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. 2021 Feb 12;16(1):83.
doi: 10.1186/s13023-021-01732-5.

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

Itana Gomes Alves Andrade  1 , Fabíola Isabel Suano-Souza  2 , Fernando Luiz Affonso Fonseca  3 , Carolina Sanchez Aranda Lago  2 , Roseli Oselka Saccardo Sarni  2   4
Affiliations

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

Itana Gomes Alves Andrade et al. Orphanet J Rare Dis. .

Abstract

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 is a trace element related to protection against damage caused by oxidative stress.

Objective: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: Ataxia-telangiectasia; Cardiovascular risk; Dyslipidemia; Oxidative stress; Primary immunodeficiency; Selenium.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Discriminatory analysis using the ROC curve of the biochemical variables evaluated in A-T patients and the control group
Fig. 2
Fig. 2
Discriminatory analysis using a multivariate ROC analysis of variables related to oxidative stress (a) and lipid status (b) with selenium levels (≤ 46 µg/L or > 46 µg/L)
See this image and copyright information in PMC

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