TY - JOUR AU - Gomez-Pastrana, David AU - Nwokoro, Chinedu AU - McLean, Mike AU - Brown, Sarah AU - Christiansen, Nanna AU - Pao, Caroline S PY - 2018 DO - 10.1016/j.anpedi.2018.05.022 UR - http://hdl.handle.net/10668/12817 T2 - Anales de pediatria AB - Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that has been shown to improve the nutritional status and lung function of cystic fibrosis patients with the G551D mutation in clinical trials. The objective of this... LA - es KW - Cystic fibrosis KW - Fibrosis quística KW - Ivacaftor KW - Real-life KW - Vida real KW - Adolescent KW - Aminophenols KW - Aspergillosis, Allergic Bronchopulmonary KW - Child KW - Chloride Channel Agonists KW - Cystic Fibrosis KW - Cystic Fibrosis Transmembrane Conductance Regulator KW - Female KW - Genotype KW - Humans KW - Male KW - Mutation KW - Mycobacterium Infections, Nontuberculous KW - Mycobacterium abscessus KW - Nutritional Status KW - Pseudomonas Infections KW - Pseudomonas aeruginosa KW - Quinolones KW - Treatment Outcome TI - [Real-world effectiveness of ivacaftor in children with cystic fibrosis and the G551D mutation]. T2 - Efectividad de ivacaftor en vida real en niños con fibrosis quística y mutación G551D. TY - research article VL - 90 ER -