title: Air stacking: effects on pulmonary function in patients with spinal muscular atrophy and in patients with congenital muscular dystrophy.
abstract: OBJECTIVE
  Respiratory complications are the main causes of morbidity and mortality in patients with neuromuscular disease (NMD). The objectives of this study were to determine the effects that routine daily home air-stacking maneuvers have on pulmonary function in patients with spinal muscular atrophy (SMA) and in patients with congenital muscular dystrophy (CMD), as well as to identify associations between spinal deformities and the effects of the maneuvers.
  
  
  METHODS
  Eighteen NMD patients (ten with CMD and eight with SMA) were submitted to routine daily air-stacking maneuvers at home with manual resuscitators for four to six months, undergoing pulmonary function tests before and after that period. The pulmonary function tests included measurements of FVC; PEF; maximum insufflation capacity (MIC); and assisted and unassisted peak cough flow (APCF and UPCF, respectively) with insufflations.
  
  
  RESULTS
  After the use of home air-stacking maneuvers, there were improvements in the APCF and UPCF. In the patients without scoliosis, there was also a significant increase in FVC. When comparing patients with and without scoliosis, the increases in APCF and UPCF were more pronounced in those without scoliosis.
  
  
  CONCLUSIONS
  Routine daily air-stacking maneuvers with a manual resuscitator appear to increase UPCF and APCF in patients with NMD, especially in those without scoliosis.
authors.affiliation: Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Tanyse Bahia Carvalho
authors.initials: TB
authors.lastname: Marques
authors.affiliation: Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Juliana de Carvalho
authors.initials: Jde C
authors.lastname: Neves
authors.affiliation: Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Leslie Andrews
authors.initials: LA
authors.lastname: Portes
authors.affiliation: Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: João Marcos
authors.initials: JM
authors.lastname: Salge
authors.affiliation: Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Edmar
authors.initials: E
authors.lastname: Zanoteli
authors.affiliation: Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
authors.forename: Umbertina Conti
authors.initials: UC
authors.lastname: Reed
mesh_terms: D000293:Adolescent; D001945:Breathing Exercises / Q000379:methods*; D002648:Child; D003371:Cough / Q000209:etiology / Q000534:rehabilitation; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008168:Lung / Q000503:physiopathology; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000150:complications* / Q000503:physiopathology; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000503:physiopathology; D010366:Peak Expiratory Flow Rate; D012129:Respiratory Function Tests; D055815:Young Adult