Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Cystic Fibrosis
- Radiologic Findings
- CT of the thorax on lung window setting reveals extensive bilateral bronchiectasis with bronchial wall thickening and areas of decreased attenuation and vascularity. CT of the abdomen shows near total fatty replacement of the pancreas.
- Brief Review
- Cystic fibrosis is an autosomal recessive hereditary disease and uncommon in nonwhites. The basic abnormality consists of abnormal secretions from various exocrine glands including the salivary and sweat glands, pancreas, large bowel and tracheobronchial tree. The major clinical manifestations are obstructive pulmonary disease, which is found in varying degree of severity in almost all patients, and pancreatic insufficiency.
The main CT manifestation of cystic fibrosis is bronchiectasis, which is present in virtually all adult patients. Bronchiectasis usually involves all lobes, but tends to be most severe in the upper lobes.
In patients with cystic fibrosis, pancreatic glandular tissue is significantly reduced in size. Pancreatic fatty replacement is the most frequent pattern in older patients with cystic fibrosis.
- References
- 1. Muller NL, Silva CS. Imaging of the Chest. Saunders 2008:1039-1044
2. Soyer P, Spelle L, Pelage JP, et al. Cystic Fibrosis in Adolescents and Adults: Fatty Replacement of the Pancreas - CT Evaluation and Functional Correlation. Radiology 1999:210;611-615
- Keywords
- Airway, Congenital,