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Effects of long-term treatment in Wilson's disease with D-penicillamine and zinc sulphate.

作者信息

Czlonkowska A, Gajda J, Rodo M

机构信息

2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.

出版信息

J Neurol. 1996 Mar;243(3):269-73. doi: 10.1007/BF00868525.

DOI:10.1007/BF00868525
PMID:8936358
Abstract

The results of treatment with D-penicillamine (D-P) or zinc sulphate (Zn) in 67 newly diagnosed cases of Wilson's disease have been compared. All patients (7 with hepatic, 1 with psychiatric and 59 with neurological or preclinical forms) were fully compliant. During 12 years of observation, 34 patients received d-P and 33 Zn as the primary treatment. Fifteen patients (44%) discontinued D-P, in 10 cases owing to side effects. Four (12%) patients discontinued Zn, in 2 cases because of side-effects. One patient who received Zn deteriorated during the first few months after the initiation of therapy. The effectiveness of long-term treatment with D-P and Zn was similar in those patients who were able to continue the initial therapy. Zn was tolerated better than D-P; we suggest, therefore, that it may be recommended as an initial therapy for patients in the preclinical stage of Wilson's disease or with neurological presentation of the disease. More observation is needed for patients with the hepatic and psychiatric forms of the disease.

摘要

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Challenges and dilemmas in pediatric hepatic Wilson's disease.小儿肝豆状核变性的挑战与困境

本文引用的文献

1
The copper and iron content of brain and liver in the normal and in hepato-lenticular degeneration.正常及肝豆状核变性情况下脑和肝脏中的铜和铁含量
Brain. 1948 Dec;71(Pt. 4):410-5. doi: 10.1093/brain/71.4.410.
2
The effect of BAL (2,3-dimercaptopropanol) on hepatolenticular degeneration (Wilson's disease).二巯丙醇(BAL)对肝豆状核变性(威尔逊病)的影响。
N Engl J Med. 1951 Dec 13;245(24):917-25. doi: 10.1056/NEJM195112132452401.
3
An improved colorimetric enzymatic assay of ceruloplasmin.一种改良的铜蓝蛋白比色酶法测定。
World J Hepatol. 2023 Oct 27;15(10):1109-1126. doi: 10.4254/wjh.v15.i10.1109.
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Integrated Metabolomics and Network Pharmacology to Reveal the Mechanisms of Gandouling Tablets Against Copper-Overload-Induced Neuronal Injury in Rats with Wilson's Disease.基于代谢组学和网络药理学的方法揭示肝豆灵片防治肝豆状核变性模型大鼠铜过载神经损伤的作用机制
Drug Des Devel Ther. 2023 Jun 13;17:1763-1782. doi: 10.2147/DDDT.S409691. eCollection 2023.
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Late-onset Wilson Disease in a Patient Followed-up for Nonalcoholic Fatty Liver Disease.非酒精性脂肪性肝病随访患者中的晚发性威尔逊病。
Intern Med. 2024 Jan 1;63(1):71-75. doi: 10.2169/internalmedicine.1789-23. Epub 2023 Apr 28.
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The Role of Zinc in the Treatment of Wilson's Disease.锌在威尔逊病治疗中的作用。
Int J Mol Sci. 2022 Aug 18;23(16):9316. doi: 10.3390/ijms23169316.
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Front Genet. 2022 Apr 4;13:875694. doi: 10.3389/fgene.2022.875694. eCollection 2022.
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Comparison of the Effectiveness and Safety of d-Penicillamine and Zinc Salt Treatment for Symptomatic Wilson Disease: A Systematic Review and Meta-Analysis.青霉胺与锌盐治疗有症状威尔逊病的有效性和安全性比较:一项系统评价和荟萃分析
Front Pharmacol. 2022 Mar 18;13:847436. doi: 10.3389/fphar.2022.847436. eCollection 2022.
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World J Hepatol. 2021 Nov 27;13(11):1552-1567. doi: 10.4254/wjh.v13.i11.1552.
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J Lab Clin Med. 1961 Jul;58:161-8.
4
Penicillamine, a new oral therapy for Wilson's disease.青霉胺,一种治疗威尔逊氏病的新型口服疗法。
Am J Med. 1956 Oct;21(4):487-95. doi: 10.1016/0002-9343(56)90066-3.
5
Chelation treatment of neurological Wilson's disease.神经型威尔逊病的螯合疗法。
Q J Med. 1993 Mar;86(3):197-204.
6
Fatal deterioration of Wilson's disease after institution of oral zinc therapy.口服锌剂治疗后威尔逊病的致命性恶化。
Arch Neurol. 1993 Oct;50(10):1007-8. doi: 10.1001/archneur.1993.00540100008005.
7
Development of neurologic symptoms in a patient with asymptomatic Wilson's disease treated with penicillamine.用青霉胺治疗的无症状威尔逊病患者出现神经症状
Arch Neurol. 1994 Mar;51(3):304-5. doi: 10.1001/archneur.1994.00540150106026.
8
Oral zinc therapy for Wilson's disease.威尔逊氏病的口服锌疗法。
Ann Intern Med. 1983 Sep;99(3):314-9. doi: 10.7326/0003-4819-99-3-314.
9
3 years of continuous oral zinc therapy in 4 patients with Wilson's disease.4例威尔逊病患者接受3年持续口服锌治疗。
Acta Neurol Scand. 1983 Jun;67(6):356-64. doi: 10.1111/j.1600-0404.1983.tb03153.x.
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Copper chelation in patients with Wilson's disease. A comparison of penicillamine and triethylene tetramine dihydrochloride.肝豆状核变性患者的铜螯合作用。青霉胺与二盐酸三乙烯四胺的比较。
Q J Med. 1973 Jul;42(167):441-52.