van Boven H H, Vandenbroucke J P, Westendorp R G, Rosendaal F R
Department of Clinical Epidemiology, University Hospital Leiden, The Netherlands.
Thromb Haemost. 1997 Mar;77(3):452-5.
To assess the contribution of inherited antithrombin deficiency to mortality, we investigated the causes of death in 14 families with inherited antithrombin deficiency. Between 1830 and 1994, 86 of 266 family members who had a probability of 0.5 or more for heterozygosity died. The causes of death were obtained for 58 of 66 deaths occurring between 1940 and 1994. Standardized mortality ratios (SMR) were calculated using mortality rates from the general population adjusted for age, sex and calendar period. The overall SMR was 0.90 from 1830 to 1994 (95% C.I. 0.72-1.11). From 1940 until 1994 44 men and 22 women died (SMR = 1.09, 95% C.I. 0.84-1.39; SMR men = 1.20, 95% C.I. 0.87-1.61; SMR women = 0.92, 95% C.I. 0.58-1.39). No excess mortality compared to the general population was found for cancer (14 deaths) or circulatory diseases (28 deaths). A slightly increased mortality caused by respiratory diseases (7 deaths, SMR = 1.68, 95% C.I. 0.68-3.47) seemed due to pneumonia (4 deaths, SMR = 2.86, 95% C.I. 0.78-7.32). Venous thromboembolic complications were listed once in association with a risk situation, and one other death could be attributed to fatal pulmonary embolism. Cerebral hemorrhages were listed three times. It could not be verified whether these hemorrhages were related to anticoagulant therapy; the frequency was slightly higher than the expected population figure (SMR = 1.49, 95% C.I. 0.31-4.36). The mean age of death for all causes was 64 years; the two fatal thromboembolic episodes occurred at age 20 and 30 years. The data show that antithrombin deficiency is associated with a normal survival and a low risk of fatal thromboembolic events. The use of long-term anticoagulant treatment in asymptomatic individuals should be considered carefully in view of the greater risk of fatal bleeding associated with long-term anticoagulant prophylaxis.
为评估遗传性抗凝血酶缺乏对死亡率的影响，我们调查了14个遗传性抗凝血酶缺乏家族的死因。在1830年至1994年间，266名杂合子概率为0.5或更高的家庭成员中有86人死亡。在1940年至1994年间发生的66例死亡中，有58例获得了死因。使用根据年龄、性别和日历时间调整后的普通人群死亡率计算标准化死亡率（SMR）。1830年至1994年的总体SMR为0.90（95%置信区间0.72 - 1.11）。1940年至1994年期间，44名男性和22名女性死亡（SMR = 1.09，95%置信区间0.84 - 1.39；男性SMR = 1.20，95%置信区间0.87 - 1.61；女性SMR = 0.92，95%置信区间0.58 - 1.39）。与普通人群相比，癌症（14例死亡）或循环系统疾病（28例死亡）未发现额外死亡率。呼吸系统疾病导致的死亡率略有增加（7例死亡，SMR = 1.68，95%置信区间0.68 - 3.47）似乎是由于肺炎（4例死亡，SMR = 2.86，95%置信区间0.78 - 7.32）。静脉血栓栓塞并发症曾有一次与风险情况相关列出，另有一例死亡可归因于致命性肺栓塞。脑出血曾列出三次。无法证实这些出血是否与抗凝治疗有关；其发生率略高于预期人群数字（SMR = 1.49，95%置信区间0.31 - 4.36）。所有原因导致的平均死亡年龄为64岁；两例致命性血栓栓塞事件分别发生在20岁和30岁。数据表明，抗凝血酶缺乏与正常生存期和致命性血栓栓塞事件的低风险相关。鉴于长期抗凝预防与致命性出血的风险更高，对于无症状个体使用长期抗凝治疗应谨慎考虑。
