Sorbonne Université, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Paris, France.
AP-HP, Groupe Hospitalier Universitaire APHP-Sorbonne Université, sites Pitié-Salpêtrière, Saint-Antoine et Tenon, Service des Explorations Fonctionnelles de la Respiration, de l'Exercice et de la Dyspnée (Département R3S), Paris, France.
Eur Respir Rev. 2021 Nov 30;30(162). doi: 10.1183/16000617.0395-2020. Print 2021 Dec 31.
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.
囊性纤维化是一种常染色体隐性遗传病，由于囊性纤维化跨膜电导调节因子的缺失或功能异常，在白种人群中是最常见的、危及生命的遗传疾病。肺部特别容易受到影响，因为浓稠而坚韧的黏液会导致实质异常，包括支气管扩张、进行性气流受限、呼吸道感染、肺部破坏，最终导致呼吸衰竭。尽管治疗方面取得了显著进展，大大提高了生存率，但大多数患者的运动能力仍逐渐受限，因此，在未来几年，越来越多的囊性纤维化患者将需要进行基于运动的评估。心肺运动试验，特别是一项有用的工具，可以评估个体患者运动不耐受的机制，这可能具有治疗和预后意义。在这篇综述中，我们将重点关注通气效率及其在成人囊性纤维化患者中的临床和预后价值。
