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. The aim of this study was to determine the associations between hand grip strength and functional measures in non-ambulatory children with DMD. 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. 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). 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.

Highlights

  • Duchenne muscular dystrophy (DMD) is a disease characterized by progressive loss of muscle fiber, gradually from proximal to distal

  • The results showed that the hand grip strength measurement may be a practical option to quickly estimate global functional capacity of children with DMD in the later phase of the disease in clinical settings, when there is not enough time for a detailed functional assessment by therapists and clinicians

  • It has been known that children with DMD produce a lower distal muscle force when compared with healthy peers, even in the early stage of their disease, and the weakness becomes more striking with age[8,11]

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Summary

Introduction

Duchenne muscular dystrophy (DMD) is a disease characterized by progressive loss of muscle fiber, gradually from proximal to distal. 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. As crianças com DMD foram avaliadas com a versão turca da Egen Klassifikation Scale Versão 2 (EK2) para capacidade funcional global, desempenho do membro superior (PUL) para desempenho funcional do membro superior e ABILHAND-Kids para a habilidade manual. Embora exista uma correlação moderada, a força de preensão manual pode ser usada na prática clínica como uma ferramenta de avaliação prática para obter imediatamente uma percepção da capacidade funcional global em crianças com DMD não ambulatoriais. Duchenne muscular dystrophy (DMD) is a neuromus- starting from the proximal muscles[1] It is diagnosed around cular disease characterized by the gradually loss of strength the age of four years when symptoms such as fatigue, frequent

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