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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Pulmonary and upper limbs function in children with early stage Duchenne muscular dystrophy compared to their healthy
Numan Bulut1, Güllü Aydın1, İpek Alemdaroğlu-Gürbüz1
1Faculty of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Turkey.
Insights
Children with early-stage Duchenne muscular dystrophy (DMD) show significant impairments in pulmonary and upper limb function compared to healthy peers. This early functional decline highlights the need for timely therapeutic interventions in pediatric DMD care.
Area of Science:
- Pediatric Neurology
- Rehabilitation Medicine
- Clinical Physiology
Background:
- Duchenne muscular dystrophy (DMD) is a progressive genetic disorder affecting muscle strength.
- Pulmonary and upper limb functions are known to decline in DMD patients over time.
- Limited data exists on the extent of functional impairment in early-stage pediatric DMD.
Purpose of the Study:
- To quantify pulmonary and upper limb functional deficits in children with early-stage DMD.
- To compare functional status between children with early DMD and healthy controls.
- To establish a baseline for monitoring disease progression.
Main Methods:
- A cohort of 31 children with Grade 1 DMD and 30 age-matched healthy controls was recruited.
- Pulmonary function was assessed using standardized pulmonary function tests.
- Upper limb function was evaluated using the Performance of Upper Limb (PUL) test.
Main Results:
- The study groups were comparable in physical characteristics.
- Children with early DMD exhibited approximately 85% of the pulmonary function of healthy peers.
- Upper limb function in children with DMD was found to be 93% of that in healthy controls.
Conclusions:
- Children in the early stages of DMD demonstrate measurable reductions in pulmonary and upper limb function.
- Understanding the rate of functional deterioration is crucial for optimizing physical therapy and care plans.
- Early identification of deficits can inform proactive management strategies for pediatric DMD patients.
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.
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