Joint status and related risk factors in patients with severe hemophilia A: a single-center cross-sectional study
Joint status and related risk factors in patients with severe hemophilia A: a single-center cross-sectional study
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严重甲型血友病患者关节状况及相关危险因素：单中心横断面研究
DOI：
10.1080/16078454.2021.2019892
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发表时间：
2021-12
期刊：
影响因子：
1.9
通讯作者：
Peng Jie
中科院分区：
文献类型：
--
作者：
Zhao Liang;Yang Hongbin;Li Yaochun;Wang Zhongqing;Zhou Li;Zhao Xielan;Peng Jie
摘要目的血友病性关节病是重症血友病A最常见的并发症。本研究旨在调查重度血友病A（PWSHA）患者的关节状况和相关危险因素。方法采用单中心研究方法，共纳入31例患者。使用中国血友病早期关节病超声检测（HEAD-US-C）和血友病关节健康评分（HJHS）评价了6个索引关节（肘部、膝盖和脚踝）。使用经验证的血友病方案治疗依从性量表-预防（VERITAS-Pro）测量治疗依从性。我们分析了年龄、治疗延迟（诊断和开始治疗之间的间隔）、预防和治疗依从性对关节结局的影响。结果所有患者均为男性（中位年龄22岁）。诊断时的中位年龄为1岁;初始治疗时的中位年龄为5岁。所有患者均发生关节出血。HEAD-US-C与HJHS评分呈正相关（R = 0.70，P < 0.0001）。中位[范围] HEAD-US-C和HJHS评分分别为15 [0-36]和32 [2-49]。年龄与HEAD-US-C（P = 0.002）和HJHS评分（P < 0.0001）呈正相关。治疗延迟≤1年和>1年组之间的HEAD-US-C评分差异接近显著性（P = 0.055）。两组HJHS评分差异有统计学意义（P = 0.03）。按需治疗组和低剂量预防治疗组的关节评估评分无显著差异。VERITAS-Pro评分与HEAD-US-C和HJHS评分相关（分别为P = 0.046和P = 0.005）。结论血友病性关节病在PWSHA中普遍存在。年龄和粘附性差与关节损伤显著相关。及时治疗和依从性改善可能会降低严重程度。
ABSTRACT Objectives Hemophilic arthropathy is the most common complication of severe hemophilia A. This study aims to investigated joint status and related risk factors in patients with severe hemophilia A (PWSHA). Methods This single-center study included 31 patients. Six index joints (both elbows, knees, and ankles) were evaluated using the Hemophilia Early Arthropathy Detection with UltraSound in China (HEAD-US-C) and Hemophilia Joint Health Score (HJHS). Treatment adherence was measured using the Validated Hemophilia Regimen Treatment Adherence Scale-Prophylaxis (VERITAS-Pro). We analyzed the influence of age, treatment delay (the interval between diagnosis and the initiation of treatment), prophylaxis, and treatment adherence on joint outcomes. Results All patients were male (median age, 22 years). The median age at diagnosis was 1 year; that at initial treatment was 5 years. All patients experienced joint bleeding. HEAD-US-C and HJHS scores were positively correlated (R = 0.70, P < 0.0001). Median [range] HEAD-US-C and HJHS scores were 15 [0-36] and 32 [2-49], respectively. Age was positively correlated with both HEAD-US-C (P = 0.002) and HJHS scores (P < 0.0001). The difference of HEAD-US-C scores between groups with ≤1 year and >1 year treatment delay was close to significant (P = 0.055). HJHS scores were significantly different between these two groups (P = 0.03). Joint assessment scores were not significantly different between on-demand and low-dose prophylaxis groups. VERITAS-Pro scores were correlated with both HEAD-US-C and HJHS scores (P = 0.046 and P = 0.005, respectively). Conclusions Hemophilic arthropathy was pervasive in PWSHA. Age and poor adherence were significantly correlated with joint damage. Prompt treatment and adherence improvement may reduce severity.
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DOI：
10.1111/hae.13275
发表时间：
2017-07
期刊：
Haemophilia : the official journal of the World Federation of Hemophilia
影响因子：
--
作者：
Soucie JM;Grosse SD;Siddiqi AE;Byams V;Thierry J;Zack MM;Shapiro A;Duncan N;Hemophilia Treatment Centers Network
通讯作者：
Hemophilia Treatment Centers Network
影响因子：
3.9
作者：
Duncan, N.;Kronenberger, W.;Shapiro, A.
通讯作者：
Shapiro, A.
影响因子：
3.9
作者：
Rodriguez-Merchan, E. C.;Jimenez-Yuste, V.;Caffarini, A.
通讯作者：
Caffarini, A.
影响因子：
6.7
作者：
Martinoli, Carlo;Alberighi, Ornella Della Casa;Morfini, Massimo
通讯作者：
Morfini, Massimo
影响因子：
3.9
作者：
A. Shapiro
通讯作者：
A. Shapiro