心脏直视手术即将开始前的活化凝血时间延长。
Prolonged Activated Clotting Time Immediately Prior to Open Cardiac Surgery.
作者信息
Baker Sarah M, Kiefer Ashley, Carollo Dominic S, Warrier Rajasekharan P
机构信息
Department of Microbiology and Immunology, Tulane University School of Medicine, New Orleans, LA.
Le Bonheur Children's Hospital, University of Tennessee, Memphis, TN.
出版信息
Ochsner J. 2018 Winter;18(4):423-424. doi: 10.31486/toj.18.0087.
BACKGROUND
Prekallikrein deficiency is an extremely rare disorder in which functional prekallikrein in the plasma is reduced or absent.
CASE REPORT
We present the case of a 15-year-old male with prolonged activated clotting time incidentally noted preoperatively prior to repair of an atrial septal defect. The patient was subsequently found to have prekallikrein (Fletcher factor) deficiency. He successfully underwent open cardiac surgical repair after fresh frozen plasma was administered at a dose of 15 mL/kg 1 hour prior to the start of the procedure.
CONCLUSION
History and routine preoperative evaluations of complete blood count, partial thromboplastin time, prothrombin time, and platelet function analysis failed to detect any abnormalities, but a prolongation of activated clotting time identified by the anesthesiologist led to specialized testing and a diagnosis of Fletcher factor deficiency. The patient tolerated the open-heart surgery well without any significant hematologic intervention or complications.
背景
前激肽释放酶缺乏症是一种极为罕见的疾病，血浆中的功能性前激肽释放酶减少或缺失。
病例报告
我们报告一例15岁男性患者，在房间隔缺损修复术前偶然发现活化凝血时间延长。该患者随后被发现患有前激肽释放酶（弗莱彻因子）缺乏症。在手术开始前1小时给予15 mL/kg剂量的新鲜冰冻血浆后，他成功接受了心脏直视手术修复。
结论
病史以及术前全血细胞计数、部分凝血活酶时间、凝血酶原时间和血小板功能分析等常规评估均未发现任何异常，但麻醉医生发现活化凝血时间延长，进而进行了专项检测，并诊断为弗莱彻因子缺乏症。该患者耐受心脏直视手术良好，未进行任何重大血液学干预或出现并发症。
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