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Liver transplantation for tyrosinemia. A review of 10 cases from the University of Pittsburgh.

Mieles, LA and Esquivel, CO and Van Thiel, DH and Koneru, B and Makowka, L and Tzakis, AG and Starzl, TE (1990) Liver transplantation for tyrosinemia. A review of 10 cases from the University of Pittsburgh. Digestive diseases and sciences, 35 (1). 153 - 157. ISSN 0163-2116

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Abstract

Results of liver transplantation in 10 patients with tyrosinemia are reviewed. The indications for transplantation were: hepatoma in three, acute liver failure in two, and progressive chronic liver disease in five. One patient died during surgery. Of the remaining nine who survived the operation, one died at six months as a result of bronchial aspiration and aspiration pneumonia, and a second transplanted for hepatoma died five months later with metastases. Seven patients are alive 6 months to 6 1/2 years following transplantation. Of these seven patients, six have normal liver function and a good performance status. One is awaiting retransplantation for chronic rejection. Hepatocellular carcinoma (HCC) was found either preoperatively or incidentally in five patients, all older than 2 years at the time of their transplant. Four of these are alive and well without evidence of tumor with follow-ups between 3 1/2 and 6 1/2. Four of the five patients less than 2 years of age had hepatocellular dysplasia without evidence of carcinoma on histologic examination of the resected liver. This experience suggests that liver transplantation should be considered seriously for children with hereditary tyrosinemia who are more than 2 years of age because beyond that age the incidence of hepatocellular carcinoma (HCC) increases substantially.


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Details

Item Type: Article
Status: Published
Creators/Authors:
CreatorsEmailPitt UsernameORCID
Mieles, LA
Esquivel, CO
Van Thiel, DH
Koneru, B
Makowka, L
Tzakis, AG
Starzl, TEtes11@pitt.eduTES11
Centers: Other Centers, Institutes, Offices, or Units > Thomas E. Starzl Transplantation Institute
Date: 1 January 1990
Date Type: Publication
Journal or Publication Title: Digestive diseases and sciences
Volume: 35
Number: 1
Page Range: 153 - 157
Institution: University of Pittsburgh
Refereed: Yes
ISSN: 0163-2116
Other ID: uls-drl:31735062116441, Starzl CV No. 1090
Date Deposited: 08 Apr 2010 17:18
Last Modified: 22 Jun 2019 12:55
URI: http://d-scholarship.pitt.edu/id/eprint/4476

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