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

项目摘要 克雅病(CJD)是一种罕见的致命性神经退行性疾病，由 不正常的普恩试剂。非变异(零星、家族性、医源性)形式的遗传性 目前尚不清楚通过输血引起的CJD，而变异型CJD(VCJD)已被证实 通过输血传播。拟议中的项目将继续在全国范围内唯一活跃的 后来诊断为CJD的献血者血液制品接受者的回顾研究。 这项研究始于1995年，在过去的24年里一直致力于以下关键目标 仔细和系统地评估这种疾病通过血液传播的风险 美国。研究的主要组成部分包括与多个来源的合作，以 在美国确认被诊断为CJD的献血者；与血液中心合作 追踪这些献血者的血液成分直到最终处置；收集生命状态和 关于这些血液成分接受者的死因信息(如果适用)；利用 这项研究的数据是为了持续监测和评估疟疾通过血液传播的风险 CJD；并向相关利益攸关方传播研究结果。 到目前为止，这项研究已经招募了76名死于CJD的献血者和1000名CJD的接受者 他们的鲜血。到2016年，这些获奖者中有211人被推定仍然健在，789人 已故的。输血后，这两组人共存活4898人年。一个 共有337名受者在输血后存活5年或更长时间，其中125人 献血者在CJD发病前60个月或更短时间接受献血。没有收件人 患有CJD的患者已被确认。 这项正在进行的大型回顾研究目前的结果显示，没有输血的证据。 CJD的传播。他们强化了这样的结论，即输血的风险，如果有的话， CJD捐赠者传播普恩病毒病的风险显著低于可比的 这种传播是由vCJD捐赠者进行的。

Project Summary Creutzfeldt-Jakob disease (CJD) is a rare, fatal, neurodegenerative disorder caused by abnormal prion agents. Transmissibility of the non-variant (sporadic, familial, iatrogenic) forms of CJD by blood transfusion is currently unknown, while variant CJD (vCJD) has been transmitted by blood transfusion. The proposed project will continue the only active nationwide look-back study on recipients of blood products from donors subsequently diagnosed with CJD. This study began in 1995 and has been working for the past 24 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 CJD in the United States; working with blood centers to trace the blood components from these donors to final disposition; collecting vital status and cause of death information (if applicable) on the recipients of these 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 76 blood donors who died of CJD and 1,000 recipients of their blood. Through 2016, 211 of these recipients were presumed to be still alive and 789 were deceased. Following transfusion, these two groups have survived a total of 4898 person-years. A total of 337 recipients survived five years or longer post transfusion and 125 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 lookback 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 CJD donors is significantly lower than the comparable risk of such transmission by vCJD donors.