Levels of HA and suggested actions in case of symptomatic undiagnosed patients
| Ammonia level (μmol/L) | Action | Investigations |
| Increased above normal limit but ≤100 | Reduce/stop protein intake, give intravenous glucose 6 mg/kg/min±insulin* | Monitor ammonia blood levels every 3 hours |
| >100 and <250 | Start intravenous L-arginine and sodium benzoate, carbamylglutamate, carnitine, vitamin B12, biotin Correct electrolytes and phosphate | Monitor ammonia, electrolytes and phosphate |
| ≥250 | As above Avoid repetitive drug boluses Begin haemodiafiltration if no rapid drop of ammonia within 3–6 hours | As above Monitor supplement early especially during haemodialysis |
Table is adapted from Häberle et al.66
*If hyperglycaemia (glucose >10 mmol/L) occurs, start insulin infusion as per local diabetic protocol, with close monitoring.