Bisphosphonates have been widely administered to children with OI based on observational trials. A randomized controlled trial of q3m intravenous pamidronate in children with types III and IV OI yielded positive vertebral changes in DXA and geometry after 1 year of treatment, but no further significant improvement during extended treatment. The treated group did not experience significantly decreased pain or long bone fractures or have increased motor function or muscle strength.Introduction: Bisphosphonates, antiresorptive drugs for osteoporosis, are widely administered to children with osteogenesis imperfecta (OI). Uncontrolled pamidronate trials in OI reported increased BMD, vertebral coronal area, and mobility, and decreased pain. We conducted a randomized controlled trial of pamidronate in children with types III and IV OI. Materials and Methods: This randomized trial included 18 children (4-13 years of age) with types III and IV OI. The first study year was controlled; 9 children received pamidronate (10 mg/m

Osteogenesis imperfecta is an autosomal dominant disorder of connective tissue, commonly known as “brittle bone disease.” The frequency of osteogenesis imperfecta identifiable at birth is estimated to be between 1 in 20,000 and 1 in 30,000. Osteogenesis imperfecta is characterized by bone fragility; affected individuals sustain fractures after very mild trauma. Because osteogenesis imperfecta is a generalized connective tissue disorder, other typical features include blue sclerae, dentinogenesis imperfecta, hearing loss, cardiopulmonary abnormalities, easy bruisability, excessive sweating, and loose joints. Current therapeutic management is discussed.

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