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.
Schreiber
Richard A
RA
Department of Pediatrics, University of British Columbia, Vancouver, British Columbia. rschreiber@cw.bc.ca
Barker
Collin C
CC
Roberts
Eve A
EA
Martin
Steven R
SR
Alvarez
Fernando
F
Smith
Lesley
L
Butzner
J Decker
JD
Wrobel
Iwona
I
Mack
David
D
Moroz
Stanley
S
Rashid
Mohsin
M
Persad
Rabin
R
Levesque
Dominique
D
Brill
Herbert
H
Bruce
Garth
G
Critch
Jeff
J
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