Real-life acute lung function changes after lumacaftor/ivacaftor first administration in pediatric patients with cystic fibrosis

摘要

Abstract The combination of lumacaftor and ivacaftor (LUM/IVA) has been reported to induce a mean acute absolute drop of ?4.1% predicted forced expiratory volume in 1s (FEV 1 ) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV 1 changes after the first dose of LUM/IVA in CF patients. A total of 32 pediatric patients were included. Respiratory manifestations occurred in only 3 patients (9.4%), but FEV 1 consistently decreased (?10.4±4.6%, range: ?1.5; ?21.8%). FEV 1 only partially resumed after salbutamol inhalation. Patients with previously known significant reversible airway obstruction and low FEV 1 were more at risk of FEV 1 decrease.