Déficit en facteur X et grossesse
Déficit en facteur X et grossesse
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X 事实赤字和总赤字
DOI：
10.1016/s0750-7658(02)00646-9
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发表时间：
2002
期刊：
影响因子：
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通讯作者：
J. Audibert
中科院分区：
文献类型：
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作者：
K. Rezig;N. Diar;D. Benabidallah;J. Audibert
因子X缺乏症是一种罕见的遗传性凝血障碍。它是一种常染色体隐性遗传病。在纯合子形式中，因子X缺乏症的患病率估计为1:50万。然而，在杂合子形式下，其频率估计为1:500至1:2000。患有先天性因子X缺乏症的妇女怀孕与不良胎儿结局有关。我们报告一例怀孕妇女与因子X缺乏症。她在分娩早期接受预防性替代凝血酶原复合物浓缩物（Kaskadil）治疗。初始输注40ui。X因子kg-1后加20 UI。Kg-1，三天内每24小时一次。在分娩和围产期间进行了产妇凝血筛查。她在怀孕33周时生下了一个健康的婴儿。未见异常出血发作。因此，在这种情况下，在分娩期间使用凝血酶原复合物浓缩液的预防性治疗确实可以预防严重出血。
Factor X deficiency is one of the rarest inherited coagulation disorders. It is an autosomal recessive inherited disease. In its homozygous form factor X deficiency has an estimated prevalence of 1: 500,000. However in its heterozygous form it has an estimated frequency of 1: 500 to 1: 2000. Pregnancy in women with congenital factor X deficiency has been associated with adverse foetal outcomes. We report a case of pregnancy in a woman with factor X deficiency. She was treated early during labour with prophylactic replacement of prothrombin complex concentrates (Kaskadil). An initial infusion of 40 UI. kg-1 of factor X was followed by 20 UI. kg-1 every 24 hours during three days. During labour and peripartum maternal coagulation was screened. She delivered a healthy baby at 33 weeks of gestation. No episode of abnormal bleeding was observed. Therefore in this case, prophylactic therapy using prothrombin complex concentrates during labour and delivery did prevent severe haemorrhages.