Qiongfen Lin, Paul Kwong-Hang Tam, Clara Sze-Man Tang
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引用次数: 0
Abstract
Biliary atresia (BA) is a severe neonatal cholestatic disorder marked by fibro-obliteration of the extrahepatic and intrahepatic bile ducts. It is the most common cause of pediatric end-stage liver disease and the leading indication for liver transplantation in children. There is significant heterogeneity in the etiology, involving various genetic and environmental factors such as viral infection, immune dysregulation and genetic predisposition to defective hepatobiliary development. In this review, we discuss the strategies to uncover the genetic factors underlying BA and highlight their associated molecular and pathological mechanisms, as well as the challenges faced in this area of research.
期刊介绍：
Seminars in Pediatric Surgery provides current state-of-the-art reviews of subjects of interest to those charged with the surgical care of young patients. Each bimontly issue addresses a single topic with articles written by the experts in the field. Guest editors, all noted authorities, prepare each issue.
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