Pulmonary Disease in Alpha-1-Antitrypsin Deficiency

  1. Gordon L. Snider, MD
  1. Boston University and Tufts University Schools of Medicine
    VA Medical Center
    Boston, Massachusetts

    Excerpt

    The current acceleration of technologic advances in medicine presents physicians and society with repeated challenges. For physicians, the challenge is how best to apply the new technology; for society, the challenge is how to pay for it. The release in December 1987 of a purified preparation of human alpha-1-antitrypsin (AAT) for augmentation therapy of AAT-deficient persons typifies these problems.

    The serum protein AAT, also known as alpha-1-protease inhibitor, is an inhibitor of serine proteases. Its main substrate in vivo is the serine protease, neutrophil elastase. Severe AAT deficiency is associated with the development of pulmonary emphysema in early or mid-life.

    This 100-word excerpt has been provided in the absence of an abstract.

    Article and Author Information

    • Grant Support: By NIH Program Project Grant HL 19717 and by the Department of Veterans Affairs.

    • Requests for Reprints: Gordon L. Snider, MD, Chief, Medical Service, VA Medical Center (111), 150 South Huntington Avenue, Boston, MA 02130.

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