Adult-Onset Cystic Fibrosis (with Pancreatic Fatty Atrophy)
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Imaging findings
In the first patient, CT reveals subtle tracheal wall thickening, diffuse bronchial thickening, and clustered centrilobular nodules in the middle lobe. Abdominal imaging demonstrates near-complete fatty atrophy of the pancreas. A comparative second patient, a 72-year-old, showed bronchial wall thickening and tree-in-bud nodularity.
Key takeaways
Adult-onset cystic fibrosis can present with subtle lung findings like mild bronchial thickening and centrilobular nodules, along with pancreatic fatty atrophy, even at older ages. It's an important differential to consider in patients with chronic cough and pancreatic atrophy, despite mild lung disease that may not be severely progressed.
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