Semin Liver Dis 1982 Nov;2(4):314-28

Alpha-1-antitrypsin: an ignored protein in understanding liver disease

Sharp HL

Abstract
alpha 1AT should no longer be ignored by hepatologists with regard to its role
in protecting the liver from injury. This protective role needs further
definition with regard to protease inhibition, immunologic control, and
functional inactivation. Severe deficiency of alpha 1AT clearly predisposes to
definable types of liver disease not only in infants and children, but at the
other end of the spectrum in elderly adults. It is unfortunate that the
pathophysiology of human and animal alpha 1AT related liver injury is not as
readily apparent as in pulmonology conditions. However, it is time to digest the
tremendous progress that has evolved in that field in relating alpha 1AT to
emphysema. Cirrhosis of the liver should be studied in relationship to the
pathophysiology demonstrated by our colleagues to be important in "cirrhosis of
the lung." The diagnosis of alpha 1AT deficiency and the course of the
associated liver disease have been reviewed and updated. Prevention and cure
have been demonstrated to be possible but neither method has universal
applicability. In contrast, other potential therapeutic endeavors, although not
presently proved to be efficacious, with time and research will provide
methodologies for the treatment of this disorder as well as insight into
mechanisms of general liver injury not previously appreciated