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遗传性出血性疾病女性的妊娠与分娩管理。

Management of pregnancy and delivery in women with inherited bleeding disorders.

机构信息

U.O.S. Dipartimentale per la Diagnosi e la Terapia delle Coagulopatie, A. Bianchi Bonomi Haemophilia and Thrombosis Centre, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Università degli Studi di Milano and Luigi Villa Foundation, Milan, Italy.

出版信息

Semin Fetal Neonatal Med. 2011 Dec;16(6):311-7. doi: 10.1016/j.siny.2011.07.006. Epub 2011 Aug 17.

Abstract

Women with inherited bleeding disorders present a wide spectrum of clinical symptoms that vary from mild or moderate bleeding tendency to severe episodes. Monthly haemostatic changes affect these women during menstruation and ovulation. These events may be associated with significant bleeding and pain leading to the limitations in conducting daily activities and adverse effect on quality of life. Likewise, pregnancy and delivery are critical times for affected women. During pregnancy, they may be at greater risk of miscarriage and bleeding complications. In particular, recurrent miscarriage was observed in women with type 3 von Willebrand disease, afibrinogenaemia and severe factor XIII deficiency, and an optimal therapeutic plan is required during their pregnancy. Precautions must be taken at delivery in these women, since they could be at risk of bleeding. The lack of adequate information makes it very difficult to prepare evidence-based guidelines for the prevention of bleedings in affected women and their treatment. A multidisciplinary team of obstetricians, haematologists and paediatricians is required with a good knowledge of these disorders and an awareness of the potential maternal neonatal complications.

摘要

患有遗传性出血性疾病的女性表现出广泛的临床症状,从轻微或中度出血倾向到严重发作不等。每月的止血变化会影响这些女性在月经和排卵期间的身体状况。这些情况可能会导致大量出血和疼痛,从而限制日常活动并对生活质量产生不利影响。同样,怀孕和分娩对受影响的女性来说是关键时刻。怀孕期间,她们可能更容易流产和出血并发症。特别是在 3 型血管性血友病、无纤维蛋白原血症和严重因子 XIII 缺乏症的女性中观察到复发性流产,需要在怀孕期间制定最佳治疗计划。在这些女性分娩时必须采取预防措施,因为她们可能有出血的风险。由于缺乏足够的信息,因此很难为受影响的女性预防出血和治疗制定基于证据的指南。需要一支由产科医生、血液学家和儿科医生组成的多学科团队,他们对这些疾病有很好的了解,并意识到潜在的母婴并发症。

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