Allotypes of α1-antitrypsin in patients with cystic fibrosis,homozygous and heterozygous for deltaF508 |
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Authors: | Gerd Dring Helle Krogh-Johansen Sebastian Weidinger Niels Hiby |
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Institution: | Gerd Döring,Helle Krogh-Johansen,Sebastian Weidinger,Niels Høiby |
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Abstract: | In cystic fibrosis (CF) neutrophil released serine proteinase activity may facilitate Pseudomonas aeruginosa lung colonization, leading to chronic infection. Since such activity is mostly controlled by α1-antitrypsin (α1-AT), we postulated that patients with CF carrying deficient α1-AT variants might be at higher risk for P. aeruginosa acquisition and might reveal other phenomena, specific for serine proteinase activity. In 215 Danish patients with CF, homozygous (80%) or heterozygous (20%) for the major CF mutation deltaF508, α1-AT variants were determined. Carriage of deficient α-AT variants was correlated to an earlier onset of P. aeruginosa lung infection (P < 0.001), higher total IgG (P < 0.001), and P. aeruginosa-specific serum antibodies (P < 0.0001). The two groups did not differ in lung function, probably due to intensive antimicrobial treatment. Pediatr Pulmonol. 1994; 18:3–7. © 1994 Wiley-Liss. Inc. |
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Keywords: | Pseudomonas aeruginosa lung infection human neutrophil elastase hyper-gammaglobulinemia |
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