Assani Karim, Karboubi Lamya, Dakhama Badr Sououd Benjelloun
Service des Urgences Pédiatriques, Hôpital d'Enfants, Rabat, CHU Ibn-Sina, Maroc.
Pan Afr Med J. 2016 Dec 9;25:233. doi: 10.11604/pamj.2016.25.233.10754. eCollection 2016.
Afibrinogenemia is a rare dyscrasia characterized by a congenital fibrinogen deficiency It is transmitted in an autosomal recessive manner. Hemorrhagic manifestations are variable and can be life-threatening. A little more than 250 cases have been published up till now. We here report a new case of congenital afibrinogenemia in a 3 1/2-year old child hospitalized for medium abundance hematemesis. This case study aims to highlight numerous aspects of this condition from a clinical, biological, genetic and therapeutic points of view.
无纤维蛋白原血症是一种罕见的血液疾病，其特征为先天性纤维蛋白原缺乏，以常染色体隐性方式遗传。出血表现多样，可能危及生命。截至目前，已发表的病例略多于250例。我们在此报告一例3岁半因中度大量呕血住院的先天性无纤维蛋白原血症新病例。本病例研究旨在从临床、生物学、遗传学和治疗学角度突出该病症的诸多方面。
