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血浆前激肽释放酶缺乏导致活化部分凝血活酶时间延长:一项病例研究及其临床意义的文献综述

Prolonged activated partial thromboplastin time due to plasma prekallikrein deficiency: a case study and literature review on its clinical significance.

作者信息

Zhou Kehua, Mehedint Diana, Khadim Haider

机构信息

Catholic Health System Internal Medicine Training Program, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo.

General Physician PC, Williamsville, New York, USA.

出版信息

Blood Coagul Fibrinolysis. 2019 Sep;30(6):300-303. doi: 10.1097/MBC.0000000000000837.

DOI:10.1097/MBC.0000000000000837
PMID:31318719
Abstract

: Activated partial thromboplastin time is a test assessing the intrinsic and common pathways of the coagulation cascade. We presented an asymptomatic case with isolated activated partial thromboplastin time prolongation. After excluding coagulation factor deficiency and lupus anticoagulant, the patient was diagnosed with plasma prekallikrein (PPK) deficiency. We reviewed the literature regarding effects of PPK deficiency which could have both antithrombotic and prothrombotic effects. At the moment, research supports that PPK deficiency in healthy adults rarely causes bleeding as it is not a major contributor of hemastasis; whereas in adults with multiple comorbidities or with predominant systemic inflammation, effects of PPK deficiency remain debatable. Further research is needed to clarify impacts of PPK deficiency in clinical settings.

摘要

活化部分凝血活酶时间是一项评估凝血级联反应内源性和共同途径的检测。我们报告了一例孤立性活化部分凝血活酶时间延长的无症状病例。在排除凝血因子缺乏和狼疮抗凝物后,该患者被诊断为血浆前激肽释放酶(PPK)缺乏。我们回顾了关于PPK缺乏影响的文献,其可能具有抗血栓形成和促血栓形成两种作用。目前,研究表明健康成年人中的PPK缺乏很少导致出血,因为它不是止血的主要因素;而在患有多种合并症或主要存在全身炎症的成年人中,PPK缺乏的影响仍存在争议。需要进一步研究以阐明PPK缺乏在临床环境中的影响。

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Prolonged activated partial thromboplastin time due to plasma prekallikrein deficiency: a case study and literature review on its clinical significance.血浆前激肽释放酶缺乏导致活化部分凝血活酶时间延长:一项病例研究及其临床意义的文献综述
Blood Coagul Fibrinolysis. 2019 Sep;30(6):300-303. doi: 10.1097/MBC.0000000000000837.
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A Rare Cause of Isolated Prolonged Activated Partial Thromboplastin Time: An Overview of Prekallikrein Deficiency and the Contact System.罕见的孤立性延长部分凝血活酶时间原因:激肽释放酶原缺乏和接触系统概述。
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Long-term safety outcomes of prekallikrein (Fletcher factor) deficiency: A systematic literature review of case reports.遗传性高分子量激肽原缺乏症(Fletcher 因子缺乏症)的长期安全性结局:病例报告的系统文献回顾。
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Prekallikrein (Fletcher factor) deficiency.前激肽释放酶(弗莱彻因子)缺乏症。
Ann Clin Lab Sci. 1985 Jul-Aug;15(4):279-85.
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Prekallikrein deficiency.前激肽释放酶缺乏症
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Prekallikrein deficiency: the characteristic normalization of the severely prolonged aPTT following increased preincubation time is due to autoactivation of factor XII.前激肽释放酶缺乏症:预孵育时间延长后,严重延长的活化部分凝血活酶时间(aPTT)出现特征性正常化是由于因子 XII 的自动激活。
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Fletcher factor deficiency with mildly prolonged activated PTT.弗莱彻因子缺乏伴活化部分凝血活酶时间轻度延长。
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Fletcher factor deficiency, source of variations of the activated partial thromboplastin time test.弗莱彻因子缺乏症,活化部分凝血活酶时间试验结果变异的原因。
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Definite diagnosis of plasma prekallikrein deficiency should not be based exclusively on shortening of the aPTT upon prolonged pre-incubation.血浆前激肽释放酶缺乏症的明确诊断不应仅基于长时间预孵育后活化部分凝血活酶时间(aPTT)的缩短。
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Complete prekallikrein deficiency masquerading as a lupus anticoagulant.表现为狼疮抗凝物的完全性前激肽释放酶缺乏症。
Thromb Res. 2014 Feb;133(2):301-2. doi: 10.1016/j.thromres.2013.11.014. Epub 2013 Nov 22.

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The Influence of Plasma Prekallikrein Oligonucleotide Antisense Therapy on Coagulation and Fibrinolysis Assays: A Post-hoc Analysis.血浆前激肽释放酶寡核苷酸反义疗法对凝血和纤溶检测的影响:一项事后分析
Thromb Haemost. 2022 Dec;122(12):2045-2049. doi: 10.1055/a-1926-2367. Epub 2022 Aug 17.
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Severe Prekallikrein Deficiency Associated with Low Level of Factor XII: A Case Report.严重前激肽释放酶缺乏症伴低水平的因子 XII:一例报告
Iran J Pathol. 2021 Summer;16(3):332-336. doi: 10.30699/IJP.2020.131638.2463. Epub 2021 May 9.
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Resolving Differential Diagnostic Problems in von Willebrand Disease, in Fibrinogen Disorders, in Prekallikrein Deficiency and in Hereditary Hemorrhagic Telangiectasia by Next-Generation Sequencing.
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Prevalence of Cardiovascular Disorders in African-Americans With Congenital Prekallikrein Deficiency Versus Caucasians-Americans With the Same Defect.先天性前激肽释放酶缺乏的非裔美国人与具有相同缺陷的高加索裔美国人中心血管疾病的患病率。
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