肝豆汤联合DMPS驱铜治疗对Wilson病湿热内蕴型认知功能障碍的影响

目的:探讨肝豆汤联合二巯基丙磺酸钠(DMPS)治疗对湿热内蕴型肝豆状核变性患者的认知功能障碍影响。方法:回顾性分析湿热内蕴型肝豆状核变性患者85例,其中治疗组45例,对照组40例,其中治疗组给予中药肝豆汤联合二巯基丙磺酸钠治疗,对照组为单独应用二巯基丙磺酸钠治疗的患者;治疗共进行4个疗程,每个疗程为8 d,DMPS驱铜治疗6 d后间歇休息2 d,在治疗前后分别进行蒙特利尔认知评估量表(MoCA)评定,并检测认知功能相关指标血清超氧化物歧化酶(SOD),同型半胱胺酸(Hcy)水平的变化,观察24 h尿铜的改变,并检测肝功能、肾功能、白细胞、血小板等相关安全性指标。结果:两组患者在治疗后,MoCA评分总分较本组治疗前均明显改善,其中以治疗组改善最为明显;治疗后两组间子项目的评分比较,其中视空间与执行功能、注意力治疗组改善显著优于对照组(P0.05);两组血清学指标SOD水平组均改善明显,治疗组显著优于对照组(P0.05),治疗后Hcy水平治疗组明显改善(P0.05),对照组无明显变化;两组驱铜效果均较为显著(P0.01),两组组间对比,治疗组优于对照组(P0.05)。结论:肝豆汤联合二巯基丙磺酸钠可显著改善Willson病认知功能,血清SOD,Hcy水平显著改善,驱铜效果明显,不良反应轻微,安全有效。

Effect of Gandou Decoction Combined with Sodium Dimercaptopropanesulfonate in Treatment of Cognitive Impairment of Patients of Wilson's Disease

Objective: To investigate the effect of Gandou decoction combined with sodium dimercaptopropanesulfonate in treatment of cognitive impairment of patients of Wilson''s disease (WD). Method: Totally 85 WD patients were collected and divided into two groups. One group was administered with Gandou decoction combined with sodium dimercaptopropanesulfonate, and the other group was given sodium dimercaptopropanesulfonate for four courses. The clinical and laboratory parameters were compared at the baseline and the fourth course. Result: After 4 courses of treatment, patients in two groups showed obvious efficacy in improving cognitive function, and the score of Montreal cognitive assessment (MoCA) in treatment group increased more obviously; among the 8 sub-items of MoCA scale, the executive capability and the linguistic function increased more obviously; the cognitive function-related index superoxide dismutase(SOD) was increased obviously in both groups, and the treatment group was superior to control group in this aspect. In addition, the level of index superoxide dismutase(SOD); homocysteine(Hcy) was significantly decreased in treatment group, but with no obvious change in control group. The 24-hour urinary was also changed obviously in treatment group compared with control group; adverse reaction was reported, which showed obvious difference between two groups. Conclusion: The regimen of Gandou decoction combined with sodium dimercaptopropanesulfonate is an effective therapy for WD patients with cognitive impairment.