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确定阿富汗血友病患者的死因。

Determining causes of death among individuals with haemophilia in Afghanistan.

作者信息

Mousavi Seyed Hamid, Arif Shamim, Madadi Shekiba, Mansouritorghabeh Hassan

机构信息

Medical Research Centre, Kateb University, Kabul, Afghanistan.

Afghanistan National Charity Organization for Special Diseases (ANCOSD), Kabul, Afghanistan.

出版信息

Haemophilia. 2022 Jan;28(1):86-90. doi: 10.1111/hae.14462. Epub 2021 Nov 26.

DOI:10.1111/hae.14462
PMID:34837287
Abstract

INTRODUCTION

Haemophilia is a well-known bleeding disorder that affects people worldwide. The main therapeutic strategy is regular infusion of exogenous factor VIII to ensure an optimal haemostatic standard. Morbidity and mortality of individuals with haemophilia has decreased in developing countries due to improvement in early detection, advanced treatments, and comprehensive population outreach efforts. However, individuals with bleeding disorders in developing countries like Afghanistan do not have access to such therapeutic facilities.

AIMS

The goals of this study were to determine the causes of death and findings related to death among in individuals with bleeding disorders in Afghanistan.

METHODS

This study conducted as a retrospective cross-sectional study of 387 individuals with bleeding disorders (mainly haemophilia) in Afghanistan.

RESULTS

All registered individuals with bleeding disorders in Afghanistan were interviewed by telephone. Among the 387 individuals with bleeding disorders, there were 136 deaths. Most deaths occurred in individuals aged 1-15 years (66.2%). Intracranial haemorrhage was the leading cause of death in individuals with haemophilia A and B.

CONCLUSION

These findings revealed that supply of coagulation factor concentrates, facilities for haemostasis diagnosis, and trained haematologists is inadequate in Afghanistan.

摘要

引言

血友病是一种广为人知的出血性疾病,影响着世界各地的人们。主要治疗策略是定期输注外源性凝血因子 VIII 以确保达到最佳止血标准。由于早期检测的改善、先进治疗方法以及全面的人群宣传工作,发展中国家血友病患者的发病率和死亡率有所下降。然而,在阿富汗等发展中国家,出血性疾病患者无法获得此类治疗设施。

目的

本研究的目的是确定阿富汗出血性疾病患者的死亡原因及与死亡相关的发现。

方法

本研究作为一项回顾性横断面研究,对阿富汗 387 例出血性疾病患者(主要是血友病患者)进行了研究。

结果

通过电话对阿富汗所有登记在册的出血性疾病患者进行了访谈。在 387 例出血性疾病患者中,有 136 例死亡。大多数死亡发生在 1 - 15 岁的个体中(66.2%)。颅内出血是 A 型和 B 型血友病患者的主要死亡原因。

结论

这些发现表明,阿富汗的凝血因子浓缩物供应、止血诊断设施以及训练有素的血液学家数量不足。

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