Abstract
High incidence of G6PD deficiency has been reported in areas of the eastern province of Saudi Arabia where sickle cell gene is also prevalent. This study was conducted to assess the co-incidence of this enzymopathy with Hb S and its influence upon the clinical and hematological expression of sickle cell disease. Eighty three children with SS disease, 145 patients with sickle cell trait and 100 random cord blood as samples with normal Hb AF, and an FS electrophoretic pattern respectively were examined. The frequency of interaction of G6PD deficiency with Hb S was found significantly increased but no effect of this enzyme defect was discerned on the clinical and hematological status of homozygous sickle cell disease.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Kirman HN, Hendrickson EM. Sex linked electrophoretic difference in glucose-6-phosphate dehydrogenase.Am J Hum Genet 1963; 15: 241–258.
Allison AC. Glucose-6-phosphate dehydrogenese deficiency in red blood cells in East Africans.Nature London 1960; 186; 531–532.
El-Hazmi MAF. Hemoglobin disorders: A pattern for thalassemia and hemoglobinopathies in Arabia.Acta Haematol 1982; 68: 43–51.
Al-Awamy BH, Al-Mouzan M, Altorkl MT, Serjeant GR. Neonatal screening for sickle cell disease in the Eastern province of Saudi Arabia.Trans Royal Soc Trop Med Hyg 1984; 78: 792–794.
Dow AP, Pattaway MM, Alperin JB. Simplified method for G6PD screening using blood collected on filter paper.Arch Clin Pathol 1974; 61: 333–336.
Pembrey MF, McWade P, Weatherall DJ. Reliable routine estimation of small amounts of et al hemoglobin by alkali denaturation.J Clin Pathol 1972; 25: 738–740.
Lewis RA, Hathron M. Glucose-6-phosphate dehydrogenase deficiency correlated with S hemoglobin.Ghana Med J 1963; 2 131.
Lewis RA, Hathorn M. Correlation of S hemoglobin with glucose-6-phosphate dehydrogenase and its significance.Blood 1965; 26: 176–180.
Naylor J, Rosenthal I, Grossman A et al. Activity of glucose-6-phosphate dehydrogenase in erythrocytes of patients with various abnormal hemoglobins.Pediatrics 1960; 26: 285–292.
Milner PF, Serjeant GR. Laboratory studies on sickle cell anemia.Blood 1969; 34: 729–738.
Piomelli S, Reindorf CA, Arzanian MT, Corash LM. Clinical and biochemical interactions of glucose-6-phosphate dehydrogenase deficiency and sickle cell anemia.New Engl J Med 1972; 287: 213–217.
Smits HF, Oski FA, Brody JI. The hemolytic crisis of sickle cell disease. The role of glucose-6-phosphate dehydrogenase deficiency.J. Pediatr 1969; 74: 544–551.
Gibbs WN, Wardle J, Serjeant GR. Glucose-6-phosphate dehydrogenase deficiency and homozygous sickle cell disease in Jamaica.Br J Haematol 1980; 45: 73–80.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Awamy, B.H. Effect of G-6 PD deficiency on sickle cell disease in Saudi Arabia. Indian J Pediatr 59, 331–334 (1992). https://doi.org/10.1007/BF02821799
Issue Date:
DOI: https://doi.org/10.1007/BF02821799