hrp0086fc6.3 | Syndromes: Mechanisms and Management | ESPE2016

Oxytocin Improves Social and Food-Related Behavior in Young Children with Prader-Willi Syndrome: A Randomized, Double-Blind, Controlled Crossover Trial

Kuppens Renske , Donze Stephany , Hokken-Koelega Anita

Background: Prader-Willi syndrome (PWS) is known for hyperphagia with impaired satiety and a specific behavioral phenotype with stubbornness, manipulative and controlling behavior and obsessive-compulsive features. PWS is associated with hypothalamic and oxytocinergic dysfunction. In humans without PWS, intranasal oxytocin administration had positive effects on social behavior and weight balance.Objective and hypotheses: To evaluate the effects of intran...

hrp0086p2-p700 | Endocrinology and Multisystemic Diseases P2 | ESPE2016

Effects of 2 Years of Growth Hormone Treatment on Glucose Tolerance in Young Adults with Prader-Willi Syndrome

Donze Stephany , Kuppens Renske , Bakker Nienke , Hokken-Koelega Anita

Background: Growth hormone treatment (GH) in children with PWS results in an improvement in height velocity, body composition and mental and motor development. Discontinuation of GH after attainment of adult height (AH) leads to a decrease in lean body mass and an increase in body fat percentage, which results in an increased risk of impaired glucose tolerance (IGT) and diabetes mellitus type 2 (DM2). Studies in adults with PWS suggest positive effects of GH, but GH is known t...

hrp0089rfc4.6 | GH & IGFs | ESPE2018

Effect of 2 Years of Growth Hormone Treatment on Glucose Tolerance in Adults with Prader-Willi Syndrome

Damen Layla , Donze Stephany , Kuppens Renske , Bakker Nienke , Hokken-Koelega Anita

Background: In children with Prader-Willi syndrome (PWS), the benefits of growth hormone (GH) treatment are well established. GH has substantially changed the phenotype of children with PWS. Discontinuation of GH after adult height (AH) attainment leads to a decrease in lean body mass and an increase in body fat percentage. Due to their abnormal body composition, adults with PWS are predisposed to develop impaired glucose tolerance (IGT) and diabetes mellitus type 2 (T2DM). Re...

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...

hrp0092fc12.3 | Growth and Syndromes (to include Turner Syndrome) | ESPE2019

Growth Hormone Treatment in Adults with Prader-Willi Syndrome has Sustained Positive Effects on Body Composition

Damen Layla , Donze Stephany , Kuppens Renske , Bakker Nienke , de Graaff Laura , Alfen-van der Velden Janiëlle van , Stikkelbroeck Nike , Hokken-Koelega Anita

Context: In children with Prader-Willi syndrome (PWS), the benefits of growth hormone (GH) treatment are well established. Currently, when young adults with PWS have attained adult height (AH), they have to stop GH treatment. Several one year studies have shown that GH treatment is also beneficial for adults with PWS, improving body composition. However, little is known about the longer-term effects.Objective: To investi...

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...