Rohenkohl A C, Bullinger M, Quitmann J
Universitätsklinikum Hamburg - Eppendorf, Institut für medizinische Psychologie, Martinistraße 52, W26, 20246, Hamburg, Deutschland,
Orthopade. 2015 Mar;44(3):212-8. doi: 10.1007/s00132-014-3020-9.
Compared to research on short-statured adults, quality of life (QoL) of children has been rarely studied. One reason for this might be the lack of appropriate disease-specific questionnaires.
The aim of this study was to analyse the quality of life in a sample of short-statured children with achondroplasia, using generic and disease-specific instruments. In addition, a comparison of patient and population norms is presented.
The sample included children (8-28 years) with achondroplasia and parents of participating children (8-17 years). Quality of life was analyzed with the KIDSCREEN, the DISABKIDS and the disease-specific Quality of Life in Short Stature Youth (QoLISSY) questionnaire. In addition group differences according to clinical and sociodemographic data were analyzed within the sample and compared to available KIDSCREEN representative population data.
The physical QoL was rated poorly in this sample of short-statured patients, while the emotional QoL was rated more favorably. Compared to the KIDSCREEN population norm, parents of children with achondroplasia rate the QoL lower.
The QoLISSY questionnaire is a reliable tool to assess the subjective wellbeing of patients with skeletal dysplasia. The instrument can now be used clinically as a screening for patient wellbeing, as an outcome criterion in clinical research and as a psychosocial indicator in orthopedic cohort studies.
与对身材矮小成年人的研究相比，儿童的生活质量（QoL）很少被研究。造成这种情况的一个原因可能是缺乏合适的针对特定疾病的问卷。
本研究的目的是使用通用和特定疾病的工具分析一组患有软骨发育不全的身材矮小儿童的生活质量。此外，还对患者和总体标准进行了比较。
样本包括患有软骨发育不全的儿童（8 - 28岁）以及参与研究儿童的父母（8 - 17岁）。使用儿童生活质量量表（KIDSCREEN）、残疾儿童生活质量量表（DISABKIDS）和特定疾病的青少年身材矮小生活质量问卷（QoLISSY）对生活质量进行分析。此外，根据临床和社会人口学数据在样本内分析组间差异，并与现有的儿童生活质量量表代表性总体数据进行比较。
在这个身材矮小患者样本中，身体生活质量评分较低，而情感生活质量评分相对较好。与儿童生活质量量表的总体标准相比，软骨发育不全儿童的父母对生活质量的评分更低。
青少年身材矮小生活质量问卷（QoLISSY）是评估骨骼发育异常患者主观幸福感的可靠工具。该工具现在可在临床上用于筛查患者的幸福感，作为临床研究的结果标准以及骨科队列研究中的社会心理指标。
