Tyrosinemia

ICD-10 Code E70.21

Tyrosinemia

Definition

An autosomal recessive inherited metabolic disorder caused by mutations in the FAH, HPD, and TAT genes. It is characterized by deficiency of one of the enzymes that are involved in the metabolism of tyrosine. It results in elevated blood tyrosine levels and accumulation of tyrosine and its byproducts in the liver, kidney, nervous system and other organs.

Also identified as

Drugs indicated

Drug Relation Source
Ascorbic Acid may treat MEDRT · Public domain (U.S. Government work)