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Recent advances in the pathogenesis and management of biliary atresia.


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.

SUBMITTER: Zagory JA 

PROVIDER: S-EPMC4447429 | biostudies-literature | 2015 Jun

REPOSITORIES: biostudies-literature

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Recent advances in the pathogenesis and management of biliary atresia.

Zagory Jessica A JA   Nguyen Marie V MV   Wang Kasper S KS  

Current opinion in pediatrics 20150601 3


<h4>Purpose of review</h4>The purpose of this study is to review advances in both the pathogenesis and clinical management of biliary atresia.<h4>Recent findings</h4>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 numbe  ...[more]

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