(Q37704053)

22 August 2017

1 reference

title

Update in cystic fibrosis 2009. (English)

1 reference

22 August 2017

0 references

Peter J Mogayzel

1

1 reference

22 August 2017

Patrick A Flume

series ordinal

2

1 reference

22 August 2017

language of work or name

English

0 references

publication date

1 March 2010

1 reference

American Journal of Respiratory and Critical Care Medicine

22 August 2017

published in

1 reference

22 August 2017

volume

181

1 reference

22 August 2017

issue

6

1 reference

22 August 2017

page(s)

539-544

1 reference

Identification of the cystic fibrosis gene: genetic analysis

22 August 2017

cites work

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Bcl-2 suppresses sarcoplasmic/endoplasmic reticulum Ca2+-ATPase expression in cystic fibrosis airways: role in oxidant-mediated cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Lubiprostone stimulates secretion from tracheal submucosal glands of sheep, pigs, and humans

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Salmeterol restores secretory functions in cystic fibrosis airway submucosal gland serous cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Epithelial sodium channel inhibition in primary human bronchial epithelia by transfected siRNA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Fenretinide corrects newly found ceramide deficiency in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Cystic fibrosis fatty acid imbalance is linked to ceramide deficiency and corrected by fenretinide.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Defective acid sphingomyelinase pathway with Pseudomonas aeruginosa infection in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

CFTR regulation of intracellular pH and ceramides is required for lung endothelial cell apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Defective organellar acidification as a cause of cystic fibrosis lung disease: reexamination of a recurring hypothesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Unimpaired lysosomal acidification in respiratory epithelial cells in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Macrophages directly contribute to the exaggerated inflammatory response in cystic fibrosis transmembrane conductance regulator-/- mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Effect of systemic inflammation on inspiratory and limb muscle strength and bulk in cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Mutations in the amiloride-sensitive epithelial sodium channel in patients with cystic fibrosis-like disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Heritability of lung disease severity in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Histo-blood group gene polymorphisms as potential genetic modifiers of infection and cystic fibrosis lung disease severity

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

IL1B polymorphisms modulate cystic fibrosis lung disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Identification of IFRD1 as a modifier gene for cystic fibrosis lung disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Genetic modifiers of liver disease in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

A susceptibility gene for type 2 diabetes confers substantial risk for diabetes complicating cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Combination antibiotic susceptibility of biofilm-grown Burkholderia cepacia and Pseudomonas aeruginosa isolated from patients with pulmonary exacerbations of cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

The DeltaF508-CFTR mutation results in increased biofilm formation by Pseudomonas aeruginosa by increasing iron availability.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Iron-binding compounds impair Pseudomonas aeruginosa biofilm formation, especially under anaerobic conditions

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Tobramycin and FDA-approved iron chelators eliminate Pseudomonas aeruginosa biofilms on cystic fibrosis cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Antibiotic-resistant obligate anaerobes during exacerbations of cystic fibrosis patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Human neutrophil peptides and phagocytic deficiency in bronchiectatic lungs.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Longitudinal assessment of lung function from infancy to childhood in patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Recovery of birth weight z score within 2 years of diagnosis is positively associated with pulmonary status at 6 years of age in children with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Impact of Pseudomonas and Staphylococcus infection on inflammation and clinical status in young children with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Acquisition and eradication of P. aeruginosa in young children with cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Regimens for eradicating early Pseudomonas aeruginosa infection in children do not promote antibiotic resistance in this organism.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

A European consensus for the evaluation and management of infants with an equivocal diagnosis following newborn screening for cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

18F-fluorodeoxyglucose-PET/CT imaging of lungs in patients with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Development and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related quality-of-life measure for cystic fibrosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Determination of the minimal clinically important difference scores for the Cystic Fibrosis Questionnaire-Revised respiratory symptom scale in two populations of patients with cystic fibrosis and chronic Pseudomonas aeruginosa airway infection

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Patient-reported respiratory symptoms in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Efficacy and safety of inhaled aztreonam lysine for airway pseudomonas in cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Lung transplantation and survival in children with cystic fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Selection of cystic fibrosis patients for lung transplantation

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Lung transplantation in older patients with cystic fibrosis: analysis of UNOS data

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

A comparison of CF and non-CF school-age children undergoing lung transplantation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Randomized Trial of a Decision Aid for Patients with Cystic Fibrosis Considering Lung Transplantation

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Correctors Enhance Maturation of ΔF508 CFTR by Promoting Interactions between the Two Halves of the Molecule

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Detection of Anaerobic Bacteria in High Numbers in Sputum from Patients with Cystic Fibrosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

European best practice guidelines for cystic fibrosis neonatal screening

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

Lung Function in Infants with Cystic Fibrosis Diagnosed by Newborn Screening

21 January 2018

1 reference

https://api.crossref.org/works/10.1164%2FRCCM.200912-1943UP

10.1164/RCCM.200912-1943UP

21 January 2018

Identifiers

DOI

1 reference

22 August 2017

1 reference