Novel outcome measures for clinical trials in cystic fibrosis

Monitoring Cystic Fibrosis Lung Disease by Computed Tomography. Radiation Risk in Perspective

(2014) Wieying Kuo 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

Magnetic Resonance Imaging Detects Changes in Structure and Perfusion, and Response to Therapy in Early Cystic Fibrosis Lung Disease

(2014) Mark O. Wielpütz et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Sweat conductivity: An accurate diagnostic test for cystic fibrosis?

(2014) Ana Claudia Veras Mattar et al.   Journal of Cystic Fibrosis

The authors reply

(2014) Marije Bakker et al.   PEDIATRIC PULMONOLOGY

Plasma sCD14 as a Biomarker to Predict Pulmonary Exacerbations in Cystic Fibrosis

(2014) Bradley S. Quon et al.   PLoS One

Candidate Markers Associated with the Probability of Future Pulmonary Exacerbations in Cystic Fibrosis Patients

(2014) Gabriella Wojewodka et al.   PLoS One

Don't let radiation scare trump patient care: 10 ways you can harm your patients by fear of radiation-induced cancer from diagnostic imaging

(2014) Alan S Brody et al.   THORAX

Airway, but not serum or urinary, levels of YKL-40 reflect inflammation in early cystic fibrosis lung disease

(2014) Emmanuelle Fantino et al.   BMC Pulmonary Medicine

Pediatric Chest Computed Tomography at a Radiation Dose Approaching a Chest Radiograph

(2013) Martin J. Willemink et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Lung Clearance Index as an Outcome Measure for Clinical Trials in Young Children with Cystic Fibrosis. A Pilot Study Using Inhaled Hypertonic Saline

(2013) Padmaja Subbarao et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE 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

Lateral Topography for Reducing Effective Dose in Low-Dose Chest CT

(2013) Dong-Ho Bang et al.   AMERICAN JOURNAL OF ROENTGENOLOGY

Sinogram-Affirmed Iterative Reconstruction of Low-Dose Chest CT: Effect on Image Quality and Radiation Dose

(2013) Mannudeep K. Kalra et al.   AMERICAN JOURNAL OF ROENTGENOLOGY

Lung [18F]fluorodeoxyglucose Uptake and Ventilation–Perfusion Mismatch in the Early Stage of Experimental Acute Smoke Inhalation

(2013) Guido Musch et al.   ANESTHESIOLOGY

Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in the Lower Airways in COPD

(2013) Mark T. Dransfield et al.   CHEST

Systematic Review of Blood Biomarkers in Cystic Fibrosis Pulmonary Exacerbations

(2013) Alborz Hakimi Shoki et al.   CHEST

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

(2013) Anthony G. Durmowicz et al.   CHEST

Impact of bronchiectasis and trapped air on quality of life and exacerbations in cystic fibrosis

(2013) Leonie A. Tepper et al.   EUROPEAN RESPIRATORY JOURNAL

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

Nasal potential difference measurements in diagnosis of cystic fibrosis: An international survey

(2013) Lutz Naehrlich et al.   Journal of Cystic Fibrosis

Lung clearance index: Evidence for use in clinical trials in cystic fibrosis

(2013) L. Kent et al.   Journal of Cystic Fibrosis

Risk Factors for Bronchiectasis in Children with Cystic Fibrosis

(2013) Peter D. Sly et al.   NEW ENGLAND JOURNAL OF MEDICINE

Small airway involvement in cystic fibrosis lung disease: Routine spirometry as an early and sensitive marker

(2013) E. Marije Bakker et al.   PEDIATRIC PULMONOLOGY

Multiple Breath Nitrogen Washout: A Feasible Alternative to Mass Spectrometry

(2013) Renee Jensen et al.   PLoS One

Optimizing Nasal Potential Difference Analysis for CFTR Modulator Development: Assessment of Ivacaftor in CF Subjects with the G551D-CFTR Mutation

(2013) Steven M. Rowe et al.   PLoS One

Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function

(2013) John P. Clancy et al.   PLoS One

In Vivo Readout of CFTR Function: Ratiometric Measurement of CFTR-Dependent Secretion by Individual, Identifiable Human Sweat Glands

(2013) Jeffrey J. Wine et al.   PLoS One

Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis

(2013) François Vermeulen et al.   THORAX

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

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

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

Chest Computed Tomography Scores Are Predictive of Survival in Patients with Cystic Fibrosis Awaiting Lung Transplantation

(2012) Martine Loeve 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

Effect of Azithromycin on Systemic Markers of Inflammation in Patients With Cystic Fibrosis Uninfected With Pseudomonas aeruginosa

(2012) Felix Ratjen et al.   CHEST

Ivacaftor in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation

(2012) Patrick A. Flume et al.   CHEST

Inhaled Hypertonic Saline in Infants and Children Younger Than 6 Years With Cystic Fibrosis

(2012) Margaret Rosenfeld et al.   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Ivacaftor

(2012) Pamela B. Davis et al.   NATURE REVIEWS DRUG DISCOVERY

Evaluation of the intestinal current measurement method as a diagnostic test for cystic fibrosis

(2012) Malena Cohen-Cymberknoh et al.   PEDIATRIC PULMONOLOGY

Sputum Biomarkers and the Prediction of Clinical Outcomes in Patients with Cystic Fibrosis

(2012) Theodore G. Liou et al.   PLoS One

Cystic Fibrosis: Detecting Changes in Airway Inflammation with FDG PET/CT

(2012) Reshma Amin et al.   RADIOLOGY

Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening

(2012) Ah-Fong Hoo et al.   THORAX

The Importance of Imaging in Cystic Fibrosis

(2011) Cori Daines et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Morphologic and functional scoring of cystic fibrosis lung disease using MRI

(2011) Monika Eichinger et al.   EUROPEAN JOURNAL OF RADIOLOGY

Randomised controlled trials in cystic fibrosis: what, when and how?

(2011) P. D. Sly et al.   EUROPEAN RESPIRATORY JOURNAL

Improved treatment response to dornase alfa in cystic fibrosis patients using controlled inhalation

(2011) E. M. Bakker et al.   EUROPEAN RESPIRATORY JOURNAL

Using bacterial biomarkers to identify early indicators of cystic fibrosis pulmonary exacerbation onset

(2011) Geraint B Rogers et al.   EXPERT REVIEW OF MOLECULAR DIAGNOSTICS

New clinical diagnostic procedures for cystic fibrosis in Europe

(2011) K. De Boeck et al.   Journal of Cystic Fibrosis

A novel neutrophil derived inflammatory biomarker of pulmonary exacerbation in cystic fibrosis

(2011) Emer P. Reeves et al.   Journal of Cystic Fibrosis

Using hyperpolarized 3He MRI to evaluate treatment efficacy in cystic fibrosis patients

(2011) Yanping Sun et al.   JOURNAL OF MAGNETIC RESONANCE IMAGING

18F-FDG Uptake Rate Is a Biomarker of Eosinophilic Inflammation and Airway Response in Asthma

(2011) R. S. Harris et al.   JOURNAL OF NUCLEAR MEDICINE

Pulmonary Magnetic Resonance Imaging for Airway Diseases

(2011) Yoshiharu Ohno et al.   JOURNAL OF THORACIC IMAGING

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

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

Lung clearance index: clinical and research applications in children

(2011) Susanne I. Fuchs et al.   Paediatric Respiratory Reviews

Improvement in pulmonary function following antibiotics in infants with cystic fibrosis

(2011) Jessica E. Pittman et al.   PEDIATRIC PULMONOLOGY

The Sensitivity of Lung Disease Surrogates in Detecting Chest CT Abnormalities in Children With Cystic Fibrosis

(2011) Don B. Sanders et al.   PEDIATRIC PULMONOLOGY

The Chitinase-Like Protein YKL-40 Modulates Cystic Fibrosis Lung Disease

(2011) Andreas Hector et al.   PLoS One

Air Trapping on Chest CT Is Associated with Worse Ventilation Distribution in Infants with Cystic Fibrosis Diagnosed following Newborn Screening

(2011) Graham L. Hall et al.   PLoS One

Lung Clearance Index and HRCT are complementary markers of lung abnormalities in young children with CF

(2011) C. M. Owens et al.   THORAX

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for theF508del-CFTRmutation

(2011) J P Clancy et al.   THORAX

Progression of early structural lung disease in young children with cystic fibrosis assessed using CT

(2011) Lauren S Mott et al.   THORAX

Acute inhalation of hypertonic saline does not improve mucociliary clearance in all children with cystic fibrosis

(2011) Beth L Laube et al.   BMC Pulmonary Medicine

Multicenter Evaluation of Infant Lung Function Tests as Cystic Fibrosis Clinical Trial Endpoints

(2010) Stephanie D. Davis et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Lung Clearance Index at 4 Years Predicts Subsequent Lung Function in Children with Cystic Fibrosis

(2010) Paul Aurora et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

An International Randomized Multicenter Comparison of Nasal Potential Difference Techniques

(2010) George M. Solomon et al.   CHEST

Bronchiectasis and Pulmonary Exacerbations in Children and Young Adults With Cystic Fibrosis

(2010) Martine Loeve et al.   CHEST

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

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

Multimodality molecular imaging of the lung

(2010) Delphine L. Chen et al.   JOURNAL OF MAGNETIC RESONANCE IMAGING

Computed tomography and magnetic resonance imaging in cystic fibrosis lung disease

(2010) Monika Eichinger et al.   JOURNAL OF MAGNETIC RESONANCE IMAGING

Sputum Induction in Routine Clinical Care of Children with Cystic Fibrosis

(2010) Suhail Al-Saleh et al.   JOURNAL OF PEDIATRICS

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

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

The role of advanced imaging techniques in cystic fibrosis follow-up: is there a place for MRI?

(2010) Michael Puderbach et al.   PEDIATRIC RADIOLOGY

Sensitivity of lung clearance index and chest computed tomography in early cf lung disease

(2010) Helmut Ellemunter et al.   RESPIRATORY MEDICINE

Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data

(2010) N. Derichs et al.   THORAX

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

(2010) R. Amin et al.   THORAX

Sputum Desmosine During Hospital Admission for Pulmonary Exacerbation in Cystic Fibrosis

(2009) Theresa A. Laguna et al.   CHEST

18 F-Fluorodeoxyglucose-PET/CT Imaging of Lungs in Patients With Cystic Fibrosis

(2009) Martine Klein et al.   CHEST

Bronchiectasis in Infants and Preschool Children Diagnosed with Cystic Fibrosis after Newborn Screening

(2009) Stephen M. Stick et al.   JOURNAL OF PEDIATRICS

Lung morphology assessment using MRI: A robust ultra-short TR/TE 2D steady state free precession sequence used in cystic fibrosis patients

(2009) Riccarda Failo et al.   MAGNETIC RESONANCE IN MEDICINE

Non-contrast-enhanced perfusion and ventilation assessment of the human lung by means of fourier decomposition in proton MRI

(2009) Grzegorz Bauman et al.   MAGNETIC RESONANCE IN MEDICINE

Lung clearance index: Normal values, repeatability, and reproducibility in healthy children and adolescents

(2009) Susanne I. Fuchs et al.   PEDIATRIC PULMONOLOGY

Lung clearance index in the assessment of airways disease

(2009) Alex Horsley   RESPIRATORY MEDICINE

The spectrum of structural abnormalities on CT scans from patients with CF with severe advanced lung disease

(2009) M Loeve et al.   THORAX

Lung Function in Infants with Cystic Fibrosis Diagnosed by Newborn Screening

(2008) Barry M. Linnane et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Radiation Dose Reduction in Chest CT: A Review

(2008) Takeshi Kubo et al.   AMERICAN JOURNAL OF ROENTGENOLOGY

A European consensus for the evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis

(2008) S.J. Mayell et al.   Journal of Cystic Fibrosis

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

Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial

(2008) Eitan Kerem et al.   LANCET

Functional MR Imaging of the Lung

(2008) Shin Matsuoka et al.   Magnetic Resonance Imaging Clinics of North America

Advances in Pediatric MR Imaging

(2008) John D. MacKenzie et al.   Magnetic Resonance Imaging Clinics of North America

Biomarkers in exhaled breath condensate: a review of collection, processing and analysis

(2008) N M Grob et al.   Journal of Breath Research