Abstract
BackgroundSleep 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.ObjectiveThe 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 and DesignPolysomnograms 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.ResultsData 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).ConclusionsCentral 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.
Highlights
Prader-Willi syndrome (PWS) is a complex genetic disorder that occurs in 1/10,000 to 1/25,000 live births
Infants when compared with older children were more likely to experience central sleep apnea (43% vs. 5%; p = 0.003)
Central sleep apnea with associated oxygen desaturations is more prevalent in infants compared with older children with Prader-Willi Syndrome
Summary
Prader-Willi syndrome (PWS) is a complex genetic disorder that occurs in 1/10,000 to 1/25,000 live births. Sleep related disordered breathing (SDB), a group of respiratory disorders specific to or exacerbated by sleep, have been reported in PWS [2]. Sleep disorders in PWS, OSA in the obese older children have gained increasing attention due to the possible association of growth hormone (GH) therapy, abnormal sleep related breathing and sudden death [6,7]. To date few studies have focused on the prevalence, severity, nature and management of sleep disorders in infants with PWS and none have compared these with older children [8,9,10]. 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. Data on the patterns of SDB and their management, in infants in this population, is lacking
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