Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences RSCI, Dublin, Ireland.
World Federation of Hemophilia, Montreal, Quebec, Canada.
Haemophilia. 2023 Jul;29(4):1104-1112. doi: 10.1111/hae.14804. Epub 2023 May 22.
Recent guidelines for von Willebrand Disease (VWD) highlighted the challenges in diagnosis and management. Identifying the number of persons with VWD (PwVWD) internationally will help target support to aid diagnosis of PwVWD.
To examine international registration rates of PwVWD, the influence of income status, geographical region and the age and sex profile. Cumulatively, these data will be used to inform future strategy from the World Federation of Haemophilia (WFH) to address unmet clinical and research needs.
Data from the 2018/2019 WFH Annual Global Survey (AGS) were analysed, providing a global perspective on VWD registration.
Registration rates are lowest in South Asia (0.6/million population) and highest in Europe/Central Asia (50.9/million population, 0.005%), but below the expected prevalence rate (0.1%). National economic status impacted VWD registration rates, reflecting variation in access to optimal healthcare infrastructure. Females represented the majority of PwVWD globally, however, in low-income countries (LIC) males predominated. Age profile varied, with markedly higher rates of paediatric registrations in North America, Middle East and North Africa and South Asia. Rates of type 3 VWD registrations were significantly influenced by economic status (81% of VWD diagnoses in LIC), suggesting only the most severe VWD types are diagnosed in resource limited settings.
Significant variation in registration rates of PwVWD exist internationally and is influenced by income status and the presence of HTC networks. Improved understanding of registration rates will enable targeting of advocacy to improve awareness, diagnosis and support for PwVWD internationally.
Registration rates of People with Von Willebrand Disease (PwVWD) vary internationally and are influenced by national income status Although females represent the majority of PwVWD globally, in low income countries (LIC) males predominated, possibly related to stigma surrounding gynaecological bleeding. Rates of type 3 VWD registration were significantly influenced by economic status (81% of VWD diagnoses in LIC), suggesting only the most severe VWD types are diagnosed in resource limited settings.
最近发布的《血管性血友病（VWD）指南》强调了该病诊断和治疗方面存在的挑战。明确国际上 VWD 患者（PwVWD）的数量有助于为 PwVWD 的诊断提供支持。
检查国际上 PwVWD 的登记率，以及收入状况、地理位置和年龄与性别分布的影响。综合这些数据，将为世界血友病联盟（WFH）提供信息，制定未来策略以满足未满足的临床和研究需求。
对 2018/2019 年 WFH 年度全球调查（AGS）的数据进行了分析，从全球角度了解 VWD 的登记情况。
南亚的登记率最低（0.6/百万人口），欧洲/中亚最高（50.9/百万人口，0.005%），但低于预期流行率（0.1%）。国家经济状况影响 VWD 登记率，反映了获得最佳医疗保健基础设施的机会存在差异。全球范围内女性是 PwVWD 的主要人群，但在低收入国家（LIC）中男性居多。年龄分布不同，北美、中东和北非以及南亚的儿科登记率明显较高。3 型 VWD 的登记率受经济状况显著影响（LIC 中 81%的 VWD 诊断），这表明仅在资源有限的环境中诊断出最严重的 VWD 类型。
国际上 PwVWD 的登记率存在显著差异，受收入状况和 HTC 网络的影响。更好地了解登记率将使有针对性地开展宣传活动成为可能，以提高国际上对 PwVWD 的认识、诊断和支持。
国际上 PwVWD 的登记率存在差异，受国家收入状况影响。虽然全球范围内女性是 PwVWD 的主要人群，但在低收入国家（LIC）中男性居多，这可能与妇科出血相关的耻辱感有关。3 型 VWD 的登记率受经济状况显著影响（LIC 中 81%的 VWD 诊断），这表明仅在资源有限的环境中诊断出最严重的 VWD 类型。
