Résumé :
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Introduction : Respiratory muscle training (RMT) enhances inspiratory muscle strength, but there is no evidence of effect on forced vital capacity. Objective : To evaluate prospectively the effects on PImax and FVC of a respiratory muscle training protocol. Material and Methods : Thirteen children of the Non Invasive Ventilatory Assistance (AVNI) Program were evaluated. PImax was measured with Black & Hyatt technique by ATS guidelines, and then RMT was started using threshold valves with loads of 30% of PImax, five days a week. Basal PImax was registered and every three months, over one year. Statistical analysis using t-student test (p<0,001) was applied for changes in PImax and FVC. Reference values of Contreras for PImax and Knudson for FVC were used. Results were expressed as percentage of lower limit and predict. Results : Total of 13 children, 10 were boys, mean age 14,8±2,3 years). Diagnosis : Duchenne muscular dystrophy 6, Spinal muscular atrophy II 3, Congenital myopathy 3 and Myelomeningocele 1. Ventilatory assistance : non invasive 6. Mean PImax : basal 66,6±22,2 %, at third month 87±26 %, at sixth month 6 89,5±27,6 %, at ninth month 97,2±33,6 %, at twelfth month 102,6±34,5 %. Mean FVC : basal 61,2±26,6 % and at twelfth month 63,2±31,7 %. Significative increases in PImax were noted at third (p<0,0003), sixth (p<0,0016), ninth (p<0,0031) and twelfth months (p<0,0008). After one year of RMT increase in mean PImax was 36% (p<0,0008). Discussion : The RMT protocol used in patients with NMD improved inspiratory muscle weakness. No effect was noted in FVC, as expected in natural history of NMD.No
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