title: Quality of life assessment in a sample of patients affected by Prader-Willi syndrome.
abstract: OBJECTIVE
  The goals of this study are to investigate the quality of life of Prader-Willi syndrome patients and to evaluate the relationship between quality of life and the clinical picture.
  
  
  METHODS
  We performed a multicentric study on 40 consecutive patients with Prader-Willi syndrome. Quality of life was evaluated through the Short Form-36 and the Child Health Questionnaire-Parent Form-50 according to the age of patients.
  
  
  RESULTS
  In patients older than 14 years old, quality of life is intensely impaired both in mental and physical aspects. Weight at the moment of the observation, birthweight and facial features are the main variables that influence quality of life. In patients who are 14 years old or younger, the Family Activity and Physical scores are lower for those patients with characteristic facial features and in patients with decreased fetal movement or infantile lethargy. Self-esteem is lower in patients with a higher Mini Mental Score.
  
  
  CONCLUSIONS
  Physical and mental aspects of quality of life are impaired in Prader-Willi patients, weight is the clinical finding which mainly influences negatively the physical aspects of quality of life. However, weight does not cause mental problems. These are mainly due to the presence of characteristic facial features. Interestingly, a high birthweight is associated with less impairment of the mental aspects of quality of life.
authors.affiliation: Institute of Neurology, University of Cattolica, Roma, Italy. p.caliandro@rm.unicatt.it
authors.forename: Pietro
authors.initials: P
authors.lastname: Caliandro
authors.forename: Graziano
authors.initials: G
authors.lastname: Grugni
authors.forename: Luca
authors.initials: L
authors.lastname: Padua
authors.forename: Yllka
authors.initials: Y
authors.lastname: Kodra
authors.forename: Pietro
authors.initials: P
authors.lastname: Tonali
authors.forename: Luigi
authors.initials: L
authors.lastname: Gargantini
authors.forename: Letizia
authors.initials: L
authors.lastname: Ragusa
authors.forename: Antonino
authors.initials: A
authors.lastname: Crinò
authors.forename: Domenica
authors.initials: D
authors.lastname: Taruscio
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D007558:Italy; D008297:Male; D011218:Prader-Willi Syndrome / Q000235:genetics / Q000503:physiopathology* / Q000523:psychology*; D011788:Quality of Life; D011795:Surveys and Questionnaires