1. iv high-dose glucose (8–12 mg/kg/min) to maintain blood glucose >100 mg/dL (start insulin infusion if hyperglycemia)2. Correct metabolic acidosis, consider hemodialysis or hemofiltration to measure severe hyperammonemia.3. iv L-carnitine at 50–100 mg/kg/day if severe carnitine deficiency4. Avoid administration of intravenous intralipids during an acute metabolic crisis. Here, INS is linked to Hyperammonemia.