Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor

Resolution of Cystic Fibrosis–related Diabetes with Ivacaftor Therapy

(2014) Don Hayes et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Improvement of Sinus Disease in Cystic Fibrosis with Ivacaftor Therapy

(2014) Don Hayes et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

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

(2014) Steven M. Rowe et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Digestive system dysfunction in cystic fibrosis: Challenges for nutrition therapy

(2014) Li Li et al.   DIGESTIVE AND LIVER DISEASE

Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp- CFTR mutation: a phase 3, open-label extension study (PERSIST)

(2014) Edward F McKone et al.   Lancet Respiratory Medicine

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

Escherichia coli Dysbiosis Correlates With Gastrointestinal Dysfunction in Children With Cystic Fibrosis

(2013) Lucas R. Hoffman et al.   CLINICAL INFECTIOUS DISEASES

Energy Expenditure and Nutritional Status in Pediatric Patients before and after Lung Transplantation

(2013) Daina Kalnins et al.   JOURNAL OF PEDIATRICS

Insulin secretion improves in cystic fibrosis following ivacaftor correction of CFTR: a small pilot study

(2013) Melena D Bellin et al.   PEDIATRIC DIABETES

The Cystic Fibrosis Intestine

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

Are Measures of Body Habitus Associated With Mortality in Cystic Fibrosis?

(2012) Andrew W. Fogarty et al.   CHEST

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

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

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

Better Nutritional Status in Early Childhood Is Associated with Improved Clinical Outcomes and Survival in Patients with Cystic Fibrosis

(2012) Elizabeth H. Yen et al.   JOURNAL OF PEDIATRICS

Persistent fat malabsorption in cystic fibrosis; lessons from patients and mice

(2011) M. Wouthuyzen-Bakker et al.   Journal of Cystic Fibrosis

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

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

Constipation in pediatric Cystic Fibrosis patients: An underestimated medical condition

(2009) H.P.J. van der Doef et al.   Journal of Cystic Fibrosis

Cystic fibrosis

(2009) Brian P O'Sullivan et al.   LANCET

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

AMPed up immunity: how antimicrobial peptides have multiple roles in immune defense

(2009) Yuping Lai et al.   TRENDS IN IMMUNOLOGY

Evidence-Based Practice Recommendations for Nutrition-Related Management of Children and Adults with Cystic Fibrosis and Pancreatic Insufficiency: Results of a Systematic Review

(2008) Virginia A. Stallings et al.   JOURNAL OF THE AMERICAN DIETETIC ASSOCIATION

Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis

(2008) Paul M Quinton   LANCET

Longitudinal Analysis of Resting Energy Expenditure in Patients with Cystic Fibrosis

(2007) Annabel Magoffin et al.   JOURNAL OF PEDIATRICS