抽象的な

Fulminant hepatic failure in children

Roberto Gugig and Philip Rosenthal

Fulminant hepatic failure (FHF) in children is a rare but often fatal event. Our knowledge of this disorder is limited by the rarity of the disorder at any single center. Initiatives are underway to accumulate the experience of several large centers in a multicenter study of pediatric FHF in children funded by the NIH (the Pediatric Acute Liver Failure Study Group). Most FHF cases in children remain without a cause. The mechanisms whereby hepatocytes undergo cell death are unknown, as is an understanding of the events leading to FHF and its progression. Therapy has focused on supportive care in an attempt to ameliorate complications, and early referral to a liver transplant center remains crucial. Outcomes are dependent upon the etiology and the degree of CNS involvement. Clinical trials of liver assist devices, hepatocyte transplantation and use of N-acetylcysteine for nonacetaminophen-induced FHF may hold promise for the future.