Alpha-1-antitrypsin deficiency
Alpha-1-antitrypsin deficiency is a rare inherited disorder of variable severity which affects mainly the liver and lungs. Alpha-1-antitrypsin is a chemical which inhibits the action of enzymes called proteases. It is produced by the liver and released in response to inflammation, when its role is to switch off the action of enzymes designed to destroy inflammatory material before they do too much damage to normal tissue. If it is reduced or totally deficient we see the results of chronic inflammation: in the liver there's the development of cirrhosis, while in the lung there is emphysema (see the lung in the chest cavity section). While there is at present no treatment available genetic therapy is a possibility. It makes sense, of course, to reduce the inflammatory stimulus, so people with alpha-1-antitrypsin deficiency would be stupid to smoke.
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