Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
J Pediatr Gastroenterol Nutr. 2013 Apr;56(4):344-54. doi: 10.1097/MPG.0b013e318282a913.

International incidence and outcomes of biliary atresia.

Author information

  • 1Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. cajimenez@cheo.on.ca

Abstract

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.

Comment in

PMID:
23263590
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Lippincott Williams & Wilkins
    Loading ...
    Write to the Help Desk