{"title":"因子 XI 缺乏症的麻醉影响:临床病例研究与文献综述。","authors":"Pedro-Rafael Martinez-Lopez, Alejandro Barroso-Gonzalez","doi":"10.7759/cureus.72594","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":93960,"journal":{"name":"Cureus","volume":null,"pages":null},"PeriodicalIF":1.0000,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11524335/pdf/","citationCount":"0","resultStr":"{\"title\":\"Anesthetic Implications of Factor XI Deficiency: A Clinical Case Study and Review of Literature.\",\"authors\":\"Pedro-Rafael Martinez-Lopez, Alejandro Barroso-Gonzalez\",\"doi\":\"10.7759/cureus.72594\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":93960,\"journal\":{\"name\":\"Cureus\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.0000,\"publicationDate\":\"2024-10-28\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11524335/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Cureus\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.7759/cureus.72594\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/10/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Cureus","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.7759/cureus.72594","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/10/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}
引用次数: 0
摘要
因子 XI 缺乏症是一种罕见但重要的凝血病,给围术期管理带来了独特的挑战,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、诊断和对麻醉的影响,从而强调了麻醉医师的作用。由于该疾病的出血表型多变,因此有必要对其进行细致入微的了解并制定有针对性的管理策略,以降低严重的围术期出血风险。传统的凝血测试虽然有用,但往往无法预测出血风险,这凸显了粘弹性测试等先进诊断工具的重要性。粘弹性测试可提供有关血凝块稳定性的实时数据,与标准凝血测试相比,这种测试可提供即时干预和更有针对性的治疗策略。一名 25 岁的因子 XI 缺乏症患者因宫外孕接受急诊手术的临床病例说明了粘弹性测试在控制急性出血和优化患者预后方面的应用,并倡导制定标准化方案、持续监测技术和强化培训计划,以改善因子 XI 缺乏症患者的围手术期护理,为麻醉医生提供必要的工具,以应对围手术期环境中因子 XI 缺乏症的复杂性。整合这些先进的诊断和治疗方法可以大大提高复杂凝血病患者的安全性和手术效果。
Anesthetic Implications of Factor XI Deficiency: A Clinical Case Study and Review of Literature.
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.