Abstract

ABSTRACT Background: The prevalence of sickle cell disease (SCD) in western Odisha is 9.1 % which is a quite high figure if one looks at the morbidity and mortality out of it. In sickle-cell disease, low-oxygen tension promotes RBC sickling and repeated episodes of sickling damages the cell membrane and makes it rigid. Free radical promotes increased and irreversible sickling of RBC membrane by damaging it. Several newly emerging biological markers have potentiality to be utilized in deciding the degree of oxidative stress and disease severity. Aims &
Objective: We studied the oxidative stress which was assessed in sickle cell disease patients of western Odisha by measuring serum MDA as a by product of lipid peroxidation, with simultaneous measurement of levels of antioxidant vitamins.
Materials and Methods: A prospective single centered case control study was done over a period of 1 year ending in July 2012. A total of 50 sickle cell disease children and 40 healthy children aged between 5 to 15 years of either sex were evaluated clinically and were screened by sickling test and diagnoses confirmed by haemoglobin electrophoresis and were lebelled as case (Both AS and SS). The estimation of uric acid, serum malondialdhyde, serum vitamin E and plasma vitamin C were done.
Results: Out of 50 patients 30 cases were homozygous (SS) and 20 cases were heterozygous (AS) for sickle cell as per Hb electrophoresis. There was a significant decrease in the level of Hb in homozygous (SS) cases (8.82 ± 1.05) while a moderate decline was noted in heterozygous (AS) patients (11.79 ± 1.14). Conclusion: The hyperbilirubinemia, hyperuricemia, decreased levels of antioxidants like vitamin C and vitamin E and simulataneous increased level of serum MDA, strongly points towards high oxidative stress in these patients. With increasing accuracy and utility of different methods for measurements of these biomarkers for sickle cell disease is shown to have a cause and effect relationship.