Nutritional Status Related to Selenium in Patients With Ataxia-Telangiectasia - A Case Control Study: an Association With Oxidative Stress and Risk of Atherosclerosis

Introduction: Ataxia-Telangiectasia (A-T) is a multi-system disorder that may be associated with endocrine changes, oxidative stress in addition to inflammation. Studies suggest that selenium (Se) is a trace element related to protection against damage caused by oxidative stress; it is postulated that adequate consumption reduces the risk of some chronic diseases. Objective: To describe the concentrations of Se and glutathione peroxidase (GPx) in patients with A-T, to relate them to markers of the lipid profile. Methods: We evaluated, through a controlled cross-sectional study, 22 A-T patients matched by sex and age with healthy individuals, conjointly evaluating: nutritional status, food intake, serum selenium, glutathione peroxidase (activity), lipid metabolism biomarkers, inflammation and lipid. Results: The median age in the A-T group was 12.2 years. A-T patients had greater impairment of lean body mass and GPx activity as well as lower abdominal circumference. A more atherogenic lipid profile was observed with higher concentrations of total cholesterol, non-HDL cholesterol, LDLox, Apo B, Apo B / Apo A-1 and LDL / HDL ratio; while a lower value was observed in the Apo A-1 / HDL ratio. It was also in the A-T group that statistical difference was detected in the three markers of liver function AST, ALT and GGT. In regard to food intake, A-T patients had lower values ​​of carbohydrate, protein, monounsaturated fat, trans fat, and Se. Conclusion: The study showed cardiovascular risk in A-T patients. A-T patients appear to be at increased risk of reduced nutritional status, impaired liver function, dyslipidemia and inflammation.