门静脉血栓形成伴食管静脉曲张破裂作为遗传性蛋白C缺乏症的首发表现
Portal vein thrombosis with ruptured oesophageal varices as presenting manifestation of hereditary protein C deficiency.
作者信息
Valla D, Denninger M H, Delvigne J M, Rueff B, Benhamou J P
机构信息
Service d'Hépatologie, Hôpital Beaujon, Clichy, France.
出版信息
Gut. 1988 Jun;29(6):856-9. doi: 10.1136/gut.29.6.856.
Abstract
The protein C system is essential in limiting the activation of coagulation in vivo. We report the case of a 45 year old man with portal vein thrombosis complicated by ruptured oesophageal varices. Low concentration of plasma protein C was found in the patient and subsequently in one brother with a history of venous thromboembolism, and also in one son and one nephew who were asymptomatic. Hereditary protein C deficiency should be considered in patients with portal hypertension due to portal vein thrombosis.
摘要
蛋白C系统在限制体内凝血激活方面至关重要。我们报告了一例45岁男性患者，患有门静脉血栓形成并伴有食管静脉曲张破裂。在该患者中发现血浆蛋白C浓度较低，随后在一名有静脉血栓栓塞病史的兄弟以及一名无症状的儿子和一名侄子中也发现了这种情况。对于因门静脉血栓形成导致门静脉高压的患者，应考虑遗传性蛋白C缺乏症。
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Portal vein thrombosis with ruptured oesophageal varices as presenting manifestation of hereditary protein C deficiency.门静脉血栓形成伴食管静脉曲张破裂作为遗传性蛋白C缺乏症的首发表现
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