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European Respiratory Journal 05/27/2020 19:05
Cystic fibrosis (CF) is a common multi-system genetically inherited condition, predominately found in individuals of Caucasian decent. Since the identification of the cystic fibrosis (CF) transmembrane conductance regulator ( CFTR ) gene in 1989, and the subsequent improvement in understanding of CF pathophysiology, significant increases in life-expectancy have followed. Initially this was related to improvements in the management and systems of care for treating the various affected organ systems. These cornerstone treatments are still essential for CF patients born today. However, over the last decade, the major advance has been in therapies that target the resultant genetic defect: the dysfunctional CFTR protein. Small molecule agents th.
European Respiratory Journal 05/27/2020 19:05
We read with interest the recent editorial by Keeley et al. [1] on the timely topic of changing pressurised metered dose inhalers (pMDIs) to dry powder inhalers (DPIs) due to the much higher carbon footprint of pMDIs [2]. We agree with the authors that global warming cannot be tackled only by focusing on inhaler devices, but as long as we can provide safe and effective treatment to our patients, we cannot overlook environmental facts either. We feel that Keeley et al. [1] gave an unjustified negative impression on the performance of DPIs. They imply that DPIs are more expensive than pMDIs, that switching from pMDI to DPI leads to poorer asthma control, and that patients using DPIs should have a pMDI+spacer rescue pack since DPIs cannot be r.
European Respiratory Journal 05/27/2020 19:05
Lymphangioleiomyomatosis (LAM) is a multisystem disease associated with progressive pulmonary disease that affects almost exclusively women. LAM is characterised pathologically by proliferation of abnormal smooth muscle-like cells carrying mutations in predominantly the tuberous sclerosis complex (TSC) gene TSC2 and, rarely, in TSC1 [1]. LAM can occur sporadically or in association with TSC. Mutations in the TSC genes lead to activation of the mammalian/mechanistic target of rapamycin complex 1 (mTORC1) [2]. In the landmark randomised controlled MILES (Multicenter International LAM Efficacy of Sirolimus) trial, the mTORC1 inhibitor rapamycin stabilised lung function and improved symptoms in LAM patients with moderate to severe changes in lu.
European Respiratory Journal 05/27/2020 19:05
The rapid outbreak of coronavirus disease 2019 (COVID-19) has been a matter of international concern as the disease is spreading fast [1, 2]. Considering that the contagious disease has led to an enormous impact globally, there is an urgent need to identify the risk populations with poor prognosis. Ageing is associated with certain changes in pulmonary physiology, pathology and function, during the period of lung infection. Therefore, age-related differences in responsiveness and tolerance become obvious and lead to worse clinical outcomes in elderly individuals [3]. Previous studies have mentioned that older COVID-19 patients are at an increased risk of death [4–7]. However, the age-related clinical characteristics, disease courses and out.
European Respiratory Journal 05/27/2020 19:05
Chronic bacterial infection may play an important role in the progression of COPD [1] with the pulmonary inflammatory response driven by neutrophils [2]. Neutrophil elastase (NE) is a serine protease stored and secreted by neutrophils [3] and is an essential defence against bacterial infection [4].
European Respiratory Journal 05/27/2020 19:05
Coronavirus disease 2019 (COVID-19) has now become a worldwide health concern. The severity of COVID-19 has been classified as mild, moderate, severe and critical [1]. To date, there have been a few studies focused on the clinical course and outcome of critical cases [2–4]. However, information regarding outcomes of mild-to-moderate cases is lacking, despite the fact that mild-to-moderate cases have accounted for approximately 80% of laboratory-confirmed patients [1, 5]. This study aimed to investigate short-term outcomes of patients rated with different severities on admission, and to identify risk factors for progression, thereby helping the management of COVID-19 in clinical practice.
European Respiratory Journal 05/27/2020 19:05
There is an emerging role for blood eosinophil count (EOS) as a biomarker to guide inhaled corticosteroid (ICS) therapy in COPD. Since ICS administration could influence EOS, we hypothesised that change in EOS following treatment with ICS may predict outcomes of long-term therapy. In a post hoc analysis of ISOLDE, a 3-year, double-blind trial comparing 500 µg fluticasone propionate twice daily with placebo in 751 patients with moderate-to-severe COPD, we evaluated whether the initial changes in EOS during ICS treatment were predictive of ICS treatment response. EOS change within 1 year after the introduction of ICS was strongly predictive of treatment response. A suppressed EOS was associated with treatment effect. Characteristically, in pa.
European Respiratory Journal 05/27/2020 19:05
The recently published Global Lung Function Initiative (GLI) carbon monoxide transfer factor ( T LCO ) reference equations provide an opportunity to adopt a current, all-age, widely applicable reference set. The aim of this study was to document the effect of changing to GLI from commonly utilised reference equations on the interpretation of T LCO results. 33 863 T LCO results (48% female, 88% Caucasian, n=930 aged <18 years) from clinical pulmonary function laboratories within three Australian teaching hospitals were analysed. The lower limit of normal (LLN) and proportion of patients with a T LCO below this value were calculated using GLI and other commonly used reference equations. The average T LCO LLN for GLI was similar or lower than
European Respiratory Journal 05/27/2020 19:05
31 May, 2020 is World Health Organization (WHO) World No Tobacco Day. The theme this year is "Protecting youth from industry manipulation and preventing them from tobacco and nicotine use" [1]. WHO wishes this year's campaign to serve the following: 1) debunk myths and expose manipulation tactics employed by the tobacco and nicotine industries, particularly marketing tactics targeted at youth, including through the introduction of new and novel products, flavours and other attractive features; 2) equip young people with knowledge about the tobacco and nicotine industries' intentions and tactics to hook current and future generations on tobacco and nicotine products; and 3) empower influencers (in pop culture, on social media, in the home, o.
European Respiratory Journal 05/27/2020 19:05
We thank L. Lehtimäki and colleagues for their letter, and acknowledge and apologise for the error they identify relating to the country of the study [1]. All respiratory inhaled treatments are a combination of the drug and device, and we cited the Icelandic study to illustrate that an enforced switch of treatment by the government, here to low-cost alternatives, led to poorer clinical outcomes [1]. In this context, the mandated recommendations of the UK government for environmental reasons, give cause for concern. Our editorial stresses the necessity for patient engagement and choice, as well as clinical efficacy, to be considered in all steps of inhaler prescribing.
European Respiratory Journal 05/26/2020 10:35
The paediatric respiratory research community uses cohort studies extensively. However, the landscape of these studies and their quality of reporting has not been assessed. We performed a systematic review of publications on cohort studies reporting on paediatric lower respiratory problems published in 2018. We searched Medline and EMBASE and extracted data on the studies' and journals' characteristics. We assessed the number of items of the STrengthening the Reporting of OBservational studies in Epidemiology (STROBE) checklist that a random sample (100 papers) reported. We analysed factors associated with the STROBE score and with the most poorly reported items, using Poisson and logistic regression. Of the 21 319 records identified, 369 fu.
European Respiratory Journal 05/26/2020 10:35
Oxygenation failure recalcitrant to increasing positive end-expiratory pressure is a feature of severe coronavirus disease 2019 (COVID-19) pneumonia [1]. A Chinese group [2] used prone positioning to improve oxygenation for intubated patients with severe COVID-19 pneumonia. However, prone positioning in unconscious patients is labour-intensive and is associated with various complications [3, 4]. As the incidence of severe COVID-19 pneumonia worldwide increases rapidly, many countries are also facing the problem of diminishing intensive care resources.
European Respiratory Journal 05/26/2020 10:35
Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including the pancreas, hepatobiliary system and reproductive organs however lung disease is responsible for the majority of morbidity and mortality. Management of CF involves CFTR modulator agents including corrector drugs to augment cellular trafficking of mutant CFTR as well as potentiators that open defective CFTR channels. These therapies are poised to help most individuals with CF, with the notable exception of individuals with class I mutations where full length CFTR protein is not produced. For these mutations, gene replacement has been suggested as a potential solution. In this work, we used a helper dependent adenoviral vector (HD-CFTR) to express CFTR in nasal
European Respiratory Journal 05/26/2020 10:35
The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) disease (COVID-19) pandemic has attracted interest because of its global rapid spread, clinical severity, high mortality rate, and capacity to overwhelm healthcare systems [1, 2]. SARS-CoV-2 transmission occurs mainly through droplets, although surface contamination contributes and debate continues on aerosol transmission [3–5].
European Respiratory Journal 05/26/2020 10:35
MicroRNAs (miRs) have been shown to disrupt normal lung development and function by interrupting alveolarization and vascularisation leading to development of bronchopulmonary dysplasia (BPD). Here we report that miR-184 has a critical role in the induction of BPD phenotype characterised by abnormal alveolarization and pulmonary angiogenesis in the developing lung. We observed an increased expression of miR-184 in BPD clinical specimens: tracheal aspirates (TA), human neonatal lungs with BPD and in fetal human lung Type II alveolar epithelial cells (TIIAECs) exposed to hyperoxia. Consistent with this, we also detected an upregulated miR-184-3p expression in whole lungs, in freshly isolated TIIAECs from lungs of hyperoxia-induced experimenta.
European Respiratory Journal 05/25/2020 15:04
The European Respiratory Society (ERS)/European Society of Thoracic Surgeons (ESTS)/European Association for Cardio-Thoracic Surgery (EACTS)/European Society for Radiotherapy and Oncology (ESTRO) task force brought together experts to update previous 2009 ERS/ESTS guidelines on management of malignant pleural mesothelioma (MPM), a rare cancer with globally poor outcome, after a systematic review of the 2009–2018 literature.
European Respiratory Journal 05/22/2020 09:03
The catastrophic impact of the coronavirus disease 2019 (COVID-19) epidemic in Italy has been described previously [1, 2]. In a recent editorial, Nava and co-authors pay a well-deserved tribute to the almost 200 health care workers who succumbed to COVID-19 in the country in less than 3 months since the first case was reported [3]. We would like to contribute with some considerations on the shortcomings of Italy's response to the pandemic.
European Respiratory Journal 05/22/2020 09:03
A strategy of early extubation to non-invasive respiratory support in preterm infants could be boosted by the availability of a decision-support tool for clinicians. Using the Heart Rate Characteristics index (HRCi) with clinical parameters, we derived and validated predictive models for extubation readiness and success. Peri-extubation demographic, clinical and HRCi data for up to 96 h were collected from mechanically ventilated infants in the control arm of a randomised trial involving 8 neonatal centres, where clinicians were blinded to the HRCi scores. The data was used to produce a multivariable regression model for the probability of subsequent re-intubation. Additionally, a survival model was produced to estimate the probability of r.
European Respiratory Journal 05/22/2020 09:03
The COVID-19 pandemic is likely to be the defining global health crisis of our generation. As the United Nations Development Programme highlighted in their recent call to action, the impact of this pandemic will extend beyond the immediate medical consequences to have far-reaching and long-lasting social and economic impacts, threatening to disproportionately affect poorer people in poorer countries [1]. Income losses are anticipated to exceed $220 billion in developing countries, where many people live day-to-day without access to social protection, and food security is precarious [1]. Strikingly, a recent United Nations study suggests that the social and economic consequences of the COVID-19 pandemic could increase the number of people li.

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