威爾森病以猛暴性肝衰竭、急性溶血性貧血及腎衰竭表現：一病例報告

威爾森病(Wilson disease)以猛暴性肝衰竭急性溶血性貧血及腎衰竭表現於文獻上的報告並不多見。本文提出一位10歲男孩病例，因腹痛、黃疸、茶色尿及貧血住院。檢查結果發現：血紅素降至6.5gm/dl、總膽紅素及直接膽紅素分別升高至63.8及26.8mg/dl、肝功能AST 241U/L，及ALT 12U/L，血中銅含量輕微升高，24小時尿中銅含量增加，血中藍胞漿素(ceruloplasmin)含量較低，Coombs'test陰性反應，兩眼角膜有凱塞佛萊氏環(Kayser-Fleischer ring)，肌胺酸(creatinine)及血尿素氮(BUN)升高，病人意識昏迷，以D-penicillamine、zinc sulphate及其他支持性療法治療，意識一度清醒，後不幸發生敗血性休克死亡。病理解剖發現：肝臓切片有肝細胞壞死、膽道增殖現象，肝內膽汁滯留及微小肝硬化結節形成，銅染色Rodanine stain呈陽性反應，含銅定量升高至586.92ug/gm dry weight，至此確定本例為威爾森病。

關鍵字

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並列摘要

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.

並列關鍵字

fulminant hepatic failure ； acute hemolytic anemia ； actue renal failure ； Wilson disease

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威爾森病以猛暴性肝衰竭、急性溶血性貧血及腎衰竭表現：一病例報告

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