The Cystic Fibrosis Foundation established a process of systematic review of evidence to
inform the development of clinical care guidelines and encourage evidence-based practice …

The survival of patients with cystic fibrosis (CF) continues to improve. The discovery and
cloning of the CFTR gene more than 21 years ago led to the identification of the structure …

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 …

Background Advanced cystic fibrosis lung disease (ACFLD) is common, is associated with
reduced quality of life, and remains the most frequent cause of death in individuals with …

Evolving technology and advances in medical knowledge mandate a need to update these
guidelines. The aim of this report is to assist physicians throughout the world in referring …

This document is the result of an European Consensus conference which took place in
Artimino, Tuscany, Italy, in March 2001 involving 33 experts on nutrition in patients with …

The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis
transmembrane regulator protein (CFTR). The majority of people with CF have a limited life …

Sensitive measures of lung function applicable to young subjects are needed to detect early
cystic fibrosis (CF) lung disease. Forty children with CF aged 2 to 5 years and 37 age …

Background: A sensitive and valid non-invasive marker of early cystic fibrosis (CF) lung
disease is sought. The lung clearance index (LCI) from multiple-breath washout (MBW) is …

Background: The German cystic fibrosis (CF) quality assurance (CFQA) project is a patient
registry for CF which was founded in 1995. Relevant clinical and laboratory data, respiratory …