I. Tlamçani, A. Krich, Fatima Zahrae El Hamdi, M. A. Hassani
{"title":"一例罕见的先天性纤维蛋白原血症","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":"36 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2018-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"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\":\"36 1\",\"pages\":\"\"},\"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}","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}
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.