Jonnavithula Nirmala, Durga Padmaja, Pochiraju Ravindra, Anne Kiran Kumar, Ramachandran Gopinath
Department of Anesthesiology and Critical Care, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh, India.
Anesth Analg. 2009 Jan;108(1):76-8. doi: 10.1213/ane.0b013e3181875e51.
Factor X deficiency is a rare hereditary coagulation disorder. We report a case of congenital factor X deficiency diagnosed preoperatively in an 8-yr-old female child scheduled to undergo corrective surgery for congenital thoracolumbar kyphoscoliosis. Her preoperative coagulation profile revealed prolonged prothrombin time and activated partial thromboplastin time values. Further evaluation showed functional activity of factor X was <8% of the normal activity and was corrected to 10%-40% of the normal activity with fresh frozen plasma. IV tranexamic acid was also administered to reduce intraoperative blood loss. There were no postoperative bleeding complications. This case emphasizes the need for routine preoperative coagulation screening, at least for major surgical procedures.
因子X缺乏症是一种罕见的遗传性凝血障碍。我们报告一例8岁女童，在计划接受先天性胸腰椎后凸脊柱侧弯矫正手术前被诊断为先天性因子X缺乏症。她术前的凝血指标显示凝血酶原时间和活化部分凝血活酶时间延长。进一步评估显示因子X的功能活性<正常活性的8%，新鲜冰冻血浆可将其活性纠正至正常活性的10%-40%。还给予了静脉注射氨甲环酸以减少术中失血。术后无出血并发症。该病例强调了至少对于大型手术常规术前凝血筛查的必要性。
