Sickle Hemoglobinopathy reseArch in Zimbabwe (SHAZ)

摘要/概要 津巴布韦镰状血红蛋白病研究（SHAZ）申请的目标是建立一个 可持续的基础设施和运营，旨在解决镰状细胞贫血症的有限数据和差距 目前在津巴布韦和赞比亚存在的疾病（SCD）管理， 撒哈拉以南非洲网络联合体登记册和生物储存库。SHAZ旨在创建 有成就的SCD临床医生和研究人员，他们将专注于介绍和实施 在SSA网络登记工作中的SCD，招募了至少4，000名来自赞比亚的SCD患者， 津巴布韦5年来SHAZ团队将致力于整合区域SCD循证 管理指南，并将开发三个队列研究，以产生更多的遗传性证据， SCD的临床表型，描述SCD中不良结局的自然史和临床相关性， SCD患者特别是异常脑血流与胎儿血红蛋白之间的关系 水平作为中风的危险因素。此外，执行科学研究将被用作一种工具， 促进采用循证实践，改善SCD患者的结局，即：新生儿 在这两个国家进行筛查、改善营养和使用羟基脲。

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.