Combined deficiency of factors II, VII, IX, and X (Borgschulte-Grigsby deficiency) in pregnancy
Combined deficiency of factors II, VII, IX, and X (Borgschulte-Grigsby deficiency) in pregnancy
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DOI：
10.1016/s0029-7844(00)01214-x
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发表时间：
2001-05-01
影响因子：
7.2
通讯作者：
James, AH
中科院分区：
文献类型：
--
作者：
McMahon, MJ;James, AH
背景：维生素 K 依赖性凝血因子（II、VII、IX、X）联合缺乏对孕妇来说是一个罕见的挑战。病例：一名 34 岁的初产妇患有先天性因子 II、VII、IX 和 X 联合缺乏，这些因子对维生素 K 不完全敏感。她的维生素 K 依赖因子发生了改变，保留了免疫活性，但缺乏凝血活性和正常补体。 γ-羧基谷氨酸残基。她一生都需要补充维生素 K。在一次平安的怀孕后，她因会阴切开术导致产后出血。给予新鲜冰冻血浆以实现止血。她的产后病程的其余部分是正常的。结论：II、VII、IX 和 X 因子组合先天性缺乏症可以在怀孕期间通过使用维生素 K 和新鲜冰冻血浆进行治疗。 (C) 2001 年，美国妇产科医师学会。
Background: Combined deficiency of vitamin K-dependent coagulation factors (II, VII, IX, X) is an uncommon challenge for the expectant gravida.Case: A 34-year-old primigravida had congenital combined deficiency of factors II, VII, IX, and X that were incompletely sensitive to vitamin K. She had an altered form of vitamin K-dependent factors that retained immunologic activity but lacked coagulant activity and the normal complement of gamma-carboxyglutamic acid residues. She required vitamin K supplementation throughout her life. After an uneventful pregnancy she had postpartum hemorrhage resulting from an episiotomy. Fresh frozen plasma was administered to achieve hemostasis. The remainder of her postpartum course was normal.Conclusion: Combined congenital deficiency of factors II, VII, IX, and X can be managed in pregnancy with the use of vitamin K and fresh frozen plasma. (C) 2001 by The American College of Obstetricians and Gynecologists.