Diurnal Cycle of Water Vapor as Documented by a Dense GPS Network in a Coastal Area during ESCOMPTE IOP2

Global positioning system (GPS) data from a dense network have been used for the analysis of the diurnal cycle of water vapor over Marseille, France, during the second intensive observation period (IOP2; 21–26 June 2001) of the Expérience sur Sites pour Contraindre les Modèles de Pollution Atmosphérique et de Transport d'Emission (ESCOMPTE) field experiment. Both tomographic analyses and integrated water vapor (IWV) contents from GPS have been used, in addition to wind profiler data and surface observations. Tomographic analysis of data from the dense GPS network and radiosondes provided the continuous temporal evolution of the vertical distribution of water vapor over the city of Marseille. The city is located on the shore of the Mediterranean Sea in southeastern France and is often under the influence of sea-breeze effects. Two different layers of breeze circulation are identified: a shallow sea breeze, blowing perpendicular to the local coastline, and a deep sea breeze, induced by the regional temperature gradient between sea and land. The origin of water vapor is shown to be mainly due to the advection of marine moist air by these sea-breeze circulations. However, the diurnal cycle of water vapor over Marseille is strongly influenced by the synoptic situation, which changes during the IOP2 (between a northerly mistral in the early stage of the IOP and an easterly wind at the end). It is shown that vertical profiles from tomographic analyses (combined with wind profiler data) allow for a proper interpretation of the diurnal cycle observed in IWV. Two-dimensional maps of IWV are also shown to complement the description of the horizontal advection of moisture by the different circulation regimes

On the use of GPS tomography to investigate water vapor variability during a Mistral//sea breeze event in southeastern France

Global Positioning System (GPS) tomography analyses of water vapor, complemented by high-resolution numerical simulations are used to investigate a Mistral/sea breeze event in the region of Marseille, France, during the ESCOMPTE experiment. This is the first time GPS tomography has been used to validate the three-dimensional water vapor concentration from numerical simulation, and to analyze a small-scale meteorological event. The high spatial and temporal resolution of GPS analyses provides a unique insight into the evolution of the vertical and horizontal distribution of water vapor during the Mistral/sea-breeze transition

Rapid Improvement after Starting Elexacaftor–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease

International audienceRationale: Elexacaftor-tezacaftor-ivacaftor is a CFTR (cystic fibrosis [CF] transmembrane conductance regulator) modulator combination, developed for patients with CF with at least one Phe508del mutation. Objectives: To evaluate the effects of elexacaftor-tezacaftor- ivacaftor in patients with CF and advanced respiratory disease. Methods: A prospective observational study, including all patients aged ⩾12 years and with a percent-predicted FEV1 (ppFEV1) <40 who initiated elexacaftor-tezacaftor-ivacaftor from December 2019 to August 2020 in France was conducted. Clinical characteristics were collected at initiation and at 1 and 3 months. Safety and effectiveness were evaluated by September 2020. National-level transplantation and mortality figures for 2020 were obtained from the French CF and transplant centers and registries. Measurements and Main Results: Elexacaftor-tezacaftor- ivacaftor was initiated in 245 patients with a median (interquartile range) ppFEV1 = 29 (24-34). The mean (95% confidence interval) absolute increase in the ppFEV1 was +15.1 (+13.8 to +16.4; P < 0.0001), and the mean (95% confidence interval) in weight was +4.2 kg (+3.9 to +4.6; P < 0.0001). The number of patients requiring long-term oxygen, noninvasive ventilation, and/or enteral tube feeding decreased by 50%, 30%, and 50%, respectively (P < 0.01). Although 16 patients were on the transplant waiting list and 37 were undergoing transplantation evaluation at treatment initiation, only 2 received a transplant, and 1 died. By September 2020, only five patients were still on the transplantation path. Compared with the previous 2 years, a twofold decrease in the number of lung transplantations in patients with CF was observed in 2020, whereas the number of deaths without transplantation remained stable. Conclusions: In patients with advanced disease, elexacaftor-tezacaftor-ivacaftor is associated with rapid clinical improvement, often leading to the indication for lung transplantation being suspended