Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:1.16.3.1 (ceruloplasmin)
 5,074 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The accuracy of the serum ceruloplasmin level in distinguishing chronic active hepatitis from Wilson disease was compared to the 24-hour urinary copper excretion and hepatic copper content in 20 untreated patients with chronic active hepatitis and 25 with Wilson disease. Serum ceruloplasmin levels were decreased in five patients (25%) with chronic active hepatitis and were normal in seven patients (28%) with Wilson disease at the time of diagnosis. The 24-hour urinary copper excretion failed to provide accurate discrimination between the two groups, being elevated in all patients with Wilson disease and in five of eight patients with chronic active hepatitis studied. All patients with Wilson disease had hepatic copper levels greater than 400 microgram/gm dry weight, whereas patients with chronic active hepatitis had levels less than 300 microgram/gm dry weight. The discriminatory value of hepatic copper concentration makes this the most reliable test for differentiating chronic active hepatitis and Wilson disease in children and adolescents. The serum ceruloplasmin level may not be significantly accurate for definitive diagnosis in this age group.
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PMID:Laboratory measures of copper metabolism in the differentiation of chronic active hepatitis and Wilson disease in children. 43 Feb 91

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

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

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

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

To investigate the diagnostic value of 24-hr urinary copper excretion testing after penicillamine challenge in the diagnosis of Wilson's disease, 75 consecutive children referred for a variety of liver problems and in whom parameters of copper metabolism had been investigated were analyzed retrospectively. Seventeen had Wilson's disease, 22 had autoimmune chronic active hepatitis, 6 had primary sclerosing cholangitis, 12 had chronic liver disease of various etiologies, 4 had cryptogenic acute liver failure, 6 had acute hepatitic illnesses and 8 had a variety of disorders featuring normal liver histological appearance. Serum ceruloplasmin and total copper levels were significantly lower in Wilson's disease patients compared with all other groups, but three children with Wilson's disease had normal ceruloplasmin levels and seven had normal total copper levels. No significant difference was found for free serum copper levels and liver copper content between Wilson's disease patients and the other groups. Baseline 24-hr urinary copper excretion was significantly higher in Wilson's disease patients compared with that of the other patients, but six children with Wilson's disease had levels just above the upper limit of normal, overlapping with values obtained in three children with liver failure, two with acute hepatitis, two with autoimmune chronic active hepatitis and three with primary sclerosing cholangitis. The 24-hr urinary copper excretion after penicillamine challenge proved the most accurate single diagnostic test; levels more than 25 mumol/24 hr were present in 15 of 17 patients with Wilson's disease, but in only 1 child with liver failure of the 58 with other disorders.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Value of urinary copper excretion after penicillamine challenge in the diagnosis of Wilson's disease. 155 38

Evaluation of chronic liver disease begins with a carefully taken history, thorough physical examination, and standard laboratory tests. Often, however, other studies are required, such as a viral hepatitis panel, serologic tests for autoimmune markers, tests for antimitochondrial antibodies, measurement of serum iron and ceruloplasmin levels, liver biopsy, and imaging studies of the extra-hepatic bile ducts. Medical treatment of chronic active hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis remains unsatisfactory. Early treatment of hemochromatosis and Wilson's disease can prevent cirrhosis and liver failure. Liver transplantation is now a viable procedure for patients with end-stage chronic liver disease.
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PMID:Chronic liver disease. The scope of causes and treatments. 158 71

Caeruloplasmin is an alpha 2 protein produced in the liver that is responsible for transporting copper in the blood. Caeruloplasmin values are usually high in patients with chronic liver diseases, including chronic active hepatitis: low values, however, are characteristic of Wilson's disease. The case of a 17 year old woman with very low caeruloplasmin values and chronic active hepatitis of the lupoid type is described. Steroid treatment resulted in an increase in the caeruloplasmin concentration and clinical improvement.
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PMID:Undetectable caeruloplasmin values in a patient with autoimmune chronic active hepatitis. 204 Apr 80

The author's 25 years experience in clinical, diagnostic, therapeutic and prognostic study of 24 patients with Wilson-Konovalov's disease (hepatilenticular degeneration) is presented. The great diagnostic importance of copper metabolism (plasma copper level, urine copper), plasma ceruloplasmin concentration, histochemical and quantitative determination of copper in liver cells (after a liver biopsy) is pointed out. These are of special importance in young patients (under 25 years) and in children with chronic parenchymal liver disease. In 1/4 of the patients the early stage of the disease resembled chronic active hepatitis (predominantly liver type of the disease). The basic therapeutic drug is D-penicillamine hydrochloride and when it is not tolerated the drug Trien may be used. Very good therapeutic results and considerable improvement of prognosis, social and working rehabilitation of the patients have been achieved. The preconditions for good therapeutic and prognostic results are the early discovery, exact diagnosis and prolonged purposeful pathogenetic treatment (chelatory and hepatoprotective). Zinc sulfate and potassium sulfide are only secondary therapeutic means in patients with hepatolenticular degeneration.
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PMID:[Clinico-diagnostic, therapeutic and prognostic studies of Wilson-Konovalov disease. The results of 25 years' experience]. 367 34

Hepatic copper content, measured by neutron activation analysis, has been studied in 70 patients during a median observation period of 3 years. In 15 patients with chronic active hepatitis the median hepatic copper content was 39 micrograms/g dry weight at the start and 28 micrograms/g dry weight at the end of the observation period, during which the clinical condition was improved in most patients. The hepatic copper content did not change significantly (41 versus 54 micrograms/g dry weight) in 28 patients with hepatobiliary disorders associated with inflammatory bowel disease. In 27 patients with primary biliary cirrhosis the median hepatic copper concentration was initially 176 micrograms/g dry weight, and at the follow up 186 micrograms/g dry weight. Serum ceruloplasmin was elevated in most of the patients with hepatobiliary disorders associated with inflammatory bowel disease and in all the patients with primary biliary cirrhosis and remained at high levels throughout the observation period. In primary biliary cirrhosis the hepatic copper content was correlated with bilirubin and alkaline phosphatases but not with ceruloplasmin, coagulation factors, or aminotransferases. Ceruloplasmin was not significantly correlated with other "liver tests". We conclude that the hepatic copper content and serum ceruloplasmin were remarkably constant during 3 years of observation in various liver disorders. Our study gave no evidence of a hepatotoxic effect of copper, since no relation between the initial hepatic copper concentration and the clinical course could be detected.
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PMID:A long-term follow-up study of the hepatic copper and serum ceruloplasmin concentrations in patients with chronic liver disease. 713 82


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