{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T16:14:45Z","timestamp":1784909685512,"version":"3.55.0"},"reference-count":36,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"12","license":[{"start":{"date-parts":[[2020,10,3]],"date-time":"2020-10-03T00:00:00Z","timestamp":1601683200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hepatol. Commun."],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:p>Approximately 50% of infants with biliary atresia (BA) undergoing Kasai portoenterostomy show survival with native liver (SNL) at age 2\u00a0years. Predictors of disease progression after age 2\u00a0years are unknown, despite estimates of 20%\u201030% undergoing liver transplant (LT) between age 2 and 18\u00a0years. We sought to address this knowledge gap by developing prognostic models in participants of the multicenter prospective National Institutes of Health\u2010supported Childhood Liver Disease Research Network. We extracted 14 clinical and biochemical variables at age 2\u00a0years to develop two models for future outcomes: 1) LT or death (LTD) and 2) first sentinel event (SE), either new onset ascites, hepatopulmonary syndrome (HPS), or gastrointestinal (GI) bleed. A total of 240 participants, enrolled between 2004 and 2017, were followed until a median age of 5.1\u00a0years (range, 2.0\u201013.3\u00a0years). Of these participants, 38 underwent LT (n\u00a0=\u00a037) or death (n\u00a0=\u00a01); cumulative incidence, 23.7% (95% confidence interval [CI], 16.2%\u201032.0%). Twenty\u2010seven experienced either new\u2010onset ascites (n\u00a0=\u00a013), HPS (n\u00a0=\u00a01), or GI bleed (n\u00a0=\u00a014). One participant had ascites and GI bleed concurrently; cumulative incidence, 21.5% (95% CI, 14.2%\u201029.8%) by age 10\u00a0years. The Cox proportional hazard model predicted risk of LTD, using total bilirubin, albumin, platelet count, and history of either ascites or cholangitis (BA LTD model), with a C\u2010index of 0.88 (range, 0.86\u20100.89). A cause\u2010specific hazard competing risk model predicted SE using platelet count and gamma glutamyltransferase levels (BA SE model) with a C\u2010index of 0.81 (range, 0.80\u20100.84). Internal model validity was assessed using Harrell\u2019s C\u2010index with cross\u2010validation. <jats:italic toggle=\"yes\">Conclusion:<\/jats:italic> Stratification using these models identified risk of poor outcomes in patients with BA SNL after age 2\u00a0years. The models may identify those who would benefit from enhanced clinical surveillance and prioritization in clinical trials.<\/jats:p>","DOI":"10.1002\/hep4.1602","type":"journal-article","created":{"date-parts":[[2020,10,3]],"date-time":"2020-10-03T09:45:31Z","timestamp":1601718331000},"page":"1824-1834","source":"Crossref","is-referenced-by-count":26,"title":["Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age"],"prefix":"10.1097","volume":"4","author":[{"given":"Veena","family":"Venkat","sequence":"first","affiliation":[{"name":"UPMC Children's Hospital of Pittsburgh,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicky L.","family":"Ng","sequence":"additional","affiliation":[{"name":"Hospital for Sick Children,University of Toronto,Toronto,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John C.","family":"Magee","sequence":"additional","affiliation":[{"name":"University of Michigan Hospitals and Health Centers,Ann Arbor,MI"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen","family":"Ye","sequence":"additional","affiliation":[{"name":"University of Michigan Hospitals and Health Centers,Ann Arbor,MI"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kieran","family":"Hawthorne","sequence":"additional","affiliation":[{"name":"Arbor Research Collaborative for Health,Ann Arbor,MI"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjiv","family":"Harpavat","sequence":"additional","affiliation":[{"name":"Texas Children's Hospital,Liver Center, Baylor College of Medicine,Houston,TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean P.","family":"Molleston","sequence":"additional","affiliation":[{"name":"Indiana University\u2010Riley Hospital for Children,Indianapolis,IN"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen F.","family":"Murray","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Children\u2019s,Cleveland,OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kasper S.","family":"Wang","sequence":"additional","affiliation":[{"name":"Children's Hospital Los Angeles,Los Angeles,CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nisreen","family":"Soufi","sequence":"additional","affiliation":[{"name":"Children's Hospital Los Angeles,Los Angeles,CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lee M.","family":"Bass","sequence":"additional","affiliation":[{"name":"Ann & Robert H. Lurie Children's Hospital of Chicago,Chicago,IL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estella M.","family":"Alonso","sequence":"additional","affiliation":[{"name":"Ann & Robert H. Lurie Children's Hospital of Chicago,Chicago,IL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge A.","family":"Bezerra","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center,Cincinnati,OH"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Kyle","family":"Jensen","sequence":"additional","affiliation":[{"name":"University of Utah School of Medicine,Primary Children\u2019s Hospital,Salt Lake City,UT"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Binita M.","family":"Kamath","sequence":"additional","affiliation":[{"name":"Hospital for Sick Children,University of Toronto,Toronto,Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen M.","family":"Loomes","sequence":"additional","affiliation":[{"name":"The Children's Hospital of Philadelphia,Philadelphia,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cara L.","family":"Mack","sequence":"additional","affiliation":[{"name":"University of Colorado School of Medicine,Children\u2019s Hospital Colorado,Aurora,CO"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Rosenthal","sequence":"additional","affiliation":[{"name":"University of California San Francisco,San Francisco,CA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin L.","family":"Shneider","sequence":"additional","affiliation":[{"name":"Texas Children's Hospital,Liver Center, Baylor College of Medicine,Houston,TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert H.","family":"Squires","sequence":"additional","affiliation":[{"name":"UPMC Children's Hospital of Pittsburgh,Pittsburgh,PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald J.","family":"Sokol","sequence":"additional","affiliation":[{"name":"University of Colorado School of Medicine,Children\u2019s Hospital Colorado,Aurora,CO"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3379-7592","authenticated-orcid":false,"given":"Saul J.","family":"Karpen","sequence":"additional","affiliation":[{"name":"Emory University School of Medicine,Atlanta,GA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the 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