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血友病患者骨质疏松症保持不变-考虑合并症的长期病程。

Osteoporosis Remains Constant in Patients with Hemophilia-Long-Term Course in Consideration of Comorbidities.

机构信息

Department of Diagnostic and Interventional Radiology, University Hospital Bonn, Bonn, Germany.

Department of Orthopaedics and Trauma Surgery, University Hospital Bonn, Bonn, Germany.

出版信息

Hamostaseologie. 2023 Jun;43(3):208-214. doi: 10.1055/a-1972-8983. Epub 2023 Mar 2.

Abstract

INTRODUCTION

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.

METHODS

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.

RESULTS

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).

CONCLUSION

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.

摘要

简介

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。

方法

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。

结果

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)。

结论

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。

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