(Q36114460)

12 August 2017

1 reference

title

Cystic fibrosis lung disease: genetic influences, microbial interactions, and radiological assessment (English)

1 reference

12 August 2017

0 references

Samuel M Moskowitz

1

1 reference

12 August 2017

Ronald L Gibson

series ordinal

2

1 reference

12 August 2017

Eric L Effmann

series ordinal

3

1 reference

12 August 2017

language of work or name

1 reference

3 May 2005

1 reference

12 August 2017

1 reference

12 August 2017

35

1 reference

12 August 2017

8

1 reference

12 August 2017

739-757

1 reference

https://scigraph.springernature.com/pub.10.1007/s00247-005-1445-3

12 August 2017

0 references

cites work

Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Inflammation and infection in naive human cystic fibrosis airway grafts

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Multiple combination bactericidal antibiotic testing for patients with cystic fibrosis infected with multiresistant strains of Pseudomonas aeruginosa

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

21 January 2018

Cystic fibrosis: 1997.

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Cystic fibrosis: a system for assessing and predicting progression.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Pediatric pulmonary disease: assessment with high-resolution ultrafast CT.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Minimizing radiation dose for pediatric body applications of single-detector helical CT: strategies at a large Children's Hospital

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

In-plane bismuth breast shields for pediatric CT: effects on radiation dose and image quality using experimental and clinical data

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Controlled Trial of Inhaled Budesonide in Patients with Cystic Fibrosis and Chronic BronchopulmonaryPseudomonas aeruginosaInfection

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Cystic fibrosis: scoring system with thin-section CT

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Improved survival among young patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00247-005-1445-3

Rapid characterization of the variable length polythymidine tract in the cystic fibrosis (CFTR) gene: association of the 5T allele with selected CFTR mutations and its incidence in atypical sinopulmonary disease

21 January 2018

1 reference

12 December 2020

Ultrafast computerized tomography of the chest in cystic fibrosis: a new scoring system

1 reference

12 December 2020

Quantitative air-trapping analysis in children with mild cystic fibrosis lung disease

1 reference

12 December 2020

Aerosolized recombinant human DNase in hospitalized cystic fibrosis patients with acute pulmonary exacerbations

1 reference

12 December 2020

Cystic fibrosis in children: HRCT findings and distribution of disease

1 reference

12 December 2020

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

1 reference

12 December 2020

Identification of the cystic fibrosis gene: chromosome walking and jumping

1 reference

12 December 2020

How much do Pseudomonas biofilms contribute to symptoms of pulmonary exacerbation in cystic fibrosis?

1 reference

12 December 2020

Pseudomonas acquisition in young patients with cystic fibrosis: pathophysiology, diagnosis, and management

1 reference

12 December 2020

Allergic bronchopulmonary aspergillosis in cystic fibrosis: role of atopy and response to itraconazole

1 reference

12 December 2020

Routine ventilation scans in children with cystic fibrosis: diagnostic usefulness and prognostic value

1 reference

12 December 2020

Antibiotic susceptibility of multiply resistant Pseudomonas aeruginosa isolated from patients with cystic fibrosis, including candidates for transplantation

1 reference

12 December 2020

Nonopsonic antibodies in cystic fibrosis. Pseudomonas aeruginosa lipopolysaccharide-specific immunoglobulin G antibodies from infected patient sera inhibit neutrophil oxidative responses

1 reference

12 December 2020

An epidemic Burkholderia cepacia complex strain identified in soil

1 reference

12 December 2020

Binding of Pseudomonas aeruginosa to respiratory epithelial cells from patients with various mutations in the cystic fibrosis transmembrane regulator

1 reference

12 December 2020

Osmotic stimuli increase clearance of mucus in patients with mucociliary dysfunction

1 reference

12 December 2020

The chest radiograph in cystic fibrosis: a new scoring system compared with the Chrispin-Norman and Brasfield scores

1 reference

12 December 2020

The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups

1 reference

12 December 2020

Mucoid Pseudomonas aeruginosa in cystic fibrosis: characterization of muc mutations in clinical isolates and analysis of clearance in a mouse model of respiratory infection

1 reference

12 December 2020

Mutations in the cystic fibrosis gene in patients with congenital absence of the vas deferens.

1 reference

12 December 2020

High-resolution computed tomography of the chest in children with cystic fibrosis: support for use as an outcome surrogate

1 reference

12 December 2020

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

1 reference

12 December 2020

Bacterial biofilms: a common cause of persistent infections

1 reference

12 December 2020

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

1 reference

12 December 2020

Dynamics and spatial distribution of beta-lactamase expression in Pseudomonas aeruginosa biofilms

1 reference

12 December 2020

Mucoid Pseudomonas aeruginosa growing in a biofilm in vitro are killed by opsonic antibodies to the mucoid exopolysaccharide capsule but not by antibodies produced during chronic lung infection in cystic fibrosis patients.

1 reference

12 December 2020

Defining a pulmonary exacerbation in cystic fibrosis

1 reference

12 December 2020

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

1 reference

12 December 2020

Long-term study of one hundred five patients with cystic fibrosis; studies made over a five- to fourteen-year period

1 reference

12 December 2020

Pseudomonas aeruginosa anaerobic respiration in biofilms: relationships to cystic fibrosis pathogenesis.

1 reference

12 December 2020

Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.

1 reference

12 December 2020

Endothelial Nitric Oxide Synthase Variants in Cystic Fibrosis Lung Disease

1 reference

12 December 2020

Cystic fibrosis: combined hyperpolarized 3He-enhanced and conventional proton MR imaging in the lung--preliminary observations

1 reference

12 December 2020

Chest physical therapy management of patients with cystic fibrosis. A meta-analysis

1 reference

12 December 2020

A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations

1 reference

12 December 2020

An automated approach to quantitative air trapping measurements in mild cystic fibrosis.

1 reference

12 December 2020

Efficacy and safety of short-term administration of aerosolized recombinant human deoxyribonuclease in patients with cystic fibrosis

1 reference

12 December 2020

Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid.

1 reference

12 December 2020

Reduction of sputum Pseudomonas aeruginosa density by antibiotics improves lung function in cystic fibrosis more than do bronchodilators and chest physiotherapy alone

1 reference

12 December 2020

Changing epidemiology of Pseudomonas aeruginosa infection in Danish cystic fibrosis patients (1974-1995).

1 reference

12 December 2020

Comparative effects of hydrofluoroalkane and chlorofluorocarbon beclomethasone dipropionate inhalation on small airways: assessment with functional helical thin-section computed tomography

1 reference

12 December 2020

High-resolution CT of the lungs in infants and young children

1 reference

12 December 2020

Comparison of a beta-lactam alone versus beta-lactam and an aminoglycoside for pulmonary exacerbation in cystic fibrosis

1 reference

12 December 2020

Variation in a repeat sequence determines whether a common variant of the cystic fibrosis transmembrane conductance regulator gene is pathogenic or benign

1 reference

12 December 2020

Disproportionate distribution of Burkholderia cepacia complex species and transmissibility markers in cystic fibrosis

1 reference

12 December 2020

Early morphologic changes in the lungs of asymptomatic infants and young children with cystic fibrosis.

1 reference

12 December 2020

The use of anti-inflammatory medications in cystic fibrosis: trends and physician attitudes

1 reference

12 December 2020

Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis

1 reference

12 December 2020

Mucus clearance as a primary innate defense mechanism for mammalian airways

1 reference

12 December 2020

Efficacy of the Flutter device for airway mucus clearance in patients with cystic fibrosis

1 reference

12 December 2020

Respiratory tract colonization with Pseudomonas aeruginosa in cystic fibrosis: correlations between anti-Pseudomonas aeruginosa antibody levels and pulmonary function

1 reference

12 December 2020

Cystic fibrosis. An overview

1 reference

12 December 2020

Antimicrobial peptides and proteins in the CF airway

1 reference

12 December 2020

Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate

1 reference

12 December 2020

Association of mannose-binding lectin gene heterogeneity with severity of lung disease and survival in cystic fibrosis

1 reference

12 December 2020

Cystic Fibrosis: When Should High-resolution Computed Tomography of the Chest Be Obtained?

1 reference

12 December 2020

A mutation in CFTR produces different phenotypes depending on chromosomal background

1 reference

12 December 2020

Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis

1 reference

12 December 2020

Helical CT of the body: are settings adjusted for pediatric patients?

1 reference

12 December 2020

Lungs in infants and young children: improved thin-section CT with a noninvasive controlled-ventilation technique--initial experience

1 reference

12 December 2020

Susceptibility testing of Pseudomonas aeruginosa isolates and clinical response to parenteral antibiotic administration: lack of association in cystic fibrosis

1 reference

12 December 2020

Standardized high-resolution CT of the lung using a spirometer-triggered electron beam CT scanner

1 reference

12 December 2020

Allergic bronchopulmonary aspergillosis in cystic fibrosis--state of the art: Cystic Fibrosis Foundation Consensus Conference

1 reference

12 December 2020

Cystic fibrosis heterozygote screening in 5,161 pregnant women

1 reference

12 December 2020

Effect of hypertonic saline, amiloride, and cough on mucociliary clearance in patients with cystic fibrosis

1 reference

12 December 2020

Passive smoking and lung function in cystic fibrosis

1 reference

12 December 2020

Genotype and phenotype in cystic fibrosis

1 reference

12 December 2020

Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study

1 reference

12 December 2020

Nontuberculous mycobacteria. I: multicenter prevalence study in cystic fibrosis.

1 reference

12 December 2020

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

1 reference

12 December 2020

Technique and clinical applications of full-inflation and end-exhalation controlled-ventilation chest CT in infants and young children

1 reference

12 December 2020

Pseudomonas aeruginosa isolates from patients with cystic fibrosis: a class of serum-sensitive, nontypable strains deficient in lipopolysaccharide O side chains

1 reference

12 December 2020

Single versus combination intravenous antibiotic therapy for people with cystic fibrosis.

1 reference

12 December 2020

Pseudomonas aeruginosa, mucoidy and the chronic infection phenotype in cystic fibrosis

1 reference

12 December 2020

Therapeutic strategies to correct malfunction of CFTR.

1 reference

12 December 2020

Structural airway abnormalities in infants and young children with cystic fibrosis.

1 reference

12 December 2020

Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis

1 reference

12 December 2020

Placebo-controlled, double-blind, randomized study of aerosolized tobramycin for early treatment of Pseudomonas aeruginosa colonization in cystic fibrosis

1 reference

12 December 2020

Infection control in cystic fibrosis

1 reference

12 December 2020

Randomized trial of ceftazidime versus placebo in the management of acute respiratory exacerbations in patients with cystic fibrosis

1 reference

12 December 2020

Nebulised hypertonic saline for cystic fibrosis

1 reference

12 December 2020

Distinct spectrum of CFTR gene mutations in congenital absence of vas deferens

1 reference

12 December 2020

Eradication of initial Pseudomonas aeruginosa colonization in patients with cystic fibrosis

1 reference

12 December 2020

Exposure of children with cystic fibrosis to environmental tobacco smoke

1 reference

12 December 2020

Serum sensitivity of a Pseudomonas aeruginosa mucoid strain.

1 reference

12 December 2020

Treatment of pseudomonas aeruginosa colonisation in cystic fibrosis

1 reference

12 December 2020

Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis

1 reference

12 December 2020

Clinically feasible biofilm susceptibility assay for isolates of Pseudomonas aeruginosa from patients with cystic fibrosis

1 reference

12 December 2020

Acquisition of Pseudomonas aeruginosa in children with cystic fibrosis

1 reference

12 December 2020

Polyvariant mutant cystic fibrosis transmembrane conductance regulator genes. The polymorphic (Tg)m locus explains the partial penetrance of the T5 polymorphism as a disease mutation

1 reference

12 December 2020

Chest radiographs in cystic fibrosis. A follow-up study with application of a quantitative system.

1 reference

12 December 2020

Alginate is not a significant component of the extracellular polysaccharide matrix of PA14 and PAO1 Pseudomonas aeruginosa biofilms

1 reference

12 December 2020

Pseudomonas aeruginosa and the in vitro and in vivo biofilm mode of growth

1 reference

12 December 2020

Nontuberculous mycobacteria. II: nested-cohort study of impact on cystic fibrosis lung disease.

1 reference

12 December 2020

Elective versus symptomatic intravenous antibiotic therapy for cystic fibrosis.

1 reference

12 December 2020

Contribution of genetic factors other than CFTR to disease severity in cystic fibrosis

1 reference

12 December 2020

Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

1 reference

12 December 2020

The systematic evaluation of the chest radiograph in cystic fibrosis

1 reference

12 December 2020

Composite Spirometric–Computed Tomography Outcome Measure in Early Cystic Fibrosis Lung Disease

1 reference

12 December 2020

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

1 reference

12 December 2020

The incidence of cystic fibrosis in Scotland calculated from heterozygote frequencies

1 reference

12 December 2020

Cystic fibrosis: when should high-resolution computed tomography of the chest be obtained?

1 reference

12 December 2020

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

1 reference

12 December 2020

Single and combination antibiotic susceptibilities of planktonic, adherent, and biofilm-grown Pseudomonas aeruginosa isolates cultured from sputa of adults with cystic fibrosis.

1 reference

12 December 2020

Antibiotic prophylaxis in infants and young children with cystic fibrosis: a randomized controlled trial

1 reference

12 December 2020

Efficacy of inhaled tobramycin in the treatment of pulmonary exacerbations in children with cystic fibrosis

1 reference

12 December 2020

High-resolution CT in the acute exacerbation of cystic fibrosis: evaluation of acute findings, reversibility of those findings, and clinical correlation

1 reference

12 December 2020

Relationship between sputum inflammatory markers, lung function, and lung pathology on high-resolution computed tomography in children with cystic fibrosis.

1 reference

12 December 2020

Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis

1 reference

12 December 2020

Cystic Fibrosis Foundation consensus conference report on pulmonary complications of cystic fibrosis

1 reference

12 December 2020

Modifier genes and variation in cystic fibrosis

1 reference

12 December 2020

Mutation in the gene responsible for cystic fibrosis and predisposition to chronic rhinosinusitis in the general population

1 reference

12 December 2020

Epidemiology and clinical course of Burkholderia cepacia complex infections, particularly those caused by different Burkholderia cenocepacia strains, among patients attending an Italian Cystic Fibrosis Center

1 reference

12 December 2020

Development and evaluation of a PCR-based, line probe assay for the detection of 58 alleles in the cystic fibrosis transmembrane conductance regulator (CFTR) gene

1 reference

12 December 2020

Pseudomonas biofilm formation and antibiotic resistance are linked to phenotypic variation

1 reference

12 December 2020

The chest roentgenogram in cystic fibrosis: a new scoring system

1 reference

12 December 2020

Pulmonary disease assessment in cystic fibrosis: comparison of CT scoring systems and value of bronchial and arterial dimension measurements

1 reference

12 December 2020

Comparison of high-frequency chest wall oscillation and oscillating positive expiratory pressure in the home management of cystic fibrosis: a pilot study

1 reference

12 December 2020

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

1 reference

12 December 2020

A genetic basis for Pseudomonas aeruginosa biofilm antibiotic resistance

1 reference

12 December 2020

Serotype-specific serum IgG antibodies to lipopolysaccharides of Pseudomonas aeruginosa in cystic fibrosis: correlation to disease, subclass distribution, and experimental protective capacity

1 reference

12 December 2020

Proportion of cystic fibrosis gene mutations not detected by routine testing in men with obstructive azoospermia

1 reference

12 December 2020

Loss of FEV1 in cystic fibrosis: correlation with HRCT features.

1 reference

12 December 2020

Complement activation by Pseudomonas aeruginosa biofilms

1 reference

12 December 2020

Nonmotility and phagocytic resistance of Pseudomonas aeruginosa isolates from chronically colonized patients with cystic fibrosis

1 reference

12 December 2020

α1-Antitrypsin Deficiency Alleles in Cystic Fibrosis Lung Disease

1 reference

12 December 2020

Computed tomography and radiation: understanding the issues.

1 reference

12 December 2020

Wisconsin cystic fibrosis chest radiograph scoring system.

1 reference

12 December 2020

Alginate overproduction affects Pseudomonas aeruginosa biofilm structure and function

1 reference

12 December 2020

Identification of the cystic fibrosis gene: genetic analysis

1 reference

12 December 2020

Acceleration of lung disease in children with cystic fibrosis after Pseudomonas aeruginosa acquisition

1 reference

12 December 2020

Colistin inhalation therapy in cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infection

1 reference

12 December 2020

Opsonophagocytic killing antibody to Pseudomonas aeruginosa mucoid exopolysaccharide in older noncolonized patients with cystic fibrosis.

1 reference

12 December 2020

Comparison between morphologic changes seen on high-resolution CT and regional pulmonary perfusion seen on SPECT in patients with cystic fibrosis

1 reference

12 December 2020

Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids

1 reference

12 December 2020

Biofilms, antimicrobial resistance, and airway infection.

1 reference

12 December 2020

Quorum-sensing signals indicate that cystic fibrosis lungs are infected with bacterial biofilms

1 reference

12 December 2020

Infection withBurkholderia cepaciaComplex Genomovars in Patients with Cystic Fibrosis: Virulent Transmissible Strains of Genomovar III Can ReplaceBurkholderia multivorans

1 reference

12 December 2020

Endemicity and inter-city spread of Burkholderia cepacia genomovar III in cystic fibrosis

1 reference

12 December 2020

Early pulmonary inflammation in infants with cystic fibrosis

1 reference

12 December 2020

Medium term treatment of stable stage cystic fibrosis with recombinant human DNase I.

1 reference

12 December 2020

Mechanisms of biofilm resistance to antimicrobial agents

1 reference

12 December 2020

Effects of reduced mucus oxygen concentration in airway Pseudomonas infections of cystic fibrosis patients

1 reference

12 December 2020

Association of poor clinical status and heavy exposure to tobacco smoke in patients with cystic fibrosis who are homozygous for the F508 deletion

1 reference

12 December 2020

Efficacy of inhaled amikacin as adjunct to intravenous combination therapy (ceftazidime and amikacin) in cystic fibrosis

1 reference

12 December 2020

Randomised controlled trial of inhaled corticosteroids (fluticasone propionate) in cystic fibrosis

1 reference

12 December 2020

Evolution of CT findings in patients with cystic fibrosis

1 reference

12 December 2020

Lung disease associated with the IVS8 5T allele of the CFTR gene

1 reference

12 December 2020

Wisconsin cystic fibrosis chest radiograph scoring system: validation and standardization for application to longitudinal studies.

1 reference

12 December 2020

Efficacy and safety of short-term administration of aerosolised recombinant human DNase I in adults with stable stage cystic fibrosis

1 reference

12 December 2020

Changes in strategies for optimal antibacterial therapy in cystic fibrosis.

1 reference

12 December 2020

Helix induction in antimicrobial peptides by alginate in biofilms

1 reference

12 December 2020

Effect of chronic intermittent administration of inhaled tobramycin on respiratory microbial flora in patients with cystic fibrosis

1 reference

12 December 2020

Siderophore synthesis by mucoid Pseudomonas aeruginosa strains isolated from cystic fibrosis patients

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

Study of pyoverdine type and production by Pseudomonas aeruginosa isolated from cystic fibrosis patients: prevalence of type II pyoverdine isolates and accumulation of pyoverdine-negative mutations

1 reference

12 December 2020

Complete mutational screening of the CFTR gene in 120 patients with pulmonary disease

1 reference

12 December 2020

Mutations of the cystic fibrosis gene in patients with chronic pancreatitis

1 reference

12 December 2020

Role of alginate and its O acetylation in formation of Pseudomonas aeruginosa microcolonies and biofilms

1 reference

12 December 2020

Correlation of six different cystic fibrosis chest radiograph scoring systems with clinical parameters

1 reference

12 December 2020

Use of computerized tomography and chest x-rays in evaluating efficacy of aerosolized recombinant human DNase in cystic fibrosis patients younger than age 5 years: a preliminary study.

1 reference

12 December 2020

10.1007/S00247-005-1445-3

Identifiers

DOI

1 reference

12 August 2017

1 reference