Case report: portal vein thrombosis associated with hereditary protein C deficiency: a report of two cases |
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Authors: | Yang Y Y Chan C C Wang S S Chiu C F Hsu H C Chiang J H Tasy S H Chang F Y Lee S D |
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Institution: | Department of Medicine, Veterans General Hospital-Taipei, Taiwan, Republic of China. |
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Abstract: | Protein C deficiency is one of the causes of curable or preventable portal vein thrombosis. We report two patients of portal vein thrombosis associated with hereditary protein C deficiency. The first patient presented with continuous right upper quadrant pain and high fever. The abdominal sonography revealed normal liver parenchyma but portal vein and superior mesenteric vein thrombosis. Based on a 55% (normal 70-140%) plasma protein C level, he was diagnosed as having protein C deficiency. A trace of his family history showed that his elder brother also had protein C deficiency with a 50% plasma C level. Both patients received anticoagulant therapy. The younger brother showed good response. Unfortunately, the elder one suffered from recurrent episodes of variceal bleeding and received a life-saving splenectomy and devascularization. We herein remind clinicians that early screening and therapy are helpful in preventing late complications of protein C deficiency with portal vein thrombosis. |
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Keywords: | portal vein protein C deficiency thrombosis |
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