Suppr超能文献

伯纳德·索利尔综合征患者的成功妊娠与分娩管理

Successful pregnancy and delivery management in a patient with Bernard Soulier Syndrome.

作者信息

Demirci Ufuk, Erbilen Esra Altan, Ümit Elif Gülsüm, İnan Cihan, Sayın N Cenk, Demir Ahmet Muzaffer

机构信息

Department of Hematology, Trakya University, Medical Faculty, Edirne, Turkey.

Department of Obstetrics and Gynecology, Trakya University, Medical Faculty, Edirne, Turkey.

出版信息

Obstet Med. 2023 Sep;16(3):203-205. doi: 10.1177/1753495X211067119. Epub 2022 Jan 13.

Abstract

Bernard Soulier Syndrome (BSS) is an inherited bleeding disorder characterized by macrothrombocytopenia and absence of ristocetin-induced platelet aggregation. Clinical findings vary from person to person. Most of the patients are diagnosed with muco-cutaneous bleeding such as purpura, epistaxis and gingival bleeding in early childhood. Few pregnant women with BSS are described in the literature. Management of thrombocytopenia during pregnancy and delivery requires a multidisciplinary approach. The family should be warned about the potentially life-threatening bleeding during pregnancy and the delivery and the decision about mode of delivery should be individualised, involving discussion with patient and multidisciplinary team.

摘要

伯纳德-索利尔综合征(BSS)是一种遗传性出血性疾病,其特征为巨血小板减少症以及瑞斯托霉素诱导的血小板聚集缺乏。临床症状因人而异。大多数患者在幼儿期被诊断为有黏膜皮肤出血,如紫癜、鼻出血和牙龈出血。文献中描述的患有BSS的孕妇很少。孕期和分娩期间血小板减少症的管理需要多学科方法。应告知家属孕期和分娩期间可能危及生命的出血情况,分娩方式的决定应个体化,需与患者及多学科团队进行讨论。

相似文献

1
Successful pregnancy and delivery management in a patient with Bernard Soulier Syndrome.
Obstet Med. 2023 Sep;16(3):203-205. doi: 10.1177/1753495X211067119. Epub 2022 Jan 13.
2
Bernard-Soulier Syndrome from the Perspective of the Obstetrician: A Case Report with a Review of the Literature.
Z Geburtshilfe Neonatol. 2023 Jun;227(3):168-178. doi: 10.1055/a-2024-0819. Epub 2023 Mar 8.
3
[Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: report of two cases].
Srp Arh Celok Lek. 2014 May-Jun;142(5-6):351-5. doi: 10.2298/sarh1406351m.
4
Bernard-Soulier syndrome caused by two novel heterozygous gene mutations: a case report and literature review.
Hematology. 2024 Dec;29(1):2334642. doi: 10.1080/16078454.2024.2334642. Epub 2024 Apr 2.
5
Use of recombinant factor VIIa in the management of severe bleeding episodes in patients with Bernard-Soulier syndrome.
Ann Hematol. 2005 Nov;84(12):816-22. doi: 10.1007/s00277-005-1080-y. Epub 2005 Nov 12.
6
Bernard Soulier syndrome in pregnancy: a systematic review.
Haemophilia. 2010 Jul 1;16(4):584-91. doi: 10.1111/j.1365-2516.2009.02137.x. Epub 2010 Jan 12.
7
Clinical Dilemma, Bernard Soulier Syndrome Immune Thrombocytopenic Purpura: A Case Report.
Cardiovasc Hematol Disord Drug Targets. 2023;23(1):72-75. doi: 10.2174/1871529X23666230419095422.
9
Bernard-Soulier syndrome: an inherited platelet disorder.
Arch Pathol Lab Med. 2007 Dec;131(12):1834-6. doi: 10.5858/2007-131-1834-BSAIPD.
10
Bernard-Soulier syndrome in pregnancy.
Clin Lab Haematol. 2006 Jun;28(3):198-201. doi: 10.1111/j.1365-2257.2006.00780.x.

本文引用的文献

1
Prophylaxis of venous thromboembolism during pregnancy.
Rev Clin Esp. 2020 Jul 6. doi: 10.1016/j.rce.2020.06.006.
2
Thrombocytopenia in Pregnancy: Approach to Diagnosis and Management.
Semin Thromb Hemost. 2020 Apr;46(3):256-263. doi: 10.1055/s-0040-1708842. Epub 2020 Apr 7.
4
[Bernard-Soulier syndrome and pregnancy: a case report].
Ann Biol Clin (Paris). 2015 Nov-Dec;73(6):737-40. doi: 10.1684/abc.2015.1101.
5
Primigravida with Bernard-Soulier Syndrome: a case report.
BMC Res Notes. 2015 May 1;8:178. doi: 10.1186/s13104-015-1145-5.
6
[Obstetric and gynecological intervention in women with Bernard-Soulier syndrome: report of two cases].
Srp Arh Celok Lek. 2014 May-Jun;142(5-6):351-5. doi: 10.2298/sarh1406351m.
7
Inherited platelet disorders including Glanzmann thrombasthenia and Bernard-Soulier syndrome.
Hematology Am Soc Hematol Educ Program. 2013;2013:268-75. doi: 10.1182/asheducation-2013.1.268.
8
Bernard Soulier syndrome in pregnancy: a systematic review.
Haemophilia. 2010 Jul 1;16(4):584-91. doi: 10.1111/j.1365-2516.2009.02137.x. Epub 2010 Jan 12.
10
Bernard-Soulier syndrome (hemorrhagiparous thrombocytic dystrophy).
Orphanet J Rare Dis. 2006 Nov 16;1:46. doi: 10.1186/1750-1172-1-46.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验