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Original communication| Volume 81, ISSUE 3, P258-261, March 1977

Liver replacement for alpha1-antitrypsin deficiency

DOI:https://doi.org/10.5555/uri:pii:0039606077902264
Liver replacement for alpha1-antitrypsin deficiency
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      Abstract

      A 16-year-old girl with advanced cirrhosis and severe alpha1-antitrypsin deficiency of the homozygous PiZZ phenotype was treated by orthotopic liver transplantation. After replacement of the liver with a homograft from a donor with the normal PiMM phenotype, the alpha1-antitrypsin concentration in the recipient's serum rose to normal; it had the PiMM phenotype. Two and a third years later, chronic rejection necessitated retransplantation. Insertion of a homograft from a heterozygous PiMZ donor was followed by the identification of that phenotype in the recipient's serum. Neither liver graft developed the alpha1-antitrypsin glycoprotein deposits seen with the deficiency state. These observations confirm that this hepatic-based inborn error of metabolism is metabolically cured by liver replacement.
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      Article info

      Footnotes

      ☆Supported by research grants MRIS 8118-01 and 7227-01 from the Veterans Administration, by Grant Nos. AM-17260 and AM-07772 from the National Institutes of Health, by Grant Nos. RR-00051 and RR-0069 from the General Clinical Research Centers Program of the Division of Research Resources, National Institutes of Health, and by the Josiah Macy, Jr., Foundation (Dr. Starzl).

      ☆☆Presented at the Second Annual Meeting of the American Society of Transplant Surgeons, Chicago, Ill., May 20–22, 1976.

      Identification

      DOI: https://doi.org/10.5555/uri:pii:0039606077902264

      Copyright

      © 1977 Published by Elsevier Inc.

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