ED Initial Management of Inborn Errors of Metabolism

1. IV fluids – If severe dehydration bolus as you normally would to adequately resuscitate a child

2. Simultaneously start IV infusion of D10 at 1.5-2x maintenance rate. This delivers 10-15mg/kg/min of glucose with the goal of minimizing catabolism.

Review: Maintenance fluids in pediatrics: 1st 10kg, 4cc/kg/hr. 11-20kg 2cc/kg/hr. >20kg add an additional 1cc/kg/hr for each kilo above 20kg.

3. Only consider bicarb if severe acidosis with pH of <7.1

4. Be sure to be checking VBG and ammonia levels in addition to your standard metabolic panel. In all cases of known inborn errors of metabolism presenting with even minor illnesses and all cases of new hyperammonemia be sure to consult you metabolic or genetic specialist very early to tailor therapy.

5. In severe situations may need to progress to hemodialysis.

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