■人类朊病毒病(PrD),一组致命和可传播的神经退行性疾病,包括克雅氏病(CJD),库鲁,致命的家族性失眠(FFI),Gerstmann-Sträussler-Scheinker病(GSS),和可变蛋白酶敏感的prionopathy(VPSPr)。牛海绵状脑病(BSE)和变异型CJD(vCJD)的出现极大地威胁了公众健康,无论是人类还是动物。自1990年代以来,数十个国家和地区开展了PrD监视计划。
■在这项研究中,从1993年到2020年,全球和不同国家或地区不同类型的PRD的病例数量和替代趋势是根据国际和国家PRD监测计划网站的数据进行收集和分析的,以及相关出版物。
■在34个国家/地区报告的PrD和零星CJD(sCJD)病例总数分别为27,872和24,623。PrD病例最多的7个国家是美国(n=5,156),法国(n=3,276),德国(n=3,212),意大利(n=2,995),中国(n=2,662),英国(n=2,521),西班牙(n=1,657),加拿大(n=1,311)。每年的PrD病例数和死亡率,无论是在全球还是在各国,在过去的27年中显示出增长趋势。遗传性PrD病例占所有报告的PrD病例的10.83%;然而,不同国家和地区之间的趋势差异很大。全球报告有485例医源性CJD(iCJD)病例和232例vCJD病例。
■拥有PrD监测项目的国家大多是高收入和中高收入国家。然而,世界上大多数低收入和中低收入国家没有进行PRD监测,甚至没有报告PRD病例,这表明全球人类PrD病例的数量明显被低估。从公共卫生的角度来看,对人类和动物进行积极的国际PrD监测对于消除pr病毒疾病的威胁仍然至关重要。
UNASSIGNED: Human prion disease (PrD), a group of fatal and transmissible neurodegenerative diseases, consists of Creutzfeldt-Jakob disease (CJD), kuru, fatal familial insomnia (FFI), Gerstmann-Sträussler-Scheinker disease (GSS), and variably protease-sensitive prionopathy (VPSPr). The emergence of bovine spongiform encephalopathy (BSE) in
cattle and variant CJD (vCJD) has greatly threatened public health, both in humans and animals. Since the 1990\'s, dozens of countries and territories have conducted PrD surveillance programs.
UNASSIGNED: In this study, the case numbers and alternative trends of different types of PrD globally and in various countries or territories from 1993 to 2020 were collected and analyzed based on the data from the websites of the international and national PrD surveillance programs, as well as from relevant publications.
UNASSIGNED: The total numbers of the reported PrD and sporadic CJD (sCJD) cases in 34 countries with accessible annual case numbers were 27,872 and 24,623, respectively. The top seven countries in PrD cases were the USA (n = 5,156), France (n = 3,276), Germany (n = 3,212), Italy (n = 2,995), China (n = 2,662), the UK (n = 2,521), Spain (n = 1,657), and Canada (n = 1,311). The annual PrD case numbers and mortalities, either globally or in the countries, showed an increased trend in the past 27 years. Genetic PrD cases accounted for 10.83% of all reported PrD cases; however, the trend varied largely among the different countries and territories. There have been 485 iatrogenic CJD (iCJD) cases and 232 vCJD cases reported worldwide.
UNASSIGNED: The majority of the countries with PrD surveillance programs were high- and upper-middle-income countries. However, most low- and lower-middle-income countries in the world did not conduct PrD surveillance or even report PrD cases, indicating that the number of human PrD cases worldwide is markedly undervalued. Active international PrD surveillance for both humans and animals is still vital to eliminate the threat of prion disease from a public health perspective.