Commentary

[摘要] Strictly speaking, fulminant hepatic failure associated with Wilson disease (WD-FHF) is not “acute liver failure,” because there is an underlying (although unrecognized) chronic liver disease. When WD-FHF was first reported in the late 1970s, it seemed that it might be extremely rare. In fact, it accounts for approximately 5% of patients with acute liver failure. Besides severe coagulopathy and encephalopathy, WD-FHF patients have a typical clinical profile: Coombs-negative hemolytic anemia with features of acute intravascular hemolysis, only modest increases in serum aminotransferases, normal or markedly subnormal serum alkaline phosphatase, and rapid progression to renal failure. Females are more likely than …

[学科分类] 过敏症与临床免疫学