TY - JOUR
T1 - A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000
AU - Shneider, Benjamin L.
AU - Brown, Morton B.
AU - Haber, Barbara
AU - Whitington, Peter F.
AU - Schwarz, Kathleen
AU - Squires, Robert
AU - Bezerra, Jorge
AU - Shepherd, Ross
AU - Rosenthal, Philip
AU - Hoofnagle, Jay H.
AU - Sokol, Ronald J.
N1 - Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2006/4
Y1 - 2006/4
N2 - Objective: To determine the prognostic factors and optimal approaches to the diagnosis and management of biliary atresia, the leading indication for liver transplantation in children. Study design: A retrospective study was performed of all children who underwent hepatoportoenterostomy (HPE) for biliary atresia between 1997 and 2000 at 9 centers in the United States. Outcome at age 24 months was correlated with demographic and clinical parameters. Results: A total of 104 children underwent HPE; 25% had congenital anomalies, and outcome was worse in those with biliary atresia splenic malformation syndrome. Diagnostic and clinical approaches varied, although specific approaches did not appear to correlate with outcome. The average age at referral was 53 days, and the average age at HPE was 61 days. At age 24 months, 58 children were alive with their native liver, 42 had undergone liver transplantation (37 alive, 5 dead), and 4 had died without undergoing transplantation. Kaplan-Meier analysis of survival without liver transplantation revealed markedly improved survival in children with total bilirubin level < 2 mg/dL at 3 months after HPE (84% vs 16%; P < .0001). Conclusions: Outcome in the study centers was equivalent to that reported in other countries. Total bilirubin in early follow-up after HPE was highly predictive of outcome. Efforts to improve bile flow after HPE may lead to improved outcome in children with biliary atresia.
AB - Objective: To determine the prognostic factors and optimal approaches to the diagnosis and management of biliary atresia, the leading indication for liver transplantation in children. Study design: A retrospective study was performed of all children who underwent hepatoportoenterostomy (HPE) for biliary atresia between 1997 and 2000 at 9 centers in the United States. Outcome at age 24 months was correlated with demographic and clinical parameters. Results: A total of 104 children underwent HPE; 25% had congenital anomalies, and outcome was worse in those with biliary atresia splenic malformation syndrome. Diagnostic and clinical approaches varied, although specific approaches did not appear to correlate with outcome. The average age at referral was 53 days, and the average age at HPE was 61 days. At age 24 months, 58 children were alive with their native liver, 42 had undergone liver transplantation (37 alive, 5 dead), and 4 had died without undergoing transplantation. Kaplan-Meier analysis of survival without liver transplantation revealed markedly improved survival in children with total bilirubin level < 2 mg/dL at 3 months after HPE (84% vs 16%; P < .0001). Conclusions: Outcome in the study centers was equivalent to that reported in other countries. Total bilirubin in early follow-up after HPE was highly predictive of outcome. Efforts to improve bile flow after HPE may lead to improved outcome in children with biliary atresia.
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U2 - 10.1016/j.jpeds.2005.12.054
DO - 10.1016/j.jpeds.2005.12.054
M3 - Article
C2 - 16647406
AN - SCOPUS:33646086401
SN - 0022-3476
VL - 148
SP - 467-467.e
JO - Journal of Pediatrics
JF - Journal of Pediatrics
IS - 4
ER -