Louis-Nicolas Veilleux1, Annie Pouliot-Laforte2, Martin Lemay3, Moira S Cheung4, Francis H Glorieux4, Frank Rauch5. 1. Shriners 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. Electronic address: lnveilleux@shriners.mcgill.ca. 2. Shriners Hospital for Children, 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; Département de Kinanthropologie, Université du Québec à Montréal, 141 Avenue du Président Kennedy Complexe des Sciences Pierre-Dansereau, Montréal, Québec H2X 1Y4, Canada. 3. Centre de Réadaptation Marie-Enfant, Research Center, Hôpital Sainte-Justine, 5200 Bélanger Street East, Montréal, Québec H1T 1C9, Canada; Département de Kinanthropologie, Université du Québec à Montréal, 141 Avenue du Président Kennedy Complexe des Sciences Pierre-Dansereau, Montréal, Québec H2X 1Y4, Canada. 4. Shriners Hospital for Children, 1529 Cedar Avenue, Montréal, Québec H3G 1A6, Canada; Department of Pediatrics, McGill University, 1529 Cedar Avenue, Montréal, Québec H3G 1A6, Canada. 5. Shriners 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.
Abstract
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.
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.
Authors: Youngjae Jeong; Salah A Daghlas; Alp S Kahveci; Daniel Salamango; Bettina A Gentry; Marybeth Brown; R Scott Rector; R Scott Pearsall; Charlotte L Phillips Journal: Muscle Nerve Date: 2017-06-15 Impact factor: 3.217
Authors: A Gomez-Bruton; A Gonzalez-Aguero; A Matute-Llorente; G Lozano-Berges; A Gomez-Cabello; L A Moreno; J A Casajus; G Vicente-Rodríguez Journal: Osteoporos Int Date: 2019-02-07 Impact factor: 4.507