PT  - JOURNAL ARTICLE
AU  - Lauren S Mott
AU  - Judy Park
AU  - Conor P Murray
AU  - Catherine L Gangell
AU  - Nicholas H de Klerk
AU  - Philip J Robinson
AU  - Colin F Robertson
AU  - Sarath C Ranganathan
AU  - Peter D Sly
AU  - Stephen M Stick
AU  - on behalf of AREST CF
TI  - Progression of early structural lung disease in young children with cystic fibrosis assessed using CT
AID  - 10.1136/thoraxjnl-2011-200912
DP  - 2012 Jun 01
TA  - Thorax
PG  - 509--516
VI  - 67
IP  - 6
4099  - http://thorax.bmj.com/content/67/6/509.short
4100  - http://thorax.bmj.com/content/67/6/509.full
SO  - Thorax2012 Jun 01; 67
AB  - Background Cross-sectional studies implicate neutrophilic inflammation and pulmonary infection as risk factors for early structural lung disease in infants and young children with cystic fibrosis (CF). However, the longitudinal progression in a newborn screened population has not been investigated.Aim To determine whether early CF structural lung disease persists and progresses over 1 year and to identify factors associated with radiological persistence and progression.Methods 143 children aged 0.2–6.5 years with CF from a newborn screened population contributed 444 limited slice annual chest CT scans for analysis that were scored for bronchiectasis and air trapping and analysed as paired scans 1 year apart. Logistic and linear regression models, using generalised estimating equations to account for multiple measures, determined associations between persistence and progression over 1 year and age, sex, severe cystic fibrosis transmembrane regulator (CFTR) genotype, pancreatic sufficiency, current respiratory symptoms, and neutrophilic inflammation and infection measured by bronchoalveolar lavage.Results Once detected, bronchiectasis persisted in 98/133 paired scans (74%) and air trapping in 178/220 (81%). The extent of bronchiectasis increased in 139/227 (63%) of paired scans and air trapping in 121/264 (47%). Radiological progression of bronchiectasis and air trapping was associated with severe CFTR genotype, worsening neutrophilic inflammation and pulmonary infection.Discussion CT-detected structural lung disease identified in infants and young children with CF persists and progresses over 1 year in most cases, with deteriorating structural lung disease associated with worsening inflammation and pulmonary infection. Early intervention is required to prevent or arrest the progression of structural lung disease in young children with CF.