Functional Respiratory Imaging Offers Value in Cystic Fibrosis
Investigators compared functional respiratory imaging with validated imaging parameters and classical respiratory outcomes in patients with cystic fibrosis.
Cystic Fibrosis
CFTR Expression in the Pathogenesis of Pulmonary Arterial Hypertension
Researchers sought to delineate the role of cystic fibrosis transmembrane conductance regulator (CFTR) in pulmonary arterial hypertension pathogenesis through observational and interventional experiments in human tissues and animal models.
Effectiveness of Antipseudomonal Antibiotics for Pulmonary Exacerbation of Cystic Fibrosis
Researchers compared clinical outcomes of single vs double antipseudomonal antibiotics used to treat pulmonary exacerbations in patients with cystic fibrosis.
Trikafta Approval Expanded to Include Younger Cystic Fibrosis Patients
The approval was based on data from a phase 3 study that evaluated Trikafta in 66 children 6 to 11 years of age with CF who were either homozygous for the F508del mutation or heterozygous for the F508del mutation and 1 minimal function mutation.
Assessing the Risk of SARS-CoV-2 Infection in Patients With Cystic Fibrosis
While a subset of people with cystic fibrosis may experience a more severe clinical course of COVID-19, they are not at an increased risk of contracting SARS-CoV-2 compared with the general population.
Elexacaftor/Tezacaftor/Ivacaftor Improved Lung Function in Cystic Fibrosis
Combination therapy with elexacaftor/tezacaftor/ivacaftor resulted in clinically significant improvements in lung function in patients with cystic fibrosis.
Predictors of Failed Pseudomonas aeruginosa Eradication in Children With Cystic Fibrosis
Investigators sought to determine whether resistance to antibiotic treatment was associated with failed eradication therapy in patients with cystic fibrosis.
Cystic Fibrosis Updates in Care: Part I — Current State of Treatment
In honor of Cystic Fibrosis Awareness Month, we present a 2-part series on treatment updates and developments in the field.
Cystic Fibrosis Updates in Care: Part II — The “Sweat Sticker” and Clinician Q&A
In honor of Cystic Fibrosis Awareness Month, we present a 2-part series on treatment updates in the field, including the “sweat sticker.”
Lumacaftor-Ivacaftor Safe, Effective for Long-Term Treatment of Cystic Fibrosis
Lumacaftor-ivacaftor is generally safe and well tolerated for long-term treatment of children with cystic fibrosis homozygous for the F508del-CFTR mutation.
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