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围产期遗传性易栓症的管理:日本的初步监测结果

Peripartum management of hereditary thrombophilia: results of primary surveillance in Japan.

作者信息

Kobayashi Takao, Sugiura Kazuko, Ojima Toshiyuki, Hirai Kyuya, Morishita Eriko

机构信息

Director Emeritus, Department of Obstetrics and Gynecology, Hamamatsu Medical Center, 328 Tomitsuka-cho, Naka-ku, Hamamatsu, Shizuoka, 432-8580, Japan.

Department of Reproductive Health Nursing/Midwifery, Nagoya City University Graduate School of Nursing, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, Aichi, 467-8601, Japan.

出版信息

Int J Hematol. 2022 Sep;116(3):364-371. doi: 10.1007/s12185-022-03354-4. Epub 2022 May 12.

Abstract

This study investigated patients with thrombophilia and current peripartum management practices based on national surveillance in Japan. Between 2014 and 2018, antithrombin (AT), protein C (PC) and protein S (PS) deficiency were observed in 84, 67, and 443 pregnancies, respectively, with incidence rates among total deliveries at 0.012%, 0.009%, and 0.061%. The percentage of institutions that measured both antigens and AT, PC, and PS activity for the diagnosis of thrombophilia was 50.2%, and 46.9% of institutions did not perform gene analysis. Prophylactic anticoagulation therapy was used in the ante- and postpartum management of patients with AT deficiency at 67.1% and 66.3% of institutions, most commonly with 10,000 units of unfractionated heparin. Ante- and postpartum management of PC and PS deficiency was performed at 75.3% and 67.1% of institutions. Approximately half of the institutions performed peripartum prophylactic AT supplementation for AT deficiency. Low trough AT activity before supplementation was most commonly 50 ≤  < 70%, and the highest AT supplementation was 1500 ≤  < 3000 units. The number of pregnancies with AT, PC and PS deficiency might be as many as 29, 23 and 151 every year in Japan if complete answers were provided.

摘要

本研究基于日本的全国监测,调查了患有血栓形成倾向的患者及当前的围产期管理实践。2014年至2018年期间,分别在84例、67例和443例妊娠中观察到抗凝血酶(AT)、蛋白C(PC)和蛋白S(PS)缺乏,在总分娩中的发生率分别为0.012%、0.009%和0.061%。为诊断血栓形成倾向而同时检测抗原及AT、PC和PS活性的机构比例为50.2%,46.9%的机构未进行基因分析。67.1%和66.3%的机构在AT缺乏患者的产前和产后管理中使用预防性抗凝治疗,最常用的是10000单位普通肝素。75.3%和67.1%的机构对PC和PS缺乏进行了产前和产后管理。大约一半的机构对AT缺乏进行围产期预防性AT补充。补充前最低谷AT活性最常见为50≤<70%,最高AT补充量为1500≤<3000单位。如果能提供完整答案,日本每年AT、PC和PS缺乏的妊娠数可能分别多达29例、23例和151例。

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