title: Fulminant hepatic failure as the first manifestation of Wilson disease: a report of two cases.
abstract: Two cases of fulminant hepatic failure as a first manifestation of Wilson disease are reported. They were diagnosed as acute hemolytic anemia initially, and took a fatal course despite intensive medical therapy. The main object of this report is to emphasize that Wilson disease must be included in the differential diagnosis of fulminant hepatic failure in children. Liver transplantation is the only effective means of treatment in patients presenting with fulminant hepatic failure, but this procedure is difficult to perform because of insufficient organ donation in Taiwan. The best treatment for Wilson disease is prevention through diagnosis at a presymptomatic stage, and institution of life-long therapy with D. penicillamine.
authors.affiliation: Department of Pediatrics, Children's Medical Center, Taipei, Taiwan, R.O.C.
authors.forename: C C
authors.initials: CC
authors.lastname: Chung
authors.forename: T C
authors.initials: TC
authors.lastname: Wu
authors.forename: C H
authors.initials: CH
authors.lastname: Sun
authors.forename: W W
authors.initials: WW
authors.lastname: Chung
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D006501:Hepatic Encephalopathy / Q000209:etiology*; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans