Ferlan G, De Pasquale C, Testini M, Agnino A, D'Agostino D
Division of Cardiac Surgery, Faculty of Medicine, University of Bari, Italy.
J Cardiovasc Surg (Torino). 1999 Apr;40(2):279-80.
Congenital Factor VII deficiency is a rare disorder associated with reduced levels of Factor VII activity. Replacement therapy is necessary to control hemorrhaging or if surgery is needed. We report operative treatment of one case of chronic abdominal aortic aneurysm in a patient affected by a severe form of congenital Factor VII deficiency (endogenous FVII level <1%). The operation was carried out after the administration of Factor VII concentrate raised the Factor VII concentration to hemostatic levels. The patient continued to receive the concentrate every 6 hrs during the first three postoperative days. Dosage was assessed to obtain Factor VII levels not lower than 25%. No postoperative bleeding or thrombotic events were observed. The patient was discharged in excellent condition.
先天性因子VII缺乏症是一种罕见的疾病,与因子VII活性水平降低有关。对于控制出血或需要进行手术的情况,替代疗法是必要的。我们报告了一例严重先天性因子VII缺乏症(内源性FVII水平<1%)患者的慢性腹主动脉瘤的手术治疗。在给予因子VII浓缩物将因子VII浓度提高到止血水平后进行了手术。术后头三天,患者每6小时继续接受一次浓缩物。评估剂量以获得不低于25%的因子VII水平。未观察到术后出血或血栓形成事件。患者出院时状况良好。