Background: Several studies found a high prevalence of sleep disorders in PWS patients. It is assumed that Prader-Willi Syndrome (PWS) patients are at a high risk of sleep disordered breathing, such as obstructive sleep apnea (OSA), because of their childhood obesity, associated with muscle hypotonia, leading to upper airway collapse.
Objective and hypotheses: We studied a group of PWS children (genetically confirmed, non-GH-treated) who performed complete sleep studies and compared to a group of non-PWS obese children (OC) matched for sex, age and BMI.
Method: All patients underwent overnight polysomnography. Data are reported as medians (interquartile range), ManneWhitney test was used for between-group.
Results: We did not find a statistically significant difference in the prevalence of OSA between obese PWS and OC (58.8% vs 50%, P=0.64) (Table 1).
|PWS (n=23)||OC (n=8)||P|
|Age (years)||9.9 [6.9÷13.9]||9.15 [6.85÷13.0]||P=0.98|
|BMI SDS||3.16 [2.2÷ 4.53]||2.9 [2.79÷ 3.4]||P=0.7|
|Tonsillar hypertrophy||30.4% (n=7)||25% (n=2)||P=0.73|
|Adenoidal hypertrophy||52.1% (n=12)||62.5% (n=5)||P=0.36|
|REM latency (min)||81.75 [63.0÷143.25]||160.5 [125.75÷205.50]||P=0.01|
|Apnea-hypopnea index||3.5 [0.6÷9.2]||1.7 [0.3÷8.1]||P=0.61|
Conclusion: There is a high prevalence of OSA in PWS children, but its not higher than in simply obese pediatric patients. However, PWS patients were found to have distinctive polysomnographic features the significantly shortened REM latency vs non-PWS children, suggesting possible dysregulation of sleep-wake cycle. Further research is necessary to clarify the mechanism of sleep related disorders in PWS.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology