A single determination of liver copper concentration may misdiagnose Wilson’s disease

2000 ◽  
Vol 33 (7) ◽  
pp. 589-590 ◽  
Author(s):  
Yuh-Min Song ◽  
Ming-Der Chen
Keyword(s):  
Copper Concentration ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Liver Copper ◽  
Single Determination

A comparison of copper-loading disease in Bedlington terriers and Wilson's disease in humans

1982 ◽  
Vol 243 (3) ◽  
pp. G226-G230 ◽  
Author(s):  
L. C. Su ◽  
S. Ravanshad ◽  
C. A. Owen ◽  
J. T. McCall ◽  
P. E. Zollman ◽  
...  
Keyword(s):  
Copper Concentration ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Normal Brain ◽  
Glutamic Pyruvic Transaminase ◽  
Serum Glutamic Pyruvic Transaminase ◽  
Hepatic Copper ◽  
Erythrocyte Survival ◽  
Ceruloplasmin Activity

Eleven Bedlington terriers were found to have a mean hepatic copper concentration of 6,321 micrograms/g dry wt (normal, 200 micrograms/g dry wt) and renal copper concentration that was three or four times normal. Brain copper levels were normal in younger dogs, were elevated in two older dogs, and were 100 times normal in one dog that died of the disease. Increased concentrations of copper in the liver, kidney, and brain also characterize Wilson's disease. Erythrocyte survival was normal in three affected dogs, but serum glutamic-pyruvic transaminase levels were usually elevated. Unlike the hypoceruloplasminemia of patients with Wilson's disease, plasma ceruloplasmin activity was not only normal but was also slightly elevated in the terriers. Despite their normal or excessive ceruloplasmin, the Bedlington terriers could convert ionic 64Cu to radioceruloplasmin but did so only very slowly. These dogs accumulated significantly more 64Cu in their livers than normal, much like patients with Wilson's disease do before symptoms develop.

The ontogeny of liver copper metabolism in the guinea pig: Clues to the etiology of Wilson's disease

Hepatology ◽  
1986 ◽  
Vol 6 (3) ◽  
pp. 427-432 ◽  
Author(s):  
Surjit K. S. Srai ◽  
Andrew K. Burroughs ◽  
Bernard Wood ◽  
Owen Epstein
Keyword(s):  
Guinea Pig ◽  
Wilson’S Disease ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Liver Copper

THE LIVER IN JUVENILE WILSON'S DISEASE

PEDIATRICS ◽  
1962 ◽  
Vol 30 (3) ◽  
pp. 402-413 ◽  
Author(s):  
Mervin Silverberg ◽  
Sydney S. Gellis
Keyword(s):  
Wilson’S Disease ◽  
Punch Biopsy ◽  
Wilson's Disease ◽  
Careful Examination ◽  
Unknown Etiology ◽  
Surgical Biopsy ◽  
Rubeanic Acid ◽  
Reliable Methods ◽  
Determination Of Copper

Twelve cases of juvenile Wilson's disease are reviewed. Eight presented with hepatic manifestations, and six of these showed a predominance of liver symptomatology throughout their entire illness. It appears that this type of onset is not uncommon when the disease begins in the pre-adolescent period. In childhood, chronic liver disease of unknown etiology should always be screened for Wilson's disease. Careful examination for Kayser-Fleischer rings should repeatedly be made. The finding of cupruria, aminoaciduria without glycosuria or albuminuria, and decreased blood uric acid levels are strongly suggestive of Wilson's disease. Punch biopsy of the liver with visualization of copper by means of an improved modification of the histo-chemical rubeanic acid method or Howell's newer histo-chemical method, or determination of copper content as well as histochemical analysis of liver obtained by surgical biopsy appear to be the only reliable methods at present for proof of diagnosis. [See Table III in Source Pdf.]

Morning (First) Urine Copper Concentration: a New Approach for the Diagnosis of Wilson’s Disease

2018 ◽  
Vol 190 (2) ◽  
pp. 283-288 ◽  
Author(s):  
A. K. M. Atique Ullah ◽  
M. A. Maksud ◽  
S. R. Khan ◽  
Shamshad B. Quraishi
Keyword(s):  
Copper Concentration ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
New Approach

Direct determination of Cu isotope ratios in dried urine spots by means of fs-LA-MC-ICPMS. Potential to diagnose Wilson's disease

2013 ◽  
Vol 28 (1) ◽  
pp. 98-106 ◽  
Author(s):  
Martín Resano ◽  
Maite Aramendía ◽  
Luis Rello ◽  
Mª Luisa Calvo ◽  
Sylvain Bérail ◽  
...  
Keyword(s):  
Wilson’S Disease ◽  
Direct Determination ◽  
Isotope Ratios ◽  
Wilson's Disease ◽  
Cu Isotope

Studies with Radioactive Copper (64Cu and 67Cu); The Incorporation of Radioactive Copper into Caeruloplasmin in Wilson's Disease and in Primary Biliary Cirrhosis

1971 ◽  
Vol 41 (3) ◽  
pp. 189-202 ◽  
Author(s):  
Kathleen Gibbs ◽  
J. M. Walshe
Keyword(s):  
Primary Biliary Cirrhosis ◽  
Control Subject ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Turnover Time ◽  
Control Group ◽  
Biliary Cirrhosis ◽  
Liver Copper ◽  
Secondary Rise ◽  
Copper Overload

1. A comparison has been made of the handling of radioactive copper by patients with Wilson's disease, patients with primary biliary cirrhosis and a small control group. The object of this study has been to assess the influence of an expanded hepatic pool of copper on the incorporation of radioactive copper into caeruloplasmin. 2. Studies with 67Cu have shown that radioactivity disappears steadily from the plasma of patients with Wilson's disease for periods up to 296 h. Both in patients with primary biliary cirrhosis and in controls, a well-marked secondary rise occurs as radioactive copper is incorporated into caeruloplasmin. 3. Caeruloplasmin has been isolated from the serum of one control subject, one patient with primary biliary cirrhosis and one with Wilson's disease. Of the plasma radioactivity 90% was present in the caeruloplasmin of the control subject and the patient with primary biliary cirrhosis; only 27% was found in the caeruloplasmin of the patient with Wilson's disease. 4. Turnover time for liver copper has been determined; in the control subjects it was of the order of 20–30 days, in the patients with primary biliary cirrhosis it was between 600 and 700 days, in Wilson's disease the time exceeded 1800 days. In all cases the turnover time for radioactive copper was significantly less. 5. We conclude (a) that newly arrived radioactive copper is preferentially handled in the presence of copper overload and (b) dilution of radioactive copper in an expanded hepatic pool of the metal cannot alone account for the delayed incorporation found in patients with Wilson's disease.

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