Keto MA 1*, Tshilolo L 2, Budiongo NA1, Mfulani G 3, Mafuta E 4, Mukaba Y 5, Phoba B 5, Aketi PL 1, Bodi MJ 1, Mabela MA 1, Monkoti M 1, and Ngiyulu MR 1
Received Date: November 09, 2022; Accepted Date: December 16, 2022; Published Date: December 23, 2022
Citation: Keto MA, Tshilolo L, Budiongo NA, Mfulani G, Mafuta E. et all (2022) Impact of G6pd Deficiency on Sickle Cell Disease in Children in Kinshasa Hospitals: a Case-Control Study. J. Biomedical Research and Clinical Reviews. 7(3); DOI:10.31579/2690-4861/135
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 children in Kinshasa.
Materials and method: This is a case-control analytical study of 103 G6PD-deficient sickle cell patients and 309 non-G6PD-deficient sickle cell patients. Analysis of G6PD activity was 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 occurring in sickle cell patients were associated with G6PD deficiency (p˂0.05), except for stroke. No statistically significant difference was noted in physical signs between sickle cell deficient and non-deficient groups (p>0.05). The hemogram showed 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 without affecting the occurrence of stroke. And has no impact on the hematological parameters of sickle cell disease children in stationary phase. Key words: G6PD deficiency, sickle cell disease, child and Kinshasa.
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