(Q37128814)

18 August 2017

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title

What's new in cystic fibrosis? From treating symptoms to correction of the basic defect (English)

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18 August 2017

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2

François Vermeulen

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18 August 2017

Marijke Proesmans

1

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18 August 2017

Kris De Boeck

series ordinal

3

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18 August 2017

publication date

4 April 2008

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European Journal of Pediatrics

18 August 2017

published in

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18 August 2017

volume

167

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18 August 2017

issue

8

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18 August 2017

page(s)

839-849

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Role of respiratory syncytial virus in early hospitalizations for respiratory distress of young infants with cystic fibrosis

18 August 2017

cites work

1 reference

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SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis

21 January 2018

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Pharmacotherapy of the ion transport defect in cystic fibrosis: role of purinergic receptor agonists and other potential therapeutics

21 January 2018

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Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional study

21 January 2018

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Five Percent of Normal Cystic Fibrosis Transmembrane Conductance Regulator mRNA Ameliorates the Severity of Pulmonary Disease in Cystic Fibrosis

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00431-008-0693-2

Spectrum and mode of action of azithromycin (CP-62,993), a new 15-membered-ring macrolide with improved potency against gram-negative organisms

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00431-008-0693-2

Repeated Aerosolized AAV-CFTR for Treatment of Cystic Fibrosis: A Randomized Placebo-Controlled Phase 2B Trial

21 January 2018

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12 December 2020

Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists

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12 December 2020

Early eradication therapy against Pseudomonas aeruginosa in cystic fibrosis patients.

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12 December 2020

Pharmacotherapy of the ion transport defect in cystic fibrosis.

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12 December 2020

Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat

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12 December 2020

Use of lung transplantation survival models to refine patient selection in cystic fibrosis.

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12 December 2020

alpha1-Antitrypsin inhalation reduces airway inflammation in cystic fibrosis patients

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12 December 2020

Gene therapy for cystic fibrosis lung disease: current status and future perspectives

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12 December 2020

Azithromycin blocks quorum sensing and alginate polymer formation and increases the sensitivity to serum and stationary-growth-phase killing of Pseudomonas aeruginosa and attenuates chronic P. aeruginosa lung infection in Cftr(-/-) mice

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12 December 2020

Pharmacologic therapy for stop mutations: how much CFTR activity is enough?

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12 December 2020

Inhaled corticosteroids for cystic fibrosis.

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12 December 2020

High-dose oral N-acetylcysteine, a glutathione prodrug, modulates inflammation in cystic fibrosis

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12 December 2020

Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects

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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

Potential of macrolide antibiotics to inhibit protein synthesis of Pseudomonas aeruginosa: suppression of virulence factors and stress response

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12 December 2020

Design, synthesis, and structure-activity relationships of novel 2-substituted pyrazinoylguanidine epithelial sodium channel blockers: drugs for cystic fibrosis and chronic bronchitis

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12 December 2020

Azithromycin inhibits quorum sensing in Pseudomonas aeruginosa

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12 December 2020

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

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12 December 2020

A double-blind randomized placebo-controlled phase III study of a Pseudomonas aeruginosa flagella vaccine in cystic fibrosis patients

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12 December 2020

A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function

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12 December 2020

Genetics and pulmonary medicine. 1. The genetics of cystic fibrosis lung disease

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12 December 2020

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

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12 December 2020

A phase I trial of intranasal Moli1901 for cystic fibrosis

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12 December 2020

A novel host defense system of airways is defective in cystic fibrosis

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12 December 2020

Safety of aerosolized INS 365 in patients with mild to moderate cystic fibrosis: results of a phase I multi-center study

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12 December 2020

Airway surface dehydration in cystic fibrosis: pathogenesis and therapy

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12 December 2020

Factors influencing outcomes in cystic fibrosis: a center-based analysis.

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12 December 2020

The effect of inhaled mannitol on bronchial mucus clearance in cystic fibrosis patients: a pilot study

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12 December 2020

The involvement of cell-to-cell signals in the development of a bacterial biofilm

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12 December 2020

Nonsense-mediated mRNA decay in health and disease

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12 December 2020

Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial.

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12 December 2020

Prospective, controlled study of a polyvalent pseudomonas vaccine in cystic fibrosis--three year results

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12 December 2020

Correlation of sweat chloride concentration with classes of the cystic fibrosis transmembrane conductance regulator gene mutations

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12 December 2020

Effect of subinhibitory concentrations of macrolides on expression of flagellin in Pseudomonas aeruginosa and Proteus mirabilis

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12 December 2020

Do inhaled corticosteroids impair long-term growth in prepubertal cystic fibrosis patients?

1 reference

12 December 2020

Cystic fibrosis mortality and survival in the UK: 1947-2003

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12 December 2020

Survival of lung transplant patients with cystic fibrosis harboring panresistant bacteria other than Burkholderia cepacia, compared with patients harboring sensitive bacteria

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12 December 2020

Novel tobramycin inhalation powder in cystic fibrosis subjects: pharmacokinetics and safety

1 reference

12 December 2020

Adult cystic fibrosis

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12 December 2020

The role of inflammation in the pathophysiology of CF lung disease

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12 December 2020

Mucus clearance and lung function in cystic fibrosis with hypertonic saline

1 reference

12 December 2020

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

1 reference

12 December 2020

Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections

1 reference

12 December 2020

Lung transplantation and survival in children with cystic fibrosis

1 reference

12 December 2020

Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX.

1 reference

12 December 2020

Potential impact of newborn screening for cystic fibrosis on child survival: a systematic review and analysis

1 reference

12 December 2020

Safety, tolerability, and pharmacokinetics of PTC124, a nonaminoglycoside nonsense mutation suppressor, following single- and multiple-dose administration to healthy male and female adult volunteers

1 reference

12 December 2020

Cystic fibrosis transmembrane conductance regulator and the etiology and pathogenesis of cystic fibrosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Restoration of W1282X CFTR activity by enhanced expression

1 reference

12 December 2020

Does centralized treatment of cystic fibrosis increase the risk of Pseudomonas aeruginosa infection?

1 reference

12 December 2020

Curcumin opens cystic fibrosis transmembrane conductance regulator channels by a novel mechanism that requires neither ATP binding nor dimerization of the nucleotide-binding domains

1 reference

12 December 2020

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

1 reference

12 December 2020

Advances in lung transplantation for patients who have cystic fibrosis

1 reference

12 December 2020

Phase 2 randomized safety and efficacy trial of nebulized denufosol tetrasodium in cystic fibrosis.

1 reference

12 December 2020

Influence of macrolides on mucoid alginate biosynthetic enzyme from Pseudomonas aeruginosa

1 reference

12 December 2020

Correction of the CF defect by curcumin: hypes and disappointments.

1 reference

12 December 2020

Cytokines and inflammatory mediators in cystic fibrosis.

1 reference

12 December 2020

Update on pathogenesis of cystic fibrosis lung disease

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12 December 2020

Prediction of cellular immune responses against CFTR in patients with cystic fibrosis after gene therapy.

1 reference

12 December 2020

Living donor lung transplantation

1 reference

12 December 2020

Evaluation of different compounds as quorum sensing inhibitors in Pseudomonas aeruginosa

1 reference

12 December 2020

Antibiotic treatment of initial colonization with Pseudomonas aeruginosa postpones chronic infection and prevents deterioration of pulmonary function in cystic fibrosis

1 reference

12 December 2020

Effect of Treatment with Dornase Alpha on Airway Inflammation in Patients with Cystic Fibrosis

1 reference

12 December 2020

Inhalation of Moli1901 in patients with cystic fibrosis.

1 reference

12 December 2020

Gentamicin-Induced Correction of CFTR Function in Patients with Cystic Fibrosis andCFTRStop Mutations

1 reference

12 December 2020

Eradication of early Pseudomonas aeruginosa infection

1 reference

12 December 2020

Sildenafil (Viagra) corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis.

1 reference

12 December 2020

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

1 reference

12 December 2020

A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis

1 reference

12 December 2020

Infant care patterns at epidemiologic study of cystic fibrosis sites that achieve superior childhood lung function.

1 reference

12 December 2020

Long term effects of azithromycin in patients with cystic fibrosis: A double blind, placebo controlled trial

1 reference

12 December 2020

Vaccination of cystic fibrosis patients against Pseudomonas aeruginosa reduces the proportion of patients infected and delays time to infection

1 reference

12 December 2020

Genotype-phenotype correlations in cystic fibrosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Influence of neonatal screening and centralized treatment on long-term clinical outcome and survival of CF patients

1 reference

12 December 2020

Structure and function of the CFTR chloride channel

1 reference

12 December 2020

Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: a European consensus.

1 reference

12 December 2020

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

1 reference

12 December 2020

The use of human deoxyribonuclease (rhDNase) in the management of cystic fibrosis.

1 reference

12 December 2020

From registry to quality management: the German Cystic Fibrosis Quality Assessment project 1995 2006.

1 reference

12 December 2020

Long term azithromycin in children with cystic fibrosis: a randomised, placebo-controlled crossover trial

1 reference

12 December 2020

Quorum-sensing blockade as a strategy for enhancing host defences against bacterial pathogens.

1 reference

12 December 2020

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

1 reference

12 December 2020

The effect of topical benzamil and amiloride on nasal potential difference in cystic fibrosis

1 reference

12 December 2020

Rescue of DeltaF508 and other misprocessed CFTR mutants by a novel quinazoline compound

1 reference

12 December 2020

Dry powder inhalation of colistin in cystic fibrosis patients: a single dose pilot study.

1 reference

12 December 2020

Lung transplantation for cystic fibrosis: current concepts and one center's experience

1 reference

12 December 2020

Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial

1 reference

12 December 2020

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

1 reference

12 December 2020

Gene therapy progress and prospects: cystic fibrosis

1 reference

12 December 2020

A multicenter, randomized, double-blind, placebo-controlled trial to evaluate the metabolic and respiratory effects of growth hormone in children with cystic fibrosis.

1 reference

12 December 2020

PTC124 targets genetic disorders caused by nonsense mutations

1 reference

12 December 2020

Calcium homeostasis is abnormal in cystic fibrosis airway epithelial cells but is normalized after rescue of F508del-CFTR.

1 reference

12 December 2020

Factors associated with poor pulmonary function: cross-sectional analysis of data from the ERCF. European Epidemiologic Registry of Cystic Fibrosis

1 reference

12 December 2020

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

1 reference

12 December 2020

Anti-inflammatory activity of macrolides: a new therapeutic potential?

1 reference

12 December 2020

10.1007/S00431-008-0693-2

Identifiers

DOI

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18 August 2017

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