0533 Age-related changes in sleep disordered breathing in pediatric Prader Willi Syndrome patients

Abstract

Abstract Introduction Prader Willi Syndrome (PWS) is a rare genetic disorder characterized by infantile hypotonia, hyperphagia leading to early childhood obesity, and multiple sleep abnormalities including increased risk of obstructive sleep apnea, central sleep apnea, and excessive daytime sleepiness. PWS patients have been reported to have a shorter lifespan with respiratory related causes as a common cause of death. Limited data exists on PSGs in pediatric PWS patients. Our aim was to compare respiratory parameters in different age groups. Methods We conducted a retrospective chart review of pediatric patients with a genetically confirmed diagnosis of Prader Willi Syndrome from 2007 to 2015. In lab polysomnograms were scored using the AASM criteria for pediatric sleep studies. As central apneas are more commonly seen in infancy with PWS, we categorized patients into < 1 years old and 1-19 years old for comparison. Results Fifty-one PSGs were reviewed of which 31 (60.7%) were males and 20 (39.3%) were females. Forty-one PSGs were initial studies and 11 were repeat studies. Age at time of sleep study ranged from 11 days-19 years old. 8 (15%) subjects were < 1 years old and 44 (84%) were 1-19 years old. For patients < 1 year old, mean obstructive hypopnea index (OAHI) was 5.65±3.7, central index (CI)2.2±2.5, mean SpO2(%) 98±.4, SpO2 nadir 86±5.8, and % of time with SpO2 < 90% .16±.314. For patients 1-19 years old, mean (OAHI) was 8.7±13.4, (CI) 0.8±1.25, mean SpO2 96±3.0, SpO2 nadir 81±.10, and % of time with SpO2 < 90% .03±.10. The OAHI trended up in the 1-19 year old group but results were not significant, CI decreased in the 1-19 year old group 2.2 vs 0.8 p < .04. Conclusion Our study demonstrated similar results to prior studies in pediatric PWS with an increase in OAHI and decrease in CI with age. SPO2 nadir decreased in the 1-19 year old group but these results were not significant. Ongoing research at our center is looking at possible contributing factors including BMI percentile, GH therapy, and adenotonsillectomy to better understand our findings. Support (If Any)