Coulthart M B, Jansen G H, Connolly T, D'Amour R, Kruse J, Lynch J, Sabourin S, Wang Z, Giulivi A, Ricketts M N, Cashman N R
Canadian Creutzfeldt-Jakob Disease Surveillance System, Centre for Foodborne, Environmental and Zoonotic Infectious Diseases, Public Health Agency of Canada, Ottawa, ON.
The Ottawa Hospital, University of Ottawa and Eastern Ontario Regional Laboratory Association, Ottawa, ON.
Can Commun Dis Rep. 2015 Aug 6;41(8):182-191. doi: 10.14745/ccdr.v41i08a01.
Human prion diseases, known collectively as Creutzfeldt-Jakob disease (CJD), are fatal, infectious neurodegenerative disorders that occur in all human populations.
To summarize national surveillance data for CJD in Canada between January 1, 1998, and December 31, 2013.
Detailed investigations were conducted of individual suspected CJD cases, with collaboration between Canadian health professionals and investigators affiliated with a central CJD surveillance registry operated by the Public Health Agency of Canada. Data were collected on the clinical profile, family history, and results of paraclinical and laboratory investigations, including post-mortem neuropathological examination.
A total of 662 deaths from definite and probable CJD were identified in Canadian residents during the study period, comprising 613 cases of sporadic CJD (92.6%), 43 cases of genetic prion disease (6.5%), 4 cases of iatrogenic CJD (0.6%), and 2 cases of variant CJD disease (0.3%). The overall crude mortality rate for sporadic CJD was 1.18 per million per year [95% confidence interval (CI): 1.08,1.27]. Age-specific rates ranged from 0.05 [95% CI: 0.03,0.08] in persons under 50 years of age to 7.11 [95% CI: 6.20,8.11] in those aged 70 to 79. A significant net upward trend in age-adjusted rates was observed over the study period. Standardized mortality ratios, calculated for 10 individual Canadian provinces with reference to national average mortality rates, did not differ significantly from 1.0.
Creutzfeldt-Jakob disease remains rare in Canada, although mortality rates vary by two orders of magnitude between older and younger age groups. The upward trend in age-standardized sporadic CJD mortality rate over the study period can be better accounted for by gradually improving case ascertainment than by a real increase in incidence.
人类朊病毒病统称为克雅氏病（CJD），是一种发生在所有人群中的致命性传染性神经退行性疾病。
总结1998年1月1日至2013年12月31日期间加拿大克雅氏病的国家监测数据。
对每例疑似克雅氏病病例进行详细调查，加拿大卫生专业人员与隶属于加拿大公共卫生署运营的中央克雅氏病监测登记处的调查人员合作。收集了临床特征、家族史以及临床旁和实验室检查结果的数据，包括尸检神经病理学检查。
在研究期间，加拿大居民中共有662例确诊和疑似克雅氏病死亡病例，其中包括613例散发性克雅氏病（92.6%）、43例遗传性朊病毒病（6.5%）、4例医源性克雅氏病（0.6%）和2例变异型克雅氏病（0.3%）。散发性克雅氏病的总体粗死亡率为每年百万分之1.18 [95%置信区间（CI）：1.08, 1.27]。年龄别死亡率范围从50岁以下人群的0.05 [95% CI：0.03, 0.08]到70至79岁人群的7.11 [95% CI：6.20, 8.11]。在研究期间观察到年龄调整率有显著的净上升趋势。根据全国平均死亡率计算的10个加拿大省份的标准化死亡比与1.0无显著差异。
克雅氏病在加拿大仍然罕见，尽管老年和年轻年龄组之间的死亡率相差两个数量级。研究期间年龄标准化散发性克雅氏病死亡率的上升趋势，通过逐步改善病例确诊情况比实际发病率增加能得到更好的解释。
