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Updated: Dec 7, 2025
Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Prevalence of smooth muscle dysfunction among children with Duchenne muscular dystrophy
Ranjith K Manokaran1,2, Sandeep Aggarwala3, Rakesh Kumar4
1Center of Excellence and Advanced Research on Childhood Neurodevelopmental Disorders, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Insights
Smooth muscle dysfunction is common in Duchenne muscular dystrophy (DMD). This study found significant prevalence in vascular, gastrointestinal, and bladder systems, impacting patient care.
Area of Science:
- Neurology
- Pediatrics
- Gastroenterology
- Urology
Background:
- Smooth muscle dysfunction is an understudied aspect of Duchenne muscular dystrophy (DMD).
- DMD primarily affects skeletal muscles, but smooth muscle involvement has significant clinical implications.
- Understanding smooth muscle function in DMD is crucial for comprehensive patient management.
Purpose of the Study:
- To estimate the prevalence of smooth muscle dysfunction in children with DMD.
- To investigate vascular, upper gastrointestinal, and bladder smooth muscle function.
- To highlight the need for integrating smooth muscle assessments into routine DMD care.
Main Methods:
- Cross-sectional study involving 99 children with DMD.
- Utilized validated questionnaires: Pediatric Bleeding Questionnaire, Pediatric Gastroesophageal Symptom Questionnaire, Dysfunctional Voiding Symptom Score.
- Included objective measures: bleeding time, gastroesophageal reflux scintigraphy, uroflowmetry.
Main Results:
- Overall smooth muscle dysfunction prevalence was 27.2% across vascular, gastrointestinal, and bladder systems.
- Prolonged mean bleeding time (117.5 seconds), 21% prevalence of gastroesophageal reflux.
- 18.2% showed bladder capacity issues, and 9.7% had abnormal uroflowmetry findings.
Conclusions:
- Smooth muscle dysfunction is a prevalent issue in children with DMD.
- Findings underscore the importance of evaluating vascular, GI, and bladder function in DMD.
- Recommendations for incorporating smooth muscle assessments into standard DMD clinical practice.
Abstract:
Smooth muscle dysfunction in Duchenne muscular dystrophy (DMD) has been rarely studied. A cross-sectional study was conducted to estimate the prevalence of smooth muscle dysfunction (vascular, upper gastrointestinal, and bladder smooth muscle) in children with DMD using questionnaires (Pediatric Bleeding Questionnaire, Pediatric Gastroesophageal Symptom Questionnaire, and Dysfunctional Voiding Symptom Score). Investigations included bleeding time estimation, nuclear scintigraphy for gastroesophageal reflux, and uroflowmetry for urodynamic abnormalities. Ninety-nine subjects were included in the study. The prevalence of vascular, upper gastrointestinal, and bladder smooth muscle dysfunction was 27.2%. Mean bleeding time was prolonged by 117.5 seconds. The prevalence of gastroesophageal reflux was 21%. Voided volume/estimated bladder capacity over 15% and abnormal flow curves on uroflowmetry were seen in 18.2% and 9.7% of the subjects, respectively. Our study highlights the need for addressing issues related to smooth muscle dysfunction in the routine clinical care of patients with DMD.
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