Nicholas J. Simmonds
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View article: Obstetric and Neonatal Outcomes in Pregnancies From a Dedicated Cystic Fibrosis‐Maternal Health Service: A Retrospective Study
Obstetric and Neonatal Outcomes in Pregnancies From a Dedicated Cystic Fibrosis‐Maternal Health Service: A Retrospective Study Open
Objective A comprehensive review of maternal, obstetric and neonatal outcomes in pregnancies in females with cystic fibrosis (fwCF) following the introduction of Elexacaftor/Tezacaftor/Ivacaftor (ETI) therapy in a novel, dedicated CF‐Mater…
View article: Spirometry thresholds for clinical trial eligibility: time for urgent re-evaluation
Spirometry thresholds for clinical trial eligibility: time for urgent re-evaluation Open
A common eligibility criterion in respiratory clinical trials is a per cent-predicted forced expiratory volume in 1 second (ppFEV 1 ) between 40% and 90%, using the ethnicity-dependent Global Lung Function Initiative (GLI)-2012 spirometry …
View article: Managing an ageing cystic fibrosis population: challenges and priorities
Managing an ageing cystic fibrosis population: challenges and priorities Open
The increasing life expectancy of people with cystic fibrosis (pwCF), largely driven by advancements in early diagnosis, multidisciplinary care and the recent introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modul…
View article: Improving ototoxicity monitoring in patients receiving aminoglycosides using a novel digital approach: a quality improvement project
Improving ototoxicity monitoring in patients receiving aminoglycosides using a novel digital approach: a quality improvement project Open
Aminoglycoside antibiotics cause ototoxicity for which baseline audiometric testing is recommended but often not done. Barriers to successful implementation include limited availability of sound-booths and audiologists. An ototoxicity moni…
View article: Increased NFAT and NFκB signalling contribute to the hyperinflammatory phenotype in response to Aspergillus fumigatus in a mouse model of cystic fibrosis
Increased NFAT and NFκB signalling contribute to the hyperinflammatory phenotype in response to Aspergillus fumigatus in a mouse model of cystic fibrosis Open
Aspergillus fumigatus ( Af ) is a major mould pathogen found ubiquitously in the air. It commonly infects the airways of people with cystic fibrosis (CF) leading to Aspergillus bronchitis or allergic bronchopulmonary aspergillosis. Residen…
View article: Increased NFAT and NFκB signalling contribute to the hyperinflammatory phenotype in response to<i>Aspergillus fumigatus</i>in cystic fibrosis
Increased NFAT and NFκB signalling contribute to the hyperinflammatory phenotype in response to<i>Aspergillus fumigatus</i>in cystic fibrosis Open
Aspergillus fumigatus ( Af ) is a major mould pathogen found ubiquitously in the air. It commonly infects the airways of people with cystic fibrosis (CF) leading to Aspergillus bronchitis or allergic bronchopulmonary aspergillosis. Residen…
View article: Measurement of treatment burden in cystic fibrosis: A systematic review
Measurement of treatment burden in cystic fibrosis: A systematic review Open
No single measure used in the reviewed studies fully the multidimensional nature of treatment burden and summarised it in a single score. Given the rapidly evolving landscape of CF care a pragmatic approach to capture a broader array of tr…
View article: Bridging the gap: Challenging lung infections and clinical trial development in cystic fibrosis
Bridging the gap: Challenging lung infections and clinical trial development in cystic fibrosis Open
View article: Health State Utilities Associated With Treatment Burden in Cystic Fibrosis
Health State Utilities Associated With Treatment Burden in Cystic Fibrosis Open
Background: Although recent advancements in the treatment of cystic fibrosis (CF) have improved survival, reducing high levels of treatment burden remains a priority issue for many people with cystic fibrosis (pwCF). However, economic eval…
View article: Levelling the playing field through the London Network of the UK clinical trials accelerator platform
Levelling the playing field through the London Network of the UK clinical trials accelerator platform Open
View article: ECFS standards of care on CFTR-related disorders: Identification and care of the disorders
ECFS standards of care on CFTR-related disorders: Identification and care of the disorders Open
View article: Inhaled antimicrobial prescribing for Pseudomonas aeruginosa infections in Europe
Inhaled antimicrobial prescribing for Pseudomonas aeruginosa infections in Europe Open
The inhaled antimicrobial prescribing decision-making process is multifactorial. Nebulised tobramycin or colistin are often used in initial eradication and chronic suppression regimens. To date, CFTR modulator therapy has had a limited imp…
View article: The effectiveness of CFTR modulators in people with CF and rare mutations: A real‐world study
The effectiveness of CFTR modulators in people with CF and rare mutations: A real‐world study Open
Cystic fibrosis (CF) is an autosomal recessive disease, caused by reduced function of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR modulators (CFTRm) directly target this defect, first becoming commercially …
View article: Cystic fibrosis modulator therapy can reverse cystic bronchiectasis
Cystic fibrosis modulator therapy can reverse cystic bronchiectasis Open
Bronchiectasis is often considered progressive and irreversible, so cases of regression or reversal are an important step in understanding the underlying pathophysiological mechanisms. Cystic fibrosis, (CF) caused by pathogenic variants in…
View article: P394 Comparisons of the personal well-being score (PWS) and health-related quality of life for people with cystic fibrosis before and during the COVID-19 pandemic
P394 Comparisons of the personal well-being score (PWS) and health-related quality of life for people with cystic fibrosis before and during the COVID-19 pandemic Open
View article: WS10.06 How representative are clinical trial cohorts of the general cystic fibrosis population? Implications for trial planning
WS10.06 How representative are clinical trial cohorts of the general cystic fibrosis population? Implications for trial planning Open
View article: WS06.01 Outcome measures for assessment of lung function – cross-sectional analysis of relationships between impulse oscillometry, electrical impedance tomography, multiple breath washout and spirometry
WS06.01 Outcome measures for assessment of lung function – cross-sectional analysis of relationships between impulse oscillometry, electrical impedance tomography, multiple breath washout and spirometry Open
View article: P178 Impact of pre-conception lung function in women with cystic fibrosis (wwCF) on obstetric and neonatal outcomes in the elexacaftor/tezacaftor/ivacaftor (ETI)-era
P178 Impact of pre-conception lung function in women with cystic fibrosis (wwCF) on obstetric and neonatal outcomes in the elexacaftor/tezacaftor/ivacaftor (ETI)-era Open
View article: P177 Cystic fibrosis (CF)-related complications and outcomes of women during pregnancy and post-partum in elexacaftor/tezacaftor/ivacaftor (ETI)-era
P177 Cystic fibrosis (CF)-related complications and outcomes of women during pregnancy and post-partum in elexacaftor/tezacaftor/ivacaftor (ETI)-era Open
View article: P101 Mediation analysis investigating potential determinants of the survival gap between the sexes in cystic fibrosis; a complex picture
P101 Mediation analysis investigating potential determinants of the survival gap between the sexes in cystic fibrosis; a complex picture Open
View article: Diagnosing Cystic Fibrosis in Adults
Diagnosing Cystic Fibrosis in Adults Open
Diagnosing cystic fibrosis (CF) in adulthood is not a rare occurrence for CF centers despite the popular belief that the diagnosis is achieved almost universally in childhood by means of newborn screening or early clinical presentation. Th…
View article: Mukoviszidose-Modulatortherapie kann zystische Bronchiektasen rückgängig machen
Mukoviszidose-Modulatortherapie kann zystische Bronchiektasen rückgängig machen Open
Bronchiektasen werden oft als progressiv und irreversibel angesehen, sodass Fälle von Rückbildung oder Umkehr ein wichtiger Schritt zum Verständnis der zugrunde liegenden pathophysiologischen Mechanismen sind. Mukoviszidose (cystic fibrosi…
View article: La terapia con moduladores para la fibrosis quística puede revertir las bronquiectasias quísticas
La terapia con moduladores para la fibrosis quística puede revertir las bronquiectasias quísticas Open
Las bronquiectasias suelen considerarse progresivas e irreversibles, por lo que los casos de regresión o reversión constituyen un paso importante para comprender los mecanismos fisiopatológicos subyacentes. El tratamiento de la fibrosis qu…
View article: Assessing the health risk of living near composting facilities on lung health, fungal and bacterial disease in cystic fibrosis: a UK CF Registry study
Assessing the health risk of living near composting facilities on lung health, fungal and bacterial disease in cystic fibrosis: a UK CF Registry study Open
View article: Mediation of the total effect of cystic fibrosis‐related diabetes on mortality: A <scp>UK</scp> Cystic Fibrosis Registry cohort study
Mediation of the total effect of cystic fibrosis‐related diabetes on mortality: A <span>UK</span> Cystic Fibrosis Registry cohort study Open
Aim To investigate whether the effect of cystic fibrosis‐related diabetes (CFRD) on the composite outcome of mortality or transplant could act through lung function, pulmonary exacerbations and/or nutritional status. Methods A retrospectiv…
View article: An invisible threat? Aspergillus positive cultures and co-infecting bacteria in airway samples
An invisible threat? Aspergillus positive cultures and co-infecting bacteria in airway samples Open
View article: Treatment Preference Among People With Cystic Fibrosis
Treatment Preference Among People With Cystic Fibrosis Open
The relative importance of treatment burden to people with CF, compared with life expectancy and lung function, suggests it should be routinely captured in clinical trials as an important secondary outcome measure. When considering the pat…
View article: VOCAL: An observational study of ivacaftor for people with cystic fibrosis and selected non–G551D-CFTR gating mutations
VOCAL: An observational study of ivacaftor for people with cystic fibrosis and selected non–G551D-CFTR gating mutations Open
These results demonstrate the positive long-term effectiveness of ivacaftor on clinical outcomes and HCRU in pwCF with non-G551D-CFTR gating mutations in real-world settings.
View article: Assessing the health risk of living near composting facilities on lung health, fungal and bacterial disease in cystic fibrosis: a UK CF Registry study
Assessing the health risk of living near composting facilities on lung health, fungal and bacterial disease in cystic fibrosis: a UK CF Registry study Open
Aim To explore the health risk of living near permitted composting sites (PCSs) on disease severity in children and adults with cystic fibrosis (CF) across the UK. Methods A semi-individual cross-sectional study was used to examine risk of…
View article: Exploring the nature of perceived treatment burden: a study to compare treatment burden measures in adults with cystic fibrosis
Exploring the nature of perceived treatment burden: a study to compare treatment burden measures in adults with cystic fibrosis Open
Background: Despite the importance of reducing treatment burden for people with cystic fibrosis (CF), it has not been fully understood as a concept. This study aims to quantify the treatment burden perceived by CF adults and explore the a…