The French compassionate programme of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del variant.

Background: The European Medicines Agency has approved the cystic fibrosis transmembrane conductance regulator (CFTR) modulator combination elexacaftor/tezacaftor/ivacaftor (ETI) for people with cystic fibrosis (CF) carrying at least one F508del variant. The United States Food and Drug Administration (FDA) also approved ETI for people with CF carrying one of 177 rare variants.

Methods: An observational study was conducted to evaluate the effectiveness of ETI in people with CF with advanced lung disease who were not eligible for ETI in Europe.

Diaphragmatic weakness related to radiotherapy: 2 cases and a review.

Post-radiation diaphragmatic weakness have rarely been described. We report two cases of post-radiation diaphragmatic weakness from our center, and review the other published cases, computing clinical, electromyography and magnetic resonance imaging data. Including our two cases, seven cases of post-radiation diaphragmatic weakness have been described.

Assessment of pulmonary arterial circulation 3 months after hospitalization for SARS-CoV-2 pneumonia: Dual-energy CT (DECT) angiographic study in 55 patients.

Background: During COVID-19, the main manifestations of the disease are not only pneumonia but also coagulation disorders. The purpose of this study was to evaluate pulmonary vascular abnormalities 3 months after hospitalization for SARS-CoV-2 pneumonia in patients with persistent respiratory symptoms.

Methods: Among the 320 patients who participated in a systematic follow-up 3 months after hospitalization, 76 patients had residual symptoms justifying a specialized follow-up in the department of pulmonology.

X-ray powder diffraction in reflection geometry on multi-beam kJ-type laser facilities.

An ultrafast x-ray powder diffraction setup for laser-driven dynamic compression has been developed at the LULI2000 laser facility. X-ray diffraction is performed in reflection geometry from a quasi-monochromatic laser-generated plasma x-ray source. In comparison to a transmission geometry setup, this configuration allows us to probe only a small portion of the compressed sample, as well as to shield the detectors against the x-rays generated by the laser-plasma interaction on the front side of the target.

Long-term evaluation of home-based pulmonary rehabilitation in patients with fibrotic idiopathic interstitial pneumonias.

Background: Few studies have examined the benefits of pulmonary rehabilitation in patients with fibrotic idiopathic pulmonary pneumonia (f-IIP). Here, we report the results of an observational study in routine clinical practice of home-based pulmonary rehabilitation for f-IIP patients.

Methods: A total of 112 consecutive patients (61 with idiopathic pulmonary fibrosis and 51 with fibrotic nonspecific interstitial pneumonitis) were enrolled, of whom 65 had mild-to-moderate disease (forced vital capacity (FVC) ≥50% predicted and diffusing capacity of the lung for carbon monoxide ( ) ≥30% predicted) and 47 had severe disease (FVC <50% predicted and/or <30% predicted).