Inflammation and Infection in Cystic Fibrosis: Update for the Clinician

Abstract

Inflammation and infection play an important role in the pathophysiology of cystic fibrosis, and they are significant causes of morbidity and mortality in CF. The presence of thick mucus in the CF airways predisposes to local hypoxia and promotes infection and inflammation. A vicious cycle of airway obstruction, inflammation, and infection is of critical importance for the progression of the disease, and new data elucidate the different factors that influence it. Recent research has been focused on improving infection and inflammation in addition to correcting the basic gene defect. This review aims to summarize important advances in infection and inflammation as well as the effect of new treatments modulating the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein. New approaches to target infection and inflammation are being studied, including gallium, nitric oxide, and phage therapy for infection, along with retinoids and neutrophil elastase inhibitors for inflammation.

E.PathDash, pathway activation analysis of publicly available pathogen gene expression data

“…Cystic Fibrosis (CF) is a genetic disease that is associated with chronic pulmonary infections 54 . Although the infections are polymicrobial in nature, comprising several bacterial genera, P. aeruginosa is the most common pathogen found in adult people with CF (pwCF) 25 .…”

“…This tool facilitated hypothesis generation resulting in a testable model of the key role propanoate metabolism may play in medically relevant microbial interactions of P. aeruginosa. Cystic Fibrosis (CF) is a genetic disease that is associated with chronic pulmonary infections 48 . Although the infections are polymicrobial in nature, comprising several bacterial genera, P. aeruginosa is the most common pathogen found in adult people with CF (pwCF) 23 .…”

“…Case Study #2: Differential effects of DNA-gyrase inhibitor classes on biofilm formation in P. aeruginosa. Sharanya Sarkar, Ph.D. candidate, Geisel School of Medicine at Dartmouth Cystic Fibrosis (CF) is a genetic disease that is associated with chronic pulmonary infections 54 . Although the infections are polymicrobial in nature, comprising several bacterial genera, P. aeruginosa is the most common pathogen found in adult people with CF (pwCF) 25 .…”

Assessing the Potential of N-Butyl-l-deoxynojirimycin (l-NBDNJ) in Models of Cystic Fibrosis as a Promising Antibacterial Agent

“…5 CF is a multisystem disease involving many organs; however, the leading cause of morbidity and mortality in people with CF (pwCF) remains the respiratory disease. 6,7 Subnormal CFTR activity mainly leads to dehydration of the airway surface liquid, accumulation of thick mucus, and decreasing of mucociliary clearance that promote the onset of a vicious cycle of airway inflammation and of recurrent and chronic bacterial infections. 8 The CF airway inflammation is characterized by an excessive production of proinflammatory mediators, such as TNF-α, GM-CSF, G-CSF, IL-1β, IL-6, and especially IL-8, mainly secreted by airway epithelial cells, and the presence of a massive number of neutrophils and macrophages.…”

New trends in pediatric pulmonology: our experience

“…The lung is the primary site of CF, to which most of the CF-associated patient morbidity and mortality is linked. CF occurs very early in infancy and is defined by the presence of infections and chronic inflammation leading to a deterioration in lung function, respiratory exacerbations, and bronchiectasis (13,14). Thus, identifying a biomarker for disease progression might be crucial for improving these patients' outcomes.…”