CF Pathophysiology

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Hiri P

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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

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CF Pathophysiology

Cards (2)

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