There is increasing awareness of bronchiectasis in children and adolescents, a chronic pulmonary disorder associated with poor quality-of-life for the child/adolescent and their parents, recurrent exacerbations and costs to the family and health systems. Optimal treatment improves clinical outcomes. Several national guidelines exist, but there are no international guidelines.The European Respiratory Society (ERS) Task Force for the management of paediatric bronchiectasis sought to identify evidence-based management (investigation and treatment) strategies. It used the ERS standardised process that included a systematic review of the literature and application of the GRADE approach to define the quality of the evidence and level of recommendations.A multidisciplinary team of specialists in paediatric and adult respiratory medicine, infectious disease, physiotherapy, primary care, nursing, radiology, immunology, methodology, patient advocacy and parents of children/adolescents with bronchiectasis considered the most relevant clinical questions (for both clinicians and patients) related to managing paediatric bronchiectasis. Fourteen key clinical questions (7 “Patient, Intervention, Comparison, Outcome” [PICO] and 7 narrative) were generated. The outcomes for each PICO were decided by voting by the panel and parent advisory group.This guideline addresses the definition, diagnostic approach and antibiotic treatment of exacerbations, pathogen eradication, long-term antibiotic therapy, asthma-type therapies (inhaled corticosteroids, bronchodilators), mucoactive drugs, airway clearance, investigation of underlying causes of bronchiectasis, disease monitoring, factors to consider before surgical treatment and the reversibility and prevention of bronchiectasis in children/adolescents. Benchmarking quality of care for children/adolescents with bronchiectasis to improve clinical outcomes and evidence gaps for future research could be based on these recommendations.

Undiagnosed and retained foreign bodies may result in serious complications such as pneumonia, atelectasis, or bronchiectasis. We reviewed a total of 174 children with foreign body aspiration (FBA). Clinical, radiological, and bronchoscopic findings of these patients were evaluated according to the nature of foreign body and elapsed time from aspiration to diagnosis. Significant differences were noted between patients with organic and inorganic FBA in terms of clinical and radiological findings. Cough, recurrent pneumonia, and fever were the most common presenting symptoms in patients with delayed diagnosis. Long-term follow-up was available for 110 patients for a mean duration of 37.8 +/- 23.7 months (range, 1-88 months). We evaluated the course of recovery after bronchoscopic removal. Organic FBA was of comparable duration as for inorganic FBA, and prolonged follow-up was associated with increased risk of persistent symptoms and bronchiectasis (P < 0.001). The risk of long-term complications increased with increasing elapsed time from aspiration to diagnosis; complications were as high as 60% in children who were diagnosed 30 days after FBA (P = 0.0035). Bronchiectasis was a major complication, found in 25% of patients whose diagnosis was delayed by more than 30 days (P = 0.0001). Three patients with bronchiectasis underwent lobectomy. Patients with persistent asthma-like symptoms such as cough and wheezing required treatment with inhaled corticosteroids and bronchodilators. The positive response to this treatment was thought to be a confirmation of the development of transient bronchial hyperresponsiveness induced by foreign bodies. We conclude that timely diagnosis and appropriate treatment of FBA is important to prevent long-term complications in affected children.

Background: Non-cystic-fibrosis (non-CF) bronchiectasis in childhood is still one of the most common causes of childhood morbidity in developing countries. The management of these patients remains problematic, and there are few studies of long-term outcome. Objective: The aim of this retrospective study was to define the general characteristics, underlying causative factors and long-term follow-up results of non-CF bronchiectasis patients. Methods: One hundred and eleven consecutive children, diagnosed with non-CF bronchiectasis were included in the study. General characteristics and underlying causes were recorded from the medical records. Clinical outcomes were evaluated in terms of lung function tests, annual exacerbation rates and patient/parent perception of health status. Results: Mean age of the patients was 7.4 ± 3.7 years at presentation, and patients had been followed 4.7 ± 2.7 years on average. In 62.2% of the patients, an underlying etiology was identified, whereas postinfectious bronchiectasis was the most common (29.7%). In spite of intensive medical treatment, 23.4% of the patients required surgery. The annual lower respiratory infection rate has decreased from a mean of 6.6 ± 4.0 to 2.9 ± 2.9 during follow-up (p < 0.0001). Lung function tests were also found to be improved (mean FEV₁% 63.3 ± 21.0 vs. 73.9 ± 27.9; p = 0.01; mean FVC% 68.1 ± 22.2 vs. 74.0 ± 24.8; p = 0.04). There was clinical improvement in both the surgical (73%) and medical (70.1%) groups (p > 0.05). Conclusion: In conclusion, bronchiectasis remains a disease of concern to pediatricians, particularly in developing countries. Infections are still important causes of bronchiectasis, and clinical improvement can be achieved by appropriate treatment. Although medical treatment is the mainstay of management, surgery should be considered in selected patients.

The European Respiratory Society Task Force on primary ciliary dyskinesia (PCD) in children recently published recommendations for diagnosis and management. This paper compares these recommendations with current clinical practice in Europe.Questionnaires were returned by 194 paediatric respiratory centres caring for PCD patients in 26 countries. In most countries, PCD care was not centralised, with a median (interquartile range) of 4 (2-9) patients treated per centre. Overall, 90% of centres had access to nasal or bronchial mucosal biopsy. Samples were analysed by electron microscopy (77%) and ciliary function tests (57%). Nasal nitric oxide was used for screening in 46% of centres and saccharine tests in 36%. Treatment approaches varied widely, both within and between countries. European region, size of centre and the country's general government expenditure on health partly defined availability of advanced diagnostic tests and choice of treatments.In conclusion, we found substantial heterogeneity in management of PCD within and between countries, and poor concordance with current recommendations. This demonstrates how essential it is to standardise management and decrease inequality between countries. Our results also demonstrate the urgent need for research: to simplify PCD diagnosis, to understand the natural history and to test the effectiveness of interventions.

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