Inherited metabolic disorders which may cause primary hyperammonaemia (HA)8
| Urea cycle disorders |
| Enzyme Deficiency |
| 1. Ornithine carbamoyl transferase deficiency |
| 2. Carbamoyl phosphate synthetase deficiency |
| 3. Citrullinaemia |
| 4. Argininosuccinic aciduria |
| 5. Argininaemia |
| 6. N-Acetylglutamate synthetase deficiency |
| Transporter Defect |
| 1. Citrin deficiency-aspartate glutamate carrier |
| 2. Hyperornithinaemia, HA and homocitrullinuria (3 hour syndrome)-ornithine transport |