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Minerva Pediatrica 2019 August;71(4):371-5

DOI: 10.23736/S0026-4946.19.05509-9


language: English

Inhaled medications in cystic fibrosis beyond antibiotics

Angela SEPE 1, 2, Valeria R. VILLELLA 3, Chiara CIMBALO 1, 2, Alice CASTALDO 1, 4, Francesco NUNZIATA 1, 2, Adele CORCIONE 1, 2, Gianni BONA 5, Luigi MAIURI 3, 5, Valeria RAIA 1, 2

1 Department of Translational Medical Sciences, University Federico II, Naples, Italy; 2 Department of Pediatrics, University Federico II, Naples, Italy; 3 European Institute for Research in Cystic Fibrosis, San Raffaele Scientific Institute, Milan, Italy; 4 Department of Public Health, University Federico II, Naples, Italy; 5 Department of Health Sciences, University of Eastern Piedmont, Novara, Italy

Structural lung disease begins very early in children with cystic fibrosis (CF), often in the first three months of life. Inhaled medications represent an attractive therapeutic approach in CF that are routinely used as early intervention strategies. Two aerosolized solutions, hypertonic saline and dornase alfa, have significant potential benefits by improving mucociliary clearance, with minimal associated side-effects. In particular, they favor rehydration of airway surface liquid and cleavage of extracellular DNA in the airways, respectively, consequently reducing rate of pulmonary disease exacerbations. Indirect anti-inflammatory effects have been documented for both drugs, addressing each of the three interrelated elements in the vicious cycle of lung disease in CF: airway obstruction, inflammation and infection. This short review aimed to summarize the main papers that support potential clinical impact of inhaled solutions on pulmonary disease in CF.

KEY WORDS: Inhalation; Therapy; Cystic fibrosis; Hypertonic solutions; Dornase alfa

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