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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.

DOI:10.1016/s0140-6736(76)90217-8
PMID:55651
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。

相似文献

1
Non-invasive quantitation of corneal copper in hepatolenticular degeneration (Wilson's disease).肝豆状核变性(威尔逊病)中角膜铜的无创定量分析
Lancet. 1976 Feb 21;1(7956):391-2. doi: 10.1016/s0140-6736(76)90217-8.
2
[Kayser-Fleischer ring in Wilson's disease].[威尔逊病中的凯泽尔-弗莱舍尔环]
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Wilson's disease. Electron microscopic, x-ray energy spectroscopic, and atomic absorption spectroscopic studies of corneal copper deposition and distribution.威尔逊氏病。角膜铜沉积与分布的电子显微镜、X射线能谱及原子吸收光谱研究。
Lab Invest. 1982 Jun;46(6):564-9.
4
Characteristics of neurological Wilson's disease without Kayser-Fleischer ring.无角膜 Kayser-Fleischer 环的神经型 Wilson 病的特征。
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Use of the stable isotope 65Cu test for the screening of Wilson's disease in a family with two affected members.使用稳定同位素65Cu检测对一个有两名患病成员的家庭进行威尔逊病筛查。
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Kayser-Fleischer-like rings in patients without Wilson's disease.无威尔逊病患者出现类似凯泽尔-弗莱舍尔环。
Gastroenterology. 1977 Jun;72(6):1331-5.
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The ophthalmologic manifestations of Wilson's disease.威尔逊氏病的眼科表现。
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[Diagnosis of Kayser Fleischer Ring: Can Early Diagnosis Improve the Outcome of Wilson's Disease?].[凯泽-弗莱舍尔环的诊断:早期诊断能否改善威尔逊病的预后?]
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In vivo confocal microstructural analysis of corneas presenting Kayser-Fleischer rings in patients with Wilson's disease.威尔逊病患者角膜呈现凯-弗环的体内共聚焦微观结构分析。
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引用本文的文献

1
Eye Involvement in Wilson's Disease: A Review of the Literature.威尔逊病的眼部受累:文献综述
J Clin Med. 2022 Apr 30;11(9):2528. doi: 10.3390/jcm11092528.
2
Rapid and reliable diagnosis of Wilson disease using X-ray fluorescence.利用 X 射线荧光快速可靠地诊断威尔逊病。
J Pathol Clin Res. 2016 Jun 6;2(3):175-86. doi: 10.1002/cjp2.48. eCollection 2016 Jul.
3
The Kayser-Fleischer ring during long-term treatment in Wilson's disease (hepatolenticular degeneration). A follow-up study.肝豆状核变性(威尔逊病)长期治疗期间的凯泽尔-弗莱施尔环。一项随访研究。
Graefes Arch Clin Exp Ophthalmol. 1986;224(2):152-5. doi: 10.1007/BF02141489.
4
Unilateral Kayser-Fleischer ring.单侧凯泽-弗莱舍尔环。
Br J Ophthalmol. 1986 Jun;70(6):469-70. doi: 10.1136/bjo.70.6.469.