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Muscle-Bone Interactions in Pediatric Bone Diseases
Louis-Nicolas Veilleux1, Frank Rauch2
1Shriners Hospital for Children, 1003 Boulevard Decarie, Montreal, QC, H4A 0A9, Canada. ln.veilleux@mcgill.ca.
Insights
Pediatric muscle disorders can cause skeletal issues, while bone diseases can impair muscle function. Understanding these interactions is key for new therapeutic targets in musculoskeletal health.
Area of Science:
- Pediatric musculoskeletal health
- Bone and muscle biology
Background:
- Muscle disorders originating in utero can cause congenital contractures.
- Pediatric muscle weakness (e.g., cerebral palsy, muscular dystrophy) is linked to reduced bone density and increased fractures.
Purpose of the Study:
- To review the skeletal effects of pediatric muscle disorders.
- To examine muscle impairment in pediatric bone disorders.
Main Methods:
- Literature review of skeletal effects in pediatric muscle disorders.
- Analysis of muscle impairment mechanisms in pediatric bone disorders.
Main Results:
- Pediatric muscle disorders are associated with long-bone shaft diameter reduction, low metaphyseal bone density, and higher fracture risk.
- Bone diseases can impact muscle function through general inactivity or specific molecular defects, like collagen issues in osteogenesis imperfecta.
Conclusions:
- Muscle-bone interactions are crucial for understanding musculoskeletal conditions.
- Investigating these interactions may reveal novel therapeutic strategies for pediatric bone and muscle disorders.
Purpose:
Here, we review the skeletal effects of pediatric muscle disorders as well as muscle impairment in pediatric bone disorders.
Recent Findings:
When starting in utero, muscle disorders can lead to congenital multiple contractures. Pediatric-onset muscle weakness such as cerebral palsy, Duchenne muscular dystrophy, spinal muscular atrophy, or spina bifida typically are associated with small diameter of long-bone shafts, low density of metaphyseal bone, and increased fracture incidence in the lower extremities, in particular, the distal femur. Primary bone diseases can affect muscles through generic mechanisms, such as decreased physical activity or in disease-specific ways. For example, the collagen defect underlying the bone fragility of osteogenesis imperfecta may also affect muscle force generation or transmission. Transforming growth factor beta released from bone in Camurati Engelman disease may decrease muscle function.
Future Directions:
Considering muscle-bone interactions does not only contribute to the understanding of musculoskeletal disorders but also can identify new targets for therapeutic interventions.
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