Department of Obstetrics and Gynecology, Hamamatsu Medical Center, Hamamatsu, Japan.
The Study Group for Hereditary Thrombophilia, Research on Blood Coagulation Abnormalities, Research Program on Rare and Intractable Diseases, The Ministry of Health, Labour and Welfare Science Research Grants, Tokyo, Japan.
J Obstet Gynaecol Res. 2021 Sep;47(9):3008-3033. doi: 10.1111/jog.14879. Epub 2021 Jun 24.
Hereditary thrombophilia is a condition in which individuals are susceptible to the formation of thrombi due to a hereditary deficiency in anticoagulant factors, antithrombin (AT), protein C (PC), or protein S (PS). Many Japanese thrombophilia patients have PS deficiency, especially PS p.K196E (also called as PS Tokushima), which is exclusive to the Japanese population, and thrombosis sometimes occurs during pregnancy. At present, no management guidelines for pregnancy and delivery in thrombophilia patients have been developed. The Study Group for Hereditary Thrombophilia, one of the research groups of blood coagulation abnormalities in the Research Program on Rare and Intractable Diseases supported with the Research Grants of the Ministry of Health, Labour and Welfare Science, has therefore developed this clinical guidance to provide healthcare workers with necessary information on safe pregnancy, parturition and neonatal management, adopting a format of responses to seven clinical questions (CQ). At the end of each answer, the corresponding Recommendation Level (A, B, C) is indicated.
遗传性血栓形成倾向是一种由于抗凝因子、抗凝血酶 (AT)、蛋白 C (PC) 或蛋白 S (PS) 的遗传性缺乏而导致个体易形成血栓的疾病。许多日本血栓形成倾向患者存在 PS 缺乏,尤其是 PS p.K196E(也称为 PS 德岛),这是日本人群特有的,血栓有时会在怀孕期间发生。目前,尚未制定针对血栓形成倾向患者妊娠和分娩的管理指南。遗传性血栓形成倾向研究小组是厚生劳动省罕见和难治性疾病研究计划中血液凝固异常研究小组之一,该小组制定了本临床指南,为医疗保健工作者提供有关安全妊娠、分娩和新生儿管理的必要信息,采用回答七个临床问题 (CQ) 的格式。在每个答案的末尾,都标明了相应的推荐级别 (A、B、C)。