Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
J Perinatol. 2013 Mar;33(3):239-41. doi: 10.1038/jp.2012.55.
Inherited or acquired protein C (PC) deficiency leads to thromboembolic events. Plasma PC activity in infancy is physiologically lower than in adults. We describe a case of neonatal asphyxia and acute renal failure associated with isolated PC deficiency. A full-term male infant was born to a healthy mother by caesarean section because of fetal distress. The small-for-gestational age infant showed 2 and 7 of Apgar scores at 1 and 5 minutes, respectively. Hypercoagulability required repeated infusions of fresh frozen plasma. Coagulation study revealed PC activity, 6%, protein S activity, 61%, and high D-dimer levels, along with normal factor VII activity and absent vitamin K deficiency. Anticoagulant and activated PC therapy improved coagulopathy and nephropathy. Imaging analyses indicated no visceral infarctions. Renal function and PC activity have been slowly normalized until 6 months of age. He had no PROC mutation or PC-deficient parents. Selective PC deficiency may occur as an acquired cause of hypercoagulable crisis in the stressed newborn.
遗传性或获得性蛋白 C (PC) 缺乏可导致血栓栓塞事件。婴儿期的血浆 PC 活性低于成人。我们描述了一例与孤立性 PC 缺乏相关的新生儿窒息和急性肾衰竭病例。一名足月男性婴儿因胎儿窘迫通过剖宫产术出生于健康母亲。该小于胎龄儿在 1 分钟和 5 分钟时分别获得 2 分和 7 分的阿普加评分。高凝状态需要反复输注新鲜冷冻血浆。凝血研究显示 PC 活性为 6%，蛋白 S 活性为 61%，D-二聚体水平升高，同时因子 VII 活性正常且不存在维生素 K 缺乏。抗凝和激活的 PC 治疗改善了凝血异常和肾病。影像学分析表明无内脏梗死。肾功能和 PC 活性已缓慢恢复正常，直到 6 个月大。他没有 PROC 突变或缺乏 PC 的父母。选择性 PC 缺乏可能是应激新生儿高凝危机的获得性原因。
