Investigation of patients with possible Wilson disease
For information see Wilson Disease Testing Algorithm.
Nephelometric Assay
Copper Oxidase
Ferroxidase
Kayser-Fleischer Ring
Wilson's Disease
For information see Wilson Disease Testing Algorithm.
Serum
Patient Preparation: Patient should be fasting: 4 hours preferred, nonfasting acceptable
Collection Container/Tube:
Preferred: Serum gel
Acceptable: Red top
Submission Container/Tube: Plastic vial
Specimen Volume: 1 mL
Collection Instructions: Centrifuge and aliquot serum into plastic vial.
If not ordering electronically, complete, print, and send 1 of the following forms with the specimen:
-Benign Hematology Test Request (T755)
-General Request (T239)
0.5 mL
Gross hemolysis | Reject |
Gross lipemia | Reject |
Gross icterus | Reject |
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Serum | Refrigerated (preferred) | 7 days | |
Frozen | 30 days |
Investigation of patients with possible Wilson disease
For information see Wilson Disease Testing Algorithm.
Ceruloplasmin is a positive acute-phase reactant and a copper-binding protein that accounts for over 95% of serum copper in normal adults. Ceruloplasmin is measured primarily to assist with a diagnosis of Wilson disease. Other indications include Menkes disease, dietary copper insufficiency, and risk of cardiovascular disease.
Wilson disease is a rare inherited disorder of copper transport that results in low serum copper and ceruloplasmin and accumulation of copper in various tissues. The pathological accumulation of copper in the liver, brain, cornea, and kidney causes cirrhosis, neuropsychiatric symptoms, Kayser-Fleischer rings, and hematuria/proteinuria, respectively. See Wilson Disease Testing Algorithm for appropriate use of clinical findings, serum biomarkers, genetic tests, and tissue biopsies when working up suspected cases.
Menkes disease is an X-linked disorder in which dietary copper is absorbed from the gastrointestinal tract but cannot be transported, so copper is not available to the liver for incorporation into ceruloplasmin.
Dietary ceruloplasmin deficiency may be due to inadequate dietary copper intake, long-term parenteral nutrition without copper supplementation, malabsorption, penicillamine therapy, or a combination of these.
Males:
0-8 weeks: 7.4-23.7 mg/dL
9 weeks-5 months: 13.5-32.9 mg/dL
6-11 months: 13.7-38.9 mg/dL
12 months-7 years: 21.7-43.3 mg/dL
8-13 years: 20.5-40.2 mg/dL
14-17 years: 17.0-34.8 mg/dL
> or =18 years: 19.0-31.0 mg/dL
Females:
0-8 weeks: 7.4-23.7 mg/dL
9 weeks-5 months: 13.5-32.9 mg/dL
6-11 months: 13.7-38.9 mg/dL
12 months-7 years: 21.7-43.3 mg/dL
8-13 years: 20.5-40.2 mg/dL
14-17 years: 20.8-43.2 mg/dL
> or =18 years: 20.0-51.0 mg/dL
Low concentrations of ceruloplasmin are consistent with Wilson disease and warrant further investigation according to the recommended algorithm; see Wilson Disease Testing Algorithm.
Ceruloplasmin is a positive acute-phase reactant. Increases in serum ceruloplasmin have been reported during pregnancy, in women taking oral contraceptives, in hepatitis, pneumonia, tuberculosis, rheumatoid arthritis, myocardial infarction, various forms of anemia, and many obscure neurological disorders.
Ceruloplasmin is a positive acute-phase reactant; therefore, levels are elevated in cases of inflammation (as in chronic hepatitis or active infection). Consequently, ceruloplasmin levels are not always extremely low in patients with Wilson disease.
Values vary considerably from patient to patient and may be in the normal range in some patients with Wilson disease (indicating a different primary defect).
Birth control pills and pregnancy increase ceruloplasmin levels.
1. Wilson Tang WH, Wu Y, Hartiala J, et al: Clinical and genetic association of serum ceruloplasmin with cardiovascular risk. Arterioscler Thromb Vasc Biol. 2012 Feb;32(2):516-522
2. Dadu RT, Dodge R, Nambi V, et al: Ceruloplasmin and heart failure in the Atherosclerosis Risk in Communities study. Circ Heart Fail. 2013 Sep 1;6(5):936-943
3. Cox DW, Tumer Z, Roberts EA: Copper transport disorders: Wilson's disease and Menkes disease. Inborn Metabolic Disease. Fernandes J, Sandubray JM, VandenBerghe F, eds. Springer-Verlag; 2000:385-391
4. Sontakke AN, More U: Changes in serum ceruloplasmin levels with commonly used methods of contraception. Indian J Clin Biochem. 2004 Jan:19(1):102-104
5. Schilsky ML: Wilson disease: Diagnosis, treatment, and follow-up. Clin Liver Dis. 2017 Nov;21(4):755-767
6. Hermann W: Classification and differential diagnosis of Wilson's disease. Ann Transl Med. 2019 Apr;7(Suppl 2):S63
Human ceruloplasmin forms a precipitate with a specific antiserum, which is then measured nephelometrically.(Package insert: Ceruloplasmin. Siemens; 08/2018)
Monday through Sunday
This test has been cleared, approved, or is exempt by the US Food and Drug Administration and is used per manufacturer's instructions. Performance characteristics were verified by Mayo Clinic in a manner consistent with CLIA requirements.
82390
Test Id | Test Order Name | Order LOINC Value |
---|---|---|
CERS | Ceruloplasmin, S | 2064-4 |
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.
|
---|---|---|
CERS | Ceruloplasmin, S | 2064-4 |