Lockwood C J
Department of Obstetrics and Gynecology, New York University School of Medicine, NY 10016, USA.
Obstet Gynecol Surv. 1999 Dec;54(12):754-65. doi: 10.1097/00006254-199912000-00004.
Heritable coagulopathies are leading causes of maternal thromboembolism and are associated with an increased risk of maternal and perinatal morbidity and mortality. The most common of these disorders are antithrombin III deficiency, protein C deficiency, protein S deficiency, activated protein C resistance resulting from the factor V Leiden mutation, elevated prothrombin activity associated with a mutation in the prothrombin gene, and hyperhomocystinemia. The maternal risk of a thromboembolic episode is increased by a factor of eight in the presence of any of these heritable states. In addition, the relative risk for a stillbirth in the presence of one of these disorders is 3.6. These conditions are also associated with intrauterine growth retardation and preeclampsia. Proper management of heritable coagulopathies during pregnancy is essential to reduce the risk of these serious sequelae. Patients with newly diagnosed deep-vein thromboses or pulmonary emboli should be treated with therapeutic levels of unfractionated or low molecular weight heparin, followed by subsequent prophylactic heparin therapy. All patients with a history of thromboembolism before pregnancy or evidence of any of these coagulopathies may be offered prophylactic therapy with low molecular weight heparin. Patients with antithrombin III deficiency should receive full therapeutic heparin therapy for the entire pregnancy, irrespective of their thromboembolic history. Postpartum therapy with either heparin or warfarin is required in all cases.
Obstetricians & Gynecologists, Family Physicians
After completion of this article, the reader will be able to describe the various heritable coagulopathies that can complicate pregnancy, to state the potential adverse effects of heritable coagulopathies in pregnancy, and to explain the management of heritable coagulopathies during pregnancy.
遗传性凝血病是孕产妇血栓栓塞的主要原因，与孕产妇及围产期发病和死亡风险增加相关。这些疾病中最常见的是抗凝血酶III缺乏症、蛋白C缺乏症、蛋白S缺乏症、因因子V莱顿突变导致的活化蛋白C抵抗、与凝血酶原基因突变相关的凝血酶原活性升高以及高同型半胱氨酸血症。在存在任何一种这些遗传状态时，孕产妇发生血栓栓塞事件的风险会增加8倍。此外，存在这些疾病之一时死产的相对风险为3.6。这些情况还与宫内生长迟缓及先兆子痫相关。孕期对遗传性凝血病进行恰当管理对于降低这些严重后遗症的风险至关重要。新诊断为深静脉血栓形成或肺栓塞的患者应接受治疗剂量的普通肝素或低分子量肝素治疗，随后进行预防性肝素治疗。所有孕前有血栓栓塞病史或有任何这些凝血病证据的患者都可给予低分子量肝素预防性治疗。抗凝血酶III缺乏症患者无论其血栓栓塞病史如何，整个孕期都应接受全剂量治疗性肝素治疗。所有病例产后均需用肝素或华法林治疗。
妇产科医生、家庭医生
阅读本文后，读者将能够描述可使妊娠复杂化的各种遗传性凝血病，陈述遗传性凝血病在孕期的潜在不良影响，并解释孕期遗传性凝血病的管理。
