Presentation
Cough and fever. History of recurrent chest infections.
Patient Data
There are bilateral predominantly central and upper lobes with lesser extent lower cylindrical bronchiectasis with bronchial wall thickening and mucoid impaction more on the right side.
Peribronchial interstitial thickening is noted.
Diffuse centrilobular nodular opacities are noted suggestive of acute infectious bronchiolitis.
Few peripheral subpleural nodules are noted.
No effusion. No pneumothorax.
Multiple mediastinal and bilateral hilar lymphadenopathy, the largest measures about 3x2 cm seen in the pre-vascular group.
In the visualized part of the upper abdomen:
Focal hepatic steatosis is noted involving the segment IV.
The pancreas shows complete fatty replacement.
Features are in favor of cystic fibrosis with bronchiolitis.
Case Discussion
Cystic fibrosis is an autosomal recessive disorder characterized by producing viscous secretions and dysfunction involving the liver, lungs, pancreas, and small bowel. Diagnosis can be made by a sweat test and genetic study. Most patients diagnosed by the age of 1 year. Most of the clinical manifestations are due to pulmonary and pancreatic insufficiency.