Lung functions and oxidative status in sickle cell disease and sickle cell trait

Abstract

Background and Aim: Sickle cell disease (SCD) is a genetic hemoglobinopathy. SCD is associated with increased production of reactive oxygen species resulting in hemolysis, endothelial dysfunction, cell adhesion, and vaso-occlusion. Pulmonary disease is a major cause of morbidity and mortality in SCD. Hence, in the present study we have planned to assess the pro-oxidant and anti-oxidant status and lung function tests in patients with SCD, and sickle cell trait (SCT). Methods: Freshly diagnosed cases of SCD and SCT (based on sickle cell hemoglobin [HbSS] and normal hemoglobin [HbA] pattern by hemoglobin electrophoresis) were included in the study. The lung function tests and oxidative stress parameters namely plasma malondialdehyde (MDA), whole blood superoxide dismutase (SOD) and plasma Vitamin C levels were assessed. Results: Forced vital capacity, forced expiratory volume in 1 s, maximum mid-expiratory flow rate, peak expiratory flow rate and maximum voluntary ventilation were reduced in patients with SCD and SCT. MDA and SOD levels increased significantly and Vitamin C levels decreased significantly in both homozygous and heterozygous cases of sickle cell anemia. Conclusion: The lung functions were compromised, and oxidative stress was increased in patients with SCD and SCT. The changes were more in SCD.