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Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
Published on: July 29, 2016
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Body composition in patients with congenital myotonic dystrophy
Delia Ceballos-Sáenz1, Eugenio Zapata-Aldana2, Stephanie Singeris1
1Paediatric Neurology Department, Children's Hospital, London Health Science Centre, 800 Commissioners Road East, PO Box 5012, London, Ontario, Canada, N6A 5W9.
Muscle & Nerve
|May 11, 2019
Summary
Older children with congenital myotonic dystrophy (CDM) show muscle mass closer to healthy peers. This finding aligns with the condition
Area of Science:
- Neuromuscular Disorders
- Pediatric Health
- Body Composition Analysis
Background:
- Congenital myotonic dystrophy (CDM) is a rare neuromuscular disorder.
- Characterized by severe hypotonia and muscle weakness at birth, symptoms often improve with age.
- Understanding lean body and bone mass is crucial for research and clinical care in CDM patients.
Purpose of the Study:
- To determine if older children with CDM have comparable muscle mass to healthy controls.
- To investigate the relationship between age and lean body mass in children with CDM.
Main Methods:
- Thirty-five children with CDM, aged 3-13 years, were enrolled.
- Lean body mass (LBM) and bone mineral content were analyzed.
- The mechanostat framework was used to calculate sex, age, and height-specific z-scores.
Main Results:
- All CDM patients exhibited low LBM z-scores.
- A positive correlation was found between higher LBM z-score and age (r = 0.45, P = 0.006).
- Older children with CDM demonstrated LBM closer to normal ranges.
Conclusions:
- Unlike other neuromuscular conditions, older children with CDM possess muscle mass approaching that of age-matched controls.
- Findings support the observed clinical improvement in strength during childhood for individuals with CDM.
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