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Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy

Journal of cystic fibrosis, 2015-07, Vol.14 (4), p.419-430

2015

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A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial

The lancet respiratory medicine, 2014-07, Vol.2 (7), p.527-538

2014

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Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis

The lancet respiratory medicine, 2016-08, Vol.4 (8), p.617-626

2016

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Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial

Journal of cystic fibrosis, 2011-01, Vol.10 (1), p.54-61

2011

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Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp- CFTR mutation: a phase 3, open-label extension study (PERSIST)

The lancet respiratory medicine, 2014-11, Vol.2 (11), p.902-910

2014

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Genetic Modifiers of Lung Disease in Cystic Fibrosis

The New England journal of medicine, 2005-10, Vol.353 (14), p.1443-1453

2005

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Risk Factors For Rate of Decline in Forced Expiratory Volume in One Second in Children and Adolescents with Cystic Fibrosis

The Journal of pediatrics, 2007-08, Vol.151 (2), p.134-139.e1

2007

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Improvements in Lung Function and Height among Cohorts of 6-Year-Olds with Cystic Fibrosis from 1994 to 2012

The Journal of pediatrics, 2014-12, Vol.165 (6), p.1091-1097.e2

2014

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Emergence of Linezolid-Resistant Staphylococcus aureus after Prolonged Treatment of Cystic Fibrosis Patients in Cleveland, Ohio

Antimicrobial Agents and Chemotherapy, 2011-04, Vol.55 (4), p.1684-1692

2011

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Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

The New England journal of medicine, 2015-07, Vol.373 (3), p.220-231

2015

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Increasing life expectancy in cystic fibrosis: Advances and challenges

Pediatric pulmonology, 2022-02, Vol.57 (S1), p.S5-S12

2022

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Association of Socioeconomic Status with the Use of Chronic Therapies and Healthcare Utilization in Children with Cystic Fibrosis

The Journal of pediatrics, 2009-11, Vol.155 (5), p.634-639.e4

2009

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Probability of Treatment Following Acute Decline in Lung Function in Children with Cystic Fibrosis is Related to Baseline Pulmonary Function

The Journal of pediatrics, 2013-10, Vol.163 (4), p.1152-1157.e2

2013

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Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution

Human gene therapy, 2004-12, Vol.15 (12), p.1255-1269

2004

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What Is Cystic Fibrosis?

JAMA : the journal of the American Medical Association, 2022-01, Vol.327 (2), p.191

2022

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Normalized T1 magnetic resonance imaging for assessment of regional lung function in adult cystic fibrosis patients--a cross-sectional study

PloS one, 2013-09, Vol.8 (9), p.e73286

2013

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Assessing time to pulmonary function benefit following antibiotic treatment of acute cystic fibrosis exacerbations

Respiratory research, 2010-10, Vol.11 (1), p.137-137, Article 137

2010

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Association between respiratory tract methicillin-resistant Staphylococcus aureus and survival in cystic fibrosis

JAMA : the journal of the American Medical Association, 2010-06, Vol.303 (23), p.2386

2010

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The role of inflammation in the pathophysiology of CF lung disease

Clinical reviews in allergy & immunology, 2002-08, Vol.23 (1), p.5-28

2002

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Disease progression in patients with cystic fibrosis treated with ivacaftor: Data from national US and UK registries

Journal of cystic fibrosis, 2020-01, Vol.19 (1), p.68-79

2020

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The Efficacy and Safety of Meropenem and Tobramycin vs Ceftazidime and Tobramycin in the Treatment of Acute Pulmonary Exacerbations in Patients With Cystic Fibrosis

Chest, 2005-10, Vol.128 (4), p.2336-2346

2005

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Classifying Severity of Cystic Fibrosis Lung Disease Using Longitudinal Pulmonary Function Data

American journal of respiratory and critical care medicine, 2006-10, Vol.174 (7), p.780-786

2006

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Inflammation in cystic fibrosis lung disease: Pathogenesis and therapy

A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial

Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study

Efficacy and safety of lumacaftor/ivacaftor combination therapy in patients with cystic fibrosis homozygous for Phe508del CFTR by pulmonary function subgroup: a pooled analysis

Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial

Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: The EAGER trial

Genomic heterogeneity underlies multidrug resistance in Pseudomonas aeruginosa: A population-level analysis beyond susceptibility testing

Long-term safety and efficacy of ivacaftor in patients with cystic fibrosis who have the Gly551Asp- CFTR mutation: a phase 3, open-label extension study (PERSIST)

Genetic Modifiers of Lung Disease in Cystic Fibrosis

Risk Factors For Rate of Decline in Forced Expiratory Volume in One Second in Children and Adolescents with Cystic Fibrosis

Improvements in Lung Function and Height among Cohorts of 6-Year-Olds with Cystic Fibrosis from 1994 to 2012

Emergence of Linezolid-Resistant Staphylococcus aureus after Prolonged Treatment of Cystic Fibrosis Patients in Cleveland, Ohio

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

Increasing life expectancy in cystic fibrosis: Advances and challenges

Association of Socioeconomic Status with the Use of Chronic Therapies and Healthcare Utilization in Children with Cystic Fibrosis

Probability of Treatment Following Acute Decline in Lung Function in Children with Cystic Fibrosis is Related to Baseline Pulmonary Function

Compacted DNA nanoparticles administered to the nasal mucosa of cystic fibrosis subjects are safe and demonstrate partial to complete cystic fibrosis transmembrane regulator reconstitution

What Is Cystic Fibrosis?

Normalized T1 magnetic resonance imaging for assessment of regional lung function in adult cystic fibrosis patients--a cross-sectional study

Assessing time to pulmonary function benefit following antibiotic treatment of acute cystic fibrosis exacerbations

Association between respiratory tract methicillin-resistant Staphylococcus aureus and survival in cystic fibrosis

The role of inflammation in the pathophysiology of CF lung disease

Disease progression in patients with cystic fibrosis treated with ivacaftor: Data from national US and UK registries

The Efficacy and Safety of Meropenem and Tobramycin vs Ceftazidime and Tobramycin in the Treatment of Acute Pulmonary Exacerbations in Patients With Cystic Fibrosis

Classifying Severity of Cystic Fibrosis Lung Disease Using Longitudinal Pulmonary Function Data

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