在肢体-腰带肌肉缩中疼痛干扰和疲劳 R9
Anna M Reelfs1, Carrie M Stephan2, Shelley R H Mockler3
1University of Iowa Carver College of Medicine, 375 Newton Rd, Iowa City, IA 52242, United States.
Neuromuscular disorders : NMD
|May 29, 2023
概括
肢体腰带肌肉衰竭 (LGMD) R9中的疼痛是可变的,而疲劳随着年龄的增长而增加. 这些症状对患有FKRP变异的儿童和成年人的日常生活产生不同的影响.
科学领域:
- 神经学 神经学
- 遗传学 是一个遗传学.
- 临床医学 临床医学
背景情况:
- 肢体腰带肌肉发育不良 (LGMD) R9,由双性福库丁相关蛋白 (FKRP) 变体引起,与疼痛有关,但其对日常生活的影响和与疲劳的关系尚不清楚.
- 了解这些方面对于管理受影响个体的生活质量至关重要.
研究的目的:
- 调查LGMD R9.9. 的儿童和成人疼痛干扰和疲劳的患病率和影响.
- 在这个患者群体中探索疼痛,疲劳,年龄,性别,行走状态,运动功能和肺功能之间的相关性.
主要方法:
- 长度研究涉及23名儿童和54名患有FKRP变异的成年人.
- 使用患者报告结果测量信息系统 (PROMIS) 进行年度评估. 疼痛干扰和疲劳短形式长达六年.
- 同时评估运动和肺功能.
主要成果:
- 疼痛干扰T-分数接近一般人群平均值 (48.5在儿童中,51.6在成人中),其中58%的人至少有一次经历过更高的水平.
- 成年人 (55.1) 的疲劳T-分数较高,但儿童 (49.0) 没有,75%的人至少一次报告过过度疲劳.
- 疼痛和疲劳是正相关的 (r=0.55) 并随着年龄的增长而增加. 运动和肺功能与成年人疲劳相关.
结论:
- 在LGMD R9中的疼痛似乎是可变的和偶发的,对大多数人的日常生活影响有限.
- 在患有LGMD R9的个体中,疲劳倾向于随着时间的推移而增加,特别是在成年人中,并且与功能下降有关.
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