Osteogenesis imperfecta is an inherited disorder of type I collagen characterized by low bone mass, bone fragility, and fractures with minimal or no trauma. Treatment for the disorder is largely supportive, but recently bisphosphonate therapy has been employed in an attempt to increase bone mineral density and potentially reduce fracture incidence in affected individuals. We have included eight randomised trials, some placebo-controlled, some with a cross-over trial design, or both, in our review. Four trials report decreased fractures in some instances in those treated with bisphosphonates; however no significant difference was found in three other trials. Another trial demonstrated decreased vertebral and upper extremity but not lower extremity fractures. Each trial independently demonstrates significant improvements in bone mineral density after treatment with oral or intravenous bisphosphonates. Fracture incidence, bone pain, growth and quality of life indicators influenced by treatment with bisphosphonates warrant further investigation. Further investigation is needed to establish whether the improvements in bone mineral density translate into fracture reduction and functional improvements and to determine the long-term effectiveness and safety of their use.
Bisphosphonate therapy for osteogenesis imperfecta
July 8, 2009
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