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Liver transplantation for hereditary tyrosinemia: the Quebec experience.

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.

journal_name

Am J Hum Genet

journal_title

American journal of human genetics

authors

Paradis K,Weber A,Seidman EG,Larochelle J,Garel L,Lenaerts C,Roy CC

subject

Has Abstract

pub_date

1990-08-01 00:00:00

pages

338-42

issue

2

eissn

0002-9297

issn

1537-6605

journal_volume

47

pub_type

杂志文章

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