Portal vein thrombosis (PVT) is an uncommon cause of presinusoidal portal hypertension. Among various hepatoportal disorders, noncirrhotic portal hypertension conditions such as idiopathic portal hypertension (IPH) are considered to have a close relation with PVT. PVT is known to have several predisposing conditions, including infection, malignancies, and coagulation disorders. There is growing interest and recognition that deficiencies in proteins C and S are associated with a hypercoagulable state. These deficiencies are regarded as key factors of systemic hypercoagulability and recurrent venous thromboembolism. We report the case of a 19-year-old male diagnosed as IPH with PVT and combined deficiencies in proteins C and S.
Humans ; Hypertension, Portal/complications/*diagnosis/pathology ; Male ; *Portal Vein ; Protein C Deficiency/*complications ; Protein S Deficiency/*complications ; Tomography, X-Ray Computed ; Venous Thrombosis/complications/*diagnosis/pathology ; Young Adult

Protein C is an important physiological anticoagulant factor. Protein C deficiency has been linked to venous thrombosis at unusual sites, including the cerebral and mesenteric veins. Hereditary protein C deficiency is inherited primarily as an autosomal dominant trait with incomplete penetrance. Protein C and S deficiencies are known to increase the risk of venous thrombosis and pulmonary thromboembolism. Testing for protein C levels and function is necessary for the detection of both type I and type II protein C deficiency. In this article, we report a case of pulmonary embolism and mesentery ischemia due to type 1 protein C deficiency.
Colonoscopy ; Humans ; Ischemia/*diagnosis/etiology ; Magnetic Resonance Angiography ; Male ; Mesenteric Veins ; Middle Aged ; Protein C Deficiency/*complications/genetics ; Pulmonary Embolism/*diagnosis/etiology/radiography ; Tomography, X-Ray Computed

Mesenteric venous thrombosis is a clinically very rare disease, and may cause bowel infarction and gangrene. Difficulty in the dignosis the disease due to its non-specific symptoms and low prevalence can cause a clinically fatal situation. Mesenteric venous thrombosis may be caused by both congenital and acquired factors, and protein C deficiency, which is a very rare genetic disorder, is one of many causes of mesenteric thrombosis. The authors experienced a case of mesenteric venous thrombosis caused by protein C deficiency in a patient with duodenal ulcer bleeding, so here we report a case together with literature review.
Duodenal Ulcer/*complications/diagnosis ; Endoscopy, Gastrointestinal ; Humans ; Male ; *Mesenteric Veins ; Middle Aged ; Peptic Ulcer Hemorrhage/*complications ; Protein C Deficiency/*complications/diagnosis ; Tomography, X-Ray Computed ; Venous Thrombosis/*diagnosis/etiology/ultrasonography