Orthotopic liver transplantation for acute liver failure secondary to autoimmune hepatitis in a child with autoimmune polyglandular syndrome type 1 |
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Authors: | Smith Dominic Stringer Mark D Wyatt Judy O'Meara Moira Davison Suzanne Cheetham Tim D McClean Patricia |
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Institution: | Children's Liver Unit, St. James's University Hospital, Leeds, UK. |
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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. |
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Keywords: | autoimmune polyglandular syndrome autoimmune hepatitis liver transplantation |
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