The primary goal of the Georgia Comprehensive Sickle Cell Center is to provide basic and clinical research, education, laboratory diagnosis, counseling and patient care in sickle cell syndromes. Research projects will address issues of pathophysiology and treatment for important complications in patients with these disorders. Project 1 will investigate factors influencing adherence of sickle erythrocytes to endothelial cells in an in vitro system with flow. Project 2 will explore effects of sickle erythrocytes and flow on endothelial cellular function. The role of complement, white cells, and cytokines in the pathophysiology and the utility of steroids in treating acute chest syndrome will be determined in Project 3. Project 4 will study the role of thrombosis in sickle crisis and if dietary omega-3 fatty acids, potent inhibitors of in vivo thrombus formation, will decrease frequency of pain episodes. The role of increased blood flow and ultrafiltration in the progression of proteinuria and renal insufficiency will be explored in Project 5. In Project 6, the therapeutic utility bone marrow transplantation in children with serious neurologic complications will be defined. Project 7 will study neurocognitive, psychological, and social functioning in children with sickle syndromes. A counseling project by the Sickle Cell Foundation of Georgia will determine if accurate diagnosis of alpha thalassemia from automated blood analysis helps in counseling parents of children with hemoglobin Barts. Finally, an education project will include an annual education program in Atlanta, new methods of education using computer systems, and computer based, problem oriented patient simulations and references. These projects will be supported by an administrative core, a laboratory core providing DNA based diagnosis of genotype, and a research- statistical core supporting study design, data collection, storage, analysis, and dissemination. These collaborative research and demonstration projects will utilize the unique resources of the Georgia Sickle Cell Center at Grady Memorial Hospital which provides acute and ongoing care for over 1200 children and adults with sickle syndromes.
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