BackgroundAtaxia 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.ResultsGlucose 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.ConclusionsMetabolic 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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Summary Objective: To evaluate the stature growth rate (GR) and the weight gain of prepubertal asthmatic children. Method: A retrospective cohort study evaluating medical records of 85 children diagnosed with asthma, aged less than 9 years, of both sexes, with at least one year of follow-up in the allergy outpatient clinic. The data on the disease, weights and heights were collected through a standardized questionnaire on two occasions, with an interval of one year. The curves proposed by Tanner were applied for the analysis of the GR, and the Z-score of the GR (ZGR) was calculated. Results: Excess weight (risk for overweight, overweight and obesity) was observed in 31.8% (27/85) of the patients, but there was no association with the severity of asthma. Low GR (ZGR < -2) was found in 13.9% (11/79) of patients, most frequently among children with moderate/severe persistent asthma compared to persistent mild and intermittent forms (7/11 - 63.6% vs. 21/68 - 30.2%, respectively, p=0.047). Use of steroids (dose, type and time of use) was not associated with GR. Conclusion: GR was most affected in children with moderate/severe asthma.

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