏÈÛÌfi ÙÔ˘ (50).

∫¿ÔÈ· Ê¿Ú̷η ¤¯Ô˘Ó Û¯ÂÙÈÛÙ› Ì ÙËÓ ÂÌÊ¿ÓÈ-

ÛË ™¢∫, fiˆ˜ Ë ı·ÏȉÔÌ›‰Ë, Ë ÎÈÓ›ÓË Î·È Ù· ·ÓÙÈÂÈ-

ÏËÙÈο (51,52). ∂›Û˘, ™¢∫ ¤¯ÂÈ ·Ó·ÊÂÚı› Û ÓÂ-

ÔÁÓfi ‰È·‚ËÙÈ΋˜ ÌËÙ¤Ú·˜ (53). ∏ ÔÚ‹ÁËÛË ÔÚÌÔÓÒÓ

ÌÔÚ› Ó· Â›Ó·È ÛËÌ·ÓÙÈ΋. ∆· ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉ‹

100 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:100-000

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·100

Page 29: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

ÚÔ¿ÁÔ˘Ó ÙËÓ ˆÚ›Ì·ÓÛË ÙˆÓ Ó¢ÌfiÓˆÓ Î·È ÛÙ·ıÂ-

ÚÔÔÈÔ‡Ó ÙÔ Ó¢ÌÔÓÈÎfi ·ÁÁÂÈ·Îfi ÙÔ›¯ˆÌ·, ÚÔη-

ÏÒÓÙ·˜ ·‡ÍËÛË ÙÔ˘ ¿¯Ô˘˜ ÙÔ˘ ÙÔȯÒÌ·Ùfi˜ ÙÔ˘˜

ÛÙ· ˙Ò· Ì ™¢∫ Î·È ·˘Í¿ÓÔ˘Ó ÙÔ˘˜ ˘Ô‰Ô¯Â›˜ ÙˆÓ

ÁÏ˘ÎÔÎÔÚÙÈÎÔÂȉÒÓ ÛÙ· ·È‰È¿ Ì ™¢∫ (54).

¶ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË

™ÙÔ ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi, ÔÈ ‰È·ÊÚ·ÁÌ·ÙÔ΋-

Ϙ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ÚÔÁÂÓÓËÙÈο ηٿ ÙÔÓ ˘Â-

Ú˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ ÂÌ‚Ú‡Ô˘. ∆Ô ÔÛÔÛÙfi

ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ‰È·Ê¤ÚÂÈ ÛËÌ·ÓÙÈο ÛÙȘ

‰È¿ÊÔÚ˜ ‰ËÌÔÛȇÛÂȘ, ·ÏÏ¿ ηٿ ̤ÛÔ fiÚÔ ‚Ú›-

ÛÎÂÙ·È ÛÙÔ 50-54% (55,7,9), Ì ‡ÚÔ˜ Ô˘ Î˘Ì·›ÓÂ-

Ù·È ·fi 29-100% (56,57).∆Ô ÔÛÔÛÙfi ·˘Ùfi ·˘Í¿ÓÂ-

Ù·È ÛÙÔ 72% fiÙ·Ó Û˘Ó˘¿Ú¯Ô˘Ó ÂÈÚfiÛıÂÙ˜ ·Óˆ-

̷ϛ˜ ‹ ·ıÔÏÔÁÈÎfi˜ ηڢfiÙ˘Ô˜ (56). ŒÓ· ÛËÌ·-

ÓÙÈÎfi˜ ·ÚÈıÌfi˜ ™¢∫ ‰È·ÁÈÁÓÒÛÎÂÙ·È ÌÂÙ¿ ÙËÓ 24Ë

‚‰ÔÌ¿‰· ·ËÛ˘. ∏ ̤ÛË ËÏÈΛ· ·ËÛ˘ ÁÈ· ÙË

‰È¿ÁÓˆÛË Ù˘ ·Ï‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ Â›Ó·È Ë

25Ë Â‚‰ÔÌ¿‰· ·ËÛ˘ (55). √È ‰È·ÊÚ·ÁÌ·ÙÔ΋Ϙ

Ì ·ÌÊÔÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË ·ÚÔ˘ÛÈ¿˙Ô˘Ó Û˘-

¯ÓfiÙÂÚ· Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜, Ô˘ Ô‰ËÁÔ‡Ó Û ˘„Ë-

ÏfiÙÂÚÔ ÔÛÔÛÙfi ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ηÈ

Ùˆ¯‹ ¤Î‚·ÛË (55). ∏ ™¢∫ ‰È·ÁÈÁÓÒÛÎÂÙ·È ˘ÂÚË-

¯ÔÁÚ·ÊÈο Ì ÙËÓ ·Ó‡ÚÂÛË ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÔÚÁ¿-

ÓˆÓ ÂÓÙfi˜ Ù˘ ıˆÚ·ÎÈ΋˜ ÎÔÈÏfiÙËÙ·˜. ŒÌÌÂÛÔÈ ‰Â›-

ÎÙ˜ Â›Ó·È ÙÔ ÔÏ˘˘‰Ú¿ÌÓÈÔ, Ô ·ÓÒÌ·ÏÔ˜ ηډȷ-

Îfi˜ ¿ÍÔÓ·˜ Î·È Ë ÌÂÙ·ÙfiÈÛË ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘.

¶ÂÚÈÛÙ·ÏÙÈΤ˜ ÎÈÓ‹ÛÂȘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘ Î·È ÙÔ˘ ÏÂ-

ÙÔ‡ ÂÓÙ¤ÚÔ˘ ÂÓÙfi˜ ÙÔ˘ ıÒڷη ÌÔÚ› Ó· Á›ÓÔ˘Ó

·ÓÙÈÏËÙ¤˜ ˘ÂÚ˯ÔÁÚ·ÊÈο ÛÙ· ÙÂÏÈο ÛÙ¿‰È·

Ù˘ ·ËÛ˘. √È ‰È·ÊÚ·ÁÌ·ÙÔ΋Ϙ Ô˘ ÂÓÙÔ›˙Ô-

ÓÙ·È ‰ÂÍÈ¿ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ÚÔÁÂÓÓËÙÈο ÈÔ ‰‡-

ÛÎÔÏ·, ‰ÈfiÙÈ ÙÔ ‹·Ú ¤¯ÂÈ ·ÚfiÌÔÈ· ˯ÔÁ¤ÓÂÈ· ÌÂ

ÙÔ˘˜ ÂÌ‚Ú˘˚ÎÔ‡˜ Ó‡ÌÔÓ˜ Î·È ÌÔÚ› Ó· Â›Ó·È ÙÔ

ÌfiÓÔ fiÚÁ·ÓÔ Ô˘ ÚÔÛ›ÙÂÈ ÂÓÙfi˜ ÙÔ˘ ıÒڷη. ∏

ı¤ÛË Ù˘ ¯ÔÏˉfi¯Ô˘ ·ÛÙ˘ Î·È ÔÈ ¤ÌÌÂÛÔÈ ‰Â›ÎÙ˜

ÌÔÚ› Ó· Ê·ÓÔ‡Ó È‰È·›ÙÂÚ· ¯Ú‹ÛÈÌÔÈ Û ·˘Ù‹ ÙËÓ

ÂÚ›ÙˆÛË (55).

∫ÏÈÓÈ΋ ÂÈÎfiÓ·

∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ·ÚÔ˘ÛÈ¿˙ÂÙ·È

Û˘¯ÓfiÙÂÚ· ÛÙË ÓÂÔÁÓÈ΋ ÂÚ›Ô‰Ô Ì ÛÔ‚·Ú‹ ·Ó·-

Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· (39). ™ÙÔ 5-30% ÙˆÓ ÂÚÈ-

ÙÒÛˆÓ, Ë ‰È¿ÁÓˆÛË Î·ı˘ÛÙÂÚ› ¤Ú·Ó Ù˘ ÓÂÔ-

ÁÓÈ΋˜ ÂÚÈfi‰Ô˘ (58,59) Î·È ÌÔÚ› Ó· ÂΉËψı›

·ÚÁfiÙÂÚ· Ì ÔÈΛÏË ÎÏÈÓÈ΋ ÂÈÎfiÓ· (60). ªÂÙ¿ ÙË

Á¤ÓÓËÛË, fiÙ·Ó Ë Î‹ÏË ÁÂÌ›˙ÂÈ Ì ·¤Ú·, Ë Û˘Ì›ÂÛË

ÙˆÓ Ó¢ÌfiÓˆÓ ÂȉÂÈÓÒÓÂÙ·È. ∏ ÚfiÁÓˆÛË ÂÍ·Ú-

Ù¿Ù·È ·fi ÙÔÓ ‚·ıÌfi Ù˘ Ó¢ÌÔÓÈ΋˜ ˘ÔÏ·Û›·˜

(61) Î·È ÙË Û˘Ó˘¿Ú¯Ô˘Û· ÂÏ¿ÙÙˆÛË ÙˆÓ Î˘„ÂÏÈ-

‰ÈÎÒÓ Î·È ·ÁÁÂÈ·ÎÒÓ ÂÈÊ·ÓÂÈÒÓ. ªÔÚ› Ó· Û˘Ó˘-

¿Ú¯ÂÈ ÛÔ‚·Ú‹ ˘ÂÚηӛ· Î·È ˘ÔÍ·ÈÌ›·, Ì·˙› ÌÂ

‚·ÚÈ¿ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË, Ì ·ÔÙ¤ÏÂÛÌ· ‰Â-

ÍÈÔ-·ÚÈÛÙÂÚ‹ ‰È·Ê˘Á‹ ÛÙÔ Â›Â‰Ô ÙˆÓ ÎfiÏˆÓ Î·È

ÙÔ˘ ·ÚÙËÚÈ·ÎÔ‡ fiÚÔ˘. ∆· Û˘¯ÓfiÙÂÚ· Û˘ÌÙÒÌ·-

Ù· ·ÊÔÚÔ‡Ó ÙÔ ·Ó·Ó¢ÛÙÈÎfi (43%), ÙÔ Á·ÛÙÚÂÓÙÂ-

ÚÈÎfi (33%), ÙÔ ·Ó·Ó¢ÛÙÈÎfi Î·È Á·ÛÙÚÂÓÙÂÚÈÎfi

Û‡ÛÙËÌ· (13%), ÂÓÒ ·Û˘Ìو̷ÙÈΤ˜ ÂÚÈÙÒÛÂȘ

·Ó·Ê¤ÚÔÓÙ·È Û ÔÛÔÛÙfi 11%. °È· Ù· ÓÂÔÁÓ¿ ÛÙ·

ÔÔ›· ‰ÂÓ Á›ÓÂÙ·È Ë ‰È¿ÁÓˆÛË ÂÓ‰ÔÌ‹ÙÚÈ·, Ë ¤Ó·Ú-

ÍË ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ Û˘Ì‚·›ÓÂÈ Û˘Ó‹ıˆ˜ Ì ·Ó·-

Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ· ̤۷ ÛÙȘ ÚÒÙ˜ ÒÚ˜ ‹

Ë̤Ú˜ ˙ˆ‹˜. ∆· ÓÂÔÁÓ¿ Ì ÙËÓ ÈÔ ‚·ÚÈ¿ Û˘ÌÙˆ-

Ì·ÙÔÏÔÁ›· ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙËÓ ·›ıÔ˘Û· ÙÔÎÂÙÔ‡ ÌÂ

‰˘Û¯¤ÚÂÈ· ÛÙËÓ ·Ó¿ÓË„Ë Î·È Ì ˘„ËÏ¿ ÔÛÔÛÙ¿

Ó¢ÌÔıÒڷη. √È ·Ó·Ó¢ÛÙÈÎÔ› ‹¯ÔÈ Â›Ó·È ·fi-

ÓÙ˜ ÛÙËÓ ¿Û¯Ô˘Û· ÏÂ˘Ú¿, Ô ıÒڷη˜ ¤¯ÂÈ Î˘-

ÏÈÓ‰ÚÈÎfi Û¯‹Ì·, Ë ÎÔÈÏÈ¿ Â›Ó·È ÛηÊÔÂȉ‹˜ Î·È ÔÈ

ηډȷÎÔ› ÙfiÓÔÈ ÌÂÙ·ÙÔ›˙ÔÓÙ·È ‰ÂÍÈ¿. ∏ ÏÂÈÔÓfi-

ÙËÙ· ÙˆÓ ‰ÂÍÈ¿ ÂÓÙÔÈ˙fiÌÂÓˆÓ ™¢∫ ÂΉËÏÒÓÂÙ·È

ÌÂ Û˘ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi (62). ∏ Û‡-

ÁÎÏÂÈÛË ÙÔ˘ ·Ó·ÙÔÌÈÎÔ‡ ÂÏÏ›ÌÌ·ÙÔ˜ ·fi ÙÔ ‹·Ú

·ÔÙÚ¤ÂÈ ÙË ÌÂÙ¤ÂÈÙ· ÚfiÙˆÛË ÎÔ›ÏˆÓ ÔÚÁ¿-

ÓˆÓ Î·È ÙËÓ ÂÌÊ¿ÓÈÛË Û˘ÌÙˆÌ¿ÙˆÓ ·fi ÙÔ Á·-

ÛÙÚÂÓÙÂÚÈÎfi.

¢ÈÂÚ‡ÓËÛË

∏ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη Î·È ÎÔÈÏ›·˜ ·ÔÙÂÏ› ÙËÓ

ÚÒÙË ÂͤٷÛË Û fiÏ· Ù· ·È‰È¿ Ì ·ÓÂÍ‹ÁËÙ· Û˘-

ÌÙÒÌ·Ù· ·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi ‹ Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡-

ÛÙËÌ· (60,62). ¢˘ÛÎÔÏ›· ÛÙËÓ ·ÎÙÈÓÔÏÔÁÈ΋ ‰È¿ÁÓˆ-

ÛË ··ÓÙ¿ Û ÔÛÔÛÙfi 25%, Ì ÙÔÓ Ó¢ÌÔıÒڷη

Î·È ÙÔÓ ˘‰ÚÔıÒڷη ˆ˜ ÈÔ ÎÔÈÓ¤˜ Ï·Óı·Ṳ̂Ó˜ ‰È·-

ÁÓÒÛÂȘ (62). ∏ ÙÔÔı¤ÙËÛË ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎÔ‡ η-

ıÂÙ‹Ú· ÁÈ· ÙË ‰È·›ÛÙˆÛË Ù˘ ı¤Û˘ ÙÔ˘ ÛÙÔÌ¿¯Ô˘

Î·È Ô ·ÎÙÈÓÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì ÛÎÈ·ÛÙÈÎfi ÙÔ˘ ·ÓÒÙÂ-

ÚÔ˘ ÂÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ‚ÔËıÔ‡Ó ÛÙË ÛˆÛÙ‹ ‰È¿-

ÁÓˆÛË Ù˘ ·ÚÈÛÙÂÚ‹˜ ™¢∫. ∏ CT ıÒڷη Â›Ó·È Ú‹ÛÈ-

ÌË ÁÈ· ÙË ‰È¿ÁÓˆÛË Ù˘ ‰ÂÍÈ¿˜ ™¢∫, fiÙ·Ó ÚÔÛ›ÙÂÈ

ÙÔ ‹·Ú ÂÓÙfi˜ Ù˘ ıˆÚ·ÎÈ΋˜ ÎÔÈÏfiÙËÙ·˜ (62).

¢È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË

∏ ‚Ï¿‚Ë ÙˆÓ Ó¢ÌfiÓˆÓ Ô˘ Û˘Ì‚·›ÓÂÈ Î·Ù¿ ÙË

‰È¿ÚÎÂÈ· Ù˘ ÂÌ‚Ú˘˚΋˜ ˙ˆ‹˜ ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ

ÛÙËÓ ÂÌÊ¿ÓÈÛË Û˘ÁÁÂÓÒÓ ·ÓˆÌ·ÏÈÒÓ, fiˆ˜ Û˘ÁÁÂÓ‹

·‰Âӈ̷ÙÒ‰Ë Î˘ÛÙÈ΋ ‰˘ÛÏ·Û›·, Û˘ÁÁÂÓ¤˜ ÏÔ‚Ò-

‰Â˜ ÂÌʇÛËÌ·, ‰È¿¯˘Ù˜ Ó¢ÌÔÓÈΤ˜ ·ÛÙÂȘ ηÈ,

Û·ÓÈfiÙÂÚ·, Ó¢ÌÔÓÈ΋ ·ÁÂÓÂÛ›·, ˘‰ÚÔıÒڷη,

Î·È ÙÂÏÈο ÛÙÔÓ ı¿Ó·ÙÔ ÙÔ˘ ÂÌ‚Ú‡Ô˘ ‹ ÙÔ˘ ÓÂÔÁÓÔ‡

·ÚÁfiÙÂÚ·. ∏ ‰È¿ÁÓˆÛË ÙˆÓ Û˘ÁÁÂÓÒÓ ·˘ÙÒÓ ·ÓˆÌ·-

ÏÈÒÓ ÚÔÁÂÓÓËÙÈο Â›Ó·È ‰‡ÛÎÔÏË. ªÂÙ·ÁÂÓ¤ÛÙÂÚ·,

Ë CT ıÒڷη ÌÔÚ› Ó· ı¤ÛÂÈ ÙË ‰È¿ÁÓˆÛË. ªÂÙ¿ ÙË

Á¤ÓÓËÛË, Ë ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÂÈ Â›-

Û˘ ÙÔÓ Ó¢ÌÔıÒڷη, ÙË ‰ÂÍÈÔηډ›· Î·È ÙË Ï·-

Ú˘ÁÁÔÙÚ·¯Âȷ΋ ·fiÊÚ·ÍË.

101™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

¶·È‰È·ÙÚÈ΋ 2009;72:101-000

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·101

Page 30: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

£Âڷ›·

∏ ‡·ÚÍË Â͈ۈ̷ÙÈ΋˜ ÌÂÌ‚Ú¿Ó˘ Ô͢ÁfiÓˆ-

Û˘ (ECMO), Ë ‰˘Ó·ÙfiÙËÙ· ÚÔÂÁ¯ÂÈÚËÙÈ΋˜ ÛÙ·-

ıÂÚÔÔ›ËÛ˘ ÙÔ˘ ÓÂÔÁÓÔ‡ Î·È Ë ÚfiÔ‰Ô˜ fiÛÔÓ ·ÊÔ-

Ú¿ ÛÙËÓ ÂÓ‰ÔÌ‹ÙÚÈÔ ·ÓÙÈÌÂÙÒÈÛË Ù˘ Û˘ÁÁÂÓÔ‡˜

‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ÛÙËÚ›˙Ô˘Ó ÙËÓ ÚÔÛ¿ıÂÈ· ÌË

ÂÈıÂÙÈ΋˜ ·ÔηٿÛÙ·Û˘ ÙˆÓ ‚Ï·‚ÒÓ. ¶·Ï·ÈfiÙÂ-

Ú·, Ë ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ıˆÚÔ‡ÓÙ·Ó Â›ÁÔ˘Û· η-

Ù¿ÛÙ·ÛË, ÛÙËÓ ÔÔ›· ›¯Â ·fiÏ˘ÙË ¤Ó‰ÂÈÍË Ë ¿ÌÂÛË

¯ÂÈÚÔ˘ÚÁÈ΋ ·Ú¤Ì‚·ÛË, ÒÛÙ ӷ ÂÈÙ¢¯ı› ÙÔ Î·-

χÙÂÚÔ ‰˘Ó·Ùfi ·ÔÙ¤ÏÂÛÌ·. ∞˘Ù‹ Ë ÛÙÚ·ÙËÁÈ΋ ¤¯ÂÈ

·ÓÂÎÙÈÌËı›, ÏfiÁˆ Ù˘ ηٷÓfiËÛ˘ ÙÔ˘ ÚfiÏÔ˘ Ù˘

Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÙËÓ

Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· Î·È ÙËÓ Â›‰Ú·ÛË Ù˘ ¯ÂÈ-

ÚÔ˘ÚÁÈ΋˜ ·Ú¤Ì‚·Û˘ ÛÙËÓ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘Ú-

Á›·. ∂›Ó·È ϤÔÓ ÍÂοı·ÚÔ fiÙÈ ÙÔ Î‡ÚÈÔ Úfi‚ÏËÌ·

Ô˘ ÚÔηÏ› ηډÈÔ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· ›-

Ó·È Ë Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Î·È Ë ˘ÔÏ·Û›· ÙˆÓ

Ó¢ÌfiÓˆÓ Î·È fi¯È Ë Û˘Ì›ÂÛË Ô˘ ÚÔηÏÔ‡Ó Ù·

ÂÓ‰ÔÂÚÈÙÔÓ·˚ο ÛÏ¿¯Ó· Ô˘ ¤¯Ô˘Ó ÂÈÛ¤ÏıÂÈ ÛÙË

ıˆÚ·ÎÈ΋ ÎÔÈÏfiÙËÙ·.

™Â ÓÂÔÁÓ¿ Ì ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Î·È Û ·˘Ù¿

Ô‡ Ù›ıÂÙ·È ¤ÁηÈÚ· Ë ˘fiÓÔÈ· Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋-

Ï˘ ÙÔÔıÂÙÂ›Ù·È ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎfi˜ ηıÂÙ‹Ú·˜ ηÈ

Á›ÓÂÙ·È Û˘Ó¯Ҙ ·Ó·ÚÚfiÊËÛË ÙÔ˘ ÛÙÔÌ¿¯Ô˘, ÒÛÙ ӷ

ÂÏ·ÙÙˆı› Ë ÔÛfiÙËÙ· ÙÔ˘ ·¤Ú· ̤۷ ÛÙËÓ Î‹ÏË Î·È

Ó· ÌÂȈı› Ë Û˘Ì›ÂÛË ÙˆÓ Ó¢ÌfiÓˆÓ. ∆Ô ÓÂÔÁÓfi

Ú¤ÂÈ Ó· ‰È·ÛˆÏËÓÒÓÂÙ·È ·Ì¤Ûˆ˜ Î·È Ó· ÂÊ·ÚÌfi-

˙ÔÓÙ·È ıÂÙÈΤ˜ ȤÛÂȘ. √ Ì˯·ÓÈÎfi˜ ·ÂÚÈÛÌfi˜ ·Ô-

ÙÂÏ› ÙË ‚¿ÛË Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ™¢∫. ™ÙËÓ

ÚÔÛ¿ıÂÈ· ÂÏ¿ÙÙˆÛ˘ ÙÔ˘ ‚·ÚÔÙÚ·‡Ì·ÙÔ˜ Î·È ÙÔ˘

Ó¢ÌÔıÒڷη ÚÔÙÈÌ¿Ù·È Ô ‹ÈÔ˜ ·ÂÚÈÛÌfi˜

(63,65). ™Â ÂÚÈÙÒÛÂȘ ·ÓıÂÎÙÈ΋˜ ·Ó·Ó¢ÛÙÈ΋˜

·Ó¿ÚÎÂÈ·˜ Î·È ·Ú·Ì¤ÓÔ˘Û·˜ Ó¢ÌÔÓÈ΋˜ ˘¤Ú-

Ù·Û˘ ÂӉ›ÎÓ˘Ù·È Ë ÂÊ·ÚÌÔÁ‹ „›Û˘¯ÓÔ˘ ·ÂÚÈÛÌÔ‡,

ÌfiÓÔ˘ ‹ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ÂÈÛÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ

ÙÔ˘ ·˙ÒÙÔ˘ (65). °È· Ù· ÓÂÔÁÓ¿ Ì ‚·ÚÈ¿ ·Ó·Ó¢ÛÙÈ-

΋ ·Ó¿ÚÎÂÈ·, Ì ÌÂÁ¿ÏË ˘ÔÛÙ‹ÚÈÍË ÛÙÔÓ Û˘Ì‚·-

ÙÈÎfi Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi, Ô˘ ÏËÚÔ‡Ó Ù· ÎÚÈÙ‹ÚÈ· ÁÈ·

ECMO, Ô ‚·ıÌfi˜ ÂÈÙ˘¯›·˜ ÙÔ˘ „›Û˘¯ÓÔ˘ ·ÂÚÈÛÌÔ‡

ÚÈÓ ÙËÓ ÂÊ·ÚÌÔÁ‹ ÙÔ˘ ECMO ÊÙ¿ÓÂÈ ÙÔ 33% (67).

∆Ô ÂÈÛÓÂfiÌÂÓÔ ÌÔÓÔÍ›‰ÈÔ ÙÔ˘ ·˙ÒÙÔ˘ ¤¯ÂÈ ¯ÚËÛÈ-

ÌÔÔÈËı› ›Û˘ Ì ÂÈÙ˘¯›· ÛÙË ıÂڷ›· Ù˘ ‚·-

ÚÈ¿˜ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ ÙÔ˘ ÓÂÔÁÓÔ‡ Î·È ‰È¿-

ÊÔÚÔÈ ÂÚ¢ÓËÙ¤˜ ‰ÔÎ›Ì·Û·Ó ·˘Ù‹Ó ÙË ıÂڷ›· ÛÂ

ÓÂÔÁÓ¿ Ì ÛÔ‚·Ú‹ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË Ô˘ ÂÈ-

ϤÎÂÈ ÙËÓ ÔÚ›· Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ (64).

∏ ¯ÔÚ‹ÁËÛË ÓÙÔ·Ì›Ó˘ ‹ ÓÙÔÌÔ˘Ù·Ì›Ó˘ Û˘-

Ó‹ıˆ˜ ‚ÂÏÙÈÒÓÂÈ ÙËÓ Î·Ú‰È·Î‹ ·ÚÔ¯‹ Î·È ÙË Û˘-

ÛÙËÌ·ÙÈ΋ ›ÂÛË, ÂÏ·ÙÙÒÓÂÈ ÙË ÌÂÙ·‚ÔÏÈ΋ ÔͤˆÛË

Î·È ¤ÌÌÂÛ· ÌÂÈÒÓÂÈ ÙËÓ ·˘ÍË̤ÓË ›ÂÛË ÛÙ· Ó¢-

ÌÔÓÈο ·ÁÁ›·. ∏ ÂÊ¿·Í ¯ÔÚ‹ÁËÛË ‰ÈÙÙ·ÓıÚ·ÎÈÎÔ‡

Ó·ÙÚ›Ô˘ ÌÔÚ› Ó· Ê·Ó› ¯Ú‹ÛÈÌË. ¶¿ÓÙˆ˜, Ô Â·Ú-

΋˜ ·ÂÚÈÛÌfi˜, Ë Ô͢ÁfiÓˆÛË Î·È ÔÈ ·Ú¿ÁÔÓÙ˜ Ô˘

·˘Í¿ÓÔ˘Ó ÙË Û˘ÛÙËÌ·ÙÈ΋ ›ÂÛË ·ÔÙÂÏÔ‡Ó ÙËÓ

·Ú΋ ıÂڷ›·. ∏ ¯ÔÚ‹ÁËÛË ÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎÔ‡

·Ú¿ÁÔÓÙ· ÌÔÚ›, fi¯È ÌfiÓÔ Ó· ·ÓÙÈÌÂÙˆ›ÛÂÈ ÙËÓ

·Ó¿ÚÎÂÈ¿ ÙÔ˘, ·ÏÏ¿ Î·È Ó· ‚ÂÏÙÈÒÛÂÈ ÙËÓ Â˘ÂÓ‰Ô-

ÙfiÙËÙ·, Ó· ÂÏ·ÙÙÒÛÂÈ ÙȘ Ó¢ÌÔÓÈΤ˜ ·ÁÁÂȷΤ˜

·ÓÙÈÛÙ¿ÛÂȘ Î·È Ó· ‚ÂÏÙÈÒÛÂÈ ÙË ÚÔ‹ ÙÔ˘ ·›Ì·ÙÔ˜

ÛÙÔÓ Ó‡ÌÔÓ· (68,69).

∏ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ™¢∫ ‰ÂÓ Â›Ó·È ¿ÌÔÈÚË ÂÈ-

ÏÔÎÒÓ. ¶Ó¢ÌÔıÒڷη˜ ÛÙËÓ ·ÓÙ›ıÂÙË ÏÂ˘Ú¿

ÌÔÚ› Ó· ÂÌÊ·ÓÈÛÙ› Ï›Á· ÏÂÙ¿ ÌÂÙ¿ ÙËÓ ÂÊ·ÚÌÔ-

Á‹ ·ÂÚÈÛÌÔ‡ Ì ˘„ËϤ˜ ȤÛÂȘ. ∆· ÓÂÔÁÓ¿ Ì ˘Ô-

Ï·ÛÙÈÎÔ‡˜ Ó‡ÌÔÓ˜ Â›Ó·È ÈÔ ÂÈÚÚ‹ ÛÙËÓ

·Ó¿Ù˘ÍË Ó¢ÌÔıÒڷη. ÕÌÂÛË ·Ó Î·È Û¿ÓÈ· ÌÂ-

ÙÂÁ¯ÂÈÚËÙÈ΋ ÂÈÏÔ΋ ·ÔÙÂÏ› Ô ¯˘ÏÔıÒڷη˜,

Èı·ÓÒ˜ ÔÊÂÈÏfiÌÂÓÔ˜ Û Ì˯·ÓÈÎfi ÙÚ·˘Ì·ÙÈÛÌfi

ÙˆÓ ÏÂÌÊ·ÁÁ›ˆÓ Á‡Úˆ ·fi ÙÔ ‰È¿ÊÚ·ÁÌ· (70,71).

¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜

∏ ÚfiÁÓˆÛË ÂÍ·ÚÙ¿Ù·È ·fi Ù· ÚÔÁÂÓÓËÙÈο ¢-

Ú‹Ì·Ù·, Ù· ÔÔ›· ÔÚÈÔıÂÙÔ‡Ó ÙË ÛÔ‚·ÚfiÙËÙ· Ù˘

™¢∫ Î·È ·ÔÙÂÏÔ‡Ó ÙË ‚¿ÛË Ù˘ ÂÓË̤ڈÛ˘ ηÈ

ÚÔÁÂÓÓËÙÈ΋˜ ηıÔ‰‹ÁËÛ˘ ÙˆÓ ÁÔÓ¤ˆÓ. ∏ ¤Î‚·ÛË

ÂÍ·ÚÙ¿Ù·È ·fi ·Ú¿ÁÔÓÙ˜ fiˆ˜ Ë ·ÚÔ˘Û›· Û˘ÓÔ-

‰ÒÓ ·ÓˆÌ·ÏÈÒÓ, ÙÔ ÛÙ¿‰ÈÔ Î‡ËÛ˘ ÛÙÔ ÔÔ›Ô Û˘Ó¤-

‚Ë Ë ÚfiÙˆÛË ÙˆÓ ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÔÚÁ¿ÓˆÓ ÂÓÙfi˜

ÙÔ˘ ıÒڷη, Ô fiÁÎÔ˜ ÙˆÓ ÛÏ¿Á¯ÓˆÓ Ô˘ ÚÔÛÙ›-

ÙÔ˘Ó ÂÓÙfi˜ ÙÔ˘ ıÒڷη Î·È Ô ‚·ıÌfi˜ Ù˘ Ó¢ÌÔ-

ÓÈ΋˜ ˘ÔÏ·Û›·˜ Î·È ÙˆÓ Î·Ú‰È·ÁÁÂÈ·ÎÒÓ ·ÏÏ·ÁÒÓ

Ô˘ ¤¯Ô˘Ó ÂÈÛ˘Ì‚Â› (¶›Ó·Î·˜ 3).

√È ¯ÚˆÌÔۈ̷ÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ ηıÒ˜ Î·È ÔÈ

ÛÔ‚·Ú¤˜ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ηډȷÁÁÂÈ·ÎÔ‡, ·›˙Ô˘Ó

ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ¤Î‚·ÛË. ¶Ùˆ¯Ô› ÚÔÁÓˆÛÙÈ-

ÎÔ› ‰Â›ÎÙ˜ ıˆÚÔ‡ÓÙ·È: Ë ‰È¿ÁÓˆÛË ÚÈÓ ·fi ÙËÓ

25Ë Â‚‰ÔÌ¿‰· ·ËÛ˘, Ë ·ÚÔ˘Û›· ÙÔ˘ ÛÙÔÌ¿¯Ô˘

ÂÓÙfi˜ ÙÔ˘ ıÒڷη (Ë ÂÈ‚›ˆÛË Â›Ó·È ˘„ËÏfiÙÂÚË

·fi 90% Â¿Ó ÙÔ ÛÙÔÌ¿¯È ‚Ú›ÛÎÂÙ·È Î¿Ùˆ ·fi ÙÔ

‰È¿ÊÚ·ÁÌ·) (72), ÙÔ ÔÏ˘˘‰Ú¿ÌÓÈÔ (ÔÊÂÈÏfiÌÂÓÔ

ÛÙËÓ ›ÂÛË ÙÔ˘ ÔÈÛÔÊ¿ÁÔ˘ ·fi ÙÔÓ ÌÂÁ¿ÏÔ ÂÓ‰Ô-

ıˆÚ·ÎÈÎfi fiÁÎÔ), Ë ÚfiÙˆÛË ÙÔ˘ ‹·ÙÔ˜ (fiÙ·Ó ÙÔ

‹·Ú ‰ÂÓ Û˘ÁηٷϤÁÂÙ·È ÛÙ· fiÚÁ·Ó· Ô˘ ÚÔÛ›-

ÙÔ˘Ó Ë ÚfiÁÓˆÛË Â›Ó·È Î·Ï‡ÙÂÚË, ·ÏÏ¿, ηıÒ˜

ÛÙÔ 75% ÙˆÓ ÂÚÈÙÒÛÂˆÓ ¤Ó· ÌÈÎÚfi ̤ÚÔ˜ ÙÔ˘

‹·ÙÔ˜ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙËÓ Î‹ÏË, Ë ·Í›· ÙÔ˘ ·-

Ú¿ÁÔÓÙ· ·˘ÙÔ‡ ıˆÚÂ›Ù·È ÌÈÎÚ‹) (73), Ô ‡‰Úˆ·˜

(Û¯ÂÙ›˙ÂÙ·È Ì و¯‹ ÚfiÁÓˆÛË) Î·È Ô ÏfiÁÔ˜ Ó‡-

ÌÔÓ· ÚÔ˜ ÎÂÊ·Ï‹ (lung to head ratio, LHR) ÌÈÎÚfi-

ÙÂÚÔ˜ ·fi 1. √ ÏfiÁÔ˜ LRH ÚÔÛ‰ÈÔÚ›˙ÂÙ·È Î·Ù¿ ÙÔ

˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÙÔ˘ ÂÌ‚Ú‡Ô˘ Î·È ÈÛÔ‡Ù·È Ì ÙÔÓ

ÏfiÁÔ Ù˘ ÂÈÊ¿ÓÂÈ·˜ ÙÔ˘ Ó‡ÌÔÓ· Ù˘ Ê˘ÛÈÔÏÔÁÈ-

΋˜ ÏÂ˘Ú¿˜ ÙÔ˘ ÛÒÌ·ÙÔ˜ ÚÔ˜ ÙËÓ ÂÚÈʤÚÂÈ·

Ù˘ ÎÂÊ·Ï‹˜ ÙÔ˘ ÂÌ‚Ú‡Ô˘ (74). √ LHR ÂÌÊ·Ó›˙Â-

Ù·È Ó· Â›Ó·È Ô Î·Ï‡ÙÂÚÔ˜ ÚÔÁÓˆÛÙÈÎfi˜ ‰Â›ÎÙ˘.

102 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:102-000

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·102

Page 31: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

LHR <1 Û¯ÂÙ›˙ÂÙ·È Ì ıÓËÙfiÙËÙ· 100%, ÂÓÒ LHR>1,4 Û¯ÂÙ›˙ÂÙ·È Ì ÂÈ‚›ˆÛË 100% (75,76).

∆Ô ÚÔÁÂÓÓËÙÈÎfi ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜ÌÔÚ› Ó· ·Ó·ÁÓˆÚ›ÛÂÈ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ Ù˘ ηÚ-‰È¿˜. ¶·Ú¿ÁÔÓÙ˜ Ùˆ¯‹˜ ¤Î‚·Û˘ ıˆÚÔ‡ÓÙ·È Ë˘ÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ (77) Î·È Ô ·˘ÍË-̤ÓÔ˜ fiÁÎÔ˜ ·›Ì·ÙÔ˜ ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›· (78).

√ ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÙÔ˘ ÂÌ‚Ú‡Ô˘ Ì ̷-ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÏÂÔÓÂÎÙ›. ¢ÂÓ ÂÚÈÔÚ›˙ÂÙ·È·fi ÙÔ ¿¯Ô˜ ÙˆÓ ÎÔÈÏÈ·ÎÒÓ ÙÔȯˆÌ¿ÙˆÓ Ù˘ ÌËÙ¤-Ú·˜ ‹ ÙÔ ÔÏÈÁÔ¸‰Ú¿ÌÓÈÔ Ì ηχÙÂÚË ·ÂÈÎfiÓÈÛËÙˆÓ Ì·Ï·ÎÒÓ ÈÛÙÒÓ, ÌÔÚ› Ó· ÂȂ‚·ÈÒÛÂÈ ÙËÓ·ÚÔ˘Û›· Û˘ÓÔ‰ÒÓ ·ÓˆÌ·ÏÈÒÓ (79) Î·È Â›Ó·È ·ÔÙÂ-ÏÂÛÌ·ÙÈÎfi˜ ÛÙË ‰È¿ÁÓˆÛË ‰ÂÍÈ¿˜ ™¢∫ Ì ÚfiÙˆÛËÙÔ˘ ‹·ÙÔ˜.

∞ÚÎÂÙÔ› ÌÂÙ·ÁÂÓÓËÙÈÎÔ› ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔ-ÓÙ˜ ¤¯Ô˘Ó ÂÚ¢ÓËı›, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤ÓÔ˘ ηÈÙÔ˘ Ùˆ¯Ô‡ ·ÂÚÈÛÌÔ‡ ÙˆÓ Ó¢ÌfiÓˆÓ ÛÙËÓ ·ÎÙÈÓÔ-ÁÚ·Ê›· ıÒڷη (80).

¡ÔÛËÚfiÙËÙ·

∂ÎÙÈÌ¿Ù·È fiÙÈ ÏÈÁfiÙÂÚÔ ·fi ÙÔ 1/3 ÙˆÓ ÂÌ‚Ú‡ˆÓÌ ™¢∫ Ô˘ ‰ÈÂÁÓÒÛıË ÚÔÁÂÓÓËÙÈο ı· ÂÈ‚ÈÒÛÂȯˆÚ›˜ ·˘ÍË̤ÓË ÓÔÛËÚfiÙËÙ·, Ë ÔÔ›· Û ÌÂÚÈΤ˜ÂÚÈÙÒÛÂȘ ÔÊ›ÏÂÙ·È Û ·Ó·ÙÔÌÈο ·›ÙÈ· Î·È Â›Ó·È·Ó·fiÊ¢ÎÙË, ÂÓÒ Û ¿ÏϘ ·ÔÙÂÏ› Û˘Ó¤ÂÈ· ÙˆÓıÂڷ¢ÙÈÎÒÓ ¯ÂÈÚÈÛÌÒÓ Î·È Â›Ó·È ‰˘Ó·Ùfi Ó· ÂÚÈÔ-ÚÈÛÙ› ÛÙÔ Ì¤ÏÏÔÓ.

∞Ó·Ó¢ÛÙÈ΋ ÏÂÈÙÔ˘ÚÁ›·

∏ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· ·ÔÙÂÏ› ÙËÓ ÈÔÛËÌ·ÓÙÈ΋ ·ÈÙ›· ÓÔÛËÚfiÙËÙ·˜.

™ÙȘ ·ÒÙÂÚ˜ ÂÈÏÔΤ˜ Ù˘ ™¢∫ ÂÚÈÏ·Ì‚¿-

ÓÔÓÙ·È Ë ÂÚÈÔÚÈÛÙÈ΋ ÓfiÛÔ˜ ÙÔ˘ Ó‡ÌÔÓ·, Ô˘

·ÓÙ·Ó·ÎÏ¿ ÙËÓ Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·, fiˆ˜ Ê·›-

ÓÂÙ·È ·fi ÙË ÏÂÈÙÔ˘ÚÁÈ΋ ˘ÔÏÂÈfiÌÂÓË ¯ˆÚËÙÈÎfi-

ÙËÙ·, Î·È Ë ·˘Í·ÓfiÌÂÓË Â›ÙˆÛË Ù˘ ‚ÚÔÁ¯È΋˜

˘ÂÚ·ÓÙȉڷÛÙÈÎfiÙËÙ·˜. ∆Ô 1/3 ÙˆÓ ·ÛıÂÓÒÓ ÌÂ

Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ¤¯ÂÈ ÎÏÈÓÈο Î·È ·ÎÙÈÓÔ-

ÏÔÁÈο Â˘Ú‹Ì·Ù· ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ‰˘ÛÏ·Û›·˜,

Ô˘ Â›Ó·È Û˘¯ÓfiÙÂÚ· Û ÓÂÔÁÓ¿ Ô˘ ·Ú¤ÌÂÈÓ·Ó ÁÈ·

ÌÂÁ·Ï‡ÙÂÚÔ ‰È¿ÛÙËÌ· Û Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi Î·È ÌÂ

¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ (81). ªÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋

·ÔηٿÛÙ·ÛË, ·Ú·ÙËÚÂ›Ù·È ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË

Ù˘ ·Ó¿Ù˘Í˘ Î·È Ù˘ ÏÂÈÙÔ˘ÚÁ›·˜ ÙˆÓ Ó¢ÌfiÓˆÓ,

Ô˘ Â›Ó·È È‰È·›ÙÂÚ· ÂÌÊ·Ó‹˜ ÌÂÙ¿ ÙÔ˘˜ ÚÒÙÔ˘˜ 6

Ì‹Ó˜ ˙ˆ‹˜ (82). ∆· ˘„ËÏ¿ ÔÛÔÛÙ¿ ‰˘ÛÏÂÈÙÔ˘Ú-

Á›·˜ ÙˆÓ Ó¢ÌfiÓˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ¿ÌÂÛ˘

ÌÂÙ·ÁÂÓÓËÙÈ΋˜ ÂÚÈfi‰Ô˘ ·ÎÔÏÔ˘ıÔ‡ÓÙ·È ·fi η-

ÓÔÓÈ΋ ‹ ۯ‰fiÓ Î·ÓÔÓÈ΋ Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›·

ÛÙË ÌÂÙ¤ÂÈÙ· ·È‰È΋ Î·È ÂÊË‚È΋ ˙ˆ‹ (82,83). √È

‰Â›ÎÙ˜ Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ‚ÂÏÙÈÒÓÔÓÙ·È

ÛËÌ·ÓÙÈο Ì ÙËÓ ·‡ÍËÛË Ù˘ ËÏÈΛ·˜, ÙÔ˘ ‡„Ô˘˜

Î·È ÙÔ˘ ‚¿ÚÔ˘˜ ÙˆÓ ·ÛıÂÓÒÓ. ∏ ÌÂȈ̤ÓË ÚfiÛÏË-

„Ë ‚¿ÚÔ˘˜ Â›Ó·È Û˘¯Ó¿ ÙÔ Î‡ÚÈÔ Úfi‚ÏËÌ· Û ÓÂÔ-

ÁÓ¿ ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, Èı·ÓÒ˜ ÂÂȉ‹

ÙÔ ·˘Í·ÓfiÌÂÓÔ ¤ÚÁÔ ·Ó·ÓÔ‹˜ ··ÈÙ› ˘„ËÏ‹ ηٷ-

ӿψÛË ÂÓ¤ÚÁÂÈ·˜, ÂÓÒ ÂÚÈÔÚ›˙ÂÈ ÙËÓ ÈηÓfiÙËÙ·

ÙÔ˘ ·ÛıÂÓÔ‡˜ Ó· ‰È·ÙÚ·Ê› ·ÚÎÒ˜. ∞ÓÙ›ıÂÙ·, Ë

‚ÂÏÙ›ˆÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ÏÂÈÙÔ˘ÚÁ›·˜ ÂÈÙÚ¤ÂÈ

ηχÙÂÚË ·Ó¿Ù˘ÍË (82). ™˘¯Ó¤˜ ÏÔÈÌÒÍÂȘ ÙÔ˘

·Ó·Ó¢ÛÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜ ÂÌÊ·Ó›˙ÔÓÙ·È ÛÙÔ 24%

ÙˆÓ ·ÛıÂÓÒÓ Ì ‹È· ¤ˆ˜ ÌÂÙÚ›Ô˘ ‚·ıÌÔ‡ Ó¢ÌÔ-

ÓÈ΋ ˘ÔÏ·Û›· (84).

∞ÎÔ‹

∂ËÚ·ÛÌfi˜ Ù˘ ·ÈÛıËÙËÚȷ΋˜ ·ÎÔ‹˜ ¤¯ÂÈ Â-

ÚÈÁÚ·Ê› Û ÓÂÔÁÓ¿ Ì ™¢∫ Î·È ·ÚÔ˘ÛÈ¿˙ÂÈ ¤Ó·

¢ڇ Ê¿ÛÌ· ›وÛ˘. ¢˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘ ·ÎÔ‹˜

·Ó·Ê¤ÚıËΠ۠ÓÂÔÁÓ¿, ›Ù ıÂڷ‡ÙËÎ·Ó ÌÂ

ECMO ›Ù fi¯È (85). ∏ ·Ú·ÙÂٷ̤ÓË ¯Ú‹ÛË ÙÔ˘

·ÓÎÔ˘Ú·Ó›Ô˘ (86) Î·È ‰ÈÔ˘ÚËÙÈÎÒÓ Û ÓÂÔÁÓ¿ ÌÂ

¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ÓfiÛÔ (85) ¤¯ÂÈ ·Ó·ÊÂÚı› ˆ˜

Èı·Ófi˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜. ø˜ ·Ú¿ÁÔÓÙ˜

ÎÈÓ‰‡ÓÔ˘ ıˆÚÔ‡ÓÙ·È ÔÈ ·Ú·Î¿Ùˆ: Ë ‰È¿ÚÎÂÈ· ıÂ-

ڷ›·˜ ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ Û˘ÛÙ‹Ì·ÙÔ˜, Û˘ÌÂÚÈ-

Ï·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ECMO, Î·È Ë ‰È¿ÚÎÂÈ· ¯Ô-

Ú‹ÁËÛ˘ ˆÙÔÙÔÍÈÎÒÓ Ê·Ú̿ΈÓ, fiˆ˜ Â›Ó·È Ù· ‰È-

Ô˘ÚËÙÈο Ù˘ ·Á·Ï˘ Î·È ÙÔ ·ÓÎÔ˘Ú¿ÓÈÔ, Ô˘

ÂËÚ¿˙ÂÈ ÙË ÓÂÊÚÈ΋ ÏÂÈÙÔ˘ÚÁ›· (87). √È Ù·ÎÙÈÎÔ›

¤ÏÂÁ¯ÔÈ Ù˘ ·ÎÔ‹˜ ˆ˜ ÙËÓ ËÏÈΛ· ÙˆÓ 3 ÂÙÒÓ ıˆ-

ÚÔ‡ÓÙ·È ··Ú·›ÙËÙÔÈ. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Ù˘ ÌÂÈ-

ˆÌ¤Ó˘ ·ÎÔ‹˜ ·ÔÙÚ¤ÂÈ ÙËÓ Î·ı˘ÛÙ¤ÚËÛË ¤Ó·Ú-

͢ Ù˘ ÔÌÈÏ›·˜ Î·È ÙËÓ ÂÌÊ¿ÓÈÛË ‰È·Ù·Ú·¯ÒÓ ÂÈ-

ÎÔÈÓˆÓ›·˜.

103™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

¶·È‰È·ÙÚÈ΋ 2009;72:103-000

¶›Ó·Î·˜ 3. ¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ÛÙË ™¢∫

ñ ¶ÚÔÁÂÓÓËÙÈ΋ ÂÎÙ›ÌËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙÔ˘ Ó‡ÌÔÓ·- √ ÏfiÁÔ˜ LRH- √ ÏfiÁÔ˜ ‰È·Ì¤ÙÚÔ˘ ÙÔ˘ Ó‡ÌÔÓ· ÚÔ˜ ÙËÓ ÂÚÈʤÚÂÈ·

ÙÔ˘ ıÒڷη- √ fiÁÎÔ˜ Ó‡ÌÔÓ· ˘ÔÏÔÁÈ˙fiÌÂÓÔ˜ Ì ˘ÂÚ˯ÔÁÚ¿ÊË-

Ì· Î·È Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ñ ¶ÚÔÁÂÓÓËÙÈ΋ ÂÎÙ›ÌËÛË ÙÔ˘ ÌÂÁ¤ıÔ˘˜ Ù˘ ‰È·ÊÚ·ÁÌ·ÙÔ-

΋Ï˘- ∂Ó‰ÔıˆÚ·ÎÈ΋ ÂÓÙfiÈÛË ÙÔ˘ ÛÙÔÌ¿¯Ô˘ ÛÙËÓ ·ÚÈÛÙÂÚ‹

™¢∫- ¶ÚfiÙˆÛË ÙÔ˘ ‹·ÙÔ˜- ¶ÚfiÙˆÛË ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ- ¶ÚÒÈÌË ‰È¿ÁÓˆÛË Ù˘ ™¢∫ (<25Ë Â‚‰ÔÌ¿‰· ·ËÛ˘)- ªÂÙ·ÙfiÈÛË ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘- ¢È¿ÌÂÙÚÔ˜ Ù˘ ÎÔÈÏÈ¿˜

ñ ™˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ñ ÃÚˆÌÔۈ̷ÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ñ ¶ÔÏ˘˘‰Ú¿ÌÓÈÔñ À‰ÚÔıÒڷη˜ñ ÀÔÏ·Û›· Ù˘ ·ÚÈÛÙÂÚ‹˜ ηډ›·˜ñ ¶Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·103

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¶ÚÔ‚Ï‹Ì·Ù· ÔÚıÔ‰Èο

√È ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ÛÎÂÏÂÙÔ‡, fiˆ˜ Â›Ó·È Ë ·Û˘Ì-

ÌÂÙÚ›·, ÔÈ ·Ú·ÌÔÚÊÒÛÂȘ ÙÔ˘ ıÒڷη Î·È Ë ÛÎÔ-

Ï›ˆÛË (88,89), Â›Ó·È Ù· ÈÔ Û˘ÓËıÈṲ̂ӷ ÔÚıÔ‰Èο

ÚÔ‚Ï‹Ì·Ù· Î·È ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Û˘¯ÓfiÙÂÚ· Û ÌÂ-

Á¿Ï˜ ™¢∫ Î·È Â›Ó·È Èı·ÓfiÓ Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ·ÚÁfi-

ÙÂÚ· Ì ÙËÓ ·Ó¿Ù˘ÍË. Ÿˆ˜ ·Ó·Ê¤ÚÂÙ·È ·fi ÙÔÓ

Lund, Ë ÛÎÔÏ›ˆÛË Î·È ÔÈ ·ÓˆÌ·Ï›Â˜ ÙÔ˘ ıÒڷη

ÌÔÚ› Ó· Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ·‡ÍËÛË ÙˆÓ ÂÓ‰Ôıˆ-

Ú·ÎÈÎÒÓ Î·È ÂÓ‰ÔÎÔÈÏÈ·ÎÒÓ È¤ÛÂˆÓ Î·Ù¿ ÙË ¯ÂÈ-

ÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË. √È ÂÚÈÛÛfiÙÂÚ˜ ·fi ÙȘ

·Ú·ÌÔÚÊÒÛÂȘ ·˘Ù¤˜ Â›Ó·È ‹È˜ Î·È ‰ÂÓ ¯Ú‹˙Ô˘Ó

¯ÂÈÚÔ˘ÚÁÈ΋˜ ·ÔηٿÛÙ·Û˘ (90).

£Ú¤„Ë Î·È ·Ó¿Ù˘ÍË

√È ‰È·Ù·Ú·¯¤˜ ·Ó¿Ù˘Í˘ ÔÊ›ÏÔÓÙ·È ÛÙȘ ·˘ÍË̤-

Ó˜ ÂÓÂÚÁÂȷΤ˜ ··ÈÙ‹ÛÂȘ (·˘ÍË̤ÓÔ ¤ÚÁÔ ·Ó·ÓÔ‹˜

Û ¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ÓfiÛÔ) Î·È ÛÙË Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ-

΋ ·ÏÈÓ‰ÚfiÌËÛË (°√¶), Ô˘ ·Ó·Ê¤ÚÂÙ·È ÛÙÔ 89%

ÙˆÓ ·ÛıÂÓÒÓ (91,88,92,90). ¶·Ú·ÙËÚÔ‡ÓÙ·È Û˘¯ÓfiÙÂ-

Ú· Û ·ÛıÂÓ›˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ (Ô˘ ·ÚÔ˘Û›·Û·Ó

Û˘ÌÙÒÌ·Ù· ÂÓÙfi˜ ÙˆÓ ÚÒÙˆÓ 6 ÚÒÓ ·fi ÙË Á¤ÓÓË-

ÛË) Ì ‰È¿Ù·ÛË ÔÈÛÔÊ¿ÁÔ˘ Î·È Û ·ÛıÂÓ›˜ ÛÙÔ˘˜ ÔÔ›-

Ô˘˜ ··ÈÙ‹ıËΠÂÊ·ÚÌÔÁ‹ ECMO (93,88). ∏ ‰È¿Ù·ÛË

ÙÔ˘ ÔÈÛÔÊ¿ÁÔ˘ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ÂÁÁÂÓ‹ ‰˘ÛÎÈÓËÛ›·

ÙÔ˘ ÔÚÁ¿ÓÔ˘, Ô˘ ·ÓÙ·Ó·ÎÏ¿ ÙËÓ Î·ı˘ÛÙÂÚË̤ÓË Î¤-

ÓˆÛË ÙÔ˘ Á·ÛÙÚÈÎÔ‡ ÂÚȯÔ̤ÓÔ˘ (100). À¿Ú¯Ô˘Ó

Ì·ÎÚÔÚfiıÂÛÌ· ÚÔ‚Ï‹Ì·Ù· ·fi ÙÔ Á·ÛÙÚÂÓÙÂÚÈÎfi,

fiˆ˜ ¯ÚfiÓÈ· ÔÈÛÔÊ·Á›Ùȉ· (Û¯ÂÙÈ˙fiÌÂÓË Ì °√¶) ηÈ

ηı˘ÛÙÂÚË̤ÓË ÂÓÙÂÚÈ΋ ΤӈÛË. ∏ ˘„ËÏ‹ ›وÛË

Ù˘ ÔÈÛÔÊ·Á›Ùȉ·˜ Î·È ÙÔ˘ ÔÈÛÔÊ¿ÁÔ˘ Barrett ÛÙÔ˘˜

ÂÓ‹ÏÈÎÔ˘˜ ·ÛıÂÓ›˜ Ì ÈÛÙÔÚÈÎfi Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·Á-

Ì·ÙÔ΋Ï˘ ηıÈÛÙ¿ ·Ó·Áη›· ÙËÓ ÂÊ·ÚÌÔÁ‹ ÚÔÁÚ¿Ì-

Ì·ÙÔ˜ ÂÚ·ÈÙ¤Úˆ ·Ú·ÎÔÏÔ‡ıËÛ‹˜ ÙÔ˘˜ ηٿ ÙË ‰È¿Ú-

ÎÂÈ· Ù˘ ÂÓ‹ÏÈ΢ ˙ˆ‹˜ ÙÔ˘˜ (90). ¶ÚÔÙ›ÓÔÓÙ·È ‰È·-

ÙÚÔÊ‹ ÏÔ‡ÛÈ· Û ıÂÚÌ›‰Â˜ Î·È Ê·Ú̷΢ÙÈ΋ ·ÓÙÈÌÂ-

ÙÒÈÛË Ù˘ Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘ ÌÂ

∏2-·ÔÎÏÂÈÛÙ¤˜ ˆ˜ ıÂڷ¢ÙÈο ̤ÙÚ· (61).

¡Â˘ÚÔ·Ó·Ù˘Íȷ΋ ÂͤÏÈÍË

∆· ÓÂÔÁÓ¿ Ì ™¢∫ ‚Ú›ÛÎÔÓÙ·È Û ·˘ÍË̤ÓÔ Î›Ó-

‰˘ÓÔ ˘ÔÍÈ΋˜-ÈÛ¯·ÈÌÈ΋˜ ÂÁÎÂÊ·ÏÔ¿ıÂÈ·˜. ∆· ÓÂ-

ÔÁÓ¿ Ì ÛÔ‚·Ú‹ Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›· ·ÓÙÈÌÂÙˆ-

›˙Ô˘Ó Î›Ó‰˘ÓÔ ˘ÔÍ·ÈÌ›·˜, ÔͤˆÛ˘ Î·È Ùˆ¯Ô‡

·ÂÚÈÛÌÔ‡. ∂ÁÎÂÊ·ÏÈ΋ ·Ú¿Ï˘ÛË ¤¯ÂÈ ·Ú·ÙËÚËı›

ÛÙÔ 4% (89) ÛÙÔ 5% (95) Î·È ÛÙÔ 9% ÙˆÓ ·ÛıÂÓÒÓ

(96) Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜. ÕÏϘ Ó¢ÚÔ·Ó·Ù˘ÍÈ·-

Τ˜ ÂÈÏÔΤ˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó Û·ÛÌÔ‡˜, Ì·ıËÛÈ·-

Τ˜ ‰˘ÛÎÔϛ˜, ·ÒÏÂÈ· ·ÎÔ‹˜ Î·È Î·ı˘ÛÙ¤ÚËÛË

·fiÎÙËÛ˘ ÎÈÓËÙÈÎÒÓ ‰ÂÍÈÔÙ‹ÙˆÓ (97).

ŒÎ‚·ÛË

∂›Ó·È ÁÂÓÈο ·o‰ÂÎÙfi fiÙÈ ÔÈ ·ÛıÂÓ›˜ Ì ‰È·-

ÊÚ·ÁÌ·ÙÔ΋ÏË ˙Ô˘Ó ÌÈ· Ê˘ÛÈÔÏÔÁÈ΋ ˙ˆ‹. øÛÙfiÛÔ,Û¯ÂÙÈΤ˜ ‰˘ÛÏÂÈÙÔ˘ÚÁ›Â˜, fiˆ˜ ηı˘ÛÙ¤ÚËÛË ·Ó¿-Ù˘Í˘, Ùˆ¯‹ ·Ó¿Ù˘ÍË, Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ΋ ·ÏÈÓ-‰ÚfiÌËÛË, ·ÒÏÂÈ· ·ÎÔ‹˜, Ì˘ÔÛÎÂÏÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜Î·È ·Ó·Ó¢ÛÙÈο ÚÔ‚Ï‹Ì·Ù·, ·Ú·Ì¤ÓÔ˘Ó Û ·Ú-ÎÂÙÔ‡˜ ·fi ·˘ÙÔ‡˜ (88,61,94). ∏ ·Ô˘Û›· Û˘ÓÙÔÓÈ-Ṳ̂ÓÔ˘ Ï¿ÓÔ˘ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÙˆÓ ·ÛıÂÓÒÓ ·˘-ÙÒÓ Ô‰ËÁ› Û ˘ÔÙ›ÌËÛË Ù˘ ÂÈÎÚ¿ÙËÛ˘ Û˘ÁÎÂ-ÎÚÈÌ¤ÓˆÓ ÂÈÏÔÎÒÓ Û ·ÛıÂÓ›˜ ÌÂÁ·Ï‡ÙÂÚ˘ ËÏÈ-Λ·˜ Ì ÈÛÙÔÚÈÎfi Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ (61).

™Â ÚfiÛÊ·ÙË ¤Ú¢ӷ ‚Ú¤ıËΠˆ˜ Ù· ÓÂÔÁÓ¿ Ù·ÔÔ›· ¯ÚÂÈ¿˙ÔÓÙ·Ó ıÂڷ›· ̤۷ ÛÙȘ ÚÒÙ˜ 6ÒÚ˜ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ıÓËÙfiÙËÙ· ÛÂÔÛÔÛÙfi 44%, ÂÓÒ ÌˉÂÓÈ΋ ıÓËÙfiÙËÙ· ·ÚÔ˘ÛÈ¿-˙Ô˘Ó ·˘Ù¿ Ô˘ ¯ÚÂÈ¿˙ÔÓÙ·Ó ıÂڷ›· ÌÂÙ¿ ÙȘ ÚÒ-Ù˜ 6 ÒÚ˜ Ù˘ ˙ˆ‹˜ ÙÔ˘˜ (98,99). √È Û˘ÓÔ‰¤˜ ·Óˆ-̷ϛ˜ ‰ÂÓ Â›Ó·È ˘Ô¯ÚˆÙÈο ‰Â˘ÙÂÚ‡ԢÛ˜ Î·È ËÛ˘Ó‡·ÚÍ‹ ÙÔ˘˜ ÌÔÚ› Ó· ¯ÂÈÚÔÙÂÚ‡ÂÈ ÙËÓ Úfi-ÁÓˆÛË. ∫·ıÔÚÈÛÙÈ΋˜ ÛËÌ·Û›·˜ ÁÈ· ÙËÓ ¤Î‚·ÛË ÙˆÓÓÂÔÁÓÒÓ ·˘ÙÒÓ Â›Ó·È Ë Û˘Ó‡·ÚÍË ÌÂÈ˙fiÓˆÓ ·Ó·ÙÔ-ÌÈÎÒÓ ‹ ¯ÚˆÌÔۈ̷ÙÈÎÒÓ ·ÓˆÌ·ÏÈÒÓ, Ô˘ ·˘Í¿ÓÂÈÙË ıÓËÙfiÙËÙ· ÛÙÔ 89% (36). ™ÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·-Ê›·, Ù· ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘ Û ۯ¤ÛË Ì ÙËÓ ÂÓÙfiÈ-ÛË Ù˘ ™¢∫ ÔÈΛÏÏÔ˘Ó. ŸÙ·Ó Ë ‚Ï¿‚Ë ÂÓÙÔ›˙ÂÙ·ÈÛÙÔ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ Ë ÂÈ‚›ˆÛË ·Ó¤Ú¯ÂÙ·È ÛÂ78%, fiÙ·Ó ÂÓÙÔ›˙ÂÙ·È ÛÙÔ ‰ÂÍÈfi Û 21%, ÂÓÒ Û ·Ì-ÊÔÙÂÚfiÏ¢ÚË ÂÓÙfiÈÛË ·Ó¤Ú¯ÂÙ·È Û 1% (101).¶ÈÔ ÚfiÛÊ·Ù˜ ÌÂϤÙ˜ ‰Â›¯ÓÔ˘Ó ·‡ÍËÛË (102,103)Î·È ÔÚÈṲ̂Ó˜ ÂÏ¿ÙÙˆÛË Ù˘ ıÓËÙfiÙËÙ·˜ (103) ÛÙˉÂÍÈ¿ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÂÓÒ ¿ÏϘ ‰Â›¯ÓÔ˘Ó fiÙÈ ËÂÓÙfiÈÛË Ù˘ ·ÓˆÌ·Ï›·˜ ‰ÂÓ ÂËÚ¿˙ÂÈ ÙËÓ ¤Î‚·ÛË(55). ∆· ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘ ÁÈ· ·Ï‹ ‰È·ÊÚ·ÁÌ·ÙÔ-΋ÏË Â›Ó·È ÂÚ›Ô˘ 47% Î·È ·˘Í¿ÓÔÓÙ·È ÛÙË ‰È¿Ú-ÎÂÈ· ÙÔ˘ ¯ÚfiÓÔ˘, ·Ú·Ì¤ÓÔ˘Ó fï˜ ¯·ÌËÏ¿ ÁÈ· ÙȘÂÚÈÙÒÛÂȘ fiÔ˘ Û˘Ó˘¿Ú¯Ô˘Ó Û˘ÓÔ‰¤˜ ·ÓˆÌ·-ϛ˜ (55). ÕÏϘ ÌÂϤÙ˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ˘„ËÏfiÙÂÚ·ÔÛÔÛÙ¿ ÂÈ‚›ˆÛ˘ Ô˘ Î˘Ì·›ÓÔÓÙ·È ·fi 67% (89)¤ˆ˜ 82% (104).

∏ ÚÔÛ¤ÁÁÈÛË ÙÔ˘ ÓÂÔÁÓÔ‡ Ì ™¢∫ ¤¯ÂÈ Û·ÊÒ˜·ÏÏ¿ÍÂÈ Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·. ∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿-ÁÓˆÛË ÂÈÙÚ¤ÂÈ ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÂÌ‚Ú‡Ô˘ ÁÈ· È-ı·Ó¤˜ ÂÈÚfiÛıÂÙ˜ ·ÓˆÌ·Ï›Â˜, ηıÒ˜ Î·È ÙËÓ ÂÓË-̤ڈÛË ÙˆÓ ÁÔÓ¤ˆÓ ÁÈ· ÙȘ Èı·Ó¤˜ ıÂڷ¢ÙÈΤ˜ÚÔÛÂÁÁ›ÛÂȘ Î·È ÙËÓ ÚfiÁÓˆÛË. ∏ ÂÈÎÔÈÓˆÓ›· ÌÂÙÔ˘˜ ÁÔÓ›˜ Ú¤ÂÈ Ó· ·Ú¯›˙ÂÈ ·fi ÙË ÛÙÈÁÌ‹ Ù˘ÚÔÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ Î·È Ó· Û˘Ó¯›˙ÂÙ·È Û fiÏËÙËÓ ·È‰È΋ ËÏÈΛ·.

µÈ‚ÏÈÔÁÚ·Ê›·

1. Pober BR, Lin A, Russell M, Ackerman KG, Chakravorty S,

Strauss B et al. Infants with Bochdalek diaphragmatic

hernia: Sibling precurrence and monozygotic twin

discordance in a hospital-based malformation surveillanceprogram. Am J Med Genet Part A 2005;138A:81-88.

104 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:104-000

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·104

Page 33: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

2. Pober BR. Overview of epidemiology, genetics, birth

defects and chromosome abnormalities associated with

CDH. Am J Med Part C Semin Med Genet 2007;145C:

158-171.

3. Skari H, Bjornland K, Frenckner B, Friberg LG, Heikkinen

M, Hurme T et al. Congenital diaphragmatic hernia in

Scandinavia from 1995 to 1998: predictors of mortality. J

Pediatr Surg 2002;37:1269-1275.

4. Dott MM, Wong LY, Rasmussen SA. Population-based

study of congenital diaphragmatic hernia: Risk factors

and survival in Metropolitan Atlanta, 1968-1999. Birth

Defects Res A Clin Mol Teratol 2003;67:261-267.

5. Yang W, Carmichael SL, Harris JA, Shaw GM.

Epidemiologic characteristics of congenital diaphragmatic

hernia among 2.5 million California births, 1989-1997.

Birth defects Res 2006;76:170-174.

6. Langham MRJ, Kays DW, Ledbetter DJ, Frentzen B,

Sanford LL, Richards DS. Congenital diaphragmatic

hernia. Epidemiology and outcome. Clin Perinatol

1996;23:671-688.

7. Arioni C, Bellini C, Mazzella M, Zullino E, Serra G, Toma

P. Congenital right diaphragmatic hernia. Pediatr Ratiol

2003;33:807-808.

8. Devine PC, Malone FD. Noncardiac thoracic anomalies.

Clin Perinatol 2000;4:865-899.

9. Harmath A, Hajdu J, Csaba A, Hauzman E, Pete B, Görbe E,

et al. Associated malformation in congenital diaphragmatic

hernia cases in the last 15 years in a tertiary referral institute.

A J Med Genet 2006;A:2298-2304.

10. Torfs CP, Curry CJ, Bateson TF, Honore LH. A

population-based study of congenital diaphragmatic

hernia. Teratology 1992;46:555-565.

11. Crane JP. Familian congenital diaphragmatic hernia:

Prenatal diagnostic approach and analysis of twelve

families. Clin Genet 1979:16:244-252.

12. Allan DW, Greer JJ. Embryogenesis of the phrenic nerve and

diaphragm in the fetal. J Comp Neurol 1997;382:459-468.

13. Slavotinek A, Warmerdam B, Lin A, Shaw G. Population-

based analysis of left and right-sided diaphragmatic hernia

demonstrates different frequencies of selected additional

anomalies. A J Med Genet Part A. 2007;143A:3127-3136.

14. Wolff G. Familiar congenital diaphragmatic defect:

Review and conclusions. Hum Genet 1980;54:1-5.

15. Tibboel D, Gaag AV. Etiologic and genetic factors in

congenital diaphragmatic hernia. Clin Perinatol 1996;23:

689-699.

16. Norio R, Kaariainen H, Rapola J, Herva R, Kekomari M.

Familial congenital diaphragmatic defects: aspects of

etiology, prenatal diagnosis and treatment. Am J Med

Genet 1984;17:471-483.

17. Smith DW. Recognizable Patterns of Human Malfor-

mation. Genetic, Embryologic and Clinical Aspects. 3

Philadelphia PA: W.B Saunders. 1982;7:1-653.

18. Enns GM, Cox VA, Goldstein RB, Gibbs DL, Harrison

MR, Golabi M. Congenital diaphragmatic defects and

associated syndromes, malformations and chromosome

anomalies: a retrospective study of 60 patients and

literature review. Am J Med Genet 1998;79:215-225.

19. Kantarci S, Casavant D, Prada C, Russell M, Byrne J, Haug

L, et al. Findings from aCGH in patients with congenital

diaphragmatic hernia: a possible locus for Fryns syndrome.

A J Med Genet 2006;140A:17-23.

20. Slavotinek A, Lee SS, Davis R, Shrit A, Leppig KA, Rhim J,

et al. Fryns syndrome phenotype caused by chromosome

microdeletions at 15q26.2 and 8p23.1. J Med Genet

2005;42:730-736.

21. Philip N, Gambarelli D, Guys JM, Camboulives J, Ayme S.

Epidemiological study of congenital diaphragmatic defects

with special reference to etiology. Eur J Pediatr 1991;150:

726-729.

22. Clerk RD, Fenner-Gonzales M. Apparent Fryns syndrome

in a boy with a tandem duplication of 1q24-31.2. Am J

Genet 1989;34:422-426.

23. Dean JC, Couzin DA, Gray ES, Lioyd DJ, Stephen GS.

Apparent Fryns syndrome and aneuploidy: evidence for a

disturbance of the midline developmental field. Clin

Genet 1991;40:349-352.

24. Nordenskjold A, Tapper-Persson M, Anvret M. No

evidence of WT1 gene mutations in children with

congenital diaphragmatic hernia. J Pediatr Surg 1996;

31:925-927.

25. Biggio JR, Descartes MD, Carroll AJ, Holt RL. Congenital

diaphragmatic hernia: Is 15q26.1-26.2 a candidate locus?

Am J Med Genet Part A 2004;126A:183-185.

26. Greer JJ, Babiuk P, Thebaud B. Etiology of congenital

diaphragmatic hernia: the retinoid hypothesis. Pediatr Res

2003;53:726-730.

27. Cushman L, Martinez W, Cherry A, Manning M, Abdul-

Rahman O, Anderson C, et al. A J Med Genet 2005;137A:

65-71.

28. Fauza DO, Wilson JM. Congenital diaphragmatic hernia

and associated anomalies: their incidence, identification

and impact on prognosis. J Pediatr Surg 1994;29:1113-

1117.

29. Lurie IW. Where to look for the genes related to

diaphragmatic hernia? Genet Couns 2003;14:75-93.

30. Mendelsohn C, Lohnes D, Decimo D, Lufkin T, LeMeur

M, Chambon P, et al. Function of the retinoic acid

receptors (RARs) during development: (ππ) multiple

abnormalities at various stages of organogenesis in RAR

double mutants. Development 1994;120:2749-2771.

31. Chen MH, McGowan A, Ward S, Bsvik C, Greer JJ. The

activation of the retinoic acid response element is

inhibited in an animal model of congenital diaphragmatic

hernia. Biol Neonate 2003;83:157-161.

32. Major D, Cadenas M, Fournier L, Leclerc S, Lefebre M,

Cloutier R. Retinal status of newborn infants with

congenital diaphragmatic hernia. Pediatr Surg Int

1998;13:547-549.

33. Jesudason EC, Smith NP, Connell, Spiller D, White M,

Fernig D, et al. Peristalsis of airway smooth muscle is

developmentally regulated and uncoupled from hypo-

plastic lung growth. Am J Physiol Lung Cell Mol Physiol

2006;291:559-565.

34. Boucherat O, Benachi A, Barlier-Mur A, Franco-Montoya

M, Martinovic J, Thebaud B, et al. Decreased lung

fibroblast growth factor 18 and elastin in human

congenital diaphragmatic hernia and animal models. Am

J Respir Crit Care Med 2004;175:1066-1077.

35. Wilcox DT, Irish MS, Holm BA, Glick PL. Animal models

in congenital diaphragmatic hernia. Clin Perinatol

1996;23:813-822.

36. Keijzer R, Liu J, Deimling J, Tibboel D, Post M. Dual-hit

hypothesis explains pulmonary hypoplasia in the nitrofen

105™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË

¶·È‰È·ÙÚÈ΋ 2009;72:105-000

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Page 34: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

model of congenital diaphragmatic hernia. Am J Pathol

2000;156:1299-1306.

37. Glick PL, Leach CL, Besner GE, Equan EA, Morin

FC,Robinson LK, et al. Pathophysiology of congenital

diaphragmatic hernia III: exogenous surfactant therapy

for the high-risk neonate with CDH. J Pediatr Surg

1992;27:866.

38. Bohn DJ, Lames I, Filler RM, Ein SH, Wesson DE,

Shandling B, et al. The relationship between PaCO2 and

ventilation parameters in predicting survival in congenital

diaphragmatic hernia. J Pediatr Surg 1984;19:666-671.

39. Weber TR, Tracy T, Bailey PV, Lewis JE. Congenital

diaphragmatic hernia beyond infancy. Am J Surg 1991;

162:643-646.

40. Adzick NS, Harrison MR, Glick PL, Villa RL, Finkbeiner

W. Experimental pulmonary hypoplasia and oligo-

hydramnios: relative contributions of lung fluid and fetal

breathing movements. J Pediatr Surg 1984;19:658-665.

41. Shochat SJ. Pulmonary vascular pathology in congenital

diaphragmatic hernia. Pediatr Surg Int 1987;2:331-334.

42. Crawford DC, Wright VM, Drake DP, Allan LD. Fetal

diaphragmatic hernia: the value of fetal echocardiography

in the prediction of postnatal outcome. Br J Obstet

Gynaecol 1989;96:705-710.

43. Schwartz SM, Vermilion RP, Hirschl RB. Evaluation of

left ventricular mass in children with left-sided congenital

diaphragmatic hernia. J Pediatr 1994;125:447-451.

44. Nakamura Y, Yamamoto I, Fukuda S, Hashimoto T.

Pulmonary acinar development in diaphragmatic hernia.

Arch Pathol Lab Med 1991;115:372-376.

45. Ijsslstijn H, Zimmermann LJ, Bunt JE, de Jongste JC,

Tibboel D. Prospective evaluation of surfactant compo-

sition in bronchoalveolar lavage fluid of infants with

congenital diaphragmatic hernia and of age-matched

controls. Crit Care Med 1998;26:573-580.

46. Berk C, Grundy M. High risk lecithin/sphingomyelin

ratios associated with neonatal diaphragmatic hernia: case

report. Br J Obstet Gynaecol 1982;89:250-251.

47. Moya FR, Thomas VL, Romaguera J, Mysore MR,

Maberry M, Bernard A, et al. Fetal lung maturation in

congenital diaphragmatic hernia. Am J Obstet Gynecol

1995;173: 1401-1405.

48. Sullivan KM, Hawgood S, Flake AW, Harrison MR,

Adzick NS. Amniotic fluid phospholipid analysis in the

fetus with congenital diaphragmatic hernia. J Pediatr Surg

1994;29: 1020-1023.

49. Cogo P, Zimmermann L, Rosso F, Tormera F, Gamba P,

Verlato G, et al. Surfactant synthesis and kinetics in

infants with congenital diaphragmatic hernia. Am Respir

Crit Care Med 2002;40:154-158.

50. Godinez MH, Godinez RI. Inspired oxygen concentration

alters the phospholipid and protein content in the

bronchoalveolar lavage-accessible space. Crit Care Med

1996;24:862-869.

51. Hagen EO. Drugs and congenital abnormalities. Lancet.

1963; I:501.

52. Sievers G, Schradle-Beielstein H. Drugs and congenital

abnormalities. Lancet. 1963; I:330.

53. Higuchi R, Minami T, Shimoyamada Y, Kamisako H,

Koike M, Takigawa H, et al. Diaphragmatic hernia in an

infant of a diabetic mother: an unusual association in

diabetic embryopathy. Pediatr Int 1999;41:581-583.

54. Solari V, Puri P. Glucocorticoid receptor gene expression

in the hypoplastic lung of newborns with congenital

diaphragmatic hernia. J Pediatr Surg 2002;37:715-718.

55. Gallot D, Boda C, Ughetto S, Perthus I, Robert-Gnansia E,

Francannet C, et al. Prenatal detection and outcome of

congenital diaphragmatic hernia: a French registry-based

study. Ultrasound Obstet Gynecol 2007;29:276-283.

56. Game E, Haeusler M, Barisic I, Gjergia R, Stoll C,

Clementi M. Congenital diaphragmatic hernia; evaluation

of prenatal diagnosis in 20 European regions. Ultrasound

Obstet Gynecol 2002;19:329-333.

57. Grandjean H, Larroque D, Levi S. The performance of

routine ultrasonographic screening of pregnancies in the

Eurofetus Study. Am J Ostet Gynecol 1999;181:446-454.

58. Newman BM, Afshari E, Karp,MP, Jewett TC Jr, Cooney

DR. Presentation of congenital diaphragmatic hernia post

the neonatal period. Arch Surg 1986;121:813-816.

59. Snyder HS, Salo DF, Kelly PH. Congenital diaphragmatic

hernia presenting as massive gastrothorax. Ann Emerg

Med 1990;19:562-56.

60. Mei-Zahav M, Solomon M, Trachsel D, Langer JC.

Bochdalek diaphragmatic hernia: not only a neonatal

disease. Arch Dis Child 2003;88:532-535.

61. Muratore C, Utter S, Jaksic T, Lund D, Wilson J. Nutri-

tional morbidity in survivors of congenital diaphragmatic

hernia. J Pediatr Surg 2001;36:1171-1176.

62. Baglaj M, Dorobisz U. Late presenting congenital

diaphragmatic hernia in children: a literature review.

Pediatr Radiol 2005;35:478-488.

63. Katz AL, Wiswell TE, Baumgact S. Contemporary contro-

versies in the management of congenital diaphragmatic

hernia. Clin Perinatol 1998;25:219-248.

64. Finer NN, Tierney A, Etchers PC, Peliowski A, Ainsworth

W. Congenital diaphragmatic hernia: developing a

protocolixed approach. J Pediatr Surg 1998;33:1331-1337.

65. Kinsalla LP, Abma SH. Recent developments in the

pathophysiology and treatment of persistent pulmonary

hypertension of the newborn. J Pediatr 1995;126:853-64.

66. Paranka MS, Walsh WF, Stancombe BB. Surfactant lavage

in a piglet model of meconium aspiration syndrome.

Pediatr Res 1992;31:625.

67. Baumgart S, Hirschl RB, Butler SZ, Coburn CE, Spitzer

AR. Diagnosis-related criteria in the consideration of

extracorporeal membrane oxygenation in neonates

previously treated with high frequency jet ventilation.

Pediatr 1992;89:491-494.

68. O’Tool SJ, Karamnukian HL, Morin FC, Holm BA, Egan

EA, Azizkhan RG, et al. Surfactant decreases pulmonary

vascular resistant and increases pulmonary blood flow in

the fetal lamb model of congenital diaphragmatic hernia.

J Pediatr Surg 1996;31:507-511.

69. Lotze A, Mitchell BR, Bulas DI, Zola EM, Shalwitz RA,

Gunkel JH. Multicenter study of surfactant (beractant)

use in the treatment of term infants with severe

respiratory failure. Survanta in Term Infants Study

Group. J Pediatr 1998;132:40-47.

70. Merces S. Factors involved in chylothorax following

repair of congenital diaphragmatic hernia. J Pediatr Surg

1986;21:809-811.

71. Naik S, Greenough A, Zhang Y, Davenport M. Prediction

of morbidity during infancy after repair of congenital

diaphragmatic hernia. J Pediatr 1996;31:1651-1654.

106 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:106-000

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·106

Page 35: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

72. Hatch EL Jr, Kendall J, Blumhagen J. Stomach position asan in utero predictor of neonatal outcome in left-sideddiaphragmatic hernia. J Pediatr Surg 1992;27:778-779.

73. Doyle NM, Lally KP. The CDH Study Group andadvances in the clinical care of the patient with congenitaldiaphragmatic hernia. Semin Perinatol 2004;28:174-184.

74. Bath ME, Jesudason EC, Losty PD. How useful is the lung-to-head ratio in predicting outcome in the fetus withcongenital diaphragmatic hernia? ∞ systematic review andmeta-analysis. Ultrasound Obstet Gynecol 2007;30:897-906.

75. Laudy JA, Van Gucht M, Van Dooren MF, Wladimiroff JW,Tibboel D. Congenital diaphragmatic hernia; an evaluationof the prognostic value of the lung-to-head ratio and otherprenatal parameters. Prenat Diagn 2003;23:634-639.

76. Lipshutz GS, Albanese CT, Feldstein VA, Jennings RW,Housley HT, Beech R, et al. Prospective analysis of lung-to-head ratio predicts survival for patients with prenatallydiagnosed congenital diaphragmatic hernia. J PediatrSurg 1997;32:1634-1636.

77. Thebaud B, Azancot A, de Lagaisie P, Vuillard E, FerkadjiL, Benali K, et al. Congenital diaphragmatic hernia:antenatal prognostic factor. Does cardiac ventriculardisproportion in utero predict outcome and pulmonaryhypoplasia? Intensive Care Med 1997;23:10062-10069.

78. Chaoui R, Kalache K, Tennstedt C, Lenz F, Vogel M.Pulmonary arterial Doppler velocimetry in fetuses withlung hypoplasia. Eur J Obstet Gynocol Reprod Biol 1999;84:179-185.

79. Levine D, Barnewolt CE, Mehta TS, Trop I, Estroff J,Wong G. Fetal thoracic abnormalities: MR imaging.Radiology 2003;228:379-388.

80. Donnelly LF, Sakurai M, Klosterman LA, Delong DM,Strife JL. Correlation between findings on chest radio-graphy and survival in neonates with congenital diaphra-gmatic hernia. AJR Am J Proentgenol 1999;173:1589-1593.

81. Bos AP, Hussain SM, Hazebroek FWJ, Tibboel D, MeradjiM, Molenaar. Radiographic evidence of broncho-pulmonary dyplasia in high-risk congenital diaphragmatichernia survivors. Pediatr Pulmonol 1993;15:231-234.

82. Koumbourlis A, Wung J, Stolar C. Lung function ininfants after repair of congenital diaphragmatic hernia. JPediatr Surg 2006;41:1716-1721.

83. Marven SS, Smith CM, Claxton D,Chapman J, DaviesHA, Primhak PA, et al. Pulmonary function, exerciseperformance, and growth in survivors of congenitaldiaphragmatic hernia. Arch Dis Child 1998;78:137-142.

84. Wischermann A, Holschneider AM, Hubner U. Long-term follow-up of children with diaphragmatic hernia.Eur J Pediatr Surg 1995;5:13-18.

85. Rasheed A, Tindall S, Cueny DL, Klein MD, Delaney-Black V. Neurodevelopmental outcome after congenitaldiaphragmatic hernia: extracorporeal membrane oxyge-nation before and after surgery. J Pediatr Surg 2001;36:539-544.

86. Cheuhg PY, Tyebkhan LM, Peliowski A, Ainsworth W,Robertson CMT. Prolonged use of pancuronium bromideand sensorineural hearing loss in childhood survivors ofcongenital diaphragmatic hernia. J Pediatr 1999;135: 233-239.

87. ªasumoto K, Nagata K, Uesugi T, Yamada T, Taguchi T.Risk factors for sensorineural hearing loss in survivorswith severe congenital diaphragmatic hernia. Eur J Pediatr2007;166:607-612.

88. Lund DP, Mitchell J, Kharasch V, Quigley S, Kuehn M,Wilson JM. Congenital diaphragmatic hernia: the hiddenmorbidity. J Pediatr Surg 1994;29:258-264.

89. Jaillard S, Pierrat V, Dubois A, Truffert P, Lequien P,Wurtz A, et al. Outcome at 2 years of infants withcongenital diaphragmatic hernia: a population-basedstudy. Ann Thorac Surg 2003;75:250-256.

90. Vanamo K, Peltonen J, Rintala R, Lindahl H, JaaskelainenJ, Louhimoo I. Chest wall and spinal deformities in adultswith congenital diaphragmatic defects. J Pediatr Surg1996; 31:851-854.

91. Van Meurs KP, Robbins ST, Reed VL, Karr SS, WagnerAE, Glass P, et al. Congenital diaphragmatic hernia; long-term outcome in neonates treated with extracorporealmembrane oxygenation. J Pediatr 1993;122:893-899.

92. Cohen MD, Beck LM. Hiatus hernia: a complication ofpostero-lateral diaphragmatic hernia (Bochdalek hernia)in infants. Clin Ratiol 1980;31:215-219.

93. Koot VCM, Bergmeijer JH, Bos AP, Molennar JC.Incidence and management of gastroesophageal refluxafter repair of congenital diaphragmatic hernia. J PediatrSurg 1993;28:48-52.

94. Stolar CJ. What do survivors of congenital diaphragmatichernia look kike when they grow up? Semin Pediatr Surg1996;5:275-279.

95. Glass P, Wagner AE, Papero PH, Rajasingham SR,Civitello LA, Kjaer MS, et al. Neurodevelopmental statusat age five years of neonates treated with extracorporealmembrane oxygenation. J Pediatr 1995;127:447-457.

96. Graziani LJ, Gringlas M, Baumgart S. Celebrovascularcomplications and neurodavelopment sequelae ofneonatal ECMO. Clin Perinatol 1997;24:655-674.

97. McGahren ED, Mallik K, Rodgers BM. Neurologicaloutcome is diminished in survivors of congenitaldiaphragmatic hernia requiring exrtacorporeal membraneoxygenation. J Pediatr Surg 1997;32:1216-1220.

98. Collins DL, Pomerance JJ, Travis KW, Turner SW,Pappelbaum SJ. A new approach to congenital postero-lateral diaphragmatic hernia. J Pediatr Surg 1977; 12:149-156.

99. Skari H, Bjornland K, Haugen G, Egeland T, Emblem R.Congenital diaphragmatic hernia: A meta-analysis ofmortality factors. J Pediatr Surg 2000;35:1187-1197.

100. Wung JT, Sahni R, Moffitt ST, Lipsitz E, Stolar CJ.Congenital diaphragmatic hernia: survival treated withvery delayed surgery, spontaneous respiration and nochest tube. J Pediatr Surg 1995;30:406-409.

101. Clark WR, Nieman GF, Thomposon JE, Paskanik AM,Rokhar JE, Bredenberg CE. Surfactant displacement bymecomium free fatty acids: an alternative explanation foratelectasis in meconiun aspiration syndrome. J Pediatr1987;110:765-770.

102. Geary M. Management of congenital diaphragmatichernia diagnosed prenatally: an update. Prenat Diagn1998;18:1155-1158.

103. Macken MB, Thompson DL. Antenatal diagnosis ofcongenital diaphragmatic hernia. Can Assoc Radiol J1993;44:439-442.

104. Sreenan C, Etches P, Osiovich H. The western Canadialexperience with congenital diaphragmatic hernia:perinatal factors predictive of extracorporeal membraneoxygenation and death. Pediatr Surg Int 2001;17:196-200.

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108 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:108-000

Quiz

1. H ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Ù˘ Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘:·. ¢ÂÓ Â›Ó·È ‰˘Ó·Ù‹‚. °›ÓÂÙ·È ÌÂÙ¿ ÙËÓ 24Ë Â‚‰ÔÌ¿‰· ·ËÛ˘ Ì ¯Ú‹ÛË ˘ÂÚ‹¯ˆÓÁ. °›ÓÂÙ·È ÌfiÓÔ ÛÙÔ 10% ÙˆÓ ÂÚÈÙÒÛˆӉ. √È Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ‰˘ÛÎÔÏÂ‡Ô˘Ó ÙË ‰È¿ÁÓˆÛË

2. ∏ ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ· ÓÂÔÁÓÔ‡ ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ·Ì¤Ûˆ˜ ÌÂÙ¿ ÙË Á¤ÓÓËÛË ÔÊ›ÏÂÙ·È ÛÂ:·. ¶Ó¢ÌÔÓÈ΋ ˘ÔÏ·Û›·‚. §Ô›ÌˆÍËÁ. ¶Ó¢ÌÔıÒڷη‰. ¶Ó¢ÌÔÓÈ΋ ·ÈÌÔÚÚ·Á›·

3. ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek ÂÓÙÔ›˙ÂÙ·È Û˘¯ÓfiÙÂÚ·:·. ¢ÂÍÈ¿‚. ∞ÚÈÛÙÂÚ¿Á. ∞ÌÊÔÙÂÚfiÏ¢ڷ‰. ∫ÂÓÙÚÈο

4. ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÂΉËÏÒÓÂÙ·È Û˘¯ÓfiÙÂÚ· ÌÂ Û˘ÌÙÒÌ·Ù· ·fi:·. ∆Ô Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ·‚. ∆Ô ÎÂÓÙÚÈÎfi Ó¢ÚÈÎfi Û‡ÛÙËÌ·Á. ∂›Ó·È ·Û˘Ìو̷ÙÈ΋‰. ∆Ô ·Ó·Ó¢ÛÙÈÎfi Û‡ÛÙËÌ·

5. ™ÙË Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Û˘¯ÓfiÙÂÚ· Û˘Ó˘¿Ú¯Ô˘Ó ·ÓˆÌ·Ï›Â˜ ·fi:·. ∆Ô Á·ÛÙÚÂÓÙÂÚÈÎfi Û‡ÛÙËÌ·‚. ∆Ô Ô˘ÚÔÁÂÓÓËÙÈÎfi Û‡ÛÙËÌ·Á. ∆Ô Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ·‰. ∆ÔÓ ÛÎÂÏÂÙfi

√È ÛˆÛÙ¤˜ ··ÓÙ‹ÛÂȘ ÛÙË ÛÂÏ. 122

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∂›‰Ú·ÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì˘ÂÏÔ‡ ÔÛÙÒÓ

ÛÙËÓ ex vivo ¤ÎÙ˘ÍË ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜*

π. ¶ÂÏ·ÁÈ¿‰Ë˜, ∂. ¢ËÌËÙÚ›Ô˘, ∂. ™ÙÂȷοÎË, Ã. ¶ÂÚ‰ÈÎÔÁÈ¿ÓÓË, ∂. ÷Ù˙ˉ¿ÎË, ª. ∫·ÏÌ·ÓÙ‹

¶ÂÚ›ÏË„Ë

∂ÈÛ·ÁˆÁ‹: ∆· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ˆ˜ ̤ÚÔ˜ ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ Ì˘ÂÏÈÎÔ‡ ÌÈÎÚÔÂÚÈ‚¿ÏÏÔÓÙÔ˜, ı·ÌÔÚÔ‡Û·Ó Ó· ÚÔ¿ÁÔ˘Ó ÙË ‰È·‰Èηۛ· ¤ÎÙ˘Í˘ ·Ú¯¤ÁÔÓˆÓ/ÚÔÁÔÓÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·›Ì·ÙÔ˜ ÔÌÊ¿ÏÈÔ˘ÏÒÚÔ˘, Ô˘ ·ÔÙÂÏ› ‡ÎÔÏ· ÚÔÛ‚¿ÛÈÌË ËÁ‹ ΢ÙÙ¿ÚˆÓ ÁÈ· ÌÂÙ·ÌfiÛ¯Â˘ÛË, ·ÏÏ¿ ··ÈÙÂ›Ù·È ex vivo

¤ÎÙ˘Í‹ ÙÔ˘˜ ÁÈ· ¯Ú‹ÛË Û ·È‰È¿ ÌÂÁ·Ï‡ÙÂÚÔ˘ ۈ̷ÙÈÎÔ‡ ‚¿ÚÔ˘˜ Î·È Û ÂÓ‹ÏÈΘ. ™ÎÔfi˜ Ù˘ ÌÂϤÙ˘‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘ ·ÏÏËÏ›‰Ú·Û˘ ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ ·›Ì·ÙÔ˜ ÔÌÊ¿ÏÈÔ˘ ÏÒÚÔ˘ (√§) Ì ÌÂÛÂÁ-¯˘Ì·ÙÈο ·ÙÙ·Ú· (ª∫) Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ (ª√) ·È‰ÈÒÓ Î·È Ë Û‡ÁÎÚÈÛË Ì ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë Â›‰Ú·ÛËÛ˘Ó‰˘·ÛÌÔ‡ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ: ÃÚËÛÈÌÔÔÈ‹ıËÎ·Ó Î‡ÙÙ·Ú· CD34+ Ô˘ ·ÔÌÔÓÒıËÎ·Ó ·fi ·›Ì· √§ (n=10) Î·È ª∫Ô˘ ·ÔÌÔÓÒıËÎ·Ó ·fi ª√ ·È‰ÈÒÓ Ì ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· (n=9). ∆· ·ÙÙ·Ú· Ù¤ıËηÓÛ ‰È·ÊÔÚÂÙÈΤ˜ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜, ·ÚÔ˘Û›· ‹ ÌË Û˘Ó‰˘·ÛÌÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ηȤÁÈÓ ·ÓÔÛÔÊ·ÈÓÔÙ˘ÈÎfi˜ ¯·Ú·ÎÙËÚÈÛÌfi˜, ·ÍÈÔÏfiÁËÛË Ù˘ ¤ÎÙ˘Í˘ Î·È ÂÎÙ›ÌËÛË Ù˘ ÎψÓÔÁÔÓÈÎfiÙËÙ·˜(·Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ CFU-GM Î·È BFU-E) ÙˆÓ CD34+ ΢ÙÙÙ¿ÚˆÓ.

∞ÔÙÂϤÛÌ·Ù·: ∏ ·ÚÔ˘Û›· ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· ÙˆÓ CD34+, ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔ-ÓÙ˜, ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙË ÌÂÁ·Ï‡ÙÂÚË ¤ÎÙ˘ÍË ÙfiÛÔ ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÙÙ¿ÚˆÓ fiÛÔ Î·ÈÙÔ˘ ·ÚÈıÌÔ‡ ÙˆÓ CD34+. ∏ ·ÚÔ˘Û›· ª∫ ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ¤‰ˆÛ ÙÔ ˘„ËÏfiÙÂÚÔ ÔÛÔ-ÛÙfi ÙˆÓ ˘ÔÏËı˘ÛÌÒÓ CD34+CD38-, CD34+CD33-, ηıÒ˜ Î·È CD34 Ô˘ ıˆÚÔ‡ÓÙ·È Î·È ÔÈ Ï¤ÔÓ ·Ú-¯¤ÁÔÓÔÈ. ∂ÈϤÔÓ, Û ·˘Ù‹ ÙË Û˘Óı‹ÎË Ù· ÂÎÙ˘Á̤ӷ ·ÙÙ·Ú· ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ˘„ËÏ‹ ÎψÓÔÁÔ-ÓÈÎfiÙËÙ·, ·ÊÔ‡ ¤‰ˆÛ·Ó ÙÔÓ ÌÂÁ·Ï‡ÙÂÚÔ ·ÚÈıÌfi ·ÔÈÎÈÒÓ ÙfiÛÔ ÎÔÎÎÈÒ‰Ô˘˜ fiÛÔ Î·È ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜.

™˘ÌÂÚ¿ÛÌ·Ù·: ™˘ÌÂÚ·ÛÌ·ÙÈο, Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· ª√ ˘ÔÛÙËÚ›˙Ô˘Ó ÙËÓ ¤ÎÙ˘ÍË Î·È Û˘Ì-‚¿ÏÏÔ˘Ó ÛÙË ‰È·Ù‹ÚËÛË Ù˘ ·ˆÚfiÙËÙ·˜ ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ ÛÂ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜, ·Ú¤¯Ô-ÓÙ·˜ ÏËı˘ÛÌfi Ô˘ Ê·›ÓÂÙ·È Ó· ‰È·ı¤ÙÂÈ ¯·Ú·ÎÙËÚÈÛÙÈο Ô˘ ÙÔÓ Î·ıÈÛÙÔ‡Ó Î·Ù¿ÏÏËÏÔ ÁÈ· ¯Ú‹ÛË ÙÔ˘Û ÌÂÙ·ÌfiÛ¯Â˘ÛË.

§¤ÍÂȘ ÎÏÂȉȿ: CD34+, ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ex vivo ¤ÎÙ˘ÍË, ·›Ì· ÔÌÊ¿ÏÈÔ˘ ÏÒÚÔ˘, Ì˘ÂÏfi˜ ÔÛÙÒÓ ·È‰ÈÒÓ.

The effect of paediatric bone-marrow-derived stromal cells on the

ex-vivo expansion of cord-blood haemopoietic stem cells

I. Pelagiadis, H. Dimitriou, E. Stiakaki, C. Perdikogianni, E. Hatzidaki, M. Kalmanti

Abstract

Background: A major limitation of the use of umbilical cord blood (UCB) haematopoietic stem cells (HSC)for allogeneic transplantation is the insufficient numbers of cells available in the graft. In order to overcomethis restriction, ex-vivo expansion of HSC has been considered. Mesenchymal stromal cells (MSC)constitute an essential element of the normal bone marrow (BM) microenvironment, participating in theregulation of haematopoiesis. The aim of this study was to evaluate the interaction between UCB CD34+

cells and MSC used as a feeder layer and to compare it with the corresponding effect of different growthfactor (GF) combinations on the expansion of HSC.

Methods: CD34+ HSC from the UCB of full term deliveries (n=10) and MSC isolated and expanded fromthe BM of children were co-cultured under different conditions in the presence or absence of GFcombinations. The expansion capability was evaluated, the various populations were immuno-phenotypically characterized and HSC clonogenicity (expressed as the development-formation of CFU-GM/BFU-E colonies) was assessed after 10 days of culture.

Results: CD34+ UCB cells cultured in the presence of both MSC and either GF combination resulted in ahigher expansion of both total cell number and CD34+ cells. Those cultured on the MSC feeder layer onlyyielded a higher percentage of CD34+, CD34+CD38- and CD34+CD33- subpopulations, and enhanced CFU-GM and BFU-E colony development.

109∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞ ORIGINAL ARTICLE

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜.¶·ÓÂÈÛÙËÌÈ·Îfi¡ÔÛÔÎÔÌÂ›Ô ∏Ú·ÎÏ›Ԣ,∆Ì‹Ì· π·ÙÚÈ΋˜¶·ÓÂÈÛÙËÌ›Ô˘ ∫Ú‹Ù˘,∏Ú¿ÎÏÂÈÔ, ∫Ú‹ÙË

AÏÏËÏÔÁÚ·Ê›·:

ª·Ú›· ∫·ÏÌ·ÓÙ‹ [email protected]¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÈÌ·ÙÔÏÔÁ›·˜-√ÁÎÔÏÔÁ›·˜,¶·ÓÂÈÛÙËÌÈ·Îfi ¡ÔÛÔÎÔÌ›Ô∏Ú·ÎÏ›Ԣ, 71110 ∏Ú¿ÎÏÂÈÔ∫Ú‹Ù˘

Department of PaediatricHaematology-Oncology,University Hospital ofHeraklion, University of CreteMedical School, Heraklion,Crete, Greece

Correspondence:

Maria [email protected] of PaediatricHaematology-Oncology,University Hospital ofHeraklion, 71110, Heraklion,Crete, Greece

¶·È‰È·ÙÚÈ΋ 2009;72:109-000

* ∏ ÂÚÁ·Û›· ·˘Ù‹ ‚Ú·‚‡ıËΠ̠ÙÔ A’ XˆÚ¤ÌÂÈÔ Œ·ıÏÔ, ηٿ ÙÔ 46Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ∫¤Ú΢ڷ 2008

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·109

Page 38: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

∂ÈÛ·ÁˆÁ‹

∆Ô ÔÌÊ·ÏÈÔÏ·ÎÔ˘ÓÙÈ·Îfi ·›Ì· (√§) ·Ô-

ÙÂÏ› ‡ÎÔÏ· ÚÔÛ‚¿ÛÈÌË ËÁ‹ ÁÈ· ÌÂÙ·Ìfi-

Û¯Â˘ÛË Î·È ‰È·ı¤ÙÂÈ ÛËÌ·ÓÙÈο ÏÂÔÓÂÎÙ‹Ì·-

Ù·, ‰ËÏ. ÌÂÁ·Ï‡ÙÂÚË ÂÚÈÂÎÙÈÎfiÙËÙ· Û ÚÔÁÔ-

ÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú·, ÌÈÎÚfiÙÂÚÔ ÔÛÔ-

ÛÙfi ÂÌÊ¿ÓÈÛ˘ GVHD Î·È ‰˘Ó·ÙfiÙËÙ· ÌÂÙ·Ìfi-

Û¯Â˘Û˘ Û ÌË Û˘Ì‚·Ùfi ‰¤ÎÙË (1-2 HLA ·ÓÙÈ-

ÁfiÓ·) (1-3). ∆·˘Ùfi¯ÚÔÓ·, fï˜, Ô Û¯ÂÙÈο ÌÈ-

ÎÚfi˜ ·ÚÈıÌfi˜ ΢ÙÙ¿ÚˆÓ Ô˘ ÂÚȤ¯ÔÓÙ·È ·Ó¿

ÌÔÓ¿‰· ·›Ì·ÙÔ˜ ÏÒÚÔ˘ ηıÈÛÙ¿ Ú·ÎÙÈο ·‰‡-

Ó·ÙË ÙË ¯ÔÚ‹ÁËÛ‹ ÙÔ˘ Û ·È‰È¿ Ì ۈ̷ÙÈÎfi

‚¿ÚÔ˜ ÌÂÁ·Ï‡ÙÂÚÔ ÙˆÓ 30 ÎÈÏÒÓ (4,5).

ªÂ ÛÎÔfi ÙËÓ Â˘Ú‡ÙÂÚË ¯Ú‹ÛË ·›Ì·ÙÔ˜ √§

ÛÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË, Á›ÓÔÓÙ·È ÚÔÛ¿ıÂȘ ÁÈ· ex

vivo ¤ÎÙ˘ÍË Î˘ÙÙ¿ÚˆÓ. √È ‰È·‰Èηۛ˜ ¤ÎÙ˘-

͢ ̤¯ÚÈ ÚfiÛÊ·Ù· ÂÚÈÏ¿Ì‚·Ó·Ó ÙË ¯Ú‹ÛË

‰È·ÊÔÚÂÙÈÎÒÓ Û˘Ó‰˘·ÛÌÒÓ ·˘ÍËÙÈÎÒÓ ·Ú·Áfi-

ÓÙˆÓ, ÔÈ Ôԛ˜ fï˜ ηٷϋÁÔ˘Ó Û ۯÂÙÈο

‰È·ÊÔÚÔÔÈË̤ÓÔ˘˜ ΢ÙÙ·ÚÈÎÔ‡˜ ˘ÔÏËı˘-

ÛÌÔ‡˜ (6-8). ¶ÚfiÛÊ·Ù· ÍÂΛÓËÛÂ Ë ‰ÈÂÚ‡ÓËÛË

Ù˘ ‰˘Ó·ÙfiÙËÙ·˜ ¯Ú‹Û˘ ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘Ù-

Ù¿ÚˆÓ (ª∫) ÛÙÚÒÌ·ÙÔ˜ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ

(ª√), ηıÒ˜ Î·È ÂÓ‰ÔıËÏÈ·ÎÒÓ Î˘ÙÙ¿ÚˆÓ, ÁÈ·

ÙË ‚ÂÏÙ›ˆÛË Ù˘ ex vivo ¤ÎÙ˘Í˘ (9-13).

∆· ª∫ ·ÔÙÂÏÔ‡Ó Ì¤ÚÔ˜ ÙÔ˘ ÌÈÎÚÔÂÚÈ-

‚¿ÏÏÔÓÙÔ˜ ÙÔ˘ ª√, ÙÔ ÔÔ›Ô Û˘ÌÌÂÙ¤¯ÂÈ ÛÙË

Ú‡ıÌÈÛË Ù˘ ·ÈÌÔÔ›ËÛ˘. √ ª√ ·ÔÙÂÏ› ÙËÓ

ÂÚÈÛÛfiÙÂÚÔ ÌÂÏÂÙËı›۷, ÈÔ ÚÔÛ‚¿ÛÈÌË Î·È

·Ú¿ÏÏËÏ· ÏÔ˘ÛÈfiÙÂÚË ËÁ‹ ·Ú¯¤ÁÔÓˆÓ ª∫

(14,15).

H Û˘Á¯ÔÚ‹ÁËÛË ·˘ÙfiÏÔÁˆÓ ª∫ Ì ÚÔÁÔ-

ÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· Û ·ÛıÂÓ›˜ ÌÂ

ηÚΛÓÔ ÙÔ˘ Ì·ÛÙÔ‡ Î·È ·ÈÌ·ÙÔÏÔÁÈΤ˜ ηÎÔ‹-

ıÂȘ ¤‰ÂÈÍ ٷ¯‡ÙÂÚË ·ÔηٿÛÙ·ÛË ÙˆÓ ÂÈ-

¤‰ˆÓ ÙˆÓ Ô˘‰ÂÙÂÚfiÊÈÏˆÓ Î·È ÙˆÓ ·ÈÌÔÂÙ·-

Ï›ˆÓ, ÂÓÒ ·Ú¿ÏÏËÏ· Ë Ì¤ıÔ‰Ô˜ Ê¿ÓËΠӷ ›-

Ó·È ·ÛÊ·Ï‹˜, ¯ˆÚ›˜ ·ÓÂÈı‡ÌËÙ˜ ÂÓ¤ÚÁÂȘ

(16,17).

™ÎÔfi˜ Ù˘ ÌÂϤÙ˘ ‹Ù·Ó Ë ‰ÈÂÚ‡ÓËÛË Ù˘

›‰Ú·Û˘ ÙˆÓ ª∫ ÛÙÚÒÌ·ÙÔ˜ Ì˘ÂÏÔ‡ ÙˆÓ

ÔÛÙÒÓ ÛÙË ‰˘Ó·ÙfiÙËÙ· ¤ÎÙ˘Í˘ CD34+ ΢ÙÙ¿-

ÚˆÓ ·›Ì·ÙÔ˜ ÔÌÊ¿ÏÈÔ˘ ÏÒÚÔ˘ Î·È Ë Û‡ÁÎÚÈÛË

Ì ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë Â›‰Ú·ÛË Û˘Ó‰˘·ÛÌÒÓ ·˘-

ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ

™˘ÏÏÔÁ‹ / ‰È·¯ˆÚÈÛÌfi˜ CD34+ ΢ÙÙ¿ÚˆÓ ·fi ·›Ì·

ÔÌÊ¿ÏÈÔ˘ ÏÒÚÔ˘

∞›Ì· √§ (n=10) Û˘ÏϤ¯ıËΠÌÂÙ¿ ÙËÓ ÔÏÔÎÏ‹Úˆ-

ÛË ÙÔ˘ ÙÔÎÂÙÔ‡ (ŒÁÎÚÈÛË ∂ÈÙÚÔ‹˜ ¢ÂÔÓÙÔÏÔÁ›·˜

ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ Ì·˜). ∆· ÌÔÓÔ‡ÚËÓ· ·ÙÙ·Ú·

(ª¡¶) ‰È·¯ˆÚ›ÛÙËÎ·Ó Ì ›ÛÙÚˆÛË Û Ficoll

Hypaque (Lymphoprep-Nycomed d=1077 g/ml) Î·È Ë

·ÔÌfiÓˆÛË ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ ¤ÁÈÓ Ì ·ÓÔÛÔ-Ì·-

ÁÓËÙÈÎfi ‰È·¯ˆÚÈÛÌfi (Mini MACS-Separation

Columns), Û‡Ìʈӷ Ì ÙȘ Ô‰ËÁ›Â˜ ÙÔ˘ ηٷÛ΢·ÛÙ‹

(Miltenyi Biotec), ÂÓÙfi˜ 24 ˆÚÒÓ ·fi ÙË Ï‹„Ë ÙÔ˘

‰Â›ÁÌ·ÙÔ˜.

AÔÌfiÓˆÛË ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ (ª∫) ·fi

Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ

∏ Û˘ÏÏÔÁ‹ ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ ª√ ¤ÁÈÓ ·fi ·È‰È¿

Ì ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· (ȉÈÔ·ı‹ ıÚÔÌ-

‚ÔÂÓÈ΋ ÔÚʇڷ, n=9). ∆· MN¶ ηÏÏÈÂÚÁ‹ıËηÓ

Û a-MEM, ÂÌÏÔ˘ÙÈṲ̂ÓÔ Ì ÂÈÏÂÁ̤ÓÔ ÔÚfi ÂÌ-

‚Ú‡Ô˘ ‚Ôfi˜ (FCS), ÂÓÈÎÈÏÏ›ÓË/ÛÙÚÂÙÔÌ˘Î›ÓË 10%,

ÁÏÔ˘Ù·Ì›ÓË 10% Î·È ·˘ÍËÙÈÎfi ·Ú¿ÁÔÓÙ· ÈÓÔ‚Ï·-

ÛÙÒÓ (FGF-2) 1 ng/ml. ŸÙ·Ó ‰ËÌÈÔ˘ÚÁ‹ıËΠÛÙÚÒÌ·

>75%, ¤ÁÈÓ ıÚ˘„ÈÓÔÔ›ËÛË (ıÚ˘„›ÓË 0,25% +

EDTA) Î·È Ù· ·ÙÙ·Ú· ·ӷηÏÏÈÂÚÁ‹ıËÎ·Ó Û η-

Ù¿ÏÏËÏË Û˘ÁΤÓÙÚˆÛË (P1). ∏ ‰È·‰Èηۛ· ·ӷϋ-

ÊıËΠ̤¯ÚÈ ƒ4.

∞Ó¿Ù˘ÍË CFU-F ·ÔÈÎÈÒÓ - ¯ÚfiÓÔ˜ ΢ÙÙ·ÚÈÎÔ‡ ‰ÈÏ·-

ÛÈ·ÛÌÔ‡: °È· ÙËÓ ·Ó¿Ù˘ÍË CFU-F ·ÔÈÎÈÒÓ 10 ª∫/cm2

ÙÔÔıÂÙ‹ıËÎ·Ó Û ÙÚ˘‚Ï›· Petri 20 cm2 Û ·-ª∂ª (ÂȘ

‰ÈÏÔ‡Ó). ªÂÙ¿ ·fi 14 Ë̤Ú˜ ηÏÏȤÚÁÂÈ·˜, ¤ÁÈÓÂ

¯ÚÒÛË Ì Giemsa Î·È Î·Ù·ÌÂÙÚ‹ıËÎ·Ó ÔÈ ·ÔÈ˘ ÛÂ

·Ó¿ÛÙÚÔÊÔ ÌÈÎÚÔÛÎfiÈÔ.

√ ¯ÚfiÓÔ˜ ΢ÙÙ·ÚÈÎÔ‡ ‰ÈÏ·ÛÈ·ÛÌÔ‡ [cell doubling

time (DT)] ˘ÔÏÔÁ›ÛÙËΠ‚¿ÛÂÈ ÙÔ˘ Ù‡Ô˘:

DT = t / n = t x log2 / log (·ÚÈıÌfi˜ ΢ÙÙ¿ÚˆÓ Ô˘

ÚԤ΢„·Ó ·fi ÙË ıÚ˘„ÈÓÔÔ›ËÛË/ ·ÚÈıÌfi ΢ÙÙ¿ÚˆÓ

Ô˘ ¿Ú¯ÈÛ ÙËÓ Î·ÏÏȤÚÁÂÈ·) (t : Ô ¯ÚfiÓÔ˜ Ô˘ ÌÂÛÔÏ¿-

‚ËÛ ·fi ÙËÓ ¤Ó·ÚÍË Ù˘ ηÏÏȤÚÁÂÈ·˜ ̤¯ÚÈ ÙË ıÚ˘„È-

ÓÔÔ›ËÛË).

∫·ÏÏȤÚÁÂȘ ‰È·ÊÔÚÔÔ›ËÛ˘ ª∫: ™ÙËÓ ƒ2 ‹ ƒ3 ¤ÁÈÓÂ

·ÁˆÁ‹ ÙˆÓ ª∫ ÚÔ˜ ÏÈÔ·ÙÙ·Ú· (§), ÔÛÙÂÔ·ÙÙ·Ú·

(√) Î·È ¯ÔÓ‰ÚÔ·ÙÙ·Ú· (Ã) Û ÂȉÈÎfi ıÚÂÙÈÎfi ˘ÏÈÎfi.

ªÂ ÈÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË ÂϤÁ¯ıËÎÂ Ë ‰È·ÊÔÚÔÔ›ËÛË

ÚÔ˜ § (Oil red O), √: (Von Kossa) Î·È Ã (Alcian Blue).

ªÂ RT-PCR ·ÓȯÓ‡ıËÎÂ Ë ¤ÎÊÚ·ÛË ÁÔÓȉ›ˆÓ ¯·Ú·ÎÙË-

ÚÈÛÙÈÎÒÓ ÁÈ· § (LPL, aP2), ÁÈ· √ (Osteocalcin,

Osteoprotegerin) Î·È ÁÈ· à (Aggrecan, Collagen II).

∫·ÏÏȤÚÁÂÈ· CD34+ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

·ÚÔ˘Û›· ª∫

°È· ÙË Û˘ÁηÏÏȤÚÁÂÈ· ÙˆÓ ª∫ Ì ٷ ·Ú¯¤ÁÔÓ·

110 π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:110-000

Conclusions: MSC derived from BM of children, used as a feeder layer, supports the ex-vivo expansionand preserves the characteristics of ‘stemness’ of CD34+ HSC in co-culture conditions, providing apopulation large enough to overcome the problems caused by limited HSC numbers for CBtransplants.

Key words: CD34+, mesenchymal stromal cells, ex vivo expansion, cord blood, children bone marrow.

Pediatri Mar-Apr 09 10-04-09 15:36 ™ÂÏ›‰·110

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CD34+ ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ª∫ ƒ3 ‹ ƒ4Ô˘ ÙÔÔıÂÙ‹ıËÎ·Ó Û ÙÚ˘‚Ï›· 24 ‚ÔıÚ›ˆÓ ÂÈÛÙڈ̤ӷ ÌÂ˙ÂÏ·Ù›ÓË, ÛÂ Û˘ÁΤÓÙÚˆÛË 3x104/‚ÔıÚ›Ô. ŸÙ·Ó Ù· ª∫ ‰ËÌÈÔ‡Ú-ÁËÛ·Ó ÛÙÚÒÌ·, ÚÔÛÙ¤ıËÎ·Ó CD34+ ·ÙÙ·Ú· (2,5x103/‚ÔıÚ›Ô )Î·È Âˆ¿ÛÙËÎ·Ó ÁÈ· 10 Ë̤Ú˜ Û 37ÔC 5% CO2, Ì ÚÔÛı‹ÎËıÚÂÙÈÎÔ‡ ˘ÏÈÎÔ‡ Î·È ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ÙËÓ 5Ë Ë̤ڷ.∆Ô Î·ÏÏÈÂÚÁËÙÈÎfi ˘ÏÈÎfi ·ÔÙÂÏ›ÙÔ ·fi a-MEM + 10% FCS +10% ÂÓÈÎÈÏÏ›ÓË /ÛÙÚÂÙÔÌ˘Î›ÓË + 10% ÁÏÔ˘Ù·Ì›ÓË, ·ÚÔ˘-Û›· ‹ ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ. ÃÚËÛÈÌÔÔÈ‹ıËηӉ‡Ô Û˘Ó‰˘·ÛÌÔ› ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ GFmix1 Î·È GFmix2.

GFmix1: SCF: 10 ng/ml, IL-3: 5 ng/ml, G-CSF: 100 ng/mlÎ·È GM-CSF: 1 ng/ml Î·È GFmix2: SCF: 20 ng/ml, TPO: 25ng/ml, IL-6: 20 ng/ml, Flt-3 ligand: 50 ng/ml.

√È Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ Ô˘ ÂÍÂÙ¿ÛıËÎ·Ó ‹Ù·Ó: ™˘Óı‹ÎË ∞: MK+CD34+

™˘Óı‹ÎË µ: MK+CD34+ +GFmix1 ™˘Óı‹ÎË °: MK+CD34+ +GFmix2™˘Óı‹ÎË ¢: CD34+ +GFmix1 ™˘Óı‹ÎË ∂: CD34+ +GFmix2 ∆Ë 10Ë Ë̤ڷ Ù˘ Û˘ÁηÏÏȤÚÁÂÈ·˜ Û˘ÏϤ¯ıËÎ·Ó Ù· ·Ù-

Ù·Ú· ÙÔ˘ ˘ÂÚÎÂÈ̤ÓÔ˘, ÂÓÒ Ù· ÚÔÛÎÔÏÏË̤ӷ ·ÙÙ·Ú··ÔÌ·ÎÚ‡ÓıËÎ·Ó Ì ıÚ˘„ÈÓÔÔ›ËÛË.

∏ ¤ÎÙ˘ÍË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ˘ÔÏÔÁ›ÛÙËÎÂ Û˘ÁÎÚ›ÓÔÓÙ·˜ÙÔÓ ·ÚÈıÌfi ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÙË ‰¤Î·ÙË Ë̤ڷ Û˘ÁηÏÏȤÚÁÂÈ·˜Ì ÙÔÓ ·Ú¯ÈÎfi ·ÚÈıÌfi ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ.

∞ÓÔÛÔÊ·ÈÓÔÙ˘ÈÎfi˜ ·Ú·ÎÙËÚÈÛÌfi˜

ªÂÙ¿ ·fi Û‹Ì·ÓÛË ÙˆÓ Î˘ÙÙ¿ÚˆÓ ÌÂ Û˘˙¢Á̤ӷ ÊıÔÚ›-˙ÔÓÙ· ÌÔÓÔÎψÓÈο ·ÓÙÈÛÒÌ·Ù· ·ÎÔÏÔ‡ıËÛ ΢ÙÙ·ÚÔÌÂÙÚ›·ÚÔ‹˜. ŒÁÈÓ ·Ó¿Ï˘ÛË ÁÈ·: CD34-PE, CD45-FITC, CD33-PC5,CD38-PC5, CD105-PE, CD146-P∂ Î·È Ë ‰È¿ÎÚÈÛË ÙˆÓ ª∫·fi Ù· ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ‚·Û›ÛÙËΠÛÙËÓ ¤ÎÊÚ·ÛË ÙÔ˘CD45 Î·È ÛÙȘ ‰È·ÊÔÚ¤˜ ÙˆÓ ‰‡Ô ΢ÙÙ·ÚÈÎÒÓ Ù‡ˆÓ ·Ó¿ÏÔÁ·Ì ÙÔ Ì¤ÁÂıÔ˜ Î·È ÙËÓ ÎÔÎΛˆÛË (FS/SS).

∞Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ ÎÔÎÎÈÒ‰Ô˘˜ (CFU-GM) Î·È ÂÚ˘ıÚ¿˜

(BFU-E) ÛÂÈÚ¿˜ - ηÏÏȤÚÁÂȘ ÌÂı˘ÏÔ΢ÙÙ·Ú›Ó˘

™Â ηÏÏÈÂÚÁËÙÈÎfi ˘ÏÈÎfi 1% ÌÂı˘ÏÔ΢ÙÙ·Ú›Ó˘ (STEMCELL Technologies HCC4230) ÚÔÛÙ¤ıËÎ·Ó 5x103 ·ÙÙ·Ú·Î·È ÔÈ ·˘ÍËÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ G-CSF: 100 ng/ml, IL-3: 5 ng/ml,GM-CSF: 1 ng/ml Î·È ÂÚ˘ıÚÔÔÈËÙ›ÓË (EPO): 4 πU/ml.

√È Î˘ÙÙ·ÚÔηÏÏȤÚÁÂȘ ˆ¿ÛÙËÎ·Ó ÁÈ· 14 Ë̤Ú˜ ÛÂ37ÔC 5% CO2 Î·È Ë Î·Ù·Ì¤ÙÚËÛË ·ÔÈÎÈÒÓ ¤ÁÈÓ Û ·Ó¿-ÛÙÚÔÊÔ ÌÈÎÚÛÎfiÈÔ. ŸÏ· Ù· ÂÈÚ¿Ì·Ù· ¤ÁÈÓ·Ó ÂȘ ‰ÈÏÔ‡Ó.

™Ù·ÙÈÛÙÈ΋ ∞Ó¿Ï˘ÛË

∆· ·ÔÙÂϤÛÌ·Ù· ÂÎÊÚ¿˙ÔÓÙ·È ˆ˜ ̤ÛË ÙÈÌ‹±ÛÙ·ıÂÚfi

ÛÊ¿ÏÌ· Ù˘ ̤Û˘ ÙÈÌ‹˜ (SEM). √È ÌÂÙ·‚ÏËÙ¤˜ Ì ÌË Î·ÓÔ-ÓÈ΋ ηٷÓÔÌ‹ Û˘ÁÎÚ›ıËÎ·Ó Ì Mann-Whitney U test. ŸÏ˜ÔÈ Û˘ÁÎÚ›ÛÂȘ Â›Ó·È ‰ÈÏ‹˜ ηÙ‡ı˘ÓÛ˘. ™Ù·ÙÈÛÙÈÎÒ˜ ÛËÌ·-ÓÙÈο ıˆڋıËÎ·Ó Ù· ·ÔÙÂϤÛÌ·Ù· Ì p<0,05. ∏ ·Ó¿Ï˘ÛˤÁÈÓ Ì ÙÔ Statistical Package for Social Sciences (SPSS).

∞ÔÙÂϤÛÌ·Ù·

÷ڷÎÙËÚÈÛÙÈο ÌÂÛÂÁ¯˘Ì·ÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

∆· ª∫ ÂÌÊ¿ÓÈÛ·Ó ÛÙËÓ ÚÒÙË Î·ÏÏȤÚÁÂÈ· ηȉȷًÚËÛ·Ó ÛÙȘ ÂfiÌÂÓ˜ ·ӷηÏÏȤÚÁÂȘ ÙÔ¯·Ú·ÎÙËÚÈÛÙÈÎfi ·ÙÚ·ÎÙÔÂȉ¤˜ Û¯‹Ì· (∂ÈÎfiÓ· 1).∞ÓÔÛÔÊ·ÈÓÔÙ˘Èο, ‹‰Ë ·fi ÙËÓ ÚÒÙË Â·Ó·Î·Ï-ÏȤÚÁÂÈ· ‰ÂÓ ·Ú·ÙËÚ‹ıËΠ¤ÎÊÚ·ÛË ÙˆÓ ·ÈÌÔÔÈË-ÙÈÎÒÓ ‰ÂÈÎÙÒÓ CD34 Î·È CD45, ÂÓÒ ÔÈ Û¯ÂÙÈ˙fiÌÂÓÔÈÌ ٷ MK ‰Â›ÎÙ˜, Ô˘ ÂÎÊÚ¿˙ÔÓÙ·Ó Û Ôχ ÌÈÎÚfiÔÛÔÛÙfi ÛÙÔÓ ÏËı˘ÛÌfi ÙˆÓ M¶¡ (CD105:1,85±0,3%, CD146: 0,93±0,3%), ÂÎÊÚ¿˙ÔÓÙ·Ó Û¢„ËÏfi ÔÛÔÛÙfi ÛÙȘ ·ӷηÏÏȤÚÁÂȘ ÙˆÓ ª∫(CD105: 99,4±0,23% Î·È CD146: 94,05±1,45%).

™ÙËÓ ƒ2 ÙÔ Û‡ÓÔÏÔ ÙˆÓ ·ÔÈÎÈÒÓ CFU-F ‹Ù·Ó38,05±3,7 Î·È ÛÙËÓ P4 ‹Ù·Ó 24,03±3. √ ¯ÚfiÓÔ˜ ΢Ù-Ù·ÚÈÎÔ‡ ‰ÈÏ·ÛÈ·ÛÌÔ‡ ÛÙËÓ ƒ2 ‹Ù·Ó 1,93±0,1 Ë̤-Ú˜ Î·È ÛÙËÓ ƒ4 2,6±0,2 Ë̤Ú˜.

∞fi ÙËÓ ÈÛÙÔ¯ËÌÈ΋ ¯ÚÒÛË Ê¿ÓËΠfiÙÈ Ù· ª∫¤¯Ô˘Ó ÈηÓfiÙËÙ· ‰È·ÊÔÚÔÔ›ËÛ˘ ÚÔ˜ ÔÛÙÂÔ·Ù-Ù·Ú·, ÏÈÔ·ÙÙ·Ú·, ¯ÔÓ‰ÚÔ·ÙÙ·Ú· (∂ÈÎfiÓ· 2). ∏·Ó¿Ï˘ÛË Ì RT-PCR ¤‰ÂÈÍ fiÙÈ Ù· ·ÙÙ·Ú· ·˘Ù¿ ÂÎ-ÊÚ¿˙Ô˘Ó ÌÔÚÈ·ÎÔ‡˜ ‰Â›ÎÙ˜ ÂȉÈÎÔ‡˜ ÁÈ· ÔÛÙÂÔ·Ù-Ù·Ú·, ÏÈÔ·ÙÙ·Ú· Î·È ¯ÔÓ‰ÚÔ·ÙÙ·Ú· (‰ÂÓ ·Ú·Ù›-ıÂÓÙ·È Ù· ‰Â‰Ô̤ӷ).

∞ÔÌfiÓˆÛË CD34+ ΢ÙÙ¿ÚˆÓ

√ ̤ÛÔ˜ fiÁÎÔ˜ Û˘ÏÏÔÁ‹˜ √§ ‹Ù·Ó 39,8±2,2 ml,ÂÓÒ ÌÂÙ¿ ÙËÓ Â›ÛÙÚˆÛË Û Ficoll-Hypaque ·ÔÌÔ-ÓÒıËÎ·Ó 2,9±0,5x108 ª¡¶. ªÂÙ¿ ÙÔÓ ·ÓÔÛÔÌ·ÁÓË-ÙÈÎfi ‰È·¯ˆÚÈÛÌfi, Ù· ·ÙÙ·Ú· ‹Ù·Ó 54,3±2,1x104 ηÈÂͤÊÚ·˙·Ó CD34 Û ÔÛÔÛÙfi 93±1,43%.

∂›‰Ú·ÛË Ù˘ ·ÚÔ˘Û›·˜ ª∫ ÛÙËÓ ¤ÎÙ˘ÍË ÙÔ˘

Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ΢ÙÙ¿ÚˆÓ

∏ Ù·˘Ùfi¯ÚÔÓË ·ÚÔ˘Û›· ª∫ Î·È ·˘ÍËÙÈÎÒÓ

111ªÂÛÂÁ¯˘Ì·ÙÈο Î·È ¤ÎÙ˘ÍË ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜

¶·È‰È·ÙÚÈ΋ 2009;72:111-000

∂ÈÎfiÓ· 1. ·) ªÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·, ‚) ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ÌÂÙ¿ ·fi ‰¤Î· Ë̤Ú˜ ηÏÏȤÚÁÂÈ·˜ ·ÚÔ˘Û›· ÌfiÓÔ ·˘ÍËÙÈÎÒÓ ·Ú·Áfi-ÓÙˆÓ, Á) ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· Û ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú· ÛÙÚÒÌ·ÙÔ˜ ÙË ‰¤Î·ÙË Ë̤ڷ Û˘ÁηÏÏȤÚÁÂÈ·˜.

· ‚ Á

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·Ú·ÁfiÓÙˆÓ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ·

ÙË ÌÂÁ·Ï‡ÙÂÚË ¤ÎÙ˘ÍË ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ

΢ÙÙ¿ÚˆÓ (Û˘Óı‹ÎË B/° Û ۇÁÎÚÈÛË ÌÂ Û˘Óı‹Î˜

¢/∂) (∂ÈÎfiÓ· 1), ÂÓÒ ÔÈ Û˘Ó‰˘·ÛÌÔ› ÙˆÓ ·˘ÍËÙÈÎÒÓ

·Ú·ÁfiÓÙˆÓ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ‰ÂÓ ‰È¤ÊÂÚ·Ó

ÛÙËÓ Â›‰Ú·ÛË Ô˘ ›¯·Ó ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ Î˘ÙÙ¿-

ÚˆÓ (¶›Ó·Î·˜ 1, Û˘Óı‹ÎË µ Î·È °). ∆· ·ÈÌÔÔÈËÙÈο

·ÙÙ·Ú· Ô˘ ηÏÏÈÂÚÁ‹ıËÎ·Ó ·ÔÎÏÂÈÛÙÈο ·ÚÔ˘-

Û›· ΢ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ (Û˘Óı‹ÎË ∞) ·˘Í‹ıËηÓ

ηٿ 10,9±5,73 ÊÔÚ¤˜ Î·È ·˘Ù‹ Ë Û˘Óı‹ÎË ·¤ÊÂÚÂ

ÙË ÌÈÎÚfiÙÂÚË ¤ÎÙ˘ÍË (p≤0,05) Û ۇÁÎÚÈÛË Ì fiϘ

ÙȘ ˘fiÏÔȘ (¶›Ó·Î·˜ 1).

∂›‰Ú·ÛË Ù˘ ·ÚÔ˘Û›·˜ ª∫ ÛÙËÓ ¤ÎÙ˘ÍË ÙˆÓ

·Ú¯¤ÁÔÓˆÓ/ÚÔÁÔÓÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ

∏ Ù·˘Ùfi¯ÚÔÓË ·ÚÔ˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ

-·ÓÂÍ·Úًو˜ Û˘Ó‰˘·ÛÌÔ‡- Î·È ÛÙÚÒÌ·ÙÔ˜ (Û˘Óı‹-

ÎË µ Î·È °, ¶›Ó·Î·˜ 1) ¤ÊÂÚ ÙË ÌÂÁ·Ï‡ÙÂÚË ¤Î-

Ù˘ÍË ÛÙ· CD34+ ·ÙÙ·Ú·, ÂÓÒ ÙÔ Ùˆ¯fiÙÂÚÔ ÔÛÔ-

ÙÈο ·ÔÙ¤ÏÂÛÌ· ›¯Â Ë Û˘Óı‹ÎË ∞ (p=0,025). ∏

ηÏÏȤÚÁÂÈ· ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ Ì ·˘ÍË-

ÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÌfiÓÔ (Û˘Óı‹Î˜ ¢ Î·È ∂) ¤ÊÂ-

Ú ÈηÓÔÔÈËÙÈ΋ ¤ÎÙ˘Í‹ ÙÔ˘˜, Ë ÔÔ›·, ·Ó ηÈ

·ÚÈıÌËÙÈο ‹Ù·Ó ÌÈÎÚfiÙÂÚË ·˘Ù‹˜ Ô˘ ÚÔ·ÙÂÈ

·ÚÔ˘Û›· ÛÙÚÒÌ·ÙÔ˜, ÂÓ ÙÔ‡ÙÔȘ ‰ÂÓ ÊÙ¿ÓÂÈ ÛÙ·

fiÚÈ· Ù˘ ÛÙ·ÙÈÛÙÈ΋˜ ÛËÌ·ÓÙÈÎfiÙËÙ·˜.

∞ÓÔÛÔÊ·ÈÓÔÙ˘Èο ·Ú·ÎÙËÚÈÛÙÈο ΢ÙÙ¿ÚˆÓ

ÛÙÚÒÌ·ÙÔ˜ Î·È ÂÎÙ˘ÁÌ¤ÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ

΢ÙÙ¿ÚˆÓ

√ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ¤ÎÊÚ·Û˘ ÙÔ˘ CD34 ¤‰ÂÈÍÂ

ˆ˜ Ë ·Ô˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ ·fi ÙËÓ Î·Ï-ÏȤÚÁÂÈ· ¤¯ÂÈ ˆ˜ Û˘Ó¤ÂÈ· ÌÂÁ·Ï‡ÙÂÚÔ ÂηÙÔÛÙÈ·›ÔÔÛÔÛÙfi ΢ÙÙ¿ÚˆÓ Ô˘ ÙÔ ÂÎÊÚ¿˙Ô˘Ó (¶›Ó·Î·˜ 1).

∆· ÔÛÔÛÙ¿ ÙˆÓ ÏËı˘ÛÌÒÓ CD34+38- ηÈCD34+33- Ô˘ Ô‰ÂÈÎÓ‡Ô˘Ó Î‡ÙÙ·Ú· Ì ·Ú·ÎÙËÚÈ-ÛÙÈο ·ˆÚfiÙËÙ·˜ ‹Ù·Ó ÌÂÁ·Ï‡ÙÂÚ· ÛÙË Û˘Óı‹ÎË ∞(·ÚÔ˘Û›· ÌfiÓÔ ª∫), Û ۇÁÎÚÈÛË Ì ÙȘ ˘fiÏÔÈÂ˜Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ (µ/°/¢ Î·È ∂). ∆Ô ÔÛÔÛÙfiÙˆÓ CD34+33- ΢ÙÙ¿ÚˆÓ ‹Ù·Ó 40,5±3,6% ÛÙË Û˘Óı‹-ÎË ∞, 4,1±0,9% ÛÙË Û˘Óı‹ÎË µ Î·È 4±1,1% ÛÙË Û˘Ó-ı‹ÎË ¢. (∂ÈÎfiÓ· 3). ∆· ·ÓÙ›ÛÙÔȯ· ÔÛÔÛÙ¿ ÁÈ· Ù·CD34+38- ·ÙÙ·Ú· ‹Ù·Ó 5,6±3,4%, 1,3±0,9% ηÈ4,2±3,8%. ¢ÂÓ ·Ú·ÙËÚ‹ıËΠ‰È·ÊÔÚ¿ ·˘ÙÒÓ ÙˆÓ΢ÙÙ·ÚÈÎÒÓ ˘ÔÏËı˘ÛÌÒÓ ÂÍ·ÚÙÒÌÂÓË ·fi ÙÔÓÛ˘Ó‰˘·ÛÌfi ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (Û˘Óı‹Î˜µ ÚÔ˜ ° Î·È ¢ ÚÔ˜ ∂) (∂ÈÎfiÓ· 3).

∞Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ ÎÔÎÎÈÒ‰Ô˘˜ (CFU-GM) ηÈ

ÂÚ˘ıÚ¿˜ (BFU-E) ÛÂÈÚ¿˜

∞Ó¿Ù˘ÍË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÔÈÎÈÒÓ CFU-GM (186,81±9,9) ·Ú·ÙËÚ‹ıËΠ·ÚÔ˘Û›· ÌfiÓÔ΢ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ (∂ÈÎfiÓ· 4). ™˘ÁÎÚ›ÓÔÓÙ·˜ ÌÂfiϘ ÙȘ ˘fiÏÔȘ Û˘Óı‹Î˜, Ê·›ÓÂÙ·È ˆ˜ Ë ·-ÚÔ˘Û›· ÌfiÓÔ ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ·, ÌÔÏÔÓfiÙÈ ‰ÂÓÚÔ¿ÁÂÈ ÙÔÓ ÔÏÏ·Ï·ÛÈ·ÛÌfi ÙˆÓ Î˘ÙÙ¿ÚˆÓ (ÌÈÎÚfi-ÙÂÚË ¤ÎÙ˘ÍË ÙÔ˘ ÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ Î·È ÙÔ˘ ·ÚÈıÌÔ‡ÙˆÓ CD34+), ÂÓ ÙÔ‡ÙÔȘ ·Ú¤¯ÂÈ Î‡ÙÙ·Ú· Ì ·˘ÍË̤-ÓË ÎψÓÔÁÔÓÈ΋ ÈηÓfiÙËÙ·. ∂›Û˘, ·Ú·ÙËÚ›ٷȉȷÊÔÚÂÙÈÎfi˜ Ú˘ıÌfi˜ ·Ó¿Ù˘Í˘ ·ÔÈÎÈÒÓ ·Ó¿ÏÔÁ·Ì ÙÔÓ Û˘Ó‰˘·ÛÌfi ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Î·È ÔGFmix1 (Û˘Óı‹ÎË ¢) ¤¯ÂÈ ˆ˜ ·ÔÙ¤ÏÂÛÌ· ·Ó¿Ù˘ÍËÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÔÈÎÈÒÓ (147,0±12,9), ÛÂ

¶›Ó·Î·˜ 1. ∞ÔÙÂϤÛÌ·Ù· ¤ÎÙ˘Í˘ ÙÔ˘ Û˘ÓÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ÙˆÓ Î˘ÙÙ¿ÚˆÓ Î·È ÙˆÓ CD34+ ΢ÙÙ¿ÚˆÓ Î·È ÔÛÔÛÙfi ¤ÎÊÚ·Û˘ ÙÔ˘ CD34 ·ÓÙÈ-ÁfiÓÔ˘ ÂÈÊ·Ó›·˜ ÌÂÙ¿ ·fi ‰¤Î· Ë̤Ú˜ Û˘ÁηÏÏȤÚÁÂÈ·˜

™˘Óı‹ÎË ŒÎÙ˘ÍË ÔÏÈÎÔ‡ ·ÚÈıÌÔ‡ ŒÎÙ˘ÍË CD34+ % CD34΢ÙÙ¿ÚˆÓ (x)

∞ CD34∆ + ª∫ 10,9 ± 5,7 2,2 ± 0 ,7 50,6 ± 5,5µ CD34∆ + ª∫ + GFmix1 164,4 ± 42,7* 20,9 ± 5,9* 13,1± 0,9*° CD34∆ + ª∫ + GFmix2 165,8 ± 35,8* 16,8 ± 0,5* 11,4 ± 2,0*¢ CD34∆ + + GFmix1 101, 9 ± 36,3* 13,7 ± 9,7 10,9 ± 5,5∂ CD34∆ + + GFmix2 134,2 ± 10,8* 14,2 ± 1,7* 10,5 ± 0,4

*ηٷ‰ÂÈÎÓ‡ÂÈ ÛÙ·ÙÈÛÙÈ΋ ÛËÌ·ÓÙÈÎfiÙËÙ·, p<0,05 Û ۇÁÎÚÈÛË Ì ÙË Û˘Óı‹ÎË ∞

∂ÈÎfiÓ· 2. πÛÙÔ¯ËÌÈΤ˜ ¯ÚÒÛÂȘ ·fi ‰È·ÊÔÚÔÔÈË̤ӷ ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·: ·) ÏÈÔ·ÙÙ·Ú·, ‚) ÔÛÙÂÔ·ÙÙ·Ú·, Á) ¯ÔÓ‰ÚÔ·ÙÙ·Ú·.

112 π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:112-000

· ‚ Á

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Û‡ÁÎÚÈÛË Ì ÙÔ GFmix2 (Û˘Óı‹ÎË ∂) (58,5±11,9)

(p=0,02) (∂ÈÎfiÓ· 4).

∞Ó·ÊÔÚÈο Ì ÙȘ ·ÔÈ˘ Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, Ë

·ÚÔ˘Û›· ÁÂÓÈο ª∫ ›¯Â ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ó¿-

Ù˘ÍË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·ÔÈÎÈÒÓ. ™˘ÁÎÂÎÚÈ̤-

Ó·, ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ·ÔÈ˘ ‰ËÌÈÔ˘ÚÁ‹ıËÎ·Ó ·fi Ù·

·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ·ÚÔ˘Û›· ÌfiÓÔ ª∫ (Û˘Óı‹ÎË

∞), ÂÓÒ ÔÈ ÏÈÁfiÙÂÚ˜ ÛÙȘ Û˘Óı‹Î˜ ¢ Î·È ∂ fiÔ˘

·Ô˘Û›·˙·Ó ÙÂÏ›ˆ˜ Ù· ÌÂÛÂÁ¯˘Ì·ÙÈο (p=0,03 ηÈ

p=0,01, ·ÓÙ›ÛÙÔȯ·, Û ۇÁÎÚÈÛË Ì ™˘Óı‹ÎË ∞) (∂ÈÎfi-

Ó· 4). ¶·ÚÔÌÔ›ˆ˜, fiˆ˜ Î·È ÁÈ· ÙȘ ·ÔÈ˘ Ù˘ ÎÔÎ-

ÎÈÒ‰Ô˘˜ ÛÂÈÚ¿˜, Ô Û˘Ó‰˘·ÛÌfi˜ ·Ú·ÁfiÓÙˆÓ GMix1

¤‰ˆÛ ÂÚÈÛÛfiÙÂÚ˜ ·ÔÈ˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜, Û ۯ¤-

ÛË Ì ÙÔÓ Û˘Ó‰˘·ÛÌfi GMix 2 (Û˘Óı‹ÎË µ/° Î·È ¢/∂).

™˘˙‹ÙËÛË

∆· ·ÙÙ·Ú· ÛÙÚÒÌ·ÙÔ˜ ·ÔÙÂÏÔ‡Ó Ì¤ÚÔ˜ ÙÔ˘ ÌÈ-

ÎÚÔÂÚÈ‚¿ÏÏÔÓÙÔ˜ ÙÔ˘ ª√ Î·È Û˘ÌÌÂÙ¤¯Ô˘Ó ÛÙË

Ú‡ıÌÈÛË Ù˘ ·˘ÙÔ·Ó·Ó¤ˆÛ˘, ÙÔ˘ ÔÏÏ·Ï·ÛÈ·ÛÌÔ‡

Î·È Ù˘ ‰È·ÊÔÚÔÔ›ËÛ˘ ÙˆÓ ·Ú¯¤ÁÔÓˆÓ/ÚÔÁÔÓÈÎÒÓ

·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. √ ·ÎÚÈ‚‹˜ Ì˯·ÓÈÛÌfi˜ ·˘-

Ù‹˜ Ù˘ ‰È·‰Èηۛ·˜ ‰ÂÓ ¤¯ÂÈ ·ÔÛ·ÊËÓÈÛÙ›, ·ÏÏ¿

A

E

A

E

A AA

40,48% 27,45%

E¢¢¢°

B

4,15%

BB

15,20%

CD

33

PC

5

CD34 PE

1,40% 4,00% 17,95% 0,45%

A

E

A

E

A AA

5,63% 7,15%

E¢¢¢°

B

1,35%

BB

2,83%

CD

38

PC

5

CD34 PE

0,40% 4,20% 20,18% 0,45%

∂ÈÎfiÓ· 3. ∞ÓÔÛÔÊ·ÈÓÔÙ˘È΋ ·Ó¿Ï˘ÛË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ √§ ÌÂÙ¿ ·fi ‰¤Î· Ë̤Ú˜ ηÏÏȤÚÁÂÈ·˜ Ì ÌÂÛÂÁ¯˘Ì·ÙÈο ·ÙÙ·Ú·ÛÙÚÒÌ·ÙÔ˜. ∏ ·Ó¿Ï˘ÛË ¤ÁÈÓ ˆ˜ ÚÔ˜ ÙËÓ ¤ÎÊÚ·ÛË CD34 CD33 Î·È CD34CD38 ÛÙÔÓ CD45+ ÏËı˘ÛÌfi.

113ªÂÛÂÁ¯˘Ì·ÙÈο Î·È ¤ÎÙ˘ÍË ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜

¶·È‰È·ÙÚÈ΋ 2009;72:113-000

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Èı·ÓÒ˜ ÂÈÙ˘Á¯¿ÓÂÙ·È Ì¤Ûˆ ÙˆÓ Î˘ÙÙ·ÚÔÎÈÓÒÓ Î·È

¿ÏÏˆÓ ÂÎÎÚÈÓfiÌÂÓˆÓ ·fi Ù· ª∫ ·Ú·ÁfiÓÙˆÓ, ηıÒ˜

Î·È Ì ÙËÓ Â·Ê‹ ·ÙÙ·ÚÔ-·ÙÙ·ÚÔ (18).

∆ËÓ ÙÂÏÂ˘Ù·›· 15ÂÙ›· ·ÂÙ¤ÏÂÛ ·ÓÙÈΛÌÂÓÔ

ÂÎÙÂٷ̤Ó˘ ÌÂϤÙ˘ Ë ex vivo ¤ÎÙ˘ÍË ·›Ì·ÙÔ˜ √§,

ÛÙËÓ ÚÔÛ¿ıÂÈ· ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ÌÂÈÔÓÂÎÙËÌ¿-

ÙˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ √§ ˆ˜ ÌÔۯ‡̷ÙÔ˜, ÒÛÙ ӷ ·ÍÈÔ-

ÔÈËıÔ‡Ó Ù· ÏÂÔÓÂÎÙ‹Ì·Ù· Ô˘ ·Ú¤¯ÂÈ ˆ˜ ËÁ‹

·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ. ™Ù· Ï·›ÛÈ·

·˘Ù¿ ‰ÔÎÈÌ¿ÛÙËÎ·Ó ‰È¿ÊÔÚ˜ Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ-

·˜, ÔÈ Ôԛ˜ ÂÚÈÂÏ¿Ì‚·Ó·Ó ΢ڛˆ˜ ‰È·ÊÔÚÂÙÈÎÔ‡˜

Û˘Ó‰˘·ÛÌÔ‡˜ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ (6-8,19).

™ÎÔfi˜ ‹Ù·Ó Ô ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ È‰·ÓÈÎÒÓ Û˘Ó-

ıËÎÒÓ Î·È Ù˘ ›‰Ú·Û˘ Û˘Ó‰˘·ÛÌÔ‡ ·Ú·ÁfiÓÙˆÓ

Ô˘, ÌÂÙ¿ ·fi Û¯ÂÙÈο Û‡ÓÙÔÌË Î·ÏÏȤÚÁÂÈ·, ı·

ÌÔÚÔ‡Û ӷ ·Ú¤¯ÂÈ ·Ú¯¤ÁÔÓ· ·ÈÌÔÔÈËÙÈο ·ÙÙ·-

Ú· Û ·˘ÍË̤ÓÔ ·ÚÈıÌfi Î·È Ì ‰È·Ù‹ÚËÛË -Û fiÛÔ ÙÔ

‰˘Ó·ÙfiÓ ÌÂÁ·Ï‡ÙÂÚÔ ‚·ıÌfi- ÙˆÓ È‰ÈÔÙ‹ÙˆÓ ÙÔ˘ «·Ú-

¯¤ÁÔÓÔ˘» ΢ÙÙ¿ÚÔ˘. ∆· ‰Â‰Ô̤ӷ ÔÏÏÒÓ ÌÂÏÂÙÒÓ,

·Ó Î·È ·Ú›¯·Ó ÂÓı·ÚÚ˘ÓÙÈο ·ÔÙÂϤÛÌ·Ù·, ‰ÂÓ

ÌÔÚ› Ó· ıˆÚËı› fiÙÈ ·ÓÙÈÌÂÙÒÈÛ·Ó ÈηÓÔÔÈËÙÈ-

ο ÙÔ Úfi‚ÏËÌ·, ·ÊÔ‡, Û‡Ìʈӷ Ì ÎÏÈÓÈΤ˜ ÌÂϤ-

Ù˜ Ê¿Û˘ π, ‰ÂÓ ·Ú·ÙËÚ‹ıËΠÛÙ·ıÂÚ‹ ·Ôηٿ-

ÛÙ·ÛË Ù˘ ·ÈÌÔÔ›ËÛ˘, Èı·ÓÒ˜ ÁÈ·Ù› ηٿ ÙË ‰È¿Ú-

ÎÂÈ· Ù˘ ¤ÎÙ˘Í˘ ÔÈ Î·ÏÏÈÂÚÁËÙÈΤ˜ Û˘Óı‹Î˜ Ô‰Ë-

ÁÔ‡Û·Ó Û ÈÔ ‰È·ÊÔÚÔÔÈË̤ӷ ·ÙÙ·Ú· (20-21).

¶ÈÔ ÚfiÛÊ·Ù·, ÂÍÂÙ¿ÛÙËÎÂ Â¿Ó Ù· ª∫ ı· ÌÔ-

ÚÔ‡Û·Ó Ó· Û˘ÁηÏÏÈÂÚÁËıÔ‡Ó Ì ·Ú¯¤ÁÔÓ· ·ÈÌÔÔÈ-

ËÙÈο ·ÙÙ·Ú· ÁÈ· Ó· ÂÈÙ¢¯ı› ηχÙÂÚË ex vivo

¤ÎÙ˘ÍË ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ (22).

™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÂÚÁ·Û›·˜ ‹Ù·Ó Ë in vitro

ÌÂϤÙË Ù˘ ›‰Ú·Û˘ ÙˆÓ ª∫ ·fi ª√ ·È‰ÈÒÓ ÌÂ

ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù· Û CD34+ ·ÙÙ·Ú·

·›Ì·ÙÔ˜ √§ Î·È Ë Û‡ÁÎÚÈÛË ÙˆÓ ·ÔÙÂϤÛÌ·ÙˆÓ ÌÂ

·ÓÙ›ÛÙÔȯ˜ ηÏÏȤÚÁÂȘ, ·ÚÔ˘Û›· Û˘Ó‰˘·ÛÌÔ‡

·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ.

∆· ·ÔÙÂϤÛÌ·Ù· ¤‰ÂÈÍ·Ó fiÙÈ Ë ·ÚÔ˘Û›· ÌfiÓÔ

ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· Û˘Ì‚¿ÏÏÂÈ Û ۯÂÙÈο ÂÚÈÔÚÈ-

Ṳ̂ÓË ¤ÎÙ˘ÍË ÙˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ/ÚÔÁÔÓÈÎÒÓ Î˘Ù-

Ù¿ÚˆÓ, ·ÏÏ¿ ·ÔʤÚÂÈ ÏËı˘ÛÌfi Ô˘ ÂÎÊÚ¿˙ÂÈ ÛÂ

ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ÙÔ ·ÓÙÈÁfiÓÔ ÂÈÊ·Ó›·˜ CD34.

∆· ·ÔÙÂϤÛÌ·Ù· ·˘Ù¿ Û˘ÁÎÏ›ÓÔ˘Ó Ì ·˘Ù¿ Ô˘

·Ó·Ê¤ÚÔÓÙ·È ·fi ÙÔ˘˜ Jang et al. (23), ÔÈ ÔÔ›ÔÈ fï˜

¯ÚËÛÈÌÔÔ›ËÛ·Ó ª∫ ·fi ·›Ì· √§. ∞Í›˙ÂÈ Ó· ÛËÌÂÈ-

ˆı› fiÙÈ Ë ÂÈÙ˘¯‹˜ ·ÔÌfiÓˆÛË ª∫ ·fi ·›Ì· √§

·Ú·Ì¤ÓÂÈ ‰‡ÛÎÔÏË ÛÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘ Î·È ·ÎfiÌË

·ÌÊÈÏÂÁfiÌÂÓË ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· (24-26). ™ÙËÓ ›‰È·

ÌÂϤÙË ¤ÁÈÓ ›Û˘ Û‡ÁÎÚÈÛË Ù˘ ¤ÎÙ˘Í˘ CD34+

Ì ٷ ·ÔÙÂϤÛÌ·Ù· Ô˘ ÚԤ΢„·Ó ·fi ÙËÓ ¤Î-

Ù˘ÍË CD34+ ÛÂ Û˘Óı‹Î˜ ηÏÏȤÚÁÂÈ·˜ Ì ª∫ Ì˘Â-

ÏÔ‡ ÙˆÓ ÔÛÙÒÓ ·fi ˘ÁÈ›˜ ÂÓ‹ÏÈΘ. ∞fi Ù· ·ÔÙÂ-

ϤÛÌ·Ù· Ê·›ÓÂÙ·È fiÙÈ Ù· ÙÂÏÂ˘Ù·›· ˘ÛÙÂÚÔ‡Ó ÛÙËÓ

·fi‰ÔÛË CD34+ ÏËı˘ÛÌÔ‡, Û ۇÁÎÚÈÛË Ì ·˘Ù¿

ÙÔ˘ √§ Î·È Û˘ÁÎÚÈÙÈο Ì ٷ ª∫ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ

·È‰ÈÒÓ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÛÙËÓ ·ÚÔ‡Û· ÌÂ-

ϤÙË. √È Yamaguchi et al. (10) ›Û˘ ·Ó·Ê¤ÚÔ˘Ó

·Ó¿ÏÔÁË ¤ÎÙ˘ÍË CD34+ Ì ÙË ¯Ú‹ÛË ÌfiÓÔ Î˘ÙÙ¿-

ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ ·fi ª√ ÂÓ‹ÏÈÎˆÓ ‰ÔÙÒÓ ‹ ÂÓ‹ÏÈ-

ÎˆÓ ·ÛıÂÓÒÓ Ì ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù·, ¯ˆÚ›˜ Ó·

ÚÔÛ‰ÈÔÚ›˙ÂÙ·È ÙÔ Â›‰Ô˜ ÙÔ˘ ·ÈÌ·ÙÔÏÔÁÈÎÔ‡ ÓÔÛ‹Ì·-

ÙÔ˜. ∆· ·ÙÙ·Ú· ÛÙÚÒÌ·ÙÔ˜ ·fi ª√, ÂÓ ÙÔ‡ÙÔȘ, ‰ÂÓ

·ÚÔ˘ÛÈ¿˙Ô˘Ó ÙËÓ ÔÌÔÈÔÁ¤ÓÂÈ· ÙÔ˘ ÏËı˘ÛÌÔ‡ ÙˆÓ

ª∫ Î·È Èı·Ó¿ ÚÔÛı¤ÙÔ˘Ó Î·È ¿ÏϘ ·Ú·Ì¤ÙÚÔ˘˜,

ÂÚÈÔÚ›˙ÔÓÙ·˜ ÙË ‰˘Ó·ÙfiÙËÙ· Û‡ÁÎÚÈÛ˘ ÙˆÓ ·ÔÙÂ-

ÏÂÛÌ¿ÙˆÓ. ∂ÈϤÔÓ, ÛÙË ÌÂϤÙË ·˘Ù‹ ‰ÂÓ ·Ó·Ê¤ÚÂ-

Ù·È ·Ó ˘‹ÚÍ·Ó ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ˘ÁÈÒÓ Î·È ·ÛıÂÓÒÓ

ÛÙËÓ ¤ÎÙ˘ÍË ÙÔ˘ ˘ÔÏËı˘ÛÌÔ‡ ÙˆÓ ÚÔÁÔÓÈÎÒÓ

·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ (10).

114 π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:114-000

180

150

120

90

60

30

0A B ° ¢ ∂

™˘Óı‹ÎË

∞Ú

ÈıÌ

fi˜

·

ÔÈÎ

ÈÒÓ/

5x1

03 Î

‡ÙÙ

·Ú

·

CFU-GM

BFU-E

∂ÈÎfiÓ· 4. ∞Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ CFU-GM Î·È BFU-E ÛÙȘ ‰È·ÊÔÚÂÙÈΤ˜ Û˘Óı‹Î˜ Û˘ÁηÏÏȤÚÁÂÈ·˜.

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·114

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°È· Ó· ÂÏÂÁ¯ıÔ‡Ó ·ÊÂÓfi˜ Ë ÈηÓfiÙËÙ· ÙˆÓ ª∫ Ó·

ÂÓÈÛ¯‡ÛÔ˘Ó ÙËÓ ¤ÎÙ˘ÍË ÙˆÓ ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈ-

ÎÒÓ Î˘ÙÙ¿ÚˆÓ Î·È ·ÊÂÙ¤ÚÔ˘ Ù· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ

ÏËı˘ÛÌÒÓ Ô˘ ÚÔ‹Ïı·Ó ·fi ÙËÓ Î·ÏÏȤÚÁÂÈ· ¤Î-

Ù˘Í˘, ¤ÁÈÓ·Ó Î·ÏÏȤÚÁÂȘ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË CFU-

GM Î·È BFU-E ·ÔÈÎÈÒÓ. ¶·Ú·ÙËÚ‹ıËΠÌÂÁ·Ï‡ÙÂ-

ÚÔ˜ ·ÚÈıÌfi˜ Î·È ÙˆÓ ‰‡Ô Ù‡ˆÓ ·ÔÈÎÈÒÓ ÛÙȘ Û˘Ó-

ı‹Î˜ fiÔ˘ ˘‹Ú¯·Ó ÌfiÓÔ ª∫. ™ÙË ‚È‚ÏÈÔÁÚ·Ê›·

·Ó·Ê¤ÚÂÙ·È (23) ÌË ·Ó¿Ù˘ÍË CFU-GM ÌÂÙ¿ ÙËÓ 7Ë

Ë̤ڷ ηÏÏȤÚÁÂÈ·˜, Ô˘ Èı·ÓÔÏÔÁÂ›Ù·È fiÙÈ ÔÊ›ÏÂ-

Ù·È ÛÙÔ fiÙÈ Ù· ·ÙÙ·Ú· fi ÙȘ Û˘ÁÎÂÎÚÈ̤Ó˜ Û˘Óı‹-

Θ ˆı‹ıËÎ·Ó Û ‰È·ÊÔÚÔÔ›ËÛË ·ÊÔ‡ ‹Ïı η-

ٷӿψÛË ‹ ·Ú·›ˆÛË ÙˆÓ ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ

Ô˘ ÚÔ¿ÁÔ˘Ó ÙËÓ ·˘ÙÔ·Ó·Ó¤ˆÛË. ∞ÓÙ›ÛÙÔÈ¯Ë ÌË

·Ó¿Ù˘ÍË ·ÔÈÎÈÒÓ ·Ú·ÙËÚ‹ıËΠ›Û˘ Î·È ·-

ÚÔ˘Û›· ÌfiÓÔ Î˘ÙÙ¿ÚˆÓ ÛÙÚÒÌ·ÙÔ˜ ÌÂÙ¿ ÙËÓ 7Ë Ë̤-

Ú· ηÏÏȤÚÁÂÈ·˜ (23). ∞Í›˙ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ ÔÈ Û˘Óı‹-

Θ ηÏÏȤÚÁÂÈ·˜ ‰ÂÓ Â›Ó·È Ù·˘ÙfiÛË̘ ÛÙȘ Û˘ÁÎÚÈ-

ÓfiÌÂÓ˜ ÌÂϤÙ˜, ηıÒ˜, ¤Ú·Ó Ù˘ ÌÂÁ·Ï‡ÙÂÚ˘

‰È¿ÚÎÂÈ·˜ Ù˘ ηÏÏȤÚÁÂÈ·˜, ¯ÚËÛÈÌÔÔÈÔ‡ÓÙ·È ÔÈÔ-

ÙÈο Î·È ÔÛÔÙÈο ‰È·ÊÔÚÂÙÈÎÔ› Û˘Ó‰˘·ÛÌÔ› ·˘ÍËÙÈ-

ÎÒÓ ·Ú·ÁfiÓÙˆÓ, ηıÒ˜ Î·È ª∫ ΢ڛˆ˜ √§, ·ÏÏ¿

Î·È ÂÓËϛΈÓ. ∞fi Ù· ‰Â‰Ô̤ӷ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤ-

Ù˘ Ê·›ÓÂÙ·È, ÏÔÈfiÓ, fiÙÈ Ë ¯Ú‹ÛË ª∫ ÛÙËÓ ¤ÎÙ˘ÍË

·¤‰ˆÛ ÙÔÓ ÌÂÁ·Ï‡ÙÂÚÔ ·ÚÈıÌfi ·ÔÈÎÈÒÓ ÙfiÛÔ Ù˘

ÎÔÎÎÈÒ‰Ô˘˜ fiÛÔ Î·È Ù˘ ÂÚ˘ıÚ¿˜ ÛÂÈÚ¿˜ ·fi ÔÔÈ·-

‰‹ÔÙ ¿ÏÏË Û˘Óı‹ÎË Î·ÏÏȤÚÁÂÈ·˜ Ô˘ ‰ÔÎÈÌ¿ÛÙË-

ÎÂ, ÂÓ‰ÂÈÎÙÈÎfi Èı·Ó‹˜ «ÔÈÔÙÈ΋˜» ‰È·ÊÔÚ¿˜ ÙˆÓ

ª∫ ·È‰ÈÒÓ, ·ÊÔ‡ Ë ·ÚÔ˘Û›· ÙÔ˘˜ ÚÔ¿ÁÂÈ ÙËÓ ¤Î-

Ù˘ÍË ÏËı˘ÛÌÔ‡ Ì ·˘ÍË̤ÓË ÎψÓÔÁÔÓÈÎfiÙËÙ·.

∏ ·ÓÔÛÔÊ·ÈÓÔÙ˘È΋ ·Ó¿Ï˘ÛË ¤‰ÂÈÍ fiÙÈ Ù·

CD34+CD38- Î·È CD34+CD33-, Ô˘ ·ÔÙÂÏÔ‡Ó ˘Ô-

ÏËı˘ÛÌÔ‡˜ ÚÔÁÔÓÈÎÒÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ,

·ÚÔ˘Û›·Û·Ó ÌÂÁ·Ï‡ÙÂÚÔ ÔÛÔÛÙfi ¤ÎÊÚ·Û˘ ›-

Û˘ ÛÙËÓ Î·ÏÏȤÚÁÂÈ· fiÔ˘ Ù· CD34+ ·ÈÌÔÔÈËÙÈο

Û˘Ó˘‹Ú¯·Ó Ì ª∫ ÌfiÓÔ. √È Chute et al. (27) ¤‰ÂÈ-

Í·Ó ÂȉÈÎfiÙÂÚ· fiÙÈ Ù· CD34+CD38- ÌÔÚÔ‡Ó, ·Áfi-

ÌÂÓ· Û ΢ÙÙ·ÚÈÎfi ·ÎÏÔ, Ó· ÂÎÙ˘¯ıÔ‡Ó in vitro. T·

·ÙÙ·Ú· Ô˘ ÚÔ¤Ú¯ÔÓÙ·È ·fi ÙËÓ ¤ÎÙ˘ÍË ‰È·ÙË-

ÚÔ‡Ó ÙËÓ ¤ÎÊÚ·ÛË ÌÔÚ›ˆÓ ÚÔÛÎfiÏÏËÛ˘, Ù· ÔÔ›·

Èı·ÓÒ˜ ¤¯Ô˘Ó ÛËÌ·ÓÙÈÎfi ÚfiÏÔ ÛÙËÓ ÂÁηٿÛÙ·ÛË

ÙˆÓ ·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ÙÔ˘ ÌÔ-

ۯ‡̷ÙÔ˜ Î·È ÛÙËÓ ·ÔηٿÛÙ·ÛË Ù˘ ·ÈÌÔÔ›Ë-

Û˘. ∏ ȉÈfiÙËÙ· ·˘Ù‹ ÙˆÓ ª∫ ÌÔÚ› Ó· ·ÍÈÔÔÈË-

ı› ÎÏÈÓÈο ÛÙË ÌÂÙ·ÌfiÛ¯Â˘ÛË.

∞Ó Î·È Ô Ì˯·ÓÈÛÌfi˜ ·ÏÏËÏ›‰Ú·Û˘ ÙˆÓ ª∫

Ì ٷ ÚÔÁÔÓÈο ·ÈÌÔÔÈËÙÈο ·ÙÙ·Ú· ‰ÂÓ ¤¯ÂÈ ‰È-

¢ÎÚÈÓÈÛÙ› (18), Â›Ó·È ÁÓˆÛÙfi fiÙÈ Ù· ·ÙÙ·Ú·

ÛÙÚÒÌ·ÙÔ˜ ·Ú¿ÁÔ˘Ó ¤Ó· Û‡ÓÔÏÔ Î˘ÙÙ·ÚÔÎÈÓÒÓ,

ÌÂٷ͇ ÙˆÓ ÔÔ›ˆÓ ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È ÔÈ SCF, IL-1,

IL-3, IL-6, IL-7, IL-11, IL-12, TPO, Flt-3L, G-CSF,

GM-CSF Î·È LIF (28,29). ∞fi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘

·ÚÔ‡Û·˜ ÌÂϤÙ˘ Ê·›ÓÂÙ·È fiÙÈ ÔÈ ‰‡Ô ‰È·ÊÔÚÂÙÈ-

ÎÔ› Û˘Ó‰˘·ÛÌÔ› ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ Ô˘ ¯ÚË-

ÛÈÌÔÔÈ‹ıËÎ·Ó ‰ÂÓ ÚÔηÏÔ‡Ó ÙÔ ›‰ÈÔ ·ÔÙ¤ÏÂÛÌ·

Ì ÙÔ˘˜ ·˘ÍËÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ÂÎÎÚ›ÓÔÓÙ·È

·fi Ù· ª∫ ÛÙËÓ Î·ÏÏȤÚÁÂÈ·. ™˘ÁÎÂÎÚÈ̤ӷ, ·-

ÚÔ˘Û›· ·˘ÍËÙÈÎÒÓ ·Ú·ÁfiÓÙˆÓ, ·Ú·ÙËÚ‹ıËÎÂ

·Ó¿Ù˘ÍË-‰È·ÊÔÚÔÔ›ËÛË Û ϤÔÓ ÒÚÈÌ· ÛÙÔÈ-

¯Â›· Ù˘ ηÏÏȤÚÁÂÈ·˜ (ÔÏÈÎfi˜ ·ÚÈıÌfi˜ ΢ÙÙ¿ÚˆÓ,

CD34+ Ì ÌÈÎÚfiÙÂÚË ÎψÓÔÁÔÓÈÎfiÙËÙ· Î.Ï.), ·ÓÂ-

Í¿ÚÙËÙ· ·fi ÙÔ Â¿Ó Ô Û˘Ó‰˘·ÛÌfi˜ ΢ÙÙ·ÚÔÎÈÓÒÓ

Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËΠÂȉڿ ΢ڛˆ˜ Û ·Ú¯¤ÁÔÓ·

‹ ÈÔ ÒÚÈÌ· ‰ÂÛÌÂ˘Ì¤Ó· ÚÔÁÔÓÈο ·ÈÌÔÔÈËÙÈο

·ÙÙ·Ú· (30).

™˘ÌÂÚ·ÛÌ·ÙÈο, Ù· ª∫ ÛÙÚÒÌ·ÙÔ˜ Ì˘ÂÏÔ‡ ÙˆÓ

ÔÛÙÒÓ ·È‰ÈÒÓ Ì ηÏÔ‹ıË ·ÈÌ·ÙÔÏÔÁÈο ÓÔÛ‹Ì·Ù·

ÚÔ¿ÁÔ˘Ó ·ÔÙÂÏÂÛÌ·ÙÈÎfiÙÂÚ· ÙËÓ ex vivo ¤ÎÙ˘ÍË

·Ú¯¤ÁÔÓˆÓ ·ÈÌÔÔÈËÙÈÎÒÓ Î˘ÙÙ¿ÚˆÓ ·fi ·›Ì· √§,

Û ۇÁÎÚÈÛË Ì ÙËÓ ¤ÎÙ˘ÍË ·ÚÔ˘Û›· ·˘ÍËÙÈÎÒÓ

·Ú·ÁfiÓÙˆÓ, ·Ô‰›‰ÔÓÙ·˜ ¤Ó·Ó ÏËı˘ÛÌfi Ì ¯·Ú·-

ÎÙËÚÈÛÙÈο ·ˆÚfiÙËÙ·˜ Î·È È‰ÈfiÙËÙ˜ ηٿÏÏËϘ ÁÈ·

ÙËÓ ·ÔηٿÛÙ·ÛË Ù˘ ·ÈÌÔÔ›ËÛ˘ ÌÂÙ¿ ·fi ÌÂ-

Ù·ÌfiÛ¯Â˘ÛË.

µÈ‚ÏÈÔÁÚ·Ê›·

1. Wynter EA, Emmerson AJ, Testa NG. Properties of

peripheral blood and cord blood stem cells. Baillieres’ Best

Pract Res Clin Haematol 1999;12:1-17.

2. Lewis ID, Verfaillie CM. Multi-lineage expansion potential

of primitive hematopoietic progenitors: superiority of

umbilical cord blood compared to mobilized peripheral

blood. Exp Hematol 2000;28:1087-1095.

3. Zhang J, Niu C, Ye L, Huang H, He X, Tong WG, et al.

Identification of the haematopoietic stem cell niche and

control of the niche size. Nature 2003;425:836-841.

4. Cohen Y, Nagler A. Umbilical cord blood transplantation:

how, when and for whom? Blood Rev 2004;18:167-179.

5. Cohen Y, Kreiser D, Mayorov M, Nagler A. Unrelated and

related cord blood banking and hematopoietic graft

engineering. Cell Tissue Bank 2003;4:29-35.

6. Gammaitoni L, Bruno S, Sanavio F, Gunetti M, Kollet O,

Cavalloni G, et al. Ex vivo expansion of human adult stem

cells capable of primary and secondary hemopoietic

reconstitution. Exp Hematol 2003;31:261-270.

7. Kobari L, Pflumio F, Giarratana M, Li X, Titeux M, Izac B,

et al. In vitro and in vivo evidence for the long-term

multilineage (myeloid, B, NK, and T) reconstitution

capacity of ex vivo expanded human CD34+ cord blood

cells. Exp Hematol 2000;28:1470-1480.

8. Piacibello W, Sanavio F, Garetto L, Severino A, Dané A,

Gammaitoni L, et al. Differential growth factor

requirement of primitive cord blood hematopoietic stem

cell for self-renewal and amplification vs. proliferation and

differentiation. Leukemia 1998;12:718-727.

9. Ueda T, Tsuji K, Yoshino H, Ebihara Y, Yagasaki H,

Hisakawa H, et al. Expansion of human NOD/ SCID-

repopulating cells by stem cell factor, Flk2/Flt3 ligand,

thrombopoietin, IL-6, and soluble IL-6 receptor. J Clin

Invest 2000;105:1013-1021.

10. Yamaguchi M, Hirayama F, Kanai M, Sato N, Fukazawa K,

115ªÂÛÂÁ¯˘Ì·ÙÈο Î·È ¤ÎÙ˘ÍË ÔÌÊ·ÏÔÏ·ÎÔ˘ÓÙÈ·ÎÔ‡ ·›Ì·ÙÔ˜

¶·È‰È·ÙÚÈ΋ 2009;72:115-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·115

Page 44: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

Yamashita K, et al. Serum-free co-culture system for exvivo expansion of human cord blood primitive progenitorsand SCID mouse-reconstituting cells using human bonemarrow primary stromal cells. Exp Hematol 2001;29:174-182.

11. Shih CC, Hu MC, Hu J, Medeiros J, Forman SJ. Long-termex vivo maintenance and expansion of transplantablehuman hematopoietic stem cells. Blood 1999;94:1623-1636.

12. Thiemann FT, Moore KA, Smogorzewska EM, LemischkaIR, Crooks GM. The murine stromal cell line AFT024 actsspecifically on human CD34-CD38- progenitors tomaintain primitive function and immunophenotype invitro. Exp Hematol 1998;26:612-619.

13. Kawada H, Ando K, Tsuji T, Shimakura Y, Nakamura Y,Chargui J, et al. Rapid ex vivo expansion of humanumbilical cord hematopoietic progenitors using a novelculture system. Exp Hematol 1999;27:904-915.

14. Woodbury D, Reynolds K, Black IB. Adult bone marrowstromal stem cells express germline, ectodermal,endodermal, and mesodermal genes prior to neurogenesis.J Neurosci Res 2002;69:908-917.

15. Campagnoli C, Roberts IA, Kumar S, Bennett PR,Bellantuono I, Fisk NM. Identification of mesenchymalstem/progenitor cells in human first-trimester fetal blood,liver, and bone marrow. Blood 2001;98:2396-2402.

16. Koc ON, Gerson SL, Cooper BW. Rapid hematopoieticrecovery after coinfusion of autologous-blood stem cellsand culture-expanded marrow mesenchymal stem cells inadvanced breast cancer patients receiving high-dosechemotherapy. J Clin Oncol 2000;18:307-316.

17. Lazarus HM., Koc ON, Devine SM. Cotransplantation ofHLA-identical sibling culture-expanded mesenchymalstem cells and hematopoietic stem cells in hematologicmalignancy patients. Biol Blood Marrow Transplant 2005;11:389-398.

18. Breems DA, Blokland EA, Siebel KE, Mayen AE, Engels LJ,Ploemacher RE. Stroma-contact prevents loss ofhematopoietic stem cell quality during ex vivo expansionof CD34+ mobilized peripheral blood stem cells. Blood.1998;91:111-117.

19. Dimitriou H, Vorgia P, Stiakaki E, Mavroudis D, MarkakiEA, Koumantakis E, et al. In vitro proliferative anddifferentiating characteristics of CD133(+) and CD34(+)cord blood cells in the presence of thrombopoietin (TPO) orerythropoietin (EPO). Potential implications for hemato-poietic cell transplantation. Leuk Res. 2003;27:1143-1151.

20. Kögler G, Nürnberger W, Fischer J, Niehues T, Somville T,

Göbel U, et al. Simultaneous cord blood transplantation ofex vivo expanded together with nonexpanded cells for highrisk leukemia. Bone Marrow Transplant 1999;24:397.

21. Koller MR, Manchel I, Maher RJ, Goltry KL, ArmstrongRD, Smith AK. Clinical-scale human umbilical cord bloodcell expansion in a novel automated perfusion culturesystem. Bone Marrow Transplant 1998;21:653.

22. XM Fei, YJ Wu, Z Chang, KR Miao, YH Tang, XY Zhou, etal. Co-culture of cord blood CD34 cells with human BMmesenchymal stromal cells enhance short-termengraftment of cord blood cells in NOD/SCID mice.Cytotherapy 2007;9:338-347.

23. Jang YK, Jung DH, Jung MH, Kim DH, Yoo KH, Sung KW,et al. Mesenchymal stem cells feeder layer from humanumbilical cord blood for ex vivo expanded growth andproliferation of hematopoietic progenitor cells. AnnHematol 2006;85:212-225.

24. Wexler SA, Donaldson C, Denning-Kendall P, Rice C,Bradley B, Hows JM. Adult bone marrow is a rich source ofhuman mesenchymal stem cells but umbilical cord andmobilized adult blood are not. Br J Haematol 2003;121:368-374.

25. Yu M, Xiao Z, Shen L, Li L. Mid-trimester fetal blood-derived adherent cells share characteristics similar tomesenchymal stem cells but full-term umbilical cord blooddoes not. Br J Haematol 2004;124:666-675.

26. Mareschi K, Biasin E, Piacibello W, Aglietta M, Madon E,Fagioli F. Isolation of human mesenchymal stem cells:bone marrow versus umbilical cord blood. Haematologica2001;86:1099-1100.

27. Chute JP, Saini AA, Kampen RL, Wells MR, Davis TA. Acomparative study of the cell cycle status and primitive celladhesion molecule profile of human CD34+ cells culturedin stromal-free versus porcine microvascular endothelialcell cultures. Exp Hematol 1999;27:370-379.

28. Haynesworth SE, Baber MA, Caplan AI. Cytokineexpression by human marrow-derived mesenchymalprogenitor cells in vitro: effects of dexamethasone and IL-1alpha. J Cell Physiol 1996;166:585-592.

29. Dormady SP, Bashayan O, Dougherty R, Zhang XM, BaschRS. Immortalized multipotential mesenchymal cells andthe hematopoietic microenvironment. J Hematother StemCell Res 2001;10:125-140.

30. Ueda T, Tsuji K, Yoshino H, Ebihara Y, Yagasaki H,Hisakawa H, et al. Expansion of human NOD/SCID-repopulating cells by stem cell factor, Flk2/Flt3 ligand,thrombopoietin, IL-6, and soluble IL-6 receptor. J ClinInvest 2000;105:1013-1021.

116 π. ¶ÂÏ·ÁÈ¿‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:116-000

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Page 45: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

¶·ÓÂÏÏ‹ÓÈ· ÔÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞ Û ·È‰È¿

ËÏÈΛ·˜ 0-14 ÂÙÒÓ*

µ. ¶··Â˘·ÁÁ¤ÏÔ˘1, ∞. ∫‡Úη1, ∞. ∆Ú·ÁÈ·ÓÓ›‰Ë˜2, ¢. ∫·Û›ÌÔ˜3, ∫. ¶·ÓÙÂÏ¿ÎË4, ∂. ¶·‡ÏÔ˘2, ª. ∆˙Ô‡ÊË5,

ª. ª·˘ÚÔÎÒÛÙ·6, ∞. ∆˙È‚¿Ú·˜4, •. ¶Â‰¤ÏË7, ∞. ÷Ù˙ËÌȯ·‹Ï3, º. ∞ı·Ó·ÛÈ¿‰Ô˘2, ¢. ∫·ÊÂÙ˙‹˜1,

∞. ∫ˆÓÛÙ·ÓÙfiÔ˘ÏÔ˜7

¶ÂÚ›ÏË„Ë

∂ÈÛ·ÁˆÁ‹: ∏ Ë·Ù›Ùȉ· ∞ ·ÔÙÂÏ› ÂÓ‰ËÌÈÎfi ÓfiÛËÌ· Û ÔÏϤ˜ ÂÚÈÔ¯¤˜ ÙÔ˘ ÎfiÛÌÔ˘. ∏ ∂ÏÏ¿‰· ηٷ-Ù¿ÛÛÂÙ·È ÛÙȘ ¯ÒÚ˜ ÂӉȿÌÂÛ˘ ÂÓ‰ËÌÈÎfiÙËÙ·˜, ·ÏÏ¿ ‰ÂÓ ˘¿Ú¯Ô˘Ó ·Ú΋ ÚfiÛÊ·Ù· ÔÚÔÂȉËÌÈÔ-ÏÔÁÈο ‰Â‰Ô̤ӷ ÁÈ· ÙË ÓfiÛÔ. ™ÎÔfi˜ Ù˘ ·ÚÔ‡Û·˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ·ÍÈÔÏfiÁËÛË Ù˘ ÔÚÔÂȉËÌÈÔÏÔÁ›·˜ÙÔ˘ ÈÔ‡ Ù˘ Ë·Ù›Ùȉ·˜ ∞, ·Ó¿ ËÏÈΛ· Î·È ÁˆÁÚ·ÊÈÎfi ‰È·Ì¤ÚÈÛÌ· Ù˘ ¯ÒÚ·˜, Û ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›Á-Ì· ·È‰ÈÒÓ ËÏÈΛ·˜ 0-14 ÂÙÒÓ.

ÀÏÈÎfi Î·È ª¤ıÔ‰ÔÈ: ™˘ÏϤ¯ıËÎ·Ó 100 ‰Â›ÁÌ·Ù· ÔÚÒÓ ·Ó¿ ËÏÈÎȷ΋ ÔÌ¿‰·, ‚¿ÛÂÈ ·ÔÁÚ·ÊÈÎÒÓ ‰Â‰Ô-̤ӈÓ, ·fi fiÏ· Ù· ÁˆÁÚ·ÊÈο ‰È·ÌÂÚ›ÛÌ·Ù· Ù˘ ¯ÒÚ·˜, Û‡Ìʈӷ Ì ÙÔ Û¯‹Ì· Û˘ÏÏÔÁ‹˜ ÂÓ·ÔÌÂÈ-Ó¿ÓÙˆÓ ÔÚÒÓ Ô˘ ¯ÚËÛÈÌÔÔÈ› ÙÔ Â˘Úˆ·˚Îfi ‰›ÎÙ˘Ô ÔÚÔÂÈÙ‹ÚËÛ˘ ÓÔÛËÌ¿ÙˆÓ Ô˘ ÚÔÏ·Ì‚¿ÓÔÓÙ·ÈÌ ÂÌ‚ÔÏÈ·ÛÌfi (∂SEN II), ÛÙÔ˘˜ ÔÔ›Ô˘˜ ¤ÁÈÓ ÚÔÛ‰ÈÔÚÈÛÌfi˜ ÙˆÓ IgG ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ Ù˘˷ٛÙȉ·˜ ∞ (AXSYM, Abbott). ∫·Ù·ÁÚ¿ÊËÎ·Ó Î·È ·Ó·Ï‡ıËÎ·Ó Ù· ÛÙÔȯ›· 1.393 ·È‰ÈÒÓ.

∞ÔÙÂϤÛÌ·Ù·: ∆· ·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ, ηıÒ˜ Î·È Ù· ·È‰È¿ Ô˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ·ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜, ›¯·ÓÛÙ·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›ËÛ˘. ™Ù· ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿, Ë ÔÚÔıÂÙÈÎfiÙËÙ··Ó‹Ïı ÛÙÔ 17,07%. ™Ù· ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ Î·Ù·ÁÚ¿ÊËΠ˘„ËÏfiÙÂÚÔ ÔÛÔÛÙfi ÔÚÔıÂÙÈ-ÎfiÙËÙ·˜ ÏfiÁˆ Ê˘ÛÈ΋˜ ÓfiÛËÛ˘, Û ۇÁÎÚÈÛË Ì ٷ ∂ÏÏËÓfiÔ˘Ï· (22,38% ¤Ó·ÓÙÈ 15,9%, OR=1,52, p=0,064).∂›Û˘, ηٷÁÚ¿ÊËÎ·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜ ÌÂٷ͇ ‰È·ÊÔÚÂÙÈÎÒÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ.

™˘ÌÂÚ¿ÛÌ·Ù·: ∞fi Ù· ÛÙÔȯ›· Ù˘ ÌÂϤÙ˘ Ì·˜ ÚÔ·ÙÂÈ fiÙÈ ÂÈ‚¿ÏÏÂÙ·È Ë ÂÊ·ÚÌÔÁ‹ ηıÔÏÈÎÔ‡ ÂÌ-‚ÔÏÈ·ÛÌÔ‡ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ ÛÙË ¯ÒÚ· Ì·˜.

§¤ÍÂȘ ÎÏÂȉȿ: ∏·Ù›Ùȉ· ∞, ÂÌ‚fiÏÈÔ, ÔÚÔÂȉËÌÈÔÏÔÁÈ΋.

Seroepidemiological study of hepatitis A in Greek children

aged 0-14 years

V. Papaevagelou1, A. Kyrka1, A. Tragiannidis2, D. Kassimos3, K. Pantelaki4, E. Pavlou2, M. Tzoufi5,

M. Mavrokosta6, A. Tzivaras4, X. Pedeli7, A. Hatzimichail3, F. Athanassiadou2, D. Kafetzis1,

A. Konstantopoulos7

Abstract

Background: Hepatitis A is a disease endemic in many areas of the world. Greece is considered a countryof intermediate endemicity, but recent data are lacking. A national cross-sectional seroprevalence surveywas conducted to evaluate the current seroepidemiology of hepatitis A in children in the age group 0-14years living in Greece.

Methods: Using the ESSEN II methodology, 100 sera per year of age, stratified by geographic region, werecollected and tested for the presence of anti-HAV IgG antibodies (AXSYM, Abbott Laboratories). Data from1,393 children were analyzed.

Results: Immigrant children and children residing in rural areas were significantly less likely to beimmunized. Among unvaccinated children, the seroprevalence was 17.07%. Nationality had a marginallysignificant effect, since nonimmunized immigrant children had a higher seroprevalence rate (22.38%versus 15.9%, OR=1.52, p=0.064). Significant differences were observed between geographic areas inboth vaccination coverage and natural immunity.

Conclusions: These data indicate that hepatitis A continues to prevail in Greece and therefore universalinfant immunization should be considered.

Key words: Hepatitis A, vaccine, seroepidemiology.

117∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞ ORIGINAL ARTICLE

1 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

2 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞.¶.£, ¡ÔÛÔÎÔÌ›Ô∞Ã∂¶∞, £ÂÛÛ·ÏÔÓ›ÎË

3 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¢.¶.£, ∞ÏÂÍ·Ó‰ÚÔ‡ÔÏË

4 ∂ÚÁ·ÛÙ‹ÚÈÔ ∞ÈÌÔ‰ÔÛ›·˜,°.¡. ¶·›‰ˆÓ «∞. ™ÔÊ›·»,∞ı‹Ó·

5 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ πˆ·ÓÓ›ÓˆÓ

6 ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,«µÂÓÈ˙¤ÏÂÈÔ» ¡ÔÛÔÎÔÌ›Ô∏Ú·ÎÏ›Ԣ

7 ™¯ÔÏ‹ ÀÁÈÂÈÓ‹˜ ηÈ∂ȉËÌÈÔÏÔÁ›·˜ ∂∫¶∞

AÏÏËÏÔÁÚ·Ê›·:

µ·ÛÈÏÈ΋ ¶··Â˘·ÁÁ¤ÏÔ˘[email protected], [email protected] µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,°ÂÓÈÎfi ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ«¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

1 2nd Department ofPaediatrics, GeneralChildren’s Hospital “P. & A. Kyriakou”,University of Athens, Athens, Greece

2 2nd Department ofPaediatrics, “AHEPA”Hospital, “Aristotelio”University of Thessaloniki,Thessaloniki, Greece

3 Department of Paediatrics,“Dimokritio” University ofThrace, Alexandroupolis,Greece

4 Laboratory for BloodDonation, “Agia Sophia”General Children’s Hospital,Athens, Greece

5 Department of Paediatrics,University of Ioannina,Ioannina, Greece

6 Department of Paediatrics,“Venizelio” Hospital ofHeraklion, Heraklion, Greece

7 School of Hygiene andEpidemiology, EKPA

Correspondence:

Vassiliki Papaevagelou [email protected],[email protected] 2nd Department ofPaediatrics, General Children’sHospital “P. & A. Kyriakou”,University of Athens, Athens,Greece

¶·È‰È·ÙÚÈ΋ 2009;72:117-000

* ∏ ÂÚÁ·Û›· ·˘Ù‹ ‚Ú·‚‡ıËΠ̠ÙÔ Œ·ıÏÔ ¢ÔÍÈ¿‰Ë, ηٿ ÙÔ 46Ô ¶·ÓÂÏÏ‹ÓÈÔ ¶·È‰È·ÙÚÈÎfi ™˘Ó¤‰ÚÈÔ, ∫¤Ú΢ڷ 2008

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·117

Page 46: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

∂ÈÛ·ÁˆÁ‹

∏ Ë·Ù›Ùȉ· ∞ ·ÔÙÂÏ› Û˘¯Ófi ÏÔÈÌ҉˜ ÓfiÛË-

Ì·, ·Ú¿ ÙËÓ Î˘ÎÏÔÊÔÚ›· ·ÔÙÂÏÂÛÌ·ÙÈÎÔ‡ ÂÌ‚ÔÏ›-

Ô˘ (1). ∞Ó Î·È Ù· ÔÛÔÛÙ¿ Ù˘ ›وۋ˜ Ù˘ ¤¯Ô˘Ó

ÌÂȈı› ÛËÌ·ÓÙÈο ηٿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÚÔËÁÔ‡-

ÌÂÓˆÓ ‰ÂηÂÙÈÒÓ ÛÙȘ ÂÚÈÛÛfiÙÂÚ˜ ·Ó·Ù˘Á̤Ó˜

¯ÒÚ˜, ÛËÌ·ÓÙÈ΋ ·ÏÏ·Á‹ ÛÙËÓ ÂȉËÌÈÔÏÔÁ›· ¤¯ÂÈ

·Ú·ÙËÚËı› ÛÙȘ ÂÚÈÔ¯¤˜ fiÔ˘ ÂÊ·ÚÌfiÛÙËΠη-

ıÔÏÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ (1-3). ™ÙËÓ ∂ÏÏ¿‰· ˘¿Ú¯Ô˘Ó

ÛÙÔȯ›· ·fi ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Û ÔÚÈṲ̂Ó˜

ÂÚÈÔ¯¤˜ Ù˘ ¯ÒÚ·˜ Ô˘ ·Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Ù· ÔÛÔ-

ÛÙ¿ ›وÛ˘ Ù˘ ÓfiÛÔ˘ ¤¯Ô˘Ó ÌÂȈı› ÛËÌ·ÓÙÈο

Î·È Ë Ì¤ÛË ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ Ì ˷ٛÙȉ· ∞ ¤¯ÂÈ

·˘ÍËı›, fiˆ˜ ÂÚÈÁÚ¿ÊÂÙ·È Î·È Û ¿ÏϘ Â˘Úˆ·˚-

Τ˜ ¯ÒÚ˜ (4-9). ∂ÓÙÔ‡ÙÔȘ, ÂıÓÈο ÔÚÔÂȉËÌÈÔÏÔ-

ÁÈο ÛÙÔȯ›· ‰ÂÓ Â›Ó·È ‰È·ı¤ÛÈÌ· ̤¯ÚÈ Û‹ÌÂÚ·, ·Ó

Î·È Ë ÔÍ›· Ë·Ù›Ùȉ· ∞ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙȘ ·Ûı¤-

ÓÂȘ Ô˘ Ú¤ÂÈ Ó· ‰ËÏÒÓÔÓÙ·È ˘Ô¯ÚˆÙÈο ÛÙÔ

∂ÏÏËÓÈÎfi ∫¤ÓÙÚÔ ∂ϤÁ¯Ô˘ Î·È ¶ÚfiÏ˄˘ ¡ÔÛËÌ¿-

ÙˆÓ (∫∂.∂§.¶.¡√).

°È· ÙËÓ ÂÈÛ·ÁˆÁ‹ ÂÓfi˜ ÂÌ‚ÔÏ›Ô˘ ÛÙÔ˘˜ ··Ú·›-

ÙËÙÔ˘˜ ÂÌ‚ÔÏÈ·ÛÌÔ‡˜ ÎÚ›ÓÂÙ·È ÛÎfiÈÌË Ë Û˘ÏÏÔÁ‹

‰Â‰ÔÌ¤ÓˆÓ ÁÈ· ÙÔÓ ÂÈÔÏ·ÛÌfi Ù˘ ÓfiÛÔ˘ Û ÌÈ·

ÏËı˘ÛÌȷ΋ ÔÌ¿‰·, ηıÒ˜ Î·È Ë Î·Ù·ÁÚ·Ê‹ ÙˆÓ

ÂÈÏÔÎÒÓ Ù˘. ¢˘ÛÙ˘¯Ò˜, Ù· ‰Â‰Ô̤ӷ ·fi ÙÔÓ ÂÏ-

ÏËÓÈÎfi ¯ÒÚÔ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÛÙÔȯ›· Û ÙÔÈÎfi ΢-

Ú›ˆ˜ Â›Â‰Ô Î·È ÂӉ¯Ô̤ӈ˜ ı· ÌÔÚÔ‡Û ӷ Á›-

ÓÂÈ ÂÎÙ›ÌËÛË Ù˘ ÓfiÛÔ˘ ÌfiÓÔ Ì ·Ó·‰ÚÔÌÈ΋ Û˘ÏÏÔ-

Á‹, ‚¿ÛÂÈ ÛÙÔȯ›ˆÓ ÙˆÓ ÂÈÛ·ÁˆÁÒÓ Û ·È‰È·ÙÚÈΤ˜

ÎÏÈÓÈΤ˜.

™ÎÔfi˜, ÂÔ̤ӈ˜, Ù˘ ·ÚÔ‡Û·˜ ÔÚÔÂȉËÌÈÔ-

ÏÔÁÈ΋˜ ÌÂϤÙ˘ ‹Ù·Ó Ë ·ÔÙ‡ˆÛË Ù˘ ·ÓÔÛ›·˜

¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞, ·Ó¿ ËÏÈΛ· Î·È ÁˆÁÚ·ÊÈÎfi

‰È·Ì¤ÚÈÛÌ· Ù˘ ¯ÒÚ·˜, Û ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›Á-

Ì· ·È‰ÈÒÓ 0-14 ÂÙÒÓ ·fi fiÏË ÙËÓ ∂ÏÏ¿‰·. ∆· ·Ô-

ÙÂϤÛÌ·Ù· Ù¤ÙÔÈ·˜ ÌÂϤÙ˘ ı· ·Ú¿Û¯Ô˘Ó ÂӉ¯Ô-

̤ӈ˜ ¯Ú‹ÛÈÌ· ÛÙÔȯ›· ÛÙËÓ ∂ıÓÈ΋ ∂ÈÙÚÔ‹

∂Ì‚ÔÏÈ·ÛÌÒÓ.

ÀÏÈÎfi Î·È Ì¤ıÔ‰ÔÈ

∞ÛıÂÓ›˜

√Úfi˜ ·›Ì·ÙÔ˜ ·ÓÙÈÚÔÛˆÂ˘ÙÈÎÔ‡ ‰Â›ÁÌ·ÙÔ˜ ·È‰ÈÒÓ 0-

14 ÂÙÒÓ ·fi fiÏË ÙËÓ ∂ÏÏ¿‰· ÂÍÂÙ¿ÛÙËΠÁÈ· ÙËÓ ·Ó›¯Ó¢ÛË

·ÓÙÈ-HAV ·ÓÙÈۈ̿وÓ. ™˘ÓÔÏÈο, ¤ÁÈÓÂ Û˘ÏÏÔÁ‹ 100 ÔÚÒÓ

ÁÈ· οı ËÏÈÎȷ΋ ÔÌ¿‰· Û ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ· ÂÓfi˜ ¤ÙÔ˘˜,

Û‡Ìʈӷ Ì ÙÔ Û‡ÛÙËÌ· Ù˘ Û˘ÏÏÔÁ‹˜ ÂÓ·ÔÌÂÈÓ¿ÓÙˆÓ ÔÚÒÓ

Ô˘ ¯ÚËÛÈÌÔÔÈ› ÙÔ Â˘Úˆ·˚Îfi ‰›ÎÙ˘Ô ÔÚÔÂÈÙ‹ÚËÛ˘ ÙˆÓ

ÓÔÛËÌ¿ÙˆÓ Ô˘ ÚÔÏ·Ì‚¿ÓÔÓÙ·È ÌÂ ÂÌ‚fiÏÈ· (∂SEN II), ÌÂ

ÛÎÔfi Ó· ¤¯Ô˘ÌÂ Û˘ÁÎÚ›ÛÈÌ· ·ÔÙÂϤÛÌ·Ù· Ì ·ÓÙ›ÛÙÔȯ˜

Â˘Úˆ·˚Τ˜ ÌÂϤÙ˜. ∆Ô ÔÛÔÛÙfi ÙˆÓ ·È‰ÈÒÓ ·fi οı Áˆ-

ÁÚ·ÊÈ΋ ÂÚÈÔ¯‹ ˘ÔÏÔÁ›ÛÙËΠ̠‚¿ÛË ÙËÓ ÂıÓÈ΋ ·ÔÁÚ·-

Ê‹ ÙÔ˘ 2001. ∂ÈϤÔÓ, ·fi ÙË ÌÂϤÙË ·ÔÎÏ›ÛÙËÎ·Ó Ù·

·È‰È¿ Ì ¯ÚfiÓÈ· ÓÔÛ‹Ì·Ù·, ÚˆÙÔ·ı›˜, ‰Â˘ÙÂÚÔ·ı›˜

·ÓÔÛÔ·Ó¿ÚÎÂȘ, ηıÒ˜ Î·È ÂΛӷ Ì ÚfiÛÊ·ÙË Ï‹„Ë ÎÔÈ-

Ó‹˜ Á-ÛÊ·ÈÚ›Ó˘. ∆· ‰Â›ÁÌ·Ù· ÙˆÓ ÔÚÒÓ ÛÙ¿ÏıËÎ·Ó Ù·¯˘‰ÚÔ-

ÌÈο ÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∞Á›· ™ÔÊ›·». ∆ËÚ‹ıËΠÙÔ

·fiÚÚËÙÔ ÙˆÓ ÚÔÛˆÈÎÒÓ ‰Â‰ÔÌ¤ÓˆÓ ÙˆÓ ·ÛıÂÓÒÓ Ô˘

Û˘ÌÌÂÙ›¯·Ó ÛÙË ÌÂϤÙË. ∆· ‰Â›ÁÌ·Ù· ‰È·ÙËÚ‹ıËÎ·Ó Û ıÂÚ-

ÌÔÎÚ·Û›· -20ÔC ̤¯ÚÈ ÙËÓ Âͤٷۋ ÙÔ˘˜. ∆· ‰Â‰Ô̤ӷ ÙˆÓ

·ÛıÂÓÒÓ ÂÈÛ‹¯ıËÛ·Ó Û ÂȉÈο ‰È·ÌÔÚʈ̤ÓË ‚¿ÛË ‰Â‰Ô̤-

ÓˆÓ Ì Έ‰ÈÎfi ·ÚÈıÌfi, ÒÛÙ ӷ ÂÍ·ÛÊ·ÏÈÛÙ› ·ÊÂÓfi˜ Ë ·Óˆ-

Ó˘Ì›· Î·È ·ÊÂÙ¤ÚÔ˘ Ë Ï‹Ú˘ ÚÔÛÙ·Û›· ÙˆÓ ÚÔÛˆÈÎÒÓ

‰Â‰ÔÌ¤ÓˆÓ Î¿ı ·ÛıÂÓÔ‡˜. °È· οı ·È‰› ¤ÁÈÓ ηٷÁÚ·Ê‹

Ù˘ ËÏÈΛ·˜, ÙÔ˘ ʇÏÔ˘, ÙÔ˘ ÙfiÔ˘ ‰È·ÌÔÓ‹˜, Ù˘ ˘ËÎÔfiÙË-

Ù·˜ Î·È Ù˘ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ ÙÔ˘ Î¿Ï˘„˘. ∆· ‰Â›ÁÌ·Ù· ÔÚÒÓ

ÂÍÂÙ¿ÛÙËÎ·Ó ÁÈ· ÙËÓ ·ÚÔ˘Û›· ·ÓÙÈÛˆÌ¿ÙˆÓ ·ÓÙÈ-HAV IgG

ÌÂ ¯Ú‹ÛË ÙÔ˘ ÙÂÛÙ HAVAB 2.0 (AXSYM, Abbott).

ŸÛÔÓ ·ÊÔÚ¿ ÛÙ· ‚Ú¤ÊË ËÏÈΛ·˜ <12 ÌËÓÒÓ, Ë ·Ó‡ÚÂÛË

ıÂÙÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ·Ô‰fiıËΠ۠·ıËÙÈ΋ ·ÓÔÛ›· ÏfiÁˆ

ÌËÙÚÈÎÒÓ ·ÓÙÈۈ̿وÓ.

™Ù·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË

∏ ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË ¤ÁÈÓ Ì ÙË ¯Ú‹ÛË ÙÔ˘ ÏÔÁÈÛÌÈÎÔ‡

·Î¤ÙÔ˘ Stata (version 8) Î·È ˆ˜ fiÚÈÔ ÛÙ·ÙÈÛÙÈ΋˜ ÛËÌ·ÓÙÈ-

ÎfiÙËÙ·˜ ıˆڋıËÎÂ Ë ÙÈÌ‹ p<0,05. ªÂ ¯Ú‹ÛË ÌÔÓÔÌÂÙ·‚ÏË-

Ù‹˜ ÏÔÁÈÛÙÈ΋˜ ·ÏÈÓ‰ÚfiÌËÛ˘, ÂϤÁÍ·Ì ÙËÓ Â›‰Ú·ÛË Ù˘

ËÏÈΛ·˜, Ù˘ ÂıÓÈÎfiÙËÙ·˜ Î·È ÙÔ˘ ÙfiÔ˘ ‰È·ÌÔÓ‹˜ ÙˆÓ ·ÛıÂ-

ÓÒÓ ÛÙËÓ Â›ÙˆÛË Ù˘ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ ÙÔ˘˜ Î¿Ï˘„˘, ·ÏÏ¿

Î·È Ù˘ Ê˘ÛÈ΋˜ ·ÓÔÛ›·˜ Û ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿. ∆·

·ÔÙÂϤÛÌ·Ù· ÂÎÊÚ¿ÛÙËÎ·Ó Ì Odds Ratio (OR) Î·È 95%

‰È¿ÛÙËÌ· ·ÍÈÔÈÛÙ›·˜. ∏ ÛÙ·ÙÈÛÙÈ΋ ÛËÌ·ÓÙÈÎfiÙËÙ· (p-

value) ˘ÔÏÔÁ›ÛÙËΠ̠ÙÔ Wald test.

∞ÔÙÂϤÛÌ·Ù·

∏ Û˘ÏÏÔÁ‹ ÙˆÓ ‰ÂÈÁÌ¿ÙˆÓ Ù˘ ÌÂϤÙ˘ ¤ÁÈÓ ÌÂ-ٷ͇ ÙÔ˘ π·ÓÔ˘·Ú›Ô˘ 2006 Î·È ÙÔ˘ ¢ÂÎÂÌ‚Ú›Ô˘ 2006,Û‡Ìʈӷ Ì ÙË ÌÂıÔ‰ÔÏÔÁ›· Ù˘ ÌÂϤÙ˘, ·fi ÙȘ‰È¿ÊÔÚ˜ ÂÚÈÔ¯¤˜ Ù˘ ∂ÏÏ¿‰·˜. ∞fi ÙËÓ ·Ó¿Ï˘ÛËÙˆÓ ‰ËÌÔÁÚ·ÊÈÎÒÓ ÛÙÔȯ›ˆÓ 1.393 ‰ÂÈÁÌ¿ÙˆÓ ÚÔ-¤Î˘„ fiÙÈ Ù· ·È‰È¿ ·fi ÔÈÎÔÁ¤ÓÂȘ ÌÂÙ·Ó·ÛÙÒÓ·ÓÙÈÚÔÛÒ¢·Ó ÙÔ 12,6% (175/1383), ÂÓÒ Û‡Ìʈ-Ó· Ì ÚfiÛÊ·Ù· ·ÔÁÚ·ÊÈο ‰Â‰Ô̤ӷ (2001), ÔÈÌÂÙ·Ó¿ÛÙ˜ ·ÔÙÂÏÔ‡Ó ÂÚ›Ô˘ ÙÔ 10% ÙÔ˘ ÏËı˘-ÛÌÔ‡ Ù˘ ¯ÒÚ·˜ Ì·˜. ™˘ÓÔÏÈο ·Ó·Ï‡ıËÎ·Ó 1.374‰Â›ÁÌ·Ù· ·›Ì·ÙÔ˜, ·ÊÔ‡ ·fi ÙÔ Û‡ÓÔÏÔ ÙˆÓ 1.393‰ÂÈÁÌ¿ÙˆÓ Ù· 19 ‹Ù·Ó ·ÈÌÔÏ˘Ì¤Ó· Î·È ÂÔ̤ӈ˜·ÔÎÏ›ÛıËÎ·Ó ·fi ÙË ÌÂϤÙË.

∞fi Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ ÌÂϤÙ˘ Ì·˜ ÚԤ΢-„ fiÙÈ ÙÔ 38% (438/1152) ÙˆÓ ·È‰ÈÒÓ ËÏÈΛ·˜ >24ÌËÓÒÓ Â›¯Â ÂÌ‚ÔÏÈ·ÛÙ› ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞. ∆ÔÔÛÔÛÙfi ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ Î¿Ï˘„˘ (·ÓÔÛ›·˜) ÛÙ··È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ ‹Ù·Ó ÛÙ·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ·-ÌËÏfiÙÂÚÔ (22,3%, 35/157), Û ۯ¤ÛË Ì ÙÔ ·ÓÙ›ÛÙÔÈ-¯Ô ÙˆÓ EÏÏËÓfiÔ˘ÏˆÓ (40,5%, 403/995) (OR=0,42,95%CI: 0,28-0.63, p<0,001) (∂ÈÎfiÓ· 1). ∂ÈϤÔÓ, Ù··È‰È¿ Ô˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ·ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜ÂÌÊ¿ÓÈ˙·Ó ¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ ο-Ï˘„˘ (·ÓÔÛ›·˜), Û ۇÁÎÚÈÛË Ì ٷ ·ÓÙ›ÛÙÔȯ··ÛÙÈÎÒÓ ÂÚÈÔ¯ÒÓ (31,04% ¤Ó·ÓÙÈ 40,88%,OR=1,54, 95% CI: 1,17-2,01, p=0,002) (∂ÈÎfiÓ· 1).

™Ù· ·ÓÔÛÔÔÈË̤ӷ ·È‰È¿, Ë ÔÚÔıÂÙÈÎfiÙËÙ··Ó‹Ïı ÛÙÔ 90,1% (393/436) ÁÈ· ·È‰È¿ Ô˘ ¤Ï·‚·Ó

118 µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.

Paediatriki 2009;72:118-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·118

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Ì›· ‰fiÛË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ Î·È ÛÙÔ 91,2% (323/354) ÁÈ·

ÂΛӷ Ô˘ ¤Ï·‚·Ó 2 ‰fiÛÂȘ. ∞fi Ù· 131 ‚Ú¤ÊË ËÏÈ-

Λ·˜ 0-12 ÌËÓÒÓ Ù˘ ÌÂϤÙ˘, 41 (31,29%) ‹Ù·Ó ÔÚÔ-

ıÂÙÈο, ÁÂÁÔÓfi˜ ÂÓ‰ÂÈÎÙÈÎfi ·ıËÙÈ΋˜ ·ÓÔÛ›·˜ ÏfiÁˆ

ÌËÙÚÈÎÒÓ ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞.

Ÿˆ˜ ‹Ù·Ó ·Ó·ÌÂÓfiÌÂÓÔ, Ù· ·È‰È¿ ËÏÈΛ·˜ <12 ÌË-

ÓÒÓ Ô˘ ÚÔ¤Ú¯ÔÓÙ·Ó ·fi ÔÈÎÔÁ¤ÓÂȘ ÌÂÙ·Ó·ÛÙÒÓ

›¯·Ó ÛÙ·ÙÈÛÙÈÎÒ˜ ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿

ÔÚÔıÂÙÈÎfiÙËÙ·˜ (23/41, 56,1%), Û ۇÁÎÚÈÛË Ì ÙÔ

·ÓÙ›ÛÙÔÈ¯Ô ÔÛÔÛÙfi ÙˆÓ ∂ÏÏËÓfiÔ˘ÏˆÓ (18/90,

20%) (OR=5,11, 95% CI: 2,29-11,43, p<0,001).

∞fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ·ÔÙÂÏÂÛÌ¿ÙˆÓ fiÛÔÓ

·ÊÔÚ¿ ÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë ¤Ó·ÓÙÈ Ù˘ Ë·Ù›-

Ùȉ·˜ ∞ ·Ó¿ ÁˆÁÚ·ÊÈ΋ ÂÚÈÔ¯‹, ÚԤ΢„·Ó ˘„Ë-

Ï¿ ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›ËÛ˘ Û ∂ÏÏËÓfiÔ˘Ï· ÚÔÂÚ-

¯fiÌÂÓ· ·fi ÙËÓ ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· (54,07%), ÂÓÒ Ù·

¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ηٷÁÚ¿ÊËÎ·Ó ÛÙËÓ ÂÚÈÔ¯‹

Ù˘ µfiÚÂÈ·˜ ∂ÏÏ¿‰·˜ (21,82%). ∆· ÔÛÔÛÙ¿ ·ÓÔÛÔ-

Ô›ËÛ˘ ∂ÏÏËÓfiÔ˘ÏˆÓ ‰È¤ÊÂÚ·Ó ÛËÌ·ÓÙÈο ÌÂÙ·-

͇ ÙˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ, fiˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ

¶›Ó·Î· 1.

∆Ô ÔÛÔÛÙfi ÔÚÔıÂÙÈÎfiÙËÙ·˜ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›ÙÈ-

‰·˜ ∞ Û ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰ÈÒÓ ËÏÈΛ·˜ >12 ÌË-

ÓÒÓ, ÏfiÁˆ Ê˘ÛÈ΋˜ ÓfiÛËÛ˘, ·Ó‹Ïı Û 17,07%

(136/797). ∆Ô ÔÛÔÛÙfi ÙˆÓ ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ÔÚÔ-

ıÂÙÈÎÒÓ ∂ÏÏËÓfiÔ˘ÏˆÓ ‹Ù·Ó ¯·ÌËÏfiÙÂÚÔ (15,90%,

104/654), Û ۇÁÎÚÈÛË Ì ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô ÛÙ· ·È‰È¿

ÌÂÙ·Ó·ÛÙÒÓ (32/143, 22,38%) (OR=1,52, 95% CI:

0,98-2,38, p=0,064), ÂÓÒ ÛÙÔÓ ¶›Ó·Î· 2 Ê·›ÓÔÓÙ·È Ù·

·ÓÙ›ÛÙÔȯ· ÔÛÔÛÙ¿ ·Ó¿ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· ηÈ

ÁˆÁÚ·ÊÈÎfi ‰È·Ì¤ÚÈÛÌ· Ù˘ ¯ÒÚ·˜.

∞fi ÙËÓ ·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ Ù˘ ÌÂϤÙ˘

Ì·˜ ·Ó¿ ÏËı˘ÛÌȷ΋ ÔÌ¿‰·, ηٷÁÚ¿ÊËΠÛÙ·ÙÈ-

ÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ·ÓÔÛ›·

ÏfiÁˆ ÓfiÛËÛ˘ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ ÌfiÓÔ ÁÈ·

·È‰È¿ ·fi ÙËÓ ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰·, Ô˘ ·ÓÙÈÚÔÛˆ-

‡ÂÈ Î·È ÙËÓ ÂÚÈÔ¯‹ Ì ÙË ÌÂÁ·Ï‡ÙÂÚË ÂÌ‚ÔÏÈ·-

ÛÙÈ΋ Î¿Ï˘„Ë (9/191, 4,71% ¤Ó·ÓÙÈ 14/76, 18,42%)

(OR=4,57, 95% CI: 1,88-11,07, p=0,001) (¶›Ó·Î·˜

3). ∂›Û˘, ·ÍÈÔÛËÌ›ˆÙ· Â›Ó·È Ù· ˘„ËÏ¿ ÔÛÔÛÙ¿

Ê˘ÛÈ΋˜ ·ÓÔÛ›·˜ ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ·È‰ÈÒÓ ·fi

ÙËÓ ÂÚÈÔ¯‹ Ù˘ µfiÚÂÈ·˜ ∂ÏÏ¿‰·˜, ÙfiÛÔ ÁÈ· Ù·

¶›Ó·Î·˜ 1. ¢È·ÊÔÚ¤˜, ·Ó¿ÏÔÁ· Ì ÙÔÓ ÙfiÔ ‰È·ÌÔÓ‹˜ Î·È ÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞, ÌÂٷ͇ ∂ÏÏËÓfiÔ˘ÏˆÓ Î·È ÌÂÙ·Ó·ÛÙÒÓ

∂ÏÏËÓfiÔ˘Ï· ¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓN (%) OR ∆ÈÌ‹ p N (%) OR ∆ÈÌ‹ p

(95% CI) (95% CI)

∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· 226/418 Ref† 26/102 Ref†

(54,07%) (25,49%)µfiÚÂÈ· ∂ÏÏ¿‰· 79/362 0,24 <0,001 5/44 0,37 0,062

(21,82%) (0,17-0,32) (11,36%) (0,13-1,05)¶ÂÏÔfiÓÓËÛÔ˜ 108/294 0,49 <0,001 4/32 0,42 0,133Î·È ÓËÛÈ¿ (36,73%) (0,36-0,67) (12,50%) (0,13-1,30)™‡ÓÔÏÔ 413/1074 35/178

(38,45%) (19,66%)

† Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜

119√ÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞

¶·È‰È·ÙÚÈ΋ 2009;72:119-000

45

40

35

30

25

20

15

10

5

0

40

35

30

25

20

15

10

5

%p<0,001

∂ÏÏËÓfiÔ˘Ï· ¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ

40,5

22,3

p=0,002

∞ÛÙÈΤ˜ ÂÚÈÔ¯¤˜ ∞ÁÚÔÙÈΤ˜ ÂÚÈÔ¯¤˜

40,8

31

∂ÈÎfiÓ· 1. ¶ÔÛÔÛÙ¿ ·ÓÔÛÔÔ›ËÛ˘ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ Û ·È‰È¿ ËÏÈΛ·˜ >24 ÌËÓÒÓ, Û ۯ¤ÛË Ì ÙËÓ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· Î·È ÙÔÓÙfiÔ ‰È·ÌÔÓ‹˜.

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·119

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∂ÏÏËÓfiÔ˘Ï· fiÛÔ Î·È ÁÈ· Ù· ·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓ

(26,98% Î·È 41,03% ·ÓÙ›ÛÙÔȯ·) (¶›Ó·Î·˜ 3).

™˘˙‹ÙËÛË

√ Èfi˜ Ë·Ù›Ùȉ·˜ ∞ (HAV) ÌÂÙ·‰›‰ÂÙ·È Ì ÙËÓ

ηٿÔÛË ÌÔÏ˘ÛÌ¤ÓˆÓ ÙÚÔÊ›ÌˆÓ ‹ ‡‰·ÙÔ˜. ∏ Ìfi-

Ï˘ÓÛË, fiˆ˜ ›Û˘ Î·È Ô ÂÌ‚ÔÏÈ·ÛÌfi˜, ·Ú¤¯Ô˘Ó

ÈÛfi‚È· ·ÓÔÛ›· (1,9). ∏ Èı·ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ Û˘-

ÌÙˆÌ¿ÙˆÓ ·fi ÙË ÌfiÏ˘ÓÛË Ì HAV Û˘Û¯ÂÙ›˙ÂÙ·È

Ì ÙËÓ ËÏÈΛ·, ‰Â‰Ô̤ÓÔ˘ fiÙÈ Û ·È‰È¿ ÌÈÎÚfiÙÂÚ·

ÙˆÓ 6 ÂÙÒÓ ÔÈ ÂÚÈÛÛfiÙÂÚ˜ ÌÔχÓÛÂȘ Â›Ó·È ·Û˘-

Ìو̷ÙÈΤ˜ ‹ ·ÓÈÎÙÂÚÈΤ˜ (1,2). ∆· ·È‰È¿ Ì ·Û˘-

Ìو̷ÙÈ΋ ÌfiÏ˘ÓÛË ÌÔÚÔ‡Ó Ó· ·ÔÙÂϤÛÔ˘Ó Ë-

Á‹ ÌfiÏ˘ÓÛ˘ ÁÈ· Ù· ËÏÈÎȈ̤ӷ ¿ÙÔÌ· (3).

∆Ș ÙÂÏÂ˘Ù·›Â˜ ‰ÂηÂٛ˜, ÙÔ ÔÛÔÛÙfi ÙˆÓ ·Ùfi-

ÌˆÓ Ì ·ÓÙÈÛÒÌ·Ù· ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ Ù˘ Ë·Ù›Ùȉ·˜ ∞

¤¯ÂÈ ÌÂȈı› ÛÙ· ÂÚÈÛÛfiÙÂÚ· ̤ÚË ÙÔ˘ ÎfiÛÌÔ˘ (8).

∂Ó ÙÔ‡ÙÔȘ, ÂÚ›Ô˘ 1,4 ÂηÙÔÌ̇ÚÈ· Ӥ˜ ÌÔχÓ-

ÛÂȘ ·fi ÙÔÓ Èfi ·Ó·Ì¤ÓÂÙ·È Ó· ÂÌÊ·ÓÈÛÙÔ‡Ó ·ÁÎÔ-

ÛÌ›ˆ˜ οı ¯ÚfiÓÔ (8). ¶·Ú¿ ÙË Ì›ˆÛË Ô˘ ηٷ-

ÁÚ¿ÊÂÙ·È ÛÙ· ÔÚÔÂȉËÌÈÔÏÔÁÈο ÔÛÔÛÙ¿, Ë Ë·Ù›-

Ùȉ· ∞ Û˘Ó¯›˙ÂÈ Ó· Â›Ó·È Ì›· ·fi ÙȘ Ôχ Û˘¯Ó¿

·Ó·ÊÂÚfiÌÂÓ˜ ·Ûı¤ÓÂȘ Ô˘ ÌÔÚÔ‡Ó Ó· ÚÔÏË-

ÊıÔ‡Ó Ì ÂÌ‚ÔÏÈ·ÛÌfi ·ÁÎÔÛÌ›ˆ˜ (8).

∏ ∂ÏÏ¿‰· ıˆÚÂ›Ù·È ¯ÒÚ· Ì ÂӉȿÌÂÛË ÂÓ‰ËÌÈ-

ÎfiÙËÙ·, ·ÚfiÏÔ Ô˘ ‰ÂÓ ¤¯Ô˘Ì ÚfiÛÊ·Ù· ‰Â‰Ô̤-

Ó·. ∫¿ÔȘ ÌÈÎÚ¤˜ ÌÂϤÙ˜ ·Ó·Ê¤ÚÔ˘Ó Ì›ˆÛË ÙÔ˘

ÂÈÔÏ·ÛÌÔ‡ Ù˘ ÓfiÛÔ˘ ‹‰Ë ·fi ÙË ‰ÂηÂÙ›· ÙÔ˘

’70 (5,6). ™‡Ìʈӷ Ì ÙȘ ÌÂϤÙ˜ ·˘Ù¤˜, ÔÛÔÛÙfi

ÌÈÎÚfiÙÂÚÔ ·fi 10% ÙˆÓ ·È‰ÈÒÓ ·ÏÏ¿ ÌÂÁ·Ï‡ÙÂÚÔ

·fi 90% ÙˆÓ ÂÓËÏ›ÎˆÓ ¿Óˆ ÙˆÓ 45 ÂÙÒÓ ¤¯ÂÈ ·ÓÙÈ-

ÛÒÌ·Ù· ¤Ó·ÓÙÈ ÙÔ˘ ÈÔ‡ (5-7). ™Â ÌÈ· ·Ó·‰ÚÔÌÈ΋ ÌÂ-

ϤÙË Ô˘ ‰ÈÂÓÂÚÁ‹ıËΠÛÙÔ ¡ÔÛÔÎÔÌÂ›Ô ∂ȉÈÎÒÓ

¶·ı‹ÛÂˆÓ £ÂÛÛ·ÏÔӛ΢, ÌÂٷ͇ ÙˆÓ ÂÙÒÓ 1985-

1992 ηٷÁÚ¿ÊËΠÛÙ·ıÂÚ‹ Ì›ˆÛË ÙˆÓ ÂÈÛ·ÁˆÁÒÓ

(6). ∂ÈϤÔÓ, ÛËÌÂÈÒıËΠ·‡ÍËÛË Ù˘ ̤Û˘ ËÏÈ-

Λ·˜ ÙˆÓ ·ÛıÂÓÒÓ, ·fi 0-5 ¤ÙË ÙÔ 1985 Û 21-25 ¤ÙË

ÙÔ 1992 (6). ∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÚÔËÁÔ‡ÌÂÓ˘ ‰Â-

ηÂÙ›·˜, ˘‹ÚÍ ÌÂÁ¿ÏË ÏËı˘ÛÌȷ΋ ·ÏÏ·Á‹ ÛÙËÓ

∂ÏÏ¿‰·, ÏfiÁˆ Ù˘ ÂÈÛfi‰Ô˘ ÛÙË ¯ÒÚ· ÔÈÎÔÓÔÌÈÎÒÓ

ÚÔÛʇÁˆÓ ·fi ÙËÓ ∞Ó·ÙÔÏÈ΋ ∂˘ÚÒË Î·È Ù· µ·Ï-

οÓÈ·. ™‡Ìʈӷ Ì ÙËÓ ÚfiÛÊ·ÙË ·ÔÁÚ·Ê‹ ÙÔ˘

2001, ÔÈ ÌÂÙ·Ó¿ÛÙ˜ Û˘Óı¤ÙÔ˘Ó ÂÚ›Ô˘ ÙÔ 10% ÙÔ˘

ÏËı˘ÛÌÔ‡ Ù˘ ¯ÒÚ·˜. ™‡Ìʈӷ Ì ÙÔ˘˜ Frosner

Î·È Û˘Ó., ˘¿Ú¯Ô˘Ó ‰Â‰Ô̤ӷ fiÙÈ ÔÈ ÌÂÙ·Ó¿ÛÙ˜

¤¯Ô˘Ó ÛËÌ·ÓÙÈο ˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ HAV ·ÓÙÈÛˆ-

Ì¿ÙˆÓ (10). ∆¤ÏÔ˜, ·Ó Î·È Ë Ë·Ù›Ùȉ· ∞ ·ÔÙÂÏ›

¤Ó· ·fi Ù· ÓÔÛ‹Ì·Ù· Ô˘ ‰ËÏÒÓÔÓÙ·È ˘Ô¯ÚˆÙÈο

¶›Ó·Î·˜ 3. ¢È·ÊÔÚ¤˜, ·Ó¿ÏÔÁ· Ì ÙËÓ ÏËı˘ÛÌȷ΋ ÔÌ¿‰· Î·È ÙË Ê˘ÛÈ΋ ·ÓÔÛ›· ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ·È‰ÈÒÓ >12 ÌËÓÒÓ, ÌÂٷ͇ ÁˆÁÚ·-ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ

∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· µfiÚÂÈ· ∂ÏÏ¿‰· ¶ÂÏÔfiÓÓËÛÔ˜ Î·È ÓËÛÈ¿N (%) OR p N (%) OR p N (%) OR p

(95% CI) (95% CI) (95% CI)

∂ÏÏËÓfiÔ˘Ï· 9/191 Ref† 75/278 Ref† 20/185 Ref†

(4,71%) (26,98%) (10,81%)ªÂÙ·Ó¿ÛÙ˜ 14/76 4,57 0,001 16/39 1,88 0,073 2/28 0,63 0,555

(18,42%) (1,88-11,07) (41,03%) (0,94-3,76) (7,14%) (0,14-2,88)™‡ÓÔÏÔ 23/267 91/317 22/213

(8,61%) (28,71%) (10,33%)

† Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜

120 µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.

Paediatriki 2009;72:120-000

¶›Ó·Î·˜ 2. ¢È·ÊÔÚ¤˜, ·Ó¿ÏÔÁ· Ì ÙÔÓ ÙfiÔ ‰È·ÌÔÓ‹˜ Î·È ÙË Ê˘ÛÈ΋ ·ÓÔÛ›· ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞ ÌË ÂÌ‚ÔÏÈ·ÛÌ¤ÓˆÓ ·È‰ÈÒÓ ËÏÈΛ·˜>12 ÌËÓÒÓ, ÌÂٷ͇ ∂ÏÏËÓfiÔ˘ÏˆÓ Î·È ÌÂÙ·Ó·ÛÙÒÓ

∂ÏÏËÓfiÔ˘Ï· ¶·È‰È¿ ÌÂÙ·Ó·ÛÙÒÓN (%) OR ∆ÈÌ‹ p N (%) OR ∆ÈÌ‹ p

(95% CI) (95% CI)

∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· 9/191 Ref† 14/76 Ref†

(4,71%) (18,42%)µfiÚÂÈ· ∂ÏÏ¿‰· 75/278 7,47 <0,001 16/39 3,08 0,011

(26,98%) (3,64-15,35) (41,03%) (1,30-7,30) 0,174¶ÂÏÔfiÓÓËÛÔ˜ 20/185 2,45 0,031 2/28 0,34Î·È ÓËÛÈ¿ (10,81%) (1,09-5,53) (7,14%) (0,07-1,61)™‡ÓÔÏÔ 104/654 32/143

(15,90%) (22,38%)

† Ref: ÔÌ¿‰· ·Ó·ÊÔÚ¿˜

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·120

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·fi Ù· Ù¤ÏË ÙÔ˘ ’90, Ë Î·Ù·ÁÚ·Ê‹ Â›Ó·È ÂÏÏÈ‹˜.

™ÙÔȯ›· ·fi ÙÔ ∫∂∂§ ‰Â›¯ÓÔ˘Ó fiÙÈ Ë Ë·Ù›Ùȉ· ∞

·ÔÙÂÏ› Úfi‚ÏËÌ· ‰ËÌfiÛÈ·˜ ˘Á›·˜ ÁÈ· ÙËÓ ∂ÏÏ¿-

‰· Î·È ÙÔ 25-50% ÙˆÓ ÂÚÈÙÒÛÂˆÓ Ô˘ ‰ËÏÒÓÔ-

ÓÙ·È ÂÙËÛ›ˆ˜ Â›Ó·È ·È‰È¿.

∆Ô ÂÌ‚fiÏÈÔ Ë·Ù›Ùȉ·˜ ∞ ¤¯ÂÈ ¿‰ÂÈ· ¯ÔÚ‹ÁËÛ˘

Û ·È‰È¿ >2 ÂÙÒÓ Î·È Û ÂÓ‹ÏÈΘ (2). ¶ÚfiÛÊ·Ù·,

ÙÔ FDA ÂÓ¤ÎÚÈÓ ÙË ¯Ú‹ÛË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ VAQTA

ÛÙ· Ó‹È· 12-24 ÌËÓÒÓ (11). ∞Ó Î·È ·Ú¯Èο Ô ÂÌ‚Ô-

ÏÈ·ÛÌfi˜ Û˘ÛÙ‹ıËΠÁÈ· ÙȘ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÔÌ¿-

‰Â˜ ÌfiÓÔ, ¤¯Ô˘Ó Á›ÓÂÈ ÚÔÁÚ¿ÌÌ·Ù· Ì·˙ÈÎÔ‡ ÂÌ‚Ô-

ÏÈ·ÛÌÔ‡ ÛÙȘ ÂÚÈÔ¯¤˜ Ì ÂӉȿÌÂÛË ÂÓ‰ËÌÈÎfiÙËÙ·

·fi ÙÔ 1997, fiˆ˜ ÁˆÁÚ·ÊÈο ‰È·ÌÂÚ›ÛÌ·Ù· ÛÙËÓ

πÙ·Ï›·, πÛ·Ó›· Î·È ∞˘ÛÙÚ·Ï›· (12). ¶ÚfiÛÊ·Ù·, ÙÔ

πÛÚ·‹Ï ·ÚÔ˘Û›·Û ÌÈ· ÛËÌ·ÓÙÈ΋ Ì›ˆÛË (>95%)

ÙÔ˘ ÂÙ‹ÛÈÔ˘ ÔÛÔÛÙÔ‡ ›وÛ˘ Ù˘ Ë·Ù›Ùȉ·˜ ∞,

5 ¯ÚfiÓÈ· ÌÂÙ¿ ÙËÓ ÂÊ·ÚÌÔÁ‹ ˘Ô¯ÚˆÙÈÎÔ‡ ÂÌ‚Ô-

ÏÈ·ÛÌÔ‡ ÙˆÓ ÌÈÎÚÒÓ ·È‰ÈÒÓ ÙÔ 1999 (13). ¶ÚfiÛÊ·-

Ù· ‰ËÌÔÛÈÂ˘Ì¤Ó· ·ÔÙÂϤÛÌ·Ù· ÂÌÊ·Ó›˙Ô˘Ó ÛËÌ·-

ÓÙÈ΋ Ì›ˆÛË Ù˘ Ë·Ù›Ùȉ·˜ ∞ Û ÔÏÈÙ›˜ ÙˆÓ

∏¶∞ fiÔ˘ ÂÊ·ÚÌfiÛÙËÎÂ Ë ÔÏÈÙÈ΋ ÙÔ˘ Ì·˙ÈÎÔ‡

ÂÌ‚ÔÏÈ·ÛÌÔ‡ (88%) (ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë 50%)

(14). ∏ ÌÂÁ·Ï‡ÙÂÚË Ì›ˆÛË ·Ú·ÙËÚ‹ıËΠ۠·È-

‰È¿ 2-9 ÂÙÒÓ, ·ÏÏ¿ Ë Â›ÙˆÛË ¤¯ÂÈ ÌÂȈı› ÛËÌ·-

ÓÙÈο Î·È ÛÙÔ˘˜ ÂÓ‹ÏÈΘ, ηٷ‰ÂÈÎÓ‡ÔÓÙ·˜ ÌÂȈ̤-

ÓË ÌÂÙ¿‰ÔÛË ÙÔ˘ ÈÔ‡ ̤۷ ÛÙËÓ ÎÔÈÓfiÙËÙ· (14).

∞fi Ù· ‰Â‰Ô̤ӷ Ù˘ ÌÂϤÙ˘ Ì·˜ ÚԤ΢„ fiÙÈ

¤Ó· ÛËÌ·ÓÙÈÎfi ÔÛÔÛÙfi ·È‰ÈÒÓ ËÏÈΛ·˜ ¿Óˆ ÙˆÓ 2

ÂÙÒÓ (38%) ÂÌ‚ÔÏÈ¿˙ÂÙ·È ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜ ∞,

·ÚfiÏÔ Ô˘ ̤¯ÚÈ Û‹ÌÂÚ· ÙÔ Û˘ÁÎÂÎÚÈ̤ÓÔ ÂÌ‚fiÏÈÔ

‰ÂÓ ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙÔ ∂ıÓÈÎfi ¶ÚfiÁÚ·ÌÌ· ∂Ì‚Ô-

ÏÈ·ÛÌÒÓ. ∂Ô̤ӈ˜, Ë ·ÁÔÚ¿ ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ ÂÈ‚·-

Ú‡ÓÂÈ ÙÔÓ ÁÔÓ¤·, ÂÓÒ Ë Û˘Ì‚ÔÏ‹ ÙÔ˘ ·È‰È¿ÙÚÔ˘ ÎÚ›-

ÓÂÙ·È Ô˘ÛÈ·ÛÙÈ΋ ÛÙËÓ ˘„ËÏ‹ ÁÈ· Ù· ÂıÓÈο ‰Â‰Ô̤-

Ó· ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë.

∞fi Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜ ÚԤ΢„Â, fiˆ˜ ¿Ï-

ψÛÙ ·Ó·ÌÂÓfiÙ·Ó, fiÙÈ Ù· ·È‰È¿ ÂÏÏËÓÈ΋˜ ηٷ-

ÁˆÁ‹˜, ηıÒ˜ Î·È ÂΛӷ ·ÛÙÈÎÒÓ ÂÚÈÔ¯ÒÓ, ›¯·Ó

˘„ËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÂÌ‚ÔÏÈ·ÛÙÈ΋˜ Î¿Ï˘„˘, ÛÂ

Û‡ÁÎÚÈÛË Ì ٷ ·ÓÙ›ÛÙÔȯ· ÌÂÙ·Ó·ÛÙÒÓ ‹ ∂ÏÏËÓfi-

Ô˘ÏˆÓ Ô˘ ‰È·‚ÈÔ‡Ó ÛÙËÓ ÂÚÈʤÚÂÈ·.

∏ ·Ó›¯Ó¢ÛË ·ÓÙÈÛˆÌ¿ÙˆÓ ¤Ó·ÓÙÈ Ù˘ Ë·Ù›Ùȉ·˜

∞ Û ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿ <12 ÌËÓÒÓ ˘Ô‰ËÏÒ-

ÓÂÈ ·ÓÔÛ›· ÌÂÙ¿ ·fi Ê˘ÛÈ΋ Ïԛ̈ÍË. ™‡Ìʈӷ ÌÂ

Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜, 17% ÙˆÓ ÌË ÂÌ‚ÔÏÈ·Ṳ̂ӈÓ

·È‰ÈÒÓ Â›¯·Ó ÓÔÛ‹ÛÂÈ ·fi Ë·Ù›Ùȉ· ∞. ∆Ô ÔÛÔ-

ÛÙfi ·˘Ùfi Â›Ó·È ÂÍ·ÈÚÂÙÈο ·˘ÍË̤ÓÔ Î·È ‰ÂÓ ÌÔÚ›

Ó· ·Ô‰Ôı› ÛÙËÓ ÂÈÏÔÁ‹ ÙÔ˘ ‰Â›ÁÌ·ÙÔ˜.

™ËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÙfiÛÔ ÛÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ ο-

Ï˘„Ë fiÛÔ Î·È ÛÙË Ê˘ÛÈ΋ ·ÓÔÛ›· ‚Ú¤ıËΠÌÂٷ͇ ÙˆÓ

‰È·ÊfiÚˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ Ù˘ ¯ÒÚ·˜ (¶›Ó·-

η˜ 3). ™˘ÁÎÂÎÚÈ̤ӷ, ÛÙËÓ ∫ÂÓÙÚÈ΋ ∂ÏÏ¿‰· ηٷ-

ÁÚ¿ÊËÎÂ Ë ˘„ËÏfiÙÂÚË ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë Î·È Ù·

¯·ÌËÏfiÙÂÚ· ÔÛÔÛÙ¿ ÔÚÔıÂÙÈÎfiÙËÙ·˜ ÏfiÁˆ Ê˘ÛÈ΋˜ÓfiÛËÛ˘. √È ‰È·ÊÔÚ¤˜ ‹Ù·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈΤ˜ÌfiÓÔ ÁÈ· Ù· ∂ÏÏËÓfiÔ˘Ï·, ÏfiÁˆ Ù˘ ¯·ÌËÏ‹˜ ÂÌ‚Ô-ÏÈ·ÛÙÈ΋˜ Î¿Ï˘„˘ ÙˆÓ ·È‰ÈÒÓ ÌÂÙ·Ó·ÛÙÒÓ.

∞Í›˙ÂÈ ÂÈϤÔÓ Ó· ÙÔÓÈÛÙ› fiÙÈ, ÂÓÒ ÛÙÔ Û˘ÓÔÏÈ-Îfi ‰Â›ÁÌ· Ë ‰È·ÊÔÚ¿ ÔÚÔıÂÙÈÎfiÙËÙ·˜ ÌÂٷ͇ ∂ÏÏË-ÓfiÔ˘ÏˆÓ Î·È ·È‰ÈÒÓ ÌÂÙ·Ó·ÛÙÒÓ ‰ÂÓ ‹Ù·Ó ÛÙ·ÙÈ-ÛÙÈο ÛËÌ·ÓÙÈ΋, ÛÙ· ·È‰È¿ Ô˘ ‰È·‚ÈÔ‡Ó ÛÙËÓ ∫Â-ÓÙÚÈ΋ ∂ÏÏ¿‰· (fiÔ˘ ηٷÁÚ¿ÊËÎÂ Ë ˘„ËÏfiÙÂÚËÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë) Ë ‰È·ÊÔÚ¿ ‹Ù·Ó ÛËÌ·ÓÙÈ΋(¶›Ó·Î·˜ 3). ∏ ‰È·ÊÔÚ¿ ÛÙËÓ Â›ÙˆÛË Ù˘ Ê˘ÛÈ΋˜·ÓÔÛ›·˜ Ô˘ ηٷÁÚ¿ÊÂÙ·È ÌÂٷ͇ ÂÚÈÔ¯ÒÓ ·Ó¿ÏÔ-Á· Ì ÙËÓ ÂÌ‚ÔÏÈ·ÛÙÈ΋ Î¿Ï˘„Ë ÂÍËÁÂ›Ù·È Èı·ÓÒ˜·fi ÙÔ ÁÂÁÔÓfi˜ fiÙÈ Û ÂÚÈÔ¯¤˜ ¯·ÌËÏ‹˜ ÂÌ‚ÔÏÈ·-ÛÙÈ΋˜ Î¿Ï˘„˘ Ë ‰È·ÛÔÚ¿ ÙÔ˘ ÈÔ‡ Ù˘ Ë·Ù›Ùȉ·˜∞ Â›Ó·È ÌÂÁ·Ï‡ÙÂÚË Î·È ÌÔχÓÔÓÙ·È Â›ÓÔÛ· ·È‰È¿·ÓÂÍ·Úًو˜ ηٷÁˆÁ‹˜.

∆· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜ ¤Ú¯ÔÓÙ·È Û ·ÓÙ›ıÂÛË ÌÂÂΛӷ ÌÈ·˜ ÚfiÛÊ·Ù˘ ÔÚÔÂȉËÌÈÔÏÔÁÈ΋˜ ÌÂϤ-Ù˘ Ô˘ ·ÊÔÚÔ‡Û ÙËÓ Ë·Ù›Ùȉ· µ, ÁÈ· ÙËÓ ÔÔ›·ÂÊ·ÚÌfi˙ÂÙ·È Ì·˙ÈÎfi˜ ÂÌ‚ÔÏÈ·ÛÌfi˜ ÙˆÓ ‚ÚÂÊÒÓ ·fiÙÔ 1998 (15). ™ÙË Û˘ÁÎÂÎÚÈ̤ÓË ÌÂϤÙË, ‰ÂÓ ‚Ú¤ıËÎÂÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿ ÌÂٷ͇ ÙˆÓ ‰È¿ÊÔ-ÚˆÓ ÁˆÁÚ·ÊÈÎÒÓ ÂÚÈÔ¯ÒÓ, ÏfiÁˆ Ù˘ ÂÓÈ·›·˜ ÔÏÈ-ÙÈ΋˜ ˘Á›·˜ Ô˘ ÂÊ·ÚÌfi˙ÂÙ·È Û fiÏË ÙË ¯ÒÚ· (15).∂Ô̤ӈ˜, ·fi Ù· ·ÔÙÂϤÛÌ·Ù¿ Ì·˜, ÛÂ Û˘Ó‰˘·ÛÌfiÌ ÙËÓ ÚÔ·Ó·ÊÂÚfiÌÂÓË ·Ú·Ù‹ÚËÛË, ‰È·Ê·›ÓÂÙ·È Ë·Ó¿ÁÎË ÂÊ·ÚÌÔÁ‹˜ Ì·˙ÈÎÔ‡ ÂÌ‚ÔÏÈ·ÛÌÔ‡ ÙˆÓ ·È-‰ÈÒÓ Î·È ÁÈ· ÙËÓ Ë·Ù›Ùȉ· ∞, fiˆ˜ Û˘Ó¤‚Ë Î·È Û¿ÏϘ ¯ÒÚ˜, Î·È Ì¿ÏÈÛÙ· ·fi ÙËÓ ËÏÈΛ· ÙˆÓ 12 ÌË-ÓÒÓ (11-14). ª¿ÏÈÛÙ·, Ì ‚¿ÛË Ù· ·ÔÙÂϤÛÌ·Ù· Ù˘ÌÂϤÙ˘ ·˘Ù‹˜, Ë ∂ıÓÈ΋ ∂ÈÙÚÔ‹ ∂Ì‚ÔÏÈ·ÛÌÒÓ ÂÈ-ÛËÁ‹ıËΠÙËÓ ¤ÓÙ·ÍË ÙÔ˘ ÂÌ‚ÔÏ›Ô˘ Ù˘ Ë·Ù›Ùȉ·˜∞ ÛÙÔ˘˜ ˘Ô¯ÚˆÙÈÎÔ‡˜ ÂÌ‚ÔÏÈ·ÛÌÔ‡˜, Ì ·ÔÙ¤ÏÂ-ÛÌ· ÙÔ ÂÌ‚fiÏÈÔ Ó· ÂÚÈÏ·Ì‚¿ÓÂÙ·È ÛÙÔ Ó¤Ô ∂ıÓÈÎfi¶ÚfiÁÚ·ÌÌ· ∂Ì‚ÔÏÈ·ÛÌÒÓ ÙÔ˘ 2008.

µÈ‚ÏÈÔÁÚ·Ê›·

1. American Academy of Pediatrics. Hepatitis A. In:Pickering LK, ed. 2003 Red Book: Report of the Committeeon Infectious Diseases. 26th ed. Elk Grove Village, IL:American Academy of Pediatrics; 2003:309-318.

2. CDC: Prevention of Hepatitis A through Active or PassiveImmunization: Recommendations of the advisoryCommittee on Immunization Practices (ACIP). MMWR1999,48(No. RR-12).

3. Staes C, Schlenker T, Risk I, et al. Source of infectionamong persons with acute hepatitis A and no identificatiedrisk factors. Salt Lake County, Utah, 1996 [Abstract 302].Clin Infect Dis 1997;24:411.

4. Lionis C, Koulentaki M, Biziagos E, Kouroumalis E.Current prevalence of hepatitis A, B nad C in a well definedarea in rural Krete, Greece. J Viral Hepat 1997;4:55-61.

5. Kremastinou J, Kalapothaki V, Trichopoulos D. Thechanging epidemiologic pattern of hepatitis A infection inurban Greece. Am J Epidemiol 1984;120:703-706.

121√ÚÔÂȉËÌÈÔÏÔÁÈ΋ ÌÂϤÙË Ë·Ù›Ùȉ·˜ ∞

¶·È‰È·ÙÚÈ΋ 2009;72:121-000

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6. Papadopoulou V, Stavrati A, Patsialis T, et al. Changes inthe Epidemiology of acute hepatitis A during 1985-1992period. Pediatr N Gr 1995;7:30-34.

7. Dalekos GN, Zervou E, Karabini F, Tsianos EV. Prevalenceof viral markers among refugees from southern Albania:increased incidence of infection with hepatitis A, B and Dviruses. Eur J Gastrenterol Hepatol 1995;7:553-558.

8. Jacobsen KH, Koopman JS. Declining hepatitis Aseroprevalance: a global review and analysis EpidemiolInfect 2004;132:1005-1022.

9. World Health Organization. Hepatitis A. WHO/CDS//CSR/EDC/2000.7

10. Frosner GG, Papaevangelou G, Butler R, Iwarson S,Lindholm A, Couroucé-Pauty A, et al. Antibody againsthepatitis A in seven European countries I: comparison ofprevalence data in different age groups. Am J Epidemiol1979;110:63-69.

11. FDA Approval of VAQTA (hepatitis A vaccine, inactivated)in children > 1 year. MMWR 2005;54:1026.

12. Van Damme P, Van Herck K. Effect of Hepatitis AVaccination Programs. JAMA 2005;294:246-248.

13. Dagan R, Leventha A, Anis E, Slatter P, Ashur Y, ShouvalD. Incidence of Hepatitis A in Israel Following UniversalImmunization of Toddlers. JAMA 2005;294:202-210.

14. Wasley A, Samandari T, Bell BP. Incidence of Hepatitis Ain the United States in the era of vaccination. JAMA2005;294:194-201.

15. Papaevangelou V, Hadjichristodoulou C, Cassimos D,Pantelaki K, Tzivaras A, Hatzimichael et al. Seroepi-demiology of hepatitis B in Greek children six years afterthe implementation of universal vaccination. Infection2008;36:135-139

122 µ. ¶··Â˘·ÁÁ¤ÏÔ˘ Î·È Û˘Ó.

Paediatriki 2009;72:122-000

™ˆÛÙ¤˜ ··ÓÙ‹ÛÂȘ ÙÔ˘ Quiz ÁÈ· ÙÔ ¿ÚıÚÔ «¡ÂfiÙÂÚ· ‰Â‰Ô̤ӷ ÁÈ· ÙË Û˘ÁÁÂÓ‹

‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË»

1. ‚ 2. ·3. ‚4. ‰5. Á

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·122

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§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿: ÂÌÂÈÚ›· 28 ÂÙÒÓ

∞. ¶Ô˘ÚÙÛ›‰Ë˜1, ¢. ¢ÔÁ¿Ó˘1, ª. ª¿Î·1, ¢. ªÔ˘¯Ô‡ÙÛÔ˘1, ª. µ·Ú‚Ô˘ÙÛ‹1, ª. ™˘ÓÔ‰ÈÓÔ‡2,

∞ÈÎ. ªÈ¯·‹Ï-™ÙÚ¿ÓÙ˙È·3, ∂. ∫ÔÛÌ›‰Ë1

¶ÂÚ›ÏË„Ë

∂ÈÛ·ÁˆÁ‹: ∆Ô Ï¤Ìʈ̷ Hodgkin ·ÔÙÂÏ› ÂÚ›Ô˘ ÙÔ 4-5% ÙˆÓ Î·ÎÔËıÂÈÒÓ ÛÙ· ·È‰È¿. ∆· ÙÂÏÂ˘Ù·›·30 ¯ÚfiÓÈ·, Ë ¯Ú‹ÛË Û˘Ó‰˘·ÛÌÔ‡ ¯ËÌÂÈÔıÂڷ¢ÙÈÎÒÓ ÚˆÙÔÎfiÏψÓ, Ì ‹ ¯ˆÚ›˜ ·ÎÙÈÓÔıÂڷ›·, Ô‰‹-ÁËÛ Û ÛËÌ·ÓÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÚfiÁÓˆÛ˘ Î·È ¤Î‚·Û˘ ÙˆÓ ·È‰ÈÒÓ ·˘ÙÒÓ.

∞ÛıÂÓ›˜ Î·È Ì¤ıÔ‰ÔÈ: ∆· ÙÂÏÂ˘Ù·›· 28 ¯ÚfiÓÈ·, ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÛÙÔ ÙÌ‹Ì· Ì·˜ 73 ·È‰È¿ Ì ÓfiÛÔHodgkin. ™Ù· ·È‰È¿ ·˘Ù¿ ÌÂÏÂÙ‹ıËÎ·Ó ÂȉËÌÈÔÏÔÁÈο, ÎÏÈÓÈο Î·È ÈÛÙÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο, Ô ÙÚfi-Ô˜ ıÂڷ›·˜ Î·È Ë ¤Î‚·ÛË Î·È ·ÍÈÔÏÔÁ‹ıËÎ·Ó ‰˘ÓËÙÈÎÔ› ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ ÂȉÚÔ‡Ó ÛÙËÓÚfiÁÓˆÛË.

AÔÙÂϤÛÌ·Ù·: ∏ ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ (46 ·ÁfiÚÈ·, 27 ÎÔÚ›ÙÛÈ·) Î˘Ì·ÈÓfiÙ·Ó ·fi 3,5 ¤ˆ˜ 15 ¤ÙË. ™ÙË Û˘-ÓÙÚÈÙÈ΋ ÏÂÈÔ„ËÊ›·, Ë ·ÈÙ›· Ù˘ ·Ú¯È΋˜ ÚÔÛ¤Ï¢Û˘ ‹Ù·Ó Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· (81%), ÂÓÒ Ë ‰È¿Ú-ÎÂÈ· ÙˆÓ Û˘ÌÙˆÌ¿ÙˆÓ ÚÈÓ ÙË ‰È¿ÁÓˆÛË Î˘Ì·ÈÓfiÙ·Ó ·fi Ï›Á˜ ̤Ú˜ ¤ˆ˜ Î·È 12 Ì‹Ó˜. ªÂٷ͇ ÙˆÓÈÛÙÔÏÔÁÈÎÒÓ ˘fiÙ˘ˆÓ ˘ÂÚ›¯Â Ë Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË (63%), ÂÓÒ 44% ÙˆÓ ·ÛıÂÓÒÓ Â›¯·Ó ÚÔ¯ˆÚË-̤ÓË ÓfiÛÔ (ÛÙ·‰›Ô˘ πππ-IV). ∏ ıÂڷ›· ÂÚÈÂÏ¿Ì‚·Ó ÌfiÓÔ ¯ËÌÂÈÔıÂڷ›· Û 5 ·ÛıÂÓ›˜, ÌfiÓÔ ·ÎÙÈ-ÓÔıÂڷ›· Û 9 Î·È Û˘Ó‰˘·ÛÌfi Û 59. ∏ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË ·Ó¤Ú¯ÂÙ·È Û 97,3%. OÎÙÒ ·È‰È¿ ˘Ô-ÙÚÔ›·Û·Ó 20-65 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË Î·È ·fi ·˘Ù¿ ˙Ô˘Ó Ù· 7, ÂÓÒ ‰Â‡ÙÂÚË ‹ ÙÚ›ÙË Î·ÎÔ‹ıÂÈ· ‰È·-ÈÛÙÒıËΠ۠4 ·È‰È¿, ·fi Ù· ÔÔ›· ˙Ô˘Ó Ù· 3. ¢ÂÓ ‰È·ÈÛÙÒıËÎ·Ó ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ Ó·Û˘Û¯ÂÙ›˙ÔÓÙ·È Ì ÙËÓ ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ.

™˘ÌÂÚ¿ÛÌ·Ù·: ∏ ¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Î·È ÂÊ‹‚ˆÓ Ì ÓfiÛÔ Hodgkin Â›Ó·È ÂÍ·ÈÚÂÙÈ΋ ÌÂ Û˘Ó‰˘·ÛÌfi¯ËÌÂÈÔıÂڷ›·˜ Î·È ·ÎÙÈÓÔıÂڷ›·˜. ∏ Û˘Ó¯È˙fiÌÂÓË ·Ú·ÎÔÏÔ‡ıËÛË ÌÂÁ·Ï‡ÙÂÚÔ˘ ·ÚÈıÌÔ‡ ·È‰ÈÒÓÎÚ›ÓÂÙ·È ··Ú·›ÙËÙË ÁÈ· Ó· ‰È¢ÎÚÈÓÈÛÙ› ·Ó ˘¿Ú¯Ô˘Ó ‰˘ÓËÙÈο ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ù˘ ¤Î‚·ÛË˜ÙˆÓ ·È‰ÈÒÓ Ì ÓfiÛÔ Hodgkin.

§¤ÍÂȘ ÎÏÂȉȿ: §¤Ìʈ̷ ∏odgkin, ·È‰È΋ ËÏÈΛ·, ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜.

Hodgkin’s lymphoma in children: 28 years of experience

A. Pourtsidis1, D. Doganis1, M. Baka1, D. Bouhoutsou1, M. Varvoutsi1, M. Synodinou2,

A. Michael-Strantzia3, E. Kosmidi1

Abstract

Background: In the past 30 years the use of combined chemotherapy regimes, with or withoutradiotherapy, has improved significantly the prognosis for patients with Hodgkin’s lymphoma.

Methods: A retrospective study was made of 73 children with Hodgkin’s lymphoma treated at this centreover the past 28 years. The following data were analyzed and correlated with the outcome: age, sex, stage,histology, treatment, follow-up period.

Results: Among patients, there were 46 boys and 27 girls. The age at diagnosis varied from 3.3 to 15 years,and 15 patients were 8 years old or younger. Most of the patients (81%) presented withlymphadenopathy, with a time interval of 3 days to 12 months between the first appearance of clinicalsigns and the diagnosis. Nodular sclerosis was the predominant histological subtype (63%), and 44% ofpatients had advanced disease (stage III or IV). Chemotherapy alone was used in 5 patients, radiation in9 and combined treatment in 59. Relapses occurred in 8 children and 4 developed second or thirdmalignancies. The overall survival was 97.3%. One patient died from the disease and one after a thirdmalignancy. No specific factors related to the outcome were detected.

Conclusions: The outcome of children with Hodgkin’s disease is excellent with chemotherapy combinedwith low dose radiation. A longer follow-up of a greater number of patients is necessary to detectprognostic factors related to the outcome of children and adolescents with Hodgkin’s disease.

Key words: Hodgkin’s lymphoma, children, prognostic factors.

123∂ƒ∂À¡∏∆π∫∏ ∂ƒ°∞™π∞ ORIGINAL ARTICLE

1 √ÁÎÔÏÔÁÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

2 ∞ÎÙÈÓÔıÂڷ¢ÙÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

3 ¶·ıÔÏÔÁÔ·Ó·ÙÔÌÈÎfi∂ÚÁ·ÛÙ‹ÚÈÔ, ¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

AÏÏËÏÔÁÚ·Ê›·:

∞fiÛÙÔÏÔ˜ ¶Ô˘ÚÙÛ›‰Ë˜[email protected]√ÁÎÔÏÔÁÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

1 Department of Oncology,“P. & A. Kyriakou”Children’s Hospital,Athens, Greece

2 Department ofRadiotherapy, “P. & A. Kyriakou”Children’s Hospital,Athens, Greece

3 Laboratory of Pathology,“P. & A. Kyriakou”Children’s Hospital,Athens, Greece

Correspondence:

Apostolos [email protected] of Oncology, “P. & A. Kyriakou” Children’sHospital, Athens, Greece

¶·È‰È·ÙÚÈ΋ 2009;72:123-000

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∂ÈÛ·ÁˆÁ‹

∆Ô Ï¤Ìʈ̷ Hodgkin ·ÔÙÂÏ› ÙÔ 4-5% ÙˆÓ Î·-

ÎÔËıÂÈÒÓ ÛÙ· ·È‰È¿ Î·È ÙÔ ¤ÎÙÔ Î·Ù¿ ÛÂÈÚ¿ Û˘¯Ófi-

ÙËÙ·˜ ÓÂÔÏ·ÛÌ·ÙÈÎfi ÓfiÛËÌ· Ù˘ ·È‰È΋˜ ËÏÈΛ·˜

ÛÙËÓ ∂˘ÚÒË Î·È ÛÙȘ ∏.¶.∞., ÌÂÙ¿ ÙË Ï¢¯·ÈÌ›·,

ÙÔ˘˜ fiÁÎÔ˘˜ ÂÁÎÂÊ¿ÏÔ˘, ÙÔ Ó¢ÚÔ‚Ï¿Ûو̷, ÙÔ ÓÂ-

ÊÚÔ‚Ï¿Ûو̷ Î·È ÙÔ ÌË Hodgkin ϤÌʈ̷. ∏ ›-

ÙˆÛË Ù˘ ÓfiÛÔ˘ ‰È·Ê¤ÚÂÈ ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ·,

ÙÔ Ê‡ÏÔ, ÙËÓ ÂıÓÈÎfiÙËÙ· Î·È ÙÔÓ ÈÛÙÔÏÔÁÈÎfi Ù‡Ô.

™‡Ìʈӷ Ì ٷ ‰Â‰Ô̤ӷ Ù˘ International Agency

for Research on Cancer (πARC), Ë ÂÙ‹ÛÈ· ›وÛË

ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin Û ·È‰È¿ ËÏÈΛ·˜ οو

ÙˆÓ 15 ¯ÚfiÓˆÓ ˘ÔÏÔÁ›˙ÂÙ·È Û 5,5 ÂÚÈÙÒÛÂȘ ·Ó¿

ÂηÙÔÌ̇ÚÈÔ ÛÙÔÓ ·ÓÂÙ˘Á̤ÓÔ ÎfiÛÌÔ, ·ÏÏ¿ ÛÙÔ˘˜

ÂÊ‹‚Ô˘˜ Î·È Ó·ÚÔ‡˜ ÂÓ‹ÏÈΘ ËÏÈΛ·˜ 15-20 ¯Úfi-

ÓˆÓ Êı¿ÓÂÈ ÙȘ 12,1. ∞ÓÙÈı¤Ùˆ˜, ÛÙË ¢˘ÙÈ΋ ∞Û›· Ë

›وÛË ÛÙ· ·È‰È¿ Â›Ó·È ÛÙ·ıÂÚ¿ ¿Óˆ ·fi ÙȘ 7

ÂÚÈÙÒÛÂȘ ·Ó¿ ÂηÙÔÌ̇ÚÈÔ (1). ™ÙËÓ ∂ÏÏ¿‰·, Ë

›وÛË ÛÙ· ·È‰È¿ ·Ó¤Ú¯ÂÙ·È Û 7,2 ·Ó¿ ÂηÙÔÌ-

̇ÚÈÔ ¿ÓıÚˆÔ-¤ÙË. ÀÔÏÔÁ›ÛÙËΠÁÈ· ÚÒÙË ÊÔÚ¿

ÛÙË ¯ÒÚ· Ì·˜ Ì ‚¿ÛË Ù· ·Ó·Ï˘ÙÈο ‰Â‰Ô̤ӷ Ô˘

Û˘ÁÎÂÓÙÚÒÓÔÓÙ·È ÛÙÔ ∂ÏÏËÓÈÎfi ∞Ú¯Â›Ô ¶·È‰ÈÎÒÓ

∞ÈÌ·ÙÔÏÔÁÈÎÒÓ ∫·ÎÔËıÂÈÒÓ, Ô˘ ÏÂÈÙÔ˘ÚÁ› ÛÙÔ

∂ÚÁ·ÛÙ‹ÚÈÔ ÀÁÈÂÈÓ‹˜ Î·È ∂ȉËÌÈÔÏÔÁ›·˜ Î·È Û˘ÁÎÂ-

ÓÙÚÒÓÂÈ ÂıÓÈο ‰Â‰Ô̤ӷ (2). ∞·ÓÙ¿Ù·È Û˘¯ÓfiÙÂÚ·

ÛÙÔ˘˜ ∫·˘Î¿ÛÈÔ˘˜, ÂÓÒ ˘¿Ú¯Ô˘Ó ÙÚÂȘ ‰È·ÎÚÈÙÔ›

Ù‡ÔÈ fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ËÏÈΛ·: ·˘Ùfi˜ Ô˘ ·ÊÔÚ¿ ·È-

‰È¿ ÌÈÎÚfiÙÂÚ· ÙˆÓ 15 ÂÙÒÓ, ·˘Ùfi˜ ÙˆÓ Ó·ÚÒÓ ÂÓË-

Ï›ÎˆÓ Î·È ·˘Ùfi˜ ÙˆÓ ÌÂÁ·Ï‡ÙÂÚˆÓ ÂÓËÏ›ÎˆÓ (·ÛıÂ-

Ó›˜ ¿Óˆ ÙˆÓ 55 ÂÙÒÓ). ∏ ÓfiÛÔ˜ ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÈ· ‰È-

ÎfiÚ˘ÊË ËÏÈÎȷ΋ ηٷÓÔÌ‹, Ì ̛· ·È¯Ì‹ ÛÙËÓ ÂÊË-

‚›· Î·È Ì›· Û ·ÛıÂÓ›˜ ¿Óˆ ÙˆÓ 50 ÂÙÒÓ, ÂÓÒ Â›Ó·È

Û·ÓÈfiÙ·ÙË Û ·È‰È¿ ÌÈÎÚfiÙÂÚ· ÙˆÓ 5 ÂÙÒÓ (3).

∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙˆÓ ÙÂÏÂ˘Ù·›ˆÓ 30 ¯ÚfiÓˆÓ, Ë

¯Ú‹ÛË Û˘Ó‰˘·ÛÌ¤ÓˆÓ ¯ËÌÂÈÔıÂڷ¢ÙÈÎÒÓ Û¯ËÌ¿-

ÙˆÓ, Ì ‹ ¯ˆÚ›˜ ·ÎÙÈÓÔıÂڷ›·, Ô‰‹ÁËÛ Û ÛËÌ·-

ÓÙÈ΋ ‚ÂÏÙ›ˆÛË Ù˘ ÚfiÁÓˆÛ˘ ÙˆÓ ·È‰ÈÒÓ Ì ϤÌ-

ʈ̷ Hodgkin, ·‡ÍËÛË ÙˆÓ ÔÛÔÛÙÒÓ ›·Û˘ ηÈ

Û˘Á¯ÚfiÓˆ˜ Ì›ˆÛË Ù˘ ·ÒÙÂÚ˘ ÙÔÍÈÎfiÙËÙ·˜. H

ÂÓÙ·ÂÙ‹˜ ÂχıÂÚË Û˘Ì‚¿Ì·ÙÔ˜ ÂÈ‚›ˆÛË (EFS)

ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ· ˘ÂÚ‚·›ÓÂÈ ÙÔ 90%

ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ ·ÌËÏÔ‡ Î·È ÂӉȿÌÂÛÔ˘ ÎÈÓ‰‡ÓÔ˘

Ì ÂÓÙÔÈṲ̂ÓË ÓfiÛÔ (4) Î·È ÙÔ 60-70% ÁÈ· ·ÛıÂÓ›˜

˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Ì ÚÔ¯ˆÚË̤ÓË ÓfiÛÔ - Û˘Ó‹ıˆ˜

ÛÙ·‰›Ô˘ IV (5, 6).

∏ ·ÚÔ‡Û· ÌÂϤÙË Î·Ù·ÁÚ¿ÊÂÈ ÂȉËÌÈÔÏÔÁÈο,

ÂÚÁ·ÛÙËÚȷο, ÈÛÙÔÏÔÁÈο Î·È ·ÂÈÎÔÓÈÛÙÈο ¯·Ú·-

ÎÙËÚÈÛÙÈο, ηıÒ˜ ›Û˘ ÙÔ Â›‰Ô˜ ıÂڷ›·˜ Î·È ÙËÓ

¤Î‚·ÛË ÙˆÓ ·È‰ÈÒÓ Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÛÙÔ ÙÌ‹-

Ì· Ì·˜. ∆·˘Ùfi¯ÚÔÓ·, ¤¯ÂÈ ˆ˜ ÛÙfi¯Ô Ó· ÚÔÛ‰ÈÔÚ›ÛÂÈ

ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ Ô˘ ‰˘ÓËÙÈο Û¯ÂÙ›˙Ô-

ÓÙ·È Ì ÙËÓ ÙÂÏÈ΋ ¤Î‚·ÛË Î·È ÙȘ ·ÒÙÂÚ˜ Û˘Ó¤ÂÈ-

˜ Ù˘ ıÂڷ›·˜ ÙˆÓ ·È‰ÈÒÓ Ì ϤÌʈ̷ Hodgkin.

∞ÛıÂÓ›˜ Î·È Ì¤ıÔ‰ÔÈ

∏ ÌÂϤÙË Â›Ó·È ·Ó·‰ÚÔÌÈ΋ Î·È ÂÚÈÏ·Ì‚¿ÓÂÈ fiÏ· Ù· ·È-

‰È¿ Ì ϤÌʈ̷ Hodgkin Ô˘ ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÛÙÔ √ÁÎÔ-

ÏÔÁÈÎfi ÙÌ‹Ì· ÙÔ˘ ¡ÔÛÔÎÔÌ›Ԣ ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡»

·fi ÙÔ 1978 ¤ˆ˜ ÙÔ 2005. ∏ ÛÙ·‰ÈÔÔ›ËÛË ÙˆÓ ·ÛıÂÓÒÓ ¤ÁÈ-

Ó Ì ¯Ú‹ÛË ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ηٿ Ann Arbor (7), ÂÓÒ Ë ‰È¿-

ÎÚÈÛË ÙˆÓ ÈÛÙÔÏÔÁÈÎÒÓ ˘fiÙ˘ˆÓ ¤ÁÈÓ ۇÌʈӷ Ì ÙËÓ Ù·-

ÍÈÓfiÌËÛË Î·Ù¿ Rye (8).

T· Û¯‹Ì·Ù· ¯ËÌÂÈÔıÂڷ›·˜ Ô˘ ¯ÚËÛÈÌÔÔÈ‹ıËηÓ

ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ·ÛıÂÓÒÓ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·-

η 1. ∏ ıÂڷ¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË ‰È¤ÊÂÚ ·Ó¿ÏÔÁ· Ì ÙË ÚÔ-

ÓÈ΋ ÂÚ›Ô‰Ô. ¶ÂÚ›Ô‰Ô˜ ∞ (1979-1988, 19 ·ÛıÂÓ›˜): ·ÓÙÈÌÂ-

Ùˆ›ÛıËÎ·Ó Ì ·ÎÙÈÓÔıÂڷ›· ÌfiÓÔ, ¯ËÌÂÈÔıÂڷ›· ÌfiÓÔ

(MOPP ‹ ABVD) ‹ Û˘Ó‰˘·ÛÌfi ¯ËÌÂÈÔıÂڷ›·˜ (ªOPP ‹

ABVD) Î·È ·ÎÙÈÓÔıÂڷ›·˜ 30-40 Gy. ¶ÂÚ›Ô‰Ô˜ µ (1989-

1993, 26 ·ÛıÂÓ›˜): ÔÈ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ π ‹ ππ∞ ¤Ï·‚·Ó 4 ·-

ÎÏÔ˘˜ ∞µVD Î·È ·ÎÔÏÔ‡ıˆ˜ ·ÎÙÈÓÔıÂڷ›· Ì 20 Gy ÛÙȘ

·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜, ÂÓÒ ÔÈ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ

ππµ, III ‹ πV ¤Ï·‚·Ó ·fi 3 ·ÎÏÔ˘˜ MOPP Î·È ABVD ÂÓ·ÏÏ¿Í

Î·È ÛÙË Û˘Ó¤¯ÂÈ· ·ÎÙÈÓÔıÂڷ›· 20 Gy ÛÙȘ ·Ú¯Èο ÚÔÛ‚Â-

‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜, ÙÔ ÛÏ‹Ó· Î·È ÙÔ˘˜ ·Ú·ÔÚÙÈÎÔ‡˜ ÏÂÌ-

Ê·‰¤Ó˜ (9). ¶ÂÚ›Ô‰Ô˜ ° (1994-2005, 28 ·ÛıÂÓ›˜): Ù· ·È‰È¿

·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Û‡Ìʈӷ Ì ÙÔ ÚˆÙfiÎÔÏÏÔ Ù˘ °·ÏÏÈ-

΋˜ ∂Ù·ÈÚ›·˜ ¶·È‰È·ÙÚÈ΋˜ √ÁÎÔÏÔÁ›·˜, MDH 90 (10): ÁÈ·

ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ π ‹ ππ ¯ÔÚËÁ‹ıËÎ·Ó 4 ·ÎÏÔÈ VBVP

Î·È ·ÎÙÈÓÔ‚ÔÏ›· Ì 20 Gy ÛÙȘ ·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ Â-

ÚÈÔ¯¤˜. √È ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ πππ ¤Ï·‚·Ó ·fi 2 ·ÎÏÔ˘˜

MOPP Î·È ABVP ÂÓ·ÏÏ¿Í Î·È ·ÎÙÈÓÔıÂڷ›· Ì 20 Gy ÛÙȘ

·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ ÂÚÈÔ¯¤˜, ÙÔ ÛÏ‹Ó· Î·È ÙÔ˘˜ ·Ú·-

ÔÚÙÈÎÔ‡˜ ÏÂÌÊ·‰¤Ó˜. √È ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ IV ¤Ï·‚·Ó 2 ·-

ÎÏÔ˘˜ OPPA Î·È 4 ·ÎÏÔ˘˜ COPP Î·È ÛÙË Û˘Ó¤¯ÂÈ· ·ÎÙÈÓÔ-

ıÂڷ›· Ì 20 Gy ÛÙÔ˘˜ ·Ú¯Èο ÚÔۂ‚ÏË̤ÓÔ˘˜ ÏÂÌÊ·-

‰¤Ó˜ Î·È Ù· ·Ú¯Èο ÚÔۂ‚ÏË̤ӷ fiÚÁ·Ó·. ∏ ‰fiÛË Ù˘

·ÎÙÈÓÔıÂڷ›·˜ ‹Ù·Ó Û fiϘ ÙȘ ÂÚÈÙÒÛÂȘ 20 Gy, ÂÊfi-

ÛÔÓ Ë ·ÂÈÎÔÓÈÛÙÈ΋ ·¿ÓÙËÛË ˘ÂÚ¤‚·ÈÓ ÙÔ 70%. ∂ȉÈο

ÁÈ· ÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÛÙ¿‰ÈÔ IV, ÁÈ· ÙÔ ‹·Ú ¯ÔÚËÁ‹ıËÎ·Ó 12

¶›Ó·Î·˜ 1. £Âڷ›· ÙˆÓ ·ÛıÂÓÒÓ Ì ÓfiÛÔ Hodgkin

1978-1988 A£ ª√¡√ (9*)ã ª√¡√ (5) ª√PP:2*

ABVD:1∞§§√:2

X£ & ∞£ (5) ª√PP:2ABVD:3

1989-1993 ã & ∞£ (26) ª√PP & ABVD: 15ABVD: 10A§§√: 1

1994-2005 X£ & ∞£ (28) ª√PP & ABVP:8VBVP:11

√PPA & COPP: 8ABVP/COP: 1

* ·ÚÈıÌfi˜ ·ÛıÂÓÒÓMOPP (mustine, vincristine, procarbazine, prednisone) ABVD/P (doxorubicin, bleomycin, vinblastine, DTIC/prednisone)VBVP (vinblastine, bleomycin, etoposide, prednisone), OPPA (vincristine, prednisone, procarbazine, doxorubicin) COPP (cyclophosfamide, vincristine, procarbazine,prednisone)

124 ∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:124-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·124

Page 53: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

Gy, ÁÈ· Ù· ÔÛÙ¿ 20-30 Gy, ÂÓÒ ÁÈ· ÙÔ˘˜ Ó‡ÌÔÓ˜ ¯ÔÚËÁ‹ıË-

Î·Ó 12 Gy ÌfiÓÔ ·Ó ‰ÂÓ ˘‹Ú¯Â Ï‹Ú˘ ‡ÊÂÛË ÌÂÙ¿ ·fi ‰‡Ô

Û¯‹Ì·Ù· √PPA. ÃÚËÛÈÌÔÔÈ‹ıËÎÂ Ë Ì¤ıÔ‰Ô˜ Kaplan-Meier

ÁÈ· ÙÔÓ Î·ıÔÚÈÛÌfi Ù˘ Û˘ÓÔÏÈ΋˜ ÂÈ‚›ˆÛ˘ Î·È Ù˘ ¯ˆÚ›˜

ÓfiÛÔ ‹ ۇ̂·Ì· ÂÈ‚›ˆÛ˘. ø˜ ۇ̂·Ì· ıˆڋıËÎÂ Ô ı¿Ó·-

ÙÔ˜, Ë ˘ÔÙÚÔ‹ Î·È Ë ‰Â‡ÙÂÚË Î·ÎÔ‹ıÂÈ·. °È· ÙË ÛÙ·ÙÈÛÙÈ΋

·Ó¿Ï˘ÛË ÙˆÓ ‰Â‰ÔÌ¤ÓˆÓ ¯ÚËÛÈÌÔÔÈ‹ıËÎ·Ó ÔÈ ÂÍ‹˜ ÛÙ·ÙÈ-

ÛÙÈΤ˜ ‰ÔÎÈ̷ۛ˜: (1) ÌË ·Ú·ÌÂÙÚÈ΋ ‰ÔÎÈÌ·Û›· Mann-

Witney, (2) chi-square test Î·È (3) Fisher’s exact test.

∞ÔÙÂϤÛÌ·Ù·

™˘ÓÔÏÈο ·ÓÙÈÌÂÙˆ›ÛıËÎ·Ó ÛÙÔ ÙÌ‹Ì· Ì·˜ 73

·È‰È¿, 46 ·ÁfiÚÈ· Î·È 27 ÎÔÚ›ÙÛÈ· (·ÁfiÚÈ·/ÎÔÚ›ÙÛÈ·:

1,7/1). ∏ ËÏÈΛ· ÙˆÓ ·ÛıÂÓÒÓ Î˘Ì·ÈÓfiÙ·Ó ·fi 3,5-15

ÂÙÒÓ (‰È¿ÌÂÛË ÙÈÌ‹ 12 ¤ÙË) (Ù· ·ÁfiÚÈ· ·fi 3,5-15

ÂÙÒÓ Î·È Ù· ÎÔÚ›ÙÛÈ· ·fi 7,5-15 ÂÙÒÓ) (∂ÈÎfiÓ· 1). √

¯ÚfiÓÔ˜ ·fi ÙËÓ ÂÌÊ¿ÓÈÛË ÙÔ˘ ÚÒÙÔ˘ Û˘ÌÙÒÌ·ÙÔ˜

‹ ÎÏÈÓÈÎÔ‡ ÛËÌ›Ԣ ¤ˆ˜ ÙË ‰È¿ÁÓˆÛË Ù˘ ÓfiÛÔ˘ ΢-

Ì·ÈÓfiÙ·Ó ·fi 3 Ë̤Ú˜ ¤ˆ˜ 12 Ì‹Ó˜ (¢∆ 2 Ì‹Ó˜),

¯ˆÚ›˜ Ó· ‰È·ÈÛÙˆı› Û˘Û¯¤ÙÈÛË ·˘ÙÔ‡ Ì ÙËÓ ËÏÈΛ·

‹ ÙÔ Ê‡ÏÔ. ™ÙËÓ ÏÂÈÔ„ËÊ›·, Ë ·ÈÙ›· Ù˘ ·Ú¯È΋˜

‰ÈÂÚ‡ÓËÛ˘ ÙÔ˘ ·ÛıÂÓÔ‡˜ ‹Ù·Ó Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·

(59 ·È‰È¿, 81%), ÂÓÒ 4 ·È‰È¿ ‰ÈÂÁÓÒÛıËÛ·Ó ÛÙ·

Ï·›ÛÈ· ‰ÈÂÚ‡ÓËÛ˘ ÂÌ˘Ú¤ÙÔ˘, 2 ÏfiÁˆ ¯ÚfiÓÈÔ˘

‚‹¯·, 3 ÏfiÁˆ Ù˘¯·›Ô˘ ·ÂÈÎÔÓÈÛÙÈÎÔ‡ ÂϤÁ¯Ô˘ Î·È 5

ÏfiÁˆ ¿ÏÏ˘ Û˘Ìو̷ÙÔÏÔÁ›·˜ ‹ ÛËÌÂÈÔÏÔÁ›·˜ (ÛÂ

‰‡Ô ·È‰È¿ Ë ‰È¿ÁÓˆÛË ÂÙ¤ıË ÛÙ· Ï·›ÛÈ· ‰ÈÂÚ‡ÓË-

Û˘ ÓÂÊÚˆÛÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘, Ô˘ ·ÔÙÂÏ› ·Ú·ÓÂ-

ÔÏ·ÛÌ·ÙÈ΋ ÂΉ‹ÏˆÛË Ù˘ ÓfiÛÔ˘ Hodgkin). ™Â 50

·È‰È¿ (68,5%) ‰ÂÓ ‰È·ÈÛÙÒıËÎ·Ó Û˘ÓÔ‰¿ Û˘ÌÙÒ-

Ì·Ù·, ÂÓÒ ÛÙ· ˘fiÏÔÈ·, ÂÎÙfi˜ ÙÔ˘ ·ÚÈÔ˘ Û˘ÌÙÒ-

Ì·ÙÔ˜/ÛËÌ›Ԣ, Û˘Ó˘‹Ú¯·Ó ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤-

ÚÂÈ·, ‚‹¯·˜-Ï¢ÚÔ‰˘Ó›·, Â̇ÚÂÙÔ, ÏÂÌÊ·‰ÂÓÔ¿-

ıÂÈ· ‹ Û˘Ó‰˘·ÛÌfi˜ ÙˆÓ ·ÓˆÙ¤Úˆ. ™˘ÌÙÒÌ·Ù· Ù‡-

Ô˘ µ ‰È·ÈÛÙÒıËÎ·Ó Û 21 ·È‰È¿ (28,8%) Î·È ÂȉÈ-

ÎfiÙÂÚ· ·Ú·ÙÂÈÓfiÌÂÓÔ ˘Ú¤ÙÈÔ (¿Óˆ ÙˆÓ 10 ËÌÂ-

ÚÒÓ) ‰È·ÈÛÙÒıËΠ۠10, ·ÒÏÂÈ· ‚¿ÚÔ˘˜ >10% ÛÂ

10, ÂÊȉÚÒÛÂȘ Û 7 Î·È ÎÓËÛÌfi˜ Û ¿ÏÏ· 2. §ÂÌÊ·-

‰ÂÓÔ¿ıÂÈ· ηٿ ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË ‰È·ÈÛÙÒıËÎÂ

Û 68 ·ÛıÂÓ›˜ (93%). ™Â 12 ÂÍ ·˘ÙÒÓ ‹Ù·Ó ·ÌÊÔÙÂ-

ÚfiÏ¢ÚË Î·È Û 56 ÂÙÂÚfiÏ¢ÚË. ∂ÓÙfiÈÛË ÛÙÔÓ

ÙÚ¿¯ËÏÔ ‰È·ÈÛÙÒıËΠ۠55 ·È‰È¿, ÛÙÔ˘˜ ˘ÂÚ-

ÎÏ›‰ÈÔ˘˜ ‚fiıÚÔ˘˜ Û 41, ÛÙȘ Ì·Û¯·ÏÈ·›Â˜ ÎÔÈÏfiÙË-

Ù˜ Û 13 Î·È ÛÙË ‚Ô˘‚ˆÓÈ΋ ¯ÒÚ· Û 4. ∫·Ù¿ ÙË Ê˘-

ÛÈ΋ ÂͤٷÛË, Ë·ÙÔÌÂÁ·Ï›· ‰È·ÈÛÙÒıËΠ۠2 ·È-

‰È¿ Î·È ÛÏËÓÔÌÂÁ·Ï›· Û 5. ∆· ÂÚÁ·ÛÙËÚȷο ¢ڋ-

Ì·Ù· ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ ηٿ ÙË ‰È¿ÁÓˆÛË ÂÚÈÁÚ¿-

ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 2.

∞ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜

ŸÏÔÈ ÔÈ ·ÛıÂÓ›˜ ˘Ô‚Ï‹ıËÎ·Ó Û ·ÂÈÎÔÓÈÛÙÈΤ˜

ÂÍÂÙ¿ÛÂȘ ÁÈ· ÛÙ·‰ÈÔÔ›ËÛË Ù˘ ÓfiÛÔ˘. ∆Ô ˘ÂÚ˯Ô-

ÁÚ¿ÊËÌ· ÙÚ·¯‹ÏÔ˘ ‹Ù·Ó ıÂÙÈÎfi ÁÈ· Â˘Ú‹Ì·Ù· Û ·Ó·-

ÏÔÁ›· 86%, Ë ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ Û 43% Î·È Ë

˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ Û ·Ó·ÏÔÁ›·

67%. MÂÁ¿ÏË ‹ ̤ÙÚÈ· ‰È‡ڢÓÛË ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘

¶›Ó·Î·˜ 2. ∂ÚÁ·ÛÙËÚȷΤ˜ ·Ú¿ÌÂÙÚÔÈ ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÌÂϤÙ˘ ηٿ ÙË ‰È¿ÁÓˆÛË

™‡ÓÔÏÔ º‡ÏÔ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·

¢∆* ŸÚÈ· ¢∆* ¢∆* ƒ

∏ÏÈΛ· (¤ÙË) 12 3,5-12 10,3 13 0,001WBC (x 103/mm3) 10,2 4,2-25 9,3 11,25 0,183¶ÔÏ˘ (%) 66 39-91 64,3 74 0,044∏ˆÛ (%) 3 0-11 3 2,5 0,768Hb (g/dl) 11,1 7,6-15 11,1 11,1 0,35∆∫∂ (mm 1˘ ÒÚ·˜) 61 5-150 52 82 0,123PLT (x 103/mm3) 383 200-820 367 387 0,614CRP (mg/l) 72 0-173 51,8 81 0,577LDH (mg/dl) 380 206-1300 381 335 0,672

*¢∆: ‰È¿ÌÂÛË ÙÈÌ‹

125§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿

¶·È‰È·ÙÚÈ΋ 2009;72:125-000

151413121110987654

%

20

10

0

∫ÔÚ›ÙÛÈ·

∞ÁfiÚÈ·

3

7

6

5

3

2

1 23

10

43

8

21

33

6

1

∏ÏÈΛ· (¤ÙË)

∂ÈÎfiÓ· 1. ¢ËÌÔÁÚ·ÊÈο ¯·Ú·ÎÙËÚÈÛÙÈο.

¶›

19

19

19

* ·MABVBOPCO

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·125

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‰È·ÈÛÙÒıËΠÛÂ Û˘¯ÓfiÙËÙ· 37% (7/19) Î·È 78%

(36/46) ÛÙÔ˘˜ ·ÛıÂÓ›˜ Ì ÈÛÙÔÏÔÁÈÎfi ˘fiÙ˘Ô ÌÈ-

ÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ Î·È Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘

·ÓÙ›ÛÙÔȯ·. ™Â ÂÚ›ÙˆÛË ‰È‡ڢÓÛ˘ ÌÂÛÔıˆÚ·Î›-

Ô˘ ηٷ‰ÂÈÎÓ˘fiÌÂÓ˘ Ì ˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›·

(100%), Ë ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· ·ÔÎ¿Ï˘Ù ÙË ‰È‡-

Ú˘ÓÛË ÛÙÔ 86%. ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ÎÔÈÏ›·˜ Î·È Ë

˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›· ÎÔÈÏ›·˜ ·ÔÎ¿Ï˘„·Ó ·-

ıÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÛÙÔ 49% Î·È 40%, ·ÓÙ›ÛÙÔȯ·.

∏·ÙÔÌÂÁ·Ï›· ‰È·ÈÛÙÒıËΠ۠7 ·È‰È¿, ÛÏËÓÔ-

ÌÂÁ·Ï›· Û 13, Ô˙Ò‰ÂȘ ‰ÈËı‹ÛÂȘ ¯·Ú·ÎÙËÚÈÛÙÈΤ˜

ÏÂÌÊÒÌ·ÙÔ˜ ∏odgkin ‰È·ÈÛÙÒıËÎ·Ó ÛÙÔ ‹·Ú Û 3

·È‰È¿, ÛÙÔ ÛÏ‹Ó· Û 14 ·È‰È¿, ÂÓÒ ·ÓÔÌÔÈÔÁ¤ÓÂÈ·

ÓÂÊÚÒÓ ‰È·ÈÛÙÒıËΠ۠¤Ó· ·È‰›. ™Â 12 ·È‰È¿

‰È·ÈÛÙÒıËÎÂ Û˘Ó‰˘·ÛÌfi˜ 2 ‹ 3 ÂÎ ÙˆÓ ·ÓˆÙ¤Úˆ

¢ÚËÌ¿ÙˆÓ, Û˘ÌÂÚÈÏ·Ì‚·ÓÔ̤Ó˘ Î·È Ù˘ ÏÂÌÊ·-

‰ÂÓÔ¿ıÂÈ·˜. ∆¤ÏÔ˜, ÏÂÌÊ·ÁÁÂÈÔÁÚ·Ê›· (ÎÏ·ÛÈ΋ ‹

Ú·‰ÈÔ˚ÛÔÙÔÈ΋) Ú·ÁÌ·ÙÔÔÈ‹ıËΠ۠17 ·È‰È¿

Î·È ‹Ù·Ó ·ıÔÏÔÁÈ΋ Û 6.

™Ù·‰ÈÔÔ›ËÛË

ªÂ ‚¿ÛË ÎÏÈÓÈο Î·È ·ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù·, 16

(22%) ·È‰È¿ Ù·ÍÈÓÔÌ‹ıËÎ·Ó ˜ ÛÙ·‰›Ô˘ π, 25 (34%)

ˆ˜ ÛÙ·‰›Ô˘ ππ (II∞: 11, IIB: 14), 20 (27,5%) ˆ˜ ÛÙ·‰›-

Ô˘ πππ (III∞: 6, IIIB: 14) Î·È 12 (16,5%) ˆ˜ ÛÙ·‰›Ô˘ IV

(IV∞: 5, IVB: 7).

™Â ¯ÂÈÚÔ˘ÚÁÈ΋ ÛÙ·‰ÈÔÔ›ËÛË Ì ÛÏËÓÂÎÙÔÌ‹

˘Ô‚Ï‹ıËÎ·Ó 3 ·È‰È¿ ÛÙ· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ÌÂϤ-

Ù˘. ¢È‹ıËÛË ÙÔ˘ Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ ‰È·ÈÛÙÒıËÎÂ

Ì ÔÛÙÂÔÌ˘ÂÏÈ΋ ‚ÈÔ„›· ÛÙÔ 5% ÙˆÓ ·È‰ÈÒÓ. ™Â

‚ÈÔ„›· ‹·ÙÔ˜ ˘Ô‚Ï‹ıËÎ·Ó 5 ·È‰È¿ Î·È Û fiÏ·

‹Ù·Ó ·ÚÓËÙÈ΋. ™˘ÌÌÂÙÔ¯‹ ·fi Ù· ÔÛÙ¿ ‰È·ÈÛÙÒ-

ıËΠ̠ÛÈÓıËÚÔÁÚ¿ÊËÌ· ÔÛÙÒÓ Û 4/73 ·È‰È¿

Î·È ·ÂÈÎÔÓÈÛÙÈο ÔÚ·Ù¤˜ Ó¢ÌÔÓÈΤ˜ ‰ÈËı‹ÛÂȘ

ÛÂ 3/73.

πÛÙÔÏÔÁÈÎfi˜ Ù‡Ô˜

™ÙËÓ ÏÂÈÔ„ËÊ›· ÙˆÓ ·È‰ÈÒÓ (46/73, 63%) ‰È·-

ÈÛÙÒıËÎÂ Ô ÈÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜ Ù˘ Ô˙Ò‰Ô˘˜

ÛÎÏ‹Ú˘ÓÛ˘. √ ˘fiÙ˘Ô˜ Ù˘ ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›-

ıÂÈ·˜ ‰È·ÈÛÙÒıËÎÂ Û˘¯ÓfiÙÂÚ· Û ·ÛıÂÓ›˜ ÛÙ·‰›-

Ô˘ π & ππI, Ë Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË Û ·ÛıÂÓ›˜ ÛÙ·‰›-

Ô˘ ππ, Ô ÏÂÌÊÔÂÈÎÚ·ÙÒÓ Û ·ÛıÂÓ›˜ ÛÙ·‰›Ô˘ π, ÂÓÒ

Ù· ‰‡Ô ·È‰È¿ Ì ÏÂÌÊÔÂÓÈÎfi ˘fiÙ˘Ô ‹Ù·Ó ÛÙ·-

‰›Ô˘ πV (¶›Ó·Î·˜ 3). ∏ Û˘Û¯¤ÙÈÛË ÙÔ˘ ÈÛÙÔÏÔÁÈÎÔ‡

˘ÔÙ‡Ô˘ Î·È ÙÔ˘ ÛÙ·‰›Ô˘ Ù˘ ÓfiÛÔ˘ Ì ÂȉËÌÈÔ-

ÏÔÁÈο Î·È ÂÚÁ·ÛÙËÚȷο ‰Â‰Ô̤ӷ ÂÚÈÁÚ¿ÊÂÙ·È

ÛÙÔÓ ¶›Ó·Î· 4.

¶›Ó·Î·˜ 4. ™˘Û¯¤ÙÈÛË ÂȉËÌÈÔÏÔÁÈÎÒÓ Î·È ÂÚÁ·ÛÙËÚÈ·ÎÒÓ ·Ú·Ì¤ÙÚˆÓ Ì ÙÔ ÛÙ¿‰ÈÔ Î·È ÙËÓ ÈÛÙÔÏÔÁ›·

™Ù¿‰ÈÔ πÛÙÔÏÔÁ›·***π ππ πππ πV P ª §¶ §∂ √™ ƒ

º‡ÏÔ (·Á:ÎÔ)* 13:3 13:12 12:8 8:4 0,393 18:1 2:0 6:0 20:26 0,000∏ÏÈΛ· (¤ÙË)** 9,3 12,1 12,1 13 0,102 10 11,6 10,5 12,7 0,017WBC (x 103/mm3)** 8,75 12,19 9 10,4 0,027 8,4 4,7 8,5 11,4 0,007¶ÔÏ˘ (%)** 55 74 62 77 0,002 63 60,5 57 70 ns#

∏ˆÛ (%)** 3 2 3 2,4 0,173 2,8 2,5 3 3 nsHb (g/dl)** 12,9 11,1 11,1 10,9 0,014 11,1 10,6 13,8 11,1 nsPLT (x 103/mm3)** 200 400 403 383 0,022 367 384 301 393 ns∆∫∂ (mm 1˘ ÒÚ·˜)** 16,5 70 75 115 0,001 59 65 16 70 nsCRP (mg/l)** 20 57 80 110 0,398 57 - 27 74 nsLDH (mg/dl)** 498 330 380 404 0,44 323 - 380 383 ns

* ·Á: ·ÁfiÚÈ·, ÎÔ: ÎÔÚ›ÙÛÈ·** ‰È¿ÌÂÛ˜ ÙÈ̤˜

*** ª: ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜, §¶: ÏÂÌÊÔÂÓÈÎfi˜, §∂: ÏÂÌÊÔÂÈÎÚ·ÙÒÓ, √™: Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘# ÌË ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈ΋ ‰È·ÊÔÚ¿, ¶√§À: ÔÏ˘ÌÔÚÊÔ‡ÚËÓ·, ∏ø™: ˈÛÈÓfiÊÈÏ·

126 ∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:126-000

¶›Ó·Î·˜ 3. πÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜ Î·È ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘

™‡ÓÔÏÔ ™Ù¿‰ÈÔπ ππ πππ IV

ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ 19* (26)** 6 (37,5) 4 (16) 7 (35) 2 (16,7)§ÂÌÊÔÂÓÈÎfi˜ 2 (2,7) - - - 2 (16,7)§ÂÌÊÔÂÈÎÚ·ÙÒÓ 6 (8,2) 4 (25) 1 (4) 1 (5) -√˙҉˘ ÛÎÏ‹Ú˘ÓÛË 46 (63) 6 (37,5) 20 (80) 12 (60) 8 (66,6)™‡ÓÔÏÔ 73 (100) 16 (22) 25 (34) 20 (27,5) 12 (16,5)

*·ÚÈıÌfi˜ ·ÛıÂÓÒÓ, **(%) ·Ó·ÏÔÁ›· ·ÛıÂÓÒÓ

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·126

Page 55: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

£Âڷ›·

∏ ıÂڷ›· ÎÚ›ıËΠˆ˜ ·Ú΋˜ Û 68 ·ÛıÂÓ›˜

Î·È ·Ó·Ú΋˜ Û 5. ™Â 68 ·È‰È¿ Ô˘ ¤Ï·‚·Ó ·ÎÙÈ-

ÓÔıÂڷ›·, ·˘Ù‹ ‹Ù·Ó ·Ú΋˜ Û 66. ™Â fiÏÔ˘˜

ÙÔ˘˜ ·ÛıÂÓ›˜ Ô˘ ¤Ï·‚·Ó ∞£, ·˘Ù‹ ‹Ù·Ó ÂÛÙȷ΋,

ÂÓÒ ÔÈ 56 ÂÍ ·˘ÙÒÓ ¤Ï·‚·Ó 20 Gy, ÔÈ 4 30 Gy Î·È ÔÈ 8

40 Gy ÛÙ· ÚÒÙ· ¯ÚfiÓÈ· Ù˘ ÌÂϤÙ˘.

ŒÎ‚·ÛË

™ÙÔ˘˜ 73 ·ÛıÂÓ›˜ Ù˘ ÌÂϤÙ˘ ÛËÌÂÈÒıËÎ·Ó 8

˘ÔÙÚÔ¤˜ (11%), 20-65 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË (¢∆

30). ∆· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ·ÚÔ˘Û›·-

Û·Ó ˘ÔÙÚÔ‹ ÂÚÈÁÚ¿ÊÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 5. ∏ ·Ú-

¯È΋ ·ÓÙÈÌÂÙÒÈÛË Ù˘ ˘ÔÙÚÔ‹˜ ÂÚÈÂÏ¿Ì‚·ÓÂ

ÌfiÓÔ Ã£ ‰Â‡ÙÂÚ˘ ÁÚ·ÌÌ‹˜ Û 3 ÂÚÈÙÒÛÂȘ ηÈ

Û˘Ó‰˘·ÛÌfi ã & ∞£ Û 5. ™Â 6/8 ·È‰È¿ ¤ÁÈÓ ηÈ

·˘ÙfiÏÔÁË ÌÂÙ·ÌfiÛ¯Â˘ÛË Ì˘ÂÏÔ‡ ÙˆÓ ÔÛÙÒÓ. ∞fi Ù·

8 ·È‰È¿ Ô˘ ·ÚÔ˘Û›·Û·Ó ˘ÔÙÚÔ‹ ˙Ô˘Ó Ù· 7, 12-

270 Ì‹Ó˜ (¢∆ 95) ·fi ÙË ‰È¿ÁÓˆÛË Ù˘ ˘ÔÙÚÔ‹˜

Î·È 32-310 Ì‹Ó˜ (¢∆ 125) ·fi ÙË ‰È¿ÁÓˆÛË Ù˘ Ófi-

ÛÔ˘ (ÂÈ‚›ˆÛË Û 2Ë ‡ÊÂÛË: 89%). ¢Â‡ÙÂÚË ‹ ÙÚ›ÙË

ηÎÔ‹ıÂÈ· ‰È·ÈÛÙÒıËΠ۠4 ·È‰È¿, ·fi Ù· ÔÔ›·

˙Ô˘Ó Ù· 3. ∆· ¯·Ú·ÎÙËÚÈÛÙÈο ÙˆÓ ·ÛıÂÓÒÓ Ô˘ ÂÌ-

Ê¿ÓÈÛ·Ó ‰Â‡ÙÂÚË ‹ ÙÚ›ÙË Î·ÎÔ‹ıÂÈ· ÂÚÈÁÚ¿ÊÔÓÙ·È

ÛÙÔÓ ¶›Ó·Î· 6. ™˘ÓÔÏÈο ˙Ô˘Ó 71/73 ·ÛıÂÓ›˜ (Û˘ÓÔ-

ÏÈ΋ ÂÈ‚›ˆÛË: 97,3%), 24-366 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆ-

ÛË (¢∆ 156 Ì‹Ó˜) (ÂÈ‚›ˆÛË ÂχıÂÚË Û˘Ì‚¿Ì·ÙÔ˜:

83,6% - 61/73) (∂ÈÎfiÓ˜ 2, 3). ∆· 64 ·fi Ù· 73 ·È‰È¿

(87,7%) ˙Ô˘Ó Û ϋÚË ÚÒÙË ‡ÊÂÛË.

¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜

∏ Û˘Û¯¤ÙÈÛË ÂȉËÌÈÔÏÔÁÈÎÒÓ, ÎÏÈÓÈÎÒÓ, ·ÂÈ-

ÎÔÓÈÛÙÈÎÒÓ, ÂÚÁ·ÛÙËÚÈ·ÎÒÓ Î·È ıÂڷ¢ÙÈÎÒÓ ·-

ڷ̤ÙÚˆÓ Ì ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘ ÂÚÈÁÚ¿ÊÂÙ·È

ÛÙÔÓ ¶›Ó·Î· 7.

™˘˙‹ÙËÛË

∆Ô Ï¤Ìʈ̷ ‹ ÓfiÛÔ˜ ÙÔ˘ Hodgkin ÂÚÈÁÚ¿ÊËÎÂ

¶›Ó·Î·˜ 6. ÷ڷÎÙËÚÈÛÙÈο ·ÛıÂÓÒÓ Ì ‰Â‡ÙÂÚË ‹ ÙÚ›ÙË Î·ÎÔ‹ıÂÈ·

Ã√ ºÀ ∏§π ™∆ π™∆ £Âڷ›· ∂›‰Ô˜ ∂›‰Ô˜ ∞£ ŒÎ‚·ÛË ÃÚfiÓԘ·Ú΋˜ ıÂڷ›·˜ ã ∂·Ú΋˜ ¢fiÛË ∂ÓÙfiÈÛË

1 204* ∞° 13** π §∂ ¡∞π ∞£ ¡∞π 40Gy ∂ÛÙȷ΋ ¢ÂÓ ˙ÂÈ2 144 ∞° 4,9 π √™ ¡∞π ã & ∞£ ∞µVD ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 178*3 48 ∫√ 12,2 ππ √™ ¡∞π ã & ∞£ VBVP + OPPA ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 614 71 ∫√ 11,2 ππ √™ ¡∞π ã & ∞£ ABVD + MOPP ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 175

Ã√: ¯ÚfiÓÔ˜ ·fi ÙË ‰È¿ÁÓˆÛË, ºÀ: ʇÏÔ, ∞°: ·ÁfiÚÈ·, ∫√: ÎÔÚ›ÙÛÈ·, ∏§π: ËÏÈΛ·, ™∆: ÛÙ¿‰ÈÔ, I™∆: ÈÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜, §∂:ÏÂÌÊÔÂÈÎÚ·ÙÒÓ, √™: Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË*Ì‹Ó˜ ·fi ‰È¿ÁÓˆÛË, **¤ÙË1. ∞‰ÂÓÔηÚΛӈ̷ ÛÙÔÌ¿¯Ô˘ (2Ë) Î·È Î·ÚΛÓÔ˜ Ì·ÛÙÔ‡ (3Ë) 228 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË, ÏfiÁˆ ÙÔ˘ ÔÔ›Ô˘ η٤ÏËÍÂ2. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜3. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜4. ∫·ÚΛÓÔ˜ ı˘ÚÂÔÂȉԇ˜ (2Ë) Î·È ÔÛÙÂÔÛ¿ÚΈ̷ (3Ë) 94 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆÛË

127§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿

¶·È‰È·ÙÚÈ΋ 2009;72:127-000

¶›Ó·Î·˜ 5. ÷ڷÎÙËÚÈÛÙÈο ·ÛıÂÓÒÓ Ì ˘ÔÙÚÔ‹

Ã√ ºÀ ∏§π ™∆ π™∆ £Âڷ›· ∂›‰Ô˜ ∂›‰Ô˜ ∞£ ŒÎ‚·ÛË ÃÚfiÓԘ·Ú΋˜ ıÂڷ›·˜ ã ∂·Ú΋˜ ¢fiÛË ∂ÓÙfiÈÛË

1 20* ∫√ 13.5** πV √™ ¡∞π ã & ∞£ OPPA + ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 32*COPP

2 30 ∞° 4.5 π ª∫ ¡∞π ∞£ ¡∞π 40 Gy ∂ÛÙȷ΋ ∑ÂÈ 2533 24 A° 8 ππ ª∫ ¡∞π ã & ∞£ VBVP ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 904 40 ∫√ 14.2 πππ √™ √Ãπ ã §√π¶∞ ¢ÂÓ ˙ÂÈ5 40 ∫√ 14 πππ √™ √Ãπ ã MOPP ∑ÂÈ 3106 30 ∞° 13.2 πππ √™ ¡∞π ã & ∞£ ABVD + ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 125

MOPP7 25 ∞° 6 πV √™ √Ãπ ã & ∞£ ABVD + ¡∞π 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 223

MOPP8 65 ∞° 14 πV √™ √Ãπ ã & ∞£ OPPA + √Ãπ 20 Gy ∂ÛÙȷ΋ ∑ÂÈ 78

COPP

Ã√: ¯ÚfiÓÔ˜ ·fi ÙË ‰È¿ÁÓˆÛË, ºÀ: ʇÏÔ, ∞°: ·ÁfiÚÈ·, ∫√: ÎÔÚ›ÙÛÈ·, ∏§π: ËÏÈΛ· ™∆: ÛÙ¿‰ÈÔ. I™∆: ÈÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜, √™: Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË, ª∫: ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜, §∂: ÏÂÌÊÔÂÈÎÚ·ÙÒÓ*Ì‹Ó˜ ·fi ‰È¿ÁÓˆÛË, **¤ÙË

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·127

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ÁÈ· ÚÒÙË ÊÔÚ¿ ·fi ÙÔÓ Thomas Hodgkin ÙÔ 1932.

∏ ·ÈÙÈÔÏÔÁ›· Ù˘ ÓfiÛÔ˘ ·Ú·Ì¤ÓÂÈ ·‰È¢ÎÚ›ÓÈÛÙË.

∞ÔÙ¤ÏÂÛ ÁÈ· ÔÏϤ˜ ‰ÂηÂٛ˜ ·ÓÙÈΛÌÂÓÔ ÂÈ-

‰ËÌÈÔÏÔÁÈÎÒÓ ÂÚ¢ÓÒÓ, Ù· Â˘Ú‹Ì·Ù· ÙˆÓ ÔÔ›ˆÓ

Ê·›ÓÂÙ·È Ó· Ô‰ÂÈÎÓ‡Ô˘Ó ‰È·ÊÔÚÂÙÈÎÔ‡˜ ·ÈÙÈÔÏÔÁÈ-

ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ·Ó¿ÏÔÁ· Ì ÙËÓ ËÏÈΛ·. ™‡Ìʈӷ

Ì ÙÔ ™‡ÛÙËÌ· ∆·ÍÈÓfiÌËÛ˘ ÙÔ˘ ¶·ÁÎfiÛÌÈÔ˘ √ÚÁ·-

ÓÈÛÌÔ‡ ÀÁ›·˜ (¶√À), ηıÔÚ›˙ÔÓÙ·È ‰‡Ô ·ÚÈÔÈ Ù‡-

ÔÈ ÏÂÌÊˆÌ¿ÙˆÓ Hodgkin, ηı¤Ó·˜ ·fi ÙÔ˘˜ ÔÔ›-

Ô˘˜ ¤¯ÂÈ ÂȉÈο ÎÏÈÓÈο Î·È ÚÔÁÓˆÛÙÈο ¯·Ú·ÎÙËÚÈ-

ÛÙÈο: Ë ÎÏ·ÛÈ΋ ÓfiÛÔ˜ (Classical Hodgkin’s

lymphoma, CHL) Î·È Ô Ù‡Ô˜ Ô˙Ò‰Ô˘˜ ÏÂÌÊÔ΢Ù-

Ù·ÚÈ΋˜ ÂÈÎÚ¿ÙËÛ˘ (Nodular lymphocyte

predominance Hodgkin’s lymphoma, NLPHL) (11).

√È ÎÏ·ÛÈÎÔ› ˘fiÙ˘ÔÈ Ù˘ ÓfiÛÔ˘ Û˘Ó‰¤ÔÓÙ·È Û˘Ó‹-

ıˆ˜ Ì ÛÙ¿‰ÈÔ ππ-πππ (63-78% ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ηÈ

62% ÛÙË ÌÂϤÙË Ì·˜). O Ù‡Ô˜ Ù˘ Ô˙Ò‰Ô˘˜ ÏÂÌÊÔ-

΢ÙÙ·ÚÈ΋˜ ÂÈÎÚ¿ÙËÛ˘ ··ÓÙ¿Ù·È Û ËÏÈ˘ 25-

45 ÂÙÒÓ, Ì ÂÈÎÚ¿ÙËÛË ÙÔ˘ ¿ÚÚÂÓÔ˜ ʇÏÔ˘, ηÈ

·ÔÙÂÏ› ÙÔ 5% ÙˆÓ ÏÂÌÊˆÌ¿ÙˆÓ Hodgkin (12).

∞fi ÙÔ ÎÏ·ÛÈÎfi ϤÌʈ̷ Hodgkin, Ô ÏÂÌÊÔÂÈÎÚ·-

ÙÒÓ ˘fiÙ˘Ô˜ ‰È·ÈÛÙÒÓÂÙ·È ÛÙÔ 10-15% ÙˆÓ ·ÛıÂ-

ÓÒÓ, Â›Ó·È ÈÔ Û˘¯Ófi˜ ÛÙÔ ¿ÚÚÂÓ Ê‡ÏÔ Î·È ÙÔ˘˜ ÌÈ-

ÎÚfiÙÂÚÔ˘˜ Û ËÏÈΛ· ·ÛıÂÓ›˜, ÂÓÒ Û˘Ó‰¤ÂÙ·È Û˘-

¯ÓfiÙÂÚ· Ì ÂÓÙÔÈṲ̂ÓË ÓfiÛÔ (3). √ ÏÂÌÊÔÂÓÈÎfi˜

˘fiÙ˘Ô˜ Â›Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈÔ˜ ÛÙ· ·È‰È¿

(~5%), ÂÓÒ Û˘Ó‰¤ÂÙ·È Û˘¯Ó¿ Ì HIV Ïԛ̈ÍË.

∞ÛıÂÓ›˜ Ì ·˘ÙfiÓ ÙÔÓ Ù‡Ô Û˘Ó‹ıˆ˜ ¤¯Ô˘Ó Û˘-

ÌÙÒÌ·Ù· Ù‡Ô˘ µ, ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È Ì ٷ¯¤ˆ˜ ÂÍÂ-

ÏÈÛÛfiÌÂÓË ÓfiÛÔ Ô˘ ÂÚÈÏ·Ì‚¿ÓÂÈ Ù· ÔÛÙ¿ Î·È ÙÔÓ

Ì˘ÂÏfi ÙˆÓ ÔÛÙÒÓ Î·È ·ÚÔ˘ÛÈ¿˙ÂÈ ÙË ¯ÂÈÚfiÙÂÚË

ÚfiÁÓˆÛË ·fi fiÏÔ˘˜ ÙÔ˘˜ ˘fiÙ˘Ô˘˜ (3,5). √ ˘fi-

Ù˘Ô˜ Ù˘ ÌÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ ÂÌÊ·Ó›˙ÂÙ·È ÛÙÔ

14-38% ÙˆÓ ·ÛıÂÓÒÓ (5,6,11), ‰È·ÈÛÙÒÓÂÙ·È Û˘¯Ófi-

ÙÂÚ· Û ·È‰È¿ 10 ÂÙÒÓ ‹ ÌÈÎÚfiÙÂÚ· Î·È ÂÌÊ·Ó›˙ÂÙ·È

Û˘¯Ó¿ ˆ˜ ÚÔ¯ˆÚË̤ÓË ÓfiÛÔ˜ Ì Â͈ÏÂÌÊ·‰ÂÓÈ΋

ÂÓÙfiÈÛË. ∏ Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË ·ÔÙÂÏ› ÙÔÓ ÈÔ

Û˘¯Ófi ˘fiÙ˘Ô ÛÙȘ ·ÓÂÙ˘Á̤Ó˜ ¯ÒÚ˜ Î·È ÂÌÊ·-

Ó›˙ÂÙ·È Û˘¯ÓfiÙÂÚ· ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ Î·È ÙÔ˘˜ Ó·ÚÔ‡˜

ÂÓ‹ÏÈΘ. ¶·Ú·ÙËÚÂ›Ù·È ÛÙÔ 40% ÙˆÓ ÓÂfiÙÂÚˆÓ

·ÛıÂÓÒÓ Î·È ÛÙÔ 70% ÙˆÓ ÂÊ‹‚ˆÓ. ∞ÔÙÂÏ› ÙÔÓ

ÌfiÓÔ ˘fiÙ˘Ô ÛÙÔÓ ÔÔ›Ô ‰ÂÓ ÂÈÎÚ·Ù› ÙÔ ¿ÚÚÂÓ

ʇÏÔ, ηıÒ˜ ‰È·ÈÛÙÒÓÂÙ·È Ì ›ÛË Û˘¯ÓfiÙËÙ· Û ¿Ú-

ÚÂÓ· Î·È ı‹Ï· (13). °ÂÓÈο, Ô ÏÂÌÊÔÂÈÎÚ·ÙÒÓ

˘fiÙ˘Ô˜ Î·È ·˘Ùfi˜ Ù˘ Ô˙Ò‰Ô˘˜ ÛÎÏ‹Ú˘ÓÛ˘ Û¯Â-

Ù›˙ÔÓÙ·È Ì ÓfiÛÔ ¯·ÌËÏÔ‡ ÛÙ·‰›Ô˘, Ô ÏÂÌÊÔÂÓÈÎfi˜

Ì ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È·, ÂÓÒ Ô ˘fiÙ˘Ô˜ Ù˘ ÌÈ-

ÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ Ì ÂӉȿÌÂÛ·. ™ÙÔ˘˜ ·ÛıÂ-

Ó›˜ Ì·˜ ÂÈÎÚ·Ù› Ë Ô˙҉˘ ÛÎÏ‹Ú˘ÓÛË (63%), ÂÓÒ

ÌfiÓÔ Û ‰‡Ô ·È‰È¿ ‰È·ÈÛÙÒıËΠÏÂÌÊÔÂÓÈÎfi˜

˘fiÙ˘Ô˜. ∆¤ÏÔ˜, fiÛÔÓ ·ÊÔÚ¿ ÙËÓ ¤Î‚·ÛË ‰ÂÓ ‰È·-

ÈÛÙÒıËΠ›‰Ú·ÛË Ù˘ ÈÛÙÔÏÔÁ›·˜, ÛÂ Û˘Ìʈӛ·

Ì ٷ Â˘Ú‹Ì·Ù· ¿ÏÏˆÓ ÌÂÏÂÙÒÓ (5,6). º·›ÓÂÙ·È ˆ˜

ÌÂÙ¿ ÙËÓ ÂÈÛ·ÁˆÁ‹ ÙˆÓ Û‡Á¯ÚÔÓˆÓ Û¯ËÌ¿ÙˆÓ ËÌÂÈ-

ÔıÂڷ›·˜, Ù· ÈÛÙÔÏÔÁÈο ¯·Ú·ÎÙËÚÈÛÙÈο ¤¯Ô˘Ó

ÌÈÎÚfiÙÂÚË ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· (14).

∏ ‰È¿ÌÂÛË ÙÈÌ‹ ËÏÈΛ·˜ ÙˆÓ ·ÛıÂÓÒÓ Ù˘ ÌÂϤ-

Ù˘ Ì·˜ ‹Ù·Ó 12 ¤ÙË, ÂÓÒ ·ÍÈÔÛËÌ›ˆÙË Â›Ó·È Ë Û˘-

¯ÓfiÙËÙ· ÙˆÓ ·ÛıÂÓÒÓ ËÏÈΛ·˜ 5 ÂÙÒÓ ‹ ÌÈÎÚfiÙÂÚ˘

(9,6%, 7 ·ÛıÂÓ›˜). ∆Ô ÔÛÔÛÙfi ·È‰ÈÒÓ Ì ËÏÈΛ·

οو ÙˆÓ 5 ÂÙÒÓ ÛÙËÓ ÌÂϤÙË Ì·˜ Â›Ó·È ˘„ËÏfiÙÂÚÔ

·fi ·ÓÙ›ÛÙÔȯ· Û ·ÓÂÙ˘Á̤Ó˜ ¯ÒÚ˜ (4), ·ÏÏ¿

ÚÔÛÂÁÁ›˙ÂÈ ‹ Û·ÊÒ˜ ÔÏ›ÂÙ·È ÙÔ ·ÓÙ›ÛÙÔÈ¯Ô Ô-

ÛÔÛÙfi ¯ˆÚÒÓ Ù˘ ¢˘ÙÈ΋˜ ∞Û›·˜ Î·È ¿ÏÏˆÓ ·Ó·-

Ù˘ÛÛfiÌÂÓˆÓ ¯ˆÚÒÓ (15,16). ø˜ ÚÔ˜ ÙÔ Ê‡ÏÔ, ÛÙË

‚È‚ÏÈÔÁÚ·Ê›· ‰È·ÈÛÙÒÓÂÙ·È ÌÈ· ÂÏ·ÊÚ¿ ÂÈÎÚ¿ÙË-

ÛË ÙˆÓ ·ÚÚ¤ÓˆÓ (1.5:1), Ô˘ fï˜ Êı›ÓÂÈ Ì ÙËÓ ¿-

ÚÔ‰Ô Ù˘ ËÏÈΛ·˜, Ì ÙËÓ ·Ó·ÏÔÁ›· Ó· Â›Ó·È ·Úfi-

ÌÔÈ· ÛÙÔ˘˜ ÂÊ‹‚Ô˘˜ (3). ™ÙËÓ ·ÚÔ‡Û· ÌÂϤÙË ‰È·-

ÈÛÙÒÓÂÙ·È Û·Ê‹˜ ÂÈÎÚ¿ÙËÛË ÙÔ˘ ¿ÚÚÂÓÔ˜ ʇÏÔ˘,

Ì ٷ ÎÔÚ›ÙÛÈ· Ó· ¤¯Ô˘Ó Û ÛËÌ·ÓÙÈÎfi ‚·ıÌfi ÌÂÁ·-

χÙÂÚË ËÏÈΛ·. ÷ڷÎÙËÚÈÛÙÈο ‰È·ÈÛÙÒÓÔ˘Ì ˆ˜

128 ∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:128-000

ª‹Ó˜4003002001000

¯1

00

%1,00

0,80

0,60

0,40

0,20

0,00

∂ÈÎfiÓ· 2. ∫·Ì‡ÏË Û˘ÓÔÏÈ΋˜ ÂÈ‚›ˆÛ˘.

ª‹Ó˜4003002001000

¯1

00

%

1,00

0,80

0,60

0,40

0,20

0,00

∂ÈÎfiÓ· 3. ∫·Ì‡ÏË ÂÈ‚›ˆÛ˘ ÂχıÂÚ˘ Û˘Ì‚¿Ì·ÙÔ˜.

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·128

Page 57: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

fiÏÔÈ ÔÈ ·ÛıÂÓ›˜ ËÏÈΛ·˜ 7 ÂÙÒÓ ‹ ÌÈÎÚfiÙÂÚ˘ ‹Ù·Ó

·ÁfiÚÈ· (∂ÈÎfiÓ· 1). ∏ ÓfiÛÔ˜ ÛÙ· ·È‰È¿ ¤¯ÂÈ Û˘Ó‰Âı›

Ì ˘„ËÏfi ÎÔÈÓˆÓÈÎÔÔÈÎÔÓÔÌÈÎfi Â›Â‰Ô (ÛÙȘ ‚ÈÔ-

Ì˯·ÓÈΤ˜ ¯ÒÚ˜), ·ÏÏ¿ Î·È Ì ¤ÎıÂÛË Û ÏÔÈÌÒ‰ÂȘ

·Ú¿ÁÔÓÙ˜ (EBV, ÂÚËÙÔ˚fi˜ 6, CMV) ‹ Ì ·ÓÔÛÔ·-

Ó¿ÚÎÂÈ· (3). ™ËÌ·ÓÙÈ΋ ·Ó·ÏÔÁ›· ÙˆÓ ·ÛıÂÓÒÓ ÌÂ

ÓfiÛÔ ∏odgkin ¤¯Ô˘Ó ˘„ËÏÔ‡˜ Ù›ÙÏÔ˘˜ ·ÓÙÈۈ̿وÓ

¤Ó·ÓÙÈ ÙÔ˘ EBV (17). ™ÙËÓ ÂÚ›ÙˆÛË Ù˘ ÎÏ·ÛÈ΋˜

ÓfiÛÔ˘ Hodgkin, ÛÙȘ ¢˘ÙÈΤ˜ ¯ÒÚ˜ Ë Û‡Ó‰ÂÛË ÌÂ

EBV Ïԛ̈ÍË Êı¿ÓÂÈ ÙÔ 50% (18). ™ÙÔ˘˜ ·ÛıÂÓ›˜

Ì·˜, Ë ·Ó·ÏÔÁ›· ·˘ÙÒÓ Ì ·ÚÔ˘Û›· EBV-IgG ¤Êı·-

Ó ÙÔ 41%, ¯ˆÚ›˜ Ó· ·Ô‰›‰Ô˘Ì ȉȷ›ÙÂÚË ÛËÌ·Û›·

ÛÙÔ Â‡ÚËÌ¿ Ì·˜, ηıÒ˜ ˘„ËÏfi ÔÛÔÛÙfi ·È‰ÈÒÓ Î·È

ÂÊ‹‚ˆÓ ÛÙË ¯ÒÚ· Ì·˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ÓÙÈÛÒÌ·Ù·

¤Ó·ÓÙÈ ÙÔ˘ EBV. ∂›Û˘, Ë ÓfiÛÔ˜ ∏odgkin ¤¯ÂÈ Û˘Ó-

‰Âı› Î·È Ì ·˘ÙÔ¿ÓÔÛ· ÓÔÛ‹Ì·Ù· (19).

∆Ô Û˘¯ÓfiÙÂÚÔ ÎÏÈÓÈÎfi ÛËÌÂ›Ô Ù˘ ÓfiÛÔ˘

Hodgkin Â›Ó·È Ë ÏÂÌÊ·‰ÂÓÔ¿ıÂÈ·, Ì ·ÚÔ˘Û›· ÛÙÔ

90% ÙˆÓ ·ÛıÂÓÒÓ (20), ·Ó Î·È Û¯Â‰fiÓ Î¿ı fiÚÁ·ÓÔ

ÌÔÚ› Ó· ÚÔÛ‚ÏËı›. ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ‰È·È-

ÛÙÒıËΠÏÂÌÊ·‰ÂÓÔ¿ıÂÈ· Û ÔÛÔÛÙfi 93%. ªË ÂÈ-

‰Èο Û˘ÌÙÒÌ·Ù· Ù˘ ÓfiÛÔ˘ ·ÔÙÂÏÔ‡Ó Ë Î·ÎÔ˘¯›·,

Ë ·ÓÔÚÂÍ›·, ÙÔ ·Ú·ÙÂÈÓfiÌÂÓÔ ˘Ú¤ÙÈÔ Î·È Ë ·Ò-

ÏÂÈ· ‚¿ÚÔ˘˜ (3). ∂›Û˘, ÌÔÚ› Ó· Û˘Ó˘¿Ú¯ÂÈ ÌË

·Ú·ÁˆÁÈÎfi˜ ¯ÚfiÓÈÔ˜ ‚‹¯·˜ ‹ ¿ÏÏ· Û˘ÌÙÒÌ·Ù·

·fi ÙÔ ·Ó·Ó¢ÛÙÈÎfi, ÏfiÁˆ Ù˘ ‰È‡ڢÓÛ˘ ÙÔ˘ ÌÂ-

ÛÔıˆÚ·Î›Ô˘, Ô˘ ÚÔηÏ› ›ÂÛË Ù˘ ÙÚ·¯Â›·˜ ηÈ

ÙˆÓ ‚ÚfiÁ¯ˆÓ. ∏ ·ÚÔ˘Û›· µ-Û˘Ìو̿وÓ, fiˆ˜

·Ó·Ê¤ÚıËΠ‹‰Ë, ıˆÚÂ›Ù·È ˆ˜ ¤¯ÂÈ ÛËÌ·ÓÙÈ΋

ÚÔÁÓˆÛÙÈ΋ ÛËÌ·Û›· Î·È Û˘Ó‰¤ÂÙ·È Ì ÂÈıÂÙÈÎfi-

ÙÂÚË ÓfiÛÔ (21). ™ÙÔ˘˜ ·ÛıÂÓ›˜ Ì·˜ ·Ó·Ê¤ÚıËηÓ

Û ·Ó·ÏÔÁ›· 29%, Ì ÛËÌ·ÓÙÈο ÌÂÁ·Ï‡ÙÂÚË ·Ó·ÏÔ-

Á›· Û ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È· (44% Û ÛÙ¿‰ÈÔ πππ ‹

IV), ·ÏÏ¿ ‰ÂÓ ‰È·ÈÛÙÒıËÎÂ Û˘Û¯¤ÙÈÛË Ì ÙËÓ ¤Î‚·-

ÛË Ù˘ ÓfiÛÔ˘, ÛÂ Û˘Ìʈӛ· Ì ¿ÏÏÔ˘˜ ÂÚ¢ÓËÙ¤˜

(5). ∞ÓÙ›ıÂÙ·, ÔÈ Smith Î·È Û˘Ó. ‰È·›ÛÙˆÛ·Ó Û˘Û¯¤-

ÙÈÛË Ù˘ ·ÚÔ˘Û›·˜ µ-Û˘ÌÙˆÌ¿ÙˆÓ Ì ‰˘ÛÌÂÓ¤-

ÛÙÂÚË ÚfiÁÓˆÛË (13). ™ÙË ÌÂϤÙË Ì·˜ ‰ÂÓ ÚԤ΢-

„Â Û˘Û¯¤ÙÈÛË ÂÚÁ·ÛÙËÚÈ·ÎÒÓ Â˘ÚËÌ¿ÙˆÓ Î·Ù¿ ÙË

‰È¿ÁÓˆÛË, fiˆ˜ Ï¢ÎÔ΢ÙÙ¿ÚˆÛË, ˈÛÈÓÔÊÈÏ›·,

129§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿

¶·È‰È·ÙÚÈ΋ 2009;72:129-000

¶›Ó·Î·˜ 7. ¶ÚÔÁÓˆÛÙÈÎÔ› ·Ú¿ÁÔÓÙ˜ ˆ˜ ÚÔ˜ ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘

∞ÚÈıÌfi˜ % ƒ·ÛıÂÓÒÓ % DFS EFS OS DFS EFS OS

∏ÏÈΛ· (¤ÙË≤ 8 15 20,5 80 73,3 100 ns ns ns> 8 58 79,5 91,4 86,2 96,6

º‡ÏÔ∞ÁfiÚÈ· 46 63 89,1 84,8 97,8 ns ns ns∫ÔÚ›ÙÛÈ· 27 37 88,9 81,5 96,3

™Ù¿‰ÈÔπ 16 22 93,8 81,3 93,8ππ 25 34,2 96 92 100 ns ns nsπππ 20 27,4 85 80 95IV 12 16,4 75 75 100

πÛÙÔÏÔÁÈÎfi˜ ˘fiÙ˘Ô˜ªÈÎÙ‹˜ ΢ÙÙ·ÚÔ‚Ú›ıÂÈ·˜ 19 26,1 89,5 89,5 100§ÂÌÊÔÂÓÈÎfi˜ 2 2,7 100 100 100 ns ns ns§ÂÌÊÔÂÈÎÚ·ÙÒÓ 6 8,2 100 83,3 83,3√˙҉˘ ÛÎÏ‹Ú˘ÓÛË 46 63 87 80,4 97,8

µ-Û˘ÌÙÒÌ·Ù·¶·ÚfiÓÙ· 21 29 90,5 90,5 95,2 ns ns ns∞fiÓÙ· 52 71 88,5 80,8 98,1

ªÂÛÔıˆÚ¿ÎÈÔªÂÁ¿ÏË ‰È‡ڢÓÛË 13 17,8 92,3 84,6 100ª¤ÙÚÈ· ‰È‡ڢÓÛË 33 45,2 87,9 84,8 97 ns ns nsŸ¯È ‰È‡ڢÓÛË 27 37 88,9 81,5 96,3

§Â˘Î¿ ·ÈÌÔÛÊ·›ÚÈ· (x103/mm3)≤10,2 34 50,7 88,2 82,4 94,1 ns ns ns>10,2 33 49,3 90,9 84,8 100

Hb (g/dl)≤11,1 36 53 88,9 80,6 100 ns ns ns>11,1 32 47 90,6 87,5 93,8

TKE (mm 1˘ ÒÚ·˜)≤61 34 50 88,2 82,4 94,1 ns ns ns>61 34 50 91,2 85,3 100

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·129

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·Ó·ÈÌ›·, ·˘ÍË̤ÓË ÙÈÌ‹ ∆∫∂ Î·È ·˘ÍË̤ÓË ÙÈÌ‹

LDH, Ì ÙËÓ ¤Î‚·ÛË Ù˘ ÓfiÛÔ˘. ™Â Û¯¤ÛË Ì ٷ

·ÂÈÎÔÓÈÛÙÈο Â˘Ú‹Ì·Ù·, Ë ¤Î‚·ÛË ‰ÂÓ ‰È·ÈÛÙÒıË-

Πӷ ÂËÚ¿˙ÂÙ·È ·fi ÙËÓ ·ÚÔ˘Û›· ‰È‡ڢÓÛ˘

ÙÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘, ·ÎfiÌË Î·È ÂÎÙÂٷ̤Ó˘, ·Ó ηÈ

ÛÙË ‚È‚ÏÈÔÁÚ·Ê›· ·Ó·Ê¤ÚÂÙ·È ˆ˜ Ì¿˙· ÌÂÛÔıˆÚ·-

ΛԢ Ô˘ ˘ÂÚ‚·›ÓÂÈ ÙÔ 1/3 Ù˘ ÌÂÁ·Ï‡ÙÂÚ˘ ÂÓ‰Ô-

ıˆÚ·ÎÈ΋˜ ‰È·Ì¤ÙÚÔ˘ Û˘Ó‰¤ÂÙ·È Ì ÚÔ¯ˆÚË̤ÓË

ÓfiÛÔ, Ùˆ¯fiÙÂÚË ÚfiÁÓˆÛË Î·È ·Ó¿ÁÎË ÂÈıÂÙÈÎfi-

ÙÂÚ˘ ıÂڷ›·˜ (22).

™˘ÓÔÏÈο, Ë ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ ‹Ù·Ó ÂÍ·È-

ÚÂÙÈ΋ Û fiϘ ÙȘ ¯ÚÔÓÈΤ˜ ÂÚ›Ô‰Ô˘˜ ·ÓÙÈÌÂÙÒÈ-

Û˘ ·˘ÙÒÓ Î·È ·ÓÂÍ¿ÚÙËÙË ·fi ÙÔ Â›‰Ô˜ Ù˘ ıÂڷ›-

·˜. ∏ Û˘ÓÔÏÈ΋ ÂÈ‚›ˆÛË Êı¿ÓÂÈ ÙÔ 97,3%, ÂÓÒ ÂÍ·È-

ÚÂÙÈ΋ Â›Ó·È Î·È Ë ¤Î‚·ÛË ÙˆÓ ·ÛıÂÓÒÓ Ì ˘ÔÙÚÔ‹

- ÂÈ‚ÈÒÓÔ˘Ó ÔÈ 7/8. ∏ ÂÈ‚›ˆÛË ÌÂÙ¿ ·fi ˘ÔÙÚÔ‹

ÁÂÓÈο Î˘Ì·›ÓÂÙ·È ÛÙÔ 50-80% (3). ™ÙË ÌÂϤÙË Ì·˜, ÔÈ

˘ÔÙÚÔ¤˜ ¤Ï·‚·Ó ¯ÒÚ· 20-65 Ì‹Ó˜ ·fi ÙË ‰È¿ÁÓˆ-

ÛË. √È ˘ÔÙÚÔ¤˜ Û˘Ì‚·›ÓÔ˘Ó Û˘Ó‹ıˆ˜ Ù· ÚÒÙ· 3

¤ÙË, ·ÏÏ¿ ÔÚÈṲ̂ÓÔÈ ·ÛıÂÓ›˜ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Î·È fi„È-

̘ ˘ÔÙÚÔ¤˜ (¿Óˆ ·fi 10 ¤ÙË) (23).

√È ·ÏÏ·Á¤˜ ÛÙÔÓ ÙÚfiÔ ·ÓÙÈÌÂÙÒÈÛ˘ Ù˘ ÓfiÛÔ˘

‰È·¯ÚÔÓÈο ÔÊ›ÏÔÓÙ·È ÛÙËÓ ÂÌÂÈÚ›· ·fi ÙËÓ ÂÊ·Ú-

ÌÔÁ‹ ·ÚfiÌÔÈˆÓ ıÂڷ¢ÙÈÎÒÓ ÚÔÛÂÁÁ›ÛˆÓ. ™ÙËÓ

ÚÒÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô, Ù· ·È‰È¿ ·ÓÙÈÌÂÙˆ›ÛÙË-

Î·Ó ˆ˜ ÂÓ‹ÏÈΘ Î·È ÛÙË ıÂڷ›· ΢ÚÈ¿Ú¯ËÛ·Ó ÔÈ

˘„ËϤ˜ ‰fiÛÂȘ ·ÎÙÈÓÔ‚ÔÏ›·˜ (̤¯ÚÈ Î·È 40 Gy) ‹ Ë

·ÓÙÈÌÂÙÒÈÛË Ì ÔÏÏ¿ Û¯‹Ì·Ù· ¯ËÌÂÈÔıÂڷ›·˜

¯ˆÚ›˜ ·ÎÙÈÓÔıÂڷ›·. ªÂÙ¿ Ù· ÚÒÙ· ÌËӇ̷ٷ ÁÈ·

ÛÔ‚·Ú¤˜ ·ÒÙÂÚ˜ Û˘Ó¤ÂȘ, fiÛÔÓ ·ÊÔÚ¿ ΢ڛˆ˜

ÛÎÂÏÂÙÈΤ˜ ·ÓˆÌ·Ï›Â˜ Î·È ÚÔ‚Ï‹Ì·Ù· ÂÓ‰ÔÎÚÈÓÔ-

ÏÔÁÈο, ‹ÚÍ ·ÏÏ·Á‹ ÛÙË ıÂڷ¢ÙÈ΋ ÛÙÚ·ÙËÁÈ΋

Î·È Ù· ·È‰È¿-·ÛıÂÓ›˜ ·ÓÙÈÌÂÙˆ›ÛÙËÎ·Ó Ì Ì›ˆÛË

Ù˘ ¯ËÌÂÈÔıÂڷ›·˜ ÛÙËÓ ÂÓÙÔÈṲ̂ÓË ÓfiÛÔ Î·È,

‚¤‚·È·, Ì ηıȤڈÛË ¯·ÌËÏÒÓ ‰fiÛÂˆÓ ·ÎÙÈÓÔ‚Ô-

Ï›·˜ (20 Gy) ÌfiÓÔ ÛÙȘ ·Ú¯Èο ÚÔۂ‚ÏË̤Ó˜ Â-

ÚÈÔ¯¤˜. ∆¤ÏÔ˜, ÛÙËÓ ÙÚ›ÙË ¯ÚÔÓÈ΋ ÂÚ›Ô‰Ô, Ë ıÂÚ·-

¢ÙÈ΋ ÔÌ¿‰·, ‰È·ÙËÚÒÓÙ·˜ ÙȘ ¯·ÌËϤ˜ ‰fiÛÂȘ

·ÎÙÈÓÔ‚ÔÏ›·˜, ÚÔ¯ÒÚËÛÂ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ

·È‰ÈÒÓ Ì ÂÓÙÔÈṲ̂ÓË ¯·ÌËÏÔ‡ Î·È ÂӉȿÌÂÛÔ˘

ÎÈÓ‰‡ÓÔ˘ ÓfiÛÔ Ì ¯ËÌÂÈÔıÂڷ¢ÙÈο Û¯‹Ì·Ù· ¯ˆ-

Ú›˜ ·Ï΢ÏÈÔ‡ÓÙ˜ ·Ú¿ÁÔÓÙ˜ Î·È ·Óıڷ΢ÎϛӘ,

Ô˘ Â›Ó·È Ê¿Ú̷η ˘Â‡ı˘Ó· ÁÈ· ÛËÌ·ÓÙÈ΋ ·ÒÙÂ-

ÚË ÙÔÍÈÎfiÙËÙ·. °ÂÓÈο, fiÏ· ·˘Ù¿ Ù· ¯ÚfiÓÈ· ÔÈ ÛÙfi¯ÔÈ

Ù˘ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ ·ÛıÂÓÒÓ Ì·˜ Ì ÓfiÛÔ

∏odgkin ‹Ù·Ó Ë ‰È·Ù‹ÚËÛË ÙˆÓ ˘„ËÏÒÓ ÔÛÔÛÙÒÓ

ÂÈ‚›ˆÛ˘, Ì ٷ˘Ùfi¯ÚÔÓË Ì›ˆÛË Ù˘ Ì·ÎÚÔÚfiıÂ-

ÛÌ˘ ÙÔÍÈÎfiÙËÙ·˜ (ηډÈÔÙÔÍÈÎfiÙËÙ·, Ó¢ÌÔÓÔ¿-

ıÂÈ·, ÛÎÂÏÂÙÈΤ˜ ‰È·Ù·Ú·¯¤˜, ÂÓ‰ÔÎÚÈÓÔÏÔÁÈΤ˜ ‰È·-

Ù·Ú·¯¤˜, ‰Â‡ÙÂÚ˜ ηÎÔ‹ıÂȘ) (24). ∞fi ÙÔ˘˜ ·ÛıÂ-

Ó›˜ Ì·˜, Ù¤ÛÛÂÚȘ ·ÚÔ˘Û›·Û·Ó ‰Â‡ÙÂÚË ‹ Î·È ÙÚ›ÙË

ηÎÔ‹ıÂÈ·. ∏ Û˘¯ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ ‰Â‡ÙÂÚ˘ η-

ÎÔ‹ıÂÈ·˜ ÂÍ·ÚÙ¿Ù·È ·fi ÙËÓ ÂÊ·ÚÌÔÁ‹ ‹ fi¯È ·ÎÙÈÓÔ-

ıÂڷ›·˜ Î·È ÙË ‰fiÛË ·˘Ù‹˜, ÙÔ ÛÙ¿‰ÈÔ Ù˘ ÓfiÛÔ˘ ‹ÙËÓ ‡·ÚÍË ˘ÔÙÚÔ‹˜ Î·È ÙËÓ ·ÚÔ˘Û›· ·ÓÔÛÔη-Ù·ÛÙÔÏ‹˜. π‰È·›ÙÂÚÔ ÚfiÏÔ Ê·›ÓÂÙ·È Ó· ·›˙ÂÈ Ë Ú‹ÛË·Ï΢ÏȈÙÈÎÒÓ ·Ú·ÁÒÁˆÓ (ª√PP), ηıÒ˜ Î·È ÔÈ ‰fi-ÛÂȘ Ù˘ ¯ËÌÂÈÔıÂڷ›·˜ (25). √ ΛӉ˘ÓÔ˜ ÁÈ· ÂÌ-Ê¿ÓÈÛË ÌË ÏÂÌÊÔ‚Ï·ÛÙÈ΋˜ Ï¢¯·ÈÌ›·˜ ‹ ÌË∏odgkin ÏÂÌÊÒÌ·ÙÔ˜ Êı¿ÓÂÈ ÙÔ 8,1% ÌÂÙ¿ ·fi ıÂ-ڷ›· ÁÈ· ÓfiÛÔ Hodgkin, ÂÓÒ Ô Î›Ó‰˘ÓÔ˜ ÁÈ· Û˘Ì·-Á‹ fiÁÎÔ Êı¿ÓÂÈ ÙÔ 13,1% ÛÙ· 20 ¤ÙË (26).

ª¤¯ÚÈ Û‹ÌÂÚ·, Ë ıÂڷ¢ÙÈ΋ ÚÔÛ¤ÁÁÈÛË Ù˘ÓfiÛÔ˘ ÛÙËÚ›˙ÂÙ·È Û ÚÔÛÂÁÁ›ÛÂȘ ÚÔÛ·ÚÌÔṲ̂Ó˜ÛÙËÓ ·¿ÓÙËÛË ÛÙËÓ ·Ú¯È΋ ıÂڷ›·, ÛÙÔ Ê‡ÏÔ Î·ÈÙËÓ ËÏÈΛ·. √ Û‡Á¯ÚÔÓÔ˜ ÙÚfiÔ˜ ·ÓÙÈÌÂÙÒÈÛ˘ ÙˆÓ·ÛıÂÓÒÓ Ì ÂÓÙÔÈṲ̂ÓË ·ÌËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ ÓfiÛÔ Â-ÚÈÏ·Ì‚¿ÓÂÈ ÏÈÁfiÙÂÚÔ ÙÔÍÈ΋ ıÂڷ›· Ì 2-4 ·ÎÏÔ˘˜¯ËÌÂÈÔıÂڷ›·˜ Î·È ¯·ÌËϤ˜ ‰fiÛÂȘ ·ÎÙÈÓÔ‚ÔÏ›·˜,ÂÓÒ ÁÈ· ÙÔ˘˜ ˘„ËÏÔ‡ ÎÈÓ‰‡ÓÔ˘ Î·È Ì ÚÔ¯ˆÚË̤ÓËÓfiÛÔ ·ÛıÂÓ›˜ ‰ÔÎÈÌ¿˙ÔÓÙ·È ÂÚÈÛÛfiÙÂÚÔ ÂÓÙ·ÙÈοۯ‹Ì·Ù· ıÂڷ›·˜ (27). ∆ÂÏÂ˘Ù·›·, ÙÔ Î˘Ú›·Ú¯ÔÂÚÒÙËÌ· ÙˆÓ ÌÂÁ¿ÏˆÓ Û˘ÓÂÚÁ·ÙÈÎÒÓ ÌÂÏÂÙÒÓ Â›Ó·È«ÔÈÔÈ ·fi ÙÔ˘˜ ·ÛıÂÓ›˜ ÌÔÚÔ‡Ó Ó· È·ıÔ‡Ó ¯ˆÚ›˜ÙË Ú‹ÛË ·ÎÙÈÓÔıÂڷ›·˜;». √ ·ӷÚÔÛ‰ÈÔÚÈÛÌfi˜ÙˆÓ ÔÌ¿‰ˆÓ ÎÈÓ‰‡ÓÔ˘ Ì ӤԢ˜ ‚ÈÔÏÔÁÈÎÔ‡˜ Î·È ÌÔ-ÚÈ·ÎÔ‡˜ ÚÔÁÓˆÛÙÈÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ‰˘ÓËÙÈο ı·Ô‰ËÁ‹ÛÂÈ Û ÂÚ·ÈÙ¤Úˆ ÂÚÈÔÚÈÛÌfi Ù˘ ıÂڷ›·˜ ‹Û ÂÓÙ·ÙÈÎÔÔ›ËÛË ·˘Ù‹˜. √È ÚfiÔ‰ÔÈ ÛÙË ıÂڷ›·ÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin ¤¯Ô˘Ó ÌÂÈÒÛÂÈ ÙËÓ ÚÔ-ÁÓˆÛÙÈ΋ ·Í›· ·Ú·‰ÔÛÈ·ÎÒÓ ÚÔÁÓˆÛÙÈÎÒÓ ·Ú·-ÁfiÓÙˆÓ, fiˆ˜ Ë ËÏÈΛ·, ÙÔ Ê‡ÏÔ, Ë ÈÛÙÔÏÔÁ›·, ÙÔ ÛÙ¿-‰ÈÔ Î·È Ù· Û˘ÌÙÒÌ·Ù· Ù‡Ô˘ µ. ∏ ·¿ÓÙËÛË ÛÙËÓ·Ú¯È΋ ıÂڷ›· ıˆÚÂ›Ù·È Û‹ÌÂÚ· ÚÔÛ‰ÈÔÚÈÛÙÈÎfi˜·Ú¿ÁÔÓÙ·˜ ÁÈ· ÙËÓ Â·ÎfiÏÔ˘ıË ıÂڷ¢ÙÈ΋ ÚÔ-Û¤ÁÁÈÛË. √È ÚfiÔ‰ÔÈ ÛÙȘ ·ÂÈÎÔÓÈÛÙÈΤ˜ ÌÂıfi‰Ô˘˜,fiˆ˜ Ë ¯Ú‹ÛË ˘ÔÏÔÁÈÛÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ ˘„ËÏ‹˜Â˘ÎÚ›ÓÂÈ·˜ (HRCT), Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜ (MRI),·ÏÏ¿, ÙÂÏÂ˘Ù·›·, Î·È Ë Ú‹ÛË ÙÔÌÔÁÚ·Ê›·˜ ÂÎÔÌ‹˜Ô˙ÈÙÚÔÓ›ˆÓ (PET) [ÂÎÙÈÌ¿ ÙËÓ ˘ÂÚÏ·ÛÙÈ΋ ‰Ú·-ÛÙËÚÈfiÙËÙ· ÙˆÓ ÏÂÌÊ·‰¤ÓˆÓ ‰È· ̤ÛÔ˘ Ù˘ ·˘ÍË̤-Ó˘ ÚfiÛÏ˄˘ ÙÔ˘ 18-fluoro-2-deoxyglucose(FDG)] ¤¯Ô˘Ó ʤÚÂÈ Â·Ó¿ÛÙ·ÛË ÛÙË ÛÙ·‰ÈÔÔ›ËÛËÙÔ˘ ÏÂÌÊÒÌ·ÙÔ˜ Hodgkin. ∏ ÙÔÌÔÁÚ·Ê›· ÂÎÔÌ‹˜Ô˙ÈÙÚÔÓ›ˆÓ Î·È ÂȉÈο Ô Û˘Ó‰˘·ÛÌfi˜ Ù˘ Ì ·ÍÔÓÈ΋ÙÔÌÔÁÚ·Ê›· (PET-CT) ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂÁ¿ÏË Â˘·ÈÛıË-Û›· ÁÈ· ÙËÓ ÂÎÙ›ÌËÛË Ù˘ ·ÓÙ·fiÎÚÈÛ˘ Î·È ÙËÓ ÔÚıfi-ÙÂÚË ¤ˆ˜ Î·È ÂÍ·ÙÔÌÈÎÂ˘Ì¤ÓË ıÂڷ›·, Ì ÚÔÔÙÈ-΋ ÙËÓ ·ÔÊ˘Á‹ Ù˘ ·ÎÙÈÓÔıÂڷ›·˜ Û ÛËÌ·ÓÙÈÎfiÔÛÔÛÙfi ·ÛıÂÓÒÓ (28).

µÈ‚ÏÈÔÁÚ·Ê›·

1. Gurney J, Bondy M. Epidemiology of childhood cancer. In:

Pizzo AP, Poplack DG (eds). Principles and Practice of

Pediatric Oncology, 5th Edt. Philadelphia, PA: Lippincott-

Raven Publishers; 2006, Chapter 1, pp. 1-13.2. Petridou E, Pourtsidis A, Dessypris N, Katsiardanis K, Baka

130 ∞. ¶Ô˘ÚÙÛ›‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:130-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·130

Page 59: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

M, Moschovi M, et al. Childhood leukaemias andlymphomas in Greece (1996-2006): a nationwide registra-tion study. Archives of Disease in Childhood. 2008;93:1027-1032.

3. Hudson M, Onciu M, Donaldson S. Hodgkin’s Disease. In:Pizzo P, Poplack D, editors. Principles and Practise ofPediatric Oncology, 5th Edt. Philadelphia; Lipincott-Raven Publishers; 2006, pp. 695-721.

4. Donaldson SS, Link MP, Weinstein HJ, Rai SN, Brain S,Billett AL, et al. Final results of a prospective clinical trialwith VAMP and low-dose involved-field radiation forchildren with low-risk Hodgkin's disease. J Clin Oncol2007;25:332-337.

5. Oguz A, Karadeniz C, Okur FV, Citak EC, Pinarli FG, BoraH, et al. Prognostic factors and treatment outcome inchildhood Hodgkin disease. Pediatr Blood Cancer 2005;45:670-675.

6. Hudson MM, Krasin M, Link MP, Donaldson SS, BillupsC, Merchant TE, et al. Risk-adapted, combined-modalitytherapy with VAMP/COP and response-based, involved-field radiation for unfavorable pediatric Hodgkin's disease.J Clin Oncol 2004;22:4541-4550.

7. Lister TA, Crowther D, Sutcliffe SB. Report of a committeeconvened to discuss the evaluation and staging of patientswith Hodgkin’s disease: Cotswolds meeting. J Clin Oncol1989;7:1630-1636.

8. Weinshel EL, Peterson BA. Hodgkin’s disease. CA: CancerJ Clin. 1993;43:327-372.

9. Oberlin O, Leverger G, Pacquement H, Raquin MA,Chompret A, Habrand JL, et al. Low-dose radiation therapyand reduced chemotherapy in childhood Hodgkin’s disease:the experience of the French Society of Pediatric Oncology. JClin Oncol 1992;10:1602-1608.

10. Landman-Parker J, Pacquement H, Leblanc T, HabrandJL, Terrier-Lacombe MJ, et al. Localized ChildhoodHodgkin’s Disease: Response-Adapted ChemotherapyWith Etoposide, Bleomycin, Vinblastine, and PrednisoneBefore Low-Dose Radiation Therapy - Results of theFrench Society of Pediatric Oncology Study MDH90. JClin Oncol 2000;18:1500-1507.

11. Feldman AL, Pittaluga S, Jaffe ES. Classification andhistopathology of the lymphomas. In: Canellos GP, ListerTA, Young B (eds) The lymphomas. 2nd ed., SaundersElsevier, Philadelphia; 2006, pp. 2-25.

12. Nogovaã L, Rudiger T, Engert A. Biology, clinical courseand management of nodular lymphocyte-predominanthodgkin lymphoma. Hematology Am Soc Hematol EducProgram 2006;1:266-272.

13. Smith RS, Chen Q, Hudson MM, Link MP, Kun L,Weinstein H et al. Prognostic factors for children withHodgkin’s disease treated with combined-modality therapy.J Clin Oncol. 2003;21:2026-2033.

14. Schwartz CL. Prognostic Factors in Pediatric HodgkinDisease. Current Oncology Reports 2003;5:498-504.

15. Belgaumi A, Al-Kofide A, Joseph N, Jamil-Malik R,Khafaga Y, Sabbah R. HL in very young children: Clinicalcharacteristics and outcome of treatment. LeukLymphoma 2008;49:910-916.

16. Viani GA, Castilho MS, Novaes PE, Antonelli CG, FerrignoR, Pellizzon CA, et al. Chemotherapy followed by low doseradiotherapy in childhood Hodgkin’s disease: Retro-spective analysis of results and prognostic factors.Radiation Oncology 2006;1:38-45.

17. Dinand V, Dawar R, Arya LS, Unni R, Mohanty B, Singh R,et al. Hodgkin’s lymphoma in Indian children: Prevalenceand significance of Epstein-Barr virus detection inHodgkin’s and Reed-Sternberg cells. European Journal ofCancer 2007;43:161-168.

18. Portis T, Dyck P, Longnecker R. Epstein-Barr Virus (EBV)LMP2A induces alterations in gene transcription similar tothose observed in Reed-Sternberg cells of Hodgkinlymphoma. Blood 2003;102:4166-4178.

19. Landgren O, Engels EA, Pfeiffer RM, Gridley G, MellemkjaerL, Olsen JH, et al. Autoimmunity and susceptibility toHodgkin lymphoma: a population-based case-control studyin Scandinavia. J Natl Cancer Inst 2006;98:1321-1330.

20. Mullen E, Zhong Y. Hodgkin Lymphoma: An Update.Journal for Nurse Practitioners 2007;3:393-403.

21. Schwartz CL. The management of Hodgkin disease in theyoung child. Curr Opin Pediatr 2003;15:10-16.

22. Diehl V, Stein H, Hummel M, Zollinger R, Connors JM.Hodgkin's lymphoma: biology and treatment strategies forprimary, refractory, and relapsed disease. Hematology. AmSoc Hematol Educ Program 2003;1:225-247.

23. Cashen ∞, Bartlett ¡. Therapy of Relapsed HodgkinLymphoma. Blood Reviews. 2007;21:233-243.

24. Bartlett ¡. Modern treatment of Hodgkin lymphoma.Current Opinion in Hematology 2008;15:408-414.

25. Ng AK, Mauch PM. The impact of treatment on the risk ofsecond malignancy after Hodgkin's disease. Ann Oncol2006;17:1727-1729.

26. van Leeuwen FE, Klokman WJ, Hagenbeek A, Noyon R,van den Belt-Dusebout AW, van Kerkhoff EH, et al.Second cancer risk following Hodgkin's disease: a 20-yearfollow-up study. J Clin Oncol 1994;12:312-325.

27. Olson MR, Donaldson SS. Treatment of pediatric Hodgkinlymphoma. Curr Treat Options Oncol 2008;9:81-94.

28. Malik E. Juweid. Utility of Positron Emission Tomography(PET) Scanning in Managing Patients with HodgkinLymphoma. Hematology, Am Soc Hematol Educ Program2006;1:259-265.

131§¤Ìʈ̷ Hodgkin ÛÙ· ·È‰È¿

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√È ÂÈÙÒÛÂȘ Ù˘ ÂÚÙÚÔÊ›·˜ ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È

·Ì˘Á‰·ÏÒÓ ÛÙËÓ Î·Ú‰È·Î‹ ÏÂÈÙÔ˘ÚÁ›· ÙˆÓ ·È‰ÈÒÓ

∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘

¶ÂÚ›ÏË„Ë: ∏ ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ ·ÔÙÂÏ› ÙÔ Û˘¯ÓfiÙÂÚÔ ·›ÙÈÔ ·fiÊڷ͢ ÙˆÓ·ÓÒÙÂÚˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ, Ô˘ ÌÔÚ› Ó· ¤¯ÂÈ ÛÔ‚·Ú¤˜ ÂÈÙÒÛÂȘ ÛÙËÓ Î·Ú‰ÈÔÓ¢ÌÔÓÈ΋ ÏÂÈÙÔ˘Ú-Á›· ÙˆÓ ·È‰ÈÒÓ. ∏ ·fiÊÚ·ÍË ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ÏfiÁˆ ÂÏ¿ÙÙˆÛ˘ ÙÔ˘ Ó¢ÚÔ-Ì˘˚ÎÔ‡ ÙfiÓÔ˘, Î·È Ô‰ËÁ› Û ˘fiÓÔÈ· ‹ ¿ÓÔȘ ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘. √È ÙÂÏÂ˘Ù·›Â˜ Û˘Óԉ‡ÔÓÙ·È ·fiÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜, ˘ÂÚηӛ·˜ Î·È ·Ó·Ó¢ÛÙÈ΋˜ ÔͤˆÛ˘ (Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜‡ÓÔ˘). ∆· ˘ÔÙÚÔÈ¿˙ÔÓÙ· ÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜ Î·È ˘ÂÚηӛ·˜ ÚÔηÏÔ‡Ó Ó¢ÌÔÓÈ΋ ·ÁÁÂÈ-ÔÛ‡Û·ÛË, Ë ÔÔ›· Ô‰ËÁ› Û ÌfiÓÈ̘ ·ÏÏÔÈÒÛÂȘ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ. √È ÂÈÙÒÛÂȘ ÙÔ˘ Û˘Ó-‰ÚfiÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi Û‡ÛÙËÌ· ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÂÂÈÛfi‰È· ˘ÂÚÙ·-ÛÈÎÒÓ ÎÚ›ÛÂˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ‰È·Ù·Ú·¯¤˜ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡ ηÈ, Û ·Ú·ÌÂÏË̤Ó˜ÂÚÈÙÒÛÂȘ, ÙËÓ ÂÌÊ¿ÓÈÛË Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ Î·È ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜. ∞Ó Î·È Ë ¯ÚfiÓÈ·Ó¢ÌÔÓÈ΋ ηډȿ Â›Ó·È Û¿ÓÈ· Ù· ÙÂÏÂ˘Ù·›· ¯ÚfiÓÈ·, ÌÔÚ› Ó· ˘¿Ú¯ÂÈ ·Û˘Ìو̷ÙÈ΋ Ó¢ÌÔÓÈ΋˘¤ÚÙ·ÛË Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜. ª¤¯ÚÈ Û‹ÌÂÚ·, ˆ˜ ıÂڷ›· ÚÒÙ˘ ÂÎÏÔÁ‹˜ Û ·È‰È¿ Ì ۇÓ-‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ ·Ú·Ì¤ÓÂÈ Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙˆÓ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ.√È ·ÛıÂÓ›˜ Ì ÛÔ‚·Ú‹ ·fiÊÚ·ÍË Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË ‹/Î·È Î·Ú‰È·Î‹ ‰˘ÛÏÂÈ-ÙÔ˘ÚÁ›· ¤¯Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ¿ÌÂÛ˜ ÌÂÙÂÁ¯ÂÈÚËÙÈΤ˜ ÂÈÏÔΤ˜, fiˆ˜ Âȉ›ӈÛË ÙÔ˘ Û˘Ó‰Úfi-ÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‹ ÂΉ‹ÏˆÛË Ó¢ÌÔÓÈÎÔ‡ Ôȉ‹Ì·ÙÔ˜. ŸÛÔÓ ·ÊÔÚ¿ ÛÙȘ ·ÒÙÂÚ˜ ÌÂÙÂÁ¯ÂÈ-ÚËÙÈΤ˜ ÂÈÏÔΤ˜, ·˘Ù¤˜ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ·Ú·ÌÔÓ‹ ΢„ÂÏȉÈÎÔ‡ ˘Ô·ÂÚÈÛÌÔ‡ (Ô˘ ÌÔÚ› Ó· ¯ÚÂÈ·-Ûı› ̤¯ÚÈ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ‹ ÙÚ·¯ÂÈÔÛÙÔÌ›·), ›ÌÔÓË ˘ÔÍ·ÈÌ›·, ‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘ ‹ Î·È Û˘Ó‰˘·-ÛÌfi ·˘ÙÒÓ. °È· ÙÔ˘˜ ·ÓˆÙ¤Úˆ ÏfiÁÔ˘˜, Û ·ÛıÂÓ›˜ Ì ÛÔ‚·Ú‹ ˘ÂÚÙÚÔÊ›· Û˘ÓÈÛÙ¿Ù·È ˘ÂÚ˯ÔηډÈÔ-ÁÚ·ÊÈÎfi˜ ¤ÏÂÁ¯Ô˜ ÚÈÓ ·fi ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË. ÃÚÂÈ¿˙ÂÙ·È Â›Û˘ ÎÏÈÓÈ΋ ·ÓÂÎÙ›ÌËÛË ·˘ÙÒÓÙˆÓ ·ÛıÂÓÒÓ ÌÂÙ¿ ·fi 6-8 ‚‰ÔÌ¿‰Â˜ ·fi ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔÌ‹-·Ì˘Á‰·ÏÂÎÙÔÌ‹, ÁÈ· Ó· ‰È·ÈÛÙˆı› ·Ó··ÈÙÂ›Ù·È ÂÈÚfiÛıÂÙË ıÂڷ›·.

§¤ÍÂȘ ÎÏÂȉȿ: ÀÂÚÙÚÔÊ›· ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ, Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘, Ó¢ÌÔÓÈ΋

˘¤ÚÙ·ÛË, ¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ηډȿ.

Cardiovascular consequences of adenotonsillar hypertrophy in

children

K. Papadopoulou-Legbelou, G. Varlamis

Abstract: Adenotonsillar hypertrophy is the most common cause of upper airway obstruction, which maylead to serious cardiopulmonary complications in children. Upper airway obstruction can be exacerbatedby the decrease in neuromuscular tone that occurs during sleep, leading to hypopnoea or obstructivesleep apnoea. Obstructive sleep apnoea produces episodic hypoxia, hypercapnia and respiratory acidosis(obstructive sleep apnoea syndrome). Episodes of recurrent hypoxia and hypercapnia are potentmediators of pulmonary vasoconstriction that can lead to chronic changes in the pulmonary vasculature.Cardiovascular consequences of adenotonsillar hypertrophy include repetitive nocturnal surges in bloodpressure, breathing rhythm disturbances and, particularly in severe cases, pulmonary hypertension andcor pulmonale. Although overt right heart failure now occurs less often, asymptomatic degrees ofpulmonary hypertension can occur. To date, adenotonsillectomy is the first line of treatment for childrenwith obstructive sleep apnoea syndrome. Children with significant airway obstruction associated withpulmonary hypertension and/or cardiac dysfunction are at greater risk for perioperative complications,such as worsening of their obstructive sleep apnoea, and pulmonary oedema. Potential postoperativecomplications are persistent airway obstruction (including the need for reintubation or tracheostomy),desaturation, sleep disturbances, or a combination of these. Thus, patients with severe obstruction shouldbe screened by echocardiography preoperatively. Clinical reevaluation is also recommended 6 to 8 weeksafter adenotonsillectomy, to determine whether additional treatment is required.

Key words: Adenotonsillar hypertrophy, obstructive sleep apnoea syndrome, pulmonary hypertension, cor

pulmonale.

132 ¶ƒ∞∫∆π∫√ £∂ª∞ PRACTICAL ISSUE

¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞ÚÈÛÙÔÙ¤ÏÂÈÔ˘ ¶·ÓÂÈÛÙ‹ÌÈÔ˘£ÂÛÛ·ÏÔӛ΢, ¡ÔÛÔÎÔÌ›ԫ¶··ÁˆÚÁ›Ô˘»

AÏÏËÏÔÁÚ·Ê›·:

∫˘Úȷ΋ ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘[email protected]¢’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋ ∞¶£,¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘»¶ÂÚÈÊÂÚÂȷ΋ Ô‰fi˜£ÂÛ/ӛ΢, ¡. ∂˘Î·Ú›·,56403, £ÂÛÛ·ÏÔÓ›ÎË

4th Department ofPaediatrics, “Aristotelio”University of Thessaloniki,“PapageÔrgiou” Hospital,Thessaloniki, Greece

Correspondence:

Kyriaki [email protected] Department ofPaediatrics, “Aristotelio”University of Thessaloniki,“PapageÔrgiou” HospitalRing Road, N. Efkarpia,56403, Thessaloniki, Greece

Paediatriki 2009;72:132-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·132

Page 61: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

™˘ÓÙÔÌÔÁڷʛ˜:

∞¶ ·ÚÙËÚȷ΋ ›ÂÛË¡™ Ó¢ÚÈÎfi Û‡ÛÙËÌ·™∞∞À Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘

∂ÈÛ·ÁˆÁ‹

∏ ˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ·ÔÙÂÏ› ÙÔ Û˘¯ÓfiÙÂÚÔ ·›ÙÈÔ ¯ÚfiÓÈ·˜ ·fiÊÚ·ÍË˜ÙˆÓ ·ÂÚÔÊfiÚˆÓ Ô‰ÒÓ, Ô˘ ÌÔÚ› Ó· ¤¯ÂÈ ÛÔ‚·Ú¤˜ÂÈÙÒÛÂȘ ÛÙËÓ Î·Ú‰ÈÔÓ¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· وӷȉÈÒÓ. ∏ ·fiÊÚ·ÍË ÂȉÂÈÓÒÓÂÙ·È Î·Ù¿ ÙË ‰È¿Ú-ÎÂÈ· ÙÔ˘ ‡ÓÔ˘, fiÔ˘ ¯·Ï·ÚÒÓÔ˘Ó ÔÈ Ê·Ú˘ÁÁÈÎԛ̇˜, ηıÒ˜ ›Û˘ ·fi Û˘Ó˘¿Ú¯Ô˘Û˜ ηٷÛÙ¿-ÛÂȘ, fiˆ˜ ˘ÔÙÚÔÈ¿˙Ô˘Û˜ ÏÔÈÌÒÍÂȘ ÙÔ˘ ·ÓÒÙÂ-ÚÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡ ‹ ·¯˘Û·ÚΛ· (1). ™Â ÛÔ‚·Ú‹˘ÂÚÙÚÔÊ›·, ·Ú·ÙËÚÔ‡ÓÙ·È Â›Û˘ ·ÔÊÚ·ÎÙÈÎÔ‡Ù‡Ô˘ ¿ÓÔȘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, ÔÈ ÔÔ›-˜ ¯·Ú·ÎÙËÚ›˙ÔÓÙ·È ·fi ·‰˘Ó·Ì›· ÂÈÛfi‰Ô˘ ·¤Ú· ·-Ú¿ ÙËÓ ‡·ÚÍË ·Ó·Ó¢ÛÙÈ΋˜ ÚÔÛ¿ıÂÈ·˜ ηȉȷÎÔ‹ ÙÔ˘ Ê˘ÛÈÔÏÔÁÈÎÔ‡ ·ÂÚÈÛÌÔ‡ Î·È Ù˘ ·Ú¯ÈÙÂ-ÎÙÔÓÈ΋˜ ÙÔ˘ ‡ÓÔ˘ (Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜¿ÓÔÈ·˜ ‡ÓÔ˘) (2).

∆Ô Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘(™∞∞À) ÛÙ· ·È‰È¿ Â›Ó·È ÈÔ Û˘¯Ófi Û ËÏÈΛ· 2-8¯ÚfiÓˆÓ, fiÔ˘ ˘¿Ú¯ÂÈ Î·È Ë ÌÂÁ·Ï‡ÙÂÚË ˘ÂÚÙÚÔ-Ê›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ÙˆÓ ·Ì˘Á‰·ÏÒÓ (2,3). ¶Ú¤-ÂÈ Ó· ‰È·ÊÔÚԉȷÁÓˆÛÙ› ·fi ÙÔ ÚˆÙÔ·ı¤˜ ÚÔ-¯·ÏËÙfi, ÛÙÔ ÔÔ›Ô ‰ÂÓ Û˘Ó˘¿Ú¯Ô˘Ó ·ÔÊÚ·ÎÙÈÎÔ‡Ù‡Ô˘ ¿ÓÔȘ, Û˘¯Ó¤˜ ·Ê˘Ó›ÛÂȘ ‹ ‰È·Ù·Ú·¯¤˜Ù˘ ·ÓÙ·ÏÏ·Á‹˜ ÙˆÓ ·ÂÚ›ˆÓ ÙÔ˘ ·›Ì·ÙÔ˜ (2). ªÂϤ-Ù˜ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ ‰ÂÓ ˘¿Ú¯ÂÈ Û˘Û¯¤ÙÈÛË ÌÂٷ͇ÙÔ˘ ÌÂÁ¤ıÔ˘˜ ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ Î·ÈÙ˘ ‡·Ú͢ ™∞∞À (4,5). ∞˘Ùfi Û˘Ì‚·›ÓÂÈ ‰ÈfiÙÈ ÙÔ™∞∞À Â›Ó·È Û˘Ó‰˘·ÛÌfi˜ ‰ÔÌÈÎÒÓ Î·È Ó¢ÚÔÌ˘˚ÎÒÓ·Ú·ÁfiÓÙˆÓ (3).

∏ Û˘¯ÓfiÙËÙ· ÙÔ˘ ™∞∞À ˘ÔÏÔÁ›˙ÂÙ·È Û 2%(2,6-8), ÂÓÒ Ë Û˘¯ÓfiÙËÙ· ÙÔ˘ ÚÔ¯·ÏËÙÔ‡ Â›Ó·È ÔχÌÂÁ·Ï‡ÙÂÚË Î·È Î˘Ì·›ÓÂÙ·È Û ‰È¿ÊÔÚ˜ ÌÂϤÙ˜ ÌÂ-ٷ͇ 8-27% (6,7,9-11). ŸÛÔÓ ·ÊÔÚ¿ ÛÙÔ Ê‡ÏÔ, Ë Û˘-¯ÓfiÙËÙ· Â›Ó·È ›ÛË ÌÂٷ͇ ÙˆÓ ‰‡Ô Ê‡ÏˆÓ (2,3).

H ·¯˘Û·ÚΛ·, Ô˘ ·˘Í¿ÓÂÙ·È ÛËÌ·ÓÙÈο Ù· ÙÂ-ÏÂ˘Ù·›· ¯ÚfiÓÈ· ÛÙËÓ ·È‰È΋ ËÏÈΛ·, ·ÔÙÂÏ› ϤÔÓ¤Ó· ÛËÌ·ÓÙÈÎfi ÚԉȷıÂÛÈÎfi ·Ú¿ÁÔÓÙ· ÙÔ˘ ™∞∞À.ŒÙÛÈ, ÙÔ ™∞∞À ‰È·ÎÚ›ÓÂÙ·È ÛÙȘ ÂÍ‹˜ ηÙËÁÔڛ˜: Ù‡-Ô˘ π, ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È ÛÔ‚·Ú‹ ˘ÂÚÙÚÔÊ›·ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ, Ù‡Ô˘ ππ, ÛÙËÓÔÔ›· ˘¿Ú¯ÂÈ ËÈfiÙÂÚË ·fiÊÚ·ÍË Ù˘ ·ÓÒÙÂÚ˘·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡ ÛÂ Û˘Ó‰˘·ÛÌfi Ì ·¯˘Û·ÚΛ·,Î·È Ù‡Ô˘ πππ, ÛÙËÓ ÔÔ›· ÙÔ ™∞∞À ÔÊ›ÏÂÙ·È Û ÎÚ·-ÓÈÔÚÔÛˆÈΤ˜ ·ÓˆÌ·Ï›Â˜ ‹ Ó¢ÚÔÌ˘˚Τ˜ ‰È·Ù·Ú·-¯¤˜ (Û‡Ó‰ÚÔÌ· Crouzon, Apert, Teacher Collins,Pierre Robin, Down, ·¯ÔÓ‰ÚÔÏ·Û›·, ÌËÓÈÁÁÔÌ˘ÂÏÔ-΋ÏË, ÂÁÎÂÊ·ÏÈ΋ ·Ú¿Ï˘ÛË) (12).

√È ·ÛıÂÓ›˜ Ì ™∞∞À Ô˘ ‰ÂÓ ·ÓÙÈÌÂÙˆ›˙ÔÓÙ·È

¤ÁηÈÚ· ¤¯Ô˘Ó ·˘ÍË̤ÓÔ Î›Ó‰˘ÓÔ ÂÌÊ¿ÓÈÛ˘ ÛÔ‚·-

ÚÒÓ ¯ÚfiÓÈˆÓ Î·Ú‰È·ÁÁÂÈ·ÎÒÓ Î·È ÌÂÙ·‚ÔÏÈÎÒÓ ‰È·-

Ù·Ú·¯ÒÓ. ∞˘Ù¤˜ Û˘Ó›ÛÙ·ÓÙ·È Û ˘„ËÏfiÙÂÚ· ›‰·

·ÚÙËÚȷ΋˜ ›ÂÛ˘, ·‡ÍËÛË Ù˘ C ·ÓÙȉÚÒÛ·˜ Úˆ-

Ù½Ó˘ (Ë ÔÔ›· Û˘Û¯ÂÙ›˙ÂÙ·È Ì ηډȷÁÁÂȷΤ˜ Ófi-

ÛÔ˘˜), ·ÓÙ›ÛÙ·ÛË ÛÙËÓ ÈÓÛÔ˘Ï›ÓË Î·È ˘ÂÚÙÚÔÊ›·

Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ (13).

ŸÛÔÓ ·ÊÔÚ¿ ÛÙÔÓ Ù‡Ô π (ÌÂÌÔӈ̤ÓË ˘ÂÚÙÚÔ-

Ê›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ), ¤¯ÂÈ ‰È·ÈÛÙˆ-

ı› fiÙÈ Ë ¯ÚfiÓÈ· ‰È·ÏÂ›Ô˘Û· ˘ÔÍ·ÈÌ›· Î·È ÙÔ ÔÍÂÈ-

‰ˆÙÈÎfi stress Ô‰ËÁÔ‡Ó ÛÙËÓ ·Ú·ÁˆÁ‹ ‰È·ÊfiÚˆÓ

ÊÏÂÁÌÔÓˆ‰ÒÓ Î˘ÙÔÎÈÓÒÓ Î·È Û ‰˘ÛÏÂÈÙÔ˘ÚÁ›· ÙÔ˘

ÂÓ‰ÔıËÏ›Ô˘ ÙˆÓ ·ÁÁ›ˆÓ (13). ∏ Û˘Ó‡·ÚÍË ÙÔ˘

™∞∞À Î·È Ù˘ ·¯˘Û·ÚΛ·˜ ÛÙÔÓ Ù‡Ô ππ ÂȉÂÈÓÒ-

ÓÂÈ ÙÔ ÔÍÂȉˆÙÈÎfi stress Î·È ÙË Û˘ÛÙËÌ·ÙÈ΋ ÊÏÂÁÌÔ-

ÓÒ‰Ë ·ÓÙ›‰Ú·ÛË Î·È ÂÈÙ·¯‡ÓÂÈ ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘

·ÓÙ›ÛÙ·Û˘ ÛÙËÓ ÈÓÛÔ˘Ï›ÓË Î·È, ηٿ Û˘Ó¤ÂÈ·, ÙÔ˘

ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ (14).

∞ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÙÒÛÂȘ ÙÔ˘ ™∞∞À

∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘, Ù· ·ÓÂÈÏËÌ̤ӷ

ÂÂÈÛfi‰È· ÌÂÚÈ΋˜ ‹ Ï‹ÚÔ˘˜ ·fiÊڷ͢ Ù˘ ·ÓÒ-

ÙÂÚ˘ ·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡ ÚÔηÏÔ‡Ó ˘Ô·ÂÚÈÛÌfi

(˘fiÓÔÈ·) ‹ ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘ ¿ÓÔȘ. √È ÙÂ-

ÏÂ˘Ù·›Â˜ ÚÔηÏÔ‡Ó ÂÂÈÛfi‰È· ˘Ô͢ÁÔÓ·ÈÌ›·˜, ÌÂ

Û˘ÓÔ‰fi ˘ÂÚηӛ· Î·È ·Ó·Ó¢ÛÙÈ΋ ÔͤˆÛË (15).

1) √Í›˜ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÙÒÛÂȘ: √È ·ÔÊÚ·-

ÎÙÈΤ˜ ¿ÓÔȘ ÚÔηÏÔ‡Ó Â·Ó·Ï·Ì‚·ÓfiÌÂÓ·

ÂÂÈÛfi‰È· ÙÒÛ˘ Ù˘ ÂÓ‰ÔıˆÚ·ÎÈ΋˜ ›ÂÛ˘, Ô˘

¤¯Ô˘Ó ˆ˜ ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·‡ÍËÛË ÙÔ˘ ÌÂÙ·ÊÔÚÙ›Ô˘

Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜, ÂÏ¿ÙÙˆÛË ÙÔ˘ fiÁÎÔ˘ ·ÏÌÔ‡

Î·È Ù˘ ηډȷ΋˜ ·ÚÔ¯‹˜. ∆·˘Ùfi¯ÚÔÓ· ·˘Í¿ÓÂÙ·È

Ë ÊÏ‚È΋ ÂÈÛÙÚÔÊ‹ ·›Ì·ÙÔ˜, ÏfiÁˆ ÂÏ¿ÙÙˆÛ˘ Ù˘

ÎÂÓÙÚÈ΋˜ ÊÏ‚È΋˜ ›ÂÛ˘, Ì ·ÎfiÏÔ˘ıÔ ÙËÓ ·‡-

ÍËÛË ÙÔ˘ ÙÂÏԉȷÛÙÔÏÈÎÔ‡ fiÁÎÔ˘ Ù˘ ‰ÂÍÈ¿˜ ÎÔÈ-

Ï›·˜. ªÂ ÙËÓ ·Ê‡ÓÈÛË ‰È·ÎfiÙÂÙ·È Ë ¿ÓÔÈ· ηÈ

·˘Í¿ÓÂÙ·È Ë Û˘ÛÙËÌ·ÙÈ΋ ·ÚÙËÚȷ΋ ›ÂÛË Î·È Ë

ηډȷ΋ ·ÚÔ¯‹ (15).

2) ÃÚfiÓȘ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÙÒÛÂȘ: ∏ ¯ÚfiÓÈ·

˘ÔÍ·ÈÌ›· ÚÔηÏ› ·ÁÁÂÈÔÛ‡Û·ÛË ÙˆÓ ÎÏ¿‰ˆÓ

Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜. ∆· ·ÓÂÈÏËÌ̤ӷ ÂÂÈ-

Ûfi‰È· ·ÁÁÂÈÔÛ‡Û·Û˘ ηٷÛÙÚ¤ÊÔ˘Ó ÙÔ Ó¢ÌÔÓÈ-

Îfi ·ÁÁÂÈ·Îfi ÂÓ‰Ôı‹ÏÈÔ, Ì ·ÔÙ¤ÏÂÛÌ· ÙËÓ ·Ó¿Ù˘-

ÍË Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ Î·È ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ-

΋˜ ηډȿ˜ (15).

∫ÏÈÓÈ΋ Û˘Ìو̷ÙÔÏÔÁ›· ÙÔ˘ ™∞∞À

™Â ·Ú¯ÈÎfi ÛÙ¿‰ÈÔ ·Ú·ÙËÚÂ›Ù·È ·‰ÂÓÔÂȉ¤˜ ÚÔ-

ۈ›Ô, ÛÙÔÌ·ÙÈ΋ ·Ó·ÓÔ‹ Î·È ÚÔ¯·ÏËÙfi ÛÙÔÓ

‡ÓÔ. ™Â ÈÔ ÚÔ¯ˆÚË̤ÓÔ ÛÙ¿‰ÈÔ ÂÌÊ·Ó›˙ÔÓÙ·È

·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘ ¿ÓÔȘ ÛÙÔÓ ‡ÓÔ, ‰È·Ù·Ú·¯¤˜

‡ÓÔ˘ (Û˘¯Ó¤˜ ·Ê˘Ó›ÛÂȘ, Ó˘¯ÙÂÚÈÓÔ› ȉÚÒÙ˜), Ó˘-

¯ÙÂÚÈÓ‹ ÂÓÔ‡ÚËÛË, ÎÂÊ·Ï·ÏÁ›· ηٿ ÙËÓ ·Ê‡ÓÈÛË,

133∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ

¶·È‰È·ÙÚÈ΋ 2009;72:133-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·133

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ËÌÂÚ‹ÛÈ· ˘ÓËÏ›· Î·È Ì·ıËÛȷΤ˜ ‰È·Ù·Ú·¯¤˜. ™Â

ÙÂÏÈÎfi ÛÙ¿‰ÈÔ ·Ú·ÙËÚÂ›Ù·È ˘ÔıÚ„›·, ·Ó·Ó¢-

ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, ΢¿ÓˆÛË Î·È Û˘ÌÙÒÌ·Ù· ‰ÂÍÈ¿˜

‹ ·ÚÈÛÙÂÚ‹˜ ηډȷ΋˜ ·Ó¿ÚÎÂÈ·˜ (2,3).

™ËÌ·ÓÙÈ΋ ËÌÂÚ‹ÛÈ· ˘ÓËÏ›· ‰ÂÓ ·Ú·ÙËÚ›ٷÈ

Û˘¯Ó¿ Û ·È‰È¿ Ô˘ ¤¯Ô˘Ó ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘

¿ÓÔȘ (16). ¶·ÚfiÏ· ·˘Ù¿, ·ÎfiÌË Î·È ·È‰È¿ ÌÂ

·Ïfi ÚÔ¯·ÏËÙfi ÌÔÚ› Ó· ¤¯Ô˘Ó ‰È·Ù·Ú·¯¤˜ Û˘ÌÂ-

ÚÈÊÔÚ¿˜ Î·È Ì·ıËÛȷΤ˜ ‰È·Ù·Ú·¯¤˜ (17,18), ÔÈ

Ôԛ˜ Â›Ó·È ÌÂÚÈÎÒ˜ ·Ó·ÛÙÚ¤„È̘, Ì ÙËÓ ¿ÚÛË

Ù˘ ·fiÊڷ͢ (19,20).

ŸÛÔÓ ·ÊÔÚ¿ ÛÙË ÌÂȈ̤ÓË ÛˆÌ·ÙÈ΋ ·Ó¿Ù˘ÍË,

Û‡Ìʈӷ Ì ÙÔ˘˜ Marcus et al. (21) ·˘Ù‹ ÔÊ›ÏÂÙ·È

ÛÙËÓ ÂÚÁÒ‰Ë ·Ó·ÓÔ‹, Ë ÔÔ›· Ô‰ËÁ› Û ·˘ÍË̤Ó˜

ÌÂÙ·‚ÔÏÈΤ˜ ·Ó¿ÁΘ, ÂÓÒ Û ÌÈ· ¿ÏÏË ÌÂϤÙË ıˆ-

Ú‹ıËΠˆ˜ Èı·Ófi˜ ÔÚÌÔÓÈÎfi˜ Ì˯·ÓÈÛÌfi˜ (ÂÏ¿ÙÙˆ-

ÛË ÙÔ˘ ·Ú¿ÁÔÓÙ· IGF-I) (22). ∞Ó Î·È Ë ÌÂȈ̤ÓË

ۈ̷ÙÈ΋ ·Ó¿Ù˘ÍË ‰ÂÓ Â›Ó·È Û˘¯Ó‹ Ù· ÙÂÏÂ˘Ù·›·

¯ÚfiÓÈ·, Ù· ·È‰È¿ Ô˘ οÓÔ˘Ó ·‰ÂÓÔÂȉÂÎÙÔÌ‹ ηÈ

·Ì˘Á‰·ÏÂÎÙÔÌ‹ ÏfiÁˆ ™∞∞À ÂÌÊ·Ó›˙Ô˘Ó ·‡ÍËÛË

ÙÔ˘ ۈ̷ÙÈÎÔ‡ ÙÔ˘˜ ‚¿ÚÔ˘˜ ÌÂÙ¿ ·fi ·˘Ù‹Ó

(21,22).

∏ ¯ÚfiÓÈ· Ó¢ÌÔÓÈ΋ ηډȿ (cor pulmonale) ÌÂ

Û˘ÓÔ‰fi ηډȷ΋ ·Ó¿ÚÎÂÈ· Â›Ó·È Û¿ÓÈ· Ù· ÙÂ-

ÏÂ˘Ù·›· ¯ÚfiÓÈ·. ¶·ÚfiÏ· ·˘Ù¿, Û˘¯Ó¿ ˘¿Ú¯ÂÈ ·Û˘-

Ìو̷ÙÈ΋ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË (3).

¢È¿ÁÓˆÛË ÙÔ˘ ™∞∞À

∂ÎÙfi˜ ·fi ÙÔ ÈÛÙÔÚÈÎfi Î·È ÙËÓ ÎÏÈÓÈ΋ ÂͤٷÛË, ÔÈ

‰È·ÁÓˆÛÙÈΤ˜ ̤ıÔ‰ÔÈ Ô˘ ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó

ÛÙË ‰È¿ÁÓˆÛË Â›Ó·È Ë Ô͢ÌÂÙÚ›· Î·È Ë ÔÏ˘Î·Ù·ÁÚ·-

ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘. ™Ùfi¯Ô˜ Ù˘ ‰È¿ÁÓˆÛ˘ Ù˘ ‡·Ú-

͢ ™∞∞À Â›Ó·È Ë ·Ó›¯Ó¢ÛË ·ÛıÂÓÒÓ ˘„ËÏÔ‡ ÎÈÓ‰‡-

ÓÔ˘ ÁÈ· ÙËÓ ÂΉ‹ÏˆÛË ÛÔ‚·ÚÒÓ ÂÈÏÔÎÒÓ.

øÛÙfiÛÔ ÙÔ ÈÛÙÔÚÈÎfi ‰ÂÓ ÌÔÚ› Ó· ‰ÒÛÂÈ ·ÍÈfiÈ-

ÛÙ˜ ÏËÚÔÊÔڛ˜ ÁÈ· ÙËÓ ‡·ÚÍË ‹ fi¯È ·ÔÊÚ·ÎÙÈ-

ÎÔ‡ Ù‡Ô˘ ·ÓÔÈÒÓ, ÁÈ· ÙÔ˘˜ ÂÍ‹˜ ÏfiÁÔ˘˜: ÚÒÙÔÓ, Ë

¤ÓÙ·ÛË ÙÔ˘ ÚÔ¯·ÏËÙÔ‡ ‰ÂÓ Û¯ÂÙ›˙ÂÙ·È Ì ÙËÓ ‡·ÚÍË

·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡Ô˘ ·ÓÔÈÒÓ. ∂›Û˘, ÔÈ ÔÏ˘Î·Ù·-

ÁÚ·ÊÈΤ˜ ÌÂϤÙ˜ ‡ÓÔ˘ ¤¯Ô˘Ó ‰Â›ÍÂÈ fiÙÈ ÔÈ ¿ÓÔȘ

·Ú·ÙËÚÔ‡ÓÙ·È Î·Ù¿ ÙȘ ÚÒÙ˜ ÚˆÈÓ¤˜ ÒÚ˜, ÛÙË

Ê¿ÛË REM ÙÔ˘ ‡ÓÔ˘, fiÔ˘ ÔÈ ÁÔÓ›˜ ÎÔÈÌÔ‡ÓÙ·È.

∞ÎfiÌË, ÌÂÚÈο ·È‰È¿ ¤¯Ô˘Ó ÌÈ· ÌfiÓÈÌË ·fiÊÚ·ÍË

Ù˘ ·ÓÒÙÂÚ˘ ·Ó·Ó¢ÛÙÈ΋˜ Ô‰Ô‡, Ô˘ Û˘Óԉ‡ÂÙ·È

·fi ‰È·Ù·Ú·¯‹ Ù˘ ·ÓÙ·ÏÏ·Á‹˜ ·ÂÚ›ˆÓ Î·È ‰ÂÓ ÂÌÊ·-

Ó›˙Ô˘Ó ÙȘ Ù˘ÈΤ˜ ΢ÎÏÈÎÔ‡ Ù‡Ô˘ ¿ÓÔȘ, ÒÛÙ ӷ

Á›ÓÔ˘Ó ·ÓÙÈÏËÙ¤˜ ·fi ÙÔ˘˜ ÁÔÓ›˜ (2).

∏ ÔÏÔÓ‡ÎÙÈ· ηٷÁÚ·Ê‹ Ù˘ Ô͢ÌÂÙÚ›·˜ ÌÔÚ›

›Û˘ Ó· ‰Â›ÍÂÈ ÂÂÈÛfi‰È· ΢ÎÏÈ΋˜ ÙÒÛ˘ ÙÔ˘

ÎÔÚÂÛÌÔ‡, ·ÏÏ¿ ÌÔÚ› Ó· ¤¯ÂÈ „¢‰Ò˜ ıÂÙÈο ·Ô-

ÙÂϤÛÌ·Ù· Û ·È‰È¿ Ì ·¯˘Û·ÚΛ· ‹ ¿ÛıÌ·. ∏ Ìfi-

ÓË Û›ÁÔ˘ÚË ‰È·ÁÓˆÛÙÈ΋ ̤ıÔ‰Ô˜, ÏÔÈfiÓ, Â›Ó·È Ë

ÔÏ˘Î·Ù·ÁÚ·ÊÈ΋ ÌÂϤÙË ‡ÓÔ˘, Ë ÔÔ›· ÌÔÚ› Ó·

‰È·ÎÚ›ÓÂÈ ÙÔ ·Ïfi ÚÔ¯·ÏËÙfi ·fi ÙÔ Û‡Ó‰ÚÔÌÔ ·Ô-

ÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ (2). ø˜ ÚÔ˜ ÙÔ fiÙ ڤ-

ÂÈ Ó· ıÂڷ‡ÂÙ·È ¤Ó· ·È‰› Ì ۇӉÚÔÌÔ Ù˘ ·Ô-

ÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜, ·˘Ùfi Ú¤ÂÈ Ó· Á›ÓÂÙ·È ÔˆÛ-

‰‹ÔÙ fiÙ·Ó ˘¿Ú¯Ô˘Ó ¿Óˆ ·fi 10 ·ÔÊÚ·ÎÙÈο

·ÓÔ˚ο ÂÂÈÛfi‰È· ·Ó¿ ÒÚ· (23).

∫ÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÙÔ˘ ™∞∞À ÛÙÔ Î·Ú‰È·ÁÁÂÈ·Îfi

Û‡ÛÙËÌ·

√È ÎÏÈÓÈΤ˜ ÂΉËÏÒÛÂȘ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó ÙËÓ ÂÎ-

‰‹ÏˆÛË ˘ÂÚÙ·ÛÈÎÒÓ ÎÚ›ÛÂˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ·

·ÓÔÈÒÓ, ‰È·Ù·Ú·¯ÒÓ ÙÔ˘ ηډȷÎÔ‡ Ú˘ıÌÔ‡, Ó¢-

ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘, ¯ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜

Î·È Û˘ÌÊÔÚËÙÈ΋˜ ηډȷ΋˜ ·Ó¿ÚÎÂÈ·˜.

™∞∞À Î·È ¤ÚÙ·ÛË

∏ ·fiÊÚ·ÍË Ô‰ËÁ› Û ·ӷϷ̂·ÓfiÌÂÓ· ÂÂÈ-

Ûfi‰È· ˘ÂÚÙ·ÛÈÎÒÓ ÎÚ›ÛÂˆÓ Î·Ù¿ ÙÔÓ ‡ÓÔ, Ô˘

Û˘Ó‰˘¿˙ÔÓÙ·È Ì ٷ ·ÓÔ˚ο ÂÂÈÛfi‰È·. ∂›Û˘

˘¿Ú¯ÂÈ ·˘ÍË̤ÓÔ˜ ΛӉ˘ÓÔ˜ Û˘ÛÙËÌ·ÙÈ΋˜ ˘¤Ú-

Ù·Û˘ ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (24). √È ·ıÔÁÂÓÂÙÈÎÔ› ÌË-

¯·ÓÈÛÌÔ› Ô˘ Èı·ÓÔÏÔÁÔ‡ÓÙ·È Â›Ó·È: ·) ·˘ÍË̤ÓË

‰Ú·ÛÙËÚÈfiÙËÙ· ÙÔ˘ Û˘Ì·ıËÙÈÎÔ‡ Ó¢ÚÈÎÔ‡ Û˘ÛÙ‹-

Ì·ÙÔ˜ (¡™), Ì ·ÔÙ¤ÏÂÛÌ· ·ÂÏ¢ı¤ÚˆÛË Î·Ù¯Ô-

Ï·ÌÈÓÒÓ (25), ‚) ·˘ÍË̤ÓË ·Ú·ÁˆÁ‹ ·ÁÁÂÈÔÛ˘Û·-

ÛÙÈÎÒÓ Ô˘ÛÈÒÓ ·fi ÙÔ ÂÓ‰Ôı‹ÏÈÔ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ

·ÁÁ›ˆÓ, fiˆ˜ Ù˘ ÂÓ‰ÔıËÏ›Ó˘ 1 (26), Î·È Á) ‰È¤-

ÁÂÚÛË ÙÔ˘ Û˘ÛÙ‹Ì·ÙÔ˜ ÚÂÓ›Ó˘-·ÁÁÂÈÔÙÂÓÛ›Ó˘-·Ï-

‰ÔÛÙÂÚfiÓ˘ ÏfiÁˆ ˘ÔÍ›·˜ (27).

∆Ô ™∞∞À ÛÙÔ˘˜ ÂÓ‹ÏÈΘ Û¯ÂÙ›˙ÂÙ·È Ì ·˘ÍË̤-

ÓË Èı·ÓfiÙËÙ· ÂÌÊ¿ÓÈÛ˘ Û˘ÛÙËÌ·ÙÈ΋˜ ˘¤ÚÙ·Û˘

(28,29). §›Á˜ ·Ó¿ÏÔÁ˜ ÌÂϤÙ˜ ˘¿Ú¯Ô˘Ó Î·È ÛÙ·

·È‰È¿. √ Marcus et al. (30) ‰È·›ÛÙˆÛ·Ó ·‡ÍËÛË

Ù˘ ‰È·ÛÙÔÏÈ΋˜ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ ηٿ ÙË ‰È¿Ú-

ÎÂÈ· ÙÔ˘ ‡ÓÔ˘, Ë ÔÔ›· ¤ÌÂÓÂ Î·È ÌÂÙ¿ ÙËÓ ¤ÁÂÚ-

ÛË. √È Amin et al. (31) ‚Ú‹Î·Ó ÛÙ·ÙÈÛÙÈο ÛËÌ·ÓÙÈο

·˘ÍË̤ÓË ‰È·Î‡Ì·ÓÛË Ù˘ ·ÚÙËÚȷ΋˜ ›ÂÛ˘ (∞¶)

ηٿ ÙËÓ ÂÁÚ‹ÁÔÚÛË Î·È ÙÔÓ ‡ÓÔ, ˘„ËÏfiÙÂÚË Û¯¤-

ÛË Ó˘¯ÙÂÚÈÓ‹˜/ËÌÂÚ‹ÛÈ·˜ ∞¶ Î·È ÌÈÎÚfiÙÂÚË ÙÒÛË

Ù˘ ̤Û˘ ∞¶ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡¯Ù·˜. ∂›Û˘,

Û ¿ÏÏË ÌÂϤÙË ÙˆÓ Amin et al. (32) Ê¿ÓËÎ·Ó ÌÂÙ·-

‚ÔϤ˜ ÙÔ˘ ¿¯Ô˘˜ ÙÔ˘ ÙÔȯÒÌ·ÙÔ˜ Ù˘ ·ÚÈÛÙÂÚ‹˜

ÎÔÈÏ›·˜ Ô˘ Â›Ó·È ÂÓ‰ÂÈÎÙÈΤ˜ ·˘ÍËÌ¤ÓˆÓ ÙÈÌÒÓ Û˘-

ÛÙÔÏÈ΋˜ ·ÚÙËÚȷ΋˜ ›ÂÛ˘. ∞Ó Î·È ¯ÚÂÈ¿˙ÔÓÙ·È

ÂÚÈÛÛfiÙÂÚ˜ ÌÂϤÙ˜ Û ·È‰È¿, Â›Ó·È ÚÔÊ·Ó¤˜ fiÙÈ

ÔÈ ÌÂÙ·‚ÔϤ˜ ÙÔ˘ ÙfiÓÔ˘ ÙÔ˘ ·˘ÙfiÓÔÌÔ˘ ¡™ Ô˘ ·-

Ú·ÙËÚÔ‡ÓÙ·È Û ·ÛıÂÓ›˜ Ì ™∞∞À Úԉȷı¤ÙÔ˘Ó ÛÂ

ÚÒÈÌË ÂÌÊ¿ÓÈÛË ˘¤ÚÙ·Û˘ (8).

™∞∞À Î·È ‰È·Ù·Ú·¯¤˜ ÙÔ˘ Ú˘ıÌÔ‡

™Â ¿ÙÔÌ· Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ·ÔÊÚ·ÎÙÈÎÔ‡ Ù‡-

Ô˘ ¿ÓÔȘ ·Ú·ÙËÚÂ›Ù·È ÂȉÂÈÓÔ‡ÌÂÓË ‚Ú·‰˘-

ηډ›· ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·ÓÔ˚ÎÔ‡ ÂÂÈÛÔ‰›Ô˘,

134 ∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘

Paediatriki 2009;72:134-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·134

Page 63: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

Ô˘ ·ÎÔÏÔ˘ıÂ›Ù·È ·fi Ù·¯˘Î·Ú‰›·. ∂ÈÚfiÛıÂÙ·,

ÌÔÚ› Ó· ·Ú·ÙËÚËıÔ‡Ó ¤ÎÙ·ÎÙ˜ ÎÔÈÏȷΤ˜ Û˘-

ÛÙÔϤ˜, ηډȷΤ˜ ·‡ÛÂȘ >3 sec Î·È 2Ô˘ ‚·ıÌÔ‡

ÎÔÏÔÎÔÈÏÈ·Îfi˜ ·ÔÎÏÂÈÛÌfi˜ Ù‡Ô˘ Mobitz II (33).

ø˜ ·ıÔÁÂÓÂÙÈÎÔ› Ì˯·ÓÈÛÌÔ› ıˆÚÔ‡ÓÙ·È: ·) ÔÈ

·˘ÍË̤Ó˜ ·Ó¿ÁΘ Û Ô͢ÁfiÓÔ Ô˘ ·Ú·ÙËÚÔ‡ÓÙ·È

ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ·ÓÔ˚ÎÔ‡ ÂÂÈÛÔ‰›Ô˘ Î·È Ô‰Ë-

ÁÔ‡Ó Û ÈÛ¯·ÈÌ›· ÙÔ˘ Ì˘Ôηډ›Ô˘ (34) Î·È ‚) Ë ¯ÚfiÓÈ·

˘ÔÍ·ÈÌ›· Î·È Ë ˘ÂÚηӛ·, ÔÈ Ôԛ˜ ÚÔηÏÔ‡Ó

‰È¤ÁÂÚÛË ÙÔ˘ Û˘Ì·ıËÙÈÎÔ‡ ¡™ Î·È ·ÂÏ¢ı¤ÚˆÛË

ηÙ¯ÔÏ·ÌÈÓÒÓ Ô˘ ¤¯Ô˘Ó ·ÚÚ˘ıÌÈÔÁfiÓÔ ‰Ú¿ÛË (35).

¶Ó¢ÌÔÓÈ΋ ¤ÚÙ·ÛË - ÚfiÓÈ· Ó¢ÌÔÓÈ΋

ηډȿ (cor pulmonale)

∆Ô ‚·ÛÈÎfi ¯·Ú·ÎÙËÚÈÛÙÈÎfi Ù˘ Ó¢ÌÔÓÈ΋˜

ηډȿ˜ Â›Ó·È Ë Ó¢ÌÔÓÈ΋ ¤ÚÙ·ÛË, Ë ÔÔ›· ›ӷÈ

˘Â‡ı˘ÓË ÁÈ· ÙËÓ ÂÌÊ¿ÓÈÛË Ù˘ ˘ÂÚÙÚÔÊ›·˜ Ù˘

‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È ÙËÓ Î·Ú‰È·Î‹ ·Ó¿ÚÎÂÈ· Ù˘ ‰Â-

ÍÈ¿˜ (36). ø˜ Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË ¯·Ú·ÎÙËÚ›˙ÂÙ·È

Ë ·‡ÍËÛË Ù˘ ̤Û˘ ›ÂÛ˘ ÛÙËÓ Ó¢ÌÔÓÈ΋ ·ÚÙË-

Ú›· >25 mm Hg Û ËÚÂÌ›· Î·È >30 mm Hg ηٿ ÙËÓ

¿ÛÎËÛË (37).

∞ÈÙÈÔÏÔÁ›· Ó¢ÌÔÓÈ΋˜ ¤ÚÙ·Û˘

√È ·ÈÙÈÔÏÔÁÈÎÔ› ·Ú¿ÁÔÓÙ˜ Ô˘ Ô‰ËÁÔ‡Ó ·fi ÙË

¯ÚfiÓÈ· ·fiÊÚ·ÍË ÛÙËÓ ÂΉ‹ÏˆÛË Ó¢ÌÔÓÈ΋˜

˘¤ÚÙ·Û˘ ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ‰È¢ÎÚÈÓÈṲ̂ÓÔÈ. ∏

¯ÚfiÓÈ· ·fiÊÚ·ÍË Î·È Ë Û˘ÓÔ‰fi˜ ˘ÔÍ·ÈÌ›·, ˘ÂÚη-

Ó›· Î·È ÔͤˆÛË Ô‰ËÁÔ‡Ó Û Ó¢ÌÔÓÈ΋ ·ÁÁÂÈÔÛ‡-

Û·ÛË (ÔÍ›· ‹ ¯ÚfiÓÈ·) (1). ∏ ÔÍ›· Á›ÓÂÙ·È Ì¤Û· ÛÂ

Ï›Á· ÏÂÙ¿ Î·È Â›Ó·È ·Ó·ÛÙÚ¤„ÈÌË Ì ÙËÓ ¿ÚÛË Ù˘

·fiÊڷ͢. ∏ ¯ÚfiÓÈ· ÔÊ›ÏÂÙ·È Û ‰ÔÌÈΤ˜ ·ÓˆÌ·-

ϛ˜ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ (˘ÂÚÙÚÔÊ›· ÙÔ˘ Ì˘-

˚ÎÔ‡ ¯ÈÙÒÓ· ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÚÙËÚÈÒÓ Ì¤ÛÔ˘ ηÈ

ÌÈÎÚÔ‡ ÌÂÁ¤ıÔ˘˜). ªÂ ÙËÓ ¿ÚÛË Ù˘ ·fiÊڷ͢, ÔÈ

ÌÂÙ·‚ÔϤ˜ ·˘Ù¤˜ ‰ÂÓ Â›Ó·È Ï‹Úˆ˜ ·Ó·ÛÙÚ¤„È̘

(1). ∏ Ó¢ÌÔÓÈ΋ ·ÁÁÂÈÔÛ‡Û·ÛË Ô‰ËÁ› Û ·‡ÍËÛË

ÙˆÓ Ó¢ÌÔÓÈÎÒÓ ·ÁÁÂÈ·ÎÒÓ ·ÓÙÈÛÙ¿ÛÂˆÓ Î·È ·‡ÍË-

ÛË ÙÔ˘ ηډȷÎÔ‡ ÌÂÙ·ÊÔÚÙ›Ô˘ Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜.

∏ ÙÂÏÂ˘Ù·›· Ô‰ËÁ› Ì ÙËÓ ¿ÚÔ‰Ô ÙÔ˘ ¯ÚfiÓÔ˘ ÛÂ

˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È Û ‰ÂÍÈ¿ ηډȷ-

΋ ·Ó¿ÚÎÂÈ· (1). ™ÙË Û˘Ó¤¯ÂÈ·, Â¿Ó ‰ÂÓ ·ÓÙÈÌÂÙˆ-

ÈÛÙ› Ë Î·Ù¿ÛÙ·ÛË, ÌÔÚ› Ó· ·ÎÔÏÔ˘ı‹ÛÂÈ ‰˘ÛÏÂÈ-

ÙÔ˘ÚÁ›· Î·È Ù˘ ·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜, Ì ÂÏ¿ÙÙˆÛË ÙÔ˘

fiÁÎÔ˘ ·ÏÌÔ‡ Î·È Û˘ÌÊÔÚËÙÈ΋ ηډȷ΋ ·Ó¿Ú-

ÎÂÈ·. √ Û˘Ó‰˘·ÛÌfi˜ Ù˘ ‰˘ÛÏÂÈÙÔ˘ÚÁ›·˜ Ù˘ ·ÚÈÛÙÂ-

Ú‹˜ Î·È ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ ÌÔÚ› Ó· ÂÈ‚·Ú‡ÓÂÈ ÙËÓ

Ó¢ÌÔÓÈ΋ ÏÂÈÙÔ˘ÚÁ›· Î·È Èı·ÓfiÓ Ó· Ô‰ËÁ‹ÛÂÈ

ÛÙËÓ ÂΉ‹ÏˆÛË Ó¢ÌÔÓÈÎÔ‡ Ôȉ‹Ì·ÙÔ˜ (1).

¶ÚÔ˜ ÙÔ ·ÚfiÓ ‰ÂÓ ˘¿Ú¯Ô˘Ó ÌÂϤÙ˜ Ô˘ Ó·

·Ô‰ÂÈÎÓ‡Ô˘Ó fiÙÈ Û ·Ô˘Û›· ËÌÂÚ‹ÛÈ·˜ ˘ÔÍ·È-

Ì›·˜ ÌÔÚ› Ó· ÚÔÎÏËı› ÌË ·Ó·ÛÙÚ¤„ÈÌË Ó¢ÌÔ-

ÓÈ΋ ¤ÚÙ·ÛË. ∞ÓÙ›ıÂÙ·, Ë Û˘Ó‡·ÚÍË ÛÔ‚·Ú‹˜ Ó˘-

¯ÙÂÚÈÓ‹˜ ˘ÔÍ·ÈÌ›·˜ Ì ‹È· ¤ˆ˜ ̤ÙÚÈ· ËÌÂÚ‹ÛÈ·

˘ÔÍ·ÈÌ›· ‹/Î·È ˘ÂÚηӛ· ÌÔÚ› Ó· Ô‰ËÁ‹ÛÂÈ

ÛÙËÓ ÂÁηٿÛÙ·ÛË Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ (38).

™˘Ìو̷ÙÔÏÔÁ›· ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜

∆· Û˘ÌÙÒÌ·Ù· Ù˘ ÂÍÂÏÈÛÛfiÌÂÓ˘ Ó¢ÌÔÓÈ΋˜

˘¤ÚÙ·Û˘ ·Ú¯Èο Â›Ó·È ·Û‹Ì·ÓÙ·, ̤¯ÚÈ Ó· Û˘Ì‚Â›

ÛÔ‚·Ú‹ ηډȷ΋ ·ÔÚÚ‡ıÌÈÛË (39). ™ÙȘ Û¿ÓȘ

ÂÚÈÙÒÛÂȘ Ô˘ ¤¯ÂÈ Î·Ù·Ï‹ÍÂÈ Û ¯ÚfiÓÈ· Ó¢ÌÔ-

ÓÈ΋ ηډȿ, ÂÌÊ·Ó›˙ÂÙ·È ‰‡ÛÓÔÈ· (·fi ÂÏ¿ÙÙˆÛË

Ù˘ ‰È·Ù·ÛÈÌfiÙËÙ·˜ ÙˆÓ Ó¢ÌfiÓˆÓ), ‡ÎÔÏË Îfiˆ-

ÛË (·fi ÂÏ¿ÙÙˆÛË Ù˘ ηډȷ΋˜ ·ÚÔ¯‹˜), Û˘ÁÎÔ-

Èο ÂÂÈÛfi‰È· (·fi ·ÚÔ‰È΋ ÂÁÎÂÊ·ÏÈ΋ ÈÛ¯·È-

Ì›·), ÚÔοډȷ ¿ÏÁË ÛÙËı·Á¯ÈÎÔ‡ ¯·Ú·ÎÙ‹Ú· ηÈ

·›ÛıËÌ· ·ÏÌÒÓ. ∂›Û˘, ÙÔ Û‡Ó‰ÚÔÌÔ ·ÔÊÚ·ÎÙÈ-

΋˜ ¿ÓÔÈ·˜ ηٿ ÙÔÓ ‡ÓÔ Ú¤ÂÈ Ó· ÙÔ ˘Ô„È·ÛÙ›

ηÓ›˜ Û ·È‰› Ô˘ ·ÚÔ˘ÛÈ¿˙ÂÈ ·ÓÂÍ‹ÁËÙË Î·Ú‰È·-

΋ ·Ó¿ÚÎÂÈ· Î·È ¤¯ÂÈ ÛÔ‚·ÚÔ‡ ‚·ıÌÔ‡ ˘ÂÚÙÚÔ-

Ê›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ (36).

∫ÏÈÓÈο Â˘Ú‹Ì·Ù· ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜ ηډȿ˜

∫·Ù¿ ÙËÓ ·ÎÚfi·ÛË Ù˘ ηډȿ˜ ·Ú·ÙËÚ›ٷÈ

·˘ÍË̤ÓÔ ÙÔ Ó¢ÌÔÓÈÎfi ÛÙÔÈ¯Â›Ô ÙÔ˘ 2Ô˘ ÙfiÓÔ˘,

Û˘ÛÙÔÏÈÎfi ʇÛËÌ· ·Ó¿ÚÎÂÈ·˜ Ù˘ ÙÚÈÁÏÒ¯ÈÓ·˜ ‹

‰È·ÛÙÔÏÈÎfi ʇÛËÌ· ·Ó¿ÚÎÂÈ·˜ Ù˘ Ó¢ÌÔÓÈ΋˜,

3Ô˜ ‹ 4Ô˜ ÙfiÓÔ˜. ∂›Û˘, ÌÔÚ› Ó· ˘¿Ú¯ÂÈ ‰È¿Ù·ÛË

ÙˆÓ ÊÏ‚ÒÓ ÙÔ˘ ÙÚ·¯‹ÏÔ˘, Ë·ÙÔÌÂÁ·Ï›· Î·È ÂÚÈ-

ÊÂÚÈο Ôȉ‹Ì·Ù· (1).

∞ÎÙÈÓÔÏÔÁÈο Â˘Ú‹Ì·Ù· ÚfiÓÈ·˜ Ó¢ÌÔÓÈ΋˜

ηډȿ˜

™ÙËÓ ·ÎÙÈÓÔÁÚ·Ê›· ıÒÚ·ÎÔ˜ ·Ú·ÙËÚÂ›Ù·È ‰È¿-

Ù·ÛË Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È ÙÔ˘ ‰ÂÍÈÔ‡ ÎfiÏÔ˘, ‰È¿-

Ù·ÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜ Î·È Ó¢ÌÔÓÈ΋

Û˘ÌÊfiÚËÛË.

∏ÏÂÎÙÚÔηډÈÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ÚfiÓÈ·˜

Ó¢ÌÔÓÈ΋˜ ηډȿ˜

∂›Ó·È ÂÌÊ·Ó‹ Û ÚÔ¯ˆÚË̤ӷ ÛÙ¿‰È·, fiÔ˘

˘¿Ú¯ÂÈ ˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È Î·Ú‰È·-

΋ ·Ó¿ÚÎÂÈ· Ù˘ ‰ÂÍÈ¿˜. ¶·Ú·ÙËÚÔ‡ÓÙ·È ‰ÂÍÈ¿

ÛÙÚÔÊ‹ ÙÔ˘ ¿ÍÔÓ·, ˘„ËÏ¿ Ô͢ÎfiÚ˘Ê· P, ȉ›ˆ˜ ÛÙȘ

··ÁˆÁ¤˜ II, V1 Î·È V2 (·fi ÙËÓ ˘ÂÚÙÚÔÊ›· ÙÔ˘ ‰Â-

ÍÈÔ‡ ÎfiÏÔ˘), ˘„ËÏ¿ R ÛÙȘ ‰ÂÍȤ˜ ÚÔοډȘ ··-

ÁˆÁ¤˜ Î·È ‚·ıÈ¿ S ÛÙȘ ·ÚÈÛÙÂÚ¤˜ ÚÔοډȘ ··-

ÁˆÁ¤˜ (·fi ˘ÂÚÙÚÔÊ›· Ù˘ ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜). ªÔ-

Ú› ›Û˘ Ó· ·Ú·ÙËÚËıÔ‡Ó ‰˘ÛÚ˘ı̛˜.

∏¯ÔηډÈÔÁÚ·ÊÈο Â˘Ú‹Ì·Ù· ÚfiÓÈ·˜

Ó¢ÌÔÓÈ΋˜ ηډȿ˜

™ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜, ˘¿Ú¯ÂÈ ‰È¿Ù·ÛË

Î·È ˘ÂÚÙÚÔÊ›· ‰ÂÍÈ¿˜ ÎÔÈÏ›·˜ Î·È ‰ÂÍÈÔ‡ ÎfiÏÔ˘ ηÈ

‰È¿Ù·ÛË Ù˘ Ó¢ÌÔÓÈ΋˜ ·ÚÙËÚ›·˜. ªÂ ÙË ÌÂϤÙË

135∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ

¶·È‰È·ÙÚÈ΋ 2009;72:135-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·135

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Doppler ‰È·ÈÛÙÒÓÂÙ·È ·Ó¿ÚÎÂÈ· ÙÚÈÁÏÒ¯ÈÓ·˜ ηÈ

Ó¢ÌÔÓÈ΋˜ ‚·Ï‚›‰·˜ Î·È ÌÂÙÚ¿Ù·È Ë ›ÂÛË ÛÙËÓ

Ó¢ÌÔÓÈ΋ ·ÚÙËÚ›·, Ë ÔÔ›· Â›Ó·È ·˘ÍË̤ÓË (1).

™Â ÈÔ ÚÔ¯ˆÚË̤Ó˜ ηٷÛÙ¿ÛÂȘ ÌÔÚ› ·Îfi-

ÌË Ó· ·Ú·ÙËÚËı› ‰È·ÛÙÔÏÈ΋ ‰˘ÛÏÂÈÙÔ˘ÚÁ›· Ù˘

·ÚÈÛÙÂÚ‹˜ ÎÔÈÏ›·˜ ‹/Î·È ˘ÂÚÙÚÔÊ›· Ù˘ ·ÚÈÛÙÂÚ‹˜

ÎÔÈÏ›·˜ (40,41).

£Âڷ›· ÙÔ˘ ™∞∞À

∞‰ÂÓÔÂȉÂÎÙÔÌ‹ Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹

∏ ¯ÂÈÚÔ˘ÚÁÈ΋ ·Ê·›ÚÂÛË ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È

·Ì˘Á‰·ÏÒÓ ·Ú·Ì¤ÓÂÈ Ë ıÂڷ›· ÚÒÙ˘ ÂÎÏÔÁ‹˜

Î·È Â›Ó·È ·Ó·Áη›· ÛÙ· ·È‰È¿ Ô˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó

™∞∞À (8,42,43).

∆· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ Ô˘ ¿Û¯Ô˘Ó ·fi ™∞∞À

‚ÂÏÙÈÒÓÔÓÙ·È ÛËÌ·ÓÙÈο ÌÂÙ¿ ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔÌ‹

Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹ (3,44-47). ∂›Û˘, ÔÈ Sdralis ηÈ

Berkovitz ÌÂϤÙËÛ·Ó ·Û˘Ìو̷ÙÈο ·È‰È¿ ÌÂ

˘ÂÚÙÚÔÊ›· ÙˆÓ ·‰ÂÓÔÂȉÒÓ Î·È ·Ì˘Á‰·ÏÒÓ Î·È

‰È·›ÛÙˆÛ·Ó fiÙÈ ÔÏÏ¿ ·fi ·˘Ù¿ ·ÚÔ˘Û›·˙·Ó ΢-

„ÂÏȉÈÎfi ˘Ô·ÂÚÈÛÌfi, Ô˘ ‚ÂÏÙÈÒıËΠÌÂÙ¿ ÙË ¯ÂÈ-

ÚÔ˘ÚÁÈ΋ ¤̂·ÛË (48).

ŸÛÔÓ ·ÊÔÚ¿ ÛÙË ÌÂÌÔӈ̤ÓË ·‰ÂÓÔÂȉÂÎÙÔÌ‹,

οÔȘ ÌÂϤÙ˜ ¤‰ÂÈÍ·Ó fiÙÈ ‰ÂÓ Â›Ó·È ·ÔÙÂÏÂÛÌ·-

ÙÈ΋ (42,49), ÂÓÒ ÌÈ· ÌÂϤÙË Ô˘ Û˘ÁÎÚ›ÓÂÈ ÙËÓ ·Ì˘-

Á‰·ÏÂÎÙÔÌ‹-·‰ÂÓÔÂȉÂÎÙÔÌ‹ Ì ÙË ÌÂÌÔӈ̤ÓË

·‰ÂÓÔÂȉÂÎÙÔÌ‹ ‰ÂÓ ¤‰ÂÈÍ ÛËÌ·ÓÙÈΤ˜ ‰È·ÊÔÚ¤˜

·Ó¿ÌÂÛ· ÛÙȘ ‰‡Ô ÔÌ¿‰Â˜ (44).

∂›Û˘, ¿ÏÏÔ˜ ¤Ó·˜ ÛËÌ·ÓÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ Ù˘

·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ·˜ Ù˘ ¤̂·Û˘ Â›Ó·È Ë ‚·Ú‡-

ÙËÙ· Ù˘ ·fiÊڷ͢. ªÈ· ÌÂϤÙË Ô˘ Û˘ÁÎÚ›ÓÂÈ ÙËÓ

·ÔÙÂÏÂÛÌ·ÙÈÎfiÙËÙ· Ù˘ ¤̂·Û˘ Ì ÙË ‚·Ú‡ÙË-

Ù· Ù˘ ·fiÊڷ͢ ¤‰ÂÈÍ fiÙÈ Ù· ·È‰È¿ Ô˘ ›¯·Ó

ÛÔ‚·Ú‹ ·fiÊÚ·ÍË Û˘Ó¤¯ÈÛ·Ó Ó· ¤¯Ô˘Ó ‰È·Ù·Ú·¯¤˜

‡ÓÔ˘ Î·È ÌÂÙ¿ ÙËÓ Â¤Ì‚·ÛË (50). ÕÏÏÔÈ ·Ú¿ÁÔ-

ÓÙ˜ Ô˘ Úԉȷı¤ÙÔ˘Ó Û ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À ÌÂ-

Ù¿ ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔÌ‹ Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹ Â›Ó·È Ë

Û˘Ó‡·ÚÍË ·¯˘Û·ÚΛ·˜ Î·È ÙÔ ıÂÙÈÎfi ÔÈÎÔÁÂÓÂÈ·Îfi

ÈÛÙÔÚÈÎfi (8,51).

√È ·ÛıÂÓ›˜ Ô˘ ¤¯Ô˘Ó ÛÔ‚·Ú‹ ·fiÊÚ·ÍË ÌÔ-

Ú› Ó· ¤¯Ô˘Ó Ó¢ÌÔÓÈ΋ ˘¤ÚÙ·ÛË ‹ Î·È Î·Ú‰È·Î‹

‰˘ÛÏÂÈÙÔ˘ÚÁ›·, ÁÈ’ ·˘Ùfi Î·È Â›Ó·È Èı·Ófi Ó· ¤¯Ô˘Ó

ÂÈÏÔΤ˜ ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË. √È ÂÈ-

ÏÔΤ˜ ÌÔÚ› Ó· Â›Ó·È ¿ÌÂÛ˜, fiˆ˜ Âȉ›ӈÛË

ÙÔ˘ Û˘Ó‰ÚfiÌÔ˘ ·ÔÊÚ·ÎÙÈ΋˜ ¿ÓÔÈ·˜ ‡ÓÔ˘ ‹

Ó¢ÌÔÓÈÎfi Ô›‰ËÌ·, Î·È ·ÒÙÂÚ˜, fiˆ˜ ·Ú·ÌÔÓ‹

·ÚÔ‰ÈÎÔ‡ ΢„ÂÏȉÈÎÔ‡ ˘Ô·ÂÚÈÛÌÔ‡ (Ô˘ ÌÔÚ›

Ó· ¯ÚÂÈ·Ûı› ̤¯ÚÈ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ‹ ÙÚ·¯ÂÈÔÛÙÔ-

Ì›·) ‹ ›ÌÔÓË ˘ÔÍ·ÈÌ›· Î·È ‰È·Ù·Ú·¯¤˜ ‡ÓÔ˘

(1,52). °È· ÙÔ˘˜ ·ÓˆÙ¤Úˆ ÏfiÁÔ˘˜, Û˘ÓÈÛÙ¿Ù·È Î·Ú-

‰ÈÔÏÔÁÈ΋ ÂÎÙ›ÌËÛË Ì ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ηډȿ˜

ÚÔÂÁ¯ÂÈÚËÙÈο Î·È ÂÈÛٷ̤ÓË ÌÂÙÂÁ¯ÂÈÚËÙÈ΋ ·-

Ú·ÎÔÏÔ‡ıËÛË Û ·˘ÙÔ‡˜ ÙÔ˘˜ ·ÛıÂÓ›˜.

ÕÏϘ ıÂڷ›˜

™Â ·È‰È¿ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À

ÌÂÙ¿ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ¤̂·ÛË Û˘ÓÈÛÙ¿Ù·È Ë ¯Ú‹ÛË

ÙÔ˘ ÚÈÓÈÎÔ‡ CPAP. ∂›Û˘, ¤¯ÂÈ ·Ú·ÙËÚËı› ‚ÂÏ-

Ù›ˆÛË ÌÂÙ¿ ·fi ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÂȉÒÓ ÂÓ‰ÔÚÚÈÓÈ-

ο Û ·È‰È¿ Ì ÂÏ·ÊÚ¿ ¤ˆ˜ ̤ÙÚÈ· ·fiÊÚ·ÍË Ô˘

‰ÂÓ ¤¯Ô˘Ó ¯ÂÈÚÔ˘ÚÁËı› (53), ηıÒ˜ ›Û˘ Û ·È-

‰È¿ Ì ˘ÔÏÂÈfiÌÂÓÔ ™∞∞À ÌÂÙ¿ ÙËÓ ·‰ÂÓÔÂȉÂÎÙÔ-

Ì‹ Î·È ·Ì˘Á‰·ÏÂÎÙÔÌ‹ (54).

∏ ¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· Ù˘ Ó‡-

ÎÙ·˜ ÁÈ· ÙËÓ ·ÔÊ˘Á‹ Ù˘ Ó˘¯ÙÂÚÈÓ‹˜ ÔÍ·ÈÌ›·˜ ‰ÂÓ

‚ÔËı¿ÂÈ Û fiÏÔ˘˜ ÙÔ˘˜ Ù‡Ô˘˜ ÙÔ˘ ™∞∞À, ‰ÈfiÙÈ ‰ÂÓ

ÚÔÊ˘Ï¿ÛÛÂÈ ·fi ÙËÓ ·fiÊÚ·ÍË ÙÔ˘ ·ÓÒÙÂÚÔ˘ ·Ó·-

Ó¢ÛÙÈÎÔ‡ Î·È ÂÈϤÔÓ ÌÔÚ› Ó· ÂȉÂÈÓÒÛÂÈ ÙÔÓ

˘Ô·ÂÚÈÛÌfi (55). ™˘ÓÈÛÙ¿Ù·È fï˜ Ë ¯ÔÚ‹ÁËÛË Ô͢-

ÁfiÓÔ˘ ηٿ ÙË ‰È¿ÚÎÂÈ· ÙÔ˘ ‡ÓÔ˘ ÛÙÔ ™∞∞À Ù‡Ô˘

ππ, ·Ú¿ÏÏËÏ· ÌÂ ÙÔ ÚÈÓÈÎfi CPAP, ÂÓÒ Ë ¯ÔÚ‹ÁËÛË

Ô͢ÁfiÓÔ˘ >18 ÒÚ˜ ËÌÂÚËÛ›ˆ˜ Û˘ÓÈÛÙ¿Ù·È Û ·ÛıÂ-

Ó›˜ Ô˘ ÂÌÊ·Ó›˙Ô˘Ó Î·È ËÌÂÚ‹ÛÈ· ˘ÔÍ·ÈÌ›· (38).

™Â ηډȷ΋ ·Ó¿ÚÎÂÈ· ÌÔÚ› ›Û˘ Ó· ÔÚË-

ÁËıÔ‡Ó ‰·ÎÙ˘Ï›Ùȉ· Î·È ‰ÈÔ˘ÚËÙÈο ̤¯ÚÈ ÙËÓ ¿ÚÛË

Ù˘ ·fiÊڷ͢ (36).

∆· ·È‰È¿ Ô˘ ¤¯Ô˘Ó ˘ÔÛÙ› ¯ÂÈÚÔ˘ÚÁÈ΋ ¤Ì-

‚·ÛË ÁÈ· ™∞∞À Ú¤ÂÈ Ó· ·ÓÂÎÙÈÌËıÔ‡Ó ÌÂÙ¿

·fi 6-8 ‚‰ÔÌ¿‰Â˜ ÁÈ· Ó· Ê·Ó› Â¿Ó ¤¯Ô˘Ó ‚ÂÏÙȈ-

ı› ‹ ¯ÚÂÈ¿˙ÔÓÙ·È ÂÈÚfiÛıÂÙË ıÂڷ›·, fiˆ˜ ÙÔ

ÚÈÓÈÎfi CPAP (1,2).

µÈ‚ÏÈÔÁÚ·Ê›·

1. Blum RH, McGowan FX Jr. Chronic upper airway obstru-

ction and cardiac dysfunction: anatomy, pathophysiology

and anesthetic implications. Paediatr Anaesth 2004;14:75-83.

2. American Academy of Pediatrics. Clinical Practice

Guideline: Diagnosis and Management of Childhood

Obstructive Sleep Apnea Syndrome. Pediatrics 2002;109:

704-712.

3. Marcus CL. Sleep-disordered breathing in children. Am J

Respir Crit Care Med 2001;164:16-30.

4. Laurikainen E, Erkinjuntti M, Alihanka J, Rikalainen H,

Suonpaa J. Radiological parameters of the bony naso-

pharynx and the adenotonsillar size compared with sleep

apnea episodes in children. Int J Pediatr Otorhinolaryngol,

1987;12:303-310.

5. Brooks LJ, Stephens BM, Bacevice AM. Adenoid size is

related to severity but not the number of episodes of

obstructive apnea in children. J Pediatr 1998;132:682-86.

6. Ali NJ, Pitson DJ, Stradling JR. Snoring, sleep disturbance

and behaviour in 4-5 years olds. Arch Dis Child 1993;68:

360-366.

7. Gislason T, Benediktsdottir B. Snoring, apneic episodes

and nocturnal hypoxemia among children 6 months to 6

years old. An epidemiologic study of lower limit of

prevalence. Chest 1995;107:963-966.

8. Lipton AJ, Gozal D. Treatment of obstructive sleep apnea

in children: do we really know how? Sleep Med Rev 2003;

7:61-80.

9. Hultcrantz E, Lofstrand-Tidestrom B, Ahlquist-Rastad J.

136 ∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘

Paediatriki 2009;72:136-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·136

Page 65: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

The epidemiology of sleep related breathing disorder in

children. Int J Pediatr Otorhinolaryngol 1995;32(suppl):

S63-S66.

10. Owen GO, Canter RJ, Robinson A. Snoring, apnoea and

ENT symptoms in the pediatric community. Clin

Otolaryngol Allied Sci 1996;21:130-134.

11. Ferreira AM, Clemente V, Gozal D, Gomes A, Pissara C,

Cesar H, et al. Snoring in Portuguese primary school

children. Pediatrics 2000;106:E64.

12. Capdevila OS, Kheirandish-Gozal L, Dayyat E, Gozal D.

Pediatric obstructive sleep apnea: complications, manage-

ment and long-term outcomes. Proc Am Thorac Soc 2008;

5:274-282.

13. Ievers-Landis CE, Redline S. Pediatric sleep apnea:

implications of the epidemic of childhood overweight. Am

J Respir Crit Care Med 2007;175:436-441.

14. Redline S, Storfer-Isser A, Rosen CL, Johnson NL,

Kirchner HL, Emancipator J, et al. Association between

metabolic syndrome and sleep-disordered breathing in

adolescents. Am J Respir Crit Care Med 2007;176:401-408.

15. Mansfield DR, Naughton MT. Sleep apnea and congestive

heart failure. Minerva Med 2004;95:257-280.

16. Gozal D, Wang M, Pope DW Jr. Objective sleepiness

measures in pediatric obstructive sleep apnea. Pediatrics

2001;108:693-697.

17. Chervin RD, Dillon JE, Bassetti C, Ganoczy D, Pituch KJ.

Symptoms of sleep disorders, inattention and hyperactivity

in children. Sleep 1997;20:1185-1192.

18. Owens J, Opipari L, Nobile C, Spirito A. Sleep and daytime

behavior in children with obstructive sleep apnea and

behavioral sleep disorders. Pediatrics 1998;102:1178-1184.

19. Ali NJ, Pitson D, Stradling JR. Sleep disordered breathing:

effects of adenotonsillectomy on behaviour and psycho-

logical functioning. Eur J Pediatr 1996;155:56-62.

20. Gozal D. Sleep-disordered breathing and school

performance in children. Pediatrics 1998;102:616-620.

21. Marcus CL, Carroll JL, Koerner CB, Hamer A, Lutz J,

Loughlin GM. Determinants of growth in children with

the obstructive sleep apnea syndrome. J Pediatr 1994;125:

556-562.

22. Bar A, Tarasiuk A, Segev Y, Phillip M, Tal A. The effect of

adenotonsillectomy on serum insulin-like growth factor-I

and growth in children with obstructive sleep apnea

syndrome. J Pediatr 1999;135:76-80.

23. Marcus CL, Omlin KJ, Basinski DJ, Bailey SL, Rachal AB,

Von Pechmann WS. Normal polysomnographic values for

children and adolescents. Am Rev Resp Dis 1992;146:1235-

1239.

24. Bixler EO, Vgontzas AN, Lin HM, Ten Have T, Leiby BE,

Vela-Bueno A, et al. Association of hypertension and sleep-

disordered breathing. Arch Intern Med 2000;160:2289-2295.

25. Somers VK, Dyken ME, Clary MP, Abboud FM.

Sympathetic neural mechanisms in obstructive sleep

apnea. J Clin Invest 1995;96:1897-1904.

26. Phillips BG, Narkiewich K, Pesek CA, Haynes WG, Dyken

ME, Somers VK. Effects of obstructive sleep apnea on

endothelin-1 and blood pressure. J Hypertens 1999;17:61-66.

27. Quan SF, Gersh BJ. Cardiovascular consequences of sleep-

disordered breathing: past, present and future. Report of a

workshop from the national center on sleep disorders

research and the national heart, lung and blood institute.

Circulation 2004,109:951-957.

28. Peled N, Greenberg A, Pillar G, Zinder O, LevI N, Lavie P.

Contributions of hypoxia and respiratory disturbance

index to sympathetic activation and blood pressure in

obstructive sleep apnea syndrome. Am J Hypertens

1998;11:1284-1289.

29. Fletcher EC. Effect of episodic hypoxia on sympathetic

activity and blood pressure. Respir Physiol 2000;119:

189-197.

30. Marcus CL, Green MG, Carroll JL. Blood pressure in

children with obstructive sleep apnea. Am J Respir Crit

Care Med 1998;157:1098-1103.

31. Amin RS, Carroll JL, Jeffries JL, Grone C, Bean JA, Chini B,

et al. Twenty-four-hour ambulatory blood pressure in

children with sleep disordered breathing. Am J Respir Crit

Care Med 2004;169:950-956.

32. Amin RS, Daniels S, Kimball T, Willging P, Cotton R.

Echocardiographic changes in children with obstructive

sleep apnea. Sleep 2000;23:A99.

33. Gula LJ, Krahn AD, Skanes AC, Yee R, Klein GJ. Clinical

relevance of arrhythmias during sleep: guidance for

clinicians. Heart 2004;90:347-352.

34. Leung RS, Bradley TD. Sleep apnea and cardiovascular

disease. Am J Respir Crit Care Med 2001;164:2147-2165.

35. Ziegler MG, Mills PJ, Loredo JS, Ancoli-Israel S, Dimsdale JE.

Effect of continuous positive airway pressure and placebo

treatment on sympathetic nervous activity in patients with

obstructive sleep apnea. Chest 2001;120:887-893.

36. Noonan JA. Cor pulmonale. In Chernick V, Boat TF,

editors. Kendig’s Disorders of the Respiratory Tract in

Children, 6th ed. Philadelphia, Pensylvania: WB Saunders

Company; 1998. p. 826.

37. Widlitz A, Barst RJ. Pulmonary arterial hypertension in

children. Eur Respir J 2003,21:155-176.

38. Kessler R, Chaouat A, Weitzenblum E, Oswald M, Ehrhart

M, Apprill M, et al. Pulmonary hypertension in the

obstructive sleep apnoea syndrome: prevalence, causes and

therapeutic consequences. Eur Respir J 1996;9:787-794.

39. Pac A, Karadag A, Kurtaran J, Aktas D. Comparison of

cardiac function and valvular damage in children with and

without adenotonsillar hypertrophy. Int J Pediatr

Otorhinolaryngol 2005;69:527-532.

40. Amin RS, Kimball TR, Bean JA, Jeffries JL, Willging JP,

Cotton RT, et al. Left ventricular hypertrophy and

abnormal ventricular geometry in children and adole-

scents with obstructive sleep apnea. Am J Respir Crit Care

Med 2002;165:1395-1399.

41. Amin RS, Kimball TR, Kalra M, Jeffries JL, Carroll JL, Bean

JA, et al. Left ventricular function in children with sleep-

disordered breathing. Am J Cardiol 2005;95:801-804.

42. Zucconi M, Strambi LF, Pestalozza G, Tessitore E, Smirne

S. Habitual snoring and obstructive sleep apnea syndrome

in children: effects of early tonsil surgery. Int J Pediatr

Otorhinolaryngol 1993;26:235-243.

43. Nieminen P, Tolonen U, Lopponen H. Snoring and

obstructive sleep apnea in children: a 6-month follow-up

study. Arch Otolaryngol Head Neck Surg 2000;126:

481-486.

44. Shintani T, Asakura K, Kataura A. The effect of adeno-

tonsillectomy in children with OSA. Int J Pediatr Oto-

rhinolaryngol 1998;44:51-58.

45. Gorur K, Doven O, Unal M, Akkus N, Ozcan C.

Preoperative and postoperative cardiac and clinical

137∫·Ú‰ÈÔÏÔÁÈΤ˜ ÂÈÙÒÛÂȘ ˘ÂÚÙÚÔÊ›·˜ ·‰ÂÓÔÂȉÒÓ-·Ì˘Á‰·ÏÒÓ

¶·È‰È·ÙÚÈ΋ 2009;72:137-000

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Page 66: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

findings of patients with adenotonsillar hypertrophy. Int JPediatr Otorhinolaryngol 2001;59:41-46.

46. Jain A, Sahni JK. Polysomnographic studies in childrenundergoing adenoindectomy and/or tonsillectomy. JLaryngol Otol 2002;116:711-715.

47. Tal A, Bar A, Leiberman A, Tarasiuk A. Sleep characte-ristics following adenotonsillectomy in children withobstructive sleep apnea syndrome. Chest 2003;124:948-953.

48. Sdralis T, Berkowitz RG. Early adenotonsillectomy forrelief of acute upper airway obstruction due to acutetonsillitis in children. Int J Pediatr Otorhinolaryngol1996;35:25-29.

49. Nieminen P, Tolonen U, Lopponen H, Lopponen T,Luotonen J, Jokinen K. Snoring children: factors predictingsleep apnea. Acta Otolaryngol Suppl 1997;529:190-194.

50. Suen JS, Arnold JE, Brooks LJ. Adenotonsillectomy fortreatment of obstructive sleep apnea in children. ArchOtolaryngol Head Neck Surg 1995;121:525-530.

51. Redline S, Tishler PV, Schluchter M, Aylor J, Clark K,

Graham G. Risk factors for sleep-disordered breathing inchildren. Associations with obesity, race and respiratoryproblems. Am J Respir Crit Care Med 1999;159:1527-1532.

52. Rosen GM, Muckle RP, Mahowald MW, Goding GS,Ulleviq C. Postoperative respiratory compromise inchildren with obstructive sleep apnea syndrome: can it beanticipated? Pediatrics 1994;93:784-788.

53. Alexopoulos EI, Kaditis AG, Kalampouka E, Kostadima E,Angelopoulos NV, Mikraki V, et al. Nasal corticosteroids forchildren with snoring. Pediatr Pulmonol 2004;38:161-167.

54. Kheiradish L, Goldbart AD, Gozal D. Intranasal steroidsand oral leukotriene modifier therapy in residual sleep-disordered breathing after tonsillectomy and adeno-idectomy in children. Pediatrics 2006;117:e61-e66.

55. Marcus CL, Carroll JL, Bamford O, Pyzik P, Loughlin GM.Supplemental oxygen during sleep in children with sleep-disordered breathing. Am J Respir Crit Care Med 1995;152:1297-1301.

138 ∫. ¶··‰ÔÔ‡ÏÔ˘-§ÂÁ̤ÏÔ˘, °. µ·ÚÏ¿Ì˘

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√Ú›˙ÔÓÙ·˜ ÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ·¯˘Û·ÚΛ· ÛÙËÓ Ú¿ÍË:

ÌÂıÔ‰ÔÏÔÁÈο ‰ÈÏ‹ÌÌ·Ù·

¶. ¶ÂÚ‚·Ó›‰Ô˘

¶ÂÚ›ÏË„Ë: √ ÔÚÈÛÌfi˜ Î·È Ë Ì¤ÙÚËÛË Ù˘ ·¯˘Û·ÚΛ·˜ ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ· ·ÊÔÚÔ‡Ó Û ÌÂ-Á¿ÏÔ ‚·ıÌfi ÙÔÓ ·È‰›·ÙÚÔ Û‹ÌÂÚ·. √È ‰ÈÂıÓ›˜ ÔÚÈÛÌÔ› Ù˘ ·¯˘Û·ÚΛ·˜, Ô˘ ‚·Û›˙ÔÓÙ·È Î·Ù¿ ·ÚÈÔÏfiÁÔ ÛÙÔÓ ¢Â›ÎÙË ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™) Î·È ÛÙ· ÔÚȷο ÛËÌ›· ÎÈÓ‰‡ÓÔ˘ ÁÈ· ÙÔ˘˜ ÂÓ‹ÏÈΘ, ¯ÚËÛÈÌÔ-ÔÈÔ‡ÓÙ·È Û ÂȉËÌÈÔÏÔÁÈΤ˜ ÌÂϤÙ˜ Î·È Ê·›ÓÂÙ·È fiÙÈ ‰ÂÓ Â›Ó·È Î·Ù¿ÏÏËÏÔÈ ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÙˆÓ·È‰ÈÒÓ ÛÙËÓ ÎÏÈÓÈ΋ Ú¿ÍË. ∏ Û˘ÓÈÛÙÒÌÂÓË ÎÏÈÓÈ΋ ·ÍÈÔÏfiÁËÛË ÙÔ˘ ·È‰ÈÔ‡ ‹ ÂÊ‹‚Ô˘ ÍÂÎÈÓ¿ Ì ÙËÓ ÙÔÔı¤ÙËÛË Ù˘ ÙÈÌ‹˜ ÙÔ˘ ¢Â›-ÎÙË ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™) ÛÙ· ÈÔ Û‡Á¯ÚÔÓ· ·ÓıÚˆÔÌÂÙÚÈο ÚfiÙ˘· ‰È·ÁÚ¿ÌÌ·Ù· ÙÔ˘ ¢ª™ ·Ó¿Ê‡ÏÔ Î·È ·Ó¿ ¯ÒÚ·. ∏ ÚÔ¤ÎÙ·ÛË ÙˆÓ ÔÚÈ·ÎÒÓ ÛËÌ›ˆÓ ÙÔ˘ ˘¤Ú‚·ÚÔ˘ Î·È ÙÔ˘ ·¯‡Û·ÚÎÔ˘ ÁÈ· ÙÔ˘˜ÂÓ‹ÏÈΘ ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı› ÁÈ· ÙËÓ ·ÓÙ›ÛÙÔÈ¯Ë Î·Ù¿Ù·ÍË ÙˆÓ ·È‰ÈÒÓ ÂÏÏËÓÈ΋˜ ηٷÁˆÁ‹˜, ÂÓÒÁÈ· ÙËÓ ÂÎÙ›ÌËÛË ÙÔ˘ ÔÛÔÛÙÔ‡ ·¯˘Û·ÚΛ·˜ ̤¯ÚÈ ÙËÓ ËÏÈΛ· ÙˆÓ 18 ÚfiÓˆÓ, Û ¤Ó· Û˘ÁÎÂÎÚÈ̤ÓÔ ÏË-ı˘ÛÌfi, Û˘ÓÈÛÙ¿Ù·È Ë Ì¤ÙÚËÛË ÙÔ˘ ¢ª™ Û ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ ·fi ÙÔ Ì¤ÛÔ fiÚÔ (z-score). ŸÏ˜ ÔÈ Ì¤-ıÔ‰ÔÈ Ì¤ÙÚËÛ˘ ·ÚÔ˘ÛÈ¿˙Ô˘Ó ÏÂÔÓÂÎÙ‹Ì·Ù· Î·È ÌÂÈÔÓÂÎÙ‹Ì·Ù·.

§¤ÍÂȘ ÎÏÂȉȿ: ¶·¯˘Û·ÚΛ·, ·È‰›, ‰Â›ÎÙ˘ Ì¿˙·˜ ÛÒÌ·ÙÔ˜, ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ, z-score.

Defining obesity in children and adolescents: methodological

dilemmas

P. Pervanidou

Abstract: πn defining obesity in children and adolescents, clinicians are attempting to identify thoseindividuals with excessive adiposity. However, the accurate estimation of body fat content and of theconsequent health implications is not an easy task. International definitions of childhood obesity, based onBody Mass Index (BMI) and adult cut-off points, which are used in large epidemiologic studies are lessvaluable in clinical practice.Definitions dependent upon a reference population, based on the concept of a certain level of deviationfrom the mean BMI of the population and using centiles and z-scores, may present variable cut-off points,as the reference curves are likely to vary between countries and may change over time within one country.In monitoring patients in the clinical setting, however, this approach provides a continuous measure in thesame patient and a comparison measure of obesity status between groups.

Key words: Obesity, child, body mass index, percentiles, z-score.

139¶ƒ∞∫∆π∫√ £∂ª∞ PRACTICAL ISSUE

π·ÙÚÂ›Ô ¶·È‰È΋˜ &∂ÊË‚È΋˜ ¶·¯˘Û·ÚΛ·˜, ∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∏ ∞Á›· ™ÔÊ›·»

AÏÏËÏÔÁÚ·Ê›·:

¶·Ó·ÁÈÒÙ· ¶ÂÚ‚·Ó›‰Ô˘[email protected]∞’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «∏ ∞Á›· ™ÔÊ›·», ∞ı‹Ó·

Childhood and AdolescenceObesity Clinic, First Department ofPediatrics, Athens UniversityMedical School, “Aghia Sophia” Children’sHospital, Athens, Greece

Correspondence:

Panagiota [email protected] for Developmental andBehavioral Paediatrics, First Department ofPaediatrics, Athens UniversityMedical School, “Aghia Sophia” Children’sHospital, Athens, Greece

¶·È‰È·ÙÚÈ΋ 2009;72:23-28

∏ ·¯˘Û·ÚΛ· ÁÂÓÈο ÔÚ›˙ÂÙ·È ˆ˜ Ë ˘ÂÚ-

‚ÔÏÈ΋ Û˘ÛÛÒÚ¢ÛË Ï›Ô˘˜ ÛÙÔÓ ÔÚÁ·ÓÈÛÌfi, ÛÂ

‚·ıÌfi Ô˘ ÂËÚ¿˙ÂÙ·È Ë ˘Á›· ÙÔ˘ ·ÙfiÌÔ˘

(1). ∏ ÔÛÔÙÈÎÔÔ›ËÛË, fï˜, ÙÔ˘ ÏÈÒ‰Ô˘˜

ÈÛÙÔ‡, ηıÒ˜ Î·È Ô ‚·ıÌfi˜ ›‰Ú·Û˘ Ù˘ Â-

Ú›ÛÛÂÈ·˜ Ï›Ô˘˜ ÛÙËÓ ˘Á›· ÙÔ˘ ·ÙfiÌÔ˘ ‰ÂÓ Â›-

Ó·È Â‡ÎÔÏÔ Ó· ·ÍÈÔÏÔÁËıÔ‡Ó ÛÙËÓ Î·ıËÌÂÚÈÓ‹

ÎÏÈÓÈ΋ Ú¿ÍË. OÈ Û‡Á¯ÚÔÓ˜ ̤ıÔ‰ÔÈ Ì¤ÙÚË-

Û˘ Ù˘ Ì¿˙·˜ ÙÔ˘ ÏÈÒ‰Ô˘˜ ÈÛÙÔ‡, fiˆ˜ Ë Ì¤-

ÙÚËÛË Ù˘ ۈ̷ÙÈ΋˜ ˘ÎÓfiÙËÙ·˜ οو ·fi ÙÔ

ÓÂÚfi, Ë ÂÚÈÂÎÙÈÎfiÙËÙ· ۈ̷ÙÈÎÔ‡ Ï›Ô˘˜

fiˆ˜ ÂÎÙÈÌ¿Ù·È Ì¤Ûˆ ÙÔ˘ ·ÔÚÚÔÊËÛÈÔÌÂÙÚË-

Ù‹ ‰ÈÏ‹˜ ÂÓ¤ÚÁÂÈ·˜ ·ÎÙ›ÓˆÓ Ã (dual-energy X-

ry absorptiometer, DEXA), ηıÒ˜ Î·È ÔÈ Ó¤Â˜

Ù¯ÓÈΤ˜, fiˆ˜ Â›Ó·È Ë ·ÂÈÎfiÓÈÛË Ì·ÁÓËÙÈÎÔ‡

Û˘ÓÙÔÓÈÛÌÔ‡ (ªagnetic Resonance Imaging,

MRI) Î·È Ë ˘ÔÏÔÁÈÛÙÈ΋ ÙÔÌÔÁÚ·Ê›·

(Computed Tomography, CT), ¤ÙÚ„·Ó Ó·

ÂÚÈÁÚ·Ê› Ì ·ÎÚ›‚ÂÈ· Ô ÏÈ҉˘ ÈÛÙfi˜, ·ÏÏ¿

·ÔÙÂÏÔ‡Ó ÛÙËÓ ÏÂÈÔ„ËÊ›· ÙÔ˘˜ ‰··ÓËÚ¤˜

Î·È ¯ÚÔÓÔ‚fiÚ˜ ÂÍÂÙ¿ÛÂȘ, ÁÂÁÔÓfi˜ Ô˘ ÂÚÈÔ-

Ú›˙ÂÈ ÙË ¯Ú‹ÛË ÙÔ˘˜ ÛÙËÓ ÎÏÈÓÈ΋ ¤Ú¢ӷ (2).

√ ¢Â›ÎÙ˘ ª¿˙·˜ ™ÒÌ·ÙÔ˜

ª¤¯ÚÈ Û‹ÌÂÚ·, Û ÌÂÁ¿Ï˜ ÏËı˘ÛÌȷΤ˜

ÌÂϤÙ˜ Î·È ·ÓȯÓ¢ÙÈÎÔ‡˜ ÂϤÁ¯Ô˘˜ ¤¯Ô˘Ó ¯ÚË-

ÛÈÌÔÔÈËı› ‰Â›ÎÙ˜ ‚¿ÚÔ˘˜ ‹ Ì¿˙·˜ ÛÒÌ·ÙÔ˜,

ÔÚÈ˙fiÌÂÓÔÈ ˜ ‰È¿ÊÔÚÔÈ Û˘Ó‰˘·ÛÌÔ› ÙÔ˘ ‚¿ÚÔ˘˜

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·139

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ÚÔ˜ ÙÔ ‡„Ô˜. ∞fi ÙÔ˘˜ ‰Â›ÎÙ˜ ·˘ÙÔ‡˜ ·Ó·‰Â›¯ÙËÎÂ

Ô ¢Â›ÎÙ˘ ª¿˙·˜ ™ÒÌ·ÙÔ˜ (¢ª™), Ô˘ ÔÚ›˙ÂÙ·È ˆ˜

‚¿ÚÔ˜ ÛÒÌ·ÙÔ˜ Û ÎÈÏ¿ (kg) ‰È·ÈÚÔ‡ÌÂÓÔ ‰È· ÙÔ˘ ÙÂ-

ÙÚ·ÁÒÓÔ˘ ÙÔ˘ ‡„Ô˘˜ Û ̤ÙÚ· (m2), ˆ˜ ¤Ó· ·Ïfi ηÈ

·Ó¤ÍÔ‰Ô Ì¤ÛÔ ÔÚÈÛÌÔ‡ Ù˘ ·¯˘Û·ÚΛ·˜ ÙfiÛÔ ÛÙÔ˘˜

ÂÓ‹ÏÈΘ fiÛÔ Î·È ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜. √

¢ª™ ¤¯ÂÈ ‚ÚÂı› fiÙÈ ¤¯ÂÈ Ôχ ÈÛ¯˘Ú‹ ıÂÙÈ΋ Û˘Û¯¤-

ÙÈÛË Ì ÙÔ ÛˆÌ·ÙÈÎfi ϛԘ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ (3,4).

√ ¢ª™ ¯ÚËÛÈÌÔÔÈÂ›Ù·È Â‡ÎÔÏ· ·fi ÎÏÈÓÈÎÔ‡˜

ÁÈ·ÙÚÔ‡˜ ÔÏÏÒÓ ÂȉÈÎÔًوÓ, ÚÔÎÂÈ̤ÓÔ˘ Ó· ‰È·-

ÎÚ›ÓÂÈ Ù· ·¯‡Û·Úη ·fi Ù· ˘¤Ú‚·Ú· Î·È Î·ÓÔÓÈ-

ÎÔ‡ ‚¿ÚÔ˘˜ ¿ÙÔÌ·. √ ¢ª™ >25 Î·È <30 ÔÚ›˙ÂÈ Ù·

˘¤Ú‚·Ú· Î·È Ô ¢ª™ ≥ 30 Ù· ·¯‡Û·Úη ÂÓ‹ÏÈη

¿ÙÔÌ· ÛÙË Ï¢΋ Ê˘Ï‹. ™Ù· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹-

‚Ô˘˜, Ë ·¯˘Û·ÚΛ· ÔÚ›˙ÂÙ·È Ì ‚¿ÛË ÙÔÓ ¢ª™ ·Ó¿

ʇÏÔ Î·È ËÏÈΛ·. ∫·Ù¿ ÙËÓ ÚÔÛ¤ÁÁÈÛË ·˘Ù‹, ¤¯Ô˘Ó

ÚÔÙ·ı› ÔÚȷο ÛËÌ›· (cut-off points) ÁÈ· ÙÔ

˘¤Ú‚·ÚÔ Î·È ÙËÓ ·¯˘Û·ÚΛ·, ˆ˜ ÔÛÔÛÙ¿ ¿Óˆ

·fi ¤Ó·Ó ‰Â‰Ô̤ÓÔ ¢ª™, ÁÈ· ÙËÓ ËÏÈΛ· Î·È ÙÔ Ê‡ÏÔ

ÙÔ˘ ·ÙfiÌÔ˘. √ ‰Â‰Ô̤ÓÔ˜ ·˘Ùfi˜ ¢ª™ Â›Ó·È Û˘Ó‹-

ıˆ˜ Ë Ì¤ÛË (mean) ‹ Ë ‰È¿ÌÂÛË (median) ÙÈÌ‹ Ù˘

ηٷÓÔÌ‹˜ ÂÓfi˜ ÏËı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜. H ·fiÛÙ·-

ÛË ·fi ÙË Ì¤ÛË ‹ ‰È¿ÌÂÛË ·˘Ù‹ ÙÈÌ‹ ÌÂÙÚÈ¤Ù·È Â›ÙÂ

̤ۈ ÙˆÓ ÂηÙÔÛÙÈ·›ˆÓ ı¤ÛÂˆÓ Â›Ù ̤ۈ Ù˘ ÛÙ·-

ıÂÚ‹˜ ·fiÎÏÈÛ˘ (˘ÔÏÔÁÈÛÌfi˜ ÙÔ˘ z-score).

√È ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ

∆· ‰È·ÁÚ¿ÌÌ·Ù· ·Ó·ÊÔÚ¿˜ ·‡ÍËÛ˘ (‚¿ÚÔ˘˜,

‡„Ô˘˜ Î·È ¢ª™) Ô˘ ¤¯Ô˘Ó ·Ó·Ù‡ÍÂÈ ÔÏϤ˜ ¯Ò-

Ú˜ ‚·Û›˙ÔÓÙ·È ÛÂ Û˘Á¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ ÙÔÌ‹˜

(cross sectional) ·ÓÙÈÚÔÛˆÂ˘ÙÈÎÔ‡ ‰Â›ÁÌ·ÙÔ˜

·È‰ÈÒÓ, ·fi ÙË Á¤ÓÓËÛË ˜ ÙËÓ ÂÓËÏÈΛˆÛË. ∆· Â-

ÚÈÛÛfiÙÂÚ· Û˘ÛÙ‹Ì·Ù· ηٿٷ͢ Û˘ÌʈÓÔ‡Ó Î·È

ÔÚ›˙Ô˘Ó ÙËÓ 85Ë ÂηÙÔÛÙÈ·›· ı¤ÛË (∂£) ÛÙÔ ‰È¿-

ÁÚ·ÌÌ· ¢ª™ ˆ˜ ÙÔ fiÚÈÔ ¿Óˆ ·fi ÙÔ ÔÔ›Ô ¤Ó·

·È‰› ‹ ¤ÊË‚Ô˜ ¯·Ú·ÎÙËÚ›˙ÂÙ·È ˆ˜ ˘¤Ú‚·ÚÔ˜ ηÈ

ÙËÓ 95Ë (‹ 97Ë) ∂£ ˆ˜ ÙÔ fiÚÈÔ ¿Óˆ ·fi ÙÔ ÔÔ›Ô

ÙÔ ¿ÙÔÌÔ Î·Ù·Ù¿ÛÛÂÙ·È ˆ˜ ·¯‡Û·ÚÎÔ (1,5-8). °È·

ÙȘ ¯ÒÚ˜ Ô˘ ¤¯Ô˘Ó ·Ó·Ù‡ÍÂÈ ÂıÓÈο ‰È·ÁÚ¿ÌÌ·-

Ù·, Ô ÎÏÈÓÈÎfi˜ ÔÚÈÛÌfi˜ ÙÔ˘ ˘¤Ú‚·ÚÔ˘ Î·È Ù˘ ·-

¯˘Û·ÚΛ·˜ Û˘ÓÈÛÙ¿Ù·È Ó· ‚·Û›˙ÂÙ·È ÛÙ· ‰È·ÁÚ¿ÌÌ·-

Ù· ·˘Ù¿ (8), ‰È·ÊÔÚÂÙÈο ÌÔÚÔ‡Ó Ó· ¯ÚËÛÈÌÔÔÈ-

Ô‡ÓÙ·È Ù· ÏËÛȤÛÙÂÚ· ‰È·ı¤ÛÈÌ·.

∞˘Ùfi˜ Ô ÙÚfiÔ˜ ηٿٷ͢, fï˜, ‚·Û›˙ÂÙ·È

ÛÙËÓ È‰¤· ˆ˜ Ë ·¯˘Û·ÚΛ· ÔÚ›˙ÂÙ·È Ì ‚¿ÛË ¤Ó·

ÔÚÈṲ̂ÓÔ Â›Â‰Ô ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘ ·fi ÙÔÓ Ì¤-

ÛÔ fiÚÔ ÛÙÔÓ Û˘ÁÎÂÎÚÈ̤ÓÔ ÏËı˘ÛÌfi Ô˘ ÌÂÏÂÙ¿-

Ù·È. √ ̤ÛÔ˜ ·˘Ùfi˜ fiÚÔ˜, fï˜, ÌÔÚ› Ó· ÔÈΛÏÏÂÈ

Û ‰È¿ÊÔÚ˜ ¯ÒÚ˜, ηıÒ˜ Î·È ÛÙËÓ ›‰È· ¯ÒÚ·, fiÙ·Ó

ÌÂ ÙËÓ ¿ÚÔ‰Ô ÙˆÓ ¯ÚfiÓˆÓ ÌÂÙ·‚¿ÏÏÔÓÙ·È Ù· ·Ó-

ıÚˆÔÌÂÙÚÈο ¯·Ú·ÎÙËÚÈÛÙÈο ·˘Ù‹˜.

∏ ¯Ú‹ÛË ÙˆÓ ‰È·ÁÚ·ÌÌ¿ÙˆÓ ∂£ ÂÈÙÚ¤ÂÈ Â›-

Û˘ ÙÔÓ ¿ÌÂÛÔ ÚÔÛ‰ÈÔÚÈÛÌfi Ù˘ ËÏÈΛ·˜ ·Ó·ÛÙÚÔ-

Ê‹˜ ÙÔ˘ ¢ª™ (adiposity rebound), ‰ËÏ·‰‹ Ù˘ ËÏÈ-

Λ·˜ ‰Â‡ÙÂÚ˘ ·‡ÍËÛ˘ ÙÔ˘ ¢ª™ ÌÂÙ¿ ÙË Ì›ˆÛ‹

ÙÔ˘ ηٿ ÙËÓ ÚÔÛ¯ÔÏÈ΋ ËÏÈΛ·. √ ‰Â›ÎÙ˘ ·˘Ùfi˜

·ÔÙÂÏ› ÈÛ¯˘Úfi ÚÔÁÓˆÛÙÈÎfi ‰Â›ÎÙË ÁÈ· ÙËÓ ·Ó¿-

Ù˘ÍË ·¯˘Û·ÚΛ·˜: fiÛÔ ÓˆÚ›ÙÂÚ· Û˘Ì‚Â› Ë ·Ó·-

ÛÙÚÔÊ‹ ÙÔ˘ ¢ª™, ÙfiÛÔ ÌÂÁ·Ï‡ÙÂÚÔ˜ Ô Î›Ó‰˘ÓÔ˜

·Ó¿Ù˘Í˘ ·¯˘Û·ÚΛ·˜.

∆Ô z-score

ª›· ÈÔ ·ÎÚÈ‚‹˜ Î·È ÔχÏÔÎË Ì¤ıÔ‰Ô˜ ÁÈ· ÙË

̤ÙÚËÛË Ù˘ ·¯˘Û·ÚΛ·˜ ·ÔÙÂÏ› Ô ˘ÔÏÔÁÈÛÌfi˜

ÙÔ˘ ∑ (z-score), Ô˘ ÚÔ·ÙÂÈ ·fi ÙËÓ ·Ê·›ÚÂÛË

Ù˘ ̤Û˘ ÙÈÌ‹˜ ·Ó·ÊÔÚ¿˜ ·fi ÙÔ ÌÂÙÚÔ‡ÌÂÓÔ Ì¤-

ÁÂıÔ˜ (¢ª™ ÛÙËÓ ÚÔΛÌÂÓË ÂÚ›ÙˆÛË) Î·È ÙË ‰È-

·›ÚÂÛË ‰È· Ù˘ ÛÙ·ıÂÚ‹˜ ·fiÎÏÈÛ˘ ÙÔ˘ ÏËı˘ÛÌÔ‡

·Ó·ÊÔÚ¿˜ (¢ª™ ·ÙfiÌÔ˘ Ô˘ ÌÂÙÚ¿Ì -- ̤ÛË ÙÈÌ‹

¢ª™ ÙÔ˘ ÏËı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜ / ÛÙ·ıÂÚ‹ ·fiÎÏÈÛË

ÏËı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜). ∆˘Èο, ¤Ó· z-score ÌÂÁ·Ï‡-

ÙÂÚÔ ‹ ›ÛÔ ÙÔ˘ +2 (2 ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ ¿Óˆ ·fi

ÙË Ì¤ÛË ÙÈÌ‹) ÔÚ›˙ÂÈ ÙËÓ ·¯˘Û·ÚΛ·.

∏ ¯Ú‹ÛË ÙÔ˘ z-score ·ÔÙÂÏ› ÈÔ ·ÎÚÈ‚‹ ̤ıÔ-

‰Ô ÔÛÔÙÈÎÔÔ›ËÛ˘ ÙÔ˘ ¢ª™ Î·È Û˘ÓÈÛÙ¿Ù·È Ó·

¯ÚËÛÈÌÔÔÈÂ›Ù·È Û ÂÚ¢ÓËÙÈΤ˜ ÂÚÁ·Û›Â˜ Ô˘ ··È-

ÙÔ‡Ó ÙË Û‡ÁÎÚÈÛË ÔÌ¿‰ˆÓ Ô˘ ÚÔ¤Ú¯ÔÓÙ·È fï˜

·fi ÙÔÓ ›‰ÈÔ ÏËı˘ÛÌfi (¤¯Ô˘Ó ÂÎÙÈÌËı› Ì ‚¿ÛË

ÙȘ ›‰È˜ η̇Ϙ) ‹ Ó· ÚËÛÈÌÔÔÈÂ›Ù·È ÁÈ· ÙËÓ ÈÔ

·ÎÚÈ‚‹ ·Ú·ÎÔÏÔ‡ıËÛË ·ÛıÂÓÒÓ Ô˘ Â›Ó·È Û Úfi-

ÁÚ·ÌÌ· ·Ú¤Ì‚·Û˘. øÛÙfiÛÔ, Ë Ì¤ıÔ‰Ô˜ ·˘Ù‹, ·Ó

Î·È ÈÔ ·ÎÚÈ‚‹˜ ·fi ÙȘ ÂηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ, ·ÚÔ˘-

ÛÈ¿˙ÂÈ Ù· ›‰È· ·ÎÚÈ‚Ò˜ ÌÂÈÔÓÂÎÙ‹Ì·Ù·, ÁÈ·Ù› ‚·Û›˙Â-

Ù·È ÛÙËÓ ›‰È· ȉ¤·: ·fiÛÙ·ÛË ·fi ÙË Ì¤ÛË ÙÈÌ‹ ÂÓfi˜

Û˘ÁÎÂÎÚÈ̤ÓÔ˘ ÏËı˘ÛÌÔ‡, ÌÈ· Û˘ÁÎÂÎÚÈ̤ÓË ¯ÚÔ-

ÓÈ΋ ÂÚ›Ô‰Ô. ∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ Ë ÚÔÛ¤ÁÁÈÛË ·˘Ù‹

‰ÂÓ ÌÔÚ› Ó· ‰Â›ÍÂÈ ÙËÓ ·‡ÍËÛË ÙÔ˘ ÂÈÔÏ·ÛÌÔ‡

Ù˘ ·¯˘Û·ÚΛ·˜ Û ¤Ó·Ó ÏËı˘ÛÌfi, ÁÈ·Ù› Ô Ì¤ÛÔ˜

¢ª™ ÁÈ· ÙÔ Ê‡ÏÔ Î·È ÙËÓ ËÏÈΛ· ·˘Í¿ÓÂÙ·È ÛÙÔÓ

ÏËı˘ÛÌfi ·Ó·ÊÔÚ¿˜. ∂›Û˘, ÔÈ Î·Ì‡Ï˜ ·Ó·ÊÔ-

Ú¿˜ ‰È·Ê¤ÚÔ˘Ó ÌÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ ˆÚÒÓ Î·È ¿Ú·

ÔÈ ÌÂÙÚ‹ÛÂȘ ÙÔ˘ z-score, fiˆ˜ Î·È Ù˘ ÂηÙÔÛÙÈ·›·˜

ı¤Û˘, ‰ÂÓ Â›Ó·È Û˘ÁÎÚ›ÛÈ̘ ÌÂٷ͇ ÙˆÓ ¯ˆÚÒÓ (9).

OÈ ‰ÈÂıÓ›˜ ÔÚÈÛÌÔ› Î·È Ë ÚÔ¤ÎÙ·ÛË ÙˆÓ

ÔÚÈ·ÎÒÓ ÛËÌ›ˆÓ ÙˆÓ ÂÓËϛΈÓ

∏ ·Ó¿ÁÎË ÁÈ· ÂÓÔÔ›ËÛË ÙÔ˘ ÔÚÈÛÌÔ‡ ÙÔ˘ ˘¤Ú-

‚·ÚÔ˘ Î·È ·¯‡Û·ÚÎÔ˘ Ì ·˘ÙfiÓ ÙˆÓ ÂÓËÏ›ÎˆÓ Ô‰‹-

ÁËÛ ÙÔÓ ¢ÈÂıÓ‹ √ÚÁ·ÓÈÛÌfi ÁÈ· ÙËÓ ∫·Ù·ÔϤÌËÛË

Ù˘ ¶·¯˘Û·ÚΛ·˜ (International Obesity Task Force-

IOTF) Ó· ÚÔÙ›ÓÂÈ ¤Ó·Ó ÎÔÈÓfi Î·È ‰ÈÂıÓ‹ ÔÚÈÛÌfi ÁÈ·

ÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ·¯˘Û·ÚΛ· (10). √È ÂÚ¢-

ÓËÙ¤˜ Û˘ÌÊÒÓËÛ·Ó fiÙÈ, ·ÚfiÙÈ Ô ¢ª™ ‰ÂÓ ·ÔÙÂÏ›

ÙÔÓ È‰·ÓÈÎfi ‰Â›ÎÙË ·¯˘Û·ÚΛ·˜, ÚÔÙ›ÓÂÙ·È ˆ˜ ÙÔ

ϤÔÓ Î·Ù¿ÏÏËÏÔ Ì¤ÛÔ ÔÚÈÛÌÔ‡ Ù˘ ·È‰È΋˜ ηÈ

ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜. ∂›Û˘, Ì ‚¿ÛË ÙÔÓ ÔÚÈÛÌfi

140 ¶. ¶ÂÚ‚·Ó›‰Ô˘

Paediatriki 2009;72:140-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·140

Page 69: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

ÙÔ˘ IOTF, Ù· ÔÚȷο ÛËÌ›· ηٿٷ͢, Î·È ¿Ú· ÎÈÓ-‰‡ÓÔ˘, ÁÈ· Ù· ·È‰È¿ ‚Ú›ÛÎÔÓÙ·È ÛÙȘ ÂηÙÔÛÙÈ·›Â˜ı¤ÛÂȘ Ô˘ ·ÔÙÂÏÔ‡Ó ÚÔ¤ÎÙ·ÛË ÙˆÓ ÙÈÌÒÓ¢ª™=30 Î·È 25 ÙˆÓ ÂÓËÏ›ÎˆÓ ÁÈ· ÙÔÓ ÔÚÈÛÌfi ÙÔ˘·¯‡Û·ÚÎÔ˘ Î·È ÙÔ˘ ˘¤Ú‚·ÚÔ˘ ·ÓÙ›ÛÙÔȯ·.

ªÂ ‚¿ÛË ÙËÓ ÚfiÙ·ÛË ÙÔ˘ IOTF, oÈ Cole et al.(11) ·Ó¤Ù˘Í·Ó ›Ó·Î˜ Ì ÔÚȷο ÛËÌ›·, ·Ó¿ÏÔÁ·Ì ÙÔ Ê‡ÏÔ Î·È ÙËÓ ËÏÈΛ· (·Ó¿ 6 Ì‹Ó˜), Ù· ÔÔ›·ÚԤ΢„·Ó ·fi Ù· ‰Â‰Ô̤ӷ ¤ÍÈ ¯ˆÚÒÓ (µÚ·˙ÈÏ›·,ªÂÁ¿ÏË µÚÂÙ·Ó›·, Hong Kong, √ÏÏ·Ó‰›·, ™ÈÁηԇ-ÚË Î·È ∏ӈ̤Ó˜ ¶ÔÏÈÙ›˜ ∞ÌÂÚÈ΋˜). ∆· ÎÚÈÙ‹ÚÈ·ÙÔ˘ Cole ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·Î· 1. ªÂ ÙÔÓÙÚfiÔ ·˘Ùfi, Ù· ÔÚȷο ÛËÌ›· ÔÚÈÛÌÔ‡ Ù˘ ·È‰È΋˜·¯˘Û·ÚΛ·˜ ‚·Û›˙ÔÓÙ·È ÛÙÔÓ ˘„ËÏfi ΛӉ˘ÓÔ ÓÔÛË-ÚfiÙËÙ·˜ ÛÙËÓ ÂÓ‹ÏÈÎË ˆ‹ Î·È fi¯È ÛÙÔÓ Ì¤ÛÔ fiÚÔ ÙÔ˘˘fi ÌÂϤÙË ÏËı˘ÛÌÔ‡, ÁÂÁÔÓfi˜ Ô˘ ÂÈÙÚ¤ÂÈ Î·ÈÙË Û‡ÁÎÚÈÛË ÙˆÓ ‰È·ÊfiÚˆÓ Ï·ÒÓ ÌÂٷ͇ ÙÔ˘˜.

∆· ÔÚȷο ÛËÌ›· ÙÔ˘ Cole ·ÔÙÂÏÔ‡Ó Û‹ÌÂÚ·

ÙËÓ ÚÔÙÂÈÓfiÌÂÓË Ì¤ıÔ‰Ô ÔÚÈÛÌÔ‡ Ù˘ ·È‰È΋˜ ηÈ

ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜ ÛÂ Û˘ÁÎÚÈÙÈΤ˜ ÂȉËÌÈÔÏÔ-

ÁÈΤ˜ ÌÂϤÙ˜, Â›Ó·È fï˜ ·Î·Ù¿ÏÏËÏË Ì¤ıÔ‰Ô˜ ‰È·-

¯ÚÔÓÈ΋˜ ·Ú·ÎÔÏÔ‡ıËÛ˘ ÂÓfi˜ ·È‰ÈÔ‡ ÛÙËÓ ÎÏÈÓÈ-

΋ Ú¿ÍË (8).

¶ÂÚÈÔÚÈÛÌÔ› ÛÙË Ú‹ÛË ÙÔ˘ ¢ª™

∞Ó Î·È Ë Û˘Û¯¤ÙÈÛË ÌÂٷ͇ ¢ª™ Î·È ÛˆÌ·ÙÈÎÔ‡

Ï›Ô˘˜ Â›Ó·È ÈÛ¯˘Ú‹ ÛÙȘ ÌÂϤÙ˜ ÂÓËϛΈÓ, Ô ¢ª™

‰ÂÓ ÌÔÚ› Ó· ‰È·¯ˆÚ›ÛÂÈ ÙË ÏÈÒ‰Ë ·fi ÙË ÌË ÏÈ-

Ò‰Ë Ì¿˙·. °È· ·Ú¿‰ÂÈÁÌ·, ÁÓˆÚ›˙Ô˘Ì fiÙÈ ÁÂÓÈο

ÙÔ ÔÛÔÛÙfi Ï›Ô˘˜ Â›Ó·È ˘„ËÏfiÙÂÚÔ ÛÙ· ı‹Ï· ·fi

fi,ÙÈ ÛÙ· ¿ÚÚÂÓ· Ì ÙÔÓ ›‰ÈÔ ¢ª™. ¶Ôχ ÂÚÈÛÛfiÙÂ-

ÚÔ, ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ Ë ·ÍÈÔÏfiÁËÛË Ì¤Ûˆ

ÙÔ˘ ¢ª™ ‰ÂÓ Ï·Ì‚¿ÓÂÈ ˘’ fi„Ë ÙËÓ Â›‰Ú·ÛË Ù˘

·‡ÍËÛ˘ Î·È Ù˘ ÂÊ˂›·˜, Ô‡Ù ÙȘ Ê˘ÛÈÔÏÔÁÈΤ˜

·ÏÏ·Á¤˜ ÛÙË Û‡ÛÙ·ÛË ÛÒÌ·ÙÔ˜. ∂ÈÚfiÛıÂÙ·, Ë ËÏÈ-

Λ· ¤Ó·Ú͢ Ù˘ ÂÊ˂›·˜ Î·È ÔÈ Û¯ÂÙÈ˙fiÌÂÓ˜ Ì ·˘-

Ù‹ ÌÂÙ·‚ÔϤ˜ ÔÈΛÏÏÔ˘Ó ÌÂٷ͇ ‰È·ÊfiÚˆÓ ÂıÓÔÏÔ-

ÁÈÎÒÓ ÔÌ¿‰ˆÓ. ∆¤ÏÔ˜, Ù· fiÚÈ· ÙÔ˘ ¢ª™ Ô˘ ÔÚ›˙Ô˘Ó

ÙËÓ ·¯˘Û·ÚΛ· Î·È ÙÔ ˘¤Ú‚·ÚÔ ÛÙ· ·È‰È¿ ‰ÂÓ

¤¯ÂÈ ·Ô‰Âȯı› fiÙÈ Û¯ÂÙ›˙ÔÓÙ·È Ì ‚ÈÔÏÔÁÈÎfi ΛӉ˘-

ÓÔ Î·Ú‰ÈÔÌÂÙ·‚ÔÏÈÎÒÓ ÂÈÏÔÎÒÓ Û¯ÂÙÈ˙fiÌÂÓˆÓ ÌÂ

ÙËÓ ·¯˘Û·ÚΛ· ÛÙËÓ ÂÓ‹ÏÈÎË ˙ˆ‹ (12,13).

H ÂÚ›ÌÂÙÚÔ˜ ̤Û˘

H ÂÚ›ÌÂÙÚÔ˜ ̤Û˘ (¶ª) ¤¯ÂÈ ÚÔÙ·ı› ·fi

ÔÏÏÔ‡˜ ÂÚ¢ÓËÙ¤˜ ˜ ¤Ó·˜ ÂÚÈÛÛfiÙÂÚÔ ·ÍÈfiÈÛÙÔ˜

‰Â›ÎÙ˘ ÙÔ˘ ÂȤ‰Ô˘ ÙÔ˘ ÂÓ‰ÔÎÔÈÏÈ·ÎÔ‡ Ï›Ô˘˜ ηÈ

·ÔÙÂÏ› ·Ú¿ÁÔÓÙ· ηډÈÔÌÂÙ·‚ÔÏÈÎÔ‡ ÎÈÓ‰‡ÓÔ˘

ÛÙÔ˘˜ ÂÓ‹ÏÈΘ. ∏ ÛÔ˘‰·ÈfiÙËÙ· Ù˘ ̤ÙÚËÛ˘ ·˘-

Ù‹˜ Ê·›ÓÂÙ·È ·fi ÙË ¯Ú‹ÛË Ù˘, ·ÓÙ› ÙÔ˘ ¢ª™, ÛÙÔÓ

ÔÚÈÛÌfi ÙÔ˘ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ,

Û‡Ìʈӷ Ì ÙËÓ ∂ıÓÈ΋ √Ì¿‰· ∂ÈÌfiÚʈÛ˘ ÁÈ· ÙË

ÃÔÏËÛÙÂÚfiÏË (National Cholesterol Education Panel,

NCEP) Î·È ÙËÓ ¶·ÁÎfiÛÌÈ· √ÌÔÛÔÓ‰›· ¢È·‚‹ÙË

(International Diabetes Federation, IDF) (15). AÎfiÌË

ÂÚÈÛÛfiÙÂÚÔ, Ë ¯Ú‹ÛË Ù˘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘, ·ÓÙ›

ÙÔ˘ ¢ª™, ÂÂÎÙ¿ıËΠÚfiÛÊ·Ù· ÛÙËÓ ·È‰È΋ ηÈ

ÂÊË‚È΋ ËÏÈΛ·, Ì ·ÔÙ¤ÏÂÛÌ· ÙË Û‡ÌʈÓË ‰‹Ïˆ-

ÛË (consensus statement) ÂÚ¢ÓËÙÒÓ Ó· ¯ÚËÛÈÌÔÔÈ-

Â›Ù·È ÛÙËÓ ·È‰È΋ ·¯˘Û·ÚΛ· Ë ÂÚ›ÌÂÙÚÔ˜ ̤Û˘

≥90˘ ∂£, ·ÓÙ› ÙÔ˘ ¢ª™, ÛÙÔÓ ÔÚÈÛÌfi ÙÔ˘ ÌÂÙ·‚ÔÏÈ-

ÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ ÛÙ· ·È‰È¿, Û‡Ìʈӷ Ì ÙËÓ ÚfiÙ·-

ÛË ÙÔ˘ πDF (16).

¶·ÚfiÙÈ ‰ÂÓ Â›Ó·È ··ÁÔÚ¢ÙÈÎfi Ó· ¯ÚËÛÈÌÔÔÈ‹-

ÛÂÈ Î·Ó›˜ ÙȘ η̇Ϙ Ô˘ ¤¯Ô˘Ó ·Ó·Ù˘¯ı› ÛÂ

¿ÏϘ ¯ÒÚ˜, Ë ÂÈÏÔÁ‹ ·˘Ù‹ Ú¤ÂÈ Ó· Â›Ó·È Ë ÙÂ-

ÏÂ˘Ù·›· Î·È Ó· Á›ÓÂÙ·È ÌfiÓÔ fiÙ·Ó ‰ÂÓ ˘¿Ú¯Ô˘Ó

ηıfiÏÔ˘ ‰È·ı¤ÛÈÌ· ÂÓ·ÏÏ·ÎÙÈο ÛÙÔȯ›· Ô˘ Ó·

ÚÔ¤Ú¯ÔÓÙ·È ·fi ÂıÓÈÎfi ÏËı˘ÛÌfi ·Ó·ÊÔÚ¿˜. °È·

ÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘ÛÌfi, ‰ËÌÔÛȇÙËÎ·Ó ÚfiÛÊ·Ù·

141√ÚÈÛÌfi˜ Ù˘ ·È‰È΋˜ Î·È ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜

¶·È‰È·ÙÚÈ΋ 2009;72:141-000

¶›Ó·Î·˜ 1. √ÚȷΤ˜ ÙÈ̤˜ ‰Â›ÎÙË Ì¿˙·˜ ÛÒÌ·ÙÔ˜ (¢ª™) ÁÈ·˘¤Ú‚·Ú· Î·È ·¯‡Û·Úη ·È‰È¿ Î·È ÂÊ‹‚Ô˘˜, ·ÓÙ›ÛÙÔȯ˜ Ì ÙȘÔÚȷΤ˜ ÙÈ̤˜ ¢ª™ ÙˆÓ 25 kg/m2 Î·È ÙˆÓ 30 kg/m2 Ô˘ ¤¯Ô˘Ó ÔÚÈ-Ûı› ÁÈ· ÙÔ˘˜ ÂÓ‹ÏÈΘ (11).

∏ÏÈΛ· À¤Ú‚·ÚoÈ ¶·¯‡Û·ÚÎÔÈ(¯ÚfiÓÈ·) ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ· ∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·

2 18,4 18,0 20,1 19,82,5 18,1 17,8 19,8 19,63 17,9 17,6 19,6 19,43,5 17,7 17,4 19,4 19,24 17,6 17,3 19,3 19,24,5 17,5 17,2 19,3 19,15 17,4 17,2 19,3 19,25,5 17,5 17,2 19,5 19,36 17,6 17,3 19,8 19,76,5 17,7 17,5 20,2 20,17 17,9 17,8 20,6 20,57,5 18,2 18,0 21,1 21,08 18,4 18,4 21,6 21,68,5 18,8 18,7 22,2 22,29 19 19,1 22,8 22,89,5 19,5 19,5 23,4 23,510 19,8 19,9 24,0 24,110,5 20,2 20,3 24,6 24,811 20,6 20,7 25,1 25,411,5 20,9 21,2 25,6 26,112 21,2 21,7 26,0 26,712,5 21,6 22,1 26,4 27,213 21,9 22,6 26,8 27,813,5 22,3 23,0 27,3 28,214 22,6 23,3 27,6 28,614,5 23 23,7 28,0 28,915 23,3 23,9 28,3 29,115,5 23,6 24,2 28,6 29,316 23,9 24,4 28,9 29,416,5 24,2 24,5 29,1 29,617 24,5 24,7 29,4 29,717,5 24,7 24,9 29,7 29,818 25 25,0 30,0 30,0

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·141

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η̇Ϙ ÁÈ· ÙËÓ ÂÚ›ÌÂÙÚÔ Ì¤Û˘ Û ·È‰È¿ ηÈ

ÂÊ‹‚Ô˘˜ Ù˘ ∫Ú‹Ù˘ (17). ™ÙÔ ¿ÚıÚÔ ·˘Ùfi, ·Ú¤¯Ô-

ÓÙ·È ›Ó·Î˜ Ì ÙȘ ∂£ ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, Ô˘

ÌÔÚÔ‡Ó Ó· ÚËÛÈÌÔÔÈËıÔ‡Ó Û ÎÏÈÓÈ΋ Ú‹ÛË, Ì·-

˙› Ì ÙÔÓ ¢ª™, ÁÈ· ·È‰È¿ Ù˘ ∫Ú‹Ù˘. ¶·Ú¤¯ÂÙ·È

›Û˘ ›Ó·Î·˜ Ì ÙË Ì¤ÛË ÙÈÌ‹ Î·È ÙË ÛÙ·ıÂÚ‹

·fiÎÏÈÛË ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, Ô˘ ÌÔÚ› Ó· ¯ÚË-

ÛÈÌÔÔÈËı› ÁÈ· ÙÔÓ ÈÔ ·ÎÚÈ‚‹ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ z-

score Ù˘ ¶ª ·ÔÎÏÂÈÛÙÈο Û ·È‰È¿ Ù˘ ∫Ú‹Ù˘.

øÛÙfiÛÔ, Ë ¤ÏÏÂÈ„Ë ‰ËÌÔÛÈÂ˘Ì¤ÓˆÓ ÛÙÔȯ›ˆÓ ÁÈ·

ÙËÓ ¶ª ·fi ¿ÏϘ ÂÚÈÔ¯¤˜ Ù˘ ∂ÏÏ¿‰·˜ ·Ó·‚¿ÏÏÂÈ

ÙË Ú‹ÛË Ù˘ ¶ª ÛÙÔ Û‡ÓÔÏÔ ÙÔ˘ ÂÏÏËÓÈÎÔ‡ ÏËı˘-

ÛÌÔ‡ ÁÈ· ÙÔ Ì¤ÏÏÔÓ.

ÕÏÏÔÈ ‰Â›ÎÙ˜ ·¯˘Û·ÚΛ·˜

∏ ̤ÙÚËÛË ÙÔ˘ ¿¯Ô˘˜ ÙˆÓ ‰ÂÚÌ·ÙÔÙ˘¯ÒÓ

·ÔÙÂÏ› ÌÈ· ·Ï‹ ̤ıÔ‰Ô ·ÍÈÔÏfiÁËÛ˘ Ù˘ ηٷ-

ÓÔÌ‹˜ ÙÔ˘ Ï›Ô˘˜, ÁÈ·Ù› ÌÔÚ› Ó· ¯ÚËÛÈÌÔÔÈËı›

Û ÔÏÏ¿ ̤ÚË ÙÔ˘ ÛÒÌ·ÙÔ˜. ∏ ̤ÙÚËÛË Ù˘ ‰ÂÚÌ·-

ÙÔÙ˘¯‹˜ ÙÔ˘ ‰ÈÎÂÊ¿ÏÔ˘ ıˆÚÂ›Ù·È fiÙÈ Û¯ÂÙ›˙ÂÙ·È

ηϿ Ì ÙË ÏÈÒ‰Ë Ì¿˙·. ∏ ̤ıÔ‰Ô˜ ·˘Ù‹ ÌÔÚ› Ó·

·Ó··Ú·¯ı› ‡ÎÔÏ·, ÏfiÁˆ ÙˆÓ Ì¤ÙÚÈˆÓ Ù¯ÓÈÎÒÓ

ÈηÓÔÙ‹ÙˆÓ Ô˘ ··ÈÙ›, ·ÏÏ¿ ·ÚÔ˘ÛÈ¿˙ÂÈ ÌÂÁ¿ÏË

‰È·Î‡Ì·ÓÛË ·Ó¿ÌÂÛ· Û ‰È¿ÊÔÚÔ˘˜ ÂÍÂÙ·ÛÙ¤˜ ηÈ

‰ÂÓ ıˆÚÂ›Ù·È ·ÎÚÈ‚‹˜ (18).

√ ÏfiÁÔ˜ Ù˘ ÂÚÈ̤ÙÚÔ˘ ̤Û˘ ÚÔ˜ ÙËÓ ÂÚ›ÌÂ-

ÙÚÔ ÁÔÊÒÓ (Waist to Hip Ratio, W/H) ¯ÚËÛÈÌÔÔÈ›-

Ù·È ÁÈ· ÙËÓ ·ÍÈÔÏfiÁËÛË Ù˘ ηٷÓÔÌ‹˜ ÙÔ˘ ۈ̷ÙÈ-

ÎÔ‡ Ï›Ô˘˜ ÛÙÔ˘˜ ÂÓ‹ÏÈΘ. ∏ ¯ÚËÛÈÌfiÙËÙ· ÙÔ˘ ‰Â›-

ÎÙË ·˘ÙÔ‡ Î·È Ë Û¯¤ÛË ÙÔ˘ Ì ÙÔÓ ÌÂÏÏÔÓÙÈÎfi ηÚ-

‰È·ÁÁÂÈ·Îfi ΛӉ˘ÓÔ ‰ÂÓ Â›Ó·È ÍÂοı·Ú˜ ÛÙËÓ ·È‰È-

΋ ËÏÈΛ·. ∂›Û˘, Â›Ó·È ‰‡ÛÎÔÏÔ Ó· ÂÎÙÈÌËıÔ‡Ó ÌÂ-

Ù·‚ÔϤ˜ ÛÙÔ˘˜ ‰Â›ÎÙ˜ ·˘ÙÔ‡˜ ÛÙËÓ ·È‰È΋ ηÈ

ÂÊË‚È΋ ËÏÈΛ·, ÏfiÁˆ ÙˆÓ ·ÏÏ·ÁÒÓ Ô˘ Û˘Ì‚·›ÓÔ˘Ó

ηٿ ÙËÓ ·‡ÍËÛË Î·È ÙËÓ ÂÊ˂›·. ∆¤ÏÔ˜, ‰ÂÓ ¤¯Ô˘Ó

·Ó·Ù˘¯ı› η̇Ϙ ÁÈ· ÙËÓ ÂÚ›ÌÂÙÚÔ ÁÔÊÒÓ ‹

ÁÈ· ÙÔÓ ÏfiÁÔ W/H ÛÙ· ·È‰È¿ (18).

√È ÔÚÈÛÌÔ› Â‰Ò Î·È ÙÒÚ·: ÚËÛÈÌÔÔÈÒÓÙ·˜ Ù·

˘¿Ú¯ÔÓÙ· ÂÏÏËÓÈο ‰Â‰Ô̤ӷ ÛÙËÓ Ú¿ÍË

√È Î·Ì‡Ï˜ ÙÔ˘ ¢ª™ ÁÈ· ÙÔÓ ÂÏÏËÓÈÎfi ÏËı˘-

ÛÌfi Ô˘ ‰ËÌÔÛȇÙËÎ·Ó ÙÔ 2004 (19) ·ÔÙÂÏÔ‡Ó ÙÔ

ÈÔ Û‡Á¯ÚÔÓÔ ÂÚÁ·ÏÂ›Ô ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi Ù˘ ·-

¯˘Û·ÚΛ·˜ ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜, ÙfiÛÔ ÛÂ

Â›Â‰Ô Ì·˙È΋˜ ·Ó›¯Ó¢Û˘ ÙˆÓ ·È‰ÈÒÓ Ì ·˘ÍË-

̤ÓÔ Î›Ó‰˘ÓÔ ÁÈ· ηډÈÔÌÂÙ·‚ÔÏÈΤ˜ ÂÈÏÔΤ˜

fiÛÔ Î·È ÁÈ· ÙËÓ ·Ú·ÎÔÏÔ‡ıËÛË ÌÂÌÔӈ̤ӈÓ

·ÛıÂÓÒÓ. ™ÙË ÌÂϤÙË ·˘Ù‹ ÌÂÙÚ‹ıËÎ·Ó 10.925 ¿ÙÔ-

Ì· ËÏÈΛ·˜ 0-18 ÂÙÒÓ, 5.537 ·ÁfiÚÈ· Î·È 5.388 ÎÔÚ›-

ÙÛÈ·, Ì ÂÚ›Ô˘ ÈÛfiÙÈÌË Î·Ù·ÓÔÌ‹ ÛÙȘ ‰È¿ÊÔÚ˜

ËÏÈ˘. ∆· ¿ÙÔÌ· Ô˘ ÌÂÙÚ‹ıËÎ·Ó ‹Ù·Ó ·fi ÙËÓ

ÂÚÈÔ¯‹ Ù˘ ∞ı‹Ó·˜ Î·È ‹Ù·Ó ÂÏÏËÓÈ΋˜ ηٷÁˆÁ‹˜

Î·È ·fi ÙÔ˘˜ ‰‡Ô ÁÔÓ›˜. ∏ ÂÚÈÔ¯‹ Ù˘ ∞ı‹Ó·˜ ‰ÂÓ

ηχÙÂÈ fiÏË ÙËÓ ∂ÏÏ¿‰·, fï˜ ÙfiÛÔ ·fi ÏÂ˘Ú¿˜

·Ó·ÏÔÁ›·˜ ÏËı˘ÛÌÔ‡ (ÂÚ›Ô˘ Ô ÌÈÛfi˜ ÏËı˘-

ÛÌfi˜ Ù˘ ¯ÒÚ·˜) fiÛÔ Î·È ·fi ÏÂ˘Ú¿˜ ÁÂÓÂÙÈÎÔ‡

˘ÔÛÙÚÒÌ·ÙÔ˜ (¿ÙÔÌ· Ì ÔÈΛÏË ÚԤϢÛË),

Ê·›ÓÂÙ·È fiÙÈ ÔÈ Î·Ì‡Ï˜ Ì ÚԤϢÛË ÙËÓ Â˘Ú‡-

ÙÂÚË ÂÚÈÔ¯‹ Ù˘ ∞ı‹Ó·˜ ·ÔÙÂÏÔ‡Ó ÈηÓÔÔÈËÙÈ-

ο ·ÓÙÈÚÔÛˆÂ˘ÙÈÎfi ‰Â›ÁÌ· ÙÔ˘ ÏËı˘ÛÌÔ‡.

øÛÙfiÛÔ, ÙfiÛÔ Ë ÚԤϢÛË ·fi ÂıÓÈÎfi ÏËı˘ÛÌfi

fiÛÔ Î·È Ë ÚfiÛÊ·ÙË Î·Ù·ÁÚ·Ê‹ ·ÔÙÂÏÔ‡Ó ‰‡Ô

ÈÛ¯˘Ú¿ ÛÙÔȯ›· ˘¤Ú Ù˘ ¯Ú‹Û˘ ÙˆÓ Î·Ì˘ÏÒÓ

·Ó·ÊÔÚ¿˜ ÙÔ˘ 2004 ÁÈ· ÙÔÓ ÔÚÈÛÌfi Ù˘ ·È‰È΋˜ ·-

¯˘Û·ÚΛ·˜ ÛÙËÓ ∂ÏÏ¿‰·.

™‡Ìʈӷ Ì ·˘Ù¤˜, Ë 97Ë ∂£ Î·È Ë 50Ë ∂£ ÛÂ

·ÁfiÚÈ· 18 ÂÙÒÓ Ù˘ ÂÚÈÔ¯‹˜ Ù˘ ∞ı‹Ó·˜ ·ÓÙÈÛÙÔȯ›

Û‹ÌÂÚ· Û ‚¿ÚÔ˜ Ô˘ Â›Ó·È Î·Ù¿ 15 kg Î·È Î·Ù¿ 6 kg,

·ÓÙ›ÛÙÔȯ·, ÌÂÁ·Ï‡ÙÂÚÔ ·fi ÙÔ ‚¿ÚÔ˜ ÙˆÓ ı¤ÛˆÓ

·˘ÙÒÓ ÚÈÓ 20 ÂÚ›Ô˘ ¤ÙË. ŒÙÛÈ, Ë ¯ÚËÛÈÌÔÔ›ËÛË

Ù˘ ÛËÌÂÚÈÓ‹˜ 97˘ ∂£ ˆ˜ ÎÚÈÙËÚ›Ô˘ ÁÈ· ÙÔÓ ÔÚÈÛÌfi

Ù˘ ·¯˘Û·ÚΛ·˜ ˘ÔÂÎÙÈÌ¿ ÙfiÛÔ Ù· ÔÛÔÛÙ¿ ·¯˘-

Û·ÚΛ·˜ ÛÙÔÓ ÏËı˘ÛÌfi fiÛÔ Î·È ÙËÓ ·Ó›¯Ó¢ÛË ·È-

‰ÈÒÓ Û ΛӉ˘ÓÔ, Û ۇÁÎÚÈÛË Ì ٷ ‰Â‰Ô̤ӷ Ù˘

ÂÚÈfi‰Ô˘ 1978 Î·È Ì ٷ ‰Â‰Ô̤ӷ ¿ÏÏˆÓ ¯ˆÚÒÓ. °È·

ÙÔ ÏfiÁÔ ·˘Ùfi, Î·È Ì ÙÔ ÛÎÂÙÈÎfi Ù˘ ·Ó›¯Ó¢Û˘ Â-

ÚÈÛÛfiÙÂÚˆÓ ·È‰ÈÒÓ Û ΛӉ˘ÓÔ, ÛÙËÓ ›‰È· ‰ËÌÔÛ›Â˘-

ÛË ÚÔÙ›ÓÂÙ·È Ë ·Ó¿ÏÔÁË Ì ٷ ÎÚÈÙ‹ÚÈ· ÙÔ˘ IOTF

ÚÔ¤ÎÙ·ÛË ÙˆÓ ı¤ÛÂˆÓ 25 Î·È 30 ÙˆÓ ÂÓËÏ›ÎˆÓ (∂È-

ÎfiÓ· 1) ˆ˜ ı¤ÛË ÔÚ›Ô˘ ÁÈ· ÙÔÓ ÔÚÈÛÌfi ÙÔ˘ ˘¤Ú‚·-

ÚÔ˘ Î·È ÙÔ˘ ·¯‡Û·ÚÎÔ˘ ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹-

‚Ô˘˜. ∂›Ó·È ÚÔÊ·Ó¤˜ ˆ˜ Ô ÔÚÈÛÌfi˜ ·˘Ùfi˜ ÚÔÙ›-

ÓÂÙ·È ÁÈ· ÙËÓ ∂ÏÏ¿‰· ÙÔ˘ Û‹ÌÂÚ·, ÁÈ·Ù› Û ¿ÏÏÔ˘˜

ÂıÓÈÎÔ‡˜ ÏËı˘ÛÌÔ‡˜ Ë ·fiÎÏÈÛË ·fi ÙË Ì¤ÛË ÙÈÌ‹

ÌÔÚ› Ó· ¤¯ÂÈ Ê·Ó› ÂÚÈÛÛfiÙÂÚÔ ·ÓÙÈÚÔÛˆÂ˘ÙÈ-

΋ ÙÔ˘ ˘¤Ú‚·ÚÔ˘ Î·È Ù˘ ·¯˘Û·ÚΛ·˜.

™˘ÌÂÚ¿ÛÌ·Ù·

™˘ÌÂÚ·ÛÌ·ÙÈο, Ë Ú‹ÛË ÙˆÓ ÌÂıfi‰ˆÓ ̤ÙÚËÛ˘

Ù˘ ·¯˘Û·ÚΛ·˜ ÛÙ· ·È‰È¿ Î·È ÙÔ˘˜ ÂÊ‹‚Ô˘˜ ÛÙËÓ

ÎÏÈÓÈ΋ Ú¿ÍË ÂÍ·ÚÙ¿Ù·È ·fi ÙÔ ÂÚÒÙËÌ· Ô˘ ¤¯ÂÈ

ηÓ›˜ Ó· ··ÓÙ‹ÛÂÈ. ™ÙÔ ÂÚÒÙËÌ· ·Ó Â›Ó·È ÙÔ ·È‰›

˘¤Ú‚·ÚÔ ‹ ·¯‡Û·ÚÎÔ (Ô˘ ¤¯ÂÈ Ú·ÎÙÈ΋ ÛËÌ·Û›·:

·Ó Î·È Û ÙÈ ‚·ıÌfi Û˘ÓÈÛÙ¿Ù·È Ó· ‰ÈÂÚ¢ÓËı› ÁÈ· Û¯Â-

ÙÈ˙fiÌÂÓË Ì ÙËÓ ·¯˘Û·ÚΛ· ÓÔÛËÚfiÙËÙ·), Ë ¯Ú‹ÛË

ÙˆÓ ÎÚÈÙËÚ›ˆÓ ÙÔ˘ π√∆F ÌÔÈ¿˙ÂÈ Ó· Â›Ó·È ÔÚıfiÙÂÚË ÁÈ·

Ù· ·È‰È¿ ÂÏÏËÓÈ΋˜ ÚÔ¤Ï¢Û˘ ÙÔ˘ Û‹ÌÂÚ· Î·È Á›ÓÂ-

Ù·È ÛÙËÓ Ú¿ÍË Ì¤Ûˆ ÙˆÓ ÚfiÙ˘ˆÓ ‰È·ÁÚ·ÌÌ¿ÙˆÓ

¢ª™ ÙÔ˘ ÂÏÏËÓÈÎÔ‡ ÏËı˘ÛÌÔ‡ ÙÔ˘ 2004 (11), Ì ÙȘ

ÚÔÂÎÙ¿ÛÂȘ ÙˆÓ ı¤ÛÂˆÓ 25 Î·È 30 ÙÔ˘ ÂÓ‹ÏÈη (∂ÈÎfi-

Ó· 1). ™ÙÔ ÂÚÒÙËÌ· fiÛÔ ·¯‡Û·ÚÎÔ Â›Ó·È ¤Ó· ·È‰›,

ÙÔ z-score, ·Ó Î·È ‚·Û›˙ÂÙ·È ÛÙËÓ ›‰È· ȉ¤· Ì ÙȘ Âη-

ÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ, ·ÔÙÂÏ› Û˘ÁÎÚÈÙÈο ·ÎÚÈ‚¤ÛÙÂÚË

̤ıÔ‰Ô. √ ˘ÔÏÔÁÈÛÌfi˜ ÙÔ˘ z-score ·Ú¤¯ÂÈ ¤Ó·Ó

·ÎÚÈ‚‹ ·ÚÈıÌfi Î·È ÁÈ· Ù· ·È‰È¿ Ô˘ ‚Ú›ÛÎÔÓÙ·È Ôχ

¿Óˆ ·fi ÙËÓ 97Ë ∂£ (ÂÎÙfi˜ ÙˆÓ Î·Ì˘ÏÒÓ) - Ù·

142 ¶. ¶ÂÚ‚·Ó›‰Ô˘

Paediatriki 2009;72:142-000

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·142

Page 71: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

ÔÔ›· ‰˘ÛÙ˘¯Ò˜ Â›Ó·È ÔÏÏ¿. ∂›Û˘, ·Ú¤¯ÂÈ ·ÎÚÈ‚‹

Î·È Û˘ÁÎÚ›ÛÈÌ· ÌÂٷ͇ ÙÔ˘˜ ÛÙÔȯ›· ÁÈ· ÙËÓ ·Ú·ÎÔ-

ÏÔ‡ıËÛË ÙÔ˘ ¢ª™ ·È‰ÈÒÓ Û ·Ú¤Ì‚·ÛË. ∆¤ÏÔ˜, ÛÂ

ÂÚ¢ÓËÙÈÎfi ›‰Ô, ‰›ÓÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ÔÛÔÙÈ΋˜

Û‡ÁÎÚÈÛ˘ ·Ó¿ÌÂÛ· Û ÔÌ¿‰Â˜. √È ›Ó·Î˜ Ì ÙȘ ̤-

Û˜ ÙÈ̤˜ Î·È ÙȘ ÛÙ·ıÂÚ¤˜ ·ÔÎÏ›ÛÂȘ -·fi fiÔ˘ ÌÔ-

Ú› Ó· ˘ÔÏÔÁÈÛÙ› ÙÔ z-score- ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·

ÛÙËÓ ∂ÏÏ¿‰· ÙÔ˘ Û‹ÌÂÚ· ·ÚÔ˘ÛÈ¿˙ÔÓÙ·È ÛÙÔÓ ¶›Ó·-

η 2 (11). ¶·ÚfiÏ· ·˘Ù¿, ηٿ ÙË Ú‹ÛË ÙÔ˘ z-score, ı·

Ú¤ÂÈ Ô ·È‰›·ÙÚÔ˜ Ó· ¤¯ÂÈ ¿ÓÙ· ÛÙÔ ÓÔ˘, Î·È Î·Ù¿

ÂÓË̤ڈÛË ÙˆÓ ÔÈÎÔÁÂÓÂÈÒÓ, fiÙÈ ÌÈÏ¿ÂÈ ÁÈ· ¤Ó·Ó ·ÚÈı-

Ìfi Ô˘ Â›Ó·È Û¯ÂÙÈ˙fiÌÂÓÔ˜ Ì ÙË Ì¤ÛË ÙÈÌ‹ ÙÔ˘ ÏË-

ı˘ÛÌÔ‡ ·Ó·ÊÔÚ¿˜, Ô ÔÔ›Ô˜ ÛÙËÓ ÚÔΛÌÂÓË ÂÚ›-

ÙˆÛË ¤¯ÂÈ ¿ÚÂÈ ·ÓÈÔ‡Û· ÔÚ›·.

µÈ‚ÏÈÔÁÚ·Ê›·

1. WHO: Obesity: Preventing and Managing the Global

Epidemic. Geneva, World Health Organization, 2000.

¶›Ó·Î·˜ 2. ∆È̤˜ ¢ª™ ·Ó¿ ʇÏÔ Î·È ËÏÈΛ·, ÁÈ· ÙÔÓ ˘ÔÏÔÁÈÛÌfi ÙÔ˘ z-score (11)

∞ÁfiÚÈ· ∫ÔÚ›ÙÛÈ·∏ÏÈΛ· ∞ÚÈıÌfi˜ ª¤ÛË ™Ù·ıÂÚ‹ ∏ÏÈΛ· ∞ÚÈıÌfi˜ ª¤ÛË ™Ù·ıÂÚ‹

(¯ÚfiÓÈ·) ÙÈÌ‹ ·fiÎÏÈÛË (¯ÚfiÓÈ·) ÙÈÌ‹ ·fiÎÏÈÛË

1 139 14,8 2,7 1 131 14,7 2,72 114 16,9 1,6 2 111 16,1 1,73 222 15,7 2,3 3 216 15,5 1,64 268 17,7 1,7 4 267 15,6 1,75 256 15,8 1,9 5 256 15,8 2,16 249 16,3 2,2 6 237 16,4 2,57 265 16,9 2,5 7 228 16,7 2,58 282 17,7 4,4 8 248 17,5 3,29 347 18,1 3,1 9 325 18,2 3,1

10 321 18,9 3,5 10 318 18,5 3,111 327 19,3 3,4 11 342 19,1 3,312 379 20,0 3,5 12 409 19,8 3,413 453 20,7 3,7 13 423 20,5 3,714 409 21,4 3,7 14 385 21,1 3,415 443 21,8 3,7 15 428 21,4 3,316 356 22,8 4,0 16 323 21,9 3,417 387 23,1 3,7 17 370 22,1 3,118 317 23,9 4,1 18 371 22,3 3,6

143√ÚÈÛÌfi˜ Ù˘ ·È‰È΋˜ Î·È ÂÊË‚È΋˜ ·¯˘Û·ÚΛ·˜

¶·È‰È·ÙÚÈ΋ 2009;72:143-000

kgr

/m2

kgr

/m2

∏ÏÈΛ· (¤ÙË)0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18

34333231302928272625242322212019181716151413

34333231302928272625242322212019181716151413

∞ÁfiÚÈ·

25

10

3

75

85

95

97

50

(µMI 25)

90 (µMI 30)

kgr

/m2

kgr

/m2

∏ÏÈΛ· (¤ÙË)0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18

33323130292827262524232221201918171615141312

33323130292827262524232221201918171615141312

∫ÔÚ›ÙÛÈ·

25

(µMI 30)

75

50

3

10

90

97

(µMI 25)

∂ÈÎfiÓ· 1. ∂ηÙÔÛÙÈ·›Â˜ ı¤ÛÂȘ Ô˘ ÔÚ›˙Ô˘Ó ÙÔ ˘¤Ú‚·ÚÔ Î·È ÙÔ ·¯‡Û·ÚÎÔ ·È‰›, ·Ó¿ ʇÏÔ, ˆ˜ ÚÔ¤ÎÙ·ÛË ÙˆÓ ÔÚÈ·ÎÒÓ ÛËÌ›ˆÓ ÙˆÓÂÓËϛΈÓ, ÛÙ· ÂÏÏËÓÈο ÚfiÙ˘· ‰È·ÁÚ¿ÌÌ·Ù·. ∞Ó·Ê·›ÓÂÙ·È Ë ∂£ Ô˘ ‰È·¯ˆÚ›˙ÂÈ ÙÔ ·¯‡Û·ÚÎÔ (¢ª™ >30) Î·È ¤Ú‚·ÚÔ (¢ª™ >25)·È‰› (11).

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·143

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2. Lee SY, Gallagher D. Assessment methods in human bodycomposition. Curr Opin Clin Nutr Metab Care 2008Sep;11(5):566-572.

3. Revicki DA, Israel RG. Relationship between body massindices and measures of body adiposity. Am J Publ Health1986;76:992-994.

4. Spiegelman D, Isrel RG, Bouchard C, Willett WC.Absolute fat mass, percent body fat, and body fatdistribution: Which is the real determinant of bloodpressure and serum glucose? Am J Clin Nutr 1992;55:1044-1044.

5. WHO: Physical Status: The use and interpretation ofAnthropometry. Geneva, World Health Organization, 1995.

6. Himes JH, Dietz WH. Guidelines for overweight inadolescent preventive services: recommendations from anexpert committee: The Expert Committee on ClinicalGuidelines for Overweigh in Adolescent PreventiveServices. Am J Clin Nutr 1994;59:307-316.

7. Guillaume M: defining obesity in childhood: currentpractice. Am J Clin Nutr 1999;70:126S-130S.

8. Speiser PW, Rudolf MCJ, Anhalt H, Camacho-Hubner C,Chiarelli F, Eliakim A, et al., on behalf of the ObesityConsensus Working Group. Consensus Statement:Childhood Obesity. JCEM 2005;90:1871-1887.

9. Lahti-Koski M, Gill T. Defining Childhood obesity. In:Obesity in childhood and adolescence / Volume Editors W.Kiess, C. Marcus, M. Wabitsch (Pediatric and adolescentMedicine, vol 9), Karger, 2004, Basel.

10. Bellizzi MC, Dietz WH. Workshop on childhood obesity:Summary of the discussion. Am J Clin Nutr 1999;70:173S-175S.

11. Cole TJ, Bellizzi MC, Flegal KM, Dietz WH. Establishing astandard definition for child overweight and obesityworldwide: International survey. BMJ 2000;320:1240-1243.

12. Pietrobelli A, Faith MS, Allison DB, et al. Body mass indexas a measure of adiposity among children and adolescents:A validation study. J Pediatr 1998;132:204-210.

13. Garn SM, Leonard WR, Hawthorne VM. Three limitationsof the body mass index. Am J Clin Nutr 1986;44:996-997.

14. Higgins PB, Gower BA, Hunter GR, Goran MI. Defininghealth-related obesity in prepubertal children. Obes Res2001;9:233-240.

15. Pervanidou P, Kanaka-Gantenbein C, Chrousos GP.Assessment of metabolic profile in a clinical setting. CurrOpin Clin Nutr Metab Care 2006;9:589-595.

16. Zimmet P, Alberti KG, Kaufman F, Tajima N, Silink M,Arslanian S, et al.; IDF Consensus Group. The metabolicsyndrome in children and adolescents - an IDF consensusreport. Pediatr Diabetes 2007;8:299-306.

17. §ÈÓ·Ú‰¿Î˘ ∂, µ·Ú‰·‚¿˜ ∫, ∫·Ê¿ÙÔ˜ ∞, Î·È Û˘Ó.Paediatriki 2007;70:300-307.

18. ¶ÂÚ‚·Ó›‰Ô˘ ¶, ∫·Ó·Î·-Gantenbein X, ¶··ÛˆÙËÚ›Ô˘ π,ÃÚÔ‡ÛÔ˜ °¶. ¢ÈÂÚ‡ÓËÛË ÙÔ˘ ÌÂÙ·‚ÔÏÈÎÔ‡ Û˘Ó‰ÚfiÌÔ˘ÛÙËÓ ·È‰È΋ Î·È ÂÊË‚È΋ ËÏÈΛ·: Ë ·¯˘Û·ÚΛ· ˆ˜ ΢-ÚÈfiÙÂÚÔ˜ ·ÈÙÈÔÏÔÁÈÎfi˜ ·Ú¿ÁÔÓÙ·˜. ¢ÂÏÙ ∞ã ¶·È‰È·ÙÚ∫ÏÈÓ ¶·Ó ∞ıËÓÒÓ 2007;54:41-52.

19. Chiotis D, Krikos X, Tsiftis G, Hatzisymeaon M, Maniati-Christidi M, Dacou-Voutetakis A. Body mass index andprevalence of obesity in subjects of Hellenic origin aged 0-18years, living in the Athens area. Ann Cli Pediatr UniveAtheniensis 2004;51:139-154.

144 ¶. ¶ÂÚ‚·Ó›‰Ô˘

Paediatriki 2009;72:23-28

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™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek ηÈ

ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘

Ã. ∆۷ηϛ‰Ë˜1, µ. §·ÌÚfiÔ˘ÏÔ˜2, Œ. √˘ÚÁηÓÙ˙fiÁÏÔ˘3, ¢. ƒ¿ÏÏ˘4, ∞. ¶ÂÙÚfiÔ˘ÏÔ˜2, ¡. ¡ÈÎÔÏ·˝‰Ë˜1

¶ÂÚ›ÏË„Ë: ∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Â›Ó·È ‰˘ÛÏ·Û›· ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È Â›ÛÔ‰Ô˜ ÂÓ-‰ÔÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ ̤۷ ÛÙÔÓ ıÒڷη, ‰È·Ì¤ÛÔ˘ ÂÓfi˜ ÂÏÏ›ÌÌ·ÙÔ˜ ÂÌ‚Ú˘ÔÁ¤ÓÂÛ˘ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·-ÙÔ˜, Î·È ÌÔÚ› Ó· ·ÊÔÚ¿ ‰È·ÊÔÚÂÙÈο ÙÌ‹Ì·Ù· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. √ ÈÔ ÎÔÈÓfi˜ Ù‡Ô˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋-Ï˘ Â›Ó·È Ë ÔÈÛıÔÏ¿ÁÈ· ΋ÏË ÙÔ˘ Bochdalek. √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ Â›Ó·È Û¿ÓÈ· ·ÓˆÌ·Ï›·Î·È ·ÓÙÈÚÔۈ‡ÂÈ ÏÈÁfiÙÂÚÔ ·fi 5% fiÏˆÓ ÙˆÓ ÓÂÊÚÈÎÒÓ ÂÎÙÔÈÒÓ. ªÔÚ› Ó· ÂÌÊ·Ó›˙ÂÙ·È ¯ˆÚ›˜ ¿ÏÏÂ˜Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ‹ Ó· Û¯ÂÙ›˙ÂÙ·È ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÌÂÙ¿ ·fi ÙÚ·˘Ì·-ÙÈÛÌfi. ¶ÂÚÈÁÚ¿ÊÔ˘Ì ÙËÓ ÂÚ›ÙˆÛË ÂÓfi˜ ¿ÚÚÂÓÔ˜ ÓÂÔÁÓÔ‡ ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Ù‡Ô˘Bochdalek Î·È Û˘ÓÔ‰fi ¤ÎÙÔÔ ÓÂÊÚfi ÛÙÔ ¿Óˆ ·ÚÈÛÙÂÚfi ËÌÈıˆÚ¿ÎÈÔ.

§¤ÍÂȘ ÎÏÂȉȿ: ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË, ÓÂÔÁÓfi.

Bochdalek congenital diaphragmatic hernia with intrathoracic

ectopic kidney - a case report

C. Tsakalidis1, V. Lampropoulos2, E. Ourgantzoglou3, D. Rallis4, A. Petropoulos2, N. Nikolaidis1

Abstract: Congenital diaphragmatic hernia (CDH) is an abnormality in which abdominal organsenter the thorax through a developmental defect of the diaphragm. The hernia may occur in differentparts of the diaphragm, but the most common type of diaphragmatic hernia is the posterolateralBochdalek hernia. Intrathoracic ectopic kidney is a rare abnormality accounting for less than 5% of allrenal ectopia. It can occur with no other malformation or can be associated with congenitaldiaphragmatic hernia or post-traumatic diaphragmatic hernia. The case is presented of a maleneonate with a congenital diaphragmatic Bochdalek hernia, associated with renal ectopy in the leftupper hemithorax.

Key worlds: Congenital diaphragmatic hernia, neonate.

145∂¡¢π∞º∂ƒ√À™∞ ¶∂ƒπ¶∆ø™∏ CASE REPORT

1 µ’ ¡ÂÔÁÓÔÏÔÁÈ΋ ∫ÏÈÓÈ΋∞¶£, ¡ÔÛÔÎÔÌ›Զ··ÁˆÚÁ›Ô˘,£ÂÛÛ·ÏÔÓ›ÎË

2 µ’ ¶·È‰Ô¯ÂÈÚÔ˘ÚÁÈ΋∫ÏÈÓÈ΋ ∞¶£, ¡ÔÛÔÎÔÌ›Զ··ÁˆÚÁ›Ô˘,£ÂÛÛ·ÏÔÓ›ÎË

3 µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋∞¶£, ¡ÔÛÔÎÔÌÂ›Ô ∞Ã∂¶∞,£ÂÛÛ·ÏÔÓ›ÎË

4 ¡ÔÛÔÎÔÌÂ›Ô ∂ȉÈÎÒÓ¶·ı‹ÛˆÓ, £ÂÛÛ·ÏÔÓ›ÎË

AÏÏËÏÔÁÚ·Ê›·:

ÃÚ‹ÛÙÔ˜ ∆۷ηϛ‰Ë˜[email protected]¡ÔÛÔÎÔÌÂ›Ô «¶··ÁˆÚÁ›Ô˘»,£ÂÛÛ·ÏÔÓ›ÎË

1 2nd Department ofNeonatal Paediatrics,“Aristotelio” University ofThessaloniki,“Papageorgiou” Hospital,Thessaloniki, Greece

2 2nd Department ofPaediatric Surgery,“Aristotelio” University ofThessaloniki,“Papageorgiou” Hospital,Thessaloniki, Greece

3 2nd Department ofPaediatrics, “Aristotelio”University of Thessaloniki,“AHEPA” Hospital,Thessaloniki, Greece

4 Infectious DiseasesHospital, Thessaloniki,Greece

Correspondence:

Christos [email protected]“Papageorgiou” Hospital,Thessaloniki, Greece

¶·È‰È·ÙÚÈ΋ 2009;72:29-40

∂ÈÛ·ÁˆÁ‹

ŒÎÙÔÔ˜ ÓÂÊÚfi˜ ··ÓÙ¿Ù·È Û 1:1.000 ÁÂÓ-Ó‹ÛÂȘ, ·ÏÏ¿ ÌfiÓÔ 1 ÛÙȘ 10 ·fi ·˘Ù¤˜ ÙȘ ÂÚÈ-ÙÒÛÂȘ ‰È·ÁÈÁÓÒÛÎÂÙ·È (1). √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜¤ÎÙÔÔ˜ ÓÂÊÚfi˜ ÂÌÊ·Ó›˙ÂÙ·È ·ÎfiÌË ÈÔ Û¿ÓÈ·Î·È ¤¯ÂÈ ÙË ÌÈÎÚfiÙÂÚË Û˘¯ÓfiÙËÙ· Û ۯ¤ÛË ÌÂÙȘ ÏÔȤ˜ ÂÎÙԛ˜ ÙÔ˘ Ô˘ÚÔÔÈËÙÈÎÔ‡ (2,3),Â›Ó·È Î·Ù¿ ηÓfiÓ· ·Û˘Ìو̷ÙÈÎfi˜ Î·È ·ÔÙÂ-Ï› Ù˘¯·›Ô ‡ÚËÌ· ÛÙËÓ ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›·ıÒÚ·ÎÔ˜. ∞ÎfiÌ· Û·ÓÈfiÙÂÚË Â›Ó·È Ë ‰È¿ÁÓˆÛËÙ˘ ‡·Ú͢ ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚԇηٿ ÙË ÓÂÔÁÓÈ΋ ËÏÈΛ·. ¶ÂÚÈÁÚ¿ÊÔ˘Ì ÙËÓÂÚ›ÙˆÛË ÂÓfi˜ ¿ÚÚÂÓÔ˜ ÓÂÔÁÓÔ‡ ÌÂ Û˘ÁÁÂÓ‹‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Ù‡Ô˘ Bochdalek ηÈÛ˘ÓÔ‰fi ¤ÎÙÔÔ ÓÂÊÚfi ÛÙÔ ¿Óˆ ·ÚÈÛÙÂÚfi ËÌÈ-ıˆÚ¿ÎÈÔ, Ë ‰È¿ÁÓˆÛË ÙˆÓ ÔÔ›ˆÓ ¤ÁÈÓ ÚÔÁÂÓ-ÓËÙÈο, Û ËÏÈΛ· ·ËÛ˘ 32 ‚‰ÔÌ¿‰ˆÓ.

¶ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘

¶ÂÚÈÁÚ¿ÊÂÙ·È Ë ÂÚ›ÙˆÛË ¿ÚÚÂÓÔ˜ ÓÂÔ-

ÁÓÔ‡ Ô˘ ÁÂÓÓ‹ıËΠÌÂÙ¿ ·fi ·ËÛË ‰È¿ÚÎÂÈ·˜

34 ‚‰ÔÌ¿‰ˆÓ Ì ηÈÛ·ÚÈ΋ ÙÔÌ‹, ÏfiÁˆ ÚÔ-

ÁÂÓÓËÙÈ΋˜ ‰È¿ÁÓˆÛ˘ ™¢∫, ÓÂÊÚÈ΋˜ ÂÎÙÔ›·˜

Î·È ÔÏ˘·ÌÓ›Ô˘.

¶ÚfiÎÂÈÙ·È ÁÈ· ÙÔ ÚÒÙÔ ·È‰› Ê·ÈÓÔÙ˘Èο

˘ÁÈÒÓ, ÌË Û˘ÁÁÂÓÒÓ ÌÂٷ͇ ÙÔ˘˜ ÁÔÓ¤ˆÓ, ÌÂ

ÔÈÎÔÁÂÓÂÈ·Îfi ÈÛÙÔÚÈÎfi ÂχıÂÚÔ. ∆Ô ‚¿ÚÔ˜

Á¤ÓÓËÛ˘ ‹Ù·Ó 2770 g (50Ë-75Ë ∂£). ∞fi ÙÔ

ÂÚÈÁÂÓÓËÙÈÎfi ÈÛÙÔÚÈÎfi ·Ó·Ê¤ÚÂÙ·È ˆ˜ ÛÙÔÓ

˘ÂÚ˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ ÂÌ‚Ú‡Ô˘, Ô˘

¤ÁÈÓ Û ËÏÈΛ·˜ ·ËÛ˘ 32 ‚‰ÔÌ¿‰ˆÓ, ‰È·È-

ÛÙÒıËÎÂ Ë ‡·ÚÍË Û˘ÁÁÂÓÔ‡˜ ‰È·ÊÚ·ÁÌ·ÙÔ-

΋Ï˘ ·ÚÈÛÙÂÚ¿, Ì ›ÛÔ‰Ô ÙÔ˘ ÛÙÔÌ¿¯Ô˘ ηÈ

ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘, ÌÂ Û˘ÓÔ‰fi ·ÚÂÎÙfiÈ-

ÛË Ù˘ ηډȿ˜ ÚÔ˜ Ù· ‰ÂÍÈ¿, ·ÏÏ¿ Î·È Û˘Ó‡-

·ÚÍË ¤ÎÙÔÔ˘ ·ÚÈÛÙÂÚÔ‡ ÓÂÊÚÔ‡ ÛÙÔ Ô›ÛıÈÔ

ÙÌ‹Ì·. √È ‚ÈÔÊ˘ÛÈΤ˜ ·Ú¿ÌÂÙÚÔÈ ÙÔ˘ ÂÌ‚Ú‡-

Ô˘ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈΤ˜.

∏ ÌËÙ¤Ú· Û fiÏË ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÁ΢ÌÔ-

Û‡Ó˘ ‚ÚÈÛÎfiÙ·Ó ˘fi ·ÁˆÁ‹ Ì Û΢¿ÛÌ·Ù·

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·145

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Ûȉ‹ÚÔ˘, ·Û‚ÂÛÙ›Ô˘, Ê˘ÏÏÈÎÔ‡ ÔͤԘ Î·È Ì·ÁÓËÛ›Ô˘.

¢ÂÓ Î¿ÓÈ˙Â Î·È ‰ÂÓ ‹Ù·Ó ¯Ú‹ÛÙ˘ ·ÏÎÔfiÏ ‹ ¿ÏψÓ

Ô˘ÛÈÒÓ. ¶ÚÔËÁ‹ıËΠˆÚ›Ì·ÓÛË ÙˆÓ Ó¢ÌfiÓˆÓ ÙÔ˘

ÂÌ‚Ú‡Ô˘ Ì ¯ÔÚ‹ÁËÛË ÎÔÚÙÈÎÔÛÙÂÚÔÂȉÒÓ ÛÙË ÌË-

Ù¤Ú· Ì›· ‚‰ÔÌ¿‰· ÚÈÓ ·fi ÙÔÓ ÙÔÎÂÙfi.

∏ Ú‹ÍË ÙˆÓ ÂÌ‚Ú˘˚ÎÒÓ ˘Ì¤ÓˆÓ ¤ÁÈÓ ηٿ ÙËÓ

¤̂·ÛË Î·È ÙÔ ·ÌÓÈ·Îfi ˘ÁÚfi ‹Ù·Ó ‰È·˘Á¤˜. ∆Ô ÓÂ-

ÔÁÓfi ÁÂÓÓ‹ıËÎÂ Î·È ¤ÎÏ·„ ·Ì¤Ûˆ˜, ·ÏÏ¿ ·Ì¤Ûˆ˜

ÂÁη٤ÛÙËÛ ·Ó·Ó¢ÛÙÈ΋ ‰˘Û¯¤ÚÂÈ·, Ì ÂÈÛÔÏΤ˜

ÌÂÛÔÏ¢ڛˆÓ ‰È·ÛÙËÌ¿ÙˆÓ, ÁÔÁÁ˘ÛÌfi Î·È Î˘¿Óˆ-

ÛË. ªÂ ÁÓˆÛÙ‹ ÙËÓ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Ù˘

™¢∫, ÙÔÔıÂÙ‹ıËΠ·Ì¤Ûˆ˜ ÛÙÔÌ·ÙÔÁ·ÛÙÚÈÎfi˜ η-

ıÂÙ‹Ú·˜ Î·È ¤ÁÈÓ ‰È·ÛˆÏ‹ÓˆÛË ÁÈ· ¿ÌÂÛË ˘ÔÛÙ‹-

ÚÈÍË ÙÔ˘ ·Ó·Ó¢ÛÙÈÎÔ‡. ∆Ô Apgar score ‹Ù·Ó 17 ηÈ

58 (‰È·ÛˆÏËӈ̤ÓÔ). ∆· ·¤ÚÈ· ÙÔ˘ ÔÌÊ·Ï›Ô˘ ÏÒÚÔ˘

‹Ù·Ó: p∏=7,32, pO2=23,6 mmHg, pCO2=58,6

mmHg, SBE=2,3 mmol/L. ∆Ô ÓÂÔÁÓfi ÌÂٷʤÚıËÎÂ

‰È·ÛˆÏËӈ̤ÓÔ Ì ambu Û ÎÏÂÈÛÙ‹ ıÂÚÌÔÎÔÈÙ›‰·.

∆· ·¤ÚÈ· ·fi ÂÚÈÊÂÚÈ΋ ·ÚÙËÚ›· ÙÔ˘ ÓÂÔÁÓÔ‡ Ï›Á·

ÏÂÙ¿ ÌÂÙ¿ ÙË Á¤ÓÓËÛË, ˘fi Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi,

‹Ù·Ó: pH=7,25, pO2=180,6 mmHg, pCO2=46,8

mmHg, SBE=-7 mmol/L.

∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒڷη ÂȂ‚·›ˆÛ ٷ

Â˘Ú‹Ì·Ù· ÙÔ˘ ÚÔÁÂÓÓËÙÈÎÔ‡ ÂϤÁ¯Ô˘ (∂ÈÎfiÓ· 1).

∏ ÛÙ·ıÂÚ‹ ÎÏÈÓÈ΋ Î·È ÂÚÁ·ÛÙËÚȷ΋ ÂÈÎfiÓ· ÙÔ˘ ÓÂ-

ÔÁÓÔ‡ ¤ÙÚ„ ÙË ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË Ù˘

‰È·ÊÚ·ÁÌ·ÙÔ΋Ï˘ ÙÔ ÚÒÙÔ 24ˆÚÔ, Ì ÙÔÔı¤ÙË-

ÛË Ï¤ÁÌ·ÙÔ˜ Goretex Î·È ÌÂÙ·ÊÔÚ¿ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡

ÓÂÊÚÔ‡ ÛÙËÓ Ô›ÛıÈ· ¿Óˆ ·ÚÈÛÙÂÚ‹ ÎÔÈÏȷ΋ ¯ÒÚ·.

ÃÔÚËÁ‹ıËΠÂÈÊ·ÓÂÈÔ‰Ú·ÛÙÈÎfi˜ ·Ú¿ÁÔÓÙ·˜ ‰‡Ô

146 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

Paediatriki 2009;72:146-000

∂ÈÎfiÓ· 1. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek Î·È ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘. AÍÔÓÈ΋ ÙÔÌÔ-ÁÚ·Ê›· ıÒÚ·ÎÔ˜.

A B

° ¢

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·146

Page 75: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

ÊÔÚ¤˜, ÙËÓ 1Ë Î·È ÙË 12Ë ÒÚ· ˙ˆ‹˜, ·Ì¤Ûˆ˜ ÌÂÙ¿

ÙÔ ¯ÂÈÚÔ˘ÚÁ›Ô. ∏ ¤ÎÙ˘ÍË ÙÔ˘ Ó‡ÌÔÓ· ‹Ù·Ó η-

Ï‹, fï˜ ÏfiÁˆ ‰È·Ù·Ú·¯ÒÓ Ù˘ Û¯¤Û˘ ·ÂÚÈÛÌÔ‡/

·ÈÌ¿ÙˆÛ˘ ·fi ÙË Û˘Ó˘¿Ú¯Ô˘Û· Ó¢ÌÔÓÈ΋

˘ÔÏ·Û›·, ÙÔ ·Ó·Ó¢ÛÙÈÎfi Û‡ÛÙËÌ· ˘ÔÛÙËÚ›-

¯ıËΠ̠˘„›Û˘¯ÓÔ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi, Ì ¯ÔÚ‹ÁË-

ÛË ÌÔÓÔÍÂȉ›Ô˘ ÙÔ˘ ·˙ÒÙÔ˘. ÃÔÚËÁ‹ıËÎ·Ó ÈÓfiÙÚÔ-

· ÁÈ· ÙËÓ ÂÓ›Û¯˘ÛË Ù˘ ÂÚÈÊÂÚÈ΋˜ ΢ÎÏÔÊÔÚ›·˜

Î·È ‰ÈÏ‹ ·ÓÙÈ‚ÈÔÙÈ΋ ·ÁˆÁ‹. ∏ ‰È¿ÁÓˆÛË Ù˘ ·-

ڷ̤ÓÔ˘Û·˜ Ó¢ÌÔÓÈ΋˜ ˘¤ÚÙ·Û˘ ÂȂ‚·ÈÒ-

ıËΠ̠˘ÂÚ˯ÔÁÚ¿ÊËÌ· Doppler ηډȿ˜ ηÈ

Ó¢ÌÔÓÈÎÒÓ ·ÁÁ›ˆÓ.

√ ·ӤÏÂÁ¯Ô˜ ÙË 12Ë Ë̤ڷ ˙ˆ‹˜ ¤‰ÂÈÍ ÛËÌ·-

ÓÙÈ΋ ‚ÂÏÙ›ˆÛË ÛÙȘ ·ÓÙÈÛÙ¿ÛÂȘ ÙˆÓ Ó¢ÌÔÓÈÎÒÓ

·ÁÁ›ˆÓ. ∆Ë 13Ë Ë̤ڷ ÓÔÛËÏ›·˜ ÂΉ‹ÏˆÛ ÂÈÎfiÓ·

ÛË„·ÈÌ›·˜ Ì ·‡ÍËÛË Ù˘ CRP Î·È Ë Î·ÏÏȤÚÁÂÈ· ·›-

Ì·ÙÔ˜ ‹Ù·Ó ıÂÙÈ΋ ÁÈ· St. epidermidis. ∏ ¯ÔÚ‹ÁËÛË

·ÓÙÈ‚ÈÔÙÈ΋˜ ·ÁˆÁ‹˜ ‹Ù·Ó ·ÔÙÂÏÂÛÌ·ÙÈ΋. ∆Ë 17Ë

Ë̤ڷ ÓÔÛËÏ›·˜ ‰È·ÈÛÙÒıËΠÂÎÚÔ‹ ¯ÔÏÒ‰Ô˘˜

˘ÁÚÔ‡ ·fi ÙÔ ¯Â›ÏÔ˜ ÙÔ˘ ÎÔÈÏÈ·ÎÔ‡ ¯ÂÈÚÔ˘ÚÁÈÎÔ‡

ÙÚ·‡Ì·ÙÔ˜ ηÈ, ÏfiÁˆ ÂÌÌÔÓ‹˜, ·ÔÊ·Û›ÛÙËΠÂÚ¢-

ÓËÙÈ΋ Ï··ÚÔÙÔÌ›·, fiÔ˘ Î·È ‰È·ÈÛÙÒıËΠË

‡·ÚÍË, ·ÎÚÈ‚Ò˜ οو ·fi ÙÔ Ï¤ÁÌ·, ‰È·ÙÂٷ̤-

Ó˘ ¤ÏÈη˜ ÏÂÙÔ‡ ÂÓÙ¤ÚÔ˘, Ô˘ ¤ÊÂÚ ÌÔÓ‹ÚË Ú‹-

ÍË ÛÙË ÌÂÛfiÙËÙ¿ Ù˘, ÌÂ Û˘Ú›ÁÁÈÔ ÚÔ˜ ÙÔ ÎÔÈÏÈ·Îfi

ÙÔ›¯ˆÌ·, ·fi fiÔ˘ ÂÍÂÚ¯fiÙ·Ó ÙÔ ˘ÎÓfiÚÚ¢ÛÙÔ

¯ÔÏ҉˜ ÂÚȯfiÌÂÓÔ. ¶Ú·ÁÌ·ÙÔÔÈ‹ıËΠÂÎÙÔÌ‹

ÙÌ‹Ì·ÙÔ˜ ÙÔ˘ ÂÓÙ¤ÚÔ˘ Î·È ‰ÈÂÓ¤ÚÁÂÈ· ‰ÈÏ‹˜ ÛÙÔ-

Ì›·˜, ÙÔÔıÂÙ‹ıËΠ·ÚÔ¯¤Ù¢ÛË ÂÓÙfi˜ Ù˘ ÂÚÈ-

ÙÔÓ·˚΋˜ ÎÔÈÏfiÙËÙ·˜ Î·È ¤ÁÈÓ ۇÁÎÏÂÈÛË ÙÔ˘ ÙÚ·‡-

Ì·ÙÔ˜ Ì ηÈÓÔ‡ÚÁÈÔ Ï¤ÁÌ· Goretex. ∏ ÈÛÙÔ·ıÔ-

ÏÔÁÈ΋ ÂͤٷÛË ÙÔ˘ ÂÓÙ¤ÚÔ˘ ¤‰ÂÈÍ ÌfiÓÔ ÛËÌ›·

ÊÏÂÁÌÔÓ‹˜.

∏ ·ÍÔÓÈ΋ ÙÔÌÔÁÚ·Ê›· ıÒڷη Ô˘ ‰ÈÂÓÂÚÁ‹ıË-

ΠÙËÓ ÂfiÌÂÓË Ë̤ڷ ¤‰ÂÈÍ ·ÙÂÏÂÎÙ·Û›· Î·È Ì›ˆ-

ÛË ÙÔ˘ fiÁÎÔ˘ ÙÔ˘ ·ÚÈÛÙÂÚÔ‡ Ó‡ÌÔÓ·. ™ÙÔÓ ·ÛıÂ-

Ó‹ ¯ÔÚËÁ‹ıËÎ·Ó ÂÈÛÓÂfiÌÂÓ· ‚ÚÔÁ¯Ô‰È·ÛÙ·ÏÙÈο

Î·È ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹. ∆Ô ÓÂÔÁÓfi ÂÙ¤ıË Û ·ÁˆÁ‹ ÌÂ

‰ÔÌÂÚȉfiÓË ÏfiÁˆ Á·ÛÙÚÔÔÈÛÔÊ·ÁÈ΋˜ ·ÏÈÓ‰Úfi-

ÌËÛ˘. ∫ÂÓÙÚÈÎfi˜ ÊÏ‚ÈÎfi˜ ηıÂÙ‹Ú·˜ Ù‡Ô˘

Hickmann ÙÔÔıÂÙ‹ıËΠÛÙÔ ÓÂÔÁÓfi Î·È ·Ú¤ÌÂÈÓÂ

Û˘ÓÔÏÈο ÁÈ· 35 Ë̤Ú˜. √ ·ÛıÂÓ‹˜ ·Ú¤ÌÂÈÓ ÛÂ

Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi Û˘ÓÔÏÈο 46 Ë̤Ú˜. ªÂÙ¿ ÙËÓ

·Ô‰¤ÛÌ¢ÛË ·fi ÙÔÓ Ì˯·ÓÈÎfi ·ÂÚÈÛÌfi ¯ÚÂÈ¿ÛıË-

Π¯ÔÚ‹ÁËÛË Ô͢ÁfiÓÔ˘ ÁÈ· 9 Ë̤Ú˜. ∆ËÓ 58Ë Ë̤ڷ

˙ˆ‹˜ ¤ÁÈÓ ¤̂·ÛË ·Ó·ÛÙfïÛ˘ ÙÔ˘ ÎÂÓÙÚÈÎÔ‡

Î·È ÙÔ˘ ÂÚÈÊÂÚÈÎÔ‡ ÎÔÏÔ‚ÒÌ·ÙÔ˜ ÙÔ˘ ÏÂÙÔ‡

ÂÓÙ¤ÚÔ˘.

∫·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÓÔÛËÏ›·˜ ÙÔ˘, Ô ·ÛıÂÓ‹˜

ÂÙ¤ıË Û ÔÏÈ΋ ·ÚÂÓÙÂÚÈ΋ ‰È·ÙÚÔÊ‹ ÁÈ· 11 Ë̤-

Ú˜, ÛÙË Û˘Ó¤¯ÂÈ· Û ÌÈÎÙ‹ Û›ÙÈÛË Î·È ·fi ÙËÓ 72Ë

Ë̤ڷ ˙ˆ‹˜ ‚ÚÈÛÎfiÙ·Ó Û ϋÚË ÂÓÙÂÚÈ΋ Û›ÙÈÛË,

ηϿ ·ÓÂÎÙ‹. ∆· ˘ÂÚ˯ÔÁÚ·Ê‹Ì·Ù· ÂÁÎÂÊ¿ÏÔ˘

‹Ù·Ó Ê˘ÛÈÔÏÔÁÈο. √ ÔÊı·ÏÌÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ηٿ

ÙËÓ ¤ÍÔ‰fi ÙÔ˘ ·fi ÙËÓ ÎÏÈÓÈ΋ ‰ÂÓ ¤‰ÂÈÍ ·ıÔÏÔÁÈ-

ο Â˘Ú‹Ì·Ù·. √ ·ÛıÂÓ‹˜ ÂÍ‹Ïı ·fi ÙËÓ ÎÏÈÓÈ΋

ÙËÓ 85Ë Ë̤ڷ ˙ˆ‹˜ Ì ‚¿ÚÔ˜ 3.680 g (<10Ë ∂£),

Ì‹ÎÔ˜ 54 cm (25Ë ∂£) Î·È ÂÚ›ÌÂÙÚÔ ÎÂÊ·Ï‹˜ 38 cm

(50Ë ∂£). ŒÏ·‚ ˆ˜ ·ÁˆÁ‹ ÂÈÛÓÂfiÌÂÓ· ‚ÚÔÁ¯Ô‰È·-

ÛÙ·ÏÙÈο Î·È ÎÔÚÙÈÎÔÛÙÂÚÔÂȉ‹ ÁÈ· ÙȘ ÂΉËÏÒÛÂȘ

Ù˘ ‚ÚÔÁ¯ÔÓ¢ÌÔÓÈ΋˜ ÙÔ˘ ‰˘ÛÏ·Û›·˜ Î·È Û΢¿-

ÛÌ·Ù· Ûȉ‹ÚÔ˘ Î·È Û‡ÌÏÂÁÌ· ‚ÈÙ·ÌÈÓÒÓ ∞ Î·È D.

∆Ô ÛÙ·ÙÈÎfi (∂ÈÎfiÓ· 2) Î·È ‰˘Ó·ÌÈÎfi ÛÈÓıËÚÔÁÚ¿-

ÊËÌ· ÓÂÊÚÒÓ Ì Tc-99m Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËηÓ

Û ËÏÈΛ· 6 ÌËÓÒÓ ¤‰ÂÈÍ·Ó Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁ›·

Î·È ÙˆÓ ‰‡Ô ÓÂÊÚÒÓ.

™˘˙‹ÙËÛË

∏ Û˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË (™¢∫) Â›Ó·È ‰˘-

ÛÏ·Û›· ÛÙËÓ ÔÔ›· ·Ú·ÙËÚÂ›Ù·È ÚfiÙˆÛË ÂÓ‰Ô-

ÎÔÈÏÈ·ÎÒÓ ÛÏ¿¯ÓˆÓ ̤۷ ÛÙÔ ıÒڷη, ‰È·Ì¤ÛÔ˘

ÂÓfi˜ ·Ó·ÙÔÌÈÎÔ‡ ÂÏÏ›ÌÌ·ÙÔ˜ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. ∏

‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ÌÔÚ› Ó· ·ÊÔÚ¿ ‰È·ÊÔÚÂÙÈο

ÙÌ‹Ì·Ù· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜. √ ÈÔ ÎÔÈÓfi˜ Ù‡Ô˜ ‰È·-

ÊÚ·ÁÌ·ÙÔ΋Ï˘ Â›Ó·È Ë Ï·ÁÈÔ›ÛıÈ· ΋ÏË ÙÔ˘

Bochdalek, Ë Û˘¯ÓfiÙËÙ· Ù˘ ÔÔ›·˜ ÂÎÙÈÌ¿Ù·È ÂÚ›-

Ô˘ ÛÙÔ 90-95% (4). ∏ ÏÂÈÔ„ËÊ›· ÙˆÓ ÂÚÈÙÒÛÂ-

ˆÓ ™¢∫ Ù‡Ô˘ Bochdalek ·ÊÔÚ¿ ÙËÓ ·ÚÈÛÙÂÚ‹ Ï¢-

Ú¿ ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜, ·ÏÏ¿ Ù· ÔÛÔÛÙ¿ ÙˆÓ ‰ÂÍÈ¿

Î·È ·ÚÈÛÙÂÚ¿ ÂÓÙÔÈ˙fiÌÂÓˆÓ ™¢∫ ÔÈΛÏÏÔ˘Ó. √È

™¢∫ ÂÓÙÔ›˙ÔÓÙ·È ·ÚÈÛÙÂÚ¿ Û ÔÛÔÛÙfi 73-90%,

147™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek

¶·È‰È·ÙÚÈ΋ 2009;72:29-40

∂ÈÎfiÓ· 2. ™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek Î·È ÂÓ‰Ô-ıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ - ÂÚÈÁÚ·Ê‹ ÂÚ›ÙˆÛ˘. ™Ù·ÙÈÎfiÛÈÓıËÚÔÁÚ¿ÊËÌ· ÓÂÊÚÒÓ.

Anterior Posterior

Left Post Oblique Right Post Oblique

Left54.5%

Right 45.5%

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·147

Page 76: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

‰ÂÍÈ¿ Û ÔÛÔÛÙfi 8-24% Î·È ·ÌÊÔÙÂÚfiÏ¢ڷ ‹ ÎÂ-

ÓÙÚÈο Û ÔÛÔÛÙfi 1,2-4,6% (5,6,7). ∏ ™¢∫ Ù‡Ô˘

Bochdalek ÂÚÈÁÚ¿ÊËÎÂ ÁÈ· ÚÒÙË ÊÔÚ¿ ÙÔ 1948 (8).

√ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ Â›Ó·È Û¿ÓÈ·

·ÓˆÌ·Ï›· Î·È ·ÓÙÈÚÔۈ‡ÂÈ ÏÈÁfiÙÂÚÔ ·fi 5%

fiÏˆÓ ÙˆÓ ÓÂÊÚÈÎÒÓ ÂÎÙÔÈÒÓ (2,9). ªÔÚ› Ó· ÂÌ-

Ê·Ó›˙ÂÙ·È ¯ˆÚ›˜ ¿ÏϘ Û˘ÓÔ‰¤˜ ·ÓˆÌ·Ï›Â˜ ‹ Ó· Û¯Â-

Ù›˙ÂÙ·È ÌÂ Û˘ÁÁÂÓ‹ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË ‹ ‰È·ÊÚ·ÁÌ·-

ÙÔ΋ÏË ÌÂÙ¿ ·fi ÙÚ·˘Ì·ÙÈÛÌfi (10). ™Â 15.919 ·˘ÙÔ-

„›Â˜ ·È‰ÈÒÓ Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËηÓ, ÌfiÓÔ 1 ÂÚ›-

ÙˆÛË ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡ ·Ú·ÙËÚ‹-

ıËÎÂ, ·Ó¿ÌÂÛ· Û 22 ÂÚÈÙÒÛÂȘ ÓÂÊÚÈ΋˜ ÂÎÙÔ›·˜

(11). ∏ ÚÒÙË ÂÚ›ÙˆÛË ÎÏÈÓÈο ‰È·ÁÓˆṲ̂Ó˘ ÂÓ-

‰ÔıˆÚ·ÎÈ΋˜ ÓÂÊÚÈ΋˜ ÂÎÙÔ›·˜ ÂÚÈÁÚ¿ÊËΠ·fi

ÙÔÓ Wolfromm ÙÔ 1940 (12). ∆Ô 1987, ÔÈ Donat ηÈ

Donat ÌÂϤÙËÛ·Ó ÂÚÈÙÒÛÂȘ Ô˘ ‰ËÌÔÛȇıËηÓ

‰ÈÂıÓÒ˜ ·fi ÙÔ 1922 ¤ˆ˜ ÙÔ 1986 Î·È Î·Ù¤ÏËÍ·Ó ÛÙÔ

Û˘Ì¤Ú·ÛÌ· fiÙÈ Ë Û˘ÁÁÂÓ‹˜ ·˘Ù‹ ·ÓˆÌ·Ï›· ÂÌÊ·Ó›-

˙ÂÙ·È Û˘¯ÓfiÙÂÚ· ÛÙËÓ ·ÚÈÛÙÂÚ‹ (62%) ·fi fi,ÙÈ ÛÙË

‰ÂÍÈ¿ ÏÂ˘Ú¿ (36%). ∆Ô 2% ÙˆÓ ·ÛıÂÓÒÓ ¤¯ÂÈ ·ÌÊÔ-

ÙÂÚfiÏ¢ÚË ÂÓ‰ÔıˆÚ·ÎÈ΋ ÓÂÊÚÈ΋ ÂÎÙÔ›·. √ ·ÚÈ-

ÛÙÂÚfi˜ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ Û˘Û¯ÂÙ›˙ÂÙ·È Ì ÛÎÂ-

ÏÂÙÈΤ˜ Î·È Î·Ú‰È·Î¤˜ ·ÓˆÌ·Ï›Â˜ (13). ∂ÈÚfiÛıÂÙ·,

Ë Û˘ÁÁÂÓ‹˜ ·˘Ù‹ ·ÓˆÌ·Ï›· ··ÓÙ¿Ù·È Û˘¯ÓfiÙÂÚ· ÛÂ

¿ÚÚÂÓ˜ (63%) ·fi fi,ÙÈ ÛÙ· ı‹Ï· (37%). ∏ Û˘¯Ófi-

ÙËÙ· ÙÔ˘ ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ÓÂÊÚÔ‡ ÛÙË ™¢∫ Ù‡Ô˘

Bochdalek ·Ó·Ê¤ÚÂÙ·È ÏÈÁfiÙÂÚÔ ·fi 0,25% (2).

∆· ·Ó·ÙÔÌÈο ¯·Ú·ÎÙËÚÈÛÙÈο ÙÔ˘ ÂÓ‰ÔıˆÚ·ÎÈ-

ÎÔ‡ ÓÂÊÚÔ‡ ÂÚÈÏ·Ì‚¿ÓÔ˘Ó: ·ÓˆÌ·Ï›· ÛÙÚÔÊ‹˜ Ù˘

‡Ï˘ ÙÔ˘ ÓÂÊÚÔ‡ Á‡Úˆ ·fi ÙÔÓ ÂÈÌ‹ÎË ¿ÍÔÓ·,

Ì·ÎÚ‡ Ô˘ÚËÙ‹Ú·, ˘„ËÏ‹ ¤ÎÊ˘ÛË ÙˆÓ ÓÂÊÚÈÎÒÓ ·Á-

Á›ˆÓ Î·È ·fiÎÏÈÛË ÙÔ˘ οو fiÏÔ˘ ÙÔ˘ ÓÂÊÚÔ‡

·fi ÙË Ì¤ÛË ÁÚ·ÌÌ‹ (14,15). √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂ-

ÊÚfi˜ ÂÌÊ·Ó›˙ÂÈ Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁÈÎfiÙËÙ·, ‰ÂÓ

·ÚÔ˘ÛÈ¿˙ÂÈ ‰˘ÛÏ·Û›· ‹ ·fiÊÚ·ÍË Ù˘ ÂÎÎÚÈÙÈ΋˜

ÙÔ˘ ÌÔ›Ú·˜ Î·È ‰ÂÓ Û˘Óԉ‡ÂÙ·È ·fi ˘ÂÚÏ·Û›·

ÙÔ˘ ¿ÏÏÔ˘ ÓÂÊÚÔ‡ (16,22). ∏ ·ÈÙ›· Ù˘ ÂÓ‰ÔıˆÚ·ÎÈ-

΋˜ ÂÎÙÔ›·˜ Â›Ó·È ¿ÁÓˆÛÙË. ∏ ıˆڛ· Ù˘ ·ÔÙ˘-

¯Ë̤Ó˘ Û‡ÁÎÏÂÈÛ˘ Ù˘ Ï¢ÚÔÂÚÈÙÔÓ·˚΋˜ Ù˘-

¯‹˜ ÁÈ· ÙË ‰ËÌÈÔ˘ÚÁ›· Ù˘ ™¢∫ Â›Ó·È Â˘Ú¤ˆ˜ ·Ô‰Â-

ÎÙ‹. ∏ ·Ó¿Ù˘ÍË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ·Ú¯›˙ÂÈ ·fi ÙÔ

ÚfiÛıÈÔ ÙÌ‹Ì· ÙÔ˘ ˆ˜ ¤Ó· ¯ÒÚÈÛÌ· ÌÂٷ͇ Ù˘ ηÚ-

‰È¿˜ Î·È ÙÔ˘ ‹·ÙÔ˜ Î·È ÚÔ¯ˆÚ¿ÂÈ ÚÔ˜ Ù· ›Ûˆ,

ÁÈ· Ó· ÎÏ›ÛÂÈ ÙÂÏÈο ÚÔ˜ ÙÔ ·ÚÈÛÙÂÚfi ÙÚ‹Ì· ÙÔ˘

Bochdalek, Á‡Úˆ ÛÙËÓ 8Ë Ì 10Ë Â‚‰ÔÌ¿‰· ·ËÛ˘.

∫·Ù¿ Ì›· ¿Ô„Ë, ıˆÚÂ›Ù·È fiÙÈ ÂÓ‰ÔıˆÚ·ÎÈ΋ ÓÂ-

ÊÚÈ΋ ÂÎÙÔ›· ÌÔÚ› Ó· ÚÔÎÏËı› ·fi ÙËÓ Î·ı˘-

ÛÙÂÚË̤ÓË Û‡ÁÎÏÂÈÛË ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ ‹ ÙËÓ ÂÈ-

Ù¿¯˘ÓÛË Ù˘ ·Ófi‰Ô˘ ÙÔ˘ ÓÂÊÚÔ‡ ÚÈÓ ·fi ÙËÓ Î·ÓÔ-

ÓÈ΋ Û‡ÁÎÏÂÈÛË (3,13). √ ÓÂÊÚfi˜ Û˘Ó‹ıˆ˜ ‚Ú›ÛÎÂÙ·È

ÛÙÔ Ï·ÁÈÔ›ÛıÈÔ ÙÌ‹Ì· ÙÔ˘ ‰È·ÊÚ¿ÁÌ·ÙÔ˜ Î·È Ô

Ô˘ÚËÙ‹Ú·˜ ÂͤگÂÙ·È Ì¤Û· ·fi ÙÔ ÛÙfiÌÈÔ Ù˘ ΋-

Ï˘. ∆Ô ‰È¿ÊÚ·ÁÌ· Û ·˘Ùfi ÙÔ ÛËÌÂ›Ô ·ÔÙÂÏ›ٷÈ

·fi ÌÈ· ÏÂÙ‹ ÌÂÌ‚Ú¿ÓË Ô˘ ÂÚÈ‚¿ÏÏÂÈ ÙÔ ÚÔ¤-

¯ÔÓ ÙÌ‹Ì· ÙÔ˘ ÓÂÊÚÔ‡ Î·È ¤ÙÛÈ Ô ÓÂÊÚfi˜ ‚Ú›ÛÎÂÙ·È

ÂÓÙfi˜ Ù˘ ıˆÚ·ÎÈ΋˜ ÎÔÈÏfiÙËÙ·˜. ¢Â˘ÙÂÚÔ·ıÒ˜

ÌÔÚ› Ó· ˘¿Ú¯ÂÈ Î·È ˘ÔÏ·Û›· ÙÔ˘ ηÙÒÙÂÚÔ˘

ÏÔ‚Ô‡ ÙÔ˘ Û‡ÛÙÔÈ¯Ô˘ Ó‡ÌÔÓ· (13). √È Hawass ND

et al., ÙÔ 1988, ÚfiÙÂÈÓ·Ó fiÙÈ Ë ÙÂÏÈ΋ ı¤ÛË ÙÔ˘ ÓÂ-

ÊÚÔ‡ ηıÔÚ›˙ÂÙ·È ·fi ÙÌ‹Ì· ÙÔ˘ ÁÂÓÓËÙÈÎÔ‡ ÙfiÓÔ˘

(ÙÔ Û˘Á¯ˆÓÂ˘Ì¤ÓÔ Ô˘Ú·›Ô ÙÌ‹Ì· ÙˆÓ ‰‡Ô Ô˘ÚÔÁÂÓ-

ÓËÙÈÎÒÓ ·ÎÚÔÏÔÊÈÒÓ, οı ̛· ·fi ÙȘ Ôԛ˜ ·Ú¤-

¯ÂÈ ¤Ó·Ó ÌÂÛÔÓÂÊÚÈÎfi Î·È ¤Ó·Ó ·Ú·ÌÂÛÔÓÂÊÚÈÎfi

fiÚÔ), ÙÔ ÔÔ›Ô ·ÔÚÚÔÊ¿Ù·È ÌÂÙ¿ ÙËÓ ¤Ó·ÚÍË Ù˘

ÓÂÊÚÔÁ¤ÓÂÛ˘ (17). ™Â fiϘ ÙȘ ÂÚÈÙÒÛÂȘ ÂÓ‰Ô-

ıˆÚ·ÎÈ΋˜ ÓÂÊÚÈ΋˜ ÂÎÙÔ›·˜, Ô ÓÂÊÚfi˜ ‚Ú›ÛÎÂÙ·È

ÂÓÙfi˜ Ù˘ ıˆÚ·ÎÈ΋˜ ÎÔÈÏfiÙËÙ·˜ Î·È fi¯È ÛÙËÓ ˘Â-

˙ˆÎÔÙÈ΋ ÎÔÈÏfiÙËÙ·. ∆· ÓÂÊÚÈο ·ÁÁ›· Î·È Ô Ô˘ÚË-

Ù‹Ú·˜ Ù˘Èο ÂͤگÔÓÙ·È ·fi ÙËÓ ˘Â˙ˆÎÔÙÈ΋ ÎÔÈ-

ÏfiÙËÙ· ‰È·Ì¤ÛÔ˘ ÙÔ˘ ÛÙÔÌ›Ô˘ Ù˘ ΋Ï˘ Î·È Û˘Ó‹-

ıˆ˜ ¤¯Ô˘Ó ÌÂÁ·Ï‡ÙÂÚÔ Ì‹ÎÔ˜ ·fi ÙÔ Ê˘ÛÈÔÏÔÁÈÎfi.

√ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ Ô˘ ‰ÂÓ Û¯ÂÙ›˙ÂÙ·È ÌÂ

™¢∫ ‚Ú›ÛÎÂÙ·È ÛÙËÓ ·Ú·ÛÔÓ‰˘ÏÈ΋ ·‡Ï·Î·. ∆·

ËÌȉȷÊÚ¿ÁÌ·Ù· Â›Ó·È Ê˘ÛÈÔÏÔÁÈο Î·È ‚Ú›ÛÎÔÓÙ·È

ÛÙÔÓ Î·ÙÒÙÂÚÔ fiÏÔ ÙÔ˘ ÓÂÊÚÔ‡.

∏ ÚÔÁÂÓÓËÙÈ΋ ‰È¿ÁÓˆÛË Â›Ó·È ÂÊÈÎÙ‹ ηٿ ÙÔÓ

˘ÂÚ˯ÔÁÚ·ÊÈÎfi ¤ÏÂÁ¯Ô ÙÔ˘ ÂÌ‚Ú‡Ô˘ (18). √ ÚÔÁÂÓ-

ÓËÙÈÎfi˜ ¤ÏÂÁ¯Ô˜ Ì ¯Ú‹ÛË Ù˘ Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·-

Ê›·˜ Û ÂÚ›ÙˆÛË ˘Ô„›·˜ ‡·Ú͢ ™¢∫ Î·È Û˘ÓÔ-

‰ÒÓ ·ÓˆÌ·ÏÈÒÓ ¤¯ÂÈ ÚfiÛÊ·Ù· ÚÔÙ·ı› (19) Î·È Û·-

ÊÒ˜ ÏÂÔÓÂÎÙ› ¤Ó·ÓÙÈ ÙÔ˘ ˘ÂÚ˯ÔÁÚ·Ê‹Ì·ÙÔ˜.

∞Ó Î·È Ë ™¢∫ ÂΉËÏÒÓÂÙ·È ÎÏÈÓÈο ·Ì¤Ûˆ˜ ÌÂÙ¿

ÙË Á¤ÓÓËÛË ‹ ÙȘ ÚÒÙ˜ ̤Ú˜ ˙ˆ‹˜, 10-20% ÙˆÓ

ÂÚÈÙÒÛÂˆÓ ‰È·ÁÈÁÓÒÛÎÔÓÙ·È ÌÂÙ¿ ÙË ÓÂÔÁÓÈ΋

ÂÚ›Ô‰Ô (20,21). ∏ ÓÂÊÚÈ΋ ÂÎÙÔ›·, fiÙ·Ó ‰ÂÓ Û˘ÓÔ-

‰Â‡ÂÙ·È ·fi ¿ÏϘ ·ÓˆÌ·Ï›Â˜, ‰ÂÓ ÚÔηÏ› Û˘-

ÌÙÒÌ·Ù·. √ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ÓÂÊÚfi˜ ·ÚÔ˘ÛÈ¿˙ÂÈ

Ê˘ÛÈÔÏÔÁÈ΋ ÏÂÈÙÔ˘ÚÁ›· ¯ˆÚ›˜ ÛËÌ›· ‰˘ÛÏ·Û›·˜,

·fiÊڷ͢ Ù˘ ·ÂÎÎÚÈÙÈ΋˜ ÌÔ›Ú·˜ ‹ Û˘ÓÔ‰fi

˘ÂÚÏ·Û›· ÙÔ˘ ¿ÏÏÔ˘ ÓÂÊÚÔ‡ (16,22). øÛÙfiÛÔ,

˘¿Ú¯Ô˘Ó ÂÚÈÔÚÈṲ̂Ó˜ ·Ó·ÊÔÚ¤˜ ÛÙË ‚È‚ÏÈÔÁÚ·-

Ê›· ÂÓ‰ÔıˆÚ·ÎÈÎÒÓ ¤ÎÙÔˆÓ ÓÂÊÚÒÓ Ì ·fiÊÚ·ÍË

Ù˘ ˘ÂÏÔÔ˘ÚËÙËÚÈ΋˜ Û˘Ì‚ÔÏ‹˜ ÙÔ˘ ÓÂÊÚÔ‡, ÌÂ

΢ÛÙÂÔÔ˘ÚËÙËÚÈ΋ ·ÏÈÓ‰ÚfiÌËÛË Î·È Ì ÔÏ˘Î˘-

ÛÙÈ΋ ‰˘ÛÏ·Û›· ÛÙÔÓ ¤ÎÙÔÔ ÓÂÊÚfi (1). ™ÙȘ ÂÚÈ-

ÙÒÛÂȘ Û˘Ó‡·Ú͢ ™¢∫, ÏfiÁˆ Ù˘ Ó¢ÌÔÓÈ΋˜

˘ÔÏ·Û›·˜ Ô˘ ÔÊ›ÏÂÙ·È ÛÙËÓ ›ÂÛË ÙˆÓ Ó¢Ìfi-

ÓˆÓ Î·Ù¿ ÙË ‰È¿ÚÎÂÈ· Ù˘ ÂÌ‚Ú˘˚΋˜ ˙ˆ‹˜ ·fi Ù·

ÂÓ‰ÔÎÔÈÏȷο ÛÏ¿¯Ó· Ô˘ ÚÔÛ›ÙÔ˘Ó ÂÓÙfi˜ ÙÔ˘

ıÒڷη, ÔÈ ·ÛıÂÓ›˜ ηٿ ÙË Á¤ÓÓËÛË ÂΉËÏÒÓÔ˘Ó

ÔÍ›· ·Ó·Ó¢ÛÙÈ΋ ·Ó¿ÚÎÂÈ·. ™Â ·˘Ù¤˜ ÙȘ ÂÚÈ-

ÙÒÛÂȘ ··ÈÙÂ›Ù·È ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË Ìfi-

ÏȘ ÛÙ·ıÂÚÔÔÈËı› Ô ·ÛıÂÓ‹˜. ™Â ·Û˘Ìو̷ÙÈ-

ÎÔ‡˜ ·ÛıÂÓ›˜, Ë ¯ÂÈÚÔ˘ÚÁÈ΋ ·ÔηٿÛÙ·ÛË ÌÔ-

Ú› Ó· Á›ÓÂÈ Ì¤Ú˜ ‹ Î·È ¯ÚfiÓÈ· ÌÂÙ¿, ·Ó¿ÏÔÁ· Ì ÙËÓ

ηٿÛÙ·Û‹ ÙÔ˘.

148 Ã. ∆۷ηϛ‰Ë˜ Î·È Û˘Ó.

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¢È¿ÊÔÚ˜ ·ÂÈÎÔÓÈÛÙÈΤ˜ ̤ıÔ‰ÔÈ ¯ÚËÛÈÌÔÔÈ-Ô‡ÓÙ·È ÁÈ· ÙË ‰È¿ÁÓˆÛË ‡·Ú͢ ÂÓ‰ÔıˆÚ·ÎÈÎÔ‡ ÓÂ-ÊÚÔ‡ Î·È ÁÈ· ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ·fi ˆÚÔηٷ-ÎÙËÙÈΤ˜ ÂÂÍÂÚÁ·Û›Â˜ ÙÔ˘ ıÒڷη ‹ ÙÔ˘ ÌÂÛÔıˆ-ڷΛԢ. ∏ ·Ï‹ ·ÎÙÈÓÔÁÚ·Ê›· ıÒڷη Û˘Ó‹ıˆ˜·ÔηχÙÂÈ ·Ú·ÛÔÓ‰˘ÏÈ΋ Ì¿˙· ÛÙÔ Ô›ÛıÈÔÌÂÛÔıˆÚ¿ÎÈÔ.

™ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÔÓÙ·È Ë Ó¢-ÚÔ˚ӈ̿وÛË, Ë ÌËÓÈÁÁÔ΋ÏË Î·È ÔÈ fiÁÎÔÈ ÙÔ˘ Ô›-ÛıÈÔ˘ ÌÂÛÔıˆÚ·Î›Ô˘, fiˆ˜ ÙÔ Ó¢ÚÔ‚Ï¿Ûو̷, ÙÔÁ·ÁÁÏÈÔÓ‡ڈ̷ Î·È Ë Ó¢ÚÔÂÓÙÂÚÈ΋ ·ÛÙË (23). ™ÙȘÂÚÈÛÛfiÙÂÚ˜ ‰ËÌÔÛÈÂ˘Ì¤Ó˜ ÂÚÈÙÒÛÂȘ, ¯ÚËÛÈÌÔ-ÔÈ‹ıËÎÂ Ë ÂÓ‰ÔÊϤ‚È· ˘ÂÏÔÁÚ·Ê›· ÁÈ· ÙË ‰È¿ÁÓˆ-ÛË (17,24). ∂›Û˘, ÙÔ ˘ÂÚ˯ÔÁÚ¿ÊËÌ· Î·È Ë CT ıÒ-ڷη ÌÔÚÔ‡Ó Ó· ‚ÔËı‹ÛÔ˘Ó ÛÙË ‰È¿ÁÓˆÛË (18,25). ∏CT ıÒڷη ÌÔÚ› Ó· ÂȂ‚·ÈÒÛÂÈ ‚Ï¿‚˜ Ù˘ ˘¤-ÏÔ˘ Î·È ÙˆÓ Î·Ï‡ÎˆÓ Î·È Ó· ‰È·ÁÓÒÛÂÈ ¿ÏϘ ‚Ï¿‚Â˜Ô˘ ÌÈÌÔ‡ÓÙ·È ÂÓ‰ÔıˆÚ·ÎÈÎfi ¤ÎÙÔÔ ÓÂÊÚfi, fiˆ˜ ÔÈfiÁÎÔÈ ÙÔ˘ ÌÂÛ·˘Ï›Ô˘ Î·È Ë ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË.

£Âڷ¢ÙÈ΋ ·Ú¤Ì‚·ÛË ··ÈÙÂ›Ù·È ÌfiÓÔ Û Â-Ú›ÙˆÛË ·fiÊڷ͢ Ù˘ ·ÂÎÎÚÈÙÈ΋˜ ÌÔ›Ú·˜ ÙÔ˘ÓÂÊÚÔ‡ Î·È Û ÂÌÊ¿ÓÈÛË Î˘ÛÙÂÔÔ˘ÚËÙËÚÈ΋˜ ·ÏÈÓ-‰ÚfiÌËÛ˘. ∂¿Ó ηٿ ÙË ‰È¿ÁÓˆÛË Ô ÓÂÊÚfi˜ ‰ÂÓ Â›Ó·ÈηÙÂÛÙÚ·Ì̤ÓÔ˜, Ë ·fiÊÚ·ÍË Î·È Ë Î˘ÛÙÂÔÔ˘ÚËÙË-ÚÈ΋ ·ÏÈÓ‰ÚfiÌËÛË ÌÔÚ› Ó· ‰ÈÔÚıˆıÔ‡Ó Ì ¤Ì-‚·ÛË. ∂¿Ó fï˜ Ë ÏÂÈÙÔ˘ÚÁ›· ÙÔ˘ ÓÂÊÚÔ‡ Â›Ó·È ÛÔ-‚·Ú¿ ÂËÚ·Ṳ̂ÓË, ÙfiÙÂ Ë ·Ê·›ÚÂÛ‹ ÙÔ˘ ·ÔÙÂÏ›ÙË Ï‡ÛË ÂÈÏÔÁ‹˜ (1). ∂›Û˘, ¯ÂÈÚÔ˘ÚÁÈ΋ ‰ÈÂÚ‡-ÓËÛË ÂÈ‚¿ÏÏÂÙ·È fiÙ·Ó Û˘Ó˘¿Ú¯ÂÈ ™¢∫ Ù‡Ô˘Bochdalek. ™Â ·˘Ù¤˜ ÙȘ ÂÚÈÙÒÛÂȘ Ô ÓÂÊÚfi˜ Û˘-Ó‹ıˆ˜ ηχÙÂÙ·È ·fi ÙÔ ÔÈÛıÔÂÚÈÙfiÓ·ÈÔ Î·ÈÌÔÚ› ‡ÎÔÏ· Ó· ÙÔÔıÂÙËı› ÛÙËÓ ÎÔÈÏȷ΋ ¯ÒÚ·,¯ˆÚ›˜ ÛËÌ·ÓÙÈΤ˜ ·ÈÌÔ‰˘Ó·ÌÈΤ˜ ÂÈÏÔΤ˜, fiˆ˜ÛÙËÓ ÂÚ›ÙˆÛ‹ Ì·˜.

√ ÂÓ‰ÔıˆÚ·ÎÈÎfi˜ ¤ÎÙÔÔ˜ ÓÂÊÚfi˜ ·ÔÙÂÏ› Û¿-ÓÈ· ÎÏÈÓÈ΋ ÔÓÙfiÙËÙ· Î·È ı¤ÙÂÈ ÔÏÏ¿ ‰È·ÁÓˆÛÙÈÎ¿Î·È ıÂڷ¢ÙÈο ‰ÈÏ‹ÌÌ·Ù· ÛÙÔ˘˜ ÎÏÈÓÈÎÔ‡˜ ÁÈ·-ÙÚÔ‡˜. ∏ Û˘Ó‡·ÚÍË ™¢∫ Ù‡Ô˘ Bochdalek Î·È ÂÓ‰Ô-ıˆÚ·ÎÈÎÔ‡ ¤ÎÙÔÔ˘ ÓÂÊÚÔ‡ Â›Ó·È ÂÍ·ÈÚÂÙÈο Û¿ÓÈ·.¶Ú¤ÂÈ Ó· ÙÔÓÈÛÙ› fiÙÈ Ë Û˘ÁÁÂÓ‹˜ ·˘Ù‹ ·ÓˆÌ·Ï›· ı·Ú¤ÂÈ Ó· Ï·Ì‚¿ÓÂÙ·È ˘fi„Ë ÛÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆ-ÛË Î¿ı ÊÔÚ¿ Ô˘ Ù›ıÂÙ·È ı¤Ì· ‡·Ú͢ ÂÓ‰ÔıˆÚ·-ÎÈ΋˜ Ì¿˙·˜. ∏ ¤ÁηÈÚË ‰È¿ÁÓˆÛË Î·È ÛˆÛÙ‹ ·ÓÙÈÌÂ-ÙÒÈÛË ÌÂÈÒÓÂÈ ÙË ÓÔÛËÚfiÙËÙ· Î·È ıÓËÙfiÙËÙ·.

µÈ‚ÏÈÔÁÚ·Ê›·

1. Bauerb SB. Anomalies of the kidney and ureteropelvicjunction. Campbell’s urology. Saunders, Philadelphia1998;1708-1755.

2. Donat SM, Donat PE. Intrathoracic kidney: a case reportwith a review of the world literature. J Urol 1987;140:131-133.

3. Franciskovic V, Martincic N. Intrathoracic kidney. Br JUrol 1959;31:156.

4. Pober BR. Overview of epidemiology, genetics, birth defectsand chromosome abnormalities associated with CDH. AmJ Med Part C Semin Med Genet 2007;145C:158-171.

5. Skari H, Bjornland K, Haugen G, Egeland T, Emblem R.Congenital diaphragmatic hernia: A meta-analysis ofmortality factors. J Pediatr Surg 2000;35:1187-1197.

6. Dott MM, Wong LY, Rasmussen SA. Population-basedstudy of congenital diaphragmatic hernia: Risk factors andsurvival in Metropolitan Atlanta, 1968-1999. Birth DefectsRes A Clin Mol Teratol 2003;67:261-267.

7. Yang W, Carmichael SL, Harris JA, Shaw GM.Epidemiologic characteristics of congenital diaphragmatichernia among 2.5 million California births, 1989-1997.Birth defects Res 2006;76:170-174.

8. Haller SA. Professor Bochdalek and his hernia: then andnow. Prog Paediatr Surg 1986;20:252-255.

9. Summer TE, Volberg FM, Smolen PM. Intrathoracickidney-diagnosis by ultrasound. Pediatr Radiol 1982;12:78-80.

10. Karaoglanoglu N, Turkyilmaz A, Eroglu A, Alici A. Right-sided Bochdalek hernia with intrathoracic kidney. PediatrSurg Int 2006;22:1029-1031.

11. Campbell MF. Renal ectopy. J Urol 1930;24:187-189.12. Wolfromm MG. Situation du rein dans l’eventration

diaphragmatique droite. Mem Acad Chir 1940;60:41-47.13. Yalcinbas À, Sasmaz H, Canbaz S. Thoracic left kidney:

differential diagnostic dilemma for thoracic Surgeons. AnnThorac Surg 2001;72:281-283.

14. Spillane RJ, Prather GC. Right diaphragmatic eventrationwith renal displacement. J Urol 1952;68:804.

15. Gondos B. High ectopy of left kidney. Am J Roent-genol1955;74:295-298.

16. Obatake M, Nakata T, Nomura M, Nanashina A, InamuraY, Tanaka K, et al. Congenital intrathoracic kidney withright Bochdalek defect. Pediatr Surg Int 2006;22:861-863.

17. Hawass ND, Kolawole TM, EI Badawi MG, Patel PJ,Malabarey T. Intrathoracic kidneys: report of 6 cases and areview of the literature. Eur Urol 1988;14:83-87.

18. Mastrurzo B, Kalache KD, Cockell A, Pierro A, RodeckCH. Prenatal diagnosis of an ectopic intrathoracic kidneyin right-sided congenital diaphragmatic hernia using colorDoppler ultrasonography. Ultrasound Obstet Gynecol2001;18:173-174.

19. Hubbard AM, Crombleholme TM, Adzick NS, ColemanBG, Howell LJ, Meyer JS, et al. Prenatal MRI evaluation ofcongenital diaphragmatic hernia. Am J Perinatol 1999;16:407-413.

20. Baglaj ª. Late-presenting congenital diaphragmatichernia. Journal of Pediatric Surgery 2004;40:1839-1843.

21. Keles S, Artac H, Elmaci M, Reishi I, Dilsiz A. Latepresenting congenital diaphragmatic hernia associated withectopic thoracic kidney. Eur J Pediatr 2006;165:571-572.

22. Ramos A, Slovis T, Reed J. Intrathoracic kidney. Urologyvol. XIII. 1979;1:14-19.

23. Vasquez J, Lupi H. Kasep J. Congenital intrathoracic rightkidney. Arch Inst Cardiol Mex 1984;54:377-379.

24. Kirshenbaum AS, Puri HC, Rao BR. Congenitalintrathorax kidney. J Urol 1981;125:412-413.

25. Grenadin DS, Larsen CR. Computed tomography ofintrathoracic kidney. Urology 1983;22:85-87.

149™˘ÁÁÂÓ‹˜ ‰È·ÊÚ·ÁÌ·ÙÔ΋ÏË Ù‡Ô˘ Bochdalek

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150 ∫§π¡π∫√ ∫√Àπ∑ CLINICAL QUIZ

Paediatriki 2009;72:66,71-73

∞ÁfiÚÈ 13 ÂÙÒÓ ÚÔÛÎÔÌ›ÛÙËΠÛÙ· Â͈ÙÂÚÈοȷÙÚ›· ·Ú·ÔÓÔ‡ÌÂÓÔ ÁÈ· ‰˘Ûη̄›· Ù˘ Ô‰Ô-ÎÓËÌÈ΋˜, ¿ÏÁÔ˜ Î·È ¯ˆÏfiÙËÙ·, ÌÂÙ¿ ·fi ηٷfi-ÓËÛË ÛÙË ‰È¿ÚÎÂÈ· ·ıÏԷȉȿ˜. ¢ÂÓ ÂÚȤÁÚ·„ÂÛ˘ÁÎÂÎÚÈ̤ÓË Î¿ÎˆÛË, ÏËÓ fï˜ ·Ó¤ÊÂÚ ˆ˜ ÙÔÙÂÏÂ˘Ù·›Ô ÂÍ¿ÌËÓÔ Â›¯Â ηْ ·ӿÏË„Ë ·Ú·ÙË-Ú‹ÛÂÈ Ô›‰ËÌ· ÛÙËÓ ¿ÚıÚˆÛË.

™ÙËÓ ·ÓÙÈÎÂÈÌÂÓÈ΋ ÂͤٷÛË, ‰È·ÈÛÙÒıËΠ‰˘-Ûη̄›· Ù˘ ·ÚÈÛÙÂÚ‹˜ Ô‰ÔÎÓËÌÈ΋˜ Î·È ÙÔÈ΋¢·ÈÛıËÛ›·, ¯ˆÚ›˜ Ô›‰ËÌ·, ÛÙËÓ ÂÚÈÔ¯‹ ÙˆÓ ÛÊ˘-ÚÒÓ. √ ‚ÈÔ¯ËÌÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‹Ù·Ó ·ÚÓËÙÈÎfi˜. √È ‰Â›-ÎÙ˜ Ïԛ̈͢ ‹Ù·Ó Ê˘ÛÈÔÏÔÁÈÎÔ›. √ Ï‹Ú˘ Ú¢-Ì·ÙÔÏÔÁÈÎfi˜ ¤ÏÂÁ¯Ô˜ ‹Ù·Ó ›Û˘ ·ÚÓËÙÈÎfi˜. ∏ÔÊı·ÏÌÔÏÔÁÈ΋ ÂͤٷÛË Û ۯÈÛÌÔÂȉ‹ Ï˘¯Ó›·, ÁÈ·ÙË ‰È·›ÛÙˆÛË Èı·Ó‹˜ ÈÚȉÔ΢ÎÏ›Ùȉ·˜, ‰ÂÓ ¤‰ÂÈ-Í ·ıÔÏÔÁÈο Â˘Ú‹Ì·Ù·.

√È ·ÎÙÈÓÔÁڷʛ˜ ÛÙËÓ ÚÔÛıÈÔ›ÛıÈ· Î·È Ï¿-ÁÈ· ÚÔ‚ÔÏ‹ ¤‰ÂÈÍ·Ó ÔÛÙÈ΋ ·ÏÏÔ›ˆÛË ÛÙÔ Ô›-ÛıÈÔ-¤Ûˆ ÙÌ‹Ì· ÙÔ˘ ıfiÏÔ˘ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘ (EÈÎfi-Ó· 1). ∆Ô ˘ÂÚ˯ÔÁÚ¿ÊËÌ· ¤‰ÂÈÍ ÌÈÎÚ‹ Û˘ÏÏÔÁ‹˘ÁÚÔ‡, ÂÓ‰ÂÈÎÙÈ΋ ‰Ú¿ÚıÚÔ˘. ∆Ô ÛÈÓıËÚÔÁÚ¿ÊË-Ì· ÙˆÓ ÔÛÙÒÓ Ì ڷ‰ÈÂÓÂÚÁfi ∆¯ӋÙÈÔ99 ¤‰ÂÈÍ ·˘-ÍË̤ÓÔ ÌÂÙ·‚ÔÏÈÛÌfi Î·È ÛÙȘ ÙÚÂȘ Ê¿ÛÂȘ. ∏ MRI(EÈÎfiÓ˜ 2 Î·È 3) ¤‰ÂÈÍ ÔÛÙÈ΋ ‚Ï¿‚Ë ÛÙÔ Ô›ÛıÈÔ-¤Ûˆ ÙÌ‹Ì· ÙÔ˘ ıfiÏÔ˘ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘, Ì ÌÈÎÚ‹Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡ Î·È Ô›‰ËÌ· ÙÔ˘ ˘ÔΛÌÂÓÔ˘ Ù˘‚Ï¿‚˘ ÔÛÙÔ‡, ¯ˆÚ›˜ ΢ÛÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ.

∆ÔÔıÂÙ‹ıËΠÁ‡„ÈÓÔ˜ Ó¿ÚıËη˜ ÎÓËÌÔÔ‰È-Îfi˜ Î·È ÌÂÙ¿ ·fi 3 ‚‰ÔÌ¿‰Â˜ ‰ÂÓ ‰È·ÈÛÙÒıËηÓÙ· ·Ú¯Èο Û˘ÌÙÒÌ·Ù· Î·È Ù· ÎÏÈÓÈο ÛËÌ›·. ∆Ô-ÔıÂÙ‹ıËΠÎÓËÌÔÔ‰ÈÎfi˜ ÎˉÂÌfiÓ·˜ ÁÈ· ¿ÏϘ 3‚‰ÔÌ¿‰Â˜, ÂÓÒ Û fiÏÔ ·˘Ùfi ÙÔ ¯ÚÔÓÈÎfi ‰È¿ÛÙËÌ·ÙÔ ¿ÎÚÔ ‰ÂÓ ÊÔÚÙ›ÛÙËÎÂ. ∆ÚÂȘ Ì‹Ó˜ ÌÂÙ¿ ÙËÓ ·Ú-¯È΋ ÂÎÙ›ÌËÛË, Ô ·ÛıÂÓ‹˜ Â›Ó·È ··ÏÏ·Á̤ÓÔ˜ ·fiÙ· ÂÓԯϋ̷ٿ ÙÔ˘ Î·È ÔÈ ·ÎÙÈÓÔÁڷʛ˜ Â›Ó·È Ê˘-ÛÈÔÏÔÁÈΤ˜.

¶Èı·Ó¤˜ ‰È·ÁÓÒÛÂȘ:

1. MË ÙÚ·˘Ì·ÙÈ΋ ÌÔÓÔ·ÚıÚ›Ùȉ· Ô‰ÔÎÓËÌÈ΋˜ 2. OÛÙÂÔÓ¤ÎÚˆÛË3. ™¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· ·ÛÙÚ·Á¿ÏÔ˘4. √ÛÙÂÔÂȉ¤˜ ÔÛÙ¤ˆÌ· 5. ∞ÔÛ·ÛÙÈÎfi οٷÁÌ·

µ’ √ÚıԷȉÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

AÏÏËÏÔÁÚ·Ê›·:

¡ÈÎfiÏ·Ô˜ ª·ÚΤ·˜[email protected]µ’ √ÚıԷȉÈÎfi ∆Ì‹Ì·,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

2nd Department ofOrthopaedics, “P. & A. Kyriakou” Children’sHospital, Athens, Greece

Correspondence:

Nikolaos [email protected] Department ofOrthopaedics, “P. & A. Kyriakou” Children’sHospital, Athens, Greece

∞ÁfiÚÈ 13 ÂÙÒÓ Ì ·ӷϷ̂·ÓfiÌÂÓ· ÂÂÈÛfi‰È· ÂÒ‰˘Ó˘

Ô‰ÔÎÓËÌÈ΋˜ ¿ÚıÚˆÛ˘

¡. ª·ÚΤ·˜, π. ¶··¯Ú‹ÛÙÔ˜

Thirteen-year-old boy with recurrent episodes of painful

ankle joint

¡. Markeas, π. Papachristos

∏ ·¿ÓÙËÛË ÛÙË ÛÂÏ›‰· 157

∂ÈÎfiÓ· 1. ∏ ·Ú¯È΋ ·ÎÙÈÓÔÁÚ·Ê›· ·Ó·‰ÂÈÎÓ‡ÂÈ ÔÛÙÈ΋·ÏÏÔ›ˆÛË ÛÙÔ ıfiÏÔ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘.

∂ÈÎfiÓ· 2. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·, ÛÙÔ ÛÙÂÊ·ÓÈ·›Ô ›-‰Ô, ·ÔηχÙÂÈ ÙËÓ ·ÏÏÔ›ˆÛË ÛÙÔ Ô›ÛıÈÔ-¤Ûˆ ÙÌ‹-Ì· ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘, ÙË ÌÈÎÚ‹ Û˘ÏÏÔÁ‹ ˘ÁÚÔ‡ Î·È ÙÔ ÔÛÙÈ-Îfi Ô›‰ËÌ·.

∂ÈÎfiÓ· 3. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›·, ÛÙÔ Ô‚ÂÏÈ·›Ô ›Â-‰Ô, ·Ó·‰ÂÈÎÓ‡ÂÈ Ù· ›‰È· Â˘Ú‹Ì·Ù·.

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·150

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151™À¡∆√ª∞ ¶∞π¢π∞∆ƒπ∫∞ ¡∂∞ PAEDIATRIC NEWS IN BRIEF

¶·È‰È·ÙÚÈ΋ 2009;72:151-000

∂ËÚ¿˙ÂÈ Ë ıÂڷ›· Ù˘ ÔÍ›·˜ ̤Û˘ˆÙ›Ùȉ·˜ (√ªø) ÙËÓ Â›ÙˆÛË Ù˘̷ÛÙÔÂȉ›Ùȉ·˜ ÛÙ· ·È‰È¿;Paula Thompson et al.Pediatrics 2009;123:424-430

™ÙÔ Ù‡¯Ô˜ ºÂ‚ÚÔ˘·Ú›Ô˘ ÙÔ˘ Pediatrics ‰Ë-ÌÔÛȇıËΠ¤Ó· ÂӉȷʤÚÔÓ ¿ÚıÚÔ Ô˘ ·ÊÔÚ¿ÛÙË ¯Ú‹ÛË ÙˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ÁÈ· ÙËÓ ·ÓÙÈÌÂÙÒ-ÈÛË Ù˘ ÔÍ›·˜ ̤Û˘ ˆÙ›Ùȉ·˜ Î·È Ò˜ ·˘Ù‹ ËıÂڷ›· ÌÔÚ› Ó· ÂËÚ¿ÛÂÈ ÙËÓ Â›ÙˆÛËÙ˘ Ì·ÛÙÔÂȉ›Ùȉ·˜, Ô˘ ·ÔÙÂÏ› Ì›· ·fi ÙȘÛÔ‚·ÚfiÙÂÚ˜ ÂÈÏÔΤ˜ Ù˘ ÓfiÛÔ˘. ∏ Û˘ÁÎÂ-ÎÚÈ̤ÓË ÌÂϤÙË ·ÌÊÈÛ‚ËÙ› Ì›· ÎÏ·ÛÈ΋ ıˆڛ·Ô˘ ηıÈÛÙ¿ ··Ú·›ÙËÙË ÙË ıÂڷ›· Ù˘√ªø. ™ÙËÓ ·Ó·‰ÚÔÌÈ΋ ·˘Ù‹ ÌÂϤÙË, Ë ÔÔ›·¤Ï·‚ ¯ÒÚ· ÛÙË ª. µÚÂÙ·Ó›·, ¯ÚËÛÈÌÔÔÈ‹ıË-Î·Ó ËÏÂÎÙÚÔÓÈÎÔ› Ê¿ÎÂÏÔÈ ·È‰ÈÒÓ ËÏÈΛ·˜ 3ÌËÓÒÓ ¤ˆ˜ 15 ÂÙÒÓ ÌÂٷ͇ ÙˆÓ ÂÙÒÓ 1990-2006,Ù· ÔÔ›· ·Ó¤Ù˘Í·Ó Ì·ÛÙÔÂȉ›Ùȉ·. ∞fi Ù· 854ÂÂÈÛfi‰È· Ì·ÛÙÔÂȉ›Ùȉ·˜, ÌfiÓÔ ÙÔ 1/3 (35,7%)›¯Â ÈÛÙÔÚÈÎfi √ªø. ∏ Èı·ÓfiÙËÙ· ·Ó¿Ù˘Í˘̷ÛÙÔÂȉ›Ùȉ·˜ ÌÂÙ¿ ·fi √ªø ÁÈ· ÙËÓ ÔÔ›·¯ÔÚËÁ‹ıËΠıÂڷ›· ‹Ù·Ó 1,8/100.000 ÂÂÈÛfi-‰È· √ªø, ÂÓÒ Ë Â›ÙˆÛË Û¯Â‰fiÓ ‰ÈÏ·ÛÈ¿˙Â-Ù·È (3,8/100.000) fiÙ·Ó ‰ÂÓ Â›¯Â ‰Ôı› ıÂڷ›·.∞˘Ùfi ÛËÌ·›ÓÂÈ fiÙÈ ÁÈ· Ó· ÚÔÏËÊı› 1 ÂÚ›Ùˆ-ÛË Ì·ÛÙÔÂȉ›Ùȉ·˜ Ú¤ÂÈ Ó· ıÂڷ‡ÛÔ˘ÌÂ4.831 ÂÚÈÙÒÛÂȘ √ªø. ™Â ·fiÏ˘ÙÔ˘˜ ·ÚÈı-ÌÔ‡˜, ·˘Ùfi ÌÂÙ·ÊÚ¿˙ÂÙ·È Û 255 ηÈÓÔ‡ÚÁÈ· Â-ÚÈÛÙ·ÙÈο Ì·ÛÙÔÂȉ›Ùȉ·˜ ÙÔ ¯ÚfiÓÔ ÁÈ· ÙË ª.µÚÂÙ·Ó›· Â¿Ó ÛÙ·Ì·ÙÔ‡ÛÂ Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛËÁÈ· ÙËÓ √ªø, ÂÓÒ Ë ¯Ú‹ÛË ÙˆÓ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ı·ÌÂȈÓfiÙ·Ó Î·Ù¿ 738.775 Û˘ÓÙ·Á¤˜.

™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ÂÈÛËÌ·›-ÓÔ˘Ó fiÙÈ Ù· ÂÚÈÛÛfiÙÂÚ· ·È‰È¿ Ô˘ ·Ó¤Ù˘Í·ÓÌ·ÛÙÔÂȉ›Ùȉ· ‰ÂÓ Â›¯·Ó ÂÈÛÎÂÊı› ÙÔÓ ÁÈ·ÙÚfiÙÔ˘˜. ∫·Ù¿ ÙÔ˘˜ Û˘ÁÁÚ·Ê›˜, Ë ·‡ÍËÛË Ù˘ ›-ÙˆÛ˘ Ù˘ Ì·ÛÙÔÂȉ›Ùȉ·˜ ÏfiÁˆ Ù˘ ÌË ¯Ú‹-Û˘ ·ÓÙÈ‚ÈÔÙÈÎÒÓ ÁÈ· ÙË ıÂڷ›· √ªø ›ӷÈÌÈÎÚ‹ ÂÙËÛ›ˆ˜ Î·È ÙÔ fiÊÂÏÔ˜ ·fi ÙË Ì›ˆÛË Ù˘¯Ú‹Û˘ ·ÓÙÈ‚ÈÔÙÈÎÒÓ Ô˘ÛÈÒÓ ıˆÚÂ›Ù·È fiÙÈ Â›Ó·ÈÛËÌ·ÓÙÈÎfiÙÂÚÔ ·fi ÙÔ ÎfiÛÙÔ˜ Ù˘ ÂÈÏÔ΋˜.

£Âڷ›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·˜: ÊÂÏ› ËÌ·ÎÚÔ¯ÚfiÓÈ· ·ÚÂÓÙÂÚÈ΋ ıÂڷ›·;Theoklis Zaoutis et al. Pediatrics 2009:2;636-642

√ Zaoutis et al. ‰ËÌÔÛ›Â˘Û·Ó ÛÙÔ PediatricsÙ· ·ÔÙÂϤÛÌ·Ù· ÌÈ·˜ ÌÂÁ¿Ï˘ ·Ó·‰ÚÔÌÈ΋˜ÌÂϤÙ˘ Ô˘ Ú·ÁÌ·ÙÔÔÈ‹ıËΠÛÙȘ ∏¶∞ ηȷÊÔÚÔ‡Û ÛÙË Û‡ÁÎÚÈÛË Ù˘ Ì·ÎÚÔ¯ÚfiÓÈ·˜ ÂÓ-‰ÔÊϤ‚È·˜ ıÂڷ›·˜ ÁÈ· ÔÍ›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·Ì ·˘Ù‹ Ù˘ Ù·¯Â›·˜ ÌÂÙ¿‚·Û˘ ·fi ÂÓ‰ÔÊϤ-‚È· ıÂڷ›· Û ıÂڷ›· ·fi ÙÔ ÛÙfiÌ·.

™Â ·˘Ù‹ ÙËÓ ÔÏ˘ÎÂÓÙÚÈ΋ ÌÂϤÙË (29 ÓÔÛÔ-ÎÔÌ›· ÙˆÓ ∏¶∞) Û˘ÌÂÚÈÂÏ‹ÊıËÛ·Ó ·È‰È¿ 2ÌËÓÒÓ ¤ˆ˜ 17 ¯ÚÔÓÒÓ Ì ÔÍ›· ÔÛÙÂÔÌ˘ÂÏ›Ùȉ·Î·Ù¿ Ù· ¤ÙË 2000-2005. ™˘ÓÔÏÈο 1.969 ·È‰È¿ÏËÚÔ‡Û·Ó Ù· ÎÚÈÙ‹ÚÈ·, ÂÎ ÙˆÓ ÔÔ›ˆÓ 1.021¤Ï·‚·Ó Ì·ÎÚ¿˜ ‰È¿ÚÎÂÈ·˜ ÂÓ‰ÔÊϤ‚È· ıÂڷ›·(>2 ‚‰ÔÌ¿‰Â˜), ÂÓÒ 948 ¤Ï·‚·Ó ‚Ú·¯Â›· ÂÓ‰Ô-ÊϤ‚È· ıÂڷ›· (<2 ‚‰ÔÌ¿‰Â˜) Î·È ÛÙË Û˘Ó¤-¯ÂÈ· ıÂڷ›· ·fi ÙÔ ÛÙfiÌ·. ∂ÈÙ˘¯‹˜ ‹Ù·Ó ËıÂڷ›· ÛÙÔ 95% ÙˆÓ ·È‰ÈÒÓ Ô˘ ¤Ï·‚·Ó Ì·-ÎÚÔ¯ÚfiÓÈ· ÂӉԂϤ‚È· ıÂڷ›·, ¤Ó·ÓÙÈ ÙÔ˘ 96%ÙˆÓ ·È‰ÈÒÓ Ô˘ ¤Ï·‚·Ó ‚Ú·¯Â›· ÂÓ‰ÔÊϤ‚È·

¡¤· ·fi ÙÔÓ ÒÚÔ ÙˆÓ ·È‰È·ÙÚÈÎÒÓ ÏÔÈÌÒ͈Ó

¡. ™˘Ú›‰Ë˜

News on paediatric infections

N. Spyridis

µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ

∞ÏÏËÏÔÁÚ·Ê›·:

¡›ÎÔ˜ ¶. ™˘Ú›‰Ë˜[email protected]µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¶·ÓÂÈÛÙ‹ÌÈÔ ∞ıËÓÒÓ

2nd Department ofPaediatrics, University ofAthens, Athens, Greece

Correspondence:

Nikos P. [email protected] Department ofPaediatrics, University ofAthens, Athens, Greece

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·151

Page 80: Παιδιατρική | Τόμος 72 • Τεύχος 2 • Απρίλιος - Μάιος - Ιούνιος 2009

ÂÈ ˜

ÈÎÔ‡

È¿·.Ô˘Ì·.Ô˘

˘Ù¤˜ÂÈÙÈ˜Ô˘,

Ó‡

Ì¿‰·Ù·È

152 ™À¡∆√ª∞ ¶∞π¢π∞∆ƒπ∫∞ ¡∂∞

Paediatriki 2009;72:152-000

ıÂڷ›·. Ÿˆ˜ ·Ó·ÌÂÓfiÙ·Ó, Ù· ·È‰È¿ Ô˘

¤Ï·‚·Ó ·ÚÂÓÙÂÚÈ΋ ıÂڷ›· ›¯·Ó Û ÔÛÔ-

ÛÙfi 3,4% ÂÈÏÔΤ˜ Ô˘ ÔÊ›ÏÔÓÙ·Ó Î˘Ú›ˆ˜

ÛÙË ¯Ú‹ÛË ÙÔ˘ ÂÓ‰ÔÊϤ‚ÈÔ˘ ηıÂÙ‹Ú·.

™˘ÓÔ„›˙ÔÓÙ·˜, ÔÈ Û˘ÁÁÚ·Ê›˜ ˘ÔÛÙËÚ›˙Ô˘Ó

fiÙÈ ÁÈ· ÙË ıÂڷ›· Ù˘ ÔÍ›·˜ ÔÛÙÂÔÌ˘ÂÏ›ÙÈ-

‰·˜, Ë Ù·¯Â›· ÌÂÙ¿‚·ÛË ·fi ÙËÓ ÂÓ‰ÔÊϤ‚È·

ÛÙËÓ ·fi ÙÔ˘ ÛÙfiÌ·ÙÔ˜ ·ÁˆÁ‹ Û¯ÂÙ›˙ÂÙ·È ÌÂ

¿ÚÈÛÙ· ·ÔÙÂϤÛÌ·Ù·, ÂÓÒ ·Ú¿ÏÏËÏ· ·Ô-

ʇÁÔÓÙ·È ÔÈ ·ÚÂÓ¤ÚÁÂȘ ·fi ÙË ¯Ú‹ÛË Î·ıÂ-

Ù‹ÚˆÓ.

§¿ıË Î·Ù¿ ÙË Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË ÛÙËÓ ·È‰È΋

ËÏÈΛ·

Ian Wong et al.

Archives of Diseases in Childhood 2009;94;161-164

√ Ian Wong et al. ‰ËÌÔÛ›Â˘Û·Ó ÛÙÔ Archives

of Diseases in Childhood ÌÈ· ÛÂÈÚ¿ ·fi„ÂˆÓ Ô˘

·ÊÔÚÔ‡Ó ÛÙȘ ÌÂıfi‰Ô˘˜ ·ÓÙÈÌÂÙÒÈÛ˘ Ï·ıÒÓ

ÛÙË Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË Ê·ÚÌ¿ÎˆÓ ÛÙËÓ ·È‰È΋

ËÏÈΛ·. ∆Ô Û˘ÓËı¤ÛÙÂÚÔ È·ÙÚÈÎfi ÛÊ¿ÏÌ· ·ÊÔÚ¿

ÛÙË Ï·Óı·Ṳ̂ÓË Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË. ∆· Ï¿ıË ·˘-

Ù¿ ·Ó¤Ú¯ÔÓÙ·È ÁÈ· ÙÔ ∏ӈ̤ÓÔ µ·Û›ÏÂÈÔ ÛÂ

500.000 ÂÚÈÙÒÛÂȘ ÂÙËÛ›ˆ˜. ∆· ΢ÚÈfiÙÂÚ· ·›-

ÙÈ· ›ӷÈ: 1) Ë ÔÏ˘ÏÔÎfiÙËÙ· ÛÙÔÓ ÔÏÔÁÈÛÌfi

Ù˘ ‰fiÛ˘, Ë ÔÔ›· ÚÔÛ‰ÈÔÚ›˙ÂÙ·È Ì ‚¿ÛË

ÔÏÏÔ‡˜ ·Ú¿ÁÔÓÙ˜, fiˆ˜ Ë ËÏÈΛ·, ÙÔ ‚¿ÚÔ˜,

Ë ÂÈÊ¿ÓÂÈ· ÛÒÌ·ÙÔ˜, Ë ÚÔˆÚfiÙËÙ· Î·È Ë ÎÏÈ-

ÓÈ΋ ÂÈÎfiÓ·, 2) ·ÚÈıÌËÙÈο Ï¿ıË, ΢ڛˆ˜ ÛÙËÓ

ÙÔÔı¤ÙËÛË ‰Âη‰ÈÎÔ‡ „ËÊ›Ô˘, ÌˉÂÓÈÎÒÓ Î·È

ÌÔÓ¿‰ˆÓ ̤ÙÚËÛ˘, 3) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË ·fi

Ó¤Ô˘˜ ¿ÂÈÚÔ˘˜ ÁÈ·ÙÚÔ‡˜, ÔÈ ÔÔ›ÔÈ Û˘ÓÙ·ÁÔ-

ÁÚ·ÊÔ‡Ó Ì ‚¿ÛË ÙËÓ ÂÌÂÈÚ›· ÙÔ˘˜ ·fi ÙÔÓ

ÂÓ‹ÏÈÎÔ ÏËı˘ÛÌfi Î·È 4) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË

Ê·ÚÌ¿ÎˆÓ Ù· ÔÔ›· ‰ÂÓ ¤¯Ô˘Ó ¿‰ÂÈ· ¯Ú‹Û˘

ÛÙÔÓ ·È‰È·ÙÚÈÎfi ÏËı˘ÛÌfi Î·È ˆ˜ ÂÎ ÙÔ‡ÙÔ˘

‰ÂÓ ˘¿Ú¯Ô˘Ó ·È‰È·ÙÚÈΤ˜ ‰fiÛÂȘ.

°È· ÙËÓ ·ÓÙÈÌÂÙÒÈÛË ·˘ÙÔ‡ ÙÔ˘ Ê·ÈÓÔ̤-

ÓÔ˘ Û˘ÓÈÛٿٷÈ: 1) Ë Û˘ÓÙ·ÁÔÁÚ¿ÊËÛË Ó· Á›ÓÂ-

Ù·È Ì¤Ûˆ ËÏÂÎÙÚÔÓÈÎÒÓ ˘ÔÏÔÁÈÛÙÒÓ Ô˘ Ó·

ÂÚȤ¯Ô˘Ó ÂÓÙÔϤ˜ ·ÛÊ·Ï›·˜, 2) Ó· Á›ÓÂÙ·È

¤ÏÂÁ¯Ô˜ ÙˆÓ ‰fiÛÂˆÓ ·fi ÙÔ˘˜ Ê·ÚÌ·ÎÔÔÈÔ‡˜,

3) Ë Âη›‰Â˘ÛË ÙˆÓ È·ÙÚÒÓ Ó· ÂÚÈÏ·Ì‚¿ÓÂÈ

Î·È ÂÈÌÔÚʈÙÈο ÛÂÌÈÓ¿ÚÈ· Û˘ÓÙ·ÁÔÁÚ¿ÊË-

Û˘, 4) Ó· ÂÚÈÁÚ¿ÊÂÙ·È Ë Ì¤ıÔ‰Ô˜ ˘ÔÏÔÁÈ-

ÛÌÔ‡ Ù˘ ‰fiÛ˘ ÛÙË Û˘ÓÙ·Á‹, 5) fiÛÔÓ ·ÊÔÚ¿ ÙË

¯ÔÚ‹ÁËÛË ÂÓ‰ÔÊÏ‚›ˆÓ Ê·Ú̿ΈÓ, Ë ¯Ú‹ÛË

ËÏÂÎÙÚÔÓÈÎÒÓ Û˘Û΢ÒÓ ÌÔÚ› Ó· ·Ó·ÁÓˆÚ›-

ÛÂÈ Èı·Ófi ÛÊ¿ÏÌ· ÛÙË ‰ÔÛÔÏÔÁ›· ÙÔ˘ Ê·ÚÌ¿-

ÎÔ˘, 6) Ó· ÂÓÈÛ¯˘ı› Ë ·Ó·ÊÔÚ¿ Ï·ıÒÓ ÛÙË Û˘-

ÓÙ·ÁÔÁÚ¿ÊËÛË, ÁÂÁÔÓfi˜ Ô˘ ÌÔÚ› Ó· Ô‰ËÁ‹-

ÛÂÈ ÛÙË Ï‹„Ë ÚÔÏËÙÈÎÒÓ Ì¤ÙÚˆÓ Î·È 7) Ó·

‚ÂÏÙȈı› Ë ÂÈÎÔÈÓˆÓ›· ÌÂٷ͇ ÙˆÓ ‰È·ÊfiÚˆÓ

ÂÌÏÂÎfiÌÂÓˆÓ Â·ÁÁÂÏÌ·ÙÈÒÓ ˘Á›·˜.

™˘Û¯¤ÙÈÛË ÙÔ˘ ÔÚfiÙ˘Ô˘ ÙÔ˘

Ó¢ÌÔÓÈfiÎÔÎÎÔ˘ Ì ÙË ÛÔ‚·ÚfiÙËÙ· Ù˘

Ïԛ̈͢ Î·È ÙË ı·Ó·ÙËÊfiÚÔ ¤Î‚·ÛË

Simon Ruckinger et al.

Pediatric Infectious Diseases Journal 2009;28:118-122

™ÙÔ ÙÂÏÂ˘Ù·›Ô Ù‡¯Ô˜ ÙÔ˘ Pediatric Infectious

Diseases Journal ‰ËÌÔÛȇÂÙ·È ·Ó·‰ÚÔÌÈ΋ ÌÂ-

ϤÙË Ô˘ ¤Ï·‚ ¯ÒÚ· ÛÙË °ÂÚÌ·Ó›· ÌÂٷ͇ ÙˆÓ

ÂÙÒÓ 1997-2003 Î·È ·ÊÔÚÔ‡Û ÛÙËÓ Ù·˘ÙÔÔ›Ë-

ÛË ÙÔ˘ Ó¢ÌÔÓÈfiÎÔÎÎÔ˘ Û ·È‰È¿ Ô˘ ¤ı·-

Ó·Ó ÏfiÁˆ Ù˘ Ïԛ̈͢ ‹ ·Ó¤Ù˘Í·Ó ‚·ÚÈ¿ ÏÔ›-

̈ÍË (ÛË„·ÈÌ›·, ÌËÓÈÁÁ›Ùȉ·) ‹ ÛÔ‚·Ú¤˜ ÌfiÓÈ-

̘ ‚Ï¿‚˜. ∞fi ÙȘ 559 ÂÚÈÙÒÛÂȘ fiÔ˘ Ô

ÔÚfiÙ˘Ô˜ Ù·˘ÙÔÔÈ‹ıËÎÂ, 494 ‹Ù·Ó ‰È·ı¤ÛÈ̘

ÁÈ· ÛÙ·ÙÈÛÙÈ΋ ·Ó¿Ï˘ÛË. ∏ Û˘ÓÔÏÈ΋ ıÓËÙfiÙËÙ·

‹Ù·Ó 5,3%, ÂÓÒ Ë ‚·ÚÈ¿ Ïԛ̈ÍË ·ÓÂÚ¯fiÙ·Ó ÛÂ

17%. √ ÔÚfiÙ˘Ô˜ 7F Û˘Û¯ÂÙÈ˙fiÙ·Ó Ì ıÓËÙfiÙË-

Ù· Û ÔÛÔÛÙfi 14,8%, ·ÎÔÏÔ˘ıÔ‡ÌÂÓÔ˜ ·fi ÙÔÓ

23F (8,3%) Î·È ÙÔÓ ÔÚfiÙ˘Ô 3 Û ÔÛÔÛÙfi 3%.

µ·ÚÈ¿ ÎÏÈÓÈ΋ ÂÈÎfiÓ· ·Ó¤Ù˘Í·Ó 17% ÙˆÓ ·È-

‰ÈÒÓ, ÂÎ ÙˆÓ ÔÔ›ˆÓ ÔÛÔÛÙfi 40,7% ÔÊÂÈÏfiÙ·Ó

ÛÙÔÓ ÔÚfiÙ˘Ô 7F.

™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ÛËÌÂÈÒ-

ÓÔ˘Ó fiÙÈ Ë ÌËÓÈÁÁ›Ùȉ· ·fi ÙÔÓ ÔÚfiÙ˘Ô 7F Û˘Ó-

‰¤ÂÙ·È Ì ·˘ÍË̤ÓË ıÓËÙfiÙËÙ· ‹ ÂÈÏÔΤ˜,

ÂÓÒ ÙÚÈÏ·ÛÈ¿˙ÂÙ·È Ë Èı·ÓfiÙËÙ· ·Ó¿Ù˘Í˘

‚·ÚÈ¿˜ ‰ÈÂÈÛ‰˘ÙÈ΋˜ Ïԛ̈͢.

∏ ·Í›· ·˘Ù‹˜ Ù˘ ÌÂϤÙ˘ ‚Ú›ÛÎÂÙ·È ÛÙÔ ÁÂ-

ÁÔÓfi˜ fiÙÈ Ô ÔÚfiÙ˘Ô˜ 7F ‰ÂÓ Û˘ÌÂÚÈÏ·Ì‚¿ÓÂ-

Ù·È ÛÙÔ ˘¿Ú¯ÔÓ Û˘˙¢Á̤ÓÔ Ó¢ÌÔÓÈÔÎÔÎÎÈ-

Îfi ÂÌ‚fiÏÈÔ, ÁÈ’ ·˘Ùfi Î·È Ë ·ÚÔ˘Û›· ÙÔ˘ ‹‰Ë

·ÚÔ˘ÛÈ¿˙ÂÈ ·‡ÍËÛË Û ¯ÒÚ˜ fiˆ˜ Ë ∞ÁÁÏ›·,

°·ÏÏ›·, °ÂÚÌ·Ó›· Î·È ∞Ï¿Ûη. ∆· ‰‡Ô Ó¤· Ó¢-

ÌÔÓÈÔÎÔÎÎÈο ÂÌ‚fiÏÈ· (10-‰‡Ó·ÌÔ Î·È 13-‰‡Ó·-

ÌÔ) Ô˘ ·Ó·Ì¤ÓÂÙ·È Ó· ΢ÎÏÔÊÔÚ‹ÛÔ˘Ó ÛÙÔ

ÚÔÛ¯¤˜ ̤ÏÏÔÓ ı· Û˘ÌÂÚÈÏ·Ì‚¿ÓÔ˘Ó Î·È ÙÔÓ

Û˘ÁÎÂÎÚÈ̤ÓÔ ÔÚfiÙ˘Ô.

¡fiÛÔ˜ Kawasaki ÛÙË ª. µÚÂÙ·Ó›·: ÂıÓÈÎfiÙËÙ·,

Û˘Óı‹Î˜ ‰È·‚›ˆÛ˘ Î·È ·Ó·Ó¢ÛÙÈÎÔ› ÈÔ›

Antony Hardnen et al.

Paediatric Infectious Diseases Journal 2009;28:21-24

™ÙËÓ ·Ó·‰ÚÔÌÈ΋ ·˘Ù‹ ÌÂϤÙË Ô˘ ¤Ï·‚ Ò-

Ú· ÛÙË ª. µÚÂÙ·Ó›·, ·Ó·˙ËÙ‹ıËÎÂ Ë Èı·Ó‹ Û˘-

Û¯¤ÙÈÛË Ù˘ ÓfiÛÔ˘ Kawasaki Ì ÙËÓ ÂıÓÈÎfiÙËÙ·

ÙÔ˘ ·ÛıÂÓÔ‡˜, ÙȘ Û˘Óı‹Î˜ ‰È·‚›ˆÛ˘ Î·È ÙËÓ

ÂȉËÌÈÔÏÔÁ›· ÙˆÓ ·Ó·Ó¢ÛÙÈÎÒÓ ÈÒÓ. ÃÚËÛÈ-

ÌÔÔÈ‹ıËÎ·Ó ËÏÂÎÙÚÔÓÈο ·Ú¯Â›· (Hospital

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·152

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153PAEDIATRIC NEWS IN BRIEF

¶·È‰È·ÙÚÈ΋ 2009;72:153-000

Episode Statistics) Ù· ÔÔ›· ÂÚÈÁÚ¿ÊÔ˘Ó ÙÔ ·›ÙÈÔÂÈÛ·ÁˆÁ‹˜ ÛÙÔ ÓÔÛÔÎÔÌ›Ô, ‰ËÌÔÁÚ·ÊÈο ÛÙÔÈ-¯Â›· ÙÔ˘ ·ÛıÂÓÔ‡˜, ·ÓÙÈÌÂÙÒÈÛË Î·È ·Ú·ÎÏÈÓÈ-Τ˜ ÂÍÂÙ¿ÛÂȘ. ªÂٷ͇ 1998 Î·È 2003 ·ÓÙÈÌÂÙˆ-›ÛÙËÎ·Ó 2.432 ·È‰È¿ Ì ÓfiÛÔ Kawasaki, ÂÎ ÙˆÓÔÔ›ˆÓ 728 ÂÚÈÙÒÛÂȘ ·ÔÙÂÏÔ‡Û·Ó Â·ÓÂÈ-Û·ÁˆÁ‹. ∞fi ÙȘ 1.704 ηÈÓÔ‡ÚÁȘ ÂÚÈÙÒÛÂȘ,ÔÈ 1.228 (72%) ‹Ù·Ó ·È‰È¿ <5 ÂÙÒÓ Î·È ÔÈ 254·È‰È¿ οو ÙÔ˘ ¤ÙÔ˘˜. √ ·ÚÈıÌfi˜ ÙˆÓ Î·ÈÓÔ‡-ÚÈˆÓ ÂÚÈÙÒÛÂˆÓ Û ¤Ó· Ì‹Ó· ÁÈ· Ù· ·È‰È¿ ο-Ùˆ ÙˆÓ 5 ÂÙÒÓ Î˘Ì¿ÓıËΠ·fi 8 (™Â٤̂ÚÈÔ˜2001) ¤ˆ˜ 41 (π·ÓÔ˘¿ÚÈÔ˜ 1999). ∏ ÂÙ‹ÛÈ· ›-ÙˆÛË Ù˘ ÓfiÛÔ˘ ‹Ù·Ó 8,39/100.000 ·È‰È¿. ∏ ÎÈ-ÓÂ˙È΋ ÂıÓÈÎfiÙËÙ· (P=0,005), Ë ‰È·‚›ˆÛË Û ÌÂ-Á¿Ï˜ fiÏÂȘ (P=0,001) Î·È Ë ÔÈÎÔÓÔÌÈ΋ ·Ó¤¯ÂÈ·(P=0,016) ·ÔÙÂÏÔ‡Û·Ó ÈÛ¯˘ÚÔ‡˜ ÚԉȷıÂÛÈ-ÎÔ‡˜ ·Ú¿ÁÔÓÙ˜ ÁÈ· ÙËÓ ·Ó¿Ù˘ÍË Ù˘ ÓfiÛÔ˘. ∏

˘„ËÏfiÙÂÚË Â›ÙˆÛË ·Ú·ÙËÚ‹ıËΠÙÔ˘˜ ¯ÂÈ-ÌÂÚÈÓÔ‡˜ Ì‹Ó˜, ¯ˆÚ›˜ fï˜ οÔÈÔ˜ ·fi ÙÔ˘˜Û˘Ó‹ıÂȘ ÈÔ‡˜ (RSV, adenovirus, influenza) Ó·ÂÌÊ·Ó›˙ÂÙ·È Û˘¯ÓfiÙÂÚ·.

™˘ÌÂÚ·ÛÌ·ÙÈο, ÔÈ Û˘ÁÁÚ·Ê›˜ ·Ó·Ê¤ÚÔ˘ÓfiÙÈ, fiˆ˜ ›¯Â ·Ú·ÙËÚËı› Î·È ÛÙËÓ π·ˆÓ›·,Ë ÌÂٷΛÓËÛË ÛÙȘ ÌÂÁ¿Ï˜ fiÏÂȘ Û˘Ó‰˘¿ÛÙË-Π̠¤Í·ÚÛË Ù˘ ÓfiÛÔ˘. √È Î·Î¤˜ Û˘Óı‹Î˜‰È·‚›ˆÛ˘ ·ÔÙÂÏÔ‡Ó ·ÚÓËÙÈÎfi ÚÔÁÓˆÛÙÈÎfi·Ú¿ÁÔÓÙ·, ÂÓÒ Ù· ÓÂԉȷÁÓˆÛı¤ÓÙ· ·È‰È¿ÂÌÊ·Ó›˙ÔÓÙ·È Î˘Ú›ˆ˜ ÙÔ˘˜ ¯ÂÈÌÂÚÈÓÔ‡˜ Ì‹Ó˜.¶·ÚfiÙÈ ‰È¿ÊÔÚÔÈ ÈÔÁÂÓ›˜ ·Ú¿ÁÔÓÙ˜ ¤¯Ô˘ÓÂÓÔ¯ÔÔÈËı›, ÔÈ ÂÚ¢ÓËÙ¤˜ ‰ÂÓ Î·Ù¿ÊÂÚ·Ó Ó·‚ÚÔ˘Ó Î¿ÔÈ· Û˘Û¯¤ÙÈÛË ÌÂ Û˘ÁÎÂÎÚÈ̤ÓÔ˘˜ÈÔ‡˜, ÂÓÈÛ¯‡ÔÓÙ·˜ ÙË ıˆڛ· fiÙÈ Ë ÁÂÓÂÙÈ΋ÚԉȿıÂÛË ·ÔÙÂÏ› ÙÔÓ ÈÛ¯˘ÚfiÙÂÚÔ Úԉȷ-ıÂÛÈÎfi ·Ú¿ÁÔÓÙ·.

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·153

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154 ∂§§∏¡π∫∞ ∂ƒ∂À¡∞ ™∆∏ ¢π∂£¡∏ µπµ§π√°ƒ∞ºπ∞

Paediatriki 2009;72:154-000

∂ÏÏËÓÈ΋ ·È‰È·ÙÚÈ΋ ¤Ú¢ӷ Û ‰ÈÂıÓ‹ ÂÚÈÔ‰Èο, 2008

∂. °·Ï·Ó¿Î˘

Hellenic paediatric research in international journals, 2008

E. Galanakis

¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘

AÏÏËÏÔÁÚ·Ê›·:

∂ÌÌ·ÓÔ˘‹Ï °·Ï·Ó¿Î˘[email protected]¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋,¶·ÓÂÈÛÙ‹ÌÈÔ ∫Ú‹Ù˘

Department of Paediatrics,University of Crete,Heraklion, Greece

Correspondence:

Emmanouil [email protected] of Paediatrics,University of Crete,Heraklion, Greece

¶·ÚÔ˘ÛÈ¿˙ÔÓÙ·È, Ôχ Û˘ÓÔÙÈο, Ï›Á˜·fi ÙȘ ÔÏϤ˜ ÂÏÏËÓÈΤ˜ ÌÂϤÙ˜ ·È‰È·ÙÚÈ-ÎÔ‡ ÂӉȷʤÚÔÓÙÔ˜ Ô˘ ‰ËÌÔÛȇıËÎ·Ó ÙÔ2008 Û ‰ÈÂıÓ‹ ÂÚÈÔ‰Èο Î·È Î·Ï‡ÙÔ˘Ó fiÏ·Ù· ‰›·, ·fi ÙÔ ÂÚÁ·ÛÙËÚÈ·Îfi ˆ˜ ÙÔ ÎÏÈÓÈÎfiÎ·È ÙÔ ÎÔÈÓˆÓÈÎfi, Î·È fiϘ ÙȘ ËÏÈ˘, ·fi ÙÔÓÂÔÁÓfi Î·È ÙÔ ‚Ú¤ÊÔ˜ ˆ˜ ÙÔ ·È‰› Î·È ÙÔÓ ¤ÊË-‚Ô. ªÂϤÙ˜ Ô˘ ›¯·Ó ‰ËÌÔÛÈ¢ı› ÙÔ˘˜ ÔχÚÒÙÔ˘˜ Ì‹Ó˜ ÙÔ˘ 2008 ¤¯Ô˘Ó ·ÚÔ˘ÛÈ·Ûı›ÛÙËÓ ¶∞π¢π∞∆ƒπ∫∏ 2008;71(3):248-250.

¡ÂÊÚÈ΋ ·Ó¿Ù˘ÍË ÛÙ· ÌÈÎÚ¿ ÓÂÔÁÓ¿.

¶ÚÔÔÙÈ΋ ÌÂϤÙË ·fi Ù· πˆ¿ÓÓÈÓ· (Drougiaet al., Nephrol Dial Transplant, Epub 2008 Aug4) ·Ú·ÎÔÏÔ‡ıËÛ ˘ÂÚ˯ÔÁÚ·ÊÈο ÙËÓ ·Ó¿-Ù˘ÍË ÙˆÓ ÓÂÊÚÒÓ Û 243 ÌÈÎÚ¿ ÁÈ· ÙËÓ ËÏÈ-Λ· ·ËÛ˘ ÓÂÔÁÓ¿ (SGA) Î·È 223 ÓÂÔÁÓ¿ ÌÂηٿÏÏËÏÔ ‚¿ÚÔ˜ ÁÈ· ÙËÓ ËÏÈΛ· ·ËÛ˘(AGA). ∏ ·Ú·ÎÔÏÔ‡ıËÛË ¤ÁÈÓ 5 ÊÔÚ¤˜ ÛÙ·2 ÚÒÙ· ¤ÙË ˙ˆ‹˜ (Û‡ÓÔÏÔ 1898 ˘ÂÚ˯ÔÁÚ·-ÊËÌ¿ÙˆÓ). ™Ù· ÙÂÏÂÈfiÌËÓ· SGA ÓÂÔÁÓ¿, Ë ÓÂ-ÊÚÈ΋ ·Ó¿Ù˘ÍË ‹Ù·Ó fiÌÔÈ· Ì ÂΛÓË ÙˆÓAGA, ˆÛÙfiÛÔ ÛÙ· ÌÈÎÚ¿ ÚfiˆÚ· ÓÂÔÁÓ¿, ÙÔ̤ÁÂıÔ˜ ÙˆÓ ÓÂÊÚÒÓ ·Ú¤ÌÂÓ ÌÈÎÚfiÙÂÚÔ·fi ÂΛÓÔ ÙˆÓ AGA ÓÂÔÁÓÒÓ ÁÈ· Ù· ‰‡Ô ÚÒ-Ù· ¤ÙË Ù˘ ˙ˆ‹˜.

°ÂÓÂÙÈ΋ ÚԉȿıÂÛË ÛÙË Ì¤ÛË ˆÙ›Ùȉ·.

ªÂϤÙË ·fi ÙÔ ∏Ú¿ÎÏÂÈÔ (Ilia S et al., PediatrInfect Dis J 2008;27:929-33) ·Ó·ÛÎÔ› Ù· ¤ˆ˜ÙÒÚ· ÁÓˆÛÙ¿ ÁÈ· ÙËÓ ·¿ÓÙËÛË ÙÔ˘ ÍÂÓÈÛÙ‹ ηÈÙË ÁÂÓÂÙÈ΋ ¢¿ıÂÈ· ÛÙËÓ ˆÙ›Ùȉ·. ∫·Ù·ÁÚ¿-ÊÂÙ·È ÌÈ· ÌÂÁ¿ÏË ÔÈÎÈÏ›· ·Ú·ÁfiÓÙˆÓ Ù˘ ÂÁ-ÁÂÓÔ‡˜ Î·È ÚÔÛ·ÚÌfiÛÈÌ˘ ·ÓÔÛ›·˜ Ô˘ ÚÔ-‰È·ı¤ÙÔ˘Ó Î¿ÔÈ· ‚Ú¤ÊË Î·È ·È‰È¿ Û ÔÏϤ˜‹ Û ÛÔ‚·Ú¤˜ ˆÙ›Ùȉ˜.

º˘Ì·Ù›ˆÛË ‚Ú¤ÊÔ˘˜ ÌÂÙ¿ ÂÚÈÙÔÌ‹. ¶Â-ÚÈÁÚ·Ê‹ ÚˆÙÔ·ıÔ‡˜ Ê˘Ì·Ù›ˆÛ˘ ¤Ô˘˜ ÌÂÛ˘ÓÔ‰fi ‚Ô˘‚ˆÓÈ΋ ÏÂÌÊ·‰ÂÓ›Ùȉ· Û ˘ÁȤ˜ η-Ù¿ Ù· ¿ÏÏ· ·ÁfiÚÈ, ËÏÈΛ·˜ 10 ÌËÓÒÓ, Ô˘ ›¯ÂÂÚÈÙÌËı› ÛÙÔ ¶·ÎÈÛÙ¿Ó ÚÔ 4Ì‹ÓÔ˘ (¡ÔÛÔ-

ÎÔÌÂ›Ô ¶·›‰ˆÓ ∞ıËÓÒÓ «∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡»,Papaevangelou V et al., J Infect Epub 2008 Dec18). ∏ ÂȂ‚·›ˆÛË ¤ÁÈÓ Ì ÔÍÂÔ¿ÓÙÔ¯Ë ¯ÚÒ-ÛË, ηÏÏȤÚÁÂÈ· Î·È PCR.

µÚԢΤÏψÛË Û ÌÈÎÚfi ‚Ú¤ÊÔ˜. ¶ÂÚÈÁÚ·-Ê‹ ÎÚÔ‡ÛÌ·ÙÔ˜ ‚ÚԢΤÏÏ·˜ Û ‚Ú¤ÊÔ˜ 2,5 ÌË-ÓÒÓ ·fi ÙËÓ ◊ÂÈÚÔ (Makis AC et al., EmergInfect Dis 2008;141:319-20). ∆Ô ‚Ú¤ÊÔ˜ ‰ÂÓ Â›¯Â·ʋ Ì ˙Ò·, ‰ÂÓ ı‹Ï·˙Â Î·È Ë ÌËÙ¤Ú· ÙÔ˘‰ÂÓ Â›¯Â ‚ÚԢΤÏψÛË, ¿Ú· ·Ú·Ì¤ÓÂÈ ·ÓÔÈÎÙfiÙÔ ı¤Ì· Ù˘ ÌÂÙ¿‰ÔÛ˘ ·fi ÌÔÏ˘Ṳ̂ӷ ·ÓÙÈ-ΛÌÂÓ·, οÙÈ ·Û˘Ó‹ıÈÛÙÔ ÁÈ· ÙË ÓfiÛÔ.

∞ÓıÂÎÙÈÎÔ› ÛÙ·Ê˘ÏfiÎÔÎÎÔÈ ÛÙËÓ ∞ı‹Ó·.

ªÂϤÙË 198 ÛÙÂϯÒÓ ÛÙ·Ê˘ÏÔÎfiÎÎˆÓ ·fi·È‰È¿ Ì ÏÔÈÌÒÍÂȘ Ù˘ ÎÔÈÓfiÙËÙ·˜ (¡ÔÛÔÎÔ-ÌÂ›Ô ¶·›‰ˆÓ ∞ıËÓÒÓ «∞ÁÏ·˝· ∫˘ÚÈ·ÎÔ‡»,Niniou T et al., Eur J Clin Microbiol Infect Dis2008;27:831-7): 44% MRSA ·ÓÙÔ¯‹ (ÛÙË ÌÂıÈ-ÎÈÏÏ›ÓË).

∞ÓıÂÎÙÈÎÔ› ÛÙ·Ê˘ÏfiÎÔÎÎÔÈ ÛÙËÓ ¶¿ÙÚ·.

∞Ó¿ÏÔÁË ÌÂϤÙË ·fi ÙÔ ∫·Ú·Ì·Ó‰¿ÓÂÈÔ ¡ÔÛÔ-ÎÔÌÂ›Ô ¶·›‰ˆÓ ¶·ÙÚÒÓ (170 ·ÛıÂÓ›˜ Ì ÏÔ›-̈ÍË ·fi S. aureus Î·È ˘ÁÈ‹ ·È‰È¿ Ì ÚÈÓÈ΋ÊÔÚ›· S. aureus, Sdougkos G et al., ClinMicrobiol Infect 2008;14:995-1001): 62% MRSA·ÓÙÔ¯‹ ÁÈ· Ù· ÛÙÂϤ¯Ë ÙˆÓ ·ÛıÂÓÒÓ Î·È 5,5%MRSA ·ÓÙÔ¯‹ ÁÈ· Ù· ÚÈÓÈο ÛÙÂϤ¯Ë ÙˆÓ ˘ÁÈÒӷȉÈÒÓ.

¶Ó¢ÌÔÓÈfiÎÔÎÎÔ˜: ·ÓÙÔ¯‹. ªÂϤÙË 612ÛÙÂϯÒÓ Streptococcus pneumoniae, ‰ÈÂÈÛ‰˘ÙÈ-ÎÒÓ Î·È ÌË, ÚÈÓ ÙËÓ Â˘Ú›· ÂÊ·ÚÌÔÁ‹ ÙÔ˘Ó¢ÌÔÓÈÔÎÔÎÎÈÎÔ‡ ÂÌ‚ÔÏ›Ô˘ (¡ÔÛÔÎÔÌ›Զ·›‰ˆÓ ∞ıËÓÒÓ «∞Á›· ™ÔÊ›·», Daikos GL et al.,Diagn Microbiol Infect Dis 2008;60:393-8) ¤‰ÂÈÍ·‡ÍËÛË Ù˘ ·ÓÙÔ¯‹˜ ÛÙ· Ì·ÎÚÔÏ›‰È· (·fi 7,4%ÙÔ 1985-1996 Û 53,7% ÙÔ 2001-2004), ·Ú¿Ï-ÏËÏË Ì ÙËÓ ·‡ÍËÛË Ù˘ ηٷӿψÛ˘ Ì·ÎÚÔ-Ïȉ›ˆÓ Î·È ÏÈÓÎÔ˙·ÌȉÒÓ ÛÙËÓ ÎÔÈÓfiÙËÙ· (·fi

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155HELLENIC RESEARCH IN THE INTERNATIONAL LITERATURE

¶·È‰È·ÙÚÈ΋ 2009;72:155-000

4,31 DI ‰fiÛÂȘ ÙÔ 1990-1996 Û 6,97 DI ‰fiÛÂȘ ÙÔ2001-2004).

¶Ó¢ÌÔÓÈfiÎÔÎÎÔ˜: ÌÂÙ¿ ÙÔ ÂÌ‚fiÏÈÔ. ªÂϤÙË·fi ÙË §¿ÚÈÛ· Û 1829 Ó‹È· (Grivea IN et al.,Pediatr Infect Dis J 2008;27:519-25) ¤‰ÂÈÍ Ì›ˆÛËÙ˘ ÚÈÓÔÊ·Ú˘ÁÁÈ΋˜ ÊÔÚ›·˜ ÙˆÓ ÔÚÔÙ‡ˆÓ ÙÔ˘ ÂÌ-‚ÔÏ›Ô˘ ÌÂÙ¿ ÙÔ ÂÙ·‰‡Ó·ÌÔ Û˘˙¢Á̤ÓÔ ÂÌ‚fiÏÈÔ,¯ˆÚ›˜ ·‡ÍËÛË Ù˘ ÊÔÚ›·˜ ÙÔ˘ ÔÚfiÙ˘Ô˘ 19∞. ™Ù·ÂÌ‚ÔÏÈ·Ṳ̂ӷ ·È‰È¿ ·Ú·ÙËÚ‹ıËΠ̛ˆÛË ÙË˜Û˘¯ÓfiÙËÙ·˜ ÛÙÂϯÒÓ ˘„ËÏ‹˜ ·ÓÙÔ¯‹˜ ÛÙËÓ ÂÓÈ-ÎÈÏÏ›ÓË.

£Âڷ›· Ì˘ÎËÙÈ·ÛÈÎÒÓ ÏÔÈÌÒ͈Ó. ªÂϤÙË·fi ÙÔ πÔÎÚ¿ÙÂÈÔ ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢(Filioti I et al., Expert Opin Pharmacother2008;9:3179-96) ·Ó·ÛÎÔ› ÙȘ ıÂڷ¢ÙÈΤ˜ ÛÙÚ·-ÙËÁÈΤ˜ ÛÙËÓ ·ÓÙÈÌÂÙÒÈÛË ÙˆÓ ‰ÈÂÈÛ‰˘ÙÈÎÒÓ ÏÔÈ-ÌÒÍÂˆÓ ·fi ∫¿ÓÙÈÓÙ· Î·È ∞Û¤ÚÁÈÏÏÔ Û ÓÂÔÁÓ¿ ηȷȉȿ, Ì ¤ÌÊ·ÛË ÛÙ· ÓÂfiÙÂÚ· ·Ú¿ÁˆÁ·.

§Â˘¯·È̛˜ Î·È ÏÂÌÊÒÌ·Ù· ÛÙËÓ ∂ÏÏ¿‰·. ¶·-ÓÂÏÏ‹ÓÈ· ÌÂϤÙË 11ÂÙ›·˜ (1996-2006) η٤ÁÚ·„Â(Petridou ET et al., Arch Dis Child 2008;93:1027-32)863 ÂÚÈÙÒÛÂȘ Ï¢¯·ÈÌ›·˜ Î·È 311 ÏÂÌÊÒÌ·ÙÔ˜ Û·ȉȿ οو ÙˆÓ 15 ÂÙÒÓ. ∏ ›وÛË Ù˘ Ï¢¯·È-Ì›·˜ (46,6 ÂÚÈÙÒÛÂȘ ·Ó¿ 1.000.000 ·È‰È¿ ·Ó¿

¤ÙÔ˜) ‹Ù·Ó ·Ó¿ÌÂÛ· ÛÙȘ ˘„ËÏfiÙÂÚ˜ Ù˘ ∂˘Úˆ·˚-΋˜ ŒÓˆÛ˘ (19% ˘„ËÏfiÙÂÚË ·fi ÙË Ì¤ÛË Â˘Úˆ-·˚΋). ∏ ›وÛË ÏÂÌÊÒÌ·ÙÔ˜ (16,8 ÂÚÈÙÒÛÂȘ·Ó¿ 1.000.000 ·È‰È¿ ·Ó¿ ¤ÙÔ˜) ‰ÂÓ ‰È¤ÊÂÚ ·fi ÙË̤ÛË Â˘Úˆ·˚΋.

§ÈıÔÙÚÈ„›· ÛÙ· ·È‰È¿. ¶·ÚÔ˘Û›·ÛË Ù˘10ÂÙÔ‡˜ ÂÌÂÈÚ›·˜ ÂÓfi˜ ΤÓÙÚÔ˘ ÛÙËÓ ·ÓÙÈÌÂÙÒÈ-ÛË Ù˘ Ô˘ÚÔÏÈı›·Û˘ Û 125 ·È‰È¿ (πÔÎÚ¿ÙÂÈÔ¡ÔÛÔÎÔÌÂ›Ô £ÂÛÛ·ÏÔӛ΢, Charalambous S et al., JEndourol 2008;22:2169-74): Û 93 ·È‰È¿ ¤ÁÈÓ ÏÈıÔ-ÙÚÈ„›· Ì ˆÛÙÈο ·̷ٷ ˘ÂÚ‹¯ˆÓ (Û‡ÓÔÏÔ 108Û˘Ó‰ÚÈÒÓ), Û 12 Ô˘ÚËÙËÚÔÛÎfiËÛË Î·È ‰ÈÔ˘ÚË-ÙËÚÈ΋ ·Ê·›ÚÂÛË, Û 9 ‰È·‰ÂÚÌ·ÙÈ΋ ÓÂÊÚÔÏÈıÔÙÔ-Ì›· Î·È Û 11 ¯ÚÂÈ¿ÛÙËΠ·ÓÔÈÎÙfi ¯ÂÈÚÔ˘ÚÁ›Ô. ™‡Ì-ʈӷ Ì ÙË ÌÂϤÙË, Ë ÏÈıÔÙÚÈ„›· Î·È ÔÈ ÂÓ‰ÔÔ˘ÚË-ÙËÚÈΤ˜ Ù¯ÓÈΤ˜ Â›Ó·È ·ÛÊ·Ï›˜ Î·È ·ÔÙÂÏÂÛÌ·ÙÈ-Τ˜ ÛÙ· ·È‰È¿.

∆Ô ‡„Ô˜ ÙˆÓ Ó¤ˆÓ ∂ÏÏ‹ÓˆÓ. ªÂϤÙË (∞ÙÙÈÎfi¡ÔÛÔÎÔÌ›Ô, Papadimitriou ∞ et al., Acta Paediatr2008;97:1105-7) Û 3.982 ÓÂÔÛ‡ÏÏÂÎÙÔ˘˜ ËÏÈΛ·˜ 18-26 ÂÙÒÓ (2006-2007) ¤‰ÂÈÍ ̤ÛÔ ‡„Ô˜ ÛÒÌ·ÙÔ˜ Ù·178,1 cm. ∞ÓÙ›ÛÙÔÈ¯Ë ÌÂϤÙË ÙÔ˘ 1990 ›¯Â ηٷÁÚ¿-„ÂÈ Ì¤ÛÔ ‡„Ô˜ 175,7. º·›ÓÂÙ·È, Û˘ÓÂÒ˜, fiÙÈ Ô ·Ó-‰ÚÈÎfi˜ ÏËı˘ÛÌfi˜ Ù˘ ∂ÏÏ¿‰·˜ ‰ÂÓ ¤¯ÂÈ ·ÎfiÌË ÂÍ·-ÓÙÏ‹ÛÂÈ Ù· ÂÚÈıÒÚÈ· ·‡ÍËÛ˘ Û ‡„Ô˜.

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πÛÙÔÛÂÏ›‰· Ù˘ µ’ ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙËÌ›Ô˘

∞ıËÓÒÓ – http://www.2pediatric.gr/

™. ∫¯·ÁÈ¿˜, ¢. ∞Ó·ÁÓÒÛÙÔ˘

Site of the 2nd Department of Paediatrics of the

University of Athens – http://www.2pediatric.gr/

S. Kehagias, D. Anagnostou

√ ‰È·‰ÈÎÙ˘·Îfi˜ ÙfiÔ˜ Ù˘ µã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ, Ô˘ ‰ڿ˙ÂÙ·È ÛÙÔ ¡ÔÛÔÎÔÌ›Զ·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ÏÂÈÙÔ˘ÚÁ› Î·È ·Ó·ÌÔÚÊÒÓÂÙ·ÈÛ˘Ó¯Ҙ Â‰Ò Î·È ‰‡Ô ¯ÚfiÓÈ·. ™Ùfi¯Ô˜ ÙÔ˘ Â›Ó·È Ó· ʤÚÂÈ Û·ʋ Ì ÙÔÓ ÒÚÔ Ù˘ ∫ÏÈÓÈ΋˜ fiÏÔ˘˜ fiÛÔ˘˜ ı· ıÂÏ‹ÛÔ˘Ó Ó·¯ÚËÛÈÌÔÔÈ‹ÛÔ˘Ó ÙȘ È·ÙÚÈΤ˜, ‰È‰·ÎÙÈΤ˜ Î·È ÂÚ¢ÓËÙÈΤ˜Ù˘ ‰Ú·ÛÙËÚÈfiÙËÙ˜. ™ÙȘ ÛÂÏ›‰Â˜ ÙÔ˘ ˘¿Ú¯Ô˘Ó ÏËÚÔÊÔ-ڛ˜ Ô˘ ı· ‰È¢ÎÔχÓÔ˘Ó ÙËÓ ÚfiÛ‚·ÛË ÛÙȘ ÂÍÂȉÈÎÂ˘Ì¤Ó˜˘ËÚÂۛ˜ Ô˘ ·Ú¤¯Ô˘Ó Ù· ÙÌ‹Ì·Ù· Ù˘ ∫ÏÈÓÈ΋˜ Î·È Ù· ÂÈ-‰Èο Â͈ÙÂÚÈο È·ÙÚ›·.

™ÙËÓ ·Ú¯È΋ ÛÂÏ›‰·, ÂÎÙfi˜ ·fi ÙËÓ Ù·¯˘‰ÚÔÌÈ΋ ‰È‡ı˘Ó-ÛË Ù˘ ÎÏÈÓÈ΋˜ Î·È Ù· ÙËϤʈӷ ÙˆÓ ÓÔÛËÏ¢ÙÈÎÒÓ ÌÔÓ¿‰ˆÓ,˘¿Ú¯Ô˘Ó ·Ó·ÚÙË̤Ó˜ Î·È ÔÈ ·Ó·ÎÔÈÓÒÛÂȘ ÙˆÓ Û˘Ó‰ڛˆÓ,ÙˆÓ ÛÂÌÈÓ·Ú›ˆÓ, ·ÏÏ¿ Î·È ÙˆÓ ÌÂÙÂÎ·È‰Â˘ÙÈÎÒÓ ÊÚÔÓÙÈÛÙË-

Ú›ˆÓ Ô˘ ÔÚÁ·ÓÒÓÂÈ Ë ∫ÏÈÓÈ΋ ÛÙÔ ·ÌÊÈı¤·ÙÚÔ «∫. ¶··‰¿ÙÔ˜» ÙÔ˘ ÓÔÛÔÎÔÌ›Ԣ Ì·˜. ™ÙËÓ ·Ú¯È΋ ÛÂÏ›‰· ›Û˘, ÛÙÔ˘˜ «ÃÚ‹ÛÈÌÔ˘˜ ™˘Ó‰¤ÛÌÔ˘˜», ¿Ú¯Ô˘Ó ÈÛÙfiÙÔÔÈ Ô˘ ÚÔÛʤÚÔ˘Ó

¢ηÈڛ˜ ÂÓË̤ڈÛ˘ ÁÈ· ÙË Û˘Ó¯Ҙ ÂÍÂÏÈÛÛfiÌÂÓË ÁÓÒÛË Û¯ÂÙÈο Ì ÙÔ ·È‰› Î·È ÙËÓ ˘Á›· ÙÔ˘, ·Ï-Ï¿ Î·È ÙËÓ ÂÊ·ÚÌÔÁ‹ Ù˘, fiˆ˜ Ê·›ÓÂÙ·È ÛÙÔÓ ÈÛÙfiÙÔÔ ÙÔ˘ ∫¤ÓÙÚÔ˘ Ù˘ π·ÙÚÈ΋˜ Ô˘ µ·Û›˙ÂÙ·È Ûª·ÚÙ˘Ú›Â˜ (Center for Evidence Based Medicine). ™ÙËÓ ›‰È· ÛÂÏ›‰·, ·ÎÔÏÔ˘ı› Ô ¯ÒÚÔ˜ fiÔ˘ ÌÔÚ› Ó·Á›ÓÂÈ Ë ËÏÂÎÙÚÔÓÈ΋ ÂÁÁÚ·Ê‹ ÛÙÔ newsletter Ù˘ ∫ÏÈÓÈ΋˜.

∏ ÛÂÏ›‰· «¶ÚÔÛˆÈÎfi» ·Ú¤¯ÂÈ ÛÙÔȯ›· ÁÈ· ÙÔ ·ÓıÚÒÈ-ÓÔ ‰˘Ó·ÌÈÎfi Î·È ÙË ÛÙÂϤ¯ˆÛË Ù˘ ∫ÏÈÓÈ΋˜. ∏ ÛÂÏ›‰· «∆Ì‹-Ì·Ù·» ‰Â›¯ÓÂÈ ÙȘ ÎÏÈÓÈΤ˜ ‰˘Ó·ÙfiÙËÙ˜ Ù˘ ∫ÏÈÓÈ΋˜. ∞ӷʤ-ÚÔÓÙ·È, ÂÎÙfi˜ ÙˆÓ ‚·ÛÈÎÒÓ ÓÔÛËÏ¢ÙÈÎÒÓ ÌÔÓ¿‰ˆÓ, Ù· ÙÌ‹-Ì·Ù· Î·È Ù· ÂȉÈο Â͈ÙÂÚÈο È·ÙÚ›· ÁÈ· ÙȘ ˘ÔÂȉÈÎfiÙËÙ˜Ù˘ ·È‰È·ÙÚÈ΋˜. ™ÙËÓ ›‰È· ÛÂÏ›‰· ›Û˘, ·Ú¤¯ÔÓÙ·È ÏË-ÚÔÊÔڛ˜ ÁÈ· Ù· ˆÚ¿ÚÈ· ÏÂÈÙÔ˘ÚÁ›·˜, ·ÏÏ¿ Î·È ÙȘ ‰Ú·ÛÙËÚÈfi-ÙËÙ˜ ÙˆÓ ÙÌËÌ¿ÙˆÓ Î·È ÙˆÓ Â͈ÙÂÚÈÎÒÓ È·ÙÚ›ˆÓ.

∞ÎÔÏÔ˘ı› Ë ÛÂÏ›‰· «∂η›‰Â˘ÛË», Ô˘ ÂÚÈÁÚ¿ÊÂÈ ÙÔ ÂÎ-·È‰Â˘ÙÈÎfi ¤ÚÁÔ Ù˘ µã ¶·È‰È·ÙÚÈ΋˜ ∫ÏÈÓÈ΋˜ ÙÔ˘ ¶·ÓÂÈÛÙË-Ì›Ô˘ ∞ıËÓÒÓ. ™ÙËÓ ›‰È· ÛÂÏ›‰· ·ÎÔÏÔ˘ıÔ‡Ó ·ÓÙ›ÛÙÔȯÔÈ ÈÛÙfiÙÔ-ÔÈ, Ì ÏËÚÔÊÔڛ˜ Û¯ÂÙÈΤ˜ Ì ÙȘ ÂοÛÙÔÙ ‰Ú·ÛÙËÚÈfiÙËÙ˜.

∆Ô ÂÚ¢ÓËÙÈÎfi ¤ÚÁÔ Ù˘ ∫ÏÈÓÈ΋˜ ÂÚÈÁÚ¿ÊÂÙ·È ÛÙË ÛÂÏ›‰· «ŒÚ¢ӷ». ∏ ÂÚ¢ÓËÙÈ΋ ‰Ú·ÛÙËÚÈfi-ÙËÙ· Ô˘ ‚Ú›ÛÎÂÙ·È Û ÂͤÏÈÍË ÂÚÈÏ·Ì‚¿ÓÂÈ ÙËÓ ÂÎfiÓËÛË ‰È‰·ÎÙÔÚÈÎÒÓ ‰È·ÙÚÈ‚ÒÓ, ÔÏ˘ÎÂÓÙÚÈÎÒÓÌÂÏÂÙÒÓ, ÂȉËÌÈÔÏÔÁÈÎÒÓ ÌÂÏÂÙÒÓ, ÎÏÈÓÈÎÔÂÚÁ·ÛÙËÚÈ·ÎÒÓ ÌÂÏÂÙÒÓ, ÌÂÏÂÙÒÓ ÎÏÈÓÈ΋˜ ·Ú·Ù‹ÚË-Û˘, ·ÏÏ¿ Î·È ‰È·¯ÚÔÓÈΤ˜ ÌÂϤÙ˜ ηٷÁÚ·Ê‹˜ ÓÔÛËÌ¿ÙˆÓ.

∆¤ÏÔ˜, ÛÙÔ Â‰›Ô «∞Ó·˙‹ÙËÛË», Ô ÈÛÙfiÙÔÔ˜ ÚÔÛʤÚÂÈ ÙË ‰˘Ó·ÙfiÙËÙ· ıÂÌ·ÙÈ΋˜ ·Ó·˙‹ÙËÛË˜ÙˆÓ ÂÚȯÔÌ¤ÓˆÓ ÙÔ˘, Ì ¯Ú‹ÛË Ï¤ÍˆÓ-ÎÏÂȉÈÒÓ.

156 ¡∂∞ ∞¶√ ∆√ ¢π∞¢π∫∆À√ NEWS FROM THE INTERNET

Paediatriki 2009;72:156

¡ÂÔÁÓÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

AÏÏËÏÔÁÚ·Ê›·:

™Ù¤Ê·ÓÔ˜ ∫¯·ÁÈ¿˜[email protected]¡ÂÔÁÓÈÎfi ∆Ì‹Ì·, µ’ ¶·È‰È·ÙÚÈ΋ ∫ÏÈÓÈ΋¶·ÓÂÈÛÙËÌ›Ô˘ ∞ıËÓÒÓ,¡ÔÛÔÎÔÌÂ›Ô ¶·›‰ˆÓ «¶. & ∞. ∫˘ÚÈ·ÎÔ‡», ∞ı‹Ó·

Neonatology Unit, 2nd Department of Paediatrics,University of Athens, “P. & A. Kyriakou” Children’sHospital, Athens, Greece

Correspondence:

Stefanos [email protected] Unit, 2nd Department of Paediatrics,University of Athens, “P. & A. Kyriakou” Children’sHospital, Athens, Greece

Pediatri Mar-Apr 09 10-04-09 15:37 ™ÂÏ›‰·156

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157∫§π¡π∫√ ∫√Àπ∑ CLINICAL QUIZ

¶·È‰È·ÙÚÈ΋ 2009;72:66,71-73

√È ÚÒÙ˜ ÂÚÈÁڷʤ˜ Ù˘ Û¯·ÏȉˆÙÈ΋˜ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ·˜ ÛÙË ‰ÈÂıÓ‹ ‚È‚ÏÈÔÁÚ·Ê›·Î¿ÓÔ˘Ó ÙËÓ ÂÌÊ¿ÓÈÛ‹ ÙÔ˘˜ ‹‰Ë ·fi Ù· Ù¤ÏËÙÔ˘ 19Ô˘ ·ÈÒÓ·, ·ÏÏ¿ ÌfiÏȘ ÙÔ 1959 ÔÈ BerndtÎ·È Harty (1) ÚÔ‚·›ÓÔ˘Ó ÛÙËÓ ÚÒÙË Ù·ÍÈÓfi-ÌËÛË. ∏ ÓfiÛÔ˜ ÚÔÛ‚¿ÏÏÂÈ Û˘Ó‹ıˆ˜ ÙÔ˘˜ ÌË-ÚÈ·›Ô˘˜ ÎÔÓ‰‡ÏÔ˘˜ Î·È Û·ÓÈfiÙÂÚ· ÙÔÓ ÎfiÓ‰˘-ÏÔ ÙÔ˘ ‚Ú·¯ÈfiÓÈÔ˘ ÔÛÙÔ‡. ∏ ÂÓÙfiÈÛ‹ Ù˘ÛÙÔÓ ıfiÏÔ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘ ıˆÚÂ›Ù·È È‰È·›ÙÂ-Ú· Û¿ÓÈ· (4% ÙÔ˘ Û˘ÓfiÏÔ˘ ÙˆÓ ÔÛÙÂÔ¯fiÓ‰ÚÈ-ÓˆÓ ‚Ï·‚ÒÓ ÙÔ˘ ÛÎÂÏÂÙÔ‡). ∏ ÈÛ¯·ÈÌÈ΋ Ó¤-ÎÚˆÛË ÙÔ˘ ˘ÔΛÌÂÓÔ˘ ˘Ô¯fiÓ‰ÚÈÔ˘ ÔÛÙԇʷ›ÓÂÙ·È ˆ˜ ·ÈÙÈÔÏÔÁ› ¿ÚÈÛÙ· ÙËÓ ·ÔÎfiÏ-ÏËÛË ÙÔ˘ ÔÛÙÈÎÔ‡ ÙÂÌ·¯›Ô˘, Ì·˙› Ì ÙÔÓ ˘ÂÚ-ΛÌÂÓÔ ·ÚıÚÈÎfi ¯fiÓ‰ÚÔ.

πÛÙÔÚÈÎfi οΈÛ˘ ·Ó·Ê¤ÚÂÙ·È Û ¤Ó· Ô-ÛÔÛÙfi 85% ÙˆÓ ·ÛıÂÓÒÓ Ì ÔÛÙÂÔ¯fiÓ‰ÚÈÓË‚Ï¿‚Ë ÛÙÔÓ ·ÛÙÚ¿Á·ÏÔ. ∂ȉÈο, ÔÈ ‚Ï¿‚˜ ÛÙÔÔ›ÛıÈÔ-¤Ûˆ ÙÌ‹Ì· ÙÔ˘ ıfiÏÔ˘ ÙÔ˘ ·ÛÙÚ·Á¿-ÏÔ˘ Â›Ó·È ÙÔ ·ÔÙ¤ÏÂÛÌ· ·ӷϷ̂·ÓfiÌÂ-ÓÔ˘ ‚›·ÈÔ˘ ˘ÙÈ·ÛÌÔ‡ ÙÔ˘ ¿ÎÚÔ˘ Ô‰fi˜, Ì·˙›Ì ÂÏÌ·ÙÈ·›· Î¿Ì„Ë Î·È Â͈ÙÂÚÈ΋ ÛÙÚÔÊ‹ÙÔ˘ ÔÛÙÔ‡ ̤۷ ÛÙËÓ Ô‰ÔÎÓËÌÈ΋. ∏ ‚Ï¿‚ËÌÔÚ› Ó· ÚÔÎÏËı› ¯ˆÚ›˜ ··Ú·›ÙËÙ· Û˘-Ó˘¿Ú¯Ô˘Û· Ú‹ÍË ÙÔ˘ ¤Íˆ Ï·Á›Ô˘ Û˘Ó‰¤-ÛÌÔ˘, ÁÂÁÔÓfi˜ Ô˘ ‰ÈηÈÔÏÔÁ› ÙÔ ‡‰Ú·ÚıÚÔÙ˘ Ô‰ÔÎÓËÌÈ΋˜ (fiˆ˜ ÛÙËÓ ÂÚÈÁÚ·Ê›۷ÂÚ›ÙˆÛË), ‰›¯ˆ˜ ÙÔ ¯·Ú·ÎÙËÚÈÛÙÈÎfi ÙÔ˘‰È·ÛÙÚ¤ÌÌ·ÙÔ˜ Ô›‰ËÌ· ÛÙËÓ ÂÚÈÔ¯‹ ÙˆÓÛÊ˘ÚÒÓ.

™ÙË ‰È·ÊÔÚÈ΋ ‰È¿ÁÓˆÛË ÂÚÈÏ·Ì‚¿ÓÔÓٷȤӷ Ï‹ıÔ˜ ·ı‹ÛˆÓ, Ì ÂÓÙfiÈÛË ÙËÓ Ô‰Ô-ÎÓËÌÈ΋ ‹ ÙÔÓ ›‰ÈÔ ÙÔÓ ·ÛÙÚ¿Á·ÏÔ. ∏ ÌË ÙÚ·˘-Ì·ÙÈ΋ ÌÔÓÔ·ÚıÚ›Ùȉ· Ù˘ Ô‰ÔÎÓËÌÈ΋˜ Û˘-Óԉ‡ÂÙ·È Û˘Ó‹ıˆ˜ ·fi ¤ÓÙÔÓ· ÂÓÔ¯Ï‹Ì·Ù·Î·È ÎÏÈÓÈο ÛËÌ›·, ÂÓÒ ÔÈ ‰Â›ÎÙ˜ ÊÏÂÁÌÔÓ‹˜Â›Ó·È ηٿ ηÓfiÓ· ·˘ÍË̤ÓÔÈ. ∏ ÔÛÙÂÔÓ¤ÎÚˆ-ÛË ¯·Ú·ÎÙËÚ›˙ÂÙ·È ·fi ÙËÓ ·Ô˘Û›· ·ÁÁ›ˆ-Û˘ ÙÔ˘ ˘ÔΛÌÂÓÔ˘ Ù˘ ‚Ï¿‚˘ ÔÛÙÔ‡, ÂÓÒÛÙË Û¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· Ë ·ÁÁ›ˆÛËÂ›Ó·È Ê˘ÛÈÔÏÔÁÈ΋. ∂Í ¿ÏÏÔ˘, Ë ÔÛÙÂÔÓ¤ÎÚˆÛËÚÔÛ‚¿ÏÏÂÈ ÌÂÁ·Ï‡ÙÂÚ˜ ËÏÈ˘. ∆Ô ÔÛÙÂÔÂÈ-‰¤˜ ÔÛÙ¤ˆÌ· ¤¯ÂÈ ÂÓÙÔÓfiÙÂÚ· Û˘ÌÙÒÌ·Ù· ηȯ·Ú·ÎÙËÚÈÛÙÈÎfi fiÓÔ, Ô˘ ÂΉËÏÒÓÂÙ·È Û˘Ó‹-ıˆ˜ ÙȘ Ó˘ÎÙÂÚÈÓ¤˜ ÒÚ˜ Î·È ˘Ê›ÂÙ·È Ì ÙË Ï‹-„Ë ÌË ÛÙÂÚÔÂȉÒÓ ·ÓÙÈÊÏÂÁÌÔÓˆ‰ÒÓ Ê·ÚÌ¿-ΈÓ. ∆Ô ·ÔÛ·ÛÙÈÎfi οٷÁÌ· Â›Ó·È ·Û˘Ì‚›-‚·ÛÙÔ Ì ÙËÓ ·Ó·ÙÔÌÈ΋ ÙÔ˘ ·ÛÙÚ·Á¿ÏÔ˘,

ÂÂȉ‹ Ô ıfiÏÔ˜ ÙÔ˘ ‰ÂÓ ‰¤¯ÂÙ·È ÚÔÛʇÛÂȘÈÛ¯˘ÚÒÓ Û˘Ó‰¤ÛÌˆÓ ‹ ÙÂÓfiÓÙˆÓ.

∏ ¿Ù˘Ë ÎÏÈÓÈ΋ ÂÈÎfiÓ· Î·È ÙÔ ·Û·Ê¤˜ ÈÛÙÔ-ÚÈÎfi ‰ÂÓ ÈηÓÔÔÈÔ‡Ó Û˘Ó‹ıˆ˜ ÙË ‰È¿ÁÓˆÛË Î·È··ÈÙÂ›Ù·È Û˘¯Ó¿ Ô ·ÂÈÎÔÓÈÛÙÈÎfi˜ ¤ÏÂÁ¯Ô˜. √È·ÎÙÈÓÔÁڷʛ˜, ÔÈ Ôԛ˜ › ÛÂÈÚ¿ ÂÙÒÓ ‹Ù·Ó ËÚÒÙË ÚÔÛ¿ıÂÈ· ‰È·ÁÓˆÛÙÈ΋˜ ÚÔÛ¤ÁÁÈÛ˘ÙÔ˘ ÚÔ‚Ï‹Ì·ÙÔ˜, ÛÙ· ·Ú¯Èο ÛÙ¿‰È· Ù˘ ÓfiÛÔ˘‰ÂÓ ˆÊÂÏÔ‡Ó, ÁÈ·Ù› ·‰˘Ó·ÙÔ‡Ó Ó· ·ÂÈÎÔÓ›ÛÔ˘ÓÙËÓ ÔÛÙÈ΋ ‚Ï¿‚Ë. ∞˘Ùfi˜ ‹Ù·Ó Ô ÏfiÁÔ˜ Ù˘ η-ı˘ÛÙÂÚË̤Ó˘ ‰È¿ÁÓˆÛ˘ ÛÙË ‰È΋ Ì·˜ ÂÚ›-ÙˆÛË. ∏ Ì·ÁÓËÙÈ΋ ÙÔÌÔÁÚ·Ê›· ¤¯ÂÈ ÙËÓ Èη-ÓfiÙËÙ· Ù˘ ¤ÁηÈÚ˘ ·ÂÈÎfiÓÈÛ˘ Ù˘ ‚Ï¿‚˘,Ù˘ ·ÎÚÈ‚Ô‡˜ ÂÓÙfiÈÛ˘ Î·È Ù˘ ¤ÎÙ·Û‹˜ Ù˘.∂›Û˘ ÌÔÚ› Ó· ·ÂÈÎÔÓ›ÛÂÈ Ì ·ÎÚ›‚ÂÈ· ÙËÓ‡·ÚÍË ÔÛÙÈÎÔ‡ Ôȉ‹Ì·ÙÔ˜. ™‡Ìʈӷ Ì ÙËÓÈÛ¯‡Ô˘Û· Ù·ÍÈÓfiÌËÛË (2-4) (Ô˘ ÛÙËÚ›˙ÂÙ·È ÛÙ·Â˘Ú‹Ì·Ù· Ù˘ Ì·ÁÓËÙÈ΋˜ ÙÔÌÔÁÚ·Ê›·˜) ηȷӿÏÔÁ· Ì ÙÔ ·ÂÈÎÔÓÈ˙fiÌÂÓÔ ÛÙ¿‰ÈÔ, ·ÔÊ·-Û›˙ÂÙ·È Ë ÂÊ·ÚÌÔÛÙ¤· ıÂڷ›·.

∆Ô ÛÙ¿‰ÈÔ 1 ÂÚÈÏ·Ì‚¿ÓÂÈ ‚Ï¿‚Ë ÙÔ˘ ·Ú-ıÚÈÎÔ‡ ¯fiÓ‰ÚÔ˘ ÌfiÓÔ, Ô˘ ‰ÂÓ Á›ÓÂÙ·È ÔÚ·Ù‹ÛÙȘ ·ÎÙÈÓÔÁڷʛ˜ Î·È ‰ÂÓ ··ÈÙ› ¿ÏÏË ıÂ-ڷ›· ·fi ÙËÓ Ù·ÎÙÈ΋ ·Ú·ÎÔÏÔ‡ıËÛË.

∆Ô ÛÙ¿‰ÈÔ 2 ÂÚÈÏ·Ì‚¿ÓÂÈ, ÂÎÙfi˜ ·fi ÙË‚Ï¿‚Ë ÙÔ˘ ·ÚıÚÈÎÔ‡ ¯fiÓ‰ÚÔ˘, οٷÁÌ· ÙÔ˘˘ÔΛÌÂÓÔ˘ ÔÛÙÔ‡, Ì ÔÛÙÈÎfi Ô›‰ËÌ· (ÛÙ¿‰ÈÔ2·, fiˆ˜ ÛÙËÓ ÂÚ›ÙˆÛË Ô˘ ÂÚÈÁÚ¿ÊÂÙ·È)‹ ¯ˆÚ›˜ Ô›‰ËÌ· (2‚). ™Ù· ÛÙ¿‰È· ·˘Ù¿, Ë ÚÔ-ÙÂÈÓfiÌÂÓË ıÂڷ›· Â›Ó·È Û˘ÓÙËÚËÙÈ΋, Ì·ÎÈÓËÙÔÔ›ËÛË Û ӿÚıËη Î·È ·ÔÊ˘Á‹ Ï‹-ÚÔ˘˜ ÊfiÚÙÈÛ˘ ÙÔ˘ ÛΤÏÔ˘˜ ÁÈ· 6 ÙÔ˘Ï¿¯ÈÛÙÔÓ‚‰ÔÌ¿‰Â˜.

™ÙÔ ÛÙ¿‰ÈÔ 3, ·Ó·Ê·›ÓÂÙ·È ‰È·¯ˆÚÈÛÌfi˜,·ÏÏ¿ fi¯È ·ÚÂÎÙfiÈÛË, ÔÛÙÂÔ¯fiÓ‰ÚÈÓÔ˘ ÙÂÌ·-¯›Ô˘. ™ÙÔ ÛÙ¿‰ÈÔ 4, ÙÔ ‰È·¯ˆÚÈṲ̂ÓÔ ÙÂÌ¿¯ÈÔ¤¯ÂÈ ·ÔÎÔÏÏËı› Î·È Û˘ÌÂÚÈʤÚÂÙ·È ˆ˜ÂχıÂÚÔ ÛÒÌ· ÛÙÔ ÂÛˆÙÂÚÈÎfi Ù˘ ¿ÚıÚˆÛ˘.

™ÙÔ ÛÙ¿‰ÈÔ 5, ÂÎÙfi˜ ·fi Ù· ·ÓˆÙ¤Úˆ, ·Ó·-Ê·›ÓÔÓÙ·È Î˘ÛÙÈΤ˜ ·ÏÏÔÈÒÛÂȘ ÛÙÔ ˘Ô¯fiÓ-‰ÚÈÔ ÔÛÙÔ‡Ó.

™Ù· ÛÙ¿‰È· 3-5, Ë ıÂڷ›· Â›Ó·È ¯ÂÈÚÔ˘Ú-ÁÈ΋. ¶ÚÔÙÈÌ¿Ù·È Ë Û˘ÁÎÚ¿ÙËÛË ÙÔ˘ ·ÔÎÔÏ-ÏË̤ÓÔ˘ ÙÂÌ·¯›Ô˘, ÂÊfiÛÔÓ Â›Ó·È ÌÂÁ¿ÏÔ Î·È·Ó‹ÎÂÈ Û ÊÔÚÙÈ˙fiÌÂÓË ·ÚıÚÈ΋ ÂÈÊ¿ÓÂÈ·, ‹Ë ·Ê·›ÚÂÛ‹ ÙÔ˘ ÂÊfiÛÔÓ Â›Ó·È ÌÈÎÚfi Î·È Ë·Ô˘Û›· ÙÔ˘ ‰ÂÓ ‰È·Ù·Ú¿ÛÛÂÈ ÙË ÏÂÈÙÔ˘ÚÁÈÎfi-ÙËÙ· Ù˘ ¿ÚıÚˆÛ˘ (5,6).

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¢È¿ÁÓˆÛË: ™¯·ÏȉˆÙÈ΋ ÔÛÙÂÔ¯ÔÓ‰Ú›Ùȉ· ·ÛÙÚ·Á¿ÏÔ˘

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µÈ‚ÏÈÔÁÚ·Ê›·

1. Berndt AL, Harty M. Transchondral fractures(osteochondritis dissecans) of the talus. J BoneJoint Surg 1959;41A:988-1020.

2. Stroud CC, Marks RM. Imaging of osteochondrallesions of the talus. Foot Ankle Clin Mar2000;5:119-133.

3. Elias I, Jung JW, Raikin SM, Schweitzer MW,Carrino JA, Morrison WB. Osteochondral lesionsof the talus: change in MRI findings over time intalar lesions without operative intervention andimplications for staging systems. Foot Ankle Int2006;27:157-166.

4. Hepple S, Winson IG, Glew D. Osteochondrallesions of the talus: a revised classification. FootAnkle Int 1999;20:789-793.

5. Tol JL, Struijs PA, Bossuyt PM, Verhagen RA, vanDijk CN. Treatment strategies in osteochondraldefects of the talar dome: a systematic review. FootAnkle Int 2000;21:119-126.

6. Giannini S, Buda R, Faldini C, Vannini F, Bevoni R,Grandi G. Surgical treatment of osteochondrallesions of the talus in young active patients. J BoneJoint Surg Am 2005;87(Suppl 2):28-41.

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