国际妇产科学杂志 ›› 2026, Vol. 53 ›› Issue (3): 267-269.doi: 10.12280/gjfckx.20251463

• 产科生理及产科疾病:病例报告 • 上一篇    下一篇

妊娠晚期合并先天性凝血因子Ⅺ缺乏症一例

王心怡, 孙丽洲, 姜子燕()   

  1. 210029 南京医科大学第一附属医院产科
  • 收稿日期:2025-12-22 出版日期:2026-06-15 发布日期:2026-07-06
  • 通讯作者: 姜子燕 E-mail:zyjiangchm@163.com
  • 基金资助:
    中国博士后科学基金(2021M691332)

A Case of Late Pregnancy Complicated with Congenital Factor Ⅺ Deficiency

WANG Xin-yi, SUN Li-zhou, JIANG Zi-yan()   

  1. Department of Obstetrics, The First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China
  • Received:2025-12-22 Published:2026-06-15 Online:2026-07-06
  • Contact: JIANG Zi-yan E-mail:zyjiangchm@163.com

摘要:

先天性凝血因子Ⅺ缺乏症是一种罕见的常染色体隐性遗传性疾病,妊娠期因生理状态改变可能使凝血功能异常加重,增加分娩期及产后出血风险,严重威胁母婴安全。报告1例32岁妊娠39+1周患者,妊娠期发现活化部分凝血活酶时间(activated partial thromboplastin time,APTT)延长,经凝血因子检测确诊为先天性凝血因子Ⅺ缺乏症。通过多学科诊疗,予新鲜冰冻血浆输注纠正凝血功能,严密监测下阴道试产成功,母儿转归良好。妊娠期凝血功能筛查及异常病因鉴别至关重要,针对先天性凝血因子缺乏症患者,围产期需制定个体化诊疗方案,多学科协同管理可有效保障母婴安全。

关键词: 妊娠末期, 因子Ⅺ缺乏, 活化部分凝血活酶时间, 血浆治疗, 多学科诊疗

Abstract:

Congenital factor Ⅺ deficiency is a rare autosomal recessive inherited disease. Physiological changes during pregnancy may aggravate coagulation abnormalities, increasing the risk of intrapartum and postpartum hemorrhage and posing a serious threat to maternal and fetal safety. We report a case of a 32-year-old patient at 39+1 weeks of gestation who was found to have prolonged activated partial thromboplastin time (APTT) during pregnancy. Congenital factor Ⅺ deficiency was confirmed by coagulation factor assays. Through multi-disciplinary team consultation, fresh frozen plasma transfusion was administered to correct the coagulation function. Successful vaginal trial of labor was achieved under close monitoring, with favorable maternal and neonatal outcomes. Coagulation function screening during pregnancy and etiological identification of abnormalities are crucial. For patients with congenital coagulation factor deficiency, individualized diagnosis and treatment plans should be developed for the perinatal period, and multidisciplinary collaborative management can effectively ensure maternal and neonatal safety.

Key words: Pregnancy trimester, third, Factor Ⅺ deficiency, Activated partial thromboplastin time, Plasma therapy, Multi-disciplinary team