TY - JOUR T1 - Ventilatory efficiency and its clinical and prognostic value in adults with cystic fibrosis JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW DO - 10.1183/16000617.0395-2020 VL - 30 IS - 162 SP - 200395 AU - Pierantonio Laveneziana AU - Paolo Palange Y1 - 2021/12/31 UR - http://err.ersjournals.com/content/30/162/200395.abstract N2 - Cystic fibrosis, due to the absence or abnormal function of the cystic fibrosis transmembrane conductance regulator, is the most common life-limiting autosomal recessive genetic disorder among the Caucasian population. The lungs are particularly affected due to thick and tenacious mucus causing parenchymal anomalies ranging from bronchiectasis, progressive airflow limitation, respiratory infections, lung destruction and ultimately respiratory failure. Despite the remarkable advances in treatment that have greatly improved survival, most patients experience progressive exercise curtailment, with the consequence that a growing number of patients with cystic fibrosis will be referred for exercise-based evaluations in the forthcoming years. Cardiopulmonary exercise testing, in particular, is a useful tool to assess the mechanisms of exercise intolerance in individual patients that may have treatment and prognostic implications. In this review, we will focus on ventilatory efficiency and its clinical and prognostic value in adults with cystic fibrosis.Cardiopulmonary exercise testing is a useful tool to assess the mechanisms of exercise intolerance in individual patients with CF and may have treatment and prognostic implications. https://bit.ly/3uH0oq9 ER -