The clinical research and patient care core programs for the Northern California Comprehensive Sickle Cell Center (NCCSCC) application are based in the East Bay at Children's Hospital and Highland Hospital Oakland, and in the West Bay at San Francisco, General Hospital/University of California, San Francisco. The large patient population enrolled in these cooperative clinical programs provides an ideal source to conduct clinical and basic research projects as proposed. Since 1978, the multi- disciplinary staff of our center has met on a regular basis to discuss patient management issues, evaluate ongoing clinical and basic research projects, and plan new research. The success of these interactions is reflected in our publications related to basic and clinical research on sickle cell disease (listed in the summary progress report), in our participation in national efforts to establish treatment guidelines for patients with sickle cell disease and in our contribution to understanding the etiology and mechanism of sickle cell clinical complications. This application describes a number of new clinical research projects: 1) anti- sickling agents including hydroxyurea-sodium phenylbutyrate, magnesium therapy, clotrimazole; 2) Deferiprone (L1) therapy; 3) CNS ischemia; 4) bone marrow transplant in the treatment of sickle cell disease; 5) core blood transplant and the development of a minority cord blood donor program. In addition the above project, the core will proved necessary samples for many basic research projects. In summary, the strength of the clinical core in our center application will enable us to complete successfully the proposed clinical research projects, participate in basic research and improve the understanding of the etiology and treatment of many sickle cell complications.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Comprehensive Center (P60)
Project #
Application #
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
Fiscal Year
Total Cost
Indirect Cost
University of California San Francisco
San Francisco
United States
Zip Code
Goodman, Jessica; Hassell, Kathryn; Irwin, David et al. (2014) The splenic syndrome in individuals with sickle cell trait. High Alt Med Biol 15:468-71
James, Ellen Butensky; Vreman, Hendrik J; Wong, Ronald J et al. (2010) Elevated exhaled carbon monoxide concentration in hemoglobinopathies and its relation to red blood cell transfusion therapy. Pediatr Hematol Oncol 27:112-21
Jenkins, Zandra A; Hagar, Ward; Bowlus, Christopher L et al. (2007) Iron homeostasis during transfusional iron overload in beta-thalassemia and sickle cell disease: changes in iron regulatory protein, hepcidin, and ferritin expression. Pediatr Hematol Oncol 24:237-43
Styles, Lori A; Abboud, Miguel; Larkin, Sandra et al. (2007) Transfusion prevents acute chest syndrome predicted by elevated secretory phospholipase A2. Br J Haematol 136:343-4
Kuypers, Frans A; Larkin, Sandra K; Emeis, Jef J et al. (2007) Interaction of an annexin V homodimer (Diannexin) with phosphatidylserine on cell surfaces and consequent antithrombotic activity. Thromb Haemost 97:478-86
Neumayr, Lynne D; Aguilar, Christine; Earles, Ann N et al. (2006) Physical therapy alone compared with core decompression and physical therapy for femoral head osteonecrosis in sickle cell disease. Results of a multicenter study at a mean of three years after treatment. J Bone Joint Surg Am 88:2573-82
Wilson, Leslie S; Moskowitz, Judith Tedlie; Acree, Michael et al. (2005) The economic burden of home care for children with HIV and other chronic illnesses. Am J Public Health 95:1445-52
Vichinsky, Elliott; Butensky, Ellen; Fung, Ellen et al. (2005) Comparison of organ dysfunction in transfused patients with SCD or beta thalassemia. Am J Hematol 80:70-4
Pakbaz, Zahra; Fischer, Roland; Treadwell, Marsha et al. (2005) A simple model to assess and improve adherence to iron chelation therapy with deferoxamine in patients with thalassemia. Ann N Y Acad Sci 1054:486-91
Fricke, Britta; Jarvis, Helen G; Reid, Cecil D L et al. (2004) Four new cases of stomatin-deficient hereditary stomatocytosis syndrome: association of the stomatin-deficient cryohydrocytosis variant with neurological dysfunction. Br J Haematol 125:796-803

Showing the most recent 10 out of 207 publications