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Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease.1型血管性血友病患者血管性血友病因子水平及血管性血友病活性随年龄的变化
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von Willebrand disease and aging: an evolving phenotype.血管性血友病和衰老:一种不断演变的表型。
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Determinants of bleeding phenotype in adult patients with moderate or severe von Willebrand disease.成年中重度血管性血友病患者出血表型的决定因素。
Thromb Haemost. 2012 Oct;108(4):683-92. doi: 10.1160/TH12-04-0244. Epub 2012 Aug 23.
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Generation and validation of the Condensed MCMDM-1VWD Bleeding Questionnaire for von Willebrand disease.血管性血友病简易MCMDM-1VWD出血问卷的编制与验证
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The mutational spectrum of type 1 von Willebrand disease: Results from a Canadian cohort study.1型血管性血友病的突变谱:一项加拿大队列研究的结果。
Blood. 2007 Jan 1;109(1):145-54. doi: 10.1182/blood-2006-05-021105..
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Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD).在欧洲开展的“1型血管性血友病诊断与管理的分子和临床标志物研究(MCMDM-1VWD)”中,一组历史上被诊断为1型血管性血友病的家族的表型和基因型。
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A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 VWD).1型血管性血友病出血症状的定量分析:一项欧洲多中心研究(MCMDM-1 VWD)的结果
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1型血管性血友病中衰老与血管性血友病因子水平及出血风险的关联

The Association of Aging With Von Willebrand Factor Levels and Bleeding Risk in Type 1 Von Willebrand Disease.

作者信息

Seaman Craig D, Ragni Margaret V

机构信息

1 Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.

2 Hemophilia Center of Western Pennsylvania, Pittsburgh, PA, USA.

出版信息

Clin Appl Thromb Hemost. 2018 Apr;24(3):434-438. doi: 10.1177/1076029617724232. Epub 2017 Sep 6.

DOI:10.1177/1076029617724232
PMID:28874064
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6714664/
Abstract

Little is known about aging in von Willebrand disease (VWD). It is uncertain whether VWD patients experience an age-related increase in von Willebrand factor (VWF) levels, and if so, it is unknown whether normalization of VWF levels with aging ameliorates bleeding risk. We aimed to determine the association of age with VWF levels and bleeding risk in patients with type 1 VWD. This is a retrospective chart review of patients with type 1 VWD presenting to the Hemophilia Clinic of Western Pennsylvania for regularly scheduled clinic visits. Data collected included VWF antigen level and condensed molecular and clinical markers for the diagnosis and management of Type 1 (MCMDM-1) VWD bleeding assessment tool (BAT) bleeding score based on bleeding symptoms during the previous 3 years. Thirty-nine patients participated in the study, and 32 were female. The average age of participants was 41.8 ± 18.0 years. The mean VWF antigen level was 0.83 ± 0.37 IU/mL, and the mean bleeding score was 2.51 ± 2.90. The bleeding score was inversely associated with age, β = -0.080 (SE = 0.023), P < .01. There was a nonsignificant association between VWF antigen levels and age. To our knowledge, this is the first report showing an association between aging and decreased bleeding symptoms in patients with type 1 VWD. Determining whether or not bleeding risk is reduced in older patients with type 1 VWD is essential for optimal clinical management. Moreover, VWF concentrate is costly, and unwarranted use represents a significant waste of health-care dollars. These findings warrant further investigation.

摘要

关于血管性血友病(VWD)的衰老情况,人们了解甚少。目前尚不确定VWD患者的血管性血友病因子(VWF)水平是否会随年龄增长而升高,即便如此,也不清楚VWF水平随年龄增长而恢复正常是否能改善出血风险。我们旨在确定1型VWD患者的年龄与VWF水平及出血风险之间的关联。这是一项对前往宾夕法尼亚西部血友病诊所进行定期门诊的1型VWD患者的回顾性病历审查。收集的数据包括VWF抗原水平以及用于1型(MCMDM - 1)VWD诊断和管理的简明分子和临床标志物、基于过去3年出血症状的VWD出血评估工具(BAT)出血评分。39名患者参与了该研究,其中32名女性。参与者的平均年龄为41.8±18.0岁。VWF抗原水平的平均值为0.83±0.37 IU/mL,出血评分的平均值为2.51±2.90。出血评分与年龄呈负相关,β = -0.080(标准误 = 0.023),P < 0.01。VWF抗原水平与年龄之间存在无统计学意义的关联。据我们所知,这是首份显示1型VWD患者衰老与出血症状减轻之间存在关联的报告。确定老年1型VWD患者的出血风险是否降低对于优化临床管理至关重要。此外,VWF浓缩物成本高昂,不必要的使用会造成医疗保健资金的重大浪费。这些发现值得进一步研究。