Determinants of quality of life in Marfan syndrome

Psychosomatics. 2008 May-Jun;49(3):243-8. doi: 10.1176/appi.psy.49.3.243.

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.

MeSH terms

  • Adaptation, Psychological
  • Adult
  • Demography
  • Depression / diagnosis
  • Depression / epidemiology
  • Depression / etiology
  • Female
  • Health Behavior
  • Humans
  • Karnofsky Performance Status
  • Male
  • Marfan Syndrome / epidemiology
  • Marfan Syndrome / physiopathology
  • Marfan Syndrome / psychology*
  • Psychometrics
  • Quality of Life / psychology*
  • Sex Factors
  • Surveys and Questionnaires