Yilmaz Ali Abbas, Yalcin Saban, Serdaroglu Hacer, Sonmezer Murat, Uysalel Asuman
Department of Anesthesiology and Intensive Care, Ankara University Medical Faculty, Cayyolu, Ankara, Turkey.
Blood Coagul Fibrinolysis. 2008 Oct;19(7):693-5. doi: 10.1097/MBC.0b013e3282f544ff.
Congenital factor VII deficiency is a rare autosomal-recessive disorder and surgery in patients with factor VII deficiency has been reported to be endangered by intraoperative or postoperative bleeding, unless a replacement therapy is used. In this paper, we report a successful prophylaxis with single and low dose rFVIIa (12.5 microg kg(-1)) in a 22-year-old homozygote factor VII deficient patient who underwent laparoscopic gynecologic surgery. Minimally invasive surgeries, such as laparoscopic surgery, could be safely performed in patients with congenital factor VII using single and low dose rFVIIa combined with vigilant clinical observation and laboratory examination.
先天性因子VII缺乏症是一种罕见的常染色体隐性疾病,据报道,除非采用替代疗法,否则因子VII缺乏症患者进行手术时会因术中或术后出血而面临危险。在本文中,我们报告了一名22岁的纯合子因子VII缺乏症患者,在接受腹腔镜妇科手术时,使用单次低剂量重组活化因子VII(rFVIIa)(12.5微克/千克)进行成功预防的案例。对于先天性因子VII缺乏症患者,采用单次低剂量rFVIIa并结合密切的临床观察和实验室检查,诸如腹腔镜手术之类的微创手术可以安全进行。