Exploring the Coexistence of Glucose-6-Phosphate Dehydrogenase Deficiency in Sickle Cell Anaemia Patients: Insights from Maiduguri, Nigeria's North-East Region

Abstract

Background: Sickle cell anaemia (SCA) and Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency are inherited red cell disorders associated with chronic haemolysis that have a similar pattern of occurrence in malaria-endemic areas, including Nigeria.  Objectives: This study aims to determine the effect of co-inheritance of red blood cell G-6-PD deficiency and SCA regarding some clinical parameters. Methods: This cross-sectional study was conducted over 13 months involving 235 SCA  patients and 235 voluntary HbAA controls. Patients were HbSS as confirmed by Hb electrophoresis in an alkaline medium (pH 8.4-8.6) in a steady state. Quantitative G-6-PD enzyme activity among the study population was assayed using the spectrophotometric method. Results: The prevalence of G-6-PD deficiency was similar in patients (29.3%) and controls (25.5%). Only 3.8% of patients had total G-6-PD deficiency, and 25.5% had partial deficiency. The mean (SD) G-6-PD activity of patients was totally deficient; 1.49(0.43), partially deficient; 4.95(1.45), and normal; 10.39(2.66). Similarly, G-6-PD activity in controls was totally deficient; 1.62(0.36), partially deficient; 4.93(1.54) and normal 9.00(1.89). The mean age at first transfusion (±SD) was lower in patients with total G-6-PD deficiency (4.89 years ± 3.96) when compared with patients with normal G-6-PD activity (10.73 years ± 2.27). Conclusion: The prevalence of G-6-PD deficiency is high in both SCA patients and normal controls. Sickle cell anaemia patients with co-existing G-6-PD deficiency commence transfusion at a younger age than those without G-6-PD deficiency.