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The Scientific World Journal
Volume 2013, Article ID 193252, 16 pages
http://dx.doi.org/10.1155/2013/193252
Review Article

Sickle Cell Disease: New Opportunities and Challenges in Africa

1Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences, P.O. Box 65001, Dar es Salaam, Tanzania
2Nuffield Department of Medicine, University of Oxford, Oxford, UK
3Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA
4School of Allied Health Sciences, College of Health Sciences, University of Ghana, Ghana
5Department of Haematology and Blood Transfusion, College of Health Sciences, University of Abuja, Abuja, Nigeria
6Division of Human Genetics, Faculty of Heath Sciences, University of Cape Town, South Africa
7Faculty of Medicine and Biomedical Sciences, University of Yaoundé I, Cameroon
8Children’s Hospital of Philadelphia, Philadelphia, PA, USA

Received 23 April 2013; Accepted 9 June 2013

Academic Editors: Y. Al-Tonbary, M. A. Badr, A. El-Beshlawy, A. Mansour, and F. Tricta

Copyright © 2013 J. Makani et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Linked References

  1. J. K. Onwubalili, “Sickle cell anaemia and reincarnation beliefs in Nigeria,” The Lancet, vol. 2, no. 8364, p. 1423, 1983. View at Google Scholar · View at Scopus
  2. E. Nzewi, “Malevolent ogbanje: recurrent reincarnation or sickle cell disease?” Social Science and Medicine, vol. 52, no. 9, pp. 1403–1416, 2001. View at Publisher · View at Google Scholar · View at Scopus
  3. S. E. Antonarakis, C. D. Boehm, G. R. Serjeant, C. E. Theisen, G. J. Dover, and H. H. Kazazian Jr., “Origin of the β(S) globin gene in Blacks: the contribution of recurrent mutation or gene conversion or both,” Proceedings of the National Academy of Sciences of the United States of America, vol. 81, no. 3, pp. 853–856, 1984. View at Google Scholar · View at Scopus
  4. A. E. Kulozik, J. S. Wainscoat, G. R. Serjeant et al., “Geographical survey of β(S)-globin gene haplotypes: evidence for an independent Asian origin of the sickle-cell mutation,” American Journal of Human Genetics, vol. 39, no. 2, pp. 239–244, 1986. View at Google Scholar · View at Scopus
  5. D. J. Weatherall, “Towards molecular medicine; reminiscences of the haemoglobin field, 1960–2000,” British Journal of Haematology, vol. 115, no. 4, pp. 729–738, 2001. View at Publisher · View at Google Scholar · View at Scopus
  6. D. J. Weatherall, “Phenotype-genotype relationships in monogenic disease: lessons from the thalassaemias,” Nature Reviews Genetics, vol. 2, no. 4, pp. 245–255, 2001. View at Publisher · View at Google Scholar · View at Scopus
  7. M. J. Stuart and R. L. Nagel, “Sickle-cell disease,” The Lancet, vol. 364, no. 9442, pp. 1343–1360, 2004. View at Publisher · View at Google Scholar · View at Scopus
  8. R. L. Nagel, R. M. Bookchin, and J. Johnson, “Structural bases of the inhibitory effects of hemoglobin F and hemoglobin A2 on the polymerization of hemoglobin S,” Proceedings of the National Academy of Sciences of the United States of America, vol. 76, no. 2, pp. 670–672, 1979. View at Google Scholar · View at Scopus
  9. C. T. Noguchi, D. A. Torchia, and A. N. Schechter, “Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity,” The Journal of Clinical Investigation, vol. 72, no. 3, pp. 846–852, 1983. View at Google Scholar · View at Scopus
  10. C. Brugnara, H. F. Bunn, and D. C. Tosteson, “Regulation of erythrocyte cation and water content in sickle cell anemia,” Science, vol. 232, no. 4748, pp. 388–390, 1986. View at Google Scholar · View at Scopus
  11. M. P. Westerman, L. Unger, O. Kucuk, P. Quinn, and L. J. Lis, “Phase changes in membrane lipids in sickle red cell shed-vesicles and sickle red cells,” American Journal of Hematology, vol. 58, no. 3, pp. 177–182, 1998. View at Google Scholar
  12. R. P. Hebbel, M. A. B. Boogaerts, J. W. Eaton, and M. H. Steinberg, “Erythrocyte adherence to endothelium in sickle-cell anemia. A possible determinant of disease severity,” The New England Journal of Medicine, vol. 302, no. 18, pp. 992–995, 1980. View at Google Scholar · View at Scopus
  13. P. S. Frenette, “Sickle cell vasoocclusion: heterotypic, multicellular aggregations driven by leukocyte adhesion,” Microcirculation, vol. 11, no. 2, pp. 167–177, 2004. View at Publisher · View at Google Scholar · View at Scopus
  14. I. Okpala, “Leukocyte adhesion and the pathophysiology of sickle cell disease,” Current Opinion in Hematology, vol. 13, no. 1, pp. 40–44, 2006. View at Google Scholar · View at Scopus
  15. K. I. Ataga and E. P. Orringer, “Hypercoagulability in sickle cell disease: a curious paradox,” American Journal of Medicine, vol. 115, no. 9, pp. 721–728, 2003. View at Publisher · View at Google Scholar · View at Scopus
  16. J.-L. Wautier and M.-P. Wautier, “Erythrocytes and platelet adhesion to endothelium are mediated by specialized molecules,” Clinical Hemorheology and Microcirculation, vol. 30, no. 3-4, pp. 181–184, 2004. View at Google Scholar · View at Scopus
  17. J. Villagra, S. Shiva, L. A. Hunter, R. F. Machado, M. T. Gladwin, and G. J. Kato, “Platelet activation in patients with sickle disease, hemolysis-associated pulmonary hypertension, and nitric oxide scavenging by cell-free hemoglobin,” Blood, vol. 110, no. 6, pp. 2166–2172, 2007. View at Publisher · View at Google Scholar · View at Scopus
  18. M. T. Gladwin and G. J. Kato, “Hemolysis-associated hypercoagulability in sickle cell disease: the plot (and blood) thickens!,” Haematologica, vol. 93, no. 1, pp. 1–3, 2008. View at Publisher · View at Google Scholar · View at Scopus
  19. C. D. Reiter, X. Wang, J. E. Tanus-Santos et al., “Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease,” Nature Medicine, vol. 8, no. 12, pp. 1383–1389, 2002. View at Publisher · View at Google Scholar · View at Scopus
  20. M. de Montalembert, M. Guilloud-Bataille, J. Feingold, and R. Girot, “Epidemiological and clinical study of sickle cell disease in France, French Guiana and Algeria,” European Journal of Haematology, vol. 51, no. 3, pp. 136–140, 1993. View at Google Scholar · View at Scopus
  21. J. R. Pattison, S. E. Jones, J. Hodgson et al., “Parvovirus infections and hypoplastic crisis in sickle-cell anaemia,” The Lancet, vol. 1, no. 8221, pp. 664–665, 1981. View at Google Scholar · View at Scopus
  22. O. S. Platt, B. D. Thorington, D. J. Brambilla et al., “Pain in sickle cell disease—rates and risk factors,” The New England Journal of Medicine, vol. 325, no. 1, pp. 11–16, 1991. View at Google Scholar · View at Scopus
  23. F. M. Gill, L. A. Sleeper, S. J. Weiner et al., “Clinical events in the first decade in a cohort of infants with sickle cell disease,” Blood, vol. 86, no. 2, pp. 776–783, 1995. View at Google Scholar · View at Scopus
  24. T. B. West, D. W. West, and K. Ohene-Frempong, “The presentation, frequency, and outcome of bacteremia among children with sickle cell disease and fever,” Pediatric Emergency Care, vol. 10, no. 3, pp. 141–143, 1994. View at Publisher · View at Google Scholar · View at Scopus
  25. K. J. J. Wierenga, I. R. Hambleton, R. M. Wilson, H. Alexander, B. E. Serjeant, and G. R. Serjeant, “Significance of fever in Jamaican patients with homozygous sickle cell disease,” Archives of Disease in Childhood, vol. 84, no. 2, pp. 156–159, 2001. View at Publisher · View at Google Scholar · View at Scopus
  26. M. H. Gaston, J. I. Verter, and G. Woods, “Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial,” The New England Journal of Medicine, vol. 314, no. 25, pp. 1593–1599, 1986. View at Google Scholar · View at Scopus
  27. “American Academy of Pediatrics. Committee on Infectious Diseases. Policy statement: recommendations for the prevention of pneumococcal infections, including the use of pneumococcal conjugate vaccine (Prevnar), pneumococcal polysaccharide vaccine, and antibiotic prophylaxis,” Pediatrics, vol. 106, no. 2, part 1, pp. 362–366, 2000.
  28. M. E. Kizito, E. Mworozi, C. Ndugwa, and G. R. Serjeant, “Bacteraemia in homozygous sickle cell disease in Africa: is pneumococcal prophylaxis justified?” Archives of Disease in Childhood, vol. 92, no. 1, pp. 21–23, 2007. View at Publisher · View at Google Scholar · View at Scopus
  29. T. N. Williams, S. Uyoga, A. Macharia et al., “Bacteraemia in Kenyan children with sickle-cell anaemia: a retrospective cohort and case-control study,” The Lancet, vol. 374, no. 9698, pp. 1364–1370, 2009. View at Publisher · View at Google Scholar · View at Scopus
  30. S. Obaro, “Pneumococcal infections and sickle cell disease in Africa: does absence of evidence imply evidence of absence?” Archives of Disease in Childhood, vol. 94, no. 9, pp. 713–716, 2009. View at Publisher · View at Google Scholar · View at Scopus
  31. L. Tshilolo, E. Kafando, M. Sawadogo et al., “Neonatal screening and clinical care programmes for sickle cell disorders in sub-Saharan Africa: lessons from pilot studies,” Public Health, vol. 122, no. 9, pp. 933–941, 2008. View at Publisher · View at Google Scholar · View at Scopus
  32. O. Bagasra, R. M. Steiner, and S. K. Ballas, “Viral burden and disease progression in HIV-1-infected patients with sickle cell anemia,” American Journal of Hematology, vol. 59, no. 3, pp. 199–207, 1998. View at Google Scholar
  33. I. Diagne, G. M. Soares, A. Gueye et al., “Infections in Senegalese children and adolescents with sickle cell anemia: epidemiological aspects,” Dakar Médical, vol. 45, no. 1, pp. 55–58, 2000. View at Google Scholar
  34. M. Hassan, S. Hasan, S. Giday et al., “Hepatitis C virus in sickle cell disease,” Journal of the National Medical Association, vol. 95, no. 10, pp. 939–942, 2003. View at Google Scholar
  35. L. M. Tshilolo, R. K. Mukendi, and S. O. Wembonyama, “Blood transfusion rate in congolese patients with sickle cell anemia,” Indian Journal of Pediatrics, vol. 74, no. 8, pp. 735–738, 2007. View at Publisher · View at Google Scholar · View at Scopus
  36. F. Blei and D. R. Puder, “Yersinia enterocolitica bacteremia in a chronically transfused patient with sickle cell anemia: case report and review of the literature,” American Journal of Pediatric Hematology/Oncology, vol. 15, no. 4, pp. 430–434, 1993. View at Google Scholar · View at Scopus
  37. E. Barrett-Connor, “Bacterial infection and sickle cell anemia. An analysis of 250 infections in 166 patients and a review of the literature,” Medicine, vol. 50, no. 2, pp. 97–112, 1971. View at Google Scholar · View at Scopus
  38. G. R. Serjeant, B. E. Serjeant, P. W. Thomas, M. J. Anderson, G. Patou, and J. R. Pattison, “Human parvovirus infection in homozygous sickle cell disease,” The Lancet, vol. 341, no. 8855, pp. 1237–1240, 1993. View at Publisher · View at Google Scholar · View at Scopus
  39. K. Smith-Whitley, H. Zhao, R. L. Hodinka et al., “Epidemiology of human parvovirus B19 in children with sickle cell disease,” Blood, vol. 103, no. 2, pp. 422–427, 2004. View at Publisher · View at Google Scholar · View at Scopus
  40. P. H. Jones, L. C. Pickett, M. J. Anderson, and G. Pasvol, “Human parvovirus infection in children and severe anaemia seen in an area endemic for malaria,” Journal of Tropical Medicine and Hygiene, vol. 93, no. 1, pp. 67–70, 1990. View at Google Scholar · View at Scopus
  41. T. Teuscher, B. Baillod, and B. R. Holzer, “Prevalence of human parvovirus B19 in sickle cell disease and healthy controls,” Tropical and Geographical Medicine, vol. 43, no. 1-2, pp. 108–110, 1991. View at Google Scholar · View at Scopus
  42. J. Yeats, H. Daley, and D. Hardie, “Parvovirus B19 infection does not contribute significantly to severe anaemia in children with malaria in Malawi,” European Journal of Haematology, vol. 63, no. 4, pp. 276–277, 1999. View at Google Scholar · View at Scopus
  43. H. A. Pearson, R. P. Spencer, and E. A. Cornelius, “Functional asplenia in sickle-cell anemia,” The New England Journal of Medicine, vol. 281, no. 17, pp. 923–926, 1969. View at Google Scholar · View at Scopus
  44. A. K. Brown, L. A. Sleeper, S. T. Miller, C. H. Pegelow, F. M. Gill, and M. A. Waclawiw, “Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease,” Archives of Pediatrics and Adolescent Medicine, vol. 148, no. 8, pp. 796–804, 1994. View at Google Scholar · View at Scopus
  45. G. J. Noel, S. Katz, and P. J. Edelson, “Complement-mediated early clearance of Haemophilus influenzae type b from blood is independent of serum lytic activity,” Journal of Infectious Diseases, vol. 157, no. 1, pp. 85–90, 1988. View at Google Scholar · View at Scopus
  46. J. A. Winkelstein and R. H. Drachman, “Deficiency of pneumococcal serum opsonizing activity in sickle-cell disease,” The New England Journal of Medicine, vol. 279, no. 9, pp. 459–466, 1968. View at Google Scholar · View at Scopus
  47. S. Ruddy, L. G. Hunsicker, and K. F. Austen, “C3b inactivator of man. 3. Further purification and production of antibody to C3b INA,” Journal of Immunology, vol. 108, no. 3, pp. 657–664, 1972. View at Google Scholar · View at Scopus
  48. R. B. Johnston Jr., S. L. Newman, and A. G. Struth, “An abnormality of the alternate pathway of complement activation in sickle-cell disease,” The New England Journal of Medicine, vol. 288, no. 16, pp. 803–808, 1973. View at Google Scholar · View at Scopus
  49. S. L. Leikin, D. Gallagher, T. R. Kinney, D. Sloane, P. Klug, and W. Rida, “Mortality in children and adolescents with sickle cell disease,” Pediatrics, vol. 84, no. 3, pp. 500–508, 1989. View at Google Scholar · View at Scopus
  50. H. O. Okuonghae, M. U. Nwankwo, and E. C. Offor, “Pattern of bacteraemia in febrile children with sickle cell anaemia,” Annals of Tropical Paediatrics, vol. 13, no. 1, pp. 55–64, 1993. View at Google Scholar · View at Scopus
  51. E. W. Hook, C. G. Campbell, H. S. Weens, and B. R. Cooper, “Salmonella osteomyelitis in patients with sickle-cell anemia,” The New England Journal of Medicine, vol. 257, no. 9, pp. 403–407, 1957. View at Google Scholar
  52. W. W. Ebong, “Acute osteomyelitis in Nigerians with sickle cell disease,” Annals of the Rheumatic Diseases, vol. 45, no. 11, pp. 911–915, 1986. View at Google Scholar · View at Scopus
  53. L. Tshilolo, R. Mukendi, and R. Girot, “Sickle cell disease in south Zaire. Study of two series of 251 and 340 patients during the period 1988–1992,” Archives de Pediatrie, vol. 3, no. 2, pp. 104–111, 1996. View at Publisher · View at Google Scholar · View at Scopus
  54. O. Castro, D. J. Brambilla, B. Thorington et al., “The acute chest syndrome in sickle cell disease: incidence and risk factors,” Blood, vol. 84, no. 2, pp. 643–649, 1994. View at Google Scholar · View at Scopus
  55. E. P. Vichinsky, L. D. Neumayr, A. N. Earles et al., “Causes and outcomes of the acute chest syndrome in sickle cell disease,” The New England Journal of Medicine, vol. 342, no. 25, pp. 1855–1865, 2000. View at Publisher · View at Google Scholar · View at Scopus
  56. O. S. Platt, D. J. Brambilla, W. F. Rosse et al., “Mortality in sickle cell disease—life expectancy and risk factors for early death,” The New England Journal of Medicine, vol. 330, no. 23, pp. 1639–1644, 1994. View at Publisher · View at Google Scholar · View at Scopus
  57. R. J. Hayes, M. Beckford, Y. Grandison, K. Mason, B. E. Serjeant, and G. R. Serjeant, “The haematology of steady state homozygous sickle cell disease: frequency distributions, variation with age and sex, longitudinal observations,” British Journal of Haematology, vol. 59, no. 2, pp. 369–382, 1985. View at Google Scholar · View at Scopus
  58. M. A. F. El-Hazmi, F. A. Jabbar, F. Z. Al-Faleh, A. R. Al-Swailem, and A. S. Warsy, “The haematological, biochemical and clinical—presentation of haemoglobin S in Saudi Arabia (i). Haematological & clinical expression,” Tropical and Geographical Medicine, vol. 39, no. 2, pp. 157–162, 1987. View at Google Scholar · View at Scopus
  59. G. Akenzua, O. Akinyanju, A. Kulozik et al., “Sickle cell anaemia in Nigeria: a comparison between Benin and Lagos,” African Journal of Medicine and Medical Sciences, vol. 23, no. 2, pp. 101–107, 1994. View at Google Scholar · View at Scopus
  60. J. W. Childs, “Sickle cell disease: the clinical manifestations,” Journal of the American Osteopathic Association, vol. 95, no. 10, pp. 593–598, 1995. View at Google Scholar · View at Scopus
  61. M. G. Neonato, M. Guilloud-Bataille, P. Beauvais et al., “Acute clinical events in 299 homozygous sickle cell patients living in france,” European Journal of Haematology, vol. 65, no. 3, pp. 155–164, 2000. View at Publisher · View at Google Scholar · View at Scopus
  62. G. R. Serjeant, J. M. Topley, K. Mason et al., “Outbreak of aplastic crises in sickle cell anaemia associated with parvovirus-like agent,” The Lancet, vol. 2, no. 8247, pp. 595–597, 1981. View at Google Scholar · View at Scopus
  63. A. I. Juwah, E. U. Nlemadim, and W. Kaine, “Types of anaemic crises in paediatric patients with sickle cell anaemia seen in Enugu, Nigeria,” Archives of Disease in Childhood, vol. 89, no. 6, pp. 572–576, 2004. View at Publisher · View at Google Scholar · View at Scopus
  64. V. G. Nolan, D. F. Wyszynski, L. A. Farrer, and M. H. Steinberg, “Hemolysis-associated priapism in sickle cell disease,” Blood, vol. 106, no. 9, pp. 3264–3267, 2005. View at Publisher · View at Google Scholar · View at Scopus
  65. G. J. Kato, V. McGowan, R. F. Machado et al., “Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with sickle cell disease,” Blood, vol. 107, no. 6, pp. 2279–2285, 2006. View at Publisher · View at Google Scholar · View at Scopus
  66. S. K. Ballas and M. J. Marcolina, “Hyperhemolysis during the evolution of uncomplicated acute painful episodes in patients with sickle cell anemia,” Transfusion, vol. 46, no. 1, pp. 105–110, 2006. View at Publisher · View at Google Scholar · View at Scopus
  67. J. G. Taylor VI, V. G. Nolan, L. Mendelsohn, G. J. Kato, M. T. Gladwin, and M. H. Steinberg, “Chronic hyper-hemolysis in sickle cell anemia: association of vascular complications and mortality with less frequent vasoocclusive pain,” PLoS One, vol. 3, no. 5, Article ID e2095, 2008. View at Publisher · View at Google Scholar · View at Scopus
  68. J. O. Olabode and W. A. Shokunbi, “Types of crises in sickle cell disease patients presenting at the haematology day care unit (HDCU), University College Hospital (UCH), Ibadan,” West African Journal of Medicine, vol. 25, no. 4, pp. 284–288, 2006. View at Google Scholar · View at Scopus
  69. C. T. Quinn, E. P. Shull, N. Ahmad, N. J. Lee, Z. R. Rogers, and G. R. Buchanan, “Prognostic significance of early vaso-occlusive complications in children with sickle cell anemia,” Blood, vol. 109, no. 1, pp. 40–45, 2007. View at Publisher · View at Google Scholar · View at Scopus
  70. J. M. Topley, D. W. Rogers, M. C. G. Stevens, and G. R. Serjeant, “Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease,” Archives of Disease in Childhood, vol. 56, no. 10, pp. 765–769, 1981. View at Google Scholar · View at Scopus
  71. A. M. Emond, R. Collis, and D. Darvill, “Acute splenic sequestration in homozygous sickle cell disease: natural history and management,” Journal of Pediatrics, vol. 107, no. 2, pp. 201–206, 1985. View at Google Scholar · View at Scopus
  72. M. Koshy, R. Entsuah, A. Koranda et al., “Leg ulcers in patients with sickle cell disease,” Blood, vol. 74, no. 4, pp. 1403–1408, 1989. View at Google Scholar · View at Scopus
  73. M. A. Durosinmi, S. M. Gevao, and G. J. Esan, “Chronic leg ulcers in sickle cell disease: experience in Ibadan, Nigeria,” African Journal of Medicine and Medical Sciences, vol. 20, no. 1, pp. 11–14, 1991. View at Google Scholar · View at Scopus
  74. A. D. Gbadoé, A. Géraldo, K. Guédénon, S. Koffi, K. Agbétiafa, and P. Akpako, “Stuttering priapism in children with sickle cell anemia in Togo,” Archives de Pediatrie, vol. 14, no. 7, pp. 861–863, 2007. View at Publisher · View at Google Scholar · View at Scopus
  75. K. Ohene-Frempong, S. J. Weiner, L. A. Sleeper et al., “Cerebrovascular accidents in sickle cell disease: rates and risk factors,” Blood, vol. 91, no. 1, pp. 288–294, 1998. View at Google Scholar · View at Scopus
  76. M. R. DeBaun, J. Schatz, M. J. Siegel et al., “Cognitive screening examinations for silent cerebral infarcts in sickle cell disease,” Neurology, vol. 50, no. 6, pp. 1678–1682, 1998. View at Google Scholar · View at Scopus
  77. T. R. Kinney, L. A. Sleeper, W. C. Wang et al., “Silent cerebral infarcts in sickle cell anemia: a risk factor analysis,” Pediatrics, vol. 103, no. 3, pp. 640–645, 1999. View at Publisher · View at Google Scholar · View at Scopus
  78. S. T. Miller, E. A. Macklin, C. H. Pegelow et al., “Silent infarction as a risk factor for overt stroke in children with sickle cell anemia: a report from the Cooperative Study of Sickle Cell Disease,” Journal of Pediatrics, vol. 139, no. 3, pp. 385–390, 2001. View at Publisher · View at Google Scholar · View at Scopus
  79. R. Marouf, R. Gupta, M. Z. Haider, and A. D. Adekile, “Silent brain infarcts in adult Kuwaiti sickle cell disease patients,” American Journal of Hematology, vol. 73, no. 4, pp. 240–243, 2003. View at Publisher · View at Google Scholar · View at Scopus
  80. R. J. Hayes, P. I. Condon, and G. R. Serjeant, “Haematological factors associated with proliferative retinopathy in sickle cell-haemoglobin C disease,” British Journal of Ophthalmology, vol. 65, no. 10, pp. 712–717, 1981. View at Google Scholar · View at Scopus
  81. O. Castro, M. Hoque, and B. D. Brown, “Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival,” Blood, vol. 101, no. 4, pp. 1257–1261, 2003. View at Publisher · View at Google Scholar · View at Scopus
  82. M. T. Gladwin, V. Sachdev, M. L. Jison et al., “Pulmonary hypertension as a risk factor for death in patients with sickle cell disease,” The New England Journal of Medicine, vol. 350, no. 9, pp. 886–895, 2004. View at Publisher · View at Google Scholar · View at Scopus
  83. K. I. Ataga, C. G. Moore, S. Jones et al., “Pulmonary hypertension in patients with sickle cell disease: a longitudinal study,” British Journal of Haematology, vol. 134, no. 1, pp. 109–115, 2006. View at Google Scholar
  84. G. J. Kato, O. C. Onyekwere, and M. T. Gladwin, “Pulmonary hypertension in sickle cell disease: relevance to children,” Pediatric Hematology and Oncology, vol. 24, no. 3, pp. 159–170, 2007. View at Publisher · View at Google Scholar · View at Scopus
  85. J. Griffiths, “Avascular necrosis of femoral head in Kenyan africans,” East African Medical Journal, vol. 45, no. 9, pp. 613–618, 1968. View at Google Scholar · View at Scopus
  86. W. W. Ebong, “Avascular necrosis of the femoral head associated with haemoglobinopathy,” Tropical and Geographical Medicine, vol. 29, no. 1, pp. 19–23, 1977. View at Google Scholar · View at Scopus
  87. R. E. J. Lee, J. S. R. Golding, and G. R. Serjeant, “The radiological features of avascular necrosis of the femoral head in homozygous sicke cell disease,” Clinical Radiology, vol. 32, no. 2, pp. 205–214, 1981. View at Google Scholar · View at Scopus
  88. K. C. Abbott, I. O. Hypolite, and L. Y. Agodoa, “Sickle cell nephropathy at end-stage renal disease in the United States: patient characteristics and survival,” Clinical Nephrology, vol. 58, no. 1, pp. 9–15, 2002. View at Google Scholar · View at Scopus
  89. A. F. Fleming, J. Storey, L. Molineaux, E. A. Iroko, and E. D. Attai, “Abnormal haemoglobins in the Sudan savanna of Nigeria. I. Prevalence of haemoglobins and relationships between sickle cell trait, malaria and survival,” Annals of Tropical Medicine and Parasitology, vol. 73, no. 2, pp. 161–172, 1979. View at Google Scholar · View at Scopus
  90. A. F. Fleming, “The presentation, management and prevention of crisis in sickle cell disease in Africa,” Blood Reviews, vol. 3, no. 1, pp. 18–28, 1989. View at Google Scholar · View at Scopus
  91. G. D. Overturf, D. Powars, and L. J. Baraff, “Bacterial meningitis and septicemia in sickle cell disease,” American Journal of Diseases of Children, vol. 131, no. 7, pp. 784–787, 1977. View at Google Scholar · View at Scopus
  92. P. J. Campbell, P. O. Olatunji, K. E. Ryan, and S. C. Davies, “Splenic regrowth in sickle cell anaemia following hypertransfusion,” British Journal of Haematology, vol. 96, no. 1, pp. 77–79, 1997. View at Publisher · View at Google Scholar · View at Scopus
  93. A. Yardumian and C. Crawley, “Sickle cell disease,” Clinical Medicine, vol. 1, no. 6, pp. 441–446, 2001. View at Google Scholar · View at Scopus
  94. M. H. Steinberg, “Pathophysiology of sickle cell disease,” Bailliere's Clinical Haematology, vol. 11, no. 1, pp. 163–184, 1998. View at Publisher · View at Google Scholar · View at Scopus
  95. H. F. Bunn, “Pathogenesis and treatment of sickle cell disease,” The New England Journal of Medicine, vol. 337, no. 11, pp. 762–769, 1997. View at Publisher · View at Google Scholar · View at Scopus
  96. D. Labie, J. Pagnier, C. Lapoumeroulie et al., “Common haplotype dependency of high (G)γ-globin gene expression and high Hb F levels in β-thalasssemia and sickle cell anemia patients,” Proceedings of the National Academy of Sciences of the United States of America, vol. 82, no. 7, pp. 2111–2114, 1985. View at Google Scholar · View at Scopus
  97. L. E. Creary, P. Ulug, S. Menzel et al., “Genetic variation on chromosome 6 influences F cell levels in healthy individuals of African descent and HbF levels in sickle cell patients,” PLoS One, vol. 4, no. 1, Article ID e4218, 2009. View at Publisher · View at Google Scholar · View at Scopus
  98. M. Uda, R. Galanello, S. Sanna et al., “Genome-wide association study shows BCL11A associated with persistent fetal hemoglobin and amelioration of the phenotype of β-thalassemia,” Proceedings of the National Academy of Sciences of the United States of America, vol. 105, no. 5, pp. 1620–1625, 2008. View at Publisher · View at Google Scholar · View at Scopus
  99. G. Lettre, V. G. Sankaran, M. A. C. Bezerra et al., “DNA polymorphisms at the BCL11A, HBS1L-MYB, and β-globin loci associate with fetal hemoglobin levels and pain crises in sickle cell disease,” Proceedings of the National Academy of Sciences of the United States of America, vol. 105, no. 33, pp. 11869–11874, 2008. View at Publisher · View at Google Scholar · View at Scopus
  100. A. E. Sedgewick, N. Timofeev, P. Sebastiani et al., “BCL11A is a major HbF quantitative trait locus in three different populations with β-hemoglobinopathies,” Blood Cells, Molecules, and Diseases, vol. 41, no. 3, pp. 255–258, 2008. View at Publisher · View at Google Scholar · View at Scopus
  101. S. L. Thein and S. Menzel, “Discovering the genetics underlying foetal haemoglobin production in adults,” British Journal of Haematology, vol. 145, no. 4, pp. 455–467, 2009. View at Publisher · View at Google Scholar · View at Scopus
  102. J. Makani, S. Menzel, S. Nkya et al., “Genetics of fetal hemoglobin in Tanzanian and British patients with sickle cell anemia,” Blood, vol. 117, no. 4, pp. 1390–1392, 2011. View at Publisher · View at Google Scholar · View at Scopus
  103. World Health Organisation, “Sickle cell anaemia. Agenda item 11.4,” in 59th World Health Assembly, 27 May 2006, World Health Organisation, Geneva, Switzerland, 2006. View at Google Scholar
  104. D. Diallo and G. Tchernia, “Sickle cell disease in Africa,” Current Opinion in Hematology, vol. 9, no. 2, pp. 111–116, 2002. View at Publisher · View at Google Scholar · View at Scopus
  105. D. J. Weatherall, O. Akinyanju, S. Fucharoen, N. F. Olivieri, and P. Musgrove, “Inherited disorders of hemoglobin,” in Disease Control Priorities in Developing Countries, D. Jamison, Ed., pp. 663–680, Oxford University Press, New York, NY, USA, 2006. View at Google Scholar
  106. D. J. Weatherall, “Hemoglobinopathies worldwide: present and future,” Current Molecular Medicine, vol. 8, no. 7, pp. 592–599, 2008. View at Publisher · View at Google Scholar · View at Scopus
  107. A. Enevold, J. P. Lusingu, B. Mmbando et al., “Reduced risk of uncomplicated malaria episodes in children with alpha+-thalassemia in Northeastern Tanzania,” American Journal of Tropical Medicine and Hygiene, vol. 78, no. 5, pp. 714–720, 2008. View at Google Scholar · View at Scopus
  108. T. N. Williams, S. Wambua, S. Uyoga et al., “Both heterozygous and homozygous α+ thalassemias protect against severe and fatal Plasmodium falciparum malaria on the coast of Kenya,” Blood, vol. 106, no. 1, pp. 368–371, 2005. View at Publisher · View at Google Scholar · View at Scopus
  109. M. Aidoo, D. J. Terlouw, M. S. Kolczak et al., “Protective effects of the sickle cell gene against malaria morbidity and mortality,” The Lancet, vol. 359, no. 9314, pp. 1311–1312, 2002. View at Publisher · View at Google Scholar · View at Scopus
  110. D. J. Weatherall and J. B. Clegg, “Inherited haemoglobin disorders: an increasing global health problem,” Bulletin of the World Health Organization, vol. 79, no. 8, pp. 704–712, 2001. View at Google Scholar · View at Scopus
  111. D. Modiano, G. Bancone, B. M. Ciminelli et al., “Haemoglobin S and haemoglobin C: ‘quick but costly’ versus ‘slow but gratis’ genetic adaptations to Plasmodium falciparum malaria,” Human Molecular Genetics, vol. 17, no. 6, pp. 789–799, 2008. View at Publisher · View at Google Scholar · View at Scopus
  112. J. Simpore, S. Pignatelli, S. Barlati, and S. Musumeci, “Modification in the frequency of Hb C and Hb S in Burkina Faso: an influence of migratory fluxes and improvement of patient health care,” Hemoglobin, vol. 26, no. 2, pp. 113–120, 2002. View at Publisher · View at Google Scholar · View at Scopus
  113. P. Beighton and M. C. Botha, “Inherited disorders in the black population of southern Africa—part I: historical and demographic background; genetic haematological conditions,” South African Medical Journal, vol. 69, no. 4, pp. 247–249, 1986. View at Google Scholar · View at Scopus
  114. World Health Organisation, Genomics and World Health, Report of the Advisory Committee on Health Research, World Health Organisation, Geneva, Switzerland, 2002.
  115. L. Molineaux, A. F. Fleming, and R. Cornille-Brogger, “Abnormal haemoglobins in the Sudan savanna of Nigeria. III. Malaria immunoglobulins and antimalarial antibodies in sickle cell disease,” Annals of Tropical Medicine and Parasitology, vol. 73, no. 4, pp. 301–310, 1979. View at Google Scholar · View at Scopus
  116. C. T. Quinn, Z. R. Rogers, and G. R. Buchanan, “Survival of children with sickle cell disease,” Blood, vol. 103, no. 11, pp. 4023–4027, 2004. View at Publisher · View at Google Scholar · View at Scopus
  117. P. Telfer, P. Coen, S. Chakravorty et al., “Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London,” Haematologica, vol. 92, no. 7, pp. 905–912, 2007. View at Publisher · View at Google Scholar · View at Scopus
  118. A. N. Thomas, C. Pattison, and G. R. Serjeant, “Causes of death in sickle-cell disease in Jamaica,” British Medical Journal, vol. 285, no. 6342, pp. 633–635, 1982. View at Google Scholar · View at Scopus
  119. M. Brozovic and E. Anionwu, “Sickle cell disease in Britain,” Journal of Clinical Pathology, vol. 37, no. 12, pp. 1321–1326, 1984. View at Google Scholar · View at Scopus
  120. V. G. Sankaran and M. V. Sapp, “Persistence of fetal hemoglobin expression in an older child with trisomy 13,” Journal of Pediatrics, vol. 160, no. 2, p. 352, 2012. View at Publisher · View at Google Scholar · View at Scopus
  121. A. Lee, P. Thomas, L. Cupidore, B. Serjeant, and G. Serjeant, “Improved survival in homozygous sickle cell disease: lessons from a cohort study,” British Medical Journal, vol. 311, no. 7020, pp. 1600–1602, 1995. View at Google Scholar · View at Scopus
  122. E. Vichinsky, D. Hurst, A. Earles, K. Kleman, and B. Lubin, “Newborn screening for sickle cell disease: effect on mortality,” Pediatrics, vol. 81, no. 6, pp. 749–755, 1988. View at Google Scholar · View at Scopus
  123. T. Frempong and H. A. Pearson, “Newborn screening coupled with comprehensive follow-up reduced early mortality of sickle cell disease in Connecticut,” Connecticut Medicine, vol. 71, no. 1, pp. 9–12, 2007. View at Google Scholar · View at Scopus
  124. Z. M. Al-Hawsawi and G. A. Ismail, “Acute splenic sequestration crisis in children with sickle cell disease,” Saudi Medical Journal, vol. 22, no. 12, pp. 1076–1079, 2001. View at Google Scholar · View at Scopus
  125. J. A. Wilimas, P. M. Flynn, S. Harris et al., “A randomized study of outpatient treatment with ceftriaxone for selected febrile children with sickle cell disease,” The New England Journal of Medicine, vol. 329, no. 7, pp. 472–476, 1993. View at Publisher · View at Google Scholar · View at Scopus
  126. M. C. Rahimy, A. Gangbo, G. Ahouignan, S. Anagonou, V. Boco, and E. Alihonou, “Outpatient management of fever in children with sickle cell disease (SCD) in an African setting,” American Journal of Hematology, vol. 62, no. 1, pp. 1–6, 1999. View at Google Scholar
  127. R. E. Ware, S. A. Zimmerman, and W. H. Schultz, “Hydroxyurea as an alternative to blood transfusions for the prevention of recurrent stroke in children with sickle cell disease,” Blood, vol. 94, no. 9, pp. 3022–3026, 1999. View at Google Scholar · View at Scopus
  128. M. C. Rahimy, A. Gangbo, G. Ahouignan et al., “Effect of a comprehensive clinical care program on disease course in severely ill children with sickle cell anemia in a sub-Saharan African setting,” Blood, vol. 102, no. 3, pp. 834–838, 2003. View at Publisher · View at Google Scholar · View at Scopus
  129. J. Knight-Madden and G. R. Serjeant, “Invasive pneumococcal disease in homozygous sickle cell disease: Jamaican experience 1973–1997,” Journal of Pediatrics, vol. 138, no. 1, pp. 65–70, 2001. View at Publisher · View at Google Scholar · View at Scopus
  130. J. A. Berkley, B. S. Lowe, I. Mwangi et al., “Bacteremia among children admitted to a rural hospital in Kenya,” The New England Journal of Medicine, vol. 352, no. 1, pp. 39–47, 2005. View at Publisher · View at Google Scholar · View at Scopus
  131. A. Roca, B. Sigaúque, L. Quintó et al., “Invasive pneumococcal disease in children >5 years of age in rural Mozambique,” Tropical Medicine and International Health, vol. 11, no. 9, pp. 1422–1431, 2006. View at Publisher · View at Google Scholar · View at Scopus
  132. M. de Montalembert, V. Brousse, and J.-R. Zahar, “Pneumococcal prophylaxis for children with sickle cell disease in Africa,” Archives of Disease in Childhood, vol. 93, no. 8, pp. 715–716, 2008. View at Google Scholar · View at Scopus
  133. J. R. Aluoch, “Higher resistance to Plasmodium falciparum infection in patients with homozygous sickle cell disease in Western Kenya,” Tropical Medicine and International Health, vol. 2, no. 6, pp. 568–571, 1997. View at Google Scholar · View at Scopus
  134. H. O. Okuonghae, M. U. Nwankwo, and E. Offor, “Brief reports malarial parasitaemia in febrile children with sickle cell anaemia,” Journal of Tropical Pediatrics, vol. 38, no. 2, pp. 83–85, 1992. View at Google Scholar · View at Scopus
  135. R. Kotila, A. Okesola, and O. Makanjuola, “Asymptomatic malaria parasitaemia in sickle-cell disease patients: how effective is chemoprophylaxis?” Journal of Vector Borne Diseases, vol. 44, no. 1, pp. 52–55, 2007. View at Google Scholar · View at Scopus
  136. O. Awotua-Efebo, E. A. Alikor, and K. E. Nkanginieme, “Malaria parasite density and splenic status by ultrasonography in stable sickle-cell anaemia (HbSS) children,” Nigerian Journal of Medicine, vol. 13, no. 1, pp. 40–43, 2004. View at Google Scholar · View at Scopus
  137. J. Makani, A. N. Komba, S. E. Cox et al., “Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization,” Blood, vol. 115, no. 2, pp. 215–220, 2010. View at Publisher · View at Google Scholar · View at Scopus
  138. O. Oniyangi and A. A. Omari, “Malaria chemoprophylaxis in sickle cell disease,” Cochrane Database of Systematic Reviews, no. 4, Article ID CD003489, 2006. View at Google Scholar · View at Scopus
  139. S. Wahl and K. C. Quirolo, “Current issues in blood transfusion for sickle cell disease,” Current Opinion in Pediatrics, vol. 21, no. 1, pp. 15–21, 2009. View at Publisher · View at Google Scholar · View at Scopus
  140. R. J. Adams, V. C. McKie, L. Hsu et al., “Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography,” The New England Journal of Medicine, vol. 339, no. 1, pp. 5–11, 1998. View at Google Scholar
  141. J. M. Turner, J. B. Kaplan, H. W. Cohen, and H. H. Billett, “Exchange versus simple transfusion for acute chest syndrome in sickle cell anemia adults,” Transfusion, vol. 49, no. 5, pp. 863–868, 2009. View at Publisher · View at Google Scholar · View at Scopus
  142. E. P. Vichinsky, C. M. Haberkern, L. Neumayr et al., “A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease,” The New England Journal of Medicine, vol. 333, no. 4, pp. 206–213, 1995. View at Publisher · View at Google Scholar · View at Scopus
  143. K. A. Stegenga, P. Ward-Smith, P. S. Hinds, J. A. Routhieaux, and G. M. Woods, “Quality of life among children with sickle cell disease receiving chronic transfusion therapy,” Journal of Pediatric Oncology Nursing, vol. 21, no. 4, pp. 207–213, 2004. View at Publisher · View at Google Scholar · View at Scopus
  144. R. Prasad, S. Hasan, O. Castro, E. Perlin, and K. Kim, “Long-term outcomes in patients with sickle cell disease and frequent vaso-occlusive crises,” American Journal of the Medical Sciences, vol. 325, no. 3, pp. 107–109, 2003. View at Publisher · View at Google Scholar · View at Scopus
  145. M. J. Telen, “Principles and problems of transfusion in sickle cell disease,” Seminars in Hematology, vol. 38, no. 4, pp. 315–323, 2001. View at Google Scholar · View at Scopus
  146. K. Ohene-Frempong, “Indications for red cell transfusion in sickle cell disease,” Seminars in Hematology, vol. 38, no. 1, supplement 1, pp. 5–13, 2001. View at Google Scholar · View at Scopus
  147. R. J. Dunlop and K. C. Bennett, “Pain management for sickle cell disease,” Cochrane Database of Systematic Reviews, no. 2, Article ID CD003350, 2006. View at Google Scholar · View at Scopus
  148. D. C. Rees, A. D. Olujohungbe, N. E. Parker, A. D. Stephens, P. Telfer, and J. Wright, “Guidelines for the management of the acute painful crisis in sickle cell disease,” British Journal of Haematology, vol. 120, no. 5, pp. 744–752, 2003. View at Publisher · View at Google Scholar · View at Scopus
  149. S. K. Ballas, “Pain management of sickle cell disease,” Hematology/Oncology Clinics of North America, vol. 19, no. 5, pp. 785–802, 2005. View at Publisher · View at Google Scholar · View at Scopus
  150. S. Charache, M. L. Terrin, R. D. Moore et al., “Effect of hydroxyurea on the frequency of painful crises in Sickle cell anemia,” The New England Journal of Medicine, vol. 332, no. 20, pp. 1317–1322, 1995. View at Publisher · View at Google Scholar · View at Scopus
  151. R. E. Ware, M. H. Steinberg, and T. R. Kinney, “Hydroxyurea: an alternative to transfusion therapy for stroke in sickle cell anemia,” American Journal of Hematology, vol. 50, no. 2, pp. 140–143, 1995. View at Publisher · View at Google Scholar · View at Scopus
  152. National Institutes of Health, National Institutes of Health: Consensus Development Conference Statement: Hydroxyurea Treatment for Sickle Cell Disease, National Institutes of Health, 2008.
  153. W. H. Waugh, C. W. Daeschner III, B. A. Files, M. E. McConnell, and S. E. Strandjord, “Oral citrulline as arginine precursor may be beneficial in sickle cell disease: early phase two results,” Journal of the National Medical Association, vol. 93, no. 10, pp. 363–371, 2001. View at Google Scholar · View at Scopus
  154. C. R. Morris, E. P. Vichinsky, J. van Warmerdam et al., “Hydroxyurea and arginine therapy: impact on nitric oxide production in sickle cell disease,” Journal of Pediatric Hematology/Oncology, vol. 25, no. 8, pp. 629–634, 2003. View at Publisher · View at Google Scholar · View at Scopus
  155. M. Oppert, A. Jörres, D. Barckow, K.-U. Eckardt, U. Frei, and U. Kaisers, “Inhaled nitric oxide for ARDS due to sickle cell disease,” Swiss Medical Weekly, vol. 134, no. 11-12, pp. 165–167, 2004. View at Google Scholar · View at Scopus
  156. D. L. Weiner and C. Brugnara, “Hydroxyurea and sickle cell disease: a chance for every patient,” Journal of the American Medical Association, vol. 289, no. 13, pp. 1692–1694, 2003. View at Publisher · View at Google Scholar · View at Scopus
  157. M. T. Gladwin, J. H. Shelhamer, F. P. Ognibene et al., “Nitric oxide donor properties of hydroxyurea in patients with sickle cell disease,” British Journal of Haematology, vol. 116, no. 2, pp. 436–444, 2002. View at Publisher · View at Google Scholar · View at Scopus
  158. F. L. Johnson, A. T. Look, and J. Gockerman, “Bone-marrow transplantation in a patient with sickle-cell anemia,” The New England Journal of Medicine, vol. 311, no. 12, pp. 780–783, 1984. View at Google Scholar · View at Scopus
  159. M. C. Walters, R. Storb, M. Patience et al., “Impact of bone marrow transplantation for symptomatic sickle cell disease: an interim report,” Blood, vol. 95, no. 6, pp. 1918–1924, 2000. View at Google Scholar · View at Scopus
  160. L. Krishnamurti, S. Abel, M. Maiers, and S. Flesch, “Availability of unrelated donors for hematopoietic stem cell transplantation for hemoglobinopathies,” Bone Marrow Transplantation, vol. 31, no. 7, pp. 547–550, 2003. View at Publisher · View at Google Scholar · View at Scopus
  161. P. Woodard, B. Lubin, and M. C. Walters, “New approaches to hematopoietic cell transplantation for hematological diseases in children,” Pediatric Clinics of North America, vol. 49, no. 5, pp. 989–1007, 2002. View at Publisher · View at Google Scholar · View at Scopus
  162. T. V. Adamkiewicz, P. S. Mehta, M. W. Boyer et al., “Transplantation of unrelated placental blood cells in children with high-risk sickle cell disease,” Bone Marrow Transplantation, vol. 34, no. 5, pp. 405–411, 2004. View at Publisher · View at Google Scholar · View at Scopus
  163. R. Pawliuk, K. A. Westerman, M. E. Fabry et al., “Correction of sickle cell disease in transgenic mouse models by gene therapy,” Science, vol. 294, no. 5550, pp. 2368–2371, 2001. View at Publisher · View at Google Scholar · View at Scopus
  164. World Health Organisation, Guidelines for the Control of Haemoglobin Disorders, World Health Organisation, Geneva, Switzerland, 1994.
  165. A. Alwan and B. Modell, “Recommendations for introducing genetics services in developing countries,” Nature Reviews Genetics, vol. 4, no. 1, pp. 61–68, 2003. View at Publisher · View at Google Scholar · View at Scopus
  166. A. Wonkam, A. K. Njamnshi, and F. F. Angwafo III, “Knowledge and attitudes concerning medical genetics amongst physicians and medical students in Cameroon (sub-Saharan Africa),” Genetics in Medicine, vol. 8, no. 6, pp. 331–338, 2006. View at Publisher · View at Google Scholar · View at Scopus
  167. M. A. Durosinmi, A. I. Odebiyi, I. A. Adediran, N. O. Akinola, D. E. Adegorioye, and M. A. Okunade, “Acceptability of prenatal diagnosis of sickle cell anaemia (SCA) by female patients and parents of SCA patients in Nigeria,” Social Science and Medicine, vol. 41, no. 3, pp. 433–436, 1995. View at Publisher · View at Google Scholar · View at Scopus
  168. A. S. Adeyemi and D. A. Adekanle, “Knowledge and attitude of female health workers towards prenatal diagnosis of sickle cell disease,” Nigerian Journal of Medicine, vol. 16, no. 3, pp. 268–270, 2007. View at Google Scholar · View at Scopus
  169. O. O. Akinyanju, R. F. Disu, J. A. Akinde, T. A. Adewole, A. I. Otaigbe, and E. E. Emuveyan, “Initiation of prenatal diagnosis of sickle-cell disorders in Africa,” Prenatal Diagnosis, vol. 19, no. 4, pp. 299–304, 1999. View at Google Scholar
  170. J. Xu, C. Peng, V. G. Sankaran et al., “Correction of sickle cell disease in adult mice by interference with fetal hemoglobin silencing,” Science, vol. 334, no. 6058, pp. 993–996, 2011. View at Publisher · View at Google Scholar · View at Scopus
  171. World Health Organisation, Sickle Cell Disease: A Strategy for the WHO Africa Region, R.o.t.R. Director, 2010.
  172. D. J. Weatherall, “Genomics and global health: time for a reappraisal,” Science, vol. 302, no. 5645, pp. 597–599, 2003. View at Publisher · View at Google Scholar · View at Scopus
  173. D. Weatherall, K. Hofman, G. Rodgers, J. Ruffin, and S. Hrynkow, “A case for developing North-South partnerships for research in sickle cell disease,” Blood, vol. 105, no. 3, pp. 921–923, 2005. View at Publisher · View at Google Scholar · View at Scopus