Down syndrome and autoimmune chronic active hepatitis: satisfactory outcome with therapy.

A case of autoimmune chronic active hepatitis (C.A.H.) in a 12-year old Down Syndrome child is described. This represents the second only documented case of autoimmune C.A.H. occurring in Down Syndrome; the first well-described case died from the disease. The patient described below had a satisfactory response to steroid therapy with restoration of normal liver histological appearances and function despite severe disease at presentation. Chronic hepatitis in Down Syndrome, although more usually of hepatitis B viral aetiology, may also be autoimmune in nature and resolve satisfactorily with appropriate therapy.