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

doi:2010.16.2.176

Korean J Hepatol > Volume 16(2); 2010 > Article

The Korean Journal of Hepatology 2010;16(2): 176-181.
doi: https://doi.org/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, M.D.1, Do Young Kim, M.D.1, Minju Kim, M.D.2, Young Eun Chon, M.D.1, Hyun Jung Lee, M.D.1, Young-Nyun Park, M.D.2, Jun Yong Park, M.D.1, Sang Hoon Ahn, M.D.1, Kwang-Hyub Han, M.D.1, Chae Yoon Chon, M.D.1

Department of Internal Medicine1 and Pathology2, Yonsei University College of Medicine, Seoul, Korea

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.

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