title: Clinically important age-related differences in sleep related disordered breathing in infants and children with Prader-Willi Syndrome.
abstract: BACKGROUND
  Sleep related disordered breathing (SDB) in pediatric Prader-Willi Syndrome is gaining increased attention due to the possible association of growth hormone therapy, SDB and sudden death. However data on the patterns of SDB and their management, particularly in infants in this population, is lacking.
  
  
  OBJECTIVE
  The aim of this study was to 1) describe patterns of SDB in growth hormone naive infants with PWS and the management of these disorders in our institution 2) Compare the patterns of sleep disorders between infants and children with PWS.
  
  
  METHODS
  Polysomnograms of infants and children (0-18 years of age) with Prader-Willi Syndrome were reviewed. Age, sex, anthropometrics, sleep architecture, obstructive and central apnea indices and oxygen saturations were recorded. Data of infants with central sleep apnea treated with oxygen were analyzed to evaluate the efficacy of this treatment. The main outcome measures were obstructive and central apnea indices on a polysomnogram.
  
  
  RESULTS
  Data of 44 patients, 23 under 2 years of age and 21 older children were included. Infants when compared with older children were more likely to experience central sleep apnea (43% vs. 5%; p = 0.003). In older children obstructive was significantly more prevalent than central sleep apnea. Supplemental oxygen was used to treat 9/23 infants with central sleep apnea. Oxygen therapy resulted in a significant decrease in the median central apnea index from 14 (5,68) to 1 (0,6; p = 0.008) events/hour and an improvement in the oxygen saturation nadir from 70% (52, 92) to 81% (64, 95; p = 0.080).
  
  
  CONCLUSIONS
  Central sleep apnea with associated oxygen desaturations is more prevalent in infants compared with older children with Prader-Willi Syndrome. Supplemental oxygen was efficacious in treating central sleep apnea in infants. Routine sleep surveillance for all children with Prader-Willi Syndrome and treatment with oxygen for central sleep apnea should be considered.
authors: Division of Endocrinology, The Hospital for Sick Children, Toronto, Ontario, Canada; The University of Toronto, Toronto, Ontario, Canada.
authors: Michal
authors: M
authors: Cohen
authors: Division of Endocrinology, The Hospital for Sick Children, Toronto, Ontario, Canada; The University of Toronto, Toronto, Ontario, Canada.
authors: Jill
authors: J
authors: Hamilton
authors: The University of Toronto, Toronto, Ontario, Canada; Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Indra
authors: I
authors: Narang
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010102:Oxygen Inhalation Therapy; D017286:Polysomnography; D011218:Prader-Willi Syndrome / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D020182:Sleep Apnea, Central / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D020181:Sleep Apnea, Obstructive / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D016896:Treatment Outcome