The Korean Journal of Hepatology  2010;16(2):176-181

doi:10.3350/kjhep.2010.16.2.176

Deficiencies in proteins C and S in a patient with idiopathic portal hypertension accompanied by portal vein thrombosis.

Sena HWANG 1 ; Do Young KIM ; Minju KIM ; Young Eun CHON ; Hyun Jung LEE ; Young Nyun PARK ; Jun Yong PARK ; Sang Hoon AHN ; Kwang Hyub HAN ; Chae Yoon CHON

Affiliations

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Keywords

Portal hypertension; Thrombosis; Protein C deficiency; Protein S deficiency

Country

Republic of Korea

Language

Korean

Abstract

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.