Fractures in Osteogenesis Imperfecta
Pathogenesis, Treatment, Rehabilitation and Prevention
Wouter Nijhuis ( University Medical Centre Utrecht)
Marjolein Verhoef ( University Medical Centre Utrecht)
Christiaan van Bergen (Amphia Hospital)
Harrie Weinans (TU Delft - Biomaterials & Tissue Biomechanics, University Medical Centre Utrecht)
Ralph Sakkers ( University Medical Centre Utrecht)
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Abstract
Fractures in patients with osteogenesis imperfecta (OI) are caused by a decreased strength of bone due to a decreased quality and quantity of bone matrix and architecture. Mutations in the collagen type 1 encoding genes cause the altered formation of collagen type I, one of the principal building blocks of bone tissue. Due to the complexity of the disease and the high variation of the clinical problems between patients, treatment for these patients should be individually tailored. In general, short immobilization periods with flexible casting material, use of intramedullary implants, and simultaneous deformity correction are preferred. Multidisciplinary care with a broad view of the support needed for the patient and his/her living environment is necessary for the optimal rehabilitation of these patients. Increasing bone strength with exercise, medication, and sometimes alignment surgery is generally indicated to prevent fractures.
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