中国2010年克-雅病监测病例特征分析  

Surveillance of Creutzfeldt-Jakob disease in China,2010

作　　者：陈操[1] 石琦[1] 周伟[1] 张宝云[1] 高晨[1] 田婵[1] 韩俊[1] 董小平[1] 

机构地区：[1]中国疾病预防控制中心病毒病预防控制所/传染病预防控制国家重点实验室,北京102206

出　　处：《疾病监测》2011年第11期844-847,共4页Disease Surveillance

基　　金：传染病预防控制国家重点实验室基金(No.2008SKLID102;2011SKLID211);the SKLID development Grant;"十一五"国家科技重大专项(No.2009ZX10004-101);China Mega-Project for Infectious Disease~~

摘　　要：目的了解中国(未包括香港、澳门和台湾地区,下同)克-雅病(Creutzfeldt-Jakob disease,CJD)的发病情况、流行病学及临床特征。方法对2010年中国CJD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方法检测脑组织中PrPSc蛋白及脑脊液中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的分析。结果共发现散发型CJD确定诊断病例1例,临床诊断病例46例,疑似诊断病例25例,致死性家族型失眠症病例3例及家族型CJD病例5例(包括R208H、P102L各1例及3例T188K突变)。病例报告无季节聚集性,长久居住地呈散在分布,职业分布广泛。临床诊断病例平均年龄为62岁,男女性别比为1.30∶1;疑似诊断病例平均年龄为57岁,男女性别比为2.14∶1。快速进行性痴呆为最常见的首发症状,占全部诊断病例的54.93%。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 2010年中国监测到的CJD病例主要以散发型为主,病例的报告时间、长久居住地分布、职业分布、性别比例以及年龄分布均符合散发型CJD的特点。Objective To understand the incidence, epidemiological and clinical characteristics of Creutzfeldt-Jakob disease （CJD） in China. Methods The analysis was conducted on the clinical and epidemiological data of suspected CJD cases collected through the surveillance in China in 2010. Cerebrospinal fluid （CSF） and blood samples were taken from the patients to detect PrPsc in brain tissue and 14 - 3 - 3 protein in CSF by using Western blot assay and analyze the polymorphism of 129 amino acid and mutation of PRNP gene extracted from blood samples by using PCR and sequencing assay. Results Totally 1 laboratory confirmed cases, 46 clinical diagnosed and 25 suspected cases of CJD, 3 fatal familial insomnia cases （FFI） and 5 genetic CJD cases were detected, including 1 R208H, 1 P102L and 3 T188K mutation cases No seasonal, geographic and population clustering of the disease were observed. The mean age of clinical diagnosed patients was 62 years old, the male to female ratio was 1.30： 1, the mean age of suspected patients was 57 years old, the male to female ratio was 2. 14： 1. Rapidly progressive dementia was the mostly common symptom, occurring firstly in 54. 93 % of the CJD patients. More typical clinical manifestations were presented in clinical diagnosed patients than in suspected patients. Conclusion CJD occurred sporadically in China in 2010, which was demonstrated by the time, geographic, population, gender and age distributions of the cases.

关 键 词：克-雅病 监测 14—3—3蛋白 脚基因 

分 类 号：R742.9[医药卫生—神经病学与精神病学]