Abstract

THE PREVALENCE OF GLUCOSE-SIX-PHOSPHATE DEHYDROGENASE DEFICIENCY IN PATIENTS WITH SICKLE CELL ANEMIA IN SUDAN

Aadil Yousif Mohamed*, Elharam Abdallah, Mosab Jafer Hamad and Rofaida Abd Alaleem Gorashi

Abstract

Background: Sickle cell disease (SCD) and glucose-6-phosphate dehydrogenase (G6PD) deficiency are inherited disorders associated with chronic haemolysis. Therefore, coinheritance of both disorders could worsen haemolysis in the former and compound a haemolytic crisis. Aim: This study aimed to determine the co-incidence of glucose-6-phosphatedehydrogenase deficiency and sickle cell anaemia in Sudan. Method: A hundred samples of patients with SCD of both sexes with ages ranged between 6 months and 16 years and 50 samples from healthy individuals between the same ages as control group were collected from these patients and investigated for Hb concentration, RBCs count, and G6PD assay. Results: The study revealed that 45 patients were homozygous (SS), 55 patients were heterozygous (AS). And we found that out of the 100 SCD patients, 81 patients (81%) were within the normal range which lies between 245-299 mU/erythrocytes per ml blood and 19 patients (19%) were deficient less than 245 mU/erythrocytes per ml blood by G6PD assay method also there were 2 of the control group were deficient. The study showed that 77% of SCD patients had RBCs count less than 3.00×109 /L and the rest 23% had count between 3.00-4.00×109 /L, the Hemoglobin (Hb) concentration level between 3.6 - 10.0 g/dl with mean of 6.4 g/dl. Conclusion: The co-incidence of G6PD deficiency with SCD affect the red blood cells count and hemoglobin concentration. G6PD deficiency may have slightly effect on the severity of hemolysis and the degree of anemia in deficient SCD patients.

Keywords: Cell Disease, Glucose 6 phosphate Dehydrogenase, Coinheritance, Haemolysis.