Thorarensen O, Ryan S, Hunter J, Younkin D P
Children's Hospital of Philadelphia, and Department of Neurology, University of Pennsylvania, 19104, USA.
Ann Neurol. 1997 Sep;42(3):372-5. doi: 10.1002/ana.410420316.
Activated protein C resistance caused by an Arg506Gln mutation in the factor V gene (factor V Leiden mutation) is the most common cause of familial thrombosis. This mutation is associated with arterial and venous thromboembolic disease in neonates, infants, and children, but is not a significant risk factor for ischemic stroke in adults. We report on 3 babies with different neonatal cerebrovascular disorders including ischemic infarction and hemorrhagic stroke who are heterozygous for factor V Leiden mutation. One infant had multiple thrombi in the fetal placental vasculature. This is the first reported association between hemiplegic cerebral palsy, placental thrombosis, and factor V Leiden mutation. We suspect that activated protein C resistance may be an important cause of in utero cerebrovascular disease and hemiplegic cerebral palsy.
由因子V基因中的Arg506Gln突变(因子V莱顿突变)引起的活化蛋白C抵抗是家族性血栓形成的最常见原因。这种突变与新生儿、婴儿和儿童的动脉和静脉血栓栓塞性疾病有关,但不是成人缺血性中风的重要危险因素。我们报告了3例患有不同新生儿脑血管疾病(包括缺血性梗死和出血性中风)的婴儿,他们为因子V莱顿突变的杂合子。一名婴儿在胎儿胎盘血管系统中有多个血栓。这是首次报道的偏瘫型脑瘫、胎盘血栓形成与因子V莱顿突变之间的关联。我们怀疑活化蛋白C抵抗可能是子宫内脑血管疾病和偏瘫型脑瘫的重要原因。