Research Study to Assess the Risk of Blood Borne Transmission of Classic Forms of Creutzfeldt-Jakob Disease

描述（申请人提供）：克雅氏病（Creutzfeldt-Jakob disease， CJD）是由异常朊病毒引起的一种罕见的致死性神经退行性疾病。经典形式的克雅氏病通过输血的传播性目前尚不清楚。拟议的项目将继续进行唯一一项活跃的全国性回顾研究，研究对象是后来被诊断为典型克雅氏病的献血者的血液制品的接受者。这项研究始于1995年，在过去的20年里一直致力于仔细和系统地评估这种疾病在美国血液传播风险的关键目标。该研究的主要组成部分包括与多个来源合作，以确定在美国被诊断为典型CJD的献血者；与血液中心合作，追踪从被诊断为典型CJD的献血者到最终处置的血液成分；收集血液成分接受者的生命状况和死亡原因信息（如适用）；利用研究数据持续监测和评估血液传播克雅氏病的风险；并将研究结果传播给相关利益相关者。到目前为止，这项研究已经招募了54名死于典型CJD的献血者和666名接受他们血液的人。截至2013年，这些接受者中有125人被认为还活着，520人已经死亡，21人失去了随访。输血后，三组患者共存活2719.5人年。共有187名接受输血的人在输血后存活了5年或更长时间，其中86人在供者患克雅氏病前60个月或更短时间内接受了献血。没有发现患有克雅氏病的接受者。这项正在进行的大型回顾性研究目前的结果显示，没有证据表明输血传播克雅氏病。它们强化了这样的结论，即典型CJD供者输血传播朊病毒疾病的风险（如果有的话）明显低于vCJD供者输血传播这种疾病的风险。

DESCRIPTION (provided by applicant): Creutzfeldt-Jakob disease (CJD) is a rare fatal neurodegenerative disorder caused by abnormal prion agents. Transmissibility of the classic forms of CJD by blood transfusion is currently unknown. The proposed project will continue the only active, nationwide look-back study on recipients of blood products from donors subsequently diagnosed with classic CJD. This study began in 1995, and has been working for the past 20 years on the key objective of carefully and systematically assessing the risk of blood borne transmission of this disease in the United States. The major components of the study include collaboration with multiple sources to identify blood donors diagnosed with classic CJD in the United States; working with blood centers to trace the blood components from donors diagnosed with classic CJD to final disposition; collecting vital status and cause of death information (if applicable) on the recipients of blood components; utilizing the data from the study to continually monitor and assess the risk of blood-borne transmission of CJD; and disseminating results of the study to relevant stakeholders. To date, the study has enrolled 54 blood donors who died of classic CJD and 666 recipients of their blood. Through 2013, 125 of these recipients were presumed to be still alive, 520 were deceased and 21 were lost to follow-up. Post transfusion, these three groups had survived a total of 2719.5 person-years. A total of 187 recipients survived five years or longer post transfusion and 86 of them had received blood donated 60 or fewer months before the onset of CJD in the donor. No recipients with CJD have been identified. The current results of this large, ongoing look-back study show no evidence of transfusion transmission of CJD. They reinforce the conclusion that the risk, if any, of transfusion transmission of prion disease by classic CJD donors is significantly lower than the comparable risk of such transmission by vCJD donors.