Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease.

Background: Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports.

Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults.

Background And Objective: Variants in surfactant genes SFTPC or ABCA3 are responsible for interstitial lung disease (ILD) in children and adults, with few studies in adults.

Methods: We conducted a multicentre retrospective study of all consecutive adult patients diagnosed with ILD associated with variants in SFTPC or ABCA3 in the French rare pulmonary diseases network, OrphaLung. Variants and chest computed tomography (CT) features were centrally reviewed.

Liver disease in germline mutations of telomere-related genes: Prevalence, clinical, radiological, pathological features, outcome, and risk factors.

Background And Aim: Germline mutations of telomere-related genes (TRG) induce multiorgan dysfunction, and liver-specific manifestations have not been clearly outlined. We aimed to describe TRG mutations-associated liver diseases.

Approach And Results: Retrospective multicenter analysis of liver disease (transaminases > 30 IU/L and/or abnormal liver imaging) in patients with TRG mutations.

Amikacin Liposomal Inhalation Suspension in the Treatment of Lung Infection: A French Observational Experience.

Background: infections remain difficult to manage in both cystic fibrosis (CF) and non-CF patients and reported clinical outcomes are largely unsatisfactory. Clinical trial data are limited and no approved therapies are currently available for the management of lung diseases. As an alternative, cohort studies may provide insightful information into the management of pulmonary disease.

Hypnosis associated with 3D immersive virtual reality technology during bronchoscopy under local anesthesia.

Background: Patients undergoing flexible bronchoscopy under local anesthesia usually experience anxiety before and during the procedure. Different non-pharmacological techniques, including music and hypnosis, are used to distract patients' attention, and to reduce anxiety. The new technique "virtual reality hypnosis (VRH)", defined as a hypnotic induction suggestion delivered by personalized virtual reality software, can generate a simulation of a lifelike environment.

Radial-EBUS and virtual bronchoscopy planner for peripheral lung cancer diagnosis: How it became the first-line endoscopic procedure.

Background: Various advanced bronchoscopy methods have been developed to reach peripheral lung lesions (PLL). In a large cohort, we aimed to assess a standardized procedure of first-line radial-endobronchial ultrasound (r-EBUS) and virtual bronchoscopy planner for the diagnosis of peripheral lung cancer.

Methods: This retrospective, single center study included patients who had r-EBUS-guided bronchoscopy for the diagnosis of a PLL between 2008 and 2019.

Therapeutic Drug Monitoring of Sputum Voriconazole in Pulmonary Aspergillosis.

Voriconazole is one of the most used antifungal azoles against pulmonary aspergillosis. Therapeutic drug monitoring (TDM) of the voriconazole concentration in plasma is recommended in clinical practice guidelines to prevent treatment failure and toxicity. The aim of this study was to evaluate the feasibility and utility of TDM of the voriconazole concentration in the sputum of patients treated for pulmonary aspergillosis.

Virtual Bronchoscopy Planner and Radial-EBUS Guided Biopsy for Organizing Pneumonia Diagnosis.

Background: The diagnosis of organizing pneumonia (OP) often requires histological confirmation. The aim of this retrospective study was to evaluate the diagnostic yield and complication rate of radial endobronchial ultrasound (r-EBUS) for OP.

Methods: All patients who had r-EBUS as a first diagnostic procedure for a peripheral pulmonary lesion at Rouen University Hospital, France, between April 2008 and December 2020 were included.

Impact of CYP2C19 genetic polymorphisms on voriconazole dosing and exposure in adult patients with invasive fungal infections.

Voriconazole (VCZ) use is limited by its narrow therapeutic range and significant interpatient variability in exposure. This study aimed to assess (i) the impact of CYP2C19 genotype on VCZ exposure and (ii) the doses required to achieve the therapeutic range in adult patients with invasive fungal infections (IFIs). Therapeutic drug monitoring (TDM) of VCZ, based on trough concentration measurement, and CYP2C19 genotyping were used to guide VCZ dosing in Caucasian patients with IFIs.