Biliary atresia: the Canadian experience.
OBJECTIVE
To determine the outcomes of Canadian children with biliary atresia.


METHODS
Health records of infants born in Canada between January 1, 1985 and December 31, 1995 (ERA I) and between January 1, 1996 and December 31, 2002 (ERA II) who were diagnosed with biliary atresia at a university center were reviewed.


RESULTS
349 patients were identified. Median patient age at time of the Kasai operation was 55 days. Median age at last follow-up was 70 months. The 4-year patient survival rate was 81% (ERA I = 74%; ERA II = 82%; P = not significant [NS]). Kaplan-Meier survival curves for patients undergoing the Kasai operation at age < or = 30, 31 to 90, and > 90 days showed 49%, 36%, and 23%, respectively, were alive with their native liver at 4 years (P < .0001). This difference continued through 10 years. The 2- and 4-year post-Kasai operation native liver survival rates were 47% and 35% for ERA I and 46% and 39% for ERA II (P = NS). A total of 210 patients (60%) underwent liver transplantation; the 4-year transplantation survival rate was 82% (ERA I = 83%, ERA II = 82%; P = NS).


CONCLUSIONS
This is the largest outcome series of North American children with biliary atresia at a time when liver transplantation was available. Results in each era were similar. Late referral remains problematic; policies to ensure timely diagnosis are required. Nevertheless, outcomes in Canada are comparable to those reported elsewhere.
Department of Pediatrics, University of British Columbia, Vancouver, British Columbia. rschreiber@cw.bc.ca
Richard A
RA
Schreiber
Collin C
CC
Barker
Eve A
EA
Roberts
Steven R
SR
Martin
Fernando
F
Alvarez
Lesley
L
Smith
J Decker
JD
Butzner
Iwona
I
Wrobel
David
D
Mack
Stanley
S
Moroz
Mohsin
M
Rashid
Rabin
R
Persad
Dominique
D
Levesque
Herbert
H
Brill
Garth
G
Bruce
Jeff
J
Critch
D001656:Biliary Atresia / Q000401:mortality / Q000601:surgery*; D001662:Biliary Tract Surgical Procedures / Q000401:mortality* / Q000706:statistics & numerical data; D002170:Canada / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D016031:Liver Transplantation / Q000401:mortality* / Q000706:statistics & numerical data; D008297:Male; D015996:Survival Rate; D016896:Treatment Outcome