0770 Prader-Willi Syndrome: The Impact of Growth Hormone on the Sleep Phenotypes

Abstract

Introduction: Children with Prader-Willi Syndrome (PWS) are at an increased risk for sleep problems. Current treatment of PWS includes growth hormone, which may modify the sleep and clinical phenotypes. We retrospectively reviewed polysomnograms (PSGs) of children with PWS on or off growth hormone (GH) to typically developing obese (TDO) children to determine how sleep phenotypes vary. Methods: After IRB approval, we compared baseline PSGs and clinical data from children with PWS on GH (PWS-G), off GH (PWS-O) and TDO (BMI > 30 kg/m2). Mann-Whitney U test (2-tailed) was used for statistical analysis. Results: There were 15 PWS-O (mean age 4.95 ±6.33 years, 10 males), 9 PWS-G (mean age 9.08 ±4.12 years, 6 males), and 25 TDO (mean age 12.00 ±3.51 years, 10 males). PWS-O compared to PWS-G had higher BMIs (25.8 ±4.9 versus 24.2 ±2.9). PWS-O demonstrated more severe obstructive sleep apnea (OSA) than PWS-G (mean AHI 13.1 ±13.3 versus 9.4 ±5.5). More of the PWS-O reported insomnia (28.6% versus 16.7%) and Pediatric Daytime Sleepiness Scale (PDSS) >15 (14.3% versus 0%) than PWS-G. TDO were more obese (BMI median 37.75 ±3.9 m/kg2) compared to PWS-G and PWS-O. TDO had milder OSA (mean AHI 7.7 ±7.64). The PWS-G and PWS-O groups had lower oxygen nadirs (mean 84.3% and 82.9%, respectively) compared to TDO (mean 91.6%). None of the subjects had central sleep apnea. TDO had prolonged REM latencies (median 169.5 ±38.0 minutes) and decreased REM amounts (median 16.0 ±3.1%, p= 0.051, 0.067) while those with PWS-O and PWS-G had normal REM latencies (median 77.3 ±38.3 minutes, 86.5 ±31.9 minutes) and amounts (median 21.8 ±3.0%, 21.0 ±2.1%), respectively. TDO group reported greater sleepiness (PDSS > 15 (44%), p= 0.00058, 0.00214) and insomnia (68%, p= 0.012, 0.013) than both PWS groups. Conclusion: GH treatment may modify the sleep phenotype in PWS. PWS-G demonstrated a lower AHI and less sleepiness than PWS-O. In comparison to TDO children, PWS-G had similar severity of OSA and REM characteristics but less sleepiness and insomnia.