Respiratory rehabilitation in neuromuscular patients: effects on exercise tolerance and respiratory musclesBackground: The pulmonary rehabilitation (PR) has acquired an important role in neuromuscular patient treatment. The aim was to investigate the effects of PR program on 6-minute walking test variables (6MWT) and respiratory muscle function (RMF) in patients with neuromuscular disease. Patients and Methods: In the study were included 13 patients, age 12.5 ± 2.8 years old. Seven of them were able to walk (2 Facioscapulohumeral Muscular Dystrophy (FSHD), 1 Becker Muscular Dystrophy, 1 Congenital Myopathy, 1 Bethlem Syndrome, 2 Duchenne Muscular Dystrophy); and 6 were unable to walk (4 Duchenne Muscular Dystrophy, 1 Spinal Muscular Atrophy (SMA) type III and 1 SMA II). The results of RMF and 6MWT before and after 13 weeks of respiratory muscle training (RMT) (40-50% Pimax and 60-70% Pemax) and aerobic training (AT) (50% heart rate reserve) were analyzed. Wilcoxon test with 95% confidence interval was used to assess statistical significance. Results: Significant changes (p < 0.05) were observed in the averages of dyspnea score that decreased in 1.8 points (from 4.4 to 2.6; -40.9%) and in sustained inspiratory pressure (Pims) that increased in 14.3 cm H 2 O (from 16 to 30.3; +89.4%). Other variables that assessed aerobic capacity (6 MWT, heart rate and leg fatigue) and RMF (Pimax and Pemax) showed a trend towards improvement, but did not reach statistical significance. Conclusion: In patients with neuromuscular diseases significant changes in dyspnea after cardiopulmonary training protocol were observed. Moreover, the moderate intensity respiratory muscle training was well tolerated and an effective method to generate significant increases in Pims, constituting an alternative to previously established protocols.

Polygraphy in children with neuromuscular diseaseIntroduction: A high prevalence of sleep-disordered breathing (SDB)