Kadir R A, Lee C A, Sabin C A, Pollard D, Economides D L
University Department of Obstetrics and Gynaecology, Royal Free Hospital, London.
Br J Obstet Gynaecol. 1998 Mar;105(3):314-21. doi: 10.1111/j.1471-0528.1998.tb10093.x.
To assess the obstetric outcome in women with von Willebrand's disease or factor XI deficiency.
Haemophilia Centre and Haemostasis Unit, The Royal Free Hospital.
Women with von Willebrand's disease (n = 31) and with factor XI deficiency (n = 11) registered at the Royal Free Hospital Haemophilia Centre who had had a pregnancy within the previous 17 years (1980-1996), including 84 in women with von Willebrand's disease and 28 in women with factor XI deficiency.
Women were interviewed and details of the obstetric history were obtained. The records of the Haemophilia Centre and the women's maternity records were also reviewed.
Threatened miscarriage occurred in 33% and 14% of pregnancies with von Willebrand's disease and factor XI deficiency, respectively. Excluding recurrent miscarriages, 14/68 (21%) of pregnancies with von Willebrand's disease and one pregnancy with factor XI deficiency miscarried spontaneously. There was an increased incidence of primary and secondary post-abortal bleeding complications. Factor VIII and von Willebrand factor antigen and activity levels increased significantly in pregnancy in all women apart from those with severe von Willebrand's disease. Factor XI, however, did not show any significant change. No neonatal haemorrhagic complications in association with the birth process were reported, although ventouse and difficult forceps deliveries were avoided. Extensive perineal bruising and haematoma was reported in three women with von Willebrand's disease; two of these were associated with forceps delivery. The incidence of primary postpartum haemorrhage was 18.5% in von Willebrand's disease and 16% in factor XI deficiency. Blood transfusion was required in six cases of von Willebrand's disease and two cases of factor XI deficiency. Ten of fourteen instances of primary postpartum haemorrhage occurred when maternal factor levels were < 50 IU/dL with no prophylactic treatment for labour. The incidence of secondary postpartum haemorrhage was 20% in von Willebrand's disease and 24% in factor XI deficiency. None of the women who had prophylactic treatment during labour or the puerperium suffered any significant bleeding complications. There were three neonatal bleeding complications.
Pregnancy, labour and the puerperium are associated with significant bleeding problems in women with von Willebrand's disease or factor XI deficiency, but these are largely preventable. Specialist obstetric care in close liaison with the haemophilia centre is essential to minimise maternal and neonatal complications.
评估血管性血友病或因子 XI 缺乏症女性的产科结局。
皇家自由医院血友病中心及止血科。
在皇家自由医院血友病中心登记的血管性血友病女性（n = 31）和因子 XI 缺乏症女性（n = 11），她们在过去 17 年（1980 - 1996 年）内有过妊娠，其中血管性血友病女性 84 例次，因子 XI 缺乏症女性 28 例次。
对女性进行访谈并获取产科病史细节。同时查阅血友病中心记录及女性的产科记录。
血管性血友病和因子 XI 缺乏症妊娠女性中，分别有 33%和 14%发生先兆流产。排除复发性流产，血管性血友病妊娠女性中有 14/68（21%）自然流产，因子 XI 缺乏症妊娠女性中有 1 例自然流产。流产后原发性和继发性出血并发症发生率增加。除重度血管性血友病女性外，所有女性孕期因子 VIII、血管性血友病因子抗原及活性水平均显著升高。然而，因子 XI 无显著变化。尽管避免了使用胎头吸引术和困难的产钳助产，但未报告与分娩过程相关的新生儿出血并发症。3 例血管性血友病女性出现广泛的会阴瘀斑和血肿，其中 2 例与产钳助产有关。血管性血友病女性原发性产后出血发生率为 18.5%，因子 XI 缺乏症女性为 16%。血管性血友病 6 例、因子 XI 缺乏症 2 例需要输血。14 例原发性产后出血中有 10 例发生在母体因子水平 < 50 IU/dL 且分娩时未进行预防性治疗的情况下。血管性血友病女性继发性产后出血发生率为 20%，因子 XI 缺乏症女性为 24%。在分娩或产褥期接受预防性治疗的女性均未发生严重出血并发症。有 3 例新生儿出血并发症。
血管性血友病或因子 XI 缺乏症女性在妊娠、分娩及产褥期存在显著的出血问题，但这些问题大多可预防。与血友病中心密切协作的专科产科护理对于将母婴并发症降至最低至关重要。
