[HTML][HTML] Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis …

J Barben, C Castellani, A Munck, JC Davies… - Journal of Cystic …, 2021 - Elsevier
Over the past two decades there has been considerable progress with the evaluation and
management of infants with an inconclusive diagnosis following Newborn Screening (NBS) …

Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening

SM Stick, S Brennan, C Murray, T Douglas… - The Journal of …, 2009 - Elsevier
OBJECTIVES: To determine the prevalence of bronchiectasis in young children with cystic
fibrosis (CF) diagnosed after newborn screening (NBS) and the relationship of …

Infection, inflammation, and lung function decline in infants with cystic fibrosis

N Pillarisetti, E Williamson, B Linnane… - American journal of …, 2011 - atsjournals.org
Rationale: Better understanding of evolution of lung function in infants with cystic fibrosis
(CF) and its association with pulmonary inflammation and infection is crucial in informing …

Lung function in infants with cystic fibrosis diagnosed by newborn screening

BM Linnane, GL Hall, G Nolan, S Brennan… - American journal of …, 2008 - atsjournals.org
Rationale: Progressive lung damage in cystic fibrosis (CF) starts in infancy, and early
detection may aid preventative strategies. Objectives: To measure lung function in infants …

Cystic fibrosis diagnosis in newborns, children, and adults

C Castellani, B Linnane, I Pranke… - … in respiratory and …, 2019 - thieme-connect.com
The diagnosis of cystic fibrosis (CF) has traditionally relied on the presence of clinical
features of the disease. Today, diagnosis through newborn screening (NBS) is becoming the …

[HTML][HTML] The expansion and performance of national newborn screening programmes for cystic fibrosis in Europe

J Barben, C Castellani, J Dankert-Roelse… - Journal of Cystic …, 2017 - Elsevier
Background Newborn screening (NBS) for cystic fibrosis (CF) is a well-established public
health strategy with international standards. The aim of this study was to provide an update …

Reduced 15-lipoxygenase 2 and lipoxin A4/leukotriene B4 ratio in children with cystic fibrosis

FC Ringholz, PJ Buchanan, DT Clarke… - European …, 2014 - Eur Respiratory Soc
Airway disease in cystic fibrosis (CF) is characterised by impaired mucociliary clearance,
persistent bacterial infection and neutrophilic inflammation. Lipoxin A4 (LXA4) initiates the …

Long-term safety and efficacy of tezacaftor–ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del …

PA Flume, RF Biner, DG Downey, C Brown… - The Lancet …, 2021 - thelancet.com
Background Tezacaftor–ivacaftor is an approved cystic fibrosis transmembrane conductance
regulator (CFTR) modulator shown to be efficacious and generally safe and well tolerated …

Longitudinal trends in real-world outcomes after initiation of ivacaftor. A cohort study from the cystic fibrosis registry of Ireland

L Kirwan, G Fletcher, M Harrington… - Annals of the …, 2019 - atsjournals.org
Rationale: Patient registries have the potential to collect and analyze high-quality
postauthorization data on new medicines. Objectives: We used cystic fibrosis (CF) registry …

[HTML][HTML] The microbial community of the cystic fibrosis airway is disrupted in early life

J Renwick, P McNally, B John, T DeSantis, B Linnane… - PloS one, 2014 - journals.plos.org
Background Molecular techniques have uncovered vast numbers of organisms in the cystic
fibrosis (CF) airways, the clinical significance of which is yet to be determined. The aim of …