IPLab:Lab 5:α1 Antitrypsin Deficiency

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Clinical Summary[edit]

This 68-year-old white female was afflicted with severe emphysema due to α1-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 initial surgery.

Autopsy Findings[edit]

There were severe emphysematous changes in all lung lobes and there was massive hemorrhage throughout the lungs (combined lung weight: 3510 grams). The liver weighed 860 grams and there was micronodular cirrhosis.


Study Questions[edit]

Additional Resources[edit]


Journal Articles[edit]


Related IPLab Cases[edit]

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.