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- W2888302472 abstract "Cystic fibrosis (CF) is an autosomal dominant chloride channelopathy caused by mutations in the CF transmembrane conductance regulator (CFTR) gene that results clinically in a multisystem disorder. The major source of morbidity and mortality in CF is lung disease, which is characterized by recurrent cycles of inflammation and infection and progressive respiratory decline. Therapeutics have traditionally focused on the downstream effects of the primary genetic defect. However, recent advances have shifted attention to modulation of upstream pathways and the defective CFTR protein itself. Areas covered: This review focuses on emerging pharmacotherapeutics for CF lung disease, with an emphasis on the evidence for CFTR modulators and a summary of emerging modulator therapies currently in phase II and III clinical trials as of July 2018. Results of relevant trials reported in peer-reviewed journals, scientific conference abstracts, and sponsor press releases are included. This manuscript also discusses new and upcoming advances in anti-inflammatory therapy, anti-infectives, mucolytics, and gene editing. Expert commentary: The therapeutic landscape in CF has changed dramatically in recent years, with significant benefits for patients. Cure is now a realistic target in those with specific mutations who commence CFTR-directed therapy prior to the onset of significant airways disease." @default.
- W2888302472 created "2018-08-31" @default.
- W2888302472 creator A5011360899 @default.
- W2888302472 creator A5013200565 @default.
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- W2888302472 creator A5023945170 @default.
- W2888302472 creator A5037444903 @default.
- W2888302472 creator A5062384147 @default.
- W2888302472 date "2018-09-19" @default.
- W2888302472 modified "2023-09-26" @default.
- W2888302472 title "Emerging pharmacotherapies in cystic fibrosis" @default.
- W2888302472 cites W1523596819 @default.
- W2888302472 cites W1525140521 @default.
- W2888302472 cites W1586387535 @default.
- W2888302472 cites W1589654967 @default.
- W2888302472 cites W1627442108 @default.
- W2888302472 cites W1646314937 @default.
- W2888302472 cites W1661091834 @default.
- W2888302472 cites W1780057455 @default.
- W2888302472 cites W1865743538 @default.
- W2888302472 cites W1878292374 @default.
- W2888302472 cites W1965400044 @default.
- W2888302472 cites W1965472603 @default.
- W2888302472 cites W1976197028 @default.
- W2888302472 cites W1977725157 @default.
- W2888302472 cites W1978129709 @default.
- W2888302472 cites W1982969769 @default.
- W2888302472 cites W1986316308 @default.
- W2888302472 cites W1988397255 @default.
- W2888302472 cites W1993871313 @default.
- W2888302472 cites W1994940189 @default.
- W2888302472 cites W1997212073 @default.
- W2888302472 cites W2002423049 @default.
- W2888302472 cites W2003171404 @default.
- W2888302472 cites W2004155358 @default.
- W2888302472 cites W2014745588 @default.
- W2888302472 cites W2015617337 @default.
- W2888302472 cites W2018306826 @default.
- W2888302472 cites W2019099644 @default.
- W2888302472 cites W2022634662 @default.
- W2888302472 cites W2024183228 @default.
- W2888302472 cites W2024882709 @default.
- W2888302472 cites W2027173302 @default.
- W2888302472 cites W2035131077 @default.
- W2888302472 cites W2040578546 @default.
- W2888302472 cites W2041831203 @default.
- W2888302472 cites W2045716945 @default.
- W2888302472 cites W2047963123 @default.
- W2888302472 cites W2048083566 @default.
- W2888302472 cites W2051836609 @default.
- W2888302472 cites W2056247812 @default.
- W2888302472 cites W2058093119 @default.
- W2888302472 cites W2066122648 @default.
- W2888302472 cites W2068244888 @default.
- W2888302472 cites W2071407356 @default.
- W2888302472 cites W2072054870 @default.
- W2888302472 cites W2073059894 @default.
- W2888302472 cites W2074207970 @default.
- W2888302472 cites W2074624473 @default.
- W2888302472 cites W2078308455 @default.
- W2888302472 cites W2078380427 @default.
- W2888302472 cites W2079791074 @default.
- W2888302472 cites W2083774511 @default.
- W2888302472 cites W2084896237 @default.
- W2888302472 cites W2085811948 @default.
- W2888302472 cites W2088707861 @default.
- W2888302472 cites W2094703380 @default.
- W2888302472 cites W2095687634 @default.
- W2888302472 cites W2098548967 @default.
- W2888302472 cites W2099252894 @default.
- W2888302472 cites W2101007662 @default.
- W2888302472 cites W2113453073 @default.
- W2888302472 cites W2115046089 @default.
- W2888302472 cites W2116707205 @default.
- W2888302472 cites W2117424591 @default.
- W2888302472 cites W2121996960 @default.
- W2888302472 cites W2122967691 @default.
- W2888302472 cites W2126330038 @default.
- W2888302472 cites W2126458759 @default.
- W2888302472 cites W2128809044 @default.
- W2888302472 cites W2128903674 @default.
- W2888302472 cites W2129119043 @default.
- W2888302472 cites W2129859001 @default.
- W2888302472 cites W2131008616 @default.
- W2888302472 cites W2132240172 @default.
- W2888302472 cites W2132347745 @default.
- W2888302472 cites W2134492797 @default.
- W2888302472 cites W2135511125 @default.
- W2888302472 cites W2140868856 @default.
- W2888302472 cites W2144292013 @default.
- W2888302472 cites W2146179684 @default.
- W2888302472 cites W2148186686 @default.
- W2888302472 cites W2149185787 @default.
- W2888302472 cites W2149850085 @default.
- W2888302472 cites W2152355434 @default.
- W2888302472 cites W2153344788 @default.
- W2888302472 cites W2153576774 @default.
- W2888302472 cites W2154038782 @default.