Résumé :
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Neuromuscular disorders (NMD) are the most important cause of hypercapnic respiratory insufficiency in childhood and respiratory failure is the major cause of morbidity and mortality in NMD. The use of non-invasive ventilation (NIV) in NMD patients can improve the prognosis by correction of hypoventilation and reduction of respiratory work. However, it does not prevent acute respiratory failure during recurrent episodes of pneumonia or atelectasis due to inability to clear airways from secretions. Combined non-invasive ventilation with mechanical insufflation-exsufflation (MI-E), by means of mechanical cough-assistance, can ameliorate these conditions in neuromuscular patients. We have performed a retrospective evaluation of the safety, tolerance and effectiveness of this combined treatment in 27 patients affected by NMD (Duchenne and Becker Muscular Dystrophy=5, Limb Girdle Muscular Dystrophy=1, Spinal Muscular Atrophy=8, Congenital Myopathy=8, Ullrich’s Syndrome=4, Congenital Myotonic Dystrophy=1), aging 2 months to 18 years. The follow-up of these patients has been developed through the guideline of the European Neuromuscular Centre and of the American Thoracic Society. All patients used nocturnal NIV and during broncopulmonary acute episodes were treated with MI-E cough-assistance, with a complete resolution. In particular, fourteen patients, who presented severe exacerbations of respiratory conditions with development of pulmonary acute infections and atelectasia, showed a significant improvement after MI-E treatment. Seven patients needed invasive ventilation, only one received tracheotomy, while six were extubated by Bach’s protocol. In conclusion, our data indicate the good tolerance and the effectiveness of the combined management of NIV and MI-E in pediatric neuromuscular patients with respiratory insufficiency.
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