Prospective evaluation of respiratory exacerbations in children with cystic fibrosis from newborn screening to 5 years of age.
| Autor: | Byrnes CA; Department of Paediatrics, University of Auckland, , Auckland, New Zealand. c.byrnes@auckland.ac.nz, Vidmar S, Cheney JL, Carlin JB, Armstrong DS, Cooper PJ, Grimwood K, Moodie M, Robertson CF, Rosenfeld M, Tiddens HA, Wainwright CE |
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| Jazyk: | angličtina |
| Zdroj: | Thorax [Thorax] 2013 Jul; Vol. 68 (7), pp. 643-51. Date of Electronic Publication: 2013 Jan 23. |
| DOI: | 10.1136/thoraxjnl-2012-202342 |
| Abstrakt: | Background: Newborn screening allows novel treatments for cystic fibrosis (CF) to be trialled in early childhood before irreversible lung injury occurs. As respiratory exacerbations are a potential trial outcome variable, we determined their rate, duration and clinical features in preschool children with CF; and whether they were associated with growth, lung structure and function at age 5 years. Methods: Respiratory exacerbations were recorded prospectively in Australasian CF Bronchoalveolar Lavage trial subjects from enrolment after newborn screening to age 5 years, when all participants underwent clinical assessment, chest CT scans and spirometry. Results: 168 children (88 boys) experienced 2080 exacerbations, at an average rate of 3.66 exacerbations per person-year; 80.1% were community managed and 19.9% required hospital admission. There was an average increase in exacerbation rate of 9% (95% CI 4% to 14%; p<0.001) per year of age. Exacerbation rate differed by site (p<0.001) and was 26% lower (95% CI 12% to 38%) in children receiving 12 months of prophylactic antibiotics. The rate of exacerbations in the first 2 years was associated with reduced forced expiratory volume in 1 s z scores. Ever having a hospital-managed exacerbation was associated with bronchiectasis (OR 2.67, 95% CI 1.13 to 6.31) in chest CT scans, and lower weight z scores at 5 years of age (coefficient -0.39, 95% CI -0.74 to -0.05). Conclusions: Respiratory exacerbations in young children are markers for progressive CF lung disease and are potential trial outcome measures for novel treatments in this age group. |
| Databáze: | MEDLINE |
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