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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。

The Kayser-Fleischer ring during long-term treatment in Wilson's disease (hepatolenticular degeneration). A follow-up study.

作者信息

Lössner A, Lössner J, Bachmann H, Zotter J

出版信息

Graefes Arch Clin Exp Ophthalmol. 1986;224(2):152-5. doi: 10.1007/BF02141489.

Abstract

The role of Kayser-Fleischer rings is described in 67 patients with Wilson's disease, both in the asymptomatic and symptomatic stage of the disease during life-long therapy, which lasted up to 22 years. The rings were missing in 60% of patients in the presymptomatic stage and in 2% of those in the symptomatic stage at the time of diagnosis. The Kayser-Fleischer rings disappeared in 81% of the patients (completely in 41% and incompletely in 59%). In 2 of the 6 asymptomatic patients the rings did not reabsorb even after therapy of more than 10 years. The fading of Kayser-Fleischer rings seems to be independent not only of the stage of the disease but also of the effectiveness of the decopperizing treatment.

摘要

本文描述了67例威尔逊氏病患者凯泽尔-弗莱舍尔环的情况,这些患者在长达22年的终身治疗期间处于疾病的无症状和有症状阶段。在诊断时,60%的症状前期患者和2%的症状期患者没有凯泽尔-弗莱舍尔环。81%的患者凯泽尔-弗莱舍尔环消失(41%完全消失,59%不完全消失)。6例无症状患者中有2例即使经过10多年治疗,环也没有重新吸收。凯泽尔-弗莱舍尔环的消退似乎不仅与疾病阶段无关,而且与排铜治疗的效果无关。

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