关节功能状态对血友病患儿生存质量的影响探究Effect of joint function on quality of life in children with hemophilia
傅彩红;徐新;鲍莹;
摘要(Abstract):
目的分析关节功能状态对血友病患儿生存质量的影响,探究改善患儿生存质量的方法。方法选取本院2013年5月至2018年5月收治的94例血友病患儿为研究对象,使用中文版血友病关节健康评估表2.1(haemophilia joint health score 2.1,HJHS 2.1)评估其关节功能状态,使用中文版儿童生活质量测定量表4.0版(pediatric quality of life inventory version 4.0,Peds QL 4.0)、血友病功能独立性评分(functional independence score in haemophilia,FISH)评估其生存质量。采用Pearson相关性分析探讨血友病患儿HJHS 2.1评分与PedsQL 4.0评分、FISH的相关性,分析关节功能状态对患儿生存质量的影响。结果 94例患儿平均HJHS 2.1评分为(19.55±2.30)分,平均PedsQL 4.0评分为(1.82±0.63)分,平均FISH为(25.26±5.17)分。Pearson相关性分析示,PedsQL 4.0评分总分及各功能项评分与HJHS 2.1评分总分及各项目评分均呈正相关(P_均<0.05),FISH总分与HJHS 2.1评分总分及各项目评分均呈负相关(P_均<0.05)。结论血友病患儿关节功能状态普遍较差,随着患儿关节功能的恶化,其生活质量、日常生活活动能力明显下降,是影响患儿生存质量的重要原因,应考虑早期干预。
关键词(KeyWords): 关节功能;血友病;生存质量;日常生活活动能力
基金项目(Foundation):
作者(Authors): 傅彩红;徐新;鲍莹;
参考文献(References):
- [1]Kempton CL,Recht M,Neff A,et al.Impact of pain and functional impairment in US adults with haemophilia:Patientreported outcomes and musculoskeletal evaluation in the pain,functional impairment and quality of life(P-FiQ)study[J].Haemophilia,2018,24(2):261-270.
- [2]李晓,浦权.血友病的临床分型与实验室诊断[J].新医学,2002,33(9):563-564.
- [3]Witkop M,Neff A,Buckner TW,et al.Self-reported prevalence,description and management of pain in adults with haemophilia:methods,demographics and results from the Pain,Func tional Impairment,and Quality of life(P-FiQ)study[J].Haem ophilia,2017,23(4):556-565.
- [4]Schnohr C,Bacher T,Andersen T,et al.Joint Mobility and Physical Function of Danish Hemophilia Patients:A ThreeWave Panel Study Spanning 24 Years[J].Acta haematol,2018,140(4):240-246.
- [5]中华医学会血液学分会血栓与止血学组,中国血友病协作组.血友病诊断与治疗中国专家共识(2013年版)[J].中华血液学杂志,2013,34(5):461-463.
- [6]Sun J,Hilliard PE,Feldman BM,et al.Chinese Hemophilia Joint Health Score 2.1 reliability study[J].Haemophilia,2014,20(3):435-440.
- [7]Varni JW,Seid M,Kurtin PS.PedsQL 4.0:reliability and validity of the Pediatric Quality of Life Inventory version 4.0generic core scales in healthy and patient populations[J].Med Care,2001,39(8):800-812.
- [8]Tlacuilo-Parra A,Villela-Rodriguez J,Garibaldi-Covarrubias R,et al.Functional independence score in hemophilia:a crosssectional study assessment of Mexican children[J].Pediatr Blood Cancer,2010,54(3):394-397.
- [9]刘颖,赵宏,杜小娟,等.血友病儿童日常生活活动能力与关节健康状况的相关性研究[J].中华物理医学与康复杂志,2017,39(2):147-149.
- [10]Oldenburg J.Optimal treatment strategies for hemophilia:achievements and limitations of current prophylactic regimens[J].Blood,2015,125(13):2038-2044.
- [11]Manco-Johnson MJ,Lundin B,Funk S,et al.Effect of late prophylaxis in hemophilia on joint status:a randomized trial[J].J Thromb Haemost,2017,15(11):2115-2124.
- [12]St-Louis J,Urajnik DJ,Ménard F,et al.Generic and diseasespecific quality of life among youth and young men with Hemophilia in Canada[J].BMC Hematol,2016,16:13.
- [13]方拥军,洪恺.关节出血对血友病关节的影响[J].中国实用儿科杂志,2017,32(1):70-75.
- [14]王梦媛,姚敏,万丹丹,等.187例血友病患儿关节状况与生活质量分析[J].浙江医学,2017,39(9):736-739.
- [15]刘淑芬,刘颖,张建宏,等.血友病儿童45例关节健康状况分析及其与步态的相关性研究[J].中华临床医师杂志(电子版),2012,6(12):3389-3391.
- [16]van Galen KPM,Timmer MA,de Kleijn P,et al.Joint assessment in von Willebrand disease.Validation of the Haemophilia Joint Health score and Haemophilia Activities List[J].Thromb Haemost,2017,117(8):1465-1470.
- [17]Rodriguez-Merchan EC,Valentino LA.Joint lavage followed by intra-articular injection of hyaluronic acid and/or corticosteroids in patients with severe hemophilic arthropathy of the knee:Is this intervention really effective?[J].Expert Rev Hematol,2018,11(6):449-454.
- [18]Shima M,Hanabusa H,Taki M,et al.FactorⅧ-Mimetic Function of Humanized Bispecific Antibody in Hemophilia A[J].NEngl J Med,2016,374(21):2044-2053.