Acute liver failure in children

Acute liver failure, defined as the onset of hepatic coagulopathy with an INR >= 1.5 (which is not corrected by vitamin K administration) with encephalopathy or with an INR >= 2.0 without encephalopathy, is a life-threatening disorder. Timely referral of the patient to a transplant center can, when all therapeutic strategies are employed, result in survival rates of approximately 70%. In neonates and infants infectious and metabolic causes as well as neonatal hemochromatosis are predominant. In small children, particularly school-age children, drug-induced or autoimmune causes as well as Wilson's disease play an increasing role. Almost 50% of cases remain etiologically uncertain. Therapy is based on etiology and is symptomatic in many cases. Since cerebral edema is the primary cause of death, restriction of fluid and sodium chloride is important; in addition, the head should be maintained in an elevated position. Despite unfavorable coagulation values, bleeding is an exception; fresh plasma should therefore be used with restraint.