Search

Duchenne Muscular Dystrophy (DMD) is a devastating neuromuscular disease of childhood which is characterized by progressive muscle weakness that limits the functional activities of daily life in course of time and result in decreased quality of life. The benefits of increasing the physical activity (PA) level and participation on general health status in children with DMD was previously mentioned in literature. Parents have been reported to have an important impact on their child's beliefs, attitudes, and behaviors in many dimensions of development throughout their lives. Studies showed that there was a significant correlation between parental impact and child's PA level and participation in healthy children. The aim of this study was to investigate the family impact on PA level, interest in PA, and participation level of their school-age children with Duchenne Muscular Dystrophy (DMD). This cross-sectional, observational study included a total of 30 ambulatory children between 8 and 18 years of age (mean: 8.70 ± 0.84). Physical Activity Questionnaire for Older Children (PAQ-C) and pedometer were used to assess PA level of children while International Physical Activity Questionnaire-Short Form (IPAQ-SF) was used to determine the PA level of parents. Children's Attraction to Physical Activity (CAPA), Pediatric Outcome Data Collection Instrument (PODCI), and Children's Physical Activity Correlates (CPAC) questionnaires were also used to evaluate interest in PA, participation level, and family impact on PA level of children, respectively. Low-to-moderate correlations were determined between family impact sub-score of CPAC and PAQ-C, CAPA and PODCI while moderate correlation was found between PAQ-C and parents' IPAQ-SF score (p < 0.05). This study revealed the importance of parents' support and encouragement as well as their own PA habit in shaping their child's behaviors and attitudes towards PA in an early-stage, school-aged DMD population. In order to achieve the goal of increasing PA level and participation of children with DMD, a family-centered rehabilitation approach may be more effective. Clinical Trial Registration Number NCT05209087; Date: 25.01.2022. Highlights: School-age children with DMD had moderate PA and participation levels as well as moderate interest in PA. Parents, especially the primary caregiver, had a significant impact on PA level and participation of DMD children. The PA levels of the parents had an impact on the PA level of children with DMD. [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate validity, reliability and feasibility of the Four Square Step Test (FSST) in children with Duchenne Muscular Dystrophy (DMD). The study included 44 children with DMD aged 5–12 years. The functional level of the children was determined by using the Brooke Lower Extremity Functional Classification (BLEFC). The concurrent validity of the FSST, a dynamic balance assessment, was investigated by analyzing the association between FSST and Timed Up and Go Test (TUG), North Star Ambulatory Assessment (NSAA) and Six-Minute Walk Test (6MWT) distance. The intra- and inter-rater reliability of the FSST was determined by using the Intra Class Correlation Coefficient (ICC) while the feasibility of the test was determined by calculating the percentage of children who successfully completed the test in accordance with the test instructions. The FSST score of the children (mean age 9.25 ± 2.14 years) was 11.91 ± 3.43 and the BLEFC level ranged from Level 1 (65.9%) to Level 3 (9.1%). FSST had positive, moderate relation with BLEFC (r = 0.447; p =.002); positive, strong relation with TUG (rs = 0.623), and negative, moderate correlations with NSAA (rs = −0.529) and 6MWT distance (rs = −0.592) (p <.001). Intra-rater (ICC: 0.965) and inter-rater (ICC: 0.991) reliability was "excellent," and the test was feasible with successful performance of 88% children. The FSST is a valid, reliable and feasible instrument to assess dynamic balance as well as having the ability to reflect falling risk and different functional levels of children with DMD. [ABSTRACT FROM AUTHOR]

Objective: To investigate the effects of lower limb flexibility on the functional performance of children with Duchenne muscular dystrophy. Methods: Thirty children, whose functional levels were at 1 or 2 according to the Brooke Lower Extremity Functional Classification Scale, were included in this study. The flexibilities of the hamstrings, hip flexors, tensor fascia latae, and gastrocnemius muscles were evaluated in the children's dominant lower limbs. The children's functional performance was assessed using 6-minute walk tests and timed performance tests. The correlations between the flexibilities of the lower limb muscles and the performance tests were examined. Results: The flexibilities of the lower extremity muscles were found to be correlated to the 6-minute walk tests and the timed performance tests. The flexibility of the hamstrings (r = −.825), the gastrocnemius muscles (r =.545), the hip flexors (r =.481), and the tensor fascia latae (r =.445) were found to be correlated with functional performance as measured by the 6-minute walk tests (P <.05). Discussion: The results of the current study indicate that the flexibility of the lower limbs has an effect on functional performance in the early stages of Duchenne muscular dystrophy. More research is needed to determine the functional effects of flexibility on performance by adding long-term flexibility exercises to the physiotherapy programs of children with Duchenne muscular dystrophy. [ABSTRACT FROM AUTHOR]

Objective: To explore the effects of aerobic training adding to home-based exercise program on motor function and muscle architectural properties in children with Duchenne muscular dystrophy. Design: This is a prospective randomized controlled study. Setting: Pediatric neuromuscular clinic in a tertiary care center. Subjects: Children with Duchenne muscular dystrophy. Interventions: Children were randomly divided into two groups whereby 12-weeks aerobic training was additionally given in treatment group in contrast to the control group which received only home-based exercise program. Main Measures: Motor Function Measure and Six Minute Walk Test were used for clinical evaluation, and muscle architectural properties (thickness, pennation angle and fascicle length) were measured by ultrasound imaging. Both groups were assessed at baseline and after 12-weeks of training. Results: Median age of children was 7.9 years in the treatment group and 8.6 years in the control group (p > 0.05). Significant improvements were obtained for Motor Function Measure and Six Minute Walk Test from baseline to 12-weeks in the treatment group; Motor Function Measure total score changed from 83.2 (6.1) to 86.9 (4.0) vs. 82.3 (10.2) to 80.4 (9.4) points in the control group (p = 0.006) ; 6 Minute Walk Test distance changed from 395.3 (46.6) to 413.0 (52.3) vs. 421.7 (64.4) to 393.8 (68.2) meters in the control group (p < 0.001). However, muscle architectural parameters did not change during study period (p > 0.05). Conclusion: Aerobic training may be of additional value in improving motor function and performance with no remarkable effect on muscle architectural properties. [ABSTRACT FROM AUTHOR]

Introduction: The aim of this study was to investigate whether trunk control is associated with the upper limb function of children with Duchenne Muscular Dystrophy (DMD). Methods: The children included in the study were divided into two groups according to the Trunk Control Measurement Scale total scores. Twenty-five children whose trunk control levels were lower than 30.5 points were included in Group 1 while 31 children who had good levels of trunk control between 30.5 and 58 points were included in Group 2. General functional levels, muscular strength, and function of the upper limb were assessed and compared between the groups. Results: According to the results of the assessments, children in Group 2 were found to have better upper limb function and muscular strength than Group 1 (p <.001). Conclusions: The current study demonstrated better upper limb functional performance of children with DMD that had good-level trunk control thus suggesting the possible association between trunk stability and upper limb function. The results reveal the need to improve and protect trunk muscle strength and stability as well as upper limb strength, and to optimize trunk stability during upper limb movement in rehabilitation programs. [ABSTRACT FROM AUTHOR]

• Pulmonary and upper limbs function start deteriorating in the early stages of Duchenne muscular dystrophy (DMD). • The decline in pulmonary function is more significant than the decline in upper limbs function in DMD. • Functional status can be followed by comparing healthy peers in each stage of DMD. 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. To investigate to what extent pulmonary and upper limbs function of children with early stage DMD are impaired. 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. 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. 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. [ABSTRACT FROM AUTHOR]