Mobility in osteogenesis imperfecta: a multicenter North American study |
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| Institution: | 1. Orthopaedic Rehabilitation and Engineering Center, Marquette University, Milwaukee, WI, USA;2. Motion Analysis Laboratory, Shriners Hospitals for Children, Chicago, IL, USA;3. Baylor College of Medicine, Houston, TX, USA;4. Texas Children’s Hospital, Houston, TX, USA;5. Department of Bone and Osteogenesis Imperfecta, Kennedy Krieger Institute, Baltimore, MD, USA;6. Department of Medicine at Uniformed Services University of the Health Sciences, Bethesda, MD, USA;7. University of Wisconsin School of Medicine and Public Health, Madison, WI, USA;8. Pediatrics and Molecular and Medical Genetics, Oregon Health & Science University, Portland, OR, USA;9. Division of Medical Genetics, Alfred I du Pont Hospital for Children, Wilmington, DE, USA;10. Osteogenesis Imperfecta Foundation, Gaithersburg, MD, USA;11. College of Medicine, University of South Florida, Tampa, FL, USA;12. Departments of Medicine and Pathology, Division of Medical Genetics, University of Washington, Seattle, WA, USA;13. Shriner’s Hospital for Children and McGill University, Montreal, QC, Canada;14. Children’s Mercy Hospital, University of Missouri–Kansas CityKansas City, MO, USA;1. Orthopaedic Rehabilitation and Engineering Center, Marquette University, Milwaukee, WI, USA;2. Motion Analysis Laboratory, Shriners Hospitals for Children, Chicago, IL, USA;3. Baylor College of Medicine, Houston, TX, USA;4. Texas Children’s Hospital, Houston, TX, USA;5. Department of Bone and Osteogenesis Imperfecta, Kennedy Krieger Institute, Baltimore, MD, USA;6. Department of Medicine at Uniformed Services University of the Health Sciences, Bethesda, MD, USA;7. University of Wisconsin School of Medicine and Public Health, Madison, WI, USA;8. Pediatrics and Molecular and Medical Genetics, Oregon Health & Science University, Portland, OR, USA;9. Division of Medical Genetics, Alfred I du Pont Hospital for Children, Wilmington, DE, USA;10. Osteogenesis Imperfecta Foundation, Gaithersburg, MD, USA;11. College of Medicine, University of South Florida, Tampa, FL, USA;12. Departments of Medicine and Pathology, Division of Medical Genetics, University of Washington, Seattle, WA, USA;13. Shriner’s Hospital for Children and McGill University, Montreal, QC, Canada;14. Children’s Mercy Hospital, University of Missouri–Kansas CityKansas City, MO, USA |
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| Abstract: | PurposeOsteogenesis imperfecta (OI) is a genetic connective tissue disorder that causes bone fragility. Phenotypic severity influences ability to walk, however, little is known about ambulatory characteristics of individuals with OI, especially in more severe forms. The purpose of this work was to characterize mobility in OI using standard clinical assessment tools and determine if patient characteristics could be used to predict mobility outcomes.MethodsWe collected mobility data at five clinical sites to analyze the largest cohort of individuals with OI (n = 491) to date. Linear mixed models were developed to explore relationships among subject demographics and mobility metrics.ResultsResults showed minor limitations in the mild group while the more severe types showed more significant limitations in all mobility metrics analyzed. Height and weight were shown to be the most significant predictors of mobility. Relationships with mobility and bisphosphonates varied with OI type and type used (oral/IV).ConclusionThese results are significant to understanding mobility limitations of specific types of OI and beneficial when developing rehabilitation protocols for this population. It is important for physicians, patients, and caregivers to gain insight into severity and classification of the disease and the influence of disease-related characteristics on prognosis for mobility. |
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