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24小时尿铜在肝豆状核变性的临床意义 被引量:8

The clinical significance of the 24h urinary copper in Wilson disease
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摘要 目的研究24h尿铜对肝豆状核变性的临床意义和应用价值。方法选取WD患者85例,其中初诊34例,复诊51例;对照组20例。初诊患者进行神经症状评分、测定24h尿铜、血清铜、脑脊液铜。使用青霉胺治疗后,每个月进行神经症状评分、测定尿铜。分析尿铜意义。结果未经治疗患者尿铜高于正常对照,肝型患者尿铜高于脑型患者。尿铜与神经症状评分无相关性。使用青霉胺治疗后,WD患者尿铜增加。单个患者尿铜量与青霉胺剂量呈正相关。出现症状加重的患者的尿铜量低于未加重患者。尿铜量随治疗时程逐渐降低。结论24h尿铜对于WD的诊断、临床分型有一定提示作用,可以反映青霉胺疗效和治疗时程。但不能反映神经症状严重程度。尿铜排出困难与青霉胺治疗导致神经症状加重有一定联系。 Objective To investigate the value of the 24h urinary copper in Wilson disease (WD). Methods 85 WD patients including 34 patients without cure and 20 normal individuals were enrolled in the study. Their neural symptom was scored ,and their urinary copper ,serum copper, CSF copper were measured before treatment. The urinary copper and CSF copper were measured every month after treatment of D-penicillamine. Results Urinary copper of WD patients without cure especially the hepatic type was higher than the control. Urinary copper was not related to the neural symptom scores. After treatment with D-penicillamine, urinary copper increased. Urinary copper was related to the dose of D-penicillamine in single patient. Urinary copper of the patients whose neural symptom worsened was lower than the others. Urinary copper declined within the cure. Conclusion The 24h urinary copper is useful for the diagnosis and clinical type of WD. And it can reflect the effect of D-penicillamine,but can not reflect the level of neural symptom.
出处 《中风与神经疾病杂志》 CAS CSCD 北大核心 2007年第4期457-459,共3页 Journal of Apoplexy and Nervous Diseases
关键词 肝豆状核变性 尿铜 青霉胺 脑脊液铜 Wolson disease urinary copper D-penicillamine CSF copper
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