A study of variations in the reported haemophilia A prevalence around the world
A study of variations in the reported haemophilia A prevalence around the world
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DOI：
10.1111/j.1365-2516.2009.02127.x
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发表时间：
2010-01-01
期刊：
影响因子：
3.9
通讯作者：
Brooker, M.
中科院分区：
文献类型：
--
作者：
Stonebraker, J. S.;Bolton-Maggs, P. H. B.;Brooker, M.
本文的目的是研究报告的血友病A患病率（每100 000名男性）在一个国家的基础上，并解决以下问题：报告的血友病A患病率是否因国家经济而异？我们从世界血友病联合会（WFH）年度全球调查和文献中收集了106个国家的患病率数据。我们发现，报告的血友病A患病率在不同国家之间差异很大，即使是在最富裕的国家。高收入国家的患病率（每10万男性）为12.8 +/- 6.0（平均值+/- SD），而世界其他地区为6.6 +/- 4.8。在一个国家内，随着时间的推移，流行率呈明显上升趋势-加拿大的流行率从1989年的10.2上升到2008年的14.2（R = 0.94和P < 0.001），联合王国的流行率从1974年的9.3上升到2006年的21.6（R = 0.94和P < 0.001）。WFH报告的患病率数据与文献中的患病率数据进行了比较。患者登记通常提供最高质量的患病率数据。缺乏准确的国家特异性患病率数据限制了血友病A患者治疗和护理的规划工作。随着信息的改善，医疗机构可以评估预算需求，为受影响的患者和家庭开发更好的诊断和治疗设施，并努力确保足够的治疗用凝血因子VIII浓缩物的供应。此外，这些信息还可以帮助制造商规划浓缩物的生产并防止未来的短缺。
The objectives of this paper were to study the reported haemophilia A prevalence (per 100 000 males) on a country-by-country basis and address the following: Does the reported prevalence of haemophilia A vary by national economies? We collected prevalence data for 106 countries from the World Federation of Hemophilia (WFH) annual global surveys and the literature. We found that the reported haemophilia A prevalence varied considerably among countries, even among the wealthiest of countries. The prevalence (per 100 000 males) for high income countries was 12.8 +/- 6.0 (mean +/- SD) whereas it was 6.6 +/- 4.8 for the rest of the world. Within a country, there was a strong trend of increasing prevalence over time - the prevalence for Canada ranged from 10.2 in 1989 to 14.2 in 2008 (R = 0.94 and P < 0.001) and for the United Kingdom it ranged from 9.3 in 1974 to 21.6 in 2006 (R = 0.94 and P < 0.001). Prevalence data reported from the WFH compared well with prevalence data from the literature. Patient registries generally provided the highest quality of prevalence data. The lack of accurate country-specific prevalence data has constrained planning efforts for the treatment and care of people with haemophilia A. With improved information, healthcare agencies can assess budgetary needs to develop better diagnostic and treatment facilities for affected patients and families and work to ensure adequate supplies of factor VIII concentrates for treatment. In addition, this information can help manufacturers plan the production of concentrates and prevent future shortages.