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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.

DOI:10.1007/BF02141489
PMID:3949188
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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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.
2
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本文引用的文献

1
PENICILLAMINE THERAPY FOR HEPATOLENTICULAR DEGENERATION.青霉胺治疗肝豆状核变性
JAMA. 1964 Sep 7;189:748-54. doi: 10.1001/jama.1964.03070100042008.
2
[Regression of a Kayser-Fleischer ring in a case of Wilson's disease during penicillamine therapy (author's transl)].青霉胺治疗威尔逊病时凯-弗环的消退(作者译)
Klin Monbl Augenheilkd. 1980 Feb;176(2):235-8. doi: 10.1055/s-2008-1057434.
3
Wilson's disease. Electron microscopic, x-ray energy spectroscopic, and atomic absorption spectroscopic studies of corneal copper deposition and distribution.
接触重金属和元素周期表中其他化学元素所产生的临床和法医体征。
J Clin Med. 2023 Mar 29;12(7):2591. doi: 10.3390/jcm12072591.
4
Chelation therapy in liver diseases of childhood: Current status and response.儿童肝脏疾病中的螯合疗法:现状与反应
World J Hepatol. 2021 Nov 27;13(11):1552-1567. doi: 10.4254/wjh.v13.i11.1552.
5
A 10-year-old boy with dystonia, expression-less facies, and tremors referred for ophthalmic examination.一名患有肌张力障碍、表情淡漠和震颤的10岁男孩前来接受眼科检查。
Oman J Ophthalmol. 2021 Jun 28;14(2):128-130. doi: 10.4103/ojo.OJO_262_2017. eCollection 2021 May-Aug.
6
Investigation of corneal topographic and densitometric properties of Wilson's disease patients with or without a Kayser-Fleischer ring.对患有或未患有凯-费环的威尔逊氏病患者的角膜地形图和密度测量特性进行研究。
Eye Vis (Lond). 2021 Mar 11;8(1):8. doi: 10.1186/s40662-021-00231-9.
7
Classification and differential diagnosis of Wilson's disease.肝豆状核变性的分类与鉴别诊断。
Ann Transl Med. 2019 Apr;7(Suppl 2):S63. doi: 10.21037/atm.2019.02.07.
8
The Hematologic Definition of Monoclonal Gammopathy of Undetermined Significance in Relation to Paraproteinemic Keratopathy (An American Ophthalmological Society Thesis).意义未明的单克隆丙种球蛋白病与副蛋白血症性角膜病变相关的血液学定义(美国眼科学会论文)
Trans Am Ophthalmol Soc. 2016 Aug;114:T7.
9
The sunflower cataract in Wilson's disease: pathognomonic sign or rare finding?威尔逊病中的向日葵样白内障:特征性体征还是罕见发现?
Acta Neurol Belg. 2016 Sep;116(3):325-8. doi: 10.1007/s13760-015-0566-1. Epub 2015 Nov 17.
10
Diagnosis and treatment of Wilson's disease.肝豆状核变性的诊断与治疗
Curr Neurol Neurosci Rep. 2002 Jul;2(4):317-23. doi: 10.1007/s11910-002-0007-4.
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
[Wilson's disease in the German Democratic Republic. III. Diagnosis and therapy].[德意志民主共和国的威尔逊氏病。III. 诊断与治疗]
Z Gesamte Inn Med. 1980 Feb 15;35(4):161-6.
5
Hudson memorial lecture: Wilson's disease: genetics and biochemistry--their relevance to therapy.哈德逊纪念讲座:威尔逊氏病:遗传学与生物化学——及其与治疗的相关性
J Inherit Metab Dis. 1983;6 Suppl 1:51-8. doi: 10.1007/BF01811324.
6
Changes in Kayser-Fleischer ring during treatment of hepatolenticular degeneration.肝豆状核变性治疗期间凯-弗环的变化
Arch Ophthalmol. 1968 Nov;80(5):622-31. doi: 10.1001/archopht.1968.00980050624008.
7
Disappearance of Kayser-Fleischer rings. Effects of penicillamine.凯泽-弗莱舍尔环的消失。青霉胺的作用。
Arch Ophthalmol. 1969 Dec;82(6):738-41. doi: 10.1001/archopht.1969.00990020730003.
8
Long-term therapy of Wilson's disease.威尔逊病的长期治疗
Ann Intern Med. 1971 Jul;75(1):57-65. doi: 10.7326/0003-4819-75-1-57.
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Br J Ophthalmol. 1970 Dec;54(12):794-800. doi: 10.1136/bjo.54.12.794.
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Presymptomatic Wilson's disease.症状前威尔逊病
Lancet. 1967 Sep 16;2(7516):575-9. doi: 10.1016/s0140-6736(67)90735-0.