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1/5. Mesenteric venous thrombosis due to antithrombin iii deficiency.

    A 19-year-old woman developed gradually worsening abdominal pain, signs of peritonitis, and hematemesis. laparotomy revealed peritonitis due to segmental small-bowel infarction, and the underlying pathologic condition was mesenteric vein thrombosis. A primary thrombotic disorder was suggested and antithrombin iii deficiency was found. Before anticoagulant therapy could be initiated, she developed hemorrhagic cerebral infarction and died. Her history included three episodes of deep vein thrombosis while taking oral contraceptives. Her father died of spontaneous mesenteric and portal vein thrombosis at age 29 years. This report underlines the importance of careful interpretation of the vascular pathology in cases of intestinal ischemia.
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keywords = cerebral
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2/5. Intestinal Buerger's disease.

    We describe a 50-year-old man who, at 26 years of age, underwent a hemicolectomy on the right side of the abdomen due to infarction of the right colon. At 35 years of age, a stenotic, ischemic segment of distal jejunum was resected. Later he had had intermittent claudication, migratory thrombophlebitis, and recurrent cerebral infarctions. The mesenteric and mural blood vessels of both resected specimens of bowel showed an occlusive process with organized and recent thrombi and marked transmural inflammation. The internal elastic lamina and media in the arteries were preserved and there was no evidence of atheroma or calcification. The histologic findings were consistent with thromboangiitis obliterans. We suggest that the same mechanism may be responsible for intestinal peripheral and cerebrovascular involvement.
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keywords = cerebral
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3/5. venous thrombosis and splenic rupture in paroxysmal nocturnal hemoglobinuria.

    A patient with an 11 year history of paroxysmal nocturnal hemoglobinuria presented with severe abdominal pain. On admission, the hematocrit value was 30 per cent and unchanged from repeated measurements during the previous three years. Abdominal angiography identified extensive thromboses of the splenic and portal venous systems. After initial improvement on heparin therapy, the patient experienced additional abdominal crises. A ruptured and multifragmented spleen was removed at the time of exploratory laparotomy. Postoperatively, after a several days' interval of improvement, the patient experienced additional thrombotic episodes of the abdomen, upper extremities and cerebral cortex. The latter was associated with disabling nerve paralysis. With continuous intravenous heparin plus steroid therapy, the patient's condition improved progressively. Despite the numerous thrombotic episodes during the prolonged hospital course, no hemolytic episodes were observed. This is the first report of documented splenic rupture in a patient with paroxysmal nocturnal hemoglobinuria.
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keywords = cerebral
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4/5. A case of protein c deficiency associated with cerebral infarction and obstruction of deep leg and inferior mesenteric veins.

    Protein C, a vitamin k-dependent protein, is a blood coagulation inhibitor. Its deficiency causes systemic thrombosis. A 31-year-old woman developed cerebral infarction followed by late psychomotor seizures, and thrombosis in the inferior mesenteric vein and bilateral crural veins. Her parents were first cousins. Her mother died of cerebral thrombosis in her 30's. Her elder brother died of suspected purpura fulminans immediately after birth. Her protein C activity and protein C antigen level decreased markedly and were less than 5% of those of normal controls and 0.3 microgram/ml, respectively. Her father, a paternal aunt and a maternal uncle also showed a low protein C activity and protein C antigen level. This patient seems to have congenital protein c deficiency which produced thrombosis in the leg veins and the mesenteric vein, probably cerebral infarction.
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ranking = 7
keywords = cerebral
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5/5. fibromuscular dysplasia of the superior mesenteric artery and coexisting cerebral berry aneurysms.

    fibromuscular dysplasia of the systemic artery and cerebral "berry" (saccular) aneurysm are nonatheromatous lesions of uncertain etiology. The coexistence of these vascular disorders in the individual reported here is unusual and may provide a possible explanation of their pathogenesis.
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ranking = 5
keywords = cerebral
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