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Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed
Due to an inborn error of metabolism causing dysfunction of all exocrine glands leading to thickened exocrine gland secretions. The lungs are normal at birth but become progressively obstructed leading to hyperinflation. The thickened mucus in the airways gives the patient a predisposition to infection, often with Pseudomonas, which over time results in bronchiectasis. Eventually as the pulmonary disease worsens the patient gets pulmonary hypertension and cor pulmonale. Pneumothorax and pulmonary hemorrhage are common complications.
The classical CXR signs are hyperinflation, peribronchial thickening, cystic bronchiectasis, fibrosis, and scattered areas of mucoid impaction. The cardiac silhouette is normally narrowed due to the pulmonary hypertension and the generalized obstructive emphysema.
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