Correct!
5. All of the Above
Bronchiectasis is a primary sign of cystic fibrosis lung disease. It typically develops in the upper lobes and presents on chest radiograph as tram-lining and cystic lesions containing air-fluid levels. On CT, bronchiectasis additionally presents as a “signet ring sign” (internal bronchial diameter exceeds that of the corresponding artery), thickening of the bronchial wall, visible bronchi within 1 cm of the costal pleura or touching the mediastinal pleura, and a lack of normal bronchial tapering (1). In addition, hyperinflation is historically recognized as one of the major causes of respiratory dysfunction in cystic fibrosis (2). Lobar collapse and atelectasis occur in 4-11% of cystic fibrosis patients and most frequently affect the upper lobes (1). Prominent pulmonary arteries are visible in cases of pulmonary hypertension, which may arise from chronic hypoxia characteristic of advanced cystic fibrosis lung disease (1).
What is the underlying molecular mechanism of cystic fibrosis? (Click on the correct answer to move to the next panel)