Copper, Serum or Plasma

CPT: 82525
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Test Details

Use

It is used, along with serum ceruloplasmin and urine copper, to test for Wilson's disease and (more often) in monitoring the nutritional adequacy of parenteral or enteral nutrition, especially when copper deficiency may be suspected because of ongoing gastrointestinal losses of the element (see table). The test is done in suspected copper toxicity in premature infants when they are acutely ill and may not be able to assimilate the copper in their prescribed nutrition; in acute copper intoxications; or in “Indian childhood cirrhosis,” an illness not limited to Indian children.1 Serum copper is low in Menkes syndrome. Copper in the CSF is reported to mirror the neurotoxicity of copper in Wilson's disease.2 Liver copper is used to confirm Wilson's disease and Menkes syndrome and may be measured in liver disease of uncertain etiology. It can confirm ICC in the right setting. Liver copper rises with time in biliary cirrhosis, but does not confirm the diagnosis.

Copper, Serum or Plasma

Deficiency, Nutritional

Menkes Syndrome

Acute Copper Toxicity

ICC and Chronic Copper Toxicity

Wilson's Disease

Smoking, Inflammatory Conditions, Pregnancy, Estrogens

N = normal, ↑ = increase, ↑↑ = large increase, ↓ = decrease.

Serum copper

↑, ↑↑

N or ↓

↑, ↑↑

Serum ceruloplasmin

N

(early)

Usually ↓; may be N in children

↑, ↑↑

Urine copper

↑, ↑↑

N

CSF copper

N or ↑

N

Liver copper

N

(early)

↑, ↑↑

↑↑

N

Limitations

Serum ceruloplasmin is an acute-phase reactant type protein, and since it binds a large portion of serum copper, both serum copper and ceruloplasmin increase under the influence of inflammatory conditions and estrogen. Serum copper is, therefore, elevated in pregnancy, in patients on estrogens and estrogen-containing contraceptive drugs, in rheumatoid arthritis, and a number of other pathologic entities. It may be low with low serum proteins as in nephrosis, malabsorption, and malnutrition without necessarily reflecting inadequate liver copper stores. It is reduced under the influence of ACTH or glucocorticoids, or valproate3 therapy. Although serum copper levels are usually ordered to work up possible cases of Wilson's disease, Menkes syndrome, and ICC, serum copper alone is of only limited value. Elevations in liver tissue copper are found in Wilson's disease but may occur also in other types of liver disease, especially in primary biliary cirrhosis.4

This test was developed and its performance characteristics determined by LabCorp. It has not been cleared or approved by the Food and Drug Administration.

Methodology

Inductively-coupled plasma/mass spectrometry (ICP/MS)

Reference Interval

Environmental exposure: 72−166 μg/dL. Levels may be somewhat higher in pregnant women and children and in patients receiving estrogen therapy.

Additional Information

The demand for sensitive noninvasive tests for Wilson's disease, especially for children in families where the disease is known to occur, has stimulated search for newer indices of copper metabolism. Urine copper after penicillamine load has recently been proposed.5

Specimen Requirements

Specimen

Serum or plasma

Volume

1 mL

Minimum Volume

0.2 mL

Container

Royal blue-top (EDTA) tube or royal blue-top without EDTA

Collection

Serum must be separated from cells within 45 minutes of collection and transferred to a certified metal-free plastic transport tube (PeopleSoft N° 111166). Plasma may be separated immediately and transferred to a certified metal-free transport tube (PeopleSoft N° 111166) for shipment to the laboratory.

Storage Instructions

Maintain specimen at room temperature.

Stability Requirements

Temperature

Period

Room temperature

14 days

Refrigerated

14 days

Frozen

14 days

Freeze/thaw cycles

Stable x3

Causes for Rejection

Certified metal-free plastic transport tube not submitted

Clinical Information

Footnotes

  • 1. Weiss M, Müller-Höcker J, Wiebecke B, et al. First description of "Indian childhood cirrhosis” in a non-Indian infant in Europe. Acta Paediatr Scand. 1989 Jan; 78(1):152-156. 2919520
  • 2. Weisner B, Hartard C, Dieu C. CSF copper concentration: A new parameter for diagnosis and monitoring therapy of Wilson's disease with cerebral manifestation. J Neurol Sci. 1987; 79(1-2):229-237. 3612170
  • 3. Kaji M, Ito M, Okuno T, et al. Serum copper and zinc levels in epileptic children with valproate treatment. Epilepsia. 1992; 33(3):555-557. 1592036
  • 4. Nadal D, Baerlocher K. Menkes' disease: Long-term treatment with copper and D-penicillamine. Eur J Pediatr. 1988; 147(6):621-625. 3181204
  • 5. Martins da Costa C, Baldwin D, Portmann B, et al. Value of urinary copper excretion after penicillamine challenge in the diagnosis of Wilson's disease. Hepatology. 1992 Apr; 15(4):609-615. 1551638

References

A unified hypothesis of copper transport and uptake. Nutr Rev. 1988 Sep; 46(9):332-333. 3067152
Clayton BE. Clinical chemistry of trace elements. Adv Clin Chem. 1980; 21:147-176. 6771970
Danks DM. Copper deficiency in humans. Annu Rev Nutr. 1988; 8:235-257. 3060166
Danks DM. Disorders of copper transport. In Scriver CR, Beaudet AL, Sly WS, et al, eds.The Metabolic Basis of Inherited Disease. 6th ed. New York, NY: McGraw-Hill Information Services Co; 1989:1411-1431.
Davidoff GN, Votaw ML, Coon WW, et al. Elevations in serum copper, erythrocytic copper, and ceruloplasmin concentrations in smokers. Am J Clin Pathol. 1978 Nov; 70(5):790-792. 717285
Pereira GR, Zucker AH. Nutritional deficiencies in the neonate. Clin Perinatol. 1986; 13(1):175-189. 3514049
Prasad AS. Trace elements: Biochemical and clinical effects of zinc and copper. Am J Hematol. 1979; 6(1):77-87. 453197
Scheinberg IH. Wilson's disease and the physiological chemistry of copper. Inorg Chem Biol Med. 1980; 21:373-380.
Wachnik A. The physiological role of copper and the problems of copper nutritional deficiency. Nahrung. 1988; 32(8):755-765. 3068548
Youssef AAR, Wood B, Baron DN. Serum copper: A marker of disease activity in rheumatoid arthritis. J Clin Pathol. 1983; 36(1):14-17.
Yuzbasiyan-Gurkan V, Johnson V, Brewer GJ. Diagnosis and characterization of presymptomatic patients with Wilson's disease and the use of molecular genetics to aid in the diagnosis. J Lab Clin Med. 1991; 118(5):458-465. 1940586

LOINC® Map

Order Code Order Code Name Order Loinc Result Code Result Code Name UofM Result LOINC
001586 Copper, Serum 5631-7 001586 Copper, Serum ug/dL 5631-7

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