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目的:评价二流基丁二酸(DMSA)对肝状核变性(HLD)的临床治疗价值。方法:60例HLD患者均予口服DMSA70mg/kg·d,Bid,疗程为4w。以改良Goldstein分级法作疗效判断,并记录治疗前后铜代谢指标、肝肾功能、周围血象的变化及副反应。结果:显效16例(26.7%),好转31例(51.7%),无效12例(21.7%);总有效率78.3%。疗后尿排铜量显著增加。但发现有鼻、齿龈出血、血小板减少等副反应。结论:DMAS是治疗HLD的有效药物,具有高效、低毒、服用方便等优点。
Objective: To evaluate the clinical value of dimethyl succinate (DMSA) in the treatment of hepatic degeneration (HLD). Methods: Sixty patients with HLD were given oral DMSA70mg / kg · d, Bid, treatment for 4w. The improved Goldstein method was used to judge the curative effect, and the changes of copper metabolism, liver and kidney function, peripheral blood and side effects were recorded before and after treatment. Results: 16 cases were markedly effective (26.7%), 31 cases were improved (51.7%), 12 cases (21.7%) were ineffective and the total effective rate was 78.3%. After treatment, urinary excretion of copper increased significantly. But found a nose, bleeding gums, thrombocytopenia and other side effects. Conclusion: DMAS is an effective drug for the treatment of HLD and has the advantages of high efficiency, low toxicity and convenient taking.