目的观察肝豆汤合驱铜疗法对Wilson病(Wilsons disease,WD)患者生活质量的影响。方法共纳入135例WD患者,在二巯基丙磺酸钠强力驱铜治疗及青霉胺和二巯基丁二酸维持驱铜治疗的基础上,口服肝豆片。治疗前及治疗1年后分别采用WHO生活质量简表评价患者的生活质量,并观察总体疗效。结果与治疗前比较,135例WD患者治疗后WHO生活质量简表的生理领域、心理领域、社会领域、环境领域得分均显著提高(P〈0.01)。135例WD患者中,临床痊愈5例,显效33例,有效88例,无效7例,恶化2例,无死亡病例,总有效率达93.3%。结论肝豆汤口服合驱铜治疗可以明显改善WD患者的生活质量。
Objective To observe the effect of Gandou Decoction combined with decoppering therapy on the quality of life in patients with Wilson's disease(WD).Methods A total of 135 patients with WD were enrolled as subjects.In addition to sodium dimercaptosulphonate for short-term decoppering,as well as penicillamine and dimercaptosuccinic acid for long-term decoppering,patients were orally given Gandou Tablet.The quality of life in patients was evaluated using the World Health Organization Quality of Life AssessmentShort Version(WHOQOL-BREF)before and one year after treatment,and the overall outcome was observed.Results In 135 patients with WD,the domain scores for physiology,psychology,society,and environment in the WHOQOL-BREF were significantly increased after treatment(P〈0.01).There were 5cases with recovery,33 cases with marked response,88 cases with response,7cases with no response,2cases with deterioration,and no death cases.The overall response rate was up to 93.3%.Conclusion Gandou Decoction combined with decoppering therapy can substantially improve the quality of life in patients with WD.