中国血友病骨骼肌肉并发症康复评估与治疗专家共识

中国血友病协作组; 中国罕见病联盟血友病学组

doi:10.12376/j.issn.2097-0501.2022.04.010

中国血友病骨骼肌肉并发症康复评估与治疗专家共识

doi: 10.12376/j.issn.2097-0501.2022.04.010

1.
中国医学科学院北京协和医院康复医学科，北京 100730
2.
中国医学科学院北京协和医院心内科，北京 100730
3.
中国医学科学院北京协和医院疑难重症及罕见病国家重点实验室，北京 100730

基金项目:

中央高水平医院临床科研业务费 2022-PUMCH-B-131

国家重点研发计划资助项目 2016YFC0901500

陈丽霞¹, E-mail：clx727@126.com; 张抒扬^2，3, E-mail：shuyangzhang@163.com

详细信息

中图分类号: R493;R554+.1
计量
- 文章访问数: 219
- HTML全文浏览量: 37
- PDF下载量: 57
- 被引次数: 0
出版历程
- 收稿日期: 2022-08-15
- 录用日期: 2022-08-22
- 网络出版日期: 2022-12-23

Expert Consensus on Assessment for Rehabilitation and Treatment of Musculoskeletal Complications of Hemophilia in China

1.
Department of Rehabilitation Medicine, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
2.
Department of Cardiology, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
3.
State Key Laboratory of Complex Severe and Rare Diseases, Peking Union MedicalCollege Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China

Funding:

National High Level Hospital Clinical Research Funding 2022-PUMCH-B-131

National Key Research and Development Program of China 2016YFC0901500

CHEN Lixia¹, E-mail: clx727@126.com; ZHANG Shuyang^{2, 3}, E-mail: shuyangzhang@163.com

摘要

摘要: 血友病是一组遗传性出血性疾病，骨骼肌肉出血是最常见表现，是患者致残的重要原因，康复医学在血友病综合管理中发挥了重要的作用，近年来，国内对血友病患者康复评估和治疗的研究增多。基于目前的循证医学证据，多学科领域专家在血友病骨骼肌肉并发症康复评估与治疗方面达成一致共识，为临床规范化诊疗提供了重要依据。
- 血友病 /
- 骨骼肌肉并发症 /
- 康复评估 /
- 康复治疗
Abstract: Hemophilia is a group of hereditary hemorrhagic diseases. Hemorrhages mostly occur in musculoskeletal system and is the main cause for disability. Rehabilitation plays an important role in the comprehensive management in hemophilia. In recent years, researches on rehabilitative assessment and treatment of hemophilia have made a lot of progress in China. Using evidence-based method, experts from different disciplines have reached a consensus on the rehabilitation assessment and treatment of hemophilia skeletal muscle complications, providing an important reference to the clinical practice.
- hemophilia /
- musculoskeletal complication /
- rehabilitative assessment /
- rehabilitative treatment
注释:

作者贡献： 张抒扬牵头制订共识框架，组建共识制订工作组，并审阅定稿；陈丽霞带领工作组复习文献，起草共识初稿，组织全国专家讨论、修改初稿，并最后定稿；刘淑芬负责查阅文献，撰写初稿，并收集专家意见、修改初稿。陈丽霞、陈晓顶、冯宾、李魁星、刘淑芬、刘颖、罗轮杰、史明楠、王书杰、王岩、翁习生、吴鸣、杨朝辉、杨仁池、尹瑞雪参与讨论并形成修订意见。

利益冲突： 共识制订工作组所有参与人员均声明不存在利益冲突。

HTML全文

表 1 GRADE证据质量分级与定义

Table 1. GRADE evidence quality classification and definition

证据等级	具体描述
高(A)	非常有把握观察值接近真实值
中(B)	对观察值有中等把握：观察值有可能接近真实值，但也有可能差别很大
低(C)	对观察值得把握有限：观察值可能与真实值有很大差别
极低(D)	对观察值几乎没有把握：观察值与真实值可能有极大差别

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表 2 GRADE推荐强度分级与定义

Table 2. GRADE recommended strength grading and definition

推荐强度	具体描述
强(1)	明确显示干预措施利大于弊或弊大于利
弱(2)	利弊不确定或无论质量高低的证据显示利弊相当

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表 3 不同血友病关节评估量表项目及分值

Table 3. Items and scores of different hemophilia joint assessment scales

Gilbert			Colorado PE-1(成人)	Colorado PE-0.5(7岁以上儿童)	HJHS(4~18岁儿童)
项目	分值	项目	分值	分值	项目	分值
肿胀	2	肿胀	3	2	肿胀	3
-	-				肿胀持续时间	1
肌肉萎缩	1	肌肉萎缩	3	2	肌肉萎缩	2
轴向畸形	2	轴向畸形	2	2	轴向畸形	-
活动时骨摩擦音	1	活动时骨摩擦音	3	2	活动时骨摩擦音	2
活动度	2	活动度	3	2	伸展度丢失	3
屈曲挛缩	2	屈曲挛缩	3	2	屈曲度丢失	3
不稳定性	2	不稳定性	2	2	-	-
疼痛	2	疼痛	2	2	疼痛	2
-	-	静息痛	3	3	-	-
-	-	步态异常	3	3	步态异常	4
-	-	肌力	2	2	肌力	4
-不包含该项目

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