Publications
164 results found
Castellani C, Simmonds NJ, Colombo C, et al., 2021, Respiratory microbiology outcomes from an observational study of ivacaftor in people with cystic fibrosis and non-G551D gating mutations (VOCAL), European-Respiratory-Society (ERS) International Congress, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Dave K, Dunk R, Madge S, et al., 2021, Could a 2-year mortality prediction model have prevented deaths from respiratory failure: a single UK centre experience, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Simmonds N, Van Der Ent K, Colombo C, et al., 2021, Observational study of ivacaftor in people with cystic fibrosis and selected non-G551D gating mutations: final results from VOCAL, European-Respiratory-Society (ERS) International Congress, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Van Koningsbruggen-Rietschel S, Dunlevy F, Bulteel V, et al., 2021, Protecting clinical trials in cystic fibrosis during the SARS-CoV-2 pandemic: risks and mitigation measures, TRIALS, Vol: 22
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- Citations: 6
Dave K, Gerovasili V, Simmonds NJ, et al., 2021, The Changing Face of Lung Transplant Waiting Lists in the Era of CFTR Modulators, Publisher: ELSEVIER SCIENCE INC, Pages: S368-S368, ISSN: 1053-2498
Cuthbertson L, Felton I, James P, et al., 2021, The fungal airway microbiome in cystic fibrosis and non-cystic fibrosis bronchiectasis, Journal of Cystic Fibrosis, Vol: 20, Pages: 295-302, ISSN: 1569-1993
BackgroundThe prevalence of fungal disease in cystic fibrosis (CF) and non-CF bronchiectasis is increasing and the clinical spectrum is widening. Poor sensitivity and a lack of standard diagnostic criteria renders interpretation of culture results challenging. In order to develop effective management strategies, a more accurate and comprehensive understanding of the airways fungal microbiome is required. The study aimed to use DNA sequences from sputum to assess the load and diversity of fungi in adults with CF and non-CF bronchiectasis.MethodsNext generation sequencing of the ITS2 region was used to examine fungal community composition (n = 176) by disease and underlying clinical subgroups including allergic bronchopulmonary aspergillosis, chronic necrotizing pulmonary aspergillosis, non-tuberculous mycobacteria, and fungal bronchitis. Patients with no known active fungal disease were included as disease controls.ResultsITS2 sequencing greatly increased the detection of fungi from sputum. In patients with CF fungal diversity was lower, while burden was higher than those with non-CF bronchiectasis. The most common operational taxonomic unit (OTU) in patients with CF was Candida parapsilosis (20.4%), whereas in non-CF bronchiectasis sputum Candida albicans (21.8%) was most common. CF patients with overt fungal bronchitis were dominated by Aspergillus spp., Exophiala spp., Candida parapsilosis or Scedosporium spp.ConclusionThis study provides a framework to more accurately characterize the extended spectrum of fungal airways diseases in adult suppurative lung diseases.
Davies J, Dobra R, Huband K, et al., 2021, Strengthening clinical trial pharmacovigilance: simple interventions improve communication over serious adverse events, Journal of Clinical Trials
Kazani S, Rowlands DJ, Bottoli I, et al., 2021, Safety and efficacy of the cystic fibrosis transmembrane conductance regulator potentiator icenticaftor (QBW251), JOURNAL OF CYSTIC FIBROSIS, Vol: 20, Pages: 250-256, ISSN: 1569-1993
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- Citations: 8
Dave K, Dobra R, Scott S, et al., 2021, Entering the era of highly effective modulator therapies, PEDIATRIC PULMONOLOGY, Vol: 56, Pages: S79-S89, ISSN: 8755-6863
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- Citations: 16
Hughes DA, Cuthbertson L, Price H, et al., 2021, PSEUDOMONAS AERUGINOSA IMPAIRS GROWTH OF ASPERGILLUS FROM CF AIRWAY SAMPLES, Publisher: BMJ PUBLISHING GROUP, Pages: A159-A159, ISSN: 0040-6376
Simmonds NJ, 2021, Introducing the Adult Cystic Fibrosis Series An Exciting Time of Change, But New Challenges Lie Ahead, CHEST, Vol: 159, Pages: 3-4, ISSN: 0012-3692
Stanford GE, Dave K, Simmonds NJ, 2021, Pulmonary Exacerbations in Adults With Cystic Fibrosis A Grown-up Issue in a Changing Cystic Fibrosis Landscape, CHEST, Vol: 159, Pages: 93-102, ISSN: 0012-3692
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- Citations: 17
Savi D, Graziano L, Giordani B, et al., 2020, New strategies of physical activity assessment in cystic fibrosis: a pilot study, BMC PULMONARY MEDICINE, Vol: 20, ISSN: 1471-2466
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- Citations: 2
Stanford G, Davies JC, Usmani O, et al., 2020, Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial, BMJ Open Respiratory Research, Vol: 7, ISSN: 2052-4439
INTRODUCTION: Airway clearance techniques (ACTs) are a gold standard of cystic fibrosis management; however, the majority of research evidence for their efficacy is of low standard; often attributed to the lack of sensitivity from outcome measures (OMs) used historically. This randomised controlled trial (RCT) investigates these standard OMs (sputum weight, forced expiratory volume in 1 s) and new OMs (electrical impedance tomography (EIT), multiple breath washout (MBW) and impulse oscillometry (IOS)) to determine the most useful measures of ACT. METHODS AND ANALYSIS: This is a single-centre RCT with crossover design. Participants perform MBW, IOS and spirometry, and then are randomised to either rest or supervised ACT lasting 30-60 min. MBW, IOS and spirometry are repeated immediately afterwards. EIT and sputum are collected during rest/ACT. On a separate day, the OMs are performed with the other intervention. Primary endpoint is difference in change in OMs before and after ACT/rest. Sample size was calculated with 80% power and significance of 5% for each OM (target n=64). ETHICS AND DISSEMINATION: Ethics approval was gained from the London-Chelsea Research Ethics Committee (reference 16/LO/0995, project ID 154635). Dissemination will involve scientific conference presentation and publication in a peer-reviewed journal. TRIAL REGISTRATION NUMBERS: ISRCTN11220163 and NCT02721498.
van der Ent K, Simmonds NJ, Colombo C, et al., 2020, AN OBSERVATIONAL STUDY OF IVACAFTOR IN PEOPLE WITH CYSTIC FIBROSIS AND SELECTED NON-G551D GATING MUTATIONS: OUTCOMES FROM THE THIRD INTERIM ANALYSIS OF THE VOCAL STUDY, Publisher: WILEY, Pages: S220-S220, ISSN: 8755-6863
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- Citations: 1
Hughes D, Cuthbertson L, Price H, et al., 2020, <i>PSEUDOMONAS AERUGINOSA</i> IMPAIRS GROWTH OF <i>ASPERGILLUS</i> FROM CF AIRWAY SAMPLES, Publisher: WILEY, Pages: S153-S153, ISSN: 8755-6863
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- Citations: 1
Silva GF, Simmonds NJ, Roth Dalcin PDT, 2020, Clinical characteristics and outcomes in adult cystic fibrosis patients with severe lung disease in Porto Alegre, southern Brazil, BMC Pulmonary Medicine, Vol: 20, Pages: 1-8, ISSN: 1471-2466
BackgroundAdvanced lung disease in adult cystic fibrosis (CF) drives most clinical care requirements. The aim was to evaluate outcome (time to death while in the study) in a cohort of adult CF patients with severe lung disease, and to determine the association among baseline patient characteristics and outcome.MethodsA retrospective cohort study was performed and clinical records between 2000 and 2015 were reviewed. Severe lung disease was defined as forced expiratory volume in the first second (FEV1) < 30% of predicted. Outcomes of all patients, including their date of death or transplantation, were determined till January 1st, 2016. Clinical data were recorded at the entry date.ResultsAmong 39 subjects included in the study, 20 (51.3%) died, 16 (41.0%) underwent bilateral lung transplantation, and 3 were alive at the end of the study period. Two variables were independently associated with death: body mass index (BMI ≥ 18.5 kg/m2) (HR = 0.78, 95% CI = 0.64–0.96 and p = 0.017) and use of tobramycin inhalation therapy (HR = 3.82, 95% CI = 1.38–10.6 and p = 0.010). Median survival was 37 (95% CI = 16.4–57.6) months. The best cut-off point for BMI was 18.5 kg/m2. Median survival in patients with BMI < 18.5 kg/m2 was 36 months (95% CI = 18.7–53.3).ConclusionMedian survival of CF subjects with FEV1 < 30% was 37 months. BMI and tobramycin inhalation therapy were independently associated with death. Median survival in patients with BMI < 18.5 kg/m2 was significantly lower than in patients with BMI ≥ 18.5 kg/m2. The association of tobramycin inhalation with death was interpreted as confounding by severity (use was reserved for advanced lung disease).
Vijayasingam A, Frost E, Wilkins J, et al., 2020, Tablet and web-based audiometry to screen for hearing loss in adults with cystic fibrosis, Thorax, Vol: 75, Pages: 632-639, ISSN: 0040-6376
INTRODUCTION: Individuals with chronic lung disease (eg, cystic fibrosis (CF)) often receive antimicrobial therapy including aminoglycosides resulting in ototoxicity. Extended high-frequency audiometry has increased sensitivity for ototoxicity detection, but diagnostic audiometry in a sound-booth is costly, time-consuming and requires a trained audiologist. This cross-sectional study analysed tablet-based audiometry (Shoebox MD) performed by non-audiologists in an outpatient setting, alongside home web-based audiometry (3D Tune-In) to screen for hearing loss in adults with CF. METHODS: Hearing was analysed in 126 CF adults using validated questionnaires, a web self-hearing test (0.5 to 4 kHz), tablet (0.25 to 12 kHz) and sound-booth audiometry (0.25 to 12 kHz). A threshold of ≥25 dB hearing loss at ≥1 audiometric frequency was considered abnormal. Demographics and mitochondrial DNA sequencing were used to analyse risk factors, and accuracy and usability of hearing tests determined. RESULTS: Prevalence of hearing loss within any frequency band tested was 48%. Multivariate analysis showed age (OR 1.127; (95% CI: 1.07 to 1.18; p value<0.0001) per year older) and total intravenous antibiotic days over 10 years (OR 1.006; (95% CI: 1.002 to 1.010; p value=0.004) per further intravenous day) were significantly associated with increased risk of hearing loss. Tablet audiometry had good usability, was 93% sensitive, 88% specific with 94% negative predictive value to screen for hearing loss compared with web self-test audiometry and questionnaires which had poor sensitivity (17% and 13%, respectively). Intraclass correlation (ICC) of tablet versus sound-booth audiometry showed high correlation (ICC >0.9) at all frequencies ≥4 kHz. CONCLUSIONS: Adults with CF have a high prevalence of drug-related hearing loss and tablet-based audiometry can be a practical, accurate screening tool within integrated ototoxicity monitoring programmes for early detection.
Aalbers BL, Yaakov Y, Derichs N, et al., 2020, Nasal potential difference in suspected cystic fibrosis patients with 5T polymorphism, JOURNAL OF CYSTIC FIBROSIS, Vol: 19, Pages: 627-631, ISSN: 1569-1993
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- Citations: 8
Keogh RH, Tanner K, Simmonds NJ, et al., 2020, The changing demography of the cystic fibrosis population: forecasting future numbers of adults in the UK, SCIENTIFIC REPORTS, Vol: 10, Pages: 1-8, ISSN: 2045-2322
Improvements in management of cystic fibrosis (CF) through specialist centres in the UK have been associated with a step-change in life expectancy. With increasing numbers of adult patients there is a need to review health care provision to ensure it is sufficient to meet future needs. We used UK CF Registry data to project the number of patients aged 16–17 and 18 and older up to 2030, and numbers therefore requiring specialist adult CF care. Survival modelling was used to estimate age-specific mortality rates. New-diagnosis rates were estimated using diagnoses observed in the Registry and national population figures. Uncertainty in projections was captured through 95% prediction intervals (PI). The number of adults (aged 18 and older) is expected to increase by 28% from 6,225 in 2017 to 7,988 in 2030 (95% PI 7,803–8,169), assuming current mortality rates. If mortality rates improve at the rate seen over recent years, the projected number increases to 8,579 (95% PI 8,386–8,764). The age distribution is also expected to change, with 36% of CF adults being over 40 in 2030, versus 21% in 2017. There is an urgent requirement to review adult CF health care provision, due to both increasing numbers and the changing care needs of an older population.
Morris-Rosendahl DJ, Edwards M, McDonnell MJ, et al., 2020, Whole-gene sequencing of CFTR reveals a high prevalence of the intronic variant c.3874-4522A>G in cystic fibrosis., American Journal of Respiratory and Critical Care Medicine, Vol: 201, Pages: 1438-1441, ISSN: 1073-449X
Cosgriff R, Ahern S, Bell SC, et al., 2020, A multinational report to characterise SARS-CoV-2 infection in people with cystic fibrosis., J Cyst Fibros, Vol: 19, Pages: 355-358
UNLABELLED: Information is lacking on the clinical impact of the novel coronavirus, SARS-CoV-2, on people with cystic fibrosis (CF). Our aim was to characterise SARS-CoV-2 infection in people with cystic fibrosis. METHODS: Anonymised data submitted by each participating country to their National CF Registry was reported using a standardised template, then collated and summarised. RESULTS: 40 cases have been reported across 8 countries. Of the 40 cases, 31 (78%) were symptomatic for SARS-CoV-2 at presentation, with 24 (60%) having a fever. 70% have recovered, 30% remain unresolved at time of reporting, and no deaths have been submitted. CONCLUSIONS: This early report shows good recovery from SARS-CoV-2 in this heterogeneous CF cohort. The disease course does not seem to differ from the general population, but the current numbers are too small to draw firm conclusions and people with CF should continue to strictly follow public health advice to protect themselves from infection.
Simmonds NJ, Castellani C, Colombo C, et al., 2019, AN OBSERVATIONAL STUDY OF IVACAFTOR IN PATIENTS WITH CYSTIC FIBROSIS (CF) AND SELECTED NON-G551D GATING MUTATIONS: OUTCOMES FROM THE SECOND INTERIM ANALYSIS OF THE VOCAL STUDY, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A11-A11, ISSN: 0040-6376
Brown L, Kent BD, Heijerman HGM, et al., 2019, Editorial introductions, Current Opinion in Pulmonary Medicine, Vol: 25, Pages: V-VI, ISSN: 1070-5287
Waller MD, Harman K, Bayfield KJ, et al., 2019, OPPORTUNISTIC ASSESSMENT OF UPPER AND LOWER AIRWAY ELECTROPHYSIOLOGY AND LUNG FUNCTION IN CYSTIC FIBROSIS, North American Cystic Fibrosis Conference, Publisher: WILEY, Pages: S163-S163, ISSN: 8755-6863
Archangelidi O, Abbott J, Bryon M, et al., 2019, QUALITY OF LIFE IN PATIENTS WITH CF USING THREE ONLINE RESEARCH QUESTIONNAIRES: A FEASIBILITY STUDY, Publisher: WILEY, Pages: S419-S419, ISSN: 8755-6863
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- Citations: 1
Savi D, Graziano L, Giordani B, et al., 2019, DIFFERENT METHODS OF MEASURING PHYSICAL ACTIVITY IN CYSTIC FIBROSIS: NEW ELECTRONIC DEVICES, Publisher: WILEY, Pages: S388-S388, ISSN: 8755-6863
Savi D, Graziano L, Schiavetto S, et al., 2019, Different methods of measuring physical activity in cystic fibrosis: accelerometer versus new electronic devices, International Congress of the European-Respiratory-Society (ERS), Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Davies JC, Scott S, Dobra R, et al., 2019, Fair selection of participants in clinical trials: The challenge to push the envelope further, Journal of Cystic Fibrosis, Vol: 18, Pages: e48-e50, ISSN: 1569-1993
Dobra R, Scott S, Davies JC, et al., 2019, Who and why; sharing our experiences of developing a standard operating procedure (SOP) to allocate screening slots for highly competitive cystic fibrosis trials, Journal of Cystic Fibrosis, Vol: 18, Pages: e45-e46, ISSN: 1569-1993
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