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Bronchiectasis

Abnormal dilation of distal airways, associated with chronic cough and excessive sputum production. 

Causes

GeneticCF, Young’s syndrome, Kartagener’s syndrome, yellow nail syndrome, α1-antitrypsin deficiency
MechanicalMalignancy, foreign body
Childhood infectionPertussis, measles, TB, pneumonia
Immunological Allergic bronchopulmonary aspergillosis, hypogammaglobulinaemia, HIV, leukaemia, autoimmune

Clinical features

Symptoms

  • Chronic productive cough
  • Haemoptysis
  • Recurrent chest infections

Signs of bronchiectasis

  • Clubbing
  • Inspiratory clicks
  • Coarse inspiratory crepitations 
  • Large airway rhonchi

Signs of cause

  • Curved yellow nails and lymphoedema (yellow nail syndrome)
  • Dextrocardia (Kartagener’s syndrome)
  • Young and thin (CF)

Investigations

  • Diagnosis
    • Chest X-ray: tramlines and ring shadows
    • High-resolution CT chest: ‘signet ring sign’ (bronchi larger than the adjacent vasculature) and bronchial wall thickening – diagnostic test
    • Spirometry: variable but often obstructive pattern
  • To ascertain cause
    • Immunoglobulins and electrophoresis
    • Aspergillus precipitins and serum IgE 
    • CF sweat test/genetic mutation analysis
    • HIV
    • Rheumatoid factor
    • α1-antitrypsin
  • Sputum culture (to determine any colonising bacteria and sensitivities)

Management

  • Exercise and improved nutrition
  • Chest physiotherapy
  • Prompt treatment of infections (choose antibiotics based on culture sensitivities)
  • Rotating antibiotics may be used for prophylaxis if recurrent infections
  • Trial of bronchodilator and nebulised hypertonic saline
  • Pneumococcal/influenza vaccines
  • Lung resection may be considered in localised disease with a poor response to medical management
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