Abstract
Two cases of type I hereditary tyrosinemia are presented. One of them died during a sepsis before beginnning a dietetic treatment; the other one, after having undergone a low in tirosine and fenilalanine diet from the age of 2 months, needed a liver transplant at the age of 7 months because of a severe hepatocelular failure and now evolving satisfactorily, after 5 years since the diagnostic was made.
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