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Clear
 
Test Code:
090-31-4014

Order Name:
C1 Esterase Inhibitor, Functional Assay, Serum **

 
Useful For:

1. Diagnosing hereditary angioedema
2. Monitoring response to therapy

 
Methodology:
Enzyme Immunoassay (EIA)
 
AliasesName:
C1 Esterase Inh,Func (QN)
Functional C'1 Esterase
Hereditary Angioedema Panel (3 tests)
Hereditary Angioedema, HAE
 
 
 
Test Code:
090-31-4014

Order Name:
C1 Esterase Inhibitor, Functional Assay, Serum **

 
Patient Preparation:
Patient should be fasting 8-12 hours. 
 
Collection Specimen Or Container:
Blood/ Plain Blood (Red Top) 5 mL, 1 tube

 
 
Specimen Testing Type:
Serum, minimum volume 3-5 mL (Ship Frozen)
 
Sub Mission Container:
Plastic tube
 
Rejection Criteria:
Icteric - Reject

 
 
Specimen Stabillity:
Specimen Type Temperature Time
Serum Frozen 28 days

 
 
 
 
Test Code:
090-31-4014

Order Name:
C1 Esterase Inhibitor, Functional Assay, Serum **

 
Method detail:
Enzyme Immunoassay (EIA)
 
Schedule:
N/A **Sent out to MAYO, USA
 
Turnaround Time:
Received specimen to reported within 14 days
 
 
Performing Location:
MAYO Laboratory
Referral Lab Services, Laboratory Department 14160-2
 
 
 
Test Code:
090-31-4014

Order Name:
C1 Esterase Inhibitor, Functional Assay, Serum **

 
 
Clinical Information:

C1 inhibitor (C1-INH) is a multispecific protease inhibitor that is present in normal human plasma and serum, and which regulates enzymes of the complement, coagulation, fibrinolytic, and kinin-forming systems. The enzymes (proteases) regulated by this protein include the C1r and C1s subunits of the activated first component of complement, activated Hageman factor (factor XIa), kallikrein (Fletcher factor), and plasmin.
A deficiency of functionally active C1-INH may lead to life-threatening angioedema. Two major forms of C1-INH deficiency have been reported: the congenital form, termed hereditary angioedema (HAE), and the acquired form that is associated with a variety of diseases, including lymphoid malignancies.
HAE is characterized by transient but recurrent attacks of nonpruritic swelling of various tissues throughout the body. The symptomatology depends upon the organs involved. Intestinal attacks lead to a diversity of symptoms including pain, cramps, vomiting, and diarrhea. The most frequent cause of death in this disease is airway obstruction secondary to laryngeal edema occurring during an attack. There are 2 types of HAE that can be distinguished biochemically. Patients with the more common type (85% of HAE patients) have low levels of functional C1-INH and C1-INH antigen. Patients with the second form (15% of HAE patients) have low levels of functional C1-INH but normal or increased levels of C1-INH antigen that is dysfunctional.
The variable nature of the symptoms at different time periods during the course of the disease makes it difficult to make a definitive diagnosis based solely on clinical observation.

 
Reference Value:

>67% normal (Normal)

41-67% normal (Equivocal)

<41% normal (Abnormal)

 
Interpretation:

Hereditary angioedema (HAE) can be definitely diagnosed by laboratory tests demonstrating a marked reduction in C1 inhibitor (C1-INH) antigen or abnormally low functional C1-INH levels in a patient's plasma or serum that has normal or elevated antigen.
Nonfunctional results are consistent with HAE.
Patients with current attacks will also have low C2 and C4 levels due to C1 activation and complement consumption.
Patients with acquired C1-INH deficiency have a low C1q in addition to low C1-INH.

 
Clinical Reference:
www.mayomedicallaboratories.com (Retrieved: 24 Jun 2020)