Liu Ying, Ge Ying, Shi Mingnan, Zhang Li, Chen Lixia, Xia Weibo
Department of Rehabilitation Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Dongcheng District, Beijing, 100730, China.
Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, China.
BMC Musculoskelet Disord. 2025 Mar 11;26(1):242. doi: 10.1186/s12891-025-08473-7.
Current research on osteoporosis (OP) in hemophilia is insufficient. The suitability of high-resolution peripheral quantitative computed tomography (HR-pQCT) for evaluating osteoporosis in hemophilia remains unclear.
To investigate the current status of osteoporosis and the applicability of HR-pQCT in adult hemophilia patients.
Thirty three hemophilia patients aged 23-49 years were recruited. X-ray examinations were performed on the bleeding joints. Dual energy X-ray absorptiometry (DXA) and HR-pQCT were used to assess bone mineral density (BMD). The HR-pQCT values of the distal tibia and radius were compared between hemophilia patients and healthy controls(HCs).
All bleeding joints showed local osteoporosis on X-ray. Only 33.3% of patients had a hip BMD lower than the expected value according to DXA. The Tb.vBMD(98.5 ± 44.2 mg/cm), Tt.Ar(612.5 ± 163.5mm),Tb.Ar(487.0 ± 175.6mm), Ct.Ar(117.0 ± 25.7mm), Tb.BV/TV(0.2 ± 0.1), Tb.N(0.9 ± 0.3 1/mm), Ct.Pm(96.3 ± 13.8 mm) of the distal tibia and Tt.Ar(248.4 ± 53.1mm),Tb.Ar(186.0 ± 55.1mm), Ct.Ar(66.1 ± 14.4 mm), Ct.Pm(68.1 ± 7.1 mm) of the distal radius in the hemophilia group was significantly lower than the HCs(tibia Tb.vBMD:186.4 ± 44.3mg/cm, Tt.Ar:906.8 ± 135.0mm,Tb.Ar:743.7 ± 137.6mm, Ct.Ar:169.3 ± 21.9mm,Tb.
BV/TV: 0.3 ± 0.1, Tb.N:1.5 ± 0.2 1/mm,Ct.Pm:117.8 ± 8.2 mm; radius Tt.Ar:285.7 ± 35.6 mm, Tb.Ar:83.8 ± 7.9mm, Ct.Ar:0.3 ± 0.1mm, Ct.Pm:80.2 ± 4.3 mm) with statistically significant differences (p < 0.05). Correlation analysis showed a positive correlation (r = 0.768, p = 0.016) between femoral neck BMD with DXA and total volumetric BMD(Tt.vBMD) at the distal tibia.
The bone health status of adult hemophilia patients in China is worrying. The occurrence of OP may be accompanied by varying degrees of bone loss, bone destruction, and structural abnormalities observed in both trabecular and cortical bones of the upper and lower limbs. The condition of the trabecular bones in the lower limbs is particularly severe. The correlation between BMD measurements obtained from HR-pQCT and DXA is strong.
目前关于血友病患者骨质疏松（OP）的研究尚不充分。高分辨率外周定量计算机断层扫描（HR-pQCT）评估血友病患者骨质疏松的适用性仍不明确。
探讨成年血友病患者骨质疏松的现状以及HR-pQCT的适用性。
招募了33例年龄在23至49岁之间的血友病患者。对出血关节进行X线检查。采用双能X线吸收法（DXA）和HR-pQCT评估骨密度（BMD）。比较血友病患者与健康对照者（HCs）胫骨远端和桡骨远端的HR-pQCT值。
所有出血关节在X线下均显示局部骨质疏松。根据DXA，仅33.3%的患者髋部骨密度低于预期值。血友病组胫骨远端的骨小梁体积骨密度（Tb.vBMD，98.5±44.2mg/cm）、总横截面积（Tt.Ar，612.5±163.5mm）、骨小梁横截面积（Tb.Ar，487.0±175.6mm）、皮质骨横截面积（Ct.Ar，117.0±25.7mm）、骨小梁骨体积分数（Tb.BV/TV，0.2±0.1）、骨小梁数量（Tb.N，0.9±0.3 1/mm）、皮质骨厚度（Ct.Pm，96.3±13.8mm）以及桡骨远端的Tt.Ar（248.4±53.1mm）、Tb.Ar（186.0±55.1mm）、Ct.Ar（66.1±14.4mm）、Ct.Pm（68.1±7.1mm）均显著低于健康对照组（胫骨：Tb.vBMD：186.4±44.3mg/cm，Tt.Ar：906.8±135.0mm，Tb.Ar：743.7±137.6mm，Ct.Ar：169.3±21.9mm，Tb.BV/TV：0.3±0.1，Tb.N：1.5±0.2 1/mm，Ct.Pm：117.8±8.2mm；桡骨：Tt.Ar：285.7±35.6mm，Tb.Ar：83.8±7.9mm，Ct.Ar：0.3±0.1mm，Ct.Pm：80.2±4.3mm），差异具有统计学意义（p<0.05）。相关性分析显示，DXA测量的股骨颈骨密度与胫骨远端的总体积骨密度（Tt.vBMD）呈正相关（r=0.768，p=0.016）。
中国成年血友病患者的骨骼健康状况令人担忧。OP的发生可能伴有不同程度的骨质流失、骨质破坏以及上下肢小梁骨和皮质骨的结构异常。下肢小梁骨的情况尤为严重。HR-pQCT与DXA测量的骨密度之间具有很强的相关性。
