A Case of Superior Mesenteric Vein Thrombosis Caused by Protein C Deficiency.

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  • プロテインＣ活性低下に伴う上腸間膜静脈血栓症の１例

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Abstract

This paper presents a case of superior mesenteric vein thrombosis caused by protein C deficiency, which is a rare disease.<br> A 27-year-old man, who had been treated with a diagnosis of acute enteritis for 3 days after the onsets of epigastralgia, diarrhea and vomiting, had a gradually intensifying severe right lower abdominal pain. He was referred to the hospital with a suspicion of acute appendicitis. Exploratory laparotomy was undertaken, but no ascites was revealed and the appendix and small intestine were normal. On the next day, the abdominal pain increased and large quantities of bloody ascites were discharged from a penrose drain. Emergency laparotomy was performed again for a diagnosis of acute abdomen due to intestinal infarction. Operative findings revealed congestive necrosis of the small intestine, approximately 130cm in length, due to thrombosis of the superior mesenteric vein. Massive resection of the small intestine, approximately 220cm in length including the necrotic lesion, was carried out, followed by side-to-side anastomosis. Patency of the superior mesenteric artery and occlusion of the superior mesenteric vein with thrombus were observed. Superior mesenteric vein thrombosis was diagnosed. The patient had no remarkable past histories, but a coagulation study showed low level as well as lowered activity of protein C antigen. It is etiologically considered that the mesenteric vein thrombosis might be caused by underlying protein C deficiency.

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