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What do Alpha1 lungs look like?

CT scan of alpha1-antitrypsin deficient patient

Diagnosing Alpha1 takes a simple test

Early detection can make a difference. Find out about the free Grifols AlphaKit you can order.

What Is Alpha1-Antitrypsin (AAT) Deficiency?

Alpha1-antitrypsin (AAT) deficiency (Alpha-1) is a genetic condition that 1:

  • Leads to decreased circulating levels of AAT
  • Significantly increases the risk of
  • Serious lung disease in adults
  • Liver disease in infants, children and adults
  • Skin disease in rare instances
  • AAT is a protein with potent protease inhibitor activity. Its main function is to protect normal lung tissue from proteolytic attack during inflammation, such as that caused by infection and inhaled irritants such as tobacco smoke

Learn more about Alpha-1 lung disease from the Alpha-1 Foundation.

AAT plays a critical role in protecting lungs2

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Protecting Lungs Images Key
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Neutrophils mobilize, delivering elastase at the alveolar surface in
response to bacterial infections, environmental pollutants, or tobacco exposure.
Normal AAT levels keep neutrophil elastase in check.
AAT inhibits excess elastase; lung structure preserved.
Low levels of AAT leave lung tissue unprotected.
Excess elastase cannot be neutralized; lung elastin destroyed; lung function compromised.
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Click arrows to advance images.

  • The liver is the primary source of circulating AAT1
  • Low circulating levels of AAT in AAT deficient patients is due to abnormal folding of the defective AAT protein in the endoplasmic reticulum of the liver, leading to polymerization and accumulation of AAT in hepatocytes1
  • The liver is the primary source of circulating AAT, so this accounts for the decreased circulating levels of this important protein1
  • Accumulation of polymerized abnormal protein in the hepatocytes appears to be the cause of the increased risk of cirrhosis and liver failure in individuals with Alpha-11

Learn more about Alpha-1 liver disease from the Alpha-1 Foundation.

Alpha-1 is more common than you may think

next: Prevalence of Alpha-1 >

Important Safety Information

PROLASTIN-C, Alpha1-Proteinase Inhibitor (Human) is indicated for chronic augmentation and maintenance therapy in adults with emphysema due to deficiency of alpha1-proteinase inhibitor (alpha1-antitrypsin deficiency). The effect of augmentation therapy with any alpha1-proteinase inhibitor (alpha1-PI) on pulmonary exacerbations and on the progression of emphysema in alpha1-antitrypsin deficiency has not been demonstrated in randomized, controlled clinical trials. PROLASTIN-C is not indicated as therapy for lung disease in patients in whom severe Alpha1-PI deficiency has not been established.

PROLASTIN-C may contain trace amounts of IgA. Patients with known antibodies to IgA, which can be present in patients with selective or severe IgA deficiency, have a greater risk of developing potentially severe hypersensitivity and anaphylactic reactions. PROLASTIN-C is contraindicated in patients with antibodies against IgA.

The most common drug related adverse reactions during clinical trials in ≥ 1% of subjects were chills, malaise, headache, rash, hot flush, and pruritus.

PROLASTIN-C is made from human plasma. Products made from human plasma may carry a risk of transmitting infectious agents, e.g., viruses, and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.

Please see accompanying PROLASTIN-C Full Prescribing Information for complete prescribing details.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.

References
  1. What is Alpha-1? Alpha-1 Foundation. http://www.alphaone.org/healthcare/?c=01-What-is-Alpha-1-Healthcare. Accessed October 6, 2009.
  2. Köhnlein T, Welte T. Introduction. Alpha-1 Antitrypsin Deficiency: Clinical Aspects and Management. Bremen, Germany: Uni-Med Verlag AG; 2007:16-39.