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

项目摘要 克雅氏病（CJD）是一种罕见的、致命的神经退行性疾病， 异常朊病毒因子非变异型（散发性、家族性、医源性）的遗传性 目前尚不清楚通过输血感染克雅氏病的情况，而变异型克雅氏病（vCJD）已被发现。 通过输血传播。拟议的项目将继续在全国范围内唯一活跃的 对后来被诊断为克雅氏病的献血者的血液制品接受者进行的回顾性研究。 这项研究始于1995年，过去24年来一直致力于实现以下主要目标： 仔细和系统地评估这种疾病在非洲经血液传播的风险， 美国的该研究的主要组成部分包括与多个来源合作， 确定在美国被诊断为CJD的献血者;与血液中心合作， 追踪这些献血者的血液成分，直至最终处置;收集生命状态， 这些血液成分接受者的死亡原因信息（如适用）;利用 研究数据，以持续监测和评估血液传播的风险， 克雅氏病;以及向有关利益攸关方传播研究结果。 到目前为止，该研究已经招募了76名死于CJD的献血者和1，000名接受CJD治疗的人。 他们的血到2016年，这些接受者中有211人被认为仍然活着，789人被认为是 死者输血后，这两组共存活4898人年。一 共有337名受者在输血后存活了5年或更长时间，其中125人 在献血者CJD发病前60个月或更短时间内接受献血。没有收件人 CJD患者已被确认。 这项大型的、正在进行的回顾性研究的当前结果显示，没有输血的证据 传播CJD。他们加强了这样的结论，即输血的风险，如果有的话， CJD捐赠者传播朊病毒疾病的风险显著低于 vCJD捐助者的这种传播。

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.