Department of Pediatric Surgery, Ibaraki Children's Hospital, Mito, 311-4145, Japan.
Expert Rev Gastroenterol Hepatol. 2009 Dec;3(6):599-606. doi: 10.1586/egh.09.61.
Biliary atresia is an idiopathic neonatal cholestatic disease characterized by the destruction of both the intra- and extra-hepatic biliary ducts. There are two clinical manifestations of the disease: an embryonal subtype, which often presents at birth and is associated with congenital malformations, and a 'perinatal' subtype, which is probably an acquired disease due to unknown etiology. Over the last two decades, researchers have focused on activation of the cell-mediated immunity as the mechanism for biliary epithelial cell destruction for the latter subtype. A proposed trigger of this immune response is an initial viral infection, inducing biliary epithelial cells to become antigen-presenting cells and thus instigating immune-mediated destruction of the biliary tract. However, putative viruses have never been confirmed. More recently, a novel hypothesis - that maternal microchimerism may initiate a host immunologic response towards the bile duct epithelia - has been proposed. This paper discusses the etiology of biliary atresia in the context of the current research.
先天性胆道闭锁是一种特发性新生儿胆汁淤积性疾病，其特征是肝内外胆管同时破坏。该病有两种临床表现：一种是胚胎亚型，常发生在出生时，与先天性畸形有关；另一种是“围生期”亚型，可能由于病因不明而导致后天获得性疾病。在过去的二十年中，研究人员一直关注细胞介导免疫的激活，作为导致后一种亚型的胆管上皮细胞破坏的机制。这种免疫反应的一个潜在触发因素是最初的病毒感染，导致胆管上皮细胞成为抗原呈递细胞，从而引发免疫介导的胆管破坏。然而，潜在的病毒从未被证实。最近，提出了一个新的假说，即母体微嵌合体可能引发宿主对胆管上皮的免疫反应。本文讨论了目前研究中先天性胆道闭锁的病因。
