Selenium levels and glutathione peroxidase activity in patients with ataxia-telangiectasia: association with oxidative stress and lipid status biomarkers

Author(s)

Alves Andrade, Itana Gomes ^[1] ; Suano-Souza, Fabiola Isabel ^[1] ; Affonso Fonseca, Fernando Luiz ^[2] ; Aranda Lago, Carolina Sanchez ^[1] ; Saccardo Sarni, Roseli Oselka ^{[1, 3]}

Affiliation

^[1] Univ Fed Sao Paulo, Dept Pediat, Escola Paulista Med, Rua Dr Diogo Faria 671, BR-04037002 Sao Paulo, SP - Brazil

^[2] Univ Fed Sao Paulo, Campus Diadema, Sao Paulo, SP - Brazil

^[3] Ctr Univ Saude ABC, Fac Med ABC, Dept Pediat, Santo Andre, SP - Brazil

• Document type: Journal article
• Source: ORPHANET JOURNAL OF RARE DISEASES; v. 16, n. 1 FEB 12 2021.
• Web of Science Citations: 0

Abstract

IntroductionAtaxia-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 is a trace element related to protection against damage caused by oxidative stress. ObjectiveTo describe the plasma levels of selenium and erythrocyte glutathione peroxidase activity in A-T patients and to relate them to oxidative stress and lipid status biomarkers. MethodsThis is a cross-sectional and controlled study evaluating 22 A-T patients (age median, 12.2 years old) matched by gender and age with 18 healthy controls. We evaluated: nutritional status, food intake, plasma selenium levels, erythrocyte glutathione peroxidase activity, lipid status, inflammation and oxidative stress biomarkers.ResultsAdequate levels of selenium were observed in 24/36 (66.7%) in this evaluated population. There was no statistically significant difference between the groups in selenium levels {[}47.6 mu g/L (43.2-57.0) vs 54.6 (45.2-62.6) mu g/dL, p=0.242]. Nine of A-T patients (41%) had selenium levels below the reference value. The A-T group presented higher levels of LDL-c, non-HDL-c, oxidized LDL, Apo B, Apo-B/Apo-A-I1, LDL-c/HDL-c ratio, malondialdehyde {[}3.8 mu g/L vs 2.8 mu g/L, p=0.029] and lower Apo-A-I1/HDL-c and glutathione peroxidase activity {[}7300 U/L vs 8686 U/L, p=0.005]. Selenium levels were influenced, in both groups, independently, by the concentrations of oxidized LDL, malonaldehyde and non-HDL-c. The oxidized LDL (AUC=0.849) and ALT (AUC=0.854) were the variables that showed the greatest discriminatory power between groups.ConclusionIn conclusion, we observed the presence of selenium below the reference value in nearly 40% and low GPx activity in A-T patients. There was a significant, inverse and independent association between selenium concentrations and oxidative stress biomarkers. Those data reinforce the importance of assessing the nutritional status of selenium in those patients. (AU)

• FAPESP's process: 15/13308-9 - Nutritional status of selenium in patients with primary immunodeficiencies: association with oxidative stress and atherosclerosis risk
• Grantee: Roseli Oselka Saccardo Sarni
• Support Opportunities: Regular Research Grants