Manual ability and upper limb performance in nonambulatory stage of Duchenne muscular dystrophy

Manual ability and upper limb performance in nonambulatory stage of Duchenne muscular dystrophy
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DOI:
10.1016/j.arcped.2020.06.007
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发表时间:
2020-08-01
影响因子:
1.8
通讯作者:
Alemdaroglu-Gurbuz, I
Alemdaroglu-Gurbuz, I
中科院分区:
医学4区
文献类型:
--
作者:
Demir, G.;Bulut, N.;Alemdaroglu-Gurbuz, I

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目的:随着杜氏肌营养不良症(DMD)患者寿命的延长,上肢在日常生活活动中的功能性使用变得越来越重要。本研究的目的是评估的手的能力和上肢性能的不能行走的儿童DMD,并确定其与可能影响的能力和performance.Methods:手的能力,确定通过ABILHAND-Kids问卷和上肢性能进行评估上肢性能(PUL)测试。可能的相关因素,如功能水平,类固醇的使用,上肢活动范围(ROM),拇指对掌,上肢肌肉力量,握力进行了评估。结果:23例DMD患儿平均年龄为13.04 ± 1.39岁。根据ABILHAND-Kids和PUL确定中度损伤,评分分别为26.30 +/- 10.74和46.22 +/- 15.02。儿童的功能水平、类固醇使用、轮椅使用时间、上肢活动度和整体上肢肌力与ABILHAND-Kids和PUL中至少一项评分呈弱到强相关(P < 0.05)。结论:DMD患儿在发展到不能行走阶段时,可能已经有严重的近端和中段上肢受累,甚至反映在远端功能上。除了肌肉力量,治疗师还应考虑许多相关因素,以评估和治疗DMD后期的上肢功能。(C)2020年法国儿科学会。由Elsevier Masson SAS出版。All rights reserved.
Purpose: The functional use of the upper extremities in daily living activities has become important in the later ages with the increasing life expectancy of patients with Duchenne muscular dystrophy (DMD). This study aimed to assess manual ability and upper limb performance of nonambulatory children with DMD, and to determine their relationship with factors that might affect ability and performance.Methods: Manual ability was determined via the ABILHAND-Kids questionnaire and upper limb performance was assessed with the Performance of Upper Limb (PUL) test. Possible related factors such as functional level, steroid usage, upper limb range of motion (ROM), thumb opposition, upper limb muscular strength, and grip strength were evaluated. Correlations of related factors with manual ability and upper limb performance were analyzed.Results: The mean age of 23 nonambulatory DMD children was 13.04 +/- 1.39 years. Moderate impairments were determined according to the ABILHAND-Kids and PUL, with scores of 26.30 +/- 10.74 and 46.22 +/- 15.02, respectively. The functional level, steroid usage, duration of wheelchair use, upper extremity ROM, and global upper limb muscle strength of children were weak-to-strongly correlated with at least one score of ABILHAND-Kids and PUL (P < 0.05).Conclusion: Children with DMD may already have severe proximal and mid-level upper extremity involvement, even reflected in distal functions, at the time when they progress to the nonambulatory stage. Besides muscular strength, many related factors should be taken into account for therapists to assess and treat upper limb performance at later stages of DMD. (C) 2020 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.