van Duijn C M, Delasnerie-Lauprêtre N, Masullo C, Zerr I, de Silva R, Wientjens D P, Brandel J P, Weber T, Bonavita V, Zeidler M, Alpérovitch A, Poser S, Granieri E, Hofman A, Will R G
Department of Epidemiology & Biostatistics, Erasmus University Medical School, Rotterdam, The Netherlands.
Lancet. 1998 Apr 11;351(9109):1081-5. doi: 10.1016/s0140-6736(97)09468-3.
Creutzfeldt-Jakob disease (CJD) is a transmissible spongiform encephalopathy. Genetic and iatrogenic forms have been recognised but most are sporadic and of unknown cause. We have studied risk factors for CJD as part of the 1993-95 European Union collaborative studies of CJD in Europe.
The 405 patients with definite or probable CJD who took part in our study had taken part in population-based studies done between 1993 and 1995 in Belgium, France, Germany, Italy, the Netherlands, and the UK. Data on putative risk factors from these patients were compared with data from 405 controls.
We found evidence for familial aggregation of CJD with dementia due to causes other than CJD (relative risk [RR] 2.26, 95% CI 1.31-3.90). No significant increased risk of CJD in relation to a history of surgery and blood transfusion was shown. There was no evidence for an association between the risk of CJD and the consumption of beef, veal, lamb, cheese, or milk. No association was found with occupational exposure to animals or leather. The few positive findings of the study include increased risk in relation to consumption of raw meat (RR 1.63 [95% CI 1.18-2.23]) and brain (1.68 [1.18-2.39]), frequent exposure to leather products (1.94 [1.13-3.33]), and exposure to fertiliser consisting of hoofs and horns (2.32 [1.38-2.91]). Additional analyses, for example stratification by country and of exposures pre-1985 and post-1985, suggest that these results should be interpreted with great caution.
Within the limits of the retrospective design of the study, our findings suggest that genetic factors other than the known CJD mutations may play an important part in CJD. Iatrogenic transmission of disease seems rare in this large population-based sample of patients with CJD. There is little evidence for an association between the risk of CJD and either animal exposure, or consumption of processed bovine meat or milk products for the period studied.
克雅氏病（CJD）是一种可传播的海绵状脑病。已认识到遗传型和医源性形式，但大多数为散发性且病因不明。作为1993 - 1995年欧盟在欧洲开展的克雅氏病协作研究的一部分，我们研究了克雅氏病的危险因素。
参与我们研究的405例确诊或疑似克雅氏病患者参加了1993年至1995年在比利时、法国、德国、意大利、荷兰和英国开展的基于人群的研究。将这些患者的假定危险因素数据与405例对照的数据进行比较。
我们发现克雅氏病与非克雅氏病所致痴呆存在家族聚集性证据（相对危险度[RR] 2.26，95%可信区间1.31 - 3.90）。未显示手术史和输血史与克雅氏病风险显著增加有关。没有证据表明克雅氏病风险与牛肉、小牛肉、羊肉、奶酪或牛奶的消费之间存在关联。未发现与职业性接触动物或皮革有关联。该研究的少数阳性发现包括与食用生肉（RR 1.63 [95%可信区间1.18 - 2.23]）和脑（1.68 [1.18 - 2.39]）、频繁接触皮革制品（1.94 [1.13 - 3.33]）以及接触由蹄和角制成的肥料（2.32 [1.38 - 2.91]）有关的风险增加。进一步分析，例如按国家分层以及对1985年前和1985年后的暴露情况进行分析，表明这些结果应极为谨慎地解释。
在该研究回顾性设计的局限性内，我们的发现表明，除已知的克雅氏病突变外，遗传因素可能在克雅氏病中起重要作用。在这个基于大量人群的克雅氏病患者样本中，医源性疾病传播似乎很少见。在所研究的时期内，几乎没有证据表明克雅氏病风险与动物接触、加工牛肉或奶制品消费之间存在关联。
Zotero 插件