Bronchiectasis

In children suspected of having bronchiectasis, diagnostic tests are necessary to confirm the disease, assess the extent and severity, identify associated organ abnormalities (e.g., cor pulmonale), and determine treatable underlying causes or disease progression factors. 

1. High-resolution chest CT (The standard diagnostic method)

• Signet ring sign: The bronchial lumen appears larger than the accompanying vessel. (In pediatric patients, the bronchus-to-artery ratio cutoff is generally set at 0.8)
• False negatives may occur in the early stages, so imaging is recommended after the acute phase. 

2. Evaluation of underlying diseases

• IgG, IgA, IgM, IgG subclasses, IgE, WBC count, Aspergillus-specific IgE, Aspergillus skin testing, total IgE, and ciliary biopsy. 
• Depending on clinical presentation, bronchoscopy, gastroesophageal reflux testing, tuberculosis testing, additional immunologic assessments, and genetic testing may be considered. 

3. Pulmonary function tests

• Typically normal in the early stages but may progress to obstructive or mixed obstructive-restrictive patterns over time.

1. Airway Clearance Techniques

• Clearing airway secretions by thinning and effectively removing sputum is essential in the treatment of bronchiectasis. 
• Mucolytics help liquefy sputum, and inhalation of hypertonic saline facilitates mucus clearance, improves lung function, and reduces the frequency of exacerbations. 
• Chest physiotherapy techniques such as positive expiratory pressure devices, Acapella, and percussion promote mucus drainage. 
• The active cycle of breathing technique, involving diaphragmatic breathing with slow nasal inhalation and forceful oral exhalation, also aids in sputum clearance. 
• Airway clearance should be performed for at least 10 minutes and up to 30 minutes, and continued until the patient produces two consecutive huffs or coughs without sputum.

2. Bronchodilators and Mucolytics

• With airflow limitation and airway hyperresponsiveness: Long-term inhaled beta-2 agonists 
• Mucolytics such as erdosteine, acetylcysteine, and ambroxol 

3. Anti-inflammatory Treatment:

• In allergic bronchopulmonary aspergillosis: Systemic steroids 
• Macrolides (azithromycin) : Long-term use has been reported to reduce exacerbations, improve lung function, and enhance quality of life, although it carries the risk of increasing macrolide-resistant organisms.

4. Antibiotic Therapy

• Antibiotics can be used to suppress chronic airway colonization and treat acute exacerbations, typically requiring 2–4 weeks of treatment. 
• Acute exacerbations: Productive cough persists or worsens for more than 72 hours, accompanied by signs such as elevated CRP, neutrophilia, changes in sputum color, dyspnea, chest pain, crackles, or wheezing.
• If an exacerbation is suspected, sputum and other tests should be performed to identify pathogens before initiating antibiotics.

5. Surgery

• Surgical resection of the affected lobe or segment may be considered when medical treatment fails and the disease is localized, especially in cases of recurrent massive hemoptysis despite bronchial artery embolization. 

6. Exercise and Vaccination

• Encourage regular physical activity, especially exercises involving moisture (e.g., swimming) or bouncing motions like trampolining
• Environmental control: Avoiding exposure to smoke
• Routine vaccinations: Measles, pertussis, Hib, pneumococcus along with annual influenza vaccination.