Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis.

RATIONALE: After recent standardization of forced expiratory maneuvers for both infants and preschool children, longitudinal measurements are now possible from birth. OBJECTIVES: The aim of this study was to investigate the evolution of lung function during the first 6 years of life after a clinical diagnosis of cystic fibrosis (CF) in infancy in children with CF and in healthy control subjects. METHODS: The raised volume technique was used during infancy and incentive spirometry during the preschool years. MEASUREMENTS AND MAIN RESULTS: Forty-eight children with CF and 33 healthy control subjects had up to seven (median, 3) measurements. Over these early years, the diagnosis of CF itself accounted for a significant mean reduction of 7.5% (95% confidence interval, 0.9 - 13.6%) in FEV(0.75) and 15.1% (95% confidence interval, 3.6 - 25.3%) in FEF(25-75). Wheeze on auscultation, recent cough, and Pseudomonas aeruginosa (PsA) infection (even if apparently effectively treated) were all independently associated with further reductions in lung function. Premorbid lung function did not predict infection with PsA. CONCLUSIONS: This is the first study to describe physiologic measurements from infancy through the preschool years in subjects with CF and healthy control subjects, the understanding of which is critical for future intervention trials. Airflow obstruction in uncomplicated CF persists through the preschool years despite treatment, with PsA acquisition being associated with further deterioration in lung function, even when apparently eradicated. This suggests that new therapies are needed to treat the airflow obstruction of uncomplicated CF, and rigorous strategies to prevent PsA acquisition.

Randomised controlled trial of nasal continuous positive airways pressure (CPAP) in bronchiolitis.

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 oxygen by nasal prongs or face mask. The change in PCO2 was compared between the groups after 12 and 24 h. Secondary outcomes were change in capillary pH, respiratory rate, pulse rate and the need for invasive ventilatory support. RESULTS: 29 of 31 children completed the study. PCO2 after 12 h fell by 0.92 kPa in children treated with CPAP compared with a rise of 0.04 kPa in those on ST (p<0.015). If CPAP was used first, there was a significantly better reduction in PCO2 than if it was used second. There were no differences in secondary outcome measures. CPAP was well tolerated with no complications identified. CONCLUSIONS: This study suggests that CPAP compared with ST improves ventilation in children with bronchiolitis and hypercapnoea.

Spirometry in the pre-school age group.

Spirometry is a widely used lung-function test in school-aged children and adults. It can also be used to test lung function in the pre-school population, provided that they are tested in a child-friendly setting, by experienced paediatric-orientated personnel and in a way that is congruent with their development. Accurate results can be obtained provided care and attention are paid to applying quality-control criteria suitable for this age group. The biggest challenge is to make the testing fun.