title: [Current diagnostic possibilities in hereditary angioedema and acquired angioedema].
abstract: Amidolytic assays for the determination of C1 esterase inhibitor have been proposed some years ago; however, the substrates employed lacked sensitivity. The recent development of a C1 esterase-sensitive substrate (N-alpha-methoxycarbonyl-l-lysyl(epsilon-CBO)-glycyl-arginyl-4-nit roanilide) provides a method that can be routinely used. Any interferences with other plasma proteases could not be observed. Normal range was ascertained to be 1.48 +/- 0.23 kU/l; within-run precision revealed C.V.'s of 1.74% (for the normal range) and 7.3% (for the pathological range). This method can be considered most suitable for functional determination of C1 esterase inhibitor and is superior to the determination of antikallikrein activity. To point out to the diagnostic relevance of a functional C1 esterase inhibitor assay some examples are illustrated.
mesh_terms: D000799:Angioedema / Q000175:diagnosis* / Q000235:genetics; D003166:Complement Activating Enzymes / Q000032:analysis; D003174:Complement C1 Inactivator Proteins / Q000097:blood*; D015922:Complement C1q; D003173:Complement C1s / Q000378:metabolism; D003181:Complement C4 / Q000032:analysis; D003165:Complement System Proteins / Q000032:analysis; D003937:Diagnosis, Differential; D006801:Humans; D005779:Immunodiffusion; D007610:Kallikreins / Q000037:antagonists & inhibitors; D009842:Oligopeptides / Q000378:metabolism; D013379:Substrate Specificity
chemical_list: D003174:Complement C1 Inactivator Proteins; D003181:Complement C4; C046044:N-methyloxycarbonyl-epsilon-lysyl-glycyl-arginine-4-nitroanilide; D009842:Oligopeptides; D015922:Complement C1q; D003165:Complement System Proteins; D003166:Complement Activating Enzymes; D007610:Kallikreins; D003173:Complement C1s
vernacular_title: Gegenwärtige diagnostische Möglichkeiten beim hereditären Angioödem (HAE) und beim erworbenen Angioödem (AAE).