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Orthotopic liver transplantation or acute liver failure secondary to autoimmune hepatitis in a child with autoimmune polyglandular syndrome type 1

Lookup NU author(s): Dianne Smith, Professor Timothy CheethamORCiD

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Abstract

Autoimmune polyglandular syndronic type 1 (APS-1) is an autosomal-recessive condition characterized by hypoparathyroidism, autoimmune Addison's disease, and chronic mucocutaneous candidiasis. autoimmune hepatitis develops in 10-20%, of affected patients and has a variable course ranging from asymptomatic chronic liver disease to lethal fulminant hepatic failure. Liver transplantation has been documented previously in only two patients. We report a 14-yr-old boy with APS-1 who developed acute liver failure secondary to associated autoimmune hepatitis. He did not respond to corticosteroid therapy and was successfully treated with an orthotopic liver transplant.

Publication metadata

Author(s): Cheetham TD; Smith D; Stringer MD; Wyatt J; O'Meara M; Davison S; McClean P

Publication type: Article

Publication status: Published

Journal: Pediatric Transplantation

Year: 2002

Volume: 6

Issue: 2

Pages: 166-170

ISSN (print): 1397-3142

ISSN (electronic): 1399-3046

Publisher: Wiley-Blackwell Publishing, Inc.

URL: http://dx.doi.org/10.1034/j.1399-3046.2002.01063.x

DOI: 10.1034/j.1399-3046.2002.01063.x

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