Muscle abnormalities in osteogenesis imperfecta

Muscle abnormalities in osteogenesis imperfecta

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 pr...

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Veröffentlicht in:Journal of musculoskeletal & neuronal interactions 2017-06, Vol.17 (2), p.1-7
Hauptverfasser: Veilleux, L N, Trejo, P, Rauch, F
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Sprache:eng
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Animals
Humans
Muscle, Skeletal - pathology
Muscular Diseases - etiology
Muscular Diseases - pathology
Original
Osteogenesis Imperfecta - complications
Osteogenesis Imperfecta - pathology
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Trejo, P
Rauch, F
description 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.
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subjects Animals
Humans
Muscle, Skeletal - pathology
Muscular Diseases - etiology
Muscular Diseases - pathology
Original
Osteogenesis Imperfecta - complications
Osteogenesis Imperfecta - pathology
title Muscle abnormalities in osteogenesis imperfecta
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