Acute liver failure (ALF) is a relatively rare but often fatal event in children. The frequency of ALF in all age groups in the United States is about 10 to 20,000 per year (about 17 cases per 100,000 population per year), but the frequency in the pediatric age group is unknown. ALF accounts for 10–15% of pediatric liver transplants performed in the United States annually. Even allowing for the probability that many children die without transplant, there may be as few as 50–100 cases per year in the United States. Little is known about pediatric ALF; the rarity of the condition has precluded accumulation of a large experience. Several studies are under way to amass the experience of major transplant centers in an effort to learn more about the condition. Most cases of ALF in children have no identifiable cause, which has prevented a focus on the mechanism by which hepatocytes are killed. The mechanism of ALF remains elusive even if the cause is known. Orthotopic liver transplantation is a substantial advance in the therapy of ALF. Other therapies being studied in clinical trials include liver assist devices and hepatocyte transplantation. Medical therapy generally consists of supportive measures, with a focus on preventing or treating complications and early referral to a transplant center. Outcomes for children vary depending upon the etiology and degree of central nervous system (CNS) involvement.
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