2015年中国克雅氏病监测病例特征分析

发布时间：2018-04-22 23:40

本文选题：克雅氏病 + 监测　；参考：《疾病监测》2016年05期

【摘要】：目的了解中国克雅氏病(Creutzfeldt-Jakob disease,CJD)的发病情况、流行病学及临床特征。方法对2015年我国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分析。结果 2015年共监测病例366例,其中散发型CJD临床诊断病例134例,疑似诊断病例29例,致死性家族型失眠症病例4例,吉斯特曼-斯特劳斯综合征病例1例及家族型CJD病例17例,包括11例T188K、4例E200K、1例E196K及1例E196A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63岁,男女性别比为1.13∶1;疑似诊断病例平均年龄为60岁,男女性别比为1.64∶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36.81%。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 2015年我国监测到的CJD病例以散发型为主,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。
[Abstract]:Objective to investigate the prevalence, epidemiology and clinical characteristics of Creutzfeldt-Jakob disease (CJDD) in China. Methods the clinical and epidemiological data of suspected CJD cases obtained from China's CJD surveillance network in 2015 were analyzed. Cerebrospinal fluid (CSF) and blood samples were collected, and 14-3-3 protein in CSF was detected by Western Blot method. The genomic DNA of whole blood was extracted and the polymorphism and mutation of PRNP gene were analyzed by PCR and sequencing. Results there were 366 cases monitored in 2015, including 134 cases of sporadic CJD, 29 cases of suspected cases, 4 cases of fatal familial insomnia, 1 case of Gustman-Strauss syndrome and 17 cases of familial CJD. The mutation of E196K and E196A were found in 11 cases of T188KN, 4 cases of E200KN, 1 case of E196K and 1 case of E196A mutation. There was no seasonal agglomeration, long-term residence was scattered and occupational distribution was widespread. The average age of clinically diagnosed cases is 63 years, the ratio of male to female is 1.13: 1, and the average age of suspected cases is 60 years old, the ratio of male to female is 1.64: 1. Rapid progressive dementia is the most common initial symptom, accounting for 36.81% of all cases. There are more typical clinical manifestations in clinically diagnosed cases than in suspected ones. Conclusion sporadic CJD cases were detected in China in 2015. The reporting time, long-term residence distribution, occupation distribution, sex ratio and age distribution all accord with the characteristics of sporadic CJD.
【作者单位】：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;
【基金】：国家自然科学基金(No.81401670,81572048,81301429) 传染病预防控制国家重点实验室项目(No.2012SKLID102,2015SKLID503)~~
【分类号】：R742.9

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