| 田婵,高晨,石琦,韩俊,周伟,张宝云,高永军,董小平.中国2008年克雅氏病监测病例特征分析[J].Chinese journal of Epidemiology,2009,30(7):713-715 |
| 中国2008年克雅氏病监测病例特征分析 |
| Study on the characteristics of patients with Creutzfeidt.Jakob disease under 2008 surveillance data in China |
| Received:February 11, 2009 |
| DOI：10.3760/cma.j.issn.0254-6450.2009.07.017 |
| KeyWord: 克雅氏病 监测 14—3—3蛋白 PRNP基因 |
| English Key Word: Creutzfeldt-Jakob disease Surveillance 14-3-3 protein PRNP gene |
| FundProject:国家科技攻关计划(2006BAD06A13.2);国家“973”项目(2007CB310505);国家自然科学基金(30771914、30800975);科研院所基金项目(2008EGl50300) |
| Author Name | Affiliation | E-mail | | TIAN Chan | State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prvention，Chinese Center for Disease Control and Prevention，beijing,100052，China | | | GA0 Chen | State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prvention，Chinese Center for Disease Control and Prevention，beijing,100052，China | | | SHI Qi | State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prvention，Chinese Center for Disease Control and Prevention，beijing,100052，China | | | HAN Jun | State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prvention，Chinese Center for Disease Control and Prevention，beijing,100052，China | | | ZHOU Wei | State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prvention，Chinese Center for Disease Control and Prevention，beijing,100052，China | | | ZHANG Baoyun | State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prvention，Chinese Center for Disease Control and Prevention，beijing,100052，China | | | GAO Yongjun | State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prvention，Chinese Center for Disease Control and Prevention，beijing,100052，China | | | DONG Xiaoping | State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prvention，Chinese Center for Disease Control and Prevention，beijing,100052，China | dongxp238@sina.com |
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| Abstract: |
| 目的 了解中国克雅氏病(CJD)的发病情况及流行病学、临床特征。方法 对2008年中国克雅氏病监测网络获得的可疑CJD病例的临床及流行病学资料进行分析, 收集患者脑脊液及血液样品, 利用Western blot方法 检测脑脊液中是否含有14—3—3蛋白, 提取全血基因组DNA并利用PCR及测序方法 对PRNP基因进行129位多态性及基因突变分析。结果 共发现散发型CJD临床诊断病例31例, 疑似诊断病例11例, 2例家族性CJD和l例格一斯一斯综合征。病例的地理分布及职业无明显聚集性;临床诊断病例发病平均年龄56.7岁, 男女比例8：9;疑似诊断病例发病平均年龄为57.4岁, 男女比例5：6。快速进行性痴呆为最常见的首发症状, 占全部诊断病例的33.3％。临床诊断病例比疑似诊断病例出现更多的典型临床表现。结论 2008年中国监测到的CJD病例以散发型为主, 病例的地理分布、职业、性别比例以及平均年龄均符合散发型CJD的分布特点。 |
| English Abstract: |
| Objective To describe the epidemiol logical and lclinica characteristics of Creutzfeldt-Jakobdisease CJD in China Methods Clinica and epidemiea data on patients from China CJD surveillance network was analyzed.Blood and cerebral spinal fluid(CSF)specimens from these patients were collected.Westem blot assay was used to detect 14-3-3 protein in CSF PCR and sequencing aSSay were used for analyzing the polymorohism of 129 amino acid and mutation of PRNP gene.Results A total number of 3 l probable and ll possible sporadic CJD patients were identified. Additionally.one patient with Gerstmann。Straussler-Scheinker syndrome(GSS)and 2 familial CJD cases were identified.No geographic.or occupational.related events were observed among these cases.The mean age of onset on the probable or possible CJD patients were 56.7 and 57.4 years old.with sex ratios of the probable CJD patients as 8：9 and the possible one as 5：6 respectively.Rapid progressive dementia was the main foremost symptom.presenting in 33.3%of the CJDpatients.Probable CJD patients showed more elinical manifestations than those possible ones.Conclusion Geography distribution,occupation, ratio of gender and the mean onset age of the CJD cases in 2008 were consistent with the charaeteristies of the sporadic CJD. |
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