Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema.
Clin Immunol
; 134(3): 354-8, 2010 Mar.
Article
en En
| MEDLINE
| ID: mdl-19945350
ABSTRACT
The diagnosis of hereditary angioedema (HAE) is based on complement tests. We studied for the first time the possible association between complement parameters measured at the time of diagnosis and disease severity in 115 patients with HAE. Serum levels of functional C1-inhibitor (C1-INH(f)), antigenic C1-inhibitor (C1-INH(a)), C4 and hemolytic activity of the classical pathway (CH50) were determined at the time of diagnosis. We found a significant correlation between severity scores and baseline C1-INH(f) levels, as determined by ELISA assay (p=0.0003). On the other hand, there was no correlation between severity scores and other complement parameters (C1-INH(a), C4, and CH50). We consider the correlation between severity scores and baseline C1-INH(f) levels an important pathophysiological observation. Our findings underlie the potential significance of monitoring functional C1-INH levels in relation to clinical disease course.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Proteína Inhibidora del Complemento C1
/
Angioedemas Hereditarios
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
/
Risk_factors_studies
Aspecto:
Patient_preference
Límite:
Adolescent
/
Adult
/
Aged
/
Child
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Child, preschool
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Female
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Humans
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Infant
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Male
/
Middle aged
Idioma:
En
Revista:
Clin Immunol
Asunto de la revista:
ALERGIA E IMUNOLOGIA
Año:
2010
Tipo del documento:
Article
País de afiliación:
Hungria