This is the second in a series of four papers updating the European Cystic Fibrosis Society
(ECFS) standards for the care of people with CF. This paper focuses on establishing and …

Cystic fibrosis (CF) is a genetic disease caused by variants in the gene encoding for the CF
transmembrane conductance regulator (CFTR) protein, a chloride and bicarbonate channel …

Advances in cystic fibrosis (CF) diagnostics and therapeutics have led to improved health
and longevity, including increased body weight and decreased malnutrition in people with …

Abstract Purpose of Review Cystic fibrosis is an inherited, multisystem disease that affects
the gastrointestinal system in numerous ways. This article reviews the nutritional …

This past year, there were many important advances for patients with cystic fibrosis (CF). Of
the many publications related to CF in 2023, there was further evaluation of highly effective …

Background The extent of cardiac involvement in cystic fibrosis (CF) remains to be
determined. The remarkable therapeutic advancements with new highly effective cystic …

Background: Cardiovascular disease (CVD) risks are increasing in people with cystic
fibrosis (pwCF). While cholesterol levels were historically low in pwCF, higher levels after …

This review synthesizes articles published in 2023, focusing on the impact of elexacaftor-
tezacaftor-ivacaftor (ETI) in cystic fibrosis (CF) care. Real-world data highlights sustained …

The introduction of elexacaftor/tezacaftor/ivacaftor (ETI) therapy has further extended life
expectancy of adults with cystic fibrosis (awCF), highlighting the need for increased attention …

Highly effective modulator therapy (HEMT) is now available to a wider range of people with
cystic fibrosis (pwCF). It has significantly improved short-term clinical outcomes and has the …