IS Case 84: Cystic Fibrosis
Imaging Sciences URMC 2008
Publication Date: 2009-05-20
Patient is a teenage male with hypoxia and shortness of breath.
Plain films demonstrate hyperinflation and predominantly upper lobe bronchiectasis. "Signet ring sign" - CT finding of dilated bronchus with adjacent smaller artery consistent with bronchiectasis. "Tree-in-bud" - CT finding of thickened secretions within peripheral centrilobular bronchioles. This finding is more commonly associated with infectious etiologies and is non-specific. Mucoid impaction ("finger in glove") may result in focal, often chronic atelectasis.
Cystic fibrosis is an autosomal recessive inherited disease predominantly affecting caucasians. Pathologic changes result from deficient chloride transport yielding thickened, viscous secretions. In the lungs, this leads to recurrent pulmonary infections which damage the airways over time. Colonization with pseudomonas is common. The pancreas, GI tract, liver, and sinuses are also frequently affected.
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- Stern RC. The diagnosis of cystic fibrosis. N Engl J Med. 1997 Feb 13;336(7):487-91. [PMID: 9017943]