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Published on: July 16, 2020
Early bronchiectasis in cystic fibrosis detected by surveillance CT
Naveen Pillarisetti1, Barry Linnane, Sarath Ranganathan
1Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Victoria, Australia.
Insights
Early detection of cystic fibrosis lung disease in infants is possible with newborn screening. Comprehensive annual assessments, including CT scans, identified lung disease and bronchiectasis early, leading to adjusted management strategies.
Area of Science:
- Pediatric Pulmonology
- Medical Imaging in Cystic Fibrosis
Background:
- Cystic fibrosis (CF) lung disease onset is increasingly recognized in infancy.
- Newborn screening (NBS) facilitates early detection and intervention for CF.
- Early surveillance is crucial for managing this life-shortening genetic disorder.
Observation:
- Two children diagnosed with CF underwent annual comprehensive assessments from diagnosis.
- Assessments included lung function tests, limited-slice high-resolution CT scans, and bronchoalveolar lavage (BAL).
Findings:
- Early, aggressive surveillance detected significant lung disease and bronchiectasis within the first few years of life.
- The findings prompted a change in clinical management strategies.
- High-resolution CT scanning demonstrated clinical utility in preschool-aged children with CF.
Implications:
- CT scanning plays a vital role in the early detection of pulmonary complications in young children with CF.
- Proactive surveillance strategies can lead to timely interventions and potentially alter disease progression.
- This approach underscores the importance of integrating advanced imaging into early CF care protocols.
Abstract:
There is emerging evidence that cystic fibrosis lung disease begins early in infancy. Newborn screening allows early detection and surveillance of pulmonary disease and the possibility of early intervention in this life-shortening condition. We report two children with cystic fibrosis who underwent a comprehensive assessment from diagnosis that included measurement of lung function, limited-slice high-resolution CT and BAL performed annually. Early aggressive surveillance enabled significant lung disease and bronchiectasis to be detected during the first few years of life and led to a change in management, highlighting a clinical role for CT scanning during the preschool years in children with cystic fibrosis.
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