FULMINANT-HEPATITIS ASSOCIATED WITH CENTRILOBULAR HEPATIC-NECROSIS IN YOUNG-CHILDREN

Objective: To describe fulminant hepatic failure (FHF) in children in the United States with clinical and histopathologic features distinctly different from those typical of FMF. Patients: Seven young children were seen in early 1994 with encephalopathy, coagulopathy, and elevated aminotransferase levels. Liver failure was preceded by a prodromal viral illness that resulted in a period of fasting without dehydration, Unlike the majority of children with FHF, these patients had serum bilirubin levels <171 mu mol/L(10 mg/dl). All children had received therapeutic doses of acetaminophen during-the prodromal illness. Histopathologic findings: Histologic findings included zonal necrosis of hepatocytes in a centrilobular distribution, which is characteristic of toxic liver injury but is atypical for viral hepatitis and sporadic non-A non-B hepatitis. Outcome: Six patients recovered spontaneously, and one died of complications of liver failure and fungal sepsis, The cause of this disorder remains unknown, but we postulate a viral or environmental insult that preferentially damages zone 3 hepatocytes, The potential for this injury may have been augmented by ingestion of therapeutic doses of acetaminophen while patients were in a fasted state, The prognosis was good compared with typical FHF in children and correlated with the degree of liver necrosis on histologic examination.