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)

We report new operative approaches to the treatment of hepatic vein occlusion due to malignant tumors in the liver and their results in four patients. Two patients had hepatoma, one had metastatic melanoma, and one had metastatic leiomyosarcoma. All of them had abdominal pain, abdominal distention secondary to ascites, and massive hepatomegaly. The right lobe and medial segment of the left lobe of the liver were involved in three patients, and the involvement was diffuse throughout the liver in one. Hepatic veins were occluded completely in one patient, and two of three veins were occluded in the others. Two patients were treated by hepatic resection and removal of tumor thrombus from the hepatic vein under isolation-perfusion technique. They lived 18 and six months, respectively, without recurrence of Budd-Chiari syndrome. Tumors in the other patients were diffuse and could not be resected. The hepatic artery was ligated and chemotherapy was given postoperatively. Ascites and abdominal pain disappeared completely in one, who survived 17 months. The other patient had significant palliation and lived nine months.
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PMID:Surgical management of hepatic vein occlusion by tumor: Budd-Chiari syndrome. 19 61

Five patients with primitive chronic Budd-Chiari syndrome were treated by Dacron interposed mesocaval shunts for medically uncontrollable ascites. In two instances, hepatomegaly and ascites disappeared for four and four and one-half years. In one patient with severe stenosis of the inferior vena cava, moderate ascites required tapping once a month one year later, despite proved prosthesis patency. In two patients, death occurred ten and 30 days after shunting due to thrombosis of the graft. Inferior vena cava stenosis appears to be the major factor for decision of opportunity and type of portacaval shunt. From our material, we can describe three types of stenosis: type I, due to caudate lobe hypertrophy, and type II, due to right lobe hypertrophy, are suitable for side-by-side portacaval or mesocaval shunts. Type III, regular and extended narrowing of inferior vena cava, observed in long term evolutive forms, is presumably due to fibrosis and is not a good indication for conventional infrahepatic shunting procedures. Since this study was completed, another patient had a side-to-side portacaval anastomosis for chronic Budd-Chiari syndrome without caval stenosis. The patient has been observed for seven months, and ascites did not reappear. This underlines the importance of a complete radiologic and hemodynamic preoperative study of inferior vena cava outflow impairment.
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PMID:Interposition mesocaval shunt for chronic primary occlusion of the hepatic veins. 43 80

A 50-year-old man presented with abdominal pain followed by marked liver and renal dysfunction. Although liver scan appearances were not diagnostic, at laparotomy a tense, enlarged liver was found with thrombosis of the inferior vena cava. Despite full supportive measures the patient died within a month of laparotomy. Subsequent necropsy confirmed the diagnosis of Budd Chiari syndrome and revealed the primary cause to be a myosarcoma of the right atrium, an occurrence hitherto unreported.
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PMID:Budd Chiari syndrome from myosarcoma of right atrium. 51 45

The Budd-Chiari syndrome caused by occlusion of the major hepatic veins, often of unknown etiology, is typically characterized by massive ascites, hepatomegaly and abdominal pain due to intense congestion of the liver. The outcome has almost always been fatal. This report describes an evaluation of side-to-side portacaval shunt in dogs with experimental Budd-Chiari syndrome and in six patients with hepatic vein thrombosis. In the animal studies, side-to-side portacaval shunt was very effective in relieving massive ascites, hepatomegaly, hepatic congestion and portal hypertension produced by ligation of the hepatic veins. Only one of 24 dogs with side-to-side anastomosis reformed ascites, 67% of the animals survived until the study was concluded after one year, and liver biopsies showed reversal of the severe pathologic abnormalities. In contrast, all 20 control dogs subjected to a sham laparotomy, and all 20 dogs that underwent end-to-side portacaval shunt reformed massive ascites and died within six months with continued hepatic congestion and necrosis. All six patients with the Budd-Chiari syndrome due to hepatic vein occlusion had massive ascites (4.4-15.9 l), hepatomegaly, abdominal pain and disturbed liver function. In all six, angiography demonstrated occlusion of the hepatic veins with a patent inferior vena cava (IVC) and a normal IVC pressure, and liver biopsy showed intense centrilobular congestion and necrosis. The most valuable diagnostic study was angiography of the IVC and hepatic veins with pressure measurements. Side-to-side portacaval shunt was performed from four to 14 weeks after the onset of symptoms, and produced dramatic and sustained relief of ascites in five of the six patients during follow-up periods of from eight months to seven years. Liver function returned to normal, hepatosplenomegaly disappeared, none of the survivors developed portal-systemic encephalopathy, and follow-up liver biopsies showed disappearance of congestion and necrosis, but mild to moderate fibrosis. One patient died following an emergency IVC thrombectomy and portacaval shunt, which was undertaken when, during the course of his workup, his condition deteriorated suddenly because the thrombotic process extended from the hepatic veins into the IVC. The everpresent risk of this complication, and the dangers associated with delaying operation were emphasized by this case. It is concluded that side-to-side portacaval shunt, which decompresses the liver by converting the portal vein into an outflow tract, provides effective treatment of the Budd-Chiari syndrome when the occlusive process is confined to the hepatic veins.
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PMID:Treatment of Budd-Chiari syndrome by side-to-side portacaval shunt: experimental and clinical results. 69 34

Symptomatic occlusion of hepatic veins, the Budd-Chiari syndrome (BCS), may be on the increase in women taking oral contraceptives. 17 liver scans in 7 patients with confirmed BCS over a 7-year period were studied. 6 of the 7 patients were women. When more than 1 vein is occluded BCS results, with hepatomegaly, abdominal pain, ascites, and hepatic histology showing centrizonal sinusoidal distention, hemorrhage, and necrosis. Mortality has been high. BCS is associated with polycythemia, oral contraceptive use, malignancy, trauma, and congenital abnormalities. The scintigram appearance with radiocolloid is usually characteristic but a similar appearance has rarely been reported in cirrhosis of the liver and in 1 case of contrictive pericarditis. Excessive uptake in the midline with markedly diminished activity at the periphery may be the 1st clue that BCS is present. Confusing conditions with incomplete BCS include partial hepatectomy, radiation injury, fortuitous segmental involvement by diffuse or focal liver disease, and rarely hepatic artery occlusion. Treatment is by the porto-caval shunting operation. Venous obstruction as shown venographically has had good correlation with liver scans. After the shunt procedure, hepatic artery flow to the affected lobes has increased as the pressure falls and underperfusion of the hypertorphied midline section. After 14 months, the midline area has shown no uptake, possibly because of atrophy. Radiocolloid uptake also appears in the ribs, spine, and lung. This uptake recedes when the liver function improves.
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PMID:Liver scan in Budd-Chiari syndrome. 126 39

Thirty-three patients with Budd-Chiari syndrome were studied for 1 to 19 years following portal decompression. All had ascites, hepatomegaly, abnormal liver function, angiographic demonstration of inferior vena cava and/or hepatic vein occlusion, and biopsy specimens showing intense hepatic congestion and necrosis. When thrombosis was confined to hepatic veins (20 patients), side-to-side portacaval shunt resulted in 95% operative survival, 90% prolonged survival, permanent shunt patency, relief of ascites, reversal of liver dysfunction, and reversal or improvement of hepatic lesions. When thrombosis involved the inferior vena cava, mesoatrial shunt (eight patients) was unsatisfactory because of a 63% mortality rate from liver failure due to shunt thrombosis. In contrast, a new procedure consisting of combined portacaval and caval-atrial shunts (five patients) has been highly successful, with 100% survival, shunt patency, relief of ascites, and reversal of pathologic abnormalities.
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PMID:Long-term results of treatment of Budd-Chiari syndrome with portal decompression. 141 83

The Budd-Chiari syndrome (BCS) was diagnosed in a 30-year-old male hospitalized with hepatomegaly, abdominal collateral vessels and hepatic veins and inferior vena cava thrombosis (IVC) in 1988. The presence of circulating lupus anticoagulant (LAC) was suspected and demonstrated on this occasion in view of an earlier diagnosis of systemic lupus erythematosus (SLE) and recurrent vein thrombosis dating from 1981. There are sporadic reports of an association of BCS with SLE and other autoimmune diseases. The recent literature also describes associations with hypercoagulability due to LAC. These are reviewed together with the personal case to provide the rationale for correct diagnosis and therapy.
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PMID:Association of the Budd-Chiari syndrome with lupus anticoagulant. Case report and critical review. 174 29

Budd-Chiari syndrome is an unusual form of portal hypertension, resulting from partial or total obstruction of the major hepatic veins or of inferior vena cava. This is illustrated by the clinical history of a two year old girl admitted by anemia, hepatomegaly, thrombocytopenia, pleural effusion, ascites and ultrasonographic, scintigraphic and angiographic evidence os suprahepatic veins and inferior vena cava obstruction. Liver biopsy showed signs of chronic congestion and fibrosis. Initial manifestation of illness may be acute or, as in this patient, with an insidious onset of symptoms. Medical therapy gives usually poor results. Several surgical approaches have been tried to alleviate liver congestion, which in this case was intended by a 6 mm wide Goretex ringed tube prosthesis extending from superior mesenteric vein throughout right atrium. Patency of the artificial shunt was proven after several days by Doppler ultrasound but the patient died of pneumonia two months after operation.
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PMID:[Budd-Chiari syndrome]. 184 28

Crohn's disease is a chronic inflammatory granulomatous disorder affecting any part of the gastrointestinal tract, particularly the terminal ileum and the colon. Familiar complications are strictures, fistulae, perforation, haemorrhages and malabsorption due to multiple resections. A patient with two rare complications of Crohn's disease is described. A 16-year-old female with ileocaecal Crohn's disease presented with anaemia and ileus. This ileus was caused by some 40 tablets of ferrosulphate with a non-soluble matrix, in the presence of an existing stenosis of the ileum due to Crohn's disease. An ileocaecal resection was performed. During an exacerbation of Crohn's disease she developed hepatic vein thrombosis with a Budd-Chiari syndrome (upper abdominal pain, hepatomegaly and ascites). Prescription of tablets with a non-soluble matrix is contraindicated in patients with a partial stenosis of the intestine. Patients with active Crohn's disease are predisposed to thromboembolic complications. Hepatic vein thrombosis in our patient may have been the result of hypercoagulability during the exacerbation of her disease.
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PMID:[Intestinal obstruction caused by non-absorbable tablets and Budd-Chiari syndrome in a patient with Crohn's disease]. 204 72

Seven cases of Budd-Chiari syndrome are reported in children. The mode of onset was fulminant in one case with rapidly lethal liver failure, acute in 5 cases with rapid appearance of hepatomegaly and ascites and insidious in one case, with isolated hepatomegaly. Hepatomegaly, which is a constant sign, was present in the 7 patients. Ascites and collateral venous circulation were present in 6, splenomegaly in 2 and moderate jaundice in one only. Liver function tests, deeply abnormal in the patient with fulminant liver failure, was only slightly abnormal in the 6 others. Diagnosis was corroborated by ultrasonography, cavography, hepatic veins angiography and liver biopsy in 6 patients and by post mortem examination in the 7th. Etiologic investigations did not allow finding the cause of Budd-Chiari syndrome. However, this series can be distinguished by associated total villous atrophy in 3 cases, psoriasis in one, hepatitis B in one, hepatitis A and intestinal giardiasis in one. Portasystemic shunts were performed in 3 patients. One died in the immediate postoperative period, the 2 others are presently in good health with a 5 and 6 1/2 year-follow-up. One patient died rapidly from fulminant liver failure. Another, untreated, died 16 years after the onset of the disease, from an unknown cause. Two patients are lost to follow-up.
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PMID:[Budd-Chiari syndrome in children. Apropos of 7 cases]. 206 74


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