IS Case 304: Cystic fibrosis

Aharon Wolf, MD

Imaging Sciences URMC 2009
Publication Date: 2009-08-06

History

Patient is a 17-year-old male with cystic fibrosis with increased cough and sputum.

Findings

Severe diffuse bronchiectasis and peribronchial thickening.

Diagnosis

Cystic fibrosis

Discussion

Cystic fibrosis is a hereditary disease affecting the exocrine glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure. Thick mucus production results in frequent lung infections resulting in bronchiectasis and peribronchial thickening. Thickened secretions from the pancreas can eventually lead to pancreatic atrophy. These features are seen in the presented images.

References

  1. Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005 May 12;352(19):1992-2001. PMID: 15888700 [PubMed]

4 images