Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor

Neonates with cystic fibrosis have a reduced nasal liquid pH; A small pilot study

(2014) Mahmoud H. Abou Alaiwa et al.   Journal of Cystic Fibrosis

Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle

(2013) Kang-Yang Jih et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype

(2012) Jenny K. Gustafsson et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis

(2012) Eugene H. Chang et al.   LARYNGOSCOPE

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

(2012) Alejandro A. Pezzulo et al.   NATURE

Hyperviscous airway periciliary and mucous liquid layers in cystic fibrosis measured by confocal fluorescence photobleaching

(2011) Nico Derichs et al.   FASEB JOURNAL

Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTRMutation

(2010) Frank J. Accurso et al.   NEW ENGLAND JOURNAL OF MEDICINE

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

(2009) F. Van Goor et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Concordant genotype of upper and lower airways P aeruginosa and S aureus isolates in cystic fibrosis

(2009) J G Mainz et al.   THORAX

Guidelines for Diagnosis of Cystic Fibrosis in Newborns through Older Adults: Cystic Fibrosis Foundation Consensus Report

(2008) Philip M. Farrell et al.   JOURNAL OF PEDIATRICS