Tyrosinemia

Hereditary (autosomal recessive) defect in tyrosine metabolism caused by an enzyme lack and resulting in liver and kidney disturbances and mental retardation. Treatment is by a diet low in tyrosine and phenylalanine.


A disease of tyrosine metabolism caused by a deficiency of the enzyme tyrosine aminotransferase. In addition to an accumulation of tyrosine in the blood, mental retardation, keratitis, and dermatitis are present. Treatment consists of controlling phenylalanine and tyrosine intake.


 


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