Ena Javier
Internal Medicine Department, Marina Baixa Hospital, Villajoyosa, Alicante, Spain.
Arch Med Res. 2005 Nov-Dec;36(6):622-7. doi: 10.1016/j.arcmed.2005.02.004.
Prion diseases, also called transmissible spongiform encephalopathies (TSEs), are a family of neurodegenerative disorders affecting both humans and animals. They are caused by the accumulation of an abnormal form of a protein known as prion that results in neuronal death and a characteristic spongiform appearance of the brain tissue. Human prion diseases can be sporadic, acquired or hereditary. Acquired prion diseases have been linked to entering contaminated food into the human food chain, failure to completely disinfect or sterilize contaminated surgical instruments, patients receiving tissues and organs from infected donors, recipients of blood and other biological contaminated products, and potentially to cross infection in dental procedures. At present, there is unfortunately no efficient therapy that can be administered to clinically infected patients with prion diseases. Moreover, there are no simple diagnostic tests that can be used to show the agent of transmissible spongiform encephalopathy during the preclinical phase of the disease. Therefore, to prevent the spread of this emerging infectious agent it is necessary to implement several health control strategies and maintain surveillance for subclinical infections.
朊病毒病，也称为传染性海绵状脑病（TSEs），是一类影响人类和动物的神经退行性疾病。它们是由一种称为朊病毒的异常蛋白质积累引起的，这种积累会导致神经元死亡，并使脑组织呈现出特征性的海绵状外观。人类朊病毒病可以是散发性的、获得性的或遗传性的。获得性朊病毒病与受污染食物进入人类食物链、受污染手术器械未完全消毒或灭菌、患者接受来自感染供体的组织和器官、血液及其他生物污染产品的接受者，以及牙科手术中的潜在交叉感染有关。不幸的是，目前没有有效的疗法可用于临床感染朊病毒病的患者。此外，在疾病的临床前期，没有简单的诊断测试可用于检测传染性海绵状脑病的病原体。因此，为防止这种新出现的传染因子传播，有必要实施多种健康控制策略并对亚临床感染进行监测。
