Sickle Cell Disease: New Opportunities and Challenges in Africa

Sickle cell disease (SCD) is one of the most common genetic causes of illness and death in the world. This is a review of SCD in Africa, which bears the highest burden of disease. The first section provides an introduction to the molecular basis of SCD and the pathophysiological mechanism of selected clinical events. The second section discusses the epidemiology of the disease (prevalence, morbidity, and mortality), at global level and within Africa. The third section discusses the laboratory diagnosis and management of SCD, emphasizing strategies that been have proven to be effective in areas with limited resources. Throughout the review, specific activities that require evidence to guide healthcare in Africa, as well as strategic areas for further research, will be highlighted.

[1]  S. Ballas.  Sickle Cell Anaemia , 2012, Drugs.

[2]  V. Sankaran,et al.  Persistence of fetal hemoglobin expression in an older child with trisomy 13. , 2012, The Journal of pediatrics.

[3]  Cong Peng,et al.  Correction of Sickle Cell Disease in Adult Mice by Interference with Fetal Hemoglobin Silencing , 2011, Science.

[4]  M. Farrall,et al.  Genetics of fetal hemoglobin in Tanzanian and British patients with sickle cell anemia. , 2011, Blood.

[5]  J. Elion,et al.  [Pathophysiology of sickle cell disease]. , 2010, Medecine tropicale : revue du Corps de sante colonial.

[6]  K. Marsh,et al.  Malaria in patients with sickle cell anemia: burden, risk factors, and outcome at the outpatient clinic and during hospitalization. , 2010, Blood.

[7]  K. Marsh,et al.  Bacteraemia in Kenyan children with sickle-cell anaemia: a retrospective cohort and case–control study , 2009, The Lancet.

[8]  Stephen K Obaro.  Pneumococcal infections and sickle cell disease in Africa: does absence of evidence imply evidence of absence? , 2009, Archives of Disease in Childhood.

[9]  H. Billett,et al.  Exchange versus simple transfusion for acute chest syndrome in sickle cell anemia adults , 2009, Transfusion.

[10]  S. Thein,et al.  Discovering the genetics underlying foetal haemoglobin production in adults , 2009, British journal of haematology.

[11]  K. Quirolo,et al.  Current issues in blood transfusion for sickle cell disease. , 2009, Current opinion in pediatrics.

[12]  M. Farrall,et al.  Genetic Variation on Chromosome 6 Influences F Cell Levels in Healthy Individuals of African Descent and HbF Levels in Sickle Cell Patients , 2009, PloS one.

[13]  P. Sebastiani,et al.  BCL11A is a major HbF quantitative trait locus in three different populations with beta-hemoglobinopathies. , 2008, Blood cells, molecules & diseases.

[14]  D. Weatherall.  Hemoglobinopathies worldwide: present and future. , 2008, Current molecular medicine.

[15]  F. Vertongen,et al.  Neonatal screening and clinical care programmes for sickle cell disorders in sub-Saharan Africa: lessons from pilot studies. , 2008, Public health.

[16]  J. Hirschhorn,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 , 2008, Proceedings of the National Academy of Sciences.

[17]  J. Zahar,et al.  Pneumococcal prophylaxis for children with sickle cell disease in Africa. , 2008, Archives of disease in childhood.

[18]  R. Macleod,et al.  How to be good at practical procedures? , 2008, Archives of disease in childhood.

[19]  B. Pollock,et al.  National Institutes of Health Consensus Development Conference statement: hydroxyurea treatment for sickle cell disease. , 2008, Annals of internal medicine.

[20]  M. Gladwin,et al.  Chronic Hyper-Hemolysis in Sickle Cell Anemia: Association of Vascular Complications and Mortality with Less Frequent Vasoocclusive Pain , 2008, PloS one.

[21]  T. Theander,et al.  Reduced risk of uncomplicated malaria episodes in children with alpha+-thalassemia in northeastern Tanzania. , 2008, The American journal of tropical medicine and hygiene.

[22]  Gonçalo R. Abecasis,et al.  Genome-wide association study shows BCL11A associated with persistent fetal hemoglobin and amelioration of the phenotype of β-thalassemia , 2008, Proceedings of the National Academy of Sciences.

[23]  M. Gladwin,et al.  Hemolysis-associated hypercoagulability in sickle cell disease: the plot (and blood) thickens! , 2008, Haematologica.

[24]  G. Modiano,et al.  Haemoglobin S and haemoglobin C: 'quick but costly' versus 'slow but gratis' genetic adaptations to Plasmodium falciparum malaria. , 2007, Human molecular genetics.

[25]  L. Tshilolo,et al.  Blood transfusion rate in congolese patients with sickle cell anemia , 2007, Indian journal of pediatrics.

[26]  M. Gladwin,et al.  Platelet activation in patients with sickle disease, hemolysis-associated pulmonary hypertension, and nitric oxide scavenging by cell-free hemoglobin. , 2007, Blood.

[27]  F. Kirkham,et al.  Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. , 2007, Haematologica.

[28]  A. Gbadoé,et al.  Priapisme intermittent chronique chez l'enfant drépanocytaire homozygote au Togo , 2007 .

[29]  A. Adeyemi,et al.  Knowledge and attitude of female healt workers towards prenatal diagnosis of sickle cell disease , 2007 .

[30]  O. Makanjuola,et al.  Asymptomatic malaria parasitaemia in sickle-cell disease patients: how effective is chemoprophylaxis? , 2007, Journal of vector borne diseases.

[31]  M. Gladwin,et al.  PULMONARY HYPERTENSION IN SICKLE CELL DISEASE: Relevance to Children , 2007, Pediatric hematology and oncology.

[32]  A. Omari,et al.  Malaria chemoprophylaxis in sickle cell disease. , 2006, The Cochrane database of systematic reviews.

[33]  W. Shokunbi,et al.  Types of crises in sickle cell disease patients presenting at the haematology day care unit (HDCU), University College Hospital (UCH), Ibadan. , 2006, West African journal of medicine.

[34]  M. Levine,et al.  Invasive pneumococcal disease in children <5 years of age in rural Mozambique , 2006, Tropical medicine & international health : TM & IH.

[35]  C. Moore,et al.  Pulmonary hypertension in patients with sickle cell disease: a longitudinal study , 2006, British journal of haematology.

[36]  A. Wonkam,et al.  Knowledge and attitudes concerning medical genetics amongst physicians and medical students in Cameroon (sub-Saharan Africa) , 2006, Genetics in Medicine.

[37]  R. J. Dunlop,et al.  Pain management for sickle cell disease. , 2006, The Cochrane database of systematic reviews.

[38]  G. Serjeant,et al.  Bacteraemia in homozygous sickle cell disease in Africa: is pneumococcal prophylaxis justified? , 2006, Archives of Disease in Childhood.

[39]  K. Blethyn,et al.  Pain management in sickle cell disease , 2006, Chronic illness.

[40]  S. Ballas,et al.  Hyperhemolysis during the evolution of uncomplicated acute painful episodes in patients with sickle cell anemia , 2006, Transfusion.

[41]  I. Okpala.  Leukocyte adhesion and the pathophysiology of sickle cell disease , 2006, Current opinion in hematology.

[42]  C. Quinn,et al.  Prognostic significance of early vaso-occlusive complications in children with sickle cell anemia. , 2005, Blood.

[43]  Mark T Gladwin,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. , 2005, Blood.

[44]  L. Farrer,et al.  Hemolysis-associated priapism in sickle cell disease. , 2005, Blood.

[45]  K. Maitland,et al.  Both heterozygous and homozygous alpha+ thalassemias protect against severe and fatal Plasmodium falciparum malaria on the coast of Kenya. , 2005, Blood.

[46]  K. Hofman,et al.  A case for developing North-South partnerships for research in sickle cell disease. , 2005, Blood.

[47]  K. Maitland,et al.  Bacteremia among children admitted to a rural hospital in Kenya. , 2005, The New England journal of medicine.

[48]  R. Nagel,et al.  Sickle-cell disease , 2004, The Lancet.

[49]  J. Wingard,et al.  Transplantation of unrelated placental blood cells in children with high-risk sickle cell disease , 2004, Bone Marrow Transplantation.

[50]  P. Hinds,et al.  Quality of Life Among Children With Sickle Cell Disease Receiving Chronic Transfusion Therapy , 2004, Journal of pediatric oncology nursing : official journal of the Association of Pediatric Oncology Nurses.

[51]  J. Krishnan,et al.  Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. , 2004, The New England journal of medicine.

[52]  C. Quinn,et al.  Survival of children with sickle cell disease. , 2004, Blood.

[53]  A. I. Juwah,et al.  Types of anaemic crises in paediatric patients with sickle cell anaemia seen in Enugu, Nigeria , 2004, Archives of Disease in Childhood.

[54]  K. Eckardt,et al.  Inhaled nitric oxide for ARDS due to sickle cell disease. , 2004, Swiss medical weekly.

[55]  P. Frenette.  Sickle Cell Vasoocclusion: Heterotypic, Multicellular Aggregations Driven by Leukocyte Adhesion , 2004, Microcirculation.

[56]  A. Cnaan,et al.  Epidemiology of human parvovirus B19 in children with sickle cell disease. , 2004, Blood.

[57]  K. Ataga,et al.  Hypercoagulability in sickle cell disease: a curious paradox. , 2003, The American journal of medicine.

[58]  D. Weatherall,et al.  Genomics and Global Health: Time for a Reappraisal , 2003, Science.

[59]  S. Giday,et al.  Hepatitis C virus in sickle cell disease. , 2003, Journal of the National Medical Association.

[60]  A. Adekile,et al.  Silent brain infarcts in adult Kuwaiti sickle cell disease patients , 2003, American journal of hematology.

[61]  E. Vichinsky,et al.  Hydroxyurea and Arginine Therapy: Impact on Nitric Oxide Production in Sickle Cell Disease , 2003, Journal of pediatric hematology/oncology.

[62]  M. C. Rahimy,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. , 2003, Blood.

[63]  C. Brugnara,et al.  Hydroxyurea and sickle cell disease: a chance for every patient. , 2003, JAMA.

[64]  M. Maiers,et al.  Availability of unrelated donors for hematopoietic stem cell transplantation for hemoglobinopathies , 2003, Bone Marrow Transplantation.

[65]  O. Castro,et al.  Long-Term Outcomes in Patients with Sickle Cell Disease and Frequent Vaso-Occlusive Crises , 2003, The American journal of the medical sciences.

[66]  Paul Telfer,et al.  Guidelines for the management of the acute painful crisis in sickle cell disease , 2003, British journal of haematology.

[67]  O. Castro,et al.  Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival. , 2003, Blood.

[68]  B. Lubin,et al.  New approaches to hematopoietic cell transplantation for hematological diseases in children. , 2002, Pediatric clinics of North America.

[69]  L. Agodoa,et al.  Sickle cell nephropathy at end-stage renal disease in the United States: patient characteristics and survival. , 2002, Clinical nephrology.

[70]  Dianne J Terlouw,et al.  Protective effects of the sickle cell gene against malaria morbidity and mortality , 2002, The Lancet.

[71]  Dapa Diallo,et al.  Sickle cell disease in Africa , 2002, Current opinion in hematology.

[72]  M. Gladwin,et al.  Nitric oxide donor properties of hydroxyurea in patients with sickle cell disease , 2002, British journal of haematology.

[73]  S. Musumeci,et al.  MODIFICATION IN THE FREQUENCY OF Hb C AND Hb S IN BURKINA FASO: AN INFLUENCE OF MIGRATORY FLUXES AND IMPROVEMENT OF PATIENT HEALTH CARE , 2002, Hemoglobin.

[74]  R. Nagel,et al.  Correction of Sickle Cell Disease in Transgenic Mouse Models by Gene Therapy , 2001, Science.

[75]  Z. M. Al-Hawsawi,et al.  Acute splenic sequestration crisis in children with sickle cell disease. , 2001, Saudi medical journal.

[76]  D. Weatherall.  Towards molecular medicine; reminiscences of the haemoglobin field, 1960–2000 , 2001, British journal of haematology.

[77]  M. Telen.  Principles and problems of transfusion in sickle cell disease. , 2001, Seminars in hematology.

[78]  B. Files,et al.  Oral citrulline as arginine precursor may be beneficial in sickle cell disease: early phase two results. , 2001, Journal of the National Medical Association.

[79]  Scott T. Miller,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. , 2001, The Journal of pediatrics.

[80]  Esther Nzewi,et al.  Malevolent ogbanje: recurrent reincarnation or sickle cell disease? , 2001, Social science & medicine.

[81]  D. J. Weatherall,et al.  Phenotype—genotype relationships in monogenic disease: lessons from the thalassaemias , 2001, Nature Reviews Genetics.

[82]  I. Hambleton,et al.  Significance of fever in Jamaican patients with homozygous sickle cell disease , 2001, Archives of disease in childhood.

[83]  J. Elion,et al.  Acute clinical events in 299 homozygous sickle cell patients living in France , 2000, European journal of haematology.

[84]  S. Dowell,et al.  Policy Statement: Recommendations for the Prevention of Pneumococcal Infections, Including the Use of Pneumococcal Conjugate Vaccine (Prevnar), Pneumococcal Polysaccharide Vaccine, and Antibiotic Prophylaxis , 2000, Pediatrics.

[85]  P. V. Ichinsky,et al.  Causes and Outcomes of the Acute Chest Syndrome in Sickle Cell Disease , 2000 .

[86]  C. Pegelow,et al.  Impact of bone marrow transplantation for symptomatic sickle cell disease: an interim report. Multicenter investigation of bone marrow transplantation for sickle cell disease. , 2000, Blood.

[87]  D. Hardie,et al.  Parvovirus B19 infection does not contribute significantly to severe anaemia in children with malaria in Malawi , 1999, European journal of haematology.

[88]  S. Anagonou,et al.  Outpatient management of fever in children with sickle cell disease (SCD) in an African setting , 1999, American journal of hematology.

[89]  O. Akinyanju,et al.  Initiation of prenatal diagnosis of sickle‐cell disorders in Africa , 1999, Prenatal diagnosis.

[90]  O. Bagasra,et al.  Viral Burden and Disease Progression in HIV‐1–Infected Patients with Sickle Cell Anemia , 1998, American journal of hematology.

[91]  Scott T. Miller,et al.  Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. , 1998, The New England journal of medicine.

[92]  L. J. Lis,et al.  Phase changes in membrane lipids in sickle red cell shed‐vesicles and sickle red cells , 1998, American journal of hematology.

[93]  J. Schatz,et al.  Cognitive screening examinations for silent cerebral infarcts in sickle cell disease , 1998, Neurology.

[94]  M. Steinberg.  6 Pathophysiology of sickle cell disease , 1998 .

[95]  H. Bunn.  Pathogenesis and treatment of sickle cell disease. , 1997, The New England journal of medicine.

[96]  J. Aluoch.  Higher resistance to Plasmodium falciparum infection in patients with homozygous sickle cell disease in western Kenya , 1997, Tropical medicine & international health : TM & IH.

[97]  S. Davies,et al.  Splenic regrowth in sickle cell anaemia following hypertransfusion , 1997, British journal of haematology.

[98]  M. Kerstein,et al.  Leg ulcers in patients with sickle cell disease. , 1996, Advances in wound care : the journal for prevention and healing.

[99]  P. Lane.  Sickle cell disease. , 1996, Pediatric clinics of North America.

[100]  R. Girot,et al.  La drépanocytose dans le sud du Zaïre. Étude de deux séries de 251 et 340 malades suivis entre 1988 et 1992 , 1996 .

[101]  G. Serjeant,et al.  Improved survival in homozygous sickle cell disease: lessons from a cohort study , 1995, BMJ.

[102]  R. Ware,et al.  Hydroxyurea: An alternative to transfusion therapy for stroke in sickle cell anemia , 1995, American journal of hematology.

[103]  J. Childs.  Sickle cell disease: The clinical manifestations , 1995, The Journal of the American Osteopathic Association.

[104]  M. Durosinmi,et al.  Acceptability of prenatal diagnosis of sickle cell anaemia (SCA) by female patients and parents of SCA patients in Nigeria. , 1995, Social science & medicine.

[105]  C. Pegelow,et al.  Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. , 1995, Blood.

[106]  M L Terrin,et al.  Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. , 1995, The New England journal of medicine.

[107]  O. Castro,et al.  The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. , 1994, Blood.

[108]  O. Platt,et al.  Mortality in sickle cell disease. Life expectancy and risk factors for early death. , 1994, The New England journal of medicine.

[109]  K. Ohene‐Frempong,et al.  The presentation, frequency, and outcome of bacteremia among children with sickle cell disease and fever , 1994, Pediatric emergency care.

[110]  A. Kulozik,et al.  Sickle cell anaemia in Nigeria: a comparison between Benin and Lagos. , 1994, African journal of medicine and medical sciences.

[111]  F. Blei,et al.  Yersinia enterocolitica bacteremia in a chronically transfused patient with sickle cell anemia. Case report and review of the literature. , 1993, The American journal of pediatric hematology/oncology.

[112]  J. Feingold,et al.  Epidemiological and clinical study of sickle cell disease in France, French Guiana and Algeria , 1993, European journal of haematology.

[113]  D. Fairclough,et al.  A randomized study of outpatient treatment with ceftriaxone for selected febrile children with sickle cell disease. , 1993, The New England journal of medicine.

[114]  M. Anderson,et al.  Human parvovirus infection in homozygous sickle cell disease , 1993, The Lancet.

[115]  M. Nwankwo,et al.  Malarial parasitaemia in febrile children with sickle cell anaemia. , 1992, Journal of tropical pediatrics.

[116]  E Vichinsky,et al.  Pain in sickle cell disease. Rates and risk factors. , 1991, The New England journal of medicine.

[117]  M. Durosinmi,et al.  Chronic leg ulcers in sickle cell disease: experience in Ibadan, Nigeria. , 1991, African journal of medicine and medical sciences.

[118]  M. Anderson,et al.  Human parvovirus infection in children and severe anaemia seen in an area endemic for malaria. , 1990, The Journal of tropical medicine and hygiene.

[119]  S. Leikin,et al.  Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease. , 1989, Pediatrics.

[120]  P. Levy,et al.  Leg ulcers in patients with sickle cell disease. , 1989, Blood.

[121]  A. F. Flening.  The presentation, management and prevention of crisis in sickle cell disease in Africa , 1989 .

[122]  M. El-Hazmi,et al.  The haematological, biochemical and clinical presentation of haemoglobin S in Saudi Arabia-(ii) biochemical expression , 1989 .

[123]  E. Vichinsky,et al.  Newborn screening for sickle cell disease: effect on mortality. , 1988, Pediatrics.

[124]  M. El-Hazmi,et al.  The haematological, biochemical and clinical--presentation of haemoglobin S in Saudi Arabia (i). Haematological & clinical expression. , 1987, Tropical and geographical medicine.

[125]  W. W. Ebong,et al.  Acute osteomyelitis in Nigerians with sickle cell disease. , 1986, Annals of the rheumatic diseases.

[126]  A. Kulozik,et al.  Geographical survey of beta S-globin gene haplotypes: evidence for an independent Asian origin of the sickle-cell mutation. , 1986, American journal of human genetics.

[127]  E Vichinsky,et al.  Prophylaxis with oral penicillin in children with sickle cell anemia. A randomized trial. , 1986, The New England journal of medicine.

[128]  D. Tosteson,et al.  Regulation of erythrocyte cation and water content in sickle cell anemia. , 1986, Science.

[129]  P. Beighton,et al.  Inherited disorders in the black population of southern Africa. Part I. Historical and demographic background; genetic haematological conditions. , 1986, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde.

[130]  D. Higgs,et al.  Acute splenic sequestration in homozygous sickle cell disease: natural history and management. , 1985, The Journal of pediatrics.

[131]  D. Labie,et al.  Common haplotype dependency of high G gamma-globin gene expression and high Hb F levels in beta-thalassemia and sickle cell anemia patients. , 1985, Proceedings of the National Academy of Sciences of the United States of America.

[132]  R. J. Hayes,et al.  The haematology of steady state homozygous sickle cell disease: frequency distributions, variation with age and sex, longitudinal observations , 1985, British journal of haematology.

[133]  M. Brozovīc,et al.  Sickle cell disease in Britain. , 1984, Journal of clinical pathology.

[134]  A. Look,et al.  Bone-marrow transplantation in a patient with sickle-cell anemia. , 1984, The New England journal of medicine.

[135]  S. Antonarakis,et al.  Origin of the beta S-globin gene in blacks: the contribution of recurrent mutation or gene conversion or both. , 1984, Proceedings of the National Academy of Sciences of the United States of America.

[136]  S. Edelstein,et al.  SICKLE CELL ANAEMIA AND REINCARNATION BELIEFS IN NIGERIA , 1983, The Lancet.

[137]  A. Schechter,et al.  Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity. , 1983, The Journal of clinical investigation.

[138]  G. Serjeant,et al.  Causes of death in sickle-cell disease in Jamaica. , 1982, British medical journal.

[139]  M. Stevens,et al.  Acute splenic sequestration and hypersplenism in the first five years in homozygous sickle cell disease. , 1981, Archives of disease in childhood.

[140]  G. Serjeant,et al.  Haematological factors associated with proliferative retinopathy in sickle cell-haemoglobin C disease. , 1981, The British journal of ophthalmology.

[141]  S. Jones,et al.  OUTBREAK OF APLASTIC CRISES IN SICKLE CELL ANAEMIA ASSOCIATED WITH PARVOVIRUS-LIKE AGENT , 1981, The Lancet.

[142]  S. Jones,et al.  PARVOVIRUS INFECTIONS AND HYPOPLASTIC CRISIS IN SICKLE-CELL ANAEMIA , 1981, The Lancet.

[143]  J. Eaton,et al.  Erythrocyte adherence to endothelium in sickle-cell anemia. A possible determinant of disease severity. , 1980, The New England journal of medicine.

[144]  A. Fleming,et al.  Abnormal haemoglobins in the Sudan savanna of Nigeria. III. Malaria, immunoglobulins and antimalarial antibodies in sickle cell disease. , 1979, Annals of tropical medicine and parasitology.

[145]  A. Fleming,et al.  Abnormal haemoglobins in the Sudan savanna of Nigeria. I. Prevalence of haemoglobins and relationships between sickle cell trait, malaria and survival. , 1979, Annals of tropical medicine and parasitology.

[146]  D. Labie,et al.  Structural bases of the inhibitory effects of hemoglobin F and hemoglobin A2 on the polymerization of hemoglobin S. , 1979, Proceedings of the National Academy of Sciences of the United States of America.

[147]  G. Overturf,et al.  Bacterial meningitis and septicemia in sickle cell disease. , 1977, American journal of diseases of children.

[148]  Ebong Ww.  Avascular necrosis of the femoral head associated with haemoglobinopathy. , 1977 .

[149]  Bender Ae.  Letter: Protein requirement. , 1974 .

[150]  R. Johnston,et al.  An abnormality of the alternate pathway of complement activation in sickle-cell disease. , 1973, The New England journal of medicine.

[151]  S. Ruddy,et al.  C3b inactivator of man. 3. Further purification and production of antibody to C3b INA. , 1972, Journal of immunology.

[152]  S. Ruddy,et al.  C3b inactivator of man. II. Fragments produced by C3b inactivator cleavage of cell-bound or fluid phase C3b. , 1971, Journal of immunology.

[153]  E. Barrett-Connor.  BACTERIAL INFECTION AND SICKLE CELL ANEMIA: AN ANALYSIS OF 250 INFECTIONS IN 166 PATIENTS AND A REVIEW OF THE LITERATURE , 1971, Medicine.

[154]  R. P. Spencer,et al.  Functional asplenia in sickle-cell anemia. , 1969, The New England journal of medicine.

[155]  J. Griffiths.  Avascular necrosis of femoral head in Kenyan africans. , 1968, East African medical journal.

[156]  J. Winkelstein,et al.  Deficiency of pneumococcal serum opsonizing activity in sickle-cell disease. , 1968, The New England journal of medicine.

[157]  G. Cooper,et al.  Salmonella osteomyelitis in patients with sickle-cell anemia. , 1957, The New England journal of medicine.

[158]  R. Ware,et al.  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Hydroxyurea as an Alternative to Blood Transfusions for the Prevention of Recurrent Stroke in Children With Sickle Cell Disease , 2017 .

[159]  G. Tešović,et al.  Invasive pneumococcal disease in children , 2011 .

[160]  H. Pearson,et al.  Newborn screening coupled with comprehensive follow-up reduced early mortality of sickle cell disease in Connecticut. , 2007, Connecticut medicine.

[161]  A. Gbadoé,et al.  [Stuttering priapism in children with sickle cell anemia in Togo]. , 2007, Archives de pediatrie : organe officiel de la Societe francaise de pediatrie.

[162]  D. Adekanle,et al.  Knowledge and attitude of female health workers towards prenatal diagnosis of sickle cell disease. , 2007, Nigerian journal of medicine : journal of the National Association of Resident Doctors of Nigeria.

[163]  P. Musgrove,et al.  Inherited Disorders of Hemoglobin , 2006 .

[164]  C. Gawad,et al.  Towards Molecular Medicine , 2005 .

[165]  J. Wautier,et al.  Erythrocytes and platelet adhesion to endothelium are mediated by specialized molecules. , 2004, Clinical hemorheology and microcirculation.

[166]  E. Alikor,et al.  Malaria parasite density and splenic status by ultrasonography in stable sickle-cell anaemia (HbSS) children. , 2004, Nigerian journal of medicine : journal of the National Association of Resident Doctors of Nigeria.

[167]  Bernadette Modell,et al.  Recommendations for introducing genetics services in developing countries , 2003, Nature Reviews Genetics.

[168]  M. Gladwin,et al.  Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease , 2002, Nature Medicine.

[169]  J. Knight-Madden,et al.  Invasive pneumococcal disease in homozygous sickle cell disease: Jamaican experience 1973-1997. , 2001, The Journal of pediatrics.

[170]  D J Weatherall,et al.  Inherited haemoglobin disorders: an increasing global health problem. , 2001, Bulletin of the World Health Organization.

[171]  K. Ohene‐Frempong.  Indications for red cell transfusion in sickle cell disease. , 2001, Seminars in hematology.

[172]  I. Diagne,et al.  [Infections in Senegalese children and adolescents with sickle cell anemia: epidemiological aspects]. , 2000, Dakar medical.

[173]  Jenifer L. Breshears.  Silent Cerebral Infarcts in Sickle Cell Anemia: A Risk Factor Analysis. , 2000 .

[174]  Scott T. Miller,et al.  Cerebrovascular accidents in sickle cell disease: rates and risk factors. , 1998, Blood.

[175]  J. Freedman,et al.  A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease , 1996 .

[176]  Rm,et al.  Sickle cell disease , 1996 .

[177]  M. Nwankwo,et al.  Pattern of bacteraemia in febrile children with sickle cell anaemia. , 1993, Annals of tropical paediatrics.

[178]  T. Teuscher,et al.  Prevalence of human parvovirus B19 in sickle cell disease and healthy controls. , 1991, Tropical and geographical medicine.

[179]  A. Fleming.  The presentation, management and prevention of crisis in sickle cell disease in Africa. , 1989, Blood reviews.

[180]  G. Noel,et al.  Complement-mediated early clearance of Haemophilus influenzae type b from blood is independent of serum lytic activity. , 1988, The Journal of infectious diseases.

[181]  R. E. Lee,et al.  The radiological features of avascular necrosis of the femoral head in homozygous sickle cell disease. , 1981, Clinical radiology.

[182]  W. W. Ebong.  Avascular necrosis of the femoral head associated with haemoglobinopathy. , 1977, Tropical and geographical medicine.