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Pulmonary exacerbations and clinical outcomes in a longitudinal cohort of infants and preschool children with cystic fibrosis
Copyright policy )Pulmonary exacerbations and clinical outcomes in a longitudinal cohort of infants and preschool children with cystic fibrosis
Background Pulmonary exacerbations (PEx) in school aged children and adults with cystic fibrosis (CF) lead to increased morbidity and lung function decline. However, the effect of exacerbations in young children with CF is not fully understood. We sought to characterize the frequency and clinical impact of PEx in a pilot study of infants and pre-school aged children with CF. Methods Thirty young children with CF [median (range) 1.5 years (0.2–4.9)] were prospectively followed for 2 years. Exacerbation frequency (hospitalizations and outpatient antibiotic use) was determined. Chest radiographs were performed at enrollment and study completion and assigned a Brasfield score. Lung function at age 7 years was assessed in a subset of children. The association between PEx frequency, chest radiograph score, and lung function was determined using Spearman correlation coefficients and corresponding 95% confidence intervals. Correlations with an absolute magnitude of 0.3 or greater were considered clinically significant. Results Over 2 years, participants experienced a median of two PEx (range 0–13). Chest radiograph scores at enrollment and study completion were inversely associated with PEx frequency (R = −0.48 and R = −0.44, respectively). The association between frequency of PEx and lung function [forced expiratory volume in 1 s (FEV1)] at age 7 years was small (R = 0.20). Higher forced vital capacity (FVC) at 7 years was associated with more frequent PEx during the study (R = 0.44). Conclusions Children with worse chest radiograph scores had more frequent PEx over the subsequent 2 years, suggesting a group of patients at higher risk for PEx. Frequent PEx in infants and young children with CF were not associated with lower FEV1 and FVC at 7 years, although spirometry in this age group may not be a sensitive marker of mild lung disease and disease progression. Electronic supplementary material The online version of this article (10.1186/s12890-017-0546-8) contains supplementary material, which is available to authorized users.
- University of Colorado System United States
Microsoft Academic Graph classification: Spirometry medicine.medical_specialty Vital capacity Exacerbation Cystic fibrosis FEV1/FVC ratio Internal medicine medicine Lung function medicine.diagnostic_test business.industry medicine.disease Confidence interval Chest radiograph business
Library of Congress Subject Headings: lcsh:RC705-779 lcsh:Diseases of the respiratory system
Male, Pulmonary and Respiratory Medicine, Cystic Fibrosis, Pilot Projects, Cohort Studies, Antibiotics, Humans, Pulmonary exacerbation, Lung, Infant, Lung function, Anti-Bacterial Agents, Respiratory Function Tests, Radiography, Child, Preschool, Disease Progression, Female, Research Article
Male, Pulmonary and Respiratory Medicine, Cystic Fibrosis, Pilot Projects, Cohort Studies, Antibiotics, Humans, Pulmonary exacerbation, Lung, Infant, Lung function, Anti-Bacterial Agents, Respiratory Function Tests, Radiography, Child, Preschool, Disease Progression, Female, Research Article
Microsoft Academic Graph classification: Spirometry medicine.medical_specialty Vital capacity Exacerbation Cystic fibrosis FEV1/FVC ratio Internal medicine medicine Lung function medicine.diagnostic_test business.industry medicine.disease Confidence interval Chest radiograph business
Library of Congress Subject Headings: lcsh:RC705-779 lcsh:Diseases of the respiratory system
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1. Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005;352:1992-2001.
2. Foundation CF. Cystic Fibrosis Foundation patient registry 2011 annual data report to the center directors: Bethesda, Martland; 2012.
3. Sanders DB, Bittner RC, Rosenfeld M, Redding GJ, Goss CH. Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis. Pediatr Pulmonol. 2011;46:393-400.
4. Khan TZ, Wagener JS, Bost T, Martinez J, Accurso FJ, Riches DW. Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med. 1995;151:1075-82.
5. Armstrong DS, Hook SM, Jamsen KM, et al. Lower airway inflammation in infants with cystic fibrosis detected by newborn screening. Pediatr Pulmonol. 2005;40:500-10.
6. Sly PD, Gangell CL, Chen L, et al. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013;368:1963-70.
7. Stick SM, Brennan S, Murray C, et al. Bronchiectasis in infants and preschool children diagnosed with cystic fibrosis after newborn screening. J Pediatr. 2009;155:623-8. e1
8. Mott LS, Park J, Murray CP, et al. Progression of early structural lung disease in young children with cystic fibrosis assessed using CT. Thorax. 2012;67: 509-16.
9. Wagener JS, Zemanick ET, Sontag MK. Newborn screening for cystic fibrosis. Curr Opin Pediatr. 2012;24:329-35. [OpenAIRE]
10. Cystic Fibrosis F, Borowitz D, Robinson KA, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155:S73-93.
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- National Institutes of Health (NIH)
- 5K23HL114883-04
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE
- National Institutes of Health (NIH)
- 5UL1TR001082-05
- NATIONAL CENTER FOR ADVANCING TRANSLATIONAL SCIENCES
- University of Colorado System United States
Background Pulmonary exacerbations (PEx) in school aged children and adults with cystic fibrosis (CF) lead to increased morbidity and lung function decline. However, the effect of exacerbations in young children with CF is not fully understood. We sought to characterize the frequency and clinical impact of PEx in a pilot study of infants and pre-school aged children with CF. Methods Thirty young children with CF [median (range) 1.5 years (0.2–4.9)] were prospectively followed for 2 years. Exacerbation frequency (hospitalizations and outpatient antibiotic use) was determined. Chest radiographs were performed at enrollment and study completion and assigned a Brasfield score. Lung function at age 7 years was assessed in a subset of children. The association between PEx frequency, chest radiograph score, and lung function was determined using Spearman correlation coefficients and corresponding 95% confidence intervals. Correlations with an absolute magnitude of 0.3 or greater were considered clinically significant. Results Over 2 years, participants experienced a median of two PEx (range 0–13). Chest radiograph scores at enrollment and study completion were inversely associated with PEx frequency (R = −0.48 and R = −0.44, respectively). The association between frequency of PEx and lung function [forced expiratory volume in 1 s (FEV1)] at age 7 years was small (R = 0.20). Higher forced vital capacity (FVC) at 7 years was associated with more frequent PEx during the study (R = 0.44). Conclusions Children with worse chest radiograph scores had more frequent PEx over the subsequent 2 years, suggesting a group of patients at higher risk for PEx. Frequent PEx in infants and young children with CF were not associated with lower FEV1 and FVC at 7 years, although spirometry in this age group may not be a sensitive marker of mild lung disease and disease progression. Electronic supplementary material The online version of this article (10.1186/s12890-017-0546-8) contains supplementary material, which is available to authorized users.