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AIMS To compare continuous positive airways pressure (CPAP) with standard treatment (ST) in the management of bronchiolitis. METHODS Children <1 year of age with bronchiolitis and capillary PCO2 >6 kPa were recruited and randomised to CPAP or ST and then crossed over to the alternative treatment after 12 h. ST was intravenous fluids and supplemental(More)
RATIONALE Newborn screening (NBS) for cystic fibrosis (CF) allows early intervention. Design of randomised controlled trials (RCT) is currently impeded by uncertainty regarding evolution of lung function, an important trial end point in such infants. OBJECTIVE To assess changes in pulmonary function during the first year of life in CF NBS infants. (More)
BACKGROUND Long-term benefits of newborn screening (NBS) for cystic fibrosis (CF) have been established with respect to nutritional status, but effects on pulmonary health remain unclear. HYPOTHESIS With early diagnosis and commencement of standardised treatment, lung function at ∼3 months of age is normal in NBS infants with CF. METHODS Lung clearance(More)
INTRODUCTION Death in childhood from cystic fibrosis (CF) is now an uncommon event in the U.K. We wished to assess the circumstances surrounding deaths (and lung transplantation) in the modern era of CF care. METHODS A retrospective review was carried out pooling data from two large paediatric specialist CF units in London for the 10-year period 2000-2009(More)
Allergic bronchopulmonary aspergillosis (ABPA) is an important complication of cystic fibrosis. It is a hypersensitivity reaction to Aspergillus fumigatus, leading to a Th2 CD4 response mediated by the release of specific IgE. If ABPA is not treated early, it can cause severe impairment in lung function and long-term lung damage. Hence, early recognition(More)
RATIONALE Sensitive outcome measures applicable in different centres to quantify and track early pulmonary abnormalities in infants with cystic fibrosis (CF) are needed both for clinical care and interventional trials. Chest CT has been advocated as such a measure yet there is no validated scoring system in infants. OBJECTIVES The objectives of this study(More)
RATIONALE With increasing use of infant pulmonary function tests (IPFTs) in both clinical and research studies, appropriate interpretation of results is essential. OBJECTIVES To investigate the potential bias associated with "normalising" IPF by expressing results as a ratio of body size and to develop reference ranges for tidal breathing parameters,(More)
PURPOSE OF REVIEW Lung disease begins early in life in cystic fibrosis (CF), yet our understanding of CF lung abnormalities in the first years of life remains limited. By facilitating earlier diagnosis, newborn screening for CF provides the opportunity to understand and characterize presymptomatic lung disease in infants and young children. This could lead(More)
BACKGROUND Information regarding recruitment of infants to research studies following the diagnosis of cystic fibrosis (CF) via newborn screening (NBS) is not currently available. This study aimed to assess parental attitudes and the feasibility of recruiting and retaining both NBS infants with CF and healthy control infants to a longitudinal, observational(More)
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