FULMINANT-HEPATITIS ASSOCIATED WITH CENTRILOBULAR HEPATIC-NECROSIS INYOUNG-CHILDREN

Objective: To describe fulminant hepatic failure (FHF) in children in the United States with clinical and histopathologic features distinctl y different from those typical of FMF. Patients: Seven young children were seen in early 1994 with encephalopathy, coagulopathy, and elevate d aminotransferase levels. Liver failure was preceded by a prodromal v iral illness that resulted in a period of fasting without dehydration, Unlike the majority of children with FHF, these patients had serum bi lirubin levels <171 mu mol/L(10 mg/dl). All children had received ther apeutic doses of acetaminophen during-the prodromal illness. Histopath ologic findings: Histologic findings included zonal necrosis of hepato cytes in a centrilobular distribution, which is characteristic of toxi c liver injury but is atypical for viral hepatitis and sporadic non-A non-B hepatitis. Outcome: Six patients recovered spontaneously, and on e died of complications of liver failure and fungal sepsis, The cause of this disorder remains unknown, but we postulate a viral or environm ental insult that preferentially damages zone 3 hepatocytes, The poten tial for this injury may have been augmented by ingestion of therapeut ic doses of acetaminophen while patients were in a fasted state, The p rognosis was good compared with typical FHF in children and correlated with the degree of liver necrosis on histologic examination.