[一种自免疫性获得的XIII因子缺乏症病例,由复发的术后出血诊断出来]
Yasushi Takashima1, Hiroaki Kawanishi1, Shinichi Kotani2
1The Department of Urology, Tenri Hospital.
Hinyokika kiyo. Acta urologica Japonica
|July 17, 2023
概括
手术后严重出血,最初无法解释,与获得的XIII因子缺乏有关. 用皮质类固醇治疗成功解决了这种情况,强调在持续出血的情况下考虑这种罕见疾病的重要性.
科学领域:
- 泌尿器科 泌尿器科 泌尿器科 泌尿器科
- 血液学 血液学 血液学
- 手术并发症 手术并发症
背景情况:
- 尿道管缩是布里克尔管管道手术后的一个已知的并发症.
- 手术后可能出现出血,但在正常凝血测试下持续和严重出血需要进一步调查.
研究的目的:
- 报告在尿道形解剖修复后出现严重,不明原因的术后出血.
- 突出诊断挑战和成功管理自身免疫性获得的XIII因子缺乏症.
主要方法:
- 进行内分泌治疗,然后进行动脉横断栓塞 (TAE) 治疗出血.
- 切除术和探索性腹腔切除术用于持续出血.
- 诊断工作包括凝血测试,XIII因子活性测定和交叉混合研究.
- 用血衍生的XIII因子治疗,然后用皮质类固醇治疗.
主要成果:
- 最初的干预措施 (TAE,切除术,腹腔切除术) 无法控制出血.
- 确认了自身免疫性获得的XIII因子缺乏症的诊断.
- 皮质类固醇治疗导致XIII因子活性迅速增加,并停止出血.
结论:
- 在患有严重,不明原因出血的患者中,尽管正常的常规凝血形状,应该怀疑获得的XIII因子缺乏症.
- 及时诊断和适当的治疗,包括用皮质类固醇抑制免疫力,对于管理这种情况至关重要.
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