Contact System Activation and Bradykinin Generation in Angioedema: Laboratory Assessment and Biomarker Utilization.
Immunol Allergy Clin North Am
; 44(3): 543-560, 2024 Aug.
Article
en En
| MEDLINE
| ID: mdl-38937015
ABSTRACT
The role of contact system activation has been clearly established in the pathogenesis of hereditary angioedema due to C1 inhibitor deficiency (HAE-C1INH). C1 inhibitor (C1INH)-protease complexes, levels of functional C1INH, plasma kallikrein activation, and cleavage of high-molecular-weight kininogen have each been associated with disease activity. More recently, HAE with normal levels of C1INH (HAE-nl-C1INH) has been recognized. Six genetic mutations have been identified which are linked to HAE-nl-C1INH phenotypes. The majority of individuals with HAE-nl-C1INH fall into the unknown category. There is substantial evidence that bradykinin generation underlies the recurrent attacks of swelling in some of these cohorts.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Bradiquinina
/
Biomarcadores
/
Proteína Inhibidora del Complemento C1
Límite:
Humans
Idioma:
En
Revista:
Immunol Allergy Clin North Am
Asunto de la revista:
ALERGIA E IMUNOLOGIA
Año:
2024
Tipo del documento:
Article
País de afiliación:
Estados Unidos
Pais de publicación:
Estados Unidos