携带天然抗凝剂者的动脉血栓形成风险：前瞻性家族队列研究。
The risk of arterial thrombosis in carriers of natural coagulation inhibitors: a prospective family cohort study.
机构信息
Chair of Internal Medicine, Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, University of Padua Medical School, Padua, Italy.
Arianna Foundation on Anticoagulation, Bologna, Italy.
出版信息
Intern Emerg Med. 2021 Jun;16(4):997-1003. doi: 10.1007/s11739-021-02656-5. Epub 2021 Feb 22.
BACKGROUND
Whether the carriership of inherited antithrombin (AT), protein C (PC), and protein S (PS) deficiency increases the risk of arterial thromboembolic events (ATE) is controversial. This information has the potential to inform the management of family members of probands with inherited deficiency of natural anticoagulants.
PATIENTS/METHODS: We conducted a large prospective family cohort study in 640 subjects (of whom 341 carriers and 299 non-carriers) belonging to 86 families with inherited deficiency of AT, PC, or PS.
RESULTS
A total of 4240 and 3810 patient-years were available for carriers and non-carriers, respectively. Risk factors for atherosclerosis were similarly distributed in the two groups. Of the 26 ATE that were recorded, 19 occurred in carriers (5.6%), as compared to 7 in non-carriers (2.3%) [p = 0.07]. After adjusting for confounders, the hazard ratio (HR) for ATE was 4.9 (95% CI 1.5-16.3) in carriers as compared to non-carriers.
CONCLUSIONS
Among family members of probands with an inherited deficiency of natural anticoagulants, carriers exhibit a risk of ATE that is almost five times higher than in non-carriers.
背景
遗传性抗凝血酶（AT）、蛋白 C（PC）和蛋白 S（PS）缺陷携带者是否会增加动脉血栓栓塞事件（ATE）的风险存在争议。这些信息有可能为携带遗传性天然抗凝剂缺陷的先证者的家庭成员的管理提供依据。
患者/方法：我们对 640 名受试者（其中 341 名携带者和 299 名非携带者）进行了一项大型前瞻性家族队列研究，这些受试者来自 86 个存在 AT、PC 或 PS 遗传性缺陷的家族。
结果
携带者和非携带者的总随访时间分别为 4240 年和 3810 年。两组的动脉粥样硬化危险因素分布相似。记录的 26 例 ATE 中，19 例发生在携带者（5.6%），而非携带者为 7 例（2.3%）[p=0.07]。调整混杂因素后，携带者的 ATE 风险比（HR）为 4.9（95%CI 1.5-16.3），而非携带者为 1。
结论
在携带遗传性天然抗凝剂缺陷的先证者的家庭成员中，携带者发生 ATE 的风险是非携带者的近五倍。
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