A Bush

..The authors consider the clinical situations in which ciliary disease should be considered, and the implications for specialist respiratory practice...

..Exhaled breath condensate (EBC) is a non-invasive method to assess airway inflammation and oxidative stress and may be useful in the assessment of childhood asthma...

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..Asthma is not one disease but many, and attention to delineating clinical phenotypes, rather than being obsessed with guidelines based on large heterogeneous groups, may allow rational individual treatment...

..New iatrogenic complications are also likely to be discovered. The procedure is very safe if performed by experienced operators with back-up from doctors skilled in airway management and the monitoring of sick children...

..It is hoped that, in the absence of good-quality evidence for most clinical scenarios, the experience-based approach described in this article may prove a useful guide to the clinician...

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..Different families will make different decisions about postnatal surgery in an asymptomatic baby. If surgery is performed, morbidity is low, particularly with a video-assisted thoracoscopic (VATS) procedure...

..Any model of COPD which does not take into account early life influences is likely to be fatally flawed...

..Prophylactic therapy on a long term basis with inhaled steroids in pre-school children does not reduce the likelihood of progression to asthma in mid-childhood, and the results of treatment in terms of symptoms are disappointing...

..This is particularly urgent to select groups of children for new, phenotype-specific therapies, and monitor their effects...

..I shall also assume that change is an ongoing process, so I shall include change in progress, provided it is supported by published literature...

..Here we offer a personal view, as a possible template for managing a vexed and emotional problem. The complex non-respiratory aspects of the holistic care of these infants will not be discussed...

..Finally, the ethical principles that must underpin future research in pre-school children is discussed, as well as the means we might use to further our understanding of the relevant early disease processes...

..Treatment is not evidence based and recommendations are largely extrapolated from cystic fibrosis and other suppurative lung diseases...

..Retrospective recall of childhood events is of limited value. In conclusion, a full understanding the multifaceted phenotypes of asthma requires a thorough knowledge of early life events and their consequences over many decades...

..Patients should be followed up by specialists familiar with the different ways of managing the upper and lower airway complications...

..In addition, the optimal treatment of patients at first isolation and early colonisation needs to be researched, including choice of antibiotic(s) and route, dosage and duration of antibiotic therapy...

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..It is important however, not to over-diagnose asthma in children who in fact have a chronic non-specific cough. Such children require no treatment, get better with time, and have normal long-term lung function...

..Thus, a complete reappraisal of the nomenclature of congenital lung disease is timely; this review does not discuss treatment options...

..The new UK initiative, 'Medicines for Children', should go a long way towards addressing the problem, and increase the evidence base for the utilization of medications in pediatric practice...

..However, despite the interest in innovative approaches, getting the basics right in children with apparently severe asthma will remain the foundation of management for the foreseeable future...

..Hypertonic saline improved lung function to the same degree as rhDNase in short-term studies. We compared the effectiveness of daily rhDNase, hypertonic saline, and alternate-day rhDNase in children with cystic fibrosis...

..Computed tomography scans do not contribute towards identifying the aetiology and, most importantly, a specific aetiological diagnosis frequently leads to a change in management...

..This hypothesis-generating work should form the basis of future interventional studies...

..Studies to explore this mechanism in the distal, more sparsely-ciliated airways could aid an understanding of pathogenesis and the development of new treatments...

..The place of these markers remains unclear and their use in clinical practice needs further investigation by long-term longitudinal studies...

..This is the first therapeutic trial of inhaled mannitol in children with CF; it was compared with recombinant human deoxyribonuclease (rhDNase), the current best established mucolytic treatment...

..93, 95% CI 0.90-0.97). Only 11% of DA children exhibited complete corticosteroid responsiveness. The rarity of complete corticosteroid responsiveness suggests alternative therapies are needed for children with DA...

..Studies of such samples would allow us to determine the early pathological changes in the CF airway and possibly find new treatments to prevent the progression to bronchiectasis and end stage airway destruction...

..Both subgroups included patients with persistent symptoms, which suggests that different patterns of difficult asthma in children exist...

..We propose that FE(NO) is associated with eosinophilic inflammation in children with difficult asthma, following prednisolone, and may help in identifying patients in whom persistent symptoms are associated with airway eosinophilia...

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..The mechanism is uncertain, and could relate either to systemic or local effects. The aim of this study was to determine, in a large cohort of children, whether MBL is present on the airway surface in health or disease...

..The procedure is acceptable to the families involved...

..A similar relationship in children with difficult asthma (DA), in whom RBM thickening is a known feature, may allow the use of HRCT as a non-invasive marker of airway remodelling. We evaluated this relationship in children with DA...

..In an attempt to reduce the use of regular oral corticosteroids, we treated a group of such children with monthly courses of intravenous immunoglobulin (IVIG)...

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..The 15c did not discriminate between a maximal and a submaximal test, and was less useful than VAS. Important information may be missed by the step test which is detected by more complex exercise tests...

..The benefits and potential side effects need to be determined before routine use can be advised...

..Cough may be a more sensitive marker of airway inflammation than simple spirometry...

..001). CONCLUSION: RBM thickening and the eosinophilic inflammation characteristic of asthma in older children and adults are not present in symptomatic infants with reversible airflow obstruction, even in the presence of atopy...

..38; confidence interval, 1.06-1.81) were significant predictors of failed reduction. These findings suggest that monitoring airway inflammation may be useful in optimizing treatment in children with asthma...

..VS at presentation gives prognostic information, which may be of use in early intervention studies. Whether using VS to guide treatment improves long-term prognosis requires a larger prospective trial...

..To evaluate and identify potentially modifiable factors in children with problematic asthma by a nurse-led assessment and home visit...

..There is currently no gold standard test; both skin prick testing (SPT) and allergen-specific IgE (sIgE) are used. Furthermore, atopy is increasingly considered to be a spectrum, not an all-or-none phenomenon...

..A positive cough swab is a strong predictor of sputum culture. However, a negative cough swab does not rule out infection. Persistent symptoms should be further investigated...

..Accurate characterisation of subjects is essential to interpret data from studies investigating preschool wheezing...

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..To determine whether similarities exist in both the imaging and histopathological features of congenital cystic lung lesions and whether a more appropriate classification would be to adopt the theory of "malinosculation"...

..This study reports on the results of measurements over 5 years...

..Little is known about disease progression through childhood and the effect on growth and spirometry. This study reviews longitudinal lung function and growth in children with non-CF bronchiectasis...

..It is important that patients continue to be referred early to tertiary CF centres...

..The variability with time in isolation of MR strains from individuals demonstrates the potential difficulties in designing segregation policies based on antibiotic sensitivity patterns...

..It may be clinically more useful to compare exhaled NO values with a subject's previous values than to compare them with a population based normal range...

..Normal values are provided for all haemodynamic parameters for rest and every exercise stage for all subgroups of children. This should allow accurate comparison of normal and disease groups in future...

..There is an urgent need for better treatment of preschool wheeze...

..We prospectively assessed how the quality of biopsy specimens obtained from children with CF compare with those from children with other airway diseases...

..Pulmonary function in preschool wheezing phenotypes based on wheeze onset and duration and atopic status has been extensively described but has not been studied in symptom-pattern phenotypes of episodic (viral) and multiple-trigger wheeze...

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..The pH of exhaled breath condensate (EBC) is a promising marker. Although pH has been measured in the EBC of adults with inflammatory airway diseases, no study has measured this in children...

..Multi-centre studies are required to test the utility of this approach...

..49) versus 1.18 (0.31-2.6)). The ratio of fibril to matrix in the thickened reticular basement membrane of asthmatics is normal, and, contrary to what is expected in fibrosis, the fibrils do not resemble those of interstitial collagen...

..Failure of medical therapy necessitated surgical reconstruction of her right pulmonary artery. Postoperatively she has normal perfusion of the right lung and normal lung function...

..HRCT is also more specific than chest radiography for categorizing airway, airspace or interstitial processes in children. The current review will concentrate on the value of CT in airway diseases and diffuse interstitial lung disease...

..However, nNO in particular is a quick and useful diagnostic marker which may be used to screen patients with a clinical suspicion of PCD...

..The distal airway is a major site of inflammation in CF, and measuring alveolar NO may be a marker of distal inflammation in this disease...

..Importantly, all abnormalities developed in parallel, not sequentially, between 2 and 3 weeks of age...

..4%. CONCLUSIONS: There was moderate agreement between both Fe(NO) and sputum eosinophils and BAL eosinophils. There was good NPV, but only poor PPV for these markers for mucosal eosinophilia...

..These initial findings support the need for further controlled studies to evaluate the use of CSIT in severe childhood asthma...

..Raised nitric oxide is only poorly predictive of sputum eosinophilia in these children...

..We also showed an influence of polymorphisms at both aa16 and aa27 on neonatal lung function. Wheezing beyond 4 years, typical of classical asthma, was unrelated to early measurements of lung function or bronchial responsiveness...

..In infants with CF, the raised volume technique identified diminished airway function more frequently than the tidal technique...

..5-39.9) vs. 1.4 (0.0-4.8) cells/mm2, P = 0.018). In conclusion, a 2-week course of prednisolone is not necessarily predictive of "target" lung function. Definitions such as PAL should be regularly reviewed on individual basis...

..To date there have been no studies exclusively in children with CF examining the effect of dry powder mannitol on the airways. The aim of this study was to determine acute tolerability of inhaled mannitol in children with CF...

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..05). There was an inverse correlation between exhaled cys-LTs and LTB4 in patients with mild persistent asthma. We conclude that exhaled cys-LTs and LTB4 may be noninvasive markers of airway inflammation in pediatric asthma...

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..Structural alterations to airway smooth muscle (ASM) are a feature of asthma and cystic fibrosis (CF) in adults...

..Genetic risk factors may be useful in identifying subtypes of asthma and determining whether intermediate phenotypes, such as elevation of the total serum IgE level, are causally linked to disease...

..This paper further considers the long-term implications of these studies for both future research and clinical practice...

..25 [0-91]%) or the "non-eosinophilic" group (27.5 [0-96] vs. 44 [9-96]%). In conclusion children with difficult asthma may benefit clinically from high-dose systemic corticosteroids even in the absence of sputum eosinophilia...

..This differs from a previous report. Furthermore, we speculate that these data may provide supportive evidence that variable flow NO measurements can assess the relative activity of iNOS and eNOS...

..The inhalation maneuver does not influence eNO measurements using the variable expiratory flow technique...

..Airway inflammation is characteristic of asthma. Distal inflammation may be particularly important...

..Pulmonary function did not change at any time following HFCC. Compared with ACBT, HFCC by Hayek Cuirass is not an effective airway clearance treatment modality for children with CF during an infective exacerbation...

..There are limited longitudinal data about respiratory morbidity and lung function after preterm birth into adulthood...

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..Lavage culture was positive in eight of 18 children without respiratory symptoms. This highlights the potential of bronchoscopy following diagnosis, even in asymptomatic children...