Impact of CFTR-modulating drugs on GH-IGF-1 axis impairment in adult patients with cystic fibrosis

A new class of drugs in the treatment of cystic fibrosis (CF) includes two agents: lumacaftor, which corrects CFTR channel protein, and ivacaftor, which increases CFTR channel activity. In our previous study we recruited 50 stable adults with CF and 16 of them showed growth hormone deficit (GHD): 7 patients severe and 9 patients partial GHD. We decided to re-evaluate ten patients with the GHRH + arginine test of whom only five were treated with...