IPLab:Lab 5:α1 Antitrypsin Deficiency
Contents
Clinical Summary[edit]
This 68-year-old white female was afflicted with severe emphysema due to alpha 1-antitrypsin deficiency. She had a 10 pack-year history of cigarette smoking but she had quit smoking 30 years ago. She elected to undergo lung volume reduction surgery to reduce complications of her emphysema. Post-operatively, she was ventilator dependent and her liver function declined. Further studies revealed hepatic cirrhosis. Her pulmonary function continued to decline and she died 26 days after her surgery.
At autopsy there were severe emphysematous changes in all lung lobes and there was massive hemorrhage throughout the lungs. The liver weighed 860 grams and there was micronodular cirrhosis.
Images[edit]
This is a gross photograph of the lungs from this case. The rough friable material on the surface of the lung (arrows) is fibrinous exudate and fibrous tissue. This reaction on the surface of the lung is due to the recent surgery. The emphysematous changes are not easily appreciated in this photograph.
Study Questions[edit]
Additional Resources[edit]
Reference[edit]
- eMedicine Medical Library: α1-Antitrypsin Deficiency
- eMedicine Medical Library: Emphysema
- eMedicine Medical Library: COPD and Emphysema
- Merck Manual: α1-Antitrypsin Deficiency
Journal Articles[edit]
- Perlmutter DH. Liver injury in α1-antitrypsin deficiency. Clin Liver Dis 2000 May;4(2):387-408, vi.
Images[edit]
- PEIR Digital Library: Emphysema Images
- PEIR Digital Library: α1-Antitrypsin Deficiency Images
- WebPath: Emphysema
Related IPLab Cases[edit]
- Lab 2: Liver: Fatty Change and Cirrhosis
- Lab 10: Lung: Cryptococcosis
- Lab 12: Lung: Chronic Obstructive Pulmonary Disease
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Pulmonary emphysema is a condition in which the air spaces distal to the terminal bronchioles are permanently increased in size due to either destruction of the wall or alveolar dilatation.
A pack-year denotes smoking one pack of cigarettes per day for one year.
Cirrhosis is a liver disease characterized by necrosis, fibrosis, loss of normal liver architecture, and hyperplastic nodules.
A normal partial thromboplastin time is 28 to 37 seconds.
Friable material is easily crumbled.
Consolidation is the filling of lung air spaces with exudate--this is a sign of pneumonia.