NBME Step 2 CK Form 8 Abnormal chloride channel in airway epithelium (NBME Answers)

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32 year old man with nasal polyps, chronic cough that is progressive with copious sputum production who has clubbing and obstructive PFT findings (FEV1:FVC ratio < 70%) found to have Pseudomonas aeruginosa colonization most consistent with bronchiectasis in the setting of cystic fibrosis
Key idea: Bronchiectasis and chronic bronchitis (type of COPD) are two conditions most known for copious sputum production
Potential etiologies of bronchiectasis: (1) Congenital: Cystic fibrosis, Kartagener’s (2) Chronic/prior infection: Aspergillosis, mycobacteria (3) Rheumatic disease: Rheumatoid arthritis, Sjogren’s (4) Immunodeficiency: Hypogammaglobulinemia (5) Airway obstruction: Cancer
Key idea: Bronchiectasis should be worked up with CF testing, PFTs, sputum culture, immunoglobulin levels and high-resolution CT of the chest

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h1333 is it not a1 antutrypsin because this would mostly be lower lobes and at this age liver probs would be present? +2