title: High unacylated ghrelin levels support the concept of anorexia in infants with prader-willi syndrome.
abstract: Prader-Willi syndrome (PWS) is a rare genetic neurodevelopmental disorder with different nutritional phases from suckling deficit with failure to thrive to early onset of obesity. Hyperghrelinemia has been described in PWS long before the development of obesity. Ghrelin is found in both acylated (AG) and unacylated (UAG) forms in the circulation. In contrast to AG, UAG has been shown to inhibit food intake and to be elevated in anorexia nervosa. The present project is aiming to determine the underlying mechanisms driving the different nutritional phases in PWS.
  
  
  
  Measurement of at least 4 h-fasting plasma acylated and unacylated ghrelin in 37 infants with a genetic diagnosis of PWS aged from 1 month to 4 years and in 100 age-matched controls without endocrine disorder recruited prior to minor surgery. One blood sampling was analysed for each patient/control and clinical data were recorded. Eleven PWS infants underwent repetitive blood samples at 3 or 6-month intervals during routine visits.
  
  
  
  In infants with PWS, AG is not elevated (p = 0.45), UAG is significantly higher (p = 0.0044; confidence interval 1.06;1.33) resulting in a low AG/UAG ratio (p = 0.0056; confidence interval 0.76;0.95) compared to controls.
  
  
  
  Unlike children and adults with PWS that have high AG and AG/UAG ratio, infants with PWS have elevated UAG that supports the concept of anorexia in the early phases of the disease. The change in AG/UAG ratio possibly drives the switch from failure to thrive to obesity.
  
  
  
  NCT02529085 .
authors.affiliation: Unité d'Endocrinologie pédiatrique, Cliniques Universitaires Saint-Luc, Université catholique de Louvain, Avenue Hippocrate 10/1300, B-1200, Brussels, Belgium. veronique.beauloye@uclouvain.be.
authors.forename: Veronique
authors.initials: V
authors.lastname: Beauloye
authors.affiliation: Unité d'Endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale. Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France.
authors.forename: Gwenaelle
authors.initials: G
authors.lastname: Diene
authors.affiliation: Dutch Growth Research Foundation, Rotterdam, The Netherlands.|Department of Pediatrics, Subdivision of Endocrinology, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Renske
authors.initials: R
authors.lastname: Kuppens
authors.affiliation: IREC, Université Catholique de Louvain, Brussels, Belgium.
authors.forename: Francis
authors.initials: F
authors.lastname: Zech
authors.affiliation: Unité d'Endocrinologie pédiatrique, Cliniques Universitaires Saint-Luc, Université catholique de Louvain, Avenue Hippocrate 10/1300, B-1200, Brussels, Belgium.
authors.forename: Coralie
authors.initials: C
authors.lastname: Winandy
authors.affiliation: Unité d'Endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale. Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France.|Axe Pédiatrique du CIC 9302/INSERM. Hôpital des Enfants, Toulouse, France.|INSERM U1043, Centre de Physiopathologie de Toulouse Purpan, Université Paul Sabatier, Toulouse, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Molinas
authors.affiliation: Unité d'Endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale. Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France.|Axe Pédiatrique du CIC 9302/INSERM. Hôpital des Enfants, Toulouse, France.
authors.forename: Sandy
authors.initials: S
authors.lastname: Faye
authors.affiliation: Unité d'Endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale. Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France.|Axe Pédiatrique du CIC 9302/INSERM. Hôpital des Enfants, Toulouse, France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Kieffer
authors.affiliation: Unité d'Endocrinologie Pédiatrique, CHU Dinant Godinne, Yvoir, Belgium.
authors.forename: Dominique
authors.initials: D
authors.lastname: Beckers
authors.affiliation: Karolinska University Hospital, Karolinska Institutet, Solna, Sweden.
authors.forename: Ricard
authors.initials: R
authors.lastname: Nergårdh
authors.affiliation: Department of Endocrinology, University Children's Hospital, Essen, Germany.
authors.forename: Berthold
authors.initials: B
authors.lastname: Hauffa
authors.affiliation: BESPEED (Belgian Society for Pediatric Endocrinology and Diabetology), Brussels, Belgium.
authors.forename: Christine
authors.initials: C
authors.lastname: Derycke
authors.affiliation: Department of Internal Medicine, Erasmus University Medical Center, Rotterdam, The Netherlands.
authors.forename: Patrick
authors.initials: P
authors.lastname: Delhanty
authors.affiliation: Dutch Growth Research Foundation, Rotterdam, The Netherlands.|Department of Pediatrics, Subdivision of Endocrinology, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: Anita
authors.initials: A
authors.lastname: Hokken-Koelega
authors.affiliation: Unité d'Endocrinologie, Obésité, Maladies Osseuses, Génétique et Gynécologie Médicale. Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France.|Axe Pédiatrique du CIC 9302/INSERM. Hôpital des Enfants, Toulouse, France.|INSERM U1043, Centre de Physiopathologie de Toulouse Purpan, Université Paul Sabatier, Toulouse, France.|Unité d'Endocrinologie, Hôpital des Enfants, 330, Avenue de Grande Bretagne, TSA 70034, 31059, Toulouse Cedex 9, France.
authors.forename: Maithé
authors.initials: M
authors.lastname: Tauber
mesh_terms: D000215:Acylation; D000855:Anorexia / Q000097:blood* / Q000378:metabolism*; D005260:Female; D054439:Ghrelin / Q000097:blood* / Q000378:metabolism*; D006801:Humans; D007223:Infant; D008297:Male; D009765:Obesity / Q000097:blood / Q000378:metabolism; D011218:Prader-Willi Syndrome / Q000097:blood* / Q000378:metabolism*
chemical_list: D054439:Ghrelin
keywords: Anorexia; Ghrelin; Infants; Nutrition; Obesity; Prader-Willi syndrome