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Long term noninvasive ventilation and continuous positive airway pressure in children with neuromuscular diseases in France

Published:September 24, 2022DOI:https://doi.org/10.1016/j.nmd.2022.09.008

      Highlights

      • In 2019, 387 children were treated at home with noninvasive ventilation in France.
      • Most children had spinal muscular atrophy or congenital myopathy/dystrophy.
      • Ninety-four percent of children were treated with noninvasive ventilation.
      • Treatment was initiated electively for 85% of children.
      • Mean objective noninvasive ventilation use was 8.0 ± 3.1 h/night.

      Abstract

      The aim of the study was to describe the characteristics of children with neuromuscular diseases treated with long term noninvasive ventilation or continuous positive airway pressure in France. On June 1st 2019, 387 patients (63% boys, mean age 11.2 ± 5.5 years) were treated with long term noninvasive ventilation/continuous positive airway pressure. Thirty three percent of patients had spinal muscular atrophy, 30% congenital myopathy/dystrophy, 20% Duchenne muscular dystrophy, 7% Steinert myotonic dystrophy, and 9% other neuromuscular diseases. Ninety-four percent of patients were treated with long term noninvasive ventilation and 6% with continuous positive airway pressure. Treatment was initiated electively for 85% of patients, mainly on an abnormal overnight gas exchange recording (38% of patients). Noninvasive ventilation/continuous positive airway pressure was initiated during a respiratory exacerbation in 15% of patients. Mean duration of noninvasive ventilation/continuous positive airway pressure was 3.3 ± 3.1 years. Mean objective long term noninvasive ventilation/continuous positive airway pressure use was 8.0 ± 3.1 h/24. Spinal muscular atrophy, congenital myopathy/dystrophy, and Duchenne muscular dystrophy represented 83% of children with neuromuscular diseases treated with long term noninvasive ventilation in France. Screening for nocturnal hypoventilation was satisfactory as noninvasive ventilation /continuous positive airway pressure was predominantly initiated electively.

      Keywords

      Abbreviations:

      AHI (apnoea-hypopnea index), BUR (back up rate), CPAP (continuous positive airway pressure), DMD (Duchenne muscular dystrophy), EPAP (expiratory positive airway pressure), IPAP (inspiratory positive airway pressure), IPPB (intermittent positive pressure breathing), IPV (intrapulmonary percussive ventilation), MI-E (mechanical in-exsufflator), NIV (long term noninvasive ventilation), NMD (neuromuscular diseases), PG (polygraphy), PSG (polysomnography), PtcCO2 (transcutaneous carbon dioxide pressure), SMA (spinal muscular atrophy), SpO2 (pulse oximetry)
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