Acute liver failure is a rare but potentially fatal disease. Adult definition of
fulminant hepatic failure, which includes the development of hepatic
necrosis and
encephalopathy within 8 weeks of onset of
liver disease does not apply to
acute liver failure in children particularly if secondary to autoimmune or metabolic
liver disease. The etiology of
acute liver failure varies with the age of the child. In neonates,
infection or an inborn error of metabolism are common, while viral
hepatitis and
drug induced
liver failure are more likely in older children. The clinical presentation of
acute liver failure includes
jaundice, coagulopathy and
encephalopathy. In neonates,
encephalopathy may be subclinical. The management of
acute liver failure includes assessment of prognosis for
liver transplantation; prevention and treatment of complications while awaiting hepatic regeneration or a donor liver and hepatic support. The major complications of
acute liver failure are
sepsis, gastro-intestinal
bleeding,
cerebral edema, renal and
cardiac failure. Selection for
liver transplantation depends on the etiology of the disease, prognostic factors, the presence or absence of multisystem disease and/or reversible brain damage. Prognostic factors for survival are less well established in children than in adults but children with metabolic
liver disease, prothrombin time > 50 seconds, rising
bilirubin and falling
transaminase, grade II or higher grade of
hepatic coma indicate poor prognosis. Most children receive a reduced or split liver graft. Living related donations for
acute liver failure are also carried out by some centres. Survival post
liver transplantation for
acute liver failure has improved and most recipients can expect a 70% five year survival.