ArticleHepatology (Baltimore, Md.)2022
Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia.
Article in Hepatology (Baltimore, Md.), 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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11 citing papers in PubMed, 23 citations in OpenAlex.
- Secondary prophylaxis of variceal hemorrhage in biliary atresia-Analysis of prospective multicenter studies.Journal of pediatric gastroenterology and nutrition · 2026Article
- Application of machine learning in constructing a diagnostic model for neonatal biliary atresia.Pediatric investigation · 2025Article
- The role of partial splenic artery embolization in the management of refractory esophageal variceal bleeding due to portal vein thrombosis.BMC pediatrics · 2025Article
- Fibrotic liver injury in biliary atresia: long-term implications.World journal of pediatric surgery · 2025Review
- Biliary atresia.Nature reviews. Disease primers · 2024Review
- Umbilical hernia and clinical course of patients with bile duct atresia.Revista medica del Instituto Mexicano del Seguro Social · 2024Observational
- The magnetic resonance imaging and age-adjusted matrix metalloproteinase-7 assist the diagnosis of biliary atresia.Journal of gastroenterology · 2024Article
- Kasai Portoenterostomy, Successful Liver Transplantation, and Immunosuppressive Therapy for Biliary Atresia in a Female Baby: A Case Report.Journal of inflammation research · 2024Article
- Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia.Hepatology (Baltimore, Md.) · 2022Article
- Impact of early Kasai portoenterostomy on short-term outcomes of biliary atresia: A systematic review and meta-analysis.Frontiers in surgery · 2022Review
- Loci Associated With Susceptibility to Biliary Atresia-A Genome-Wide Association Study in Taiwan.Pediatrics international : official journal of the Japan Pediatric SocietyArticle
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Authors and funding
20 authors at 17 institutions in 2 countries.
Funding
Abstract
BACKGROUND AND
aimsThe natural history of gastroesophageal variceal hemorrhage (VH) in biliary atresia (BA) is not well characterized. We analyzed risk factors, incidence, and outcomes of VH in a longitudinal multicenter study. APPROACH AND
resultsParticipants enrolled in either an incident (Prospective Database of Infants with Cholestasis [PROBE]) or prevalent (Biliary Atresia Study of Infants and Children [BASIC]) cohort of BA were included. Variceal hemorrhage (VH) was defined based on gastrointestinal bleeding in the presence of varices accompanied by endoscopic or nontransplant surgical intervention. Cumulative incidence of VH and transplant-free survival was compared based on features of portal hypertension (e.g., splenomegaly, thrombocytopenia) and clinical parameters at baseline in each cohort (PROBE: 1.5 to 4.5 months after hepatoportoenterostomy [HPE]; BASIC: at enrollment > 3 years of age). Analyses were conducted on 869 children with BA enrolled between June 2004 and December 2020 (521 in PROBE [262 (51%) with a functioning HPE] and 348 in BASIC). The overall incidence of first observed VH at 5 years was 9.4% (95% CI: 7.0-12.4) in PROBE and 8.0% (5.2-11.5) in BASIC. Features of portal hypertension, platelet count, total bilirubin, aspartate aminotransferase (AST), albumin, and AST-to-platelet ratio index at baseline were associated with an increased risk of subsequent VH in both cohorts. Transplant-free survival at 5 years was 45.1% (40.5-49.6) in PROBE and 79.2% (74.1-83.4) in BASIC. Two (2.5%) of 80 participants who had VH died, whereas 10 (12.5%) underwent transplant within 6 weeks of VH.
conclusionsThe low risk of VH and associated mortality in children with BA needs to be considered in decisions related to screening for varices and primary prophylaxis of VH.
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