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Alpha-1 antitrypsin deficiency - Treatment

Alternative Names

AAT deficiency

Treatment:

Treatment involves replacing the missing AAT protein. The protein is given through a vein each week. However, it is not yet known who may benefit from such therapy.

Quitting smoking is crucial.

Other treatments specifically for emphysema and cirrhosis are also used.

Expectations (prognosis):

Some people with this deficiency will not develop liver or lung disease. However, emphysema and cirrhosis can be deadly.

Complications:

Cirrhosis or liver failure
Emphysema
Liver cancer

Calling your health care provider:

Call your health care provider if you develop symptoms of AAT deficiency.

Lungs

Liver anatomy

Reviewed last on: 8/29/2008
Sean O. Stitham, MD, private practice in Internal Medicine, Seattle, WA; Benjamin Medoff, MD, Assistant Professor of Medicine, Harvard Medical School, Pulmonary and Critical Care Unit, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Köhnlein T, Welte T. Alpha-1 antitrypsin deficiency: pathogenesis, clinical presentation, diagnosis, and treatment. JAMA. 2008;121(1):3-9.

Stoller JK, Tomashefski J, Crystal RG, et al. Mortality in individuals with severe deficiency of alpha-1-antitrypsin: Findings from the National Heart, Lung, and Blood Institute Registry. Chest. April 2005;127:1196-1204.

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