Comparison of bone microstructure and strength in the distal radius and tibia between the different types of primary hypertrophic osteoarthropathy: an HR-pQCT study.

Comparison of bone microstructure and strength in the distal radius and tibia between the different types of primary hypertrophic osteoarthropathy: an HR-pQCT study.
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DOI:
10.1007/s00198-023-06784-w
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发表时间:
2023-08
影响因子:
4
通讯作者:
Xia, Weibo
Xia, Weibo
中科院分区:
医学2区
文献类型:
--
作者:
Pang, Qianqian;Xu, Yuping;Liao, Ruoxi;Li, Ye;Zhang, Li;Chi, Yue;Qi, Xuan;Li, Mei;Jiang, Yan;Wang, Ou;Xing, Xiaoping;Qin, Ling;Xia, Weibo

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原发性肥大性骨关节病(PHO)是一种遗传性骨病,由于致病基因不同,分为PHO常染色体隐性遗传1型(PHOAR 1)和PHO常染色体隐性遗传2型(PHOAR 2)。比较两种亚型之间骨微结构的数据很少。这是首次发现PHOAR 1患者的骨显微结构低于PHOAR 2患者的研究。本研究的主要目的是评估PHOAR 1和PHOAR 2患者的骨微结构和强度,并将其与年龄和性别匹配的健康对照(HC)进行比较。次要目标是评估PHOAR 1和PHOAR 2患者之间的差异。从北京协和医院招募了27名男性PHO患者(PHOAR 1 = 7; PHOAR 2 = 20)。采用双能X线骨密度仪(DXA)测量骨密度(aBMD)。通过高分辨率外周定量计算机断层扫描(HR-pQCT)评价桡骨远端和胫骨的外周骨微结构。研究了PGE 2、骨转换和Dickkopf-1(DKK 1)的生化标志物。与HC相比,PHOAR 1和PHOAR 2患者的骨几何形状明显更大,桡骨和胫骨的vBMD明显更低,桡骨的皮质微结构受损。对于骨小梁,PHOAR 1和PHOAR 2患者在胫骨显示不同的变化。PHOAR 1患者的骨小梁室有显著缺陷,导致骨强度估计值较低。相反,PHOAR 2患者显示出比HC更高的骨小梁数量、更窄的骨小梁分离和更低的骨小梁网络不均匀性,转化为保留的或略高的估计骨强度。与PHOAR 2患者和HC相比,PHOAR 1患者的骨显微结构和强度较差。此外,这项研究是第一个发现PHOAR 1和PHOAR 2患者之间骨微结构差异的研究。在线版本包含补充材料,可通过10.1007/s 00198 -023-06784-w获得。
Primary hypertrophic osteoarthropathy (PHO) is a hereditary bone disease that is grouped into PHO autosomal recessive 1 (PHOAR1) and PHO autosomal recessive 2 (PHOAR2) due to different causative genes. Data comparing bone microstructure between the two subtypes are scarce. This is the first study to find that PHOAR1 patients had inferior bone microstructure compared with PHOAR2 patients. The primary goal of this study was to assess bone microarchitecture and strength in PHOAR1 and PHOAR2 patients and to compare them with age- and sex-matched healthy controls (HCs). The secondary goal was to assess the differences between PHOAR1 and PHOAR2 patients. Twenty-seven male Chinese PHO patients (PHOAR1 = 7; PHOAR2 = 20) were recruited from Peking Union Medical College Hospital. The areal bone mineral density (aBMD) was assessed by dual-energy X-ray absorptiometry (DXA). Peripheral bone microarchitecture at the distal radius and tibia were evaluated by high-resolution peripheral quantitative computed tomography (HR-pQCT). Biochemical markers of PGE2, bone turnover, and Dickkopf-1 (DKK1) were investigated. Compared with HCs, PHOAR1 and PHOAR2 patients had distinctively larger bone geometry, substantially lower vBMD at the radius and tibia, and compromised cortical microstructure at the radius. For trabecular bone, PHOAR1 and PHOAR2 patients showed different changes at the tibia. PHOAR1 patients had significant deficits in the trabecular compartment, resulting in lower estimated bone strength. Conversely, PHOAR2 patients showed a higher trabecular number, narrower trabecular separation, and lower trabecular network inhomogeneity than HCs, translating into preserved or slightly high estimated bone strength. PHOAR1 patients had inferior bone microstructure and strength compared with PHOAR2 patients and HCs. Additionally, this study was the first to find differences in the bone microstructure between PHOAR1 and PHOAR2 patients. The online version contains supplementary material available at 10.1007/s00198-023-06784-w.
DOI: 10.1002/jbmr.111
发表时间: 2010-10
期刊: Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
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