Research Study to Assess the Risk of Blood Borne Transmission of Creutzfeldt-Jako

描述（由申请人提供）：克雅氏病（CJD）是由朊病毒引起的一种致命的神经退行性疾病。1996年，英国出现了另一种人类朊病毒疾病——变异型克雅氏病。本申请的目的是继续1995年开始的一项积极的、全国性的研究，研究对象是被诊断为典型克雅氏病的献血者的血液制品接受者，以评估这种疾病在美国经血液传播的风险。该研究将进行如下工作：1)维持并加强与美国血库的合作关系，以确定在美国被诊断患有CJD的献血者，并收集这些献血者的可用、相关、医疗和人口统计信息；2)追踪CJD献血者血液成分直至最终处置；3)维护和加强与最终处置地点的合作关系，从既往的CJD血液成分受者记录中收集生命统计信息；4)维持本研究中先前确定的CJD供者血液成分受体的研究信息，并继续监测这些受体的生命状态，包括死亡原因；5)继续执行一项计划，该计划将(a)搜索国家、州和地方组织的数据库，以匹配组成接受者的生命统计数据和死亡原因，包括利用非国家死亡指数数据库确认组成接受者的生命状况，以及(b)评估血液传播克雅氏病的风险；6)发表和传播研究结果。从这项研究中系统收集的数据将有助于确定经典的克雅氏病是否通过血液和血液成分在美国传播。

DESCRIPTION (provided by applicant): Creutzfeldt Jakob disease (CJD) is a fatal neurodegenerative disorder caused by prion agents. In 1996, another human prion disease, variant CJD, emerged in the United Kingdom. The purpose of this application is to continue an active, nationwide study begun in 1995 of recipients of blood products from blood donors subsequently diagnosed with classic CJD to assess the risk of blood borne transmission of this disease in the United States. The study will be conducted as follows: 1) maintain and increase collaborative relationships with US blood banks to identify blood donors diagnosed with CJD in the United States and gather available, relevant, medical and demographic information on such donors; 2) trace CJD donor blood components to final disposition; 3) maintain and enhance collaborative relations with final disposition sites and collect vital statistics information from pre-existing records about recipients of CJD blood components; 4) maintain study information about the recipients of the blood components from CJD donors who were previously identified in this study and continue to monitor these recipients' vital status, including the causes of death should they die; 5) continue a plan that will (a) search national, state, and local organizations databases to match vital statistics and causes of death for the component recipients, including utilizing non-National Death Index databases to confirm the vital status of the component recipients and (b) assess the risk of blood-borne transmission of CJD; 6) publish and disseminate results of the study. Data that will be systematically collected from this study will help determine whether classic CJD is being transmitted by blood and blood components in the United States. PUBLIC HEALTH RELEVANCE: This study collects recipient outcome data to assess the potential risk of transmission of Creutzfeldt-Jakob disease (CJD) by blood components from donors who subsequently developed CJD. There are two phases to this study. A retrospective phase which includes identifying the CJD infected blood donors and the recipients of those blood units and a prospective phase that tracks the recipients of blood donated by CJD donors. During the retrospective phase we receive reports from blood centers, family members and the Centers for Disease Control and Prevention (CDC). Once we receive the report we notify the blood center (if previously not notified), the hospitals and/or transfusion centers identify the recipients and report the survival status and if deceased the date and cause of death of that recipient. If the recipient is listed as deceased we verify the date and cause of death with the National Death Index (NDI). If the recipient is listed as alive we move onto the prospective phase of the study. This consists of tracking the recipient to see if they are currently alive (as of the last available NDI data set) and if not the date and cause of death. We track the recipients by using the National Death Index plus database and other databases when needed. We match the recipient's information to the information in the database to determine if it is a match. The risk to the human subjects is minimal. For donors who are reported by the CDC and Blood Centers only the donor initials are available. For those individuals whose family members report the case, they receive an information letter and Consent for Participation and Release of Information. Investigation does not proceed unless the consent for participation is signed. Names of the family reported donors are kept in a secure cabinet with very limited personnel access. Only the donor initials are used when imputing the data in the database. The risk is minimal for the recipients. The surviving recipients are not notified of the tracking because to date there has not been a transfusion transmission of classic CJD. Notifying the recipient would cause undue stress and mental anguish. This would be an unnecessary harm to the recipient. All recipient information both death and alive are kept in a secure cabinet with very limited personnel access. Names, Date of Birth and Social Security Number of the recipients are reported in the database and the database can only be accessed by specific co-investigators.