Marfan Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Marfan Syndrome, including details on genetics, causes, symptoms, treatment.

Determinants of quality of life in Marfan syndrome.

Fusar-Poli P, Klersy C, Stramesi F, Callegari A, Arbustini E, Politi P

DSSAeP, Sezione di Psichiatria, Università di Pavia, Pavia, Italy. p.fusar@libero.it

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.

Published 1 May 2008 in Psychosomatics, 49(3): 243-8.
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