Treatment varies depending on the specific type; a low-protein diet combined with the use of a specially engineered formula to supply protein is required in most cases. Experience with nitisinone has shown it to be effective, especially when started within the first month of life, and it is now the standard course of treatment. It is a 4-hydroxyphenylpyruvate dioxygenase inhibitor indicated for the treatment of hereditary<unk>  type 1 (HT-1) in combination with dietary restriction of tyrosine and phenylalanine. Liver transplant is indicated for patients with<unk>  type I who do not respond to nitisinone, as well as those with acute liver failure and hepatomas.
Tyrosinemia
Hawkinsinuria
Cystathioninuria
Aspartylglucosaminuria
Argininemia
Alkaptonuria
Orotic aciduria
Urocanic aciduria