hrp0084p2-350 | Fat | ESPE2015

The Switch in Eating Behaviour in Infants with Prader–Willi Syndrome is Associated with an Increase in the Acylated:Unacylated Ghrelin Ratio: Results of a Longitudinal Study

Kuppens Renske , van der Lely Aart Jan , Delhanty Patric , Huisman Martin , Hokken-Koelega Anita

Background: Prader–Willi syndrome (PWS) is characterized by a switch from failure to thrive to excessive weight gain and hyperphagia with impaired satiety in early childhood. An elevated, more unfavorable ratio between acylated:unacylated ghrelin (AG:UAG) may be involved in the underlying mechanisms of this switch.Objective and hypotheses: To assess the evolution of the appetite regulating hormones AG, UAG, and the AG:UAG ratio in infants with PWS a...

hrp0098rfc5.1 | Growth and Syndromes | ESPE2024

About caring for patients and understanding their pathways: 10 years of ‘the rotterdam centre for adults with rare genetic syndromes’

Goos Jacqueline , Davidse Kirsten , Baan Janneke , Pellikaan Karlijn , Rosenberg Anna , Carrasco García Álvaro , Delhanty Patric , Visser Jenny , Graaff Laura

Introduction: Patients with rare genetic syndromes (RGS) often have a combination of hormone deficiencies, organ problems and neurodevelopmental disorders. Due to excellent paediatric care, a growing number of patients is reaching adult age. Transition of these patients to adult health care system has proved to be challenging. Due to the rarity and complexity of their syndromes, patients with RGS often suffer from medical complications due to needless diagnost...

hrp0082p1-d3-128 | Fat Metabolism & Obesity (2) | ESPE2014

Acylated and Unacylated Ghrelin Levels in Children and Young Adults with Prader–Willi Syndrome

Kuppens Renske , Diene Gwenaelle , Bakker Nienke , Molinas Catherine , Faye S , Nicolino Marc , Bernoux Delphine , Delhanty Patric , Jan van der Lelij Aart , Allas Soraya , Julien Michiel , Delale Thomas , Tauber Maithe , Hokken-Koelega Anita

Background: Prader–Willi syndrome (PWS) is characterized by a switch in early childhood from failure to thrive to excessive weight gain and hyperphagia with impaired satiety. The underlying mechanism for this switch may involve hyperghrelinemia, but only poor data exists regarding levels of acylated ghrelin (AG), unacylated ghrelin (UAG), and the AG/UAG ratio in PWS.Objective and hypotheses: To investigate plasma levels of AG and UAG in PWS, compare...