Abstract
Sickle cell disease is characterized by a chain of polymerization reactions in the deoxygenated phase, manifesting in debilitating conditions like inflammation, painful vaso-occlusive crisis and disruption of the bodily defense systems. This vaso-occlusion accompanied by cell adhesion and ischemia-reperfusion injury is linked to a vicious pathway resulting in oxidative stress and an enhanced free radical generation. In children with sickle cell disease, the antioxidant defense system is continuously challenged, resulting in a compromised immunity, and a host of complications. An early, easy, and rapid technique to assess the oxidative stress would help in early therapeutic interventions. As prevalence of sickle cell disease is high in Saudi Arabia, the need for early interventions in children with the problem is of dire necessity. Currently hydroxyurea is the only drug of choice administered. We therefore utilized the free oxygen radical transference (FORT), and free oxygen radical defense (FORD) measurements in children with sickle cell disease on hydroxyurea therapy and compared them with patients not taking the drug. Though patients of both the groups exhibited oxidative stress, the values of free radical transference were considerably higher in the group which did not undertake any treatment as compared to those on hydroxyurea therapy. No appreciable changes were noticed in the FORD values representing the antioxidant capacity. Our results show that the technique is feasible for quick measurements of oxidative stress, and intervention with hydroxyurea therapy benefits in decreasing it. Its incorporation in screening practices would help understand the disease stage better. Abbreviations- SCD-sickle cell disease, FORT- free oxygen radical transference, FORD- free oxygen radical defense
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