USMLE/COMLEX - Step 2 - Cystic Fibrosis
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Pathophysiology
Transmembrane conductance regulator (CFTR) gene mutation of the chloride channel on chromosome 7
Etiologies
Caucasian
Symptoms
Recurrent respiratory infections
Chronic cough
Chronic sinusitis
Digital clubbing
Diabetes
Findings
Rhonchi
Rales
Hyperresonance to percussion
Nasal polyps
Diagnostics
Sweat chloride test is the best initial test
Treatment
Treat respiratory infections
Bronchodilators
Corticosteroids
Fat soluble vitamins A, D, E, K
High-calorie and high-protein diet
Dornase alfa
Ibuprofen
Ivacaftor