胆道闭锁发病机制与治疗的最新进展
Recent advances in the pathogenesis and management of biliary atresia.
作者信息
Zagory Jessica A, Nguyen Marie V, Wang Kasper S
机构信息
Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, California, USA.
出版信息
Curr Opin Pediatr. 2015 Jun;27(3):389-94. doi: 10.1097/MOP.0000000000000214.
Abstract
PURPOSE OF REVIEW
The purpose of this study is to review advances in both the pathogenesis and clinical management of biliary atresia.
RECENT FINDINGS
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 biliary atresia. A number of genetic polymorphisms have been characterized as leading to susceptibility for biliary atresia. Postoperative corticosteroid therapy is not associated with greater transplant-free survival. Newborn screening may improve outcomes of infants with biliary atresia and may also provide a long-term cost benefit.
SUMMARY
Although recent advances have enhanced our understanding of pathogenesis and clinical management, biliary atresia remains a significant challenge requiring further investigation.
摘要
综述目的
本研究旨在综述胆道闭锁发病机制及临床管理方面的进展。
最新发现
免疫研究进一步明确了辅助性T细胞、B细胞和自然杀伤细胞在病毒在胆管上皮内复制及损伤后免疫失调中的作用。表达Prominin-1的门周成纤维细胞可能在与胆道闭锁相关的胆管纤维化中起重要作用。一些基因多态性已被确定为导致胆道闭锁易感性的因素。术后皮质类固醇治疗与更高的无移植生存率无关。新生儿筛查可能改善胆道闭锁婴儿的预后，也可能带来长期成本效益。
总结
尽管最近的进展增进了我们对发病机制和临床管理的理解，但胆道闭锁仍然是一个重大挑战，需要进一步研究。
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