Miguel López, Matías Fica, Verónica Soto, Francisco Samaniego
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引用次数: 0
摘要
组织因子-因子 VII 复合物在凝血活化过程中至关重要。先天性因子 VII 缺乏症是一种罕见的常染色体隐性遗传疾病。临床表现多种多样,既有无症状的携带者,也有需要进行因子 VII 替代治疗的严重出血表型。治疗方法有血浆提取物、重组 FVII 浓缩物或新鲜冷冻血浆,以备不时之需。轻度和中度缺乏的孕妇体内 VII 因子水平会升高,而重度缺乏的孕妇体内 VII 因子水平不会升高。对 FVII 缺乏症孕妇的治疗必须由血液科、产科和麻醉科医生组成的多学科团队进行,并以孕妇的出血史、凝血试验、FVII 因子水平和分娩路线为指导。我们介绍了一名 31 岁孕妇的病例,由于凝血酶原时间的改变,她被诊断为因子 VII 缺乏症,并对其进行了相应的产科处理。
[Management of pregnant women with deficiency of factor VII. Report of one case].
Tissue Factor-Factor VII complex is essential in coagulation activation. Congenital factor VII deficiency is a rare disorder that has an autosomal recessive inheritance. Clinical presentations are heterogeneous, ranging from asymptomatic carriers to severe bleeding phenotypes with factor VII replacement therapy requirements. Treatments options are plasma derived and recombinants FVII concentrates or fresh frozen plasma in case that first options are not available. In pregnancy factor VII levels increase in women with mild and moderate deficiencies but not in severe deficiency. The management of pregnant women with factor FVII deficiency must be done by a multidisciplinary team of hematologist, obstetrics and anesthetist and should be guided by the women bleeding history, the coagulations test, levels of factor FVII and rout of delivery. We present the case of a 31-year-old pregnant woman who, due to an alteration in prothrombin time, is diagnosed with Factor VII deficiency and its respective obstetric management.