ERJ Open Research
Top medRxiv preprints most likely to be published in this journal, ranked by match strength.
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IntroductionPatients with primary ciliary dyskinesia (PCD) present a variety of respiratory symptoms. Spirometry, particularly forced expiratory volume in 1 s (FEV1), is the most commonly used outcome measure in clinical follow-up, however, it is not known how well it captures the range of respiratory symptoms experienced by patients. MethodsWe sent the FOLLOW-PCD questionnaire to all patients [≥]14 years and parents of children, registered in the Swiss PCD Registry, asking about upper and l...
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BackgroundThe Primary Ciliary Dyskinesia Rule (PICADAR) is a diagnostic predictive tool currently recommended by the European Respiratory Society (ERS) to assess the likelihood of a primary ciliary dyskinesia (PCD) diagnosis. Despite its recommendation according to the current ERS PCD diagnostic guideline, the performance of the PICADAR remains insufficiently studied. MethodsWe evaluated the sensitivity of PICADAR in 269 individuals with genetically confirmed PCD. Using an initial question, PIC...
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BackgroundQuality of life (QoL), the highest prioritised outcome by children with bronchiectasis and their parents, is a patient-reported outcome measure increasingly considered essential when evaluating health and interventions. Despite this, there are no validated instruments that specifically measure QoL related to bronchiectasis in children. We aimed to develop and validate a new bronchiectasis child-specific parent-proxy QoL instrument (BC-QoL). MethodsWe developed a draft 44-item BC-QoL a...
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IntroductionPeople living with chronic diseases can provide a unique perspective for research that often differs from that of healthcare professionals. This is particularly important in rare diseases like primary ciliary dyskinesia (PCD), with many knowledge gaps and limited research resources. We aimed to assess participation of patients and caregivers in PCD research and identify their research priorities in a mixed- method study. MethodsWe conducted in-depth, semi-structured interviews with ...
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BackgroundPulmonary Radioaerosol Mucociliary Clearance (PRMC) is an in vivo whole lung ciliary function test reliable for assessing mucociliary clearance for diagnostic purposes in individuals suspected of primary ciliary dyskinesia (PCD). We aimed to evaluate expanded use of PRMC by providing advantages and limitations for its potential use in providing outcome parameters in future trials aiming to restore ciliary activity. Material and MethodsIn this retrospective study, PRMC tests performed ...
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Aims of the studyWe know little about the level of physical activity, respiratory physiotherapy practices, and nutritional status of people with primary ciliary dyskinesia (PCD), although these are important aspects of patients with chronic respiratory disease. We assessed physical activity, respiratory physiotherapy practices, and nutritional status among people with PCD in Switzerland, investigated how these vary by age, and identified factors associated with regular physical activity. Method...
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IntroductionPulmonary exacerbations contribute to disease progression in chronic lung diseases. In a large prospective cohort study, we studied the incidence and predictors of pulmonary exacerbations among persons with primary ciliary dyskinesia (PCD), which can inform follow-up care. We also assessed healthcare use, changes in management, and pathogens during exacerbations. MethodsParticipants in the Living with PCD study reported increased respiratory symptoms in the past seven days, indicati...
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BackgroundPrimary ciliary dyskinesia (PCD) is a rare genetic disorder characterized by deficient mucociliary clearance and development of chronic lung disease. Pulmonary exacerbations (PEx) in chronic lung diseases increase morbidity and lung function decline, but their frequency in PCD has been understudied. We aimed to prospectively determine the annual frequency of PEx in PCD and identify related risk factors. MethodsIn a multicentre, observational study conducted in 11 centres from seven co...
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ObjectiveExercise-induced respiratory symptoms (EIS) are common in childhood and reflect different diseases that can be difficult to diagnose. In children referred to respiratory outpatient clinics for EIS, we compared the diagnosis proposed by the referring primary care physician with the final diagnosis from the outpatient clinic and described diagnostic tests performed and treatment prescribed after the diagnostic evaluation. DesignObservational study nested in the Swiss Paediatric Airway Co...
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BackgroundOscillometry may be a feasible and sensitive tool for objective remote monitoring of paediatric asthma. MethodsSchool-aged cohorts of healthy, well controlled and poorly controlled asthma (defined as [≥]2 exacerbations within the preceding 12m) performed daily home-based oscillometry for 3-4 months (C-100 tremoflo, Thorasys Ltd), alongside objective measures of asthma control (ACQ weekly, ACT monthly), medication use (Hailie(R)) and exacerbations. Day-to-day variability was calcul...
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BackgroundEffective initial asthma treatment in primary care requires a comprehensive assessment of airway inflammation and lung function impairment, including small airway dysfunction (SAD). We investigated the prevalence of type 2 (T2) inflammation and SAD in treatment-naive patients with uncontrolled asthma who received primary care, and assessed the utility of the Asthma Control Questionnaire-5 (ACQ-5) as a potential indicator of these factors. MethodsThis single-center retrospective study ...
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BackgroundChildren with night cough but no wheeze might have a mild form of asthma (cough variant asthma), sharing risk factors with children who wheeze, and possibly developing wheeze later. MethodsWe compared risk factors of children with isolated night cough and children with wheeze in the Leicester Respiratory Cohort study at ages 1, 4, 6, and 9 years. We also compared prognoses of children with isolated night cough, children with wheeze, and asymptomatic children. ResultsAmong 4,101 child...
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BackgroundAsthma is an umbrella diagnosis encompassing distinct pathophysiological mechanisms. While a global problem, our understanding of the interplay between respiratory microbiology and airway inflammation is largely from populations in high income settings. As a result, treatment approaches align poorly with asthma characteristics in less studied populations. ObjectiveTo identify conserved and geographically distinct relationships between airway inflammation and microbiota characteristics...
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BackgroundThe imposition of restrictions on social mixing early in the COVID-19 pandemic was followed by a reduction in asthma exacerbations in multiple settings internationally. Temporal trends in social mixing, incident acute respiratory infections (ARI) and asthma exacerbations following relaxation of COVID-19 restrictions have not yet been described. MethodsWe conducted a population-based longitudinal study in 2,312 UK adults with asthma between November 2020 and April 2022. Details of face...
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BackgroundClinical practice guidelines for asthma diagnosis are rarely evaluated in real-life practice. Within the Swiss Paediatric Airway Cohort (SPAC), we initiated the SPAC-asthma project to develop a standardised diagnostic approach for school-aged asthma, based on the algorithm recommended by the European Respiratory Society (ERS) guideline. Here, we report the development and feasibility of this approach after implementation across multiple paediatric pulmonology clinics. MethodWe used a ...
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RationaleWhether asthma constitutes a risk factor for COVID-19 is unclear. MethodsWe performed a systematic literature search in three stages: First, we reviewed PubMed, EMBASE and CINAHL for systematic reviews of SARS-CoC-2 and COVID-19 in pediatric populations, and reviewed their primary articles; next, we searched PubMed for studies on COVID-19 or SARS-CoV-2 and asthma/wheeze, and evaluated whether the resulting studies included pediatric populations; lastly, we repeated the second search in...
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BACKGROUNDVariation in genetic ancestry among admixed racial/ethnic groups may influence the fit of guideline-recommended spirometry reference equations, which rely on self-identified race/ethnicity. RESEARCH QUESTIONWhat is the influence of genetic ancestry on the fit of the guideline-recommended racial/ethnic-based spirometry reference equations in populations of genetically admixed children? STUDY DESIGN AND METHODSCross-sectional fit of guideline-recommended racial/ethnic-based spirometry ...
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BackgroundAsthma is a complex chronic inflammatory disease affecting approximately 339 million people worldwide. The ADAM33 gene, encoding a disintegrin and metalloproteinase, has emerged as a key susceptibility gene for asthma, with the rs2280091 (T1) polymorphism showing variable associations across different populations. This study represents the first genetic investigation of asthma in the Syrian population. MethodsA case-control study was conducted at Aleppo University Hospital from April ...
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Mucociliary clearance is a key component of the pathophysiology of bronchiectasis but cilia function is poorly defined. This study aims to characterize nasal ciliary function in bronchiectasis and examine associations with disease severity, infection, inflammation and outcome. Adults with bronchiectasis and healthy volunteers were recruited to the international observational study EMBARC-BRIDGE. Individuals with a known diagnosis of Primary Ciliary Dyskinesia (PCD) were excluded. Nasal respira...
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BackgroundLung oscillometry is an emerging lung function test for assessing obstructive airway disease. Comparisons of oscillometry parameters and their bronchodilator responsiveness (BDR) between bronchial asthma and chronic obstructive pulmonary disease (COPD) patients are limited. Research QuestionDo oscillometry parameters and their BDR differ between stable asthma and COPD patients with similar severity of airflow obstruction? Study Design and MethodsWe included 467 consecutive adult pati...