Anti-liver-kidney microsome antibody-positive autoimmune hepatitis presenting as fulminant liver failure.

A 17-month-old girl presented with acute hepatitis, which took a fulminant course leading to death 2 months after onset. No known cause of fulminant liver failure could be identified. Postmortem examination of the liver showed massive multilobular necrosis and areas of severe piecemeal necrosis. A high level of total serum gamma-globulins raised the possibility of autoimmune hepatitis. Search for anti-liver-kidney microsome antibody in the patient's serum was positive by immunofluorescence and enzyme-linked immunosorbent assay. Western blot analysis showed reactivity of the antibody with a 50-kDa protein identical to that observed in children with autoimmune hepatitis. This patient's history strongly suggests that autoimmune hepatitis can present as fulminant liver failure in children. Early diagnosis in such a patient could lead to early immunosuppressive therapy.