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The leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as well as development of innovative therapeutic interventions, have been compromised by the lack of a natural animal model. The utility of the CFTR-knockout mouse in studying(More)
The effects of hypoxia and intracellular acidification were examined on the mechanical properties of the non-pregnant and pregnant rat uterus. Isolated uteri were investigated during control conditions and in the presence of cyanide, to simulate hypoxia or the salts of weak acids and bases to change intracellular pH at constant external pH (pH 7.4). Both(More)
AIM Therapeutic aerosols are routinely used in the management of infant obstructive airways disease. Infants often become distressed during administration. The aim of this study was to determine the influence of distress and disease severity on the absorption of aerosolised drug in this age group. METHODS Fifteen infants, eight with resolving chronic lung(More)
AIM To investigate the longitudinal changes of interstitial and airways disease in resolving chronic lung disease of prematurity (CLD). METHODS Thirty three infants were studied between 35 and 40 weeks of postconceptional age, and then at three monthly intervals throughout their first year. Measurements of mean arterial oxygen saturation (MSaO2) and its(More)
BACKGROUND Newborn screening for cystic fibrosis (CF) relies on the measurement of immunoreactive trypsinogen (IRT) originating from the pancreas. The Norfolk, Suffolk and Cambridgeshire screening programme initially exploited the persistent increase in IRT seen in CF (IRT-IRT protocol) and later changed to include mutation analysis as a second tier test(More)
BACKGROUND Confidential enquiries into asthma deaths can identify inadequacies in medical management and factors which contribute to patients' death. AIMS To identify risk factors for paediatric asthma deaths over a 6-year period. METHODS Observational case-series study of paediatric asthma deaths between 2001-2006 in the UK Eastern Region. Hospital,(More)
BACKGROUND Bronchopulmonary dysplasia (BPD) is a chronic disorder associated with prematurity. Systemic steroids induce at least a temporary improvement in respiratory function, but are associated with adverse side effects. Inhaled steroids have fewer side effects. OBJECTIVES To determine if inhaled corticosteroids are effective in alleviating the(More)
BACKGROUND The cost effectiveness of inhaled TOBIR tobramycin nebuliser solution (TNS) in CF and chronic pulmonary Pseudomonas aeruginosa infection has been shown in US but not in European studies. METHODS An economic evaluation of TNS was undertaken in children and adults. Lung function and resource utilisation were recorded for 24 months before and(More)