[Wilson disease presenting as fulminant hepatic failure, acute hemolytic anemia and renal failure: report of one case].
Wilson disease presenting as fulminant hepatic failure, severe hemolysis and renal failure is rare in the literature. A ten-year-old boy--complaining of abdominal pain, jaundice, tea-colored urine, and anemia was admitted to this hospital; examination showed Kayser-Fleischer rings, anemia associated with hemolysis, mildly elevated serum transaminases, extremely elevated bilirubin levels, low serum ceruloplasmin level, slightly elevated serum copper, excessive 24-hour urine copper excretion, and severe renal function insufficiencies. Under the impression of Wilson disease with fulminant hepatic failure, the patient was treated by oral D-penicillamine 1 gm per day, intravenous zinc sulphate (about 8 mg per day elemental zinc), and given other supportive treatment. Unfortunately, the patient died of hepatic failure complicated with septic shock 21 days after the onset of symptoms. Autopsy found liver copper content was 586.92 ug/gm dry weight and kidney copper content: 300.19 ug/gm dry weight, abnormally high as compared with normal tissue. A review of the literature led to conclusion that the best treatment for Wilson fulminant hepatic failure is liver transplantation.
Tseng
C L
CL
Department of Pediatrics, National Taiwan University Hospital, Taipei, R.O.C.
Tsai
S L
SL
Lin
K H
KH
Chang
M H
MH
Wang
T R
TR
Hsu
Y H
YH
Hsu
H C
HC
D000208:Acute Disease; D000743:Anemia, Hemolytic; D002648:Child; D006501:Hepatic Encephalopathy; D006527:Hepatolenticular Degeneration / Q000175:diagnosis*; D006801:Humans; D007674:Kidney Diseases; D008297:Male