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Bronchiectasis secondary to primary immunodeficiency in children: longitudinal changes in structure and function.
Katerina Haidopoulou1, Alistair Calder, Alison Jones
1Fourth Department of Pediatrics, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Pediatric Pulmonology
|June 11, 2009
Summary
Bronchiectasis in children with primary immunodeficiency may not worsen after treatment. This study suggests that appropriate treatment could slow or prevent disease progression in pediatric patients.
Area of Science:
- Pediatric Pulmonology
- Immunology
- Radiology
Background:
- Primary immunodeficiency (PI) is a frequent cause of bronchiectasis in children.
- Bronchiectasis is often considered irreversible, but its progression post-treatment in PI patients is debated.
Purpose of the Study:
- To assess the progression of bronchiectasis in children diagnosed with primary immunodeficiency following the initiation of treatment.
Main Methods:
- Retrospective review of medical records for children with PI and confirmed bronchiectasis.
- Analysis of high-resolution computed tomography (HRCT) chest scans at diagnosis and follow-up (≥2 years interval) using the Bhalla score.
- Correlation of HRCT scores with spirometry (FEV1, FVC, FEV1:FVC) where available; statistical analysis using Wilcoxon signed rank test and Spearman's rank order correlation.
Main Results:
- Eighteen subjects were analyzed; PI diagnosis at median 3.4 years, bronchiectasis at 9.3 years.
- No significant difference in HRCT-chest Bhalla scores between baseline and follow-up (P=0.21).
- Significantly improved FEV1 (P<0.005) and FVC (P<0.05) at follow-up compared to baseline; no significant change in FEV1:FVC ratio. No correlation found between HRCT score changes and lung function changes.
Conclusions:
- Bronchiectasis associated with primary immunodeficiency in children is not invariably progressive.
- Findings suggest that timely and appropriate treatment may halt or reverse disease progression in pediatric bronchiectasis secondary to PI.
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