Impact of G6pd Deficiency on Sickle Cell Disease in Children in Kinshasa Hospitals: A Case-Control Study

Abstract

Background and aim: Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency and sickle cell disease are two genetic diseases of the red blood cell that both cause hemolytic anemia. The objective of this study is to determine the clinico- biological impact of G6PD deficiency on sickle cell disease in childrenin Kinshasa. Materials and method: This is a case-control analytical study of 103 G6PD-deficient sickle cell patientsand 309 non-G6PD- deficientsickle cell patients. Analysis of G6PD activitywas performed by ELISA, and hemoglobin electrophoresis by capillaris to confirm sickle cell disease.For all children, sociodemographic, clinical and biological variables were analyzed. Results: The mean age of sickle cell deficient and non-deficient patients was 9.82±4.5 years and 9.48±3.8 years respectively. There were slightly more female sickle cell deficient patients (55.2%) while in the non- deficient group, there was a male predominance (51.8%) but no statistically significant difference (p>0.05). All morbid events occurring in sickle cell patients were greater in the G6PD deficiency group (p˂0.05). Evaluated antecedent complications occurringin sickle cell patients wereassociated with G6PD deficiency (p˂0.05),except for stroke.No statistically significant difference was noted in physicalsigns between sicklecell deficient and non-deficient groups(p>0.05). The hemogramshowed no difference between the two groups in steady state and the hemolysis markers evaluated also showed no significant differences in steady state (p>0.05) between the two groups. Conclusion: G6PD deficiency aggravates the acute clinical manifestations and complications of sickle cell disease withoutaffecting the occurrence of stroke. And has no impact on the hematological parameters of sickle cell disease children in stationary phase.