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肝豆状核变性(威尔逊病)中角膜铜的无创定量分析

Non-invasive quantitation of corneal copper in hepatolenticular degeneration (Wilson's disease).

作者信息

Belkin M, Zeimer R, Chajek T, Friedman G, Melamed E

出版信息

Lancet. 1976 Feb 21;1(7956):391-2. doi: 10.1016/s0140-6736(76)90217-8.

Abstract

The corneal copper content was measured by X-ray excitation spectrometry in two controls and in seven patients with Wilson's disease. Patients who were treated irregularly or not treated at all showed a high corneal copper content. Those who were adequately treated had low levels, comparable to the controls. In one case the corneal copper content declined 45%, after a course of dimercaprol. The corneal copper measured showed no correlation with the slit-lamp appearance of the Kayser-Fleischer ring. It is suggested that non-invasive X-ray excitation spectrometry can provide a fast and reliable method for the early diagnosis of Wilson's disease and for the objective evaluation of the efficacy of the treatment of this disease.

摘要

通过X射线激发光谱法测量了2名对照者和7名威尔逊氏病患者的角膜铜含量。治疗不规律或未接受治疗的患者角膜铜含量较高。接受充分治疗的患者角膜铜含量较低,与对照者相当。有1例患者在使用二巯丙醇治疗一个疗程后,角膜铜含量下降了45%。所测得的角膜铜含量与凯-弗环的裂隙灯表现无相关性。研究表明,非侵入性X射线激发光谱法可为威尔逊氏病的早期诊断和该病治疗效果的客观评估提供一种快速且可靠的方法。

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