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一名患有先天性因子 VII 缺乏症和 Glanzmann 血小板无力症变异型的智利患者的孕期管理

Management of Pregnancy in a Chilean Patient with Congenital Deficiency of Factor VII and Glanzmann's Thrombasthenia Variant.

作者信息

Murray Nigel P, Garcia Claudio, Ilabaca Javier, Lagos Nestor

机构信息

Department of Hematology, Hospital de Carabineros de Chile, Simon Bolivar 2200, Nuñoa, 7770199 Santiago, Chile ; Faculty of Medicine, University Mayor, Renato Sanchez 4369, Las Condes, 7550224 Santiago, Chile.

Faculty of Medicine, University Mayor, Renato Sanchez 4369, Las Condes, 7550224 Santiago, Chile ; Feto-Maternity Unit, Hospital de Carabineros de Chile, Simon Bolivar 2200, Nuñoa, 7770199 Santiago, Chile.

出版信息

Case Rep Obstet Gynecol. 2014;2014:628386. doi: 10.1155/2014/628386. Epub 2014 Dec 1.

DOI:10.1155/2014/628386
PMID:25525535
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4266761/
Abstract

Patients with inherited bleeding disorders are rare in obstetric practice but present with prolonged bleeding even after minor invasive procedures. They require a combined approach with obstetric and hematological management of each case, including the neonatal management of a possibly affected fetus. We present the case of a pregnancy in a patient with combined Factor VII deficiency and Glanzmann's thrombasthenia, the successful obstetric and hematological management of the case, and a review of the literature.

摘要

遗传性出血性疾病患者在产科实践中较为罕见,但即使在进行小的侵入性手术后也会出现出血时间延长的情况。对于每一例患者,都需要产科和血液科联合管理,包括对可能受影响胎儿的新生儿管理。我们报告一例合并因子VII缺乏症和Glanzmann血小板无力症患者的妊娠病例,该病例成功的产科和血液科管理过程,并对相关文献进行综述。

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1
Management of Pregnancy in a Chilean Patient with Congenital Deficiency of Factor VII and Glanzmann's Thrombasthenia Variant.一名患有先天性因子 VII 缺乏症和 Glanzmann 血小板无力症变异型的智利患者的孕期管理
Case Rep Obstet Gynecol. 2014;2014:628386. doi: 10.1155/2014/628386. Epub 2014 Dec 1.
2
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Am J Perinatol. 1999;16(6):297-301. doi: 10.1055/s-2007-993875.
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J Perinat Med. 2004;32(5):456-8. doi: 10.1515/JPM.2004.147.
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Eptacog alfa activated: a recombinant product to treat rare congenital bleeding disorders.活化依普他肽:一种用于治疗罕见先天性出血性疾病的重组产品。
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Dental considerations for a Glanzmann's thrombasthenia patient: case report.一名Glanzmann血小板无力症患者的牙科注意事项:病例报告
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引用本文的文献

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Vaginal Delivery in a Primipara with Glanzmann Thrombasthenia.初产妇合并血小板无力症的阴道分娩
Matern Fetal Med. 2023 Jul;5(3):192-194. doi: 10.1097/FM9.0000000000000187. Epub 2023 Apr 4.
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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.
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Diagnosis and treatment discussion of congenital factor VII deficiency in pregnancy: A case report.妊娠合并先天性凝血因子Ⅶ缺乏症的诊断与治疗探讨:一例报告
World J Clin Cases. 2021 Jul 26;9(21):6091-6101. doi: 10.12998/wjcc.v9.i21.6091.

本文引用的文献

1
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.
2
[Prophylactic use of a recombinant activated factor VII in delivery haemorrhage by caesarean in a woman with major factor VII deficiency: a case report].[重组活化凝血因子 VII 在严重凝血因子 VII 缺乏的剖宫产产妇分娩出血中的预防性应用:一例报告]
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Haemophilia. 2011 Sep;17(5):e858-69. doi: 10.1111/j.1365-2516.2011.02516.x. Epub 2011 Apr 4.
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[Postpartum hemorrhage successfully treated with recombinant factor VIIa in Glanzmann thromboasthenia].[重组凝血因子VIIa成功治疗血小板无力症患者产后出血]
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A review of inherited platelet disorders with guidelines for their management on behalf of the UKHCDO.代表英国血液学临床医师组织对遗传性血小板疾病及其管理指南进行的综述。
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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。
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A case of Glanzmann's thrombasthenia successfully treated with recombinant factor viia during a surgical procedure: observations on the monitoring and the mechanism of action of this drug.一例在外科手术期间用重组凝血因子VIIa成功治疗的Glanzmann血小板无力症:关于该药物监测及作用机制的观察
Haematologica. 2006 Jun;91(6 Suppl):ECR20.
8
Congenital SPCA deficiency: a hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fractions.先天性血清凝血活酶原复合物缺乏症:一种迄今未被认识的凝血缺陷,可通过血清和血清成分纠正出血。
J Clin Invest. 1951 Jun;30(6):596-608. doi: 10.1172/JCI102477.
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Blood. 1956 Nov;11(11):965-76.
10
Molecular basis of Glanzmann's Thrombasthenia and current strategies in treatment.血小板无力症的分子基础及当前治疗策略
Blood Rev. 2002 Sep;16(3):193-202. doi: 10.1016/s0268-960x(02)00030-9.