Biliary atresia clinically manifests by 2 weeks of age with jaundice due to a conjugated hyperbilirubinemia and pale acholic stools. All babies with jaundice persisting beyond 2 weeks should have a blood test for total and conjugated (direct) bilirubin. If the conjugated (direct) bilirubin fraction is >20% of the total bilirubin, prompt referral to assess for biliary atresia is necessary. Timely diagnosis and early surgical intervention before 30 days of age offers the best outcomes for patient survival with their own liver without the need for liver transplantation. For more information please see cbar.ca.

Conditions: Infant Jaundice, Jaundice in Newborn, Biliary Atresia

Diagnostic Tests: Total Bilirubin, Conjugated Bilirubin, Direct Bilirubin

Sources

• Guidelines for detection, management and prevention of hyperbilirubinemia in term and late preterm newborn infants (35 or more weeks' gestation) - Summary. Paediatr Child Health. 2007 May;12(5):401-18. (PubMed)
• Schreiber RA, Barker CC, Roberts EA, Martin SR, Alvarez F, Smith L, et al. Biliary atresia: the Canadian experience. J Pediatr. 2007 Dec;151(6):659-65, 665.e1. (PubMed)
• Wildhaber BE, Majno P, Mayr J, Zachariou Z, Hohlfeld J, Schwoebel M, et al. Biliary atresia: Swiss national study, 1994-2004. J Pediatr Gastroenterol Nutr. 2008 Mar;46(3):299-307. (PubMed)