Ransmann Pia, Brühl Marius, Hmida Jamil, Goldmann Georg, Oldenburg Johannes, Schildberg Frank Alexander, Ossendorff Robert, Tomschi Fabian, Schmidt Alexander, Hilberg Thomas, Strauss Andreas Christian
Department of Sports Medicine, University of Wuppertal, Wuppertal, Germany.
Department of Orthopedics and Trauma Surgery, University Hospital Bonn, Bonn, Germany.
PLoS One. 2025 Mar 26;20(3):e0319951. doi: 10.1371/journal.pone.0319951. eCollection 2025.
Patients with haemophilia (PwH) might be restricted in physical activity (PA) depending on the severity phenotype. It is well-known that PA affects overall health including bone quality. This study aims to evaluate the level of PA within the different haemophilia severity phenotypes and to elaborate on the interplay of PA in regard to bone quality (bone mineral density (BMD) and trabecular bone score (TBS)) as well as lean mass.
This investigation was part of a large prospective single-center cohort study examining the relation between haemophilia and osteoporosis registered at clinicaltrials.gov (ID: NCT04524481). PwH underwent a dual x-ray screening using Horizon™ to examine BMD, TBS, and lean mass. Step activity was tracked electronically for seven consecutive days after clinical examination, supported by a self-reported activity diary for seven days. Handgrip strength was examined as an overall fitness proxy.
Data of 223 patients with either mild (N = 45), moderate (N = 46), or severe (N = 132) haemophilia A or B, aged 43.6 ± 15.6 years were analyzed. There was no significant difference in objective (p = 0.162) and subjective (p = 0.459) PA levels between severity phenotypes. The most frequent type of PA in all severities was walking (n = 72, 53.3%) and cycling (n = 60, 44.4%). Step activity positively correlated with TBS (rho = 0.202, p = 0.005) and lean mass positively correlated with BMD (rho = 0.309, p < 0.001). Handgrip strength correlated with BMD (rho = 0.361, p < 0.001) as well as TBS (rho = 0.221, p = 0.021) and lean mass (rho = 0.287, p = 0.003).
PA does not differ significantly between the severity phenotypes. The majority of PwH in all severity phenotypes performed low-impact PA, which is most likely insufficient to positively affect BMD. However, handgrip strength correlates with BMD and TBS. Despite restrictions in movement function or reduced BMD, it is of major importance to promote PA to maintain a healthy or even increase bone quality.
血友病患者(PwH)的身体活动(PA)可能因严重程度表型而受到限制。众所周知,身体活动会影响包括骨质在内的整体健康。本研究旨在评估不同血友病严重程度表型中的身体活动水平,并阐述身体活动在骨质(骨矿物质密度(BMD)和骨小梁评分(TBS))以及瘦体重方面的相互作用。
本调查是一项大型前瞻性单中心队列研究的一部分,该研究在clinicaltrials.gov(ID:NCT04524481)上注册,研究血友病与骨质疏松症之间的关系。PwH使用Horizon™进行双能X线筛查,以检查BMD、TBS和瘦体重。临床检查后连续七天通过电子方式跟踪步数活动,并辅以七天的自我报告活动日记。将握力作为整体健康状况的指标进行检查。
分析了223例年龄为43.6±15.6岁的轻度(N = 45)、中度(N = 46)或重度(N = 132)甲型或乙型血友病患者的数据。严重程度表型之间的客观(p = 0.162)和主观(p = 0.459)身体活动水平没有显著差异。所有严重程度中最常见 的身体活动类型是步行(n = 72,53.3%)和骑自行车(n = 60,44.4%)。步数活动与TBS呈正相关(rho = 0.202,p = 0.005),瘦体重与BMD呈正相关(rho = 0.309,p < 0.001)。握力与BMD(rho = 0.361,p < 0.001)、TBS(rho = 0.221,p = 0.021)和瘦体重(rho = 0.287,p = 0.003)相关。
严重程度表型之间的身体活动没有显著差异。所有严重程度表型中的大多数PwH进行的是低强度身体活动,这很可能不足以对BMD产生积极影响。然而,握力与BMD和TBS相关。尽管存在运动功能限制或BMD降低,但促进身体活动对于维持健康甚至提高骨质至关重要。