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一名遗传性因子XI缺乏症(丙型血友病)患者的麻醉经验:病例报告。

Anesthetic experience of a patient with hereditary factor XI deficiency (Hemophilia C) : A case report.

作者信息

Kim Yee Suk, Chung Eun Yong, Yoon Jun Rho, Han In Soo, Park Ah Reum, Kim Tae Kwan, Lee Chul Woo

机构信息

Department of Anesthesiology and Pain Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.

出版信息

Korean J Anesthesiol. 2009 Jun;56(6):706-708. doi: 10.4097/kjae.2009.56.6.706.

Abstract

Factor XI deficiency (also called Hemophilia C) rarely occurs among ethnicities other than Ashkenazi Jews. A boy was scheduled for frontoethmoidectomy due to bilateral chronic rhinosinusitis. He was incidentally found to have factor XI deficiency due to prolonged aPTT on preoperative laboratory finding. His medical history reveals frequent epistaxis 2 or 3 times per day and his factor XI and XII activity were 17% (normal; 60-140%) and 34% (normal; 60-140%), respectively on furthermore laboratory evaluation. He was diagnosed as hereditary factor XI deficiency. He underwent the operation with administration of the fresh frozen plasma without complication.

摘要

除阿什肯纳兹犹太人外,其他种族很少发生因子XI缺乏症(也称为血友病C)。一名男孩因双侧慢性鼻窦炎计划进行额筛窦切除术。术前实验室检查发现其活化部分凝血活酶时间(aPTT)延长,偶然发现他患有因子XI缺乏症。他的病史显示每天经常鼻出血2至3次,进一步实验室评估显示他的因子XI和因子XII活性分别为17%(正常范围60 - 140%)和34%(正常范围60 - 140%)。他被诊断为遗传性因子XI缺乏症。他在输注新鲜冰冻血浆的情况下接受了手术,未出现并发症。

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