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)

Budd-Chiari syndrome (BCS) is the liver disease resulting from hepatic venous outflow obstruction comprising a triad of abdominal discomfort, hepatomegaly and ascites. Advances in the management of this disorder over the last three decades have dramatically improved survival. We present a review of the management of BCS followed by a case which illustrates some key points in the diagnosis and treatment of this condition.
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PMID:Budd-Chiari syndrome--a review of the diagnosis and management. 2157 56

Budd-Chiari syndrome (BCS) is a rare disorder characterized by hepatic venous obstruction. A 41-year-old male patient presented with right upper quadrant pain, abdominal distension, and dyspnea. He had a history of BCS that was associated with polycythemia vera. Abdominal computed tomography showed hepatomegaly and a hypodense filling defect suggestive of thrombus formation in the hepatic, splenic, and portal veins, and suprahepatic part of the inferior vena cava. Transthoracic echocardiography performed to assess the extension of this pathological process and its relation with intracardiac structures showed a mass lesion in the right atrium, about 4 x 3 cm in diameter. The lesion manifested as an intracardiac thrombus extending from the inferior vena cava. The patient underwent surgical treatment to remove the atrial mass. At surgery, the lesion turned out to be an atrial tumor, which was diagnosed as atrial myxoma in histopathologic examination. The symptoms of the patient resolved after surgery.
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PMID:Right atrial myxoma associated with portal and splenic vein thrombosis in a patient with Budd-Chiari syndrome. 2164 35

Budd-Chiari syndrome (BCS) consists of a group of disorders with obstruction of hepatic venous outflow leading to increased hepatic sinusoidal pressure and portal hypertension. Clinically, two forms of disease (acute and chronic) are recognized. Mostly the patients present with ascites, hepatomegaly, and portal hypertension. In acute disease the liver is enlarged with thrombosed hepatic veins (HV) and ascites, whereas in the chronic form of the disease there may be membranous occlusion of HV and/or the inferior vena cava (IVC), or there may be short or long segment fibrotic constriction of HV or the suprahepatic IVC. Due to advances in radiological interventional techniques and hardware, there have been changes in the management protocol of BCS with surgery being offered to patients not suitable for radiological interventions or having acute liver failure requiring liver transplantation. The present article gives an insight into various imaging findings and interventional techniques employed in the management of BCS.
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PMID:Imaging and interventions in Budd-Chiari syndrome. 2186 Jul 12

Abdominal venous thrombosis may present as splanchnic venous thrombosis (SVT) (occlusion of portal, splenic, superior or inferior mesenteric veins) or Budd- Chiari Syndrome (BCS) (thrombosis of inferior vena cava and/or hepatic veins). The aim of this review is to report the scanty data available for SVT in the South Mediterranean area. In one Egyptian study, the possible circumstantial risk factors for portal vein thrombosis (PVT) were found in 30% of cases: 19% neonatal sepsis, 8.7% umbilical catheterization, 6% severe gastroenteritis and dehydration. Another Egyptian study concluded that hereditary thrombophilia was common in children with PVT (62.5%), the commonest being factor V Leiden mutation (FVL) (30%). Concurrence of more than one hereditary thrombophilia was not uncommon (12.5%). The first international publication on hepatic veno-occlusive disease (VOD) in Egypt was in 1965 in children who rapidly develop abdominal distention with ascites and hepatomegaly. This disease was more frequent in malnourished children coming from rural areas; infusions given at home may contain noxious substances that were hepatotoxic and infections might play a role. VOD of childhood is rarely seen nowadays. Data from South Mediterranean area are deficient and this may be attributable to reporting in local medical journals that are difficult to access. Medical societies concerned with this topic could help distribute this information.
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PMID:Splanchnic vein thrombosis in the mediterranean area in children. 2186 13

Budd-Chiari syndrome (BCS) is rare in infancy. Three cases are presented. Case 1, an 8-month-old boy, presented with abdominal distension and oliguria. Doppler study of the abdomen showed ascites, hepatomegaly and normal hepatic veins. However, a CT scan demonstrated hepatic vein thrombosis. Case 2, a 5-month-old boy, presented with abdominal distension and diarrhoea. Ultrasound of the abdomen showed hepatic vein thrombosis and hepatomegaly. Case 3, a 7-month-old girl, presented with abdominal distension, diarrhoea and oliguria. Ultrasound of the abdomen showed hepatomegaly and obstructed hepatic veins. None of the cases had fever or jaundice before presentation. Case 1 developed fungal septicaemia and was lost to follow-up. Cases 2 and 3 succumbed to the disease before further intervention.
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PMID:Budd-Chiari disease in infancy: three cases. 2259 16

Budd-Chiari syndrome (BCS) is a rare disease defined by congestive hepatopathy with obstruction of the hepatic venous outflow tract. Classical symptoms and signs include ascites, hepatomegaly, abdominal pain and various degrees of liver dysfunction. BCS is predominantly caused by thrombosis, malformations and venous compression. We present a case, in which BCS was the cause of liver cirrhosis complicated with refractory ascites and which can be misinterpreted as hepatocellular carcinoma nodules. The diagnosis was confirmed during the transjugular intrahepatic portosystemic shunt procedure with successful vascular stenting resolving the ascites formation.
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PMID:[Chronic Budd-Chiari syndrome can cause liver cirrhosis]. 2268 96

Budd Chiari syndrome or hepatic venous outflow obstruction is a complex entity with multiple etiologies and various clinical manifestations. It is often difficult to establish the diagnosis. The most common cause is a hypercoagulable state due to either genetic disorders of blood coagulation or several acquired conditions such as hematological diseases, tumors, infections, chronic inflammatory diseases, pregnancy. The most common clinical presentation is hepatomegaly, abdominal pain and ascites, but the onset can also be dramatical and life threatening with upper digestive bleeding due to portal hypertension through postsinusoidal blockage. We report the case of a young patient with a coagulation disorder secondary to a mutation of factor V Leiden, who presented with upper digestive bleeding as the first manifestation of Budd Chiari syndrome and who also was associated with myocardial infarction in his past medical history.
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PMID:Upper gastrointestinal bleeding in a young patient with Budd Chiari syndrome due to a mutation of factor V Leiden: a case report. 2379 22

Diffuse liver disease, including all causes of chronic liver disease, affects tens of millions of people worldwide. There is a growing need for diagnostic evaluation as treatments become more readily available, particularly for viral liver disease. Magnetic resonance imaging (MRI) provides unique capabilities for noninvasive characterization of liver tissue that rival or surpass the diagnostic utility of liver biopsies. There has been incremental improvement in the use of standardized MRI sequences, acquired before and after administration of contrast for the evaluation of diffuse liver disease, and this includes study of the liver parenchyma and blood supply. More recent developments have led to methods for quantifying important liver metabolites, including fat and iron, and liver fibrosis, which is the hallmark for chronic liver disease. In this study, we review the MRI techniques and diagnostic features associated with common and uncommon etiologies of diffuse liver diseases, including processes that lead to abnormal perfusion (e.g. Budd-Chiari syndrome, congestive hepatomegaly), deposition diseases (e.g. fatty liver, hemochromatosis, Wilson's disease), and abnormalities that are related to inflammation and fibrosis (e.g. primary sclerosing cholangitis, sarcoidosis).
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PMID:MRI of diffuse liver disease: the common and uncommon etiologies. 2392 Dec 68

In this report, we describe a case of pyrrolizidine alkaloid-related Budd-Chiari syndrome in Hong Kong. A 10-month-old boy presented with ascites, right pleural effusion, and hepatomegaly after consumption of herbal drinks for 3 months. His clinical (including imaging) features were compatible with Budd-Chiari syndrome. Budd-Chiari syndrome is a rare disease entity in paediatric patients. In our case, extensive workup performed to look for the underlying cause of Budd-Chiari syndrome was unrevealing, except for toxic pyrrolizidine alkaloid exposure in his herbal drinks.
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PMID:Budd-Chiari syndrome secondary to toxic pyrrolizidine alkaloid exposure. 2431 Jun 65

Budd-Chiari syndrome refers to hepatic pathology secondary to diminished venous outflow, most commonly associated with venothrombotic disease. Clinically, patients with Budd-Chiari present with hepatomegaly, ascites, abdominal distension, and pain. On imaging, Budd-Chiari syndrome is hallmarked by occluded IVC and or hepatic veins, caudate lobe enlargement, heterogeneous liver enhancement, intrahepatic collaterals, and hypervascular nodules. Etiopathological factors for Budd-Chiari syndrome include several systemic thrombotic and nonthrombotic conditions that can cause venous outflow obstruction at hepatic veins and/or IVC. While the transjugular intrahepatic portosystemic shunt (TIPS) is used as a treatment option for Budd-Chiari syndrome, Budd-Chiari syndrome is not a well-known complication of TIPS procedure. We report a case of Budd-Chiari syndrome that occurred in a transplanted cirrhotic liver from malpositioned proximal portion of the TIPS in IVC causing occlusion of the ostia of hepatic veins which was subsequently diagnosed on contrast-enhanced CT.
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PMID:Budd-Chiari Syndrome Caused by TIPS Malposition: A Case Report. 2482 68


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