hrp0089p2-p247 | Growth & Syndromes P2 | ESPE2018

GH Unmasked Laryngomalacia and worsened Obstructive Sleep Apnea in Infants with Prader-Willi Syndrome

Salehi Parisa , Wrede Joanna E , Johnson Kaalan E , Chen Maida L

Background: Prader-Willi Syndrome (PWS), due to loss of paternal gene expression on chromosome 15q11.2-13, is characterized by hypotonia, hypothalamic-pituitary dysregulation, abnormal respiratory drive, and hyperphagia. GH, often started in infancy, improves tone, body composition, and height. Concerns about sudden death in children with PWS started on GH, hypothesized secondary to worsening obstructive sleep apnea (OSA) from adenotonsillar hypertrophy, resulted in guidelines...