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Test ID: CUS Copper, Serum


Specimen Required


Patient Preparation: High concentrations of gadolinium, iodine, and barium are known to interfere with most metals tests. If gadolinium-, iodine, or barium-containing contrast media has been administered, a specimen should not be collected for at least 96 hours.

Supplies:

-Metal Free B-D Tube (No Additive), 6 mL (T184)

-Metal Free Specimen Vial (T173)

Collection Container/Tube: 6-mL Plain, royal blue-top Vacutainer plastic trace element blood collection tube

Submission Container/Tube: 7-mL Metal-free, screw-capped, polypropylene vial

Specimen Volume: 0.8 mL

Collection Instructions:

1. Allow the specimen to clot for 30 minutes; then centrifuge the specimen to separate serum from the cellular fraction.

2. Remove the stopper. Carefully pour specimen into metal-free, polypropylene vial, avoiding transfer of the cellular components of blood. Do not insert a pipet into the serum to accomplish transfer, and do not ream the specimen with a wooden stick to assist with serum transfer.

3. See Trace Metals Analysis Specimen Collection and Transport in Special Instructions for complete instructions.


Forms

If not ordering electronically, complete, print, and send 1 of the following forms with the specimen:

-General Request (T239)

-Inborn Errors of Metabolism Test Request (T798)

-Gastroenterology and Hepatology Client Test Request (T728)

Useful For

Diagnosis of:

-Wilson disease

-Primary biliary cirrhosis

-Primary sclerosing cholangitis

Method Name

Dynamic Reaction Cell-Inductively Coupled Plasma-Mass Spectrometry (DRC-ICP-MS)

Reporting Name

Copper, S

Specimen Type

Serum

Specimen Minimum Volume

0.2 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 28 days METAL FREE
  Ambient  28 days METAL FREE
  Frozen  28 days METAL FREE

Clinical Information

Copper (Cu) is an important trace element that is associated with a number of metalloproteins. Cu in biological material is complexed with proteins, peptides, and other organic ligands. Up to 90% of Cu exported from the liver into peripheral blood is in the protein bound form either to ceruloplasmin, transcuprein, or metallothionein. A smaller amount of Cu in plasma (<10%) is bound to albumin by specific peptide sequences, and this Cu is in equilibrium with plasma amino acids. The ceruloplasmin molecule contains 6 to 8 atoms of Cu per molecule with 6 atoms of Cu involved in the protein's ferroxidase and free radical scavenging activities. The other 1 to 2 atoms of Cu are termed "labile" and may allow ceruloplasmin to act as a Cu transporter, with a pool of Cu being exchanged between albumin, transcuprein, and the labile sites of ceruloplasmin.

 

Low serum copper, most often due to excess iron or zinc ingestion and infrequently due to dietary copper deficit, results in severe derangement in growth and impaired erythropoiesis. Low serum copper is also observed in hepatolenticular degeneration (Wilson disease) due to a decrease in the synthesis of ceruloplasmin and allelic variances in cellular metal ion transporters. In Wilson disease, the albumin-bound copper may actually be increased, but ceruloplasmin copper is low, resulting in low serum copper. However, during the acute phase of Wilson disease (fulminant hepatic failure), ceruloplasmin and copper may be normal; in this circumstance, hepatic inflammation causes increased release of ceruloplasmin. It is useful to relate the degree of liver inflammation to the ceruloplasmin and copper-see discussion on hypercupremia below. Significant hepatic inflammation with normal ceruloplasmin and copper suggest acute Wilson disease.

 

Other disorders associated with decreased serum copper concentrations include malnutrition, hypoproteinemia, malabsorption, nephrotic syndrome, Menkes disease, copper toxicity, and megadosing of zinc-containing vitamins (zinc interferes with normal copper absorption from the gastrointestinal tract).

 

Hypercupremia is found in primary biliary cirrhosis, primary sclerosing cholangitis, hemochromatosis, malignant diseases (including leukemia), thyrotoxicosis, and various infections. Serum copper concentrations are also elevated in patients taking contraceptives or estrogens and during pregnancy.

 

Since the gastrointestinal (GI) tract effectively excludes excess copper, it is the GI tract that is most affected by copper ingestion. Increased serum concentration does not, by itself, indicate copper toxicity.

Reference Values

0-2 months: 0.40-1.40 mcg/mL

3-6 months: 0.40-1.60 mcg/mL

7-9 months: 0.40-1.70 mcg/mL

10-12 months: 0.80-1.70 mcg/mL

13 months-10 years: 0.80-1.80 mcg/mL

≥11 years: 0.75-1.45 mcg/mL

Interpretation

Serum copper below the normal range is associated with Wilson disease, as well as a variety of other clinical situations (see Clinical Information). Excess use of denture cream containing zinc can cause hypocupremia.

 

Serum concentrations above the normal range are seen in primary biliary cirrhosis and primary sclerosing cholangitis, as well as a variety of other clinical situations (see Clinical Information).

Clinical Reference

1. McCullough AJ, Fleming CR, Thistle JL, et al: Diagnosis of Wilson's disease presenting as fulminant hepatic failure. Gastroenterology. 1983;84:161-167

2. Wiesner RH, LaRusso NF, Ludwig J, Dickson ER: Comparison of the clinicopathologic features of primary sclerosing cholangitis and primary biliary cirrhosis. Gastroenterology. 1985;88:108-114

3. Spain RI, Leist TP, De Sousa EA: When metals compete: a case of copper-deficiency myeloneuropathy and anemia. Nat Clin Pract Neurol. 2009 Feb;5(2):106-111

4. Kale SG, Holmes CS, Goldstein DS, et al: Neonatal Diagnosis and Treatment of Menkes Disease. N Engl J Med. 2008 Feb 7;358(6):605-614

5. Nations SP, Boyer PJ, Love LA, et al: Denture cream: An unusual source of excess zinc, leading to hypocupremia and neurologic disease. Neurology. 2008;71;639-643

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

Day(s) and Time(s) Performed

Monday; 2 p.m.

Tuesday through Friday; 5 p.m.

Saturday; 2 p.m.

Analytic Time

1 day

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
CUS Copper, S 5631-7

 

Result ID Test Result Name Result LOINC Value
8612 Copper, S 5631-7
Mayo Clinic Laboratories | Gastroenterology Catalog Additional Information:

mml-gi-liver-genetic