Showing papers in "Breathe in 2014"

How to avoid interface problems in acute noninvasive ventilation

Abstract: Summary Noninvasive ventilation (NIV) applied via different interfaces is increasingly used in the treatment of acute respiratory failure. One of the key factors determining the success of NIV is the choice of interface. Interface selection, fitting and handling can be challenging as NIV application can be complicated by discomfort, air leaks, skin damage or conjunctivitis. The aim of this article is to provide practical information on interface choice, technical aspects of mask fitting and prevention of mask-related problems during the acute delivery of NIV. Educational aims To help the reader to recognise the different features of interfaces and to discuss common interface-related problems To provide practical recommendations to facilitate the choice and application of interfaces in order to reduce complications

Long-acting bronchodilators in COPD: where are we now and where are we going?

Abstract: Educational aims To discuss fundamental questions relating to the use of bronchodilators that can lead to an optimisation of their utilisation. To describe new bronchodilators that have recently been approved in some countries or are currently undergoing clinical development Summary Bronchodilators are central to the treatment of chronic obstructive pulmonary disease (COPD) because they alleviate bronchial obstruction and airflow limitation, reduce hyperinflation, and improve emptying of the lung and exercise performance. For this reason, all guidelines highlight that inhaled bronchodilators are the mainstay of the current management of all stages of COPD. However, there are still fundamental questions regarding their use that require clarification to optimise utilisation of these drugs. It is crucial to address the following questions. Is it appropriate to treat all COPD patients with long-acting bronchodilators? Is it better to start treatment with a β2-agonist or with an anti-muscarinic agent in patients with stable mild/moderate COPD? Is it useful to use a bronchodilator with rapid onset of action? Is it preferable to administer a bronchodilator on a once- or twice-daily basis? Can a second bronchodilator can be introduced for patients with stable COPD (“dual” bronchodilator therapy), and if so when? Are inhaled corticosteroids (ICSs) really useful in COPD patients without chronic bronchitis, since long-lasting bronchodilators may prevent exacerbations even in the absence of an ICS in frequent exacerbators? Finally, is combined therapy really useful in non-frequent exacerbators? Due to the the central role of bronchodilators in the treatment of COPD, there is still considerable interest in finding novel classes of bronchodilator drugs. However, new classes of bronchodilators have proved difficult to develop because either new emerging targets are not really important and/or it is difficult to find substances capable of interacting with them. As a consequence, many research groups have sought to improve the existing classes of bronchodilators.

Breathing exercises for asthma

Abstract: Educational aims To summarise the evidence of the role of breathing control approaches in the management of asthma To provide information on the content of evidence-based breathing exercises programmes Summary Asthma is a complex, multi-dimensional condition that affects patients in many ways. Having asthma is inherently stressful and psychological problems are common and associated with poor asthma outcomes. Although most patients in clinical trials can achieve high levels of control with optimised pharmacotherapy, in “real-life” practice, poor control is common, with over-reliance on rescue bronchodilator medication and ongoing symptoms and quality-of-life impairment. Many patients are interested in non-pharmacological treatments to improve asthma control, particularly breathing control exercises but, until recently, the evidence base has been inadequate. The place of breathing exercises has been controversial, partly because some proponents have made exaggerated, implausible claims of effectiveness. Recent evidence, however, has resulted in endorsement of breathing exercises as add-on treatment in asthma in systematic reviews and guidelines. This review summarises the current evidence of effectiveness of breathing exercises programmes as an adjuvant treatment to pharmacological strategies for people with asthma. The types of breathing training programmes used and the content of effective programmes are discussed. We conclude that patients whose asthma continues to cause symptoms and quality-of-life impairment, despite adequate pharmacological treatment, or who have high bronchodilator use, should be offered access to an effective breathing training programme as a part of holistic, integrated asthma care. Key points Asthma is frequently poorly controlled despite effective modern medication Psychological factors can be as important as physiological ones in affecting symptom perception and disease impact Breathing exercises can improve patient-reported outcomes and psychological state Breathing exercises should be offered to all asthma patients with symptoms or impaired quality of life despite standard treatment

Delivering telemedicine interventions in chronic respiratory disease

Abstract: Educational Aims To explain the basic principles of telemedicine applicable to chronic respiratory diseases To review telemedicine interventions for patients with chronic respiratory diseases To outline the advantages and limitations (including cost and barriers to implementation) of telemedicine for patients with chronic respiratory diseases To propose recommendations for clinical management of patients receiving telemedicine for chronic respiratory diseases

A core syllabus for post-graduate training in respiratory physiotherapy

Abstract: Physiotherapy contributes significantly to improving quality of life for patients with respiratory disease [1, 2]. Physiotherapists specialised in dealing with respiratory pathology and its associated problems are not only central in the delivery of pulmonary rehabilitation but also provide strategies and techniques for exercise testing, airway clearance, breathlessness management, mobility and function improvement and pain management [2]. Published evidence-based recommendations have paved the way for standardised practice [1–6] while also unravelling the extended scope of responsibilities of the respiratory physiotherapist [7]. The breakdown of traditional roles and allocation of new responsibilities is not confined to the respiratory physiotherapist within healthcare systems. Team-based healthcare and interprofessional treatment of patients is prevalent in the provision of care across the globe. New methods of healthcare delivery indicate that tasks are entrusted to those deemed competent to perform them [8]. It has therefore been necessary to train allied health professionals to take over parts of clinical care [9]. Mobility of the healthcare workforce has been at the forefront of discussion among healthcare providers and policy makers [10]. We now live in a “global village” [11], the consequence of which has seen a marked increase in health professionals completing part of their training and work experience abroad [10]. In this transition to international healthcare, more and more institutions, countries and regions will need standards to meet public, patient and healthcare provider expectations. This reform in medical practice is thus motivated by the breakdown of traditional roles and responsibilities within the healthcare system [12], increasing trends towards mobility [13] and more complex patient needs. These motivators for change have emphasised a new reality for healthcare systems bounded by a skilled healthcare …

Primary ciliary dyskinesia

Abstract: Educational aims To describe the emerging genetics of primary ciliary dyskinesia (PCD) and the heterogeneity of the disease To highlight the clinical symptoms and signs suggestive of PCD that should lead to consideration of diagnostic testing To highlight the difficulties in diagnosing PCD emphasising the need for specialist diagnostic centres To discuss current treatment strategies and highlight the lack of an evidence base for these Summary Primary ciliary dyskinesia (PCD) is a rare heterogeneous genetic disorder affecting ciliary function. Genes coding for various ciliary structural proteins or cytoplasmic proteins responsible for the assembly of cilia can be mutated resulting in abnormal ciliary function. However, despite the diversity of genotypes that can cause PCD the clinical phenotypes of PCD are all remarkably similar. The main clinical symptoms are caused by a lack of mucociliary clearance. Worryingly many patients are diagnosed late despite their classical, lifelong symptoms of a daily wet sounding cough and rhinosinusitis. Even when PCD is suspected, poor access to specialist diagnostic centres may delay diagnosis. Currently, diagnostic testing includes screening of nasal nitric oxide, followed by nasal brushing to obtain ciliated epithelial strips for high-speed video analysis of ciliary function. This is typically followed by transmission electron microscopy and in difficult cases by ciliated cell culture. Emerging tests including immunofluorescence and genetic examination are the focus of intense research and are likely to feature more in the future. Despite a greater understanding of the pathogenesis of PCD and improved diagnostic testing, management strategies are currently based on expert opinion with little, if any, evidence base.

How to: Bronchial thermoplasty in asthma

Abstract: Educational aims To provide an overview on how to perform bronchial thermoplasty for the treatment of asthma To summarise the development, mode of action and evidence for the effectiveness of bronchial thermoplasty as well as its place in the management of asthma Summary Bronchial thermoplasty, which involves the delivery of radio frequency energy to the airways, is a nonpharmacological intervention developed for the treatment of moderate-to-severe asthma. The mode of action of bronchial thermoplasty is not established, but could be due to reduction in airway smooth muscle mass induced by thermal energy. Bronchial thermoplasty results in modest improvements in asthma quality of life questionnaire scores and clinically worthwhile reductions in severe exacerbations and emergency department visits in the year post-treatment, which may persist for up to 5 years. The procedure involves systematic, controlled heating of the airways using dedicated radiofrequency equipment during a series of three bronchoscopy sessions. Bronchial thermoplasty causes short-term increases in asthma-related morbidity, including hospital admissions. Follow-up data, to date, supports the long-term safety of the procedure. Bronchial thermoplasty is a novel treatment option for selected patients with moderate-to-severe asthma that is poorly controlled despite maximal therapy. Key points Bronchial thermoplasty, which involves the delivery of radio frequency energy to the airways, is a nonpharmacological intervention developed for the treatment of patients with moderate-to-severe asthma that is poorly controlled despite maximal therapy Randomised controlled clinical trials of bronchial thermoplasty in patients with moderate and severe asthma have shown modest improvements in asthma quality of life and clinically worthwhile reductions in severe exacerbations and emergency department visits The treatment involves three bronchoscopy sessions with repeated, precise and carefully recorded activations of a radiofrequency catheter within the medium and large airways Bronchial thermoplasty causes short-term increases in asthma-related morbidity including increased admissions to hospital for asthma; follow-up data, to date, supports the longterm safety of the procedure

Monitoring early lung disease in cystic fibrosis: where are we now?

Abstract: Educational aims To understand which techniques are available to monitor early lung disease in cystic fibrosis, recognise difficulties inherent to each measure, and describe the use of tests in clinical practice or interventional trials. Summary It is a particular challenge to detect and monitor lung disease in children younger than 6 years old. Various methods exist that can reveal abnormality in young children, but each technique presents difficulties on performing the test or interpreting results. Most children with cystic fibrosis are now diagnosed shortly after birth, but it is still unclear how to evaluate new therapies, or at what age to initiate them. This review summarises current options for monitoring early disease, limitations of individual techniques and how evidence to date influences their use in research and clinical practice. Key points Lung disease remains the major cause of morbidity and mortality in cystic fibrosis Despite clear improvements in outcomes such as nutrition, the benefit of early diagnosis by newborn screening on pulmonary health in cystic fibrosis has yet to be proven. Early lung disease is challenging to detect and monitor in young children with cystic fibrosis, but there is an urgent requirement to define useful outcome measures in this population, particularly as new treatments emerge. Many techniques are available, but difficulties with measurement or interpretation must be recognised when appraising past studies, considering their use in practice or as endpoints in clinical trials. There is no superior monitoring test at present, but emerging evidence from longitudinal studies in children with cystic fibrosis will help define which are most useful.

Childhood asthma phenotypes in the twenty-first century

Abstract: Summary A substantial proportion of asthma has its origins in childhood but attempts to identify modifiable exposures that trigger asthma inception have yielded disappointing results. It is increasingly recognised that asthma may not be a single disease entity but instead consists of a number of phenotypes that share common features perhaps with different aetiologies and pathophysiological pathways leading to disease. The aim of this review is to describe approaches to defining asthma phenotypes on the basis of clinical and epidemiological data and to consider the clinical implications of the phenotypes described. Differences in clinical features (atopy, lung function, prognosis) have been associated with phenotypes derived from unsupervised statistical approaches to phenotype definitions. Greater understanding of the genetic pathways underpinning asthma has also led to some insights into asthma endotypes that manifest as different clinical phenotypes. However, the challenge to translate these findings into interventions to change the natural history of asthma has yet to be overcome. Technological advances are driving the creation of massive data resources that can be brought to bear on this problem. Whether they will finally solve the problem of phenotypic complexity in asthma remains to be discovered. If they do, the possibility of personalised interventions for asthma may eventually be realised.

Breathe clean air : the role of physicians and healthcare professionals

Abstract: Summary The health effects of air pollution have a lot in common with those related to active or passive smoking. However, environmental problems pose a rather different set of challenges to physicians and other healthcare professionals. Four target levels of action may result in a reduction of the health impact of air pollution. The first two levels act on the environment rather than the individual: 1) abatement of ambient air pollution at the source to improve ambient air quality; and 2) reduction of pollution in the indoor environments where people spend most of their time. The other two downstream strategies depend entirely on the individual: 3) individual action to reduce personal exposure or dose; and 4) treatments taken to modify personal responses to air pollution, and/or to strengthen defence mechanisms.

How to make sense of a Cochrane systematic review

Abstract: More and more papers are published in medical journals every day, so how do you decide which ones to read and, having read a paper, how do you decide whether to change your practice as a result of what you have read? Perhaps the paper was atypical in some way. What does the other research on the topic say? The purpose of systematic reviews is to summarise all the available, high-quality evidence that can be found on a particular topic. A narrative review, in which an expert can cite a selection of papers that support a particular viewpoint, says very little about the papers that do not. In contrast, a systematic review involves a search for all available literature, whatever the findings may be. Systematic reviews start with a well-defined clinical question, and aim to identify, appraise, synthesise and then apply all the available good-quality evidence that can be found (published or unpublished) that is relevant to the question. In particular, Cochrane systematic reviews have to meet a defined set of quality standards and the authors and editors set out to make them the best around. They are the current gold standard in the systematic review field. The Cochrane Collaboration is an international group which is now 20 years old. The collaboration depends upon the voluntary contribution of thousands of authors and is supported by editorial bases and methodologists. In the UK, these bases are supported by funding from the National Institute of Health Research (NIHR), and any funding for editorial bases must be free from commercial interests. Review groups are divided up into areas of clinical interest and, within the respiratory field, there are groups for lung cancer (based in France), acute respiratory infections (based in Australia), cystic fibrosis (based in the UK) and airways (based in the UK …

Improving the quality of tracheostomy care

Abstract: Educational aims To understand the current challenges in the care of tracheostomy patients To understand principles of quality improvement collaboration and how this can improve the quality of care for tracheostomy patients Summary The UK National Confidential Enquiry into Patient Outcomes and Death illustrates that there remains significant morbidity and mortality relating to patients with a tracheostomy, with much preventable harm. Challenges include the inherent complexity of the patient9s underlying condition, wide variations in tracheostomy management, variable delivery of education for staff, patients and families, and difficult coordination of care between such a variety of individuals involved in performing, managing and ultimately removing tracheostomies. Quality-improvement collaboratives are groups of institutions with a common purpose who work together to drive positive change. They help support clinicians in developing skills and teams necessary to design and sustain quality-improvement cycles. They are a cost-effective way of rapidly disseminating improvement strategies and engaging in shared learning across institutions around the world. The Global Tracheostomy Collaborative aims to improve quality of care and outcomes through five interdependent key drivers: coordinated multidisciplinary team care, education, institution-wide protocols, family and patient-centred care, and metrics and outcomes using a specifically designed database.

e-Learning for the medical team: the present and future of ERS Learning Resources

Abstract: The term “e-learning” is a comprehensive concept that involves the use of all kinds of electronic media and information and communication technology in education. The broad concept of e-learning in general includes the implementation of all kinds of educational technology in learning and teaching procedures, including online learning and computer-delivered instructions. The foundations of modern e-learning were laid down in 1953–1956 by Burrhus Frederic Skinner (fig. 1) who developed the radical behaviourism theory. Skinner invented the operant conditioning chamber and constructed the first teaching machine which simply presented problems in random order to the learner and gave feedback after each response. In the following years, he developed early computer-based training (CBT) programmes that followed the idea of "programmed instruction" [1], in which material was carefully sequenced in small parts to build a systematic process of descriptions, questions and answers. Skinner's early teaching machines had substantial limitations. For example, while learners were rewarded for correct responses, they received no feedback or explanation when their responses were incorrect. Figure 1 B.F. Skinner: an early pioneer of e-learning. Image: Silly rabbit, Wikimedia Commons under CC BY-3.0. In 1960, Norman Crowder tried to solve this problem with a technique called "intrinsic programming”. The basic principle of this approach was that the learner’s responses determined which material was subsequently presented. At the end of each section, a multiple-choice question was presented to the learner and each choice was linked to another section. If the learner's response was correct, new material was presented. If it was incorrect, review or reinforcement material was presented [2]. Another early computer-assisted instruction system called PLATO (Programmed Logic for Automated Teaching Operation) was developed in 1960 at the University of Illinois (Champaign, IL, USA). The "PLATO compiler" allowed the development of various forms of "teaching logic" and was the …

Respiratory Critical Care HERMES: European curriculum recommendations

Abstract: As intensive care medicine has progressed and advanced in terms of technology, diagnostics and therapeutics, the demand for intensive care medicine has increased continuously, resulting in pressures on resources and healthcare budgets. For these reasons, there is a need to create adequate legal and administrative structures, plus an increasing requirement for qualified specialised personnel and internationally standardised, mutually recognised training programmes [1]. This is especially relevant considering the multidisciplinary nature of intensive care medicine. Bearing this in mind, a way forward for intensive care medicine is to involve more respiratory physicians because they can be significant providers of care to critically ill respiratory patients [2]. Intermediate care units providing noninvasive monitoring and noninvasive ventilation allow a more efficient and cost-effective approach without decreasing the quality of care or adversely affecting outcome [3]. They make it possible for patients with respiratory diseases of intermediate severity to be cared for in specialised units headed by respiratory physicians [4]. The development of weaning centres and long-term care facilities, including home ventilation, provide important economic advantages that decrease the burden on regular intensive care units (ICUs) by reducing admissions and facilitating discharge to step-down areas [1]. The respiratory physician can provide advantages to patients in these critical care settings, adapting relatively easily to the needs of the patient [2]. Historically in Europe, respiratory physicians have not usually been in the forefront of caring for the critically ill respiratory patient. This is in contrast with the situation in the USA, where pulmonologists are among the first specialists to participate in the treatment of ICU patients [5]. There is however a growing interest in Europe for the role of the respiratory physician, reflected by a trend towards collaboration between intensive care specialists …

Doing Science: Writing conference abstracts

Abstract: In this edition of Doing Science, we will address abstract writing, with a focus on conference abstracts. By providing an opportunity for discussing your work with your peers in specialised meetings, writing and submitting an abstract is often the very first step when you want to show the world the results of your work; be it your research, a clinical case or a review of the literature [1]. However, it can be be a daunting task to condense hours and hours of hard work into abstract format. But fear not! This edition of Doing Science will give you several approaches to writing abstracts, using your own data as well as that of others. Before we begin, a key message: always remember that writing an abstract follows typically the same path as writing a paper [2]. Begin by planning it, before actually writing it, proofreading it, sharing it with colleagues and finally doing the final revision and editing before you submit. In most cases, the keys to success are an important research question and interesting material to analyse in the hope of answering it. That being said, good abstract writing skills will increase your acceptance rate even for data of moderate importance, or seemingly complicated research ideas. So let’s begin... As every researcher knows, the function of a scientific abstract is to provide an overview of your work. But keep in mind that the abstract is what the referees will use to decide whether your work is accepted or rejected for presentation on the meeting. Also, have you remembered that the abstract is the only part of a paper that is published in conference proceedings [3]? Many researchers will even acknowledge that when they scroll through a conference programme, they look only at the titles …

LAM: Lymphangioleiomyomatosis: The patient and healthcare professional perspective

Abstract: I remember experiencing shortness of breath several months, or even years, before my lymphangioleiomyomatosis (LAM) diagnosis. Then, in 2007, I started to have major symptoms, including dry cough, dyspnoea and a high fever, and I was hospitalised for X-rays and a CT scan. The doctors saw that my lungs were filled with cysts, and a bronchoscopy revealed LAM cells in my tissues. My LAM diagnosis was confirmed in September 2007 at Dr Harari’s hospital in Milan. Before receiving my final diagnosis, I had a lot of panic attacks due to the fact that I did not know what was wrong with me. Doctors told me that my condition was stressrelated and they did not understand that I had an illness, which made me feel very anxious. Finding out that I had LAM was frightening but, in a way, it was good to finally have an explanation for my symptoms. It surprised me, as I’ve always led a very active lifestyle and have never smoked. When I was told that my lungs were like those of someone who smokes 100 cigarettes a day, I was very shocked. I felt despair but tried to seek a solution. I told myself that, just because there is currently no cure for LAM, it does not mean that there will not be in the future. I founded LAM Italia and the European LAM Federation, through which I try to promote research and therapy for the disease. After my diagnosis, I carried on experiencing cough and fatigue, and was quite weak. I tried to improve my symptoms by taking care of my body: eating a healthier diet rich in fruit and vegetables and doing mild exercise like yoga and lifting light weights. I was also prescribed bronchodilators. Since my diagnosis, my condition has been relatively stable. My daily symptoms are cough and anxiety due to the fact that I have a rare disease, but I have not experienced the more severe effects, like pneumothorax. I have worked hard to manage my negative state of mind, which can affect breathing and worsen the effects of LAM. I speak to many patients who get stuck in a vicious cycle of fear and panic, which causes them to not look after themselves properly and leads to more physical weakness stopping them from being able to face everyday life. As the President of LAM Italia and the European LAM Federation, I am very often in contact with people newly diagnosed with LAM. The first thing that I tell them is to make sure that they have ongoing support from a national centre with experience of LAM. I also try to give them a message of hope. LAM is not the same in everyone, there are both moderate and aggressive forms, so I tell them not to compare themselves with other people with LAM as this causes unnecessary stress. I also advise them to stay as healthy as possible, and recommend that they share their experiences with other patients through patient organisations and support groups. I think that it is very important to raise awareness of LAM among family doctors, who are often the first point of contact for women with the disease. And it would be great if, in the future, everyone were to know about LAM. It is also vital to promote trials of new potential drugs at a European level and to ERS 2014 The patient and healthcare professional perspective

The problems and limitations of cohort studies

Abstract: Summary Chronic obstructive pulmonary disease (COPD) is the third most common cause of mortality worldwide and it is important to discover whether risk factors can be identified from studies undertaken in childhood. Numerous longitudinal cohort studies have been developed in many parts of the world to better understand the long-term outcomes of chronic respiratory diseases. Using data they have generated, it should be possible to identify specific risk factors in children and develop a model to prioritise their importance when found, in order to consider ways to reduce the prevalence and/or severity of disease in adults. However, this does require the sharing of data within the field, as is happening in other related fields, such as the Virtual International Stroke Trial Archive (www.vista.gla.ac.uk). Pooling of the raw data could be very informative and an organisation such as the European Respiratory Society could play an important role in ensuring this happens. Unfortunately, cohort studies vary widely in their inclusion criteria, their methodology and the format in which lung function data are presented. The raw data required to develop a model to assess the impact of childhood risk factors on future lung function have not been made available from many of the published articles. Our initial belief that recognised risk factors are independent variables was naive and a different approach is required to better understand their interdependence.

Patient voices at the ERS International Congress 2014: highlighting LAM

Abstract: Lymphangioleiomyomatosis (LAM) is a rare disease (the mean±sd incidence of LAM per million female population between 2004 and 2008 was 0.31±0.058 cases per year in the USA, 0.23±0.076 in the UK and 0.37±0.26 in Switzerland [1]), which almost exclusively affects women at the stage of their lives where they may start to have children. The condition itself is characterised by the development of lung cysts formed from the proliferation of abnormal smooth muscle cells and chyle-filled cystic lesions in the lymphatic system. The main symptoms of LAM include breathlessness, wheeze, chest tightness and can include cough. Patients with LAM often experience one or more pneumothorax, particularly during pregnancy; and it is the development of these which may indicate a diagnosis of LAM [2]. Figure 1 Iris Bassi: European LAM Federation President The impact of LAM is huge: primarily women in the prime of their life with new families and their hopes and expectations for the future being limited by an attack on their lungs. One of the major issues surrounding LAM, as with many other rare diseases, is the lack of awareness amongst …

Doing Science: Peer reviewing

Abstract: External peer review is a hallmark of science. “Published in a peer-reviewed journal” is a sign of quality, meaning that the work has been scrutinised by knowledgeable and independent peers. Moreover, peer review also serves the purpose of improving the work after the authors have done their best and, thus, being a reviewer carries a responsibility. However, it comes with little reward; reviewing is usually done without financial compensation and often outside of work hours. But reviewing is rewarding in itself! Besides being pro bono , it exposes you, as a reviewer, to novel findings and adds a new perspective to your own research and scientific writing. And you can add “Peer reviewer for Journal of X” to your CV. This Doing Science article will deal with peer reviewing of journal articles and conference abstracts. For those of you familiar with the series, the format will be slightly different. Different people tend to have rather personal approaches to reviewing, and rather than generic tips, this article is built around an interview with a very experienced reviewer, and our own personal reflections as reviewers and editors. Figure 1 The typical review process for a scientific peer-reviewed journal. After the author, maybe with trembling hands, hits the “Submit article” button, an e-mail titled “Please be associate editor for a new manuscript” appears in my mailbox. After scanning the title and abstract of the manuscript, I click “agree” if the manuscript is not clearly outside my area of expertise. You would be surprised how wide the range of topics of received manuscripts is! As an author, it is mandatory to carefully select the journal to submit to and to choose a clear, descriptive title and adequate key words, to increase your chances of reaching the right Editor immediately, and also aid the Editor in finding …

Healthy Lungs for Life campaign: a festival of lung health for the ERS International Congress, Munich and the globe

Abstract: The European Respiratory Society (ERS) will hold its annual International Congress this year in Munich, Germany. The ERS congress has been growing and evolving over the last 10 years, with attendance soaring to a steady 20 000. The congress is home to all stakeholders in respiratory medicine including researchers, clinicians, allied professionals and industy. More recently, working together with the European Lung Foundation (ELF), patients and policy makers have started to attend the congress and play a key role in discussions and activities. Each year the ERS congress is becoming more diverse and more relevant to all key players in the field, but clearly it has limitations as in order to attend the congress people have to travel, which is not always possible. With this in mind, in 2014, we want to make sure that the ERS congress, and all of us who take part in it, will have a bigger impact than previously realised, not just on those attending the ERS congress, but to a truly global audience. Figures from the latest edition of the European Lung White Book [1] give the startling message that one in eight Europeans currently die from a lung condition. Lung diseases, over the past two decades, have been a globally important cause of death and disability, and are set to remain as such. However the nature of lung …

News from the Underground: What were the most important scientific/clinical take-home messages for juniors?

Abstract: The ERS International Congress has traditionally been a splendid opportunity to present current updates in clinical respiratory medicine and new research findings. This year’s programme was packed, making it impossible to attend all sessions, but juniors interested in specific fields were there for you to highlight the most important take home messages from the 2014 ERS International Congress in Munich. Amongst the most important news for the clinicians was the publication of a Global Initiative for Asthma (GINA) guidelines update [1]. In the updated guidelines, the heterogeneity of asthma is emphasised, suggesting the need of “4P” (Personalised, Predictive, Preventive and Participatory) treatment regimes. Another clinically important issue recognised in GINA and the Global Initiative for Obstructive Lung Disease (GOLD) guidelines [2], is asthma–chronic obstructive pulmonary disease overlap syndrome (ACOS). ACOS has a special pathophysiological and clinical characteristic and large-scale studies have been initiated to investigate it. In the future of individualised medicine, validated biomarkers are urgently required that can assist in the correct phenotyping of lung diseases and may help to predict treatment responsiveness. A huge number of novel molecules has been introduced for the treatment of airway disorders. The presented studies clearly highlighted the priorities of future research topics, namely the need to investigate methodological and physiological factors and to cross-validate biomarker studies between different centres in large cohorts of subjects. A number of sessions at Munich showed that epidemiology and environmental exposure are vital factors in translational research. The biggest buzz was surrounding the Sunday Grand Round session “Chemicals made to harm”. New …

GPs Meet Rare Lung Disorders Task Force factsheet: lung disease in children with immunodeficiencies

Abstract: Chronic lung diseases in children are classified as primary and secondary lung disease. Secondary lung disease may develop due to several systemic disorders including primary ciliary dyskinesia, cystic fibrosis, due to oesophagus atresia and trachea–oesophagal fistula, neuromuscular disease and immunodeficiencies. In the lung, there are several different defence mechanisms against infections, both local and general. In this respect we relates to systemic causes of reduced immune defence. Primary immunodeficiencies may present with different clinical pictures due to different pathogenic mechanisms involved. There are disorders of antibody function, T-cell disorders, phagocytic disorders and others including severe combined immunodeficiency (SCID). The antibody function disorders are the most common and include X-linked and non-X-linked a- and hypo-γ-globulinaemia, common variable hypo-γ-globulinaemia, immunoglobulin (Ig)G subclass deficiencies, IgA …

GPs Meet Rare Lung Disorders Task Force factsheet: childhood interstitial lung disease

Abstract: Definition Childhood interstitial lung disease (chILD) includes a range of rare disorders of the lung parenchyma (and, sometimes, airways) that have certain clinical features in common Specific conditions within chILD include surfactant protein B or C deficiency, alveolar capillary dysplasia with misalignment of lung veins, pulmonary interstitial glycogenosis, neuroendocrine cell hyperplasia, hypersensitivity pneumonitis, eosinophilic pneumonia, lymphocytic interstitial pneumonia, alveolar proteinosis, granulomatosis with polyangiitis, sarcoidosis, lung alveolar proteinosis, and Langerhans’ cell histiocytosis chILD may complicate other disorders including inflammatory bowel disease and liver disease In many children with chILD, no specific diagnosis can be made Key messages Think about chILD if a child has chronic, unremitting tachypnoea, retractions, crackles and failure to thrive Symptoms of early chILD are very unspecific and may be missed unless there is a high level of suspicion Refer the patient to a specialist centre in paediatric pulmonology Follow your patient longitudinally in collaboration with the reference centre, and be alert to comorbidities, complications of disease or treatment, or signs of recurrent or worsening lung disease

State of the art: pulmonary hypertension

Abstract: Key points Pulmonary arterial hypertension is still a chronic and incurable disease The most common mutation found in familial pulmonary arterial hypertension is a defect in the bone morphogenetic protein receptor 2 gene Usually the final stage of the disease is right heart failure Educational aims To provide definitions and diagnostic methods for pulmonary hypertension To provide a general classification of pulmonary hypertension To provide an overview of the currently available treatments To provide a future vision of next-generation treatments

Curable hypoxia in an octogenarian with an undiagnosed inherited condition

Abstract: An 84-year-old gentleman was referred to the chest clinic with low oxygen saturations on pulse oximetry. He reported breathlessness and wheeze since his retirement as a surveyor 16 years previously. His exercise tolerance was 350 m. He had pre-existing diagnoses of chronic obstructive pulmonary disease and hypertension. He had no avian or occupational exposures, a minimal smoking history while in his teens and had not smoked since, and no history of transient ischaemic attack or cerebrovascular accident. On examination, he was comfortable with no respiratory distress but had slight peripheral cyanosis and oxygen saturations of 89% on room air. Auscultation of his chest revealed increased vocal fremitus and crackles over the right lower lobe with occasional wheeze. The patient was investigated using lung function testing, capillary blood gas sampling, a chest radiograph and thoracic computed tomography (CT). His haemoglobin at presentation was 16.1 g·dL−1. ### Lung function The results of the lung function investigations performed as shown in table 1. Supine and erect spirometry demonstrated oxygen saturations of 93% when lying down and 86% on sitting. There was no significant reversibility to 2.5 mg nebulised salbutamol. View this table: Table 1 Lung function A capillary blood gas taken on air at rest showed an oxygen tension ( P O2) of 8.0 kPa (normal 12.0–15.0 kPa), a carbon dioxide tension ( P CO2) of 4.4 kPa (normal 4.5–6.1 kPa) and pH of 7.44 (normal 7.36–7.42). ### CT CT images are shown in figure 1. Figure 1 a) Axial and b) coronal CT slice. ### Task 1 1. What is seen on the initial CT scan (fig. 1). 2. What conditions can be associated with this? 3. Can you interpret the lung function results (table 1)? ### Answer 1 1. There is a 2-cm vascular abnormality in the right lower lobe consistent with a pulmonary arteriovenous malformation (PAVM). 2. PAVMs are most commonly seen with hereditary haemorrhagic telangiectasia (>90%) and post-cardiac …

GPs Meet Rare Lung Disorders Task Force factsheet: cystic fibrosis

Abstract: Cystic fibrosis (CF) is a complex autosomal recessive disease caused by a mutation in a gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, the main function of which is to regulate liquid volume on epithelial surfaces through chloride secretion and inhibition of sodium absorption. The disease mainly affects the upper and lower airways, but also the pancreas, bowel, liver and reproductive tracts. Lung disease, the cause of death in 85% of affected subjects, begins early in life and is characterised by impaired mucociliary clearance and consequent chronic airway inflammation and bacterial infection. The prevalence of CF varies from country to country. It is most common in white populations of northern-European descent, in which it occurs in approximately 1 in 2500 births. CF is uncommon in Africa and Asia. In Europe, about one in 25 individuals carries a mutated CFTR gene but carriers do not have symptoms of the disease. CF manifests in many organs and, depending on the type of CFTR mutation and other factors, with different severity. The main symptoms of CF, which often appear in infancy and childhood, are fatty stools, poor growth and weight gain despite normal food intake, frequent chest infections, coughing and shortness of breath. In newborn babies, bowel obstruction due to meconium ileus can occur. Males can be infertile due to congenital absence of the vas deferens. The diagnosis of CF should be considered in any child or adult who presents with the signs or symptoms listed in the table. CF can be diagnosed by sweat testing. People with CF have increased amounts of sodium and chloride in their sweat (>60 mmol·L−1 diagnostic; 40–60 mmol·L−1 intermediate; <40 mmol·L−1, normal). View this table: Table 1 Main symptoms and findings of CF according to typical age of onset The test needs to be performed by trained and experienced staff. CF can also be diagnosed by genetic …

Curable hypoxia in an octogenarian with an undiagnosed inherited condition: a case commentary

Abstract: The accompanying case is an excellent illustration of why pulmonary arteriovenous malformations (PAVMs) have been considered very rare. Patients with PAVMs are generally asymptomatic and may be highly athletic, even in the presence of profound hypoxaemia due to right-to-left shunting through PAVMs. Data in papers published this year help explain why patients are so rarely symptomatic [1–3]; why some become symptomatic [1, 2]; and why, irrespective of respiratory symptoms, all PAVMs should be considered for treatment [4, 5]. PAVM development is usually complete by the end of puberty, and the octogenarian in this case would have spent decades undiagnosed with “silent” PAVMs and hypoxaemia. To understand how this could be the case, it is important to realise that, although this patient had low blood oxygen levels, his tissues were not more “hypoxic” than normal. Instead, as recently emphasised [1–3], PAVM patients use multiple mechanisms to maintain oxygen delivery. First, the relevant term for oxygen transport is not the arterial oxygen tension ( P aO2) or arterial oxygen saturation ( S aO2), but the arterial oxygen content ( C aO2) which also depends upon the concentration of haemoglobin [6]. Hypoxaemic PAVM patients generally utilise polycythaemic responses so that, irrespective of the S aO2, C aO2 on standing is preserved at just under 18 mL·dL−1 [1]. Secondly, the overall transport of oxygen to the tissues also depends on the volume of blood reaching the tissues in any given period (cardiac output) [6]. Cardiac output is the product of the heart rate and stroke volume, and both are increased in PAVM patients to compensate for hypoxaemia [2, 3]. For example, in the 165 PAVM patients reported …

A 22-year-old patient presenting with seizures and migratory pulmonary infiltrates

Abstract: A 22-year-old male patient presented with a fever for the previous 10 days, breathlessness with dry cough for the previous 3 days and decreased urine output since the morning of admission. His history was unremarkable. He denied any history of joint pain, rashes, eye symptoms, haemoptysis or sinus disease and there was no history of illicit drug abuse. He was a nonsmoker with no history of alcohol intake and was not on any regular medications. Clinical examination revealed icterus, blood pressure of 130/85 mmHg, tachycardia (130 beats·min−1), tachypnoea (30 breaths·min−1) and a temperature of 39°C. His upper airways were normal on examination and there were reduced breath sounds and crackles at the right lung base. The rest of the system examinations was unremarkable. Initial relevant investigations are summarised in table 1. The capillary blood glucose was 117 mg·dL−1. His typhoid and dengue serology along with dual malarial antigens were all-negative. An electrocardiogram showed sinus tachycardia. View this table: Table 1 Initial investigations Arterial blood gases on air showed type 1 respiratory failure with a pH of 7.39, an oxygen tension ( P O2) of 56 mmHg, a carbon dioxide tension ( P CO2) of 32 mmHg, bicarbonate 25 mmol·L−1 and lactate of 2.9 mmol·L−1. Urine dipsticks showed 1+ proteinuria. A chest radiograph was performed (fig. 1). Figure 1 Chest radiography Task 1 What does the chest radiograph show? Answer 1 The chest radiograph shows right and left lower zone patchy consolidation. A diagnosis of community-acquired pneumonia was made and he was started on high-flow oxygen at 8 L·min−1 with mask, intravenous fluids and empirical antibiotics (penicillin with macrolide) after sending blood cultures for analysis. On the second day of admission, the patient developed generalised tonic clonic seizures in the early morning and was started on intravenous phenytoin. However, seizures …

Support Healthy Lungs for Life: holding a spirometry event

Abstract: Summary The aim of this article is to introduce you to the new European Respiratory Society (ERS)/European Lung Foundation (ELF) initiative “Healthy Lungs for Life”, and to provide you with ideas and guidance on ways that you can join in and take part in this exciting campaign. The article will focus on spirometry and how running a testing event in your community can help put lung health at the forefront of public thinking.

Doing Science: Oral presentations

Abstract: In the last edition of Doing Science, we covered the creation and presentation of a poster [1], and so we shall move on to the other main way of presenting at scientific meetings: the oral presentation. Early in your career, getting your abstract accepted for an oral presentation may raise some eyebrows, your pulse and quite a few questions. After all, only 13% of the accepted abstracts were granted oral presentations at the ERS Annual Congress 2013 in Barcelona and, in 2012, the corresponding number was 14% [2]. Thus, naturally, getting accepted for an oral presentation is generally harder than getting a poster. Rather than reflecting merely a scoring system, the two formats represent two different sides of the same coin, and present different challenges as well as opportunities. This article will serve as a walk-through for first-time presenters, and will hopefully help also more seasoned speakers improve their presentations and presentation technique. After reading the last article in the Doing Science series [1], you should be familiar with the unique features of a poster. The standard oral presentation at, for example, an ERS International Congress is 10 minutes plus 5 additional minutes for questions from the audience [3]. Thus, considerably less text can (and should!) be used in an oral presentation. The trick is to know what should be in text, in figures, merely said or, maybe most importantly, completely left out. Use the on-screen/voice dynamics to your advantage. Now put yourself as presenter aside for a moment, and consider the audience [4] (after all, you are presenting to …