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患有中度先天性第七因子缺乏症患者的外科手术期间的挑战:一个病例报告
Tahira El Ansari1, Hassane Mamad1, Souad Benkirane1
1Hematology, Ibn Sina University Hospital, Mohammed V University of Rabat, Rabat, MAR.
Cureus
|December 15, 2025
概括
遗传性VII因子缺乏症会带来不可预测的出血风险,即使因子水平中等. 使用复合激活因子VII (rFVIIa) 和胺酸进行个性化的术后管理对于安全的手术至关重要.
科学领域:
- 血液学 血液学 血液学
- 遗传学 遗传学 是一个
- 内部医学 内部医学
背景情况:
- 遗传性第七因子 (FVII) 缺乏症是一种罕见的出血障碍,具有不可预测的临床变异性.
- 血FVII活性与出血严重程度之间的联系往往很差,使手术管理复杂化.
- 适度的FVII缺陷可能会在侵入性手术期间带来显著的出血风险.
研究的目的:
- 在患有中度先天性FVII缺陷的患者中报告一个成功的术后管理的案例.
- 突出针对FVII缺陷的手术干预进行量身定制的管理策略的重要性.
主要方法:
- 一名25岁的男性患有中度FVII缺乏症 (FVII:C 39%) 接受了大脑切除.
- 术后治疗包括复合激活因子VII (rFVIIa) 在15-25μg/kg IV,松酸和局部静血.
- 手术前的实验室证实了单独的中度FVII缺陷与长时间PT (57%的活动) 和正常的aPTT比率 (1.14).
主要成果:
- 根据实施的外科手术期间的协议,患者可以很好地忍受腹切除.
- 没有观察到术后出血或血栓性并发症.
- 在拔牙后之前的严重出血与成功的手术结果形成对比.
结论:
- 中度先天性FVII缺陷需要仔细,个性化的术后规划.
- 结合rFVIIa,抗纤维解质疗法和局部静血,可确保手术的安全性.
- 这一案例强调了多学科方法在手术期间管理出血障碍时的有效性.
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