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Shriners Hospitals for Children - Chicago
Transitional Care in Osteogenesis Imperfecta: Advances in biology, Technology, and Clinical Practice
Osteogenesis inperfecta (OI) is associated with bone fragility. Long bone fractures are a common occurrence in individuals with OI. Although there have been significant advances in understanding the genetic defects associated with OI, the mechanisms behind bone fragility in this patient population are not yet well understood. This fragility is believed to stem in part from characteristic bone mass deficiencies. Research further suggests that the material properties of the bone are also compromised in individuals with this disorder. There is currently on quantitative method available to assess bone fracture risk in individuals with OI. This chapter examines several critical elements needed to assess bone fracture risk through a unified biomechanical modeling approach. Finite element modeling (FEM) lies at the core of this approach with reliance upon material property and load data. The former stems from micro- and macrostructural scale characterization of bone material properties while the latter derives from assessment of mobility and other activities. As improved tools are developed for fracture risk assessment, clinicians will be afforded more effective methods to examine interventional effects and rehabilitative strategies in the short-and long-term with an ultimate goal of fracture reduction.