TY - JOUR T1 - Bridging for lung transplantation with lumacaftor/ivacaftor JF - Breathe JO - Breathe SP - e68 LP - e71 DO - 10.1183/20734735.019318 VL - 14 IS - 2 AU - Søren Sperling Pedersen AU - Søren Jensen-Fangel AU - Majbritt Jeppesen Y1 - 2018/06/01 UR - http://breathe.ersjournals.com/content/14/2/e68.abstract N2 - Cystic fibrosis (CF) is a multi-organ disease caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene, with consequent dysfunction of the CFTR channel. The most common mutation in CF is delta F508. CFTR-modulating therapy with lumacaftor/ivacaftor corrects (lumacaftor) the misfolded CFTR channel and potentiates (ivacaftor) its function at the cell surface [1].The case of a young female cystic fibrosis patient, homozygous for delta F508 and with terminal respiratory insufficiency, who started treatment with lumacaftor/ivacaftor http://ow.ly/4I0t30kftDx ER -