<unk> tients with<unk>  should be started as early as possible on a low protein diet. In addition to a protein mixture that is devoid of methionine, threonine, valine, and isoleucine, the<unk> tient should also receive L-carnitine treatment and should be given antibiotics 10 days per month in order to remove the intestinal propiogenic flora. The<unk> tient should have diet protocols prepared for them with a “well day diet” with low protein content, a “half emergency diet” containing half of the protein requirements, and an “emergency diet” with no protein content. These<unk> tients are under the risk of severe hyperammonemia during infections that can lead to comatose states. Liver transplant is gaining a role in the management of these<unk> tients, with small series showing improved quality of life.
Propionic acidemia
Organic acidemia
Isovaleric acidemia
Sulfhemoglobinemia
Hyperammonemia
Pipecolic acidemia
Inert gas asphyxiation
Methemoglobinemia