Annotation: vCJD - predicting the future?
Hilton DA
Department of Histopathology, Derriford Hospital, Plymouth, UK.
[Medline record in process]
近来英国变异型克雅氏病的紧急情况以及证明了变异型克雅氏病的病因就是引起牛海绵状脑病的朊病毒菌株这两件事使大家更加关心是不是在人类中也有这种疾病流行的可能。虽然目前只有79个变异型克雅氏病病例发生,但是很有可能在80年代末期和90年代早期有成千上万的受感染的牛进入人类食物链,而且变异型克雅氏病的平均潜伏期还不为所知。数学模型还不能对今后有多少病历发生进行有效的预测,而且缺乏变异型克雅氏病的血液测试,现在想减少将来该病的发病数量只能依靠朊病毒在淋巴网状组织中的积累了。到目前为止,在所有变异型克雅氏病病例中都观察到了广泛的淋巴网状组织中PrPSc积聚,在一个病例中早在症状发生前8个月就已经出现了积聚现象。朊病毒病动物模型说明早在潜伏期之前淋巴网状组织就已经受到牵涉,以此为据可以可靠的预测将来的神经病变。以这些数据为基础,在手术切除的扁桃体和阑尾标本中开展了大规模寻找PrP积聚的研究。首次检测的3000个标本中没有发现阳性标本,但现在明显的阴性结果是不可靠的。我们预测当研究结束的时候,可以获得更多的数据说明PrP在淋巴网状组织中出现会有多早,随着进一步样本检测的结果我们才可能对人类发生大规模变异型克雅氏病流行的可能性提出一些意见。
The recent emergence of variant Creutzfeldt-Jakob disease (vCJD) in the UK, and demonstration that vCJD is caused by the same prion strain that causes bovine spongiform encephalopathy, have led to concerns about the possibility of a human epidemic. Although only 79 cases of vCJD have occurred to date, it is likely that hundreds of thousands of infected cattle entered the human food chain in the late 1980s and early 1990s, and the average incubation period of vCJD is unknown. Mathematical models have not yet been able to give useful predictions of future numbers of cases, and in the absence of a blood test for vCJD, current attempts to reduce uncertainties about future numbers of cases are based on the accumulation of PrPSc in lymphoreticular tissues. Extensive lymphoreticular PrPSc accumulation has been seen in all cases of symptomatic vCJD so far examined, and in one case 8 months prior to the onset of symptoms. Animal models of prion disease suggest that lymphoreticular involvement occurs early in the incubation period and reliably predicts future neurological disease. Based on these data, large scale anonymous studies looking for PrP accumulation in surgically removed tonsillectomy and appendicectomy specimens are underway. Examination of the first 3000 specimens has not revealed any positive samples, but at the moment the significance of negative findings is uncertain. It is anticipated that by the time these studies are complete more data will be available on how early PrP can be demonstrated in lymphoreticular tissue in vCJD, which together with the results from examination of further samples, will allow some comment as to the likelihood of a large human vCJD epidemic.
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