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Liver replacement for alpha<inf>1</inf>-antitrypsin deficiency

Putnam, CW and Porter, KA and Peters, RL and Ashcavai, M and Redeker, AG and Starzl, TE (1977) Liver replacement for alpha<inf>1</inf>-antitrypsin deficiency. Surgery, 81 (3). 258 - 261. ISSN 0039-6060

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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. © 1977.


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Item Type: Article
Status: Published
CreatorsEmailPitt UsernameORCID
Putnam, CW
Porter, KA
Peters, RL
Ashcavai, M
Redeker, AG
Starzl, TEtes11@pitt.eduTES11
Centers: Other Centers, Institutes, Offices, or Units > Thomas E. Starzl Transplantation Institute
Date: 1 January 1977
Date Type: Publication
Journal or Publication Title: Surgery
Volume: 81
Number: 3
Page Range: 258 - 261
Institution: University of Pittsburgh
Refereed: Yes
ISSN: 0039-6060
Other ID: uls-drl:31735062109792, Starzl CV No. 378
Date Deposited: 08 Apr 2010 17:06
Last Modified: 22 Jun 2021 10:55


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