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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical, radiologic, and histologic features in 22 children with Budd-Chiari syndrome are reported. Three children had acute refractory ascites; all the others had hepatomegaly, which was detected either fortuitously or because of abdominal pain or distention. Results of liver function tests were normal or only moderately abnormal. In most cases a combination of ultrasonography and needle liver biopsy pointed to the diagnosis of Budd-Chiari syndrome, which was confirmed by angiography. Eighteen children underwent surgery involving various techniques, depending on the degree of patency of the inferior vena cava. Five children died postoperatively. Histologic studies of the liver, carried out in 12 of the surviving children, showed disappearance or regression of centrilobular hemorrhagic infiltration. Half of the surviving surgical patients are now free of complications after a follow-up of 7 months to 7 years; the others have either secondary thrombosis of the inferior vena cava or stenosis of the shunt or have experienced late pulmonary complications. Our results suggest that (1) Budd-Chiari syndrome must be considered a possible diagnosis in children with firm hepatomegaly and normal or near normal liver function, (2) surgery provides good results in many instances, and (3) the possibility of late complications requires careful follow-up.
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PMID:Budd-Chiari syndrome in children: report of 22 cases. 329 Apr 15

Although cases of hepatic vein thrombosis (Budd Chiari Syndrome) in oral contraceptive (OC) users have been reported in the literature, the association has not been definitively established. Hepatic vein thrombosis, an uncommon disorder, presents with right upper quadrant abdominal pain, hepatomegaly, and ascites. Diagnostic procedures include hepatic scintiscans, ultrasonography, computerized tomography and magnetic resonance imaging, determination of intrahepatic pressure, liver biopsy, and inferior vena cava and hepatic venography. Hepatic vein thrombosis may develop without an apparent underlying cause or as a complication of an illness known to be associated with vascular thromboses such as polycythemia rubra vera or paroxysmal nocturnal hemoglobinuria. In relation to the large numbers of women taking OCs, there have been very few cases of hepatic vein thrombosis. Evidence linking OC use to the development of hepatic adenomas is far more convincing. In a multicenter case-control study of 33 cases of hepatic vein thrombosis in women 15-45 years of age, each of whom was matched to 3-4 controls, the relative risk of hepatic vein thrombosis in OC users compared with nonusers was 2.37 (p 0.02). It was noted that the 1 patient with paroxysmal nocturnal hemoglobinuria, 5 of 12 patients with overt primary myeloproliferative disorder, and 7 of 8 patients with a forme fruste of a myeloproliferative disorder were OC users, suggesting that OCs--through their thrombogenic action--augmented the thrombotic tendency of the underlying condition. The objectives of therapy in hepatic vein thrombosis are to relieve the hepatic congestion and prevent further clot formation. The majority of patients die within 3 years of diagnosis.
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PMID:Hepatic vein thrombosis (Budd Chiari syndrome): possible association with the use of oral contraceptives. 329 14

Membranous obstruction of the inferior vena cava (IVC) with Budd-Chiari syndrome is a rare condition in children. Nine children with this lesion are described, eight of them from the Ovambo tribe in Namibia. Clinical features included hepatomegaly, splenomegaly, ascites, and dilated superficial abdominal veins draining cephalad below the umbilicus. Liver enzymes and serum proteins were slightly abnormal in most cases, and the ascitic fluid, when present, had a high protein content. Liver histology showed a wide range of changes, from almost normal to signs of severe chronic congestion. Angiographic appearance varied from a thin membrane between the IVC and right atrium to nonvisualization of the IVC except for dilated collaterals. Simultaneous injection of contrast medium into catheters positioned in the upper IVC and right atrium, through both the femoral and antecubital routes, is the most accurate way of demonstrating the extent of the obstruction and the patency of the hepatic veins.
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PMID:Membranous obstruction of the inferior vena cava with Budd-Chiari syndrome in children: a report of nine cases. 331 85

A 31-yr-old man presented with the Budd-Chiari syndrome. A liver biopsy specimen demonstrated noncaseating centrilobular granulomas and an idiopathic necrotizing granulomatous venulitis involving hepatic vein radicles with superimposed thrombotic occlusion. High-dose prednisone therapy produced dramatic clinical improvement with resolution of the hepatomegaly, ascites, and portal hypertension and normalization of the hepatic venous angiogram. A repeat liver biopsy specimen showed resolution of the venulitis and disappearance of the hepatic vein thrombosis. Idiopathic granulomatous venulitis that is reversible with prednisone therapy should be considered in the differential diagnosis of the Budd-Chiari syndrome.
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PMID:Response to steroids in Budd-Chiari syndrome caused by idiopathic granulomatous venulitis. 333 22

Budd-Chiari syndrome, a well known entity, is often difficult to diagnose, mostly due to the nonspecificity of its symptomatology. Radiocolloid liver scans were evaluated in eight cases of this disease, proven by surgical biopsy. Five cases showed the "classic" scintigraphic pattern of caudate lobe hypertrophy (62.5%), and other abnormalities observed included segmental hepatic insufficiency, diffuse hepatic insufficiency, and relative hypertrophy of both the caudate lobe and a portion of the parenchyma of segment VI (one case each). An experimental study of hepatic venous drainage performed on livers at autopsy revealed four groups of accessory hepatic veins in addition to the main hepatic veins. The occlusion of various parts of this drainage appears to relate to the various scintigraphic patterns that were encountered in patients with Budd-Chiari syndrome. A review of the literature revealed three additional patterns previously reported in association with Budd-Chiari syndrome (normal scan, diffuse hepatomegaly, and multiple filling defects). If all these variations are appreciated, liver scanning can be a valuable screening tool for Budd-Chiari syndrome and may also serve as a noninvasive means of follow-up.
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PMID:Budd-Chiari syndrome: typical and atypical scintigraphic aspects. 357 42

Both the Budd-Chiari and the nephrotic syndrome have previously been reported to occur secondary to occlusion of the vena cava. However, they have never been reported to occur simultaneously, nor have either of these entities been reported to occur secondary to massive hepatomegaly. This is the first report of both the Budd-Chiari and the nephrotic syndromes secondary to massive hepatomegaly with vena caval obstruction due to acute fatty liver from heavy alcohol intake. With shrinkage of the liver, the vena caval obstruction cleared as did the Budd-Chiari and nephrotic syndromes.
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PMID:Budd-Chiari and the nephrotic syndromes secondary to massive hepatomegaly. 381 22

Three patients with the Budd-Chiari syndrome are presented. This is a rare condition characterized by hepatomegaly, progressive and refractory ascites, distension of the abdominal wall veins, abdominal pain and leg oedema. These features are attributed to congestion of the liver and portal hypertension. The condition has been notoriously difficult to treat medically. Surgical measures are directed towards relieving the liver congestion and lowering pressure in the portal system by portal-systemic shunting operations. In some cases refractory ascites may be treated by peritoneovenous shunting with a Le Veen shunt. In a select group of patients orthotopic liver transplantation has proved to be worth while.
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PMID:Budd-Chiari syndrome. A report of 3 cases. 396 23

We have performed a side-to-side portacaval shunt as the treatment of choice in six cases of primary Budd-Chiari syndrome. All the patients (three men and three women, mean age 33 years) had marked hepatomegaly and massive ascites. Four patients had endoscopically proved esophageal varices with no episodes of bleeding. Preoperative angiographic studies, caval pressure measurement, laparoscopy, liver biopsy specimens, liver scanning, and liver function tests confirmed the diagnosis. A possible etiologic factor was evident in only two cases. A plain side-to-side portacaval shunt was performed in four patients, while two required the interposition of a graft. One patient died after surgery of hepatorenal syndrome. Four of the surviving patients are free of ascites and doing well at 29, 27, 25, and 6 months. The remaining patient subsequently developed cirrhosis and died 76 months after surgery. None of the patients who survived developed encephalopathy. Shunt patency was confirmed endoscopically by variceal decompression in the four patients with esophageal varices. We believe the side-to-side portacaval shunt is a reliable and effective procedure for the definitive management of primary budd-Chiari syndrome.
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PMID:Portacaval shunt in the treatment of primary Budd-Chiari syndrome. 402 24

Budd Chiari Syndrome, characterized by massive ascites, hepatomegaly, abdominal pain, and tenderness, nausea, and vomiting, is caused by obstruction of the hepatic venous outflow. Of the known causes of polycythemia rubra vera, hypernephroma, and other tumors invading the inferior vena cava have been most often reported, while pregnancy and oral contraceptives (OCs) have also been held as causes. In this paper the case is presented of a young woman, previously on OCs for 4 months, who developed the syndrome 2 weeks after delivery; she was also found to have multiple hepatic adenomas on laparotomy. The longterm use of OCs has been estimated to be associated with an annual incidence of liver cell adenoma of 3-4/100,000. Evidence suggests that the estrogen components, rather than the progesterone, of OCs seem more likely to cause liver cell adenoma since estrogens are carcinogenic in other organs and promote liver cell regeneration in rats. By interference with the metabolism of oncogenic bile salt derivatives, estrogen may exert its oncogenic effect. The patient is this case was told never to use OCs again since there is also evidence that the tumor may regress on stopping OCs, and she was advised against further pregnancies.
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PMID:Budd-Chiari syndrome and hepatic adenomas associated with oral contraceptives. A case report. 627 27

The article reports on the CT image of a venous thrombosis of the liver with severe parenchymal damage (Budd-Chiari's syndrome) in a young woman, resulting in her death. The x-ray morphology image showed a liver pattern of reduced density in spot form with hepatomegaly and ascites. The hypodense areas were both circumscribed and disseminated over both lobes with density values between +15 and +30 HE. Enlargement of the lobus caudatus is considered as typical of the disease (4, 7).
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PMID:[Budd-Chiari syndrome: the CT image]. 664 May 82


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