双膦酸盐治疗成骨不全症的研究进展  

Bisphosphonate in the treatment of osteogenesis imperfecta

作　　者：韩兰稳[1] 李梅[1] 

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院内分泌科,卫生部内分泌重点实验室,100730

出　　处：《国际内分泌代谢杂志》2011年第3期158-160,164,共4页International Journal of Endocrinology and Metabolism

基　　金：国家科技支撑计划项目（2008BAI58B02）;中华医学会骨质疏松和骨矿盐疾病分会骨质疏松科研项目（2008-2011）

摘　　要：成骨不全症是以骨骼脆性增加和骨量减少为特征的一种遗传性疾病,可导致患者反复发生骨折,致畸率和致残率较高.该疾患主要由重要的骨基质蛋白Ⅰ型胶原编码基因突变所致.以往成骨不全症缺乏有效治疗药物,近年来临床研究显示双膦酸盐类药物能够有效抑制破骨细胞介导的骨吸收,增加成骨不全症患者的骨密度,降低骨折发生率,缓解骨痛,提高患者生活质量.Osteogenesis imperfecta（OI） is a heritable disease characterized by bone fragility and reduced bone mass,which can lead to multiple fractures in the patients and result in serious skeletal deformity.Genetic researches have demonstrated that OI mainly resulted from mutations of the genes encoding collagen type Ⅰ.No effective drugs for OI is avmlable previously.However,current clinical studies indicate that bisphosphonate can improve life quality of patients with OI through inhibiting osteoclast-induced bone resorption,increasing bone mineral density,decreasing bone fracture rates,and alleviating pain.

关 键 词：双膦酸盐 成骨不全症 治疗 

分 类 号：R580[医药卫生—内分泌]