Bonvini G, Cotta-Ramusino A, Ricciardi G
Divisione di Pediatria, Ospedale Città di Sesto S. Giovanni (MI), Italia.
Pediatr Med Chir. 1994 Jan-Feb;16(1):93-4.
The Authors describe a case of congenital coagulation factor V deficiency with severe prenatal intraventricular hemorrhage. Factor V is an essential enzyme catalysing the process of thrombin generation. Low levels of enzyme were also found in both parents, who demonstrate subclinic alterations of coagulation parameters. The severe deficiency of factor V in the newborn (8%) caused frequent and severe intracranial and gastric hemorrhages that brought newborn to death in few weeks.
作者描述了一例伴有严重产前脑室内出血的先天性凝血因子V缺乏症病例。凝血因子V是催化凝血酶生成过程的一种必需酶。在父母双方中也发现了低水平的该酶，他们表现出凝血参数的亚临床改变。新生儿中严重的凝血因子V缺乏（8%）导致频繁且严重的颅内和胃出血，使新生儿在几周内死亡。
