Premium
Liver transplantation in tyrosinaemia type 1: the dilemma of timing the operation
Author(s) -
Salt A,
Barnes ND,
Rolles K,
Calne RY,
Clayton PT,
Leonard JV
Publication year - 1992
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1992.tb12270.x
Subject(s) - medicine , hepatocellular carcinoma , occult , liver transplantation , transplantation , liver disease , surgery , pediatrics , pathology , alternative medicine
Four children with tyrosinaemia type I 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.