BSPED2014 Main Symposia Endocrine Nurse Session (5 abstracts)
University of Southampton, Southampton, UK.
PraderWilli syndrome is a complex genetic disorder. The characteristics of hypotonia put children at increased risk of respiratory problems during childhood. These range from an increased propensity to respiratory infection to problems during sleep of central and obstructive sleep apnoea. Obesity a common finding in children with PraderWilli syndrome has the tendency to exacerbate these increased risks. There are often concerns around the use of GH in children with PraderWilli syndrome and the effect that this has on the risks for obstructive sleep apnoea.
This presentation aims to provide an overview of respiratory problems including the patterns of sleep disordered breathing in children with PraderWilli syndrome and the treatments available. The rationale behind screening children for sleep disordered breathing will be discussed as well as the integration of sleep studies into current consensus guidelines. Respiratory management of the severely hypotonic PraderWilli infant will be presented.