HAEMOPHILIA：血友病患者的全因死亡率和死亡原因: 系统评价和META分析

2021-10-05 MedSci原创 MedSci原创

A型血友病和B型血友病分别是由因子VIII(FVIII)和因子IX(FIX)缺乏引起的遗传性出血疾病

A型血友病和B型血友病分别是由因子VIII(FVIII)和因子IX(FIX)缺乏引起的遗传性出血疾病。凝血因子替代术是预防和治疗出血的主要治疗方法。在引入凝血因子浓缩物之前，血友病(PWH)患者的平均预期寿命为<30年，主要是由于出血。经历了过去几十年血友病治疗的改进也让血友病患者 (PWH) 的预期寿命增加。

在制定当代优化血友病护理的优先事项时，了解死亡原因的流行病学变化是重要的。许多基于人群的队列研究报告称，与一般人群相比，PWH的标准化死亡率(SMR)范围广泛。这可能与不同的时代、不同的医疗资源和血友病严重程度分布的异质性有关。国外一团队对其进行了系统评价和meta分析，以评估过去几十年来PWH中全因和全因特异性SMR的时间趋势。此外，还探讨了该人群中不同死亡原因的比例。并假设，由于预防措施的不断采用和支持性护理的进展，血友病人群和普通人群之间的死亡率差距随着时间的推移越来越窄。其文章刊登在HAEMOPHILIA上。

该团队根据流行病学观察性研究的meta分析指南对文献进行了系统的回顾，系统地检索了 EMBASE、MEDLINE、Web of Science、CINAHL 和 Cochrane 从开始到 2021 年 3 月 15 日的对照试验中央登记册。纳入了报告 PWH 与一般人群相比的死亡率估计值和/或报告的死因的研究。使用具有逆方差方法的随机效应荟萃分析来获得汇总估计值。并且按队列进入年份（2000 年之前与 2000 年之后）对分析进行分层。

在确定的 4769 项研究中，52 项符合资格标准。PWH 中 9 项研究的汇总全因标准化死亡率 (SMR) 为 1.93（95% CI 1.38–2.70；I ² = 97%）。2000 年之前和之后的合并 SMR 分别为 2.40（95% CI 1.92–3.00；I ² = 87%）和 1.20（95% CI 1.03–1.40；I ² = 62%）。在 2000 年之前，31.2% 的死亡是由 HIV 导致的，其次是出血 (26.0%)、心血管疾病 (18.2%)、肝病 (9.0%) 和癌症 (8.9%)。2000 年后，由于出血导致的死亡比例保持不变，因 HIV 导致的死亡人数减少（13.9%）。

综上得出结论，随着治疗的进步，PWH 的死亡率在过去几十年中已经下降，接近普通人群的死亡率。然而，出血仍然是需要进一步关注的主要死亡原因

原始出处：

Alam, AUl, Karkhaneh, M, Attia, T, Wu, C, Sun, H (L). All-cause mortality and causes of death in persons with haemophilia: A systematic review and meta-analysis. Haemophilia. 2021; 1- 14. https://doi.org/10.1111/hae.14423

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2022-01-12 一闲

#MET#

59 0
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2021-12-17 changfy

#HAE#

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2022-03-26 guojianrong

#Meta#

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2021-12-30 tigerlr98

#死亡原因#

42 0
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2021-10-07 老龙一点

学习

64 0
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2021-10-07 lvliquan

#全因死亡率#

54 0
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2021-10-07 huagfeg

#评价#

40 0
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2021-10-06 与狼共舞

学习学习

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