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

Bibliographic Details
Main Author: Paulino, Talita Lemos [UNIFESP]
Publication Date: 2017
Other Authors: Rafael, Marina Neto [UNIFESP], Hix, Sonia, Shigueoka, David Carlos [UNIFESP], Ajzen, Sergio Aron [UNIFESP], Kochi, Cristiane, Suano-Souza, Fabiola Isabel [UNIFESP], da Silva, Rosangela, Costa-Carvalho, Beatriz T. [UNIFESP], Sarni, Roseli O. S. [UNIFESP]
Format: Article
Language: eng
Source: Repositório Institucional da UNIFESP
Download full: http://dx.doi.org/10.1186/s13023-017-0689-y
http://repositorio.unifesp.br/handle/11600/51399
Summary: 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
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spelling Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?Ataxia TelangiectasiaAtherosclerosisCarotid Intima-media thicknessInsulin resistanceFatty liver diseaseDyslipidemiaNutritional statusDiabetesBackground: 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 IRliver involvementcarotid 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 = 0.619p < 0.01), LDL/HDL-c (r = 0.490p < 0.05) and the Apo-B levels (r = 0.545p < 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.Fed Univ São Paulo UNIFESP, Escola Paulista Med, Dept Pediat, Rua Otonis 725, BR-04025002 São Paulo, SP, BrazilABC Fdn FMABC, Fac Med ABC, Dept Morphol & Physiol, Santo Andre, SP, BrazilFed Univ São Paulo UNIFESP, Escola Paulista Med, Dept Diagnost Imaging, São Paulo, SP, BrazilSanta Casa São Paulo Sch Med Sci FCMSCSP, São Paulo, SP, BrazilFed Univ Alfenas UNIFAL, Sch Nutr, Alfenas, MG, BrazilFed Univ São Paulo UNIFESP, Escola Paulista Med, Dept Pediat, Rua Otonis 725, BR-04025002 São Paulo, SP, BrazilFed Univ São Paulo UNIFESP, Escola Paulista Med, Dept Diagnost Imaging, São Paulo, SP, BrazilWeb of ScienceCAPES Foundation, Ministry of Education of Brazil, Brasilia DF, BrazilBiomed Central Ltd2019-08-19T11:49:46Z2019-08-19T11:49:46Z2017info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion-application/pdfhttp://dx.doi.org/10.1186/s13023-017-0689-yOrphanet Journal Of Rare Diseases. London, v. 12, p. -, 2017.10.1186/s13023-017-0689-yWOS000406954900001.pdf1750-1172http://repositorio.unifesp.br/handle/11600/51399WOS:000406954900001enginfo:eu-repo/semantics/openAccessPaulino, Talita Lemos [UNIFESP]Rafael, Marina Neto [UNIFESP]Hix, SoniaShigueoka, David Carlos [UNIFESP]Ajzen, Sergio Aron [UNIFESP]Kochi, CristianeSuano-Souza, Fabiola Isabel [UNIFESP]da Silva, RosangelaCosta-Carvalho, Beatriz T. [UNIFESP]Sarni, Roseli O. S. [UNIFESP]reponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESP2024-08-10T23:15:21Zoai:repositorio.unifesp.br/:11600/51399Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestbiblioteca.csp@unifesp.bropendoar:34652024-08-10T23:15:21Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false
dc.title.none.fl_str_mv 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?
spellingShingle Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
Paulino, Talita Lemos [UNIFESP]
Ataxia Telangiectasia
Atherosclerosis
Carotid Intima-media thickness
Insulin resistance
Fatty liver disease
Dyslipidemia
Nutritional status
Diabetes
title_short 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_sort Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
author Paulino, Talita Lemos [UNIFESP]
author_facet Paulino, Talita Lemos [UNIFESP]
Rafael, Marina Neto [UNIFESP]
Hix, Sonia
Shigueoka, David Carlos [UNIFESP]
Ajzen, Sergio Aron [UNIFESP]
Kochi, Cristiane
Suano-Souza, Fabiola Isabel [UNIFESP]
da Silva, Rosangela
Costa-Carvalho, Beatriz T. [UNIFESP]
Sarni, Roseli O. S. [UNIFESP]
author_role author
author2 Rafael, Marina Neto [UNIFESP]
Hix, Sonia
Shigueoka, David Carlos [UNIFESP]
Ajzen, Sergio Aron [UNIFESP]
Kochi, Cristiane
Suano-Souza, Fabiola Isabel [UNIFESP]
da Silva, Rosangela
Costa-Carvalho, Beatriz T. [UNIFESP]
Sarni, Roseli O. S. [UNIFESP]
author2_role author
author
author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv Paulino, Talita Lemos [UNIFESP]
Rafael, Marina Neto [UNIFESP]
Hix, Sonia
Shigueoka, David Carlos [UNIFESP]
Ajzen, Sergio Aron [UNIFESP]
Kochi, Cristiane
Suano-Souza, Fabiola Isabel [UNIFESP]
da Silva, Rosangela
Costa-Carvalho, Beatriz T. [UNIFESP]
Sarni, Roseli O. S. [UNIFESP]
dc.subject.por.fl_str_mv Ataxia Telangiectasia
Atherosclerosis
Carotid Intima-media thickness
Insulin resistance
Fatty liver disease
Dyslipidemia
Nutritional status
Diabetes
topic Ataxia Telangiectasia
Atherosclerosis
Carotid Intima-media thickness
Insulin resistance
Fatty liver disease
Dyslipidemia
Nutritional status
Diabetes
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
publishDate 2017
dc.date.none.fl_str_mv 2017
2019-08-19T11:49:46Z
2019-08-19T11:49:46Z
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://dx.doi.org/10.1186/s13023-017-0689-y
Orphanet Journal Of Rare Diseases. London, v. 12, p. -, 2017.
10.1186/s13023-017-0689-y
WOS000406954900001.pdf
1750-1172
http://repositorio.unifesp.br/handle/11600/51399
WOS:000406954900001
url http://dx.doi.org/10.1186/s13023-017-0689-y
http://repositorio.unifesp.br/handle/11600/51399
identifier_str_mv Orphanet Journal Of Rare Diseases. London, v. 12, p. -, 2017.
10.1186/s13023-017-0689-y
WOS000406954900001.pdf
1750-1172
WOS:000406954900001
dc.language.iso.fl_str_mv eng
language eng
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv -
application/pdf
dc.publisher.none.fl_str_mv Biomed Central Ltd
publisher.none.fl_str_mv Biomed Central Ltd
dc.source.none.fl_str_mv reponame:Repositório Institucional da UNIFESP
instname:Universidade Federal de São Paulo (UNIFESP)
instacron:UNIFESP
instname_str Universidade Federal de São Paulo (UNIFESP)
instacron_str UNIFESP
institution UNIFESP
reponame_str Repositório Institucional da UNIFESP
collection Repositório Institucional da UNIFESP
repository.name.fl_str_mv Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)
repository.mail.fl_str_mv biblioteca.csp@unifesp.br
_version_ 1841453516278726656

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