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Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist

The Journal of clinical investigation, 2023-05, Vol.133 (10), p.1-16

2023

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Airway microbiota across age and disease spectrum in cystic fibrosis

The European respiratory journal, 2017-11, Vol.50 (5), p.1700832-1700832

2017

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Tgf-beta downregulation of distinct chloride channels in cystic fibrosis-affected epithelia

PloS one, 2014-09, Vol.9 (9), p.e106842-e106842

2014

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CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity

Respiratory research, 2023-08, Vol.24 (1), p.198-198, Article 198

2023

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Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease

Annals of the American Thoracic Society, 2016-11, Vol.13 (11), p.1923-1931

2016

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Phenotypic Alteration of an Established Human Airway Cell Line by Media Selection

International journal of molecular sciences, 2023-01, Vol.24 (2), p.1246

2023

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Rapid therapeutic advances in CFTR modulator science

Pediatric pulmonology, 2018-11, Vol.53 (S3), p.S4-S11

Clancy, John P.

2018

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Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial

Human gene therapy, 2007-08, Vol.18 (8), p.726-732

2007

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Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation

PloS one, 2013-07, Vol.8 (7), p.e66955

2013

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Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function

PloS one, 2013-09, Vol.8 (9), p.e73905-e73905

2013

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Safety and efficacy of alternative alglucosidase alfa regimens in Pompe disease

Neuromuscular disorders : NMD, 2015-04, Vol.25 (4), p.321-332

2015

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A semiparametric approach to estimate rapid lung function decline in cystic fibrosis

Annals of epidemiology, 2013-12, Vol.23 (12), p.771-777

2013

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Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence

Therapeutic advances in respiratory disease, 2013-10, Vol.7 (5), p.288-296

2013

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Personalized Medicine in Cystic Fibrosis: Dawning of a New Era

American journal of respiratory and critical care medicine, 2012-10, Vol.186 (7), p.593-597

2012

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Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial

American journal of respiratory and critical care medicine, 2022-03, Vol.205 (5), p.529-539

2022

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Data driven decision making to characterize clinical personas of parents of children with cystic fibrosis: a mixed methods study

BMC pulmonary medicine, 2020-06, Vol.20 (1), p.174-174, Article 174

2020

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Seasonality, mediation and comparison (SMAC) methods to identify influences on lung function decline

MethodsX, 2021-01, Vol.8, p.101313-101313, Article 101313

2021

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Improving Detection of Rapid Cystic Fibrosis Disease Progression-Early Translation of a Predictive Algorithm Into a Point-of-Care Tool

IEEE journal of translational engineering in health and medicine, 2019-01, Vol.7, p.1-8

2019

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Reduced Sodium Transport With Nasal Administration of the Prostasin Inhibitor Camostat in Subjects With Cystic Fibrosis

Chest, 2013-07, Vol.144 (1), p.200-207

2013

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Social-environmental phenotypes of rapid cystic fibrosis lung disease progression in adolescents and young adults living in the United States

Environmental advances, 2023-12, Vol.14, p.100449, Article 100449

2023

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Management of Pseudomonas aeruginosa infection in cystic fibrosis patients using inhaled antibiotics with a focus on nebulized liposomal amikacin

Future microbiology, 2015-12, Vol.10 (12), p.1901-1912

2015

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Sweat Chloride Testing

JAMA : the journal of the American Medical Association, 2019-02, Vol.321 (7), p.701

2019

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Home Monitoring of Patients with Cystic Fibrosis to Identify and Treat Acute Pulmonary Exacerbations. eICE Study Results

American journal of respiratory and critical care medicine, 2017-11, Vol.196 (9), p.1144-1151

2017

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Bayesian regularization for a nonstationary Gaussian linear mixed effects model

Statistics in medicine, 2022-02, Vol.41 (4), p.681-697

2022

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Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood

American journal of respiratory and critical care medicine, 2017-08, Vol.196 (4), p.471-478

2017

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Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist

Airway microbiota across age and disease spectrum in cystic fibrosis

Tgf-beta downregulation of distinct chloride channels in cystic fibrosis-affected epithelia

CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity

Ultrashort Echo-Time Magnetic Resonance Imaging Is a Sensitive Method for the Evaluation of Early Cystic Fibrosis Lung Disease

Phenotypic Alteration of an Established Human Airway Cell Line by Media Selection

Rapid therapeutic advances in CFTR modulator science

Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial

Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation

Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function

Safety and efficacy of alternative alglucosidase alfa regimens in Pompe disease

A semiparametric approach to estimate rapid lung function decline in cystic fibrosis

Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence

Personalized Medicine in Cystic Fibrosis: Dawning of a New Era

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial

Data driven decision making to characterize clinical personas of parents of children with cystic fibrosis: a mixed methods study

Seasonality, mediation and comparison (SMAC) methods to identify influences on lung function decline

Improving Detection of Rapid Cystic Fibrosis Disease Progression-Early Translation of a Predictive Algorithm Into a Point-of-Care Tool

Reduced Sodium Transport With Nasal Administration of the Prostasin Inhibitor Camostat in Subjects With Cystic Fibrosis

Social-environmental phenotypes of rapid cystic fibrosis lung disease progression in adolescents and young adults living in the United States

Management of Pseudomonas aeruginosa infection in cystic fibrosis patients using inhaled antibiotics with a focus on nebulized liposomal amikacin

Sweat Chloride Testing

Home Monitoring of Patients with Cystic Fibrosis to Identify and Treat Acute Pulmonary Exacerbations. eICE Study Results

Bayesian regularization for a nonstationary Gaussian linear mixed effects model

Phenotypes of Rapid Cystic Fibrosis Lung Disease Progression during Adolescence and Young Adulthood

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