Hirschsprung's associated enterocolitis.
Hirschsprung's associated enterocolitis.
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DOI：
10.1097/mop.0000000000000210
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发表时间：
2015-06
影响因子：
3.6
通讯作者：
Brinkman AS
中科院分区：
文献类型：
--
作者：
Gosain A;Brinkman AS
先天性巨结肠（Hirschsprung's Disease，HSCR）的特征是远端后肠缺乏神经节细胞，从直肠向近端延伸可变距离，并且是由颅-尾神经嵴细胞迁移失败引起的。先天性巨结肠相关性小肠结肠炎（HAEC）是一种具有典型表现的疾病，包括腹胀、发热和恶臭粪便，是HSCR的重要且危及生命的并发症。这篇综述的目的是批判性地评价最近的研究结果有关的HAEC的病理生理。最近的几项研究已经调查了人类和小鼠模型中HAEC的病因。这些研究表明，肠道屏障的改变，包括杯状细胞数量和功能以及潘氏细胞功能，胃肠道粘膜免疫受损，包括B淋巴细胞运输或功能以及分泌型伊加产生，以及肠道微生物群的生态失调可能有助于HAEC的发展。最近的研究增加了文献的主体，表明在HSCR中观察到的肠道缺陷不限于无神经节段，而是延伸到胃肠道内外的粘膜免疫系统。进一步剖析HAEC机制并在人类患者中验证这些发现的未来研究将允许开发定向治疗干预。
Hirschsprung’s Disease (HSCR) is characterized by an absence of ganglion cells in the distal hindgut, extending from the rectum to a variable distance proximally, and results from a failure of cranial-caudal neural crest cell migration. Hirschsprung’s-Associated Enterocolitis (HAEC) is a condition with classic manifestations that include abdominal distention, fever and foul-smelling stools, and is a significant and life-threatening complication of HSCR. The purpose of this review is to critically evaluate recent findings regarding the pathophysiology of HAEC. Several recent studies have investigated the etiology of HAEC in humans and mouse models. These studies suggest that alterations in the intestinal barrier, including goblet cell number and function and Paneth cell function, impaired gastrointestinal mucosal immunity, including B-lymphocyte trafficking or function and secretory IgA production, and dysbiosis of the intestinal microbiota may contribute to the development of HAEC. Recent studies add to the body of literature suggesting that the intestinal defects observed in HSCR are not restricted to the aganglionic segment but extend to the mucosal immune system within and beyond the gastrointestinal tract. Future studies further dissecting mechanisms of HAEC and validating these findings in human patients will allow for development of directed therapeutic interventions.