Format

Send to

Choose Destination

Muscle abnormalities in osteogenesis imperfecta.

Author information

1
Shriners Hospital for Children and McGill University, Montreal, Quebec, Canada.

Abstract

Osteogenesis imperfecta (OI) is mainly characterized by bone fragility but muscle abnormalities have been reported both in OI mouse models and in children with OI. Muscle mass is decreased in OI, even when short stature is taken into account. Dynamic muscle tests aiming at maximal eccentric force production reveal functional deficits that can not be explained by low muscle mass alone. However, it appears that diaphyseal bone mass is normally adapted to muscle force. At present the determinants of muscle mass and function in OI have not been clearly defined. Physiotherapy interventions and bisphosphonate treatment appear to have some effect on muscle function in OI. Interventions targeting muscle mass have shown encouraging results in OI animal models and are an interesting area for further research.

PMID:
28574406
PMCID:
PMC5492314
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for International Society of Musculoskeletal and Neuronal Interactions Icon for PubMed Central
Loading ...
Support Center