Chronic active hepatitis in children

Twenty-eight children with chronic active hepatitis are reported. The majority were diagnosed within the last 5 years. Their mode of presentation was usually indistinguishable from acute viral hepatitis (63%). Extrahepatic manifestations were more common in those patients with an insidious onset. All presented sporadic cases without known exposure or secondary spread. The 19 patients tested for hepatitis-associated antigen (HAA) were all negative and SGOT values were higher (mean, 1080 IU/liter) than those reported in adult patients with chronic active hepatitis. Twenty-seven patients had marked elevation of serum gammaglobulin (mean, 4.04 g/100 ml) and 42% had positive LE cells. All patients had histologic features of aggressive hepatitis; in addition, five biopsies also showed areas of submassive lobular necrosis. The initial response to corticosteroid therapy was excellent in all but 3 patients. To date, drug therapy has been suspended in 4 patients for periods of 4 months to 2 years without clinical or biochemical relapse. There have been 4 deaths while on therapy, 2 with hepatic insufficiency and 2 with sepsis. One patient with end-stage liver disease underwent orthotopic liver transplantation and subsequently died.Supported in part by a grant (RR-69) from the General Clinical Research Centers Program of the Division of Research Resources. National Institutes of Health.