Trinh Thi Thuy, Nguyen Pham Anh Hoa, Hoang Thi Van Anh, Bui Huong Thuy, Do Van Do
Background: Wilson disease (WD) is an autosomal recessively inherited disorder of copper metabolism. Mutation of the ATP7B gene on chromosome 13 leads to accumulation of copper in the liver, brain, kidney and cornea. Clinical presentation is particularly in liver and central nervous system. Fuminal hepatic failure in WD is rarely and mortality rate is very high.
Methods: Retrospective description. Describe clinical, preclinical characteristics of patients acute liver failure due to WD.
Results: A total of 6 patients with acute liver failure due to WD (Leipzig 2001) at Hepatology Department in NHP from January 2014 to June 2015. Common symptoms: jaundice, edema, ascites, hepatic encephalopathy, hyperbilirubinemia, hemolytic anemia, hypoalbuminemia, severe coagulation disorder, ceruloplasmin <0.2 g/L, urinary copper/24 h >100 mcg/dL. Five patients improved after treatment, one patient died due to fulminant liver failure.
Conclusions: Fulminant hepatic failure which caused by WD is very rare in children. The disease can be rescued if it is diagnosed and treated promptly. WD should be suspected in fuminal hepatic failure unknown the origin.
