Novel Insights into the Pathogenesis of Hirschsprung's-associated Enterocolitis.
Novel Insights into the Pathogenesis of Hirschsprung's-associated Enterocolitis.
复制标题
DOI：
10.4103/0366-6999.183433
复制
发表时间：
2016-06-20
影响因子：
6.1
通讯作者：
Feng JX
中科院分区：
文献类型：
--
作者：
Jiao CL;Chen XY;Feng JX
系统总结先天性巨结肠相关性小肠结肠炎（HAEC）发病机制的最新研究成果。并在此基础上讨论了今后的研究重点和方向。我们在本综述中引用的数据主要来自1975年至2015年的PubMed，关键词为“Hirschsprung小肠结肠炎”、“Hirschsprung小肠结肠炎”、“Hirschsprung相关小肠结肠炎”、“Hirschsprung相关小肠结肠炎”、“HAEC”和“EC”。选择与HAEC发病机制相关的文献，排除主要与诊断、手术入路、治疗和随访相关的文献。HAEC的发病与多种因素有关，主要包括粘液屏障、肠道菌群、免疫功能等，同时也与遗传变异、手术原因等因素有关。粘液的数量和屏障性质的改变、微生物群的不同组成以及异常的免疫状态共同或单独地触发HAEC。肠道内环境稳定的维持是由于微生物群、粘液屏障和免疫系统的良好合作。如果任何一个部位出现异常，肠道内稳态就会被打破。同时，在先天性巨结肠（Hirschsprung 'sdisease，HAEC）患者中，这些部位功能紊乱，因此，HAEC的发生可能主要是由于肠道菌群、粘液屏障和免疫系统的紊乱。
To systematically summary the updated results about the pathogenesis of Hirschsprung's-associated enterocolitis (HAEC). Besides, we discussed the research key and direction based on these results. Our data cited in this review were obtained mainly from PubMed from 1975 to 2015, with keywords “Hirschsprung enterocolitis”, “Hirschsprung's enterocolitis”, “Hirschsprung's-associated enterocolitis”, “Hirschsprung-associated enterocolitis”, “HAEC”, and “EC”. Articles regarding the pathogenesis of HAEC were selected, and the articles mainly regarding the diagnosis, surgical approach, treatment, and follow-up were excluded. Several factors, mainly including mucus barrier, intestinal microbiota, and immune function, as well as some other factors such as genetic variations and surgical reasons, have been found to be related to the pathogenesis of HAEC. Changed quantity and barrier property of mucus, different composition of microbiota, and an abnormal immune state work together or separately trigger HAEC. The maintenance of intestinal homeostasis is due to a well cooperation of microbiota, mucus barrier, and immune system. If any part presents abnormal, intestinal homeostasis will be broken. Meanwhile, for patients with Hirschsprung's disease or HAEC, dysfunction of these parts has been found. Thus, the happening of HAEC may be mainly attributed to the disorders of intestinal microbiota, mucus barrier, and immune system.