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巨膀胱-小结肠-肠蠕动不良综合征:一名男婴的存活情况

Megacystis-microcolon-intestinal hypoperistalsis syndrome: survival of a male infant.

作者信息

Gillis D A, Grantmyre E B

出版信息

J Pediatr Surg. 1985 Jun;20(3):279-81. doi: 10.1016/s0022-3468(85)80122-6.

DOI:10.1016/s0022-3468(85)80122-6
PMID:3925110
Abstract

The combination of megacystis with microcolon and severe intestinal hypoperistalsis constitutes a rarely encountered syndrome in neonates. Of 16 previously reported cases, 14 have occurred in females and all but one have been fatal. Various forms of intestinal diversions have been unsuccessful. Aganglionosis has been excluded in every case. A method of management involving prolonged total parenteral nutrition, followed by graduated jejunostomy and gastrostomy feedings, has resulted in the survival of a male infant with this syndrome. No previous male survivors have been reported.

摘要

巨大膀胱合并小结肠及严重肠道蠕动减弱是新生儿中一种罕见的综合征。在先前报道的16例病例中,14例为女性,除1例之外全部死亡。各种形式的肠道改道术均未成功。每例均排除了无神经节症。一种治疗方法包括长期全胃肠外营养,随后逐步进行空肠造口术和胃造口术喂养,该方法使一名患有此综合征的男婴存活下来。此前尚无男婴存活的报道。

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