title: Functional health status of adults with achondroplasia.
abstract: Little is known regarding the functional health status of individuals with achondroplasia. This cross-sectional survey of adults with achondroplasia was undertaken to assess the functional health status of this population and its determinants. The study sample consisted of members of the Little People of America (LPA) who completed a mailed questionnaire consisting of a demographics component, a general and disease-specific comorbidities component, and the Short Form 36 (SF-36) health status questionnaire. Univariate analyses and multivariate linear regression models were used for data analysis. Four hundred thirty-seven individuals with a mean age of 38 years completed the survey. The age- and gender-adjusted Mental Component Summary (MCS) scores did not significantly differ from those of the general population. In contrast, the age- and gender-adjusted Physical Component Summary (PCS) scores were significantly lower than the general population starting in the fourth decade of life. Musculoskeletal diseases were most prevalent and had the greatest impact on the PCS scores. Two-thirds of this cohort had undergone at least one operation. Only musculoskeletal procedures were significantly associated with PCS and MCS scores. The functional health status of adults with achondroplasia, as measured by the SF-36, is not drastically reduced in comparison with that of the general U.S. population.
authors.affiliation: Robert Brigham Multipurpose Arthritis and Musculoskeletal Diseases Center, Department of Orthopaedics, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: N N
authors.initials: NN
authors.lastname: Mahomed
authors.forename: M
authors.initials: M
authors.lastname: Spellmann
authors.forename: M J
authors.initials: MJ
authors.lastname: Goldberg
mesh_terms: D000130:Achondroplasia / Q000503:physiopathology* / Q000601:surgery; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015331:Cohort Studies; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D008875:Middle Aged