How Should the Effects of CFTR Modulator Therapy on Cystic Fibrosis Lung Disease Be Monitored?
, 2022 Elad Ben Meir, Hartmut Grasemann
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176 Cystic fibrosis airway inflammation enhances the efficacy of CFTR modulators [PDF]
, 2023 Carla M. P. Ribeiro +7 more
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Expanding Cystic Fibrosis Registries to the Rest of the World
Pediatric Pulmonology, Volume 61, Issue 1, January 2026.ABSTRACT Challenge Cystic fibrosis (CF) is a global challenge. The epidemiological knowledge is incomplete and focused on patient registries in the United States, Canada, Europe, Australia and New Zealand, Brazil, and South Africa. To complete the global picture of CF, we have to learn from each individual with CF, as well as each cohort and population.
Lutz Naehrlich
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Personalised medicine of Cystic fibrosis [PDF]
, 2017 Treballs Finals de Grau de Farmàcia, Facultat de Farmàcia, Universitat de Barcelona, 2017. Tutor: Carlos Julián Ciudad i Gómez[en] The European commission defines personalised medicine as a medical approach that uses molecular insights into health and ...
Cabré Juan, Antoni
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Interrater Reliability of a Modified Bronchoscopy Scoring Tool in Children With Cystic Fibrosis
Pediatric Pulmonology, Volume 61, Issue 1, January 2026.ABSTRACT Background Flexible bronchoscopy (FB) is widely used in the management of children with Cystic Fibrosis (CF) to visualize airway abnormalities, assess inflammation and detect infection. While previous scoring systems have been proposed to quantify visual airway findings in general pediatric populations, no standardized tool exists for ...
Alexandra Bosetti +5 more
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Clinical pharmacology of CFTR modulators
With the development of cystic fibrosis transmembrane receptor (CFTR) modulating drugs, the landscape in cystic fibrosis (CF) care has changed dramatically. These drugs enable the treatment of the underlying cause of the disease. Although CFTR modulators show an impressive clincal effect at group level in people with CF (pwCF) with specific mutations ...
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Frontiers in PharmacologyThe combination of pharmacological modulators such as lumacaftor, tezacaftor, and elexacaftor restore CFTR activity at the plasma membrane and improve lung function in patients carrying CFTR mutations such as F508del, their effects on inflammation are ...
Amal Kouadri +20 more
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P347 Mental health after initiating triple CFTR modulators in a Polish paediatric cystic fibrosis centre - a preliminary report [PDF]
, 2023 U. Borawska-Kowalczyk +4 more
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Impact of Elexacaftor‐Tezacaftor‐Ivacaftor on Quality of Life in Children With Cystic Fibrosis
Pediatric Pulmonology, Volume 61, Issue 1, January 2026.ABSTRACT Objectives CFTR modulators have revolutionized cystic fibrosis (CF) management by targeting the defective protein rather than its consequences. Their impact on quality of life (QoL) have been studied in numerous trials, but few data are available on QoL in patients receiving Elexacaftor‐Tezacaftor‐Ivacaftor (ETI), notably in children given its
Sara Kümmerli +6 more
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P031 Perspectives for gene therapy with the use of CFTR modulators in patients with cystic fibrosis [PDF]
, 2023 Luana da Silva Baptista Arpini +4 more
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