Lopes Paloma Silva, Serra Diógenes Pires, Matos Marcos Antônio Almeida
Escola Bahiana de Medicina e Saúde Pública (EBMSP), Salvador, BA, Brazil.
Santa Casa de Misericórdia da Bahia, Salvador, BA, Brazil.
Acta Ortop Bras. 2019 Jul-Aug;27(4):212-215. doi: 10.1590/1413-785220192704187274.
To measure the functional independence to perform activities of daily living of pediatric patients diagnosed with mucopolysaccharidoses.
A descriptive cross-sectional study was carried out with the population of pediatric patients with a confirmed enzymatic diagnosis of mucopolysaccharidoses, enrolled in the Orthopedics outpatient clinic of a hospital in the State of Bahia. The data were collected between October 2016 and March 2017, based on the documentary analysis of the assessment forms used in the department. The variables of this study comprised sex, age, type of MPS and level of functional independence, measured by the Functional Independence Measure scale.
Twenty-six patients participated in the study. These were predominantly male (61.5%), with a mean age of 10 ± 4.5 years, affected by MPS VI (73.1%). In the motor domain, the mean score was 65 (± 19.9 points); the cognitive domain obtained a mean score equal to 28 (± 8.2 points); and the total FIM score was 93 (± 26.5).
Impaired functional independence was observed among children and adolescents with mucopolysaccharidoses. Tasks related to dressing, toileting, bathing, problem solving and social interaction were those that required the most assistance and/or supervision.
评估确诊患有黏多糖贮积症的儿科患者进行日常生活活动的功能独立性。
对巴伊亚州一家医院骨科门诊登记的确诊患有黏多糖贮积症的儿科患者群体进行了描述性横断面研究。数据收集于2016年10月至2017年3月期间，基于该科室使用的评估表格的文献分析。本研究的变量包括性别、年龄、黏多糖贮积症类型和功能独立性水平，通过功能独立性测量量表进行测量。
26名患者参与了研究。这些患者以男性为主（61.5%），平均年龄为10±4.5岁，受MPS VI影响（73.1%）。在运动领域，平均得分为65（±19.9分）；认知领域的平均得分等于28（±8.2分）；FIM总分是93（±26.5）。
观察到患有黏多糖贮积症的儿童和青少年功能独立性受损。与穿衣、洗漱、洗澡、解决问题和社交互动相关的任务是最需要协助和/或监督的。
