title: Quantifying Health Status and Function in Marfan Syndrome.
abstract: Two hundred thirty patients were prospectively enrolled in this study and completed various portions of the Short Form 36 and a study-specific questionnaire (visual analog scale 1 to 10, comprising three separate questionnaires) to evaluate quality of life and function in patients with Marfan syndrome. The greatest health concern was cardiac problems (high in 70% of patients), followed by spine issues and generalized fatigue (both high, in 53%). The most severe reported pain involved the back: 105 patients (46%) rated pain as 6 to 10 on the visual analog scale. Among the 72 patients who responded to work life questions, work hours were reduced because of treatment in 59 (82%) or directly because of Marfan syndrome in 29 (40%). Across all Short Form 36 domains, patients scored significantly lower than United States population norms (p<.05); physical health scores were considerably lower than mental health scores.
authors.affiliation: Department of Orthopaedic Surgery, The Johns Hopkins University, Baltimore, Maryland.
authors.forename: Sandesh S
authors.initials: SS
authors.lastname: Rao
authors.forename: Kristen D
authors.initials: KD
authors.lastname: Venuti
authors.forename: Harry C
authors.initials: HC
authors.lastname: Dietz
authors.forename: Paul D
authors.initials: PD
authors.lastname: Sponseller
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D004651:Employment; D005221:Fatigue / Q000209:etiology / Q000503:physiopathology* / Q000523:psychology; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000503:physiopathology* / Q000523:psychology; D008603:Mental Health; D008875:Middle Aged; D010146:Pain / Q000209:etiology / Q000503:physiopathology* / Q000523:psychology; D011446:Prospective Studies; D011788:Quality of Life; D011795:Surveys and Questionnaires; D014937:Work; D055815:Young Adult