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Home > Patients & Visitors > Health Library > COPD and Alpha-1 Antitrypsin (AAT) Deficiency
Alpha-1 antitrypsin (AAT) is a protein normally found in
the lungs and the bloodstream. It helps protect the lungs from the damage
caused by inflammation that can lead to
chronic obstructive pulmonary disease (COPD). People
whose bodies do not produce enough of this protein (AAT deficiency) are more
likely to develop emphysema and to do so at a younger-than-normal age (30 to 40
years old). AAT deficiency is a rare disorder and is the only known
genetic (inherited) factor that increases your risk of
Your doctor may suspect you have an AAT
deficiency if you:1
An AAT deficiency test measures the level of AAT in the
blood. The test is not routinely done in most people with COPD. But the
American Thoracic Society and the European Respiratory Society recommend
testing for an AAT deficiency for people who have:1
Screening for an AAT deficiency in the general public is not
It is extremely important that you do not
smoke if you have an AAT deficiency. Smokers with this condition may suffer
devastating disease at a young age. People with this condition who have never
smoked usually do not have significant symptoms at any age.
People with an AAT deficiency may be treated with injections of man-made
alpha-1 antitrypsin protein (also called an alpha-1 proteinase inhibitor) that
has been obtained from human
plasma. Examples include Aralast, Prolastin, and
Zemaira. To be considered for this treatment, you must meet the following
Injections of replacement alpha-1 antitrypsin are given
either weekly or every 2 to 4 weeks. Benefits of the therapy are not clear at
American Thoracic Society/European Respiratory Society (2003). ATS/ERS: Standards for the diagnosis and management of individuals with alpha1-antitrypsin deficiency. American Journal of Respiratory and Critical Care Medicine, 168(7): 820–822.
November 29, 2011
E. Gregory Thompson, MD - Internal Medicine & Ken Y. Yoneda, MD - Pulmonology
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