文章摘要
DMPS驱铜联合甲泼尼龙治疗DWI高信号脑型Wilson病患者的疗效观察
The clinical efficacy of DMPS copper displacement combined with methylprednisolone sodium succinate in the treatment of patients with DWI hyperintense cerebral Wilson’s disease
投稿时间:2024-12-01  
DOI:10.3969/j.issn.1000-0399.2025.06.004
中文关键词: Wilson病|弥散加权成像|统一WD等级评定量表|甲泼尼龙琥珀酸钠|二巯丙磺酸钠|铜代谢
英文关键词: Wilson’s disease|Diffusion-weighted imaging|UWDRS|Methylprednisolone sodium succinate|Sodium dimercaptopropane sulfonate|Copper metabolism
基金项目:安徽省高校科研重大项目(编号:2024AH040140);安徽中医药大学自然科学研究重大项目(编号:2021sfylco1)
作者单位E-mail
李瑶瑶 230012 安徽合肥 安徽中医药大学研究生院  
胡文彬 230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区 hwbzhx@163.com 
苏贝晴 230012 安徽合肥 安徽中医药大学研究生院  
刘娅 230012 安徽合肥 安徽中医药大学研究生院  
严彦 230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区  
吴君霞 230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区  
孙权 230012 安徽合肥 安徽中医药大学研究生院
230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区 
 
徐银 230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区  
喻绪恩 230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区  
王训 230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区  
胡纪源 230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区  
韩咏竹 230061 安徽合肥 安徽中医药大学神经病研究所附属医院神经内科二病区  
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中文摘要:
      目的 探讨二巯丙磺酸钠(DMPS)常规驱铜疗法和DMPS驱铜联合甲泼尼龙琥珀酸钠(MPS)治疗磁共振弥散加权成像(DWI)高信号的脑型Wilson病(WD)患者临床疗效的差异。方法 回顾性分析2023年1月至2024年12月在安徽中医药大学神经病学研究所附属医院住院诊治的磁共振DWI高信号的70例脑型WD患者临床资料,依据治疗方式分为使用DMPS常规驱铜治疗的对照组(n=40),以及DMPS常规驱铜治疗基础上加用MPS治疗的观察组(n=30)。两组患者均驱铜治疗8个疗程,每个疗程为8 d。比较两组患者治疗前后改良版中文统一WD等级评定量表-神经障碍(UWDRS-Ⅰ)和改良版中文统一WD等级评定量表-精神障碍(UWDRS-Ⅲ)评分,24 h尿铜、同型半胱氨酸以及血常规、肝肾功能等临床指标。结果 治疗前两组患者UWDRS-Ⅰ评分、UWDRS-Ⅲ评分、24 h尿铜,以及血常规、同型半胱氨酸、肝肾功能和头颅磁共振DWI高信号部位的比较,差异无统计学意义(P>0.05)。与治疗前相比,疗程后两组患者的组内UWDRS-Ⅰ与UWDRS-Ⅲ评分、同型半胱氨酸指标均有明显改善,24 h尿铜均明显增加(P<0.05);观察组的总胆红素和清蛋白指标均改善(P<0.05)。与对照组比较,观察组患者治疗前后UWDRS-Ⅰ与UWDRS-Ⅲ评分、清蛋白指标均明显改善,并且24 h尿铜增加明显(P<0.05)。结论 使用DMPS驱铜联合MPS方案治疗DWI高信号脑型WD患者的疗效更佳,可有效地改善患者的神经精神症状及部分临床检验指标。
英文摘要:
      Objective To investigate the differences in the clinical efficacy of conventional copper displacement therapy with DMPS and DMPS combined with MPS in the treatment of patients with cerebral Wilson’s disease with magnetic resonance DWI hyperintensity. Methods A total of 70 patients with cerebral Wilson’s disease with magnetic resonance DWI hyperintensity who were hospitalized in the Affiliated Hospital of the Institute of Neurology of Anhui University of Traditional Chinese Medicine from January 2023 to December 2024 were collected, of which 40 patients(control group) were treated with conventional copper removal with DMPS. The other 30 patients(observation group) were treated with MPS in the first course on the basis of conventional copper displacement therapy, and the other treatments were the same as those in the control group. Each copper displacement treatment lasted for 8 days, a total of 8 courses. The differences in UWDRS-Ⅰ and UWDRS-Ⅲ. scores, 24-hour urine copper, homocysteine, blood routine, liver and kidney function and other clinical indicators between the two groups before and after treatment were compared. Results There were no significant differences in UWDRS-Ⅰ score, UWDRS-Ⅲ score, 24-hour urine copper, blood routine, homocysteine, liver and kidney function, and brain magnetic resonance DWI hyperintensity between the two groups before treatment(P>0.05). Compared with before treatment, the UWDRS-Ⅰ, UWDRS-Ⅲ. scores and homocysteine indexes in the two groups were significantly improved after treatment, and the 24-hour urine copper significantly increased(P<0.05), and the total bilirubin and albumin indexes in the observation group were significantly improved(P<0.05). Compared with the control group after treatment, the UWDRS-I and UWDRS-Ⅲ scores and albumin indexes in the observation group were significantly improved after treatment, and the 24-hour urine copper increased significantly(P<0.05). Conclusion The use of DMPS copper displacement combined with MPS in the treatment of patients with DWI hyperintense cerebral WD has better efficacy, since it can improve the neuropsychiatric symptoms and some clinical test indicators.
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