A5071474299- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Tracheal and airway disorders
- Pediatric health and respiratory diseases
- Respiratory viral infections research
- Asthma and respiratory diseases
- Congenital Diaphragmatic Hernia Studies
- Genetic and Kidney Cyst Diseases
- Adolescent and Pediatric Healthcare
- Pleural and Pulmonary Diseases
- Antibiotic Resistance in Bacteria
- Dysphagia Assessment and Management
- Pediatric Hepatobiliary Diseases and Treatments
- Olfactory and Sensory Function Studies
- Childhood Cancer Survivors' Quality of Life
- Bacterial biofilms and quorum sensing
- Nematode management and characterization studies
- Immune Cell Function and Interaction
- Nosocomial Infections in ICU
- Antifungal resistance and susceptibility
- Infant Nutrition and Health
- Fetal and Pediatric Neurological Disorders
- Pneumonia and Respiratory Infections
- Congenital Ear and Nasal Anomalies
- T-cell and B-cell Immunology
British Columbia Children's Hospital
2015-2025
University of British Columbia
2015-2025
Alder Hey Children's Hospital
2021
University of Liverpool
2021
State Research Institute of Highly Pure Biopreparations
2019
American Thoracic Society
2018
Montreal Children's Hospital
2018
University of British Columbia Hospital
2017
Cystic Fibrosis Canada
2017
Duke Medical Center
2016
Ivacaftor (VX-770), a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, has been shown to improve lung function, pulmonary exacerbation rate, respiratory symptoms, and weight gain compared with placebo in patients aged 12 years or older G551D-CFTR mutation.This randomized, double-blind, placebo-controlled trial evaluated ivacaftor 6-11 mutation on at least one allele.Patients were randomly assigned receive administered orally 150 mg (n = 26) every hours for 48 weeks...
This is the second in a series of four papers updating European Cystic Fibrosis Society (ECFS) standards for care people with CF. paper focuses on establishing and maintaining health. The guidance produced using an evidence-based framework wide stakeholder engagement, including from CF community. Authors provided narrative description their topic statements, which were more directive. These statements reviewed by Delphi exercise, achieving good levels agreement group all statements....
The main symptoms of primary ciliary dyskinesia (PCD) are nasal rhinorrhea or blockage and moist-sounding cough. Diagnosis can be difficult is based on an abnormal beat frequency, accompanied by specific abnormalities the axoneme. It unknown whether determining pattern related to ultrastructural defects might help in diagnosis PCD.We sought determine frequency (CBF) associated with 5 common responsible for PCD.Nasal brushings were performed 56 children PCD. Ciliary movement was recorded...
<h3>BACKGROUND</h3> The aim of this study was to determine the relationship power and recovery stroke respiratory cilia using digital high speed video imaging. Beat frequency measurements made were also compared with those obtained photomultiplier modified photodiode techniques. <h3>METHOD</h3> Ciliated epithelium by brushing inferior nasal turbinate 20 healthy subjects. edges observed microscopy deviation during their relative path travelled measured. analysis photodiode. <h3>RESULTS</h3>...
OBJECTIVES:. To prospectively study infants with an inconclusive diagnosis of cystic fibrosis (CF) identified by newborn screening (NBS; "CF screen positive, diagnosis" [CFSPID]) for disease manifestations.METHODS:. Infants CFSPID and CF based on NBS from 8 centers were evaluated monitored. Genotype, phenotype, repeat sweat test, serum trypsinogen, microbiology data compared between subjects who did (CFSPID→CF) not (CFSPID→CFSPID) fulfill the criteria during first 3 years life.RESULTS:....
KIWI (NCT01705145) was a 24-week, single-arm, pharmacokinetics, safety, and efficacy study of ivacaftor in children aged 2 to 5 years with cystic fibrosis (CF) CFTR gating mutation. Here, we report the results KLIMB (NCT01946412), an 84-week, open-label extension KIWI.Children received age- weight-based dosages for 84 weeks. The primary outcome safety. Other outcomes included sweat chloride, growth parameters, measures pancreatic function.All 33 who completed enrolled KLIMB; 28 weeks...
We have been collecting Burkholderia species bacteria from patients with cystic fibrosis (CF) for the last 30 years. During this time, our understanding of their multispecies taxonomy and infection control has evolved substantially.To evaluate long-term (30 year) epidemiology clinical outcome in CF, fully define risks associated by each species.Isolates Burkholderia-positive (n=107) were speciated typed annually infected patient. Microbiological data evaluated thorough review patient charts,...
Human coronavirus (HCoV) accounts for 15–30% of common colds, but only one case report has described the effect a infection, that was asymptomatic, on human respiratory epithelium. The authors examined effects infection with HCoV ciliary structure and function in healthy volunteers infected by intranasal inoculation 229E. A further four were sham ultraviolet-inactivated virus. Immediately before (day 0) 3 days later 3), ciliated epithelium obtained brushing inferior nasal turbinate. Ciliary...
<b>Background:</b> There are very few data on normal ciliary beat frequency, pattern, and ultrastructure in healthy children adults. A study was undertaken to define structure, frequency pattern a paediatric young adult population. <b>Methods:</b> Ciliated epithelial samples were obtained from 76 volunteers aged 6 months 43 years by brushing the inferior nasal turbinate. Beating cilia recorded using digital high speed video camera which allowed analysis of frequency. Tissue fixed for...
Electron microscopy (EM) of ciliated epithelium is widely used to diagnose primary ciliary dyskinesia (PCD). Ciliary beat frequency (CBF) has been screen samples determine whether EM indicated. Beat pattern analysis advocated as an additional diagnostic test. Neither subject formal review.To the ability CBF and predict EM-diagnosed PCD.CBF calculation analysis, using high-speed video microscopy, were performed on nasal tissue from 371 patients consecutively referred Leicester Royal Infirmary...
New treatments are needed to improve the health of people with cystic fibrosis (CF). Reducing lung-damaging inflammation is likely be beneficial, but specific anti-inflammatory targets have not been identified. By combining cellular immunology a population-based genetic modifier study, we examined TLR5 as an target and gene in CF. Using two pairs human CF control airway epithelial cells, demonstrated that TLR5-flagellin interaction major mediator following exposure Pseudomonas aeruginosa. To...
Cystic fibrosis (CF) screen-positive infants with an inconclusive diagnosis (CFSPID) are in whom sweat testing and genetic analysis does not resolve a CF diagnosis. Lack of knowledge about the health outcome these children who require clinical follow-up challenges effective consultation. Early predictive biomarkers to delineate risk would allow more targeted approach children.Prospective, longitudinal, multicenter, Canada-wide cohort study positive-screened newborns 1 2 cystic transmembrane...
Background Inflammation and infection are major determinants of disease severity consequently, the quality life outcome for patients with cystic fibrosis (CF). Interleukin-1 beta (IL-1β) is a key inflammatory mediator. Secretion biologically active IL-1β involves inflammasome-mediated processing. Little known about contribution inflammasomes in CF disease. This study examines production bronchial epithelial cell lines human CF. Results Bronchial were found to produce negligible amounts basal...