Paediatric Respiratory Reviews RSS feed: Current Issue. NEW 2008 IMPACT FACTOR: 0.938 Submit your article online to Paediatric Respiratory Reviews http://ees.elsevier.com/yprrv Paediatric Respiratory Reviews NEW Free Online CME Program Paediatric Respiratory Reviews offers authors the opportunity to submit their own editorials, educational reviews and short communications on topics relevant to paediatric respiratory medicine. These peer reviewed contributions will complement the commissioned reviews which will continue to form an integral part of the journal. Subjects covered include: • epidemiology • immunology and cell biology • physiology • occupational disorders • the role of allergens and pollutants A particular emphasis is given to the recommendation of "best practice" for primary care physicians and paediatricians. Paediatric Respiratory Reviews is aimed at general paediatricians but it should also be read by specialist paediatric physicians and nurses, respiratory physicians and general practitioners. It is a journal for those who are busy and do not have time to read systematically through literature, but who need to stay up to date in the field of paediatric respiration. Stay up to date and let the journal do the work for you! http://www.prrjournal.com/?rss=yesElsevier Inc.en © 2009 Elsevier Ltd. All rights reserved. Paediatric Respiratory Reviews1526-0542111March 2010 © 2009 Elsevier Ltd. All rights reserved. healthpermissions@elsevier.comhttp://www.prrjournal.com/article/PIIS1526054209000748/abstract?rss=yesThe life expectancy for subjects with progressive neuromuscular disorders has increased over the last 40 years. Whether this is the result of more aggressive use of mechanical ventilation and airway clearance devices, better detection of sleep-related breathing disorders, identifying and treating cardiac complications, creation of centres of excellence, or combinations of these factors remains a matter of discussion. Nevertheless, the early 21st century ushers in a time of great excitement and hope for patients with neuromuscular disease (NMD), as investigators not only gain a better understanding of the cellular and biochemical mechanisms that cause many of the disorders, but also potentially develop ways to circumvent production of faulty proteins so as perhaps to curtail the loss of muscle function. At the same time, clinicians have recognized the role and benefits of prompt recognition of cardiopulmonary dysfunction in patients with NMD, and when possible, of supportive intervention.Paediatric neuromuscular diseases: The future is nowHoward B. Panitch10.1016/j.prrv.2009.10.006Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Mini-symposium: Cardio-respiratory considerations12http://www.prrjournal.com/article/PIIS1526054209000736/abstract?rss=yesSummary: Subjects with progressive neuromuscular diseases undergo a typical sequence of respiratory compromise, leading from normal unassisted gas exchange to nocturnal hypoventilation with normal daytime gas exchange, and eventually to respiratory failure requiring continuous ventilatory support. Several different abnormalities in respiratory pump function have been described to explain the development of respiratory failure in subjects with neuromuscular weakness. Early in the progression of respiratory failure, the use of nocturnal assisted ventilation can reverse both night- and day-time hypercapnia. Eventually, however, diurnal hypercapnia will persist despite correction of nocturnal hypoventilation. The likely beneficial effects of mechanical ventilatory support include resting fatigue-prone respiratory muscles and resetting of the central chemoreceptors to PaCO2. Recent experience shows that select patients who require daytime ventilation can be supported with non-invasive ventilation continuously to correct gas exchange abnormalities while avoiding detrimental aspects of tracheostomy placement.Diurnal hypercapnia in patients with neuromuscular diseaseHoward B. Panitch10.1016/j.prrv.2009.10.005Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Mini-symposium: Cardio-respiratory considerations38http://www.prrjournal.com/article/PIIS1526054209000773/abstract?rss=yesSummary: Pulmonary compromise is common in neuromuscular disease. Respiratory failure may be a presenting feature of neuromuscular disease and remains a major cause of morbidity and mortality. This article will review the current understanding of the more commonly encountered neuromuscular disorders in childhood and emphasize related pulmonary issues.Overview of paediatric neuromuscular disorders and related pulmonary issues: diagnostic and therapeutic considerationsM.L. Yang, R.S. Finkel10.1016/j.prrv.2009.10.009Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Mini-symposium: Cardio-respiratory considerations917http://www.prrjournal.com/article/PIIS1526054209000700/abstract?rss=yesSummary: Paediatric neuromuscular disease compromises both the gas exchange and pump functions of the respiratory system. This can have profound implications for both growth and development of the respiratory system, as well as morbidity and mortality. Aspiration lung disease is common, and leads to increasingly restrictive pulmonary physiology over time. Abnormal lung and chest wall mechanics, and weak respiratory muscles, can combine to cause respiratory failure. Improving the balance between the work of breathing (by decreasing the respiratory load) and the respiratory pump (by improving respiratory muscle strength and decreasing respiratory muscle fatigue) can help prevent the onset of respiratory failure. Airway clearance techniques and non-invasive ventilation are two important tools in this effort. Better ways of assessing the respiratory pump, mechanical function, control and fatigue are needed especially in children.Pulmonary complications of neuromuscular disease: A Respiratory mechanics perspectiveJulian Allen10.1016/j.prrv.2009.10.002Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Mini-symposium: Cardio-respiratory considerations1823http://www.prrjournal.com/article/PIIS1526054209000712/abstract?rss=yesSummary: Sleep disordered breathing (SDB) is now well recognized in children with neuromuscular diseases (NMD) and may lead to significant morbidity and increased mortality. Predisposing factors to SDB in children with NMD include reduced ventilatory responses, reduced activity of respiratory muscles during sleep and poor lung mechanics due to the underlying neuro-muscular disorder. SDB may present long before signs of respiratory failure emerge. When untreated, SDB may contribute to significant cardiovascular morbidities, neuro-cognitive deficits and premature death. One of the problems in detecting SDB in patients with NMD is the lack of correlation between lung function testing and daytime gas exchange. Polysomnography is the preferred method to evaluate for SDB in children with NMD. When the diagnosis of SDB is confirmed, treatment by non-invasive ventilation (NIV) is usually recommended. However, other modalities of mechanical ventilation do exist and may be indicated in combination with or without other supportive measures.Sleep, sleep disordered breathing, and nocturnal hypoventilation in children with neuromuscular diseasesRaanan Arens, Hiren Muzumdar10.1016/j.prrv.2009.10.003Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Mini-symposium: Cardio-respiratory considerations2430http://www.prrjournal.com/article/PIIS152605420900075X/abstract?rss=yesSummary: Airway clearance consists of two linked processes: mucociliary clearance and cough clearance. Patients with neuromuscular weakness are at risk for impaired cough clearance and therefore the development of pneumonia and atelectasis. Aiding airway clearance in the patient with neuromuscular weakness is critical to the maintenance of health and the prevention of significant respiratory morbidity. This can be achieved using both manual and mechanical techniques. This review will discuss the physiology of cough and the mechanics of aiding cough clearance in the patient with neuromuscular weakness. In addition, technologies and techniques used to improve mucociliary clearance will also be discussed. Newer technologies such as mechanical insufflation-exsufflation have gained widespread acceptance in the management of airway clearance in the patient with neuromuscular weakness.Airway clearance modalities in neuromuscular diseaseJonathan D. Finder10.1016/j.prrv.2009.10.007Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Mini-symposium: Cardio-respiratory considerations3134http://www.prrjournal.com/article/PIIS1526054209000724/abstract?rss=yesSummary: Cardiac abnormalities occur in association with many of the neuromuscular disorders that present in childhood. Genetic defects involving the cytoskeleton, nuclear membrane, and mitochondrial function have all been described in patients with skeletal myopathy and cardiac involvement. The most common classes of neuromuscular disorders with cardiac manifestations are the muscular dystrophies- Duchenne, Becker, limb-girdle and Emery Dreifuss. Friedreich Ataxia and myotonic dystrophy also have important cardiac involvement. The type and extent of cardiac manifestations are specific to the type of neuromuscular disorder. The most common cardiac findings include dilated or hypertrophic cardiomyopathy, atrioventricular conduction defects, atrial fibrillation and ventricular arrhythmias. Screening for cardiac involvement should be performed in all children with neuromuscular disorders that have the potential for cardiac involvement. This review discusses the cardiac findings associated with specific neuromuscular disorders and outlines the indications for evaluation and treatment.Cardiac manifestations of neuromuscular disorders in childrenDaphne T. Hsu10.1016/j.prrv.2009.10.004Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Mini-symposium: Cardio-respiratory considerations3538http://www.prrjournal.com/article/PIIS1526054209000694/abstract?rss=yesSummary: Acute viral bronchiolitis remains a cause of substantial morbidity and health care costs in young infants. It is the most common lower respiratory tract condition and most common reason for admission to hospital in infants. Many respiratory viruses have been associated with acute viral bronchiolitis although respiratory syncytial virus (RSV) remains the most frequently identified virus. Most infants have a mild self limiting illness while others have more severe illness and require hospital admission and some will need ventilatory support. Differences in innate immune function in response to the respiratory viral insult as well as differences in the geometry of the airways may explain some of the variability in clinical pattern. Young age and history of prematurity remain the most important risk factors although male gender, indigenous status, exposure to tobacco smoke, poor socioeconomic factors and associated co-morbidities such as chronic lung disease and congenital heart disease increase the risks of more severe illness. Supportive therapy remains the major treatment option as no specific treatments to date have been shown to provide clinically important benefits except for inhaled hypertonic saline. Prophylaxis of high risk infants with palivizumab should be considered although the cost effectiveness is still unclear. Many questions remain regarding optimal management approaches for infants requiring hospitalisation with bronchiolitis including use of nasogastric feeding, the optimal role of supplemental oxygen, optimal use of hypertonic saline and the role of combinations of therapies, the use of heliox or modern physiotherapy approaches.Acute viral bronchiolitis in children- a very common condition with few therapeutic optionsClaire Wainwright10.1016/j.prrv.2009.10.001Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1CME Reviews3945http://www.prrjournal.com/article/PIIS1526054209000761/abstract?rss=yesSummary: Helium-oxygen (heliox) gas mixtures have been studied for over 70 years as an adjunctive therapy for airway obstruction in a variety of respiratory diseases. The medical use of heliox is based on the physical properties of helium as its low density makes it advantageous in promoting more efficient flow through narrowed passages. Clinical evidence of the efficacy of heliox in treating paediatric respiratory diseases is increasing in the medical literature. This article consists of a comprehensive review of the literature investigating the utility of heliox in the treatment of paediatric respiratory disorders, including upper and lower airway obstruction, mechanical ventilation, and aerosol delivery.The Role of Heliox in Paediatric Respiratory DiseaseMarie D. Frazier, Ira M. Cheifetz10.1016/j.prrv.2009.10.008Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1CME Reviews4653http://www.prrjournal.com/article/PIIS152605420900089X/abstract?rss=yesSummary: Lung transplantation is well-established in the treatment of end-stage lung disease in children. Our understanding of the problems associated with transplantation has increased rapidly over the past 25 years. Recent figures suggest this knowledge is starting to translate into improvements in management and survival. The common indications for lung transplantation in children, the process of assessment and the outcomes and complications of transplantation are reviewed. We discuss briefly some of the ethical issues relevant to lung transplantation and review strategies for the future. This information may help the respiratory paediatrician prepare potential candidates and their families for the process of assessment and help him or her anticipate common problems that may occur.Update on Paediatric Lung TransplantationGary M. Doherty, Paul Aurora10.1016/j.prrv.2009.11.001Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Reviews5461http://www.prrjournal.com/article/PIIS1526054209000943/abstract?rss=yesProbably most senior people can recall the teaching (or lack of it) when they were students. Most will recall being presented with opportunities to learn, but largely being left to their own devices and resources in exploiting them. Teachers varied from the charismatic to the bored (and boring), and it was really up to the students to find their own paths from entrance to the medical school to exit with the final diploma. Parts of the system worked very well – most if not all can remember time spent with brilliant bedside teachers, sharing years of priceless wisdom and experience – but many teachers fell far short of this standard.“The days that make us happy, make us wise” (John Masefield)Andrew Bush10.1016/j.prrv.2009.11.006Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Educational Strategy6263http://www.prrjournal.com/article/PIIS1526054209000918/abstract?rss=yesThe European Union (EU) is an economic and political union of 27 member states with approximately 500 million citizens. The EU has developed a single market, including free access for European medical specialists to the European job market via two relevant directives (issued in 1995 and 2005) with an automatic recognition of the diplomas and certificates of qualification in medicine in all member countries. However, within Europe, countries are in different stages of development of medical training and some countries even do not formally recognise Paediatric Respiratory Medicine (PRM) as a subspecialty. This has at least two important implications: 1) a strategy has to be developed to further strengthen PRM in Europe and 2) harmonised training standards across the entire European Union have to be ensured to make an open job market within the EU acceptable.Paediatric Respiratory Medicine – The European PerspectiveM. Gappa, on behalf of the Paediatric HERMES ERS Task Force (J. Paton, E. Baraldi, A. Bush, K-H. Carlsen, J. de Jongste, E. Eber, B. Fauroux, S. McKenzie, J-L. Noël, P. Pohunek, K. Priftis, T. Séverin, J. Wildhaber, Z. Zivkovic and M. Zach)10.1016/j.prrv.2009.11.003Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Educational Strategy6465http://www.prrjournal.com/article/PIIS1526054209000906/abstract?rss=yesPediatric pulmonology is a recognized pediatric subspecialty in the United States. Individuals are certified as Pediatric Pulmonologists by the American Board of Pediatrics, and Fellowship Training Programs are accredited by the Accreditation Council on Graduate Medical Education (ACGME). Guidelines exist for Fellowship curricula, and a body of knowledge has been assembled which the American Board of Pediatrics uses to test candidates for recognition as competent subspecialists. Fellowship training has advanced to the point where subspecialists must not only demonstrate clinical competence by the end of fellowship training, but they must also demonstrate competence in the scholarship skills of research and proficiency in teaching. The successful completion of a research project is required before a candidate can sit for the Pediatric Pulmonology Subboard Examination. Medical knowledge advances at a rapid rate, especially within subspecialties, so maintenance of certification in the subspecialty is also required. The above represent a long road to reach the current point. No doubt further improvements and advances in medical education will continue to improve the training of Pediatric Pulmonologists in the future.Postdoctoral Fellowship Training in Pediatric Pulmonology in the United States of AmericaThomas G. Keens10.1016/j.prrv.2009.11.002Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Educational Strategy6667http://www.prrjournal.com/article/PIIS1526054209000931/abstract?rss=yesThe assessment of knowledge is important in the evaluation of competence in any professional activity, especially medicine. Paediatric Pulmonology has been one of the most rapidly advancing sub-specialties of children's care, particularly in the developed world and has now reached the stage where formal examinations of competency at a tertiary level are appropriate. As in other specialties in Europe, the level of knowledge and experience to be tested has now been comprehensively set out according to a detailed syllabus relevant to clinical practice. This is delivered through a clearly defined curriculum of training.The Merits and Pitfalls of Exam Based Assessments in Clinical MedicineRobert Dinwiddie, Specialist Question Setting Group10.1016/j.prrv.2009.11.005Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Educational Strategy6869http://www.prrjournal.com/article/PIIS152605420900092X/abstract?rss=yesPaediatric Respiratory Medicine has evolved as a distinct discipline in Australia and New Zealand from the early 1970s. It now focuses on providing expertise to professional colleagues and the community in addressing the new paediatric pulmonary morbidity including asthma, cystic fibrosis, congenital abnormalities, sleep related disorders, management of the acute and long term respiratory illnesses in the extremely preterm baby and management of pulmonary complications of the immuno-compromised child. The discipline must adopt useful new technologies related to endoscopy, ventilation, lung function measurements particularly in the younger child and sleep physiology. Guidelines have been developed for the management of common paediatric pulmonary conditions such as asthma, cough, cystic fibrosis, home oxygen and bronchiolitis.Paediatric Respiratory Medicine in Australia and New ZealandLou Landau10.1016/j.prrv.2009.11.004Paediatric Respiratory Reviews 11, 1 (2010)2010-03-01Paediatric Respiratory Reviews2010-03-01111S1526-0542(10)X0002-1Educational Strategy7071