title: Systematic review of the psychosocial aspects of living with Marfan syndrome.
abstract: The purpose of this study was to explore the literature on the psychosocial aspects of Marfan syndrome (MFS), to critically appraise and to synthesize relevant literature. A mixed-method systematic review was performed by searching the published literature databases using available medical, psychological, pedagogical and social databases and other sources. All studies that addressed psychosocial aspects of MFS, published in peer-reviewed journals were assessed. Of 81 search results, 15 articles (four articles based on same study population) satisfied the eligibility criteria. All studies were cross-sectional; no intervention or randomized controlled trial (RCT) studies were found. Most studies were of small sample sizes, had low response rate or participants without a verified diagnosis. Despite these limitations, all studies described, that MFS has a significant impact on the psychosocial aspects of people's lives: Decreased quality of life; challenges in education, work and family life, depression and anxiety. Some studies indicated that the subjective perception of discomfort did not necessarily match the medical severity of a disease. The research of the psychosocial aspects of MFS is limited in size and quality. More research is needed on the psychosocial aspects of MFS in samples with a verified diagnosis to develop evidence-based knowledge and appropriate guidelines.
authors.affiliation: Sunnaas Rehabilitation Hospital, TRS National Resource Centre for Rare Disorders, Nesoddtangen, Akershus, Norway; Norway Faculty of Social Sciences, Department of Social Work, Child Welfare and Social Policy, Oslo and Akershus University College of Applied Sciences, Oslo, Norway.
authors.forename: G
authors.initials: G
authors.lastname: Velvin
authors.forename: T
authors.initials: T
authors.lastname: Bathen
authors.forename: S
authors.initials: S
authors.lastname: Rand-Hendriksen
authors.forename: A Ø
authors.initials: AØ
authors.lastname: Geirdal
mesh_terms: D001007:Anxiety / Q000453:epidemiology / Q000523:psychology; D003863:Depression / Q000453:epidemiology / Q000523:psychology; D006801:Humans; D008382:Marfan Syndrome / Q000453:epidemiology* / Q000473:pathology / Q000523:psychology*; D011788:Quality of Life; D016032:Randomized Controlled Trials as Topic
keywords: Marfan syndrome; psychosocial aspects; quality of life; review