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

Abstract

Autoimmune polyglandular syndrome 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.

• Authors: Dominic Smith, Mark D Stringer, Judy Wyatt, Moira O'Meara, Suzanne Davison, Tim D Cheetham, Patricia McClean
• Journal: Pediatric transplantation (Pediatr Transplant) Vol. 6 Issue 2 Pg. 166-70 (Apr 2002) ISSN: 1397-3142 [Print] Denmark
• PMID: 12000475 (Publication Type: Case Reports, Journal Article)

Topics

• Adolescent
• Follow-Up Studies
• Graft Survival
• Hepatitis, Autoimmune (complications, diagnosis)
• Humans
• Liver Failure, Acute (etiology, surgery)
• Liver Transplantation (methods)
• Male
• Polyendocrinopathies, Autoimmune (complications, diagnosis)
• Risk Assessment
• Treatment Outcome