The Haemophilia Centre and Thrombosis Unit, The Royal Free Hospital, London, UK.
J Thromb Haemost. 2013 Jun;11 Suppl 1:170-9. doi: 10.1111/jth.12267.
The past few decades have seen major advances in multidisciplinary obstetric care and management of gynecological conditions in women with bleeding disorders. Awareness of the impact of bleeding disorders has improved among the obstetric and gynecological community. Undiagnosed bleeding disorders can be the underlying cause for a significant proportion of women with heavy menstrual bleeding. They may also be the cause or a contributory factor for other gynecological problems, such as dysmenorrhea, intermenstrual bleeding, and endometriosis. Hemostatic assessment should be considered in women referred for menstrual abnormalities if they have a positive bleeding history as quantified by bleeding assessment tools. The reproductive choices and options for prenatal diagnosis are also expanding for families with hemophilia with a drive toward achieving a non-invasive approach. Current non-invasive prenatal diagnostic techniques are limited to identification of fetal gender. Research is ongoing to overcome the specific diagnostic challenges of identifying hemophilia mutations, utilizing free fetal DNA circulating in maternal plasma. The management of obstetric hemorrhage has recently evolved to include a greater focus on the identification of and early treatment for coagulation disorders. Deficiencies in certain hemostatic variables are associated with progression to more severe bleeding; therefore, specific interventions have been proposed to target this. Evidence is still lacking to support such strategy, and future research is required to assess the efficacy and the safety of these hemostatic interventions in women with persistent PPH.
过去几十年,在多学科产科护理和出血性疾病女性妇科疾病管理方面取得了重大进展。产科和妇科医生对出血性疾病的认识有所提高。未确诊的出血性疾病可能是相当一部分月经过多女性的根本原因。它们也可能是其他妇科问题(如痛经、经间期出血和子宫内膜异位症)的原因或促成因素。如果接受月经异常转诊的女性有阳性出血史(如出血评估工具所量化的),则应考虑进行止血评估。对于血友病患者家庭来说,产前诊断的生殖选择和方案也在扩大,他们希望采用非侵入性方法。目前的非侵入性产前诊断技术仅限于识别胎儿性别。正在进行研究,以利用游离胎儿 DNA 在母体血浆中的循环来克服识别血友病突变的具体诊断挑战。产科出血的管理最近已经发展到更加关注识别和早期治疗凝血障碍。某些止血变量的缺乏与更严重出血的进展有关;因此,提出了针对这些情况的具体干预措施。目前仍缺乏支持这种策略的证据,需要进一步的研究来评估这些止血干预措施在持续性 PPH 女性中的疗效和安全性。