Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada
Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada
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DOI：
10.1212/01.wnl.0000160117.56690.b2
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发表时间：
2005-05-10
期刊：
影响因子：
9.9
通讯作者：
Zerr, I
中科院分区：
文献类型：
--
作者：
Ladogana, A;Puopolo, M;Zerr, I
背景资料：1993年建立了一项关于克雅氏病（CJD）流行病学特征的国际研究，包括法国、德国、意大利、荷兰、斯洛伐克和英国的国家登记处。1997年，这项研究扩展到澳大利亚、奥地利、加拿大、西班牙和瑞士。研究方法：收集了所有参与国家1993年至2002年的数据，包括所有病因亚型的确诊或疑似CJD死亡病例。结果如下：共有4441例病例可供分析，其中包括3,720例散发性CJD病例、455例遗传性病例、138例医源性病例和128例变异病例。1999年至2002年间，所有病例的总体年死亡率为每百万人1.67例，散发性克雅氏病的年死亡率为每百万人1.39例。所有国家的死亡率相似。病例按病因亚型的分布存在异质性，意大利和斯洛伐克有过多的遗传病例，法国和英国有过多的医源性病例，英国有过多的变异型克雅氏病。结论：本研究在一项以多国人群为基础的研究中确立了所有类型克雅氏病（CJD）的总体流行病学特征。国家间的比较并没有表明在英国散发性CJD的特征有任何相对变化，本研究的证据也没有表明除了变异型CJD之外，还发生了一种新的人牛海绵状脑病感染。然而，这仍然是一种可能性，目前未受变异型克雅氏病影响的国家可能仍有病例。
Background: An international study of the epidemiologic characteristics of Creutzfeldt-Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland. Methods: Data were pooled from all participating countries for the years 1993 to 2002 and included deaths from definite or probable CJD of all etiologic subtypes. Results: Four thousand four hundred forty-one cases were available for analysis and included 3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and 128 variant cases. The overall annual mortality rate between 1999 and 2002 was 1.67 per million for all cases and 1.39 per million for sporadic CJD. Mortality rates were similar in all countries. There was heterogeneity in the distribution of cases by etiologic subtype with an excess of genetic cases in Italy and Slovakia, of iatrogenic cases in France and the UK, and of variant CJD in the UK. Conclusions: This study has established overall epidemiologic characteristics for Creutzfeldt-Jakob disease (CJD) of all types in a multinational population-based study. Intercountry comparisons did not suggest any relative change in the characteristics of sporadic CJD in the United Kingdom, and the evidence in this study does not suggest the occurrence of a novel form of human bovine spongiform encephalopathy infection other than variant CJD. However, this remains a possibility, and countries currently unaffected by variant CJD may yet have cases.