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目的:研究二巯基丙磺钠(DMPS)联合锌剂治疗肝豆状核变性(WD)的疗效及最佳治疗方案。方法:选取WD患者50例(脑型38例,肝型12例),接受DMPS治疗16个疗程,每疗程用改良Young量表对脑型DM患者进行神经症状评分,全部WD患者行血常规、肝功能、出凝血功能、脑脊液铜、血清铜、尿铜等检查。患者出院后用锌剂维持治疗。结果进行统计学分析。结果:DMPS治疗后,脑型WD患者的神经症状总评分、语言、肌张力增高、震颤评分较治疗前下降,吞咽功能、精神异常评分升高。治疗前咽喉肌功能评分值、血清铜、年龄是影响是否出现症状加重的因素。治疗后脑脊液铜、血清铜较治疗前降低,尿铜较治疗前升高。血清铜与DMPS剂量、疗程、治疗方式成负相关;尿铜与治疗药物剂量、治疗方式呈正相关,与疗程呈负相关。WD患者治疗前后肝功能无明显变化,肝型患者治疗后凝血功能下降。锌剂长期维持过程中,神经症状持续好转。结论:DMPS对于肝型WD治疗效果不佳。但DMPS可有效改善脑型WD的神经症状,排铜效果优于青霉胺。治疗过程中,可能出现吞咽、语言、精神症状一过性加重,但并未出现类似青霉胺的不可逆神经症状加重。DMPS的治疗以长疗程、大剂量、静脉推注的方案为佳。间断DMPS结合锌剂长期维持,可能是脑型WD患者的有效治疗方案。
Objective: To study the efficacy and optimal treatment of hepatic degeneration (WD) with DMPS combined with zinc. Methods: Fifty WD patients (38 brain and 12 liver) were enrolled in this study. Sixteen courses of DM were treated with DMPS. Neurological symptoms were scored in DM patients with Modified Young Scale. All WD patients underwent routine blood tests, Liver function, a coagulation function, cerebrospinal fluid copper, serum copper, urine copper and other tests. Patients were left with zinc treatment to maintain treatment. Results were statistically analyzed. Results: After DMPS treatment, the total score of neurological symptoms, language, and muscle tone were increased in patients with brain WD, and the tremor score was lower than before treatment. The swallowing function and mental abnormality score were increased. Throat function scores before treatment, serum copper, age is the impact of whether the symptoms of aggravating factors. After treatment, cerebrospinal fluid copper and serum copper were lower than before treatment, and urinary copper was elevated before treatment. Serum copper and DMPS dose, treatment, treatment is negatively correlated; urinary copper and treatment of drug dose, treatment was positively correlated with the course was negatively correlated. WD patients before and after treatment had no significant changes in liver function, liver function after treatment, coagulation decreased. Long-term maintenance of zinc, neurological symptoms continued to improve. Conclusion: DMPS is not effective in the treatment of hepatic WD. But DMPS can effectively improve the neurological symptoms of brain WD, copper discharge effect is better than penicillamine. During treatment, swallowing, language, mental symptoms may be transient, but did not appear similar penicillamine irreversible neurological symptoms worse. DMPS treatment with long course, high-dose, intravenous bolus program is better. Intermittent DMPS with zinc long-term maintenance may be an effective treatment of brain WD patients.