Liver replacement for alpha1-antitrypsin deficiency

Charles W. Putnam, Kendrick A. Porter, Robert L. Peters, Mary Ashcavai, Allan G. Redeker, Thomas E. Starzl

Research output: Contribution to journalArticlepeer-review

47 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)258-261
Number of pages4
JournalSurgery
Volume81
Issue number3
StatePublished - Mar 1977
Externally publishedYes

ASJC Scopus subject areas

  • Surgery

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