Brandel J-P, Salomon D, Alpérovitch A
Cellule nationale de référence des maladie de Creutzfeldt-Jakob, hôpital de la Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
Transfus Clin Biol. 2006 Nov;13(5):304-6. doi: 10.1016/j.tracli.2006.11.002. Epub 2006 Dec 22.
Variant Creutzfeldt-Jakob disease (vCJD) is the only form of prion diseases linked to bovine spongiform encephalopathy (BSE). The disease was first described in the United-Kingdom (UK) and France is the second affected country with 21 cases. Clinical, genetic and neuropathological features are the same in both countries. Comparison of the total number of cases in France and in the UK, according to dates of onset, shows that, in France, the maximum incidence seems to be five years delayed and that, in the UK, the number of vCJD cases regularly decreases since 1999. Delayed exposure to contaminated beef products in France compared to the UK could explain this temporal gap. Three cases of vCJD after transfusion of labile blood products were observed in the UK. No such of cases were observed in France but three patients developing signs of vCJD in 2004 were blood donors. A total of 42 recipients were identified with 17 recipients still alive.
变异型克雅氏病(vCJD)是唯一一种与牛海绵状脑病(BSE)相关的朊病毒疾病。该病最初在英国被发现,法国是第二个受影响的国家,有21例病例。两国的临床、遗传和神经病理学特征相同。根据发病日期对法国和英国的病例总数进行比较,结果显示,在法国,最高发病率似乎推迟了五年,而在英国,自1999年以来vCJD病例数一直在定期减少。与英国相比,法国接触受污染牛肉产品的时间较晚,这可能解释了这种时间上的差距。在英国观察到3例因输注不稳定血液制品而感染vCJD的病例。法国未观察到此类病例,但2004年有3名出现vCJD症状的患者曾是献血者。共确定了42名受血者,其中17名受血者仍在世。