{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T09:01:59Z","timestamp":1792314119419,"version":"4.4.0"},"reference-count":36,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2010,3,1]],"date-time":"2010-03-01T00:00:00Z","timestamp":1267401600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2010,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background:<\/jats:title><jats:p>Graft rejection and disease recurrence are well\u2010recognized complications of liver transplantation (LT) for autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (AISC). We describe indications and outcome of LT for childhood AIH and AISC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods:<\/jats:title><jats:p>Twenty\u2010year retrospective review of a cohort of children (n = 101) with AIH, AISC, or AIH\/sclerosing cholangitis overlap syndrome from a single center.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>AIH type 1 (AIH1, n = 67) was more common than AIH type 2 (AIH2, n = 18), AISC (n = 8), or overlap syndrome (n = 8). Overall, 18 patients (18%) required LT, the indications being failure of medical therapy (n = 16) and fulminant liver failure (n = 2). Patients with AIH who required LT had a more prolonged prothrombin time at presentation compared with those who did not undergo transplantation (<jats:italic>P<\/jats:italic> = 0.01). Patients with AIH1 who received LT had a lower aspartate transaminase (<jats:italic>P<\/jats:italic> = 0.009) and alanine transaminase (<jats:italic>P<\/jats:italic> = 0.02) levels at initial diagnosis compared with those with AIH1 who did not undergo transplantation. Post\u2010LT, 11 patients (61%) had 18 episodes of rejection, most were steroid sensitive. Disease recurrence was observed in 7 patients (39%, median duration post\u2010LT 33 months), more common in AIH2 (80% recurrence rate), and those taking cyclosporine (71%, 5\/7 patients) compared with those taking tacrolimus (18%, 2\/11 patients; <jats:italic>P<\/jats:italic> &lt; 0.05) and in 3 of 3 children who did not have maintenance steroids post\u2010LT. The overall 5\u2010 and 7\u2010year post\u2010LT survival rate was 94% and 88%, respectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>LT is a good therapeutic option for progressive AIH and AISC, although recurrence of the primary autoimmune process limits the outcome.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e3181bf0ef7","type":"journal-article","created":{"date-parts":[[2010,2,25]],"date-time":"2010-02-25T06:43:05Z","timestamp":1267080185000},"page":"295-302","source":"Crossref","is-referenced-by-count":59,"title":["Childhood Autoimmune Liver Disease: Indications and Outcome of Liver Transplantation"],"prefix":"10.1002","volume":"50","author":[{"given":"Pei Fan","family":"Chai","sequence":"first","affiliation":[{"name":"Department of Paediatrics University of Malaya Medical Centre Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Way Seah","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Malaya Medical Centre Kuala Lumpur Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel M","family":"Brown","sequence":"additional","affiliation":[{"name":"Department of Pathology Birmingham Children's Hospital Birmingham United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo L","family":"McPartland","sequence":"additional","affiliation":[{"name":"Department of Paediatric Histopathology Royal Liverpool Children's Hospital, Eaton Road Liverpool United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katharine","family":"Foster","sequence":"additional","affiliation":[{"name":"Department of Radiology United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick J","family":"McKiernan","sequence":"additional","affiliation":[{"name":"Liver Unit Birmingham Children's Hospital Birmingham United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deirdre A","family":"Kelly","sequence":"additional","affiliation":[{"name":"Liver Unit Birmingham Children's Hospital Birmingham United 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