Programa de Pós-graduação em Medicina e Saúde, Faculdade de Medicina da Bahia, Universidade Federal da Bahia, Salvador, BA, Brasil; Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, BA, Brasil.
Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, BA, Brasil.
J Pediatr (Rio J). 2023 Jan-Feb;99(1):94-98. doi: 10.1016/j.jped.2022.07.002. Epub 2022 Aug 5.
To evaluate the functional status of individuals with Osteogenesis Imperfecta (OI) followed up at a reference center in the state of Bahia.
This is an observational, cross-sectional, descriptive study, which evaluated individuals with OI, based on a non-probabilistic sampling. To assess motor function, the Motor Function Measure (MFM) score was used, in addition to the measurement of muscle strength using the Medical Research Council (MRC) score. Functional performance was measured using the Pediatric Assessment of Disability Inventory, Computerized Adaptive Testing (PEDI-CAT).
Thirty-one individuals aged between two and 18 years old were evaluated. The overall score of MFM was 74.2%, and the lowest score was found in participants with type III OI (56.3%). The median of the MRC index was 80. The mobility domain was the most affected in the PEDI-CAT evaluation, with a mean T score of 23.9, (14.2 in type III OI).
Among the evaluated individuals, functional alterations were identified, reduced global gross motor functionality and muscle strength, impacting the mobility domain, with the most relevant findings in individuals with type III OI.
评估在巴伊亚州参考中心接受治疗的成骨不全症（OI）患者的功能状态。
这是一项观察性、横断面、描述性研究，采用非概率抽样对 OI 患者进行评估。为了评估运动功能，使用运动功能测量（MFM）评分，此外还使用医学研究委员会（MRC）评分测量肌肉力量。使用儿童残疾评估量表，计算机自适应测试（PEDI-CAT）来测量功能表现。
共评估了 31 名年龄在 2 至 18 岁之间的患者。MFM 的总体评分为 74.2%，而 III 型 OI 患者的得分最低（56.3%）。MRC 指数的中位数为 80。在 PEDI-CAT 评估中，受影响最严重的是移动能力领域，平均 T 分数为 23.9，（III 型 OI 为 14.2）。
在所评估的患者中，存在功能障碍，全身粗大运动功能和肌肉力量下降，影响移动能力领域，在 III 型 OI 患者中发现的相关性最强。
