title: Noninvasive ventilation in children with spinal muscular atrophy types 1 and 2.
abstract: OBJECTIVE
  Our aim was to assess the efficacy of noninvasive ventilation (NIV) for the treatment of thoracoabdominal asynchrony during sleep in children with spinal muscular atrophy (SMA) types 1 and 2.
  
  
  METHODS
  Nine subjects underwent assessment for sleep apnea/hypopnea index (AHI), mean oxyhemoglobin saturation (SpO2), oxygen desaturation index, transcutaneous carbon dioxide tension (tcpCO2), and mean phase angle during sleep as a measure of thoracoabdominal coordination. A second sleep study was performed with use of NIV.
  
  
  RESULTS
  The nine patients (7 mos of age, range 2-33) had a baseline AHI of 2.1 events per hour (range 0.5-55.8), oxygen desaturation index of 3.7 events per hour (range 1.6-46.1), mean tcpCO2 of 46 mm Hg (range 37-60), and phase angle of 127 degrees (range 72.7-151.7). Comparing baseline and NIV sleep studies, we found significant improvement in oxygen desaturation index (P < 0.010), mean tcpCO2 (P < 0.001), and phase angle (P < 0.001). For five patients, phase-angle improvement became significant when using high-span bilevel positive airway pressure (PAP).
  
  
  CONCLUSIONS
  NIV improved sleep breathing parameters and thoracoabdominal coordination during sleep in SMA types 1 and 2. Phase-angle improvement correlated with bilevel PAP pressures. Phase angle may be useful for the evaluation and monitoring of therapeutic interventions such as NIV.
authors.affiliation: Respiratory Unit, Bambino Gesù Children Hospital, Research Institute, Rome, Italy.
authors.forename: Albino
authors.initials: A
authors.lastname: Petrone
authors.forename: Martino
authors.initials: M
authors.lastname: Pavone
authors.forename: Maria B Chiarini
authors.initials: MB
authors.lastname: Testa
authors.forename: Francesca
authors.initials: F
authors.lastname: Petreschi
authors.forename: Enrico
authors.initials: E
authors.lastname: Bertini
authors.forename: Renato
authors.initials: R
authors.lastname: Cutrera
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010108:Oxyhemoglobins / Q000032:analysis; D017286:Polysomnography; D011175:Positive-Pressure Respiration / Q000379:methods*; D012890:Sleep; D012891:Sleep Apnea Syndromes / Q000628:therapy; D014897:Spinal Muscular Atrophies of Childhood / Q000503:physiopathology / Q000534:rehabilitation*
chemical_list: D010108:Oxyhemoglobins