Department of Medicine, School of Clinical Sciences at Monash Health, Monash University, Clayton, Victoria, Australia.
Australian Centre for Blood Diseases, Monash University, Melbourne, Australia.
Haemophilia. 2022 May;28(3):388-396. doi: 10.1111/hae.14540. Epub 2022 Mar 15.
Patients with haemophilia (PWH) have a high prevalence of osteoporosis, falls and fractures at all ages. The role of haemophilia itself may contribute to low bone mineral density (BMD) due to coagulation factor deficiency. Guidelines for the management of osteoporosis, fracture and fall risk may help to reduce fracture and fall risk, and delay osteoporosis onset.
We aim to review current haemophilia guidelines regarding osteoporosis prevention, screening, diagnosis and management, and fall prevention.
A database search (Ovid MEDLINE) revealed two haemophilia guidelines (World and British) published within the last ten years. Local Australian haemophilia guidelines were identified through a manual search.
All haemophilia guidelines were found to contain inadequate recommendations for osteoporosis management and fall prevention due to a lack of evidence in the literature.
Further studies are required to assess the trajectory of bone health in PWH, the mechanism of bone loss in PWH, and the effectiveness of weight-bearing exercises, interventions for fall prevention, screening programmes, and use of anti-osteoporosis medications in PWH across the lifecourse.
血友病患者（PWH）在各个年龄段均存在骨质疏松症、跌倒和骨折的高发风险。由于凝血因子缺乏，血友病本身可能导致骨矿物质密度（BMD）降低。骨质疏松症、骨折和跌倒风险管理指南有助于降低骨折和跌倒风险，延缓骨质疏松症的发生。
我们旨在回顾目前有关血友病预防骨质疏松症、筛查、诊断和管理以及预防跌倒的指南。
通过数据库搜索（Ovid MEDLINE）发现了最近十年发布的两份血友病指南（世界和英国）。通过手动搜索确定了澳大利亚当地血友病指南。
由于文献中缺乏证据，所有血友病指南都发现对骨质疏松症管理和预防跌倒的建议不足。
需要进一步研究来评估 PWH 骨健康的轨迹、PWH 中骨丢失的机制，以及负重锻炼、预防跌倒的干预措施、筛查计划以及在整个生命周期中使用抗骨质疏松症药物的有效性。
