Cystic fibrosis

Case contributed by Abdallah Al Khateeb
Diagnosis certain

Presentation

Known case of a congenital disorder. Developed respiratory decompensation.

Patient Data

Age: 20 years
Gender: Male
x-ray
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Info

Findings of advanced bilateral bronchiectasis; ring shadows, tram-track lines and ill defined vascular markings.

ct
This study is a stack
Axial
non-contrast
This study is a stack
Axial lung
window
This study is a stack
Coronal
lung window
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Info

Better definition of the changes are seen on the CT scan. Cylinderical, varicose and cystic bronchiectasis are well demonstrated. Peribronchial thickening and multiple ground glass opacities.

Case Discussion

This patient has cystic fibrosis. Other findings seen on this case related to cystic fibrosis include:

  • extensive pancreatic parenchymal fatty replacement
  • numerous hypodense nodules involving the liver, separated by bands of residual parenchyma, which is best explained by cirrhosis with multiple regenerative nodules
  • splenomegaly with multiple prominent venous channels at its hilum, with a prominent portal system; changes of portal hypertension.
  • thin patient

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