Glucose-6-Phosphate Dehydrogenase Status of Sickle Cell Disease Patients in Ibadan, Oyo State, Nigeria
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2022-12
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Lead City University
Abstract
Glucose-6-phosphate dehydrogenase (G6PD) is an enzyme involved in the pentose monophosphate pathway. Deficiency of this enzyme leads to free radical-mediated oxidative damage to red blood cells, and in turn causes haemolysis. G6PD deficiency is the most common genetic enzymatic disorder of red blood cells, affecting 400 million people worldwide. Sickle cell disease (SCD) is a genetic disorder in which individual inherit two abnormal copies of β-globin subunits given rise to haemoglobin S. The HbS red cells fail to return to normal shape when normal oxygen tension is restored thereby leading to vessel occlusion, ischaemia, and destruction of the red blood cells (haemolysis). Sickle cell disease and glucose-6-phosphate dehydrogenase deficiency are inherited disorders associated with chronic haemolysis. Therefore, coinheritance of both disorders could worsen haemolysis in the former and compound a haemolytic crisis. This work aims at determining the glucose-6-phosphate dehydrogenase status of sickle cell disease patients in Ibadan. Blood samples were collected from 147 sickle cell anaemia patients recruited from The Haematology Day Care Unit UCH Ibadan and Sickle Cell Clinic Adeoyo Ibadan. Haemoglobin electrophoresis was carried out to determine the haemoglobin genotype. The haemoglobin variants obtained were 142 (96.6%) homozygous haemoglobin S (SS), 4 (2.7%) heterozygous haemoglobin SC and 1 (0.7%) homozygous haemoglobin CC with. Qualitative analysis of G6PD was carried out using fluorescent spot test resulting to 21 (14.3%) full deficient and 11 (7.5%) partial deficient. Quantitative analysis of G6PD was determined using G6PD RANDOX reagent and 21.8% were G6PD deficient. DNA extraction and amplification was carried out and the gel electrophoresis was used to determine the band size of 308bp of G6PD variants (rs1050829, 376 A→G, chrX:154535277; 156 asn → asp). All that were G6PD A variants were digested with FokI restriction enzyme to determine the G6PD A variant polymorphism in which none was observed. This study has shown that G6PD deficiency is highly prevalent among those with HbSS and that all SCD patients should be screened for G6PD deficiency to avoid the use of medications and agents that could aggravate haemolysis during the treatment and management. In view of the findings in this study, it is recommended that G6PD status of everyone should be determined in order to prevent haemolysis after exposure to oxidative agents.
Key words: Haemolysis, G6PD (glucose-6-phosphate dehydrogenase), FokI restriction enzymes, oxidative agent.
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Kate Turabian