Washington Area Comprehensive Sickle Cell Center

描述（由申请人提供）：拟议的华盛顿地区综合镰状细胞中心包括霍华德大学，这是美国首都历史上的黑人教学机构，儿童国家医疗中心，这是该地区镰状细胞病（SCD）儿童医疗保健的主要提供者，以及NHLBI的血管医学分支，这是一个国际公认的单位，在SCD血管并发症的调查中处于领先地位。拟议中的中心还包括与尼日利亚扎里亚的Ahmadu Bello大学的合作，该大学治疗许多尚未接触过输血或羟脲的SCD患者。该中心的主要重点是提高对SCD血管并发症的认识、预防和治疗。该项目的组成部分是：

DESCRIPTION (provided by applicant): The proposed Washington Area Comprehensive Sickle Cell Center includes Howard University, a historically-black teaching institution in the nation's capital, Children's National Medical Center, the premier provider of healthcare to children with sickle cell disease (SCD) in the area, and the Vascular Medicine Branch of NHLBI, an internationally recognized unit that leads in the investigation of the vascular complications of SCD. The proposed Center also includes collaboration with Ahmadu Bello University in Zaria, Nigeria, an institution that treats many SCD patients who have not yet been exposed to blood transfusions or hydroxyurea. The primary focus of the proposed center is to improve understanding, prevention and treatment of the vascular complications of SCD. The components of the project are: 1. An inter-Center Phase II Study of therapy with a nitric oxide-donating combination compared to an angiotensin converting enzyme inhibitor or placebo in preventing renal dysfunction, pulmonary hypertension, stroke and other complications in SCD patients with systemic systolic blood pressure of 120-139 mm Hg. 2. An intra-Center clinical study to determine the prevalence of pulmonary hypertension in Nigerian SCD patients using echocardiographic measurements of the tricuspid regurgitant jet velocity, the contribution of HIV/AIDS and malaria, and the role of genetic polymorphisms in candidate genes that regulate endothelial function and adhesion. 3. A patient services research project to identify the prevalence of developmental problems among infants and toddlers with SCD compared to age and demographically matched controls. 4. A translational research project that seeks to raise levels of HDL and therefore apoA-1 to improve endothelium-dependent vasorelaxation by administration of the NAD precursor, niacin. 5. An Administrative Core. 6. A Clinical Core that includes the adult and pediatric sickle cell clinics at Howard University, the pediatric sickle cell clinic at Children's National Medical Center, the adult sickle cell clinic at NHLBI, and their associated personnel. 7. A Patient Services Core focusing on education, community outreach, genetic counseling and diagnostic testing. 8. A Sickle Cell Scholar Component.