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Liver Transplantation for Hereditary Tyrosinemia: the Quebec Experience

Overview
Journal Am J Hum Genet
Publisher Cell Press
Specialty Genetics
Date 1990 Aug 1
PMID 2378360
Citations 16
Authors
K Paradis
A Weber
E G Seidman
J Larochelle
L Garel
C Lenaerts
C C Roy
Affiliations
Soon will be listed here.
Abstract

Sixteen tyrosinemic patients were evaluated in our institution for a possible liver transplantation. All patients showed biochemical and/or radiological evidence of liver dysfunction. Renal involvement was found to be more abnormal than expected. Seven patients have been transplanted, with two patients receiving a combined liver-kidney transplant. Hepatocarcinoma was detected in two of eight patients in whom the whole liver was examined. Six (37.5%) of the initial 16 patients have died since evaluation, one of the six dying after combined liver-kidney transplantation. Posttransplantation survival was 86%, with normal liver function, normal growth, and no recurrence of neurological crises on a normal diet.

Citing Articles

Diagnosis, treatment, management and monitoring of patients with tyrosinaemia type 1: Consensus group recommendations from the German-speaking countries.

Das A, Ballhausen D, Haas D, Haberle J, Hagedorn T, Janson-Mutsaerts C J Inherit Metab Dis. 2024; 48(1):e12824.

PMID: 39676394 PMC: 11647197. DOI: 10.1002/jimd.12824.

Case of hepatocellular carcinoma in a patient with hereditary tyrosinemia in the post-newborn screening era.

Imseis E, Bynon J, Thornhill C World J Hepatol. 2017; 9(9):487-490.

PMID: 28396719 PMC: 5368626. DOI: 10.4254/wjh.v9.i9.487.

Early nitisinone treatment reduces the need for liver transplantation in children with tyrosinaemia type 1 and improves post-transplant renal function.

Bartlett D, Lloyd C, McKiernan P, Newsome P J Inherit Metab Dis. 2014; 37(5):745-52.

PMID: 24515874 DOI: 10.1007/s10545-014-9683-x.

Clinical and para clinical findings in the children with tyrosinemia referring for liver transplantation.

Dehghani S, Haghighat M, Imanieh M, Karamnejad H, Malekpour A Int J Prev Med. 2014; 4(12):1380-5.

PMID: 24498493 PMC: 3898443.

Hepatorenal tyrosinemia.

Kitagawa T Proc Jpn Acad Ser B Phys Biol Sci. 2012; 88(5):192-200.

PMID: 22687740 PMC: 3410490. DOI: 10.2183/pjab.88.192.

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