Reproducibility of spirometry during cystic fibrosis pulmonary exacerbations.

OBJECTIVES: To compare the within day variation of spirometry between hospital admission, discharge, and outpatient follow up among children with cystic fibrosis (CF) hospitalized for a pulmonary exacerbation. HYPOTHESIS: Within day variation of spirometry will be greater at hospital admission than at hospital discharge or outpatient follow up. METHODS: We performed a retrospective review of spirometry data for all patients with CF >or=6 years old admitted to our pediatric CF center for a pulmonary exacerbation in 2004 or 2005. For patients who had previously performed spirometry successfully, measurements were used from one admission only during 2004-2005 if the spirometry occurred within 3 days of hospital admission, 3 days of discharge, or at a follow up clinic visit when well. We compared the within day coefficients of variation (CV) for FVC, FEV(1), and FEF(25-75) between time points using the Wilcoxon signed rank-test. We also determined the change in spirometry that is likely to be beyond measurement variability during inpatient treatment of a pulmonary exacerbation. RESULTS: Spirometry data were available from 40 subjects at admission and follow up and 35 at hospital discharge. There was no significant difference in CV at admission, discharge, and follow up for FVC, FEV(1), or FEF(25-75). The mean (SD) CV was 3.1% (2.7) for FVC, 3.2% (2.1) for FEV(1), and 9.7% (7.0) for FEF(25-75) at admission, 2.8% (2.2) for FVC, 3.1% (2.1) for FEV(1), and 8.1% (6.7) for FEF(25-75) at discharge, and 2.7% (1.7) for FVC, 2.8% (2.0) for FEV(1), and 8.4% (7.8) for FEF(25-75) at follow up. These are similar to previous reports of outpatients with CF. The improvement in spirometry that exceeded measurement variability for our cohort was 80 ml for FVC, 70 ml for FEV(1), and 220 ml/sec for FEF(25-75). CONCLUSIONS: The presence of an acute pulmonary exacerbation in children and adolescents with CF does not substantially contribute to the within day variation in spirometry. Within day variation of spirometry for children with CF during pulmonary exacerbations is similar to previously reported values from clinically stable CF patients.

Early radiographic and clinical features associated with bronchiectasis in children.

Bronchiectasis among children living in developing regions is associated with respiratory infections during early childhood, but specific risk factors that precede childhood bronchiectasis are not fully characterized. We hypothesized that severe respiratory syncytial viral (RSV) infection in infancy would increase the risk of bronchiectasis among Alaska Native children in rural Alaska. This was a follow-up cohort study of a 1993-1996 case-control study of RSV-hospitalized case patients and their controls. For each 5-8-year-old former case-patient and control subject, we reviewed medical records, interviewed parents, performed physical examinations and spirometry, collected sera, and analyzed all historical chest radiographs. Ten (11%) RSV cases and 10 (9%) controls had radiographic evidence of bronchiectasis. The mean age at radiographic diagnosis of bronchiectasis was 3.3 years (range, 1.2-6.1 years). Children were more likely to develop bronchiectasis if their chest radiographs, when they were < 2 years of age, showed lung parenchymal densities (RR = 3.9, P < 0.013), persistent parenchymal densities > 6 months' duration (RR = 3.0, P = 0.02), or infiltrates on multiple episodes (test for trend, P = 0.003). Radiographic features of hyperinflation and atelectasis among children < 2 years old were not associated with eventual bronchiectasis. A single severe infection with RSV alone did not predispose Alaska Native infants to bronchiectasis. Childhood bronchiectasis was associated with lung and hence airway injury, manifested on radiographs by parenchymal densities or "pneumonia" rather than by hyperinflation or atelectasis.