AN INTERNATIONAL, MULTICENTER, RANDOMIZED, OPEN-LABEL, PARALLEL EFFICACY AND

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 这项试验的主要目的是评估与帕米磷酸钠治疗的患者相比，接受唑来膦酸治疗的严重成骨不全患者在12个月时腰椎骨密度相对于基线的百分比变化。如果证明唑来膦酸的疗效不逊于帕米磷酸钠(即12个月后骨密度与基线的百分比变化小于13%，低于帕米磷酸钠)，则证明该药的疗效。 二次疗效目标： -评估接受唑来膦酸(0.05 mg/kg)治疗的严重成骨不全患者与接受帕米磷酸钠(3.0 mg/kg)治疗的患者在12个月时的腰椎z评分 -与帕米磷酸钠相比，评估唑来膦酸对一年内发生的临床骨折数量的影响

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The primary objective of this trial is to assess the percentage change in lumbar spine bone mineral density at month 12 relative to baseline in zoledronic acid treated patients with severe osteogenesis imperfecta compared to pamidronate treated patients. The efficacy of zoledronic acid will be demonstrated if it is shown to be not inferior to pamidronate (i.e. the percentage change from baseline in bone mineral density after 12 months is less than 13% inferior to pamidronate). Secondary efficacy objectives: -To assess the z score of the lumbar spine at month 12 in zoledronic acid (0.05 mg/kg) treated patients with severe osteogenesis imperfecta compared to pamidronate (3.0 mg/ kg) treated patients -To assess the effect of zoledronic acid on the number of clinical fractures that occur over a one year period compared to pamidronate