Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator

Abstract Objective : To determine whether there is an association between mutations of the cystic fibrosis transmembrane regulator (CFTR) and the predilection of patients with cystic fibrosis (CF) for Pseudomonas aeruginosa infection. Method: We quantified the adherence of P. aeruginosa PA01, labeled with sulfur 35-methionine, to epithelial monolayers derived from nasal scrapings of patients with specific CFTR mutations, and of carriers and normal subjects. Results: Adherence of P. aeruginosa to epithelial cells from patients with CF was significantly greater than to cells from either carriers ( t = 2.94; p = 0.009) or normal subjects ( t = 3.32; p = 0.004). Adherence to epithelial cells from patients with CF who were homozygous for the ΔF508 mutation ranged from 12% to 35% (mean, 23.7%) of the added inoculum, which was significantly greater than the binding to cells from patients with other mutations, which ranged from 3% to 18% (mean, 9.4%; t = 3.71; p = 0.002), from heterozygote carriers (3% to 11%; mean, 7.9%; t = 4.87; p = 0.002), or from normal subjects (2% to 10%: mean, 7.0%; t = 5.21; p = 0.002). Conclusion: Adherence to P. aeruginosa can be correlated with homozygosity for the D508 mutation; CFTR dysfunction may be one of the factors involved in the pathogenesis of pulmonary infection in CF. (J PEDIATR 1995;126:230-3)