321 Changes in lung function and airway microbiology following ivacaftor therapy in an adult G551D homozygote
نویسندگان
چکیده
منابع مشابه
Ivacaftor in severe cystic fibrosis lung disease and a G551D mutation
Ivacaftor is gene-specific oral therapy for patients with cystic fibrosis who have a cystic fibrosis transmembrane conductance regulator mutation, G551D. To date, limited information is available about the benefit in patients with severe CF related lung disease, as such patients were excluded from the phase III trials. We report the early results on clinical outcomes, sweat electrolytes and C-r...
متن کاملImpact of the CFTR-Potentiator Ivacaftor on Airway Microbiota in Cystic Fibrosis Patients Carrying A G551D Mutation
BACKGROUND Airway microbiota composition has been clearly correlated with many pulmonary diseases, and notably with cystic fibrosis (CF), an autosomal genetic disorder caused by mutation in the CF transmembrane conductance regulator (CFTR). Recently, a new molecule, ivacaftor, has been shown to re-establish the functionality of the G551D-mutated CFTR, allowing significant improvement in lung fu...
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15 صفحه اولIvacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.
Cystic fibrosis (CF) is an autosomal recessive lethal disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that encodes for CFTR, an epithelial cell-surface expressed protein responsible for the transport of chloride (Cl(-)). Gating mutations associated with defective conductance can be modulated by CFTR potentiators. Ivacaftor is a CFTR potentiator...
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ژورنال
عنوان ژورنال: Journal of Cystic Fibrosis
سال: 2015
ISSN: 1569-1993
DOI: 10.1016/s1569-1993(15)30495-1