Abstract:To analyze the epidemiology, clinical features and outcome of the patients with Creutzfeldt-Jakob diseases(CJD) in Anhui Province from 2009 to 2018. The epidemiology, clinical charcteristics and follow-up data of CJD suspected patients were obtained from Anhui CJD surveillance network and then analyzed combining the laboratory results from cerebrospinal fluid and blood samples. A total of 29 CJD cases were found from 2009 to 2018, including 17 clinically CJD cases, 9 suspected CJD and 3 genetic or familial Creutzfeldt-Jakob diseases. There is no seasonal clusterings in the case reports. The long-term residence is scattered and the occupations are widely distributed. Clinical data analysis showed that progressive dementia was the most common initial symptom, accounting for 65.52% (19/29); the median age of onset of sporadic CJD cases was 61.11 years old, and the ratio of male to female was 1.23∶1, all of them were Han nationality. Brain magnetic resonance imaging (MRI) abnormalities were the main auxiliary examinations (80.00%). The positive rate of 14-3-3 protein in cerebrospinal fluid was 37.93%. The 129 amino acid polymorphisms detected by PRNP in blood samples were all M/M type. There were 3 mutations related to genotype, specifically 1 mutation site of T188K, 1 mutation site of D178N and 1 mutation site of R208H. The epidemiological characteristics of CJD cases in Anhui Province from 2009 to 2018 were consistent with the national surveillance situation in the same period. The first case of CJD with R208H mutation was first found in Anhui.
李青, 史永林. 2009-2018年安徽省克-雅病病例监测特征分析[J]. 中国人兽共患病学报, 2020, 36(2): 130-133.
LI Qing, SHI Yong-lin. Characteristics of Creutzfeldt-Jakob disease cases in Anhui Province,China, 2009-2018. Chinese Journal of Zoonoses, 2020, 36(2): 130-133.
[1] 陈操,周伟,张宝云,等.头颅磁共振成像在克雅氏病监测中的特点分析[J].疾病监测,2016,31(5):370-373. DOI:10.3784/ j.issn.1003-9961.2016.05.006 [2] 何桂香.5例散发型克雅病临床诊断病例的特征研究[J].中国病毒病杂志,2018,8(2):151-155. [3] Shi Q,Zhang XC,Zhou W, et al.Analysis of the advantage features of Beijing surveillance network for Creutzfeldt-Jakob disease[J].Prion,2015:9(4):304-314.DOI:10.1080/19336896.2015.1075115 [4] WS/T 562-2017,克-雅病诊断[S]. 北京:中国标准出版社,2017. [5] 高晨,韩俊,周伟,等.2006年1-8月份我国克雅病监测病例分析[J].中华实验和临床病毒学杂志,2007,21(3):205-207. DOI: 10.3760/cma.j.issn.1003-9279.2007.03.004 [6] WHO. World Health Organization Communicable Disease surveillance and Response[R].Geneva:WHO,2003: 71-72. [7] Chen C,Wang JC,Shi Q, et al.Analyses of the survival time and the influencing factors of chinese patients with prion diseases based on the surveillance data from 2008-2011[J]. PLoS One,2013,8(5):e62553.DOI: 10.1371/journal.pone.0062553 [8] Nagoshi K,Sadakane A,Nakamura Y,et al.Duration of prion disease is longer in Japan than in other countries[J].J Epidemiol,2011,21(4):255-262. [9] Collins SJ,Sanchez-Juan P,Masters CL, et al.Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease[J].Brain,2006,129(Pt9):2278-2287. DOI:10.1093/brain/awl159 [10] 王晶,周伟,肖康,等.2017年中国克-雅病监测网络病例特征分析[J].疾病监测, 2019,34(3):226-231.DOI: CNKI:SUN:JBJC.0.2019-02-005 [11] 边宏伟,蒋龙,谢宝钦.克雅氏病与新变异型克雅氏病[J].山东精神医学,2006,19(13):231-232.DOI: 10.3969/j.issn.1009-7201.2006.03.034 [12] 陈操,周伟,张宝云,等.2015年中国克雅氏病监测病例特征分析[J].疾病监测,2016,31(5):365-369. DOI:10.3784/j.issn.1003-9961.2012.12.003 [13] 董建华,王丽,史伟,等.2011-2015年陕西省克雅氏病监测病例特征分析[J].中国人兽共患病学报,2017,33(11):1013-1017. DOI:10.3969/j.issn.1002-2694.2017.11.012 [14] 李青,王俊,胡兴强,等.安徽省21例克-雅氏病病例分析[J].安徽预防医学杂志,2012,18(2):82-86.DOI: CNKI:SUN:AHYF.0.2012-02-003 [15] Shi Q,Zhou W,Chen C, et al.The features of genetic prion diseases based on Chinese surveillance program[J]. PLoS One,2015,10(10):e0139552.DOI:10.1371/journal.pone.0139552