Biliary Atresia: Clinical Lessons Learned. J Pediatr Gastroenterol Nutr 2015 Aug;61(2):167-75
Date
02/07/2015Pubmed ID
25658057DOI
10.1097/MPG.0000000000000755Scopus ID
2-s2.0-84942832146 (requires institutional sign-in at Scopus site) 84 CitationsAbstract
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.
Author List
Feldman AG, Mack CLAuthor
Cara Lynn Mack MD Chief, Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Adaptive ImmunityBiliary Atresia
Bilirubin
Cholangitis
Cholestasis
Diagnosis, Differential
Enterostomy
Failure to Thrive
Humans
Hypertension, Portal
Hypertension, Pulmonary
Infant
Infant, Newborn
Liver Cirrhosis, Biliary
Liver Transplantation
Neonatal Screening
Treatment Outcome