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血友病 A 的社会负担。I - 血友病 A 在澳大利亚及其他地区的概况。

The societal burden of haemophilia A. I - A snapshot of haemophilia A in Australia and beyond.

机构信息

NATSEM, Institute for Governance and Policy Analysis, University of Canberra, Canberra, ACT, Australia.

Roche Products Pty Limited, Sydney, NSW, Australia.

出版信息

Haemophilia. 2020 Aug;26 Suppl 5:3-10. doi: 10.1111/hae.14102.

Abstract

INTRODUCTION

Few studies, both in Australia and overseas, have examined the social impacts of living with haemophilia A (HA) or the economic costs associated with the disorder. The purpose of this paper is to examine the epidemiology and societal burden of people with HA (PwHA) in Australia, with a particular focus on men with this disorder.

METHODS

The epidemiology and societal burden of HA in Australia, with a particular focus on men with this disorder, were assessed, using data available in the Australian and international literature and publicly available data.

RESULTS

The mean annual prevalence of HA is approximately 1-2 per 10 000 males. Prophylactic treatment is used in one-quarter (25.1%) of people with moderate HA, and 82.2% of people with severe HA. Within the latter group, 16.1% have inhibitors for Factor VIII, predisposing them to worse morbidity, mortality and quality of life when compared to the non-inhibitor population. Joint pain and joint disease occur commonly in PwHA, with up to 70% of adults with HA experiencing joint problems. HA is associated with poor physical health, and PwHA miss school and work due to bleeding-related events.

CONCLUSION

HA is associated with substantial economic burden; with large differences in costs reported between countries. Overall, HA imposes a significant burden of disease on PwHA, their families and the community at large.

摘要

简介

在澳大利亚和海外,很少有研究探讨血友病 A(HA)患者的社会影响或与该疾病相关的经济成本。本文旨在研究澳大利亚 HA 患者(PwHA)的流行病学和社会负担,特别关注患有这种疾病的男性。

方法

使用澳大利亚和国际文献中的现有数据以及公开可用的数据,评估了澳大利亚 HA 的流行病学和社会负担,特别关注患有这种疾病的男性。

结果

HA 的年平均患病率约为每 10000 名男性中有 1-2 名。四分之一(25.1%)中度 HA 患者和 82.2%重度 HA 患者接受预防性治疗。在后一组中,16.1%的人有针对凝血因子 VIII 的抑制剂,这使他们在发病率、死亡率和生活质量方面比非抑制剂人群更差。关节疼痛和关节疾病在 PwHA 中很常见,多达 70%的 HA 成年人有关节问题。HA 与身体健康状况不佳有关,PwHA 因出血相关事件而缺课和旷工。

结论

HA 与巨大的经济负担相关;不同国家报告的成本存在较大差异。总体而言,HA 给 PwHA、他们的家庭和整个社区带来了巨大的疾病负担。

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