St. Vincent Hospital, Medical Department II, Academic Teaching Hospital of Medical University Vienna, The VINFORCE Study Group, Stumpergasse 13, 1060, Vienna, Austria.
Skeletal Radiol. 2013 Feb;42(2):187-94. doi: 10.1007/s00256-012-1512-4. Epub 2012 Sep 7.
Osteogenesis imperfecta (OI) is an inherited disorder characterized by increased bone fragility with recurrent fractures that leads to skeletal deformities in severe cases. Consequently, in most OI patients, the hip is the only reliable measuring site for estimating future fracture risk. The aim of the study was to assess the applicability of hip structure analysis (HSA) by DXA in adult patients with osteogenesis imperfecta.
We evaluated bone mineral density (BMD) and hip structure analysis (HSA) by DXA, including cross-sectional area (CSA), cross-sectional moment of inertia (CSMI) and femoral strength index (FSI) in 30 adult patients with different types of OI and 30 age-matched healthy controls (CO). The OI total group (OI-tot) was divided into two subgroups: the mild OI I group (OI-I) and the more severe OI III and IV group (OI-III-IV).
The mean neck BMD of OI-I and OI-III-IV were significantly lower compared to CO (-15.9 %, p < 0.005 and -37.5 %, p < 0.001 respectively). Similar results were observed at trochanter and total hip. CSA and the CSMI value were significantly lower for OI-I (-23.2 %, p < 0.001) and OI-III-IV (-45.9 %, p < 0.001) in comparison to CO. In addition, significant differences were found between the mild OI-I and the severe OI-III-IV group (-29.6 %, p < 0.05). FSI was significantly decreased in the OI-III-IV (25.7 %, p < 0.05) in comparison to the CO. Furthermore, significant correlations between BMD and HSA and between HSA and height and weight were found in osteogenesis imperfecta and controls.
BMD measurement in osteogenesis imperfecta patients is very critical. The combination of BMD and geometric structural measurements at the hip in osteogenesis imperfecta patients may represent an additional helpful means in estimating bone strength and fracture risk.
成骨不全症（OI）是一种遗传性疾病，其特征是骨脆性增加，反复骨折，导致严重病例骨骼畸形。因此，在大多数 OI 患者中，髋关节是唯一可靠的测量部位，用于估计未来的骨折风险。本研究旨在评估 DXA 髋关节结构分析（HSA）在成骨不全症成年患者中的适用性。
我们评估了 30 名不同类型 OI 成年患者和 30 名年龄匹配的健康对照组（CO）的骨密度（BMD）和髋关节结构分析（HSA），包括横截面积（CSA）、横截面惯性矩（CSMI）和股骨强度指数（FSI）。OI 总组（OI-tot）分为两个亚组：轻度 OI I 组（OI-I）和更严重的 OI III 和 IV 组（OI-III-IV）。
OI-I 和 OI-III-IV 的颈 BMD 平均值明显低于 CO（分别为-15.9%，p<0.005 和-37.5%，p<0.001）。在转子间区和全髋关节也观察到类似的结果。CSA 和 CSMI 值在 OI-I（-23.2%，p<0.001）和 OI-III-IV（-45.9%，p<0.001）与 CO 相比显著降低。此外，在轻度 OI-I 和严重 OI-III-IV 组之间也发现了显著差异（-29.6%，p<0.05）。与 CO 相比，OI-III-IV 的 FSI 明显降低（25.7%，p<0.05）。此外，在成骨不全症和对照组中，均发现 BMD 与 HSA 之间以及 HSA 与身高和体重之间存在显著相关性。
在成骨不全症患者中，BMD 测量非常关键。在成骨不全症患者中，BMD 与髋关节的几何结构测量相结合，可能代表了一种额外的有用手段，用于估计骨强度和骨折风险。
