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首次在妊娠期因鼻出血就诊的遗传性因子VII缺乏症的成功治疗:病例报告

Successful treatment of hereditary factor VII deficiency presented for the first time with epistaxis in pregnancy: a case report.

作者信息

Aynaoğlu Gülşah, Durdağ Gülşen Doğan, Ozmen Batuhan, Söylemez Feride

机构信息

Ankara University School of Medicine, Department of Obstetrics and Gynecology, Ankara, Turkey.

出版信息

J Matern Fetal Neonatal Med. 2010 Sep;23(9):1053-5. doi: 10.3109/14767050903440463.

DOI:10.3109/14767050903440463
PMID:20476876
Abstract

OBJECTIVE

To present a rare case with factor VII deficiency (FVIID) that first diagnosed during pregnancy, and to disccuss the ante-, peri- and postpartum management.

STUDY DESIGN

A case report and review of the literature.

RESULTS

FVIID was diagnosed for the first time in a 23-year-old pregnant woman at the 24th week of gestation due to complaint of intermittent epistaxis. She was successfully treated by ante-, peripartum and postpartum replacement of recombinant FVII (rFVII). She was uneventfully discharged on the 3rd postoperative day and referred to hematology department for further follow-up.

CONCLUSION

FVIID, a rare cause of ante- and postpartum hemorrhages that may differently present by other hemorrhages (i.e.: epistaxis), can be optimally managed with replacement of rFVII.

摘要

目的

报告1例孕期首次诊断为Ⅶ因子缺乏症(FVIID)的罕见病例,并探讨产前、产时及产后的管理。

研究设计

病例报告及文献复习。

结果

一名23岁孕妇在妊娠24周时因间歇性鼻出血首次诊断为FVIID。通过产前、围产期和产后重组Ⅶ因子(rFVII)替代治疗,她获得成功治疗。术后第3天顺利出院,并转诊至血液科进行进一步随访。

结论

FVIID是产前和产后出血的罕见原因,可能与其他出血(如鼻出血)表现不同,通过rFVII替代治疗可得到最佳管理。

相似文献

1
Successful treatment of hereditary factor VII deficiency presented for the first time with epistaxis in pregnancy: a case report.首次在妊娠期因鼻出血就诊的遗传性因子VII缺乏症的成功治疗:病例报告
J Matern Fetal Neonatal Med. 2010 Sep;23(9):1053-5. doi: 10.3109/14767050903440463.
2
Factor VII deficiency and pregnancy.
Obstet Gynecol. 1989 Mar;73(3 Pt 2):453-4.
3
[Inherited factor VII deficiency and pregnancy. Apropos of 1 case].
J Gynecol Obstet Biol Reprod (Paris). 1999 Dec;28(8):842-5.
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Factor VII deficiency detected in pregnancy: a case report.
Am J Perinatol. 1999;16(5):223-6. doi: 10.1055/s-2007-993862.
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Factor VII deficiency in pregnancy and delivery: a case report.妊娠期及分娩期凝血因子VII缺乏症:一例报告
Haemophilia. 2011 Nov;17(6):e1005. doi: 10.1111/j.1365-2516.2011.02532.x. Epub 2011 Apr 15.
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Hereditary factor VII deficiency in pregnancy: peripartum treatment with factor VII concentrate.
Am J Hematol. 1992 May;40(1):38-41. doi: 10.1002/ajh.2830400108.
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A case of heterozygous factor VII deficiency in pregnancy.一例妊娠期杂合子因子 VII 缺乏症病例。
J Obstet Gynaecol. 2020 Oct;40(7):1025-1026. doi: 10.1080/01443615.2019.1674792. Epub 2019 Dec 4.
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Severe hereditary deficiency of factor VII during pregnancy. Evidence for the absence of transplacental diffusion of factor VII.妊娠期严重遗传性因子 VII 缺乏。因子 VII 不存在经胎盘扩散的证据。
Thromb Diath Haemorrh. 1970 Oct 31;24(1):146-51.
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Congenital factor VII deficiency: a case report.
Malays J Pathol. 2004 Jun;26(1):65-7.
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Pregnancy in women with congenital factor VII deficiency.先天性因子VII缺乏症女性的妊娠情况。
Haemophilia. 2006 Jul;12(4):413-6. doi: 10.1111/j.1365-2516.2006.01287.x.

引用本文的文献

1
Rhinologic Conditions of Pregnancy: A Retrospective Cohort Study.孕期鼻科疾病:一项回顾性队列研究。
OTO Open. 2025 Apr 25;9(2):e70114. doi: 10.1002/oto2.70114. eCollection 2025 Apr-Jun.
2
Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review.先天性因子 VII 缺乏症女性和女童的生殖健康和止血问题:系统评价。
J Thromb Haemost. 2022 Dec;20(12):2758-2772. doi: 10.1111/jth.15872.
3
Accidentally discovered high INR in pregnancy unmasks an inherited factor VII (FVII) deficiency that is paradoxically associated with thrombotic tendency.
怀孕期间偶然发现的高国际标准化比值(INR)揭示了一种遗传性因子 VII(FVII)缺乏症,这种缺乏症与血栓倾向呈矛盾相关。
BMJ Case Rep. 2021 Feb 4;14(2):e237781. doi: 10.1136/bcr-2020-237781.
4
Management of Severe Epistaxis during Pregnancy: A Case Report and Review of the Literature.孕期严重鼻出血的管理:一例病例报告及文献综述
Case Rep Obstet Gynecol. 2019 Jan 20;2019:5825309. doi: 10.1155/2019/5825309. eCollection 2019.
5
Is prophylaxis required for delivery in women with factor VII deficiency?有因子 VII 缺乏的妇女分娩时需要预防吗?
Haemophilia. 2013 Nov;19(6):827-32. doi: 10.1111/hae.12167. Epub 2013 Apr 22.