2006-2010年陕西省克雅氏病监测病例特征分析
发布时间：2018-11-29 07:48
【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。方法对2006-2010年陕西省临床医院报告的42例可疑CJD病例的临床及流行病学资料进行分析,收集了42份血液标本、41份脑脊液及1份脑组织样品,利用免疫印迹(western blot,WB)方法检测脑组织的异常折叠朊蛋白(prion protein scrapie,Pr PSc)和脑脊液的14-3-3蛋白,提取全血基因组DNA并利用聚合酶链式反应(polymerase chain reaction,PCR)及测序方法对血液中朊蛋白(Prion protein,PRNP或Pr P)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18例,疑似诊断病例3例,家族型CJD 2例。病例的地理分布和职业无明显聚集性;临床诊断病例发病平均年龄55.9岁,男女比例1.25∶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34.78%。结论陕西监测到的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。随访有助于CJD正确诊断。
[Abstract]:Objective to investigate the incidence, clinical manifestations and epidemiological characteristics of Creutzfeldt-Jakob disease (Creutzfeldt-Jakob disease,CJD) in Shaanxi Province. Methods the clinical and epidemiological data of 42 suspected CJD cases reported by Shaanxi Provincial Clinical Hospital from 2006 to 2010 were analyzed. 42 blood samples, 41 cerebrospinal fluid samples and 1 brain tissue sample were collected. Western blot (western blot, was used. WB) was used to detect the abnormal folding prion protein (prion protein scrapie,Pr PSc) in brain tissue and 14-3-3 protein in cerebrospinal fluid (CSF). Genomic DNA was extracted from whole blood, and polymerase chain reaction (polymerase chain reaction,) was used. PCR and sequencing methods were used to analyze the polymorphism and mutation of prion protein (Prion protein,PRNP or Pr P) gene) in blood. Results 18 cases of sporadic CJD, 3 cases of suspected cases and 2 cases of familial CJD were found. The average age of clinical diagnosis was 55.9 years old, the ratio of male to female was 1.25: 1. Rapid progressive dementia was the most common initial symptom, accounting for 34.78% of all cases. Conclusion sporadic type is the main type of CJD in Shaanxi Province. The geographical distribution, occupation, sex ratio and average age of the cases accord with the distribution characteristics of sporadic CJD. Follow-up is helpful for the correct diagnosis of CJD.
【作者单位】： 陕西省疾病预防控制中心病毒病预防控制所;
【基金】：“十二五”国家科技重大专项(2013ZX10004202-001-002)
【分类号】：R742.9
，
本文编号：2364378
[Abstract]:Objective to investigate the incidence, clinical manifestations and epidemiological characteristics of Creutzfeldt-Jakob disease (Creutzfeldt-Jakob disease,CJD) in Shaanxi Province. Methods the clinical and epidemiological data of 42 suspected CJD cases reported by Shaanxi Provincial Clinical Hospital from 2006 to 2010 were analyzed. 42 blood samples, 41 cerebrospinal fluid samples and 1 brain tissue sample were collected. Western blot (western blot, was used. WB) was used to detect the abnormal folding prion protein (prion protein scrapie,Pr PSc) in brain tissue and 14-3-3 protein in cerebrospinal fluid (CSF). Genomic DNA was extracted from whole blood, and polymerase chain reaction (polymerase chain reaction,) was used. PCR and sequencing methods were used to analyze the polymorphism and mutation of prion protein (Prion protein,PRNP or Pr P) gene) in blood. Results 18 cases of sporadic CJD, 3 cases of suspected cases and 2 cases of familial CJD were found. The average age of clinical diagnosis was 55.9 years old, the ratio of male to female was 1.25: 1. Rapid progressive dementia was the most common initial symptom, accounting for 34.78% of all cases. Conclusion sporadic type is the main type of CJD in Shaanxi Province. The geographical distribution, occupation, sex ratio and average age of the cases accord with the distribution characteristics of sporadic CJD. Follow-up is helpful for the correct diagnosis of CJD.
【作者单位】： 陕西省疾病预防控制中心病毒病预防控制所;
【基金】：“十二五”国家科技重大专项(2013ZX10004202-001-002)
【分类号】：R742.9
，
本文编号：2364378
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