Youssef Aarjouni, Oumayma Elbouni, Chikhi Brahim, Mohamed Benani, Houmed Housein, Laila Drouzi, Nezha Oudghiri, Sofia Lahbabi, Rajaa Tachinante
{"title":"围生期偶然发现严重先天性第七因子缺乏1例。","authors":"Youssef Aarjouni, Oumayma Elbouni, Chikhi Brahim, Mohamed Benani, Houmed Housein, Laila Drouzi, Nezha Oudghiri, Sofia Lahbabi, Rajaa Tachinante","doi":"10.11604/pamj.2025.51.15.47005","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":48190,"journal":{"name":"Pan African Medical Journal","volume":"51 ","pages":"15"},"PeriodicalIF":1.0000,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12296818/pdf/","citationCount":"0","resultStr":"{\"title\":\"[Severe congenital Factor VII deficiency discovered incidentally in the peripartum period: a case report].\",\"authors\":\"Youssef Aarjouni, Oumayma Elbouni, Chikhi Brahim, Mohamed Benani, Houmed Housein, Laila Drouzi, Nezha Oudghiri, Sofia Lahbabi, Rajaa Tachinante\",\"doi\":\"10.11604/pamj.2025.51.15.47005\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":48190,\"journal\":{\"name\":\"Pan African Medical Journal\",\"volume\":\"51 \",\"pages\":\"15\"},\"PeriodicalIF\":1.0000,\"publicationDate\":\"2025-05-14\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12296818/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pan African Medical Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.11604/pamj.2025.51.15.47005\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q4\",\"JCRName\":\"PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pan African Medical Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.11604/pamj.2025.51.15.47005","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/1/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"PUBLIC, ENVIRONMENTAL & OCCUPATIONAL HEALTH","Score":null,"Total":0}
[Severe congenital Factor VII deficiency discovered incidentally in the peripartum period: a case report].
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.