Table 1.

Protocol for acute management of primary hyperammonemia based on ammonia level

Ammonia level (μmol/L)	Undiagnosed case	Diagnosed case
Above upper limit of normal	• Stop protein intake	Same as Undiagnosed case
	• Give IV glucose at an appropriate dosage to prevent catabolism ± insulin
>100 but <250 (in neonate	• Same as above	Start medications and nitrogen scavengers according to the protocol of each disorder
>150 but <250)	• Start drug treatment with nitrogen scavengers (L-arginine and AMMONUL®)
	• Start carnitine, biotin, vitamin B12
	• Start Carbaglu®
	• Start lipid IV 2–3 g/kg to give higher calories
250–500	• Same as above	Same as Undiagnosed case
	• Prepare for CRRT
	• Begin CRRT, if no rapid drop of ammonia within 3–6 hours
>500	Start CRRT with above measure

Abbreviations: IV, intravenous; CRRT, continuous renal replacement therapy.