Shanbhogue Vikram V, Hansen Stinus, Jørgensen Niklas Rye, Brixen Kim, Gravholt Claus H
Department of Endocrinology, Odense University Hospital, Odense, Denmark; Institute of Clinical Research, University of Southern Denmark, Odense, Denmark.
J Bone Miner Res. 2014 Nov;29(11):2474-82. doi: 10.1002/jbmr.2272.
Although the expected skeletal manifestations of testosterone deficiency in Klinefelter's syndrome (KS) are osteopenia and osteoporosis, the structural basis for this is unclear. The aim of this study was to assess bone geometry, volumetric bone mineral density (vBMD), microarchitecture, and estimated bone strength using high-resolution peripheral quantitative computed tomography (HR-pQCT) in patients with KS. Thirty-one patients with KS confirmed by lymphocyte chromosome karyotyping aged 35.8 ± 8.2 years were recruited consecutively from a KS outpatient clinic and matched with respect to age and height with 31 healthy subjects aged 35.9 ± 8.2 years. Dual-energy X-ray absorptiometry (DXA) and HR-pQCT were performed in all participants, and blood samples were analyzed for hormonal status and bone biomarkers in KS patients. Twenty-one KS patients were on long-term testosterone-replacement therapy. In weight-adjusted models, HR-pQCT revealed a significantly lower cortical area (p < 0.01), total and trabecular vBMD (p = 0.02 and p = 0.04), trabecular bone volume fraction (p = 0.04), trabecular number (p = 0.05), and estimates of bone strength, whereas trabecular spacing was higher (p = 0.03) at the tibia in KS patients. In addition, cortical thickness was significantly reduced, both at the radius and tibia (both p < 0.01). There were no significant differences in indices of bone structure, estimated bone strength, or bone biomarkers in KS patients with and without testosterone therapy. This study showed that KS patients had lower total vBMD and a compromised trabecular compartment with a reduced trabecular density and bone volume fraction at the tibia. The compromised trabecular network integrity attributable to a lower trabecular number with relative preservation of trabecular thickness is similar to the picture found in women with aging. KS patients also displayed a reduced cortical area and thickness at the tibia, which in combination with the trabecular deficits, compromised estimated bone strength at this site.
虽然克氏综合征（KS）中睾酮缺乏预期的骨骼表现是骨质减少和骨质疏松，但其结构基础尚不清楚。本研究的目的是使用高分辨率外周定量计算机断层扫描（HR-pQCT）评估KS患者的骨几何形态、体积骨密度（vBMD）、微结构和估计的骨强度。从KS门诊连续招募了31例经淋巴细胞染色体核型分析确诊的KS患者，年龄为35.8±8.2岁，并与31名年龄为35.9±8.2岁的健康受试者在年龄和身高方面进行匹配。所有参与者均进行了双能X线吸收法（DXA）和HR-pQCT检查，并对KS患者的血液样本进行了激素状态和骨生物标志物分析。21例KS患者接受长期睾酮替代治疗。在体重校正模型中，HR-pQCT显示KS患者胫骨的皮质面积显著降低（p<0.01）、总vBMD和小梁vBMD（p=0.02和p=0.04）、小梁骨体积分数（p=0.04）、小梁数量（p=0.05）以及骨强度估计值降低，而小梁间距更高（p=0.03）。此外，桡骨和胫骨的皮质厚度均显著降低（均p<0.01）。接受和未接受睾酮治疗的KS患者在骨结构指数、估计的骨强度或骨生物标志物方面没有显著差异。本研究表明，KS患者的总vBMD较低，小梁骨区受损，胫骨小梁密度和骨体积分数降低。小梁数量减少而小梁厚度相对保留导致小梁网络完整性受损，这与老年女性的情况相似。KS患者胫骨的皮质面积和厚度也降低，这与小梁缺陷相结合，损害了该部位的估计骨强度。
