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Received:July 14, 2023 Published Online:April 20, 2024
Received:July 14, 2023 Published Online:April 20, 2024
中文摘要: 目的 评估还原型谷胱甘肽(rGSH)联合二巯丙磺钠(DMPS)治疗神经型Wilson病(NWD)的有效性和安全性,同时探讨联合治疗对氧化应激水平的影响。
方法 回顾分析2020年5月2022年3月安徽中医药大学第一附属医院118例接受rGSH联合DMPS(观察组, n=62)或DMPS单药治疗(对照组, n=56)的NWD患者的临床资料。收集年龄、性别、临床疗效、不良反应、改良Young量表评分、白细胞、血小板、铜蓝蛋白(CER)、天冬氨酸氨基转移酶(AST)、丙氨酸氨基转移酶(ALT)、超氧化物歧化酶(SOD)和丙二醛(MDA)等资料并进行统计学分析。
结果 观察组62例患者中,有46例神经系统症状改善,15例保持稳定,1例在治疗期间恶化;对照组56例患者中,31例神经系统症状改善,18例趋于稳定,7例恶化;观察组神经系统症状改善情况优于对照组(U=2.381, P=0.017)。两组患者的改良Young量表评分均明显下降(P<0.05),且观察组显著低于对照组(P<0.05)。两组的白细胞、血小板和CER水平都保持稳定(P>0.05)。联合治疗在促进24 h尿铜排泄、改善肝功能指标(AST、ALT)和机体抗氧化应激水平(SOD、MDA)方面均优于DMPS单药治疗(P<0.05)。两组的不良反应发生率差异无统计学意义(χ2=0.102,P>0.05)。
结论 与DMPS单药治疗相比,rGSH联合DMPS治疗NWD患者能有效改善神经系统症状,增强尿铜排泄,提高抗氧化应激水平,安全性高。
Abstract:Objective To evaluate the effectiveness and safety of reduced glutathione (rGSH) in combination with sodium dimercaptosulphonate (DMPS) for neurological Wilson’s disease (NWD), and to explore the effect of the combined treatment on oxidative stress levels.
Methods Retrospective analysis of clinical data of 118 NWD patients who received rGSH combined with DMPS (observation group, n=62) or DMPS monotherapy (control group, n=56) at the First Affiliated Hospital of Anhui University of Traditional Chinese Medicine from May 2020 to March 2022. Clinical data such as age, gender, clinical outcome, adverse effects, modified Young scale score, white blood cells, platelets, ceruloplasmin (CER), aspartate aminotransferase (AST), alanine aminotransferase (ALT), superoxide dismutase (SOD) and malondialdehyde (MDA) were collected and statistically analysed.
Results Of the 62 NWD patients in the observation group, neurological symptoms improved in 46 cases and remained stable in 15 cases, while worsened in 1 case. Of the 56 NWD patients in the control group, neurological symptoms improved in 31 cases and remained stable in 18 cases, while worsened in 7 cases. The improvement of neurological symptoms in the observation group was better than that in the control group (U=2.381, P=0.017). The modified Young scale score was significantly decreased in both groups (P<0.05), and that in the observation group was lower compared to the control group (P<0.05). The levels of white blood cell, platelet, and CER remained stable in both groups (P>0.05). The combination treatment greatly facilitated 24-hour urinary copper excretion, and improved liver function (AST, ALT) and anti-oxidative stress levels (SOD, MDA) (P<0.05). Similar adverse reactions occurred in both groups (χ2=0.102, P>0.05).
Conclusion Compared to DMPS monotherapy, rGSH plus DMPS for NWD patients can effectively ameliorate neurological symptoms, enhance urinary copper excretion and improve anti-oxidative stress levels with a high safety.
keywords: Wilson’s disease Reduced glutathione Sodium dimercaptosulphonate Abnormal copper metabolism Neurological symptom Oxidative stress
文章编号: 中图分类号:R453.9 文献标志码:A
基金项目:国家中医药管理局中医药循证能力建设项目(2019XZZX-NB001);国家自然科学基金区域创新发展联合基金项目(U22A20366);安徽高校协同创新项目(GXXT-2020-025)
Author Name | Affiliation |
GAO Yannan, ZHANG Jing, TANG Lulu, YANG Wenming | Department of Neurology, The First Affiliated Hospital of Anhui University of Chinese Medicine, Hefei, Anhui 230031, China |
Author Name | Affiliation |
GAO Yannan, ZHANG Jing, TANG Lulu, YANG Wenming | Department of Neurology, The First Affiliated Hospital of Anhui University of Chinese Medicine, Hefei, Anhui 230031, China |
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