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...