Department of Obstetrics and Gynaecology, Royal Free Hospital, Pond Street, London, UK.
Best Pract Res Clin Obstet Gynaecol. 2012 Feb;26(1):103-17. doi: 10.1016/j.bpobgyn.2011.10.005. Epub 2011 Nov 18.
Women with inherited bleeding disorders may face several haemostatic challenges during pregnancy and childbirth. Pregnancy in these women requires specialised and individualised care. Prenatal diagnosis is primarily considered in families affected by severe bleeding disorder such as haemophilia. Non-invasive fetal sex determination by analysis of free fetal DNA in maternal blood offers carriers of haemophilia a means of avoiding invasive testing and its associated risks in female pregnancies. With the exception of fibrinogen and factor XIII deficiencies, it is currently unclear whether women with inherited bleeding disorders are at increased risk of miscarriage or antepartum haemorrhage. However, they are at increased risk of primary and secondary postpartum haemorrhage. The fetus, if severely affected, is at risk of cranial bleeding during labour and delivery. Appropriate haemostatic cover during labour and delivery, avoidance of prolonged labour and traumatic delivery, and active management of third stage of labour can minimise the risk of bleeding complications for the mother and her fetus.
患有遗传性出血性疾病的女性在妊娠和分娩期间可能会面临多种止血挑战。这些女性的妊娠需要专门的个体化护理。产前诊断主要考虑在受严重出血性疾病（如血友病）影响的家庭中进行。通过分析母体血液中的游离胎儿 DNA 进行非侵入性胎儿性别鉴定，为血友病携带者提供了一种避免女性妊娠中侵入性检测及其相关风险的方法。除纤维蛋白原和因子 XIII 缺乏症外，目前尚不清楚遗传性出血性疾病女性是否有更高的流产或产前出血风险。然而，她们有更高的原发性和继发性产后出血风险。如果胎儿受到严重影响，分娩过程中会有颅出血的风险。分娩和分娩期间适当的止血覆盖、避免产程延长和创伤性分娩以及第三产程的积极管理，可以最大限度地降低母亲及其胎儿出血并发症的风险。
