Alpha-1 antitrypsin is an anti protease that protects the lungs from endogenous elastases, collagenases, and protease. These enzymes are responsible for the break down of proteins.
In smoking patients with early onset liver disease or panlobular emphysema, the diagnosis of alpha-1 antitrypsin deficiency should be considered.
Why does it cause cirrhosis?
The cirrhosis results from the inability to release the mutated alpha-1 antitrypsin protein which is made in the liver. It accumulates in the liver and results in cellular death and necrosis.
REFERENCES
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