Alpha-1-antitrypsin deficiency

Case contributed by Francis Fortin
Diagnosis certain

Presentation

Follow-up.

Patient Data

Age: 80 years
Gender: Female

Relatively mild basal-predominant panlobular emphysema with diffuse basal-predominant bronchiectasis and some endobronchial impactions. Secondary to the bronchiectasis, there are findings of both acute and chronic bronchiolar infection/inflammation.

Case Discussion

Genetically proven case of alpha-1-antitrypsin deficiency. For the patient's age, the emphysema is relatively mild. Also, bronchiectasis is rather marked here, which isn't very frequent but has been reported.

Note how panlobular emphysema can be quite subtle if viewed only in the axial plane. As in this case, it is easier to notice on the coronal images that there is a mildly decreased density in the basal segments of the lungs as compared to the upper segments. The lung parenchyma should never have a density similar to air, but should rather always be homogoneously a little bit denser, roughly -800 HU (Hounsfield units). A useful trick is to compare the lung parenchyma to pure air found in the trachea; if the parenchyma is nearly as black as the tracheal air, it is not normal.

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