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血友病性关节病和血肿的最佳管理。

Optimal management of hemophilic arthropathy and hematomas.

作者信息

Lobet Sébastien, Hermans Cedric, Lambert Catherine

机构信息

Hemostasis-Thrombosis Unit, Division of Hematology, Cliniques Universitaires Saint-Luc, Brussels, Belgium ; Division of Physical Medicine and Rehabilitation, Cliniques Universitaires Saint-Luc, Brussels, Belgium.

Hemostasis-Thrombosis Unit, Division of Hematology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.

出版信息

J Blood Med. 2014 Oct 17;5:207-18. doi: 10.2147/JBM.S50644. eCollection 2014.

DOI:10.2147/JBM.S50644
PMID:25378964
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4207585/
Abstract

Hemophilia is a hematological disorder characterized by a partial or complete deficiency of clotting factor VIII or IX. Its bleeding complications primarily affect the musculoskeletal system. Hemarthrosis is a major hemophilia-related complication, responsible for a particularly debilitating chronic arthropathy, in the long term. In addition to clotting factor concentrates, usually prescribed by the hematologist, managing acute hemarthrosis and chronic arthropathy requires a close collaboration between the orthopedic surgeon and physiotherapist. This collaboration, comprising a coagulation and musculoskeletal specialist, is key to effectively preventing hemarthrosis, managing acute joint bleeding episodes, assessing joint function, and actively treating chronic arthropathy. This paper reviews, from a practical point of view, the pathophysiology, clinical manifestations, and treatment of hemarthrosis and chronic hemophilia-induced arthropathy for hematologists, orthopedic surgeons, and physiotherapists.

摘要

血友病是一种血液系统疾病,其特征是凝血因子VIII或IX部分或完全缺乏。其出血并发症主要影响肌肉骨骼系统。关节积血是一种主要的血友病相关并发症,长期来看会导致特别使人衰弱的慢性关节病。除了血液科医生通常开具的凝血因子浓缩物外,处理急性关节积血和慢性关节病需要骨科医生和物理治疗师密切合作。这种由凝血和肌肉骨骼专家组成的合作对于有效预防关节积血、处理急性关节出血发作、评估关节功能以及积极治疗慢性关节病至关重要。本文从实际角度为血液科医生、骨科医生和物理治疗师综述了关节积血和慢性血友病性关节病的病理生理学、临床表现及治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/6a510e579202/jbm-5-207Fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/74192e9a72a0/jbm-5-207Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/b6d1f25edf4e/jbm-5-207Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/eb83fbd86a1d/jbm-5-207Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/7463d9246f27/jbm-5-207Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/6a510e579202/jbm-5-207Fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/74192e9a72a0/jbm-5-207Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/b6d1f25edf4e/jbm-5-207Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/eb83fbd86a1d/jbm-5-207Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/7463d9246f27/jbm-5-207Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0d55/4207585/6a510e579202/jbm-5-207Fig5.jpg

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Haemophilia. 2013 Jul;19(4):487-98. doi: 10.1111/hae.12118. Epub 2013 Mar 28.
2
The effect of cooling on coagulation and haemostasis: should "Ice" be part of treatment of acute haemarthrosis in haemophilia?冷却对凝血和止血的影响:在血友病急性关节积血的治疗中,“冰”是否应该成为治疗手段的一部分?
Haemophilia. 2012 Nov;18(6):843-50. doi: 10.1111/j.1365-2516.2012.02918.x. Epub 2012 Aug 23.
3
Consequences of intra-articular bleeding in haemophilia: science to clinical practice and beyond.
The Use of Primary Care Physiotherapy for Persons With Bleeding Disorders in the Netherlands-A Retrospective Study of Health Records.
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Haemophilia. 2025 May;31(3):526-534. doi: 10.1111/hae.70034. Epub 2025 Mar 18.
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Evaluating the Cost-Effectiveness of Etranacogene Dezaparvovec Gene Therapy for Hemophilia B Treatment in the USA.评估依特那考基因德扎帕罗韦克基因疗法在美国治疗B型血友病的成本效益。
Appl Health Econ Health Policy. 2025 May;23(3):467-478. doi: 10.1007/s40258-024-00932-x. Epub 2024 Dec 2.
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Superior Prophylactic Effectiveness of a Recombinant FVIIIFc Over Standard Half-Life FVIII in Hemophilia A: A-SURE Study.重组FVIIIFc对比标准半衰期FVIII在A型血友病中的卓越预防效果:A-SURE研究
Eur J Haematol. 2025 Feb;114(2):248-257. doi: 10.1111/ejh.14309. Epub 2024 Oct 21.
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J Clin Med. 2024 Aug 29;13(17):5121. doi: 10.3390/jcm13175121.
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