Document Detail

International incidence and outcomes of biliary atresia.
MedLine Citation:
PMID:  23263590     Owner:  NLM     Status:  MEDLINE    
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.
Carolina Jimenez-Rivera; Kheira S Jolin-Dahel; Kyle J Fortinsky; Peter Gozdyra; Eric I Benchimol
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Journal of pediatric gastroenterology and nutrition     Volume:  56     ISSN:  1536-4801     ISO Abbreviation:  J. Pediatr. Gastroenterol. Nutr.     Publication Date:  2013 Apr 
Date Detail:
Created Date:  2013-03-26     Completed Date:  2013-09-17     Revised Date:  2013-10-09    
Medline Journal Info:
Nlm Unique ID:  8211545     Medline TA:  J Pediatr Gastroenterol Nutr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  344-54     Citation Subset:  IM    
Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.
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MeSH Terms
Biliary Atresia / diagnosis,  epidemiology*,  physiopathology,  therapy
Referral and Consultation
Survival Analysis
World Health*
Comment In:
J Pediatr Gastroenterol Nutr. 2013 Sep;57(3):e19-20   [PMID:  23799455 ]
J Pediatr Gastroenterol Nutr. 2013 Sep;57(3):e20   [PMID:  23799456 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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