Seminars in Liver Disease ( IF 4.3 ) Pub Date : 2021-06-15 , DOI: 10.1055/s-0041-1729972 Fernando Bessone 1 , Nelia Hernández 2 , Mario Tanno 1 , Marcelo G Roma 3
The most concerned issue in the context of drug/herb-induced chronic cholestasis is vanishing bile duct syndrome. The progressive destruction of intrahepatic bile ducts leading to ductopenia is usually not dose dependent, and has a delayed onset that should be suspected when abnormal serum cholestasis enzyme levels persist despite drug withdrawal. Immune-mediated cholangiocyte injury, direct cholangiocyte damage by drugs or their metabolites once in bile, and sustained exposure to toxic bile salts when biliary epithelium protective defenses are impaired are the main mechanisms of cholangiolar damage. Current therapeutic alternatives are scarce and have not shown consistent beneficial effects so far. This review will summarize the current literature on the main diagnostic tools of ductopenia and its histological features, and the differential diagnostic with other ductopenic diseases. In addition, pathomechanisms will be addressed, as well as the connection between them and the supportive and curative strategies for ductopenia management.
中文翻译:
药物性胆管消失综合征:从发病机制到诊断和治疗
在药物/草药引起的慢性胆汁淤积的背景下,最受关注的问题是胆管消失综合征。导致胆管减少的肝内胆管的进行性破坏通常不是剂量依赖性的,并且具有延迟发作,如果尽管停药但血清胆汁淤积酶水平异常持续存在,则应怀疑这种情况。免疫介导的胆管细胞损伤、药物或其代谢物一旦进入胆汁直接损伤胆管细胞,以及胆管上皮保护性防御受损时持续暴露于有毒胆汁盐是胆管损伤的主要机制。目前的治疗替代品很少,到目前为止还没有显示出一致的有益效果。本综述将总结目前关于输卵管减少症的主要诊断工具及其组织学特征的文献,以及与其他肾小管疾病的鉴别诊断。此外,还将讨论病理机制,以及它们与胆管减少症管理的支持和治疗策略之间的联系。