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一名患有严重Glanzmann血小板无力症患者的妊娠与分娩

Pregnancy and parturition in a patient with severe Glanzmann's thrombasthenia.

作者信息

Sundqvist S B, Nilsson I M, Svanberg L, Cronberg S

出版信息

Scand J Haematol. 1981 Sep;27(3):159-64. doi: 10.1111/j.1600-0609.1981.tb00467.x.

DOI:10.1111/j.1600-0609.1981.tb00467.x
PMID:7313543
Abstract

A 35-year-old woman with severe Glanzmann's thrombasthenia became pregnant involuntarily. As a late abortion might cause a bleeding as severe as a delivery, legal abortion was not considered indicated. Treatment with ordinary platelet concentrates was not considered since even HLA-matched platelets did not enhance the platelet function, measured as the aggregability of transfused platelets. This inability was thought to indicate that the patient might have developed antibodies against one of the glycoproteins deficient in this disease. A delivery per vaginam was then preferred because of the local compressing effect. Spontaneous delivery occurred at the end of the 42nd week of pregnancy. The patient delivered a boy with transient thrombocytopenia, probably due to maternal antibodies. To stimulate the haemostasis, uterine contracting drugs were used in unusually large doses and for a relatively long period of time. Tranexamic acid was also given. No haemorrhagic complications could he observed during the puerperium.

摘要

一名35岁患有严重Glanzmann血小板无力症的女性意外怀孕。由于晚期流产可能导致与分娩一样严重的出血,因此不考虑进行合法流产。普通血小板浓缩物治疗未被采用,因为即使是 HLA 匹配的血小板也无法增强血小板功能(通过输注血小板的聚集性来衡量)。这种无能力被认为表明患者可能已经产生了针对该疾病中缺乏的一种糖蛋白的抗体。由于局部压迫作用,当时更倾向于经阴道分娩。妊娠42周结束时自然分娩。患者产下一名患有短暂性血小板减少症的男婴,可能是由于母体抗体所致。为促进止血,使用了超大剂量且相对较长时间的子宫收缩药物。还给予了氨甲环酸。产褥期未观察到出血并发症。

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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
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Menstrual and obstetrical bleeding in women with inherited platelet receptor defects-A systematic review.女性遗传性血小板受体缺陷相关的月经和产科出血:系统评价。
Haemophilia. 2020 Mar;26(2):216-227. doi: 10.1111/hae.13927. Epub 2020 Jan 31.
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Glanzmann's Thrombasthenia Complicating Pregnancy.妊娠合并Glanzmann血小板无力症
J Obstet Gynaecol India. 2014 Dec;64(Suppl 1):3-5. doi: 10.1007/s13224-012-0319-5. Epub 2013 Apr 11.
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Investigation and management of haemorrhagic disorders in pregnancy. Haemostasis and Thrombosis Task Force.妊娠期出血性疾病的调查与管理。止血与血栓形成特别工作组。
J Clin Pathol. 1994 Feb;47(2):100-8. doi: 10.1136/jcp.47.2.100.