Sidi A, Seligsohn U, Jonas P, Many M
J Urol. 1978 Apr;119(4):528-30. doi: 10.1016/s0022-5347(17)57537-0.
Hereditary factor XI deficiency may remain undiagnosed until severe bleeding is observed after an operation or trauma. Two such cases were encountered and, therefore, a regular screening test for coagulation disorders among urological patients was initiated. During 2 years (1975 and 1976) 10 additional cases of factor XI deficiency were detected: 5 severe and 5 partial. All patients were Ashkenazic Jews of Eastern European origin. The 5 patients with severe factor XI deficiency underwent an operation without any complications. They were transfused with 5 to 20 ml./kg./day of fresh frozen plasma from the day before the operation until 10 to 14 days postoperatively. Of the 5 patients with partial XI deficiency 4 underwent an operation. In 1 of 3 patients who did not receive transfusions postoperative bleeding was observed. A minimal level of 0.3 U./ml. (30 per cent) factor XI was found necessary to ensure good hemostasis during and after an operation. In view of a recent finding of relatively high gene frequency of factor XI deficiency in Ashkenazic Jews it seems warranted to do a partial thromboplastin time in such patients who need an operation.
遗传性因子 XI 缺乏症可能在手术或创伤后出现严重出血时才被诊断出来。我们遇到了两例这样的病例，因此，开始对泌尿外科患者进行凝血障碍的常规筛查试验。在两年（1975 年和 1976 年）期间，又检测到 10 例因子 XI 缺乏症病例：5 例严重缺乏，5 例部分缺乏。所有患者均为东欧裔阿什肯纳兹犹太人。5 例严重因子 XI 缺乏症患者接受了手术，无任何并发症。从手术前一天开始，他们每天接受 5 至 20 毫升/千克的新鲜冷冻血浆输注，直至术后 10 至 14 天。5 例部分 XI 缺乏症患者中有 4 例接受了手术。在 3 例未接受输血的患者中，有 1 例术后出现出血。发现因子 XI 水平至少为 0.3 单位/毫升（30%）对于确保手术期间和术后良好的止血是必要的。鉴于最近发现阿什肯纳兹犹太人中因子 XI 缺乏症的基因频率相对较高，对于需要手术的此类患者，似乎有必要进行部分凝血活酶时间检测。
