Wilson病的临床特征及早期康复干预效应  

Clinical features of Wilson disease and the effect of early rehabilitation intervention

作　　者：杨文明[1] 陈彪[1] 

机构地区：[1]首都医科大学宣武医院神经内科,北京市100053

出　　处：《中国临床康复》2005年第7期139-141,共3页Chinese Journal of Clinical Rehabilitation

基　　金：国家科技攻关计划资助项目(2002BA711A07);北京市卫生重点学科基金;安徽省优秀青年科技基金;安徽省高校学科拔尖人才基金;安徽省人才开发基金(2003Z026);安徽省自然科学基金(99044591)~~

摘　　要：目的:目前Wilson病的误诊及漏诊率仍较高,提高对Wilson病临床诊断特征的认识是早期诊断、降低Wilson病的误诊及漏诊率的重要举措,同时也为早期康复干预提供条件。资料来源:应用计算机检索Medline1990-01/2004-04和Embase1990-08/2004-04的相关文章,检索词“Wilsondisease,hepatolenticu-lardegeneration,diagnosis,feature,earlyintervention,pharmacology,reha-bilitation,并限定文章语言种类为English。资料选择:对资料进行初审,并查找全文,进行归纳分析总结。纳入标准:①随机对照试验(RCT),无论是否为单盲,双盲或非盲法。②平行对照试验。③无对照的临床治疗研究。④临床诊断研究。排除标准:个案研究,综述文献。资料提炼:共收集到122篇关于Wilson病临床特征及其早期干预的论文,排除的11篇个案报道研究。资料综合:对患者发病年龄、临床表现以及实验诊断指标进行评价,并对以药物治疗为主的早期干预进行总结。结论:Wilson病临床的一些特征性表现,对确定诊断有重要价值,早期干预可延缓或阻止Wilson病症状的发生或发展,减轻功能障碍,有利于患者的康复。OBJECTIVE:At present, rates of misdiagnosis and missed diagnosis about Wilson disease(WD) are still high. Improving the identification of the early clinical features is the important method to decrease the rates of misdiagnosis and missed diagnosis about Wilson disease and make early diagnosis.Meanwhile, it could provide condition for early intervention. DATA SOURCES:Using the key terms“Wilson's disease,hepatolenticular degeneration,diagnosis,feature,early intervention,pharmacology, rehabilitation”,we searched the MEDLINE database between January 1900 and April 2004 and the EMBASE database between August 1900 and April 2004 in English. STUDY SELECTION:All articles were selected firstly,and the full text was looked up and summarized and analyzed.The selected criteria included: ①randomized controlled trial(RCT),no matter it was single-blind,double-blind or non-blind;②parallel controlled trial;③study of the clinical treatment;④study of the clinical diagnosis.Excluded criteria: individual case reports and summarized literature. DATA EXTRACTION:Totally 122 articles about clinical features and early intervention were collected and 11 articles of individual case reported were excluded. DATA SYNTHESIS:The age of onset of illness,clinical manifestations and experimental diagnostic indexes of WD patients were evaluated and early intervention with drugs were summarized. CONCLUSION:The clinical manifestations of WD have very important value in definitive diagnosis. Early intervention may delay and prevent occurrence and development of clinical symptoms of WD,relieve functional disturbance and also can accelerate rehabilitation of patients.

关 键 词：肝豆状核变性 康复 综述文献 

分 类 号：R72[医药卫生—儿科]