II. Epidemiology

  1. Onset: middle aged

III. Pathophysiology

  1. Chronic inflammatory or infectious pulmonary process
  2. Results in multiple dilatations of small Bronchi
  3. Bronchi exude pustular discharge

IV. Causes

  1. Bronchial obstruction
  2. Recurrent or severe pulmonary infections
    1. Necrotizing pulmonary infection
    2. Pulmonary Abscess
    3. Tuberculosis
    4. Aspergillosis
    5. Measles
    6. Pertussis
    7. RSV Bronchiolitis
  3. Hypergammaglobulinemia
  4. Dyskinetic cilia syndrome
  5. Kartagener's Syndrome
  6. Alpha-1 Antitrypsin Deficiency
  7. Cystic Fibrosis
  8. Inhalation of noxious chemicals

V. Symptoms

  1. Productive cough
    1. Copious Sputum (200-500 ml/day)
    2. Sputum thick, mucopurulent and foul-smelling
    3. Hemoptysis
  2. Wheezing
  3. Dyspnea
  4. Halitosis
  5. Fatigue
  6. Weight loss to Emaciation

VI. Signs

  1. Lung auscultation
    1. Coarse or moist crackles
    2. Rales and Rhonchi
    3. Wheezing
    4. Diminished breath sounds
  2. Cyanosis
  3. Digital Clubbing

VIII. Labs: Sputum

  1. Sputum forms layers on standing
    1. Top: Mucus
    2. Middle: Clear fluid
    3. Bottom: Pus
  2. Sputum Culture not diagnostic (mixture of organisms)
  3. Fungal Culture

IX. Imaging: Chest XRay

  1. Often normal, even in advanced disease
  2. May show increased density at lung bases
  3. Airways may be dilated and thickened ("ring shadow")
  4. Atelectasis may be present

X. Diagnosis

  1. Pulmonary Function Tests
    1. Airflow obstruction with reversible component
  2. Diagnostic postural drainage
    1. Patient lies prone in Trendelenburg for 5-15 minutes
    2. Head over edge of table, and pan on floor
    3. Patient coughs several times and pus rolls into pan
  3. Bronchograms with opaque medium
  4. Bronchoscopy
  5. High-resolution Chest CT
    1. Airways are thick and filled with mucous

XI. Management

  1. Pulmonary toilet (chest PT or VEST Therapy)
  2. Inhaled Corticosteroids
  3. Hypertonic Saline nebs
  4. Macrolide antibiotics

XII. Course

  1. Chronic progressive with exacerbations

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