凝血障碍的生存分析:一项回顾性研究,随访5年
Arman Jahangiri1, Sara Ahmadi2, Hassan Rafieemehr3
1Department of Emergency Medicine, School of Medicine, Hamadan University of Medical Sciences, Hamadan, Iran.
Heliyon
|June 5, 2023
概括
这项研究评估了罕见出血障碍 (RBD) 患者5年生存率. 虽然生存率各不相同,但识别高风险个体可以通过及时干预改善结果.
科学领域:
- 血液学 血液学 血液学
- 罕见疾病 罕见疾病
- 凝血障碍 凝血障碍 凝血障碍
背景情况:
- 罕见的出血障碍 (RBDs) 涉及凝血因子缺乏.
- 有效的患者监测是管理RBD的一个重大挑战.
研究的目的:
- 评估诊断为罕见出血障碍的患者的五年生存率.
- 根据凝血因子缺乏的类型来确定生存的潜在差异.
主要方法:
- 在146名RBD患者中进行了一项描述性的横截面研究.
- 卡普兰-梅尔曲线被用来评估五年随访期间的生存率.
- 逻辑等级测试比较了不同因素缺陷的生存率.
主要成果:
- 总体生存率为54.42±1.3个月.
- 第八因子 (FVIII) 缺乏是最常见的 (65.8%),而FXIII缺乏是最罕见的 (4.8%).
- 虽然FXIII缺乏症的生存率较低 (44±9.6个月),但组之间没有发现统计学上显著的差异 (P ≥0.05).
结论:
- 在罕见的出血障碍中,患者的生存率表现出变化.
- 识别高风险患者对于通过及时的治疗干预来提高生存率至关重要.
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