Sickle Hemoglobinopathy reseArch in Zimbabwe (SHAZ)

摘要/摘要 津巴布韦镰刀血红蛋白病研究(SHAZ)应用的目标是建立一种 旨在解决Sickle cell中有限数据和缺口的可持续基础设施和运营 津巴布韦和赞比亚目前存在的疾病(SCD)管理，通过在#年对SCD做出贡献 撒哈拉以南非洲(SSA)网络联盟登记和生物库。Shaz的目标是创造 有经验的SCD临床医生和研究人员将专注于介绍和实施 SCD参与了SSA网络注册工作，从赞比亚和赞比亚招募了至少4,000名SCD患者 津巴布韦在五年的时间里。Shaz团队将致力于整合基于证据的区域性SCD 管理指南，并将开展三项队列研究，以产生更多关于遗传性的证据 SCD的临床表型，描述了不良结局的自然病史和临床相关性 SCD患者脑血流异常与胎儿血红蛋白的关系 水平是中风的危险因素。此外，将利用实施科学研究作为一种工具 促进循证实践的接受，以改善SCD患者的预后，即：新生儿 这两个国家的筛查、改善营养和羟基尿素的使用。

Abstract/Summary The goal of the Sickle Hemoglobinopathy reseArch in Zimbabwe (SHAZ) application is to establish a sustainable infrastructure and operation aimed at addressing the limited data and gaps in Sickle cell disease (SCD) management that currently exists in Zimbabwe and Zambia by contributing to the SCD in Sub-Saharan Africa (SSA) network consortium registry and biorepository. SHAZ aims to create accomplished SCD clinicians and research personnel who will focus on introduction and implementation of the SCD in SSA network registry efforts, enrolling at least 4,000 individuals with SCD from Zambia and Zimbabwe over a five-year period. The SHAZ team will aim to integrate regional SCD evidence-based management guidelines and will develop three cohort studies to generate more evidence on heritability of clinical phenotype of SCD, describing the natural history and clinical correlates of adverse outcomes in SCD patients specifically the association between abnormal cerebral blood flow and foetal hemoglobin level as a risk factor for stroke. Furthermore, implementation science research will be utilized as a tool to promote uptake of the evidence-based practices that improve outcomes in SCD patients namely: newborn screening, improved nutrition and hydroxyurea use in the two countries.