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Clear
 
Test Code:
090-31-4013

Order Name:
C1 Esterase (C1ES) Inhibitor Antigen, Serum **

 
Useful For:

1. Diagnosis of Hereditary angioedema.

2. Monitoring C1 esterase inhibitor levels in response to therapy

 
Methodology:
Nephelometry
 
AliasesName:
C1 Esterase Inhibitor Antigen, S
C1 Esterase Inhibitor Quantitative
C1 inhibitor level
 
 
 
Test Code:
090-31-4013

Order Name:
C1 Esterase (C1ES) Inhibitor Antigen, 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:
Lipemic - Reject

 
 
Specimen Stabillity:
Specimen Type Temperature Time
Serum Frozen (preferred) 28 days
  Refrigerated 28 days
  Ambient 72 hours

 
 
 
 
Test Code:
090-31-4013

Order Name:
C1 Esterase (C1ES) Inhibitor Antigen, Serum **

 
Method detail:
Nephelometry
 
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:
090-31-4013

Order Name:
C1 Esterase (C1ES) Inhibitor Antigen, Serum **

 
 
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

 
Reference Value:
19-37 mg/dL
 
Interpretation:

Abnormally low results are consistent with a heterozygous C1 esterase inhibitor deficiency and hereditary angioedema.
Fifteen percent of hereditary angioedema patients have a normal or elevated level but nonfunctional C1 esterase inhibitor protein. Detection of these patients requires a functional measurement of C1 esterase inhibitor; FC1EQ / C1 Esterase Inhibitor, Functional Assay, Serum.
Measurement of C1q antigen levels; C1Q / Complement C1q, Serum, is key to the differential diagnoses of acquired or hereditary angioedema. Those patients with the hereditary form of the disease will have normal levels of C1q, while those with the acquired form of the disease will have low levels.
Studies in children show that adult levels of C1 inhibitor are reached by 6 months of age.

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