Clinical Mechanism of the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor in G551D-mediated Cystic Fibrosis

Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with aG551DMutation

(2013) Jane C. Davies et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Change in Sweat Chloride as a Clinical End Point in Cystic Fibrosis Clinical Trials

(2013) Anthony G. Durmowicz et al.   CHEST

Multisite Comparison of Mucociliary and Cough Clearance Measures Using Standardized Methods

(2013) William D. Bennett et al.   Journal of Aerosol Medicine and Pulmonary Drug Delivery

Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene

(2013) Patrick R Sosnay et al.   NATURE GENETICS

Sixty-five years since the New York heat wave: Advances in sweat testing for cystic fibrosis

(2013) Jake T.B. Collie et al.   PEDIATRIC PULMONOLOGY

Inflammation and Airway Microbiota during Cystic Fibrosis Pulmonary Exacerbations

(2013) Edith T. Zemanick et al.   PLoS One

The Cystic Fibrosis Intestine

(2013) R. C. De Lisle et al.   Cold Spring Harbor Perspectives in Medicine

Personalized Medicine in Cystic Fibrosis

(2012) John P. Clancy et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

β-Adrenergic Sweat Secretion as a Diagnostic Test for Cystic Fibrosis

(2012) Paul Quinton et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Sputum Biomarkers of Inflammation and Lung Function Decline in Children with Cystic Fibrosis

(2012) Scott D. Sagel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Gastrointestinal Complications of Cystic Fibrosis

(2012) Daniel Gelfond et al.   Clinical Gastroenterology and Hepatology

Intestinal pH and Gastrointestinal Transit Profiles in Cystic Fibrosis Patients Measured by Wireless Motility Capsule

(2012) Daniel Gelfond et al.   DIGESTIVE DISEASES AND SCIENCES

Regulatory Aspects of Phase 3 Endpoints for New Inhaled Antibiotics for Cystic Fibrosis Patients with Chronic Pseudomonas aeruginosa Infections

(2012) Alan Bruce Montgomery et al.   Journal of Aerosol Medicine and Pulmonary Drug Delivery

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

Intractable cough in a preterm infant with ventriculoperitoneal shunt

(2012) Ronen Bar-Yoseph et al.   PEDIATRIC PULMONOLOGY

Progress in cystic fibrosis and the CF Therapeutics Development Network

(2012) Steven M Rowe et al.   THORAX

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

(2011) Bonnie W. Ramsey et al.   NEW ENGLAND JOURNAL OF MEDICINE

Initial Pseudomonas aeruginosa treatment failure is associated with exacerbations in cystic fibrosis

(2011) Nicole Mayer-Hamblett et al.   PEDIATRIC PULMONOLOGY

Role of epithelial HCO3− transport in mucin secretion: lessons from cystic fibrosis

(2010) Paul M. Quinton   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

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

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

Airway Microbiota and Pathogen Abundance in Age-Stratified Cystic Fibrosis Patients

(2010) Michael J. Cox et al.   PLoS One

Determination of the Minimal Clinically Important Difference Scores for the Cystic Fibrosis Questionnaire-Revised Respiratory Symptom Scale in Two Populations of Patients With Cystic Fibrosis and Chronic Pseudomonas aeruginosa Airway Infection

(2009) Alexandra L. Quittner et al.   CHEST

Patient-reported respiratory symptoms in cystic fibrosis

(2009) C.H. Goss et al.   Journal of Cystic Fibrosis