Alpha 1 antitrypsin deficiency

Case contributed by Dr Andrew Dixon

Presentation

Worsening shortness of breath.

Patient Data

Age: 35
Gender: Female
X-ray

PA and lateral chest x-ray in this young patient demonstrates marked hyperinflation of the lungs (note over 11 posterior ribs visible on PA projection, flattened diaphragm, increased retrosternal air space) and hyperlucency of the lungs with distorted parenchymal architecture. 

Annotated image

Annotated images

Both lungs are markedly hyper-inflated with 11 posterior ribs easily visible above the diaphragmatic domes (yellow dotted lines). The diaphragms are depressed and flattened (best seen on lateral projection, with loss of the normal acute angle formed by the costophrenic pleural reflection (dotted blue line).  

The lungs appear very radiolucent with some areas devoid of lung markings ( * ) suggesting bullous formation. 

On lateral projection the retrosternal air space (RS) is markedly widened (aorta outlined in red). 

Diagram

Diagram of the three types of emphysema. 

Case Discussion

Severe mid and lower zone emphysema (pan-lobular) in a patient with alpha 1 antitrypsin deficiency.  Note the basal predominance which helps differentiate this condition from the far more common centrilobular emphysema of smoking.

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Case information

rID: 9674
Case created: 11th May 2010
Last edited: 28th Apr 2017
System: Chest
Inclusion in quiz mode: Included

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