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Cystic Fibrosis

HomePatientsLung Disease Week at the ATS2020Cystic Fibrosis ▶ Information for Experts
Information for Experts

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ClinicianATS Educational Products

 

INTERSTITIAL LUNG DISEASE: CURRENT TRENDS IN DIAGNOSIS AND MANAGEMENT

CYSTIC FIBROSIS: HETEROGENEITY IN A MONOGENIC DISEASE

WHEN THERE IS NO RIGHT ANSWER: A PRO/CON DEBATE ON CONTROVERSIES IN ILD

DISCOVERING THE ROLE OF STEM CELL FATE IN LUNG INJURY AND FIBROSIS

BRONCHIECTASIS AND ADULT CYSTIC FIBROSIS: WHERE DO WE STAND TODAY?

 


 

ClinicianATS Journal articles on Cystic Fibrosis

 

American Journal of Respiratory and Critical Care Medicine

Targeting Proteases in Cystic Fibrosis Lung Disease. Paradigms, Progress, and Potential
2020
Link to Abstract

Real-Life Safety and Effectiveness of Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis
2020
Link to Abstract

TMEM16A Potentiators: Is There a Need for New Modulators in Cystic Fibrosis?
2020
Link to Abstract

Early Aspergillosis in Cystic Fibrosis and Air Trapping: Guilt by Association?
2020
Link to Abstract

TMEM16A Potentiation: A Novel Therapeutic Approach for the Treatment of Cystic Fibrosis
2020
Link to Abstract

Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications
2019
Link to Abstract

Females with Cystic Fibrosis Demonstrate a Differential Response Profile to Ivacaftor Compared to Males
2019
Link to Abstract

A Perspective: Division of Lung Diseases at Fifty
2019
Link to Abstract

Evaluating Long-Term Benefits of Chronic Azithromycin: Furthering Our Quest for Precision Medicine
2019
Link to Abstract

Specific Inhibition of the NLRP3 Inflammasome as an Antiinflammatory Strategy in Cystic Fibrosis
2019
Link to Abstract

Lower Airway Infection in Preschool Children with Cystic Fibrosis – An International Comparison
2019
Link to Abstract

Selective Sampling of the Lower Airway in Children with CF: What Are We Missing?
2019
Link to Abstract

Aspergillus Infections and Progression of Structural Lung Disease in Children with Cystic Fibrosis
2019
Link to Abstract

Pre-Clinical Modeling for Therapeutic Development in Cystic Fibrosis
2019
Link to Abstract

Effect of Including Important Clinical Variables on Accuracy of the Lung Allocation Score for Cystic Fibrosis and Chronic Obstructive Pulmonary Disease
2019
Link to Abstract

Losartan Rescues Inflammation-Related Mucociliary Dysfunction in Relevant Models of Cystic Fibrosis
2019
Link to Abstract

Risk Factors for Functional Decline and Impaired Quality of Life after Pediatric Respiratory Failure
2019
Link to Abstract

Changing Prevalence of Lower Airway Infections in Young Children with Cystic Fibrosis
2019
Link to Abstract

Preventive Inhalation of Hypertonic Saline in Infants with Cystic Fibrosis (PRESIS). A Randomized, Double-Blind, Controlled Study
2019
Link to Abstract

Correlating Cystic Fibrosis Transmembrane Conductance Regulator Function with Clinical Features to Inform Precision Treatment of Cystic Fibrosis
2019
Link to Abstract

Cardiopulmonary Exercise Testing Provides Additional Prognostic Information in Cystic Fibrosis
2019
Link to Abstract

Elastase Exocytosis by Airway Neutrophils Is Associated with Early Lung Damage in Children with Cystic Fibrosis
2019
Link to Abstract

Distinct “Immunoallertypes” of Disease and High Frequencies of Sensitization in Non–Cystic Fibrosis Bronchiectasis
2019
Link to Abstract

Islet Hormone and Incretin Secretion in Cystic Fibrosis after Four Months of Ivacaftor Therapy
2019
Link to Abstract

 

American Journal of Respiratory Cell and Molecular Biology

Subacute TGFβ Exposure Drives Airway Hyperresponsiveness in CF Mice through the PI3K Pathway
2020
Link to Abstract

Air Space Distension Precedes Spontaneous Fibrotic Remodeling and Impaired Cholesterol Metabolism in the Absence of Surfactant Protein C
2020
Link to Abstract

Airway Surface Liquid Has Innate Antiviral Activity That Is Reduced in Cystic Fibrosis
2020
Link to Abstract

High CO2 Downregulates Skeletal Muscle Protein Anabolism via AMP-activated Protein Kinase α2–mediated Depressed Ribosomal Biogenesis
2020
Link to Abstract

Embedding of Precision-Cut Lung Slices in Engineered Hydrogel Biomaterials Supports Extended Ex Vivo Culture
2020
Link to Abstract

S-Nitrosylation of CHIP Enhances F508Del-CFTR Maturation
2019
Link to Abstract

Augmentation of Cystic Fibrosis Transmembrane Conductance Regulator Function in Human Bronchial Epithelial Cells via SLC6A14-Dependent Amino Acid Uptake. Implications for Treatment of Cystic Fibrosis
2019
Link to Abstract

DNAse to the Rescue! Clearing Mitochondrial DNA May Have NET Benefits in Lung Transplantation
2019
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Methods in Lung Microbiome Research
2019
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“Shocking the System” to Achieve Efficient Gene Targeting in Primary Human Airway Epithelia
2019
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Efficient RNP-Directed Human Gene Targeting Reveals SPDEF is Required for IL-13-Induced Mucostasis
2019
Link to Abstract

The Epithelial Expressions of YAP and TAZ are Sequentially Required in Lung Development
2019
Link to Abstract

Cystic Fibrosis Plasma Blunts the Immune Response to Bacterial Infection
2019
Link to Abstract

A Flow Cytometric Method for Isolating Cystic Fibrosis Airway Macrophages from Expectorated Sputum
2019
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Pendrin Mediates Bicarbonate Secretion and Enhances Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Surface Epithelia
2019
Link to Abstract

Contractile Properties of Intrapulmonary Airway Smooth Muscle in Cystic Fibrosis
2019
Link to Abstract

IL-9 Blockade Suppresses Silica-induced Lung Inflammation and Fibrosis in Mice
2019
Link to Abstract

 

Annals of ATS

Overview and Challenges of Bronchiolar Disorders
2019
Link to Abstract

Measures of Cystic Fibrosis Airway Microbiota during Periods of Clinical Stability
2019
Link to Abstract

Reflux-Aspiration in Chronic Lung Disease
2019
Link to Abstract

Ivacaftor Is Associated with Reduced Lung Infection by Key Cystic Fibrosis Pathogens. A Cohort Study Using National Registry Data
2019
Link to Abstract

The Extra-pulmonary Effects of CFTR Modulators in Cystic Fibrosis
2019
Link to Abstract

Quantifying Long-term Changes in Lung Function and Exacerbations after Initiation of Azithromycin in Cystic Fibrosis
2019
Link to Abstract

Changes in Airway Microbiome and Inflammation with Ivacaftor Treatment in Patients with Cystic Fibrosis and the G551D Mutation
2019
Link to Abstract

Shedding Light on Cystic Lung Disease
2019
Link to Abstract

Mortality in Adults with Cystic Fibrosis Requiring Mechanical Ventilation. Cross-Sectional Analysis of Nationwide Events
2019
Link to Abstract

Oral Azithromycin Use and the Recovery of Lung Function from Pulmonary Exacerbations Treated with Intravenous Tobramycin or Colistimethate in Adults with Cystic Fibrosis
2019
Link to Abstract

2019 Clinical Practice Guideline Summary for Clinicians: Home Oxygen Therapy for Children
2019
Link to Abstract

Developing Inhaled Antibiotics in Cystic Fibrosis: Current Challenges and Opportunities
2019
Link to Abstract

CFTR Modulator Use Is Associated with Higher Hemoglobin Levels in Individuals with Cystic Fibrosis
2019
Link to Abstract

Longitudinal Trends in Real-World Outcomes after Initiation of Ivacaftor. A Cohort Study from the Cystic Fibrosis Registry of Ireland
2019
Link to Abstract

 


 

ClinicianATS Statements, Guidelines and Reports

ATS Statements, Guidelines and Reports are printed in the American Journal of Respiratory and Critical Care Medicine. These documents are also available in PDF format. You may download one copy of any ATS document; there is no charge for this service. For examples, please Click Here.

An American Thoracic Society Official Research Statement: Future Directions in Lung Fibrosis Research