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Query: UMLS:C0038002 (splenomegaly)
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Hemodynamic studies were performed in 5 vinyl chloride monomer workers in whom splenomegaly or thrombocytopenia was detected during a screening program at major chemical plant. Three patients had portal hypertension and collateral venous circulations, with intrasplenic pressures between 20 and 29 mm Hg and normal wedged hepatic venous pressures, but the gradient between the wedged and free hepatic vein pressures was also increased. Splenic blood flows were increased in both hypertensive and normotensive patients. There was no correlation between the splenic blood flow and the portal pressure or the presence of portal fibrosis. The portal hypertension associated with vinyl chloride exposure is mainly presinusoidal in type, and may be attributed to an abnormality of the portal vein radicles, or hepatic sinusoids.
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PMID:Portal hypertension in vinyl chloride monomer workers. A hemodynamic study. 66 6

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

Fifteen male workers exposed to vinyl chloride developed angiosarcoma of the liver. Thirteen died of disease and two are currently living for short periods after diagnosis. Their ages ranged from 36 to 58 years (average 47.5 years). Their exposure time ranged from 4 to 27.8 years (average 16.9 years). The most common presenting symptoms were fatigue, weight loss, and abdominal pain. Hepatomegaly followed by splenomegaly were the most common physical findings. Biochemical profiles yielded variable results and proved to be of little value in the detection or diagnosis. Of eight patients autopsied, distant organ involvement was present in two cases, duodenal involvement in one, and direct extension of tumor to adjacent organs or tissues in four additional ones. The remainder, diagnosed by open liver biopsy, revealed no tumor extension. The gross features of the tumors were hemorrhagic necrosis, cystic degeneration, fibrosis, and apparent multicentricity. The histologic features were those of the typical angiosarcoma found in a variety of sites with a wide range of cellular differentiation. The histologic diagnosis was often impaired by the extensive tumor necrosis. Elsewhere in the liver subcapsular fibrosis, a distinct type of portal fibrosis, and endothelial cell hyperplasia with or without sinusoidal dilatation were noted. The reduction of industrial chemical exposure has already been achieved and will hopefully eliminate this chemically related tumor in the future. There is, however, a significant group of previously exposed workers who will require careful monitoring to detect functional abnormalities of the liver and possible early neoplastic changes.
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PMID:Clinical and morphologic features of hepatic angiosarcoma in vinyl chloride workers. 94 81

Hepatic angiosarcoma, recently discovered in a large series of vinyl chloride workers, demonstrates characteristic angiographic and radionuclide changes. Tumors exhibiting central hypovascularity with puddling are usually surrounded by a peripheral stain. A negative peripheral defect is demonstrated on hepatic scan. Healing hepatic infarction secondary to wedged hepatic venography creates a false-positive lesion on angiography similar to angiosarcoma. Splenomegaly and systemic venous hypertension develop in a number of these patients.
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PMID:Angiographic and radionuclide characteristics of hepatic angiosarcoma found in vinyl chloride workers. 94 24

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

Preliminary review of hepatic biopsy and autopsy specimens obtained from workers engaged for prolonged periods in the polymerization of vinyl chloride indicates a fibrotic precursor lesion in the liver. It is the only lesion in some instances but also was found in the uninvolved liver of patients with angiosarcoma and in two instances in liver biopsy specimens obtained before angiosarcoma developed. This precursor stage is characterized by a conspicuous subcapsular fibrosis, a nonpathognomonic progressive portal fibrosis, and a borderline increase of intralobular connective tissue, all associated with focal stimulation of sinusoidal lining cells and hepatocytes. This precursor stage is often accompanied by splenomegaly with enlarged Malpighian follicles and in some instances by portal hypertension requiring portacaval shunt because of variceal hemorrhage. The portal hypertension is explained by increased splenic blood flow in the face of nondistensible fibrotic portal tracts as well as Glisson's capsule. Transition to angiosarcoma is preceded by focal dilatation of sinusoids with even greater activation but dedifferentiation of their lining cells. This lesion is presumably the result of stimulation of various hepatic as well as splenic cells by vinyl chloride or its metabolites. The demonstrated evolution is identical with that following prolonged exposure to inorganic arsenicals. It is postulated also that other instances of inconspicuous hepatic fibrosis associated with splenomegaly and often with portal hypertension, a combination designated as Banti's syndrome or "idiopathic portal hypertension," are the result of a toxic, possibly environmental agent, particularly since the disease is found with greater frequency in some parts of the world. The delineation of the fibrotic precursor stage in the liver may assist in the epidemiologic studies of the vinyl chloride-induced injury.
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PMID:Alterations of liver and spleen among workers exposed to vinyl chloride. 105 51

Idiopathic portal hypertension is reported in five cases including one case of chronic arsenical intake and one case of chronic industrial vinyl chloride exposure. In all five cases the patients presented with gastrointestinal bleeding as the chief complaint. Physical examination was within normal limits except for splenomegaly in all. Results of liver function tests were normal, except for the relative clearance of sulfobromophtalein. A surgical liver biopsy specimen was obtained in all cases and showed moderate degrees of portal fibrosis, but no cirrhosis. Combined umbilicoportal, hepatic vein and superior mesenteric artery catheterization was performed in all cases. Hepatoportographies showed distortion of the intrahepatic portal venous system and cut-off of small portal venules. Porto-hepatic gradients ranged from 14.0 to 20.5 mm Hg. The portal hypertension was both sinusoidal and presinusoidal in nature but mainly presinusoidal. Hepatic extraction of indocyanine green and of albumin microaggregates was normal, thereby suggesting normal functional portal blood supply to the liver. The patients with arsenical or vinyl chloride exposure could not be differentiated from the other three patients with idiopathic portal hypertension. These results suggest that idiopathic portal hypertension may be related to domestic or industrial exposure to other hepatotoxins.
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PMID:Idiopathic portal hypertension. 108 57

Histologic examination of liver tissue (eight autopsy and 18 biopsy specimens) and five spleens from 20 workers with vinyl chloride polymerization showed hepatic angiosarcomas in 15. In addition, a peculiar pattern of progressive portal-tract, inconspicuous intralobular and conspicuous capsular fibrosis was observed in the five workers without angiosarconma, in all the seven patients with angiosarcoma from whom tumor-free portions of the liver were available, and in two tumor-free biopsies from patients subsequently found to have angiosarcoma. The fibrosis was accompanied by splenomegaly. Hypertrophy and hyperplasia of both hepatocytes and hepatic and splenic mesenchymal cells were also seen. The histologic similarity to chronic inorganic arsenical poisoning, in which angiosarcomas also occur, and to idiopathic portal hypertension (Banti's syndrome) suggests that the latter syndrome at times results from unknown toxic, possible environmental, chemicals.
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PMID:Vinyl-chloride-induced liver disease. From idiopathic portal hypertension (Banti's syndrome) to Angiosarcomas. 116 15

Peculiar clinical symptoms observed in polyvinyl chloride production workers of a nearby chemical plant arose the suspicion of liver and spleen disease in these workers before first reports on vinyl chloride-induced angiosarcoma of the liver became known. Starting at the end of 1972, 44 workers were followed up by biochemical investigations, peritoneoscopy and liver biopsy. As early as 1973 it could be ascertained, that advanced vinyl chloride-induced liver damage presents as hepatic fibrosis with splenomegaly, portal hypertension, and thrombocytopenia without conspicuous deterioration of hepatic parenchyma function. Similar symptoms have been observed after chronic intoxication by arsenic and thorium dioxide (thorotrast). The clinical and peritoneoscopic aspects of the disease are described.
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PMID:[Liver disease in polyvinyl chloride production workers - Clinical and peritoneoscopic aspects (author's transl)]. 121 65

Thirteen workers with persistent abnormalities in one or more liver function tests (LFT) at a vinyl chloride monomer (VCM) polymerization plant were investigated. Twelve workers were found to have VCM-induced liver dysfunction based on circumstantial evidence. They were employed between 1971 and 1982 when the VCM levels ranged from 1 to 21 p.p.m. After 1982 when the environmental VCM levels were controlled to below 1 p.p.m., no cases of VCM-induced liver dysfunction were detected. In most cases, glutamic pyruvic transaminase was the earliest parameter to be raised. The second most common parameter is serum gamma glutamyl transpeptidase. The latent period ranged from 1 to 13 years. An improvement in their LFT results was shown by 83.3 per cent of workers within 6 months to 2 years after removal from further VCM exposure. For workers who returned to VCM work, their LFT became abnormal again. Liver scans showed hepato and/or splenomegaly in most cases. Liver biopsies on 9 workers were reported as 'non-specific fatty changes' of varying degrees. These observations highlight the need for continual vigilance with environmental monitoring and medical surveillance of VCM-exposed workers.
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PMID:Persistent liver dysfunction among workers at a vinyl chloride monomer polymerization plant. 201 Oct


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