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The Th17 Pathway in Cystic Fibrosis Lung Disease

American journal of respiratory and critical care medicine, 2011-07, Vol.184 (2), p.252-258

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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High Rhinovirus Burden in Lower Airways of Children With Cystic Fibrosis

Chest, 2013-03, Vol.143 (3), p.782-790

2013

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Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

The New England journal of medicine, 2017-11, Vol.377 (21), p.2024-2035

2017

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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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Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation

American journal of respiratory and critical care medicine, 2013-06, Vol.187 (11), p.1219-1225

2013

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COUNTERPOINT: In the Era of Cystic Fibrosis Transmembrane Regulator Protein Modulator Therapy, Are the Treatment Goals for Adults Now Different From Those for Children With Cystic Fibrosis? No

Chest, 2022-01, Vol.161 (1), p.21-24

2022

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Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function Mutation: A Phase 3b, Randomized, Placebo-controlled Study

American journal of respiratory and critical care medicine, 2022-12, Vol.206 (11), p.1361-1369

2022

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Clinical pharmacokinetics and dose recommendations for posaconazole gastroresistant tablets in children with cystic fibrosis

Journal of antimicrobial chemotherapy, 2021-11, Vol.76 (12), p.3247-3254

2021

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Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)

Journal of cystic fibrosis, 2021-09, Vol.20 (5), p.810-819

2021

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Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial

The lancet respiratory medicine, 2015-09, Vol.3 (9), p.684-691

2015

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Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged ⩾6 Years with Cystic Fibrosis and at Least One F508del Allele: A Phase 3, Open-Label Clinical Trial

American journal of respiratory and critical care medicine, 2023-07, Vol.208 (1), p.68-78

2023

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Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis

Thorax, 2021-12, Vol.76 (12), p.1255-1265

2021

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Update on Clinical Outcomes of Highly Effective Modulator Therapy

Clinics in chest medicine, 2022-12, Vol.43 (4), p.677-695

2022

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How representative are clinical trial cohorts of the general CF population? Implications for trial planning

Journal of cystic fibrosis, 2024-01, Vol.23 (1), p.68-72

2024

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Efficacy and safety of lumacaftor and ivacaftor in patients aged 6–11 years with cystic fibrosis homozygous for F508del-CFTR : a randomised, placebo-controlled phase 3 trial

The lancet respiratory medicine, 2017-07, Vol.5 (7), p.557-567

2017

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Increased incidence and severity of the systemic inflammatory response syndrome in patients deficient in mannose-binding lectin

Intensive care medicine, 2004-07, Vol.30 (7), p.1438-1445

2004

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Developments in multiple breath washout testing in children with cystic fibrosis

Current medical research and opinion, 2017-04, Vol.33 (4), p.613-620

2017

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A Short extension to multiple breath washout provides additional signal of distal airway disease in people with CF: A pilot study

Journal of cystic fibrosis, 2022-01, Vol.21 (1), p.146-154

2022

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Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: A UK registry study

Journal of cystic fibrosis, 2022-01, Vol.21 (1), p.129-135

2022

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Airway remodelling and its relationship to inflammation in cystic fibrosis

Thorax, 2011-07, Vol.66 (7), p.624-629

2011

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Quality, Size, and Composition of Pediatric Endobronchial Biopsies in Cystic Fibrosis

Chest, 2007-06, Vol.131 (6), p.1710-1717

2007

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Cystic fibrosis: bridging the treatment gap in early childhood

The lancet respiratory medicine, 2013-08, Vol.1 (6), p.433-434

Davies, Jane C

2013

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Multiple breath washouts in children can be shortened without compromising quality

The European respiratory journal, 2015-12, Vol.46 (6), p.1814-1816

2015

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The Th17 Pathway in Cystic Fibrosis Lung Disease

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)

High Rhinovirus Burden in Lower Airways of Children With Cystic Fibrosis

Tezacaftor–Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis

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

Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation

COUNTERPOINT: In the Era of Cystic Fibrosis Transmembrane Regulator Protein Modulator Therapy, Are the Treatment Goals for Adults Now Different From Those for Children With Cystic Fibrosis? No

Efficacy and Safety of Elexacaftor/Tezacaftor/Ivacaftor in Children 6 Through 11 Years of Age with Cystic Fibrosis Heterozygous for F508del and a Minimal Function Mutation: A Phase 3b, Randomized, Placebo-controlled Study

Clinical pharmacokinetics and dose recommendations for posaconazole gastroresistant tablets in children with cystic fibrosis

Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID)

Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial

Long-Term Safety and Efficacy of Elexacaftor/Tezacaftor/Ivacaftor in Children Aged ⩾6 Years with Cystic Fibrosis and at Least One F508del Allele: A Phase 3, Open-Label Clinical Trial

Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis

Update on Clinical Outcomes of Highly Effective Modulator Therapy

How representative are clinical trial cohorts of the general CF population? Implications for trial planning

Efficacy and safety of lumacaftor and ivacaftor in patients aged 6–11 years with cystic fibrosis homozygous for F508del-CFTR : a randomised, placebo-controlled phase 3 trial

Increased incidence and severity of the systemic inflammatory response syndrome in patients deficient in mannose-binding lectin

Developments in multiple breath washout testing in children with cystic fibrosis

A Short extension to multiple breath washout provides additional signal of distal airway disease in people with CF: A pilot study

Clinical characteristics of Pseudomonas and Aspergillus co-infected cystic fibrosis patients: A UK registry study

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

Airway remodelling and its relationship to inflammation in cystic fibrosis

Quality, Size, and Composition of Pediatric Endobronchial Biopsies in Cystic Fibrosis

Cystic fibrosis: bridging the treatment gap in early childhood

Multiple breath washouts in children can be shortened without compromising quality

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