Table 3. Number and types of aminoacidopathies and organic acidurias detected in high-risk Brazilian patients from 2006 to 2016.

Amino acid disorders	Number	Percentage
Maple syrup urine disease	40	34
Phenylketonuria	24	21
Non-ketotic hyperglycinemia	17	14
Tyrosinemia	12	10
Homocystinúria	7	6
Citrullinemia	7	6
Ornithine transcarbamylasedeficiency	4	3
Arginase deficiency	3	3
Carbamoyl phosphate synthetase deficiency	2	2
Argininosuccinic aciduria	1	1
Total	117
Organic acid disorders
Lactic acidemia	64	24.8
Glutaric aciduria type I	59	22.8
Methylmalonic aciduria	40	15.5
3-Hydroxy-3-methylglutaric aciduria	25	9.6
Isovaleric aciduria	12	4.6
Propionic aciduria	12	4.6
L-2-hydroxyglutaric aciduria	11	4.2
Alkaptonuria	10	3.8
Canavan disease	6	2.3
3-Methylglutaconic aciduria	4	1.5
D-2-hydroxyglutaric aciduria	4	1.5
3-Ketothiolase deficiency	4	1.5
Mevalonic aciduria	3	1.1
3-Methyl-Crotonyl-CoA Carboxylase deficiency	3	1.1
Glycerol Kynase deficiency	1	0.3
Total	258