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Mucus plugging, air trapping, and bronchiectasis are important outcome measures in assessing progressive childhood cystic fibrosis lung disease
Author(s) -
Robinson Terry E.,
Goris Michael L.,
Moss Richard B.,
Tian Lu,
Kan Peiyi,
Yilma Mignote,
McCoy Karen S.,
Newman Beverley,
Jong Pim A.,
Long Frederick R.,
Brody Alan S.,
Behrje Rhett,
Yates Denise P.,
Cornfield David N.
Publication year - 2020
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.24646
Subject(s) - bronchiectasis , medicine , air trapping , interquartile range , spirometry , cystic fibrosis , vital capacity , lung volumes , lung , asthma , diffusing capacity , lung function
Objective To determine which outcome measures could detect early progression of disease in school‐age children with mild cystic fibrosis (CF) lung disease over a two‐year time interval utilizing chest computed tomography (CT) scores, quantitative CT air trapping (QAT), and spirometric measurements. Methods Thirty‐six school‐age children with mild CF lung disease (median [interquartile range] age 12 [3.7] years; percent predicted forced expiratory volume in 1 second (ppFEV 1 ) 99 [12.5]) were evaluated by serial spirometer‐controlled chest CT scans and spirometry at baseline, 3‐month, 1‐ and 2‐years. Results No significant changes were noted at 3‐month for any variable except for decreased ppFEV 1 . Mucus plugging score (MPS) and QAT A1 and A2 increased at 1‐ and 2‐years. The bronchiectasis score (BS), and total score (TS) were increased at 2‐year. All variables tested with the exception of bronchial wall thickness score, parenchymal score (PS), and ppFEV 1 , were consistent with longitudinal worsening of lung disease. Multivariate analysis revealed baseline PS, baseline TS, and 1‐year changes in BS and air trapping score were predictive of 2‐year changes in BS. Conclusions MPS and QAT A1‐A2 were the most sensitive indicators of progressive childhood CF lung disease. The 1‐year change in the bronchiectasis score had the most positive predictive power for 2‐year change in bronchiectasis.