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.
|Tatari-Calderone, Zohreh; Gordish-Dressman, Heather; Fasano, Ross et al. (2016) Protective effect of HLA-DQB1 alleles against alloimmunization in patients with sickle cell disease. Hum Immunol 77:35-40|
|Glass, Penny; Brennan, Tara; Wang, Jichuan et al. (2013) Neurodevelopmental deficits among infants and toddlers with sickle cell disease. J Dev Behav Pediatr 34:399-405|
|Luban, Naomi L C; McBride, Eileen; Ford, Jason C et al. (2012) Transfusion medicine problems and solutions for the pediatric hematologist/oncologist. Pediatr Blood Cancer 58:1106-11|
|Sachdev, Vandana; Kato, Gregory J; Gibbs, J Simon R et al. (2011) Echocardiographic markers of elevated pulmonary pressure and left ventricular diastolic dysfunction are associated with exercise intolerance in adults and adolescents with homozygous sickle cell anemia in the United States and United Kingdom. Circulation 124:1452-60|
|Nouraie, Mehdi; Reading, Noel S; Campbell, Andrew et al. (2010) Association of G6PD with lower haemoglobin concentration but not increased haemolysis in patients with sickle cell anaemia. Br J Haematol 150:218-25|
|Aliyu, Zakari Y; Suleiman, Aisha; Attah, Ester et al. (2010) NT-proBNP as a marker of cardiopulmonary status in sickle cell anaemia in Africa. Br J Haematol 150:102-7|
|Kasvosve, Ishmael; Debebe, Zufan; Nekhai, Sergei et al. (2010) Ferroportin (SLC40A1) Q248H mutation is associated with lower circulating plasma tumor necrosis factor-alpha and macrophage migration inhibitory factor concentrations in African children. Clin Chim Acta 411:1248-52|