<unk> , also called<unk>  aciduria, is an autosomal recessive metabolic disorder that disrupts normal amino acid metabolism. It is a classical type of organic acidemia. The result of this condition is the inability to properly digest specific fats and proteins, which in turn leads to a buildup of a toxic level of<unk>  acid in the blood.<unk>  stems from several genotypes, all forms of the disorder usually diagnosed in the early neonatal period, presenting progressive encephalopathy, and secondary hyperammonemia. The disorder can result in death if undiagnosed or left untreated. It is estimated that this disorder has a frequency of 1 in 48,000 births, though the high mortality rate in diagnosed cases make exact determination difficult.<unk> s are found with an equal frequency across ethnic boundaries.
Methylmalonic acidemia
Hawkinsinuria
Phenylketonuria
Alkaptonuria
Glutaric acidemia type 2
Orotic aciduria
3-Methylglutaconic aciduria
Urocanic aciduria