Pseudomonas aeruginosa in cystic fibrosis patients with CFTRI1234V in a large kindred family

ABSTRACT

To describe the characteristics of Pseudomonas aeruginosa [P. aeruginosa] in cystic fibrosis [CF] patients with CFTRI1234V in a large kindred family in Qatar, medical and laboratory records were reviewed of 46 patients of CF with CFTRI1234V from 25 families arising from a single large Arab kindred tribe who attended the CF clinic between May 2002 and June 2008. Thirty eight CF patients, who were negative for P. aeruginosa at first lower respiratory culture, were enrolled in the study. The diagnosis of CF was confirmed by sweat test and molecular genetic study. Trypticase soy agar with 5% sheep blood, McConkey agar, oxidative-fermentative polymyxin B-bacitracin lactose agar, Hemophilus isolation agar and mannitol salt agar were used as selective media for the isolation of important CF respiratory pathogens. The prevalence of [P. aeruginosa] in lower respiratory cultures from 46 patients was 60.9%. Twenty [52.6%] of 38 CF patients with CFTRI1234V acquired P. aeruginosa with no seasonal variation. Positive siblings with P. aeruginosa were found in 70% indicating cross-infection within the family. The median [range] age at first detection of P. aeruginosa was 12.5 [1-19] years. There was no significant difference between males and females in acquisition of P. aeruginosa. P. aeruginosa of mucoid phenotype was detected in 11 [55%] of 20 with additional organisms, S. aureus being the second isolated organism in nine [81.8%] of the 11. A larger prospective study in a cohort of CF patients with CFTRI1234V in the Arabian Gulf region is required to determine the risk factors for acquisition of P aeruginosa with early aggressive antipseudomonal therapy