770 results found
Andersson CK, Adams A, Nagakumar P, et al., 2017, Intraepithelial neutrophils in pediatric severe asthma are associated with better lung function, JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, Vol: 139, Pages: 1819-+, ISSN: 0091-6749
Arigliani M, Raywood E, Verger N, et al., 2017, Shortened Multiple Breath Washout Is Not Suitable for Scond Calculation in Patients with Cystic Fibrosis., PEDIATRIC PULMONOLOGY, Vol: 52, Pages: S154-S154, ISSN: 8755-6863
Artiso L, Walker S, Fleming L, et al., 2017, Sputum Eosinophil Peroxidase (EPX) Differentiates Pediatric Severe Therapy Resistant Asthma (STRA) from Difficult Asthma (DA)., PEDIATRIC PULMONOLOGY, Vol: 52, Pages: S105-S106, ISSN: 8755-6863
Brugha R, Hall A, Bush A, 2017, It ain't necessarily so: a surprising lower airway infection, THORAX, Vol: 72, Pages: 96-97, ISSN: 0040-6376
Bush A, 2017, Markers of Severity in Difficult-To-Treat Asthma, PEDIATRIC PULMONOLOGY, Vol: 52, Pages: S36-S37, ISSN: 8755-6863
Bush A, 2017, Diffuse lung disease in children: transcending continental boundaries, INTERNATIONAL JOURNAL OF TUBERCULOSIS AND LUNG DISEASE, Vol: 21, Pages: 837-839, ISSN: 1027-3719
Bush A, Custovic A, 2017, Formula one: best is no formula, EUROPEAN RESPIRATORY JOURNAL, Vol: 49, ISSN: 0903-1936
Bush A, Fleming L, Saglani S, 2017, Severe asthma in children, RESPIROLOGY, Vol: 22, Pages: 886-897, ISSN: 1323-7799
Cook J, Beresford F, Fainardi V, et al., 2017, Managing the pediatric patient with refractory asthma: a multidisciplinary approach, JOURNAL OF ASTHMA AND ALLERGY, Vol: 10, Pages: 123-130, ISSN: 1178-6965
De Simoni A, Horne R, Fleming L, et al., 2017, What do adolescents with asthma really think about adherence to inhalers? Insights from a qualitative analysis of a UK online forum, BMJ OPEN, Vol: 7, ISSN: 2044-6055
Halvorsen T, Walsted ES, Bucca C, et al., 2017, Inducible laryngeal obstruction: an official joint European Respiratory Society and European Laryngological Society statement., Eur Respir J, Vol: 50
Inducible laryngeal obstruction (ILO) describes an inappropriate, transient, reversible narrowing of the larynx in response to external triggers. ILO is an important cause of a variety of respiratory symptoms and can mimic asthma. Current understanding of ILO has been hampered by imprecise nomenclature and variable approaches to assessment and management. A task force of the European Respiratory Society (ERS) and European Laryngological Society (ELS) was thus set up to address this, and to identify research priorities.A literature search identified relevant articles published until June 2016, using all identifiable terms for ILO, although including only articles using laryngoscopy. In total, 172 out of 252 articles met the inclusion criteria, summarised in sections on diagnostic approach, aetiology, comorbidities, epidemiology and treatment. The consensus taxonomy published by ERS, ELS and the American College of Chest Physicians (ACCP) in 2015 is used throughout this statement.We highlight the high prevalence of ILO and the clinical impact for those affected. Despite recent advances, most aspects of this condition unfortunately remain incompletely understood, precluding firm guidance. Specifically, validated diagnostic and treatment algorithms are yet to be established, and no randomised control studies were identified in this search; hence we also make recommendations for future research.
Hashimoto S, Brinkman P, Lefaudeux D, et al., 2017, Clinical Phenotypes Of Asthma In Childhood And Adolescence: Clustering Analysis From The Paediatric U-Biopred Cohorts, International Conference of the American-Thoracic-Society (ATS), Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Irving S, Carr S, Hogg C, et al., 2017, Lung Clearance Index (LCI) is Stable in Most Primary Ciliary Dyskinesia (PCD) Patients Managed in a Specialist Centre: a Pilot Study, LUNG, Vol: 195, Pages: 441-443, ISSN: 0341-2040
Lucas JS, Barbato A, Collins SA, et al., 2017, European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia, EUROPEAN RESPIRATORY JOURNAL, Vol: 49, ISSN: 0903-1936
McKibben S, Bush A, Thomas M, et al., 2017, The use of electronic alerts in primary care computer systems to identify the over-prescription of short-acting beta(2)-agonists in people with asthma: a protocol for a systematic review, NPJ PRIMARY CARE RESPIRATORY MEDICINE, Vol: 27, ISSN: 2055-1010
Nagakumar P, Artusio L, Fainardi V, et al., 2017, Role Of Airway Ilc2 And Ilc3 Compared To Th2 And Th17 Cells In Paediatric Severe Therapy Resistant Asthma (stra), International Conference of the American-Thoracic-Society (ATS), Publisher: AMER THORACIC SOC, ISSN: 1073-449X
Olcese C, Patel MP, Shoemark A, et al., 2017, X-linked primary ciliary dyskinesia due to mutations in the cytoplasmic axonemal dynein assembly factor PIH1D3, NATURE COMMUNICATIONS, Vol: 8, ISSN: 2041-1723
Pifferi M, Bush A, Caramella D, et al., 2017, Matrix metalloproteinases and airway remodeling and function in primary ciliary dyskinesia, RESPIRATORY MEDICINE, Vol: 124, Pages: 49-56, ISSN: 0954-6111
Puranik S, Forno E, Bush A, et al., 2017, Predicting Severe Asthma Exacerbations in Children, AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, Vol: 195, Pages: 854-859, ISSN: 1073-449X
Saglani S, Bush A, 2017, Asthma Attacks in Children: Does Blocking IgE Reduce Rhinoviral Infections?, Am J Respir Crit Care Med
Saunders C, Bayfield K, Irving S, et al., 2017, Developments in multiple breath washout testing in children with cystic fibrosis, CURRENT MEDICAL RESEARCH AND OPINION, Vol: 33, Pages: 613-620, ISSN: 0300-7995
Shoemark A, Frost E, Dixon M, et al., 2017, Accuracy of Immunofluorescence in the Diagnosis of Primary Ciliary Dyskinesia, AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, Vol: 196, Pages: 94-101, ISSN: 1073-449X
Shoemark A, Moya E, Hirst RA, et al., 2017, High prevalence of CCDC103 p.His154Pro mutation causing primary ciliary dyskinesia disrupts protein oligomerisation and is associated with normal diagnostic investigations., Thorax
RATIONALE: Primary ciliary dyskinesia is a genetically heterogeneous inherited condition characterised by progressive lung disease arising from abnormal cilia function. Approximately half of patients have situs inversus. The estimated prevalence of primary ciliary dyskinesia in the UK South Asian population is 1:2265. Early, accurate diagnosis is key to implementing appropriate management but clinical diagnostic tests can be equivocal. OBJECTIVES: To determine the importance of genetic screening for primary ciliary dyskinesia in a UK South Asian population with a typical clinical phenotype, where standard testing is inconclusive. METHODS: Next-generation sequencing was used to screen 86 South Asian patients who had a clinical history consistent with primary ciliary dyskinesia. The effect of a CCDC103 p.His154Pro missense variant compared with other dynein arm-associated gene mutations on diagnostic/phenotypic variability was tested. CCDC103 p.His154Pro variant pathogenicity was assessed by oligomerisation assay. RESULTS: Sixteen of 86 (19%) patients carried a homozygous CCDC103 p.His154Pro mutation which was found to disrupt protein oligomerisation. Variable diagnostic test results were obtained including normal nasal nitric oxide levels, normal ciliary beat pattern and frequency and a spectrum of partial and normal dynein arm retention. Fifteen (94%) patients or their sibling(s) had situs inversus suggesting CCDC103 p.His154Pro patients without situs inversus are missed. CONCLUSIONS: The CCDC103 p.His154Pro mutation is more prevalent than previously thought in the South Asian community and causes primary ciliary dyskinesia that can be difficult to diagnose using pathology-based clinical tests. Genetic testing is critical when there is a strong clinical phenotype with inconclusive standard diagnostic tests.
Smith WD, Bardin E, Cameron L, et al., 2017, Current and future therapies for Pseudomonas aeruginosa infection in patients with cystic fibrosis., FEMS Microbiol Lett, Vol: 364
Pseudomonas aeruginosa opportunistically infects the airways of patients with cystic fibrosis and causes significant morbidity and mortality. Initial infection can often be eradicated though requires prompt detection and adequate treatment. Intermittent and then chronic infection occurs in the majority of patients. Better detection of P. aeruginosa infection using biomarkers may enable more successful eradication before chronic infection is established. In chronic infection P. aeruginosa adapts to avoid immune clearance and resist antibiotics via efflux pumps, β-lactamase expression, reduced porins and switching to a biofilm lifestyle. The optimal treatment strategies for P. aeruginosa infection are still being established, and new antibiotic formulations such as liposomal amikacin, fosfomycin in combination with tobramycin and inhaled levofloxacin are being explored. Novel agents such as the alginate oligosaccharide OligoG, cysteamine, bacteriophage, nitric oxide, garlic oil and gallium may be useful as anti-pseudomonal strategies, and immunotherapy to prevent infection may have a role in the future. New treatments that target the primary defect in cystic fibrosis, recently licensed for use, have been associated with a fall in P. aeruginosa infection prevalence. Understanding the mechanisms for this could add further strategies for treating P. aeruginosa in future.
Zdrenghea MT, Makrinioti H, Bagacean C, et al., 2017, Vitamin D modulation of innate immune responses to respiratory viral infections, REVIEWS IN MEDICAL VIROLOGY, Vol: 27, ISSN: 1052-9276
de Benedictis D, Bush A, 2017, Asthma in Adolescence: Is There Any News?, PEDIATRIC PULMONOLOGY, Vol: 52, Pages: 129-138, ISSN: 8755-6863
de Benedictis FM, Bush A, 2017, Infantile wheeze: rethinking dogma, ARCHIVES OF DISEASE IN CHILDHOOD, Vol: 102, Pages: 371-375, ISSN: 0003-9888
de Benedictis FM, Bush A, 2017, Respiratory manifestations of gastro-oesophageal reflux in children., Arch Dis Child
Gastro-oesophageal reflux disease (GORD) is a complex problem in children. Suspected respiratory manifestations of GORD, such as asthma, chronic cough and laryngitis, are commonly encountered in the paediatric practice, but continue to be entities with more questions than answers. The accuracy of diagnostic tests (ie, pH or pH-impedance monitoring, laryngoscopy, endoscopy) for patients with suspected extraoesophageal manifestations of GORD is suboptimal and therefore whether there is a causal relationship between these conditions remains largely undetermined. An empiric trial of proton pump inhibitors can help individual children with undiagnosed respiratory symptoms and suspicion of GORD, but the response to therapy is unpredictable, and in any case what may be being observed is spontaneous improvement. Furthermore, the safety of these agents has been called into question. Poor response to antireflux therapy is an important trigger to search for non-gastro-oesophageal reflux causes for patients' symptoms. Evidence for the assessment of children with suspected extraoesophageal manifestations of GORD is scanty and longitudinal studies with long-term follow-up are urgently required.
Bardin E, Bolt F, Cameron S, et al., 2016, METABOLOMIC CHARACTERIZATION OF PSEUDOMONAS AERUGINOSA ISOLATES FROM CYSTIC FIBROSIS PATIENTS USING RAPID EVAPORATIVE IONISATION MASS SPECTROMETRY, PEDIATRIC PULMONOLOGY, Vol: 51, Pages: 312-313, ISSN: 8755-6863
Bossley CJ, Fleming L, Ullmann N, et al., 2016, Assessment of corticosteroid response in pediatric patients with severe asthma by using a multidomain approach, JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, Vol: 138, Pages: 413-+, ISSN: 0091-6749
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