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利用国际功能、残疾和健康分类为脊髓性肌萎缩症患者设定多学科干预目标:小样本量的试点研究

Setting multidisciplinary intervention goals for spinal muscular atrophy patients utilizing the international classification of functioning, disability, and health: a pilot study in a small sample sizes.

作者信息

Giannotta Gabriele, Ruggiero Marta, Pirani Greta, Oliva Maria Carmela, Ferrante Camilla, Trabacca Antonio

机构信息

Associazione "La Nostra Famiglia"- IRCCS "E. Medea" - Scientific Hospital for Neurorehabilitation - Unit for Severe Disabilities in Developmental Age and Young Adults (Developmental Neurology and Neurorehabilitation), Brindisi, Italy.

Scientific Institute I.R.C.C.S. "E. Medea", Scientific Direction, Bosisio Parini (LC), Via Don L. Monza 20, 23842, Bosisio Parini (LC), Italy.

出版信息

Acta Neurol Belg. 2025 Mar 26. doi: 10.1007/s13760-025-02771-6.

DOI:10.1007/s13760-025-02771-6
PMID:40133732
Abstract

BACKGROUND

Spinal Muscular Atrophy (SMA) is a genetic neuromuscular disorder causing progressive motor function loss, respiratory and swallowing impairments, and significant limitations in activities of daily living (ADLs) and social participation. While gene therapies have enhanced life expectancy, functional management remains crucial. Despite the International Classification of Functioning Disability and Health for Children and Youth (ICF-CY) being a valuable framework for patient-centered care, its integration in setting multidisciplinary rehabilitation goals for SMA patients is underexplored.

OBJECTIVE

This study aimed to assess the use of the ICF-CY framework in developing multidisciplinary intervention goals for SMA patients.

METHODS

A cross-sectional study involving 6 SMA patients was conducted at a pediatric neurorehabilitation hospital. Data were collected using a standardized ICF-CY-based case record form and analysed to identify patterns of impairment, activity limitations, and environmental factors.

RESULTS

Results demonstrated the multifaceted nature of SMA, with significant impairments in motor function, respiratory function, and swallowing, as well as limitations in activities of daily living and social participation. Environmental factors, particularly social support and accessibility, emerged as crucial determinants of overall well-being.

CONCLUSIONS

The study underscores the importance of a multidisciplinary approach and the use of ICF-CY framework for developing comprehensive and patient-centered care plans for SMA patients.

摘要

背景

脊髓性肌萎缩症(SMA)是一种遗传性神经肌肉疾病,会导致进行性运动功能丧失、呼吸和吞咽障碍,以及日常生活活动(ADL)和社会参与方面的显著限制。虽然基因疗法提高了预期寿命,但功能管理仍然至关重要。尽管《儿童和青少年功能、残疾与健康国际分类》(ICF-CY)是患者为中心的护理的宝贵框架,但其在为SMA患者设定多学科康复目标中的整合尚未得到充分探索。

目的

本研究旨在评估ICF-CY框架在为SMA患者制定多学科干预目标中的应用。

方法

在一家儿科神经康复医院对6名SMA患者进行了横断面研究。使用基于ICF-CY的标准化病例记录表收集数据,并进行分析以确定损伤模式、活动限制和环境因素。

结果

结果显示了SMA的多方面性质,在运动功能、呼吸功能和吞咽方面存在显著损伤,以及在日常生活活动和社会参与方面存在限制。环境因素,特别是社会支持和可达性,成为总体幸福感的关键决定因素。

结论

该研究强调了多学科方法以及使用ICF-CY框架为SMA患者制定全面且以患者为中心的护理计划的重要性。

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Measuring Fatigue and Fatigability in Spinal Muscular Atrophy (SMA): Challenges and Opportunities.测量脊髓性肌萎缩症(SMA)中的疲劳和易疲劳性:挑战与机遇
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