scholarly journals Role of Mutant CFTR in Hypersusceptibility of Cystic Fibrosis Patients to Lung Infections

Science ◽  
1996 ◽  
Vol 271 (5245) ◽  
pp. 64-67 ◽  
Author(s):  
G. B. Pier ◽  
M. Grout ◽  
T. S. Zaidi ◽  
J. C. Olsen ◽  
L. G. Johnson ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Infections

scholarly journals Risk factors for respiratory Aspergillus fumigatus in German Cystic Fibrosis patients and impact on lung function

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Uta Düesberg ◽  
Julia Wosniok ◽  
Lutz Naehrlich ◽  
Patience Eschenhagen ◽  
Carsten Schwarz
Keyword(s):  
Risk Factors ◽  
Cystic Fibrosis ◽  
Lung Function ◽  
Aspergillus Fumigatus ◽  
Age Groups ◽  
Antibiotic Use ◽  
Lower Lung ◽  
Lung Infections ◽  
The Impact

Abstract Airway inflammation and chronic lung infections in cystic fibrosis (CF) patients are mostly caused by bacteria, e.g. Pseudomonas aeruginosa (PA). The role of fungi in the CF lung is still not well elucidated, but evidence for a harmful and complex role is getting stronger. The most common filamentous fungus in CF is Aspergillus fumigatus (AF). Age and continuous antibiotic treatment have been discussed as risk factors for AF colonisation but did not differentiate between transient and persistent AF colonisation. Also, the impact of co-colonisation of PA and AF on lung function is still under investigation. Data from patients with CF registered in the German Cystic Fibrosis Registry database in 2016 and 2017 were retrospectively analysed, involving descriptive and multivariate analysis to assess risk factors for transient or persistent AF colonisation. Age represented an independent risk factor for persistent AF colonisation. Prevalence was low in children less than ten years, highest in the middle age and getting lower in higher age (≥ 50 years). Continuous antibiotic lung treatment was significantly associated with AF prevalence in all age groups. CF patients with chronic PA infection had a lower lung function (FEV1%predicted), which was not influenced by an additional AF colonisation. AF colonisation without chronic PA infection, however, was significantly associated with a lower function, too. Older age up to 49 years and continuous antibiotic use were found to be the main risk factors for AF permanent colonisation. AF might be associated with decrease of lung function if not disguised by chronic PA infection.

scholarly journals Cystic Fibrosis Lung Immunity: The Role of the Macrophage

2016 ◽  
Vol 8 (6) ◽  
pp. 550-563 ◽  
Author(s):  
Emanuela M. Bruscia ◽  
Tracey L. Bonfield
Keyword(s):  
Cystic Fibrosis ◽  
Modifier Genes ◽  
Cystic Fibrosis Lung ◽  
Environmental Cues ◽  
Major Morbidity ◽  
Adaptive Immune ◽  
Lung Infections ◽  
Lung Immunity

Cystic fibrosis (CF) pathophysiology is hallmarked by excessive inflammation and the inability to efficiently resolve lung infections, contributing to major morbidity and eventually the mortality of patients with this disease. Macrophages (MΦs) are major players in lung homeostasis through their diverse contributions to both the innate and adaptive immune networks. The setting of MΦ function and activity in CF is multifaceted, encompassing the response to the unique environmental cues in the CF lung as well as the intrinsic changes resulting from CFTR dysfunction. The complexity is further enhanced with the identification of modifier genes, which modulate the CFTR contribution to disease, resulting in epigenetic and transcriptional shifts in MΦ phenotype. This review focuses on the contribution of MΦ to lung homeostasis, providing an overview of the diverse literature and various perspectives on the role of these immune guardians in CF.

scholarly journals Role of Pseudomonas aeruginosa exoenzymes in lung infections of patients with cystic fibrosis.

1985 ◽  
Vol 49 (3) ◽  
pp. 557-562 ◽  
Author(s):  
G Döring ◽  
W Goldstein ◽  
A Röll ◽  
P O Schiøtz ◽  
N Høiby ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Infections

scholarly journals Role of bacteriocins in mediating interactions of bacterial isolates taken from cystic fibrosis patients

Microbiology ◽  
2010 ◽  
Vol 156 (7) ◽  
pp. 2058-2067 ◽  
Author(s):  
Suphan Bakkal ◽  
Sandra M. Robinson ◽  
Claudia L. Ordonez ◽  
David A. Waltz ◽  
Margaret A. Riley
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Inhibitory Activity ◽  
Burkholderia Cepacia ◽  
Species Boundaries ◽  
Ecological Interactions ◽  
Bacterial Isolates ◽  
Novel Approach ◽  
Lung Infections

Pseudomonas aeruginosa (Pa) and Burkholderia cepacia complex (Bcc) lung infections are responsible for much of the mortality in cystic fibrosis (CF). However, little is known about the ecological interactions between these two, often co-infecting, species. This study provides what is believed to be the first report of the intra- and interspecies bacteriocin-like inhibition potential of Pa and Bcc strains recovered from CF patients. A total of 66 strains were screened, and shown to possess bacteriocin-like inhibitory activity (97 % of Pa strains and 68 % of Bcc strains showed inhibitory activity), much of which acted across species boundaries. Further phenotypic and molecular-based assays revealed that the source of this inhibition differs for the two species. In Pa, much of the inhibitory activity is due to the well-known S and RF pyocins. In contrast, Bcc inhibition is due to unknown mechanisms, although RF-like toxins were implicated in some strains. These data suggest that bacteriocin-based inhibition may play a role in governing Pa and Bcc interactions in the CF lung and may, therefore, offer a novel approach to mediating these often fatal infections.

scholarly journals Adapting to the Airways: Metabolic Requirements of Pseudomonas aeruginosa during the Infection of Cystic Fibrosis Patients

Metabolites ◽  
2019 ◽  
Vol 9 (10) ◽  
pp. 234 ◽  
Author(s):  
La Rosa ◽  
Johansen ◽  
Molin
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Physiological Changes ◽  
Metabolic Evolution ◽  
Physiological Conditions ◽  
Metabolic Functions ◽  
Lung Infections ◽  
Genome Scale

Pseudomonas aeruginosa is one of the major causes of morbidity and mortality of cystic fibrosis patients. During the infection, the bacteria colonize the nutritional rich lung mucus, which is present in the airway secretions in the patients, and they adapt their phenotype accordingly to the lung environment. In the airways, P. aeruginosa undergoes a broad metabolic rewiring as a consequence of the nutritional and stressful complexity of the lungs. However, the role of such metabolic rewiring on the infection outcome is poorly understood. Here, we review the metabolic evolution of clinical strains of P. aeruginosa during a cystic fibrosis lung infection and the metabolic functions operating in vivo under patho-physiological conditions. Finally, we discuss the perspective of modeling the cystic fibrosis environment using genome scale metabolic models of P. aeruginosa. Understanding the physiological changes occurring during the infection may pave the way to a more effective treatment for P. aeruginosa lung infections.

scholarly journals Bacteriocin-mediated competition in cystic fibrosis lung infections

2015 ◽  
Vol 282 (1814) ◽  
pp. 20150972 ◽  
Author(s):  
Melanie Ghoul ◽  
Stuart A. West ◽  
Helle Krogh Johansen ◽  
Søren Molin ◽  
Odile B. Harrison ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Experimental Studies ◽  
Natural Populations ◽  
Competitive Dynamics ◽  
Major Influence ◽  
Human Pathogens ◽  
Bacterial Strains ◽  
Lung Infections ◽  
Different Strains

Bacteriocins are toxins produced by bacteria to kill competitors of the same species. Theory and laboratory experiments suggest that bacteriocin production and immunity play a key role in the competitive dynamics of bacterial strains. The extent to which this is the case in natural populations, especially human pathogens, remains to be tested. We examined the role of bacteriocins in competition using Pseudomonas aeruginosa strains infecting lungs of humans with cystic fibrosis (CF). We assessed the ability of different strains to kill each other using phenotypic assays, and sequenced their genomes to determine what bacteriocins (pyocins) they carry. We found that (i) isolates from later infection stages inhibited earlier infecting strains less, but were more inhibited by pyocins produced by earlier infecting strains and carried fewer pyocin types; (ii) this difference between early and late infections appears to be caused by a difference in pyocin diversity between competing genotypes and not by loss of pyocin genes within a lineage over time; (iii) pyocin inhibition does not explain why certain strains outcompete others within lung infections; (iv) strains frequently carry the pyocin-killing gene, but not the immunity gene, suggesting resistance occurs via other unknown mechanisms. Our results show that, in contrast to patterns observed in experimental studies, pyocin production does not appear to have a major influence on strain competition during CF lung infections.

scholarly journals Role of Alginate O Acetylation in Resistance of Mucoid Pseudomonas aeruginosa to Opsonic Phagocytosis

2001 ◽  
Vol 69 (3) ◽  
pp. 1895-1901 ◽  
Author(s):  
Gerald B. Pier ◽  
Fadie Coleman ◽  
Martha Grout ◽  
Michael Franklin ◽  
Dennis E. Ohman
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Molecular Basis ◽  
Host Defenses ◽  
Specific Antibodies ◽  
Human Sera ◽  
Normal Human ◽  
Lung Infections

ABSTRACT Establishment and maintenance of chronic lung infections with mucoid Pseudomonas aeruginosa in patients with cystic fibrosis (CF) require that the bacteria avoid host defenses. Elaboration of the extracellular, O-acetylated mucoid exopolysaccharide, or alginate, is a major microbial factor in resistance to immune effectors. Here we show that O acetylation of alginate maximizes the resistance of mucoid P. aeruginosa to antibody-independent opsonic killing and is the molecular basis for the resistance of mucoid P. aeruginosa to normally nonopsonic but alginate-specific antibodies found in normal human sera and sera of infected CF patients. O acetylation of alginate appears to be critical for P. aeruginosa resistance to host immune effectors in CF patients.

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