Human prion diseases: surgical lessons learned from iatrogenic prion transmission.
Human prion diseases: surgical lessons learned from iatrogenic prion transmission.
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DOI：
10.3171/2016.5.focus15126
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发表时间：
2016-07
影响因子：
4.1
通讯作者：
Cali I
中科院分区：
文献类型：
--
作者：
Bonda DJ;Manjila S;Mehndiratta P;Khan F;Miller BR;Onwuzulike K;Puoti G;Cohen ML;Schonberger LB;Cali I
人类朊病毒疾病，或传染性海绵状脑病，自从20世纪50年代在巴布亚新几内亚的福尔语系人群中发现以来，一直吸引着我们的想象力。这种神秘而又知之甚少的“传染性蛋白质”在地球仪的许多地区已经成为家喻户晓的名字。从牛海绵状脑病（BSE），通常被认为是疯牛病，到内食人症，媒体都利用了这些致命的神经系统疾病。有趣的是，自从他们被发现以来，已经有超过492起朊病毒疾病的医源性传播事件，主要是由朊病毒污染的生长激素和硬脑膜移植引起的。尽管全世界报告的可能的医源性神经外科克雅氏病（CJD）病例不到9例，但一些漏诊病例的可能性和神经外科手术引起朊病毒传播的可能性引起了相当大的关注。实验室研究表明，标准的去污和灭菌程序可能不足以完全去除朊病毒污染器械的传染性。在这种不幸的情况下，这些仪器可能会将朊病毒疾病传染给他人。因此，在缺乏有力证据反对神经外科患者存在朊病毒疾病的情况下，应谨慎行事。虽然疾病控制和预防中心（CDC）和世界卫生组织（WHO）已经制定了风险评估和净化协议，以预防朊病毒疾病的医源性传播，但不幸的是，在美国发生了可能接触朊病毒的事件。在这篇文章中，作者概述了从库鲁病到病理性朊病毒蛋白的鉴定和分离的历史发现，此外还简要描述了人类朊病毒疾病和医源性CJD形式，朊病毒疾病医院传播的简史，以及CDC和WHO预防朊病毒疾病传播和朊病毒污染神经外科器械去污指南的摘要。
The human prion diseases, or transmissible spongiform encephalopathies, have captivated our imaginations since their discovery in the Fore linguistic group in Papua New Guinea in the 1950s. The mysterious and poorly understood “infectious protein” has become somewhat of a household name in many regions across the globe. From bovine spongiform encephalopathy (BSE), commonly identified as mad cow disease, to endocannibalism, media outlets have capitalized on these devastatingly fatal neurological conditions. Interestingly, since their discovery, there have been more than 492 incidents of iatrogenic transmission of prion diseases, largely resulting from prion-contaminated growth hormone and dura mater grafts. Although fewer than 9 cases of probable iatrogenic neurosurgical cases of Creutzfeldt-Jakob disease (CJD) have been reported worldwide, the likelihood of some missed cases and the potential for prion transmission by neurosurgery create considerable concern. Laboratory studies indicate that standard decontamination and sterilization procedures may be insufficient to completely remove infectivity from prion-contaminated instruments. In this unfortunate event, the instruments may transmit the prion disease to others. Much caution therefore should be taken in the absence of strong evidence against the presence of a prion disease in a neurosurgical patient. While the Centers for Disease Control and Prevention (CDC) and World Health Organization (WHO) have devised risk assessment and decontamination protocols for the prevention of iatrogenic transmission of the prion diseases, incidents of possible exposure to prions have unfortunately occurred in the United States. In this article, the authors outline the historical discoveries that led from kuru to the identification and isolation of the pathological prion proteins in addition to providing a brief description of human prion diseases and iatrogenic forms of CJD, a brief history of prion disease nosocomial transmission, and a summary of the CDC and WHO guidelines for prevention of prion disease transmission and decontamination of prion-contaminated neurosurgical instruments.