Vaara P, Sintonen H, Peltonen J, Hokkanen H, Poussa M, Ryöppy S
Department of Paediatric Orthopaedics, Hospital for Children and Adolescents, University of Helsinki, Finland.
Scand J Public Health. 1999 Mar;27(1):38-42.
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 evaluations 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 evaluations of the patients with DD were used. The study showed that, although the overall HRQOL of the patients with DD was inferior to that of the controls, the patients showed greater adaptability to deviations in health status than did the controls.
脊柱骨骺发育不良（DD）是一种先天性骨骼发育不良，其特征为身材矮小、比例失调、多处严重脊柱和关节畸形，以及精神状态正常。采用标准化的十五维度（15D）方法对DD患者的健康相关生活质量（HRQOL）进行测量，该方法包括一份问卷和一项评估任务。18名患者（5名男性和13名女性），平均年龄23岁（范围17 - 31岁），代表了该年龄组芬兰DD患者的一半，完成了问卷和评估任务。将获得的数据与273名年龄和性别匹配的对照组的数据进行比较。绘制了15D概况，并计算了两组各维度的平均重要性权重和维度内平均水平值。使用每组的评估结果得出两组描述总体HRQOL的平均15D分数。两组的健康概况在几个维度上有显著差异。当涉及维度内的健康水平时，对照组对所有偏离“正常”的值的评分低于患者。DD患者的平均15D分数显著低于对照组。当使用DD患者的评估结果时，两组的15D分数都更高。该研究表明，尽管DD患者的总体HRQOL低于对照组，但患者比对照组对健康状况偏差的适应性更强。
