{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T12:31:34Z","timestamp":1782909094424,"version":"3.54.5"},"reference-count":38,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2022,10,31]],"date-time":"2022-10-31T00:00:00Z","timestamp":1667174400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005762","name":"National Taiwan University Hospital","doi-asserted-by":"publisher","award":["108\u2010S4392"],"award-info":[{"award-number":["108\u2010S4392"]}],"id":[{"id":"10.13039\/501100005762","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005762","name":"National Taiwan University Hospital","doi-asserted-by":"publisher","award":["109\u2010S4686"],"award-info":[{"award-number":["109\u2010S4686"]}],"id":[{"id":"10.13039\/501100005762","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["JGH Open"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and Aim<\/jats:title><jats:p>Alagille syndrome (ALGS) is a multisystem disorder with variable clinical courses. This study investigated the clinical and genetic features of ALGS patients with different outcomes and analyzed the liver pathology at liver transplantation (LT) compared with that in biliary atresia (BA).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We report the clinical characteristics, outcomes, and genetic mutations of 25 children with ALGS followed for a median of 7.3\u2009years. Patients were classified into (i) jaundice\u2010free (JF) group (resolving jaundice after 2\u2009years of age); (ii) progressive disease (PD) group (persistent jaundice or progressive cholestasis). In addition, we analyzed the explant liver in 10 ALGS patients compared with 20 age\u2010matched BA patients at the time of LT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Nine patients (36%) in the JF group had a favorable outcome, with longer native liver survival than patients with PD (<jats:italic>n<\/jats:italic>\u00a0=\u00a016, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.001). Fourteen of the PD group patients received LT or died. We identified 18 different <jats:italic>JAG1<\/jats:italic> mutations in 22 patients. Three unrelated probands in the JF group had the same de novo mutation in <jats:italic>JAG1<\/jats:italic>, c.2122\u20102125delCAGT. Compared with BA children, ALGS patients had lower METAVIR scores in liver pathology, higher serum albumin levels, and lower weight\u2010for\u2010age <jats:italic>z<\/jats:italic>\u2010scores when receiving LT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>One\u2010third of ALGS patients had JF and a favorable course. Children with ALGS presenting with persistent jaundice beyond 2\u2009years of age should be cautioned for poor prognosis. ALGS patients tend to have a lesser extent of cirrhosis, and more growth problems than BA patients at the time of LT.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jgh3.12830","type":"journal-article","created":{"date-parts":[[2022,11,1]],"date-time":"2022-11-01T06:30:26Z","timestamp":1667284226000},"page":"839-845","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Different clinical and genetic features of Alagille patients with progressive disease <i>versus<\/i> a jaundice\u2010free course"],"prefix":"10.1002","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3065-6583","authenticated-orcid":false,"given":"Che\u2010Ming","family":"Chiang","sequence":"first","affiliation":[{"name":"Department of Pediatrics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Pediatrics, Tri\u2010Service General Hospital National Defense Medical Center  Taipei 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