hrp0095t10 | Section | ESPE2022

Aromatase Inhibitor (anastrazole) vs placebo delays bone age maturation in prepubertal children with Silver Russell or Prader-Willi Syndrome and pathological adrenarche

Dufourg Marie-Noëlle , Diene Gwenaelle , Cachanado Marine , Vu-Hong Thuy-Ai , Berard Laurence , Rousseau Alexendra , Soussi Nora , Pinto Graziella , Rouleau Stéphanie , Bernoux Delphine , Chalard François , Ducou Le Pointe Hubert , Lamaziere Antonin , Tauber Maite , Netchine Irène

Background: Silver Russell syndrome (SRS) is a rare imprinting disorder with prenatal and postnatal growth retardation and feeding difficulties. The main molecular causes are loss of methylation of the 11p15.5 imprinting control region (H19/IGF2) and maternal uniparental disomy of chromosome 7. Prader-Willi syndrome (PWS) is a complex neurodevelopmental imprinting disorder with neonatal muscular hypotonia, failure to thrive to insatiable appetite, sho...

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...