hrp0089fc13.6 | Pituitary, Neuroendocrinology and Puberty 2 | ESPE2018

Teamwork Saves Lives: How Pediatric Multidisciplinary care can Prevent ‘Unexplained Deaths’ in Adults with Prader-Willi Syndrome

Davidse Kirsten , Pellikaan Karlijn , Rosenberg Anna , Baan Janneke , de Graaff Laura

Introduction: Prader-Willi Syndrome (PWS) is a complex hypothalamic disorder, causing hypotonia, intellectual disability (ID), pituitary hormone deficiencies and hyperphagia. Up to 4% of young patients with PWS die unexpectedly, every year. The mean age of reported deaths in PWS is 29.5 years; 20% of deaths even occur below age 18 years. Mortality data show that more than 50% of deaths are of cardio-pulmonary origin. Morbid obesity, diabetes and hypertension are strong risk fa...