The Lexiebean Foundation, Current News

Giving those affected by Cystic Fibrosis reason to Believe

Current News

Association between the introduction of a new cystic fibrosis inhaled antibiotic class and change in prevalence of patients receiving multiple inhaled antibiotic classes

In 2010, aztreonam for inhalation solution joined aminoglycosides and colistimethate as a new cystic fibrosis (CF) chronic inhaled antimicrobial therapy. We studied how the introduction of this new inhaled antibiotic class changed the management of US CF patients.

Pulmonary function outcomes for assessing cystic fibrosis care

Assessing cystic fibrosis (CF) patient quality of care requires the choice of an appropriate outcome measure. We looked systematically and in detail at pulmonary function outcomes that potentially reflect clinical practice patterns.


The growing threat of nontuberculous mycobacteria in CF

There has been recent growing awareness of the threat of nontuberculous mycobacteria (NTM) to individuals with cystic fibrosis (CF) and an increasing appreciation of the difficulties in screening, diagnosing and treating NTM-pulmonary infection in the context of CF lung disease. Forthcoming joint US CFF-ECFS guidelines have been developed to assist clinicians with the management of NTM in individuals with CF.

Osteopontin is increased in cystic fibrosis and can skew the functional balance between ELR-positive and ELR-negative CXC-chemokines

The glycoprotein osteopontin plays important roles in several states of disease associated with inflammation, for example by recruiting neutrophils but its expression and possible roles in cystic fibrosis (CF) have not been investigated.

Initial evaluation of the Parent Cystic Fibrosis Questionnaire—Revised (CFQ-R) in infants and young children

There is an urgent need to evaluate treatments for young children with cystic fibrosis (CF); however, efforts have been hampered by a lack of reliable, practical endpoints. To examine whether a patient-reported outcome could be reliable in children 4 to 60months of age, we assessed the psychometric properties of the modified Parent Cystic Fibrosis Questionnaire—Revised (CFQ-R) using data from the Infant Study of Inhaled Saline (ISIS). We also characterized patterns of symptom presentation and daily functioning in children in this age range to inform future measure development.

The p.Gly622Asp (G622D) mutation, frequently found in Reunion Island and in black populations, is associated with a wide spectrum of CF and CFTR-RD phenotypes

Examination of genotype–phenotype correlations along with functional evaluation of CFTR mutations may not be straightforward. The c.1865G>A, p.Gly622Asp (G622D), located at the NBD1 C terminus of the CFTR protein, was initially reported in patients with male infertility. However, the substitution of Gly622 by an aspartic acid in vitro would perturb the local structure or even affect the CFTR folding itself. In order to determine whether p.Gly622Asp affects the risk of developing a CFTR-Related disorder (CFTR-RD) or cystic fibrosis (CF), we analyzed the phenotype of subjects bearing the p.Gly622Asp mutation.