McCullough A J, Fleming C R, Thistle J L, Baldus W P, Ludwig J, McCall J T, Dickson E R
Gastroenterology. 1983 Jan;84(1):161-7.
The clinical course, results of standard laboratory tests, parameters of copper metabolism, and hepatic morphology in 9 cases (3 of our own and 6 from the literature) of Wilson's disease presenting as fulminant hepatic failure were compared with the findings in 5 cases of idiopathic fulminant hepatic failure. Patients with Wilson's disease were usually younger, and 7 of the 9 patients had Kayser-Fleischer rings. Patients with idiopathic fulminant hepatic failure had elevated 24-h urinary copper, decreased ceruloplasmin, and low or normal serum copper. Fulminant hepatic failure with Wilson's disease differed from idiopathic fulminant hepatic failure by the following biochemical findings: (a) higher copper levels in serum, urine and liver; (b) less pronounced elevations of transaminase levels; (c) higher concentrations of total bilirubin; and (d) lower hemoglobin values. Serum copper was the most useful biochemical test in diagnosing Wilson's disease before death. At autopsy, only hepatic copper concentrations clearly separated the two groups. Serial serum copper levels (antemortem) and quantitative analysis of hepatic copper (after recovery or postmortem) in patients with fulminant hepatic failure should help to exclude Wilson's disease.
将9例（其中3例为我们自己的病例，6例来自文献）表现为暴发性肝衰竭的威尔逊病患者的临床病程、标准实验室检查结果、铜代谢参数和肝脏形态，与5例特发性暴发性肝衰竭患者的检查结果进行了比较。威尔逊病患者通常较年轻，9例患者中有7例有凯-弗环。特发性暴发性肝衰竭患者24小时尿铜升高、铜蓝蛋白降低，血清铜低或正常。威尔逊病所致暴发性肝衰竭与特发性暴发性肝衰竭在以下生化检查结果方面存在差异：（a）血清、尿液和肝脏中的铜水平较高；（b）转氨酶水平升高不那么明显；（c）总胆红素浓度较高；（d）血红蛋白值较低。血清铜是生前诊断威尔逊病最有用的生化检查。尸检时，只有肝脏铜浓度能清楚地区分这两组。暴发性肝衰竭患者的系列血清铜水平（生前）和肝脏铜的定量分析（恢复后或死后）应有助于排除威尔逊病。
