Microbiology of Cystic Fibrosis Airway Disease

Abstract

Although survival of individuals with cystic fibrosis (CF) has been continuously improving for the past 40 years, respiratory failure secondary to recurrent pulmonary infections remains the leading cause of mortality in this patient population. Certain pathogens such as Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and species of the Burkholderia cepacia complex continue to be associated with poorer clinical outcomes including accelerated lung function decline and increased mortality. In addition, other organisms such as anaerobes, viruses, and fungi are increasingly recognized as potential contributors to disease progression. Culture-independent molecular methods are also being used for diagnostic purposes and to examine the interaction of microorganisms in the CF airway. Given the importance of CF airway infections, ongoing initiatives to promote understanding of the epidemiology, clinical course, and treatment options for these infections are needed.

Phenotypical Characteristics of the Black Yeast Exophiala dermatitidis Are Affected by Pseudomonas aeruginosa in an Artificial Sputum Medium Mimicking Cystic Fibrosis–Like Conditions

“…Pa often dominates the CF lung microbiome in both children and adults; it is present in the lungs of approximately 52% of all CF patients (Hauser et al, 2011). Chronic Pa infections among CF patients are commonly caused by biofilm-growing mucoid strains (Bjarnsholt et al, 2009) and are associated with poorer clinical outcome and higher mortality (Blanchard and Waters, 2019). In the past, P. aeruginosa has been reported to gain in resistance mechanisms towards antimicrobial therapy, among them the formation of biofilms (Breidenstein et al, 2011).…”

COVID-19 and Cystic Fibrosis: Diagnostic Difficulties or Incorrect Methods?

“…It is recognized that viral infections in patients with cystic fibrosis are a factor of poor prognosis and deterioration of lung function and increased frequency of PE and its severity 2,7,9,10 . The symptoms of a SARS-CoV-2 infection differ from the symptoms of PE that most patients with cystic fibrosis know how to recognize 5,7 .…”

“…CF patients have multiple events of viral, fungal, and bacterial infections throughout their lives, with chronic diseases due to Pseudomonas aeruginosa, Burkholderia cepacia, and Staphylococcus aureus being the most frequent. These recurrent infections are associated with pulmonary exacerbations (PE) that are the leading cause of death in CF 1,2 . Viral infections are estimated to cause between 10 to 60% of PE.…”

“…Unfortunately, samples are not usually taken to assess the presence of viruses 3 . The infections described in the PE of CF patients are the respiratory syncytial virus, human rhinovirus, influenza types A and B, parainfluenza virus, human metapneumovirus, picornavirus, coronavirus, and coxsackie/echovirus, which are responsible for continually keeping inflammatory markers elevated during mixed exacerbations, causing antibiotic treatments to be prolonged [2][3][4][5] .…”

Proteomics and Metabolomics for Cystic Fibrosis Research

“…The CF condition leads to severe and frequent infections with bronchiolitis and hemoptysis; the main microorganism that colonizes the airways in CF is Pseudomonas aeruginosa, although other bacterial species are frequently detected, such Propionibacterium, Staphylococcus aureus and Clostridiaceae, including Clostridium difficile, and a low abundance of Eggerthella, Eubacterium and Ruminococcus. Bacterial colonization results in acute and chronic infections, recurrent pulmonary exacerbations (PEx) and irreversible loss in lung function, with substantial morbidity and mortality [85]. The characterization of the bacterial charge is of primary importance in the therapy against CF.…”