Published ahead of print on April 10, 2008, doi:10.1164/rccm.200710-1599OC Am. J. Respir. Crit. Care Med., Volume 178, Number 1, July 2008, 42-49 A more recent version of this article appeared on July 1, 2008
Submitted on October 30, 2007 Lung Function from Infancy to the Preschool Years Following Clinical Diagnosis of Cystic FibrosisWanda J Kozlowska1*,1 Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, UCL Institute of Child Health, London, United Kingdom, 2 Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom, 3 Centre for Peadiatric Epidemiology and Biostatistics, UCL Institute of Child Health, London, United Kingdom, 4 Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, UCL Institute of Child Health, London, United Kingdom; Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 5 Department of Respiratory Paediatrics, Barts and The London Children's Hospital, London, United Kingdom, 6 Department of Child Health, Kings College Hospital, London, United Kingdom, 7 Department of Paediatrics, Royal Children's Hospital Melbourne, Murdoch Children's Research Institute and Deartment of Paediatrics, University of Melbourne, Melbourne, Australia, 8 Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, UCL Institute of Child Health, London, United Kingdom; Centre for Peadiatric Epidemiology and Biostatistics, UCL Institute of Child Health, London, United Kingdom, 9 Department of Child Health, University Hospital Lewisham, London, United Kingdom, 10 Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom * To whom correspondence should be addressed. E-mail: W.Kozlowska{at}ich.ucl.ac.uk.
Rationale: Following recent standardization of forced expiratory maneuvers for both infants and preschool children, longitudinal measurements are now possible from birth. The AIM of this study was to investigate the evolution of lung function during the 1st six years of life following a clinical diagnosis of cystic fibrosis (CF) in infancy and in healthy controls. Measurements and Main Results: The raised volume technique was used during infancy and incentive spirometry during the preschool years. Forty-eight children with CF and 33 healthy controls had up to 7 (median 3) measurements. Over these early years, the diagnosis of CF itself accounted for a significant mean [95% CI] reduction of 7.5% [0.9, 13.6%] in FEV0.75 and 15.1% [3.6, 25.3%] in FEF25-75. 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 CF and healthy controls, 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. Key words: Spirometry, Pseudomonas aeruginosa, infant, preschool child
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