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

• Published in: Orphanet journal of rare diseases Vol. 16; no. 1; p. 83
• Main Authors: Andrade, Itana Gomes Alves, Suano-Souza, Fabíola Isabel, Fonseca, Fernando Luiz Affonso, Lago, Carolina Sanchez Aranda, Sarni, Roseli Oselka Saccardo
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 12.02.2021; BioMed Central; BMC
• Subjects: Ataxia; Ataxia telangiectasia; Atherosclerosis; Biomarkers; Body composition; Cardiovascular risk; Cholesterol; Classification; Complications and side effects; Disease; Dyslipidemia; Dyslipidemias; Food; Food intake; Glucose; Glutathione peroxidase; Health aspects; High density lipoprotein; Immunology; Insulin resistance; Lipids; Lipoproteins; Low density lipoprotein; Malondialdehyde; Measurement; Medical research; Metabolic disorders; Nutritional status; Oxidative stress; Patients; Peroxidase; Plasma levels; Primary immunodeficiency; Rare diseases; Risk factors; Selenium; Software; Statistical analysis; Teenagers; Trace elements (nutrients); Triglycerides; Values; Variables; Brazil; Oxidative stress; Ataxia-telangiectasia; Selenium; Cardiovascular risk; Dyslipidemia; Primary immunodeficiency
• ISSN: 1750-1172; 1750-1172
• DOI: 10.1186/s13023-021-01732-5

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 is a trace element related to protection against damage caused by oxidative stress. To 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. This 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. Adequate 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 μg/L (43.2-57.0) vs 54.6 (45.2-62.6) μg/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 µg/L vs 2.8 µg/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. In 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.