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分析 34 名患有五种不同类型遗传性血小板功能障碍的女性中的 65 例妊娠。

Analysis of 65 pregnancies in 34 women with five different forms of inherited platelet function disorders.

机构信息

Department of Internal Medicine, University of Pavia-IRCCS Policlinico San Matteo Foundation, Pavia, Italy.

Service of Biometry & Statistics, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.

出版信息

Br J Haematol. 2015 Aug;170(4):559-63. doi: 10.1111/bjh.13458. Epub 2015 Apr 21.

Abstract

This study evaluated 65 pregnancies in 34 women with five different inherited platelet function disorders. Gestation was similar to that of the general population. Severe bleeds requiring blood transfusions were observed in 50% of deliveries in Glanzmann thrombasthenia (GT), but not in the patients with delta storage pool disease, Hermansky-Pudlak syndrome, P2Y12 defect or defect of thromboxane A2 receptor. Of note, severe haemorrhage also occurred in women with GT who had received prophylactic platelet transfusions, suggesting that better preventive treatments are required. Diagnosis and degree of spontaneous bleeding tendency before pregnancy were reliable parameters to predict the delivery-related bleeding risk.

摘要

本研究评估了 34 名患有五种不同遗传性血小板功能障碍的女性的 65 例妊娠。妊娠情况与普通人群相似。在 Glanzmann 血小板无力症(GT)患者中,50%的分娩出现严重出血需要输血,但在 delta 储存池病、Hermansky-Pudlak 综合征、P2Y12 缺陷或血栓烷 A2 受体缺陷患者中未出现这种情况。值得注意的是,接受预防性血小板输注的 GT 患者也发生严重出血,这表明需要更好的预防治疗。妊娠前的诊断和自发性出血倾向程度是预测分娩相关出血风险的可靠参数。

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