The pathogenesis of Hirschsprung's disease-associated enterocolitis

被引：89

作者：

Austin, Kelly Miller ^{[1
]}

机构：

[1] Univ Pittsburgh, Sch Med, Childrens Hosp Pittsburgh, Div Pediat Gen & Thorac Surg, Pittsburgh, PA 15224 USA

来源：

SEMINARS IN PEDIATRIC SURGERY | 2012年 / 21卷 / 04期

关键词：

Hirschsprune disease; Enterocolitis; Etiology; Intestinal barrier function; Intestinal microbiota; ENDORECTAL PULL-THROUGH; ENTERIC NERVOUS-SYSTEM; CLINICAL RISK-FACTORS; MUCOSAL DEFENSE; DOWNS-SYNDROME; PREOPERATIVE ENTEROCOLITIS; CLOSTRIDIUM-DIFFICILE; SURGICAL-CORRECTION; INTESTINAL BARRIER; 28-YEAR EXPERIENCE;

D O I：

10.1053/j.sempedsurg.2012.07.006

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Hirschsprung's disease-associated enterocolitis (HAEC) remains the most life-threatening complication in Hirschsprung disease (HD) patients. The pathogenesis of HAEC has not been determined and many hypotheses regarding the etiology of HAEC have been proposed. These include a possible causal relationship between the abnormal enteric nervous system development in HD and the development of enterocolitis. Based on the complex genetic causes of HD that have been discovered and the resultant heterogeneous group of patients that exists, the causes of HAEC are likely multiple. New insights regarding the relationship of the role of the enteric nervous system and its interaction between intestinal barrier function, innate host immunity, and commensal microflora have been discovered, which may shed light on this perplexing problem. This review presents current known risk factors of HAEC and the proposed theories and supporting evidence for the potential etiologies of HAEC. (C) 2012 Published by Elsevier Inc.

引用

页码：319 / 327

页数：9

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