This work was presented in part at the NIH research workshop: “Screening and outcomes in biliary atresia,” September 11-12, 2006.
Received 18 September 2006; received in revised form 8 May 2007; accepted 31 May 2007. published online 22 October 2007.
Objective
To determine the outcomes of Canadian children with biliary atresia.
Study design
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 ≤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.
aDepartment of Pediatrics: the University of British Columbia, Vancouver, British Columbia
bDepartment of Pediatrics: University of Toronto, Toronto, Ontario
cDepartment of Pediatrics: University of Montreal, Montreal, Quebec
dDepartment of Pediatrics: University of Alberta, Edmonton, Alberta
eDepartment of Pediatrics: University of Calgary, Calgary, Alberta
fDepartment of Pediatrics: University of Ottawa, Ottawa, Ontario
gDepartment of Pediatrics: University of Manitoba, Winnipeg, Manitoba
hDepartment of Pediatrics: Dalhousie University, Halifax, Nova Scotia
iDepartment of Pediatrics: McMaster University, Hamilton, Ontario
jDepartment of Pediatrics: McGill University, Montreal, Quebec
kDepartment of Pediatrics: University of Saskatchewan, Saskatoon, Saskatchewan
lDepartment of Pediatrics: Memorial University, St. John’s, Newfoundland, Canada.
Reprint requests: Dr. Richard Schreiber, Clinical Professor of Pediatrics, University of British Columbia, Division of Gastroenterology, BC Children’s Hospital, Room K4-200, 4480 Oak Street, Vancouver, BC, Canada V6H 3V4.
ABSTRACT