Publications by authors named "Freddy Frost"

17 Publications

  • Page 1 of 1

Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis.

Cochrane Database Syst Rev 2021 12 10;12:CD013079. Epub 2021 Dec 10.

Adult CF Centre, Liverpool Heart & Chest Hospital, Liverpool, UK.

Background: Cystic fibrosis (CF) a life-limiting inherited disease affecting a number of organs, but classically associated with chronic lung infection and progressive loss of lung function. Chronic infection by Burkholderia cepacia complex (BCC) is associated with increased morbidity and mortality and therefore represents a significant challenge to clinicians treating people with CF. This review examines the current evidence for long-term antibiotic therapy in people with CF and chronic BCC infection.

Objectives: The objective of this review is to assess the effects of long-term oral and inhaled antibiotic therapy targeted against chronic BCC lung infections in people with CF. The primary objective is to assess the efficacy of treatments in terms of improvements in lung function and reductions in exacerbation rate. Secondary objectives include quantifying adverse events, mortality and changes in quality of life associated with treatment.

Search Methods: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched online trial registries and the reference lists of relevant articles and reviews. Date of last search: 12 April 2021.

Selection Criteria: Randomised controlled trials (RCTs) of long-term antibiotic therapy in people with CF and chronic BCC infection.

Data Collection And Analysis: Two authors independently extracted data, assessed risk of bias and assessed the quality of the evidence using GRADE.

Main Results: We included one RCT (100 participants) which lasted 52 weeks comparing continuous inhaled aztreonam lysine (AZLI) and placebo in a double-blind RCT for 24 weeks, followed by a 24-week open-label extension and a four-week follow-up period. The average participant age was 26.3 years, 61% were male and average lung function was 56.5% predicted. Treatment with AZLI for 24 weeks was not associated with improvement in forced expiratory volume in one second (FEV), mean difference 0.91% (95% confidence interval (CI) -3.15 to 4.97) (moderate-quality evidence). The median time to the next exacerbation was 75 days in the AZLI group compared to 51 days in the placebo group, but the difference was not significant (P = 0.27) (moderate-quality evidence). Similarly, the number of participants hospitalised for respiratory exacerbations showed no difference between groups, risk ratio (RR) 0.88 (95% CI 0.53 to 1.45) (moderate-quality evidence). Overall adverse events were similar between groups, RR 1.08 (95% CI 0.98 to 1.19) (moderate-quality evidence). There were no significant differences between treatment groups in relation to mortality (moderate-quality evidence), quality of life or sputum density. In relation to methodological quality, the overall risk of bias in the study was assessed to be unclear to low risk.

Authors' Conclusions: We found insufficient evidence from the literature to determine an effective strategy for antibiotic therapy for treating chronic BCC infection.
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http://dx.doi.org/10.1002/14651858.CD013079.pub3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8662788PMC
December 2021

Continuous glucose monitoring systems for monitoring cystic fibrosis-related diabetes.

Cochrane Database Syst Rev 2021 11 29;11:CD013755. Epub 2021 Nov 29.

Adult CF Centre, Liverpool Heart & Chest Hospital, Liverpool, UK.

Background: Cystic fibrosis (CF) is one of the most common life-shortening autosomal-recessive genetic conditions with around 100,000 people affected globally. CF mainly affects the respiratory system, but cystic fibrosis-related diabetes (CFRD) is a common extrapulmonary co-morbidity and causes excess morbidity and mortality in this population. Continuous glucose monitoring systems (CGMS) are a relatively new technology and, as yet, the impact of these on the monitoring and subsequent management of CFRD remains undetermined.

Objectives: To establish the impact of insulin therapy guided by continuous glucose monitoring compared to insulin therapy guided by other forms of glucose data collection on the lives of people with CFRD.

Search Methods: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. Date of latest search: 23 September 2021. We also searched the reference lists of relevant articles and reviews and online trials registries. Date of last search: 23 September 2021.

Selection Criteria: Randomised controlled studies comparing insulin regimens led by data from CGMS (including real-time or retrospective data, or both) with insulin regimens guided by abnormal blood glucose measurements collected through other means of glycaemic data collection in people with CFRD. Studies with a cross-over design, even with a washout period between intervention arms, are not eligible for inclusion due to the potential long-term impact of each of the interventions and the potential to compromise the outcomes of the second intervention.

Data Collection And Analysis: No studies were included in the review, meaning that no data were available to be collected for analysis.

Main Results: Review authors screened 14 studies at the full-text stage against the review's inclusion criteria. Consequently, seven were excluded due to the study type being ineligible (not randomised), two studies were excluded due to their cross-over design, and two studies was excluded since the intervention used was not eligible and one was a literature review. One study in participants hospitalised for a pulmonary exacerbation is ongoing. Investigators are comparing insulin dosing via insulin pump with blood sugar monitoring by a CGMS to conventional diabetes management with daily insulin injections (or on an insulin pump if already on an insulin pump in the outpatient setting) and capillary blood glucose monitoring. The participants in the control arm will wear a blinded continuous glucose monitoring system for outcome assessment. In addition to this, one further study is still awaiting classification, and will be screened to determine whether it is eligible for inclusion, or is to be excluded, in an update of this review.

Authors' Conclusions: No studies were included in the review, indicating that there is currently insufficient evidence to determine the impact of insulin therapy guided by CGMS compared to insulin therapy guided by other forms of glucose data collection on the lives of people with CFRD, nor on potential adverse effects of continuous glucose monitoring in this context. Randomised controlled studies are needed to generate evidence on the efficacy and safety of continuous glucose monitoring in people with CFRD. There is one relevant ongoing study that may be eligible for inclusion in a future update of this Cochrane Review, and whose results may help answer the review question.
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http://dx.doi.org/10.1002/14651858.CD013755.pub2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8629645PMC
November 2021

The rapid development and deployment of a new multidisciplinary CPAP service outside of a critical care environment during the early stages of the COVID-19 pandemic.

Future Healthc J 2021 Mar;8(1):e156-e159

Royal Liverpool Hospital, Liverpool, UK.

The COVID-19 pandemic has led to a dramatic increase in patients presenting with type 1 respiratory failure. In order to protect our limited critical care capacity, we rapidly developed a new ward-based inpatient continuous positive airway pressure (CPAP) service with direct input from the respiratory, infectious diseases and critical care teams. Close collaboration between these specialties and new innovative solutions were required to facilitate this. CPAP equipment (normally reserved for domiciliary care) was adapted to reduce the pressure on our strained oxygen infrastructure. Side rooms on the infectious diseases ward were swiftly converted into new negative pressure areas using temporary installed ventilatory equipment, reducing the viral aerosol risk for staff. Novel patient monitoring solutions were used to protect staff while also ensuring patient safety. Staff training and specialist oversight was organised within days. The resulting service was successful, with over half (17/26 (65%)) of patients avoiding invasive ventilation.
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http://dx.doi.org/10.7861/fhj.2020-0167DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8004335PMC
March 2021

Assessing the validity and applicability of the French 3-year prognostic score in the UK cystic fibrosis population - a national cohort study.

Transpl Int 2021 03 24;34(3):436-444. Epub 2021 Jan 24.

Manchester University NHS Foundation Trust, Wythenshawe, UK.

Models that predict outcomes, aid prognostication and inform the assessment of urgency for lung transplantation (LT) in CF are in demand. A prognostic score derived from the French adult CF registry to predict death or LT over 3-year follow-up was described in 2017 and validated using Canadian CF registry data. We assessed its performance in the UK CF population. The French prognostic score was applied to untransplanted adults with CF. The index year (2014) and outcomes (Death or LT) were evaluated to 2017. Receiver operator characteristics plots and area under curve (AUC) was computed. 4407 adults with CF met the inclusion criteria. After 3 years, 7.1% (P < 0.001) were dead or had received LT compared to the French (12.8%) and Canadian (9.4%) cohorts. The French score deemed 592 (26.2%) 'High-risk' - death/LT occurred in 189/592 (30.2%), less than previously reported in France and Canada (P < 0.0001). The discriminatory power of the French score was lower (AUC 0.830) than reported. Recalibration yielded only marginal improvement in model performance (AUC 0.833). The French prognostic score does not perform as well in the UK as reported elsewhere. Bespoke UK scores are needed to aid prognostication and inform LT decision-making.
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http://dx.doi.org/10.1111/tri.13805DOI Listing
March 2021

The clinical and microbiological utility of inhaled aztreonam lysine for the treatment of acute pulmonary exacerbations of cystic fibrosis: An open-label randomised crossover study (AZTEC-CF).

J Cyst Fibros 2021 11 7;20(6):994-1002. Epub 2021 Jan 7.

Adult CF Centre, Liverpool Heart & Chest Hospital, UK; Institute of Infection & Global Health, University of Liverpool, UK.

Background: The objective of this study was to explore the clinical and microbiological outcomes associated with substituting inhaled aztreonam lysine for an intravenous antibiotic in the treatment of acute pulmonary exacerbations of CF.

Methods: An open-label randomised crossover pilot trial was conducted at a UK CF centre among 16 adults with CF and P. aeruginosa infection. Median [IQR] age was 29.5 [24.5-32.5], mean ± SD forced expiratory volume in 1 second (FEV1) was 52.4 ± 14.7 % predicted. Over the course of two exacerbations, participants were randomised to sequentially receive 14 days of inhaled aztreonam lysine plus IV colistimethate (AZLI+IV), or dual IV antibiotics (IV+IV). Primary outcome was absolute change in % predicted FEV1. Other outcomes evaluated changes in quality of life, bacterial load and the lung microbiota.

Results: The difference between mean change in lung function at day 14 between AZLI+IV and IV+IV was +4.6% (95% CI 2.1-7.2, p=0.002). The minimum clinically important difference of the Cystic Fibrosis Revised Questionnaire (CFQ-R) was achieved more frequently with AZLI+IV (10/12, 83.3%) than IV+IV (7/16, 43.8%), p=0.05. No differences were observed for modulation of serum white cell count, C-reactive protein or sputum bacterial load. Microbiome compositional changes were observed with IV+IV (Bray-Curtis r=0.14, p=0.02), but not AZLI+IV (r=0.03, p=0.64).

Conclusion: In adults with CF and P. aeruginosa infection experiencing an acute pulmonary exacerbation, AZLI+IV improved lung function and quality of life compared to the current standard treatment. These findings support the need for larger definitive trials of inhaled antibiotics in the acute setting.

Clinical Trial Registration: EudraCT 2016-002832-34 ClinicalTrials.org NCT02894684.
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http://dx.doi.org/10.1016/j.jcf.2020.12.012DOI Listing
November 2021

Utility of established prognostic scores in COVID-19 hospital admissions: multicentre prospective evaluation of CURB-65, NEWS2 and qSOFA.

BMJ Open Respir Res 2020 12;7(1)

Institute of Infection, Veterinary and Ecological Sciences, University of Liverpool, Liverpool, UK

Introduction: The COVID-19 pandemic is ongoing, yet, due to the lack of a COVID-19-specific tool, clinicians must use pre-existing illness severity scores for initial prognostication. However, the validity of such scores in COVID-19 is unknown.

Methods: The North West Collaborative Organisation for Respiratory Research performed a multicentre prospective evaluation of adult patients admitted to the hospital with confirmed COVID-19 during a 2-week period in April 2020. Clinical variables measured as part of usual care at presentation to the hospital were recorded, including the Confusion, Urea, Respiratory Rate, Blood Pressure and Age Above or Below 65 Years (CURB-65), National Early Warning Score 2 (NEWS2) and Quick Sequential (Sepsis-Related) Organ Failure Assessment (qSOFA) scores. The primary outcome of interest was 30-day mortality.

Results: Data were collected for 830 people with COVID-19 admitted across seven hospitals. By 30 days, a total of 300 (36.1%) had died and 142 (17.1%) had been in the intensive care unit. All scores underestimated mortality compared with pre-COVID-19 cohorts, and overall prognostic performance was generally poor. Among the 'low-risk' categories (CURB-65 score<2, NEWS2<5 and qSOFA score<2), 30-day mortality was 16.7%, 32.9% and 21.4%, respectively. NEWS2≥5 had a negative predictive value of 98% for early mortality. Multivariable logistic regression identified features of respiratory compromise rather than circulatory collapse as most relevant prognostic variables.

Conclusion: In the setting of COVID-19, existing prognostic scores underestimated risk. The design of new prognostic tools should focus on features of respiratory compromise rather than circulatory collapse. We provide a baseline set of variables which are relevant to COVID-19 outcomes and may be used as a basis for developing a bespoke COVID-19 prognostication tool.
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http://dx.doi.org/10.1136/bmjresp-2020-000729DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7722817PMC
December 2020

Identification of high-risk non-ST elevation myocardial infarction at presentation to emergency department. A prospective observational cohort study in North West England.

BMJ Open 2020 06 8;10(6):e030128. Epub 2020 Jun 8.

Liverpool Clinical Trials Centre, University of Liverpool, UK, Liverpool, England.

Objectives: Early access to invasive coronary angiography and revascularisation for high-risk non-ST elevation myocardial infarction (NSTEMI) improves outcomes and is supported by current guidelines. We sought to determine the most effective criteria at presentation to emergency department (ED) to identify high-risk NSTEMI.

Setting: Secondary care centre northwest England with national follow-up.

Participants: 1642 consecutive patients (median age 59, 52% male) presenting to ED with a primary symptom of chest pain in whom there is suspicion of NSTEMI.

Primary And Secondary Measures: Multivariate logistic regression analysis for the prediction of all-cause death (primary) and major adverse cardiac event (MACE defined as all-cause death, unplanned coronary revascularisation and adjudicated NSTEMI (third universal definition)) (secondary measure) at 1 year.

Results: The incidence of adjudicated NSTEMI was 10.7%, and 1-year mortality was 6.3%. Independent predictors for all-cause death at 1 year were Global Registry of Acute Coronary Events (GRACE) >140, age (per decade increase) and high-sensitive cardiac troponin T (hs-cTnT) >50 ng/L. hs-cTnT >50 ng/L was associated with adjudicated index presentation NSTEMI in the greatest proportion of patients (61.7%). When using MACE at 12 months, as opposed to all-cause death, as an end point History, ECG, Age, Risk factors and Troponin (HEART) score ≥7 was included in the multivariate model and had better prediction of index NSTEMI than GRACE>140. Combining hs-cTnT >50 ng/L and a second independent predictor identified both a high proportion of index NSTEMI and elevated risk of all-cause death at 1 year.

Conclusions: hs-cTnT >50 ng/L or HEART score ≥7 appear effective strategies to identify high-risk NSTEMI at presentation to emergency room with chest pain. Multicentre prospective studies enriched with early presenters, and with competitor high-sensitive and point-of-care troponins, are required to validate and extend these findings.

Trial Registration Number: NCT02581540.
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http://dx.doi.org/10.1136/bmjopen-2019-030128DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7282291PMC
June 2020

Ivacaftor Is Associated with Reduced Lung Infection by Key Cystic Fibrosis Pathogens. A Cohort Study Using National Registry Data.

Ann Am Thorac Soc 2019 11;16(11):1375-1382

Liverpool Adult Cystic Fibrosis Centre, Liverpool Heart and Chest Hospital NHS, Foundation Trust, Liverpool, United Kingdom.

Ivacaftor can greatly improve clinical outcomes in people with cystic fibrosis (CF) and has been shown to have antibacterial properties, yet the long-term microbiological outcomes of treatment are unknown. To investigate changes in respiratory microbiology associated with long-term ivacaftor use. This was a retrospective cohort study using data from the UK CF Registry 2011-2016. Primary outcome was the annual prevalence ratios for key CF pathogens between ivacaftor users and their contemporaneous comparators. Multivariable log-binomial regression models were designed to adjust for confounders. Changes in status were compared between groups using nonparametric maximum likelihood estimate for the purposes of Kaplan-Meier approximation. Ivacaftor use was associated with early and sustained reduction in rates (2016 adjusted prevalence ratio, 0.68; 95% confidence interval, 0.58-0.79;  < 0.001) via a combination of increased clearance in those with infection (ivacaftor: 33/87 [37.9%] vs. nonivacaftor: 432/1,872 [22.8%];  < 0.001) and reduced acquisition in those without infection (49/134 [36.6%] vs. 1,157/2,382 [48.6%];  = 0.01). The improved prevalence of infection was independent of reduced sampling in the ivacaftor cohort. Ivacaftor was also associated with reduced prevalence of and spp. but not complex. In this study, long-term ivacaftor use was associated with reduced infection with important CF pathogens including . These findings have implications for antibiotic stewardship and the need for ongoing chronic antimicrobial therapy in this cohort.
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http://dx.doi.org/10.1513/AnnalsATS.201902-122OCDOI Listing
November 2019

Antibiotic therapy for chronic infection with Burkholderia cepacia complex in people with cystic fibrosis.

Cochrane Database Syst Rev 2019 06 13;6:CD013079. Epub 2019 Jun 13.

Adult CF Centre, Liverpool Heart & Chest Hospital, Thomas Drive, Liverpool, Merseyside, UK, L3 9BZ.

Background: Cystic fibrosis (CF) a life-limiting inherited disease affecting a number of organs, but classically associated with chronic lung infection and progressive loss of lung function. Chronic infection by Burkholderia cepacia complex (BCC) is associated with increased morbidity and mortality and therefore represents a significant challenge to clinicians treating people with CF. This review examines the current evidence for long-term antibiotic therapy in people with CF and chronic BCC infection.

Objectives: The objective of this review is to assess the effects of long-term oral and inhaled antibiotic therapy targeted against chronic BCC lung infections in people with CF. The primary objective is to assess the efficacy of treatments in terms of improvements in lung function and reductions in exacerbation rate. Secondary objectives include quantifying adverse events, mortality and changes in quality of life associated with treatment.

Search Methods: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched online trial registries and the reference lists of relevant articles and reviews.Date of last search: 29 May 2019.

Selection Criteria: Randomised controlled trials (RCTs) of long-term antibiotic therapy in people with CF and chronic BCC infection.

Data Collection And Analysis: Two authors independently extracted data, assessed risk of bias and assessed the quality of the evidence using GRADE.

Main Results: We included one RCT (100 participants) which lasted 52 weeks comparing continuous inhaled aztreonam lysine (AZLI) and placebo in a double-blind RCT for 24 weeks, followed by a 24-week open-label extension and a four-week follow-up period. The average participant age was 26.3 years, 61% were male and average lung function was 56.5% predicted.Treatment with AZLI for 24 weeks was not associated with improvement in forced expiratory volume in one second (FEV), mean difference 0.91% (95% confidence interval (CI) -3.15 to 4.97) (moderate-quality evidence). The median time to the next exacerbation was 75 days in the AZLI group compared to 51 days in the placebo group, but the difference was not significant (P = 0.27) (moderate-quality evidence). Similarly, the number of participants hospitalised for respiratory exacerbations showed no difference between groups, risk ratio (RR) 0.88 (95% CI 0.53 to 1.45) (moderate-quality evidence). Overall adverse events were similar between groups, RR 1.08 (95% CI 0.98 to 1.19) (moderate-quality evidence). There were no significant differences between treatment groups in relation to mortality (moderate-quality evidence), quality of life or sputum density.In relation to methodological quality, the overall risk of bias in the study was assessed to be unclear to low risk.

Authors' Conclusions: We found insufficient evidence from the literature to determine an effective strategy for antibiotic therapy for treating chronic BCC infection.
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http://dx.doi.org/10.1002/14651858.CD013079.pub2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6564086PMC
June 2019

Cystic fibrosis-related diabetes: optimizing care with a multidisciplinary approach.

Diabetes Metab Syndr Obes 2019 26;12:545-552. Epub 2019 Apr 26.

Respiratory Medicine, Adult CF Centre, Liverpool Heart & Chest Hospital, Liverpool L14 3PE, UK.

Cystic fibrosis-related diabetes (CFRD) is a common complication of cystic fibrosis and can be present in over 50% of adults with the disease. CFRD is associated with poorer clinical outcomes, including accelerated pulmonary function decline and excess morbidity. The management of CFRD is complex and differs from that of type 1 and type 2 diabetes mellitus such that clinicians responsible for the care of people with CFRD must work closely with colleagues across a number of different specialities and disciplines. This review aims to discuss why a multi-disciplinary approach is important and how it can be harnessed to optimize the care of people with CFRD.
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http://dx.doi.org/10.2147/DMSO.S180597DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6499442PMC
April 2019

Cystic fibrosis related diabetes is not independently associated with increased Stenotrophomonas maltophilia infection: Longitudinal data from the UK CF Registry.

J Cyst Fibros 2019 03 25;18(2):294-298. Epub 2018 Oct 25.

Adult Cystic Fibrosis Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK. Electronic address:

Introduction: Stenotrophomonas maltophilia is common in the sputum of people with cystic fibrosis related diabetes (CFRD), raising the question as to whether this is a risk factor for its acquisition. We investigated this at a population level.

Methods: We analysed national Cystic Fibrosis Registry data 2011-2015 for 8047 people with CF > age 6 years, looking at demographics, diagnosis of CFRD, lung function and sputum microbiology; using descriptive and multivariate strategies to establish independent predictors for S. maltophilia culture and associated outcomes.

Results: S. maltophilia was present in 1148 (14.1%). Although univariate analysis confirmed it was more prevalent in those with CFRD, when adjusted for other clinical parameters there was no longer a relationship. Markers of more severe lung disease were independent risk-factors for S. maltophilia.

Conclusion: Although S. maltophilia is more common in people with CFRD, it is not an independent risk-factor for S. maltophilia acquisition.
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http://dx.doi.org/10.1016/j.jcf.2018.10.011DOI Listing
March 2019

Between a Rock and an Airspace: Pneumothorax After Extracorporeal Shock Wave Lithotripsy for Renal Stones in a Patient With Cystic Fibrosis.

Chest 2018 09;154(3):e61-e63

Adult CF Centre, Liverpool Heart and Chest Hospital, Liverpool, UK.

Renal disease is a well-recognized manifestation of cystic fibrosis (CF) and people with CF are at increased risk of nephrolithiasis. Lithotripsy is the preferred treatment but has occasionally been associated with pulmonary complications. Here we report the case of a person with CF who developed a pneumothorax soon after lithotripsy and discuss the potential mechanism of injury. We hope this case highlights some of the additional considerations clinicians should take into account when managing patients with advanced pulmonary disease in CF.
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http://dx.doi.org/10.1016/j.chest.2018.03.023DOI Listing
September 2018

Pulmonary function testing is safe in patients with thoracic aortic aneurysms.

Eur Respir J 2018 11 15;52(5). Epub 2018 Nov 15.

Respiratory Dept, Liverpool Heart and Chest Hospital, Liverpool, UK.

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http://dx.doi.org/10.1183/13993003.00928-2018DOI Listing
November 2018

Case Report: First report of infection in a patient with cystic fibrosis.

F1000Res 2018 10;7:440. Epub 2018 Apr 10.

Liverpool Heart & Chest Hospital, Liverpool, L14 3PE, UK.

is a rare non-fermenting Gram-negative rod that has previously been reported to be associated with blood stream and pulmonary abscess infections, but never before in cystic fibrosis (CF). Here we present the first reported case of infection in a patient with CF and discuss the management options. We describe a patient with CF in whom we observed clinical and spirometric evidence of pulmonary exacerbation with the associated growth of in sputum culture. The period of clinical instability was observed to coincide with the obtainment of four sputum samples from which was cultured; improvement was seen following treatment with ciprofloxacin and the subsequent eradication of . We conclude that is able to survive in the CF lung and in this case was associated with pulmonary exacerbation. Empirical treatment with fluoroquinolones is appropriate, based on our experience.
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http://dx.doi.org/10.12688/f1000research.14441.2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6058470PMC
April 2018

Continuous glucose monitoring guided insulin therapy is associated with improved clinical outcomes in cystic fibrosis-related diabetes.

J Cyst Fibros 2018 11 7;17(6):798-803. Epub 2018 Jun 7.

Liverpool Adult CF Centre, Liverpool Heart and Chest Hospital, Liverpool L14 3PE, UK.

Introduction: Continuous glucose monitoring (CGM) allows assessment of day to day glycaemic excursions and detects early glucose handling abnormalities that may not be apparent on oral glucose tolerance testing (OGTT). However, there is little published evidence as to whether these early dysglycaemic changes are amenable to treatment. We present outcomes following CGM guided insulin initiation at our centre.

Methods: Adults without a prior diagnosis of cystic fibrosis related diabetes (CFRD) whom underwent >72 h CGM at our adult CF centre were included in the study. Clinical outcomes including weight and pulmonary function changes over the next 12 months were compared between groups based on CGM results and subsequent management.

Results: CGM profiles for 59 patients were analysed. Insulin was commenced in 37 patients who had evidence of hyperglycaemia on CGM. Significant improvements in mean [95% confidence intervals] forced expiratory volume in 1 s (FEV) (+4.3% predicted [1.06-7.48], p = 0.01) and weight (+1.2 kg [0.32-2.15], p = 0.01) were observed at 3 months in the insulin group. Annual rate of pulmonary function decline was also improved following insulin initiation.

Conclusion: Insulin treatment targeted towards glycaemic excursions seen on CGM is associated with improvements in lung function and weight with subsequent reduced pulmonary function decline.
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http://dx.doi.org/10.1016/j.jcf.2018.05.005DOI Listing
November 2018

Case Report: Haemolytic anaemia with ceftazidime use in a patient with cystic fibrosis.

F1000Res 2018 18;7:475. Epub 2018 Apr 18.

Liverpool Heart and Chest Hospital NHS Trust, Liverpool, L14 3PE, UK.

Drug-induced Immune Haemolytic Anaemia (DIIHA) is a rare but serious complication of cephalosporin use. Ceftazidime is recognized to be a rare cause of DIIHA. We report and discuss a case of DIIHA in a person with cystic fibrosis who developed severe haemolytic anaemia following use of ceftazidime in the management of an acute pseudomonal pulmonary exacerbation.
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http://dx.doi.org/10.12688/f1000research.14505.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5920311PMC
April 2018

Ghost of chest drain past.

Thorax 2018 06 29;73(6):595-596. Epub 2017 Nov 29.

Respiratory Medicine, Liverpool Heart and Chest Hospital, Liverpool, UK.

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http://dx.doi.org/10.1136/thoraxjnl-2017-210991DOI Listing
June 2018
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