Bisphosphonate therapy for osteogenesis imperfecta.

摘要: Background Osteogenesis imperfecta is caused by a genetic defect resulting in an abnormal type I collagen bone matrix which typically results multiple fractures with little or no trauma. Bisphosphonates are used attempt to increase mineral density and reduce these people osteogenesis imperfecta. This update of previously published Cochrane Review. Objectives To assess the effectiveness safety bisphosphonates increasing density, reducing improving clinical function imperfecta. Search methods We searched Cystic Fibrosis Genetic Disorders Group Inborn Errors Metabolism Trials Register comprises references identified from comprehensive electronic database searches, handsearches journals conference proceedings. We additionally PubMed major proceedings. Date most recent search Group's Register: 28 April 2016. Selection criteria Randomised quasi-randomised controlled trials comparing placebo, treatment, comparator interventions all types imperfecta. Data collection analysis Two authors independently extracted data assessed risk bias included trials. Main results Fourteen (819 participants) were included. Overall, mainly at low bias, although selective reporting was issue several trials. Data for oral versus placebo could not be aggregated; statistically significant difference favouring fracture reduction number noted two No differences reported remaining three commented on incidence. Five spine density; found increased lumbar z scores least one time point studied. For intravenous aggregated showed participants fracture, ratio 0.56 (95% confidence interval 0.30 1.06). In trial trials, mean 9.96 -2.51 22.43). per cent change score favoured six 12 months. describing growth, pain, functional outcomes after bisphosphonate therapy, both, as compared incomplete among studies, but do show consistent improvements outcomes. Two studies different doses bisphosphonates. between when fractures, height length assessed. One primary zoledronic acid pamidronate. There outcome. However, odds relative benefit over pamidronate lumbosacral months. Authors' conclusions Bisphophonates commonly prescribed individuals Current evidence, albeit limited, demonstrates children adults this condition. These shown their ability density. It unclear whether treatment consistently decreases though report rate treatment. The here conclusively improve status (reduce pain; growth mobility) Given current widespread expected continued use, optimal method, duration therapy long-term require further investigation. addition, attention should given improvement quality life indicators.