Considerations for the use of inhaled antibiotics for Pseudomonas aeruginosa in people with cystic fibrosis receiving CFTR modulator therapy. [PDF]
Burgel PR +10 more
europepmc +1 more source
Laboratory Tools to Predict CFTR Modulator Therapy Effectiveness and to Monitor Disease Severity in Cystic Fibrosis. [PDF]
Bacalhau M, Camargo M, Lopes-Pacheco M.
europepmc +1 more source
Qualitative study exploring the views and perceptions of parents/carers of young children with CF regarding the introduction of CFTR modulator therapy (The REVEAL study; PaRents pErspectiVEs of KAftrio in chiLdren aged 2-5). [PDF]
Davies SH +4 more
europepmc +1 more source
Changes in vitamins and trace elements after initiation of highly effective CFTR modulator therapy in children and adults with cystic fibrosis - a real-life insight. [PDF]
Fabricius D +6 more
europepmc +1 more source
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Impact of interruption of CFTR modulator therapies
Journal of Cystic FibrosisNovel drug therapy targeting the defective cystic fibrosis transmembrane conductance regulator protein has the potential to significantly enhance the quality of life for numerous patients with cystic fibrosis. However, in some countries social insurance does not pay for modulators because these drugs are extremely expensive.
Ebru Yalcin, Didem Alboga
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The impact of CFTR modulators on bone health
Pediatric Pulmonology, 2022We are hopeful that CTFR modulation does have a positive impact of bone health, but we have concerns about this study design being used to support the hypothesis.
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CFTR is a modulator of airway inflammation
American Journal of Physiology-Lung Cellular and Molecular Physiology, 2007although it has been 17 years since the identification of the cystic fibrosis transmembrane ion receptor (CFTR) gene and protein, it remains enigmatic how abnormalities in CFTR can cause chronic and persistent pulmonary infection and inflammation that lead to bronchiectasis and end-stage ...
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WNK1 and WNK4 modulate CFTR activity
Biochemical and Biophysical Research Communications, 2007The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-gated chloride channel. WNK kinases are widely expressed modulators of ion transport. WNK1 and WNK4, two WNK kinases that are mutated in familial hyperkalemic hypertension (FHHt), are co-expressed with CFTR in several organs, raising the possibility that WNK kinases might alter ...
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CFTR Modulators for the Gastroenterologist
Journal of Pediatric Gastroenterology and Nutrition, 2023Sabina, Sabharwal, Sarah Shrager, Lusman
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CFTR Modulator Therapies for Cystic Fibrosis
Pediatric Allergy, Immunology, and Pulmonology, 2015The cloning of cystic fibrosis transmembrane conductance regulator (CFTR) set into motion a cascade of discoveries that have helped to reveal the underlying pathophysiologic basis of cystic fibrosis (CF). This discovery and the knowledge that followed have also provided the opportunity to target this basic defect, with the hope of reversing or ...
Aaron T, Trimble, Scott H, Donaldson
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