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X 因子缺乏:青春期月经过多的罕见病因。

Factor X deficiency: a rare cause of puberty menorrhagia.

机构信息

Department of Pediatrics, SMGS Hospital, Shalamar, Jammu, India.

出版信息

Indian J Pediatr. 2013 Jul;80(7):607-8. doi: 10.1007/s12098-012-0796-7. Epub 2012 Jun 14.

DOI:10.1007/s12098-012-0796-7
PMID:22696092
Abstract

Factor X deficiency is an extremely rare coagulation defect inherited as an autosomal recessive disorder with variable bleeding manifestations. The authors report case of a 16 y-old girl born from a consanguineous marriage who presented with excessive bleeding at the start of menarche. Investigations revealed severe anemia, prolongation of both prothrombin time and activated partial thromboplastin time and moderate deficiency of factor X (1 %). She was given multiple transfusions including packed cells and fresh frozen plasma and was advised to remain under regular follow up.

摘要

X 因子缺乏症是一种极为罕见的遗传性凝血缺陷疾病,呈常染色体隐性遗传,具有不同的出血表现。作者报告了一例 16 岁女孩的病例,她出生于近亲婚姻,初潮时出现过度出血。检查发现严重贫血,凝血酶原时间和激活部分凝血活酶时间延长,X 因子中度缺乏(1%)。她接受了多次输血,包括浓缩红细胞和新鲜冷冻血浆,并被建议定期随访。

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Factor X deficiency: a rare cause of puberty menorrhagia.X 因子缺乏:青春期月经过多的罕见病因。
Indian J Pediatr. 2013 Jul;80(7):607-8. doi: 10.1007/s12098-012-0796-7. Epub 2012 Jun 14.
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Presurgical plasma exchange is ineffective in correcting amyloid associated factor X deficiency.术前血浆置换在纠正淀粉样变相关的因子X缺乏方面无效。
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Successful treatment of transient acquired factor X deficiency by plasmapheresis with concomitant intravenous immunoglobulin and steroid therapy.通过血浆置换联合静脉注射免疫球蛋白和类固醇疗法成功治疗短暂获得性因子X缺乏症。
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J Obstet Gynaecol India. 2016 Oct;66(Suppl 1):400-6. doi: 10.1007/s13224-015-0770-1. Epub 2015 Oct 16.

本文引用的文献

1
Routine preoperative coagulation screening detects a rare bleeding disorder.常规术前凝血筛查检测出一种罕见的出血性疾病。
Anesth Analg. 2009 Jan;108(1):76-8. doi: 10.1213/ane.0b013e3181875e51.
2
Factor X deficiency: clinical manifestation of 102 subjects from Europe and Latin America with mutations in the factor 10 gene.凝血因子X缺乏症:来自欧洲和拉丁美洲的102名凝血因子10基因发生突变的受试者的临床表现
Haemophilia. 2006 Sep;12(5):479-89. doi: 10.1111/j.1365-2516.2006.01303.x.
3
Polymorphisms of the protein Z-dependent protease inhibitor (ZPI) gene and the risk of venous thromboembolism.
蛋白Z依赖性蛋白酶抑制剂(ZPI)基因多态性与静脉血栓栓塞风险
Thromb Haemost. 2006 May;95(5):909-10.
4
Hereditary coagulation factor X deficiency.遗传性凝血因子X缺乏症
Indian Pediatr. 2005 Dec;42(12):1240-2.
5
Factor X deficiency in North Pakistan.巴基斯坦北部的凝血因子X缺乏症。
J Ayub Med Coll Abbottabad. 2004 Jul-Sep;16(3):1-4.
6
Factor X deficiency.凝血因子X缺乏症。
Blood Rev. 2002 Jun;16(2):97-110. doi: 10.1054/blre.2002.0191.
7
Congenital factor X deficiency: spectrum of bleeding symptoms in 32 Iranian patients.先天性因子X缺乏症:32例伊朗患者的出血症状谱
Br J Haematol. 1998 Jul;102(2):626-8. doi: 10.1046/j.1365-2141.1998.00806.x.