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01.12.2017 | Research article | Ausgabe 1/2017 Open Access

BMC Pulmonary Medicine 1/2017

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

BMC Pulmonary Medicine > Ausgabe 1/2017
Jordana E. Hoppe, Brandie D. Wagner, Scott D. Sagel, Frank J. Accurso, Edith T. Zemanick
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Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1186/​s12890-017-0546-8) contains supplementary material, which is available to authorized users.



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.


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.


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).


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.
Additional file 1: Figure S1. Study Design: 30 subjects with CF were enrolled in the study. Study visits were performed at quarterly CF visits and at the time of an exacerbation over a two-year period. At each study visit, subjects underwent a history, physical, medication history and a culture obtained by oropharyngeal (OP) swab. Chest radiographs were done at study enrollment and at study completion (2 years) during periods of clinical stability and assigned a Brasfield score. (JPEG 27 kb)
Additional file 2: Table S1. Components of Chest X-ray Score for Individual Subjects: Components of the chest x-ray score at study enrollment and study completion are provided for each individual subject. (DOCX 32 kb)
Additional file 3: Table S2. Pulmonary function testing results: Absolute, z-score and percent predicted values of FEV1, FVC and FEV1/FVC are provided for each subject. (DOCX 14 kb)
Additional file 4: Figure S2. Relationship between lung function at 7 years and Wisconsin cough score at enrollment: A loess curve (grey line) was used to display the association and is plotted with a 95% confidence interval (shaded band). Higher FEV1 percent predicted at school age was associated with a lower Wisconsin cough score at enrollment. The corresponding linear association for the rank transformed variables is indicated by the Spearman’s rank-based correlation coefficient in the upper right hand corner. (PDF 83 kb)
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