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先天性因子V缺乏症患者的流产和复发性流产:伊朗6例报告

Miscarriage and recurrent miscarriage in patients with congenital factor V deficiency: a report of six cases in Iran.

作者信息

Naderi Majid, Tabibian Shadi, Shamsizadeh Morteza, Dorgalaleh Akbar

机构信息

Department of Pediatrics Haematology and Oncology, Ali Ebn-e Abitaleb Hospital Research Center for Children and Adolescents Health [RCCAH], Zahedan University of Medical Sciences, Zahedan, Iran.

Department of Haematology and Blood Transfusion, School of Allied Medical Sciences, Iran University of Medical Sciences, Tehran, Iran.

出版信息

Int J Hematol. 2016 Jun;103(6):673-5. doi: 10.1007/s12185-016-1981-7. Epub 2016 Mar 15.

DOI:10.1007/s12185-016-1981-7
PMID:26980222
Abstract

Miscarriage and recurrent miscarriage have not been reported in women with congenital factor V (FV) deficiency. Here we describe cases of both miscarriage and recurrent miscarriage in women with congenital FV deficiency (FVD). We investigated six women with FVD from the southeast of Iran who had experienced miscarriage and recurrent miscarriage. Consequent diagnosis was made by routine coagulation tests as well as FV activity and antigen assays. To evaluate the presence of an inhibitor, a mixing study via prothrombin time (PT) assay was performed. All patients were investigated, and found to be negative for antiphospholipid syndrome. Demographic data and clinical presentations were obtained by standard questionnaire. The factor assays determined that all six women were suffering from moderate FVD. One had experienced eight miscarriages, while the others experienced two (two patients), three, and four episodes. Only one patient had a single miscarriage. Three of the women experienced successful delivery without medical intervention. Miscarriage and recurrent miscarriage should be considered as possible presentations of FVD to prevent its life-threatening consequences.

摘要

先天性因子V(FV)缺乏的女性中尚未有流产和复发性流产的报道。在此,我们描述了先天性FV缺乏(FVD)女性发生流产和复发性流产的病例。我们调查了来自伊朗东南部的6名患有FVD且经历过流产和复发性流产的女性。通过常规凝血试验以及FV活性和抗原检测进行确诊。为评估抑制剂的存在,通过凝血酶原时间(PT)检测进行了混合试验。对所有患者进行了调查,发现抗磷脂综合征均为阴性。通过标准问卷获取人口统计学数据和临床表现。因子检测确定所有6名女性均患有中度FVD。其中1名经历了8次流产,其他女性分别经历了2次(2名患者)、3次和4次流产。只有1名患者有1次流产。3名女性在未进行医学干预的情况下成功分娩。应将流产和复发性流产视为FVD可能的表现形式,以防止其危及生命的后果。

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引用本文的文献

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Recurrent miscarriage in a woman with congenital factor V deficiency: a case report.先天性因子 V 缺乏症妇女的复发性流产:病例报告。
BMC Pregnancy Childbirth. 2022 Dec 8;22(1):915. doi: 10.1186/s12884-022-05273-y.

本文引用的文献

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Establishment of a prenatal diagnosis schedule as part of a prophylaxis program of factor XIII deficiency in the southeast of Iran.制定产前诊断时间表,作为伊朗东南部 XIII 因子缺乏症预防计划的一部分。
Blood Coagul Fibrinolysis. 2016 Jan;27(1):97-100. doi: 10.1097/MBC.0000000000000374.
2
Morbidity and mortality in a large number of Iranian patients with severe congenital factor XIII deficiency.大量伊朗严重先天性凝血因子 XIII 缺乏症患者的发病率和死亡率。
Ann Hematol. 2016 Feb;95(3):451-5. doi: 10.1007/s00277-015-2568-8. Epub 2015 Dec 22.
3
Factor XIII deficiency in Iran: a comprehensive review of the literature.
伊朗的因子 XIII 缺乏症:文献综述。
Semin Thromb Hemost. 2015 Apr;41(3):323-9. doi: 10.1055/s-0034-1395350. Epub 2015 Jan 23.
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Congenital factor V deficiency: comparison of the severity of clinical presentations among patients with rare bleeding disorders.先天性因子V缺乏症:罕见出血性疾病患者临床表现严重程度的比较。
Acta Haematol. 2015;133(2):148-54. doi: 10.1159/000363598. Epub 2014 Sep 26.
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Clinical manifestations and management of life-threatening bleeding in the largest group of patients with severe factor XIII deficiency.最大组严重因子XIII缺乏症患者危及生命出血的临床表现及处理
Int J Hematol. 2014 Nov;100(5):443-9. doi: 10.1007/s12185-014-1664-1. Epub 2014 Sep 18.
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Haemophilia. 2009 Sep;15(5):990-1005. doi: 10.1111/j.1365-2516.2009.01984.x. Epub 2009 Feb 27.
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Haemophilia. 2005 Jan;11(1):26-30. doi: 10.1111/j.1365-2516.2005.01056.x.