title: Longitudinal evaluation of sleep disordered breathing in infants with Prader-Willi syndrome.
abstract: To evaluate the course of sleep disordered breathing (SDB) in infants with Prader-Willi syndrome (PWS).
  
  
  
  Retrospective longitudinal observational study.
  
  
  
  Sleep laboratory at The Hospital for Sick Children, Toronto, Canada.
  
  
  
  Infants with PWS.
  
  
  
  The natural history of SDB in infants with PWS within 2 years from baseline assessment.
  
  
  
  We identified 28 (12 male) infants with PWS who had a baseline polysomnography (PSG) at a median age (interquartile (IQR)) of 0.9 (0.5, 1.1) years. The median central apnoea index (CAI) at baseline was 6.6 events/hour (IQR 2.6, 12.1). Of these, 15/28 (53%) infants with PWS were diagnosed with significant central sleep apnoea (CSA) (CAI≥5 events/hour). Median age (IQR) at follow-up PSG was 2.1 (1.5, 2.6) years. The median CAI improved from 6.6 to 2.3 events/hour (p<0.0001). Only four infants with PWS had persistent CSA at the time of the follow-up PSG. Furthermore, three infants with PWS were diagnosed with mild-to-moderate obstructive sleep apnoea (OSA) that has improved at follow-up studies whereas two patients with PWS with no evidence of OSA at baseline were diagnosed with severe OSA on the follow-up PSG requiring adenotonsillectomy. The overall median obstructive apnoea-hypopnoea index was similar between baseline and follow-up studies (0.6 and 0.8, respectively, p=0.91).
  
  
  
  CSA is prevalent in infants with PWS but usually improves with age. However, these patients continue to require ongoing PSG surveillance because some infants will have persistent CSA and others are at risk of developing OSA.
authors.affiliation: University of Toronto, Toronto, Ontario, Canada.|Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Abdullah
authors.initials: A
authors.lastname: Khayat
authors.affiliation: University of Toronto, Toronto, Ontario, Canada.|Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Indra
authors.initials: I
authors.lastname: Narang
authors.affiliation: University of Toronto, Toronto, Ontario, Canada.|Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Saadoun
authors.initials: S
authors.lastname: Bin-Hasan
authors.affiliation: University of Toronto, Toronto, Ontario, Canada.|Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Reshma
authors.initials: R
authors.lastname: Amin
authors.affiliation: University of Toronto, Toronto, Ontario, Canada.|Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Suhail
authors.initials: S
authors.lastname: Al-Saleh
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D017286:Polysomnography; D011218:Prader-Willi Syndrome / Q000150:complications*; D011446:Prospective Studies; D012891:Sleep Apnea Syndromes / Q000209:etiology*; D020182:Sleep Apnea, Central / Q000209:etiology*
keywords: Central sleep apnea; Obstructive sleep apnea; Prader-Willi Syndrome.; Sleep disordered breathing; infants