State of progress in treating cystic fibrosis respiratory disease (Q34375926)

1 August 2017

5 references

13 March 2019

21 March 2019

22 April 2019

28 January 2021

6 February 2021

State of progress in treating cystic fibrosis respiratory disease (English)

1 reference

1 August 2017

4 references

13 March 2019

21 March 2019

22 April 2019

28 January 2021

1 reference

6 February 2021

Patrick A Flume

1

1 reference

1 August 2017

Donald R Van Devanter

series ordinal

2

1 reference

1 August 2017

language of work or name

English

0 references

publication date

10 August 2012

6 references

1 August 2017

13 March 2019

21 March 2019

22 April 2019

28 January 2021

6 February 2021

6 references

1 August 2017

13 March 2019

21 March 2019

22 April 2019

28 January 2021

6 February 2021

10

1 reference

1 August 2017

88

1 reference

Creative Commons Attribution 2.0 Generic

1 August 2017

5 references

13 March 2019

21 March 2019

22 April 2019

28 January 2021

on focus list of Wikimedia project

6 February 2021

copyright status

copyrighted

0 references

ScienceSource

0 references

exact match

https://scigraph.springernature.com/pub.10.1186/1741-7015-10-88

0 references

cites work

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6 July 2018

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Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation

6 July 2018

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Progress in gene and cell therapy for cystic fibrosis lung disease

6 July 2018

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Effect of dornase alfa on inflammation and lung function: potential role in the early treatment of cystic fibrosis

6 July 2018

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A CFTR potentiator in patients with cystic fibrosis and the G551D mutation

6 July 2018

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Stratified assessment of the role of inhaled hypertonic saline in reducing cystic fibrosis pulmonary exacerbations: a retrospective analysis

6 July 2018

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

6 July 2018

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Cystic fibrosis lung transplantation

6 July 2018

1 reference

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Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis.

6 July 2018

1 reference

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P2Y receptors in health and disease

6 July 2018

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Clinical use of dornase alpha is associated with a slower rate of FEV1 decline in cystic fibrosis.

6 July 2018

1 reference

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Nebulized hypertonic saline decreases IL-8 in sputum of patients with cystic fibrosis

6 July 2018

1 reference

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Denufosol tetrasodium in patients with cystic fibrosis and normal to mildly impaired lung function

6 July 2018

1 reference

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Airway-rehydrating agents for the treatment of cystic fibrosis: past, present, and future

6 July 2018

1 reference

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

6 July 2018

1 reference

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Role of epithelial HCO3⁻ transport in mucin secretion: lessons from cystic fibrosis

6 July 2018

1 reference

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

6 July 2018

1 reference

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Trends in the use of routine therapies in cystic fibrosis: 1995-2005.

6 July 2018

1 reference

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Pulmonary outcome prediction (POP) tools for cystic fibrosis patients.

6 July 2018

1 reference

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Cystic Fibrosis Pulmonary Guidelines

6 July 2018

1 reference

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An 18-month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis

6 July 2018

1 reference

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Location and duration of treatment of cystic fibrosis respiratory exacerbations do not affect outcomes

6 July 2018

1 reference

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Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation

6 July 2018

1 reference

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Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis.

6 July 2018

1 reference

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

6 July 2018

1 reference

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Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis

6 July 2018

1 reference

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Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations

6 July 2018

1 reference

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A phase II study on safety and efficacy of high-dose N-acetylcysteine in patients with cystic fibrosis

6 July 2018

1 reference

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Airway-clearance therapy guidelines and implementation

6 July 2018

1 reference

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Nebulised hypertonic saline for cystic fibrosis

6 July 2018

1 reference

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Cystic fibrosis pulmonary guidelines: airway clearance therapies

6 July 2018

1 reference

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Nasal and sinus disease in cystic fibrosis

6 July 2018

1 reference

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Inhaled Aztreonam Lysine for Chronic Airway Pseudomonas aeruginosa in Cystic Fibrosis

6 July 2018

1 reference

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Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report

6 July 2018

1 reference

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PTC124 is an orally bioavailable compound that promotes suppression of the human CFTR-G542X nonsense allele in a CF mouse model

6 July 2018

1 reference

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Clinical use of Ibuprofen is associated with slower FEV1 decline in children with cystic fibrosis

6 July 2018

1 reference

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Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health.

6 July 2018

1 reference

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Inhalation of Moli1901 in patients with cystic fibrosis.

6 July 2018

1 reference

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Exacerbations in cystic fibrosis. 1: Epidemiology and pathogenesis

6 July 2018

1 reference

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Evidence for airway surface dehydration as the initiating event in CF airway disease

6 July 2018

1 reference

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Anti-inflammatory effects of azithromycin in cystic fibrosis airway epithelial cells

6 July 2018

1 reference

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Sodium channel blockers for cystic fibrosis

6 July 2018

1 reference

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Cystic fibrosis pulmonary exacerbations

6 July 2018

1 reference

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Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

6 July 2018

1 reference

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EM703 improves bleomycin-induced pulmonary fibrosis in mice by the inhibition of TGF-beta signaling in lung fibroblasts

6 July 2018

1 reference

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Azithromycin exhibits bactericidal effects on Pseudomonas aeruginosa through interaction with the outer membrane

6 July 2018

1 reference

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A pilot study of the effect of inhaled buffered reduced glutathione on the clinical status of patients with cystic fibrosis

6 July 2018

1 reference

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Treatment with tobramycin solution for inhalation reduces hospitalizations in young CF subjects with mild lung disease

6 July 2018

1 reference

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The phenotypic consequences of CFTR mutations

6 July 2018

1 reference

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The role of inflammation in the pathophysiology of CF lung disease

6 July 2018

1 reference

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Effects of tobramycin solution for inhalation on global ratings of quality of life in patients with cystic fibrosis and Pseudomonas aeruginosa infection

6 July 2018

1 reference

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Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis

6 July 2018

1 reference

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Predictive 5-year survivorship model of cystic fibrosis

6 July 2018

1 reference

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The osmolyte xylitol reduces the salt concentration of airway surface liquid and may enhance bacterial killing

6 July 2018

1 reference

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Coordinated clearance of periciliary liquid and mucus from airway surfaces.

6 July 2018

1 reference

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Effect of increasing doses of hypertonic saline on mucociliary clearance in patients with cystic fibrosis

6 July 2018

1 reference

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Cystic fibrosis: genotypic and phenotypic variations

6 July 2018

1 reference

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Prognosis in cystic fibrosis treated with continuous flucloxacillin from the neonatal period

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Role of interleukin-8 (IL-8) and an inhibitory effect of erythromycin on IL-8 release in the airways of patients with chronic airway diseases

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Profiles of outer membrane proteins and lipopolysaccharide of Pseudomonas aeruginosa grown in the presence of sub-MICs of macrolide antibiotics and their relation to enhanced serum sensitivity.

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Effect of high-dose ibuprofen in patients with cystic fibrosis.

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Frequent antibiotic therapy improves survival of cystic fibrosis patients with chronic Pseudomonas aeruginosa infection

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Management of Pseudomonas aeruginosa lung infection in Danish cystic fibrosis patients.

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Erythromycin-induced suppression of pulmonary antibacterial defenses. A potential mechanism of superinfection in the lung

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Identification of the cystic fibrosis gene: chromosome walking and jumping

6 July 2018

1 reference

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Identification of the cystic fibrosis gene: genetic analysis

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Prevention of chronic Pseudomonas aeruginosa colonisation in cystic fibrosis by early treatment

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Cystic fibrosis: molecular biology and therapeutic implications

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Action of nonsteroidal, anti-inflammatory drugs on human and rat peripheral leucocyte migration in vitro

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Long-term inhaled dry powder mannitol in cystic fibrosis: an international randomized study.

6 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Reduced mortality in cystic fibrosis patients treated with tobramycin inhalation solution

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis: the ELITE trial

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening.

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Bronchiectasis in an asymptomatic infant with cystic fibrosis diagnosed following newborn screening.

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening.

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Relationship between inhaled corticosteroid therapy and rate of lung function decline in children with cystic fibrosis

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Early aggressive eradication therapy for intermittent Pseudomonas aeruginosa airway colonization in cystic fibrosis patients: 15 years experience

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Risk factors for rate of decline in forced expiratory volume in one second in children and adolescents with cystic fibrosis.

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Macrolide resistance of Staphylococcus aureus and Haemophilus species associated with long-term azithromycin use in cystic fibrosis

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Mucus clearance and lung function in cystic fibrosis with hypertonic saline

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Dornase alfa reduces air trapping in children with mild cystic fibrosis lung disease: a quantitative analysis.

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Macrolides with promotive activity of monocyte to macrophage differentiation

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Evaluation of nebulised hypertonic saline (7%) as an adjunct to physiotherapy in patients with stable bronchiectasis

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

One-year outcome after severe pulmonary exacerbation in adults with cystic fibrosis.

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

Azithromycin blocks neutrophil recruitment in Pseudomonas endobronchial infection

27 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3425089

A phase I trial of intranasal Moli1901 for cystic fibrosis

27 October 2018

1 reference

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27 October 2018

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Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality.

27 October 2018

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Pharmacology of INS37217 [P(1)-(uridine 5')-P(4)- (2'-deoxycytidine 5')tetraphosphate, tetrasodium salt], a next-generation P2Y(2) receptor agonist for the treatment of cystic fibrosis

27 October 2018

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27 October 2018

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Impact of recent pulmonary exacerbations on quality of life in patients with cystic fibrosis.

27 October 2018

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A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities

27 October 2018

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Effect of inhaled tobramycin on early Pseudomonas aeruginosa colonisation in patients with cystic fibrosis

27 October 2018

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Genotypic characterization of Pseudomonas aeruginosa strains recovered from patients with cystic fibrosis after initial and subsequent colonization

27 October 2018

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27 October 2018

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Effects of non-steroidal anti-inflammatory agents on human neutrophil functions in vitro and in vivo

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9 December 2018

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Effect of continuous antistaphylococcal therapy on the rate of P. aeruginosa acquisition in patients with cystic fibrosis

9 December 2018

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12 December 2020

alpha1-Antitrypsin inhalation reduces airway inflammation in cystic fibrosis patients

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12 December 2020

Inhalation of dry powder mannitol improves clearance of mucus in patients with bronchiectasis

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12 December 2020

Long-term azithromycin may improve lung function in children with cystic fibrosis

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12 December 2020

Aerosol α1 -antitrypsin treatment for cystic fibrosis

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12 December 2020

The influence of azithromycin on the biofilm formation of Pseudomonas aeruginosa in vitro

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12 December 2020

Ibuprofen modifies the inflammatory response of the murine lung to Pseudomonas aeruginosa

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12 December 2020

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

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12 December 2020

Mislocalization of ΔF508 CFTR in cystic fibrosis sweat gland

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12 December 2020

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12 December 2020

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12 December 2020

Erythromycin inhibits neutrophil chemotaxis in bronchoalveoli of diffuse panbronchiolitis

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12 December 2020

Influence of macrolides on mucoid alginate biosynthetic enzyme from Pseudomonas aeruginosa

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12 December 2020

Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis

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12 December 2020

Mechanisms and applications of hypertonic saline.

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12 December 2020

Long term effects of denufosol tetrasodium in patients with cystic fibrosis

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12 December 2020

Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis

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12 December 2020

Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group

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12 December 2020

High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial

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12 December 2020

Protease-antiprotease imbalance in the lungs of children with cystic fibrosis

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12 December 2020

Inhaled corticosteroids and lower lung function decline in young children with cystic fibrosis

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12 December 2020

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

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12 December 2020

Alternate pathways for chloride conductance activation in normal and cystic fibrosis airway epithelial cells

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12 December 2020

Systemic deficiency of glutathione in cystic fibrosis

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12 December 2020

Population variation of common cystic fibrosis mutations. The Cystic Fibrosis Genetic Analysis Consortium

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12 December 2020

Early pulmonary inflammation in infants with cystic fibrosis

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12 December 2020

Levofloxacin inhalation solution (MP-376) in patients with cystic fibrosis with Pseudomonas aeruginosa

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12 December 2020

Quality of well-being before and after antibiotic treatment of pulmonary exacerbation in patients with cystic fibrosis

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12 December 2020

Risk of persistent growth impairment after alternate-day prednisone treatment in children with cystic fibrosis

1 reference

12 December 2020

Erythromycin reduces neutrophils and neutrophil-derived elastolytic-like activity in the lower respiratory tract of bronchiolitis patients

1 reference

12 December 2020

Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function

1 reference

12 December 2020

A multicenter study of alternate-day prednisone therapy in patients with cystic fibrosis. Cystic Fibrosis Foundation Prednisone Trial Group

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12 December 2020

Fractional analysis of bronchoalveolar lavage fluid cytology in cystic fibrosis patients with normal lung function. Bronchoalveolar lavage for the evaluation of anti-inflammatory treatment (BEAT) study group

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12 December 2020

Lung transplantation in cystic fibrosis

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12 December 2020

Identifiers

DOI

10.1186/1741-7015-10-88

1 reference

Dimensions Publication ID

1 August 2017

0 references

1 reference

1 August 2017

1 reference