Acta Paediatr 1992 May;81(5):449-52

Liver transplantation in tyrosinaemia type 1: the dilemma of timing the
operation

Salt A, Barnes ND, Rolles K, Calne RY, Clayton PT, Leonard JV

Abstract
Four children with tyrosinaemia type 1 received liver transplants. The metabolic
disorder was corrected and all four had normal liver function on an unrestricted
diet. Two children, transplanted at age five and seven years, proved to have
occult hepatocellular carcinoma and both subsequently developed pulmonary
metastases. One child was well 32 months after removal of a single pulmonary
metastasis but the other child died with multiple metastases. The two younger
children, transplanted at age 19 and 21 months, were well 28 and 44 months after
operation, one after a second liver transplant. Our experience confirms the high
risk of hepatocellular carcinoma in this disease and the potential value of
early liver transplantation