Boujrad Saloua, El Hasbaoui Brahim, Echahdi Hanae, Malih Mohamed, Agadr Aomar
Service de Pédiatrie, Hôpital Militaire d'Instruction Mohamed V, Faculté de Médecine et de Pharmacie, Université Mohammed V, Rabat, Maroc.
Pan Afr Med J. 2017 Jul 6;27:182. doi: 10.11604/pamj.2017.27.182.12285. eCollection 2017.
Factor V congenital deficiency is a rare coagulation disorder initially described by Owren in 1947 and known as para hemophilia. It is transmitted through autosomal-recessive inheritance and homozygous cases are usually symptomatic. Factor V is an essential cofactor in the conversion of prothrombin to thrombin by activated factor X. In the absence of factor V, thrombin generation is slowed down and fibrin formation is delayed. This results in a bleeding tendency. We report a case of factor V congenital deficiency in an infant with recurrent epistaxis.
先天性因子V缺乏症是一种罕见的凝血障碍，最初由奥伦于1947年描述，称为副血友病。它通过常染色体隐性遗传传递，纯合子病例通常有症状。因子V是活化因子X将凝血酶原转化为凝血酶过程中的必需辅因子。在缺乏因子V的情况下，凝血酶生成减慢，纤维蛋白形成延迟。这导致出血倾向。我们报告一例患有复发性鼻出血的婴儿先天性因子V缺乏症病例。
