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Bronchiectasis in Children: A Comparative Analysis of Cystic Fibrosis and Non-Cystic Fibrosis Etiologies Using the
Handan Kekeç1, Tuğba Şişmanlar Eyüboğlu1, Ayse Tana Aslan1
1Department of Pediatric Pulmonology, Gazi University Faculty of Medicine, Ankara, Türkiye
Insights
Childhood bronchiectasis (BE) shows different outcomes in cystic fibrosis (CF) and non-CF patients. Structured management improved CF patients, while non-CF BE requires standardized follow-up for better results.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Medical Imaging
Background:
- Childhood bronchiectasis (BE) is an under-recognized chronic lung disease characterized by irreversible bronchial widening, recurrent infections, and progressive lung damage.
- BE in children significantly impacts health, necessitating better understanding and management strategies.
Purpose of the Study:
- To compare clinical presentations and radiological severity in children with cystic fibrosis (CF) related BE versus non-CF BE.
- To examine the association between clinical features and radiological severity using the Bhalla scoring system.
Main Methods:
- Retrospective observational study of 157 children (0-18 years) with CT-confirmed BE.
- Data collected included demographics, clinical characteristics, Bhalla scores for severity, and pulmonary function tests.
- Patients were stratified into CF-BE and non-CF BE groups for comparative analysis.
Main Results:
- CF-BE patients presented earlier, had longer follow-up, and more hospitalizations than non-CF BE patients.
- Significant improvements in growth z-scores were observed in CF-BE but not non-CF BE patients.
- Severe Bhalla scores were more common in CF-BE; Bhalla scores correlated with FEV1 in univariate CF analysis but not in multivariable or non-CF analyses.
Conclusions:
- Distinct clinical trajectories exist between pediatric CF-BE and non-CF BE.
- Structured management appears beneficial for CF-BE, while non-CF BE necessitates standardized follow-up protocols.
- The Bhalla score is a supplementary tool for assessing structural lung disease in BE, not an independent predictor of lung function.
Background:
Childhood bronchiectasis (BE) is a chronic lung condition that remains under recognized, marked by irreversible widening of the bronchi, frequent respiratory infections, a persistent wet cough, and progressive lung damage, often leading to significant health burdens.
Aims:
To evaluate children with cystic fibrosis (CF) and non-CF BE and to examine the association between clinical presentation and radiological severity of BE using the Bhalla scoring system.
Study Design:
Retrospective observational study.
Methods:
Children aged 0-18 years with a CT-confirmed diagnosis of BE were enrolled. Data on demographics, clinical characteristics, and imaging results were collected retrospectively from medical records. The Bhalla score was used to assess BE severity. Patients were categorized into CF-related and non-CF BE groups. The two groups were compared with respect to clinical features, growth z-scores, hospitalization frequency, and pulmonary function test outcomes.
Results:
A total of 157 patients were analyzed. Among them, CF accounted for 23.6% of cases, and while the leading causes in the non-CF group were post-infectious BE (28%), immunodeficiency (19.8%), and primary ciliary dyskinesia (12.8%). The CF group presented at an earlier age, had a longer follow-up period, and experienced more frequent hospitalizations (p < 0.001). In the CF group, weight, height, and body mass index z-scores significantly improved from the initial to the final assessment (p = 0.010, p = 0.006, and p = 0.026, respectively), whereas no such improvement was observed in the non-CF group. Severe Bhalla scores were more frequently observed in the CF group (p < 0.001). Among CF patients, Bhalla scores showed a strong correlation with forced expiratory volume in one second (FEV1) in univariate analysis (r = 0.846, p < 0.001), though this was not significant in multivariable analysis (p = 0.434). In the non-CF group, there was no correlation between Bhalla scores and final FEV1 values (p = 0.148, r = 0.212).
Conclusion:
The results underscore distinct clinical trajectories between CF and non-CF BE in children. Improvements in CF patients suggest the effectiveness of structured clinical management, whereas inconsistent outcomes in non-CF patients point to the need for standardized follow-up protocols. While the Bhalla score may indicate the extent of structural lung disease in CF, it does not independently predict lung function, and therefore should be used as a supplementary, not solitary, measure of disease severity.
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