title: [Extreme obesity in Prader-Willi Syndrome (PWS)].
abstract: BACKGROUND
  Prader-Willi Syndrome (PWS) belong to genetic obesities and we report a caricatural observation.
  
  
  METHODS
  The early onset of (PWS) is characterised by a severe neonatal hypotonia with poor suck reflex--which may lead to tube feeding--and poor weight gain. Later appears insatiable appetite, morbid obesity associated with short stature, dysmorphic syndrome with small hands and behavioural disorders. Although diagnosis is based on clinical features, it must be confirmed by genetic test looking for the characteristic deletion of the chromosome 15q11-q13 region. PWS is the first example in humans of genetic imprinting.
  
  
  CONCLUSIONS
  Today, the challenge in PWS is it early management which may authorise Growth Hormone administration.
authors.affiliation: Service d'endocrinologie, hôpital Bégin, 69, avenue de Paris, 94160 Saint-Mandé, France.
authors.forename: J
authors.initials: J
authors.lastname: Damiano
authors.forename: C
authors.initials: C
authors.lastname: Ficko
authors.forename: E
authors.initials: E
authors.lastname: Garrabé
authors.forename: H
authors.initials: H
authors.lastname: Mayaudon
authors.forename: O
authors.initials: O
authors.lastname: Dupuy
authors.forename: T
authors.initials: T
authors.lastname: Carmoi
authors.forename: L
authors.initials: L
authors.lastname: Bordier
authors.forename: B
authors.initials: B
authors.lastname: Bauduceau
mesh_terms: D000328:Adult; D001827:Body Height; D002884:Chromosomes, Human, Pair 15; D005260:Female; D005820:Genetic Testing; D019382:Human Growth Hormone / Q000627:therapeutic use; D006801:Humans; D009767:Obesity, Morbid / Q000209:etiology* / Q000473:pathology; D011218:Prader-Willi Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics
chemical_list: D019382:Human Growth Hormone
vernacular_title: Obésité extrême au cours d'un syndrome de Willi-Prader.