先天性纤维蛋白原缺乏症儿童的扁桃体腺样体切除病例分析1例及文献复习

doi:10.6040/j.issn.1673-3770.0.2024.514

摘要/Abstract

摘要： 目的探讨先天性低纤维蛋白原血症(congenital hypofibrinogenemia, CFD)患者腺样体扁桃体切除术的围手术期管理策略。方法回顾性分析1例CFD患者行等离子腺样体扁桃体切除术的围手术期管理,并结合相关文献进行分析。结果围手术期纤维蛋白原替代治疗使患者的纤维蛋白原水平从0.23 g/L提升至0.92 g/L,顺利完成腺样体扁桃体切除术。术后患者恢复良好,未出现任何出血并发症。文献检索显示,目前尚未有关于CFD患者行腺样体扁桃体切除术的报道。然而在CFD患者接受其他手术时,纤维蛋白原补充的目标水平通常为0.5~1.0 g/L或适当提高。结论 CFD患者通过有效的围手术期纤维蛋白原替代治疗,可以安全地进行腺样体扁桃体切除术。

关键词: 先天性纤维蛋白原缺乏症, 扁桃体肥大, 腺样体肥大, 腺样体扁桃体切除术

Abstract: Objective To investigate the perioperative management strategies for adenotonsillectomy in pediatric patients with Congenital Hypofibrinogenemia(CFD). Methods We reviewed and analyzed the perioperative management of a pediatric patient with CFD who underwent adenotonsillectomy using plasma ablation at our hospital, in conjunction with a review of the relevant literature. Results With perioperative fibrinogen replacement therapy, the patient's fibrinogen level was successfully raised from 0.23 g/L to 0.92 g/L, allowing for the safe performance of adenotonsillectomy. The patient recovered well postoperatively without any bleeding complications. A literature search revealed no previous reports of adenotonsillectomy in patients with CFD. However, the target fibrinogen level for supplementation in CFD patients undergoing other surgical procedures is typically 0.5-1.0 g/L or appropriately higher. Conclusion Adenotonsillectomy can be safely performed in pediatric patients with CFD through effective perioperative fibrinogen replacement therapy.

Key words: Congenital hypofibrinogenemia, Tonsil hypertrophy, Adenoidal hypertrophy, Tonsillectomy &, adenoidectomy

中图分类号:

R762.3

王尧,陈静怡,杨颖超,苏开明. 先天性纤维蛋白原缺乏症儿童的扁桃体腺样体切除病例分析1例及文献复习[J]. 山东大学耳鼻喉眼学报, 2025, 39(3): 77-80.

WANG Yao, CHEN Jingyi, YANG Yingchao, SU Kaiming. Adenotonsillectomy in a child with congenital fibrinogen deficiency and literature review[J]. Journal of Otolaryngology and Ophthalmology of Shandong University, 2025, 39(3): 77-80.

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