A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency

• DOI: 10.1136/jcp.55.2.145
• Publication Date: 2011-04-27T13:21:51Z
• Authors: M M Gompels; R J Lock; J E Morgan; J Osborne; A Brown; P F Virgo
• Keywords: C1-inhibitor

Aim: To determine the diagnostic efficiency of assays routinely used in investigation hereditary angiooedema.Methods: Over a four year period, 1144 samples were received for analysis from 907 patients suspected C1 inhibitor deficiency.Analyses performed C4 and (functional immunochemical).Notes reviewed retrospectively on with low serological indicators to diagnosis.Results: These are first data indicate sensitivity, specificity, predictive values most frequently screen deficiency.A combination function has 98% specificity deficiency this population 96% negative value, is thus very effective screen.All untreated had value.Conclusions: All considered diagnosis should have serum examined measure both functional inhibitor.If either normal at presentation essentially excludes deficiency.These tests can be sequentially.If it not necessary proceed analysis.If then repeat sample obtained confirm findings.. .