MODERN ASPECTS OF PATHOGENETIC TREATMENT WITH ZINC SALTS OF PATIENTS WITH WILSON'S DISEASE IN UKRAINE

Ivan Voloshyn-Gaponov
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Abstract

The aim – to study the effectiveness of zinc salts in the treatment of patients with neurological forms of Wilson's disease (WD). Materials and methods. The analysis of the treatment results of 128 patients with hepatocerebral degeneration (71 men and 57 women) in the State Institution “Institute of Neurology, Psychiatry and Narcology of the National Academy of Medical Sciences of Ukraine” was carried out. At the time of hospitalization, the age of patients ranged from 5 to 55 years, an average of 27.3 years, and at the time of debut of the disease - from 1 year to 40 years, an average of 21.3 years. 33 patients underwent monotherapy with zinc salts, 63 – combined therapy with small doses of penicillamine and zinc salts, 32 – monotherapy with penicillamine. Results. Because of the treatment, 67.1 % of patients showed an improvement in neuropsychiatric status: speech improved significantly, tremor of the extremities and the amplitude of hyperkinesis decreased, muscle tone decreased, and cognitive functions improved. According to the international two-level rating scale (UWDRS), the total pathology index decreased by 21 points. Zinc salts are effective and low-toxic and can be the drug of choice in the treatment of patients with hepatocerebral degeneration in the pre-symptomatic stage of the disease, as well as at the stage of maintenance treatment, both as monotherapy and in combination with penicillamine. However, zinc salts and penicillamine are not enough for the treatment and rehabilitation of patients with hepatocerebral degeneration. Therefore, taking into account the clinical picture and the data of additional research methods, it is necessary to conduct courses of symptomatic treatment at least 1-2 times a year. Conclusions. Thus, it can be noted that zinc salts are very effective and low toxic, and, therefore, can be the drug of choice in the treatment of patients with HCD in the pre-symptomatic stage of the disease, as well as at the stage of maintenance therapy as a single drug, and in combination with penicillamine or other chelate drugs.
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锌盐对乌克兰威尔森氏病患者发病治疗的现代意义
目的是研究锌盐治疗神经系统型威尔逊氏病(WD)的有效性。材料和方法。对“乌克兰国家医学科学院神经病学、精神病学和神经科研究所”的128名肝脑变性患者(71名男性和57名女性)的治疗结果进行了分析。住院时,患者的年龄从5岁到55岁不等,平均27.3岁;发病时,患者的年龄从1岁到40岁不等,平均21.3岁。33例患者采用锌盐单药治疗,63例采用小剂量青霉胺和锌盐联合治疗,32例采用青霉胺单药治疗。结果。由于治疗,67.1%的患者表现出神经精神状态的改善:语言明显改善,四肢震颤和运动亢进的幅度减少,肌肉张力下降,认知功能改善。按照国际二级评定量表(UWDRS),总病理指数下降21分。锌盐有效且低毒,可作为治疗症状前阶段肝脑退行性疾病患者以及维持治疗阶段的首选药物,无论是单独治疗还是与青霉胺联合治疗。然而,锌盐和青霉胺不足以治疗和康复肝脑变性患者。因此,考虑到临床情况和其他研究方法的数据,每年至少进行1-2次对症治疗是必要的。结论。因此,可以注意到锌盐非常有效且毒性低,因此可以作为治疗HCD患者在疾病症状前阶段的首选药物,也可以作为维持治疗阶段的单药,也可以与青霉胺或其他螯合药物联合使用。
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