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Since the 1960s, <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content> has been investigated with extensive clinical research aimed at effective therapeutic intervention. It was one of the first liver diseases where treatment was demonstrated to prolong survival. <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content> occurs in children, as well as in adults. Its clinical manifestations in children may differ from classic adult <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content>. These differences have elucidated certain aspects of <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content> and hepatobiliary disease in general. There are two major patterns of <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content>: type 1, with anti\u2010smooth muscle antibodies and type 2, with anti\u2010liver\/kidney microsomal antibodies. The second type of <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content> was first identified in children and is more common in younger patients. <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content> often presents as acute disease in children and also in adults: the nomenclature has dropped the allusion to chronicity. Some children who have sclerosing cholangitis present with clinical disease closely resembling <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content>; this <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content>\u2010like <jats:styled-content style=\"fixed-case\">PSC<\/jats:styled-content>, termed autoimmune sclerosing cholangitis (<jats:styled-content style=\"fixed-case\">ASC<\/jats:styled-content>), is also found in adults. Children with <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content> may have identifiable monogenic disorders of immune regulation such as autoimmune polyendocrinopathy\u2010candidiasis\u2010ectodermal dystrophy (<jats:styled-content style=\"fixed-case\">APECED<\/jats:styled-content>). Like adults with <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content>, children with <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content> usually respond very favourably to immunosuppressive treatment with corticosteroids\u00a0\u00b1\u00a0azathioprine. True cures seem to be rare, although many children achieve a stable remission. Nonetheless children with <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content> may develop cirrhosis and some require liver transplantation. Early diagnosis and improved treatment strategies may further improve the outlook for children with <jats:styled-content style=\"fixed-case\">AIH<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/j.1478-3231.2011.02603.x","type":"journal-article","created":{"date-parts":[[2011,9,1]],"date-time":"2011-09-01T06:04:05Z","timestamp":1314857045000},"page":"1424-1431","source":"Crossref","is-referenced-by-count":28,"title":["Autoimmune hepatitis from the paediatric perspective"],"prefix":"10.1111","volume":"31","author":[{"given":"Eve A.","family":"Roberts","sequence":"first","affiliation":[{"name":"Division of Gastroenterology Hepatology and Nutrition The Hospital for Sick Children and Departments of Paediatrics Medicine and Pharmacology University of Toronto Toronto Ontario 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