Established and emerging concepts in Hirschsprung's-associated enterocolitis.
Established and emerging concepts in Hirschsprung's-associated enterocolitis.
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DOI：
10.1007/s00383-016-3862-9
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发表时间：
2016-04
影响因子：
1.8
通讯作者：
Gosain A
中科院分区：
文献类型：
--
作者：
Gosain A
先天性巨结肠是新生儿肠梗阻的常见原因，其诊断和手术治疗方法已被广泛接受。先天性巨结肠相关性小肠结肠炎（HAEC）仍然是术前和术后发病率和死亡率的常见原因，几十年来治疗指南没有改变。最近的进展，在我们的理解遗传学的基础HSCR允许动物模型的发展，其中一些概括的HAEC表型。这些动物模型，沿着最近的转化研究，在HAEC的发展中涉及粘膜免疫和微生物组生态失调的多个方面。在此，我们将回顾HAEC的流行病学，诊断和治疗模式。此外，我们将探索这种疾病发病机制中的新概念;包括动物模型，粘膜免疫的改变，肠道微生物组的生态失调，特定的遗传易感性和新的治疗方式。
Hirschsprung’s disease (HSCR) is a common cause of neonatal bowel obstruction and the approach to diagnosis and surgical treatment is well defined and accepted. Hirschsprung’s-associated enterocolitis (HAEC) remains a frequent cause of pre-operative and post-operative morbidity and mortality, with unchanged treatment guidelines over multiple decades. Recent advances in our understanding of the genetics underlying HSCR have allowed the development of animal models, some of which recapitulate the HAEC phenotype. These animal models, along with recent translational studies, have implicated multiple facets of mucosal immunity and microbiome dysbiosis in the development of HAEC. Here, we will review the established epidemiology, modes of diagnosis and treatment of HAEC. Furthermore, we will explore emerging concepts in the pathogenesis of this disease; including animal models, alterations in mucosal immunity, dysbiosis of the intestinal microbiome, specific genetic susceptibility, and novel treatment modalities.