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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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. © 1977.

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Details

Item Type:	Article
Status:	Published
Creators/Authors:	Creators Email Pitt Username ORCID Putnam, CW Porter, KA Peters, RL Ashcavai, M Redeker, AG Starzl, TE tes11@pitt.edu TES11
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
URI:	http://d-scholarship.pitt.edu/id/eprint/3764

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