血管性血友病患者的关节出血对生活质量和关节完整性有重大影响：一项横断面研究。
Joint bleeds in von Willebrand disease patients have significant impact on quality of life and joint integrity: a cross-sectional study.
机构信息
Department of Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
Department of Haematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands.
出版信息
Haemophilia. 2015 May;21(3):e185-e192. doi: 10.1111/hae.12670. Epub 2015 Apr 9.
BACKGROUND
Joint bleeds (JB) are reported in a minority of patients with von Willebrand disease (VWD) but may lead to structural joint damage. Prevalence, severity and impact of JB in VWD are largely unknown.
OBJECTIVES
The aim of this study was to assess JB prevalence, onset, treatment and impact on health-related quality of life (HR-QoL) and joint integrity in moderate and severe VWD.
METHODS
In the Willebrand in the Netherlands study 804 moderate and severe VWD patients [von Willebrand factor (VWF) activity ≤30U dL(-1)] completed a questionnaire on occurrence, sites and consequences of JB. To analyse JB number, onset, treatment and impact on joint integrity we additionally performed a patient-control study on medical file data comparing patients with JB to age, gender, factor VIII (FVIII)- and VWF activity matched VWD patients without JB.
RESULTS
Of all VWD patients 23% (184/804) self-reported JB. These 184 patients reported joint damage more often (54% vs. 18%, P < 0.001) and had lower HR-QoL (SF36, P < 0.05) compared to VWD patients not reporting JB. Of 55 patients with available JB data, 65% had the first JB before age 16. These 55 patients used more clotting factor concentrate (CFC; median dose 43 vs. 0 IE FVIII kg(-1) year(-1) , P < 0.001), more often had X-ray joint damage (44% vs. 11%, P = 0.001] and chronic joint pain (44% vs. 18%, P = 0.008) compared to 55 control VWD patients without JB.
CONCLUSION
In conclusion, joint bleeds are reported by 23% of moderate and severe VWD patients, mostly start in childhood, are associated with more CFC use, joint pain, lower HR-QoL and significantly more radiological and self-reported joint damage.
背景
关节出血（JB）在少数血管性血友病（VWD）患者中报告，但可能导致结构性关节损伤。VWD 中 JB 的患病率、严重程度和影响在很大程度上尚不清楚。
目的
本研究旨在评估中度和重度 VWD 中 JB 的患病率、发病、治疗以及对健康相关生活质量（HR-QoL）和关节完整性的影响。
方法
在荷兰的 VWF 研究中，804 名中度和重度 VWD 患者[血管性血友病因子（VWF）活性≤30U dL（-1）]完成了一份关于 JB 发生、部位和后果的调查问卷。为了分析 JB 的数量、发病、治疗和对关节完整性的影响，我们还对病历数据进行了患者对照研究，将 JB 患者与年龄、性别、VIII 因子（FVIII）和 VWF 活性匹配、无 JB 的 VWD 患者进行比较。
结果
所有 VWD 患者中，23%（184/804）自述有 JB。这些 184 名患者报告关节损伤更频繁（54% vs. 18%，P < 0.001），HR-QoL 更低（SF36，P < 0.05），与无 JB 的 VWD 患者相比。在 55 名有 JB 数据的患者中，65%的患者在 16 岁之前首次出现 JB。这些 55 名患者使用更多的凝血因子浓缩物（CFC；中位数剂量 43 vs. 0 IE FVIII kg（-1）年（-1），P < 0.001），更频繁地出现 X 射线关节损伤（44% vs. 11%，P = 0.001）和慢性关节疼痛（44% vs. 18%，P = 0.008），与 55 名无 JB 的对照 VWD 患者相比。
结论
总之，中度和重度 VWD 患者中有 23%报告了关节出血，大多数在儿童时期开始，与更多的 CFC 使用、关节疼痛、较低的 HR-QoL 以及明显更多的影像学和自我报告的关节损伤相关。
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