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甘豆灵联合二巯丁二钠治疗中国神经型肝豆状核变性患者的疗效和安全性评估。

Evaluation of efficacy and safety of gandouling plus sodium dimercaptosulphonate in treatment of patients with neurological Wilson's disease from China.

机构信息

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

Department of Pediatrics, the First Affiliated Hospital of Anhui University of Traditional Chinese Medicine, Hefei 230031, China.

出版信息

J Tradit Chin Med. 2018 Oct;38(5):781-786.

PMID:32185997
Abstract

OBJECTIVE

To evaluate the efficacy and safety of gandouling plus sodium dimercaptosulphonate (DMPS) on neurological Wilson's disease (WD) in patients.

METHODS

We retrospectively evaluated the clinical records of 125 WD patients with neurological syndromes who were treated with gandouling plus sodium DMPS or DMPS used alone. All patients had a history of neurological deterioration during their diseases courses. The clinical efficacies, adverse reactions, and results of the various hematological and biochemical investigations were recorded for statistical analysis.

RESULTS

92.30% (60 patients) of the WD patients treated with the combined therapy experienced an improved or stable neurological condition paralleled by a significantly improved GAS score. Meanwhile, the WBC and PLT counts stabilized, liver function and renal function were improved or remained stable. The combined therapy also obviously promoted the 24-h urinary copper excretion. In particular, only 30.76% of the WD patients experienced mild adverse reactions, including neurological deterioration in 5 patients (7.69%), hepatic worsening in 1 subject (1.89%), which was less frequently than those in the control group treated with DMPS only.

CONCLUSION

Our findings indicate that the safety and efficacy of gandou-ling plus DMPS is superior to those of DMPS used alone in the WD patients with neurological symptoms.

摘要

目的

评估甘豆苓联合二巯丁二酸钠(DMPS)治疗神经型肝豆状核变性(WD)的疗效和安全性。

方法

回顾性分析 125 例神经型 WD 患者的临床资料,患者均有病情加重的病史,分别采用甘豆苓联合 DMPS 或 DMPS 治疗,记录其临床疗效、不良反应及各项血液生化检查结果。

结果

联合治疗组患者的 GAS 评分显著提高,92.30%(60 例)患者的神经症状改善或稳定;同时白细胞、血小板计数稳定,肝功能、肾功能改善或稳定,24 h 尿铜排泄量明显增加。仅 30.76%的患者出现不良反应,其中神经症状加重 5 例(7.69%),肝功能恶化 1 例(1.89%),明显少于单纯 DMPS 治疗组。

结论

甘豆苓联合 DMPS 治疗神经型 WD 患者的安全性和疗效优于单纯 DMPS 治疗。

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