Heijboer H, Brandjes D P, Büller H R, Sturk A, ten Cate J W
Center for Thrombosis, Haemostasis, and Atherosclerosis Research, Academic Medical Center, Amsterdam, The Netherlands.
N Engl J Med. 1990 Nov 29;323(22):1512-6. doi: 10.1056/NEJM199011293232202.
Isolated deficiencies of antithrombin III, protein C, protein S, and plasminogen have been implicated as a cause of deep-vein thrombosis. It is assumed that patients with recurrent, familial, or juvenile thrombosis are very likely to have such a deficiency.
We studied the prevalence of isolated deficiencies of these proteins in 277 consecutive outpatients with venographically proved acute deep-vein thrombosis, as compared with 138 age-matched and sex-matched controls without deep-vein thrombosis, and calculated the positive predictive value of a history of recurrent, familial, or juvenile venous thromboembolism for the presence of such a deficiency.
The overall prevalence of deficiencies of any of these proteins in the patients with venous thrombosis was 8.3 percent (23 of 277 patients) (95 percent confidence interval, 5.4 to 12.4), as compared with 2.2 percent in the controls (3 of 138 subjects) (95 percent confidence interval, 0.5 to 6.1; P less than 0.05 for the comparison between groups). The positive predictive values for the presence of an isolated protein deficiency in patients with recurrent, familial, or juvenile deep-vein thrombosis, defined as the proportion of patients with the clinical finding who had a deficiency of one or more of the proteins, were 9, 16, and 12 percent, respectively.
The cause of acute venous thrombosis in most outpatients (91.7 percent) cannot be explained by abnormalities of coagulation-inhibiting and fibrinolytic proteins. The information obtained from the medical history concerning recurrent or familial venous thrombosis or the onset of the disease at a young age is not useful for the identification of patients with protein deficiencies.
抗凝血酶III、蛋白C、蛋白S和纤溶酶原的单独缺乏被认为是深静脉血栓形成的一个原因。据推测，复发性、家族性或青少年血栓形成的患者很可能存在这种缺乏。
我们研究了277例经静脉造影证实为急性深静脉血栓形成的连续门诊患者中这些蛋白单独缺乏的患病率，并与138例年龄和性别匹配且无深静脉血栓形成的对照者进行比较，计算复发性、家族性或青少年静脉血栓栓塞病史对存在这种缺乏的阳性预测值。
静脉血栓形成患者中任何一种这些蛋白缺乏的总体患病率为8.3%（277例患者中有23例）（95%置信区间为5.4%至12.4%），而对照组为2.2%（138例受试者中有3例）（95%置信区间为0.5%至6.1%；两组比较P<0.05）。复发性、家族性或青少年深静脉血栓形成患者中存在单独蛋白缺乏的阳性预测值，定义为有临床发现且一种或多种蛋白缺乏的患者比例，分别为9%、16%和12%。
大多数门诊患者（91.7%）急性静脉血栓形成的原因不能用凝血抑制和纤溶蛋白异常来解释。从病史中获得的有关复发性或家族性静脉血栓形成或年轻时发病的信息，对于识别蛋白缺乏患者并无用处。
