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先天性出血性疾病女性的妇科出血:来自伊朗的综合队列研究。

Gynecological bleeding in women with congenital bleeding disorders: A comprehensive cohort from Iran.

机构信息

Cellular and Molecular Research Center, Iran University of Medical Sciences, Tehran, Islamic Republic of Iran; Department of Hematology and Blood Banking, Faculty of Allied Medicine, Iran University of Medical Sciences, Tehran, Islamic Republic of Iran.

Department of Hematology and Blood Banking, Faculty of Allied Medicine, Iran University of Medical Sciences, Tehran, Islamic Republic of Iran.

出版信息

Transfus Apher Sci. 2024 Aug;63(4):103941. doi: 10.1016/j.transci.2024.103941. Epub 2024 Apr 30.

Abstract

BACKGROUND

Gynecological bleeding including menorrhagia and postpartum hemorrhage (PPH) face women's quality of life constantly with difficulties, especially those suffering from inherited bleeding disorders. In this study, we aim to evaluate gynecological bleeding particularly menorrhagia among Iranian women patients with inherited bleeding disorders admitted to the Iranian Comprehensive Hemophilia Care Center (ICHCC).

METHODS

This study was conducted on 156 females aged ≥ 12 diagnosed with an inherited bleeding disorder in ICHCC. Demographic and laboratory data were documented for all patients. Bleeding questionnaires (the International Society on Thrombosis and Hemostasis bleeding assessment tool (ISTH-BAT), Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand disease (MCMDM-1), and Pictorial blood loss assessment chart (PBAC)) were filled out for all patients. For comparing metric and categorical parameters, Mann-Whitney was performed. Spearman's rho test was used for analyzing correlation.

RESULTS

The mean age of patients was 33. Von Willebrand disease (VWD), Factor (F) VII deficiency and combined factor deficiency were the most diagnosed disorders. The median of ISTH-BAT, MCMDM-1, and PBAC was 7,7, and 517, respectively. Menorrhagia was the most common reason for diagnosis. Menorrhagia and PPH domain scores ≥ 2 were recorded in 82 and 34 patients, respectively, and PBAC scores > 100 were seen in 118 patients. Significant positive correlations were observed between bleeding scores and menorrhagia and PPH scores. No significant correlations were recorded for VWF: Ag and VWF: RCo with menorrhagia and PPH scores; however, significant correlations were seen for VWF: Ag and VWF: RCo with bleeding score questionnaires.

CONCLUSION

Menorrhagia is the most common problem in females affected by different types of inherited bleeding disorders, particularly VWD. Increased awareness among gynecologists and hematologists about bleeding disorders in cases with unexplained menorrhagia is an essential step for optimal management.

摘要

背景

妇科出血,包括月经过多和产后出血(PPH),不断给女性的生活质量带来困难,尤其是那些患有遗传性出血性疾病的患者。本研究旨在评估伊朗综合血友病护理中心(ICHCC)就诊的患有遗传性出血性疾病的伊朗女性患者的妇科出血,特别是月经过多。

方法

对 ICHCC 诊断为遗传性出血性疾病的 156 名≥12 岁的女性患者进行了这项研究。为所有患者记录了人口统计学和实验室数据。所有患者均填写了出血问卷(国际血栓和止血学会出血评估工具(ISTH-BAT)、用于 1 型血管性血友病诊断和管理的分子和临床标志物(MCMDM-1)和图片出血评估图表(PBAC))。为比较计量和分类参数,采用 Mann-Whitney 检验。Spearman's rho 检验用于分析相关性。

结果

患者的平均年龄为 33 岁。血管性血友病(VWD)、因子(F)VII 缺乏症和联合因子缺乏症是最常见的诊断疾病。ISTH-BAT、MCMDM-1 和 PBAC 的中位数分别为 7、7 和 517。月经过多是诊断最常见的原因。82 例患者月经过多和 PPH 域评分≥2,34 例患者 PBAC 评分>100。出血评分与月经过多和 PPH 评分呈显著正相关。VWF:Ag 和 VWF:RCo 与月经过多和 PPH 评分无显著相关性,但与出血评分问卷有显著相关性。

结论

月经过多是不同类型遗传性出血性疾病女性患者最常见的问题,尤其是 VWD。妇科医生和血液科医生对不明原因月经过多患者的出血性疾病有更高的认识,是优化管理的重要步骤。

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