Fulminant hepatic failure in childhood: an analysis of 31 cases

Abstract

To document the clinical features and complications of fulminant hepatic failure in childhood, 31 consecutive cases (of whom only 9 survived) were reviewed. Of 26 children with acute hepatitis (HbSAg-negative), liver function steadily deteriorated in all but 2, and encephalopathy occurred within 3 weeks of the onset of symptoms in all except 3 of them. Eight of these patients survived, as did one of 3 in which this deterioration was caused by paracetamol overdosage. Single cases due to Amanita phalloides and halothane died. Encephalopathy lasted from 2 to 16 days in the survivors, and from one to 20 days in the fatal cases. The severity fluctuated by more than one grade in 9 patients. The outcome was not related to the age or sex of patient, clinical or biochemical abnormalities at presentation, or to the duration of the encephalopathy. Prothrombin time was prolonged by more than 90 seconds in 10 fatal cases, but in none of the survivors. The outcome was related to the severity of the encephalopathy, only one (6%) of 19 children in grade 4 coma surviving, and to the occurrence of neurological complications—particularly brain stem dysfunction (9 cases), decerebrate posturing (12 cases), and convulsions (7 cases). Massive gastrointestinal bleeding (14 cases) and renal failure (10 cases) were confined to the fatal group. At necropsy 7 (54%) of 13 had cerebral oedema.

Hypoglycaemia, septicaemia, respiratory tract infections, ascites, and haemopoietic complications occurred both in fatal cases and survivors. Although liver function tests and liver biopsy appearances remained abnormal in survivors for 24 and 30 months respectively, these children developed normally without evident disease during or after this period.

Children with fulminant hepatic failure and severe encephalopathy develop major pathophysiological complications affecting almost every system. Such complications must be prevented or vigorously treated. The mortality is no lower than in adults. Effective treatment must be instituted before grade 4 coma is established.