The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology
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Multicenter Study Observational Study
Transcriptomic gene signatures associated with persistent airflow limitation in patients with severe asthma.
A proportion of severe asthma patients suffers from persistent airflow limitation (PAL), often associated with more symptoms and exacerbations. Little is known about the underlying mechanisms. Here, our aim was to discover unexplored potential mechanisms using Gene Set Variation Analysis (GSVA), a sensitive technique that can detect underlying pathways in heterogeneous samples. ⋯ Differentially enriched gene signatures were identified in nasal brushings (n=1), sputum (n=9), bronchial brushings (n=1) and bronchial biopsies (n=4) that were associated with response to inhaled steroids, eosinophils, interleukin-13, interferon-α, specific CD4+ T-cells and airway remodelling. PAL in severe asthma has distinguishable underlying gene networks that are associated with treatment, inflammatory pathways and airway remodelling. These findings point towards targets for the therapy of PAL in severe asthma.
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Randomized Controlled Trial Multicenter Study
Selexipag for the treatment of connective tissue disease-associated pulmonary arterial hypertension.
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag. ⋯ Adverse events were predominately prostacyclin-related and known for selexipag treatment. GRIPHON has allowed the comprehensive characterisation of patients with PAH-CTD. Selexipag delayed progression of PAH and was well-tolerated among PAH-CTD patients, including those with PAH-SSc and PAH-SLE.
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Randomized Controlled Trial Multicenter Study
Automatic versus manual oxygen administration in the emergency department.
Oxygen is commonly administered in hospitals, with poor adherence to treatment recommendations. We conducted a multicentre randomised controlled study in patients admitted to the emergency department requiring O2 ≥3 L·min-1 Patients were randomised to automated closed-loop or manual O2 titration during 3 h. Patients were stratified according to arterial carbon dioxide tension (PaCO2 ) (hypoxaemic PaCO2 ≤45 mmHg; or hypercapnic PaCO2 >45-≤55 mmHg) and study centre. ⋯ O2 could be weaned at the end of the study in 14.1% versus 4.3% patients in the automated and manual titration group, respectively (p<0.001). O2 duration during the hospital stay was significantly reduced (5.6±5.4 versus 7.1±6.3 days, p=0.002). Automated O2 titration in the emergency department improved oxygenation parameters and adherence to guidelines, with potential clinical benefits.
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Multicenter Study
Health-related quality of life and risk factors associated with spirometric restriction.
The restrictive spirometric pattern is associated with a substantial morbidity and mortality burden. We sought to determine to what extent spirometric restriction is associated with impaired quality of life. We used data from two large population-based European cohorts: 6698 European Community Respiratory Health Survey (ECRHS) and 6069 Swiss Cohort Study on Air Pollution and Lung and Heart Diseases in Adults (SAPALDIA) adult participants. ⋯ In univariate analyses, compared with the normal group, both the restrictive and obstructive pattern had significant Physical Component Summary deficits (-2.77 and -2.08, respectively, in ECRHS; -3.25 and -2.14, respectively, in SAPALDIA; all p-values <0.001). However, in models adjusted for sex, age, education, body mass index, smoking, comorbidities and respiratory symptoms, only the restrictive pattern remained significantly associated with Physical Component Summary deficits (p=0.004 in ECRHS; p=0.001 in SAPALDIA). The restrictive spirometric pattern is associated with deficits in the physical component of quality of life that are partly independent of the presence of respiratory symptoms.
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Health-related quality of life or health status is significantly impaired in bronchiectasis. There is a paucity of brief, simple-to-use, disease-specific health status measures. The aim of this study was to develop and validate the Bronchiectasis Health Questionnaire (BHQ), a new health status measure that is brief and generates a single overall score. ⋯ The BHQ was highly repeatable after 2 weeks (intraclass correlation coefficient 0.89). The BHQ is a brief, valid and repeatable, self-completed health status questionnaire for bronchiectasis that generates a single total score. It can be used in the clinic to assess bronchiectasis from the patient's perspective.