A Still Rare Case of Congenital Afibrinogenemia

I. Tlamçani, A. Krich, Fatima Zahrae El Hamdi, M. A. Hassani
{"title":"A Still Rare Case of Congenital Afibrinogenemia","authors":"I. Tlamçani, A. Krich, Fatima Zahrae El Hamdi, M. A. Hassani","doi":"10.33140/jcei/03/02/00002","DOIUrl":null,"url":null,"abstract":"Congenital afibrinogenemia is characterized by the decrease or the absence of fibrinogen synthesis. It is a rare pathology that is transmitted autosomal recessive mode, with variable clinical demonstrations. The biological diagnosis consists in the presence of traces or absence of fibrogen with blood incoagulability. The coverage of this disease bases itself on the preventive treatment and replacement therapy based on fresh frozen plasma or fibrinogen concentrate. Through this case, we recall the various aspects of these rare condition clinical, biological, genetical as well as therapeutic plans.","PeriodicalId":73657,"journal":{"name":"Journal of clinical & experimental immunology","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2018-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of clinical & experimental immunology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.33140/jcei/03/02/00002","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

Abstract

Congenital afibrinogenemia is characterized by the decrease or the absence of fibrinogen synthesis. It is a rare pathology that is transmitted autosomal recessive mode, with variable clinical demonstrations. The biological diagnosis consists in the presence of traces or absence of fibrogen with blood incoagulability. The coverage of this disease bases itself on the preventive treatment and replacement therapy based on fresh frozen plasma or fibrinogen concentrate. Through this case, we recall the various aspects of these rare condition clinical, biological, genetical as well as therapeutic plans.
一例罕见的先天性纤维蛋白原血症
先天性纤维蛋白原血症的特点是纤维蛋白原合成减少或缺乏。这是一种罕见的常染色体隐性遗传病,临床表现不一。生物学诊断包括存在或不存在纤维原伴血液不凝固性。这种疾病的覆盖范围基于预防性治疗和基于新鲜冷冻血浆或纤维蛋白原浓缩物的替代治疗。通过这个病例,我们回顾了这些罕见疾病的临床、生物学、遗传学以及治疗计划的各个方面。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
自引率
0.00%
发文量
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信