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International Incidence and Outcomes of Biliary Atresia

Jimenez-Rivera, Carolina*; Jolin-Dahel, Kheira S.*; Fortinsky, Kyle J.*; Gozdyra, Peter; Benchimol, Eric I.*

Journal of Pediatric Gastroenterology and Nutrition: April 2013 - Volume 56 - Issue 4 - p 344–354
doi: 10.1097/MPG.0b013e318282a913
Invited Review

Objectives: International trends in incidence and outcomes of biliary atresia (BA) are controversial and a wide range of estimates have been reported worldwide. We reviewed the population-based literature to assess international variation of BA incidence and outcomes, and to assess the evidence for seasonal variation in incidence, centralization of Kasai hepatoportoenterostomy, and newborn screening.

Methods: We conducted a systematic review (registration number CRD42011001441) of observational or interventional research within MEDLINE, EMBASE, and the Cochrane Database, which reported incidence, prevalence, or outcomes of infants with BA. Population-based studies, defined by inclusion of an entire population or representative sample, were included. Outcomes included overall survival, native liver survival (NLS), and time to Kasai hepatoportoenterostomy. Single- or multicenter studies were excluded unless those centers captured all potential patients within a jurisdiction. Two independent data extractors reviewed the abstracts and articles.

Results: A total of 40 studies were included following review of 3128 references. A wide range of incidence was reported internationally. Ten-year overall survival ranged from 66.7% to 89%. NLS ranged from 20.3% to 75.8% at 1 to 3 years and 24% to 52.8% at 10 years. Earlier age at Kasai was a predictor of improved NLS. Seasonality was reported in 11 studies, and 3 reported an increased incidence during the months of August to March. The evidence for centralization of Kasai to high-volume centers is promising but does not account for all case-mix, provider, or health system factors involved in volume–outcome relations. Stool color card screening resulted in earlier Kasai and improved NLS in Taiwan.

Conclusions: Large, international studies could help fill the gaps in knowledge identified by this review.

Supplemental Digital Content is available in the text

*Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada

Institute for Clinical Evaluative Sciences, Toronto, ON, Canada.

Address correspondence and reprint requests to Carolina Jimenez-Rivera, MD, Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON K1H 8L1, Canada (e-mail:

Received 27 July, 2012

Accepted 11 December, 2012

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E.I.B. was supported by a Career Development Award from the Canadian Child Health Clinician Scientist Program.

The authors report no conflicts of interest.

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