Is age a risk factor for liver disease and metabolic alterations in ataxia Telangiectasia patients?
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Data
2017
Autores
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]
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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.
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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Orphanet Journal Of Rare Diseases. London, v. 12, p. -, 2017.