Progression of early structural lung disease in young children with cystic fibrosis assessed using CT.
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.


OBJECTIVE
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.


CONCLUSIONS
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.
Telethon Institute for Child Health Research and Centre for Child Health Research, University of Western Australia, Perth, Australia.
Lauren S
LS
Mott
Judy
J
Park
Conor P
CP
Murray
Catherine L
CL
Gangell
Nicholas H
NH
de Klerk
Philip J
PJ
Robinson
Colin F
CF
Robertson
Sarath C
SC
Ranganathan
Peter D
PD
Sly
Stephen M
SM
Stick
D001987:Bronchiectasis / Q000000981:diagnostic imaging / Q000209:etiology*; D018893:Bronchoalveolar Lavage; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000235:genetics / Q000473:pathology / Q000503:physiopathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics*; D018450:Disease Progression; D061345:Early Medical Intervention; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007249:Inflammation / Q000000981:diagnostic imaging; D016014:Linear Models; D016015:Logistic Models; D008137:Longitudinal Studies; D008297:Male; D015997:Neonatal Screening; D009504:Neutrophils; D012123:Pulmonary Ventilation; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012307:Risk Factors; D014057:Tomography, X-Ray Computed
D019005:Cystic Fibrosis Transmembrane Conductance Regulator