[Clinical characteristics of non-invasive ventilation treatment in children with spinal muscular atrophy and sleep disordered breathing].

To study the short-term and long-term efficacy of the non-invasive ventilation treatment in children with spinal muscular atrophy (SMA) and sleep-disordered breathing. This was a prospective research to study the effect of night-time non-invasive ventilation in children with SMA and moderate to severe sleep-disordered breathing during March 2016 to January 2018, from the Pulmonary Department of Capital Institute of Pediatrics Affiliated Children's Hospital. Patients were divided into the treated group (with night-time non-invasive ventilation) and the control group (without ventilator). Sleep breathing pressure titration was suggested to the patients who were prepared to receive non-invasive ventilation. All cases were followed up for one year. Parameters'changes in polysomnography were assessed (paired -test) in titration patients. Frequency of respiratory tract infection during the next year in the patients with and without ventilation was collected and compared (Mann-Whitney -test). Seventeen cases were recruited. The average age was (5.1±2.9) years, 10 cases were boys and 7 cases were girls. In the titration group (8 patients), after non-invasive ventilation, the average apnea hypopnea index was (3.8±2.5) times/h (4.086, 0.005), hypopnea index was (2.4±1.2) times/h (2.779, 0.027), average oxygen saturation during total sleep time was 0.966±0.007 (5.292, 0.001), and the minimum oxygen saturation was 0.906±0.023 (-3.938, 0.006). All the above parameters were significantly improved after treatment. Than before, which was (16.6±9.7) times/h, (7.2±4.7) times/h, 0.946±0.015, 0.786±0.092 respectively. Ventilator mode for the 9 children with long time non-invasive ventilation at home was Bi-level positive airway pressure S/T. The positive airway pressure was set at 8-14 cmH(2)O (1 cmH(2)O=0.098 kPa) in inspiratory phase and 4-6 cmH(2)O in expiratory phase. In the treated group (9 patients), the average frequency of upper respiratory tract infection was 1.0 (0, 3.0) times/year (-2.245, 0.023), the lower respiratory tract infection was 0 (0, 0) times/year (-3.189, 0.001), hospitalization was 0 (0, 0) times/year (-3.420, 0.01), and admission to intensive care unit was 0 (0, 0) times/year (-3.353, 0.029). All the above indexes were significantly decreased compared with the control group (8 patients), which was 3.0 (2.3, 7.0) times/year, 2.0 (1.3, 4.5) times/year, 1.0 (1.0, 4.3) times/year, 0.5 (0, 1.0) times/year respectively. Non-invasive ventilation is efficient to SMA children with sleep-disordered breathing, and also can reduce the incidence of respiratory tract infections for children with SMA.