Biliary Atresia: Clinical Lessons Learned.

Abstract

Biliary atresia is a rare disease of unclear etiology, in which obstruction of the biliary tree causes severe cholestasis leading to cirrhosis and ultimately death if left untreated. Biliary atresia is the leading cause of neonatal cholestasis and the most frequent indication for pediatric liver transplantation. Any infant with persistent jaundice beyond 2 weeks of life needs to be evaluated for biliary atresia with fractionation of the bilirubin into conjugated and unconjugated portions. Early performance of a hepatoportoenterostomy in the first 45 days of life to restore bile flow and lessen further damage to the liver is thought to optimize outcome. Despite surgery, progressive liver scarring occurs, and 80% of patients with biliary atresia will require liver transplantation during childhood.

• Authors: Amy G Feldman, Cara L Mack
• Journal: Journal of pediatric gastroenterology and nutrition (J Pediatr Gastroenterol Nutr) Vol. 61 Issue 2 Pg. 167-75 (Aug 2015) ISSN: 1536-4801 [Electronic] United States
• PMID: 25658057 (Publication Type: Journal Article, Review)

Chemical References

• Bilirubin

Topics

• Adaptive Immunity
• Biliary Atresia (complications, diagnosis, surgery)
• Bilirubin (blood)
• Cholangitis (etiology)
• Cholestasis (etiology)
• Diagnosis, Differential
• Enterostomy
• Failure to Thrive
• Humans
• Hypertension, Portal (etiology)
• Hypertension, Pulmonary (etiology)
• Infant
• Infant, Newborn
• Liver Cirrhosis, Biliary (etiology, surgery)
• Liver Transplantation
• Neonatal Screening
• Treatment Outcome