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Pathogens
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Respiratory Pathogens in Cystic Fibrosis
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Respiratory Pathogens in Cystic Fibrosis

A special issue of Pathogens (ISSN 2076-0817).

Deadline for manuscript submissions: closed (15 November 2021) | Viewed by 20237

Special Issue Editor

Dr. Joanna B. Goldberg

E-Mail Website
Guest Editor
Emory University School of Medicine, Atlanta, USA
Interests: Pseudomonas aeruginosa; bacterial physiology; pulmonary infections; cystic fibrosis (CF); bacterial genetics and genomics

Special Issue Information

Dear Colleagues,

Cystic fibrosis (CF) is an autosomal recessive genetic disease that affects many organs. Over 2500 mutations in the CF transmembrane conductance regulator (CFTR) gene have been recognized, resulting in differences in disease severity. Over the past few decades, there have been exciting breakthroughs in managing nutritional complications, using gastrointestinal therapeutics, providing effective pulmonary therapies such as DNase and inhaled antibiotics, and dramatic breakthroughs in using CFTR modulators that promote mutant protein function. However, the predicted survival age for an individual born today with CF is still less than 50 years. Persistent lung infections remain the leading cause of morbidity and mortality in this population. Staphylococcus aureus and Pseudomonas aeruginosa are the two most commonly recognized bacterial pathogens associated with chronic lung infections in CF. Other bacterial species such as Hemophilus spp., members of the Burkholderia cepacia complex, Stenotrophomonas maltophilia, Achromobacter spp., and non-tuberculous Mycobacteria can also be cultured from CF sputum. Respiratory viruses and fungi have also been associated with initiation and/or increasing the severity of bacterial infection. With newer culture-independent methods to identify microbes in the lung, a number of additional non-traditional organisms, including anaerobic bacteria such as Prevotella and Veillonella have been associated with increased pathogenesis. However, their role in infection remains somewhat less clear.

In this Special Issue of Pathogens, we invite submissions of original research articles, case reports, short communications, and review articles focused on “Respiratory Pathogens in Cystic Fibrosis” with the goal of increasing our understanding of how the CF lung environment promotes these unique host–microbe interactions and how this information can be leveraged into new therapeutic approaches for this population. The topics of interest include the viruses, bacteria, and fungi associated with CF, the CF microbiome, microbial community dynamics, antibiotic susceptibility and resistance, virulence factors and anti-virulence strategies, new infection models of CF, novel therapeutics, and host response to infection.

Dr. Joanna B. Goldberg
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Pathogens is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2200 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • cystic fibrosis
  • interspecies communication
  • biofilms
  • quorum sensing
  • lung infections

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Published Papers (5 papers)

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Research

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14 pages, 2316 KiB  
Article
Cystic Fibrosis Clinical Isolates of Aspergillus fumigatus Induce Similar Muco-inflammatory Responses in Primary Airway Epithelial Cells
by Samantha A. McLean, Leilani Cullen, Dianne J. Gardam, Craig J. Schofield, Daniel R. Laucirica, Erika N. Sutanto, Kak-Ming Ling, Stephen M. Stick, Christopher S. Peacock, Anthony Kicic, Luke W. Garratt, on behalf of AREST CF and WAERP
Pathogens 2021, 10(8), 1020; https://doi.org/10.3390/pathogens10081020 - 13 Aug 2021
Cited by 2 | Viewed by 2525
Abstract
Aspergillus is increasingly associated with lung inflammation and mucus plugging in early cystic fibrosis (CF) disease during which conidia burden is low and strains appear to be highly diverse. It is unknown whether clinical Aspergillus strains vary in their capacity to induce epithelial [...] Read more.
Aspergillus is increasingly associated with lung inflammation and mucus plugging in early cystic fibrosis (CF) disease during which conidia burden is low and strains appear to be highly diverse. It is unknown whether clinical Aspergillus strains vary in their capacity to induce epithelial inflammation and mucus production. We tested the hypothesis that individual colonising strains of Aspergillus fumigatus would induce different responses. Ten paediatric CF Aspergillus isolates were compared along with two systemically invasive clinical isolates and an ATCC reference strain. Isolates were first characterised by ITS gene sequencing and screened for antifungal susceptibility. Three clusters (A−C) of Aspergillus isolates were identified by ITS. Antifungal susceptibility was variable, particularly for itraconazole. Submerged CF and non-CF monolayers as well as differentiated primary airway epithelial cell cultures were incubated with conidia for 24 h to allow germination. None of the clinical isolates were found to significantly differ from one another in either IL-6 or IL-8 release or gene expression of secretory mucins. Clinical Aspergillus isolates appear to be largely homogenous in their mucostimulatory and immunostimulatory capacities and, therefore, only the antifungal resistance characteristics are likely to be clinically important. Full article
(This article belongs to the Special Issue Respiratory Pathogens in Cystic Fibrosis)
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22 pages, 4595 KiB  
Article
Cystic Fibrosis Sputum Impairs the Ability of Neutrophils to Kill Staphylococcus aureus
by Kayla Fantone, Samantha L. Tucker, Arthur Miller, Ruchi Yadav, Eryn E. Bernardy, Rachel Fricker, Arlene A. Stecenko, Joanna B. Goldberg and Balázs Rada
Pathogens 2021, 10(6), 703; https://doi.org/10.3390/pathogens10060703 - 4 Jun 2021
Cited by 5 | Viewed by 4294
Abstract
Cystic fibrosis (CF) airway disease is characterized by chronic microbial infections and infiltration of inflammatory polymorphonuclear (PMN) granulocytes. Staphylococcus aureus (S. aureus) is a major lung pathogen in CF that persists despite the presence of PMNs and has been associated with CF lung [...] Read more.
Cystic fibrosis (CF) airway disease is characterized by chronic microbial infections and infiltration of inflammatory polymorphonuclear (PMN) granulocytes. Staphylococcus aureus (S. aureus) is a major lung pathogen in CF that persists despite the presence of PMNs and has been associated with CF lung function decline. While PMNs represent the main mechanism of the immune system to kill S. aureus, it remains largely unknown why PMNs fail to eliminate S. aureus in CF. The goal of this study was to observe how the CF airway environment affects S. aureus killing by PMNs. PMNs were isolated from the blood of healthy volunteers and CF patients. Clinical isolates of S. aureus were obtained from the airways of CF patients. The results show that PMNs from healthy volunteers were able to kill all CF isolates and laboratory strains of S. aureus tested in vitro. The extent of killing varied among strains. When PMNs were pretreated with supernatants of CF sputum, S. aureus killing was significantly inhibited suggesting that the CF airway environment compromises PMN antibacterial functions. CF blood PMNs were capable of killing S. aureus. Although bacterial killing was inhibited with CF sputum, PMN binding and phagocytosis of S. aureus was not diminished. The S. aureus-induced respiratory burst and neutrophil extracellular trap release from PMNs also remained uninhibited by CF sputum. In summary, our data demonstrate that the CF airway environment limits killing of S. aureus by PMNs and provides a new in vitro experimental model to study this phenomenon and its mechanism. Full article
(This article belongs to the Special Issue Respiratory Pathogens in Cystic Fibrosis)
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Review

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27 pages, 2589 KiB  
Review
Staphylococcus aureus and Cystic Fibrosis—A Close Relationship. What Can We Learn from Sequencing Studies?
by Christine Rumpf, Jonas Lange, Bianca Schwartbeck and Barbara C. Kahl
Pathogens 2021, 10(9), 1177; https://doi.org/10.3390/pathogens10091177 - 13 Sep 2021
Cited by 21 | Viewed by 4610
Abstract
Staphylococcus aureus is next to Pseudomonas aeruginosa the most isolated pathogen from the airways of cystic fibrosis (CF) patients, who are often infected by a dominant S. aureus clone for extended periods. To be able to persist, the pathogen has to adapt to [...] Read more.
Staphylococcus aureus is next to Pseudomonas aeruginosa the most isolated pathogen from the airways of cystic fibrosis (CF) patients, who are often infected by a dominant S. aureus clone for extended periods. To be able to persist, the pathogen has to adapt to the hostile niche of the airways to counteract host defence, antibiotic therapy and the competition with coinfecting pathogens. S. aureus is equipped with many virulence factors including adhesins, toxins that are localized on the chromosome, on plasmids or are phage-related. S. aureus is especially versatile and adaptation and evolution of the pathogen occurs by the acquisition of new genes by horizontal gene transfer (HGT), changes in nucleotides (single nucleotide variations, SNVs) that can cause a selective advantage for the bacteria and become fixed in subpopulations. Methicillin-resistant S. aureus are a special threat to CF patients due to the more severe lung disease occurring in infected patients. Today, with decreasing costs for sequencing, more and more studies using S. aureus isolates cultured from CF patients are being published, which use whole genome sequencing (WGS), multilocus sequence typing (MLST) or spa-sequence typing (spa-typing) to follow the population dynamics of S. aureus, elucidate the underlying mechanisms of phenotypic variants, newly acquired resistance or adaptation to the host response in this particular niche. In the first part of this review, an introduction to the genetic make-up and the pathogenesis of S. aureus with respect to CF is provided. The second part presents an overview of recent studies and their findings using genotypic methods such as single or multilocus sequencing and whole genome sequencing, which identify factors contributing to the adaptation of S. aureus and its evolution in the airways of individuals with CF. Full article
(This article belongs to the Special Issue Respiratory Pathogens in Cystic Fibrosis)
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11 pages, 1823 KiB  
Review
Pseudomonas aeruginosa Consumption of Airway Metabolites Promotes Lung Infection
by Sebastián A. Riquelme and Alice Prince
Pathogens 2021, 10(8), 957; https://doi.org/10.3390/pathogens10080957 - 29 Jul 2021
Cited by 7 | Viewed by 3990
Abstract
Prevailing dogma indicates that the lung of cystic fibrosis (CF) individuals is infected by multiple pathogens due to the abundant accumulation of mucus, which traps most of inhaled organisms. However, this hypothesis does not explain how specific opportunists, like Pseudomonas aeruginosa, are [...] Read more.
Prevailing dogma indicates that the lung of cystic fibrosis (CF) individuals is infected by multiple pathogens due to the abundant accumulation of mucus, which traps most of inhaled organisms. However, this hypothesis does not explain how specific opportunists, like Pseudomonas aeruginosa, are selected in the CF lung to cause chronic disease. This strongly suggests that other factors than mucus are accrued in the human airway and might predispose to bacterial disease, especially by P. aeruginosa. In this review we discuss the role of macrophage metabolites, like succinate and itaconate, in P. aeruginosa pneumonia. We analyze how dysfunction of the CF transmembrane conductance regulator (CFTR) favors release of these metabolites into the infected airway, and how P. aeruginosa exploits these elements to induce transcriptomic and metabolic changes that increase its capacity to cause intractable disease. We describe the host and pathogen pathways associated with succinate and itaconate catabolism, mechanisms of bacterial adaptation to these determinants, and suggest how both experimental settings and future therapies should consider macrophage metabolites abundance to better study P. aeruginosa pathogenesis. Full article
(This article belongs to the Special Issue Respiratory Pathogens in Cystic Fibrosis)
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13 pages, 556 KiB  
Review
Fungal Infection and Inflammation in Cystic Fibrosis
by T. Spencer Poore, Gina Hong and Edith T. Zemanick
Pathogens 2021, 10(5), 618; https://doi.org/10.3390/pathogens10050618 - 18 May 2021
Cited by 8 | Viewed by 3470
Abstract
Fungi are frequently recovered from lower airway samples from people with cystic fibrosis (CF), yet the role of fungi in the progression of lung disease is debated. Recent studies suggest worsening clinical outcomes associated with airway fungal detection, although most studies to date [...] Read more.
Fungi are frequently recovered from lower airway samples from people with cystic fibrosis (CF), yet the role of fungi in the progression of lung disease is debated. Recent studies suggest worsening clinical outcomes associated with airway fungal detection, although most studies to date are retrospective or observational. The presence of fungi can elicit a T helper cell type 2 (Th-2) mediated inflammatory reaction known as allergic bronchopulmonary aspergillosis (ABPA), particularly in those with a genetic atopic predisposition. In this review, we discuss the epidemiology of fungal infections in people with CF, risk factors associated with development of fungal infections, and microbiologic approaches for isolation and identification of fungi. We review the spectrum of fungal disease presentations, clinical outcomes after isolation of fungi from airway samples, and the importance of considering airway co-infections. Finally, we discuss the association between fungi and airway inflammation highlighting gaps in knowledge and future research questions that may further elucidate the role of fungus in lung disease progression. Full article
(This article belongs to the Special Issue Respiratory Pathogens in Cystic Fibrosis)
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