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- W4385454828 abstract "Cystic fibrosis (CF) is an inherited genetic disorder caused by mutations in the gene that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which results in the malfunction of the CFTR protein. 1 Shteinberg M. Haq I.J. Polineni D. Davies J.C. Cystic fibrosis. Lancet. 2021; 397: 2195-2211https://doi.org/10.1016/S0140-6736(20)32542-3 Abstract Full Text Full Text PDF PubMed Scopus (200) Google Scholar The dysfunction of the CFTR protein leads to the development and accumulation of thick mucus, particularly in the lungs, which greatly restricts mucociliary clearance. For a full description on the pathophysiology of CF, please see the review by Shteinberg and colleagues. 1 Shteinberg M. Haq I.J. Polineni D. Davies J.C. Cystic fibrosis. Lancet. 2021; 397: 2195-2211https://doi.org/10.1016/S0140-6736(20)32542-3 Abstract Full Text Full Text PDF PubMed Scopus (200) Google Scholar Such limited or absence of CFTR functionality contributes to creating conditions for commensal, environmental and pathogenic bacterial, particularly including Pseudomonas aeruginosa (PA), to colonise, survive and persist, potentially leading to chronic infection. 2 Blanchard A.C. Waters V.J. Microbiology of cystic fibrosis airway disease. Seminars in respiratory and critical care medicine. 2019; 40: 727-736https://doi.org/10.1055/s-0039-1698464 Crossref PubMed Scopus (61) Google Scholar As a result, people with CF are particularly vulnerable to lung infections, which are responsible for increased morbidity and mortality, where pulmonary infection continues to be the main cause of death. 2 Blanchard A.C. Waters V.J. Microbiology of cystic fibrosis airway disease. Seminars in respiratory and critical care medicine. 2019; 40: 727-736https://doi.org/10.1055/s-0039-1698464 Crossref PubMed Scopus (61) Google Scholar" @default.
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- W4385454828 date "2023-10-01" @default.
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- W4385454828 title "Cystic fibrosis and chronic pulmonary infection with Pseudomonas aeruginosa: Is Gross Domestic Product (GDP) involved?" @default.
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- W4385454828 doi "https://doi.org/10.1016/j.jinf.2023.07.019" @default.
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