Pseudomonas aeruginosa in cystic fibrosis patients with G551D-CFTR treated with ivacaftor.

ABSTRACT:

Background

Ivacaftor improves outcomes in cystic fibrosis (CF) patients with the G551D mutation; however, effects on respiratory microbiology are largely unknown. This study examines changes in CF respiratory pathogens with ivacaftor and correlates them with baseline characteristics and clinical response.

Methods

The G551D Observational Study enrolled a longitudinal observational cohort of US patients with CF aged 6 years and older with at least 1 copy of the G551D mutation. Results were linked with retrospective and prospective culture data in the US Cystic Fibrosis Foundation's National Patient Registry. Pseudomonas aeruginosa infection category in the year before and year after ivacaftor was compared and correlated with clinical findings.

Results

Among 151 particip

SUBMITTER: Heltshe SL 

PROVIDER: S-EPMC4342673 | biostudies-literature | 2015 Mar

REPOSITORIES: biostudies-literature