Department of Pathophysiology and Allergy Research, Center for Pathophysiology, Infectiology and Immunology, Medical University of Vienna, 1090, Vienna, Austria.
Department of Physiology, School of Pharmacy, and Institute of Nutrition and Food Technology, University of Granada, Granada, Spain.
Calcif Tissue Int. 2019 Jul;105(1):15-25. doi: 10.1007/s00223-019-00539-8. Epub 2019 Mar 8.
Although Paget's disease of bone (PDB) is the second most common metabolic bone disease, there is only limited information about the microarchitecture of affected bones. Therefore, the aim of this study was to determine cortical and trabecular bone properties in clinically relevant locations by microcomputed tomography (µCT). Ten femora and ten tibiae affected by Paget's disease taken from the Natural History Museum Vienna were compared to 13 femora and 10 tibiae of non-affected body donors. Digitization of the cortical and trabecular bone microarchitecture was performed with an X-ray-based µCT scanner. Additionally, semi-quantitative gradings of trabecular and cortical architectural parameters of the femora and the tibiae were generated. Microcomputed tomography images showed changes in the thickness of cortices, cortical porosity, and trabecularization of cortical structures. Moreover, severe disorganization of trabecular structures, trabecular defects, and thickening of (remaining) trabeculae were detected. Numerical cortical analyses showed lower total bone volume (BV) and lower BV in the outer region (66-100%) (- 36%, p = 0.004, and - 50%, p < 0.001, respectively), lower total volume (TV) in the outer region (66-100%) (- 42%, p < 0.001), lower total bone volume fraction (BV/TV) and BV/TV in the outer region (66-100%) (- 23%, and - 12%, p < 0.001, respectively), higher BV and TV in the middle region (33-66%) and higher BV/TV in the inner region (0-33%) (123%, p = 0.011, 147%, p = 0.010, and 33%, p = 0.025, respectively) in Pagetic compared to non-affected bones. Trabecular analyses showed higher BV/TV (96%, p = 0.008) and Tb.Th (43%, p = 0.004) in Pagetic compared to non-affected bones. There is a major and consistent structural alteration of PDB at cortical and trabecular sites in weight-bearing long bones. Our findings are relevant for the differential diagnosis of PDB and for the pathogenesis of associated complications, since the disorder produces abnormalities in the structure that might lead to bone fragility.
尽管佩吉特氏病（Paget's disease of bone，PDB）是第二常见的代谢性骨病，但有关受影响骨骼的微观结构的信息却十分有限。因此，本研究旨在通过微计算机断层扫描（microcomputed tomography，µCT）确定临床相关部位的皮质和小梁骨特性。我们对维也纳自然历史博物馆提供的 10 根股骨和 10 根胫骨的 Paget 病病变部位与 13 根非病变股骨和 10 根胫骨进行了比较。采用基于 X 射线的µCT 扫描仪对皮质和小梁骨微观结构进行数字化处理。此外，还对股骨和胫骨的小梁和皮质结构的半定量分级进行了生成。µCT 图像显示出皮质厚度、皮质孔隙率和皮质结构的小梁化变化。此外，还检测到小梁结构严重紊乱、小梁缺陷和（剩余）小梁变厚。皮质的数值分析显示，外层（66-100%）的总骨体积（total bone volume，BV）和 BV 减少（-36%，p=0.004 和-50%，p<0.001），外层的总容积（total volume，TV）减少（-42%，p<0.001），外层的总骨体积分数（total bone volume fraction，BV/TV）和 BV/TV 减少（-23%和-12%，p<0.001），中层（33-66%）的 BV 和 TV 增加和内层（0-33%）的 BV/TV 增加（123%，p=0.011、147%，p=0.010 和 33%，p=0.025）。与非病变骨相比，Paget 病中小梁的 BV/TV 更高（96%，p=0.008）和 Tb.Th 更高（43%，p=0.004）。负重长骨的皮质和小梁部位的 PDB 存在明显且一致的结构改变。我们的发现与 PDB 的鉴别诊断和相关并发症的发病机制有关，因为这种疾病会导致结构异常，从而导致骨骼脆弱。
