title: French database of children and adolescents with Prader-Willi syndrome.
abstract: BACKGROUND
  Prader-Willi syndrome (PWS) is a rare multisystem genetic disease leading to severe complications mainly related to obesity. We strongly lack information on the natural history of this complex disease and on what factors are involved in its evolution and its outcome. One of the objectives of the French reference centre for Prader-Willi syndrome set-up in 2004 was to set-up a database in order to make the inventory of Prader-Willi syndrome cases and initiate a national cohort study in the area covered by the centre.
  
  
  METHODS
  the database includes medical data of children and adolescents with Prader-Willi syndrome, details about their management, socio-demographic data on their families, psychological data and quality of life of the parents. The tools and organisation used to ensure data collection and data quality in respect of good clinical practice procedures are discussed, and main characteristics of our Prader-Willi population at inclusion are presented.
  
  
  CONCLUSIONS
  this database covering all the aspects of PWS clinical, psychological and social profiles, including familial psychological and quality of life will be a powerful tool for retrospective studies concerning this complex and multi factorial disease and could be a basis for the design of future prospective multicentric studies. The complete database and the Stata.do files are available to any researcher wishing to use them for non-commercial purposes and can be provided upon request to the corresponding author.
authors.affiliation: Centre de Référence du syndrome de Prader-Willi, Division of Endocrinology, Genetics, Gynaecology and Bone Diseases, Hôpital des Enfants, and University Paul Sabatier, Toulouse, France. molinas.c@chu-toulouse.fr
authors.forename: Catherine
authors.initials: C
authors.lastname: Molinas
authors.forename: Laurent
authors.initials: L
authors.lastname: Cazals
authors.forename: Gwenaelle
authors.initials: G
authors.lastname: Diene
authors.forename: Melanie
authors.initials: M
authors.lastname: Glattard
authors.forename: Catherine
authors.initials: C
authors.lastname: Arnaud
authors.forename: Maithe
authors.initials: M
authors.lastname: Tauber
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011218:Prader-Willi Syndrome / Q000453:epidemiology / Q000235:genetics*; D011788:Quality of Life; D011795:Surveys and Questionnaires