Sickle cell disorders are very common in Africa. In West Africa, 15-20% of the coastal and forest populations have sickle cell trait and 1% of all babies have a form of sickle cell disease (SCD). In most African countries SCD is not considered a major public health problem because the impact of SCD on childhood mortality has not been carefully studied. In some parts of West Africa it is estimated that 98% of children with SCD do not live past 5 years of age. In Africa, the leading cause of death in children with SCD is malaria, followed by pneumococcal infection. The effect of malaria makes the clinical course of SCD in Africa quite different from that in the U.S. The reasons underlying this increased risk of death due to malaria and the extent of the problem have not been carefully studied. Young children with SCD in Africa, like their counterparts in the U.S., face increased mortality from infection. In the U.S. this problem is being addressed through newborn screening and penicillin prophylaxis therapy. In fact, most children born with SCD in Africa are not diagnosed prior to serious illness and death. This is because there are no programs organized for the screening of newborns for SCD, and there are no longitudinal studies in which children with SCD have been followed closely from birth to collect accurate data on the clinical course of SCD from infancy. It is only through such programs that the true morbidity and mortality of SCD in the African environment can be determined. The main objective of this project is to design and implement a pilot newborn screening program for SCD in Kumasi, the second largest city in Ghana as a demonstration project for West Africa. Secondary objectives are to &fin the staff who will work in the screening project, and to design a longitudinal study of the clinical course of SCD into which babies found to have SCD in early childhood will be enrolled for long-term close monitoring. The project is planned as a collaborative one between this Sickle Cell Center and the Noguchi Memorial Institute for Medical Research in Ghana, with participation by the Ministry of Health of Ghana and the School of Medical Sciences in Kumasi. Laboratory support for the project will be based at Noguchi and the clinical services will be based at Komfo Anokye Teaching Hospital in Kumasi. Technical assistance will be provided by this Center and the Core Laboratory (Project 9001) will serve as a reference laboratory for the laboratory at Noguchi. In conjunction with this project, other research studies to study the effects of malaria on children with SCD, and the efficacy of antimalarial and penicillin prophylactic therapy are planned.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Comprehensive Center (P60)
Project #
2P60HL038632-06
Application #
3780637
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
6
Fiscal Year
1993
Total Cost
Indirect Cost
Name
Children's Hospital of Philadelphia
Department
Type
DUNS #
073757627
City
Philadelphia
State
PA
Country
United States
Zip Code
19104
Ballas, Samir K; Connes, Philippe; Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia (2018) Rheological properties of sickle erythrocytes in patients with sickle-cell anemia: The effect of hydroxyurea, fetal hemoglobin, and ?-thalassemia. Eur J Haematol 101:798-803
Kwiatkowski, Janet L; Zimmerman, Robert A; Pollock, Avrum N et al. (2009) Silent infarcts in young children with sickle cell disease. Br J Haematol 146:300-5
Adachi, Kazuhiko; Ding, Min; Asakura, Toshio et al. (2009) Relationship between beta4 hydrogen bond and beta6 hydrophobic interactions during aggregate, fiber or crystal formation in oversaturated solutions of hemoglobin A and S. Arch Biochem Biophys 481:137-44
Kiryu, Shigeru; Sundaram, Tessa; Kubo, Shigeto et al. (2008) MRI assessment of lung parenchymal motion in normal mice and transgenic mice with sickle cell disease. J Magn Reson Imaging 27:49-56
Niebanck, Alison E; Pollock, Avrum N; Smith-Whitley, Kim et al. (2007) Headache in children with sickle cell disease: prevalence and associated factors. J Pediatr 151:67-72, 72.e1
Uematsu, Hidemasa; Takahashi, Masaya; Hatabu, Hiroto et al. (2007) Changes in T1 and T2 observed in brain magnetic resonance imaging with delivery of high concentrations of oxygen. J Comput Assist Tomogr 31:662-5
Obata, Kazuo; Mattiello, Julian; Asakura, Kenji et al. (2006) Exposure of blood from patients with sickle cell disease to air changes the morphological, oxygen-binding, and sickling properties of sickled erythrocytes. Am J Hematol 81:26-35
Akbar, Mohammed G K; Tamura, Yutaka; Ding, Min et al. (2006) Inhibition of hemoglobin S polymerization in vitro by a novel 15-mer EF-helix beta73 histidine-containing peptide. Biochemistry 45:8358-67
Adachi, Kazuhiko; Ding, Min; Surrey, Saul et al. (2006) The Hb A variant (beta73 Asp-->Leu) disrupts Hb S polymerization by a novel mechanism. J Mol Biol 362:528-38
Asakura, Toshio; Asakura, Kenji; Obata, Kazuo et al. (2005) Blood samples collected under venous oxygen pressure from patients with sickle cell disease contain a significant number of a new type of reversibly sickled cells: constancy of the percentage of sickled cells in individual patients during steady state. Am J Hematol 80:249-56

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