Portal vein thrombosis with ruptured oesophageal varices as presenting manifestation of hereditary protein C deficiency.
The protein C system is essential in limiting the activation of coagulation in vivo. We report the case of a 45 year old man with portal vein thrombosis complicated by ruptured oesophageal varices. Low concentration of plasma protein C was found in the patient and subsequently in one brother with a history of venous thromboembolism, and also in one son and one nephew who were asymptomatic. Hereditary protein C deficiency should be considered in patients with portal hypertension due to portal vein thrombosis.
Valla
D
D
Service d'Hépatologie, Hôpital Beaujon, Clichy, France.
Denninger
M H
MH
Delvigne
J M
JM
Rueff
B
B
Benhamou
J P
JP
D004932:Esophageal and Gastric Varices / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011169:Portal Vein; D020151:Protein C Deficiency; D012422:Rupture, Spontaneous; D013927:Thrombosis / Q000209:etiology*