印度血友病患者的死亡率:国家队列研究
Shrimati Shetty1, Cecil Ross2, M Joseph John3
1Advanced Center for Oncology, Hematology and Rare Disorders (ACOHRD), K.J. Somaiya Super Speciality Hospital & Research Center, Somaiya Ayurvihar, Sion East, Mumbai, Maharashtra, India.
Haemophilia : the official journal of the World Federation of Hemophilia
|December 31, 2024
概括
内出血 (ICH) 是印度血友病患者的首要死亡原因,特别是那些在插曲性治疗中患有严重疾病的人. 改进的管理策略对于增加血友病 (PWH) 患者的预期寿命至关重要.
科学领域:
- 血液学 血液学 血液学
- 公共卫生 公共卫生
- 流行病学 流行病学
背景情况:
- 全球在诊断和治疗方面的进步已经降低了血友病患者 (PWH) 的死亡率和发病率.
- 然而,这些改进的影响在不同国家并非均,突出显示了护理方面的区域差异.
研究的目的:
- 评估印度PWH中所有原因和内出血 (ICH) 特定死亡率.
- 确定与该人口死亡率相关的关键因素.
主要方法:
- 一项回顾性,观察性,多中心的队列研究,涉及来自印度三个中心的1020名血友病患者.
- 通过个人采访收集的死亡率数据,并通过血友病治疗中心 (HTC) 确认.
- 从医疗记录中提取了人口统计,临床数据和并发症.
主要成果:
- 内出血 (ICH) 是死亡的主要原因 (42.9%),其次是意外/创伤 (25.9%) 和胃肠道出血 (15.9%).
- 死亡的患者年轻 (平均年龄为27.7岁),并且主要是没有预防或免疫耐受性诱导 (ITI) 治疗的严重血友病.
- 与一般血友病患者相比,在已故患者中,抑制剂和高血压的患病率明显高 (p < 0.05).
结论:
- 严重的血友病,偶发性治疗,高血压和抑制剂与增加的死亡率有显著的关联.
- 在印度,ICH仍然是PWH的主要死亡原因,这强调了疾病管理的挑战.
- 改善治疗策略对于提高印度PWH的预期寿命至关重要.
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