Results of previous studies suggest that children and adolescents with osteogenesis imperfecta (OI) type IV have muscle force deficits. However, muscle function remains to be objectively quantified in this population. This study aimed to assess upper and lower extremity muscle function in patients with OI type IV. It was carried out in the outpatient department of a pediatric orthopedic hospital; 27 individuals with OI type IV (7–21 years; 13 males), 27 age- and sex-matched individuals with OI type I, and 27 age- and sex-matched controls. Upper extremity muscle force was assessed with hydraulic hand dynamometry, and lower extremity muscle function (peak force per body weight and peak power per body mass) was measured by mechanography through five tests: multiple two-legged hopping, multiple one-legged hopping, single two-legged jump, chair-rise test, and heel-rise test. Upper-limb grip force was normal for patients with OI type IV when compared to height and sex reference data (average z-score = 0.17 ± 1.30; P = 0.88). Compared to age- and sex-matched controls, patients with OI type IV had approximately 30% lower-limb peak force and 50% peak power deficits (P values <0.05). At the lower-limb level, they had a 50% lower peak power than age- and sex-matched patients with OI type I (P < 0.05). Patients with OI type IV have normal upper-limb muscle force but a muscle function deficit at the lower-limb level. These results suggest that lower-limb muscle weakness may contribute to functional deficits in these individuals.
Osteogenesis imperfect Mechanography Maximum isometric grip force Upper-limb muscle force Lower-limb muscle function Muscle power
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This study was supported by the Shriners International. F.R. received salary support from the Chercheur-Boursier Clinicien program of the Fonds de Recherche du Québec—Santé. L.N.V. received postdoctoral fellowships from the Fonds de la Recherche du Québec—Santé and the MENTOR-RSBO (Network for Oral and Bone Health Research), supported by the Canadian Institutes of Health Research (MENTOR) and the Fonds de la Recherche du Québec—Santé (RSBO). The funding agencies were not involved in the design of the study, collection, analysis, and interpretation of data and in writing the manuscript.
Veilleux LN conceptualized and designed the study, carried out the analyses, drafted the initial manuscript. He is the guarantor. Darsaklis VB participated in data collection and interpretation. Montpetit K participated in data collection and interpretation. Glorieux FH contributed patient information and participated in the interpretation of data. Rauch F contributed patient information, conceptualized and designed the study and participated in the interpretation of data. All authors revised the paper critically for intellectual content and approved the final version. All authors agree to be accountable for the work and to ensure that any questions relating to the accuracy and integrity of the paper are investigated and properly resolved.
Compliance with Ethical Standards
Conflicts of interest
The authors declare that there are no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Human and Animal Rights and Informed Consent
All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000. Informed consent was obtained from study participants or the legal guardians.
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