Hwang Sena, Kim Do Young, Kim Minju, Chon Young Eun, Lee Hyun Jung, Park Young-Nyun, Park Jun Yong, Ahn Sang Hoon, Han Kwang-Hyub, Chon Chae Yoon
Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Korean J Hepatol. 2010 Jun;16(2):176-81. doi: 10.3350/kjhep.2010.16.2.176.
Portal vein thrombosis (PVT) is an uncommon cause of presinusoidal portal hypertension. Among various hepatoportal disorders, noncirrhotic portal hypertension conditions such as idiopathic portal hypertension (IPH) are considered to have a close relation with PVT. PVT is known to have several predisposing conditions, including infection, malignancies, and coagulation disorders. There is growing interest and recognition that deficiencies in proteins C and S are associated with a hypercoagulable state. These deficiencies are regarded as key factors of systemic hypercoagulability and recurrent venous thromboembolism. We report the case of a 19-year-old male diagnosed as IPH with PVT and combined deficiencies in proteins C and S.
门静脉血栓形成（PVT）是窦性前门静脉高压的一种罕见病因。在各种肝门静脉疾病中，诸如特发性门静脉高压（IPH）等非肝硬化性门静脉高压症被认为与PVT关系密切。已知PVT有多种诱发因素，包括感染、恶性肿瘤和凝血障碍。人们越来越关注并认识到蛋白C和蛋白S缺乏与高凝状态有关。这些缺乏被视为全身性高凝性和复发性静脉血栓栓塞的关键因素。我们报告一例19岁男性病例，该患者被诊断为患有PVT的IPH，同时合并蛋白C和蛋白S缺乏。
