The cystic fibrosis transmembrane regulator gene, one of the most commonly mutated in the European population, was cloned in 1989 and since then has been extensively analysed in patients of various ethnic backgrounds. We have screened the entire coding sequences of the cystic fibrosis transmembrane regulator gene and identified many mutations and polymorphisms. In this paper we propose a general strategy to improve prenatal diagnosis and genetic counselling of cystic fibrosis (CF). As this approach based on denaturing gradient gel electrophoresis is adaptable to different populations, it greatly increases the sensibility and specificity of CF prenatal diagnosis.
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http://dx.doi.org/10.1159/000263850 | DOI Listing |
Eur Clin Respir J
January 2025
Adult Cystic Fibrosis Centre, The Prince Charles Hospital, Brisbane, Queensland, Australia.
Therapeutic drug monitoring (TDM) of elexacaftor/tezacaftor/ivacaftor (ETI) remains challenging due to a lack of clarity around the parameters that govern ETI plasma concentrations, whilst the use of concomitant CYP3A inducers rifabutin and rifampicin is not recommended. We present the complexities of TDM for ETI performed in a person with cystic fibrosis and refractory pulmonary disease. Utilising National Association of Testing Authorities (NATA) accredited assays and target considerations published by the Therapeutic Goods Administration (TGA), Australia, ETI plasma concentration variability was monitored over the course of an acute admission with added complexity from an antibiotic regimen including rifabutin, a moderate cytochrome P450 3A (CYP3A) inducer, and clofazimine, a mild CYP3A inhibitor.
View Article and Find Full Text PDFERJ Open Res
January 2025
Respiratory Medicine, Department of Translation Medicine, University of Ferrara, Ferrara, Italy.
https://bit.ly/4gyJHFW.
View Article and Find Full Text PDFERJ Open Res
January 2025
B. Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel.
https://bit.ly/45DjFw9.
View Article and Find Full Text PDFJ Mater Chem B
January 2025
Institute of Materials Science and Technology, Technische Universität Wien, Vienna, Austria.
The reason why certain bacteria, , (PA), produce acetylated alginate (Alg) in their biofilms remains one of the most intriguing facts in microbiology. Being the main structural component of the secreted biofilm, like the one formed in the lungs of cystic fibrosis (CF) patients, Alg plays a crucial role in protecting the bacteria from environmental stress and potential threats. Nonetheless, to investigate the PA biofilm environment and its lack of susceptibility to antibiotic treatment, the currently developed biofilm models use native seaweed Alg, which is a non-acetylated Alg.
View Article and Find Full Text PDFGut Pathog
January 2025
Francis I Proctor Foundation, University of California San Francisco, 490 Illinois St, San Francisco, CA, 94158, USA.
Background: Asymptomatic carriers significantly influence the transmission dynamics of C. difficile. This study aimed to assess the prevalence of toxigenic C.
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