title: Central sleep-disordered breathing and the effects of oxygen therapy in infants with Prader-Willi syndrome.
abstract: OBJECTIVE
  To describe breathing patterns in infants with Prader-Willi Syndrome (PWS), as well as the effects of supplemental oxygen (O2) on breathing patterns. Children with PWS commonly have sleep-disordered breathing, including hypersomnolence and obstructive sleep apnoea, as well as central sleep breathing abnormalities that are present from infancy.
  
  
  METHODS
  Retrospective cohort study.
  
  
  METHODS
  Infants with a diagnosis of PWS.
  
  
  METHODS
  Tertiary children's hospital.
  
  
  METHODS
  Infants with PWS underwent full polysomnography, and in those with frequent desaturations associated with central events, supplemental O2 during sleep was started and followed with regular split-night studies (periods in both air and O2).
  
  
  RESULTS
  Thirty split-night studies on 10 infants (8 female) aged 0.06-1.79 (median 0.68, IQR 0.45, 1.07) years were undertaken. At baseline (ie, air), children with PWS had a median (IQR) central apnoea index (CAI) of 4.7 (1.9, 10.6) per hour, with accompanying falls in oxygen saturation (SpO2). O2 therapy led to statistically significant reductions in CAI to 2.5/hour (p=0.002), as well as a reduced central event index (CEI) and improved SpO2. No change in the number of obstructive events was noted. Central events were more prevalent in rapid-eye movement/active sleep.
  
  
  CONCLUSIONS
  It is concluded that infants with PWS may have central sleep-disordered breathing, which, in some children, may cause frequent desaturations. Improvements in CAI and CEI as well as oxygenation were noted with O2 therapy. Longitudinal work with this patient group would help to establish the timing of onset of obstructive symptoms.
authors.affiliation: Department of Paediatric Respiratory and Sleep Medicine, Mater Children's Hospital, South Brisbane, Queensland, Australia.
authors.forename: D S
authors.initials: DS
authors.lastname: Urquhart
authors.forename: T
authors.initials: T
authors.lastname: Gulliver
authors.forename: G
authors.initials: G
authors.lastname: Williams
authors.forename: M A
authors.initials: MA
authors.lastname: Harris
authors.forename: O
authors.initials: O
authors.lastname: Nyunt
authors.forename: S
authors.initials: S
authors.lastname: Suresh
mesh_terms: D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010102:Oxygen Inhalation Therapy / Q000379:methods*; D017286:Polysomnography; D011218:Prader-Willi Syndrome / Q000150:complications / Q000503:physiopathology* / Q000628:therapy; D012189:Retrospective Studies; D012890:Sleep; D020182:Sleep Apnea, Central / Q000150:complications / Q000503:physiopathology* / Q000628:therapy
keywords: Respiratory; Sleep