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Test Code C1ES C1 Esterase Inhibitor Antigen, Serum

Reporting Name

C1 Esterase Inhibitor Antigen, S

Useful For

Diagnosis of hereditary angioedema

 

Monitoring levels of C1 esterase inhibitor in response to therapy

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Specimen Type

Serum


Specimen Required


Patient Preparations: Fasting preferred but not required.

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 1 mL

Collection Instructions:

1. Immediately after specimen collection, place the tube on wet ice.

2. Centrifuge and aliquot serum into plastic vial.

3. Freeze specimen within 30 minutes.


Specimen Minimum Volume

0.5 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Frozen (preferred) 28 days
  Refrigerated  28 days
  Ambient  72 hours

Reference Values

19-37 mg/dL

Day(s) Performed

Monday through Friday

Test Classification

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.

CPT Code Information

83883

Clinical Information

C1 esterase inhibitor blocks the activation of C1 (first component of the complement cascade) to its active form. The deficiency of C1 esterase inhibitor results in the inappropriate activation of C1 and the subsequent release of an activation peptide from C2 with kinin-like activity. This kinin-like peptide enhances vascular permeability. C1 esterase inhibitor deficiency results in hereditary or acquired angioedema. This disease is an autosomal dominant inherited condition, in which exhaustion of the abnormally low levels of C1 esterase inhibitor results in C1 activation, breakdown of C2 and C4, and subsequent acute edema of subcutaneous tissue, the gastrointestinal tract, or the upper respiratory tract. The disease responds to attenuated androgens.

 

Because 15% of C1 inhibitor deficiencies have nonfunctional protein, some patients will have abnormal functional results (FC1EQ / C1 Esterase Inhibitor, Functional Assay, Serum) in the presence of normal (or elevated) antigen levels.

Report Available

2 to 5 days

Reject Due To

Gross hemolysis OK
Gross lipemia Reject
Gross icterus OK

Method Name

Nephelometry