Ghana-SPARCO: Ghana Sickle Pan-African Research Consortium

加纳-SPARCO：加纳镰刀泛非研究联盟

• 批准号: 10402928
• 负责人: Solomon Fiifi Ofori-Acquah
• 金额: $ 26.53万
• 依托单位: KWAME NKRUMAH UNIVERSITY/SCIENCE/TECH
• 依托单位国家: 美国
• 财政年份: 2021
• 资助国家: 美国
• 起止时间: 2021-05-10 至 2026-04-30
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/10402928
• 关键词: Abnormal Hemoglobins; Acute; Address; Adolescence; Adolescent; Adoption; Adult; Advocacy; Affect; Africa; Africa South of the Sahara; African; Analgesics; Area; Benchmarking; Blood Transfusion; Capital; Caring; Cessation of life; Child; Chronic; Cities; Clinic; Clinical; Coagulation Process; Cohort Studies; Consent; Country; Data; Decision Making; Disease; Disease Management; Endothelium; Enrollment; Erythrocytes; Ethics; Etiology; Event; Future; Genes; Genetic Diseases; Genotype; Ghana; Goals; Government; Grant; Guidelines; Harvest; Health Policy; Healthcare; Hematology; Hemoglobin; Hemolytic Anemia; Hereditary Disease; Hospitals; Hydration status; Impaired cognition; Incidence; Individual; Infection; Infection prevention; Infrastructure; Intervention; Kidney; Kidney Diseases; Laboratories; Longevity; Medical center; Morbidity - disease rate; Neonatal Screening; Neurologic; Nigeria; Online Systems; Ontology; Organ; Outcome; Patient Care; Patients; Penicillins; Pharmaceutical Preparations; Phase; Phenotype; Pneumococcal vaccine; Policies; Prenatal Diagnosis; Prophylactic treatment; Prospective cohort; Proteinuria; Protocols documentation; Quality of life; Recurrence; Registries; Renin-Angiotensin-Aldosterone System; Research; Resource-limited setting; Resources; Sickle Cell; Sickle Cell Anemia; Site; Specimen; Standardization; Stroke; Structure; Syndrome; System; Tanzania; Training; Transfusion; Treatment outcome; Ursidae Family; Vascular Endothelium; Vision; Work; base; care delivery; cell registry; clinical care; cohort; evidence base; feasibility testing; functional status; hydroxyurea; implementation facilitators; implementation research; implementation strategy; implementation study; improved; mHealth; mortality; neurovascular; oxidant stress; patient registry; point of care testing; preventive intervention; programs; prophylactic; prospective; recruit; research study; screening; screening program; sickling; sociodemographics; statistics; systemic inflammatory response

PROJECT SUMMARY Sickle Cell Disease (SCD) is a recessively inherited disorder characterized by disordered hemoglobin structure, aberrant endothelial interactions, systemic inflammation, oxidant stress, and activation of the coagulation system. These abnormalities underlie a myriad of complications including acute vaso-occlusive syndromes and a multi-system progressive chronic end-organ damage. Sub-Saharan Africa (SSA) bears a disproportionately higher burden with an estimated 15 million of the world’s 25 million SCD individuals; 75% of all babies born with SCD; & mortality rate for children under 5 range between 50%-90%. These include absence of National control programs, dearth of basic facilities to manage patients, absence of systematic screening for SCD, pervasive lack of implementation of evidence-based preventative interventions, cultural misconceptions about the genesis of the disease, and lack of governmental support. By the sheer numbers of patients in Africa, there is a potential for large volumes of clinical information to be harvested though operational research for analyses to inform evidence-based decision-making on healthcare, as well as healthcare policies for SCD on the continent. To this end, the Sickle Pan-African Research Consortium (SPARCo) in 2017 started a registry across multiple sites in three countries: Ghana (an applicant for the current U01 grant), Nigeria, and Tanzania. In the first phase of SPARCO, we established a collaborative site in Kumasi and enrolled 2,915 SCD patients including 1,453 from our Newborn screening program into a registry, and built capacity for future research studies. In this second phase, we have added an in-country Satellite site in the capital city Accra, which has well developed clinical, training and research programs in SCD, to form Ghana-SPARCO. Our overarching goal is to establish a prospective cohort of 6,000 individuals living with SCD across the lifespan with a dual purpose of understanding: 1) the determinants of the protean manifestations of acute and chronic complications of SCD in a resource-limited setting, and 2) to conduct implementation research to address, surmount & integrate the best resource-based standards of clinical care. Our vision is to establish hubs of excellence for research and care for SCD in Ghana. We will work with the Coordinating Center to; (i) establish a harmonized patient registry to, (ii) integrate standards of care for SCD, (iii) conduct cohort studies focusing on an assessment of burden and predictors of neurovascular, renal and infectious complications of SCD across the lifespan & (iv) conduct implementation research studies on newborn screening, retention of adolescence in SCD clinics & develop an implementation strategy for routine integration of preventative interventions for SCD. Achieving these benchmarks will help establish the infrastructure to develop a SCD in SSA Research Network.

项目概要 镰状细胞病 (SCD) 是一种隐性遗传性疾病，其特征是血红蛋白紊乱 结构、异常的内皮相互作用、全身炎症、氧化应激和激活 凝血系统。这些异常是多种并发症的基础，包括急性血管闭塞 综合征和多系统进行性慢性终末器官损伤。撒哈拉以南非洲 (SSA) 全球 2500 万 SCD 个体中估计有 1500 万的负担过高； 75% 所有出生时患有 SCD 的婴儿； 5岁以下儿童的死亡率在50%-90%之间。这些包括 缺乏国家控制计划、缺乏管理患者的基本设施、缺乏系统的 SCD 筛查、普遍缺乏基于证据的预防性干预措施、文化 对疾病起源的误解以及缺乏政府支持。从纯粹的数字来看 通过对非洲患者的调查，有可能收集大量临床信息 运筹学分析，为医疗保健方面的循证决策提供信息，以及 非洲大陆 SCD 的医疗保健政策。为此，镰刀泛非研究联盟 (SPARCo) 于 2017 年在三个国家的多个地点启动了注册： 加纳（SPARCo 的申请人） 目前的 U01 赠款）、尼日利亚和坦桑尼亚。在SPARCO第一期，我们建立了协作站点 在库马西，招募了 2,915 名 SCD 患者，其中包括来自我们新生儿筛查项目的 1,453 名患者 注册，并建立未来研究的能力。在第二阶段，我们增加了一个国内 位于首都阿克拉的卫星站点，在以下领域拥有完善的临床、培训和研究项目 SCD，组建加纳-SPARCO。我们的总体目标是建立一个由 6,000 人组成的前瞻性队列 在整个生命周期中与 SCD 一起生活，具有双重理解目的：1) 多变的决定因素 在资源有限的环境中出现 SCD 急性和慢性并发症的表现，以及 2) 进行 实施研究，以解决、超越和整合基于最佳资源的临床标准 关心。我们的愿景是在加纳建立卓越的 SCD 研究和护理中心。我们将与 协调中心； (i) 建立统一的患者登记处，(ii) 整合患者的护理标准 SCD，(iii) 进行队列研究，重点评估神经血管、肾脏的负担和预测因素 以及整个生命周期中 SCD 的感染性并发症 & (iv) 开展实施研究 新生儿筛查、在 SCD 诊所保留青春期并制定常规实施策略 整合 SCD 的预防性干预措施。实现这些基准将有助于建立 在 SSA 研究网络中开发 SCD 的基础设施。