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症状性血友病 A:妊娠及产后期间的管理。

Symptomatic haemophilia A: management during pregnancy and the postpartum period.

机构信息

Department of Women and Reproductive Medicine, Centro Hospitalar Universitário do Porto EPE Centro Materno-Infantil do Norte Dr Albino Aroso, Porto, Portugal

Endocrine, Cardiovascular & Metabolic Research, Unit for Multidisciplinary Research in Biomedicine (UMIB), University of Porto, Porto, Portugal.

出版信息

BMJ Case Rep. 2022 Jan 7;15(1):e245474. doi: 10.1136/bcr-2021-245474.

DOI:10.1136/bcr-2021-245474
PMID:34996767
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8744121/
Abstract

As an X linked disorder, the presence of severe symptomatic haemophilia A is an extremely rare disorder in women. Therefore there are no high-level evidence-based guidelines when it comes to pregnancy. Although there have been advances in the fields of prenatal counselling and maternal-fetal care, the management of these gestations continues to embody a challenge for any medical team. We report the successful management of a pregnant woman with symptomatic haemophilia A, from pregnancy to the postpartum period. Our aim is to enhance knowledge on this topic, and further improve outcomes for these mothers and their offspring.

摘要

作为一种 X 连锁疾病,女性中严重症状性血友病 A 的存在是一种极其罕见的疾病。因此,在妊娠方面没有高水平的循证指南。尽管产前咨询和母婴保健领域取得了进展,但这些妊娠的管理仍然对任何医疗团队都是一个挑战。我们报告了一例症状性血友病 A 孕妇从妊娠到产后期间的成功管理。我们的目的是增强对这一主题的了解,并进一步改善这些母亲及其后代的结局。

相似文献

1
Symptomatic haemophilia A: management during pregnancy and the postpartum period.症状性血友病 A:妊娠及产后期间的管理。
BMJ Case Rep. 2022 Jan 7;15(1):e245474. doi: 10.1136/bcr-2021-245474.
2
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本文引用的文献

1
Bleeding complications during pregnancy and delivery in haemophilia carriers and their neonates in Western France: An observational study.在法国西部,血友病携带者及其新生儿在妊娠和分娩期间的出血并发症:一项观察性研究。
Haemophilia. 2020 Nov;26(6):1046-1055. doi: 10.1111/hae.14117. Epub 2020 Aug 25.
2
Perinatal Management of Haemophilia.围产期血友病管理。
Hamostaseologie. 2020 May;40(2):226-232. doi: 10.1055/a-1141-1252. Epub 2020 May 28.
3
Women with bleeding disorders.患有出血性疾病的女性。
Haemophilia. 2018 May;24 Suppl 6:29-36. doi: 10.1111/hae.13502.
4
Management of pregnancy in a patient with severe hemophilia type a.重度甲型血友病患者的妊娠管理
AJP Rep. 2013 May;3(1):29-32. doi: 10.1055/s-0032-1331376. Epub 2012 Dec 31.
5
Inherited bleeding disorders in pregnancy.妊娠相关遗传性出血性疾病。
Best Pract Res Clin Obstet Gynaecol. 2012 Feb;26(1):103-17. doi: 10.1016/j.bpobgyn.2011.10.005. Epub 2011 Nov 18.
6
What is the optimal mode of delivery for the haemophilia carrier expecting an affected infant-vaginal delivery or caesarean delivery?对于怀有患病胎儿的血友病携带者而言,最佳分娩方式是顺产还是剖宫产?
Haemophilia. 2010 May;16(3):425-6. doi: 10.1111/j.1365-2516.2010.02238.x.
7
The optimal mode of delivery for the haemophilia carrier expecting an affected infant is caesarean delivery.对于预期生育血友病患儿的携带者,最佳分娩方式是剖宫产。
Haemophilia. 2010 May;16(3):420-4. doi: 10.1111/j.1365-2516.2009.02142.x. Epub 2009 Dec 16.
8
Management of carriers and babies with haemophilia.血友病携带者及患儿的管理
Haemophilia. 2008 Jul;14 Suppl 3:181-7. doi: 10.1111/j.1365-2516.2008.01721.x.
9
Thrombosis in a pregnant hemophilia A carrier after intrapartum recombinant factor VIII.产后重组凝血因子VIII治疗的甲型血友病携带者孕妇发生血栓形成
Obstet Gynecol. 2005 Apr;105(4):875-6. doi: 10.1097/01.AOG.0000141648.05771.24.
10
Management of pregnancy in a patient with severe haemophilia A.重度甲型血友病患者的妊娠管理
Br J Anaesth. 2003 Sep;91(3):432-5. doi: 10.1093/bja/aeg177.