Cystic fibrosis: a review

T Ong, BW Ramsey - Jama, 2023 - jamanetwork.com
Importance Cystic fibrosis, a genetic disorder defined by variants in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene, affects more than 30 000 individuals in …

Increasing life expectancy in cystic fibrosis: Advances and challenges

KA McBennett, PB Davis, MW Konstan - Pediatric pulmonology, 2022 - Wiley Online Library
Since the first description of cystic fibrosis in 1938, there have been significant advances in
both quality of life and longevity for people living with this disease. In this article we describe …

[HTML][HTML] Effect of elexacaftor-tezacaftor-ivacaftor on body weight and metabolic parameters in adults with cystic fibrosis

MC Petersen, L Begnel, M Wallendorf, M Litvin - Journal of Cystic Fibrosis, 2022 - Elsevier
Background Though weight gain has been reported in some clinical trials of CFTR
modulators, the effect of elexacaftor-tezacaftor-ivacaftor on body weight, body mass index …

[HTML][HTML] Dose adjustments of Elexacaftor/Tezacaftor/Ivacaftor in response to mental health side effects in adults with cystic fibrosis

G Spoletini, L Gillgrass, K Pollard, N Shaw… - Journal of Cystic …, 2022 - Elsevier
Introduction Deterioration in mental health has been reported in a minority of individuals with
cystic fibrosis starting elexacafor/tezacaftor/ivacaftor (ELX/TEZ/IVA). We report our …

[HTML][HTML] Standards of care for CFTR variant-specific therapy (including modulators) for people with cystic fibrosis

KW Southern, C Castellani, E Lammertyn… - Journal of Cystic …, 2023 - Elsevier
Cystic fibrosis (CF) has entered the era of variant-specific therapy, tailored to the genetic
variants in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CFTR …

[HTML][HTML] Efficacy and safety of elexacaftor-tezacaftor-ivacaftor in the treatment of cystic fibrosis: a systematic review

N Kapouni, M Moustaki, K Douros, I Loukou - Children, 2023 - mdpi.com
Elexacaftor/Tezacaftor/Ivacaftor (ELX/TEZ/IVA) is a new CFTR (Cystic Fibrosis
Transmembrane Conductance Regulator) modulator treatment, used over the last few years …

Role of inhaled antibiotics in the era of highly effective CFTR modulators

JS Elborn, F Blasi, PR Burgel… - European Respiratory …, 2023 - Eur Respiratory Soc
Recurrent and chronic bacterial infections are common in people with cystic fibrosis (CF)
and contribute to lung function decline. Antibiotics are the mainstay in the treatment of …

[HTML][HTML] Mycobacterium abscessus Infections in Cystic Fibrosis Individuals: A Review on Therapeutic Options

D Recchia, G Stelitano, A Stamilla… - International Journal of …, 2023 - mdpi.com
Mycobacterium abscessus is an opportunistic pathogen that mainly colonizes and infects
cystic fibrosis patients' lungs. M. abscessus is naturally resistant to many antibiotics such as …

Management of neuropsychiatric symptoms in adults treated with elexacaftor/tezacaftor/ivacaftor

E Baroud, N Chaudhary… - Pediatric …, 2023 - Wiley Online Library
Introduction Reports of neuropsychiatric symptoms proximal to cystic fibrosis
transmembrane conductance regulator (CFTR) modulator initiation are emerging, but their …

[HTML][HTML] How Three Self-Secreted Biofilm Exopolysaccharides of Pseudomonas aeruginosa, Psl, Pel, and Alginate, Can Each Be Exploited for Antibiotic Adjuvant …

J Chung, S Eisha, S Park, AJ Morris… - International Journal of …, 2023 - mdpi.com
In cystic fibrosis (CF), pulmonary infection with Pseudomonas aeruginosa is a cause of
increased morbidity and mortality, especially in patients for whom infection becomes chronic …