Fusar-Poli Paolo, Klersy Catherine, Stramesi Francesca, Callegari Aliria, Arbustini Eloisa, Politi Pierluigi
DSSAeP, Sezione di Psichiatria, Università di Pavia, Pavia, Italy.
Psychosomatics. 2008 May-Jun;49(3):243-8. doi: 10.1176/appi.psy.49.3.243.
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.
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.
Thirty-six patients affected by MFS were interviewed and were administered the SF-36 psychometric questionnaire.
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.
The authors found that MFS negatively influences QoL, increases psychological distress, and may be a possible risk for some psychiatric disorders.
马凡综合征（MFS）是一种罕见的遗传性疾病，会影响结缔组织。男性和女性受影响的几率相同。临床表现涉及多个部位，尤其是骨骼、韧带以及心脏和血管。
作者试图调查马凡综合征患者的生活质量（QoL），评估社会人口统计学的积极和消极因素以及自我感知的幸福感和功能状态。
对36名马凡综合征患者进行了访谈，并让他们填写了SF-36心理测量问卷。
与健康人群相比，马凡综合征患者报告在心理领域的生活质量受损，但在身体领域没有。男性和年龄较大与较差的心理生活质量感知显著相关。
作者发现马凡综合征对生活质量有负面影响，增加心理困扰，并且可能是某些精神疾病的潜在风险。
