Progression of early structural lung disease in young children with cystic fibrosis assessed using CT.
Thorax
; 67(6): 509-16, 2012 Jun.
Article
em En
| MEDLINE
| ID: mdl-22201161
ABSTRACT
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.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Bronquiectasia
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Regulador de Condutância Transmembrana em Fibrose Cística
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Ventilação Pulmonar
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Fibrose Cística
Tipo de estudo:
Etiology_studies
/
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Child, preschool
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Female
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Humans
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Infant
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Male
/
Newborn
Idioma:
En
Revista:
Thorax
Ano de publicação:
2012
Tipo de documento:
Article
País de afiliação:
Austrália