Variant CJD
Variant CJD
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变异型克雅氏病
作者：
Abigail B. Diack;M. Head;S. Mccutcheon;A. Boyle;R. Knight;J. Ironside;J. Manson;R. Will
自英国首次发现vCJD病例以来，已经过去了18年。从那时起，有很多关于vCJD如何影响人类健康的猜测。迄今为止，英国已有177例病例报告，全球11个不同国家有51例病例报告。由于确定疯牛病和vCJD是同一株病原体，我们还表明，英国和非英国的vCJD病例在小鼠的第一次传代中存在广泛的相似性。转基因小鼠研究表明，所有密码子129基因型都对vCJD易感，基因型可能影响疾病是以临床形式出现还是以无症状形式出现，首例潜在无症状vCJD感染病例在PRNP 129MV患者中的出现支持了这一点。根据输血作为传播途径的证据，我们已经确定，在vCJD羊模型中，所有血液成分和低白细胞的血液都具有传播疾病的能力。重要的是，我们最近发现，无症状感染且脾脏PrPSc沉积有限的PRNP 129MV患者血液受体很容易将疾病传播给小鼠，这表明在没有临床疾病的情况下可能发生外周感染。这与最近的附录调查（在对32441例病例的研究中发现了16个阳性附录）一起，强调了继续监测克雅氏病和维持现有控制措施以保护人类健康的重要性。
It is now 18 years since the first identification of a case of vCJD in the UK. Since that time, there has been much speculation over how vCJD might impact human health. To date there have been 177 case reports in the UK and a further 51 cases worldwide in 11 different countries. Since establishing that BSE and vCJD are of the same strain of agent, we have also shown that there is broad similarity between UK and non-UK vCJD cases on first passage to mice. Transgenic mouse studies have indicated that all codon 129 genotypes are susceptible to vCJD and that genotype may influence whether disease appears in a clinical or asymptomatic form, supported by the appearance of the first case of potential asymptomatic vCJD infection in a PRNP 129MV patient. Following evidence of blood transfusion as a route of transmission, we have ascertained that all blood components and leucoreduced blood in a sheep model of vCJD have the ability to transmit disease. Importantly, we recently established that a PRNP 129MV patient blood recipient with an asymptomatic infection and limited PrPSc deposition in the spleen could readily transmit disease into mice, demonstrating the potential for peripheral infection in the absence of clinical disease. This, along with the recent appendix survey which identified 16 positive appendices in a study of 32 441 cases, underlines the importance of continued CJD surveillance and maintaining control measures already in place to protect human health.