双膦酸盐治疗成骨不全研究进展

成骨不全是一组以骨骼脆性增加及胶原代谢紊乱为特征的全身性结缔组织疾病，主要由编码Ⅰ型胶原的基因发生突变所致，此病以骨脆性增加、骨关节进行性畸形、蓝巩膜、牙本质发育不全及听力下降为常见表现。目前治疗成骨不全的最理想的药物是双膦酸盐，本文复习国内外相关文献，就其治疗的最适剂量与时间、最佳给药途径、最佳药物选择以及药物与成骨不全类型和患者年龄的最适匹配等问题的研究进展作一综述。

Effect of Pak1 on embryonic vascular development and the related mechanisms

Osteogenesis imperfecta is a group of systemic connective tissue diseases characterized by increase in bone fragility and collagen metabolic disorder. It mainly caused by mutations in the genes that encode type I collagen. The common clinical features of OI are bone fragility, bone and joint progressive deformity, blue sclera, dentinogenesis imperfecta and hearing loss. Currently, the most promising drug is bisphosphonate. However, there is no unified standard in some problems, such as the optimal dose and duration of treatment, the best route of administration, the best drug choice, as well as drugs with OI type and age of patients with optimum match. This article reviews domestic and abroad recent research on these issues.