Cases reported "Hypertension, Portal"

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1/93. Emergency and elective surgical treatment of portal hypertension. A review of 23 years' experience.

    A retrospective review of surgical treatment for portal hypertension during a 23-year period in a regional unit is reported and the immediate and subsequent management of patients with bleeding oesophageal varices is discussed. Fifty-four patients with recurrent varix haemorrhage uncontrolled by conservative methods have been treated by oesophageal transection with a mortality of 22.2% (26.6% for cirrhotic patients). Thirty-two per cent of the cirrhotics were alive after 2 years. Only a minority (12%) of the survivors were considered suitable for a subsequent shunt procedure. Therapeutic portacaval anastomosis has been performed on 65 patients with a 51.2% 5-year survival (43-5% for cirrhotic patients). Further haemorrhage due to shunt thrombosis occurred in 5-3% of cases. The frequent occurrence of portal-systemic encephalopathy, increasing with duration of time following a shunt, is emphasized. The high morbidity and mortality in the poor-risk cirrhotic indicated that this type of patient is unsuitable for a portal-systemic shunt and is better treated by medical means.
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ranking = 1
keywords = thrombosis
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2/93. Chemotherapy for hepatocellular carcinoma with portal hypertension due to tumor thrombus.

    A case of hepatocellular carcinoma (HCC) complicated by tumor thrombosis of the main trunk is presented. Four courses of hepatic arterial infusion therapy, via a subcutaneously implanted injection port, were performed using cisplatin (10 mg for 1 hour on days 1-5) and 5-fluorouracil (250 mg for 5 hours on days 1-5). After four courses of the chemotherapy, marked reduction in size of HCC and the tumor markers were noted. The esophageal varices and ascites were improved after the chemotherapy with a recanalization of the left branch of the portal vein. The patient was doing well with a survival period of 28 months after the chemotherapy. These encouraging results suggested that the present therapy, based on the biochemical modulation, was a useful option for advanced HCC with portal hypertension due to tumor thrombosis of the main portal vein.
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ranking = 2
keywords = thrombosis
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3/93. Disappearance of "pseudocholangiocarcinoma sign" in a patient with portal hypertension due to complete thrombosis of left portal vein and main portal vein web after web dilatation and transjugular intrahepatic portosystemic shunt.

    The main portal vein web is probably a consequence of portal vein thrombosis, which is a very rare cause of portal hypertension. Principal manifestations are related to the degree of portal hypertension. In the literature, no data has been found for the treatment modality of portal vein web. We report, herein, the clinical and laboratory findings of a 38-year-old woman with angiographically proven incomplete main portal vein web and complete thrombotic occlusion of the left portal vein causing pseudocholangiocarcinoma sign (PCCS) on the common bile duct. She was treated by transjugular intrahepatic portosystemic shunt (TIPS) and membrane dilatation, which resulted in complete disappearance of collaterals and PCCS. It appears that TIPS and balloon dilatation of the portal vein web via transjugular approach was effective in decreasing portal pressure and its consequences.
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ranking = 5
keywords = thrombosis
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4/93. Laparoscopic cholecystectomy in patient with portal cavernoma and portal hypertension.

    Successful laparoscopic cholecystectomy has been reported in patients with cirrhosis of liver with portal hypertension; the procedure has, however, not been reported in patients with portal vein thrombosis, portal cavernoma and portal hypertension. We report an 18-year-old man with portal hypertension due to portal vein thrombosis and portal cavernoma who had symptomatic gallstone disease and was successfully treated with laparoscopic cholecystectomy.
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ranking = 2
keywords = thrombosis
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5/93. antiphospholipid syndrome presenting as portopulmonary hypertension.

    The association of pulmonary hypertension with portal hypertension, also called portopulmonary hypertension, is a well-described condition. The pathogenesis of this association remains unclear. We describe a 34-year-old female patient with "primary antiphospholipid syndrome" and portopulmonary hypertension. Our finding supports that in situ microthrombosis associated with the presence of anticardiolipin antibodies could be the pathophysiologic explanation for both portal and pulmonary hypertension.
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ranking = 1
keywords = thrombosis
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6/93. Gamna-Gandy bodies of the spleen detected with MR imaging: a case report.

    We report a case of a female patient with portal hypertension due to liver cirrhosis. In this case, MR imaging revealed small siderotic nodules of the spleen, called Gamna-Gandy bodies. These lesions are found in patients with portal vein or splenic vein thrombosis, hemolytic anemia, leukemia, or lymphoma, patients receiving blood transfusions, acquired hemochromatosis, or paroxysmal nocturnal hemoglobinuria. There are only few reports in the literature about these siderotic nodules which are not very familiar. MR imaging seems to be the superior imaging method for detection of these lesions. It is important to consider Gamna-Gandy bodies in the differential diagnosis of portal hypertension and the other diseases mentioned above.
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ranking = 1
keywords = thrombosis
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7/93. splenectomy in a case of splenic vein thrombosis unmasks essential thrombocythemia.

    We report a patient with splenic vein thrombosis (SVT) in whom splenectomy resulted in the unmasking of essential thrombocythemia (ET). He had portal hypertension with haematemesis, resulting in anaemia requiring repeated blood transfusions. Investigations revealed SVT. Following splenectomy, he suffered a transient ischaemic attack episode, associated with persistent thrombocytosis (> 2000 x 10(9)/l). Other myeloproliferative disorders were excluded and a diagnosis of ET was established. He responded to hydroxyurea but, due to financial constraints, he discontinued treatment and subsequently relapsed. The association of ET with SVT is rare and the diagnosis of ET was missed initially as the platelet count was normal prior to splenectomy.
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ranking = 5
keywords = thrombosis
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8/93. association of Dubin-Johnson syndrome and portal vein thrombosis.

    Dubin-Johnson syndrome is neither complicated by liver cell necrosis nor associated with portal hypertension. We report a 22-year-old man who had recurrent episodes of jaundice (conjugated hyperbilirubinemia) because of Dubin-Johnson syndrome and portal hypertension secondary to portal vein thrombosis. The relationship between Dubin-Johnson syndrome and portal vein thrombosis in this case is most likely a chance occurrence.
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ranking = 6
keywords = thrombosis
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9/93. Cure of gastric antral vascular ectasia by liver transplantation despite persistent portal hypertension: a clue for pathogenesis.

    gastric antral vascular ectasia (GAVE) is a rare cause of chronic bleeding in cirrhotic patients. It has been suggested that these gastric lesions might be related to portal hypertension, hepatic insufficiency, or both parameters. We report two cases of cirrhotic patients in whom GAVE was the source of recurrent bleeding. These patients also had complete portal vein thrombosis. liver transplantation was performed and an end-to-end cavoportal anastomosis was performed, leaving patients with persistent portal hypertension after surgery. We observed complete disappearance of the antral lesions several weeks after transplantation, which shows that the GAVE is not related to portal hypertension but is rather a direct consequence of liver failure. Possible pathophysiologic mechanisms are discussed.
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ranking = 1
keywords = thrombosis
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10/93. Multivisceral transplantation for portal hypertension and diffuse mesenteric thrombosis caused by protein c deficiency.

    BACKGROUND: Protein C is a vitamin k-dependent glycoprotein synthesized in the liver. Homozygous deficiency usually manifests as purpura fulminans in infancy and is often fatal. An unlikely, but potentially life-threatening, manifestation of protein c deficiency is mesenteric venous thrombosis. methods: A patient with undiagnosed familial protein c deficiency and a history of intestinal infarction developed refractory duodenal and jejunal variceal bleeding as a result of diffuse visceral splanchnic thrombosis and portal hypertension. Because his life-threatening bleeding was unresponsive to all therapies, we performed multivisceral transplantation. RESULTS: Multivisceral transplantation cured the patient's underlying disease and was lifesaving. Functional protein C, undetectable before transplantation, was normal after transplantation. He was discharged tolerating a normal diet and is alive at home 6 months later. CONCLUSIONS: Multivisceral transplantation should be considered as a treatment option for patients with diffuse mesenteric thrombosis, even in the absence of short gut syndrome, when portal hypertension causes life-threatening complications unresponsive to less aggressive therapies.
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ranking = 7
keywords = thrombosis
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