Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Using single radial immunodiffusion, ten glycoproteins from non purulent pleural fluids have been estimated in different diseases. For five proteins (prealbumin, ceruloplasmin, alpha2HS-glycoprotein, transferrin, beta2-glycoprotein 1) the results have been found not to correlate with the causal disease. However for orosomucoid, alpha1-antitrypsin, haptoglobin, alpha2-macroglobulin and hemopexin, there was good correlation between proteins levels and aetiology. The glycoprotein concentration was low in mechanical effusions from cirrhosis and chronic cardiac failure. It was high in inflammatory, post-embolism and particularly neoplastic effusions. A raised orosomucoid level occurred as the most characteristic of cancer states especially when associated with a parallel increase of the four other glycoproteins. A simultaneously elevated level of these five pleural glycoproteins seems to be a good and significant biological sign for neoplastic effusion diagnosis.
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PMID:[Glycoproteins of pleural effusions (author's transl)]. 40 7

In 45 patients with inflammatory bowel disease (9 with Crohn's disease and 36 with ulcerative colitis) and associated liver disorders, increased liver copper content (above 100 microgram/g dry weight) was found in 14 (31%). These patients represented about 50% of the patients with either biliary cirrhosis or pericholangitis. Four of the patients had levels regarded as compatible with hepatolenticular degeneration (greater than 250 microgram/g dry weight). In patients with chronic active hepatitis or non-specific changes in liver tissue, normal levels were found. The patients with Crohn's disease also had normal levels. Plasma ceruloplasmin was normal or increased in all. Determination of urinary copper output gave little diagnostic information. Alkaline phosphatases were markedly increased in most of the patients with increased liver copper concentration. In patients with ulcerative colitis and enhanced alkaline phosphatases, elevated liver copper content should be suspected and chelation therapy should be considered.
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PMID:Liver copper content in patients with inflammatory bowel disease and associated liver disorders. 53 3

Three patients, one with cryptogenic cirrhosis, one with active chronic hepatitis and one with neonatal hepatitis, were found to have corneal pigmentation rings indistinguishable from early Kayser-Fleischer rings on slit lamp examination. They did not have the clinical features of Wilson's disease and their serum copper and ceruloplasmin concentrations were normal. Urinary copper excretion rates and hepatic concentrations were only slightly raised but were below the range found in symptomatic Wilson's disease. It is concluded that the Kayser-Fleischer ring would no longer be considered as pathognomonic of Wilson's disease.
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PMID:Kayser-Fleischer-like rings in patients without Wilson's disease. 55 26

Increased liver copper concentration and raised serum ceruloplasmin were demonstrated in primary biliary cirrhosis and disorders of the biliary tract, and occasionally in chronic active hepatitis and cirrhosis of the liver. Eight of 13 patients with primary biliary cirrhosis had liver copper content as high as seen in patients with hepatolenticular degeneration (is greater than 250 mjg/g dry weight). Normal liver content was found in patients with acute hepatitis, steatosis of the liver, hepatic amuloidosis, haemochromatosis, and Gilbert's syndrome. The urinary copper excretion was increased (is greater than 75 mjg/24 h) in half the patients with primary biliary cirrhosis and occasionally in the other patient groups. Serum ceruloplasmin was raised in more than half of all patients, and none had levels below the reference range. Raised heaptic copper content did not always coincide with enhanced urinary copper excretion, but was significantly correlated with this parameter and also with ceruloplasmin, alkaline phosphatases, and vitamin-K-dependent clotting factors, but not with ALAT. Combination of laboratory data, as found in typical cases of hepatolenticular degeneration, was not observed in this study, including 66 patients.
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PMID:Hepatic copper content, urinary copper excretion, and serum ceruloplasmin in liver disease. 83 74

Kayser-Fleischer rings are pigmented corneal rings at the limbus of the cornea in Descemet's membrane that have been deemed pathognomonic of Wilson's disease. However, we have observed four exceptions in patients with non-Wilsonian liver disease. Three patients had primary biliary cirrhosis and one patient had chronic aggressive hepatitis with cirrhosis. Pigmented corneal rings were seen only by slit-lamp examination. Hepatic, serum, and urinary copper and serum ceruloplasmin levels were significantly elevated in the patients with primary biliary cirrhosis. Radiocopper (64Cu or 67Cu) studies in patients with primary biliary cirrhosis showed plasma disappearance curves which allowed a clear distinction from Wilson's disease in that all three patients with primary biliary cirrhosis showed a secondary rise in radiocopper that presumably represented copper incorporation into ceruloplasmin. In one patient, in whom 64Cu in ceruloplasmin was studied specifically, incorporation was found to be normal.
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PMID:Pigmented corneal rings in non-Wilsonian liver disease. 84 86

In two sibships 7 of 24 siblings were homozygous for Wilson's disease. In family A, the largest kindred of this recessively inherited disease thus far reported, the proband presented with chronic active hepatitis, one sibling died of cirrhosis, a second had clinical evidence of chronic liver disease and two others had biochemical and histologic changes in liver biopsy specimens. In family B the proband had cirrhosis and portal hypertension and one sibling had biochemical and histologic evidence of liver disease. All six living patients had low serum concentrations of ceruloplasmin and copper and a high 24-hour urinary excretion of copper, which was greatly increased by administration of D-penicillamine. None showed neurologic abnormalities and only one had Kayser-Fleischer rings (detectable only by slit-lamp examination). Each patient had an erythrocyte sedimentation rate (ESR) of 8 mm/h or less. After 3 and 2 years, respectively, of D-penicillamine therapy the conditions of the two probands had improved. Liver function became normal in three siblings, and no abnormalities developed in the remaining one. Thus, since Wilson's disease may present with chronic active hepatitis or cirrhosis with a normal ESR and without ocular or neurologic signs, it may be a more common cause of liver disease in young people than has been appreciated.
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PMID:Wilson's disease: a common liver disorder? 86 10

A patient who developed chronic cholestatic jaundice during the 1st year of life and eventually died of liver cell failure at the age of 18 years is described. During the terminal illness Kayser-Fleischer-like rings were observed and the serum concentrations of total copper and ceruloplasmin were elevated. At autopsy, a mixed macronodular and micronodular cirrhosis was found and cholangiography and dissection of bile ducts revealed no obstructive lesion of the biliary tract. There was no family history of hepatobiliary disease. Liver biopsies obtained at the ages of 5 and 7 years showed accumulation of bile droplets in hepatocytes, normal-appearing bile ducts, no significant fibrosis, and intact lobular architecture. Striking features of the terminal cirrhosis were the presence of Mallory bodies and a marked excess of copper in the liver (2,175 mug per g dry weight). The latter two findings, as well as the elevated serum concentrations of total copper and ceruloplasmin, may be attributable to chronic cholestasis per se. This study emphasizes the clinical and therapeutic problems posed by chronic cholestasis of unknown etiology in childhood.
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PMID:Progressive intrahepatic cholestasis of infancy and childhood. A clinicopathological study of patient surviving to the age of 18 years. 95 55

Wilson's disease usually has its onset in childhood, adolescence, or early adulthood. The clinical picture of hepatic dysfunction without dysfunction of the central nervous system is more typical of the disease in the child or the adolescent than in the adult. We are presenting the case of a man whose age at onset of the disease was 55 years and who had the hepatic complications of Wilson's disease without clinical evidence of disease of the central nervous system. All patients with chronic hepatitis (chronic active liver disease) or cirrhosis of unknown etiology should be screened for the possibility of Wilson's disease. This screening should include slit-lamp biomicroscopy for Kayser-Fleischer rings, determination of serum ceruloplasmin concentration, and measurement of 24-hour urinary excretion of copper. If doubt exists concerning the diagnosis, either a radiocopper kinetic study, using 64Cu or 67Cu, or, if the patient's condition permits, a liver biopsy with measurement of hepatic copper concentration should be done. The rubeanic stain of hepatic tissue for copper is unreliable in making or excluding the diagnosis of Wilson's disease.
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PMID:Wilson's disease (hepatolenticular degeneration) of late adult onset: report of case. 115 37

Two cases of acute hepatic failure are reported in which the diagnosis of Wilson's disease was considered because of low serum ceruloplasmin, low serum copper levels and high 24 h urinary copper. Case 1 had Kayser-Fleischer rings, haemolysis and a high 24 h urinary copper, and so Wilson's disease was confidently diagnosed. Case 2 had high urinary copper excretion, but [64Cu] study indicated a 24:2 h ratio of 0.7 and made the diagnosis of Wilson's disease uncertain. Both patients underwent orthotopic hepatic transplantation, and multiple biopsies were taken from the resected specimen in order to estimate hepatic copper levels. In both cases, hepatic copper levels revealed considerable variation: 0.8-5.2 mumol/g dry wt (case 1) vs 0.02-12.65 mumol/g dry wt (case 2). In case 1, only two of 14 levels were within the diagnostic range for Wilson's disease (greater than 4 mumol/g dry wt), whereas hepatic copper levels in case 2 were in the Wilsonian disease range in three of 16 specimens. These results were in contrast to uniformly high hepatic copper levels in one patient with established cirrhosis secondary to Wilson's disease and two cases of primary biliary cirrhosis. This report indicates that hepatic copper levels vary greatly in acute liver failure, and that estimates from a single biopsy specimen may be misleading as to the cause of the underlying liver disease.
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PMID:Striking variability of hepatic copper levels in fulminant hepatic failure. 151 66

Indian childhood cirrhosis is a fatal liver disease characterized by a striking accumulation of copper-containing granules within hepatocytes. A two-year-old American boy, the product of a third-cousin marriage, with clinical, biochemical, and histological signs of Indian childhood cirrhosis was studied. Liver biopsies at 22 and 30 months of age revealed a rapid progression from fibrosis to micronodular cirrhosis, with many of the remaining hepatocytes staining strongly for copper and copper-binding proteins. Electron microscopy showed characteristic dense granules containing copper and sulfur by electron probe analysis. Hepatic copper content was 1500 micrograms/g dry weight (normal, 20-50). Urinary copper was 3.6 mumol/d (229 micrograms/24 hours; normal, 15-20), and serum ceruloplasmin was 352 mg/L (normal, 150-320). The case suggests that both genetic and environmental components contribute to the manifestations of Indian childhood cirrhosis, and that the diagnosis of Indian childhood cirrhosis should be considered even in non-Indian infants with progressive liver disease.
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PMID:Indian childhood cirrhosis in an American child. 142 97


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