Springer Nature is making SARS-CoV-2 and COVID-19 research free. View research | View latest news | Sign up for updates

Aspects of sickle cell gene in Saudi Arabia—interaction with glucose-6-phosphate dehydrogenase deficiency

  • 53 Accesses

  • 31 Citations


Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency and sickle cell haemoglobin (Hb S) are red cell genetic abnormalities that occur at a high frequency in several areas of the world including several areas of Saudi Arabia. Genetic and clinical interactions between these two disorders are reported to occur in some populations.

In the present investigations, samples from affected individuals were studied for the prevalence of G-6-PD deficiency and Hb S genes. The results of haematological parameters and common clinical findings in the Hb S homozygotes with and without G-6-PD deficiency are presented and the possibility that the two conditions interact beneficially is discussed.

This is a preview of subscription content, log in to check access.


  1. Bernstein SC, Bowman JE, Noche LK (1980) Interactions of sickle cell trait and glucose-6-phosphate dehydrogenase deficiency in Cameroon. Hum Hered 30:7–11

  2. Betke K, Marti HR, Schlicht L (1959) Estimation of small percentage of foetal haemoglobin. Nature 184:1877–1878

  3. Beutler E (1971) Red cell metabolism: a manual of biochemical methods. Grune and Stratton, New York

  4. Beutler E (1978) Hemolytic anaemia in disorders of red cell metabolism. Plenum Medical Book Company, New York, pp 23–168

  5. Beutler E (1982) G-6-PD: historical perspective and current status. In: Weatherall DJ, Fiorelli G, Gorin S (eds) Advances of red blood cell biology. Raven Press, New York, pp 297–308

  6. Beutler E, Johnson C, Powars D, West C (1974) Prevalence of glucose-6-phosphate dehydrogenase deficiency in sickle-cell disease. N Engl J Med 290:826–828

  7. Bienzle U, Sodeinde O, Effiong CE, Luzzatto L (1975) Glucose-6-phosphate dehydrogenase deficiency and sickle cell anaemia: frequency and features of the association in an African community. Blood 46:591–597

  8. Burka ER, Weaver Z, Marks PA (1966) Clinical spectrum of hemolytic anemia associated with glucose-6-phosphate dehydrogenase deficiency. Ann Intern Med 64:817–825

  9. Dacie JV, Lewis SM (1975) Practical haematology, 5th edn. Churchill Livingstone, London

  10. El-Hazmi MAF (1982) Haemoglobin disorders: a pattern for thalassaemia and haemoglobinopathies in Arabia. Acta Haematol (Basel) 68:43–51

  11. El-Hazmi MAF, Jabbar FA, Al-Faleh FZ, Swailem AR (1983) The frequency of red cell glucose-6-phosphate dehydrogenase, pyruvate kinase and hexokinase deficiency in Riyadh, Al-Hafouf and Khaiber—comparative studies. 8th Saudi Medical Conference, November 1984, Riyadh (Abstract). National Guard Printing Press, Riyadh, Saudi Arabia

  12. Erfandson ME, Schulman I, Smith CH (1960) Studies on congenital hemolytic syndromes. III. Rates of destruction and production of erythrocytes in sickle cell anemia. Pediatrics 25:629–644

  13. Gelpi AP (1965) Glucose-6-phosphate dehydrogenase deficiency in Saudi Arabia: a survey. Blood 25:486–493

  14. Gelpi AP (1967) Glucose-6-phosphate dehydrogenase deficiency, the sickling trait and malaria in Saudi Arab children. J Pediatr 71:138–146

  15. Gelpi AP (1982) Sickle cell disease in the Middle East. In: Fried W (ed) Comparative clinical aspects of sickle cell disease. Elsevier/North Holland, New York, pp 35–50

  16. Gelpi AP, King MC (1977) New data on glucose-6-phosphate dehydrogenase deficiency in Saudi Arabia. G-6-PD variant and the association between enzyme deficiency and hemoglobin S. Hum Hered 27:285–291

  17. Gibbs WN, Wardle J, Serjeant GR (1980) Glucose-6-phosphate dehydrogenase deficiency and homozygous sickle cell disease in Jamaica. Br J Haematol 45:73–80

  18. Kattamis CA, Tjortjatou F (1970) The hemolytic process of viral hepatitis in children with normal and deficient glucose-6-phosphate dehydrogenase activity. J Pediatr 77:422–430

  19. Konotey-Ahulu FID (1972) Glucose-6-phosphate dehydrogenase deficiency and sickle cell anemia. N Engl J Med 287:887–888

  20. Lewis RA (1967) Sickle cell anaemia in G-6-PD deficiency. Lancet I:852–853

  21. Lewis RA, Hathorn M (1963) Glucose-6-phosphate dehydrogenase deficiency correlated with S hemoglobin. Ghana Med J 2:131

  22. Luzzatto L, Allan NC (1968) Relationship between the genes for glucose-6-phosphate dehydrogenase and for haemoglobin S in a Nigerian population. Nature 219:1041–1042

  23. Marengo-Rowe AJ (1965) Rapid electrophoresis and quantitation of haemoglobins on cellulose acetate. J Clin Pathol 18:790–792

  24. Marti HR, Schoepf K, Gsell OR (1965) Frequency of haemoglobin S and glucose-6-phosphate dehydrogenase deficiency in Southern Tanzania. Br Med J I:1476–1477

  25. Morrow RH, Sai FT, Edgcomb JH, Smetana HF (1969) Epidemiology of viral hepatitis in Accra, Ghana. Trans R Soc Trop Med Hyg 63:755–767

  26. Nance WE (1977) Quantitative studies of glucose-6-phosphate dehydrogenase. Am J Hum Genet 29:537–543

  27. Naylor J, Rosenthal I, Grossman A, Schulman I, Hsia DY-Y (1960) Activity of glucose-6-phosphate dehydrogenase in erythrocytes of patients with various abnormal haemoglobins. Pediatrics 26:285–292

  28. Necheles TR, Gorshein D (1968) Virus-induced hemolysis in erythrocytes deficient in glucose-6-phosphate dehydrogenase. Science 160:535–536

  29. Phillips SM, Silver NP (1969) Glucose-6-phosphate dehydrogenase deficiency, infectious hepatitis, acute hemolysis and renal failure. Ann Intern Med 70:99–104

  30. Piomelli S, Reindorf CA, Arzanian MT, Corash LM (1972) Clinical and biochemical interactions of glucose-6-phosphate dehydrogenase deficiency and sickle-cell anemia. N Engl J Med 287:213–217

  31. Robinson AR, Robson M, Harrison AP, Zuelzer WW (1957) A new technique for differentiation of hemoglobin. J Lab Clin Med 50:745–752

Download references

Author information

Correspondence to M. A. F. El-Hazmi.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

El-Hazmi, M.A.F., Warsy, A.S. Aspects of sickle cell gene in Saudi Arabia—interaction with glucose-6-phosphate dehydrogenase deficiency. Hum Genet 68, 320–323 (1984). https://doi.org/10.1007/BF00292593

Download citation


  • Internal Medicine
  • Metabolic Disease
  • Genetic Abnormality
  • Clinical Finding
  • Sickle Cell