Prognosis of Biliary Atresia After 2‐year Survival With Native Liver

• Published in: Journal of pediatric gastroenterology and nutrition Vol. 67; no. 6; pp. 689 - 694
• Main Authors: Witt, Mauri, Wessel, Daan B.E., Kleine, Ruben H.J., Bruggink, Janneke L.M., Hulscher, Jan B.F., Verkade, Henkjan J.
• Format: Journal Article
• Language: English
• Published: 01.12.2018
• Subjects: liver disease; liver transplantation; native liver survival; neonatal cholestasis; pediatrics
• ISSN: 0277-2116; 1536-4801
• DOI: 10.1097/MPG.0000000000002130

ABSTRACT Objectives: The aim of the study is to determine the prognosis of patients with biliary atresia after 2 years of native liver survival (NLS) and to identify prognostic factors for continued NLS after 2 years of age. Methods: We retrospectively analyzed perioperative, laboratory, and outcome parameters of all biliary atresia patients in The Netherlands between January 1987 and June 2015 with NLS of at least 2 years. We compared parameters between patients who continued to have their native liver (NLS+) to those who did not, either by transplant or death (NLS−). Results: We included 100 patients. Upon a median follow‐up of 16.4 years, NLS ended in 37% by liver transplantation (LTx) and in 6% by (pre‐transplant) mortality. NLS rates at 5, 10, 15, 18 years of age were 89%, 72%, 60%, 54%, respectively. Corresponding overall survival rates were 98%, 90%, 87%, 87%, respectively. Six months post‐Kasai, NLS+ patients had higher clearance of jaundice (COJ) rate, significantly lower total and direct serum bilirubin, aspartate‐aminotransferase and alkaline phosphatase levels, compared with NLS− patients (each P < 0.05). Cox regression could only assess a significant effect of COJ on continued NLS. Main indications for LTx after the age of 2 were irreversible jaundice and portal hypertension. Conclusions: Eighty‐seven percent of patients with 2‐year NLS reach adult age and more than 50% with their native liver. A pre‐transplant mortality of 6%, however, exists among patients who reach the age of 2 years with their native livers. Early life parameters, other than COJ, did not have a significant effect on continued NLS after 2 years of age.