首页> 美国卫生研究院文献>Journal of the National Medical Association >Two unusual cases with Wilsons disease: hepatoma and fulminant hepatitis treated with plasma exchange.
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Two unusual cases with Wilsons disease: hepatoma and fulminant hepatitis treated with plasma exchange.

机译:威尔逊氏病的两个不寻常病例:通过血浆置换治疗的肝癌和暴发性肝炎。

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摘要

We report two atypical cases of Wilson's disease. The first case is a 22-year-old male patient with a history of disease for 15 years and diagnosed as Wilson's disease upon investigations. Alpha-fetoprotein level was found elevated and computed tomography showed a 3.5-cm liver mass. Hepatocellular carcinoma was diagnosed. Radiofrequency ablation and liver transplantation were performed successfully. The second case is a 24-year-old female patient who presented with fulminant hepatitis. Urinary copper excretion and ceruloplasmin levels were suggestive of Wilson's disease. Despite chelation therapy, no improvement was observed. Plasma exchange therapy was performed for seven days. Her clinical status improved, and transplantation was no longer needed. To conclude, although hepatoma is rarely seen in Wilson's disease, patients should be examined regularly to diagnose it in a treatable stage. Removal of copper and toxic metabolites with plasma exchange therapy may be a way of treatment for fulminant hepatitis associated with Wilson's disease.
机译:我们报告了威尔逊氏病的两个非典型病例。第一例是22岁的男性患者,有15年的病史,经调查被诊断为威尔逊氏病。发现甲胎蛋白水平升高,计算机断层扫描显示肝脏肿块为3.5厘米。诊断为肝细胞癌。成功进行了射频消融和肝移植。第二例是一名患有暴发性肝炎的24岁女性患者。尿铜排泄和铜蓝蛋白水平暗示了威尔逊氏病。尽管进行了螯合疗法,但未观察到任何改善。进行了7天的血浆置换治疗。她的临床状况得到改善,不再需要移植。总之,尽管在威尔逊氏病中很少见到肝癌,但应定期检查患者以在可治疗的阶段进行诊断。血浆置换疗法去除铜和有毒代谢物可能是治疗与威尔逊氏病相关的暴发性肝炎的一种方法。

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