Airway inflammation accelerates pulmonary exacerbations in cystic fibrosis.

Airway inflammation underlies cystic fibrosis (CF) pulmonary exacerbations. In a prospective multicenter study of randomly selected, clinically stable adolescents and adults, we assessed relationships between 24 inflammation-associated molecules and the future occurrence of CF pulmonary exacerbation using proportional hazards models. We explored relationships for potential confounding or mediation by clinical factors and assessed sensitivities to treatments including CF transmembrane regulator (CFTR) protein synthesis modulators.

Delayed HIV diagnosis in a cystic fibrosis patient: Not just another exacerbation.

Patients with cystic fibrosis (CF) are living longer due to advancements in treatment. We present a patient with CF in whom diagnoses of Human Immunodeficiency Virus (HIV) and severe pneumocystis pneumonia were delayed due to anchor bias. Our case highlights the importance of routine age-appropriate health screenings in patients with CF.

Prospective multicenter randomized patient recruitment and sample collection to enable future measurements of sputum biomarkers of inflammation in an observational study of cystic fibrosis.

Background: Biomarkers of inflammation predictive of cystic fibrosis (CF) disease outcomes would increase the power of clinical trials and contribute to better personalization of clinical assessments. A representative patient cohort would improve searching for believable, generalizable, reproducible and accurate biomarkers.

Methods: We recruited patients from Mountain West CF Consortium (MWCFC) care centers for prospective observational study of sputum biomarkers of inflammation.

A Proof of Concept Study to Detect Urease Producing Bacteria in Lungs Using Aerosolized C-Urea.

This is a "proof of concept" study to determine whether inhalation of C-urea can be safely used to detect the presence of urease producing bacteria in the airways of patients with cystic fibrosis (CF) by detecting CO in breath. This was a prospective, 2-part, open label, single-center, single-arm, single-administration, dose-escalation investigational device exemption trial. First, the safety of 20 and 50 mg inhaled C-urea was evaluated in 6 healthy adult participants.