Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
| Autor(a) principal: | |
|---|---|
| Data de Publicação: | 2017 |
| Outros Autores: | , , , , , , , , |
| Tipo de documento: | Artigo |
| Idioma: | eng |
| Título da fonte: | Repositório Institucional da UNIFESP |
| Texto Completo: | http://repositorio.unifesp.br/handle/11600/51399 http://dx.doi.org/10.1186/s13023-017-0689-y |
Resumo: | 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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Paulino, 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]2019-08-19T11:49:46Z2019-08-19T11:49:46Z2017Orphanet Journal Of Rare Diseases. London, v. 12, p. -, 2017.1750-1172http://repositorio.unifesp.br/handle/11600/51399http://dx.doi.org/10.1186/s13023-017-0689-yWOS000406954900001.pdf10.1186/s13023-017-0689-yWOS:000406954900001Background: 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.CAPES Foundation, Ministry of Education of Brazil, Brasilia DF, BrazilFed 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 Science-engBiomed Central LtdAtaxia TelangiectasiaAtherosclerosisCarotid Intima-media thicknessInsulin resistanceFatty liver diseaseDyslipidemiaNutritional statusDiabetesIs age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleinfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESPORIGINALWOS000406954900001.pdfapplication/pdf665252${dspace.ui.url}/bitstream/11600/51399/1/WOS000406954900001.pdfea46ca360f2a97f0b041f9742fac81e0MD51open accessTEXTWOS000406954900001.pdf.txtWOS000406954900001.pdf.txtExtracted texttext/plain35078${dspace.ui.url}/bitstream/11600/51399/2/WOS000406954900001.pdf.txtae323a6b558ac93a64e261448da0f8fcMD52open accessTHUMBNAILWOS000406954900001.pdf.jpgWOS000406954900001.pdf.jpgIM Thumbnailimage/jpeg7019${dspace.ui.url}/bitstream/11600/51399/4/WOS000406954900001.pdf.jpg2458871b11a7769c764dea2d7531ba15MD54open access11600/513992022-08-01 18:20:28.532open accessoai:repositorio.unifesp.br:11600/51399Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestopendoar:34652022-08-01T21:20:28Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false |
| dc.title.en.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.eng.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 |
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2017 |
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2019-08-19T11:49:46Z |
| dc.date.available.fl_str_mv |
2019-08-19T11:49:46Z |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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article |
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publishedVersion |
| dc.identifier.citation.fl_str_mv |
Orphanet Journal Of Rare Diseases. London, v. 12, p. -, 2017. |
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http://repositorio.unifesp.br/handle/11600/51399 http://dx.doi.org/10.1186/s13023-017-0689-y |
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1750-1172 |
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WOS000406954900001.pdf |
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10.1186/s13023-017-0689-y |
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WOS:000406954900001 |
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Orphanet Journal Of Rare Diseases. London, v. 12, p. -, 2017. 1750-1172 WOS000406954900001.pdf 10.1186/s13023-017-0689-y WOS:000406954900001 |
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http://repositorio.unifesp.br/handle/11600/51399 http://dx.doi.org/10.1186/s13023-017-0689-y |
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eng |
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eng |
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openAccess |
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- |
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Biomed Central Ltd |
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Biomed Central Ltd |
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