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伊朗的一项横断面研究:杂合子 FXIII 缺乏症妇女与健康妇女产后出血的比较。

Postpartum Hemorrhage in Heterozygote Factor XIII Deficient Women Compared With Healthy Women. A Cross-Sectional Experience From Iran.

机构信息

48538Zahedan University of Medical Sciences, Zahedan, Iran.

Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

Clin Appl Thromb Hemost. 2021 Jan-Dec;27:10760296211051714. doi: 10.1177/10760296211051714.

DOI:10.1177/10760296211051714
PMID:34697946
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8552392/
Abstract

Postpartum hemorrhage (PPH) is a major cause of maternal mortality, which is a common clinical manifestation in women with rare bleeding disorders. In this study, we compare PPH and its complications in heterozygote factor XIII (FXIII) deficient women with healthy women. In this cross sectional case study, 50 women with heterozygote FXIII deficiency and 50 healthy women are evaluated. Data were initially collected by interviewing the women who were receiving FXIII replacement therapy after their childbirths. Data were analysed using SPSS (Version 22) and a -value of less than .05 was considered statistically significant. The mean age in the patient and control groups were 31.2 and 32.5 years respectively. The occurring rate of PPH in the patient group was significantly higher than the control group (34% vs 2%) (-value <.0001). None of the confounding variables such as maternal age, gestational age, numbers, and types of delivery in women with PPH showed any significant differences between the control and patient groups. According to the results of this study, the risk of PPH (early and late), miscarriage, and menorrhagia in women who are heterozygous for FXIII deficiency is significantly higher than healthy women. However, the effect of other factors such as maternal age, gestational age, number, and type of delivery require further studies to delineate any confounding factors.

摘要

产后出血(PPH)是产妇死亡的主要原因,在罕见出血性疾病的女性中是一种常见的临床表现。在这项研究中,我们比较了杂合子因子 XIII(FXIII)缺乏症女性与健康女性的 PPH 及其并发症。在这项横断面病例研究中,评估了 50 名杂合子 FXIII 缺乏症女性和 50 名健康女性。数据最初通过对产后接受 FXIII 替代治疗的女性进行访谈收集。使用 SPSS(版本 22)进行数据分析,p 值小于 0.05 被认为具有统计学意义。患者组和对照组的平均年龄分别为 31.2 岁和 32.5 岁。患者组的 PPH 发生率明显高于对照组(34%对 2%)(p 值<.0001)。在患有 PPH 的女性中,母体年龄、胎龄、分娩次数和类型等混杂变量均无显著差异。根据这项研究的结果,FXIII 杂合子缺乏症女性发生 PPH(早期和晚期)、流产和月经过多的风险明显高于健康女性。然而,其他因素(如母体年龄、胎龄、分娩次数和类型)的影响需要进一步研究以阐明任何混杂因素。

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引用本文的文献

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本文引用的文献

1
Practice Bulletin No. 183: Postpartum Hemorrhage.实践公告第 183 号:产后出血。
Obstet Gynecol. 2017 Oct;130(4):e168-e186. doi: 10.1097/AOG.0000000000002351.
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Update on inherited disorders of haemostasis and pregnancy.遗传性止血障碍与妊娠的最新进展
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Blood Coagul Fibrinolysis. 2016 Jan;27(1):97-100. doi: 10.1097/MBC.0000000000000374.
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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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Congenital factor XIII deficiency in women: a systematic review of literature.先天性因子 XIII 缺乏症在女性中的研究:文献系统综述。
Haemophilia. 2013 Nov;19(6):e349-57. doi: 10.1111/hae.12259. Epub 2013 Aug 28.
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Impact of inherited bleeding disorders on pregnancy and postpartum hemorrhage.遗传性出血性疾病对妊娠及产后出血的影响。
Blood Coagul Fibrinolysis. 2012 Oct;23(7):603-7. doi: 10.1097/MBC.0b013e3283566af9.
8
Successful delivery in patients with FXIII deficiency receiving prophylaxis: report of 17 cases in Iran.在接受预防治疗的 FXIII 缺乏症患者中成功分娩:伊朗 17 例报告。
Haemophilia. 2012 Sep;18(5):773-6. doi: 10.1111/j.1365-2516.2012.02785.x. Epub 2012 Mar 28.
9
Arg77His and Trp187Arg are the most common mutations causing FXIII deficiency in Iran.Arg77His 和 Trp187Arg 是导致伊朗人群 FXIII 缺乏症最常见的突变。
Clin Appl Thromb Hemost. 2012 Jan-Feb;18(1):100-3. doi: 10.1177/1076029611412363. Epub 2011 Dec 6.
10
Bleeding symptoms in heterozygous carriers of inherited coagulation disorders in southern Iran.伊朗南部遗传性凝血障碍杂合子携带者的出血症状
Blood Coagul Fibrinolysis. 2011 Jul;22(5):396-401. doi: 10.1097/MBC.0b013e328345f566.