Baseline level of functional C1-inhibitor correlates with disease severity scores in hereditary angioedema
| Autor: | Enikő Mihály, Dumitru Moldovan, Gábor Széplaki, Henriette Farkas, Lilian Varga, George Füst, Beáta Visy, Zsuzsanna Kelemen, Dorottya Csuka |
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| Rok vydání: | 2010 |
| Předmět: |
Adult
Male medicine.medical_specialty Allergy Adolescent Immunology Complement Hemolytic Activity Assay Severity of Illness Index Gastroenterology Statistics Nonparametric C1-inhibitor Correlation Young Adult Classical complement pathway Disease severity Complement C1 Immunopathology Internal medicine medicine Humans Immunology and Allergy heterocyclic compounds Child Aged Retrospective Studies biology business.industry Angioedemas Hereditary Infant Complement C4 Middle Aged bacterial infections and mycoses medicine.disease Pathophysiology respiratory tract diseases Child Preschool Hereditary angioedema biology.protein Female business Complement C1 Inhibitor Protein |
| Zdroj: | Clinical Immunology. 134:354-358 |
| ISSN: | 1521-6616 |
| DOI: | 10.1016/j.clim.2009.11.002 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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