title: Systematic review of chronic pain in persons with Marfan syndrome.
abstract: The purpose of this study was to explore the literature on chronic pain in adults with Marfan syndrome (MFS), critically appraising and synthesizing relevant literature. A systematic review was conducted by searching the published literature databases using available medical, physical, psychological, social databases and other sources. All studies that addressed pain in MFS, published in peer-reviewed journals were assessed. Of 351 search results, 18 articles satisfied the eligibility criteria. All studies were cross-sectional and quantitative; no randomized controlled trials or intervention studies were found. Most studies had small sample sizes, low response rates and mainly dealt with other aspects of the diagnosis than pain. Only one article dealt mainly with pain. The research on chronic pain in MFS is limited in size and quality. Despite these limitations, studies describe that the prevalence of pain in patients with MFS is high, varying from 47 to 92% and affecting several anatomic sites. In addition, chronic pain limits daily function and few studies describe treatment options for pain in patients with MFS. Research is needed to obtain more evidence-based knowledge for developing more appropriate rehabilitation programs for people with MFS.
authors.affiliation: TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, Nesodden, 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.affiliation: TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, Nesodden, Norway.
authors.forename: T
authors.initials: T
authors.lastname: Bathen
authors.affiliation: TRS National Resource Centre for Rare Disorders, Sunnaas Rehabilitation Hospital, Nesodden, Norway.|Institute of clinical medicine, Faculty of Medicine, University of Oslo, Oslo, Norway.
authors.forename: S
authors.initials: S
authors.lastname: Rand-Hendriksen
authors.affiliation: 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: A Ø
authors.initials: AØ
authors.lastname: Geirdal
mesh_terms: D059350:Chronic Pain / Q000235:genetics / Q000523:psychology* / Q000628:therapy*; D003430:Cross-Sectional Studies; D000071838:Fibrillin-1 / Q000235:genetics; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D008382:Marfan Syndrome / Q000235:genetics / Q000523:psychology* / Q000628:therapy*; D009154:Mutation; D011788:Quality of Life / Q000523:psychology; D012107:Research Design; D016896:Treatment Outcome; D054392:Adipokines
chemical_list: D000071838:Fibrillin-1; C000605722:FBN1 protein, human; D054392:Adipokines
keywords: Marfan syndrome; associated factors; chronic pain; prevalence and systematic review