Comparative Study of Haitian and Miami Cohorts of Haitian-American and Haitian Immigrant Children with Sickle Cell Disease (CSHSCD)

项目摘要/摘要 镰状细胞病(SCD)是一种慢性疾病，在美国主要影响少数族裔。它也是一种 加勒比海流行的疾病。患有SCD的儿童容易出现许多并发症，例如 感染、疼痛和中风。新生儿筛查及青霉素预防和综合护理 在其他护理标准措施中，免疫接种提供了改善生活质量和给予 生存的机会。海地是加勒比海地区最贫穷的国家之一， 目前有资源用于实施其中一些措施。海地人在南方是一个相当大的少数民族 来自佛罗里达州，是迈阿密大学镰刀细胞诊所三分之一的患者。此应用程序 提供了在数据库开发和队列监测研究方面进行合作研究的机会 在美国和海地，患有SCD的新生儿和6岁以下儿童。这项研究 问题是这两个地理区域的发病率和健康结果的差异有多大 (佛罗里达州的迈阿密和海地的几个地点)。该建议有3个具体目标：1.确定 迈阿密戴德县海地裔患有SCD的新生儿使用现有的新生儿数据库 放映。2.扩大以医院为基础的新生儿筛查，使用护理点式设备和中央实验室 海地的筛查能力，以便确认SCD的发病率，以便与目标1.3进行比较。 并比较以美国为基地的海地民族队列(与非裔美国人)在护理和健康结果方面的障碍 镰状细胞控制)和海地队列。我们将建立在以前获得的数据的基础上，SCD在 海地新生儿中每125名儿童中就有一名患有SCD，并将扩大红帽 海地海地角的儿童数据库。将使用PhenX标准化措施来扩展 迈阿密和海地网站的数据库。人口统计，身高和体重随时间的变化(增长)， 根据经颅多普勒超声，贫血严重程度，SCD相关并发症，包括中风风险 测试，治疗依从性，通过问卷评估的护理障碍，海地移民的文化适应， 死亡率将会被收集起来。实施经颅多普勒超声(TCD)，这是一种非 筛选中风风险的侵入性方法在海地将是新的。我们将通过员工培训和电话- 辅导。中心阅读将在迈阿密大学进行。我们希望从低谷中吸取教训并从中受益 加勒比地区(海地)的资源配置，以影响生活在美国的海地人，并促进强大的国际 协作。

Project Summary/Abstract Sickle cell disease (SCD) is a chronic illness which affects mostly minorities in the United States. It is also a prevalent disease in the Caribbean. Children afflicted with SCD are prone to many complications, such as infections, pain and stroke. Newborn screening and comprehensive care with penicillin prophylaxis and immunizations, among other standard-of-care measures, provide the chance to improve quality of life and give the opportunity for survival. Haiti is one of the poorest countries in the Caribbean which does not count with resources at the present time to implement some of these measures. Haitians are a significant minority in South Florida, and are a third of the patients who attend the University of Miami Sickle Cell Clinic. This application presents the opportunity for collaborative research in database development and cohort surveillance studies for newborns and young children less than 6 years of age with SCD in the United States and Haiti. The research question is to what extent there are differences in incidence and health outcomes in the two geographic areas (Miami in Florida and in several Haiti sites). The proposal has 3 specific aims: 1. Determine the incidence of newborns of Haitian ethnicity with SCD in Miami Dade County using available databases from newborn screening. 2. Expand hospital-based newborn screening with point-of-care device and centralized laboratory screening capability in Haiti so that SCD incidence is confirmed, allowing for comparison with Aim 1. 3. Examine and compare barriers to care and health outcomes of the US-based Haitian ethnic cohort (with African-American sickle cell controls) and Haitian cohorts. We will build on previously obtained data that the incidence of SCD in Haiti among newborns is as high as 1 child out of 125 children born has SCD, and will expand the RED Cap database of children followed in Cap Haitien, Haiti. PhenX standardized measures will be used to expand the database for the Miami and Haitian sites. Demographics, changes in height and weight over time (growth), anemia severity, SCD-related complications including stroke risk according to transcranial Doppler ultrasound testing, treatment adherence, barriers to care via questionnaire assessment, acculturation in Haitian immigrants, and mortality will be collected. The implementation of transcranial Doppler ultrasound (TCD), which is a non- invasive method to screen for stroke risk, will be new in Haiti. We will accomplish this with staff training and tele- mentoring. Central reading will be done at the University of Miami. We expect to learn from and benefit a low resource setting in the Caribbean (Haiti), to impact Haitians living in the US, and promote strong international collaboration.