Putting lung function and physiology into perspective: cystic fibrosis in adults

Lack of Neutrophil Elastase Reduces Inflammation, Mucus Hypersecretion, and Emphysema, but Not Mucus Obstruction, in Mice with Cystic Fibrosis–like Lung Disease

(2014) Stefanie Gehrig et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Effects of Ivacaftor in Patients With Cystic Fibrosis Who Carry the G551D Mutation and Have Severe Lung Disease

(2014) Peter J. Barry et al.   CHEST

Question everything

(2014) R. Pellegrino et al.   EUROPEAN RESPIRATORY JOURNAL

Cystic fibrosis: An inherited disease affecting mucin-producing organs

(2014) Camille Ehre et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

WS13.5 Visualising ventilation heterogeneity in mild CF using hyperpolarised 3He MRI, and comparison with lung clearance index (LCI)

(2014) A. Horsley et al.   Journal of Cystic Fibrosis

Lung clearance index in adults with non-cystic fibrosis bronchiectasis

(2014) Sherif Gonem et al.   RESPIRATORY RESEARCH

Measurement of FEF25-75% and FEF75% does not contribute to clinical decision making

(2013) P. H. Quanjer et al.   EUROPEAN RESPIRATORY JOURNAL

Quantification of regional fractional ventilation in human subjects by measurement of hyperpolarized 3He washout with 2D and 3D MRI

(2013) Felix C. Horn et al.   JOURNAL OF APPLIED PHYSIOLOGY

Prognostic relevance of dynamic hyperinflation during cardiopulmonary exercise testing in adult patients with cystic fibrosis

(2013) D. Stevens et al.   Journal of Cystic Fibrosis

The impact of switching to the new global lung function initiative equations on spirometry results in the UK CF Registry

(2013) Sanja Stanojevic 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

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

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

Evaluation of the Impact of Alveolar Nitrogen Excretion on Indices Derived from Multiple Breath Nitrogen Washout

(2013) Niklas Nielsen et al.   PLoS One

Pulmonary Emphysema in Cystic Fibrosis Detected by Densitometry on Chest Multidetector Computed Tomography

(2013) Mark O. Wielpütz et al.   PLoS One

Acinar and conductive ventilation heterogeneity in severe CF lung disease: Back to the model

(2013) Sylvia Verbanck et al.   RESPIRATORY PHYSIOLOGY & NEUROBIOLOGY

Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation

(2013) Alex R Horsley et al.   THORAX

Reasons for heterogeneous change in LCI in children with cystic fibrosis after antibiotic treatment

(2013) Sophie Yammine et al.   THORAX

Lung clearance index in adult cystic fibrosis patients: the role of convection-dependent lung units

(2012) Sylvia Verbanck et al.   EUROPEAN RESPIRATORY JOURNAL

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

Risk factors for rate of decline in FEV1 in adults with cystic fibrosis

(2012) Michael W. Konstan et al.   Journal of Cystic Fibrosis

46 Hyperpolarized Gas MRI of ivacaftor therapy in subjects with cystic fibrosis who have the G551D-CFTR mutation

(2012) T. Altes et al.   Journal of Cystic Fibrosis

Understanding the natural progression in %FEV1decline in patients with cystic fibrosis: a longitudinal study

(2012) David Taylor-Robinson et al.   THORAX

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

(2012) Ah-Fong Hoo et al.   THORAX

Hyperpolarized 3He Magnetic Resonance Functional Imaging Semiautomated Segmentation

(2011) Miranda Kirby et al.   ACADEMIC RADIOLOGY

Relationships between the lung clearance index and conductive and acinar ventilation heterogeneity

(2011) Sylvia Verbanck et al.   JOURNAL OF APPLIED PHYSIOLOGY

A CFTR Potentiator in Patients with Cystic Fibrosis and theG551DMutation

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

Ventilation inhomogeneity in children with primary ciliary dyskinesia

(2011) Kent Green et al.   THORAX

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

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

Quantification of the Relative Contribution of Environmental and Genetic Factors to Variation in Cystic Fibrosis Lung Function

(2010) J. Michael Collaco et al.   JOURNAL OF PEDIATRICS

Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis

(2010) Don B. Sanders et al.   PEDIATRIC PULMONOLOGY

S18 Longitudinal assessment of biomarkers for clinical trials of novel therapeutic agents: the run-in study

(2010) E. W. F. W. Alton et al.   THORAX

Assessment of hyperpolarized3He lung MRI for regional evaluation of interventional therapy: A pilot study in pediatric cystic fibrosis

(2009) Neil Woodhouse et al.   JOURNAL OF MAGNETIC RESONANCE IMAGING

Using index of ventilation to assess response to treatment for acute pulmonary exacerbation in children with cystic fibrosis

(2009) Paul D. Robinson et al.   PEDIATRIC PULMONOLOGY

Inert Gas Washout: Theoretical Background and Clinical Utility in Respiratory Disease

(2009) Paul D. Robinson et al.   RESPIRATION

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

Effects of cystic fibrosis lung disease on gas mixing indices derived from alveolar slope analysis

(2008) Alex R. Horsley et al.   RESPIRATORY PHYSIOLOGY & NEUROBIOLOGY

Neutrophils in cystic fibrosis

(2008) D G Downey et al.   THORAX

A model-based method for flow limitation analysis in the heterogeneous human lung

(2007) Adam G. Polak   COMPUTER METHODS AND PROGRAMS IN BIOMEDICINE