How are deficient enzymes replaced in the treatment of alpha1-antitrypsin deficiency (AATD)?

Updated: Sep 11, 2020
  • Author: Dora E Izaguirre Anariba, MD, MPH; Chief Editor: John J Oppenheimer, MD  more...
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Answer

Alpha1-antitrypsin-deficient individuals who have or show signs of developing significant emphysema can be treated with Prolastin, a pooled, purified, human plasma protein concentrate replacement for the missing enzyme that has been screened for HIV and hepatitis viruses, although practitioners should immunize patients against hepatitis regardless. It also is heat-treated as an additional precaution against transmission of infection. The US Food and Drug Administration (FDA) has approved 2 other alpha1-antitrypsin protein concentrates, Aralast and Zemaira, for augmentation therapy.

Weekly IV infusions of alpha1-antitrypsin protein concentrates restore serum and alveolar alpha1-antitrypsin concentrations to protective levels. Although other dosing regimens have been used, only the weekly infusion schedule has US FDA approval.


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