Sickle Africa Data Coordinating Center

项目摘要/摘要(30行) 据估计，全球每年有30.58万名婴儿出生时患有镰状细胞病(SCD)，其中近 撒哈拉以南非洲(SSA)75%的出生人口。尽管发病率很高，但目前还没有有效的 SSA国家的公共卫生计划侧重于SCD。因此，高达90%的婴儿患有 SSA中的SCD被认为在五岁前不必要地死亡。来自高收入国家的证据 表明新生儿筛查和青霉素预防等干预措施可以减少 SSA中因SCD导致的可预防死亡。这样的计划使死亡率降低了约70% 在美国SCD儿童中的发病率。然而，美国成年SCD患者的死亡率 由于多器官并发症，在过去的30年里没有改善。因此，有一个主要的需求 为世界各地的SCD开发有效的终生治疗方法。高 预计SSA的区域疾病流行情况将有助于开展关于SCD的研究。2017年， 我们建立了镰刀非洲数据协调中心(SADaCC)，以支持 镰刀泛非研究联盟(SPARCO)在坦桑尼亚、尼日利亚和加纳的站点。 这项U24申请旨在继续SADaCC活动，以维持、加强和发展SCD 非洲网络，以促进与SCD相关的流行病学、转化性和临床 研究。这一总体目标将通过五个具体目标来实现：目标1：全面提供 对SCD特别服务协议网络的协调和行政支持，这将包括：促进 互动和功能，以及标准作业程序和网站的进一步发展；目标2：确保 维护和扩展中央电子镰刀状血红蛋白病数据库 将包括：更新数据捕获表格和注册SOP；一个安全和可扩展的数据系统 收集目标3：提供数据管理支持，包括：和开发公共用途 质量控制数据集；生物检验库管理系统；执行 针对具体国家的人体受试者研究条例。目标4：提供专业知识和支持 实施研究研究，包括：规划、设计、执行、分析、报告、 出版和传播研究报告。目标5：在整个财团范围内提供有效的 可持续发展研究网络绩效和影响的参与性里程碑和衡量标准。这个 SADaCC团队作为SickleInAfrica协调中心的成功经验预示着它的规定 对扩大的可持续发展研究网络的可持续支持。

Project Summary/Abstract (30 lines) An estimated 305,800 babies are born worldwide each year with Sickle Cell Disease (SCD), with nearly 75% of births in sub-Saharan Africa (SSA). Despite this high incidence, there are currently no effective public health programs in SSA countries focused on SCD. As a consequence, up to 90% of infants with SCD in SSA are believed to die needlessly by five years of age. Evidence from high income countries indicates that interventions such as newborn screening and penicillin prophylaxis can reduce preventable deaths due to SCD in SSA. Such programs are credited with an ~70% reduction in mortality rate among children with SCD in the USA. However, the death rate in adult SCD patients in the USA has not improved in the last thirty years, due to multi-organ complications. There is thus a major need for research to develop effective therapies across the life span for SCD in all parts of the world. High regional disease prevalence in SSA would be expected to facilitate research studies on SCD. In 2017, we established the Sickle Africa Data Coordinating Center (SADaCC) to support the activities of the Sickle Pan African Research Consortium (SPARCo) sites in Tanzania, Nigeria, and Ghana. This U24 application seeks to continue SADaCC activities to sustain, enhance, and grow the SCD Network in Africa, in order to advance SCD-related epidemiological, translational, and clinical research. This overall objective will be accomplished by five specific aims: Aim 1: To provide overall coordination and administrative support to the SCD SSA Network, that will include: the facilitation of interactions and functions, and further development of SOPS and websites; Aim 2: To ensure maintenance and expansion of the centralized electronic sickle hemoglobinopathy database that will include: updating data capture forms and registry SOPs; a secure and scalable system for data collection Aim 3: To provide data management support including: and developing the public-use quality controlled data sets; a repository management system for biospecimens; the enforcement of country-specific human subject research regulations. Aim 4: To provide expertise and support for implementation of research studies, including: planning, design, execution, analysis, reporting, publication, and dissemination of research studies. Aim 5: To provide effective consortium-wide participatory milestones and metrics for the SCD Research Network performance and impact. The SADaCC team’s successful experience as the coordinating center for SickleInAfrica augurs its provision of sustainable support to an expanded SCD Research Network.