Orthotopic liver transplantation for acute liver failure secondary to autoimmune hepatitis in a child with autoimmune polyglandular syndrome type 1
Dominic Smith
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorMark D. Stringer
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorJudy Wyatt
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorMoira O'Meara
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorSuzanne Davison
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorTim D. Cheetham
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorPatricia McClean
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorDominic Smith
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorMark D. Stringer
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorJudy Wyatt
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorMoira O'Meara
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorSuzanne Davison
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorTim D. Cheetham
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorPatricia McClean
1 Children's Liver Unit and Departments of 2 Histopathology and 3 Anaesthesia, St. James's University Hospital, Leeds, UK, 4 Department of Child Health, University of Newcastle upon Tyne, Newcastle, UK
Search for more papers by this authorAbstract
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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