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We evaluated the efficacy of a monthly infusion of pamidronate on the frequency of fractures, biochemical effects, and bone mineral density in children with osteogenesis imperfecta. Eleven patients from 0.9 to 13.8 yr of age were included in this study. The patients were administered pamidronate intravenously (30 mg/m2) over a 4-hr period monthly for a period ranging from 6 to 37 months. Height and weight Z-scores did not change significantly. The frequency of fractures was decreased from 2.3±1.01 times per year before treatment to 0.6±0.69 times per year during treatment. There were no long-term changes in biochemical markers during pamidronate therapy. The mean bone mineral density of the spine and femur increased significantly. Monthly intravenous pamidronate therapy decreased frequencies of fracture and increased bone mineral density without significant adverse events in Korean patients with osteogenesis imperfecta.