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Review
. 2015 Jun;27(3):389-94.
doi: 10.1097/MOP.0000000000000214.

Recent advances in the pathogenesis and management of biliary atresia

Jessica A Zagory  1 , Marie V Nguyen, Kasper S Wang
Affiliations
Review

Recent advances in the pathogenesis and management of biliary atresia

Jessica A Zagory et al. Curr Opin Pediatr. 2015 Jun.

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.

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