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REVIEW STRATEGY
Year : 2022  |  Volume : 1  |  Issue : 1  |  Page : 30-34

Cystic fibrosis: Mutations, modulators and microbiology


1 School of Medicine, Dentistry and Biomedical Sciences, The Wellcome-Wolfson Institute for Experimental Medicine, Queen's University, Belfast, Northern Ireland, United Kingdom
2 School of Medicine, Dentistry and Biomedical Sciences, The Wellcome-Wolfson Institute for Experimental Medicine, Queen's University; Laboratory for Disinfection and Pathogen Elimination Studies, Northern Ireland Public Health Laboratory, Belfast City Hospital; Northern Ireland Regional Adult Cystic Fibrosis Centre, Level 8, Belfast City Hospital, Belfast, Northern Ireland, United Kingdom

Correspondence Address:
Beverley Cherie Millar
Northern Ireland Public Health Laboratory, Belfast City Hospital, BT9 7AD, Belfast, Northern Ireland
United Kingdom
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpdtsm.jpdtsm_10_22

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The last decade has witnessed an unprecedented arrival and introduction of several new innovations in the treatment and management of cystic fibrosis (CF), all for the benefit of people with CF (PwCF). Such innovations have been largely led by the CF transmembrane conductance regulator (CFTR) modulator medicines, which have also been accompanied by new antibiotics, nutritional formulations, as well as advances in the delivery of medicine through nebulization. Many of these have had an influence on the microbiology of the CF lung and the rebalancing of microbial taxa and cell density within the airways. Simultaneously, certain aspects of the new treatments have led to difficulties in PwCF being able to produce sufficient sputum to enable routine microbiological analyses to be performed. Coupled with this, the COVID-19 pandemic has accelerated the emergence of the virtual CF clinical, where individuals with CF do not have to physically travel to CF clinic as frequently as before, with the disadvantage of not producing sputum specimens for routine microbiological analyses. This review examines the interaction between CF mutations and CFTR modulators, with particular focus on CF microbiology.


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