Related Experiment Video
Updated: Apr 12, 2026
Subject-specific Musculoskeletal Model for Studying Bone Strain During Dynamic Motion
Published on: April 11, 2018
The functional muscle-bone unit in patients with osteogenesis imperfecta type I
Louis-Nicolas Veilleux1, Annie Pouliot-Laforte2, Martin Lemay3
1Shriners Hospital for Children, 1529 Cedar Avenue, Montréal, Québec H3G 1A6, Canada; Department of Pediatrics, McGill University, 1529 Cedar Avenue, Montréal, Québec H3G 1A6, Canada; Centre de Réadaptation Marie-Enfant, Research Center, Hôpital Sainte-Justine, 5200 Bélanger Street East, Montréal, Québec H1T 1C9, Canada.
Context:
Osteogenesis imperfecta (OI) type I is a heritable bone fragility disorder that is caused by mutations affecting collagen type I. We recently showed that patients with OI type I frequently have muscle weakness. As muscle force and bone mass are usually closely related, we hypothesized that muscle weakness in OI type I could contribute to increase bone mass deficit in the lower extremities.
Objective:
To assess the muscle-bone relationship in the lower extremities of children and adolescents with OI type I.
Setting:
The study was carried out in the outpatients department of a pediatric orthopedic hospital. Patients and other participants Thirty children and adolescents with OI type I (20 females; mean age [SD]: 11.2 years [3.9]) were compared with 30 healthy age- and sex-matched controls (mean age [SD]: 11.1 years [4.5]).
Main Outcome Measures:
Tibia bone mineral content (BMC; mg/mm) was measured by peripheral quantitative computed tomography to estimate bone strength at the 4% and 14% sites. Lower extremity peak force (kN) was measured by mechanography using the multiple two-legged hopping test.
Results:
Compared with age- and sex-matched controls, patients with OI type I had 17% lower peak force (1.3 kN vs. 1.7 kN; p=0.002) as well as a 22% lower BMC (128 mg/mm vs. 165 mg/mm; p<0.001). Stepwise regression analysis showed that muscle force and tibia length were positively related to bone strength (r(2)=0.90, p<0.001) whereas there was no effect of the disease status (OI vs. control).
Conclusions:
These results suggest that the muscle-bone relationship is similar between children and adolescents with OI type I and healthy age and sex-matched controls. It also suggests that muscle weakness may contribute to decreased bone strength in individuals with OI type I.
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