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Many epithelia, including the superficial epithelia of the airways, are thought to secrete "volume sensors," which regulate the volume of the mucosal lining fluid. The epithelial Na(+) channel (ENaC) is often the rate limiting factor in fluid absorption, and must be cleaved by extracellular and/or intracellular proteases before it can conduct Na(+) and(More)
Mammalian airways are protected from infection by a thin film of airway surface liquid (ASL) which covers airway epithelial surfaces and acts as a lubricant to keep mucus from adhering to the epithelial surface. Precise regulation of ASL volume is essential for efficient mucus clearance and too great a reduction in ASL volume causes mucus dehydration and(More)
In patients with cystic fibrosis (CF), the isolation of filamentous fungi, in particular Aspergillus spp. in the respiratory secretions is a common occurrence. Most of these patients do not fulfil the clinical criteria for a diagnosis of allergic bronchopulmonary aspergillosis (ABPA). The clinical relevance of filamentous fungi and whether antifungal(More)
Filamentous fungi are commonly isolated from the respiratory tract of CF patients, but their clinical significance is uncertain and the reported incidence variable. We report on the degree of Aspergillus fumigatus airway colonization in a tertiary pediatric CF cohort, evaluate the sensitivity of routine clinical sampling at detecting A. fumigatus, and(More)
Since the publication of the European Respiratory Society Task Force report in 2008, significant new evidence has become available on the classification and management of preschool wheezing disorders. In this report, an international consensus group reviews this new evidence and proposes some modifications to the recommendations made in 2008. Specifically,(More)
INTRODUCTION Bronchiectasis is generally considered irreversible in the adult population, largely based on studies employing bronchography in cases with a significant clinical history. It is assumed, that the same is true for children. Few studies have examined the natural history of bronchiectasis in children and diagnostic criteria on high-resolution(More)
Maternal smoking during pregnancy increases childhood asthma risk, but health effects in children of nonsmoking mothers passively exposed to tobacco smoke during pregnancy are unclear. We examined the association of maternal passive smoking during pregnancy and wheeze in children aged ≤2 years.Individual data of 27 993 mother-child pairs from 15 European(More)
Airway liquid content and insufficient absorptive airway ion transport at birth are potentially important factors in the development and severity of neonatal respiratory disease. The role of deficient absorptive airway ion transport in the development of chronic lung disease of prematurity is unknown. Additionally, lung inflammatory mediators modulate(More)
At birth the mammalian airway switches from liquid secretion to absorption, an important mechanism in lung liquid clearance. Airway ion transport was examined on the first postnatal day in 38 moderately preterm infants (29-36 weeks gestation). The absorptive airway ion transport capacity was well developed regardless of respiratory condition and there was(More)
The prevalence and clinical significance of specific polysaccharide antibody deficiency (SAD) in children are poorly understood. The authors sought to determine the prevalence of SAD in children with chronic wet cough, through a retrospective study of all children with chronic wet cough attending our tertiary respiratory clinic over a 12-month period.(More)