Recent advances in the pathogenesis and management of biliary atresia.
Recent advances in the pathogenesis and management of biliary atresia.
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DOI：
10.1097/mop.0000000000000214
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发表时间：
2015-06
影响因子：
3.6
通讯作者：
Wang KS
中科院分区：
文献类型：
--
作者：
Zagory JA;Nguyen MV;Wang KS
本文就胆道闭锁的发病机制及临床治疗进展作一综述。免疫学研究进一步表征了辅助T细胞、B细胞和自然杀伤细胞在胆管上皮内病毒复制和损伤后免疫失调中的作用。表达PROMININ-1的门静脉成纤维细胞可能在与BA相关的胆道纤维化中发挥不可或缺的作用。许多遗传多态性已被表征为导致对BA的易感性。术后皮质类固醇治疗与更高的无移植存活率无关。新生儿筛查可以改善BA婴儿的结局，也可以提供长期的成本效益。虽然最近的进展已经提高了我们的发病机制和临床管理的理解，BA仍然是一个重大的挑战，需要进一步调查。
The purpose of this study is to review advances in both the pathogenesis and clinical management of biliary atresia (BA). Immunologic studies have further characterized roles of helper T-cells, B-cells, and natural killer cells in the immune dysregulation following viral replication within and damage of biliary epithelium. PROMININ-1 expressing portal fibroblasts may play an integral role in the biliary fibrosis associated with BA. A number of genetic polymorphisms have been characterized as leading to susceptibility for BA. Postoperative corticosteroid therapy is not associated with greater transplant-free survival. Newborn screening may improve outcomes of infants with BA and may also provide a long-term cost benefit. Although recent advances have enhanced our understanding of pathogenesis and clinical management, BA remains a significant challenge requiring further investigation.