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J Korean Pediatr Soc. 2002 Oct;45(10):1302-1307. Korean. Case Report.
Hong JY , Bae SH , Kim WS .
Department of Pediatrics, School of Medicine, Eulji University, Seoul, Korea.
Department of Pathology, School of Medicine, Eulji University, Seoul, Korea.

Autoimmune hepatitis is a chronic necroinflammatory liver disorder of unknown cause associated with circulating autoantibodies and hypergammaglobulinemia. We report two pediatric cases of type 1 autoimmune hepatitis with normal serum immunoglobulin G levels who presented with fulminant hepatitis. The cases were associated with hereditary spherocytosis or atrioventricular septal defect. The first case was a 13-year-old boy with hereditary spherocytosis and gallstones. Acute cholecystitis and cholangitis developed during his clinical course and cholecystectomy, splenectomy and liver wedge biopsy were performed. The second case was an 8-year-old girl with repaired atrioventricular septal defect. In both cases, antinuclear antibody tests were positive and immunoglobulin G levels were normal. Interface hepatitis with moderate lobular inflammation were noted on their liver histology. They responded to the treatment of prednisolone and ursodeoxycholic acid.

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