Name |
C1 Esterase Inhibitor (Level) |
Alternative name/Profile |
Hereditory Angioedema, CI, C1q, C1 Inhibitor Function, CIF |
Department |
Immunology |
Investigation |
Turbidimetry (Binding Site, SPAplus) |
Specimen type |
Clotted blood |
Sample type |
Serum |
Sample container & volume |
5ml Red |
Frequency of analysis |
Once weekly |
Turnaround time |
10 days |
Availability |
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Notes |
Useful for:
- Diagnosing Hereditory Angioedema.
- Monitoring C1 Esterase Inhibitor levels during therapy.
Clinical Information:
- C1 Esterase Inhibitor inhibits C1 activation in the classical pathway of the complement cascade.
- A C1 Esterase Inhibitor deficiency results in inappropriate C1 activation leading to C2 and C4 breakdown. This can result in acute oedema of subcutaneous tissue, the gastrointestinal tract and/or the upper respiratory track.
- A C1 Esterase Inhibitor deficiency can be acquired or hereditory.
- The hereditory deficiency is an autosomal dominant condition.
Please Note:
- As 15% of hereditory C1 Esterase Inhibitor deficiencies are due to a non-functional C1 Esterase Inhibitor protein, a functional C1 Esterase Inhibitor assay my be required to diagnose these patients.
- To distinguish between hereditory and acquired C1 Esterase Inhibitor deficiency, C1q levels are required. Hereditory deficiencies will have normal C1q levels while acquired deficiencies will have low C1q levels.
- Where External Institiutions cannot guarantee immediate delivery, samples must be centrifuged, separated and stored at 4oC. Samples can then be transported, as soon as possible, to St. James's Hospital for processing.
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Related links |
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Reference range |
0.15-0.43 g/L
Reference range was sourced from the Protein Reference Unit (PRU), Sheffield. |
Last updated |
Tue, 09 Jun 2020 15:43:33 IST |
