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Non-cystic fibrosis bronchiectasis in children and adolescents: Neglected and emerging issues
Marco Poeta1, Marco Maglione1, Melissa Borrelli1
1Department of Translational Medical Sciences, Section of Pediatrics, Federico II University, Naples, Italy.
Insights
Pediatric non-cystic fibrosis (CF) bronchiectasis management is reviewed, focusing on novel assessment techniques and treatment advances. A new diagnostic algorithm is proposed to aid clinicians in managing this complex pediatric lung disease.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
Background:
- Pediatric non-cystic fibrosis (CF) bronchiectasis presents with airway inflammation and mucus issues, often leading to recurrent infections and cough.
- Current management guidelines largely rely on expert opinion or extrapolation from CF practices.
- There is a need for evidence-based strategies tailored to non-CF bronchiectasis in children.
Purpose of the Study:
- To review current knowledge and address open questions in managing pediatric non-CF bronchiectasis.
- To explore the role of technological advancements in lung disease assessment.
- To summarize recent developments in disease prevention and treatment, and propose a diagnostic algorithm.
Main Methods:
- Narrative review of existing literature.
- Analysis of novel chest imaging techniques and pulmonary function tests.
- Synthesis of information on disease prevention, treatment, and diagnostic approaches.
Main Results:
- Technological advances offer new possibilities for assessing lung disease in pediatric non-CF bronchiectasis.
- Novelties in prevention and treatment strategies are emerging.
- A proposed diagnostic algorithm can assist physicians in clinical decision-making.
Conclusions:
- Further research with larger cohorts and longer trials is crucial.
- Identifying new clinical and laboratory endpoints is needed to understand disease progression.
- Evidence-based management strategies are essential for improving outcomes in pediatric non-CF bronchiectasis.
Abstract:
Pediatric non-cystic fibrosis (CF) bronchiectasis is characterized by endobronchial suppuration, airway neutrophilic inflammation and poor mucus clearance and is associated with persistent productive cough due to recurrent airway infections. Most recommendations are based on expert opinion or extrapolated from CF practice. The present narrative review aims to address some issues on the management of children or adolescents with non CF-bronchiectasis that still require attention, and analyze what available literature offers to reply to open questions. We focused on the potential offered by technological advances on lung disease assessment through novel chest imaging techniques and new or old pulmonary function tests. We also summarized the main novelties in the disease prevention and treatment. Finally, a novel diagnostic algorithm is proposed, that might help physicians in the daily clinical decision-making process. Future directions for research on pediatric non-CF bronchiectasis should include larger study populations and longer prospective clinical trials, as well as new clinical and laboratory endpoints to determine the underlying mechanisms of lung disease progression and support the role of new and existing treatments.
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