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并发因子 VII 和因子 XI 缺乏症表现为自发性下肢骨筋膜室综合征的病例。

Case of concurrent factor VII and factor XI deficiencies manifesting as spontaneous lower extremity compartment syndrome.

作者信息

Marshalek Joseph P, Yashar David, Huynh Karen, Tomassetti Sarah

机构信息

Department of Internal Medicine Harbor-UCLA Medical Center Torrance California USA.

Division of Hematology/Oncology Department of Internal Medicine Harbor-UCLA Medical Center Torrance California USA.

出版信息

Clin Case Rep. 2022 Apr 25;10(4):e05710. doi: 10.1002/ccr3.5710. eCollection 2022 Apr.

DOI:10.1002/ccr3.5710
PMID:35498352
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9036193/
Abstract

Factor VII and XI deficiencies are rare bleeding disorders typically associated with mild or provoked bleeding. This case report describes a patient with factor VII and XI deficiencies with an unprovoked episode of lower extremity hematoma causing compartment syndrome requiring multiple surgeries, extensive transfusion of blood products, and ultimately amputation.

摘要

凝血因子VII和XI缺乏症是罕见的出血性疾病,通常与轻度出血或诱发性出血有关。本病例报告描述了一名患有凝血因子VII和XI缺乏症的患者,其下肢无缘无故出现血肿,导致骨筋膜室综合征,需要进行多次手术、大量输注血液制品,最终进行了截肢。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28b9/9036193/302007a79fd3/CCR3-10-e05710-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28b9/9036193/302007a79fd3/CCR3-10-e05710-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28b9/9036193/302007a79fd3/CCR3-10-e05710-g001.jpg

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本文引用的文献

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Conversion Total Hip Arthroplasty Following Failed Hip Fracture Fixation in a Patient with Factor XI Deficiency: A Case Report.11因子缺乏患者髋部骨折内固定失败后行全髋关节置换术:一例报告
J Orthop Case Rep. 2020 May-Jun;10(3):80-84. doi: 10.13107/jocr.2020.v10.i03.1760.
2
Hemophilia C: A Case Report With Updates on Diagnosis and Management of a Rare Bleeding Disorder.血友病C:一例罕见出血性疾病诊断与治疗进展的病例报告
J Hematol. 2019 Sep;8(3):144-147. doi: 10.14740/jh522. Epub 2019 Sep 30.
3
Pharmacokinetics and pharmacodynamics of a recombinant fusion protein linking activated coagulation factor VII with human albumin (rVIIa-FP) in patients with congenital FVII deficiency.
重组融合蛋白(活化凝血因子VII与人白蛋白连接体,rVIIa-FP)在先天性FVII缺乏症患者中的药代动力学和药效学
Hematology. 2020 Dec;25(1):17-25. doi: 10.1080/16078454.2019.1700329.
4
A new perspective on perioperative coagulation management in a patient with congenital factor VII deficiency: A case report.先天性因子 VII 缺乏症患者围手术期凝血管理的新视角:一例报告
Medicine (Baltimore). 2018 Nov;97(44):e12776. doi: 10.1097/MD.0000000000012776.
5
Abnormal plasma clot formation and fibrinolysis reveal bleeding tendency in patients with partial factor XI deficiency.部分因子 XI 缺乏症患者的血浆异常凝结形成和纤维蛋白溶解揭示了出血倾向。
Blood Adv. 2018 May 22;2(10):1076-1088. doi: 10.1182/bloodadvances.2017015123.
6
Successful perioperative management in a patient with factor XI deficiency.XI因子缺乏患者的围手术期成功管理。
BMJ Case Rep. 2018 Feb 21;2018:bcr-2017-222434. doi: 10.1136/bcr-2017-222434.
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Haemophilia. 2018 Mar;24(2):e50-e55. doi: 10.1111/hae.13378. Epub 2017 Nov 27.
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Exploring the global landscape of genetic variation in coagulation factor XI deficiency.探索凝血因子 XI 缺乏症的全球遗传变异景观。
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9
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