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Archives of Osteoporosis

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Open Access 01-12-2023 | Hemophilia | Review

Pathogenesis and treatment of osteoporosis in patients with hemophilia

Authors: Xiaoyun Lin, Peng Gao, Qian Zhang, Yan Jiang, Ou Wang, Weibo Xia, Mei Li

Published in: Archives of Osteoporosis | Issue 1/2023

Abstract

Introduction

Hemophilia is a rare X-linked recessive inherited bleeding disorder caused by mutations of the genes encoding coagulation factor VIII (FVIII) or IX (FIX). Patients with hemophilia (PWH) often have a high risk of osteoporosis and fractures that is usually ignored. Herein, we review the underlying mechanisms of osteoporosis and the increased risk of fractures and their treatment in patients with FVIII or FIX deficiency.

Methods

The PubMed, Web of Science, Embase, and Cochrane Library databases were searched to identify original research articles, meta-analyses, and scientific reviews on the mechanisms or treatment of osteoporosis in PWH.

Results

The pathogenic mechanisms of osteoporosis in PWH are multifactorial and remain unclear. The available evidence shows that FVIII and FIX deficiency may directly affect bone metabolism by interfering with the RANK/RANKL/OPG pathway. Other potential mechanisms of osteoporosis in PWH include thrombin deficiency and the unloading and immobilization of bone, which will affect osteoblast and osteoclast activity by changing the cytokine profiles. The treatment of osteoporosis in PWH includes antiresorptive, anabolic, and dual-action drugs; weight-bearing exercise; fall prevention; and prophylactic coagulation factor replacement therapy. However, clinical studies of the efficacy of anti-osteoporotic agents in osteoporosis of PWH are urgently needed.

Conclusion

This review summarizes recent progress in research on the pathogenesis of osteoporosis in PWH and provides insights into potential treatment for osteoporosis in PWH.

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Title: Pathogenesis and treatment of osteoporosis in patients with hemophilia
Authors: Xiaoyun Lin
Peng Gao
Qian Zhang
Yan Jiang
Ou Wang
Weibo Xia
Mei Li
Publication date: 01-12-2023
Publisher: Springer London
Keywords: Hemophilia
Osteoporosis
Osteoporosis
Published in: Archives of Osteoporosis / Issue 1/2023
Print ISSN: 1862-3522
Electronic ISSN: 1862-3514
DOI: https://doi.org/10.1007/s11657-022-01203-9

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Pathogenesis and treatment of osteoporosis in patients with hemophilia

Abstract

Introduction

Methods

Results

Conclusion

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Introduction

Methods

Results

Conclusion

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