Biliary atresia: will blocking inflammation tame the disease?
Biliary atresia: will blocking inflammation tame the disease?
复制标题
DOI：
10.1146/annurev-med-042909-093734
复制
发表时间：
2011
影响因子：
10.5
通讯作者：
Bezerra JA
中科院分区：
文献类型：
--
作者：
Bessho K;Bezerra JA
胆道闭锁是儿童最常见的胆道疾病。当肝外胆管段或全长完全梗阻时，及时行肝肠口造口术是恢复胆汁引流的最佳策略。然而，即使及时进行手术干预，持续的肝内胆管损伤和进行性胆管病变也会导致终末期肝硬化。疾病进展的速度并不统一；它可能与疾病的临床形式和/或诊断时肝脏病理的分期有关。虽然疾病的病因尚未确定，但一些生物学过程与胆管损伤的致病机制有关。其中，越来越多的证据表明，免疫系统以胆管上皮为靶点，破坏胆汁流动。在这篇综述中，我们讨论了仔细的临床表型、疾病分期和临床前研究如何深入了解对正在进行的试验的反应和合理设计新疗法以阻止疾病进展。
Biliary atresia is the most common cholangiopathy of childhood. With complete obstruction of segments or the entire length of extrahepatic bile ducts, the timely pursuit of hepatoportoenterostomy is the best strategy to restore bile drainage. However, even with prompt surgical intervention, ongoing injury of intrahepatic bile ducts and progressive cholangiopathy lead to end-stage cirrhosis. The pace of disease progression is not uniform; it may relate to clinical forms of disease and/or staging of liver pathology at diagnosis. Although the etiology of disease is not yet defined, several biological processes have been linked to pathogenic mechanisms of bile duct injury. Among them, there is increasing evidence that the immune system targets the duct epithelium and disrupts bile flow. In this review, we discuss how careful clinical phenotyping, staging of disease, and pre-clinical research give insight into response to ongoing trials and rational design of new therapies to block progression of disease.
登录
查看更多内容
影响因子：
2.4
作者：
Jafri, Mubeen;Donnelly, Bryan;Bondoc, Alex;Allen, Steven;Tiao, Greg
通讯作者：
Tiao, Greg
影响因子：
3.1
作者：
Arikan, Cigdem;Berdeli, Afig;Aydogdu, Sema
通讯作者：
Aydogdu, Sema
影响因子：
2.4
作者：
Davenport, M;Gonde, C;Howard, ER
通讯作者：
Howard, ER
影响因子：
1.1
作者：
HARPER, P;PLANT, JW;UNGER, DB
通讯作者：
UNGER, DB
影响因子：
2.4
作者：
Hayashida, Makoto;Nishimoto, Yuko;Taguchi, Tomoaki
通讯作者：
Taguchi, Tomoaki