The Effects of Ivacaftor on Bone Density and Microarchitecture in Children and Adults with Cystic Fibrosis

Context Cystic fibrosis (CF) transmembrane conductance (CFTR) dysfunction may play a role in CF-related bone disease (CFBD). Ivacaftor is a CFTR potentiator effective in improving pulmonary and nutritional outcomes in patients with the G551D-CFTR mutation. The effects of ivacaftor on bone health are unknown. Objective To determine the impact of ivacaftor on bone density and microarchitecture in children and adults with CF. Design Prospective...