背景:凝血因子V缺乏是罕见的,这种情况的患者的围手术期管理尤为重要,尤其是在腹部大手术期间。我们介绍了一例胰管结石合并凝血因子V缺乏的患者。我们分享我们的围手术期管理经验。
方法:一名31岁男子表现为上腹痛复发2年。
方法:通过腹部计算机断层扫描和磁共振成像检查确定患者胰管结石的诊断。最初通过凝血功能检查确定了因子V缺乏的诊断,显示aPTT和PT均显著延长。随后的凝血因子和抑制剂测试表明患者存在凝血因子V缺乏。基因检测显示,在这种情况下,因子V缺乏是遗传性的。
方法:患者接受胰头部分切除术,手术前1小时输注FFP。在手术开始前1小时滴注600mL的FFP和10U的冷沉淀。术中加入600mlFFP。术后治疗包括在术后前5天间歇性补充FFP,同时监测凝血功能。
结果:患者接受了一次成功的手术,手术过程中没有任何异常出血或渗出。术后恢复顺利,没有异常出血.
结论:凝血因子V缺乏的患者没有手术禁忌。适当的新鲜冰冻血浆(FFP)替代疗法可以确保外科手术的安全进行。对于凝血功能异常的患者,我们建议检测凝血因子和抑制剂,以及进行异常凝血因子的基因检测,可以为婚姻和分娩提供指导。
BACKGROUND: Coagulation factor V deficiency is rare, and perioperative management of patients with this condition is particularly important, especially during major abdominal surgery. We present a
case of a patient with pancreatic duct stones combined with coagulation factor V deficiency. We share our perioperative management experience.
METHODS: A 31-year-old man presented with recurrent upper abdominal pain for 2 years.
METHODS: The diagnosis of pancreatic duct stones in the patient has been established through abdominal computed tomography and magnetic resonance imaging examinations. The diagnosis of factor V deficiency was initially identified through coagulation function tests, revealing significant prolongation of both aPTT and PT. Subsequent testing of coagulation factors and inhibitors demonstrated that the patient has a deficiency in coagulation factor V. Finally, genetic testing revealed that the factor V deficiency in this
case is hereditary.
METHODS: The patient underwent a partial resection of the pancreatic head, and FFP was infused 1 hour before surgery. 600 mL of FFP was instilled 1 hour before the start of surgery along with 10 U of cryoprecipitate. and 600 ml of FFP were added during surgery. Postoperative treatment included intermittent FFP supplemental infusion in the first 5 days after surgery while monitoring the coagulation function.
RESULTS: The patient underwent a successful surgery without any abnormal bleeding or oozing during the procedure. The postoperative recovery was smooth, with no abnormal bleeding.
CONCLUSIONS: Patients with a deficiency of coagulation factor V are not contraindicated for surgery. Appropriate Fresh Frozen Plasma (FFP) replacement therapy can ensure the safe conduct of the surgical procedure. For patients with abnormal blood coagulation function, we recommend testing for coagulation factors and inhibitors, as well as performing genetic testing for abnormal coagulation factors, which can provide guidance on marriage and childbirth.