Radiological and functional changes over 3 years in young children with cystic fibrosis

¹ Dept of Paediatric Pulmonology, University Medical Centre Utrecht, Utrecht, the Netherlands

^* To whom correspondence should be addressed. E-mail: s.terheggen{at}wkz.azu.nl.

	Abstract

The aim of this study was to evaluate airway disease progression assessed by chest radiology, expiratory interrupter resistance (Rint_exp), and spirometry in young children with CF over a 3-year interval.

Two chest radiographs combined with two Rint_exp measurements, in a 3 year interval, were performed in 21 preschool children (mean (SD) age 3.2 (0.9) years) and 30 school children with CF (mean (SD) age 7.2 (1.9) years). Chest radiographs were scored using five different CF scoring systems and Rint_exp measurements were expressed as height adjusted Z-scores. Spirometry was assessed in school children and results were expressed as percent predicted.

Chest radiograph scores worsened significantly over the 3 year period and there was a tendency towards more pronounced changes, especially for the Wisconsin score in preschool children. Most preschool and school children had Rint_exp Z-scores within normal range at start and follow-up, and annual change in Rint_exp Z-score was not significant. In school children only forced expiratory volume in 1 second as percentage of forced vital capacity (FEV₁%FVC) declined significantly during the study period.

In young children with CF, chest radiograph scores worsen significantly over time even while lung function remains stable.