Weinstock D J, Schwartz A D
Department of Pediatrics, Sinai Hospital of Baltimore, MD, USA.
J Pediatr Surg. 1995 Dec;30(12):1746-7. doi: 10.1016/0022-3468(95)90472-7.
Although inherited coagulation factor deficiencies with negative bleeding histories are rare, cases such as the one presented herein may not be diagnosed at the time of surgery and may experience severe hemostatic problems postoperatively. It may be prudent to obtain an activated partial thromboplastin time preoperatively for all Ashkenazi Jews, including those with a negative history for a hemostatic disorder, because of the high incidence of factor XI deficiency in their population. Surgeons and anesthesiologists should be aware of the existence of this and other uncommon hemostatic disorders and their treatment when faced with serious, apparently unexplained postoperative hemorrhage.
尽管有阴性出血史的遗传性凝血因子缺乏症很少见，但本文所呈现的这类病例在手术时可能无法被诊断出来，术后可能会出现严重的止血问题。鉴于阿什肯纳兹犹太人中因子XI缺乏症的高发病率，对于所有阿什肯纳兹犹太人，包括那些有止血障碍阴性史的人，术前进行活化部分凝血活酶时间检测可能是谨慎的做法。当面对严重的、明显原因不明的术后出血时，外科医生和麻醉医生应该意识到这种及其他罕见止血障碍的存在及其治疗方法。
