Determinants of quality of life in Marfan syndrome.
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.
Fusar-Poli
Paolo
P
DSSAeP, Sezione di Psichiatria, Università di Pavia, Pavia, Italy. p.fusar@libero.it
Klersy
Catherine
C
Stramesi
Francesca
F
Callegari
Aliria
A
Arbustini
Eloisa
E
Politi
Pierluigi
P
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