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Article(id=1241675631411384793, tenantId=1146029695717560320, journalId=1227665162245664772, issueId=1241675628051755031, articleNumber=null, orderNo=null, doi=10.20043/j.cnki.MPM.202408150, pmid=null, cstr=null, oa=null, hot=null, price=null, onlineType=0, articleFormat=0, articleType=null, articleTypeStr=null, receivedDate=1723392000000, receivedDateStr=2024-08-12, revisedDate=null, revisedDateStr=null, acceptedDate=null, acceptedDateStr=null, onlineDate=1773968139745, onlineDateStr=2026-03-20, pubDate=1732464000000, pubDateStr=2024-11-25, doiRegisterDate=null, doiRegisterDateStr=null, onlineIssueDate=1773968139745, onlineIssueDateStr=2026-03-20, onlineJustAcceptDate=null, onlineJustAcceptDateStr=null, onlineFirstDate=null, onlineFirstDateStr=null, sourceXml=null, magXml=null, createTime=1773968139745, creator=13701087609, updateTime=1773968139745, updator=13701087609, issue=Issue{id=1241675628051755031, tenantId=1146029695717560320, journalId=1227665162245664772, year='2024', volume='51', issue='22', pageStart='4033', pageEnd='4224', issueExtLink='null', onlineDate='null', pubDate='null', beforeIssueId=null, nextIssueId=null, price=null, status=1, issueComplete=1, articleOrder=1, issueType=-1, specialIssue=null, createTime=1773968138945, creator=13701087609, updateTime=1773968595676, updator=13701087609, preIssue=null, nextIssue=null, ext={EN=IssueExt(id=1241677543783322543, tenantId=1146029695717560320, journalId=1227665162245664772, issueId=1241675628051755031, language=EN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=), CN=IssueExt(id=1241677543783322544, tenantId=1146029695717560320, journalId=1227665162245664772, issueId=1241675628051755031, language=CN, specialIssueTitle=, coverIllustrator=null, specialIssueEditor=, specialIssueAbout=)}, issueFiles=null}, startPage=4219, endPage=4224, ext={EN=ArticleExt(id=1241675632174748139, articleId=1241675631411384793, tenantId=1146029695717560320, journalId=1227665162245664772, language=EN, title=Disease severity and influencing factors in patients with Wilson’s disease who had previously undergone copper removal therapy, columnId=1228016569138213037, journalTitle=Modern Preventive Medicine, columnName=Clinical Medicine and Prevention, runingTitle=null, highlight=null, articleAbstract=
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 with copper removal still have different degrees of neurological symptoms, and the treatment of neurological symptoms needs to be further improved. Education level, classification of first-time diagnosed disease, low-copper diet, social support, and medication adherence were the influencing factors of disease severity in patients with WD.

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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。

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杨春霞，E-mail: ；
廖娟，E-mail:
, copyrightStatement=本刊刊出的所有文章不代表中华预防医学会和本刊编委会的观点，除非特别声明。, copyrightOwner=中华预防医学会和四川大学华西公共卫生学院, extLink=null, articleAbsUrl=null, sourceXml=pxD9RKClCJCpowOPknmr/g==, magXml=vq+s3ZomC8eD2265zX+2sQ==, pdfUrl=null, pdf=x0liWaeAF3JQJS11piVbjw==, pdfFileSize=611593, pdfExtLink=null, richHtmlUrl=null, mobilePdfUrl=null, reviewReport=null, pdfFirstPage=null, abstractGraph=null, abstractGraphContent=null, abstractVideo=null, citation=null, cebUrl=null, magXmlContent=Q+F/CjAaD18A2i2nJ15sNw==, mapNumber=null, authorCompany=null, fund=null, authors=

龙文成（1999—），男，硕士在读，研究方向：流行病学

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龙文成（1999—），男，硕士在读，研究方向：流行病学

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龙文成（1999—），男，硕士在读，研究方向：流行病学

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(In Chinese), articleTitle=Correlation between the Life quality and social supports in patients with hepatolenticular degeneration, refAbstract=null)], funds=null, companyList=[AuthorCompany(id=1241826535053791529, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, xref=1., ext=[AuthorCompanyExt(id=1241826535074763051, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, companyId=1241826535053791529, language=EN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=Department of Epidemiology and Health Statistics, West China School of Public Health/West China Fourth Hospital, Sichuan University, Chengdu, Sichuan 610041, China), AuthorCompanyExt(id=1241826535083151660, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, companyId=1241826535053791529, language=CN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=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)]), AuthorCompany(id=1241826535221563707, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, xref=2., ext=[AuthorCompanyExt(id=1241826535229952316, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, companyId=1241826535221563707, language=CN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=2.四川大学华西公共卫生学院/华西第四医院，消化内科)]), AuthorCompany(id=1241826535401918794, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, xref=3., ext=[AuthorCompanyExt(id=1241826535422890319, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, companyId=1241826535401918794, language=CN, country=null, province=null, city=null, postcode=null, companyName=null, departmentName=null, remark=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)])], figs=[ArticleFig(id=1241826543790527205, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, language=EN, label=Table 1, caption=

UWDRS score characteristics of study subjects

, figureFileSmall=null, figureFileBig=null, tableContent=
症状频数(n=103)频率(%)单项平均分
手轮替_左6664.11.12
手指捏合_左6563.11.13
手指捏合_右6361.21.14
流涎6159.21.05
手轮替_右6159.21.09
步态异常5957.30.57
言语障碍5452.41.04
其他流血或血肿5452.40.88
书写能力障碍5250.51.02
), ArticleFig(id=1241826543891190508, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, language=CN, label=表1, caption=

研究对象UWDRS评分特征

, figureFileSmall=null, figureFileBig=null, tableContent=
症状频数(n=103)频率(%)单项平均分
手轮替_左6664.11.12
手指捏合_左6563.11.13
手指捏合_右6361.21.14
流涎6159.21.05
手轮替_右6159.21.09
步态异常5957.30.57
言语障碍5452.41.04
其他流血或血肿5452.40.88
书写能力障碍5250.51.02
), ArticleFig(id=1241826544008631029, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, language=EN, label=Table 2, caption=

General information of the survey respondents and the results of the univariate analysis of the influencing factors of UWDRS total scores (n=103)

, figureFileSmall=null, figureFileBig=null, tableContent=
项目例数
［例(百分比, %)］		UWDRS总评分
［/M(P25,P75)］		P值
性别
男44(42.7)27.5(17.2, 55.5)0.692b
女59(57.3)27.0(10.0, 68.5)
年龄(岁)
10~<209(8.7)55.0(10.0, 71.0)0.066c
20~<3037(35.9)22.0(7.0, 43.0)
30~<4040(38.8)39.0(18.8, 73.2)
>=4017(16.5)26.0(15.0, 35.0)
BMI
<18.530(29.1)41.5(16.8, 69.0)0.026c
18.5~<24.055(53.4)28.0(12.5, 62.5)
>=24.018(17.5)16.5(8.3, 24.8)
户籍类型
城镇40(38.8)17.0(8.0, 35.0)0.007b
农村63(61.2)39.0(16.5, 70.5)
文化程度
初中及以下38(36.9)39.5(15.0, 71.0)0.002c
高中或中专25(24.3)39.0(22.0, 82.0)
大学及以上40(38.8)18.5(7.8, 29.0)
婚姻状况
未婚48(46.6)23.5(10.0, 69.5)0.433c
已婚45(43.7)25.0(12.0, 43.0)
离异8(7.8)37.0(32.8, 51.5)
丧偶2(1.9)31.0(26.5, 35.5)
职业
学生及托幼儿童12(11.7)11.0(5.3, 69.5)0.001c
机关、企事业单位10(9.7)14.0(11.2, 18.0)
服务业、工业及建筑13(12.6)15.0(5.0, 27.0)
待业或无业60(58.3)39.5(23.0, 72.5)
其他8(7.8)11.5(8.0, 24.5)
家庭人均月收入(元)
<2 50041(39.8)40.0(24.0, 71.0)0.002c
2 500~<5 00028(27.2)27.0(14.2, 58.5)
5 000~<10 00019(18.4)12.0(7.0, 26.0)
≥10 00015(14.6)21.0(10.0, 31.5)
家族史
有28(27.2)27.0(10.8, 45.8)0.596b
无75(72.8)27.0(12.5, 60.5)
发病年龄(岁)
<1848(46.6)29.0(9.8, 61.0)0.908b
≥1855(53.4)26.0(12.5, 60.0)
病程(年)
<524(23.3)20.5(10.0, 60.8)0.588c
5~<1022(21.4)29.5(9.8, 65.0)
10~<1524(23.3)25.5(13.2, 42.5)
≥1533(32.0)35.0(15.0, 63.0)
延迟诊断(月)
<652(50.5)26.5(9.0, 41.5)0.084b
≥651(49.5)34.0(15.5, 70.5)
延迟治疗(月)a
<692(89.3)27.5(11.0, 59.8)0.297b
≥68(7.8)48.5(26.0, 71.0)
初次诊断类型a
肝型25(24.3)10.0(5.0, 26.0)<0.001c
脑型27(26.2)42.0(29.5, 74.0)
混合型12(11.7)56.5(24.8, 73.0)
不清楚36(35.0)20.5(9.8, 36.0)
定期复查a
完全做到78(75.7)25.5(10.0, 61.2)0.400c
基本做到12(11.7)32.0(24.8, 60.5)
偶尔做到6(5.8)34.5(20.5, 38.0)
没做到4(3.9)72.0(44.8, 89.5)
治疗方案a
单纯输液4(3.9)35.5(10.2, 64.2)0.876c
输液+口服锌剂4(3.9)20.0(9.8, 36.0)
输液+口服螯合剂36(35.0)37.0(14.2, 63.8)
输液+口服螯合剂和锌剂56(54.4)27.0(11.5, 64.0)
是否接受过中药治疗a
是46(44.7)34.5(22.2, 58.5)0.100b
否54(52.4)22.0(8.0, 69.8)
药物依从性
差83(80.6)29.0(12.0, 68.0)0.139b
中等20(19.4)21.5(10.2, 36.2)
好0(0.0)-
低铜饮食
一直坚持55(53.4)39.0(18.0, 74.0)0.002c
尽量坚持41(39.8)16.0(8.0, 35.0)
偶尔或不低铜7(6.8)62.0(25.0, 99.0)
最近6个月体育锻炼(次/周)
< 153(51.5)35.0(16.0, 69.0)0.084c
1~<315(14.6)12.0(7.5, 74.0)
3~<513(12.6)12.0(6.0, 25.0)
≥522(21.4)23.0(15.8, 39.0)
社会支持a
低水平45(43.7)39.0(21.0, 82.0)0.004c
中等水平48(46.6)23.5(9.0, 46.8)
高水平10(9.7)13.0(7.3, 17.5)
), ArticleFig(id=1241826544172208892, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, language=CN, label=表2, caption=

调查对象UWDRS量表评分影响因素的单因素分析（n=103）

, figureFileSmall=null, figureFileBig=null, tableContent=
项目例数
［例(百分比, %)］		UWDRS总评分
［/M(P25,P75)］		P值
性别
男44(42.7)27.5(17.2, 55.5)0.692b
女59(57.3)27.0(10.0, 68.5)
年龄(岁)
10~<209(8.7)55.0(10.0, 71.0)0.066c
20~<3037(35.9)22.0(7.0, 43.0)
30~<4040(38.8)39.0(18.8, 73.2)
>=4017(16.5)26.0(15.0, 35.0)
BMI
<18.530(29.1)41.5(16.8, 69.0)0.026c
18.5~<24.055(53.4)28.0(12.5, 62.5)
>=24.018(17.5)16.5(8.3, 24.8)
户籍类型
城镇40(38.8)17.0(8.0, 35.0)0.007b
农村63(61.2)39.0(16.5, 70.5)
文化程度
初中及以下38(36.9)39.5(15.0, 71.0)0.002c
高中或中专25(24.3)39.0(22.0, 82.0)
大学及以上40(38.8)18.5(7.8, 29.0)
婚姻状况
未婚48(46.6)23.5(10.0, 69.5)0.433c
已婚45(43.7)25.0(12.0, 43.0)
离异8(7.8)37.0(32.8, 51.5)
丧偶2(1.9)31.0(26.5, 35.5)
职业
学生及托幼儿童12(11.7)11.0(5.3, 69.5)0.001c
机关、企事业单位10(9.7)14.0(11.2, 18.0)
服务业、工业及建筑13(12.6)15.0(5.0, 27.0)
待业或无业60(58.3)39.5(23.0, 72.5)
其他8(7.8)11.5(8.0, 24.5)
家庭人均月收入(元)
<2 50041(39.8)40.0(24.0, 71.0)0.002c
2 500~<5 00028(27.2)27.0(14.2, 58.5)
5 000~<10 00019(18.4)12.0(7.0, 26.0)
≥10 00015(14.6)21.0(10.0, 31.5)
家族史
有28(27.2)27.0(10.8, 45.8)0.596b
无75(72.8)27.0(12.5, 60.5)
发病年龄(岁)
<1848(46.6)29.0(9.8, 61.0)0.908b
≥1855(53.4)26.0(12.5, 60.0)
病程(年)
<524(23.3)20.5(10.0, 60.8)0.588c
5~<1022(21.4)29.5(9.8, 65.0)
10~<1524(23.3)25.5(13.2, 42.5)
≥1533(32.0)35.0(15.0, 63.0)
延迟诊断(月)
<652(50.5)26.5(9.0, 41.5)0.084b
≥651(49.5)34.0(15.5, 70.5)
延迟治疗(月)a
<692(89.3)27.5(11.0, 59.8)0.297b
≥68(7.8)48.5(26.0, 71.0)
初次诊断类型a
肝型25(24.3)10.0(5.0, 26.0)<0.001c
脑型27(26.2)42.0(29.5, 74.0)
混合型12(11.7)56.5(24.8, 73.0)
不清楚36(35.0)20.5(9.8, 36.0)
定期复查a
完全做到78(75.7)25.5(10.0, 61.2)0.400c
基本做到12(11.7)32.0(24.8, 60.5)
偶尔做到6(5.8)34.5(20.5, 38.0)
没做到4(3.9)72.0(44.8, 89.5)
治疗方案a
单纯输液4(3.9)35.5(10.2, 64.2)0.876c
输液+口服锌剂4(3.9)20.0(9.8, 36.0)
输液+口服螯合剂36(35.0)37.0(14.2, 63.8)
输液+口服螯合剂和锌剂56(54.4)27.0(11.5, 64.0)
是否接受过中药治疗a
是46(44.7)34.5(22.2, 58.5)0.100b
否54(52.4)22.0(8.0, 69.8)
药物依从性
差83(80.6)29.0(12.0, 68.0)0.139b
中等20(19.4)21.5(10.2, 36.2)
好0(0.0)-
低铜饮食
一直坚持55(53.4)39.0(18.0, 74.0)0.002c
尽量坚持41(39.8)16.0(8.0, 35.0)
偶尔或不低铜7(6.8)62.0(25.0, 99.0)
最近6个月体育锻炼(次/周)
< 153(51.5)35.0(16.0, 69.0)0.084c
1~<315(14.6)12.0(7.5, 74.0)
3~<513(12.6)12.0(6.0, 25.0)
≥522(21.4)23.0(15.8, 39.0)
社会支持a
低水平45(43.7)39.0(21.0, 82.0)0.004c
中等水平48(46.6)23.5(9.0, 46.8)
高水平10(9.7)13.0(7.3, 17.5)
), ArticleFig(id=1241826544344175363, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, language=EN, label=Table 3, caption=

Multiple linear regression analysis of the UWDRS total scores in the survey respondents (n=103)

, figureFileSmall=null, figureFileBig=null, tableContent=
项目回归系数标准误P值
常量62.0109.889<0.001
文化程度 (初中及以下)
高中0.8948.3620.915
大学及以上-19.0817.1390.009
首次确诊疾病分型 (肝型)
脑型18.7908.7390.034
混合型16.90810.9960.128
不清楚4.5998.5520.592
低铜饮食 (一直坚持)
尽量坚持-19.7636.7460.004
偶尔或不低铜2.56812.6190.839
药物依从性 (差)
中等-17.0047.8220.033
社会支持 (低水平)
中等水平-17.1896.4860.010
高水平-34.05111.2030.003
), ArticleFig(id=1241826544482587404, tenantId=1146029695717560320, journalId=1227665162245664772, articleId=1241675631411384793, language=CN, label=表3, caption=

调查对象UWDRS量表总评分影响因素的多元线性回归分析 (n=103)

, figureFileSmall=null, figureFileBig=null, tableContent=
项目回归系数标准误P值
常量62.0109.889<0.001
文化程度 (初中及以下)
高中0.8948.3620.915
大学及以上-19.0817.1390.009
首次确诊疾病分型 (肝型)
脑型18.7908.7390.034
混合型16.90810.9960.128
不清楚4.5998.5520.592
低铜饮食 (一直坚持)
尽量坚持-19.7636.7460.004
偶尔或不低铜2.56812.6190.839
药物依从性 (差)
中等-17.0047.8220.033
社会支持 (低水平)
中等水平-17.1896.4860.010
高水平-34.05111.2030.003
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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
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龙文成 1 , 彭介入 1, 3 , 蔡琳 2 , 汪玥杉 1 , 沈晓丽 1 , 张璐 1 , 董尧 1 , 张琦文 1 , 杨代兰 2 , 廖娟 2 , 杨春霞 1
现代预防医学 | 临床与预防 2024,51(22): 4219-4224
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现代预防医学 | 临床与预防 2024, 51(22): 4219-4224
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
全屏
龙文成1, 彭介入1, 3, 蔡琳2, 汪玥杉1, 沈晓丽1, 张璐1, 董尧1, 张琦文1, 杨代兰2, 廖娟2 , 杨春霞1
作者信息
  • 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
  • 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

    • 龙文成（1999—），男，硕士在读，研究方向：流行病学

通讯作者:

杨春霞，E-mail: ；
廖娟，E-mail:
Disease severity and influencing factors in patients with Wilson’s disease who had previously undergone copper removal therapy
Wen-cheng LONG1, Jie-ru PENG1, 3, Lin CAI2, Yue-shan WANG1, Xiao-li SHEN1, Lu ZHANG1, Yao DONG1, Qi-wen ZHANG1, Dai-lan YANG2, Juan LIAO2 , Chun-xia YANG1
Affiliations
  • Department of Epidemiology and Health Statistics, West China School of Public Health/West China Fourth Hospital, Sichuan University, Chengdu, Sichuan 610041, China
出版时间: 2024-11-25 doi: 10.20043/j.cnki.MPM.202408150
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摘要
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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。

肝豆状核变性  /  统一肝豆状核变性量表 (UWDRS)  /  影响因素分析
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 with copper removal still have different degrees of neurological symptoms, and the treatment of neurological symptoms needs to be further improved. Education level, classification of first-time diagnosed disease, low-copper diet, social support, and medication adherence were the influencing factors of disease severity in patients with WD.

Wilson’s disease  /  Unified Wilson’s Disease Rating Scale (UWDRS)  /  Analysis of influencing factors
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. 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. 现代预防医学, 2024 , 51 (22) : 4219 -4224 . DOI: 10.20043/j.cnki.MPM.202408150
Wen-cheng LONG, Jie-ru PENG, Lin CAI, Yue-shan WANG, Xiao-li SHEN, Lu ZHANG, Yao DONG, Qi-wen ZHANG, Dai-lan YANG, Juan LIAO, Chun-xia YANG. Disease severity and influencing factors in patients with Wilson’s disease who had previously undergone copper removal therapy[J]. Modern Preventive Medicine, 2024 , 51 (22) : 4219 -4224 . DOI: 10.20043/j.cnki.MPM.202408150
正文
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肝豆状核变性(Wilson disease, WD)是13号染色体ATP7B基因突变导致的常染色体隐性遗传病[1-2]。ATP7B基因突变导致其编码的铜转运P型ATP酶功能异常，进而导致胆道排铜障碍，铜离子在肝脏、脑等脏器积蓄产生损害[3]。WD患者临床表现多样，以肝病、神经症状、精神症状为主，呈现出明显的个体异质性[4]。WD患者确诊后需终生用药，有效驱铜治疗能够较好改善临床症状，约80%~85%接受治疗的WD患者长期预后良好[5]。治疗过程中的监测和疗效评价，对治疗方案的调整和改进具有重要意义。目前，对于经历常规驱铜治疗患者的症状评估和监测数据较少，本研究旨在通过UWDRS量表对既往确诊且经历过驱铜治疗的WD患者疾病严重程度进行评估，并对其影响因素进行分析，为WD患者管理和改善患者健康状况提供参考。
1　对象与方法
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1.1　研究对象
本研究基于“华西肝豆队列研究”项目，选取2023年7月至2024年3月于四川大学华西第四医院住院治疗的WD患者为调查对象。纳入标准：(1) 按照诊断标准确诊且经治疗的WD患者；(2) 能够正确理解问卷及量表问题，并配合完成UWDRS量表。本研究已获得四川大学华西第四医院/华西公共卫生学院医学伦理委员会伦理审查批准(伦理审批号：Gwll2023126)，所有调查对象均自愿参加，并签署知情同意书。
1.2　研究方法
1.2.1　问卷调查
采用自行设计的结构化调查问卷，由统一培训后调查员开展一对一面访调查。问卷包括一般人口学资料、家族史、生活饮食行为习惯、社会支持状况、疾病诊疗情况、药物依从性及预后情况等内容。其中，社会支持状况根据社会支持评定量表(Social Support Rating Seale, SSRS)进行评分，包括主观支持、客观支持和对支持利用三个维度[6]。总分66分，≤33分为低水平社会支持，33~44分为中等水平社会支持，≥45分为高水平社会支持。药物依从性由8条目Morisky用药依从性量表(Eight-Item Morisky Medical Adherence Scale, MMAS-8)进行评分，分值为0~8分，<6分为依从性差，6~8分为依从性中等，8分为依从性好[7]。
1.2.2　UWDRS量表评估
由专业神经内科医生或经培训且考察合格的医务工作人员，采用双人评估；评估结果不一致时，由两人讨论并达成一致意见后确认最终评分。
UWDRS量表分为神经症状评分、肝脏症状评分和精神症状评分3个子量表，共55个条目组成。26个条目由患者回答，29个条目由观察者评估。每个条目选项为有序分类变量，由轻到重5个等级(0~4分)。量表总评分为320分，其中神经症状208分、肝脏症状36分、精神症状76分，根据最终得分的高低反映患者疾病严重程度，得分越高表示越严重。中文版UWDRS量表由韩永升等人[8]翻译并进行信效度评价，结果表明量表的Cronbach α系数为0.967，神经功能为0.975，肝脏功能为0.493，精神症状为0.798，具有良好的信度和效度。
1.2.3　诊断标准及相关定义
肝豆状核变性的诊断：符合 2012 年欧洲肝病学会的临床指南，按照2001年莱比锡第 8 届 Wilson 病国际会议的诊断标准(Leipzig 评分系统)，根据诊断流程分步骤评分，总分≥4分则可确诊[5，9]。
1.2.4　统计分析方法
采用R 4.4.1软件进行统计分析，连续性变量不服从正态分布采用中位数和四分位数［M(P25, P75)］表示。分类变量采用频数(n)和构成比(%)描述。采用Wilcoxon秩和检验、Kruskal-Wallis H检验进行疾病严重程度影响因素的单因素分析，采用多元线性回归分析进行多因素分析。检验水准α=0.05。
2　结果
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2.1　调查对象的一般资料
本研究共调查既往确诊且经历过驱铜治疗的WD患者104人，其中1人未完整完成问卷调查，信息缺失过多被排除。最终纳入分析103人。男性44人 (42.7%)，女性59人 (57.3%)；年龄中位数为31.3 (25.1, 35.8)岁，年龄、发病年龄、病程范围分别为12.6~57.4岁、3.8~49.4岁、0.6~32.3年。
2.2　UWDRS量表评估特征
UWDRS量表评估结果显示，经历驱铜治疗的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)。发生频率超过50.0%的疾病症状或体征有9项，分别为左手快复轮替、左手指捏合、右手指捏合、流涎、右手快复轮替、步态异常、言语障碍、其他流血肿或血肿、书写能力障碍。见表1。
2.3　UWDRS量表评分的单因素分析
单因素分析结果显示，UWDRS评分在不同BMI、户籍类型、文化程度、职业、家庭人均月收入、首次确诊疾病分型、低铜饮食、社会支持之间差异有统计学意义(P<0.05)。UWDRS评分在不同性别、年龄、婚姻状况、家族史、发病年龄、病程、延迟诊断、延迟治疗、定期复查和最近6个月体育锻炼频率间差异无统计学意义(P>0.05)。见表2。
2.4　UWDRS量表评分影响因素的多因素分析
以UWDRS量表总评分为因变量，以单因素分析中具有统计学意义（P<0.05）的变量以及其他潜在的影响因素为自变量进行多元线性回归分析。使用逐步回归法筛选自变量。结果显示，文化程度、首次确诊疾病分型、低铜饮食、药物依从性和社会支持是UWDRS总评分的独立影响因素(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。见表3。
3　讨论
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本文中研究对象的UWDRS总评分中位数为27.0(11.5, 60.50)，均数为39.5(35.80)，平均水平高于Leinweber[10]、章悦[11]研究中的15.9(16.80)、37.33(24.81)。一项已知依从性WD患者的真实世界回顾性多国调查结果显示，UWDRS评分在服药完全依从患者和次优依从患者中分别为20.6(27.98)、39.8(43.12)[12]。本研究结果介于二者之间且与服药次优依从患者更为接近，这可能和本研究中患者服药依从性也不高有关。提示相比其他WD人群，本研究中患者病情更为严重。此外，本研究中超过50.0%患者存在左手快复轮替(64.1%)、左手指捏合(63.1%)、右手指捏合(61.2%)、流涎(59.2%)、右手快复轮替(59.2%)、步态异常(57.3%)、言语障碍(52.4%)、其他流血肿或血肿(52.4%)、书写能力障碍(50.5%)症状/体征。除步态异常和其他流血或血肿2项外，其余7项症状/体征的单项平均评分均大于1分，与既往研究结果相似[8]。然而以上症状患病率仍较高，表明WD患者神经症状发生后不易治愈，相关症状的治疗改善还有待进一步优化。
本研究多元线性回归分析结果显示，大学及以上患者UWDRS评分低于中学及以下患者。这可能和更高学历患者具有更好的健康意识和更高的社会经济水平，从而维持更好的健康状况有关。脑型患者UWDRS评分高于肝型患者，提示脑型患者的疾病严重程度高于肝型患者，这可能和脑型患者持续性的神经症状有关。饮食和饮水是人体摄入铜的主要途径[13]。目前，临床指南建议WD患者保持低铜饮食，以延迟WD症状出现和控制疾病进展[5]。但有学者提出，除贝类和肝脏两种食物外，限铜饮食对接受药物治疗的患者可能是不必要的，长期限铜饮食会影响患者的生活质量，诱发或加剧营养不良[14-15]。
在本研究中，与自我报告一直坚持低铜饮食和偶尔/不低铜饮食的患者相比，尽量坚持低铜饮食的患者疾病严重程度最轻。提示适度低铜饮食对于WD患者预后具有重要意义，过度限制或不限制饮食铜摄入均不利于WD患者的疾病改善；但由低铜饮食带来的获益与营养不良风险需进一步探讨。WD患者需要终生服药，不规律用药或停止服药可能导致铜继续在体内蓄积，引起症状加重[5]。本研究中患者药物依从性MMAS-8评分结果为0~6.75分，均低于7分，表明药物依从性并不理想，处于中等和差水平。牛飞[16]研究发现，家庭月收入、文化程度、服用药物种类、用药知识水平是WD患者药物依从性的独立影响因素，认为症状已经缓解自行停药是患者药物依从性差的主要原因。因此，临床诊疗过程中应加强WD患者的健康教育，制定方便患者执行的用药方案，提高用药依从性以保证出院后维持治疗的效果。
社会支持指来自家庭、朋友、团体等社会各方面给予的精神和物质上的帮助和支援,反映个人与社会联系的密切程度和质量[17]。本研究显示社会支持水平越高，UWDRS评分越低。刘艳华[18]对WD患者生存质量和社会支持的研究发现得到社会支持越多生存质量越好。提高社会支持有利于患者从社会关系中获取更多帮助和更好的身心体验，增强应对疾病信心，提高健康水平。
综上所述，本研究表明既往确诊且经驱铜治疗的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。在临床诊疗和疾病管理过程中应重点关注初诊为脑型、文化程度低、社会支持水平低、药物依从性差的患者，加强健康宣教，提高治疗依从性，改善治疗效果。此外，适度的低铜饮食对于WD维持治疗中疾病改善仍是具有重要意义的。
文献
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Li XY, Zhang YP, Guo XY.Social support and nursing care［J］. Foreign Medical Sciences, 2002, 21(12): 545-549. (In Chinese)
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刘艳华．肝豆状核变性患者生存质量与社会支持状况及其相关分析［J］．现代临床护理, 2013(3): 44-47.
Liu YH. Correlation between the Life quality and social supports in patients with hepatolenticular degeneration［J］. Modern Clinical Nursing, 2013(3): 44-47. (In Chinese)
2024年第51卷第22期
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doi: 10.20043/j.cnki.MPM.202408150
  • 接收时间：2024-08-12
  • 首发时间：2026-03-20
  • 出版时间：2024-11-25
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  • 收稿日期：2024-08-12
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    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
    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

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杨春霞，E-mail: ；
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2种不同金属材料的力学参数
科
Family		 属数
Number of
genus		 种数
Number of
species		 占总种数比例
Percentage of
total species (%)		 属
Genus		 种数
Number of
species		 占总种数比例
Percentage of total
species (%)
鹅膏菌科Amanitaceae 2 11 5.26 鹅膏菌属 Amanita 10 4.78
小菇科 Mycenaceae 2 12 5.74 丝盖伞属 Inocybe 5 2.39
多孔菌科 Polyporaceae 8 14 6.70 蜡蘑属 Laccaria 5 2.39
红菇科 Russulaceae 3 23 11.00 小皮伞属 Marasmius 6 2.87
小菇属 Mycena 11 5.26
光柄菇属 Pluteus 5 2.39
红菇属 Russula 17 8.13
栓菌属 Trametes 5 2.39
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