{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T13:52:38Z","timestamp":1788357158500,"version":"build-2803163510"},"reference-count":34,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2022,4,18]],"date-time":"2022-04-18T00:00:00Z","timestamp":1650240000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Hepatology"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>The 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Approach and Results<\/jats:title>\n                    <jats:p>Participants 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\u2010free 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 &gt; 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\u201312.4) in PROBE and 8.0% (5.2\u201311.5) in BASIC. Features of portal hypertension, platelet count, total bilirubin, aspartate aminotransferase (AST), albumin, and AST\u2010to\u2010platelet ratio index at baseline were associated with an increased risk of subsequent VH in both cohorts. Transplant\u2010free survival at 5 years was 45.1% (40.5\u201349.6) in PROBE and 79.2% (74.1\u201383.4) in BASIC. Two (2.5%) of 80 participants who had VH died, whereas 10 (12.5%) underwent transplant within 6 weeks of VH.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The 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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hep.32451","type":"journal-article","created":{"date-parts":[[2022,3,10]],"date-time":"2022-03-10T15:52:56Z","timestamp":1646927576000},"page":"712-726","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":23,"title":["Risk of variceal hemorrhage and pretransplant mortality in children with biliary atresia"],"prefix":"10.1097","volume":"76","author":[{"given":"Lee M.","family":"Bass","sequence":"first","affiliation":[{"name":"Department of Pediatrics,Ann & Robert H. Lurie Children\u2019s Hospital of Chicago,Northwestern University Feinberg School of Medicine,Chicago,Illinois,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen","family":"Ye","sequence":"additional","affiliation":[{"name":"Department of Biostatistics,University of Michigan,Ann Arbor,Michigan,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kieran","family":"Hawthorne","sequence":"additional","affiliation":[{"name":"51330,Arbor Research Collaborative for Health,Ann Arbor,Michigan,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel H.","family":"Leung","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition,Department of Pediatrics,Baylor College of Medicine and Texas Children\u2019s Hospital,Houston,Texas,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen F.","family":"Murray","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology,Department of Pediatrics,Hepatology,Seattle Children\u2019s Hospital and the University of Washington School of Medicine,Seattle,Washington State,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean P.","family":"Molleston","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition,Department of Pediatrics,Riley Hospital for Children,Indiana University,Indianapolis,Indiana,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rene","family":"Romero","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition,Department of Pediatrics,Children\u2019s Healthcare of Atlanta and Emory University School of Medicine,Atlanta,Georgia,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saul","family":"Karpen","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition,Department of Pediatrics,Children\u2019s Healthcare of Atlanta and Emory University School of Medicine,Atlanta,Georgia,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Rosenthal","sequence":"additional","affiliation":[{"name":"Department of Pediatrics,University of California, San Francisco,San Francisco,California,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen M.","family":"Loomes","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition,The Children\u2019s Hospital of Philadelphia and Department of Pediatrics,Perelman School of Medicine at the University of Pennsylvania,Philadelphia,Pennsylvania,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kasper S.","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery,Children\u2019s Hospital Los Angeles,Los Angeles,California,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert H.","family":"Squires","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition,Department of Pediatrics,University of Pittsburgh,School of Medicine and Children\u2019s Hospital of Pittsburgh of University of Pittsburgh Medical Center,Pittsburgh,Pennsylvania,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Miethke","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology, and Nutrition,Department of Pediatrics,University of Cincinnati,Cincinnati,Ohio,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicky L.","family":"Ng","sequence":"additional","affiliation":[{"name":"Division of GI, Hepatology and Nutrition,Hospital for Sick Children and University of Toronto,Toronto,Ontario,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Horslen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics,Seattle Children\u2019s Hospital,University of Washington School of Medicine,Seattle,Washington State,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Kyle Jensen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics,University of Utah,Salt Lake City,Utah,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald J.","family":"Sokol","sequence":"additional","affiliation":[{"name":"Department of Pediatrics\u2010Gastroenterology, Hepatology and Nutrition,University of Colorado School of Medicine and Children\u2019s Hospital Colorado,Aurora,Colorado,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C.","family":"Magee","sequence":"additional","affiliation":[{"name":"Department of Surgery,University of Michigan School of Medicine,Ann Arbor,Michigan,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin L.","family":"Shneider","sequence":"additional","affiliation":[{"name":"Section of Pediatric Gastroenterology, Hepatology and Nutrition,Texas Children\u2019s Hospital and Baylor College of Medicine,Houston,Texas,USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"ChiLDReN","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2022,4,18]]},"reference":[{"key":"hep32451-bib-0001-20250824","doi-asserted-by":"crossref","first-page":"1750","DOI":"10.1001\/jama.2014.2623","article-title":"Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical 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