title: Determinants of quality of life in Marfan syndrome.
abstract: BACKGROUND
  Marfan syndrome (MFS) is a rare, heritable disorder that affects connective tissue. Men and women are equally affected. Clinical manifestations involve multiple sites, especially bones and ligaments and heart and blood vessels.
  
  
  OBJECTIVE
  Authors sought to investigate quality of life (QoL) in MFS patients, assessing positive and negative sociodemographic factors and self-perceived well-being and functional status.
  
  
  METHODS
  Thirty-six patients affected by MFS were interviewed and were administered the SF-36 psychometric questionnaire.
  
  
  RESULTS
  Subjects affected by MFS reported an impaired quality of life in the psychological domain but not in the physical domain, as compared with a healthy population. Being male and older was significantly associated with a poorer perceived mental QoL.
  
  
  CONCLUSIONS
  The authors found that MFS negatively influences QoL, increases psychological distress, and may be a possible risk for some psychiatric disorders.
authors.affiliation: DSSAeP, Sezione di Psichiatria, Università di Pavia, Pavia, Italy. p.fusar@libero.it
authors.forename: Paolo
authors.initials: P
authors.lastname: Fusar-Poli
authors.forename: Catherine
authors.initials: C
authors.lastname: Klersy
authors.forename: Francesca
authors.initials: F
authors.lastname: Stramesi
authors.forename: Aliria
authors.initials: A
authors.lastname: Callegari
authors.forename: Eloisa
authors.initials: E
authors.lastname: Arbustini
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Politi
mesh_terms: D000223:Adaptation, Psychological; D000328:Adult; D003710:Demography; D003863:Depression / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology; D005260:Female; D015438:Health Behavior; D006801:Humans; D017567:Karnofsky Performance Status; D008297:Male; D008382:Marfan Syndrome / Q000453:epidemiology / Q000503:physiopathology / Q000523:psychology*; D011594:Psychometrics; D011788:Quality of Life / Q000523:psychology*; D012737:Sex Factors; D011795:Surveys and Questionnaires