淀粉样变性患者的功能损害:物理治疗评估

IF 0.9 Q4 HEMATOLOGY Hemato Pub Date : 2022-06-23 DOI:10.3390/hemato3030028
E. Redder, Qiuhong Zhao, N. Bumma, R. Kahwash, A. Vallakati, Courtney M. Campbell, Samir M. Parikh, S. Almaani, M. Freimer, Y. Efebera, Nidhi Sharma
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引用次数: 0

摘要

淀粉样变性是一种罕见的系统性疾病,可导致严重的功能损伤。淀粉样变性患者康复评估的具体指南尚未制定。本研究的目的是确定淀粉样变性患者的功能缺陷,并根据疾病类型、器官受累、年龄和性别评估差异。材料和方法:俄亥俄州立大学多学科淀粉样变性综合诊所(CAC)为淀粉样变性患者制定了结构化评估指南。对2017年12月至2020年4月期间在CAC评估的患者进行了回顾性单机构审查。通过图表回顾收集来自定时上下(TUG)、30秒坐到站和SF 36身体功能部分的结果测量数据。比较CAC患者评分和标准数据。Kruskal–Wallis检验用于比较不同疾病类型(轻链、转甲状腺素野生型和遗传变异型转甲状腺素)的得分,Mann–Whitney U检验用于疾病类型和心脏受累的成对比较。线性回归模型用于评估患者特征(包括年龄、性别、疾病类型和心脏受累)与表现得分之间的相关性。结果:对64名患者的数据进行了评估。在30-s坐立式试验中,轻链淀粉样变性患者的重复次数比转甲状腺素野生型患者少3.32次,p=0.03。心脏受累患者的重复次数比无心脏受累患者少2.55次,p=0.03。发现老年患者的TUG表现较慢,年龄增加10年与TUG评分增加11%相关。结论:研究结果表明,与其他淀粉样变性患者相比,轻链淀粉样变性和心脏受累患者表现出更多的身体损伤。
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Functional Impairments of Amyloidosis Patients: Physical Therapy Assessment
Amyloidosis is a rare, systemic disease that can result in significant functional impairment. Specific guidelines for the rehabilitation assessment of amyloidosis patients have yet to be established. The purpose of this study was to identify functional deficits and assess differences based on disease type, organ involvement, age, and gender of patients with amyloidosis. Materials and Methods: The multidisciplinary Comprehensive Amyloidosis Clinic (CAC) at Ohio State University (OSU) has developed structured assessment guidelines for amyloidosis patients. A retrospective, single-institution review of patients assessed in CAC between December 2017 and April 2020 was performed. Outcome measure data from the Timed Up and Go (TUG), 30 s sit-to-stand, and physical function portion of the SF 36 were gathered by chart review. Comparisons were made between CAC patient scores and normative data. Kruskal–Wallis tests were used to compare scores across the disease types (light chain, transthyretin wild-type, and hereditary variant transthyretin) and the Mann–Whitney U test was used for pairwise comparisons within disease types and cardiac involvement. Linear regression models were used to assess associations between patient characteristics (including age, gender, disease type, and cardiac involvement) and performance scores. Results: Data from sixty-four patients was evaluated. On the 30-s sit-to-stand test, patients with light chain amyloidosis performed 3.32 fewer repetitions than patients with transthyretin wild-type, p = 0.03. Patients with cardiac involvement had 2.55 fewer repetitions than patients without cardiac involvement, p = 0.03. Older patients were found to have slower TUG performance, and a 10-year increase in age was associated with an 11% increase in TUG scores. Conclusions: Findings indicate patients with light chain amyloidosis and patients with cardiac involvement, when compared to other amyloidosis patients, present with more physical impairments.
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