Verghese Lynda, Tingi Efterpi, Thachil Jecko, Hay Charles, Byrd Louise
St. Mary's Hospital, Central Manchester University Hospitals Foundation Trust, Manchester Academic Health Science Centre, Manchester M13 9WL,UK.
St. Mary's Hospital, Central Manchester University Hospitals Foundation Trust, Manchester Academic Health Science Centre, Manchester M13 9WL,UK.
Eur J Obstet Gynecol Reprod Biol. 2017 Aug;215:85-92. doi: 10.1016/j.ejogrb.2017.06.008. Epub 2017 Jun 6.
This is an article reviewing the management of pregnant women with factor XI (FXI) deficiency. Retrospective review of the electronic records of 67 pregnancies in 25 women with FXI deficiency over a ten-year period was undertaken. All women received care at St Mary's Tertiary Referral Obstetric/Haematology Clinic for some or all of their pregnancies. Outcome measures included antenatal complications, mode of delivery, anaesthesia provided and postpartum haemorrhage (PPH) and management required. A positive bleeding history was identified in 50% of women prior to pregnancy. Fifteen pregnancies (22%) ended in first trimester miscarriage; there was 1 termination of pregnancy. Two pregnancies were complicated by Antepartum haemorrhage. Of the remaining 51 pregnancies there were 50 live births - 2 preterm and 48 at term. There was one antenatal (34 weeks gestation) stillbirth of a growth restricted baby and one neonatal death secondary to severe prematurity (24 weeks gestation). Twenty -five babies delivered vaginally (20 spontaneous and 5 instrumental). The remaining 26 were delivered by Caesarean section (9 elective and 17 emergency). A sub-analysis of 22 operative deliveries was reviewed; this suggested that regional anaesthesia was safe in selected women with FXI deficiency - a selection that was based on FXI level/range, presence/absence of bleeding history and intended operative intervention.Solvent detergent treated Fresh Frozen Plasma (SD-FFP/Octaplas) and Tranexamic Acid (TXA) were given to those considered vulnerable -an individualised decision made by the multidisciplinary team in accordance with BCSH guidance. Primary PPH complicated 10/51 (15%) deliveries. The commonest cause of PPH was due to atony. Secondary PPH was only seen in only one case. Bleeding in women with FXI deficiency is highly variable and, whilst it does not directly correlate with Factor XI levels, provision of replacement therapy is required if FXI levels are <15 IU/dL as per BCSH guidance. Women with Factor XI levels >40 IU/dL are considered safe for regional anaesthesia following prophylactic FFP as suggested by sub group analysis. Treatment of women with rare bleeding disorders during pregnancy should be by a multidisciplinary team of specialists, to include Haematologist, Anaesthetist and Obstetrician, all of whom have an interest in bleeding disorders in pregnancy. Decisions should then be individualised, based on the presence/absence of a bleeding history and the third trimester FXI levels. Delivery does not have to be by Elective Caesarean. With appropriate care both operative vaginal delivery and regional anaesthesia can be facilitated.
这是一篇关于对患有凝血因子XI（FXI）缺乏症的孕妇进行管理的综述文章。对25名患有FXI缺乏症的女性在十年期间的67次妊娠电子记录进行了回顾性分析。所有女性在圣玛丽三级转诊产科/血液科诊所接受了部分或全部孕期护理。观察指标包括产前并发症、分娩方式、所提供的麻醉以及产后出血（PPH）及其所需的处理措施。50%的女性在怀孕前有阳性出血史。15次妊娠（22%）在孕早期流产；有1次妊娠终止。2次妊娠并发产前出血。在其余51次妊娠中，有50例活产——2例早产，48例足月产。有1例妊娠34周时的产前死产，胎儿生长受限，还有1例因严重早产（妊娠24周）导致的新生儿死亡。25例经阴道分娩（20例自然分娩，5例器械助产）。其余26例通过剖宫产分娩（9例择期剖宫产，17例急诊剖宫产）。对22例手术分娩进行了亚组分析；结果表明，对于部分患有FXI缺乏症的女性，区域麻醉是安全的——这种选择是基于FXI水平/范围、有无出血史以及预期的手术干预情况。对那些被认为易出血的患者给予了溶剂去污剂处理的新鲜冰冻血浆（SD - FFP/Octaplas）和氨甲环酸（TXA）——这是多学科团队根据英国血液学标准委员会（BCSH）的指导做出的个体化决定。原发性PPH使10/51（15%）的分娩过程复杂化。PPH最常见的原因是子宫收缩乏力。继发性PPH仅在1例中出现。FXI缺乏症女性的出血情况差异很大，虽然它与凝血因子XI水平没有直接关联，但根据BCSH的指导，如果FXI水平<15 IU/dL，则需要进行替代治疗。亚组分析表明，凝血因子XI水平>40 IU/dL的女性在预防性输注新鲜冰冻血浆后进行区域麻醉被认为是安全的。孕期患有罕见出血性疾病的女性应由多学科专家团队进行治疗，包括血液科医生、麻醉科医生和产科医生，他们都对孕期出血性疾病感兴趣。然后应根据有无出血史和孕晚期FXI水平进行个体化决策。分娩不一定必须通过择期剖宫产。通过适当的护理，手术阴道分娩和区域麻醉都可以顺利进行。
