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血友病B伴颅内出血在高依赖病房的康复:一例报告

Hemophilia B With Intracranial Hemorrhage Rehabilitation in High-Dependency Unit: A Case Report.

作者信息

Feng Xiaoyan, Zhu Hongjun, Han Liying, Xu Hongxing, He Lei

机构信息

Department of Physical Medicine & Rehabilitation The First Affiliated Hospital of Soochow University Suzhou Jiangsu China.

出版信息

Clin Case Rep. 2025 Apr 21;13(4):e70470. doi: 10.1002/ccr3.70470. eCollection 2025 Apr.

DOI:10.1002/ccr3.70470
PMID:40264735
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12011989/
Abstract

Hemophilia B, an X-linked recessive coagulation disorder, poses significant risks of life-threatening intracranial hemorrhage (ICH). This case report details the multidisciplinary rehabilitation of a 41-year-old male with moderate hemophilia B (FIX activity: 3.9%) and ICH in a resource-limited setting. Admitted to a high-dependency unit postneurosurgical intervention, the patient received low-dose prophylactic coagulation factor IX (maintained at 34.6%-66.2%) alongside real-time coagulation monitoring. A stepwise rehabilitation protocol was implemented, including early passive joint mobilization, neuromuscular electrical stimulation, and progressive task-oriented training, tailored to minimize bleeding risk. Over 7 weeks, the patient achieved marked functional improvement: Activity of Daily Living score increased from 0 to 80, modified Rankin Scale improved from 5 to 3, and Fugl-Meyer Assessment (FMA) rose from 0 to 60, with no secondary bleeding. This case highlights the feasibility of integrating low-dose prophylaxis with early rehabilitation in developing countries, offering a cost-effective model to enhance functional recovery and reduce disability in hemophilia-related ICH.

摘要

乙型血友病是一种X连锁隐性凝血障碍疾病,具有危及生命的颅内出血(ICH)的重大风险。本病例报告详细介绍了一名41岁中度乙型血友病(FIX活性:3.9%)且发生颅内出血的男性患者在资源有限环境下的多学科康复情况。在神经外科干预后入住高依赖病房,患者接受了低剂量预防性凝血因子IX治疗(维持在34.6%-66.2%)并进行实时凝血监测。实施了逐步康复方案,包括早期被动关节活动、神经肌肉电刺激以及渐进性任务导向训练,以尽量降低出血风险。在7周内,患者功能显著改善:日常生活活动评分从0提高到80,改良Rankin量表评分从5改善到3,Fugl-Meyer评估(FMA)从0升至60,且未发生二次出血。本病例突出了在发展中国家将低剂量预防与早期康复相结合的可行性,提供了一种具有成本效益的模式,以促进血友病相关颅内出血患者的功能恢复并减少残疾。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7251/12011989/482293bdf002/CCR3-13-e70470-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7251/12011989/d184c709d904/CCR3-13-e70470-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7251/12011989/482293bdf002/CCR3-13-e70470-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7251/12011989/d184c709d904/CCR3-13-e70470-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7251/12011989/482293bdf002/CCR3-13-e70470-g001.jpg

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