Giant-cell hepatitis is a frequent pattern of liver injury in the neonate, but it is rare after infancy. Such cases have been attributed to autoimmune disease, to non-A, non-B hepatitis and, most recently, to paramgxovirus infection. To better define the entity of postinfantile (syncytial) giant-cell hepatitis, we reviewed 24 biopsy specimens from 20 patients with this finding, either alone or in combination with other diacplosee. The number of multinucleated giant cells varied greatly from one specimen to another. Varying degrees of portal inflnmmation appeared in all but one of the patients, and all had hepatitislike acinar i d a mmation associated with hepatocellular injury. Fibrosis was a common finding, varying from mild periportal fibrosis to established cirrhosis (33%). The changes were interpreted as acute giant-cell hepatitis in 26%, as CAH in 42% and as active cirrhosis in the remainder.
The patients ranged in age from 2 to 80 yr, with a mean of 36 yr and a maldfemale ratio of approxhately 1:l. The signs and symptoms of liver disease were present for more than 1 mo in most patients. A positive antinuclear antibody titer was found in seven of the patients. Three patients had a direct Coombs reaction and anemia Overall, evidence of autoimmune disease was found in 40% of the patients. One patient had non-Hodgkin's lymphoma involving the liver. Only one patient had a hietory of blood transfusion or risk factors for hepatitis C. No patient underwent serological study for paramysovirus antibodies. Liver tissue from one patient was examined ultrastructarally, but no viral particles could be identified. Follow-up information was available in 17 of the patients. Four had died (one of causes unrelated to liver disease); one of the surviving patients underwent successfd orthotopic liver transplantation. It would appear that postinfantile giant-cell hepatitis is best regarded as an unusual reaction pattern that can occur in both acute and chronic hepatitis. The most frequently identitied underlying cause in our series was autoimmune disease, which may have significant implications for treatment of many of these patients.