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

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

Author Paulino, Talita Lemos Autor UNIFESP Google Scholar
Rafael, Marina Neto Autor UNIFESP Google Scholar
Hix, Sonia Google Scholar
Shigueoka, David Carlos Autor UNIFESP Google Scholar
Ajzen, Sergio Aron Autor UNIFESP Google Scholar
Kochi, Cristiane Google Scholar
Suano-Souza, Fabiola Isabel Autor UNIFESP Google Scholar
da Silva, Rosangela Google Scholar
Costa-Carvalho, Beatriz T. Autor UNIFESP Google Scholar
Sarni, Roseli O. S. Autor UNIFESP Google Scholar
Abstract 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.
Keywords Ataxia Telangiectasia
Atherosclerosis
Carotid Intima-media thickness
Insulin resistance
Fatty liver disease
Dyslipidemia
Nutritional status
Diabetes
Language English
Sponsor CAPES Foundation, Ministry of Education of Brazil, Brasilia DF, Brazil
Date 2017
Published in Orphanet Journal Of Rare Diseases. London, v. 12, p. -, 2017.
ISSN 1750-1172 (Sherpa/Romeo, impact factor)
Publisher Biomed Central Ltd
Extent -
Origin http://dx.doi.org/10.1186/s13023-017-0689-y
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000406954900001
URI http://repositorio.unifesp.br/handle/11600/51399

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