Abstract
Background: Both glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease (SCD) are prevalent in malaria-endemic regions. Controversy however persists as to whether G6PD deficiency is commoner in SCD subjects compared with the general population. Co-existence of the enzyme-deficient state with a chronic haemolytic disorder, like SCD, could potentially predispose to fatal haemolytic episodes. There is however a dearth of paediatric studies on this subject. Aims and Objectives: To determine the prevalence and demographic determinants of G6PD deficiency in children with SCD. Methods: Red cell G6PD activity was determined in 115 steady-state sickle cell disease children aged 0.5 - 17 years, in steady state and equal number of age & gender-matched non-SCD controls using the quantitative method. A measured G6PD activity of <6.97 U/gHb defined G6PD deficiency while values ≥6.97 U/gHb were regarded as normal. Data were analysed using SPSS version 20. Statistical analyses done include chi-square, student t-test and ANOVA. For all statistical analyses, p values less than 0.05 were considered significant. Results: There were 64 (55.7%) males and 51 (44.3%) females in each of the arms of the study. The mean age of the study population was 8.4 ± 4.7 years. Seven of the subjects were G6PD-deficient giving a prevalence of 6.1% which was not significantly different from the 7.0% obtained in the controls (p = 0.789). G6PD deficiency was more frequently encountered in the older age groups although the difference was not statistically significant (p = 0.438). Similarly, mean G6PD activity was highest in the under-5 age-group compared to the older age-groups analysed (p = 0.573). The condition was also commoner in the males than females although the difference did not attain statistical significance (p = 0.897). Conclusions: The prevalence of G6PD deficiency among SCD children was 6.1%. The condition is marginally commoner in males and older children.
Highlights
Glucose-6-phosphate dehydrogenase (G6PD) deficiency, like sickle cell disease (SCD), is prevalent in malaria-endemic regions such as Africa, Asia and the Mediterranean regions [1]
Red cell G6PD activity was determined in 115 steady-state sickle cell disease children aged 0.5 - 17 years, in steady state and equal number of age & gender-matched non-SCD controls using the quantitative method
Seven of the subjects were G6PD-deficient giving a prevalence of 6.1% which was not significantly different from the 7.0% obtained in the controls (p = 0.789)
Summary
Glucose-6-phosphate dehydrogenase (G6PD) deficiency, like sickle cell disease (SCD), is prevalent in malaria-endemic regions such as Africa (especially sub-Saharan Africa), Asia and the Mediterranean regions [1]. This has been attributed to the partial protection this condition offers against severe malaria, conferring a survival advantage on sufferers residing in malaria-endemic regions [1] [2]. The commonest manifestation of the condition is recurrent, acute (and often life-threatening) haemolytic episodes, which commonly follows exposure to a wide range of oxidant medications or infectious agents [3] [7] Both glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell disease (SCD) are prevalent in malaria-endemic regions.
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