Discontinuation versus continuation of hypertonic saline or dornase alfa in modulator treated people with cystic fibrosis (SIMPLIFY): results from two parallel, multicentre, open-label, randomised, controlled, non-inferiority trials

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles

(2022) Simon Y. Graeber et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Cystic Fibrosis Airway Mucus Hyperconcentration Produces a Vicious Cycle of Mucin, Pathogen, and Inflammatory Interactions that Promote Disease Persistence

(2022) Bethany Batson et al.   AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY

The effect of inhaled hypertonic saline on lung structure in children aged 3–6 years with cystic fibrosis (SHIP-CT): a multicentre, randomised, double-blind, controlled trial

(2022) Harm A W M Tiddens et al.   Lancet Respiratory Medicine

Treatment Preference Among People With Cystic Fibrosis

(2022) Rory A. Cameron et al.   CHEST

Withdrawal of dornase alfa increases ventilation inhomogeneity in children with cystic fibrosis

(2021) Christian Voldby et al.   Journal of Cystic Fibrosis

Cystic fibrosis

(2021) Michal Shteinberg et al.   LANCET

Clinical Effectiveness of Elexacaftor/Tezacftor/Ivacaftor in People with Cystic Fibrosis

(2021) David P Nichols et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

An observational study of the lung clearance index throughout childhood in cystic fibrosis: Early years matter

(2020) Gwyneth Davies et al.   EUROPEAN RESPIRATORY JOURNAL

A four week trial of hypertonic saline in children with mild cystic fibrosis lung disease: Effect on mucociliary clearance and clinical outcomes

(2020) Scott H. Donaldson et al.   Journal of Cystic Fibrosis

Evaluating assumptions of definition-based pulmonary exacerbation endpoints in cystic fibrosis clinical trials

(2020) Donald R. VanDevanter et al.   Journal of Cystic Fibrosis

Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial

(2019) Felix Ratjen et al.   Lancet Respiratory Medicine

Integrating the multiple breath washout test into international multicentre trials

(2019) Clare Saunders et al.   Journal of Cystic Fibrosis

Answering the call to address cystic fibrosis treatment burden in the era of highly effective CFTR modulator therapy

(2019) Alex H. Gifford et al.   Journal of Cystic Fibrosis

Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial

(2019) Harry G M Heijerman et al.   LANCET

Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele

(2019) Peter G. Middleton et al.   NEW ENGLAND JOURNAL OF MEDICINE

Microbiology of Cystic Fibrosis Airway Disease

(2019) Ana C. Blanchard et al.   SEMINARS IN RESPIRATORY AND CRITICAL CARE MEDICINE

Changes in Lung Clearance Index in Preschool Cystic Fibrosis Patients Treated with Ivacaftor

(2018) Felix Ratjen et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Cystic Fibrosis Sputum Rheology Correlates With Both Acute and Longitudinal Changes in Lung Function

(2018) Jonathan T. Ma et al.   CHEST

Time-lagged predictors of daily medication nonadherence beliefs during the month post-hospital discharge in patients with psychotic–spectrum disorders

(2018) Brandon A. Gaudiano et al.   PSYCHIATRY RESEARCH

VX-445–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

(2018) Dominic Keating et al.   NEW ENGLAND JOURNAL OF MEDICINE

Ecological Momentary Assessment in Behavioral Research: Addressing Technological and Human Participant Challenges

(2017) Lora E Burke et al.   JOURNAL OF MEDICAL INTERNET RESEARCH

The top 10 research priorities in cystic fibrosis developed by a partnership between people with CF and healthcare providers

(2017) Nicola J Rowbotham et al.   THORAX

Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis

(2016) T. Lahiri et al.   PEDIATRICS

Inflammation and its genesis in cystic fibrosis

(2015) David P. Nichols et al.   PEDIATRIC PULMONOLOGY

Pulmonary Medication Adherence and Health-care Use in Cystic Fibrosis

(2014) Alexandra L. Quittner et al.   CHEST

Cystic Fibrosis Pulmonary Guidelines

(2013) Peter J. Mogayzel et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D- CFTR mutation and preserved spirometry: a randomised controlled trial

(2013) Jane Davies et al.   Lancet Respiratory Medicine

Multi-ethnic reference values for spirometry for the 3–95-yr age range: the global lung function 2012 equations

(2012) Philip H. Quanjer et al.   EUROPEAN RESPIRATORY JOURNAL

Text Messaging to Measure Asthma Medication Use and Symptoms in Urban African American Emerging Adults: A Feasibility Study

(2012) Karen MacDonell et al.   JOURNAL OF ASTHMA

Psychometric evaluation of the Cystic Fibrosis Questionnaire-Revised in a national sample

(2011) Alexandra L. Quittner et al.   QUALITY OF LIFE RESEARCH

The effect of dornase alfa on ventilation inhomogeneity in patients with cystic fibrosis

(2010) R. Amin et al.   EUROPEAN RESPIRATORY JOURNAL

Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function

(2010) R. Amin et al.   THORAX

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

High treatment burden in adults with cystic fibrosis: Challenges to disease self-management

(2008) Gregory S. Sawicki et al.   Journal of Cystic Fibrosis