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Emphysema & Alpha-1
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About Emphysema
Emphysema is a form of chronic obstructive pulmonary disease (COPD). Emphysema is caused by destruction of alveoli, small sac-like structures in the lungs where oxygen from the air is exchanged for carbon dioxide in the blood.
As a result of the damage, the fragile alveolar walls become filled with “holes.” Destruction of the alveolar walls leads to less surface area for gas exchange, which leads to less oxygen in the blood. The loss of elasticity also leads to air becoming trapped in the lungs, resulting in too much carbon dioxide in the blood.
Emphysema and Alpha-1
Individuals with alpha-1 antitrypsin (AAT) deficiency* – also called Alpha-1 – are at risk for developing emphysema because of the unique role that AAT plays in protecting the lungs. An enzyme called neutrophil elastase (NE) normally is present in the lungs and plays a role in fighting bacteria. However, NE can destroy lung tissue if it is not neutralized by AAT.
*Also known as Alpha-1, AAT Deficiency, AATD, A1-PI deficiency, alpha-1 proteinase deficiency, A1AD, or hereditary or genetic emphysema.
Individuals with alpha-1 antitrypsin deficiency do not have enough AAT to maintain the balance between AAT and NE, and so have inadequate protection against the damaging effects of NE. 1
The symptoms and effects of emphysema are the same whether the emphysema is caused by AAT deficiency or by other factors, such as smoking. But there are several factors which differentiate typical emphysema from AAT-related emphysema.
| Typical Emphysema | AAT-related Emphysema | |
|---|---|---|
| Affects older individuals | Younger age of onset | |
| Not associated with manifestations outside the lungs | May be associated with liver abnormalities and panniculitis | |
| Usually associated with heavy smoking history | May occur without smoking history (Individuals with AAT deficiency who do smoke may experience earlier onset of symptoms.) |
References
- American Lung Association. Alpha-1 Antitrypsin Deficiency Emphysema. Available at http://www.lungusa.org/site/apps/nlnet/content3.aspx?c=dvLUK9O0E&b=4294229&ct=3004003 (Accessed September 18, 2008).
