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Osteogenesis imperfecta is a genetic disorder of extracellular matrix, characterized with decreased bone mass, and increased bone fragility. Sodium bisphosphonate treatment leads to a quick increase in bone mineral density with resultant reduced bone pain, fracture rate, and immobility in osteogenesis imperfecta. In this study we evaluated the data of 31 children, of 16 female, aged 8.80±5.04 years, with osteogenesis imperfecta who were treated with cyclic bisphosphonates. Nine patients were diagnosed as type I, eleven were type III, and eleven were type IV. All patients received bisphosphonate at a mean dose of 12.2 mg/kg/year, once every two-four months. Mean duration of this treatment is 3.43±1.91 years (9 months-6 years). Before treatment number of fracture were minimum 15 in eight patients. The number of fracture were decreased to 1.70±1.90 from 7.45±5.33 with bisphosphonate treatment. Although ten patients with osteogenesis imperfecta type I and IV had no fracture after treatment, we did not observe a decrease in the number of fractures in two patients. Bone mineral density Z-scores increased in the first three years of bisphosp-honates treatment, however this increment was stopped and decreased slow rate in the following years. Ambulation scores and muscle were decreased in all patients. We did not observe any beneficial effects of bisphosphonates on the height SDS. In conclusions our data demonstrated that bisphosphonates have dramatically beneficial effect on bone mineral density, rate of fracture, bone pain, and mobility, which leads a higher quality of life in children with osteogenesis imperfecta. |
