If left untreated,<unk>  will lead to death due to central neurological function failure and respiratory failure. Early detection, diet low in branched-chain amino acids, and close monitoring of blood chemistry can lead to a good prognosis with little or no abnormal developments. Average intellectual development is below that of the general population and the severity of the deficit is a related to the time the condition remained undiagnosed and the effectiveness of dietary control including during metabolic crises.
Maple syrup urine disease
Urocanic aciduria
Alpha-aminoadipic and alpha-ketoadipic aciduria
Hawkinsinuria
3-Methylglutaconic aciduria
Homocystinuria
Alkaptonuria
2-Hydroxyglutaric aciduria