Recent advances in the pathogenesis and management of biliary atresia
- Curr Opin Pediatr. 2015 Jun;27(3):389-94. doi: 10.1097/MOP.0000000000000214.
- 1. Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, California, USA.
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.