RESUMEN
ABSTRACT Background: Muscle imaging methods such as ultrasound and magnetic resonance imaging have been used for many years to determine the dystrophic process in muscular dystrophies. However, the knowledge regarding muscle architecture in children at early-stage Duchenne muscular dystrophy (DMD) with different functional levels is limited. Objective: To explore the effect of functional level on muscle architectural properties in children with early stage DMD and the difference between DMD and typically developing (TD) peers. Methods: Thirty children with DMD (15 Grade 1 and 15 Grade 2 according to the Vignos Scale) and 5 TD peers were included. Ultrasound imaging was used to measure muscle thickness (MT), fascicle length (FL), and pennation angle (PA) of vastus lateralis (VL) and medial gastrocnemius (MG) muscles bilaterally. Results: The MT and FL values for VL, and MT, FL and PA values for MG muscles were higher in children with DMD compared with those of TD peers (p<0.05). The FL of VL, and MT and FL of GM muscles of children with DMD Grade 2 were higher than those of children with DMD Grade 1 (p<0.05). Conclusions: MT and FL are increased in children with DMD compared with TD peers. Additionally, muscle architecture seems to be affected even at the early stages of the disease.
RESUMO Antecedentes: Métodos de imagem muscular, como ultrassom e ressonância magnética, têm sido usados há muitos anos para determinar o processo distrófico em distrofias musculares. No entanto, o conhecimento a respeito da arquitetura muscular em crianças com distrofia muscular de Duchenne (DMD) em estágio inicial, com diferentes níveis funcionais, é limitado. Objetivo: Explorar o efeito do nível funcional nas propriedades arquitetônicas do músculo em crianças com DMD em estágio inicial e a diferença entre DMD e seus pares em desenvolvimento típico (DT). Métodos: Trinta crianças com DMD (15 Grau 1 e 15 Grau 2 de acordo com a Escala de Vignos) e cinco colegas DT foram incluídos. A ultrassonografia foi usada para medir a espessura muscular (EM), o comprimento do fascículo (FL) e o ângulo de penetração (PA) dos músculos vasto lateral (VL) e gastrocnêmio medial (MG) bilateralmente. Resultados: Os valores de EM e FL para VL e os valores de EM, FL e PA para músculos MG foram maiores em crianças com DMD em comparação com os de seus pares DT (p<0,05). O FL do VL e o EM e o FL dos músculos GM de crianças com DMD Grau 2 foram maiores do que aqueles de crianças com DMD Grau 1 (p<0,05). Conclusões: TM e FL estão aumentados em crianças com DMD em comparação com seus pares DT. Além disso, a arquitetura muscular parece ser afetada mesmo nos estágios iniciais da doença.
RESUMEN
BACKGROUND: Muscle imaging methods such as ultrasound and magnetic resonance imaging have been used for many years to determine the dystrophic process in muscular dystrophies. However, the knowledge regarding muscle architecture in children at early-stage Duchenne muscular dystrophy (DMD) with different functional levels is limited. OBJECTIVE: To explore the effect of functional level on muscle architectural properties in children with early stage DMD and the difference between DMD and typically developing (TD) peers. METHODS: Thirty children with DMD (15 Grade 1 and 15 Grade 2 according to the Vignos Scale) and 5 TD peers were included. Ultrasound imaging was used to measure muscle thickness (MT), fascicle length (FL), and pennation angle (PA) of vastus lateralis (VL) and medial gastrocnemius (MG) muscles bilaterally. RESULTS: The MT and FL values for VL, and MT, FL and PA values for MG muscles were higher in children with DMD compared with those of TD peers (p<0.05). The FL of VL, and MT and FL of GM muscles of children with DMD Grade 2 were higher than those of children with DMD Grade 1 (p<0.05). CONCLUSIONS: MT and FL are increased in children with DMD compared with TD peers. Additionally, muscle architecture seems to be affected even at the early stages of the disease.
Asunto(s)
Distrofia Muscular de Duchenne , Niño , Humanos , Extremidad Inferior/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Músculo Esquelético/diagnóstico por imagen , Distrofia Muscular de Duchenne/diagnóstico por imagenRESUMEN
BACKGROUND: Pulmonary and upper limbs function of children with Duchenne muscular dystrophy (DMD) are known to deteriorate throughout the disease process. However, there is a lack of information on the extent of impairments in the early stages of DMD when compared to healthy peers. OBJECTIVE: To investigate to what extent pulmonary and upper limbs function of children with early stage DMD are impaired. METHODS: Sixty-one children participated in the study: 31 with Grade 1 DMD (study group) according to the Brooke Upper and Lower Extremity Functional Classification Systems, and 30 age matched healthy peers (control group). Pulmonary function was determined with pulmonary function tests. The Performance of Upper Limb test was used to evaluate the upper limbs function. RESULTS: Study and control groups were homogenous in terms of physical characteristics (p>0.05). Pulmonary and upper limbs function of children with DMD were about 85% and 93% of healthy peers, respectively. CONCLUSION: This study provides evidence for deterioration of pulmonary and upper limbs function in children with early stage DMD. Better knowledge of deterioration rate over time may help therapists to better plan and update their plan of care.
Asunto(s)
Extremidad Inferior/fisiopatología , Distrofia Muscular de Duchenne/fisiopatología , Extremidad Superior/fisiopatología , Adolescente , Niño , Humanos , Fuerza Muscular/fisiología , Pruebas de Función Respiratoria/métodosRESUMEN
INTRODUCTION: 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.
Asunto(s)
Fuerza de la Mano/fisiología , Distrofia Muscular de Duchenne/fisiopatología , Rendimiento Físico Funcional , Adolescente , Niño , Femenino , Humanos , Masculino , Fuerza Muscular/fisiología , Dinamómetro de Fuerza Muscular , Valores de Referencia , Estadísticas no Paramétricas , Encuestas y Cuestionarios , Factores de Tiempo , Extremidad Superior/fisiopatologíaRESUMEN
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.
RESUMO A distrofia muscular de Duchenne (DMD) é uma doença caracterizada por perda progressiva da fibra muscular, gradualmente de proximal a distal. Embora poucos estudos tenham investigado a força de preensão manual em pacientes com DMD não ambulatoriais, foi observada uma falta de literatura para determinar suas relações com a capacidade funcional. Objetivo: O objetivo deste estudo foi determinar as associações entre força de preensão manual e medidas funcionais em crianças não ambulatoriais com DMD. Métodos: A força de preensão manual foi avaliada com dinamômetro em crianças com DMD. 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. Resultados: A idade média de trinta e oito crianças com DMD foi de 12,02 ± 1,99. A força de preensão manual dominante das crianças com DMD foi maior que a da mão não dominante (p < 0,05). A EK2 foi calculada em 13,02 ± 5,50, PUL em 49,86 ± 14,34 e ABILHAND-Kids em 26,81 ± 7,59. A força de preensão manual foi correlacionada com a EK2 (p < 0,05). Conclusões: Sabe-se que medir a capacidade funcional e força em crianças muito fracas com DMD tem sido difícil e complexo para terapeutas / clínicos em ambiente clínico. 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.