The association of hand grip strength with functional measures in non-ambulatory children with Duchenne muscular dystrophy

ABSTRACT Duchenne muscular dystrophy (DMD) is a disease characterized by progressive loss of muscle fiber, gradually from proximal to distal. Although a few studies have investigated hand grip strength in non-ambulatory DMD patients, a lack of literature was found determining its relationship with functional capacity. Objective: The aim of this study was to determine the associations between hand grip strength and functional measures in non-ambulatory children with DMD. Methods: Hand grip strength was evaluated using a dynamometer in children with DMD. The children with DMD were evaluated with the Turkish version of the Egen Klassifikation Scale Version 2 (EK2) for global functional capacity, the Performance of Upper Limb (PUL) for upper limb functional performance and the ABILHAND-Kids for hand ability. Results: The mean age of 38 DMD children was 12.02 ± 1.99 years. Dominant hand grip strength of the children with DMD was higher than the non-dominant hand (p < 0.05). The EK2 was 13.02 ± 5.50, PUL was 49.86 ± 14.34 and ABILHAND-Kids was 26.81 ± 7.59. Hand grip strength was found to be correlated with the EK2 (p < 0.05). Conclusions: It is known that measuring functional ability and strength in very weak children with DMD has been difficult and complex for therapists/clinicians in the clinical environment. Although there is a moderate correlation, hand grip strength may be used in clinical practice as a practical assessment tool to have an immediate insight into the global functional capacity in non-ambulatory DMD children.

摘要 杜氏肌营养不良症（Duchenne muscular dystrophy, DMD）是一种以肌纤维进行性丢失为特征的疾病，病变呈近端向远端逐渐进展。尽管已有少量研究针对非行走型DMD患者的握力展开探讨，但目前尚未有文献明确其与功能容量的关联。研究目的：本研究旨在明确非行走型杜氏肌营养不良症患儿的握力与各项功能评估指标之间的关联。研究方法：采用握力计对DMD患儿的握力进行评估。本研究使用土耳其语版Egen Klassifikation量表第2版（Egen Klassifikation Scale Version 2, EK2）评估患儿的整体功能容量，采用上肢功能表现量表（Performance of Upper Limb, PUL）评估上肢功能表现，并使用ABILHAND-Kids量表评估手部功能。研究结果：38名DMD患儿的平均年龄为12.02±1.99岁。患儿的利手握力显著高于非利手握力（p<0.05）。患儿的EK2评分为13.02±5.50，PUL评分为49.86±14.34，ABILHAND-Kids评分为26.81±7.59。研究发现握力与EK2评分呈显著相关（p<0.05）。研究结论：众所周知，在临床环境中，针对病情严重的DMD患儿进行功能能力与肌力评估对治疗师/临床医师而言颇具难度与复杂性。尽管握力与整体功能容量仅呈中等程度相关，但握力可作为一种实用的评估工具应用于临床实践，从而快速了解非行走型DMD患儿的整体功能容量。