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重组凝血因子VIIa(rfVIIa)用于先天性凝血因子VII缺乏孕妇的围产期出血预防:一例病例报告及文献综述

Prophylaxis of peripartum haemorrhage using recombinant factor VIIa (rfVIIa) in pregnant women with congenital factor VII deficiency: A case report and literature review.

作者信息

Loddo Alessandro, Cornacchia Stefania, Cane Fiorenza Lo, Barcellona Doris, Marongiu Francesco, Melis Gian Benedetto, Angioni Stefano, Paoletti Anna Maria, Neri Manuela

机构信息

Department of Surgical Sciences, Clinic of Obstetrics and Gynecology, University Hospital of Cagliari, Italy.

Department of Surgical Sciences, Clinic of Obstetrics and Gynecology, University Hospital of Cagliari, Italy.

出版信息

Eur J Obstet Gynecol Reprod Biol. 2019 Apr;235:77-80. doi: 10.1016/j.ejogrb.2019.02.017. Epub 2019 Feb 22.

Abstract

Congenital factor VII deficiency is a rare autosomal recessive disorder associated to different haemorrhagic manifestations. Labour and delivery may cause bleeding risk in patients with this coagulation deficit, thus it is appropriate to clarify whether prophylaxis of peripartum haemorrhage is necessary. To date, there are very few cases in scientific literature which report the management of women with congenital factor VII deficiency during labour, and a consensus for prophylaxis does not exist. In this manuscript we present the management of a 35 years old woman with factor VII deficiency, treated with recombinant factor VIIa before delivery, without haemorrhagic complications either for the woman and for the infant. Therefore, we present a review of similar cases managed with a peripartum prophylaxis with recombinant factor VIIa, and discuss its usefulness and effectiveness, in view of the severity of the deficit and the doses used.

摘要

先天性因子 VII 缺乏症是一种罕见的常染色体隐性疾病,与不同的出血表现相关。分娩可能会给患有这种凝血缺陷的患者带来出血风险,因此,明确围产期出血的预防是否必要是恰当的。迄今为止,科学文献中报道分娩期间先天性因子 VII 缺乏症女性管理情况的病例非常少,且不存在预防的共识。在本手稿中,我们介绍了一名 35 岁因子 VII 缺乏症女性的管理情况,该女性在分娩前接受了重组因子 VIIa 治疗,无论是对母亲还是婴儿均未出现出血并发症。因此,鉴于缺陷的严重程度和所使用的剂量,我们对使用重组因子 VIIa 进行围产期预防管理的类似病例进行了综述,并讨论了其有用性和有效性。

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