Fractures in Osteogenesis Imperfecta
Pathogenesis, Treatment, Rehabilitation and Prevention
Wouter Nijhuis (University Medical Center Utrecht)
Marjolein Verhoef (University Medical Center Utrecht)
Christiaan van Bergen (Amphia Hospital)
Harrie Weinans (University Medical Center Utrecht, TU Delft - Biomaterials & Tissue Biomechanics)
Ralph Sakkers (University Medical Center Utrecht)
More Info
expand_more
Other than for strictly personal use, it is not permitted to download, forward or distribute the text or part of it, without the consent of the author(s) and/or copyright holder(s), unless the work is under an open content license such as Creative Commons.
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.
help_outline