克雅二氏病研究与诊断进展

Progress in the study on and diagnosis of Creutzfeldt-Jakob Disease(CJD)

克雅二氏病(Creutzfeldt-Jakob disease,CJD)是朊粒(prion)类疾病在人类中最常见的病种,与其他常见的传染病不同的是,这个疾病不是由类似于含有基因的病毒或细菌的病原造成的疾病,而是由变构蛋白朊粒直接造成的疾病。尽管如此,它的公共健康的意义很大。因为朊粒在常规消毒情况下是不被灭活的。这类患者的朊粒会通过输血、手术或其他途径把病原传给他人,加上疾病发病的潜伏期长,很难马上被发现,给临床诊断造成很大困难。目前诊断主要限于临床表现及晚期的影像检查和组织学检查,特异性也比较低。早期和主动的、特异性的诊断方法一直是此领域的热点和亟待攻克的难题。近年来,克雅二氏病的研究和诊断有很大进展,因此,对目前国内外关于克雅二氏病的研究和诊断作一综述,特别是目前在世界少数国家出现的Real-time quaking-induced conversion(简称RT-QuIC,此处翻译为"实时震动诱导转化")作了详细介绍。

Creutzfeldt Jakob disease （CJD） is the most common type of disease caused by prion .Different from other type of infectious diseases , this disease is caused by pathogens that contain genetic elements , such as viruses or bacteria . It is caused directly by a deformed protein called prion . Even so , the disease has important impact in public health , since prion can＇t be destroyed in routine sterilization procedures . The prion from CJD patients can be transferred through blood transfusion , surgery , and other approaches . The disease has a long latent phase , and is hard to be identified . This causes a big problem in clinical diagnosis . The current diagnosis involves clinical signs , imaging , and histology with low specificity . Early and active diagnosis with high specificity is urgently needed . There are some big developments in the diagnosis of CJD in recent years . This paper summarizes the progress in this area domestically and internationally , especially the recently developed real time quaking induced conversion （ RTQuIC） in details .