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

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
Aurélie LabastePhilippe Reix

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 (FEV1) after a unique administration in healthy subjects. The aim of the present study was to assess acute FEV1 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 FEV1 consistently decreased (-10.4±4.6%, range: -1.5; -21.8%). FEV1 only partially resumed after salbutamol inhalation. Patients with previously known significant reversible airway obstruction and low FEV1 were more at risk of FEV1 decrease.

Citations

Apr 2, 2019·Expert Review of Respiratory Medicine·Pi Chun ChengRonald C Rubenstein
Oct 12, 2019·American Journal of Respiratory and Critical Care Medicine·Pierre-Régis BurgelUNKNOWN French Cystic Fibrosis Reference Network Study Group
Mar 22, 2020·European Respiratory Review : an Official Journal of the European Respiratory Society·Michal Shteinberg, Jennifer L Taylor-Cousar
Nov 17, 2019·The Journal of Pharmacology and Experimental Therapeutics·Ashvani K SinghChris Tse
Dec 31, 2020·Journal of Clinical Medicine·Renée V E DagenaisBradley S Quon

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