Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic thrombotic occlusion of the extrahepatic portal vein is one of the commonest causes of portal hypertension in adult Blacks. The condition occurs more frequently in men and may be accompanied by evidence of minor functional impairment of the liver. Associated pancytopenia is quantitatively related to the degree of splenomegaly. Assessment of a spectrum of variables of blood coagulation and fibrinolysis, while failing to shed light on the possible pathogenesis, does suggest a rational basis for thrombolytic therapy.
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PMID:Portal vein thrombosis in adults. 86 83

Cruveilhier-Baumgarten Disease is a rare clinical entity. The distinction between C-B Disease and C-B syndrome rests solely on the liver morphology. In C-B Disease, the liver is morphologically normal. A case of a 27-year-old juvenile diabetic female with C-B Disease is presented. She manifested many of the metabolic and hemodynamic changes of cirrhosis including portal hypertension, encephalopathy, and a hyperdynamic cardiovascular state. There was also associated splenomegaly, hypersplenism, and splenic artery aneurysms. Splenectomy and splenic artery ligation were performed. Liver biopsies had normal morphology. The patient has had no recurrence of her symptoms. The pathogenesis of C-B Disease is unknown.
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PMID:Cruveilhier-Baumgarten disease with associated splenic artery aneurysms. 86 36

Two patients with portal hypertension following chronic exposure to vinyl chloride monomer were studied histologically and ultrastructurally. Both cases showed non-cirrhotic portal hypertension accompanied by esophageal varices and splenomegaly. They proved hepatic fibrosis with conventional microscope. Ultrastructurally, there were large amounts of small myelin-like lamellar materials with electron density and remarkable proliferation and vesiculation of smooth endoplasmic reticulum in the cytoplasms of hepatocytes. The widened Disse's spaces, were the sinusoidal lining cells were arranged in multilayers, were plugged by collagenous fiber bundles. The finding may be consistent with the development of portal hypertension.
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PMID:Electron microscopic observation of the liver in portal hypertension following chronic exposure to vinyl chloride monomer. 89 37

Portal hypertension occurs in approximately 10% of patients with myelofibrosis. Increased portal blood flow secondary to splenomegaly has been proposed to explain its development. In a 60-year-old woman with proven myelofibrosis of 10 years' duration and gross splenomegaly, portal hypertension developed with esophageal varices and ascites. There was no demonstrable obstruction to portal blood flow. Following splenectomy the ascites and esophageal varices disappeared. Despite the presence of splenic myeloid metaplasia, splenectomy did not impair the patient's hematologic status. Portal hypertension complicating myelofibrosis has a poor prognosis, so careful attention should be given to its detection. Splenectomy may be preferable to portal-systemic shunting in the management of this complication.
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PMID:Portal hypertension complicating myelofibrosis: reversal following splenectomy. 90 49

Extra- and intrasplenic arterial aneurysms have occasionally been encountered in cases of portal hypertension both with and without cirrhosis. The exact pathogenesis is speculative; however, it is postulated that hyperkinetic splenic circulation, probably related to hepatic arterial insufficiency or hypoplasia, constitutes the primary pathogenic mechanism. Other factors such as splenomegaly and hormonal influence may be contributory.
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PMID:Extra- and intrasplenic artery aneurysms in portal hypertension. 91 41

Besides lymphodenopathy and splenomegaly, hepatomegaly may also be detected in 25-50% of children with juvenile rheumatoid arthritis. This is particularly evident in patients with complete Still's syndrome. The hepatomegaly increases during relapse situations and disappears during remissions. Transient icterus, elevation of aminotransferases and delayed bromsulfalein excretion have been reported, particularly in patients with complete Still's syndrome, and indicate impairment of liver function. Liver biopsies have been performed only rarely and show nonspecific infiltrations of portal fields with lymphocytes and, in a few cases, "autoimmune" hepatitis and even cirrhosis with portal hypertension. Plasma cell hepatitis with affection of joints can be readily differentiated from juvenile rheumatoid arthritis: the synovitis is merely transiet and disappears with institution of steroid therapy. As in the adult, severe liver dysfunction leads to remission of arthritis. Amyloidosis should be considered in every case of long-lasting hepatomegaly.
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PMID:[Liver pathology in juvenile chronic polyarthritis]. 91 83

Groups of young hamsters were exposed to 3, 20, 40, 80, or 160 cercariae. A highly significant correlation was observed between the number of cercariae, worm burdens, and liver and fecal egg counts. The most heavily infected animals were the first to lose weight and die. Hamsters exposed to 20 or more cercariae and harboring a mean of 4.2 or more worm pairs developed significant hepatosplenic disease by 10 weeks after infection as assessed by hepatomegaly, splenomegaly and the development of portal hypertension. Lightly infected animals with single worm pairs did not develop significant disease.
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PMID:Relation of intensity of infection to disease in hamsters with acute schistosomiasis mansoni. 96 80

Recognition that vinyl chloride could be hepatotoxic led to a survey of workers to determine whether changes had been induced by past exposure, and to evaluate standard liver function tests as monitors of early liver abnormalities. Standard liver function tests were found to be unsuitable for the detection of such abnormalities in the population at risk. Of 487 workers examined, 102 (20-9%) had abnormalities on initial testing but only two were finally shown to have portal hypertension; in both cases, thrombocytopaenia provided the first diagnostic evidence since liver function tests were normal. Furthermore, 40 (35-7%) of 112 control subjects had initial test abnormalities. A sample of 19 workers with various exposures to vinyl chloride monomer were examined blind by greyscale ultrasonography. Five with minimal or no exposure were confirmed as normal but 12 of the remainder had abnormalities. These consisted of an enlarged portal vein (seven instances), splenomegaly (eight), and changes in hepatic texture (seven). Five of these 12 cases had previously been considered normal. It was concluded that greyscale ultrasonography had many advantages over standard methods for screening workers exposed to hepatotoxic chemicals, and should be the subject of a large scale evaluation.
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PMID:Monitoring liver disorders in vinyl chloride monomer workers using greyscale ultrasonography. 96 99

Selective celiac and superior mesenteric arteriographies were performed in patients with portal hypertension. An arterioarterial (A-A) shunt between the superior mesenteric artery and the celiac axis via pancreatic arcades was found in fifteen of forty-three patients with associated massive splenomegaly. A mild A-A shunt disappeared after portacaval anastomosis alone, whereas a prominent A-A shunt was reduced but persisted. The persisting A-A shunt disappeared after splenectomy. These findings led us to suggest that the paucity of the blood flow in the common hepatic artery concomitant with increased splenic arterial flow to the massively enlarged spleen may result in a compensatory supply to the liver from the superior mesenteric artery via the shunt.
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PMID:Significance of selective arteriographic patterns in the celiac axis and superior mesenteric artery in portal hypertension. 98 16

The authors report the incidence of portal thrombosis in their experience, of patients who have been operated on for splenectomy as treatment for portal hypertension with splenomegaly and hypersplenism without a portal systemic shunt. In 161 patients studied angiographically for portal hypertension due to cirrhosis, portal thrombosis was shown in 19 patients. Of these 19 patients 13 previously splenectomized. Only 6 were never operated upon.
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PMID:Portal thrombosis: high incidence following splenectomy for portal hypertension. 105 64


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