Synonyms

Alpha2-neuroaminoglycoprotein

Clinical Indications

Diagnosis of hereditary angioedema (HAE). C1 esterase inhibitor deficiency is the commonest of the inherited complement deficiencies (approx. 1:50,000) and is autosomal dominant. Several members of a family are usually affected.

Test Includes

Functional C1 esterase inhibitor, C3 and C4 levels.

Request Form

Combined Pathology Blood form (Yellow/Black)

Availability

Analysed by referral laboratory if specific criteria met.

Specific Criteria

Investigation of hereditary angioedema. Usually C1 esterase inhibitor analysis will only be undetaken C4 levels are low with normal C3 levels.
The request form should show:
- Site, duration, frequency and clinical nature of attacks
- Details of relatives similarly affected

Turnaround Time

2 weeks

Specimen

Serum

Volume

2 ml

Container

Yellow top (SST) tube

Lab. Handling

Aliquot and store at 4C. Samples should be posted as soon as possible. (C1 & send; NOC1 & save)

Causes for Rejection

Inadequate clinical information.

Reference Range

Adult: 0.15 - 0.35 g/L (functional assay 75 - 125% of normal)