IPLab:Lab 5:α1 Antitrypsin Deficiency

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Clinical Summary

This 68-year-old white female was afflicted with severe emphysemaPulmonary 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. due to α1-antitrypsin deficiency. She had a 10 pack-yearA pack-year denotes smoking one pack of cigarettes per day for one 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 emphysemaPulmonary 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.. Post-operatively, she was ventilator dependent and her liver function declined. Further studies revealed hepatic cirrhosisCirrhosis is a liver disease characterized by necrosis, fibrosis, loss of normal liver architecture, and hyperplastic nodules.. Her pulmonary function continued to decline and she died 26A normal partial thromboplastin time is 28 to 37 seconds. days after her initial surgery.

Autopsy Findings

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 cirrhosisCirrhosis is a liver disease characterized by necrosis, fibrosis, loss of normal liver architecture, and hyperplastic nodules..

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