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

Content Provider	Springer Nature : BioMed Central
Author	Andrade, Itana Gomes Alves Suano-Souza, Fabíola Isabel Fonseca, Fernando Luiz Affonso Lago, Carolina Sanchez Aranda Sarni, Roseli Oselka Saccardo
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.
Related Links	https://ojrd.biomedcentral.com/counter/pdf/10.1186/s13023-021-01732-5.pdf
Ending Page	10
Page Count	10
Starting Page	1
File Format	HTM / HTML
ISSN	17501172
DOI	10.1186/s13023-021-01732-5
Journal	Orphanet Journal of Rare Diseases
Issue Number	1
Volume Number	16
Language	English
Publisher	BioMed Central
Publisher Date	2021-02-12
Access Restriction	Open
Subject Keyword	Medicine Public Health Pharmacology Toxicology Human Genetics Ataxia-telangiectasia Primary immunodeficiency Dyslipidemia Oxidative stress Cardiovascular risk Selenium Medicine/Public Health Pharmacology/Toxicology
Content Type	Text
Resource Type	Article
Subject	Pharmacology (medical) Genetics (clinical)
Journal Impact Factor	3.4/2023
5-Year Journal Impact Factor	3.9/2023

Sl.	Authority	Responsibilities	Communication Details
1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
2	Indian Institute of Technology Kharagpur	Host Institute of the Project: The host institute of the project is responsible for providing infrastructure support and hosting the project	https://www.iitkgp.ac.in
3	National Digital Library of India Office, Indian Institute of Technology Kharagpur	The administrative and infrastructural headquarters of the project	Dr. B. Sutradhar bsutra@ndl.gov.in
4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
5	Website/Portal (Helpdesk)	Queries regarding NDLI and its services	support@ndl.gov.in
6	Contents and Copyright Issues	Queries related to content curation and copyright issues	content@ndl.gov.in
7	National Digital Library of India Club (NDLI Club)	Queries related to NDLI Club formation, support, user awareness program, seminar/symposium, collaboration, social media, promotion, and outreach	clubsupport@ndl.gov.in
8	Digital Preservation Centre (DPC)	Assistance with digitizing and archiving copyright-free printed books	dpc@ndl.gov.in
9	IDR Setup or Support	Queries related to establishment and support of Institutional Digital Repository (IDR) and IDR workshops	idr@ndl.gov.in

Selenium-related nutritional status in patients with common variable immunodeficiency: association with oxidative stress and atherosclerosis risk

Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?

Oxidative stress assessment by glutathione peroxidase activity and glutathione levels in response to selenium supplementation in patients with Mucopolysaccharidosis I, II and VI

Body composition, muscle strength and hormonal status in patients with ataxia telangiectasia: a cohort study

Growth in ataxia telangiectasia

SELENIUM STATUS, GLUTATHIONE PEROXIDASE ACTIVITY AND LIPID PEROXIDES CONCENTRATION IN BLOOD OF MULTIPLE SCLEROSIS PATIENTS

Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia

Ataxia telangiectasia: a review

Glutathione peroxidase activity, lipid peroxides and selenium status in blood in patients with down's syndrome (1986).

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

Similar Documents

Selenium-related nutritional status in patients with common variable immunodeficiency: association with oxidative stress and atherosclerosis risk

Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?

Oxidative stress assessment by glutathione peroxidase activity and glutathione levels in response to selenium supplementation in patients with Mucopolysaccharidosis I, II and VI

Body composition, muscle strength and hormonal status in patients with ataxia telangiectasia: a cohort study

Growth in ataxia telangiectasia

SELENIUM STATUS, GLUTATHIONE PEROXIDASE ACTIVITY AND LIPID PEROXIDES CONCENTRATION IN BLOOD OF MULTIPLE SCLEROSIS PATIENTS

Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia

Ataxia telangiectasia: a review

Glutathione peroxidase activity, lipid peroxides and selenium status in blood in patients with down's syndrome (1986).

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