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凝血因子 XIII 缺乏产妇的椎管内镇痛管理:一例病例报告及建议的管理算法

Management of Neuraxial Analgesia in a Parturient with Factor XIII Deficiency: A Case Report and Proposed Management Algorithm.

作者信息

Carroll David B, Myler Conrad, Songdej Natthapol, Sedeek Khaled, Bezinover Dmitri

机构信息

Department of Anesthesiology and Perioperative Medicine, Milton S. Hershey Medical Canter, 500 University Dr, Hershey, PA 17033, USA.

Department of Medicine, Division of Hematology and Oncology, Milton S. Hershey Medical Center, 500 University Dr, Hershey, PA 17033, USA.

出版信息

Case Rep Anesthesiol. 2020 Dec 31;2020:8892225. doi: 10.1155/2020/8892225. eCollection 2020.

DOI:10.1155/2020/8892225
PMID:33489380
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7790575/
Abstract

Factor XIII (FXIII) deficiency is a rare coagulation defect that can be associated with significant bleeding. A 28-year-old pregnant woman, with a history of hemorrhagic stroke secondary to severe congenital FXIII deficiency, presented in active labor requesting an epidural. Factor XIII levels had been monitored throughout her pregnancy and treated with intermittent factor XIII infusions to maintain factor levels above 30% of normal. After careful multidisciplinary peripartum evaluation and FXIII replacement, neuraxial analgesia was performed without complication. Neuraxial analgesia can be performed without complication in patients with FXIII deficiency if FXIII levels are carefully managed and no other coagulopathy exists.

摘要

因子 XIII(FXIII)缺乏是一种罕见的凝血缺陷,可伴有严重出血。一名 28 岁的孕妇,有严重先天性 FXIII 缺乏继发出血性中风病史,在活跃期分娩时要求进行硬膜外麻醉。在整个孕期监测了 FXIII 水平,并通过间歇性输注 FXIII 进行治疗,以维持因子水平高于正常水平的 30%。经过仔细的多学科围产期评估和 FXIII 替代治疗后,进行了神经轴镇痛,未出现并发症。如果 FXIII 水平得到仔细管理且不存在其他凝血病,FXIII 缺乏患者可进行神经轴镇痛且无并发症。

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本文引用的文献

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Updated Australian consensus statement on management of inherited bleeding disorders in pregnancy.更新的澳大利亚妊娠期间遗传性出血性疾病管理共识声明。
Med J Aust. 2019 Apr;210(7):326-332. doi: 10.5694/mja2.50123. Epub 2019 Mar 29.
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Acquired factor XIII deficiency: A review.获得性因子 XIII 缺乏症:综述
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Minimal factor XIII activity level to prevent major spontaneous bleeds.预防自发性大出血的最小因子 XIII 活性水平。
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Coagulation factor activity and clinical bleeding severity in rare bleeding disorders: results from the European Network of Rare Bleeding Disorders.罕见出血性疾病的凝血因子活性和临床出血严重程度:来自欧洲罕见出血性疾病网络的结果。
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