Pediatric emergency medicine trisk 2442 2442

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 <7.0–7.2) slowly, cautiously
Sodium bicarbonate and/or potassium acetate: 0.25–0.5 mEq/kg/hr (up to 1–2 mEq/kg/hr) IV; if
intractable acidosis, consider hemodialysis (peritoneal dialysis, hemofiltration, exchange
transfusion much less effective)
Eliminate toxic metabolites
Hyperammonemia therapy
For organic acidopathies, fatty acid oxidation defects, hyperammonemia is usually corrected by
treatment of dehydration, acidosis, and hypoglycemia . Hemodialysis should be considered for
persistent hyperammonemia for these conditions or suspected IEM
For urea cycle defects, recommendations of the New England Consortium are to perform dialysis
for ammonia >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 <20 kg load 250 mg/kg (2.5 mL/kg) in 10%
glucose via central line over 90–120 min, then 250 mg/kg/day (2.5 mL/kg/day) in 10% glucose via
central line continuous infusion, if ≥20 kg 5.5 g/m2 (55 mL/m2) over 90–120 min, then 5.5
g/m2/day (55 mL/m2/day) via central line; arginine HCl 600 mg/kg (6 mL/kg) IV in 10% glucose
over 90–120 min, then 600 mg/kg/day IV continuous infusion. Ammonul must be given by central
line. Arginine HCl can be mixed with Ammonul. Can decrease arginine HCl doses to 200 mg/kg if
carbamoyl phosphate deficiency, ornithine transcarbamylase deficiency. l-carnitine conjugates

with and inactivates sodium benzoate; therefore, it must not be given with Ammonul. Has also
been used for neonatal hyperammonemic coma of unknown etiology
Administer cofactors if indicated
Pyridoxine (B6 ) 100 mg IV for possible pyridoxine-responsive disorder (seizures unresponsive to
conventional anticonvulsants)
Folic acid as leucovorin; 2.5 mg IV for possible folate-responsive disorder (seizures unresponsive to
conventional anticonvulsants)
Biotin 10–40 mg NG tube for possible biotin-responsive disorder (seizures unresponsive to
conventional anticonvulsants)
L -carnitine 25–50 mg/kg over 2–3 min or as an infusion added to the maintenance fluid, followed
by 25–50 mg/kg over 24 hrs, max 100 mg/kg not to exceed 3 g/day for presumed carnitine
deficiency if life-threatening manifestations. Use is controversial, consultation with an IEM
specialist is recommended
Adapted from Weiner DL. Inborn errors of metabolism. In: Aghababian RV, ed. Emergency Medicine: The Core Curriculum . Philadelphia,
PA: Lippincott-Raven; 1999:707.