CF Pathophysiology

• Pathophysiology – lungs:

  • Pulmonary function normal at birth
  • Decrease in height of peri-ciliary layer -> mucus plugging
  • Mucus leads to bronchial obstruction & inflammatory response → vicious cycle of infection & persistent inflammation → bronchiectasis and irreversible damage
  • Inflammation present at a very early age in infants and children with CF who have minimal or no evidence of lung disease
• Pathophysiology – lungs:

  • Predominantly affected in upper lobes
  • Areas of consolidation (due to inflammation) and atelectasis (due to mucus plugging)
  • Formation of cysts
  • Alveoli relatively spared
  • Obstructive disease as the airways are getting narrowed due to sputum