Background: Cystic fibrosis (CF) is one of the most common genetic disorders in children. CF patients are susceptible to chronic lung infections and malabsorption. Although patient longevity is increased by multidisciplinary care, patients still suffer from respiratory failure and low quality of life. In this situation, CF patients tend to use comple...

Nebulized antibiotics are being used increasingly in children with cystic fibrosis. We assessed the effect of nebulized antibiotic solutions of varying tonicity on lung function in 12 children aged 5-15 yrs with cystic fibrosis. Baseline forced expiratory volume in one second and (FEV1) was measured, followed by a single nebulization of normal saline (272 mosmol.kg-1), tobramycin (248 mosmol.kg...

Objectives: BEAT CF (Bayesian Evidence Adaptive Treatment of Cystic Fibrosis) is a study platform implemented in Australian cystic fibrosis centres. The core protocol aims to identify the comparative effectiveness interventions (e.g., intravenous antibiotics) routinely used management pulmonary exacerbations requiring intensive therapy (PERIT) children and adults with CF, respect improvements l...

A method that maximises the yield of viable enterocytes has been developed for the isolation of enterocytes from human jejunal biopsy specimens. These enterocytes have been used to study the values of intracellular free calcium and the rises in adenosine 3'5'-cyclic monophosphate (cAMP) induced by secretagogues in normal and cystic fibrosis cells. Basal intracellular free calcium of cystic fibr...

In 10 children with cystic fibrosis and persisting steatorrhoea, supplementation with taurine (30-40 mg/kg/day) was given for two months as an adjunct to the usual pancreatic enzyme treatment. A three day fat and energy balance was performed in patients with cystic fibrosis, before and after the supplementation, and in seven healthy controls who did not receive taurine. Faecal fat was measured ...

BACKGROUND Disease severity in patients with cystic fibrosis shows marked variability. Attempts to explain this phenotypic heterogeneity on the basis of CFTR genotype have had limited success. A study was undertaken to test the hypothesis that naturally occurring variants of the pro-inflammatory cytokine tumour necrosis factor alpha (TNF-alpha) and the detoxifying enzyme glutathione S-transfera...