{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,2]],"date-time":"2025-08-02T16:40:58Z","timestamp":1754152858443,"version":"3.41.2"},"reference-count":24,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2017,3,15]],"date-time":"2017-03-15T00:00:00Z","timestamp":1489536000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2017,10]]},"abstract":"Abstract<\/jats:title>Background & Aims<\/jats:title>Adult studies of autoimmune hepatitis (AIH<\/jats:styled-content>) have shown that the model of end\u2010stage liver disease is associated with resistance to first\u2010line treatment. Using a multicentre retrospective database, we sought to determine if the paediatric end\u2010stage liver disease (PELD<\/jats:styled-content>) score would similarly predict treatment resistance in paediatric AIH<\/jats:styled-content>.<\/jats:p><\/jats:sec>Methods<\/jats:title>One hundred and seventy\u2010one children from 13 Canadian centres who fulfilled the International Autoimmune Hepatitis Group (IAIHG<\/jats:styled-content>) criteria were included and assessed for change to second\u2010line therapy within 24\u00a0months of primary treatment onset. Those with PSC<\/jats:styled-content> overlap at presentation, or missing data on the PELD<\/jats:styled-content> variables were excluded. PELD<\/jats:styled-content> was calculated for all remaining patients. Univariate analysis and receiver\u2010operator characteristic (ROC<\/jats:styled-content>) curves were performed to determine the predictive ability of the PELD<\/jats:styled-content> score to change to second\u2010line therapy.<\/jats:p><\/jats:sec>Results<\/jats:title>A total of 103 children were included with median age of 11\u00a0years (range 2\u201017). Mean PELD<\/jats:styled-content> was \u22122.51\u00b18.58. Second\u2010line therapy was used within 24\u00a0months of diagnosis in 13 patients. Univariate analysis revealed that change to second\u2010line therapy was associated with higher PELD<\/jats:styled-content> (P<\/jats:italic>=.028) and internal normalized ratio (INR<\/jats:styled-content>) (P<\/jats:italic>=.011). ROC<\/jats:styled-content> curves for PELD<\/jats:styled-content> and its individual components were performed. The strength of association was strongest with INR<\/jats:styled-content> (AUC<\/jats:styled-content> 0.72; CI<\/jats:styled-content>: 0.58\u20100.86) although the composite PELD<\/jats:styled-content> score also showed some predictive ability (AUC<\/jats:styled-content> 0.67; CI<\/jats:styled-content>: 0.52\u20100.81).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>In this paediatric AIH<\/jats:styled-content> cohort, higher PELD<\/jats:styled-content> at presentation predicted change to second\u2010line therapy within the first 2\u00a0years of follow\u2010up. INR<\/jats:styled-content> appeared to be the main contributor to that association.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.13387","type":"journal-article","created":{"date-parts":[[2017,2,15]],"date-time":"2017-02-15T16:36:53Z","timestamp":1487176613000},"page":"1562-1570","source":"Crossref","is-referenced-by-count":1,"title":["A national retrospective study of paediatric end\u2010stage liver disease as a predictor of change to second\u2010line therapy in children with autoimmune hepatitis"],"prefix":"10.1111","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6350-2057","authenticated-orcid":false,"given":"Andr\u00e9anne N.","family":"Zizzo","sequence":"first","affiliation":[{"name":"Division of Gastroenterology Department of Paediatrics London Children's Hospital Western University London ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolina","family":"Jimenez\u2010Rivera","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Department of Paediatrics Children's Hospital of Eastern Ontario University of Ottawa Ottawa ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Medicine University Health Network, University of\u00a0Toronto \u00a0Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard A.","family":"Schreiber","sequence":"additional","affiliation":[{"name":"Division of Paediatric Gastroenterology, Hepatology and Nutrition Department of Paediatrics University of British Columbia Vancouver BC Canada"},{"name":"Child and Family Research Institute Vancouver BC Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simon C.","family":"Ling","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition The Hospital for Sick Children Toronto ON Canada"},{"name":"Department of Paediatrics University of Toronto Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jason","family":"Yap","sequence":"additional","affiliation":[{"name":"Division of Paediatric Gastroenterology and Nutrition Department of Paediatrics University of Alberta Edmonton AB Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeff","family":"Critch","sequence":"additional","affiliation":[{"name":"Department of Paediatrics Faculty of Medicine Memorial University St. John's NL Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Najma","family":"Ahmed","sequence":"additional","affiliation":[{"name":"Division of Paediatric Gastroenterology and Nutrition Montreal Children's Hospital McGill University Montreal QC Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fernando","family":"Alvarez","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Department of Paediatrics H\u00f4pital Sainte\u2010Justine Universit\u00e9 de Montr\u00e9al Montreal QC Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Binita M.","family":"Kamath","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition The Hospital for Sick Children Toronto ON Canada"},{"name":"Department of Paediatrics University of Toronto Toronto ON Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"The Canadian Paediatric Hepatology Research Group (CPHRG)","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,3,15]]},"reference":[{"key":"e_1_2_9_2_1","first-page":"727A","article-title":"Incidence and characteristics of autoimmune hepatitis in children and adolescents in Canada: a preliminary report of The Canadian Pediatric Hepatology Research 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