scholarly journals Course of the disease with evaluation of the chloride channel function and selection of target therapy in vitro in an adult with cystic fibrosis with 2184insA/L138ins genotype

2020 ◽  
Vol 15 (2) ◽  
Author(s):  
Julia Leonidovna Melyanovskaya ◽  
Stanislav Aleksandrovich Krasovsky ◽  
Anna Sergeevna Efremova ◽  
Natalya Vadimovna Bulatenko ◽  
Marina Alekseevna Makarova
Keyword(s):  
Cystic Fibrosis ◽  
Chloride Channel ◽  
Target Therapy ◽  
Channel Function ◽  
Selection Of

Primary Sclerosing Cholangitis in Childhood is Associated with Abnormalities in Cystic Fibrosis–Mediated Chloride Channel Function

2007 ◽  
Vol 151 (3) ◽  
pp. 255-259 ◽  
Author(s):  
Harpreet Pall ◽  
Julian Zielenski ◽  
Maureen M. Jonas ◽  
Deborah A. DaSilva ◽  
Kimberly M. Potvin ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Primary Sclerosing Cholangitis ◽  
Chloride Channel ◽  
Sclerosing Cholangitis ◽  
Channel Function

Primary sclerosing cholangitis is associated with abnormalities in cystic fibrosis-mediated chloride channel function

2000 ◽  
Vol 118 (4) ◽  
pp. A958 ◽  
Author(s):  
Sunil G. Sheth ◽  
Michele D. Bishop ◽  
Julie C. Shea ◽  
Isabel K. Hopper ◽  
Sanjiv Chopra ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Primary Sclerosing Cholangitis ◽  
Chloride Channel ◽  
Sclerosing Cholangitis ◽  
Channel Function

scholarly journals Filamentous bacteriophages are associated with chronic Pseudomonas lung infections and antibiotic resistance in cystic fibrosis

2019 ◽  
Vol 11 (488) ◽  
pp. eaau9748 ◽  
Author(s):  
Elizabeth B. Burgener ◽  
Johanna M. Sweere ◽  
Michelle S. Bach ◽  
Patrick R. Secor ◽  
Naomi Haddock ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Antibiotic Resistance ◽  
Genomic Analysis ◽  
Cross Sectional ◽  
Filamentous Bacteriophages ◽  
Lung Infections ◽  
Vitro Analysis ◽  
In Vitro Analysis ◽  
Selection Of

Filamentous bacteriophage (Pf phage) contribute to the virulence of Pseudomonas aeruginosa infections in animal models, but their relevance to human disease is unclear. We sought to interrogate the prevalence and clinical relevance of Pf phage in patients with cystic fibrosis (CF) using sputum samples from two well-characterized patient cohorts. Bacterial genomic analysis in a Danish longitudinal cohort of 34 patients with CF revealed that 26.5% (n = 9) were consistently Pf phage positive. In the second cohort, a prospective cross-sectional cohort of 58 patients with CF at Stanford, sputum qPCR analysis showed that 36.2% (n = 21) of patients were Pf phage positive. In both cohorts, patients positive for Pf phage were older, and in the Stanford CF cohort, patients positive for Pf phage were more likely to have chronic P. aeruginosa infection and had greater declines in pulmonary function during exacerbations than patients negative for Pf phage presence in the sputum. Last, P. aeruginosa strains carrying Pf phage exhibited increased resistance to antipseudomonal antibiotics. Mechanistically, in vitro analysis showed that Pf phage sequesters these same antibiotics, suggesting that this mechanism may thereby contribute to the selection of antibiotic resistance over time. These data provide evidence that Pf phage may contribute to clinical outcomes in P. aeruginosa infection in CF.

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