Case report: portal vein thrombosis associated with hereditary protein C deficiency: a report of two cases.
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.
Yang
Y Y
YY
Department of Medicine, Veterans General Hospital-Taipei, Taiwan, Republic of China.
Chan
C C
CC
Wang
S S
SS
Chiu
C F
CF
Hsu
H C
HC
Chiang
J H
JH
Tasy
S H
SH
Chang
F Y
FY
Lee
S D
SD
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*