Episode 11a: Crashing Neonate and Metabolic Disorders with Austin Larson

On this episode, host Jason Woods speaks with Dr. Austin Larson, a specialist in pediatric inherited metabolic disorders (and also someone way smarter than I), about what do do with the crashing neonate if there are concerns for a metabolic disorder. We review the diagnostic approach, emergent treatment, and what resources are available to read/consult in this situation.

Important points

  1. If an ill neonate presents and there is any concern for inherited metabolic disorder, starting D10 fluids at 1.5 x maintenance is generally the recommendation, unless there is a reason why glucose/fluids will otherwise harm the patient.
  2. If there are concerns for inherited metabolic disorder, in addition to glucose, checking ketones (urine or serum, though serum preferred), ammonia, and lactate can be very helpful.
  3. If the ammonia level is >200 micromols/L and rising, dialysis will likely be needed and you need to start making plans for this.
  4. Neonates generally can not generate a significant ketosis, even if fasting or ill, without an inherited metabolic disorder.
  5. Other labs to consider obtaining in an ill child with hypoglycemia or concern for metabolic disorder
    1. Cortisol
    2. Serum Amino Acids
    3. Urine Organic Acids
    4. Serum ketones
    5. Free fatty acids
    6. Growth Hormone
    7. Insulin
    8. Acylcarnitine profile

Point of Care Resources

  1. New England Consortium for treatment protocols
  2. Vademecum Metabolicum handbook in book form
  3. App version of Vademecum Metabolicum – eVM

Guests

Austin Larson, MD – Assistant Professor of Pediatrics and Metabolic and Clinical Genetics, University of Colorado School of Medicine and Children’s Hospital Colorado

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