Patients with diagnosed<unk><unk>  often have pre-established plans for "sick day" or emergency guidance
Early consultation with specialists
Keep NPO
Removes potential inciting metabolic substrates
IVF
Normal saline 10-20 mL/kg boluses
Reassess after each bolus, as congenital heart disease may mimic symptoms of<unk><unk> 
IVF with dextrose (D10) and 0.45 or 0.9% NS at 1.5-2x maintenance
Aggressive hydration increases urinary excretion of toxic metabolites, dextrose provides metabolic substrate
Removes potential inciting metabolic substrates
Normal saline 10-20 mL/kg boluses
Reassess after each bolus, as congenital heart disease may mimic symptoms of<unk><unk> 
IVF with dextrose (D10) and 0.45 or 0.9% NS at 1.5-2x maintenance
Aggressive hydration increases urinary excretion of toxic metabolites, dextrose provides metabolic substrate
Reassess after each bolus, as congenital heart disease may mimic symptoms of<unk><unk> 
Aggressive hydration increases urinary excretion of toxic metabolites, dextrose provides metabolic substrate
<500 micromoles/L
sodium phenylacetate/sodium benzoate (Ammonul) 250mg/kg in D10 over 90min; then 250mg/kg/d infusion
Arginine 210mg/kg IV/IO in D10 over 90min; then 210mg/kg/d infusion
+/- carnitine, 400 mg IV/IO in consultation w/specialist
May require dialysis if refractory/severe
sodium phenylacetate/sodium benzoate (Ammonul) 250mg/kg in D10 over 90min; then 250mg/kg/d infusion
Arginine 210mg/kg IV/IO in D10 over 90min; then 210mg/kg/d infusion
+/- carnitine, 400 mg IV/IO in consultation w/specialist
May require dialysis if refractory/severe
Sodium bicarbonate 0.5 mEq/kg/h if pH <7.0 
Reserve for severe and/or refractory acidosis due to potential side effects of sodium overload, cerebral edema, cardiac dysfunction
Caution intubating any child in metabolic crisis due to potential worsening of acidosis
Reserve for severe and/or refractory acidosis due to potential side effects of sodium overload, cerebral edema, cardiac dysfunction
Hyperammonemia is risk factor
Mannitol 0.5gm/kg IV/IO
Do not give steroids (worsens hyperammonemia)
Mannitol 0.5gm/kg IV/IO
Do not give steroids (worsens hyperammonemia)
Consider vitamin B6 (pyridoxine) in addition to usual care
Consider empiric antibiotics in all infants in metabolic crisis
Inborn errors of metabolism
THE MISFITS [1]
Thalassemia
Trauma
Retinoic acid syndrome
Heart
Neonatal jaundice
Congenital heart disease