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. 2017 Dec 11;17(1):188.
doi: 10.1186/s12890-017-0546-8.

Pulmonary exacerbations and clinical outcomes in a longitudinal cohort of infants and preschool children with cystic fibrosis

Jordana E Hoppe  1 , Brandie D Wagner  2   3 , Scott D Sagel  2 , Frank J Accurso  2 , Edith T Zemanick  2
Affiliations

Pulmonary exacerbations and clinical outcomes in a longitudinal cohort of infants and preschool children with cystic fibrosis

Jordana E Hoppe et al. BMC Pulm Med. .

Abstract

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.

Keywords: Antibiotics; Cystic fibrosis; Lung function; Pulmonary exacerbation.

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Conflict of interest statement

Ethics approval and consent to participate

The Colorado Multiple Institutional Review Board approved the study (COMIRB #: 07–0839). Written informed consent was obtained for all patients. Consent to participate for all subjects under 16 years old was given by a parent or legal guardian. HIPAA standards were maintained during the study.

Consent for publication

Not applicable

Competing interests

Dr. Zemanick reports grants from Cystic Fibrosis Foundation, during the conduct of the study and grants from NIH/ NHLBI, grants from Cystic Fibrosis Foundation outside the submitted work. Dr. Hoppe reports grants from Cystic Fibrosis Foundation, during the conduct of the study, outside the submitted work. Dr. Sagel reports grants from National Institutes of Health, grants from Cystic Fibrosis Foundation, outside the submitted work. Dr. Accurso and Dr. Wagner have nothing to disclose.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Relationship between chest radiograph score at enrollment and study completion and number of exacerbations: A loess curve (solid line) was used to display the association and is plotted with a 95% confidence interval (shaded band). Chest radiograph scores at enrollment and study completion were associated with the frequency of pulmonary exacerbations, indicating that those with better chest radiograph scores had fewer exacerbations. The Spearman’s coefficient suggests a moderate association
Fig. 2
Fig. 2
Chest x-ray scores at enrollment and study completion: Chest x-ray scores for individual subjects are depicted. Points and whiskers at each point correspond to means and standard deviation, respectively
Fig. 3
Fig. 3
Relationship between lung function at 7 years and number of exacerbations: A loess curve for FEV1 (blue line) and FVC (red line) was used to display the association and is plotted with a 95% confidence interval (shaded bands). Higher FVC was associated with increased exacerbation frequency whereas no association was seen between FEV1 and exacerbation frequency
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References

    1. Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005;352:1992–2001. doi: 10.1056/NEJMra043184. - DOI - PubMed
    1. Foundation CF. Cystic Fibrosis Foundation patient registry 2011 annual data report to the center directors: Bethesda, Martland; 2012.
    1. 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. doi: 10.1002/ppul.21374. - DOI - PubMed
    1. 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;151:1075–1082. - PubMed
    1. Armstrong DS, Hook SM, Jamsen KM, et al. Lower airway inflammation in infants with cystic fibrosis detected by newborn screening. Pediatr Pulmonol. 2005;40:500–510. doi: 10.1002/ppul.20294. - DOI - PubMed

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