TABLE 95.7 EMERGENT TREATMENT Access and establish airway, breathing, circulation Fluid boluses normal saline, avoid lactated Ringer’s Avoid hypotonic fluid load due to risk of cerebral edema, particularly if hyperammonemia Discontinue intake of offending agents, provide adequate glucose to prevent catabolism NPO (especially no protein, galactose, or fructose) Glucose for hypoglycemia, 0.25–1 g/kg (i.e., D10 neonates; D10 or D25 infant, child) D10 to D15 with electrolytes: 8–12 mg/kg/min IV at 1–1.5 × maintenance to maintain serum glucose level at 120–170 mg/dL If necessary, treat hyperglycemia with insulin to further prevent hyperglycemia Correct metabolic acidosis (pH 300 μg/dL if concentration is rising, prepare for possible dialysis for ammonia >200–250 μg/dL, engaging receiving dialysis unit/facility as soon as possible If dialysis not immediately available or levels >100–125 μg/dL, use sodium phenylacetate, sodium benzoate as Ammonul (Ucyclyd Pharma, 1-888-829-2593) If