Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages
The interplay between airway inflammation and infection is now recognized as a major factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen throughout the CF airway resulting in classic marked and enduring neutrophilic infiltrations, irreversibly damaging the lung....
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-02-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/24/4/4052 |
| _version_ | 1797620409643827200 |
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| author | Christiaan Yu Tom Kotsimbos |
| author_facet | Christiaan Yu Tom Kotsimbos |
| author_sort | Christiaan Yu |
| collection | DOAJ |
| description | The interplay between airway inflammation and infection is now recognized as a major factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen throughout the CF airway resulting in classic marked and enduring neutrophilic infiltrations, irreversibly damaging the lung. Although this is seen to occur early, independent of infection, respiratory microbes arising at different timepoints in life and the world environment perpetuate this hyperinflammatory state. Several selective pressures have allowed for the CF gene to persist until today despite an early mortality. Comprehensive care systems, which have been a cornerstone of therapy for the past few decades, are now revolutionized by CF transmembrane conductance regulator (CTFR) modulators. The effects of these small-molecule agents cannot be overstated and can be seen as early as in utero. For an understanding of the future, this review looks into CF studies spanning the historical and present period. |
| first_indexed | 2024-03-11T08:40:56Z |
| format | Article |
| id | doaj.art-804d139e9e204ec88ea8165bc5c5b44a |
| institution | Directory Open Access Journal |
| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-11T08:40:56Z |
| publishDate | 2023-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-804d139e9e204ec88ea8165bc5c5b44a2023-11-16T21:09:10ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672023-02-01244405210.3390/ijms24044052Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the AgesChristiaan Yu0Tom Kotsimbos1Department of Respiratory Medicine, Alfred Health, Melbourne, VIC 3004, AustraliaDepartment of Respiratory Medicine, Alfred Health, Melbourne, VIC 3004, AustraliaThe interplay between airway inflammation and infection is now recognized as a major factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen throughout the CF airway resulting in classic marked and enduring neutrophilic infiltrations, irreversibly damaging the lung. Although this is seen to occur early, independent of infection, respiratory microbes arising at different timepoints in life and the world environment perpetuate this hyperinflammatory state. Several selective pressures have allowed for the CF gene to persist until today despite an early mortality. Comprehensive care systems, which have been a cornerstone of therapy for the past few decades, are now revolutionized by CF transmembrane conductance regulator (CTFR) modulators. The effects of these small-molecule agents cannot be overstated and can be seen as early as in utero. For an understanding of the future, this review looks into CF studies spanning the historical and present period.https://www.mdpi.com/1422-0067/24/4/4052cystic fibrosisCTFR modulatorsenvironmenthostinfectioninflammation |
| spellingShingle | Christiaan Yu Tom Kotsimbos Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages International Journal of Molecular Sciences cystic fibrosis CTFR modulators environment host infection inflammation |
| title | Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages |
| title_full | Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages |
| title_fullStr | Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages |
| title_full_unstemmed | Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages |
| title_short | Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages |
| title_sort | respiratory infection and inflammation in cystic fibrosis a dynamic interplay among the host microbes and environment for the ages |
| topic | cystic fibrosis CTFR modulators environment host infection inflammation |
| url | https://www.mdpi.com/1422-0067/24/4/4052 |
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