A5091903544- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Tracheal and airway disorders
- Asthma and respiratory diseases
- Nematode management and characterization studies
- Antifungal resistance and susceptibility
- Inhalation and Respiratory Drug Delivery
- Child Nutrition and Feeding Issues
- Dysphagia Assessment and Management
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Respiratory viral infections research
- Health Systems, Economic Evaluations, Quality of Life
- Pharmaceutical studies and practices
- Congenital Ear and Nasal Anomalies
- Esophageal and GI Pathology
- Adolescent and Pediatric Healthcare
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Pediatric health and respiratory diseases
- Respiratory Support and Mechanisms
- Childhood Cancer Survivors' Quality of Life
- Gut microbiota and health
- Genomics and Rare Diseases
- Delphi Technique in Research
- Fungal Infections and Studies
- Pneumonia and Respiratory Infections
Royal Brompton Hospital
2016-2025
Imperial College London
2016-2025
Harefield Hospital
2024
Guy's and St Thomas' NHS Foundation Trust
2023-2024
Lung Institute
2020-2023
Royal Brompton & Harefield NHS Foundation Trust
2012-2022
St Mary's Hospital
2022
Cooperative Trials Group for Neuro-Oncology
2022
RELX Group (United States)
2022
Footscray Hospital
2022
Lung delivery of plasmid DNA encoding the CFTR gene complexed with a cationic liposome is potential treatment option for patients cystic fibrosis. We aimed to assess efficacy non-viral therapy in fibrosis.We did this randomised, double-blind, placebo-controlled, phase 2b trial two fibrosis centres recruited from 18 sites UK. Patients (aged ≥12 years) forced expiratory volume 1 s (FEV1) 50-90% predicted and any combination mutations, were randomly assigned, via computer-based randomisation...
<b>Objectives</b> To evaluate the survival of patients with cystic fibrosis whose lung function has deteriorated to a forced expiratory volume in one second (FEV<sub>1</sub>) below 30% predicted recent treatment era and explore factors associated any change survival. <b>Design </b>Cohort study. <b>Setting</b> Adult unit London. <b>Participants</b> 276 (147 (53%) male) FEV<sub>1</sub> was first observed be less than between 1 January 1990 31 December 2003. <b>Main outcome measure</b> Survival...
Objective Cystic fibrosis associated liver disease (CFLD) is the third largest cause of mortality in CF. Our aim was to define burden CFLD UK using national registry data and identify risk factors for progressive disease. Methods A longitudinal population-based cohort study conducted. Cases were defined as all patients with identified from CF Registry, 2008–2013 (n = 3417). Denominator derived entire Registry. The characterised. Regression analysis undertaken cirrhosis progression. Results...
The prevalence of fungal disease in cystic fibrosis (CF) and non-CF bronchiectasis is increasing the clinical spectrum widening. Poor sensitivity a lack standard diagnostic criteria renders interpretation culture results challenging. In order to develop effective management strategies, more accurate comprehensive understanding airways microbiome required. study aimed use DNA sequences from sputum assess load diversity fungi adults with CF bronchiectasis.Next generation sequencing ITS2 region...
BackgroundPrescribers have an increasing range of inhaled antimicrobial formulations to choose from when prescribing both eradication and chronic suppression regimens in cystic fibrosis (CF). This study aimed investigate the decision-making process behind antimicrobials for Pseudomonas aeruginosa infections.MethodsA questionnaire was developed using Microsoft Forms then forwarded 57 Principal Investigators (PIs), at each CF centres within European Cystic Fibrosis Society-Clinical Trials...
For at least two years our attention has been attracted to the possibility that there might be more than one kind of rickets. We were led think this as result consideration certain peculiar manifestations disease and associations with other diseases. The facts are follows: Rickets occurs great frequency in premature children, even when breast fed. It seems affect head extremities or ribs. In a group cases rickets shows an especial tendency involve shafts long bones declaring itself...
Aspergillus fumigatus ( Af ) is a major mould pathogen found ubiquitously in the air. It commonly infects airways of people with cystic fibrosis (CF) leading to bronchitis or allergic bronchopulmonary aspergillosis. Resident alveolar macrophages and recruited neutrophils are important first lines defence for clearance lung. However, their contribution inflammatory phenotype CF during infection not well understood. Here, utilising CFTR deficient mice we describe hyperinflammatory both acute...
The clinical course of patients with cystic fibrosis (CF) is variable and probably determined by many interacting factors. We aimed to examine the influence early social factors on long-term survival. A case–control study adult CF was used compare survivors (aged ≥40 yrs) who died before reaching 30 yrs age. Each case (n = 78) matched birth date at least one control 152), after exclusion “late diagnosis” patients. Probability-weighted logistic regression models were identify influences...
Background Cystic fibrosis (CF) is a chronic, life-limiting disease caused by mutations in the CF transmembrane conductance regulator ( CFTR ) gene leading to abnormal airway surface ion transport, chronic lung infections, inflammation and eventual respiratory failure. With exception of small-molecule potentiator, ivacaftor (Kalydeco ® , Vertex Pharmaceuticals, Boston, MA, USA), which suitable for small proportion patients, there are no licensed therapies targeting basic defect. The UK...
The best clinical practice to investigate aerobic fitness includes measurements obtained during cardiopulmonary exercise testing (CPET), however it remains an underutilised measure in cystic fibrosis (CF). To this further, different methods of quantifying capacity CF are required. possibility that measuring physical activity (PA) by a portable accelerometer could be used assess the state and added among CPET surrogates has not been investigated. aim study was examine relationship between PA...