巴西A型血友病患者的年均出血率:系统性审查
Alessandra Nl Prezotti1, Débora Mc Rocha1, Endi L Galvão2
1Centro de Hematologia e Hemoterapia do Espírito Santo, Vitória, ES, Brazil.
Hematology, transfusion and cell therapy
|March 29, 2025
概括
这次审查发现巴西严重血友病A治疗的数据有限,强调需要更好地收集关于出血发作和遵守第八因子预防的数据.
科学领域:
- 血液学 血液学 血液学
- 药理学 药理学是指药理学的学科.
背景情况:
- 血友病A是一种遗传性出血障碍,由VIII因子缺乏引起.
- 根据第八因子 (FVIII) 活性水平对患者进行分类:严重 (<1%),中度 (1-5%) 和轻度 (6-40%).
- 预防是标准的护理,但仍然会发生血关节炎和关节损伤.
研究的目的:
- 为了评估严重血友病A患者在巴西没有抑制剂的治疗结果.
- 评估在预防治疗的患者中出血事件的频率和治疗坚持程度.
主要方法:
- 从2014年开始对MEDLINE,Embase,LILACS和SciElo数据库进行系统文献审查.
- 包括的研究集中在接受预防的患者的出血发作和坚持率.
- 五个出版物符合描述性审查的资格标准.
主要成果:
- 搜索了536篇文章,选了417篇,并评估了104篇的全文评论.
- 只有五篇出版物被选中用于最终的描述性审查.
- 审查发现现有数据稀缺.
结论:
- 目前关于巴西严重血友病A管理的信息有限,可能有偏见.
- 在巴西,对血友病A患者进行结构化数据收集,分析和报告非常需要.
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