Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

One group of 8 patients with primary biliary cirrhosis and one group of 7 patients with common duct stone both had generally high levels of blood coagulation factors and a wide range of other plasma proteins. In contrast, one group of 6 patients with chronic active hepatitis generally had low levels of the same coagulation factors and plasma proteins. Analyses of factor II-VII-X, plasminogen, ceruloplasmin, beta1C/1A-globulin, IgG and IgM are especially helpful in the differentiation between primary biliary cirrhosis and chronic active hepatitis. The data are interpreted as suggesting a generally increased synthesis of liver-produced proteins in cholestasis, although more specific factors may modify the degree of increase of the individual proteins.
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PMID:Observations of increased levels of blood coagulation factors and other plasma proteins in cholestatic liver disease. 93

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

Many hepatic lesions, ranging from subcellular alterations to malignant tumors, have been attributed to the use of anabolic steroids (AS) and contraceptive steroids (CS). These lesions that have been attributed to AS and CS are discussed with focus on the following: biochemical changes; subcellular alterations; intrahepatic cholestasis; vascular complications (sinusoidal dilatation, peliosis hepatitis, Budd-Chiari syndrome); hyperplasia and neoplasia (diffuse hyperplasia, nodular transformation, focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma, and miscellaneous malignant tumors); and miscellaneous effects (effects of preexisting liver disease, cholelithiasis, and pancreatitis). OCs have a number of physiologic effects on the liver. These include decreased bile flow, diminished secretion of organic anions, and decreased synthesis and secretion of bile acids. Retention of bromosulfophthalein has been noted with AS during late pregnancy and in the puerperium. It is well established that the CS can lead to elevations of serum ceruloplasmin and copper levels. Subcellular alterations have been reported in both humans and rats on AS or women on CS and involve multiple organelles of the several systems of the liver. Both AS and CS have been implicated in intrahepatic cholestasis. Jaundice usually develops after 2-5 months of therapy with AS or after 3 months of OC use. The lesions attributed to CS and AS can involve any of the systems of the liver. At times more than 1 system is affected simultaneously. Most of the steroid related lesions resemble similar ones caused by other etiologies. Some, such as peliosis hepatitis, are rarely related to other etiologies, but others can be termed steroid specific. A number of diseases associated with the CS or AS also occur in pregnancy. Acute fatty metamorphosis of pregnancy and the periportal hemorrhagic necrosis characteristic of eclampsia have not been reported in patients on CS. Spontaneous rupture of the liver during pregnancy has not been attributed to the CS.
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PMID:Hepatic lesions caused by anabolic and contraceptive steroids. 628 45

Total complement activity was normal in 18 patients with primary biliary cirrhosis using two hemolytic assays capable of distinguishing between defects in classical and alternative pathways. Activation of the classical pathway was demonstrated in all patients by formation of complexes between C1r, C1s, and C1 inactivator. Large amounts of free C1q, not in complex with C1r and C1s, were demonstrated in the majority of patient sera. Furthermore, C4 levels were within the normal range or slightly subnormal. No evidence for alternative pathway activation was found. Increased mean levels of several complement components, in particular C1 inactivator, C2, C3, factor B, factor H, were noted. A significant correlation between these complement factors, derived mainly from the liver, and ceruloplasmin suggests that this elevation might be secondary to cholestasis. In contrast, no significant correlation with levels of early reacting acute phase reactants, immunoglobulins, or circulating immune complex-like material were observed. It is concluded that activation of the complement system by the classical pathway is common in patients with primary biliary cirrhosis.
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PMID:Complement components and activation in primary biliary cirrhosis. 642 Mar 6

The glycoproteins (GP) of 329 patients with liver diseases and 60 clinically healthy subjects were complexly studied: sialic acid, orozomucoid, haptoglobin, ceruloplasmin, cholinesterase, hexosamine and fucose. Modern laparoscopic, bioscopic, histochemical and histomorphological methods were used in making the diagnosis and determination of the disease phase; The liver diseases are characterized by quantitative and qualitative differences in the character of the GP changes in serum. GP are mostly changed in acute viral hepatitis, cirrhosis, extrahepatic cholestasis and liver tumours, less in chronic aggressive hepatitis and no change in chronic persistent hepatitis and steatosis. The complex GP study is of significance in the characteristic of the activity of the pathological process, in the specification of the liver function as well as for the prognosis of a certain disease.
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PMID:[Complex study of glycoproteins in liver disease]. 710 92

Blood samples of 104 Iranian women aged 17 to 49 years of age were collected during the 2nd half of their menstrual cycle. Serum copper level and ceruloplasmin activity were measured using COC 1 (mestranol 0.15 mg, lynestrol 2.5 mg) for 3 to 108 months. Results were compared with those from 27 normal individuals aged 21 to 45 years of age from the same social class who had never taken OCs (oral contraceptives). All test subjects (100%) exhibited significant increases in serum copper level and ceruloplasmin activity (p 0.005); the increase was equivalent to 11-175% of mean controls (p 0.005). The increase in activity of ceruloplasmin was said to be aggravated by cholestasis and the liver undergoing a physiological stress. After 48 months of OC intake, the observed increases appeared to decline, probably because the liver has adjusted to the physiological stress caused by COC intake. The variations in this study were higher than those obtained in Nigerian women and comparable to those of other countries.
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PMID:Variations in serum copper and ceruloplasmin activity following a long term intake of combined oral contraceptives in Iranian women. 739 23

Wilson's disease-like lesion was seen in a 2-month-old calf. Fibrosis was apparent in the liver, but there was no cholestasis or icterus. Marked spongy degeneration was seen in the central nervous system. Hepatic and brain copper values were significantly higher than normal: 1970 and 113 ppm dry matter, respectively. Serum ceruloplasmin was lower than normal: 17.5 U/liter. Etiology of this disease was unknown, but there is no evidence that the calf ingested a large quantity of copper.
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PMID:Wilson's disease-like lesion in a calf. 857 46

A reciprocal type of the relationship between superoxide dismutase (SOD) and hepatic ceruloplasmin levels in the liver and plasma has been demonstrated. This acts as an integrating mechanism of antioxidant resistance in hepatobiliary diseases. The value of SOD/ceruloplasmin ratio is presented which was low in primary biliary cirrhosis and chronic cholestatic hepatitis. A statistical significance for the differences between biochemical indices of cholestasis (bilirubin, cholesterol, alkaline phosphatase) was less than for SOD/ceruloplasmin ratio. The latter proved more informative in the diagnosis of cholestasis.
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PMID:[A new approach to the diagnosis of cholestasis by the activity of copper-containing enzymes]. 913 6

A 32-year-old man with active Crohn's disease and recurrent small bowel strictures underwent abdominal surgery and was subsequently given total parenteral nutrition (TPN). Severe cholestasis developed and copper was removed from the TPN. Although serum ceruloplasmin levels were within normal limits, 8 weeks after copper removal, he developed pancytopenia. Serum copper levels were severely depressed. Bone marrow biopsy was consistent with copper deficiency; cytoplasmic vacuolization of both myeloid and erythroid precursors, megaloblastic erthropoiesis, and marked hypocellularity were observed. IV replacement with copper sulfate resulted in improvement in the patient's anemia, neutropenia, and thrombocytopenia, but the patient died suddenly from cardiac tamponade. Postmortem examination revealed fibrinous and hemorrhagic pericarditis. Despite the rare occurrence of overt copper deficiency, this case emphasizes the need to recognize copper deficiency as an important etiology of iron-resistant anemia in patients receiving TPN. Furthermore, the relative rapidity with which our patient developed pancytopenia suggests that, in view of the established recommendation that copper be removed from TPN in cholestatic conditions, serum copper levels must be measured periodically.
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PMID:Rapid development of severe copper deficiency in a patient with Crohn's disease receiving parenteral nutrition. 1033 25

Significant increases in serum levels and decreases in hair copper levels have been previously described in epileptic patients treated with anticonvulsant drugs. A condition not directly related to copper nutriture, such as chronic treatment with these drugs, could increase the serum concentrations of copper and ceruloplasmin and would mask a possible copper deficiency produced by drug-increased biliary copper excretion. Serum immunoreactive ceruloplasmin concentration and its oxidase activity were determined in 90 adult epileptic patients treated with phenobarbital (n=60), phenytoin (n=70), carbamazepine (n=33) and valproic acid (n=8). The levels of ceruloplasmin and oxidase activity were significantly higher (P<0.001) than in an age and gender-matched control group (n=49). The significant correlations (P<0.01) between ceruloplasmin and the urinary excretion of D-glucaric acid, serum gamma-glutamyltransferase (GGT) and drug score in the patients group, would suggest that phenobarbital-type enzyme-inducing agents may increase the hepatic synthesis of ceruloplasmin. In 11 patients with a beta-globulin migrating GGT isoform (GGT3), a sensitive marker of cholestasis, the levels of ceruloplasmin, oxidase activity and total GGT activity were significantly higher (P<0.05) than in the group of 79 patients without the GGT3 isoform; consequently, in some cases a drug-induced cholestasis may also contribute to the increase of serum copper and ceruloplasmin. The values obtained for the specific oxidase activity of ceruloplasmin (activity per unit mass of enzyme protein) suggest that in the most of the cases, chronic administration of phenobarbital, phenytoin, carbamazepine or valproic acid, does not produce marginal or moderate copper deficiency.
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PMID:Assessment of copper status in epileptic patients treated with anticonvulsant drugs by measuring the specific oxidase activity of ceruloplasmin. 1464 99


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