Importance of a Liver Biopsy in the Management of Wilson Disease
Importance of a Liver Biopsy in the Management of Wilson Disease
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DOI：
10.2169/internalmedicine.3440-19
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发表时间：
2020-01-01
影响因子：
1.2
通讯作者：
Harada, Masaru
中科院分区：
文献类型：
--
作者：
Oe, Shinji;Honma, Yuichi;Harada, Masaru
一位37岁威尔逊病患者接受d -青霉胺治疗来我院评估肝功能。实验室数据显示低血清铜水平和铜蓝蛋白。停用d -青霉胺4天后，尿铜与尿肌酐的比值在0.1以下。我们得出结论，铜螯合作用过度，将d -青霉胺转化为醋酸锌。然而，他的肝功能检查结果没有恢复正常。我们做了肝脏活检，发现铜含量很高。恢复螯合治疗后肝功能改善。通过活组织检查准确测量肝脏铜含量对该病的适当治疗很重要。
A 37-year-old Wilson disease patient treated with D-penicillamine visited our hospital for the evaluation of his liver function. Laboratory data showed a low serum copper level and ceruloplasmin. The ratio of urinary copper to urinary creatinine in a spot urinary analysis after 4 days' cessation of D-penicillamine was under 0.1. We concluded that the copper chelation was excessive and changed D-penicillamine to zinc acetate. However, his liver function test results did not normalize. We performed a liver biopsy and discovered a high copper content. The liver dysfunction was improved after resuming chelating therapy. Accurate measurement of the hepatic copper content via a biopsy is important for the adequate management of this disease.