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[Factor X deficiency and pregnancy].

作者信息

Rezig K, Diar N, Benabidallah D, Audibert J

机构信息

Service d'anesthésie-réanimation, CHI André Grégoire, 93150 Montreuil, France.

出版信息

Ann Fr Anesth Reanim. 2002 Jun;21(6):521-4. doi: 10.1016/s0750-7658(02)00646-9.

DOI:10.1016/s0750-7658(02)00646-9
PMID:12134597
Abstract

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.

摘要

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引用本文的文献

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Rare case of combined factor V and factor X deficiency in pregnancy: presenting as secondary postpartum haemorrhage in first pregnancy and successful outcome in second pregnancy.孕期罕见的联合因子V和因子X缺乏病例:首次妊娠时表现为继发性产后出血,第二次妊娠结局良好。
Obstet Med. 2013 Sep;6(3):134-135. doi: 10.1258/om.2012.110094. Epub 2013 May 3.
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Blood product support for delivery in severe factor X deficiency: the use of thrombin generation to guide therapy.严重因子X缺乏症分娩时的血液制品支持:利用凝血酶生成指导治疗。
Blood Transfus. 2007 Nov;5(4):204-9. doi: 10.2450/2007.0023-07.