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Sickle cell disease (SCD) is an inherited disorder that causes many harmful pathological effects. Patients with SCD were found to exhibit a state of chronic oxidative stress with a higher potential for oxidative damage due to increased resting oxygen consumption and circulating pro-oxidative free hemoglobin [1]. Further sources of oxidants which are unique to SCD vasculature include xanthine oxidase, nitric oxide and secondary oxides of nitrogen [2]. Previous studies had shown correlation between markers of oxidative stress and common secondary diseases in SCD, such as acute chest syndrome and pulmonary hypertension [3]. Other disease related complications include infections, neurological, cardiac manifestations, and cognitive deficits [4]. Vitamins and trace minerals are known key buffers against oxidative damage. Chronic increase in the oxidative stress results in overuse of oxidative buffering capacity and may produce conditional deficiencies in key amino acids and enzymatic cofactors [5]. There are several published series demonstrating reduced levels of antioxidant vitamins, enzymes and trace elements like; zinc, selenium, glutathione, vitamins A, C, riboflavin, D, and E [6–8], as well as increased markers of lipid peroxidation [9] in SCD patients. The cognitive disabilities in SCD patients include deficits in short-term memory and difficulties in verbal tasks that lead to declining IQ scores and learning difficulties [10]. Few previous studies had evaluated the cognition in relation to oxidative stress in SCD patients, but ‘to our knowledge’ none of them evaluated the effects of antioxidant on cognition in children. In this work, we aimed to assess the cognitive impairment in Egyptian children with sickle cell disease and to investigate two antioxidants (vitamin E and selenium) levels and their relationship to IQ values among such group of patients. Materials and methods A case-control study that included fifty-five SCD patients less than 18 years old, who presented to the Hematology Clinic for follow-up. Patients were diagnosed as SCD based on conventional clinical and hematologic criteria. Patients on antioxidant medications and those with known organic brain lesions, autistic spectrum disorder, acute infection or inflammation were excluded from the study. Fifty-five apparently healthy subjects with frequency matched age & sex were included and served as a control group. Consents were obtained from the patients and their legal guardians before enrollment in the study. The study protocol was approved by the Ethical Committee of Cairo University, according to the 18th World Medical Assembly, Helsinki, Finland. Clinical data and the most recent routine laboratory data were retrieved from medical records as well as patient interviewing. Disease severity was assessed using scoring system for clinical evaluation of SCD patients [11] (Table 1). Samples were collected and processed according to clinical laboratory specifications, and results were expressed as absolute values as well as percent of patients outside the reference ranges. A blood sample (10 ml) was collected from subjects in the morning after at least 8 h fasting and all samples from patients were collected just before the transfusion. Blood was transported at room temperature to the analytical laboratory within 20 min of venipuncture for cell isolation and oxidation analysis. Serum, plasma and cells were separated by centrifugation. Selenium level was assessed using Varian Spectr AA 220 Atomic Absorption Spectrometer equipped with a graphite furnace atomizer and an auto-sampler. Vitamin E assessment has been carried out by Enzyme Linked Immunosorbent Assay (ELlSA); which is based on the competitive binding enzyme immunoassay technique and the concentration of Vitamin E in the samples was determined by comparing the optical density of the samples to the standard curve [12]. Assessment of cognitive functions was carried out for all patients and controls using The Stanford-Binet Intelligence Scale, 4th Ed. (SB-IV) [13]; it consists of 15

Cognitive functions and anti-oxidant in children with sickle cell disease: A single center based study