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.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Comprehensive Center (P60)
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Special Emphasis Panel (SRC (SH))
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Emory University
Internal Medicine/Medicine
Schools of Medicine
United States
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Walmet, Paula S; Eckman, James R; Wick, Timothy M (2003) Inflammatory mediators promote strong sickle cell adherence to endothelium under venular flow conditions. Am J Hematol 73:215-24
Montes, Richard A O; Eckman, James R; Hsu, Lewis L et al. (2002) Sickle erythrocyte adherence to endothelium at low shear: role of shear stress in propagation of vaso-occlusion. Am J Hematol 70:216-27
Boni, L C; Brown, R T; Davis, P C et al. (2001) Social information processing and magnetic resonance imaging in children with sickle cell disease. J Pediatr Psychol 26:309-19
Frank, N C; Brown, R T; Blount, R L et al. (2001) Predictors of affective responses of mothers and fathers of children with cancer. Psychooncology 10:293-304
Ievers-Landis, C E; Brown, R T; Drotar, D et al. (2001) Situational analysis of parenting problems for caregivers of children with sickle cell syndromes. J Dev Behav Pediatr 22:169-78
Brown, M D; Wick, T M; Eckman, J R (2001) Activation of vascular endothelial cell adhesion molecule expression by sickle blood cells. Pediatr Pathol Mol Med 20:47-72
Tomer, A; Harker, L A; Kasey, S et al. (2001) Thrombogenesis in sickle cell disease. J Lab Clin Med 137:398-407
Brown, R T; Davis, P C; Lambert, R et al. (2000) Neurocognitive functioning and magnetic resonance imaging in children with sickle cell disease. J Pediatr Psychol 25:503-13
Brown, R T; Lambert, R; Devine, D et al. (2000) Risk-resistance adaptation model for caregivers and their children with sickle cell syndromes. Ann Behav Med 22:158-69
Guasch, A; Zayas, C F; Eckman, J R et al. (1999) Evidence that microdeletions in the alpha globin gene protect against the development of sickle cell glomerulopathy in humans. J Am Soc Nephrol 10:1014-9

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