二因子和五因子缺陷:当前的管理,护理缺口和呼吁采取行动,以获得治疗进展
Samantha Pasca1, Massimo Franchini2, Pier Mannuccio Mannucci3
1Immunohematology and Transfusion Department, Azienda Sanitaria Universitaria Integrata del Trentino (ASUIT), Trento, Italy.
概括
血栓激素和V因子缺乏有效的治疗方法,阻碍了预防和出血事件的管理. 目前的治疗方法是不理想的,需要简化开发新的因子缩剂和替代治疗方法.
科学领域:
- 血液学 血液学 血液学
- 罕见的遗传性凝血障碍 罕见的遗传性凝血障碍
- 因子替代疗法是指因子替代治疗.
背景情况:
- 血栓激素 (因子II) 和因子V (FV) 缺乏是罕见的遗传性凝血障碍 (RICD).
- 患有这些缺陷的患者无法获得单因素替代产品,造成了显著的临床护理差距.
- 这种差距阻碍了预防和偶发性治疗策略的实施.
研究的目的:
- 进行叙述性审查,评估RICD中出血预防的实施情况.
- 评估目前可用的治疗药物的有效性,新鲜冷血和前素复合物缩剂,用于预防和偶发性治疗.
- 审查急性出血发作和突发血栓和V因子缺乏症患者的侵入性手术的结果.
主要方法:
- 发表的案例报告和文献的叙事审查.
- 针对前列血和V因子缺陷的预防实施的分析.
- 对出血发作和侵入性手术的治疗结果的评估.
主要成果:
- 即使在严重出血事件后,对前列血和V因子缺乏症的预防措施的实施有限.
- 很少有报道有效治疗急性出血事件的病例,特别是在前列血缺乏症.
- 在手术或侵袭性手术期间对偶发性治疗的数据稀缺.
结论:
- 目前治疗前热血素和V因子缺乏症的治疗选择在很大程度上是不理想的.
- 对于开发单因子缩剂的监管途径进行简化至关重要.
- 需要进一步评估凝血再平衡,预防的非替代产品.
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