title: Fulminant hepatic failure in autoimmune polyendocrine syndrome type-1.
abstract: Fulminant hepatic failure is liver disease that causes encephalopathy within 8 weeks of onset of symptoms or within 2 weeks of onset of jaundice in a patient without prior evidence of liver disease. Autoimmune polyendocrine syndrome type-1 is an autoimmune autosomal-recessive condition causing parathyroid and adrenal insufficiency, alopecia, chronic mucocutaneous candidiasis, ectodermal dystrophy and, rarely, hepatitis. Although the liver can be affected as a consequence of the autoimmune process, the spectrum of disease activity is varied. Autoimmune hepatitis develops in 10-20% of patients and successful liver transplantation has been reported in pediatric patients who failed immunosuppressive treatment. We report fulminant hepatic failure in an adult patient with autoimmune polyendocrine syndrome type-1 who responded to medical treatment and did not require liver transplantation. We highlight the diagnostic scoring system for autoimmune hepatitis and the referral criteria for liver transplantation in fulminant hepatic failure.
authors.affiliation: R Sinha, Scottish Liver Transplant Unit, Royal Infirmary of Edinburgh, Little France, Edinburgh EH16 4SA, UK. Email rohits78@gmail.com.
authors.forename: R
authors.initials: R
authors.lastname: Sinha
authors.forename: A R
authors.initials: AR
authors.lastname: Chapman
authors.forename: G T
authors.initials: GT
authors.lastname: Reid
authors.forename: P C
authors.initials: PC
authors.lastname: Hayes
mesh_terms: D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000473:pathology*; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D016031:Liver Transplantation; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications* / Q000188:drug therapy; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents
keywords: autoimmune hepatitis; autoimmune polyendocrine syndrome type-1; fulminant hepatic failure; liver transplantation