[Angioneurotic edema in hereditary alpha-1-antitrypsin deficiency].
A young man with angioedema presented with normal concentration and function of C1-esterase inhibitor, but with a hereditary deficiency of alpha-1 antitrypsin (Pi-ZZ type). The pathogenetic relevance of this defect is discussed with respect to the partial deficiency of the complement component C4 (C4BQ0), which was found in addition.
Vaith
P
P
Abteilung Rheumatologie, Medizinischen Universitätsklinik Freiburg.
Bender
K
K
Peter
H H
HH
D000328:Adult; D000799:Angioedema / Q000209:etiology*; D003174:Complement C1 Inactivator Proteins / Q000032:analysis; D003181:Complement C4 / Q000172:deficiency; D006801:Humans; D008297:Male; D019896:alpha 1-Antitrypsin Deficiency
D003174:Complement C1 Inactivator Proteins; D003181:Complement C4
Angioneurotisches Odem bei hereditärem Alpha-1-Antitrypsinmangel.