İnce Zeynep, Bulut Özgül, Tuğrul-Aksakal Melike, Ünüvar Ayşegül, Devecioğlu Ömer, Çoban Asuman
Divisions of Neonatology, İstanbul University İstanbul Faculty of Medicine, İstanbul, Turkey.
Hematology and Oncology, Department of Pediatrics, İstanbul University İstanbul Faculty of Medicine, İstanbul, Turkey.
Turk J Pediatr. 2018;60(5):562-565. doi: 10.24953/turkjped.2018.05.014.
İnce Z, Bulut Ö, Tuğrul-Aksakal M, Ünüvar A, Devecioğlu Ö, Çoban A. Asymptomatic intracranial hemorrhage in a newborn with congenital factor VII deficiency and successful treatment with recombinant activated factor VII. Turk J Pediatr 2018; 60: 562-565. Intracranial hemorrhage is considered the most common cause of death in newborns with congenital factor VII (FVII) deficiency. Recombinant activated FVII (rFVIIa) provides specific replacement therapy, however there is limited experience with its neonatal use. We describe our experience about the treatment of intracranial hemorrhage in a newborn with congenital FVII deficiency and emphasize the importance of imaging in asymptomatic patients. She presented with ecchymoses on her skin, no other pathological clinical signs, prolonged PT, normal PTT and FVII activity of 2%. Intracranial hemorrhage was diagnosed while screening for internal bleedings. Treatment with rFVIIa resulted in stabilization and regression of the hematoma.
因斯 Z、布卢特 Ö、图格鲁尔-阿克萨卡尔 M、于努瓦尔 A、德韦乔奥卢 Ö、乔班 A。先天性因子 VII 缺乏新生儿的无症状颅内出血及重组活化因子 VII 成功治疗。《土耳其儿科学杂志》2018 年;60: 562 - 565。颅内出血被认为是先天性因子 VII(FVII)缺乏新生儿最常见的死亡原因。重组活化 FVII(rFVIIa)提供特异性替代治疗,然而其在新生儿中的使用经验有限。我们描述了我们对一名先天性 FVII 缺乏新生儿颅内出血治疗的经验,并强调了影像学在无症状患者中的重要性。她皮肤出现瘀斑,无其他病理性临床体征,凝血酶原时间延长,活化部分凝血活酶时间正常,FVII 活性为 2%。在筛查内出血时诊断出颅内出血。rFVIIa 治疗使血肿稳定并消退。