[Severe congenital Factor VII deficiency discovered incidentally in the peripartum period: a case report].

IF 1 Q4 PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH
Pan African Medical Journal Pub Date : 2025-05-14 eCollection Date: 2025-01-01 DOI:10.11604/pamj.2025.51.15.47005
Youssef Aarjouni, Oumayma Elbouni, Chikhi Brahim, Mohamed Benani, Houmed Housein, Laila Drouzi, Nezha Oudghiri, Sofia Lahbabi, Rajaa Tachinante
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引用次数: 0

Abstract

Factor VII, also known as proconvertin, is a vitamin K-dependent coagulation protein involved in the extrinsic coagulation pathway. It is a congenital deficiency that is a rare autosomal recessive disorder that can manifest as bleeding episodes of varying severity. We here report the case of a 22-year-old primigravida patient in whom a coagulation disorder was incidentally discovered during the peripartum period, following an episode of epistaxis. Biological investigations revealed a significant decrease in prothrombin time and a markedly prolonged INR, leading to the diagnosis of severe factor VII deficiency. Given the high risk of hemorrhage, a specific obstetric management plan was implemented. Delivery was performed by cesarean section under general anesthesia, following preoperative preparation that included a transfusion of fresh frozen plasma and the administration of an antifibrinolytic agent. The procedure was uneventful, and the postoperative course was favorable, with no significant bleeding episodes. This study highlights the importance of early detection and appropriate management of factor VII deficiency in an obstetric context to minimize hemorrhagic risks for both mother and newborn.

Abstract Image

围生期偶然发现严重先天性第七因子缺乏1例。
因子VII,也称为原转化素,是一种维生素k依赖性凝血蛋白,参与外源性凝血途径。这是一种先天性缺陷,是一种罕见的常染色体隐性遗传病,可以表现为出血发作的不同严重程度。我们在这里报告的情况下,22岁的初产妇患者凝血障碍是偶然发现在围产期期间,继发作鼻出血。生物学检查显示凝血酶原时间明显减少,INR明显延长,导致诊断为严重的七因子缺乏。鉴于出血的高风险,实施了一项具体的产科管理计划。在全身麻醉下剖宫产分娩,术前准备包括输注新鲜冷冻血浆和使用抗纤溶药物。手术过程顺利,术后过程良好,无明显出血发作。这项研究强调了在产科环境中早期发现和适当管理因子7缺乏的重要性,以尽量减少母亲和新生儿的出血风险。
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来源期刊
Pan African Medical Journal
Pan African Medical Journal PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH-
CiteScore
1.80
自引率
0.00%
发文量
691
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