Search Results - "Carr, Siobhan"
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Childhood bronchiectasis, so little is known
Published in Archives of disease in childhood (01-06-2022)“…Correspondence to Professor Siobhán B Carr, Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield Hospitals, London, UK;…”
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De Novo Mutations in FOXJ1 Result in a Motile Ciliopathy with Hydrocephalus and Randomization of Left/Right Body Asymmetry
Published in American journal of human genetics (07-11-2019)“…Hydrocephalus is one of the most prevalent form of developmental central nervous system (CNS) malformations. Cerebrospinal fluid (CSF) flow depends on both…”
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Eradication success for non-tuberculous mycobacteria in children with cystic fibrosis
Published in The European respiratory journal (01-05-2021)“…Non-tuberculous mycobacteria (NTM) are an emerging pathogen worldwide in both cystic fibrosis (CF) and non-CF pulmonary disease (PD), with reports suggesting…”
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Treatment Preference Among People With Cystic Fibrosis: The Importance of Reducing Treatment Burden
Published in Chest (01-12-2022)“…There is a growing consensus that the perspective of the patient should be considered in the evaluation of novel interventions. What treatment outcomes matter…”
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Data Resource Profile: The UK Cystic Fibrosis Registry
Published in International journal of epidemiology (01-02-2018)Get full text
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Lung Clearance Index at 4 Years Predicts Subsequent Lung Function in Children with Cystic Fibrosis
Published in American journal of respiratory and critical care medicine (15-03-2011)“…The markedly improved life expectancy of children with cystic fibrosis (CF) has created a new challenge, as traditional markers of lung disease are frequently…”
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Incidence and risk factors of cancer in individuals with cystic fibrosis in the UK; a case-control study
Published in Journal of cystic fibrosis (01-03-2022)“…•What is the key question? Which are the most common cancers in the UK cystic fibrosis population and what factors are associated with the incidence of all…”
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Future therapies for cystic fibrosis
Published in Nature communications (08-02-2023)“…We are currently witnessing transformative change for people with cystic fibrosis with the introduction of small molecule, mutation-specific drugs capable of…”
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Topological data analysis reveals genotype-phenotype relationships in primary ciliary dyskinesia
Published in The European respiratory journal (01-08-2021)“…Primary ciliary dyskinesia (PCD) is a heterogeneous inherited disorder caused by mutations in approximately 50 cilia-related genes. PCD genotype-phenotype…”
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Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry Cohort study
Published in The European respiratory journal (01-09-2021)“…Median survival for cystic fibrosis (CF) patients in Europe is unknown and is likely to be influenced by socioeconomic factors. Using the European Cystic…”
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Using Negative Control Outcomes and Difference-in-Differences Analysis to Estimate Treatment Effects in an Entirely Treated Cohort: The Effect of Ivacaftor in Cystic Fibrosis
Published in American journal of epidemiology (01-03-2022)“…Abstract When an entire cohort of patients receives a treatment, it is difficult to estimate the treatment effect in the treated because there are no directly…”
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Are inhaled corticosteroids prescribed rationally in primary ciliary dyskinesia?
Published in The European respiratory journal (01-03-2018)“…Primary ciliary dyskinesia (PCD) is a chronic suppurative lung disease characterised by abnormal mucociliary clearance due to genetic defects of motile cilia…”
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Recovery of baseline lung function after pulmonary exacerbation in children with primary ciliary dyskinesia
Published in Pediatric pulmonology (01-12-2016)“…Summary Rationale Spirometry in children with cystic fibrosis (CF) frequently fails to return to baseline after treatment for a pulmonary exacerbation. It is…”
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An observational study of the lung clearance index throughout childhood in cystic fibrosis: early years matter
Published in The European respiratory journal (01-10-2020)“…The London Cystic Fibrosis Collaboration (LCFC) has prospectively followed a clinically diagnosed cohort of infants with cystic fibrosis (CF) born in South…”
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Projecting the impact of triple CFTR modulator therapy on intravenous antibiotic requirements in cystic fibrosis using patient registry data combined with treatment effects from randomised trials
Published in Thorax (01-09-2022)“…Cystic fibrosis (CF) is a life-threatening genetic disease, affecting around 10 500 people in the UK. Precision medicines have been developed to treat specific…”
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Non-invasive ventilation and clinical outcomes in cystic fibrosis: Findings from the UK CF registry
Published in Journal of cystic fibrosis (01-09-2019)“…AbstractBackgroundNon-invasive ventilation (NIV) for respiratory failure and airway clearance is an established intervention in cystic fibrosis (CF), but its…”
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Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study
Published in Journal of cystic fibrosis (01-01-2021)“…The presence of co-morbidities, including underlying respiratory problems, has been identified as a risk factor for severe COVID-19 disease. Information on the…”
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Impact of cystic fibrosis on birthweight: a population based study of children in Denmark and Wales
Published in Thorax (01-05-2019)“…Poor growth during infancy and childhood is a characteristic feature of cystic fibrosis (CF). However, the impact of CF on intrauterine growth is unclear. We…”
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Combination antifungal therapy for Scedosporium species in cystic fibrosis
Published in Pediatric pulmonology (01-08-2020)“…Objective To evaluate safety and efficacy of oral posaconazole and terbinafine for Lomentospora prolificans and Scedosporium apiospermum in children with…”
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