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Research
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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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.
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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On 21 Jan, 2021
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On 21 Jan, 2021
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Editorial decision: Minor revision
On 01 Jan, 2021
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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
Version 1
Posted 09 Sep, 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
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On 02 Sep, 2020
Editor invited
On 02 Sep, 2020
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A new preprint design is coming!
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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.
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
Version 1
Posted 09 Sep, 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 (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.
Figure 1
Figure 2
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