妊娠合并严重纯合子遗传性凝血因子Ⅴ缺乏症并产后大出血一例  

Pregnancy Complicated with Severe Homozygous Hereditary Coagulation Factor Ⅴ Deficiency and PostpartumMassive Hemorrhage: A Case Report

作　　者：范倩倩 何荣霞[2] FAN Qian-qian;HE Rong-xia(The Second Clinical College of Lanzhou University,Lanzhou 730030,China;The Second Hospital of Lanzhou University,Lanzhou 730030,China)

机构地区：[1]兰州大学第二临床医学院,730030 [2]兰州大学第二医院

出　　处：《国际妇产科学杂志》2023年第4期433-436,共4页Journal of International Obstetrics and Gynecology

基　　金：甘肃省自然科学基金(21JR11RA124)。

摘　　要：凝血因子Ⅴ(coagulation factorⅤ,FⅤ)缺乏症是一种罕见的遗传疾病,主要表现为FⅤ活性(FⅤactivity,FⅤC)下降和广泛的临床出血症状,妊娠合并FⅤ缺乏症更为少见,且发生不良预后的风险更高。报告1例妊娠合并严重纯合子遗传性FⅤ缺乏症并产后大出血病例,患者入院明确FⅤ缺乏症诊断后连续、多次大剂量输注血液制品,但FⅤC上升不理想。后因急性胎儿窘迫经剖宫产助娩一活婴,并因合并产后严重出血行双侧子宫动脉栓塞术,术后复查FⅤC未恢复至正常水平,经对症治疗后病情稳定出院。通过回顾该病例的病史资料及临床诊疗过程并复习相关文献,旨在增加临床医师对本病的认识,积累更多的临床诊疗经验。Coagulation factorⅤ(FⅤ)deficiency is a rare genetic disorder that is primarily characterized by decreased FⅤactivity(FⅤC)and extensive clinical bleeding symptoms.Pregnancy combined with FⅤdeficiency is even more rare,and the risk of poor prognosis is higher.In this article,we report a case of severe homozygote hereditary FⅤdeficiency and postpartum hemorrhage occurring simultaneously.The patient was treated with ongoing high-dose blood product transfusions after being admitted to the hospital with a clear diagnosis of FⅤdeficiency,but the FⅤC did not improve adequately.Later,a live infant was delivered by cesarean section for acute fetal distress,bilateral uterine artery embolization was done for combined postpartum hemorrhage,and postoperative review revealed that the FⅤC had not returned to the normal level,and the patient was discharged with stable condition after symptomatic treatment.We now go over the medical history,the clinical course of this case,and the pertinent literature,with the aim of increasing clinicians′understanding of this disease and accumulate more clinical experience.

关 键 词：因子Ⅴ 缺乏症 妊娠 产后出血 病例报告 

分 类 号：R714.254[医药卫生—妇产科学]