ABSTRACT
Background: Sickle cell anemia (SCA), which is an inherited blood disorder characterized primarily by chronic anemia and oxidative stress plays a major role in pathophysiology. Objective: This study aims to evaluate vitamin A (serum retinol) status and hematological parameters in children with homozygous and heterozygous sickle cell disorders and compared with age- and sex-matched healthy controls. Materials and Methods:A sample of 80 referred cases (37 sickle cell disorders and 43 normal cases) aged 2-40 years were included in the study. Hematological parameters were measured in cell counter and serum retinol by high-performance liquid chromatography. Results: The mean hemoglobin (Hb) and serum retinol were significantly lower among cases with sickle cell disease than in sickle cell trait and normal. Vitamin A deficiency (retinol < 20 μg/dl) reported to be higher in homozygous cases (46.2%) as compared to either heterozygous (29.2%) or control (23.2%) groups. Serum retinol was correlated directly with Hb, RBC count, and hematocrit levels, and inversely with percentage of sickling among sickle cell disorder cases. Conclusion: The results indicate that deprived vitamin A status with inductive oxidative stress is mainly due to sickling and hemolysis in SCA cases.