Table 5

Levels of HA and suggested actions in case of symptomatic undiagnosed patients

Ammonia level (μmol/L)ActionInvestigations
Increased above normal limit but ≤100Reduce/stop protein intake, give intravenous glucose
6 mg/kg/min±insulin*
Monitor ammonia blood levels every 3 hours
>100 and <250Start intravenous L-arginine and sodium benzoate, carbamylglutamate, carnitine,
vitamin B12, biotin
Correct electrolytes and phosphate
Monitor ammonia, electrolytes and phosphate
≥250As 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.