Treatment of liver disease in alpha-1

Liver disease due to alpha-1-antitrypsin (A1AT) deficiency (also known as alpha-1) is treated using the same general supportive measures as other types of liver disease. The goal is to lessen the impact of complications (eg, cirrhosis) or prevent them altogether.1

  • In cases where the liver disease becomes life-threatening, liver transplantation has become common.
    • Assuming that the donor does not have alpha-1 (eg, has a Pi MM genotype), the transplant will also resolve the alpha-1 and serum levels of A1AT will return to the normal range.
  • Most patients with a Pi ZZ genotype are followed annually by a liver specialist, but management is typically conservative in the absence of signs or symptoms of liver disease.

References

  1. Teckman JH. Liver disease in alpha-1 antitrypsin deficiency: current understanding and future therapy. COPD. 2013;10(Suppl 1):35-43.

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