Publications
164 results found
Simmonds NJ, Thomson AH, 2016, Aminoglycosides: old friend ... new foe?, JOURNAL OF CYSTIC FIBROSIS, Vol: 15, Pages: 411-412, ISSN: 1569-1993
Hippolyte S, Pabary R, Waller M, et al., 2016, Clinical Trials of Novel Treatments for Cystic Fibrosis, AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, Vol: 193, Pages: 569-571, ISSN: 1073-449X
Burgess JC, Bridges N, Winston B, et al., 2016, HbA1c as a screening tool for cystic fibrosis related diabetes Response, JOURNAL OF CYSTIC FIBROSIS, Vol: 15, Pages: 265-266, ISSN: 1569-1993
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- Citations: 2
Burgess JC, Bridges N, Banya W, et al., 2016, HbA1c as a screening tool for cystic fibrosis related diabetes, JOURNAL OF CYSTIC FIBROSIS, Vol: 15, Pages: 251-257, ISSN: 1569-1993
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- Citations: 45
Savi D, Simmonds N, Di Paolo M, et al., 2015, Relationship between pulmonary exacerbations and daily physical activity in adults with cystic fibrosis, BMC Pulmonary Medicine, Vol: 15, ISSN: 1471-2466
Background: The aim of this study was to examine the relationship between pulmonary exacerbations andphysical activity (PA) in adults with cystic fibrosis (CF).Methods: We grouped adults with CF according to their exacerbation status in the year before study enrolment:(1) <1 exacerbation/year; (2) 1–2 exacerbations/year; and (3) >2 exacerbations/year. PA was assessed objectively bymeans of an accelerometer at the time of study enrolment.Results: Patients with >2 exacerbations/year spent less time in PA; specifically, fewer activities of mild intensity [>3metabolic equivalents (METs)], and lower active energy expenditure (P = 0.01 and P = 0.03, respectively). Aftercorrecting for relevant confounders, PA levels were not related to the exacerbation frequency in the preceding year.PA at moderate intensity (4.8–7.2 METs) or greater (>7.2 METs) was independently associated with gender and FEV1% predicted (P = 0.007 and P = 0.04, respectively). Compared with men, women had reduced vigorous activities(P = 0.01) and active energy expenditure (P = 0.01).Conclusions: Adult CF patients with more pulmonary exacerbations in the preceding year have more advanceddisease and are less active than their peers. PA was independently associated with gender and airflow obstruction.Gender differences in PA are evident in CF adults
Hippolyte S, Keogh R, MacNeill S, et al., 2015, THE FEMALE DISADVANTAGE IN UK CF REGISTRY DATA 2008-2013, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A219-A219, ISSN: 0040-6376
Geake JB, Reid DW, Currie BJ, et al., 2015, An international, multicentre evaluation and description of Burkholderia pseudomallei infection in cystic fibrosis, BMC Pulmonary Medicine, Vol: 15, ISSN: 1471-2466
Gill DR, Alton EW, Armstrong DK, et al., 2015, A Phase 2b clinical trial of non-viral gene therapy in Cystic Fibrosis patients: randomized, double-blind, placebo-controlled repeated aerosol delivery to the lungs., Collaborative Congress of the European-Society-of-Gene-and-Cell-Therapy (ESGCT) and Finnish-Society-of-Gene-Therapy (FSGT), Publisher: MARY ANN LIEBERT, INC, Pages: A18-A18, ISSN: 1043-0342
Simmonds NJ, 2015, Cystic Fibrosis Papers of the Year, 2013-2014, PAEDIATRIC RESPIRATORY REVIEWS, Vol: 16, Pages: 9-11, ISSN: 1526-0542
Barry PJ, Plant BJ, Simmonds NJ, et al., 2015, IVACAFTOR DECREASES MORTALITY IN G551D PATIENTS WITH SEVERE LUNG DISEASE, PEDIATRIC PULMONOLOGY, Vol: 50, Pages: 275-276, ISSN: 8755-6863
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- Citations: 3
Brown LK, Williams A, Yankaskas J, et al., 2015, Editorial introductions, Current Opinion in Pulmonary Medicine, Vol: 21, ISSN: 1070-5287
Alton EWFW, Armstrong DK, Ashby D, 2015, Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial, The Lancet Respiratory Medicine, Vol: 3, Pages: 684-691, ISSN: 2213-2600
BackgroundLung delivery of plasmid DNA encoding the CFTR gene complexed with a cationic liposome is a potential treatment option for patients with cystic fibrosis. We aimed to assess the efficacy of non-viral CFTR gene therapy in patients with cystic fibrosis.MethodsWe did this randomised, double-blind, placebo-controlled, phase 2b trial in two cystic fibrosis centres with patients recruited from 18 sites in the UK. Patients (aged ≥12 years) with a forced expiratory volume in 1 s (FEV1) of 50–90% predicted and any combination of CFTR mutations, were randomly assigned, via a computer-based randomisation system, to receive 5 mL of either nebulised pGM169/GL67A gene–liposome complex or 0·9% saline (placebo) every 28 days (plus or minus 5 days) for 1 year. Randomisation was stratified by % predicted FEV1 (<70 vs ≥70%), age (<18 vs ≥18 years), inclusion in the mechanistic substudy, and dosing site (London or Edinburgh). Participants and investigators were masked to treatment allocation. The primary endpoint was the relative change in % predicted FEV1. The primary analysis was per protocol. This trial is registered with ClinicalTrials.gov, number NCT01621867.FindingsBetween June 12, 2012, and June 24, 2013, we randomly assigned 140 patients to receive placebo (n=62) or pGM169/GL67A (n=78), of whom 116 (83%) patients comprised the per-protocol population. We noted a significant, albeit modest, treatment effect in the pGM169/GL67A group versus placebo at 12 months' follow-up (3·7%, 95% CI 0·1–7·3; p=0·046). This outcome was associated with a stabilisation of lung function in the pGM169/GL67A group compared with a decline in the placebo group. We recorded no significant difference in treatment-attributable adverse events between groups.InterpretationMonthly application of the pGM169/GL67A gene therapy formulation was associated with a significant, albeit modest, benefit in FEV1 compared with placebo at 1 yea
Ali H, Orchard C, Mariveles M, et al., 2015, Effective strategies for managing new <i>Pseudomonas</i> cultures in adults with cystic fibrosis, EUROPEAN RESPIRATORY JOURNAL, Vol: 46, Pages: 862-865, ISSN: 0903-1936
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- Citations: 4
Alton EWFW, Armstrong DK, Ashby D, 2015, Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fi brosis: a randomised, double-blind, placebo-controlled, phase 2b trial (vol 3, pg 684, 2015), LANCET RESPIRATORY MEDICINE, Vol: 3, Pages: E33-E33, ISSN: 2213-2600
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- Citations: 2
Alton E, Griesenbach U, Davies JC, et al., 2015, A randomised, double-blind, placebo-controlled trial of repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis, Lancet Respiratory Medicine, ISSN: 2213-2619
Westaby J, Butt MA, Shahid S, et al., 2015, GENOTYPE-PHENOTYPE CORRELATIONS IN CYSTIC FIBROSIS PATIENTS WITH PANCREATITIS HIGHLIGHTS AN INCREASED LIKELIHOOD OF PANCREATIC INSUFFICIENCY AND IDENTIFIES UNIQUE MUTATION PAIRS THAT POTENTIALLY PREDISPOSE TO AND PROTECT FROM PANCREATITIS, 2nd Digestive-Disorders-Federation Conference, Publisher: BMJ PUBLISHING GROUP, Pages: A305-A305, ISSN: 0017-5749
Savi D, Di Paolo M, Simmonds N, et al., 2015, Relationship between daily physical activity and aerobic fitness in adults with cystic fibrosis, BMC Pulmonary Medicine, Vol: 15, ISSN: 1471-2466
Jamali A, Harrison M, Bilton D, et al., 2015, Use of aprepitant as an antiemetic in cystic fibrosis patients., BMJ Support Palliat Care, Vol: 5, Pages: 118-119
INTRODUCTION: Aprepitant is a NK1 receptor antagonist licensed for treatment of nausea and vomiting (n&v) associated with emetogenic chemotherapy. There are case reports of use for n&v post-operatively or secondary to diabetic gastroparesis. There is no published evidence in patients with cystic fibrosis (CF). Tigecycline is used in treatment of Non-Tuberulosis Mycobacteria in CF. However uncontrolled n&v can result in dose reduction, interruption or discontinuation, and treatment failure. AIMS: To evaluate the efficiacy and side-effects of aprepitant (added to domperidone and ondansetron) in CF patients requiring tigecycline treatment. METHODS: Retrospective and prospective audits (studies) were conducted with case note review of consecutive CF patients receiving IV tigecycline, using a standardised proforma. Consecutive admissions for IV tigecycline therapy were consented and commenced on aprepitant 80mg OD for the first five days of tigecycline therapy. They completed daily n&v and side-effect diaries. RESULTS: 16 case notes were evaluated retrospectively and 10 patients prospectively audited. Aprepitant was likely to have contributed to reduction in n&v in 17/26 (65%) cases. 9/10 (90%) patients in prospective audit completed the full course/dose of tigecycline. 8/26 (31%) cases reported mild side-effects (headache (3), hiccups, dyspepsia (2), abdominal pain, atrial tachycardia); all resolved on continuing aprepitant or persisted despite stopping aprepitant. CONCLUSIONS: Aprepitant was well tolerated and appeared to be effective for a number of CF patients. Ongoing work will further evaluate the benefit of aprepitant in this population and inform clinical guidelines for management of n&v in CF patients.
Hall H, Gadhok R, Alshafi K, et al., 2015, Eradication of respiratory tract MRSA at a Large adult cystic fibrosis centre, RESPIRATORY MEDICINE, Vol: 109, Pages: 357-363, ISSN: 0954-6111
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- Citations: 17
Jones A, Simmonds N, Gyi KM, et al., 2015, Clinical Differences In Mycobacterium Abscessus Subspecies Infection In Cystic Fibrosis Patients, International Conference of the American-Thoracic-Society (ATS), Publisher: AMER THORACIC SOC, ISSN: 1073-449X
CFelton I, Benson I, Nicholson A, et al., 2014, PRELIMINARY EVALUATION OF THE FUNGAL AIRWAY MICROBIOME IN ADULT CYSTIC FIBROSIS BY NEXT-GENERATION SEQUENCING, CULTURE AND STAINING TECHNIQUES, Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A164-A164, ISSN: 0040-6376
Felton IC, Simmonds NJ, 2014, Aspergillus and cystic fibrosis: old disease - new classifications, CURRENT OPINION IN PULMONARY MEDICINE, Vol: 20, Pages: 632-638, ISSN: 1070-5287
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- Citations: 16
Waddingham P, Simmonds N, Bilton D, 2014, Safety, tolerabili and efficacy of intravenous aminophylline in adult patients th pulmonary exacerbations of cystic fibrosis, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936
Mukherjee S, Khan SA, Toledano MB, et al., 2014, THE BURDEN OF CYSTIC FIBROSIS ASSOCIATED LIVER DISEASE IN THE UNITED KINGDOM: A NATIONAL REGISTRY REVIEW, 2008-2012, PEDIATRIC PULMONOLOGY, Vol: 49, Pages: 414-415, ISSN: 8755-6863
Shaw F, Beverley Z, Russell J, et al., 2014, IS THE GOAL ATTAINMENT SCALE (GAS) A USEFUL OUTCOME MEASURE FOR ADULT CYSTIC FIBROSIS INPATIENT THERAPY? A PILOT STUDY, PEDIATRIC PULMONOLOGY, Vol: 49, Pages: 373-374, ISSN: 8755-6863
Barry PJ, Plant BJ, Nair A, et al., 2014, Effects of Ivacaftor in Patients With Cystic Fibrosis Who Carry the G551D Mutation and Have Severe Lung Disease, CHEST, Vol: 146, Pages: 152-158, ISSN: 0012-3692
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- Citations: 74
Savi D, De Biase RV, Amaddeo A, et al., 2014, Burkholderia pyrrocinia in Cystic Fibrosis Lung Transplantation: A Case Report, TRANSPLANTATION PROCEEDINGS, Vol: 46, Pages: 295-297, ISSN: 0041-1345
Green H, Gadhok R, Alshafi K, et al., 2013, SUCCESSFUL ERADICATION OF RESPIRATORY TRACT MRSA IN CYSTIC FIBROSIS: A RETROSPECTIVE STUDY, Winter Meeting of the British-Thoracic-Society, Publisher: BMJ PUBLISHING GROUP, Pages: A120-A120, ISSN: 0040-6376
Pabary R, Waller M, Harman K, et al., 2013, INTERPRETATION OF NASAL POTENTIAL DIFFERENCE MEASUREMENTS IN DIFFICULT CASES OF POSSIBLE CYSTIC FIBROSIS AND THE ROLE OF PUBLISHED EQUATIONS, PEDIATRIC PULMONOLOGY, Vol: 48, Pages: 257-257, ISSN: 8755-6863
Hallett Z, Madge S, Thrift L, et al., 2013, ANNUAL ASSESSMENTS: A QUALITY IMPROVEMENT ( QI) PROJECT INVOLVING PATIENTS AND PROFESSIONALS, PEDIATRIC PULMONOLOGY, Vol: 48, Pages: 392-392, ISSN: 8755-6863
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