缺乏XIII因子:一个沉默的出血者背后一个正常的凝血形状
Ramesh Yadav1, Madhav Raj Karki2, Dikshya Pokhrel2
1Department of Paediatrics Birat Medical College Teaching Hospital Morang Nepal.
Clinical case reports
|March 9, 2026
概括
一种罕见的出血障碍XIII因子缺乏症,可以通过标准测试错过. 及时诊断和新鲜冷血 (FFP) 治疗对于管理出血并发症至关重要.
科学领域:
- 血液学 血液学 血液学
- 儿科医学 儿科医学
- 罕见疾病 罕见疾病
背景情况:
- 十三因子缺乏症是一种不常见的遗传性出血障碍.
- 婴儿可能会出现轻微创伤或自发性严重出血.
研究的目的:
- 要突出一个婴儿的XIII因子缺乏病例.
- 强调诊断挑战和管理策略.
主要方法:
- 一个8个月大的男婴持续出血的案例报告.
- 常规凝血测试正常;进行了特定测试 (尿液凝块溶解度,XIII因子测定).
- 管理包括新鲜冷血 (FFP) 和支持性护理.
主要成果:
- 婴儿表现出长时间的出血,皮和.
- 尽管正常的常规凝血参数,但XIII因子缺乏被证实.
- 这位患者通过FFP治疗成功从外周血瘤中恢复过来.
结论:
- 诊断XIII因子缺乏症需要除了常规的凝血测试之外进行特定的测试.
- 及时诊断和FFP管理对于预防严重出血并发症至关重要.
- 即使在资源有限的环境中,也可以进行有效的管理.
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