Table 2 List of new therapies in CF
From: Cystic fibrosis lung environment and Pseudomonas aeruginosa infection
Type of therapy | Name of the therapy | Benefits of the therapy | References |
|---|---|---|---|
Airway clearance therapy | Dornase-alfa | Breakdown of excess DNA in cell debris and mucous of chronically inflamed airways | Mogayzel et al., 2013 [224] |
| Â | Nebulization of hypertonic saline | Increases airway hydration and mucociliary clearance | Donaldson et al., 2006 [225] |
Antibiotics therapy | Tobramycin | Helpful in chronic stages of cystic fibrosis | Mogayzel et al., 2013 [224] |
| Â | Aztreonam lysinate | Improves lung function and reduces incidences of pulmonary exacerbations | Assael et al., 2013 [226] |
| Â | Colistin (colistimethate sodium) | Improves lung function | Schuster et al., 2013 [227] |
Anti-Inflammatory therapy | Azithromycin | Macrolide antibiotic. Long term usage in CF reduces neutrophilic inflammation and pulmonary exacerbations. Improves lung function | Uzun et al., 2014 [228] Saiman et al., 2010 [229] |
| Â | Ibuprofen | Reduces the decline in lung function. Especially effective in pediatric patients | Lands et al., 2007 [230] Mogayzel et al., 2013 [224] |
Gene therapy | 1.Zinc finger nucleases 2.Transcription activator –like effector nucleases (TALENs) 3.RNA guide engineered nucleases (derived from CRISPR) | These nucleases cleave the DNA at a specific site of interest and allow genomic modifications | De Boeck et al., 2014 [231] Dekkers et al., 2012 [232] |
CFTR modulation | 1.Potentiators (VX-770, VX661 Ivacaftor, Lumacaftor, Riociguat, QBW251). 2.PTC therapeutics or ataluren | Aimed at correcting the dysfunction of CFTR like nonsense, frameshift, splice mutations and non-functional CFTR mutations. | Van Goor et al., 2009 [233] Du et al., 2008 62 62 [234] |