Osteogenesis Imperfecta Program, Kennedy Krieger Institute, 707 North Broadway, Baltimore, MD 21205, USA.
Calcif Tissue Int. 2010 Aug;87(2):120-9. doi: 10.1007/s00223-010-9383-y. Epub 2010 Jun 11.
The effect of bisphosphonate treatment on bone mineral density (BMD) and fracture rates was assessed in adults with osteogenesis imperfecta (OI). This observational nonrandomized study included 90 OI adults treated with intravenous pamidronate (n = 28), oral alendronate (n = 10), or oral residronate (n = 17) or not treated (n = 35). There were 63 type I, 15 type III, and 12 type IV OI patients. BMD results were observed for up to 161 months and an average of 52 months of treatment. For type I and grouped type III/IV patients, treatment with pamidronate showed an increasing rate in L1-L4 BMD from baseline (0.006 [P = 0.03] and 0.016 [P < 0.001] gm/cm(2)/year, respectively); oral bisphosphonate treatment showed a significant increasing rate in L1-L4 BMD (0.004 gm/cm(2)/year [P = 0.047]) for type I patients. Pamidronate-treated type III/IV and oral bisphosphonate-treated type I patients showed significant increases in total-hip BMD (0.006 [P = 0.003] and 0.011 [P = 0.046] gm/cm(2)/year, respectively). Bisphosphonate effect on fracture rate was assessed for 5-year periods before and after treatment in 51 treated and 22 nontreated individuals matched for age at which bisphosphonate was first administered to the treated group. Bisphosphonate treatment did not decrease fracture rate in type I OI patients. Fracture rate decreased in type III/IV patients following pamidronate but not following oral bisphosphonate treatment. These results underscore a need to consider whether bisphosphonate treatment is appropriate for all adults with OI.
双磷酸盐治疗对成骨不全症（OI）成人的骨密度（BMD）和骨折率的影响进行了评估。这项观察性非随机研究包括 90 名接受静脉帕米膦酸盐（n=28）、口服阿仑膦酸盐（n=10）或口服利塞膦酸盐（n=17）或未治疗（n=35）的 OI 成人。患者中有 63 例 I 型、15 例 III 型和 12 例 IV 型 OI。BMD 结果观察时间最长达 161 个月，平均治疗时间为 52 个月。对于 I 型和 III/IV 型合并组患者，帕米膦酸盐治疗显示 L1-L4 BMD 从基线开始呈上升趋势（分别为 0.006[P=0.03]和 0.016[P<0.001]gm/cm(2)/年）；I 型患者口服双磷酸盐治疗显示 L1-L4 BMD 有显著的上升趋势（0.004 gm/cm(2)/年[P=0.047]）。帕米膦酸盐治疗的 III/IV 型和口服双磷酸盐治疗的 I 型患者总髋部 BMD 显著增加（分别为 0.006[P=0.003]和 0.011[P=0.046]gm/cm(2)/年）。在治疗前后的 5 年期间，对 51 名接受治疗的患者和 22 名未接受治疗的患者进行了骨折率评估，这些患者按接受治疗的患者首次使用双磷酸盐的年龄进行了匹配。双磷酸盐治疗并未降低 I 型 OI 患者的骨折率。帕米膦酸盐治疗后 III/IV 型患者的骨折率下降，但口服双磷酸盐治疗后无此现象。这些结果强调了需要考虑是否所有 OI 成人都需要接受双磷酸盐治疗。
