有驱铜治疗史肝豆状核变性患者疾病严重程度及其影响因素分析  

Disease severity and influencing factors in patients with Wilson’s disease who had previously undergone copper removal therapy

作　　者：龙文成 彭介入 蔡琳 汪玥杉 沈晓丽 张璐 董尧 张琦文 杨代兰 廖娟 杨春霞 LONG Wen-cheng;PENG Jie-ru;CAI Lin;WANG Yue-shan;SHEN Xiao-li;ZHANG Lu;DONG Yao;ZHANG Qi-wen;YANG Dai-lan;LIAO Juan;YANG Chun-xia(Department of Epidemiology and Health Statistics,West China School of Public Health/West China Fourth Hospital,Sichuan University,Chengdu,Sichuan 610041,China;不详)

机构地区：[1]四川大学华西公共卫生学院/华西第四医院,流行病与卫生统计学系,四川成都610041 [2]四川大学华西公共卫生学院/华西第四医院,消化内科,四川成都610041 [3]电子科技大学医学院附属妇女儿童医院·成都市妇女儿童中心医院,医务部

出　　处：《现代预防医学》2024年第22期4219-4224,共6页Modern Preventive Medicine

摘　　要：目的基于统一肝豆状核变性评分量表(UWDRS)综合评估既往确诊且经历过驱铜治疗的肝豆状核变性(WD)患者疾病严重程度,并分析其影响因素,为患者管理和预后改善提供参考。方法以2023年7月至2024年3月在华西第四医院入院治疗的按照诊断标准确诊的WD患者为研究对象。使用自行设计的结构性调查问卷、UWDRS量表进行调查,采用单因素分析和多元线性回归分析患者疾病严重程度及影响因素。结果纳入符合条件且数据完整的患者103名,WD患者UWDRS总评分为27.0(11.5,60.5)、神经症状评分为21.0(4.5,48.5)、肝脏症状评分为3.0(1.0,4.0)、精神症状评分为5.0(2.0,8.0)。超过50%的患者仍然存在手快复轮替、手指捏合、流涎、步态异常、言语障碍、书写能力缺陷、其他流血或血肿的症状/体征。多元线性回归分析结果显示,文化程度、首次确诊疾病分型、低铜饮食、社会支持和药物依从性是UWDRS总评分的影响因素(P<0.05)。患者文化程度越高、药物依从性越好、社会支持水平越高,UWDRS评分越低;肝型患者比脑型患者评分更低,尽量坚持低铜饮食比一直坚持低铜饮食者评分更低。结论既往确诊且经历过驱铜治疗的WD患者中仍有超过50%的患者存在不同程度的神经症状,有关神经症状的治疗还有待进一步改善。文化程度、首次确诊疾病分型、低铜饮食、社会支持和药物依从性是WD患者疾病严重程度的影响因素。Objective To comprehensively assess the disease severity in patients with Wilson’s disease(WD)who had been previously diagnosed and treated with copper removal by Unified Wilson’s Disease Rating Scale(UWDRS)and analyze its influencing factors,in order to provide a reference for patient management and improvement of patients’health.Methods Wilson’s disease patients who were admitted to West China Fourth Hospital of Sichuan University from July 2023 to March 2024 were selected as the study subjects,and all patients have been diagnosed according to the diagnostic criteria.The survey was conducted using self-designed structured questionnaires and UWDRS scales.Univariate analysis and multiple linear regression analysis were used to analyze the disease severity and influencing factors of patients with WD who had been previously diagnosed and treated with copper removal.Results A total of 103 patients with eligible and complete data were included.The total UWDRS score of patients with WD who underwent copper repulsion therapy was 27.0(11.5,60.5),neurological symptom score of 21.0(4.5,48.5),hepatic symptom score of 3.0(1.0,4.0),and psychiatric symptom score of 5.0(2.0,8.0).More than 50%of patients still have symptoms or signs of rapid alternating movements of hand,finger tapping,salivation,gait abnormalities,speech impairment(dysarthria),impaired handwriting,and other bleeding or hematoma.Multiple linear regression analysis showed that education level,classification of first-time diagnosed disease,low-copper diet,social support and medication adherence were the influencing factors of UWDRS total score(P<0.05).The higher the patient’s level of education,medication adherence,and social support are,the lower the UWDRS score is.Patients with hepatic type had lower scores than those with cerebral type,and those who tried to adhere to a low-copper diet had lower scores than those who adhered to a low-copper diet all the time.Conclusion More than 50%of the previously diagnosed patients with WD who have been treated wi

关 键 词：肝豆状核变性 统一肝豆状核变性量表(UWDRS) 影响因素分析 

分 类 号：R742.4[医药卫生—神经病学与精神病学]