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

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.

Growth velocity and weight gain in prepubertal asthmatic children.

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.

Growth velocity and weight gain in prepubertal asthmatic children / Velocidade de crescimento e ganho de peso em crianças pré-púberes asmáticas

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.

Resumo Objetivo: Avaliar a velocidade de crescimento estatural e o ganho de peso de crianças asmáticas pré-púberes. Método: Estudo de coorte retrospectiva que avaliou 85 prontuários de crianças com diagnóstico de asma, menores de 9 anos, de ambos os sexos, com no mínimo um ano de acompanhamento no ambulatório de alergia. Os dados relativos à doença, aos pesos e às alturas foram coletados por meio de questionário padronizado em dois momentos com intervalo de um ano. Para análise da velocidade de crescimento (VC), foram empregadas as curvas propostas por Tanner e realizado o cálculo do escore Z da VC (ZVC). Resultados: O excesso de peso (risco para sobrepeso, sobrepeso e obesidade) foi observado em 31,8% (27/85) dos pacientes, mas sem associação com a gravidade da asma. Velocidade de crescimento baixa (ZVC < -2) foi encontrada em 13,9% (11/79) dos pacientes, com maior frequência entre as crianças com asma persistente moderada/grave em relação às formas persistente leve e intermitente (7/11 - 63,6% vs. 21/68 - 30,2%, respectivamente; p=0,047). O uso de corticosteroides (dose, tipo e tempo de uso) não mostrou associação com a VC. Conclusão: Foi constatado um maior comprometimento da VC em crianças com asma moderada/grave.