Orthotopic liver transplantation for acute liver failure secondary to autoimmune hepatitis in a child with autoimmune polyglandular syndrome type 1.
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.
Smith
Dominic
D
Children's Liver Unit, St. James's University Hospital, Leeds, UK.
Stringer
Mark D
MD
Wyatt
Judy
J
O'Meara
Moira
M
Davison
Suzanne
S
Cheetham
Tim D
TD
McClean
Patricia
P
D000293:Adolescent; D005500:Follow-Up Studies; D006085:Graft Survival; D019693:Hepatitis, Autoimmune / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D017114:Liver Failure, Acute / Q000209:etiology* / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D016884:Polyendocrinopathies, Autoimmune / Q000150:complications* / Q000175:diagnosis; D018570:Risk Assessment; D016896:Treatment Outcome