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. METHOD: 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. CONCLUSION: The authors found that MFS negatively influences QoL, increases psychological distress, and may be a possible risk for some psychiatric disorders.
Determinants of quality of life in Marfan syndrome
Fusar Poli P;STRAMESI, FRANCESCA;ARBUSTINI, ELOISA;POLITI, PIERLUIGI
2008-01-01
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. METHOD: 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. CONCLUSION: The authors found that MFS negatively influences QoL, increases psychological distress, and may be a possible risk for some psychiatric disorders.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
