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因子 XI 缺乏症的麻醉影响:一项临床病例研究及文献综述

Anesthetic Implications of Factor XI Deficiency: A Clinical Case Study and Review of Literature.

作者信息

Martinez-Lopez Pedro-Rafael, Barroso-Gonzalez Alejandro

机构信息

Anesthesiology and Reanimation, Hospital Regional Universitario of Málaga, Malaga, ESP.

出版信息

Cureus. 2024 Oct 28;16(10):e72594. doi: 10.7759/cureus.72594. eCollection 2024 Oct.

DOI:10.7759/cureus.72594
PMID:39478767
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11524335/
Abstract

Factor XI deficiency, a rare but significant coagulopathy, poses unique challenges in perioperative management, particularly in obstetric settings. This review provides an in-depth exploration of the pathophysiology, diagnosis, and anesthetic implications of factor XI deficiency, thereby emphasizing the useful role of anesthesiologists. The variable bleeding phenotype of the disorder necessitates a nuanced understanding and tailored management strategies to mitigate severe perioperative bleeding risks. Conventional coagulation tests, while useful, often fall short in predicting bleeding risks, underscoring the importance of advanced diagnostic tools, such as viscoelastic testing. Viscoelastic testing provides real-time data on clot stability, which allows for immediate intervention and more targeted therapeutic strategies compared to standard coagulation tests. A clinical case of a 25-year-old patient with factor XI deficiency undergoing emergency surgery for an ectopic pregnancy illustrates the application of viscoelastic testing in managing acute bleeding and optimizing patient outcomes and advocates for the development of standardized protocols, continuous monitoring techniques, and enhanced training programs to improve the perioperative care of patients with factor XI deficiency, providing anesthesiologists with the tools necessary to navigate the complexities of factor XI deficiency in the perioperative environment. Integrating these advanced diagnostic and therapeutic approaches could significantly improve patient safety and surgical outcomes in patients with complex coagulopathy.

摘要

因子 XI 缺乏症是一种罕见但严重的凝血病,在围手术期管理中带来独特挑战,尤其是在产科环境中。本综述深入探讨了因子 XI 缺乏症的病理生理学、诊断及麻醉影响,从而强调麻醉医生的重要作用。该疾病多变的出血表型需要细致的理解和定制化管理策略,以降低围手术期严重出血风险。传统凝血试验虽有用,但在预测出血风险方面往往不足,凸显了如粘弹性检测等先进诊断工具的重要性。粘弹性检测可提供有关血凝块稳定性的实时数据,与标准凝血试验相比,能实现即时干预和更具针对性的治疗策略。一名 25 岁因子 XI 缺乏症患者因异位妊娠接受急诊手术的临床病例,说明了粘弹性检测在处理急性出血和优化患者结局方面的应用,并倡导制定标准化方案、持续监测技术及强化培训项目,以改善因子 XI 缺乏症患者的围手术期护理,为麻醉医生提供在围手术期应对因子 XI 缺乏症复杂性所需的工具。整合这些先进的诊断和治疗方法可显著提高复杂凝血病患者的患者安全性和手术结局。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9c22/11524335/4c602b552957/cureus-0016-00000072594-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9c22/11524335/dc0f7902a580/cureus-0016-00000072594-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9c22/11524335/6c31436ba7d5/cureus-0016-00000072594-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9c22/11524335/4c602b552957/cureus-0016-00000072594-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9c22/11524335/dc0f7902a580/cureus-0016-00000072594-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9c22/11524335/6c31436ba7d5/cureus-0016-00000072594-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9c22/11524335/4c602b552957/cureus-0016-00000072594-i03.jpg

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本文引用的文献

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Perioperative management and neuraxial analgesia in women with factor XI deficiency (<60 IU/dL): a French multicenter observational study of 314 pregnancies.伴有因子 XI 缺乏(<60 IU/dL)女性的围手术期管理与神经轴索镇痛:一项针对 314 例妊娠的法国多中心观察性研究
Res Pract Thromb Haemost. 2024 May 27;8(4):102462. doi: 10.1016/j.rpth.2024.102462. eCollection 2024 May.
2
The role of viscoelastic hemostatic assays for postpartum hemorrhage management and bedside intrapartum care.用于产后出血管理和产时床边护理的黏弹性止血检测的作用。
Am J Obstet Gynecol. 2024 Mar;230(3S):S1089-S1106. doi: 10.1016/j.ajog.2022.09.008. Epub 2023 Aug 23.
3
Factor XI deficiency: phenotypic age-related considerations and clinical approach towards bleeding risk assessment.
因子 XI 缺乏症:表型与年龄相关的考虑因素及出血风险评估的临床方法。
Blood. 2024 Apr 11;143(15):1455-1464. doi: 10.1182/blood.2023020721.
4
Viscoelastic testing: Critical appraisal of new methodologies and current literature.黏弹性测试:新方法和现有文献的批判性评价。
Int J Lab Hematol. 2023 Oct;45(5):643-658. doi: 10.1111/ijlh.14144. Epub 2023 Aug 9.
5
Role of Viscoelastic and Conventional Coagulation Tests for Management of Blood Product Replacement in the Bleeding Patient.黏弹性和常规凝血试验在出血患者血液制品替代管理中的作用。
Semin Thromb Hemost. 2022 Oct;48(7):785-795. doi: 10.1055/s-0042-1756192. Epub 2022 Sep 29.
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A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More.心脏手术期间严重因子 XI 缺乏症的病例报告:少即是多。
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