Generation and validation of the Condensed MCMDM-1VWD Bleeding Questionnaire for von Willebrand disease
Generation and validation of the Condensed MCMDM-1VWD Bleeding Questionnaire for von Willebrand disease
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DOI：
10.1111/j.1538-7836.2008.03182.x
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发表时间：
2008-12-01
影响因子：
10.4
通讯作者：
James, P.
中科院分区：
文献类型：
--
作者：
Bowman, M.;Mundell, G.;James, P.
背景：考虑到获得准确出血史所涉及的挑战，标准化的尝试已经出现，量化出血症状的价值已被认识到。患者/方法：通过消除所有不直接影响出血评分（BS）的细节，简化了一份经过广泛验证的出血问卷（MCMDM-1VWD），并测试了两个版本之间的相关性。此外，对浓缩版的诊断效用进行了前瞻性测试。结果：这里展示了259名接受问卷调查的人的数据；217人正在接受血管性血友病（VWD）的前瞻性调查（1组），42人先前已知患有1、2或3型血管性血友病（2组）。在217例前瞻性调查中，35例BS阳性（>= 4），182例阴性。7人（均为BS阳性）实验室结果符合1型VWD。结果灵敏度为100%，特异性为87%。阳性预测值为0.20，阴性预测值为1。完整的MCMDM-1VWD与浓缩版本之间的相关性非常好（Spearman's 0.97, P < 0.001，线性回归r(2) = 96.4）。精简版的观察者间信度合理（Spearman's 0.72, P < 0.001，类内相关系数0.805,P < 0.001）。不同VWD亚型间BS有显著差异，3型>型>型>型>型VWD （anovaP < 0.001）。VWF:Ag水平与BS呈显著负相关（Spearman’s -0.411, P < 0.001）。结论：浓缩版MCMDM-1VWD出血问卷是评估VWD患者的有效工具。
Background: Given the challenges involved in obtaining accurate bleeding histories, attempts at standardization have occurred and the value of quantifying hemorrhagic symptoms has been recognized. Patients/methods: An extensive validated bleeding questionnaire (MCMDM-1VWD) was condensed by eliminating all details that did not directly affect the bleeding score (BS) and the correlation between the two versions was tested. Additionally, the diagnostic utility of the condensed version was prospectively tested. Results: Data on 259 individuals who were administered the questionnaire are presented here; 217 being prospectively investigated for von Willebrand disease (VWD) (group 1) and 42 previously known to have type 1, 2 or 3 VWD (group 2). Of the 217 prospectively investigated, 35 had positive BS (>= 4) and 182 had negative scores. Seven individuals (all with positive BS) had laboratory results consistent with type 1 VWD. This results in a sensitivity of 100% and a specificity of 87%. The positive predictive value is 0.20 and the negative predictive value is 1. The correlation between the full MCMDM-1VWD and condensed versions is excellent (Spearman's 0.97, P < 0.001, linear regression r(2) = 96.4). Inter-observer reliability for the condensed version is reasonable (Spearman's 0.72, P < 0.001 and intra-class correlation coefficient 0.805, P < 0.001). There was a significant difference in BS between subtypes of VWD, with type 3 >> type 2 >> type 1 VWD (anovaP < 0.001). There is a strong inverse relationship between VWF:Ag level and BS (Spearman's -0.411, P < 0.001). Conclusions: The Condensed MCMDM-1VWD Bleeding Questionnaire is an efficient, effective tool in the evaluation of patients for VWD.