INTRODUCTION Patients with pulmonary hypertension are classified according to clinical criteria to inform treatment decisions. Knowledge of the molecular drivers of pulmonary hypertension might better inform treatment choice. METHODS Between 2013 and 2021, 470 patients with pulmonary hypertension, 136 disease controls and 59 healthy controls were enrolled as a discovery cohort. Plasma levels of 7288

RATIONALE We hypothesized that the disproportionate impact of social determinants of health (SDoH) captured in survey data could help explain a larger proportion of racial gaps in lung function than previously reported. METHODS We defined a series of nested, increasingly healthy reference populations using data from NHANES 2007-2012. Starting with non-smokers without respiratory symptoms or diagnoses

RATIONALE Hyper IgE syndrome (STAT3-HIES), also known as Job's syndrome, is a rare immunodeficiency disease typically caused by dominant-negative STAT3 mutations. STAT3-HIES is characterized by chronic pulmonary infection and inflammation, suggesting impaired innate host defense. OBJECTIVES To identify airway epithelial host defense defects caused by STAT3 mutations that, together with immune dysfunction

RATIONALE Managing critically ill patients in the ICU often involves organ-support therapies (OST), such as mechanical ventilation, extracorporeal membrane oxygenation, renal replacement therapy, and various pharmacologic strategies. Clinical trials in this context pursue diverse goals-including improving survival, reducing OST use, facilitating weaning, or comparing timing of OST initiation-which

RATIONALE Industrial facilities emit known lung carcinogens into air, but the association of these agents with lung cancer risk at environmental levels is unknown. OBJECTIVES To investigate industrial emissions and lung cancer risk. METHODS We used a U.S. regulatory database to estimate airborne exposure to known and probable human carcinogens (N=31) emitted from industrial sources (1987-1995) for

RATIONALE Patient-important gastrointestinal bleeding is an endpoint developed by patients and family members; however, risk factors for this outcome are unknown. OBJECTIVE To identify risk factors for patient-important upper gastrointestinal bleeding among invasively ventilated adults. METHODS This pre-planned regression analysis of an international trial database evaluated baseline and time-varying

RATIONALE Increased risk of COVID-19 hospitalization and death has been reported among patients with clinical lung disease. OBJECTIVE To test the association of objective measures of pre-pandemic lung function and structure with COVID-19 outcomes in US adults. METHODS Pre-pandemic obstruction (FEV1/FVC<0.70) and restriction (FEV1/FVC≥0.7, FVC<80%) were defined based on the most recent spirometry exam

RATIONALE Clinical and real-world studies show elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) is efficacious and safe in people with cystic fibrosis (CF) ≥12 years of age with at least one F508del allele. OBJECTIVES Given the potential for life-long ELX/TEZ/IVA use, long-term safety and efficacy of ELX/TEZ/IVA was assessed. METHODS In this phase 3, open-label, single-arm extension study, participants

BACKGROUND Patients with sickle cell disease hospitalised for acute chest syndrome (ACS) are at high risk of in situ pulmonary microthrombosis. We evaluated whether therapeutic anticoagulation could shorten ACS duration. METHODS TASC is a randomized, controlled, double-blind trial conducted in 12 French hospitals (December 2016-April 2021) in adult ACS patients with no initial thrombosis on chest computerised

RATIONALE While idiopathic pulmonary fibrosis (IPF) has been widely studied, progressive non-IPF interstitial lung disease (ILD) remains poorly understood. OBJECTIVE To identify and validate proteomic biomarkers of non-IPF ILD survival. METHODS High-throughput proteomic data were generated using plasma collected as part of prospective registries at the Universities of California and Texas (discovery

Rationale: The lungs are the most prevalent site for invasive fungal infections, and the diagnosis and treatment of pulmonary fungal infections pose significant challenges, accompanied by a substantial disease burden. Global factors will likely enhance the risk of it in the future. Assessing the global burden of pulmonary fungal infections is crucial for implementing appropriate measures for prevention

RATIONALE Small airway dysfunction (SAD) is a key feature of COPD and might present in tobacco-exposed adults with normal spirometry. So far, the role of oscillometry-defined SAD in this population is largely unexplored. OBJECTIVE To investigate the prevalence of oscillometry-defined SAD and its associations with airway structural changes, quality of life (QoL), metabolic and cardiovascular disease

INTRODUCTION High-risk pulmonary thromboembolism (PE) is often fatal due to right ventricular heart failure. However, right ventricular echocardiographic parameters that are associated with adverse outcome in PE are incompletely characterized. Our objective was to evaluate if right ventricular global longitudinal strain (RVGLS) and right ventricular outflow tract velocity-time integral (RVOT VTI) might

RATIONALE Randomised clinical trials in intensive care often prioritize disease-focused outcomes rather than patient-centred outcomes. OBJECTIVE To report and evaluate the 'Daily Organ Support for patients on extracorporeal membrane oxygenation (ECMO)', the DOSE outcome. This is a new longitudinal ordinal outcome for clinical trials in patients receiving ECMO. METHODS Prospective, multicentre study