CFTR and cystic fibrosis: COPD results from damage to airways and lung parenchyma [2]; CF is caused by a mutation in the Cystic Fibrosis Transmembrane conductance Regulator gene (CFTR), affecting bronchial epithelium mucus production leading to lung impairment and infection [3]; PAH is a condition involving a remodelling of pulmonary vessels causing right heart failure [4], [5].