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The Sickle Cell Diseases: Clinical Manifestations Including the Sickle Crisis

The Sickle Cell Diseases: Clinical Manifestations Including the Sickle Crisis Abstract Working definitions of "sickle cell disease," "sickle-cell trait," and "the sickle crisis" are given. The pathogenesis of the crisis is explained and the complications of the disease are presented in a quantitative manner. The need to look at the patient as a whole and to assess the natural history of the disease against the background of an often prevalent social and environmental pathologic condition is stressed. A call for effective public health measures, including raising of living standards and providing comprehensive health care facilities for sickle cell disease patients is made. References 1. Herrick JB: Peculiar elongated and sickle shaped red blood corpuscles in a case of severe anaemia. Arch Intern Med 6:517-521, 1910.Crossref 2. Konotey-Ahulu FID: Hereditary qualitative and quantitative erythrocyte defects in Ghana: An historical and geographical survey. Ghana Med J 7:118-119, 1968. 3. Konotey-Ahulu FID: Sickle-cell disease in successive Ghanaian generations for three centuries , in Sickle-cell Disease: Natural History From a Clinico-Epidemiological Study of 1,550 Consecutive Patients . Ghana, Ghana University Press, to be published. 4. Konotey-Ahulu FID: Computer assisted analysis of data on 1,697 patients attending the sickle cell/haemoglobinopathy clinic of Korle Bu Teaching Hospital, Accra, Ghana: Clinical features: I. Sex, genotype, age, rheumatism and dactylitis frequencies. Ghana Med J 10:241-260, 1971. 5. Lehmann H, Huntsman RC: Man's Haemoglobin . Amsterdam, North Holland Publishing Co, 1966. 6. Ringelhann B, Khorsandi M: Hemoglobin crystallization test to differentiate cells with Hb SC and CC genotype from SS cells without electrophoresis. Am J Clin Pathol 57:467-470, 1972. 7. WHO Scientific Group on the standardization of procedures for the study of glucose-6-phosphatedehydrogenase. WHO Tech Rep Ser 366:1-53, 1967. 8. Ringelhann B: A simple laboratory procedure for the recognition of A (African type) G 6PD deficiency in acute haemolytic crisis. Clin Chim Acta 36:272-274, 1972.Crossref 9. Woodruff AW, et al: Terminology of the hereditary haemoglobinopathies with haemoglobin variants. Br Med J 1:1235, 1957.Crossref 10. WHO Scientific Group Report: Hemoglobinopathies and allied disorders. WHO Tech Rep Ser 338:1-40, 1966. 11. Konotey-Ahulu FID: Definition of sickle trait and sickle cell disease. Ghana Med J 11:417-420,1972. 12. Konotey-Ahulu FID: Sickle-cell trait and altitude. Br Med J 1:177-178, 1972.Crossref 13. Konotey-Ahulu FID: Sickle cell and altitude. Br Med J 2:231, 1972.Crossref 14. Addae RO: Sickle cell trait and altitude. Br Med J 1:53, 1972.Crossref 15. Konotey-Ahulu FID: Balanced polymorphism and hereditary qualitative and quantitative erythrocyte defects. Ghana Med J 11:274-285, 1972. 16. Konotey-Ahulu FID: Malaria and sickle cell disease. Br Med J 2:710-711, 1971.Crossref 17. Konotey-Ahulu FID: Treatment and prevention of sickle-cell crisis. Lancet 2:1255, 1971.Crossref 18. Konotey-Ahulu FID: Sicklaemic human hygrometers. Lancet 1:1003-1004, 1965.Crossref 19. Konotey-Ahulu FID: Management of patients with sickle-cell disease. Lancet 2:772, 1972.Crossref 20. Diggs LW: Sickle-cell crises. Am J Clin Pathol 44:1-19, 1965. 21. Fullerton WT, Watson-Williams EJ: Haemoglobin SC disease and megaloblastic anaemia of pregnancy. J Obstet Gynaecol Br Commonw 69:729-735, 1962.Crossref 22. Fullerton WT, Hendrickse JP de V, Watson-Williams EJ: Haemoglobin SC disease in pregnancy: A review of 190 cases , in Jonxis JHP (ed): Abnormal Haemoglobins in Africa . Oxford, England, Blackwell Scientific Publications, 1965, pp 411-433. 23. Watson-Williams EJ: The management of patients with abnormal haemoglobin disorders in pregnancy , in Maegraith BG, Gilles HM (eds): Management and Treatment of Tropical Diseases . Oxford, England, Blackwell Scientific Publications, 1971, pp 56-58. 24. Konotey-Ahulu FID: Sickle cell Disease: The Case for Family Planning . Accra, Ghana, Astab Books Ltd, 1973, p 11. 25. Konotey-Ahulu FID: The pathogenesis of sickle-cell crisis in Ghana. Ghana Med J 11:304-306, 1972. 26. Addae S: Mechanism for the high incidence of sickle-cell crisis in the tropical cool season. Lancet 2:1256, 1971.Crossref 27. Addae SK: Aspects of renal function in sicklecell crisis. Ghana Med J 11:242-247, 1972. 28. Addae SK: The Effect of Different Ambient Temperatures on Some Renal Functions in Sickle Cell Haemoglobinopathy, thesis. London, 1972. 29. Lowe RC, Adams CC: Studies on the pathophysiology of sickle-cell disease. Ann Intern Med 22:192-200, 1945.Crossref 30. Grover V: Clinical manifestations of sickle-cell anaemia. Ann Intern Med J 26:843-851, 1947.Crossref 31. Konotey-Ahulu FID: Epistaxis in sickle-cell anaemia. Br Med J 1:234, 1966.Crossref 32. Konotey-Ahulu FID: Patterns of clinical haemoglobinopathy. East Afr Med J 46:149-156, 1949. 33. Konotey-Ahulu FID: Mental-nerve neuropathy: A complication of sickle-cell crisis. Lancet 2:388, 1972.Crossref 34. Konotey-Ahulu FID: Effect of environment on sickle cell disease in West Africa: Epidemiologic and clinical considerations , in Abramson H, Bertles JF, Wethers D (eds): Symposium on Sickle-Cell Disease: Diagnosis, Management, Education and Research . St Louis, CV Mosby Co, 1973, pp 20-38. 35. Konotey-Ahulu FID: The liver in sickle-cell disease: Clinical aspects. Ghana Med J 8:104-118, 1969. 36. Serjeant GR: The clinical features in adults with sickle-cell anaemia in Jamaica. West Ind Med J 19:1-8, 1970. 37. Konotey-Ahulu FID, Kuma E: Skeletal crumbling in sickle cell anaemia complicated by Salmonella typhi infection. Br J Clin Pract 19:575-578, 1965. 38. Konotey-Ahulu FID: Hip disease in Africans. Lancet 1:999, 1970.Crossref 39. Konotey-Ahulu FID, Ringelhann B: Sickle-cell anaemia, sickle-cell thalassaemia, sickle-cell haemoglobin C disease and asymptomatic haemoglobin C thalassaemia in one Ghanaian family. Br Med J 1:607-612, 1969.Crossref 40. Ringelhann B, Konotey-Ahulu FID: Immunological studies in sickle cell crisis in Ghana. Afr J Med Sci 4:17-22, 1973. 41. WHO Scientific Group Report: Treatment of haemoglobinopathies and allied disorders. WHO Tech Rep Ser 509:1-83, 1972. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Internal Medicine American Medical Association

The Sickle Cell Diseases: Clinical Manifestations Including the Sickle Crisis

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Publisher
American Medical Association
Copyright
Copyright © 1974 American Medical Association. All Rights Reserved.
ISSN
0003-9926
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1538-3679
DOI
10.1001/archinte.1974.00320160105009
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Abstract

Abstract Working definitions of "sickle cell disease," "sickle-cell trait," and "the sickle crisis" are given. The pathogenesis of the crisis is explained and the complications of the disease are presented in a quantitative manner. The need to look at the patient as a whole and to assess the natural history of the disease against the background of an often prevalent social and environmental pathologic condition is stressed. A call for effective public health measures, including raising of living standards and providing comprehensive health care facilities for sickle cell disease patients is made. References 1. Herrick JB: Peculiar elongated and sickle shaped red blood corpuscles in a case of severe anaemia. Arch Intern Med 6:517-521, 1910.Crossref 2. Konotey-Ahulu FID: Hereditary qualitative and quantitative erythrocyte defects in Ghana: An historical and geographical survey. Ghana Med J 7:118-119, 1968. 3. Konotey-Ahulu FID: Sickle-cell disease in successive Ghanaian generations for three centuries , in Sickle-cell Disease: Natural History From a Clinico-Epidemiological Study of 1,550 Consecutive Patients . Ghana, Ghana University Press, to be published. 4. Konotey-Ahulu FID: Computer assisted analysis of data on 1,697 patients attending the sickle cell/haemoglobinopathy clinic of Korle Bu Teaching Hospital, Accra, Ghana: Clinical features: I. Sex, genotype, age, rheumatism and dactylitis frequencies. Ghana Med J 10:241-260, 1971. 5. Lehmann H, Huntsman RC: Man's Haemoglobin . Amsterdam, North Holland Publishing Co, 1966. 6. Ringelhann B, Khorsandi M: Hemoglobin crystallization test to differentiate cells with Hb SC and CC genotype from SS cells without electrophoresis. Am J Clin Pathol 57:467-470, 1972. 7. WHO Scientific Group on the standardization of procedures for the study of glucose-6-phosphatedehydrogenase. WHO Tech Rep Ser 366:1-53, 1967. 8. Ringelhann B: A simple laboratory procedure for the recognition of A (African type) G 6PD deficiency in acute haemolytic crisis. Clin Chim Acta 36:272-274, 1972.Crossref 9. Woodruff AW, et al: Terminology of the hereditary haemoglobinopathies with haemoglobin variants. Br Med J 1:1235, 1957.Crossref 10. WHO Scientific Group Report: Hemoglobinopathies and allied disorders. WHO Tech Rep Ser 338:1-40, 1966. 11. Konotey-Ahulu FID: Definition of sickle trait and sickle cell disease. Ghana Med J 11:417-420,1972. 12. Konotey-Ahulu FID: Sickle-cell trait and altitude. Br Med J 1:177-178, 1972.Crossref 13. Konotey-Ahulu FID: Sickle cell and altitude. Br Med J 2:231, 1972.Crossref 14. Addae RO: Sickle cell trait and altitude. Br Med J 1:53, 1972.Crossref 15. Konotey-Ahulu FID: Balanced polymorphism and hereditary qualitative and quantitative erythrocyte defects. Ghana Med J 11:274-285, 1972. 16. Konotey-Ahulu FID: Malaria and sickle cell disease. Br Med J 2:710-711, 1971.Crossref 17. Konotey-Ahulu FID: Treatment and prevention of sickle-cell crisis. Lancet 2:1255, 1971.Crossref 18. Konotey-Ahulu FID: Sicklaemic human hygrometers. Lancet 1:1003-1004, 1965.Crossref 19. Konotey-Ahulu FID: Management of patients with sickle-cell disease. Lancet 2:772, 1972.Crossref 20. Diggs LW: Sickle-cell crises. Am J Clin Pathol 44:1-19, 1965. 21. Fullerton WT, Watson-Williams EJ: Haemoglobin SC disease and megaloblastic anaemia of pregnancy. J Obstet Gynaecol Br Commonw 69:729-735, 1962.Crossref 22. Fullerton WT, Hendrickse JP de V, Watson-Williams EJ: Haemoglobin SC disease in pregnancy: A review of 190 cases , in Jonxis JHP (ed): Abnormal Haemoglobins in Africa . Oxford, England, Blackwell Scientific Publications, 1965, pp 411-433. 23. Watson-Williams EJ: The management of patients with abnormal haemoglobin disorders in pregnancy , in Maegraith BG, Gilles HM (eds): Management and Treatment of Tropical Diseases . Oxford, England, Blackwell Scientific Publications, 1971, pp 56-58. 24. Konotey-Ahulu FID: Sickle cell Disease: The Case for Family Planning . Accra, Ghana, Astab Books Ltd, 1973, p 11. 25. Konotey-Ahulu FID: The pathogenesis of sickle-cell crisis in Ghana. Ghana Med J 11:304-306, 1972. 26. Addae S: Mechanism for the high incidence of sickle-cell crisis in the tropical cool season. Lancet 2:1256, 1971.Crossref 27. Addae SK: Aspects of renal function in sicklecell crisis. Ghana Med J 11:242-247, 1972. 28. Addae SK: The Effect of Different Ambient Temperatures on Some Renal Functions in Sickle Cell Haemoglobinopathy, thesis. London, 1972. 29. Lowe RC, Adams CC: Studies on the pathophysiology of sickle-cell disease. Ann Intern Med 22:192-200, 1945.Crossref 30. Grover V: Clinical manifestations of sickle-cell anaemia. Ann Intern Med J 26:843-851, 1947.Crossref 31. Konotey-Ahulu FID: Epistaxis in sickle-cell anaemia. Br Med J 1:234, 1966.Crossref 32. Konotey-Ahulu FID: Patterns of clinical haemoglobinopathy. East Afr Med J 46:149-156, 1949. 33. Konotey-Ahulu FID: Mental-nerve neuropathy: A complication of sickle-cell crisis. Lancet 2:388, 1972.Crossref 34. Konotey-Ahulu FID: Effect of environment on sickle cell disease in West Africa: Epidemiologic and clinical considerations , in Abramson H, Bertles JF, Wethers D (eds): Symposium on Sickle-Cell Disease: Diagnosis, Management, Education and Research . St Louis, CV Mosby Co, 1973, pp 20-38. 35. Konotey-Ahulu FID: The liver in sickle-cell disease: Clinical aspects. Ghana Med J 8:104-118, 1969. 36. Serjeant GR: The clinical features in adults with sickle-cell anaemia in Jamaica. West Ind Med J 19:1-8, 1970. 37. Konotey-Ahulu FID, Kuma E: Skeletal crumbling in sickle cell anaemia complicated by Salmonella typhi infection. Br J Clin Pract 19:575-578, 1965. 38. Konotey-Ahulu FID: Hip disease in Africans. Lancet 1:999, 1970.Crossref 39. Konotey-Ahulu FID, Ringelhann B: Sickle-cell anaemia, sickle-cell thalassaemia, sickle-cell haemoglobin C disease and asymptomatic haemoglobin C thalassaemia in one Ghanaian family. Br Med J 1:607-612, 1969.Crossref 40. Ringelhann B, Konotey-Ahulu FID: Immunological studies in sickle cell crisis in Ghana. Afr J Med Sci 4:17-22, 1973. 41. WHO Scientific Group Report: Treatment of haemoglobinopathies and allied disorders. WHO Tech Rep Ser 509:1-83, 1972.

Journal

Archives of Internal Medicine – American Medical Association

Published: Apr 1, 1974

References

  • Peculiar elongated and sickle shaped red blood corpuscles in a case of severe anaemia.
    Herrick JB
  • Hereditary qualitative and quantitative erythrocyte defects in Ghana: An historical and geographical survey.
    Konotey-Ahulu FID
  • Sickle-cell Disease: Natural History From a Clinico-Epidemiological Study of 1,550 Consecutive Patients
    Konotey-Ahulu FID
  • Computer assisted analysis of data on 1,697 patients attending the sickle cell/haemoglobinopathy clinic of Korle Bu Teaching Hospital, Accra, Ghana: Clinical features: I. Sex, genotype, age, rheumatism and dactylitis frequencies.
    Konotey-Ahulu FID
  • Man's Haemoglobin
    Lehmann H, Huntsman RC
  • Hemoglobin crystallization test to differentiate cells with Hb SC and CC genotype from SS cells without electrophoresis.
    Ringelhann B, Khorsandi M
  • WHO Scientific Group on the standardization of procedures for the study of glucose-6-phosphatedehydrogenase.
  • A simple laboratory procedure for the recognition of A (African type) G 6PD deficiency in acute haemolytic crisis.
    Ringelhann B
  • Terminology of the hereditary haemoglobinopathies with haemoglobin variants.
    Woodruff AW, et al
  • Hemoglobinopathies and allied disorders.
    WHO Scientific Group Report
  • Definition of sickle trait and sickle cell disease.
    Konotey-Ahulu FID
  • Sickle-cell trait and altitude.
    Konotey-Ahulu FID
  • Sickle cell and altitude.
    Konotey-Ahulu FID
  • Sickle cell trait and altitude.
    Addae RO
  • Balanced polymorphism and hereditary qualitative and quantitative erythrocyte defects.
    Konotey-Ahulu FID
  • Malaria and sickle cell disease.
    Konotey-Ahulu FID
  • Treatment and prevention of sickle-cell crisis.
    Konotey-Ahulu FID
  • Sicklaemic human hygrometers.
    Konotey-Ahulu FID
  • Management of patients with sickle-cell disease.
    Konotey-Ahulu FID
  • Sickle-cell crises.
    Diggs LW
  • Haemoglobin SC disease and megaloblastic anaemia of pregnancy.
    Fullerton WT, Watson-Williams EJ
  • Abnormal Haemoglobins in Africa
    Fullerton WT, Hendrickse JP de V, Watson-Williams EJ
  • Management and Treatment of Tropical Diseases
    Watson-Williams EJ
  • Sickle cell Disease: The Case for Family Planning
    Konotey-Ahulu FID
  • The pathogenesis of sickle-cell crisis in Ghana.
    Konotey-Ahulu FID
  • Mechanism for the high incidence of sickle-cell crisis in the tropical cool season.
    Addae S
  • Aspects of renal function in sicklecell crisis.
    Addae SK
  • Studies on the pathophysiology of sickle-cell disease.
    Lowe RC, Adams CC
  • Clinical manifestations of sickle-cell anaemia.
    Grover V
  • Epistaxis in sickle-cell anaemia.
    Konotey-Ahulu FID
  • Patterns of clinical haemoglobinopathy.
    Konotey-Ahulu FID
  • Mental-nerve neuropathy: A complication of sickle-cell crisis.
    Konotey-Ahulu FID
  • Symposium on Sickle-Cell Disease: Diagnosis, Management, Education and Research
    Konotey-Ahulu FID
  • The liver in sickle-cell disease: Clinical aspects.
    Konotey-Ahulu FID
  • The clinical features in adults with sickle-cell anaemia in Jamaica.
    Serjeant GR
  • Skeletal crumbling in sickle cell anaemia complicated by Salmonella typhi infection.
    Konotey-Ahulu FID, Kuma E
  • Hip disease in Africans.
    Konotey-Ahulu FID
  • Sickle-cell anaemia, sickle-cell thalassaemia, sickle-cell haemoglobin C disease and asymptomatic haemoglobin C thalassaemia in one Ghanaian family.
    Konotey-Ahulu FID, Ringelhann B
  • Immunological studies in sickle cell crisis in Ghana.
    Ringelhann B, Konotey-Ahulu FID
  • Treatment of haemoglobinopathies and allied disorders.
    WHO Scientific Group Report