See the published version of this preprint at Orphanet Journal of Rare Diseases.
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Itana Gomes Alves Andrade
Universidade Federal de Sao Paulo Escola Paulista de Medicina
Corresponding Author
ORCiD: https://orcid.org/0000-0003-2229-5359
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This work is licensed under a CC BY 4.0 License. Read Full License
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 ug/L (43.2-57.0) vs 54.6 (45.2-62.6) ug/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-I, LDL-c/HDL-c ratio, malondialdehyde [3.8 µg/L vs 2.8 µg/L, p=0.029] and lower Apo-A-I/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, Primary Immunodeficiency, dyslipidemia, oxidative stress, cardiovascular risk, selenium.
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View the published article at Orphanet Journal of Rare Diseases.
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Received 31 Dec, 2020
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On 25 Dec, 2020
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Received 25 Dec, 2020
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On 21 Dec, 2020
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On 21 Dec, 2020
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Editorial decision: Major revision
On 09 Nov, 2020
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Received 02 Nov, 2020
Review #2 received
Received 02 Nov, 2020
Reviewer #4 agreed
On 26 Oct, 2020
Reviewer #3 agreed
On 25 Oct, 2020
Reviewer #2 agreed
On 23 Oct, 2020
Review #1 received
Received 18 Sep, 2020
Reviewers invited
Invitations sent on 07 Sep, 2020
Reviewer #1 agreed
On 07 Sep, 2020
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On 02 Sep, 2020
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See the published version of this preprint at Orphanet Journal of Rare Diseases.
This is a preprint, a preliminary version of a manuscript that has not completed peer review at a journal. Research Square does not conduct peer review prior to posting preprints. The posting of a preprint on this server should not be interpreted as an endorsement of its validity or suitability for dissemination as established information or for guiding clinical practice.
Learn more about In Review
Research
Itana Gomes Alves Andrade
Universidade Federal de Sao Paulo Escola Paulista de Medicina
Corresponding Author
ORCiD: https://orcid.org/0000-0003-2229-5359
Badges
Prescreen
This manuscript passed our Prescreen assessment
Complete author information
Appropriate declaration statements
Potential risks to human health
Prescreen
Peer Review Timeline
CURRENT STATUS: Published
View the published article at Orphanet Journal of Rare Diseases.
Version 3
Posted 28 Jan, 2021
No community comments so far
Editor assigned
On 21 Jan, 2021
Submission checks complete
On 21 Jan, 2021
Editor invited
On 21 Jan, 2021
No comments provided
Editorial decision: Minor revision
On 01 Jan, 2021
Review #2 received
Received 31 Dec, 2020
Reviewer #2 agreed
On 25 Dec, 2020
Reviewer #1 agreed
On 25 Dec, 2020
Review #1 received
Received 25 Dec, 2020
Reviewers invited
Invitations sent on 22 Dec, 2020
Editor assigned
On 21 Dec, 2020
Submission checks complete
On 21 Dec, 2020
Editor invited
On 21 Dec, 2020
No comments provided
Editorial decision: Major revision
On 09 Nov, 2020
Review #3 received
Received 02 Nov, 2020
Review #2 received
Received 02 Nov, 2020
Reviewer #4 agreed
On 26 Oct, 2020
Reviewer #3 agreed
On 25 Oct, 2020
Reviewer #2 agreed
On 23 Oct, 2020
Review #1 received
Received 18 Sep, 2020
Reviewers invited
Invitations sent on 07 Sep, 2020
Reviewer #1 agreed
On 07 Sep, 2020
Editor assigned
On 03 Sep, 2020
First submitted
On 02 Sep, 2020
Submission checks complete
On 02 Sep, 2020
Editor invited
On 02 Sep, 2020
Metrics
Comments: 0
PDF Downloads: ...
HTML Views: ...
License:
This work is licensed under a CC BY 4.0 License. Read Full License
View the published article at Orphanet Journal of Rare Diseases.
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 ug/L (43.2-57.0) vs 54.6 (45.2-62.6) ug/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-I, LDL-c/HDL-c ratio, malondialdehyde [3.8 µg/L vs 2.8 µg/L, p=0.029] and lower Apo-A-I/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.
Figure 1
Figure 2
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