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ID:56 C1 Esterase Inhibitor
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DescriptionImmunochemical levels C1 esterase inhibitor are assessed along with complement C3 and C4. If results indicate, a functional assay will be performed with an appropriate sample.
IndicationInvestigation of anginoneurotic / hereditary/ aquired angioedema. Recurrent episodes of nonpruritic, nonpitting, subcutaneous or submucosal oedema typically involving the arms, legs, hands, feet, bowels, genitalia, trunk, face, tongue, or larynx.
Additional InfoC1 esterase inhibitor deficiency is transmitted as an autosomal dominant disorder resulting in Hereditary Angioedema (HAE). A rare, acquired form (AAE) may occur with lymphoproliferative disease and Autoimmune disorders.
Concurrent TestsComplement C3 and C4
Dietary Requirementsna
InterpretationTwo inherited forms exist: in classic Type 1 deficiency C1 esterase levels are low whereas in the less common Type 2 deficiency C1 esterase is produced but is not functional. Samples collected during an acute attack of angioedema due to C1 inhibitor deficiency have a very low C4 level and thus a normal C4 virtually excludes the condition.

DepartmentClinical Immunology
TubeSerum Gel
Tube Picture
Collection ConditionsNo restrictions however the functional assay requires a serum sample frozen within one hour of collection.
Min. Vol2 mL

Ref. Range (Male)See note
Ref. Range (Female)
Ref. Range (Paed)
Ref. Range NotesAntigenic assay 0.15 - 0.35 g/L.
IP Acute TAT- Contact Laboratory
IP Routine TAT7 days
GP Acute TAT- Contact Laboratory
GP Routine TAT7 days
Turnround CommentNA

Originally edited by : BD. Review due on 14/09/2018 14:24:15. Published By AM on 14/09/2017 14:24:15.