Titre
Prekallikrein activation and high-molecular-weight kininogen consumption in hereditary angioedema
Type
article
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Auteur(s)
Schapira, M.
Auteure/Auteur
Silver, L. D.
Auteure/Auteur
Scott, C. F.
Auteure/Auteur
Schmaier, A. H.
Auteure/Auteur
Prograis, L. J., Jr.,
Auteure/Auteur
Curd, J. G.
Auteure/Auteur
Colman, R. W.
Auteure/Auteur
Liens vers les personnes
Liens vers les unités
ISSN
0028-4793
Statut éditorial
Publié
Date de publication
1983-05
Volume
308
Numéro
18
Première page
1050
Dernière page/numéro d’article
3
Notes
Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S. --- Old month value: May 5
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S. --- Old month value: May 5
Résumé
Patients with hereditary angioedema lack C-1 inhibitor, a plasma alpha 2-glycoprotein that inhibits both the proteolytic action of C1, the activated first component of the complement system, and the activity of components of the contact phase of coagulation: kallikrein, factor XIa, and factor XIIa. Such patients have been shown to have low levels of C4 and C2, the natural substrates for C-1, but the levels were not correlated with the presence of symptoms. We studied three patients with angioedema for evidence of activation of the contact system and found that during a symptomatic period they had decreased levels of prekallikrein, a substrate for the activated forms of factor XII, and reductions in high-molecular-weight kininogen, a substrate for plasma kallikrein. These observations suggest that zymogens of the contact system are activated during attacks of hereditary angioedema and that some of the clinical manifestations may be mediated through products of this pathway, such as kinins.
Sujets
PID Serval
serval:BIB_4370370F12E8
PMID
Date de création
2008-01-25T14:28:08.003Z
Date de création dans IRIS
2025-05-20T17:06:56Z