Kurtz D, Morrish K, Shapiro J
Calcif Tissue Int. 1985 Jan;37(1):14-8. doi: 10.1007/BF02557672.
Quantitative computed tomography of the lumbar spine was carried out in 28 patients with osteogenesis imperfecta (OI) in order to measure vertebral trabecular bone mineral concentration (BMC). The patients ranged in age from 6-73 years, and included 3 of the 4 major clinical subtypes of the disease. The findings underscore the heterogeneity of osteogenesis imperfecta even among family members with the same disease type. In addition, cross-sectional analysis of Type I OI patients suggests that BMC during young adulthood averages about 70% of normal, and subsequently falls more rapidly than in normal patients. BMC tends to be lower in the more severe forms of OI. Decreased BMC was not found in a few otherwise normal relatives with scoliosis or joint laxity.
对28例成骨不全症(OI)患者进行了腰椎定量计算机断层扫描,以测量椎体小梁骨矿物质浓度(BMC)。患者年龄在6至73岁之间,包括该疾病4种主要临床亚型中的3种。研究结果强调了成骨不全症的异质性,即使在患有相同疾病类型的家庭成员中也是如此。此外,对I型OI患者的横断面分析表明,年轻成年期的BMC平均约为正常水平的70%,随后下降速度比正常患者更快。在更严重的OI形式中,BMC往往较低。在一些患有脊柱侧弯或关节松弛但其他方面正常的亲属中未发现BMC降低。