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[遗传性蛋白C缺乏症与门静脉血栓形成]

[Hereditary protein C deficiency and portal vein thrombosis].

作者信息

dos Santos J N, Mello e Silva A, Alexandrino P, Saldanha T, Carreira M O, Ferreira E, de Pádua F

机构信息

Serviço de Medicina 4, Hospital de Santa Maria, Faculdade de Medicina de Lisboa.

出版信息

Acta Med Port. 1990 Jan-Feb;3(1):27-30.

PMID:2333774
Abstract

Inherited defects of the natural coagulation inhibitors predispose patients to thrombosis. These disorders have similar clinical presentations with a strong family of thrombosis, episodes of recurrent venous thromboembolism, beginning in early adulthood. We report a case of upper gastrointestinal bleeding in a patient with portal hypertension due to portal-vein thrombosis secondary to hereditary protein C deficiency, an association that has seldom been reported. We conclude that protein C deficiency should be investigated in thrombotic states, namely after more frequent causes have been excluded.

摘要

天然凝血抑制剂的遗传性缺陷使患者易患血栓形成。这些疾病具有相似的临床表现,有强烈的血栓形成家族史,复发性静脉血栓栓塞发作始于成年早期。我们报告了一例因遗传性蛋白C缺乏继发门静脉血栓形成导致门静脉高压的患者出现上消化道出血的病例,这种关联很少有报道。我们得出结论,在血栓形成状态下,即在排除更常见的原因后,应调查蛋白C缺乏情况。

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