2011-2015年陕西省克雅氏病监测病例特征分析  

Characteristics of Creutzfeldt-Jakob disease cases in Shaanxi Province,China,2011-2015

作　　者：董建华[1] 王丽[1] 史伟[1] 李慎[1] 魏菁[1] 郑媛[1] 余鹏博[1] 梦遥 徐艺[1] 

机构地区：[1]陕西省疾病预防控制中心病毒病预防控制所,西安710054

出　　处：《中国人兽共患病学报》2017年第11期1013-1017,共5页Chinese Journal of Zoonoses

基　　金："十二.五"国家科技重大专项(No.2013ZX10004202-001-002)~~

摘　　要：目的了解陕西省克雅氏病(Creutzfeldt-Jakob disease,CJD)的发病情况、临床表现及流行病学特征,为科学防控CJD提供依据。方法采集了2011-2015年陕西省7家医院报告的49例可疑CJD病例的临床及流行病学信息,收集了患者的血液及脑脊液样本。对脑脊液样本,通过免疫印迹法检测14-3-3蛋白。对血液样本,提取核酸,通过聚合酶链反应,扩增朊蛋白基因;利用测序技术,测定其突变情况,同时对129位和219位氨基酸进行多态性分析。结果确定散发型CJD临床诊断病例16例,散发型CJD疑似诊断病例4例,遗传型CJD 1例。病例的地理分布和职业无明显聚集性;散发型CJD临床诊断病例发病年龄中位数为62岁,男女比例1.29∶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47.62%。结论陕西监测到的CJD病例主要为散发型CJD,收集到的病例的年龄、性别比例、职业情况及住址分布等均符合散发型CJD的分布特点。We investigated the epidemiological and clinical characteristics,to provide evidence for the control and prevention of Creutzfeldt-Jakob disease(CJD)in Shaanxi Province,China.Clinical and epidemiological datas on 49 suspicious CJD patients from 7 hospitals in Shaanxi Province from 2011 to 2015 was collected.Blood and cerebral spinal fluid(CSF)specimens from the cases were gathered.For CSF sample,14-3-3 protein were tested by Western blot.For blood sample,PCR and sequencing were used,and then the mutation of PRNPgene and the polymorphism of 129 and 219 amino acids were analyzed.It showed that a total number of 16 probable and 4 possible sporadic CJD patients,and 1 familial CJD case were identified.Among these cases,neither geographic nor occupational-related ones were found.The median age of onset for the probable sporadic CJD cases was 62 years old,and the gender ratio of male to female was 1.29 to 1.The most initial symptom was rapid progressive dementia,which accounted for 47.62% of the CJD patients.This report indicates that the main type of CJD in Shaanxi Province is sporadic CJD with its distinctive characteristics including geography distribution,occupation,gender ratio and age of onset.

关 键 词：克雅氏病 监测 14-3-3蛋白 朊病毒 朊病毒基因 

分 类 号：R349.6[医药卫生—基础医学] R181