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

Our goal is to provide a detailed review of veno-occlusive disease (VOD), Budd-Chiari syndrome (BCS), and congestive hepatopathy (CH), all of which results in hepatic venous outflow obstruction. This is the first article in which all three syndromes have been reviewed, enabling the reader to compare the characteristics of these disorders. The histological findings in VOD, BCS, and CH are almost identical: sinusoidal congestion and cell necrosis mostly in perivenular areas of hepatic acini which eventually leads to bridging fibrosis between adjacent central veins. Tender hepatomegaly with jaundice and ascites is common to all three conditions. However, the clinical presentation depends mostly on the extent and rapidity of the outflow obstruction. Although the etiology and treatment are completely different in VOD, BCS, and CH; the similarities in clinical manifestations and liver histology may suggest a common mechanism of hepatic injury and adaptation in response to increased sinusoidal pressure.
 ...
PMID:Hepatic venous outflow obstruction: three similar syndromes. 1746 90

A 68-year-old woman was admitted to our hospital with severe ascites, hepatomegaly and hypereosinophilia. We initially suspected Budd-Chiari Syndrome (BCS), but that was ruled out after confirming the presence of no obstruction in the major veins. A molecular biologic examination proved the clonality of the eosinophils and she was therefore diagnosed as having chronic eosinophilic leukemia (CEL). The pathologic findings of a liver biopsy showed dilation of the sinusoids with infiltration of eosinophils, portal eosinophilic infiltrations with fibrosis, and biliary damage. These findings thus suggested infiltration of the liver by the CEL. A relationship between myeloproliferative disorders and BCS has been commonly reported, however there have so far been very few reports which describe the pathology of CEL liver infiltrates. As a result, the present case in which CEL occurred while demonstrating symptoms and findings similar to BCS is therefore considered to be extremely rare. Further accumulation of such cases should therefore be carried out in the future.
 ...
PMID:[Chronic eosinophilic leukemia with symptoms resembling Budd-Chiari syndrome due to liver infiltration]. 1763

Budd-Chiari syndrome is a rare, heterogenous and potentially lethal condition due to hepatic venous outflow obstruction. Classic triads include hepatomegaly, ascites and abdominal pain. Most cases are caused by abnormal coagulopathy, infection, inflammation or tumor invasion. Status post liver transplantation was rarely mentioned in the pediatric group. Here we report a case of 4-year-old girl with biliary atresia status post living donor liver transplantation. Marked ascites developed 2 months later and Budd-Chiari syndrome was diagnosed. Cardiac catheterization showed significant stenoses of the hepatic vein and inferior vena cava. Initial balloon angioplasty of inferior vena cava only resulted in mild improvement. After successful balloon angioplasty of the hepatic vein stenosis, the stenosis of the inferior vena cava improved significantly. The ascites resolved soon, and no more happened up to 8 months' follow-up.
 ...
PMID:Transcatheter therapy of Budd-Chiari syndrome in a child after liver transplantation: report of one case. 1825 78

Budd-Chiari syndrome (BCS) is a venous outflow obstruction of the liver that has a dismal outcome if left untreated. Most cases of BCS in the Western world are caused by thrombosis of the hepatic veins, sometimes in combination with thrombosis of the inferior vena cava. Typical presentation consists of abdominal pain, hepatomegaly and ascites, although symptoms may vary significantly. Currently, a prothrombotic risk factor, either inherited or acquired, can be identified in the majority of patients. Moreover, in many patients with BCS a combination of risk factors is present. Myeloproliferative disorders are the most frequent underlying cause, occurring in approximately half of the patients. Recent discovery of the Janus Kinase 2 (JAK2) mutation has significantly contributed to the diagnosis of myeloproliferative disorders. Anticoagulation is indicated for all patients with BCS and additional therapy depends on the severity of symptoms and the extent of venous obstruction. A stepwise therapeutic approach is recommended, with increasing invasiveness and guided by the response to previous treatment. A transjugular intrahepatic portosystemic shunt (TIPS) is proving to be a good therapeutic option in patients with BCS, diminishing the need for surgical shunts. When all other therapy is unsuccessful or in patients with fulminant hepatic failure, a liver transplantation should be considered. Advances in diagnosis and treatment have dramatically improved the prognosis of patients with BCS. Still, many aspects of this complicated disorder remain to be clarified.
 ...
PMID:Vascular liver disorders (I): diagnosis, treatment and prognosis of Budd-Chiari syndrome. 1915 47

Budd-Chiari syndrome is a rather unusual clinical situation caused by occlusion of the hepatic vein of inferior vena cava, the classical triad of which are abdominal pain, ascites and hepatomegaly. A 29-year-old gravida 3 para 1 woman delivered an immature male baby weighing 2172 g with an Apgar score of 9 points at 35 weeks and 3 days of gestation. She was transferred to the National Hospital Organization Osaka National Hospital 112 days after delivery due to the sudden development of massive ascites. Magnetic resonance angiography and enhanced computed tomography detected the occlusion by thrombosis of both the middle and left hepatic veins, so she was diagnosed with Budd-Chiari syndrome. Her protein C antigen and activity were 37% and 50%, respectively, corresponding to type 1 protein C deficiency. Conservative treatment by continuous oral treatment of spironolactone (25 mg/day), furosemide (20 mg/day) and prophylactic warfarin (2 mg/day) much improved the ascites.
 ...
PMID:Case of Budd-Chiari syndrome 3 months after vaginal delivery. 1884 Jan 63

Budd-Chiari syndrome is a clinical disorder caused by hepatic venous obstruction with manifestations of abdominal pain, hepatomegaly, and ascites. Secondary Budd-Chiari syndrome is defined as an obstruction that results from material not originating from the venous system. We describe a rare case of fulminant Budd-Chiari syndrome secondary to renal cell carcinoma with tumor thrombus of the inferior vena cava and hepatic veins. The 59-year-old man was admitted to our hospital because of progressive appetite loss and markedly elevated serum transaminase. Abdominal ultrasonography revealed thrombosis in the hepatic veins and the inferior vena cava. Renal cell carcinoma with hepatic vein invasion was suggested by abdominal computed tomography and confirmed after a biopsy was taken from the hepatic venous thrombus. The patient died of fulminant liver failure within 10 days after admission. The clinical scenario and rationale for the selected management are further discussed.
 ...
PMID:Fulminant Budd-Chiari syndrome caused by renal cell carcinoma with hepatic vein invasion: report of a case. 1921 12

Alveolar echinococcosis, a parasitic disease due to the larval stage of the cestode Echinococcus multilocularis, is initially located in the liver in 97% of cases. Progression is very slow and the disease remains silent for many years. The developing larva behaves like a slow-growing liver tumor that gradually invades the liver parenchyma, vessels and bile ducts. Marked granulomatosis around the larva, and the subsequent strong reactive fibrosis, contribute to the severity of the disease. Gradual extension to adjacent organs and distant metastases due to haematogenous spread can also occur. Purely extrahepatic alveolar echinococcosis is rare, but physicians in endemic areas should be aware of this possibility. Diagnostic methods have dramatically improved over the past twenty years. The clinical presentation used to be similar to that of liver cancer, with slowly progressivejaundice (due to involvement of the hilum), huge, hard and irregular hepatomegaly, and a chronic Budd-Chiari syndrome due to hepatic vein involvement. Currently, with extensive use of abdominal ultrasonography, alveolar echinococcosis is commonly diagnosed when still asymptomatic. Alveolar echinococcosis may also be revealed by a complication, such as cholangitis due to communication between the parasite mass and the lumen of a bile duct or to pigment stones accumulating above a parasitic biliary stenosis; liver abscess related to centro-parasitic necrosis; or hematemesis due to esophagal varices in case of portal vein involvement. Metastases, especially in the lungs, reveal the disease in 5% of cases.
 ...
PMID:[Alveolar echinococcosis: a disease comparable to a slow growing cancer]. 1923 77

The Budd-Chiari syndrome is a rare disease associated with occlusion of the hepatic vein by a tumor or a thrombus. It develops due to progressive narrowing or occlusion of the hepatic veins and may occasionally proceed through the chronic disease within months, rarely years as individual recurrences, with pains, enlarged liver, and mild jaundice. These patients generally have partial hepatic vein occlusion. The paper describes a long (more than 20 years) course of the Budd-Chiari syndrome in which only a special angiographic study could verify the presumptive diagnosis and reveal the cause of evolving liver cirrhosis.
 ...
PMID:[A case of chronic Budd-Chiari syndrome]. 2038 79

Alveolar echinococcosis of the liver is a rare larval cestode disease which is due to the intrahepatic growth of the tapeworm Echinococcus multilocularis. This cestode naturally evolves as a larval stage within cysts in the body of carnivores. Humans are accidental intermediate hosts and become infected, either by eating food contaminated with carnivore-originated eggs or by touching foxes. It behaves as malignant liver tumour and rarely causes Budd-Chiari syndrome and variceal bleeding. Budd-Chiari syndrome is a hepatic venous outflow tract obstruction and may be present abdominal pain, hepatomegaly and ascites. Parasitic cysts may cause compression and thrombosis of the hepatic venous outflow tract. It may present as portal hypertension and variceal upper gastrointestinal bleeding. We here in report a 47-year-old woman without a prior history of liver disease presented with Budd-Chiari syndrome and variceal bleeding due to Alveolar echinococcosis. The course of this rare disease is demonstrated by means of the most important laboratory, serologic and radiologic parameters.
 ...
PMID:[Case report: Budd-Chiari syndrome and esophageal variceal bleeding due to alveolar echinococcosis]. 2095 22

Hepar lobatum carcinomatosum is an unusual cause of chronic liver failure, usually maskerading as cirrhosis. The pathogenesis of this syndrome is unclear. We report a case of liver failure revealing an occult lobular carcinoma of the breast, which offers the opportunity to gain further insight into the mechanisms of this rare cause of chronic liver disease. A 57-year-old woman, without history of malignancy, presented with hepatomegaly, ascites and altered liver tests (serum transaminase activity >5 N and hyperbilirubinemia). The transjugular liver biopsy performed at diagnosis showed an extensive fibrosis, containing scattered tumor cells, typical of metastatic lobular carcinoma of the breast. Four months later, after discovery of a rectal adenocarcinoma, a laparoscopy was performed; peritoneal carcinomatosis was discovered. A surgical biopsy of the liver was taken during the procedure: it showed histological features suggestive of chronic Budd-Chiari syndrome, with venocentric fibrosis and reversed lobulation. Intraluminal invasion of small hepatic veins and sinusoidal obstruction by neoplastic cells were observed. A small focus of lobular carcinoma was eventually discovered in the left mammary gland. The present case report expands the spectrum of clinical presentations associated with hepar lobatum carcinomatosum and points out to the importance of vascular injury in the pathogenesis of this rare cause of chronic liver disease.
 ...
PMID:Hepar lobatum carcinomatosum revealing an occult metastatic lobular carcinoma of the breast. 2107 93


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>