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: Y Y Yang, C C Chan, S S Wang, C F Chiu, H C Hsu, J H Chiang, S H Tasy, F Y Chang, S D Lee
• Journal: Journal of gastroenterology and hepatology (J Gastroenterol Hepatol) Vol. 14 Issue 11 Pg. 1119-23 (Nov 1999) ISSN: 0815-9319 [Print] Australia
• PMID: 10574141 (Publication Type: Case Reports, Journal Article)

Topics

• Abdomen, Acute (etiology)
• Adult
• Diagnosis, Differential
• Diagnostic Imaging
• Humans
• Male
• Pedigree
• Portal Vein
• Protein C Deficiency (diagnosis, genetics)
• Thrombosis (diagnosis, genetics)