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)

Hepatic venous outflow obstruction also called the Budd-Chiari syndrome is increasingly being recognized as a cause of portal hypertension. In western countries the obstruction is usually in the hepatic veins while in reports from South Africa, Japan and India the predominant cause is a block in the IVC at the level of the diaphragm above the entry of the hepatic veins. A hypercoagulable state caused by myeloproliferative haematological disorders, clonal defects in haemopoietic stem cells, lupus anticoagulant, contraceptive pills and postpartum state are some of the aetiological conditions described. However in 25% to 75% cases no cause can be identified. The predominant presenting features in patients with hepatic vein obstruction are hepatomegaly and ascites while those with IVC obstruction show prominent veins on the trunk and back. Ultrasound examination should be the first investigative step. However a liver biopsy is the gold standard of diagnosis. To confirm the site of obstruction inferior vena cavography or functional hepatography may be required. In the acute phase thrombolytic therapy may be useful but for established cases either surgical intervention in the form of shunts or recently balloon angioplasty may be helpful. For patients with established cirrhosis and end-stage liver failure the only alternative is liver transplantation. All these patients however should be put on long term anticoagulants to prevent rethrombosis. Some series have reported that upto 45% of patients may develop hepatocellular carcinoma on long term followup. With proper management a larger proportion of patients can be returned to a useful productive life.
 ...
PMID:Hepatic venous outflow obstruction. 982 3

Budd-Chiari syndrome is a rare manifestation of hereditary or acquired thrombophilia. We saw a case of Budd-Chiari syndrome in a 30-year-old woman leading to initial diagnostic difficulties. She underwent surgical side-to-side shunt and 9 weeks later an almost normal liver could be demonstrated on computerized tomography. Budd-Chiari syndrome should be considered if the Chiari triad with abdominal pain, hepatomegaly and ascites occurs in a patient. If necessary, invasive diagnostic procedures (e.g. angiography) must be performed. Therapeutic options are anticoagulative therapy and porto-systemic shunt, either as a TIPS or a surgical shunt. If severe liver failure occurs or liver cirrhosis is present, orthotopic liver transplantation is an additional option which also cures hereditary thrombophilia.
 ...
PMID:[Budd-Chiari syndrome--a rare manifestation of hereditary thrombophilia]. 1084 Jun 19

This study was to evaluate the clinical effects of percutaneous balloon angioplasty of Budd-Chiari syndrome (BCS) caused by inferior vena cava (IVC) obstruction. Between 1993 and 1999, 28 men and 14 women with mean age of 44+/-12 years underwent percutaneous balloon angioplasty for primary BCS. Color Doppler ultrasound and venography showed membranous and segmental obstruction of IVC in 29 and 13 patients, respectively. Fourteen patients also had left- and/or mid-hepatic vein obstruction. Angioplasty of IVC was successful in 41 patients (97.6%), resulting in a reduction of pressure gradient between IVC and the right atrium from 15.0+/-2.5 to 5.5+/-0.8 mmHg (P<0.01). A stent was placed in the site of obstruction in the patient with unsuccessful balloon angioplasty. Patients with successful angioplasty or stent placement had significant improvement in clinical symptoms indicated by a reduction in hepatomegaly and the degree of ascites. No specific attempt was made to treat the occluded left- and/or mid-hepatic vein due to the presence of potent right hepatic vein. Over the follow-up period of 32+/-12 months, restenosis of IVC occurred in only one patient (2.4%), which was redilated successfully. Percutaneous balloon angioplasty is a safe and effective therapy for Budd-Chiari syndrome caused by IVC obstruction, therefore should be the first choice of treatment for this condition.
 ...
PMID:Percutaneous balloon angioplasty of inferior vena cava in Budd-Chiari syndrome-R1. 1200 92

Three patients with rapid onset decompensated liver disease who were referred to our hospital with presumed Budd-Chiari syndrome are described. This diagnosis was based on marked hepatomegaly, caudate lobe hypertrophy and failure to visualize hepatic veins by Doppler-ultrasound examination. Detailed history, biochemistry, and histology were, however, highly suggestive of alcoholic liver cirrhosis with steatohepatitis-induced hepatomegaly. On angiographic examination narrowed but patent hepatic veins were demonstrated in 2 patients. A third patient died before further work-up could be performed; autopsy showed patent hepatic veins. These cases show that the radiological diagnosis of Budd-Chiari syndrome due to hepatic vein obstruction can be false positive and that suspected hepatic vein occlusion on Doppler-ultrasound should be confirmed by angiographic studies and on liver histology.
 ...
PMID:Pseudo-Budd-Chiari Syndrome: decompensated alcoholic liver disease mimicking hepatic venous outflow obstruction. 1206 96

Possible hepatic effects of oral contraceptives (OCs) include tumors, intrahepatic cholestasis, and less well known vascular lesions such as Budd-Chiari syndrome and peliosis, a disseminated pseudocystic dilatation of the sinusoid capillaries of the liver. A 29-year-old woman with a history of 4 pregnancies, hypertension and diabetes both requiring daily medication, and use since April 1983 of an oral contraceptive (OC) containing .15 mg levonorgestrel and .03 mg of ethinyl estradiol complained in March 1984 of epigastric pain and increased abdominal volume. Ascitis was diagnosed and the patient was hospitalized. She had experienced a generalized pruritus for several months and had lost weight. The bilirubin, alcaline phosphatase, and Gamma GT levels were slightly elevated. Sonography showed a hypertrophied liver. Incipient esophageal varices were seen with gastric fibroscopy. The small subhepatic venous branches had a cloudy aspect. The peliosis hepatis was diagnosed by a transjugular puncture biopsy of the liver. With discontinuation of the OCs, the ascites did not reappear after puncture and the perturbations of the liver functioning normalized. On follow-up in April 1985, slight hepatomagaly persisted but the patient reported no further symptoms. She continued her medication for hypertension and diabetes. Peliosis hepatis was 1st described in 1964 and several cases related to OC use have been reported since 1972. Peliosis has the aspect of multiple small congestive cavities of 1-3 mm in diameter in the parenchyma. The lesions consist of areas of hepatocellular necrosis secondarily filled with blood. The cysts may be voluminous and subcortical, creating a risk of hemoperitoneum. The lesions may also be associated with a benign or malignant liver tumor. Regression of the lesions is possible with termination of the etiologic agent. Clinically, hepatomegaly, painful or not, sometimes associated with splenomegaly, is often found with peliosis. Moderate jaundice is very frequent. Ascites or edema of the legs are observed. Hyperbilirubinemia and augmentation of phosphatases and Gamma GT are the main laboratory findings. Transaminases may be slightly elevated, and the rate of prothrombin may be diminished. The condition is sometimes diagnosed with laparoscopy, celiomesenteric arteriography, or phlebography, but hepatic puncture biopsy usually establishes the diagnosis. The contition may improve if the etiologic agent is removed or it may worsen because of liver failure or a complication such as hemoperitoneum or an associated tumor.
 ...
PMID:[Peliosis hepatis and oral contraceptives: a case report]. 1228 Oct 5

Complications of oral contraceptives (OCs) affecting the gastrointestinal tract, liver and pancreas are rare but potentially serious. Hepatobiliary complications are by far the most frequent and varied. Hepatic lesions will probably decline in frequency as low-dose OCs replace higher dosed pills. Intrahepatic cholestasis induced by OCs resembles that of pregnancy. There may be a genetic predisposition to both conditions involving a dose-dependent estrogen effect of decreasing bile secretion. Intrahepatic cholestasis appears within 6 cycles of OC use. Symptoms include pruritus with anorexia, asthenia, vomiting, and weight loss without fever, rash or abdominal pain. Termination of OCs clears the condition without sequelae within 1-3 months, sometimes after a temporary aggravation. A moderate and asymptomatic cytolysis may appear when OC treatment is begun. Sinusoidal dilatation has been conclusively linked to OCs although few cases have been published. Clinical manifestations other than hepatomegaly are variable. Abdominal pain and fever are the most common. The condition is not related to duration of use and disappears 5-15 days after OC use is terminated. The relative risk of Budd-Chiari syndrome in OC users is estimated at 2.37. OCs increase the prevalence of hepatic adenomas as a function of duration of treatment. They are usually discovered fortuitously but may be revealed by vague abdominal pains. Hemorrhagic complications are more likely in OC users. It may be difficult to distinguish between adenomas, hepatocellular carcinoma, and focal nodular hyperplasia. A puncture biopsy guided by sonography may aid diagnosis. The natural history of adenomas is poorly understood and treatment remains controversial. OCs do not appear to increase the risk of focal nodular hyperplasia but they increase the size of the tumor and the risk of hemorrhage. OCs should be terminated because of risk of hemorrhage. Surgical resection is not indicated unless there are complication or diagnostic doubts. While hepatocellular carcinoma is very rare, its risk is increased by a factor of 7-20 in women using OCs for 8 years or more. Use of combined OCs appears to speed development of lithiasis in predisposed women. Risk of lithiasis is linked to estrogen content in women under 30. Several cases of acute pancreatitis in the 1st 3 months of treatment have been reported in women with preexisting lipid metabolic anomalies. Cases of ischemic lesions of the small intestine or colon have been reported in OC users with A positive blood type. Such lesions can be fatal without early diagnosis and termination of OCs. Gastric esophageal reflux is increased by progestins. Preexisting constipation may be aggravated and the incidence of Crohn's disease increased by OCs. It is advisable to rule out preexisting hepatic pathology before prescribing OCs. OCs should be stopped in case of viral hepatitis.
 ...
PMID:[Contraception and hepatogastroenterology]. 1231 76

Budd-Chiari syndrome (BCS) is an uncommon disorder caused by hepatic venous outflow obstruction. It is characterized by ascites, hepatomegaly and abdominal pain. Percutaneous interventions have recently been used for the treatment of BCS. We present a case of BCS with a closed mesocaval shunt which was reopened with a self-expandable metallic stent.
 ...
PMID:Stent angioplasty of closed mesocaval shunt in a patient with Budd-Chiari syndrome. 1232 21

Here we describe two women with polycythemia vera presented with fulminant hepatic failure due to acute Budd-Chiari syndrome. Both had a history of severe abdominal pain and distention of short duration. Clinical and laboratory findings showed fulminant hepatic failure due to acute Budd-Chiari syndrome. Diagnosis was confirmed with abdominal ultrasonography and Doppler ultrasonography showing ascites, hepatomegaly, portal hypertension and total occlusion of hepatic veins. Complete blood count and other clinical findings were compatible with polycythemia vera in both patients. Patients were treated successfully with early administration of continuous heparin infusion, repeated phlebotomies and hydroxyurea. We emphasize here early diagnosis and effective treatment in such fulminant cases may be life saving.
 ...
PMID:Early medical treatment is life saving in acute Budd-Chiari due to polycythemia vera. 1274 60

The case of a 42-year-old female with Budd-Chiari syndrome (BCS) and lupus anticoagulant is reported. This patient, who had been on chronic anticoagulants for her lupus anticoagulant, presented with abdominal pain, dyspnea on exertion, engorged abdominal wall venous collaterals, and hepatomegaly. A dynamic computerized tomography of the abdomen showed complete suprahepatic inferior vena caval occlusion at the junction with the right atrium. IVC venogram confirmed the diagnosis and also demonstrated patency of the hepatic veins. Free hepatic venous pressure was 25-26 mm Hg. Histopathologic examination of the liver showed marked central venous congestion with significant bridging fibrosis. The total caval occlusion and overt calcification of the clot precluded radiologic angioplasty, and the patient underwent a successful surgical thrombectomy with cavoplasty utilizing an autologous venous patch. Several weeks following surgery, she was free of symptoms and resumed her usual daily activities. Follow-up venography showed a widely opened cava with normal free hepatic vein pressures. Repeat liver biopsy at 6 months showed complete resolution of the hepatic venous congestion and a decrease in the degree of fibrosis.
 ...
PMID:Suprahepatic Budd-Chiari syndrome treated with thrombectomy and cavoplasty. 1292 61

Budd-Chiari syndrome (BCS) is a disorder caused by occlusion of the hepatic vein or inferior vena cava. The clinical presentation include abdominal pain, hepatomegaly, ascites, leg edema, collateral venous dilatation of the body trunk, and portal hypertension. In addition, BCS can cause hepatocellular carcinoma (HCC) in some patients, although its pathogenesis is not yet completely understood. The average reported time lag from diagnosis of BCS to full-blown HCC ranges from several years to several decades. Hepatic carcinogenesis in patients with BCS perhaps reflects a prolonged and persistent liver injury in that it occurs in the primary inferior vena cava obstruction rather than the primary hepatic vein thrombosis. Among patients with BCS, membranous obstruction of the vena cava (MOVC) usually presents an insidious and chronic illness, whereas primary hepatic vein thrombosis presents an acute or subacute illness. We experienced a case of a patient with BCS, which progressed rapidly that HCC developed only nine months after the diagnosis of BCS. The factors causing this rapid progression are still unclear and remain to be investigated.
 ...
PMID:Rapidly progressing Budd-Chiari syndrome complicated by hepatocellular carcinoma. 1461 90


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