A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000.
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.


METHODS
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.
Shneider
Benjamin L
BL
Department of Pediatrics, Mount Sinai Medical Center, New York, NY 10029, Department of Biostatistics, University of Michigan, Ann Arbor, MI, and Department of Pediatrics, Children's Hospital of Philadelphia, PA, USA. Benjamin.Shneider@mssm.edu
Brown
Morton B
MB
Haber
Barbara
B
Whitington
Peter F
PF
Schwarz
Kathleen
K
Squires
Robert
R
Bezerra
Jorge
J
Shepherd
Ross
R
Rosenthal
Philip
P
Hoofnagle
Jay H
JH
Sokol
Ronald J
RJ
D000015:Abnormalities, Multiple / Q000453:epidemiology; D000367:Age Factors; D001656:Biliary Atresia / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000601:surgery*; D001663:Bilirubin / Q000378:metabolism; D015415:Biomarkers; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D019990:Perioperative Care; D011171:Portoenterostomy, Hepatic; D011183:Postoperative Complications / Q000453:epidemiology; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology
D015415:Biomarkers; D001663:Bilirubin