Diastrophic dysplasia (DD), a congenital skeletal dysplasia, is characterized by short, disproportionate stature, multiple severe spinal and joint deformities, and normal mental status.
The health-related quality of life (HRQOL) of patients with DD was measured by a standardized fifteen-dimensional (15D) method, that includes a questionnaire and a valuation task.
Eighteen patients (5 males and 13 females) with a mean age of 23 years (range 17 - 31 years), representing half of the Finnish patients with DD in this age group, completed the questionnaire and the valuation task.
The data obtained were compared with those of 273 age-and sex-matched controls.
A 15D profile was drawn, and the average importance weight of each dimension and the average within-dimension level values were calculated for both groups.
The average 15D score, describing the overall HRQOL, was derived for both groups using the valuations of each group.
The health profiles of the groups differed significantly in several dimensions.
When the health levels within the dimensions were concerned, the controls rated all values deviating from « normal » lower than did the patients.
The average 15D scores of the patients with DD were significantly lower than those of the controls.
The 15D scores were higher in both groups when the valuations of the patients with DD were used. (...)
Mots-clés Pascal : Nanisme diastrophique, Qualité vie, Evaluation, Echelle évaluation, Homme, Questionnaire, Système ostéoarticulaire pathologie, Ostéochondrodysplasie, Maladie héréditaire
Mots-clés Pascal anglais : Diastrophic dysplasia, Quality of life, Evaluation, Evaluation scale, Human, Questionnaire, Diseases of the osteoarticular system, Osteochondrodysplasia, Genetic disease
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0285670
Code Inist : 002B15H. Création : 16/11/1999.