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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
陈丽霞1, E-mail：clx727@126.com; 张抒扬2，3, E-mail：shuyangzhang@163.com
Expert Consensus on Assessment for Rehabilitation and Treatment of Musculoskeletal Complications of Hemophilia in China
CHEN Lixia1, E-mail: clx727@126.com; ZHANG Shuyang2, 3, E-mail: shuyangzhang@163.com
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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。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.
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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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[1] Bolton-Maggs PH, Pasi KJ. Haemophilias A and B[J]. Lancet, 2003, 361: 1801-1809. doi: 10.1016/S0140-6736(03)13405-8 [2] 邵宗鸿, 杨天楹. 中国血友病患病率与八个地区生存率调查[J]. 中华血液学杂志, 1992, 13: 461-463. [3] 杨仁池. 中国血友病管理指南(2021年版)[M]. 北京: 中国协和医科大学出版社, 2021. [4] Atilla B, Güney-Deniz H. Musculoskeletal treatment in haemophilia[J]. EFORT Open Rev, 2019, 4: 230-239. doi: 10.1302/2058-5241.4.180068 [5] Song X, Zhong J, Xue F, et al. An overview of patients with haemophilia A in China: epidemiology, disease severity and treatment strategies[J]. Haemophilia, 2021, 27: e51-e59. [6] Gilbert MS. Prophylaxis: musculoskeletal evaluation[J]. Semin Hematol, 1993, 30: 3-6. [7] Manco-Johnson MJ, Nuss R, Funk S, et al. Joint evalua-tion instruments for children and adults with haemophilia[J]. Haemophilia, 2000, 6: 649-657. doi: 10.1046/j.1365-2516.2000.00439.x [8] Hilliard P, Funk S, Zourikian N, et al. Hemophilia joint health score reliability study[J]. Haemophilia, 2006, 12: 518-525. doi: 10.1111/j.1365-2516.2006.01312.x [9] Timmer MA, Foppen W, Schutgens RE, et al. Comparing findings of routine Haemophilia Joint Health Score and Haemophlia Early Arthropathy Detection with Ultra Sound assessments in adults with haemophilia[J]. Haemophilia, 2017, 23: e141-e143. doi: 10.1111/hae.13147 [10] Parhampour B, Alizadeh V, Torkaman G, et al. Muscle thickness and pennation angle in overweight persons with moderate haemophilia A after resistance and combined training: a randomized controlled trial[J]. Haemophilia, 2022, 28: 505-514. doi: 10.1111/hae.14539 [11] 徐小静, 杨朝辉, 肖喜玲, 等. 影响成人血友病患者活动能力的相关因素分析[J]. 中国康复医学杂志, 2018, 33: 1178-1182. doi: 10.3969/j.issn.1001-1242.2018.10.009 [12] van Genderen FR, Westers P, Heijnen L, et al. Measur-ing patients' perceptions on their functional abilities: validation of the Haemophilia Activities List[J]. Haemophilia, 2006, 12: 36-46. [13] Groen WG, van der Net J, Helders PJ, et al. Development and preliminary testing of a paediatric version of the haemophilia activities list (pedhal)[J]. Haemophilia, 2010, 16: 281-289. doi: 10.1111/j.1365-2516.2009.02136.x [14] 汪衍雪, 杨朝辉, 王刚, 等. 中文版成人血友病活动能力评估量表的信度和效度研究[J]. 中华物理医学与康复杂志, 2018, 40: 608-612. doi: 10.3760/cma.j.issn.0254-1424.2018.08.012 [15] Poonnoose PM, Thomas R, Keshava SN, et al. Psychometric analysis of the functional independence score in haemophilia (FISH)[J]. Haemophilia, 2007, 13: 620-626. doi: 10.1111/j.1365-2516.2007.01508.x [16] Arranz P, Remor E, Quintana M, et al. Development of a new disease-specific quality-of-life questionnaire to adults living with haemophilia[J]. Haemophilia, 2004, 10: 376-382. doi: 10.1111/j.1365-2516.2004.00918.x [17] Remor E, Young NL, Von Mackensen S, et al. Disease-specific quality-of-life measurement tools for haemophilia patients[J]. Haemophilia, 2004, 10 Suppl 4: 30-34. [18] Oladapo AO, Epstein JD, Williams E, et al. Health-related quality of life assessment in haemophilia patients on prophylaxis therapy: a systematic review of results from prospective clinical trials[J]. Haemophilia, 2015, 21: e344-e358. doi: 10.1111/hae.12759 [19] Zhang W, Xie S, Xue F, et al. Health-related quality of life among adults with haemophilia in China: a comparison with age-matched general population[J]. Haemophilia, 2022, 28: 776-783. doi: 10.1111/hae.14615 [20] Wu R, Zhang J, Luke KH, et al. Cross-cultural adaptation of the CHO-KLAT for boys with hemophilia in rural and urban China[J]. Health Qual Life Outcomes, 2012, 10: 112. doi: 10.1186/1477-7525-10-112 [21] Tang L, Xu W, Li CG, et al. Describing the quality of life of boys with haemophilia in China: results of a multicentre study using the CHO-KLAT[J]. Haemophilia, 2018, 24: 113-119. doi: 10.1111/hae.13349 [22] von Mackensen S, Bullinger M; Haemo-QoL Group. Development and testing of an instrument to assess the quality of life of children with haemophilia in Europe (Haemo-QoL)[J]. Haemophilia, 2004, 10 Suppl 1: 17-25. [23] Srivastava A, Santagostino E, Dougall A, et al. WFH guidelines for the management of hemophilia panelists and co-authors. WFH Guidelines for the Management of Hemophilia, 3rd edition[J]. Haemophilia, 2020, 26 Suppl 6: 1-158. [24] Browne GJ, Barnett PLj. Common sports-related musculoskeletal injuries presenting to the emergency department[J]. J Paediatr Child Health, 2016, 52: 231-236. doi: 10.1111/jpc.13101 [25] Manners PJ, Price P, Buurman D, et al. Joint aspiration for acute hemarthrosis in children receiving factor Ⅷ prophylaxis for severe hemophilia: 11-year safety data[J]. J Rheumatol, 2015, 42: 885-890. doi: 10.3899/jrheum.141236 [26] 曾艳, 韩红, 陈慧, 等. 凝血因子输注联合激光照射对血友病患者急性皮肤、肌肉及关节出血的疗效[J]. 武汉大学学报(医学版), 2019, 40: 492-495. doi: 10.14188/j.1671-8852.2019.0012 [27] 陈丽霞, 华宝来, 刘颖, 等. 41例血友病患者髂腰肌出血的临床特征及康复治疗[J]. 中华血液学杂志, 2012, 33: 406-408. doi: 10.3760/cma.j.issn.0253-2727.2012.05.013 [28] De la Corte-Rodriguez H, Rodriguez-Merchan EC. The role of physical medicine and rehabilitation in haemophiliac patients[J]. Blood Coagul Fibrinolysis, 2013, 24: 1-9. doi: 10.1097/MBC.0b013e32835a72f3 [29] 陈丽霞, 华宝来, 刘颖, 等. 血友病髂腰肌血肿合并股神经损伤的临床特点与康复治疗效果[J]. 协和医学杂志, 2012, 3: 302-305. doi: 10.3969/j.issn.1674-9081.2012.03.012 [30] Seuser A, Djambas Khayat C, Negrier C, et al. Evaluation of early musculoskeletal disease in patients with haemo-philia: results from an expert consensus[J]. Blood Coagul Fibrinolysis, 2018, 29: 509-520. doi: 10.1097/MBC.0000000000000767 [31] Hanley J, McKernan A, Creagh MD, et al. Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia: a United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO) guideline[J]. Haemophilia, 2017, 23: 511-520. doi: 10.1111/hae.13201 [32] Rodriguez-Merchan EC, De la Corte-Rodriguez H. Complications of hemophilia in the elbow: current management[J]. Expert Rev Hematol, 2020, 13: 991-1001. doi: 10.1080/17474086.2020.1803061 [33] 陈丽霞. 血友病骨关节病变的物理治疗[J]. 继续医学教育, 2006, 20: 89-93. https://www.cnki.com.cn/Article/CJFDTOTAL-JYJY200630023.htm [34] 杜威, 李要春, 唐银波, 等. 血友病性慢性疼痛的综合康复治疗[J]. 中国现代医学杂志, 2019, 29: 117-120. https://www.cnki.com.cn/Article/CJFDTOTAL-ZXDY201907023.htm [35] Escobar MA, Brewer A, Caviglia H, et al. Recommendations on multidisciplinary management of elective surgery in people with haemophilia[J]. Haemophilia, 2018, 24: 693-702. doi: 10.1111/hae.13549 [36] 童培建, 翁习生, 杨仁池, 等. 中国血友病性骨关节病髋膝关节置换围手术期管理指南[J]. 中华骨与关节外科杂志, 2022, 15: 481-490. https://www.cnki.com.cn/Article/CJFDTOTAL-ZGJW202207001.htm [37] Wu Y, Xue H, Zhang W, et al. Application of enhanced recovery after surgery in total knee arthroplasty in patients with haemophilia A: a pilot study[J]. Nurs Open, 2020, 8: 80-86. [38] 高娜, 佟冰渡, 张燕, 等. 血友病性骨关节病患者关节置换术围术期管理策略的应用效果研究[J]. 中国护理管理, 2019, 19: 607-611. https://www.cnki.com.cn/Article/CJFDTOTAL-GLHL201904030.htm [39] Negrier C, Seuser A, Forsyth A, et al. The benefits of exercise for patients with haemophilia and recommendations for safe and effective physical activity[J]. Haemophilia, 2013, 19: 487-498. [40] 陈丽霞, 吴润晖, 张光宇, 等. 本体感觉训练对儿童血友病患者下肢靶关节出血频率的影响[J]. 中国康复医学杂志, 2010, 25: 340-342. https://www.cnki.com.cn/Article/CJFDTOTAL-ZGKF201004015.htm [41] Runkel B, Von Mackensen S, Hilberg T. RCT-subjective physical performance and quality of life after a 6-month programmed sports therapy (PST) in patients with haemophilia[J]. Haemophilia, 2017, 23: 144-151. [42] Bladen M, Carroll L, Dodd C, et al. Results of feasibility and safety of randomised controlled trial of a musculoskeletal exercise intervention versus usual care for children with haemophilia[J]. Haemophilia, 2020, 26: e223-e225. -
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