Department of Diagnostic and Interventional Radiology, University Hospital Bonn, Bonn, Germany.
Department of Orthopaedics and Trauma Surgery, University Hospital Bonn, Bonn, Germany.
Hamostaseologie. 2023 Jun;43(3):208-214. doi: 10.1055/a-1972-8983. Epub 2023 Mar 2.
Patients with hemophilia (PWHs) suffer from an increased risk of osteoporosis. Multiple hemophilia and hemophilic arthropathy associated factors correlate with a low bone mineral density (BMD) in PWHs. The aim of this study was to assess the long-term development of BMD in PWH as well as to analyze potentially influencing factors.
A total of 33 adult PWHs were evaluated in a retrospective study. General medical history, specific-hemophilia-associated comorbidities, joint status using the Gilbert score, calcium level, and vitamin D level as well as at least two results of bone density measurements with a minimum range of 10 years per patient were taken into account.
The BMD did not change significantly from one point of measurement to the other. A total of 7 (21.2%) cases of osteoporosis and 16 (48.5%) cases of osteopenia were identified. The two following significant correlations could be revealed: the higher the patients' body mass index, the higher their BMD ( = 0.41; = 0.022). Moreover, a high Gilbert score came along with a low BMD ( = -0.546; = 0.003).
Even if PWHs frequently suffer from a reduced BMD, our data suggest that their BMD remains constant on a low level in the course of time. A risk factor of osteoporosis often found in PWHs is a vitamin D deficiency and joint destruction. Therefore, a standardized screening of PWHs on BMD reduction by collecting vitamin D blood level and assessing joint status seems appropriate.
血友病患者(PWH)存在骨质疏松风险增加的问题。多种血友病和血友病性关节病相关因素与 PWH 的骨密度(BMD)降低相关。本研究旨在评估 PWH 的 BMD 长期发展情况,并分析潜在的影响因素。
在一项回顾性研究中,共评估了 33 名成年 PWH。考虑了一般病史、特定血友病相关合并症、吉尔伯特评分的关节状况、钙水平和维生素 D 水平,以及至少两次骨密度测量结果,每个患者的测量范围至少为 10 年。
BMD 从一次测量到另一次测量没有显著变化。共发现 7 例(21.2%)骨质疏松症和 16 例(48.5%)骨量减少症。可以揭示以下两个显著相关性:患者的体重指数越高,其 BMD 越高(=0.41;=0.022)。此外,高吉尔伯特评分与低 BMD 相关(= -0.546;=0.003)。
尽管 PWH 经常出现 BMD 降低的情况,但我们的数据表明,他们的 BMD 在一段时间内保持在低水平上的稳定。在 PWH 中经常发现的骨质疏松症的一个危险因素是维生素 D 缺乏和关节破坏。因此,通过收集维生素 D 血液水平并评估关节状况,对 PWH 进行 BMD 降低的标准化筛查似乎是合适的。