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一名患有凝血因子 VII 缺乏症患者的复发性颅内出血:病例报告

Recurrent intracranial haemorrhages in a patient with factor seven deficiency: a case report.

作者信息

Fadzil F

机构信息

Jabatan Neurosains, Pusat Pengajian Sains Perubatan, Universiti Sains Malaysia, Kampus Kesihatan, 16150 Kubang Kerian, Kelantan.

出版信息

Med J Malaysia. 2011 Aug;66(3):261-3.

Abstract

Inherited factor VII (FVII) deficiency is a rare autosomal recessive hemorrhagic disorder. Clinical bleeding can vary widely and does not always correlate with the level of FVII coagulant activity measured in plasma. Most severe cases of factor VII (FVII) deficiency are diagnosed during childhood, often during the first 6 months of life. In infancy, the most common sites of bleeding occur in the gastrointestinal tract or CNS, accounting for 60-70% of bleeds in this age group. Recombinant factor VIIa (rFVIIa) is one such agent, which has been shown to prevent hematoma expansion and improve outcome in acute intracranial haemorrhages. The purpose of this case report is to share our experience regarding the usefulness of rFVIIa in the management of acute intracranial haemorrhage.

摘要

遗传性因子 VII(FVII)缺乏症是一种罕见的常染色体隐性出血性疾病。临床出血情况差异很大,并不总是与血浆中测得的 FVII 凝血活性水平相关。大多数严重的因子 VII(FVII)缺乏症病例在儿童期被诊断出来,通常是在出生后的前 6 个月。在婴儿期,最常见的出血部位发生在胃肠道或中枢神经系统,占该年龄组出血的 60 - 70%。重组因子 VIIa(rFVIIa)就是这样一种药物,已被证明可预防血肿扩大并改善急性颅内出血的预后。本病例报告的目的是分享我们关于 rFVIIa 在急性颅内出血管理中的有用性的经验。

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