- : Ms Word, Ms Word Format
- : 80 Pages
- : ₦5000
- : 1-5 Chapters
GLUCOSE -6- PHOSPHATE DEHYDROGENASE ACTIVITY IN ADULT SICKLE CELL DISEASE PATIENTS IN IBADAN SOUTH WESTERN NIGERIA
Abstract
Background: Glucose-6-phosphate dehydrogenase is a metabolic enzyme involved in the pentose phosphate pathway, an important pathway in red blood cell metabolism. Glucose-6-phosphate dehydrogenase deficiency is an X-linked recessive disorder characterized by abnormally low levels of glucose-6-phosphate dehydrogenase. Sickle cell disease and glucose-6-phosphate dehydrogenase deficiency are both inherited chronic haemolytic disorders prevalent in Nigeria.
Objective: The main objective of this study was to determine and compare disease severity among sickle cell disease patients with or without glucose -6- phosphate dehydrogenase deficiency with haemoglobin A individuals, with or without the deficiency.
Materials and Methods: A total of 166 sickle cell disease patents in steady state and 166 haemoglobin A controls were recruited in a case-control study at the haematology clinic of the University College Hospital, Ibadan Nigeria. Haemoglobin electrophoresis with cellulose acetate membrane was carried out for both patients and controls to confirm their phenotype. The glucose-6-phosphate dehydrogenase activity was assessed by spectrophotometry method.
Results: The sickle cell disease patients consist of 75(45.2%) males and 91(54.8%) females. The controls included 87(52.4%) males and 79 (47.6%) females. The patients and controls were aged from 18-59 years with a mean of 27.3 ± 9.4 and 35.9 ± 9.7 respectively. The prevalence of G6PD deficiency among the 166 sickle cell disease patients confirmed that 28.9% were deficient but 22.3% in the controls. Lower haemoglobin, higher white cell and platelet counts, increased reticulocyte count and bilirubin levels were found in G6PD deficient patients when compared with non-G6PD deficient patients. There was a significant difference in haemoglobin, white cell and platelet counts, red cell indices and bilirubin assay when G6PD deficient patients were compared with G6PD deficient controls.
Conclusion: This study suggests that G6PD deficiency has significant effect on sickle cell disease patients and may worsen the clinical course. Routine screening of all sickle cell disease patients for G6PD deficiency is therefore advocated.
