Biliary atresia: cellular dynamics and immune dysregulation.

Biliary atresia: cellular dynamics and immune dysregulation.
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DOI:
10.1053/j.sempedsurg.2012.05.003
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发表时间:
2012-08
影响因子:
1.7
通讯作者:
Mack CL
Mack CL
中科院分区:
医学4区
文献类型:
--
作者:
Feldman AG;Mack CL

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胆道闭锁(BA)的病因尚不清楚,在过去的几十年里,与发病机制相关的研究大多集中在病毒感染和免疫方面。获得性或围产期的BA会导致胆管的进行性炎症损伤,导致肝外和肝内胆管的纤维化和闭塞。发病机制理论包括病毒感染、慢性炎症或自身免疫介导的胆管损伤和胆管发育异常。这篇综述将仅集中于与诊断时胆管损伤的潜在病毒触发有关的人类研究,并对先天性和获得性免疫反应在疾病发病机制中的相互作用提供洞察力。
The cause of biliary atresia (BA) is unknown and in the past few decades the majority of investigations related to pathogenesis have centered on virus infections and immunity. The acquired or perinatal form of BA entails a progressive, inflammatory injury of bile ducts, leading to fibrosis and obliteration of both the extrahepatic and intrahepatic bile ducts. Theories of pathogenesis include viral infection, chronic inflammatory or autoimmune-mediated bile duct injury and abnormalities in bile duct development. This review will focus solely on human studies pertaining to a potential viral trigger of bile duct injury at diagnosis and provide insight into the interplay of the innate and adaptive immune responses in the pathogenesis of disease.
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