-Primary biliary cholangitis
-Primary sclerosing cholangitis
Dynamic Reaction Cell-Inductively Coupled Plasma-Mass Spectrometry (DRC-ICP-MS)
Kaiser Fleischer Ring
Patient Preparation: High concentrations of gadolinium, iodine, and barium are known to interfere with most metal 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 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
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 for complete instructions.
If not ordering electronically, complete, print, and send General Test Request (T239) with the specimen.
|Specimen Type||Temperature||Time||Special Container|
|Serum||Refrigerated (preferred)||28 days||METAL FREE|
|Ambient||28 days||METAL FREE|
|Frozen||28 days||METAL FREE|
-Primary biliary cholangitis
-Primary sclerosing cholangitis
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 protein bound 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-bound copper is low, resulting in low serum copper. However, during the acute phase of Wilson disease (fulminant hepatic failure), ceruloplasmin and copper levels 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 [GI] tract).
Hypercupremia is found in primary biliary cholangitis (formerly 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 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.
0-2 months: 40-140 mcg/dL
3-6 months: 40-160 mcg/dL
7-9 months: 40-170 mcg/dL
10-12 months: 80-170 mcg/dL
13 months-10 years: 80-180 mcg/dL
11-17 years: 75-145 mcg/dL
> or =18 years: 73-129 mcg/dL
> or =18 years: 77-206 mcg/dL
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).
No significant cautionary statements
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. Strathmann FG, Blum LM: Toxic elements. In: Rifai N, Chiu RWK, Young I, Burnham CAD, Wittwer CT, eds. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. 7th ed. Elsevier; 2023:chap 44
Copper in serum is analyzed by inductively-coupled plasma mass spectrometry in dynamic reaction cell mode using gallium as an internal standard and a salt matrix calibration.(Unpublished Mayo method)
Monday through Saturday
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 US Food and Drug Administration.
|Test Id||Test Order Name||Order LOINC Value|
|Result Id||Test Result Name||
Result LOINC Value
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|Change Type||Effective Date|