Thirty-eight children with chronic active hepatitis are reported. Their mode of presentation was usually indistinguishable from acute viral hepatitis (63%). Extra hepatic manifestations were more common in those patients with an insidious onset. The twenty-eight patients tested for hepatitis B antigen (HB Ag) were all negative and SGOT values were higher (mean 1000 i.u./l) than those reported in adult patients with chronic active hepatitis. Thirty-three patients had marked elevation of serum gammaglobulin (mean 3·78 g/100 ml) and 34% had positive LE cells. All patients had histologic features of ‘aggressive’ hepatitis; in addition five biopsies also showed areas of submassive hepatic necrosis. The initial response to corticosteroid was excellent in all but three patients. To date drug therapy has been suspended in fifteen patients for periods of 3 months to 4 years and 7 months. There have been eight deaths. Three with hepatic insufficiency and two with sepsis. Three others have subsequently died following orthotopic liver transplantation.
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