Escola Bahiana de Medicina e Saúde Pública, Salvador, Bahia, Brasil.
University of Rochester Medical Center, Rochester-NY, USA.
Colomb Med (Cali). 2020 Sep 30;51(3):e213996. doi: 10.25100/cm.v51i3.3996.
To assess the functional independence of a group of patients with mucopolysaccharidosis using the Functional Independence Measure as a tool that accomplishes this purpose.
This is a cross-sectional study of patients with mucopolysaccharidosis. Our data was collected between June 2015 and July 2016. In addition to history of present illness and physical examination each study participant was asked to answer a questionnaire to specifically evaluate their functional independence using the functional independence measure. the internal consistency of the functional independence measure was assessed using Cronbach's alpha coefficient.
We collected data on 20 patients with mucopolysaccharidosis. The average age was 10.8 (8.67-13.03) years, the average weight was 23.6 (19.91-27.37) kg and the average height was 1 (0.83-1.17) m. The most prevalent type of mucopolysaccharidosis in the study was type VI (n= 14). The average total functional independence measure score was 104.4 (97.61-111.19), the average for the mobility domain was 73.50 (68.22-78.78) and the average for the cognitive function domain was 30.90 (28.68-33.13). The internal consistency of the entire questionnaire was 0.859, with values of 0.966 for the mobility domain and 0.624 for the cognitive function domain.
The lowest functional independence measure scores were obtained in the following sub-domains: self-care, locomotion and cognitive function. The functional independence measure questionnaire demonstrated internal consistency for the evaluation of functional independence in patients with mucopolysaccharidosis, being able to value all the affected sub-domains separately.
使用功能独立性评定量表（FIM）作为评估工具，评估一组黏多糖贮积症患者的功能独立性。
这是一项黏多糖贮积症患者的横断面研究。我们的数据收集时间为 2015 年 6 月至 2016 年 7 月。除了现病史和体格检查外，每位研究参与者还被要求回答一份问卷，该问卷专门使用功能独立性评定量表评估他们的功能独立性。使用 Cronbach's α 系数评估功能独立性评定量表的内部一致性。
我们收集了 20 例黏多糖贮积症患者的数据。平均年龄为 10.8 岁（8.67-13.03 岁），平均体重为 23.6kg（19.91-27.37kg），平均身高为 1m（0.83-1.17m）。研究中最常见的黏多糖贮积症类型为 VI 型（n=14）。平均总功能独立性评定量表评分为 104.4 分（97.61-111.19 分），移动域平均评分为 73.50 分（68.22-78.78 分），认知功能域平均评分为 30.90 分（28.68-33.13 分）。整个问卷的内部一致性为 0.859，移动域的评分为 0.966，认知功能域的评分为 0.624。
功能独立性评定量表得分最低的领域为自我护理、移动和认知功能。功能独立性评定量表问卷在评估黏多糖贮积症患者的功能独立性方面具有内部一致性，能够分别评估所有受影响的子领域。
