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联合二巯丁二酸和锌治疗青霉胺诱导过敏或早期神经恶化的神经型肝豆状核变性患者。

Combined dimercaptosuccinic acid and zinc treatment in neurological Wilson's disease patients with penicillamine-induced allergy or early neurological deterioration.

机构信息

Department of Neurology, The First Affiliated Hospital of Anhui Medical University, Hefei 230022, China.

Department of Neurology, The First Affiliated Hospital of Anhui University of Traditional Chinese Medicine, Hefei 230031, China.

出版信息

Biosci Rep. 2020 Aug 28;40(8). doi: 10.1042/BSR20200654.

Abstract

The clinical data of safety and efficacy of a combined treatment with dimercaptosuccinic acid (DMSA) and Zinc with 2 years' follow-up in 60 neurological Wilson's disease (WD) patients was retrospectively analyzed. All the patients included in the present study were newly diagnosed and initialized with D-penicillamine (DPA) treatment but were found to have either neurological deterioration or allergy, and their treatment was switched to a combined treatment of DMSA and Zinc. Fifty-one patients (85%) had the neurological symptoms improved 1 and 2 years after treatment, 7 (11.67%) experienced a stable neurological condition, and 2 (3.33%) suffered deterioration of neurological symptoms. No early neurological deterioration was observed in all patients. Twenty-five percent patients experienced mild adverse reactions which did not require a discontinuation of the DMSA and Zinc treatment. Our study confirmed the safety and efficacy of the combined DMSA and Zinc therapy as an initial and probably long-term treatment in neurological WD patients.

摘要

回顾性分析了 60 例神经型 Wilson 病(WD)患者联合应用二巯丁二酸(DMSA)和锌治疗 2 年的安全性和疗效的临床数据。本研究纳入的所有患者均为新诊断并初始采用 D-青霉胺(DPA)治疗,但发现有神经恶化或过敏,故治疗方案改为 DMSA 和锌联合治疗。治疗 1 年和 2 年后,51 例(85%)患者的神经症状改善,7 例(11.67%)神经状况稳定,2 例(3.33%)神经症状恶化。所有患者均未观察到早期神经恶化。25%的患者出现轻度不良反应,但无需停止 DMSA 和锌治疗。本研究证实了联合应用 DMSA 和锌作为神经型 WD 患者初始和可能长期治疗的安全性和有效性。

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Combination Therapy Using Chelating Agent and Zinc for Wilson's Disease.使用螯合剂和锌联合治疗肝豆状核变性。
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Early neurological worsening in patients with Wilson's disease.威尔逊病患者的早期神经功能恶化。
J Neurol Sci. 2015 Aug 15;355(1-2):162-7. doi: 10.1016/j.jns.2015.06.010. Epub 2015 Jun 7.
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