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.
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.
Authors: Sanjiv Harpavat; Philip J Lupo; Loriel Liwanag; John Hollier; Mary L Brandt; Milton J Finegold; Benjamin L Shneider Journal: J Pediatr Gastroenterol Nutr Date: 2018-06 Impact factor: 2.839
Authors: Benjamin L Shneider; John C Magee; Saul J Karpen; Elizabeth B Rand; Michael R Narkewicz; Lee M Bass; Kathleen Schwarz; Peter F Whitington; Jorge A Bezerra; Nanda Kerkar; Barbara Haber; Philip Rosenthal; Yumirle P Turmelle; Jean P Molleston; Karen F Murray; Vicky L Ng; Kasper S Wang; Rene Romero; Robert H Squires; Ronen Arnon; Averell H Sherker; Jeffrey Moore; Wen Ye; Ronald J Sokol Journal: J Pediatr Date: 2015-12-24 Impact factor: 4.406
Authors: Mary Elizabeth M Tessier; Sanjiv Harpavat; Ross W Shepherd; Girish S Hiremath; Mary L Brandt; Amy Fisher; John A Goss Journal: World J Gastroenterol Date: 2014-08-28 Impact factor: 5.742
Authors: Suzan L Carmichael; Chen Ma; Alissa R Van Zutphen; Cynthia A Moore; Gary M Shaw Journal: Birth Defects Res Date: 2018-05-15 Impact factor: 2.344