Biliary Atresia: The Canadian Experience

Schreiber, Richard A.; Barker, Collin C.; Roberts, Eve A.; Martin, Steven R.; Alvarez, Fernando; Smith, Lesley; Butzner, J. Decker; Wrobel, Iwona; Mack, David; Moroz, Stanley; Rashid, Mohsin; Persad, Rabin; Levesque, Dominique; Brill, Herbert; Bruce, Garth; Critch, Jeff; Canadian Pediatric Hepatology Research Group,

doi:10.1016/j.jpeds.2007.05.051

« Previous Next »The Journal of Pediatrics
Volume 151, Issue 6 , Pages 659-665.e1, December 2007

Biliary Atresia: The Canadian Experience

This work was presented in part at the NIH research workshop: “Screening and outcomes in biliary atresia,” September 11-12, 2006.

Richard A. Schreiber, MD, FRCP(C)
- Department of Pediatrics: the University of British Columbia, Vancouver, British Columbia
- 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.
Collin C. Barker, MSc(Epid), MD, FRCP(C)
- Department of Pediatrics: the University of British Columbia, Vancouver, British Columbia
Eve A. Roberts, MD, FRCP(C)
- Department of Pediatrics: University of Toronto, Toronto, Ontario
Steven R. Martin, MD, FRCP(C)
- Department of Pediatrics: University of Montreal, Montreal, Quebec
Fernando Alvarez, MD
- Department of Pediatrics: University of Montreal, Montreal, Quebec
Lesley Smith, MD, FRCP(C)
- Department of Pediatrics: University of Alberta, Edmonton, Alberta
J. Decker Butzner, MD, FRCP(C)
- Department of Pediatrics: University of Calgary, Calgary, Alberta
Iwona Wrobel, MD, FRCP(C)
- Department of Pediatrics: University of Calgary, Calgary, Alberta
David Mack, MD, FRCP(C)
- Department of Pediatrics: University of Ottawa, Ottawa, Ontario
Stanley Moroz, MD, FRCP(C)
- Department of Pediatrics: University of Manitoba, Winnipeg, Manitoba
Mohsin Rashid, MD, FRCP(C)
- Department of Pediatrics: Dalhousie University, Halifax, Nova Scotia
Rabin Persad, MD, FRCP(C)
- Department of Pediatrics: McMaster University, Hamilton, Ontario
Dominique Levesque, MD, FRCP(C)
- Department of Pediatrics: McGill University, Montreal, Quebec
Herbert Brill, MD, FRCP(C)
- Department of Pediatrics: McGill University, Montreal, Quebec
Garth Bruce, MD, FRCP(C)
- Department of Pediatrics: University of Saskatchewan, Saskatoon, Saskatchewan
Jeff Critch, MD, FRCP(C)
- Department of Pediatrics: Memorial University, St. John’s, Newfoundland, Canada.
Canadian Pediatric Hepatology Research Group

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.

Abbreviations: BASM, Biliary atresia splenic malformation syndrome