Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case report of a young female patient with personal history of primary thrombocythaemia, treated with interferon alpha, admitted to our medical department for severe abdominal pain, hepatomegaly, ascites and alteration of hepatic function is presented. Magnetic resonance imaging showed the picture typical for Budd-Chiari syndrome caused by external obstruction of the intrahepatal portion of inferior vena cava. The cause of the syndrome remains uncertain, possibility of the haematogenic infiltration of the liver or venal thrombosis within primary or secondary (interferon-induced) antiphospholipid syndrome is discussed. Liver biopsy could elucidate the exact cause, but it was not performed for technical problems.
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PMID:[The Budd-Chiari syndrome in a patient with primary thrombocythemia treated with interferon alfa and transjugular portosystemic shunt]. 1513 42

Budd-Chiari Syndrome (BCS) refers to hepatic venous outflow obstruction, resulting in simultaneous occurrence of hepatic congestion and portal hypertension, leading to a typical clinical triad consisting of right upper quadrant pain, hepatomegaly and ascites. Contrary to Asia and Africa, where BCS is caused primarily by an obstructing membranous web, BCS in the western world is considered a thrombotic complication of an underlying hypercoagulable state. Recognition of the contribution of hypercoagulability as a causative factor in BCS, has led to acknowledgement of the importance of anti-coagulant therapy in BCS. Indeed, a conservative approach consisting of diuretics and anti-coagulant therapy is considered an appropriate treatment strategy for the BCS patient, in the absence of significant hepatic insult. However, once disease progression is noted, based on clinical symptoms, hepatic laboratory disturbance or histological evidence of irreversible hepatic damage, a definite invasive treatment should be applied. The specific procedure to be used is dependent upon the extent of hepatic insult and the anatomical characteristics of the venous obstruction in any individual patient. In the absence of significant hepatic damage, one may employ surgical shunting or invasive roentgenic measures, such as TransJugular Intrahepatic Porto-Systemic Shunt procedure, for the decompression of the portal system. Alternatively, in cases of a single localized obstruction, one may use balloon angioplasty with stent insertion. In contrast, upon evidence of significant hepatic damage, liver transplantation becomes necessary. To date, numerous studies report excellent results regarding the success of liver transplantation for patients with advanced BCS disease accompanied by significant hepatic damage.
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PMID:[Budd-Chiari syndrome: updated treatment modalities]. 1519 Aug 52

We report the case of a 41-year-old woman, affected by Vaquez syndrome, admitted to our hospital for a severe pain in the right hypochondrium, suddenly followed by hepatomegaly and ascites. The clinical and laboratory data were suggestive of hepatic insufficiency and abdominal ultrasonography, integrated by color Doppler and computed tomography, revealed an interrupted hepatic venous outflow. In addition a spontaneous prolonged partial thromboplastin time was present and the patient was found to be positive for lupus anticoagulant. A transient clinical improvement, with a partial reperfusion of suprahepatic veins, was achieved with medical treatment by using anticoagulants, diuretics and paracentesis. However, the patient showed a subsequence of suprahepatic venous thrombosis, although two transjugular intrahepatic portosystemic shunts with stent placement and local thrombolysis were performed. The polycythemia vera is a disease mainly associated with Budd-Chiari syndrome but, in our patient, the thrombotic event occurred in spite of normal values of hematocrit and platelet count. Certainly in this case the lupus anticoagulant positivity represents an additional thrombogenic factor. Nowadays the antiphospholipid antibody syndrome is a recognized and not unusual cause of Budd-Chiari syndrome but, to our knowledge, this is the first case characterized by the presence of polycythemia vera and antiphospholipid antibody syndrome to be reported.
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PMID:[Budd-Chiari syndrome with fatal outcome in a patient with polycythemia vera and antiphospholipid antibody syndrome]. 1552 39

Budd-Chiari Syndrome is one of the several causes of portal hypertension and is characterised by hepatomegaly, ascites and abdominal pain. The treatment requires either medical or interventional/surgical procedures. A case of Budd-Chiari Syndrome with a membranous web that causes obstruction in the ostium of the inferior right hepatic vein is reported here which was treated by percutaneous transluminal angioplasty (PTA). The patient was asymptomatic and off medication and there was no recurrence after 18-months follow-up.
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PMID:Percutaneous transluminal angioplasty of the inferior right hepatic vein for the treatment of Budd-Chiari syndrome. 1558 42

Budd-Chiari syndrome is a rare condition resulting from outflow obstruction of the liver. This syndrome due to a pyogenic abscess is rarely documented in the English literature. Here a male patient with acute Budd Chiari syndrome is presented. A 21-year-old male patient was admitted to the hospital because of severe right upper quadrant pain, jaundice, hepatomegaly and fever. The examination of liver by computerized tomography and ultrasound revealed a large lesion 120 x l00 mm in size located in the right lobe of liver, which was compressing the inferior vena cava, the right and middle hepatic veins. Twenty-three days after percutaneous catheter drainage and medical treatment, the patient was discharged with complete healing. Although many disorders including malignant diseases can cause Budd-Chiari Syndrome, a pyogenic liver abscess compressing the inferior vena cava, and hepatic veins leading to acute Budd-Chiari syndrome has been rarely reported in English medical literature. Patients presenting with abdominal pain, hepatomegaly, and ascites should be carefully evaluated from this point of view.
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PMID:Acute Budd-Chiari syndrome resulting from a pyogenic liver abscess. 1620 Nov 17

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. Patients with hepatic vein thrombosis (Budd-Chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. Prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.
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PMID:Catheter-directed thrombolysis and thrombectomy for the Budd-Chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients. 1651 88

The thrombophilia which can be either congenital or acquired in adult life has major implications in the abdominal vessels. The resulting portal vein thrombosis, Budd-Chiari syndrome and mesenteric vein thrombosis have a variety of consequences ranging from acute abdomen to chronic hepatomegaly and even totally asymptomatic patient in whom the only finding is pancytopenia. The complications like esophageal varices, portal gastropathy, ascites, severe hypersplenism, liver failure requiring liver transplantation are well known. Interesting features of collateral venous circulation showing itself as pseudocholangiocarcinoma sign and its possible clinical reflection as cholestasis are also known from a long time. The management strategies for these complications of intraabdominal vessel thrombosis are not different from their counterpart which is cirrhotic portal hypertension, but the prognosis is unquestionably better in former cases. In this review we presented and discussed the abdominal venous thrombosis, etiology and the resulting clinical pictures. There are controversial issues both in nomenclature, and management including anticoagulation problems and follow up strategies. In light of the current knowledge, we discussed some controversial issues in literature and presented our experience and our proposals about this group of patients.
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PMID:Etiology and consequences of thrombosis in abdominal vessels. 1653 66

Budd-Chiari syndrome is a spectrum of disease states, including anatomic abnormalities and hypercoagulable disorders, resulting in hepatic venous outflow occlusion. Clinical manifestations observed in the majority of patients include hepatomegaly, right upper quadrant pain, and abdominal ascites. This article outlines the approach to clinical diagnosis and supportive medical therapy in patients who have BCS and reviews the clinical data supporting surgical shunting and liver transplantation as viable treatment options in this patient population.
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PMID:Budd-Chiari syndrome. 1697 Dec 61

The etiology and pathology of Budd-Chiari syndrome (BCS) remain unclear. The membrane in some membranous BCS may be derived from the absorption and organization of the thrombus of inferior vena cava (IVC). The long-term efficacies of currently available graft shunt operations are unsatisfactory. Interventional therapy or radical resection of lesion should be recommended. The IVC stenosis actually results from the compression of hepatomegaly and should not be classified as BCS. The membranous BCS is an acquired disease.
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PMID:[Diagnosis and treatment of Budd-Chiari syndrome]. 1738 Jun 60

Afibrinogenemia is a rare hereditary coagulation disorder characterized by a propensity toward bleeding. A 21-year-old Hispanic woman with afibrinogenemia developed ascites, a distended abdomen, an enlarged liver, scleral icterus, and umbilical vein and abdominal wall vein distension. Computed tomography (CT) scan showed occlusion of the hepatic veins and the infrahepatic vena cava consistent with Budd-Chiari syndrome. These developments led to her evaluation for, and placement on, the liver transplantation waiting list. The patient's unmeasurable international normalized ratio (INR) artifactually increased her Model for End-Stage Liver Disease (MELD) score to 40. Immediately before transplant surgery, an initial thrombelastogram (TEG) showed a flat line, indicating a complete lack of fibrin clot formation. Preoperatively, 20 units of cryoprecipitate were infused. The first intraoperative TEG demonstrated a tracing consistent with normal clot formation. Coagulation studies normalized with the newly functioning liver. The liver transplantation appears to have corrected the fibrinogen deficiency, presumably limiting the chance of recurrent Budd-Chiari syndrome. This case report discusses the essential role of fibrinogen in the coagulation cascade, as visualized by thrombelastography, and exposes the interplay between plasma fibrinogen and thrombin levels, which determine coagulation or fibrinolysis.
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PMID:Perioperative coagulation management in a patient with afibrinogenemia undergoing liver transplantation. 1745 67


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