Sign in →

Test ID: CUT Copper, Liver Tissue

Reporting Name

Copper, Liver Ts

Useful For

Diagnosing Wilson disease and primary biliary cirrhosis using liver tissue specimens

Specimen Type

Liver Tissue


Specimen Required


Patient Preparation: Gadolinium is known to interfere with most metal tests. If gadolinium-containing contrast media has been administered a specimen should not be collected for 96 hours.

Supplies: Metal Free Specimen Vial (T173)

Container/Tube:

Preferred: Mayo metal-free specimen vial (blue label)

Acceptable: Paraffin block if no more than 1 or 2 cuts have been made to it for slides

Specimen Volume: 2 mg

Collection Instructions:

1. Two mg of liver tissue is required. This is typically a piece of tissue from a 22-gauge needle biopsy at least 2 cm long. If an 18-gauge needle is used, the tissue must be at least 1 cm in length.

2. Any specimen vial other than a Mayo metal-free vial used should be plastic, leached with 10% nitric acid for 2 days, rinsed with redistilled water, and dried in clean air.

Additional Information: Paraffin blocks will be returned 3 days after analysis.


Specimen Minimum Volume

2 cm (22-gauge needle)
1 cm (18-gauge needle)
2 mm x 2 mm (punch) 0.3 mg by dry weight

Specimen Stability Information

Specimen Type Temperature Time Special Container
Liver Tissue Refrigerated (preferred)
  Ambient 
  Frozen 

Reference Values

<50 mcg/g dry weight

Day(s) and Time(s) Performed

Monday, Wednesday, Friday; 11 a.m.

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the U.S. Food and Drug Administration.

CPT Code Information

82525

LOINC Code Information

Test ID Test Order Name Order LOINC Value
CUT Copper, Liver Ts 8198-4

 

Result ID Test Result Name Result LOINC Value
8687 Copper, Liver Ts 8198-4

Clinical Information

Homeostatic regulation of copper metabolism is very complex. The liver is the key organ to facilitate copper storage and incorporation of copper into the transport protein ceruloplasmin. Intestinal absorption and biliary excretion also play major roles in the regulation of copper homeostasis.

 

Abnormal copper metabolism is associated with liver disease. Elevated serum copper concentrations are seen in portal cirrhosis, biliary tract disease, and hepatitis, probably because excess copper that would normally be excreted in the bile is retained in circulation. In primary biliary cirrhosis, ceruloplasmin is high, resulting in high serum copper. Lesser elevations of hepatic copper are found in chronic copper poisoning, obstructive jaundice, and certain cases of hepatic cirrhosis. Reduced serum copper concentration is typical of Wilson disease (hepatolenticular degeneration). Wilson disease is characterized by liver disease, neurologic abnormalities, and psychiatric disturbances. Kayser-Fleischer rings are normally present and urinary copper excretion is increased, while serum copper and ceruloplasmin are low.

Interpretation

The constellation of symptoms associated with Wilson disease (WD), which includes Kayser-Fleischer rings, behavior changes, and liver disease, is commonly associated with liver copper concentration above 250 mcg/g dry weight.

 

VERY HIGH >1000 mcg/g dry weight:

This finding is strongly suggestive of Wilson disease.

 

HIGH 250-1000 mcg/g dry weight:

This finding is suggestive of possible Wilson disease.  

 

MODERATELY HIGH 50-250 mcg/g dry weight:

Excessive copper at this level can be associated with cholestatic liver disease, such as primary biliary cirrhosis, primary sclerosing cholangitis, autoimmune hepatitis, and familial cholestatic syndrome. Heterozygous carriers for Wilson disease occasionally have modestly elevated values, but rarely higher than 125 mcg/g of dry weight. In general, the liver copper content is higher than 250 mcg/g dried tissue in patients with Wilson disease.

 

If any of the above findings are without supporting histology and other biochemical test results, contamination during collection, handling, or processing should be considered. Genetic testing for Wilson disease (WDZ / Wilson Disease, Full Gene Analysis, Varies) is available at Mayo Clinic Laboratories, call 800-533-1710 if you need additional assistance.

 

In patients with elevated levels of copper without supporting histology and other biochemical test results, contamination during collection, handling, or processing should be considered.

Clinical Reference

1. Korman J, Volenberg I, Balko J, et al: Screening for Wilson disease in acute liver failure: a comparison of currently available diagnostic tests. Hepatology. 2008 Oct;48(4):1167-1174

2. Roberts EA, Schlisky ML: Diagnosis and Treatment of Wilson Disease: AASLD Practice Guidelines. Hepatology. 2008;47:2089-2111

3. de Bie P, Muller P, Wijmenga C, Klomp LW: Molecular pathogenesis of Wilson and Menkes disease: correlation of mutations with molecular defects and disease phenotypes. J Med Genet. 2007 November;44(11):673-688

4. Merle U, Schaefer M, Ferenci P, Stremmel W: Clinical presentation, diagnosis and long-term outcome of Wilson's disease: a cohort study. Gut. 2007;56:115-120

5. Rifai N, Horwath AR, Wittwer CT, eds. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. 6th ed. Elsevier; 2018

Analytic Time

2 days

Method Name

Inductively Coupled Plasma-Mass Spectrometry (ICP-MS)

Forms

If not ordering electronically, complete, print, and send a Gastroenterology and Hepatology Client Test Request (T728) with the specimen.

Mayo Clinic Laboratories | Gastroenterology Catalog Additional Information:

mml-gi-liver-genetic