Abstract
A 40–year–old man seen in the emergency room for sudden epigastric pain and fever was found in contrast–enhanced abdominal computed tomographey (CT) to have bowel wall thickening and a superior mesenteric vein thrombus. The man felt no peritoneal irritation, so we considered intestinal necrosis unlikely and administered systemic urokinase and heparin. After 1 month of hospitalization, contrast–enhanced CT indicated intestinal necrosis, necessitating emergency surgery. Laparotomy showed that the ileum had formed a mass and that the intestinal tract had necrosed in the small intestine from 60 cm to the anal side of the Treitz′s ligament to the cecum, necessitating massive small–bowel resection and right colectomy. A protein C antigen deficiency and decreased protein C activity postoperatively led to protein C genetic mutation analysis using direct sequencing. A missense mutation was detected causing amino acid substitution of Met for Ile at position 364. The man shows no evidence of recurrence and is currently being treated with oral warfarin and followed up regularly as an outpatient.