title: Recent advances in the pathogenesis and management of biliary atresia.
abstract: OBJECTIVE
  The purpose of this study is to review advances in both the pathogenesis and clinical management of biliary atresia.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  Although recent advances have enhanced our understanding of pathogenesis and clinical management, biliary atresia remains a significant challenge requiring further investigation.
authors.affiliation: Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, California, USA.
authors.forename: Jessica A
authors.initials: JA
authors.lastname: Zagory
authors.forename: Marie V
authors.initials: MV
authors.lastname: Nguyen
authors.forename: Kasper S
authors.initials: KS
authors.lastname: Wang
mesh_terms: D001653:Bile Ducts, Intrahepatic / Q000473:pathology*; D001656:Biliary Atresia / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D042241:Early Diagnosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation / Q000706:statistics & numerical data; D011171:Portoenterostomy, Hepatic / Q000379:methods*; D011379:Prognosis; D016896:Treatment Outcome