Gekas J, Broermann L, Heidenreich W
Frauenklinik, Allgemeines Krankenhaus Celle, Germany.
Z Geburtshilfe Neonatol. 2007 Apr;211(2):90-2. doi: 10.1055/s-2007-960585.
Haemophilia B is a congenital coagulation disorder with a low activity of the coagulation factor IX. The means of transmission is recessively gender dependent. Usually the female carrier of the haemophilia B has normal activity of the coagulation factor. However, in very rare cases the factor-IX-activity may be reduced considerably, even in a female carrier (Lyon-hypothesis). Both for mother and the child, who has potentially inherited the disorder, there is a dramatically increased tendency for bleeding with the potential for developing further complications.
The deliveries of two conductors with a low activity of coagulation factor IX are presented. With a monitored substitution of factor-IX-concentrate it was possible to prolong the pregnancies nearly before the date of birth. In one case ended the pregnancy in a caesarean section, the second patient had a normal delivery.
Today interdisciplinary treatment and exactly planning of the birth may led even high risk pregnancies to a successful end.
乙型血友病是一种先天性凝血障碍，凝血因子IX活性较低。传播方式为隐性性别依赖。通常，乙型血友病的女性携带者凝血因子活性正常。然而，在极少数情况下，即使是女性携带者，因子IX活性也可能大幅降低（莱昂假说）。对于母亲和可能遗传了该疾病的孩子来说，出血倾向显著增加，并有进一步发展为并发症的可能。
介绍了两名凝血因子IX活性较低的携带者的分娩情况。通过监测因子IX浓缩物的替代治疗，几乎可以将妊娠延长至预产期。其中一例通过剖宫产结束妊娠，另一例患者顺产。
如今，跨学科治疗和精确的分娩计划甚至可以使高风险妊娠成功结束。
