Cystic fibrosis

Case contributed by Jane McEniery
Diagnosis certain

Presentation

Left sided pleuritic chest pain

Patient Data

Age: 15 years
Gender: Female
x-ray

Right-sided Port-a-Cath, with tip in the right atrium.  Normal heart size.
The lungs are hyperinflated. Bilaterally there is bronchiectasis characterized by bronchial dilation and wall thickening. The upper lobes are more severely affected than the lower lobes. There is focal opacification in the upper zones suggesting mucus plugging. No confluent lobar consolidation or pleural effusion. Pleuroparenchymal scarring at both lung apices. 

Case Discussion

The appearances are typical for cystic fibrosis. The diagnosis was established on a newborn screening test. 

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