Fulminant hepatic failure: pediatric aspects.

ABSTRACT

In children, fulminant hepatic failure is a rare multisystem disorder in which severe impairment of liver function, with or without encephalopathy, occurs in association with hepatocellular necrosis in a patient with no recognized underlying chronic liver disease. Recognized etiologies include infections, toxins, metabolic disorders, infiltrative diseases, autoimmune hepatitis, ischemic or irradiation damage; a proportion of cases are cryptogenic. The diagnosis of the cause is essential to institute lifesaving medical treatment, decide if transplantation is indicated, and offer genetic counseling. The maximum International Normalized Ratio *(INR) reached during the course of the illness is the most sensitive predictor of outcome, mortality being 86% with an INR > or = 4, and 27% with an INR < 4 in our own series. Prognosis is worse in children younger than 2 years. Thus, urgent transplantation should be considered when the INR reaches 4, particularly in very young children. Survival after transplantation is 60% to 68%. Children with fulminant hepatic failure must be treated in specialized centers with facilities for liver transplantation.