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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Renal vein thrombosis in early infancy is a complication of dehydration and prolonged hypotension. The onset is usually acute and the most common clinical signs are uni- or bilateral frank masses, hematuria, proteinuria and thrombocytopenia. In most cases, with conservative management, the late outcome is favorable. In the adult, renal vein thrombosis is often a silent complication of the nephrotic syndrome, the hypercoagulability of which may be an important factor in the pathogenesis of the thrombosis. Clinically, the presentation of a sudden complete occlusion is that of severe abdominal and lumbar pain with hematuria and loss of function of the kidney that suffers hemorrhagic infarction. Physical examination often reveals an enlarged kidney. With gradual occlusion, renal function is preserved. The initial diagnostic approach is with ultrasound studies and computed tomography; definitive diagnosis is established by renal venography or by selective renal arteriography. In general, a conservative approach including the use of anticoagulant treatment is preferred to surgical intervention. Priapism is a persistent painful penile erection due to ischemic or non-ischemic causes; therapeutic intracavernosal injection of papaverine is becoming the most common cause. In early and mild stages, aspiration of blood from the corpora cavernosa supplemented with intracavernosal irrigation with alpha-stimulating agents is the procedure of first choice; in late and severe ischemia, a shunt procedure may become necessary. Hepatic vein thrombosis occurs in association with a number of conditions considered predisposing factors including the use of oral contraceptives. The clinical picture may be that of an acute illness with abdominal pain, hepatomegaly, ascites and hepatic failure as well as early death. More often, the onset is insidious with slowly developing ascites and wasting. For the diagnosis, hepatic scintigraphy may be helpful but, at present, ultrasonography, computed tomography and magnetic resonance scanning are procedures of choice. There is, as yet, no adequate treatment. A fatal outcome may be prevented by surgical decompression of the congested liver and, in recent years, liver transplantation has been employed. Portal vein thrombosis, in children, is usually considered a complication of umbilical sepsis or a result of a congenital abnormality of the portal vein. In adults, the most frequent causes are hepatic cirrhosis and neoplasia. Clinically, there may be a sudden appearance of ascites with resolution in a symptom-free interval until the onset of other features of portal hypertension occur. Currently, ultrasound real-time imaging supplemented with Doppler capability, computed tomography and magnetic resonance scanning provide the necessary diagnostic information. Variceal hemorrhage is often the first major complication requiring treatment.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Thrombosis in particular organ veins. 268 Aug 53

Budd-Chiari syndrome is an unusual form of portal hypertension caused by occlusion of the hepatic venous outflow, and it is often confused with portal hypertension caused by liver cirrhosis. Its prognosis is poor, and optimal therapy remains to be established. This is a report of 100 confirmed cases of this syndrome treated from December 1982 to March 1988 at two vascular centers in China. Sixty-two male and 38 female patients, 15 to 62 years of age (mean age, 32.6 years) were treated. Seventy-six patients had intractable ascites, 56 had esophageal varices, and 22 had upper gastrointestinal bleeding. There were 37 cases of membranous obstruction, 57 cases of occlusion of the inferior vena cava above the confluence of the hepatic veins, and 6 cases of occlusion of the hepatic veins. Eighty-one patients were operated on. Operative mortality rate was 8.6% (7/81). Follow-up from 2 to 66 months revealed that 58 of the patients operated on (72%) had good results, whereas 11 of 19 (58%) patients treated nonoperatively died within 2 months after admission. On the basis of these data we conclude that the operative procedure must be tailored to the cause and underlying pathologic characteristics. Mesoatrial shunting is the operation of choice for patients with occlusion of the retrohepatic inferior vena cava and hepatic veins, and inferior vena cava--atrial shunting is the operation of choice for patients with occlusion of the inferior vena cava and patency of the hepatic veins. Membranotomy is used for patients with inferior vena cava webbing, and mesocaval shunting is used for patients with intrahepatic venous occlusion only.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Recognition and management of Budd-Chiari syndrome: report of one hundred cases. 276 Sep 94

Of 501 patients with chronic myeloproliferative diseases (c-MPD) 18 developed thrombosis of major abdominal vessels including 6 with hepatic vein thrombosis (Budd-Chiari syndrome). The complication was seen in 14 of 140 (10%) patients with polycythemia vera (PV), 3 of 23 (13%) patients with essential thrombocythemia (ET), 1 of 106 (1%) patients with idiopathic myelofibrosis (IMF), and none of 232 patients with chronic myelogenous leukemia (CML). Leading symptoms and signs were abdominal pain, progressive splenomegaly, widening abdominal girth, ascites, venous collaterals, and nausea and vomiting. The diagnostic modalities with highest specificity were angiography and explorative laparotomy. A causal relationship between the thrombotic event and hematocrit, thrombocyte count, or hemostatic abnormalities at the time of diagnosis could not be established. Detailed laboratory tests of platelet function and coagulation and fibrinolytic parameters of 5 surviving patients did not show any specific defect. Despite medical and surgical intervention, 39% of the patients died within 2 months after diagnosis of the thrombosis. The majority of the survivors developed further complications like liver cirrhosis with portal hypertension and esophageal varices or the short bowel syndrome after extensive bowel resection for mesenterial infarction.
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PMID:Budd-Chiari syndrome and thrombosis of other abdominal vessels in the chronic myeloproliferative diseases. 279 52

Three patients are reported who had liver hydatidosis that was not resolved by conventional surgery and who eventually underwent liver transplantation. In view of the satisfactory results obtained, with 100% postoperative survival, possible indications for liver transplant in this type of patients are discussed, fundamentally for secondary sclerosing cholangitis, secondary biliary cirrhosis and acute Budd-Chiari syndrome.
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PMID:[Role of liver transplant in the treatment of hepatic hydatidosis]. 279 31

Budd-Chiari syndrome secondary to membranous obstruction of the intrahepatic inferior vena cava is a treatable form of chronic liver disease. I report a patient with portal hypertension in whom distortion of the inferior vena cava by cirrhosis and increased intraabdominal pressure initially suggested this condition. The correct diagnosis was made by obtaining lateral views during inferior vena cavography, which demonstrated a tapered, rather than membranous, obstruction, along with normal hepatic venous anatomy and pressure and markedly increased portal vein pressure during transhepatic puncture with a thin needle. That extrinsic deformity of the inferior vena cava may mimic membranous obstruction has not been emphasized recently. This distinction is important as surgical membranotomy is not indicated in patients with cirrhosis and secondary deformity of the inferior vena cava.
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PMID:Pseudo-Budd-Chiari syndrome: extrinsic deformity of the intrahepatic inferior vena cava mimicking membranous obstruction. 292 98

The authors report a rare case of congenital Budd-Chiari syndrome in a twenty-eight-year-old male mongoloid. The patient was submitted to azygous-portal disconnection, because of the syndrome of portal hypertension supposedly due to cirrhosis of the liver. He died of hemorrhage of the liver on the third postoperative day. Autopsy revealed a congenital fibrotic obstruction of all suprahepatic veins, with a wide, round ligament containing a functional umbilical vein, which had been routinely ligated during surgery. An extensive review of the literature showed no similar report. The authors speculate that the inadvertent interruption of the round ligament, which until then had served as a pathway for venous draining of the liver, followed by ligation of the anastomoses between the portal and azygous systems, was the factor that triggered the lethal outcome. Thus, this appears to be the first case of congenital Budd-Chiari syndrome predominantly maintained at the expense of the round ligament of the liver, with a patent vascular branch.
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PMID:An unusual case of Budd-Chiari syndrome--a case report. 296

A rare chronic course of Budd-Chiari syndrome associated with thrombosis of the portal vein was observed in a 30-year-old male patient suffering from postmyocarditic cardiosclerosis. At the age of 24 the patient had infectious allergic myocarditis, was hospitalized and rehospitalized for circulatory insufficiency. Upon 3 years since the disease onset the patient was admitted to a hematological department for progressive enlargement of the spleen. The diagnosis on discharge was idiopathic myelofibrosis with portal hypertension. The treatment included prednisolone, blood transfusions, myelosan. In 1987 the patient presented with enlarged liver and spleen, ascites, gastric and esophageal varicosis, augmenting hepatic insufficiency clinically evaluated as hepatic cirrhosis. Postmortem examination revealed macrofocal cardiosclerosis, splenomegaly, ascites, portal varicosis, enlarged nutmeg liver with smooth surface. Microscopically there was phlebosclerosis and phlebothrombosis varying in duration and involving predominantly medial branches of the hepatic and portal veins, liver fibrosis. The findings provided evidence for the final diagnosis of Budd-Chiari syndrome running an uncommon chronic course.
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PMID:[The chronic form of the Budd-Chiari syndrome]. 297 4

Spontaneous bacterial peritonitis (SBP) is an infectious process that usually occurs in patients with cirrhosis. There are few reports of SBP in patients with other pathologies such as nephrotic syndrome, acute and chronic hepatitis, cardiac ascites, and ascites secondary to neoplastic disease. We report a patient with polycythemia vera in whom recurrent episodes of SBP occurred 8 months following a portacaval shunt operation for Budd-Chiari syndrome. Conceivably, the polycythemia vera (PV) complicated by hepatic vein thrombosis and portacaval shunt resulted in significant loss of hepatic reticuloendothelial system function and predisposed the patient to bacterial peritonitis.
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PMID:Recurrent spontaneous bacterial peritonitis in a patient with polycythemia vera. 305 45

Chronic constrictive pericarditis may complicate both rheumatoid arthritis and systemic lupus erythematosus. To our knowledge, however, this is the first time it has been described in a patient with dermatomyositis. This association should be kept in mind as constrictive pericarditis should be considered in the differential diagnosis of liver cirrhosis and Budd-Chiari syndrome in rheumatic diseases.
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PMID:Constrictive pericarditis complicating dermatomyositis. 320 78

A case of a patient with clinical picture of hepatosplenomegaly, portal hypertension, dilatation of hepatic veins and inferior vena cava, without venous thrombosis or other causes of obstruction of right-sided heart, is described. This picture is compatible with the Budd-Chiari syndrome. Echocardiography has shown a hypertrophic cardiomyopathy causing relevant dilatation of both atria and it has allowed us to exclude the presence of a constrictive pericarditis. The hypertrophic cardiomyopathy is first considered as a cardiac cause of cirrhosis mimicking the Budd-Chiari syndrome.
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PMID:[Hypertrophic cardiomyopathy mimicking clinical picture of Budd-Chiari syndrome]. 321 58


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