Marlène Murris-Espin

TL;DR: Lumacaftor-ivacaftors safety and effectiveness was associated with improvement in lung disease and nutritional status in patients who tolerated treatment and adults who discontinued, often due to adverse events, were found at high risk of clinical deterioration.

TL;DR: Use of the eFlow rapid nebulizer reduced TSI nebulization time and the systemic exposure to tobramycin appeared to be broadly similar in this exploratory study.

TL;DR: Patients with CF exhibit poor sleep quality that does not predict nocturnal gas exchange, and Nocturnal hypoxaemia and hypercapnia can be identified by simple tools.

TL;DR: A relative homogeneity in these French centers' stated indications for and use of Niv is found, which highlights their numerous expectations about the benefits of NIV, which contrasts with the few validated benefits.

TL;DR: In this paper , lung transplant candidates eligible for elexacaftor-tezacaftor -ivacaftors were evaluated over one year with a reduction in treatment burden and lung transplantation could be safely deferred in most patients.