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The global burden of bronchiectasis in children and adolescents is being recognised increasingly. However, marked inequity exists between, and within, settings and countries for resources and standards of care afforded to children and adolescents with bronchiectasis compared with those with other chronic lung diseases. The European Respiratory Society (ERS) clinical practice guideline for the management of bronchiectasis in children and adolescents was published recently. Here we present an international consensus of quality standards of care for children and adolescents with bronchiectasis based upon this guideline. The panel used a standardised approach that included a Delphi process with 201 respondents from the parents and patients’ survey, and 299 physicians (across 54 countries) who care for children and adolescents with bronchiectasis. The seven quality standards of care statements developed by the panel address the current absence of quality standards for clinical care related to paediatric bronchiectasis. These internationally derived, clinician-, parent-and patient-informed, consensus-based quality standards statements can be used by parents and patients to access and advocate for quality care for their children and themselves, respectively. They can also be used by healthcare professionals to advocate for their patients, and by health services as a monitoring tool, to help optimise health outcomes. © ERS 2022.

Bronchiectasis is being diagnosed increasingly in children and adolescents. Recurrent respiratory exacerbations are common in children and adolescents with this chronic pulmonary disorder. Respiratory exacerbations are associated with an impaired quality-of-life, poorer long-term clinical outcomes and substantial costs to the family and health systems. The European Respiratory Society (ERS) clinical practice guideline for the management of children and adolescents with bronchiectasis provided a definition of acute respiratory exacerbations for clinical use but to date there is no comparable universal definition for clinical research. Given the importance of exacerbations in the field, this ERS task force sought to obtain robust definitions of respiratory exacerbations for clinical research. The panel was a multidisciplinary team of specialists in paediatric and adult respiratory medicine, infectious disease, physiotherapy, primary care, nursing, radiology, methodology, patient advocacy and parents of children and adolescents with bronchiectasis. We used a standardised process that included a systematic literature review, parents' survey and a Delphi involving 299 physicians (54 countries) caring for children and adolescents with bronchiectasis. Consensus was obtained for all four statements drafted by the panel as the disagreement rate was very low (range 3.6% to 6.4%). The panel unanimously endorsed the four consensus definitions for: non-severe and severe exacerbations as an outcome measure; non-severe exacerbation for studies initiating treatment and; resolution of a non-severe exacerbation; for clinical trials involving children and adolescents with bronchiectasis. This ERS task force proposes using these internationally derived, consensus-based definitions of respiratory exacerbations for future clinical paediatric bronchiectasis research. Copyright © The authors 2022.

Background: The dorsal anterior cingulate cortex (dACC) and its neurocircuits are central in impulsivity, and maladaptive dACC activity has been implicated in psychological disorders c

Bronchiectasis is one of the most neglected diseases in respiratory medicine. There are no approved therapies and few large-scale, representative epidemiological studies. The EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration) registry is a prospective, pan-European observational study of patients with bronchiectasis. The inclusion criterion is a primary clinical diagnosis of bronchiectasis consisting of: 1) a clinical history consistent with bronchiectasis; and 2) computed tomography demonstrating bronchiectasis. Core exclusion criteria are: 1) bronchiectasis due to known cystic fibrosis; 2) age <18 years; and 3) patients who are unable or unwilling to provide informed consent. The study aims to enrol 1000 patients by April 2016 across at least 20 European countries, and 10 000 patients by March 2020. Patients will undergo a comprehensive baseline assessment and will be followed up annually for u p to 5 years with the goal of providing high-quality longitudinal data on outcomes, treatment patterns and quality of life. Data from the registry will be available in the form of annual reports. and will be disseminated in conference presentations and peer-reviewed publications. The European Bronchiectasis Registry aims to make a major contribution to understanding the natural history of the disease, as well as guiding evidence-based decision making and facilitating large randomised controlled trials.

PURPOSE:: To investigate the usefulness of adjunctive electromyographic (EMG) polygraphy in the diagnosis of clinical events captured during long-term video-EEG monitoring. METHOD(S):: A total of 40 patients (21 women, 19 men) aged between 19 and 72 years (mean 43) investigated using video-EEG monitoring were studied. Electromyographic activity was simultaneously recorded with EEG in four patients selected on clinical grounds. In these patients, surface EMG electrodes were placed over muscles suspected to be activated during a typical clinical event. RESULT(S):: Of the 40 patients investigated, 24 (60%) were given a diagnosis, whereas 16 (40%) remained undiagnosed. All four patients receiving adjunctive EMG polygraphy obtained a diagnosis, with three of these diagnoses being exclusively reliant on the EMG recordings. Specifically, one patient was diagnosed with propriospinal myoclonus, another patient was diagnosed with facio-mandibular myoclonus, and a third patient was found to have bruxism and periodic leg movements of sleep. CONCLUSION(S):: The information obtained from surface EMG recordings aided the diagnosis of clinical events captured during video-EEG monitoring in 7.5% of the total cohort. This study suggests that EEG-EMG polygraphy may be used as a technique of improving the diagnostic yield of video-EEG monitoring in selected cases. Copyright © 2014 by the American Clinical Neurophysiology Society.