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.
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.
Authors: Nicole Mayer-Hamblett; Margaret Kloster; Margaret Rosenfeld; Ronald L Gibson; George Z Retsch-Bogart; Julia Emerson; Valeria Thompson; Bonnie W Ramsey Journal: Clin Infect Dis Date: 2015-05-13 Impact factor: 9.079
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Authors: Jessica E Pittman; Elizabeth H Calloway; Michelle Kiser; John Yeatts; Stephanie D Davis; Mitchell L Drumm; Michael S Schechter; Margaret W Leigh; Mary Emond; Annelies Van Rie; Michael R Knowles Journal: Pediatr Pulmonol Date: 2010-12-30
Authors: Margaret Rosenfeld; Philip M Farrell; Margaret Kloster; Jonathan O Swanson; Thuy Vu; Lyndia Brumback; James D Acton; Robert G Castile; Andrew A Colin; Carol K Conrad; Meeghan A Hart; Gwendolyn S Kerby; Peter W Hiatt; Peter J Mogayzel; Robin C Johnson; Stephanie D Davis Journal: Eur Respir J Date: 2013-05-30 Impact factor: 16.671
Authors: Drucy Borowitz; Karen A Robinson; Margaret Rosenfeld; Stephanie D Davis; Kathryn A Sabadosa; Stephanie L Spear; Suzanne H Michel; Richard B Parad; Terry B White; Philip M Farrell; Bruce C Marshall; Frank J Accurso Journal: J Pediatr Date: 2009-12 Impact factor: 4.406