title: Case report: portal vein thrombosis associated with hereditary protein C deficiency: a report of two cases.
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.
authors.affiliation: Department of Medicine, Veterans General Hospital-Taipei, Taiwan, Republic of China.
authors.forename: Y Y
authors.initials: YY
authors.lastname: Yang
authors.forename: C C
authors.initials: CC
authors.lastname: Chan
authors.forename: S S
authors.initials: SS
authors.lastname: Wang
authors.forename: C F
authors.initials: CF
authors.lastname: Chiu
authors.forename: H C
authors.initials: HC
authors.lastname: Hsu
authors.forename: J H
authors.initials: JH
authors.lastname: Chiang
authors.forename: S H
authors.initials: SH
authors.lastname: Tasy
authors.forename: F Y
authors.initials: FY
authors.lastname: Chang
authors.forename: S D
authors.initials: SD
authors.lastname: Lee
mesh_terms: D000006:Abdomen, Acute / Q000209:etiology; D000328:Adult; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D006801:Humans; D008297:Male; D010375:Pedigree; D011169:Portal Vein; D020151:Protein C Deficiency / Q000175:diagnosis / Q000235:genetics*; D013927:Thrombosis / Q000175:diagnosis / Q000235:genetics*