ESPE Abstracts (2016) 86 P-P1-476

aEndocrine Research Centre, Moscow, Russia; bI.M. Sechenov First Moscow State Medical University, Moscow, Russia


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), Manne–Whitney 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).

Table 1. Polysomnography characteristics of PWS and non-PWS obese children (for abstract P1-P476)
PWS (n=23)OC (n=8)P
Age (years)9.9 [6.9÷13.9]9.15 [6.85÷13.0]P=0.98
Boys/girls14/93/5P=0.4
BMI SDS3.16 [2.2÷ 4.53]2.9 [2.79÷ 3.4]P=0.7
Tonsillar hypertrophy30.4% (n=7)25% (n=2)P=0.73
Adenoidal hypertrophy52.1% (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 index3.5 [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 it’s 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.

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