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

BACKGROUND: Ataxia telangiectasia (A-T) is a neurodegenerative disease that leads to mitochondrial dysfunction and oxidative stress. Insulin resistance (IR), type 2 diabetes and the risk for development of cardiovascular disease was recently associated as an extended phenotype of the disease. We aim...

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Autores principales: Paulino, Talita Lemos, Rafael, Marina Neto, Hix, Sonia, Shigueoka, David Carlos, Ajzen, Sergio Aron, Kochi, Cristiane, Suano-Souza, Fabíola Isabel, da Silva, Rosangela, Costa-Carvalho, Beatriz T., Sarni, Roseli O. S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5545012/
https://www.ncbi.nlm.nih.gov/pubmed/28778179
http://dx.doi.org/10.1186/s13023-017-0689-y
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author Paulino, Talita Lemos
Rafael, Marina Neto
Hix, Sonia
Shigueoka, David Carlos
Ajzen, Sergio Aron
Kochi, Cristiane
Suano-Souza, Fabíola Isabel
da Silva, Rosangela
Costa-Carvalho, Beatriz T.
Sarni, Roseli O. S.
author_facet Paulino, Talita Lemos
Rafael, Marina Neto
Hix, Sonia
Shigueoka, David Carlos
Ajzen, Sergio Aron
Kochi, Cristiane
Suano-Souza, Fabíola Isabel
da Silva, Rosangela
Costa-Carvalho, Beatriz T.
Sarni, Roseli O. S.
author_sort Paulino, Talita Lemos
collection PubMed
description BACKGROUND: Ataxia telangiectasia (A-T) is a neurodegenerative disease that leads to mitochondrial dysfunction and oxidative stress. Insulin resistance (IR), type 2 diabetes and the risk for development of cardiovascular disease was recently associated as an extended phenotype of the disease. We aimed to assess IR; liver involvement; carotid intima-media thickness (cIMT) and metabolic alterations associated to cardiovascular risk in A-T patients, and relate them with age. RESULTS: Glucose metabolism alterations were found in 54.6% of the patients. Hepatic steatosis was diagnosed in 11/17 (64.7%) A-T patients. AST/ALT ratio > 1 was observed in 10/17 (58.8%). A strong positive correlation was observed between insulin sum concentrations with ALT (r = 0.782, p < 0.004) and age (r = 0.818, p = 0.002). Dyslipidemia was observed in 55.5% of the patients. The apolipoprotein (Apo-B)/ApoA-I ratio (r = 0.619; p < 0.01), LDL/HDL-c (r = 0.490; p < 0.05) and the Apo-B levels (r = 0.545; p < 0.05) were positively correlated to cIMT. CONCLUSIONS: Metabolic disorders implicated in cardiovascular and liver diseases are frequently observed in adolescent A-T patients and those tend to get worse as they become older. Therefore, nutritional intervention and the use of drugs may be necessary.
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spelling pubmed-55450122017-08-07 Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients? Paulino, Talita Lemos Rafael, Marina Neto Hix, Sonia Shigueoka, David Carlos Ajzen, Sergio Aron Kochi, Cristiane Suano-Souza, Fabíola Isabel da Silva, Rosangela Costa-Carvalho, Beatriz T. Sarni, Roseli O. S. Orphanet J Rare Dis Research BACKGROUND: Ataxia telangiectasia (A-T) is a neurodegenerative disease that leads to mitochondrial dysfunction and oxidative stress. Insulin resistance (IR), type 2 diabetes and the risk for development of cardiovascular disease was recently associated as an extended phenotype of the disease. We aimed to assess IR; liver involvement; carotid intima-media thickness (cIMT) and metabolic alterations associated to cardiovascular risk in A-T patients, and relate them with age. RESULTS: Glucose metabolism alterations were found in 54.6% of the patients. Hepatic steatosis was diagnosed in 11/17 (64.7%) A-T patients. AST/ALT ratio > 1 was observed in 10/17 (58.8%). A strong positive correlation was observed between insulin sum concentrations with ALT (r = 0.782, p < 0.004) and age (r = 0.818, p = 0.002). Dyslipidemia was observed in 55.5% of the patients. The apolipoprotein (Apo-B)/ApoA-I ratio (r = 0.619; p < 0.01), LDL/HDL-c (r = 0.490; p < 0.05) and the Apo-B levels (r = 0.545; p < 0.05) were positively correlated to cIMT. CONCLUSIONS: Metabolic disorders implicated in cardiovascular and liver diseases are frequently observed in adolescent A-T patients and those tend to get worse as they become older. Therefore, nutritional intervention and the use of drugs may be necessary. BioMed Central 2017-08-04 /pmc/articles/PMC5545012/ /pubmed/28778179 http://dx.doi.org/10.1186/s13023-017-0689-y Text en © The Author(s). 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research
Paulino, Talita Lemos
Rafael, Marina Neto
Hix, Sonia
Shigueoka, David Carlos
Ajzen, Sergio Aron
Kochi, Cristiane
Suano-Souza, Fabíola Isabel
da Silva, Rosangela
Costa-Carvalho, Beatriz T.
Sarni, Roseli O. S.
Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
title Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
title_full Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
title_fullStr Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
title_full_unstemmed Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
title_short Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
title_sort is age a risk factor for liver disease and metabolic alterations in ataxia telangiectasia patients?
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5545012/
https://www.ncbi.nlm.nih.gov/pubmed/28778179
http://dx.doi.org/10.1186/s13023-017-0689-y
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