“ This is an autosomal recessive defect in the gene regulating chloride transport, resulting in thick secretions. The lungs and pancreas are...
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This is an autosomal recessive defect in the gene regulating chloride transport, resulting in thick secretions. The lungs and pancreas are affected most severely.
PLAIN FILM
• Bronchiectasis (cylindrical at first).
• Bronchial wall thickening.
• Hyperinflation (due to air-trapping).
• Any part of the lungs may be affected, but particularly central zones, upper lobes and apical lower lobes.
• Look for mucous impaction with atelectasis.
• Spontaneous pneumothorax.
• Pulmonary artery enlargement.
HRCT
• Most sensitive for detecting early change.
Cystic fibrosis. Chest x-ray demonstrating hyperexpanded lungs with upper zone fibrosis, bronchial wall thickening and bronchiectasis affecting the central and upper zones. There is a cavity with a soft tissue focus outlined by a crescent of air (Monad sign; white arrow) in keeping with an aspergilloma.
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